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Sample records for arnold chiari malformation

  1. Acute Porphyria in a Patient with Arnold Chiari Malformation

    PubMed Central

    Shen, Jianbin; O’Keefe, Kevin; Webb, Lisa B.; DeGirolamo, Angela

    2015-01-01

    Patient: Female, 33 Final Diagnosis: Acute porphyria Symptoms: Abdominal pain • alternating bowel habits Medication: Metronidazole • bactrim • oxybutynin Clinical Procedure: EMG • porhyria workup Specialty: Neurology Objective: Rare disease Background: Acute porphyria and Arnold Chiari malformation are both uncommon genetic disorders without known association. The insidious onset, non-specific clinical manifestations, and precipitating factors often cause diagnosis of acute porphyria to be missed, particularly in patients with comorbidities. Case Report: A women with Arnold Chiari malformation type II who was treated with oxybutynin and antibiotics, including Bactrim for neurogenic bladder and recurrent urinary tract infection, presented with non-specific abdominal pain, constipation, and diarrhea. After receiving Flagyl for C. difficile colitis, the patient developed psychosis, ascending paralysis, and metabolic derangements. She underwent extensive neurological workup due to her congenital neurological abnormalities, most of which were unremarkable. As a differential diagnosis of Guillain Barré syndrome, acute porphyria was then considered and ultimately proved to be the diagnosis. After hematin administration and intense rehabilitation, the patient slowly recovered from the full-blown acute porphyria attack. Conclusions: This case report, for the first time, documents acute porphyria attack as a result of a sequential combination of 3 common medications. This is the first case report of the concomitant presence of both acute porphyria and Arnold Chiari malformation, 2 genetic disorders with unclear association. PMID:25697467

  2. Arnold Chiari Malformation With Sponastrime (Spondylar and Nasal Changes, With Striations of the Metaphyses) Dysplasia

    PubMed Central

    Jeong, Je Hoon; Lee, A Leum; Cho, Sung Yoon; Jin, Dong Kyu; Im, Soo-Bin

    2016-01-01

    Abstract SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain. We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated. PMID:27149441

  3. The Arnold-Chiari Malformation and Its Implications for Individuals with Spina Bifida and Hydrocephalus.

    ERIC Educational Resources Information Center

    Mittler, Joel E.

    1986-01-01

    The Arnold-Chiari malformation is present in most infants born with myelomeningocele (a form of spina bifida) and hydrocephalus. The syndrome is responsible for structural abnormalities in the brain, and peripheral nervous system. Etiology, symptoms, impact on central nervous system structures, surgical treatment, and implications for education…

  4. Arnold Chiari Malformation With Sponastrime (Spondylar and Nasal Changes, With Striations of the Metaphyses) Dysplasia: A Case Report.

    PubMed

    Jeong, Je Hoon; Lee, A Leum; Cho, Sung Yoon; Jin, Dong Kyu; Im, Soo-Bin

    2016-05-01

    SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated. PMID:27149441

  5. Nuclear magnetic resonance (NMR) imaging of Arnold-Chiari type I malformation with hydromyelia

    SciTech Connect

    DeLaPaz, R.L.; Brady, T.J.; Buonanno, F.S.; New, P.F.; Kistler, J.P.; McGinnis, B.D.; Pykett, I.L.; Taveras, J.M.

    1983-02-01

    Saturation recovery nuclear magnetic resonance (NMR) images and metrizamide computed tomography (CT) scans were obtained in an adult patient with a clinical history suggestive of syringomyelia. Both NMR and CT studies showed low lying cerebellar tonsils. The CT study demonstrated central cavitation of the spinal cord from the midthoracic to midcervical levels but could not exclude an intramedullary soft tissue mass at the cervico-medullary junction. The NMR images in transverse, coronal, and sagittal planes demonstrated extension of an enlarged central spinal cord cerebrospinal fluid space to the cervico-medullary junction. This was felt to be strong evidence for exclusion of an intramedullary soft tissue mass and in favor of a diagnosis of Arnold-Chiari Type I malformation with hydromyelia. The noninvasive nature of spinal cord and cervico-medullary junction evaluation with NMR is emphasized.

  6. Chiari Malformation

    MedlinePlus

    Chiari malformations (CMs) are structural defects in the cerebellum. The cerebellum is the part of the brain that controls balance. With CM, brain tissue extends into the spinal canal. It can happen when part of the skull is too ...

  7. Chiari Malformation

    MedlinePlus

    ... Ones & When? Smart School Lunches Emmy-Nominated Video "Cerebral Palsy: Shannon's Story" 5 Things to Know About Zika & ... spine that can lead to nerve damage or paralysis. Chiari malformations also can happen after brain or spinal surgery. Doctors haven't identified a ...

  8. Viewing condition dependence of the gaze-evoked nystagmus in Arnold Chiari type 1 malformation.

    PubMed

    Ghasia, Fatema F; Gulati, Deepak; Westbrook, Edward L; Shaikh, Aasef G

    2014-04-15

    Saccadic eye movements rapidly shift gaze to the target of interest. Once the eyes reach a given target, the brainstem ocular motor integrator utilizes feedback from various sources to assure steady gaze. One of such sources is cerebellum whose lesion can impair neural integration leading to gaze-evoked nystagmus. The gaze evoked nystagmus is characterized by drifts moving the eyes away from the target and a null position where the drifts are absent. The extent of impairment in the neural integration for two opposite eccentricities might determine the location of the null position. Eye in the orbit position might also determine the location of the null. We report this phenomenon in a patient with Arnold Chiari type 1 malformation who had intermittent esotropia and horizontal gaze-evoked nystagmus with a shift in the null position. During binocular viewing, the null was shifted to the right. During monocular viewing, when the eye under cover drifted nasally (secondary to the esotropia), the null of the gaze-evoked nystagmus reorganized toward the center. We speculate that the output of the neural integrator is altered from the bilateral conflicting eye in the orbit position secondary to the strabismus. This could possibly explain the reorganization of the location of the null position. PMID:24559612

  9. [The basilar impression and the Arnold-Chiari malformation. Techno-surgical considerations apropos of 13 cases].

    PubMed

    Gonçalves da Silva, J A; Gonçalves da Silva, C E; de Farias Brito, J C; Rodrigues de Sousa, M; Gonçalves da Silva, J B

    1978-03-01

    The surgical technic for decompression of the posterior fossa in cases of basilar impression and malformation of Arnold-Chiari mainly based in (1) endotracheal intubation without any flexion of the head, (2) position of the head without anterior flexion during the surgery, (3) plastic of the posterior fossa with dura-mater of cadaver conserved in glicerina, was emploied in 13 patients. The plastic was made to create space at the craniocervical joint in order to avoid cerebrospinal fluid fistula and to restore the integrity of the dura. Infection was not observed. PMID:637743

  10. Unusual Ventilatory Response to Exercise in Patient with Arnold-Chiari Type 1 Malformation after Posterior Fossa Decompression

    PubMed Central

    Smith, Keely; Gomez-Rubio, Ana M.; Harris, Tomika S.; Brooks, Lauren E.

    2016-01-01

    We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship. No new pathology of the brainstem was discovered by MRI or neurological evaluation to explain this phenomenon. The patient was placed on continuous noninvasive open ventilation (NIOV) during the day and CPAP at night for a period of 6 months. His pCO2 level decreased to normal limits and his symptoms improved; specifically, he experienced less headaches and fatigue during exercise. In this report, we describe the abnormal response to exercise that patients with AC-Type 1 could potentially experience, even after decompression, characterized by the impairment of ventilator response to hypercapnia during exertion, reflecting a complete loss of chemical influence on breathing with no evidence of abnormality in the corticospinal pathway. PMID:27418995

  11. [Otoneurologic symptoms associated with Arnold-Chiari syndrome type I].

    PubMed

    Urban, Irena; Namysłowski, Grzegorz; Morawski, Krzysztof; Wojtacha, Maciej

    2004-01-01

    This study presents two cases of Arnold-Chiari malformation type I. In a 26-year old man, right side deafness and left side sensorineural hearing loss at high frequencies occurred. Another patient, a 48-year old man also complained of sensorineural hearing loss and dizziness, that appeared a year and half ago. In addition, this patient had episodes of vertigo with nausea and vomit that occurred about one year before main symptoms. In both patients ENT examinations were performed as well as an audiological diagnostic battery including tonal- and impedance-audiometry, auditory brainstem responses, distortion product otoacoustic emissions and electroencephalography. Magnetic resonance imaging (MRI) showed pathological changes in the cerebello-pontine angle region that allowed diagnosing Arnold-Chiari malformation in both cases. Additionally, angio-MRI performed in patient with right side deafness revealed cochleovestibular nerve compression syndrome on the same side. Presumably, both anomalies occurring simultaneously in this patient might be responsible for deafness in the right ear, instead of mild or moderate hearing loss and tinnitus usually expected according to the literature. The paper presented two cases of Arnold-Chiari malformation with co-existing cochleovestibular nerve compression syndrome in one case. The importance of both audiological diagnostic battery and MRI in diagnostic procedures of this malformation has been demonstrated. PMID:15307473

  12. Chiari-like Malformation.

    PubMed

    Loughin, Catherine A

    2016-03-01

    Chiari-like malformation is a condition of the craniocervical junction in which there is a mismatch of the structures of the caudal cranial fossa causing the cerebellum to herniate into the foramen magnum. This herniation can lead to fluid buildup in the spinal cord, also known as syringomyelia. Pain is the most common clinical sign followed by scratching. Other neurologic signs noted are facial nerve deficits, seizures, vestibular syndrome, ataxia, menace deficit, proprioceptive deficits, head tremor, temporal muscle atrophy, and multifocal central nervous system signs. MRI is the diagnostic of choice, but computed tomography can also be used. PMID:26631589

  13. Chiari Malformation: Diagnosis

    MedlinePlus

    ... Chiari, but can be a result of life-style changes due to Chiari The broad array of ... is measuring CSF Flow? • Bridging The Patient- Doctor Communication Gap (Part 1 of 3) • Bridging The Patient- ...

  14. Chiari Malformation: Treatment

    MedlinePlus

    ... endorse any doctors, procedures, or products. © 2012 C&S Patient Education Foundation, ® Treatment Options Patients evaluated for Chiari-like ... when there is a significant syrinx © 2012 C&S Patient Education Foundation, ® This presentation is for informational purposes, consult ...

  15. [Brain stem dysfunction in Arnold-Chiari II syndrome].

    PubMed

    Holschneider, A M; Bliesener, J A; Abel, M

    1990-04-01

    Among 76 patients suffering from myelomeningocele treated during 1978 to 1987 there were 12 children with brain stem signs as a sequel to Arnold-Chiari II syndrome. In 2 of these patients only stridor was seen, in 4 stridor with attacks of apnoea, in 2 attacks of apnoea with dysphagia, and in 4 children stridor, attacks of apnoea and dysphagia. Hence, it will be necessary to modify the classification given by Charney et al (4) in respect of brain stem patterns of signs according to three grades, since the signs of stridor, apnoea and dysphagia can be combined with each other in different ways. The prognosis is infaust if all 3 signs and hence grade III of brain stem lesions are present. On the whole, 6 out of 12 patients with brain stem signs died. For this reason, a possible Arnold-Chiari malformation should always be considered if stridor is observed, and, if necessary, early decompression treatment by means of a shunt revision should be performed. PMID:2360371

  16. Chiari Malformations and Syringohydromyelia in Children.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2016-04-01

    Chiari malformations are a heterogeneous group of disorders with distinct clinical anatomical features all of which involve the hindbrain. Our understanding of Chiari malformations increased tremendously over the past decades, and progress in neuroimaging was instrumental for that. Conventional and advanced neuroimaging of the brain and spine play a key role in the workup of children with suspected Chiari malformations. In addition, neuroimaging studies in Chiari malformations may guide the management, serve as a predictor of outcome, and shed light on the pathogenesis. PMID:27063663

  17. Visual Fixation in Chiari Type II Malformation

    PubMed Central

    Salman, Michael S.; Sharpe, James A.; Lillakas, Linda; Dennis, Maureen; Steinbach, Martin J.

    2011-01-01

    Chiari type II malformation is a congenital deformity of the hindbrain. Square wave jerks are horizontal involuntary saccades that interrupt fixation. Cerebellar disorders may be associated with frequent square wave jerks or saccadic oscillations such as ocular flutter. The effects of Chiari type II malformation on visual fixation are unknown. We recorded eye movements using an eye tracker in 21 participants with Chiari type II malformation, aged 8 to 19 years while they fixated a target for 1 minute. Thirty-eight age-matched healthy participants served as controls. Square wave jerks’ parameters were similar in the 2 groups. Saccadic oscillations were not seen. Chiari type II malformation is not associated with pathological square wave jerks or abnormal saccadic oscillations. The congenital nature of this deformity may permit compensation that preserves stable visual fixation. Alternatively, the deformity of Chiari type II malformation may spare parts of the cerebellum that usually cause fixation instability when damaged. PMID:19182152

  18. Revision surgery for Chiari malformation decompression.

    PubMed

    Mazzola, Catherine A; Fried, Arno H

    2003-09-15

    Chiari malformations comprise four different hindbrain anomalies originally described by Hans Chiari, a professor of pathology at the German University in Prague. There are four basic Chiari malformations. The reasons for revision of Chiari malformation decompression may be for conservative or inadequate initial decompression or the development of postoperative complications. Another reason involves cases of both hindbrain herniation and syringomyelia in patients who have undergone adequate posterior fossa decompression without resolution of symptoms, signs, or radiological appearance of their syrinx cavity. Additionally, symptom recurrence has been reported in association with various types of dural grafts. Reoperation or revision surgery for patients with Chiari malformations is common and may not be due to technical error or inadequate decompression. The types of revision surgeries, their indications, and initial presentations will be reviewed. PMID:15347221

  19. Advanced Imaging of Chiari 1 Malformations.

    PubMed

    Fakhri, Akbar; Shah, Manish N; Goyal, Manu S

    2015-10-01

    Type I Chiari malformations are congenital deformities involving cerebellar tonsillar herniation downward through the foramen magnum. Structurally, greater than 5 mm of tonsillar descent in adults and more than 6 mm in children is consistent with type I Chiari malformations. However, the radiographic severity of the tonsillar descent does not always correlate well with the clinical symptomatology. Advanced imaging can help clinically correlate imaging to symptoms. Specifically, cerebrospinal fluid (CSF) flow abnormalities are seen in patients with type I Chiari malformation. Advanced MRI involving cardiac-gated and phase-contrast MRI affords a view of such CSF flow abnormalities. PMID:26408061

  20. Neuro-ophthalmology of type 1 Chiari malformation

    PubMed Central

    Shaikh, Aasef G.; Ghasia, Fatema F.

    2016-01-01

    Chiari malformation is a congenital deformity leading to herniation of cerebellar tonsils. Headache is a typical symptom of this condition, but patients with Chiari malformation often present with double vision and vertigo. Examination of eye movements in such patients often reveals nystagmus and strabismus. Eye movement deficits in the context of typical symptomatic presentation are critical clinical markers for the diagnosis of Chiari malformation. We will review eye movement deficits that seen in patients with type 1 Chiari malformation. We will then discuss the underlying pathophysiology and therapeutic options for such deficits. PMID:26843890

  1. Neurogenic dysphagia resulting from Chiari malformations.

    PubMed

    Pollack, I F; Pang, D; Kocoshis, S; Putnam, P

    1992-05-01

    Between 1980 and 1989, 15 of 46 patients (11 children, 4 adults) who underwent suboccipital craniectomy and cervical laminectomy for symptomatic Chiari malformations presented with manifestations of neurogenic dysphagia. Each of these patients had normal swallowing function before the development of dysphagic symptoms. Dysphagia was progressive in all 15 and, in most cases, preceded the onset of other severe brain stem signs. The rate of symptom progression varied depending on the age of the patient. Whereas the six infants (all Chiari II) deteriorated rapidly after the onset of initial symptoms, the five older children (two Chiari I, three Chiari II) and four adults (all Chiari I) showed a more gradual deterioration. In 11 patients with severe dysphagia, barium video esophagograms, pharyngoesophageal motility studies, continuous esophageal pH monitoring, and appropriate scintigraphic studies were useful in defining the scope of the swallowing impairment and determining whether perioperative nasogastric or gastrostomy feedings, gastric fundoplication, and/or tracheostomy were needed to maintain adequate nutrition and avoid aspiration. These patients all had widespread dysfunction of the swallowing mechanism, with a combination of diffuse pharyngoesophageal dysmotility, cricopharyngeal achalasia, nasal regurgitation, tracheal aspiration, and gastroesophageal reflux. The pathophysiology of these swallowing impairments and their relation to the hindbrain malformation is discussed. Postoperative outcome with regard to swallowing function correlated with the severity of preoperative symptoms. The four patients with mild dysphagia showed rapid improvement in swallowing function after surgery. Seven patients with more severe impairment but without other signs of severe brain stem compromise, such as central apnea or complete bilateral vocal cord paralysis, also improved, albeit more slowly. In contrast, the outcome in the four patients who developed other signs of severe

  2. Chiari I malformations: clinical and radiologic reappraisal.

    PubMed

    Elster, A D; Chen, M Y

    1992-05-01

    Clinical findings and magnetic resonance (MR) images in 68 patients with Chiari I malformations were retrospectively analyzed to identify those radiologic features that correlated best with clinical symptoms. A statistically significant (P = .03) female predominance of the malformation was observed, with a female: male ratio of approximately 3:2. Associated skeletal anomalies were seen in 24% of patients. Syringomyelia was detected in 40% of patients, most commonly between the C-4 and C-6 levels. Of the 25 patients who presented with spinal symptoms, 23 (92%) proved to have a syrinx at MR imaging. When the syrinx extended into the medulla (n = 3), however, brain stem symptoms predominated. Patients with objective brain stem or cerebellar signs had the largest mean tonsillar herniations. Patients with tonsillar herniations greater than 12 mm were invariably symptomatic, but approximately 30% of patients with tonsils herniating 5-10 mm below the foramen magnum were asymptomatic at MR imaging. "Incidental" Chiari I malformations are thus much more common than previously recognized, and careful clinical assessment remains the cornerstone for proper diagnosis and management. PMID:1561334

  3. Chiari I malformation: classification and management.

    PubMed

    Bindal, A K; Dunsker, S B; Tew, J M

    1995-12-01

    Considerable debate exists about which surgical options are best for the management of the Chiari I malformation. We present a classification system for the Chiari I malformation that improves the prediction of outcome and guides the selection of surgical treatment. Twenty-seven adult patients with Chiari I malformations were grouped on the basis of the presence of signs and symptoms of brain stem compression, syringomyelia, or both. To objectively assess changes in clinical status postoperatively, a scale was developed to quantify the signs and symptoms, which were statistically analyzed by the paired t test. Five patients were asymptomatic and underwent no treatment. Ten patients had symptoms of brain stem compression without associated syringomyelia and underwent brain stem decompression, including anterior decompression in one patient with basilar invagination; all 10 patients had significant improvement at 4-year mean follow-up visits (P < 0.0001). In 12 patients with syringomyelia, 5 were symptomatic from syringomyelia only, 6 were symptomatic from both brain stem compression and syringomyelia, and 1 was symptomatic from brain stem compression only. The median length of symptoms before presentation was longer for patients with syringomyelia than for patients without (2 yr versus 9 mo; P < 0.025); the mean follow-up was 4 years. Surgical procedures included posterior brain stem decompression in 12 patients, plugging of the obex in 7, and placement of syringosubarachnoid shunts in 7, a syringopleural shunt in 1, and fourth ventricular stents in 2. In the 12 patients with syringomyelia, symptoms from brain stem compression dramatically improved with surgical decompression (P < 0.025), whereas symptoms from syringomyelia less dramatically improved or stabilized. The slight improvement or stabilization of syrinx symptoms represents a successful result, given the documented progressive nature of syringomyelia in this group. We conclude that surgical treatment for

  4. Sports participation with Chiari I malformation.

    PubMed

    Strahle, Jennifer; Geh, Ndi; Selzer, Béla J; Bower, Regina; Himedan, Mai; Strahle, MaryKathryn; Wetjen, Nicholas M; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

    2016-04-01

    OBJECT There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I. METHODS A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months. RESULTS Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries. CONCLUSIONS No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe. PMID:26636249

  5. Chiari malformations: An important cause of pediatric aspiration.

    PubMed

    Fuller, Jennifer C; Sinha, Sumi; Caruso, Paul A; Hersh, Cheryl J; Butler, William E; Krishnamoorthy, Kalpathy S; Hartnick, Christopher J

    2016-09-01

    Chronic aspiration poses a major health risk to the pediatric population. We describe four cases in which work up for chronic aspiration with a brain MRI revealed a Chiari I malformation, a poorly described etiology of pediatric aspiration. All patients had at least one non-specific neurologic symptom but had swallow studies more characteristic of an anatomic than a neurologic etiology. Patients were referred to neurosurgery and underwent posterior fossa decompression with symptom improvement. A high index of suspicion for Chiari malformation should be maintained when the standard work up for aspiration is non-diagnostic, particularly when non-specific neurologic symptoms are present. PMID:27497399

  6. Conquer Chiari

    MedlinePlus

    ... Article: Understanding How Chiari Symptoms Affect Daily Activity Chiari Journeys NEW! A Battle to Conquer the Unknown ... shares her journey with Chiari Malformation Participate in Chiari Research NEW! Do you have Chiari? Do you ...

  7. Ventral Decompression in Chiari Malformation, Basilar Invagination, and Related Disorders.

    PubMed

    Ridder, Thomas; Anderson, Richard C E; Hankinson, Todd C

    2015-10-01

    Ventral brainstem compression is an uncommon clinical diagnosis seen by pediatric neurosurgeons and associated with Chiari malformation, type I. Presenting clinical symptoms often include headaches, lower cranial neuropathies, myelopathy, central sleep apnea, ataxia, and nystagmus. When ventral decompression is required, both open and endoscopic transoral/transnasal approaches are highly effective. PMID:26408067

  8. Osteopetrosis and Chiari type I malformation: a rare association

    PubMed Central

    Ekici, Mehmet Ali; Cıkla, Ulaş; Bauer, Andrew; Başkaya, Mustafa K.

    2015-01-01

    Osteopetrosis (OP) is hereditary X-linked, autosomal recessive (ARO), or autosomal dominant (ADO) skeletal disease. ARO has two subtypes, which are infantile malignant and intermediate type. ARO and X-linked OP have poor clinical outcome. ADO is called adult benign type because of the normal life expectancy, which has type I and type II. Here, the authors present an ADO patient with Chiari type I. Concomitant ADO with Chiari type I malformation is an extremely rare condition. Literature research yielded only one case report to date. PMID:26503583

  9. Autism Spectrum Disorder and Chiari 1 Malformation Co-occurring in a Child.

    PubMed

    Osuagwu, Ferdnand C; Amalraj, Benedict; Noveloso, Bernard D; Aikoye, Salisu A; Bradley, Ronald

    2016-04-01

    Very few studies have shown associations between autism spectrum disorder, attention deficit hyperactivity disorder and Chiari 1 malformation. Here, we report an 10-year-old male that presented after having seizures with a history of Chiari 1 malformation, autism spectrum disorder and ADHD with moderate mental retardation and speech delay. This case highlights the fact that autism spectrum disorder as biologically based neurodevelopmental disorder with altered brain growth may be associated with Chiari 1 malformation and ADHD. PMID:27050897

  10. Headache and Chiari I Malformation in Children and Adolescents.

    PubMed

    Victorio, M Cristina; Khoury, Chaouki K

    2016-02-01

    Headache is a common problem in children and adolescents. Its recurrent and disabling nature may lead to use of neuroimaging to exclude secondary causes of headache such as Chiari I malformation (CM I). CM I has a variety of presentation with headache being the most common symptom. CM I can be asymptomatic and is also often found incidentally in neuroimaging done for conditions other than headache. This article reviews the spectrum of headache in patients with CM I. PMID:27017020

  11. Chiari malformation type I: a new MRI classification.

    PubMed

    Amer, T A; el-Shmam, O M

    1997-01-01

    Thirty patients with Chiari I malformation were examined by MRI over 2-year period. All patients underwent MRI scan before and after surgical decompression of the posterior fossa. Images of the craniocervical junction confirmed tonsillar herniation in all cases and allowed the definition of two anatomically distinct types of Chiari malformation. Twenty-one of the 30 patients (70%) had concomitant syringomyelia and were classified as type A, while the remaining 9 patients (30%) had evidence of frank herniation of the cerebellar tonsils below the foramen magnum without evidence of syringomyelia and were labeled type B. Type A patients had a predominant central cord symptomatology; type B patients exhibited signs and symptoms of brain stem or cerebellar compression. The concomitant cord cavitary lesions (syringomyelia) were noncommunicating (isolated syrinxes), which were separated from the fourth ventricle by a syrinx-free segment of normal spinal cord. Holocord hydromyelic cavities were seen in 8 out of 21 patients with syringomyelia, isolated cervical cavities were seen in 4 patients, while combined cervical and thoracic cavities were seen in 9 patients. Kinking of the medullocervical junction and brain stem was seen in 20 out of 30 patients (67%). MRI has proved to be an excellent, noninvasive means of studying of the craniocervical anatomy; it has allowed a classification of Chiari malformation based on objective anatomic criteria with prognostic and clinical relevance. PMID:9223040

  12. Chiari type I malformation, syncope, headache, hypoglycemia and hepatic steatosis in an 8-year old girl: a causal association?

    PubMed Central

    Tarani, Luigi; Del Balzo, Francesca; Costantino, Francesco; Properzi, Enrico; D’Eufemia, Patrizia; Liberati, Natascia; Spalice, Alberto

    2010-01-01

    Chiari type I malformation (CMI) is a congenital hindbrain anomaly characterized by downward displacement of the cerebellar tonsils through the foramen magnum. Chiari type I malformation often presents with a complex clinical picture and can be sporadic or linked to a variety of genetic conditions. We report on a girl in whom Chiari type I malformation was associated with hypoglycemia, headache, vertigo, syncope and hepatic steatosis. We hypothesize that these symptoms are primarily a consequence of Chiari type I malformation. PMID:21589844

  13. Isolated sleep apnea due to Chiari type I malformation and syringomyelia.

    PubMed

    Shiihara, T; Shimizu, Y; Mitsui, T; Saitoh, E; Sato, S

    1995-10-01

    We report an 11-year-old girl with Chiari type I malformation and syringomyelia, who experienced isolated sleep apnea without other neurologic problems. Monitoring with oximetry and movement of thoracic and abdominal walls indicated mixed-type sleep apnea. Chiari type I malformation should be differentiated from other disorders causing sleep apnea. PMID:8554669

  14. NREM Sleep Parasomnia Associated with Chiari I Malformation

    PubMed Central

    Daftary, Ameet S.; Walker, James M.; Farney, Robert J.

    2011-01-01

    Parasomnias are common sleep disorders in children, and most cases resolve naturally by adolescence.1 They represent arousal disorders beginning in NREM sleep and are generally non-concerning in children. The diagnosis can usually be made by clinical assessment, and testing with polysomnography is not routinely indicated.2 However, in certain cases with atypical features, polysomnography and more extensive neurologic evaluation are medically indicated. Citation: Daftary AS; Walker JM; Farney RJ. NREM Sleep Parasomnia associated with Chiari I malformation. J Clin Sleep Med 2011;7(5):526-529. PMID:22003350

  15. Type I Chiari malformation presenting with laryngomalacia and dysphagia.

    PubMed

    Liu, Christopher; Ulualp, Seckin O

    2015-08-01

    We describe clinical features of an infant with laryngomalacia and dysphagia caused by type I Chiari malformation (CM-I). A 12-month-old child presented with a 6 month history of progressive stridor, dysphagia, and gastroesophageal reflux. Examination of the airway and swallowing function indicated mild laryngomalacia and aspiration with all consistencies. Magnetic resonance imaging of the brain indicated CM-I. Symptoms were resolved after posterior fossa decompression. CM-I, typically diagnosed later in life, should be considered in the differential diagnosis of laryngomalacia and dysphagia. High clinical suspicion and thorough search for abnormalities ensure early diagnosis and proper management of children with neurologic variant laryngomalacia. PMID:26031456

  16. Isolated unilateral trismus as a presentation of Chiari malformation: case report.

    PubMed

    Feinberg, Michelle; Babington, Parker; Sood, Shawn; Keating, Robert

    2016-05-01

    The authors present a case of Chiari malformation manifesting as isolated trismus, describe the typical symptoms associated with Chiari malformation, and discuss the potential anatomical causes for this unique presentation. A 3-year-old boy presented with inability to open his jaw for 6 weeks with associated significant weight loss. The results of medical and radiological evaluation were negative except for Type I Chiari malformation with cerebellar tonsils 12 mm below the level of the foramen magnum. The patient underwent Chiari decompression surgery. Postoperatively, his ability to open his mouth was significantly improved, allowing resumption of a regular diet. Postoperative MRI revealed almost complete resolution of the syringobulbia. To the best of the authors' knowledge, this is the first reported case of isolated trismus from Chiari malformation with syringobulbia. PMID:26722762

  17. Treatment of Syringomyelia due to Chiari Type I Malformation with Syringo-Subarachnoid-Peritoneal Shunt

    PubMed Central

    Akakın, Akın; Yılmaz, Baran; Kılıç, Türker

    2015-01-01

    Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system. PMID:25932303

  18. Long-term evolution of a laughing headache associated with Chiari type 1 malformation.

    PubMed

    Morales-Asín, F; Mauri, J A; Iñiguez, C; Larrode, M P; Mostacero, E

    1998-01-01

    We describe an acute-onset, sharp, short-lasting (few seconds) headache at the vertex, that first occurred 10 years ago in a 44-year-old woman. Attacks were triggered by vigorous laughing. A magnetic resonance imaging study showed a Chiari type 1 malformation. To our knowledge, laugh-induced headache with long-term evolution in association with Chiari type 1 malformation has not been previously reported. PMID:15613173

  19. Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism

    PubMed Central

    Pang, Chang Hwan; Kim, Chi Heon; Chung, Chun Kee

    2015-01-01

    The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery. PMID:26512271

  20. Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism.

    PubMed

    Pang, Chang Hwan; Lee, Soo Eon; Kim, Chi Heon; Chung, Chun Kee

    2015-09-01

    The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery. PMID:26512271

  1. Identical twin sisters with Rubinstein-Taybi syndrome associated with Chiari malformations and syrinx.

    PubMed

    Parsley, Lea; Bellus, Gary; Handler, Michael; Tsai, Anne Chun-Hui

    2011-11-01

    Chiari malformations are multifactorial and heterogeneous entities, characterized by abnormalities in the posterior fossa. They have been identified in association with various genetic syndromes in recent years. Two previous studies have noted an association of Chiari malformations with Rubinstein-Taybi syndrome (RTS). In this clinical report, we highlight identical twins with RTS caused by a mutation in CREBBP that presented with slightly different Chiari malformations in association with an extensive multiloculated syrinx and scoliosis. RTS has been found to be associated with craniocervical abnormalities in literature review, and this clinical report demonstrates the prudent consideration of the physician who cares for patients impacted by RTS to effectively screen via symptomatology and physical examination for Chiari pathology or other craniocervical abnormalities. PMID:21932317

  2. The Chiari Severity Index: A Preoperative Grading System for Chiari Malformation Type 1

    PubMed Central

    Greenberg, Jacob K; Yarbrough, Chester K; Radmanesh, Alireza; Godzik, Jakub; Yu, Megan; Jeffe, Donna B; Smyth, Matthew D; Park, Tae Sung; Piccirillo, Jay F; Limbrick, David D

    2014-01-01

    Background To develop evidence-based treatment guidelines for Chiari Malformation Type 1 (CM-1), preoperative prognostic indices capable of stratifying patients for comparative trials are needed. Objective To develop a preoperative Chiari Severity Index (CSI) integrating the clinical and neuroimaging features most predictive of long-term patient-defined improvement in quality of life (QOL) after CM-1 surgery. Methods We recorded preoperative clinical (e.g. headaches, myelopathic symptoms) and neuroimaging (e.g. syrinx size, tonsillar descent) characteristics. Brief follow-up surveys were administered to assess overall patient-defined improvement in QOL. We used sequential sequestration to develop clinical and neuroimaging grading systems and conjunctive consolidation to integrate these indices to form the CSI. We evaluated statistical significance using the Cochran-Armitage test and discrimination using the c-statistic. Results Our sample included 158 patients. Sequential sequestration identified headache characteristics and myelopathic symptoms as the most impactful clinical parameters, producing a clinical grading system with improvement rates ranging from 81% (grade 1) to 58% (grade 3) (p=0.01). Based on sequential sequestration, the neuroimaging grading system included only the presence (55% improvement) or absence (74% improvement) of a syrinx ≥ 6 mm (p=0.049). Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged from 83% (grade 1) to 45% (grade 3) (p=0.002). The combined CSI had moderately better discrimination (c=0.66) than the clinical (c=0.62) or neuroimaging (c=0.58) systems alone. Conclusion Integrating clinical and neuroimaging characteristics, the CSI is a novel tool that predicts patient-defined improvement following CM-1 surgery. The CSI may aid preoperative counseling and stratify patients in comparative effectiveness trials. PMID:25584956

  3. Syringomyelia with Chiari I malformation presenting as hip charcot arthropathy: a case report and literature review.

    PubMed

    Memarpour, Roya; Tashtoush, Basheer; Issac, Lydia; Gonzalez-Ibarra, Fernando

    2015-01-01

    Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine. PMID:25692057

  4. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

    PubMed Central

    Memarpour, Roya; Gonzalez-Ibarra, Fernando

    2015-01-01

    Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine. PMID:25692057

  5. Fractal dimension analysis of cerebellum in Chiari Malformation type I.

    PubMed

    Akar, Engin; Kara, Sadık; Akdemir, Hidayet; Kırış, Adem

    2015-09-01

    Chiari Malformation type I (CM-I) is a serious neurological disorder that is characterized by hindbrain herniation. Our aim was to evaluate the usefulness of fractal analysis in CM-I patients. To examine the morphological complexity features of this disorder, fractal dimension (FD) of cerebellar regions were estimated from magnetic resonance images (MRI) of 17 patients with CM-I and 16 healthy control subjects in this study. The areas of white matter (WM), gray matter (GM) and cerebrospinal fluid (CSF) were calculated and the corresponding FD values were computed using a 2D box-counting method in both groups. The results indicated that CM-I patients had significantly higher (p<0.05) FD values of GM, WM and CSF tissues compared to control group. According to the results of correlation analysis between FD values and the corresponding area values, FD and area values of GM tissues in the patients group were found to be correlated. The results of the present study suggest that FD values of cerebellar regions may be a discriminative feature and a useful marker for investigation of abnormalities in the cerebellum of CM-I patients. Further studies to explore the changes in cerebellar regions with the help of 3D FD analysis and volumetric calculations should be performed as a future work. PMID:26189156

  6. Optical Coherence Tomography in Patients with Chiari I Malformation

    PubMed Central

    Perrini, Paolo; Miccoli, Mario; Baggiani, Angelo; Nardi, Marco

    2015-01-01

    Background/Aims. To evaluate optic nerve head with spectral domain optical coherence tomography (OCT) in patients with Chiari I malformation (CMI) compared to healthy controls. Methods. Cross-sectional study. OCT of the optic nerve head of 22 patients with CMI and 22 healthy controls was quantitatively analyzed. The healthy controls were matched for age and sex with the study population. Mean retinal nerve fiber layer (RNFL) thickness was calculated for both eyes; the mean thickness value was also registered for each quadrant and for each subfield of the four quadrants. Results. CMI patients showed a reduction of the RNFL thickness in both eyes. This reduction was more statistically significant (P < 0.05) for the inferior quadrant in the right eye and in each quadrant than nasal one in the left eye. Conclusion. A distress of the retinal nerve fibers could explain the observed reduction of the RNFL thickness in patients with CMI; in our series the reduction of the RNFL thickness seems lower when CMI is associated with syringomyelia. PMID:25815335

  7. Quantitative Analysis of Chiari-Like Malformation and Syringomyelia in the Griffon Bruxellois Dog

    PubMed Central

    Knowler, Susan P.; McFadyen, Angus K.; Freeman, Courtenay; Kent, Marc; Platt, Simon R.; Kibar, Zoha; Rusbridge, Clare

    2014-01-01

    This study aimed to develop a system of quantitative analysis of canine Chiari-like malformation and syringomyelia on variable quality MRI. We made a series of measurements from magnetic resonance DICOM images from Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables. We found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes. PMID:24533070

  8. Stereological and Morphometric Analysis of MRI Chiari Malformation Type-1

    PubMed Central

    Alkoç, Ozan Alper; Songur, Ahmet; Eser, Olcay; Toktas, Muhsin; Esi, Ertap; Haktanir, Alpay

    2015-01-01

    Objective In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements. Methods A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development. Results Tonsillar herniation length was measured 9.09±3.39 mm below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased. Conclusion Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition. PMID:26713146

  9. Chiari Type I Malformations in Young Adults: Implications for the College Health Practitioner

    ERIC Educational Resources Information Center

    Elam, Mary Jane; Vaughn, John A.

    2011-01-01

    In this article, the authors describe 2 cases of Chiari type I malformation (CM-I) in students presenting to a college health center within a 6-month period. A review of CM-I, including epidemiology, typical presentation, evaluation, and management, is followed by a discussion of the clinical and functional implications of the disorder in an…

  10. Recurrent subdural hygromas after foramen magnum decompression for Chiari Type I malformation.

    PubMed

    Pereira, Erlick A C; Steele, Louise F; Magdum, Shailendra A

    2014-06-01

    A paediatric case of foramen magnum decompression for Chiari Type I malformation complicated by recurrent subdural hygromas (SH) and raised intracranial pressure without ventriculomegaly is described. SH pathogenesis is discussed, with consideration given to arachnoid fenestration. We summarise possibilities for treatment and avoidance of this unusual consequence of foramen magnum decompression. PMID:23952134

  11. Bilateral Vocal Fold Paralysis After Surgery Immediately in Adult Patient With Chiari Malformation.

    PubMed

    Chen, Yan; Yue, Jianhong; Yuan, Weixiu

    2016-06-01

    The authors report the case of a 50-year-old woman with a bilateral vocal fold paralysis after foramen magnum decompression and resection of partial cerebellar tonsil for Chiari malformation. The possible mechanisms of postoperative bilateral vocal fold paralysis are discussed. PMID:27152564

  12. Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment).

    PubMed

    Fernández, Alfredo Avellaneda; Guerrero, Alberto Isla; Martínez, Maravillas Izquierdo; Vázquez, María Eugenia Amado; Fernández, Javier Barrón; Chesa i Octavio, Ester; Labrado, Javier De la Cruz; Silva, Mercedes Escribano; de Araoz, Marta Fernández de Gamboa Fernández; García-Ramos, Rocío; Ribes, Miguel García; Gómez, Carmen; Valdivia, Joaquín Insausti; Valbuena, Ramón Navarro; Ramón, José R

    2009-01-01

    Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neuropathic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarily based on spinal or peripheral electrical stimulation. It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms. Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition. It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients. PMID:20018097

  13. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment)

    PubMed Central

    2009-01-01

    Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and remission. The diagnosis of Chiari type I malformation in patients with or without symptoms is established with neuroimaging techniques. The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neurophatic pain: either pharmacological and non-pharmacological. Pharmacological therapy use drugs that act on different components of pain. Non-pharmacological therapies are primarly based on spinal or peripheral electrical stimulation. It is important to determine the needs of the patients in terms of health-care, social, educational, occupational, and relationship issues, in addition to those derived from information aspects, particularly at onset of symptoms. Currently, there is no consensus among the specialists regarding the etiology of the disease or how to approach, monitor, follow-up, and treat the condition. It is necessary that the physicians involved in the care of people with this condition comprehensively approach the management and follow-up of the patients, and that they organize interdisciplinary teams including all the professionals that can help to increase the quality of life of patients. PMID:20018097

  14. Symptomatic posterior fossa and supratentorial subdural hygromas as a rare complication following foramen magnum decompression for Chiari malformation Type I.

    PubMed

    Bahuleyan, Biji; Menon, Girish; Hariharan, Easwer; Sharma, Mridul; Nair, Suresh

    2011-02-01

    Symptomatic subdural hygroma due to foramen magnum decompression for Chiari malformation Type I is extremely rare. The authors present their experience with 2 patients harboring such lesions and discuss treatment issues. They conclude that the possibility of subdural hygromas should be considered in all patients presenting with increased intracranial tension following foramen magnum decompression for Chiari malformation Type I. Immediate neuroimaging and appropriate surgical intervention provides a good outcome. PMID:20849216

  15. Presentation and management of hydromyelia in children with Chiari type-II malformation.

    PubMed

    La Marca, F; Herman, M; Grant, J A; McLone, D G

    1997-02-01

    Hydromyelia in patients with myelomeningocele and Chiari-II malformation is a relatively frequent finding on MRI studies. However, not all children develop symptoms from the hydromyelia that requires treatment. Furthermore, treatment of hydromyelia in spina bifida patients is rather complex due to the associated malformations. The authors retrospectively analyzed 231 MRI studies carried out on spina bifida patients who presented neurological deterioration. Hydromyelia was found in 48.5% of the patients. Forty-five children with severe hydromyelia required treatment. These patients were first divided into 2 groups: those with holocord hydromyelia, and those with a segmental lesion. Fifteen patients presented symptoms characteristic of symptomatic Chiari-II malformation: neck rigidity; swallowing difficulty; pain in the upper extremeties; weakness or spasticity in the upper extremeties. Eighteen patients presented symptoms typical of the tethered cord syndrome: scoliosis; worsening bladder and/or bowel function; pain in the lower extremeties; weakness or spasticity in the lower extremeties. Twelve patients presented a mixed-type symptomatology. These patients subsequently underwent posterior cervical decompression, tethered cord release or insertion of a hydromyelia-pleural shunt according to the type of presenting symptoms and to the extent of the hydromyelic lesion. A pattern of successful treatment was identified for each type of presenting clinical and radiological picture. This has allowed the authors to determine an algorithm for optimal treatment of hydromyelia associated with Chiari-II malformation and myelomeningocele, which is proposed here. PMID:9419035

  16. A case report of Charcot arthropathy caused by syringomyelia and Chiari malformation complicated with scoliosis

    PubMed Central

    2014-01-01

    Background Although Charcot arthropathy, also known as neuropathic arthropathy, of which early diagnosis and treatment is extremely difficult, associated with other cause factor has been widely described, Charcot arthropathy caused by syringomyelia and Chiari malformation complicated with scoliosis has never been described in the literature. Case presentation A 44-year-old male was hospitalized for diagnosis and treatment due to complaining the progressively swelling and limitation of motion in his left shoulder joint for 1 year. The patient has no significant past medical history except for scoliosis 8 years prior to his presentation to our clinic; He denied any constitutional symptoms, trauma, or pain in the upper extremities at this time of presentation. Based on history, physical and auxiliary examination, following diagnoses were made: Charcot arthropathy of the left shoulder, syringomyelia, Chiari malformation and scoliosis. Conclusion Once Charcot arthritis was found, it was mostly in advanced stage and very difficult to treat. So we recommended that if patient suffering from scoliosis visited in clinic, further examination such as magnetic resonance imaging (MRI) and regular follow-up should be carried out, and early-stage of this devastating disease caused by syringomyelia and Chiari malformation may be diagnosed easily. PMID:24886292

  17. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report

    PubMed Central

    Moscote-Salazar, Luis Rafael; Zabaleta-Churio, Nasly; Alcala-Cerra, Gabriel; M. Rubiano, Andres; Calderon-Miranda, Willem Guillermo; Alvis-Miranda, Hernando Raphael; Agrawal, Amit

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition. PMID:27162930

  18. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report.

    PubMed

    Moscote-Salazar, Luis Rafael; Zabaleta-Churio, Nasly; Alcala-Cerra, Gabriel; M Rubiano, Andres; Calderon-Miranda, Willem Guillermo; Alvis-Miranda, Hernando Raphael; Agrawal, Amit

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition. PMID:27162930

  19. Chiari Type I malformation and syringomyelia in unrelated patients with blepharophimosis. Report of two cases.

    PubMed

    Paquis, P; Lonjon, M; Brunet, M; Lambert, J C; Grellier, P

    1998-11-01

    Syringomyelia is a rare, mainly sporadic disease of the spinal cord, which is associated with 80% of cases in which a Chiari Type I malformation is also present. A mendelian transmission of syringomyelia (autosomal dominant or recessive) has been proposed in approximately 2% of reported cases. The association of syringomyelia with hereditary diseases (Noonan's syndrome, phacomatoses) has been mentioned frequently in the literature. The authors report the presence of a Chiari Type I malformation accompanied by syringomyelia in two unrelated patients affected by a familial Type II blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). The first patient was a 35-year-old woman who presented with a right C-8 root paresia. The second case involved a 20-year-old man who complained of cervical radicular pain. Both belong to families in which BPES was segregated in an autosomal dominant modality, but other family members had no known neurological symptoms. To the authors' knowledge, such a combination has never been described. Perhaps the possible involvement of a genetic component in some cases of Chiari Type I-associated syringomyelia will someday be debated. PMID:9817424

  20. The relationship between obesity and symptomatic Chiari I malformation in the pediatric population

    PubMed Central

    Lam, Sandi; Auffinger, Brenda; Tormenti, Matthew; Bonfield, Christopher; Greene, Stephanie

    2015-01-01

    Background: Concomitant with the rise in childhood obesity in the United States is an increase in the diagnosis of Chiari I malformation (CM1). Objective: To discern a correlation between obesity and CM1, defined as >5 mm of cerebellar tonsillar descent on sagittal magnetic resonance imaging. Methods: Charts of CM1 patients aged 2–20 years were retrospectively reviewed. Chiari size, age, body mass index (BMI), and CM1 signs/symptoms were recorded. Patients were stratified by age: 2–9, 10–14, and 15–20 years. Mixed-effect linear models and linear regression analysis were applied to investigate the relationship between BMI-for-age percentiles and CM1 signs/symptoms. Results: One hundred sixty-seven patients were included (mean age 14.5 ± 2.97 years, BMI 22.98 ± 6.5, and Chiari size 12.27 mm ± 5.91). When adjusted for age, 42% were overweight or obese–higher than normative BMI for children in the studied area (29.6%). When stratified by age, patients between 2 and 9 years were most commonly obese and presented the highest mean BMI (25.66), largest Chiari size (13.58), and highest incidence of headache (75%) and syringomyelia (66.67%). Patients between 15 and 20 years were most commonly overweight and presented the smallest Chiari size (11.76 mm), but the highest incidence of cerebellar (50%) and brainstem (8.55%) compression symptoms. A significant positive correlation existed between BMI and headache in the first two age groups: (R2: 0.36, P = 0.03; R2: 0.39, P = 0.01, respectively). Obese patients had higher incidence of headache in the 10–14 group (R2: 0.37, P = 0.02) and the largest Chiari size in the 15–20 group (R2: 0.40, P = 0.03). Conclusions: The pediatric CM1 population is more likely to be overweight or obese. Younger obese patients presented the highest incidence of Chiari-related headache symptoms, and older obese patients, the highest incidence of findings other than headache. Thus, body weight and age should be considered when

  1. Evidence of familial syringomyelia in discordant association with Chiari type I malformation.

    PubMed

    Robenek, M; Kloska, S P; Husstedt, I W

    2006-07-01

    We report a sister and two half brothers who presented with magnetic resonance imaging (MRI)-proven syringomyelia and associated Chiari type I malformation in two cases. The individuals have the same mother but two different fathers. The mother shows no clinical signs of syringomyelia. The two fathers died through unknown causes. In a third healthy son of the mother by a relationship with a third father syringomyelia was excluded by MRI. We believe that an autosomal-dominant predisposition is the primary factor in the appearance of syringomyelia in these cases. PMID:16834711

  2. Posterior reversible encephalopathy syndrome in a patient with a Chiari I malformation

    PubMed Central

    Hansberry, David R.; Agarwal, Nitin; Tomei, Krystal L.; Goldstein, Ira M.

    2013-01-01

    Background: The authors describe a unique case of a patient who developed posterior reversible encephalopathy syndrome (PRES) following postoperative treatment of a Chiari I malformation. Case Decsription: A 25-year-old female presented with complaints of left upper and lower extremity paresthesias and gait disturbances. A magnetic resonance imaging (MRI) of the brain and cervical spine showed a Chiari I malformation with tonsillar descent beyond the level of the C1 lamina. She underwent a suboccipital craniectomy and C1 laminectomy with cerebellar tonsillar cauterization and duraplasty. Postoperatively, an MRI showed bilateral acute infarcts of the cerebellar vermis. She was initially treated for cerebellar ischemia with hypertensive therapy with a subsequent decline in her neurologic status and generalized tonic–clonic seizure. Further workup showed evidence of PRES. After weaning pressors, the patient had a significant progressive improvement in her mental status. Conclusion: Although the mechanism of PRES remains controversial given its diverse clinical presentation, several theories implicate hypertension and steroid use as causative agents. PMID:24232171

  3. Spinal cord detethering in children with tethered cord syndrome and Chiari type 1 malformations.

    PubMed

    Glenn, Chad; Cheema, Ahmed A; Safavi-Abbasi, Sam; Gross, Naina L; Martin, Michael D; Mapstone, Timothy B

    2015-11-01

    We discuss the association between tethered cord syndrome (TCS) and Chiari type 1 malformation (CM1), and report on the surgical outcomes of children with CM1 and TCS who underwent sectioning of the filum terminale (SFT). The relationship between TCS and CM1 is unclear. A retrospective review of 170 consecutive spinal cord detetherings between 2008 and 2012 was performed. We identified 17 children with CM1 who underwent SFT. Information regarding clinical presentation, radiographic findings, surgical procedures, and clinical outcomes was analyzed. A mean tonsillar herniation of 10.0mm (range: 5-21) was noted. Children with a fatty or thickened filum terminale demonstrated a greater amount of tonsillar displacement (p<0.005). A low conus medullaris was found in 12 children and a syrinx was present in three. The preoperative symptoms improved in all children. The postoperative MRI (mean 21.8 months) revealed an unchanged tonsillar position in all but one child. No worsening of neurologic function was noted. Pediatric patients who have both CM1 and TCS, but do not demonstrate classic Chiari-related symptoms, may experience symptomatic improvement after filum terminale sectioning. PMID:26165471

  4. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation*

    PubMed Central

    do Vale, Jorge Marques; Silva, Eloísa; Pereira, Isabel Gil; Marques, Catarina; Sanchez-Serrano, Amparo; Torres, António Simões

    2014-01-01

    The Chiari malformation type I (CM-I) has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery. PMID:25410846

  5. Chiari Malformation

    MedlinePlus

    ... indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening ... through an abnormal opening in the back or skull. Type III causes severe neurological defects. Type IV ...

  6. Chiari Malformation

    MedlinePlus

    ... Tel: 914-997-4488; 888-MODIMES (663-4637) Fax: 914-428-8203 National Organization for Rare Disorders ( ... 744-0100; Voice Mail: 800-999-NORD (6673) Fax: 203-798-2291 Spina Bifida Association 4590 MacArthur ...

  7. Chiari Malformation

    MedlinePlus

    ... meaning they are present from birth. In normal anatomy, the cerebellar tonsils are located just above this ( ... Support Groups Telephone Support Groups Online Support Groups Kids For A Cure Club Newsletter BJO Scholarships BJO ...

  8. PROGRESSIVE SYRINGOHYDROMYELIA AND DEGENERATIVE AXONOPATHY IN A BOBCAT (LYNX RUFUS) FOLLOWING SURGICAL CORRECTION OF A CHIARI-LIKE MALFORMATION.

    PubMed

    Sadler, Ryan; Schumacher, Juergen; Ramsay, Edward; McCleery, Brynn; Baine, Katherine; Thomas, William; Nobrega-Lee, Michelle; Henry, George A; Newman, Shelley J

    2016-03-01

    A 3-yr-old male captive bobcat (Lynx rufus) presented with chronic ataxia and right-sided head tilt. Magnetic resonance imaging (MRI) revealed cerebellar crowding and compression consistent with Chiari-like malformation. The clinical signs did not improve after surgical occipital craniectomy, and 2 mo postoperatively a second MRI showed hydromyelia and continued cerebellar compression. The bobcat was euthanized, and necropsy showed chronic focal cerebellar herniation and chronic multifocal atlanto-occipital joint osteophyte proliferation. Histology confirmed the presence of a thick fibrous membrane along the caudal aspect of the cerebellar vermis, suggestive of postoperative adhesions, and axonal degeneration of the cervical spinal cord, even in sections without a central canal lesion. These lesions appear to have been complications associated with surgical correction of the Chiari-like malformation. PMID:27010296

  9. Relationship between pharyngitis and peri-odontoid pannus: A new etiology for some Chiari I malformations?

    PubMed

    Tubbs, R Shane; Griessenauer, Christoph J; Hendrix, Philipp; Oakes, Peter; Loukas, Marios; Chern, Joshua J; Rozzelle, Curtis J; Oakes, W Jerry

    2015-07-01

    The pathophysiology underlying Chiari I malformations (CIMs) provides room for debate with several theories attempting to address this issue. We retrospectively reviewed many of our past patients with pediatric CIMs (specifically, those with peri-odontoid pannus), and present a hypothesis for the development of the malformation in some of said patients. Our experience with the pediatric CIM has shown that almost 1 in 20 patients who present with symptoms is found to have a peri-odontoid pannus. These masses ranged in size from 4 to 11 mm in diameter. Forty percent had a history of clinically significant pharyngitis or pharyngeal abscess. Pannus formation around the dens (odontoid) resulted in ventral compression of the craniocervical junction in each of these patients. Highlighting the hypermobility that causes such lesions, following fusion, the pannus and symptoms in several patients were diminished. Impairment of normal cerebrospinal fluid circulation out of the fourth ventricle and across the craniocervical junction appears to be a plausible endpoint in this discussion and a suitable explanation for some patients with CIM. Still, the mechanisms by which cerebrospinal fluid circulation is compromised may be variable and are not well understood. This is the first study dedicated to the evaluation of pannus formation in the CIM population. We hypothesize that pharyngeal inflammatory conditions contribute to the formation and progression of hindbrain herniation in a small subset of patients with CIMs. PMID:25974330

  10. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance.

    PubMed

    Pillay, P K; Awad, I A; Little, J R; Hahn, J F

    1991-05-01

    Thirty-five consecutive adults with Chiari malformation and progressive symptoms underwent surgical treatment at a single institution over a 3-year period. All patients underwent magnetic resonance imaging scan before and after surgery. Images of the craniovertebral junction confirmed tonsillar herniation in all cases and allowed the definition of two anatomically distinct categories of the Chiari malformation in this age group. Twenty of the 35 patients had concomitant syringomyelia and were classified as Type A. The remaining 15 patients had evidence of frank herniation of the brain stem below the foramen magnum without evidence of syringomyelia and were labeled Type B. Type A patients had a predominant central cord symptomatology; Type B patients exhibited signs and symptoms of brain stem or cerebellar compression. The principal surgical procedure consisted of decompression of the foramen magnum, opening of the fourth ventricular outlet, and plugging of the obex. Significant improvement in preoperative symptoms and signs was observed in 9 of the 20 patients (45%) with syringomyelia (Type A), as compared to 13 of the 15 patients (87%) without syringomyelia (Type B). Postoperative reduction in syrinx volume was observed in 11 of the 20 patients with syringomyelia, including all 9 patients with excellent results. Magnetic resonance imaging has allowed a classification of the adult Chiari malformation in adults based on objective anatomic criteria, with clinical and prognostic relevance. The presence of syringomyelia implies a less favorable response to surgical intervention. PMID:1876240

  11. Inheritance of Chiari-Like Malformation: Can a Mixed Breeding Reduce the Risk of Syringomyelia?

    PubMed Central

    Knowler, Susan P.; v/d Berg, Henny; McFadyen, Angus; La Ragione, Roberto M.; Rusbridge, Clare

    2016-01-01

    Canine Chiari-like malformation (CM) is a complex abnormality of the skull and craniocervical junction associated with miniaturization and brachycephaly which can result in the spinal cord disease syringomyelia (SM). This study investigated the inheritance of CM in a Griffon Bruxellois (GB) family and feasibility of crossbreeding a brachycephalic CM affected GB with a mesaticephalic normal Australian terrier and then backcrossing to produce individuals free of the malformation and regain GB breed characteristics. The study family cohort (n = 27) included five founder dogs from a previous baseline study of 155 GB which defined CM as a global malformation of the cranium and craniocervical junction with a shortened skull base and increased proximity of the cervical vertebrae to the skull. T1-weighted sagittal DICOM images of the brain and craniocervical junction were analysed for five significant traits (two angles, three lines) identified from the previous study and subsequent Qualitative Trait Loci analysis. Mean measurements for mixed breed, pure-breed and baseline study groups were compared. Results indicated that mixed breed traits posed less risk for CM and SM and were useful to distinguish the phenotype. Moreover on the MR images, the filial relationships displayed by the traits exhibited segregation and those presenting the greatest risk for CM appeared additive towards the severity of the condition. The external phenotypes revealed that by outcrossing breed types and with careful selection of appropriate conformation characteristics in the first generation, it is possible to regain the GB breed standard and reduce the degree of CM. The four GB affected with SM in the study all exhibited reduced caudal skull development compared to their relatives. The craniocervical traits may be useful for quantifying CM and assessing the possibility of SM thus assisting breeders with mate selection. However, such a system requires validation to ensure appropriateness for

  12. Simultaneous cerebral and spinal fluid pressure recordings in surgical indications of the Chiari malformation without myelodysplasia.

    PubMed

    Häckel, M; Benes, V; Mohapl, M

    2001-09-01

    Patients with Chiari's malformation without myelodysplasia often suffer from another associated spinal cord lesion--syringomyelia. A condition entirely overriding the clinical picture, affecting adversely the results of surgery as well as the prognosis of this congenital developmental defect. Proceeding from the results of their surgical treatment of 40 patients with hindbrain malformation, the authors recommend, in accordance with the literature, a different approach to and classification of patients depending on the presence of syringomyelia (A/B classification; A: syringomyelia on MR--present, B: absent). The pathological development of the syrinx is caused by obstruction to the natural CSF circulation in the subarachnoid spaces of the craniocervical junction (Williams' dissociation theory). The authors are convinced that routine (static) imaging methods (CT, MR) cannot prove the presence or behaviour of a CSF block, and that they cannot help choose reliably the optimum type of treatment. In contrast, direct measurement (and monitoring) of CSF pressure in different compartments of the CSF pathways (intracranial/intraspinal compartment, i.e., in front of and beyond the suspected block) are a method which can help ascertain with precision the presence and behaviour of a CSF circulatory block, and which also provides dynamic information on such a block during the diurnal rhythm for a period of several days. The authors present the results of a prospective study of 25 patients with hindbrain malformation and describe a technique of parallel monitoring of the two CSF compartments using an adaptation of the Williams method, as well as two different types of dissociation tests designed to prove the presence of a CSF block. CSF flow obstruction was found in 11 patients, in 14 patients it could not be proved. In 8 out of 9 patients with a fully developed syrinx (group A) the block was found as expected. It was, however, diagnosed also in 3 patients until then without any

  13. Identification of Chiari Type I Malformation subtypes using whole genome expression profiles and cranial base morphometrics

    PubMed Central

    2014-01-01

    Background Chiari Type I Malformation (CMI) is characterized by herniation of the cerebellar tonsils through the foramen magnum at the base of the skull, resulting in significant neurologic morbidity. As CMI patients display a high degree of clinical variability and multiple mechanisms have been proposed for tonsillar herniation, it is hypothesized that this heterogeneous disorder is due to multiple genetic and environmental factors. The purpose of the present study was to gain a better understanding of what factors contribute to this heterogeneity by using an unsupervised statistical approach to define disease subtypes within a case-only pediatric population. Methods A collection of forty-four pediatric CMI patients were ascertained to identify disease subtypes using whole genome expression profiles generated from patient blood and dura mater tissue samples, and radiological data consisting of posterior fossa (PF) morphometrics. Sparse k-means clustering and an extension to accommodate multiple data sources were used to cluster patients into more homogeneous groups using biological and radiological data both individually and collectively. Results All clustering analyses resulted in the significant identification of patient classes, with the pure biological classes derived from patient blood and dura mater samples demonstrating the strongest evidence. Those patient classes were further characterized by identifying enriched biological pathways, as well as correlated cranial base morphological and clinical traits. Conclusions Our results implicate several strong biological candidates warranting further investigation from the dura expression analysis and also identified a blood gene expression profile corresponding to a global down-regulation in protein synthesis. PMID:24962150

  14. The Vestibulo-ocular Reflex During Active Head Motion in Chiari II Malformation

    PubMed Central

    Salman, Michael S.; Sharpe, James A.; Lillakas, Linda; Dennis, Maureen; Steinbach, Martin J.

    2008-01-01

    Background Chiari type II malformation (CII) is a developmental anomaly of the cerebellum and brainstem, which are important structures for processing the vestibulo-ocular reflex (VOR). We investigated the effects of the deformity of CII on the angular VOR during active head motion. Methods Eye and head movements were recorded using an infrared eye tracker and magnetic head tracker in 20 participants with CII [11 males, age range 8-19 years, mean (SD) 14.4 (3.2) years]. Thirty-eight age-matched healthy children and adolescents (21 males) constituted the control group. Participants were instructed to ‘look’ in darkness at the position of their thumb, placed 25 cm away, while they made horizontal and vertical sinusoidal head rotations at frequencies of about 0.5 Hz and 2 Hz. Parametric and non-parametric tests were used to compare the two groups. Results The VOR gains, the ratio of eye to head velocities, were abnormally low in two participants with CII and abnormally high in one participant with CII. Conclusion The majority of participants with CII had normal VOR performance in this investigation. However, the deformity of CII can impair the active angular VOR in some patients with CII. Low gain is attributed to brainstem damage and high gain to cerebellar dysfunction. PMID:18973069

  15. Atypical Cerebellar Slump Syndrome and External Hydrocephalus following Craniocervical Decompression for Chiari I Malformation: Case Report

    PubMed Central

    THAKAR, Sumit; DADLANI, Ravi; TAWARI, Manish; HEGDE, Alangar S

    2014-01-01

    Symptomatic cerebellar slump (CS) and external hydrocephalus (EH) are amongst the rarer complications of foramen magnum decompression (FMD) for Chiari I malformation (CM). CS typically presents with delayed onset headache related to dural traction or with neurological deficit offsetting the benefit of FMD. EH, consisting of ventriculomegaly along with subdural fluid collection(s) (SFCs), has been related to cerebrospinal fluid egress from a tiny breach in an otherwise intact arachnoid. We describe the case of a 21-year-old man with CM and syringomyelia who presented with impaired gag, spastic quadriparesis, and raised intracranial pressure 1 week following an uneventful FMD during which the arachnoid had been widely fenestrated. Magnetic resonance imaging (MRI) showed an infratentorial SFC, dilated aqueduct and triventriculomegaly, features of CS, and a residual but resolving syrinx. His symptoms resolved following a high pressure ventriculo-peritoneal shunt. At a 6-month follow-up visit, he was asymptomatic and demonstrated partial resolution of the syrinx, with no recurrence of the SFC. The unusual features in the clinical course of this patient were an atypical CS syndrome presenting with concomitantly resolving syringomyelia, and the development of EH after a wide arachnoidal fenestration. This is the first case in indexed literature describing such a combination of unusual postoperative complications of a FMD. A hypothesis is presented to explain the clinico-radiological findings of the case. PMID:24257499

  16. Neurologic variant laryngomalacia associated with Chiari malformation and cervicomedullary compression: case reports.

    PubMed

    Petersson, Rajanya S; Wetjen, Nicholas M; Thompson, Dana M

    2011-02-01

    Two infants presented with intermittent stridor and evidence of laryngomalacia on flexible laryngoscopy. The first was a 10-month-old girl who had undergone 3 supraglottoplasty surgeries at an outside institution, without long-term resolution of symptoms. She was found during our evaluation to have a Chiari malformation. Laryngomalacia symptoms resolved after suboccipital decompression and C1 laminectomy, and the patient remained symptom-free at 6-month follow-up. The second infant was a 24-day-old boy with velocardiofacial syndrome who was found to have posterior cervicomedullary junction compression at the level of C1. He underwent C1 laminectomy for decompression of the brain stem, which resulted in immediate resolution of symptoms, and he remained symptom-free at 12-month follow-up. Neurologic abnormalities have been reported in up to 50% of infants with laryngomalacia. As such, brain stem dysfunction should be considered among the causes of laryngomalacia during evaluation, especially in patients with failure of supraglottoplasty. Both of these infants had resolution of symptoms after their neurosurgical procedures. PMID:21391421

  17. Diagnosis and surgical treatment of a Chiari I-like malformation in an African lion (Panthera leo).

    PubMed

    McCain, Stephanie; Souza, Marcy; Ramsay, Ed; Schumacher, Juergen; Hecht, Silke; Thomas, William

    2008-09-01

    A 13-mo-old intact male African lion (Panthera leo) presented with a 3-mo history of lethargy, ventral flexion of the neck, abnormal vocalization, and ataxia. Hemogram and serum biochemistries were within normal limits except for the presence of hypokalemia (2.7 mEq/L) and hypochloridemia (108 mEq/L). When no improvement was noted with oral potassium gluconate supplementation, a computed tomography scan of the brain and skull was performed, and no abnormalities were noted. However, magnetic resonance imaging detected occipital bone thickening, crowding of the caudal cranial fossa with cerebellar compression and herniation, and cervical syringohydromyelia, which was consistent with a Chiari I-like malformation. Foramen magnum decompression was performed to relieve the compression of the cerebellum. The animal recovered well with subsequent resolution of clinical signs. Hypovitaminosis A has been proposed previously as the underlying etiology for this malformation in lions with similar clinical presentations. This lion's serum and liver vitamin A concentrations were low (100 ng/ml and 25.31 microg/g, respectively) compared to concentrations reported for domestic carnivores and support hypovitaminosis A as the underlying cause of this animal's Chiari I-like malformation. PMID:18817006

  18. Volumetric analysis of syringomyelia following hindbrain decompression for Chiari malformation Type I: syringomyelia resolution follows exponential kinetics

    PubMed Central

    Coumans, Jean-Valery; Walcott, Brian P.; Butler, William E.; Nahed, Brian V.; Kahle, Kristopher T.

    2013-01-01

    Object Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution. Methods A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R2 (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model. Results Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e−0.0180804x and 13.2556e−0.00615859x. Decay of the syringomyelia followed an exponential model in both patients (R2 = 0.989582 and 0.948864). Conclusions Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I. PMID:21882909

  19. Subacute subdural hygroma and presyrinx formation after foramen magnum decompression with duraplasty for Chiari type 1 malformation.

    PubMed

    Suzuki, Fumio; Kitagawa, Tadashi; Takagi, Kenji; Nozaki, Kazuhiko

    2011-01-01

    A 15-year-old girl developed a rare case of subdural hygroma after foramen magnum decompression for Chiari type 1 malformation manifesting as rapid symptom deterioration around 10 days after uncomplicated operation with uneventful immediate postoperative course. Progressive enlargement of the subdural hygroma in both supra- and infratentorial spaces was followed by the development of hydrocephalus. Syringomyelia improved shortly after the first operation but then deteriorated with massive presyrinx formation. Reoperation with wide opening of the arachnoid membrane lead to a rapid resolution of the hydrocephalus and the presyrinx. The present case shows that wide opening of the arachnoid membrane is an effective therapeutic option. PMID:21613769

  20. Cerebrospinal fluid flow impedance is elevated in Type I Chiari malformation.

    PubMed

    Shaffer, Nicholas; Martin, Bryn A; Rocque, Brandon; Madura, Casey; Wieben, Oliver; Iskandar, Bermans J; Dombrowski, Stephen; Luciano, Mark; Oshinski, John N; Loth, Francis

    2014-02-01

    Diagnosis of Type I Chiari malformation (CMI) is difficult because the most commonly used diagnostic criterion, cerebellar tonsillar herniation (CTH) greater than 3-5 mm past the foramen magnum, has been found to have little correlation with patient symptom severity. Thus, there is a need to identify new objective measurement(s) to help quantify CMI severity. This study investigated longitudinal impedance (LI) as a parameter to assess CMI in terms of impedance to cerebrospinal fluid motion near the craniovertebral junction. LI was assessed in CMI patients (N = 15) and age-matched healthy controls (N = 8) using computational fluid dynamics based on subject-specific magnetic resonance imaging (MRI) measurements of the cervical spinal subarachnoid space. In addition, CTH was measured for each subject. Mean LI in the CMI group (551 ± 66 dyn/cm5) was significantly higher than in controls (220 ± 17 dyn/cm5, p < 0.001). Mean CTH in the CMI group was 9.0 ± 1.1 mm compared to -0.4 ± 0.5 mm in controls. Regression analysis of LI versus CTH found a weak relationship (R2 = 0.46, p < 0.001), demonstrating that CTH was not a good indicator of the impedance to CSF motion caused by cerebellar herniation. These results showed that CSF flow impedance was elevated in CMI patients and that LI provides different information than a standard CTH measurement. Further research is necessary to determine if LI can be useful in CMI patient diagnosis. PMID:24362680

  1. Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I

    PubMed Central

    Kennedy, Benjamin C.; Kelly, Kathleen M.; Phan, Michelle Q.; Bruce, Samuel S.; McDowell, Michael M.; Anderson, Richard C. E.; Feldstein, Neil A.

    2015-01-01

    Object Symptomatic pediatric Chiari malformation Type I (CM-I) is most often treated with posterior fossa decompression (PFD), but controversy exists over whether the dura needs to be opened during PFD. While dural opening as a part of PFD has been suggested to result in a higher rate of resolution of CM symptoms, it has also been shown to lead to more frequent complications. In this paper, the authors present the largest reported series of outcomes after PFD without dural opening surgery, as well as identify risk factors for recurrence. Methods The authors performed a retrospective review of 156 consecutive pediatric patients in whom the senior authors performed PFD without dural opening from 2003 to 2013. Patient demographics, clinical symptoms and signs, radiographic findings, intraoperative ultrasound results, and neuromonitoring findings were reviewed. Univariate and multivariate regression analyses were performed to determine risk factors for recurrence of symptoms and the need for reoperation. Results Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up (mean 32 months). There were no major complications. The mean length of hospital stay was 2.0 days. In a multivariate regression model, partial C-2 laminectomy was an independent risk factor associated with reoperation (p = 0.037). Motor weakness on presentation was also associated with reoperation but only with trend-level significance (p = 0.075). No patient with < 8 mm of tonsillar herniation required reoperation. Conclusions The vast majority (> 90%) of children with symptomatic CM-I will have improvement or resolution of symptoms after a PFD without dural opening. A non–dural opening approach avoids major complications. While no patient with tonsillar herniation < 8 mm required reoperation, children with tonsillar herniation at or below C-2 have a higher risk for failure when this approach is used. PMID:25932779

  2. Patient-reported Chiari malformation type I symptoms and diagnostic experiences: a report from the national Conquer Chiari Patient Registry database.

    PubMed

    Fischbein, Rebecca; Saling, Julia R; Marty, Paige; Kropp, Denise; Meeker, James; Amerine, Jenna; Chyatte, Michelle Renee

    2015-09-01

    Chiari malformation (CM) is a condition in which cerebellar tonsillar ectopia may manifest with various clinical presentations. This study reports from the only national, online patient registry available, the symptoms, comorbid neurocognitive and psychological conditions, and diagnostic experiences of patients living with CM type I (CM I). The current research is one component of a large investigation designed to collect information from individuals with CM through the online Conquer Chiari Patient Registry questionnaire. Analyses included descriptive statistics to study body system impact and patient diagnostic experiences. Participants were 768 individuals with CM I and were predominantly female (86.8 %) and Caucasian (93.8 %) with an average age of 35 years. Pain was the most frequently reported symptom (76.69 %) experienced prior to diagnosis with headaches implicated most often (73.44 %). Neurocognitive comorbidities included memory difficulties (43.88 %) and aphasia (43.75 %) and psychological disorders such as depression (31.77 %) and anxiety disorders (19.92 %) were reported. Average time to diagnosis from first physician visit to diagnosis was 3.43 years, and only 8.46 % of patients had previous awareness of CM. CM I diagnosis was found incidentally for 24.87 % of participants. Common misdiagnoses were classified as psychological (19.26 %) and neurological (19.26 %). Fear was the most frequent emotion elicited at the time of correct diagnosis (42.19 %). CM I can be a challenging condition for patients and physicians, during both the search for diagnosis and management of symptoms. Patient and physician education about CM I may permit early intervention and the prevention of further deterioration and patient suffering. PMID:25972139

  3. Natural and surgical history of Chiari malformation Type I in the pediatric population.

    PubMed

    Pomeraniec, I Jonathan; Ksendzovsky, Alexander; Awad, Ahmed J; Fezeu, Francis; Jane, John A

    2016-03-01

    OBJECT The natural and surgical history of Chiari malformation Type I (CM-I) in pediatric patients is currently not well described. In this study the authors discuss the clinical and radiological presentation and outcomes in a large cohort of pediatric CM-I patients treated with either conservative or surgical management. METHODS The authors retrospectively reviewed 95 cases involving pediatric patients with CM-I who presented between 2004 and 2013. The patients ranged in age from 9 months to 18 years (mean 8 years) at presentation. The cohort was evenly split between the sexes. Twenty-five patients underwent posterior fossa decompression (PFD) with either dural splitting or duraplasty. Seventy patients were managed without surgery. Patients were followed radiologically (mean 44.8 months, range 1.2-196.6 months) and clinically (mean 66.3 months, range 1.2-106.5 months). RESULTS Seventy patients were treated conservatively and followed with serial outpatient neurological and radiological examinations, whereas 25 patients were treated with PFD. Of these 25 surgical patients, 11 were treated with duraplasty (complete dural opening) and 14 were treated with a dura-splitting technique (incomplete dural opening). Surgical intervention was associated with better clinical resolution of symptoms and radiological resolution of tonsillar ectopia and syringomyelia (p = 0.0392). Over the course of follow-up, 20 (41.7%) of 48 nonsurgical patients who were symptomatic at presentation experienced improvement in symptoms and 18 (75%) of 24 symptomatic surgical patients showed clinical improvement (p = 0.0117). There was no statistically significant difference in resolution of symptoms between duraplasty and dura-splitting techniques (p = 0.3572) or between patients who underwent tonsillectomy and tonsillopexy (p = 0.1667). Neither of the 2 patients in the conservative group with syrinx at presentation showed radiological evidence of resolution of the syrinx, whereas 14 (87.5%) of

  4. Cerebellar and hindbrain motion in Chiari malformation with and without syringomyelia.

    PubMed

    Leung, Vannessa; Magnussen, John S; Stoodley, Marcus A; Bilston, Lynne E

    2016-04-01

    OBJECTIVE The pathogenesis of syringomyelia associated with Chiari malformation type I (CM-I) is unclear. Theories of pathogenesis suggest the cerebellar tonsils may obstruct CSF flow or alter pressure gradients, or their motion might act as a piston to increase CSF pressure in the spinal subarachnoid space. This study was performed to measure cerebellar tonsillar and hindbrain motion in CM-I and assess the potential contributions to syrinx formation. METHODS Sixty-four CM-I patients and 25 controls were retrospectively selected from a clinical database, and all subjects had undergone cardiac-gated cine balanced fast-field echo MRI. There were a total of 36 preoperative CM-I scans, which consisted of 15 patients with and 21 patients without syringomyelia. Nineteen patients underwent paired pre- and postoperative imaging. Anteroposterior (AP) and superoinferior (SI) movements of the tip of the cerebellar tonsils, obex, fastigium of the fourth ventricle, pontomedullary junction, and cervicomedullary junction were measured. The distance between the fastigium and tip of the tonsils was used to calculate tonsillar tissue strain (Δi/i0). RESULTS CM-I patients had significantly greater cerebellar tonsillar motion in both the AP and SI directions than controls (AP +0.34 mm [+136%], p < 0.001; SI +0.49 mm [+163%], p < 0.001). This motion decreased after posterior fossa decompression (AP -0.20 mm [-33%], p = 0.001; SI -0.29 mm [-36%]; p < 0.001), but remained elevated above control levels (AP +56%, p = 0.021; SI +67%, p = 0.015). Similar trends were seen for all other tracked landmarks. There were no significant differences in the magnitude or timing of motion throughout the hindbrain between CM-I patients with and without syringomyelia. Increased tonsillar tissue strain correlated with Valsalva headaches (p = 0.03). CONCLUSIONS Cerebellar tonsillar motion may be a potential marker of CM-I and may have use in tailoring surgical procedures. The lack of association with

  5. Imaging-Based Features of Headaches in Chiari Malformation Type I

    PubMed Central

    Alperin, Noam; Loftus, James R.; Oliu, Carlos J.; Bagci, Ahmet M.; Lee, Sang H.; Ertl-Wagner, Birgit; Sekula, Raymond; Lichtor, Terry; Green, Barth A.

    2016-01-01

    Background Sub-occipital cough-induced headaches are considered a hallmark symptom of Chiari Malformation Type I (CMI). However, non-Valsalva–related suboccipital headaches and headaches in other locations are also common in CMI. The diagnostic significance and the underlying factors associated with these different headaches types are not well understood. Objective This imaging-based study compares cranial morphology and hydrodynamics in three types of headaches in CMI to better understand the pathophysiological basis for the different headache characteristics. Methods Twenty-two cranial physiologic and morphologic measures were obtained using specialized MRI scans from 63 symptomatic pretreated CMI patients, 40 with suboccipital-headaches induced by Valsalva maneuvers (34F, 36±10years), 15 with non-Valsalva related suboccipital-headaches (10F, 33±9years), 8 with non-suboccipital non-Valsalva induced headaches (8F, 39±13years), and 37 control subjects (24F, age 36±12years). Group differences were identified using two-tailed Student’s t-test. Results Posterior cranial fossa markers of CMI were similar among the three headache subtypes. However, the Valsalva-related suboccipital-headaches cohort demonstrated significantly lower intracranial compliance index than the non-Valsalva-related suboccipital-headaches cohort (7.5±3.4 vs. 10.9±4.9), lower intracranial volume change during the cardiac cycle (0.48±0.19 vs. 0.61±0.16mL), and higher MRICP (11.1±4.3 vs. 7.7±2.8mmHg, p=0.02). The Valsalva-related suboccipital-headaches cohort had smaller intracranial and lateral ventricles volumes compared to the healthy cohort. The non-Valsalva-related suboccipital-headaches cohort had reduced venous drainage through the jugular veins. Conclusion Valsalva-induced worsening of occipital-headaches appears related to a small intracranial volume rather than the smaller posterior cranial fossa. This explains the reduced intracranial compliance and corresponding higher

  6. Neurofibromatosis type 1 and Chiari type 1 malformation: A case report and literature review of a rare association

    PubMed Central

    Pozetti, Marianne; Belsuzarri, Telmo Augusto Barba; Belsuzarri, Natalia C. B.; Seixas, Naira B.; Araujo, João F. M.

    2016-01-01

    Background: The association between neurofibromatosis type 1 (NF-I) and Chiari I malformation (CMI) is rare, and not many studies are reported in the literature. Performing magnetic resonance imaging (MRI) in patients with NF-1 is essential because several cases of Chiari type I are completely asymptomatic. We emphasize the need for inclusion of Chiari I as diagnosis in association with NF-1. Case Description: The patient was a 51-year-old black man who presented with complaints of pain and decreased motion and sensibility of his hands, wrists, and forearms, along with progressive dysarthria. Even though the computed tomography (CT) scan of the skull did not show changes, the MRI showed hydro/syringomyelia in the cervical spine area. Midline suboccipital craniectomy with total laminectomy of c1 and partial laminectomy of c2 was performed; tonsillectomy was also performed for cistern expansion because of intense thickening and obliteration of the obex by the cerebellar tonsils. Following treatment, the patient showed remission of symptoms. Conclusion: NF-1 in association with CMI is rare, and early diagnosis and surgical treatment are essential to slow down the myelopathy; although they prevent neurological damages, patients with NF-1 must remain under doctor's attention in case of association with CMI. Our literature review showed that symptoms can vary and include headache, gait disturbance, and sensory/motor diminution, until asymptomatic patients. Moreover, the incidence of NF-1 is considerably higher in CMI patients in comparison to the global incidence (8.6–11.8% and 0.775%, respectively). The surgical technique must be evaluated case by case according to the degree of cerebrospinal fluid obstruction. PMID:27500008

  7. Chiari 1 Malformation Presenting as Central Sleep Apnea during Pregnancy: A Case Report, Treatment Considerations, and Review of the Literature

    PubMed Central

    St. Louis, Erik K.; Jinnur, Praveen; McCarter, Stuart J.; Duwell, Ethan J.; Benarroch, Eduardo E.; Kantarci, Kejal; Pichelmann, Mark A.; Silber, Michael H.; Boeve, Bradley F.; Olson, Eric J.; Morgenthaler, Timothy I.; Somers, Virend K.

    2014-01-01

    Purpose: Chiari malformation (CM) type-1 frequently causes obstructive or central sleep-disordered breathing (SDB) in both adults and children, although SDB is relatively rare as a presenting manifestation in the absence of other neurological symptoms. The definitive treatment of symptomatic CM is surgical decompression. We report a case that is, to our knowledge, a novel manifestation of central sleep apnea (CSA) due to CM type-1 with severe exacerbation and initial clinical presentation during pregnancy. Methods: Case report from tertiary care comprehensive sleep medicine center with literature review of SDB manifestations associated with CM type-1. PubMed search was conducted between January 1982 and October 2013. Results: We report a 25-year-old woman with severe CSA initially presenting during her first pregnancy that eventually proved to be caused by CM type-1. The patient was successfully treated preoperatively by adaptive servoventilation (ASV), with effective resolution of SDB following surgical decompression, and without recurrence in a subsequent pregnancy. Our literature review found that 58% of CM patients with SDB had OSA alone, 28% had CSA alone, 8 (10%) had mixed OSA/CSA, and 6 (8%) had hypoventilation. Of CM patients presenting with SDB, 50% had OSA, 42% had CSA, 8% had mixed OSA/CSA, and 10.4% had hypoventilation. We speculate that CSA may develop in CM patients in whom brainstem compression results in excessive central chemoreflex sensitivity with consequent hypocapnic CSA. Conclusion: Chiari malformation type-1 may present with a diversity of SDB manifestations, and timely recognition and surgical referral are necessary to prevent further neurological deficits. ASV therapy can effectively manage CSA caused by CM type-1, which may initially present during pregnancy. PMID:25386156

  8. Quantitative Trait Loci (QTL) Study Identifies Novel Genomic Regions Associated to Chiari-Like Malformation in Griffon Bruxellois Dogs

    PubMed Central

    Lemay, Philippe; Knowler, Susan P.; Bouasker, Samir; Nédélec, Yohann; Platt, Simon; Freeman, Courtenay; Child, Georgina; Barreiro, Luis B.; Rouleau, Guy A.; Rusbridge, Clare; Kibar, Zoha

    2014-01-01

    Chiari-like malformation (CM) is a developmental abnormality of the craniocervical junction that is common in the Griffon Bruxellois (GB) breed with an estimated prevalence of 65%. This disease is characterized by overcrowding of the neural parenchyma at the craniocervical junction and disturbance of cerebrospinal fluid (CSF) flow. The most common clinical sign is pain either as a direct consequence of CM or neuropathic pain as a consequence of secondary syringomyelia. The etiology of CM remains unknown but genetic factors play an important role. To investigate the genetic complexity of the disease, a quantitative trait locus (QTL) approach was adopted. A total of 14 quantitative skull and atlas measurements were taken and were tested for association to CM. Six traits were found to be associated to CM and were subjected to a whole-genome association study using the Illumina canine high density bead chip in 74 GB dogs (50 affected and 24 controls). Linear and mixed regression analyses identified associated single nucleotide polymorphisms (SNPs) on 5 Canis Familiaris Autosomes (CFAs): CFA2, CFA9, CFA12, CFA14 and CFA24. A reconstructed haplotype of 0.53 Mb on CFA2 strongly associated to the height of the cranial fossa (diameter F) and an haplotype of 2.5 Mb on CFA14 associated to both the height of the rostral part of the caudal cranial fossa (AE) and the height of the brain (FG) were significantly associated to CM after 10 000 permutations strengthening their candidacy for this disease (P = 0.0421, P = 0.0094 respectively). The CFA2 QTL harbours the Sall-1 gene which is an excellent candidate since its orthologue in humans is mutated in Townes-Brocks syndrome which has previously been associated to Chiari malformation I. Our study demonstrates the implication of multiple traits in the etiology of CM and has successfully identified two new QTL associated to CM and a potential candidate gene. PMID:24740420

  9. A minimally invasive technique for decompression of Chiari malformation type I (DECMI study): study protocol for a randomised controlled trial

    PubMed Central

    Hu, Yu; Liu, Jiagang; Chen, Haifeng; Jiang, Shu; Li, Qiang; Fang, Yuan; Gong, Shuhui; Wang, Yuelong; Huang, Siqing

    2015-01-01

    Introduction Chiari malformation type I (CM-I) is a congenital hindbrain anomaly that requires surgical decompression in symptomatic patients. Posterior fossa decompression with duraplasty (PFDD) has been widely practiced in Chiari decompression, but dural opening carries a high risk of surgical complications. A minimally invasive technique, dural splitting decompression (DSD), preserves the inner layer of the dura without dural opening and duraplasty, potentially reducing surgical complications, length of operative time and hospital stay, and cost. If DSD is non-inferior to PFDD in terms of clinical improvement, DSD could be an alternative treatment modality for CM-I. So far, no randomised study of surgical treatment of CM-I has been reported. This study aims to evaluate if DSD is an effective, safe and cost-saving treatment modality for adult CM-I patients, and may provide evidence for using the minimally invasive procedure extensively. Methods and analysis DECMI is a randomised controlled, single-masked, non-inferiority, single centre clinical trial. Participants meeting the criteria will be randomised to the DSD group and the PFDD group in a 1:1 ratio. The primary outcome is the rate of clinical improvement, which is defined as the complete resolution or partial improvement of the presenting symptoms/signs. The secondary outcomes consist of the incidence of syrinx reduction, postoperative morbidity rates, reoperation rate, quality of life (QoL) and healthcare resource utilisation. A total of 160 patients will be included and followed up at 3 and 12 months postoperatively. Ethics and dissemination The study protocol was approved by the Biological and Medical Ethics Committee of West China Hospital. The findings of this trial will be published in a peer-reviewed scientific journal and presented at scientific conferences. Trial registration number ChiCTR-TRC-14004099. PMID:25926152

  10. The odontoid process invagination in normal subjects, Chiari malformation and Basilar invagination patients: Pathophysiologic correlations with angular craniometry

    PubMed Central

    Ferreira, Jânio A.; Botelho, Ricardo V.

    2015-01-01

    Background: Craniometric studies have shown that both Chiari malformation (CM) and basilar invagination (BI) belong to a spectrum of malformations. A more precise method to differentiate between these types of CVJM is desirable. The Chamberlain's line violation (CLV) is the most common method to identify BI. The authors sought to clarify the real importance of CLV in the spectrum of craniovertebral junction malformations (CVJM) and to identify possible pathophysiological relationships. Methods: We evaluated the CLV in a sample of CVJM, BI, CM patients and a control group of normal subjects and correlated their data with craniocervical angular craniometry. Results: A total of 97 subjects were studied: 32 normal subjects, 41 CM patients, 9 basilar invagination type 1 (BI1) patients, and 15 basilar invagination type 2 (BI2) patients. The mean CLV violation in the groups were: The control group, 0.16 ± 0.45 cm; the CM group, 0.32 ± 0.48 cm; the BI1 group, 1.35 ± 0.5 cm; and the BI2 group, 1.98 ± 0.18 cm. There was strong correlation between CLV and Boogard's angle (R = 0.82, P = 0.000) and the clivus canal angle (R = 0.7, P = 0.000). Conclusions: CM's CLV is discrete and similar to the normal subjects. BI1 and BI2 presented with at least of 0.95 cm CLV and these violations were strongly correlated with a primary cranial angulation (clivus horizontalization) and an acute clivus canal angle (a secondary craniocervical angle). PMID:26229733

  11. American Syringomyelia & Chiari Alliance Project

    MedlinePlus

    ... Expectations Patients, Family & Friends: For those living with Chiari malformation and syringomyelia — and the families and friends ... resources you need, organized for easy retrieval. Conditions • Chiari Malformation • Syringomyelia • Newsletter • Medical Articles • Related Disorders • BJO ...

  12. Is tooth agenesis related to brainstem anomalies in myelomeningocele patients with Chiari II malformations?

    PubMed

    Linderström, Annelie; Samuelsson, Lars; Huggare, Jan

    2002-12-01

    Defects in the prenatal development of the brainstem can result in cranial nerve deficiencies. As the development of tooth germ is dependent on n. trigeminus, which originates from the brainstem, the hypothesis underlying this study was that anomalies of the brainstem would lead to an increased prevalence of tooth agenesis. Twenty-three patients (13 F and 10 M, age range 6-37 years) were studied, all with myclomeningocele and brainstem anomalies (Chiari II). They were examined retrospectively from the data in journals and dental radiographs and compared to available data on the prevalence of tooth agenesis in the Swedish population. Our hypothesis was rejected, since there was insignificant difference in the frequency of agenesis in our material (8.7%) compared with that of the Swedish population (7.4%). PMID:12512882

  13. Changes over time in craniocerebral morphology and syringomyelia in cavalier King Charles spaniels with Chiari-like malformation

    PubMed Central

    2012-01-01

    Background Chiari-like malformation (CM) and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS). The natural progression of this disease with time has not been described. The objectives of this study were to i) determine if syringomyelia progresses with time ii) determine if features of craniocrebral morphology previously associated with CM are progressive (including caudal cranial fossa volume, caudal cranial fossa parenchymal volume, ventricular dimensions, height of the foramen magnum and degree of cerebellar herniation). A retrospective morphometric analysis was undertaken in 12 CKCS with CM for which repeat magnetic resonance images were available without surgical intervention. Results The maximal syrinx width, height of the foramen magnum, length of cerebellar herniation and caudal cranial fossa volume increased over time. Ventricular and caudal fossa parenchymal volumes were not significantly different between scans. Conclusions The results of this study suggest that syringomyelia progresses with time. Increased caudal cranial fossa volume may be associated with active resorption of the supraoccipital bone, which has previously been found in histology specimens from adult CKCS. We hypothesise that active resorption of the supraoccipital bone occurs due to pressure from the cerebellum. These findings have important implications for our understanding of the pathogenesis and variable natural clinical progression of CM and syringomyelia in CKCS. PMID:23136935

  14. The association between sleep-disordered breathing and magnetic resonance imaging findings in a pediatric cohort with Chiari 1 malformation

    PubMed Central

    Amin, Reshma; Sayal, Priya; Sayal, Aarti; Massicote, Colin; Pham, Robin; Al-Saleh, Suhail; Drake, James; Narang, Indra

    2015-01-01

    BACKGROUND: The prevalence of sleep-disordered breathing (SDB) reported in the literature for Chiari malformation type 1 (CM1) is uniformly high (24% to 70%). In Canada, there is limited access to pediatric polysomnography (PSG). Therefore, the identification of clinical features would be invaluable for triaging these children. OBJECTIVE: To identify demographic features, clinical symptoms/signs and radiological findings associated with SDB in a large pediatric cohort with CM1. METHODS: A retrospective review was conducted on children with CM1 who underwent baseline PSG. Data were collected on patient demographics (age, sex, weight, height, body mass index), clinical symptoms (chart review and clinical questionnaires), diagnostic imaging of the brain and cervicothoracic spine, and medical history at the time of referral. RESULTS: A total of 68 children were included in the review. The mean (± SD) age of the children at the time of PSG was 7.33±4.01 years; 56% (n=38) were male. There was a 49% prevalence of SDB in this cohort based on the overall apnea-hypopnea index. Obstructive sleep apnea was the predominant type of SDB. Tonsillar herniation was significantly correlated with obstructive apnea-hypopnea index (r=0.24; P=0.036). CONCLUSIONS: A direct relationship between the degree of cerebellar tonsillar herniation and obstructive sleep apnea was demonstrated. However, further prospective studies that include neurophysiological assessment are needed to further translate the central nervous system imaging findings to predict the presence of SDB. PMID:25379655

  15. Precocious puberty in two girls with Chiari I malformation: a contribution to a larger use of brain MRI in the diagnosis of central precocious puberty.

    PubMed

    Pucarelli, I; Accardo, F; Tarani, L; Demiraj, V; Segni, M; Pasquino, A M

    2010-06-01

    Up to now Chiari malformation has been reported only in four subjects with precocious puberty, with a prevalence among boys. This article describes the case of two female children affected by progressive precocious puberty detected through brain magnetic resonance imaging (MRI). Brain imaging, even without neurological signs, can identify patients at risk of developing subsequently severe neurological symptoms. Our observation supports the usefulness of brain MRI both in males and females, even when no symptoms are present, to identify and detect high risk cases. However, there is no consensus in Literature in performing MRI in all the patients of both sexes with central precocious puberty, due to its high costs. PMID:20467384

  16. Intraoperative somatosensory evoked potential recovery following opening of the fourth ventricle during posterior fossa decompression in Chiari malformation: case report.

    PubMed

    Grossauer, Stefan; Koeck, Katharina; Vince, Giles H

    2015-03-01

    The most appropriate surgical technique for posterior fossa decompression in Chiari malformation (CM) remains a matter of debate. Intraoperative electrophysiological studies during posterior fossa decompression of Type I CM (CM-I) aim to shed light on the entity's pathomechanism as well as on the ideal extent of decompression. The existing reports on this issue state that significant improvement in conduction occurs after craniotomy in all cases, but additional durotomy contributes a further improvement in only a minority of cases. This implies that craniotomy alone might suffice for clinical improvement without the need of duraplasty or even subarachnoid manipulation at the level of the craniocervical junction. In contrast to published data, the authors describe the case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. Postoperatively, the patient recovered completely from her preoperative neurological deficits. To the authors' knowledge, this is the first report of significant SSEP recovery after opening the fourth ventricle in the decompression of a CM-I. The electrophysiological and operative techniques are described in detail and the findings are discussed in the light of available literature. The authors conclude that there might be a subset of CM-I patients who require subarachnoid dissection at the level of the craniocervical junction to benefit clinically. Prospective studies with detailed electrophysiological analyses seem warranted to answer the question regarding the best surgical approach in CM-I decompression. PMID:25526275

  17. Chiari malformation Type I surgery in pediatric patients. Part 1: validation of an ICD-9-CM code search algorithm.

    PubMed

    Ladner, Travis R; Greenberg, Jacob K; Guerrero, Nicole; Olsen, Margaret A; Shannon, Chevis N; Yarbrough, Chester K; Piccirillo, Jay F; Anderson, Richard C E; Feldstein, Neil A; Wellons, John C; Smyth, Matthew D; Park, Tae Sung; Limbrick, David D

    2016-05-01

    OBJECTIVE Administrative billing data may facilitate large-scale assessments of treatment outcomes for pediatric Chiari malformation Type I (CM-I). Validated International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code algorithms for identifying CM-I surgery are critical prerequisites for such studies but are currently only available for adults. The objective of this study was to validate two ICD-9-CM code algorithms using hospital billing data to identify pediatric patients undergoing CM-I decompression surgery. METHODS The authors retrospectively analyzed the validity of two ICD-9-CM code algorithms for identifying pediatric CM-I decompression surgery performed at 3 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-I), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression or laminectomy). Algorithm 2 restricted this group to the subset of patients with a primary discharge diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated. RESULTS Among 625 first-time admissions identified by Algorithm 1, the overall PPV for CM-I decompression was 92%. Among the 581 admissions identified by Algorithm 2, the PPV was 97%. The PPV for Algorithm 1 was lower in one center (84%) compared with the other centers (93%-94%), whereas the PPV of Algorithm 2 remained high (96%-98%) across all subgroups. The sensitivity of Algorithms 1 (91%) and 2 (89%) was very good and remained so across subgroups (82%-97%). CONCLUSIONS An ICD-9-CM algorithm requiring a primary diagnosis of CM-I has excellent PPV and very good sensitivity for identifying CM-I decompression surgery in pediatric patients. These results establish a basis for utilizing administrative billing data to assess pediatric CM-I treatment outcomes. PMID:26799412

  18. Hydrodynamic and Longitudinal Impedance Analysis of Cerebrospinal Fluid Dynamics at the Craniovertebral Junction in Type I Chiari Malformation

    PubMed Central

    Martin, Bryn A.; Kalata, Wojciech; Shaffer, Nicholas; Fischer, Paul; Luciano, Mark; Loth, Francis

    2013-01-01

    Elevated or reduced velocity of cerebrospinal fluid (CSF) at the craniovertebral junction (CVJ) has been associated with type I Chiari malformation (CMI). Thus, quantification of hydrodynamic parameters that describe the CSF dynamics could help assess disease severity and surgical outcome. In this study, we describe the methodology to quantify CSF hydrodynamic parameters near the CVJ and upper cervical spine utilizing subject-specific computational fluid dynamics (CFD) simulations based on in vivo MRI measurements of flow and geometry. Hydrodynamic parameters were computed for a healthy subject and two CMI patients both pre- and post-decompression surgery to determine the differences between cases. For the first time, we present the methods to quantify longitudinal impedance (LI) to CSF motion, a subject-specific hydrodynamic parameter that may have value to help quantify the CSF flow blockage severity in CMI. In addition, the following hydrodynamic parameters were quantified for each case: maximum velocity in systole and diastole, Reynolds and Womersley number, and peak pressure drop during the CSF cardiac flow cycle. The following geometric parameters were quantified: cross-sectional area and hydraulic diameter of the spinal subarachnoid space (SAS). The mean values of the geometric parameters increased post-surgically for the CMI models, but remained smaller than the healthy volunteer. All hydrodynamic parameters, except pressure drop, decreased post-surgically for the CMI patients, but remained greater than in the healthy case. Peak pressure drop alterations were mixed. To our knowledge this study represents the first subject-specific CFD simulation of CMI decompression surgery and quantification of LI in the CSF space. Further study in a larger patient and control group is needed to determine if the presented geometric and/or hydrodynamic parameters are helpful for surgical planning. PMID:24130704

  19. Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia

    PubMed Central

    Godzik, Jakub; Kelly, Michael P.; Radmanesh, Alireza; Kim, David; Holekamp, Terrence F.; Smyth, Matthew D.; Lenke, Lawrence G.; Shimony, Joshua S.; Park, Tae Sung; Leonard, Jeffrey; Limbrick, David D.

    2014-01-01

    Object Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I–associated syringomyelia [AQ? Edit okay? If not, please advise. JG: edit correct]. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics for additional risk factors for scoliosis. Methods The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis. Results Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63–40.57, p < 0.001) and moderate (5–12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3–25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location. Conclusions The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting

  20. Case Report: Acute obstructive hydrocephalus associated with infratentorial extra-axial fluid collection following foramen magnum decompression and durotomy for Chiari malformation type I

    PubMed Central

    Munakomi, Sunil; Bhattarai, Binod; Chaudhary, Pramod

    2016-01-01

    Acute obstructive hydrocephalus due to infratentorial extra-axial fluid collection (EAFC) is an extremely rare complication of foramen magnum decompression (FMD) and durotomy for Chiari malformation type I. Presence of infratentorial  EAFC invariably causes obstruction at the level of the fourth ventricle or aqueduct of Silvius, thereby indicating its definitive role in hydrocephalus. Pathogenesis of EAFC is said to be a local arachnoid tear as a result of durotomy, as this complication is not described in FMD without durotomy. Controversy exists in management. Usually EAFC is said to resolve with conservative management; so hydrocephalus doesn’t require treatment. However, in this case EAFC was progressive and ventriculo-peritoneal shunting (VPS) was needed for managing progressive and symptomatic hydrocephalus. PMID:27303624

  1. Rubinstein-Taybi syndrome associated with Chiari type I malformation caused by a large 16p13.3 microdeletion: a contiguous gene syndrome?

    PubMed

    Wójcik, Cezary; Volz, Kim; Ranola, Maria; Kitch, Karla; Karim, Tariza; O'Neil, Joseph; Smith, Jodi; Torres-Martinez, Wilfredo

    2010-02-01

    Rubinstein-Taybi Syndrome (RSTS, OMIM 180849) is a rare condition, which in 65% of cases is caused by haploinsufficiency of CREBBP (cAMP response element binding protein binding protein) localized to 16p13.3. A small subset of RSTS cases caused by 16p13.3 microdeletions involving neighboring genes have been recently suggested to be a true contiguous gene syndrome called severe RSTS or 16p13.3 deletion syndrome (OMIM 610543). In the present report, we describe a case of a 2-year-old female with RSTS who, besides most of the typical features of RSTS has corpus callosum dysgenesis and a Chiari type I malformation which required neurosurgical decompression. CGH microarray showed a approximately 520.7 kb microdeletion on 16p13.3 involving CREBBP, ADCY9, and SRL genes. We hypothesize that the manifestations in this patient might be influenced by the haploinsufficiency for ADCY9 and SRL. PMID:20101707

  2. Chiari Malformation: Symptoms

    MedlinePlus

    ... may cause tinnitus (ringing in the ears), depth perception, running into walls and tripping. © 2012 C&S Patient ... Of or pertaining to the mental processes of perception, memory, judgment, and reasoning What We Know: Many ...

  3. Foramen Magnum Decompression and Duraplasty is Superior to Only Foramen Magnum Decompression in Chiari Malformation Type 1 Associated with Syringomyelia in Adults

    PubMed Central

    Berkman, Mehmet Zafer; Ünal, Emre; Akpınar, Elif; Gök, Şevki; Orakdöğen, Metin; Aydın, Salih

    2015-01-01

    Study Design Retrospective cohort study. Purpose To compare surgical results of foramen magnum decompression with and without duraplasty in Chiari malformation type 1 (CM-1) associated syringomyelia (SM). Overview of Literature The optimal surgical treatment of CM-1 associated with SM is unclear. Methods Twenty-five cases of CM-1 with SM were included. There were 12 patients (48%) in the non-duraplasty group and 13 patients (52%) in the duraplasty group. The rate of improvement, state of postoperative SM size, amount of tonsillar herniation, preoperative symptom duration, complications and reoperation rates were analysed. Results The rate of clinical improvement was significantly higher with duraplasty (84.6%) than without (33.3%, p <0.05). The rate of postoperative syrinx regression was significantly higher in the duraplasty group (84.6%) than in the non-duraplasty group (33.3%, p <0.05). One case in the duraplasty group needed a reoperation compared with five cases in the non-duraplasty group (p =0.059). Conclusions Duraplasty is superior to non-duraplasty in CM-1 associated with SM despite a slightly higher complication rate. PMID:26435790

  4. Clinical significance of changes in pB-C2 distance in patients with Chiari Type I malformations following posterior fossa decompression: a single-institution experience.

    PubMed

    Bonney, Phillip A; Maurer, Adrian J; Cheema, Ahmed A; Duong, Quyen; Glenn, Chad A; Safavi-Abbasi, Sam; Stoner, Julie A; Mapstone, Timothy B

    2016-03-01

    OBJECT The coexistence of Chiari malformation Type I (CM-I) and ventral brainstem compression (VBSC) has been well documented, but the change in VBSC after posterior fossa decompression (PFD) has undergone little investigation. In this study the authors evaluated VBSC in patients with CM-I and determined the change in VBSC after PFD, correlating changes in VBSC with clinical status and the need for further intervention. METHODS Patients who underwent PFD for CM-I by the senior author from November 2005 to January 2013 with complete radiological records were included in the analysis. The following data were obtained: objective measure of VBSC (pB-C2 distance); relationship of odontoid to Chamberlain's, McGregor's, McRae's, and Wackenheim's lines; clival length; foramen magnum diameter; and basal angle. Statistical analyses were performed using paired t-tests and a mixed-effects ANOVA model. RESULTS Thirty-one patients were included in the analysis. The mean age of the cohort was 10.0 years. There was a small but statistically significant increase in pB-C2 postoperatively (0.5 mm, p < 0.0001, mixed-effects ANOVA). Eleven patients had postoperative pB-C2 values greater than 9 mm. The mean distance from the odontoid tip to Wackenheim's line did not change after PFD, signifying postoperative occipitocervical stability. No patients underwent transoral odontoidectomy or occipitocervical fusion. No patients experienced clinical deterioration after PFD. CONCLUSIONS The increase in pB-C2 in patients undergoing PFD may occur as a result of releasing the posterior vector on the ventral dura, allowing it to relax posteriorly. This increase appears to be well-tolerated, and a postoperative pB-C2 measurement of more than 9 mm in light of stable craniocervical metrics and a nonworsened clinical examination does not warrant further intervention. PMID:26613273

  5. Stratified Whole Genome Linkage Analysis of Chiari Type I Malformation Implicates Known Klippel-Feil Syndrome Genes as Putative Disease Candidates

    PubMed Central

    Markunas, Christina A.; Soldano, Karen; Dunlap, Kaitlyn; Cope, Heidi; Asiimwe, Edgar; Stajich, Jeffrey; Enterline, David; Grant, Gerald; Fuchs, Herbert

    2013-01-01

    Chiari Type I Malformation (CMI) is characterized by displacement of the cerebellar tonsils below the base of the skull, resulting in significant neurologic morbidity. Although multiple lines of evidence support a genetic contribution to disease, no genes have been identified. We therefore conducted the largest whole genome linkage screen to date using 367 individuals from 66 families with at least two individuals presenting with nonsyndromic CMI with or without syringomyelia. Initial findings across all 66 families showed minimal evidence for linkage due to suspected genetic heterogeneity. In order to improve power to localize susceptibility genes, stratified linkage analyses were performed using clinical criteria to differentiate families based on etiologic factors. Families were stratified on the presence or absence of clinical features associated with connective tissue disorders (CTDs) since CMI and CTDs frequently co-occur and it has been proposed that CMI patients with CTDs represent a distinct class of patients with a different underlying disease mechanism. Stratified linkage analyses resulted in a marked increase in evidence of linkage to multiple genomic regions consistent with reduced genetic heterogeneity. Of particular interest were two regions (Chr8, Max LOD = 3.04; Chr12, Max LOD = 2.09) identified within the subset of “CTD-negative” families, both of which harbor growth differentiation factors (GDF6, GDF3) implicated in the development of Klippel-Feil syndrome (KFS). Interestingly, roughly 3–5% of CMI patients are diagnosed with KFS. In order to investigate the possibility that CMI and KFS are allelic, GDF3 and GDF6 were sequenced leading to the identification of a previously known KFS missense mutation and potential regulatory variants in GDF6. This study has demonstrated the value of reducing genetic heterogeneity by clinical stratification implicating several convincing biological candidates and further supporting the hypothesis that

  6. Increase in Cerebellar Volume in Cavalier King Charles Spaniels with Chiari-like Malformation and Its Role in the Development of Syringomyelia

    PubMed Central

    Shaw, Thomas A.; McGonnell, Imelda M.; Driver, Colin J.; Rusbridge, Clare; Volk, Holger A.

    2012-01-01

    Previous research in Cavalier King Charles Spaniels (CKCS) has found that Chiari-like malformation and syringomyelia (CM/SM) are associated with a volume mismatch between the caudal cranial fossa (CCF) and the brain parenchyma contained within. The objectives of this study were to i) compare cerebellar volume in CKCS (a “high risk’ group which frequently develops CM/SM), small breed dogs (medium risk – occasionally develop CM/SM), and Labradors (low risk – CM/SM not reported); ii) evaluate a possible association between increased cerebellar volume and CM/SM in CKCS; iii) investigate the relationship between increased cerebellar volume and crowding of the cerebellum in the caudal part of the CCF (i.e. the region of the foramen magnum). Volumes of three-dimensional, magnetic resonance imaging derived models of the CCF and cerebellum were obtained from 75 CKCS, 44 small breed dogs, and 31 Labradors. As SM is thought to be a late onset disease process, two subgroups were formed for comparison: 18 CKCS younger than 2 years with SM (CM/SM group) and 13 CKCS older than 5 years without SM (CM group). Relative cerebellar volume was defined as the volume of the cerebellum divided by the total volume of brain parenchyma. Our results show that the CKCS has a relatively larger cerebellum than small breed dogs and Labradors and provide evidence that increased cerebellar volume in CKCS is associated with crowding of cerebellum in the caudal part of the CCF. In CKCS there is an association between increased cerebellar volume and SM. These findings have implications for the understanding of the pathological mechanisms of CM/SM, and support the hypothesis that it is a multifactorial disease process governed by increased cerebellar volume and failure of the CCF to reach a commensurate size. PMID:22506005

  7. Chiari malformation Type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York.

    PubMed

    Greenberg, Jacob K; Olsen, Margaret A; Yarbrough, Chester K; Ladner, Travis R; Shannon, Chevis N; Piccirillo, Jay F; Anderson, Richard C E; Wellons, John C; Smyth, Matthew D; Park, Tae Sung; Limbrick, David D

    2016-05-01

    OBJECTIVE Chiari malformation Type I (CM-I) is a common and often debilitating pediatric neurological disease. However, efforts to guide preoperative counseling and improve outcomes research are impeded by reliance on small, single-center studies. Consequently, the objective of this study was to investigate CM-I surgical outcomes using population-level administrative billing data. METHODS The authors used Healthcare Cost and Utilization Project State Inpatient Databases (SID) to study pediatric patients undergoing surgical decompression for CM-I from 2004 to 2010 in California, Florida, and New York. They assessed the prevalence and influence of preoperative complex chronic conditions (CCC) among included patients. Outcomes included medical and surgical complications within 90 days of treatment. Multivariate logistic regression was used to identify risk factors for surgical complications. RESULTS A total of 936 pediatric CM-I surgeries were identified for the study period. Overall, 29.2% of patients were diagnosed with syringomyelia and 13.7% were diagnosed with scoliosis. Aside from syringomyelia and scoliosis, 30.3% of patients had at least 1 CCC, most commonly neuromuscular (15.2%) or congenital or genetic (8.4%) disease. Medical complications were uncommon, occurring in 2.6% of patients. By comparison, surgical complications were diagnosed in 12.7% of patients and typically included shunt-related complications (4.0%), meningitis (3.7%), and other neurosurgery-specific complications (7.4%). Major complications (e.g., stroke or myocardial infarction) occurred in 1.4% of patients. Among children with CCCs, only comorbid hydrocephalus was associated with a significantly increased risk of surgical complications (OR 4.5, 95% CI 2.5-8.1). CONCLUSIONS Approximately 1 in 8 pediatric CM-I patients experienced a surgical complication, whereas medical complications were rare. Although CCCs were common in pediatric CM-I patients, only hydrocephalus was independently

  8. Dexmedetomidine for an awake fiber-optic intubation of a parturient with Klippel-Feil syndrome, Type I Arnold Chiari malformation and status post released tethered spinal cord presenting for repeat cesarean section

    PubMed Central

    Shah, Tanmay H.; Badve, Manasi S.; Olajide, Kowe O.; Skorupan, Havyn M.; Waters, Jonathan H.; Vallejo, Manuel C.

    2011-01-01

    Patients with Klippel-Feil Syndrome (KFS) have congenital fusion of their cervical vertebrae due to a failure in the normal segmentation of the cervical vertebrae during the early weeks of gestation and also have myriad of other associated anomalies. Because of limited neck mobility, airway management in these patients can be a challenge for the anesthesiologist. We describe a unique case in which a dexmedetomidine infusion was used as sedation for an awake fiber-optic intubation in a parturient with Klippel-Feil Syndrome, who presented for elective cesarean delivery. A 36-year-old female, G2P1A0 with KFS (fusion of cervical vertebrae) who had prior cesarean section for breech presentation with difficult airway management was scheduled for repeat cesarean delivery. After obtaining an informed consent, patient was taken in the operating room and non-invasive monitors were applied. Dexmedetomidine infusion was started and after adequate sedation, an awake fiber-optic intubation was performed. General anesthetic was administered after intubation and dexmedetomidine infusion was continued on maintenance dose until extubation. Klippel-Feil Syndrome (KFS) is a rare congenital disorder for which the true incidence is unknown, which makes it even rare to see a parturient with this disease. Patients with KFS usually have other congenital abnormalities as well, sometimes including the whole thoraco-lumbar spine (Type III) precluding the use of neuraxial anesthesia for these patients. Obstetric patients with KFS can present unique challenges in administering anesthesia and analgesia, primarily as it relates to the airway and dexmedetomidine infusion has shown promising result to manage the airway through awake fiberoptic intubation without any adverse effects on mother and fetus. PMID:24765318

  9. Basilar invagination, Chiari malformation, syringomyelia: a review.

    PubMed

    Goel, Atul

    2009-01-01

    Institute and personal experience (over 25 years) of basilar invagination was reviewed. The database of the department included 3300 patients with craniovertebral junction pathology from the year 1951 till date. Patients with basilar invagination were categorized into two groups based on the presence (Group A) or absence (Group B) of clinical and radiological evidence of instability of the craniovertebral junction. Standard radiological parameters described by Chamberlain were used to assess the instability of the craniovertebral junction. The pathogenesis and clinical features in patients with Group A basilar invagination appeared to be related to mechanical instability, whereas it appeared to be secondary to embryonic dysgenesis in patients with Group B basilar invagination. Treatment by facetal distraction and direct lateral mass fixation can result in restoration of craniovertebral and cervical alignment in patients with Group A basilar invagination. Such a treatment can circumvent the need for transoral or posterior fossa decompression surgery. Foramen magnum bone decompression appears to be a rational surgical treatment for patients having Group B basilar invagination. The division of patients with basilar invagination on the basis of presence or absence of instability provides insight into the pathogenesis of the anomaly and a basis for rational surgical treatment. PMID:19587461

  10. Surgical management of syringomyelia-Chiari complex.

    PubMed

    Ergün, R; Akdemir, G; Gezici, A R; Tezel, K; Beskonakli, E; Ergüngör, F; Taskin, Y

    2000-12-01

    Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia-Chiari complex. More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This report presents 18 consecutive adult symptomatic syringomyelia patients with Chiari malformation who underwent foramen magnum decompression and syringosubarachnoid shunting. The principal indication for the surgery was significant progressive neurological deterioration. All patients underwent preoperative and postoperative MRI scans and were studied clinically and radiologically to assess the changes in the syrinx and their neurological picture after surgical intervention. All patients have been followed up for at least 36 months. No operative mortality was encountered; 88.9% of the patients showed improvement of neurological deficits together with radiological improvement and 11.1% of them revealed collapse of the syrinx cavity but no change in neurological status. None of the patients showed further deterioration of neurological function. The experience obtained from this study demonstrates that foramen magnum decompression to free the cerebro-spinal fluid (CSF) pathways combined with a syringosubarachnoid shunt performed at the same operation succeeds in effectively decompressing the syrinx cavity, and follow-up MR images reveal that this collapse is maintained. In view of these facts, we strongly recommend this technique, which seems to be the most rational surgical procedure in the treatment of syringomyelia-Chiari complex. PMID:11189926

  11. 75 FR 17169 - Nextera Energy Duane Arnold, LLC, Duane Arnold Energy Center; Exemption

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-05

    ... COMMISSION Nextera Energy Duane Arnold, LLC, Duane Arnold Energy Center; Exemption 1.0 Background NextEra Energy Duane Arnold, LLC, formerly FPL Energy Duane Arnold, LLC (the licensee) is the holder of Facility Operating License No. DPR- 49, which authorizes operation of the Duane Arnold Energy Center (Duane...

  12. 09-NIF Dedication: Arnold Schwarzenegger

    ScienceCinema

    Governor Arnold Schwarzenegger

    2010-09-01

    The National Ignition Facility, the world's largest laser system, was dedicated at a ceremony on May 29, 2009 at Lawrence Livermore National Laboratory. These are the remarks by California Governor Arnold Schwarzenegger.

  13. 09-NIF Dedication: Arnold Schwarzenegger

    SciTech Connect

    Governor Arnold Schwarzenegger

    2009-07-02

    The National Ignition Facility, the world's largest laser system, was dedicated at a ceremony on May 29, 2009 at Lawrence Livermore National Laboratory. These are the remarks by California Governor Arnold Schwarzenegger.

  14. 75 FR 13318 - Nextera Energy Duane Arnold, LLC; Duane Arnold Energy Center; Environmental Assessment and...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-03-19

    ... COMMISSION Nextera Energy Duane Arnold, LLC; Duane Arnold Energy Center; Environmental Assessment and Finding... Operating License No. DPR-49, issued to NextEra Energy Duane Arnold, LLC (the licensee), for operation of the Duane Arnold Energy Center, located in Palo, Iowa. In accordance with 10 CFR 51.21, the...

  15. Budd-Chiari syndrome

    PubMed Central

    Martens, Pieter

    2015-01-01

    Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract at any level between the junction of the inferior vena cava with the right atrium and the small hepatic veins. In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less-invasive treatment regimen. The application of these treatment strategies allows for a five-year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post-transplant lymphoma in transplant patients. PMID:26668741

  16. Sutures - separated

    MedlinePlus

    The problem may be caused by: Arnold-Chiari malformation Battered child syndrome Bleeding inside the brain (intraventricular hemorrhage) Brain tumor Certain vitamin deficiencies Dandy-Walker malformation Down syndrome Hydrocephalus Infections that are ...

  17. Neurosurgical management of congenital malformations of the brain.

    PubMed

    Hervey-Jumper, Shawn L; Cohen-Gadol, Aaron A; Maher, Cormac O

    2011-08-01

    Congenital malformations encompass a diverse group of disorders that often present at birth, either as the result of genetic abnormalities, infection, errors of morphogenesis, or abnormalities in the intrauterine environment. Congenital disorders affecting the brain are now often diagnosed before delivery with the use of prenatal ultrasonography. Over the past several decades, there have been major advances in the understanding and management of these conditions. This review focuses on the most common cranial congenital malformations, limiting the discussion to the neurosurgically relevant aspects of arachnoid cysts, pineal cysts, Chiari malformations, and encephaloceles. PMID:21807319

  18. Amphibian malformations

    USGS Publications Warehouse

    National Wildlife Health Center

    1998-01-01

    Frog malformations have been reported from 42 states. The broad geographic distribution of these malformations warrants national attention. Scientists at the USGS National Wildlife Health Center in Madison, Wisconsin are studying this problem in an effort to document its scope and to determine the causes of the observed malformations.

  19. Arnold Tongues in Cell Dynamics

    NASA Astrophysics Data System (ADS)

    Jensen, Mogens

    In a recent work with Leo Kadanoff we studied the synchronization between an internal and an external frequency. One obtains a highly structured diagram with details that in essence are related to the difference between rational and irrational number. The synchronized regions appear as Arnold tongues that widen as the coupling between the frequencies increases. Such tongues have been observed in many physical systems, like in the Libchaber convective cell in the basement of the University of Chicago. In biological systems, where oscillators appear in in a broad variety, very little research on Arnold tongues has been performed. We discuss single cell oscillating dynamics triggered by an external cytokine signal. When this signal is overlaid by an oscillating variation, the two oscillators might couple leading to Arnold tongue diagram. When the tongues overlap, the cell dynamics can shift between the tongues eventually leading to a chaotic response. We quantify such switching in single cell experiments and in model systems based on Gillespie simulations. Kadanoff session.

  20. ARNOLD MESA ROADLESS AREA, ARIZONA.

    USGS Publications Warehouse

    Wolfe, Edward W.; McColly, Robert A.

    1984-01-01

    Geologic geochemical, and aeromagnetic investigations and a survey of mines and prospects in the Arnold Mesa Roadless Area, Arizona, provide little evidence for the occurrence of mineral or energy resources. Buried Proterozoic basement rocks are possible hosts of porphyry-type copper and massive sulfide deposits but the thick cover of Paleozoic sedimentary rocks and upper Cenozoic volcanic rocks precluded assessment of this possibility. Chemistry and temperature of spring and well waters suggest that a geothermal resource may exist near the eastern margin of the roadless area, but the anomaly has not been tested by drilling and this resource remains unverified. No other energy resources were identified.

  1. Arnold Hely and Australian Adult Education

    ERIC Educational Resources Information Center

    Morris, Roger

    2011-01-01

    Arnold Hely (1907-1967) was a most significant figure in the history of adult education in New Zealand, in Australia and internationally. Arnold Hely, a New Zealander, Director of Tutorial Classes (later Adult Education) at the University of Adelaide from 1957 to 1965, was the prime mover in the establishment in 1964 of the Asian South Pacific…

  2. Benedict Arnold: A Question of Honor.

    ERIC Educational Resources Information Center

    Nicolosi, Annie; O'Connell, Libby Haight; Rust, Mead

    2003-01-01

    The spring 2003 The Idea Book for Educators highlights television programming from the Arts and Entertainment Network (A&E), the History Channel, and the Biography Channel, with a focus on an A&E original movie premiere, "Benedict Arnold: A Question of Honor." The booklet contains the following materials: "A&E Study Guide: Benedict Arnold: A…

  3. Arteriovenous Malformation

    MedlinePlus

    ... to the formation of brain AVMs. NIH Patient Recruitment for Arteriovenous Malformation Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 Brain Aneurysm Foundation 269 Hanover Street, ...

  4. Brain Malformations

    MedlinePlus

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  5. Three-dimensional tori and Arnold tongues

    NASA Astrophysics Data System (ADS)

    Sekikawa, Munehisa; Inaba, Naohiko; Kamiyama, Kyohei; Aihara, Kazuyuki

    2014-03-01

    This study analyzes an Arnold resonance web, which includes complicated quasi-periodic bifurcations, by conducting a Lyapunov analysis for a coupled delayed logistic map. The map can exhibit a two-dimensional invariant torus (IT), which corresponds to a three-dimensional torus in vector fields. Numerous one-dimensional invariant closed curves (ICCs), which correspond to two-dimensional tori in vector fields, exist in a very complicated but reasonable manner inside an IT-generating region. Periodic solutions emerge at the intersections of two different thin ICC-generating regions, which we call ICC-Arnold tongues, because all three independent-frequency components of the IT become rational at the intersections. Additionally, we observe a significant bifurcation structure where conventional Arnold tongues transit to ICC-Arnold tongues through a Neimark-Sacker bifurcation in the neighborhood of a quasi-periodic Hopf bifurcation (or a quasi-periodic Neimark-Sacker bifurcation) boundary.

  6. Profile: Prof. Sir Arnold Wolfendale FRS

    NASA Astrophysics Data System (ADS)

    2008-08-01

    Born in 1927, Arnold Wolfendale is now Emeritus Professor in the Department of Physics at Durham University. Here he discusses his career, from Stretford Grammar School to particle physics to being appointed 14th Astronomer Royal in 1991 - and beyond.

  7. Arnold, Lyons: Meeting Report: Granta MIST

    NASA Astrophysics Data System (ADS)

    Arnold, Neil; Lyons, Andrew

    2005-08-01

    The magnetosphere, ionosphere and solar-terrestrial physics community gathered at Selwyn College, University of Cambridge, for three days between Tuesday 5 April and Thursday 7 April 2005. Neil Arnold and Andrew Lyons report.

  8. Three-dimensional tori and Arnold tongues

    SciTech Connect

    Sekikawa, Munehisa; Inaba, Naohiko; Kamiyama, Kyohei; Aihara, Kazuyuki

    2014-03-15

    This study analyzes an Arnold resonance web, which includes complicated quasi-periodic bifurcations, by conducting a Lyapunov analysis for a coupled delayed logistic map. The map can exhibit a two-dimensional invariant torus (IT), which corresponds to a three-dimensional torus in vector fields. Numerous one-dimensional invariant closed curves (ICCs), which correspond to two-dimensional tori in vector fields, exist in a very complicated but reasonable manner inside an IT-generating region. Periodic solutions emerge at the intersections of two different thin ICC-generating regions, which we call ICC-Arnold tongues, because all three independent-frequency components of the IT become rational at the intersections. Additionally, we observe a significant bifurcation structure where conventional Arnold tongues transit to ICC-Arnold tongues through a Neimark-Sacker bifurcation in the neighborhood of a quasi-periodic Hopf bifurcation (or a quasi-periodic Neimark-Sacker bifurcation) boundary.

  9. Quantum Realization of Arnold Scrambling for IFRQI

    NASA Astrophysics Data System (ADS)

    Sang, Jianzhi; Wang, Shen; Shi, Xue; Li, Qiong

    2016-08-01

    This paper is concerned with the feasibility of the Arnold scrambling based on Improved Flexible Representation of Quantum Images (IFRQI). Firstly, the flexible representation of quantum image is updated to the improved flexible representation of quantum image (IFRQI) to represent a quantum image with arbitrary size L × B. Then, by making use of Control-NOT gate and Adder-Modular operation, the concrete quantum circuit of Arnold scrambling for IFRQI is designed. Simulation results show the effectiveness of the proposed circuit.

  10. Quantum Realization of Arnold Scrambling for IFRQI

    NASA Astrophysics Data System (ADS)

    Sang, Jianzhi; Wang, Shen; Shi, Xue; Li, Qiong

    2016-04-01

    This paper is concerned with the feasibility of the Arnold scrambling based on Improved Flexible Representation of Quantum Images (IFRQI). Firstly, the flexible representation of quantum image is updated to the improved flexible representation of quantum image (IFRQI) to represent a quantum image with arbitrary size L × B. Then, by making use of Control-NOT gate and Adder-Modular operation, the concrete quantum circuit of Arnold scrambling for IFRQI is designed. Simulation results show the effectiveness of the proposed circuit.

  11. Budd–Chiari Syndrome Following Laparoscopic Cholecystectomy

    PubMed Central

    Amarapurkar, Pooja D.; Parekh, Sunil J.; Sundeep, Punamiya; Amarapurkar, Deepak N.

    2013-01-01

    Patients with thrombophilic disorder while undergoing intra-abdominal surgery may develop splanchnic vein thrombosis which can have dire consequences. Here we report a case of a 38-year-old female who developed acute Budd–Chiari syndrome after a laparoscopic cholecystectomy. She had polycythemia vera which was not diagnosed before surgery. In this report we want to highlight presurgical evaluation of routine biochemical tests and ultrasonography suggestive of myeloproliferative disorders were missed which led to the Budd–Chiari syndrome. We recommend a meticulous look at the routine evaluation done prior to cholecystectomy is essential. PMID:25755508

  12. Atlas Assimilation Patterns in Different Types of Adult Craniocervical Junction Malformations

    PubMed Central

    Ferreira, Edson Dener Zandonadi; Botelho, Ricardo Vieira

    2015-01-01

    Study Design. This is a cross-sectional analysis of resonance magnetic images of 111 patients with craniocervical malformations and those of normal subjects. Objective. To test the hypothesis that atlas assimilation is associated with basilar invagination (BI) and atlas's anterior arch assimilation is associated with craniocervical instability and type I BI. Summary of Background Data. Atlas assimilation is the most common malformation in the craniocervical junction. This condition has been associated with craniocervical instability and BI in isolated cases. Methods. We evaluated midline Magnetic Resonance Images (MRIs) (and/or CT scans) from patients with craniocervical junction malformation and normal subjects. The patients were separated into 3 groups: Chiari type I malformation, BI type I, and type II. The atlas assimilations were classified according to their embryological origins as follows: posterior, anterior, and both arches assimilation. Results. We studied the craniometric values of 111 subjects, 78 with craniocervical junction malformation and 33 without malformations. Of the 78 malformations, 51 patients had Chiari type I and 27 had BI, of whom 10 presented with type I and 17 with type II BI. In the Chiari group, 41 showed no assimilation of the atlas. In the type I BI group, all patients presented with anterior arch assimilation, either in isolation or associated with assimilation of the posterior arch. 63% of the patients with type II BI presented with posterior arch assimilation, either in isolation or associated with anterior arch assimilation. In the control group, no patients had atlas assimilation. Conclusion. Anterior atlas assimilation leads to type I BI. Posterior atlas assimilation more frequently leads to type II BI. Separation in terms of anterior versus posterior atlas assimilation reflects a more accurate understanding of the clinical and embryological differences in craniocervical junction malformations. Level of Evidence: N/A PMID

  13. Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome.

    PubMed

    Segal, Devorah; Heary, Robert F; Sabharwal, Sanjeev; Barry, Maureen T; Ming, Xue

    2016-07-01

    The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1-3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement. Surgery was complicated by bleeding from intracranial vascular malformations. This report describes a very rapidly developing, massive holocord syringomyelia related to CSF obstruction due to an unusual congenital brain malformation and associated vascular overgrowth at the site. Serial, premorbid MRI studies demonstrated the very rapid progression from no Chiari malformation, to progressively greater cerebellar tonsillar herniation, to holocord syrinx. This complication has never been reported in MCAP syndrome and should be considered in any affected MCAP patient with a progressive neurological decline, even if previous spine imaging findings were normal. Surgical complications due to hemorrhage also need to be considered in this vascular brain malformation. PMID:27035547

  14. INTERIOR OF BARN HAYLOFT, LOOKING WEST (Charles Arnold added a ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    INTERIOR OF BARN HAYLOFT, LOOKING WEST (Charles Arnold added a Cleaning Mill to the barn's hayloft c. 1960. This photograph shows the elevator, chaff shoot, and metal funnel that still remain. The barn's gambrel roof is supported by a three-hinged arch truss system) - Arnold Farm, Barn, 1948 Arnold Road, Coupeville, Island County, WA

  15. Prosocial Youth: The Legacy of Arnold Goldstein

    ERIC Educational Resources Information Center

    Amendola, Mark; Oliver, Robert

    2008-01-01

    Arnold P. Goldstein served for over 30 years at Syracuse University where he directed the Center for Research on Aggression. His model of Aggression Replacement Training (ART) was enriched by diverse perspectives of many colleagues. This article highlights the ideas of three persons who strongly influenced Goldstein's work, namely, Jerome Frank,…

  16. Discourse Integration by Manipulation: Matthew Arnold.

    ERIC Educational Resources Information Center

    Crismore, Avon

    In the writing of Matthew Arnold, integration, one great impression rather than many great individual lines, is the most important goal. In his essay, "The Function of Criticism at the Present Time," the "blocs" of his thought are in sets of two, three, or even four sentences: in effect, he writes much like a poet, in couplets, triplets, and…

  17. Congratulations to Vladimir Igorevich Arnol'd

    NASA Astrophysics Data System (ADS)

    2007-06-01

    12 June 2007 was the seventieth birthday of a member of the editorial board of this journal, Academician Vladimir Igorevich Arnol'd. We warmly congratulate Vladimir Igorevich on his birthday and wish him good health, happiness and continuing success in his scientific activities.

  18. 75 FR 82091 - NextEra Energy Duane Arnold, LLC; Duane Arnold Energy Center; Notice of Issuance of Renewed...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-12-29

    ... proposed issuance of the renewed license was published in the Federal Register on February 17, 2009 (73 FR... environmental impact statement (NUREG-1437, Supplement 42), for the Duane Arnold Energy Center, published in... COMMISSION NextEra Energy Duane Arnold, LLC; Duane Arnold Energy Center; Notice of Issuance of...

  19. Anorectal malformations

    PubMed Central

    Gangopadhyay, Ajay Narayan; Pandey, Vaibhav

    2015-01-01

    Anorectal malformations (ARMs) are among the more frequent congenital anomalies encountered in paediatric surgery, with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live births. Antenatal diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period. There is a wide spectrum of presentation ranging from low anomalies with perineal fistula having simple management to high anomalies with complex management. Advances in the imaging techniques with improvement in knowledge of the embryology, anatomy and physiology of ARM cases have refined diagnosis and initial management. There has been marked improvement in survival of such patient over the last century. The management of ARM has moved forward from classical procedures to PSARP to minimal invasive procedures. But still the fecal and urinary incontinence can occur even with an excellent anatomic repair, mainly due to associated problems. There has been a paradigm shift in approach to these patients which involves holistic approach to the syndrome of Anorectal malformations with a long term goal of achievement of complete fecal and urinary continence with excellent quality of life. PMID:25552824

  20. Allergy to Prolene Sutures in a Dural Graft for Chiari Decompression.

    PubMed

    Cajigas, Iahn; Burks, S Shelby; Gernsback, Joanna; Fine, Lauren; Moshiree, Baharak; Levi, Allan D

    2015-01-01

    Allergy to Prolene suture is exceedingly rare with only 5 cases reported in the literature. There have been no such cases associated with neurosurgical procedures. Diagnosis is nearly always delayed in spite of persistent symptomatology. A 27-year-old girl with suspected Ehlers-Danlos, connective tissue disorder, underwent posterior fossa decompression for Chiari Type 1 malformation. One year later, the patient presented with urticarial rash from the neck to chest. Cerebrospinal fluid and blood testing, magnetic resonance imaging, and intraoperative exploration did not suggest allergic reaction. Eventually skin testing proved specific Prolene allergy. After suture material was removed, the patient no longer complained of pruritus or rash. This single case highlights the important entity of allergic reaction to suture material, namely, Prolene, which can present in a delayed basis. Symptomatology can be vague but has typical allergic characteristics. Multidisciplinary approach is helpful with confirmatory skin testing as a vital part of the workup. PMID:26798347

  1. Allergy to Prolene Sutures in a Dural Graft for Chiari Decompression

    PubMed Central

    Cajigas, Iahn; Burks, S. Shelby; Gernsback, Joanna; Fine, Lauren; Moshiree, Baharak; Levi, Allan D.

    2015-01-01

    Allergy to Prolene suture is exceedingly rare with only 5 cases reported in the literature. There have been no such cases associated with neurosurgical procedures. Diagnosis is nearly always delayed in spite of persistent symptomatology. A 27-year-old girl with suspected Ehlers-Danlos, connective tissue disorder, underwent posterior fossa decompression for Chiari Type 1 malformation. One year later, the patient presented with urticarial rash from the neck to chest. Cerebrospinal fluid and blood testing, magnetic resonance imaging, and intraoperative exploration did not suggest allergic reaction. Eventually skin testing proved specific Prolene allergy. After suture material was removed, the patient no longer complained of pruritus or rash. This single case highlights the important entity of allergic reaction to suture material, namely, Prolene, which can present in a delayed basis. Symptomatology can be vague but has typical allergic characteristics. Multidisciplinary approach is helpful with confirmatory skin testing as a vital part of the workup. PMID:26798347

  2. A Single-Center Experience of CNS Anomalies or Neural Tube Defects in Patients With Jarcho-Levin Syndrome.

    PubMed

    Demir, Nihat; Peker, Erdal; Gülşen, İsmail; Ağengin, Kemal; Kaba, Sultan; Tuncer, Oğuz

    2016-03-01

    Jarcho-Levin syndrome (JLS) is a genetic disorder characterized by distinct malformations of the ribs and vertebrae, and/or other associated abnormalities such as neural tube defect, Arnold-Chiari malformation, renal and urinary abnormalities, hydrocephalus, congenital cardiac abnormalities, and extremity malformations. The study included 12 cases at 37-42 weeks of gestation and diagnosed to have had Jarcho-Levin syndrome, Arnold-Chiari malformation, and meningmyelocele. All cases of Jarcho-Levin syndrome had Arnold-Chiari type 2 malformation; there was corpus callosum dysgenesis in 6, lumbosacral meningmyelocele in 6, lumbal meningmyelocele in 3, thoracal meningmyelocele in 3, and holoprosencephaly in 1 of the cases. With this article, the authors underline the neurologic abnormalities accompanying Jarcho-Levin syndrome and that each of these abnormalities is a component of Jarcho-Levin syndrome. PMID:26239489

  3. Arnold A. Lazarus (1932-2013).

    PubMed

    Davison, Gerald C; Wilson, G Terence

    2014-09-01

    Arnold A. Lazarus, distinguished professor emeritus at the Graduate School of Applied and Professional Psychology, Rutgers University, passed away on October 1, 2013. He is regarded as one of the founders of behavior therapy and one of its leading practitioners and teachers. Throughout his career Lazarus enjoyed the rare distinction of being one of the most influential, creative, and highly regarded clinical practitioners in the field of clinical psychology. To those fortunate to have been his students, colleagues, or friends, Lazarus's intelligence, creativity, kindness, and mischievous and often irreverent wit made him very special indeed. PMID:25197839

  4. Author! Author! Creator of Frog and Toad: Arnold Lobel

    ERIC Educational Resources Information Center

    Brodie, Carolyn S.

    2005-01-01

    This article presents a brief biography of author Arnold Lobel, perhaps best known for giving the world Frog and Toad. Arnold Lobel was born in Los Angeles, California, on May 22, 1933, and was raised by his grandparents in New York. He loved checking out books from the library when he was a little boy and sharing with his classmates the stories…

  5. Arnold Gesell and the maturation controversy.

    PubMed

    Dalton, Thomas C

    2005-01-01

    This article examines the work of Arnold Lucius Gesell and argues that he not only paved the way for contemporary research in motor development, but that he and colleagues anticipated fundamental issues about growth that must be addressed by psychologists and neuroscientists who are committed to the advancement of developmental science. Arnold Lucius Gesell was a pioneer in developmental psychology when the field was in its infancy. He worked diligently for the rights of physically and mentally handicapped children to receive special education that would enable them to find gainful employment. Gesell's writings in books and popular magazines increased public awareness of and support for preschool education and better foster care for orphans. Despite these achievements, many of his successors have questioned his views about infant development. Developmental psychologists have criticized Gesell for proposing a stage theory of infant growth that has fallen into disfavor among contemporary researchers. His conception of development as a maturational process has been challenged for allegedly reducing complex behavioral, perceptual, and learning processes to genetic factors. The author rejects this overly simplistic interpretation and contends that Gesell's work continues to stand the test of time. PMID:17549936

  6. Arnold B. Arons (1916-2001)

    NASA Astrophysics Data System (ADS)

    Warren, Bruce A.

    Arnold B. Arons died of a heart attack at his home in Seattle on February 28, 2001, aged 84. He was a long-time member of the American Geophysical Union (1950; Ocean Sciences) and of the American Association of Physics Teachers (AAPT), of which he was president in 1961. He was a Fellow of the American Physical Society, and a Fellow of the American Association for the Advancement of Science.Arnold Arons was a teacher of physics. He taught it to the freshmen at Amherst College from 1952 to 1968, and then, at the University of Washington, he taught prospective teachers of physics how to teach it. He stressed meaning in physical concepts: how it is derived from shared experience, is founded on operational definitions, and is deepened and broadened with growing sophistication, individual and historical. He derided glib chatter about complex ideas (“Gibberish!”), or mere manipulation of symbols and formulas, and insisted—fiercely—that students know what they were talking about. His presence in the lecture hall at Amherst was sometimes terrifying; that technique might not be readily accepted in present, more tender times, but it was effective in shaking high school hotshots loose from some of their delusions. Despite perceived indignities, they usually gave him a standing ovation at the end of the spring semester.

  7. 75 FR 64748 - Nextera Energy Duane Arnold, LLC; Duane Arnold Energy Center; Notice of Availability of the Final...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-10-20

    ... of license renewal for energy- planning decision-makers. The final Supplement 42 to the GEIS is... COMMISSION Nextera Energy Duane Arnold, LLC; Duane Arnold Energy Center; Notice of Availability of the Final Supplement 42 to the Generic Environmental Impact Statement for License Renewal of Nuclear Plants Notice...

  8. Budd-Chiari syndrome: computed tomographic and ultrasonographic findings

    SciTech Connect

    Yang, P.J.; Glazer, G.M.; Bowerman, R.A.

    1983-02-01

    A case of Budd-Chiari syndrome is presented in which computed tomography (CT) and ultrasonography suggested the correct diagnosis and excluded adjacent tumor as the cause of hepatic vein occlusion. The CT appearance in this case (homogeneously increased attenuation of an enlarged caudate lobe) differed from the appearance previously reported (patchy liver enhancement) in Budd-Chiari syndrome. Underlying mechanisms responsible for the different CT appearance are discussed.

  9. Arnold diffusion in a driven optical lattice.

    PubMed

    Boretz, Yingyue; Reichl, L E

    2016-03-01

    The effect of time-periodic forces on matter has been a topic of growing interest since the advent of lasers. It is known that dynamical systems with 2.5 or more degrees of freedom are intrinsically unstable. As a consequence, time-periodic driven systems can experience large excursions in energy. We analyze the classical and quantum dynamics of rubidium atoms confined to a time-periodic optical lattice with 2.5 degrees of freedom. When the laser polarizations are orthogonal, the system consists of two 1.5 uncoupled dynamical systems. When laser polarizations are turned away from orthogonal, an Arnold web forms and the dynamics undergoes a fundamental change. For parallel polarizations, we find huge random excursions in the rubidium atom energies and significant entanglement of energies in the quantum dynamics. PMID:27078351

  10. Arnold diffusion in a driven optical lattice

    NASA Astrophysics Data System (ADS)

    Boretz, Yingyue; Reichl, L. E.

    2016-03-01

    The effect of time-periodic forces on matter has been a topic of growing interest since the advent of lasers. It is known that dynamical systems with 2.5 or more degrees of freedom are intrinsically unstable. As a consequence, time-periodic driven systems can experience large excursions in energy. We analyze the classical and quantum dynamics of rubidium atoms confined to a time-periodic optical lattice with 2.5 degrees of freedom. When the laser polarizations are orthogonal, the system consists of two 1.5 uncoupled dynamical systems. When laser polarizations are turned away from orthogonal, an Arnold web forms and the dynamics undergoes a fundamental change. For parallel polarizations, we find huge random excursions in the rubidium atom energies and significant entanglement of energies in the quantum dynamics.

  11. Budd- Chiari Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus

    PubMed Central

    Sathyaseelan, Arumugam

    2016-01-01

    Budd- Chiari syndrome is caused by obstruction of hepatic venous outflow. There are numerous causes for Budd-Chiari syndrome. One of the causes is systemic lupus erythematosus due to antiphospholipid antibodies. Only few cases have reported Budd-Chiari syndrome as an initial manifestation of systemic lupus erythematosus (SLE). This is a case report of Budd-Chiari syndrome due to SLE. PMID:27190864

  12. Budd- Chiari Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus.

    PubMed

    Pandiaraja, Jayabal; Sathyaseelan, Arumugam

    2016-04-01

    Budd- Chiari syndrome is caused by obstruction of hepatic venous outflow. There are numerous causes for Budd-Chiari syndrome. One of the causes is systemic lupus erythematosus due to antiphospholipid antibodies. Only few cases have reported Budd-Chiari syndrome as an initial manifestation of systemic lupus erythematosus (SLE). This is a case report of Budd-Chiari syndrome due to SLE. PMID:27190864

  13. Pregnancy in Budd-Chiari Syndrome

    PubMed Central

    Merz, Waltraut M.; Rüland, Anna M.; Hippe, Valeria; Poetzsch, Bernd; Meyer, Carsten; Pollok, Joerg M.; Gembruch, Ulrich; Trebicka, Jonel

    2016-01-01

    Abstract Due to its rarity, experience with pregnancy in Budd–Chiari syndrome (BCS) is limited. With the advent of new treatment modalities, transjugular intrahepatic portosystemic shunt in particular, numbers of affected women seeking pregnancy with BCS are expected to rise. Here, we use a case that ended lethal within 2 years after delivery to discuss the effect of pregnancy on BCS and vice versa, and to highlight the necessity of a multidisciplinary teamwork. Additionally, a risk classification is proposed which may serve as a framework for preconception counseling and assist in the establishment and evaluation of treatment algorithms; its criteria need to be defined and assessed for their applicability in further studies. PMID:27258526

  14. MR imaging of hindbrain deformity in Chiari II patients with and without symptoms of brainstem compression.

    PubMed

    Curnes, J T; Oakes, W J; Boyko, O B

    1989-01-01

    We examined the MR appearance of the hindbrain deformity, including the upper cervical spinal canal and craniovertebral junction, in 33 patients with Chiari II malformation. In this disorder, there is impaction at birth of the medulla and cerebellar vermis into the upper cervical spine, resulting in obliteration of the subarachnoid space and scalloping of the dens. Spinal canal enlargement during the child's growth, combined with dorsal displacement of neural tissue, eventually causes marked widening of the precervical subarachnoid space. This enlargement may simulate an intradural mass. Our series documents the changes seen at birth and the progression of the widened precervical space through the first and second decades. Twelve (36%) of the 33 patients studied were symptomatic, with brainstem or longtract symptomatology, and 11 of these required surgery. This group was compared with the remaining 21 asymptomatic Chiari II patients to identify MR features associated with clinical deterioration. The level of descent of the hindbrain hernia was critical; eight of 12 symptomatic patients had a cervicomedullary kink at C4 or lower, while no asymptomatic patients had a fourth ventricle, medulla, or kink below C3-C4. The precervical cord subarachnoid space was slightly wider in asymptomatic patients, although there was great overlap. In five patients with follow-up scans, this space was seen to increase in width after laminectomy. A CSF flow void was present in the precervical space in about 25% of patients in both groups. In nine of 12 symptomatic patients, C1 arch indentation of the dura (causing significant compression) was confirmed surgically. However, seven (33%) of the 21 asymptomatic patients also had this appearance. Absolute measurement of the anteroposterior diameter of the canal at C1 ranged from 11 to 25 mm in both groups. Retrocollis, which persisted despite sedation for MR, was seen in two patients, both symptomatic. Recognition of the vermis, medullary

  15. An Interview with Arnold Bank: Designer, Letterer, and Master Calligrapher.

    ERIC Educational Resources Information Center

    Gregory, Anne

    1985-01-01

    Arnold Bank, emeritus professor of design at Carnegie-Mellon University in Pittsburgh, Pennsylvania, has been one of the most inspiring teachers of calligraphy, paleography, and typography in the United States. His life and work are discussed. (RM)

  16. Budd-Chiari Syndrome in a Patient with Hepatitis C

    PubMed Central

    Hennemeyer, Charles; Flores, Michael S.

    2016-01-01

    Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari syndrome was a primary disease due to hepatic venous webs. Angioplasty was performed in this case, which resolved the patient's symptoms related to portal hypertension. Follow-up venography 5 months after angioplasty demonstrated continued patency of the hepatic veins. A biopsy was obtained in the same setting, which showed centrilobular fibrosis indicating that venous occlusion was indeed the cause of cirrhosis. It is important to consider a second disease when treating a patient with difficult to manage portal hypertension. PMID:27525135

  17. Budd-Chiari Syndrome in a Patient with Hepatitis C.

    PubMed

    Frankl, Joseph; Hennemeyer, Charles; Flores, Michael S; Desai, Archita P

    2016-01-01

    Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari syndrome was a primary disease due to hepatic venous webs. Angioplasty was performed in this case, which resolved the patient's symptoms related to portal hypertension. Follow-up venography 5 months after angioplasty demonstrated continued patency of the hepatic veins. A biopsy was obtained in the same setting, which showed centrilobular fibrosis indicating that venous occlusion was indeed the cause of cirrhosis. It is important to consider a second disease when treating a patient with difficult to manage portal hypertension. PMID:27525135

  18. Intraoral venous malformation with phleboliths

    PubMed Central

    Mohan, Ravi Prakash S.; Dhillon, Manu; Gill, Navneet

    2011-01-01

    The most common type of vascular malformation is the venous malformation and these are occasionally associated with phleboliths. We report a case of a 45 year old woman with intraoral venous malformation with phleboliths. PMID:24151422

  19. Budd-Chiari Syndrome: an unnoticed diagnosis.

    PubMed

    Falcão, Camila Kruschewsky; Fagundes, Gustavo C Freitas; Lamos, Gustavo Checolli; Felipe-Silva, Aloisio; Lovisolo, Silvana Maria; Martines, João Augusto; de Campos, Fernando Peixoto Ferraz

    2015-01-01

    Budd-Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy. PMID:26484330

  20. Cerebellar and Brainstem Malformations.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2016-08-01

    The frequency and importance of the evaluation of the posterior fossa have increased significantly over the past 20 years owing to advances in neuroimaging. Conventional and advanced neuroimaging techniques allow detailed evaluation of the complex anatomic structures within the posterior fossa. A wide spectrum of cerebellar and brainstem malformations has been shown. Familiarity with the spectrum of cerebellar and brainstem malformations and their well-defined diagnostic criteria is crucial for optimal therapy, an accurate prognosis, and correct genetic counseling. This article discusses cerebellar and brainstem malformations, with emphasis on neuroimaging findings (including diagnostic criteria), neurologic presentation, systemic involvement, prognosis, and recurrence. PMID:27423798

  1. Pediatric neuroanesthesia. Arteriovenous malformations.

    PubMed

    Newfield, P; Hamid, R K

    2001-06-01

    Intracranial arteriovenous malformations can occur singly, multiply, and in conjunction with aneurysms and denovo, family, or in conjunction with connective-tissue disorders. Intracranial hemorrhage is the most common presentation, occurring in 20% to 50% of cases. In children, seizures are the second most common presentation occurring in 15% to 20% of cases. The modalities available treatment of arteriovenous malformations are microsurgery, embolization, and stereotactic radiosurgery with heavy particles, alpha knife, or linear accelerator. Induction, maintenance, and emergence from anesthesia are designed to prevent rupture of arteriovenous malformation and aneurysm and to improve intracranial compliance in the presence of an intracranial hematoma, during both diagnostic (CT, MR scanning) and therapeutic procedures. PMID:11469062

  2. CSF Flow in Chiari I and Syringomyelia from the Perspective of Computational Fluid Dynamics.

    PubMed

    Støverud, K-H; Mardal, K-A; Haughton, V; Langtangen, H P

    2011-03-29

    Phase contrast MR in patients with the Chiari I malformation demonstrates abnormal CSF flow in the foramen magnum and upper cervical spinal canal, related to abnormal pressure gradients. The purpose of this study was to analyze the role of CSF pressure in the pathogenesis of syringomyelia, with computational models. The spinal cord was modeled as a cylindrical poro-elastic structure with homogenous and isotropic permeability. The permeability was then made heterogeneous and anisotropic to represent the different properties of the central canal, gray and white matter. Fluid with a defined pressure, varying both in time and space, was prescribed in the SAS. Simulations were performed to quantify deformations and fluid movement within the cord. In the simulations with uniform permeability fluid moved into the cord in regions of higher pressure and out of the cord in regions of lower pressure. With permeability differences simulating gray and white matter the pattern was more complex, but similar. Adding the central spinal canal, fluid moved into the cord as in the previous case. However, preferential flow along the central canal hindered fluid from flowing back into the SAS. Pressure gradients in the SAS produce movement of fluid in the spinal cord. Assuming different relative permeability in gray matter, white matter and the central spinal canal, abnormal CSF gradients lead to accumulation of fluid within and adjacent to the spinal cord central canal. PMID:24059568

  3. A unifying hypothesis for hydrocephalus and the Chiari malformations part two: The hydrocephalus filling mechanism.

    PubMed

    Williams, Helen

    2016-09-01

    It is proposed that negative central nervous system (CNS) pressure is one of the filling mechanisms of the fluid spaces of the CNS. Negative CNS pressure is caused by the combination of gravitational force and body movement. The venous system imposes pressure fluctuations on the CNS due to changes in posture and body cavity pressure. It is proposed here that filling of veins, arteries and cerebrospinal (CSF) spaces are all assisted by negative CNS pressure. Hyperemia in the CNS in response to pressure changes with movement was described in the first part of this hypothesis. By this means parenchyma water levels may increase (Williams, 2008). In the developmental forms of hydrocephalus expansion of CSF spaces is a more prominent feature than parenchyma water changes. This feature is explained by this second part of the hypothesis where the negative pulsatility of pressure that can accompany positive pressure pulsatility, which occurs with body movement, is described as the pathological force that leads to cavity filling. When CNS compliance is lost there is overrepresentation of low and well as high pressure pulsations in response to body movements. Pressure that leads to the development of hydrocephalus can be described as being abnormally labile. Negative CNS pressure causes cavity filling in an analogous way to pleural cavity filling, with water passing from parenchyma tissue. Positive pressure within the pressure profile may cause expansion of regions of the CNS skeletal system that are able to grow such as the cranial vault so that large head size is a frequent accompaniment to hydrocephalus that is caused by this mechanism. Hydrocephalic disorders that are characterised by negative pressure filling mechanism often have a skeletal anatomical abnormality that causes reduced CNS compliance and adversely affect neural development. This is often in the form of obstruction to CSF flow around the base of the brain that then leads to vault expansion by means of high pressure pulsation and ventricle enlargement by means of low pressure pulsation. In health pressure pulsatility does not lead to enduring changes in water distribution within the CNS compartments but it assists physiological water balance. PMID:27515195

  4. Myelomeningocele associated with split cord malformation type I -three case reports-.

    PubMed

    Higashida, Tetsuhiro; Sasano, Mari; Sato, Hironobu; Sekido, Ken'ichi; Ito, Susumu

    2010-01-01

    Three neonates presented with split cord malformation (SCM) associated with myelomeningocele (MMC), complicated with various coexisting anomalies. All patients were female and classified as SCM type I. All patients had a syrinx located rostral to the SCM. One patient had hydrocephalus and Chiari malformation causing serious respiratory problems. Two patients had partial hypertrichosis located close to the MMC, suggesting association with SCM. One patient had sacral hypoplasty and right kidney agenesis, suggesting that some embryologic errors may affect not only neural but also mesodermal development. All patients underwent surgical treatment for SCM after detailed evaluation and management of concomitant anomalies, and developed no new neurological deficits. Delayed surgery is an alternative treatment strategy for SCM in patients with both SCM and MMC with similar complications. PMID:20505305

  5. Arnold L. Gesell: The Paradox of Nature and Nurture.

    ERIC Educational Resources Information Center

    Thelen, Esther; Adolph, Karen E.

    1992-01-01

    Examines the impact of Arnold L. Gesell on developmental psychology. Gesell is best remembered for his developmental norms, acquired from observations of infants and children. Gesell's ideas about maturation have lost favor, but his belief in infants' native abilities is still a dominant theme in theories. (BC)

  6. Stability of planar multifluid plasma equilibria by Arnold's method

    SciTech Connect

    Holm, D.D.

    1983-01-01

    A method developed by Arnold to prove nonlinear stability of certain steady states for ideal incompressible flow in two dimensions is extended to the case of barotropic, compressible, multifluid plasmas. This extension is accomplished by constructing conserved functionals derived from degeneracy of Poisson brackets. The results are applied to planar shear flows of the plasma.

  7. 15. Historic American Buildings Survey, Arnold Moses, Photographer December 1, ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    15. Historic American Buildings Survey, Arnold Moses, Photographer December 1, 1936, VIEW LOOKING SOUTH FROM THIRD FLOOR OF 29 1/2 CHERRY STREET SHOWING ROOF LINE OF BRICK FILLED TIMBER HOUSE RECENTLY DEMOLISHED. - John Beekman House, 29-29 1/2 Cherry Street, New York County, NY

  8. Pilot-scale in situ vitrification at Arnold Engineering Development Center, Arnold AFB, TN

    SciTech Connect

    Lominac, J.K.; Edwards, R.C. ); Timmerman, C.L. )

    1989-11-01

    The Department of Defense has the Installation Restoration Program (IRP) to identify and permanently remediate hazardous material disposal sites at its military bases across the United States. Pursuant to this guidance, Arnold Engineering Development Center (AEDC) selected In Situ Vitrification (ISV) to remediate an old fire training area, Fire Protection Training Area (FPTA) No. 2. The ISV technology was developed by Pacific Northwest Laboratory, Richland, WA, for the US Department of Energy (DOE) and will allow for the destruction and encapsulation of the petroleum-oil-lubricants (POL) and heavy metal-constituents found at the FPTA and adjacent overflow pond. ISV operates by passing a measured current of electricity into the ground through a set of electrodes. The resulting heat causes the soil to melt and form a solid vitreous (glass) mass similar to naturally occurring obsidian or basalt. In the process, organic constituents will be pyrolyzed (changed by heat) by the ensuing heat whereas the non-organic material will be incorporated into the glass matrix. 2 refs., 9 figs.

  9. Interventional Radiology in the Management of Budd Chiari Syndrome

    SciTech Connect

    Beckett, David; Olliff, Simon

    2008-09-15

    Budd Chiari syndrome is an uncommon condition in the Western world but interventional radiology can contribute significantly to the management of the majority of patients. This review examines the role and technique of interventions including hepatic vein dilatation and stent insertion as well as thrombolysis and TIPS. Liver transplantation and surgical shunt surgery are discussed in relation to radiological interventions. With appropriate selection and technique, surgery is only required in a minority of patients.

  10. Difusión de Arnold en un modelo simple

    NASA Astrophysics Data System (ADS)

    Cincotta, P. M.; Nuñez, J. A.; Miloni, O.

    Investigadores como Merritt, Valluri, Pfenniger o Contopoulos (por citar los más relevantes) consideran seriamente la hipótesis que la difusión de Arnold juega un rol importante en la evolución dinámica de los sistemas estelares y planetarios. Sin embargo no existe ninguna evidencia. El mayor problema para investigar fenómenos como éste es que uno debe estudiar numéricamente sistemas multidimensionales (más de dos grados de libertad) y visualizar de alguna manera el espacio de fases (más de cuatro dimensiones). Más complicado aún es el caso de difusión de Arnold, donde deben considerarse tiempos de movimiento extremadamente largos y tratar con parámetros exponencialmente pequeños. El propósito de este trabajo es estudiar, en un modelo simple 3D, la existencia o no de difusión de Arnold mediante experimentos numéricos y estimaciones analíticas. Siguiendo los trabajos de Cincotta (2000) y Cincotta, Nuñez y Simo (2000), aquí se pretende ``visualizar" la difusión y, a la vez, determinar la escala de tiempo en la que ésta se podría manifestar. Este trabajo es el escalón inicial para luego abordar este problema en modelos.

  11. PEOPLE IN PHYSICS: Interview with Sir Arnold Wolfendale FRS

    NASA Astrophysics Data System (ADS)

    Wiltsher, Conducted by Nicholas

    1997-11-01

    Sir Arnold Wolfendale was born on 25 June 1927, the son of Arnold and Doris Wolfendale. His BSc in Physics with First Class honours from the University of Manchester in 1948 was followed by a PhD in 1953 and a DSc in 1970. He was elected a Fellow of the Royal Society in 1977, and of the Royal Astronomical Society in 1973. In 1951 he married Audrey Darby, and they have twin sons. Sir Arnold's career has included lecturing posts at the Universities of Manchester, Durham, Ceylon and Hong Kong, and he was head of department at Durham. He retired from teaching in 1992 and was knighted in 1995. From 1991 to 1995 he was Astronomer Royal. Since 1996 he has been Professor of Experimental Physics with the Royal Institution of Great Britain. He has given lectures in many countries and in many places, and has had several books published on the subject of cosmic rays and astrophysics. He lives in Durham.

  12. Pulmonary vascular malformations.

    PubMed

    Liechty, Kenneth W; Flake, Alan W

    2008-02-01

    Pulmonary vascular malformations have historically been diagnosed in a wide range of age groups, but the extensive use of prenatal imaging studies has resulted in the majority of lesions being diagnosed in utero. Among this group of lesions, bronchopulmonary sequestrations (BPS), hybrid lesions with both congenital cystic adenomatoid malformation (CCAM) and BPS, aberrant systemic vascular anastomoses, and pulmonary arteriovenous malformations (PAVM), are the most common. The biologic behavior of these lesions and the subsequent therapy is, in large part, determined by the age of the patient at diagnosis. In the fetus, large BPS or hybrid lesions can result in fetal hydrops and in utero fetal demise. In the perinatal period, pulmonary hypoplasia from the mass effect or air trapping within the cystic component of hybrid lesions can result in life-threatening respiratory distress. In the postnatal period, communication of the lesion with the aero-digestive system can result in recurrent pneumonia. Alternatively, increased pulmonary blood flow from the systemic arterial supply can result in hemorrhage, hemoptysis, or high output cardiac failure. In addition, there have been several reports of malignant degeneration. Finally, the broad spectrum encompassed by these lesions makes classification and subsequent communication of the lesions confusing and difficult. This paper will review the components of these lesions, their associated anomalies, the diagnosis and natural history, and finally, current concepts in the management of pulmonary vascular malformations. PMID:18158137

  13. Etiologies of uterine malformations.

    PubMed

    Jacquinet, Adeline; Millar, Debra; Lehman, Anna

    2016-08-01

    Ranging from aplastic uterus (including Mayer-Rokitansky-Kuster-Hauser syndrome) to incomplete septate uterus, uterine malformations as a group are relatively frequent in the general population. Specific causes remain largely unknown. Although most occurrences ostensibly seem sporadic, familial recurrences have been observed, which strongly implicate genetic factors. Through the study of animal models, human syndromes, and structural chromosomal variation, several candidate genes have been proposed and subsequently tested with targeted methods in series of individuals with isolated, non-isolated, or syndromic uterine malformations. To date, a few genes have garnered strong evidence of causality, mainly in syndromic presentations (HNF1B, WNT4, WNT7A, HOXA13). Sequencing of candidate genes in series of individuals with isolated uterine abnormalities has been able to suggest an association for several genes, but confirmation of a strong causative effect is still lacking for the majority of them. We review the current state of knowledge about the developmental origins of uterine malformations, with a focus on the genetic variants that have been implicated or associated with these conditions in humans, and we discuss potential reasons for the high rate of negative results. The evidence for various environmental and epigenetic factors is also reviewed. © 2016 Wiley Periodicals, Inc. PMID:27273803

  14. Diagnosis and Management of Budd Chiari Syndrome: An Update

    SciTech Connect

    Copelan, Alexander; Remer, Erick M. Sands, Mark; Nghiem, Hanh; Kapoor, Baljendra

    2015-02-15

    Imaging plays a crucial role in the early detection and assessment of the extent of disease in Budd Chiari syndrome (BCS). Early diagnosis and intervention to mitigate hepatic congestion is vital to restoring hepatic function and alleviating portal hypertension. Interventional radiology serves a key role in the management of these patients. The interventionist should be knowledgeable of the clinical presentation as well as key imaging findings, which often dictate the approach to treatment. This article concisely reviews the etiology, pathophysiology, and clinical presentation of BCS and provides a detailed description of imaging and treatment options, particularly interventional management.

  15. Budd-Chiari syndrome management: Lights and shadows

    PubMed Central

    Mancuso, Andrea

    2011-01-01

    Budd-Chiari syndrome (BCS) is a rare disease whose management should follow a step by step strategy. Anticoagulation and medical therapy should be the first line treatment. Revascularization or TIPS are indicated in case of no response to medical therapy. OLT should be indicated as a rescue therapy and anticoagulation be started soon after OLT. However, no clear indication can actually be given about the timing of different treatments. Moreover, there is some concern about treatment of some subgroup of patients, especially regarding the risk of recurrence after liver transplantation. The topic of this paper is to critically review the actual knowledge of BCS management. PMID:22059108

  16. Research status of Budd-Chiari syndrome in China

    PubMed Central

    Dang, Xiaowei; Li, Luhao; Xu, Peiqin

    2014-01-01

    Budd-Chiari syndrome (B-CS) is a disease with a low incidence and has obvious geographical difference in subtype and clinical characteristics. The pathogenesis of B-CS in China is significantly different from that in western countries and is a complex process involving multiple factors. However, the specific cause of this disease is not yet clear. In-depth understanding of B-CS pathogenesis will be of great importance in preventing and treating the disease and improving the quality of life of the patients. PMID:25663961

  17. Budd-Chiari syndrome: an update on imaging features.

    PubMed

    Faraoun, Sid Ahmed; Boudjella, Mohamed El Amine; Debzi, Nabil; Benidir, Naima; Afredj, Nawel; Guerrache, Youcef; Bentabak, Kamel; Soyer, Philippe; Bendib, Salah Eddine

    2016-01-01

    Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver failure. This condition is characterized by an impaired hepatic venous drainage. The diagnosis of BCS is based on imaging, which helps initiate treatment. Imaging findings can be categorized into direct and indirect signs. Direct signs are the hallmarks of BCS and consist of visualization of obstructive lesions of the hepatic veins or the upper portion of the inferior vena cava. Indirect signs, which are secondary to venous obstruction, correspond to intra- and extrahepatic collateral circulation, perfusion abnormalities, dysmorphy and signs of portal hypertension. PMID:27317208

  18. The Presence of a Large Chiari Network in a Patient with Atrial Fibrillation and Stroke

    PubMed Central

    Schwimmer-Okike, Nneka; Niebuhr, Johannes; Schramek, Grit Gesine Ruth; Frantz, Stefan

    2016-01-01

    The Chiari network is an embryological remnant found in the right atrium, mostly without any significant pathophysiological consequences. However, several cardiac associations are reported in the literature including supraventricular tachyarrhythmias. We present a case of a 96-year-old body donor with a stroke episode and intermittent atrial fibrillations. The dissection of the heart revealed the presence of an immense Chiari network with a large central thrombus. The role of a Chiari network in the pathogenesis of stroke and pulmonary embolism is discussed. PMID:27547469

  19. Classification of Arnold-Beltrami flows and their hidden symmetries

    NASA Astrophysics Data System (ADS)

    Fré, P.; Sorin, A. S.

    2015-07-01

    In the context of mathematical hydrodynamics, we consider the group theory structure which underlies the so named ABC flows introduced by Beltrami, Arnold and Childress. Main reference points are Arnold's theorem stating that, for flows taking place on compact three manifolds ℳ3, the only velocity fields able to produce chaotic streamlines are those satisfying Beltrami equation and the modern topological conception of contact structures, each of which admits a representative contact one-form also satisfying Beltrami equation. We advocate that Beltrami equation is nothing else but the eigenstate equation for the first order Laplace-Beltrami operator ★ g d, which can be solved by using time-honored harmonic analysis. Taking for ℳ3, a torus T 3 constructed as ℝ3/Λ, where Λ is a crystallographic lattice, we present a general algorithm to construct solutions of the Beltrami equation which utilizes as main ingredient the orbits under the action of the point group B A of three-vectors in the momentum lattice *Λ. Inspired by the crystallographic construction of space groups, we introduce the new notion of a Universal Classifying Group which contains all space groups as proper subgroups. We show that the ★ g d eigenfunctions are naturally arranged into irreducible representations of and by means of a systematic use of the branching rules with respect to various possible subgroups we search and find Beltrami fields with non trivial hidden symmetries. In the case of the cubic lattice the point group is the proper octahedral group O24 and the Universal Classifying Group is a finite group G1536 of order |G1536| = 1536 which we study in full detail deriving all of its 37 irreducible representations and the associated character table. We show that the O24 orbits in the cubic lattice are arranged into 48 equivalence classes, the parameters of the corresponding Beltrami vector fields filling all the 37 irreducible representations of G1536. In this way we obtain an

  20. Fibrin sealant augmentation with autologous pericranium for duraplasty after suboccipital decompression in Chiari 1 patients: A case series

    PubMed Central

    Lam, Fred C.; Penumaka, Anirudh; Chen, Clark C.; Fischer, Edwin G.; Kasper, Ekkehard M.

    2013-01-01

    Background: The Chiari 1 malformation (CM1) involves decent of the tonsils of the cerebellum through the foramen magnum. Symptomatic disease requires a posterior fossa decompression with or without an expansile duraplasty. To date, the optimal surgical treatment for CM1 has not been delineated. The extent of bony removal, size of the dural opening, necessity for expansion of the dural space, choice of materials for the duraplasty, and possible need for augmentation with dural sealant are all factors that continue to be debated amongst neurological surgeons worldwide. We herein evaluate the use of fibrin sealant augmentation in combination with locally harvested autologous pericranium for duraplasty in adult CM1 decompression. Methods: Retrospective data collected from January 2006 to December 2011. Data were reviewed for surgical site infection or meningitis, cerebrospinal fluid leak, symptomatic pseudomeningocele, radiographic improvement of hindbrain compression, and postoperative recurrence of symptoms at a minimum of 1 year of follow-up. Outcomes were studied clinically, radiographically, as well as by using a patient-specific questionnaire. Results: Twenty-two consecutive patients were included. One patient required a revision for a delayed graft dehiscence in the setting of a rare form of aseptic meningitis with cerebrospinal fluid (CSF) pleocytosis due to a nonsteroidal anti-inflammatory drug (NSAID) allergy. All remaining patients had successful decompressions with full resolution of their symptoms except for one patient who had persistent headaches. Conclusion: Autologous pericranium with dural sealant augmentation is an effective technique for expansile duraplasty in CM1 decompressions. PMID:23493237

  1. An Awkward Echo: Matthew Arnold and John Dewey. Research in Curriculum and Instruction

    ERIC Educational Resources Information Center

    Dietz, Mark David

    2010-01-01

    Matthew Arnold, 19th century English poet, literary critic and school inspector, felt that each age had to determine that philosophy that was most adequate to its own concerns and contexts. This study looks at the influence that Matthew Arnold had on John Dewey and attempts to fashion a philosophy of education that is adequate for our own…

  2. Treatment of congenital malformations.

    PubMed

    Brucker, Sara Yvonne; Rall, Katharina; Campo, Rudi; Oppelt, Peter; Isaacson, Keith

    2011-03-01

    The prevalence of müllerian malformations is 1 in 200, or 0.5%. A third of the anomalies are septate, a third bicornuate uteri, 10% arcuate uterus, 10% didelphis and unicornuate uterus, and < 5% uterine and vaginal aplasia. Correct diagnosis of the malformation is most important but often very difficult. Correct treatment can only be performed if the malformation is clear. Longitudinal vaginal septums have to be removed due to potential obstetric problems. Transverse vaginal septums can cause hematocolpos and pain and have to be incised crosswise and excised so as not to shorten the vagina at the same time. Congenital vaginal agenesis occurs in Mayer-Rokitansky-Kuster-Hauser syndrome patients and in androgen insensitivity syndrome. The first choice for surgical treatment should be the new laparoscopic-assisted creation of a neovagina. Septate uterus has to be distinguished from a bicornuate uterus. Even if it is not proven to be a cause for infertility, the chance of miscarriage can be diminished by performing hysteroscopic metroplasty. Repair of a uterine septum in infertility patients often improves pregnancy rates. In contrast, surgical repair of a bicornuate uterus requires an abdominal metroplasty. This should only be performed if the patient has recurrent fetal loss due to the uterine structural defect. In a unicornuate uterus it is most important to determine if there is a second uterine horn that can cause cyclic pain if it has functioning endometrium. The only surgical option in these cases is to remove the rudimentary uterus with endometrium and hematometra, respectively. PMID:21437824

  3. Arteriovenous Malformation Management

    SciTech Connect

    Yakes, Wayne F.; Rossi, Plinio; Odink, Henk

    1996-11-15

    Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the initial noninvasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is performed under general anesthesia. Depending on the size of the lesion, additional Swan-Ganz line and arterial line monitoring are performed. Patients are usually observed overnight and uneventfully discharged the following day if no complication occurs. Patients are followed at periodic intervals despite cure of their lesion. Long-term follow-up is essential in AVM management.

  4. NFIA Haploinsufficiency Is Associated with a CNS Malformation Syndrome and Urinary Tract Defects

    PubMed Central

    Alkuraya, Fowzan S; Donovan, Diana J; Xi, Qiongchao; Turbe-Doan, Annick; Li, Qing-Gang; Campbell, Craig G; Shanske, Alan L; Sherr, Elliott H; Ahmad, Ayesha; Peters, Roxana; Rilliet, Benedict; Parvex, Paloma; Bassuk, Alexander G; Harris, David J; Ferguson, Heather; Kelly, Chantal; Walsh, Christopher A; Gronostajski, Richard M; Devriendt, Koenraad; Higgins, Anne; Ligon, Azra H; Quade, Bradley J; Morton, Cynthia C; Gusella, James F; Maas, Richard L

    2007-01-01

    Complex central nervous system (CNS) malformations frequently coexist with other developmental abnormalities, but whether the associated defects share a common genetic basis is often unclear. We describe five individuals who share phenotypically related CNS malformations and in some cases urinary tract defects, and also haploinsufficiency for the NFIA transcription factor gene due to chromosomal translocation or deletion. Two individuals have balanced translocations that disrupt NFIA. A third individual and two half-siblings in an unrelated family have interstitial microdeletions that include NFIA. All five individuals exhibit similar CNS malformations consisting of a thin, hypoplastic, or absent corpus callosum, and hydrocephalus or ventriculomegaly. The majority of these individuals also exhibit Chiari type I malformation, tethered spinal cord, and urinary tract defects that include vesicoureteral reflux. Other genes are also broken or deleted in all five individuals, and may contribute to the phenotype. However, the only common genetic defect is NFIA haploinsufficiency. In addition, previous analyses of Nfia−/− knockout mice indicate that Nfia deficiency also results in hydrocephalus and agenesis of the corpus callosum. Further investigation of the mouse Nfia+/− and Nfia−/− phenotypes now reveals that, at reduced penetrance, Nfia is also required in a dosage-sensitive manner for ureteral and renal development. Nfia is expressed in the developing ureter and metanephric mesenchyme, and Nfia+/− and Nfia−/− mice exhibit abnormalities of the ureteropelvic and ureterovesical junctions, as well as bifid and megaureter. Collectively, the mouse Nfia mutant phenotype and the common features among these five human cases indicate that NFIA haploinsufficiency contributes to a novel human CNS malformation syndrome that can also include ureteral and renal defects. PMID:17530927

  5. Chronic tonsillar herniation: an attempt at classifying chronic hernitations at the foramen magnum.

    PubMed

    Friede, R L; Roessmann, U

    1976-03-30

    A system is presented for the classification of chronic herniations of the cerebellar tonsils in the absence of space-occupying intracranial lesions, based on a survey of the literature and 13 own cases. The Arnold-Chiari malformation in adults typically involves herniation of the cerebellar tonsils instead of herniation of the vermis as is typical when it occurs in infancy. Identification of chronic tonsillar herniation with the Arnold-Chiari malformation in adults was thought to require at least one other sign of the Arnold-Chiari complex, e.g. a medullary deformity. Cases for which chronic herniation and sclerosis of the cerebellar tonsils present as the only nervous lesion are classified as "chronic tonsillar herniation". Such cases may manifest with neurological symptoms during adult life, or they may be found incidentally at autopsy. Attention is drawn to the occurrence of chronic tonsillar herniation in 7 infants and children where it apparently represents a cause of sudden unexpected death. The overlap between chronic tonsillar herniation and the Arnold-Chiari malformation of adults is discussed in regard to the frequence of associated osseous anomalies, hydrocephalus and syringomyelia. PMID:1266580

  6. Arnold Gesell's progressive vision: child hygiene, socialism and eugenics.

    PubMed

    Harris, Ben

    2011-08-01

    In October 1913, The American Magazine published an article by Arnold Gesell that portrayed Alma, Wisconsin (his hometown) as overflowing with the mentally and morally unfit. In "The Village of a Thousand Souls", Gesell called for the observation and segregation of the unfit as a eugenic measure. This article explores the reasons behind this infamous article by someone who became a famous developmental psychologist and pediatrician. Gesell's papers at the Library of Congress reveal his socialist views of poverty, injustice, and human development. The archives of his father's photography studio at the Wisconsin Historical Society reveal his manipulation of the photographic record to fit his negative view of Alma. Typical of the era, Gesell's Progressive vision combined social control and negative eugenics with egalitarianism and the benevolent engineering of the environment. PMID:21936236

  7. "Tangible as tissue": Arnold Gesell, infant behavior, and film analysis.

    PubMed

    Curtis, Scott

    2011-09-01

    From 1924 to 1948, developmental psychologist Arnold Gesell regularly used photographic and motion picture technologies to collect data on infant behavior. The film camera, he said, records behavior "in such coherent, authentic and measurable detail that ... the reaction patterns of infant and child become almost as tangible as tissue." This essay places his faith in the fidelity and tangibility of film, as well as his use of film as evidence, in the context of developmental psychology's professed need for legitimately scientific observational techniques. It also examines his use of these same films as educational material to promote his brand of scientific child rearing. But his analytic techniques - his methods of extracting data from the film frames - are the key to understanding the complex relationship between his theories of development and his chosen research technology. PMID:21995223

  8. Optical image encryption using multilevel Arnold transform and noninterferometric imaging

    NASA Astrophysics Data System (ADS)

    Chen, Wen; Chen, Xudong

    2011-11-01

    Information security has attracted much current attention due to the rapid development of modern technologies, such as computer and internet. We propose a novel method for optical image encryption using multilevel Arnold transform and rotatable-phase-mask noninterferometric imaging. An optical image encryption scheme is developed in the gyrator transform domain, and one phase-only mask (i.e., phase grating) is rotated and updated during image encryption. For the decryption, an iterative retrieval algorithm is proposed to extract high-quality plaintexts. Conventional encoding methods (such as digital holography) have been proven vulnerably to the attacks, and the proposed optical encoding scheme can effectively eliminate security deficiency and significantly enhance cryptosystem security. The proposed strategy based on the rotatable phase-only mask can provide a new alternative for data/image encryption in the noninterferometric imaging.

  9. Budd-Chiari syndrome: current perspectives and controversies.

    PubMed

    Liu, L; Qi, X-S; Zhao, Y; Chen, H; Meng, X-C; Han, G-H

    2016-07-01

    Budd-Chiari syndrome (BCS) is a rare disorder caused by hepatic venous outflow obstruction with a wide spectrum of etiologies. Clinical manifestations are so heterogeneous that the diagnosis should be considered in any patients with acute or chronic liver disease. Therapeutic modalities for BCS have improved dramatically during the last few years. The concept of a step-wise treatment strategy has been established, including anticoagulation, thrombolysis, percutaneous recanalization, transjugular intrahepatic portosystemic shunt, surgery and liver transplantation. However, this strategy is primarily based on experts' opinions and retrospective case series, rather than prospective randomized trials. Furthermore, an earlier use of TIPS has been proposed in selected cases because of a relatively high mortality from BCS patients who underwent medical therapy alone. Herein, we review the advances in the classification, etiology, clinical presentation, diagnosis and treatment of BCS. PMID:27467004

  10. Brain arteriovenous malformations.

    PubMed

    Ferrara, Adi R

    2011-01-01

    An arteriovenous malformation (AVM) is a particular abnormality of blood vessels. Brain AVMs are congenital, but symptoms usually do not appear until the second decade of life - if at all. The most common presenting symptom is a brain hemorrhage, but other possible symptoms include neurological deficits, seizures and headaches. Until recently, the gold standard for diagnosing AVM was conventional angiography. However, computed tomography and magnetic resonance angiography are now the first-line diagnostic tools for AVMs. This article reviews the presenting symptoms, diagnostic procedures and treatment options for brain AVMs, including embolization, micro-surgery and radiosurgery. This article is a Directed Reading. Your access to Directed Reading quizzes for continuing education credit is determined by your CE preference. For access to other quizzes, go to www.asrt.org/store. PMID:21771938

  11. Redefining Budd-Chiari syndrome: A systematic review

    PubMed Central

    Shin, Naomi; Kim, Young H; Xu, Hao; Shi, Hai-Bin; Zhang, Qing-Qiao; Colon Pons, Jean Paul; Kim, Ducksoo; Xu, Yi; Wu, Fei-Yun; Han, Samuel; Lee, Byung-Boong; Li, Lin-Sun

    2016-01-01

    AIM: To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same disorder. METHODS: A systematic review of observational studies conducted in adult subjects with primary BCS, hepatic vein outflow tract obstruction, membranous obstruction of the inferior vena cava (IVC), obliterative hepatocavopathy, or HVT during the period of January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed and Scopus. RESULTS: Of 1299 articles identified, 26 were included in this study. Classical BCS is more common in women with a pure hepatic vein obstruction (49%-74%). HVC-BCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization, with few managed with medical management alone. CONCLUSION: Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow. PMID:27326316

  12. Systemic to pulmonary vascular malformation.

    PubMed

    Pouwels, H M; Janevski, B K; Penn, O C; Sie, H T; ten Velde, G P

    1992-11-01

    A case is reported of life-threatening haemoptysis as a result of an anomalous communication between a bronchial artery and pulmonary vein, demonstrated by angiography. The patient recovered following bilobectomy of the right lower and middle lobes. When a systemic artery is involved in an arteriovenous malformation of the lung, haemodynamics are different compared with those present in malformations fed by the pulmonary artery. This implicates other clinical features, options for surgical intervention and prognosis. In reviewing the literature, a relationship with Rendu-Osler-Weber disease is absent in these specific malformations. PMID:1486979

  13. Potentiometric surfaces of the Arnold Engineering Development Complex Area, Arnold Air Force Base, Tennessee, May and September 2011

    USGS Publications Warehouse

    Haugh, Connor J.; Robinson, John A.

    2016-01-01

    During May 2011, when water levels were near seasonal highs, water-level data were collected from 374 monitoring wells; and during September 2011, when water levels were near seasonal lows, water-level data were collected from 376 monitoring wells. Potentiometric surfaces were mapped by contouring altitudes of water levels measured in wells completed in the shallow aquifer, the upper and lower parts of the Manchester aquifer, and the Fort Payne aquifer. Water levels are generally 2 to 14 feet lower in September compared to May. The potentiometric-surface maps for all aquifers indicate a groundwater depression at the J4 test cell. Similar groundwater depressions in the shallow and upper parts of the Manchester aquifer are within the main testing area at the Arnold Engineering Development Complex at dewatering facilities.

  14. Arteriovenous malformations of the bladder.

    PubMed

    Grunberger, I; DeAsis, A; Torno, R; Godec, C J

    1989-01-01

    We report a rare case of a localized arteriovenous malformation of the bladder mimicking a bladder tumor and presenting with gross hematuria. The mass was successfully resected transurethrally. PMID:2908934

  15. Embolization of uterine arteriovenous malformation

    PubMed Central

    Chen, Yan; Wang, Guoyun; Xie, Fubo; Wang, Bo; Tao, Guowei; Kong, Beihua

    2013-01-01

    Background: Uterine arteriovenous malformation is a rare but potential life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can be lead to massive hemorrhage. Case: We describe here a case of uterine arteriovenous malformation. A 32-year-old woman presented abnormal vaginal bleeding following the induced abortion. A diagnosis of uterine arteriovenous malformation made on the basis of Doppler ultrasonraphy was confirmed through pelvic angiography. The embolization of bilateral uterine arteries was performed successfully. Conclusion: Uterine arteriovenous malformation should be suspected in patient with abnormal vaginal bleeding, especially who had the past medical history incluing cesarean section, induced abortion, or Dillation and Curethage and so on. Although angiography remains the gold standard, Doppler ultrasonography is also a good noninvasive technique. The transcatheter uterine artery embolization offers a safe and effective treatment PMID:24639742

  16. Malformations of cortical development

    PubMed Central

    Pang, Trudy; Atefy, Ramin; Sheen, Volney

    2012-01-01

    Background Malformations of cortical development (MCD) are increasingly recognized as an important cause of epilepsy and developmental delay. MCD encompass a wide spectrum of disorders with various underlying genetic etiologies and clinical manifestations. High resolution imaging has dramatically improved our recognition of MCD. Review Summary This review will provide a brief overview of the stages of normal cortical development, including neuronal proliferation, neuroblast migration, and neuronal organization. Disruptions at various stages lead to characteristic MCD. Disorders of neurogenesis give rise to microcephaly (small brain) or macrocephaly (large brain). Disorders of early neuroblast migration give rise to periventricular heterotopia (neurons located along the ventricles), whereas abnormalities later in migration lead to lissencephaly (smooth brain) or subcortical band heterotopia (smooth brain with a band of heterotopic neurons under the cortex). Abnormal neuronal migration arrest give rise to over-migration of neurons in cobblestone lissencephaly. Lastly, disorders of neuronal organization cause polymicrogyria (abnormally small gyri and sulci). This review will also discuss the known genetic mutations and potential mechanisms that contribute to these syndromes. Conclusion Identification of various gene mutations has not only given us greater insight into some of the pathophysiologic basis of MCD, but also an understanding of the processes involved in normal cortical development. PMID:18469675

  17. Evidence of Holes in the Arnold Tongues of Flow Past Two Oscillating Cylinders

    NASA Astrophysics Data System (ADS)

    Papaioannou, Georgios V.; Yue, Dick K. P.; Triantafyllou, Michael S.; Karniadakis, George Em

    2006-01-01

    The wake of two oscillating cylinders in a tandem arrangement is a nonlinear system that displays Arnold tongues. We show by numerical simulations that their geometry depends on the phase difference θ between the two oscillating cylinders. At θ=0 there may be holes inside these intraresonance regions unlike the solid Arnold tongues encountered in single-cylinder oscillations. This implies that, surprisingly, self-excitation of the system may be suppressed inside these holes, at conditions close to its natural frequency.

  18. Plasma Propulsion Testing Capabilities at Arnold Engineering Development Center

    NASA Technical Reports Server (NTRS)

    Polzin, Kurt A.; Dawbarn, Albert; Moeller, Trevor

    2007-01-01

    This paper describes the results of a series of experiments aimed at quantifying the plasma propulsion testing capabilities of a 12-ft diameter vacuum facility (12V) at USAF-Arnold Engineering Development Center (AEDC). Vacuum is maintained in the 12V facility by cryogenic panels lining the interior of the chamber. The pumping capability of these panels was shown to be great enough to support plasma thrusters operating at input electrical power >20 kW. In addition, a series of plasma diagnostics inside the chamber allowed for measurement of plasma parameters at different spatial locations, providing information regarding the chamber's effect on the global plasma thruster flowfield. The plasma source used in this experiment was Hall thruster manufactured by Busek Co. The thruster was operated at up to 20 kW steady-state power in both a lower current and higher current mode. The vacuum level in the chamber never rose above 9 x 10(exp -6) torr during the course of testing. Langmuir probes, ion flux probes, and Faraday cups were used to quantify the plasma parameters in the chamber. We present the results of these measurements and estimates of pumping speed based on the background pressure level and thruster propellant mass flow rate.

  19. Pulmonary Arteriovenous Malformations

    PubMed Central

    2014-01-01

    Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ∼1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Paradoxical emboli lead to strokes and cerebral abscesses, and these commonly occur in individuals with previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of maternal death in pregnancy. PAVM occlusion by embolization is the standard of care to reduce these risks. However, recent data demonstrate that currently recommended management protocols can result in levels of radiation exposure that would be classified as harmful. Recent publications also provide a better appreciation of the hematologic and cardiovascular demands required to maintain arterial oxygen content and oxygen consumption in hypoxemic patients, identify patient subgroups at higher risk of complications, and emphasize the proportion of radiologically visible PAVMs too small to treat by embolization. This review, therefore, outlines medical states that exacerbate the consequences of PAVMs. Chief among these is iron deficiency, which is commonly present due to concurrent hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia compensations by restricting erythropoiesis and increases the risk of ischemic strokes. Management of periodontal disease, dental interventions, pulmonary hypertension, and pregnancy also requires specific consideration in the setting of PAVMs. The review concludes by discussing to what extent previously recommended protocols may benefit from modification or revision. PMID:25420112

  20. Pulmonary arteriovenous malformations.

    PubMed

    Shovlin, Claire L

    2014-12-01

    Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ~1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Paradoxical emboli lead to strokes and cerebral abscesses, and these commonly occur in individuals with previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of maternal death in pregnancy. PAVM occlusion by embolization is the standard of care to reduce these risks. However, recent data demonstrate that currently recommended management protocols can result in levels of radiation exposure that would be classified as harmful. Recent publications also provide a better appreciation of the hematologic and cardiovascular demands required to maintain arterial oxygen content and oxygen consumption in hypoxemic patients, identify patient subgroups at higher risk of complications, and emphasize the proportion of radiologically visible PAVMs too small to treat by embolization. This review, therefore, outlines medical states that exacerbate the consequences of PAVMs. Chief among these is iron deficiency, which is commonly present due to concurrent hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia compensations by restricting erythropoiesis and increases the risk of ischemic strokes. Management of periodontal disease, dental interventions, pulmonary hypertension, and pregnancy also requires specific consideration in the setting of PAVMs. The review concludes by discussing to what extent previously recommended protocols may benefit from modification or revision. PMID:25420112

  1. Tethered cord: natural history, surgical outcome and risk for Chiari malformation 1 (CM1): a review of 110 detethering.

    PubMed

    Valentini, Laura Grazia; Selvaggio, Giorgio; Visintini, Sergio; Erbetta, Alessandra; Scaioli, Vidmer; Solero, Carlo Lazzaro

    2011-12-01

    The surgical results of this series of occult spina bifida seem better than the natural history registered in the long pre-operative period in terms of neurological deterioration. The major contribution to this result is attributed to neurophysiological monitoring that lowers the risks of permanent damage and increases the percentage of effective detethering. The present series of TCS, due to conus and filar lipoma, documents that CM1 is a really rare association occurring in less than 6% of the patients, despite the low position of conus. The detethering procedure did not influence the tonsillar position, thus excluding the correlation between the tethering and the tonsillar descent. The genetic alteration documented in a girl reinforces the hypothesis of a rare complex polymaformative picture deserving multiple procedures according to the prevailing clinical symptoms. PMID:21922314

  2. Multiple liver lesions in a patient with Budd-Chiari syndrome secondary to polycythemia vera.

    PubMed

    Putra, Juan; Toor, Arifa; Noce, Todd A; Thung, Swan N; Suriawinata, Arief A; Lisovsky, Mikhail

    2015-01-01

    Focal nodular hyperplasia and nodular regenerative hyperplasia are occasionally seen in patients with hepatic venous outflow obstruction as a consequence of circulatory stress in the liver. In addition, neoplastic processes such as hepatic adenoma, hepatocellular carcinoma, and metastatic disease may arise in these patients. Histologic evaluation is necessary when imaging modalities are unable to distinguish these lesions. We present a case of multiple hepatic lesions, suspicious for metastases, in a patient with Budd-Chiari syndrome secondary to polycythemia vera. However, the biopsy findings were consistent with focal nodular hyperplasia. Budd-Chiari syndrome may be associated with multiple nodules of focal nodular hyperplasia, which may be difficult to diagnose radiologically. PMID:26019042

  3. 3-D simulation of posterior fossa reduction in Chiari I.

    PubMed

    Fernandes, Yvens Barbosa; Perestrelo, Pedro Fábio Mendonça; Noritomi, Pedro Yoshito; Mathias, Roger Neves; Silva, Jorge Vicente Lopes da; Joaquim, Andrei Fernandes

    2016-05-01

    We proposed a 3D model to evaluate the role of platybasia and clivus length in the development of Chiari I (CI). Using a computer aided design software, two DICOM files of a normal CT scan and MR were used to simulate different clivus lengths (CL) and also different basal angles (BA). The final posterior fossa volume (PFV) was obtained for each variation and the percentage of the volumetric change was acquired with the same method. The initial normal values of CL and BA were 35.65 mm and 112.66º respectively, with a total PFV of 209 ml. Ranging the CL from 34.65 to 29.65 - 24.65 - 19.65, there was a PFV decrease of 0.47% - 1.12% - 1.69%, respectively. Ranging the BA from 122.66º to 127.66º - 142.66º, the PFV decreased 0.69% - 3.23%, respectively. Our model highlights the importance of the basal angle and clivus length to the development of CI. PMID:27191237

  4. Results of portal systemic shunts in Budd-Chiari syndrome.

    PubMed Central

    Vons, C; Smadja, C; Bourstyn, E; Szekely, A M; Bonnet, P; Franco, D

    1986-01-01

    Nine patients with Budd-Chiari syndrome (BCS) were treated by a portal systemic shunt. One had thrombosis of the superior mesenteric vein (SMV) and another had complete obstruction of the retrohepatic inferior vena cava (IVC). All other patients had a marked stenosis of the retrohepatic IVC with caval pressure ranging from 12 to 24 mmHg (mean: 17 mmHg). Seven patients had an interposition mesocaval shunt using an autologous jugular vein. The patient with a thrombosed SMV had a portoatrial shunt. The patient with an obstructed IVC had a cavoatrial shunt after an erroneous portacaval shunt had failed to relieve ascites. There were no operative deaths and no major postoperative complications. One patient died 19 months after operation of acute leukemia complicating polycythemia rubra vera. All other patients were alive and well 8 months to 6 years after operation. None of them had encephalopathy. These results suggest several comments: Portal systemic shunts are a good treatment for BCS and have a low operative risk. The mesocaval shunt is an efficient procedure, even when there is stenosis of the IVC with high caval pressure; shunts to the right atrium should be performed only in the case of complete obstruction or inaccessibility of the IVC. The long-term prognosis is excellent, except in patients with potential malignancies. Therefore, portal systemic shunts should be indicated early in patients with symptomatic BCS. PMID:3963896

  5. Interventional therapeutic techniques in Budd-Chiari syndrome

    SciTech Connect

    Bilbao, Jose Ignacio; Pueyo, Jesus Ciro; Longo, Jesus Maria; Arias, Mercedes; Herrero, Jose Ignacio; Benito, Alberto; Barettino, Maria Dolores; Perotti, Juan Pablo; Pardo, Fernando

    1997-03-15

    Purpose. To analyze the results obtained with percutaneous therapeutic procedures in patients with Budd-Chiari syndrome (BCHS). Methods. Between August 1991 and April 1993, seven patients with BCHS were treated in our hospital. Three presented with a congenital web; in another three cases the hepatic veins and/or the inferior vena cava (IVC) were compromised after major hepatic surgery; one patient presented with a severe stenosis of the intrahepatic IVC due to hepatomegaly. Results. One of the patients with congenital web has required several new dilatations due to restenosis; one patient required a transjugular intrahepatic portosystemic shunt procedure while awaiting a liver transplantation. The two postsurgical patients with stenosed hepatic veins did not require any new procedure after the placement of metallic endoprostheses. However, the patient with liver transplantation presented IVC restenosis after balloon angioplasty that required the deployment of metallic endoprostheses. In the patient with hepatomegaly a self-expandable prosthesis was placed in the intrahepatic portion of the IVC before (4 months) a liver transplantation. Conclusion. Interventional therapeutic techniques offer a wide variety of possibilities for the treatment of patients with BCHS. For IVC stenoses, the results obtained with balloon angioplasty are at least as good as those obtained with surgery.

  6. Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

    PubMed Central

    Bacha, Dhouha; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; BelHadj, Najet

    2015-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties. PMID:26900444

  7. Why study human limb malformations?

    PubMed Central

    Wilkie, Andrew OM

    2003-01-01

    Congenital limb malformations occur in 1 in 500 to 1 in 1000 human live births and include both gross reduction defects and more subtle alterations in the number, length and anatomy of the digits. The major causes of limb malformations are abnormal genetic programming and intra-uterine disruption to development. The identification of causative gene mutations is important for genetic counselling and also provides insights into the mechanisms controlling limb development. This article illustrates some of the lessons learnt from the study of human limb malformation, organized into seven categories. These are: (1) identification of novel genes, (2) allelic mutation series, (3) pleiotropy, (4) qualitative or (5) quantitative differences between mouse and human development, (6) physical and teratogenic disruption, and (7) unusual biological phenomena. PMID:12587917

  8. Evolutionary origin of cardiac malformations.

    PubMed

    Taussig, H B

    1988-10-01

    The author has proposed in previous publications that isolated cardiac malformations have an evolutionary origin. This is partly supported by the fact that isolated cardiac malformations found in humans occur also in other placental mammals as well as in birds. External gross examination of the heart in just over 5,000 birds was carried out during a 3 year period. Anomalies included one instance of duplicate hearts, two specimens in which no heart could be identified and in a fourth, a yellow-rumped warbler, the heart lay in the neck outside of the thoracic cavity. Published reports of similar occurrences of an ectopically placed heart concern birds, cattle and humans. The fact that various species of both placental mammals and birds show evidence of heritability for heart defects, and that these species cannot interbreed, combined with the fact that birds and mammals have many similar malformations, points to either a common external causative factor or a common origin. Genes that code the malformed heart must be transmitted with that part of the genetic makeup common to all birds and mammals. Malformations caused by teratogens produce widespread organ injury to a potentially normal embryo whereas the evolutionary malformation is an organ-specific anomaly in an otherwise normal mammal or bird and occurs in widely separated species. The implications of this theory are important for parents of children with an isolated congenital heart defect who may have ingested one or another drug or chemical or have been exposed to toxins or infectious agents before or after conception of the affected offspring. PMID:3047192

  9. Imaging of congenital pulmonary malformations.

    PubMed

    Praticò, Francesco Emanuele; Corrado, Michele; Della Casa, Giovanni; Parziale, Raffaele; Russo, Giuseppe; Gazzani, Silvia Eleonora; Rossi, Enrica; Borgia, Daniele; Mostardi, Maurizio; Bacchini, Emanuele; Cella, Simone; De Filippo, Massimo

    2016-01-01

    Congenital pulmonary malformations represent a broad spectrum of anomalies that may result in varied clinical and pathologic pictures, ranging from recurrent pulmonary infections and acute respiratory distress syndrome, which require timely drug therapy, up to large space-occupying lesions needing surgical treatment. This classification includes three distinct anatomical and pathological entities, represented by Congenital Cystic Adenomatoid Malformation, Bronchopulmonary Sequestration and Congenital Lobar Emphysema. The final result in terms of embryological and fetal development of these alterations is a Congenital Lung Hypoplasia. Since even Bronchial Atresia, Pulmonary Bronchogenic Cysts and Congenital Diaphragmatic Hernias are due to Pulmonary Hypoplasia, these diseases will be discussed in this review (1, 2). PMID:27467867

  10. Genetics Home Reference: Dandy-Walker malformation

    MedlinePlus

    ... All Close All Description Dandy-Walker malformation affects brain development, primarily development of the cerebellum , which is the ... Walker malformation , signs and symptoms caused by abnormal brain development are present at birth or develop within the ...

  11. Intracranial vascular malformations: MR and CT imaging

    SciTech Connect

    Kucharczyk, W.; Lemme-Pleghos, L.; Uske, A.; Brant-Zawadzki, M.; Dooms, G.; Norman, D.

    1985-08-01

    Twenty-four patients with 29 cerebrovascular malformations were evaluated with a combination of computed tomography (CT), angiography, and magnetic resonance (MR) imaging. Characteristics of the malformations on MR images were reviewed retrospectively, and a comparative evaluation of MR and CT images was made. Of 14 angiographically evident malformations, 13 intra-axial lesions were detected on both CT and MR images, and one dural malformation gave false-negative results on both modalities. The appearance of parenchymal lesions on MR images closely mirrored characteristic CT findings. Angiographically evident malformations have a highly characteristic appearance on MR images. MR may be more sensitive than CT in the detection of small hemorrhagic foci associated with cryptic arteriovenous malformations and may add specificity in the diagnosis of occult malformations in some cases, but MR is less sensitive than CT for the detection of small calcified malformations.

  12. Arteriovenous malformation of nose-revision surgery.

    PubMed

    Srinivas, C V; Kailash, N; Kailas, Gayattre; Divya Jyothi, N

    2012-12-01

    Areteriovenous malformations are rare in the head and neck region and generally arise from intracranial vessels. We present one rare case with spontaneous arteriovenous malformations related to the nose. PMID:24294582

  13. Budd-Chiari Syndrome Complicating Hydatid Cyst of the Liver Managed by Venoplasty and Stenting

    SciTech Connect

    Sarawagi, Radha Keshava, Shyamkumar N. Surendrababu, Narayanam R. S.; Zachariah, Uday G. Eapen, Eapen C.

    2011-02-15

    Budd-Chiari syndrome (BCS) and portal hypertension is an uncommon complication of hydatid cyst of the liver. Previous reports describe cyst excision or portosystemic shunt surgery for such patients. Here we present a case of hydatid cyst of the liver with BCS that was treated successfully with hepatic venoplasty and transjugular stent placement.

  14. Stent Angioplasty of Closed Mesocaval Shunt in a Patient with Budd-Chiari Syndrome

    SciTech Connect

    Sancak, Tanzer; Karagulle, Ayse Tuba; Bilgic, Sadik; Sanlidilek, Umman; Yerdel, Mehmet Ali

    2002-08-15

    Budd-Chiari syndrome (BCS) is an uncommon disorder caused by hepatic venous outflow obstruction. It is characterized by ascites, hepatomegaly and abdominal pain. Percutaneous intervention shave recently been used for the treatment of BCS. We present a case of BCS with a closed mesocaval shunt which was reopened with a self-expandable metallic stent.

  15. Reconstruction of middle ear malformations

    PubMed Central

    Schwager, Konrad

    2008-01-01

    Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients. PMID:22073077

  16. Overgrowth syndromes with vascular malformations.

    PubMed

    Hagen, Solveig L; Hook, Kristen P

    2016-03-01

    This review provides a clinically-oriented summary of the most commonly encountered overgrowth syndromes associated with vascular malformations. This manuscript will outline morphologic features, clinical evaluation and management of this complex group of patients. Recent genetic advances have aided in classification and help to explain overlapping clinical features in many cases. PMID:27607325

  17. Origin of a leucogranitic gneiss (Gneiss Chiari) from Orobic Alps (N Italy)

    NASA Astrophysics Data System (ADS)

    Bergomi, M. A.; Boriani, A.

    2003-04-01

    The Orobic basement (Southern Alps) mainly consists of pelitic and psammitc metasiliciclastic rocks with minor metagranitoids. A peculiar orthogneiss occurs at the top of Southalpine basement near the contact with Permo-Mesozoic cover rocks: the Gneiss Chiari del Corno Stella (or Gneiss Chiari). The Gneiss Chiari show a very homogeneous chemical composition similar to that of tourmaline leucogranites. They show a restricted silica range (75-80 wt%), high Al2O3 (>13 wt%) and alkali contents. They are poor in CaO (<0.35 wt%), MgO (<0.3 wt%), Fe2O3t (< 0.60wt%) and TiO2 ("0.05 wt%). Several characteristic and discriminant trace elements, such as Rb, Ba, Sr, Y and Zr have been chosen to underline the exceptional chemical composition and homogeneity of the granitic protholith of Gneiss Chiari. Although K2O is only 20 to 30% higher than in normal biotite granites, Rb is enriched more than 10%, reaching even 500 ppm. Rb is probably related to muscovite as well as to Kfs. The Gneiss Chiari are also characterized by high K/Rb and Rb/Zr ratios, low Ba, Sr and HFSE. In the Qtz-Ab-Or system, the Gneiss Chiari plot very close to the minimum in the range between 0.5-1.0 Kb, suggesting that they may have crystallized from a near 100%-liquid magma under water-saturated conditions at very high crustal levels. The deviation of some samples away from the minimum is probably a result of fractionation and therefore some samples do not represent the true bulk composition of the original granitic melt. In this system liquidus temperatures of these compositions ranges between 750-700 oC. The same range is obtdined from Zr saturation temperatures ( Watson &Harrison, 1983). Fractionation is dominated by Kfs and the best evidence for crystal fractionation consist of: (1) enrichment in Rb, Cs and depletion in Ba, Sr and Eu; (2) Sr and Ba both increase; (3) Rb/Sr ratios increase with decreasing Ba; (4) TiO2, LREE, Th, Ba, Sr, Y and Ce/Y decrease with decreasing Zr, whereas Rb/Sr and Sr

  18. Galenic arteriovenous malformation with precocious puberty.

    PubMed

    Ventureyra, E C; Badejo, A

    1984-01-01

    Pineal lesions may appear with precocious puberty. In this report, a patient with precocious puberty and macrogenitosomia caused by an arteriovenous malformation in the pineal region is presented. This vascular malformation was not visualized during investigations 3 years before the present series. It appears that the vascular malformation increased considerably in size within a 3-year period. This case suggests that some arteriovenous malformations may take a malignant course, increasing rapidly in size and behaving like tumors by causing destruction and compression of surrounding structures. This case seems to be unique because, to the best of our knowledge, an arteriovenous malformation associated with precocious puberty has never been described previously. PMID:6689808

  19. Colon Perforation and Budd-Chiari Syndrome in Behçet’s Disease

    PubMed Central

    Baş, Yılmaz; Güney, Güven; Uzbay, Pınar; Zobacı, Ethem; Ardalı, Selin; Özkan, Ayşegül Taylan

    2015-01-01

    Patient: Female, 38 Final Diagnosis: Behçet’s disease Symptoms: Severe abdominal pain • fever Medication: — Clinical Procedure: Parsiyel colectomy Specialty: Surgery Objective: Unusual clinical course Background: Behçet’s disease is a chronic inflammatory disease involving multiple systems, with vasculitis being the most important pathological feature. Multiple colon perforations are thought to be secondary to vasculitis and they occur in patients with ulcers. These may be encountered within the entire colon but most commonly in the ileocecal region. Intestinal perforation and Budd-Chiari syndrome are infrequent in Behçet’s disease, and are associated with high mortality and morbidity. Budd-Chiari syndrome results from occlusion of either hepatic veins or adjacent inferior vena cava, or both. Case Report: We report a patient with Behçet’s disease having multiple perforations in the transverse colon, descending colon, and sigmoid colon. The patient also had Budd-Chiari syndrome due to inferior vena cava thrombosis extending into the right and middle hepatic vein. Our observations are presented with a review of the literature. Conclusions: In Behçet’s disease, treatment of colon perforation necessitates urgent surgery, whereas management of Budd-Chiari syndrome is directed towards the underlying cause. Behçet’s disease, as a chronic multisystemic disease with various forms of vasculitis, is resistant to medical and surgical treatment. Prognosis is worse in Behçet’s disease with colon perforation than that in Budd-Chiari syndrome alone. PMID:25934795

  20. 75 FR 6737 - FPL Energy Duane Arnold, LLC; Notice of Availability of the Draft Supplement 42 to the Generic...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-02-10

    ... alternative energy sources. The draft Supplement 42 to the GEIS is publicly available at the NRC Public... COMMISSION FPL Energy Duane Arnold, LLC; Notice of Availability of the Draft Supplement 42 to the Generic... of Duane Arnold Energy Center Notice is hereby given that the U.S. Nuclear Regulatory Commission...

  1. Intraneural Venous Malformations of the Median Nerve

    PubMed Central

    González Rodríguez, Alba; Midón Míguez, José

    2016-01-01

    Venous malformations arising from the peripheral nerve are a rare type of vascular malformation. We present the first case of an intraneural venous malformation of the median nerve to be reported in a child and review the previous two cases of median nerve compression due to a venous malformation that have been reported. These cases presented with painless masses in the volar aspect of the wrist or with symptoms suggestive of carpal tunnel syndrome. Clinical suspicion should lead to the use of Doppler ultrasonography as the first-line diagnostic tool. Magnetic resonance imaging and histopathology can confirm the diagnosis, as phleboliths are pathognomonic of venous malformations. Surgical treatment appears to be the only modality capable of successfully controlling the growth of an intraneural malformation. Sclerotherapy and radiotherapy have never been used to treat this type of malformation. PMID:27462571

  2. Congenital malformations in diabetic offspring.

    PubMed

    Temesio, P; Belitzky, R; Gallego, L; Martell, M; Pose, S V

    1977-01-01

    A retrospective study of 215 deliveries in diabetic mothers at Hospital de Clínicas (Montevideo, Uruguay) has been performed. Presence of congenital malformations (CM) was considered in relation to age of the mothers, class of maternal diabetes, maternal angiopathy, treatment and metabolic control. The prevalence of CM in the series was 9.8%. None of the factors analyzed seems to be statistically linked to the prevalence of CM. PMID:613685

  3. [Maternal imagination and congenital malformations].

    PubMed

    Van Heiningen, Teunis Willem

    2011-01-01

    Since antiquity philosophers and scientists tried to explain the cause of congenital malformations. In early modern medicine maternal imagination was largely accepted as their true cause, This concept was rejected by Blondel, a London physician. Around 1750 Wolff introduced the Hemmungsbildung as the cause of congenital malformations, a concept adopted in 1781 by Blumenbach. Later on Soemmerring (1784), Crichton (1785) and Meckel the younger adopted Blumenbach's concept. In 1824 Suringar further developed it. More and more the excessive development of fetal blood vessels or nerves was rejected as a possible cause, although from time to time these ideas were adopted again. In the early 1800s Etienne Geoffroy Saint-Hilaire (1811) and Vrolik (1817) developed a classification of monstra. These attempts urged Isidore Geoffroy Saint-Hilaire (Etienne's son) and Vrolik the younger (Gerard's son) to develop it further. Nevertheless, around 1840 Vrolik had to admit that although we are well acqainted with the various malformations, we are still ignorant of the primary cause of these phenomena. Meanwhile the dispute between the adherents of the theory of preformation and those who had adopted the concept of epigenesis exercised many minds. In the second half of the eighteenth century the latter theory became more and more adopted and this fact cleared the way for the ideas introduced by Wolff and Blumenbach, because it was consistent with the idea of a gradual development of fetal structures. PMID:22073754

  4. Arnold's Advantages: How Governor Schwarzenegger Acquired English through De Facto Bilingual Education

    ERIC Educational Resources Information Center

    Ramos, Francisco; Krashen, Stephen

    2013-01-01

    Governor Arnold Schwarzenegger has repeatedly mentioned that immigrants to the United States should do what he did to acquire English: Avoid using their first languages and speak, listen to, and read a vast amount of materials in English--a combination he referred to as "immersion." Yet, Schwarzenegger's real path to successful…

  5. Internal Evaluation a Quarter-Century Later: A Conversation with Arnold J. Love

    ERIC Educational Resources Information Center

    Volkov, Boris B.

    2011-01-01

    This chapter features a recent conversation with Dr. Arnold J. Love, a long-time proponent of internal evaluation and one of the most cited internal evaluation authors. In 1983, Love edited the first issue of "New Directions for Program Evaluation" on the topic of internal evaluation. He is the author of the book "Internal Evaluation: Building…

  6. Seedbed of Reform: Arnold Guyot and School Geography in Massachusetts, 1849-1855

    ERIC Educational Resources Information Center

    Koelsch, William A.

    2008-01-01

    Swiss-born Arnold Henri Guyot (1807-1884) was the first professionally trained geographer to hold an academic position in the United States. After his migration to this country in 1848 he lived for several years in Massachusetts. During this period he introduced contemporary German-Swiss ideas of geography to key opinion leaders in an important…

  7. Boston's Arnold Arboretum: A Place for Study and Recreation. Teaching with Historic Places.

    ERIC Educational Resources Information Center

    Banks, Alan

    This lesson is based on the National Register of Historic Places registration file for the Arnold Arboretum (Massachusetts) and other source material about the Arboretum and Frederick Law Olmstead. The lesson focuses on the first arboretum in the United States, which was part of Olmstead's plan for Boston's park system, known as the "Emerald…

  8. What Rural Education Research is of Most Worth? A Reply to Arnold, Newman, Gaddy, and Dean

    ERIC Educational Resources Information Center

    Howley, Craig B.; Theobald, Paul; Howley, Aimee

    2005-01-01

    Offering a response to the question, "What rural education research is of most worth?", the authors recommend an approach very different from the one taken by Arnold, Newman, Gaddy, and Dean (2005) in their consideration of the rural education research literature. They remind readers that about 150 years ago, Herbert Spencer put a similar…

  9. A Bold Perspective on Counseling with Couples and Families: An Interview with Arnold A. Lazarus.

    ERIC Educational Resources Information Center

    Christensen, Teresa M.

    2001-01-01

    Illuminates a collaborative effort between the author and Dr. Arnold A. Lazarus, an internationally known presenter, author, and therapist. Many consider Lazarus to be a pioneer of behavior therapy and brief psychotherapy. Presents details about his unique perspective regarding therapy with couples and families. Provides case examples that…

  10. Cryptic vascular malformations involving the brainstem

    SciTech Connect

    Yeates, A.; Enzmann, D.

    1983-01-01

    Six patients with angiographically cryptic vascular malformations involving the brainstem were examined with computed tomography (CT). The clinical and CT findings of cryptic vascular malformations of the brainstem are described and distinguished from those of brainstem glioma and multiple sclerosis. Calcification within a brainstem lesion that displays relatively little mass effect and shows little contrast enhancement, particularly when associated with a long history of waxing and waning brainstem symptoms, should suggest a vascular malformation.

  11. Accidental Entrapment of Electrical Mapping Catheter by Chiari's Network in Right Atrium during Catheter Ablation Procedure

    PubMed Central

    Sakamoto, Atsushi; Urushida, Tsuyoshi; Sakakibara, Tomoaki; Sano, Makoto; Suwa, Kenichiro; Saitoh, Takeji; Saotome, Masao; Katoh, Hideki; Satoh, Hiroshi; Hayashi, Hideharu

    2016-01-01

    A 78-year-old male was admitted to our hospital due to frequent palpitation. His electrocardiogram (ECG) presented regular narrow QRS tachycardia with 170 bpm, and catheter ablation was planned. During electroanatomical mapping of the right atrium (RA) with a multiloop mapping catheter, the catheter head was entrapped nearby the ostium of inferior vena cava. Rotation and traction of the catheter failed to detach the catheter head from the RA wall. Exfoliation of connective tissue twined around catheter tip by forceps, which were designed for endomyocardial biopsy, succeeded to retract and remove the catheter. Postprocedural echocardiography and pathologic examination proved the existence of Chiari's network. The handling of complex catheters in the RA has a potential risk of entrapment with Chiari's network. PMID:27366332

  12. Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

    PubMed Central

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm. PMID:26491527

  13. An embolus in the right atrium caught in the Chiari network and resistant to thrombolysis.

    PubMed

    Zuzana, Motovska; Petr, Widimsky; Dana, Bilkova; Martin, Penicka; Hana, Linkova; Dana, Kautznerova; Miroslav, Kolesar; Ludmila, Koldová; Jan, Kvasnicka

    2010-01-01

    This case report describes a patient with thromboemboli trapped in the Chiari network within the right side of the heart and resistant to thrombolysis. The right atrial masses were completely removed under cardiopulmonary bypass. Histological evaluation confirmed a mixed thromboemboli, with thrombus structures showing signs of organisation and surrounded by a fibrous capsule. The plasma level of the plasminogen activator inhibitor type-1 (PAI-1) was 50% higher than the normal upper limit. In this presented case, the Chiari network displayed a protective function but the expansion and organisation of the thromboembolus found there made it resistant to lytic treatment. Another important factor that could have influenced the resistance to thrombolysis was the high level of PAI-1. PMID:22791494

  14. Acute Budd-Chiari syndrome: Treatment with transjugular intrahepatic portosystemic shunt

    SciTech Connect

    Strunk, Holger M.; Textor, Jochen; Brensing, Karl-August; Schild, Hans H.

    1997-07-15

    The case of a 28-year-old man with acute Budd-Chiari syndrome due to veno-occlusive disease is reported. Transjugular intrahepatic portosystemic shunt (TIPS) was performed after upper gastrointestinal endoscopy, duplex sonographic and abdominal computed tomographic examination, inferior cavogram with hepatic venous catheterization, and transvenous biopsy. A 10-mm parenchymal tract was created. The patient did well after the procedure; ascites resolved and liver function improved markedly. The shunt has remained patent up to now for 6 months.

  15. Late Intrahepatic Hematoma Complicating Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome

    SciTech Connect

    Terreni, Natalia; Vangeli, Marcello; Raimondo, Maria Luisa; Tibballs, Jonathan M.; Patch, David; Burroughs, Andrew K.

    2007-09-15

    Late intrahepatic hematoma is a rare complication of the transjugular intrahepatic portosystemic shunt (TIPS) procedure. We describe a patient with Budd-Chiari syndrome (BCS), who presented with a large inrahepatic hematoma 13 days after TIPS. Review of the literature reveals only two previous cases, both occurring in patients with BCS and presenting after a similar time interval. This potentially serious complication appears to be specific for TIPS in BCS.

  16. Diffuse malformations of cortical development.

    PubMed

    Bahi-Buisson, Nadia; Guerrini, Renzo

    2013-01-01

    Malformations of cortical development (MCD) represent a major cause of developmental disabilities and severe epilepsy. Advances in imaging and genetics have improved the diagnosis and classification of these conditions. Up to now, eight genes have been involved in different types of MCD. Lissencephaly-pachygyria and subcortical band heterotopia (SBH) represent a malformative spectrum resulting from mutations of either LIS1 or DCX genes. LIS1 mutations cause a more severe malformation in the posterior brain regions. DCX mutations usually cause anteriorly predominant lissencephaly in males and SBH in female patients. Additional forms are X-linked lissencephaly with corpus callosum agenesis and ambiguous genitalia associated with mutations of the ARX gene. Lissencephaly with cerebellar hypoplasia (LCH) encompass heterogeneous disorders named LCH types a to d. LCHa is related to mutation in LIS1 or DCX, LCHb with mutation of the RELN gene, and LCHd could be related to the TUBA1A gene. Polymicrogyria encompasses a wide range of clinical, etiological, and histological findings. Among several syndromes, recessive bilateral fronto-parietal polymicrogyria has been associated with mutations of the GPR56 gene. Bilateral perisylvian polymicrogyria has been associated with mutations in the SRPX2 gene in a few individuals and with linkage to chromosome Xq28 in a some other families. X-linked bilateral periventricular nodular heterotopia (PNH) consists of PNH with focal epilepsy in females and prenatal lethality in males. Filamin A (FLNA) mutations have been reported in some families and in sporadic patients. It is possible to infer the most likely causative gene by brain imaging studies and other clinical findings. PMID:23622213

  17. Jupiter's winds and Arnol'd's second stability theorem: Slowly moving waves and neutral stability

    NASA Technical Reports Server (NTRS)

    Stamp, Andrew P.; Dowling, Timothy E.

    1993-01-01

    Since the Voyager encounters in 1979, it has been known that Jupiter's cloud-top zonal winds violate the barotropic stability criterion. A vortex-tube stretching analysis of the Voyager wind data indicates that the more general Charney-Stern stability criterion is also violated. On the other hand, the zonal winds determined by tracking cloud features in Hubble Space Telescope images taken in 1991 precisely match the zonal winds determined by tracking cloud features in Voyager images, and it is hard to understand how a complicated zonal wind profile like Jupiter's could be unstable and yet not change at all in 12 years. In fact, there are at least two unknown ways to violate the Charney-Stern stability criterion and still have a stable flow. The better known of these is called Fjortoft's theorem, or Arnol'd's 1st theorem for the case of large-amplitude perturbations. Although the Fjortoft-Arnol'd theorem has been extended from the quasi-geostrophic equations to the primitive equations, the basic requirement that the potential vorticity be an increasing function of streamfunction is opposite to the case found in Jupiter, where the Voyager data indicate that the potential vorticity is a decreasing function of streamfunction. But this second case is precisely that which is covered by Arnol'd's 2nd stability theorem. In fact, the Voyager data suggest that Jupiter's zonal winds are neutrally stable with respect to Arnol'd's 2nd stability theorem. Here, we analyze the linear stability problem of a one-parameter family of sinusoidal zonal wind profiles that are close to neutral stability with respect to Arnol'd's 2nd stability theorem. We find numerically that the most unstable mode is always stationary, which may help to explain the slowly moving mode 10 waves observed on Jupiter. We find that violation of Arnol'd's 2nd stability theorem is both necessary and sufficient for instability of sinusoidal profiles. However, there appears to be no simple extension of Arnol'd's 2

  18. Developmental venous anomaly, capillary telangiectasia, cavernous malformation, and arteriovenous malformation: spectrum of a common pathological entity?

    PubMed

    Kalani, M Yashar S; Zabramski, Joseph M; Martirosyan, Nikolay L; Spetzler, Robert F

    2016-03-01

    Vascular malformations of the central nervous system are thought to originate from abnormal developmental processes during embryogenesis. Reports have cited the dynamic nature of these lesions and their "maturation" into other types of malformations. Herein we report on three patients with vascular malformations who exhibited dynamic alterations with stepwise progression of their lesions. These cases lend support to the hypothesis that these malformations may constitute the spectrum of a single disease caused by alterations in a common developmental program and that accumulating injury (e.g., by radiation) may allow one malformation type to mature into another. This concept warrants further investigation. PMID:26743915

  19. Distant outcomes of the Chiari osteotomy 30 years follow up evaluation.

    PubMed

    Piontek, Tomasz; Szulc, Andrzej; Głowacki, Maciej; Strzyzewski, Wojciech

    2006-02-28

    Background. The aim of my paper was to assess distant treatment results of patients who were treated because of hip displasia and the Chiari osteotomy was performed on those patients. Material and methods. I evaluated treatment outcomes of 27 patients who were treated at Poznan University of Medical Sciences, Clinic of Orthopedics, in 1965-1990. The Chiari osteotomy was performed on all patients for hip decenteration or hip subluxation after developmental displasia. 34 hips were evaluated (20 female and 7 male). The average age of patients was 13 years in the moment of the operation. The follow up examination was conducted 10-36 years after the Chiari osteotomy Results. After many years (30 years after the operation on the average), 37% of patients could fit into groups of very good and good results. I could assess the hips before the operation using only parameters marked on anteroposterior films. The radiographs of the hips showed displasia characterized by shallowness and steepness of the acetabulum, an increased apparent neck-corpus angle, low submersion of the femoral head in the acetabulum, and insufficient coverage of the femoral head by the acetabulum. On the radiographs taken one year after the operation I observed very good coverage of the femoral head proved by higher values (above quota) of the Wiberg angle, the Sharp angle, the Heyman and Herndon acetabulum-head indicator. I defined also a course of the osteotomy drafting an osteotomy angle, setting a degree of bone fragments displacement (medialisation), and a height of the osteotomy. Conclusions. 1. The Chiari osteotomy as a hip saving operation allows patients to function in satisfactory clinical conditions for many years. 2. When conducted correctly, the Chiari osteotomy considerably improves femoral head coverage not only in the frontal plane but also in the transverse plane. 3. Too wide osteotomy angle, more than 20 degrees , further than 50% displacement of a distal bone fragment and too low

  20. Megadolicho vascular malformation of the intracranial arteries.

    PubMed

    Lodder, J; Janevski, B; van der Lugt, P J

    1981-01-01

    A patient is presented suffering a hemiparesis. Megadolicho-vascular malformation of the intracranial part of the internal carotid arteries and some of its branches and of the basilar artery was suggested by CT and confirmed by angiography. The value of CT compared with angiography in relation to intracranial megadolicho vascular malformations is discussed. PMID:6273040

  1. Translation of the V. I. Arnold paper "From Superpositions to KAM Theory" (Vladimir Igorevich Arnold. Selected — 60, Moscow: PHASIS, 1997, pp. 727-740)

    NASA Astrophysics Data System (ADS)

    Sevryuk, Mikhail B.

    2014-11-01

    V. I. Arnold (12 June 1937-3 June 2010) published several papers where he described, in the form of recollections, his two earliest research problems (superpositions of continuous functions and quasi-periodic motions in dynamical systems), the main results and their interrelations: [A1], then [A2] (reprinted as [A4, A6]), and [A3] (translated into English by the author as [A5]). The first exposition [A1] has never been translated into English; however, it contains many details absent in the subsequent articles. It seems therefore that publishing the English translation of the paper [A1] would not be superfluous. What follows is this translation. In many cases, the translator gives complete bibliographic descriptions of various papers mentioned briefly in the original Russian text. The English translations of papers in Russian are also pointed out where possible. A related material is contained also in Arnold's recollections "On A.N. Kolmogorov". Slightly different versions of these reminiscences were published several times in Russian and English [A7-A12]. The early history of KAM theory is also discussed in detail in the recent brilliant semi-popular book [A13].

  2. Minimal D = 7 supergravity and the supersymmetry of Arnold-Beltrami flux branes

    NASA Astrophysics Data System (ADS)

    Fré, P.; Grassi, P. A.; Ravera, L.; Trigiante, M.

    2016-06-01

    In this paper we study some properties of the newly found Arnold-Beltrami flux-brane solutions to the minimal D = 7 supergravity. To this end we first single out the appropriate Free Differential Algebra containing both a gauge 3-form B [3] and a gauge 2-form B [2]: then we present the complete rheonomic parametrization of all the generalized curvatures. This allows us to identify two-brane configurations with Arnold-Beltrami fluxes in the transverse space with exact solutions of supergravity and to analyze the Killing spinor equation in their background. We find that there is no preserved supersymmetry if there are no additional translational Killing vectors. Guided by this principle we explicitly construct Arnold-Beltrami flux two-branes that preserve 0, 1/8 and 1/4 of the original supersymmetry. Two-branes without fluxes are instead BPS states and preserve 1/2 supersymmetry. For each two-brane solution we carefully study its discrete symmetry that is always given by some appropriate crystallographic group Γ. Such symmetry groups Γ are transmitted to the D = 3 gauge theories on the brane world-volume that would occur in the gauge/gravity correspondence. Furthermore we illustrate the intriguing relation between gauge fluxes in two-brane solutions and hyperinstantons in D = 4 topological sigma-models.

  3. [Budd-Chiari syndrome and Behçet's disease. A case treated by mesenterico-atrial prosthesis].

    PubMed

    Le Treut, Y P; Comiti, Y; Bremondy, A; Magnan, P E; Raoult, D; Botta, D; Bricot, R

    1988-03-01

    A 36 year-old North African man, with Behçet's syndrome complicated by an inferior vena caval thrombosis, developed a chronic Budd-Chiari syndrome associated with bleeding esophageal varices. He was treated by an emergency mesoatrial shunt. Results at 2 years were good. Analysis of this case and the 13 other similar cases with associated Budd-Chiari syndrome and Behçet's syndrome found in the literature showed that hepatic veins thrombosis: a) is often due to inferior vena caval thrombosis or membranous obstruction; b) has a high spontaneous mortality rate by acute liver failure; c) remains a potential indication for porto-systemic shunt, as are other causes of Budd-Chiari syndrome. PMID:3286358

  4. Pathogenesis of Brain Arteriovenous Malformations

    PubMed Central

    KOMIYAMA, Masaki

    2016-01-01

    Brain arteriovenous malformations (bAVMs) represent a high risk of intracranial hemorrhages, which are substantial causes of morbidity and mortality of bAVMs, especially in children and young adults. Although a variety of factors leading to hemorrhages of bAVMs are investigated extensively, their pathogenesis is still not well elucidated. The author has reviewed the updated data of genetic aspects of bAVMs, especially focusing on clinical and experimental knowledge from hereditary hemorrhagic telangiectasia, which is the representative genetic disease presenting with bAVMs caused by loss-of-function in one of the two genes: endoglin and activin receptor-like kinase 1. This knowledge may allow us to infer the pathogensis of sporadic bAVMs and in the development of new medical therapies for them. PMID:27076383

  5. Split cord malformations: A two years experience at AIIMS

    PubMed Central

    Borkar, Sachin A.; Mahapatra, A. K.

    2012-01-01

    Background: Over a 2-year period, 2008-2009, a total of 53 cases of split cord malformation (SCM) were treated at the All India Institute of Medical Sciences (AIIMS). This study is a retrospective analysis of clinical features, radiological findings, and surgical outcome of these patients. Materials and Methods: During this period, 53 cases of SCM were treated at AIIMS. They constitute around 27% of all spinal dysraphism surgeries performed at the department of Neurosurgery, AIIMS; as 200 cases of spinal dysraphism were operated during the study period. The data was obtained from case files, operation notes, discharge summaries, and follow-up files. Observations: There were 30 cases of SCM type I and 23 cases of type II SCM. Seven patients were adult above 18 years of age. Except 7 patients, remaining 46 were symptomatic. Bony deformity of spine was recorded in 24 patients; of them, 19 had scoliosis and 4 had kyphosis. Deformity of foot was recorded in 10 patients. Thirteen patients had hypertrichosis, while four had dermal sinus. Magnetic resonance imaging (MRI) was performed in all patients. MRI revealed syringomyelia in 14 patients; however, only one patient had associated Chiari malformation. Six patients had meningomyelocele. Intra-operative; thick filum was noticed in 10 cases and in another 9 cases, there was filum lipoma. Dermoid was encountered in 4 patients, one patient had epidermoid tumor. Site of split was thoracic in 22, followed by lumbar region in 21 patients. Only 3 patients had split in cervical spinal cord. Seven patients had two separate splits at two different levels. Two patients had posteriorly located bony spur. All patients underwent surgery. Seven patients, those who had no neurological deficits pre-op, remained unchanged post-op. Amongst the 46 patients who had preoperative neurological deficits, eight had neurological deterioration post-op; five had deterioration in motor power and three had urinary problem. Five of these patients had

  6. Vascular Malformations: Approach by an Interventional Radiologist

    PubMed Central

    Pimpalwar, Sheena

    2014-01-01

    Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology. PMID:25045335

  7. Use of gallium-67 liver imaging for the early diagnosis of Budd-Chiari syndrome

    SciTech Connect

    Garty, I.; Horovitz, I.; Keynan, A.

    1984-03-01

    A gallium-67 study, performed in a rare case of Budd-Chiari syndrome (BCS) in a 6-yr-old child, showed marked concentration of the tracer in the left and caudate lobes of the liver. Ten days later, a less specific and clear-cut appearance of left- and caudate-lobe concentration of Tc-99m phytate was obtained. The possible use of Ga-67 citrate imaging to diagnose BCS in its earliest stage is suggested, although no firm conclusion can be made from this single case.

  8. Early diagnosis of Budd-Chiari syndrome by computed tomography and ultrasonography: report of five cases

    SciTech Connect

    Baert, A.L.; Fevery, J.; Marchal, G.; Goddeeris, P.; Wilms, G.; Ponette, E.; De Groote, J.

    1983-03-01

    In 5 patients with Budd-Chiari syndrome, computed tomography after intravenous bolus injection of iodinated contrast agents demonstrated images not previously seen in other diseases. The images are compatible with stagnation of contrast material at the periphery of the liver. In 3 of the 5 cases, grey-scale ultrasonography failed to document the normal hepatic veins draining into the inferior caval vein, but showed an intrahepatic network of comma-shaped venous structures. It is proposed that these two noninvasive approaches can help in establishing an early diagnosis.

  9. Update on the management of anorectal malformations.

    PubMed

    Bischoff, Andrea; Levitt, Marc A; Peña, Alberto

    2013-09-01

    Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a "Pandora's box" that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period. PMID:23913263

  10. The Microcephaly-Capillary Malformation Syndrome

    PubMed Central

    Mirzaa, Ghayda M.; Paciorkowski, Alex R.; Smyser, Christopher D.; Willing, Marcia C.; Lind, Anne C.; Dobyns, William B.

    2012-01-01

    We report on three children from two families with a new pattern recognition malformation syndrome consisting of severe congenital microcephaly (MIC), intractable epilepsy including infantile spasms, and generalized capillary malformations that was first reported recently in this journal [Carter et al. (2011); Am J Med Genet A 155: 301–306]. Two of our reported patients are an affected brother and sister, suggesting this is an autosomal recessive severe congenital MIC syndrome. PMID:21815250