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1

'Sneeze syncope', basilar invagination and Arnold-Chiari type I malformation  

Microsoft Academic Search

Syncope precipitated by sneezing in an adult male associated with an Arnold-Chiari type I malformation and basilar invagination presents a clinical problem in the differential diagnosis and pathological anatomy of Valsalva-related syncope. An abnormally acute clivoaxial angle, small foramen magnum, and type I Arnold-Chiari malformation appear to be a combination of features intolerant of Valsalva-induced changes in cerebral volume, brain-stem

J J Corbett; A B Butler; B Kaufman

1976-01-01

2

Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children.  

PubMed

Healthy children, aged 3, 9, and 13 years referred with sleep disordered breathing had marked central apnoea and bradypnoea on polysomnography, necessitating the use of non-invasive bilevel ventilation in two cases. Each had normal neurological examinations and an Arnold Chiari type 1 malformation was confirmed on magnetic resonance imaging. All underwent urgent posterior fossa decompression which normalised their sleep breathing. PMID:16551789

Murray, C; Seton, C; Prelog, K; Fitzgerald, D A

2006-04-01

3

Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children  

PubMed Central

Healthy children, aged 3, 9, and 13 years referred with sleep disordered breathing had marked central apnoea and bradypnoea on polysomnography, necessitating the use of non?invasive bilevel ventilation in two cases. Each had normal neurological examinations and an Arnold Chiari type 1 malformation was confirmed on magnetic resonance imaging. All underwent urgent posterior fossa decompression which normalised their sleep breathing.

Murray, C; Seton, C; Prelog, K; Fitzgerald, D A

2006-01-01

4

Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children  

Microsoft Academic Search

Healthy children, aged 3, 9, and 13 years referred with sleep disordered breathing had marked central apnoea and bradypnoea on polysomnography, necessitating the use of non-invasive bilevel ventilation in two cases. Each had normal neurological examinations and an Arnold Chiari type 1 malformation was confirmed on magnetic resonance imaging. All underwent urgent posterior fossa decompression which normalised their sleep breathing.

C Murray; C Seton; K Prelog; D A Fitzgerald

2006-01-01

5

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

ERIC Educational Resources Information Center

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…

Mittler, Joel E.

1986-01-01

6

Natural history of vocal fold paralysis in Arnold-Chiari malformation  

Microsoft Academic Search

Objective: Neurologic disease is the most common cause of bilateral vocal fold paralysis in children. Arnold-Chiari malformations (ACM) account for the majority. Early decompression results in the resolution of preoperative symptoms in the majority of patients. The purpose of present study is to describe the time to vocal fold function recovery following neurosurgical management of children with vocal fold paralysis

Ericka F. King; Marike Zwienenberg-Lee; Steve Maturo; Peter Siao Tick Chong; Christopher Hartnick; David J. Brown

7

Chiari Malformation  

MedlinePLUS

... signs and symptoms may not occur until late childhood or adulthood. The most common pediatric form, called ... I, signs and symptoms usually appear during late childhood or adulthood. Chiari malformation type II is usually ...

8

Chiari Malformation  

MedlinePLUS

... Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. ... of the skull is smaller than normal, the cerebellum and brain stem can be pushed downward. The ...

9

Arnold-Chiari Type I malformation: a look at two cases in the adult  

PubMed Central

This paper reviews the varied presentations of Arnold-Chiari Malformation (ACM) in terms of anatomy, clinical presentation, diagnostic workup, and therapeutic considerations. Emphasis is placed on the Type I condition as it appears in the adult. ACM is a developmental anomaly in which the cerebellar tonsils and portions of the posterior fossa structures herniate through the foramen magnum. It is frequently accompanied by a broad spectrum of additional anatomical variations and can present clinically in a variety of ways. Two cases of ACM Type I in the adult are presented. Both patients were treated initially by conservative measures before MRI could be obtained to establish the diagnosis. Neither one of these patients showed improvement with such a therapeutic regimen. It is important for practitioners to be aware of the varied presentations of ACM in the adult. ImagesFigure 1Figure 2Figure 3

McArthur, Ross A

1994-01-01

10

Foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patient  

Microsoft Academic Search

Study design: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression.Objective: To report this rare case along with a literature review.Setting: Gifu, Japan.Methods: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred to

I Takigami; K Miyamoto; H Kodama; H Hosoe; S Tanimoto; K Shimizu

2005-01-01

11

Chiari Malformation  

MedlinePLUS

... of the back of the head or the neck and contains brain matter. The covering of the brain or spinal ... fusion of segments of the bones in the neck, and extra folds in the brain. How common are Chiari malformations? In the past, ...

12

Sudden death revealing Chiari type 1 malformation in two children  

Microsoft Academic Search

We report the first two fatal cases of suddden unprovoked cardiorespiratory arrest in children with previously undiagnosed Arnold-Chiari type 1 malformation. This diagnosis should be considered in children with unexplained cardiorespiratory arrest. The risk of sudden death in Arnold-Chiari type 1 malformation reinforces the indication for early neurosurgical procedure.

A. Martinot; V. Hue; F. Leclerc; L. Vallee; M. Closset; J. P. Pruvo

1993-01-01

13

Type I Arnold-Chiari malformation with bronchiectasis, respiratory failure, and sleep disordered breathing: a case report  

PubMed Central

Arnold Chiari Malformation (ACM) is defined as a condition where part of the cerebellar tissue herniates into the cervical canal toward the medulla and spinal cord resulting in a number of clinical manifestations. Type I ACM consists of variable displacement of the medulla throughout the formamen magnum into the cervical canal, with prominent cerebellar herniation. Type I ACM is characterized by symptoms related to the compression of craniovertebral junction, including ataxia, dysphagia, nistagmus, headache, dizziness, and sleep disordered breathing. We report a case of a life-long non-smoker, 54 years old woman who presented these symptoms associated with bronchiectasis secondary to recurrent inhalation pneumonia, hypercapnic respiratory failure, and central sleep apnea (CSA). CSA was first unsuccessfully treated with nocturnal c-PAP. The subsequent treatment with low flow oxygen led to breathing pattern stabilization with resolution of CSA and related clinical symptoms during sleep. We suggest that in patients with type I ACM the presence of pulmonary manifestations aggravating other respiratory disturbances including sleep disordered breathing (SDB) should be actively investigated. The early diagnosis is desirable in order to avoid serious and/or poorly reversible damages.

2013-01-01

14

Anesthetic management during Cesarean section in a woman with residual Arnold-Chiari malformation Type I, cervical kyphosis, and syringomyelia  

PubMed Central

Background: Type I Arnold–Chiari malformation (ACM) has an adult onset and consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum. There is paucity of literature on the anesthetic management during pregnancy of residual ACM Type I, with cervical xyphosis and persistent syringomyelia. Case Description: A 34-year-old woman with surgically corrected ACM Type I presented for Cesarean delivery. A recent MRI demonstrated worsening of cervical xyphosis after several laminectomies and residual syringomyelia besides syringopleural shunt. Awake fiberoptic intubation was performed under generous topical anesthesia to minimize head and neck movement during endotracheal intubation. We used a multimodal general anesthesia without neuromuscular blockade. The neck was maintained in a neutral position. Following delivery, the patient completely recovered in post-anesthesia care unit (PACU), with no headache and no exacerbation or worsening of neurological function. Conclusions: The present case demonstrates that patients with partially corrected ACM, syringomyelia, cervical kyphosis, and difficult intubation undergoing Cesarean delivery require an interdisciplinary team approach, diligent preparation, and skilled physicians.

Ghaly, Ramsis F.; Candido, Kenneth D.; Sauer, Ruben; Knezevic, Nebojsa Nick

2012-01-01

15

The association of hypopituitarism with small pituitary, invisible pituitary stalk, type 1 Arnold-Chiari malformation, and syringomyelia in seven patients born in breech position: a further proof of birth injury theory on the pathogenesis of “idiopathic hypopituitarism”  

Microsoft Academic Search

We report seven cases of hypopituitarism all having a history of breech delivery, asphyxia at birth, and syringomyelia. A small pituitary gland was found on MRI or CT in six cases, invisible pituitary stalk on MRI in five cases, and type 1 Arnold-Chiari malformation in six cases. A constellation of these abnormalities are best explained by traction of brain and

K. Fujita; N. Matsuo; O. Mori; N. Koda; E. Mukai; Y. Okabe; N. Shirakawa; S. Tamai; Y. Itagane; I. Hibi

1992-01-01

16

Arnold-Chiari in a Fetal Rat Model of Dysraphism  

Microsoft Academic Search

Dysraphism is a defect in neural tube development, leading to dysplastic growth of the spinal cord and meninges. Myelomeningocele (MM) is just one of its forms. Hydrocephalus is among the most important alterations in MM and occurs as a consequence of Arnold-Chiari malformation (AC). Experimental models have been developed in sheep, rabbits and rats to study MM physiopathology, allowing a

Maria Weber Guimarăes Barreto; Marcelo M. Ferro; Daniel Bittencourt; Luis A. Violin Pereira; Ricardo Barini; Lourenço Sbragia

2005-01-01

17

Neuroradiological diagnosis of Chiari malformations  

Microsoft Academic Search

Chiari malformations 1, 2, 3 represent different degrees of herniation of posterior fossa content into the cervical canal\\u000a (Chiari 1 and 2), or through an upper-cervical meningocele (Chiari 3), whereas Chiari 4 anomaly consists of hypoplasia of\\u000a the cerebellum. Chiari 1 malformation (CM1) is the commonest anomaly; it is probably related to a mesodermal defect that create\\u000a a congenitally small

Luisa Chiapparini; Veronica Saletti; Carlo Lazzaro Solero; Maria Grazia Bruzzone; Laura Grazia Valentini

18

Reoperation for Chiari Malformations  

Microsoft Academic Search

Background: We undertook this study to characterize those patients who required reoperations for Chiari malformation and to determine whether modifications in surgical technique at the initial procedure might have obviated the need for repeat surgery. Methods: We reviewed the hospital records, imaging studies, operative reports, and follow-up data of those patients who were undergoing a second operation as part of

David Sacco; R. Michael Scott

2003-01-01

19

Novel cAMP binding protein-BP (CREBBP) mutation in a girl with Rubinstein-Taybi syndrome, GH deficiency, Arnold Chiari malformation and pituitary hypoplasia  

PubMed Central

Background Rubinstein-Taybi syndrome (RTS) is a rare autosomal dominant disorder (prevalence 1:125,000) characterised by broad thumbs and halluces, facial dysmorphism, psychomotor development delay, skeletal defects, abnormalities in the posterior fossa and short stature. The known genetic causes are point mutations or deletions of the cAMP-response element binding protein-BP (CREBBP) (50-60% of the cases) and of the homologous gene E1A-binding protein (EP300) (5%). Case presentation We describe, for the first time in literature, a RTS Caucasian girl, 14-year-old, with growth hormone (GH) deficiency, pituitary hypoplasia, Arnold Chiari malformation type 1, double syringomyelic cavity and a novel CREBBP mutation (c.3546insCC). Conclusion We hypothesize that CREBBP mutation we have identified in this patient could be responsible also for RTS atypical features as GH deficiency and pituitary hypoplasia.

2013-01-01

20

Chiari II Malformation and Syringomyelia  

Microsoft Academic Search

The Chiari II malformation was described at the end of the nineteenth century (1891–1896) by Hans Chiari, a German pathologist,\\u000a as a congenital malformation in a post-mortem examination of a child who died from a constellation of malformations including\\u000a prolapse of the cerebellum, part of the brain stem and part of the hindbrain, involving the upper part of the cervical

Spyros Sgouros

21

Controversies in Chiari I malformations  

PubMed Central

Background: The diagnosis and management of Chiari I malformations (CMI) remains controversial, particularly since it is often an incidental finding on cervical MR scans performed for neck pain and/or headaches. Recently, some surgeons “over-operated” on asymptomatic patients with Chiari I malformations, or even on those without the requisite radiographic diagnostic features for Chiari I malformations: unfortunately, only a subset were admonished for indiscriminate surgery. Nevertheless, when this hindbrain malformation is truly symptomatic, contributing to impaired cerebrospinal fluid (CSF) circulation, various valid surgical management strategies may be adopted. Methods: This review focuses on the current literature regarding the clinical presentation, diagnosis, and surgical management of Chiari I malformation. Variations in the surgical technique are also presented and critiqued. Results: The recommended treatment for Chiari I malformations I consists of decompressive suboccipital craniectomy and duroplasty when abnormal cine-flow MRI is observed preoperatively and blockage of CSF flow persists intraoperatively despite bony decompression. Conclusions: Controversy continues regarding the optimal surgical technique to address Chiari malformations I. Proper diagnostic studies and patient selection are needed to optimize patient outcomes, while avoiding unnecessary surgical procedures.

Baisden, Jamie

2012-01-01

22

The association of hypopituitarism with small pituitary, invisible pituitary stalk, type 1 Arnold-Chiari malformation, and syringomyelia in seven patients born in breech position: a further proof of birth injury theory on the pathogenesis of "idiopathic hypopituitarism".  

PubMed

We report seven cases of hypopituitarism all having a history of breech delivery, asphyxia at birth, and syringomyelia. A small pituitary gland was found on MRI or CT in six cases, invisible pituitary stalk on MRI in five cases, and type 1 Arnold-Chiari malformation in six cases. A constellation of these abnormalities are best explained by traction of brain and spinal cord of the subjects exerted during breech delivery and further support the primary role of birth trauma in the genesis of "idiopathic hypopituitarism". PMID:1499578

Fujita, K; Matsuo, N; Mori, O; Koda, N; Mukai, E; Okabe, Y; Shirakawa, N; Tamai, S; Itagane, Y; Hibi, I

1992-04-01

23

Radiological Evaluation of Myelomeningocele — Chiari II Malformation  

Microsoft Academic Search

Myelomeningocele (MMC) is a malformation characterized by the failure of closure of the neural tube, usually (but not only)\\u000a at the lumbo-sacral level. Synonyms are spina bifida aperta, open spinal dysra — phism, and Chiari II malformation complex.\\u000a MMC is typically associated with a metamerically consistent paraplegia, a posterior fossa deformity known as the Chiari II\\u000a malformation, hydrocephalus, and a

Charles Raybaud; Elka Miller

24

Chiari malformation with thick occipital bone.  

PubMed

A case of a Chiari malformation with an extraordinarily thick occipital bone is described. The thick occipital bone might make the posterior fossa narrow with consequent herniation of the cerebellar tonsils to the foramen magnum and formation of a syrinx. At dural plasty, well-developed marginal and occipital sinuses should be deliberately handled with the preservation of normal venous drainage. This case gives us the essence of the occurrence mechanisms of Chiari malformation and foramen magnum decompression. PMID:21339798

Yasuhara, Takao; Miyoshi, Yasuyuki; Date, Isao

2011-02-01

25

The Chiari II malformation: cause and impact  

Microsoft Academic Search

IntroductionIt is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida.DiscussionThe cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed. An open neural tube defect allows fluid to

David G. McLone; Mark S. Dias

2003-01-01

26

Chiari malformation in female monozygotic twins.  

PubMed

We describe the cases of female monozygotic twins who presented almost synchronously with symptomatic Chiari malformation type I. Both were successfully treated with foramen magnum decompression. We analyse these findings in the context of previously reported cases and discuss the genetic implications. PMID:20649405

Solth, A; Barrett, C; Holliman, D; Mitchell, P

2010-10-01

27

Anatomical progression of the Chiari II malformation  

Microsoft Academic Search

To evaluate whether anatomic change of the relationship of the Chiari II malformation and the cranial base was occurring, 22 children with meningomyelocele had serial MRI scans reviewed. A ratio (B\\/A) was established between the distance from the foramen magnum to the caudalmost portion of herniated cerebellum (B) and the diameter of the foramen magnum (A) and this ratio was

John R. Ruge; Jeff Masciopinto; Bruce B. Storrs; David G. McLone

1992-01-01

28

Cerebral Anomalies and Chiari Type 1 Malformation  

Microsoft Academic Search

Objective: To analyze the association of diverse cerebral anomalies in a series of pediatric patients with cerebellar tonsillar ectopia. Methods: We reviewed the medical records of 60 children diagnosed with Chiari type 1 malformation (CM1), of these, 20 patients (11 boys and 9 girls; mean age 7.2 years, range 2–16 years) had an associated cerebral anomaly. Symptoms of tonsillar ectopia

Marcelo Galarza; Juan F. Martínez-Lage; Steven Ham; Sandeep Sood

2010-01-01

29

CSF flow study in Chiari I malformation  

Microsoft Academic Search

ObjectiveThe aim of this prospective study was to define the role of cardiac gated phase-contrast ciné magnetic resonance imaging in deciding the therapeutic strategy in patients with Chiari I malformation.Materials and methodsTwenty-one patients operated on between February 2000 and July 2002 were enrolled in the study. All patients underwent a detailed preoperative neurological examination. MRI of the craniovertebral junction and

M. Panigrahi; B. Praveen Reddy; A. K. Reddy; J. J. M. Reddy

2004-01-01

30

The surgical treatment of Chiari I malformation  

Microsoft Academic Search

Summary A retrospective study was undertaken on 133 patients with a Chiari I malformation treated within the last 16 years at the Departments of Neurosurgery at the Nordstadt Hospital Hannover, Germany, and the University of California, Los Angeles, U.S.A. Ninety-seven patients presented with symptoms related to accompanying syringomyelia and 4 with associated syringobulbia. They underwent 149 surgical procedures and were

J. Klekamp; U. Batzdorf; M. Samii; H. W. Bothe

1996-01-01

31

The pediatric Chiari I malformation: a review  

Microsoft Academic Search

Background  Both the diagnosis and treatment regimens for the Chiari I malformation (CIM) are varied and controversial. The present paper\\u000a analyzes the literature regarding this form of hindbrain herniation in regard to definition, anatomy, pathobiology, symptoms,\\u000a findings, treatment, and outcomes.\\u000a \\u000a \\u000a \\u000a Discussions  Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome,\\u000a and treatment for

R. Shane Tubbs; Michael J. Lyerly; Marios Loukas; Mohammadali M. Shoja; W. Jerry Oakes

2007-01-01

32

Craniofrontonasal dysplasia associated with Chiari malformation.  

PubMed

Craniofrontonasal dysplasia (CFND) is a rare developmental anomaly associated with an X-linked inheritance. It is predominantly expressed in females. A Chiari malformation (CM) has not been reported in such patients earlier. The authors report on a family with 3 female members who have marked and generalized CFND. The generalized bone dysplasia/hypertrophy resulted in reduction in the posterior cranial fossa volume in all 3 patients, and in a CM associated with syringomyelia in 2 of them. One of the 2 affected family members who had a CM and syringomyelia was symptomatic and was treated by foramen magnum decompression surgery. The 3 family members had remarkable similarity in their external facial features and in their radiologically revealed morphological features. A review of the relevant literature, genetic abnormalities, and pattern of inheritance is presented. PMID:20367343

Mahore, Amit; Shah, Abhidha; Nadkarni, Trimurti; Goel, Atul

2010-04-01

33

Evolutionary hypothesis for Chiari type I malformation.  

PubMed

Chiari I malformation (CM-I) is classically defined as a cerebellar tonsillar herniation (?5mm) through the foramen magnum. A decreased posterior fossa volume, mainly due to basioccipital hypoplasia and sometimes platybasia, leads to posterior fossa overcrowding and consequently cerebellar herniation. Regardless of radiological findings, embryological genetic hypothesis or any other postulations, the real cause behind this malformation is yet not well-elucidated and remains largely unknown. The aim of this paper is to approach CM-I under a broader and new perspective, conjoining anthropology, genetics and neurosurgery, with special focus on the substantial changes that have occurred in the posterior cranial base through human evolution. Important evolutionary allometric changes occurred during brain expansion and genetics studies of human evolution demonstrated an unexpected high rate of gene flow interchange and possibly interbreeding during this process. Based upon this review we hypothesize that CM-I may be the result of an evolutionary anthropological imprint, caused by evolving species populations that eventually met each other and mingled in the last 1.7million years. PMID:23948602

Fernandes, Yvens Barbosa; Ramina, Ricardo; Campos-Herrera, Cynthia Resende; Borges, Guilherme

2013-08-12

34

Chiari-I malformation associated with asymmetric sensorineural hearing loss.  

PubMed

Chiari-I malformation is a deformity of the structures of the posterior fossa in which there is inferior herniation of the cerebellar tonsils through the foramen magnum without significant caudal dislocation of the brainstem. Patients are usually asymptomatic until adulthood, when they commonly present with recurrent headaches, weakness, vertigo and/or imbalance, nystagmus and hearing loss. A review of 226 consecutive patients evaluated for asymmetric sensorineural hearing loss revealed 32 patients with retrocochlear pathology. Three of these patients were discovered to have a Chiari-I malformation by magnetic resonance imaging as their only pathology. We suggest a possible association between a Chiari-I malformation and isolated asymmetric sensorineural hearing loss secondary to long-standing traction on the eighth cranial nerve. PMID:1583702

Hendrix, R A; Bacon, C K; Sclafani, A P

1992-04-01

35

Surgical Management of Chiari Malformation: Analysis of 128 Cases  

Microsoft Academic Search

Objective: A variety of surgical interventions have been recommended for patients with Chiari malformations (CMs). In this study, we have evaluated the intraoperative findings and clinical outcome in different-aged patients with CMs undergoing posterior fossa decompression. Methods: Sixteen pediatric and 112 adult cases with CMs underwent suboccipital craniectomy and wide duraplasty as well as autogenous bone grafting in selected cases.

Fuyou Guo; Meiyun Wang; Jiang Long; Huaili Wang; Hongwei Sun; Bo Yang; Laijun Song

2007-01-01

36

Clival dysgenesis associated with Chiari Type 1 malformation and syringomyelia  

Microsoft Academic Search

We report an 11-year-old boy with dysgenesis of the clivus. The boy presented complaining of progressive difficulty in walking. The clival anomaly was associated with severe basilar invagination, Chiari Type 1 malformation and syringomyelia. Our literature search did not reveal any previous reports of this combination of conditions. The patient obtained relief from his symptoms following a foramen magnum decompression.

Abhidha Shah; Atul Goel

2010-01-01

37

Presentation and Management of Chiari I Malformation in Children  

Microsoft Academic Search

To determine the efficacy of operative treatment for children with Chiari I malformation, the medical records and magnetic resonance imaging (MRI) studies of 68 consecutive patients cared for at The Children’s Hospital, Boston, Mass., USA, from December, 1988 to November, 1996 were retrospectively reviewed. All patients underwent suboccipital craniectomy, Cl laminectomy, and dural grafting. Bipolar coagulation to shrink and reduce

John K. Park; P. Langham Gleason; Joseph R. Madsen; Liliana C. Goumnerova; Michael Scott

1997-01-01

38

The Chiari II Malformation: Lesions Discovered within the Fourth Ventricle  

Microsoft Academic Search

Structural lesions associated with the Chiari II malformation have been identified within the fourth ventricle in 8 patients. During the 42-month period encompassing the operations of the 7 patients treated surgically, only 9 other patients were explored without the discovery of some associated structural lesion. The patients ranged in age from 2 to 26 years. The following lesions were identified:

Joseph H. Piatt Jr.; Anthony D’Agostino

1999-01-01

39

The Chiari Pseudotumor Cerebri Syndrome: Symptom Recurrence after Decompressive Surgery for Chiari Malformation Type I  

Microsoft Academic Search

Introduction: The etiology of Chiari malformation type I (CM1) as well as other anomalies associated with CM1 remains poorly defined. We have noted the presence of elevated CSF pressures with small ventricles, consistent with the pseudotumor cerebri (PTC) syndrome in a group of CM1 patients that did not respond over the long term to posterior fossa decompression. In order to

Lisa H. Fagan; Sherise Ferguson; Reza Yassari; David M. Frim

2006-01-01

40

[Chiari type I malformation in a patient with Poland's syndrome].  

PubMed

Chiari malformation type I and Poland's syndrome are two rare diseases and their simultaneous presentation had not been previously described in the literature. We report the case of a 27 year old male with history of Poland's syndrome, who referred headache and motor impairment of the intrinsic muscles of the left hand. In a cervical spine MR a Chiari I malformation with syringomyelia from C1 to T2 was found, which was treated by foramen magnum decompression, dural plasty and removal of the posterior arch of the atlas. A discussion of the embryological mechanisms that might be involved in the coexistence of these two entities is presented, emphasizing the role of para-axial mesoderm. PMID:22167290

Apellido, Nombre; Alcalá-Cerra, G; Sabogal-Barrios, R; Nińo-Hernández, L M; Gutiérrez-Paternina, J J; Moscote-Salazar, L R; Preciado-Mesa, E

2011-12-01

41

Clival dysgenesis associated with Chiari Type 1 malformation and syringomyelia.  

PubMed

We report an 11-year-old boy with dysgenesis of the clivus. The boy presented complaining of progressive difficulty in walking. The clival anomaly was associated with severe basilar invagination, Chiari Type 1 malformation and syringomyelia. Our literature search did not reveal any previous reports of this combination of conditions. The patient obtained relief from his symptoms following a foramen magnum decompression. PMID:20074957

Shah, Abhidha; Goel, Atul

2010-01-13

42

De novo Chiari-I Malformation in Infants Demonstrated by Sequential Magnetic Resonance Imaging Scans  

Microsoft Academic Search

In this report we demonstrate the de novo development of Chiari-I malformation with hydrosyringomyelia in 2 toddlers on serial brain magnetic resonance imaging scans. We speculate that a mismatch in bony and neural development is the underlying mechanism of these de novo Chiari malformations.

Sepideh Amin-Hanjani; Sumeer Sathi; Michael Scott

1995-01-01

43

Review Article: Chiari Type I Malformation with or Without Syringomyelia: Prevalence and Genetics  

Microsoft Academic Search

Chiari type I malformation has traditionally been defined as a downward herniation of the cerebellar tonsils of =5 mm through the foramen magnum and it is likely associated with a volumetrically reduced posterior fossa. Syringomyelia is commonly associated with Chiari type I malformation. We estimate the prevalence of these two conditions and determine that they are more common than previously

Marcy C. Speer; David S. Enterline; Lorraine Mehltretter; Preston Hammock; Judith Joseph; Margaret Dickerson; Richard G. Ellenbogen; Thomas H. Milhorat; Michael A. Hauser; Timothy M. George

2003-01-01

44

Pediatric syringomyelia with Chiari malformation: its clinical characteristics and surgical outcomes  

Microsoft Academic Search

BACKGROUNDMost reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects

Kazutoshi Hida; Yoshinobu Iwasaki; Izumi Koyanagi; Hiroshi Abe

1999-01-01

45

A late complication of CSF shunting: acquired Chiari I malformation  

Microsoft Academic Search

Purpose  Acquired Chiari I malformation developing after cerebrospinal fluid (CSF) shunting is an intriguing late complication of CSF\\u000a shunt surgery and not only raises questions as to its pathogenesis but also poses many queries about the possible adverse\\u000a effects on the subsequent child development as well as on the indications and possibilities of surgical correction.\\u000a \\u000a \\u000a \\u000a Materials and methods  We report a series

Massimo Caldarelli; Federica Novegno; Concezio Di Rocco

2009-01-01

46

Presentation of occult Chiari I malformation following spinal anesthesia  

PubMed Central

Chiari I malformation (CM-I) manifests with tonsillar herniation below foramen magnum. These patients are at high risk of respiratory depression and bulbar dysfunction in the perioperative period with underlying obstructive sleep apnea. However, the safe use of both general and regional anaesthesia has been documented in a known CM-I parturients. We describe the successful management of a patient who had hypercapnic respiratory failure in the post-anaesthetic care unit following an uneventful subarachnoid block for left knee replacement surgery. This patient was retrospectively diagnosed with occult CM-I and moderate to severe obstructive sleep apnea in the postoperative period.

Ankichetty, Saravanan P; Khunein, Saleh; Venkatraghavan, Lashmi

2012-01-01

47

Is Chiari type I malformation a reason for chronic daily headache?  

Microsoft Academic Search

This article briefly reviews the spectrum of headaches associated with Chiari type I malformation and specifically analyzes\\u000a current data on the possibility of this malformation as an etiology for some cases of chronic daily headache (CDH). Chiari\\u000a type I malformation is definitely associated with cough headache and not with primary episodic headaches, with the rare exception\\u000a of basilar migraine-like cases.

Carmińa Riveira; Julio Pascual

2007-01-01

48

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

Microsoft Academic Search

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

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

2009-01-01

49

Endoscopic third ventriculostomy in the management of Chiari I malformation and syringomyelia associated with hydrocephalus  

Microsoft Academic Search

BackgroundChiari malformation with syringomyelia is conventionally managed with foramen magnum decompression and duroplasty. In presence of hydrocephalus, a ventriculoperitoneal shunt insertion has been the initial preferred mode of treatment.

Aaron Mohanty; Ravi Suman; S. Ravi Shankar; Satyanarayana Satish; Shanti Shankar Praharaj

2005-01-01

50

Surgical Management of Patients with Chiari I Malformation  

PubMed Central

Chiari malformations (CMs) constitute a variety of four mainly syndromes (I, II, III, and IV), which describe the protrusion of brain tissue into the spinal canal through the foramen magnum. These malformations frequently occur in combination with other pathological entities such as myelomeningocele, hydrocephalus, and/or hydrosyringomyelia. The recent improvement of imaging techniques has increased not only the rate of CM diagnosis but also the necessity for its early treatment. Several different surgical techniques have been employed in the treatment of patients with symptomatic CM-I. In our current study, a systematic and critical review of the pertinent literature was made for identifying the most commonly employed surgical procedures in the management of these patients. Emphasis was given in outlining the advantages and disadvantages of each surgical approach. Moreover, an attempt was made for defining those parameters that may be prognostic factors for their surgical outcome. There is a consensus that surgical treatment is reserved only for symptomatic patients with CM-I. It has also been postulated that early surgically intervention is usually associated with better outcome. Despite the large number of previously published clinical series, further clinical research with large-scale studies is necessary for defining surgical treatment guidelines in these patients.

Siasios, John; Kapsalaki, Eftychia Z.; Fountas, Kostas N.

2012-01-01

51

Presentation and Management of Hydromyelia in Children with Chiari Type-II Malformation  

Microsoft Academic Search

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

Frank La Marca; Martin Herman; John A. Grant; David G. McLone

1997-01-01

52

Aggressive Surgical Management of Patients with Chiari II Malformation and Brainstem Dysfunction  

Microsoft Academic Search

Background\\/Aims: Chiari II malformation represents a group of developmental abnormalities involving the caudal displacement of the cervicomedullary junction, pons, fourth ventricle, and medulla. This constellation of malformations is strongly associated with myelomeningocele (MM) and is a known cause of neurologic deterioration in older MM patients. We describe the evaluation and management of 4 adult MM patients who presented with brainstem

Maryam Rahman; Leigh Ann Perkins; David W. Pincus

2009-01-01

53

Chiari I Malformation Associated with Syringomyelia and Scoliosis A Twenty-Year Review of Surgical and Nonsurgical Treatment in a Pediatric Population  

Microsoft Academic Search

Study Design. Retrospective review of patients with Chiari I malformation with or without associated scoliosis. Objectives. Determine the effect of decompression of Chiari I malformation with syringomyelia on stabilization or improvement of associated scoliosis. Summary of Background Data. Chiari malformations are often associated with spinal deformities, including scoliosis. Studies have suggested a causal relation be- tween syringomyelia and scoliosis. Methods.

James M. Eule; Mark A. Erickson; Michael F. O'Brien; Michael Handler

54

Treatment of delayed Chiari malformation and syringomyelia after lumboperitoneal shunt placement: Case report and treatment recommendations  

PubMed Central

Background: Delayed Chiari malformation with syringomyelia is an uncommon and sparsely reported complication after lumbar cisternal shunting. A number of treatments have been implemented with varying degrees of success. After review of these modalities, a treatment plan was devised and implemented. Case Description: A 15-year-old girl was diagnosed with idiopathic intracranial hypertension (pseudotumor cerebri) and had placement of a Medtronic Spetzler lumboperitoneal shunt. She did well for 10 years and then developed a 3-week history of headache, balance problems, and sensory loss. Workup demonstrated a new Chiari malformation and cervical syrinx. She underwent initial placement of a ventriculoperitoneal shunt, followed by ligation of the lumboperitoneal shunt. She then had a suboccipital decompression and duraplasty. Follow-up at 6 months showed relief of her symptoms and MRI demonstrated resolution of the Chiari malformation and syrinx. Conclusion: Delayed Chiari malformation and/or syringomyelia after lumbar shunting is best treated with placement of a ventricular device and ligation of the lumbar shunt. The Chiari malformation and/or syrinx should be surgically addressed for persistent symptoms or progression of the structural abnormality.

Peleggi, Analise F.; Lovely, Thomas J.

2012-01-01

55

MRI findings and sleep apnea in children with Chiari I malformation.  

PubMed

Chiari I malformation is characterized by downward herniation of the cerebellar tonsils through the foramen magnum. Scant data are available on the clinical course, relationship to the extent of herniation on magnetic resonance imaging in Chiari I malformation and the presence of sleep-disordered breathing on polysomnography. Retrospective analysis was performed looking at polysomnographic findings of children diagnosed with Chiari I malformation. Details on how Chiari I malformation was diagnosed, brainstem magnetic resonance imaging findings, and indications for obtaining the polysomnogram in these patients were reviewed. We also reviewed available data on children who had decompression surgery followed by postoperative polysomnography findings. Twenty-two children were identified in our study (11 males, median age 10 years, range 1 to 18). Three had central sleep apnea, five had obstructive sleep apnea, and one had both obstructive and central sleep apnea. Children with sleep-disordered breathing had excessive crowding of the brainstem structures at the foramen magnum and were more likely to have a greater length of herniation compared with those children without sleep-disordered breathing (P = 0.046). Patients with central sleep apneas received surgical decompression, and their conditions were significantly improved on follow-up polysomnography. These data suggest that imaging parameters may correlate with the presence of sleep-disordered breathing in children with Chiari I malformation. PMID:23498564

Khatwa, Umakanth; Ramgopal, Sriram; Mylavarapu, Alexander; Prabhu, Sanjay P; Smith, Edward; Proctor, Mark; Scott, Michael; Pai, Vidya; Zarowski, Marcin; Kothare, Sanjeev V

2013-04-01

56

Osteopetrosis with Chiari I malformation: presentation and surgical management.  

PubMed

Osteopetrosis is a heterogeneous group of rare, inherited disorders of the skeleton that results in neurological manifestations due to restriction of growth of cranial foramina and calvarial thickening. A 25-year-old woman with a history of autosomal dominant osteopetrosis presented to the authors' institution with headache worsened by exertion and radiating from the occipital region forward with episodes of choking/coughing when eating and a loss of gag reflex on physical examination. On MR imaging, she was found to have severe posterior fossa calvarial thickening resulting in a small posterior fossa and tonsillar ectopia of 9 mm and compression and deformation of the brainstem. She underwent posterior fossa craniectomy, foramen magnum decompression, and partial C-1 laminectomy with external durotomy. The patient did well postoperatively with resolution of symptoms. This case describes a new neurological manifestation of autosomal dominant osteopetrosis. To the authors' knowledge, this report represents the first described case of extreme posterior fossa calvarial thickening from autosomal dominant osteopetrosis with associated Chiari I malformation (CM-I) requiring posterior fossa decompression and extradural decompression. Given previously published MR imaging data that demonstrate the association of osteopetrosis and CM may be more common than in this case alone, the authors discuss the need for further investigation of the incidence of CM-I in patients with autosomal dominant osteopetrosis. Additionally, they review osteopetrosis and other diagnoses of calvarial hyperostosis presenting as CM-I. PMID:21456907

Dlouhy, Brian J; Menezes, Arnold H

2011-04-01

57

Chiari I malformation and acute acquired comitant esotropia: case report and review of the literature.  

PubMed

The authors describe a rare case of a Chiari I malformation presenting with acute acquired comitant esotropia (AACE) in a 5-year-old boy. A posterior fossa decompression with duraplasty and a C1-2 laminectomy were performed. There was an immediate postoperative improvement in the esotropia, which completely resolved by 7 months following surgery. The pertinent literature is discussed and reasons are presented for recommending posterior fossa decompression in certain patients, rather than strabismus surgery, as the initial treatment for esotropia. The authors suggest that in patients with AACE, even subtle symptoms and signs of Chiari I malformation should prompt imaging of the posterior fossa. Strong consideration should be given to performing posterior fossa decompression in patients with Chiari I malformation and AACE before strabismus surgery because the esotropia may completely resolve with decompression. PMID:15926393

Hentschel, Stephen J; Yen, Kimberly G; Lang, Frederick F

2005-05-01

58

Spontaneous resolution of Chiari type 1 malformation. A case report and literature review.  

PubMed

We describe a 62-year-old woman who presented with a history of ataxia, dizziness and urinary urgency. Neurological examination disclosed a positive Romberg sign, ataxia and postural instability. A magnetic resonance imaging (MRI) scan showed Chiari type 1 malformation (CM1). Forty-eight months later, the patient was clinically improved and underwent a second MRI examination, which showed complete resolution of the Chiari 1 malformation. Spontaneous resolution of CM1 is exceptionally rare and has to be considered in the radiological and clinical management. PMID:23859287

Briganti, F; Leone, G; Briganti, G; Orefice, G; Caranci, F; Maiuri, F

2013-07-16

59

Distribution of cerebellar tonsil position: implications for understanding Chiari malformation.  

PubMed

Object Prior attempts to define normal cerebellar tonsil position have been limited by small numbers of patients precluding analysis of normal distribution by age group. The authors' objective in the present study was to analyze cerebellar tonsil location in every age range. Methods Two thousand four hundred patients were randomly selected from a database of 62,533 consecutive patients undergoing MRI and were organized into 8 age groups. Magnetic resonance images were directly examined for tonsil location, morphology, and other features. Patients with a history or imaging findings of posterior fossa abnormalities unrelated to Chiari malformation (CM) were excluded from analysis. The caudal extent of the cerebellar tonsils was measured at the midsagittal and lowest parasagittal positions. Results The mean tonsil height decreased slightly with advancing age into young adulthood and increased with advancing age in the adult age range. An increasing age in the adult age range was associated with a decreased likelihood of a tonsil position 5 mm or more below the foramen magnum (p = 0.0004). In general, the lowest tonsil position in each age group was normally distributed. Patients with pegged morphology were more likely to have a tonsil location at least 5 mm below the foramen magnum (85%), as compared with those having intermediate (38%) or rounded (2%) morphology (p < 0.0001). Female sex was associated with a lower mean tonsil position (p < 0.0001). Patients with a lower tonsil position also tended to have an asymmetrical tonsil position, usually lower on the right (p < 0.0001). Conclusions Cerebellar tonsil position follows an essentially normal distribution and varies significantly by age. This finding has implications for advancing our understanding of CM. PMID:23767890

Smith, Brandon W; Strahle, Jennifer; Bapuraj, J Rajiv; Muraszko, Karin M; Garton, Hugh J L; Maher, Cormac O

2013-06-14

60

Surgical treatment of Chiari I malformation complicated with syringomyelia  

PubMed Central

The aim of this study was to evaluate the curative effects of various surgical procedures on Chiari I malformation (CMI) complicated with syringomyelia. A total of 185 patients with CMI complicated with syringomyelia who received treatment between January 1997 and December 2011 were recruited. All patients underwent posterior fossa decompression in which the lamina of the first cervical vertebra was removed, with the removal of the second or third depending on the severity of the cerebellar tonsil herniation. Of the patients, 76 underwent large-bone-window decompression and duraplasty, while 109 underwent small-bone-window decompression, displaced cerebellar tonsil resection and duraplasty. The curative effects of the different surgical procedures were analyzed retrospectively. Clinical symptoms were eliminated or improved in 156 patients (84.3%) by the time of discharge from hospital. A total of 148 patients were evaluated using magnetic resonance imaging (MRI) which revealed that the cisterna magna was reconstructed in 92 patients and spinal syrinx was reduced in 75. Follow-up was performed on 147 patients (79.5%) for between 3 months and 12 years. During the follow-up, symptoms were eliminated or improved in 110 patients (74.8%), not improved in 26 (17.7%) and deteriorated in 11 (7.5%). MRI was performed on 95 patients during follow-up examinations and the cisterna magna was reconstructed in 87 patients and spinal syrinx was reduced in 79. Small-bone-window decompression plus duraplasty is an effective surgical procedure for treating CMI complicated with syringomyelia and intraoperative cerebellar tonsillectomy significantly aids patient recovery.

BAO, CHANGSHUN; YANG, FUBING; LIU, LIANG; WANG, BING; LI, DINGJUN; GU, YINGJIANG; ZHANG, SHULING; CHEN, LIGANG

2013-01-01

61

Posterior cranial fossa dimensions in the Chiari I malformation: Relation to pathogenesis and clinical presentation  

Microsoft Academic Search

Skull dimensions were measured on lateral skull radiographs in 33 adult patients with MRI-verified Chiari I malformations and in 40 controls. The posterior cranial fossa was significantly smaller and shallower in patients than in controls. In the patients, there was a positive correlation between posterior fossa size and the degree of the cerebellar ectopia, which might indicate that a posterior

L. J. Stovner; U. Bergan; G. Nilsen; O. Sjaastad

1993-01-01

62

Three Sisters with Chiari I Malformation with and without Associated Syringomyelia  

Microsoft Academic Search

Two daughters of non-consanguineous Ashkenazi Jewish parentage presented with occipital headaches in the second decade of life. Each had a symptomatic Chiari I malformation (CMI) and a large cervicothoracic syrinx. A third sister was diagnosed as having CMI without syrinx after MR screening of first-degree relatives. A fourth (the eldest) sister was asymptomatic and did not have CMI or syrinx.

James D. Weisfeld-Adams; Michael R. Carter; Marcus J. Likeman; Julia Rankin

2007-01-01

63

Chiari I malformation related syringomyelia: radionuclide cisternography as a predictor of outcome  

Microsoft Academic Search

Summary Background. This prospective study, conducted in patients with Chiari I malformation (C I) related syringomyelia who underwent posterior decompression and duroplasty, utilizes radionuclide cisternography in order to study the cerebrospinal fluid (CSF) dynamics at the foramen magnum and to predict the clinical outcome following surgery. Methods. 17 consecutive patients of C I with syringomyelia (but without hydrocephalus or fixed

P. Arora; P. K. Pradhan; S. Behari; D. Banerji; B. K. Das; D. K. Chhabra; V. K. Jain

2004-01-01

64

Surgical results of arachnoid-preserving posterior fossa decompression for Chiari I malformation with associated syringomyelia  

Microsoft Academic Search

We analyzed the outcome of posterior fossa decompression accompanied by widening of the cisterna magna, without disturbing the arachnoid, in patients with Chiari I malformation (CMI) associated with syringomyelia. Twenty-five adult patients with CMI and syringomyelia, who underwent surgery between October 2000 and December 2008, were enrolled in this study. All patients underwent foramen magnum decompression with C1 decompression, with

Hyun Seok Lee; Sun-Ho Lee; Eun Sang Kim; Jong-Soo Kim; Jung-Il Lee; Hyung Jin Shin; Whan Eoh

65

Delayed pseudomyelomeningocele: a rare complication after foramen magnum decompression for Chiari malformation  

Microsoft Academic Search

BackgroundA variety of complications after FMD with or without duraplasty for Chiari malformation have been described. Although cerebellar ptosis through the dural opening and pseudomeningocele are well-known rare complications of this procedure, spinal cord herniation manifesting as pseudomyelomeningocele formation has not previously been cited in the literature. In this report, we present a case of delayed pseudomyleomeningocele after FMD.

Deniz Belen; Uygur Er; Levent Gurses; Kazim Yigitkanli

2009-01-01

66

Supracerebellar arachnoid cyst – A rare cause of acquired Chiari I malformation  

Microsoft Academic Search

Chiari I malformation (CM) associated with a cervico-thoracic syrinx due to supracerebellar arachnoid cyst has not been reported in the literature. We report such a case, managed by fenestration of the arachnoid cyst and foramen magnum decompression (FMD), aiming to reduce the inferiorly directed pressure on the cerebellum and eliminate the craniospinal pressure dissociation respectively. Imaging done post-operatively showed upward

Biji Bahuleyan; Ankamma Rao; Ari George Chacko; Roy Thomas Daniel

2007-01-01

67

Basilar invagination and Chiari malformation associated with cerebellar atrophy: report of two treated cases  

Microsoft Academic Search

We report two patients with an unusual complex of anomalies wherein the basilar invagination and Chiari malformation was associated with marked cerebellar atrophy. Both patients presented with relatively severe lower cranial nerve deficits and showed clinical improvement following a posterior foramen magnum bony decompression. The pathogenesis of the anomalies is discussed and the rationale of treatment is analysed.

Atul Goel; Ketan Desai; Mohinish Bhatjiwale; Dattatraya P. Muzumdar

2002-01-01

68

Effect of Craniocervical Decompression on Peak CSF Velocities in Symptomatic Patients with Chiari I Malformation  

Microsoft Academic Search

BACKGROUND AND PURPOSE: Peak CSF velocities detected in individual voxels in the subarachnoid space in patients with Chiari I malformations exceed those in similar locations in the subarachnoid space in healthy subjects. The purpose of this study was to test the hypothesis that the peak voxel velocities are decreased by craniocervical decompression. METHODS: A consecutive series of patients with symptomatic

Maria T. Dolar; Victor M. Haughton; Bermans J. Iskandar; Mark Quigley

2004-01-01

69

A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida  

Microsoft Academic Search

This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly

Helen Williams

2008-01-01

70

Cerebral oedema following EVD insertion for delayed hydrocephalus after foramen magnum decompression in Chiari I malformation  

Microsoft Academic Search

Case report. A case of Chiari I malformation is reported, in which foramen magnum decompression was followed by delayed onset of acute hydrocephalus after a week. Initial treatment of the hydrocephalus was further complicated by severe cerebral oedema. Discussion. A short survey of the literature suggests that both complications are extremely uncommon. The possible mechanism of development of cerebral oedema

Jark Bosma; Nagarajan Kumaran; Paul May

2002-01-01

71

Acquired Chiari malformation secondary to atlantoaxial vertical subluxation in a patient with rheumatoid arthritis combined with atlanto-occipital assimilation.  

PubMed

A 65-year-old woman with a history of rheumatoid arthritis presented with a rare case of acquired Chiari malformation secondary to atlantoaxial vertical subluxation, associated with congenital atlanto-occipital assimilation. Syringomyelia and tetraparesis improved immediately after posterior fossa decompression and simultaneous occipito-cervical junction fusion. The progression of acquired Chiari malformation is not well known. We concluded that coexisting assimilation accelerated crowded foramen magnum following atlantoaxial vertical subluxation and induced acquired Chiari malformation over the course of a few years. PMID:23006887

Kimura, Yuiko; Seichi, Atsushi; Gomi, Akira; Kojima, Masahiro; Inoue, Hirokazu; Kimura, Atsushi

2012-01-01

72

Tethering of the cervico-medullary junction with central cord oedema after foramen magnum decompression for Chiari malformation.  

PubMed

An unusual complication following foramen magnum decompression for Chiari malformation is described: adhesive tethering of the cervico-medullary junction with oedema of the upper spinal cord and lower medulla. Further surgery was required to correct this condition. PMID:21513448

Vergani, Francesco; Nicholson, Claire; Jenkins, Alistair

2011-04-22

73

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

PubMed

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

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

2010-09-17

74

A case of bilateral uveitis and optic disc swelling with Chiari I malformation  

PubMed Central

We report a case of bilateral uveitis and optic disc swelling with Chiari I malformation. A 16-year-old girl was admitted to our clinic due to conjunctival hyperaemia and blurred vision in her right eye. Ophthalmologic and systemic examinations were performed. Visual acuity was 0.7 (OD) and 1.0 (OS). Bilateral optic disc swelling was observed. Fluorescein angiography demonstrated bilateral retinal vasculitis as well as optic disc hyperflourescence due to leakage. Laboratory examinations were within normal limits. Cranial magnetic resonance venography imaging revealed neither cranial mass nor cerebral venous thrombosis but a Chiari I malformation. The patient was started oral cetazolamid, topical and oral corticosteroids. After six months follow-up, bilateral optic disc swelling was resolved completely and visual acuity was 1.0 in both eyes. Optic disc swelling may be associated with intraocular inflammation; however, patients with bilateral optic disc swelling should be suspected of having an accompanying intracranial pathology.

Eken, Volkan; Nilufer Yalc?ndag, F.; Bat?oglu, Figen; Is?kay, Canan Togay

2012-01-01

75

Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder  

PubMed Central

Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

Kumar, Shantanu; Sharma, Vineet; Kumar, Santosh; Jain, Sonal

2011-01-01

76

Glossopharyngeal neuralgia in the context of a Chiari type I malformation.  

PubMed

A 34-year-old woman presented with disabling right-sided glossopharyngeal neuralgia. MRI revealed a Chiari I malformation with an asymmetric herniation of the right tonsil. Surgical inspection of the lower cranial nerves through a suboccipital approach showed no vascular compromise. The patient was pain free for three months before the same symptoms recurred. MRI showed a persisting crowding at the level of the foramen magnum. A second intervention with extension of the suboccipital craniectomy and resection of the right tonsil achieved definitive pain relief. To our knowledge this is the fourth report of a glossopharyngeal neuralgia caused by a Chiari I malformation. Recurrence of the pain after incomplete decompression of the posterior fossa underlines the importance of tonsillectomy in these patients. PMID:22277563

Ruiz-Juretschke, Fernando; García-Leal, Roberto; Garcia-Duque, Sara; Panadero, Teresa; Aracil, Cristina

2012-01-25

77

Transient Quadriparesis following neck injury: presentation of a Chiari 1 malformation  

Microsoft Academic Search

We present the case of a 17-year-old male who presented with quadriparesis following an acute neck injury from a tackle in\\u000a a football match. MRI scans of cervical spine showed a Type-1 Chiari malformation. The quadriparesis resolved over 24 h. We\\u000a also present an overview of the differing presenting features of this condition from the literature. Whether these patients\\u000a have to

Harish Kurup; Tim Lawrence; David Hargreaves

2005-01-01

78

Radiographic Formation and Progression of Cervical Syringomyelia in a Child with Untreated Chiari I Malformation  

Microsoft Academic Search

We present the case of a 17-month-old girl with an untreated Chiari I malformation who developed radiographic progression of syringomyelia over a 23-month period. The patient presented with initial symptoms of airway difficulties and gait ataxia, which did not progress clinically over this period of observation, despite recommendations for surgical decompression. At 17 months, there was a region of T2

Adam C. Lipson; Richard G. Ellenbogen; Anthony M. Avellino

2008-01-01

79

Expansive Suboccipital Cranioplasty for the Treatment of Syringomyelia Associated with Chiari Malformation  

Microsoft Academic Search

Summary  ?In order to treat syringomyelia associated with adult type Chiari malformation, the authors developed a method of expansive\\u000a suboccipital cranioplasty (ESC) that involves enlarging the small posterior fossa to obtain a sufficient flow of cerebrospinal\\u000a fluid (CSF). The relative effectiveness of ESC with the obex plugged and not plugged was also examined, as well as other factors\\u000a influencing the operative

H. Sakamoto; M. Nishikawa; A. Hakuba; T. Yasui; S. Kitano; N. Nakanishi; Y. Inoue

1999-01-01

80

Chiari 1 malformation and holocord syringomyelia presenting as abrupt onset foot drop.  

PubMed

We present two patients with a Chiari 1 malformation and holocord syringomyelia who presented with abrupt onset unilateral foot drop. Neurophysiologic testing was consistent with a proximal nerve root lesion. This assisted with localization and directed magnetic resonance (MR) imaging to lumbosacral spine and nerve roots. Each child underwent a suboccipital craniectomy and laminectomy with duroplasty to decompress the foramen magnum. Each child also showed rapid and complete clinical recovery despite the significant electromyographic and MRI abnormalities on initial study. PMID:20811896

McMillan, Hugh J; Sell, Erick; Nzau, Munyao; Ventureyra, Enrique C G

2010-09-02

81

Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type 1)  

Microsoft Academic Search

Summary The authors report the surgical results of 28 patients with syringomyelia associated with Chiari malformation (type 1). 28 patients underwent 34 operative procedures. Syringo-subarachnoid shunt was performed in 28 patients, foramen magnum decompression with syringo-subarachnoid shunt in three, ventriculo-peritoneal shunt in one, terminal syringostomy in one, and foramen magnum decompression with terminal syringostomy in one. In an average postoperative

T. Isu; Y. Iwasaki; M. Akino; H. Abe

1990-01-01

82

Peak Systolic and Diastolic CSF Velocity in the Foramen Magnum in Adult Patients with Chiari I Malformations and in Normal Control Participants  

Microsoft Academic Search

BACKGROUND AND PURPOSE: Abnormal flow of CSF through the foramen magnum has been implicated in the pathogenesis of clinical deficits in association with Chiari I malforma- tion. The purpose of this study was to test the hypothesis that peak CSF velocities in the foramen magnum are increased in patients with Chiari I malformations. METHODS: Eight adult patients with symptomatic Chiari

Victor M. Haughton; Frank R. Korosec; Joshua E. Medow; Maria T. Dolar; Bermans J. Iskandar

83

Combined anterior and posterior approach for managing basilar invagination associated with type I Chiari malformation.  

PubMed

Ventral brainstem compression is common in patients with basilar invagination associated with type I Chiari malformation. The aim of this study was to investigate the reliability of a combined one-session anterior-posterior surgical approach for these patients. Nine patients underwent transoral odontoidectomy, posterior foramen magnum decompression, occipitocervical fixation, and bone grafting. All but two patients had ventral and dorsal operations in one surgical sitting. One of these two individuals had previously undergone posterior decompressive surgery at another hospital, but his condition had deteriorated rapidly afterward. The mean follow-up time was 19 months (range, 14-30 months). There was no mortality and no significant morbidity in this series. All but one of the patients showed significant improvement in their symptoms. Chiari malformations have a surprisingly high association with basilar invagination, and patients may have symptoms in both conditions. If there is a marked anterior compression, anterior transoral and posterior decompression, fusion, and instrumentation is an optimal strategy for treating patients with basilar invagination associated with type I Chiari malformation. PMID:12177543

Zileli, Mehmet; Cagli, Sedat

2002-08-01

84

Syringobulbia Caused by Delayed Postoperative Tethering of the Cervical Spinal Cord – Delayed Complication of Foramen Magnum Decompression for Chiari Malformation  

Microsoft Academic Search

Summary  ?Postoperative tethering of the high cervical spinal cord is a rare cause of neurological deterioration after foramen magnum\\u000a decompression (FMD) with duraplasty for Chiari type I malformation. A review of the literature revealed that only 5 cases\\u000a have been reported. This entity is not widely known to occur as a complication of the common surgical procedure for Chiari\\u000a type I

Y. Takahashi; Y. Tajima; S. Ueno; T. Tokutomi; M. Shigemori

1999-01-01

85

Phase-Contrast MR Imaging of the Cervical CSF and Spinal Cord: Volumetric Motion Analysis in Patients with Chiari I Malformation  

Microsoft Academic Search

BACKGROUND AND PURPOSE: Most previous MR studies of the dynamics of Chiari I malformation have been confined to sagittal images and operator-dependent measurement points in the midline. To obtain a deeper insight into the pathophysiology of the Chiari I malformation, we performed a prospective study using axial slices at the level of C2 to analyze volumetric motion data of the

Erich Hofmann; Monika Warmuth-Metz; Martin Bendszus

86

Pierre-Robin syndrome associated with Chiari type I malformation  

Microsoft Academic Search

Case reportPierre-Robin syndrome (PRS) is a rare congenital malformation that shows severe micrognathia and cleft soft palate. A 15-year-old boy who was admitted with occipital headache and gait disturbance was diagnosed with PRS. Radiological evaluation revealed severe herniation of the cerebellar tonsil and multiple craniovertebral osseous anomalies. We carried out foramen magnum decompression (FMD) with duroplasty. Postoperative MRI showed the

Jangbo Lee; Kazutoshi Hida; Toshitaka Seki; Jun Kitamura; Yosinobu Iwasaki

2003-01-01

87

Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management  

PubMed Central

Background The pathogenesis of Chiari malformations is incompletely understood. We tested the hypothesis that different etiologies have different mechanisms of cerebellar tonsil herniation (CTH), as revealed by posterior cranial fossa (PCF) morphology. Methods In 741 patients with Chiari malformation type I (CM-I) and 11 patients with Chiari malformation type II (CM-II), the size of the occipital enchondrium and volume of the PCF (PCFV) were measured on reconstructed 2D-CT and MR images of the skull. Measurements were compared with those in 80 age- and sex-matched healthy control individuals, and the results were correlated with clinical findings. Results Significant reductions of PCF size and volume were present in 388 patients with classical CM-I, 11 patients with CM-II, and five patients with CM-I and craniosynostosis. Occipital bone size and PCFV were normal in 225 patients with CM-I and occipitoatlantoaxial joint instability, 55 patients with CM-I and tethered cord syndrome (TCS), 30 patients with CM-I and intracranial mass lesions, and 28 patients with CM-I and lumboperitoneal shunts. Ten patients had miscellaneous etiologies. The size and area of the foramen magnum were significantly smaller in patients with classical CM-I and CM-I occurring with craniosynostosis and significantly larger in patients with CM-II and CM-I occurring with TCS. Conclusions Important clues concerning the pathogenesis of CTH were provided by morphometric measurements of the PCF. When these assessments were correlated with etiological factors, the following causal mechanisms were suggested: (1) cranial constriction; (2) cranial settling; (3) spinal cord tethering; (4) intracranial hypertension; and (5) intraspinal hypotension.

Nishikawa, Misao; Kula, Roger W.; Dlugacz, Yosef D.

2010-01-01

88

Morphometric Measurements of the Cranium in Patients with Chiari Type I Malformation and Comparison with the Normal Population  

Microsoft Academic Search

Summary.\\u000a Summary.  \\u000a ?\\u000a \\u000a \\u000a \\u000a \\u000a Background:   To determine the degree of development of the posterior fossa and signs of occipital dysplasia in patients with Chiari type\\u000a I malformation by morphometric measurements.\\u000a \\u000a ?\\u000a \\u000a \\u000a \\u000a \\u000a Methods:   In 22 patients with Chiari type I malformation, distance, surface area and angle values reflecting the degree of development\\u000a of the posterior fossa were measured and compared with the

F. Karagöz; N. ?zgi; S. Kap?c?o?lu Sencer

2002-01-01

89

Hidden aqueductal stenosis associated to bilateral idiopathic foramina of Monro stenosis mimicking a Chiari I malformation? Case report.  

PubMed

A 39-year old man came to our outpatient clinic with long history of unspecific symptoms and signs. Cerebral MRI showed herniation of the cerebellar tonsils of more than 1 cm below the foramen magnum and a triventricular hydrocephalus. A diagnosis of Chiari I malformation was retained. After an osteo-dural decompression of the posterior fossa, post-operative MRI revealed an aqueductal stenosis with triventricular hydrocephalus. An endoscopic-third- ventriculostomy showed an idiopathic stenosis of the right foramen of Monro. Residual symptoms and persistence of biventricular hydrocephalus justified a ventriculo-peritoneal shunt. Aqueductal and foramina of Monro stenosis can mimick a Chiari I malformation. PMID:22955871

Bartoli, Andrea; Ghinda, Cristina Diana; Radovanovic, Ivan; Momjian, Shahan

2012-09-07

90

Acute deterioration of a Chiari I malformation: an uncommon neurosurgical emergency  

Microsoft Academic Search

Objectives  Report and discuss acute neurological deterioration of Chiari I malformation (CMI), surgical treatment and timing, clinical\\u000a outcome and literature review.\\u000a \\u000a \\u000a \\u000a \\u000a Case report  We report a recent case of a 15-year-old girl admitted for an acute neurological deterioration caused by an undiagnosed hindbrain\\u000a herniation that required an urgent foramen magnum decompression and external ventricular drainage insertion. Moreover, we\\u000a report and discuss the

Benedetta Ludovica Pettorini; Anna Gao; Desiderio Rodrigues

2011-01-01

91

Analysis of the Volumes of the Posterior Cranial Fossa, Cerebellum, and Herniated Tonsils Using the Stereological Methods in Patients with Chiari Type I Malformation  

PubMed Central

Objective. The aim of this study was to determine the posterior cranial fossa volume, cerebellar volume, and herniated tonsillar volume in patients with chiari type I malformation and control subjects using stereological methods. Material and Methods. These volumes were estimated retrospectively using the Cavalieri principle as a point-counting technique. We used magnetic resonance images taken from 25 control subjects and 30 patients with chiari type I malformation. Results. The posterior cranial fossa volume in patients with chiari type I malformation was significantly smaller than the volume in the control subjects (P < 0.05). In the chiari type I malformation group, the cerebellar volume was smaller than the control group, but this difference was not statistically significant (P > 0.05). In the chiari type I malformation group, the ratio of cerebellar volume to posterior cranial fossa volume was higher than in the control group. We also found a positive correlation between the posterior cranial fossa volume and cerebellar volume for each of the groups (r = 0.865, P < 0.001). The mean (±SD) herniated tonsillar volume and length were 0.89 ± 0.50?cm3 and 9.63 ± 3.37?mm in the chiari type I malformation group, respectively. Conclusion. This study has shown that posterior cranial fossa and cerebellum volumes can be measured by stereological methods, and the ratio of these measurements can contribute to the evaluation of chiari type I malformation cases.

Vurdem, Umit Erkan; Acer, Niyazi; Ertekin, Tolga; Savranlar, Ahmet; Inci, Mehmet Fatih

2012-01-01

92

Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation  

PubMed Central

A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes. Radiographs showed occipitalization of the atlas, C2–C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ) compression from the odontoid, a Chiari type I malformation, and syringomyelia extending from the foramen magnum to C5. The patient underwent transoral atlantoaxial release followed by posterior internal fixation from the occiput to the axis, which resulted in a significant improvement in motor function in all extremities. Post-operative images showed anatomical reduction of the atlantoaxial joint. However, an MRI performed 8 days following surgery showed a new retro-odontoid pannus had developed that was compressing the spinal cord at CMJ. A follow-up CT scan performed at 6 months post-operatively demonstrated a solid bony fusion between the occiput and C2, while an MRI at that time showed complete resolution of the retro-odontoid soft tissue mass with correction of the Chiari I malformation, and resolution of the syringomyelia. Final follow-up at 2-years revealed an excellent clinical outcome.

Wang, Shenglin; Yan, Ming; Zhou, Haitao; Jiang, Liang

2009-01-01

93

Syringomyelia with irreducible atlantoaxial dislocation, basilar invagination and Chiari I malformation.  

PubMed

A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes. Radiographs showed occipitalization of the atlas, C2-C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ) compression from the odontoid, a Chiari type I malformation, and syringomyelia extending from the foramen magnum to C5. The patient underwent transoral atlantoaxial release followed by posterior internal fixation from the occiput to the axis, which resulted in a significant improvement in motor function in all extremities. Post-operative images showed anatomical reduction of the atlantoaxial joint. However, an MRI performed 8 days following surgery showed a new retro-odontoid pannus had developed that was compressing the spinal cord at CMJ. A follow-up CT scan performed at 6 months post-operatively demonstrated a solid bony fusion between the occiput and C2, while an MRI at that time showed complete resolution of the retro-odontoid soft tissue mass with correction of the Chiari I malformation, and resolution of the syringomyelia. Final follow-up at 2-years revealed an excellent clinical outcome. PMID:19941013

Wang, Shenglin; Wang, Chao; Yan, Ming; Zhou, Haitao; Jiang, Liang

2009-11-26

94

Chiari Malformation Type I, presenting as scapulothoracic pain: a case report.  

PubMed

Patients presenting with recalcitrant symptoms, which are resistant to a range of physical interventions over an extended period of time, need to be examined with care. A 'forensic' approach to the assessment of these patients, with a sensitive approach to the results of the test procedures is required to ensure the practitioner does not miss the more obscure causes. This case report presents a patient who was referred for a clinical assessment for long standing scapular pain, which had been labelled myofascial pain. Through a thorough approach to examination a number of flags appeared which suggested a need for further investigation. These investigations identified that the patient demonstrated a Chiari Malformation Type 1. On review 5 months following neurosurgical intervention her symptoms were significantly reduced. Chiari Malformations Type 1 are often diagnosed in adulthood when symptoms usually first appear. These symptoms may mimic those found with musculoskeletal conditions. Whilst we lack specific clinical tests for this condition, a thorough assessment should identify sufficient 'flags' for referral for further investigations. PMID:22245421

Worth, David R; Milanese, Steven

2012-01-14

95

Increased pulsatile movement of the hindbrain in syringomyelia associated with the Chiari malformation: cine-MRI with presaturation bolus tracking  

Microsoft Academic Search

Cine-MRI with presaturation bolus tracking was used in patients with syringomyelia associated with a Chiari malformation to study pulsatile movement of the hindbrain, cervical spinal cord, cerebrospinal fluid and the fluid within the syrinx. Nine patients had 13 examinations, 6 preoperative, 3 after syringosubarachnoid shunting and 4 after posterior fossa decompression. Five controls were also examined. Dynamic display of the

S. Terae; K. Miyasaka; S. Abe; H. Abe; K. Tashiro

1994-01-01

96

Spontaneous C1 anterior arch fracture as a postoperative complication of foramen magnum decompression for Chiari malformation type 1  

PubMed Central

Background: C1 fracture accounts for 2% of all spinal column injuries and 10% of cervical spine fractures, and is most frequently caused by motor vehicle accidents and falls. We present a rare case of C1 anterior arch fracture following standard foramen magnum decompression for Chiari malformation type 1. Case Description: A 63-year-old man underwent standard foramen magnum decompression (suboccipital craniectomy and C1 laminectomy) under a diagnosis of Chiari malformation type 1 with syringomyelia in June 2009. The postoperative course was uneventful until the patient noticed progressive posterior cervical pain 5 months after the operation. Computed tomography of the upper cervical spine obtained 7 months after the operation revealed left C1 anterior arch fracture. The patient was referred to our hospital at the end of January 2010 and C1–C2 posterior fusion with C1 lateral mass screws and C2 laminar screws was carried out in March 2010. Complete pain relief was achieved immediately after the second operation, and the patient resumed his daily activities. Conclusion: Anterior atlas fracture following foramen magnum decompression for Chiari malformation type 1 is very rare, but C1 laminectomy carries the risk of anterior arch fracture. Neurosurgeons should recognize that fracture of the atlas, which commonly results from an axial loading force, can occur in the postoperative period in patients with Chiari malformation.

Hirano, Yoshitaka; Sugawara, Atsushi; Mizuno, Junichi; Takeda, Masaaki; Watanabe, Kazuo; Ogasawara, Kuniaki

2011-01-01

97

Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation  

Microsoft Academic Search

Summary Background. Spinal cord traction caused by a tight filum terminale may be considered a pathogenic mechanism involved in the development of syringomyelia, the Chiari malformation (type I) and scoliosis. Section of the filum terminale is proposed as a useful surgical approach in these conditions.

M. B. Royo-Salvador; J. Solé-Llenas; J. M. Doménech; R. González-Adrio

2005-01-01

98

A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida  

PubMed Central

This work is a modified version of the Casey Holter Memorial prize essay presented to the Society for Research into Hydrocephalus and Spina Bifida, June 29th 2007, Heidelberg, Germany. It describes the origin and consequences of the Chiari malformation, and proposes that hydrocephalus is caused by inadequate central nervous system (CNS) venous drainage. A new hypothesis regarding the pathogenesis, anencephaly and spina bifida is described. Any volume increase in the central nervous system can increase venous pressure. This occurs because veins are compressible and a CNS volume increase may result in reduced venous blood flow. This has the potential to cause progressive increase in cerebrospinal fluid (CSF) volume. Venous insufficiency may be caused by any disease that reduces space for venous volume. The flow of CSF has a beneficial effect on venous drainage. In health it moderates central nervous system pressure by moving between the head and spine. Conversely, obstruction to CSF flow causes localised pressure increases, which have an adverse effect on venous drainage. The Chiari malformation is associated with hindbrain herniation, which may be caused by low spinal pressure relative to cranial pressure. In these instances, there are hindbrain-related symptoms caused by cerebellar and brainstem compression. When spinal injury occurs as a result of a Chiari malformation, the primary pathology is posterior fossa hypoplasia, resulting in raised spinal pressure. The small posterior fossa prevents the flow of CSF from the spine to the head as blood enters the central nervous system during movement. Consequently, intermittent increases in spinal pressure caused by movement, result in injury to the spinal cord. It is proposed that posterior fossa hypoplasia, which has origins in fetal life, causes syringomyelia after birth and leads to damage to the spinal cord in spina bifida. It is proposed that hydrocephalus may occur as a result of posterior fossa hypoplasia, where raised pressure occurs as a result of obstruction to flow of CSF from the head to the spine, and cerebral injury with raised pressure occurs in anencephaly by this mechanism. The current view of dysraphism is that low central nervous system pressure and exposure to amniotic fluid, damage the central nervous system. The hypothesis proposed in this essay supports the view that spina bifida is a manifestation of progressive hydrocephalus in the fetus. It is proposed that that mesodermal growth insufficiency influences both neural tube closure and central nervous system pressure, leading to dysraphism.

Williams, Helen

2008-01-01

99

Pediatric Chiari  

MedlinePLUS

... been living with Chiari Malformation. Parker age 5, Gracie Claire age 6, Zoey age 7 and I ( ... to have their surgeries on the same day. Gracie Claire, began to have headaches which prompted a ...

100

Three sisters with Chiari I malformation with and without associated syringomyelia.  

PubMed

Two daughters of non-consanguineous Ashkenazi Jewish parentage presented with occipital headaches in the second decade of life. Each had a symptomatic Chiari I malformation (CMI) and a large cervicothoracic syrinx. A third sister was diagnosed as having CMI without syrinx after MR screening of first-degree relatives. A fourth (the eldest) sister was asymptomatic and did not have CMI or syrinx. The girls' mother had platybasia on screening MR and a history of cough headaches. All four sisters also had demonstrable platybasic features on MR. The girls' father was asymptomatic and radiologically normal. This family represents the first reported case of three siblings in one family with confirmed CMI with or without syrinx. We discuss the possible genetic and mechanical mechanisms for the development of these abnormalities in this family. PMID:17992048

Weisfeld-Adams, James D; Carter, Michael R; Likeman, Marcus J; Rankin, Julia

2007-01-01

101

Patient with Chiari malformation Type I presenting with inducible hemifacial weakness.  

PubMed

In this report, the authors describe the first known case of inducible hemifacial weakness in a patient with Chiari malformation Type I (CM-I). The patient was a 14-year-old girl with a 1-year history of right facial paresis induced by sustained leftward head rotation. These episodes were characterized by weak activation of her right facial muscles with preserved eye opening and closure. Additionally, she had hypernasal speech, persistent headaches, and intermittent left arm twitching. Magnetic resonance imaging demonstrated a CM-I. A suboccipital craniectomy and C-1 laminectomy were performed for decompression of the CM-I, with duraplasty and coagulation of the pial surface of the cerebellar tonsils. At the 9-month follow-up, the patient's inducible hemifacial weakness had completely resolved. Her symptoms were thought to have resulted from the CM-I, perhaps due to traction on the right facial nerve by the ectopic tonsils with head rotation. PMID:22132921

Witt, Cordelie E; Wang, Anthony C; Maher, Cormac O; Than, Khoi D; Garton, Hugh J L; Muraszko, Karin M

2011-12-01

102

Section of the filum terminale: is it worthwhile in Chiari type I malformation?  

PubMed

A section of the filum terminale (SFT) is used for the surgical treatment of isolated tethered cord or that resulting from neurulation disorders. More recently, it has been proposed for the management of the occult tethered cord syndrome (OTCS), though it is still under debate. Even more controversial appears to be the use of SFT in patients with Chiari type I malformation (CIM), which is based on the possible presence of OTCS. This review shows that: (1) there are issues both in favor and against the occurrence of OTCS, (2) there is no significant correlation between CIM and tethered cord, the old "caudal traction theory" being not supported by clinical or experimental evidences. On these grounds, a relationship between CIM and OTCS is hard to be demonstrated, (3) a subgroup of patients with CIM suffering from OTCS may exist and benefit from SFT. PMID:21800080

Massimi, Luca; Peraio, Simone; Peppucci, Elisabetta; Tamburrini, Gianpiero; Di Rocco, Concezio

2011-12-01

103

The association between Chiari-like malformation, ventriculomegaly and seizures in cavalier King Charles spaniels.  

PubMed

Cavalier King Charles spaniels (CKCSs) with Chiari-like malformation (CM) and associated seizures are frequently diagnosed with idiopathic epilepsy. There could be an association between ventriculomegaly (V) or caudal fossa overcrowding (CCFP) and seizures. A retrospective case-control study was performed using MRI to investigate the possible association between these morphological abnormalities and seizures. Seizure semiology and, where possible, electroencephalographic (EEG) abnormalities were documented. Eighty-five CKCS with CM were included, 27 with seizures. There was no association between V or CCFP and seizures (P=0.10 and 0.71, respectively). Seizures were classified as having partial onset in 61% of individuals in the study population (95% CI 42.41-76.43%). Another cause of recurrent seizures in CKCS (such as familial epilepsy) is suspected, as previously reported. PMID:22749114

Driver, C J; Chandler, K; Walmsley, G; Shihab, N; Volk, H A

2012-06-28

104

Sleep Disordered Breathing in Children and Adolescents with Chiari Malformation Type I  

PubMed Central

Study Objectives: Chiari malformation Type I (CM-I) has been associated with sleep disordered breathing (SDB). The aim of this study was to evaluate the prevalence of SDB in CM-I and its clinical correlates in a population of children and adolescents. Methods: Fifty-three consecutive children and adolescents affected by CM-I were enrolled (27 girls and 26 boys, mean age 10.3 ± 4.3, range: 3-18 years). All patients underwent neurological examination, MRI, and polysomnography (PSG). Otorhinolaryngologic clinical evaluation was performed in patients with polysomnographic evidence of sleep-related upper airway obstruction. Results: Mean size of the herniation was 9.5 ± 5.4 mm. Fourteen patients had syringomyelia, 5 had hydrocephalus, 31 presented neurological signs, 14 had epileptic seizures, and 7 reported poor sleep. PSG revealed SDB in 13 subjects. Patients with SDB, compared to those without SDB, had a higher prevalence hydrocephalus (p = 0.002), syringomyelia (p = 0.001), and neurological symptoms (p = 0.028). No significant difference was observed in age, gender, prevalence of epilepsy, and size of the herniation. Obstructive SDB was associated with syringomyelia (p = 0.004), whereas central SDB was associated with hydrocephalus (p = 0.034). Conclusions: In our population of CM-I patients the prevalence of SDB was 24%, lower than that reported in literature. Moreover, our findings suggest that abnormalities in cerebrospinal fluid dynamics in CM-I, particularly syringomyelia and hydro-cephalus, are associated with SDB. Citation: Losurdo A; Dittoni S; Testani E; Di Blasi C; Scarano E; Mariotti P; Paternoster G; Di Rocco C; Massimi L; Della Marca G. Sleep disordered breathing in children and adolescents with Chiari malformation Type I. J Clin Sleep Med 2013;9(4):371-377.

Losurdo, Anna; Dittoni, Serena; Testani, Elisa; Di Blasi, Chiara; Scarano, Emanuele; Mariotti, Paolo; Paternoster, Giovanna; Di Rocco, Concezio; Massimi, Luca; Della Marca, Giacomo

2013-01-01

105

Chiari Malformation  

MedlinePLUS

... is a brain abnormality that affects the lower part of the brain , called the cerebellum. Many kids with this abnormality ... a brain abnormality that causes the cerebellum, the part of the brain that controls coordination and muscle movement, to protrude ...

106

Duraplasty with freeze-dried cadaveric dura versus occipital pericranium for Chiari type I malformation: Comparative study  

Microsoft Academic Search

Summary  During the period from October 1, 1989 to October 1, 1995 a total of 26 cases of Chiari type I malformation not associated\\u000a with syringomyelia were attended in our Hospital. All patients underwent cranio-cervical decompression, with occipital craniectomy\\u000a and removal of the posterior arch of C1. In 3\\/26 (11.5%) cases an additional C2 laminectomy had to be performed and in

V. Vanaclocha; N. Saiz-Sapena

1997-01-01

107

Transverse Microincisions of the Outer Layer of the Dura mater Combined with Foramen Magnum Decompression as Treatment for Syringomyelia with Chiari I Malformation  

Microsoft Academic Search

Summary   Numerous surgical procedures have been proposed for treatment of syringomyelia associated with Chiari I malformation, but\\u000a the optimal treatment has not yet been uniformly standardised. The main aim of the surgical treatment of syringomyelia\\/Chiari\\u000a I complex is directed toward restoration of physiological cerebrospinal fluid dynamic at the craniovertebral junction. We\\u000a report the surgical results of eight patients, affected by

G. Gambardella; G. Caruso; M. Caffo; A. Germanň; G. La Rosa; F. Tomasello

1998-01-01

108

Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases.  

PubMed

We analyze the results of surgical treatment of 60 patients presenting syringomyelia (SM) associated with Chiari malformation (CM) who were operated in the period 1982-2000. For each case, analysis covered 15 signs and 16 symptoms included in a protocol that separated SM signs and symptoms from those of CM. A score system was established in parallel with the protocol to make the evaluation of treatment results easier. All cases were submitted to craniovertebral decompression by C1 and eventually C2 laminectomy and cerebellar tonsillectomy with duramater graft. To evaluate the results, statistical proportion difference tests and variance analyses were made to a reliability index of 95% (p=0.05). We conclude that the statistical improvement of CM signs and symptoms was very significant (p=0). Syringomyelia signs and symptoms also improved significantly, except for "upper limb hyporeflexia", which did not improve. No statistical difference in the improvement of SM symptoms as compared to CM symptom was found. Syringomyelia signs improved statistically more than CM signs. In half of patients, the percent improvement of signs and symptoms ranged between 40% and 60%. PMID:15235724

Arruda, José Arnaldo Motta de; Costa, Carlos Maurício de Castro; Tella Jr, Oswaldo Inácio de

2004-06-23

109

Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients.  

PubMed

The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI), Chiari malformation (CM), and syringomyelia (SM). The patients were divided into two groups. Group I (24 patients) underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients) underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation) in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior- caudal direction, avoiding, in this way, its adherence to the cerebellum. PMID:12894269

da Silva, José Alberto Gonçalves; Holanda, Maurus Marques de Almeida

2003-07-28

110

Retrospective study of patients with Chiari: malformation submitted to surgical treatment.  

PubMed

The Chiari malformation (CM) is characterized by variable herniation of one or both cerebellar tonsils, associated or not with displacement of the hindbrain structures into the vertebral canal. This is a retrospective study of 29 patients with CM submitted to surgical procedure between 1990 and 2003. There were 14 females and 15 males whose ages ranged from 16 to 65 years. There were seven patients with isolated CM, 12 associated with syringomyelia (SM), three associated with basilar impression (BI) and seven associated with SM and BI. The surgery was based on posterior fossa decompression. In seven patients a catheter was introduced from the subarachnoid space into the III ventricle and five were submitted to tonsillectomy. Twenty-one patients improved, one worsened, one remained unchanged, four missed follow up and two died. We conclude that the best results with CM surgery are obtained by an effective posterior fossa decompression. Those CM cases associated with other abnormalities, such as SM and BI, probably need complementary techniques which will be the theme for new prospective studies. PMID:18545779

Taricco, Mário Augusto; Melo, Luiz Ricardo Santiago

2008-06-01

111

Remote cerebellar hemorrhage after foramen magnum decompression surgery for Chiari I malformation--case report.  

PubMed

A 47-year-old woman underwent decompressive suboccipital craniectomy and C1 laminectomy with duroplasty in the prone position for Chiari malformation type I and syringomyelia. The arachnoid membrane was not injured. Intraoperative echography showed good enlargement of the subarachnoid space. No closed subcutaneous drain was used. The patient complained of repeated nausea and vomiting 3 hours after the operation, and computed tomography revealed remote cerebellar hemorrhage on postoperative day 1. The cerebellar hemorrhage was treated conservatively, and the symptoms continued only for 3 days after surgery. Dural opening with rapid loss of cerebrospinal fluid (CSF) has occurred in every reported case of remote cerebellar hemorrhage complicating intracranial and spinal procedures. Loss of CSF is the main pathogenesis of this condition. In our case, the most probable pathomechanism seems to involve stretching of the infratentorial cerebellar bridging veins due to cerebellar sagging because of dural opening in the prone position and drop in CSF pressure. Such a complication is rare but should be considered after foramen magnum decompression surgery if the patient shows unusual symptoms of repeated vomiting. PMID:21358158

Kaneko, Takahisa; Koyanagi, Izumi; Murakami, Tomohiro

2011-01-01

112

Infantile osteopetrosis, craniosynostosis, and Chiari malformation type I with novel OSTEM1 mutation  

PubMed Central

We report a case of a 9-month-old Arab infant, with novel OSTEM mutation and unpublished triad of osteopetrosis (OP), craniosynostosis (CS), and Chiari malformation type I (CM1). The index presented with progressive irritability, abnormal movements, following an accidental fall. The history revealed early onset of irritability, progressive visual loss, and global developmental delay, more prominent at the gross motor level and a suspected congenital cytomegalovirus infection. The pregnancy was uneventful with subsequent unremarkable delivery. The parents are Arabs?first cousins with no apparent symptoms or signs of bone disease. Three dimensional brain computed tomography (CT) showed ventriculomegaly, thick calvaria, and CS of the coronal and sagittal sutures. Patient had signs of left lower motor neuron facial palsy, and CT of petrous bones confirms the presence of osteopetrotic petrous with slim mastoid portions of the facial nerve canals both sides. Brain magnetic resonance imaging showed CM1. Skeletal survey showed sclerotic skeleton. He needed ventriculoperitoneal shunt and died at 18 months of age. Molecular testing for OSTEM1 gene revealed novel homozygous mutation that segregated from his parents. This novel OSTEM1 gene novel mutation and the combination of OP, infantile CS, and CM1 is to our knowledge never been reported.

Mahmoud Adel, A. H.; Abdullah, A. Al Jabri; Eissa, Faqeih

2013-01-01

113

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

PubMed Central

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.

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

2008-01-01

114

[Arnold-Chiari malformation with multiple paroxysmal manifestations induced by coughing].  

PubMed

We present a 66-year old woman suffering from a chronic disorder characterized by multiple paroxysmal symptoms precipitated by coughing. These included cephalalgia, syncope, binocular photopsia phenomena with blurred vision, and an "electric-like" paroxysmal tingling of the hands. In addition to a central spinal cord cavity and hindbrain herniation, magnetic resonance imaging showed multiple skeletal anomalies and the craniospinal junction which included a narrow clivo-axial angle, basilar impression of the skull and a tight foramen magnum. Resonance magnetic imaging showed a high-signal intensity lesion on T2-weighted images at the posterior medullo-spinal junction suggesting focal demyelination. We propose that paroxysmal symptoms induced by coughing in patients bearing hindbrain ectopia and skeletal anomalies at the foramen magnum region may involve different pathogenetic mechanisms, including ectopic axonal activity and ephaptic transmission at the sensory pathways. This caused a Lhermitte-like phenomenon precipitated by coughing, rather than by forward flexion of the neck. However, increased pressure at the posterior fossa presumably underlies all these phenomena, and may therefore be potentially relieved by suboccipital decompressive craniotomy. PMID:1596400

Alarcón, J; Dobato, J L; Mateo, D; Benito, C; Giménez-Roldán, S

1992-01-01

115

Normalization of hindbrain morphology after decompression of Chiari malformation Type I  

PubMed Central

Object Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I. Methods A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3–6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group). Results Before surgery, the patients’ posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3–6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001). Conclusions The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.

Heiss, John D.; Suffredini, Giancarlo; Bakhtian, Kamran D.; Sarntinoranont, Malisa; Oldfield, Edward H.

2013-01-01

116

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

PubMed Central

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.

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

2013-01-01

117

Surgical outcomes of foramen magnum decompression for syringomyelia associated with Chiari I malformation: relation between the location of the syrinx and body pain  

Microsoft Academic Search

Background  There have been few reports about the relation between the morphology of syrinxes and body pain in syringomyelia associated\\u000a with Chiari I malformation. To investigate this phenomenon, the relation between the location of the syrinx and body pain\\u000a before and after foramen magnum decompression (FMD) were evaluated.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  The subjects were 20 patients with Chiari I malformation associated with syringomyelia who

Atsushi Ono; Takuya Numasawa; Kanichiro Wada; Toru Yokoyama; Kazunari Takeuchi; Futoshi Suetsuna; Kazumasa Ueyama; Satoshi Toh

2010-01-01

118

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

PubMed

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

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

2011-01-01

119

Chiari Malformation Type I: A Case-Control Association Study of 58 Developmental Genes  

PubMed Central

Chiari malformation type I (CMI) is a disorder characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa (PCF), often causing progressive neurological symptoms. The etiology of CMI remains unclear and is most likely multifactorial. A putative genetic contribution to CMI is suggested by familial aggregation and twin studies. Experimental models and human morphometric studies have suggested an underlying paraxial mesoderm insufficiency. We performed a case-control association study of 303 tag single nucleotide polymorphisms (SNP) across 58 candidate genes involved in early paraxial mesoderm development in a sample of 415 CMI patients and 524 sex-matched controls. A subgroup of patients diagnosed with classical, small-PCF CMI by means of MRI-based PCF morphometry (n?=?186), underwent additional analysis. The genes selected are involved in signalling gradients occurring during segmental patterning of the occipital somites (FGF8, Wnt, and retinoic acid pathways and from bone morphogenetic proteins or BMP, Notch, Cdx and Hox pathways) or in placental angiogenesis, sclerotome development or CMI-associated syndromes. Single-marker analysis identified nominal associations with 18 SNPs in 14 genes (CDX1, FLT1, RARG, NKD2, MSGN1, RBPJ1, FGFR1, RDH10, NOG, RARA, LFNG, KDR, ALDH1A2, BMPR1A) considering the whole CMI sample. None of these overcame corrections for multiple comparisons, in contrast with four SNPs in CDX1, FLT1 and ALDH1A2 in the classical CMI group. Multiple marker analysis identified a risk haplotype for classical CMI in ALDH1A2 and CDX1. Furthermore, we analyzed the possible contributions of the most significantly associated SNPs to different PCF morphometric traits. These findings suggest that common variants in genes involved in somitogenesis and fetal vascular development may confer susceptibility to CMI.

Urbizu, Aintzane; Toma, Claudio; Poca, Maria A.; Sahuquillo, Juan; Cuenca-Leon, Ester; Cormand, Bru; Macaya, Alfons

2013-01-01

120

Surgical results of arachnoid-preserving posterior fossa decompression for Chiari I malformation with associated syringomyelia.  

PubMed

We analyzed the outcome of posterior fossa decompression accompanied by widening of the cisterna magna, without disturbing the arachnoid, in patients with Chiari I malformation (CMI) associated with syringomyelia. Twenty-five adult patients with CMI and syringomyelia, who underwent surgery between October 2000 and December 2008, were enrolled in this study. All patients underwent foramen magnum decompression with C1 decompression, with or without C2 decompression. Three surgeons performed a dura opening with duraplasty in 20 patients, and another surgeon excised the outer layer of the dura without duraplasty in five patients. Clinical and radiological assessments were performed preoperatively and during the follow-up period. After surgery, 20 (80%) patients achieved a significant improvement in their clinical symptoms. However, four patients (16%) achieved only a stable state, and one patient's symptoms worsened. Radiological analysis showed that 17 patients (68%) had a favorable result; that is, a total collapse, or a marked reduction, of the syrinx. Seven patients (28%) were stable in terms of syrinx size. However, the syrinx enlarged in one patient who had undergone excision of the outer dura. Twenty-four patients achieved a widened cisterna magna with ascent of the cerebellar tonsils into the posterior fossa and acquisition of a more rounded shape. Postoperative complications included a transient headache and vomiting in three patients and transient motor weakness in one patient. Two patients developed a superficial wound infection. This study shows that arachnoid-preserving posterior fossa decompression is a safe and effective treatment for patients with CMI with associated syringomyelia. PMID:22300790

Lee, Hyun Seok; Lee, Sun-Ho; Kim, Eun Sang; Kim, Jong-Soo; Lee, Jung-Il; Shin, Hyung Jin; Eoh, Whan

2012-02-01

121

The Cerebellar Dysplasia of Chiari II Malformation as Revealed by Eye Movements  

PubMed Central

Introduction Chiari type II malformation (CII) is a developmental deformity of the hindbrain. We have previously reported that many patients with CII have impaired smooth pursuit, while few make inaccurate saccades or have an abnormal vestibulo-ocular reflex. In contrast, saccadic adaptation and visual fixation are normal. In this report, we correlate results from several eye movement studies with neuroimaging in CII. We present a model for structural changes within the cerebellum in CII. Methods Saccades, smooth pursuit, the vestibulo-ocular reflex, and visual fixation were recorded in 21 patients with CII, aged 8–19 years and 39 age-matched controls, using an infrared eye tracker. Qualitative and quantitative MRI data were correlated with eye movements in 19 CII patients and 28 controls. Results Nine patients with CII had abnormal eye movements. Smooth pursuit gain was subnormal in eight, saccadic accuracy abnormal in four, and vestibulo-ocular reflex gain abnormal in three. None had fixation instability. Patients with CII had a significantly smaller cerebellar volume than controls, and those with normal eye motion had an expanded midsagittal vermis compared to controls. However, patients with abnormal eye movements had a smaller (non-expanded) midsagittal vermis area, posterior fossa area and medial cerebellar volumes than CII patients with normal eye movements. Conclusions The deformity of CII affects the structure and function of the cerebellum selectively and differently in those with abnormal eye movements. We propose that the vermis can expand when compressed within a small posterior fossa in some CII patients, thus sparing its ocular motor functions.

Salman, Michael S.; Dennis, Maureen; Sharpe, James A.

2011-01-01

122

Rapidly progressing monoparesis caused by Chiari malformation type I without syringomyelia  

PubMed Central

Background: Patients with Chiari malformation type I (CM-I) can manifest neurological symptoms, such as headache, neck pain, dysesthesia, swallowing disturbance, and paresis, which are usually stable or slowly progressive even if syringomyelia is coexistent. In some instances, however, acute onset of neurological symptoms has been reported but the pathogenetic mechanism and subsequent clinical course have not been explained. In those cases, it was reported that urgent treatment of foramen magnum decompression (FMD) was very effective. This work reports that an 11-year-old girl with CM-I subacutely developed unique symptoms and that urgent treatment of FMD was very effective. Case Description: We present here an 11-year-old girl with CM-I who subacutely developed dysphagia, left upper extremity monoparesis and sensory dysesthesia, with the limb assuming a peculiar posture at rest, with the wrist in extension and the elbow joint in flexion. Although her symptoms were assumed to be due to previously diagnosed CM-I without syringomyelia, no differences on magnetic resonance imaging (MRI) could be found except for slight change in the shape of tonsils compared with the previous ones. FMD and C1 removal with duraplasty was performed and resulted in an excellent neurological recovery. Conclusion: This case is a reminder that the presence of a new neurological deficit referable to nuclei within, or tracts that traverse, the cerebromedullary junction is a firm surgical indication for FMD in a patient with CM-I. MRI was nearly identical during the asymptomatic and symptomatic periods in this case, and did not explain the timing of symptom onset.

Oishi, Masahiro; Hayashi, Yasuhiko; Kita, Daisuke; Fukui, Issei; Shinohara, Moeko; Heiss, John D.; Hamada, Jun-ichiro

2013-01-01

123

The neurophysiological balance in Chiari type 1 malformation (CM1), tethered cord and related syndromes.  

PubMed

The Chiari malformation (CM) is a syndrome embodied in heterogeneous groups of malformations, spanning from the more benign and known, the CM1, to more complex syndromes such as the rare association with the tethered cord, as spinal lipomas, and the CM2, associated to open spina bifida. The clinical picture may be well expressed and detected at birth or even during intrauterine life, as for CM2, but in the other cases they may run a rather subtle clinical course. The diagnosis of these syndromes is driven by clinical examination and MRI, and it usually requires a multidisciplinary approach in order to plan the therapeutic strategies, such as surgery. Among the diagnostic investigations, the imaging techniques represent the most useful, for their capabilities to detect subclinical lesions, such as syringomyielia and lipoma; the urological investigation is useful to evaluate the urogenital dysfunctions. The neurophysiological investigations represent a non invasive diagnostic procedure to investigate the peripheral nerve, the spinal cord, the brainstem functionalities and more higher brain functions; the nerve conduction studies and the cranial reflexes, the brainstem (BAEP) and the somatosensory (SEPs) evoked potentials (EPs), alone or in combination, can be used for the diagnosis, follow-up and intraoperative monitoring. The most useful diagnostic tools in CM1 are likely represented by the brainstem auditory evoked potentials (BAEPs) and the blink-reflex (BR), while the usefulness of SEPs is still doubtful and debated; in CM2 and tethered cord the neurophysiological techniques can be combined in different ways in order to make a functional balance and to answer specific questions. BAEPs and BR can be useful to investigate the brain stem functionality and SEP to evaluate whether the ascending sensory pathway to the cortex can be hampered at some level; the visual EPs are particularly useful to evaluate the integrity of posterior visual pathway and visual cortex in the case of associated hydrocephalus. In the tethered cord, both nerve conduction study and somatosensory evoked potentials (SEPs) are useful to evaluate motor and sensory dysfunction of the lombosacral roots and nerves and spinal cord for their capability to detect subclinical impairment of conduction along the sensory and motor pathway. Finally, last but not the least, the neurophysiological techniques are remarkably useful during surgery; the intraoperative monitoring (IOM) by means of electromyography and direct nerve stimulation and recordings are able to detect early nerve damage, minimize nerve lesions and optimise the surgical techniques. In the operated children with incomplete removal of lipoma and/or persistent tethering, the recordings of SEP and BAEP are useful to demonstrate a conduction deterioration along the ascending sensory pathway due to increasing tethering of the spinal cord due to somatic growth. PMID:22012627

Vidmer, Scaioli; Sergio, Curzi; Veronica, Saletti; Flavia, Tripaldi; Silvia, Esposito; Sara, Bulgheroni; Valentini, Laura Grazia; Daria, Riva; Solero, Carlo Lazzaro

2011-12-01

124

Resolution of cervical syringomyelia after transoral odontoidectomy and occipitocervical fusion in a patient with basilar invagination and Type I Chiari malformation.  

PubMed

We present a 16-year-old male patient with Marfan's syndrome who presented with quadriparesis from a Type I Chiari malformation (CM) with basilar invagination and a syrinx. The condition resolved after transoral odontoidectomy and occipitocervical fusion without posterior decompression of the CM. Thus, ventral decompression alone can resolve a cervical syrinx in patients with compression of the foramen magnum. PMID:22989794

Dickman, Curtis A; Kalani, M Yashar S

2012-09-16

125

A simple technique for expansive suboccipital cranioplasty following foramen magnum decompression for the treatment of syringomyelia associated with Chiari I malformation  

Microsoft Academic Search

The benefits of osteoplastic suboccipital craniotomies over the traditional suboccipital craniectomies have been recognized. We describe a simple method of expansive suboccipital cranioplastic craniotomy using a free bone flap and report satisfactory clinical results in 16 patients with syringomyelia associated with Chiari I malformation. A free suboccipital bone flap is created from the rostral part of the occiput by placing

Masakazu Takayasu; Teruhide Takagi; Masahito Hara; Masaoki Anzai

2004-01-01

126

Chiari I malformation associated with premature unilateral closure of the posterior intraoccipital synchondrosis in a preterm infant.  

PubMed

The authors report a case of a preterm infant at 29 weeks of gestation who gradually developed a Chiari malformation Type I (CM-I) with hydrocephalus due to a premature unilateral fusion of the posterior intraoccipital synchondrosis. Brain ultrasonography results in the 1st week of life were normal. Follow-up ultrasonography showed progressive development of triventricular hydrocephalus. Brain MRI demonstrated the presence of a CM-I and a deformation of the occipital bone. A complementary CT scan was obtained, showing a closure of the right posterior intraoccipital synchondrosis, resulting in a deformation of the posterior cranial fossa. This case shows the close relationship between a malformation of the skull base and the secondary development of a brain malformation. The authors discuss the anatomy of the occipital bone and suggest a probable theory for the premature closure of this synchondrosis and the consequent development of a CM-I. The originality of this case lies in the observation of the natural history of a brain malformation in a preterm infant. PMID:23581638

Kanavaki, Aikaterini; Jenny, Benoit; Hanquinet, Sylviane

2013-04-12

127

Decompression for Chiari type I-malformation ( with or without syringomyelia ) by extreme lateral foramen magnum opening and expansile duraplasty with arachnoid preservation: comparison with other technical modalities (Literature review)  

Microsoft Academic Search

Posterior craniocervical decompression is the procedure most currently used for treating Chiari I malformation (alone or in\\u000a association with syringomyelia in the absence of hydrocephalus). We reviewed the various technical modalities reported in\\u000a the literature. We present a personal series of 44 patients harboring Chiari type I malformation (CM-I) operated with a suboccipital\\u000a craniectomy and a C1 (or C1\\/C2) laminectomy,

M. Sindou; E. Gimbert

128

Syringomyelia Associated with Type I Chiari Malformation A 21Year Retrospective Study on 75 Cases Treated by Foramen Magnum Decompression with a Special Emphasis on the Value of Tonsils Resection  

Microsoft Academic Search

Summary  ?The purpose of the present study is to evaluate retrospectively the effects of several intra-operative manipulations on the\\u000a results of foramen magnum decompression (FMD) in patients having syringomyelia associated with type I Chiari malformation.\\u000a Seventy-five patients having syringomyelia associated with Chiari I malformation were operated on between 1975 and 1996. This\\u000a population was grouped into 4 subgroups according to the

J. Guyotat; P. Bret; E. Jouanneau; A.-C. Ricci; C. Lapras

1998-01-01

129

Placement of occipital condyle screws for occipitocervical fixation in a pediatric patient with occipitocervical instability after decompression for Chiari malformation.  

PubMed

In cadaveric studies and recently in one adult patient the occipital condyle has been studied as an option to allow bone purchase by fixation devices. In the current case the authors describe the use of occipital condyle screws in a child undergoing occipitocervical fixation. To the best of the authors' knowledge this case is the first reported instance of this technique in a pediatric patient. This girl had a history of posterior fossa decompression for Chiari malformation Type I when she was 22 months of age. When she was 6 years old she presented with neck pain on flexion and extension of her head. Magnetic resonance imaging in flexion and extension revealed occipitocervical instability. She underwent an occiput to C-2 posterior arthrodesis with bilateral screw placement in the occipital condyles, C-2 lamina, and C-1 lateral masses. Postoperatively, she was neurologically intact. Computed tomography demonstrated a stable construct, and her cervical pain had resolved on follow-up. PMID:20672939

Bekelis, Kimon; Duhaime, Ann-Christine; Missios, Symeon; Belden, Clifford; Simmons, Nathan

2010-08-01

130

Posterior fossa decompression with tonsillectomy in 104 cases of basilar impression, Chiari malformation and/or syringomyelia.  

PubMed

The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM) and/or syringomyelia (SM) is based on the restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction through the creation of a large artificial cisterna magna. A small suboccipital craniectomy has been emphasized to avoid caudal migration of the hindbrain structures into the vertebral canal. Nevertheless, the results showed downward migration of the hindbrain related to that type of craniectomy. The authors present, otherwise, the results of 104 cases of BI, CM and/or SM, whose surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. A significant upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging. PMID:22042188

da Silva, José Alberto Gonçalves; dos Santos, Adailton Arcanjo; Melo, Luiz Ricardo Santiago; de Araújo, Antônio Fernandes; Regueira, Giseuda Pessoa

2011-10-01

131

Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature  

PubMed Central

Introduction Cystic fibrosis, an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator gene, is not generally associated with malformations of the central nervous system. We review eight previously published reports detailing an infrequent association between cystic fibrosis and Chiari I malformation. Case presentation To the best of our knowledge, our report describes only the ninth case of a baby presenting with a new diagnosis of cystic fibrosis and Chiari I malformation, in this case in a 10-month-old, full-term Caucasian baby boy from the United States of America. Neurosurgical consultation was obtained for associated developmental delay, macrocephaly, bulging anterior fontanel, and papilledema. An MRI scan demonstrated an extensive Chiari I malformation with effacement of the fourth ventricle, obliteration of the outlets of the fourth ventricle and triventricular hydrocephalus without aqueductal stenosis. Our patient was taken to the operating room for ventriculoperitoneal shunt placement. Conclusions It is possible that the cystic fibrosis transmembrane conductance regulator gene may play a previously unrecognized role in central nervous system development; alternatively, this central nervous system abnormality may have been acquired due to constant valsalva from recurrent coughing or wheezing or metabolic and electrolyte imbalances that occur characteristically in cystic fibrosis.

2011-01-01

132

Cognitive and functional outcome in spina bifida–Chiari II malformation  

Microsoft Academic Search

Purpose  The long-term outcome in spina bifida–Chiari II–hydrocephalus complex is poorly understood. Traditional neurosurgical outcome\\u000a measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health,\\u000a disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  Adult patients under routine follow-up were assessed in a joint neurosurgery\\/neuropsychology clinic. Patients completed lifestyle\\u000a questionnaires, the hydrocephalus

Michael D. Jenkinson; Sophie Campbell; Caroline Hayhurst; Simon Clark; Jothy Kandasamy; Maggie K. Lee; Ann Flynn; Peter Murphy; Conor L. Mallucci

2011-01-01

133

Three-Dimensional Computerized Tomography in the Presurgical Evaluation of Chiari Malformations  

Microsoft Academic Search

Summary  \\u000a Malformations of the cranio-cervical junction represent a complex entity, comprising neuro-meningeal and bone anomalies. Malformations\\u000a of the central nervous system are nowadays easily explored using magnetic resonance imaging (MRI), while osseous malformations\\u000a are classically assessed with standard radiographic techniques, which can give only incomplete information, and are often\\u000a difficult to realize and analyse.\\u000a \\u000a \\u000a \\u000a We report a retrospective study of

H. Duffau; M. Sahel; J.-P. Sichez; B. Marro

1998-01-01

134

Chiari I malformation, delayed gross motor skills, severe speech delay, and epileptiform discharges in a child with FOXP1 haploinsufficiency.  

PubMed

Human FOXP2 deficiency has been identified as a cause of hereditary developmental verbal dyspraxia. Another member of the same gene family, FOXP1, has expression patterns that overlap with FOXP2 in some areas of the brain, and FOXP1 and FOXP2 have the ability to form heterodimers. These findings suggest the possibility that FOXP1 may also contribute to proper speech development. However, no such role of FOXP1 has been established to date. Recently, a child was reported who presented with a 3p13-14.1 deletion of four genes, including FOXP1, and a constellation of deficits that included speech delay. In this study, we report the case of a patient with a single deletion of FOXP1. This patient presented with speech and motor developmental delays, a Chiari I malformation, and epileptiform discharges. The nature of the speech deficit is different from the primary oromotor verbal dyspraxia found in patients with FOXP2 deficiency. The patient's developmental deficits may support a role for FOXP1 in the development of verbal and motor skills. PMID:20571508

Carr, Christopher W; Moreno-De-Luca, Daniel; Parker, Colette; Zimmerman, Holly H; Ledbetter, Nikki; Martin, Christa Lese; Dobyns, William B; Abdul-Rahman, Omar A

2010-06-23

135

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

PubMed Central

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.

2012-01-01

136

MRI-based Morphometric Analysis of Posterior Cranial Fossa in the Diagnosis of Chiari Malformation Type I.  

PubMed

BACKGROUND AND PURPOSE: The diagnosis of Chiari malformation type I (CMI) relies on MRI identification of a tonsillar descent (TD) through the foramen magnum, reflecting the overcrowding of an underdeveloped posterior cranial fossa (PCF). However, TD occurs in some patients with normal-sized PCF and, conversely, some patients with borderline or no TD have small PCF. We thus sought to identify a set of prototypic PCF measures for the diagnosis of CMI. METHODS: We performed nineteen measurements of the PCF on sagittal MRI of 100 cases with cerebellar TD ?5 mm and 50 control individuals, compared the average values in both cohorts and used logistic regression to devise a probability model to predict CMI status. RESULTS: Significant decrements were detected for several PCF-related measures in the patients' cohort. We developed a probability model that combined seven of these parameters to predict diagnosis with 93% sensitivity and 92% specificity. CONCLUSIONS: The addition of simple morphometric measurements in the diagnostic work-up of patients with suspected CMI may facilitate radiological diagnosis. Moreover, identification of the subset of CMI that arise from basichondrocranium underdevelopment is important for both, selection of the most appropriate therapeutic approach as well as proper CMI categorization in research studies. PMID:23324118

Urbizu, Aintzane; Poca, Maria-Antonia; Vidal, Xavier; Rovira, Alex; Sahuquillo, Juan; Macaya, Alfons

2013-01-16

137

The complex Chiari: issues and management strategies.  

PubMed

"Complex Chiari" malformations may be defined as cerebellar tonsil herniation combined with one or more of the following radiographic findings: brainstem herniation through the foramen magnum (Chiari 1.5 malformation), medullary kink, retroflexed odontoid, abnormal clival-cervical angle, occipitalization of the atlas, basilar invagination, syringomyelia or scoliosis. Patients with "complex Chiari" malformation are different from those with typical Chiari 1 malformation in that their management strategy has a higher chance of including other operative interventions aside from a typical suboccipital decompression. These other operative procedures include odontoid resection and craniocervical fusion. This paper outlines specific scenarios where these other operative procedures must be considered and carried out. PMID:21822705

Brockmeyer, Douglas L

2011-12-01

138

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

PubMed Central

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.

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

2013-01-01

139

Hydrodynamic and longitudinal impedance analysis of cerebrospinal fluid dynamics at the craniovertebral junction in type I Chiari malformation.  

PubMed

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

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

2013-10-10

140

Chiari Malformation: Treatment  

MedlinePLUS

... technique which involves rebuilding bony support for the cerebellum while still maintaining an adequate decompression. Complications 12 ... of the more serious complications, which involves the cerebellum slumping down even further into the spinal area ...

141

Syringomyelia associated with Chiari I malformation treated with foramen magnum decompression and duraplasty using a polyglycolic acid patch and fibrin glue: a case report.  

PubMed

A 31-year-old woman presented with worsening numbness and pain in the arms and chest. Neurological findings at admission were decreased pain sensation and temperature sensation in the arms and chest. Magnetic resonance demonstrated a large cervical syrinx from the level of C4 to Th4 associated with Chiari I malformation. Occipital craniectomy and C1 laminectomy were performed for foramen magnum decompression. Intraoperative ultrasonography, performed after removal of the outer membrane of the dura mater at the level of the foramen magnum, revealed insufficient decompression. Therefore, the dura mater was completely opened and duraplasty was performed with a polyglycolic acid patch and fibrin glue. Sufficient decompression was thus achieved. The neurological symptoms and signs improved within the first postoperative month, and magnetic resonance showed a decrease in the size of the syrinx and no cerebrospinal fluid leakage. In patients undergoing foramen magnum decompression with duraplasty, the use of a polyglycolic acid patch and fibrin glue renders suturing unnecessary and avoids the common complications associated with suture duraplasty. PMID:20818142

Sugawara, Atsushi; Isu, Toyohiko; Kim, Kyongsong; Matsumoto, Ryoji; Isobe, Masanori; Ogasawara, Kuniaki

2010-08-01

142

Cranial malformations in related white lions (Panthera leo krugeri).  

PubMed

White lions (Panthera leo krugeri) have never been common in the wild, and at present, the greatest population is kept in zoos where they are bred for biological and biodiversity conservation. During the years 2003 to 2008 in a zoological garden in northern Italy, 19 white lions were born to the same parents, who were in turn paternally consanguineous. Out of the 19 lions, 4 (21%) were stillborn, 13 (69%) died within 1 month, and 1 (5%) was euthanatized after 6 months because of difficulty with prehension of food. Six lions (32%) showed malformations involving the head (jaw, tongue, throat, teeth, and cranial bones). One lion (5%) still alive at 30 months revealed an Arnold-Chiari malformation upon submission for neurological evaluation of postural and gait abnormalities. Paternal consanguinity of the parents, along with inbreeding among white lions in general, could account for the high incidence of congenital malformations of the head in this pride of white lions. PMID:20826844

Scaglione, F E; Schröder, C; Degiorgi, G; Zeira, O; Bollo, E

2010-09-08

143

CranioCervical Decompression for Chiari Type I-Malformation, Adding Extreme Lateral Foramen Magnum Opening and Expansile Duroplasty with Arachnoid Preservation. Technique and Long-Term Functional Results in 44 Consecutive Adult Cases – Comparison with Literature Data  

Microsoft Academic Search

Summary.  \\u000a ?Background: Posterior cranio-cervical decompression by opening at least foramen magnum and C1-lamina usually with corresponding dural\\u000a and arachnoid opening, is the procedure most currently used for treating Chiari I malformation (alone or in association with\\u000a syringomyelia). To optimize decompressive effects together with reducing risks, a procedure was developed which consists of\\u000a a sub-occipital craniectomy and a C1 (or C1\\/C2)

M. Sindou; J. Chávez-Machuca; H. Hashish

2002-01-01

144

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

PubMed Central

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 multiple, distinct mechanisms are responsible for CMI.

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

2013-01-01

145

Effect of symptomatic pseudomeningocele on improvement in pain, disability, and quality of life following suboccipital decompression for adult Chiari malformation Type I.  

PubMed

Object Suboccipital decompression is a common procedure for patients with Chiari malformation Type I (CMI). Published studies have reported complication rates ranging from 3% to 40%, with pseudomeningocele being one of the most common complications. To date, there are no studies assessing the effect of this complication on long-term outcome. Therefore, the authors set out to assess the effect of symptomatic pseudomeningocele on patient outcomes following suboccipital decompression for CM-I. Methods The study comprised 50 adult patients with CM-I who underwent suboccipital craniectomy and C-1 laminectomy with or without duraplasty. Clinical presentation, radiological studies, operative variables, and complications were assessed for each case. Baseline and 1-year postoperative patient-reported outcomes were assessed to determine improvement in pain, disability, and quality of life. The extent of improvement was compared for patients with and without development of a postoperative symptomatic pseudomeningocele. Results A symptomatic pseudomeningocele developed postoperatively in 9 patients (18%). There was no difference with regard to clinical, radiological, or operative variables for patients with or without a postoperative pseudomeningocele. Patients without a pseudomeningocele had significant improvement in all 9 patient-reported outcome measures assessed. On the other hand, patients with pseudomeningocele only had significant improvement in headache (as measured on the Numeric Rating Scale) and headache-related disability (as measured on the Headache Disability Index) but no improvement in quality of life. Twenty-nine (71%) of 41 patients without a pseudomeningocele reported improvement in health status postoperatively compared with only 3 (33%) of 9 patients with a postoperative pseudomeningocele (p = 0.05). Conclusions Surgical management of CM-I in adults provides significant and sustained improvement in pain, disability, general health, and quality of life. Development of a postoperative symptomatic pseudomeningocele has lingering effects at 1 year, and it significantly diminishes the overall benefit of suboccipital decompression for CM-related symptoms. Further research is needed to accurately predict which patients may benefit from decompression alone without duraplasty. PMID:24010974

Parker, Scott L; Godil, Saniya S; Zuckerman, Scott L; Mendenhall, Stephen K; Tulipan, Noel B; McGirt, Matthew J

2013-09-06

146

Conquer Chiari  

MedlinePLUS

... in quicker diagnoses for children. Click on Parker, Gracie Claire and Zoey's picture to enter our pediatric ... literature and support. Chiari Siblings - Parker, age 5 | Gracie Claire, age 6 | Zoey, age 7 CCWAA '13 ...

147

Chiari Drop Attacks: Surgical Decompression and the Role of Tilt Table Testing  

Microsoft Academic Search

Background: Chiari I malformation (CM1) is characterized by impaired CSF flow through the foramen magnum. Dysfunctional autonomic cardiovascular regulation may result in syncope. Syncope may be the primary presenting symptom of CM1: a syndrome termed Chiari drop attack. It has been postulated that Chiari drop attack is secondary to dysautonomia caused by hindbrain compression. There has been recent debate regarding

David Straus; Kimberly Foster; Frank Zimmerman; David Frim

2009-01-01

148

Symptomatic Subdural Hygroma and Hydrocephalus following Chiari I Decompression  

Microsoft Academic Search

Background: The aim of the present case report is to describe an unusual complication of foramen magnum decompression (FMD) for the Chiari 1 hindbrain malformation and its successful management with non-operative measures. Methods: A 2-year-old girl with the Chiari 1 malformation underwent FMD, including suboccipital craniotomy, C1 laminectomy and durotomy without opening the arachnoid. Results: After initial postoperative improvement, the

Andreas K. Filis; Karam Moon; Alan R. Cohen

2009-01-01

149

Arnold Hague's Saddle  

USGS Multimedia Gallery

This is Arnold Hague's saddle. The saddle is likely about 120 years old and in the western style with a sheepskin liner. Arnold Hague used this saddle on his expeditions in the west. Object ID: USGS-000015...

2009-07-22

150

Arnold’s Treason: The French Connection  

Microsoft Academic Search

This paper speculates on Benedict Arnold's hatred of the French as a cause of his treason. It includes the text of Arnold's Proclamation ... To all the officers and soldiers of the continental army which is presented as an apologia for his treason. It also includes an editorial comment from the first issue of the Gazette Francoise published in Newport,

Norman Desmarais

2005-01-01

151

C1–C2 arthrodesis after transoral odontoidectomy and suboccipital craniectomy for ventral brain stem compression in Chiari I patients  

Microsoft Academic Search

Chiari I malformations are often associated with congenital craniocervical anomalies such as platybasia, basilar invagination,\\u000a and retroflexion of the odontoid process. Management of ventral brain stem compression associated with Chiari I malformations\\u000a remains controversial, but several authors report a significant rate of failure with suboccipital decompression alone in the\\u000a presence of pronounced ventral brain stem compression (VBSC). Treatment options described

Steven W. Hwang; Carl B. Heilman; Ron I. Riesenburger; James Kryzanski

2008-01-01

152

Basilar invagination, Chiari malformation, syringomyelia: a review.  

PubMed

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

Goel, Atul

153

Matthew Arnold and Rereading  

Microsoft Academic Search

This essay probes the general topic of how an idea of rereading a text and seeing it differently from previous readings can be accommodated in rigorous criticism without slipping into the assertion that texts are merely radically unstable and meaning subjective. It surveys the absence of serious analyses of this problem and compares what has been said with Matthew Arnold's

Francis OGorman

2012-01-01

154

Primary Craniovertebral Anomalies and the Hindbrain Herniation Syndrome (Chiari I): Data Base Analysis  

Microsoft Academic Search

This prospective study analyzes 100 patients with Chiari malformation and primary craniovertebral junction (CVJ) anomalies (3–66 years). Neurodiagnostic investigations employed tomography, gas myelography, CT and CT myelography, and MRI. Factors considered were reducibility, mode of encroachment, cerebrospinal fluid (CSF) dynamics and syringohydromyelia. Sixty-six patients with irreducible pathology underwent ventral or ventrolateral decompression and dorsal stabilization. Dorsal occipitocervical fixation was performed

Arnold H. Menezes

1995-01-01

155

Arnold Schoenberg Center  

NSDL National Science Digital Library

Perhaps best remembered for his musical forays into the world of atonality, Arnold Schoenberg remains at the focal point of this particular Web site, sponsored by the Arnold Schoenberg Center. The site contains an amazing amount of material on the composer, ranging from an extended biographical essay to a complete listing of his compositions, complete with first performance date, location, and other germane information. The site also contains material on the mission of the Center (located in Vienna) and links to information about using the archives at the Center, which contain a great deal of Schoenberg's correspondence and personal effects. The site also has a section where visitors can purchase Schoenberg related material, such as audio recordings and the journal published by the Center.

156

1900 - Arnolds Arrhythmia  

Microsoft Academic Search

This essay considers Matthew Arnold’s “Empedocles on Etna” (1852) and the piece that displaced it, “Preface to Poems” (1853), as halves of a single fractured document. It argues that in his turn from poetry to critical prose, Arnold never abandoned the problem of verse, but rather developed a figurative and formal prosaics designed to transmit poetic rhythms intermittently. Accordingly, it

C. D. Blanton

2008-01-01

157

Angular craniometry in craniocervical junction malformation.  

PubMed

The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences in the several types of craniocervical junction malformation. The objectives of this study were to evaluate craniometric angles compared with normal subjects and elucidate the main angular differences among the types of craniocervical junction malformation and the correlation between craniocervical and cervical angles. Angular craniometries were studied using primary cranial angles (basal and Boogard's) and secondary craniocervical angles (clivus canal and cervical spine lordosis). Patients with basilar invagination had significantly wider basal angles, sharper clivus canal angles, larger Boogard's angles, and greater cervical lordosis than the Chiari malformation and control groups. The Chiari malformation group does not show significant differences when compared with normal controls. Platybasia occurred only in basilar invagination and is suggested to be more prevalent in type II than in type I. Platybasic patients have a more acute clivus canal angle and show greater cervical lordosis than non-platybasics. The Chiari group does not show significant differences when compared with the control, but the basilar invagination groups had craniometric variables significantly different from normal controls. Hyperlordosis observed in the basilar inavagination group was associated with craniocervical kyphosis conditioned by acute clivus canal angles. PMID:23640096

Botelho, Ricardo Vieira; Ferreira, Edson Dener Zandonadi

2013-05-03

158

Delamination technique together with longitudinal incisions for treatment of Chiari I\\/syringomyelia complex: a prospective clinical study  

Microsoft Academic Search

BACKGROUND: Treatment modalities in Chiari malformation type 1(CMI) accompanied by syringomyelia have not yet been standardized. Pathologies such as a small posterior fossa and thickened dura mater have been discussed previously. Various techniques have been explored to enlarge the foramen magnum and to expand the dura. The aim of this clinical study was to explore a new technique of excision

Kadir Kotil; Tu?rul Ton; Rabia Tari; Yildiray Savas

2009-01-01

159

Comparison of dural grafts in Chiari decompression surgery: Review of the literature  

PubMed Central

Background: Decompression of Chiari malformation is a common procedure in both pediatric and adult neurosurgery. Although the necessity for some bony removal is universally accepted, other aspects of Chiari surgery are the subject of debate. The most controversial points include the optimal amount of bony removal, the use of duraplasty (and the type of material), the need for subarachnoid dissection, and the need for tonsillar shrinkage. Material and Methods: We critically reviewed the literature to elucidate the risks and benefits of different graft types and to clarify optimal treatment options therein. Based on our search results, 108 relevant articles were identified. With specific inclusion and exclusion criteria, we noted three studies that directly compared two tlpes of dural substitutes in Chiari malformation surgery. Results: Our review did not support the superiority of either autologous or nonautologous grafts when duraplasty is employed. Our institutional experience, however, dictates that when the pericranium is available and of good quality, it should be utilized for duraplasty. It is non-immunogenic, inexpensive, and capable of creating a watertight closure with the dura. Conclusions: Discrepancies between the three comparative studies analyzed are likely attributable to increases in pericranial quality and thickness with maturity. Future randomized studies with large numbers and the power to resolve differences in the relatively low rates of complications in Chiari surgery are warranted.

Abla, Adib A.; Link, Timothy; Fusco, David; Wilson, David A.; Sonntag, Volker K.H.

2010-01-01

160

Concurrent Chiari decompression and spinal cord untethering in children: feasibility in a small case series  

Microsoft Academic Search

Background and purpose  We describe the rationale and safety of concurrent decompression of Chiari type 1 malformation (CM1) and untethering of the\\u000a spinal cord. Spinal cord traction is considered one of the pathogenic mechanisms involved in the development of CM, and 14%\\u000a of patients with CM1 have tethered cord syndrome (Milhorat et al., Surg Neurol 7:20–35, 2009; Roth, Neuroradiology 21:133–138, 1981;

Vicko Gluncic; Michael Turner; Delilah Burrowes; David Frim

2011-01-01

161

Magda B. Arnold's contributions to emotions research  

Microsoft Academic Search

This paper provides an overview to the special issue of Cognition and Emotion devoted to Magda B. Arnold's (1903–2002) contributions to the psychology of human emotion. Contributors provide insight into the intellectual forebears and theoretical scope of Arnold's emotion theory, and the interconnections of Arnold's theories and contemporary trends in research on emotion, motivation, and social neuroscience. A biography of

Stephanie A. Shields; Arvid Kappas

2006-01-01

162

Pathogenesis of cerebral malformations in human fetuses with meningomyelocele  

PubMed Central

Background Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation) may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA) with MMC and 6 fetuses with normal cerebral development (22–41 week GA) were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US). After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation and/or hydrocephalus. Since denuded areas cannot re-establish cell function, neuro-developmental consequences could induce permanent cerebral pathology.

de Wit, Olga A; den Dunnen, Wilfred FA; Sollie, Krystyne M; Munoz, Rosa Iris; Meiners, Linda C; Brouwer, Oebele F; Rodriguez, Esteban M; Sival, Deborah A

2008-01-01

163

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. Types of brain malformations include missing parts ...

164

Arnold Hely and Australian Adult Education  

ERIC Educational Resources Information Center

|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…

Morris, Roger

2011-01-01

165

ARNOLD MESA ROADLESS AREA, ARIZONA.  

USGS Publications Warehouse

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.

Wolfe, Edward, W.; McColly, Robert, A.

1984-01-01

166

American Syringomyelia & Chiari Alliance Project  

MedlinePLUS

... Malformation • Syringomyelia • Newsletter • Medical Articles • Related Disorders • BJO Scholarships • Patient Handbook • Get Free Info • BJO Equipment • Online ... Professional Login • Newsletter • Syringomyelia • Create an Account • BJO Scholarships • Medical Articles • BJO Equipment • Related Disorders • Parent Resources • ...

167

Antithetical Developments in the Poetry and Criticism of Matthew Arnold  

Microsoft Academic Search

My thesis involves a study of a development in Arnold's poetry that is antithetical to the development of Arnold's prose. At first, Arnold's poetry preached an active, moral involvement in the world. This stance gradually collapsed, and, from quite early in his career, Arnold's poetry is characterized by detachment and withdrawal from life.\\u000aArnold's literary criticism exhibits an opposite movement.

Donald Bradley Adams

1981-01-01

168

HAP: Henry H. Arnold, Military Aviator.  

National Technical Information Service (NTIS)

Henry H. (Hap) Arnold championed the cause of military air power for over 40 years. This book offers a synopsis of his career and achievements from his flight training by the Wright brothers through leadership in World War II.

R. G. Davis

1997-01-01

169

Arnold gesell and the maturation controversy  

Microsoft Academic Search

This article examines the work of Arnold Lucius Gesell and argues that he not only paved the way for contemporary research\\u000a in motor development, but that he and colleagues anticipated fundamental issues about growth that must be addressed by psychologists\\u000a and neuroscientists who are committed to the advancement of developmental science. Arnold Lucius Gesell was a pioneer in developmental\\u000a psychology

Thomas C. Dalton

2005-01-01

170

C1-C2 arthrodesis after transoral odontoidectomy and suboccipital craniectomy for ventral brain stem compression in Chiari I patients.  

PubMed

Chiari I malformations are often associated with congenital craniocervical anomalies such as platybasia, basilar invagination, and retroflexion of the odontoid process. Management of ventral brain stem compression associated with Chiari I malformations remains controversial, but several authors report a significant rate of failure with suboccipital decompression alone in the presence of pronounced ventral brain stem compression (VBSC). Treatment options described in the literature for these patients involve anterior, posterior, or combined decompressions with or without concurrent arthrodesis. A combined anterior and posterior approach provides a definitive circumferential decompression but also significantly disrupts the stability of the occipitocervical junction usually necessitating occipitocervical fixation. We describe an alternative surgical treatment for Chiari I patients with significant ventral brain stem compression where a combined anterior and posterior decompression was considered necessary. We report two patients who underwent transoral odontoidectomy with preservation of the anterior arch of the atlas and suboccipital craniectomy with C1 laminectomy followed by C1-C2 arthrodesis. Preservation of the anterior arch of the atlas in conjunction with C1-C2 arthrodesis stabilizes the occipito-atlanto-axial segments while conserving more cervical mobility as compared to an occipitocervical fusion. PMID:18629549

Hwang, Steven W; Heilman, Carl B; Riesenburger, Ron I; Kryzanski, James

2008-07-16

171

C1-C2 arthrodesis after transoral odontoidectomy and suboccipital craniectomy for ventral brain stem compression in Chiari I patients  

PubMed Central

Chiari I malformations are often associated with congenital craniocervical anomalies such as platybasia, basilar invagination, and retroflexion of the odontoid process. Management of ventral brain stem compression associated with Chiari I malformations remains controversial, but several authors report a significant rate of failure with suboccipital decompression alone in the presence of pronounced ventral brain stem compression (VBSC). Treatment options described in the literature for these patients involve anterior, posterior, or combined decompressions with or without concurrent arthrodesis. A combined anterior and posterior approach provides a definitive circumferential decompression but also significantly disrupts the stability of the occipitocervical junction usually necessitating occipitocervical fixation. We describe an alternative surgical treatment for Chiari I patients with significant ventral brain stem compression where a combined anterior and posterior decompression was considered necessary. We report two patients who underwent transoral odontoidectomy with preservation of the anterior arch of the atlas and suboccipital craniectomy with C1 laminectomy followed by C1–C2 arthrodesis. Preservation of the anterior arch of the atlas in conjunction with C1–C2 arthrodesis stabilizes the occipito–atlanto-axial segments while conserving more cervical mobility as compared to an occipitocervical fusion.

Heilman, Carl B.; Riesenburger, Ron I.; Kryzanski, James

2008-01-01

172

[Anorectal malformations].  

PubMed

Anorectal malformations (ARM) are the result of an abnormal development of the terminal part of the digestive tract interesting anus and/or rectum that occur early between the sixth and tenth week of embryonic development. They carry a malformation spectrum of severity depending on the level of disruption of the anorectal canal and of the associated caudal malformations (sacrum and spine). ARM are associated in over half the cases with other malformations that can be integrated in some cases in known syndromes. If surgical treatment to restore anatomy as normal as possible is indispensable, post-operative care is essential for these patients whose defecation mechanisms are altered, to reach if not continence, at least a socially acceptable cleanliness. PMID:23992833

Cretolle, C; Rousseau, V; Lottmann, H; Irtan, S; Lortat-Jacob, S; Alova, I; Michel, J L; Aigrain, Y; Podevin, G; Lehur, P A; Sarnacki, S

2013-09-01

173

The Budd-Chiari syndrome in pregnancy  

Microsoft Academic Search

A case of Budd-Chiari syndrome in a young woman, which started probably in the last trimester of pregnancy, is described. The diagnosis was made clinically and was confirmed by inferior venacavography and on exploratory laparotomy. The possible connection of the syndrome with the pregnancy is discussed.

M. Tiliacos; D. Tsantoulas; A. Tsoulias; E. Kokka; E. Eudaimon; S. Aphentoglou; C. Metzantonakis

1978-01-01

174

Congratulations to Vladimir Igorevich Arnol'd  

NASA Astrophysics Data System (ADS)

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.

2007-06-01

175

Prosocial Youth: The Legacy of Arnold Goldstein  

ERIC Educational Resources Information Center

|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,…

Amendola, Mark; Oliver, Robert

2008-01-01

176

Surgical management of syringomyelia–Chiari complex  

Microsoft Academic Search

Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia–Chiari complex.\\u000a More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique\\u000a opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This\\u000a report presents 18 consecutive adult symptomatic syringomyelia

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

2000-01-01

177

Clinical and radiological outcome of craniocervical osteo-dural decompression for Chiari I-associated syringomyelia.  

PubMed

The aim of this study was to analyze the long-term clinical and radiological outcomes of craniocervical decompression for patients affected by Chiari I-related syringomyelia. We performed a retrospective analysis of a group of patients affected by Chiari I-associated syringomyelia treated by craniocervical decompression (CCD). Surgical and technical aspects and preoperative factors predicting outcome were discussed. A total of 36 patients were reviewed. There were 17 men and 19 women (female/male ratio 1.11), and the mean age was 40.4 (range 18-68). The most important preoperative symptoms were related to myelopathy (pain, weakness, atrophy, spasticity, sensory loss, and dysesthesias). Most syrinxes were in the cervico-thoracic region (61.1%), and the majority of patients had tonsillar descent between the foramen magnum and C1. All patients underwent a craniectomy less than 3 cm in diameter followed by a duroplasty with dura substitute. No arachnoid manipulation was necessary. Three patients (8.1%) experienced cerebrospinal fluid leaks that resolved without complications. At a mean follow up of 40 months (range 16-72) 80.5% of patients exhibited improvement over their preoperative neurological examination while 11.1% stabilized. The syrinx shrank in 80.5% of patients. Chi-square test showed that preoperative syrinx extension and degree of tonsillar descent did not correlate with clinical and neuroradiological postoperative evolution. Treating syringomyelia associated in Chiari I malformation with CCD leads to a large percentage of patients with satisfying results and no irreversible complications. PMID:20437070

Spena, Giannantonio; Bernucci, Claudio; Garbossa, Diego; Valfrč, Walter; Versari, Pietro

2010-05-01

178

Budd-Chiari syndrome: illustrated review of current management: Budd-Chiari review  

Microsoft Academic Search

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruc- tion at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

2008-01-01

179

The Arnold Arboretum of Harvard University: Plants  

NSDL National Science Digital Library

The Arnold Arboretum of Harvard University provides this in-depth Web site featuring plants found at the Arboretum and related information. Visitors to this site will find sections featuring selected plants at the Arboretum, collections management, on-site projects, a plant-information hotline, and a detailed table listing bloom times for dozens of plants; guidelines for adjusting dates for localities beyond New England are provided. The Featured Plants section, for example, includes a look at 18 of the Arboretum's 600+ trees and shrubs over 100 years old and a detailed introduction to the art of bonsai. Plant lovers anywhere should enjoy this interesting and nicely presented Web site.

2003-01-01

180

Arnold Gesell and the maturation controversy.  

PubMed

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

Dalton, Thomas C

181

Arnold B. Arons (1916-2001)  

NASA Astrophysics Data System (ADS)

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.

Warren, Bruce A.

182

In utero Repair of Myelomeningocele: Rationale, Initial Clinical Experience and a Randomized Controlled Prospective Clinical Trial  

Microsoft Academic Search

Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Arnold-Chiari II malformation, incontinence, sexual dysfunction, skeletal deformations, and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Studies in animals provide compelling evidence that the primary cause of the neurological deficit associated with MMC is not

Enrico Danzer; Alan W. Flake

2008-01-01

183

32. Historic American Buildings Survey, Arnold Moses, Photographer February 23, ...  

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

32. Historic American Buildings Survey, Arnold Moses, Photographer February 23, 1937, DETAIL OF CARVED SWAG ON PULPIT, CLERK'S DESK. - St. Paul's Chapel, Broadway & Fulton Streets, New York, New York County, NY

184

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

Federal Register 2010, 2011, 2012, 2013

...operation for the Duane Arnold Energy Center (DAEC). The DAEC is...the town of Palo. Possible alternatives to the proposed action (license...include no action and reasonable alternative energy sources. As discussed in Section...

2010-10-20

185

Budd-Chiari Syndrome Recurring in a Transplanted Liver  

PubMed Central

A patient with Budd-Chiari syndrome who underwent orthotopic liver transplantation and developed recurrent disease is described. The immediate postoperative period was complicated by multiple thrombotic episodes, followed by a period of apparent remission associated with the initiation of coumadin and persantine therapy. After discontinuation of such antithrombotic therapy in order to biopsy the liver, the patient experienced another series of clinically overt vascular thromboses and ultimately died of sepsis 15 mo posttransplantation after a prolonged and complicated terminal hospital course. At autopsy, recurrent Budd-Chiari syndrome as well as thromboses in numerous other organs was demonstrated.

SELTMAN, HOWARD J.; DEKKER, ANDREW; VAN THIEL, DAVID H.; BOGGS, DANE R.; STARZL, THOMAS E.

2010-01-01

186

[Budd-Chiari syndrome. Report of 2 surgical cases].  

PubMed

Two female patients with Budd-Chiari syndrome and suffering of Polycythemia Vera are reported. Both patients were operated performing a central spleno renal shunt with preservation of the spleen and ligation of the splenic artery. The patients are well 6 and 24 months after the operation respectively. The principal features and therapeutic alternatives of this condition are reviewed briefly. PMID:8248645

Llanos, O; Guzmán, S

1993-03-01

187

Interventional therapeutic techniques in Budd-Chiari syndrome  

Microsoft Academic Search

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

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

1997-01-01

188

Taking Chiari to School: A Guide for Parents  

MedlinePLUS

... C&S Patient Education Foundation. Fact sheets, access to social networking with other Chiari families, and opportunities to become ... APPROPRIATE PUBLIC EDUCATION FERPA FAMILY ... on Students' School Engagement and Performance. RMLE Online: Research In Middle Level Education , 31 (10), 1- ...

189

78 FR 7818 - Duane Arnold Energy Center; Application for Amendment to Facility Operating License  

Federal Register 2010, 2011, 2012, 2013

...NRC-2013-0022] Duane Arnold Energy Center; Application for Amendment...granted the request of NextEra Energy Duane Arnold, LLC (the licensee...for proposed amendment to Renewed Facility Operating License No. DPR-49 for the Duane Arnold Energy Center, located in...

2013-02-04

190

'Poetry is the Reality': Matthew Arnold Tackles the Athletes of Logic (and Theory)  

Microsoft Academic Search

For more than thirty years the ideas of Matthew Arnold have endured sustained and sometimes violent criticism at the hands of New Left and postcolonial thinkers. The roots of this criticism, however, are already discernible among Arnold's fellow Victorian men of letters. This essay traces the most prominent polemical tropes of the Arnold bashers from their nineteenth-century origin to their

James Walter Caufield

2010-01-01

191

'Poetry is the Reality': Matthew Arnold Tackles the Athletes of Logic (and Theory)  

Microsoft Academic Search

:For more than thirty years the ideas of Matthew Arnold have endured sustained and sometimes violent criticism at the hands of New Left and postcolonial thinkers. The roots of this criticism, however, are already discernible among Arnold's fellow Victorian men of letters. This essay traces the most prominent polemical tropes of the Arnold bashers from their nineteenth-century origin to their

James Walter Caufield

2010-01-01

192

Spinal arteriovenous malformation  

Microsoft Academic Search

An 11-year-old girl presented with acute onset of paraplegia with bowel and bladder incontinence. She had spinal tenderness\\u000a and a sensory level at mid thoracic region. MRI spine revealed an intradural extramedullary arterio-venous malformation with\\u000a hemorrhage into the intramedullary region. Hence a diagnosis of spinal arterio-venous malformation was entertained.

S. Balasubramanian; So. Shivbalan; Sandeep D. Jaipurkar

2004-01-01

193

Intramedullary spinalcord cavernous malformations  

Microsoft Academic Search

Intramedullary spinal cord cavernous malformations are rare lesions and account for a small percentage of clinically evident cavernous malformations. However, the reported incidence rates likely underestimate their true rate of occurrence. The advent of magnetic resonance imaging has increased the diagnosis of these lesions as it has that of their intracranial counterparts. Our understanding of their natural history, however, is

Frank P. K. Hsu; Richard E. Clatterbuck; Louis J. Kim; Robert F. Spetzler

2003-01-01

194

Mapping the Arnold web with a graphic processing unit  

NASA Astrophysics Data System (ADS)

The Arnold diffusion constitutes a dynamical phenomenon which may occur in the phase space of a non-integrable Hamiltonian system whenever the number of the system degrees of freedom is M >= 3. The diffusion is mediated by a web-like structure of resonance channels, which penetrates the phase space and allows the system to explore the whole energy shell. The Arnold diffusion is a slow process; consequently, the mapping of the web presents a very time-consuming task. We demonstrate that the exploration of the Arnold web by use of a graphic processing unit-supercomputer can result in distinct speedups of two orders of magnitude as compared with standard CPU-based simulations.

Seibert, A.; Denisov, S.; Ponomarev, A. V.; Hänggi, P.

2011-12-01

195

Cervical Myelocystocele with Chiari II Malformation: Magnetic Resonance Imaging and Surgical Treatment  

Microsoft Academic Search

BACKGROUNDThe myelocystocele is a rare clinical entity, and there have only been six case reports concerning the cervical myelocystocele, including ours.METHODSThe case of a female neonate with cervical myelocystocele, who presented with respiratory distress beginning at birth is reported.RESULTSMagnetic resonance (MR) imaging disclosed a cystic lesion surrounded by two layers of membrane, which were recognized to be the dural and

Akiko Nishino; Reizo Shirane; Keiten So; Hiroaki Arai; Hiroyoshi Suzuki; Yoshiharu Sakurai

1998-01-01

196

Medical management of eosinophilic meningitis following bovine graft duraplasty for Chiari malformation Type I repair.  

PubMed

Eosinophilic meningitis is a known complication of duraplasty, including that using bovine tissues. Previous authors have relied on surgical removal of the graft for treatment. Authors of the present report describe a 7-year-old girl with eosinophilic meningitis following duraplasty with a bovine pericardium graft who was successfully treated using corticosteroid therapy alone. PMID:23909615

Ostendorf, Adam P; Connolly, Anne M

2013-08-02

197

Automated Posterior Cranial Fossa Volumetry by MRI: Applications to Chiari Malformation Type I  

PubMed Central

BACKGROUND AND PURPOSE Quantification of PCF volume and the degree of PCF crowdedness were found beneficial for differential diagnosis of tonsillar herniation and prediction of surgical outcome in CMI. However, lack of automated methods limits the clinical use of PCF volumetry. An atlas-based method for automated PCF segmentation tailored for CMI is presented. The method performance is assessed in terms of accuracy and spatial overlap with manual segmentation. The degree of association between PCF volumes and the lengths of previously proposed linear landmarks is reported. MATERIALS AND METHODS T1-weighted volumetric MR imaging data with 1-mm isotropic resolution obtained with the use of a 3T scanner from 14 patients with CMI and 3 healthy subjects were used for the study. Manually delineated PCF from 9 patients was used to establish a CMI-specific reference for an atlas-based automated PCF parcellation approach. Agreement between manual and automated segmentation of 5 different CMI datasets was verified by means of the t test. Measurement reproducibility was established through the use of 2 repeated scans from 3 healthy subjects. Degree of linear association between PCF volume and 6 linear landmarks was determined by means of Pearson correlation. RESULTS PCF volumes measured by use of the automated method and with manual delineation were similar, 196.2 ± 8.7 mL versus 196.9 ± 11.0 mL, respectively. The mean relative difference of ?0.3 ± 1.9% was not statistically significant. Low measurement variability, with a mean absolute percentage value of 0.6 ± 0.2%, was achieved. None of the PCF linear landmarks were significantly associated with PCF volume. CONCLUSIONS PCF and tissue content volumes can be reliably measured in patients with CMI by use of an atlas-based automated segmentation method.

Bagci, A.M.; Lee, S.H.; Nagornaya, N.; Green, B.A.; Alperin, N.

2013-01-01

198

Frequency modulation indicator, Arnold’s web and diffusion in the Stark Quadratic-Zeeman problem  

NASA Astrophysics Data System (ADS)

We notice that the fundamental frequencies of a slightly perturbed integrable Hamiltonian system are not time-constant inside a resonance but frequency modulated, as is evident from pendulum models and wavelet analysis. Exploiting an intrinsic imprecision inherent to the numerical frequency analysis algorithm itself, hence transforming a drawback into an opportunity, we define the Frequency Modulation Indicator, a very sensitive tool in detecting where fundamental frequencies are modulated, localizing so the resonances without having to resort, as in other methods, to the integration of variational equations. For the Kepler problem, the space of the orbits with a fixed energy has the topology of the product of two 2-spheres. The perturbation Hamiltonian, averaged over the mean anomaly, has surely a maximum and a minimum, to which correspond two periodic orbits in physical space. Studying the neighbourhood of these two elliptic stable points, we are able to define adapted action-angle variables, for example, the usual but “SO(4)-rotated” Delaunay variables. The procedure, implemented in the program KEPLER, is performed transparently for the user, providing a general scheme suited for generic perturbation. The method is then applied to the Stark Quadratic-Zeeman problem, displaying very clearly the Arnold web of the resonances. Sectioning transversely one of the resonance strips so highlighted and performing a numerical frequency analysis, one is able to locate with great precision the thin stochastic layer surrounding a separatrix. Another very long (108 revolutions) frequency analysis on an orbit starting here reveals, as expected, a well defined pattern, which ensures that the integration errors do not eject the point out of the layer, and moreover a very slow drift in the frequency values, clearly due to Arnold diffusion.

Cordani, Bruno

2008-11-01

199

Resolution of life-threatening dysphagia caused by caudal occipital malformation syndrome following foramen magnum decompressive surgery.  

PubMed

A Cavalier King Charles Spaniel was presented with acute onset, life-threatening dysphagia suspected to be secondary to medulla oblongata compression caused by caudal occipital malformation syndrome. The patient required urgent tracheostomy tube placement to remain stable and was subsequently cured of the presenting neurological deficits by foramen magnum decompressive surgery. Neurogenic dysphagia is a relatively common presenting sign in human Chiari malformation syndromes, but has not been described as a major clinical sign in veterinary patients. Caudal occipital malformation syndrome should be included in the differential diagnosis list for susceptible breeds presenting with dysphagia. Early recognition favours expeditious surgical intervention and a positive outcome in human patients, and this may also be the case in veterinary patients. PMID:22827623

Graham, K J; Black, A P; Brain, P H

2012-08-01

200

Vascular malformations in childhood.  

PubMed

Vascular malformations are inborn errors of vascular embryogenesis present at birth that should be diagnosed in childhood and, when necessary, treated to prevent later complications. The current trend is to classify these lesions according to flow characteristics and the predominant type of vascular channel affected. Given the complexity, and in many cases, the rarity, of vascular malformations, they should be managed by multidisciplinary teams at vascular anomalies centers. Furthermore, because the association between vascular malformations and certain syndromes is becoming increasingly recognized, a better understanding of these lesions will help to improve overall patient management in this setting. PMID:22483320

Del Pozo, J; Gómez-Tellado, M; López-Gutiérrez, J C

2012-04-06

201

Marketing muscular masculinity in Arnold: the education of a bodybuilder  

Microsoft Academic Search

This article examines Arnold Schwarzenegger's ability to cross the wide divide between bodybuilding subculture, ‘an oddball sport’ shunned for its homoerotic imagery, and mainstream culture where he has become a popular icon of muscular masculinity. Where no other bodybuilder before or following him has been so successful, I show that it was Schwarzenegger's ability to mould himself to popular discourses

Ellexis Boyle

2010-01-01

202

Interventional Radiology in the Management of Budd Chiari Syndrome  

SciTech Connect

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.

Beckett, David [Sheffield Vascular Institute, Vascular and Interventional Radiology (United Kingdom); Olliff, Simon, E-mail: simon.olliff@uhb.nhs.u [Queen Elizabeth Hospital, University Hospital Birmingham NHS Foundation Trust, Clinical Radiology Department (United Kingdom)

2008-09-15

203

Congenital Hepatic Vascular Malformations  

Microsoft Academic Search

\\u000a Congenital hepatic vascular malformations are rare entities that result in abnormal shunting of blood through the liver. Three\\u000a different types of shunting can occur: arteriovenous (hepatic artery to hepatic vein), arterioportal (hepatic artery to portal\\u000a vein) and portovenous (portal vein to hepatic vein). Malformations result from alterations in the formation of blood vessels\\u000a during fetal development and can occur as

Guadalupe Garcia-Tsao

204

Birth injury as a causative factor of syringomyelia with Chiari type I deformity.  

PubMed Central

The epidemiology of syringomyelia with Chiari type I deformity was investigated with particular reference to perinatal problems. All subjects in our study were born by vaginal delivery and had a high incidence of perinatal accidents (abnormal presentations, birth injuries, and neonatal asphyxia). This study suggests that these may be strong causative factors for syringomyelia associated with Chiari type I deformity.

Hida, K; Iwasaki, Y; Imamura, H; Abe, H

1994-01-01

205

Liver transplantation in a patient with Budd-Chiari syndrome secondary to factor V Leiden mutation  

Microsoft Academic Search

A point mutation in the factor V Leiden gene is the most common hereditary thrombophilic state and an important risk factor for Budd-Chiari syndrome. We report on a patient with Budd-Chiari syndrome secondary to factor V Leiden mutation, who underwent successful liver transplantation. Following liver transplantation, his thrombophilic state was corrected and he did not require anticoagulation therapy. There has

Z Karasu; D Nart; E Lebe; T Demirbas; A Memis; M Kilic; M Akyildiz; Y Tokat

2003-01-01

206

Abruptio Placentae during Fetal Myelomeningocele Repair  

Microsoft Academic Search

Myelomeningocele (MM) is a congenital neural tube defect with serious consequences, including hydrocephaly. An important hope for intrauterine repair is that hydrocephaly may be prevented by reversing the Arnold-Chiari malformation. Three medical centers in the United States are doing trials with this objective. We describe an intrauterine correction of MM in a Brazilian research center of fetal medicine, which resulted

Ricardo Barini; Maria Weber G. Barreto; Kleber Cursino; Herder Zambelli; Adilson Prando; Lourenço Sbragia

2006-01-01

207

Exophtalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy  

Microsoft Academic Search

We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II Arnold-Chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar

George Kahaly; Wibke Müller-Forell; Barbara Tettenborn; Victor Mihaljevic; Jürgen Beyer

1988-01-01

208

Quantum Arnol'd diffusion in a simple nonlinear system.  

PubMed

We study the fingerprint of the Arnol'd diffusion in a quantum system of two coupled nonlinear oscillators with a two-frequency external force. In the classical description, this peculiar diffusion is due to the onset of a weak chaos in a narrow stochastic layer near the separatrix of the coupling resonance. We have found that global dependence of the quantum diffusion coefficient on model parameters mimics, to some extent, the classical data. However, the quantum diffusion happens to be slower than the classical one. Another result is the dynamical localization that leads to a saturation of the diffusion after some characteristic time. We show that this effect has the same nature as for the studied earlier dynamical localization in the presence of global chaos. The quantum Arnol'd diffusion represents a new type of quantum dynamics and can be observed, for example, in two-dimensional semiconductor structures (quantum billiards) perturbed by time-periodic external fields. PMID:12366228

Demikhovskii, V Ya; Izrailev, F M; Malyshev, A I

2002-09-20

209

Clocking convergence to Arnold tongues - The H-rank approach  

NASA Astrophysics Data System (ADS)

Computational techniques based on ranks of Hankel matrices (H-ranks) is used to study the convergence to Arnold tongues in the circle map. It appears that the process of convergence to the phase-locked mode of the discrete stationary attractor is far from being trivial. Figures of pseudoranks of Hankel matrices constructed from transient solutions of the circle map carry important physical information about complex nonlinear processes and are also beautiful from the aesthetical point of view.

Landauskas, Mantas; Ragulskis, Minvydas

2013-10-01

210

Rare malformation of glans penis: arteriovenous malformation.  

PubMed

Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children. PMID:23771468

Akin, Y; Sarac, M; Yucel, S

211

Color image encryption based on gyrator transform and Arnold transform  

NASA Astrophysics Data System (ADS)

A color image encryption scheme using gyrator transform and Arnold transform is proposed, which has two security levels. In the first level, the color image is separated into three components: red, green and blue, which are normalized and scrambled using the Arnold transform. The green component is combined with the first random phase mask and transformed to an interim using the gyrator transform. The first random phase mask is generated with the sum of the blue component and a logistic map. Similarly, the red component is combined with the second random phase mask and transformed to three-channel-related data. The second random phase mask is generated with the sum of the phase of the interim and an asymmetrical tent map. In the second level, the three-channel-related data are scrambled again and combined with the third random phase mask generated with the sum of the previous chaotic maps, and then encrypted into a gray scale ciphertext. The encryption result has stationary white noise distribution and camouflage property to some extent. In the process of encryption and decryption, the rotation angle of gyrator transform, the iterative numbers of Arnold transform, the parameters of the chaotic map and generated accompanied phase function serve as encryption keys, and hence enhance the security of the system. Simulation results and security analysis are presented to confirm the security, validity and feasibility of the proposed scheme.

Sui, Liansheng; Gao, Bo

2013-06-01

212

Acetabular reconstruction with Chiari pelvic osteotomy: report of 2 cases.  

PubMed

We performed cementless total hip arthroplasty combined with acetabular reconstruction with Chiari pelvic osteotomy on 3 hips in 2 patients. This procedure increased the osseous coverage of the shell and decreased the load around the hip by medialization of the hip center. Osteosynthesis of the proximal and distal bone fragment was performed using a shell with screws. Nonunion between the proximal and distal fragments was not noticed to include the case with small contact area. Postoperative dislocation had not occurred in these cases as of 1 year after surgery. This method may be an effective solution for severe dysplasia and revision surgery. PMID:22127396

Hara, Toshihiko; Maekawa, Masayuki; Kaminomachi, Shigekazu; Ikemura, Satoshi; Nakamura, Tetsuro

2011-11-29

213

Interventional therapeutic techniques in Budd-Chiari syndrome  

Microsoft Academic Search

Purpose  To analyze the results obtained with percutaneous therapeutic procedures in patients with Budd-Chiari syndrome (BCHS).\\u000a \\u000a \\u000a \\u000a Methods  Between August 1991 and April 1993, seven patients with BCHS were treated in our hospital. Three presented with a congenital\\u000a web; in another three cases the hepatic veins and\\/or the inferior vena cava (IVC) were compromised after major hepatic surgery;\\u000a one patient presented with a

José Ignacio Bilbao; Jesfis Ciro Pueyo; Jesús María Longo; Mercedes Arias; José Ignacio Herrero; Alberto Benito; María Dolores Barettino; Juan Pablo Perotti; Fernando Pardo

1997-01-01

214

Familial cerebral cavernous malformation.  

PubMed

Cavernous malformations (CMs) occur in approximately 0.5% of the general population and represent 5-10% of the central nervous system vascular malformations. The majority of CMs appear sporadically but genetically determined familial forms account for 10% to 15% of all cases. The aim of this study was to discuss the clinical, pathological and genetic aspects of familial cerebral cavernous malformations (CCMs). We report on five members of a family who underwent surgery due to CCMs. However, only two members were treated in our Department. The age of onset of symptoms in these cases (4 men and 1 women) ranged from 3 to 28 years. Three members of the family were asymptomatic but it turned out that they were obligatory gene carriers and in one of them the cavernous malformation was confirmed by neuroimaging study. The clinical symptoms of CCMs included seizure (three patients) and focal neurological deficit (two patients). Multiple CCMs were identified in two symptomatic patients (two lesions) and in one asymptomatic patient (three lesions). The lesions were located superficially (4), in the basal ganglia (1), in the brainstem (2) and in the cerebellar vermis (1). In two patients, the subsequent imaging studies showed a single de novo CCM formation. Only one patient with mutation of CCM2 gene was treated surgically. In patients with cavernous malformations the detailed clinical and family history of neurological events ought to be collected. This is particular important in patients with multiple changes or with de novo CCMs formation, identified in subsequent imaging studies. A well-documented family history can help to establish the final diagnosis and makes it possible to offer all members of the family proper neurological and genetic care. PMID:22773461

Dziedzic, Tomasz; Kunert, Przemys?aw; Matyja, Ewa; Ziora-Jakutowicz, Karolina; Sidoti, Antonella; Marchel, Andrzej

2012-01-01

215

Beh?et's disease presenting with Budd-Chiari syndrome and intracardial thrombus: a case report  

PubMed Central

Budd–Chiari syndrome has been described as a late complication of Behçet’s disease. Although the mortality rate associated with Behçet’s disease is low, it can escalate in the presence of Budd–Chiari syndrome and may be further complicated by intracardial thrombus formation. It is therefore important to detect and initiate management early in the disease course. The imaging modalities of choice should be minimally invasive as certain procedures may aggravate Behçet’s disease by initiating a thrombosis or aggravating an existing one. In Behçet’s disease-induced Budd–Chiari syndrome, cardiac investigation is crucial in the work-up in order to identify any cardiac involvement and determine the etiology of intracardial thrombus. Furthermore, the treatment should ultimately focus on controlling the activity of Behçet’s disease. We report an unusual case of Behçet’s disease presenting with Budd–Chiari syndrome complicated by intracardial thrombus in a young Korean man.

Kim, Minji Jennifer; Jo, Yong Suk; Kim, Jee Hyun; Kim, Yong-Jin; Kim, Kyung Hwan; Lee, Eun Bong; Song, Yeong Wook; Lee, Eun Young

2011-01-01

216

Imparting wisdom: Magda Arnold's contribution to research on emotion and motivation  

Microsoft Academic Search

To highlight the wisdom that Magda Arnold's grand theory can still impart on the field of emotion and motivation, we consider how her writings on wanting, thinking, knowing, and doing resonate in current research on mood and information processing, emotion regulation, emotional intelligence, and intrinsic motivation. In the wanting section, we examine Arnold's hypothesis that emotion alters action by shaping

Karen Gasper; Kosha D. Bramesfeld

2006-01-01

217

Origins of Airpower. Hap Arnold's Early Career in Aviation Technology, 1903-1935.  

National Technical Information Service (NTIS)

Henry Harley ('Hap') Arnold was not supposed to enter the Army. His older brother, Thomas, was to attend West Point and continue the Arnold family tradition of American military service that began during the War for Independence. Henry Harley, Hap's names...

D. Daso

1996-01-01

218

The Chiari\\/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention  

Microsoft Academic Search

Objective: To determine how adults with myelomeningocoele who develop the Chiari\\/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. Methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari\\/hydrosyringomyelia

JJ Craig; WJ Gray; JP McCann

1999-01-01

219

Ebstein’s malformation  

Microsoft Academic Search

Opinion statement  Ebstein’s malformation of the tricuspid valve is a complex and heterogeneous disorder that has diverse hemodynamic and arrhythmic\\u000a manifestations. Hemodynamic dysfunction is due to valvar regurgitation and decreased pulmonary blood flow, varying in severity\\u000a from mild tricuspid insufficiency to severe dysplasia of the right ventricle without forward flow. Atrioventricular reentrant\\u000a tachycardia and atrial tachyarrhythmias are commonly associated with Ebstein’s

Adel K. Younoszai; Michael M. Brook; Norman H. Silverman

1999-01-01

220

ArterioVenous Malformations  

Microsoft Academic Search

Intracranial arterio-venous malformations (AVM) are relatively uncommon, but increasingly recognized lesions that can cause\\u000a serious neurological symptoms or death. Although AVM can initially present with hemorrhages or seizures, an increasing number\\u000a is detected before symptomatic bleeding due to the recently developed imaging techniques. Over the last decades, the management\\u000a of AVM has been widely modified due to the availability of

Markus W. Gross; Rita Engenhart-Cabillic

221

Vein of Galen malformation  

Microsoft Academic Search

Since 1984, 43 patients with true vein of Galen ancurysmal malformations have been referred to us and managed according to our patient selection, technique, and follow-up guidelines. Thirty-four were embolized transarterially with bucrilate (isobutyl cyanoacrylate) or enbucrilate (N-butyl cyanoacrylate) embolization. No cutdown or hypotension during or after the embolization was used and no balloon catheter was employed. Forty-seven percent of

P. Lasjaunias; R. Garcia-Monaco; G. Rodesch; K. Ter Brugge; M. Zerah; M. Tardieu; D. Victor

1991-01-01

222

Treatment of congenital malformations.  

PubMed

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

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

2011-03-24

223

Significant overlap and possible identity of macrocephaly capillary malformation and megalencephaly polymicrogyria-polydactyly hydrocephalus syndromes.  

PubMed

We report on three patients with macrocephaly and polymicrogyria, and additional anomalies seen in megalencephaly polymicrogyria-polydactyly hydrocephalus (MPPH) and macrocephaly capillary malformation (MCM) syndromes. Based on their characteristic brain malformations they were originally diagnosed with MPPH. In one patient the phenotype evolved during early infancy, and ultimately resulted in a diagnosis of MCM. A second was prenatally diagnosed with MPPH, but postnatally visualized capillary malformations led to a diagnosis of MCM. In a third, the original MPPH diagnosis was reconsidered after a critical review revealed additional subtle findings suggestive of MCM. Characteristic brain malformations are thought to distinguish between MPPH with perisylvian polymicrogyria, and MCM with megalencephaly with Chiari 1 malformation. However, polymicrogyria was reported in a significant number of patients with MCM. Conversely, upon review of imaging studies of patients with MPPH, we noted progressive crowding of the posterior fossa and acquired tonsillar herniation, a process deemed characteristic for MCM. Thus, neither polymicrogyria nor acquired tonsillar herniation are distinguishing features, and occur in both disorders. In addition to brain abnormalities, shared findings include cognitive impairment, coarse facial features and postaxial polydactyly. Facial nevus flammeus and cutis marmorata are most noticeable in infancy, and ligamentous laxity and redundant soft tissue are somewhat subjective findings. While asymmetric overgrowth is considered typical for MCM, it is not universally present. These variable and subtle findings can be identified in patients with MPPH. We propose that MPPH and MCM may not represent distinct entities and that the term MPPH-CM syndrome be used to describe this spectrum. PMID:19353582

Gripp, Karen W; Hopkins, Elizabeth; Vinkler, Chana; Lev, Dorit; Malinger, Gustavo; Lerman-Sagie, Tally; Dobyns, William B

2009-05-01

224

Uterine arteriovenous malformation.  

PubMed

Uterine arteriovenous malformation (AVM) is a rare condition, with fewer than 100 cases reported in the literature. Despite it being rare, it is a potentially life-threatening condition. This case report describes a 33-year-old woman who presented with secondary post-partum hemorrhage. Transabdominal ultrasound (US) of the pelvis showed increased vascularity with multidirectional flow of the uterus and a prominent vessel, located on the left lateral wall. She also had retained product of conception, which complicated the diagnosis. A uterine artery angiogram confirmed an AVM in the fundal region with an early draining vein. Embolisation of the AVM was performed successfully. PMID:23983582

Hashim, Hilwati; Nawawi, Ouzreiah

2013-03-01

225

Arteriovenous Malformation Management  

SciTech Connect

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.

Yakes, Wayne F. [Interventional Radiology and Interventional Neuroradiology, Radiology Imaging Associates, P.C., Colorado NeurologicalInstitute, Swedish Medical Center, 501 E. Hampden Avenue, Englewood, CO 80110 (United States); Rossi, Plinio [Department of Radiology, Universita Degli Studi Di Roma, 'La Sapienza', 00161 Rome (Italy); Odink, Henk [Department of Radiology, De Wever Hospital, Henri Dunanstrat 5, 6419 PC Heerlen (Netherlands)

1996-11-15

226

Uterine Arteriovenous Malformation  

PubMed Central

Uterine arteriovenous malformation (AVM) is a rare condition, with fewer than 100 cases reported in the literature. Despite it being rare, it is a potentially life-threatening condition. This case report describes a 33-year-old woman who presented with secondary post-partum hemorrhage. Transabdominal ultrasound (US) of the pelvis showed increased vascularity with multidirectional flow of the uterus and a prominent vessel, located on the left lateral wall. She also had retained product of conception, which complicated the diagnosis. A uterine artery angiogram confirmed an AVM in the fundal region with an early draining vein. Embolisation of the AVM was performed successfully.

Hashim, Hilwati; Nawawi, Ouzreiah

2013-01-01

227

Cervico-Facial Vascular Malformations  

Microsoft Academic Search

\\u000a Craniofacial vascular lesions are best categorized into hemangiomas (i.e., showing proliferation and potential involution) and vascular malformations (i.e., not showing such behavior) [1]. While hemangiomas and PHACE syndrome will briefly be dealt with at the end of this chapter, vascular malformations are\\u000a the main focus of this paper.

Jeyaledchumy Mahadevan; Hortensia Alvarez; Pierre Lasjaunias

228

Polycythemia and the Budd-Chiari syndrome: study of serum erythropoietin and bone marrow erythroid progenitors.  

PubMed

The mechanism of polycythemia associated with the Budd-Chiari syndrome is unknown. Erythropoiesis in 10 patients with Budd-Chiari syndrome was studied in an attempt to distinguish prior unrecognized polycythemia vera from secondary polycythemia. Serum erythropoietin was assayed using a mouse fetal liver erythroblast assay. High concentrations of serum erythropoietin were observed in 6 of 7 patients with acute primary Budd-Chiari syndrome. Levels were normal in four patients who were investigated during the chronic phase and were increased in one with persisting polycythemia. In one patient, erythropoietin concentration in the hepatic vein was twice the level measured in peripheral, caval and renal venous blood. Bone marrow erythroid progenitors developed in vitro in the absence of exogenous erythropoietin in all polycythemia vera cases studied in acute and chronic phases, whether polycythemia persisted or not. These findings indicate that hepatic erythropoietin production occurs in the acute phase of Budd-Chiari syndrome and suggest that, in some cases of Budd Chiari syndrome, polycythemia which resolves after the acute phase may be secondary to liver disease. PMID:4029896

Levy, V G; Ruskone, A; Baillou, C; Theirman-Duffaud, D; Najman, A; Boffa, G A

229

In utero Repair of Myelomeningocele: Rationale, Initial Clinical Experience and a Randomized Controlled Prospective Clinical Trial  

PubMed Central

Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Arnold-Chiari II malformation, incontinence, sexual dysfunction, skeletal deformations, and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Studies in animals provide compelling evidence that the primary cause of the neurological deficit associated with MMC is not simply incomplete neurulation but rather chronic mechanical injury and amniotic-fluid-induced chemical trauma that progressively damage the exposed neural tissue during gestation. Initial results suggest that the surgical repair of MMC before 25 weeks of gestation may preserve neurological function, reverse the hindbrain herniation of the Arnold-Chiari II malformation, and obviate the need for postnatal placement of a ventriculoperitoneal shunt. As it is currently unknown whether fetal surgery for MMC is truly beneficial compared to standard postnatal care, a randomized, controlled clinical trial has been initiated within the United States.

Danzer, Enrico; Flake, Alan W.

2008-01-01

230

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

PubMed

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

Harris, Ben

2011-08-01

231

On the arc filtration for the singularities of Arnold's lists  

NASA Astrophysics Data System (ADS)

In a previous paper, the authors introduced a filtration on the ring {cal O}_{V,0} of germs of functions on a germ (V,0) of a complex analytic variety defined by arcs on the singularity and called the arc filtration. The Poincaré series of this filtration were computed for simple surface singularities in the 3-space. Here they are computed for surface singularities from Arnold's lists including uni- and bimodular ones. The classification of the unimodular singularities by these Poincaré series turns out to be in accordance with their hierarchy defined by E. Brieskorn using the adjacency relations. We also give a general formula for the Poincaré series of the arc filtration for isolated surface singularities which are stabilizations of plane curve ones.

Ebeling, Wolfgang; Gusein-Zade, Sabir M.

2005-03-01

232

Arnold Arboretum: South Central China and Tibet: Hotspot of Diversity  

NSDL National Science Digital Library

For well over one hundred years, The Arnold Arboretum has sent scientists to document and explore the various corners of Asia. In the early 1920s, the Arboretum sent an expedition that remained in the Hengduan Mountain region for three years. Upon their return, the expedition returned with all types of flora and fauna, including stuffed birds, seeds, and hundreds of photographic images. This online collection contains digitized materials from that notable expedition, and subsequent expeditions that have taken place in the 1990s and the 2000s. Visitors can dive right in by clicking on the "Search Expedition Collections" section where they can look at images of rock specimens, read reports from the expeditions, and browse interactive maps of the region. It's a tremendous collection, and one that will warrant several return visits.

233

Arnold Arboretum: South Central China and Tibet: Hotspot of Diversity  

NSDL National Science Digital Library

For well over one hundred years, The Arnold Arboretum has sent scientists to document and explore the various corners of Asia. In the early 1920s, the Arboretum sent an expedition that remained in the Hengduan Mountain region for three years. Upon their return, the expedition returned with all types of flora and fauna, including stuffed birds, seeds, and hundreds of photographic images. This online collection contains digitized materials from that notable expedition, and subsequent expeditions that have taken place in the 1990s and the 2000s. Visitors can dive right in by clicking on the "Search Expedition Collections" section where they can look at images of rock specimens, read reports from the expeditions, and browse interactive maps of the region. It's a tremendous collection, and one that will warrant several return visits.

2009-06-01

234

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

PubMed

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

Curtis, Scott

2011-09-01

235

Management of venous malformations.  

PubMed

Venous malformations (VMs) frequently occur in the head and neck with a predilection for the parotid gland, submandibular triangle, buccal space, muscles of mastication, lips, and upper aerodigestive tract. They are composed of congenitally disrupted ectatic veins with inappropriate connections and tubular channels. Because VMs have poorly defined boundaries and a tendency to infiltrate normal tissue, they require calculated treatment decisions in the effort to preserve surrounding architecture. Sclerotherapy, surgical excision, neodymium:yttrium aluminum garnet laser therapy, or a combination of these modalities is employed in the management of VMs. Although many small VMs can be cured, the objective is often to control the disease with periodic therapy. Location, size, and proximity to vital structures dictate the type of therapy chosen. Vigilance with long-term follow up is important. This review outlines current diagnostic and therapeutic approaches to simple and extensive cervicofacial VMs. PMID:23188688

Richter, Gresham T; Braswell, Leah

2012-11-27

236

Uterine arteriovenous malformation.  

PubMed

Uterine arteriovenous malformation (AVM) is a little known condition of which, to date, very few cases have been described. It has a very diverse symptomatology, even though in most cases, it is diagnosed during a severe and acute haemorrhagic event. Its treatment can vary from expectant management to hysterectomy; however, current evidence suggests that the embolisation of uterine arteries is the most effective approach, especially if fertility is to be preserved. We present a case report classified as AVM, with additional images that show the appearance of this pathology in a short span of time. This case has a number of peculiarities: unusual persistence of human chorionic gonadotropin hormone (?-HCG), asymptomatic patient, quick establishment of the lesion and its duration with unchanging characteristics and finally its spontaneous resolution without further consequences. This entity shows an aetiopathogenesis, that is, not well established or described. We discuss its physiopathology and aetiopathogenesis. PMID:23396842

Sellers, Francisco; Palacios-Marqués, Ana; Moliner, Belen; Bernabeu, Rafael

2013-02-07

237

Analysis and Classification of Cerebellar Malformations  

Microsoft Academic Search

BACKGROUND AND PURPOSE: Because of improved visualization of posterior fossa struc- tures with MR imaging, cerebellar malformations are recognized with increasing frequency. Herein we attempt to describe and propose a rational classification of cerebellar malformations. METHODS: MR images obtained in 70 patients with cerebellar malformations were retro- spectively reviewed. The cerebellar malformations were initially divided into those with hyp- oplasia

Sandeep Patel; A. James Barkovich

2002-01-01

238

Pathologie neurovasculaire malformative de l'enfant : malformations anévrismales de la veine de Galien (MAVG), malformations artérioveineuses piales (MAVP), malformations des sinus duraux (MSD)  

Microsoft Academic Search

The purpose of this article is to give an overview of the cerebral and spinal cord pediatric malformations; we particularly describe three of them: Vein of Galen arteriovenous Malformation, Pial Arteriovenous Malformation, and Dural Sinus Malformation. We report the experience of Bicętre since 1981 to 2003, with 317 VGAM, 302 Pial AVM and 30 DSM. We describe natural history, clinical

A. Ozanne; H. Alvarez; T. Krings; P. Lasjaunias

2007-01-01

239

Interventional therapeutic techniques in Budd-Chiari syndrome  

SciTech Connect

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.

Bilbao, Jose Ignacio; Pueyo, Jesus Ciro; Longo, Jesus Maria; Arias, Mercedes [Universidad de Navarra, Department of Radiology, Clinica Universitaria, Facultad de Medicina (Spain); Herrero, Jose Ignacio [Universidad de Navarra, Department of Internal Medicine, Clinica Universitaria, Facultad de Medicina (Spain); Benito, Alberto; Barettino, Maria Dolores; Perotti, Juan Pablo [Universidad de Navarra, Department of Radiology, Clinica Universitaria, Facultad de Medicina (Spain); Pardo, Fernando [Universidad de Navarra, Department of Surgery, Clinica Universitaria, Facultad de Medicina (Spain)

1997-03-15

240

Epidemiologic characteristics of kidney malformations  

Microsoft Academic Search

Infants with kidney agenesis or dysgenesis, infants with cystic kidneys, and infants with horse-shoe kidneys were studied, based on data from three large and population-based congenital malformation registers: a total of 2666 infants among 5.83 million births. There is a strong variability between programs of the rates of registered unilateral kidney malformations and these are strongly over-represented in dead infants

John Harris; Elisabeth Robert; Bengt Källén

2000-01-01

241

Congenital malformations of human dermatoglyphs  

PubMed Central

A classification for congenital malformations of dermatoglyphs is presented, dividing them into ridge aplasia, ridge hypoplasia, ridge dissociation, ridges-off-the-end, and a combination of the last two. The medical and genetic significance of these are considered in the light both of previous published cases and of new material. Malformations of dermatoglyphs are important as physical signs in paediatric diagnosis. ImagesFIG. 1FIG. 2FIG. 3FIG. 4FIG. 5FIG. 6FIG. 7

David, T. J.

1973-01-01

242

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

PubMed

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

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

2011-12-01

243

[Unruptured brain arteriovenous malformations].  

PubMed

Cerebral arteriovenous malformations (AVMs) often become symptomatic in young adults with a variety of neurological symptoms such as epileptic seizures, recurrent headaches, progressive neurological deficits or sudden intracranial hemorrhage. However, recent epidemiological data suggest a large number of AVMs are found without signs of hemorrhage and a relatively low rupture risk of less than 1% per year. Continuous technical progress has led to several specific treatment options for brain AVMs including endovascular embolization, microneurosurgery, and stereotactic radiotherapy, either alone or in any combination. Depending on AVM topography and vascular anatomy, interventional treatment strategies have shown successful occlusion rates ranging between 50 and 100% and a relatively low average procedural morbidity of 10% overall. For unruptured brain AVMs, however, the clinical benefit of invasive treatment remains as yet to be determined. To address this issue, A randomized trial of unruptured brain AVMs (ARUBA) is currently underway evaluating long-term outcome of best possible standard interventional therapy as compared to the natural history risk in a prospective multidisciplinary international study (www.arubastudy.org). PMID:18786684

Stapf, C

2008-09-10

244

Arnold Wesker'in Dünyasi ve Son Oyunlarindan ?ki Örnek: Boylam ve ?nkâr Arnold Wesker's World and Two Examples From His Last Plays: Longitude and Denial  

Microsoft Academic Search

Arnold Wesker, the writer of Longitude and Denial, belongs to the postwar English writers generation. Wesker, doing the philosophy of drama with his ideas about theatre, and with his independent and original tendency, is one of the prolific writers of English Drama feeding it with their works today. Wesker reflects his childhood and youth years, passed happily but with troubles,

Nurhan Tekerek

245

[Unusual presentation of rib malformation].  

PubMed

Rib malformation and anatomical variations are not well known and are still often underdiagnosed. Usually, rib malformations are fortuitously discovered. We describe here the case of a girl, 4 years and 4 months old, who presented at the emergency unit for fever and an anterior tumefaction of the ribcage, without any other symptoms. She was eupneic with a normal pulmonary auscultation and viral tonsillitis with a negative streptococcus test. The thoracic tumefaction was parasternal, painless, and fixed and measured approximately 2.5 × 2cm. Ultrasound findings consisted of a duplicated and hypoechogenic hypertrophy of the sterno-costal cartilage of the 4th left rib. Magnetic resonance imaging (MRI) confirmed the diagnosis of chondral bifidity of the sterno-costal junction of the 4th left rib. Fever, due to the viral tonsillitis, disappeared after 4 days. Rib malformations are rare, often anterior, unilateral, and preferentially located on the 3rd or the 4th rib. The main malformative rib lesions are bifid ribs, rib spurs, and widened ribs. Very rarely, they can be associated with Gorlin-Goltz syndrome or with other malformations such as VATER complex. The main differential diagnoses of these rib malformations are traumatic, tumoral, and infectious etiologies. In case of tumoral diseases, the topography of the lesion focuses the etiologic diagnosis: whereas an anterior and cartilaginous lesion is always benign, a lateral or posterior lesion can be an Ewing sarcoma. Rib malformation investigation consists in meticulous questioning, a complete clinical examination looking for any associated anomaly, completed by basic imaging explorations such as plain thoracic radiography focused on the ribcage and ultrasound. Finally, complementary computerized tomography or preferably MRI, depending on the anatomic location of the lesion, confirms the final diagnosis, as presented in our case report, and removes any uncertainty. PMID:23037576

Cosson, M-A; Breton, S; Aprahamian, A; Grevent, D; Cheron, G

2012-10-01

246

Synchronization mechanism and Arnold tongues for dust density waves  

NASA Astrophysics Data System (ADS)

The nonlinear phenomenon of synchronization is characterized experimentally for dust density waves, i.e., dust acoustic waves, which are self-excited due to an ion streaming instability. The waves propagate in a dust cloud with a natural frequency of 22 Hz. We synchronize these waves to a different frequency using a driving electrode that sinusoidally modulates the ion density. We study four synchronized states, with frequencies that are multiples of 1, 2, 3, and 1/2 of the driving frequency. Comparing to phenomena that are typical of the van der Pol paradigm, we find that synchronization of our waves exhibit the signature of the suppression mechanism but not that of the phaselocking mechanism. Additionally, synchronization of our waves exhibits three characteristics that differ from the van der Pol paradigm: a threshold amplitude that can be seen in the Arnold tongue diagram, a branching of the 1:1 harmonic tongue at its lower extremity, and a nonharmonic state. The latter state appears to be a nonlinear oscillation; it is neither at the natural frequency nor a synchronized state.

Ruhunusiri, W. D. Suranga; Goree, J.

2012-04-01

247

Kolmogorov Arnold Moser aspects of the periodic Hamiltonian Hopf bifurcation  

NASA Astrophysics Data System (ADS)

In this work we consider a 1 : -1 non-semi-simple resonant periodic orbit of a three degrees of freedom real analytic Hamiltonian system. From the formal analysis of the normal form, we prove the branching off of a two-parameter family of two-dimensional invariant tori of the normalized system, whose normal behaviour depends intrinsically on the coefficients of its low-order terms. Thus, only elliptic or elliptic together with parabolic and hyperbolic tori may detach from the resonant periodic orbit. Both patterns are mentioned in the literature as the direct and inverse, respectively, periodic Hopf bifurcation. In this paper we focus on the direct case, which has many applications in several fields of science. Our target is to prove, in the framework of Kolmogorov-Arnold-Moser (KAM) theory, the persistence of most of the (normally) elliptic tori of the normal form, when the whole Hamiltonian is taken into account, and to give a very precise characterization of the parameters labelling them, which can be selected with a very clear dynamical meaning. Furthermore, we give sharp quantitative estimates on the 'density' of surviving tori, when the distance to the resonant periodic orbit goes to zero, and show that the four-dimensional invariant Cantor manifold holding them admits a Whitney-C? extension. Due to the strong degeneracy of the problem, some standard KAM methods for elliptic low-dimensional tori of Hamiltonian systems do not apply directly, so one needs to properly suit these techniques to the context.

Ollé, Mercč; Pacha, Juan R.; Villanueva, Jordi

2008-08-01

248

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

SciTech Connect

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.

Sarawagi, Radha, E-mail: sarawagi_r@yahoo.co.uk; Keshava, Shyamkumar N., E-mail: aparna_shyam@yahoo.com; Surendrababu, Narayanam R. S., E-mail: nrssbabu@yahoo.com [Christian Medical College, Department of Radiology (India); Zachariah, Uday G., E-mail: udayzachariah@cmcvellore.ac.in; Eapen, Eapen C., E-mail: eapen@cmcvellore.ac.in [Christian Medical College, Department of Gastroenterology (India)

2011-02-15

249

Liver transplantation for Budd-Chiari syndrome-palliation or cure?  

Microsoft Academic Search

This report documents two cases of Budd-Chiari syndrome (BCS) with essential thrombocytosis and antithrombin (AT) III deficiency as underlying etiological factors. Orthotopic liver transplantation was successfully performed in both patients but with different therapeutic intention. In the patient with essential thrombocytosis, hepatic transplantation only relieved the symptoms of the predisposing thrombogenic condition; it děd not cure the underlying disorder. Prophylactic

H. Lang; K. J. Oldhafer; E. Kupsch; B. Ringe; R. Pichlmayr

1994-01-01

250

Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils  

Microsoft Academic Search

This is an analysis of 19 consecutive cases of symptomatic patients with Chiari I deformities, undertaken to evaluate the long-term effect of posterior fossa decompression and duraplasty, assessed by postoperative imaging. Sixteen of the patients had syringomyelia and three had foramen magnum syndromes without a syrinx. Eighteen patients underwent posterior fossa craniectomy, subpial resection of the cerebellar tonsils, and duraplasty.

Edwin G. Fischer

1995-01-01

251

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

SciTech Connect

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.

Sancak, Tanzer; Karagulle, Ayse Tuba; Bilgic, Sadik; Sanlidilek, Umman [Ankara University, Medical Faculty, Radiology Department, Section of Vascular and Interventional Radiology, Ibn-i Sina Hospital, Hasircilar Street, Sihhiye, 06100 Ankara (Turkey); Yerdel, Mehmet Ali [Ankara University, Medical Faculty, General Surgery Department, Section of Vascular Surgery, Ibn-i Sina Hospital, HasircilarStreet, Sihhiye, 06100 Ankara (Turkey)

2002-08-15

252

Fulminant Budd–Chiari syndrome associated with polycythemia rubra vera and factor V Leiden mutation  

Microsoft Academic Search

Budd–Chiari syndrome (BCS) is a severe disorder characterized by hepatic venous outflow obstruction. Hypercoagulable states are the major etiological factors for the development of BCS and can be identified in about 75% of patients. Multiple etiological factors can be found in the same patient. Hematologic abnormalities, especially myeloproliferative disorders, are the most common causes of BCS. Furthermore, the prevalence of

Murat Akyildiz; Zeki Karasu; Hamad Dheir; Necla Osmanoglu; Sinan Akay; Tankut Ilter

2006-01-01

253

Suboccipital craniectomy with opening of the fourth ventricle and duraplasty: study of 192 cases of craniovertebral malformations.  

PubMed

The prime objective in the surgical treatment of basilar impression (BI), Chiari malformation (CM), and/or syringomyelia (SM) is based on restoration of the normal cerebrospinal fluid (CSF) dynamics at the craniovertebral junction and creation of a large artificial cisterna magna, avoiding the caudal migration of the hindbrain. It is observed that a large craniectomy might facilitate an upward migration of the posterior fossa structures. There are many surgical techniques to decompress the posterior fossa; however, a gold standard approach remains unclear. The authors present the results of 192 cases of BI, CM, and SM treated between 1975 and 2008 and whose surgical treatment was characterized by a large craniectomy without tonsillectomy with the patient in the sitting position, large opening of the fourth ventricle, and duraplasty. PMID:24141441

Silva, José Alberto Gonçalves da; Santos, Adailton Arcanjo Dos; Costa, Maria do Desterro Leiros da; Almeida, Everardo Bandeira de

2013-09-01

254

Compartmentalization of massive vascular malformations.  

PubMed

A total of 18 patients with massive vascular malformations of the head and neck region were treated with compartmentalization using nonabsorbable sutures followed by injection of a sclerosant agent into each compartment. The indication for compartmentalization was either to stop potentially uncontrollable, life-threatening hemorrhage during the dissection of the lesion or to reduce its vascularity to allow a less dangerous subsequent resection. Compartmentalization was used in both high-flow and low-flow vascular malformations. In this technique, large nonabsorbable sutures are placed deeply in multiple areas within the lesion. The aim is to divide the malformation into multiple compartments by changing the direction of the suturing; in this way the sclerosing agent is provided with a more effective environment. The sclerosant used was either sodium tetradecyl sulfate 3%, absolute alcohol, or both. The total amount of infiltrate varied from 3 to 35 cc, according to the size of malformation. After compartmentalization, swelling was the most noticeable complication. With this technique, it was possible to treat what were considered untreatable malformations using standard techniques and to control the inevitable serious bleeding. PMID:15622226

Jackson, Ian T; Keskin, Mustafa; Yavuzer, Reha; Kelly, Christopher P

2005-01-01

255

Arteriovenous malformations: ethanolamine oleate sclerotherapy.  

PubMed

Peripheral vascular malformations are now described according to some accepted guidelines, and the principle of proper treatment (nodus ablation) is becoming clear. An appropriate classification schema for vascular anomalies and definite indications for treatment are important to successful treatment overall. Non-invasive imaging (US, CT, and MRI) in association with clinical findings is critical in establishing the diagnosis, evaluating the extent of the malformation, and planning appropriate treatment. Direct nidus phlebography is useful not only in making a correct diagnosis but also in treating the lesion by sclerotherapy. When a patient suffers clinical complications, the nidus sclerotherapy becomes mandatory. If the vascular malformation remains bloodstream to a drainage vein during nidus opacification, flow control is necessary to achieve complete nidus ablation. A multidisciplinary approach is needed in the treatment of a high-flow lesion. A dedicated team approach is necessary for appropriate management in most cases. PMID:17988037

Hyodoh, H; Hyodoh, K

2007-01-01

256

Arteriovenous Malformation of the Pancreas  

PubMed Central

Pancreatic arteriovenous malformation (PAVM) is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.

Charalabopoulos, Alexandros; Macheras, Nikolas; Krivan, Sylvia; Petropoulos, Konstantinos; Misiakos, Evangelos; Macheras, Anastasios

2011-01-01

257

Glioblastoma Mimicking an Arteriovenous Malformation  

PubMed Central

Abnormal cerebral vasculature can be a manifestation of a vascular malformation or a neoplastic process. We report the case of a patient with angiography-negative subarachnoid hemorrhage (SAH) who re-presented 3?years later with a large intraparenchymal hemorrhage. Although imaging following the intraparenchymal hemorrhage was suggestive of arteriovenous malformation, the patient was ultimately found to have an extensive glioblastoma associated with abnormal tumor vasculature. The case emphasizes the need for magnetic resonance imaging to investigate angiography-negative SAH in suspicious cases to rule out occult etiologies, such as neoplasm. We also discuss diagnostic pitfalls when brain tumors are associated with hemorrhage and abnormal vasculature.

Khanna, Arjun; Venteicher, Andrew S.; Walcott, Brian P.; Kahle, Kristopher T.; Mordes, Daniel A.; William, Christopher M.; Ghogawala, Zoher; Ogilvy, Christopher S.

2013-01-01

258

Joseph Toynbee and his son Arnold: unpublished manuscript written by Adam Politzer.  

PubMed

In 1914, Adam Politzer (1835-1920) was invited to the Viennese Toynbee Hall to give a lecture. To prepare for his lecture, Politzer wrote a manuscript of 22 pages, seven of them are particularly interesting concerning biographical information on Joseph and Arnold Toynbee. These seven pages are presented in translation from German to English. Politzer particularly emphasizes the social qualities of these two men. This manuscript is representative of his high regard for Joseph and Arnold Toynbee. It completes the few existing biographies of Joseph Toynbee. PMID:15068512

Mudry, Albert; Kutter, Jurg

2004-03-01

259

Sudden unexpected death in young adults with chronic hydrocephalus  

Microsoft Academic Search

We present four cases of sudden unexpected death in young adults with chronic hydrocephalus. The patients were between 20\\u000a and 28 years of age and had suffered from aqueduct stenosis (two patients), spina bifida in combination with Arnold-Chiari\\u000a malformation (type II) and fragile X-syndrome. The patients suddenly collapsed with cardiorespiratory failure and could not\\u000a be resuscitated and none had a

C. H. Rickert; F. Grabellus; K. Varchmin-Schultheiß; H. Stöß; W. Paulus

2001-01-01

260

Fetal surgery for myelomeningocele.  

PubMed

Fetal intervention for myelomeningocele (MMC) may improve hydrocephalus and hindbrain herniation associated with the Arnold-Chiari II malformation and may reduce the need for ventriculoperitoneal shunting. As of now, there is little evidence that prenatal repair of MMC improves neurologic function. MMC is the first nonlethal disease under consideration and study for fetal surgery. As a result, potential improvements in outcome must be balanced with maternal safety and well-being, in addition to that of the unborn patient. PMID:19559329

Hirose, Shinjiro; Farmer, Diana L

2009-06-01

261

Detection of venous air embolism by continuous intraarterial oxygen monitoring  

Microsoft Academic Search

In a recent study, we compared a new intraarterial fiberoptic “optode” probe to continuously measure arterial oxygen and carbon\\u000a dioxide tensions and pH with intermittently drawn blood samples in patients undergoing surgery. In one patient with a diagnosis\\u000a of Arnold-Chiari type I malformation with outflow obstruction of the fourth ventricle, a major pulmonary air embolism occurred\\u000a while the patient was

Garth Greenblott; Steven J. Barker; Kevin K. Tremper; Steven Gerschultz; John L. Gehrich

1990-01-01

262

Cyclopism as a Hereditary Malformation  

Microsoft Academic Search

CYCLOPISM is a malformation which has been recognized for many years and which may have furnished the model for the Homeric monsters. It has been suggested that this developmental disorder is hereditary, but until recently there has been no proof of this.

Peter Pfitzer; Horst Müntefering

1968-01-01

263

Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis -- case report.  

PubMed

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis. PMID:23793200

Bittencourt, Maraya de Jesus Semblano; Dias, Carolina Moraes; Lage, Thaiane Lima; Barros, Renata Silva; Paz, Otávio Augusto Gomes; Vieira, Waldonio de Brito

264

The Budd-Chiari syndrome and hepatic veno-occlusive disease. Recognition and treatment.  

PubMed

In a review of 29 patients who were surgically treated by combined hepatic and portal decompression for intractable ascites, 18 were identified as falling into the category of the Budd-Chiari syndrome, with varying causes. Of this group, 2 patients were distinguished by the classical hepatic venous endophlebitis described by Chiari and later by Bras et al. Recently, this disease entity has been recognized as being due to the toxic effects of pyrrolizidine alkaloids contained in the Senecio and Crotolaria plants. In the first of these two cases the patient had emigrated from Jamaica and was exposed to "bush trees," but no chemical measurements were done. The second patient had consumed a large amount of comfrey teas, which were shown to contain high levels of pyrrolizidine alkaloids. These two cases add further weight to the existing evidence of the toxic effect of these alkaloids, and also demonstrate the effectiveness of hepatic and portal decompression. PMID:2322120

McDermott, W V; Ridker, P M

1990-04-01

265

Beh?et disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report*  

PubMed Central

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis.

Bittencourt, Maraya de Jesus Semblano; Dias, Carolina Moraes; Lage, Thaiane Lima; Barros, Renata Silva; Paz, Otavio Augusto Gomes; Vieira, Waldonio de Brito

2013-01-01

266

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

ERIC Educational Resources Information Center

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…

Volkov, Boris B.

2011-01-01

267

VERSO Graphic Interface to Reference Sources: A Project of Quinnipiac University's Arnold Bernhard Library  

Microsoft Academic Search

Librarians at the Arnold Bernhard Library of Quinnipiac University were concerned with the usage levels of their electronic reference titles. They added the titles to the library's Web page, made individual records in the online catalog, and even established links between an OPAC keyword search and a search through electronic reference titles. It was still felt that the collection was

Terry Ballard

2008-01-01

268

Psychoanalysis and Neuroscience in Dialogue: Commentary on Paper by Arnold H. Modell  

Microsoft Academic Search

Arnold H. Modell has been engaged in an ongoing effort to advance psychoanalysis as well as to integrate psychoanalytic theory and relevant domains of science, particularly neuroscience, with psychoanalytic practice. He has been articulating a biology and construction of meaning and the role of metaphor as he attempts to understand the relationship between mind and brain. Modell strives to understanding

Brian Koehler

2011-01-01

269

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

ERIC Educational Resources Information Center

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…

Koelsch, William A.

2008-01-01

270

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

ERIC Educational Resources Information Center

|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…

Koelsch, William A.

2008-01-01

271

American Airpower Comes of Age: General Henry H. 'Hap' Arnold's World War II Diaries. Volume 1.  

National Technical Information Service (NTIS)

Although the need for a comprehensive biography of Gen Henry H. 'Hap' Arnold exists, this volume does not constitute such a biography. Nor is this work intended as a history of the Army Air Forces in World War II. The aim of the editor has been to place i...

J. W. Huston

2002-01-01

272

American Airpower Comes of Age: General Henry H. 'Hap' Arnold's World War II Diaries. Volume 2.  

National Technical Information Service (NTIS)

Although the need for a comprehensive biography of Gen Henry H. 'Hap' Arnold exists, this volume does not constitute such a biography. Nor is this work intended as a history of the Army Air Forces in World War II. The aim of the editor has been to place i...

J. W. Huston

2002-01-01

273

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

ERIC Educational Resources Information Center

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…

Banks, Alan

274

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

ERIC Educational Resources Information Center

|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…

Volkov, Boris B.

2011-01-01

275

Acute external hydrocephalus complicating craniocervical decompression for syringomyelia–Chiari I complex  

Microsoft Academic Search

The occurrence of subdural cerebrospinal fluid collections and ventricular dilatation (external hydrocephalus) after foramen\\u000a magnum decompression is extremely rare. The authors report on a 37-year-old man who developed symptomatic subdural fluid collections\\u000a (SFC) after uncomplicated foramen magnum decompression for Chiari I–syringomyelia complex. Postoperative magnetic resonance\\u000a imaging revealed a supratentorial SFC with prominent midline shift. In addition, bilateral infratentorial SFCs extending

Paolo Perrini; Alexander Rawlinson; Richard Alfred Cowie; Andrew Thomas King

2008-01-01

276

Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet’s disease  

Microsoft Academic Search

Budd-Chiari syndrome (BCS) is a very rare vascular complication of Behçet’s disease (BD) which often leads to death as a result of portal hypertension and liver failure. We report a 45-year-old BD patient who presented with BCS. Diagnosis was confirmed with CT scan and contrast-enhanced MR angiography which showed ascites, short-segment stenosis of the inferior vena cava (IVC), and middle

Seung Woo Han; Gun Woo Kim; Jongmin Lee; Yong Joo Kim; Young Mo Kang

2005-01-01

277

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

SciTech Connect

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.

Terreni, Natalia; Vangeli, Marcello; Raimondo, Maria Luisa [Royal Free Hospital, Sheila Sherlock Hepatobiliary Pancreatic and Liver Transplantation Unit (United Kingdom); Tibballs, Jonathan M. [Royal Free Hospital, Department of Radiology (United Kingdom); Patch, David; Burroughs, Andrew K. [Royal Free Hospital, Sheila Sherlock Hepatobiliary Pancreatic and Liver Transplantation Unit (United Kingdom)], E-mail: Andrew.Burroughs@royalfree.nhs.uk

2007-09-15

278

Budd-Chiari syndrome: hepatic venous obstruction by an elevated diaphragm  

Microsoft Academic Search

We describe two cases of Budd-Chiari syndrome detected by magnetic resonance imaging that resulted from compression of the\\u000a inferior vena cava by an elevated right hemidiaphragm. Magnetic resonance images demonstrated elevation of the right hemidiaphragm\\u000a and medial deviation of the inferior vena cava with short segmental narrowing. The hepatic veins and inferior vena cava were\\u000a patent but discontinuous. Hepatic venous

P. N. Kim; D. G. Mitchell; E. K. Outwater

1999-01-01

279

The management of arteriovenous malformations.  

PubMed

Arteriovenous malformations (AVM) can occur in the entire central nervous system with a predilection of the supratentorial intracranial compartment. Intracerebral hemorrhage is the most common clinical presentation of AVM and associated with a high morbidity and mortality rate. Correct management of these lesions is therefore of utmost importance. In this review, the authors present actual diagnostic and interdisciplinary treatment modalities based on their experience in a major neurovascular center and taking into consideration actual literature data. Different treatment strategies are discussed. PMID:21464810

Sandalcioglu, I E; Wanke, I; Zappala, V; Forsting, M; Sure, U

2011-03-01

280

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

...of operation for Duane Arnold Energy Center (DAEC). DAEC is located...Cedar Rapids, Iowa. Potential alternatives to the proposed action (license...include no action and reasonable alternative energy sources. The draft Supplement 42...

2010-02-10

281

[Malformations of the esophagus: diagnosis and therapy].  

PubMed

Esophageal malformations are rare and can occur sporadically or as a component of various syndromes. The variations and classifications are manifold. With the available modern operation techniques most malformations can be resolved with good results. However, esophageal malformations are often combined with further malformations which limit the prognosis. The separation of the trachea and esophagus after gastrulation is not yet completely researched. The results so far indicate that the localized expression of various homeodomain transcription factors is essential for normal development of the trachea and esophagus. PMID:23423505

Falkeis, C; Hager, T; Freund-Unsinn, K; Wohlschläger, J; Veits, L; Hager, J

2013-03-01

282

Maternal distress and congenital malformations: do mothers of malformed fetuses have more problems?  

Microsoft Academic Search

As compared with 580 randomly chosen pregnant women without malformed offspring, 161 women with malformed offspring at the index pregnancy had a more frequent history of previous multiple offspring deaths and somewhat increased maternal age but were not different on social class, marital or cohabitation status or parity. As compared with demographically similar reproducing women (n=54) interviewed, malformation cases (n=98)

Gun Torstensson Nimby; Louise Lundberg; Tomas Sveger; Thomas F McNeil

1999-01-01

283

Diffuse malformations of cortical development.  

PubMed

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

Bahi-Buisson, Nadia; Guerrini, Renzo

2013-01-01

284

Tertiary Lymphoid Organs in Lymphatic Malformations  

PubMed Central

Abstract Background Examine lymphatic malformation lymphoid aggregates for the expression of tertiary lymphoid organ markers. Determine how lymphoid aggregate density relates to lymphatic malformation clinical features. Methods and Results Retrospective cohort study. Tissue and clinical data were reviewed from 29 patients in the Vascular Anomaly Database who represented the spectrum of head and neck lymphatic malformations and had >5 years of follow-up. Archived formalin-fixed, paraffin-embedded lymphatic malformation tissue was immunohistochemically stained with antibodies for tertiary lymphoid organ markers, which included follicular and mature myeloid dendritic cells, high endothelial venules, segregated B and T-cells, lymphatic endothelial cells, and lymphoid homing chemokines (CXCL13, CCL21). Lymphoid aggregate density (count/mm2) was quantified by 2 independent, blinded reviewers. Lymphoid aggregate density and lymphatic malformation clinical features were characterized using analysis of variance. Larger lymphatic malformation tissue lymphoid aggregates stained consistently for tertiary lymphoid organ markers. In oral cavity and neck specimens from the same patients (n?=?9), there were more tertiary lymphoid organ in oral cavity than in neck specimens (p?=?0.0235). In lymphatic malformation neck tissue, de Serres stage 4 lymphatic malformations displayed the highest tertiary lymphoid organ density. No significant association was seen between tertiary lymphoid organ density and other clinical features. Conclusion This study demonstrates that some lymphoid aggregates within lymphatic malformations represent tertiary lymphoid organs. There was an association between tertiary lymphoid organ density and lymphatic malformation location. Further study is required to define the role of lymphoid neogenesis and tertiary lymphoid organ formation in lymphatic malformation pathogenesis.

Kirsh, Andrew L.; Cushing, Sharon L.; Chen, Eunice Y.; Schwartz, Stephen M.

2011-01-01

285

["Arnold Näf: an extract from notes of the entire veterinary medicine of 1864"].  

PubMed

Arnold Näf studied Veterinary Medicine at the Tierarzneischule in Zürich from 1861 to 1864. During the lectures in clinical medicine he made several notes, which were published in the book: "Arnold Näf: Notes from the entire veterinary medicine 1864". In the present work the person of prosector Meyer together with his lectures about obstetrics, horse shoeing, specific pathology and therapy as well as prescription science are described in more detail. Hans Jakob Meyer was born in Schlieren, January 10, 1831 and died May 8, 1895. He was teaching Veterinary Medicine from 1855 to 1895 at the Tierarzneischule in Zürich. The therapy about glanders, mange and strangles in the horse was compared with the contemporary literature. Partial agreement with the application of substances used at that time has been found. PMID:18306935

Baumgartner, M

2008-01-01

286

ARNold: a web tool for the prediction of Rho-independent transcription terminators.  

PubMed

Rho-independent termination is a major mechanism of transcriptional arrest in bacteria that controls both normal 3' termination and a wide array of regulatory attenuation events. Detecting Rho-independent terminators is an obliged step in the annotation of bacterial operons. Yet, while several efficient algorithms are available for this purpose, there is no freely available web site enabling a rapid scanning of raw genomic sequence for the presence of terminators. Here we implemented such a web server, which combines two published prediction algorithms, Erpin and RNAmotif, and performs nearly as well as more complex procedures while being accessible to the non specialist. The ARNold Web server is available at : http://rna.igmors.u-psud.fr/toolbox/arnold/ PMID:21282983

Naville, Magali; Ghuillot-Gaudeffroy, Adrien; Marchais, Antonin; Gautheret, Daniel

2011-01-01

287

Capillary malformation-arteriovenous malformation syndrome: a report of 2 cases, diagnostic criteria, and management.  

PubMed

Capillary malformation-arteriovenous malformation syndrome is a rare type of vascular malformation first described in 2003. It is an autosomal dominant inherited disorder that has been reported in association with heterozygous mutations in the RASA1 gene, which encodes the protein RASp21. The clinical picture is characterized by multiple small capillary malformations which are associated with either arteriovenous malformations or arteriovenous fistulas in both the affected individual and other members of their family. We describe 2 new familial cases of this syndrome that were clinically and genetically diagnosed and studied in our hospital. PMID:23933248

Catalŕ, A; Roé, E; Vikkula, M; Baselga, E

2013-08-08

288

Maternal distress and congenital malformations: do mothers of malformed fetuses have more problems?  

PubMed

As compared with 580 randomly chosen pregnant women without malformed offspring. 161 women with malformed offspring at the index pregnancy had a more frequent history of previous multiple offspring deaths and somewhat increased maternal age but were not different on social class, marital or cohabitation status or parity. As compared with demographically similar reproducing women (n = 54) interviewed, malformation cases (n = 98) reported having had significantly more strong stress before identification of the malformation, as well as a clear tendency toward less appropriate timing of the pregnancy. Women with malformed offspring represent a psychosocially vulnerable group and should receive special clinical and personal support. PMID:10404467

Nimby, G T; Lundberg, L; Sveger, T; McNeil, T F

289

COMMENTS ON HENRY FRIEDMAN'S (2002) REVIEW OF ARNOLD GOLDBERG'SBEING OF TWO MINDS  

Microsoft Academic Search

This commentary is a response to a review byFriedman (2002) ofArnold Goldberg's (2000) bookBeing of Two Minds: The Vertical Split in Psychoanalysis and Psychotherapy. The writer argues against Friedman's contention that Goldberg is judgmental and not empathic toward his misbehaving patients, that he does not appreciate the importance of his own subjectivity as context for the patient's splitting, and that

Barbara Fajardo

2003-01-01

290

Seedbed of Reform: Arnold Guyot and School Geography in Massachusetts, 1849–1855  

Microsoft Academic Search

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 series of lectures (published as Earth and Man),

William A. Koelsch

2008-01-01

291

Numerical stability of the Alekseenko-Arnold evolution system compared to the ADM and BSSN systems  

SciTech Connect

We explore the numerical stability properties of an evolution system suggested by Alekseenko and Arnold. We examine its behavior on a set of standardized testbeds, and we evolve a single black hole with different gauges. Based on a comparison with two other evolution systems with well-known properties, we discuss some of the strengths and limitations of such simple tests in predicting numerical stability in general.

Jansen, Nina; Bruegmann, Bernd; Tichy, Wolfgang [Center for Gravitational Physics and Geometry and Center for Gravitational Wave Physics, Penn State University, University Park, Pennsylvania 16802 (United States); Institute of Theoretical Physics, University of Jena, 07743 Jena (Germany); Department of Physics, Florida Atlantic University, Boca Raton, Florida 33431 (United States)

2006-10-15

292

Arteriovenous malformation of the spinal cord  

Microsoft Academic Search

We report a case of spinal arteriovenous malformation (AVM) in which detailed morphological examination of the intramedullary vascular lesion was carried out employing serial section studies. The relationship of the malformation to parenchymal lesions was evaluated. An abnormal vessel at the spinomedullary transition, whose lamina elastica was partially interrupted, was suggestive of a shunt vessel. The location of the intramedullary

Tsuyoshi Ishida; Shigeo Murayama; Kazuyoshi Yamaguchi; Yoshinori Urano; Makoto Iwata

1989-01-01

293

A Framework for Detecting Malformed SMS Attack  

Microsoft Academic Search

Malformed messages in different protocols pose a serious threat because they are used to remotely launch malicious activity. Furthermore, they are capable of crashing servers and end points, sometimes with a single message. Recently, it was shown that a malformed SMS can crash a mobile phone or gain unfettered access to it. In spite of this, little research has been

M Zubair Rafique; Muhammad Khurram Khan; Khaled Alghathbar; Muddassar Farooq

2011-01-01

294

Congenital malformations due to antiepileptic drugs  

Microsoft Academic Search

To identify the major risk factors for the increased incidence of congenital malformations in offspring of mothers being treated for epilepsy with antiepileptic drugs (AEDs) during pregnancy and, to determine the relative teratogenic risk of AEDs, we prospectively analyzed 983 offspring born in Japan, Italy, and Canada. The incidence of congenital malformations in offspring without drug exposure was 3.1%, versus

S Kaneko; D Battino; E Andermann; K Wada; R Kan; A Takeda; Y Nakane; Y Ogawa; G Avanzini; C Fumarola; T Granata; F Molteni; G Pardi; L Minotti; R Canger; L Dansky; M Oguni; I Lopes-Cendas; A Sherwin; F Andermann; M.-H Seni; M Okada; T Teranishi

1999-01-01

295

Early presentation of an extremity arteriovenous malformation  

Microsoft Academic Search

We report a very rare case of a high flow arteriovenous malformation (AVM) of the upper limb that caused high output cardiac failure at birth. There was early transfer of the baby to the care of a multidisciplinary team. After radiological intervention, the arm distal to the malformation became ischaemic and an urgent amputation through the upper-humerus followed. Methods of

Jill B. Webb; Mary O'Brien; Philip R. John; Hiroshi Nishikawa

2004-01-01

296

Imaging of head and neck venous malformations  

Microsoft Academic Search

Venous malformations (VMs) are non proliferative lesions that consist of dysplastic venous channels. The aim of imaging is to characterise the lesion and define its anatomic extent. We will describe the plain film, ultrasound (US) (including colour and duplex Doppler), computed tomography (CT), magnetic resonance imaging (MRI), conventional angiographic and direct phlebographic appearances of venous malformations. They will be illustrated

Christine M. Flis; Stephen E. Connor

2005-01-01

297

Maternal Age and Malformations in Singleton Births  

Microsoft Academic Search

Objective: To examine the effect of maternal age on inci- dence of nonchromosomal fetal malformations. Methods: Malformations detected at birth or in the new- born nursery were catalogued prospectively for 102,728 pregnancies, including abortions, stillbirths, and live births, from January 1, 1988 to December 31, 1994. Maternal age was divided into seven epochs. Relative risks (RRs) were used to compare

LISA M. HOLLIER; KENNETH J. LEVENO; MARY ANN KELLY; DONALD D. MCINTIRE; F. GARY CUNNINGHAM

2000-01-01

298

Maternal age and malformations in singleton births  

Microsoft Academic Search

Objective: To examine the effect of maternal age on incidence of nonchromosomal fetal malformations.Methods: Malformations detected at birth or in the newborn nursery were catalogued prospectively for 102,728 pregnancies, including abortions, stillbirths, and live births, from January 1, 1988 to December 31, 1994. Maternal age was divided into seven epochs. Relative risks (RRs) were used to compare demographic variables and

Lisa M Hollier; Kenneth J Leveno; Mary Ann Kelly; Donald D MCIntire; F. Gary Cunningham

2000-01-01

299

Identification of brain malformations: neuropathological approach  

Microsoft Academic Search

IntroductionThe sophistication of prenatal brain imaging (US, MRI) has awakened interest in fetal neuropathology and changed the concept of brain malformations, defined until recently through descriptive terms and considered as senseless accidents of development. Usually, most CNS malformations are documented from a clinical and radiological point of view. However, only a detailed neuropathological study permits their exact phenotype to be

Férechté Encha-Razavi

2003-01-01

300

North American Reporting Center for Amphibian Malformations  

NSDL National Science Digital Library

Created in June, 1997 and funded by the US Geological Survey and Environmental Protection Agency, this site is a response to the discovery of numerous deformed amphibians (mostly frogs) in areas as widespread as Minnesota, California, and Florida. Malformations include extra or missing limbs, missing eyes, and split limbs. The site is intended to serve as a central repository for data on the type and relative frequency of such malformations throughout the US. Researchers hope to use the site to help discover the cause(s) of these deformities. The site contains a map of where malformations have been reported, background on the occurrence and possible causes of defects, numerous images of malformed amphibians, a searchable bibliography, instructions on how to report the discovery of a malformed amphibian, and links to eight related web sites. NARCAM's site also contains a toll-free number for citizen reports.

Center., Northern P.

1997-01-01

301

The use of gallium-67 liver imaging for the early diagnosis of Budd-Chiari syndrome.  

PubMed

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. PMID:6583339

Garty, I; Horovitz, I; Keynan, A

1984-03-01

302

Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: A study in 17 explanted livers  

Microsoft Academic Search

Hepatic parenchymal changes associated with Budd-Chiari syndrome (BCS) have been tentatively explained by combined arterial and portal perfusion disturbances in addition to the complete occlusion of hepatic veins. The aim of this study was to correlate pretransplant course and vascular imaging with pathologic findings in livers explanted for BCS. Seventeen consecutive white patients who underwent transplantation for severe classic BCS

Dominique Cazals-Hatem; Valérie Vilgrain; Pascal Genin; Marie-Hélčne Denninger; François Durand; Jacques Belghiti; Dominique Valla; Claude Degott

2003-01-01

303

Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt  

Microsoft Academic Search

BACKGROUND: Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and\\/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic

Alexandra Zahn; Daniel Gotthardt; Karl Heinz Weiss; Götz Richter; Jan Schmidt; Wolfgang Stremmel; Peter Sauer

2010-01-01

304

Ground-Water Hydrology and Water-Quality Data for Wells, Springs, and Surface-Water Sites in the Bradley-Brumalow Creek Area Near Arnold Air Force Base, Tennessee, September to December 1999.  

National Technical Information Service (NTIS)

Arnold Air Force Base (AAFB) occupies about 40,000 acres in Coffee and Franklin Counties, Tennessee. The primary mission of AAFB is to support the development of aerospace systems. This mission is accomplished through test facilities at Arnold Engineering...

R. A. Aycock C. J. Haugh

2001-01-01

305

Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome.  

PubMed Central

Over 12 years, 22 patients with the Budd-Chiari syndrome were treated surgically. Eighteen underwent a mesenterico-caval shunt (MCS); two, a side-to-side portacaval shunt; one, a mesenterico-atrial shunt (MAS); and one, a liver transplantation (OLT). One patient died after operation from the precipitating condition, and two MCS grafts that thrombosed were restored. All 21 surviving patients remain well, free from ascites, and all shunts are patent after a mean follow-up of 5.6 +/- 1 years, five patients with more than 10 years' follow-up. This long-term survival achieved by portasystemic shunts suggests that they have a major role in the treatment of the Budd-Chiari syndrome. The authors prefer the mesenterico-caval shunt using a jugular graft. This ensures a total portasystemic shunt, avoids subhepatic surgery, and reduces the long-term risk of prosthetic graft thrombosis. The MAS was reserved for cases with complete caval thrombosis. Patients with significant degrees of caval compression were satisfactorily decompressed by MCS. In patients not promptly treated, the disease progresses to cirrhosis, and such patients must be evaluated for transplantation similarly to those with other hepatopathies. Images Fig. 1. Fig. 3. Fig. 4. Fig. 5A and B.

Bismuth, H; Sherlock, D J

1991-01-01

306

Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome.  

PubMed

Over 12 years, 22 patients with the Budd-Chiari syndrome were treated surgically. Eighteen underwent a mesenterico-caval shunt (MCS); two, a side-to-side portacaval shunt; one, a mesenterico-atrial shunt (MAS); and one, a liver transplantation (OLT). One patient died after operation from the precipitating condition, and two MCS grafts that thrombosed were restored. All 21 surviving patients remain well, free from ascites, and all shunts are patent after a mean follow-up of 5.6 +/- 1 years, five patients with more than 10 years' follow-up. This long-term survival achieved by portasystemic shunts suggests that they have a major role in the treatment of the Budd-Chiari syndrome. The authors prefer the mesenterico-caval shunt using a jugular graft. This ensures a total portasystemic shunt, avoids subhepatic surgery, and reduces the long-term risk of prosthetic graft thrombosis. The MAS was reserved for cases with complete caval thrombosis. Patients with significant degrees of caval compression were satisfactorily decompressed by MCS. In patients not promptly treated, the disease progresses to cirrhosis, and such patients must be evaluated for transplantation similarly to those with other hepatopathies. PMID:1953111

Bismuth, H; Sherlock, D J

1991-11-01

307

Interventional occlusion of congenital vascular malformations  

Microsoft Academic Search

Background  New materials and devices have been used in the management of cardiac malformations. In this paper, we present our experience\\u000a with interventional occlusion of congenital vascular malformations.\\u000a \\u000a \\u000a \\u000a Methods  Between January 1997 and December 2005, 139 patients with congenital vascular malformations who had undergone interventional\\u000a occlusion in the Children’s Hospital, Zhejiang University School of Medicine were studied. The clinical data of the

Chun-Hong Xie; Cheng-Sen Xia; Fang-Qi Gong; Yin-Bao Zhou; Wei-Hua Zhu

2009-01-01

308

Fetal MRI clues to diagnose cloacal malformations  

Microsoft Academic Search

Background  Prenatal US detection of cloacal malformations is challenging and rarely confirms this diagnosis.\\u000a \\u000a \\u000a \\u000a Objective  To define the prenatal MRI findings in cloacal malformations.\\u000a \\u000a \\u000a \\u000a Materials and methods  We performed a retrospective study of patients with cloacal malformations who had pre- and post-natal assessment at our institution.\\u000a Fetal MRI was obtained in six singleton pregnancies between 26 and 32 weeks of gestation. Imaging analysis was

Maria A. Calvo-Garcia; Beth M. Kline-Fath; Marc A. Levitt; Foong-Yen Lim; Leann E. Linam; Manish N. Patel; Steven Kraus; Timothy M. Crombleholme; Alberto Peńa

309

Huge arteriovenous malformation in masseter muscle.  

PubMed

Arteriovenous malformation is a tumor characterized by direct connection between an artery and vein without capillaries in-between, and it is commonly located intracranially. Intramuscular arteriovenous malformations are rare in the head and neck region. Less than 1% of the vascular tumors are localized in a muscle, 15% of them are in the head and neck muscles. Among the head and neck muscles, masseter muscle is the most common location, with the rate of 4.9%. The condition of a 36-year-old patient who applied to our clinic with the complaints of progressively increasing pain and progressively growing mass in the right cheek that appeared 1.5 years ago was diagnosed as arteriovenous malformation located in the masseter muscle. After preoperative embolization, the mass was successfully treated with total excision. In this case report, diagnostic and therapeutic tools addressing arteriovenous malformation located in the masseter muscle are discussed in the light of current literature. PMID:19625853

Karaman, Emin; Mercan, Hasan; Ozdilek, Alper; Alimoglu, Yalcin; Korkut, Nazim

2009-07-01

310

Arnol'd diffusion in a system with 2.5 degrees of freedom: Classical and quantum mechanical approaches  

SciTech Connect

Arnol'd diffusion, a universal phenomenon in nonlinear dynamics, is analyzed for a model system with 2.5 degrees of freedom. Only the three primary order resonances are taken into account, and the results obtained by using classical and quantum mechanical approaches are compared. It is shown that the parameter dependence of the rate of quantum Arnol'd diffusion is similar to the classical one, but the quantum diffusion coefficient is smaller by approximately an order of magnitude. It is found that the existence of a threshold with respect to perturbation parameters, pointed out earlier, is not an indispensable feature of quantum Arnol'd diffusion. It is shown that a quantum system with weakly overlapping resonances can exhibit mixed dynamics that has no classical counterpart (diffusion along a resonance superimposed by oscillations across the overlapped resonances).

Malyshev, A. I., E-mail: malyshev@phys.unn.ru; Chizhova, L. A. [Nizhni Novgorod State University (Russian Federation)

2010-05-15

311

Imaging diagnosis--split cord malformation.  

PubMed

The features of a calf with a split cord malformation are described. Clinically, there was severe cervicothoracic kyphoscoliosis and an interscapular dermal sinus associated with cerebrospinal fluid drainage. Using magnetic resonance imaging, complete duplication of the spinal cord at the cervical intumescence was detected. There was associated syringohydromyelia, multiple cervicothoracic vertebral malformations resulting in kyphoscoliosis and rachischisis, herniation of the cerebellar vermis, meningoencephalocele, and calvarial defects. PMID:20166395

Zani, Davide D; De Zani, Donatella; Morandi, Nicola; Biggi, Marianna; Belloli, Angelo G; Riccaboni, Pietro; Rondena, Marco; Di Giancamillo, Mauro; Pravettoni, Davide

312

Maternal diabetes mellitus and infant malformations  

Microsoft Academic Search

OBJECTIVE:To investigate the effects of pregestational, as opposed to gestational, diabetes on infant malformations.METHODS:All women delivering infants at Parkland Hospital between January 1, 1991, and December 31, 2000, were ascertained. Screening for gestational diabetes was methodically employed throughout the study period using National Diabetes Data Group criteria for diagnosis of pregestational and gestational diabetes. Standardized definitions of major infant malformations

Jeanne S Sheffield; Erin L Butler-Koster; Brian M Casey; Donald D McIntire; Kenneth J Leveno

2002-01-01

313

The Microcephaly-Capillary Malformation Syndrome  

PubMed Central

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.

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

2012-01-01

314

Optical color image encryption based on Arnold transform and interference method  

NASA Astrophysics Data System (ADS)

In this paper, we propose a novel method to encrypt a color image based on Arnold transform (ART) and interference method. A color image is decomposed into three independent channels, i.e., red, green and blue, and each channel is then encrypted into two random phase masks based on the ART and interference method. Light sources with corresponding wavelengths are used to illuminate the retrieved phase-only masks during image decryption. The influence of security parameters on decrypted images is also analyzed. Numerical simulation results are presented to illustrate the feasibility and effectiveness of the proposed method.

Chen, W.; Quan, C.; Tay, C. J.

2009-09-01

315

The Quantum Arnold Transformation for the damped harmonic oscillator: from the Caldirola-Kanai model toward the Bateman model  

NASA Astrophysics Data System (ADS)

Using a quantum version of the Arnold transformation of classical mechanics, all quantum dynamical systems whose classical equations of motion are non-homogeneous linear second-order ordinary differential equations (LSODE), including systems with friction linear in velocity such as the damped harmonic oscillator, can be related to the quantum free-particle dynamical system. This implies that symmetries and simple computations in the free particle can be exported to the LSODE-system. The quantum Arnold transformation is given explicitly for the damped harmonic oscillator, and an algebraic connection between the Caldirola-Kanai model for the damped harmonic oscillator and the Bateman system will be sketched out.

López-Ruiz, F. F.; Guerrero, J.; Aldaya, V.; Cossío, F.

2012-08-01

316

A Case of Budd-Chiari Syndrome Associated with Alveolar Echinococcosis  

PubMed Central

Although alveolar echinococcosis (AE) can cause a serious disease with high mortality and morbidity similar to malign neoplasms. A 62-year-old woman admitted to a hospital located in Sivas, Turkey, with the complaints of fatigue and right upper abdominal pain. On contrast abdominal CT, a 54×70×45 mm sized cystic lesion was detected in the left lobe of the liver that was seen to extend to the posterior mediastinum and invade the diaphragm, esophagus, and pericardium. The cystic lesion was seen to be occluding the inferior vena cava and left hepatic vein at the level where the hepatic veins poured into the inferior vena cava. Bilateral pleural effusion was also detected. We discussed this secondary Budd-Chiari Syndrome (BCS) case, resulting from the AE occlusion of the left hepatic vein and inferior vena cava, in light of the information in literature.

Alagozlu, Hakan; Gumus, Cesur; Ali, Celiksoz

2013-01-01

317

A case of budd-Chiari syndrome associated with alveolar echinococcosis.  

PubMed

Although alveolar echinococcosis (AE) can cause a serious disease with high mortality and morbidity similar to malign neoplasms. A 62-year-old woman admitted to a hospital located in Sivas, Turkey, with the complaints of fatigue and right upper abdominal pain. On contrast abdominal CT, a 54×70×45 mm sized cystic lesion was detected in the left lobe of the liver that was seen to extend to the posterior mediastinum and invade the diaphragm, esophagus, and pericardium. The cystic lesion was seen to be occluding the inferior vena cava and left hepatic vein at the level where the hepatic veins poured into the inferior vena cava. Bilateral pleural effusion was also detected. We discussed this secondary Budd-Chiari Syndrome (BCS) case, resulting from the AE occlusion of the left hepatic vein and inferior vena cava, in light of the information in literature. PMID:24039293

Cakmak, Erol; Alagozlu, Hakan; Gumus, Cesur; Alí, Celiksöz

2013-08-30

318

Suboccipital craniotomy for Chiari I results in evoked potential conduction changes  

PubMed Central

Background: Management of Chiari I is controversial, in part because there is no widely used quantitative measurement of decompression. It has been demonstrated that brainstem auditory evoked responses (BAER) and somatosensory evoked potentials (SSEP) have decreased conduction latencies after wide craniectomy. We analyzed these parameters in a suboccipital craniectomy/craniotomy procedure. Methods: Thirteen consecutive patients underwent suboccipital decompression for treatment of symptomatic Chiari I. Craniectomy was restricted to the inferior aspect of the nuchal line, and in most cases the bone flap was replaced. Neuronal conduction was monitored continuously with median nerve somatosensory evoked potentials (M-SEP), posterior tibial nerve somatosensory evoked potentials (T-SEP), BAER, or a combination. The M-SEP N20, T-SEP P37, and BAER V latencies were recorded at four milestones – preoperatively, following craniotomy, following durotomy, and following closure. Results: Five males and eight females, with average age of 9 years, were studied. Clinical improvement was noted in all 13 patients. M-SEP N20 latency decreased from a mean of 18.55 at baseline to 17.75 ms after craniotomy (P = 0.01); to 17.06 ms after durotomy (P = 0.01); and to 16.68 ms after closing (P = 0.02). T-SEP P37 latency did not change significantly. BAER V latency decreased from a mean of 6.25 ms at baseline to 6.14 ms after craniotomy (P = 0.04); to 5.98 ms after durotomy (P = 0.01); and to 5.95 ms after closing (P = 0.45). Conclusion: Significant improvements in conduction followed both craniectomy and durotomy. Bone replacement did not affect these results.

Chen, Jason A.; Coutin-Churchman, Pedro E.; Nuwer, Marc R.; Lazareff, Jorge A.

2012-01-01

319

Confessions of a Would-Be Non-Provincial--Or, the English Teacher and Matthew Arnold's Ghost.  

ERIC Educational Resources Information Center

|Although Matthew Arnold may appear to be the representative of an increasingly irrelevant elitist vision by advocating a culture ultimately dependent on the exclusion of all but the very best in thought and expression, in fact he remains the writer who reminds us of the necessity for a social vision of ourselves superior to any mere…

Walling, W.

320

CONTROL OF SOYBEAN RUST IN A DETERMINATE CULTIVAR AT THE RATTRAY ARNOLD RESEARCH STATION, ZIMBABWE, 2004-05.  

Technology Transfer Automated Retrieval System (TEKTRAN)

Fungicide trials for the management of soybean rust were done at the Rattray Arnold Research Station, Zimbabwe during the 2003-04 growing season. Soybean rust was first recorded in the plots on 10 Mar 05, at 83 DAP, between the second and third fungicide application, thus all treatments were applie...

321

Pilot-scale testing of in situ vitrification of Arnold Engineering Development Center Site 10 contaminated soils  

Microsoft Academic Search

In situ vitrification (ISV), was initially developed to demonstrate a potential technology for disposal of soil contaminated with transuranic waste at the Hanford Site in southeastern Washington. This report presents evidence that ISV also has applicability to Arnold Engineering Development Center (AEDC) contaminated soils, which are contaminated with fuel oils and heavy metals from fire training exercises. This report presents

C. L. Timmerman; M. E. Peterson

1990-01-01

322

An Immigrant's Dream and the Audacity of HopeThe 2004 Convention Addresses of Barack Obama and Arnold Schwarzenegger  

Microsoft Academic Search

Smith and Nimmo assert that contemporary political conventions orchestrate an important legitimation ritual. Asignificant part of this ritual is composed of speechmaking. The 2004 Democratic and Republican conventions had a few notable speech moments. Specifically, the speeches by Illinois State Senator Barack Obama and California Governor Arnold Schwarzenegger garnered attention for both them and their party leaders. Along with the

Babak Elahi; Grant Cos

2005-01-01

323

Entrapment of a floating thrombus in the right atrium by persistent Chiari's network: a barrier to massive pulmonary embolism.  

PubMed

Syncope may be a manifestation of pulmonary embolism. Occlusion of a pulmonary artery with cardiac output drop, sometimes resulting in cardiac arrest, is the main underlying mechanism. In this letter we describe the case of a patient in which the persistence of a long remnant of the embryonal Chiari's network could have worked as an anatomic barrier preventing massive pulmonary embolism and cardiac arrest. PMID:18035436

Rizzello, Vittoria; Lombardo, Antonella; Colizzi, Christian; Pennestrě, Faustino

2007-11-26

324

Massive localised lymphoedema: a rare vascular malformation.  

PubMed

Lymphatic malformations are a subset of congenital vascular malformations, and are caused by a defect in lymphatic development during embryogenesis. When lymphatic mesoderm development is prematurely arrested, it retains it proliferative potential. Stimulus in the future can cause the lesion to proliferate locally without coordination or regulation, resulting in the rare condition known as massive localised lymphoedema (MLL). We present a case report of MLL, a rare and ill-defined soft tissue mass reported in the morbidly obese, with reference to the existing literature. PMID:23761611

Williams, K J; Al-Sakkal, M N; Alsafi, A; Davies, A H

2013-06-12

325

Multiple hamartomas associated with intracranial malformation.  

PubMed

We examined a newborn infant with multiple hamartomas, including an epidermal nevus syndrome and a giant pigmented congenital nevocellular nevus, associated with other structural developmental abnormalities such as nevus flammeus, vascular malformation, cutis aplasia congenita of the scalp, cartilage hamartoma, and a lipodermoid of the conjunctiva. This child had a significant brain malformation, diagnosed by sonography and computerized tomography, consisting of a significant enlargement of the left hemisphere not associated with asymmetry of the skull or facial bones. We suggest a careful investigation of the intracranial structures by computerized tomography and/or ultrasonography in case of either extensive linear nevus sebaceous sequence and/or giant pigmented nevocellular nevus. PMID:3725699

Mimouni, F; Han, B K; Barnes, L; Ballard, J L; Dignan, P S; Kiessling, M A; Lucky, A W

1986-06-01

326

Venous malformations of the genitals: a therapeutic dilemma.  

PubMed

Venous malformations may occur anywhere in the body but are rare in the genitourinary tract and external genitalia. The authors report a case of a venous malformation in the glans penis and discuss the controversy over optimal management. PMID:20442077

Kaufman, Daniel; Feber, Kevin M; Palmer, Lane S; Freedman, Alan M

2010-01-01

327

Diffusion tensor imaging of midline posterior fossa malformations  

Microsoft Academic Search

Background  Diffusion tensor imaging and tractography have been used to evaluate a variety of brain malformations. However, these studies have focused mainly on malformations involving the supratentorial compartments. There is a paucity of data on diffusion tensor imaging of posterior fossa malformations.Objective  To describe the color vector maps and modified or abnormal tracts of midline posterior fossa malformations.Materials and Methods  Diffusion tensor imaging

Elysa Widjaja; Susan Blaser; Charles Raybaud

2006-01-01

328

Hypertension as a presentation of bilateral intrarenal arateriovenous malformation.  

PubMed

Congenital arteriovenous malformations are rare lesions of the kidneys. The first case of bilateral renal arteriovenous malformations was described in 1987. A case of extensive bilateral intrarenal arterivenous malformations presented to us as a case of hypertension. Renal angiography confirmed the diagnosis. Magnetic resonance angiography ruled out these malformations in cerebral circulation, and enhanced abdominal CT scan was normal. The blood pressure of the patient was controlled by medical therapy only. PMID:18212459

El-Lozi, M S; Hadad, A F

329

Congenital malformations by the parental occupation in finland  

Microsoft Academic Search

The Finnish Register of Congenital Malformations, a case-referent register, was used to analyze the associations between the parental occupation and the children born with malformations. The women working in industrial and construction occupations had more children with central nervous system (CNS) and musculoskeletal malformations than the referent mothers. The women employed in transport and communication occupations had more children with

K. Hemminki; P. Mutanen; K. Luoma; I. Saloniemi

1980-01-01

330

Fraser-Cryptophthalmos syndrome with cardiovascular malformations: a rare association.  

PubMed

Fraser-Cryptophthalmos syndrome is a multiple malformation disorder associated variably with cryptophthalmos (hidden eye), anomalies of the head, nose and ears; syndactyly, renal and genital malformations. In this report, we describe a case of Fraser syndrome with cardiovascular malformations: coarctation of aorta, an association not previously described. PMID:14530551

Hambire, Srinivas D; Bhavsar, Priyanka P; B, Meenakshi; Jayakar, Anagha V

2003-09-01

331

Multiple color images encryption by triplets recombination combining the phase retrieval technique and Arnold transform  

NASA Astrophysics Data System (ADS)

We propose a new method for multiple color images encryption by using triplets recombination. In this proposed technique, triplet (R, G and B) components of the secret color image are recorded simultaneously as a real and positive gray image exploiting parallel multi-wavelength recording technology. Arnold transform and phase retrieval algorithm adapted to Fresnel transform domain are used to encode the parallel hybrid result. By using of the proposed technique, each color image is encrypted into a pure phase distribution and combined by superposition. Phase modulation is introduced to eliminate the cross-talk caused by multiple images superposition. The wavelength and transform parameters are provided as keys to enhance the system security. In addition, computer simulations are performed and numerical results are presented to show the validity and efficiency of the proposed method.

Shi, Xiaoyan; Zhao, Daomu; Huang, Yinbo; Pan, Jianjiang

2013-10-01

332

Weak chaos in the disordered nonlinear Schroedinger chain: Destruction of Anderson localization by Arnold diffusion  

SciTech Connect

Research Highlights: > In a one-dimensional disordered chain of oscillators all normal modes are localized. > Nonlinearity leads to chaotic dynamics. > Chaos is concentrated on rare chaotic spots. > Chaotic spots drive energy exchange between oscillators. > Macroscopic transport coefficients are obtained. - Abstract: The subject of this study is the long-time equilibration dynamics of a strongly disordered one-dimensional chain of coupled weakly anharmonic classical oscillators. It is shown that chaos in this system has a very particular spatial structure: it can be viewed as a dilute gas of chaotic spots. Each chaotic spot corresponds to a stochastic pump which drives the Arnold diffusion of the oscillators surrounding it, thus leading to their relaxation and thermalization. The most important mechanism of equilibration at long distances is provided by random migration of the chaotic spots along the chain, which bears analogy with variable-range hopping of electrons in strongly disordered solids. The corresponding macroscopic transport equations are obtained.

Basko, D.M., E-mail: denis.basko@grenoble.cnrs.fr [Laboratoire de Physique et Modelisation des Milieux Condenses, Universite de Grenoble 1 and CNRS, BP166, 38042 Grenoble (France)

2011-07-15

333

Heat conduction in systems with Kolmogorov-Arnold-Moser phase space structure.  

PubMed

We study heat conduction in a billiard channel formed by two sinusoidal walls and the diffusion of particles in the corresponding channel of infinite length; the latter system has an infinite horizon, i.e., a particle can travel an arbitrary distance without colliding with the rippled walls. For small ripple amplitudes, the dynamics of the heat carriers is regular and analytical results for the temperature profile and heat flux are obtained using an effective potential. The study also proposes a formula for the temperature profile that is valid for any ripple amplitude. When the dynamics is regular, ballistic conductance and ballistic diffusion are present. The Poincaré plots of the associated dynamical system (the infinitely long channel) exhibit the generic transition to chaos as ripple amplitude is increased. When no Kolmogorov-Arnold-Moser (KAM) curves are present to forbid the connection of all chaotic regions, the mean square displacement grows asymptotically with time t as tln(t). PMID:23030897

Herrera-González, I F; Pérez-Aguilar, H I; Mendoza-Suárez, A; Tututi, E S

2012-09-25

334

Determining role of Krein signature for three-dimensional Arnold tongues of oscillatory dynamos  

NASA Astrophysics Data System (ADS)

Using a homotopic family of boundary eigenvalue problems for the mean-field ?2 dynamo with helical turbulence parameter ?(r)=?0+???(r) and homotopy parameter ??[0,1] , we show that the underlying network of diabolical points for Dirichlet (idealized, ?=0 ) boundary conditions substantially determines the choreography of eigenvalues and thus the character of the dynamo instability for Robin (physically realistic, ?=1 ) boundary conditions. In the (?0,?,?) space the Arnold tongues of oscillatory solutions at ?=1 end up at the diabolical points for ?=0 . In the vicinity of the diabolical points the space orientation of the three-dimensional tongues, which are cones in first-order approximation, is determined by the Krein signature of the modes involved in the diabolical crossings at the apexes of the cones. The Krein space-induced geometry of the resonance zones explains the subtleties in finding ? profiles leading to spectral exceptional points, which are important ingredients in recent theories of polarity reversals of the geomagnetic field.

Kirillov, Oleg N.; Günther, Uwe; Stefani, Frank

2009-01-01

335

Interventional treatment of pulmonary arteriovenous malformations  

PubMed Central

Pulmonary arteriovenous malformations (PAVM) are congenital vascular communications in the lungs. They act as right to left shunts so that the blood running through these malformations is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high risk of paradoxical emboli to the brain and other organs. These malformations are most commonly seen in hereditary haemorrhagic telangiectasia (HHT) (Mb. Osler-Weber-Rendu syndrome). Nowadays, the generally accepted treatment strategy of first choice is embolization of the afferent arteries to the arteriovenous malformations. It is a minimally invasive procedure and at the same time a lung preserving treatment with a very high technical success, high effectiveness and low morbidity and mortality. Embolization prevents cerebral stroke and abscess as well as pulmonary haemorrhage and further raises the functional level. Embolization is a well-established method of treating PAVM, with a significant effect on oxygenation of the blood. Screening for PAVM in patients at risk is recommended, especially in patients with HHT.

Andersen, Poul Erik; Kjeldsen, Anette Dr?hse

2010-01-01

336

Cardiovascular malformations in experimental congenital diaphragmatic hernia  

Microsoft Academic Search

Background\\/Purpose: Newborns with congenital diaphragmatic hernia (CDH) frequently have associated anomalies that have a major impact on survival rate independent of pulmonary hypoplasia and pulmonary hypertension. Cardiovascular malformations (CVM) represent a major group of lethal extrapulmonary abnormalities that often assume greatest prognostic significance in most CDH studies. Animal models resembling human CDH may aid knowledge of the basic embryology that

Paul D Losty; M. Gwen Connell; Ralf Freese; Stefan Laval; Bruce O Okoye; Audrey Smith; Dietrich Kluth; David A Lloyd

1999-01-01

337

Familial Dandy-Walker malformation and leukodystrophy  

Microsoft Academic Search

We report the first familial cases with two different types of posterior fossa cystic malformation and a leukodystrophic-like aspect on cerebral magnetic resonance imaging (MRI). The girl and her brother had severe encephalopathy, marked hypotonia, absent deep tendon reflexes, macrocrania, gigantism, and dysmorphic face and extremities. The girl had generalized seizures. The boy had unilateral cataract and bilateral optic atrophy.

Véronique T. Humbertclaude; Philippe A. Coubes; Nicolas Leboucq; Bernard B. Echenne

1997-01-01

338

Unilateral regional odontodysplasia with ipsilateral mandibular malformation.  

PubMed

Regional odontodysplasia is a rare developmental anomaly with an unknown cause. This disorder involves both the ectodermal and mesodermal dental layers. The affected teeth generally cannot be rehabilitated for functional use; therefore, the treatment of choice is extraction with prosthetic replacement. A unique case of unilateral regional odontodysplasia with ipsilateral mandibular malformation is reported. PMID:2356083

Raez, A G

1990-06-01

339

Obstetric complications and congenital malformation in schizophrenia  

Microsoft Academic Search

Recent years have witnessed increasingly intense research activity concerning early life somatic trauma and dysmorphogenesis which are associated with the later development of schizophrenia. The two somatic factors that have received the most extensive scientific attention as antecedents of schizophrenia are obstetric complications (OCs) and the congenital malformations termed `minor physical anomalies' (MPAs). Head circumference (HC) at birth has also

Thomas F McNeil; Elizabeth Cantor-Graae; Baher Ismail

2000-01-01

340

Gastrointestinal malformations in Gorgan, North of Iran: epidemiology and associated malformations  

Microsoft Academic Search

The aim of this prospective study was to evaluate the prevalence and pattern of gastrointestinal malformations (GIM) among\\u000a Iranian newborns in Gorgan, North of Iran. From 1998 through 2003, 37,951 live births in Dezyani hospital in Gorgan, North\\u000a of Iran, were screened for gastrointestinal malformations. Clinical and demographic factors of diagnosed cases were recorded\\u000a in a pre-designed questionnaire for analysis;

Mohammad Jafar Golalipour; Elham Mobasheri; Kaniz-Reza Hoseinpour; Abbas Ali Keshtkar

2007-01-01

341

Ground-Water Levels and Water-Quality Data for Wells in the Spring Creek Area Near Arnold Air Force Base, Tennessee, April and May 2000.  

National Technical Information Service (NTIS)

Arnold Air Force Base (AAFB) occupies about 40,000 acres in Coffee and Franklin Counties, Tennessee. Numerous site-specific groundwater contamination investigations have been conducted at designated solid waste management units (SWMU's) at AAFB. Seve...

S. D. Williams R. A. Aycock

2001-01-01

342

Humanizing Democracy: Matthew Arnold's Nineteenth-Century Call for a Common, Higher, Educative Pursuit of Happiness and Its Relevance to Twenty-first Century Democratic Life.  

ERIC Educational Resources Information Center

Examines a nineteenth century recommendation by Matthew Arnold for democratic educational reform calling for a broadly accessible liberal education to cultivate a magnanimous and civic-minded democratic populace. Discusses how the old vision might become a new blueprint. (SLD)

Novak, Bruce

2002-01-01

343

Congenital occipitoatlantoaxial malformations in the horse.  

PubMed

From a clinical, radiological and morphological study of 9 horses with congenital malformations of the occiput, atlas and axis, and from a study of 2 reported cases, 3 diseases were defined: A. Familial occipitalisation of the atlas with atlantalisation of the axis in Arabian horses (7 cases in this report and the case reported by Leipold, et al., 1974). These horses had congenital atlantooccipital fusion, hypoplasia of the atlas and dens, malformation of the axis and modification of the atlantoaxial joint. B. Congenital asymmetrical occipitoatlantoaxial malformation (2 cases in this report). A Standardbred and a Morgan horse had atlantooccipital fusion, a wedge shaped vertebral piece attached to the caudal end of the axis and sigmoid scoliosis of the cervical vertebrae. C. Asymmetrical atlantooccipital fusion (the case reported by Schmaltz, 1915). This horse of an unknown breed had asymmetrical fusion between the atlas and occiput and cervical scoliosis. The clinical syndromes shown by horses with these malformations were variable but were broadly classified as: 1. Foal dead at birth, seen in one foal with A. 2. Tetraparesis at birth, seen in 5 foals with A. These foals were born with signs varying from tetraparesis to tetraplegia. 3. Progressive ataxia, seen in 2 foals with A. Clinical signs were due to a progressive focal cervical compressive myelopathy. 4. Congenital cervical scoliosis/deviated head, seen in the 2 horses with B and the horse with C. These horses had no signs of spinal cord or brain disease. The diagnoses were made clinically by palpation of the occipitoatlantoaxial region and were confirmed radiographically and/or by post mortem examination in all except one case. Pedigree analysis showed the familial nature of the particular occipitoatlantoaxial malformation seen in horses of only the Arabian breed. PMID:565704

Mayhew, I G; Watson, A G; Heissan, J A

1978-04-01

344

Extensive Intramuscular Venous Malformation in the Lower Extremity  

PubMed Central

Typical venous malformations are easily diagnosed by skin color changes, focal edema or pain. Venous malformation in the skeletal muscles, however, has the potential to be missed because their involved sites are invisible and the disease is rare. In addition, the symptoms of intramuscular venous malformation overlaps with myofascial pain syndrome or muscle strain. Most venous malformation cases have reported a focal lesion involved in one or adjacent muscles. In contrast, we have experienced a case of intramuscular venous malformation that involved a large number of muscles in a lower extremity extensively.

Chul, Jung Ho; Park, Byung Kyu; Park, Myung Kyu

2012-01-01

345

Hepatocellular carcinoma associated with budd-chiari syndrome: imaging features and transcatheter arterial chemoembolization  

PubMed Central

Background Budd–Chiari syndrome (BCS) often leads to hepatocellular carcinoma (HCC). Transcatheter arterial chemoembolization (TACE) has been increasingly used to treat BCS patients with HCC. The purposes of this study were to illustrate imaging features in BCS patients with HCC, and to analyze the effects of TACE on BCS patients with HCC. Methods 246 consecutive patients with primary BCS were retrospectively studied. 14 BCS patients with HCC were included in this study. BCS were treated with angioplasty and/or stenting, and HCC were managed with TACE. Imaging features on ultrasonography, CT, MRI, and angiography and the serum AFP level were analyzed. Results Inferior vena cava block and stricture of hepatic venous outflow tract more frequently occurred. Portal vein invasion was found in only 2 patients (14.2%). Imaging studies showed that most nodules of HCC were near the edge of liver, irregular, more than 3 cm in diameter, heterogeneous mass and solitary (?3 nodules). HCC in patients associated with BCS was isointense or hypointense in nonenhanced CT images, and exhibited heterogeneous enhancement during the arterial phase and washout during the portal venous phase on enhanced CT and MRI. The serum AFP level significantly declined after TACE treatment. Conclusions BCS patients with inferior vena cava block and stricture of hepatic venous outflow tract seems to be associated with HCC. A single, large, irregular nodule with a peripheral location appears to be HCC. TACE can effectively treat HCC in BCS patients.

2013-01-01

346

Thrombophilic dimension of Budd chiari syndrome and portal venous thrombosis--a concise review.  

PubMed

Budd chiari syndrome (BCS) is characterized by venous outflow obstruction either at hepatic veins or inferior vena cava, while portal vein thrombosis (PVT) is the consequence of thrombotic occlusion in the extrahepatic venous system. The aetiology of both these disorders is complicated wherein genetic, acquired and local factors interact in the pathogenesis. Among the inherited thrombophilia, factor V Leiden mutation has shown stronger association with BCS than PVT while the converse is true for prothrombin G20210A mutation. Very few studies are available on the role of fibrinolytic potential or the single nucleotide polymorphisms (SNPs) of fibrinolysis proteins, in both BCS and PVT. Among the acquired thrombophilia, myeloproliferative disorders (MPD) are the most frequent cause, while antiphospholipid antibodies (APA) and hyperhomocysteinemia have not shown very strong association with BCS and PVT. Oral contraceptives, infection, chronic inflammatory diseases like Behcets syndrome, inflammatory bowel disease, tumors, paroxysmal nocturnal hemoglobinuria (PNH), pregnancy, puerperium, poor nutrition are some of the other acquired and local risk factors associated with both these disorders. There exists a clear geographical variation both in the clinical manifestation and the underlying aetiology. Almost all the studies have proved that a multifactorial aetiology is the requisite for the manifestation. Evaluation of an extensive thrombophilia profile is essential for optimal management of patients which is further justified with the availability of specific treatment options for at least some thrombophilia markers. PMID:20961602

Shetty, Shrimati; Ghosh, Kanjaksha

2010-10-20

347

Technical evaluation of the monitoring of electric power to the reactor protection system for the Duane Arnold Energy Center  

SciTech Connect

This report documents the technical evaluation of the monitoring of electric power to the reactor protection system (RPS) at the Duane Arnold Energy Center. The evaluation is to determine if the proposed design modification will protect the RPS from abnormal voltage and frequency conditions which could be supplied from the power supplies and will meet certain requirements set forth by the Nuclear Regulatory Commission. The proposed design modifications will protect the RPS from sustained abnormal voltage and frequency conditions from the supplying sources.

Selan, J.C.

1982-03-01

348

GI-Associated Hemangiomas and Vascular Malformations  

PubMed Central

Hemangiomas and vascular malformations of the gastrointestinal tract, rare clinical entities, present as overt or occult bleeding. They can be distributed throughout the intestinal digestive system, or present as a singular cavernous hemangioma or malformation, which is often located in the rectosigmoid region. Misdiagnosis is common despite characteristic radiographic features such as radiolucent phleboliths on plain film imaging and a purplish nodule on endoscopy. Adjunctive imaging such as computed tomography and magnetic resonance imaging are suggested as there is potential for local invasion. Endorectal ultrasound with Doppler has also been found to be useful in some instances. Surgical resection is the mainstay of treatment, with an emphasis on sphincter preservation. Nonsurgical endoscopic treatment with banding and sclerotherapy has been reported with success, especially in instances where an extensive resection is not feasible.

Yoo, Stephen

2011-01-01

349

Diphallus with anorectal malformation-case report.  

PubMed

Diphallus is a very rare condition. We report a case of a newborn with absent anal opening and duplication of external genitalia. Examination of the external genitalia showed two well formed penises with fully descended testis within each of the separate hemiscrotums along with soft tissue mass resembling accessory buttock behind the hemiscrotum. Staged reconstruction of duplicated genitalia and anorectal malformation was done. We achieved excellent cosmetic and functional external genitalia. PMID:20223333

Mukunda, Ramachandra; Bendre, Pradnya S; Redkar, Rajeev G; Hambarde, Sandeep

2010-03-01

350

The spectrum of anorectal malformations in Africa  

Microsoft Academic Search

Anorectal malformations (ARM) remain a significant birth defect with geographic variation in incidence, individual phenotypes\\u000a and regional geographic subtypes. Although early studies indicated a low incidence in Black patients, there is a great paucity\\u000a of knowledge as to the types, frequency and incidence of ARMs encountered in the African continent and their associated anomalies.\\u000a Current evidence suggests a significant clinical

S. W. Moore; A. Alexander; D. Sidler; J. Alves; G. P. Hadley; A. Numanoglu; B. Banieghbal; M. Chitnis; D. Birabwa-Male; B. Mbuwayesango; A. Hesse; K. Lakhoo

2008-01-01

351

Imaging of arteriovenous malformation following stereotactic radiosurgery  

Microsoft Academic Search

Background. Stereotactic radiosurgery allows for a high dose of focused radiation to be delivered to a small lesion such as an arteriovenous\\u000a malformation (AVM). The clinical change and brain response over time to this localized high-dose radiation can be quite striking.\\u000a Objective. The objective of this study to describe and analyse the imaging changes following radiotherapy for AVMs. Materials and

Jeffrey V. Tranchida; Christopher J. Mehall; Thomas L. Slovis; Miguel Lis-Planells

1997-01-01

352

Bronchopulmonary foregut malformations: embryology, radiology and quandary  

Microsoft Academic Search

.  Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present\\u000a at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions\\u000a with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each\\u000a lesion, both antenatally and postnatally, is described and illustrated. A

N. A. Barnes; D. W. Pilling

2003-01-01

353

Percutaneous Treatment of Peripheral Vascular Malformations  

Microsoft Academic Search

Vascular malformations arise from errors in the morphological processes that shape the embryonic\\u000avascular system during fetal development. These developmental errors result in abnormal clusters of\\u000ablood vessels. Although these lesions are present at birth, they might not become visible until weeks or\\u000aeven years after birth. Typically, the lesions grow in proportion to the growth of the child. A

Linden van der E

2011-01-01

354

The challenge of large vascular malformations  

Microsoft Academic Search

Vascular malformations are of the low- or high-flow variety, the latter variety also having shunting characteristics. In this\\u000a manuscript, significant and challenging conditions will be presented. The high-flow lesions can cause excessive growth in\\u000a the local area, and severe bleeding is always a possibility, either spontaneous or during surgery. Clinical examination, angiography,\\u000a and Doppler studies confirm the diagnosis. In treatment

Ian T. Jackson

2009-01-01

355

Rhombencephalosynapsis - isolated anomaly or complex malformation?  

PubMed Central

Summary Background: Rhombencephalosynapsis (RES) is a rare malformation of the posterior cranial fossa, characterized by fusion of the cerebellar hemispheres, medial cerebellar peduncles and dentate nuclei. Over the period of 7 years 8 cases of this anomaly have been diagnosed in two pediatric centers in Warsaw including one on the prenatal magnetic resonance imaging (MRI). Material/Methods: Material consists of involves one fetus examined at the gestational age of 27 and 33 weeks and 7 children (5 girls and 2 boys) aged 8 months – 16 years. All of them underwent brain MRI with the use of 1.5T scanners. Results: In 1 case RES was an isolated anomaly, in 1 case it was accompanied by hydrocephalus only, in the remaining 6 cases RES was an element of a complex malformation. The additional anomalies were as follows: callosal hypoplasia in 3 children, abnormalities of gyration in 2, brainstem hypoplasia in 2, isolated fourth ventricle in 1, abnormal white matter signal intensity in 4 (in 2 cases in supratentorial compartment, in 1 in the cerebellum and in 1 in the pons), abnormally dilated extraaxial fluid collections in 2, syringohydromyelia in 2. In 5 cases RES was total, in 3 – partial. Conclusions: Rhombencephalosynapsis has a very characteristic appearance on magnetic resonance imaging which allows diagnosis of this malformation at any age, including prenatal period.

Bekiesinska-Figatowska, Monika; Jurkiewicz, Elzbieta; Szkudlinska-Pawlak, Sylwia; Malczyk, Katarzyna; Nowak, Katarzyna

2012-01-01

356

Malformations of cortical development and epilepsy  

PubMed Central

Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures, A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etioiogic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD, The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaiy, classical iissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented.

Leventer, Richard J.; Guerrini, Renzo; Dobyns, William B.

357

[Current problems of the treatment of spina bifida aperta].  

PubMed

Experience in the treatment of 311 children with spina bifida aperta is generalized. The authors emphasize the hazard of infectious complications, which were the cause of death of 26.3% of children who were operated on 2 days after birth and before epithelization of the meningeal cavity. Internal hydrocephalus is congenital and linked etiopathogenetically with the Arnold-Chiari malformation. Treatment of internal hydrocephalus consists in ventriculoatrial and ventriculoperitoneal shunting. Spinal draining operations are not recommended. Postoperative mortality in spina bifida aperta has reduced to 3% in the recent years. PMID:3389012

Vetska, P; Tsekov, Kh; Undzhiian, S

358

Malformations in neotropical viperids: qualitative and quantitative analysis.  

PubMed

Malformations can occur in all living species, but there is little information about anomalies that occur in snakes and their frequency. This study assessed malformations in newborn South American pit vipers (Bothrops jararaca) and South American rattlesnakes (Crotalus durissus) from wild captured pregnant females (240 and 35 litters, respectively). Newborn snakes were measured, weighed, sexed and studied grossly and by radiography for the presence of malformations. Ninety-five malformed pit vipers were identified from 4,087 births (2.3%), while 36 malformed rattlesnakes were found from 324 births (11.1%). Spinal abnormalities were the most common in both species, followed by fusion of ventral scales. Pit vipers showed a greater range of malformations including schistosomia (22.1%), kinked tail (13.7%), bicephaly (3.1%) and hydrocephaly (2.1%). PMID:23885804

Sant'anna, S S; Grego, K F; Lorigados, C A B; Fonseca-Pinto, A C B C; Fernandes, W; Sá-Rocha, L C; Catăo-Dias, J L

2013-07-23

359

Cytoarchitecture and Transcriptional Profiles of Neocortical Malformations in Inbred Mice  

PubMed Central

Malformations of neocortical development are associated with cognitive dysfunction and increased susceptibility to epileptogenesis. Rodent models are widely used to study neocortical malformations and have revealed important genetic and environmental mechanisms that contribute to neocortical development. Interestingly, several inbred mice strains commonly used in behavioral, anatomical, and/or physiological studies display neocortical malformations. In the present report we examine the cytoarchitecture and myeloarchitecture of the neocortex of 11 inbred mouse strains and identified malformations of cortical development, including molecular layer heterotopia, in all but one strain. We used in silico methods to confirm our observations and determined the transcriptional profiles of cells found within heterotopia. These data indicate cellular and transcriptional diversity present in cells in malformations. Furthermore, the presence of dysplasia in nearly every inbred strain examined suggests that malformations of neocortical development are a common feature in the neocortex of inbred mice.

Ramos, Raddy L.; Smith, Phoebe T.; DeCola, Christopher; Tam, Danny; Corzo, Oscar

2008-01-01

360

Congenital malformations at birth — A prospective study from south India  

Microsoft Academic Search

Congenital malformations were studied prospectively from September 1989 to December 1992 covering 12,797 consecutive deliveries.\\u000a The overall incidence of malformations was 3.7% and it was 3.2% among live births and 15.7% among still births. Three hundred\\u000a and ninety seven birth defects were observed among 308 live births and 72 among 45 still births. The incidence of malformation\\u000a was significantly higher

B. Vishnu Bhat; Lokesh Babu

1998-01-01

361

Assessment of dysphagia in infants with facial malformations  

Microsoft Academic Search

In infants with facial malformation, dysphagia is frequent and can lead to respiratory and nutritional complications whatever\\u000a the phenotype. The aim of our study was to assess the severity and mechanisms of dysphagia in infants with facial malformations\\u000a in order to guide therapeutic management. Forty-two newborn infants with dysphagia and recognizable malformation patterns\\u000a other than isolated Pierre Robin sequence had:

Jean-Jacques Baudon; Francis Renault; Jean-Michel Goutet; Valérie Biran-Mucignat; Georges Morgant; Erea-Noel Garabedian; Marie-Paule Vazquez

2009-01-01

362

Hemangiomas and Vascular Malformations: Current Theory and Management  

PubMed Central

Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies.

Richter, Gresham T.; Friedman, Adva B.

2012-01-01

363

Periodic orbits of the ensemble of Sinai-Arnold cat maps and pseudorandom number generation  

NASA Astrophysics Data System (ADS)

We propose methods for constructing high-quality pseudorandom number generators (RNGs) based on an ensemble of hyperbolic automorphisms of the unit two-dimensional torus (Sinai-Arnold map or cat map) while keeping a part of the information hidden. The single cat map provides the random properties expected from a good RNG and is hence an appropriate building block for an RNG, although unnecessary correlations are always present in practice. We show that introducing hidden variables and introducing rotation in the RNG output, accompanied with the proper initialization, dramatically suppress these correlations. We analyze the mechanisms of the single-cat-map correlations analytically and show how to diminish them. We generalize the Percival-Vivaldi theory in the case of the ensemble of maps, find the period of the proposed RNG analytically, and also analyze its properties. We present efficient practical realizations for the RNGs and check our predictions numerically. We also test our RNGs using the known stringent batteries of statistical tests and find that the statistical properties of our best generators are not worse than those of other best modern generators.

Barash, L.; Shchur, L. N.

2006-03-01

364

Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt  

PubMed Central

AIM: To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre. METHODS: Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men, median age 40 years (range 17-66 years)]. Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals. Management consisted of tests for underlying haematological, endocrinological, or hypercoagulative disorders parallel to initiation of specific treatment of BCS. RESULTS: BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders. Forty-three percents had symptoms for less than one week with ascites as the most prevalent finding. Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics. The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo), and none required subsequent liver transplantation. Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics. A total of 14 TIPS revisions were needed, mostly of uncovered stents. Two died during follow-up: One non-TIPS patient worsened after 6 mo and died in relation to transplantation, and one TIPS patient died 4 years after the TIPS-procedure, unrelated to BCS. CONCLUSION: In our BCS cohort TIPS-treated patients have near-complete survival, reduced need for diuretics and compared to historical data a reduced need for liver transplantation.

Neumann, Anders Bay; Andersen, Stine Degn; Nielsen, Dennis T?nner; Holland-Fischer, Peter; Vilstrup, Hendrik; Gr?nbaek, Henning

2013-01-01

365

Associated malformations among infants with radial ray deficiency.  

PubMed

Infants with radial ray deficiencies very often have other associated congenital anomalies. The reported frequency and types of associated malformations vary between different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with radial ray deficiencies in a geographically well-defined population from 1979 to 2004 of 346,831 consecutive births. Of the 73 infants with radial ray deficiencies born during this period (prevalence at birth of 2.1 per 10,000), 75% had associated malformations. Infants with associated malformation were divided into recognizable conditions (16 (22%) infants with chromosomal and 20 (27%) with non chromosomal conditions), and non recognizable conditions (19 (26%) infants with multiple malformations). Trisomies 18 and autosomal deletions were the most frequent chromosomal abnormalities. VACTERL association, thrombocytopenia absent radii syndrome, Fanconi anemia and Holt-Oram syndrome were most often present in recognizable non chromosomal conditions. Malformations in the musculoskeletal, cardiovascular and urogenital systems were the most common other anomalies in infants with multiple malformations and non recognizable conditions. The frequency of associated malformations in infants with radial ray deficiencies emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations especially musculoskeletal, cardiac and urogenital systems anomalies may need to be considered in infants with radial ray deficiencies, and referral of these infants for genetic evaluation and counseling seems warranted. PMID:24032294

Stoll, C; Dott, B; Alembik, Y; Roth, M-P

2013-01-01

366

Facial nerve mapping and monitoring in lymphatic malformation surgery  

Microsoft Academic Search

ObjectiveEstablish the efficacy of preoperative facial nerve mapping and continuous intraoperative EMG monitoring in protecting the facial nerve during resection of cervicofacial lymphatic malformations.

Jospeh Chiara; Greg Kinney; Jefferson Slimp; Gi Soo Lee; Sepehr Oliaei; Jonathan A. Perkins

2009-01-01

367

The numerical detection of the Arnold web and its use for long-term diffusion studies in conservative and weakly dissipative systems  

NASA Astrophysics Data System (ADS)

The celebrated KAM and Nekhoroshev theorems provide essential informations about the long term dynamics of quasi-integrable Hamiltonian systems. In particular, long-term instability of the action variables can be observed only in the so-called Arnold web, which is the complement in the phase-space of all KAM invariant tori, and only on the very long times which depend exponentially on an inverse power of the perturbation parameter. Though the structure of the Arnold's web was clearly explained already on Arnold's 1963 article, its numerical detection with a precision sufficient to reveal exponentially slow diffusion of the actions through the web itself has become possible only in the last decade with the extensive computation of dynamical indicators. In this paper, we first review the detection method that allowed us to compute the Arnold web, and then we discuss its use to study the long-term diffusion through the web itself. We also show that the Arnold web of a quasi-integrable Hamiltonian system is useful to track the diffusion of orbits of weakly dissipative perturbations of the same Hamiltonian system.

Guzzo, Massimiliano; Lega, Elena

2013-06-01

368

Endovascular management of spinal arteriovenous malformations.  

PubMed

Spinal arteriovenous malformations (sAVMs) are rare vascular lesions whose natural history remains incompletely defined. Several classification schemes for sAVMs have evolved based on an improved understanding of the anatomic characteristics as well as pathophysiologic behavior of these arteriovenous shunts. Advances in endovascular technology have inspired the adoption of interventional techniques both as stand-alone treatment and as part of a multi-modality management paradigm for sAVMs. Further refinements in liquid embolic agents as well as improved microcatheter navigability will contribute to an ever-expanding role for endovascular intervention in the management of these lesions. PMID:22935350

Ducruet, Andrew F; Crowley, R Webster; McDougall, Cameron G; Albuquerque, Felipe C

2012-08-30

369

Congenital malformation associated with intrauterine contraceptive device.  

PubMed Central

Two infants with fibular aplasia and related limb-reduction defects were born to mothers who were using copper-containing intrauterine contraceptive devices. The possibility of a casual association is consistent with the known hazards of intrauterine devices and cannot be excluded on the available experimental evidence. More information is urgently needed, and searches, including radiography of placenta and pelvis, should be made for such devices in mothers who have used them in the past and who give birth to malformed infants. Images FIG 1 FIG 2

Barrie, H

1976-01-01

370

Intracranial arteriovenous malformation presenting with papilloedema.  

PubMed

Intracranial arteriovenous malformations (AVM) typically present clinically with haemorrhage, seizures or focal neurological deficit. AVM do not usually have a substantial mass effect or cause raised intracranial pressure. Rarely shunting of arterial blood into the venous system can cause raised venous pressure and consequently raised CSF pressure. The case described here is of a 37 year old man with an AVM who presented with headache and papilloedema secondary to raised CSF pressure. Following partial embolisation, to reduce fistulous flow, and stereotactic radiosurgery (SRS), to reduce the size of the AVM, both the headache and papilloedema resolved. PMID:24108890

Hayton, Tom; Gan, Peter; Sadalage, Girija; Burdon, Michael

2013-11-01

371

[Pelvic arteriovenous malformation : a case report].  

PubMed

A 70-year-old male presented with intermittent macroscopic hematuria. There was no history of previous trauma or pelvic operation. At first, we were unable to clarify the origin of the hematuria, but 3D computed tomography revealed an arteriovenous malformation (AVM) consisting of multiple feeding vessels arising from the bilateral, especially right, internal iliac artery. Treatment with transcatheter arterial embolization (TAE) with a combination of lipiodol and N-butyl-2-cyanoacrylate twice was effective. He needs to be followed up carefully for recurrence of AVM. PMID:23945325

Suzuki, Kotaro; Kusuda, Yuji; Yamada, Yuji; Nose, Ryuichiro; Matsui, Takashi; Sanda, Yuki; Mori, Takeki; Sugimoto, Koji

2013-07-01

372

Base-flow data in the Arnold Air Force Base area, Tennessee, June and October 2002  

USGS Publications Warehouse

Arnold Air Force Base (AAFB) occupies about 40,000 acres in Coffee and Franklin Counties, Tennessee. The primary mission of AAFB is to support the development of aerospace systems. This mission is accomplished through test facilities at Arnold Engineering Development Center (AEDC), which occupies about 4,000 acres in the center of AAFB. Base-flow data including discharge, temperature, and specific conductance were collected for basins in and near AAFB during high base-flow and low base-flow conditions. Data representing high base-flow conditions from 109 sites were collected on June 3 through 5, 2002, when discharge measurements at sites with flow ranged from 0.005 to 46.4 ft3/s. Data representing low base-flow conditions from 109 sites were collected on October 22 and 23, 2002, when discharge measurements at sites with flow ranged from 0.02 to 44.6 ft3/s. Discharge from the basin was greater during high base-flow conditions than during low base-flow conditions. In general, major tributaries on the north side and southeastern side of the study area (Duck River and Bradley Creek, respectively) had the highest flows during the study. Discharge data were used to categorize stream reaches and sub-basins. Stream reaches were categorized as gaining, losing, wet, dry, or unobserved for each base-flow measurement period. Gaining stream reaches were more common during the high base-flow period than during the low base-flow period. Dry stream reaches were more common during the low base-flow period than during the high base-flow period. Losing reaches were more predominant in Bradley Creek and Crumpton Creek. Values of flow per square mile for the study area of 0.55 and 0.37 (ft3/s)/mi2 were calculated using discharge data collected on June 3 through 5, 2002, and October 22 and 23, 2002, respectively. Sub-basin areas with surplus or deficient flow were defined within the basin. Drainage areas for each stream measurement site were delineated and measured from topographic maps. Change in flow per square mile for each sub-basin was calculated using data from each base-flow measurement period. The calculated values were used to define the areas of surplus or deficient flow for high and low base-flow conditions. Many areas of deficient flow were present throughout the study area under high and low base-flow conditions. Most areas of deficient flow were in the headwater basins. Fewer areas of surplus flow were present under low base-flow conditions than during the high base-flow conditions. The flow per square mile for each major tributary basin in the study area also was calculated. The values of flow per square mile for the Dry Creek, Spring Creek, and Wiley Creek basins were greatest under both high and low base-flow conditions.

Robinson, John A.; Haugh, Connor J.

2004-01-01

373

Jaw development and malformation in cultured striped trumpeter Latris lineata  

Microsoft Academic Search

Intensive culture of striped trumpeter (Latris lineata) has resulted in a high incidence of jaw malformation in juveniles. In this study, cranial and jaw development in striped trumpeter was described in cultured larvae reared in greenwater on rotifers and Artemia. Jaw malformation was only evident in post-flexion larvae greater than the standard length of 10 mm and was characterised by

J. M Cobcroft; P. M Pankhurst; J Sadler; P. R Hart

2001-01-01

374

Congenital malformations of the external and middle ear  

Microsoft Academic Search

With the focus on imaging, this paper gives a summarized view of the present knowledge on fields, which are necessary to know for a profound understanding of congenital malformations of the external and middle ear. Typical and less typical combinations of malformed parts of the ear can be derived from the embryogenesis. Clinical signs and audiometric findings lead to diagnosis

S. Kösling; M. Omenzetter; S. Bartel-Friedrich

2009-01-01

375

Tumeurs et malformations vasculaires, classification anatomopathologique et imagerie  

Microsoft Academic Search

The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects

M. Wassef; R. Vanwijck; P. Clapuyt; L. Boon; G. Magalon

2006-01-01

376

Microsurgery for cerebral arteriovenous malformation management: a Siberian experience  

Microsoft Academic Search

Cerebral vascular malformations remain among the most difficult neurosurgical entities to treat. We report a retrospective study of the outcome in 95 consecutive patients with angiographically revealed arteriovenous malformations (AVMs). Fifty-four patients underwent microsurgical total AVM removal (group I). Forty-one patients who refused open surgery (group II) were managed either by endovascular embolisation (16 cases), radiosurgery (three) or followed up

Alexei L. Krivoshapkin; Evstafy G. Melidy

2005-01-01

377

Vascular malformations in the uterus: ultrasonographic diagnosis and conservative management  

Microsoft Academic Search

Objective: To investigate the presence and outcome of uterine vascular malformations in women with abnormal premenopausal bleeding. Study design: In this observational study 265 consecutive patients with abnormal premenopausal bleeding were examined by the same ultrasonographer with transvaginal gray-scale ultrasonography and color Doppler imaging. A final diagnosis of uterine vascular malformation was based on ultrasonographic findings, hysteroscopy or histological findings.

Dirk Timmerman; Thierry Van den Bosch; Karen Peeraer; Ellen Debrouwere; Dominique Van Schoubroeck; Luc Stockx; Bernard Spitz

2000-01-01

378

Ischio-spinal dysostosis: a previously unrecognised combination of malformations  

Microsoft Academic Search

Background. Ischial hypoplasia is an extremely rare malformation, both as an isolated anomaly and as a syndromic constituent. Objective. To elucidate the clinical and radiological characteristics in five patients with the combination of ischial hypoplasia and\\u000a spinal malformations. Materials and methods. The clinical records and radiographs of two females and three males, ranging in age from 3 months to 38

Gen Nishimura; Mamori Kimizuka; Ryouji Shiro; Eiji Nii; Masaki Nishiyama; Toshio Kawano; Tetsui Kaku; Yuko Kawada

1999-01-01

379

The ear and its malformations: strange beliefs and misconceptions  

Microsoft Academic Search

Objective. To explore the strange beliefs and misconceptions related to the ear and its malformations, and how these have changed from ancient times until today.Methods. Ancient documents, journal articles, and history books were studied to research ancient and current beliefs and misconceptions with regard to the ear and its malformations.Results. The ear has been the centre of various beliefs and

Irene E Gamatsi; Thomas P Nikolopoulos; Dimitra E Lioumi

2003-01-01

380

Cardiovascular malformations in congenital diaphragmatic hernia: Human and experimental studies  

Microsoft Academic Search

Background\\/Purpose: Cardiovascular malformations (CVM) associated with congenital diaphragmatic hernia (CDH) account in part for the high mortality caused by this defect. The aim of this study is to examine the nature of these malformations in a large series of autopsies and to assess if similar defects are also present in rat fetuses with experimental CDH.Methods: The incidence of CVM and

Lucia Migliazza; Christian Otten; Huimin Xia; Jose I. Rodriguez; Juan A. Diez-Pardo; Juan A. Tovar

1999-01-01

381

Bronchogenic Cysts: A Rare Congenital Cystic Malformation of the Lung  

Microsoft Academic Search

Purpose Bronchogenic cysts are rare congenital cystic malformations of the lung. We retrospectively analyzed ten cases of bronchogenic cyst (BC) to reinforce the importance of recognizing this malformation. Methods Between 1985 and 2000, ten pediatric patients with BC were treated surgically in our department. Their clinical presentation, radiological, operative, and pathological findings were analyzed retrospectively. Results There were five boys

Gülay A. Tireli; Hüseyin Özbey; Abdülkerim Temiz; Tansu Salman; Alaaddin Çelik

2004-01-01

382

Spinal meningeal malformations in children (without meningoceles or meningomyeloceles)  

Microsoft Academic Search

Multiple meningeal malformations are described: anterior or lateral meningoceles, extradural meningeal cysts, and intradural arachnoid cysts. All diverticulae appear to be extensions of the subarachnoid space, producing symptoms early or later. It is impossible to unify all these lesions because they cause multiple pathological conditions, depending upon the anatomical form or level, other systemic malformations, spinal abnormalities, or associated familial

Jacques Richaud

1988-01-01

383

Successful percutaneous coil occlusion of a large pulmonary arteriovenous malformation.  

PubMed

Pulmonary arteriovenous malformation is one of the rarest congenital anomalies of cardiovascular system. We present a case of 30-year-old female with a large pulmonary arteriovenous malformation (PAVM) arising from the right lower pulmonary artery and draining to the left atrium. She underwent successful embolization using three detachable Cook coils. PMID:15530285

Jameel, Al-Ata; Arfi, Amin Muhammed; Ayman, M S; Nasser, Mahdi; Amjad, Kouatli; Iskandar, Al-Githmi

2004-11-01

384

Classical-Quantum correspondence in isomerization dynamics: quantum eigenstates and classical Arnol'd web  

NASA Astrophysics Data System (ADS)

Recently, there has been a renaissance of sorts in chemical dynamics with researchers critically examining the validity of the two pillars of reaction rate theory - transition state theory and the Rice-Ramsperger-Kassel-Marcus (RRKM) theory. Since both theories have classical dynamics at their foundation, advances in our understanding of nonlinear dynamics and continuing efforts to characterize the phase space structure of systems with three or more degrees of freedom are beginning to yield crucial mechanistic insights into the dynamics. This talk focuses on a mechanistic understanding of the deviations from RRKM theory for a model isomerization problem with three degrees of freedom. Several studies have established that such systems are prime candidates for observing non-RRKM behaviorootnotetextD. M. Leitner, Int. J. Quant. Chem. 75, 523 (1999).. The model is inspired, and generalized, from a much earlier study by De Leon and BerneootnotetextN. De Leon and B. J. Berne, J. Chem. Phys. 75, 3495 (1981).. We try to answer two of the questions posed in this early work by studying the intramolecular vibrational energy flow in the system from both classical and quantum viewpoints. Using a wavelet-based local frequency analysis it is possible to construct a useful representation of the classical phase space (Arnol'd web) highlighting the important dynamical structures. Insights into the dynamics originate from the various nonlinear resonances and phase space traps which potentially result in quantum eigenstates of varying degree of localizationootnotetextD. M. Leitner and M. Gruebele, Mol. Phys. 106, 433 (2008)..

Keshavamurthy, S.

2009-03-01

385

From the 'Village of a Thousand Souls' to 'Race Crossing in Jamaica': Arnold Gesell, eugenics and child development.  

PubMed

Perhaps best known for providing age-related norms in early development, norms that are still used as a basis for measures of developmental maturity, Arnold Gesell was a key figure in developmental psychology from the 1920s through the 1950s. After examining Gesell's reputation and status in the field, we explore Gesell's changing relationship to eugenics, both in terms of Gesell's often contradictory attitudes about the role of hereditary and environmental influences in development, and in terms of the broader relationship between the eugenics movement and science. PMID:20623743

Weizmann, Fredric

2010-01-01

386

Technical evaluation of the adequacy of station electric-distribution-system voltages for the Duane Arnold Energy Center  

SciTech Connect

The technical evaluation of the adequacy of the station electric distribution system voltages for the Duane Arnold Energy Center is documented. The evaluation is to determine if the onsite distribution system in conjunction with the offsite power sources has sufficient capacity to automatically start and operate all Class 1E loads within the equipment voltage ratings under certain conditions established by the Nuclear Regulatory Commission. The analyses submitted demonstrate that the offsite power sources in conjunction with the onsite distribution system has sufficient capacity and capability to supply adequate voltages to the Class 1E equipment under worst case conditions.

Selan, J.C.

1981-10-12

387

[Thoracic surgery in neonates and infants with congenital malformations].  

PubMed

Congenital malformations of the lung and diaphragm are a challenge in paediatric surgery. Depending on the malformation they show a broad spectrum of symptoms and a varying age at manifestation. Thus there are many diagnostic and therapeutic options, which require a good knowledge of the pathology. The treatment of these complex cases should lead to early referral to paediatric centres containing an interdisciplinary team with neonatologists, paediatric pulmonologists and cardiologists, ENT surgeons, anaesthesiologists, radiologists and paediatric surgeons. Some malformations are diagnosed prenatally and need intrauterine interventions. Decisive is the early diagnosis and treatment of these malformations. Nowadays the surgical therapy of neonates and infants with malformations of the lung and diaphragm is enriched by a number of endoscopic and endoluminal techniques, which are discussed critically in this article. PMID:20020383

Metzger, R; Till, H

2009-12-17

388

Comment: valproate dose effects differ across congenital malformations.  

PubMed

Fetal valproate exposure has been associated with the highest risk of congenital malformations among antiepileptic drugs.(1) Valproate's effect is dose-dependent(1) and has been associated with multiple specific malformations.(2,3) Vadja et al.(4) examined data from the Australian Pregnancy Registry (1999-2012 data), which included 1,705 pregnancies with 436 valproate exposures.(4) They found that the use and dosages of valproate have fallen over the last 5 years. The rates of spina bifida and hypospadius in those exposed dropped with reducing dosages of valproate, but the rates of other malformations did not. Mean dosages for malformations were higher for spina bifida (2,000 mg/d) and hypospadius (2,417 mg/d) than all other malformations (1,083 mg/d). PMID:23911754

Meador, Kimford J

2013-08-02

389

The study of malformations "by the company they keep".  

PubMed Central

Our study of individuals with thalidomide embryopathy reaffirms many of the principles of clinical teratology concerning sensitivity of developing structures and specificity of action. It also highlights important features of ocular teratology that differ from those of other organ systems. Because malformations of the eye and associated structures do not significantly threaten the survival of the embryo, fetus, or neonate, any variation in prevalence in older individuals is not due to the nature of the ocular malformation but may be significantly modified by "the company they keep," the systemic malformations occurring at the same time as the original insult, whether it be an environmental, a genetic, or a local disturbance. Additionally, the presence of clusters of malformations caused by a teratogen at a specific time supports the concept of a common pathogenic mechanism and provides suggested avenues for further research into the cause of these malformations.

Miller, M T; Stromland, K

1992-01-01

390

A classification for congenital limb malformations.  

PubMed

The classification for congenital limb malformations adopted by the American Society for Surgery of the Hand, the International Federation of Societies for Surgery of the Hand, and the International Society of Prosthetics and Orthotics is presented. This method groups similar patterns of deficiencies according to the parts that have been primarily affected by certain embryological failures, whether the insult involves a total part (skeletal and soft tissue) or only the dermomyofascial structures. The main categories of this classification are (I) failure of formation of parts. (II) failure of differentiation (separation) of parts, (III) duplication, (IV) overgrowth, (V) undergrowth, (VI) congenital constriction band syndrome, and (VII) generalized skeletal abnormalities. The rationale and method of use of the classification are discussed. This method has been tested and used in a variety of centers and has been found to be properly conceived and practical. PMID:1021591

Swanson, A B

1976-07-01

391

Treatment of arteriovenous malformations of the brain.  

PubMed

The treatment of ruptured and unruptured brain arteriovenous malformations (AVMs) is driven by the need to prevent incident or recurrent intracranial hemorrhages. Improving feasibility of the rapidly developing endovascular, neurosurgical, and radiotherapeutic procedures leads to invasive treatment of an increasing number of neurologically intact patients with accidentally diagnosed AVMs. Recent data confirm that the natural history risk of unruptured AVMs is significantly lower than the risk of those presenting with rupture, and the treatment risk of invasive management of unruptured AVMs seems higher than their natural history risk. The treatment decision algorithm for these patients remains unsettled, as no randomized clinical trial data exist on the benefit of invasive AVM treatment for patients with bled or with unbled AVMs. The recently launched study A Randomized Trial of Unruptured Brain AVMs (ARUBA) will be the first trial randomizing patients with nonhemorrhaged AVMs to invasive versus conservative management. PMID:17217851

Hartmann, Andreas; Mast, Henning; Choi, Jae H; Stapf, Christian; Mohr, Jay P

2007-01-01

392

Surgical treatment of intracranial arteriovenous malformation.  

PubMed

The surgical indication and techniques of intracranial arteriovenous malformations are discussed, on the basis of 203 experiences with AVMs. A special key point in our surgical techniques is described as follows. The feeding arteries of the AVM--or the main artery at the base of the brain, which is the original source of the feeding artery--are identified and prepared, and then temporary clips are placed on these arteries to produce vascular occlusion prior to the dissection of the AVM itself. During these procedures, 20% of mannitol should be administered to prolong the permissible time of cerebral artery occlusion. The administration method of mannitol is described in detail including dose and time schedule. The new prolongation method of temporary occlusion time, for which mannitol with fluorochemicals permits longer occlusion time, is also introduced. PMID:6129587

Suzuki, J; Onuma, T; Kayama, T

1982-01-01

393

Familial Dandy-Walker malformation and leukodystrophy.  

PubMed

We report the first familial cases with two different types of posterior fossa cystic malformation and a leukodystrophic-like aspect on cerebral magnetic resonance imaging (MRI). The girl and her brother had severe encephalopathy, marked hypotonia, absent deep tendon reflexes, macrocrania, gigantism, and dysmorphic face and extremities. The girl had generalized seizures. The boy had unilateral cataract and bilateral optic atrophy. The parents were first cousins, suggesting autosomal recessive transmission. MRI showed Dandy-Walker variant in the girl, with cerebellar vermis hypoplasia and expansion of the cisterna magna, which communicated with the fourth ventricle. Her brother had mega cisterna magna communicating with the fourth ventricle and a normal cerebellum. The 2 children had abnormally high signal in the supratentorial white matter. Visual and auditory evoked potentials revealed prolonged latencies. Motor and sensory conduction velocities were normal. Muscle and nerve biopsies were normal. Metabolic exploration demonstrated no abnormality. PMID:9258968

Humbertclaude, V T; Coubes, P A; Leboucq, N; Echenne, B B

1997-05-01

394

Small Rho-GTPases and cortical malformations  

PubMed Central

Rho-GTPases have been found to be crucial for cytoskeleton remodelling and cell polarity, as well as key players in directed cell migration in various tissues and organs, therefore becoming good candidates for involvement in neuronal migration disorders. We recently found that genetic deletion of the small GTPase RhoA in the developing mouse cerebral cortex results in three distinct cortical malformations: a defect in the proliferation of progenitor cells during development that leads to a bigger cerebral cortex in the adult mouse, a change in the morphology of radial glial cells that results in the formation of a subcortical band heterotopia (SBH, also called Double Cortex) and an increase in the speed of migrating newborn neurons. The latter, together with the aberrant radial glial shape, is likely to be the cause of cobblestone lissencephaly, where neurons protrude beyond layer I at the pial surface of the brain.

2013-01-01

395

Malformations and the Manx Syndrome in Cats  

PubMed Central

Breeding experiments were conducted on cats with congenital taillessness, to test the dissemination pattern of taillessness in their offspring. Clinical evaluation, radiographic analysis of the vertebral column and histological studies of the digestive tract and central nervous tissue were conducted to determine the association of malformations of these systems in cats born with different degrees of taillessness noted in the rumpy and stumpy cats. The mode of transmission of the tailless (Manx) condition assumed to be through an autosomal dominant factor (M) was confirmed by this investigation. It is hypothesized that the problems associated with the tailless condition such as spina bifida, urinary and faecal incontinence and locomotor disturbances of the pelvic limbs may all be related to a disturbance affecting the development of the central nervous system in the early embryonic life. ImagesFigure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10.

DeForest, M. E.; Basrur, P. K.

1979-01-01

396

Complications of Embolization for Cerebral Arteriovenous Malformations  

PubMed Central

Summary Embolization is recognized as an important adjunct in the treatment of cerebral arteriovenous malformations (AVMs). We reviewed our results of embolizations for AVMs and discussed procedure-related complications. Eleven complications were recorded in 68 consecutive patients (16%). Of these, four were technical problems including a glued catheter, inability to withdraw the catheter, vessel perforation by the microcatheter, and coil migration. Other complications included three cases of ischemic symptoms due to retrograde thrombosis, two cases of asymptomatic cerebral infarction, one case of asymptomatic small haemorrhage due to venous occlusion, and one case of post-embolization haemorrhage of unknown etiology. Our morbidity rate was 7%, mortality rate was 0%, and asymptomatic complication torospectively. Further improvements to endovascular techniques and devices are required.

Sugiu, K.; Tokunaga, K.; Sasahara, W.; Watanabe, K.; Nishida, A.; Ono, S.; Nishio, S.; Date, I.; Rufenacht, D. A.

2004-01-01

397

Gastrointestinal malformations in Gorgan, North of Iran: epidemiology and associated malformations.  

PubMed

The aim of this prospective study was to evaluate the prevalence and pattern of gastrointestinal malformations (GIM) among Iranian newborns in Gorgan, North of Iran. From 1998 through 2003, 37,951 live births in Dezyani hospital in Gorgan, North of Iran, were screened for gastrointestinal malformations. Clinical and demographic factors of diagnosed cases were recorded in a pre-designed questionnaire for analysis; sex, ethnicity, type of GIM and associated anomalies. The overall prevalence rate of gastrointestinal malformations was 10 per 10,000 births. The imperforate anus (5 per 10,000) was the commonest birth defect in gastrointestinal tract. The prevalence rate of GIM was 8.2 per 10,000 in males and 10.7 per 10,000 in females. According to the parental ethnicity, the prevalence rates of GIM were 6.7, 15.8 and 17.6 per 10,000 in Fars, Turkman, and Sistani, respectively. There were eight cases (21%) with associated anomalies. The prevalence rate of GIM in North of Iran is not similar to the previous studies in Iran and Middle East and ethnic background may be a causative factor in the rate of GIM in this area. PMID:17043876

Golalipour, Mohammad Jafar; Mobasheri, Elham; Hoseinpour, Kaniz-Reza; Keshtkar, Abbas Ali

2006-10-17

398

Seizure risk from cavernous or arteriovenous malformations  

PubMed Central

Objectives: To determine the risk of epileptic seizures due to a brain arteriovenous malformation (AVM) or cavernous malformation (CM). Methods: In a prospective population-based study of new diagnoses of AVMs (n = 229) or CMs (n = 139) in adults in Scotland in 1999–2003, we used annual medical records surveillance, general practitioner follow-up, and patient questionnaires to quantify the risk of seizures between clinical presentation and AVM/CM treatment, last follow-up, or death. Results: The 5-year risk of first-ever seizure after presentation was higher for AVMs presenting with intracranial hemorrhage or focal neurologic deficit (ICH/FND: n = 119; 23%, 95% confidence interval [CI] 9%–37%) than for incidental AVMs (n = 40; 8%, 95% CI 0%–20%), CMs presenting with ICH/FND (n = 38; 6%, 95% CI 0%–14%), or incidental CMs (n = 57; 4%, 95% CI 0%–10%). For adults who had never experienced ICH/FND, the 5-year risk of epilepsy after first-ever seizure was higher for CMs (n = 23; 94%, 95% CI 84%–100%) than AVMs (n = 37; 58%, 95% CI 40%–76%; p = 0.02). Among adults who never experienced ICH/FND and presented with or developed epilepsy, there was no difference in the proportions achieving 2-year seizure freedom over 5 years between AVMs (n = 43; 45%, 95% CI 20%–70%) and CMs (n = 35; 47%, 95% CI 27%–67%). Conclusions: AVM-related ICH confers a significantly higher risk of a first-ever seizure compared to CMs or incidental AVMs. Adults with a CM have a high risk of epilepsy after a first-ever seizure but achieve seizure freedom as frequently as those with epilepsy due to an AVM.

Josephson, C.B.; Leach, J.-P.; Duncan, R.; Roberts, R.C.; Counsell, C.E.

2011-01-01

399

The use of mobile Raman spectroscopy to compare three full-page miniatures from the Breviary of Arnold of Egmond.  

PubMed

The Breviary of Arnold of Egmond is one of the most wealthily illuminated fifteenth century manuscripts in the Northern Netherlands. The manuscript originally contained a number of full-page miniatures, which were all removed at an unknown date before 1902. The three remaining miniatures studied here, are today part of different collections, but they were brought together for an exhibition. Although several historical and art historical details of this breviary have extensively been studied, no examination of the materials used was undertaken before. Analytical techniques, such as mobile Raman spectroscopy, can be used to characterise and identify these materials in a non-invasive way. This paper presents the results of the in situ Raman analysis of three full-page miniatures of the Breviary of Arnold of Egmond. During this study, different pigments could be identified, such as lead white (2PbCO(3)·Pb(OH)(2)), lead-tin yellow type I (Pb(2)SnO(4)), ultramarine (Na(8-10)Al(6)Si(6)O(24)S(2-4)), massicot (PbO), vermilion (HgS) and red lead (Pb(3)O(4)). Next to identification of the pigments, visual analysis was used to detect differences and similarities between the stylistic elements of the three analysed folios. PMID:21943711

Deneckere, A; Leeflang, M; Bloem, M; Chavannes-Mazel, C A; Vekemans, B; Vincze, L; Vandenabeele, P; Moens, L

2011-09-08

400

Living donor liver transplantation for Budd-Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures.  

PubMed

Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient's suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5 years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation. PMID:23188387

Fukuda, Akinari; Ogura, Yasuhiro; Kanazawa, Hiroyuki; Mori, Akira; Kawaguchi, Michiya; Takada, Yasutsugu; Uemoto, Shinji

2012-11-28

401

[Budd-Chiari syndrome and splanchnic vein thrombosis: masked myeloproliferative neoplasms and JAK2V617F].  

PubMed

The Budd-Chiari Syndrome (BCS) and the splanchnic vein thrombosis are characterized by hepatic venous outflow obstruction, generally due to venous thrombosis. These rare diseases are usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Since the diagnosis of myeloproliferative neoplasms (MPNs) is often difficult in patients with BCS and splanchnic vein thrombosis because of spleen enlargement, secondary pancytopenia and bleeding disorders, recent observations have included in the diagnostic work-up the analysis of the JAK2 mutation. The revision of several recent reports clarify the importance of the JAK2V617F detection in the diagnostic work-up of the BCS and splanchnic vein thrombosis, allowing the demonstration of masked MPNs among these cases that may benefit, in the near future, of target molecular therapies directed toward the JAK2 mutation. PMID:20499034

Fama, A; Rago, A; Gioiosa, F; Marzano, C; Latagliata, R; Mammě, C; Laganŕ, C; D'Elia, G M; Bizzoni, L; Trasarti, S; Ferretti, A; Breccia, M; Riggio, O; Tafuri, A

2010-01-01

402

Contribution of Rare Copy Number Variants to Isolated Human Malformations  

PubMed Central

Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n?=?7) or very uncommon (n?=?15, <1/2,000) in the control population] in 20/95 fetuses with congenital malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases.

Serra-Juhe, Clara; Rodriguez-Santiago, Benjamin; Cusco, Ivon; Vendrell, Teresa; Camats, Nuria; Toran, Nuria; Perez-Jurado, Luis A.

2012-01-01

403

Surgical treatment of arteriovenous malformations of the posterior fossa.  

PubMed

Nine cases of arteriovenous malformations of the posterior fossa were operated upon, using microsurgical techniques. The excision was radical in eight patients. Seven of them, as well as the sole patient who had a partial removal of the malformation, returned to their previous occupations without neurological signs. In one case the preoperative deficit was unchanged. Some lesions that appear to penetrate the brain stem actually lie on its surface and can be dissected through an extrapial plane of cleavage. Extension of the malformation into the cerebellar peduncles requires dissection of the tangle and opening of the IVth ventricle. PMID:515935

Viale, G L; Pau, A; Viale, E S

1979-11-01

404

Cerebral arteriovenous malformation in pregnancy: Presentation and neurologic, obstetric, and ethical significance  

Microsoft Academic Search

Cerebral arteriovenous malformations infrequently complicate pregnancy. We sought to determine the neurologic, obstetric, and ethical significance of such malformations. We present the clinical course of 2 pregnant women with arteriovenous malformations who experienced cerebral hemorrhage and a loss of capacity for decision making. We also review the neurologic and obstetric significance of arteriovenous malformations in pregnancy. Various treatment options with

James J. Finnerty; Christian A. Chisholm; Helen Chapple; JoAnn V. Pinkerton

1999-01-01

405

Well-Construction, Water-Level, Geophysical, and Water-Quality Data for Ground-Water Monitoring Wells for Arnold Air Force Base, Tennessee.  

National Technical Information Service (NTIS)

Sixty-five wells were installed at 39 sites in the Arnold Air Force Base area in Coffee and Franklin Counties, Tennessee. The wells were installed to provide information on subsurface lithology, aquifer characteristics, ground- water levels, and ground-wa...

C. J. Haugh E. N. Mahoney J. A. Robinson

1992-01-01

406

Well-Construction, Water-Level, and Water-Quality Data for Ground-Water Monitoring Wells for the J4 Hydrogeologic Study, Arnold Air Force Base, Tennessee.  

National Technical Information Service (NTIS)

Between December 1993 and March 1994, 27 wells were installed at 12 sites near the J4 test cell at Arnold Engineering Development Center (AEDC) in Coffee County, Tennessee. The wells ranged from 28 to 289 feet deep and were installed to provide informatio...

C. J. Haugh

1996-01-01

407

A new species of the lycopsid Gilboaphyton Arnold from the Devonian of Venezuela and New York State, with a revision of the closely related genus Archaeosigillaria Kidston  

Microsoft Academic Search

A new species of the small, probably herbaceous lycopsid Gilboaphyton Arnold is based on new material from the Devonian of western Venezuela. The excellently preserved compressions allow the following characteristics to be established: (1) the presence below the leaf bases of large oval areas of parenchymatous tissue within a toughened outer stem tissue (outer cortex) which could be infilled with

Christopher M. Berry; Dianne Edwards

1997-01-01

408

Store Separation Testing Techniques at the Arnold Engineering Development Center. Volume III. Description and Validation of Captive Trajectory Store Separation Testing in the Von Karman Facility.  

National Technical Information Service (NTIS)

Tests were conducted on a four-finned, ogive-cylinder store model which was mounted on the von Karman Gas Dynamics Facility (VKF) Captive Trajectory System (CTS) in the Arnold Engineering Development Center (AEDC) Supersonic Wind Tunnel A. The purpose of ...

J. P. Billingsley J. T. Best R. H. Burt

1979-01-01

409

Constructing Masculinities under Thomas Arnold of Rugby (1828-1842): Gender, Educational Policy and School Life in an Early-Victorian Public School  

ERIC Educational Resources Information Center

Thomas Arnold has been a controversial figure for historians of the English public schools. He has been depicted either as the great reformer of these famous institutions or as an ordinary head master who did not do better than his contemporary colleagues. This article seeks to continue the debate about the assessment of his head master-ship by…

Neddam, Fabrice

2004-01-01

410

[Chiari's network: persistence of the embryonic remnants of the valve of the sinus venosus in the right atrium. Echocardiographic and clinical analysis in a group of subjects].  

PubMed

As echocardiography is being used more often, its value and accuracy are becoming more fully appreciated, especially for identifying normal anatomic variants and their possible erroneous interpretation as pathologic states. We report the echocardiographic and clinical findings observed in sixteen subjects examined at our Cardiological Service in the period from January 1987 to April 1988. Mean age of these subjects is 40 +/- 27.04. Of these subjects, six are affected by other cardiac pathologies and ten are unaffected (Group A). First, we describe in all the patients M-mode and two dimensional patterns of persistence of the right venous sinus valve known as the Chiari network. This structure can present as a highly mobile, highly reflective echo target, which can be seen especially by means of the bi-dimensional technique. The Chiari network could be seen with all four standard approaches. The two most diagnostic views are, in our experience, the short axis parasternal view (62.5%), and the subcostal view (87.5%). In a great number of subjects (75%) the Chiari network could be seen in at least two approaches. Second, in Group A we make a clinical examination. Nine subjects in this group show the presence of a cardiac systolic murmur with vibratory characters from grade 1/6 to grade 3/6. 50% of the same patients presented supraventricular arrhythmias (particularly, two presented reciprocating paroxysmal supraventricular tachycardia and one paroxysmal atrial fibrillation). The significance of these findings is not clear yet. We, at least, emphasise that the Chiari network could be confused with other curvilinear highly mobile, echo targets such as right-heart vegetations, flail tricuspid leaflets, a small right-heart thrombus or even a pedunculated right heart tumor (especially right atrial myxoma). On the contrary, this structure might be considered a "normal anatomic variant". PMID:2608180

Chesi, G; Farinelli, F; Di Donato, C; Marani, L; Reverzani, A

411

EVALUATION OF PORTAL VEIN THROMBOSIS IN LIVER GRAFT TEN YEARS AFTER LIVER TRANSPLANTATION DUE TO BUDD-CHIARI SYNDROME USING DOPPLER ULTRASOUND  

PubMed Central

Budd-Chiari syndrome is a rare but life-threatening disorder characterized by obstruction of the hepatic venous outflow. Treatment depends on underlying cause, extent of the obstruction and functional capacity of the liver. When all other therapy options are unsuccessful, liver transplant should be considered. Portal vein thrombosis (PVT) is a frequent event in patients with cirrhosis which can be treated with anticoagulants, but there are limited data regarding safety and efficacy of this approach.

Husic-Selimovic, Azra; Gornjakovic, Srdjan; Schuchmann, Marcus; Vukobrat-Bijedic, Zora

2012-01-01

412

Evaluation of portal vein thrombosis in liver graft ten years after liver transplantation due to budd-Chiari syndrome using Doppler ultrasound.  

PubMed

Budd-Chiari syndrome is a rare but life-threatening disorder characterized by obstruction of the hepatic venous outflow. Treatment depends on underlying cause, extent of the obstruction and functional capacity of the liver. When all other therapy options are unsuccessful, liver transplant should be considered. Portal vein thrombosis (PVT) is a frequent event in patients with cirrhosis which can be treated with anticoagulants, but there are limited data regarding safety and efficacy of this approach. PMID:23322979

Husic-Selimovic, Azra; Gornjakovic, Srdjan; Schuchmann, Marcus; Vukobrat-Bijedic, Zora

2012-09-01

413

Long-Term Follow-Up After Successful Transjugular Intrahepatic Portosystemic Shunt Placement in a Pediatric Patient with Budd-Chiari Syndrome  

SciTech Connect

Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.

Carnevale, Francisco Cesar, E-mail: fcarnevale@uol.com.br; Szejnfeld, Denis, E-mail: denis@cura.com.br; Moreira, Airton Mota [University of Sao Paulo Medical School, Service of Interventional Radiology, Instituto de Radiologia (Brazil); Gibelli, Nelson [University of Sao Paulo Medical School, Service of Pediatric Surgery, Instituto da Crianca Prof. Pedro de Alcantara (Brazil); Gregorio, Miguel Angel De [University of Zaragoza, Interventional Radiology Unit (Spain); Tannuri, Uenis [University of Sao Paulo Medical School, Service of Pediatric Surgery, Instituto da Crianca Prof. Pedro de Alcantara (Brazil); Cerri, Giovanni Guido [University of Sao Paulo Medical School, Service of Interventional Radiology, Instituto de Radiologia (Brazil)

2008-11-15

414

Macrocephaly-capillary malformation presenting with fetal arrhythmia.  

PubMed

Macrocephaly-capillary malformation (OMIM 602501) is a rare overgrowth and asymmetry syndrome. Cardiac arrhythmias were reported to occur in few patients. We present a case in which fetal arrhythmia was the presenting symptom of the syndrome. PMID:22329570

Kuint, Jacob; Globus, Omer; Ben Simon, Guy J; Greenberger, Shoshana

2012-02-13

415

MALLEABLE INSPECTION STATION WHERE WORKERS ELIMINATE MALFORMED CASTINGS AND SEPARATED ...  

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

MALLEABLE INSPECTION STATION WHERE WORKERS ELIMINATE MALFORMED CASTINGS AND SEPARATED GOOD CASTINGS PRIOR TO ANNEALING. - Stockham Pipe & Fittings Company, Malleable Annealing Building, 4000 Tenth Avenue North, Birmingham, Jefferson County, AL

416

Malformations among the X-linked intellectual disability syndromes.  

PubMed

Malformations are significant contributions to childhood mortality and disability. Their co-occurrence with intellectual disability may compound the health burden, requiring additional evaluation and management measures. Overall, malformations of greater or lesser severity occur in at least some cases of almost half of the 153 XLID syndromes. Genitourinary abnormalities are most common, but tend to contribute little or no health burden and occur in only a minority of cases of a given XLID syndrome. Some malformations (e.g., lissencephaly, hydranencephaly, long bone deficiency, renal agenesis/dysplasia) are not amenable to medical or surgical intervention; others (e.g., hydrocephaly, facial clefting, cardiac malformations, hypospadias) may be substantially corrected. © 2013 Wiley Periodicals, Inc. PMID:24166814

Stevenson, Roger E; Schwartz, Charles E; Rogers, R Curtis

2013-09-24

417

Histopathological study of congenital aortic valve malformations in 32 children  

Microsoft Academic Search

The histopathological characteristics of congenital aortic valve malformations in children were investigated. All the native\\u000a surgically excised aortic valves from 32 pediatric patients suffering from symptomatic aortic valve dysfunction due to congenital\\u000a aortic valve malformations between January 2003 and December 2005 were studied macroscopically and microscopically. The patients’\\u000a medical records were reviewed and the clinical information was extracted. The diagnosis

Ping Huang; Hongwei Wang; Yanping Li; Peixuan Cheng; Qingjun Liu; Zhenlu Zhang; Jianying Liu

2007-01-01

418

Post-mortem examination of prenatally diagnosed fatal renal malformation  

Microsoft Academic Search

Objective:Renal malformations can be associated with genetic syndromes and chromosomal disorders. Fetal autopsy including histopathological examination of kidney is important to arrive at definite diagnosis. The objective was to assess importance of fetal autopsy and histopathology.Study Design:Retrospective analysis of cases with fetal renal malformations was done. All fetuses terminated were examined with whole body radiograph, external and internal examination and

N Kumari; M Pradhan; V H Shankar; N Krishnani; S R Phadke

2008-01-01

419

Contemporary Themes: Congenital Malformations and the Problem of their Control*  

PubMed Central

The possibility of preventing or reducing the prevalence of human congenital malformations is discussed in the light of current knowledge about their aetiology. The original data presented are derived from an epidemiological study of all the infants (92,982) born in the three years 1964-6 to women resident in a defined area of South Wales and all the congenital malformations (3,242) discovered in those infants by the second anniversary of their birth.

Lowe, C. R.

1972-01-01

420

Macrocephaly-Cutis Marmorata Telangiectatica Congenita (Macrocephaly-Capillary Malformation)  

Microsoft Academic Search

This recently recognised entity (OMIM # 602501) (OMIM 2006) is characterised by the association of macrocephaly (megalencephaly),\\u000a capillary malformation of the cutis marmorata telangectatica congenita type, cavernous haemangioma, asymmetric growth pattern,\\u000a central nervous system malformations, and neurological abnormalities (Clayton-Smith et al. 1997, Gerritsen et al. 2000, Moore et al. 1997, Lapunzina et al. 2004). Despite extensive investigation of many of

Pablo Lapunzina; Jill Clayton-Smith

421

Dandy-Walker malformation: analysis of 38 cases  

Microsoft Academic Search

Thirty-eight cases of Dandy-Walker malformation (DWM) are presented. A female predominance of 3:1 was found. Thirty-two cases (84%) were diagnosed within the 1st year of life. Of these, 17 cases (44.7%) were diagnosed at birth. Ten (26%) were delivered by cesarean section. Thirteen infants (34%) had a birth weight below 3000 g. Several associated malformations were observed, the most frequent

I. Pascual-Castroviejo; A. Velez; S. I. Pascual-Pascual; M. C. Roche; F. Villarejo

1991-01-01

422

MRI characteristics and classification of peripheral vascular malformations and tumors  

Microsoft Academic Search

Vascular malformations and tumors comprise a broad spectrum of lesions that can cause significant morbidity and even mortality\\u000a in children and adults. Classification of vascular malformations into high flow and low flow has significant impact on management\\u000a since the main treatment of the former is transarterial embolization and the later percutaneous sclerotherapy. Magnetic resonance\\u000a imaging (MRI) is a noninvasive effective

Hicham Moukaddam; Jeffrey Pollak; Andrew H. Haims

2009-01-01

423

Vein of Galen malformations: epidemiology, clinical presentations, management.  

PubMed

The vein of Galen aneurysmal malformation is a congenital vascular malformation that comprises 30% of the pediatric vascular and 1% of all pediatric congenital anomalies. Treatment is dependent on the timing of presentation and clinical manifestations. With the development of endovascular techniques, treatment paradigms have changed and clinical outcomes have significantly improved. In this article, the developmental embryology, clinical features and pathophysiology, diagnostic workup, and management strategies are reviewed. PMID:22107867

Recinos, Pablo F; Rahmathulla, Gazanfar; Pearl, Monica; Recinos, Violette Renard; Jallo, George I; Gailloud, Philippe; Ahn, Edward S

2012-01-01

424

Multiple medullary venous malformations decreasing cerebral blood flow: Case report  

SciTech Connect

A rare case of multiple medullary venous malformations in the right cerebral hemisphere is reported. The literature review yielded only one case of multiple medullary venous malformations. Computed tomography scan showed multiple calcified lesions with linear contrast enhancement representing abnormal dilated vessels and mild atrophic change of the right cerebral hemisphere. Single-photon emission computed tomography using N-isopropyl-p-({sup 123}I) iodoamphetamine demonstrated decreased cerebral blood flow in the right cerebral hemisphere.

Tomura, N.; Inugami, A.; Uemura, K.; Hadeishi, H.; Yasui, N. (Research Institute for Brain and Blood Vessels, Akita (Japan))

1991-02-01

425

Occupational exposure to glycol ethers and human congenital malformations  

Microsoft Academic Search

ObjectivesThis commentary reviews toxicological information and critically evaluates epidemiological information on the relationship between glycol ethers and congenital malformations.MethodsThe authors identified and assessed toxicological and epidemiological research on glycol ethers used in occupational settings and congenital malformations. Sensitivity analyses evaluated the possible role of methodological problems in explaining the findings of the epidemiological studies.ResultsExposure to certain glycol ethers, including ethylene

George Maldonado; Elizabeth Delzell; Rochelle W. Tyl; Lowell E. Sever

2003-01-01

426

Capillary-venous malformation in the lower limb.  

PubMed

Regional capillary malformation of a lower extremity is associated with the overgrowth of bone or soft tissue in several disorders, most commonly Klippel-Trenaunay syndrome and Parkes Weber syndrome. We have observed a subset of patients with a capillary malformation of the leg, minor growth disturbance, and prominent veins. The objective of the current study is to describe a series of patients with regional capillary malformation of the lower extremity in association with phlebectasia. This is a retrospective series of 17 patients diagnosed with capillary-venous malformation of the lower extremity. We excluded patients with clinical or radiographic evidence of lymphatic or arteriovenous malformation. Age, presentation, associated features, radiographic findings, and management were documented. In most patients the capillary malformation covered a large area without sharply demarcated borders. Four patients had one or more discrete, well-defined capillary stains involving less than 5% of the total surface area of the affected lower limb. Prominent veins were most common in the popliteal fossa and on the knee and dorsal foot. Approximately two-thirds of patients had a leg length discrepancy, with the affected leg being longer (n = 6) or shorter (n = 4); in many the affected leg was also slightly larger (n = 8) or smaller (n = 4) in girth. Radiographic imaging showed dilatation of superficial (n = 16), muscular (n = 9), and deep veins (n = 6). We characterize a subset of patients with regional capillary-venous malformation of the lower extremity with prominent veins and minor hypotrophy/hypertrophy that differs from Klippel-Trenaunay syndrome (capillary-lymphatic-venous malformation) but belongs at the minor end of the spectrum of vascular disorders with overgrowth. PMID:23829172

Uihlein, Lily C; Liang, Marilyn G; Fishman, Steven J; Alomari, Ahmad I; Mulliken, John B

2013-07-07

427

Ethical Aspects of Prenatal Diagnosis of Fetal Malformations  

PubMed Central

Fetal malformations complicate approximately 3% of all pregnancies. Many of these are minor or can be corrected after birth, but there are certain malformations that are lethal and others that are severe and others, that, even if corrected lead to permanent disability. Advances in prenatal diagnosis made possible the diagnosis of many fetal malformations. This led to the concept of the fetus a patient, independent of the pregnant woman, even though the moral status of the fetus is in dispute. Many of the lethal malformations are untreatable. However, for some, innovative in utero treatments, both medical and surgical, became possible. These interventions should be evaluated for the relative benefit and risk for both the fetus and the mother, because any such treatment has to involve the integrity of her body. This raises the ethical question of beneficence (to the fetus) versus the autonomy of the pregnant woman. The process of resolving this issue will be discussed, especially how to obtain a truly informed consent. For the lethal malformations or for those severe or multiple malformations whose treatment is theoretically possible but the results of such treatment are unpredictable or may lead to life long disabilities and serious burdens for the infant or child and the family, prenatal counseling should include “prenatal advance directive” and a plan for palliative care, the components of which will be described.

Fadel, Hossam E.

2012-01-01

428

Giant Cystic Cerebral Cavernous Malformation with Multiple Calcification - Case Report  

PubMed Central

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2×4.6×6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review.

Kim, Il-Chun; Rhee, Jong-Joo; Lee, Jong-Won; Hur, Jin-Woo; Lee, Hyun-Koo

2013-01-01

429

Giant cystic cerebral cavernous malformation with multiple calcification - case report.  

PubMed

Cerebral cavernous malformation with giant cysts is rare and literature descriptions of its clinical features are few. In this case study, the authors describe the clinical symptoms, radiological findings, and pathological diagnosis of cerebral cavernous malformations with giant cysts, reviewing the relevant literature to clearly differentiate this from other disease entities. The authors present a case of a 19-year-old male with a giant cystic cavernous malformation, who was referred to the division of neurosurgery due to right sided motor weakness (grade II/II). Imaging revealed a large homogenous cystic mass, 7.2×4.6×6 cm in size, in the left fronto-parietal lobe and basal ganglia. The mass had an intra-cystic lesion, abutting the basal portion of the mass. The initial diagnosis considered this mass a glioma or infection. A left frontal craniotomy was performed, followed by a transcortical approach to resect the mass. Total removal was accomplished without post-operative complications. An open biopsy and a histopathological exam diagnosed the mass as a giant cystic cavernous malformation. Imaging appearances of giant cavernous malformations may vary. The clinical features, radiological features, and management of giant cavernous malformations are described based on pertinent literature review. PMID:24167810

Kim, Il-Chun; Kwon, Ki-Young; Rhee, Jong-Joo; Lee, Jong-Won; Hur, Jin-Woo; Lee, Hyun-Koo

2013-09-30

430

Limb malformations and abnormal sex hormone concentrations in frogs.  

PubMed Central

Declines in amphibian populations, and amphibians with gross malformations, have prompted concern regarding the biological status of many anuran species. A survey of bullfrogs, Rana catesbeiana, and green frogs, Rana clamitans, conducted in central and southern New Hampshire showed malformed frogs at 81% of the sites sampled (13 of 16 sites). Brain gonadotropin-releasing hormone (GnRH) and the synthesis of androgens and estradiol, hormones essential to reproductive processes, were measured from limb-malformed and normal (no limb malformation) frogs. Normal frogs had significantly higher concentrations (nearly 3-fold) of in vitro produced androgens and of brain GnRH than malformed frogs. Because most malformations are thought to occur during development, we propose that environmental factors or endocrine-disrupting chemicals that may cause developmental abnormalities also act during early development to ultimately cause abnormally reduced GnRH and androgen production in adult frogs. The consequences of reduced GnRH and androgens on anuran reproductive behavior and population dynamics are unknown but certainly may be profound and warrant further research.

Sower, S A; Reed, K L; Babbitt, K J

2000-01-01

431

Syndromes associated with vascular tumors and malformations: a pictorial review.  

PubMed

Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system has been strongly recommended in recent years because of the need for separate therapeutic measures for patients with vascular tumors and malformations. In the ISSVA classification system, vascular tumors, which are neoplastic, are distinguished from vascular malformations, which are caused by vascular structural anomalies and are not neoplastic, on the basis of the presence or absence of neoplastic proliferation of vascular endothelial cells. It is important that radiologists be familiar with the development, diagnosis, and treatment of vascular tumors and malformations, especially the imaging features of low- and high-flow vascular malformations. Some vascular tumors and malformations develop in isolation, whereas others develop within the phenotype of a syndrome. Syndromes that are associated with vascular tumors include PHACE syndrome. Syndromes that are associated with vascular malformations include Sturge-Weber, Klippel-Trénaunay, Proteus, blue rubber bleb nevus, Maffucci, and Gorham-Stout syndromes, all of which demonstrate low flow, and Rendu-Osler-Weber, Cobb, Wyburn-Mason, and Parkes Weber syndromes, all of which demonstrate high flow. Because imaging findings may help identify such syndromes as systemic, it is important that radiologists familiarize themselves with these conditions. PMID:23322836

Nozaki, Taiki; Nosaka, Shunsuke; Miyazaki, Osamu; Makidono, Akari; Yamamoto, Asako; Niwa, Tetsu; Tsutsumi, Yoshiyuki; Aida, Noriko; Masaki, Hidekazu; Saida, Yukihisa

432

Partial "targeted" embolisation of brain arteriovenous malformations  

PubMed Central

The treatment of pial arteriovenous brain malformations is controversial. Little is yet known about their natural history, their pathomechanisms and the efficacy and risks of respective proposed treatments. It is known that only complete occlusion of the AVM can exclude future risk of haemorrhage and that the rates of curative embolisation of AVMs with an acceptable periprocedural risk are around 20 to 50%. As outlined in the present article, however, partial, targeted embolisation also plays a role. In acutely ruptured AVMs where the source of bleeding can be identified, targeted embolisation of this compartment may be able to secure the AVM prior to definitive treatment. In unruptured symptomatic AVMs targeted treatment may be employed if a defined pathomechanism can be identified that is related to the clinical symptoms and that can be cured with an acceptable risk via an endovascular approach depending on the individual AVM angioarchitecture. This review article gives examples of pathomechanisms and angioarchitectures that are amenable to this kind of treatment strategy.

Hans, Franz-Josef; Geibprasert, Sasikhan; Terbrugge, Karel

2010-01-01

433

Partial tetrasomy 14 associated with multiple malformations.  

PubMed

We report on an 8-year-old female patient with multiple malformations including bilateral cleft lip and palate, coloboma, and craniosynostosis. She presented with severe intellectual disability, seizures, and gastrointestinal dysfunction. Mitochondrial investigations in a muscle biopsy revealed reduced activity in complex I of the mitochondrial respiratory chain. Chromosome analysis and fluorescent in situ hybridization (FISH) studies showed an isodicentric marker chromosome 14 that was identified in all cells analyzed in peripheral blood lymphocytes and cultured fibroblasts. Parental chromosome studies were normal. To further characterize the marker chromosome and determine its origin, we performed array-based comparative genomic hybridization (CGH) and polymorphic marker analysis with quantitative fluorescent PCR (QF-PCR). The combined results from cytogenetic and array-CGH analyses showed tetrasomy 14p13q13.1 and results from the QF-PCR point to formation of the marker chromosome in the maternal meiosis. Isodicentric chromosomes involving partial 14q have previously been reported in four cases; however, this is the first patient with tetrasomy 14p13q13.1 in non-mosaic form surviving beyond infancy. PMID:23613323

Winberg, Johanna; Lagerstedt Robinson, Kristina; Naess, Karin; Lesko, Nicole; Wibom, Rolf; Liedén, Agne; Anderlid, Britt-Marie; Graff, Caroline; Nordenskjöld, Agneta; Nordgren, Ann; Gustavsson, Peter

2013-04-23

434

Hypofractionated stereotactic radiotherapy for large arteriovenous malformations  

PubMed Central

Cerebral arteriovenous malformations (AVMs) are abnormal connections between the arteries and veins, with possible serious consequences of intracranial hemorrhage. The curative treatment for AVMs includes microsurgery and radiosurgery, sometimes with embolization as an adjunct. However, controversies exist with the treatment options available for large to giant AVMs. Hypofractionated stereotactic radiotherapy (HSRT) is one treatment option for such difficult lesions. We aim to review recent literature, looking at the treatment outcome of HSRT in terms of AVM obliteration rate and complications. The rate of AVM obliteration utilizing HSRT as a primary treatment was comparable with that of stereotactic radiosurgery (SRS). For those not totally obliterated, HSRT makes them smaller and turns some lesions manageable by single-dose SRS or microsurgery. Higher doses per fraction seemed to exhibit better response. However, patients receiving higher total dose may be at risk for higher rates of complications. Fractionated regimens of 7 Gy × 4 and 6–6.5 Gy × 5 may be accepted compromises between obliteration and complication. Embolization may not be beneficial prior to HSRT in terms of obliteration rate or the volume reduction. Future work should aim on a prospectively designed study for larger patient groups and long-term follow-up results.

Wang, Huan-Chih; Chang, Rachel J.; Xiao, Furen

2012-01-01

435

Mapping of the gene for cleidocranial dysplasia in the historical Cape Town (Arnold) kindred and evidence for locus homogeneity  

Microsoft Academic Search

Cleidocranial dysplasia (CCD) is an autosomal dominant disorder, features of which include a patient anterior fontanelle, a bulging calvarium, hypoplasia or aplasia of the clavicles, a wide public symphysis, dental anomalies, vertebral malformation, and short stature. The Cape Town kindred which is under our genetic management was originally described more than four decades ago and now consists of more than

R S Ramesar; J Greenberg; R Martin; R Goliath; S Bardien; S Mundlos; P Beighton

1996-01-01

436

Multistaged volumetric management of large arteriovenous malformations.  

PubMed

We sought to define the long-term outcomes and risks of arteriovenous malformation (AVM) management using 2 or more stages of stereotactic radiosurgery (SRS) for symptomatic large-volume AVMs unsuitable for surgery. Two decades ago, we prospectively began to stage anatomical components in order to deliver higher single doses to AVMs>10 cm3 in volume. Forty-seven patients with large AVMs underwent volume-staged SRS. The median interval between the two SRS procedures was 4.9 months (range, 3-14 months). The median nidus volume was 11.5 cm3 (range, 4.0-26 cm3) in the first stage of SRS and 9.5 cm3 in the second. The median margin dose was 16 Gy (range, 13-18 Gy) for both SRS stages. The actuarial rates of total obliteration after 2-staged SRS were 7, 20, 28 and 36% at 3, 4, 5 and 10 years, respectively. Sixteen patients needed additional SRS at a median interval of 61 months (range, 33-113 months) after the 2-staged SRS. After repeat procedure(s), the eventual obliteration rate was 66% at 10 years. The cumulative rates of AVM hemorrhage after SRS were 4.3, 8.6, 13.5 and 36.0% at 1, 2, 5 and 10 years, respectively. Symptomatic adverse radiation effects were detected in 13% of patients. Successful prospective volume-staged SRS for large AVMs unsuitable for surgery requires 2 or more procedures to complete the obliteration process. Patients remain at risk for hemorrhage if the AVM persists. PMID:23258511

Kano, Hideyuki; Kondziolka, Douglas; Flickinger, John C; Park, Kyung-Jae; Parry, Phillip V; Yang, Huai-che; Sirin, Sait; Niranjan, Ajay; Novotny, Josef; Lunsford, L Dade

2012-12-11

437

Epidemiology and natural history of arteriovenous malformations.  

PubMed

The epidemiology and natural history of cerebral arteriovenous malformations (AVMs) remains incompletely elucidated. Several factors are responsible. With regard to the incidence and prevalence of AVMs, the results of prior studies have suffered because of the retrospective design, the use of nonspecific ICD-9 codes, and a focus on small genetically isolated populations. Recent data from the New York Islands AVM Hemorrhage Study, an ongoing, prospective, population-based survey determining the incidence of AVM-related hemorrhage and the associated rates of morbidity and mortality in a zip code-defined population of 10 million people, suggests that the AVM detection rate is 1.21/100,000 person-years (95% confidence interval [CI] 1.02-1.42) and the incidence of AVM-hemorrhage is 0.42/100,000 person-years (95% CI 0.32-0.55). Contemporaneous data from the Northern Manhattan Stroke Study, a prospective, longitudinal population-based study of nearly 150,000 patients in which the focus is to define the incidence of stroke, suggest the crude incidence for first-ever AVM-related hemorrhage to be 0.55/100,000 person-years (95% CI 0.11-1.61). Efforts are ongoing to study the natural history of both ruptured and unruptured AVMs in these datasets to examine the relevance of prior studies of patients selected for conservative follow up in Finland. In addition, data are being gathered to determine whether risk factors for future hemorrhage, which have previously been established in small case series, are valid when applied to whole populations. Together, these data should help inform therapeutic decisionmaking. PMID:16466233

Stapf, C; Mohr, J P; Pile-Spellman, J; Solomon, R A; Sacco, R L; Connolly, E S

2001-11-15

438

AUTISM WITH OPHTHALMOLOGIC MALFORMATIONS: THE PLOT THICKENS  

PubMed Central

ABSTRACT Purpose To review the association of autism spectrum disorder (ASD) in individuals manifesting thalidomide embryopathy and Möbius sequence and compare them with three new studies in which ASD was also associated with ocular and systemic malformations: (1) a Swedish study of individuals with CHARGE association (Coloboma, Heart, choanal Atresia, developmental or growth Retardation, Genital anomaly, and Ear involvement); (2) a Swedish study of Goldenhar syndrome; and (3) Brazilian Möbius syndrome (sequence) study. Methods In the Swedish CHARGE study, 31 patients met the inclusion criteria (3+ or 4 of the common characteristics of the CHARGE syndrome). The same team of investigators also evaluated 20 Swedish patients with Goldenhar syndrome. In the Brazilian Möbius study, 28 children with a diagnosis of Möbius sequence were studied; some children had a history of exposure during their mother’s pregnancy to the abortifacient drug misoprostol in an unsuccessful abortion attempt Results In the CHARGE study, five patients had the more severe autism disorder and five had autistic-like condition. In the Goldenhar study, two had autism disorder and one had autistic-like condition. In the Brazilian Möbius study, the systemic findings of the misoprostol-exposed and misoprostol-unexposed patients were almost undistinguishable, and ASD was present in both groups (autism disorder in five and autistic-like condition in three). Conclusion Autism spectrum disorder has been reported in two conditions with known early pregnancy exposure to the teratogenic agents thalidomide and misoprostol. In the Brazilian Möbius study, autism also occurred in both the misoprostol-exposed and misoprostol-unexposed groups. Autism also was present in patients with both CHARGE association and Goldenhar syndrome.

Miller, Marilyn T; Stromland, Kerstin; Ventura, Liana; Johansson, Maria; Bandim, Jose M; Gillberg, Christopher

2004-01-01

439

Altered Intrinsic Properties of Neuronal Subtypes in Malformed Epileptogenic Cortex  

PubMed Central

Neuronal intrinsic properties control action potential firing rates and serve to define particular neuronal subtypes. Changes in intrinsic properties have previously been shown to contribute to hyperexcitability in a number of epilepsy models. Here we examined whether a developmental insult producing the cortical malformation of microgyria altered the identity or firing properties of layer V pyramidal neurons and two interneuron subtypes. Trains of action potentials were elicited with a series of current injection steps during whole cell patch clamp recordings. Cells in malformed cortex identified as having an apical dendrite had firing patterns similar to control pyramidal neurons. The duration of the second action potential in the train was increased in paramicrogyral (PMG) pyramidal cells, suggesting that these cells may be in an immature state, as was previously found for layer II/III pyramidal neurons. Based on stereotypical firing patterns and other intrinsic properties, fast-spiking (FS) and low threshold-spiking (LTS) interneuron subpopulations were clearly identified in both control and malformed cortex. Most intrinsic properties measured in malformed cortex were unchanged, suggesting that subtype identity is maintained. However, LTS interneurons in lesioned cortex had increased maximum firing frequency, decreased initial afterhyperpolarization duration, and increased total adaptation ratio compared to control LTS cells. FS interneurons demonstrated decreased maximum firing frequencies in malformed cortex compared to control FS cells. These changes may increase the efficacy of LTS while decreasing the effectiveness of FS interneurons. These data indicate that differential alterations of individual neuronal subpopulations may endow them with specific characteristics that promote epileptogenesis.

George, Amanda L.; Jacobs, Kimberle M.

2011-01-01

440

Neuroimaging of dandy-walker malformation: new concepts.  

PubMed

Dandy-Walker malformation (DWM) is the most common human cerebellar malformation, characterized by hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa with upward displacement of the lateral sinuses, tentorium, and torcular. Although its pathogenesis is not completely understood, there are several genetic loci related to DWM as well as syndromic malformations and congenital infections. Dandy-Walker malformation is associated with other central nervous system abnormalities, including dysgenesis of corpus callosum, ectopic brain tissue, holoprosencephaly, and neural tube defects. Hydrocephalus plays an important role in the development of symptoms and neurological outcome in patients with DWM, and the aim of surgical treatment is usually the control of hydrocephalus and the posterior fossa cyst. Imaging modalities, especially magnetic resonance imaging, are crucial for the diagnosis of DWM and distinguishing this disorder from other cystic posterior fossa lesions. Persistent Blake's cyst is seen as a retrocerebellar fluid collection with cerebrospinal fluid signal intensity and a median line communication with the fourth ventricle, commonly associated with hydrocephalus. Mega cisterna magna presents as an extraaxial fluid collection posteroinferior to an intact cerebellum. Retrocerebellar arachnoid cysts frequently compress the cerebellar hemispheres and the fourth ventricle. Patients with DWM show an enlarged posterior fossa filled with a cystic structure that communicates freely with the fourth ventricle and hypoplastic vermis. Comprehension of hindbrain embryology is of utmost importance for understanding the cerebellar malformations, including DWM, and other related entities. PMID:24132069

Correa, Gustavo Gumz; Amaral, Lázaro Faria; Vedolin, Leonardo Modesti

2011-12-01

441

Treatment of a patient with classical paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome, with complement inhibitor eculizumab: Case Report  

PubMed Central

Background. Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired clonal disorder of hematopoietic stem cells involving all blood cells. Erythrocytes have increased susceptibility to complement-mediated haemolysis. Thrombosis is the leading cause of mortality and follows episodes of acute hemolysis. Eculizumab, a monoclonal antibody blocking activation of complement C5 is currently used in the treatment of PNH. Recent results demonstrated that eculizumab effectively reduces thrombosis. Description of case. We present a 30-year-old male patient admitted with abdominal and lumbar pain. Thorough investigation revealed severe hemolytic anemia requiring transfusions and hepatosplenomegaly. Imaging findings were compatible with a Budd-Chiari syndrome. Flow cytometry confirmed the PNH diagnosis. Due to refractory ascites he underwent a transjugular intrahepatic portal-systemic shunt (TIPS) and eculizumab administration was started. Results. He has already completed three years of eculizumab treatment and he is transfusion independent. There is also a significant reduction in fatigue with improvement in his quality of life. Doppler scans of his TIPS persistently show it to be patent. Conclusions. Classical PNH patients with thrombosis and severe intravascular hemolysis are particularly challenging to manage. For these patients, eculizumab is a reasonable therapeutic option, expecting that by decreasing the risk for thrombosis, life expectancy may be increased.

Mandala, E; Lafaras, C; Goulis, I; Tsioni, K; Georgopoulou, V; Ilonidis, G

2013-01-01

442

Catheter-directed thrombolytic therapy combined with angioplasty for hepatic vein obstruction in Budd-Chiari syndrome complicated by thrombosis  

PubMed Central

The aim of this study was to assess the efficacy and safety of catheter-directed thrombolysis combined with angioplasty in the treatment of hepatic vein obstruction in Budd-Chiari syndrome (BCS) complicated by thrombosis. In 14 cases of BCS, the patients with hepatic vein obstruction complicated by thrombosis who underwent catheter-directed urokinase thrombolysis, balloon dilatation and/or stent placement were followed up with an ultrasound examination of the liver. Among the 13 cases of successful treatment, one hepatic vein was recanalized in 12 patients (right hepatic vein, seven cases; left hepatic vein, three cases; middle hepatic vein, one case and accessory hepatic vein, one case) and two hepatic veins (right and left) were recanalized in one patient without serious complications, such as bleeding and pulmonary embolism. There was one patient in whom the treatment was unsuccessful. During an average follow-up period of 24.8±19.6 months, hepatic vein restenosis was observed in one patient in the sixth month after opperation; however, a successful result was obtained following a second balloon dilatation. The remaining 12 patients did not demonstrate any recurrence of restenosis or thrombosis. Catheter-directed thrombolysis combined with angioplasty was observed to be an effective and safe method for the treatment of hepatic vein obstruction in BCS complicated by thrombosis.

ZHANG, QINGQIAO; XU, HAO; ZU, MAOHENG; GU, YUMING; WEI, NING; WANG, WENLIANG; GAO, ZHIKANG; SHEN, BIN

2013-01-01

443

Fatal Cardiovascular Collapse During Ethanol Sclerotherapy of a Venous Malformation  

PubMed Central

Summary We report a case of fatal cardiovascular collapse that occurred during Ethanol sclerotherapy of a venous malformation in a 21-year-old woman. The malformation was located on the anterior part of the thigh. Fifty ml of a mixture of Ethanol, Ethibloc and Lipiodol containing 35 ml of Ethanol (0.52 ml / kg) were injected under fluoroscopy. A major drop in arterial pressure was recorded after release of the tourniquet placed at the thigh root. The patient died after four hours of intensive cardiac reanimation. Her blood alcohol level was 0.4 g/l one hour after the end of the intervention. The cardiac toxicity of ethanol depends more on the potential acute venous contamination than on the blood alcohol concentration. The currently admitted “safety limit” of 1 ml/kg of bodyweight for ethanol sclerotherapy of venous malformations is certainly unsafe and must be redefined.

Chapot, R.; Laurent, A.; Enjolras, O.; Payen, D.; Houdart, E.

2002-01-01

444

Basic genetic principles applied to posterior fossa malformations.  

PubMed

Recent advances in neuroimaging techniques turned possible for neuroradiologists to be frequently the first one to detect possible brain structural anomalies. However, with all the recent advances in genetics and embryology, understanding posterior fossa malformation's principles is being hardest to be achieved than previously. Studies in vertebrate models provide a developmental framework in which to categorize human hindbrain malformations and serve to inform our thinking regarding candidate genes involved in disrupted developmental processes. The main focus of this review was to survey the basic principles of the rhombomere division, anteroposterior and dorsoventral patterning, alar and basal zone concept, and axonal path finding to integrate the knowledge of human hindbrain malformations for better understanding the genetic basis of hindbrain development. PMID:24132065

Nunes, Renato Hoffmann; Littig, Ingrid Aguiar; da Rocha, Antonio Jose; Vedolin, Leonardo

2011-12-01

445

Neurosurgical vascular malformations in children under 1 year of age  

Microsoft Academic Search

Purpose  This study aims to analyze the clinical and radiological findings, timing and type of treatment, and outcome in children under\\u000a 1 year of age that presented with neurosurgical vascular malformations.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  A retrospective review of 23 children under 1 year of age with neurosurgical vascular malformations was performed at a single\\u000a institution between 1999 and 2009.\\u000a \\u000a \\u000a \\u000a \\u000a Results  The lesions found in this age group

Graciela Zuccaro; Romina Argańaraz; Francisco Villasante; Alejandro Ceciliano

2010-01-01

446

Uncommon cavernous malformation of the optic chiasm: a case report.  

PubMed

Cavernous malformation (CM) is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans. PMID:22892383

Ning, Xianbin; Xu, Kan; Luo, Qi; Qu, Limei; Yu, Jinlu

2012-08-14

447

Hydrosalpinx in a patient with complex genitourinary malformation.  

PubMed

We describe an adolescent female patient born with a complex genitourinary malformation including bilateral duplex system and duplication of the müllerian structures (bicornuate uterus and septate vagina). She presented with a symptomatic hydrosalpinx. The typical imaging of this condition is described along with the issues associated with the differential diagnosis in this complex scenario. The diagnosis of hydrosaplinx should be suspected in patients with complex genitourinary malformations and a pelvic fluid collection. Associated genital and renal anomalies are noted in 30% of cases. PMID:21034959

Zhapa, Evisa; Rigamonti, Waifro; Castagnetti, Marco

2010-11-01

448

Renal-hepatic-pancreatic dysplasia: an autosomal recessive malformation.  

PubMed Central

We report two brothers with a cystic malformation of the kidneys, liver, and pancreas. In both cases the malformation was fatal and the children died shortly after birth. The pathological findings, consisting of multicystic dysplastic kidneys, dilated and dysgenetic bile ducts, dilated pancreatic ducts, and polysplenia, correspond to those reported by Ivemark as renal-hepatic-pancreatic dysplasia. Many polymalformation syndromes include cystic affectation of these three organs, so this syndrome could be an isolated entity or a final common pathway of response of these organs to a variety of developmental disturbances, which could also include splenic abnormalities. We propose an autosomal recessive pattern of inheritance for renal-hepatic-pancreatic dysplasia. Images

Torra, R; Alos, L; Ramos, J; Estivill, X

1996-01-01

449

Diffusion tensor imaging and fiber tractography in brain malformations.  

PubMed

Diffusion tensor imaging (DTI) is an advanced MR technique that provides qualitative and quantitative information about the micro-architecture of white matter. DTI and its post-processing tool fiber tractography (FT) have been increasingly used in the last decade to investigate the microstructural neuroarchitecture of brain malformations. This article aims to review the use of DTI and FT in the evaluation of a variety of common, well-described brain malformations, in particular by pointing out the additional information that DTI and FT renders compared with conventional MR sequences. In addition, the relevant existing literature is summarized. PMID:23288476

Poretti, Andrea; Meoded, Avner; Rossi, Andrea; Raybaud, Charles; Huisman, Thierry A G M

2013-01-04

450

Uncommon cavernous malformation of the optic chiasm: a case report  

PubMed Central

Cavernous malformation (CM) is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans.

2012-01-01

451

Parry-Romberg syndrome associated with intracranial vascular malformations.  

PubMed

We describe a 23-year-old woman with iridocyclitis, enophthalmos, facial hemiatrophy, and transient numbness of her contralateral upper and lower extremities. The patient was found to have white matter densities in the right hemisphere in magnetic resonance T2-weighted images and vascular malformations involving right vertebral, right carotid, and right anterior cerebral arteries. Histopathologic evaluation of a biopsy specimen of anterior orbital fat and lacrimal gland revealed fibrosis and chronic inflammation. These findings were consistent with the diagnosis of progressive facial hemiatrophy (Parry-Romberg syndrome) in association with iridocyclitis and intracranial vascular malformations. PMID:9747688

Miedziak, A I; Stefanyszyn, M; Flanagan, J; Eagle, R C

1998-09-01

452

Multiple spinal cavernous malformations in Klippel-Trenaunay-Weber syndrome.  

PubMed

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital vascular disorder characterized by cutaneous haemangiomas, venous varicosities, and hypertrophy of the osseous and soft tissue. Various vascular anomalies of the central nervous system have been described in this syndrome. Two previous associations between KTWS and spinal cord cavernous malformations have been reported in the English literature. In this report, we present a patient in whom multiple cavernous malformations located in the conus medullaris region and cauda equina were associated with KTWS. General physical examination as well as neuroradiological and operative findings are described. PMID:23161195

Göksu, Ethem; Alpsoy, Erkan; Uçar, Tanju; Tuncer, Recai

453

[Polysplenia syndrome: two cases in adults revealed by biliary and pancreatic malformations].  

PubMed

Polysplenia Syndrome (PS) associates multiple spleens with other malformations usually cardiac, vascular, visceral and biliary. The diversity of these malformations and their embryological mechanisms are described in relation to two cases of PS that were diagnosed in adults. PMID:18166868

Puche, Pierre; Jacquet, Eric; Godlewski, Guilhem; Carabalona, Jean-Pierre; Domergue, Jacques; Navarro, Francis; Prudhomme, Michel

2007-10-01

454

Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.  

PubMed

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients. PMID:22000870

Redondo, Pedro; Aguado, Leyre; Martínez-Cuesta, Antonio

2011-11-01

455

Notch-1 Signalling Is Activated in Brain Arteriovenous Malformations in Humans  

ERIC Educational Resources Information Center

|A role for the Notch signalling pathway in the formation of arteriovenous malformations during development has been suggested. However, whether Notch signalling is involved in brain arteriovenous malformations in humans remains unclear. Here, we performed immunohistochemistry on surgically resected brain arteriovenous malformations and found…

ZhuGe, Qichuan; Zhong, Ming; Zheng, WeiMing; Yang, Guo-Yuan; Mao, XiaoOu; Xie, Lin; Chen, Gourong; Chen, Yongmei; Lawton, Michael T.; Young, William L.; Greenberg, David A.; Jin, Kunlin

2009-01-01

456

Changing role for preoperative embolisation in the management of arteriovenous malformations of the brain  

Microsoft Academic Search

Background: The aim of this study was to analyse the results of the use of preoperative embolisation in the management of arteriovenous malformations of the brain at one institution between 1989 and 1999.Methods : Two hundred and fifty consecutive cases of angiographically confirmed arteriovenous malformations underwent surgery by one surgeon. Cases of dural or spinal arteriovenous malformations have been excluded.

M. K. Morgan; A. A. R. Zurin; T. Harrington; N. Little

2000-01-01

457

Clinical implications of uterine malformations and hysteroscopic treatment results  

Microsoft Academic Search

Uterine malformations consist of a group of miscellaneous congenital anomalies of the female genital system. Their mean prevalence in the general population and in the population of fertile women is ~4.3%, in infertile patients ~3.5% and in patients with recurrent pregnancy losses ~13%. Septate uterus is the commonest uterine anomaly with a mean incidence of ~35% followed by bicornuate uterus

Grigoris F. Grimbizis; Michel Camus; Basil C. Tarlatzis; John N. Bontis; Paul Devroey

458

Spinal vascular malformations--typical and atypical findings.  

PubMed

Vascular malformations of the spinal cord and its meninges are rare diseases which comprise true inborn cavernomas and arteriovenous malformations (AVM), including perimedullary fistulae, glomerular and juvenile AVMs, and presumably acquired dural arteriovenous fistulae. This article gives an overview of the imaging features on magnetic resonance imaging (MRI) and digital subtraction angiography of both typical and atypical findings to describe the wide variety of possible pathological entities encountered. Clinical differential diagnoses, the neurological symptomatology and potential therapeutic approaches of these diseases, which may vary depending on the underlying pathology, are given. Although MRI constitutes the first choice diagnostic modality for suspected spinal vascular malformations, we conclude that the definite diagnosis of the disease and thus the choice of the appropriate therapeutic approach rests on selective spinal angiography which should be performed at a specialized center. Treatment in symptomatic patients offers an improvement in prognosis. Microsurgical treatment is recommended for symptomatic spinal cord cavernomas. Dural arteriovenous shunts can either be treated by microsurgical or endovascular approaches, the former being a simple, quick and secure approach to obliterate the fistula while the latter is technically demanding. In spinal arteriovenous malformations of both the fistulous and the glomerular type, the endovascular approach is the method of first choice; in selected cases, surgery or a combined therapy may be necessary. PMID:17966075

Bostroem, A; Thron, A; Hans, F J; Krings, T

2007-10-26

459

Thalidomide-induced limb malformations — a prosthetic review  

Microsoft Academic Search

The embryopathy-producing drug 'thalidomide' resulted in an estimated 349 babies born in the UKwith congenital malformations during the period 1959-62. All known affected babies were registered, and a 'Thalidomide Society and Trust' was formed to administer the financial settlements offered by the manufacturers of the drug and the British Government. Individual prosthetic centres found great difficulty at the time in

Nabil M Mustapha

1990-01-01

460

Mathematical Models for Tubular Malformations during Virus Assembly of Papovaviridae  

Microsoft Academic Search

An important stage of the viral life cycle is the formation of a protein shell, called the viral capsid, that protects the viral genome. While the viral capsids of infectious virus particles correspond to closed shells, there also occur tubular open structures during assembly as non-infectious malformations. In (1, 2) viral tiling theory (VT-theory) has been introduced as a new

REIDUN TWAROCK

461

Neurodevelopmental Outcomes in Children with Cerebellar Malformations: A Systematic Review  

ERIC Educational Resources Information Center

|Cerebellar malformations are increasingly diagnosed in the fetal period. Consequently, their consideration requires stressful and often critical decisions from both clinicians and families. This has resulted in an emergent need to understand better the impact of these early life lesions on child development. We performed a comprehensive…

Bolduc, Marie-Eve; Limperopoulos, Catherine

2009-01-01

462

Proximity to pollution sources and risk of amphibian limb malformation.  

PubMed

The cause of limb deformities in wild amphibian populations remains unclear, even though the apparent increase in prevalence of this condition may have implications for human health. Few studies have simultaneously assessed the effect of multiple exposures on the risk of limb deformities. In a cross-sectional survey of 5,264 hylid and ranid metamorphs in 42 Vermont wetlands, we assessed independent risk factors for nontraumatic limb malformation. The rate of nontraumatic limb malformation varied by location from 0 to 10.2%. Analysis of a subsample did not demonstrate any evidence of infection with the parasite Ribeiroia. We used geographic information system (GIS) land-use/land-cover data to validate field observations of land use in the proximity of study wetlands. In a multiple logistic regression model that included land use as well as developmental stage, genus, and water-quality measures, proximity to agricultural land use was associated with an increased risk of limb malformation (odds ratio = 2.26; 95% confidence interval, 1.42-3.58; p < 0.001). The overall discriminant power of the statistical model was high (C = 0.79). These findings from one of the largest systematic surveys to date provide support for the role of chemical toxicants in the development of amphibian limb malformation and demonstrate the value of an epidemiologic approach to this problem. PMID:16263502

Taylor, Brynn; Skelly, David; Demarchis, Livia K; Slade, Martin D; Galusha, Deron; Rabinowitz, Peter M

2005-11-01

463

The tethered spinal cord in patients with anorectal malformations  

Microsoft Academic Search

The aims of this study were to find the prevalence of tethered cord in patients with anorectal malformations; to determine if the presence of tethered cord relates to the severity of the anorectal defect, and to certain symptoms, signs, radiologic findings, and associated anomalies; and finally to determine whether tethered cord impacted on a patient's functional prognosis and whether surgical

Marc A Levitt; Mahendra Patel; George Rodriguez; Daniel S Gaylin; Alberto Peńa

1997-01-01

464

Prenatal exposure to thalidomide, altered vasculogenesis, and CNS malformations  

Microsoft Academic Search

Malformations of cortical development (MCD) result from abnormal neuronal positioning during corticogenesis. MCD are believed to be the morphological and perhaps physiological bases of several neurological diseases, spanning from mental retardation to autism and epilepsy. In view of the fact that during development, an appropriate blood supply is necessary to drive organogenesis in other organs, we hypothesized that vasculogenesis plays

K. L. Hallene; E. Oby; B. J. Lee; S. Santaguida; S. Bassanini; M. Cipolla; N. Marchi; M. Hossain; G. Battaglia; D. Janigro

2006-01-01

465

Occurrence of Split Cord Malformation in Meningomyelocele: Complex Spina bifida  

Microsoft Academic Search

Objective: To describe the clinical features and surgical outcome of a combined anomaly, i.e. split cord malformation (SCM) with meningomyelocele (MMC), and to propose an addition to Pang’s classification of SCM to accommodate a combined form of anomaly. Methods: We retrospectively analyzed 16 cases of such a combination, out of a total of 106 cases of spinal dysraphism treated and

Raj Kumar; Krishan Kumar Bansal; Devendra Kumar Chhabra

2002-01-01

466

Congenital malformations and genetic diseases in Iranian infants  

Microsoft Academic Search

Data of 13,037 live-born infants from a hospital in Tehran, Iran were analysed for congenital malformations and genetic diseases. The results showed that the rates of joint dislocation, eleft lip, cleft palate and finger anomalies are similar to those of the other populations. The rates of chromosomal, thorax and abdominal, external genital anomalies and other syndromes were higher compared with

D. D. Farhud; Gh.-R. Walizadeh; M. Sharif Kamali

1986-01-01

467

Management of the fetus with a cystic adenomatoid malformation  

Microsoft Academic Search

Prenatal detection and serial sonographic study of fetuses with congenital cystic adenomatoid malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and

N. Scott Adzick; Michael R. Harrison

1993-01-01

468

Diagnosis and Nonsurgical Management of Uterine Arteriovenous Malformation  

SciTech Connect

Uterine arteriovenous malformation (AVM) is an uncommon problem and traditional treatment by hysterectomy excludes the possibility of future pregnancy. Developments in interventional techniques make transcatheter embolization of the feeding vessel(s) a therapeutic alternative, potentially preserving the patient's fertility. We present a case of successful endovascular treatment of uterine AVM.

Rangarajan, R. D.; Moloney, J. C.; Anderson, H. J. [Eastbourne District General Hospital, Department of Radiology (United Kingdom)], E-mail: Hugh.Anderson@esht.nhs.uk

2007-11-15

469

Aplasia cutis congenita associated with an immature arteriovenous malformation  

Microsoft Academic Search

The first case of congenital defect of the skull and scalp in Japan, probably due to an immature arteriovenous malformation, is presented. In this case, an island of healthy scalp remained intact in the central area of the scalp defect, unlike previously reported cases. The dilated, tortuous scalp vessels apparently disappeared spontaneously only after multiple ligation. Because of slow wound

H. Ohtsuka; M. Yamamoto; N. Okayama

1990-01-01

470

Diencephalic-Mesencephalic Junction Dysplasia: A Novel Recessive Brain Malformation  

ERIC Educational Resources Information Center

|We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic "butterfly"-like contour of the midbrain on…

Zaki, Maha S.; Saleem, Sahar N.; Dobyns, William B.; Barkovich, A. James; Bartsch, Hauke; Dale, Anders M.; Ashtari, Manzar; Akizu, Naiara; Gleeson, Joseph G.; Grijalvo-Perez, Ana Maria

2012-01-01

471

Hypospadias and anorectal malformations mediated by Eph/ephrin signaling  

PubMed Central

Purpose Despite extensive research, the molecular basis of hypospadias and anorectal malformations is poorly understood, likely due to a multifactorial basis. The incidence of hypospadias is increasing, thus making research in this area warranted and timely. This review presents recent molecular work broadening our understanding of these disorders. Materials and Methods A brief review of our recent work and the literature on the role of Eph/ephrin signaling in hypospadias and anorectal malformations is presented. Results Genetically engineered mice mutant for ephrin-B2 or EphB2;EphB3 manifest a variety of genitourinary and anorectal malformations. Approximately 40% of adult male heterozygous mice demonstrate perineal hypospadias. Although homozygous mice die soon after birth, 100% of homozygous males demonstrate high imperforate anus with urethral anomalies and 100% of homozygous females demonstrate persistent cloaca. Male mice compound homozygous for EphB2ki/ki;EphB3?/?/ also demonstrate hypospadias. Conclusions These mouse models provide compelling evidence of the role of B-class Eph/ephrin signaling in genitourinary/anorectal development and add to our mechanistic and molecular understanding of normal and abnormal embryonic development. As research on the B-class Ephs and ephrins continues, they will likely be shown to be molecular contributors to the multifactorial basis of hypospadias and anorectal malformations in humans as well.

Yucel, Selcuk; Dravis, Christopher; Garcia, Nilda; Henkemeyer, Mark; Baker, Linda A.

2007-01-01

472

Surgical treatment of venous malformations in Klippel-Trénaunay syndrome  

Microsoft Academic Search

Purpose: Klippel-Trénaunay syndrome (KTS) is a complex congenital anomaly, characterized by varicosities and venous malformations (VMs) of one or more limbs, port-wine stains, and soft tissue and bone hypertrophy. Venous drainage is frequently abnormal because of embryonic veins, agenesis, hypoplasia, valvular incompetence, or aneurysms of deep veins. We previously reported on the surgical management of KTS. In this article, we

Audra A Noel; Peter Gloviczki; Kenneth J Cherry; Thom W Rooke; Anthony W Stanson; David J Driscoll

2000-01-01

473

Arnold Durig (1872-1961): life and work. An Austrian pioneer in exercise and high altitude physiology.  

PubMed

Arnold Durig (1872-1961) grew up in the Austrian mountains in the period when intense exploration of the Alps started. As an enthusiastic mountaineer, scientist, and physician, he became one of the pioneers exploring physiological and pathophysiological aspects of humans sojourning to high altitudes. At the beginning of the 20(th) century, Durig was one of the great physiologists whose knowledge covered the whole field of physiology. Durig founded a renowned School and his students spread all over the world. He stayed in close contact with many colleagues and famous scientists, such as Albert Einstein and Sigmund Freud. Although he was an extremely productive and acknowledged physiologist and teacher at that time, his work and life are not very well known at the beginning of the 3(rd) millennium, even by high altitude physiologists. Thus, this article provides an overview on Durig's life and work, highlighting the most important scientific studies he performed at moderate and high altitudes, in an attempt to provide a few links to the development of high altitude research in the late 19(th) and early 20(th) centuries, complemented by some comments from a current point of view. PMID:22994523

Burtscher, Martin; Gnaiger, Erich; Burtscher, Johannes; Nachbauer, Werner; Brugger, Andreas

2012-09-01

474

Periorbital lymphatic malformation: clinical course and management in 42 patients.  

PubMed

Lymphatic malformation in the orbital cavity and surrounding region often causes disfigurement and visual problems. To better clarify the evolution and treatment of this condition, the authors studied a retrospective cohort of 42 consecutive patients seen between 1971 and 2003 and analyzed anatomic features, complications, and management. The ratio of female to male patients was 1:1. Most periorbital lymphatic malformations were noted at birth (59 percent), presenting as either unilateral swelling (60 percent) or a periorbita