Sella turcica morphology and the pituitary gland-a new contribution to craniofacial diagnostics based on histology and neuroradiology.

PubMed

Kjær, Inger

2015-02-01

The present review summarizes two decades of published and unpublished studies on normal and pathological development of sella turcica and pituitary gland in humans. The pathological conditions are studied in known genotype deviations, syndromes, and other malformations. The studies include histological analyses of human prenatal material and profile radiographic analyses of human postnatal material, supplemented in a few cases with neuroradiology. Prenatal and postnatal results are compared. Similarities between prenatal and postnatal deviations in sella turcica morphology were demonstrated. Malformations in the pituitary gland were observed in several cases. For diagnostic purposes, the review distinguishes between deviations in the anterior wall and in the posterior wall of the sella turcica. Deviations in the anterior wall seem to be associated with deviations specifically in the frontonasal developmental field, while deviations in the posterior wall are often connected with malformations in the posterior structures, e.g. the cerebellum. In normal cases, minor variations in morphology are observed. In each pathological case, a specific malformation pattern was observed in sella turcica morphology, varying from mild to severe phenotype. The malformation in the sella turcica/pituitary gland can be associated with a malformation within a developmental field that forms the craniofacial region (frontonasal, maxillary, palatal, and mandibular fields), sometimes also involving the brain stem, thymus, thyroid, and heart (velocardiofacial syndrome). Pathological sella turcica morphology can also be associated with malformations in the cerebellum and larynx (Cri-du-Chat syndrome). This review demonstrates the value of combining profile radiographic diagnostics with neuroradiological diagnostics in cases with malformed sella turcicae. © The Author 2012. Published by Oxford University Press on behalf of the European Orthodontic Society. All rights reserved. For

A cone beam computed tomographic evaluation of the size of the sella turcica in patients with cleft lip and palate.

PubMed

Paknahad, Maryam; Shahidi, Shoaleh; Khaleghi, Iman

2017-09-01

Changes in the size of the sella turcica are frequently related to pathologies and syndromes. The aim of this was to compare the sella turcica dimensions in patients with unilateral and bilateral cleft lip and palate and non-cleft subjects. Cone beam computed tomography (CBCT) images of three groups consisted of 20 patients with unilateral cleft lip and palate; 20 patients with bilateral cleft lip and palate and a control group consisting of 20 non-cleft subjects were the research population in this pilot study. The sella turcica linear dimensions in terms of length, depth and diameter were measured for all subjects. One-way ANOVA test was used to determine any significant differences among the three groups for the measured parameters. The length, depth and diameter of sella turcica were found to be significantly smaller in the unilateral and bilateral groups compared with the normal age and gender matched group. No significant differences were found in the measured variables between the unilateral and bilateral cleft patients. CBCT images showed a greater likelihood of abnormal sella turcica dimensions in patients with unilateral and bilateral cleft lip and palate. Therefore, the sella turcica dimensions may have an intrinsic relationship to the cleft condition.

Assessment of sella turcica area and skeletal maturation patterns of children with unilateral cleft lip and palate.

PubMed

Cesur, E; Altug, A T; Toygar-Memikoglu, U; Gumru-Celikel, D; Tagrikulu, B; Erbay, E

2018-05-01

The aim of this case-control study was to assess sella turcica area and skeletal maturity in children with unilateral cleft lip and palate (UCLP) and compare with those of non-cleft children. A total of 85 UCLP patients aged 7.5-17.08 years (Group 1: age 7-11 years, Group 2: age 11-14 years and Group 3: age 14-18 years) were compared with 85 control subjects without clefts who were divided into similar age groups. Hand-wrist radiographs and cervical vertebra maturation stages (CVMS) were used to evaluate growth. Lateral cephalograms were traced, and reference points of sella were determined. Sella turcica area was measured using a digital planimeter. Comparison of overall growth on hand-wrist radiographs revealed no significant difference between cleft and non-cleft subjects. However, according to the chronological age groups, Group 1-UCLP showed statistically significant delay in skeletal maturation when compared with the age-matched control subjects (P = .05). This difference was due to the delay among male subjects (P = .05). As for CVMS, more significant maturation delay was observed in Group 1-UCLP (P = .001) and was attributable to both male and female subjects (P = .05). Comparison of sella turcica area showed no significant difference between UCLP patients and controls. Although children with CLP showed significant delay in growth when they are younger compared with the non-cleft children, sella turcica area measurements were similar for individuals in both groups. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Pituitary stalk compression by the dorsum sellae: possible cause for late childhood onset growth disorders.

PubMed

Taoka, Toshiaki; Iwasaki, Satoru; Okamoto, Shingo; Sakamoto, Masahiko; Nakagawa, Hiroyuki; Otake, Shoichiro; Fujioka, Masayuki; Hirohashi, Shinji; Kichikawa, Kimihiko

2006-06-01

The purpose of this study was to evaluate the relationship between pituitary stalk compression by the dorsum sellae and clinical or laboratory findings in short stature children. We retrospectively reviewed magnetic resonance images of the pituitary gland and pituitary stalk for 34 short stature children with growth hormone (GH) deficiency and 24 age-matched control cases. We evaluated the degree of pituitary stalk compression caused by the dorsum sellae. Body height, GH level, pituitary height and onset age of the short stature were statistically compared between cases of pituitary stalk compression with associated stalk deformity and cases without compression. Compression of the pituitary stalk with associated stalk deformity was seen in nine cases within the short stature group. There were no cases observed in the control group. There were no significant differences found for body height, GH level and pituitary height between the cases of pituitary stalk compression with associated stalk deformity and cases without compression. However, a significant difference was seen in the onset age between cases with and without stalk compression. Pituitary stalk compression with stalk deformity caused by the dorsum sellae was significantly correlated with late childhood onset of short stature.

Clinical characteristics of acromegalic patients with empty sella and their outcomes following transsphenoidal surgery.

PubMed

Sasagawa, Yasuo; Hayashi, Yasuhiko; Tachibana, Osamu; Oishi, Masahiro; Fukui, Issei; Iizuka, Hideaki; Nakada, Mitsutoshi

2017-08-01

To analyze the clinical characteristics of acromegalic patients with empty sella (ES, herniation of the subarachnoid space within the sella turcica) and the impact of ES on transsphenoidal surgery in such patients. Seventy-eight patients, newly diagnosed with acromegaly who underwent transsphenoidal surgery were included. ES was defined as the pituitary gland and adenoma occupying less than 50% of the sella turcica on midsagittal magnetic resonance (MR) imaging. Twelve patients (15.4%), predominantly female (10 women, p = .047), had ES in preoperative MR imaging. ES patients had smaller mean tumor diameter (6.3 mm) than non-ES patients (11.2 mm, p = .001). In preoperative MR imaging, occult adenoma was found in three (25%) ES and three (4.5%) non-ES patients (p = .044). Intraoperative cerebrospinal fluid (CSF) leakage was more frequent in the ES patients than in the non-ES patients (58.3 vs. 25.8%, p = .024). This led to an increased rate of sellar floor reconstruction using abdominal fat and/or postoperative lumber drainage in the ES patients (ES: 41.7 vs. non-ES: 16.7%, p = .063). Endocrinological remission after surgery was more frequent in the non-ES patients (72.7%) than in the ES patients (58.3%) (p = .248). Co-existence of acromegaly with ES is not rare, and is associated with occult adenoma, intra/postoperative CSF leakage, and a worse endocrinological outcome after transsphenoidal surgery; although, the underlying mechanism remains unclear.

Panhypopituitarism with empty sella a sequel of pituitary hyperplasia due to chronic primary hypothyroidism.

PubMed

Dutta, Deep; Maisnam, Indira; Ghosh, Sujoy; Mukhopadhyay, Pradip; Mukhopadhyay, Satinath; Chowdhury, Subhankar

2012-12-01

Asymptomatic reversible pituitary hyperplasia is common in patients with untreated primary hypothyroidism. Occurrence of empty sella (ES) in this scenario is extremely rare (only three reports till the date) and panhypopituitarism has not been reported in such patients. We report a 27 year man with severe short stature (height-133 cm; standard deviation score-7.36) and delayed puberty who had symptoms suggestive of hypothyroidism along with chronic persistent headache since 6 years of age. Pituitary imaging done for headache at 11 years age showed pituitary hyperplasia. He was diagnosed of primary hypothyroidism for the 1(st) time at 21 year age, a diagnosis which was likely missed for 15 years. Levothyroxine therapy leads to resolution of all symptoms and a height gain of 28 cm over last 6 years. Evaluation for lack of progression of puberty along with chronic nausea, vomiting, fatigue and weight loss for the last 1 year revealed secondary hypocortisolism (9 am cortisol-4.8 mcg/dl; ACTH-3.2 pg/ml), growth hormone deficiency (IGF-1: 65 ng/ml; normal: 117-325 ng/ml) and hypogonadotrophic hypogonadism (9 am testosterone: 98 ng/dl; [280-1500] LH-0.01 mIU/L [1.14-5.75]) with ES on magnetic resonance imaging (MRI) brain. Uncontrolled thyrotroph hyperplasia due to chronic untreated primary hypothyroidism for 15 years may have been damaging the adjacent corticotrophs, somatotrophs and gonadotrophs resulting in panhypopituitarism and empty sella. This is perhaps the first report of panhypopituitarism with empty sella syndrome developing in a patient with pituitary hyperplasia, a sequel of chronic untreated primary hypothyroidism.

Schizophernia and empty sella – casual or correlated?

PubMed Central

Wix-Ramos, Richard Joseph; Capote, Eduardo; Mendoza, Milet; Garcia, Margreth; Ezequiel, Uribe

2011-01-01

Summary A male patient, 44 years old, with schizophrenia which started at the age of 18. At his last follow-up visit, laboratory tests and brain magnetic resonance imaging (MRI) were performed, revealing the presence of a sellar arachnoidocele. To our knowledge, there is only one similar case report of a set of male monozygotic triplets with schizophrenia and empty sella syndrome. High-resolution chromosome analysis found an extra band at chromosome 15p in all the triplets and their father. We performed a similar evaluation in our patient and his family to compare the results and identify new information on neuroanatomical abnormalities, hormonal alterations or genetic origins of schizophrenia. PMID:22802833

Diabetes Insipidus and Polydipsia in a Patient with Asperger's Disorder and an Empty Sella: A Case Report.

ERIC Educational Resources Information Center

Raja, Michele; Azzoni, Antonella; Giammarco, Vincenzo

1998-01-01

Describes an Italian patient with Asperger disorders, Neurogenic Diabetes Insipidus, and Primary Empty Sella. His response to vasopressin treatment suggested a concomitant presence of primary polydipsia. Implications of the observed concurrence of these rare disorders are discussed in relation to diagnosis and pathogenesis. (Author/CR)

Anatomical features of skull base and oral cavity: a pilot study to determine the accessibility of the sella by transoral robotic-assisted surgery.

PubMed

Amelot, Aymeric; Trunet, Stephanie; Degos, Vincent; André, Olivier; Dionnet, Aurore; Cornu, Philippe; Hans, Stéphane; Chauvet, Dorian

2015-10-01

The role of transoral robotic surgery (TORS) in the skull base emerges and represents the natural progression toward miniinvasive resections in confined spaces. The accessibility of the sella via TORS has been recently described on fresh human cadavers. An anatomic study is mandatory to know if this approach would be feasible in the majority of patients regardless of their oral morphological features. From 30 skull base CT scans from patients who were asked to open their mouth as wide as they can, we measured specific dimensions of the oral cavity and the skull base, such as length of the palate, mouth opening and distance from the sella to the palate. All data were acquired on a sagittal midline plane and on a 25° rotation plane, which simulated the axis of the robotic instruments. Looking at the projection of the dental palatine line on the sella, we studied possible predictive factors of sellar accessibility and tried to bring objective data for surgical feasibility. We also proposed an angle α to study the working angle at the skull base. We observed that the maximal mouth opening was a good predictive factor of sellar accessibility by TORS (p < 0.05). The mouth aperture threshold value for a good sensitivity, over 80 %, was comparable to the mean value of mouth opening in our series, 38.9 and 39.4 mm respectively. Moreover, we showed a statistically significant increase of the working angle α at the skull base comparing the lateral access to the midline one (p < 0.05). This seemed to quantitatively demonstrate that the robotic arms placed at the labial commissure of the mouth can reach the sella. From these anatomical features and previous cadaveric dissections, we assume that TORS may be feasible on a majority of patients to remove pituitary adenomas.

Associations among sella turcica bridging, atlas arcuate foramen (ponticulus posticus) development, atlas posterior arch deficiency, and the occurrence of palatally displaced canine impaction.

PubMed

Haji Ghadimi, Mona; Amini, Fariborz; Hamedi, Shayesteh; Rakhshan, Vahid

2017-03-01

Head and neck skeletal anomalies or normal variants might predict the occurrence of palatally displaced impacted maxillary canines. Despite their clinical importance, studies in this regard are rare, especially when it comes to vertebral anomalies. This case-control study was performed on cephalographs of 35 orthodontic patients (11 male, 24 female) with palatally displaced canines (PDC) and 75 patients without them (29 male, 46 female). PDC were diagnosed on panoramic and lateral cephalographs and from clinical reports. The occurrence and severity of sella turcica bridge and the atlas ponticulus posticus, and deficiency of the posterior atlas arch were evaluated twice on lateral cephalographs. The associations between the occurrence and level of these skeletal anomalies and variations of PDC occurrence as well as additional correlations were assessed using multivariable and bivariate statistics (α = 0.05; β ≤0.2). The patients' mean age was 18.4 ± 1.9 years. In the control and patient groups, 23 (30.7%) and 21 subjects (60%) had sella turcica bridging, respectively (chi-square, P = 0.003). Ponticulus posticus was observed in 14 (18.7%) controls and 15 (42.9%) patients (chi-square, P = 0.007). Posterior atlas arch deficiency was observed in 4 (5.3%) controls and 5 (14.3%) patients (chi-square, P = 0.111). The presence of ponticulus posticus and sella turcica bridging might be associated with increased odds of PDC occurrence for about odds ratios of 3.1 and 3.5 times, respectively (binary logistic regression). PDC is positively associated with the occurrence and severity of sella turcica bridging and ponticulus posticus. The association between PDC and posterior atlas arch deficiency was inconclusive. Copyright © 2016 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

TSH and prolactin responses to thyrotropin releasing hormone (TRH) and domperidone in patients with empty sella syndrome.

PubMed

Valensi, P; Combes, M E; Perret, G; Attali, J R

1996-05-01

The aim of this study was to investigate TSH and PRL response to TRH and domperidone, an antidopaminergic drug which does not cross the blood-brain barrier, in 16 patients with primary empty sella (PES) and either normal or elevated plasma PRL level and to compare it with the response observed in 8 patients with prolactinoma. In the patients with PES and hyperprolactinemia, the PRL response to TRH was significantly lower than in the controls and the patients with PES and normal PRL, which suggests there is impaired PRL synthesis and release in cases of PES with hyperprolactinemia. The TSH response to domperidone was significantly elevated in patients with PES and either normal or elevated PRL, as in patients with prolactinoma. The PRL response to domperidone was significantly reduced in patients with PES and hyperprolactinemia as in patients with prolactionoma. These results suggestthat in PES with prolactinoma the inhibiting dopaminergic tone is increased on the thyrotropic cells and reduced on the lactotropic cells in PES with elevated PRL and that some patients with PES might bear a microprolactinoma in the bottom of the sella which remained undetected by the CT scan.

Painful ophthalmoplegia secondary to a mucocele involving the sella turcica, superior orbital fissure, and sphenoid sinus.

PubMed

Clarke, H; Clarke, V; Gill, J; St John, A; Lashley, M

1992-03-01

A case of painful ophthalmoplegia associated with an extensive lesion involving the sella turcica, superior orbital fissure, and sphenoid sinus in a 57-year-old man is reported. Even though nasal and ocular symptoms and signs represent the usual features of sphenoidal mucoceles, extension to the intracranial cavity as seen in this lesion is rare. Surgical exploration via a sublabial, transseptal approach revealed a mucocele of the sphenoid sinus. This case exhibited extensive and aggressive behavior simulating a malignant neoplasm.

High frequency of empty sella syndrome in children with growth hormone deficiency.

PubMed

Pocecco, M; de Campo, C; Marinoni, S; Tommasini, G; Basso, T; Muzzolini, C; Sacher, B

1989-02-01

Computer-assisted tomography (CT) with 2 mm axial sections and reconstructions was carried out in 31 children affected by GH deficiency (GHD): 18 with idiopathic complete isolated GHD, 3 with idiopathic partial isolated GHD, 2 with idiopathic panhypopituitarism, 4 with isolated acquired GHD and 4 with acquired panhypopituitarism. Density in the intrasellar area on CT corresponded to that of cerebrospinal fluid in 13/20 cases with idiopathic hypopituitarism and in 2/8 cases with acquired hypopituitarism. The overall incidence of primary empty sella syndrome (PESS) in the GH deficient patients studied was thus over 48%, while in children without endocrine dysfunction, it was only 5/213 (2.4%). It is concluded that PESS is more frequent in childhood than assumed until now and that it is frequently associated with GHD.

TFE3-Expressing Perivascular Epithelioid Cell Neoplasm (PEComa) of the Sella Turcica.

PubMed

Hyrcza, Martin D; Winer, Daniel A; Shago, Mary; Au, Karolyn; Zadeh, Gelareh; Asa, Sylvia L; Mete, Ozgur

2017-03-01

We report a primary central nervous system (CNS) perivascular epithelioid cell tumor (PEComa) in a middle-aged female patient. The tumor occurred in suprasellar location with secondary extension into the sella turcica. The patient presented with intracranial hemorrhage and an altered level of consciousness. The tumor had morphologic features matching those of other previously described TFE3-translocated PEComas, including epithelioid morphology, diffuse and strong nuclear immunoreactivity for TFE3, and minimal staining with myoid markers. The TFE3 break-apart FISH testing showed a slight splitting of one of the TFE3 signals in 49.5 % of nuclei. This case illustrates that PEComas should be added to the growing list of mesenchymal tumors that can be encountered in the CNS and specifically in the vicinity of the pituitary gland. The recognition of this entity is of significance given their underlying pathogenesis and possible management implications.

Extended endoscopic transsphenoidal approach infrachiasmatic corridor.

PubMed

Ceylan, Savas; Anik, Ihsan; Koc, Kenan; Cabuk, Burak

2015-01-01

An extended endoscopic transsphenoidal approach is required for skull base lesions extending to the suprasellar area. Inferior approach using the infrachiasmatic corridor allows access to the lesions through the tumor growth that is favorable for the extended transsphenoidal approaches. Infrachiasmatic corridor is a safer route for the inferior approaches that is made up by basal arachnoid membrane and Liliequist's membrane with its leaves (diencephalic and mesencephalic leaf). This area extends from the optic canal and tuberculum sella to the corpus mamillare. We performed extended endoscopic approach using the infrachiasmatic corridor in 52 cases, including tuberculum sella meningiomas (n:23), craniopharyngiomas (n:16), suprasellar Rathke's cleft cyst (n:6), pituitary adenoma (n:2), fibrous dysplasia (n:1), infundibular granulosa cell tumor (n:2), and epidermoid tumor (n:2). Total resection was achieved in 17 of 23 (74%) with tuberculum sellae meningioma using infrachiasmatic approach. Twenty patients presented with visual disorders and 14 of them improved. There were two postoperative cerebrospinal fluid (CSF) leakages and one transient diabetes insipidus and one permanent diabetes insipidus. Sixteen patients were operated on by the infrachiasmatic approach for craniopharyngiomas. Improvement was reached in seven of eight patients presented with visual disorders. Complete tumor resection was performed in 10 of 16 cases and cyst aspiration in 4 cases, and there were remnants in two cases. Postoperative CSF leakage was seen in two patients. Infrachiasmatic corridor provides an easier and safer inferior route for the removal of middle midline skull base lesions in selected cases.

Pituitary Adenoma Recurrence Suspected on Central Hyperthyroidism Despite Empty Sella and Confirmed by 68Ga-DOTA-TOC PET/CT.

PubMed

Gauthé, Mathieu; Sarfati, Julie; Bourcigaux, Nathalie; Christin-Maitre, Sophie; Talbot, Jean-Noël; Montravers, Françoise

2017-06-01

Thyrotropin-secreting pituitary adenomas are very rare tumors, known to present overexpression of somatostatin receptor subtype 2 and which may consequently demonstrate abnormal uptake on Ga-DOTA-TOC PET/CT. A 67-year-old woman with a history of operated pituitary macroadenoma presented with symptoms of hyperthyroidism including a large goiter. Her serum thyroid hormone levels were in favor of central hyperthyroidism. Pituitary MRI depicted an empty sella but visualized an ambiguous lesion centered on the left sphenoidal sinus. Complementary Ga-DOTA-TOC PET/CT finally demonstrated intense uptake by the sphenoidal lesion, confirming recurrence of the pituitary adenoma.

Evolution of Minimally Invasive Approaches to the Sella and Parasellar Region

PubMed Central

Louis, Robert G.; Eisenberg, Amy; Barkhoudarian, Garni; Griffiths, Chester; Kelly, Daniel F.

2014-01-01

Introduction Given advancements in endoscopic image quality, instrumentation, surgical navigation, skull base closure techniques, and anatomical understanding, the endonasal endoscopic approach has rapidly evolved into a widely utilized technique for removal of sellar and parasellar tumors. Although pituitary adenomas and Rathke cleft cysts constitute the majority of lesions removed via this route, craniopharyngiomas, clival chordomas, parasellar meningiomas, and other lesions are increasingly removed using this approach. Paralleling the evolution of the endonasal route to the parasellar region, the supraorbital eyebrow craniotomy has also been increasingly used as an alternative minimally invasive approach to reach this skull base region. Similar to the endonasal route, the supraorbital route has been greatly facilitated by advances in endoscopy, along with development of more refined, low-profile instrumentation and surgical navigation technology. Objectives This review, encompassing both transcranial and transsphenoidal routes, will recount the high points and advances that have made minimally invasive approaches to the sellar region possible, the evolution of these approaches, and their relative indications and technical nuances. Data Synthesis The literature is reviewed regarding the evolution of surgical approaches to the sellar region beginning with the earliest attempts and emphasizing technological advances, which have allowed the evolution of the modern technique. The surgical techniques for both endoscopic transsphenoidal and supraorbital approaches are described in detail. The relative indications for each approach are highlighted using case illustrations. Conclusions Although tremendous advances have been made in transitioning toward minimally invasive transcranial and transsphenoidal approaches to the sella, further work remains to be done. Together, the endonasal endoscopic and the supraorbital endoscope-assisted approaches are complementary

Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease.

PubMed

Mehta, Gautam U; Bakhtian, Kamran D; Oldfield, Edward H

2014-09-01

Primary empty sella syndrome (ESS) results from herniation of arachnoid mater into the pituitary fossa. It has been suggested to have a negative effect on pituitary surgery; however, outcomes in this cohort have not been defined. This study was performed to determine the effect of ESS on immediate and long-term biochemical outcome after pituitary surgery for Cushing's disease (CD). Using a matched cohort study design, the authors followed patients treated with pituitary surgery for CD with and without ESS. Complete ESS was defined as pituitary gland height ≤ 2 mm, whereas partial ESS was defined as pituitary gland height > 2 mm but less than three-quarters of the total sellar depth. The primary end points were immediate and long-term biochemical outcome. Cerebrospinal fluid leaks were recorded as a secondary end point. Seventy-eight patients with CD and primary ESS were identified and matched with 78 patients with CD without ESS. After surgical management, immediate biochemical remission was achieved in 69 patients (88%) with ESS and 75 controls (96%, p = 0.10). Long-term remission was achieved in most patients in both groups (5-year cure: 85% vs 92%, p = 0.10). Among patients with ESS, the presence of complete ESS predicted a worse long-term outcome (p = 0.04). Intraoperative CSF leaks were significantly more frequent with ESS (54% vs 24%, p < 0.001), and despite sellar floor repair, the rate of postoperative CSF leaks was also increased (6% vs 3%, p = 0.27). Biochemical outcome after pituitary surgery for CD was worse in patients with complete ESS, and the risk of a CSF leak was increased with both partial and complete ESS. However, as outcome remains superior to those following alternative therapies and the biology of these tumors is unchanged in the setting of ESS, pituitary surgery should remain the initial treatment of choice.

Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome

PubMed Central

Arzamendi, Audrey E.; Shahlaie, Kiarash; Latchaw, Richard E.; Lechpammer, Mirna; Arzumanyan, Hasmik

2016-01-01

Objective  To describe the work-up and treatment of rare ectopic acromegaly caused by a biopsy-proven somatotroph pituitary adenoma located within the bony intersphenoid septum of a patient with empty sella syndrome (ESS). Methods  We report the presentation, clinical course, diagnostic work-up, and lesion localization and treatment challenges encountered in a 55-year-old patient, with a brief review of relevant literature. Results  A 55-year-old African-American man presented with acromegaly and ESS. Attempts to definitively localize the causative tumor were unsuccessful, though petrosal sinus sampling supported central growth hormone production and imaging suggested bone-enclosed subsellar pituitary tissue. Endoscopic endonasal transphenoidal exploration was undertaken with resection of a somatotroph pituitary microadenoma, and subsequent clinical improvement and biochemical remission. Retrospective review revealed the patient's pituitary to have been located ectopically within a unique bony intersphenoid septum. Conclusion  This report describes the first known case of an ectopic pituitary adenoma located within the midline bony intersphenoid septum, which we postulate to have resulted from anomalous embryological pituitary migration. Intra-intersphenoid septal tumors should be considered in cases of apparent central acromegaly with ESS or absence of tumor tissue within the paranasal sinuses or other peripheral locations. Indexing  Acromegaly, ESS, pituitary adenoma, sphenoid sinus septum. PMID:27468406

Safety of drilling for clinoidectomy and optic canal unroofing in anterior skull base surgery.

PubMed

Spektor, Sergey; Dotan, Shlomo; Mizrahi, Cezar José

2013-06-01

Skull base drilling is a necessary and important element of skull base surgery; however, drilling around vulnerable neurovascular structures has certain risks. We aimed to assess the frequency of complications related to drilling the anterior skull base in the area of the optic nerve (ON) and internal carotid artery (ICA), in a large series of patients. We included anterior skull base surgeries performed from 2000 to 2012 that demanded unroofing of the optic canal, with extra- or intradural clinoidectomy and/or drilling of the clinoidal process and lateral aspect of the tuberculum sella. Data was retrieved from a prospective database and supplementary retrospective file review. Our IRB waived the requirement for informed consent. The nature and location of pathology, clinical presentation, surgical techniques, surgical morbidity and mortality, pre- and postoperative vision, and neurological outcomes were reviewed. There were 205 surgeries, including 22 procedures with bilateral optic canal unroofing (227 optic canals unroofed). There was no mortality, drilling-related vascular damage, or brain trauma. Complications possibly related to drilling included CSF leak (6 patients, 2.9 %), new ipsilateral blindness (3 patients, 1.5 %), visual deterioration (3 patients, 1.5 %), and transient oculomotor palsy (5 patients, 2.4 %). In all patients with new neuropathies, the optic and oculomotor nerves were manipulated during tumor removal; thus, new deficits could have resulted from drilling, or tumor dissection, or both. Drilling of the clinoid process and tuberculum sella, and optic canal unroofing are important surgical techniques, which may be performed relatively safely by a skilled neurosurgeon.

Expanded Endoscopic Endonasal Approaches to Skull Base Meningiomas

PubMed Central

Prosser, J. Drew; Vender, John R.; Alleyne, Cargill H.; Solares, C. Arturo

2012-01-01

Anterior cranial base meningiomas have traditionally been addressed via frontal or frontolateral approaches. However, with the advances in endoscopic endonasal treatment of pituitary lesions, the transphenoidal approach is being expanded to address lesions of the petrous ridge, anterior clinoid, clivus, sella, parasellar region, tuberculum, planum, olfactory groove, and crista galli regions. The expanded endoscopic endonasal approach (EEEA) has the advantage of limiting brain retraction and resultant brain edema, as well as minimizing manipulation of neural structures. Herein, we describe the techniques of transclival, transphenoidal, transplanum, and transcribiform resections of anterior skull base meningiomas. Selected cases are presented. PMID:23730542

Visual disability rates in a ten-year cohort of patients with anterior visual pathway meningiomas.

PubMed

Bor-Shavit, Elite; Hammel, Naama; Nahum, Yoav; Rappaport, Zvi Harry; Stiebel-Kalish, Hadas

2015-01-01

To examine the visual outcome of anterior visual pathway meningioma (AVPM) patients followed for at least one year. Data were collected on demographics, clinical course and management. Visual disability was classified at the first and last examination as follows: I--no visual disability; II--mild visual defect in one eye; III--mild visual defect in both eyes; IV--loss of driver's license; V--legally blind. Eight-one AVPM patients had their tumor originate in the clinoid process in 23 (28%), sphenoid-wing area in 18 (22%), cavernous sinus in 15 (19%), tuberculum sellae in 8 (10%), and mixed in 17 (21%). On last examination, 46 patients (57%) had good visual acuity in one or both eyes (Class I or II) and 17 (21%) were mildly affected in both eyes. The rate of Class IV disability was 16%, and Class V disability was 6%. Attention needs to be addressed to the considerable proportion of patients with AVPM (22% in this study) who may lose their driver's license or become legally blind. Occupational therapists should play an important role in the multidisciplinary management of those patients to help them adapt to their new physical and social situation. Anterior visual pathway meningiomas (AVPMs) are commonly not life-threatening but they can lead to profound visual disability, especially when the tumor originates in the tuberculum sellae and cavernous sinus. Particular attention should be paid to visual acuity and visual field deficits, as these can profoundly affect the patient's quality of life including ability to drive and activities of daily living. The interdisciplinary management of patients with AVPM should include the neurosurgeon, neuro-ophthalmologist and occupational therapist. Also, early intervention by the occupational therapist can help patients adapt to their current physical and social situation and return to everyday tasks more rapidly.

[The growing skull. Part I. Neurocranium. Statistical considerations (author's transl)].

PubMed

Gefferth, K

1976-01-01

Measurements were made on the radiographs of the skull of 540 boys and 496 girls obtained in the years 1951-1968. Distances and angles were established with the Tuberculum sellae as the centre. The subjects ranged in age from the first day of life till late puberty. They were divided into 26 groups comprising smaller periods in earlier life, and increasing with age. Results are presented of measurements of 9 distances and 3 angles exclusively concerning the neurocranium. The neurocranium of the girls is smaller than that of the boys from the first day of life and the difference is growing with age. The angles displayed little sex differences. The greater part of the growth of distance takes place in the earliest period of life.

Morphological configuration of the cranial base among children aged 8 to 12 years.

PubMed

Cossio, Lina; López, Jorge; Rueda, Zulma Vanessa; Botero-Mariaca, Paola

2016-06-14

Cranial base is used as reference structure to determine the skeletal type in cephalometric analysis. The purpose was to assess the cranial base length on lateral cephalic radiographs of children between 8 and 12 and compare these measurements with baseline studies in order to evaluate the relationship between the length and the cranial base angle, articular angle, gonial angle and skeletal type. A Cross-sectional study in 149 children aged 8-12 years, originally from Aburrá Valley, who had lateral cephalic radiographs and consented to participate in this study. The variables studied included: age, sex, sella-nasion, sella-nasion-articular, sella-nasion-basion, articular-gonion-menton, gonion-menton, sella-nasion-point B, sella-nasion-point A y point A-nasion-point B. These variables were digitally measured through i-dixel 2 digital software. One-way ANOVA was used to determine mean values and mean value differences. The values obtained were compared with previous studies. A p value <0.05 was considered significant. Cranial base lengths are smaller in each age and sex group, with differences exceeding 10 mm for measurement, compared both with the study by Riolo (Michigan) and the study carried out in Damasco (Antioquia). No relation was found between the skeletal type and the anterior cranial base length, the sella angle and the cranial base angle. Also, no relation was found between the gonial angle and sella angle or the cranial base angle. The cranial base varies from one population to another. Accordingly, compared to other studies it is shorter for the assessed sample.

The Extended Nasoseptal Flap for Skull Base Reconstruction of the Clival Region: An Anatomical and Radiological Study

PubMed Central

Peris-Celda, Maria; Pinheiro-Neto, Carlos Diogenes; Funaki, Takeshi; Fernandez-Miranda, Juan C.; Gardner, Paul; Snyderman, Carl; Rhoton, Albert L.

2013-01-01

Objective Reconstruction of large clival defects after an endoscopic endonasal procedure is challenging. The objective is to analyze the morphology, indications, and limitations of the extended nasoseptal flap, which adds the nasal floor and inferior meatus mucosa, compared with the standard nasoseptal flap, for clival reconstruction. Design Twenty-seven sides of formalin-fixed anatomical specimens and 13 computed tomography (CT) scans were used. Under 0-degree endoscopic visualization, a standard flap on one side and an extended flap on the other side were performed, as well as exposure of the sella, cavernous sinus, and clival dura mater. Coverage of both flaps was assessed, and they were incised and extracted for measurements. Results The extended flap has two parts: septal and inferior meatal. The extended flaps are 20 mm longer and add 774 mm2 of mucosal area. They cover a clival defect from tuberculum to foramen magnum in 66.6% cases and from below the sella in 91.6%. They cover both parasellar and paraclival segments of the internal carotid arteries. The lateral inferior limits are the medial aspect of the hypoglossal canals and Eustachian tubes. CT scans can predict the need or limitation of an extended nasoseptal flap. Conclusions The nasal floor and inferior meatus mucosa adds a significant area for reconstruction of the clivus. A defect laterally beyond the hypoglossal canals is not likely covered with this variation of the flap. Preoperative CT scans are useful to guide the reconstruction techniques. PMID:24436940

Empty sella syndrome

MedlinePlus

Kaiser U, Ho KKY. Pituitary physiology and diagnostic evaluation. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 13th ed. Philadelphia, PA: Elsevier; ...

Empty Sella Syndrome

MedlinePlus

... hormone prolactin, which can interfere with the normal function of the testicles and ovaries. Primary ESS is most common in adults and women, ... hormone prolactin, which can interfere with the normal function of the testicles and ovaries. Primary ESS is most common in adults and women, ...

Thin-plate spline analysis of the cranial base in subjects with Class III malocclusion.

PubMed

Singh, G D; McNamara, J A; Lozanoff, S

1997-08-01

The role of the cranial base in the emergence of Class III malocclusion is not fully understood. This study determines deformations that contribute to a Class III cranial base morphology, employing thin-plate spline analysis on lateral cephalographs. A total of 73 children of European-American descent aged between 5 and 11 years of age with Class III malocclusion were compared with an equivalent group of subjects with a normal, untreated, Class I molar occlusion. The cephalographs were traced, checked and subdivided into seven age- and sex-matched groups. Thirteen points on the cranial base were identified and digitized. The datasets were scaled to an equivalent size, and statistical analysis indicated significant differences between average Class I and Class III cranial base morphologies for each group. Thin-plate spline analysis indicated that both affine (uniform) and non-affine transformations contribute toward the total spline for each average cranial base morphology at each age group analysed. For non-affine transformations, Partial warps 10, 8 and 7 had high magnitudes, indicating large-scale deformations affecting Bolton point, basion, pterygo-maxillare, Ricketts' point and articulare. In contrast, high eigenvalues associated with Partial warps 1-3, indicating localized shape changes, were found at tuberculum sellae, sella, and the frontonasomaxillary suture. It is concluded that large spatial-scale deformations affect the occipital complex of the cranial base and sphenoidal region, in combination with localized distortions at the frontonasal suture. These deformations may contribute to reduced orthocephalization or deficient flattening of the cranial base antero-posteriorly that, in turn, leads to the formation of a Class III malocclusion.

Facies interfingering and synsedimentary tectonics on late Ladinian-early Carnian carbonate platforms (Dolomites, Italy)

NASA Astrophysics Data System (ADS)

Keim, Lorenz; Brandner, Rainer

2001-11-01

A stratigraphic model for carbonate platform evolution in the Dolomites during the late Ladinian-early Carnian is presented. New light on pre-Raibl growth of individual carbonate platforms of the western Dolomites was shed by biostratigraphic data combined with a revised lithostratigraphy. At the Schlern, Langkofel and Sella, the carbonate factory (Upper Schlern Dolomite) remained productive into the lowermost Carnian (Cordevolian = Aon Zone), and caused a levelling-out of the former steep platform-to-basin relief. In the eastern Dolomites, platforms were producing till basal Julian 2 (Austriacum Zone). At the Sella and Langkofel, the sedimentation pattern after deposition of the Upper Schlern Dolomite was strongly influenced by synsedimentary tectonics. A first phase of extensional tectonics led to local fissures, block-tilting, graben structures and breccia deposits. Composition and fabric of the reworked clasts argue for local-source sediments and short transport distances. The extensional structures are sealed by sediments of Lower Carnian age. Two facies belts (Schlernplateau beds and Dürrenstein Dolomite), which interfinger at the western side of the Sella, reflect the shallow marine environment with terrigenous-volcanoclastic input in the western Dolomites. A second generation of breccias at the Sella documents local fracturing of the Dürrenstein and Upper Schlern Dolomite. Depositional environments across the western and eastern Dolomites were largely dependent on differential subsidence. The sediments of early Carnian age on top of the Schlern platform are a few metres thick only, whereas, in the eastern Dolomite, up to 400-m-thick carbonate sediments ('Richthofen reef' and Settsass platform) were deposited. The most incomplete stratigraphic record is present at the Mendel platform in the west, where Ladinian volcanics are unconformably overlain by late Carnian 'Raibl beds'. The increase in sediment thickness towards the eastern Dolomites becomes partly

Long-term maxillomandibular changes after maxillary distraction osteogenesis in growing children with cleft lip with or without palate.

PubMed

Honda, Aya; Baba, Yoshiyuki; Ogawa, Takuya; Suzuki, Shoichi; Moriyama, Keiji

2013-03-01

Objective : To evaluate the long-term maxillomandibular changes after maxillary distraction osteogenesis in growing children having cleft lip with or without cleft palate. Patients : Eight Japanese patients with cleft lip with or without cleft palate aged 9.3 to 13.1 years. Measures : The maxillary and mandibular positions before (T0), immediately after (T1), and 1, 3, and 5 years after distraction osteogenesis (T2, T3, and T4, respectively) measured on cephalograms superimposed at the sella turcica with the Frankfort horizontal plane as the horizontal reference. The anterior nasal spine (x, y), pogonion (x), and menton (y) were used for linear measurements, and sella turcica-nasion-point A, sella turcica-nasion-point B, and point A-nasion-point B angles were used for angular measurements. Results : The mean horizontal maxillary advancement (anterior nasal spine [x]) was 12.3 mm during T0 to T1, but -2.7, -1.1, and -0.1 mm of the posttreatment changes were observed during T1 to T2, T2 to T3, and T3 to T4, respectively. Anterior nasal spine (y) shifted 2.3 mm downward during T0 to T1, and further downward changes were observed during T1 to T2 and T2 to T3 (P < .05). Pogonion (x) did not show distinct changes due to individual variance, but menton (y) shifted downward from T1 to T4. Sella turcica-nasion-point A significantly decreased during T1 to T2 and T2 to T3 but not during T3-T4. Point A-nasion-point B significantly decreased only during T2 to T3, and sella turcica-nasion-point B did not show any distinct change. Conclusions : There was no further maxillary advancement after distraction osteogenesis in the growing children with cleft lip with or without cleft palate. Therefore, long-term observation and management of occlusion in case of the mandibular growth pattern are important.

Intracranial metastatic disease rarely involves the pituitary: retrospective analysis of 935 metastases in 155 patients and review of the literature.

PubMed

Marsh, James C; Garg, Shalini; Wendt, Julie A; Gielda, Benjamin T; Turian, Julius V; Herskovic, Arnold M

2010-09-01

We present a case report of a patient recently treated at our institution for an isolated non-small cell lung cancer metastatic lesion to the sella, report the lack of involvement of the pituitary gland in a large single-institution series of treated intracranial parenchymal metastases, and review the pertinent literature. We reviewed cranial imaging studies (CT and MRI) for 935 metastases in 155 patients treated at our institution over the previous 3 years for intracranial metastatic disease. Special attention was paid to the skull base to document the presence of any metastatic disease involving the pituitary gland, infundibular stalk, sella turcica (including anterior and posterior clinoids), or diaphragm sellae. We found no other involvement of the pituitary gland or other sellar structures by metastatic disease in this series. Intracranial metastatic disease rarely involves the pituitary gland and infundibular stalk parenchyma, suggesting that this structure may be safely omitted from the treatment field during WBRT and prophylactic cranial irradiation (PCI). This treatment approach should reduce the late sequelae of treatment to this critical organ.

Maxillary Distraction Osteogenesis Using a Rigid External Distractor: Which Clinical Factors Are Related With Relapse?

PubMed

Kim, Jeenam; Uhm, Ki-Il; Shin, Donghyeok; Lee, Jina; Choi, Hyungon

2015-06-01

Maxillary distraction osteogenesis is a reliable treatment for cleft lip and palate with midfacial retrusion. The purpose of this study was to evaluate the results of long-term follow-up in patients with cleft lip and palate after maxillary distraction osteogenesis and to find clinical factors related to relapse. From February 2002 to June 2008, 21 patients with severe class III malocclusion were treated at our hospital. We performed distraction osteotomy with a rigid external distractor device. The distraction length was more than 15 mm in all patients. Preoperative and postoperative lateral cephalometric radiographs were used for analysis. The sella-nasion-subnasale, sella-nasion-supramentale, and point-A-point-B-nasion (sella-nasion-subnasale-sella-nasion-supramentale) angles were recorded. The timelines for follow-up were preoperatively, after distraction, after consolidation, at 3 years, and once fully grown (5- to 8-year follow-ups). A comparative analysis of clinical factors was performed for the relapsing and nonrelapsing groups. Of the 21 patients, 14 had relapsed. The mean age in the relapsing group was 9.1 years (7 boys and 7 girls) with 9 patients with unilateral cleft palate and 5 c bilateral cleft palate. The mean age in the nonrelapsing group was 11.7 years (4 boys and 3 girls) with 5 patients with unilateral cleft palate and 2 patients with bilateral cleft palate. Despite greater anterior overcorrection, relapse occurred owing to scar tissue retraction and mandibular compensatory hypertrophy. The results suggest that the younger the patient, the more likely relapse will occur.

Skull Base Meningiomas and Cranial Nerves Contrast Using Sodium Fluorescein: A New Application of an Old Tool

PubMed Central

da Silva, Carlos Eduardo; da Silva, Vinicius Duval; da Silva, Jefferson Luis Braga

2014-01-01

Objective The identification of cranial nerves is one of the most challenging goals in the dissection of skull base meningiomas. The authors present an application of sodium fluorescein (SF) in skull base meningiomas with the purpose of improving the identification of cranial nerves. Design A prospective study within-subjects design. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with skull base meningiomas. Main Outcomes Measures Cranial nerve identification. Results The group of nine meningiomas was composed of one cavernous sinus, three petroclival, one tuberculum sellae, two sphenoid wing, one olfactory groove, and one temporal floor meningioma. The SF enhancement in all tumors was strong, and the contrast with cranial nerves clearly evident. There were one definite olfactory nerve deficit, one transient abducens deficit, and one definite hemiparesis. All lesions were resected (Simpson grades 1 and 2). The analysis of the difference of the delta SF wavelength between the meningiomas and cranial nerve contrast was performed by the Wilcoxon signed rank test and showed p = 0.011. Conclusions The contrast between the enhanced meningiomas and cranial nerves was evident and assisted in the visualization and microsurgical dissection of these structures. The anatomical preservation of these structures was improved using the contrast. PMID:27054056

Skull Base Meningiomas and Cranial Nerves Contrast Using Sodium Fluorescein: A New Application of an Old Tool.

PubMed

da Silva, Carlos Eduardo; da Silva, Vinicius Duval; da Silva, Jefferson Luis Braga

2014-08-01

Objective The identification of cranial nerves is one of the most challenging goals in the dissection of skull base meningiomas. The authors present an application of sodium fluorescein (SF) in skull base meningiomas with the purpose of improving the identification of cranial nerves. Design A prospective study within-subjects design. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with skull base meningiomas. Main Outcomes Measures Cranial nerve identification. Results The group of nine meningiomas was composed of one cavernous sinus, three petroclival, one tuberculum sellae, two sphenoid wing, one olfactory groove, and one temporal floor meningioma. The SF enhancement in all tumors was strong, and the contrast with cranial nerves clearly evident. There were one definite olfactory nerve deficit, one transient abducens deficit, and one definite hemiparesis. All lesions were resected (Simpson grades 1 and 2). The analysis of the difference of the delta SF wavelength between the meningiomas and cranial nerve contrast was performed by the Wilcoxon signed rank test and showed p = 0.011. Conclusions The contrast between the enhanced meningiomas and cranial nerves was evident and assisted in the visualization and microsurgical dissection of these structures. The anatomical preservation of these structures was improved using the contrast.

Endoscopic endonasal skull base surgery: advantages, limitations, and our techniques to overcome cerebrospinal fluid leakage: technical note.

PubMed

Ishii, Yudo; Tahara, Shigeyuki; Teramoto, Akira; Morita, Akio

2014-01-01

In recent years, resections of midline skull base tumors have been conducted using endoscopic endonasal skull base (EESB) approaches. Nevertheless, many surgeons reported that cerebrospinal fluid (CSF) leakage is still a major complication of these approaches. Here, we report the results of our 42 EESB surgeries and discuss the advantages and limits of this approach for resecting various types of tumors, and also report our technique to overcome CSF leakage. All 42 cases involved midline skull base tumors resected using the EESB technique. Dural incisions were closed using nasoseptal flaps and fascia patch inlay sutures. Total removal of the tumor was accomplished in seven pituitary adenomas (33.3%), five craniopharyngiomas (62.5%), five tuberculum sellae meningiomas (83.3%), three clival chordomas (100%), and one suprasellar ependymoma. Residual regions included the cavernous sinus, the outside of the intracranial part of the internal carotid artery, the lower lateral part of the posterior clivus, and the posterior pituitary stalk. Overall incidence of CSF leakage was 7.1%. Even though the versatility of the approach is limited, EESB surgery has many advantages compared to the transcranial approach for managing mid-line skull base lesions. To avoid CSF leakage, surgeons should have skills and techniques for complete closure, including use of the nasoseptal flap and fascia patch inlay techniques.

Feasibility of Piezoelectric Endoscopic Transsphenoidal Craniotomy: A Cadaveric Study

PubMed Central

Tomazic, Peter Valentin; Gellner, Verena; Koele, Wolfgang; Hammer, Georg Philipp; Braun, Eva Maria; Gerstenberger, Claus; Clarici, Georg; Holl, Etienne; Braun, Hannes; Stammberger, Heinz; Mokry, Michael

2014-01-01

Objective. Endoscopic transsphenoidal approach has become the gold standard for surgical treatment of treating pituitary adenomas or other lesions in that area. Opening of bony skull base has been performed with burrs, chisels, and hammers or standard instruments like punches and circular top knives. The creation of primary bone flaps—as in external craniotomies—is difficult.The piezoelectric osteotomes used in the present study allows creating a bone flap for endoscopic transnasal approaches in certain areas. The aim of this study was to prove the feasibility of piezoelectric endoscopic transnasal craniotomies. Study Design. Cadaveric study. Methods. On cadaveric specimens (N = 5), a piezoelectric system with specially designed hardware for endonasal application was applied and endoscopic transsphenoidal craniotomies at the sellar floor, tuberculum sellae, and planum sphenoidale were performed up to a size of 3–5 cm2. Results. Bone flaps could be created without fracturing with the piezoosteotome and could be reimplanted. Endoscopic handling was unproblematic and time required was not exceeding standard procedures. Conclusion. In a cadaveric model, the piezoelectric endoscopic transsphenoidal craniotomy (PETC) is technically feasible. This technique allows the surgeon to create a bone flap in endoscopic transnasal approaches similar to existing standard transcranial craniotomies. Future trials will focus on skull base reconstruction using this bone flap. PMID:24689037

Persisting Embryonal Infundibular Recess (PEIR): two case reports and systematic literature review.

PubMed

Belotti, Francesco; Lupi, Isabella; Cosottini, Mirco; Ambrosi, Claudia; Gasparotti, Roberto; Bogazzi, Fausto; Fontanella, Marco M; Doglietto, Francesco

2018-05-16

The persisting embryonal infundibular recess (PEIR) is a rare anomaly of the floor of the third ventricle with a debated pathogenesis. It can be a cause of misdiagnosis in case of cystic lesions of the sellar and suprasellar area. To describe two recently evaluated cases and provide a systematic literature review. PEIR has been previously reported in 6 adult patients. As in some cases it was associated to hydrocephalus and/or empty sella, a possible role of altered intracranial pressure in PEIR formation has been postulated. We evaluated two female patients, aged 34 and 50, referred to the Pituitary Surgery Clinic of the University of Brescia with the diagnosis of a sellar cyst and craniopharyngioma, respectively. Endocrine screening and visual field testing were normal. No signs of hydrocephalus or empty sella, as well as other indirect signs of intracranial hypertension, were visible on MRI scans. After a multidisciplinary re-evaluation, diagnosis of PEIR was made in both cases. Both patients are followed but have not developed any disturbance related to the PEIR in the following 18 months. PEIR is a rare condition, probably unrecognized and the result of dysembriogenesis, which should be included in the differential diagnosis of cystic sellar lesions. Imaging features (funnel pituitary stalk and cyst in the sella) appear pathognomonic. A normal endocrine evaluation might help in the diagnosis and warrants conservative treatment.

Effectiveness of the cervical vertebral maturation method to predict postpeak circumpubertal growth of craniofacial structures.

PubMed

Fudalej, Piotr; Bollen, Anne-Marie

2010-01-01

Our aim was to assess effectiveness of the cervical vertebral maturation (CVM) method to predict circumpubertal craniofacial growth in the postpeak period. The CVM stage was determined in 176 subjects (51 adolescent boys and 125 adolescent girls) on cephalograms taken at the end of treatment (T2; mean ages, 15.75 years [boys] and 15.23 years [girls]) in subjects from the postretention database at the University of Washington in Seattle. Craniofacial growth was evaluated from the following measurements on cephalograms at T2 and end of follow-up (T3) (mean ages, 29.01 years [men] and 28.08 years [women]): condylion to gnathion, condylion to gonion, gonion to gnathion, sella to gnathion, nasion to menton, anterior nasal spine to menton, and sella to gonion. The change of each variable from T2 to T3 was assessed with paired t tests. Parametric (t tests or analysis of variance [ANOVA]) or nonparametric (Mann-Whitney or Kruskal-Wallis) tests were used to detect intergroup differences. One hundred eight subjects (35 boys, 73 girls) demonstrated CVM stage 3, 56 (16 boys, 40 girls) were in CVM stage 4, and 12 (all girls) were in CVM stage 5 at T2. Intrasex comparisons showed that boys in CVM stages 3 and 4 could be differentiated regarding changes of all variables. In the girls, only those in CVM stages 3 and 4 could be differentiated based on the amount of changes of 2 measurements: condylion to gonion and sella to gonion. Intersex comparisons showed that boys in CVM stage 3 had significantly more changes than girls (P <0.01). Boys in CVM stage 4 showed significant differences compared with girls in CVM stage 4 for only 2 variables (sella to gonion and condylion to gonion; P <0.001 and P = 0.012, respectively). The CVM method was modestly effective in determining the amount of postpeak circumpubertal craniofacial growth. Copyright 2010 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.

Olfactory groove meningiomas.

PubMed

Hentschel, Stephen J; DeMonte, Franco

2003-06-15

Olfactory groove meningiomas (OGMs) arise over the cribriform plate and may reach very large sizes prior to presentation. They can be differentiated from tuberculum sellae meningiomas because OGMs arise more anterior in the skull base and displace the optic nerve and chiasm inferiorly rather than superiorly. The authors searched the neurosurgery database at the M. D. Anderson Cancer Center for cases of OGM treated between 1993 and 2003. The records of these patients were then reviewed retrospectively for details regarding clinical presentation, imaging findings, surgical results and complications, and follow-up status. Thirteen patients, (12 women and one man, mean age 56 years) harbored OGMs (mean size 5.7 cm). All patients underwent bifrontal craniotomies and biorbital osteotomies. There were 11 complete resections (including the hyperostotic bone and dura of the cribriform plate and any extension into the ethmoid sinuses) and two subtotal resections with minimal residual tumor left in patients with recurrent lesions. No complication directly due to the surgery occurred in any patient. There were no recurrences in a mean follow-up period of 2 years (range 0-5 years). With current microsurgical techniques, the results of OGM resection are excellent, with a high rate of total resection and a low incidence of complications. All hyperostotic bone should be removed with the dura of the anterior skull base to minimize the risk of recurrence.

Endoscopic Endonasal Skull Base Surgery: Advantages, Limitations, and Our Techniques to Overcome Cerebrospinal Fluid Leakage: Technical Note

PubMed Central

ISHII, Yudo; TAHARA, Shigeyuki; TERAMOTO, Akira; MORITA, Akio

2014-01-01

In recent years, resections of midline skull base tumors have been conducted using endoscopic endonasal skull base (EESB) approaches. Nevertheless, many surgeons reported that cerebrospinal fluid (CSF) leakage is still a major complication of these approaches. Here, we report the results of our 42 EESB surgeries and discuss the advantages and limits of this approach for resecting various types of tumors, and also report our technique to overcome CSF leakage. All 42 cases involved midline skull base tumors resected using the EESB technique. Dural incisions were closed using nasoseptal flaps and fascia patch inlay sutures. Total removal of the tumor was accomplished in seven pituitary adenomas (33.3%), five craniopharyngiomas (62.5%), five tuberculum sellae meningiomas (83.3%), three clival chordomas (100%), and one suprasellar ependymoma. Residual regions included the cavernous sinus, the outside of the intracranial part of the internal carotid artery, the lower lateral part of the posterior clivus, and the posterior pituitary stalk. Overall incidence of CSF leakage was 7.1%. Even though the versatility of the approach is limited, EESB surgery has many advantages compared to the transcranial approach for managing mid-line skull base lesions. To avoid CSF leakage, surgeons should have skills and techniques for complete closure, including use of the nasoseptal flap and fascia patch inlay techniques. PMID:25446379

Distraction rate and latency: factors in the outcome of paediatric maxillary distraction.

PubMed

Higuera, Stephen; Cole, Patrick; Stephenson, J B; Hollier, Larry

2009-12-01

Over 50 years ago, current tenets of distraction osteogenesis were developed through work on the lower extremity; however, the application of these tenets in the paediatric craniofacial skeleton remains questionable. Prompted by recent concern that traditional aspects of distraction may be either outdated or wholly inapplicable to the paediatric maxilla, we retrospectively evaluated maxillary distraction protocol using a 24-h latency period in conjunction with a distraction rate of 2mm/day. Following maxillary advancement via a distraction protocol consisting of a 24-h latency period and a distraction rate of 2mm/day, seven consecutive paediatric cases were evaluated. Standard profile photos and cephalometric films taken preoperatively, at device removal and at 1-year follow-up were compared. With the sella as the point of registration, pre- and post-distraction films were superimposed on the sella-nasion plane. Sella-nasion-subspinale, the angle of convexity, the distance from incisal edges to the y-axis, and angulation of the upper incisor to the sella-nasion plane were analysed to evaluate hard-tissue changes. Patient age ranged from 3 to 14 years (mean=7.43 years). Maxillary distraction length averaged 11 mm (range=10-12 mm). Interval from device application to removal averaged 98 days (range=75-180 days). The interval of the active distraction ranged from 11 to 65 days (mean=24 days). From distraction completion to device removal averaged 85 days (range=60-150). Follow-up intervals ranged from 52 to 24 months (mean=34 months). All patients demonstrated substantial clinical advancement of the maxilla with correction of midfacial deficiencies. A single patient developed mild cellulitis at one skin-device interface; no other complications were noted. Cephalometric and clinical evaluations at 1 year post-distraction demonstrated stable results, and parental satisfaction was qualitatively high. The surgical dogma of lower-extremity distraction osteogenesis is not

Variability of the inclination of anatomic horizontal reference planes of the craniofacial complex in relation to the true horizontal line in orthognathic patients.

PubMed

Zebeib, Ameen M; Naini, Farhad B

2014-12-01

The purpose of this study was to assess the reliability of the Frankfort horizontal (FH), sella-nasion horizontal, and optic planes in terms of their variabilities in relation to a true horizontal line in orthognathic surgery patients. Thirty-six consecutive presurgical orthognathic patients (13 male, 23 female; age range, 16-35 years; 30 white, 6 African Caribbean) had lateral cephalometric radiographs taken in natural head position, with a plumb line orientating the true vertical line, and the true horizontal line perpendicular to the true vertical. The inclinations of the anatomic reference planes were compared with the true horizontal. The FH plane was found to be on average closest to the true horizontal, with a mean of -1.6° (SD, 3.4°), whereas the sella-nasion horizontal and the optic plane had means of 2.1° (SD, 5.1°) and 3.2° (SD, 4.7°), respectively. The FH showed the least variability of the 3 anatomic planes. The ranges of variability were high for all anatomic planes: -8° to 8° for the FH, -8° to 15° for the sella-nasion horizontal, and -6° to 13° for the optic plane. No significant differences were found in relation to patients' sex, skeletal patterns, or ethnic backgrounds. The clinically significant variability in the inclinations of anatomic reference planes in relation to the true horizontal plane makes their use unreliable in orthognathic patients. Copyright © 2014 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

Variability of the cranial and dental phenotype in Williams syndrome.

PubMed

Axelsson, Stefan

2005-01-01

Williams syndrome (WS) is a rare congenital disorder involving the cardiovascular system, connective tissue, and the central nervous system, resulting in mild to moderate mental retardation, a specific cognitive profile, unique personality characteristics, distinctive facial features, and cardiovascular disease. The majority of individuals with a clinical diagnosis of WS have a contiguous gene deletion at chromosome 7 (7q11.23). Physical features include characteristic facial features with full prominent cheeks, wide mouth, long philtrum, small nose with depressed nasal bridge, heavy orbital ridges, medial eyebrow flare, dental abnormalities, hoarse voice, growth retardation, and cardiovascular abnormalities (most commonly supravalvular aortic stenosis and/or peripheral pulmonary artery stenosis). The cognitive profile is distinctive, consisting of strengths in auditory memory, language, and face-processing, but extreme weakness in visuospatial, numerical and problem-solving abilities. Neurological studies have identified a significantly decreased brain volume in adult individuals with WS with relatively normal development of the limbic, frontal and cerebellar structures. The aims were to analyse the neurocranium, the craniofacial region, and the dentition in a well defined Norwegian group of individuals with WS. In order to accomplish this, normative cephalometric standards for the neurocranium, including the cranial base and the sella turcica, were established for Norwegian males and females from 6 to 21 years of age, using lateral radiographic cephalograms from the Oslo University Craniofacial Growth Archive. The study material comprised radiographic lateral cephalograms, orthopantomograms and dental casts from 62 individuals with WS ranging from 4 to 44 years. The lateral cephalograms, orthopantomograms and dental casts were analysed using standard methods reported in the literature. Neurocranium: The results from the cephalometric analyses showed that the size

[Etiological diagnosis of central diabetes insipidus: about 41 cases].

PubMed

Chaker, Fatma; Chihaoui, Melika; Yazidi, Meriem; Slimane, Hedia

2016-01-01

The occurrence of polyuria-polydipsia syndrome with hypotonic urine requires careful diagnostic strategy. This study aims to evaluate diagnostic modalities for central diabetes insipidus. We conducted a retrospective study of 41 cases with central diabetes insipidus (CDI). Data were collected at the Department of Endocrinology, University Hospital La Rabta, Tunis, from 1990 to 2013. We identified the circumstances for detecting CDI, the abnormalities in anterior pituitary assessment and pituitary imaging. CDI occurred in the postoperative period in 20 patients. The average urine 24-hour volume was significantly higher in patients with CDI outside a surgical setting. Water deprivation test was successful in all patients who benefited from it. Outside of neurosurgery, infiltration causes were found in 6 patients and tumor causes were found in 6 patients. CDI was associated with empty sella turcica in 1 case and idiopathic sella turcica in 3 patients. Hypothalamic-pituitary magnetic resonance imaging and anterior pituitary balance sheet are systematic outside pituitary surgery setting and obvious primary polydipsia.

Preoperative Visualization of Cranial Nerves in Skull Base Tumor Surgery Using Diffusion Tensor Imaging Technology.

PubMed

Ma, Jun; Su, Shaobo; Yue, Shuyuan; Zhao, Yan; Li, Yonggang; Chen, Xiaochen; Ma, Hui

2016-01-01

To visualize cranial nerves (CNs) using diffusion tensor imaging (DTI) with special parameters. This study also involved the evaluation of preoperative estimates and intraoperative confirmation of the relationship between nerves and tumor by verifying the accuracy of visualization. 3T magnetic resonance imaging scans including 3D-FSPGR, FIESTA, and DTI were used to collect information from 18 patients with skull base tumor. DTI data were integrated into the 3D slicer for fiber tracking and overlapped anatomic images to determine course of nerves. 3D reconstruction of tumors was achieved to perform neighboring, encasing, and invading relationship between lesion and nerves. Optic pathway including the optic chiasm could be traced in cases of tuberculum sellae meningioma and hypophysoma (pituitary tumor). The oculomotor nerve, from the interpeduncular fossa out of the brain stem to supraorbital fissure, was clearly visible in parasellar meningioma cases. Meanwhile, cisternal parts of trigeminal nerve and abducens nerve, facial nerve were also imaged well in vestibular schwannomas and petroclival meningioma cases. The 3D-spatial relationship between CNs and skull base tumor estimated preoperatively by tumor modeling and tractography corresponded to the results determined during surgery. Supported by DTI and 3D slicer, preoperative 3D reconstruction of most CNs related to skull base tumor is feasible in pathological circumstances. We consider DTI Technology to be a useful tool for predicting the course and location of most CNs, and syntopy between them and skull base tumor.

Expanded Endoscopic Endonasal Resection of Retrochiasmatic Craniopharyngioma.

PubMed

Davanzo, Justin R; Goyal, Neerav; Zacharia, Brad E

2018-02-01

This video abstract demonstrates the use of the expanded endoscopic endonasal approach for the resection of a retrochiasmatic craniopharyngioma. These tumors are notoriously difficult to treat, and many approaches have been tried to facilitate safe and effective resection. The endoscopic endonasal approach has been increasingly utilized for selected sellar/suprasellar pathology. We present the case of a 39-year-old man who was found to have a cystic, partially calcified suprasellar mass consistent with a craniopharyngioma. To facilitate robust skull base repair, a vascularized nasoseptal flap was harvested. A wide sphenoidotomy was performed and the sella and tuberculum were exposed. After the dural opening and arachnoid dissection, the stalk was identified, merging seamlessly with the tumor capsule. The lesion was then internally debulked with the use of an ultrasonic aspirator. The capsule was then dissected off of the optic chiasm, thalamus, and hypothalamus. The cavity was inspected with an angled endoscope to ensure complete resection. A multilayered reconstruction was performed using autologous fascia lata, the previously harvested nasoseptal flap, and dural sealant. Postoperatively, the patient did have expected panhypopituitarism but remained neurologically intact and had improvement in his vision. In conclusion, this video demonstrates how an expanded endonasal approach can be used to safely resect a craniopharyngioma, even when in close proximity to delicate structures such as the optic chiasm. The link to the video can be found at: https://youtu.be/tahjHmrXhc4 .

Postoperative evaluation of palatoplasty using the modified Kaplan method.

PubMed

Isago, Tsukasa; Nozaki, Motohiro; Honda, Takashi; Kikuchi, Yuji

2005-01-01

Bilateral buccal mucosal flaps were used for a pushback operation of the nasal mucosa as well as the oral mucosa. Two flaps from the buccal mucosal areas were inserted into the nasal mucosal raw surface at the hard and soft palatal border to achieve nasal mucosal pushback. Forty-four children with cleft palates were retrospectively studied to investigate the velopharyngeal function, articulation, and craniofacial morphology following primary palatoplasty using the modified Kaplan method. 72.7% showed good velopharyngeal function. Articulation disorders were found in 26 of the 44 subjects (59.1%). A craniofacial morphological analysis revealed no major changes in the SNA (sella-nasion point A angle) and SNB (sella-nasion point B angle) and no cases of a marked loss of maxillary mandibular balance. In addition, no significant differences were observed in the maxillary length and width. The results of these palatoplasties by the modified Kaplan method indicated that the use of bilateral buccal mucosal flaps for the pushback of the nasal mucosa was effective in preventing the contraction of the maxillary and mandibular corpal lengths.

[Unconsciousness due to hyponatremia in a patient with short stature with panhypopituitarism].

PubMed

Notsu, K; Takagi, C; Umaki, I

1995-03-20

An unconscious woman of short stature (141 cm) was admitted to our hospital in March, 1994. She had hyponatremia (120 mEq/l) and had experienced massive bleeding during delivery. No increment of either plasma ACTH or cortisol levels was observed after insulin-induced hypoglycemia. However, urinary 17OHCS levels gradually increased after repeated intramuscular injections of ACTH. Plasma free T3 and free T4 levels were low. Neither plasma TSH nor prolactin (PRL) levels increased after an intravenous injection of TRH. Basal plasma LH, FSH and growth hormone (GH) levels were low and there were no observable responses to any of the stimulation tests. A magnetic resonance image (MRI) of her pituitary gland showed an empty sella. These results showed that she had a panhypopituitarism with primary empty sella. Replacement therapy with glucocorticoid was started and serum sodium levels normalized immediately. Levothyroxine was also administered. The possibility of pituitary dwarfism during her youth and a gradual postpartum reduction of other pituitary hormones may have caused an impairment of the hypothalamo-pituitary-adrenal axis.

Frontobasal Midline Meningiomas: Is It Right To Shed Doubt on the Transcranial Approaches? Updates and Review of the Literature.

PubMed

Ruggeri, Andrea Gennaro; Cappelletti, Martina; Fazzolari, Benedetta; Marotta, Nicola; Delfini, Roberto

2016-04-01

Traditionally, the surgical removal of tuberculum sellae meningioma (TSM) and olfactory groove meningioma (OGM) requires transcranial approaches and microsurgical techniques, but in the last decade endoscopic expanded endonasal approaches have been introduced: transcribriform for OGMs and transtuberculum-transplanum for TSM. A comparative analysis of the literature concerning the two types of surgical treatment of OGMs and TSM is, however, difficult. We conducted a literature search using the PubMed database to compare data for endoscopic and microsurgical techniques in the literature. We also conducted a retrospective analysis of selected cases from our series presenting favorable characteristics for an endoscopic approach, based on the criteria of operability of these lesions as generally accepted in the literature, and we compared the results obtained in these patients with those in the endoscopic literature. We believe that making the sample more homogeneous, the difference between microsurgical technique and endoscopic technique is no longer so striking. A greater radical removal rate, a reduced incidence of cerebrospinal fluid fistula and, especially, the possibility of removing lesions of any size are advantages of transcranial surgery; a higher percentage of improvement in visual outcome and a lower risk of a worsening of a pre-existing deficit or onset of a new deficit are advantages of the endoscopic technique. At present, the microsurgical technique is still the gold standard for the removal of the anterior cranial fossa meningiomas of all sizes, and the endoscopic technique remains a second option in certain cases. Copyright © 2016 Elsevier Inc. All rights reserved.

A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors

PubMed Central

Graffeo, Christopher S.; Dietrich, August R.; Grobelny, Bartosz; Zhang, Meng; Goldberg, Judith D.; Golfinos, John G.; Lebowitz, Richard; Kleinberg, David; Placantonakis, Dimitris G.

2014-01-01

Endoscopic endonasal surgery has been established as the safest approach to pituitary tumors, yet its role in other common skull base lesions has not been established. To answer this question, we carried out a systematic review of reported series of open and endoscopic endonasal approaches to four major skull base tumors: olfactory groove meningiomas (OGM), tuberculum sellae meningiomas (TSM), craniopharyngiomas (CRA), and clival chordomas (CHO). Data from 162 studies containing 5,701 patients were combined and compared for differences in perioperative mortality, gross total resection (GTR), cerebrospinal fluid (CSF) leak, neurological morbidity, post-operative visual function, post-operative anosmia, post-operative diabetes insipidus (DI), and post-operative obesity/hyperphagia. Weighted average rates for each outcome were calculated using relative study size. Our findings indicate similar rates of GTR and perioperative mortality between open and endoscopic approaches for all tumor types. CSF leak was increased after endoscopic surgery. Visual function symptoms were more likely to improve after endoscopic surgery for TSM, CRA, and CHO. Post-operative DI and obesity/hyperphagia were significantly increased after open resection in CRA. Recurrence rates per 1,000 patient-years of follow-up were higher in endoscopy for OGM, TSM, and CHO. Trends for open and endoscopic surgery suggested modest improvement in all outcomes over time. Our observations suggest that endonasal endoscopy is a safe alternative to craniotomy and may be preferred for certain tumor types. However, endoscopic surgery is associated with higher rates of CSF leak, and possibly increased recurrence rates. Prospective study with long-term follow-up is required to verify these preliminary observations. PMID:24014055

A panoramic view of the skull base: systematic review of open and endoscopic endonasal approaches to four tumors.

PubMed

Graffeo, Christopher S; Dietrich, August R; Grobelny, Bartosz; Zhang, Meng; Goldberg, Judith D; Golfinos, John G; Lebowitz, Richard; Kleinberg, David; Placantonakis, Dimitris G

2014-08-01

Endoscopic endonasal surgery has been established as the safest approach to pituitary tumors, yet its role in other common skull base lesions has not been established. To answer this question, we carried out a systematic review of reported series of open and endoscopic endonasal approaches to four major skull base tumors: olfactory groove meningiomas (OGM), tuberculum sellae meningiomas (TSM), craniopharyngiomas (CRA), and clival chordomas (CHO). Data from 162 studies containing 5,701 patients were combined and compared for differences in perioperative mortality, gross total resection (GTR), cerebrospinal fluid (CSF) leak, neurological morbidity, post-operative visual function, post-operative anosmia, post-operative diabetes insipidus (DI), and post-operative obesity/hyperphagia. Weighted average rates for each outcome were calculated using relative study size. Our findings indicate similar rates of GTR and perioperative mortality between open and endoscopic approaches for all tumor types. CSF leak was increased after endoscopic surgery. Visual function symptoms were more likely to improve after endoscopic surgery for TSM, CRA, and CHO. Post-operative DI and obesity/hyperphagia were significantly increased after open resection in CRA. Recurrence rates per 1,000 patient-years of follow-up were higher in endoscopy for OGM, TSM, and CHO. Trends for open and endoscopic surgery suggested modest improvement in all outcomes over time. Our observations suggest that endonasal endoscopy is a safe alternative to craniotomy and may be preferred for certain tumor types. However, endoscopic surgery is associated with higher rates of CSF leak, and possibly increased recurrence rates. Prospective study with long-term follow-up is required to verify these preliminary observations.

Superior interhemispheric approach for midline meningioma from the anterior cranial base.

PubMed

Lévêque, S; Derrey, S; Martinaud, O; Gérardin, E; Langlois, O; Fréger, P; Hannequin, D; Castel, H; Proust, F

2011-07-01

For suprasellar meningioma, the fronto-basal exposure is considered the standard approach. The superior interhemispheric (IH) approach is less described in the literature. To assess the surgical complications, functional outcome (visual, olfaction), morbidity and mortality rates and late recurrence, after resection by superior IH approach of midline skull base meningioma. Between 1998 and 2008, 52 consecutive patients with midline meningioma on the anterior portion of the skull base (mean age: 63.8 ± 13.1; sex ratio F/M: 3.7) were operated on via the superior IH approach. After a mean follow-up of 56.9 ± 32.9 months, an independent neurosurgeon proposed a prospective examination of functional outcome to each patient, as well as a visual and olfactory function assessment. Fifty-two patients were divided into a group with olfactory groove meningioma (n=34) and another with tuberculum sellae meningioma (n=18). The outcome was characterized by postoperative complications in 13 patients (25%), mortality rate in two (3.8%) and long-term morbidity at in 17 (37%) of 50 surviving patients. Based on multivariate analysis, no prognosis factor was significant as regards the favorable outcome. The mean postoperative KPS score (86.6 ± 9.4) was significantly improved. However, dysexecutive syndrome was observed in four patients (8%), hyposmia-anosmia in 34 (68%) and visual acuity deteriorated in one (2%). The superior IH approach could be considered a safe anteriorly orientated midline approach for removal OGM and TSM meningioma. Copyright © 2011. Published by Elsevier Masson SAS.

Alteration of maxillary and mandibular growth of adult patients with unoperated isolated cleft palate.

PubMed

Yang, Yunqiang; Wu, Yeke; Gu, Yifei; Yang, Qijian; Shi, Bing; Zheng, Qian; Wang, Yan

2013-07-01

The objective of this study was to investigate the effects of cleft palate itself on the growth of maxilla and mandible. Fifty-two adult female patients with unoperated isolated cleft palate and 52 adult female individuals with normal occlusion were included in our study. Computer software was used for lateral cephalometry measurement. Manual measurement was performed for dental cast measurements, and sample t test analysis was applied to analyze the differences between the 2 groups using SPSS 17.0. The sella-nasion-subspinale point angle, subspinale-nasion-supramentale point angle, and maxillary arch length of the cleft group were significantly smaller than those of the control group (P < 0.01). Both maxillary and mandibular posterior dental arch widths of the cleft group were significantly larger compared with the control group (P < 0.01), whereas the sella-nasion-supramentale point angle, mandible arch length, palate height, and palate shelf inclination did not differ between the 2 groups. The measurements did not differ between the submucosal cleft and the overt cleft patients. Cleft palate itself has adverse effects on the maxilla growth with shorter maxillary arch length and wider posterior dental arch width.

Congenital hypopituitarism in a 48-year old adult. Natural course, hormonal study and MRI evidence.

PubMed

Pentimone, F; Riccioni, S; Del Corso, L

1999-06-01

A case of Congenital Hypopituitarism (CH) in an untreated 48 yr-old-man is reported. The hormonal studies demonstrated a panhypopituitarism and MR imaging revealed absence of pituitary stalk, small anterior pituitary remnant on the sella floor and ectopic neurohypophysis at the tuber cinereum. The pattern of hormonal responsiveness suggests that CH encompasses findings typical of primary anterior pituitary disease and those of hypothalamic dysfunction.

[Diagnostic significance of T2W hypointensity of the sella].

PubMed

Rousset, P; Cattin, F; Chiras, J; Bonneville, J F; Bonneville, F

2009-06-01

Normal anatomical structures and lesions characterized by low T2W signal intensity are reviewed in this pictorial essay. The purpose is to demonstrate how evaluation of the appearance, shape and exact anatomical location of the T2W hypointense sellar region structure, correlated with its T1W signal intensity, can based on the clinical context lead to an appropriate differential diagnosis.

The International Conference on Hemorrhagic Fever with Renal Syndrome (1st) Held in Seoul, Korea on 4-6 May 1989

DTIC Science & Technology

1990-05-01

suggest that the body is important for a maker of 1lFRS-virus infection. 69 PROSPECTIVE STUDY ON PANHYPOPITUITARISM AS A SEQUELA OF HEMORRHAGIC FEVER...necrosis is a characteristic and common autopsy finding. Contrary to this, only 14 cases of clinical panhypopituitarism have been reported. Recently we...confirmed six patients with panhypopituitarism by high resolution sella computed tomo-,raphy(CT) and combined anterior pituitary stimulation lest(CAPS I

Spontaneous remission of acromegaly or gigantism due to subclinical apoplexy of pituitary growth hormone adenoma.

PubMed

Wang, Xian-Ling; Dou, Jing-Tao; Lü, Zhao-Hui; Zhong, Wen-Wen; Ba, Jian-Ming; Jin, Du; Lu, Ju-Ming; Pan, Chang-Yu; Mu, Yi-Ming

2011-11-01

Subclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion. The typical presence of pituitary growth hormone (GH) adenoma is gigantism and/or acromegaly. We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma. Six patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed. In these cases, the initial clinical presences were diabetes mellitus or hypogonadism. No abrupt headache, vomiting, visual function impairment, or conscious disturbance had ever been complained of. The base levels of GH and insulin growth factor-1 (IGF-1) were normal or higher, but nadir GH levels were all still > 1 µg/L in 75 g oral glucose tolerance test. Magnetic resonance imaging detected enlarged sella, partial empty sella and compressed pituitary. The transsphenoidal surgery was performed in 2 cases, and the other patients were conservatively managed. All the patients were in clinical remission. When the clinical presences, endocrine evaluation, biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly, the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed. To these patients, conservative therapy may be appropriate.

Modular Classification of Endoscopic Endonasal Transsphenoidal Approaches to Sellar Region: Anatomic Quantitative Study.

PubMed

Belotti, Francesco; Doglietto, Francesco; Schreiber, Alberto; Ravanelli, Marco; Ferrari, Marco; Lancini, Davide; Rampinelli, Vittorio; Hirtler, Lena; Buffoli, Barbara; Bolzoni Villaret, Andrea; Maroldi, Roberto; Rodella, Luigi Fabrizio; Nicolai, Piero; Fontanella, Marco Maria

2018-01-01

Endoscopic visualization does not necessarily correspond to an adequate working space. The need for balancing invasiveness and adequacy of sellar tumor exposure has recently led to the description of multiple endoscopic endonasal transsphenoidal approaches. Comparative anatomic data on these variants are lacking. We sought to quantitatively compare endoscopic endonasal transsphenoidal approaches to the sella and parasellar region, using the concept of "surgical pyramid." Four endoscopic transsphenoidal approaches were performed in 10 injected specimens: 1) hemisphenoidotomy; 2) transrostral; 3) extended transrostral (with superior turbinectomy); and 4) extended transrostral with posterior ethmoidectomy. ApproachViewer software (part of GTx-Eyes II, University Health Network, Toronto, Canada) with a dedicated navigation system was used to quantify the surgical pyramid volume, as well as exposure of sellar and parasellar areas. Statistical analyses were performed with Friedman's tests and Nemenyi's procedure. Hemisphenoidotomy provided limited exposure of the sellar area and a small working volume. A transrostral approach was necessary to expose the entire sella. Exposure of lateral parasellar areas required superior turbinectomy or posterior ethmoidectomy. The differences between each of the modules was statistically significant. The present study validates, from an anatomic point of view, a modular classification of endoscopic endonasal transsphenoidal approaches to the sellar region. Copyright © 2017 Elsevier Inc. All rights reserved.

[Clinical feature of chronic compressive optic neuropathy without optic atrophy].

PubMed

Jiang, Libin; Shi, Jitong; Liu, Wendong; Kang, Jun; Wang, Ningli

2014-12-01

To investigate the clinical feature of the chronic compressive optic neuropathy without optic atrophy. Retrospective cases series study. The clinical data of 25 patients (37 eyes) with chronic compressive optic neuropathy without optic atrophy, treated in Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, from October, 2005 to March, 2014, were collected. Those patients had been showing visual symptoms for 6 months or longer, but missed diagnosed or misdiagnosed as other eye diseases due to their normal or slightly changed fundi. The collected data including visual acuities, visual fields, neuroimaging and/or pathologic diagnosis were analyzed. Among the 25 patients, there were 5 males and 20 females, and their ages range from 9 to 74 years [average (47.5 ± 13.4) years]. All patients suffered progressive impaired vision in single eye or both eyes, without exophthalmos or abnormal eye movements. Except one patient had a headache, other patients did not show systemic symptoms. The corrected visual acuities were between HM to 1.0, and their appearances of optic discs and colors of fundi were normal. After neuroimaging and/or pathological examination, it was proven that 14 patients suffered tuberculum sellae meningiomas, 5 patients with hypophysoma, 3 patient with optic nerve sheath meningioma in orbital apex, 1 patient with cavernous hemangioma, 1 patient with vascular malformation in orbital apex and 1 patient with optic nerve glioma. Among the 19 patients whose suffered occupied lesions of saddle area, 14 patients underwent visual field examinations, and only 4 patients showed classic visual field defects caused by optic chiasmal lesions. Occult progressive visual loss was the most important clinical feature of the disease.

Frontal lobe morphometry with MRI in a normal age group of 6-17 year-olds.

PubMed

Ilkay Koşar, M; Otağ, Ilhan; Sabancıoğulları, Vedat; Atalar, Mehmet; Tetiker, Hasan; Otağ, Aynur; Cimen, Mehmet

2012-12-01

Morphometric data of the frontal lobe are important for surgical planning of lesions in the frontal lobe and its surroundings. Magnetic resonance imaging (MRI) techniques provide suitable data for this purpose. In our study, the morphometric data of mid-sagittal MRI of the frontal lobe in certain age and gender groups of children have been presented. In a normal age group of 6-17-year-old participants, the length of the line passing through predetermined different points, including the frontal pole (FP), commissura anterior (AC), commissura posterior (PC), the outermost point of corpus callosum genu (AGCC), the innermost point of corpus callosum genu (IGCC), tuberculum sella (TS), AGCC and IGCC points parallel to AC-PC line and the point such line crosses at the frontal lobe surface (FCS) were measured in three age groups (6-9, 10-13 and 14-17 years) for each gender. The frontal lobe morphometric data were higher in males than females. Frontal lobe measurements peak at the age group of 10-13 in the male and at the age group of 6-13 in the female. In boys, the length of FP-AC increases 4.1% in the 10-13 age group compared with the 6-9-year-old group, while this increase is 2.3% in girls. Differences in age and gender groups were determined. While the length of AGCC-IGCC increases 10.4% in adults, in children aged 6-17, the length of AC-PC is 11.5% greater than adults. These data will contribute to the preliminary assessment for developing a surgical plan in fine interventions in the frontal lobe and its surroundings in children.

Condylar response to functional therapy with Twin-Block as shown by cone-beam computed tomography.

PubMed

Yildirim, Ersin; Karacay, Seniz; Erkan, Mustafa

2014-11-01

To evaluate the condylar changes through cone-beam computed tomography (CBCT) images in patients treated with Twin-Block functional appliance. In this retrospective study, CBCT images of 30 patients who were treated with the Twin-Block appliance were used. Mandible was segmented and pretreatment and posttreatment (T0 and T1) condylar volume was compared. The angle between sella-nasion-Point A (SNA), angle between sella-nasion-Point B (SNB), angle between Point A-nasion-Point B (ANB), midfacial length (Co-A), mandibular length (Co-Gn), and the distances from right condylion to left condylion (CoR-CoL) were also measured on three-dimensional images. Differences were analyzed with Wilcoxon signed rank tests, and Mann-Whitney U-tests were used to compare the scores of male and female participants. Significance was set at P < .05. In this study, a decrease in SNA and ANB (P < .05 and P < .01, respectively) and an increase in SNB (P < .01) were found. Additionally, CoR-CoL, Co-Gn, and condylar volume increased at both the left and right sides (P < .01). However, increase at Co-A was not statistically significant (P > .05). Comparison of differences by sex was not statistically significant for all measurements (P > .05). Twin-Block appliance increases condylar volume, mandibular length, and intercondylar distance by stimulating growth of condyle in an upward and backward direction.

Manual tracing versus smartphone application (app) tracing: a comparative study.

PubMed

Sayar, Gülşilay; Kilinc, Delal Dara

2017-11-01

This study aimed to compare the results of conventional manual cephalometric tracing with those acquired with smartphone application cephalometric tracing. The cephalometric radiographs of 55 patients (25 females and 30 males) were traced via the manual and app methods and were subsequently examined with Steiner's analysis. Five skeletal measurements, five dental measurements and two soft tissue measurements were managed based on 21 landmarks. The durations of the performances of the two methods were also compared. SNA (Sella, Nasion, A point angle) and SNB (Sella, Nasion, B point angle) values for the manual method were statistically lower (p < .001) than those for the app method. The ANB value for the manual method was statistically lower than that of app method. L1-NB (°) and upper lip protrusion values for the manual method were statistically higher than those for the app method. Go-GN/SN, U1-NA (°) and U1-NA (mm) values for manual method were statistically lower than those for the app method. No differences between the two methods were found in the L1-NB (mm), occlusal plane to SN, interincisal angle or lower lip protrusion values. Although statistically significant differences were found between the two methods, the cephalometric tracing proceeded faster with the app method than with the manual method.

Preliminary comparison of the endoscopic transnasal vs the sublabial transseptal approach for clinically nonfunctioning pituitary macroadenomas.

PubMed

Sheehan, M T; Atkinson, J L; Kasperbauer, J L; Erickson, B J; Nippoldt, T B

1999-07-01

To assess the advantages and disadvantages of an endoscopic transnasal approach to pituitary surgery for a select group of clinically nonfunctioning macroadenomas and to compare results of this approach with the sublabial transseptal approach at a single institution. We retrospectively reviewed the records of 26 patients with clinically nonfunctioning pituitary macroadenomas approached endoscopically and 44 matched control patients with the same tumors approached sublabially between January 1, 1995, and October 31, 1997. At baseline, the groups were not significantly different for age, sex distribution, number of comorbid conditions, visual field defects, degree of anterior pituitary insufficiency, or preoperative assessment of tumor volume or invasiveness. Mean (SD) operative times were significantly reduced in the endoscopic group vs the sublabial group: 2.7 (0.7) hours vs 3.4 (0.9) hours (P < .001). Postoperative assessment of surgical resection and postoperative alterations of anterior pituitary function or visual fields were not significantly different between groups, and complication rates were similar in both groups. This endoscopic transnasal approach to pituitary resection results in significantly shorter operative time without compromising the extent of tumor resection. The distinct disadvantage of this approach is an off-center view of the sella and a diminished working channel to the sella turcica. For these reasons, the endoscopic approach or its variation is an alternative to the sublabial approach but should be considered only by experienced pituitary neurosurgeons.

Identification of 'Point A' as the prevalent source of error in cephalometric analysis of lateral radiographs.

PubMed

Grogger, P; Sacher, C; Weber, S; Millesi, G; Seemann, R

2018-04-10

Deviations in measuring dentofacial components in a lateral X-ray represent a major hurdle in the subsequent treatment of dysgnathic patients. In a retrospective study, we investigated the most prevalent source of error in the following commonly used cephalometric measurements: the angles Sella-Nasion-Point A (SNA), Sella-Nasion-Point B (SNB) and Point A-Nasion-Point B (ANB); the Wits appraisal; the anteroposterior dysplasia indicator (APDI); and the overbite depth indicator (ODI). Preoperative lateral radiographic images of patients with dentofacial deformities were collected and the landmarks digitally traced by three independent raters. Cephalometric analysis was automatically performed based on 1116 tracings. Error analysis identified the x-coordinate of Point A as the prevalent source of error in all investigated measurements, except SNB, in which it is not incorporated. In SNB, the y-coordinate of Nasion predominated error variance. SNB showed lowest inter-rater variation. In addition, our observations confirmed previous studies showing that landmark identification variance follows characteristic error envelopes in the highest number of tracings analysed up to now. Variance orthogonal to defining planes was of relevance, while variance parallel to planes was not. Taking these findings into account, orthognathic surgeons as well as orthodontists would be able to perform cephalometry more accurately and accomplish better therapeutic results. Copyright © 2018 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Familial acromegaly with pituitary adenoma. Report of three affected siblings.

PubMed

Abbassioun, K; Fatourehchi, V; Amirjamshidi, A; Meibodi, N A

1986-03-01

The authors report the cases of three brothers with pituitary adenomas who had classical findings of acromegaly and gigantism. Two had irreducibly elevated growth hormone (GH) values and underwent transsphenoidal microsurgical extirpation of their tumors. The third acromegalic brother had a normal GH value and evidence of panhypopituitarism; he had a small intrasellar tumor and a partially empty sella. The pattern of inheritance was probably autosomal recessive. A review of literature indicated that familial incidence of isolated acromegaly with pituitary adenomas is rare.

Deep Explosive Volcanism on the Gakkel Ridge and Seismological Constraints on Shallow Recharge at TAG Active Mound

DTIC Science & Technology

2013-02-01

Siberian margin (6.5 mm yr-1 full rate) [Vogt et al., 1979; DeMets et al., 1994; Sella et al., 2002]. For comparison, the ultraslow- spreading Southwest...that systemati~:ally decrea:;e from 12.8 mm yr 1 <~l its western eod {near Greeolund) to 6.5 nun yr 1 at its eastern end (the Siberian margin ) [Vogt et...perennial pack ice has made it possible to test these hypotheses. In 2007 the AGAVE expedi- tion utilized a oovcl vduclc (CAMPER) to acquire high

Pseudotumor Cerebri Resulting in Empty Sella Syndrome and Multiple Pituitary Hormone Deficiencies

DTIC Science & Technology

2017-09-16

of chronic headaches, back pain, decreased energy, and frequent nausea and vomiting. His growth velocity had slowed over the previous 3 years. On...exam, he had a eunuchoid body habitus without gynecomastia. He had sparse axillary hair , Tanner II pubic hair , and a phallus smaller than expected for...notable progression of puberty and linear growth acceleration. Subsequently, physiologic hydrocortisone replacement therapy resulted in resolution of

Pseudotumor Cerebri Resulting in Empty Sella Syndrome and Multiple Pituitary Hormone Deficiencies

DTIC Science & Technology

2017-09-14

of chronic headaches, back pain, decreased energy, and frequent nausea and vomiting. His growth velocity had slowed over the previous 3 years. On...exam, he had a eunuchoid body habltus without gynecomastia. He had sparse axillary hair , Tanner II pubic hair , and a phallus smaller than expected...with notable progression of puberty and linear growth acceleration. Subsequently, physiologic hydrocortisone replacement therapy resulted in resolution

Pure endoscopic transsphenoidal surgery for treatment of acromegaly: results of 67 cases treated in a pituitary center.

PubMed

Gondim, Jackson A; Almeida, João Paulo; de Albuquerque, Lucas Alverne F; Gomes, Erika; Schops, Michele; Ferraz, Tania

2010-10-01

Acromegaly is a chronic disease related to the excess of growth hormone (GH) and insulin-like growth factor–I secretion, usually by pituitary adenomas. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The introduction of endoscopy as an additional tool for surgical treatment of pituitary adenomas and, therefore, acromegaly represents an important advance of pituitary surgery in the recent years. The aim of this retrospective study is to evaluate the results of pure transsphenoidal endoscopic surgery in a series of patients with acromegaly who were operated on by a pituitary specialist surgeon. The authors discuss the advantages, outcome, complications, and factors related to the success of the endoscopic approach in cases of GHsecreting adenomas. The authors retrospectively analyzed data from cases involving patients with GH-secreting adenomas who underwent pure transsphenoidal endoscopic surgery at the Department of Neurosurgery of the General Hospital in Fortaleza, Brazil, between 2000 and 2009. Tumors were classified according to size as micro- or macroadenomas, and tumor extension was analyzed based on suprasellar/parasellar extension and sella floor destruction. All patients were followed up for at least 1 year. The criteria of disease control were GH levels < 1 ng/L after oral glucose tolerance test and normal insulin-like growth factor–I levels for age and sex. During the study period, 67 patients underwent pure endoscopic transsphenoidal surgery for treatment of acromegaly. Disease control was obtained in 50 cases (74.6%). The rate of treatment success was higher in patients with microadenomas (disease control achieved in 12 [85.7%] of 14 cases) than in those with larger lesions. Suprasellar/parasellar extension and high levels of sella floor erosion were associated with lower rates of disease control (p = 0.01 and p = 0.02, respectively). Complications related to the endoscopic surgery included

Postnatal treatment factors affecting craniofacial morphology of unilateral cleft lip and palate (UCLP) patients in a Japanese population.

PubMed

Alam, M K; Iida, J; Sato, Y; Kajii, Takashi S

2013-12-01

We have evaluated the craniofacial morphology of Japanese patients with unilateral cleft lip and palate (UCLP) and assessed the various postnatal factors that affect it. Lateral cephalograms of 140 subjects (mean (SD) aged 7 (2) years) with UCLP were taken before orthodontic treatment. Surgeons from Hokkaido University Hospital had done the primary operations. The craniofacial morphology was assessed by angular and linear cephalometric measurements. Cheiloplasty, palatoplasty, and preoperative orthopaedic treatment were chosen as postnatal factors. To compare the assessments of the postnatal factors, we made angular and linear cephalometric measurements for each subject and converted them into Z scores in relation to the mean (SD) of the two variables. Subjects treated by the modified Millard cheiloplasty had larger sella-nasion-point A (SNA) and nasion-point A-pogonion (NA-POG) measurements than subjects treated by the modified Millard with a vomer flap cheiloplasty. Two-stage palatoplasty showed consistently better craniofacial morphology than the other palatoplasty. Subjects who had preoperative orthopaedic treatment with a Hotz plate had significantly larger upper incisor/sella-nasion (U1-SN) measurements than who had no preoperative orthopaedic treatment or an active plate. We conclude that in subjects treated by a modified Millard type of cheiloplasty, a two-stage palatoplasty, and a Hotz plate there were fewer adverse effects on craniofacial morphology. Copyright © 2012 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Orthodontic camouflage versus orthognathic surgery for class III deformity: comparative cephalometric analysis.

PubMed

Martinez, P; Bellot-Arcís, C; Llamas, J M; Cibrian, R; Gandia, J L; Paredes-Gallardo, V

2017-04-01

The objective of this study was to compare different cephalometric variables in adult patients with class III malocclusions before and after treatment, in order to determine which variables are indicative of orthodontic camouflage or orthognathic surgery. The cases of 156 adult patients were assessed: 77 treated with orthodontic camouflage and 79 treated with orthodontics and orthognathic surgery. The following cephalometric variables were measured on pre-treatment (T1) and post-treatment (T2) lateral cephalograms: sella-nasion-A-point (SNA), sella-nasion-B-point (SNB), and A-point-nasion-B-point (ANB) angles, Wits appraisal, facial axis angle, mandibular plane angle, upper and lower incisor inclination, and inter-incisal angle. There were statistically significant differences in cephalometric variables before and after treatment between the two groups. The percentage of normal pre-treatment measurements in the camouflage orthodontics group was 30.7%, which worsened slightly to 28.4% post-treatment. However in the group receiving surgery, this was 24.5% pre-treatment, improving to 33.5% after surgery. SNA, SNB, Wits appraisal, lower incisor inclination, and inter-incisal angle showed differences between the two groups before and after treatment. Wits appraisal, lower incisor inclination, and inter-incisal angle were indicative of one or other treatment. Upper and lower incisor decompensation in both groups did not reach ideal values, which impeded complete skeletal correction in 52% of surgical cases. Copyright © 2016 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

De novo Craniopharyngioma of the Fourth Ventricle: Case Report and Review of Literature.

PubMed

Algahtani, Abdulhadi Y; Algahtani, Hussein A; Jamjoom, Abdulhakim B; Samkari, Alaa M; Marzuk, Yousef I

2018-01-01

Craniopharyngiomas usually involve the sella and suprasellar space. Their occurrence in the posterior fossa without extension to the suprasellar region is uncommon with only 16 cases reported in the literature. We report a case of a primary posterior fossa craniopharyngioma that was managed by complete excision with a good recovery. Our case was unique in that the craniopharyngioma occurred in the fourth ventricle and extended downward to the level of C1, a manifestation that was reported only twice in the past. The literature on the topic is reviewed.

Resection of a Retrochiasmatic Craniopharyngioma by Combined Modified Orbital Craniotomy and Transnasal Endoscopic Techniques.

PubMed

Patel, Nirav J; Dunn, Ian

2018-04-01

A 20-year-old patient presented with hydrocephalus but intact vision and hormone function. The MRI showed a large seller, suprasellar and third ventricular mass. We chose a combined approach utilizing the translyvian, lamina terminals route, with a possible interhemispheric approach. But, we also utilized a transnasal endoscopic approach for the tumor that remained below the diaphragma sellae. The patient did well, with complete tumor resection via a staged approach, but did require hormone replacement. The link to the video can be found at: https://youtu.be/yzpfOxzI4cQ .

Rapid spontaneous cerebrospinal fluid leak detected in the gastrointestinal tract.

PubMed

Ma, Hong Yun; Sen, Papia; Stein, Evan G; Freeman, Leonard M

2014-02-01

There are many causes of cerebrospinal (CSF) leaks. Most cases are secondary to blunt trauma and iatrogenic trauma caused by postoperative sequelae. Occasionally, CSF leakage may occur from nontraumatic or "spontaneous" causes, such as benign intracranial hypertension and "empty sella syndrome." Mass effect due to an encephalocele or meningocele may also be seen. Radionuclide cisternography is a sensitive method of determining CSF leak when combined with intranasal cotton pledget placement and analysis. We present a spontaneous CSF fluid leak that was detected when scintigraphic activity appeared first in the gastrointestinal tract.

Lack of normal MR enhancement of the pituitary gland: findings in three siblings with combined pituitary hormone deficiency.

PubMed

Falcone, S; Sanchez, J; Quencer, R M

1998-02-01

We present the MR appearance of the sella turcica in three sibling dwarfs with combined pituitary hormone deficiency in which MR images revealed a peripheral curvilinear band of enhancement about the pituitary gland in all three patients, a normal-size pituitary gland in two siblings, a mildly enlarged pituitary gland in one sibling, and a thin infundibulum and a normal posterior pituitary bright spot in all three siblings. Possible antecedents include an abnormal vascular supply, pituitary gland replacement by a nonfunctioning adenoma, a proteinaceous cyst, or a hamartoma.

Morphometric appraisal of the skull of Caroline Crachami, the Sicilian "dwarf" 1815?-1824: a contribution to the study of primordial microcephalic dwarfism.

PubMed

Jeffery, Nathan; Berkovitz, B K B

2002-08-15

The skeleton of Caroline Crachami is a rare historical example of primordial microcephalic dwarfism (PMD). Studies show the condition to be heterogeneous, with at least three types, for which the assessment criteria rely on descriptive evaluations and/or simple measures with regard to cranial features. Advances in noninvasive imaging allow for a more complete morphometric examination of the skull of Caroline Crachami with the aim of clarifying aspects of the condition. In the present study, the skull of Caroline Crachami was three-dimensionally imaged with computed tomography (CT) and reconstructed in virtual space. Coordinates for a number of measurements were taken to represent interesting anatomies with an emphasis on those measures not easily replicated on the skull itself. Volumes of the endocranial cavity and sella turcica were also computed. These data were compared with normative values taken from the literature and measured from CT images of the Bosma collection. Findings indicate that the general size of the skull is equivalent to that of a 6- to 8-month-old, that the endocranial volume and cranial base angle are commensurate with that of a newborn, and that the sella volume is the same as that for an 8- to 15-month-old. Apart from these traits, the skull was well proportioned and within the range of normal variation for a skull aged between 2-9 years. We conclude that further quantitative analysis on related skulls is warranted in the study of PMD using the methods and techniques described. Copyright 2002 Wiley-Liss, Inc.

Do craniopharyngioma molecular signatures correlate with clinical characteristics?

PubMed

Omay, Sacit Bulent; Chen, Yu-Ning; Almeida, Joao Paulo; Ruiz-Treviño, Armando Saul; Boockvar, John A; Stieg, Philip E; Greenfield, Jeffrey P; Souweidane, Mark M; Kacker, Ashutosh; Pisapia, David J; Anand, Vijay K; Schwartz, Theodore H

2018-05-01

OBJECTIVE Exome sequencing studies have recently demonstrated that papillary craniopharyngiomas (PCPs) and adamantinomatous craniopharyngiomas (ACPs) have distinct genetic origins, each primarily driven by mutually exclusive alterations: either BRAF ( V600E), observed in 95% of PCPs, or CTNNB1, observed in 75%-96% of ACPs. How the presence of these molecular signatures, or their absence, correlates with clinical, radiographic, and outcome variables is unknown. METHODS The pathology records for patients who underwent surgery for craniopharyngiomas between May 2000 and March 2015 at Weill Cornell Medical College were reviewed. Craniopharyngiomas were identified and classified as PCP or ACP. Patients were placed into 1 of 3 groups based on their genomic mutations: BRAF mutation only, CTNNB1 mutation only, and tumors with neither of these mutations detected (not detected [ND]). Demographic, radiological, and clinical variables were collected, and their correlation with each genomic group was tested. RESULTS Histology correlated strongly with mutation group. All BRAF tumors with mutations were PCPs, and all CTNNB1 with mutations and ND tumors were ACPs. Preoperative and postoperative clinical symptoms and radiographic features did not correlate with any mutation group. There was a statistically significant relationship (p = 0.0323) between the age group (pediatric vs adult) and the mutation groups. The ND group tumors were more likely to involve the sella (p = 0.0065). CONCLUSIONS The mutation signature in craniopharyngioma is highly predictive of histology. The subgroup of tumors in which these 2 mutations are not detected is more likely to occur in children, be located in the sella, and be of ACP histology.

Comparisons of chewing rhythm, craniomandibular morphology, body mass and height between mothers and their biological daughters.

PubMed

Cho, Catherine; Louie, Ke'ale; Maawadh, Ahmed; Gerstner, Geoffrey E

2015-11-01

To study and compare the relationships between mean chewing cycle duration, selected cephalometric variables representing mandibular length, face height, etc., measured in women and in their teenage or young-adult biological daughters. Daughters were recruited from local high schools and the University of Michigan School of Dentistry. Selection criteria included healthy females with full dentition, 1st molar occlusion, no active orthodontics, no medical conditions nor medication use that could interfere with normal masticatory motor function. Mothers had to be biologically related to their daughters. All data were obtained in the School of Dentistry. Measurements obtained from lateral cephalograms included: two "jaw length" measures, condylion-gnathion and gonion-gnathion, and four measures of facial profile including lower anterior face height, and angles sella-nasion-A point (SNA), sella-nasion-B point (SNB) and A point-nasion-B point (ANB). Mean cycle duration was calculated from 60 continuous chewing cycles, where a cycle was defined as the time between two successive maximum jaw openings in the vertical dimension. Other variables included subject height and weight. Linear and logistic regression analyses were used to evaluate the mother-daughter relationships and to study the relationships between cephalometric variables and chewing cycle duration. Height, weight, Co-Gn and Go-Gn were significantly correlated between mother-daughter pairs; however, mean cycle duration was not (r(2)=0.015). Mean cycle duration was positively correlated with ANB and height in mothers, but negatively correlated with Co-Gn in daughters. Chewing rate is not correlated between mothers and daughters in humans. Copyright © 2015 Elsevier Ltd. All rights reserved.

Imaging Evaluation of the Location and Fenestration of Sellar Floor during Endonasal Transsphenoidal Surgery in Patients with Pituitary Adenomas.

PubMed

Wang, Shousen; Qin, Yong; Xiao, Deyong; Wu, Zhifeng; Wei, Liangfeng

2018-05-03

To evaluate the clinical value of three-dimensional (3D) CT reconstruction of the sphenoidal sinus separation in localizing sellar floor during endonasal transsphenoidal surgery, and determine the size and location of sellar floor fenestration. After exclusion,51 patients were eligible for study inclusion. A pre-operative CT scan of the paranasal sinus and CT scan and MRI of the pituitary gland were obtained. Sphenoidal sinus separation was reconstructed using Mimics 15.0 software and the quantity, shape, and orientation were observed and compared with intra-operative data, the purpose of which was to guide the localization of sellar floor. Anatomic variation of the sphenoidal sinus and adjacent structures, tumor and sella turcica morphology, minimal distance between the cavernous segment of the internal carotid artery（CSICA） bilaterally, and the shortest distance from the midline were measured. Based upon the shape of the sphenoidal sinus separation, sellar floor was accurately localized in all cases. Intra-operative sphenoidal sinus separation was consistent with pre-operative 3D CT reconstruction images. The sellar floor was extremely small in two patients, and insufficient fenestration of the sellar floor negatively affected tumor resection. Pre-operative 3D CT reconstruction is helpful for accurate and rapid localization of the saddle floor. The anatomic variation of sphenoidal sinus and adjacent structures, the characteristics of tumor and Sella, the minimum distance between bilateral CSICA and the shortest distance from the midline are helpful for the establishment of individualized Sellar bottom fenestration. Copyright © 2018. Published by Elsevier Inc.

Ectopic Prolactinoma Presenting as Bacterial Meningitis: A Diagnostic Conundrum.

PubMed

Akinduro, Oluwaseun O; Akinduro, Olutomi T; Gupta, Vivek; Reimer, Ronald; Olomu, Osarenoma

2018-04-01

Prolactinomas may rarely present with meningitis and cerebrospinal fluid (CSF) rhinorrhea secondary to erosion of the wall of the sella turcica. It is even more uncommon for this abnormal communication to be caused by an ectopic prolactinoma arising from the sphenoid sinus and eroding into the sella. This atypical growth pattern makes diagnosis very difficult because there may be no displacement of the normal pituitary gland. The first reported case of a patient with an ectopic prolactinoma originating within the sphenoid sinus presenting primarily with meningitis is presented, and the management strategy and surgical and nonsurgical treatment options are discussed. A 48-year-old woman presented with confusion, low-pressure headache, and fever. A lumbar puncture revealed Streptococcus pneumoniae meningitis, and she was placed on intravenous penicillin G. After initiation of antibiotics, she noticed salty tasting postnasal fluid leakage. Imaging was remarkable for bony erosion of the sphenoid sinus wall by a soft tissue mass growing from within the sinus, with no disruption of the normal pituitary gland. A biopsy was then performed with an endoscopic transnasal transsphenoidal approach, and the CSF leak was repaired with a pedicled nasoseptal flap. The final pathology was prolactinoma, and she was placed on cabergoline. Ectopic prolactinomas may rarely present as meningitis secondary to retrograde transmission of bacteria through a bony defect in the sphenoid sinus, and must be included in the differential diagnosis of any sphenoid sinus mass. Management should first address the infection, followed by surgical repair of the bony defect. Copyright © 2018 Elsevier Inc. All rights reserved.

Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis.

PubMed

van der Pol, R; Nieuwenhuis, M G; Mourits, M P

1999-03-01

Multifocal fibrosclerosis (MF) is a rare disease that may be misdiagnosed as Graves' orbitopathy. The combination of localisations of MF presented here has not been reported before. A 44-year-old man was referred with progressive bilateral exophthalmos. CT of his chest and abdomen revealed an intrathoracic and retroperitoneal peri-aortal soft-tissue process with hydronephrosis. Histological examination of orbital masses showed a fibrous process with low inflammatory activity. Later the sella was found to be involved as well. A diagnosis of MF was made. MF should be considered in the differential diagnosis of bilateral exophthalmos.

Two interesting cases highlighting an oblivious specialty of psychoneuroendocrinology.

PubMed

Hari Kumar, K V S; Dhull, Pawan; Somasekharan, Manoj; Seshadri, K

2012-01-01

Psychoneuroendocrinology deals with the overlap disorders pertaining to three different specialties. Awareness about the somatic manifestations of psychiatric diseases and vice versa is a must for all the clinicians. The knowledge of this interlinked specialty is essential because of the obscure presentation of certain disorders. Our first case was treated as depressive disorder, whereas the diagnosis was hypogonadism with empty sella. Our second patient was managed as schizophrenia and the evaluation revealed bilateral basal ganglia calcification and a diagnosis of Fahr's disease. We report these cases for their unusual presentation and to highlight the importance of this emerging specialty.

An unusual intracranial metallic foreign bodies and panhypopituitarism.

PubMed

Lakouichmi, Mohammed; Baïzri, Hicham; Mouhsine, Abdelilah; Boukhira, Abderrahmane; Akhaddar, Ali

2014-01-01

A 49 years old man, with a history of aggression at the age of 18 years by a pair of scissors, who consulted for unilateral migraine headaches look straight. Paraclinical explorations concluded that trauma to anterior pituitary by a metallic foreign body from the right nostril to the sella, responsible for panhypopituitarism and sinusitis. The headaches are frequent causes of consultation, often treated symptomatically but rarely explored. The direct trauma to the pituitary gland, by a metallic foreign body, is exceptional. We report the case of neglected panhypopituitarism, discovered 31 years after injury with a pair of scissors.

An unusual intracranial metallic foreign bodies and panhypopituitarism

PubMed Central

Lakouichmi, Mohammed; Baïzri, Hicham; Mouhsine, Abdelilah; Boukhira, Abderrahmane; Akhaddar, Ali

2014-01-01

A 49 years old man, with a history of aggression at the age of 18 years by a pair of scissors, who consulted for unilateral migraine headaches look straight. Paraclinical explorations concluded that trauma to anterior pituitary by a metallic foreign body from the right nostril to the sella, responsible for panhypopituitarism and sinusitis. The headaches are frequent causes of consultation, often treated symptomatically but rarely explored. The direct trauma to the pituitary gland, by a metallic foreign body, is exceptional. We report the case of neglected panhypopituitarism, discovered 31 years after injury with a pair of scissors. PMID:25667695

Medical Therapy for Cushing's Syndrome in the Twenty-first Century.

PubMed

Tritos, Nicholas A; Biller, Beverly M K

2018-06-01

Medical therapy has a useful adjunctive role in many patients with Cushing's syndrome. Patients with pituitary corticotroph adenomas who have received radiation therapy to the sella require medical therapy until the effects of radiation therapy occur. In addition, patients with Cushing's syndrome who cannot undergo surgery promptly, including those who are acutely ill and cannot safely undergo tumor resection, may benefit from medical therapy as a bridge to surgery. Other possible candidates for medical therapy are those with unresectable tumors or those whose tumor location remains unknown despite adequate diagnostic evaluation. Copyright © 2018 Elsevier Inc. All rights reserved.

Anatomic comparison of the endonasal and transpetrosal approaches for interpeduncular fossa access.

PubMed

Oyama, Kenichi; Prevedello, Daniel M; Ditzel Filho, Leo F S; Muto, Jun; Gun, Ramazan; Kerr, Edward E; Otto, Bradley A; Carrau, Ricardo L

2014-01-01

The interpeduncular cistern, including the retrochiasmatic area, is one of the most challenging regions to approach surgically. Various conventional approaches to this region have been described; however, only the endoscopic endonasal approach via the dorsum sellae and the transpetrosal approach provide ideal exposure with a caudal-cranial view. The authors compared these 2 approaches to clarify their limitations and intrinsic advantages for access to the interpeduncular cistern. Four fresh cadaver heads were studied. An endoscopic endonasal approach via the dorsum sellae with pituitary transposition was performed to expose the interpeduncular cistern. A transpetrosal approach was performed bilaterally, combining a retrolabyrinthine presigmoid and a subtemporal transtentorium approach. Water balloons were used to simulate space-occupying lesions. "Water balloon tumors" (WBTs), inflated to 2 different volumes (0.5 and 1.0 ml), were placed in the interpeduncular cistern to compare visualization using the 2 approaches. The distances between cranial nerve (CN) III and the posterior communicating artery (PCoA) and between CN III and the edge of the tentorium were measured through a transpetrosal approach to determine the width of surgical corridors using 0- to 6-ml WBTs in the interpeduncular cistern (n = 8). Both approaches provided adequate exposure of the interpeduncular cistern. The endoscopic endonasal approach yielded a good visualization of both CN III and the PCoA when a WBT was in the interpeduncular cistern. Visualization of the contralateral anatomical structures was impaired in the transpetrosal approach. The surgical corridor to the interpeduncular cistern via the transpetrosal approach was narrow when the WBT volume was small, but its width increased as the WBT volume increased. There was a statistically significant increase in the maximum distance between CN III and the PCoA (p = 0.047) and between CN III and the tentorium (p = 0.029) when the WBT volume

The endoscopic endonasal approach is not superior to the microscopic transcranial approach for anterior skull base meningiomas-a meta-analysis.

PubMed

Muskens, Ivo S; Briceno, Vanessa; Ouwehand, Tom L; Castlen, Joseph P; Gormley, William B; Aglio, Linda S; Zamanipoor Najafabadi, Amir H; van Furth, Wouter R; Smith, Timothy R; Mekary, Rania A; Broekman, Marike L D

2018-01-01

In the past decade, the endonasal transsphenoidal approach (eTSA) has become an alternative to the microsurgical transcranial approach (mTCA) for tuberculum sellae meningiomas (TSMs) and olfactory groove meningiomas (OGMs). The aim of this meta-analysis was to evaluate which approach offered the best surgical outcomes. A systematic review of the literature from 2004 and meta-analysis were conducted in accordance with the PRISMA guidelines. Pooled incidence was calculated for gross total resection (GTR), visual improvement, cerebrospinal fluid (CSF) leak, intraoperative arterial injury, and mortality, comparing eTSA and mTCA, with p-interaction values. Of 1684 studies, 64 case series were included in the meta-analysis. Using the fixed-effects model, the GTR rate was significantly higher among mTCA patients for OGM (eTSA: 70.9% vs. mTCA: 88.5%, p-interaction < 0.01), but not significantly higher for TSM (eTSA: 83.0% vs. mTCA: 85.8%, p-interaction = 0.34). Despite considerable heterogeneity, visual improvement was higher for eTSA than mTCA for TSM (p-interaction < 0.01), but not for OGM (p-interaction = 0.33). CSF leak was significantly higher among eTSA patients for both OGM (eTSA: 25.1% vs. mTCA: 10.5%, p-interaction < 0.01) and TSM (eTSA: 19.3%, vs. mTCA: 5.81%, p-interaction < 0.01). Intraoperative arterial injury was higher among eTSA (4.89%) than mTCA patients (1.86%) for TSM (p-interaction = 0.03), but not for OGM resection (p-interaction = 0.10). Mortality was not significantly different between eTSA and mTCA patients for both TSM (p-interaction = 0.14) and OGM resection (p-interaction = 0.88). Random-effect models yielded similar results. In this meta-analysis, eTSA was not shown to be superior to mTCA for resection of both OGMs and TSMs.

Portlandemys gracilis n. sp., a New Coastal Marine Turtle from the Late Jurassic of Porrentruy (Switzerland) and a Reconsideration of Plesiochelyid Cranial Anatomy

PubMed Central

Anquetin, Jérémy; Püntener, Christian; Billon-Bruyat, Jean-Paul

2015-01-01

Background Several groups of stem cryptodires became adapted to coastal marine environments as early as the Late Jurassic, 40 million years before the Pan-Chelonioidea. The Plesiochelyidae are a major component of this first radiation of crown-group turtles into marine habitats. They are abundant in many European localities, but their systematics is still greatly confused. Only three species are represented by cranial material: Plesiochelys etalloni, Plesiochelys planiceps, and Portlandemys mcdowelli. Methodology/Principal Findings In the present study, we describe a cranium and a mandible from the Kimmeridgian of Porrentruy (Switzerland), which we refer to a new species, Portlandemys gracilis n. sp. This new taxon differs from Portlandemys mcdowelli in several aspects of the cranium and mandible, notably in being generally more gracile, but the two species share a narrow skull, a more acute angle between the labial ridges on the mandible, and a unique configuration of the anterodorsal part of the basicranium. The cranial anatomy of plesiochelyid turtles is discussed in details based primarily on these new specimens and new cranial material of Plesiochelys etalloni from Solothurn, Switzerland. Conclusions/Significance Several characters (e.g., the contribution of the parietal to the foramen nervi trigemini, the configuration of the dorsum sellae and sella turcica, the presence of an infolding ridge on the posterior surface of the quadrate) appear as potential candidates to help elucidate plesiochelyid relationships. Some of these characters are included in a previously published phylogenetic dataset and help to stabilize the relationships of plesiochelyid turtles and closely related taxa. For the first time, our results suggest that plesiochelyids, 'Thalassemys' moseri, and Solnhofia parsonsi (representing the Eurysternidae) form a clade at the base of Eucryptodira. PMID:26106888

Development of pituitary adenoma in women with hyperprolactinaemia: clinical, endocrine, and radiological characteristics.

PubMed Central

Pontiroli, A E; Falsetti, L

1984-01-01

Sixty eight women referred for treatment of hyperprolactinaemia entered a three year follow up study to determine the clinical and endocrine course of the disease and its association with microadenoma of the pituitary. Details recorded before treatment included medical history, gonadotrophin and ovarian hormonal concentrations, and release of prolactin in response to protirelin (thyrotrophin releasing hormone), benserazide, cimetidine, and nomifensine. Sellar tomography was then performed yearly for three years in all women, 54 of them also undergoing computed coronal and sagittal tomography. At baseline evaluation 27 women showed radiological evidence of pituitary adenoma; at the end of the follow up period the number had increased to 41. Amenorrhoea, steady and raised serum prolactin concentrations, a low ratio of luteinising hormone to follicle stimulating hormone, a longer duration of disease, and low serum progesterone concentrations were more common in women with a final diagnosis of pituitary adenoma than in those whose sella remained normal. Tests for release of prolactin had yielded abnormal results from the outset in all 41 women with radiological evidence of pituitary adenoma and in about half of those whose sella had remained radiologically normal. Response to medical treatment (metergoline in 20 patients, bromocriptine in 21) was similar and showed no difference between patients with tumorous and non-tumorous hyperprolactinaemia. These findings suggest that a large proportion of women with hyperprolactinaemia may harbour a prolactin secreting pituitary adenoma which becomes apparent over a relatively short period. Amenorrhoea and steady and raised serum prolactin concentrations are more common in these women. Tests for release of prolactin are of predictive value in identifying women who will develop a pituitary adenoma. PMID:6421360

[Pituitary gland tumors].

PubMed

Jesser, J; Schlamp, K; Bendszus, M

2014-10-01

This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15% of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65% of pituitary gland adenomas secrete hormones whereby approximately 50% secrete prolactin, 10% secrete growth hormone (somatotropin) and 6% secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10% of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland.

Sellar reconstruction without intrasellar packing after endoscopic surgery of pituitary macroadenomas is better than its reputation.

PubMed

Ismail, Mostafa; Fares, Abd Alla; Abdelhak, Balegh; D'Haens, Jean; Michel, Olaf

2016-01-01

Sellar reconstruction with intrasellar packing following endoscopic resection of pituitary macroadenomas remains a subject of clinical and radiological discussion particularly, when an intraoperative cerebrospinal fluid (CSF) leakage is absent. This study was conducted to contribute our experience with sellar reconstruction after a standard endoscopic surgery of pituitary macroadenomas without intraoperative CSF leakage to the ongoing discussion between techniques with and without intrasellar packing. A consecutive series of 47 pituitary macroadenomas undergoing excision via a standard endoscopic endonasal transsphenoidal surgery (EETS) without evident intraoperative CSF leakage were retrospectively evaluated over a 10-months mean follow-up period. According to the sellar reconstruction technique, three groups could be identified: Group A - with no intrasellar packing, Group B - with haemostatic materials packing, and Group C - with abdominal fat packing. Postoperative clinical and radiological assessments of the three groups were documented and analyzed for differences in outcome. Postoperative clinical assessment did not differ significantly between the three groups. In group A, postoperative CSF leakage, sphenoid sinusitis and empty sella syndrome were not observed. However, a significant difference in radiological assessment could be identified; the interpretation of sellar contents in postoperative MRI of group A succeeded earlier and more reliably than in other groups with intrasellar packing. There is no difference in the incidence of postoperative CSF leakage and empty sella syndrome among the various reconstructive techniques with and without intrasellar packing, irrespective of size and extension of the pituitary adenoma. Sellar reconstruction without intrasellar packing following a standard EETS is not inferior to other techniques with packing and even shows more radiological advantages, which made it our preferred technique, at least if no

Combined pituitary hormone deficiency in a girl with 48, XXXX and Rathke's cleft cyst.

PubMed

Uppal, Surabhi; Jee, Youn Hee; Lightbourne, Marissa; Han, Joan C; Stratakis, Constantine A

2017-01-01

Tetrasomy X is a rare chromosomal aneuploidy seen in girls, associated with facial dysmorphism, premature ovarian insufficiency and intellectual disability. A Rathke's cleft cyst (RCC) is a remnant of Rathke's pouch which may cause multiple pituitary hormone deficiencies by exerting pressure on the pituitary gland in the sella. The patient was diagnosed with tetrasomy X by karyotyping during infancy. Brain MRI and multiple endocrine stimulation tests revealed RCC and combined pituitary hormone deficiency (growth hormone deficiency, secondary adrenal insufficiency and central hypothyroidism) likely due to RCC. We report the first case in the literature of a girl with 48, XXXX and combined pituitary hormone deficiency due to Rathke's cyst.

Ruptured Intrasellar Superior Hypophyseal Artery Aneurysm Presenting with Pure Subdural Haematoma

PubMed Central

Hornyak, M.; Hillard, V.; Nwagwu, C.; Zablow, B. C.; Murali, R.

2004-01-01

Summary Subdural haemorrhage from a ruptured intracranial aneurysm is a well-known entity when associated with subarachnoid haemorrhage. However, haemorrhage confined only to the subdural space is rare because there are limited anatomical sites where extravasation can be purely subdural. We report the rare case of a patient who suffered pure subdural haematoma after the rupture of a left superior hypophyseal artery aneurysm located within the sella turcica. The patient was treated with endovascular coil embolization of the aneurysm. Angiography immediately after treatment and one month later revealed complete obliteration of the aneurysm. Six months after treatment, the patient remained symptom free. PMID:20587264

Internal maxillary distraction with a new bimalar device.

PubMed

Kahn, David M; Broujerdi, Joseph; Schendel, Stephen A

2008-04-01

Distraction osteogenesis of the Le Fort I segment is advocated for patients who require significant advancement of the maxilla or who have a soft tissue envelope compromised by scar tissue. We present a technique for maxillary distraction using an interconnecting intraoral device anchored to the malar prominences above the osteotomy and either the maxilla and/or the dentition below the level of the osteotomy. Ten patients with nonsyndromic cleft lip and palate, mean age of 18, underwent Le Fort I maxillary distraction osteogenesis for management of maxillary hypoplasia. A Le Fort I osteotomy is performed and a Spectrum Intraoral Midface Multi-Vector Distractor (OsteoMed, Addison, TX) is placed leaving a 1 mm to 2 mm distraction gap. After a 2 to 4 day latency period, distraction begins at a rate of 1 mm a day. Once the desired occlusion is achieved the device is left in place for a minimum of 2 months for consolidation. Preoperative Sella-Nasion-A point measurements from lateral cephalograms averaged 74 degrees (range, 70-76 degrees). Postoperative Sella-Nasion-A point averaged 81 degrees (range, 75-89 degrees). Preoperative overjet averaged -7.4 mm (range, -3 to -13 mm). Postoperative overjet averaged 2.6 mm (range, 1-3 mm). Average distraction was 9 mm (range, 6-16 mm). The average vertical movement was 7.2 mm in an inferior direction (range, 0-15 mm). The results remained stable at a follow-up of 30 months. We report on distraction of the Le Fort I segment using an internal device. The device design allows the forces of distraction to be shared across a larger surface area delivering a uniform and reliable vector of distraction with increased stability.

MRI features of growth hormone deficiency in children with short stature caused by pituitary lesions.

PubMed

Xu, Chao; Zhang, Xinxian; Dong, Lina; Zhu, Bin; Xin, Tao

2017-06-01

We verified the advantages of using magnetic resonance imaging (MRI) for improving the diagnostic quality of growth hormone deficiency (GHD) in children with short stature caused by pituitary lesions. Clinical data obtained from 577 GHD patients with short stature caused by pituitary lesions were retrospectively analyzed. There were 354 cases (61.3%) with anterior pituitary dysplasia; 45 cases (7.8%) of pituitary stalk interruption syndrome (PSIS); 15 cases (2.6%) of pituitary hyperplasia due to primary hypothyroidism; 38 cases (6.6%) of Rathke cleft cyst; 68 cases (11.8%) of empty sella syndrome; 16 cases (2.8%) of pituitary invasion from Langerhans cell histiocytosis; 2 cases (0.3%) of sellar regional arachnoid cyst and 39 cases (6.8%) of craniopharyngioma. MRI results showed that the height of anterior pituitary in patients was less than normal. Location, size and signals of posterior pituitary and pituitary stalk were normal in anterior pituitary dysplasia. In all cases pituitary hyperplasia was caused by hypothyroidism. MRI results showed that anterior pituitary was enlarged, and we detected upward apophysis and obvious homogeneous enhancement. There were no pituitary stalk interruption and abnormal signal. We also observed that after hormone replacement therapy the size of pituitary gland was reduced. Anterior pituitary atrophy was observed in Rathke cleft cyst, empty sella syndrome, sellar regional arachnoid cyst and craniopharyngioma. The microstructure of hypophysis and sellar region was studied with MRI. We detected pituitary lesions, and the characteristics of various pituitary diseases of GHD in children with short stature. It was concluded that in children with GHD caused by pituitary lesions, MRI was an excellent method for early diagnosis. This method offers clinical practicability and we believe it can be used for differential diagnosis and to monitor the therapeutic effects.

Collision tumors of the sella: coexistence of pituitary adenoma and craniopharyngioma in the sellar region

PubMed Central

2013-01-01

Collision tumors of the sellar region are relatively uncommon and consist mainly of more than one type of pituitary adenoma or a cyst or cystic tumor. The association of a pituitary adenoma and a craniopharyngioma is particularly rare. This study describes a rare occurrence in which a pituitary adenoma and a craniopharyngioma coexisted in the sellar region. The case involves a 47-year-old woman who underwent transsphenoidal surgery with subtotal tumor resection and reoperation using an interhemispheric transcallosal approach for total microsurgical resection of the tumor because the visual acuity in her left eye had re-deteriorated. Histopathological and immunohistochemical examinations of the excised tissue revealed a pituitary adenoma in the first operation and a craniopharyngioma in the second operation. Retrospective analysis found the coexistence of a pituitary adenoma and a craniopharyngioma, known as a collision tumor. Instead of the transsphenoidal approach, a craniotomy should be performed, to explore the suprasellar region. PMID:23919255

Folate receptor overexpression can be visualized in real time during pituitary adenoma endoscopic transsphenoidal surgery with near-infrared imaging.

PubMed

Lee, John Y K; Cho, Steve S; Zeh, Ryan; Pierce, John T; Martinez-Lage, Maria; Adappa, Nithin D; Palmer, James N; Newman, Jason G; Learned, Kim O; White, Caitlin; Kharlip, Julia; Snyder, Peter; Low, Philip S; Singhal, Sunil; Grady, M Sean

2017-08-25

OBJECTIVE Pituitary adenomas account for approximately 10% of intracranial tumors and have an estimated prevalence of 15%-20% in the general US population. Resection is the primary treatment for pituitary adenomas, and the transsphenoidal approach remains the most common. The greatest challenge with pituitary adenomas is that 20% of patients develop tumor recurrence. Current approaches to reduce recurrence, such as intraoperative MRI, are costly, associated with high false-positive rates, and not recommended. Pituitary adenomas are known to overexpress folate receptor alpha (FRα), and it was hypothesized that OTL38, a folate analog conjugated to a near-infrared (NIR) fluorescent dye, could provide real-time intraoperative visual contrast of the tumor versus the surrounding nonneoplastic tissues. The preliminary results of this novel clinical trial are presented. METHODS Nineteen adult patients who presented with pituitary adenoma were enrolled. Patients were infused with OTL38 2-4 hours prior to surgery. A 4-mm endoscope with both visible and NIR light capabilities was used to visualize the pituitary adenoma and its margins in real time during surgery. The signal-to-background ratio (SBR) was recorded for each tumor and surrounding tissues at various endoscope-to-sella distances. Immunohistochemical analysis was performed to assess the FRα expression levels in all specimens and classify patients as having either high or low FRα expression. RESULTS Data from 15 patients (4 with null cell adenomas, 1 clinically silent gonadotroph, 1 totally silent somatotroph, 5 with a corticotroph, 3 with somatotrophs, and 1 somatocorticotroph) were analyzed in this preliminary analysis. Four patients were excluded for technical considerations. Intraoperative NIR imaging delineated the main tumors in all 15 patients with an average SBR of 1.9 ± 0.70. The FRα expression level of the adenomas and endoscope-to-sella distance had statistically significant impacts on the fluorescent

Radiological features of the skull in Klinefelter's syndrome and male hypogonadism.

PubMed

Kosowicz, J; Rzymski, K

1975-07-01

Skull radiographs were performed in 21 cases of Klinefelter's syndrome and in 30 cases of eunuchoidism. The radiographic changes of the skull in Klinefelter's syndrome are: temporal flattening, decreased width of the vault, narrowing of the mandible, decreased length of the skull, shortening of the anterior fossa cranii, decrease in the angle of the base, thinning of the vault bones at the major fontanelle, premature and excessive calcification of the coronal suture, deepening of the posterior fossa and shortening of the mandibular rami. In hypogonadotropic eunuchoidism the skull radiographs show: small mastoid processes, fine bones of the vault, small sella turcica, club-shaped clinoid processes, excessive development of sphenoidal sinuses and in the fourth and later decades of life a diminished bone density (osteoporosis).

Endoscopic transnasal resection of anterior cranial fossa meningiomas.

PubMed

de Divitiis, Enrico; Esposito, Felice; Cappabianca, Paolo; Cavallo, Luigi M; de Divitiis, Oreste; Esposito, Isabella

2008-01-01

The extended transnasal approach, a recent surgical advancements for the ventral skull base, allows excellent midline access to and visibility of the anterior cranial fossa, which was previously thought to be approachable only via a transcranial route. The extended transnasal approach allows early decompression of the optic canals, obviates the need for brain retraction, and reduces neurovascular manipulation. Between 2004 and 2007, 11 consecutive patients underwent transnasal resection of anterior cranial fossa meningiomas--4 olfactory groove (OGM) and 7 tuberculum sellae (TSM) meningiomas. Age at surgery, sex, symptoms, and imaging studies were reviewed. Tumor size and tumor extension were estimated, and the anteroposterior, vertical, and horizontal diameters were measred on MR images. Medical records, surgical complications, and outcomes of the patients were collected. A gross-total removal of the lesion was achieved in 10 patients (91%), and in 1 patient with a TSM only a near-total (> 90%) resection was possible. Four patients with preoperative visual function defect had a complete recovery, whereas 3 patients experienced a transient worsening of vision, fully recovered within few days. In 3 patients (2 with TSMs and 1 with an OGM), a postoperative CSF leak occurred, requiring a endoscopic surgery for skull base defect repair. Another patient (a case involving a TSM) developed transient diabetes insipidus. The operative time ranged from 6 to 10 hours in the OGM group and from 4.5 to 9 hours in the TSM group. The mean duration of the hospital stay was 13.5 and 10 days in the OGM and TSM groups, respectively. Six patients (3 with OGMs and 3 with TSMs) required a blood transfusion. Surgery-related death occurred in 1 patient with TSM, in whom the tumor was successfully removed. The technique offers a minimally invasive route to the midline anterior skull base, allowing the surgeon to avoid using brain retraction and reducing manipulation of the large vessels and

A role for 11C-methionine PET imaging in ACTH-dependent Cushing's syndrome.

PubMed

Koulouri, Olympia; Steuwe, Andrea; Gillett, Daniel; Hoole, Andrew C; Powlson, Andrew S; Donnelly, Neil A; Burnet, Neil G; Antoun, Nagui M; Cheow, Heok; Mannion, Richard J; Pickard, John D; Gurnell, Mark

2015-10-01

We report our experience of functional imaging with (11)C-methionine positron emission tomography-computed tomography (PET-CT) co-registered with 3D gradient echo (spoiled gradient recalled (SPGR)) magnetic resonance imaging (MRI) in the investigation of ACTH-dependent Cushing's syndrome. Twenty patients with i) de novo Cushing's disease (CD, n=10), ii) residual or recurrent hypercortisolism following first pituitary surgery (±radiotherapy; n=8) or iii) ectopic Cushing's syndrome (n=2) were referred to our centre for functional imaging studies between 2010 and 2015. Six of the patients with de novo CD and five of those with persistent/relapsed disease had a suspected abnormality on conventional MRI. All patients underwent (11)C-methionine PET-CT. For pituitary imaging, co-registration of PET-CT images with contemporaneous SPGR MRI (1 mm slice thickness) was performed, followed by detailed mapping of (11)C-methionine uptake across the sella in three planes (coronal, sagittal and axial). This allowed us to determine whether suspected adenomas seen on structural imaging exhibited focal tracer uptake on functional imaging. In seven of ten patients with de novo CD, asymmetric (11)C-methionine uptake was observed within the sella, which co-localized with the suspected site of a corticotroph microadenoma visualised on SPGR MRI (and which was subsequently confirmed histologically following successful transsphenoidal surgery (TSS)). Focal (11)C-methionine uptake that correlated with a suspected abnormality on pituitary MRI was seen in five of eight patients with residual or recurrent Cushing's syndrome following first TSS (and pituitary radiotherapy in two cases). Two patients elected to undergo repeat TSS with histology confirming a corticotroph tumour in each case. In two patients with the ectopic ACTH syndrome, (11)C-methionine was concentrated in sites of distant metastases, with minimal uptake in the sellar region. (11)C-methionine PET-CT can aid the detection of

Extended endoscopic endonasal skull base surgery: from the sella to the anterior and posterior cranial fossa.

PubMed

Oostra, Amanda; van Furth, Wouter; Georgalas, Christos

2012-03-01

Skull base surgery has gone through significant changes with the development of extended endoscopic endonasal approaches over the last decade. Initially used for the transphenoidal removal of hypophyseal adenomas, the endoscopic transnasal approach gradually evolved into a way of accessing the whole ventral skull base. Improved visualization, avoidance of brain retraction, the ability to access directly tumours with minimal damage to critical neurosurgical structures as well lack of external scars are among its obvious benefits. However, it presents the surgeons with a number of challenges, including the need to deal endoscopically with potential arterial bleeding, complicated reconstruction requirements as well as the need for a true team approach. In this review drawing from our experience as well as published series, we present an overview of current indications, challenges and limitations of the expanded endonasal approaches to the skull base. © 2012 The Authors. ANZ Journal of Surgery © 2012 Royal Australasian College of Surgeons.

Easy sellar reconstruction in endoscopic endonasal transsphenoidal surgery with polyester-silicone dural substitute and fibrin glue: technical note.

PubMed

Cappabianca, P; Cavallo, L M; Mariniello, G; de Divitiis, O; Romero, A D; de Divitiis, E

2001-08-01

To describe a simple method of sellar reconstruction after endoscopic endonasal transsphenoidal surgery that will allow rapid watertight closure of the sellar floor. A bent sheet of a polyester-silicone dural substitute, fashioned for this purpose with scissors, is introduced into the sella after removal of the lesion. Because of the consistency of the sheet, it opens spontaneously and becomes stuck. Autologous fat tissue or gelatin foam is positioned thereafter, followed by another layer of the dural substitute; a film of fibrin glue completes the sealing. Fifteen patients underwent this method and no postoperative cerebrospinal leak or other complication was experienced. This easy method of sellar reconstruction represents an effective and fast possibility to perform the final step of the endoscopic transsphenoidal procedure, which otherwise may cause maneuverability problems in the limited space of one nostril.

Pituitary adenoma with seizures: PET demonstration of reduced glucose utilization in the medial temporal lobe

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bairamian, D.; Di Chiro, G.; Blume, H.

1986-05-01

A patient with a benign chromophobe adenoma, who had incomplete surgical removal followed by radiotherapy, continued to have epileptic seizures up to two or three times a day. She was studied with positron emission tomography using /sup 18/F-2-deoxyglucose (FDG). This technique showed a high level of glucose utilization in the area of the operated tumor but also clear reduction of glucose utilization in the left medial temporal region adjacent to the sella and the scar tissue from the neoplasm. This area of reduced glucose utilization corresponded well to the same finding observed in other patients with complex partial epilepsy. Amore » left temporal anterior lobectomy was carried out followed by improved control of the epilepsy. Positron emission tomography using FDG, together with electrophysiological examinations, may assist in the management of epilepsy related to pituitary tumors.« less

Natura abhorret a vacuo--use of fibrin glue as a filler and sealant in neurosurgical "dead spaces". Technical note.

PubMed

Cappabianca, Paolo; Esposito, Felice; Magro, Francesco; Cavallo, Luigi Maria; Solari, Domenico; Stella, Lucio; de Divitiis, Oreste

2010-05-01

The objective of this study is to report our experience and illustrate our technique in the use of fibrin glue in the treatment of post-operatory cerebrospinal fluid (CSF) leaks and collections following different neurosurgical procedures. In a 3-year period, 40 subjects underwent endoscopic endonasal approach for different sellar and skull base lesions (three tuberculum sellae meningiomas, six craniopharyngiomas, three Rathke's cleft cysts and 28 pituitary macroadenomas), in which an intraoperative CSF leakage was evident. In such subjects, the fibrin glue was used as a first step of the final phase of the procedure-i.e. the reconstruction of the skull base defect-followed by the other materials employed. Furthermore, ten other patients, who had undergone transsphenoidal (four cases), spinal (two cases), posterior fossa (three cases) and transcortical intraventricular tumour removal (one case) neurosurgical procedures and developed CSF leaks or collections, were conservatively treated by single or repeated in situ injections of "modified" fibrin glue under local anaesthesia according to different described techniques. In total, 50 patients constitute the clinical material of the present study. In the cases where the fibrin glue was used during the reconstruction phase of the procedure (40 cases), the glue was injected inside the tumour cavity to fill the dead space left by the removal of the lesion. In case of post-operative CSF leak or CSF fluid collection (ten cases), after discarding 50-80% of the thrombin solution to obtain prevalence of the product's adhesive properties, fibrin glue was injected directly in the path of the CSF leak or into the collection cavity after aspiration of the collection's content. This was performed with the provided application system or through lumbar or Tuohy needles. Applications were repeated every 48 h until the disappearance of the leak. In all the treated cases, the disappearance of CSF leaks or collections was obtained with

Endosphenoidal coil for intraoperative magnetic resonance imaging of the pituitary gland during transsphenoidal surgery.

PubMed

Chittiboina, Prashant; Talagala, S Lalith; Merkle, Hellmut; Sarlls, Joelle E; Montgomery, Blake K; Piazza, Martin G; Scott, Gretchen; Ray-Chaudhury, Abhik; Lonser, Russell R; Oldfield, Edward H; Koretsky, Alan P; Butman, John A

2016-12-01

OBJECTIVE Pituitary MR imaging fails to detect over 50% of microadenomas in Cushing's disease and nearly 80% of cases of dural microinvasion. Surface coils can generate exceptionally high-resolution images of the immediately adjacent tissues. To improve imaging of the pituitary gland, a receive-only surface coil that can be placed within the sphenoid sinus (the endosphenoidal coil [ESC]) during transsphenoidal surgery (TSS) was developed and assessed. METHODS Five cadaver heads were used for preclinical testing of the ESC. The ESC (a double-turn, 12-mm-diameter surface coil made from 1-mm-diameter copper wire) was developed to obtain images in a 1.5-T MR scanner. The ESC was placed (via a standard sublabial TSS approach) on the anterior sella face. Clinical MR scans were obtained using the 8-channel head coil and ESC as the receiver coils. Using the ESC, ultra-high-resolution, 3D, balanced fast field echo (BFFE) and T1-weighted imaging were performed at resolutions of 0.25 × 0.25 × 0.50 mm 3 and 0.15 × 0.15 × 0.30 mm 3 , respectively. RESULTS Region-of-interest analysis indicated a 10-fold increase in the signal-to-noise ratio (SNR) of the pituitary when using the ESC compared with the 8-channel head coil. ESC-related improvements (p < 0.01) in the SNR were inversely proportional to the distance from the ESC tip to the anterior pituitary gland surface. High-resolution BFFE MR imaging obtained using ESC revealed a number of anatomical features critical to pituitary surgery that were not visible on 8-channel MR imaging, including the pituitary capsule, the intercavernous sinus, and microcalcifications in the pars intermedia. These ESC imaging findings were confirmed by the pathological correlation with whole-mount pituitary sections. CONCLUSIONS ESC can significantly improve SNR in the sellar region intraoperatively using current 1.5-T MR imaging platforms. Improvement in SNR can provide images of the sella and surrounding structures with unprecedented resolution

Endosphenoidal coil for intraoperative magnetic resonance imaging of the pituitary gland during transsphenoidal surgery

PubMed Central

Chittiboina, Prashant; Talagala, S. Lalith; Merkle, Hellmut; Sarlls, Joelle E.; Montgomery, Blake K.; Piazza, Martin G.; Scott, Gretchen; Ray-Chaudhury, Abhik; Lonser, Russell R.; Oldfield, Edward H.; Koretsky, Alan P.; Butman, John A.

2016-01-01

OBJECTIVE Pituitary MR imaging fails to detect over 50% of microadenomas in Cushing’s disease and nearly 80% of cases of dural microinvasion. Surface coils can generate exceptionally high-resolution images of the immediately adjacent tissues. To improve imaging of the pituitary gland, a receive-only surface coil that can be placed within the sphenoid sinus (the endosphenoidal coil [ESC]) during transsphenoidal surgery (TSS) was developed and assessed. METHODS Five cadaver heads were used for preclinical testing of the ESC. The ESC (a double-turn, 12-mm-diameter surface coil made from 1-mm-diameter copper wire) was developed to obtain images in a 1.5-T MR scanner. The ESC was placed (via a standard sublabial TSS approach) on the anterior sella face. Clinical MR scans were obtained using the 8-channel head coil and ESC as the receiver coils. Using the ESC, ultra–high-resolution, 3D, balanced fast field echo (BFFE) and T1-weighted imaging were performed at resolutions of 0.25 × 0.25 × 0.50 mm3 and 0.15 × 0.15 × 0.30 mm3, respectively. RESULTS Region-of-interest analysis indicated a 10-fold increase in the signal-to-noise ratio (SNR) of the pituitary when using the ESC compared with the 8-channel head coil. ESC-related improvements (p < 0.01) in the SNR were inversely proportional to the distance from the ESC tip to the anterior pituitary gland surface. High-resolution BFFE MR imaging obtained using ESC revealed a number of anatomical features critical to pituitary surgery that were not visible on 8-channel MR imaging, including the pituitary capsule, the intercavernous sinus, and microcalcifications in the pars intermedia. These ESC imaging findings were confirmed by the pathological correlation with whole-mount pituitary sections. CONCLUSIONS ESC can significantly improve SNR in the sellar region intraoperatively using current 1.5-T MR imaging platforms. Improvement in SNR can provide images of the sella and surrounding structures with unprecedented

Significant improvement of intractable headache after transsphenoidal surgery in patients with pituitary adenomas; preoperative neuroradiological evaluation and intraoperative intrasellar pressure measurement.

PubMed

Hayashi, Yasuhiko; Kita, Daisuke; Iwato, Masayuki; Fukui, Issei; Oishi, Masahiro; Tsutsui, Taishi; Tachibana, Osamu; Nakada, Mitsutoshi

2016-04-01

Headache is the most common symptom of both primary and metastatic brain tumor, and is generally considered the primary symptom in patients with large pituitary adenomas. However, patients with small pituitary adenomas rarely complain of intractable headache, and neurosurgeons are unsure whether such small adenomas actually contribute to headache. If conventional medical treatments for headache prove ineffective, surgical removal of the adenoma can be considered as an alternative management strategy. We conducted a retrospective review of 180 patients who underwent transsphenoidal surgery (TSS) for pituitary adenomas at Kanazawa University Hospital between 2006 and 2014. Patients with acute phase intratumoral hemorrhage were excluded. We identified nine patients with intractable headache as the chief complaint associated with small pituitary adenoma (diameters 15.8 ± 2.6 mm, 11-20 mm), non-functioning in eight, and prolactin-secreting in one. The preoperative neuroradiological studies and headache characteristics were assessed retrospectively, and the intrasellar pressure evaluation was performed during TSS in the last seven patients. All nine patients had complete or substantial resolution of their formerly intractable headache after TSS. Headaches consisted of ocular pain ipsilateral to the adenoma localization within the sella in four cases and bifrontal headache in five. Magnetic resonance imaging of these patients revealed small diaphragmatic foramen, which were so narrow that only the pituitary stalk could pass. Computed tomography scans showed ossification beneath the sellar floor in the sphenoid sinus, presellar type in six cases, and choncal type in three. The adenomas included cysts in seven cases. There was no cavernous sinus invasion. Intrasellar pressure measurements averaged 41.5 ± 8.5 mmHg, range 34-59, significantly higher than in control patients without headache (n = 12), namely 22.2 ± 10.6 mmHg (16-30). In this study, the authors demonstrated

Apparently new osteodysplastic and primordial short stature with severe microdontia, opalescent teeth, and rootless molars in two siblings.

PubMed

Kantaputra, Piranit N

2002-09-01

A Thai man and his sister affected with a newly recognized syndrome of proportionate primordial short stature are reported. The patients had severe intrauterine and postnatal growth retardation, prominent nose and nasal bridge, small pinnae, large sella turcica, areas of hypo- and hyperpigmentation of skin, dry and thin scalp hair, and long and straight clavicles. Ivory epiphyses and cone-shaped epiphyses of the hands were found when they were young, but most of them disappeared as they grew up. Scaphoid and trapezium had angular appearance. The second toes were unusually long. Distal symphalangism of toes and barchymesophalangy of fingers were noted. The findings that appear to distinguish this syndrome from the previously reported syndromes are long second toes, opalescent and rootless teeth, severe microdontia, severely hypoplastic alveolar process, and unerupted tooth. The mode of inheritance is suspected to be autosomal recessive. Copyright 2002 Wiley-Liss, Inc.

Cavernous sinus syndrome due to osteochondromatosis in a cat.

PubMed

Perazzi, Anna; Bernardini, Marco; Mandara, Maria T; De Benedictis, Giulia M; De Strobel, Francesca; Zotti, Alessandro

2013-12-01

A 1-year-old sexually intact male Korat cat was referred for ophthalmological consultation due to anisocoria. Mydriasis with external ophthalmoplegia and absence of pupillary light responses in the right eye and nasofacial hypalgesia were seen. Cavernous sinus syndrome (CSS) was suspected. Bilateral deformities of the jaw and phalangeal bones, severe spinal pain and abnormal conformation of the lumbar spine were also present. Radiographic examination revealed several mineralised masses in the appendicular and axial skeleton, indicative of multiple cartilaginous exostoses. For further investigation of the CSS-related neurological deficits, the cat underwent computed tomography (CT) examination of the skull. CT images revealed a non-vascularised, calcified, amorphous mass originating from the right lateral skull base and superimposing on the sella turcica. Based on the severity of diffuse lesions and owing to the clinical signs of extreme pain, the cat was euthanased. A diffuse skeletal and intracranial osteochondromatosis was diagnosed histologically.

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves.

PubMed

Kim, Soo Ryang; Kanda, Fumio; Kobessho, Hiroshi; Sugimoto, Koji; Matsuoka, Toshiyuki; Kudo, Masatoshi; Hayashi, Yoshitake

2006-11-07

We describe a rare case of HCV-related recurrent multiple hepatocellular carcinoma (HCC) metastasizing to the skull base involving multiple cranial nerves in a 50-year-old woman. The patient presented with symptoms of ptosis, fixation of the right eyeball, and left abducens palsy, indicating disturbances of the right oculomotor and trochlear nerves and bilateral abducens nerves. Brain contrast-enhanced computed tomography (CT) revealed an ill-defined mass with abnormal enhancement around the sella turcica. Brain magnetic resonance imaging (MRI) disclosed that the mass involved the clivus, cavernous sinus, and petrous apex. On contrast-enhanced MRI with gadolinium-chelated contrast medium, the mass showed inhomogeneous intermediate enhancement. The diagnosis of metastatic HCC to the skull base was made on the basis of neurological findings and imaging studies including CT and MRI, without histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.

Partial hypopituitarism and Langerhans cell histiocytosis

PubMed Central

Balaguruswamy, S; Chattington, P D

2011-01-01

A case of multisystem Langerhans cell histiocytosis with pituitary involvement nearly 20 years after initial presentation. A 48-year-old man had histiocytosis X 22 years ago initially involving the groin; subsequently his external auditory meatus, scalp, gum, mandibular bone, perineum and axilla were involved and treated. The pituitary gland was involved 4 years ago. A thyrotropin-releasing hormone test showed delayed response suggestive of hypothalamic disease. Prolactin levels were normal. A gonadotropin-releasing hormone test showed impaired testosterone and gonadotrophin response in keeping with pituitary disease. A glucagon stimulation test showed an impaired growth hormone response but a normal cortisol increase. MRI pituitary showed an empty sella. There was no evidence of diabetes insipidus. Bone mineral densitometry was normal. He has partial hypopituitarism needing thyroxine and testosterone replacement. He also developed type 2 diabetes mellitus 9 years ago. He is closely monitored for any development of diabetes insipidus and the need for growth hormone supplementation. PMID:22715201

Comparison of linear cephalometric dimensions in Americans of European descent (Ann Arbor, Cleveland, Philadelphia) and Americans of African descent (Nashville).

PubMed

Dibbets, Jos M H; Nolte, Kai

2002-08-01

Eleven dimensions, extracted from four commercially available cephalometric atlases were compared. Three populations were American of European descent and one was American of African descent. The source data were carefully corrected for linear enlargement. The confounding effect of linear radiographic enlargement is exemplified by depicting the often-used distance, sella-nasion, before and after correction. Total face height was smallest in the Cleveland population and largest in the Nashville population. The difference was fully accounted for by differences in lower face height and that was the most variable of all dimensions studied. Upper face height was almost identical in all four populations. Posterior face height was largest in the Nashville population. The mandible in the Nashville population had an average ramus height, but a longer corpus. Mandibular dimensions were equal in the three other populations. The maxilla was clearly shortest in the Cleveland population and almost of equal length in the three others.

Transsphenoidal pituitary resection with intraoperative MR guidance: preliminary results

NASA Astrophysics Data System (ADS)

Pergolizzi, Richard S., Jr.; Schwartz, Richard B.; Hsu, Liangge; Wong, Terence Z.; Black, Peter M.; Martin, Claudia; Jolesz, Ferenc A.

1999-05-01

The use of intraoperative MR image guidance has the potential to improve the precision, extent and safety of transsphenoidal pituitary resections. At Brigham and Women's Hospital, an open-bore configuration 0.5T MR system (SIGNA SP, GE Medical Systems, Milwaukee, WI) has been used to provide image guidance for nine transsphenoidal pituitary adenoma resections. The intraoperative MR system allowed the radiologist to direct the surgeon toward the sella turcica successfully while avoiding the cavernous sinus, optic chiasm and other sensitive structures. Imaging performed during the surgery monitored the extent of resection and allowed for removal of tumor beyond the surgeon's view in five cases. Dynamic MR imaging was used to distinguish residual tumor from normal gland and postoperative changes permitting more precise tumor localization. A heme-sensitive long TE gradient echo sequence was used to evaluate for the presence of hemorrhagic debris. All patients tolerated the procedure well without significant complications.

Construction and validation of the midsagittal reference plane based on the skull base symmetry for three-dimensional cephalometric craniofacial analysis.

PubMed

Kim, Hak-Jin; Kim, Bong Chul; Kim, Jin-Geun; Zhengguo, Piao; Kang, Sang Hoon; Lee, Sang-Hwy

2014-03-01

The objective of this study was to determine the reliable midsagittal (MS) reference plane in practical ways for the three-dimensional craniofacial analysis on three-dimensional computed tomography images. Five normal human dry skulls and 20 normal subjects without any dysmorphoses or asymmetries were used. The accuracies and stability on repeated plane construction for almost every possible candidate MS plane based on the skull base structures were examined by comparing the discrepancies in distances and orientations from the reference points and planes of the skull base and facial bones on three-dimensional computed tomography images. The following reference points of these planes were stable, and their distribution was balanced: nasion and foramen cecum at the anterior part of the skull base, sella at the middle part, and basion and opisthion at the posterior part. The candidate reference planes constructed using the aforementioned reference points were thought to be reliable for use as an MS reference plane for the three-dimensional analysis of maxillofacial dysmorphosis.

Unilateral external and internal ophthalmoplegia caused by intracranial meningioma in a dog.

PubMed

Larocca, R.D.

2000-01-01

Unilateral internal and external ophthalmoplegia caused by an intracranial meningioma occurred in a 15-year-old Belgian Sheepdog. The dog initially presented with ventro-lateral strabismus of the left eye, and ptosis of the left upper eyelid. Anisocoria was present with the left pupil fixed and dilated. Both eyes were visual. Neuro-ophthalmic evaluation revealed a lesion located in the left oculomotor nerve. Pharmacological testing with dilute pilocarpine (0.1% in artificial tears) revealed evidence of parasympathetic denervation of the left eye. Further evaluation via magnetic resonance imaging (MRI) revealed a well-defined mass to the left of midline and lateral to the sella turcica. An attempt was made to excise/debulk the mass due to worsening conditions and the dog died the following day. Necropsy revealed a mass of randomly arranged bundles and streams of spindle cells. Immunohistochemistry demonstrated a strong avidity for vimentin and a negative response for S-100 protein. These findings suggest a diagnosis of meningioma.

Orthodontic camouflage in the case of a skeletal class III malocclusion.

PubMed

Costa Pinho, Teresa M; Ustrell Torrent, Josep M; Correia Pinto, João G R

2004-01-01

To describe the clinical problem of a male patient, 15 years of age, who had a dolichofacial biotype and a Class III skeletal type at the beginning of treatment, manifesting itself at the dental level. To resolve the dental problems, orthodontic camouflage (dentoalveolar compensation) with the extraction of two mandibular premolars was performed. This procedure allowed a more harmonious occlusal relationship at the canine level and provided better occlusal stability of the final result. The procedure choice was based on the fact that some cephalometric values were favorable to attenuation of the skeletal Class III. For example, according to the analyses of Björk and Jarabak, these values are the total sum of 1, 2, 3 (sella angle, articular angle, and gonial angles) and the anterior/posterior facial height (S-Go/Na-Me). However, the same cephalometric data indicate a possible worsening of the existing open bite, which might be corrected with dental extractions and the use of intermaxillary elastics.

Rathke cleft cyst in seven-year-old girl presenting with central diabetes insipidus and review of literature.

PubMed

Evliyaoglu, Olcay; Evliyaoglu, Cetin; Ayva, Sebnem

2010-05-01

Rathke cleft cysts (RCC) are benign cysts derived from remnants of Rathke cleft, and are rarely symptomatic in children. Symptoms due to RCC are associated with mass effect and pituitary hormone deficiencies. Slow growth rate of the cyst makes its incidence increase with aging. Here we report on a seven-year-old girl who presented with central diabetes insipidus (CDI). Her sella MRI revealed a lesion in the sellar region which grew rapidly in follow-up. She underwent microneurosurgical operation and the lesion was totally excised. Pathologic examination revealed RCC with degenerative changes. In her follow-up, growth hormone deficiency developed in addition to arginine vasopressin deficiency. Rapid growth of the cyst is not the usual course of RCC's. Mechanisms regarding the cyst growth are unclear as they are in this case. This is the youngest child to date presenting with central diabetes insipidus due to RCC. Rapid growth of RCC can cause CDI in young children.

Trans-nasal-trans-sphenoidal brain injury by a fencing foil: an unusual case report and brief literature review.

PubMed

Özay, Rafet; Balkan, Mehmet S; Tönge, Çağhan; Şekerci, Zeki

2017-11-01

In this report, the authors present an unusual case of a 10-year-old child who suffered a severe headache and rhinorrhea that occurred as a result of fencing foil sports injury via trans-nasal-trans-sphenoidal (TNTS) pathway. Following trauma, the child had shown neurological symptoms such a pupil dilatation, change in consciousness and mild hemiparesia. Imaging demonstrated destruction of bone structures including posterior wall of sphenoid sinus and antero-superior part of sella turcica, and also a contusion at right thalamic region. For treatment of rhinorrhea lumbar drainage system (LDS) had planted in order to relieve cerebrospinal fluid (CSF) leakage. After the treatment, the patient had fully recovered without any need of further surgical intervention. CSF leakage had prevented and neurological symptoms were completely treated. This case represents the first report of brain injury via TNTS pathway in a sports practice. Diagnosis, clinic follow-up and treatment options of this rare accidental sports injury are discussed.

Autoimmune hyperthyroidism due to secondary adrenal insufficiency: resolution with glucocorticoids.

PubMed

Skamagas, Maria; Geer, Eliza B

2011-01-01

To describe the course of autoimmune hyperthyroid disease in a patient with corticotropin (ACTH) deficiency treated with glucocorticoids. We report the clinical presentation, laboratory data, imaging studies, and management of a patient with weight loss, fatigue, apathy, hallucinations, and arthritis. Autoimmune hyperthyroidism (positive thyroperoxidase and thyroglobulin antibodies and borderline positive thyrotropin receptor antibody) was diagnosed in a 71-year-old woman. New psychotic symptoms prompted brain magnetic resonance imaging, which revealed a partially empty sella. Undetectable morning cortisol, undetectable ACTH, and failure to stimulate cortisol with synthetic ACTH (cosyntropin 250 mcg) secured the diagnosis of long-standing secondary adrenal insufficiency. Hydrocortisone replacement improved the patient's symptoms, resolved the thyroid disease, and decreased thyroid antibody titers. In retrospect, the patient recalled severe postpartum hemorrhage requiring blood transfusion at age 38 years. A Sheehan event probably occurred 33 years before the patient presented with corticotropin deficiency. Hyperthyroidism accelerated cortisol metabolism and provoked symptoms of adrenal insufficiency. The hypocortisolemic state may precipitate hyperimmunity and autoimmune thyroid disease. Rapid resolution of hyperthyroidism and decreased thyroid antibody titers with glucocorticoid treatment support this hypothesis.

A Histologically Diagnosed Case with Infantile Osteopetrosis Complicated by Hypopituitarism

PubMed Central

Calkavur, Sebnem; Buyukinan, Muammer; Altay, Canan

2015-01-01

Malignant infantile osteopetrosis is a rarely seen severe disorder which appears early in life with general sclerosis of the skeleton. It is caused by functionally defective osteoclasts which fail to resorb bone. Affected infants can exhibit a wide spectrum of clinical manifestations including impaired hematopoiesis, hepatosplenomegaly, visual impairment, and hypocalcemia. With the exception of secondary hyperparathyroidism, involvement of the endocrine system seems to be quite rare. Hypopituitarism is defined as underproduction of the growth hormone in combination with deficiencies of other pituitary hormones. Any lesion that damages hypothalamus, pituitary stalk, or anterior pituitary can cause secondary hypopituitarism. In this report, we presented a rare combination of malignant infantile osteopetrosis and secondary hypopituitarism in a newborn who presented predominantly with endocrinological symptoms. This is the first case report of malignant infantile osteopetrosis accompanied by hypopituitarism secondary to sclerosis of the sella turcica. On the other hand, this is a very interesting case which was diagnosed based on histological examination of bone marrow biopsy specimens despite lack of any clinical suspicion. PMID:26576309

Coexistence of sporadic cerebellar hemangioblastoma and pituitary null cell adenoma: simultaneous expression of von Hippel-Lindau gene product. Case report.

PubMed

Shimoda, Yoshiteru; Ogawa, Yoshikazu; Endo, Hidenori; Watanabe, Mika; Tominaga, Teiji

2012-01-01

Coexistence of brain tumors of different pathologies is rare, and the majority of the cases were related to genetic disorders or secondary tumors occurring after radiotherapy. A 73-year-old man was introduced to the outpatient department suffering from severe nausea and vertigo. Magnetic resonance imaging showed a cystic tumor in the left cerebellar hemisphere and another lesion in the sella turcica. There was no evident family history of von Hippel-Lindau (VHL) disease, and the systemic investigation failed to detect any other tumors or signs of VHL disease. Treatment was performed in two stages, and he was discharged with remaining slight ataxic gait. The diagnoses were cerebellar hemangioblastoma and pituitary null cell adenoma. Additional immunohistochemical investigation using VHL disease gene-related protein in both tumors showed minute granular positive staining in the cytoplasm of stromal cells in the former, and diffuse and strong granular cytoplasmic positive staining in the latter. Further analysis is required to confirm the true implication of the VHL gene mutation, and the possible involvement of VHL gene-related protein in the pathogenesis of these coexisting tumors.

A case of sibutramine-induced hyperprolactinemia.

PubMed

Soares Leaes, Carolina Garcia; Pereira-Lima, Júlia Fernanda Semmelmann; da Costa Oliveira, Miriam

2011-01-01

Several drugs may cause hyperprolactinemia, especially antipsychotic drugs and prokynetic drugs. Serum prolactin concentrations increase within hours after acute administration of these drugs and return to normal within two to four days after cessation of chronic therapy. So far, sibutramine, a sympathomimetic drug used in the management of obesity, was not described to be associated with altered prolactin levels. The purpose of this study is to present a case of sibutramine-induced hiperprolactinemia. A 38-year-old white female patient seeks medical attention complaining of weight gain (Body mass index: 35) associated with anxiety. She started sibutramine treatment and presented with amenogalactorrhea. Hyperprolactinemia was diagnosed (prolactin of 46 and 89.6 ng/mL) with normal thyroid, renal and hepatic function, and a negative pregnancy test. A sella MRI was performed and sibutramine was suspended. Prolactin levels returned to normal within 15 days of sibutramine cessation and remained normal within 90 days of follow-up, with resolution of the amenogalactorrhea syndrome. sibutramine may be considered in differential diagnosis of drug-induced hyperprolactinemia.

Metastatic melanoma to the pituitary gland.

PubMed

McCutcheon, Ian E; Waguespack, Steven G; Fuller, Gregory N; Couldwell, William T

2007-08-01

Metastasis to the pituitary gland is unusual, and occurs most often in patients with carcinomas of the breast or lung. Despite its propensity for spread to the brain, metastatic melanoma has rarely been described within the sella. We report two cases of malignant melanoma pathologically confirmed within the pituitary, both metastatic from a primary site on the chest wall. In each patient, transsphenoidal resection of the tumor was incomplete and each received local radiotherapy after surgery. One patient recurred quickly and developed brain metastasis as well. He died four months after resection of the pituitary metastasis, but the second patient survived six months without recurrence. As intrasellar metastasis portends widespread systemic disease and may be synchronous with parenchymal brain metastasis, survival in such patients is limited regardless of adjunctive therapy. Such cases are likely to arise more commonly in future due to the increasing incidence of melanoma. Identifying them by imaging alone is difficult due to inconsistent signal characteristics on MRI (as shown by these cases) and the confusion introduced by any associated intratumoral hemorrhage.

Cerebrospinal meningitis in a 30 -year -old patient as first manifestation of pituitary macroadenoma.

PubMed

Andrysiak -Mamos, Elżbieta; Żochowska, Ewa; Kaźmierczyk -Puchalska, Agnieszka; Sagan, Leszek; Sowińska -Przepiera, Elżbieta; Zając -Marczewska, Małgorzata; Kojder, Ireneusz; Syrenicz, Anhelli

The most common clinical and neurological signs and symptoms of pituitary macroadenomas include headache, vision impairment and cranial nerve palsy. The patient presented in this article was admitted to the Intensive Care Unit at regional hospital; at admission, the patient was unconscious, he had convulsions and spasms, and a 3 -day history of headache and body temperature up to 41.5°C. The patient with suspected neuroinfection was transferred to the Department of Infectious Diseases of the Pomeranian Medical University in Szczecin (PMU), where cerebrospinal meningitis of bacterial etiology was established based on cerebrospinal fluid investigations and the presence of pituitary abscess was suggested based on magnetic resonance imaging (MRI). Magnetic resonance imaging findings included an extensive pathological lesion with the diameter of 27 × 28 × 38 mm located in the sellar-suprasellar region, with intensive peripheral contrast enhancement. The lesion protrudes into the sphenoid sinus through the lowered bottom of sella turcica and the fluid content has also been visualized in the sphenoid sinus. After 10 -day antibiotic therapy, the patient was transferred to neurosurgery ward for surgical treatment. The pathological lesion was partially evacuated during right frontotemporal craniotomy. The patient’s general condition after the surgery was moderately severe; the patient was conscious, able to follow simple commands, presenting hemiparesis of the left side of the body, particularly affecting left lower limb and with speech disturbances. The signs of hypopituitarism affecting all hormonal axes were also observed and the patient was transferred to the Department of Endocrinology of the PMU for further treatment. Follow -up MRI scan continued to show the presence of pathological mass in the sellar -suprasellar region, which penetrated into the sphenoid sinus through damaged sellar bottom. After correction of reduced hormone levels and several weeks of

Blockade of arginine vasotocin signaling reduces aggressive behavior and c-Fos expression in the preoptic area and periventricular nucleus of the posterior tuberculum in male Amphiprion ocellaris.

PubMed

Yaeger, C; Ros, A M; Cross, V; Deangelis, R S; Stobaugh, D J; Rhodes, J S

2014-05-16

Many marine fishes change sex in response to social cues when the dominance hierarchy is perturbed. Arginine-vasotocin (AVT) and the mammalian homolog arginine vasopressin are neuropeptides involved in social and reproductive behaviors across vertebrate taxa. The goal of this study was to determine whether AVT signaling influences aggression and expression of c-Fos, a marker of neuroplasticity, in key brain regions of the social decision circuit in Amphiprion ocellaris clownfish, a species where behavioral dominance precedes gonadal sex change from male to female. In experiment 1, juvenile clownfish (average mass 2.5g) were paired together in a tank (a total of 24 pairs), matched approximately for size with one fish randomly receiving either an intraperitoneal injection of the arginine vasopressin V1a receptor antagonist (Manning compound) or saline vehicle, and evaluated for aggressive and submissive behaviors over a 10-min period. The second experiment was a repeat of the first using five pairs of mature, reproductive males, except the animals interacted for 90-min immediately followed by euthanasia for immunohistochemical detection of c-Fos protein. Numbers of c-Fos-positive cells were quantified in the preoptic area of the hypothalamus (POA), the anterior tuberal nucleus (aTn), and periventricular nucleus of the posterior tuberculum (TPp). Manning compound significantly reduced aggression and the probability of winning the contest relative to saline (vehicle) controls. In experiment 2, saline-treated fish displayed approximately twice as many c-Fos-positive cells in the POA and 25% more in the TPp than the Manning-treated fish, no differences were observed in the aTn. Taken together, results suggest AVT signaling is necessary for aggressive behavior and expression of neuroplasticity in the POA and TPp that likely contributes to behavioral dominance and hence, sex change in A. ocellaris. Published by Elsevier Ltd.

Surgical outcomes after reoperation for recurrent skull base meningiomas.

PubMed

Magill, Stephen T; Lee, David S; Yen, Adam J; Lucas, Calixto-Hope G; Raleigh, David R; Aghi, Manish K; Theodosopoulos, Philip V; McDermott, Michael W

2018-05-04

OBJECTIVE Skull base meningiomas are surgically challenging tumors due to the intricate skull base anatomy and the proximity of cranial nerves and critical cerebral vasculature. Many studies have reported outcomes after primary resection of skull base meningiomas; however, little is known about outcomes after reoperation for recurrent skull base meningiomas. Since reoperation is one treatment option for patients with recurrent meningioma, the authors sought to define the risk profile for reoperation of skull base meningiomas. METHODS A retrospective review of 2120 patients who underwent resection of meningiomas between 1985 and 2016 was conducted. Clinical information was extracted from the medical records, radiology data, and pathology data. All records of patients with recurrent skull base meningiomas were reviewed. Demographic data, presenting symptoms, surgical management, outcomes, and complications data were collected. Kaplan-Meier analysis was used to evaluate survival after reoperation. Logistic regression was used to evaluate for risk factors associated with complications. RESULTS Seventy-eight patients underwent 100 reoperations for recurrent skull base meningiomas. Seventeen patients had 2 reoperations, 3 had 3 reoperations, and 2 had 4 or more reoperations. The median age at diagnosis was 52 years, and 64% of patients were female. The median follow-up was 8.5 years. Presenting symptoms included cranial neuropathy, headache, seizure, proptosis, and weakness. The median time from initial resection to first reoperation was 4.4 years and 4.1 years from first to second reoperation. Seventy-two percent of tumors were WHO grade I, 22% were WHO grade II, and 6% were WHO grade III. The sphenoid wing was the most common location (31%), followed by cerebellopontine angle (14%), cavernous sinus (13%), olfactory groove (12%), tuberculum sellae (12%), and middle fossa floor (5%). Forty-four (54%) tumors were ≥ 3 cm in maximum diameter at the time of the first

A rare case of autoimmune hypophysitis presenting as temperature dysregulation.

PubMed

Jain, Ankur; Dhanwal, Dinesh K

2015-02-01

Autoimmune hypophysitis is a rare cause of pan-hypopituitarism. Hypothalamic involvement in autoimmune hypophysitis is rare and usually manifests as central diabetes insipidus due to antibodies against arginine vasopressin. Temperature dysregulation is occasionally seen with suprasellar tumours but has never been reported with hypophysitis. We report a case of a middle aged man who presented to us with the complaints of documented body temperature fluctuations since two months followed gradually by hoarseness of voice, sexual dysfunction and syncope. Examination was remarkable for postural hypotension, dry coarse skin and delayed ankle reflexes. Patient's hormone profile revealed pan-hypopituitarism and elevated titre of anti-TPO antibodies. Patient's work up for secondary causes of hypopituitarism was negative. MRI brain revealed typical findings of hypophysitis. Patient was suspected as a case of autoimmune hypophysitis and was immediately treated with prednisolone along with hormone replacement. Rapid response within 30 days was observed in the form of subsidence of temperature fluctuations, improvement in general well being, sexual function and repeat MRI done after one month which revealed a partial empty sella. Autoimmune hypophysitis as a potentially treatable cause of temperature dysregulation has been highlighted in this case.

The endonasal endoscopic harvest and anatomy of the buccal fat pad flap for closure of skull base defects.

PubMed

Markey, Jeff; Benet, Arnau; El-Sayed, Ivan H

2015-10-01

Extirpation via expanded endonasal approaches (EEA) to the skull base can result in defects requiring vascularized rotational flap reconstruction. The buccal fat pad (BFP) is a vascularized graft described in open skull base resections, but its harvest and adequacy of vascular supply have not been examined for use with EEA. A transfacial cadaveric dissection was carried forth in a latex-injected specimen to characterize the BFP blood supply. Then a cadaveric dissection series was performed involving the endoscopic harvest and rotation of 10 buccal fat pads in five cadaveric specimens to assess defect coverage. An endoscopic medial maxillectomy combined with an anterior maxillotomy was performed prior to endoscopic harvest in cadaveric specimens. The BFP was rotated to assess its capability to reconstruct seven possible ventral skull base defects. Finally, the BFP vascular anatomy was further characterized following harvest and transposition. The BFP reconstructed defects at the greater sphenoid wing, inferior and superior clivus, sella, planum, and bilateral ethmoids in all cadaveric specimens. In some cases it covered two sites concurrently. The BFP pedicled rotational flap is a potential alternate flap following EEA in select cases. NA © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

A Rare Case of Autoimmune Hypophysitis Presenting as Temperature Dysregulation

PubMed Central

Dhanwal, Dinesh K.

2015-01-01

Autoimmune hypophysitis is a rare cause of pan-hypopituitarism. Hypothalamic involvement in autoimmune hypophysitis is rare and usually manifests as central diabetes insipidus due to antibodies against arginine vasopressin. Temperature dysregulation is occasionally seen with suprasellar tumours but has never been reported with hypophysitis. We report a case of a middle aged man who presented to us with the complaints of documented body temperature fluctuations since two months followed gradually by hoarseness of voice, sexual dysfunction and syncope. Examination was remarkable for postural hypotension, dry coarse skin and delayed ankle reflexes. Patient’s hormone profile revealed pan-hypopituitarism and elevated titre of anti-TPO antibodies. Patient’s work up for secondary causes of hypopituitarism was negative. MRI brain revealed typical findings of hypophysitis. Patient was suspected as a case of autoimmune hypophysitis and was immediately treated with prednisolone along with hormone replacement. Rapid response within 30 days was observed in the form of subsidence of temperature fluctuations, improvement in general well being, sexual function and repeat MRI done after one month which revealed a partial empty sella. Autoimmune hypophysitis as a potentially treatable cause of temperature dysregulation has been highlighted in this case. PMID:25859485

Mandibular dimensional changes and skeletal maturity.

PubMed

Subramaniam, Priya; Naidu, Premila

2010-10-01

Growth and development of the human face provides a fascinating interplay of form and function. Among the various facial bones, the mandible plays a very important role during various growth-modification therapies. These treatment modalities will yield a better result in less time if properly correlated with skeletal maturity. It is very essential to know where the site of growth occurs and also the time when it occurs or ceases to occur. This study was conducted to assess the mandibular dimensions at various stages of skeletal maturation. The subjects included 6 to 18-year-old children who were grouped according to their middle phalanx of the third finger stages of skeletal maturity. Lateral cephalographs were taken and, from their cephalometric tracings, linear and angular measurements of the mandible were made. The values obtained were subjected to statistical analysis. Results showed that the mandibular height, length and symphysis thickness increased with skeletal maturity. An increase in angles SNB (Sella, Nasion, Supramentale) and L1-MP (Long axis lower incisors- Mandibular plane) and a decrease in the gonial angle and ANB (Subspinale, Nasion, Supramentale) angle were observed. The study showed a significant correlation between mandibular growth and skeletal maturity.

Multiple Fractures in Patient with Graves' Disease Accompanied by Isolated Hypogonadotropic Hypogonadism.

PubMed

Yi, Hyon-Seung; Kim, Ji Min; Ju, Sang Hyeon; Lee, Younghak; Kim, Hyun Jin; Kim, Koon Soon

2016-02-01

Isolated hypogonadotropic hypogonadism (IHH) is known to decrease bone mineral density due to deficiency of sex steroid hormone. Graves' disease is also an important cause of secondary osteoporosis. However, IHH does not preclude the development of primary hyperthyroidism caused by Graves' disease, leading to more severe osteoporosis rapidly. Here, we describe the first case of 35-year-old Asian female patient with IHH accompanied by Graves' disease and osteoporosis-induced multiple fractures. Endocrine laboratory findings revealed preserved anterior pituitary functions except for secretion of gonadotropins and showed primary hyperthyroidism with positive autoantibodies. Sella magnetic resonance imaging showed slightly small sized pituitary gland without mass lesion. Dual energy X-ray absorptiometry revealed severe osteoporosis in lumbar spine and femur neck of the patient. Plain film radiography of the pelvis and shoulder revealed a displaced and nondisplaced fracture, respectively. After surgical fixation with screws for the femoral fracture, the patient was treated with antithyroid medication, calcium, and vitamin D until now and has been recovering fairly well. We report a patient of IHH with Graves' disease and multiple fractures that is a first case in Korea.

Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature.

PubMed

Manjila, Sunil; Miller, Erin A; Vadera, Sumeet; Goel, Rishi K; Khan, Fahd R; Crowe, Carol; Geertman, Robert T

2012-01-01

Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case. We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging. The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature

PubMed Central

Manjila, Sunil; Miller, Erin A.; Vadera, Sumeet; Goel, Rishi K.; Khan, Fahd R.; Crowe, Carol; Geertman, Robert T.

2012-01-01

Background: Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case. Case Description: We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging. Conclusion: The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome. PMID:22439114

Detection of transgenerational barium dual-isotope marks in salmon otoliths by means of LA-ICP-MS.

PubMed

Huelga-Suarez, Gonzalo; Fernández, Beatriz; Moldovan, Mariella; García Alonso, J Ignacio

2013-03-01

The present study evaluates the use of an individual-specific transgenerational barium dual-isotope procedure and its application to salmon specimens from the Sella River (Asturias, Spain). For such a purpose, the use of laser ablation inductively coupled plasma mass spectrometry (LA-ICP-MS) in combination with multiple linear regression for the determination of the isotopic mark in the otoliths of the specimens is presented. In this sense, a solution in which two barium-enriched isotopes ((137)Ba and (135)Ba) were mixed at a molar ratio of ca. 1:3 (N Ba137/N Ba135) was administered to eight returning females caught during the spawning period. After injection, these females, as well as their offspring, were reared in a governmental hatchery located in the council of Cangas de Onís (Asturias, Spain). For comparison purposes, as well as for a time-monitoring control, egg and larva data obtained by solution analysis ICP-MS are also given. Otoliths (9-month-old juveniles) of marked offspring were analysed by LA-ICP-MS demonstrating a 100 % marking efficacy of this methodology. The capabilities of the molar fraction approach for 2D imaging of fish otoliths are also addressed.

[Euclid and Pythagorus in the 21st century. A proposal on various harmonious craniofacial and occlusal constants].

PubMed

Blocquel, H

2001-01-01

The aim of this work was to study the architectural relationships between different squeletal and dental pieces participating in the mastication, and to prove their fixity. We have at our disposition a collection of teleradiographies taken from profile on the same subject, over the course of several years; 3000 of them have been selected. With the help of tracings, where are drawn 36 degrees angles; or 72 degrees and 108 degrees; we systematize our locations. Together with our ten experimenters, we have noticed the existence of a parallelism between the graphic representations of the Bouvet occlusal plane, the sphenoïdal planum (sphenoïdale bone) and the course of the second trigeminal branch (trigeminus nerve) in its infra-orbital section. The angle of those different marks with the superior clivus (quadrilateral strip of the sphenoïd) (dorsum sellae) still measures 72 degrees. It is equal to 108 degrees with the graphic representation of the third antero-inferior pterygoïd process. (pterygoïdeus process). The angle between the superior clivus and the pterygoïd process still measures 36 degrees. These works are new contributions to the orthodontic, occlusodontic or prosthetic conceptions.

Numerical study of the effect of the noseleaf on biosonar beamforming in a horseshoe bat.

PubMed

Zhuang, Qiao; Müller, Rolf

2007-11-01

Around 300 bat species are known to emit their ultrasonic biosonar pulses through the nostrils. This nasal emission coincides with the presence of intricately shaped baffle structures surrounding the nostrils. Some prior experimental evidence indicates that these "noseleaves" have an effect on the shape of the animals' radiation patterns. Here, we present a numerical acoustical analysis of the noseleaf of a horseshoe bat species. We show that all three distinctive parts of its noseleaf ("lancet," "sella," "anterior leaf") have an effect on the acoustic near field as well as on the directivity pattern. Furthermore, we show that furrows in one of the parts (the lancet) also exert such an influence. The underlying physical mechanisms suggested by the properties of the estimated near field are cavity resonance, as well as reflection and shadowing of the sound waves emitted by the nostrils. In their effects on the near field, the noseleaf parts showed a tendency toward spatial partitioning with the effects due to each part dominating a certain region. However, interactions between the acoustic effects of the parts were also evident, most notably, a synergism between two frequency-dependent effects (cavity resonance and shadowing) to produce an even stronger frequency selectivity.

Radiological features for the approach in trans-sphenoidal pituitary surgery.

PubMed

Twigg, Victoria; Carr, Simon D; Balakumar, Ramkishan; Sinha, Saurabh; Mirza, Showkat

2017-08-01

In order to perform trans-sphenoidal endoscopic pituitary surgery safely and efficiently it is important to identify anatomical and pituitary disease features on the pre-operative CT and MRI scans; thereby minimising the risk to surrounding structures and optimising outcomes. We aim to create a checklist to streamline pre-operative planning. We retrospectively reviewed pre-operative CT and MRI scans of 100 adults undergoing trans-sphenoidal endoscopic pituitary surgery. Radiological findings and their incidence included deviated nasal septum (62%), concha bullosa (32%), bony dehiscence of the carotid arteries (18%), sphenoid septation overlying the internal carotid artery (24% at the sella) and low lying CSF (32%). The mean distance of the sphenoid ostium to the skull base was 10 mm (range 2.7-17.6 mm). We also describe the 'teddy bear' sign which when present on an axial CT indicates the carotid arteries will be identifiable intra-operatively. There are significant variations in the anatomical and pituitary disease features between patients. We describe a number of features on pre-operative scans and have devised a checklist including a new 'teddy bear' sign to aid the surgeon in the anatomical assessment of patients undergoing trans-sphenoidal pituitary surgery.

Robotic Anterior and Midline Skull Base Surgery: Preclinical Investigations

DOE Office of Scientific and Technical Information (OSTI.GOV)

O'Malley, Bert W.; Weinstein, Gregory S.

Purpose: To develop a minimally invasive surgical technique to access the midline and anterior skull base using the optical and technical advantages of robotic surgical instrumentation. Methods and Materials: Ten experimental procedures focusing on approaches to the nasopharynx, clivus, sphenoid, pituitary sella, and suprasellar regions were performed on one cadaver and one live mongrel dog. Both the cadaver and canine procedures were performed in an approved training facility using the da Vinci Surgical Robot. For the canine experiments, a transoral robotic surgery (TORS) approach was used, and for the cadaver a newly developed combined cervical-transoral robotic surgery (C-TORS) approach wasmore » investigated and compared with standard TORS. The ability to access and dissect tissues within the various areas of the midline and anterior skull base were evaluated, and techniques to enhance visualization and instrumentation were developed. Results: Standard TORS approaches did not provide adequate access to the midline and anterior skull base; however, the newly developed C-TORS approach was successful in providing the surgical access to these regions of the skull base. Conclusion: Robotic surgery is an exciting minimally invasive approach to the skull base that warrants continued preclinical investigation and development.« less

Skeletal manifestations of juvenile hypothyroidism and the impact of treatment on skeletal system.

PubMed

Gutch, Manish; Philip, Rajeev; Philip, Renjit; Toms, Ajit; Saran, Sanjay; Gupta, K K

2013-10-01

Thyroid hormone mediates growth and development of the skeleton through its direct effects and through its permissive effects on growth hormone. The effect of hypothyroidism on bone is well described in congenital hypothyroidism, but the impact of thyroid hormone deficiency on a growing skeleton, as it happens with juvenile hypothyroidism, is less defined. In addition, the extent to which the skeletal defects of juvenile hypothyroidism revert on the replacement of thyroid hormone is not known. A study was undertaken in 29 juvenile autoimmune hypothyroid patients to study the skeletal manifestations of juvenile hypothyroidism and the impact of treatment of hypothyroidism on the skeletal system of juvenile patients. Hypothyroidism has a profound impact on the skeletal system and delayed bone age, dwarfism, and thickened bands at the metaphyseal ends being the most common findings. Post treatment, skeletal findings like delayed bone age and dwarfism improved significantly, but there were no significant changes in enlargement of sella, presence of wormian bones, epihyseal dysgenesis, vertebral changes and thickened band at the metaphyseal ends. With the treatment of hypothyroidism, there is an exuberant advancement of bone age, the catch up of bone age being approximately double of the chronological age advancement.

Suppression of Tinnitus in a Patient with Unilateral Sudden Hearing Loss: A Case Report

PubMed Central

Fioretti, Alessandra; Peri, Giorgia; Eibenstein, Alberto

2012-01-01

We describe a case of a 67-year-old woman with severe disabling right-sided tinnitus, mild hyperacusis, and headache. The tinnitus was associated with sudden right hearing loss and vertigo, which occurred about 18 months before. Magnetic resonance imaging (MRI) resulted in normal anatomical structures of the cochlea and of the cranial nerves showing a partial empty sella syndrome with suprasellar cistern hernia. Angio-MR revealed a bilateral contact between the anterior-inferior cerebellar artery (AICA) and the acoustic-facial nerve with a potential neurovascular conflict. Surgery was considered unnecessary after further evaluations. The right ear was successfully treated with a combination device (hearing aid plus sound generator). Shortly after a standard fitting procedure, the patient reported a reduction of tinnitus, hyperacusis, and headache which completely disappeared at the follow-up evaluation after 3, 6, and 12 months. This paper demonstrates that the combination device resulted in a complete tinnitus and hyperacusis suppression in a patient with unilateral sensorineural sudden hearing loss. Our paper further supports the restoration of peripheral sensory input for the treatment of tinnitus associated with hearing loss in selected patients. PMID:23227400

Eruption of the permanent maxillary canines in relation to mandibular second molar maturity.

PubMed

Perinetti, Giuseppe; Callovi, Marilena; Salgarello, Stefano; Biasotto, Matteo; Contardo, Luca

2013-07-01

To evaluate the timing of spontaneous maxillary canine eruption in relation to stages of mandibular second molar maturation. Potential confounding effects from such factors as age, growth phase, and facial features were also explored. A sample of 106 healthy subjects (48 females and 58 males; age range, 9.4-14.3 years) with both permanent maxillary canines during the final phase of intraoral eruption were included. Mandibular second molar maturation (stages E to H) was assessed according to the method of Demirjian. Skeletal maturity was determined using the cervical vertebral maturational (CVM) method. Facial vertical and sagittal relationships were evaluated by recording the Sella-Nasion/mandibular plane (SN/MP) angle and the ANB angle. An ordered multiple logistic regression was run to evaluate adjusted correlation of each parameter with the mandibular second molar maturational stage. Overall, the prevalence of the different second molar maturational stages was 36.8%, 37.8%, and 27.4% for stages E, F and G, respectively. According to the regression model, this relation was not influenced by sex, CVM stage, SN/MP angle, and ANB angle. Irrespective of sex, growth phase, and facial features, the maturational stage of the mandibular second molar may be a reliable indicator for the timing of spontaneous eruption of the maxillary canine.

Pituitary tumour causing gigantism. Morphology and in vitro hormone secretion.

PubMed

Anniko, M; Ritzén, E M

1986-01-01

True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in a 13-year-old boy. The clinical history indicated that the tumour had caused an oversecretion of GH since the age of 4-5 years. At diagnosis, the sella turcica was markedly enlarged. No infiltrative growth was noted at surgery. Endocrine investigations showed elevated GH and PRL secretion. Light and electron microscopy of tumour tissue revealed densely packed pleomorphic cells of both GH and PRL type. In addition, oncocyte-like cells were observed. Organ culture of pieces of tumour tissue demonstrated continued secretion of GH and PRL into the medium for more than 5 days in vitro. Addition of bromocriptine to the medium caused a rapid decline in PRL secretion while GH secretion remained the same. X-ray irradiation in vitro also caused a decrease in PRL secretion. These effects of bromocriptine and X-ray on hormone secretion in vitro mirrored the corresponding effect of treatment, when the patient showed signs of tumour recurrence after pituitary surgery. It is concluded that also in childhood, the in vitro response of tumour tissue to various treatments may be explored as a possible way to predict the efficacy of pharmacological or irradiation treatment of pituitary tumours.

A Purely Endoscopic and Simultaneous Transsphenoidal and Transcranial Keyhole Approach for Giant Pituitary Adenoma Resection: A Technical Case Report

PubMed Central

Takeuchi, Kazuhito; Nagatani, Tetsuya; Watanabe, Tadashi; Okumura, Eriko; Sato, Yusuke; Wakabayashi, Toshihiko

2015-01-01

A combined transsphenoidal-transcranial approach for the resection of pituitary adenomas has previously been reported. While this approach is useful for specific types of pituitary adenomas, it is an invasive technique. To reduce the invasiveness of this approach, we adopted the keyhole concept for pituitary adenoma resection. A 23-year-old man presented at a local hospital with a 6-month history of bilateral hemianopia. Magnetic resonance imaging revealed a large pituitary adenoma extending from the sella turcica toward the right frontal lobe. Endoscopic transsphenoidal surgery was planned at a local hospital; however, the operation was abandoned at the start of the resection because of the firm and fibrous nature of the tumor. The patient was subsequently referred to our hospital for additional surgery. The tumor was removed purely endoscopically via a transsphenoidal and transcranial route. Keyhole craniotomy, 3 cm in diameter, was performed, and a tubular retractor was used to achieve a wider surgical corridor; this enabled better visualization and dissection from the surrounding brain and provided enough room for the use of surgical instruments under endoscopic view. The tumor was successfully removed without complication. This is the first case report to describe the resection of a giant pituitary adenoma using a purely endoscopic and simultaneous transsphenoidal and transcranial keyhole approach. PMID:28663976

Upper Nasopharyngeal Corridor for Transnasal Endoscopic Drainage of Petroclival Cholesterol Granulomas: Alternative Access in Conchal Sphenoid Patients.

PubMed

Turan, Nefize; Baum, Griffin R; Holland, Christopher M; Ahmad, Faiz U; Henriquez, Oswaldo A; Pradilla, Gustavo

2016-03-01

Background Cholesterol granulomas arising at the petrous apex can be treated via traditional open surgical, endoscopic, and endoscopic-assisted approaches. Endoscopic approaches require access to the sphenoid sinus, which is technically challenging in patients with conchal sphenoidal anatomy. Clinical Presentation A 55-year-old woman presented with intermittent headaches and tinnitus. Formal audiometry demonstrated moderately severe bilateral hearing loss. CT of the temporal bones and sella revealed a well-demarcated expansile lytic mass. MRI of the face, orbit, and neck showed a right petrous apex mass measuring 22 × 18 × 19 mm that was hyperintense on T1- and T2-weighted images without enhancement, consistent with a cholesterol granuloma. The patient had a conchal sphenoidal anatomy. Operative Technique Herein, we present an illustrative case of a low-lying petroclival cholesterol granuloma in a patient with conchal sphenoidal anatomy to describe an alternative high nasopharyngeal corridor for endoscopic transnasal transclival access. Postoperative Course Postoperatively, the patient's symptoms recovered and no complications occurred. Follow-up imaging demonstrated a patent drainage tract without evidence of recurrence. Conclusion In patients with a conchal sphenoid sinus, endoscopic transnasal transclival access can be gained using a high nasopharyngeal approach. This corridor facilitates safe access to these lesions and others in this location.

Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects

PubMed Central

Julayanont, Parunyou; Karukote, Amputch; Ruthirago, Doungporn; Panikkath, Deepa; Panikkath, Ragesh

2016-01-01

Idiopathic intracranial hypertension (IIH) is an uncommon disorder characterized by increased intracranial pressure without radiological or laboratory evidence of intracranial pathology except empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. Persistent headache is the most common symptom. Visual impairment is a serious complication that may not be recognized by the patients. This paper reviews clinical manifestations, diagnostic challenges, and current treatments of IIH in adults. Various imaging modalities have been studied on their validity for detection of IIH and papilledema. This review also includes new studies on medical, surgical, and interventional management of this condition. Acetazolamide and topiramate are the only two medications that have been studied in randomized controlled trials about their efficacy in treatment of IIH. In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. The efficacy and complications of cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in the last 3 decades have been summarized in this review. Finally, the prospective aspects of biomarkers and treatments are proposed for future research. PMID:26929666

Dynamics of biosonar systems in Horseshoe bats

NASA Astrophysics Data System (ADS)

Müller, R.

2015-12-01

Horseshoe bats have an active ultrasonic sonar system that allows the animals to navigate and hunt prey in structure-rich natural environments. The physical components of this biosonar system contain an unusual dynamics that could play a key role in achieving the animals' superior sensory performance. Horseshoe bat biosonar employs elaborate baffle shapes to diffract the outgoing and incoming ultrasonic wave packets; ultrasound is radiated from nostrils that are surrounded by noseleaves and received by large outer ears. Noseleaves and pinnae can be actuated while ultrasonic diffraction takes place. On the emission side, two noseleaf parts, the anterior leaf and the sella, have been shown to be in motion in synchrony with sound emission. On the reception side, the pinnae have been shown to change their shapes by up to 20% of their total length within ˜100 milliseconds. Due to these shape changes, diffraction of the incoming and outgoing waves is turned into a dynamic physical process. The dynamics of the diffraction process results in likewise dynamic device characteristics. If this additional dynamic dimension was found to enhance the encoding of sensory information substantially, horseshoe bat biosonar could be a model for the use of dynamic physical processes in sensing technology.

Diabetes insipidus--diagnosis and management.

PubMed

Di Iorgi, Natascia; Napoli, Flavia; Allegri, Anna Elsa Maria; Olivieri, Irene; Bertelli, Enrica; Gallizia, Annalisa; Rossi, Andrea; Maghnie, Mohamad

2012-01-01

Central diabetes insipidus (CDI) is the end result of a number of conditions that affect the hypothalamic-neurohypophyseal system. The known causes include germinoma/craniopharyngioma, Langerhans cell histiocytosis (LCH), local inflammatory, autoimmune or vascular diseases, trauma resulting from surgery or an accident, sarcoidosis, metastases and midline cerebral and cranial malformations. In rare cases, the underlying cause can be genetic defects in vasopressin synthesis that are inherited as autosomal dominant, autosomal recessive or X-linked recessive traits. The diagnosis of the underlying condition is challenging and raises several concerns for patients and parents as it requires long-term follow-up. Proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress to more sophisticated tools. Specifically, MRI identification of pituitary hyperintensity in the posterior part of the sella, now considered a clear marker of neurohypophyseal functional integrity, together with the careful analysis of pituitary stalk shape and size, have provided the most striking findings contributing to the diagnosis and understanding of some forms of 'idiopathic' CDI. MRI STIR (short-inversion-time inversion recovery sequencing) is a promising technology for the early identification of LCH-dependent CDI. Copyright © 2012 S. Karger AG, Basel.

[Analysis of the factors contributing to diabetes insipidus after surgeries for craniopharyngiomas].

PubMed

Luo, Shi; Pan, Jun; Qi, Song-Tao; Fang, Lu-Xiong; Fan, Jun; Liu, Bao-Guo

2009-03-01

To analyze the factors contributing to the occurrence of diabetes insipidus after operations for craniopharyngiomas. A total of 121 cases of diabetes insipidus following surgeries for craniopharyngiomas were retrospectively analyzed and the factors associated with postoperative diabetes insipidus were analyzed. The incidence of diabetes insipidus was 27.3% (33/121 cases) before the operation, 89.9% (107/1119) early after the operation and 39.8%(37/93) in later stages after the operation. The occurrence of early postoperative diabetes insipidus showed a significant relation to the classification and calcification of the craniopharyngioma. Patients with supradiaphragmatic and extraventricular tumors had the lowest incidence of postoperative diabetes insipidus. Late postoperative diabetes insipidus was closely correlated to such factors as age, classification of craniopharyngioma, and intraoperative treatment of the pituitary stalk, but not to the scope of tumor resection or tumor calcification. Late diabetes insipidus was more frequent in children and patients with severed pituitary stalk. The incidence of late postoperative diabetes insipidus was significantly higher in patients with supradiaphragmatic and extra-intraventricular tumors than in those with tumors beneath the diaphragma sellae and extraventricular tumors. Postoperative diabetes insipidus following surgeries for craniopharyngiomas is closely related to the tumor classification, calcification and pituitary stalk protection.

Metric analysis of basal sphenoid angle in adult human skulls

PubMed Central

Netto, Dante Simionato; Nascimento, Sergio Ricardo Rios; Ruiz, Cristiane Regina

2014-01-01

Objective To analyze the variations in the angle basal sphenoid skulls of adult humans and their relationship to sex, age, ethnicity and cranial index. Methods The angles were measured in 160 skulls belonging to the Museum of the Universidade Federal de São Paulo Department of Morphology. We use two flexible rules and a goniometer, having as reference points for the first rule the posterior end of the ethmoidal crest and dorsum of the sella turcica, and for the second rule the anterior margin of the foramen magnum and clivus, measuring the angle at the intersection of two. Results The average angle was 115.41°, with no statistical correlation between the value of the angle and sex or age. A statistical correlation was noted between the value of the angle and ethnicity, and between the angle and the horizontal cranial index. Conclusions The distribution of the angle basal sphenoid was the same in sex, and there was correlation between the angle and ethnicity, being the proportion of non-white individuals with an angle >125° significantly higher than that of whites with an angle >125°. There was correlation between the angle and the cranial index, because skulls with higher cranial index tend to have higher basiesfenoidal angle too. PMID:25295452

Anterior maxillary segmental distraction for correction of maxillary hypoplasia and dental crowding in cleft palate patients: a preliminary report.

PubMed

Wang, X-X; Wang, X; Li, Z-L; Yi, B; Liang, C; Jia, Y-L; Zou, B-S

2009-12-01

To evaluate the feasibility of anterior maxillary segmental distraction (AMSD) to correct maxillary hypoplasia and severe dental crowding in cleft lip and palate (CLP) patients, 7 patients (average age 16.4 years) with maxillary hypoplasia, shortened maxillary dental arch length and severe anterior dental crowding secondary to CLP were selected for this study. After anterior maxillary segmental osteotomy, 3 patients were treated using bilateral internal distraction devices, and 4 patients were treated using rigid external distraction devices. Photographs and radiographs were taken to review the improvement in facial profile and occlusion after distraction. An average 10.25 mm anterior maxillary advancement was obtained in all patients after 10-23 days of distraction and 9-16 weeks of consolidation. The sella-nasion-point A (SNA) angle increased from 69.5 degrees to 79.6 degrees. Midface convexity was greatly improved and velopharyngeal competence was preserved. The maxillary dental arch length was greatly increased by 10.1 mm (P<0.01). Dental crowding and malocclusion were corrected by orthodontic treatment. These results show that AMSD can effectively correct the hypoplastic maxilla and severe dental crowding associated with CLP by increasing the midface convexity and dental arch length while preserving velopharyngeal function, and dental crowding can be corrected without requiring tooth extraction.

Acromegaly: Role of Surgery in the Therapeutic Armamentarium

PubMed Central

Guinto, Gerardo; Abdo, Miguel; Zepeda, Erick; Aréchiga, Norma; Mercado, Moisés

2012-01-01

Acromegaly is a complex disease that requires the intervention of a multidisciplinary team. The most frequent clinical manifestations are growing of distal parts of the body and some areas of the face. Patients may also present arterial hypertension, diabetes mellitus, colonic polyps, cardiomegaly, neurological and endocrine changes secondary to the presence of a GH-secreting tumor in pituitary or extrapituitary origin, or eutopic hypothalamic GHRH hypersecretion and peripheral GHRH hypersecretion. Surgery is the first treatment used for most patients, regardless of the cause. In the great majority of cases, pituitary tumor can be removed through a transsphenoidal approach. Craniotomy is reserved for those cases with giant tumors, particularly when they grow toward the middle or posterior cranial fossa. Best surgical results are obtained when the tumor is confined into the sella turcica or if it has a regular suprasellar extension. When the disease cannot be controlled with surgery, medical treatment is indicated. Somatostatin analogues are included as the first line of medication, followed by dopamine agonist and growth hormone receptors antagonists. Radiation therapy can be also indicated in two main forms for residual tumor with medically refractory patients: radiosurgery for small tumors or fractionated stereotactic radiotherapy for larger ones. PMID:23209463

Development of the orbital region in the chondrocranium of Caretta caretta. Reconsideration of the vertebrate neurocranium configuration.

PubMed

Kuratani, S

1989-01-01

By studying the development of the orbital region in the Loggarhead turtle (Caretta caretta) and some placental mammals, it has become clear that the orbital region of the neurocranium should not be regarded as merely a "bowl" to contain the brain, but rather that its ventral part is originally flexured along with the cephalic flexure of the neural tube. At this flexure, the neurocranium is to be divided into 2 parts, the anterior and posterior. The anterior part of the neurocranial sheet is medially perforated by the infundibulum and gives rise to pila metoptica laterally. The post orbital cartilage represents the posterior part. From the above "Bauplan" of the neurocranium, the following conclusions can be drawn: (1) the simple homology of the reptilian and placental mammalian pila metoptica is questionable; (2) the pila antotica is produced by the absorption of the mid-dorsal part of the postorbital cartilage, while the dorsum sellae in mammals is produce by the chondrification of the middle part of the same anlage; (3) homology of the ala hypochiasmatica in mammals with the supratrabecular cartilage in reptiles is more feasible than with the cartilago hypochiasmatica; and (4) the crista sellaris in reptiles is not a part of the primary cranial wall but probably of secondary production.

Ectopic sphenoid sinus pituitary adenoma (ESSPA) with normal anterior pituitary gland: a clinicopathologic and immunophenotypic study of 32 cases with a comprehensive review of the english literature.

PubMed

Thompson, Lester D R; Seethala, Raja R; Müller, Susan

2012-03-01

Ectopic sphenoid sinus pituitary adenoma (ESSPA) may arise from a remnant of Rathke's pouch. These tumors are frequently misdiagnosed as other neuroendocrine or epithelial neoplasms which may develop in this site (olfactory neuroblastoma, neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, paraganglioma, melanoma). Thirty-two patients with ESSPA identified in patients with normal pituitary glands (intact sella turcica) were retrospectively retrieved from the consultation files of the authors' institutions. Clinical records were reviewed with follow-up obtained. An immunohistochemical panel was performed on available material. Sixteen males and 16 females, aged 2-84 years (mean, 57.1 years), presented with chronic sinusitis, headache, obstructive symptoms, and visual field defects, although several were asymptomatic (n = 6). By definition, the tumors were centered within the sphenoid sinus and demonstrated, by imaging studies or intraoperative examination, a normal sella turcica without a concurrent pituitary adenoma. A subset of tumors showed extension into the nasal cavity (n = 5) or nasopharynx (n = 9). Mean tumor size was 3.4 cm. The majority of tumors were beneath an intact respiratory epithelium (n = 22), arranged in many different patterns (solid, packets, organoid, pseudorosette-rosette, pseudopapillary, single file, glandular, trabecular, insular). Bone involvement was frequently seen (n = 21). Secretions were present (n = 16). Necrosis was noted in 8 tumors. The tumors showed a variable cellularity, with polygonal, plasmacytoid, granular, and oncocytic tumor cells. Severe pleomorphism was uncommon (n = 5). A delicate, salt-and-pepper chromatin distribution was seen. In addition, there were intranuclear cytoplasmic inclusions (n = 25) and multinucleated tumor cells (n = 18). Mitotic figures were infrequent, with a mean of 1 per 10 HPFs and a <1% proliferation index (Ki-67). There was a vascularized to sclerotic or calcified

Quantitative evaluation of bone development of the distal phalanx of the cow hind limb using computed tomography.

PubMed

Tsuka, T; Ooshita, K; Sugiyama, A; Osaki, T; Okamoto, Y; Minami, S; Imagawa, T

2012-01-01

Computed tomography (CT) was performed on 400 claws (200 inner and 200 outer claws) of 100 pairs of bovine hind limbs to investigate the etiological theory that an exacerbating factor for ulceration is exostosis of the tuberculum flexorium within the distal phalanx. A variety of morphological changes of the tuberculum flexorium of bovine hind limb claws was visualized by 3-dimensional CT, and the geometry of these claws suggested a growth pattern of bone development with respect to the assumed daily loading patterns. This growth occurs initially at the abaxial caudal aspect of the distal phalanx and is followed by horizontal progression toward the axial aspect. The length of downward bone development on the solar face of the distal phalanx was 2.73±1.32 mm in the outer claws, significantly greater than in the inner claws (2.38±0.96 mm). Ratios of downward (vertical) bone development to the thickness of the subcutis and the corium (VerBD ratios) did not differ between the outer and inner claws (36.7 vs. 38.3%, respectively). Ratios of horizontal bone development to the axial-to-abaxial line of the tuberculum flexorium (HorBD ratios) were approximately 60% for both outer and inner claws. These quantitative measures regarding horizontal and vertical bone development within the distal phalanx were positively correlated with age and VerBD ratios (r=0.53 and r=0.36 for the inner and outer claws, respectively). Correlations between claw width of the outer claw and length of vertical bone development (r=0.43), the HorBD ratio (r=0.51), and the VerBD ratio (r=0.42) suggested that the relative size difference between the inner and outer claws enhances bone development in the outer claw. Correlation coefficients between VerBD and HorBD ratios (r=0.52 and 0.63 for the inner and outer claws, respectively) suggested that horizontal and vertical bone development occurs as a synchronized process within the tuberculum flexorium. This age-related progress of bone development

Case Report: The Specter of Untreated Congenital Hypothyroidism in Immigrant Families

PubMed Central

Hamdoun, Elwaseila; Karachunski, Peter; Nathan, Brandon; Fischer, Melissa; Torkelson, Jane L.; Drilling, Amy

2016-01-01

Newborn screening has dramatically reduced rates of untreated congenital hypothyroidism (CH). However, in low-income nations where newborn screening programs do not exist, untreated CH remains a significant health and societal challenge. The goal of this report is to alert health care providers about the potential of undiagnosed CH in unscreened immigrant children. We report 3 siblings of Somali descent with CH who started treatment with levothyroxine at age 0.5 years, 7.7 years, and 14.8 years and were followed for 8 years. This case series demonstrates a spectrum of severity, response to treatment, and neurocognitive and growth outcomes depending on the age at treatment initiation. Patient 1, now 22 years old, went undiagnosed for 14.8 years. On diagnosis, his height was –7.5 SDs with a very delayed bone age of –13.5 SDs. His longstanding CH was associated with empty sella syndrome, static encephalopathy, and severe musculoskeletal deformities. Even after treatment, his height (–5.2 SDs) and cognitive deficits remained the most severe of the 3 siblings. Patient 2, diagnosed at 7.7 years, had moderate CH manifestations and thus a relatively intermediate outcome after treatment. Patient 3, who had the earliest diagnosis at 0.5 years, displayed the best response, but continues to have residual global developmental delay. In conclusion, untreated CH remains an important diagnostic consideration among immigrant children. PMID:27244801

Craniofacial and brain abnormalities in Laron syndrome (primary growth hormone insensitivity).

PubMed

Kornreich, L; Horev, G; Schwarz, M; Karmazyn, B; Laron, Z

2002-04-01

To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron syndrome. Eleven patients with classical Laron syndrome, nine untreated adults aged 36-68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium.

Vascular Complications of Intercavernous Sinuses during Transsphenoidal Surgery: An Anatomical Analysis Based on Autopsy and Magnetic Resonance Venography.

PubMed

Deng, Xuefei; Chen, Shijun; Bai, Ya; Song, Wen; Chen, Yongchao; Li, Dongxue; Han, Hui; Liu, Bin

2015-01-01

Vascular complications induced by intercavernous sinus injury during dural opening in the transsphenoidal surgery may contribute to incomplete tumour resections. Preoperative neuro-imaging is of crucial importance in planning surgical approach. The aim of this study is to correlate the microanatomy of intercavernous sinuses with its contrast-enhanced magnetic resonance venography (CE-MRV). Eighteen human adult cadavers and 24 patients were examined based on autopsy and CE-MRV. Through dissection of the cadavers and CE-MRV, the location, shape, number, diameter and type of intercavernous sinuses were measured and compared. Different intercavernous sinuses were identified by their location and shape in all the cadavers and CE-MRV. Compared to the cadavers, CE-MRV revealed 37% of the anterior intercavernous sinus, 48% of the inferior intercavernous sinus, 30% of the posterior intercavernous sinus, 30% of the dorsum sellae sinus and 100% of the basilar sinus. The smaller intercavernous sinuses were not seen in the neuro-images. According to the presence of the anterior and inferior intercavernous sinus, four types of the intercavernous sinuses were identified in cadavers and CE-MRV, and the corresponding operative space in the transsphenoidal surgical approach was implemented. The morphology and classification of the cavernous sinus can be identified by CE-MRV, especially for the larger vessels, which cause bleeding more easily. Therefore, CE-MRV provides a reliable measure for individualized preoperative planning during transsphenoidal surgery.

Intraoperative magnetic resonance imaging assessment of non-functioning pituitary adenomas during transsphenoidal surgery.

PubMed

Patel, Kunal S; Yao, Yong; Wang, Renzhi; Carter, Bob S; Chen, Clark C

2016-04-01

To review the utility of intraoperative imaging in facilitating maximal resection of non-functioning pituitary adenomas (NFAs). We performed an exhaustive MEDLINE search, which yielded 5598 articles. Upon careful review of these studies, 31 were pertinent to the issue of interest. Nine studies examined whether intraoperative MRI (iMRI) findings correlated with the presence of residual tumor on MRI taken 3 months after surgical resection. All studies using iMRI of >0.15T showed a ≥90% concordance between iMRI and 3-month post-operative MRI findings. 24 studies (22 iMRI and 2 intraoperative CT) examined whether intraoperative imaging improved the surgeon's ability to achieve a more complete resection. The resections were carried out under microscopic magnification in 17 studies and under endoscopic visualization in 7 studies. All studies support the value of intraoperative imaging in this regard, with improved resection in 15-83% of patients. Two studies examined whether iMRI (≥0.3T) improved visualization of residual NFA when compared to endoscopic visualization. Both studies demonstrated the value of iMRI in this regard, particularly when the tumor is located lateral of the sella, in the cavernous sinus, and in the suprasellar space. The currently available literature supports the utility of intraoperative imaging in facilitating increased NFA resection, without compromising safety.

A novel truncating AIP mutation, p.W279*, in a familial isolated pituitary adenoma (FIPA) kindred.

PubMed

Cansu, Güven Barış; Taşkıran, Bengür; Trivellin, Giampaolo; Faucz, Fabio R; Stratakis, Constantine A

2016-07-01

Familial isolated pituitary adenomas (FIPA) constitute 2-3% of pituitary tumours. AIP is the most commonly mutated gene in FIPA. We herein report a novel germline mutation of the AIP gene in a family with FIPA. We present two patients, a father and his 12-year-old daughter, diagnosed clinically and using laboratory measures with acromegaly-gigantism. Both underwent transsphenoidal hypophyseal surgery for macroadenomas. We initially detected a novel heterozygous germline AIP mutation, c.836G>A (p.W279*), in the father's DNA. We then found the same mutation in his affected daughter. Pituitary adenomas associated with AIP mutations mostly present as FIPA (68%) at an early age (78% occur at <30 years old). They are often growth hormone (GH) - or prolactin - secreting macroadenomas (88%) that have already extended beyond the sella at the time of diagnosis. Acromegalic cases are resistant to somatostatin analogues and multimodal management is frequently essential to control the disease. Our patients had normalized GH/IGF-1 values soon after surgery, although enough time may not have elapsed to reach final cure. While penetrance of the disease can be as low as 10% in FIPA, especially children and young patients with somatotropinoma and prolactinoma should be surveyed for inactivating mutations or deletions in AIP. Determining the causative mutations may be of assistance in early diagnosis, treatment success, and genetic counseling.

Craniofacial and pharyngeal airway morphology in patients with acromegaly.

PubMed

Balos Tuncer, Burcu; Canigur Bavbek, Nehir; Ozkan, Cigdem; Tuncer, Cumhur; Eroglu Altinova, Alev; Gungor, Kahraman; Akturk, Mujde; Balos Toruner, Fusun

2015-08-01

The aim of this study was to assess differences in craniofacial characteristics, upper spine and pharyngeal airway morphology in patients with acromegaly compared with healthy individuals. Twenty-one patients with acromegaly were compared with 22 controls by linear and angular measurements on cephalograms. The differences between the mean values of cephalometric parameters were analyzed with Mann-Whitney U-test. With respect to controls, anterior (p<0.05), middle (p<0.01) and posterior (p<0.05) cranial base lengths were increased, sella turcica was enlarged (p<0.001) and upper spine morphology demonstrated differences in the height of atlas (p<0.01) and axis (p<0.05) in patients with acromegaly. Craniofacial changes were predominantly found in the frontal bone (p<0.01) and the mandible (p<0.05). As for the airway, patients with acromegaly exhibited diminished dimensions at nasal (p<0.001), uvular (p<0.01), mandibular (p<0.01) pharyngeal levels and at the narrowest point of the pharyngeal airway space (p<0.001) compared to healthy controls. Soft palate width was significantly higher (p<0.001) and the hyoid bone was more vertically positioned (p<0.01) in patients with acromegaly. Current results point to the importance of the reduced airway dimensions and that dentists and/or orthodontists should be aware of the cranial or dental abnormalities in patients with acromegaly.

Human primitive meninges in and around the mesencephalic flexure and particularly their topographical relation to cranial nerves.

PubMed

Cho, Kwang Ho; Rodríguez-Vázquez, Jose Francisco; Han, Eui Hyeog; Verdugo-López, Samuel; Murakami, Gen; Cho, Baik Hwan

2010-09-20

Development of the meninges in and around the plica ventralis encephali has not been well documented. A distinct mesenchymal structure, the so-called plica ventralis encephali, is sandwiched by the fetal mesencephalic flexure. We histologically examined paraffin-embedded sections from 18 human embryos and fetuses at 6-12 weeks of gestation. In the loose tissues of the plica, the first meninx appeared as a narrow membrane along the oculomotor nerve at 7-8 weeks. Subsequently, the plica ventralis evolved into 3 parts: bilateral lateral mesenchymal condensations and a primitive membranous meninx extending between. Notably, the topographical anatomy of the oculomotor, trochlear and trigeminal nerves did not change: the oculomotor nerve ran along the rostral aspect of the membranous meninx, the trigeminal nerve ran along the caudal side of the lateral mesenchymal condensation, and the trochlear nerve remained embedded in the lateral condensation. Up to 9-10 weeks, the lateral mesenchymal condensations became tongue-like folds; i.e., the primitive form of the tentorium cerebelli, while the membranous meninx became the diaphragma sellae. The falx cerebri seemed to develop from the tongue-like folds. Overall, the final tentorium cerebelli corresponded to the regressed plica ventralis, while the parasellar area originated from the base of the plica and other tissues along the ventral aspects of the basisphenoid and basioccipital. Copyright © 2010 Elsevier GmbH. All rights reserved.

Patients cured of acromegaly do not experience improvement of their skull deformities.

PubMed

Rick, Jonathan W; Jahangiri, Arman; Flanigan, Patrick M; Aghi, Manish K

2017-04-01

Acromegaly is a rare disease that is associated with many co-morbidities. This condition also causes progressive deformity of the skull which includes frontal bossing and cranial thickening. Surgical and/or medical management can cure this condition in many patients, but it is not understood if patients cured of acromegaly experience regression of their skull deformities. We performed a retrospective analysis on patients treated at our dedicated pituitary center from 2009 to 2014. We looked at all MRI images taken during the treatment of these patients and recorded measurements on eight skull dimensions. We then analyzed these measurements for changes over time. 29 patients underwent curative treatment for acromegaly within our timeframe. The mean age for this population was 45.0 years old (range 19-70) and 55.2 % (n = 16) were female. All of these patients were treated with a transsphenoidal resection for a somatotropic pituitary adenoma. 9 (31.1%) of these patients required further medical therapy to be cured. We found statically significant variation in the coronal width of the sella turcica after therapy, which is likely attributable to changes from transsphenoidal surgery. None of the other dimensions had significant variation over time after cure. Patients cured of acromegaly should not expect natural regression of their skull deformities. Our study suggests that both frontal bossing and cranial thickening do not return to normal after cure.

The G-Axis: a growth vector for the mandible.

PubMed

Braun, Stanley; Kittleson, Russell; Kim, Kyonghwan

2004-06-01

On the basis of the G-point, defined as the center of the largest circle that is tangent to the internal inferior, anterior, and lingual surfaces of the mandibular symphysis in the sagittal view, a growth axis and its direction are described for each gender from age six to 19.25 years. Incremental growth along the G-Axis, defined by Sella-G-point, is described by regression formulas with correlation coefficients of 0.673 for female subjects and 0.749 for male subjects. The vector (direction) of the growth axis, defined by the angle alpha ((G-Axis)-(S-N)) does not materially alter in the age range studied. At age six in female subjects the angle alpha is 67.16 degrees +/- 3.03 degrees and at age 19.25 it is 66.87 degrees +/- 3.03 degrees, whereas in male subjects it is 66.12 degrees +/- 4.00 degrees and 67.93 degrees +/- 4.00 degrees, respectively. These changes and gender differences are not clinically significant. The data is based on 444 serial lateral cephalograms of 24 female subjects and 24 male subjects. The G-Axis incremental growth change and its vector offer an improved means of quantifying complex mandibular growth in the sagittal plane by using cephalometric measurements relative to and correlated with other craniofacial structures.

Unilateral agenesis of internal carotid artery associated with congenital combined pituitary hormone deficiency and pituitary stalk interruption without HESX1, LHX4 or OTX2 mutation: a case report.

PubMed

Lamine, Faïza; Kanoun, Faouzi; Chihaoui, Melika; Saveanu, Alexandru; Menif, Emna; Barlier, Anne; Enjalbert, Alain; Brue, Thierry; Slimane, Hédia

2012-12-01

Agenesis of internal carotid artery (ICA) is an unusual finding in subjects with congenital Combined Pituitary hormone deficiency (CPHD) with only nine cases being reported to date but to our best knowledge none of them was genetically investigated. A 10-years old girl presented with severe growth failure (height 103 cm) with substantial bone age delay (3 years). She had no history of perinatal insults or familial CPHD. There was no evidence of mental retardation or craniofacial dysmorphism or ophtalmological abnormalities. She was first diagnosed with GH and TSH deficiency. Cerebral magnetic resonance imaging (MRI) showed hypoplastic anterior pituitary, flat sella turcica, absent pituitary stalk with ectopic posterior pituitary as well as agenesis of the left ICA and the left carotid canal. Genomic analysis of pituitary transcription factor HESX1, LHX4 and OTX2 showed no mutations. Treatment with GH and thyroxine was started. The patient remained free of neurovascular symptoms for 5 years but she presented at the age of 15 years with delayed puberty related to an evolving gonadotropin deficiency. ICA agenesis associated with CPHD is unusual and is often asymptomatic in children. Since the CPHD with pituitary stalk interruption cannot be due to HESX1, LHX4 or OTX2 mutation in our case, other pathogenetic mechanisms may be responsible for CPHD associated with unilateral ICA agenesis.

[Dwarfism due to familial panhypopituitarism].

PubMed

Cos Welsh, J; Espinosa de los Monteros, A; de la Luz Ajuria, M; Morillo Almao, E

1977-01-01

Three sisters of 27 7/12, 13 8/12 and 9 1/12 years of age, respectively, with proportionate dwarfism, high pitched voice and lack of sexual development are described. All the patients had very low serum levels of immunoreactive growth hormone (GH), as well as of LH and FSH. Hypoglycemia induced by insulin and arginine infusion failed to increase GH levels, and the administration of the hypothalamic LH-FSH releasing hormone (LH-RH) did not elicit any response in the secretion of gonadotropins. The oldest sister developed hypothyroidism in recent years, since the I131 thyroid uptake was normal ten years before; her serum TSH was low and did not change with TRH stimulation. In addition, a low pituitary ACTH reserve was demonstrated by the hypoglycemia and metirapone tests. Case 2 showed partial pituitary TSH and ACTH reserve, whereas the youngest child only had low TSH pituitary reserve. These patients had all the clinical and laboratory characteristics of familial panhypopituitarism, with normal sella turcica. Genetic transmission in this cases is consistent with the autosomal recessive form, which is the most frequent type of inheritance of this entity. Consanguinity can not be ruled out. The results of the hypothalamic-pituitary functional tests apparently suggest that the primary defect could be located at the pituitary level. It is also possible that the pathological process may have a progressive evolution.

Gorlin-Goltz syndrome: A case series of 5 patients in North Indian population with comparative analysis of literature.

PubMed

Lata, Jeevan; Verma, Nitin; Kaur, Amandeep

2015-09-01

In Indian scenario, Gorlin-Goltz syndrome (nevoid basal cell carcinoma syndrome [NBCCS]) has been rarely reported. The clinical, radiological, and histopathological findings and major and minor criteria in five cases of NBCCS in North Indian population have been presented along with a discussion of the role of gene mutation analysis in early diagnosis of syndrome. The diagnostic findings of Gorlin-Goltz syndrome in 5 patients were compared with other reports in Indian population and with reports of this syndrome in other parts of the world. The most common features seen were keratocystic odontogenic tumors (100%), calcifications of falx cerebri (60%), palmar-plantar pits (80%), rib anomalies (80%), macroencephaly (60%), ocular hypertelorism (80%), and frontal bossing (60%) in our series. Retained deciduous teeth seen in 80% patients whose association has not been previously reported has been presented. None of our patients had basal cell carcinoma, syndactyly or polydactyly, pectus deformity, bridging of sella turcica, pigmented nevi, or family history of this syndrome in contrast to such findings in other Indian patients. Medulloblastoma has not been reported in any Indian patient so far compared to this finding in other studies conducted worldwide. Combining the features of 48 patients in 38 cases of NBCCS being published in Indian literature with five cases of our series and on comparison with other studies in the world, a wide disparity in different ethnic groups and a wide variation in presentation of syndrome within the same population is suggested.

The eye as a window to rare endocrine disorders

PubMed Central

Chopra, Rupali; Chander, Ashish; Jacob, Jubbin J.

2012-01-01

The human eye, as an organ, can offer critical clues to the diagnosis of various systemic illnesses. Ocular changes are common in various endocrine disorders such as diabetes mellitus and Graves’ disease. However there exist a large number of lesser known endocrine disorders where ocular involvement is significant. Awareness of these associations is the first step in the diagnosis and management of these complex patients. The rare syndromes involving the pituitary hypothalamic axis with significant ocular involvement include Septo-optic dysplasia, Kallman's syndrome, and Empty Sella syndrome all affecting the optic nerve at the optic chiasa. The syndromes involving the thyroid and parathyroid glands that have ocular manifestations and are rare include Mc Cune Albright syndrome wherein optic nerve decompression may occur due to fibrous dysplasia, primary hyperparathyroidism that may present as red eye due to scleritis and Ascher syndrome wherein ptosis occurs. Allgrove's syndrome, Cushing's disease, and Addison's disease are the rare endocrine syndromes discussed involving the adrenals and eye. Ocular involvement is also seen in gonadal syndromes such as Bardet Biedl, Turner's, Rothmund's, and Klinefelter's syndrome. This review also highlights the ocular manifestation of miscellaneous syndromes such as Werner's, Cockayne's, Wolfram's, Kearns Sayre's, and Autoimmune polyendocrine syndrome. The knowledge of these relatively uncommon endocrine disorders and their ocular manifestations will help an endocrinologist reach a diagnosis and will alert an ophthalmologist to seek specialty consultation of an endocrinologist when encountered with such cases. PMID:22629495

The 360 photography: a new anatomical insight of the sphenoid bone. Interest for anatomy teaching and skull base surgery.

PubMed

Jacquesson, Timothée; Mertens, Patrick; Berhouma, Moncef; Jouanneau, Emmanuel; Simon, Emile

2017-01-01

Skull base architecture is tough to understand because of its 3D complex shape and its numerous foramen, reliefs or joints. It is especially true for the sphenoid bone whom central location hinged with most of skull base components is unique. Recently, technological progress has led to develop new pedagogical tools. This way, we bought a new real-time three-dimensional insight of the sphenoid bone that could be useful for the teacher, the student and the surgeon. High-definition photography was taken all around an isolated dry skull base bone prepared with Beauchêne's technique. Pictures were then computed to provide an overview with rotation and magnification on demand. From anterior, posterior, lateral or oblique views and from in out looks, anatomical landmarks and subtleties were described step by step. Thus, the sella turcica, the optic canal, the superior orbital fissure, the sphenoid sinus, the vidian canal, pterygoid plates and all foramen were clearly placed relative to the others at each face of the sphenoid bone. In addition to be the first report of the 360 Photography tool, perspectives are promising as the development of a real-time interactive tridimensional space featuring the sphenoid bone. It allows to turn around the sphenoid bone and to better understand its own special shape, numerous foramen, neurovascular contents and anatomical relationships. This new technological tool may further apply for surgical planning and mostly for strengthening a basic anatomical knowledge firstly introduced.

Endoscopic-guided direct endonasal approach for pituitary surgery.

PubMed

Badie, B; Nguyen, P; Preston, J K

2000-02-01

Submucosal dissection of the nasal septum is often performed as part of the transseptal approach to the sella. To evaluate whether this submucosal dissection is a necessary component of this operation, we compared the morbidity of a direct transmucosal endonasal approach to that of the transseptal approach in patients undergoing pituitary surgery. Forty-one consecutive patients undergoing pituitary surgery from January 1996 to March 1999 were included in this study. The first 21 patients underwent the standard transseptal operation through either a sublabial or columellar incision. The latter 20 patients were operated on through an endoscopically guided, direct endonasal exposure, without any submucosal dissection of the nasal septum. The operative morbidity, the duration of surgery, and the length of hospitalization for each group were compared. The sphenoid sinus exposure obtained through the endonasal route was comparable with the transseptal approach and was adequate for resection of most pituitary tumors. Although the morbidity of the two approaches was similar, patients undergoing the endonasal operation had less postoperative facial pain. Furthermore, the endonasal approach significantly decreased the length of the operation (116 minutes vs. 161 minutes, p = 0.002) and the duration of hospitalization (3.6 vs. 5.1 days, p = 0.003) as compared with the transseptal route. Morbidity of the endonasal approach to the sphenoid sinus is comparable to that of a conventional transseptal approach. By eliminating the submucosal dissection, the endonasal approach reduces postoperative facial discomfort and decreases length of surgery and hospitalization.

Relationship between vertical facial patterns and dental arch form in class II malocclusion.

PubMed

Grippaudo, Cristina; Oliva, Bruno; Greco, Anna Lucia; Sferra, Simone; Deli, Roberto

2013-11-07

The purpose of this study is to evaluate the relationship between dental arch form and the vertical facial pattern determined by the angle between the mandibular plane and the anterior cranial base (Sella-nasion/mandibular plane angle (SN-MP)) in skeletal class II untreated patients. A sample of 73 Caucasians patients with untreated skeletal class II in permanent dentition was divided into three groups according to the values of the angle SN-MP. An evaluation of the arch form was performed by angular and linear relation values on each patient. Regression analysis was used to determine the statistical significance of the relationships between SN-MP angle and dental arch form. The differences among the three groups were analyzed for significance using a variance analysis. A decrease of the upper arch transversal diameters in high SN-MP angle patients and an increase in low angle SN-MP ones (P<0.05) were shown. Result analysis showed a change in upper arch shape, with a smaller intercanine width in patients with high SN-MP angle and a greater one in low angle patients. As SN-MP angle increased, the upper arch form tended to be narrower. No statistically significant difference in mandibular arch form among the three groups was found, except the angle value related to incisors position. The results showed the association between the upper dental arch form and the vertical facial pattern. On the contrary, the lower arch form was not related to the mandibular divergence.

Non-ablative fractional resurfacing in combination with topical tretinoin cream as a field treatment modality for multiple actinic keratosis: a pilot study and a review of other field treatment modalities.

PubMed

Prens, Sebastiaan P; de Vries, Karin; Neumann, H A Martino; Prens, Errol P

2013-06-01

Actinic keratoses (AK) are premalignant lesions occurring mainly in sun-damaged skin. Current topical treatment options for AK and photo-damaged skin such as liquid nitrogen and electrosurgery are not suitable for field treatment. Otherwise, therapies suitable for field treatment bring along considerable patient discomfort. Non-ablative fractional resurfacing has emerged as a logical treatment option especially for field treatment of AK. To evaluate the clinical efficacy of fractional laser therapy for clearing AK and improving skin quality. To compare patient friendliness of the "fractional" therapy with those reported for other field treatment modalities. Ten patients with Fitzpatrick skin type I to III with multiple AK and extensive sun-damaged skin, received 5-10 sessions with a 4-week interval using a 1550 nm Erbium-Glass Fractionated laser (Sellas, Korea). Four weeks and 24 weeks after the last treatment the clinical results were evaluated by an independent physician. The mean degree of improvement, in terms of reduction in the number of AK and improvement of skin texture, was 54% on a 4 point PGA scale, and persisted for approximately 6 months. The biggest advantage of fractional laser treatment, besides the eradication of AK and a clear rejuvenation effect, is the absence of "downtime". Fractional non-ablative resurfacing induces significant reduction in the number of AK and improves the skin quality. Also all patients preferred fractional laser therapy above other AK treatment modalities.

A novel AVP gene mutation in a Turkish family with neurohypophyseal diabetes insipidus.

PubMed

Ilhan, M; Tiryakioglu, N O; Karaman, O; Coskunpinar, E; Yildiz, R S; Turgut, S; Tiryakioglu, D; Toprak, H; Tasan, E

2016-03-01

Familial neurohypophyseal diabetes insipidus (FNDI) is a rare, autosomal dominant, inherited disorder which is characterized by severe polydipsia and polyuria generally presenting in early childhood. In the present study, we aimed to analyze the AVP gene in a Turkish family with FNDI. Four patients with neurohypophyseal diabetes insipidus and ten healthy members of the family were studied. Diabetes insipidus was diagnosed by the water deprivation test in affected family members. Mutation analysis was performed by sequencing the whole coding region of AVP-NPII gene using DNA isolated from peripheral blood samples. Urine osmolality was low (<300 mOsm/kg) during water deprivation test, and an increase more than 50 % in urine osmolality and recovery of the symptoms were observed by the administration of desmopressin in all patients. Plasma copeptin levels were lower than expected according to plasma osmolality. Pituitary MRI revealed partial empty sella with a bright spot in index patient and a normal neurohypophysis in the other affected subjects. Genetic screening revealed a novel, heterozygous mutation designated as c.-3A>C in all patients. c.-3A>C mutation in 5'UTR of AVP gene in this family might lead to the truncation of signal peptide, aggregation of AVP in the cytoplasm instead of targeting in the endoplasmic reticulum, thereby could disrupt AVP secretion without causing neuronal cytotoxicity, which might explain the presence of bright spot. The predicted effect of this mutation should be investigated by further in vitro molecular studies.

Paleopathological findings in radiographs of ancient and modern Greek skulls.

PubMed

Papagrigorakis, Manolis J; Karamesinis, Kostas G; Daliouris, Kostas P; Kousoulis, Antonis A; Synodinos, Philippos N; Hatziantoniou, Michail D

2012-12-01

The skull, when portrayed radiologically, can be a useful tool in detecting signs of systemic diseases and results of pathological growth mechanisms. The aim of this study was therefore to examine, compare, and classify findings in cranial configuration of pathological origin, in modern and ancient skulls. The material consists of 240 modern and 141 ancient dry skulls. Three radiographs for each skull (lateral, anteroposterior, basilar) provide enough evidence for differential diagnoses. Cases of osteoporosis are among the interesting pathological findings. A prevalence of female modern skulls in those determined as osteoporotic skulls is noted. Special interest is placed on the area of the sella turcica and many variations, regarding the shape and texture, are recognized both in ancient and modern skulls. Malignancies and important causes of cranial destruction are identified in both skull collections. Diploid thickening and osteolytic areas appear commonly among ancient remains. Moreover, from the ancient skull collection, one case possibly recognizable as fibrous dysplasia is noted while another case with an unusual exostosis gives rise to many questions. Interpreted with caution, the results of the present study, which can serve as an approach of paleopathology and paleoradiology, indicate similarity trends in cranial configuration of pathologic origin in modern and ancient people. Radiography and cephalometry were the main diagnostic tools used to gather evidence and are evaluated as a quite appropriate method to examine anthropological material and assess the internal structure of skeletal remains since they are non-destructive techniques.

Spontaneous recovery from isolated post-traumatic central hypogonadism in a woman.

PubMed

Ruggeri, Rosaria M; Smedile, Giovanni; Granata, Francesca; Longo, Marcello; Cannaíò, Salvatore; Sarlis, Nicholas J; Trimarchi, Francesco; Benvenga, Salvatore

2010-01-01

Hypopituitarism is a recognized complication of Traumatic Brain Injury (TBI). Resolution of established anterior pituitary hormones deficiency is rare. A woman was initially presented at the age of 22 years with amenorrhoea. Two years earlier she had been involved in a car accident with consequent TBI. At our evaluation, serum oestradiol (E2) was low (3 pmol/l), Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH) were borderline low [1.7 and 2.5 mIU/l, normal range (NR) for 20-35 yr old women being 1.4-13 and 2.5-13, respectively] with poor response to Gonadotropin Releasing Hormone (GnRH) (ΔLH = 2.4 and, ΔFSH = 4.0 mIU/l), while serum Prolactin (PRL) was elevated (951 mIU/l; NR: 102-496). No other pituitary hormone deficiencies were observed. Magnetic resonance imaging (MRI) showed a partially empty sella and very thin stalk. Ten years later a new endocrine evaluation was performed. On day 4 of her menstrual cycle, serum values of PRL (196 mIU/l), FSH (4.7 mIU/l), LH (4.8 mIU/l) and E2 (103 pmol/l) were within normal limits for women aged 20-35. Six months after this evaluation (that is, 12 years after trauma), the patient became pregnant and delivered a healthy baby. Our case shows that spontaneous resolution of post-head trauma anterior hypopituitarism occur even many years after head injury. Medicolegal implications are self-evident.

Predictors of favorable soft tissue profile outcomes following Class II Twin-block treatment

PubMed Central

Kim, Ji-Eun; Mah, Su-Jung; Kim, Tae-Woo; Kim, Su-Jung; Park, Ki-Ho

2018-01-01

Objective The aim of this study was to determine cephalometric factors that help predict favorable soft-tissue profile outcomes following treatment with the Class II Twin-block appliance. Methods Pre- and post-treatment lateral cephalograms of 45 patients treated with the Class II Twin-block appliance were retrospectively analyzed. Profile silhouettes were drawn from the cephalograms and evaluated by three orthodontists in order to determine the extent of improvement. Samples were divided into a favorable group (upper 30% of visual analogue scale [VAS] scores, n = 14) and an unfavorable group (lower 30% of VAS scores, n = 14). Skeletal and soft-tissue measurements were performed on the cephalograms and an intergroup comparison was conducted. Results An independent t-test revealed that the following pre-treatment values were lower in the favorable group compared to the unfavorable group: lower incisor to mandibular plane angle, lower incisor to pogonion distance, point A-nasion-point B angle, sella-nasion line (SN) to maxillary plane angle, SN to mandibular plane angle, gonial angle, and symphysis inclination. The favorable group had a larger incisor inclination to occlusal plane. Moreover, the favorable group showed larger post-treatment changes in gonial angle, B point projection, and pogonion projection than did the unfavorable group. Conclusions Class II malocclusion patients with a low divergent skeletal pattern and reduced lower incisor protrusions are likely to show more improvement in soft-tissue profile outcomes following Class II Twin-block treatment. PMID:29291184

Posterior cranial base natural growth and development: A systematic review.

PubMed

Currie, Kris; Sawchuk, Dena; Saltaji, Humam; Oh, Heesoo; Flores-Mir, Carlos; Lagravere, Manuel

2017-11-01

To provide a synthesis of the published studies evaluating the natural growth and development of the human posterior cranial base (S-Ba). The search was performed on MEDLINE, Embase, PubMed, and all EBM Reviews electronic databases. In addition, reference lists of the included studies were hand-searched. Articles were included if they analyzed posterior cranial-base growth in humans specifically. Study selection, data extraction, and risk of bias assessment were completed in duplicate. A meta-analysis was not justified. Finally, 23 published studies were selected: 5 cross-sectional and 18 cohort studies. Articles were published between 1955 and 2015, and all were published in English. The sample sizes varied between 20 and 397 individuals and consisted of craniofacial measurements from either living or deceased human skulls. Validity of the measurements was not determined in any of the studies, while six papers reported some form of reliability assessment. All the articles included multiple time points within the same population or data from multiple age groups. Growth of S-Ba was generally agreed to be from spheno-occipital synchondrosis growth. Basion displaced downward and backward and sella turcica moved downward and backward during craniofacial growth. Timing of cessation of S-Ba growth was not conclusive due to limited identified evidence. Current evidence suggests that S-Ba is not totally stable, as its dimensions change throughout craniofacial growth and a minor dimensional change is observed even in late adulthood.

Case Report: The Specter of Untreated Congenital Hypothyroidism in Immigrant Families.

PubMed

Hamdoun, Elwaseila; Karachunski, Peter; Nathan, Brandon; Fischer, Melissa; Torkelson, Jane L; Drilling, Amy; Petryk, Anna

2016-05-01

Newborn screening has dramatically reduced rates of untreated congenital hypothyroidism (CH). However, in low-income nations where newborn screening programs do not exist, untreated CH remains a significant health and societal challenge. The goal of this report is to alert health care providers about the potential of undiagnosed CH in unscreened immigrant children. We report 3 siblings of Somali descent with CH who started treatment with levothyroxine at age 0.5 years, 7.7 years, and 14.8 years and were followed for 8 years. This case series demonstrates a spectrum of severity, response to treatment, and neurocognitive and growth outcomes depending on the age at treatment initiation. Patient 1, now 22 years old, went undiagnosed for 14.8 years. On diagnosis, his height was -7.5 SDs with a very delayed bone age of -13.5 SDs. His longstanding CH was associated with empty sella syndrome, static encephalopathy, and severe musculoskeletal deformities. Even after treatment, his height (-5.2 SDs) and cognitive deficits remained the most severe of the 3 siblings. Patient 2, diagnosed at 7.7 years, had moderate CH manifestations and thus a relatively intermediate outcome after treatment. Patient 3, who had the earliest diagnosis at 0.5 years, displayed the best response, but continues to have residual global developmental delay. In conclusion, untreated CH remains an important diagnostic consideration among immigrant children. Copyright © 2016 by the American Academy of Pediatrics.

Unusual association of turner syndrome and hypopituitarism in a Tunisian family.

PubMed

Bougacha-Elleuch, N; Elleuch, M; Charfi, N; Mnif, F; Belghith, N; Abdelhedi, F; Kammoun, H; Hachicha, M; Mnif, M; Abid, M

2016-01-01

Familial occurrence of either Turner syndrome or hypopituitarism is very rare. Particularly, their association is an uncommon finding. In this context, we describe for the first time 4 sisters with Turner syndrome, hypopituitarism was reported in three among them. Our cohort consists of four Tunisian adult sisters belonging to a consanguineous family. Biochemical analysis, resonance magnetic imaging and cytogenetic analyses were performed. Turner syndrome was diagnosed at the ages of 14, 17, 31 and 43 years in cases 1, 2, 3 and 4 respectively. They suffered from short stature, dysmorphic syndrome and/or delayed puberty. Interestingly, 3 among them showed also hypopituitarism, hypogonadotrophic hypogonadism and central hypothyroidism. Somatotropic insufficiency was proven in one case. Pituitary MRI has shown an empty sella turcica with hypoplastic pituitary gland in three cases. Their karyotypes were compatible with 45X in one case, 45X/46XX in the second and 45X/46XX/47XXY with x label in two cases. Hence, the presence of these familial cases of TS must evoke new etiopathogenetic arguments. Coincidence of hypopituitarism in this family, might suggest common genetic background for the two diseases. This particular family would be a precious tool for an extensive molecular analysis. More attention should be given to other family's members mainly in the presence of delayed puberty and sterility in other members. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

A case of stiff-person syndrome due to secondary adrenal insufficiency.

PubMed

Mizuno, Yuri; Yamaguchi, Hiroo; Uehara, Taira; Yamashita, Kenichiro; Yamasaki, Ryo; Kira, Jun-Ichi

2017-06-28

We report a case of flexion contractures in a patient's legs secondary to postpartum hypopituitarism. A 56-year-old woman presented with a 3-year history of worsening flexion contractures of the hips and knees. On admission, her hips and knees could not be extended, and she had muscle stiffness and tenderness to palpation of the lower extremities. We first suspected stiff-person syndrome or Isaacs' syndrome because of her muscle stiffness. However, multiple hormones did not respond to stimulation tests, and an MRI of the brain showed atrophy of the pituitary gland with an empty sella. A subsequent interview revealed that she had suffered a severe hemorrhage while delivering her third child. She was diagnosed with panhypopituitarism and started on cortisol replacement therapy. After 1 week of treatment with hydrocortisone (10 mg/day), her symptoms quickly improved. We then added 75 μg/day of thyroid hormone. During the course of her treatment, autoantibodies against VGKC complex were found to be weakly positive. However, we considered the antibodies to be unrelated to her disease, because her symptoms improved markedly with low-dose steroid treatment. There are a few reports describing flexion contractures of the legs in patients with primary and secondary adrenal insufficiency. As these symptoms are similar to those seen in stiff-person syndrome, adrenal and pituitary insufficiency should be taken into account to achieve the correct diagnosis and treatment in patients with flexion contractures and muscle stiffness.

Skull deformations in craniosynostosis and endocrine disorders: morphological and tomographic analysis of the skull from the crypt of the Silesian Piasts in Brzeg (16th-17th century), Poland.

PubMed

Kozłowski, T; Cybulska, M; Błaszczyk, B; Krajewska, M; Jeśman, C

2014-10-01

of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed. Those results suggested the occurrence of the very rare Arnold-Chiari syndrome. Lesions found in the sella turcica indicated the development of pituitary macroadenoma, which resulted in the occurrence of discreet features of acromegaly in the facial bones. The studied skull was characterized by a significantly smaller size of the neurocranium (horizontal circumference 471 mm, cranial capacity ∼ 1080 ml) and strongly expressed brachycephaly (cranial index=86.3), while its height remained within the range for non-deformed skulls. A narrow face, high eye-sockets and prognathism were also observed. Signs of alveolar process hypertrophy with rotation and displacement of the teeth were noted. The skull showed significant morphological differences compared to both normal and other pathological skulls such as those with pituitary gigantism, scaphocephaly and microcephaly. Copyright © 2014 Elsevier GmbH. All rights reserved.

Transsphenoidal surgery assisted by a new guidance device: results of a series of 747 cases.

PubMed

Tao, Yang; Jian-wen, Gu; Yong-qin, Kuang; Li-bin, Yang; Hai-dong, Huang; Wen-tao, Yang; Xue-min, Xing

2011-10-01

The objective of this study is to report the efficacy and safety of microsurgical transsphenoidal surgery using a frame for sella guidance in a series of patients with untreated pituitary adenoma. In this study, seven hundred and forty-seven patients undergoing transsphenoidal resection of a pituitary adenoma involving use of the frame were included. Follow-up of twelve to one hundred months was performed in all patients. During the procedures using the frame, pituitary adenomas were fully exposed, and no cavernous sinus haemorrhage due to anteroposterior displacement or internal carotid artery lesion due to right-and-left deviation occurred. The duration of the surgical procedure ranged from 28 min to 87 min with a mean of 44 min. The most frequent tumour type was prolactin-secreting adenoma (32.4%), followed by clinically non-functioning adenoma (NFPA) (28.5%), growth hormone-secreting adenoma (25.0%), and adrenocorticotropin-secreting adenoma (13.7%). Normalisation of visual defects occurred in 226 (42.2%) of the 535 patients with visual disturbances. Normalisation of hormone occurred in 458 of 551 patients with endocrine-active tumour in the follow-up period. Two patients died as a consequence of surgery. The endonasal transsphenoidal technique is a safe, quick, and effective approach to pituitary adenomas. Our guidance frame allows the surgeon to open and close the wound rapidly, which avoids trajectory deviation and shortens the duration of the surgical procedure. Copyright © 2011 Elsevier B.V. All rights reserved.

Predictors of favorable soft tissue profile outcomes following Class II Twin-block treatment.

PubMed

Kim, Ji-Eun; Mah, Su-Jung; Kim, Tae-Woo; Kim, Su-Jung; Park, Ki-Ho; Kang, Yoon-Goo

2018-01-01

The aim of this study was to determine cephalometric factors that help predict favorable soft-tissue profile outcomes following treatment with the Class II Twin-block appliance. Pre- and post-treatment lateral cephalograms of 45 patients treated with the Class II Twin-block appliance were retrospectively analyzed. Profile silhouettes were drawn from the cephalograms and evaluated by three orthodontists in order to determine the extent of improvement. Samples were divided into a favorable group (upper 30% of visual analogue scale [VAS] scores, n = 14) and an unfavorable group (lower 30% of VAS scores, n = 14). Skeletal and soft-tissue measurements were performed on the cephalograms and an intergroup comparison was conducted. An independent t -test revealed that the following pre-treatment values were lower in the favorable group compared to the unfavorable group: lower incisor to mandibular plane angle, lower incisor to pogonion distance, point A-nasion-point B angle, sella-nasion line (SN) to maxillary plane angle, SN to mandibular plane angle, gonial angle, and symphysis inclination. The favorable group had a larger incisor inclination to occlusal plane. Moreover, the favorable group showed larger post-treatment changes in gonial angle, B point projection, and pogonion projection than did the unfavorable group. Class II malocclusion patients with a low divergent skeletal pattern and reduced lower incisor protrusions are likely to show more improvement in soft-tissue profile outcomes following Class II Twin-block treatment.

Endoscopic Endonasal Transsphenoidal Drainage of a Spontaneous Candida glabrata Pituitary Abscess.

PubMed

Strickland, Ben A; Pham, Martin; Bakhsheshian, Joshua; Carmichael, John; Weiss, Martin; Zada, Gabriel

2018-01-01

Noniatrogenic pituitary abscess remains a rare clinical entity, and is the indication for surgery in <1% of transsphenoidal approaches. Correct diagnosis of this rare entity is often delayed. Without timely treatment, morbidity and mortality are high. Of the 200 cases reported to date, less than one-half have identified a causative organism. We report the second case of a pituitary abscess caused by Candida species, and also provide an intraoperative video showing the endoscopic management of this pathology. A 33-year-old woman presented with headache, hypopituitarism, and vision loss in the setting of diabetic ketoacidosis, and was found to have multiple abscesses in the liver, lung, kidney, and uterus. Brain magnetic resonance imaging revealed a 15-mm cystic sellar mass with restricted diffusion. The patient underwent urgent evacuation of the abscess via an endoscopic endonasal transsphenoidal route, with obvious purulent material filling the sella, later identified as Candida glabrata. Antimicrobial therapy was refined appropriately, and she exhibited significant improvement in neurologic function, although endocrinopathy has persisted. With timely management, including a combination of surgical drainage and appropriate antimicrobial therapy, neurologic outcomes are good in most cases of pituitary abscess; however, endocrinopathy often does not improve. Although most reported cases with identified causative organisms speciate bacteria, some cases are of fungal etiology and require different antimicrobial agents. This further underscores the importance of identifying the causative agent. Copyright © 2017 Elsevier Inc. All rights reserved.

Anterior pituitary failure (panhypopituitarism) with balanced chromosome translocation 46,XY,t(11;22)(q24;q13).

PubMed

Yang, C Y; Chou, C W; Chen, S Y; Cheng, H M

2001-04-01

Hypopituitarism is the clinical syndrome that results from failure of the anterior pituitary gland to produce its hormones. Hypopituitarism can result from: (1) intrinsic or primary pituitary disease; (2) intrinsic hypothalamic or secondary pituitary disease; or (3) extrinsic extrasellar or parasellar disease. The etiologies of primary hypopituitarism are miscellaneous. The dominant clinical picture of hypopituitarism in the adult is that of hypogonadism. Reports have associated hypopituitarism with anti-pituitary-antibodies, hereditary syndrome and chromosome defects, but hypopituitarism has rarely been associated with balanced chromosome translocation (11;22)(q24;q13). Here, we describe a case of anterior pituitary failure with balanced chromosome translocation. A 19-year-old Chinese teenager presented with failure of pubertal development and sexual infantilism. On examination, the patient had the classic appearance of hypogonadism. Endocrine studies and three combined pituitary function tests revealed panhypopituitarism. A chromosomal study revealed 46,XY,t(11;22)(q24;q13), a balanced translocation between 11q24 and 22q13. Chest films showed delayed fusion of bilateral humeral head epiphyses and bilateral acromions. Scrotal sonography revealed testes were small bilaterally. Magnetic resonance imaging (MRI) of the sella revealed pituitary dwarfism. The patient received 19 months replacement therapy, including steroids (prednisolone 5 mg each day), L-thyroxine (Eltroxin 100 ug each day), and testosterone enanthate 250 mg every two weeks. His height increased 4 cm with secondary sexual characteristics developed, and muscle power increased.

Glacial isostatic adjustment using GNSS permanent stations and GIA modelling tools

NASA Astrophysics Data System (ADS)

Kollo, Karin; Spada, Giorgio; Vermeer, Martin

2013-04-01

Glacial Isostatic Adjustment (GIA) affects the Earth's mantle in areas which were once ice covered and the process is still ongoing. In this contribution we focus on GIA processes in Fennoscandian and North American uplift regions. In this contribution we use horizontal and vertical uplift rates from Global Navigation Satellite System (GNSS) permanent stations. For Fennoscandia the BIFROST dataset (Lidberg, 2010) and North America the dataset from Sella, 2007 were used respectively. We perform GIA modelling with the SELEN program (Spada and Stocchi, 2007) and we vary ice model parameters in space in order to find ice model which suits best with uplift values obtained from GNSS time series analysis. In the GIA modelling, the ice models ICE-5G (Peltier, 2004) and the ice model denoted as ANU05 ((Fleming and Lambeck, 2004) and references therein) were used. As reference, the velocity field from GNSS permanent station time series was used for both target areas. Firstly the sensitivity to the harmonic degree was tested in order to reduce the computation time. In the test, nominal viscosity values and pre-defined lithosphere thicknesses models were used, varying maximum harmonic degree values. Main criteria for choosing the suitable harmonic degree was chi-square fit - if the error measure does not differ more than 10%, then one might use as well lower harmonic degree value. From this test, maximum harmonic degree of 72 was chosen to perform calculations, as the larger value did not significantly modify the results obtained, as well the computational time for observations was kept reasonable. Secondly the GIA computations were performed to find the model, which could fit with highest probability to the GNSS-based velocity field in the target areas. In order to find best fitting Earth viscosity parameters, different viscosity profiles for the Earth models were tested and their impact on horizontal and vertical velocity rates from GIA modelling was studied. For every

Clinical importance of the basal cavernous sinuses and cavernous carotid arteries relative to the pituitary gland and macroadenomas: quantitative analysis of the complete anatomy.

PubMed

Yilmazlar, Selcuk; Kocaeli, Hasan; Eyigor, Ozhan; Hakyemez, Bahattin; Korfali, Ender

2008-08-01

It is unusual to encounter hemorrhagic complications caused by arterial or venous damage during TSS. Problems with these structures can lead to permanent disability or death. Our aim was to quantitatively analyze anatomical and radiologic relationships among the BCS, the CCA, and the pituitary gland, as these structures are accessed during TSS. Forty-nine formaldehyde-fixed, sellar-parasellar tissue blocks from adult cadavers were used to simulate accessing the BCSs via TSS. In each specimen, size of the pituitary gland and specific characteristics of each BCS and the horizontal segment of each CCA were recorded. Nine other specimens were used for histologic investigation and microanatomical measurements. To attest correlation between clinical data and cadaveric measurements, coronal MRI scans of 22 healthy adults as well as of 28 patients with macroadenomas were analyzed. In cadaveric specimens, distances between both CCAs in the BCS were 17.1 +/- 4.0 mm anteriorly, 20.3 +/- 4.2 mm medially, and 18.8 +/- 4.6 mm posteriorly. In this study, the anterior medial space of the BCS was dominant in 12 specimens on the right side and in 5 specimens on the left; the posterior medial space of the BCS was dominant in 23 specimens on the right side and in 9 specimens on the left side. The right medial BCS was dominant in 35 specimens. On histologic coronal sections, some part of the carotid artery's (CA's) diameter was located below the line passing from the basal dural layer ranging from 5.3% to 65.4%. In normal-sella images, distances between both CCAs were 15.4 +/- 1.8 mm anteriorly, 16.0 +/- 2.8 mm medially, 16.2 +/- 3.4 mm posteriorly. On coronal normal-sella images, some part of the CA's diameter was located below the line passing from the basal dural layer ranging from 16.4% to 66.7%. In macroadenomas, distances between both CCAs were 22.0 +/- 3.6 mm anteriorly, 21.5 +/- 3.8 mm medially, and 20.7.2 +/- 3.7 mm posteriorly . On coronal images, in only 6 of 28

Gorlin–Goltz syndrome: A case series of 5 patients in North Indian population with comparative analysis of literature

PubMed Central

Lata, Jeevan; Verma, Nitin; Kaur, Amandeep

2015-01-01

Objective: In Indian scenario, Gorlin–Goltz syndrome (nevoid basal cell carcinoma syndrome [NBCCS]) has been rarely reported. The clinical, radiological, and histopathological findings and major and minor criteria in five cases of NBCCS in North Indian population have been presented along with a discussion of the role of gene mutation analysis in early diagnosis of syndrome. Materials and Methods: The diagnostic findings of Gorlin–Goltz syndrome in 5 patients were compared with other reports in Indian population and with reports of this syndrome in other parts of the world. Results: The most common features seen were keratocystic odontogenic tumors (100%), calcifications of falx cerebri (60%), palmar-plantar pits (80%), rib anomalies (80%), macroencephaly (60%), ocular hypertelorism (80%), and frontal bossing (60%) in our series. Retained deciduous teeth seen in 80% patients whose association has not been previously reported has been presented. None of our patients had basal cell carcinoma, syndactyly or polydactyly, pectus deformity, bridging of sella turcica, pigmented nevi, or family history of this syndrome in contrast to such findings in other Indian patients. Medulloblastoma has not been reported in any Indian patient so far compared to this finding in other studies conducted worldwide. Conclusions: Combining the features of 48 patients in 38 cases of NBCCS being published in Indian literature with five cases of our series and on comparison with other studies in the world, a wide disparity in different ethnic groups and a wide variation in presentation of syndrome within the same population is suggested. PMID:26604574

Hybrid Microscopic-Endoscopic Surgery for Craniopharyngioma in Neurosurgical Suite: Technical Notes.

PubMed

Ichikawa, Tomotsugu; Otani, Yoshihiro; Ishida, Joji; Fujii, Kentaro; Kurozumi, Kazuhiko; Ono, Shigeki; Date, Isao

2016-01-01

The best chance of curing craniopharyngioma is achieved by microsurgical total resection; however, its location adjacent to critical structures hinders complete resection without neurologic deterioration. Unrecognized residual tumor within microscopic blind spots might result in tumor recurrences. To improve outcomes, new techniques are necessary to visualize tissue within these blind spots. We examined the success of hybrid microscopic-endoscopic neurosurgery for craniopharyngioma in a neurosurgical suite. Four children with craniopharyngiomas underwent microscopic resection. When the neurosurgeon was confident that most of the visible tumor was removed but was suspicious of residual tumor within the blind spot, he or she used an integrated endoscope-holder system to inspect and remove any residual tumor. Two ceiling monitors were mounted side by side in front of the surgeon to display both microscopic and endoscopic views and to view both monitors simultaneously. Surgery was performed in all patients via the frontobasal interhemispheric approach. Residual tumors were observed in the sella (2 patients), on the ventral surface of the chiasm and optic nerve (1 patient), and in the third ventricle (1 patient) and were resected to achieve total resection. Postoperatively, visual function was improved in 2 patients and none exhibited deterioration related to the surgery. Simultaneous microscopic and endoscopic observation with the use of dual monitors in a neurosurgical suite was ergonomically optimal for the surgeon to perform microsurgical procedures and to avoid traumatizing surrounding vessels or neural tissues. Hybrid microscopic-endoscopic neurosurgery may contribute to safe, less-invasive, and maximal resection to achieve better prognosis in children with craniopharyngioma. Copyright © 2016 Elsevier Inc. All rights reserved.

Quantitative study of the opticocarotid and carotid-oculomotor windows for the interpeduncular fossa, before and after internal carotid artery mobilization and posterior communicating division.

PubMed

Kim, Young-Don; Elhadi, Ali M; Mendes, George A C; Maramreddy, Naveen; Agrawal, Abhishek; Kalb, Samuel; Nakaji, Peter; Spetzler, Robert F; Preul, Mark C

2015-03-01

The management of basilar apex (BX) aneurysms remains problematic. We quantified the surgical exposure of the BX through the opticocarotid window (OCW) and the carotid-oculomotor window (COW), before and after mobilization of the internal carotid artery and division of the posterior communicating artery (PCoA). Eleven silicone-injected cadaveric heads were dissected bilaterally. The surgical dissection was divided into 4 major steps: (1) supraorbital modified orbitozygomatic craniotomy, (2) mobilization of the internal carotid artery after drilling out the anterior clinoid process intradurally and cutting the distal dural ring, (3) drilling out the posterior clinoid process and dorsum sellae, and (4) dividing the PCoA from the posterior third portion of the vessel. A frameless navigation system was used to quantify the surgical exposure area of the BX through the OCW and COW. The total surgical area increased significantly from steps 1 to 4 (P < .001) in both OCW and COW groups. Overall, there was a larger total surgical area obtained in the COW compared with the OCW (P = .010). ICA mobilization increased the surgical area for temporary (P < .001) and permanent (P < .003) clip application in both windows. The division of PCoA significantly increased the overall surgical area for permanent clip application (P < .003). Compared with the OCW, the COW had a significantly increased change in the area for permanent clip application in the low-lying group (P = .03). When approaching the BX via the pterion route, the appropriate surgical step and window should be selected according to characteristics of the PCoA and height of the BX.

Morphometric analysis of the infraorbital foramen, canal and groove using cone beam CT: considerations for creating artificial organs.

PubMed

Orhan, Kaan; Misirli, Melis; Aksoy, Secil; Seki, Umut; Hincal, Evren; Ormeci, Tugrul; Arslan, Ahmet

2016-01-01

The aim of this study was to examine the anatomy and variations of the infraorbital foramen and its surroundings via morphometric measurements using cone beam computed tomography (CBCT) scans derived from a 3D volumetric rendering program. 354 sides of CBCT scans from 177 patients were examined in this study. DICOM data from these images were exported to Maxilim® software in order to generate 3D surface models. The morphometric measurements were done for infraorbital foramen (IOF), infraorbital groove (IOG) and infraorbital canal (IOC). All images were evaluated by 1 radiologist. To assess intra-observer reliability, the Wilcoxon matched-pairs signed rank test was used. Differences between sex, side, age and measurements were evaluated using chi-square and paired t-test and measurements were evaluated using 1-way ANOVA tests. Differences were considered significant when p<0.05. The most common shape was oval for IOF and parallel for IOC without any accessory foramen. The results showed that females have smaller dimensions for the measurements between the two foramen rotundum (FR), FR-IOF, sella-FR, center of the IOF (cIOF)-nasion (N), cIOF-NB (nasion-B) (p>0.05). No significant difference was found according to age groups (p>0.05). These results provide detailed knowledge of the anatomical characteristics in this particular area. CBCT imaging with lower radiation dose and thin slices can be a powerful tool for anesthesia procedures like infra orbital nerve blocks, for surgical approaches like osteotomies and neurectomies and also for generating artificial prostheses.

The diagnosis of children with central diabetes insipidus.

PubMed

Ghirardello, Stefano; Garrè, Maria-Luisa; Rossi, Andrea; Maghnie, Mohamad

2007-03-01

Central diabetes insipidus is the end result of a number of different diseases that affect the hypothalamic-neurohypophyseal system. In many patients, especially children and young adults, it is caused by the destruction or degeneration of neurons that originate in the supraoptic and paraventricular nuclei of the hypothalamus. The known causes of these lesions include germinoma or craniopharyngioma; Langerhans cell histiocytosis; local inflammatory, autoimmune or vascular diseases; trauma resulting from surgery or an accident; sarcoidosis; metastases; and midline cerebral and cranial malformations. In rare cases, genetic defects in AVP synthesis that are inherited as autosomal dominant, autosomal recessive or X-linked recessive traits are the underlying cause. Accurate diagnostic differentiation is essential for both safe and effective disease management. Proper etiological diagnosis can be achieved via a series of steps that start with clinical observations and then progress, as needed, to more sophisticated methods. Indeed, magnetic resonance imaging (MRI) represents the examination method of choice for evaluating hypothalamic-pituitary-related endocrine diseases due to its ability to provide strongly-contrasted high-resolution multi-planar and spatial images. Specifically, MRI allows a detailed and precise anatomical study of the pituitary gland by differentiating between the anterior and posterior pituitary lobes. MRI identification of pituitary hyperintensity in the posterior part of the sella, now considered to be a clear marker of neurohypophyseal functional integrity, together with careful analysis of pituitary stalk shape and size, have provided the most striking recent findings contributing to the diagnosis and understanding of some forms of 'idiopathic' central diabetes insipidus.

Central diabetes insipidus: clinical profile and factors indicating organic etiology in children.

PubMed

Bajpai, Anurag; Kabra, Madhulika; Menon, P S N

2008-06-01

To evaluate the profile of children with central diabetes insipidus (DI) and identify factors indicating organic etiology. Retrospective chart review. Tertiary referral hospital. Fifty-nine children with central DI (40 boys, 19 girls). Features of organic and idiopathic central DI were compared using students t test and chi square test. Odds ratio was calculated for factors indicating organic etiology. Diagnosis included post-operative central DI (13, 22%), central nervous system (CNS) malformations (5, 8.6% holoprosencephaly 4 and hydrocephalus 1), histiocytosis (11, 18.6%), CNS pathology (11, 18.6%; craniopharyngioma 3, empty sella 2, germinoma 2, neuro-tuberculosis 2, arachnoid cyst 1 and glioma 1) and idiopathic central DI (19, 32.2%). Children with organic central DI were diagnosed later (7.8+/- 3.1 years against 5.3+/-2.4 years, P=0.03) and had lower height standard deviation score (-2.7+/-1.0 versus -1.0+/- 1.0, P<0.001) compared to idiopathic group. A greater proportion of children with organic central DI had short stature (81.8% against 10.5%, P <0.001, odds ratio 38.25), neurological features (45.5% against 0%, p 0.009) and anterior pituitary hormone deficiency (81.8% against 5.3%, P<0.001, odds ratio 81) compared to idiopathic group. A combination of short stature and onset after five years of age led to discrimination of organic central DI from idiopathic group in all cases. Organic central DI should be suspected in children presenting after the age of five years with growth retardation and features of anterior pituitary deficiency.

A randomized cross-over study comparing cabergoline and quinagolide in the treatment of hyperprolactinemic patients.

PubMed

De Luis, D A; Becerra, A; Lahera, M; Botella, J I; Valero; Varela, C

2000-01-01

Quinagolide (QUI) and cabergoline (CAB) are dopamine agonists recently introduced for the treatment of hyperprolactinemia. In the present study, these drugs have been compared in terms of effectiveness and tolerability. Twenty patients (18 females and 2 males) with hyperprolactinemia (8 with microprolactinomas, 6 with idiopathic hyperprolactinemia and 6 with empty sella turcica syndrome) were treated with oral QUI (75 microg once daily) and CAB (0,5 mg twice weekly), in a randomized cross-over trial with placebo between both drugs. Each drug was administered for 12 weeks, separated by other 12 weeks with placebo. PRL levels decreased with both drugs at 2 or 4 weeks of starting the treatment, without differences between both drugs at weeks 4, 8 and 12. At week 12, normal PRL levels (<20 ng/ml) were attained in 90% patients with CAB and only in 75% patients with QUI (p<0.05). After discontinuation of treatment, significant increase in serum PRL was higher after QUI withdrawal than after CAB. Clinical efficacy of both treatments was similar in terms of improvement amenorrhea, oligomenorrhea, galactorrhea, and impotence. All patients completed both cycles of treatment, and the most frequent side-effects were nausea, headache and dizziness, without significant differences between CAB (30%) and QUI (55%). Our study indicates that, at the doses employed here, CAB showed a high percentage of patients with normal PRL at the end of treatment and long-lasting efficacy in the levels of PRL. Clinical response and side-effects were similar in both drugs.

Association of craniopharyngioma and pituitary adenoma.

PubMed

Guaraldi, Federica; Prencipe, Nunzia; di Giacomo, Valentina; Scanarini, Massimo; Gasco, Valentina; Gardiman, Marina Paola; Berton, Alessandro M; Ghigo, Ezio; Grottoli, Silvia

2013-08-01

Intracranial tumors of different histologic types infrequently affect patients with pituitary adenomas and no history of head irradiation. The association with craniopharyngioma is extremely rare. Aims of this paper are: (1) to provide a critical literature review of typical features of pituitary adenoma presenting in association with craniopharyngioma; (2) to describe the first documented (clinically, biochemically, histologically, and radiologically) case of aggressive, suprasellar papillary craniopharyngioma presenting with amenorrhea, progressive reduction of visual field, and severe headache in a 38-year-old woman, a decade after surgical cure for microprolactinoma associated with empty sella, during which she had carried two pregnancies; and (3) to discuss common etiopathogenetic mechanisms, in relation to the management of these lesions. Systematic literature search for English literature focusing on the association of craniopharyngioma and pituitary adenoma was performed using PubMed database. Additional relevant articles from references lists were also included. Clinical, laboratory, and radiological examinations performed in our patient for the two brain lesions at diagnosis and follow up were collected. Literature search retrieved nine articles. Typically, craniopharyngioma were of adamantinomatous type, occurred simultaneously to pituitary adenoma, presented with headache and visual loss, and affected men. No case of clearly documented metachronous lesion affecting a woman after pregnancy had been described before. Although very rare and with uncertain etiopathogenesis, second tumors (i.e., craniopharyngioma) should be considered in patients with a history of pituitary adenoma, presenting with suggestive signs and symptoms, even after a long disease-free period, in order to provide proper and prompt treatment.

Assessment of Antero-Posterior Skeletal and Soft Tissue Relationships of Adult Indian Subjects in Natural Head Position and Centric Relation

PubMed Central

Latif, Vishnu Ben; Keshavaraj; Rai, Rohan; Hegde, Gautham; Shajahan, Shabna

2015-01-01

Background: The aim of this study was to verify the intra-individual reproducibility of natural head position (NHP) in centric relation (CR) position, to prove the inter-individual differences in the Frankfort horizontal plane and sella-nasion line compared with the true horizontal line, and to establish linear norms from A-point, B-point, Pog as well as soft tissue A-point, soft tissue B-point, and soft tissue Pog to nasion true vertical line (NTVL) in adult Indian subjects. Methods: Lateral cephalograms (T1) of Angle’s Class I subjects were taken in NHP and with bite in CR. A second lateral cephalogram (T2) of these subjects with ANB angle in the range 1-4° were taken after 1 week using the same wax bite and both the radiographs were analyzed based on six angular parameters using cephalometric software (Do-it, Dental studio NX version 4.1) to assess the reproducibility of NHP. Linear values of six landmarks were taken in relation to NTVL, and the mean values were calculated. A total of 116 subjects were included in this study. Results: When the cephalometric values of T1 and T2 were analyzed, it was found that, the parameters showed a P < 0.001, indicating the reproducibility of NHP in CR. Mean values for point A, point B, Pog and their soft tissue counterparts were also obtained. Conclusion: The study proved that NHP is a reproducible and accurate when recorded with the mandible in CR. Linear norms for skeletal Class I subjects in relation to NTVL were established. PMID:26124598

Sphenoid sinus types, dimensions and relationship with surrounding structures.

PubMed

Štoković, Nikola; Trkulja, Vladimir; Dumić-Čule, Ivo; Čuković-Bagić, Ivana; Lauc, Tomislav; Vukičević, Slobodan; Grgurević, Lovorka

2016-01-01

The human sphenoid sinus is an extremely variable cavity and an important landmark in hypophyseal surgery. The aim of this study was to investigate the relationship between the sphenoid sinus type, size, extent of pneumatization and occurrence of protrusions of the adjacent neurovascular structures. A total of 51 randomly selected skulls (≥20 years of age, 33 male; 102 sinuses) were analyzed using cone beam computed tomography to estimate pneumatization extension beyond the body of the sphenoid (planum sphenoidale, pterygoid process, greater wings, clivus, dorsum sellae) and protrusions of the maxillary, mandibular, optic or pterygoid nerve or the internal carotid artery. Difference in pneumatization type between the left and the right-sided sinus was observed in 45% of the skulls. Conchal pneumatization was registered in 2%, presellar in 24%, sellar in 41% and postsellar in 33% of total sinuses. Presellar sinuses frequently pneumatized planum sphenoidale and sporadically other structures, and were characterized by sporadic optic nerve protrusions. Sellar and particularly postsellar sinuses were characterized by simultaneous pneumatization extensions and neurovascular protrusions. In the case of postsellar-type sinuses, the probability of these multiple interactions was not affected by their actual size, while it increased with the increasing sinus dimensions in the case of sellar-type sinuses. A more detailed analysis indicated that increasing sinus height, length or width increased the probability of interactions and pneumatization of particular surrounding structures. Data suggest that the sphenoid sinus pneumatization type and dimensions might be used to estimate the risks of iatrogenic injury during transsphenoidal surgical procedures. Copyright © 2015 Elsevier GmbH. All rights reserved.

Facial artery musculomucosal flap for reconstruction of skull base defects: a cadaveric study.

PubMed

Xie, Liyue; Lavigne, François; Rahal, Akram; Moubayed, Sami Pierre; Ayad, Tareck

2013-08-01

Failure in skull base defects reconstruction following tumor resection can have serious consequences such as ascending meningitis and pneumocephaly. The nasoseptal flap showed a very low incidence of cerebrospinal fluid leak but is not always available. The superiorly pedicled facial artery musculomucosal (FAMM) flap has been successfully used for reconstruction of head and neck defects. Our objective is to show that the FAMM flap can be used as a new alternative in skull base reconstruction. Cadaveric study. Feasibility. Thirteen specimens underwent bilateral FAMM flap dissection. Two new modifications of the traditional FAMM flap have been developed. Feasibility in FAMM flap transfer to the skull base was investigated through endoscopic skull base dissection and maxillectomy in four specimens. Measurements were recorded for each harvested flap. The mean surface area of the modified FAMM flap efficient for reconstruction was 15.90 cm(2) . The flaps easily covered the simulated defects of the frontal sinus and the fovea ethmoidalis areas. Modifications of the traditional FAMM flap were necessary for a tension-free coverage of the planum sphenoidale and sella turcica. The FAMM flap holds high potential as a new alternative vascular flap in skull base reconstruction. However, it has not been used in patients yet and should be considered only when other options are not available. New modifications developed in this article can elongate the traditional FAMM flap, potentially contributing to a tighter seal of the skull base defect than FAMM flap alone. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

Management of Severely Atrophic Maxilla in Ectrodactyly Ectodermal Dysplasia-cleft Syndrome.

PubMed

Rachmiel, Adi; Turgeman, Shahar; Emodi, Omri; Aizenbud, Dror; Shilo, Dekel

2018-02-01

Ectrodactyly ectodermal dysplasia-cleft syndrome is a rare genetic syndrome with an incidence of 1/90,000 live births, characterized by cleft lip and palate, severely hypoplastic maxilla, and hypodontia. Patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome suffer from a severely hypoplastic maxilla that is highly difficult to treat using traditional orthognathic methods. In this study, we propose using distraction osteogenesis to achieve a major advancement while maintaining good stability and minimal relapse. To our knowledge, this is the first description of patients with this syndrome treated using distraction osteogenesis. Five patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome were included in the study. All patients had been operated on according to the well-established protocol of cleft lip and palate reconstruction before maxillary distraction osteogenesis. Hard and soft-tissue changes were evaluated by cone beam computed tomography and lateral cephalograms before distraction osteogenesis (T1), at the postdistraction point (T2) and after 1 year of follow-up (T3). Examination revealed marked maxillary advancement in all our patients with a significant mean difference in hard tissue parameters (condylion to A point = 18 mm; nasion-sella line to A point = 15.2 degrees) and a notable improvement in facial convexity (20.9 degrees). One year follow-up measurements demonstrated mild relapse rates of 6% in the horizontal plane. We conclude that despite the challenging anatomic and physiological features of ectrodactyly ectodermal dysplasia-cleft patients, by enhancing current surgical techniques, there is promising potential for improved patient outcomes, achieving normognathic facial appearance with implant supported rehabilitation.

Anatomic Dissection of Arachnoid Membranes Encircling the Pituitary Stalk on Fresh, Non-Formalin-Fixed Specimens: Anatomoradiologic Correlations and Clinical Applications in Craniopharyngioma Surgery.

PubMed

Ciappetta, Pasqualino; Pescatori, Lorenzo

2017-12-01

The anatomy of the arachnoid membranes and cisternal spaces around the pituitary stalk has not been yet exhaustively described and understood. In this study, we performed a detailed anatomic study on fresh, non-formalin-fixed cadavers of the arachnoid membranes encircling the pituitary stalk and correlate our anatomic findings with magnetic resonance imaging (MRI). Ten fresh, non-formalin-fixed, non-silicon-injected adult cadaveric heads were analyzed in this study. The membrane and cisterns that were studied for our study were as follows: 1) the diaphragma sellae and its dural components; 2) the basal arachnoid membrane; 3) the Liliequist membrane with its diencephalic and mesencephalic portion; 4) the medial carotid membrane; 5) the chiasmatic cistern; and 6) the pituitary stalk. MRI examinations of the sellar region were performed in 15 healthy volunteers (9 men, mean age 40 years; and 6 women mean age, 37 years) to visualize the arachnoid membrane encircling the pituitary stalk. MRI examinations were performed with a 3-T unit. A 3-dimensional constructive interference in steady state pulse magnetic resonance sequence was used. All the membranes examined were visualized clearly in all the dissections performed. Their 3-dimensional organization around the pituitary stalk was clarified and confirmed by MRI. Our study gives a detailed description of the pituitary stalk arachnoid sheets on fresh, non-formalin-fixed cadavers. This technique allowed us to clearly identify a funnel-shaped arachnoid collar encircling the pituitary stalk and delimiting a distinct cisternal space belonging to the stalk itself. Copyright © 2017 Elsevier Inc. All rights reserved.

A probable case of Hand-Schueller-Christian's disease in an Egyptian mummy revealed by CT and MR investigation of a dry mummy.

PubMed

Cavka, Mislav; Petaros, Anja; Ivanac, Gordana; Aganović, Lejla; Janković, Ivor; Reiter, Gert; Speier, Peter; Nielles-Vallespin, Sonja; Brkljacić, Boris

2012-03-01

The challenging mission of paleopathologists is to be capable to diagnose a disease just on the basis of limited information gained by means of one or more paleodiagnostic techniques. In this study a radiologic, anthropologic and paleopathologic analysis of an ancient Egyptian mummy through X-rays, CT and MR was conducted. An Ancient Egyptian mummy ("Mistress of the house", Archeological Museum, Zagreb, Croatia) underwent digital radiography, computed tomography and magnetic resonance imaging employing 3-dimensional ultra-short-echo time (UTE) sequence, that allows to image ancient dry tissue. Morphological observations on the skull and pelvis, the stages of epiphyseal union and dental wear indicated that the remains are those of a young adult male. Multiple osseous lytic lesions were observed throughout the spine as well as on the frontal, parietal, and occipital bone, orbital wall and the sella turcica of the sphenoid. Considering the sex and age of the individual and the features of the lesions, the authors propose the diagnosis of Hand-Schueller-Christian's disease. This is the first study to have effectively used MR images in the differential diagnosis of a disease. It also confirmed the CT value in revealing central nervous system involvement just by detecting skeletal lesions. Although the mummy was previously dated to 3rd century B.C. based on the properties of the sarcophagi, the sex of the mummy suggests that it was most probably transferred into these sarcophagi in later times. The mummification techniques used and radiometric data (C14) dated it to 900-790. B.C.

Features of lateral cephalograms associated with difficult laryngoscopy in Japanese children undergoing oral and maxillofacial surgery.

PubMed

Kohjitani, Atsushi; Miyawaki, Takuya; Miyawaki, Shouichi; Nakamura, Norifumi; Iwase, Yoko; Nishihara, Kazuhide; Ohno, Sachi; Shimada, Masahiko; Sugiyama, Kazuna

2013-11-01

Difficult laryngoscopy and tracheal intubation are occasionally encountered in children with congenital anomalies or micrognathia. However, no study has elucidated anatomical etiology in relation to craniofacial development. Two hundred ten patients aged 8 months-18 years were analyzed. We analyzed the lateral cephalograms of: (i) eight patients in whom laryngoscopy was anticipated as difficult before anesthesia and who were unable to be intubated by direct laryngoscopy and needed fiberoptic bronchoscopy (group A); (ii) 11 patients in whom laryngoscopy was anticipated as difficult before anesthesia but who were able to be intubated by direct laryngoscopy (group B); and (iii) 191 patients in whom laryngoscopy was anticipated as easy before anesthesia and was actually found to be easy (group C). Eight cephalometric parameters were measured and age-parameter relationships were plotted. Logistic regression analysis was performed to characterize group A children for each of the cephalometric variables. Apparently insufficient growth of the mandible was observed in the group A children. Furthermore, the group A children of aged <4 years had undeveloped maxilla, longer mandibular plane-hyoid distances (≥1.3 cm), and deeper depth of the oropharynx; those of aged ≥4 years showed increased inclination of the mandible (sella-nasion plane to mandibular plane angle of ≥46.5°). Difficult laryngoscopy and tracheal intubation are expected in children aged <4 years with lower-positioned hyoid bone caused by caudal larynx as well as undeveloped maxilla and mandible, and in those aged ≥4 years with increased inclination of the mandible as well as undeveloped mandible. © 2013 John Wiley & Sons Ltd.

Significant Improvement in Chronic Persistent Headaches Caused by Small Rathke Cleft Cysts After Transsphenoidal Surgery.

PubMed

Fukui, Issei; Hayashi, Yasuhiko; Kita, Daisuke; Sasagawa, Yasuo; Oishi, Masahiro; Tachibana, Osamu; Nakada, Mitsutoshi

2017-03-01

Rathke cleft cysts (RCC) usually are asymptomatic and can be observed via the use of conservative methods. Some patients with RCCs, however, have severe headaches even if they are small enough to be confined to the sella, and these small RCCs seldom have been discussed. This study presents an investigation into clinical characteristics of small RCCs associated with severe headaches, demonstrating efficacy and safety of endoscopic transsphenoidal surgery (ETSS) to relieve headaches. In this study, 13 patients with small RCCs (maximum diameter <10 mm) who presented with headaches and were treated by ETSS at our institute from 2009 to 2014 were recruited. These RCCs were treated Headache Impact Test-6 (HIT-6) score was calculated both pre- and postoperatively to evaluate headache severity. All patients complained of severe headaches, which disturbed their daily life. Most headaches were nonpulsating and localized in the frontal area. Characteristically, 6 patients (46%) experienced severe headaches with sudden onset that continued chronically. HIT-6 score was 64 on average, meaning headaches affected daily life severely. After surgical decompression of the cyst, headache in all of the patients improved dramatically and HIT-6 score decreased significantly to 37, suggesting that headaches were diminished. No newly developed deficiencies of the anterior pituitary lobe function were detected. Postoperative occurrence of diabetes insipidus was found in 2 patients, both of which were transient. No recurring cysts were found. Severe headaches can develop from small RCCs. In the present study, ETSS was performed on such patients effectively and safely to relieve their headaches. Copyright © 2017 Elsevier Inc. All rights reserved.

A cadaveric study of the endoscopic endonasal transclival approach to the basilar artery.

PubMed

Lai, Leon T; Morgan, Michael K; Chin, David C W; Snidvongs, Kornkiat; Huang, June X Z; Malek, Joanne; Lam, Matthew; McLachlan, Rohan; Harvey, Richard J

2013-04-01

The anterior transclival route to basilar artery aneurysms is not widely performed. The objective of this study was to carry out a feasibility assessment of the transclival approach to basilar aneurysms with advanced endonasal techniques on 11 cadaver heads. Clival dura was exposed from the sella to the foramen magnum between the paraclival segments of the internal carotid arteries (ICA) laterally. An inverted dural "U" flap was reflected inferiorly to expose the basilar artery. The maximal dimensions from operative measurements were recorded. Surgical manoeuvrability of multiple instruments and the proficiency to place proximal and distal vascular clips were evaluated. The mean operative depth (± standard deviation), measured from the anterior choanae to the basilar artery, was 110±6mm. The lateral corridors were limited distally by the medial pterygoids (mean width 21±2mm) and paraclival ICA (mean width 20±2mm). The mean transclival craniectomy dimensions were 19±2mm (width) and 23±4mm (height). Exposure of the basilar-anterior inferior cerebellar artery junction, superior cerebellar artery, and the basilar caput were possible in 100%, 91%, and 64% of instances, respectively. Placements of proximal and distal aneurysm clips were achieved in all instances. Based on our findings, the transclival endoscopic endonasal surgery approach provides excellent visualisation of the basilar artery. Clip application and manoeuvrability of instruments was considered adequate for basilar aneurysm surgery. Surgical skills and instrumentation to control significant haemorrhage can potentially limit the clinical applicability of this technique. Crown Copyright © 2012. Published by Elsevier Ltd. All rights reserved.

Comparative study of different osteotomy modalities in maxillary distraction osteogenesis for cleft lip and palate.

PubMed

Yu, Hongbo; Wang, Xudong; Fang, Bing; Shen, Steve Guofang

2012-11-01

Conventional maxillary distraction osteogenesis and anterior maxillary segmental distraction were applied in the treatment of severe maxillary hypoplasia secondary to cleft clip and palate. The aim of the present study was to compare the difference between these 2 osteotomy modalities used for rigid external distraction. Ten patients with severe maxillary hypoplasia secondary to CLP were enrolled in our study. They were randomly divided into 2 groups. Conventional maxillary distraction osteogenesis was performed in 5 patients and anterior maxillary segmental distraction in 5 patients. The preoperative and postoperative lateral cephalograms were compared, and cephalometric analysis was performed. The independent sample t test was used to evaluate the differences between the 2 groups. All patients healed uneventfully, and the maxillae moved forward satisfactorily. The sella-nasion-point A angles, nasion-point A-Frankfort horizontal plane angles, overjets, and 0-meridian to subnasale distances had increased significantly after distraction osteogenesis. Significant differences were found in the changes in palatal length between the 2 groups (P < .05). A mean increase of 7.50 mm in palatal length was found in the anterior maxillary segmental distraction group. No significant difference in the changes in palatopharyngeal depth or soft palatal length was found. With the ability of increasing the palatal and arch length, avoiding changes in palatopharyngeal depth, and preserving palatopharyngeal closure function, anterior maxillary segmental distraction has great value in the treatment of maxillary hypoplasia secondary to CLP. It is a promising and valuable technique in this potentially complicated procedure. Copyright © 2012 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Syringohydromyelia in Patients with Chiari I Malformation: A Retrospective Analysis.

PubMed

Gad, K A; Yousem, D M

2017-09-01

The association of syringohydromyelia with Chiari I malformation has a wide range, between 23% and 80% of cases in the current literature. In our experience, this range might be overestimated compared with our observations in clinical practice. Because there is an impact of Chiari I malformation-associated syringohydromyelia on morbidity and surgical intervention, its diagnosis is critical in this patient population. Identifying related variables on the basis of imaging would also help identify those patients at risk of syrinx formation during their course of disease. We performed a retrospective analysis of the MR imaging studies of 108 consecutive cases of Chiari I malformation. A multitude of factors associated with syrinx formation were investigated, including demographic, morphometric, osseous, and dynamic CSF flow evaluation. Thirty-nine of 108 (36.1%) patients with Chiari I malformation had syringohydromyelia. On the basis of receiver operating characteristic curve analysis, a skull base angle (nasion-sella-basion) of 135° was found to be a statistically significant classifier of patients with Chiari I malformation with or without syringohydromyelia. Craniocervical junction osseous anomalies (OR = 4.3, P = .001) and a skull base angle of >135° (OR = 4.8, P = .0006) were most predictive of syrinx formation. Pediatric patients (younger than 18 years of age) who developed syringohydromyelia were more likely to have associated skull base osseous anomalies than older individuals ( P = .01). Our findings support evidence of the role of foramen magnum blockage from osseous factors in the development of syringohydromyelia in patients with Chiari I malformation. © 2017 by American Journal of Neuroradiology.

Endoscopic endonasal trans-sphenoid surgery of pituitary adenoma

PubMed Central

Yadav, YR; Sachdev, S; Parihar, V; Namdev, H; Bhatele, PR

2012-01-01

Endoscopic endonasal trans-sphenoid surgery (EETS) is increasingly used for pituitary lesions. Pre-operative CT and MRI scans and peroperative endoscopic visualization can provide useful anatomical information. EETS is indicated in sellar, suprasellar, intraventricular, retro-infundibular, and invasive tumors. Recurrent and residual lesions, pituitary apoplexy and empty sella syndrome can be managed by EETS. Modern neuronavigation techniques, ultrasonic aspirators, ultrasonic bone curette can add to the safety. The binostril approach provides a wider working area. High definition camera is much superior to three-chip camera. Most of the recent reports favor EETS in terms of safety, quality of life and tumor resection, hospital stay, better endocrinological, and visual outcome as compared to the microscopic technique. Nasal symptoms, blood loss, operating time are less in EETS. Various naso-septal flaps and other techniques of CSF leak repair could help reduce complications. Complications can be further reduced after achieving the learning curve, good understanding of limitations with proper patient selection. Use of neuronavigation, proper post-operative care of endocrine function, establishing pituitary center of excellence and more focused residency and endoscopic fellowship training could improve results. The faster and safe transition from microscopic to EETS can be done by the team concept of neurosurgeon/otolaryngologist, attending hands on cadaveric dissection, practice on models, and observation of live surgeries. Conversion to a microscopic or endoscopic-assisted approach may be required in selected patients. Multi-modality treatment could be required in giant and invasive tumors. EETS appears to be a better surgical option in most pituitary adenoma. PMID:23188987

Preoperative clonidine use in trans-sphenoidal pituitary adenoma surgeries - a randomized controlled trial.

PubMed

Bajaj, Jitin; Mittal, Radhe Shyam; Sharma, Achal

2017-02-01

Pituitary masses are common lesions accounting for about 15-20% of all brain tumours. Oozing blood is an annoyance in microscopic sublabial trans-sphenoidal approach for these masses. There have been many ways of reducing the ooze, having their own pros and cons. To find out the efficacy and safety of clonidine in reducing blood loss in pituitary adenoma surgery through a randomized masked trial. It was a prospective randomized controlled trial done. Total 50 patients of pituitary adenomas were randomized into two groups. Group A (25 patients) was given 200 μg clonidine orally, while Group B (25 patients) was given placebo. Surgeon, anaesthesiologist and patient were blinded for the trial. Sublabial trans-septal trans-sphenoidal approach to sella and excision of mass was performed in each patient. Patients were studied for pre-, intra- and post-operative blood pressure and heart rate, pre- and post-operative imaging findings, intra-operative blood loss, bleeding grading by surgeon, surgeon's satisfaction about condition of specific part and quality of surgical field, operative time and extent of resection. Blood loss during the surgery, operative time and bleeding grading by the surgeon were found significantly less in the clonidine group, while quality of surgical field, condition of the specific part and extent of resection were found significantly better in the clonidine group (p value <.05). There was no untoward adverse effect of the drug in the test group. Clonidine is a safe and effective drug to reduce bleeding in trans-sphenoidal microscopic pituitary adenoma surgeries.

A practical 3D printed simulator for endoscopic endonasal transsphenoidal surgery to improve basic operational skills.

PubMed

Wen, Guodao; Cong, ZiXiang; Liu, KaiDong; Tang, Chao; Zhong, Chunyu; Li, Liwen; Dai, XuJie; Ma, Chiyuan

2016-06-01

We aimed to present a practical three-dimensional (3D) printed simulator to comprehensively and effectively accelerate the learning curve of endoscopic endonasal transsphenoidal surgery (EETS). The 3D printed simulator consists of three parts: (1) skull frame, (2) the nasal passage and the nasal alar of the face, and (3) a modified sella turcica. We aimed to improve three basic operational skills of surgeons: drilling, curetting, and aspirating. Eighteen neurosurgeons and five post-graduates were recruited and consented for the training. For trainees, (1) as the training progressed, the scores increased gradually, (2) a significant increase in the average scores was observed in the tenth training compared to the first training, and (3) there is a significant decrease in trainee variability in the shortening of the gap. The 18 neurosurgeons were divided into three groups: experts, assistants, and observers. For all three basic operations, (1) the average score of experts was obviously higher than that of the assistants, observers, and trainees' tenth training and (2) the average scores of assistants and observers were obviously higher than that of trainees' first training. A significant high in the average score between the assistants and the observers was seen for aspirating, but not for drilling or curetting. For curetting and aspirating, the tenth training average score of trainees was obviously higher than that of assistants and observers. This 3D printed simulator allows different endoscopic basic operations to be simulated and improves the EETS techniques of surgeons. We believed it to be a practical, simple, and low-cost simulator.

Pituitary abscess following transsphenoidal surgery: the experience of 12 cases from a single institution.

PubMed

Wang, Lei; Yao, Yong; Feng, Feng; Deng, Kan; Lian, Wei; Li, Guilin; Wang, Renzhi; Xing, Bing

2014-09-01

To explore possible reasons for the incidence of a pituitary abscess following transsphenoidal surgery and determine the most effective treatment. A series of 12 patients who had undergone transsphenoidal surgery in other hospitals before being treated at Peking Union Medical College Hospital were reviewed. The presence of a pituitary abscess was confirmed when pus was intraoperatively observed within the sella turcica. All patients were treated with debridement of the abscess, nine among whom through a transsphenoidal approach and the other three via a craniotomy, followed by antibiotic treatment and hormone replacement therapy. The mean follow-up time was 27.0 months (range from 3.0 to 79.0 months). Headache (92%), panhypopituitarism (58%) and visual disturbance (50%) were the most common clinical indicators of a pituitary abscess. Imaging tests demonstrated a pituitary mass in all patients, with seven (58%) manifested with typical magnetic resonance features of an abscess. Ten patients (83%) were correctly diagnosed preoperatively. During surgical exploration, six presented with severe inflammation or an abscess within the sphenoidal sinus. Causative organisms were identified in five patients (42%). After surgical and antibiotic therapies, all patients fully recovered except for two presenting with severe visual impairment. Six patients (50%) required hormone replacement therapy. Retrograde infection from the sphenoid sinus may be a vital mechanism underlying the formation of a pituitary abscess following transsphenoidal surgery. Debridement of the abscess through surgical approaches combined with antibiotic treatment has been found to yield positive outcomes. Copyright © 2014 Elsevier B.V. All rights reserved.

Usefulness of the dynamic gadolinium-enhanced magnetic resonance imaging with simultaneous acquisition of coronal and sagittal planes for detection of pituitary microadenomas.

PubMed

Lee, Han Bee; Kim, Sung Tae; Kim, Hyung-Jin; Kim, Keon Ha; Jeon, Pyoung; Byun, Hong Sik; Choi, Jin Wook

2012-03-01

Does dynamic gadolinium-enhanced imaging with simultaneous acquisition of coronal and sagittal planes improve diagnostic accuracy of pituitary microadenomas compared with coronal images alone? Fifty-six patients underwent 3-T sella MRI including dynamic simultaneous acquisition of coronal and sagittal planes after gadolinium injection. According to conspicuity, lesions were divided into four scores (0, no; 1, possible; 2, probable; 3, definite delayed enhancing lesion). Additional information on supplementary sagittal images compared with coronal ones was evaluated with a 4-point score (0, no; 1, possible; 2, probable; 3, definite additional information). Accuracy of tumour detection was calculated. Average scores for lesion detection of a combination of two planes, coronal, and sagittal images were 2.59, 2.32, and 2.18. 6/10 lesions negative on coronal images were detected on sagittal ones. Accuracy of a combination of two planes, of coronal and of sagittal images was 92.86%, 82.14% and 75%. Six patients had probable or definite additional information on supplementary sagittal images compared with coronal ones alone (10.71%). Dynamic MRI with combined coronal and sagittal planes was more accurate for detection of pituitary microadenomas than routinely used coronal images. Simultaneous dynamic enhanced acquisition can make study time fast and costs low. We present a new dynamic MRI technique for evaluating pituitary microadenomas • This technique provides simultaneous acquisition of contrast enhanced coronal and sagittal images. • This technique makes the diagnosis more accurate and reduces the examination time. • Such MR imaging only requires one single bolus of contrast agent.

[Tyrosine hydroxylase in telencephalon and diencephalon of Rhodeus sericeus (Cyprinidae)].

PubMed

Pushchina, E V

2009-01-01

Immunohistochemical labeling of tyrosine hydroxylase was used to demonstrate catecholaminergic neuronal populations in the telencephalon and diencephalonof adult cypryniform fish Rhodeus sericeus. Various immunoreactive cell populations have been found in the telencephalon (ventral, central and lateral nuclei of ventral telencephalic area). Immunoreactive cells and fibers were discovered in dorsal nucleus of ventral telencephalic area and supracomissural nucleus in the caudal part of the telencephalon. In the diencephalon, periventricular nuclei (preoptic, periventricular nucleus of posterior tuberculum and periventricular organ) contained considerable TH-ergic cells. High activity of tyrosine hydroxylase was revealed in the pretectal, ventro-medial, ventro-lateral and suprachiasmatic nuclei. Periventricular hypothalamic nuclei also displayed high activity of tyrosine hydroxylase. Pseudounipolar neurons prevailed in all TH-immunereactive structures of the telencephalon and diencephalon: numerous bipolar liquor-contacting cells were discovered in the periventricular nuclei. Large pear-shaped cells and bipolar TH-ergic cells were found in posterior tuberculum. These cells may be functionally related to the dopamine-acquiring system.

Extreme elevation of intrasellar pressure in patients with pituitary tumor apoplexy: relation to pituitary function.

PubMed

Zayour, Dany H; Selman, Warren R; Arafah, Baha M

2004-11-01

The dominant mechanism for hypopituitarism and hyperprolactinemia commonly observed in patients with pituitary macroadenomas was postulated to be increased intrasellar pressure (ISP) caused by the slow and gradual expansion of adenomas within the sella turcica. Hemorrhagic infarction of adenomas (pituitary tumor apoplexy) is associated with a rapid, rather than gradual, increase in intrasellar contents. The impacts of the sudden increase in intrasellar contents on ISP and pituitary function are unknown. ISP and pituitary function were determined in 13 patients with pituitary tumor apoplexy who had surgical decompression within 1 wk of symptoms' onset. ISP measurements were remarkably high (median, 47 mm Hg), whereas serum prolactin (PRL) concentrations were generally low (median, 3.5 microg/liter). There was an inverse correlation (r = -0.76; P < 0.01) between ISP measurements and serum PRL concentrations. Postoperatively, partial recovery or maintenance of pituitary function was noted in seven of 13 patients. These seven patients had higher (P = 0.013) serum PRL levels (9.3 +/- 7.4 microg/liter) and lower (P < 0.001) ISP measurements (35.9 +/- 7.3 mm Hg) than the respective values in the remaining six with persistent postoperative hypopituitarism (1.6 +/- 0.6 microg/liter and 55.9 +/- 2.4 mm Hg, respectively). The low serum PRL levels in patients with tumor apoplexy suggested that ischemic necrosis of the anterior pituitary resulting from sudden and extreme elevation of ISP was commonly observed in this setting. A normal or elevated serum PRL level in patients with non-PRL-secreting macroadenomas indicates the presence of viable pituitary cells and the high likelihood of postoperative recovery of pituitary function.

History of endonasal skull base surgery.

PubMed

Wang, Amy J; Zaidi, Hasan A; Laws, Edward D

2016-12-01

While the endonasal approach to the skull base continues to advance, this paper invokes its long history. The centuries of medieval neuroanatomy and early neurosurgery enabled the conception of the first transfacial approaches in the late 1800s; Henry Schloffer performed the first transsphenoidal surgery in 1907. Although the procedure was initially met with much interest, Harvey Cushing eventually led the field of neurosurgery to abandon the transsphenoidal approach in the 1920s. The following three generations of neurosurgeons contained several key figures including Norman Dott, Gerard Guiot, and Jules Hardy who were steadfast in preserving the technique as well as in addressing its shortcomings. The endoscopic approach developed simultaneously, and advances in magnifying and fiberoptics further resolved limitations previously inherent to the transsphenoidal approach. At last, in the 1960s, the transsphenoidal approach entered its renaissance. Today, the momentum of its development persists in the endoscopic endonasal approach, which has recently expanded the indications for transsphenoidal surgery across the skull base, far beyond its original jurisdiction of the sella. Continued progress must not take for granted the rich history of the transsphenoidal approach, which was developed over centuries by surgeons around the world. The authors present the evolution of modern endonasal surgery as a dynamic interplay between technology, medicine, and surgery over the past 100 years. Progress can be attributed to courageous surgeons who affirmed their contemporary practices despite gaps in technology or medicine, and to visionary individuals who produced and incorporated new elements into transsphenoidal surgery. And so while the new endoscopic technique brings forth new challenges, its development reaffirms the principles laid down by the pioneers of transsphenoidal surgery.

Ectopic prolactin secretion from a perivascular epithelioid cell tumor (PEComa).

PubMed

Korytnaya, Evgenia; Liu, Jiayan; Camelo-Piragua, Sandra; Sullivan, Stephen; Auchus, Richard J; Barkan, Ariel

2014-11-01

The diagnosis of ectopic pituitary hormone secretion requires abnormally high circulating hormone levels, absence of a pituitary tumor, and localization of the hormone in question to the extrapituitary malignant neoplasm. No case of a malignant solid tumor producing prolactin has been documented thus far. A 47-year-old woman presented with amenorrhea and galactorrhea of 3-year duration. Serum prolactin ranged from 300 to > 900 ng/mL, and other pituitary and thyroid indices were normal, including testing for macroprolactinemia. Pituitary magnetic resonance imaging revealed a partially empty sella but no tumor. Cabergoline 0.5 mg twice weekly did not affect her prolactinemia (1700 to 1900 ng/mL), and the medication was stopped. In the meantime, she developed abdominal pain, and a computed tomography scan showed a 17 × 13 × 8-cm mass abutting the distal stomach, proximal duodenum, and right colon. After the tumor was excised, her galactorrhea resolved, menstrual periodicity resumed within the first month, and serum prolactin fell to 5 ng/mL. Pathological examination of the excised tumor was consistent with perivascular epithelioid cell tumor. Between 5 and 10% of the tumor cells were strongly positive for prolactin on immunohistochemistry. RT-PCR detected prolactin mRNA in the tumor cell extract, confirming the diagnosis of ectopic prolactin synthesis and secretion. We present the first example of massive and symptomatic hyperprolactinemia due to ectopic prolactin production by a solid extrapituitary mesenchymal tumor confirmed with both mRNA analysis and immunohistochemistry. Ectopic prolactin secretion should be suspected in patients with a prolactin >200 ng/mL and negative sellar MRI.

Factors influencing superimposition error of 3D cephalometric landmarks by plane orientation method using 4 reference points: 4 point superimposition error regression model.

PubMed

Hwang, Jae Joon; Kim, Kee-Deog; Park, Hyok; Park, Chang Seo; Jeong, Ho-Gul

2014-01-01

Superimposition has been used as a method to evaluate the changes of orthodontic or orthopedic treatment in the dental field. With the introduction of cone beam CT (CBCT), evaluating 3 dimensional changes after treatment became possible by superimposition. 4 point plane orientation is one of the simplest ways to achieve superimposition of 3 dimensional images. To find factors influencing superimposition error of cephalometric landmarks by 4 point plane orientation method and to evaluate the reproducibility of cephalometric landmarks for analyzing superimposition error, 20 patients were analyzed who had normal skeletal and occlusal relationship and took CBCT for diagnosis of temporomandibular disorder. The nasion, sella turcica, basion and midpoint between the left and the right most posterior point of the lesser wing of sphenoidal bone were used to define a three-dimensional (3D) anatomical reference co-ordinate system. Another 15 reference cephalometric points were also determined three times in the same image. Reorientation error of each landmark could be explained substantially (23%) by linear regression model, which consists of 3 factors describing position of each landmark towards reference axes and locating error. 4 point plane orientation system may produce an amount of reorientation error that may vary according to the perpendicular distance between the landmark and the x-axis; the reorientation error also increases as the locating error and shift of reference axes viewed from each landmark increases. Therefore, in order to reduce the reorientation error, accuracy of all landmarks including the reference points is important. Construction of the regression model using reference points of greater precision is required for the clinical application of this model.

Endoscopic Endonasal Surgery for Remission of Cushing Disease Caused by Ectopic Intracavernous Macroadenoma: Case Report and Literature Review.

PubMed

Koutourousiou, Maria; Winstead, Welby I

2017-02-01

Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES). A 36-year-old woman presented with Cushing syndrome. Increased ACTH, serum cortisol, and free urine cortisol levels were identified; however, pituitary magnetic resonance imaging failed to show a pituitary tumor; instead, a parasellar lesion in the left cavernous sinus (CS) was noticed. Inferior petrosal sinus sampling showed a significant central to peripheral and lateralized left-sided ACTH gradient. The patient underwent EES. No tumor was found in the sella; however, the left CS was widely explored and a tumor was found lateral to the paraclival segment of the carotid artery. There were no complications after EES. Pathology confirmed the diagnosis of an ACTH-secreting adenoma. During the immediate postoperative course, serum cortisol levels decreased lower than 5 μg/dL. Postoperative magnetic resonance imaging showed complete tumor resection. At 20 months follow-up, the patient remained in clinical and biochemical remission of Cushing disease. Only 12 cases of ectopic intracavernous ACTH-secreting adenomas have been reported and all were microadenomas. The presence of an ectopic ACTH-secreting macroadenoma in the CS represents a surgical challenge. EES is the ideal approach for complete resection of ectopic intracavernous adenomas, allowing for a wide exploration of the CS with no surgical complications. Copyright © 2016 Elsevier Inc. All rights reserved.

Multidimensional esthetic evaluation of patients with a cleft lip and palate wearing a maxillary partial removable dental prosthesis: A 5-year retrospective study.

PubMed

Yu, Dedong; Xing, Guofang; Nie, Ping; Zhang, Xiuyin; Steve Shen, Guofang

2016-04-01

No published quantitative or qualitative studies are available of the 2-dimensional (2D) and 3D esthetic evaluation of patients with a cleft lip and palate (CLP) wearing a maxillary partial removable dental prosthesis (MPRDP). The purpose of this retrospective clinical study was to qualitatively and quantitatively evaluate the facial esthetic improvements of patients with CLP wearing an MPRDP for 5 years by using 2D cephalometric and 3D photogrammetry methods. Six patients, 2 men and 4 women, with CLP deformity were recruited and treated with an MPRDP by the same dentist over 5 years. Results of the clinical examination were recorded before and after wearing the MPRDP. Sella-nasion-A (SNA) point, the U1-SN, 0-meridian to SN, nasolabial angle, and maxillary incisor exposure were measured by 2D cephalometric analysis before and after wearing the MPRDP. 3D photographs were captured by 3dMD software. Geomagic Spark Studio software was also used to measure the patients' improved upper lip projection after the MPRDP was worn. The paired sample t test was used to compare the participants (α=.05). U1-SN (P<.05) and 0-meridian to SN (P<.01) had significant deviation statistically. After patients underwent clinical examination, SNA and nasiolabial angles were found to have improved, although no statistical significance was observed. A maxillary partial removable dental prosthesis (MPRDP) can advance the upper lip forward, restoring the subnasal and upper lip projection. Patients' nasiolabial angles with MPRDPs were decreased, which led to a more harmonious facial contour. Copyright © 2016 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

Preoperative Assessment of Craniopharyngioma Adherence: Magnetic Resonance Imaging Findings Correlated with the Severity of Tumor Attachment to the Hypothalamus.

PubMed

Prieto, Ruth; Pascual, José M; Rosdolsky, Maria; Barrios, Laura

2018-02-01

Craniopharyngioma (CP) adherence represents a heterogeneous pathologic feature that critically influences the potentially safe and radical resection. The aim of this study was to define the magnetic resonance imaging (MRI) predictors of CP adherence severity. This study retrospectively investigated a cohort of 200 surgically treated CPs with their corresponding preoperative conventional MRI scans. MRI findings related to the distortions of anatomic structures along the sella turcica-third ventricle axis caused by CPs, in addition to the tumor's shape and calcifications, were analyzed and correlated with the definitive type of CP adherence observed during the surgical procedures. CP adherence is defined by 3 components, as follows: 1) the specific structures attached to the tumor, 2) the adhesion's extent, and 3) its strength. Combination of these 3 components determines 5 hierarchical levels of adherence severity with gradually increasing surgical risk of hypothalamic injury. Multivariate analysis identified 4 radiologic variables that allowed a correct overall prediction of the levels of CP adherence severity in 81.5% of cases: 1) the position of the hypothalamus in relation to the tumor-the most discriminant factor; 2) the type of pituitary stalk distortion; 3) the tumor shape; and 4) the presence of calcifications. A binary logistic regression model including the first 3 radiologic variables correctly identified the CPs showing the highest level of adherence severity (severe/critical) in almost 90% of cases. A position of the hypothalamus around the middle portion of the tumor, an amputated or infiltrated appearance of the pituitary stalk, and the elliptical shape of the tumor are reliable predictors of strong and extensive CP adhesions to the hypothalamus. Copyright © 2017 Elsevier Inc. All rights reserved.

Pituitary imaging findings in male patients with hypogonadotrophic hypogonadism.

PubMed

Hirsch, Dania; Benbassat, Carlos; Toledano, Yoel; S'chigol, Irena; Tsvetov, Gloria; Shraga-Slutzky, Ilana; Eizenberg, Yoav; Shimon, Ilan

2015-08-01

Data on pituitary imaging in adult male patients presenting with hypogonadotrophic hypogonadism (HH) and no known pituitary disease are scarce. To assess the usefulness of pituitary imaging in the evaluation of men presenting with HH after excluding known pituitary disorders and hyperprolactinemia. A historical prospective cohort of males with HH. Men who presented for endocrine evaluation from 2011 to 2014 with testosterone levels <10.4 nmol/L (300 ng/mL), normal LH and FSH levels and no known pituitary disease. Seventy-five men were included in the analysis. Their mean age and BMI were 53.4 ± 14.8 years and 30.7 ± 5.2 kg/m2, respectively. Mean total testosterone, LH, and FSH were 6.2 ± 1.7 nmol/L, 3.4 ± 2 and 4.7 ± 3.1 mIU/L, respectively. Prolactin level within the normal range was obtained in all men (mean 161 ± 61, range 41-347 mIU/L). Sixty-two men had pituitary MRI and 13 performed CT. In 61 (81.3%) men pituitary imaging was normal. Microadenoma was found in 8 (10.7%), empty sella and thickened pituitary stalk in one patient (1.3%) each. In other four patients (5.3%) a small or mildly asymmetric pituitary gland was noted. No correlation was found between testosterone level and the presence of pituitary anomalies. This study suggests that the use of routine hypothalamic-pituitary imaging in the evaluation of IHH, in the absence of clinical characteristics of other hormonal loss or sellar compression symptoms, will not increase the diagnostic yield of sellar structural abnormalities over that reported in the general population.

Topographic Diagnosis of Craniopharyngiomas: The Accuracy of MRI Findings Observed on Conventional T1 and T2 Images.

PubMed

Prieto, R; Pascual, J M; Barrios, L

2017-11-01

The topography of craniopharyngiomas has proved fundamental in predicting the involvement of vital brain structures and the possibility of achieving a safe radical resection. Beyond the imprecise term "suprasellar," indiscriminately used for craniopharyngiomas, an accurate definition of craniopharyngioma topography should be assessed by preoperative MR imaging. The objective of this study was to investigate the MRI findings that help define craniopharyngioma topography. This study retrospectively investigated a cohort of 200 surgically treated craniopharyngiomas with their corresponding preoperative midsagittal and coronal conventional T1- and T2-weighted MR images, along with detailed descriptions of the surgical findings. Radiologic variables related to the occupation of the tumor of intracranial compartments and the distortions of anatomic structures along the sella turcica-third ventricle axis were analyzed and correlated with the definitive craniopharyngioma topography observed during the surgical procedures. A predictive model for craniopharyngioma topography was generated by multivariate analysis. Five major craniopharyngioma topographies can be defined according to the degree of hypothalamic distortion caused by the tumor: sellar-suprasellar, pseudointraventricular, secondary intraventricular, not strictly intraventricular, and strictly intraventricular. Seven key radiologic variables identified on preoperative MRI allowed a correct overall prediction of craniopharyngioma topography in 86% of cases: 1) third ventricle occupation, 2) pituitary stalk distortion, 3) relative level of the hypothalamus in relation to the tumor, 4) chiasmatic cistern occupation, 5) mammillary body angle, 6) type of chiasm distortion, and 7) tumor shape. Systematic assessment of these 7 variables on conventional preoperative T1 and T2 MRI is a useful and reliable method to ascertain individual craniopharyngioma topography. © 2017 by American Journal of Neuroradiology.

Management of Severely Atrophic Maxilla in Ectrodactyly Ectodermal Dysplasia-cleft Syndrome

PubMed Central

Rachmiel, Adi; Emodi, Omri; Aizenbud, Dror; Shilo, Dekel

2018-01-01

Background: Ectrodactyly ectodermal dysplasia-cleft syndrome is a rare genetic syndrome with an incidence of 1/90,000 live births, characterized by cleft lip and palate, severely hypoplastic maxilla, and hypodontia. Patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome suffer from a severely hypoplastic maxilla that is highly difficult to treat using traditional orthognathic methods. In this study, we propose using distraction osteogenesis to achieve a major advancement while maintaining good stability and minimal relapse. To our knowledge, this is the first description of patients with this syndrome treated using distraction osteogenesis. Methods: Five patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome were included in the study. All patients had been operated on according to the well-established protocol of cleft lip and palate reconstruction before maxillary distraction osteogenesis. Hard and soft-tissue changes were evaluated by cone beam computed tomography and lateral cephalograms before distraction osteogenesis (T1), at the postdistraction point (T2) and after 1 year of follow-up (T3). Results: Examination revealed marked maxillary advancement in all our patients with a significant mean difference in hard tissue parameters (condylion to A point = 18 mm; nasion-sella line to A point = 15.2 degrees) and a notable improvement in facial convexity (20.9 degrees). One year follow-up measurements demonstrated mild relapse rates of 6% in the horizontal plane. Conclusions: We conclude that despite the challenging anatomic and physiological features of ectrodactyly ectodermal dysplasia-cleft patients, by enhancing current surgical techniques, there is promising potential for improved patient outcomes, achieving normognathic facial appearance with implant supported rehabilitation. PMID:29616174

The posterior nasoseptal flap: A novel technique for closure after endoscopic transsphenoidal resection of pituitary adenomas

PubMed Central

Barger, James; Siow, Matthew; Kader, Michael; Phillips, Katherine; Fatterpekar, Girish; Kleinberg, David; Zagzag, David; Sen, Chandranath; Golfinos, John G.; Lebowitz, Richard; Placantonakis, Dimitris G.

2018-01-01

Background: While effective for the repair of large skull base defects, the Hadad-Bassagasteguy nasoseptal flap increases operative time and can result in a several-week period of postoperative crusting during re-mucosalization of the denuded nasal septum. Endoscopic transsphenoidal surgery for pituitary adenoma resection is generally not associated with large dural defects and high-flow cerebrospinal fluid (CSF) leaks requiring extensive reconstruction. Here, we present the posterior nasoseptal flap as a novel technique for closure of skull defects following endoscopic resection of pituitary adenomas. This flap is raised in all surgeries during the transnasal exposure using septal mucoperiosteum that would otherwise be discarded during the posterior septectomy performed in binostril approaches. Methods: We present a retrospective, consecutive case series of 43 patients undergoing endoscopic transsphenoidal resection of a pituitary adenoma followed by posterior nasoseptal flap placement and closure. Main outcome measures were extent of resection and postoperative CSF leak. Results: The mean extent of resection was 97.16 ± 1.03%. Radiographic measurement showed flap length to be adequate. While a defect in the diaphragma sellae and CSF leak were identified in 21 patients during surgery, postoperative CSF leak occurred in only one patient. Conclusions: The posterior nasoseptal flap provides adequate coverage of the surgical defect and is nearly always successful in preventing postoperative CSF leak following endoscopic transsphenoidal resection of pituitary adenomas. The flap is raised from mucoperiosteum lining the posterior nasal septum, which is otherwise resected during posterior septectomy. Because the anterior septal cartilage is not denuded, raising such flaps avoids the postoperative morbidity associated with the larger Hadad-Bassagasteguy nasoseptal flap. PMID:29527390

Is further evaluation for growth hormone (GH) deficiency necessary in fibromyalgia patients with low serum insulin-like growth factor (IGF)-I levels?

PubMed

Yuen, Kevin C J; Bennett, Robert M; Hryciw, Cheryl A; Cook, Marie B; Rhoads, Sharon A; Cook, David M

2007-02-01

Fibromyalgia (FM) is characterized by diffuse pain, fatigue, and sleep disturbances; symptoms that resemble the adult growth hormone (GH) deficiency syndrome. Many FM patients have low serum GH levels, with a hypothesized aetiology of dysregulated GH/insulin-like growth factor (IGF)-I axis. The aim of this study was to assess the GH reserve in FM patients with low serum IGF-I levels using the GH-releasing hormone (GHRH)-arginine test. We retrospectively reviewed the GHRH-arginine data of 77 FM patients with low serum IGF-I levels referred to our tertiary unit over a 4-year period. Of the 77 FM patients, 13 patients (17%) failed the GHRH-arginine test. Further evaluation with pituitary imaging revealed normal pituitary glands (n=7), coincident microadenomas (n=4), empty sella (n=1) and pituitary cyst (n=1), and relevant medical histories such as previous head injury (n=4), Sheehan's syndrome (n=1), and whiplash injury (n=1). In contrast, the remaining 64 patients (83%) that responded to the GHRH-arginine test demonstrated higher peak GH levels compared to age and BMI-matched controls (n=24). Our data shows that a subpopulation of FM patients with low serum IGF-I levels will fail the GHRH-arginine test. We, thus, recommend that the GH reserve of these patients should be evaluated further, as GH replacement may potentially improve the symptomatology of those with true GH deficiency. Additionally, the increased GH response rates to GHRH-arginine stimulation in the majority of FM patients with low serum IGF-I levels further supports the hypothesis of a dysregulated GH/IGF-I axis in the pathophysiology of FM.

Can commonly used profile planes be used to evaluate changes in lower lip position?

PubMed

Buschang, Peter H; Fretty, Kimberly; Campbell, Phillip M

2011-07-01

To determine the validity of five profile planes commonly used to describe the horizontal changes of the lower lip during orthodontic treatment. Pretreatment and posttreatment cephalograms of 79 patients (12.4 ± 2.8 years of age) were evaluated. Lower lip (labrale inferiorus) changes over time were measured relative to the Rickett's E-line, Steiner's S1-line, Burstone's B-line, Sushner's S2-line, and Holdaway's H-line. As an independent measure of actual horizontal lip changes, the labrale inferiorus was measured relative to a stable reference plane registered on the sella and oriented on the SN-7°. The lower lip actually moved anteriorly 2.35 ± 3.35 mm during orthodontic treatment; the five profile planes indicated that the lower lip moved to a more retrusive, posterior position. The five profile planes also showed no statistically significant sex differences in terms of the treatment changes that occurred, while the actual lip changes showed that males exhibited significantly greater changes than females. Actual treatment changes showed that the lower lip moved to a more protrusive position with nonextraction than with extraction treatments, changes that were not evident based on the five profile lines. While lip changes based on the five profile planes demonstrated moderately high to high intercorrelations ranging from 0.81 to 0.97, they showed only weak correlations (r < .35) with the actual horizontal changes of the labrale inferiorus. While all five planes measured similar aspects of positional change, none of them closely reflected the actual lower lip changes that occurred. These planes should not be used to measure changes in lip position that occur during treatment.

Cleft-Related Orthognathic Surgery Based on Maxillary Vertical Lengthening of the Anterior Facial Height.

PubMed

Jeong, Woo Shik; Jeong, Hyung Hwa; Kwon, Soon Man; Koh, Kyung S; Choi, Jong Woo

2018-03-01

The conventional approach during orthognathic surgery for cleft-related deformities has focused largely on restoration of maxilla retrusion, using the maxillary advancement as a standard treatment objective. However, the authors thought that maxillary vertical shortening and deficient incisor show could be additional key elements of cleft-related deformities. Although slight vertical lengthening can be obtained with only maxillary advancement, it would not be enough to obtain satisfactory aesthetic results in terms of the anterior facial height. The authors hypothesized that vertical deficiency and anteroposterior maxillary retrusion exists in cleft-related deformities. Therefore, orthognathic surgery including intentional vertical lengthening and advancement would be better than the conventional simple advancement. A new approach was used to treat consecutive patients with dentofacial deformities between December of 2007 and December of 2016. Patients with cleft-related dentofacial deformities were included in the study. Forty-one patients with cleft-related dentofacial deformities were included. The distance from the nasion to the incisor tip was 76.70 mm; the angle of the lines connecting the sella, nasion, and point A was 74.03 degrees; and the incisor show was 1.02 mm. Corresponding measurements in 40 patients without clefts were 81.57 mm, 80.08 degrees, and 3.38 mm, respectively. Postoperatively, the average vertical lengthening achieved was 0.88 mm in the simple maxillary advancement group and 7.65 mm in the intentional vertical lengthening accompanied by maxillary advancement group. The authors' results suggest that simultaneous maxillary vertical lengthening with horizontal advancement is a viable approach to orthognathic surgery for cleft-related deformities, given the long-term stability of outcomes demonstrated in this study. Therapeutic, III.

Antibodies to pituitary surface antigens during various pituitary disease states.

PubMed

Keda, Y M; Krjukova, I V; Ilovaiskaia, I A; Morozova, M S; Fofanova, O V; Babarina, M B; Marova, E I; Pankov, Y A; Kandror, V I

2002-11-01

Autoantibodies to cell surface antigens of human somatotropinoma (ASAS), human prolactinoma (ASAP) and rat adenohypophysis (ASARA) were assayed in the serum of patients with pituitary diseases associated with GH deficiency (GHD), such as pituitary dwarfism and primary empty sella syndrome (ESS), and in the serum of patients with hyperprolactinaemia of different etiologies: idiopathic hyperprolactinaemia, prolactinoma and ESS. The investigation was carried out with a cellular variant of an ELISA. Among children with GHD, the highest percentage of antibody-positive patients was found in the group with idiopathic isolated GHD (89% of ASAS(+) patients and 30% of ASARA(+) patients vs 33.3% and 0% respectively in the group with idiopathic combined pituitary hormone deficiency, and 33.3% and 9% in patients with pituitary hypoplasia associated with isolated GHD or combined pituitary hormone deficiency). Among hyperprolactinaemic patients, the highest ASAP and ASARA frequency was observed in patients with idiopathic hyperprolactinaemia (67.7% and 41.9% respectively) where it was twice as high as in the group of patients with prolactinoma. The proportion of ASAS(+) and ASARA(+) did not differ significantly between the groups of patients with ess with or without GHD. Similarly, there was no significant difference between the number of ESS ASAP(+) and ASARA(+) patients with or without hyperprolactinaemia. The data obtained suggested that autoimmune disorders may be primary, and responsible, at least in part, for pituitary dysfunction in the cases of idiopathic isolated GHD and idiopathic hyperprolactinaemia. At the same time, the autoimmune disorders in the patients with prolactinoma or ESS are probably secondary to the organic pituitary lesion and their significance in the development of the pituitary dysfunction is obscure.

Brain imaging in normal kids: a community-based MRI study in Malawian children.

PubMed

Potchen, M J; Kampondeni, S D; Mallewa, M; Taylor, T E; Birbeck, G L

2013-04-01

To collect normative MRI data for effective clinical and research applications. Such data may also offer insights into common neurological insults. We identified a representative, community-based sample of children aged 9-14 years. Children were screened for neurodevelopmental problems. Demographic data, medical history and environmental exposures were ascertained. Eligible children underwent the Neurologic Examination for Subtle Signs (NESS) and a brain MRI. Descriptive findings and analyses to identify risk factors for MRI abnormalities are detailed. One hundred and two of 170 households screened had age-appropriate children. Two of 102 children had neurological problems - one each with cerebral palsy and epilepsy. Ninety-six of 100 eligible children were enrolled. Mean age was 11.9 years (SD 1.5), and 43 (45%) were boys. No acute MRI abnormalities were seen. NESS abnormalities were identified in 6 of 96 children (6%). Radiographic evidence of sinusitis in 29 children (30%) was the most common MRI finding. Brain abnormalities were found in 16 (23%): mild diffuse atrophy in 4 (4%), periventricular white matter changes/gliosis in 6 (6%), multifocal punctuate subcortical white matter changes in 2 (2%), vermian atrophy in 1 (1%), empty sella in 3 (3%) and multifocal granulomas with surrounding gliosis in 1 (1%). Having an abnormal MRI was not associated with age, sex, antenatal problems, early malnutrition, febrile seizures, an abnormal neurological examination or housing quality (all P values >0.05). No predictors of radiographic sinusitis were identified. Incidental brain MRI abnormalities are common in normal Malawian children. The incidental atrophy and white matter abnormalities seen in this African population have not been reported among incidental findings from US populations, suggesting Malawi-specific exposures may be the cause. © 2013 Blackwell Publishing Ltd.

Re-evaluating TTF-1 immunohistochemistry in diffuse gliomas: Expression is clone-dependent and associated with tumor location.

PubMed

Pratt, Drew; Afsar, Nina; Allgauer, Michael; Fetsch, Patricia; Palisoc, Maryknoll; Pittaluga, Stefania; Quezado, Martha

TTF-1 is widely used as a marker in routine surgical pathology in the work-up of malignancy. Aberrant expression of TTF-1 in extrapulmonary and extrathyroidal malignancies is a frequently reported phenomenon. In addition to the recently characterized pituicyte-derived tumors of the sella, immunoreactivity has been reported in diffuse gliomas with the SPT24 clone. Here, we sought to evaluate TTF-1 expression with three commercially available clones in a large series of gliomas. Expression was compared across the newly defined diagnostic entities in the 2016 WHO Classification of CNS Tumors. Using tissue microarrays (TMA), 212 diffuse gliomas (WHO grades II - IV) were systematically evaluated with TTF-1 immunohistochemistry using three clones: SPT24, 8G7G3/1, and SP141, and results correlated with clinicopathologic features. 14 high-grade diffuse gliomas demonstrated nuclear staining with the SP141 and SPT24 clones. Two tumors showed weak positivity with the 8G7G3/1 clone. All tumors were high grade by histology (WHO grades III and IV). 86% (12/14) of TTF-1-positive gliomas involved the frontal lobes at diagnosis. No relationship with IDH R132H, ATRX, p53, H3K27M, or EGFR immunohistochemistry was identified. TTF-1 expression in gliomas was not independently prognostic of overall survival. TTF-1 expression in diffuse gliomas is a rare but potentially misleading occurrence. In our cohort, staining occurred with both the SPT24 and SP141 clones at equal intensity and frequency. Clustering of TTF-1-positive tumors in the frontal lobe(s) suggests lineage-specific expression. Due to clone-specific expression in diffuse gliomas, caution must be exercised in the work-up of intracranial tumors with TTF-1.
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Effects of active acromegaly on bone mRNA and microRNA expression patterns.

PubMed

Belaya, Zhanna; Grebennikova, Tatiana; Melnichenko, Galina; Nikitin, Alexey; Solodovnikov, Alexander; Brovkina, Olga; Grigoriev, Andrey; Rozhinskaya, Liudmila; Lutsenko, Alexander; Dedov, Ivan

2018-04-01

To evaluate the response of bone to chronic long-term growth hormone (GH) and insulin-like growth factor-1 (IGF1) excess by measuring the expression of selected mRNA and microRNA (miR) in bone tissue samples of patients with active acromegaly. Case-control study. Bone tissue samples were obtained during transsphenoidal adenomectomy from the sphenoid bone (sella turcica) from 14 patients with clinically and biochemically confirmed acromegaly and 10 patients with clinically non-functioning pituitary adenoma (NFPA) matched by sex and age. Expression of genes involved in the regulation of bone remodeling was studied using quantitative polymerase chain reaction (qPCR). Of the genes involved in osteoblast and osteoclast activity, only alkaline phosphatase (ALP) mRNA was 50% downregulated in patients with acromegaly. GH excess caused increased expression of the Wnt signaling antagonists ( DKK1) and agonists ( WNT10B) and changes in the levels of miR involved in mesenchymal stem cell commitment to chondrocytes (miR-199a-5p) or adipocytes (miR-27-5p, miR-125b-5p, miR-34a-5p, miR-188-3p) P  < 0.05; q  < 0.1. Relevant compensatory mechanisms were found through the changes in miR involved in osteoblastogenesis (miR-210-5p, miR-135a-5p, miR-211, miR-23a-3p, miR-204-5p), but the expression of TWIST1 was 50% downregulated and RUNX2 was unchanged. Acromegaly had minimal effects on tested mRNAs specific to osteoblast or osteoclast function except for downregulated ALP expression. The expressions of miR known to be involved in mesenchymal stem cell commitment and downregulated TWIST1 expression suggest acromegaly has a negative effect on osteoblastogenesis. © 2018 European Society of Endocrinology.

A Case of Acromegaly in which a Pituitary Gland Tumor was Reduced Significantly by Administering Octreotide Long Acting Release (LAR) and Could Be Removed Surgically.

PubMed

Arao, Tadashi; Okada, Yosuke; Uemura, Fumi; Nishizawa, Shigeru; Tanaka, Yoshiya

A 54-year-old woman was admitted to our hospital for detailed examination of acromegaly because she noticed bilateral hand and finger swelling at the age of 43 and plantar thickening, facial changes and unclear articulation at the age of 49. She had prominent brow ridges, mandibular protrusion, and enlargement of the hands, feet, nasal wings, lips and tongue. Her growth hormone (GH) level was 39.8 ng/ml, insulin-like growth factor-1 (IGF-1) level was 717 ng/ml, GH level was not suppressed (22.9 ng/ml) during a 75-g oral glucose tolerance test (OGTT). Radiography showed cauliflower-like enlargement of the distal phalanx of the fingers, thickening/enlargement of the plantar soft tissues, and increased antero-posterior diameter of the sella turcica. Magnetic resonance imaging showed a mass (21×17 mm) growing towards the right suprasellar region and invading the cavernous sinus. She was diagnosed with acromegaly based on the characteristic physical findings, GH excess, high IGF-1, lack of GH suppression during the 75-g OGTT, and the presence of a pituitary tumor. She was started on octreotide long acting release (Oct-LAR) 20 mg/4w for tumor shrinkage. After three doses, her GH and IGF-1 levels decreased to 2.19 ng/ml (1.69 during the 75-g OGTT) and 205 ng/ml, respectively, meeting cure criteria for acromegaly. In this case, a decrease in GH and IGF-1 levels, tumor shrinkage, and resolution of cavernous sinus invasion allowed the patient to undergo surgery with curative intent (the first-line treatment for acromegaly) without postoperative complications. Thus, preoperative Oct-LAR administration has the potential to improve treatment outcomes of acromegaly.

Radiation Dose Reduction by Indication-Directed Focused z-Direction Coverage for Neck CT.

PubMed

Parikh, A K; Shah, C C

2016-06-01

The American College of Radiology-American Society of Neuroradiology-Society for Pediatric Radiology Practice Parameter for a neck CT suggests that coverage should be from the sella to the aortic arch. It also recommends using CT scans judiciously to achieve the clinical objective. Our purpose was to analyze the potential dose reduction by decreasing the scan length of a neck CT and to assess for any clinically relevant information that might be missed from this modified approach. This retrospective study included 126 children who underwent a neck CT between August 1, 2013, and September 30, 2014. Alteration of the scan length for the modified CT was suggested on the topographic image on the basis of the indication of the study, with the reader blinded to the images and the report. The CT dose index volume of the original scan was multiplied by the new scan length to calculate the dose-length product of the modified study. The effective dose was calculated for the original and modified studies by using age-based conversion factors from the American Association of Physicists in Medicine Report No. 96. Decreasing the scan length resulted in an average estimated dose reduction of 47%. The average reduction in scan length was 10.4 cm, decreasing the overall coverage by 48%. The change in scan length did not result in any missed findings that altered management. Of the 27 abscesses in this study, none extended to the mediastinum. All of the lesions in question were completely covered. Decreasing the scan length of a neck CT according to the indication provides a significant savings in radiation dose, while not altering diagnostic ability or management. © 2016 by American Journal of Neuroradiology.

Usefulness of magnetic resonance findings of the hypothalamic-pituitary region in the management of short children with growth hormone deficiency: evidence from a longitudinal study.

PubMed

Kalina, Maria A; Kalina-Faska, Barbara; Gruszczyńska, Katarzyna; Baron, Jan; Małecka-Tendera, Ewa

2012-01-01

The purpose of this study is to assess the relationship between magnetic resonance images (MRI) of the hypothalamic-pituitary (H-P) region and response to recombinant human growth hormone (rhGH) treatment in short children with growth hormone deficiency, basing on changes of auxologic parameters, as well as to answer the question if MRI may serve for selecting and monitoring the rhGH responders. The study group comprised 85 children treated with rhGH, aged 7.3-18.7 years, followed for the mean period of 3.2 years (range, 2.1-9.5 years). Auxologic parameters (height deficit hSDS, deviation from the mid-parental height hSDS-mpSDS, bone delay index bone age/chronological age ratio (BA/CA)) were assessed before, during and at the end of rhGH treatment; growth velocity was calculated before and during rhGH therapy. Parameters were correlated with the MRI of the H-P region. Structural anomalies of the H-P region were found in 22 (25.9%) children: empty sella syndrome (ESS) in 12 (14.1%) patients, ectopic posterior pituitary (EPP) in ten (11.8%). Patients' height deficit and their deviation from parental height before rhGH therapy was significantly greater in the EPP group (median hSDS = -3.8; hSDS-mpSDS = -2.5), bone age delay was the greatest in the ESS group (median BA/CA = 0.69), after therapy - in the EPP group (median BA/CA = 0.82). Growth velocity improved in the first year of the rhGH therapy in all groups; however, the most significant acceleration was observed in the EPP group (median delta hSDS = 0.9), then stabilised and was comparable in all groups. MRI may be helpful in predicting response to the rhGH treatment, providing midline abnormalities are taken into account.

Progression from isolated growth hormone deficiency to combined pituitary hormone deficiency.

PubMed

Cerbone, Manuela; Dattani, Mehul T

2017-12-01

Growth hormone deficiency (GHD) can present at any time of life from the neonatal period to adulthood, as a result of congenital or acquired insults. It can present as an isolated problem (IGHD) or in combination with other pituitary hormone deficiencies (CPHD). Pituitary deficits can evolve at any time from GHD diagnosis. The number, severity and timing of occurrence of additional endocrinopathies are highly variable. The risk of progression from IGHD to CPHD in children varies depending on the etiology (idiopathic vs organic). The highest risk is displayed by children with abnormalities in the Hypothalamo-Pituitary (H-P) region. Heterogeneous data have been reported on the type and timing of onset of additional pituitary hormone deficits, with TSH deficiency being most frequent and Diabetes Insipidus the least frequent additional deficit in the majority, but not all, of the studies. ACTH deficiency may gradually evolve at any time during follow-up in children or adults with childhood onset IGHD, particularly (but not only) in presence of H-P abnormalities and/or TSH deficiency. Hence there is a need in these patients for lifelong monitoring for ACTH deficiency. GH treatment unmasks central hypothyroidism mainly in patients with organic GHD, but all patients starting GH should have their thyroid function monitored closely. Main risk factors for development of CPHD include organic etiology, H-P abnormalities (in particular pituitary stalk abnormalities, empty sella and ectopic posterior pituitary), midline brain (corpus callosum) and optic nerves abnormalities, genetic defects and longer duration of follow-up. The current available evidence supports longstanding recommendations for the need, in all patients diagnosed with IGHD, of a careful and indefinite follow-up for additional pituitary hormone deficiencies. Copyright © 2017 Elsevier Ltd. All rights reserved.

The long-term effects of megavoltage radiotherapy as sole or combined therapy for large prolactinomas: studies with high definition computerized tomography.

PubMed

Johnston, D G; Hall, K; Kendall-Taylor, P; Ross, W M; Crombie, A L; Cook, D B; Watson, M J

1986-06-01

The long-term sequelae of external pituitary irradiation alone or in combination with surgery and/or bromocriptine therapy have been studied in 14 patients with large prolactinomas over an observation period of 6-22 years (mean 13 years). Galactorrhoea was abolished in four of the five females with this symptom, but menstrual disturbance persisted in five of six patients. Sexual function was normal without sex hormone replacement in only one of the eight males after treatment. Neurological deficits were abolished or improved by treatment in all 9 patients with this presentation. Serum prolactin levels declined after treatment (P less than 0.001) and fell to within the normal range off bromocriptine therapy in six of the 14 patients at a mean of 9 years (range 5-17 years) after radiotherapy. All patients had anterior pituitary deficiency of some degree at reassessment, and 13 required replacement treatment. Serial skull radiographs revealed remineralization of the fossa floor in five patients and a decrease in fossa size in three. All five patients who did not also have surgery had evidence of tumour shrinkage without bromocriptine treatment (on CT scan or metrizamide cisternography). Fourth generation CT scans on completion of the study revealed a decrease in tumour mass in all patients, with varying degrees of empty sella in 13 and a cystic intrasellar tumour in the remaining one. Residual tumour was demonstrated in 10 patients, three of whom had normal serum prolactin levels, while one patient without visible tumour had persistent hyperprolactinaemia. Radiotherapy, alone or in combination with surgery and bromocriptine, effectively decreases prolactin secretion and tumour size in patients with large prolactinomas at the expense of other anterior pituitary function. Circulating prolactin levels are a poor marker of residual tumour volume.

Dysfunction of hypothalamic-hypophysial axis after traumatic brain injury in adults.

PubMed

Krahulik, David; Zapletalova, Jirina; Frysak, Zdenek; Vaverka, Miroslav

2010-09-01

Traumatic brain injury (TBI) is a major cause of serious morbidity and mortality. The incidence is 100-500/100,000 inhabitants/year. Chronic pituitary dysfunction is increasingly recognized after TBI. To define the incidence of endocrine dysfunction and risk factors, the authors describe a prospectively assessed group of patients in whom they documented hormonal functions, early diagnosis, and treatment of neuroendocrine dysfunction after TBI. Patients aged 18-65 years were prospectively observed from the time of injury to 1 year postinjury; the Glasgow Coma Scale score ranged from 3 to 14. Patients underwent evaluation of hormonal function at the time of injury and at 3, 6, and 12 months postinjury. Magnetic resonance imaging was also conducted at 1 year postinjury. During the study period, 89 patients were observed. The mean age of the patients was 36 years, there were 23 women, and the median Glasgow Coma Scale score was 7. Nineteen patients (21%) had primary hormonal dysfunction. Major deficits included growth hormone dysfunction, hypogonadism, and diabetes insipidus. Patients in whom the deficiency was major had a worse Glasgow Outcome Scale score, and MR imaging demonstrated empty sella syndrome more often than in patients without a deficit. To the authors' knowledge, this is the third largest study of its kind worldwide. The incidence of chronic hypopituitarism after TBI was higher than the authors expected. After TBI, patients are usually observed on the neurological and rehabilitative wards, and endocrine dysfunction can be overlooked. This dysfunction can be life threatening and other clinical symptoms can worsen the neurological deficit, extend the duration of physiotherapy, and lead to mental illness. The authors recommend routine pituitary hormone testing after moderate or severe TBI within 6 months and 1 year of injury.

Male hypogonadism at a tertiary care hospital in Karachi, Pakistan.

PubMed

Ram, Nanik; Asghar, Ali; Hashmi, Fauzan; Islam, Najmul

2012-01-01

Male hypogonadism is defined as 'inadequate gonadal function, manifested by deficiency in gametogenesis and/or secretion of gonadal hormones'. Signs and symptoms of hypogonadism depend primarily on the age of onset. It can be classified according to the site primarily involved: the gonads, the hypothalamus, or the pituitary gland. The objective this study was to determine the presentation and aetiology of male hypogonadism seen in a tertiary care hospital. This cross-sectional study was conducted at Endocrine Clinics, Aga Khan University Hospital Karachi. Data of male patients with hypogonadism who attended clinics during January 2009 to August 2011 were reviewed. All male patients with clinical and biochemical evidence of hypogonadism were included in the study. Patients with Diabetes Mellitus, Metabolic Syndrome, Andropause, AIDS, Chronic Renal Failure, and Cirrhosis were excluded. Mean +/- SD were computed for quantitative variables. Frequency and percentages were computed for qualitative variables. Aetiology of male hypogonadism was categorised as primary and secondary hypogonadism. A total of 85 patients with male hypogonadism attended the endocrine clinic. Mean age of patients was 25 +/- 10 years. Clinical presentations were small genitalia (65%), absent secondary sexual characteristics (53%), not attained puberty (47%), infertility (53%), erectile dysfunction (41%) and loss of libido (29%). Seventy-three (86%) patients had hypogonadotrophic hypogonadism (secondary hypogonadism) and 12 (14%) patients had hypergonadotrophic hypogonadism (primary hypogonadism). Among the patients with hypogonadotrophic hypogonadism 38 had idiopathic hypogonadotrophic hypogonadsim, 7 had pituitary adenoma, 6 had empty sella syndrome, 3 had Kallman's syndrome, and 1 patient had haemosiderosis due to thalassaemia major; 18 patients did not undergo brain imaging. Small genitalia, absent secondary sexual characteristics and infertility were the main presenting features of hypogonad

Extended endoscopic endonasal surgery for clival chordoma and chondrosarcoma: Our experience in 14 cases.

PubMed

Culebras, Diego; Torales, Jorge; Reyes, Luis Alberto; Zapata, Luis; García, Sergio; Roldán, Pedro; Langdon, Cristóbal; Alobid, Issam; Enseñat, Joaquim

2018-04-21

To report our experience in the management of chordoma and chondrosarcoma with extended endoscopic endonasal surgery. We performed a retrospective analysis of a series of 14 patients with clival chordoma or chondrosarcoma who had extended endoscopic endonasal surgery from 2008 to 2016 performed by the same multidisciplinary team. We had fourteen patients (male/female 2:1), with a mean age of 49years for chordoma and 32 for chondrosarcoma. The most common clinical presentation was diplopia in 78.5% of cases, followed by dysphagia in 28.6%. Histologically, 71.4% were chordomas and 28.6% were chondrosarcomas. In addition, invasion of at least two thirds or more of the clivus was found in 81% of the cases; in 57.1% there was intradural invasion, and in 35.7% invasion of the sella turcica. In 42.8% of cases, the degree of resection was total and in 21.5% subtotal. The most common complication was CSF fistula, occurring in 28.6% of the cases, with only one case requiring surgery to repair it. Adjuvant treatment with Proton Beam was performed in 35.7% of cases and with conventional radiotherapy in 21.5%. Mean follow-up was 53.5months and tumour recurrence or progression was found in 21.5% of the cases, two of which had not received adjuvant treatment. There were no deaths. The extended endoscopic endonasal approach (EEEA) performed by an experienced team is a good alternative for the management of these lesions. Intradural invasion may be related to an increased risk of complications and worse clinical presentation, in addition to a lower rate of total resection. Copyright © 2018 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

[Suprasellar arachnoid cyst--report of a case (author's transl)].

PubMed

Takahashi, T; Kawai, S; Kaminoh, T; Hiramatsu, K; Maekawa, M; Yuasa, T; Miyamoto, N; Hattori, Y

1982-04-01

A 4-year-old boy with suprasellar arachnoid cyst was reported. At the age of 30-month-old his aunt was aware of his squint. During the observation by ophthalmologists from the age of 1y. to 3y., enlargement of the head and impairment of the visual acuity were manifested. Cranial CT scan revealed the enlargement of the ventricular system and a round low density area located superior to the sella. Absorption coefficient of the lesion was similar to that of the cerebrospinal fluid. No abnormal contrast enhancement was seen. Examination revealed the head circumference of 53.3 cm larger than doubled standard deviation, the right external strabismus, impaired vision (R:0.03, L:0.3) and optic atrophy but no other neurological signs. Cerebral angiography showed suprasellar mass lesion. After the ventriculography with water-soluble contrast medium, V-P shunt operation was performed and then the patient was transferred to the CT room. CSF enhanced CT scan showed no communication between the ventricles and the cyst. By frontotemporal approach, microsurgical removal of the cystwall was performed and the histological diagnosis was arachnoid membrane. Several days after the operation, bilateral subdural effusion was seen on CT scan and was treated with bilateral S-P shunt and the removal of V-P shunt. Follow up CT scan disclosed the disappearance of the subdural effusion and the suprasellar cyst. The visual acuity was improved well and the endocrinological study was normal. Analysis of the 45 reported cases of suprasellar arachnoid cyst suggested that direct removal of the cyst wall is better than the V-P shunt operation and the cyst shunting is advisable for repeat recurrence of the cyst. Removal of the ventricular shunting system may be effective for the prevention of the subdural effusion as a complication after direct operation.

[Preliminary results of noncontact temperature measuring using an infrared thermometer on the tarsus pf swine with osteoarthrosis tarsi deformans].

PubMed

Sabec, D; Lazar, P

1990-01-01

The surface temperature on tarsus of 54 performance tested boars of about 60 kg of Swedish Landrace was measured by means of infra-red thermometer at three measuring points (MP1, MP2 and MP3). MP1 was situated above the tuberculum of os metatarsale III or, as visualised in 21 slaughtered boars, above pathoanatomical alterations of osteoarthrosis tarsi deformans (OATD). MP2 was located directly medially and MP3 laterally from MP1. A clear palpatory symptom was found only with 10 boars, while all 21 slaughtered boars showed different morphological lesions of OATD. The average temperature at MP1 was 35.69 degrees C and was significantly higher (P = .01) than at MP2 (35.34) and MP3 (35.30). The temperature differences between MP1 and MP2 or MP3 were statistically reliable. Boars, who had a clear palpatory symptoms at tuberculum, showed an average temperature at MT1 of 36.27 degrees C, which was higher (P = .0114) than of those with an unclear palpatory symptom (35.64 degrees C). The results show that the temperature differences are the consequence of OATD.

The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature.

PubMed

Bando, Hironori; Iguchi, Genzo; Fukuoka, Hidenori; Taniguchi, Masaaki; Yamamoto, Masaaki; Matsumoto, Ryusaku; Suda, Kentaro; Nishizawa, Hitoshi; Takahashi, Michiko; Kohmura, Eiji; Takahashi, Yutaka

2014-02-01

The prevalence and clinical characteristics of IgG4-related hypophysitis remain unclear due to the limited number of case reports. Therefore, in this study, we screened consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. A total of 170 consecutive outpatients with hypopituitarism and/or central DI were screened at Kobe University Hospital for detecting IgG4-related hypophysitis by pituitary magnetic resonance imaging, measuring serum IgG4 concentrations, assessing the involvement of other organs, and carrying out an immunohistochemical analysis to detect IgG4-positive cell infiltration. Among the screened cases, 116 cases were excluded due to diagnosis of other causes such as tumors and congenital abnormalities. Additionally, 22 cases with isolated ACTH deficiency were analyzed and were found not to meet the criteria of IgG4-related hypophysitis. The remaining 32 cases were screened and seven were diagnosed with IgG4-related hypophysitis, of which three cases were diagnosed by analyzing pituitary specimens. IgG4-related hypophysitis was detected in 30% (seven of 23 patients) of hypophysitis cases and 4% of all hypopituitarism/DI cases. The mean age at the onset of IgG4-related hypophysitis was 61.8±8.8 years, and the serum IgG4 concentration was 191.1±78.3 mg/dl (normal values 5-105 mg/dl and values in IgG4-related disease (RD) ≥135 mg/dl). Pituitary gland and/or stalk swelling was observed in six patients, and an empty sella was observed in one patient. Multiple co-existing organ involvement was observed in four of the seven patients prior to the onset of IgG4-related hypophysitis. These data suggest that the prevalence of IgG4-related hypophysitis has been underestimated. We should also consider the possibility of the development of hypopituitarism/DI caused by IgG4-related hypophysitis during the clinical course of other IgG4-RDs.

A phantom study of the immobilization and the indications for using virtual isocenter in stereoscopic X‐ray image guidance system referring to position localizer in frameless radiosurgery

PubMed Central

Chang, Hsiao‐Han; Lee, Hsiao‐Fei; Sung, Chien‐Cheng; Liao, Tsung‐I

2013-01-01

A frameless radiosurgery system is using a set of thermoplastic mask for fixation and stereoscopic X‐ray imaging for alignment. The accuracy depends on mask fixation and imaging. Under certain circumstances, the guidance images may contain insufficient bony structures, resulting in lesser accuracy. A virtual isocenter function is designed for such scenarios. In this study, we investigated the immobilization and the indications for using virtual isocenter. Twenty‐four arbitrary imaginary treatment targets (ITTs) in phantom were evaluated. The external Localizer with positioner films was used as reference. The alignments by using actual and virtual isocenter in image guidance were compared. The deviation of the alignment after mask removing and then resetting was also checked. The results illustrated that the mean deviation between the alignment by image guidance using actual isocenter (Isoimg) and the localizer(Isoloc) was 2.26mm±1.16mm (standard deviation, SD), 1.66mm±0.83mm for using virtual isocenter. The deviation of the alignment by the image guidance using actual isocenter to the localizer before and after mask resetting was 7.02mm±5.8mm. The deviations before and after mask resetting were insignificant for the target center from skull edge larger than 80 mm on craniocaudal direction. The deviations between the alignment using actual and virtual isocenter in image guidance were not significant if the minimum distance from target center to skull edge was larger or equal to 30 mm. Due to an unacceptable deviation after mask resetting, the image guidance is necessary to improve the accuracy of frameless immobilization. A treatment isocenter less than 30 mm from the skull bone should be an indication for using virtual isocenter to align in image guidance. The virtual isocenter should be set as caudally as possible, and the sella of skull should be the ideal point. PACS numbers: 87.55.kh, 87.55.ne, 87.55.tm PMID:23835379

Wolfram (DIDMOAD) syndrome: a multidisciplinary clinical study in nine Turkish patients and review of the literature.

PubMed

Simsek, E; Simsek, T; Tekgül, S; Hosal, S; Seyrantepe, V; Aktan, G

2003-01-01

To study Wolfram syndrome (WFS) with multidisciplinary consultations and compare the results with the literature. Nine patients fulfilled the ascertainment criteria of WFS (insulin-dependent diabetes mellitus and optic atrophy). All patients were evaluated by the departments of paediatrics, ophthalmology, audiology, urology and medical biology. The earliest manifestation of WFS was insulin-dependent diabetes mellitus (at a median age of 6.9 y), followed by optic atrophy (8.9 y), diabetes insipidus (10.2 y) and deafness (10.5 y). Short stature was found in five cases, delayed puberty in two cases and hypergonadotropic hypogonadism in one case. Audiography disclosed hearing loss at high frequency in all patients (100%), but only five patients had clinical subjective hearing problems. Intravenous pyelography revealed hydroureteronephrosis in eight patients. Urodynamics revealed a normal bladder in only one patient. Three patients had a low-capacity, low-compliance bladder, detrusor external sphincteric dyssynergia and emptying problem, while five had an atonic bladder. Ocular findings were optic atrophy, low visual acuity and colour vision defects. Visual field tests revealed concentric and/or peripheral diminution in five patients. Visual evoked potentials were abnormal (reduced amplitude to both flash and pattern stimulation) in seven patients. Cranial magnetic resonance imaging showed mild or moderate atrophy of the optic nerves, chiasm, cerebellum, basal ganglia and brainstem in six patients; there was a partially empty sella in one case. There was no evidence of mitochondrial tRNA(Leu) (UUR) A to G (nucleotide 3243) mutation. Wolfram syndrome should be evaluated in a multidisciplinary manner. Some specific and dynamic tests are necessary to make a more precise estimate of the prevalence and median age of the components of WFS. Short stature is a common feature in WFS. Hypogonadism may be hypogonadotropic or hypergonadotropic. Bladder dysfunction does not always

Surgical treatment of pituitary adenomas using low-field intraoperative magnetic resonance imaging.

PubMed

Tabakow, Paweł; Czyz, Margin; Jarmundowicz, Włodzimierz; Lechowicz-Głogowska, Ewa

2012-01-01

Intraoperative magnetic resonance imaging (iMRI) is a new technique for imaging of the brain and is used with increasing frequency during neurosurgical operations, enabling the surgeon to make decisions based on real-time images. This paper presents the technique for the surgical treatment of pituitary adenomas using low-field iMRI, evaluates the safety of iMRI usage in pituitary surgery and examines the influence of iMRI on the extent of tumor removal. From October 2008 to December 2010, 18 patients were treated for pituitary adenomas using the low-field iMRI system Polestar N20. The procedures were conducted via the transsphenoidal approach, using the microscopic technique in 15 cases and endoscopically in three cases. The patients' mean age was 56 +/- 15 years; their mean American Society of Anesthesiologists (ASA) score was 2; 67% of them were male. Most of the patients were operated on for macroadenomas, 83% of which were hormonally inactive. The analysis concerned the technical aspects of iMRI usage, such as preparation and surgery time and the quality of the iMRI-scans performed. The safety of iMRI and its influence on decisions regarding further tumor resection. The operations on pituitary adenomas using iMRI were safe. Only two hemorrhagic complications were noted, and they were not related to iMRI usage. The mean preparation and surgery times were 109 +/- 37 minutes and 238 +/- 188 minutes, respectively. The iMRI images of sella turcica were of satisfactory quality in 16 patients. In 50% of the cases, iMRI conducted when the surgeon believed that the desired extent of tumor resection had been attained showed that there were still tumor remnants to be resected. In 67% of these cases, continued tumor removal lead to achievement of the desired degree of resection. Low-field iMRI-guided operations on pituitary tumors are safe and feasible, and they ensure an increased radicality of tumor resection.

Genetically induced abnormal cranial development in human trisomy 18 with holoprosencephaly: comparisons with the normal tempo of osteogenic-neural development.

PubMed

Reid, Shaina N; Ziermann, Janine M; Gondré-Lewis, Marjorie C

2015-07-01

Craniofacial malformations are common congenital defects caused by failed midline inductive signals. These midline defects are associated with exposure of the fetus to exogenous teratogens and with inborn genetic errors such as those found in Down, Patau, Edwards' and Smith-Lemli-Opitz syndromes. Yet, there are no studies that analyze contributions of synchronous neurocranial and neural development in these disorders. Here we present the first in-depth analysis of malformations of the basicranium of a holoprosencephalic (HPE) trisomy 18 (T18; Edwards' syndrome) fetus with synophthalmic cyclopia and alobar HPE. With a combination of traditional gross dissection and state-of-the-art computed tomography, we demonstrate the deleterious effects of T18 caused by a translocation at 18p11.31. Bony features included a single developmentally unseparated frontal bone, and complete dual absence of the anterior cranial fossa and ethmoid bone. From a superior view with the calvarium plates removed, there was direct visual access to the orbital foramen and hard palate. Both the eyes and the pituitary gland, normally protected by bony structures, were exposed in the cranial cavity and in direct contact with the brain. The middle cranial fossa was shifted anteriorly, and foramina were either missing or displaced to an abnormal location due to the absence or misplacement of its respective cranial nerve (CN). When CN development was conserved in its induction and placement, the respective foramen developed in its normal location albeit with abnormal gross anatomical features, as seen in the facial nerve (CNVII) and the internal acoustic meatus. More anteriorly localized CNs and their foramina were absent or heavily disrupted compared with posterior ones. The severe malformations exhibited in the cranial fossae, orbital region, pituitary gland and sella turcica highlight the crucial involvement of transcription factors such as TGIF, which is located on chromosome 18 and contributes

Development of a monitoring platform for slope instability and sliding prevention : preliminary results

NASA Astrophysics Data System (ADS)

Drakatos, G.; Paradissis, D.; Anastasiou, D.; Elias, P.; Marinou, A.; Chousianitis, K.; Papanikolaou, X.; Zacharis, V.; Argyrakis, P.; Papazisi, K.; Makropoulos, K.

2012-04-01

Land sliding, as a consequence of slope instability, constitutes a natural catastrophe resulting mainly from geological cause often followed by disastrous impact on both the natural and man-made environment. The reasons causing land slides can vary from purely geological factors, to other relevant or not natural catastrophes, urban or residential expansion, tourist growth in areas under inappropriate geological background, or even a combination of the aforementioned causes The respective consequences, also span a wide range of negative impacts, both for the man-made (e.g. destruction of transportation infrastructure, constructions and urban or sub-urban areas) and the natural environment. Unfortunately, prevention of land slides is still largely ineffective adding to an inefficient and inadequate addressing of the problem, mainly due to the lack of systematic monitoring of such regions and due to the fact that "treatment" overwhelms "prevention". Recent developments in Global Navigation Satellite Systems (GNSS), Satellite Geodesy and satellite differential interferometry (DinSAR), have established these fields as fully equipped, from a scientific and engineering perspective, to act --either as autonomous techniques or in conjunction-- as prevention and/or early warning systems. Such state-of-the-art technology was implemented, in a project undertaken by the Institute of Geodynamics Dionysos Satellite Observatory/Higher Geodesy Laboratory and the Institute for Space Applications and Remote Sensing, in order to evaluate the potential of monitoring slide stability and the assessment of hazard evaluation. Therefore, for the first time in Greece, an attempt was made to develop a monitoring platform for slope instability and sliding prevention at two of the most hazardous, regarding soil instabilities, regions of Peloponnese, namely Sellas and Chalkio (in Messinia and Korinthia respectively). GPS campaigns were carried through, cGPS stations were installed and a network

Class III camouflage treatment: what are the limits?

PubMed

Burns, Nikia R; Musich, David R; Martin, Chris; Razmus, Thomas; Gunel, Erdogan; Ngan, Peter

2010-01-01

The purpose of this study was to determine the skeletal, dental, and soft-tissue changes in response to camouflage Class III treatment. Thirty patients (average age, 12.4 + or - 1.0 years) with skeletal Class III malocclusions who completed comprehensive nonextraction orthodontic treatment were studied. Skeletal, dental, and soft-tissue changes were determined by using published cephalometric analyses. The quality of orthodontic treatment was standardized by registering the peer assessment rating index on the pretreatment and posttreatment study models. The change in the level of gingival attachment with treatment was determined on the study casts. The results were compared with a group of untreated subjects. Data were analyzed with repeated measures analysis and paired t tests. The average change in the Wits appraisal was greater in the treated group (1.2 + or - 0.1 mm) than in the control group (-0.5 + or - 0.3 mm). The average peer assessment rating index score improved from 33.5 to 4.1. No significant differences were found for the level of gingival attachments between the treatment and control groups. The sagittal jaw relationship (ANB angle) did not improve with camouflage treatment. A wide range of tooth movements compensated for the skeletal changes in both groups. The upper and lower limits for incisal movement to compensate for Class III skeletal changes were 120 degrees to the sella-nasion line and 80 degrees to the mandibular plane, respectively. Greater increases in the angle of convexity were found in the treated group, indicating improved facial profiles. Greater increases in length of the upper lip were found in the treated group, corresponding to the changes in the hard tissues with treatment. Significant dental and soft-tissue changes can be expected in young Class III patients treated with camouflage orthodontic tooth movement. A wide range of skeletal dysplasias can be camouflaged with tooth movement without deleterious effects to the

A study on the reproducibility of cephalometric landmarks when undertaking a three-dimensional (3D) cephalometric analysis

PubMed Central

Llamas, José M.; Cibrián, Rosa; Gandia, José L.; Paredes, Vanessa

2012-01-01

Objectives: Cone Beam Computerized Tomography (CBCT) allows the possibility of modifying some of the diagnostic tools used in orthodontics, such as cephalometry. The first step must be to study the characteristics of these devices in terms of accuracy and reliability of the most commonly used landmarks. The aims were 1- To assess intra and inter-observer reliability in the location of anatomical landmarks belonging to hard tissues of the skull in images taken with a CBCT device, 2- To determine which of those landmarks are more vs. less reliable and 3- To introduce planes of reference so as to create cephalometric analyses appropriated to the 3D reality. Study design: Fifteen patients who had a CBCT (i-CAT®) as a diagnostic register were selected. To assess the reproducibility on landmark location and the differences in the measurements of two observers at different times, 41 landmarks were defined on the three spatial axes (X,Y,Z) and located. 3.690 measurements were taken and, as each determination has 3 coordinates, 11.070 data were processed with SPSS® statistical package. To discover the reproducibility of the method on landmark location, an ANOVA was undertaken using two variation factors: time (t1, t2 and t3) and observer (Ob1 and Ob2) for each axis (X, Y and Z) and landmark. The order of the CBCT scans submitted to the observers (Ob1, Ob2) at t1, t2, and t3, were different and randomly allocated. Multiple comparisons were undertaken using the Bonferroni test. The intra- and inter-examiner ICC´s were calculated. Results: Intra- and inter-examiner reliability was high, both being ICC ≥ 0.99, with the best frequency on axis Z. Conclusions: The most reliable landmarks were: Nasion, Sella, Basion, left Porion, point A, anterior nasal spine, Pogonion, Gnathion, Menton, frontozygomatic sutures, first lower molars and upper and lower incisors. Those with less reliability were the supraorbitals, right zygion and posterior nasal spine. Key words:Cone Beam

Domain Modeling and Application Development of an Archetype- and XML-based EHRS. Practical Experiences and Lessons Learnt.

PubMed

Kropf, Stefan; Chalopin, Claire; Lindner, Dirk; Denecke, Kerstin

2017-06-28

Access to patient data within the hospital or between hospitals is still problematic since a variety of information systems is in use applying different vendor specific terminologies and underlying knowledge models. Beyond, the development of electronic health record systems (EHRSs) is time and resource consuming. Thus, there is a substantial need for a development strategy of standardized EHRSs. We are applying a reuse-oriented process model and demonstrate its feasibility and realization on a practical medical use case, which is an EHRS holding all relevant data arising in the context of treatment of tumors of the sella region. In this paper, we describe the development process and our practical experiences. Requirements towards the development of the EHRS were collected by interviews with a neurosurgeon and patient data analysis. For modelling of patient data, we selected openEHR as standard and exploited the software tools provided by the openEHR foundation. The patient information model forms the core of the development process, which comprises the EHR generation and the implementation of an EHRS architecture. Moreover, a reuse-oriented process model from the business domain was adapted to the development of the EHRS. The reuse-oriented process model is a model for a suitable abstraction of both, modeling and development of an EHR centralized EHRS. The information modeling process resulted in 18 archetypes that were aggregated in a template and built the boilerplate of the model driven development. The EHRs and the EHRS were developed by openEHR and W3C standards, tightly supported by well-established XML techniques. The GUI of the final EHRS integrates and visualizes information from various examinations, medical reports, findings and laboratory test results. We conclude that the development of a standardized overarching EHR and an EHRS is feasible using openEHR and W3C standards, enabling a high degree of semantic interoperability. The standardized

Management of cerebrospinal fluid leak during endoscopic pituitary surgery.

PubMed

Berker, Mustafa; Aghayev, Kamran; Yücel, Taşkın; Hazer, Derya Burcu; Onerci, Metin

2013-08-01

Dural opening and closures are major steps in endoscopic pituitary surgery. Restoring the normal anatomy at the end of the procedure creates a natural barrier between the intrasellar compartment and the sinonasal cavity. In this study, we present a relatively simple dural opening and closure technique for endoscopic pituitary surgery. This technique provides a better alternative to the use of a more complex nasoseptal flap or the multilevel closure with artificial materials as it restores the normal anatomy after the tumor removal and provides a better physiological barrier between the sinonasal cavity and the intrasellar compartment. Incision is performed in circular or horseshoe fashion leaving a small peduncle, and then the dura is reflected. Of the 733 endoscopic transsphenoidal procedures in 667 patients conducted between January 2006 and May 2012, we used this described technique in 50 cases (7.4%). In these 50 cases with dural flap, there was no postoperative CSF leakage. Intraoperative CSF leakage was observed in 135 (20.2%) of the 667 patients. In 15 (11.1%) of these 135 patients we used the dural flap technique accompanied with fat and/or fascia lata support. There was no postoperative leakage in these patients. In the remaining 120 (89.9%) patients who had intraoperative CSF leakage, we used fat and/or fascia lata for the reconstruction of the sella floor. But we observed postoperative CSF leakage in 12 (10%) of the 120 patients without the dural flap which were reoperated. The dural flap technique we employ has several advantages. First of all, it allows optimal physiological reconstruction after the surgery. Secondly, the bridge between the flap and the main dura helps maintain the vascular supply, which in turn can radically shorten the healing time. Thirdly, this technique is obviously a better alternative to the time consuming and expensive multilevel closures with tissue sealants and artificial grafts. Copyright © 2012 Elsevier Ireland Ltd. All

A Comparison of Dexmedetomidine and Clonidine in Attenuating the Hemodynamic Responses at Various Surgical Stages in Patients Undergoing Elective Transnasal Transsphenoidal Resection of Pituitary Tumors

PubMed Central

Jan, Summaira; Ali, Zulfiqar; Nisar, Yasir; Naqash, Imtiaz Ahmad; Zahoor, Syed Amer; Langoo, Shabir Ahmad; Azhar, Khan

2017-01-01

Background: Transsphenoidal approach to pituitary tumors is a commonly performed procedure with the advantage of a rapid midline access to the sella with minimal complications. It may be associated with wide fluctuations in hemodynamic parameters due to intense noxious stimulus at various stages of the surgery. As duration of the surgery is short and the patients have nasal packs, it is prudent to use an anesthestic technique with an early predictable recovery. Materials and Methods: A total of 60 patients of either sex between 18 and 65 years of age, belonging to the American Society of Anesthesiologists I and II who were undergoing elective transnasal transsphenoidal pituitary surgery were chosen for this study. Patients were randomly allocated into two groups, Group C (clonidine) and Group D (dexmedetomidine), with each group consisting of 30 patients. Patients in Group C received 200 μg tablet of clonidine and those in Group D received a pantoprazole tablet as placebo at the same time. Patients in the Group D received an intravenous infusion of dexmedetomidine diluted in 50 ml saline (200 μg in 50 ml saline) 10 min before induction and patients in Group C received 0.9% normal saline (50 ml) as placebo. The hemodynamic variables (heart rate, mean arterial pressure) were noted at various stages of the surgery. Statistical analysis of the data was performed. Results: A total of 60 patients were recruited. The mean age, sex, weight and duration of surgery among the two groups were comparable (P > 0.05). Both dexmedetomidine and clonidine failed to blunt the increase in hemodynamic responses (heart rate and blood pressure) during intubation, nasal packing, speculum insertion and extubation. However when the hemodynamic response was compared between the patients receiving dexmedetomidine and clonidine it was seen that patients who received dexmedetomidine had a lesser increase in heart rate and blood pressure (P < 0.05) when compared to clonidine. Conclusions: A

A Comparison of Dexmedetomidine and Clonidine in Attenuating the Hemodynamic Responses at Various Surgical Stages in Patients Undergoing Elective Transnasal Transsphenoidal Resection of Pituitary Tumors.

PubMed

Jan, Summaira; Ali, Zulfiqar; Nisar, Yasir; Naqash, Imtiaz Ahmad; Zahoor, Syed Amer; Langoo, Shabir Ahmad; Azhar, Khan

2017-01-01

Transsphenoidal approach to pituitary tumors is a commonly performed procedure with the advantage of a rapid midline access to the sella with minimal complications. It may be associated with wide fluctuations in hemodynamic parameters due to intense noxious stimulus at various stages of the surgery. As duration of the surgery is short and the patients have nasal packs, it is prudent to use an anesthestic technique with an early predictable recovery. A total of 60 patients of either sex between 18 and 65 years of age, belonging to the American Society of Anesthesiologists I and II who were undergoing elective transnasal transsphenoidal pituitary surgery were chosen for this study. Patients were randomly allocated into two groups, Group C (clonidine) and Group D (dexmedetomidine), with each group consisting of 30 patients. Patients in Group C received 200 μg tablet of clonidine and those in Group D received a pantoprazole tablet as placebo at the same time. Patients in the Group D received an intravenous infusion of dexmedetomidine diluted in 50 ml saline (200 μg in 50 ml saline) 10 min before induction and patients in Group C received 0.9% normal saline (50 ml) as placebo. The hemodynamic variables (heart rate, mean arterial pressure) were noted at various stages of the surgery. Statistical analysis of the data was performed. A total of 60 patients were recruited. The mean age, sex, weight and duration of surgery among the two groups were comparable ( P > 0.05). Both dexmedetomidine and clonidine failed to blunt the increase in hemodynamic responses (heart rate and blood pressure) during intubation, nasal packing, speculum insertion and extubation. However when the hemodynamic response was compared between the patients receiving dexmedetomidine and clonidine it was seen that patients who received dexmedetomidine had a lesser increase in heart rate and blood pressure ( P < 0.05) when compared to clonidine. A continuous intravenous infusion of dexmedetomidine as

Lycopene and Beta-Carotene Induce Growth Inhibition and Proapoptotic Effects on ACTH-Secreting Pituitary Adenoma Cells

PubMed Central

Leite de Oliveira, Felipe; Soares, Nathália; de Mattos, Rômulo Medina; Hecht, Fábio; Dezonne, Rômulo Sperduto; Vairo, Leandro; Goldenberg, Regina Coeli dos Santos; Gomes, Flávia Carvalho Alcântara; de Carvalho, Denise Pires; Gadelha, Mônica R.; Nasciutti, Luiz Eurico; Miranda-Alves, Leandro

2013-01-01

Pituitary adenomas comprise approximately 10–15% of intracranial tumors and result in morbidity associated with altered hormonal patterns, therapy and compression of adjacent sella turcica structures. The use of functional foods containing carotenoids contributes to reduce the risk of chronic diseases such as cancer and vascular disorders. In this study, we evaluated the influence of different concentrations of beta-carotene and lycopene on cell viability, colony formation, cell cycle, apoptosis, hormone secretion, intercellular communication and expression of connexin 43, Skp2 and p27kip1 in ACTH-secreting pituitary adenoma cells, the AtT20 cells, incubated for 48 and 96 h with these carotenoids. We observed a decrease in cell viability caused by the lycopene and beta-carotene treatments; in these conditions, the clonogenic ability of the cells was also significantly decreased. Cell cycle analysis revealed that beta-carotene induced an increase of the cells in S and G2/M phases; furthermore, lycopene increased the proportion of these cells in G0/G1 while decreasing the S and G2/M phases. Also, carotenoids induced apoptosis after 96 h. Lycopene and beta-carotene decreased the secretion of ACTH in AtT20 cells in a dose-dependent manner. Carotenoids blocked the gap junction intercellular communication. In addition, the treatments increased the expression of phosphorylated connexin43. Finally, we also demonstrate decreased expression of S-phase kinase-associated protein 2 (Skp2) and increased expression of p27kip1 in carotenoid-treated cells. These results show that lycopene and beta-carotene were able to negatively modulate events related to the malignant phenotype of AtT-20 cells, through a mechanism that could involve changes in the expression of connexin 43, Skp2 and p27kip1; and suggest that these compounds might provide a novel pharmacological approach to the treatment of Cushing’s disease. PMID:23667519

Normal dimensions of the posterior pituitary bright spot on magnetic resonance imaging.

PubMed

Côté, Martin; Salzman, Karen L; Sorour, Mohammad; Couldwell, William T

2014-02-01

The normal pituitary bright spot seen on unenhanced T1-weighted MRI is thought to result from the T1-shortening effect of the vasopressin stored in the posterior pituitary. Individual variations in its size may be difficult to differentiate from pathological conditions resulting in either absence of the pituitary bright spot or in T1-hyperintense lesions of the sella. The objective of this paper was to define a range of normal dimensions of the pituitary bright spot and to illustrate some of the most commonly encountered pathologies that result in absence or enlargement of the pituitary bright spot. The authors selected normal pituitary MRI studies from 106 patients with no pituitary abnormality. The size of each pituitary bright spot was measured in the longest axis and in the dimension perpendicular to this axis to describe the typical dimensions. The authors also present cases of patients with pituitary abnormalities to highlight the differences and potential overlap between normal and pathological pituitary imaging. All of the studies evaluated were found to have pituitary bright spots, and the mean dimensions were 4.8 mm in the long axis and 2.4 mm in the short axis. The dimension of the pituitary bright spot in the long axis decreased with patient age. The distribution of dimensions of the pituitary bright spot was normal, indicating that 99.7% of patients should have a pituitary bright spot measuring between 1.2 and 8.5 mm in its long axis and between 0.4 and 4.4 mm in its short axis, an interval corresponding to 3 standard deviations below and above the mean. In cases where the dimension of the pituitary bright spot is outside this range, pathological conditions should be considered. The pituitary bright spot should always be demonstrated on T1-weighted MRI, and its dimensions should be within the identified normal range in most patients. Outside of this range, pathological conditions affecting the pituitary bright spot should be considered.

Retinal projections in the bowfin, Amia calva: cytoarchitectonic and experimental analysis.

PubMed

Butler, A B; Northcutt, R G

1992-01-01

The retinofugal projections in the bowfin, a non-teleost actinopterygian, were studied by autoradiographic and horseradish peroxidase methods, and the cytoarchitecture of retinorecipient regions of the diencephalon was analyzed with serially sectioned, Bodian stained material. Nuclei were identified in the thalamus, the periventricular portion of the posterior tuberculum, synencephalon, and pretectum which are homologous to like-named nuclei in teleosts and other non-teleost actinopterygian fishes. Of particular note, a posterior pretectal nucleus and, possibly, a homologue of nucleus corticalis were found to be present in the pretectum. These nuclei have previously been identified only in teleosts. The posterior pretectal nucleus is relatively small in the bowfin, and the distribution of a small, versus a large, posterior pretectal nucleus in Teleostei and Halecomorphi suggests that this nucleus was small plesiomorphically. The pattern of retinofugal projections in the bowfin is similar to that in other non-teleost actinopterygian fishes and in teleosts in most regards. Contralaterally, the retina projects to nuclei in the dorsal and ventral thalamus, superficial and central pretectum, dorsal and ventral accessory optic nuclei, and to the optic tectum. Additionally, there are sparse projections to the suprachiasmatic nucleus in the preoptic area, the periventricular nucleus of the posterior tuberculum, and the dorsal and ventral periventricular pretectal nuclei. Ipsilateral projections are sparse and are derived from fibers which do not decussate in the optic chiasm. Undecussated ipsilateral retinal projections, as present in the bowfin, are a widely distributed character in vertebrates and appear to be plesiomorphic for vertebrates.

Temporal distribution and behaviour of sand flies (Diptera: Psychodidae) in a cutaneous leishmaniasis focus of the Kani Tribe settlements in the Western Ghats, India.

PubMed

Srinivasan, R; Jambulingam, P; Kumar, N Pradeep; Selvakumar, M; Edwin, B; Kumar, T Dilip

2015-08-01

The temporal distribution of sand flies in relation to environmental factors was studied in the Kani tribe settlements located on the southernmost part of the Western Ghats, Kerala, India, between June 2012 and May 2013. This area is known for occurrence of cutaneous leishmaniasis (CL) cases. Employing hand-held aspirator, light trap and sticky-trap collection methods, a total of 7874 sand fly specimens, comprising 19 species was collected. Sergentomyia baghdadis was predominant species, followed by Phlebotomus argentipes. Sand fly abundance was significantly higher indoors (χ(2)=9241.8; p=0.0001) than outdoors. Mean density of P. argentipes in human dwellings, cattle sheds and outdoors was 7.2±2.9, 27.33±21.1 and 0.64±0.2 females/per man-hour (MHR), respectively. No sand fly species other than P. argentipes was obtained from cattle sheds. Although, sand fly populations were prevalent throughout the year, their abundance fluctuated with seasonal changes. Multiple regression analysis with backward elimination indicated that the increase in precipitation and relative humidity contributed to a significant positive association with the increase in sand fly abundance, while the increase in temperature showed no association. Fully engorged female sand flies tested for blood meal source showed multiple host-blood feeding. Analysis of resting populations of sand flies collected from human shelters indicated that the populations were found maximum on interior walls at 6-8 and >8 ft height, including ceiling during summer (F=83.7, df=6, p=0.001) and at the lower half of the wall at 0 and 0-2 ft height, during monsoon season (F=41.4, df=6, p=0.001). In cooler months, no preference to any height level (F=1.67, df=6, p=0.2) was observed. Proportion of females sand flies with Sella's classification of abdominal stages, namely full-fed, half-gravid and gravid females did not vary significantly (t=1.98, p=0.13827) indoors, confirming their endophilic behaviour. Risk of CL

Comparison of axial and flexural stresses in lordosis and three buckled configurations of the cervical spine.

PubMed

Harrison, D E; Harrison, D D; Janik, T J; William Jones, E; Cailliet, R; Normand, M

2001-05-01

To calculate and compare combined axial and flexural stresses in lordosis versus buckled configurations of the sagittal cervical curve. Digitized measurements from lateral cervical radiographs of four different shapes were used to calculate axial loads and bending moments on the vertebral bodies of C2-C7.Background. Osteoarthritis and spinal degeneration are factors in neck and back pain. Calculations of stress in clinically occurring configurations of the sagittal cervical spine are rare. Center of gravity of the head (inferior-posterior sella turcica) and vertebral body margins were digitized on four different lateral cervical radiographs: lordosis, kyphosis, and two "S"-shapes. Polynomials (seventh degree) and stress concentrations on the concave and convex margins were derived for the shape of the sagittal cervical curvatures from C1 to T1. Moments of inertia were determined from digitizing and the use of an elliptical shell model of cross-section. Moment arms from a vertical line through the center of gravity of the head to the atlas and scaled neck extensor moment arms from the literature were used to compute the vertical component of extensor muscle effort. Segmental lever arms were calculated from a vertical line through C1 to each vertebra. In lordosis, anterior and posterior stresses in the vertebral body are nearly uniform and minimal. In kyphotic areas, combined stresses changed from tension to compression at the anterior vertebral margins and were very large (6-10 times as large in magnitude) compared to lordosis. In kyphotic areas at the posterior vertebral body, the combined stresses changed from compression (in lordosis) to tension. The stresses in kyphotic areas are very large and opposite in direction compared to a normal lordosis. This analysis provides the basis for the formation of osteophytes (Wolff's Law) on the anterior margins of vertebrae in kyphotic regions of the sagittal cervical curve. This indicates that any kyphosis is an undesirable

[Evaluation of three methods for constructing craniofacial mid-sagittal plane based on the cone beam computed tomography].

PubMed

Wang, S W; Li, M; Yang, H F; Zhao, Y J; Wang, Y; Liu, Y

2016-04-18

To compare the accuracyof interactive closet point (ICP) algorithm, Procrustes analysis (PA) algorithm,and a landmark-independent method to construct the mid-sagittal plane (MSP) of the cone beam computed tomography.To provide theoretical basis for establishing coordinate systemof CBCT images and symmetric analysis. Ten patients were selected and scanned by CBCT before orthodontic treatment.The scan data was imported into Mimics 10.0 to reconstructthree dimensional skulls.And the MSP of each skull was generated by ICP algorithm, PA algorithm and landmark-independent method. MSP extracted by ICP algorithm or PA algorithm involvedthree steps. First, the 3D skull processing was performed by reverse engineering software geomagic studio 2012 to obtain the mirror skull. Then, the original and its mirror skull was registered separately by ICP algorithm in geomagic studio 2012 and PA algorithm in NX Imageware 11.0. Finally, the registered data were united into new data to calculate the MSP of the originaldata in geomagic studio 2012. The mid-sagittal plane was determined by SELLA (S), nasion (N), basion (Ba) as traditional landmark-dependent methodconducted in software InVivoDental 5.0. The distance from 9 pairs of symmetric anatomical marked points to three sagittal plane were measured and calculated to compare the differences of the absolute value. The one-way ANOVA test was used to analyze the variable differences among the 3 MSPs. The pairwise comparison was performed with LSD method. MSPs calculated by the three methods were available for clinic analysis, which could be concluded from the front view.However, there was significant differences among the distances from the 9 pairs of symmetric anatomical marked points to the MSPs (F=10.932,P=0.001).LSD test showed there was no significant difference between the ICP algorithm and landmark-independent method (P=0.11), while there was significant difference between the PA algorithm and landmark-independent methods (P=0

Prevalence of hypopituitarism after intracranial operations not directly associated with the pituitary gland

PubMed Central

2013-01-01

Background Over the last few years, awareness and detection rates of hypopituitarism following traumatic brain injury (TBI) and subarachnoid hemorrhage (SAH) has steadily increased. Moreover, recent studies have found that a clinically relevant number of patients develop pituitary insufficiency after intracranial operations and radiation treatment for non-pituitary tumors. But, in a substantial portion of more than 40%, the hypopituitarism already exists before surgery. We sought to determine the frequency, pattern, and severity of endocrine disturbances using basal and advanced dynamic pituitary testing following non-pituitary intracranial procedures. Methods 51 patients (29 women, 22 men) with a mean age of 55 years (range of 20 to 75 years) underwent prospective evaluation of basal parameters and pituitary function testing (combined growth hormone releasing hormone (GHRH)/arginine test, insulin tolerance test (ITT), low dose adrenocorticotropic hormone (ACTH) test), performed 5 to 168 months (median 47.2 months) after intracranial operation (4 patients had additional radiation and 2 patients received additional radiation combined with chemotherapy). Results We discovered an overall rate of hypopituitarism with distinct magnitude in 64.7% (solitary in 45.1%, multiple in 19.6%, complete in 0%). Adrenocorticotropic hormone insufficiency was found in 51.0% (partial in 41.2%, complete in 9.8%) and growth hormone deficiency (GHD) occurred in 31.4% (partial in 25.5%, severe in 5.9%). Thyrotropic hormone deficiency was not identified. The frequency of hypogonadism was 9.1% in men. Pituitary deficits were associated with operations both in close proximity to the sella turcica and more distant regions (p = 0.91). Age (p = 0.76) and gender (p = 0.24) did not significantly differ across patients with versus those without hormonal deficiencies. Groups did not significantly differ across pathology and operation type (p = 0.07). Conclusion Hypopituitarism

Prevalence of hypopituitarism after intracranial operations not directly associated with the pituitary gland.

PubMed

Fleck, Steffen Kristian; Wallaschofski, Henri; Rosenstengel, Christian; Matthes, Marc; Kohlmann, Thomas; Nauck, Matthias; Schroeder, Henry Werner Siegfried; Spielhagen, Christin

2013-11-04

Over the last few years, awareness and detection rates of hypopituitarism following traumatic brain injury (TBI) and subarachnoid hemorrhage (SAH) has steadily increased. Moreover, recent studies have found that a clinically relevant number of patients develop pituitary insufficiency after intracranial operations and radiation treatment for non-pituitary tumors. But, in a substantial portion of more than 40%, the hypopituitarism already exists before surgery. We sought to determine the frequency, pattern, and severity of endocrine disturbances using basal and advanced dynamic pituitary testing following non-pituitary intracranial procedures. 51 patients (29 women, 22 men) with a mean age of 55 years (range of 20 to 75 years) underwent prospective evaluation of basal parameters and pituitary function testing (combined growth hormone releasing hormone (GHRH)/arginine test, insulin tolerance test (ITT), low dose adrenocorticotropic hormone (ACTH) test), performed 5 to 168 months (median 47.2 months) after intracranial operation (4 patients had additional radiation and 2 patients received additional radiation combined with chemotherapy). We discovered an overall rate of hypopituitarism with distinct magnitude in 64.7% (solitary in 45.1%, multiple in 19.6%, complete in 0%). Adrenocorticotropic hormone insufficiency was found in 51.0% (partial in 41.2%, complete in 9.8%) and growth hormone deficiency (GHD) occurred in 31.4% (partial in 25.5%, severe in 5.9%). Thyrotropic hormone deficiency was not identified. The frequency of hypogonadism was 9.1% in men. Pituitary deficits were associated with operations both in close proximity to the sella turcica and more distant regions (p = 0.91). Age (p = 0.76) and gender (p = 0.24) did not significantly differ across patients with versus those without hormonal deficiencies. Groups did not significantly differ across pathology and operation type (p = 0.07). Hypopituitarism occurs more frequently than expected in

Giovanni Verga (1879-1923), author of a pioneering treatise on pituitary surgery: the foundations of this new field in Europe in the early 1900s.

PubMed

Pascual, José M; Mongardi, Lorenzo; Prieto, Ruth; Castro-Dufourny, Inés; Rosdolsky, María; Strauss, Sewan; Carrasco, Rodrigo; Winter, Eduard; Mazzarello, Paolo

2017-10-01

The field of pituitary surgery was born in the first decade of the twentieth century in Europe, and it evolved rapidly with the development of numerous innovative surgical techniques by some of the founding fathers of neurosurgery. This study investigates the pioneering Italian treatise on pituitary surgery, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), published in 1911 by Giovanni Verga (1879-1923), a surgeon from Pavía and one of Golgi's disciples. This little-known monograph compiles the earliest experience on pituitary surgery through the analysis of the first 50 procedures performed between 1903 and 1911. We conducted a biographical survey of Giovanni Verga and the motivations for his work on pituitary surgery. In addition, a systematic analysis of all original reports and historical documents about these pituitary procedures referenced in Verga's treatise was carried out. Verga's treatise provides a summary of the techniques employed and surgical outcomes for the first 50 attempted procedures of pituitary tumor removal. This monograph is the only scientific source that includes a complete account of the series of 10 pituitary tumors operated on by Sir Victor Horsley in the 1900s. Three major types of surgery were employed: (i) palliative procedures of craniectomy (n = 6); (ii) transcranial approaches to the pituitary gland, either subfrontal or subtemporal (n = 13); and (iii) transphenoidal routes to expose the sella turcica, either using an upper transnasal-transethmoidal approach (n = 19) or a lower sublabial/endonasal-transeptal one (n = 12). An operative mortality rate of 36% (n = 17) was observed in these early series. The pathological nature of the tumors operated on was available in 42 cases. There were 28 adenomas and 15 craniopharyngiomas. Sir Victor Horsley (1857-1916) and the Viennese surgeons Anton von Eiselsberg (1860-1939) and Oskar Hirsch (1877-1965) were the leading European figures in the development

Somatotroph Hyperplasia

PubMed Central

Thorner, M. O.; Perryman, R. L.; Cronin, M. J.; Rogol, A. D.; Draznin, M.; Johanson, A.; Vale, W.; Horvath, E.; Kovacs, K.

1982-01-01

A 21-yr-old woman with Turner's syndrome presented with signs and symptoms of acromegaly. The serum growth hormone (GH) (95±9.4 ng/ml; mean±SEM) and somatomedin C (11 U/ml) levels were elevated, and an increase in GH levels after glucose instead of normal suppression, increase after thyrotropin-releasing hormone (TRH) administration instead of no change, and decrease after dopamine administration instead of stimulation were observed. The pituitary fossa volume was greater than normal (1,440 mm3) and the presence of a pituitary tumor was assumed. After tissue removal at transsphenoidal surgery, histological study revealed somatotroph hyperplasia rather than a discrete adenoma. Postoperatively, she remained clinically acromegalic and continued to show increased GH and somatomedin levels. A search was made for ectopic source of a growth hormone-releasing factor (GRF). Computer tomographic scan revealed a 5-cm Diam tumor in the tail of the pancreas. Following removal of this tumor, serum GH fell from 70 to 3 ng/ml over 2 h, and remained low for the subsequent 5 mo. Serum somatomedin C levels fell from 7.2 to normal by 6 wk postoperatively. There were no longer paradoxical GH responses to glucose, TRH, and dopamine. Both the medium that held the tumor cells at surgery and extracts of the tumor contained a peptide with GRF activity. The GRF contained in the tumor extract coeluted on Sephadex G-50 chromatography with rat hypothalamic GH-releasing activity. Stimulation of GH from rat somatotrophs in vitro was achieved at the nanomolar range, using the tumor extract. The patient's course demonstrates the importance of careful interpretation of pituitary histology. Elevated serum GH and somatomedin C levels in a patient with an enlarged sella turcica and the characteristic responses seen in acromegaly to TRH, dopamine, and glucose do not occur exclusively in patients with discrete pituitary tumors and acromegaly. This condition can also occur with somatotroph hyperplasia

Accuracy of Orthognathic Surgical Outcomes Using 2- and 3-Dimensional Landmarks-The Case for Apples and Oranges?

PubMed

Borba, Alexandre Meireles; José da Silva, Everton; Fernandes da Silva, André Luis; Han, Michael D; da Graça Naclério-Homem, Maria; Miloro, Michael

2018-01-12

To verify predicted versus obtained surgical movements in 2-dimensional (2D) and 3-dimensional (3D) measurements and compare the equivalence between these methods. A retrospective observational study of bimaxillary orthognathic surgeries was performed. Postoperative cone-beam computed tomographic (CBCT) scans were superimposed on preoperative scans and a lateral cephalometric radiograph was generated from each CBCT scan. After identification of the sella, nasion, and upper central incisor tip landmarks on 2D and 3D images, actual and planned movements were compared by cephalometric measurements. One-sample t test was used to statistically evaluate results, with expected mean discrepancy values ranging from 0 to 2 mm. Equivalence of 2D and 3D values was compared using paired t test. The final sample of 46 cases showed by 2D cephalometry that differences between actual and planned movements in the horizontal axis were statistically relevant for expected means of 0, 0.5, and 2 mm without relevance for expected means of 1 and 1.5 mm; vertical movements were statistically relevant for expected means of 0 and 0.5 mm without relevance for expected means of 1, 1.5, and 2 mm. For 3D cephalometry in the horizontal axis, there were statistically relevant differences for expected means of 0, 1.5, and 2 mm without relevance for expected means of 0.5 and 1 mm; vertical movements showed statistically relevant differences for expected means of 0, 0.5, 1.5 and 2 mm without relevance for the expected mean of 1 mm. Comparison of 2D and 3D values displayed statistical differences for the horizontal and vertical axes. Comparison of 2D and 3D surgical outcome assessments should be performed with caution because there seems to be a difference in acceptable levels of accuracy between these 2 methods of evaluation. Moreover, 3D accuracy studies should no longer rely on a 2-mm level of discrepancy but on a 1-mm level. Copyright © 2018 American Association of Oral and Maxillofacial

Management of Cushing's disease: outcome in patients with microadenoma detected on pituitary magnetic resonance imaging.

PubMed

Prevedello, Daniel M; Pouratian, Nader; Sherman, Jonathan; Jane, John A; Vance, Mary Lee; Lopes, M Beatriz; Laws, Edward R

2008-10-01

Outcomes of therapy for Cushing's disease (CD) vary depending on different aspects of presentation and diagnostic studies. The authors designed this study to verify the remission rate and outcomes after transsphenoidal surgery (TSS) for patients with CD who had positive findings on MR imaging. Patients who had presented with CD at the University of Virginia for initial treatment between July 1992 and December 2005 were retrospectively reviewed. The patients included in the study were considered to be optimal surgical candidates, defined as an adult (>18 years of age) with classic clinical features of CD, laboratory studies confirming a central (pituitary/hypothalamic) adrenocorticotropic hormone-dependent source of disease, and an MR imaging study revealing a microadenoma in the sella turcica. A total of 167 patients fulfilled the criteria. Thirty were men (18%) and 137 were women (82%). The mean age was 42.3 years (range 18.2-77 years). All patients underwent TSS. Surgical remission was achieved in 148 patients (88.6%), which was correlated with the surgeon's intraoperative identification of an adenoma (p=0.03). Histopathological confirmation of an adrenocorticotropic hormone-secreting adenoma strongly correlated with remission (p=0.0001). Three patients (1.8%) had postoperative cerebrospinal fluid leaks, and 1 patient had meningitis. Transient diabetes insipidus was diagnosed and treated in 10 patients (6%), whereas permanent diabetes insipidus occurred in 8 patients (4.8%). Panhypopituitarism followed the surgery in 14 patients (8.3%), 13 of whom underwent total hypophysectomy (9 initially and 4 with early reoperations), and in 1 of 10 patients who underwent subtotal hypophysectomy. Nineteen patients (12.8%) who were initially in remission developed recurrent CD after an average of 50 months. The mean follow-up for the 167 patients was 39 months (range 6-157 months). Gamma Knife surgery was the most common modality of radiotherapy used to treat 31 patients (18

The pituitary gland in patients with Langerhans cell histiocytosis: a clinical and radiological evaluation.

PubMed

Kurtulmus, Neslihan; Mert, Meral; Tanakol, Refik; Yarman, Sema

2015-04-01

Langerhans cell histiocytosis (LCH) is a rare disease in which the most common endocrine manifestation is diabetes insipidus (DI). Data on anterior pituitary function in patients with LCH are limited. Thus, the present study investigated anterior pituitary function in LCH patients with DI via the evaluation of clinical and radiological findings at disease onset and during follow-up. The present study retrospectively evaluated nine patients with LCH (five males and four females). All diagnoses of LCH were made following histological and/or immunophenotypic analyses of tissue biopsies, bronchoalveolar lavage, or cerebrospinal fluid (CSF). Basal and, if necessary, dynamic pituitary function tests were used to assess anterior pituitary function, and magnetic resonance imaging (MRI) scans were used to image the pituitary. The LCH treatment modality was based on organ involvement. The mean age at onset of DI was 27.6 years (range 15-60 years). One patient (11%) exhibited single organ involvement, while eight patients (89%) displayed multisystem organ involvement. On admittance, one patient had hypogonadotropic hypogonadism, one patient exhibited panhypopituitarism [hypogonadotropic hypogonadism, central hypothyroidism, hypocortisolism, and growth hormone (GH) deficiency], and four patients (44%) displayed hyperprolactinemia. The MRI data revealed infundibular enlargement in seven patients (78%), a thalamic mass in one patient (11%), and the absence of the bright spot in all patients. A single patient (11%) showed a mass in the pons that had a partially empty sella. The patients were treated with radiation therapy (RT), chemotherapy (CT), or a combination of both (RT+CT) and were followed up for a median of 91.8 months (range 2-318 months). Seven patients were assessed during the follow-up period, of whom four patients (57.1%) developed anterior pituitary hormone deficiency, three (43%) were diagnosed with GH deficiency, and one (14%) exhibited gonadotropin deficiency

Influence of orthodontic appliance-derived artifacts on 3-T MRI movies.

PubMed

Ozawa, Erika; Honda, Ei-Ichi; Parakonthun, Kulthida Nunthayanon; Ohmori, Hiroko; Shimazaki, Kazuo; Kurabayashi, Tohru; Ono, Takashi

2018-02-19

Magnetic resonance imaging (MRI) has been used to study configurations of speech organs in the resting state. However, MRI is sensitive to metals, and numerous types of metallic appliances, most of which have a large magnetic susceptibility, are used in orthodontic treatment and may cause severe artifacts on MRI. We have developed techniques for obtaining MRI movies of the oral region, to evaluate articulatory changes, especially movement of the tongue, palate, and teeth, pre- and post-orthodontic/orthognathic treatment. We evaluated the influence of artifacts caused by orthodontic appliances, including fixed retainers, metal brackets, and wires, on measurements in 3-T MRI movies. Sixteen healthy young adults (nine males, seven females; average age, 27 years) with normal occlusion were recruited. Four types of customized maxillary and mandibular plates were prepared by incorporating one of the following into the plate: (a) nothing, (b) a fixed canine-to-canine retainer, (c) metal brackets for the anterior and molar teeth, or (d) clear brackets for the anterior teeth and metal brackets for molars. A 3-T MRI movie, in segmented cine mode, was generated for each plate condition while participants pronounced a vowel-consonant-vowel syllable (/asa/). The size of the artifact due to the metallic brackets was measured. The face size and orthodontically important anatomical structures, such as the velum, the hard palate, and the laryngeal ventricle, were also measured. A large artifact was observed over the entire oral region around orthodontic appliances, altering regional visibility. The velopharyngeal height was measured as significantly longer in the presence of metal brackets. The maximum artifact size due to a metallic bracket was > 8 cm. Our results show that even if it is possible to obtain the measurements of palate length, nasion to sella, and nasion to basion in individuals wearing metal brackets for molars, the measurements might be affected due to the

Multimodal Navigation in Endoscopic Transsphenoidal Resection of Pituitary Tumors using Image-based Vascular and Cranial Nerve Segmentation: A Prospective Validation Study

PubMed Central

Dolati, Parviz; Eichberg, Daniel; Golby, Alexandra; Zamani, Amir; Laws, Edward

2016-01-01

Introduction Transsphenoidal surgery (TSS) is a well-known approach for the treatment of pituitary tumors. However, lateral misdirection and vascular damage, intraoperative CSF leakage, and optic nerve and vascular injuries are all well-known complications, and the risk of adverse events is more likely in less experienced hands. This prospective study was conducted to validate the accuracy of image-based segmentation in localization of neurovascular structures during TSS. Methods Twenty-five patients with pituitary tumors underwent preoperative 3TMRI, which included thin-sectioned 3D space T2, 3D Time of Flight and MPRAGE sequences. Images were reviewed by an expert independent neuroradiologist. Imaging sequences were loaded in BrainLab iPlanNet (16/25 cases) or Stryker (9/25 cases) image guidance platforms for segmentation and pre-operative planning. After patient registration into the neuronavigation system and subsequent surgical exposure, each segmented neural or vascular element was validated by manual placement of the navigation probe on or as close as possible to the target. The audible pulsations of the bilateral ICA were confirmed using a micro-Doppler probe. Results Pre-operative segmentation of the ICA and cavernous sinus matched with the intra-operative endoscopic and micro-Doppler findings in all cases (Dice Similarity Coefficient =1). This information reassured the surgeons with regard to the lateral extent of bone removal at the sellar floor and the limits of lateral exploration. Excellent correspondence between image-based segmentation and the endoscopic view was also evident at the surface of the tumor and at the tumor-normal gland interfaces. This assisted in preventing unnecessary removal of the normal pituitary gland. Image-guidance assisted the surgeons in localizing the optic nerve and chiasm in 64% of the cases and the diaphragma sella in 52% of cases, which helped to determine the limits of upward exploration and to decrease the risk of CSF

Central projections of the nervus terminalis and the nervus praeopticus in the lungfish brain revealed by nitric oxide synthase.

PubMed

Schober, A; Meyer, D L; Von Bartheld, C S

1994-11-01

Lungfishes possess two cranial nerves that are associated with the olfactory system: the nervus terminalis enters the telencephalon with the olfactory nerve, and the nervus praeopticus enters the diencephalon at the level of the optic nerve. We investigated the central projections of the nervus terminalis and the nervus praeopticus in the Australian lungfish (Neoceratodus forsteri) and in the African lungfish (Protopterus dolloi) by NADPH-diaphorase histochemistry (nitric oxide synthase; NOS) and compared them with the projections of the nervus terminalis of the frog (Xenopus laevis). In Neoceratodus, NOS-positive fascicles of the nervus terminalis divide and project with a ventral component through the septum and with a dorsal component through the pallium; fibers of both trajectories extend caudally beyond the anterior commissure and join the lateral forebrain bundle. In the nervus praeopticus, about 300 fibers contain NOS; they innervate the preoptic nucleus and continue their course through the diencephalon; many fibers cross in the commissure of the posterior tuberculum. In Protopterus, ganglion cells of the nervus terminalis and of the nervus praeopticus contain NOS. NOS-positive fibers of the nervus terminalis project through the septal region but not through the pallium. Several major fascicles cross in the rostral part of the anterior commissure, where they are joined by a small number of NOS-containing fibers of the nervus praeopticus. Both nerves innervate the preoptic nucleus. The number and pathways of the fascicles of the nervus terminalis are not always symmetric between the two sides. The nervus terminalis fascicles remain in a ventral position, whereas the nervus praeopticus gives rise to the more dorsal fascicles. Many fibers of the two nerves extend throughout the diencephalon and cross in the commissure of the posterior tuberculum. These findings demonstrate many similarities but also significant differences between the contributions of the

Orthognathic surgery in the young cleft patient: preliminary study on subsequent facial growth.

PubMed

Wolford, Larry M; Cassano, Daniel Serra; Cottrell, David A; El Deeb, Mohamed; Karras, Spiro C; Goncalves, Joao Roberto

2008-12-01

This study evaluated the long-term effects of orthognathic surgery on subsequent growth of the maxillomandibular complex in the young cleft patient. We evaluated 12 young cleft patients (9 male and 3 female patients), with a mean age of 12 years 6 months (range, 9 years 8 months to 15 years 4 months), who underwent Le Fort I osteotomies, with maxillary advancement, expansion, and/or downgrafting, by use of autogenous bone or hydroxyapatite grafts, when indicated, for maxillary stabilization. Five patients had concomitant osteotomies of the mandibular ramus. All patients had presurgical and postsurgical orthodontic treatment to control the occlusion. Radiographs taken at initial evaluation (T1) and presurgery (T2) were compared to establish the facial growth vector before surgery, whereas radiographs taken immediately postsurgery (T3) and at longest follow-up (T4) were used to determine postsurgical growth. Each patient's lateral cephalograms were traced, and 16 landmarks were identified and used to compute 11 measurements describing presurgical and postsurgical growth. Before surgery, all patients had relatively normal growth. After surgery, cephalograms showed statistically significant growth changes from T3 to T4, with the maxillary depth decreasing by -3.3 degrees +/- 1.8 degrees , Sella-nasion-point A by -3.3 degrees +/- 1.8 degrees, and point A-nasion-point B by -3.6 degrees +/- 2.8 degrees. The angulation of the maxillary incisors increased by 9.2 degrees +/- 11.7 degrees. Of 12 patients, 11 showed disproportionate postsurgical jaw growth. Maxillary growth occurred predominantly in a vertical vector with no anteroposterior growth, even though most patients had shown anteroposterior growth before surgery. The distance increased in the linear measurement from nasion to gnathion by 10.3 +/- 7.9 mm. Four of 5 patients operated on during the mixed dentition phase had teeth that erupted through the cleft area. A variable impairment of postoperative growth was seen

Three-dimensional adult male head and skull contours.

PubMed

Lee, Calvin; Loyd, Andre M; Nightingale, Roger; Myers, Barry S; Damon, Andrew; Bass, Cameron R

2014-01-01

Traumatic brain injury (TBI) is a major public health issue, affecting millions of people annually. Anthropomorphic test devices (ATDs) and finite element models (FEMs) provide a means of understanding factors leading to TBI, potentially reducing the occurrence. Thus, there is a need to ensure that these tools accurately model humans. For example, the Hybrid III was not based on 3-dimensional human head shape data. The objective of this study is to produce average head and skull contours for an average U.S. male that can be used for ATDs and FEMs. Computed tomography (CT) scans of adult male heads were obtained from a database provided by the University of Virginia Center for Applied Biomechanics. An orthographic viewer was used to extract head and skull contours from the CT scans. Landmarks were measured graphically using HyperMesh (Altair, HyperWorks). To determine the head occipital condyle (OC) centroid, surface meshes of the OCs were made and the centroid of the surfaces was calculated. The Hybrid III contour was obtained using a MicroScribe Digitizer (Solution Technologies, Inc., Oella, MD). Comparisons of the average male and ATD contours were performed using 2 methods: (1) the midsagittal and midcoronal ATD contours relative to the OC centroid were compared to the corresponding 1 SD range of the average male contours; (2) the ATD sagittal contour was translated relative to the average male sagittal contour to minimize the area between the 2 contours. Average male head and skull contours were created. Landmark measurements were made for the dorsum sellae, nasion skin, nasion bone, infraorbital foramen, and external auditory meatus, all relative to the OC centroid. The Hybrid III midsagittal contour was outside the 1 SD range for 15.2 percent of the average male head contour but only by a maximum distance of 1.5 mm, whereas the Hybrid III midcoronal head contour was outside the 1 SD range for 12.2 percent of the average male head contour by a maximum distance

Radiologically defining horizontal gaze using EOS imaging-a prospective study of healthy subjects and a retrospective audit.

PubMed

Hey, Hwee Weng Dennis; Tan, Kimberly-Anne; Ho, Vivienne Chien-Lin; Azhar, Syifa Bte; Lim, Joel-Louis; Liu, Gabriel Ka-Po; Wong, Hee-Kit

2018-06-01

As sagittal alignment of the cervical spine is important for maintaining horizontal gaze, it is important to determine the former for surgical correction. However, horizontal gaze remains poorly-defined from a radiological point of view. The objective of this study was to establish radiological criteria to define horizontal gaze. This study was conducted at a tertiary health-care institution over a 1-month period. A prospective cohort of healthy patients was used to determine the best radiological criteria for defining horizontal gaze. A retrospective cohort of patients without rigid spinal deformities was used to audit the incidence of horizontal gaze. Two categories of radiological parameters for determining horizontal gaze were tested: (1) the vertical offset distances of key identifiable structures from the horizontal gaze axis and (2) imaginary lines convergent with the horizontal gaze axis. Sixty-seven healthy subjects underwent whole-body EOS radiographs taken in a directed standing posture. Horizontal gaze was radiologically defined using each parameter, as represented by their means, 95% confidence intervals (CIs), and associated 2 standard deviations (SDs). Subsequently, applying the radiological criteria, we conducted a retrospective audit of such radiographs (before the implementation of a strict radioimaging standardization). The mean age of our prospective cohort was 46.8 years, whereas that of our retrospective cohort was 37.2 years. Gender was evenly distributed across both cohorts. The four parameters with the lowest 95% CI and 2 SD were the distance offsets of the midpoint of the hard palate (A) and the base of the sella turcica (B), the horizontal convergents formed by the tangential line to the hard palate (C), and the line joining the center of the orbital orifice with the internal occipital protuberance (D). In the prospective cohort, good sensitivity (>98%) was attained when two or more parameters were used. Audit using Criterion B

[Monoamine oxidase activity in rat pineal gland: comparison with brain areas, alteration during aging].

PubMed

Razygraev, A V; Taborskaya, K I; Volovik, K Yu; Bunina, A A; Petrosyan, M A

Using benzylamine as a substrate, the amine oxidase activity was determined in the pineal gland of adult rats and compared with the same activity in brain areas and pituitary. Two groups of rats aged 6-8 and 14-15 months were also compared on the basis of this activity. Benzylamine deaminating activity in the pineal gland was significantly higher than in the area preoptica medialis, the corpus mamillare, the tuberculum olfactorium, and the hypophysis, and lower than in the eminentia mediana. The significant increase of the activity in the pineal gland in animals of age from 6-8 to 14-15-months was revealed. Benzylamine deaminating activity in the pineal gland was totally inhibited by 0,002 mM R deprenyl, indicating the B type monoamine oxidase (MAO B) activity. Age-associated increase of MAO B activity in the pineal gland accompanied by decrease of glutathione peroxidase activity, reported earlier, can promote the oxidative damage in the pineal gland during aging.

Anatomical basis of LMA variations drive to different photosynthetic and water storage strategies in two Sesleria species from mountain dry grasslands

NASA Astrophysics Data System (ADS)

Puglielli, Giacomo; Fiore Crescente, Maria; Frattaroli, Anna Rita; Gratani, Loretta

2016-04-01

Plant and leaf traits directly affect ecosystem processes ensuring carbon, nutrient and water exchanges between soil and atmosphere through the photosynthetic activity. Nevertheless, a great within sites variation in plant and leaf traits can be found resulting in different adaptive strategies in coexisting species. Leaf mass per unit of leaf area (LMA) is an important trait to understand plant functional ecology being the outcome of leaf anatomy and related to photosynthesis. We hypothesized that LMA was the main predictor of the adaptive strategies of Sesleria nitida (S1) and Sesleria juncifolia (S2), growing on the screes and on the crests of the summit area, respectively, on Mount Terminillo (Central Apennines, Loc. Sella di Leonessa, 1895 m a.s.l.). To test our hypothesis we broke LMA down into anatomical components, leaf tissue density (LTD) and thickness (LT) and then relating them to gas exchange parameters on twenty plants per species cultivated ex situ. LTD explained 69% of LMA variations in S1 while the relationship with LT was not significant. Moreover, LTD was negatively correlated with LT in S1 driving to a 17% higher volume of the intercellular air spaces, which increases the CO2 partial pressure at the carboxylation sites. This result was also attested by the significant relationship between LTD and both net photosynthesis per unit leaf area (Aa) and mass (Am) (R= 0.56 and -0.49, respectively), highlighting the role of LTD in determining the photosynthetic process in S1. LMA scaled with both LTD and LT explaining 82% and 70% of LMA variations in S2. Moreover, the positive relationship between LTD and LT (R2 = 0.52) highlighted a coordination between the variables in controlling the photosynthetic process. In particular, LTD and LT controlled the transactions of carbon and water through the leaf surface, being positively related to Aa (R= 0.93 and 0.79 for LTD and LT, respectively). Nevertheless, an increase in LT and LTD decreased Am (R = -0.9 and

Penetrating gunshots to the head and lack of immediate incapacitation. II. Review of case reports.

PubMed

Karger, B

1995-01-01

Because of the enhanced intracranial tissue disruption (see companion paper) and the functional significance of the central nervous system, penetrating gunshot wounds of the head commonly result in immediate incapacitation. However, in the last century numerous publications reported sustained capability to act following penetrating gunshot wounds of the head. These are reviewed. A large number of case reports had to be excluded from re-examination because of doubtful capability to act or lack of morphological documentation. There remained 53 case reports from 42 sources for systematic analysis. Favourable conditions for sustained capability to act are present in cases where the additional wounding resulting from the special wound ballistic qualities of the head (see companion paper) are minimized. Thus, more than 70% of the guns used fired slow and lightweight bullets: 6.35 mm Browning, .22 rimfire or extremely ineffective projectiles (ancient, inappropriate or selfmade). A centre-fire rifle or a shotgun from close range were never employed in cases involving intracerebral tracts. A coincidence of several lucky circumstances made sustained capability to act possible in two cases of military centrefire rifle bullets passing longitudinally between the frontal lobes without direct contact with brain tissue. Only two large handguns resulting in intracerebral wounding were used: one firing a .38 special bullet, which solely wounded the base of the right temporal lobe and one firing a .45 lead bullet, which seriously injured the left frontal lobe but whose trajectory was limited to the anterior fossa of the skull. Of the trajectories, 28% were outside the neurocranium. At least 70% of the craniocerebral tracts passed above the anterior fossa of the skull, wounding the frontal parts of the brain. Apart from a neurophysiological approach, this preference can be explained by the fact that the base of the anterior cranial fossa and the sella turcica area serve as a bony

Circadian Modulation of Dopamine Levels and Dopaminergic Neuron Development Contributes to Attention Deficiency and Hyperactive Behavior

PubMed Central

Huang, Jian; Zhong, Zhaomin; Wang, Mingyong; Chen, Xifeng; Tan, Yicheng; Zhang, Shuqing; He, Wei; He, Xiong; Huang, Guodong; Lu, Haiping; Wu, Ping; Che, Yi; Yan, Yi-Lin; Postlethwait, John H.; Chen, Wenbiao

2015-01-01

Attention-deficit/hyperactivity disorder (ADHD) is one of the most prevalent psychiatric disorders in children and adults. While ADHD patients often display circadian abnormalities, the underlying mechanisms are unclear. Here we found that the zebrafish mutant for the circadian gene period1b (per1b) displays hyperactive, impulsive-like, and attention deficit-like behaviors and low levels of dopamine, reminiscent of human ADHD patients. We found that the circadian clock directly regulates dopamine-related genes monoamine oxidase and dopamine β hydroxylase, and acts via genes important for the development or maintenance of dopaminergic neurons to regulate their number and organization in the ventral diencephalic posterior tuberculum. We then found that Per1 knock-out mice also display ADHD-like symptoms and reduced levels of dopamine, thereby showing highly conserved roles of the circadian clock in ADHD. Our studies demonstrate that disruption of a circadian clock gene elicits ADHD-like syndrome. The circadian model for attention deficiency and hyperactive behavior sheds light on ADHD pathogenesis and opens avenues for exploring novel targets for diagnosis and therapy for this common psychiatric disorder. PMID:25673850

Central nervous system.

PubMed

Adamson, D Cory; Rasheed, B Ahmed K; McLendon, Roger E; Bigner, Darell D

2010-01-01

Several different types of tumors, benign and malignant, have been identified in the central nervous system (CNS). The prognoses for these tumors are related to several factors, such as the age of the patient and the location and histology of the tumor. In adults, about half of all CNS tumors are malignant, whereas in pediatric patients, more than 75% are malignant. For most benign CNS tumors that require treatment, neurosurgeons can offer curative resections or at least provide significant relief from mass effect. Unfortunately, we still lack effective treatments for most primary and secondary malignant CNS tumors. However, the past decade has witnessed an explosion in the understanding of the early molecular events in malignant primary CNS tumors, and for the first time in history, oncologists are seeing that a plethora of new therapies targeting these molecular events are being tested in clinical trials. There is hope on the horizon for the fight against these deadly tumors. The distribution of CNS tumors by location has remained constant for numerous years. The majority of primary CNS tumors arise in the major cortical lobes. Twenty nine percent of primary CNS tumors arise from the dural meninges that encase the CNS structures. The vast majority of these are meningiomas, of which over 90% are benign. About 10% of primary CNS tumors are found in the sella turcica region, where the pituitary gland resides. Other much less common sites of primary CNS tumors include the pineal region, ventricular system, cerebellum, brain stem, cranial nerves, and spinal cord. The distribution of CNS tumors by histology has seen a slight increase in more malignant tumors over the past decade, possibly due to increased neuroimaging practices or environmental exposures. Arising from glial cells, gliomas represent over 36% of all primary CNS tumors and consist of astrocytomas, oligodendrogliomas, ependymomas, mixed gliomas, and neuroepithelial tumors. The benign meningiomas make up 32

Prospective study of hypothalamo-hypophyseal dysfunction in children and adolescents following traumatic brain injury.

PubMed

Krahulik, David; Aleksijevic, Darina; Smolka, Vratislav; Klaskova, Eva; Venhacova, Petra; Vaverka, Miroslav; Mihal, Vladimir; Zapletalova, Jirina

2017-03-01

Retrospective studies of TBI have found a neuroendocrine dysfunction following traumatic brain injury in 23 to 60% of adults and 15 to 21% of children. Our aims were to determine the prevalence of hypothalamo-hypophyseal dysfunction in children following brain injury, assess its relationship to the type of injury and the course of the acute post-traumatic phase. Body development (growth, pubertal development, and skeletal maturity) were evaluated in 58 patients (21 girls) after a brain injury rated 3 to 12 on the Glasgow Coma Scale (GCS). The patients underwent standard endocrine tests - TSH, fT4, IGF-1, PRL, morning cortisol, FSH, LH, and testosterone in boys and estradiol in girls - in the early post-traumatic period (2 to 14 days; T0) and at 3, 6, and 12 months after the injury (T3, T6, and T12). Dynamic tests were carried out in patients with abnormalities in their clinical examination and/or laboratory results. An MRI was performed on all patients at T12. The median age at the time of injury was 11.3 (0.5 to 18.7) years. Of the 58 patients, 23 had GCS < 8, corresponding to severe brain injury. At T0, diabetes insipidus (DI) was diagnosed in 12 patients, and the syndrome of inappropriate antidiuretic hormone secretion (SIADH) was found in 4 patients. Frequent hormonal changes simulated central hypothyroidism (in 45% of patients) and hypogonadotropic hypogonadism (in 25% of adolescents who were already pubertal at the time of injury > Tanner II). Examination at T3 (n = 58) confirmed a combined pituitary hormone deficiency in two boys and DI in another one. At T6 (n = 49), hormonal dysfunctions were diagnosed in two boys (precocious puberty and growth hormone deficiency). At T12 (n = 39), a new endocrine dysfunction was diagnosed in five patients (growth hormone deficiency in two, hypogonadotropic hypogonadism in two, and in one patient, already diagnosed with a growth hormone deficiency, central hypothyroidism, as well). Brain MRI revealed an empty sella in two

Morphological study of the lingual papillae of the giant panda (Ailuropoda melanoleuca) by scanning electron microscopy

PubMed Central

Pastor, J F; Barbosa, M; De Paz, F J

2008-01-01

Due to the scarcity of giant pandas, there are few descriptions of their morphology and even fewer of their microscopic anatomy and the ultrastructure of their organs. In this study of the complete tongue of an adult male giant panda, we describe the morphology of its lingual surface, the different types of papillae, their characteristics and topographic distribution. It was seen that there are four main types of lingual papillae: filiform, conical, fungiform and vallate. There was no sign of foliate papillae, tuberculum intermolare or sublingua. Papilla distribution was not limited to the dorsum of the tongue, but was also seen on the anterior and ventral surfaces of the tongue. In the anterior third of the midline there is a smooth area with no papillae at all. Morphology of the microgrooves and pores is similar to that observed in other mammals. The papillae share characteristics encountered in Carnivora and herbivorous species of mammals. A narrow bamboo-based diet and specialized manner of eating have together resulted in modification of the tongue of a carnivoran, giving it some characteristics typical of an herbivore. PMID:18254792

Anatomical risk evaluation of iatrogenic injury to the infrapatellar branch of the saphenous nerve during medial meniscus arthroscopic surgery.

PubMed

Koch, Guillaume; Kling, Agathe; Ramamurthy, Nitin; Edalat, Faramarz; Cazzato, Roberto Luigi; Kahn, Jean-Luc; Garnon, Julien; Clavert, Philippe

2017-06-01

To determine the relationship of the medial meniscus with the infrapatellar branches of the saphenous nerve, the primary goal is to define and characterize different risk areas for these nerves during medial meniscus surgery. After dissecting 20 embalmed cadaver knees, we defined 7 readily identifiable anatomical landmarks. For each knee, we recorded 2 morphological criteria and 16 measurements. The most common anatomical course is a main trunk that is 8 mm anterior to the tuberculum adductorium and 60 mm posterior to the midpoint of the medial patellar margin. It has two main infrapatellar branches. The nerve division is 23 mm above the joint line. The path is oblique with an angle of 55.5°. The anterior meniscal landmark is 24 mm from the upper branch and 42.5 mm from the lower branch. The posterior meniscal landmark is 55 mm from the upper branch and 38 mm from the lower branch. We defined a common anatomical course for the saphenous nerve and its infrapatellar branches. Then, three different areas were defined at risk for iatrogenic nerve injuries during medial meniscus.

Strain Accumulation Estimated from Seafloor Crustal Deformation at the Nankai Trough, Japan

NASA Astrophysics Data System (ADS)

Tadokoro, K.; Watanabe, T.; Nagai, S.; Ikuta, R.; Okuda, T.; Kenji, Y.; Sakata, T.

2012-12-01

Our research has developed an observation system for seafloor crustal deformation composed of the kinematic GPS and acoustic ranging techniques [Tadokoro et al., 2006; Ikuta et al., 2008]. We monitored crustal deformation at the Nankai Trough, Japan, where the Philippine Sea Plate subducts beneath the Amurian Plate. The convergence rate is predicted at 60 mm/y in the N59W direction by the Euler vector of REVEL [Sella et al., 2002]. We installed three monitoring sites (named KMN, KMS, and KME) on the seafloor at depths of about 1920-2030 m. The sites KMN and KMS are installed perpendicular to the trough axis with a spacing of 20 km; the site KME is 50 km from KMN and KMS in the direction parallel to the trough axis. The monitoring was started in 2004, 2005, and 2008 at KMS, KMN, and KME, respectively. The numbers of measurements are 16, 20, and 5 times at KMN, KMS, and KME, respectively. We obtained 3-7 years averaged horizontal site velocities within ITRF2000 adopting a robust estimation method with Tukey's biweight function to the time series of site position measured until the end of 2011. Substituting the synthetic rigid block motions of the Amurian Plate from the velocities within ITRF2000, we obtained the following site velocities with respect to the Amurian Plate [Tadokoro et al., 2012]: KMN 41±4 mm/y, N77±7W KMS 43±5 mm/y, N80±6W KME 42±5 mm/y, N80±7W In contrast, the on-land GPS horizontal velocities along the coast is 23-33 mm/y toward N74-80W. The present observational results show: (1) the velocity vectors are all the same length and direction, which indicates no internal deformation in this region; (2) the back-slip model predicts that the plate interface beneath the region is uniformly locked with coupling ratios of 60-80 %, indicating strain accumulation that will be released during the anticipated mega-thrust Tonankai earthquake; and (3) the directions of site velocities differ from that of convergence vector by 20 degrees, which is affected by

Active faulting induced by the slip partitioning in the Lesser Antilles arc

NASA Astrophysics Data System (ADS)

Leclerc, Frédérique; Feuillet, Nathalie

2010-05-01

ée. Some faults, located between Guadeloupe and Montserrat have throws up to thousand meters. Between St Lucia and Martinique, the St Lucia channel is crosscut by several normal faults with scarps up to 100m-high. These faults extend onshore and cut the southern shore of Martinique. Given their length (~20km), they could produce magnitude 6 or more earthquakes in the most tourist towns of the island (St Anne, St Lucie). Recent coseismic offsets could be identified along most faults in the chirp profiles. Turbidite deposits recognized in the Küllenberg cores could be related to damaging earthquakes. High resolution SAR imagery (25 cm) reveals several coseismic scarps in Les Saintes channel along the faults that ruptured in 2004. References: Feuillet, N., I. Manighetti, and P. Tapponnier, Arc parallel extension and localization of volcanic complexes in guadeloupe, lesser antilles, Journal of Geophysical Research, 107, 2002. Feuillet, N., P. Tapponnier, I. Manighetti, B. Villemant, and G. C. P. King, Differential uplift and tilt of pleistocene reef platforms and quaternary slip Lopez, A.M., S. Stein, T. Dixon, G. Sella, E. Calais, P. Jansma, J. Weber, and P. La Femina, Is there a northern lesser antilles forearc block ?, Geophysical Research Letters, 33, 2006.

Afferent Connectivity of the Zebrafish Habenulae

PubMed Central

Turner, Katherine J.; Hawkins, Thomas A.; Yáñez, Julián; Anadón, Ramón; Wilson, Stephen W.; Folgueira, Mónica

2016-01-01

The habenulae are bilateral nuclei located in the dorsal diencephalon that are conserved across vertebrates. Here we describe the main afferents to the habenulae in larval and adult zebrafish. We observe afferents from the subpallium, nucleus rostrolateralis, posterior tuberculum, posterior hypothalamic lobe, median raphe; we also see asymmetric afferents from olfactory bulb to the right habenula, and from the parapineal to the left habenula. In addition, we find afferents from a ventrolateral telencephalic nucleus that neurochemical and hodological data identify as the ventral entopeduncular nucleus (vENT), confirming and extending observations of Amo et al. (2014). Fate map and marker studies suggest that vENT originates from the diencephalic prethalamic eminence and extends into the lateral telencephalon from 48 to 120 hour post-fertilization (hpf). No afferents to the habenula were observed from the dorsal entopeduncular nucleus (dENT). Consequently, we confirm that the vENT (and not the dENT) should be considered as the entopeduncular nucleus “proper” in zebrafish. Furthermore, comparison with data in other vertebrates suggests that the vENT is a conserved basal ganglia nucleus, being homologous to the entopeduncular nucleus of mammals (internal segment of the globus pallidus of primates) by both embryonic origin and projections, as previously suggested by Amo et al. (2014). PMID:27199671

Localization of BDNF expression in the developing brain of zebrafish

PubMed Central

De Felice, E; Porreca, I; Alleva, E; De Girolamo, P; Ambrosino, C; Ciriaco, E; Germanà, A; Sordino, P

2014-01-01

The brain-derived neurotrophic factor (BDNF) gene is expressed in differentiating and post-mitotic neurons of the zebrafish embryo, where it has been implicated in Huntington's disease. Little is known, however, about the full complement of neuronal cell types that express BDNF in this important vertebrate model. Here, we further explored the transcriptional profiles during the first week of development using real-time quantitative polymerase chain reaction (RT-qPCR) and whole-mount in situ hybridization (WISH). RT-qPCR results revealed a high level of maternal contribution followed by a steady increase of zygotic transcription, consistent with the notion of a prominent role of BDNF in neuronal maturation and maintenance. Based on WISH, we demonstrate for the first time that BDNF expression in the developing brain of zebrafish is structure specific. Anatomical criteria and co-staining with genetic markers (shh, pax2a, emx1, krox20, lhx2b and lhx9) visualized major topological domains of BDNF-positive cells in the pallium, hypothalamus, posterior tuberculum and optic tectum. Moreover, the relative timing of BDNF transcription in the eye and tectum may illustrate a mechanism for coordinated development of the retinotectal system. Taken together, our results are compatible with a local delivery and early role of BDNF in the developing brain of zebrafish, adding basic knowledge to the study of neurotrophin functions in neural development and disease. PMID:24588510

Case of Subarachnoid Hemorrhage Caused by Tuberculous Aneurysm.

PubMed

Liu, Wei; Li, Chuanfeng; Liu, Xianming; Xu, Zhiming; Kong, Lu

2018-02-01

Subarachnoid hemorrhage caused by rupture of tuberculosis associated aneurysm is a rare complication. In this paper, we report a case of intracranial tuberculum with adjacent intracerebral inflammatory aneurysm which caused subarachnoid hemorrhage and brain abscess formation. A 28-year-old man presented with sudden onset of severe headache. He was diagnosed with pulmonary tuberculosis 8 years ago, and had been treated with antituberculosis medications for 6 months. Head computed tomography showed a small hematoma in the left sylvian fissure with subarachnoid hemorrhage. Cerebral digital subtraction angiography was performed and no aneurysm was found. He was discharged after nonsurgical treatment. Three weeks later, he came back to our department with complaint of aphasia. Magnetic resonance images showed a cystic lesion with mass effect. During operation, we encounter the brain abscess and were surprised to find a middle cerebral artery aneurysm while dissecting. The abscess was totally removed, and the aneurysm was secured by clipping. The aneurysm was suspected of being inflammatory in nature and associated with the patient's tuberculosis. Tuberculosis in the central nervous system may present as tuberculoma and tuberculous meningitis. Vasculitis secondary to tuberculous meningitis can cause infarcts, and, rarely, aneurysm formation. This case report illustrated a rare case of intracranial infectious aneurysm related to tuberculosis and complicated by hemorrhage and brain abscess. Copyright © 2017 Elsevier Inc. All rights reserved.

Rib kinematics during lung ventilation in the American alligator (Alligator mississippiensis): an XROMM analysis

PubMed Central

Moritz, Sabine; Codd, Jonathan; Sellers, William I.

2017-01-01

ABSTRACT The current hypothesis regarding the mechanics of breathing in crocodylians is that the double-headed ribs, with both a capitulum and tuberculum, rotate about a constrained axis passing through the two articulations; moreover, this axis shifts in the caudal thoracic ribs, as the vertebral parapophysis moves from the centrum to the transverse process. Additionally, the ventral ribcage in crocodylians is thought to possess additional degrees of freedom through mobile intermediate ribs. In this study, X-ray reconstruction of moving morphology (XROMM) was used to quantify rib rotation during breathing in American alligators. Whilst costovertebral joint anatomy predicted overall patterns of motion across the ribcage (decreased bucket handle motion and increased calliper motion), there were significant deviations: anatomical axes overestimated pump handle motion and, generally, ribs in vivo rotate about all three body axes more equally than predicted. The intermediate ribs are mobile, with a high degree of rotation measured about the dorsal intracostal joints, especially in the more caudal ribs. Motion of the sternal ribs became increasingly complex caudally, owing to a combination of the movements of the vertebral and intermediate segments. As the crocodylian ribcage is sometimes used as a model for the ancestral archosaur, these results have important implications for how rib motion is reconstructed in fossil taxa, and illustrate the difficulties in reconstructing rib movement based on osteology alone. PMID:28855323

Localization of BDNF expression in the developing brain of zebrafish.

PubMed

De Felice, E; Porreca, I; Alleva, E; De Girolamo, P; Ambrosino, C; Ciriaco, E; Germanà, A; Sordino, P

2014-05-01

The brain-derived neurotrophic factor (BDNF) gene is expressed in differentiating and post-mitotic neurons of the zebrafish embryo, where it has been implicated in Huntington's disease. Little is known, however, about the full complement of neuronal cell types that express BDNF in this important vertebrate model. Here, we further explored the transcriptional profiles during the first week of development using real-time quantitative polymerase chain reaction (RT-qPCR) and whole-mount in situ hybridization (WISH). RT-qPCR results revealed a high level of maternal contribution followed by a steady increase of zygotic transcription, consistent with the notion of a prominent role of BDNF in neuronal maturation and maintenance. Based on WISH, we demonstrate for the first time that BDNF expression in the developing brain of zebrafish is structure specific. Anatomical criteria and co-staining with genetic markers (shh, pax2a, emx1, krox20, lhx2b and lhx9) visualized major topological domains of BDNF-positive cells in the pallium, hypothalamus, posterior tuberculum and optic tectum. Moreover, the relative timing of BDNF transcription in the eye and tectum may illustrate a mechanism for coordinated development of the retinotectal system. Taken together, our results are compatible with a local delivery and early role of BDNF in the developing brain of zebrafish, adding basic knowledge to the study of neurotrophin functions in neural development and disease. © 2014 Anatomical Society.

Morphological variation, phylogenetic relationships, and geographic distribution of the Baenidae (Testudines), based on new specimens from the Uinta Formation (Uinta Basin), Utah (USA)

PubMed Central

Hutchison, J. Howard; Townsend, K. E. Beth; Adrian, Brent; Jager, Daniel

2017-01-01

We described newly discovered baenid specimens from the Uintan North American Land Mammal Age (NALMA), in the Uinta Formation, Uinta Basin, Utah. These specimens include a partial skull and several previously undescribed postcranial elements of Baena arenosa, and numerous well-preserved shells of B. arenosa and Chisternon undatum. Baenids from the Uintan NALMA (46.5–40 Ma) are critical in that they provide valuable insight into the morphology and evolution of the diverse and speciose baenid family near the end of its extensive radiation, just prior to the disappearance of this clade from the fossil record. These Uintan specimens greatly increase the known variation in these late-surviving taxa and indicate that several characters thought to define these species should be reassessed. The partial cranium of B. arenosa, including portions of the basicranium, neurocranium, face, and lower jaw, was recently recovered from Uinta B sediments. While its morphology is consistent with known specimens of B. arenosa, we observed several distinct differences: a crescent-shaped condylus occipitalis that is concave dorsally, tuberculum basioccipitale that flare out laterally, and a distinct frontal-nasal suture. The current sample of plastral and carapacial morphology considerably expands the documented variation in the hypodigms of B. arenosa and C. undatum. Novel shell characters observed include sigmoidal extragular-humeral sulci, and small, subtriangular gular scutes. Subadult specimens reveal ontogenetic processes in both taxa, and demonstrate that diagnostic morphological differences between them were present from an early developmental age. PMID:28686718

Morphological variation, phylogenetic relationships, and geographic distribution of the Baenidae (Testudines), based on new specimens from the Uinta Formation (Uinta Basin), Utah (USA).

PubMed

Smith, Heather F; Hutchison, J Howard; Townsend, K E Beth; Adrian, Brent; Jager, Daniel

2017-01-01

We described newly discovered baenid specimens from the Uintan North American Land Mammal Age (NALMA), in the Uinta Formation, Uinta Basin, Utah. These specimens include a partial skull and several previously undescribed postcranial elements of Baena arenosa, and numerous well-preserved shells of B. arenosa and Chisternon undatum. Baenids from the Uintan NALMA (46.5-40 Ma) are critical in that they provide valuable insight into the morphology and evolution of the diverse and speciose baenid family near the end of its extensive radiation, just prior to the disappearance of this clade from the fossil record. These Uintan specimens greatly increase the known variation in these late-surviving taxa and indicate that several characters thought to define these species should be reassessed. The partial cranium of B. arenosa, including portions of the basicranium, neurocranium, face, and lower jaw, was recently recovered from Uinta B sediments. While its morphology is consistent with known specimens of B. arenosa, we observed several distinct differences: a crescent-shaped condylus occipitalis that is concave dorsally, tuberculum basioccipitale that flare out laterally, and a distinct frontal-nasal suture. The current sample of plastral and carapacial morphology considerably expands the documented variation in the hypodigms of B. arenosa and C. undatum. Novel shell characters observed include sigmoidal extragular-humeral sulci, and small, subtriangular gular scutes. Subadult specimens reveal ontogenetic processes in both taxa, and demonstrate that diagnostic morphological differences between them were present from an early developmental age.

Commentary on "Comparison of abiraterone acetate versus ketoconazole in patients with metastatic castration resistant prostate cancer refractory to docetaxel." Peer A, Gottfried M, Sinibaldi V, Carducci MA, Eisenberger MA, Sella A, Leibowitz-Amit R, Berger R, Keizman D, Department of Oncology, Rambam Medical Center, Haifa, Israel.: Prostate 2014 Apr;74(4):433-40; doi:10.1002/pros.22765. [Epub 2013 Dec 11].

PubMed

Trump, Donald

2016-05-01

Abiraterone, a potent CYP 17 inhibitor, is standard treatment in docetaxel refractory, metastatic castrate resistant prostate cancer (mCRPC). However, in countries where abiraterone has not been approved yet, or for patients who cannot afford it, ketoconazole is used as an alternative CYP 17 inhibitor. Although preclinical data suggests that ketoconazole is a less potent inhibitor of CYP 17, there are limited clinical data comparing both agents. We aimed to compare the clinical effectiveness of abiraterone versus ketoconazole in docetaxel refractory mCRPC. Records from mCRPC patients treated with ketoconazole (international multicenter database, n = 162) were reviewed retrospectively. Twenty-six patients treated post docetaxel were individually matched by clinicopathologic factors to patients treated with abiraterone (national multicenter database, n = 140). We compared the PSA response, biochemical and radiological progression free survival (PFS), and overall survival (OS) between the groups. PFS and OS were determined by Cox regression. The groups were matched by Gleason score, pre-treatment disease extent, ECOG PS, pre-treatment risk category (Keizman, Oncologist 2012). Furthermore, they were balanced regarding other known confounding risk factors. In the groups of abiraterone versus ketoconazole, PSA response was 46% versus 19% (OR 4.3, P = 0.04), median biochemical PFS 7 versus 2 months (HR 1.54, P = 0.02), median radiological PFS 8 versus 2.5 months (HR 1.8, P = 0.043), median OS 19 versus 11 months (HR 0.53, P = 0.79), and treatment interruption d/t severe adverse events 8% (n = 2) versus 31% (n = 8) (OR 0.6, P = 0.023). In docetaxel refractory mCRPC, the outcome of abiraterone treatment may be superior to ketoconazole. Copyright © 2016 Elsevier Inc. All rights reserved.

Bolstering the Nasoseptal Flap Using Sphenoid Sinus Fat Packing: A Technical Case Report.

PubMed

Abou-Al-Shaar, Hussam; Zaidi, Hasan A; Cote, David J; Laws, Edward R

2017-03-01

Resection of extensive skull base lesions often necessitates relatively large dural openings and arachnoid, resulting in skull base defects with the potential for a postoperative cerebrospinal fluid leak. A nasoseptal flap (NSF) is a vascularized graft that has greatly diminished the incidence of cerebrospinal fluid leak. Annealing of flaps against the ventral skull base can be tenuous within the first few days after surgery. We report the use of sphenoid sinus fat packing as a buttress to support the nasoseptal flap during skull base reconstruction. A 37-year-old man presented with pan-hypopituitarism, bitemporal hemianopsia, and imaging consistent with a craniopharyngioma. He underwent an endoscopic endonasal approach with resection of the planum and tuberculum sphenoidale for resection of this mass. An NSF was harvested, and a combination of suprasellar fat packing, tensor fasciae lata graft, and Porex plate along with the flap were used to reconstruct the skull base. Postoperatively, he precipitously experienced copious rhinorrhea necessitating surgical re-exploration. A redundant segment of the NSF had retracted into the sphenoid sinus, and was no longer supported against the ventral skull base. We repositioned the NSF and used sphenoid sinus fat packing to help support the graft against the ventral skull base. A postoperative computed tomographic scan demonstrated a clear delineation between the vascularized graft and the fat packing, confirming proper positioning of the flap. Sphenoid sinus fat packing can be an important technical adjunct in bolstering the nasoseptal flap against the ventral skull base in the tenuous early perioperative period. Copyright © 2016 Elsevier Inc. All rights reserved.

Catecholaminergic connectivity to the inner ear, central auditory and vocal motor circuitry in the plainfin midshipman fish, Porichthys notatus

PubMed Central

Forlano, Paul M.; Kim, Spencer D.; Krzyminska, Zuzanna M.; Sisneros, Joseph A.

2014-01-01

Although the neuroanatomical distribution of catecholaminergic (CA) neurons has been well documented across all vertebrate classes, few studies have examined CA connectivity to physiologically and anatomically identified neural circuitry that controls behavior. The goal of this study was to characterize CA distribution in the brain and inner ear of the plainfin midshipman fish (Porichthys notatus) with particular emphasis on their relationship with anatomically labeled circuitry that both produces and encodes social acoustic signals in this species. Neurobiotin labeling of the main auditory endorgan, the saccule, combined with tyrosine hydroxylase immunofluorescence (TH-ir) revealed a strong CA innervation of both the peripheral and central auditory system. Diencephalic TH-ir neurons in the periventricular posterior tuberculum, known to be dopaminergic, send ascending projections to the ventral telencephalon and prominent descending projections to vocal-acoustic integration sites, notably the hindbrain octavolateralis efferent nucleus, as well as onto the base of hair cells in the saccule via nerve VIII. Neurobiotin backfills of the vocal nerve in combination with TH-ir revealed CA terminals on all components of the vocal pattern generator which appears to largely originate from local TH-ir neurons but may include diencephalic projections as well. This study provides strong evidence for catecholamines as important neuromodulators of both auditory and vocal circuitry and acoustic-driven social behavior in midshipman fish. This first demonstration of TH-ir terminals in the main endorgan of hearing in a non-mammalian vertebrate suggests a conserved and important anatomical and functional role for dopamine in normal audition. PMID:24715479

Intracranial meningiomas managed at Memfys hospital for neurosurgery in Enugu, Nigeria.

PubMed

Mezue, Wilfred C; Ohaegbulam, Samuel C; Ndubuisi, Chika C; Chikani, Mark C; Achebe, David S

2012-09-01

The epidemiology and pathology of meningioma in Nigeria are still evolving and little has been published about this tumor in Nigeria, especially in the southeast region. The aim of this paper is to compare the characteristics of intracranial meningioma managed in our center with the pattern reported in the literature worldwide. Retrospective analysis of prospectively recorded data of patients managed for intracranial meningioma between January 2002 and December 2010 at a Private neurosurgery Hospital in Enugu, Nigeria. We excluded patients whose histology results were inconclusive. Meningiomas constituted 23.8% of all intracranial tumors seen in the period. The male to female ratio was 1:1.1. The peak age range for males and females were in the fifth and sixth decades, respectively. The most common location is the Olfactory groove in 26.5% of patients followed by convexity in 23.5%. Presentation varied with anatomical location of tumor. Patients with olfactory groove meningioma (OGM) mostly presented late with personality changes and evidence of raised ICP. Tuberculum sellar and sphenoid region tumors presented earlier with visual impairment with or without hormonal abnormalities. Seizures occurred in 30.9% of all patients and in 45% of those with convexity meningiomas. Only 57.4% of the patients were managed surgically and there was no gender difference in this group. WHO grade1 tumors were the most common histological types occurring in 84.6%. One patient had atypical meningioma and two had anaplastic tumors. The pattern of meningioma in our area may have geographical differences in location and histology. Childhood meningioma was rare.

Attention and Motivated Response to Simulated Male Advertisement Call Activates Forebrain Dopaminergic and Social Decision-Making Network Nuclei in Female Midshipman Fish.

PubMed

Forlano, Paul M; Licorish, Roshney R; Ghahramani, Zachary N; Timothy, Miky; Ferrari, Melissa; Palmer, William C; Sisneros, Joseph A

2017-10-01

Little is known regarding the coordination of audition with decision-making and subsequent motor responses that initiate social behavior including mate localization during courtship. Using the midshipman fish model, we tested the hypothesis that the time spent by females attending and responding to the advertisement call is correlated with the activation of a specific subset of catecholaminergic (CA) and social decision-making network (SDM) nuclei underlying auditory- driven sexual motivation. In addition, we quantified the relationship of neural activation between CA and SDM nuclei in all responders with the goal of providing a map of functional connectivity of the circuitry underlying a motivated state responsive to acoustic cues during mate localization. In order to make a baseline qualitative comparison of this functional brain map to unmotivated females, we made a similar correlative comparison of brain activation in females who were unresponsive to the advertisement call playback. Our results support an important role for dopaminergic neurons in the periventricular posterior tuberculum and ventral thalamus, putative A11 and A13 tetrapod homologues, respectively, as well as the posterior parvocellular preoptic area and dorsomedial telencephalon, (laterobasal amygdala homologue) in auditory attention and appetitive sexual behavior in fishes. These findings may also offer insights into the function of these highly conserved nuclei in the context of auditory-driven reproductive social behavior across vertebrates. © The Author 2017. Published by Oxford University Press on behalf of the Society for Integrative and Comparative Biology. All rights reserved. For permissions please email: journals.permissions@oup.com.

Prospective study of vocal fold function after loss of the neuromonitoring signal in thyroid surgery: The International Neural Monitoring Study Group's POLT study.

PubMed

Schneider, Rick; Randolph, Gregory; Dionigi, Gianlorenzo; Barczyński, Marcin; Chiang, Feng-Yu; Triponez, Frédéric; Vamvakidis, Kyriakos; Brauckhoff, Katrin; Musholt, Thomas J; Almquist, Martin; Innaro, Nadia; Jimenez-Garcia, Antonio; Kraimps, Jean-Louis; Miyauchi, Akira; Wojtczak, Beata; Donatini, Gianluca; Lombardi, Davide; Müller, Uwe; Pezzullo, Luciano; Ratia, Tomas; Van Slycke, Sam; Nguyen Thanh, Phuong; Lorenz, Kerstin; Sekulla, Carsten; Machens, Andreas; Dralle, Henning

2016-05-01

Intraoperative neuromonitoring identifies recurrent laryngeal nerve (RLN) injury and gives prognostic information regarding postoperative glottic function. Loss of the neuromonitoring signal (LOS) signifies segmental type 1 or global type 2 RLN injury. This study aimed at identifying risk factors for RLN injury and determining vocal fold (VF) function initially and 6 months after definitive LOS. Prospective study encompassing 21 hospitals from 13 countries. Included in this study were patients with persistent intraoperative LOS. At first postoperative laryngoscopy, early VF palsy was present in 94 of all 115 patients with LOS (81.7%): in 53 of 56 patients (94.6%) with type 1 injury and 41 of 59 patients (69.5%) with type 2 injury. In LOS type 1, women outnumbered men >5-fold. Traction produced LOS type 1 in 38 of 56 patients (67.9%) and LOS type 2 in 54 of 59 patients (91.5%). Course of the RLN posterior and/or anterior to the inferior thyroid artery, extralaryngeal branching, or tuberculum of Zuckerkandl did not increase VF palsy rates. Permanent VF palsy rates were also lower (P = .661) after LOS type 2 than after LOS type 1: 6.8% (four of 59 patients) versus 10.7% (six of 56 patients). Intraoperative administration of steroids did not diminish postoperative VF palsy rates. LOS type 1 entails more severe nerve damage than LOS type 2, affecting women disproportionately. Both LOS types, being primarily associated with traction injury, are unaffected by variant neck anatomy in expert hands and unresponsive to steroids. 2b Laryngoscope, 126:1260-1266, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Right atrial and right ventricular ultrasound-guided biopsy technique in standing horses.

PubMed

Decloedt, A; de Clercq, D; Ven, S; van der Vekens, N; Chiers, K; van Loon, G

2016-05-01

Endomyocardial biopsies could be a valuable tool in equine cardiology for diagnosing myocardial disease, which is probably underdiagnosed in horses because of lack of specific diagnostic measures and limitations of currently available diagnostic methods. To describe a technique for serial right atrial and right ventricular endomyocardial biopsy in standing horses using a percutaneous approach through the jugular vein. Prospective study. Biopsy was performed in 10 healthy standing horses sedated with detomidine, under continuous electrocardiography monitoring. A 10 cm (n = 6), 45 cm (n = 1) or 98 cm (n = 3) introducer sheath was inserted into the right jugular vein. Under echocardiographic guidance, a biopsy forceps was introduced through the sheath into the right ventricle and right atrium and endomyocardial biopsies were acquired. In all horses, 3 right ventricular biopsies were obtained from the right ventricular apex and 3 right atrial biopsies were obtained from the dorsal right atrial wall near the tuberculum intervenosum. The presence of myocardial tissue was confirmed by histology. All horses showed atrial and ventricular premature depolarisations associated with acquisition of the biopsies. In 9 horses, the arrhythmia disappeared after retraction of the forceps and introducer sheath. In one horse, ventricular premature depolarisations disappeared only after 8 h. No other complications were observed. Endomyocardial biopsy of the right atrium and right ventricle could be performed in standing horses using a percutaneous approach through the jugular vein and was not associated with complications other than temporary arrhythmias. This technique may be useful for research purposes or as a diagnostic tool, although further research is needed to establish the safety of the technique in clinical patients with myocardial disease. © 2015 EVJ Ltd.

BDNF Expression in Larval and Adult Zebrafish Brain: Distribution and Cell Identification

PubMed Central

Cacialli, Pietro; Gueguen, Marie-Madeleine; Coumailleau, Pascal; D’Angelo, Livia; Kah, Olivier; Lucini, Carla; Pellegrini, Elisabeth

2016-01-01

Brain-derived neurotrophic factor (BDNF), a member of the neurotrophin family, has emerged as an active mediator in many essential functions in the central nervous system of mammals. BDNF plays significant roles in neurogenesis, neuronal maturation and/or synaptic plasticity and is involved in cognitive functions such as learning and memory. Despite the vast literature present in mammals, studies devoted to BDNF in the brain of other animal models are scarse. Zebrafish is a teleost fish widely known for developmental genetic studies and is emerging as model for translational neuroscience research. In addition, its brain shows many sites of adult neurogenesis allowing higher regenerative properties after traumatic injuries. To add further knowledge on neurotrophic factors in vertebrate brain models, we decided to determine the distribution of bdnf mRNAs in the larval and adult zebrafish brain and to characterize the phenotype of cells expressing bdnf mRNAs by means of double staining studies. Our results showed that bdnf mRNAs were widely expressed in the brain of 7 days old larvae and throughout the whole brain of mature female and male zebrafish. In adults, bdnf mRNAs were mainly observed in the dorsal telencephalon, preoptic area, dorsal thalamus, posterior tuberculum, hypothalamus, synencephalon, optic tectum and medulla oblongata. By combining immunohistochemistry with in situ hybridization, we showed that bdnf mRNAs were never expressed by radial glial cells or proliferating cells. By contrast, bdnf transcripts were expressed in cells with neuronal phenotype in all brain regions investigated. Our results provide the first demonstration that the brain of zebrafish expresses bdnf mRNAs in neurons and open new fields of research on the role of the BDNF factor in brain mechanisms in normal and brain repairs situations. PMID:27336917

Tinea atypica: report of nine cases.

PubMed

Zisova, Liliya Georgieva; Dobrev, Hristo Petrov; Tchernev, Georgi; Semkova, Kristina; Aliman, Anastasia Atanasova; Chorleva, Kristina Ivanova; Chapanova, Antonina Teneva; Vutova, Nina Ivanova; Wollina, Uwe

2013-12-01

Fungal infections of the skin are a common condition, usually easy to diagnose and treat. When the infection is clinically mimicking another cutaneous disorder or when the clinical presentation is modified by the use of inappropriate treatment, it is referred to as tinea atypica or tinea incognito.We report a series of nine cases of patients with tinea atypica, imitating and diagnosed initially as different skin diseases. Two patients were defined as pyoderma in the facial and pubic regions (caused respectively by Trichophyton mentagrophytes var. mentagrophytes and Microsporum canis) and one as herpes zoster ophthalmicus (caused by Trichophyton rubrum). Six additional patients were initially misdiagnosed: (1) Plaque-like formation of the skin misdiagnosed as an impetiginized eczema (with isolated agent Trichophyton verrucosum). (2) A rare form of skin infection of the hand caused by T. rubrum, imitating clinically cutaneous infection with tuberculum mulgentium. (3) Rosacea-like dermatitis with an isolated agent Fusarium. (4) A patient with the typical clinical symptoms of seborrheic dermatitis of the face (and with isolated T. rubrum as a causative agent). (5) Another patient presented with a widespread folliculitis by Trichophyton mentagrophytes. (6) In a patient with bullous pemphigoid and immunosuppression pemphigoid-like eruptions were caused by Malassezia pachydermatis and T. rubrum. The diagnosis in the presented cases was based on direct microscopic examination with KOH and a culture on Sabouraud agar.After the diagnosis of tinea, treatment with topical and systemic antifungal agents was administrated, followed by complete clinical remissions in all cases.The clinical manifestations of tinea atypica can mimic a large number of other dermatoses, which often leads to misdiagnosing, and as a consequence--to serious difficulties in the management of clinical symptoms and in offering appropriate therapy.

Exposure to advertisement calls of reproductive competitors activates vocal-acoustic and catecholaminergic neurons in the plainfin midshipman fish, Porichthys notatus.

PubMed

Petersen, Christopher L; Timothy, Miky; Kim, D Spencer; Bhandiwad, Ashwin A; Mohr, Robert A; Sisneros, Joseph A; Forlano, Paul M

2013-01-01

While the neural circuitry and physiology of the auditory system is well studied among vertebrates, far less is known about how the auditory system interacts with other neural substrates to mediate behavioral responses to social acoustic signals. One species that has been the subject of intensive neuroethological investigation with regard to the production and perception of social acoustic signals is the plainfin midshipman fish, Porichthys notatus, in part because acoustic communication is essential to their reproductive behavior. Nesting male midshipman vocally court females by producing a long duration advertisement call. Females localize males by their advertisement call, spawn and deposit all their eggs in their mate's nest. As multiple courting males establish nests in close proximity to one another, the perception of another male's call may modulate individual calling behavior in competition for females. We tested the hypothesis that nesting males exposed to advertisement calls of other males would show elevated neural activity in auditory and vocal-acoustic brain centers as well as differential activation of catecholaminergic neurons compared to males exposed only to ambient noise. Experimental brains were then double labeled by immunofluorescence (-ir) for tyrosine hydroxylase (TH), an enzyme necessary for catecholamine synthesis, and cFos, an immediate-early gene product used as a marker for neural activation. Males exposed to other advertisement calls showed a significantly greater percentage of TH-ir cells colocalized with cFos-ir in the noradrenergic locus coeruleus and the dopaminergic periventricular posterior tuberculum, as well as increased numbers of cFos-ir neurons in several levels of the auditory and vocal-acoustic pathway. Increased activation of catecholaminergic neurons may serve to coordinate appropriate behavioral responses to male competitors. Additionally, these results implicate a role for specific catecholaminergic neuronal groups in

Exposure to Advertisement Calls of Reproductive Competitors Activates Vocal-Acoustic and Catecholaminergic Neurons in the Plainfin Midshipman Fish, Porichthys notatus

PubMed Central

Petersen, Christopher L.; Timothy, Miky; Kim, D. Spencer; Bhandiwad, Ashwin A.; Mohr, Robert A.; Sisneros, Joseph A.; Forlano, Paul M.

2013-01-01

While the neural circuitry and physiology of the auditory system is well studied among vertebrates, far less is known about how the auditory system interacts with other neural substrates to mediate behavioral responses to social acoustic signals. One species that has been the subject of intensive neuroethological investigation with regard to the production and perception of social acoustic signals is the plainfin midshipman fish, Porichthys notatus, in part because acoustic communication is essential to their reproductive behavior. Nesting male midshipman vocally court females by producing a long duration advertisement call. Females localize males by their advertisement call, spawn and deposit all their eggs in their mate’s nest. As multiple courting males establish nests in close proximity to one another, the perception of another male’s call may modulate individual calling behavior in competition for females. We tested the hypothesis that nesting males exposed to advertisement calls of other males would show elevated neural activity in auditory and vocal-acoustic brain centers as well as differential activation of catecholaminergic neurons compared to males exposed only to ambient noise. Experimental brains were then double labeled by immunofluorescence (-ir) for tyrosine hydroxylase (TH), an enzyme necessary for catecholamine synthesis, and cFos, an immediate-early gene product used as a marker for neural activation. Males exposed to other advertisement calls showed a significantly greater percentage of TH-ir cells colocalized with cFos-ir in the noradrenergic locus coeruleus and the dopaminergic periventricular posterior tuberculum, as well as increased numbers of cFos-ir neurons in several levels of the auditory and vocal-acoustic pathway. Increased activation of catecholaminergic neurons may serve to coordinate appropriate behavioral responses to male competitors. Additionally, these results implicate a role for specific catecholaminergic neuronal groups

Early Pleistocene hominin deciduous teeth from the Homo antecessor Gran Dolina-TD6 bearing level (Sierra de Atapuerca, Spain).

PubMed

Bermúdez de Castro, José María; Martinón-Torres, María; Martín-Francés, Laura; Martínez de Pinillos, Marina; Modesto-Mata, Mario; García-Campos, Cecilia; Wu, Xiujie; Xing, Song; Liu, Wu

2017-07-01

During the last 13 years, the late Early Pleistocene Gran Dolina-TD6-2 level (Sierra de Atapuerca, northern Spain) has yielded an additional sample of 26 dental specimens attributed to Homo antecessor. In this report, we present a descriptive and comparative study of the six deciduous teeth. We provide external and internal morphological descriptions following classical terminology, as well as the mesiodistal and buccolingual measurements of the teeth. The internal morphology was described by means of micro-CT technique. The TD6 deciduous teeth preserve primitive features regarding the Homo clade, such as the presence of styles in lower and upper canines and developed anterior and posterior foveae in the dm 2 . However, other features related to the complexity of the crown morphology (e.g., cingulum) are not present in this sample. Furthermore, the great reduction of the talonid of the dm 1 s is also noteworthy. Despite the limited comparative evidence, the presence of a remarkably well-developed tuberculum molare in the dm 1 and dm 1 s from TD6 can be also considered a derived feature in the genus Homo. The TD6 hominins exhibit dental dimensions similar to those of other Pleistocene hominins. The dm 1 s are buccolingually elongated and the buccolingual diameter of ATD6-93 is the largest recorded so far in the Homo fossil record. This study expands the list of plesiomorphic features of H. antecessor, and provides some information on the evolutionary status of this species. However, the identification of some advanced traits evinces a step towards the derived morphology of European Pleistocene teeth. The study of the deciduous dentition confirms the mosaic pattern of H. antecessor morphology revealed in previous studies of this hominin sample. © 2017 Wiley Periodicals, Inc.

Apomorphine and piribedil in rats: biochemical and pharmacologic studies.

PubMed

Butterworth, R F; Poignant, J C; Barbeau, A

1975-01-01

We studied the biochemical and pharmacologic modes of action of piribedil and apomorphine in the rat. Although both drugs have many points in common, they are also different in many of their manifestations. Apomorphine causes high-intensity, short-duration stereotyped behavior; it is distributed within the brain in uneven fashion, the striatum being the area of lowest concentration as measured by fluorometry. Direct stereotactic injection within the dopaminergic mesolimbic system, and particularly the tuberculum olfactorium, produced constant intense responses. All effects of apomorphine can be blocked by pimozide, but propanolol, a beta blocker, only reduces aggression and ferocity, leaving stereotyped behaviors intact. Finally, L-5-HTP tends to reduce aggression, ferocity, and to a lesser extent stereotypy; MIF or piribedil, as well as reserpine, potentiates the stereotyped behaviors induced by apomorphine, whereas L-DOPA usually decreases them. Piribedil, on the other hand, causes low-intensity, long-duration stereotyped behavior. It is distributed within the brain almost uniformly. Most effects of piribedil can be blocked by pimozide, but propanolol blocks only aggression and ferocity, leaving stereotyped behaviors intact. On the other hand, clonidine, an alpha-receptor agonist, blocks stereotyped behaviors induced by piribedil but markedly increases aggression, ferocity, and motor activity. L-5-HTP and L-DOPA have little effect on piribedil-induced manifestations. Reserpine decreases piribedil stereotypy. The main metabolite of piribedil, S 584, had no clear-cut pharmacologic action in our hands at the dosage used. It is concluded that both apomorphine and piribedil produce stereotyped behavior by modifying the physiologic balance between mesolimbic and nigrostriatal dopaminergic systems. The other actions of apomorphine and piribedil upon aggression, ferocity, and motor activity are not always in parallel and depend probably on the fact that piribedil is less

Ancient views on the hypothalamic-pituitary-thyroid axis: an historical and epistemological perspective.

PubMed

Toni, R

2000-10-01

The modern views on the anatomical and physiological interactions between the hypothalamus, pituitary and thyroid gland have emerged only in the last fifty years, although their historical roots may be found in a number of ancient and still not widely known ideas and observations. The regulation of energy body stores and temperature by the hypothalamic-pituitary-thyroid axis, for example, is a classical case of "fixitè du mileu interieur" in the sense originally suggested by Claude Bernard in the late 1800s, i.e. a homeostatic mechanism, but already 2100 year previously Aristotle had stated that the brain was necessary for the maintenance of body integrity by regulating food intake and behavior in relation to body temperature, the latter primarily determined by the heat of the blood. Five hundred years later Galen of Pergamon reported fundamental discoveries in the anatomy of the third ventricle region, including the location of the pituitary gland inside the sella turcica embodied in a vascular network, the rete mirabilis, and observed nerves adjoining the "soft flesh" in the neck, i.e. the thyroid gland. He first proposed that the energy of the body (the vital spirit) was carried through the arteries at the level of the rete mirabilis, where it was transformed into nerve impulse (the animal spirit), eventually transferred by the nerves to the periphery of the body, "glands" included, raising implicitly the possibility for a nervous influence over the thyroid activity. The Galenic model remained virtually unaltered up to the beginning of the 14th century, when the mediaeval anatomist Mondino de' Liuzzi put forth the idea that the thyroid gland interacted with the heat of the blood present in the internal carotid arteries due to their anatomical relation with the thyroid. This interaction enriched the vital spirit, i.e. the energy of the body, prior to its transformation into animal spirit, i.e. to nerve impulse directed to the periphery of the body. In addition

Retrograde lag screw placement in anterior acetabular column with regard to the anterior pelvic plane and midsagittal plane -- virtual mapping of 260 three-dimensional hemipelvises for quantitative anatomic analysis.

PubMed

Ochs, Bjoern Gunnar; Stuby, Fabian Maria; Ateschrang, Atesch; Stoeckle, Ulrich; Gonser, Christoph Emanuel

2014-10-01

Percutaneous screw placement can be used for minimally invasive treatment of none or minimally displaced fractures of the anterior column. The complex pelvic geometry can pose a major challenge even for experienced surgeons. The present study examined the preformed bone stock of the anterior column in 260 hemipelvises (130 male and 130 female). Screws were virtually implanted using iPlan(®) CMF (BrainLAB AG, Feldkirchen, Germany); the maximal implant length and the maximal implant diameter were assessed. The study showed, that 6.5mm can generally be used in men; in women however individual planning is essential in regard to the maximal implant diameter since we found that in 15.4% of women, screws with a diameter less than 6.5mm were necessary. The virtual analysis of the preformed bone stock corridor of the anterior column showed two constrictions of crucial clinical importance. These can be found after 18% and 55% (men) respectively 16% and 55% (women) measured from the entry point along the axis of the implant. The entry point of the retrograde anterior column screw in our collective was located lateral of tuberculum pubicum at the level of the superior-medial margin of foramen obturatum. In female patients, the entry point was located significantly more lateral of symphysis and closer to the cranial margin of ramus superior ossis pubis. The mean angle between the screw trajectory and the anterior pelvic plane in sagittal section was 31.6 ± 5.5°, the mean angle between the screw trajectory and the midsagittal plane in axial section was 55.9 ± 4.6° and the mean angle between the screw trajectory and the midsagittal plane in coronal section was 42.1 ± 3.9° with no significant deviation between both sexes. The individual angles formed by the screw trajectory and the anterior pelvic and midsagittal plane are independent from anthropometric parameters sex, age, body length and weight. Therefore, they can be used for orientation in lag screw placement keeping

Commentary on "Cabozantinib in patients with advanced prostate cancer: results of a phase II randomized discontinuation trial." Smith DC, Smith MR, Sweeney C, Elfiky AA, Logothetis C, Corn PG, Vogelzang NJ, Small EJ, Harzstark AL, Gordon MS, Vaishampayan UN, Haas NB, Spira AI, Lara PN Jr, Lin CC, Srinivas S, Sella A, SchoffskiSchöffski P, Scheffold C, Weitzman AL, Hussain M, University of Michigan, Ann Arbor, MI. J Clin Oncol 2013;31(4):412-9. doi: 10.1200/JCO.2012.45.0494. Epub 2012 Nov 19.

PubMed

Trump, Donald L

2013-11-01

Cabozantinib (XL184) is an orally bioavailable tyrosine kinase inhibitor with activity against MET and vascular endothelial growth factor receptor 2. We evaluated the activity of cabozantinib in patients with castration-resistant prostate cancer (CRPC) in a phase II randomized discontinuation trial with an expansion cohort. Patients received 100mg of cabozantinib daily. Those with stable disease per RECIST at 12 weeks were randomly assigned to cabozantinib or placebo. Primary end points were objective response rate at 12 weeks and progression-free survival (PFS) after random assignment. One hundred seventy-one men with CRPC were enrolled. Random assignment was halted early based on the observed activity of cabozantinib. Seventy-two percent of patients had regression in soft tissue lesions, whereas 68% of evaluable patients had improvement on bone scan, including complete resolution in 12%. The objective response rate at 12 weeks was 5%, with stable disease in 75% of patients. Thirty-one patients with stable disease at week 12 were randomly assigned. Median PFS was 23.9 weeks (95% CI, 10.7 to 62.4 weeks) with cabozantinib and 5.9 weeks (95% CI, 5.4 to 6.6 weeks) with placebo (hazard ratio, 0.12; P<.001). Serum total alkaline phosphatase and plasma cross-linked C-terminal telopeptide of type I collagen were reduced by ≥ 50% in 57% of evaluable patients. On retrospective review, bone pain improved in 67% of evaluable patients, with a decrease in narcotic use in 56%. The most common grade 3 adverse events were fatigue (16%), hypertension (12%), and hand-foot syndrome (8%). Cabozantinib has clinical activity in men with CRPC, including reduction of soft tissue lesions, improvement in PFS, resolution of bone scans, and reductions in bone turnover markers, pain, and narcotic use. Copyright © 2013 Elsevier Inc. All rights reserved.

The neurologist facing pain in dementia.

PubMed

Álvaro González, Luis Carlos

2015-01-01

Ageing, a common background in dementia, is usually associated with painful disorders. Nevertheless, the use of analgesics is limited due to poor communication. On the other hand, dementia lesions are placed in the nociceptive pathways. For this reason, the painful experience becomes different and distinctive for every lesional type. The lateral nociceptive pathway (lateral thalamic nuclei and primary parietal cortex), which is in charge of the primary pain perception, is preserved in dementia. Thereafter, the shear painful perception, including pain intensity and threshold, remains unmodified. Distinctly, the medial pain pathways are affected by dementia lesions. In this pathway are included: the intralaminar thalamic nuclei, the pons (locus ceruleus:LC), the mesencephalon (periaacueductal grey substance: PGS), the hypothalamus (paraventricular nuclei, mamilary tuberculum) and different areas of the parietal (primary, secondary, operculum), temporal (amigdala, hypoccampus) and frontal (anterior cingular: ACC). As a consequence, the features of pain executed by these areas will be compromised: the cognitive assessment, the mood and emotion inherent to pain, the pain memory or the autonomic responses are modified in dementia. Specifically, in Alzheimer's disease (AD) there is a reduction in the anticipatory and avoidance responses and also a flattening of the autonomic responses. These are essentially secondary to the degenerative changes in the medial temporal (pain memory) and ACC (cognitive and mood aspects) areas. In vascular dementias, there is a cortico-subcortical deafferentation secondary to the white matter lesions. The consequence is the presence of hyperpathy and hyperalgesia. In the frontotemporal dementias, there is a reduction in pain expressivity. It is linked to the lesions in the orbitofrontal and anterior temporal areas, which are responsible of the emotional aspects of pain. In Parkinson's disease, painful conditions are a common characteristic