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1

The Arnold-Chiari Type II Malformation at Midgestation  

Microsoft Academic Search

The Arnold-Chiari malformation type II (ACMII) is reported to be reversible after closure of a myelomeningocele at midgestation. To elucidate the developmental state of the ACMII malformation at the approximate time fetal surgery is performed, the ACMII of a 20-week human fetus was investigated in vitro using high-field magnetic resonance microscopy at 9.4 T and compared with the hindbrain of

E. Beuls; L. Vanormelingen; J. van Aalst; M. Vandersteen; P. Adriaensen; E. Cornips; H. Vles; Y. Temel; J. Gelan

2003-01-01

2

Arnold-Chiari malformation in a captive African lion cub.  

PubMed

Progressive ataxia, delayed growth, dementia and tremors were noted in a female African lion (Panthera leo) cub at the Tel-Aviv Ramat-Gan Zoological Center (Israel). The lioness was 3-mo-old when clinical signs were first noticed. Repeated neurological evaluations and blood tests were conducted in an attempt to establish a diagnosis. A congenital abnormality was suspected and the lioness died 6 molater. Post mortem examination revealed an Arnold-Chiari malformation. The abnormality was classified as a Chiari type 2 malformation, based on the herniation of the cerebellar vermis and paravermis and the slight caudal displacement of the medulla, combined with lack of displacement in other parts of the brainstem. PMID:9706583

Shamir, M H; Horowitz, I H; Yakobson, B; Ofri, R

1998-07-01

3

Symptomatic Arnold-Chiari Malformation and Cranial Nerve Dysfunction: a Case Study of Applied Kinesiology Cranial Evaluation and Treatment  

Microsoft Academic Search

ObjectiveTo present an overview of possible effects of Arnold-Chiari malformation (ACM) and to offer chiropractic approaches and theories for treatment of a patient with severe visual dysfunction complicated by ACM.

Scott Cuthbert; Charles Blum

2005-01-01

4

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

5

Arnold-Chiari Malformation Type III With Meningoencephalocele: A Case Report  

PubMed Central

Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III. PMID:25024966

Jeong, Dae Ho; Kim, Chang Hwan; Kim, Myeong Ok; Chung, Hyung; Kim, Tae Hyun

2014-01-01

6

Syringomyelia and Arnold-Chiari malformation associated with neck pain and left arm radiculopathy treated with spinal manipulation.  

PubMed

An 18-year-old female patient presented with left dominant neck pain after a motor vehicle collision. Her cervical spine MRI revealed syringomyelia with associated Type I Arnold-Chiari malformation. Some researchers have reported that these might be considered contraindications to spinal manipulation. Nevertheless, her benign and functional clinical examination suggested otherwise and she underwent four manipulative treatments in 2?weeks. By the end of the treatment plan and after 1-month follow-up, she was asymptomatic, no adverse effects were noted and her outcome assessment score decreased from 56% to 0%. This case illustrates that spinal manipulation may be a useful adjunctive treatment procedure for spinal pain, even in the presence of syringomyelia and Chiari malformation, which may not necessarily be a contraindication to spinal manipulation, when performed by a skilled and well-trained physician. PMID:25385566

Tieppo Francio, Vinicius

2014-01-01

7

Chiari Malformation  

MedlinePLUS

... blocked. X-rays produce images of bones on film and can detect bone abnormalities caused by Chiari ... guidelines Share this page using: What are these? Note: Clicking these links will take you to a ...

8

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

9

Congenital Chiari malformations.  

PubMed

Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available. PMID:20228456

Vannemreddy, Prasad; Nourbakhsh, Ali; Willis, Brian; Guthikonda, Bharat

2010-01-01

10

Chiari malformation in craniosynostosis  

Microsoft Academic Search

Introduction: Chiari mal- formation (CM) is a frequent finding in multisutural and syndromic cra- niosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still con- troversial. Discussion: Since its first description in 1972, several factors have been

Giuseppe Cinalli; Pietro Spennato; Christian Sainte-Rose; Eric Arnaud; Ferdinando Aliberti; Francis Brunelle; Emilio Cianciulli; Dominique Renier

2005-01-01

11

Chiari II Malformation and Occult Spinal Dysraphism  

Microsoft Academic Search

We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the ‘closed’ form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither

R. Shane Tubbs; John C. Wellons III; Paul A. Grabb; W. Jerry Oakes

2003-01-01

12

The Chiari II Malformation: A Surgical Series  

Microsoft Academic Search

Thirty patients between the ages of 7 months and 24 years were treated surgically for symptomatic Chiari II malformation at the Arkansas Children’s Hospital. All patients underwent at least bony decompression of the malformation. Assessments of the patients’ conditions were made at 6 weeks and 1 year after surgery, and complications of surgery were noted. For a majority of the

Charles Teo; Erik C. Parker; Sharon Aureli; Frederick A. Boop

1997-01-01

13

Esophageal dysphagia as the sole symptom in type I chiari malformation  

Microsoft Academic Search

Summary  Chiari malformations, also called Arnold-Chiari deformities, are rare hindbrain herniations that may present in children or adults. The most common symptoms include headache, syncope, disordered eye movement, sensory loss, weakness, and cerebellar features such as ataxia. Dysphagia occurs in 5–15% of patients, although only a few reports describe dysphagia as the only presenting symptom. We report a case of a

Grace H. Elta; Cary A. Caldwell; Timothy T. Nostrant

1996-01-01

14

Spontaneous resolution of isolated Chiari I malformation  

Microsoft Academic Search

Introduction  Spontaneous resolution of Chiari I abnormality is very rare. In most patients, the radiological abnormality either stays unchanged with time or deriorates.Case report  We present a male patient who was diagnosed at the age of 18 months as having radiological evidence of Chiari I malformation without syringomyelia, which had resolved 5 years later on a subsequent MR scan. At the time

N. S. Jatavallabhula; J. Armstrong; S. Sgouros; W. Whitehouse

2006-01-01

15

Atypical Presentations in Chiari II Malformation  

Microsoft Academic Search

Myelomeningocele with Chiari II malformation and hydrocephalus is a common association seen in infants with a congenital failure of neurulation. Here we report two cases of such an association presenting with different sets of problems. The first patient presented with severe inspiratory stridor due to bilateral abductor vocal cord paralysis, which was relieved completely within 24 h of definitive surgery.

G. P. Rath; P. K. Bithal; A. Chaturvedi

2006-01-01

16

Epilepsy in type 1 Chiari malformation  

Microsoft Academic Search

In patients with Chiari malformation type 1 (CMI), epileptic seizures are occasionally reported both in symptomatic patients\\u000a candidate to surgery and in patients without symptoms of tonsillar displacement in whom CM1 is often an incidental finding\\u000a in the diagnostic work up for idiopathic epilepsies. In both groups of patients, the course of epilepsy is almost invariably\\u000a favorable, with a few

Tiziana GranataLaura; Laura Grazia Valentini

17

History, anatomic forms, and pathogenesis of Chiari I malformations  

Microsoft Academic Search

IntroductionChiari I malformations constitute a group of entities of congenital or acquired etiology that have in common descent of the cerebellar tonsils into the cervical spinal canal. In recent years, since the advent of magnetic resonance imaging, an increasing number of asymptomatic, doubtfully symptomatic, and minimally symptomatic patients with Chiari I malformations have been diagnosed. This has resulted in controversy

Edgardo Schijman; C. Durand

2004-01-01

18

Surgical Treatment of Chiari I Malformation, with Preservation of Arachnoid  

Microsoft Academic Search

Objective: The author describe the results of performing a standard posterior craniovertebral decompression and duroplasty, with preservation of arachnoid layer, for the treatment of patients with symptomatic chiari I malformation with and without syringomyelia Patients and methods: This is a retrospectively analyzed series of ten patients with symptomatic chiari I malformation underwent surgical treatment from June 2003 to January 2007.

Alaa A. Farag

2007-01-01

19

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

20

Chiari Type I Malformation: Overview of Diagnosis and Treatment  

Microsoft Academic Search

Chiari Type I malformation (Chiari I) is a congenital hindbrain anomaly characterized by downward dis- placement of the cerebellar tonsils through the fora- men magnum. This can lead to compression of cere- bellar components, the lower brainstem, and the upper cervical spinal cord. In turn, a variety of neu- rological deficits and permanent nervous system damage may evolve. This review

John Nash; Joseph S. Cheng; Glenn A. Meyer; Bernd F. Remler

21

Imaging of Chiari type I malformation and syringohydromyelia.  

PubMed

Chiari malformations are anatomic anomalies that comprise a broad spectrum of neurologic conditions. The most common malformation, a Chiari type I malformation, can present with a variety of signs and symptoms, most frequently an occipital Valsalva-induced headache. Cranial and spinal magnetic resonance (MR) imaging is used to identify the degree of tonsillar descent and document the presence of syringohydromyelia. The advent of cine-MR flow imaging (cine as in "cinema") has provided new insight as to the dynamic process involved in the evolution of this pathophysiology. This article reviews the neuroimaging of this fascinating disorder. PMID:24287386

McVige, Jennifer W; Leonardo, Jody

2014-02-01

22

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

23

[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; Niño-Hernández, L M; Gutiérrez-Paternina, J J; Moscote-Salazar, L R; Preciado-Mesa, E

2011-12-01

24

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

PubMed Central

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

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

2010-01-01

25

Evaluation of Craniocervical Decompression with Duraplasty for Symptomatic Syringomyelia Associated with Chiari Type 1 Malformation in Adults  

Microsoft Academic Search

Objectives: many modalities for treatment of syringomyelia associated with Chiari type 1- malformation have evolved. The aim of our study is to evaluate the results of cranio-cervical decompression together with arachnoid lysis, and duraplasty for symptomatic syringomyelia associated with Chiari type- I malformation in 16 adult patients. Methods: Sixteen adult patients with symptomatic syringomyelia associated with Chiari type 1- malformation

Khaled El

2008-01-01

26

Treatment of the Chiari malformation by Drs. H.E. James and A. Brant  

Microsoft Academic Search

the secondary distortion, atrophy, or fibrosis. A common anomaly in Chiari types I and II is hypoplasia of the posterior fossa, which is usually not accompanied by histological malformations in the brain. We suggest that the central nervous system anomalies in Chiari anomalies types I and II are not the primary CNS malformation but only an adaptation to the hypoplasia

Akira Hori; A. Brant

2002-01-01

27

A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation  

Microsoft Academic Search

A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported. The patient developed congenital hydrocephalus, upper airway obstruction and breath holding spells. Ventriculo-peritoneal shunt improved the hydrocephalus, but the patient progressively developed apneic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervical vertebrae and craniectomy at the occipital bone

Shuichi Ito; Kiyoshi Matsui; Etsuro Ohsaki; Akiko Goto; Kimiyo Takagi; Mitsuhiko Koresawa; Susumu Ito; Kenichi Sekido; Masami Suzuki; Katsuyuki Torikai; Noriko Aida

1996-01-01

28

Transverse sinus stenosis in adult patients with Chiari malformation type I.  

PubMed

OBJECTIVE. Transverse sinus stenosis is an imaging finding very highly associated with elevated intracranial pressure (ICP). Patients with the Chiari I malformation may potentially have elevated ICP due to impairment of CSF flow at the foramen magnum. This study evaluated whether patients with Chiari I malformation have transverse sinus stenosis and other imaging findings indicative of elevated ICP. MATERIALS AND METHODS. Thirty patients with Chiari I malformation treated surgically and 76 control subjects were identified retrospectively. All control subjects and all patients with Chiari I malformation (preoperatively) underwent standardized contrast-enhanced brain MRI including a contrast-enhanced 3D T1-weighted sequence from which curved reformats of the transverse sinuses were generated. Two different readers blinded to the diagnosis then independently evaluated these curved reformats for severity of transverse sinus stenosis. Orbital and skull-base findings previously described in association with elevated ICP were also evaluated. Frequency of MRI findings between the two groups was compared. RESULTS. Patients with Chiari I malformation had significantly greater frequency of unilateral or bilateral transverse sinus stenosis than did control subjects (p < 0.001). There was complete interreader agreement on presence or absence of transverse sinus stenosis by patient (? = 1.0 [95% CI, 0.89-1.0]). Logistic regression analysis controlling for age, sex, and body mass index found that transverse sinus stenosis significantly predicted Chiari I malformation versus control status (odds ratio, 11.2 [95% CI, 2.1-59.0]; p = 0.004) but that no other features were significantly associated with the Chiari I malformation. Patients with Chiari I malformation who had transverse sinus stenosis had significantly greater pituitary flattening than did those without transverse sinus stenosis (p = 0.02). CONCLUSION. Patients with Chiari I malformation have higher likelihood of trans-verse sinus stenosis, which may reflect associated elevated ICP. PMID:25247957

Saindane, Amit M; Bruce, Beau B; Desai, Nilesh K; Roller, Lauren A; Newman, Nancy J; Biousse, Valérie

2014-10-01

29

A rare course of scoliosis associated with Chiari malformation and syringomyelia.  

PubMed

Spinal deformity is an important clinical manifestation of Chiari I malformation (CM-I) and syringomyelia. Here we report the result of an 8-year follow-up of a 13-year-old girl with severe scoliosis associated with Chiari malformation and a large syringomyelia. The patient presented at our hospital at the age of 13 with a 68° scoliosis. Magnetic resonance imaging showed Chiari malformation and a large syringomyelia. Neurosurgical treatment involved foramen magnum decompression and partial C1 laminectomy, but the scoliosis still progressed. We present the first case report of a rare course of scoliosis in a patient with CM-I and a large syringomyelia. PMID:25338487

Tanaka, Masato; Sugimoto, Yoshihisa; Arataki, Shinya; Takigawa, Tomoyuki; Ozaki, Toshifumi

2014-10-01

30

Interventional spinal procedures in the presence of a Chiari malformation: a potential contraindication.  

PubMed

Although Chiari malformations are much more prevalent than once believed, no study has described treatment with an interventional spinal procedure. The purpose of this report was to discuss the clinical course of a patient who was diagnosed with a Chiari malformation and treated with three cervical epidural injections. In 2012, a 50-yr-old woman presented to a neurology clinic with chronic suboccipital headaches, diplopia, and increasing numbness/tingling in her upper extremities. Magnetic resonance imaging confirmed a type I Chiari malformation and a cervical syrinx. The patient was treated with three cervical epidural injections, after which her symptoms exacerbated. Consequently, a posterior fossa suboccipital craniectomy with C1 laminectomy and excision of extradural and intradural adhesions was performed. After surgical intervention, notable neurologic improvements were observed. Given the marked worsening of symptoms, the present report suggests that interventional spinal procedures may be a contraindication in the presence of a Chiari malformation with a syrinx. PMID:24743463

Smith, Jason A; Cuccurullo, Sara J; Protzman, Nicole M; Kooch, Jason E

2014-08-01

31

Chiari I malformation, caudal regression syndrome, and Pierre Robin Syndrome: a previously unreported combination  

Microsoft Academic Search

Case   The authors report a child born with a constellation of findings including Chiari I malformation, Pierre Robin syndrome, and caudal regression syndrome. In addition, this child was found to have a fatty-infiltrated filum terminale and a low conus medullaris.Discussion and conclusion  In isolation, the Chiari I malformation has been reported in patients with caudal regression and Pierre Robin syndrome. Speculation

R. Shane Tubbs; W. Jerry Oakes

2006-01-01

32

International survey on the management of Chiari I malformation and syringomyelia  

Microsoft Academic Search

IntroductionThe availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken.MethodA questionnaire on the expected

Edgardo Schijman; Paul Steinbok

2004-01-01

33

Chiari malformation: CSF flow dynamics in the craniocervical junction and syrinx  

Microsoft Academic Search

Summary  Background. A CSF flow study in patients with Chiari malformation (ChM) who undergo craniocervical junction decompression (CCJD).\\u000a \\u000a Methods. Using spatial modulation of magnetization (SPAMM), cerebrospinal fluid (CSF) flow velocities were measured at the prepontine\\u000a (PP), anterior cervical (AC), and posterior cervical (PC) subarachnoid spaces (SAS) in healthy subjects (n = 11) and patients\\u000a with Chiari malformation (ChM) before and\\/or after

D. E. Sakas; S. I. Korfias; S. C. Wayte; D. J. Beale; K. P. Papapetrou; G. S. Stranjalis; K. W. Whittaker; H. L. Whitwell

2005-01-01

34

The Cerebellum in Children with Spina Bifida and Chiari II Malformation: Quantitative Volumetrics by Region  

Microsoft Academic Search

Few volumetric MRI studies of the entire cerebellum have been published; even less quantitative information is available in\\u000a patients with hindbrain malformations, including the Chiari II malformation which is ubiquitous in patients with spina bifida\\u000a meningomyelocele (SBM). In the present study, regional volumetric analyses of the cerebellum were conducted in children with\\u000a SBM\\/Chiari II and typically developing (TD) children. Total

Jenifer Juranek; Maureen Dennis; Paul T. Cirino; Lyla El-Messidi; Jack M. Fletcher

2010-01-01

35

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. PMID:22811732

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

2012-01-01

36

Pseudotumor cerebri: as a cause for early deterioration after Chiari I malformation surgery  

Microsoft Academic Search

Introduction  Chiari I malformation is associated with a small posterior fossa which cannot accommodate a growing hindbrain. Pseudotumor\\u000a cerebri has been linked to developmental posterior fossa malformations.\\u000a \\u000a \\u000a \\u000a Case reports  The authors present two cases of early-deterioration post-Chiari I malformation surgery in a young and an adult patient, which\\u000a were linked to raised intracranial pressure and had a stormy post-operative period. The pathophysiology

Sunil V. Furtado; K. Visvanathan; Kalyan Reddy; A. S. Hegde

2009-01-01

37

Intradiploic occipital pseudomeningocele in a patient with remote history of surgical treatment of Chiari malformation.  

PubMed

An intradiploic CSF pseudocyst is a rare entity that has been described in association with trauma, as a sequela of untreated hydrocephalus, or occasionally as a congenital finding in older adults. The authors present the case of a woman with a remote history of a posterior fossa intradural procedure, in which she underwent Chiari malformation decompression, Silastic substitute-assisted duraplasty, and occipitocervical fusion; she presented 19 years later with recurrent symptoms of Chiari malformation. She was found to have an occipital intradiploic pseudomeningocele, arising within her dorsal occipitocervical fusion mass and resulting in dorsal hindbrain compression. She underwent a posterior fossa decompression and revision of her failed duraplasty, and she had a good recovery. This case demonstrates intradiploic CSF pseudomeningocele as a rare potential delayed complication of an intradural procedure for the treatment of Chiari malformation with occipitocervical fusion. PMID:25147975

Mahaney, Kelly B; Menezes, Arnold H

2014-11-01

38

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

PubMed Central

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

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

2014-01-01

39

Correlation of hindbrain CSF flow and outcome after surgical decompression for Chiari I malformation  

Microsoft Academic Search

Objective  Many patients with symptomatic Chiari I malformation experience symptom recurrence after surgical decompression. Improved\\u000a radiographic predictors of outcome are needed to better select patients most likely to benefit from surgical intervention.\\u000a We examined whether ventral or dorsal cerebrospinal fluid (CSF) flow dynamics assessed by cine phase-contrast MRI scans could\\u000a predict response to posterior fossa decompression for Chiari I malformation.\\u000a \\u000a \\u000a \\u000a Methods  Forty-four

Matthew J. McGirt; April Atiba; Frank J. Attenello; Bruce A. Wasserman; Ghazala Datoo; Muraya Gathinji; Benjamin Carson; John D. Weingart; George I. Jallo

2008-01-01

40

Relevance of surgical strategies for the management of pediatric Chiari type I malformation  

Microsoft Academic Search

Objective  In face of continuing controversy to the optimal treatment of Chiari type I malformation, the authors analyzed three different\\u000a surgical strategies.\\u000a \\u000a \\u000a \\u000a Materials and methods  Sixty patients (30 boys and 30 girls, mean age 8.2 years) presented with clinical Chiari malformation. Additional syringomyelia\\u000a was present in 24 children, whereas cine flow studies showed no flow in the craniocervical junction in 48 children. All

Marcelo Galarza; Sandeep Sood; Steven Ham

2007-01-01

41

Cervical spine instability following cervical laminectomies for Chiari II malformation: a retrospective cohort study  

Microsoft Academic Search

Objective  The treatment of symptomatic Chiari II malformations typically involves multilevel cervical laminectomies in very young children.\\u000a These patients are at significant risk of cervical instability. The purpose of this study was to determine the incidence and\\u000a significance of cervical instability after multilevel cervical laminectomies in a cohort of patients decompressed for Chiari\\u000a II malformation.\\u000a \\u000a \\u000a \\u000a Methods  Postoperative dynamic lateral cervical spine radiographs

Fred C. Lam; Beverly J. Irwin; Kenneth J. Poskitt; Paul Steinbok

2009-01-01

42

Pathogenesis of cerebellar deformity in experimental Chiari type I malformation caused by mumps virus  

Microsoft Academic Search

We sought to elucidate the pathogenesis of Chiari type I malformation using an experimental model of hydrocephalus produced by inoculating hamsters with mumps virus. Dilatation of the lateral ventricles was detected in all brains inoculated at 2, 10, and 25 days of age. The cerebellum in hamsters inoculated at 2 and 10 days of age showed elongation and flattening of

Tomoyuki Takano; Masaaki Uno; Tsunekazu Yamano; Morimi Shimada

1994-01-01

43

Posterior cranial fossa morphometry in symptomatic adult Chiari I malformation patients: Comparative clinical and anatomical study  

Microsoft Academic Search

Background and objectivesThe cerebellar tonsillar herniation occurring in Chiari malformation Type I (CMI) mainly results from overcrowding of a normally developing hindbrain within a primary small posterior cranial fossa (PCF) due to an anomaly in the embryological development of the occipital bone. In the present study, the lengths of PCF parameters were studied in adult CMI patients.

Ahmet Dagtekin; Emel Avci; Engin Kara; Deniz Uzmansel; Oykut Dagtekin; Aslihan Koseoglu; Derya Talas; Celal Bagdatoglu

2011-01-01

44

Posterior fossa morphometry in symptomatic pediatric and adult Chiari I malformation  

Microsoft Academic Search

The linear dimensions and volume of the posterior fossa, and the length of the supra-occiput and the clivus in children with Chiari I malformations (CMI) were studied. A statistical relationship between patient demographics, radiological features, posterior fossa and occipital bone morphometry in the study and control groups was investigated. The results of 21 pediatric patients was compared to those of

Sunil V. Furtado; Kalyan Reddy; A. S. Hegde

2009-01-01

45

Acquired Chiari I Malformation and Syringomyelia after Valveless Lumboperitoneal Shunt in Infancy  

Microsoft Academic Search

The authors present the case of a 13-year-old boy who experienced frequent disabling episodes of typical postural induced headaches, which revealed a Chiari I malformation, syringomyelia and cervical spinal cord edema. This boy had received a valveless lumboperitoneal (LP) shunt at the age of 18 months for macrocephaly. At this time, cranial CT scan showed moderate enlargement of the subarachnoid

Laurent Riffaud; Carolina Moughty; Pierre-Louis Henaux; Claire Haegelen; Xavier Morandi

2008-01-01

46

Chiari I Malformation Presenting as Downbeat Nystagmus: Clinical Presentation, Diagnosis, and Management  

Microsoft Academic Search

Introduction Chiari I malformation is a congenital, neurological condition that is characterized by defects of the skull base resulting in herniation of the cerebellum through the foramen magnum into the cervical spinal canal. Because the condition can result in visual symptoms, patients will often search for answers from their eye care providers. -Case Report A 28-year-old Hispanic diabetic male with

Denise Goodwin; Ami R. Halvorson

47

Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations  

Microsoft Academic Search

Laryngeal respiratory obstruction associated with Chiari malformations was first described in 1932. We studied this type of obstruction in six children with one or several disorders pointing to brainstem dysfunction (failure to thrive, velopharyngeal incompetence, gastroesophageal reflux, or vagal hypertonia). The nature of the laryngeal obstruction was highly variable (vocal cord paralysis, paradoxical vocal cord motion, laryngomalacia) as were the

F. Portier; R. Marianowski; M. P. Morisseau-Durand; M. Zerah; Y. Manac'h

2001-01-01

48

Treatment and management of the Chiari II malformation: an evidence-based review of the literature  

Microsoft Academic Search

Objective Multiple surgical strategies exist for the management of the symptomatic Chiari II malformation. To date, no comprehensive analysis of this medical literature in an attempt to seek out standards or guidelines has been performed, thus serving as the impetus for this present review. Methods A computerized search of the database of the National Library of Medicine was performed on

R. Shane Tubbs; W. Jerry Oakes

2004-01-01

49

Toward a Simpler Surgical Management of Chiari I Malformation in a Pediatric Population  

Microsoft Academic Search

A wide variety of surgical adjuvants to the standard bony decompression have been advocated in the treatment of the Chiari I malformation, especially when the tonsillar herniation is associated with hydrosyringomyelia. These include various shunting procedures, duroplasty, obex plugging, and resection of the cerebellar tonsils. Our practice has been to avoid these adjuvants and to perform a simple limited occipital

Mark D. Krieger; J. Gordon McComb; Michael L. Levy

1999-01-01

50

Estimation of Posterior Fossa Volume in Pediatric Patients with Chiari Malformations by the Cavalieri Principle  

Microsoft Academic Search

Chiari malformations (CMs) involve pathological hindbrain abnormalities reported to be correlated with a hypoplastic posterior fossa. This study calculates posterior fossa volume estimate using MRI. A retrospective review was conducted on patients who were admitted to our hospital. Clinical and radiological assessments were performed. Posterior fossa volumes (PFV) and intracranial volumes (ICV) were measured from head MRI scans using the

Chunquan Cai; Qingjiang Zhang; Changhong Shen; Weidong Yang; Ouyan Shi

2008-01-01

51

Treatment of the Chiari malformation with bone decompression without durotomy in children and young adults  

Microsoft Academic Search

Objective. The purpose of this study was to provide more information on alternative operative approaches to the treatment of symptomatic Chiari type I and\\/or type II malformations in children and young adults. Methods. All patients were treated with craniocervical decompression without durotomy or duroplasty. The cervical decompression was carried on to the lowest level of tonsillar herniation. Conclusions. In 22

Hector E. James; Adam Brant

2002-01-01

52

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

53

A Novel Endoscopic Technique to Suboccipital Decompression and Atlas Laminectomy for Chiari Malformation Type I: Technical Note  

Microsoft Academic Search

Objective: In order to make Chiari decompression simple and less invasive while using direct visualization, a noval solely endoscopic procedure has been employed for the decompression of Chiari malformation type 1. Methods: Zero degree and 30°-angled lens endoscope have been used for the resections of occiput and C1 lamina, and to maximally widen the rim of craniovertebral junction (CVJ) bilaterally

Xiao Di; Mark Luciano

54

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

PubMed Central

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

2009-01-01

55

Gowers' intrasyringeal hemorrhage associated with Chiari type I malformation in Noonan syndrome  

PubMed Central

Background: Idiopathic hemorrhage in a syrinx is a rare entity known as Gowers’ intrasyringeal hemorrhage. Bleeding confined to the syrinx cavity causes severe, sometimes acute, neurological deficits. We report a case of intrasyringeal hemorrhage into a preexisting lumbosacral syrinx associated with Chiari type I malformation. Case Description: A 39-year-old female with Noonan syndrome underwent foramen magnum decompression and a cervical syrinx-subarachnoid shunt for Chiari type I malformation-associated syringomyelia 7 years ago. She presented progressive gait deterioration and acute urinary dysfunction, indicating conus medullaris syndrome. Initial magnetic resonance imaging revealed massive hemorrhage in the intrasyringeal cavity of the conus medullaris. The patient underwent surgical removal of the intrasyringeal hematoma and her neurological symptoms improved postoperatively. Conclusion: Although Gowers’ intrasyringeal hemorrhage is rare, this entity should be taken into consideration in patients with syringomyelia showing acute neurological deterioration. PMID:24575321

Mitsuhara, Takafumi; Yamaguchi, Satoshi; Takeda, Masaaki; Kurisu, Kaoru

2014-01-01

56

[A case of congenital hypomyelination neuropathy with type I Chiari malformation and mental retardation].  

PubMed

A four-year old boy with congenital hypomyelination neuropathy (CHMN) had mental retardation and a tic disorder, the latter commencing at 2 years of age. The diagnosis of CHMN was confirmed by electron microscopy of his biopsied sural nerve which showed loss of large myelinated fibers, decreased density of myelinated fibers, many naked axons and atypical onion bulb formation. MR imaging showed type I Chiari malformation in the absence of clinical signs attributable to it. Auditory brainstem response (ABR) showed delayed wave I peak latency, prolonged I--III interpeak latency, broad wave II duration and normal III--V interpeak latency, suggesting abnormal peripheral and normal central myelination. The association of CHMN with mental retardation. Chiari malformation and tic disorder has never been reported, the significance of which remained unclear. PMID:9294313

Saito, Y; Sugai, K; Sasaki, M; Hirano, S; Hanaoka, S; Hashimoto, T; Nonaka, I; Nagai, T

1997-09-01

57

Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformations  

Microsoft Academic Search

Introduction  Chiari I malformations (CMI) involve pathological hindbrain abnormalities reported to be correlated with a hypoplastic posterior\\u000a fossa. CMI was traditionally characterized by the downward herniation of the cerebellar tonsils with a descent of 5 mm or\\u000a more below the foramen magnum. The fullness of the cisterna magna and CSF flow at the level of the cervicomedullary junction\\u000a have been shown to

T. Trigylidas; B. Baronia; M. Vassilyadi; E. C. G. Ventureyra

2008-01-01

58

Decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation  

Microsoft Academic Search

Study design: Review and analysis of seven cases of syringomyelia treated surgically.Objective: To demonstrate the beneficial role of decompressive surgery for the altered cerebrospinal fluid (CSF) flow dynamics in syringomyelia not associated with Chiari I malformation. A comparison between the pre- and post-operative syrinx size and CSF flow in the subarachnoid space was made using cine-mode magnetic resonance imaging (cine-MRI)

J-H Lee; C-K Chung; HJ Kim

2002-01-01

59

What Differences Exist in the Appropriate Treatment of Congenital Versus Acquired Adult Chiari Type I Malformation?  

Microsoft Academic Search

Chiari type I malformation is found in 1 out of 20 magnetic resonance imaging (MRI) studies. Isolated tonsillar herniation\\u000a is of limited utility and should be considered within the clinical context because these patients can be asymptomatic. Cine\\u000a MRI showing compression of the cerebrospinal fluid (CSF) spaces in the foramen magnum area is a crucial technique for making\\u000a treatment decisions.

César Ramón; Andrés Gonzáles-Mandly; Julio Pascual

2011-01-01

60

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

61

Concomitant achondroplasia and Chiari II malformation: A double-hit at the cervicomedullary junction  

PubMed Central

We report the first case of a neonate with concurrent Chiari II malformation and achondroplasia. Although rare, both these conditions contribute to several deleterious anatomical changes at the cervicomedullary junction and thus predispose to acute hydrocephalus. Although our patient was initially asymptomatic, hydrocephalus ensued several weeks after birth and required cerebral spinal fluid diversion. We discuss the potential links between the two conditions, the pathophysiology, and the important clinical implications for the management of the increased risk of hydrocephalus.

Awad, Al-Wala; Aleck, Kyrieckos A; Bhardwaj, Ratan D

2014-01-01

62

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

PubMed Central

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

2014-01-01

63

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. PMID:20440631

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

2010-01-01

64

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

65

Treatment of failed Adult Chiari Malformation decompression with CSF drainage: observations in six patients  

Microsoft Academic Search

Summary.  \\u000a ?Objective: We report the use of CSF drainage for the management of failed Adult Chiari Malformation (ACM) decompression.\\u000a \\u000a \\u000a \\u000a ?Methods: All patients with more than one year follow-up after treatment of their failed ACM were included in this study. They underwent\\u000a initial decompression between September 1998 and April 2000. Clinical and radiological data were collected initially and at\\u000a recurrence. Lumbar

G. K. Bejjani; K. P. Cockerham; W. E. Rothfus; J. C. Maroon; M. Maddock

2003-01-01

66

Craniosynostosis, hydrocephalus, Chiari I malformation and radioulnar synostosis: probably a new syndrome.  

PubMed

We report on clinical and molecular findings of two brothers that both presented with sagittal craniosynostosis, hydrocephalus, Chiari I malformation, blepharophimosis, small low-set ears, hypoplastic philtrum, radioulnar synostosis, kidney malformation, and hypogenitalism. Their father presented mild brachydactyly. Conventional cytogenetic and array CGH screening did not show any chromosomal gains or losses. Furthermore, molecular genetic screening of genes involved in different craniosynostosis syndromes, namely FGFR1, FGFR2, FGFR3, TWIST, RECQL4, and POR genes failed to detect any mutations in genomic DNA. The unique range of clinical manifestations in these two patients and the negative findings of the molecular genetic screening suggest the hypothesis of a previously unrecognized syndrome. PMID:19022412

Capra, Valeria; De Marco, Patrizia; Merello, Elisa; Baffico, Ave Maria; Baldi, Maurizia; Divizia, Maria Teresa; Gimelli, Stefania; Mallet, Delphine; Raso, Alessandro; Mascelli, Samantha; Tomà, Paolo; Rossi, Andrea; Pavanello, Marco; Cama, Armando; Magnani, Cinzia

2009-01-01

67

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. PMID:22629166

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

2012-01-01

68

Postoperative epidural hematoma contributes to delayed upper cord tethering after decompression of Chiari malformation type I  

PubMed Central

Background: Symptomatic arachnoiditis after posterior fossa surgical procedures such as decompression of Chiari malformation is a possible complication. Clinical presentation is generally insidious and delayed by months or years. It causes disturbances in the normal flow of cerebrospinal fluid and enlargement of a syrinx cavity in the upper spinal cord. Surgical de-tethering has favorable results with progressive collapse of the syrinx and relief of the associated symptoms. Case Description: A 30-year-old male with Chiari malformation type I was treated by performing posterior fossa bone decompression, dura opening and closure with a suturable bovine pericardium dural graft. Postoperative period was uneventful until the fifth day in which the patient suffered intense headache and progressive loose of consciousness caused by an acute posterior fossa epidural hematoma. It was quickly removed with complete clinical recovering. One year later, the patient experienced progressive worsened of his symptoms. Upper spinal cord tethering was diagnosed and a new surgery for debridement was required. Conclusions: The epidural hematoma compressing the dural graft against the neural structures contributes to the upper spinal cord tethering and represents a nondescribed cause of postoperative fibrosis, adhesion formation, and subsequent recurrent hindbrain compression.

Lopez-Gonzalez, Antonio; Plaza, Estela; Marquez-Rivas, Francisco Javier

2014-01-01

69

Amelioration of Chiari type 1 malformation and syringomyelia following posterior calvarial distraction in Crouzon's syndrome--a case report.  

PubMed

Multisutural craniosynostosis as seen in Crouzon's syndrome can result in raised intracranial pressure, Chiari malformation (CM) and syringomyelia. Posterior calvarial distraction (PD) is a technique for addressing cranio-cephalic disproportion, and this case report describes the reversal of both CM and syrinx in a 6-year-old child who underwent PD initially for raise intracranial pressure. PMID:23780407

Ahmad, Fateh; Evans, Martin; White, Nicholas; Nishikawa, Hiroshi; Dover, Stephen; Solanki, Guirish; Rodrigues, Desiderio

2014-01-01

70

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. PMID:18405364

Williams, Helen

2008-01-01

71

Acquired Chiari malformation Type I associated with a supratentorial arteriovenous malformation. Case report and review of the literature.  

PubMed

Chiari malformation Type I (CM-I), a condition defined by caudal descent of the cerebellar tonsils through the foramen magnum, is generally considered a congenital lesion. Several authors, however, have described an acquired form that appears identical to the congenital lesion on neuroimages. The most commonly reported cause of an acquired CM-I is cerebrospinal fluid diversion through a lumboperitoneal shunt. In this paper, the authors report the case of a patient in whom an acquired CM-I developed in association with a supratentorial arteriovenous malformation (AVM) of the brain. Development of the acquired CM was documented on serial magnetic resonance images. Moreover, the CM was seen to originate and worsen in concert with the clinicoradiological progression of the AVM. The underlying mechanism responsible for the acquired CM in this case is thought to be a high-flow venopathy of the transverse and sigmoid sinuses causing occlusion on the right and redirection of venous outflow into posterior fossa veins, with consequent venous congestion and swelling of the posterior fossa structures. PMID:16509477

O'Shaughnessy, Brian A; Bendok, Bernard R; Parkinson, Richard J; Shaibani, Ali; Walker, Matthew T; Shakir, Ebrahim; Batjer, H Hunt

2006-01-01

72

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

73

Atypical cerebellar slump syndrome and external hydrocephalus following craniocervical decompression for Chiari I malformation: case report.  

PubMed

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

Thakar, Sumit; Dadlani, Ravi; Tawari, Manish; Hegde, Alangar S

2014-01-01

74

Intracranial hypotension syndrome in a patient due to suboccipital craniectomy secondary to Chiari type malformation.  

PubMed

Intracranial hypotension syndrome (IHS) is a rare disorder characterized by a decrease in cerebrospinal fluid pressure to less than 60 mm H2O. The syndrome is associated with occipital headache radiating to the frontal and temporal zones. The current clinical case describes the manifestation of IHS in a twenty-five year old female with a history of suboccipital craniectomy due to Chiari I malformation nine years earlier. The patient was admitted to the hospital complaining about postural, mainly occipital, headache during the last three months, aggravated by being in an upright position. The magnetic resonance imaging (MRI) revealed engorgement of the dural venous sinuses, significant enlargement of the pituitary gland and download displacement or sagging of the brain with effacement of the perichiasmatic cisterns and the prepontine cistern, while the spinal T2W MRI revealed a 7 mm × 2.5 mm dural defect with an extradural cerebrospinal fluid collection at the dorsal soft tissues of the cervical spine. The previous imaging did not reveal subdural effusions. PMID:24364025

Dora, Barkoula; Nikolaos, Bontozoglou; Stylianos, Gatzonis; Damianos, Sakas

2013-12-16

75

Computational fluid dynamics modelling of cerebrospinal fluid pressure in Chiari malformation and syringomyelia.  

PubMed

The pathogenesis of syringomyelia in association with Chiari malformation (CM) is unclear. Studies of patients with CM have shown alterations in the CSF velocity profile and these could contribute to syrinx development or enlargement. Few studies have considered the fluid mechanics of CM patients with and without syringomyelia separately. Three subject-specific CFD models were developed for a normal participant, a CM patient with syringomyelia and a CM patient without syringomyelia. Model geometries, CSF flow rate data and CSF velocity validation data were collected from MRI scans of the 3 subjects. The predicted peak CSF pressure was compared for the 3 models. An extension of the study performed geometry and flow substitution to investigate the relative effects of anatomy and CSF flow profile on resulting spinal CSF pressure. Based on 50 monitoring locations for each of the models, the CM models had significantly higher magnitude (p<0.01) peak CSF pressure compared with normal. When using the same CSF input flow waveform, changing the upper spinal geometry changed the magnitude of the CSF pressure gradient, and when using the same upper spinal geometry, changing the input flow waveform changed the timing of the peak pressure. This study may assist in understanding syringomyelia mechanisms and relative effects of CSF velocity profile and spinal geometry on CSF pressure. PMID:23769174

Clarke, Elizabeth C; Fletcher, David F; Stoodley, Marcus A; Bilston, Lynne E

2013-07-26

76

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. PMID:22978540

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

2013-01-01

77

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

PubMed

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

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

2008-09-01

78

Chiari malformation type I: a case-control association study of 58 developmental genes.  

PubMed

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. PMID:23437350

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

2013-01-01

79

Sudden unexpected nocturnal death in Chiari type 1 malformation and potential role of opioid analgesics  

PubMed Central

Background: Chiari malformation type 1 (CM1) is a common congenital anomaly of the craniocervical junction. CM1 is reported to run a usually benign course and patients typically experience no symptoms or chronic, slowly progressive symptoms. However, recent reports indicate that a subset of patients with CM1 may present with acute deterioration and sudden unexpected death (SUD). We report a case of SUD during sleep in a young man with CM1, which we believe was related to the administration of common and therapeutic doses of narcotic analgesics for the management of pain. We will clarify the pathophysiology of acute deterioration and SUD in CM1 and the possibility that the adverse effects of opiate analgesics likely were the leading cause of death in our patient. Case Description: In this review, we present a 29-year-old male with worsening headache secondary to previously diagnosed CM1. The patient died suddenly and unexpectedly after administration of common and therapeutic doses of narcotic analgesics for the management of pain. Conclusion: The mechanism(s) of acute neurological deterioration and sudden death in patients with CM1 remains poorly understood. We believe the rapid fatal deterioration in our patient following administration of opioids suggests that this category of medication may cause sudden unexpected “neurogenic” cardiac death in CM1 patients by inducing sleep-related breathing difficulties and associated hypercapnia. Hypercapnia by further increasing intracranial pressure can result in a sudden pressure-induced decompensation of the cardiopulmonary control centers in the brain stem and cause instantaneous cardiorespiratory arrest. PMID:24778905

Roohi, Fereydoon; Gropen, Toby; Kula, Roger W.

2014-01-01

80

Task-Specific and General Cognitive Effects in Chiari Malformation Type I  

PubMed Central

Objective Our objective was to use episodic memory and executive function tests to determine whether or not Chiari Malformation Type I (CM) patients experience cognitive dysfunction. Background CM is a neurological syndrome in which the cerebellum descends into the cervical spine causing neural compression, severe headaches, neck pain, and number of other physical symptoms. While primarily a disorder of the cervico-medullary junction, both clinicians and researchers have suspected deficits in higher-level cognitive function. Design and Methods We tested 24 CM patients who had undergone decompression neurosurgery and 24 age- and education-matched controls on measures of immediate and delayed episodic memory, as well as three measures of executive function. Results The CM group showed performance decrements relative to the controls in response inhibition (Stroop interference), working memory computational speed (Ospan), and processing speed (automated digit symbol substitution task), but group differences in recall did not reach statistical significance. After statistical control for depression and anxiety scores, the group effects for working memory and processing speed were eliminated, but not for response inhibition. This response inhibition difference was not due to overall general slowing for the CM group, either, because when controls' data were transformed using the linear function fit to all of the reaction time tasks, the interaction with group remained statistically significant. Furthermore, there was a multivariate group effect for all of the response time measures and immediate and delayed recall after statistical control of depression and anxiety scores. Conclusion These results suggest that CM patients with decompression surgery exhibit cognitive dysfunction compared to age- and education-matched controls. While some of these results may be related to anxiety and depression (likely proxies for chronic pain), response inhibition effects, in particular, as well as a general cognitive deficit persisted even after control for anxiety and decompression. PMID:24736676

Allen, Philip A.; Houston, James R.; Pollock, Joshua W.; Buzzelli, Christopher; Li, Xuan; Harrington, A. Katherine; Martin, Bryn A.; Loth, Francis; Lien, Mei-Ching; Maleki, Jahangir; Luciano, Mark G.

2014-01-01

81

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

82

Chiari type 1 malformation in an infant with type 2 Pfeiffer syndrome: further evidence of acquired pathogenesis.  

PubMed

There seems to be an association between type 1 Chiari malformation (CM) and some congenital craniosynostosis syndromes. Type 2 Pfeiffer syndrome is a condition associated with premature fusion of multiple cranial sutures, cloverleaf skull (kleeblatschädel deformity), prominent ptosis, thumb and first toe abnormalities, variable syndactyly, and mutated genes for type 1 or 2 fibroblast growth factor receptor. These children generally do poorly because of significant often severe neurologic and cognitive defects, and many die very young. Roughly half of all patients with Pfeiffer syndrome, and virtually all with type 2 disease, also have type 1 CM. Chiari malformation may not be congenital but acquired as a consequence of the skull deformities and other associated intracranial factors in patients with craniosynostosis. We report a term male infant with type 2 Pfeiffer syndrome, who was not noted to have any CM on initial brain imaging done at 2 months but in whom repeated imaging demonstrated clear evidence of CM by 4 months, despite reconstructive craniotomies and unilateral ventriculoperitoneal shunt insertion. Posterior fossa decompression yielded a good result. This patient provides further evidence to support the concept of acquired tonsillar herniation in patients with craniosynostosis syndromes. The etiology seems multifactorial and related to (1) the disproportionately slow growth of the skull relative to the brain, particularly in the posterior fossa, secondary to early fusion of skull sutures, in turn secondary to congenital deficiencies in fibroblast growth factor receptors; (2) impaired venous sinus drainage; (3) hydrocephalus; and (4) resultant elevations in intracranial pressure. PMID:20186072

Ranger, Adrianna; Al-Hayek, Ali; Matic, Damir

2010-03-01

83

Current Opinions for the Treatment of Syringomyelia and Chiari Malformations: Survey of the Pediatric Section of the American Association of Neurological Surgeons  

Microsoft Academic Search

Introduction: A variety of surgical interventions have been recommended for patients with syringomyelia and Chiari malformation. To obtain a better understanding of currently used management strategies and surgical interventions, this study evaluates current opinions in the treatment of these entities. Methods: Members of the Pediatric Section of the American Association of Neurological Surgeons (AANS) were surveyed in July of 1998.

Raymond I. Haroun; Michael Guarnieri; Jeffery J. Meadow; Michael Kraut; Benjamin S. Carson

2000-01-01

84

Endovascular Treatment of a Ruptured Giant Serpentine Aneurysm of the Superior Cerebellar Artery in a Patient with a Chiari II Malformation  

Microsoft Academic Search

Summary: We present a case of a ruptured giant serpentine aneurysm (GSA) of the superior cerebellar artery in a patient with a Chiari II malformation. The fusiform aneu- rysm was successfully treated with endovascular parent artery occlusion of the GSA by using detachable coils. Giant serpentine aneurysms (GSAs) are rare, fusi- form aneurysms greater than 2.5 cm, which have a

Jason Zicherman; Sudipta Roychowdhury; James K. Demarco; Scott Shepard; Steven Schonfeld; Irwin Keller; Scott Schlesinger

85

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

2013-06-01

86

Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations  

PubMed Central

Object Although Chiari Type I (CM-I) and Type 0 (CM-0) malformations have been previously characterized clinically and radiologically, there have been no studies focusing on the possible genetic link between these disorders. The goal of this study was to identify families in whom CM-0 and CM-I co-occurred and to further assess the similarities between these disorders. Methods Families were ascertained through a proband with CM-I. Detailed family histories were obtained to identify first-degree relatives diagnosed with CM-0. Several criteria were used to exclude individuals with acquired forms of CM-I and/or syringomyelia. Individuals were excluded with syndromic, traumatic, infectious, or tumor-related syringomyelia, as well as CM-I due to a supratentorial mass, hydrocephalus, history of cervical or cranial surgery unrelated to CM-I, or development of symptoms following placement of a lumbar shunt. Medical records and MR images were used to characterize CM-I and CM-0 individuals clinically and radiologically. Results Five families were identified in which the CM-I proband had a first-degree relative with CM-0. Further assessment of affected individuals showed similar clinical and radiological features between CM-0 and CM-I individuals, although CM-I patients in general had more severe symptoms and skull base abnormalities than their CM-0 relatives. Overall, both groups showed improvement in symptoms and/or syrinx size following craniocervical decompression surgery. Conclusions There is accumulating evidence suggesting that CM-0 and CM-I may be caused by a common underlying developmental mechanism. The data in this study are consistent with this hypothesis, showing similar clinical and radiological features between CM-0 and CM-I individuals, as well as the occurrence of both disorders within families. Familial clustering of CM-0 and CM-I suggests that these disorders may share an underlying genetic basis, although additional epigenetic and/or environmental factors are likely to play an important role in the development of CM-0 versus CM-I. PMID:22462700

Markunas, Christina A.; Tubbs, R. Shane; Moftakhar, Roham; Ashley-Koch, Allison E.; Gregory, Simon G.; Oakes, W. Jerry; Speer, Marcy C.; Iskandar, Bermans J.

2012-01-01

87

Dimensions of the posterior fossa in patients symptomatic for Chiari I malformation but without cerebellar tonsillar descent  

PubMed Central

Background Chiari I malformation (CMI) is diagnosed by rigid radiographic criteria along with appropriate clinical symptomatology. The aim of this study was to investigate the dimensions of the posterior cranial fossa in patients without significant tonsillar descent but with symptoms comparable to CMI. Methods Twenty-two patients with signs and symptoms comparable to CMI but without accepted radiographic criteria of tonsillar descent > 3–5 mm were referred to our clinic for evaluation. A history and physical examination were performed on all patients. In reviewing their MRI scans, nine morphometric measurements were recorded. The measurements were compared to measurements from a cohort of twenty-five individuals with cranial neuralgias from our practice. Results For patients with Chiari-like symptomatology, the following statistically significant abnormalities were identified: reduced length of the clivus, reduced length of basisphenoid, reduced length of basiocciput, and increased angle of the tentorium. Multiple morphometric studies have demonstrated similar findings in CMI. Conclusion The current classification of CMI is likely too restrictive. Preliminary morphologic data suggests that a subgroup of patients exists with tonsillar descent less than 3 mm below the foramen magnum but with congenitally hypoplastic posterior fossa causing symptomatology consistent with CMI. PMID:16359556

Sekula, Raymond F; Jannetta, Peter J; Casey, Kenneth F; Marchan, Edward M; Sekula, L Kathleen; McCrady, Christine S

2005-01-01

88

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

PubMed Central

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

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

2014-01-01

89

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

90

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. PMID:23136935

2012-01-01

91

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. PMID:24130704

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

2013-01-01

92

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

PubMed Central

Object Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I–associated syringomyelia [AQ? Edit okay? If not, please advise. JG: edit correct]. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics for additional risk factors for scoliosis. Methods The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis. Results Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63–40.57, p < 0.001) and moderate (5–12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3–25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location. Conclusions The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting with larger maximum syrinx diameters (> 6 mm) have an increased risk of scoliosis. PMID:24527859

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

2014-01-01

93

Rate of Chiari I Malformation in Children of Mothers with Depression with and without Prenatal SSRI Exposure.  

PubMed

Selective serotonin reuptake inhibitors (SSRIs) are frequently prescribed to pregnant women. Therefore, research on in utero exposure to SSRIs can be helpful in informing patients and clinicians. The aim of this retrospective two-cohort study was to determine whether there is a statistically significant increase in Chiari I malformations (CIM) in children exposed to SSRIs during pregnancy. A total of 33 children whose mothers received a diagnosis of depression and took SSRIs during pregnancy (SSRI-exposed cohort) were matched to 66 children with no history of maternal depression and no SSRI exposure. In addition, 30 children whose mothers received a diagnosis of depression, but did not receive antidepressants during pregnancy (history of maternal depression cohort), were matched to 60 children with no history of maternal depression and no SSRI exposure. Main outcome was presence/absence of CIM on MRI scans at 1 and/or 2 years of age. Scans were reviewed by two independent neuroradiologists who were blind to exposure status. The SSRI-exposed children were significantly more likely to be classified as CIM than comparison children with no history of maternal depression and no SSRI exposure (18% vs 2%, p=0.003, OR estimate 10.32, 95% Wald confidence limits 2.04-102.46). Duration of SSRI exposure, SSRI exposure at conception, and family history of depression increased the risk. The history of maternal depression cohort did not differ from comparison children with no history of maternal depression and no SSRI exposure in occurrence of CIM (7% vs 5%, p=0.75, OR estimate 1.44, 95% Wald confidence limits 0.23-7.85). Replication is needed, as is additional research to clarify whether SSRIs directly impact risk for CIM or whether this relationship is mediated by severity of depressive symptoms during pregnancy. We would discourage clinicians from altering their prescribing practices until such research is available. PMID:24837031

Knickmeyer, Rebecca C; Meltzer-Brody, Samantha; Woolson, Sandra; Hamer, Robert M; Smith, J Keith; Lury, Kenneth; Gilmore, John H

2014-10-01

94

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

2013-01-01

95

Effect of craniovertebral decompression on CSF dynamics in Chiari malformation Type I studied with computational fluid dynamics.  

PubMed

Object The effect of craniovertebral decompression surgery on CSF flow dynamics in patients with Chiari malformation Type I (CM-I) has been incompletely characterized. The authors used computational fluid dynamics to calculate the effect of decompression surgery on CSF flow dynamics in the posterior fossa and upper cervical spinal canal. Methods Oscillatory flow was simulated in idealized 3D models of the normal adult and the CM-I subarachnoid spaces (both previously described) and in 3 models of CM-I post-craniovertebral decompressions. The 3 postoperative models were created from the CM model by virtually modifying the CM model subarachnoid space to simulate surgical decompressions of different magnitudes. Velocities and pressures were computed with the Navier-Stokes equations in Star-CD for multiple cycles of CSF flow oscillating at 80 cycles/min. Pressure gradients and velocities were compared for 8 levels extending from the posterior fossa to the C3-4 level. Relative pressures and peak velocities were plotted by level from the posterior fossa to C3-4. The heterogeneity of flow velocity distribution around the spinal cord was compared between models. Results Peak systolic velocities were generally lower in the postoperative models than in the preoperative CM model. With the 2 larger surgical defects, peak systolic velocities were brought closer to normal model velocities (equal values at C-3 and C-4) than with the smallest surgical defect. For the smallest defect, peak velocities were decreased, but not to levels in the normal model. In the postoperative models, heterogeneity in flow velocity distribution around the spinal cord increased from normal model levels as the degree of decompression increased. Pressures in the 5 models differed in magnitude and in pattern. Pressure gradients along the spinal canal in the normal and CM models were nonlinear, with steeper gradients below C3-4 than above. The CM model had a steeper pressure gradient than the normal model above C3-4 and the same gradient below. The postoperative models had lower pressure gradients than the CM model above C2-3. The most conservative decompression had lower pressure gradients than the normal model above C2-3. The two larger decompression defects had CSF pressure gradients below those in the normal model above C2-3. These 2 models had a less steep gradient above C-3 and a steeper gradient below. Conclusions In computer simulations, craniovertebral surgical defects generally diminished CSF velocities and CSF pressures. PMID:25084032

Linge, Svein O; Mardal, Kent-A; Helgeland, Anders; Heiss, John D; Haughton, Victor

2014-10-01

96

Extra-arachnoidal cranio-cervical decompression for syringomyelia associated with Chiari I malformation in adults: technique assessment  

Microsoft Academic Search

Summary  \\u000a Background. The osteo-dural decompression of the cerebellar tonsils at the cranio-cervical junction is generally considered the most\\u000a effective treatment for syringomyelia-Chiari I complex. However much controversy concerning a great number of surgical adjuvants\\u000a to the standard bony decompression is still present. In this work an extra-arachnoidal cranio-cervical decompression (CCD)\\u000a without duroplasty is described and the surgical results are reported.

P. Perrini; N. Benedetto; R. Tenenbaum; N. Di Lorenzo

2007-01-01

97

Suboccipital decompression for Chiari I malformation: outcome comparison of duraplasty with expanded polytetrafluoroethylene dural substitute versus pericranial autograft  

Microsoft Academic Search

Objective  Treatment failure for Chiari decompression is frequently associated with scarring, intradural adhesions, and recurrent loss\\u000a of hindbrain space. While autograft has been our standard for hindbrain duraplasty, we investigated whether introducing anti-adhesive\\u000a synthetic GORE PRECLUDE® MVP® Dura Substitute (expanded polytetrafluoroethylene [ePTFE] graft) was associated with improved\\u000a patient outcomes.\\u000a \\u000a \\u000a \\u000a Materials and methods  We retrospectively reviewed records of patients undergoing first-time suboccipital decompression\\/duraplasty

Frank J. Attenello; Matthew J. McGirt; Giannina L. Garcés-Ambrossi; Kaisorn L. Chaichana; Benjamin Carson; George I. Jallo

2009-01-01

98

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

2010-11-01

99

Scoliosis in children with Chiari I-related syringomyelia  

Microsoft Academic Search

Objective  To study the relationship between scoliosis and Chiari I malformation, with reference to the possible role of cranio-vertebral decompression in preventing the need for scoliosis correction.Material and methods  Out of a total of 36 patients with symptomatic Chiari I, who underwent primary cranio-vertebral decompression by a single paediatric neurosurgeon with an interest in Chiari malformation between 1998 and 2003, 13 had

Ranjeev Bhangoo; Spyros Sgouros

2006-01-01

100

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

101

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. PMID:23620759

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

2013-01-01

102

The need in dural graft suturing in Chiari I malformation decompression: A prospective, single-blind, randomized trial comparing sutured and sutureless duraplasty materials  

PubMed Central

Background: This study compared the use of two commonly utilized dural closure techniques used in augmentation duraplasty for Chiari malformation I (CM I) and evaluated their efficacy and outcome in terms of quality of life assessments. Methods: This prospective randomized study compared sutureless (DuraGen) and suturable (Dura-Guard) techniques in CM I decompression. Clinical parameters, cost analysis, and SF-36 Quality of Life Questionnaire (QLQ) were utilized to assess outcome. Results: Thirty-four patients were enrolled. Average age was 38.7 ± 12.2 years (mean ± SD (Standard Deviation)) and 82% of patients were female. Sixteen patients received DuraGen and 18 Dura-Guard. Age and gender were similar among groups. Postoperative complications did not differ between groups. Operative cost and time were less for DuraGen, whereas hospital stay was less with Dura-Guard, neither was statistically significant. Average QLQ scores at months 1, 2, and 3 improved in both groups. Dura-Guard patients showed greater improvement in quality of life at month 2 (P < 0.05) but groups did not differ at final survey. All patient's physical health (P < 0.005) and function (P < 0.005) were significantly improved. Outcome did not differ between groups and all patients showed significant improvement (P < 0.05). Conclusion: Both techniques are effective in reaching the goals of decompressive surgery for CM I and did not differ in quality of life at final survey. All patients showed significant improvement in physical function, physical health, and outcome following surgery. With all variables being equal the choice of duraplasty material may be based upon surgeon's preference. PMID:23532455

Williams, Leena E.; Vannemreddy, Prasad S.; Watson, Karriem S.; Slavin, Konstantin V.

2013-01-01

103

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

104

Chiari 2 without spinal dysraphism: does it blow a hole in the pathogenesis?  

PubMed

Chiari malformation type 2 is characterized by hindbrain protrusion and a constellation of supratentorial malformations. Chiari malformation type 2 is thought to be causally related to myelomeningocele due to intrauterine cerebrospinal fluid overdrainage. This relationship is so strong that it has become a rule. A 14-year-old girl affected by mental retardation, spastic triparesis, and epilepsy is presented. Brain magnetic resonance imaging disclosed a severe Chiari malformation type 2, whereas spine magnetic resonance imaging was unremarkable. The authors discuss previous literature describing rare cases of Chiari malformation type 2 without open spinal dysraphism and the relevance of concomitant, sometimes overlooked, neuroimaging findings, underlying how exceptions might be hurdles but might also eventually strengthen the rules. PMID:22378677

Citton, Valentina; Toldo, Irene; Balao, Laura; Pettenazzo, Andrea; Emanuelli, Enzo; Sartori, Stefano; Calderone, Milena; Manara, Renzo

2012-04-01

105

Pediatric Chiari  

MedlinePLUS

... it. He contacted the pediatrician and asked him to write a referral for an MRI. The next day ... Malformation 1 and two syrinxes. I asked him to write everything down. I couldn’t concentrate to comprehend ...

106

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

PubMed Central

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

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

2011-01-01

107

Arnold Flames Resonance Surface Folds*  

E-print Network

Arnold Flames and Resonance Surface Folds* Richard P. McGehee School of Mathematics University parameter plane bifurcation diagrams are "(Arnold) resonance horns" emanating from zero forcing ampli- tude families by Arnold [1983] and Hall [1984] indi- cate the presence of (Arnold) resonance horns emanating

Peckham, Bruce B.

108

Arnold Hague's Chair  

USGS Multimedia Gallery

A wooden, collapsible chair from Arnold Hague's expedition. Made of sassafras wood with interchangeable canvas covers. This chair can be collapsed into a roll that is packed up and transported easily. Object ID: USGS-000031...

2009-07-22

109

Budd-Chiari Syndrome  

Microsoft Academic Search

\\u000a The Budd-Chiari syndrome (BCS) is an uncommon and life-threatening disorder defined as the obstruction of hepatic venous outflow\\u000a regardless of its causative mechanism or level of obstruction. The clinical presentation of BCS is highly variable and can\\u000a range from asymptomatic cases to ­fulminant hepatic failure with encephalopathy. In the vast majority of cases, it is possible\\u000a to identify an inherited

Susana Seijo-Ríos; Puneeta Tandon; Jaime Bosch; Juan Carlos García-Pagán

110

Chart Parsing Doug Arnold  

E-print Network

Chart Parsing Doug Arnold doug@essex.ac.uk 1 The Problems · Inefficiency of backtracking parsers baby PP with a telescope The use of a chart offers three advantages: · it avoids multiplication for `partial parses'. Moreover, (`active') chart parsing, that is, parsing where the chart itself drives

Lin, Dekang

111

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

112

Neurosurgical management of congenital malformations of the brain.  

PubMed

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

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

2011-08-01

113

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.

114

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. PMID:20890412

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

2010-01-01

115

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY .................................................................................... 33 3.3 BIOMASS POWER PLANT OPERATION MODELS AND DATA

116

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY and continuously between the earth's biomass and atmosphere. From a greenhouse gas perspective, forest treatments

117

Sutures - separated  

MedlinePLUS

Arnold-Chiari malformation Battered child syndrome Bleeding inside the brain (intraventricular hemorrhage) Brain tumor Certain vitamin deficiencies Dandy-Walker malformation Down syndrome Hydrocephalus Infections that are present at birth (congenital ...

118

Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression  

Microsoft Academic Search

Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum.\\u000a CMI has been shown to be closely associated with the development of syringomyelia (SM). Objects: Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development\\u000a of SM. However, the exact mechanism of the evolution

Ziv Gil; Sujay Rao; Shlomo Constantini

2000-01-01

119

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

120

George W. Arnold George Arnold was appointed National Coordinator for Smart Grid Interoperability at the  

E-print Network

George W. Arnold George Arnold was appointed National Coordinator for Smart Grid Interoperability the development of standards underpinning the nation's Smart Grid. Dr. Arnold joined NIST in September 2006

Magee, Joseph W.

121

09-NIF Dedication: Arnold Schwarzenegger  

ScienceCinema

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

Governor Arnold Schwarzenegger

2010-09-01

122

Arnold Schwarzenegger HIGHLY CONDUCTIVE, WATER  

E-print Network

Arnold Schwarzenegger Governor HIGHLY CONDUCTIVE, WATER INSOLUBLE AND THERMALLY STABLE PROTON Integration · Environmentally-Preferred Advanced Generation · Industrial/Agricultural/Water End-Use Energy EXCHANGE MEMBRANES FROM FUNCTIONALIZED POLYOXOMETALATES Prepared For: California Energy Commission Public

123

Arnold Schwarzenegger CALIFORNIA OCEAN WAVE  

E-print Network

Arnold Schwarzenegger Governor CALIFORNIA OCEAN WAVE ENERGY ASSESSMENT Prepared For: California this report as follows: Previsic, Mirko. 2006. California Ocean Wave Energy Assessment. California Energy Systems Integration · Transportation California Ocean Wave Energy Assessment is the final report

124

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY to treatment prescriptions and anticipated outputs of sawlogs and biomass fuel? How many individual operations biomass fuel removed. Typically in plantations. 50% No harvest treatment

125

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY PRODUCTION, AND OTHER BENEFITS PIERFINALPROJECTREPORT APPENDICES Prepared For: California Energy Commission Public Interest Energy Research Program Prepared By: USDA Forest Service Pacific Southwest Research

126

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY Citation: USDA Forest Service, Pacific Southwest Research Station. 2009. Biomass to Energy: Forest PRODUCTION, AND OTHER BENEFITS PIERFINALPROJECTREPORT Prepared For: California Energy Commission Public

127

Michael L. Arnold Publications PUBLICATIONS Books  

E-print Network

1 Michael L. Arnold ­ Publications PUBLICATIONS ­ Books 1. Arnold, M.L. 2008. Reticulate Evolution Evolution. Gene (ISSN 2073-4425). PUBLICATIONS ­ Journal Articles, Review Articles, Book Chapters and Essays

Arnold, Jonathan

128

Arnold's Theory of Emotion in Historical Perspective  

Microsoft Academic Search

Magda B. Arnold's theory of emotion is examined from three historical viewpoints. First, I look backward from Arnold to precursors of her theory of emotion in 19th century introspectionist psychology and in classical evolutionary psychology. I try to show that Arnold can be regarded as belonging intellectually to the cognitive tradition of emotion theorizing that originated in Brentano and his

Rainer Reisenzein

129

Arnold's theory of emotion in historical perspective  

Microsoft Academic Search

Magda B. Arnold's theory of emotion is examined from three historical viewpoints. First, I look backward from Arnold to precursors of her theory of emotion in 19th century introspectionist psychology and in classical evolutionary psychology. I try to show that Arnold can be regarded as belonging intellectually to the cognitive tradition of emotion theorising that originated in Brentano and his

Rainer Reisenzein

2006-01-01

130

Anorectal malformations  

Microsoft Academic Search

Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage,

Marc A Levitt; Alberto Peña

2007-01-01

131

arnold school of public health arnold school of public health  

E-print Network

, environmental protection, and healthcare delivery. The Arnold School has more than 7,600 alumni, approximately 4 ­ and for change in the culture of health ­ depends heavily upon a timely and successful capital campaign. #12 are committed to excellence in teaching, research, and service. Several SmartState centers, funded through the S

Almor, Amit

132

Arteriovenous Malformation  

Microsoft Academic Search

\\u000a Intracranial arteriovenous malformations (AVMs) are congenital lesions arising from abnormal blood vessel formation [1]–[3]. Whereas normal embryogenesis results in the differentiation of primordial vascular channels into mature arteries, veins,\\u000a and capillaries, patients with AVMs develop direct arteriovenous shunts without the appropriate intervening vascular beds.\\u000a Recent large, prospective, population-based studies have determined the incidence of newly diagnosed AVM patients to range

Bruce E. Pollock

133

Arnold Schwarzenegger California Wind Energy  

E-print Network

Arnold Schwarzenegger Governor California Wind Energy Resource Modeling and Measurement Prepared the Shasta sodar site and provided technical support. Scott Quitadamo, Kael Murray and Clear Channel as well as James Ruedlinger of the rest of Electronic Research and Dave Schulgen, Shawn Bachara Lupe Castanon

134

MEMORIAL RESOLUTION JOHN E. ARNOLD  

E-print Network

the educational television program for two years at M.I.T., appearing in or directing more than a hundred, he died while traveling in Italy. Educator, engineer, philosopher, consultant to industry, amateur the community's loss at his death. John Arnold was, perhaps, best known for his contributions to educational

Prinz, Friedrich B.

135

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY;6-2 #12;APPENDIX 6: Cumulative Watershed Effects Analysis for the Biomass to Energy Project 1. Principal the findings or recommendations of the study. Cumulative watershed effects (CWE) of the Biomass to Energy (B2E

136

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY or recommendations of the study. 1. INTRODUCTION 1.1 Domain Description The study area for the Biomass to Energy (B2 and environmental costs and benefits of using forest biomass to generate electrical power while changing fire

137

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY as a result of emerging biomass opportunities on private industrial and public multiple-use lands (tracked in the vegetation domain) and the quantity of biomass consumed by the wildfire (tracked

138

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY;12-2 #12;Appendix 12: Biomass to Energy Project Team, Committee Members and Project Advisors Research Team. Nechodom's background is in biomass energy policy development and public policy research. Peter Stine

139

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY;10-2 #12;Appendix 10: Power Plant Analysis for Conversion of Forest Remediation Biomass to Renewable Fuels and Electricity 1. Report to the Biomass to Energy Project (B2E) Principal Authors: Dennis Schuetzle, TSS

140

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY;5-2 #12;APPENDIX 5: BIOMASS TO ENERGY PROJECT:WILDLIFE HABITAT EVALUATION 1. Authors: Patricia Manley Ross management scenarios. We evaluated the potential effects of biomass removal scenarios on biological diversity

141

Arnold Schwarzenegger BIOMASS TO ENERGY  

E-print Network

Arnold Schwarzenegger Governor BIOMASS TO ENERGY: FOREST MANAGEMENT FOR WILDFIRE REDUCTION, ENERGY not substantively affect the findings or recommendations of the study. 2. Introduction The Biomass to Energy (B2E) Project is developing a comprehensive forest biomass-to- electricity model to identify and analyze

142

Arnold Schwarzenegger THE CARBON DIOXIDE  

E-print Network

i Arnold Schwarzenegger Governor THE CARBON DIOXIDE ABATEMENT POTENTIAL OF CALIFORNIA'S MID, Afzal Siddiqui, and Judy Lai. 2011. The Carbon Dioxide Abatement Potential of California's Mid/Agricultural/Water EndUse Energy Efficiency · Renewable Energy Technologies · Transportation The Carbon Dioxide

143

Cerebral Cavernous Malformation  

MedlinePLUS

NINDS Cerebral Cavernous Malformation Information Page Synonym(s): Cavernomas, Cavernous Angioma, Congenital Vascular Cavernous Malformations, Familial Hemangioma, Nevus Cavernosus, Vascular Erectile Tumor, Cavernous Malformation Table of Contents (click to jump to sections) ...

144

Matthew Arnold, the Masses, and Arts Education  

ERIC Educational Resources Information Center

In this article, the author examines the current arts education policies in the United States through the lens of Matthew Arnold's work and prose during his time as one of Her Majesty's School Inspectors in England and Wales in the nineteenth century. Arnold argued for government funding of public school systems, setting standards of quality for…

Pratt, Linda Ray

2007-01-01

145

Matthew Arnold, the Masses, and Arts Education  

Microsoft Academic Search

In this article, the author examines the current arts education policies in the United States through the lens of Matthew Arnold's work and prose during his time as one of Her Majesty's School Inspectors in England and Wales in the nineteenth century. Arnold argued for government funding of public school systems, setting standards of quality for teacher education, and many

Linda Ray Pratt

2007-01-01

146

2010, 12, 2, . 8384 ARNOLD IS GONE  

E-print Network

of thousands of modern scientists who mastered the art of solving ordinary differential equations with his the lapse of a century. However, the name of Arnold will never vanish from the Russian culture while our Russian language survives for Arnold made a discovery of an extraordinary brightness and talent

Kutateladze, Semen Samsonovich

147

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

148

Congenital malformations in Shimla  

Microsoft Academic Search

Congenital malformations were studied over a period of five years in 10, 100 consecutive births including still births at\\u000a Indira Gandhi Medical College, Shimla. Out of these, 180 babies had one or the other congenital malformations and the overall\\u000a incidence was 1.78%. Amongst the 311 still born babies 47 had congenital malformations indicating that the incidence of congenital\\u000a malformations was

Neelam Grover

2000-01-01

149

Three-dimensional tori and Arnold tongues.  

PubMed

This study analyzes an Arnold resonance web, which includes complicated quasi-periodic bifurcations, by conducting a Lyapunov analysis for a coupled delayed logistic map. The map can exhibit a two-dimensional invariant torus (IT), which corresponds to a three-dimensional torus in vector fields. Numerous one-dimensional invariant closed curves (ICCs), which correspond to two-dimensional tori in vector fields, exist in a very complicated but reasonable manner inside an IT-generating region. Periodic solutions emerge at the intersections of two different thin ICC-generating regions, which we call ICC-Arnold tongues, because all three independent-frequency components of the IT become rational at the intersections. Additionally, we observe a significant bifurcation structure where conventional Arnold tongues transit to ICC-Arnold tongues through a Neimark-Sacker bifurcation in the neighborhood of a quasi-periodic Hopf bifurcation (or a quasi-periodic Neimark-Sacker bifurcation) boundary. PMID:24697399

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

2014-03-01

150

Arnold Schwarzenegger WATER HEATERS AND HOT WATER  

E-print Network

Arnold Schwarzenegger Governor WATER HEATERS AND HOT WATER DISTRIBUTION SYSTEMS;#12;Appendices Appendix A. Multifamily Water Heating Construction Practices, Pricing and Availability Survey Report Appendix B. Multifamily Water Heating Controls Performance Field Report Appendix C. Pipe

151

Cees Snoek & Arnold Smeulders University of Amsterdam  

E-print Network

Expert--driven searchdriven search http://e-culture.multimedian.nl #12;Cees Snoek & Arnold Smeulders University of Amsterdam viewpoint, ... ·· ThisThis is theis the sensorysensory gapgap Suit Basketball Tree 1101011011011

Worring, Marcel

152

Arnold Schwarzenegger CLIMATE CHANGE IMPLICATIONS FOR  

E-print Network

Arnold Schwarzenegger Governor CLIMATE CHANGE IMPLICATIONS FOR MANAGING NORTHERN CALIFORNIA WATER development). Please cite this report as follows: HRCGWRI, 2011. Climate Change Implications Commission. It does not necessarily represent the views of the Energy Commission, its employees or the State

153

Three-dimensional tori and Arnold tongues  

NASA Astrophysics Data System (ADS)

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

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

2014-03-01

154

Arnold Schwarzenegger AIRCRAFT MEASUREMENTS OF THE  

E-print Network

Arnold Schwarzenegger Governor AIRCRAFT MEASUREMENTS OF THE IMPACTS OF POLLUTION AEROSOLS ON CLOUDS of Jerusalem Seeding Operation & Atmospheric Research Desert Research Institute, University of Nevada Seeding Operations & Atmospheric Research, POB 130, Plains TX 79355 James G. Hudson Desert Research

155

The speed of Arnold diffusion  

NASA Astrophysics Data System (ADS)

A detailed numerical study is presented of the slow diffusion (Arnold diffusion) taking place around resonance crossings in nearly integrable Hamiltonian systems of three degrees of freedom in the so-called ‘Nekhoroshev regime’. The aim is to construct estimates regarding the speed of diffusion based on the numerical values of a truncated form of the so-called remainder of a normalized Hamiltonian function, and to compare them with the outcomes of direct numerical experiments using ensembles of orbits. In this comparison we examine, one by one, the main steps of the so-called analytic and geometric parts of the Nekhoroshev theorem. Thus: (i) we review and implement an algorithm Efthymiopoulos (2008) [45] for Hamiltonian normalization in multiply resonant domains which is implemented as a computer program making calculations up to a high normalization order. (ii) We compute the dependence of the optimal normalization order on the small parameter ? in a specific model and compare the result with theoretical estimates on this dependence. (iii) We examine in detail the consequences of assuming simple convexity conditions for the unperturbed Hamiltonian on the geometry of the resonances and on the phase space structure around resonance crossings. (iv) We discuss the dynamical mechanisms by which the remainder of the optimal Hamiltonian normal form drives the diffusion process. Through these steps, we are led to two main results: (i) We construct in our concrete example a convenient set of variables, proposed first by Benettin and Gallavotti (1986) [12], in which the phenomenon of Arnold diffusion in doubly resonant domains can be clearly visualized. (ii) We determine, by numerical fitting of our data, the dependence of the local diffusion coefficient D on the size ?R? of the optimal remainder function, and we compare this with a heuristic argument based on the assumption of normal diffusion. We find a power law D??, where the constant b has a small positive value depending also on the multiplicity of the resonance considered.

Efthymiopoulos, C.; Harsoula, M.

2013-05-01

156

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

157

Magda B. Arnold's life and work in context  

Microsoft Academic Search

This paper provides a biographical and historical context for understanding and appreciating Magda B. Arnold's (1903–2002) theory and research on emotion. It situates Arnold's work in the context of mid-century emotion theory, the status of women psychologists, and pre-Cognitive Revolution psychology more generally. In considering Arnold's life and work, three themes stand out and deserve emphasis: (1) Arnold's lifelong passion

Stephanie A. Shields

2006-01-01

158

Intermingled fractal Arnold tongues V. Paar and N. Pavin  

E-print Network

Intermingled fractal Arnold tongues V. Paar and N. Pavin Department of Physics, Faculty of Science at low dissipation and weak forcing: Strips of 2 2 Arnold tongues form a truncated fractal structure and the tonguelike regions in between are filled by finely intermingled fractal-like 1 1 and 3 3 Arnold tongues

Pavin, Nenad

159

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

160

Arnold Schwarzenegger HIGH-VOLUME MANUFACTURING FOR  

E-print Network

Arnold Schwarzenegger Governor HIGH-VOLUME MANUFACTURING FOR LOW-COST, FLEXIBLE SOLAR CELL Prepared Manager Elaine Sison-Lebrilla Renewables Program Area Lead Elaine Sison-Lebrilla Office Manager Energy-VOLUME MANUFACTURING FOR LOW-COST, FLEXIBLE SOLAR CELL EISG AWARDEE InterPhases Research 166 N. Moorpark Rd. Suite 204

161

Arnold Schwarzenegger NOVEL APPROACHES FOR THE  

E-print Network

Arnold Schwarzenegger Governor NOVEL APPROACHES FOR THE RECLAMATION AND REUSE OF POWER PLANT that the uses of this information will not infringe upon privately owned rights. This report has not been Plant Effluents. California Energy Commission, PIER Energy-Related Environmental Research. CEC-500

162

Arnold Schwarzenegger DATA SOURCES FOR CLIMATE CHANGE  

E-print Network

Arnold Schwarzenegger Governor DATA SOURCES FOR CLIMATE CHANGE RESEARCH WITH A COMPUTABLE GENERAL-Holst, University of California, Berkeley PIERPROJECTREPORT June 2007 CEC-500-2006-080 #12;California Climate Change for Climate Change Research with a Computable General Equilibrium (CGE) Model of the California Economy

163

Pelvic vascular malformations.  

PubMed

Vascular malformations (VMs) comprise a wide spectrum of lesions that are classified by content and flow characteristics. These lesions, occurring in both focal and diffuse forms, can involve any organ and tissue plane and can cause significant morbidity in both children and adults. Since treatment strategy depends on the type of malformation, correct diagnosis and classification of a vascular lesion are crucial. Slow-flow VMs (venous and lymphatic malformations) are often treated by sclerotherapy, whereas fast-flow lesions (arteriovenous malformations) are generally managed with embolization. In addition, some cases of VMs are best treated surgically. This review will present an overview of VMs in the female pelvis as well as a discussion of endovascular therapeutic techniques. PMID:24436563

Christenson, Brian M; Gipson, Matthew G; Smith, Mitchell T

2013-12-01

164

Posterior fossa malformations.  

PubMed

Understanding embryologic development of the cerebellum and the 4th ventricle is essential for understanding posterior fossa malformations. Posterior fossa malformations can be conveniently classified into those that have a large posterior fossa and those with normal or small posterior fossa. Disorders associated with a large posterior fossa include classic Dandy-Walker malformation, Blake's pouch cyst, mega cisterna magna, and posterior fossa arachnoid cyst. Disorders associated with normal or small posterior fossa include Dandy-Walker variant, Joubert syndrome, tecto-cerebellar dysraphia, rhombencephalosynapsis, the neocerebellar hypoplasias, and cerebellar atrophy. Neuro-imaging features should enable the imager to provide the referring physician a logical approach to these complex posterior fossa malformations. PMID:21596278

Shekdar, Karuna

2011-06-01

165

Management of Vascular Malformations  

PubMed Central

Background: Even though vascular malformations are well categorized, further details are relatively unknown. Of treated patients regarding the frequency, demographic distributions, and other related factors by multivariate regression analyses in proportion to total vascular malformations, methods of treatment and how to manage them have not been elucidated thoroughly. Methods: From January 2006 to March 2012, consecutively treated patients with vascular anomalies were included in this investigation at least 1-year follow-up. Results: Of the total of 123 cases, 86 females and 37 males, the mean follow-up was 3.5?±?1.68 years, and the frequency of treatment was 1–8 times (1.8?±?1.30). Surgery was performed for 22 cases (17.9%) of venous malformations and arteriovenous malformations. In multivariate regression, the frequency of treatment was significantly correlated with the length of follow-up (P < 0.001), age (P < 0.05), and type of malformations (P < 0.05) (R2 = 0.18). Need for surgery was significantly increased with age at odds ratio (OR) of 1.06 [95% confidence interval (CI), 1.03–1.80] (P < 0.001), and head/face/neck, and upper limb are more performed at OR of 0.24 (95% CI, 0.07–0.85) (P < 0.05). The satisfaction score varied from 1 to 5 (3.9?±?0.68). Complications occurred in 3 cases (2.4%). In logistic regression of complications, the OR of the satisfaction score was 0.13 (95% CI, 0.02–0.80) (P < 0.05). Conclusions: Treatment of vascular malformations is an integral part of multidisciplinary approaches. Venous malformations are more frequent in combination surgery, and if there are fewer complications, the patients’ satisfaction increases.

Houbara, Seiji; Hirano, Akiyoshi

2014-01-01

166

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

2013-01-01

167

Lighting Arnold Flames: Resonance in Doubly Forced Periodic Oscillators  

E-print Network

Lighting Arnold Flames: Resonance in Doubly Forced Periodic Oscillators Bruce B. Peckham Ioannis G@arnold.princeton.edu July 15, 2002 Abstract We study doubly forced nonlinear planar oscillators: x = V(x) + 1W1(x, 1t) + 2W2 x = V(x) has an attracting limit cycle -- the unforced pla- nar oscillator -- with frequency 0

Peckham, Bruce B.

168

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

ERIC Educational Resources Information Center

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

Brodie, Carolyn S.

2005-01-01

169

Diffusion tensor imaging evaluation of white matter in adolescents with myelomeningocele and Chiari II malformation  

Microsoft Academic Search

Background  Macrostructural abnormalities in cerebral white matter in patients with myelomeningocele are well known, but microstructural\\u000a abnormalities are not as well studied.\\u000a \\u000a \\u000a \\u000a \\u000a Objective  The aim of this study was to evaluate cerebral white matter in adolescents with myelomeningocele using diffusion tensor imaging\\u000a (DTI), and to investigate the effects of ventricular dilation and CSF shunt presence on white matter microstructure in these\\u000a patients.

Xiawei Ou; Charles M. Glasier; Jeffrey H. Snow

170

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. PMID:23493894

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

2013-01-01

171

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

Microsoft Academic Search

A 27-year-old woman presented with bilateral weakness of her all extremities for 5 years. She had a spastic gait and was unable\\u000a to ambulate without assistance. Neurologic examination revealed increased deep tendon reflexes and positive pathologic reflexes.\\u000a Radiographs showed occipitalization of the atlas, C2–C3 congenital fusion and fixed atlantoaxial dislocation with an atlanto-dental\\u000a interval of 10 mm. MRI demonstrated cervicomedullary junction (CMJ)

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

2010-01-01

172

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.

173

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

PubMed

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

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

2011-03-29

174

Budd-Chiari syndrome associated with oral contraceptive steroids  

Microsoft Academic Search

Oral contraceptive steroids (OCS) have been implicated as the cause of a number of instances of hepatic vein thrombosis (Budd-Chiari syndrome). Survival appears to be related to early diagnosis and treatment, but there does not appear to be a consensus as to the most appropriate management of these patients. Portosystemic shunting has frequently been advocated, although the results have been

James H. Lewis; Howard L. Tice; Hyman J. Zimmerman

1983-01-01

175

[Pseudo-tumoral neurological affections in adults localized to the middle and posterior sections of base of skull (author's transl)].  

PubMed

Many neurological affections of the middle and posterior sections of base of skull may simulate tumors. Vascular disorders include diabetic lesions, diffuse cerebral vascularitis, vascular malformations, posterior fossa angioma, telangiectasis or cavernous hemangioma, and basilar artery aneurysms. Degenerative or deficiency nervous system affections may also simulate tumors, as well as bone or nerve malformations, such as erosions due to basilar artery compression. Arnold-Chiaris malformation, aqueduct stenosis, or an empty sella turcica. PMID:7325514

Rondot, P

1981-01-01

176

Multiple cerebral arteriovenous malformations (AVMs)  

Microsoft Academic Search

From our series of 203 patients with cerebral vascular lesions, 18 (9%) could be included in the multiple arteriovenous malformation category. There were five patients with Rendu-Osler-Weber, one with Wyburn-Mason syndromes and two with concurrent arteriovenous malformations. The remaining ten patients (4%) had multiple brain arteriovenous malformations. Careful angiography with magnification is necessary to try to diagnose multiple brain AVMs,

R. A. Willinsky; P. Lasjaunias; K. Terbrugge; P. Burrows

1990-01-01

177

Vladimir I. Arnold From superpositions to KAM theory  

E-print Network

. Sevryuk. A related material is contained also in Arnold's recollections "On A. N. Kolmogorov". Slightly childhood when he had read Flammarion's Astronomy,2 but the success had come only after Stalin's death

178

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

179

Anencephaly and its Associated Malformations  

PubMed Central

Introduction: Anencephaly is a serious neural tube defect in which parts of the brain and skull are not developed. But apart from this it is also associated with other malformations which are not related to neural tube in development. Aim: The present study was undertaken to find the associated congenital malformations in western region of India and establish a aetiological correlation. Materials and Methods: The study was conducted using 20 anencephalic fetuses. Results: Nearly 80% of fetuses had associated malformations. Spina bifida was seen in 9 fetuses and cleft palate in 8. Female fetus with cleft palate had other severe associated gastrointestinal and skeletal malformation. Conclusion: In cases of anencephaly other associated malfor–mations like spina bifida and cleft palate are commonly seen. PMID:25386414

Meshram, Pritee Madan; Hattangdi, Shanta Sunil

2014-01-01

180

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

181

Brain arteriovenous malformations.  

PubMed

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

Ferrara, Adi R

2011-01-01

182

Radiosurgery for arteriovenous malformations.  

PubMed

Stereotactic radiosurgery is the term coined by Lars Leksell to describe the application of a single, high dose of radiation to a stereotactically defined target volume. In the 1970s, reports began to appear documenting the successful obliteration of arteriovenous malformations (AVMs) with radiosurgery. When an AVM is treated with radiosurgery, a pathologic process appears to be induced that is similar to the response-to-injury model of atherosclerosis. Radiation injury to the vascular endothelium is believed to induce the proliferation of smooth-muscle cells and the elaboration of extracellular collagen, which leads to progressive stenosis and obliteration of the AVM nidus thereby eliminating the risk of hemorrhage. The advantages of radiosurgery - compared to microsurgical and endovascular treatments - are that it is noninvasive, has minimal risk of acute complications, and is performed as an outpatient procedure requiring no recovery time for the patient. The primary disadvantage of radiosurgery is that cure is not immediate. While thrombosis of the lesion is achieved in the majority of cases, it commonly does not occur until two or three years after treatment. During the interval between radiosurgical treatment and AVM thrombosis, the risk of hemorrhage remains. Another potential disadvantage of radiosurgery is possible long term adverse effects of radiation. Finally, radiosurgery has been shown to be less effective for lesions over 10 cc in volume. For these reasons, selection of the optimal treatment for an AVM is a complex decision requiring the input of experts in endovascular, open surgical, and radiosurgical treatment. In the pages below, we will review the world's literature on radiosurgery for AVMs. Topics reviewed will include the following: radiosurgical technique, radiosurgery results (gamma knife radiosurgery, particle beam radiosurgery, linear accelerator radiosurgery), hemorrhage after radiosurgery, radiation induced complications, repeat radiosurgery, and radiosurgery for other types of vascular malformation. PMID:22004703

Friedman, William A; Bova, Frank J

2011-10-01

183

Management strategy for facial venous malformations  

PubMed Central

Venous malformations (VMs) are slow-flow vascular malformations, caused by abnormalities in the development of the veins. Venous malformations vary in size and location within the body. When the skin or tissues just under the skin are affected, they appear as slightly blue-colored skin stains or swellings. These can vary in size from time to time because of swelling within the malformation. As these are vascular malformations, they are present at birth and grow proportionately with the child. Venous malformations can be very small to large in size, and sometimes, can involve a significant area within the body, When the venous malformation is well localized, this may cause localized swelling, however, when the venous malformation is more extensive, there may be more widespread swelling of the affected body part. Some patients with venous malformations have abnormal blood clotting within the malformation. Most venous malformations cause no life-threatening problems for patients. Some venous malformations cause repeated pain due to intermittent swelling and congestion of the malformation or due to the formation of blood clots within the malformation. Rarely, venous malformations may be part of a syndrome (an association of several clinically recognizable features) or be linked to an underlying genetic abnormality. We present 12 cases of venous malformations of the head and neck area, which have been managed at our hospital.

Kumar, Shailendra; Kumar, Vijay; Kumar, Sanjeev; Kumar, Surender

2014-01-01

184

Mapping the Arnold web with a graphic processing unit.  

PubMed

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. PMID:22225360

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

2011-12-01

185

Embolization of uterine arteriovenous malformation  

PubMed Central

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

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

2013-01-01

186

Cerebral malformations without antenatal diagnosis  

Microsoft Academic Search

Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms),\\u000a or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in\\u000a utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered\\u000a in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital

Nadine J. Girard

2010-01-01

187

Cerebral Cavernous Malformations: Surgical Perspective  

Microsoft Academic Search

\\u000a Cerebral cavernous malformations (CCMs) are rare vascular lesions [1], [2]; however, because hemorrhage of CCMs can result in significant morbidity and mortality [3], [4], a great deal of effort has been devoted to their detection and treatment. Although previously thought to be solely congenital,\\u000a it is now well recognized that many cavernous malformations are acquired [5], arising de novo or

Robert L. Dodd; Gary K. Steinberg

188

Pancreatic Arteriovenous Malformation  

PubMed Central

An unusual case of pancreatic arteriovenous malformation (P-AVM) combined with esophageal cancer is reported. A 59-year-old man was admitted with upper abdominal pain. Contrast-enhanced computed tomography showed numerous strongly enhanced abnormal vessels and a hypovascular lesion in the area of the pancreatic tail. Angiographic study of the celiac artery confirmed racemose vascular networks in the tail of the pancreas. Endoscopic retrograde pancreatography revealed narrowing and displacement of the main pancreatic duct in the tail of the pancreas. Screening esophagoscopy showed a 0-IIa+IIc type tumor in the lower thoracic esophagus. Histological examination of esophagoscopic biopsies showed squamous cell carcinoma. Based on these findings, P-AVM or pancreatic cancer and esophageal cancer were diagnosed. Video-assisted thoracoscopic esophagectomy and distal pancreatectomy were performed. Histological examination of the resected pancreas revealed abundant abnormal vessels with intravascular thrombi. In addition, rupture of a dilated pancreatic duct with pancreatic stones and both severe atrophy and fibrosis of the pancreatic parenchyma were observed. The final diagnoses were P-AVM consequent to severe chronic pancreatitis and esophageal carcinoma. The patient's postoperative course was relatively good. PMID:24574946

Yamabuki, Takumi; Ohara, Masanori; Kimura, Noriko; Okamura, Kunishige; Kuroda, Aki; Takahashi, Ryo; Komuro, Kazuteru; Iwashiro, Nozomu

2014-01-01

189

Pulmonary arteriovenous malformations.  

PubMed

Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. As a consequence, patients with PAVM can have hypoxemia and paradoxical embolization complications, including stroke and brain abscess. PAVMs may be single or multiple, unilateral or bilateral, and simple or complex. Most PAVMs are hereditary and occur in hereditary hemorrhagic telangiectasia, an autosomal dominant vascular disorder, and screening for PAVM is indicated in this subgroup. PAVMs may also be idiopathic, occur as a result of trauma and infection, or be secondary to hepatopulmonary syndrome and bidirectional cavopulmonary shunting. Diagnostic testing involves identifying an intrapulmonary shunt, with the most sensitive test being transthoracic contrast echocardiography. Chest CT scan is useful in characterizing PAVM in patients with positive intrapulmonary shunting. Transcatheter embolotherapy is the treatment of choice for PAVM. Lifelong follow-up is important because recanalization and collateralization may occur after embolization therapy. Surgical resection is rarely necessary and reserved for patients who are not candidates for embolization. Antibiotic prophylaxis for procedures with a risk of bacteremia (eg, dental procedures) is recommended in all patients with PAVM because of the risk of cerebral abscess. PMID:24008954

Cartin-Ceba, Rodrigo; Swanson, Karen L; Krowka, Michael J

2013-09-01

190

Pediatric dural arteriovenous malformations.  

PubMed

Pediatric dural arteriovenous malformations (dAVMs) are rare lesions that have a high mortality rate and require complex management. The authors report 3 cases of pediatric dAVMs that presented with macrocrania and extracranial venous distension. Dural sinus thrombosis developed in 2 of the cases prior to any intervention, which is an unusual occurrence for this particular disease. All 3 cases were treated using staged endovascular embolization with a favorable outcome in 1 case and a poor outcome in the other 2 cases. Complications developed in all cases and included dural sinus thrombosis, parenchymal hemorrhage, intracranial venous hypertension, and seizures. The strategies and challenges used in managing these patients will be presented and discussed, along with a review of the literature. While outcomes remain poor, the authors conclude that prompt treatment with endovascular embolization provides the best results for children with these lesions. A well-established venous collateral circulation draining directly to the internal jugular veins may further improve the rate of favorable outcome after embolization. PMID:24867128

Appaduray, Shaun P; King, James A J; Wray, Alison; Lo, Patrick; Maixner, Wirginia

2014-07-01

191

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 (10 8 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

192

Embolization of Arteriovenous Malformation  

PubMed Central

Summary Treatment options for cerebral arteriovenous malformation (AVM) are still controversial due to the recent result of stereotactic radiosurgery and the improved result of microsurgical resection. We investigated previously treated AVM cases and discussed the efficacy and safety of preoperative embolization especially for microsurgical resection of high-grade AVM in the Spetzler-Martin grading. Efficacy of preoperative embolization was evaluated based on 126 previously treated AVM cases at Shinshu University Hospital during the last 25 years. The safety of embolization was evaluated based on our previously-embolized 58 AVM cases (91 procedures) in the last 11 years after introduction of preoperative embolization for AVM. In all 126 cases, 82 were treated before introduction of embolization and 44 were treated after introduction of embolization. In 82 cases of the pre-embolization era, 63 lesions were removed totally in 63 AVMs (77%), partially resected in 11 (13%) and untreated in eight (10%). In 74 surgically removed cases, 11 (15%) cases showed severe intra/postoperative bleeding. In 44 cases of the embolization era, lesions were removed totally in 29 AVMs (66%), disappeared only with embolization in one (2%), disappeared with radiosurgery in seven (16%) and were untreated in five (11%). In 32 surgically removed cases, only one (2%) case showed severe intra/postoperative bleeding. In all 58 embolized cases, 44 were surgically removed, six were treated with radiosurgery, one was eliminated with embolization alone and six were partially obliterated and followed up for their location. In 91 procedures for 58 cases, two haemorrhagic and three ischemic complications occurred, three were transient and two remained having neurological deficits. The introduction of preoperative embolization improved the total removal rate and reduced the intra/postoperative bleeding rate in surgical removal of AVM. The total risk of embolization is low and well-designed preoperative embolization makes surgical resection safer even in high-grade AVM in the Spetzler-Martin grading. PMID:20587250

Nagashima, H.; Hongo, K.; Kobayashi, S.; Takamae, T.; Okudera, H.; Koyama, J.I.; Oya, F.; Matsumoto, Y.

2004-01-01

193

Please Join Us as Dr. Arnold F. Stancell Presents  

E-print Network

recommendations for improved offshore drilling safety following the BP oil spill tragedy. Dr. StancellPlease Join Us as Dr. Arnold F. Stancell Presents "BP Oil Tragedy: What Went Wrong and Leadership in an explosion and fire aboard the Deep Water Horizon drilling rig. The spill gushed five million barrels of oil

Miami, University of

194

Arnold Schwarzenegger TIME-OF-USE WATER METER EFFECTS  

E-print Network

, and Katherine Hardy; California Urban Water Conservation Council ­ Chris Brown; Electric Power Research Water and Power ­ Peter Kavounas; Valencia Water Company ­ Robert DiPrino; Tehachapi Cummins WaterArnold Schwarzenegger Governor TIME-OF-USE WATER METER EFFECTS ON CUSTOMER WATER USE

195

Arnold L. Gesell: The Paradox of Nature and Nurture  

Microsoft Academic Search

Arnold Gesell (1880–1961) has had an important and lasting impact on the field of developmental psychology He is best remembered for his developmental norms, which were acquired from decades of detailed observations of infants and children and are still the basis of most early assessments of behavioral functioning. Gesell's influence as a theorist is less direct. His maturationism quickly lost

Esther Thelen; Karen E. Adolph

1992-01-01

196

Improving weapons of mass destruction intelligence Arnold Kanter  

E-print Network

1 Improving weapons of mass destruction intelligence Arnold Kanter The Scowcroft Group 900;2 Combating the spread of weapons of mass destruction (WMD) is one of the most important foreign policy of nuclear capability by sub-national states and the security of WMD weapons, materials, and technology

Deutch, John

197

[Arteriovenous malformation in the mandibula].  

PubMed

A 15-year-old autistic boy of subnormal intelligence appeared at the office of his dental practitioner complaining about pain in the third quadrant. Investigation revealed that the second molar could be intruded easily. No other irregularities, such as caries or periodontal disease were apparent. Due to the severe pain it was decided that the tooth should be extracted. Extraction of the tooth induced massive bleeding. Hemostasis was achieved by repositioning the molar in its alveolus, according to the principle of putting a cork in a bottle. Radiographic investigation showed the presence of an intraosseous arteriovenous malformation. These malformations are potentially life-threatening lesions. Treatment of choice is a combination of transarterial embolization and surgical removal of the malformation. PMID:17715775

Kruizinga, E H W; Meijer, G J; Koole, R; van Es, R J J

2007-07-01

198

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. PMID:24137154

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

199

Glioblastoma mimicking an arteriovenous malformation.  

PubMed

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. PMID:24137154

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

200

Arteriovenous malformation of the pancreas.  

PubMed

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. PMID:21765845

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

2011-01-01

201

Arnold's nerve cough reflex: evidence for chronic cough as a sensory vagal neuropathy  

PubMed Central

Arnold’s nerve ear-cough reflex is recognised to occur uncommonly in patients with chronic cough. In these patients, mechanical stimulation of the external auditory meatus can activate the auricular branch of the vagus nerve (Arnold’s nerve) and evoke reflex cough. This is an example of hypersensitivity of vagal afferent nerves, and there is now an increasing recognition that many cases of refractory or idiopathic cough may be due to a sensory neuropathy of the vagus nerve. We present two cases where the cause of refractory chronic cough was due to sensory neuropathy associated with ear-cough reflex hypersensitivity. In both cases, the cough as well as the Arnold’s nerve reflex hypersensitivity were successfully treated with gabapentin, a treatment that has previously been shown to be effective in the treatment of cough due to sensory laryngeal neuropathy (SLN). PMID:25383210

Gibson, Peter G.; Birring, Surinder S.

2014-01-01

202

Radiosurgery for Cerebral Arteriovenous Malformations  

Microsoft Academic Search

From September 1991 to May 1997, a total of 59 patients diagnosed with cerebral arteriovenous malformations were treated using radiosurgery. There were 29 men and 30 women, with an age range of 5 to 75 years (mean 36). Of these patients, 39 patients were treated using a LINAC-based system (group 1) and 20 using the Leksell ?-knife unit (group 2).

Lucia Zamorano; Amgad Matter; Laurie Caspar; Arturo Saenz; Razvan Buciuc; James Fontanesi; Azucena Garzon; Fernando Diaz

1998-01-01

203

Congenital Pulmonary Malformation in Children  

PubMed Central

Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life. PMID:24278678

Nadeem, Montasser; Elnazir, Basil; Greally, Peter

2012-01-01

204

Omphalocele and gastroschisis and associated malformations.  

PubMed

The etiology of gastroschisis and omphalocele is unclear and their pathogenesis is controversial. Because previous reports have inconsistently noted the type and frequency of malformations associated with omphalocele and gastroschisis, we assessed these associated malformations ascertained between 1979 and 2003 in 334,262 consecutive births. Of the 86 patients with omphalocele, 64 (74.4%) had associated malformations. These included patients with chromosomal abnormalities (25, 29.0%); non-chromosomal syndromes including Beckwith-Wiedemann syndrome, Goltz syndrome, Marshall-Smith syndrome, Meckel-Gruber syndrome, Oto-palato-digital type II syndrome, CHARGE syndrome, and fetal valproate syndrome; malformation sequences, including ectopia cordis, body stalk anomaly, exstrophy of bladder, exstrophy of cloaca, and OEIS (Omphalocele, Exstrophy of bladder, Imperforate anus, Spinal defect); malformation complexes including Pentalogy of Cantrell, and non-syndromic multiple congenital anomalies (MCA) (26, 30.2%). Malformations of the musculoskeletal system (31, 23.5%), urogenital system (27, 20.4%), cardiovascular system (20, 15.1%), and central nervous system (12, 9.1%) were the most common other congenital malformations in patients with omphalocele and non-syndromic MCA. Of the 60 patients with gastroschisis, 10 (16.6%) had associated malformations. In contrast to omphalocele, gastroschisis was rarely associated with a complex pattern of malformation, that is, one each (1.7%) with a chromosomal abnormality (trisomy 21), sequence (amyoplasia congenita), unspecified dwarfism, and 7 (11.7%) with MCA. We observed a striking difference in the prevalence of total malformations (74.4% vs. 16.6%, P < 0.001) and specific patterns of malformations associated with omphalocele and gastroschisis which emphasizes the need to evaluate all patients with omphalocele and gastroschisis for possible associated malformations. Malformation surveillance programs should be aware that the malformations associated with omphalocele can be often classified into a recognizable malformation syndrome or pattern (44.2%). PMID:18386803

Stoll, Claude; Alembik, Yves; Dott, Beatrice; Roth, Marie-Paule

2008-05-15

205

Arteriovenous Malformation of the Oral Cavity  

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 tumors. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. Arteriovenous malformation of the head and neck is a rare vascular anomaly but when present is persistent and progressive in nature and can represent a lethal benign disease. Here we present a case report of a 25-year-old male patient with arteriovenous malformation involving the base of tongue. PMID:24660070

Manjunath, S. M.; Shetty, Sujan; Moon, Ninad J.; Metta, Kiran Kumar; Gupta, Nitin; Goyal, Sandeep

2014-01-01

206

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

207

Cerebellar arteriovenous malformations in children  

Microsoft Academic Search

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM).\\u000a This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques.\\u000a All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the\\u000a study had MRI. Of the

P. D. Griffiths; S. Blaser; D. Armstrong; S. Chuang; R. P. Humphreys; D. Harwood-Nash

1998-01-01

208

Arteriovenous malformations in the brain  

Microsoft Academic Search

Opinion statement  Arteriovenous malformations (AVM) are a leading cause of intracerebral hemorrhage, especially among the young. Because they\\u000a pose a lifelong risk of serious bleeding, definitive treatment to obliterate the AVM should be pursued in the majority of\\u000a patients. Microsurgical resection of a small AVM located in the superficial or noneloquent brain achieves high cure rates\\u000a with low morbidity, and is

Glenn D. Graham

2002-01-01

209

Cerebral arteriovenous malformations in children  

Microsoft Academic Search

The treatment of cerebral arteriovenous malformations (AVM) or vascular anomalies are challenging neurosurgical procedures\\u000a for an anaesthetist. Large AVMs are uncommon in children. Only 18% of AVMs become symptomatic before the age of 15 yr. This\\u000a series reviews the experience at this institution during the period of 1982 to 1992. The symptoms at the time of presentation\\u000a are varied and

Carol Millar; Bruno Bissonnette; R. P. Humphreys

1994-01-01

210

Endovascular Treatment of Arteriovenous Malformation  

Microsoft Academic Search

Vascular anomalies are common congenital or neonatal abnormalities. According to the approved classification of vascular lesions\\u000a by Glowacki and Mulliken, hemangiomas and vascular malformations are distinguishable. Hemangiomas usually appear during the\\u000a first days or weeks after birth and grow faster than the whole body of the infant. They are proliferating benign tumors that\\u000a often involute. The opposite of hemangiomas, vascular

Robert Juszkat; Bartosz ?abicki; Pawe? Ch?ci?ski; Marcin Gabriel; Nawal Matar

2009-01-01

211

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

Microsoft Academic Search

A 13-mo-old intact male African lion (Panthera leo) presented with a 3-mo history of lethargy, ventral flexion of the neck, abnormal vocalization, and ataxia. Hemogram and serum biochemistries were within normal limits except for the presence of hypokalemia (2.7 mEq\\/L) and hypochloridemia (108 mEq\\/L). When no improvement was noted with oral potassium gluconate supplementation, a computed tomography scan of the

Stephanie McCain; Marcy J. Souza; Edward C Ramsay; Juergen Schumacher; William B Thomas; Silke Hecht

2008-01-01

212

Hydrocephalus and Chiari type 1 malformation in macrocephaly-cutis marmorata telangiectatica congenita: a case-based update  

Microsoft Academic Search

Background  Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is a newly described condition characterized by macrocephaly\\u000a (megalencephaly), cutis marmorata telangiectatica congenita, macrosomia and\\/or asymmetric growth, central nervous system abnormalities\\u000a and neurological manifestations.\\u000a \\u000a \\u000a \\u000a Discussion  This condition is usually documented in the Genetics’ literature but it has been scarcely disseminated among neurosurgeons.\\u000a Sudden death of uncertain origin has been reported in children with M-CMTC. Hydrocephalus and

Juan F. Martínez-Lage; Encarna Guillén-Navarro; María-José Almagro; Matías Felipe-Murcia; Antonio López López-Guerrero; Marcelo Galarza

2010-01-01

213

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

Microsoft Academic Search

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,

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

2010-01-01

214

Quantitative results on heating events in the quiet corona Arnold O. Benz and Sm Krucker  

E-print Network

Quantitative results on heating events in the quiet corona Arnold O. Benz and Säm Krucker Citation;Quantitative Results on Heating Events in the Quiet Corona Arnold O. Benz 1 and Krucker2 1Institute constitute a major energy input into the corona, suggesting that the lower corona is not just heated

215

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

ERIC Educational Resources Information Center

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

Dietz, Mark David

2010-01-01

216

[Pulmonary arteriovenous malformation: two sibling cases].  

PubMed

Pulmonary arteriovenous malformations, are abnormal connections between pulmonary arteries and veins. However it can be presented as an isolated single anomaly, also may be multiple when accompanying with autosomal dominant hereditary hemorrhagic telengiectasia (Rendu-Osler-Weber Syndrome; ROWS). In this case report, two patients operated with the diagnosis of multiple pulmonary arteriovenous malformation familial screening done with the possibility of ROWS and pulmonary arteriovenous malformation found in her sister, are presented. PMID:21554236

Yenigün, Bülent Mustafa; Yüksel, Cabir; Enön, Serkan; Kay? Cang?r, Ayten; Atasoy, Kayhan Cetin

2011-01-01

217

Genetics Home Reference: Cerebral cavernous malformation  

MedlinePLUS

... Information Center Information about genetic conditions and rare diseases Additional NIH Resources ... cavernous malformations are collections of small blood vessels (capillaries) in the brain that are enlarged and ...

218

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

PubMed Central

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 of the external dura accompanied by widening the cisterna magna and making longitudinal incisions in the internal dura, without disturbing the arachnoid. Methods Ten patients with CMI and syringomyelia, operated between 2004 and 2006, formed this prospective series. All cases underwent foramen magnum decompression of 3 × 3 cm area with C1–C2 (partial) laminectomy, resection of foramen magnum fibrous band, excision of external dura, delamination and widening of internal dura with longitudinal incisions. Results Patients were aged between 25 and 58 years and occipital headache was the most common complaint. The mean duration of preoperative symptoms was 4 years and the follow-up time was 25 months. Clinical progression was halted for all patients; eight patients completely recovered and two reported no change. In one patient, there was a transient cerebrospinal fluid (CSF) fistula that was treated with tissue adhesive. While syringomyelia persisted radiologically with radiological stability in five patients; for three patients the syringomyelic cavity decreased in size, and for the remaining two it regressed completely. Conclusion Removal of the fibrous band and the outer dural layer, at level of foramen magnum, together with the incision of inner dural layer appears to be good technique in adult CMI patients. The advantages are short operation time, no need for duraplasty, sufficient posterior fossa decompression, absence of CSF fistulas as a result of extra arachnoidal surgery, and short duration of hospitalization. Hence this surgical technique has advantages compared to other techniques. PMID:19545443

Kotil, Kadir; Ton, Tugrul; Tari, Rabia; Savas, Yildiray

2009-01-01

219

Review of holographic instrumentation at Arnold Engineering Development Center  

NASA Technical Reports Server (NTRS)

The employment of holographic instrumentation systems at the Arnold Engineering Development Center is discussed. A large number of applications were researched to improve the systems which are presently in use and to determine the feasibility of new applications. Operational systems and research include the application of holography to particle sizing, three-dimensional visualization, flow visualization, nondestructive testing, and optical data processing. A summary of the systems and research is provided and new techniques which have evolved in the development of these systems are discussed. Emphasis is placed on those systems in which holography has provided a data capability which was not obtainable by other methods.

Trolinger, J. D.

1973-01-01

220

Graphical evolution of the arnold web: from order to chaos  

PubMed

We represent graphically the evolution of the set of resonances of a quasi-integrable dynamical system, the so-called Arnold web, whose structure is crucial for the stability properties of the system. The basis of our representation is the use of an original numerical method, whose definition is directly related to the dynamics of orbits, and the careful choice of a model system. We also show the transition from the Nekhoroshev stability regime to the Chirikov diffusive one, which is a generic nontrivial phenomenon occurring in many physical processes, such as slow chaotic transport in the asteroid belt and beam-beam interaction. PMID:11000109

Froeschle; Guzzo; Lega

2000-09-22

221

Arnold cat map, Ulam method and time reversal  

E-print Network

We study the properties of the Arnold cap map on a torus with a several periodic sections using the Ulam method. This approach generates a Markov chain with the Ulam matrix approximant. We study numerically the spectrum and eigenstates of this matrix showing their relation with the Fokker-Plank relaxation and the Kolmogorov-Sinai entropy. We show that, in the frame of the Ulam method, the time reversal property of the map is preserved only on a short Ulam time which grows only logarithmically with the matrix size. Parallels with the evolution in a regime of quantum chaos are also discussed.

Leonardo Ermann; Dima L. Shepelyansky

2011-07-03

222

Management of pulmonary arteriovenous malformations.  

PubMed

Pulmonary arteriovenous malformations are rare lesions with significant clinical complications. These lesions are commonly seen in patients with hereditary hemorrhagic telangiectasia (formerly Osler-Weber-Rendu syndrome). Interventional radiologists are a key part of the treatment team in this complex disease, and a thorough understanding of the disease process is critical to providing good patient care. In this article, the authors review the disease course and its association with hereditary hemorrhagic telangiectasia, discusses the clinical evaluation and treatment of these complex patients, and outlines complications and follow-up. PMID:22379273

Meek, Mary E; Meek, James C; Beheshti, Michael V

2011-03-01

223

Aspects cliniques des malformations capillaires  

Microsoft Academic Search

Capillary malformations (CM) are defects of the dermal capillary bed. These slow-flow malformations can affect any part of the body and are always lateralized, despite Unna's naevus. Present at birth, they grow proportionally with the child. In rare instance, they can be part of a more complex syndrome such as Sturge-Weber syndrome. Ectatic CMs of telangiectatic types can be cutaneous,

A.-C. Bataille; L.-M. Boon

2006-01-01

224

[Ureteral triplication whitout other associates malformations].  

PubMed

We report a case of ureteral type III triplication according to Smith's classification, without associated anomalies of the urinary tract. The revision of the bibliography indicates the rarity of this maldformacion and its frequent association with other urinary and extraurinary malformations. The absence of associate malformations in this case increases his exceptionality. PMID:19537075

Diz Rodríguez, Ramón; Arance Gil, Ignacio; Vírseda Chamorro, Miguel; Quijano Barroso, Pablo; Alpuente Román, Carlos; Sáenz Benito, Domingo

2009-03-01

225

Congenital bronchopulmonary foregut malformations: concepts and controversies  

Microsoft Academic Search

This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions

Beverley Newman

2006-01-01

226

Isospectral flow and Liouville-Arnold integration in loop algebrast  

NASA Astrophysics Data System (ADS)

Some standard examples of Hamiltonian systems that are integrable by classical means are cast within the framework of isospectral flows in loop algebras. These include: the Neumann oscillator, the cubically nonlinear Schrödinger systems and the sine-Gordon equation. Each system has an associated invariant spectral curve and may be integrated via the Liouville-Arnold technique. The linearizing map is the Abel map to the associated Jacobi variety, which is deduced through separation of variables in hyperellipsoidal coordinates. More generally, a family of moment maps is derived, embedding certain finite dimensional symplectic manifolds, which arise through Hamiltonian reduction of symplectic vector spaces, into rational coadjoint orbits of loop algebras widetilde{mathfrak{g}}^+ ? widetilde{mathfrak{g}mathfrak{l}}(r)^+.Integrable Hamiltonians are obtained by restriction of elements of the ring of spectral invariants to the image of these moment maps; the isospectral property follows from the Adler-Kostant-Symes theorem. The structure of the generic spectral curves arising through the moment map construction is examined. Spectral Darboux coordinates are introduced on rational coadjoint orbits in widetilde{mathfrak{g}mathfrak{l}}(r)^{ + *}, and these are shown to generalize the hyperellipsoidal coordinates encountered in the previous examples. Their relation to the usual algebro-geometric data, consisting of linear flows of line bundles over the spectral curves, is given. Applying the Liouville-Arnold integration technique, the Liouville generating function is expressed in completely separated form as an abelian integral, implying the Abel map linearization in the general case.

Harnad, J.

227

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

228

Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres  

Microsoft Academic Search

Background\\/Aims: The results of liver transplantation for Budd-Chiari syndrome (BCS) are poorly known and the role and timing of the procedure are still controversial. The aim of this study was to investigate the results of transplantation for BCS, focusing on overall outcome, on prognostic factors and on the impact of the underlying disease. Methods: An enquiry on 248 patients representing

Gilles Mentha; Emiliano Giostra; Pietro E. Majno; Wolf O. Bechstein; Peter Neuhaus; John O'Grady; Raaj K. Praseedom; Andrew K. Burroughs; Yves P. Le Treut

229

Update on the classification, assessment of prognosis and therapy of Budd–Chiari syndrome  

Microsoft Academic Search

Budd–Chiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS—as fulminant,

Marco Senzolo; Evangelos C Cholongitas; David Patch; Andrew K Burroughs

2005-01-01

230

Arnold's web and diffusion in the Stark-Quadratic-Zeeman problem  

NASA Astrophysics Data System (ADS)

The Arnold web and the Arnold diffusion arise when an integrable Hamiltonian system is slightly perturbed: the first concerns the peculiar topology characterizing the set of the resonance lines in phase space, the latter the extremaly slow motion (if any) along these lines. While Arnold has proved the possibility of diffusion, it is still unknown if the phenomenon is generic in realistic physical systems. The system we consider is the Hydrogen atom (or Kepler problem) subject to the combined action of a constant electric and magnetic field, which is known as Stark-Zeeman problem. We describe the results of numerical experiments: the Arnold web is clearly highlighted and, looking at the behaviour of the KAM frequencies on orbits of 108 revolutions, evidence for the diffusion existence is reached.

Cordani, B.

2008-02-01

231

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY" with "project owner" for consistency with the Commission's current administrative format. It is Energy, 2005, the California Energy Commission received a petition from the Bottle Rock Power Corporation (BRPC

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 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

234

Generalizations of the Ermakov system through the Quantum Arnold Transformation  

NASA Astrophysics Data System (ADS)

An Ermakov system consists of a pair of coupled non-linear differential equations which share a joint constant of motion named Ermakov invariant. One of those equations, non-linear, is frequently referred to as the Ermakov-Pinney equation; the other equation may be thought of as describing a dynamical system: a harmonic oscillator with time-dependent frequency. In this paper, we revise the Quantum Arnold Transformation, a unitary operator mapping the solutions of the Schrödinger equation for time-dependent (even damped) harmonic oscillators, described by the Generalized Caldirola-Kanai equation, into solutions for the free particle. With this tool, we elucidate the existence of Ermakov-type invariants in classically linear systems at the classical and quantum levels. We also provide more general Ermakov-type systems and the corresponding invariants, together with a physical interpretation.

López-Ruiz, Francisco F.; Guerrero, Julio

2014-10-01

235

Optical image encryption using multilevel Arnold transform and noninterferometric imaging  

NASA Astrophysics Data System (ADS)

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

Chen, Wen; Chen, Xudong

2011-11-01

236

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

NASA Astrophysics Data System (ADS)

The Orobic basement (Southern Alps) mainly consists of pelitic and psammitc metasiliciclastic rocks with minor metagranitoids. A peculiar orthogneiss occurs at the top of Southalpine basement near the contact with Permo-Mesozoic cover rocks: the Gneiss Chiari del Corno Stella (or Gneiss Chiari). The Gneiss Chiari show a very homogeneous chemical composition similar to that of tourmaline leucogranites. They show a restricted silica range (75-80 wt%), high Al2O3 (>13 wt%) and alkali contents. They are poor in CaO (<0.35 wt%), MgO (<0.3 wt%), Fe2O3t (< 0.60wt%) and TiO2 ("0.05 wt%). Several characteristic and discriminant trace elements, such as Rb, Ba, Sr, Y and Zr have been chosen to underline the exceptional chemical composition and homogeneity of the granitic protholith of Gneiss Chiari. Although K2O is only 20 to 30% higher than in normal biotite granites, Rb is enriched more than 10%, reaching even 500 ppm. Rb is probably related to muscovite as well as to Kfs. The Gneiss Chiari are also characterized by high K/Rb and Rb/Zr ratios, low Ba, Sr and HFSE. In the Qtz-Ab-Or system, the Gneiss Chiari plot very close to the minimum in the range between 0.5-1.0 Kb, suggesting that they may have crystallized from a near 100%-liquid magma under water-saturated conditions at very high crustal levels. The deviation of some samples away from the minimum is probably a result of fractionation and therefore some samples do not represent the true bulk composition of the original granitic melt. In this system liquidus temperatures of these compositions ranges between 750-700 oC. The same range is obtdined from Zr saturation temperatures ( Watson &Harrison, 1983). Fractionation is dominated by Kfs and the best evidence for crystal fractionation consist of: (1) enrichment in Rb, Cs and depletion in Ba, Sr and Eu; (2) Sr and Ba both increase; (3) Rb/Sr ratios increase with decreasing Ba; (4) TiO2, LREE, Th, Ba, Sr, Y and Ce/Y decrease with decreasing Zr, whereas Rb/Sr and Sr/Ba increase. These trends also suggest that disturbance by late- or post-magmatic hydrothermal alteration is minimal. Y abundances are unusually high, though unevenly distributed. This element may be bound to apatite and to a smaller extent to tourmaline. All the samples exhibit low total REE and negative Eu anomaly, more pronounced with increasing Rb/Sr ratios. This features are consistent with the scarcity of zircon and monazite and suggest feldspars fractionation. The highly peraluminous and low-Ca composition of the Gneiss Chiari is typical of pelite-derived anatectic magma. The enrichment of the Gneiss Chiari in the incompatible elements K, Rb and Th strongly supports their crustal source. LILE (Rb, Sr and Ba) abundances in the Gneiss Chiari are compatible with their derivation by partial melting of pelites under fluid-absent condition implying dehydration melting of muscovite. A fluid-saturated melting regime, in which sufficient fluid was available to produce the maximum possible melt would produce a Sr enriched melt, which is not observed in Gneiss Chiari. It is important to emphasize that the critical melt fraction (>20%) does not represent the lowest melt fraction at which a melt may migrate, as small-degree partial melts may be efficiently separated from source by deformation-enhanced processes rather than their buoyancy alone.

Bergomi, M. A.; Boriani, A.

2003-04-01

237

[Hemangiomas and vascular malformations. Review and update].  

PubMed

In 1982, Mulliken and Glowacki classificated congenital vascular lesions in two biological groups with different treatment, evolution and prognosis: Haemangiomas and Vascular malformations. We have seen 230 patients, 145 with haemangiomas and 85 with vascular malformations (74 with low flow and 11 with high flow). We have reviewed clinical, evolutive and ecographic findings. Depending on the type and localization of the lesion, we completed the study with CT, MRI and angiography. Among all the patients with haemangiomas; 45 were treated with local or systemic steroids because of bleeding, ulceration or affection in functional areas. In two more, we used interferon alfa 2a because of Kassabach-Merritt Syndrome. 23 were operated on with good aesthetic results. Patients with vascular malformations were managed with embolization, sclerotherapy, surgical approach or conservatively. Capillary malformations were treated with lasertherapy. We emphasize in correct classification of vascular lesions prior to start diagnosis, treatment or parents information. PMID:9376236

Lobato, R; Martínez, L; Leal, N; Díaz, M; Díez-Pascual, R; Velasco, B; Ros, Z; López-Gutiérrez, J C

1997-07-01

238

MR imaging of fetal brain malformations  

Microsoft Academic Search

MethodsFrom the generally accepted data on the morphogenesis of the brain, the principles for the classification of brain malformations are given, and the salient features of each malformation which may be considered as independent from the developmental stage and therefore practical for MR imaging in the fetus after mid-gestation, are discussed.Results and discussionHowever, the correlation with the clinical results in

Charles Raybaud; Olivier Levrier; Hervé Brunel; Nadine Girard; Philippe Farnarier

2003-01-01

239

Interdisciplinary management of craniofacial vascular malformations  

Microsoft Academic Search

Beside their sometimes unsightly appearance, vascular malformations of the head and neck area can lead to very severe functional\\u000a problems. Reduced field of vision and impairments in breathing and swallowing can occur and are serious problems to deal with.\\u000a During the last 4 years, 17 patients with vascular malformations of the head and neck region were treated in our department.\\u000a Initially,

H. Kubiena; M. Cejna; S. Kreuzer; E. Frey; M. Schoder; M. Frey

2007-01-01

240

Animal models for human craniofacial malformations.  

PubMed

Holoprosencephaly malformations, of which the fetal alcohol syndrome appears to be a mild form, can result from medial anterior neural plate deficiencies as demonstrated in an ethanol treated animal model. These malformations are associated with more medial positioning of the nasal placodes and resulting underdevelopment or absence of the medial nasal prominences (MNPs) and their derivatives. Malformations seen in the human retinoic acid syndrome (RAS) can be produced by administration of the drug 13-cis-retinoic acid in animals. Primary effects on neural crest cells account for most of these RAS malformations. Many of the malformations seen in the RAS are similar to those of hemifacial microsomia, suggesting similar neural crest involvement. Excessive cell death, apparently limited to trigeminal ganglion neuroblasts of placodal origin, follows 13-cis retinoic acid administration at the time of ganglion formation and leads to malformations virtually identical to those of the Treacher Collins syndrome (TCS). Secondary effects on neural crest cells in the area of the ganglion appear to be responsible for the TCS malformations. Malformations of the DiGeorge Syndrome are similar to those of the RAS and can be produced in mice by ethanol administration or by "knocking out" a homeobox gene (box 1.5). Human and animal studies indicate that cleft lips of multifactorial etiology may be generically susceptible because of small MNP)s or other MNP developmental alterations, such as those found in A/J mice, that make prominence contact more difficult. Experimental maternal hypoxia in mice indicates that cigarette smoking may increase the incidence of cleft lip by interfering with morphogenetic movements. Other human cleft lips may result from the action of a single major gene coding for TGF-alpha variants. A study with mouse palatal shelves in culture and other information suggest that a fusion problem may be involved. PMID:1812129

Johnston, M C; Bronsky, P T

1991-01-01

241

Surgical treatment for venous malformation.  

PubMed

Sclerotherapy is generally the preferred treatment for venous malformation (VM) with surgery usually playing an adjunctive role. This study presents our experience with surgical treatment of VMs. Consecutive patients were identified from our vascular anomalies database 1996-2011 and patient demographics, location of the lesion, type of tissue(s) affected and symptoms were analysed. The patients completed a questionnaire to assess the impact of surgery on the severity of symptoms, appearance, function and overall quality of life (QoL), using a visual analogue scale of 0 (no symptom) to 10 (maximal symptom). They also rated their overall satisfaction of treatment using a scale of 0 (complete dissatisfaction) to 10 (complete satisfaction). Fifty patients with VM underwent a total of 58 procedures. Complication occurred in six patients (9.7% of operations), including transient sensory loss (n=3) and permanent frontal branch palsy (n=1), haematoma formation (n=1) and minor wound dehiscence (n=1). At least 50% improvement in symptoms of background pain, acute episodic pain, contour deformity and skin discolouration occurred in 88.9%, 92.3%, 83.3% and 75.0% of patients, respectively. At least 50% improvement in the appearance, function and overall QoL occurred in 54.3%, 71.4% and 70.4% of patients, respectively. The mean overall patient satisfaction with the treatment was 8.9 (range, 1-10). Surgery remains an important treatment modality for selected patients with VM having low complication rates and high patient satisfaction. It improves the appearance, function and overall QoL for the majority of the patients by reducing the severity of pain, contour deformity and skin discolouration. PMID:24012651

Steiner, Frederica; FitzJohn, Trevor; Tan, Swee T

2013-12-01

242

[High flow vascular malformations in children].  

PubMed

Unlike hemangiomas and low-flow vascular malformations which are very common in children, arterial anomalies have small incidence. Differential diagnosis is difficult, and needs a physician familiarized with vascular anomalies. Appropriate treatment must be planned by multidisciplinary team considering the patient's age, and anatomical location. Twenty-eight children with high flow vascular malformations have been treated since 1990 at La Paz Children's Hospital Vascular Anomalies Program. We excluded of the study group patients with central nervous system lesions. 85% of the patients had malformation in stage I or II (according the ISSVA accepted Schöbinger stating) and most of them were erroneously diagnosed as hemangioms with a variety of inappropriate treatments previously performed. Doppler Ultrasound and Magnetic Resonance confirmed malformation flow and extension. Angiography and selective embolization was only considered as therapeutic approach in candidates to surgical resection. 16 patients underwent complete resection of the malformation including one foot and two fingers amputation and five more incomplete resection of the ulcerate area. In conclusion, we did not find age at onset, sex and symptoms relationship. Laser, radiotherapy, surgical ligation or partial resection must be considered inappropriate therapies which may stimulate AVM exacerbation. Only radical surgical procedure after selective endovascular embolization will be successful but then reconstructive surgery should be performed to achieve good aesthetic and functional results. PMID:12601971

López Gutiérrez, J C; Ros, Z; Martínez, L; Díaz, M; Leal, N; Rivas, S; Hernández, F

2002-10-01

243

Plasma Propulsion Testing Capabilities at Arnold Engineering Development Center  

NASA Technical Reports Server (NTRS)

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

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

2007-01-01

244

Distant skeletal muscle metastasis from intrahepatic cholangiocarcinoma presenting as Budd-Chiari syndrome  

PubMed Central

Intrahepatic cholangiocarcinoma is a malignant neoplasm arising from the biliary epithelium, which frequently invades adjacent organs or metastasizes to other visceral organs such as the lungs, bones, adrenals, and brain. However, distant skeletal muscle metastasis of cholangiocarcinoma has never been described before to the best of our knowledge and, furthermore, Budd-Chiari syndrome secondary to intrahepatic cholangiocarcinoma is also extremely rare. Here we present the first case overall of distant muscle metastasis from intrahepatic cholangiocarcinoma presenting as Budd-Chiari syndrome. A 44-year-old man admitted to the hospital with complaints of abdominal distension, edema of both legs, back pain and anorexia of 30 d' duration. Computed tomography and ultrasonography-guided percutaneous muscle biopsy established intrahepatic cholangiocarcinoma with disseminated thrombosis from inferior vena cava to bilateral iliac and femoral veins, and multiple skeletal muscle metastases in bilateral buttock and erector spinal muscle. PMID:17589935

Kwon, Oh Sung; Jun, Dae Won; Kim, Sang Heum; Chung, Mee Yeon; Kim, Nam In; Song, Moon Hee; Lee, Han Hyo; Kim, Seung Hwan; Jo, Yoon Ju; Park, Young Sook; Joo, Jong Eun

2007-01-01

245

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

246

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

247

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

SciTech Connect

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

Strunk, Holger M.; Textor, Jochen [University of Bonn, Department of Radiology (Germany); Brensing, Karl-August [University of Bonn, Department of Internal Medicine (Germany); Schild, Hans H. [University of Bonn, Department of Radiology (Germany)

1997-07-15

248

Congenital malformations and testicular germ cell tumors  

PubMed Central

Cryptorchidism is one of the few known risk factors for testicular germ cell tumors (TGCT). It has been postulated that other congenital malformations, in particular hypospadias, are also associated with increased risk; however, associations with birth defects have not been extensively studied. Using Swedish population-based registries we evaluated the relationship between birth defects and risk of TGCT. TGCT cases (n=6,593) diagnosed between 15 and 65 years of age were identified from the Swedish Cancer Registry between 1964 and 2008. Five controls per case were randomly selected from the population register and matched on birth year and birth county. Congenital malformations were identified via linkage with the Hospital Discharge Register. Odds ratios (OR) and 95% confidence intervals (CI) for the association between each group of malformations and TGCT were estimated using conditional logistic regression. In addition to the expected association between cryptorchidism and TGCT risk [OR (95% CI): 3.18 (2.50 to 4.04)], hypospadias [2.41 (1.27 to 4.57)], inguinal hernia [1.37 (1.11–1.68)] and other genital malformations [2.19 (1.17 to 4.10)] were associated with an increased risk of TGCT. Mutual adjustment for cryptorchidism, hypospadias, inguinal hernia, and other genital malformations did not appreciably change the associations (ORs: 3.16; 2.25; 1.30; 1.90, respectively). The other (non-genital) malformations evaluated were not associated with TGCT. These data suggest that developmental urogenital abnormalities, specifically cryptorchidism, hypospadias, and inguinal hernia, are associated with an increased risk of TGCT; further supporting the hypothesis that prenatal exposure(s) related to proper genital development are related to this tumor. PMID:23580254

Trabert, Britton; Zugna, Daniela; Richiardi, Lorenzo; McGlynn, Katherine A; Akre, Olof

2014-01-01

249

Percutaneous Cryotherapy of Vascular Malformation: Initial Experience  

SciTech Connect

The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

Cornelis, F., E-mail: francoiscornelis@hotmail.com [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France)

2013-06-15

250

Extrapyramidal dysfunction with cerebral arteriovenous malformations 1  

PubMed Central

Arteriovenous malformations have only rarely been implicated as a cause of basal ganglia dysfunction. In four instances where such a lesion was uncovered, abnormal involuntary movements were present. In two, tremor involving the contralateral limbs occurred, while in others the head and neck were involved in dystonic movements and posture. The clinical and angiographic characteristics of these four patients have been assessed and are presented in detail in this report. The possible mechanism by which arteriovenous malformations may disturb the internal circuitry of the basal ganglia and induce symptoms are discussed. Images PMID:4829531

Lobo-Antunes, Joao; Yahr, Melvin D.; Hilal, Sadek K.

1974-01-01

251

Image encryption based on singular value decomposition and Arnold transform in fractional domain  

NASA Astrophysics Data System (ADS)

In this paper, we have proposed a new image encryption algorithm based on singular value decomposition and Arnold transform. An original image is first transformed in fractional domain by FRFT, and then decomposed into three segments by SVD. All these three parts are Arnold transformed different number of times to enhance the security. The three encoded images can be kept in several places or assigned to a group of authorized persons. But all the three parts should be provided in the decryption, and inverse Arnold transformed corresponding times, multiplied in correct order and inverse fractional Fourier transformed with right fractional orders, and finally the total correct information can be reconstructed. Otherwise, people cannot obtain the information easily. Some computer simulations are presented to verify the possibility.

Chen, Linfei; Zhao, Daomu; Ge, Fan

2013-03-01

252

Segmental colonic motility in patients with anorectal malformations  

Microsoft Academic Search

Background: Constipation is one of the most important functional sequelae in patients with anorectal malformations. The cause of this motility disorder is unknown. Aim: The purpose of this study was to assess total colonic transit time (TCT) and segmental colonic transit time (SCT) in patients with anorectal malformations. Method: Ninety patients with anorectal malformations (40 low and 50 high; median

R. J Rintala; E Marttinen; K Virkola; M Rasanen; C Baillie; H Lindahl

1997-01-01

253

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

254

Differential Gene Expression in Human Cerebrovascular Malformations  

PubMed Central

OBJECTIVE We sought to identify genes with differential expression in cerebral cavernous malformations (CCMs), arteriovenous malformations (AVMs), and control superficial temporal arteries (STAs) and to confirm differential expression of genes previously implicated in the pathobiology of these lesions. METHODS Total ribonucleic acid was isolated from four CCM, four AVM, and three STA surgical specimens and used to quantify lesion-specific messenger ribonucleic acid expression levels on human gene arrays. Data were analyzed with the use of two separate methodologies: gene discovery and confirmation analysis. RESULTS The gene discovery method identified 42 genes that were significantly up-regulated and 36 genes that were significantly down-regulated in CCMs as compared with AVMs and STAs (P = 0.006). Similarly, 48 genes were significantly up-regulated and 59 genes were significantly down-regulated in AVMs as compared with CCMs and STAs (P = 0.006). The confirmation analysis showed significant differential expression (P < 0.05) in 11 of 15 genes (angiogenesis factors, receptors, and structural proteins) that previously had been reported to be expressed differentially in CCMs and AVMs in immunohistochemical analysis. CONCLUSION We identify numerous genes that are differentially expressed in CCMs and AVMs and correlate expression with the immunohistochemistry of genes implicated in cerebrovascular malformations. In future efforts, we will aim to confirm candidate genes specifically related to the pathobiology of cerebrovascular malformations and determine their biological systems and mechanistic relevance. PMID:12535382

Shenkar, Robert; Elliott, J. Paul; Diener, Katrina; Gault, Judith; Hu, Ling-Jia; Cohrs, Randall J.; Phang, Tzulip; Hunter, Lawrence; Breeze, Robert E.; Awad, Issam A.

2009-01-01

255

Split Cord Malformations of the Lumbar Region  

Microsoft Academic Search

From a group of 84 patients with split cord malformations presenting to our Department between 1976 and 1990, we have selected 47 cases in whom the split cord was confined to the lower dorsal-lumbar region and in whom there were no other dysraphic features such as meningocele, lipoma or dermoid cyst. We have studied these cases of ‘pure split cord

U. B Andar; W. F. J. Harkness; R. D. Hayward

1997-01-01

256

Pancreatic arteriovenous malformation with duodenal ulcer  

Microsoft Academic Search

Summary We report the color Doppler ultrasonography features of arteriovenous malformation (AVM) of the pancreas, a very rare disease. The patient was a 52-year-old man with congenital AVM of the pancreas and a duodenal ulcer that had been resistant to medication. Endoscopic color Doppler ultrasonography (color Doppler EUS) revealed many abnormal color signals showing pulsatile wave form at the portion

Shigeo Tano; Norio Ueno; Tomio Ueno; Shin-Ichi Wada; Toshiyuki Aizawa; Ken Kimura

1996-01-01

257

Body growth in urinary tract malformations  

Microsoft Academic Search

Body height and height velocity were analysed in 54 children with obstructive urinary tract malformations over a mean period of 8.7 years, using new auxological methods. At the time of diagnosis, 9% of patients had a height of more than 2 standard deviations below the normal mean. Mean relative height changed significantly from the first to the last observation, the

C. Seidel; F. Schaefer; K. Schärer

1993-01-01

258

A 6-year experience treating vascular malformations with foam sclerotherapy.  

PubMed

In this study, the authors present an analysis of the outcomes of 105 low-flow vascular malformation patients treated over a 6-year period and report specific lesion characteristics that correlate with those vascular malformations that will benefit from sodium tetradecyl sulfate foam sclerotherapy (STS FS) versus surgical resection as well as morphological characteristics of vascular malformations that are associated with a poor response to FS treatment. Improvement in symptoms was documented in 92.9% of patients treated with STS FS. There were no complications. Low-flow vascular malformations that were morphologically characterized by microcystic, septated vessels did not respond to FS, and these vascular malformations are best treated with surgical resection. Primary surgical resection is also the treatment of choice for localized, microcystic, and superficial low-flow vascular malformations. Symptomatic, diffuse, extensive, macrocystic malformations that involve multiple tissue planes and vital structures are best treated with FS. PMID:22918935

Markovic, Jovan N; Kim, Charles Y; Lidsky, Michael E; Shortell, Cynthia K

2012-06-01

259

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

260

Security impact ratings considered harmful Jeff Arnold, Tim Abbott, Waseem Daher, Gregory Price,  

E-print Network

Security impact ratings considered harmful Jeff Arnold, Tim Abbott, Waseem Daher, Gregory Price In this paper, we question the common practice of as- signing security impact ratings to OS updates. Specifi easily elude developers, so that the true security implications of bugs are commonly not discovered un

261

Aerodynamic Stability and the Growth of Triangular Snow Crystals K. G. Libbrecht and H. M. Arnold  

E-print Network

Aerodynamic Stability and the Growth of Triangular Snow Crystals K. G. Libbrecht and H. M. Arnold Department of Physics, California Institute of Technology Pasadena, California 91125 Keywords: Crystal growth, crystal morphology, ventilation effect, diffusion-limited growth, growth instabilities, crystal faceting

Libbrecht, Kenneth G.

262

HEATING EVENTS IN THE QUIET SOLAR CORONA ARNOLD O. BENZ and SM KRUCKER  

E-print Network

HEATING EVENTS IN THE QUIET SOLAR CORONA ARNOLD O. BENZ and SÃ?M KRUCKER Institute of Astronomy, ETH originating in the corona are presented. The thermal radiation of the quiet corona is found to fluctutate that the brightenings are caused by additional plasma injected from below and heated to slightly higher temperature than

263

Heating Events in the Quiet Solar Corona Arnold O. Benz and Sam Krucker  

E-print Network

Heating Events in the Quiet Solar Corona Arnold O. Benz and S¨am Krucker Institute of Astronomy for heating (Shimizu et al. 1994). In the quiet corona (excluding active regions and coronal holes per second over the whole Sun. Their estimated energy #12; Heating Events in the Quiet Solar Corona 3

264

ENERGY DISTRIBUTION OF MICROEVENTS IN THE QUIET SOLAR CORONA Arnold O. Benz  

E-print Network

of information on the heating process of the corona. Subject headings: Sun: activity -- Sun: corona -- Sun: flares -- Sun: X-rays, gamma rays 1. INTRODUCTION The heating of the solar corona has been a riddle sinceENERGY DISTRIBUTION OF MICROEVENTS IN THE QUIET SOLAR CORONA Arnold O. Benz Institute of Astronomy

265

Steadystate solutions in a nonlinear pool boiling model Michel Speetjens # , Arnold Reusken # , Wolfgang Marquardt +  

E-print Network

Steady­state solutions in a nonlinear pool boiling model Michel Speetjens # , Arnold Reusken a relatively simple model for pool boiling processes. This model in­ volves only the temperature distribution within the heater and desribes the heat exchange with the boiling fluid via a nonlinear boundary

266

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

, and reduces air pollution impacts on environmental justice communities. Local air districts administerSTATE OF CALIFORNIA ­ THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY (08-AFC-4) DATA REQUESTS 1 to 73 (SET #1) Pursuant to Title 20, California Code of Regulations

267

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

and to the Monterey Bay Unified Air Pollution Control District (District) Permit to Operate and Title V OperatingSTATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY of Staff Analysis for Modifications to Air Emission Monitoring Requirements On April 25, 2005

268

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

269

On analytical applications of stable homotopy (the Arnold conjecture, critical points)  

Microsoft Academic Search

We prove the Arnold conjecture for closed symplectic manifolds with\\u000a$\\\\pi_2(M)=0$ and $\\\\cat M=\\\\dim M$. Furthermore, we prove an analog of the\\u000aLusternik-Schnirelmann theorem for functions with ``generalized hyperbolicity''\\u000aproperty.

Yuli B. Rudyak

1999-01-01

270

Fixed point theorems in the Arnol'd model about instability of the actionvariables in phasespace  

E-print Network

tools suggested by Arnol'd i.e. the contraction mapping method togheter with the ``conical metric = R=2�Z); '' and ¯ are real parameters. Physically H represents a pendulum ( 1 2 I 2 1 + ''(cos ' 1 and ¯ = 0; following [A], we consider the motions that ``develop along the separatrix of the pendulum''. We

Perfetti, Paolo

271

ENDOWED CHAIR POSITION University of South Carolina, Arnold School of Public Health,  

E-print Network

ENDOWED CHAIR POSITION University of South Carolina, Arnold School of Public Health, Department of Exercise Science The University of South Carolina (USC) seeks to hire an endowed chair of Technology School of Public Health (http://sph.sc.edu), Department of Exercise Science. This endowed chair is one

Almor, Amit

272

Opening Remarks by George W. Arnold, National Coordinator for Smart Grid Interoperability  

E-print Network

Opening Remarks by George W. Arnold, National Coordinator for Smart Grid Interoperability Conference on Smart Grid Interoperability Standards January 31, 2011 Introduction Chairman Wellinghoff the opportunity to escribe NIST's and our partners efforts to develop standards for an interoperable d smart grid

Magee, Joseph W.

273

EL Program: Smart Grid Program Manager: George Arnold, Designated Goal Liaison; David Wollman,  

E-print Network

EL Program: Smart Grid Program Manager: George Arnold, Designated Goal Liaison; David Wollman, Smart Grid and Cyber-Physical Systems Program Office, Engineering Laboratory Office, x2433; Dean and power flows, and additional advancements to create a smart grid. In response to a mandate given

Magee, Joseph W.

274

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

STATE OF CALIFORNIA ­ THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION 1516 NINTH STREET SACRAMENTO, CA 95814-5512 www.energy.ca.gov TO: All Parties RE: Orange Grove for the above-mentioned project, current as of October 27, 2008. The new addition(s) to the Proof of Service

275

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY of Regulations, section 1769). Energy Commission staff is currently analyzing the request and will publish COMMISSION 1516 NINTH STREET SACRAMENTO, CA 95814-5512 NOTICE OF RECEIPT PETITION TO AMEND THE ENERGY

276

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY COMMISSION  

E-print Network

STATE OF CALIFORNIA THE RESOURCES AGENCY ARNOLD SCHWARZENEGGER, Governor CALIFORNIA ENERGY). Energy Commission staff is currently analyzing the request and will publish an analysis in the near COMMISSION 1516 NINTH STREET SACRAMENTO, CA 95814-5512 NOTICE OF RECEIPT PETITION TO AMEND THE ENERGY

277

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

278

Evolution, Game Theory, & the Social Contract Arnold and Mabel Beckman Center  

E-print Network

1 Evolution, Game Theory, & the Social Contract Conference Arnold and Mabel Beckman Center 100 of trickery. We see the former category in the evolution of mate-choice signals; we see the latter in the evolution of immune strategies to deal with pathogens. I will discuss the problem of deception in biological

Barrett, Jeffrey A.

279

Singing Through the Trumpet: Arnold Jacobs’ ‘Song and Wind’ and the Trumpet  

Microsoft Academic Search

As with any instrument, performing successfully on a brass instrument is a challenging task that many musicians, including professionals, have encountered for decades. In an attempt to find a solution to these challenges, Arnold Jacobs, principal tubist for the Chicago Symphony Orchestra from 1944-1988, spent many years poring over neurology and physiology books, analyzing the playing of fellow members of

Erin Johnson

2012-01-01

280

FINITE ELEMENTS FOR SYMMETRIC TENSORS IN THREE DOUGLAS N. ARNOLD, GERARD AWANOU, AND RAGNAR WINTHER  

E-print Network

PREPRINT FINITE ELEMENTS FOR SYMMETRIC TENSORS IN THREE DIMENSIONS DOUGLAS N. ARNOLD, GERARD AWANOU, AND RAGNAR WINTHER Abstract. We construct finite element subspaces of the space of symme- tric tensors- dard discontinuous finite element spaces are used to approximate the displace- ment field. These finite

Awanou, Gerard

281

Integrity Under Attack: The State of Scholarly Publishing Douglas N. Arnold, SIAM President  

E-print Network

to produce the impression of a serious scholarly journal without the substance. Another is the deliberateIntegrity Under Attack: The State of Scholarly Publishing Douglas N. Arnold, SIAM President To appear in SIAM News, December 2009. Scientific journals are surely important. They provide the most

Wild, Marcel

282

MIXED FINITE ELEMENTS FOR ELASTICITY ON QUADRILATERAL DOUGLAS N. ARNOLD, GERARD AWANOU, AND WEIFENG QIU  

E-print Network

MIXED FINITE ELEMENTS FOR ELASTICITY ON QUADRILATERAL MESHES DOUGLAS N. ARNOLD, GERARD AWANOU, AND WEIFENG QIU Abstract. We present stable mixed finite elements for planar linear elasticity on general r for all the variables. The methods use Raviart­Thomas elements for the stress, piecewise tensor

Arnold, Douglas N.

283

Embolization and radiosurgery for arteriovenous malformations  

PubMed Central

The treatment of arteriovenous malformations (AVMs) requires a multidisciplinary management including microsurgery, endovascular embolization, and stereotactic radiosurgery (SRS). This article reviews the recent advancements in the multimodality treatment of patients with AVMs using endovascular neurosurgery and SRS. We describe the natural history of AVMs and the role of endovascular and radiosurgical treatment as well as their interplay in the management of these complex vascular lesions. Also, we present some representative cases treated at our institution. PMID:22826821

Plasencia, Andres R.; Santillan, Alejandro

2012-01-01

284

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

285

Dandy-Walker malformation: An incidental finding  

PubMed Central

Dandy–Walker malformation (DWM) is a rare intracranial congenital abnormality that affects the cerebellum and some of its components; particularly cerebellar vermis, fourth ventricle and is characterized by an enlarged posterior fossa. Although there is an extensive list of signs attributed to DWM, final diagnosis is solely dependent on imaging techniques as there are no signs that are characteristic of DWM. This article reports a case with DWM who was diagnosed by magnetic resonance imaging. PMID:20838490

Tadakamadla, Jyothi; Kumar, Santhosh; Mamatha, G. P.

2010-01-01

286

Neuroembryology and brain malformations: an overview.  

PubMed

Modern neuroembryology integrates descriptive morphogenesis with more recent insight into molecular genetic programing and data enabled by cell-specific tissue markers that further define histogenesis. Maturation of individual neurons involves the development of energy pumps to maintain membrane excitability, ion channels, and membrane receptors. Most malformations of the nervous system are best understood in the context of aberrations of normal developmental processes that result in abnormal structure and function. Early malformations usually are disorders of genetic expression along gradients of the three axes of the neural tube, defective segmentation, or mixed lineages of individual cells. Later disorders mainly involve cellular migrations, axonal pathfinding, synaptogenesis, and myelination. Advances in neuroimaging now enable the diagnosis of many malformations in utero, at birth, or in early infancy in the living patient by abnormal macroscopic form of the brain. These images are complimented by modern neuropathological methods that disclose microscopic, immunocytochemical, and subcellular details beyond the resolution of MRI. Correlations may be made of both normal and abnormal ontogenesis with clinical neurological and EEG maturation in the preterm or term neonate for a better understanding of perinatal neurological disease. Precision in terminology is a key to scientific communication. PMID:23622157

Sarnat, Harvey B; Flores-Sarnat, Laura

2013-01-01

287

Gynecologic concerns in patients with anorectal malformations.  

PubMed

Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an imperforate anus with a rectovestibular fistula, may seem straightforward, treated with a posterior sagittal anorectoplasty; however, it is vital to recognize the association with gynecologic anomalies. Girls with the most complex anorectal defect, cloacal malformation, have significant associated urological and gynecologic anomalies, which should be recognized and treated. An opportunity to diagnose and treat such anomalies may be missed in the newborn period or at the definitive surgery, unless the pediatric surgeon is aware of this association. With the knowledge of the associated anomalies and long-term sequelae, surgeons can provide better care for girls and important counseling for parents. Important gynecologic issues to consider may be divided into several categories, such as infancy, puberty, sexual intimacy, and obstetrical concerns. Knowledge of reproductive-related issues in females with ARMs allows the pediatric surgeon and their gynecology colleagues to provide optimal surgical management throughout infancy, childhood, and into young adulthood. Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility. PMID:20307850

Breech, Lesley

2010-05-01

288

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. PMID:18472484

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

289

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

PubMed

Alveolar echinococcosis of the liver is caused by the tapeworm Echinococcus multilocularis. Budd-Chiari Syndrome (BCS) is a hepatic venous outflow tract obstruction and involves abdominal pain, hepatomegaly and high-gradient ascites. A low-gradient ascites in connection with the syndrome rarely occurs in case of alveolar echinococcosis of the liver. Here we report a 20-year-old man with the syndrome and low-gradient ascites due to a huge liver mass. Further diagnostic examination revealed alveolar echinococcosis of the liver. Diuretic agents and albendazole were used as palliative therapy in the case. PMID:24864647

Karadas, Sevdegul; Cumhur, Dulger Ahmet; Bilge, Gultepe; Mehmet, Beyazal; Mustafa, Kosem

2014-04-01

290

Vascular malformations of the gastrointestinal tract.  

PubMed

The advent of fiberoptic endoscopy, which became widespread in the evaluation of gastrointestinal bleeding throughout the late 1970s and 1980s, has dramatically changed both our understanding of the extent to which vascular malformations account for gastrointestinal blood loss and our ability to treat these lesions at the time of diagnosis. Colonic vascular malformations appear to be the single most common cause of acute or recurrent gastrointestinal bleeding episodes in patients over 60 years of age, being responsible for the bleeding in as many as 35% of such patients. Although less common as a cause of upper gastrointestinal bleeding, these lesions still account for 2% to 5% of bleeding lesions in older patients. Diagnosis is accomplished by endoscopy, and the vascular malformations can then be coagulated via the endoscope using one of a number of thermal systems. The argon laser, the heater probe, and the BICAP system are all effective and safe throughout the gastrointestinal tract, especially in the cecum and right colon, where the majority of sporadic vascular malformations occur. Monopolar cautery and the Nd:YAG laser are equally efficacious, but their greater and less predictable depth of coagulation make them much less safe in the cecum and right colon. There are no apparent advantages in terms of efficacy and safety between laser treatment and the other thermal modalities. The laser has the advantage of being quicker, which is especially important when treating large or multiple lesions. The other modalities have the advantages of portability and low relative cost. Endoscopic therapy with lasers or other thermal devices is nonspecific. The effects are achieved by thermally coagulating the mucosal vascular lesions, allowing the coagulated tissue to slough, and leaving a mucosal ulceration that subsequently heals with re-epithelialization. Endoscopic coagulation has thus been reported effective in the treatment of gastrointestinal mucosal vascular lesions regardless of their etiology or characteristics. It has been effective for sporadic vascular malformations, hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), radiation proctocolitis, the blue rubber-bleb nevus syndrome, and diffuse gastric antral vascular ectasia (the watermelon stomach). As we move through the 1990s and beyond, these endoscopic modalities offer an effective, relatively safe, and clearly less invasive treatment option for the many patients who experience acute, recurrent, or chronic gastrointestinal bleeding from any of these lesions. PMID:1589831

Buchi, K N

1992-06-01

291

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; Catão-Dias, J L

2013-11-01

292

Malformations Among the X-Linked Intellectual Disability Syndromes  

PubMed Central

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. PMID:24166814

Stevenson, Roger E.; Schwartz, Charles E.; Rogers, R. Curtis

2013-01-01

293

[Budd-Chiari syndrome induced by hormonal oral contraception in the patient with congenital thrombophilia-factor V Leiden mutation--a case report].  

PubMed

The Budd-Chiari syndrome is a rare pathology resulting from various etiological factors which often contribute to its late diagnosis. Liver cirrhosis, malignant tumors and haematological disorders resulting in hypercoagulability, are the most common reasons of Budd-Chiari syndrome. The syndrome is characterized by portal hypertension and splanchnic congestion due to obstruction of hepatic venous outflow. The first symptoms include pain, ascites and hepatosplenomegaly. The diagnosis of Budd-Chiari syndrome can be achieved by Doppler ultrasonography, Computed Tomography scan, Magnetic Resonance or Single Photon Emission Computed Tomography. In the following article, a case report of a patient with diagnosed Budd-Chiari syndrome as a result of congenital thrombophilia-factor V Leiden gene mutation is presented. Clinical symptoms, diagnostic process, as well as treatment options, were shown in the article. PMID:19058526

Samborek, Ma?gorzata; Drosdzol, Agnieszka; Stojko, Rafa?; Wilk, Krzysztof; Witek, Andrzej

2008-10-01

294

Epileptogenic brain malformations: clinical presentation, malformative patterns and indications for genetic testing  

Microsoft Academic Search

We review here those malformations of the cerebral cortex which are most often observed in epilepsy patients, for which a genetic basis has been elucidated or is suspected and give indications for genetic testing.There are three forms of lissencephaly (agyria-pachygyria) resulting from mutations of known genes, which can be distinguished because of their distinctive imaging features. They account for about

Romeo Carrozzo

2001-01-01

295

Laser treatment of oral vascular malformations  

NASA Astrophysics Data System (ADS)

Oral Vascular Malformations (OVM) are congenital anomalies characterized by morph-structural and/or functional changes of nature in severity and extension. OVM can affect any type of vessels arterial, venous or lymphatic and any capillary or anatomical. They are divided into two categories: low and high flow. In this study were treated 40 patients with OVM with a range size from 2 mm to 44 mm; they were subjected to clinical examination supported by Colour-Doppler Ultrasound instrumental examination and only for doubt cases the Magnetic Resonance Imaging (MRI) was prescribed. Only low flow venous and capillary malformations were treated by GaAlAs laser (Wiser®, Lambda, Brindole,Italy, 980nm) and KTP laser (SmartLite®, DEKA, Florence, Italy, 532nm) with two different techniques: the Transmucosal Thermophotocoagulation (TMT) and the Intralesional Photocoagulation (ILP). These techniques permitted a good control of haemostasis, avoiding bleeding both during surgery and in the postoperative. It is obtained an excellent and good healing respectively in 10% and 60% of cases, a moderate and poor resolution respectively in 22.5% and 7.5% of cases. A clear diagnosis allowed the management of Venous malformations (VM) by laser devices with wavelengths highly absorbed in haemoglobin in safety and efficacy and according to the principles of minimal invasive surgery. The aim of this study was to verify if the laser is effective in the treatment of OVM for the purpose of the clinical findings and the postoperative course. The Authors concluded that the laser can be considered the "gold standard" for treating OVM.

Romeo, U.; Gaimari, G.; Mohsen, M.; Tenore, G.; Palaia, G.

2014-01-01

296

On Arnold's 14 `exceptional' mathcal{N} = 2 superconformal gauge theories  

NASA Astrophysics Data System (ADS)

We study the four-dimensional superconformal mathcal{N} = 2 gauge theories engineered by the Type IIB superstring on Arnold's 14 exceptional unimodal singularities ( a.k.a. Arnold's strange duality list), thus extending the methods of arXiv:1006.3435 to singularities which are not the direct sum of minimal ones. In particular, we compute their BPS spectra in several `strongly coupled' chambers. From the TBA side, we construct ten new periodic Y-systems, providing additional evidence for the existence of a periodic Y-system for each isolated quasi-homogeneous singularity with ? < 2 (more generally, for each mathcal{N} = 2 superconformal theory with a finite BPS chamber whose chiral primaries have dimensions of the form {{mathbb{N}} left/ {ell } right.} ).

Cecotti, Sergio; Del Zotto, Michele

2011-10-01

297

Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd-Chiari syndrome  

Microsoft Academic Search

Background\\/Aims: Hepatic vein thrombosis (Budd-Chiari syndrome) is associated with various hypercoagulable states, such as polycythemia vera (PV), presence of the lupus anticoagulant, paroxysmal nocturnal hemoglobinuria (PNH) and deficiencies of antithrombin III, protein C and protein S. In recent years, it has become evident that patients with the Budd-Chiari syndrome may have more than one risk factor that may cause a

Boaz Hirshberg; Daniel Shouval; Eitan Fibach; Gideon Friedman; Dina Ben-Yehuda

2000-01-01

298

Stereotactic radiosurgery of intracranial arteriovenous malformations.  

PubMed

Stereotactic radiosurgery for intracranial arteriovenous malformations (AVMs) has been performed since the 1970s. When an AVM is treated with radiosurgery, radiation injury to the vascular endothelium induces the proliferation of smooth muscle cells and the elaboration of extracellular collagen, which leads to progressive stenosis and obliteration of the AVM nidus. Obliteration after AVM radiosurgery ranges from 60% to 80%, and relates to the size of the AVM and the prescribed radiation dose. The major drawback of radiosurgical AVM treatment is the risk of bleeding during the latent period (typically 2 years) between treatment and AVM thrombosis. PMID:24093574

Friedman, William A

2013-10-01

299

Surgical management of pediatric cerebral arteriovenous malformations  

Microsoft Academic Search

Background  Arteriovenous malformations (AVMs) are the most common cause of intracerebral hemorrhage in children. Different options exist\\u000a for their successful management consisting of surgery, endovascular embolization, stereotactic radiosurgery, or a combination\\u000a of these treatments.\\u000a \\u000a \\u000a \\u000a \\u000a Discussion  In this paper, we discuss the different treatment modalities in the treatment of pediatric cerebral AVMs emphasizing the role\\u000a of surgery and endovascular embolization as a preoperative

David Rubin; Alejandro Santillan; Jeffrey P. Greenfield; Mark Souweidane; Howard A. Riina

2010-01-01

300

Toward postnatal reversal of ocular congenital malformations.  

PubMed

Aniridia is a panocular disorder that severely affects vision in early life. Most cases are caused by dominantly inherited mutations or deletions of the PAX6 gene, which encodes a transcription factor that is essential for the development of the eye and the central nervous system. In this issue of the JCI, Gregory-Evans and colleagues demonstrate that early postnatal topical administration of an ataluren-based formulation reverses congenital malformations in the postnatal mouse eye, providing evidence that manipulation of PAX6 after birth may lead to corrective tissue remodeling. These findings offer hope that ataluren administration could be a therapeutic paradigm applicable to some major congenital eye defects. PMID:24355915

Sahel, José-Alain; Marazova, Katia

2014-01-01

301

[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

302

Fixed point theorems in the Arnol'd model about instability of the actionvariables in phasespace  

E-print Network

tools suggested by Arnol'd i.e. the contraction mapping method togheter with the ``conical metric = R=2�Z); '' and ¯ are real parameters. Physically H represents a pendulum ( 1 2 I 2 1 + ''(cos ' 1 of the pendulum''. We therefore write the equations H 1 (I 1 ; ' 1 ) = 1 2 I 2 1 + ''(cos ' 1 \\Gamma 1) = 0; H 2

303

Bifurcations of circle maps: Arnol'd tongues, bistability and rotation intervals  

Microsoft Academic Search

We study the bifurcations of two parameter families of circle maps that are similar tofb,w(x)=x+w+(b\\/2p) sin (2px) (mod1). The bifurcation diagram is constructed in terms of setsTr, whereTr is the set of parameter values (b, w) for whichfb, w has an orbit with rotation numberr. We show that the known structure whenbr rational,Tr is an Arnol'd tongue and forr irrational,

Philip L. Boyland

1986-01-01

304

On a problem of Arnold: the average multiplicative order of a given integer  

E-print Network

For g,n coprime integers, let l_g(n) denote the multiplicative order of g modulo n. Motivated by a conjecture of Arnold, we study the average of l_g(n) as n the Generalized Riemann Hypothesis, we show that this average is essentially as large as the average of the Carmichael lambda function. We also determine the asymptotics of the average of l_g(p) as p <= x ranges over primes.

Kurlberg, Par

2011-01-01

305

Scanning electron microscopic studies on floral malformation in mango  

Microsoft Academic Search

Studies with scanning electron microscope in mango variety Amrapali revealed the presence of many hair line cracks, pin-sized to large holes, disorganised cells and fungal mycelial infection at the base of the malformed bud during bud-inception stages. Among other possible reasons, tissue damage caused by the fungi and sucking pests may lead to the abnormal development of the malformed tissues.

K. Usha; A. M. Goswami; H. C. Sharma; Bhupinder Singh; P. C. Pande

1997-01-01

306

Pulmonary arteriovenous malformation: a rare anterior mediastinal mass.  

PubMed

Pulmonary arteriovenous malformations are rare pulmonary vascular lesions which are associated with Osler Weber Rendu syndrome (hereditary haemorrhagic telangectasia). They act as right-to-left shunts and have cardiovascular and embolic complications. We present a patient with an apparent anterior mediastinal mass secondary to a pulmonary arteriovenous malformations which was successfully treated percutaneously. PMID:23043574

Walklin, Ryan Patrick; Entwisle, James; Cheung, Ying Kei; Dayal, Viswas

2012-10-01

307

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

308

Oculocerebrocutaneous syndrome: the brain malformation defines a core phenotype  

PubMed Central

Background: Oculocerebrocutaneous syndrome (OCCS) is characterised by orbital cysts and anophthalmia or microphthalmia, focal aplastic or hypoplastic skin defects, skin appendages, and brain malformations. The eye and skin abnormalities are well described but the neuropathological features less so. To date, 28 patients with an unequivocal diagnosis of OCCS have been reported, with a preponderance of males. Objective: To evaluate the brain imaging studies, clinical records, photographs, and pathological material of two new and nine previously reported cases of OCCS. Results: There was a consistent pattern of malformations in eight of the 11 cases, consisting of frontal predominant polymicrogyria and periventricular nodular heterotopia, enlarged lateral ventricles or hydrocephalus, agenesis of the corpus callosum sometimes associated with interhemispheric cysts, and a novel mid-hindbrain malformation. The latter consisted of a giant and dysplastic tectum, absent cerebellar vermis, small cerebellar hemispheres in most cases, and a large posterior fossa fluid collection. Conclusions: The mid-hindbrain malformation appears pathognomonic for OCCS. The eye and skin features of OCCS show considerable overlap with several other syndromes, such as encephalocraniocutaneous lipomatosis, oculo-auriculo-vertebral spectrum, and focal dermal hypoplasia, none of which has a comparable pattern of brain malformations. In particular the unique mid-hindbrain malformation also distinguishes OCCS from related syndromes with comparable forebrain anomalies. The pattern of malformation described thus helps in differentiating OCCS from other entities. The mid-hindbrain malformation points to a defect of the mid-hindbrain organiser as the underlying pathogenic mechanism. PMID:15879499

Moog, U; Jones, M; Bird, L; Dobyns, W

2005-01-01

309

Field Guide to Malformations of Frogs and Toads  

E-print Network

Field Guide to Malformations of Frogs and Toads With Radiographic Interpretations U.S. Department recently metamorphosed frogs that were collected in Minnesota, Vermont, Wisconsin and Maine and examined on the northern leopard frog (Rana pipiens). The malformations and the method of their classification are reported

Torgersen, Christian

310

Abernethy malformation with portal vein aneurysm in a child  

PubMed Central

Abernethy malformation is an extremely rare anomaly of the splanchnic venous system. We describe multidetector computed tomography findings of an incidentally detected Abernethy malformation with portal vein aneurysm in a two-and-half-year old child. The computed tomography scan was performed for the evaluation of respiratory distress, poor growth, and loss of appetite. PMID:21430844

Chandrashekhara, Sheragaru H.; Bhalla, Ashu Seith; Gupta, Arun Kumar; Vikash, C. S.; Kabra, Susheel Kumar

2011-01-01

311

Counseling pregnant women treated with paroxetine Concern about cardiac malformations  

Microsoft Academic Search

QUESTION I have always reassured my patients that taking selective serotonin reuptake inhibitors (SSRIs) during pregnancy would not increase their risk of having children with major malformations. A recent warning from Health Canada, based on results of a study from GlaxoSmithKline, stated that infants exposed to paroxetine might be at higher risk of congenital malformations, specifi cally cardiovascular defects. Some

Adrienne Einarson; Gideon Koren

312

Nursery care of the newborn with malformation syndrome  

Microsoft Academic Search

Congenital malformations are not so rare, and represent a relevant challenge to health care providers. We delineate a methodology for the clinical approach to the malformed newborn, that takes into account the needs of the patient as single person and as part of a familiar and social context. We consider first the medical problem that the neonatologists face in the

Francesca Romana Pluchinotta; Luigi Memo

2009-01-01

313

Cerebral cavernous malformations (cavernomas) in the pediatric age-group  

Microsoft Academic Search

Cavernomas are vascular malformations composed of a compact mass of sinusoidal-type vessels that are immediately contiguous with one another and have no intervening parenchyma. Cavernous malformations were previously held to be a rare pathology occurring predominantly in adults. New neuroradiological techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) demonstrate, on the contrary, that these lesions are also

C. Mazza; R. Scienza; A. Beltramello; R. Da Pian

1991-01-01

314

Intralesional laser therapy for vascular malformations.  

PubMed

Intralesional laser therapy for the treatment of vascular malformations (VMs) has been previously reported for select patient populations. Larger studies, over a wider variety of indications, are needed to better define the potential role of this technology. In the current study, a 12-year, retrospective review of 44 patients who underwent 73 intralesional Nd:YAG or diode laser treatments of VMs was performed. The most commonly encountered lesions were venous malformations (66%) and the most commonly involved anatomic locations were the head and neck regions (41%) and lower extremity (39%). Primary indications for treatment were enlargement (73%) and pain (52%). Lesion size was reduced in 94% of cases after treatment and pain was improved in 91% of cases. Minor postoperative complications occurred in 16 (36%) patients. There was no difference in treatment response among various VM subtypes or anatomic locations (P = 0.497, P = 0.866) or in the incidence of complications (P = 0.531, P = 0.348). Age was the only factor associated with an increased risk of complications (odds ratio, 1.034; P = 0.038). When used in accordance with the suggested guidelines, intralesional laser therapy is a safe and effective treatment modality for VMs of varying compositions and locations. PMID:24625513

Ma, Linda W; Levi, Benjamin; Oppenheimer, Adam J; Kasten, Steven J

2014-11-01

315

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. PMID:1494822

Miller, M T; Stromland, K

1992-01-01

316

Approche psychologique de la malformation congénitale de la main.La malformation congénitale, le désir de savoir  

Microsoft Academic Search

The psychological approach to congenital malformation is determined by the stares of the others and the individual?s needs. The relationship of the body of the person afflicted with a congenital malformation is a question of aesthetic suffering and not of functional use. Caught up in a network of a priori and anxiety- based projections, it is difficult for patients, children,

A. Didierjean-Pillet

2002-01-01

317

Associated malformations in patients with limb reduction deficiencies.  

PubMed

Infants with limb reduction deficiencies (LRD) often have other associated congenital malformations. The purpose of this investigation was to assess the prevalence and the types of associated malformations in a defined population. This study included special strengths: each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for malformations was continued until 1 year of age. The associated malformations in infants with LRD were collected in all livebirths, stillbirths and terminations of pregnancy during 25 years in 347,810 consecutive births in the area covered by our population based registry of congenital malformations. Of the 271 LRD infants born during this period, representing a prevalence of 7.8 per 10,000, 57.9% had associated malformations. There were 17(6.3%) patients with chromosomal abnormalities including 10 trisomies 18, and 62 (22.9%) nonchromosomal recognized dysmorphic conditions. There were no predominant recognized dysmorphic conditions, but VA(C)TER(L) association. However numerous recognized dysmorphic conditions were registered including Poland, ectrodactyly-ectodermal dysplasia-clefting, oral-facial-digital, Klippel-Trenaunay-Weber, oculo-auriculo-vertebral defect spectrum, CHARGE, Townes-Brocks, Moebius, Du Pan, Smith-Lemli-Opitz, hypoglossia-hypodactyly, amniotic band, De Lange, Rubinstein-Taybi, Fanconi, radius aplasia- thrombocytopenia, Roberts, Holt-Oram, and fetal diethylstilbestrol. Seventy eight (28.8%) of the patients were multiply, non-syndromic, non chromosomal malformed infants (MCA). Malformations in the cardiac system, in the genital system, and in the central nervous system were the most common other malformations, 11.4%, 9.4%, and 7.7% of the associated malformations, respectively, followed by malformations in the renal system (4.8%), and in the digestive system (4.6%). Prenatal diagnosis was performed in 48.4% of dysmorphic syndromes with LRD. The overall prevalence of associated malformations, which was more than one in two infants, emphasizes the need for a thorough investigation of infants with LRD.A routine screening for other malformations especially cardiovascular system, urogenital system, central nervous system, and digestive system may be considered in infants and in fetuses with LRD. PMID:20670696

Stoll, Claude; Alembik, Yves; Dott, Beatrice; Roth, Marie-Paule

2010-01-01

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. PMID:23372981

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

2012-01-01

319

Multimodal device for assessment of skin malformations  

NASA Astrophysics Data System (ADS)

A variety of multi-spectral imaging devices is commercially available and used for skin diagnostics and monitoring; however, an alternative cost-efficient device can provide an advanced spectral analysis of skin. A compact multimodal device for diagnosis of pigmented skin lesions was developed and tested. A polarized LED light source illuminates the skin surface at four different wavelengths - blue (450 nm), green (545 nm), red (660 nm) and infrared (940 nm). Spectra of reflected light from the 25 mm wide skin spot are imaged by a CMOS sensor. Four spectral images are obtained for mapping of the main skin chromophores. The specific chromophore distribution differences between different skin malformations were analyzed and information of subcutaneous structures was consecutively extracted.

Bekina, A.; Garancis, V.; Rubins, U.; Spigulis, J.; Valeine, L.; Berzina, A.

2013-11-01

320

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. PMID:393376

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

1979-01-01

321

[The hand: embryology and main malformative mechanisms].  

PubMed

Upper limb bud appears in the cervical region of the embryo during the fifth week of development. It is made of epithelia and underlying mesenchyme. Diffusible growth factors, expressed by the apical ectodermal ridge, direct the proximal-distal growth. Other factors are expressed by zone of polarizing activity and ectoderm. They induce together anterior-posterior growth and dorsal-ventral polarity of the limb bud. The development of axial skeleton pattern is controlled by transcription factors from the HOX family, which are expressed in a stripe along the proximal and distal edges of the limb bud. Embryologic mechanisms of the main hand malformations are described, as well as their known genetic or mechanical aetiologies. PMID:18838286

Perrin, J; Geoffroy-Siraudin, C; Metzler-Guillemain, C

2008-12-01

322

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

323

Late onset ulcerative colitis complicating a patient with Budd-Chiari syndrome: a case report and review of the literature.  

PubMed

We report a case of a 33-year-old female patient with Budd-Chiari syndrome because of polycythemia vera. A transjugular intrahepatic portal-systemic shunt was performed because of refractory ascites 7 months after diagnosis. She had a stable hepatic function receiving anticoagulants until 3 years later when she presented with bloody diarrheas, liver function deterioration with prolonged prothrombin time and hypoalbuminemia, encephalopathy, and ascites. Colonoscopy revealed ulcerative pancolitis and the patient was treated with corticosteroids and antibiotics. Hepatic function was stabilized in parallel to controlling ulcerative colitis and the patient is in good health until now receiving maintenance therapy for ulcerative colitis and anticoagulants for Budd-Chiari syndrome. PMID:19060635

Vassiliadis, Themistoklis; Mpoumponaris, Alexander; Giouleme, Olga; Hatzidakis, Adam; Patsiaoura, Kalliopi; Zezos, Petros; Vakalopoulou, Sofia; Kargiotis, Konstantinos; Gkisakis, Dimitrios; Katsinelos, Panagiotis; Evgenidis, Nikolaos

2009-01-01

324

Chiari type I anomalies in children and adolescents: minimally invasive management in a series of 53 cases  

Microsoft Academic Search

The authors studied the role of the sole posterior fossa bony decompression in the management of symptomatic children affected\\u000a by Chiari type I anomalies. The series in the pediatric literature on this subject were reviewed and compared with that presented\\u000a in this article. From May 1994 to December 1998, 53 patients (3 months to 26 years) were observed. They were

L. Genitori; Paola Peretta; Chiara Nurisso; Luigi Macinante; Federico Mussa

2000-01-01

325

Laparoscopic excision of a retroperitoneal lymphatic malformation in a newborn.  

PubMed

Abdominal lymphatic malformations may be challenging to eradicate. Retroperitoneal lesions may more difficult to resect than mesenteric ones; however, the latter may predispose to intestinal volvulus, leading to calls for their prompt excision. Such lesions identified perinatally may pose particular challenges: in one case, respiratory failure caused by abdominal distension required emergency drainage followed by later laparoscopic excision; laparoscopy has also been used promptly to diagnose and resect neonatal mesenteric lymphatic malformations with their inherent volvulus risk. We illustrate that even if neonatal laparoscopy identifies a retroperitoneal rather than mesenteric lymphatic malformation, curative endosurgical excision remains feasible. PMID:21292064

Solari, Valeria; Mullassery, Dhanya; Lansdale, Nick; Jesudason, Edwin C

2011-02-01

326

Complex malformations of the female genital tract. New types and revision of classification  

Microsoft Academic Search

BACKGROUND: Complex malformations of the female genital tract are often incorrectly identified, treated and reported, probably due to not considering the malformation as a cause of the clinical symptoms and neither the embryological origin of the different elements of the genitourinary tract. METHODS: Complex malformations are studied and classified, and new types are presented. The new types of complex malformations

Pedro Acien; Maribel Acien; Marisa Sanchez-Ferrer

327

Juan Arellano 956-251-0636 arellano@math.montana.edu X X X X X X X X Liz Arnold 707-499-7341 arnold@math.montana.edu X X X  

E-print Network

Juan Arellano 956-251-0636 arellano@math.montana.edu X X X X X X X X Liz Arnold 707-499-7341 arnold@math.montana.edu X X X Katie Banner 541-231- banner@math.montana.edu X X X X X X X X X X X Michael Broome 406-480-6579 broomemike@gmail.com X X X X X X X X X X X X X X Jacob Brown 406-461-5978 jbrown@math.montana.edu X X X X X X

Dyer, Bill

328

On the Lusternik–Schnirelmann category of symplectic manifolds and the Arnold conjecture  

Microsoft Academic Search

We prove that the Lusternik-Schnirelmann category $cat(M)$ of a closed\\u000asymplectic manifold $(M, \\\\omega)$ equals the dimension $dim(M)$ provided that\\u000athe symplectic cohomology class vanishes on the image of the Hurewicz\\u000ahomomorphism. This holds, in particular, when $\\\\pi_2(M)=0$. The Arnold\\u000aconjecture asserts that the number of fixed points of a Hamiltonian\\u000asymplectomorphism of $M$ is greater than or equal to

Yuli B. Rudyak; John Oprea

1999-01-01

329

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

330

Malformation syndromes associated with disorders of sex development.  

PubMed

When embryological development of the internal and/or external genitalia is disrupted, the patient presents with a disorder of sex development (DSD) in the neonatal period or sometime later in life. Some of these patients have other, nongenital malformations, which makes their overall management more complex than if they just had a DSD. This Review summarises these malformation syndromes and discusses the recent research into their aetiology. The genetic causes of these malformation syndromes, when they are known, will also be described. Many specific genetic mutations are now known in malformation syndromes with a defect in hormonal function. By contrast, the genetic causes remain unknown in many nonhormonal morphological anomalies that affect the genitalia. PMID:24913517

Hutson, John M; Grover, Sonia R; O'Connell, Michele; Pennell, Samuel D

2014-08-01

331

Genetics Home Reference: Megalencephaly-capillary malformation syndrome  

MedlinePLUS

... Registry: Megalencephaly cutis marmorata telangiectatica congenita M-CM Network: How is M-CM Diagnosed? You might also find information on the diagnosis or management of megalencephaly-capillary malformation syndrome in Educational resources ...

332

[Cystic adenomatoid malformation of the lung. Importance of prenatal diagnosis].  

PubMed

Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications. PMID:21465061

Cabeza, Beatriz; Oñoro, Gonzalo; Cantarín Extremera, Verónica; Sanz Santiago, Verónica; Sequeiros, Adolfo

2011-04-01

333

Cloacal malformations: lessons learned from 490 cases.  

PubMed

In this review we describe lessons learned from the authors' series of patients born with the most complex of congenital anorectal problems, cloacal malformations, with the hope to convey the improved understanding and surgical treatment of the condition's wide spectrum of complexity learned from patients cared for over the last 25 years. This includes a series of 490 patients, 397 of whom underwent primary operations, and 93 who underwent reoperations after attempted repairs at other institutions. With regard to the newborn, we have learned that the clinician must make an accurate neonatal diagnosis, drain a hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through, and a vaginal replacement if needed. A correct diagnosis will avoid repairing only the rectal component. For the definitive reconstruction, all patients in the series were managed with a posterior sagittal approach; 184 of whom also required a laparotomy. The average length of the common channel was 4.6 cm for patients who required a laparotomy and 2.5 cm for those who did not. Hydrocolpos was present in 139 patients (30%). Vaginal reconstruction involved a vaginal pull-through in 308 patients, a vaginal flap in 44, vaginal switch in 48, and vaginal replacement in 90 (33 with rectum, 15 with colon, and 42 with small bowel). A total of 220 underwent total urogenital mobilization, which was first introduced in 1996. Complications included rectal prolapse in 26, vaginal stricture or atresia in 18, urethrovaginal fistula in 13, and urethral atresia in 6. A total of 53% of all cases have voluntary bowel movements. The others are kept clean with a mechanical daily emptying (an enema) as part of a bowel management program. Indications for reoperations included persistent urogenital sinus after initial repair in 39 patients. Fifty-one had problems such as rectal prolapse, stricture, retraction, dehiscence or atresia, 29 had a mislocated rectum, 34 had vaginal stricture, retraction, dehiscence, atresia, or stenosis, 16 had urethrovaginal or rectovaginal fistulae, and 5 had urethral stricture or atresia. The series was divided into 2 distinct groups of patients where common channel measurement was known (n = 400): group A were those with a common channel < or = 3.0 cm (n = 225, 56%) and group B had a common channel >3 cm (n = 175, 44%). The separation into these 2 groups has important therapeutic and prognostic implications. Patients in Group A can be repaired posterior sagittally with a reproducible operation. Because they represent most patients, most well-trained pediatric surgeons should be able to repair these types of malformations, and the prognosis is good. Patients in Group B (those with a common channel >3 cm), usually require a laparotomy, have a much higher incidence of associated urological problems, and often require special maneuvers for vaginal reconstruction. Surgeons who repair Group B malformations require special training in urology; the operations are prolonged and technically demanding, and the functional results are not as good as in group A. PMID:20307849

Levitt, Marc A; Peña, Alberto

2010-05-01

334

Coil Embolization of a Neonatal Pulmonary Arteriovenous Malformation  

Microsoft Academic Search

Pulmonary arteriovenous malformation (PAVM), as a part of Osler-Weber-Rendu Syndrome, in the neonate, is a rare hereditary vascular malformation. Large intrapulmonary right-to-left shunting, causing hypoxaemia and cyanosis, can be a life-threatening condition. Repeated transcatheter coil embolization procedures proved to be a favorable strategy to improve systemic arterial oxygen saturation, with a good outcome in a newborn child. While the radiation

R. G. Bennhagen; G. Holje; S. Laurin; E. Pesonen

2002-01-01

335

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

336

Lymphatic malformations: A dilemma in diagnosis and management  

PubMed Central

Lymphatic malformations of the head and neck, also known as lymphangiomas or cystic hygromas, are a diverse group of lesions. Lymphangiomas represent benign hamartomatous tumors of lymphatic vessels with a marked predilection for the head, neck and oral cavity. These lesions, like most of the swellings occurring in the neck, frequently pose a dilemma in diagnosis and treatment. This is a case report of a lymphatic malformation which presented as a painless mass in the lower border of mandible. PMID:24808711

Ramashankar; Prabhakar, Chandan; Shah, Nishit Kumar; Giraddi, Girish

2014-01-01

337

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. PMID:4262651

Lowe, C. R.

1972-01-01

338

[Lung perfusion scintigraphy in pediatric patients with congenital malformations].  

PubMed

Congenital malformations are an heterogeneous group of disorders that can lead changes in pulmonary perfusion and then can be evaluated with lung perfusion scintigraphy. We selected five patients in which the role of lung perfusion scintigraphy emerges as a reliable and non-invasive imaging technique. Lung perfusion scintigraphy is a useful tool in pediatric patients with congenital malformations allowing an accurate evaluation of the best therapeutic strategy and its results. PMID:24042425

Niccoli Asabella, Artor; Stabile Ianora, Amato Antonio; Di Palo, Alessandra; Rubini, Domenico; Pisani, Antonio Rosario; Ferrari, Cristina; Notaristefano, Antonio; Rubini, Giuseppe

2013-01-01

339

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

340

Three-dimensional spiral CT of craniofacial malformations in children  

Microsoft Academic Search

Objective. To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial\\u000a malformations.¶Materials and methods. Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations,\\u000a underwent craniofacial spiral CT. 3D reformatting was performed using an independent workstation.¶Results. 3D CT allowed the preoperative evaluation of

S. Binaghi; F. Gudinchet; B. Rilliet

2000-01-01

341

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. PMID:23610506

Fadel, Hossam E.

2012-01-01

342

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

2013-01-01

343

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. PMID:11102301

Sower, S A; Reed, K L; Babbitt, K J

2000-01-01

344

A review of cavernous malformations with trigeminal neuralgia.  

PubMed

Cavernous malformation with trigeminal neuralgia is relatively rare; only 10 cases have been reported. In deciding treatment strategies, it is helpful to classify cavernous malformation according to its origin, as follows: in the Gasserian ganglion (Type G); between the cisternal and intra-axial portions of the trigeminal nerve root (Type C); in the intra-axial trigeminal nerve root in the pons (Type P); or in the spinal tract of the trigeminal nerve root (Type S). A 62-year-old male presented with left trigeminal neuralgia (V2 area) and left facial hypoesthesia. Imaging studies revealed a cerebellopontine angle mass lesion with characteristics of a cavernous malformation and evidence of hemorrhage. The lesion was completely removed via a left anterior transpetrosal approach. The mass was attached to the trigeminal nerve root; it was located between the cisternal and intra-axial portions of the nerve root, and feeding off microvessels from the trigeminal nerve vascular plexus. Histological examination confirmed a cavernous malformation. In this case, the cavernous malformation was Type C. We review cases of cavernous malformation with trigeminal neuralgia and discuss therapeutic strategies according to the area of origin. PMID:25129386

Adachi, Kazuhide; Hasegawa, Mitsuhiro; Hayashi, Takuro; Nagahisa, Shinya; Hirose, Yuichi

2014-10-01

345

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. PMID:22826813

Wang, Huan-Chih; Chang, Rachel J.; Xiao, Furen

2012-01-01

346

Stereotactic linac radiosurgery for arteriovenous malformations.  

PubMed Central

Stereotactic linear accelerator (linac) radiosurgery has been in operation in the West Midlands since 1987, the first of its kind in the United Kingdom. Forty two patients with high-flow cerebral arteriovenous malformations have been treated, 26 of whom have been followed up. Angiography one year after treatment showed that five lesions were obliterated, 11 were reduced in size and/or flow rate and 10 were unchanged. Overall results show that nine out of 10 patients reviewed at 24 months had total obliteration. Three patients had complications; one has fully recovered, one died of an unrelated cause at 36 months and the other died from recurrent haemorrhage at nine months. Two patients had recurrent non-fatal haemorrhage within 24 months of treatment; both recovered without further deficit. All patients are fit to work but eight are unemployed. Although the follow up period is short, the early results indicate a success rate similar to those published by others using linac radiosurgery. Images PMID:1640237

Kenny, B G; Hitchcock, E R; Kitchen, G; Dalton, A E; Yates, D A; Chavda, S V

1992-01-01

347

The management of arteriovenous malformations in children.  

PubMed

Between January 1941 and June 1989, 46 children below the age of 18 with an arteriovenous malformation (AVM) were managed. There were 7 patients with AVM diagnosed before the age of 2; 10 patients were diagnosed between the ages of 3 and 10; and 29 patients were diagnosed between 11 and 18. There were equal numbers of male and female patients. Twenty-five of the AVMs were large (greater than 5 cm longest diameter). All 7 AVMs diagnosed before the age of 2 were large. The usual clinical presentation was congestive heart failure, bruit and an enlarging head. Three patients underwent excision with 2 deaths and 1 excellent result. In 11 patients (aged 3-18) with AVM without history of hemorrhage, 3 had excision with 2 excellent and 1 fair result. Four remained stable. Four developed progressive deficits or hemorrhage. In 10 patients (aged 3-18) with AVM and hemorrhage who were treated medically, 7 (70%) had an episode of re-hemorrhage. Three patients had excision of AVM after re-hemorrhage, but before the age of 18 with an excellent result. Eighteen patients (aged 3-18) with AVM and a single episode of hemorrhage underwent excision with 17 excellent or good results and 1 fair result. The overall mortality was 7%. Eighty-five percent of the children with excision of AVM had an excellent or good result. The best treatment for AVM in children is surgical excision. PMID:2054808

Malik, G M; Sadasivan, B; Knighton, R S; Ausman, J I

1991-02-01

348

Primary tethered cord syndrome: a new hypothesis of its origin.  

PubMed

Primary tethered cord syndrome is defined as low placement of the spinal cord and thickened filum terminale with associated anomalies. This definition excludes anomalies concomitant with overt myelomeningocele and spinal cord tethering secondary to myelomeningocele repair. Embryologically, the primary tethered cord syndrome is an entirely different entity from overt myelomeningocele and associated Arnold-Chiari type II malformation, but its origins have not been satisfactorily explained. The authors postulate that primary tethered cord syndrome is a manifestation of local dysmorphogenesis of all three germ layers at the lumbosacral area, possibly triggered by a hemorrhagic, inflammatory, or some other local lesion occurring in embryogenesis. PMID:6426276

Sarwar, M; Virapongse, C; Bhimani, S

1984-01-01

349

A Chaotic Cryptosystem for Images Based on Henon and Arnold Cat Map  

PubMed Central

The rapid evolution of imaging and communication technologies has transformed images into a widespread data type. Different types of data, such as personal medical information, official correspondence, or governmental and military documents, are saved and transmitted in the form of images over public networks. Hence, a fast and secure cryptosystem is needed for high-resolution images. In this paper, a novel encryption scheme is presented for securing images based on Arnold cat and Henon chaotic maps. The scheme uses Arnold cat map for bit- and pixel-level permutations on plain and secret images, while Henon map creates secret images and specific parameters for the permutations. Both the encryption and decryption processes are explained, formulated, and graphically presented. The results of security analysis of five different images demonstrate the strength of the proposed cryptosystem against statistical, brute force and differential attacks. The evaluated running time for both encryption and decryption processes guarantee that the cryptosystem can work effectively in real-time applications. PMID:25258724

Sundararajan, Elankovan

2014-01-01

350

RASA1 mutations and associated phenotypes in 68 families with capillary malformation-arteriovenous malformation.  

PubMed

Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal-dominant disorder, caused by heterozygous RASA1 mutations, and manifesting multifocal CMs and high risk for fast-flow lesions. A limited number of patients have been reported, raising the question of the phenotypic borders. We identified new patients with a clinical diagnosis of CM-AVM, and patients with overlapping phenotypes. RASA1 was screened in 261 index patients with: CM-AVM (n = 100), common CM(s) (port-wine stain; n = 100), Sturge-Weber syndrome (n = 37), or isolated AVM(s) (n = 24). Fifty-eight distinct RASA1 mutations (43 novel) were identified in 68 index patients with CM-AVM and none in patients with other phenotypes. A novel clinical feature was identified: cutaneous zones of numerous small white pale halos with a central red spot. An additional question addressed in this study was the "second-hit" hypothesis as a pathophysiological mechanism for CM-AVM. One tissue from a patient with a germline RASA1 mutation was available. The analysis of the tissue showed loss of the wild-type RASA1 allele. In conclusion, mutations in RASA1 underscore the specific CM-AVM phenotype and the clinical diagnosis is based on identifying the characteristic CMs. The high incidence of fast-flow lesions warrants careful clinical and radiologic examination, and regular follow-up. PMID:24038909

Revencu, Nicole; Boon, Laurence M; Mendola, Antonella; Cordisco, Maria Rosa; Dubois, Josée; Clapuyt, Philippe; Hammer, Frank; Amor, David J; Irvine, Alan D; Baselga, Eulalia; Dompmartin, Anne; Syed, Samira; Martin-Santiago, Ana; Ades, Lesley; Collins, Felicity; Smith, Janine; Sandaradura, Sarah; Barrio, Victoria R; Burrows, Patricia E; Blei, Francine; Cozzolino, Mariarosaria; Brunetti-Pierri, Nicola; Vicente, Asuncion; Abramowicz, Marc; Désir, Julie; Vilain, Catheline; Chung, Wendy K; Wilson, Ashley; Gardiner, Carol A; Dwight, Yim; Lord, David J E; Fishman, Leona; Cytrynbaum, Cheryl; Chamlin, Sarah; Ghali, Fred; Gilaberte, Yolanda; Joss, Shelagh; Boente, Maria Del C; Léauté-Labrèze, Christine; Delrue, Marie-Ange; Bayliss, Susan; Martorell, Loreto; González-Enseñat, Maria-Antonia; Mazereeuw-Hautier, Juliette; O'Donnell, Brid; Bessis, Didier; Pyeritz, Reed E; Salhi, Aicha; Tan, Oon T; Wargon, Orli; Mulliken, John B; Vikkula, Miikka

2013-12-01

351

Energy Distribution of Heating Processes in the Quiet Solar Sam Krucker 1;2 and Arnold O. Benz 1  

E-print Network

Energy Distribution of Heating Processes in the Quiet Solar Corona S¨am Krucker 1;2 and Arnold O that emerging magnetic flux may provide enough energy to heat the corona by the release of magnetic energy (e can be readily estimated from the thermal energy of the newly heated material injected into the corona

352

The Colonizer Demands its “Fair Share,” and More: Contemporary American Anti-Tribalism from Arnold Schwarzenegger to the Extreme Right  

Microsoft Academic Search

In no small part, Arnold Schwarzenegger's victory in the 2003 California recall can be credited to his campaign's ability to define him as an ‘outsider’ who would save the state from casino-owning indigenous tribes that were seemingly taking over the political system and refusing to pay their ‘fair share.’ This strategy invoked the American sentiment of colonialist anti-tribalism: the view

Kevin Bruyneel

2006-01-01

353

The Multiparticle Quantum Arnol'd Cat: a test case for the decoherence approach to quantum chaos  

E-print Network

A multi-particle extension of the Arnol'd Cat Hamiltonian system is defined and examined. We propose to compute its Alicki-Fannes quantum dynamical entropy, to validate (or disprove) the validity of the decoherence approach to quantum chaos. A first set of numerical experiments is presented and discussed.

Giorgio Mantica

2010-07-06

354

Epidemiologic features of male genital malformations and subtypes in Texas.  

PubMed

Although distinct categories of male genital malformations share some common risk factors, few studies have systematically compared epidemiologic features across phenotypes. We evaluated the relationship between several maternal and infant characteristics and five categories of male genital malformations: second- or third-degree hypospadias, hypospadias (regardless of degree), small penis, cryptorchidism, and any male genital malformation. Data for 16,813 cases with isolated male genital malformations and 1,945,841 male live births delivered from 1999 to 2008 were obtained from the Texas Birth Defects Registry. For each phenotype category, 13 maternal and infant variables were assessed, and adjusted prevalence ratios were estimated based on the same multivariable Poisson regression model. A significant negative association was observed between previous live births versus no previous live births and four phenotypes (e.g., adjusted prevalence ratio [aPR] for any male genital malformation: 0.78, 95% confidence interval [CI]: 0.75-0.81). The prevalence of 4 of the phenotypes was significantly higher among multiple versus singleton pregnancies (e.g., aPR for any male genital malformation: 1.35, 95% CI: 1.25-1.47). We also observed significant associations between multiple phenotypes and residential region at delivery, delivery year, month of conception, and maternal age, race/ethnicity, education, and birthplace, including significant associations for trends (maternal age, maternal education, and birth year modeled ordinally). Our results allow for comparison of characteristics across phenotypes and suggest that there may be some common risk factors for multiple male genital malformations (e.g., characteristics related to maternal estrogen levels), while other risk factors may be unique to specific defects. PMID:24458943

Agopian, A J; Langlois, Peter H; Ramakrishnan, Anushuya; Canfield, Mark A

2014-04-01

355

Stereotactic radiosurgery after embolization for arteriovenous malformations.  

PubMed

We sought to define the long-term benefits and risks of stereotactic radiosurgery (SRS) for arteriovenous malformation (AVM) patients who underwent prior embolization. Between 1987 and 2006, we performed Gamma Knife® SRS on 120 patients with AVM who underwent embolization followed by SRS. Sixty-four patients (53%) had at least 1 prior hemorrhage. The median number of embolizations varied from 1 to 5. The median target volume was 6.6 cm3 (range, 0.2-26.3 cm3). The median margin dose was 18 Gy (range, 13.5-25 Gy). After embolization, 25 patients (21%) developed symptomatic neurological deficits. The overall rates of total obliteration documented by either angiography or magnetic resonance imaging were 35, 53, 55 and 59% at 3, 4, 5 and 10 years, respectively. Nine patients (8%) had a hemorrhage during the latency interval and 7 patients died due to hemorrhage. The actuarial rates of AVM hemorrhage after SRS were 0.8, 3.5, 5.4, 7.7 and 7.7% at 1, 2, 3, 5 and 10 years, respectively. Permanent neurological deficits due to adverse radiation effects developed in 3 patients (2.5%) after SRS. Using a case-match control technique, we found that embolization prior to SRS was associated with a lower rate of total obliteration (p=0.028) in comparison to radiosurgery alone. In this 20-year experience, we found that prior embolization reduced the rate of total obliteration after SRS and latency interval hemorrhage risks were not affected by prior embolization. In the future, the role of embolization after SRS should be explored. PMID:23258513

Kano, Hideyuki; Kondziolka, Douglas; Flickinger, John C; Park, Kyung-Jae; Iyer, Aditya; Yang, Huai-che; Liu, Xiaomin; Monaco, Edward A; Niranjan, Ajay; Lunsford, L Dade

2013-01-01

356

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. PMID:15747750

Miller, Marilyn T; Stromland, Kerstin; Ventura, Liana; Johansson, Maria; Bandim, Jose M; Gillberg, Christopher

2004-01-01

357

Investigation on etiology of hepatic venous obstruction budd-Chiari syndrome.  

PubMed

Budd-Chiari syndrome (BCS) is an uncommon clinical condition with a complex etiology. Pathogenesis of BCS is still poorly understood. We included hepatic veno-occlusive lesion tissues of 20 patients (patients group) with hepatic venous obstruction BCS and compared with 20 similar tissues with other etiologies (control group). Morphological changes in hepatic veno-occlusive lesion tissues and the positive expression of proliferating cell nuclear antigen (PCNA), C-myc, and P-53 were observed by the pathological examination (H&E staining) and immunohistochemistry assay. Our results showed that PCNA and C-myc positive cell densities were significantly higher in patient group than control group. P-53 positive cell density showed increasing trends in patients than control group. Moreover, we observed irregular hyperplasia in intimal tissue, fibrous connective tissue, and smooth muscle cell, accompanied by tissue degeneration (hyaloid degeneration and fibrinoid degeneration) and a large quantity of inflammatory cell infiltration. In conclusion, an overexpression of PCNA, C-myc, and a weak positive expression of P53 might launch the extremely irregular hepatic venous intimal hyperplasia, which is probably one of the etiologies of hepatic venous obstruction BCS. PMID:25096503

Tian, Zhi-Long; Jia, Gao-Lei; Xi, Hai-Lin; Feng, Su; Wang, Xiao-Kai; Li, Rui

2014-12-01

358

Defining anural malformations in the context of a developmental problem  

USGS Publications Warehouse

This paper summarizes terminology and general concepts involved in animal development for the purpose of providing background for the study and understanding of frog malformations. The results of our radiographic investigation of rear limb malformations in Rana pipiens provide evidence that frog malformations are the product of early developmental errors. Although bacteria, parasites and viruses were identified in these metamorphosed frogs, the relevant window to look for the teratogenic effect of these agents is in the early tadpole stage during limb development. As a result, our microbiological findings must be regarded as inconclusive relative to determining their contribution to malformations because we conducted our examinations on metamorphosed frogs not tadpoles. Future studies need to look at teratogenic agents (chemical, microbial, physical or mechanical) that are present in the embryo, tadpole, and their environments at the stages of development that are relevant for the malformation type. The impact of these teratogenic agents then needs to be assessed in appropriate animal models using studies that are designed to mimic field conditions. The results of these laboratory tests should then be analyzed in such a way that will allow comparison with the findings in the wild-caught tadpoles and frogs.

Meteyer, C.U.; Cole, R.A.; Converse, K.A.; Docherty, D.E.; Wolcott, M.; Helgen, J.C.; Levey, R.; Eaton-Poole, L.; Burkhart, J.G.

2000-01-01

359

Transgenerational transmission of radiation damage: genomic instability and congenital malformation.  

PubMed

The congenital malformation gastroschisis has a genetic disposition in the inbred mouse strain HLG/Zte. It is increased after preconceptional irradiation of males or females. Radiation exposures during the meiotic stages are most efficient. This malformation can also be induced by ionising radiation when the exposure takes place during the preimplantation period especially during the zygote stage. This latter effect can be transmitted to the next mouse generation. Other macroscopically visible or skeletal malformations are not significantly induced under these experimental conditions. These latter malformations are increased by radiation exposures during major organogenesis. The mechanisms for the development of the effects are different. Radiation exposure of the mouse zygote (1 to 3 hours p.c.) also leads to the induction of genomic instability in skin fibroblasts of the fetus. This phenomenon also occurs in a mouse strain (C57BL/6J) which is not susceptible to radiation-induced gastroschisis during the preimplantation period. The genomic instability is transmitted to the next mouse generation. During genomic instability chromatide breaks are dominating as in non-exposed cells. With respect to "spontaneous" malformations gastroschisis is dominating in HLG/Zte mice. Late radiation effects seem to have similar patterns as observed in non-exposed subjects, however, the rates are increased after irradiation. PMID:17019048

Streffer, Christian

2006-01-01

360

Population dependent differences of distribution of congenital heart malformations.  

PubMed

The aim of the work was to study the pattern of congenital circulatory system malformations in the newborns in Upper Silesia, Poland (N = 355), and to compare it with the data known from the literature, predominantly with genetically very distant Chinese population from Hong Kong (N = 744). There were significantly more boys (about 60%) than girls (about 40%) in both groups. Much higher rate of the heart defects with pulmonary outflow obstruction was found in Hong Kong neonates (37.8%) than in the Polish ones (16.1%). Left ventricular outflow obstruction malformations were more common in Chinese group (22.7%) than in Polish one (14.1%), as well. On the other hand, Polish neonates were significantly more often admitted to cardiological hospital because of left-to-right shunts (37.7%) as compared to Chinese population (15.5%). The rate of complete transposition of great arteries was similar in both studied groups (12.1% in Poland versus 12.4% in Hong Kong). So called common mixing malformations were not significantly more often found in Polish newborns (12.1%) than in Hong Kong ones (8.3%). It is concluded that it significant race dependent distribution difference of congenital heart malformations exists between Polish (Silesian) and Chinese (Hong Kong) newborn populations. On the other hand the pattern of congenital heart malformations found in Polish newborns seems to be not very different from these ones, known from literature, described in the USA or in Europe. PMID:12901266

Rokicki, W?adys?aw; Leung, Maurice; Kohut, Joanna; Durma?a, Jacek; Borowicka, Ewa

2003-01-01

361

Vein of Galen arteriovenous malformation mimicking coarctation of the aorta.  

PubMed

Arteriovenous malformation of the vein of Galen is a rare congenital intracranial anomaly lacking a capillary bed and subsequent aneurysmal enlargement of the arterial and venous system, warranting careful management due to associated morbidity and mortality. Coarctations of aorta demonstrate similar neonatal echocardiographic signs to the vein of Galen arterial malformation (VGAM). We present a boy at 37 weeks of gestation whose initial ultrasound and echocardiographic investigations showed a dominant right ventricle and isthmal hypoplasia, suggestive of coarctation of aorta. Follow-up ultrasound and echocardiography revealed an arteriovenous malformation involving middle and posterior cerebral artery branches, eliminating coarctation of aorta. VGAM was confirmed by further ultrasound and angiographic investigation, which demonstrated a tangle of cerebral and choroidal arterial branches centrally feeding into an enlarged vein of Galen. The boy's hemodynamic and neurological statuses were confirmed to be stable despite increased venous pressure. Elective embolization at 7 months of age was complicated by a cerebrovascular accident, resulting in right hemiparesis despite no residual cardiac issues. This case demonstrates that rarely, arteriovenous malformations such as the vein of Galen malformations may be the primary cause of patients presenting with coarctation of aorta. The rarity of this condition and its guarded prognosis make our case of special interest to cardiologists and the perinatal care team. PMID:25368688

Firdouse, Mohammed; Agarwal, Arnav; Mondal, Tapas

2014-12-01

362

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

363

Brain Vascular Malformation Consortium: Overview, Progress and Future Directions  

PubMed Central

Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in “research silos” with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation, and HHT Foundation International. Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations.

Akers, Amy L.; Ball, Karen L.; Clancy, Marianne; Comi, Anne M.; Faughnan, Marie E.; Gopal-Srivastava, Rashmi; Jacobs, Thomas P.; Kim, Helen; Krischer, Jeffrey; Marchuk, Douglas A.; McCulloch, Charles E.; Morrison, Leslie; Moses, Marsha; Moy, Claudia S.; Pawlikowska, Ludmilla; Young, William L.

2013-01-01

364

Evaluations of non-contrast enhanced MR venography with inflow inversion recovery sequence in diagnosing Budd-Chiari syndrome.  

PubMed

Non-contrast-enhanced (NCE) inflow inversion recovery magnetic resonance venography (MRV)and digital subtraction angiography (DSA) examination were carried out in 35 patients who were suspected of having Budd-Chiari syndrome (BCS). With DSA as the reference, NCE MRV got 100% in the diagnostic sensitivity, 67.8% in the specificity, 97.1% in the accuracy, 100% in the negative predictive value, and 96.9% in the positive predictive value. The consistency between NCE MRV and DSA in the depiction of IVC was excellent. NCE MRV is regarded as an alternative method in the diagnosis of BCS. PMID:25043534

Wu, Minghui; Xu, Junling; Shi, Dapeng; Shen, Hao; Wang, Meiyun; Li, Yongli; Han, Xinwei; Zhai, Shuiting

2014-01-01

365

Venous malformations: Sclerotherapy with a mixture of ethanol and lipiodol  

SciTech Connect

Purpose. To evaluate the usefulness of a mixture of absolute ethanol and lipiodol in the management of venous malformations. Methods. Percutaneous sclerotherapy was performed with a mixture of absolute ethanol and lipiodol (9:1) in 17 patients with venous malformations, once in 12 patients, twice in 5. The therapeutic efficacy was evaluated by pain reduction. Conventional radiographs (n=15) and posttreatment magnetic resonance imaging (n=5) were obtained for the follow-up evaluation. Results. Sclerotherapy was successful in all but two patients. The therapeutic effect was excellent in two patients, good in seven, fair in five, and poor in one. Radiopacity of lipiodol was beneficial for monitoring the procedure rather than for follow-up evaluations. Areas with low signal-intensity strands were increased on T2-weighted images obtained after the sclerotherapy. Conclusion. Sclerotherapy with a mixture of ethanol and lipiodol is effective in treating venous malformations.

Suh, Jin-Suck [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Shin, Kyoo-Ho [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of); Na, Jae-Bum [Kyungsang University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Won, Jong-Yun [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Hahn, Soo-Bong [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of)

1997-07-15

366

Radiation therapy for arteriovenous malformations: a review.  

PubMed

There have been numerous case reports and series of patients treated with partial brain irradiation, linear accelerator-based radiosurgery, gamma knife radiosurgery, and Bragg peak therapy for inoperable arteriovenous malformations (AVMs). These cases are summarized and compared. There is convincing evidence that radiation therapy does have a role in obliterating carefully chosen inoperable lesions. The changes that occur in vessel walls after radiation are reviewed. Data about x-ray and gamma radiation are mostly historical and difficult to evaluate because of the techniques of partial brain irradiation. There is a lack of data about the volume of AVM treated and the minimum dose delivered to the AVM nidus. For gamma knife, heavy particle, and linear accelerator therapy, more complete data are available. The incidence of hemorrhage during the first 2 years after treatment, when radiation-induced vascular changes are proposed to occur, is approximately 2.6% per year for gamma knife therapy, 2% per year for proton beam therapy, 2.3% per year for helium beam therapy, and 2.3% per year for linear accelerator therapy. These rates are similar to the recurrence rate for hemorrhage of 2.2 to 3% per year expected based on the natural history of untreated AVMs. If AVM obliteration after therapy is not achieved, the incidence of recurrent hemorrhage remains between 2% per year after treatment with gamma knife therapy. The incidence of hemorrhage for all patients treated was reported as 0.15% per year in one study and 20% over 8 years in a follow-up study using proton beam therapy. Mortality from hemorrhage after treatment was 0.6% after gamma knife therapy, 2.3% after helium beam therapy, and 2 to 5% after proton beam therapy. These figures for mortality are all lower than the 11% observed for the natural history of untreated AVMs. Permanent neurological deficits experienced as a complication of radiation occurred in 2 to 3% of patients treated with gamma knife therapy, 4% of patients treated with helium beam therapy, 1.7% of patients treated with proton beam therapy, and 3% of patients treated with stereotactic linear accelerator therapy. Proton beam therapy has been used for both small and large lesions. The majority of lesions in patients treated with gamma knife, helium beam, and linear accelerator therapy have been small (usually less than 3.0 cm average diameter) lesions. In these patients with small inoperable lesions treated with accurately directed fields of isocentric radiation, the greatest incidence of AVM obliteration has been observed on follow-up angiograms.(ABSTRACT TRUNCATED AT 400 WORDS) PMID:2191236

Ogilvy, C S

1990-05-01

367

Predictive simulations of ITER including neutral beam driven toroidal Federico D. Halpern, Arnold H. Kritz, Glenn Bateman, Alexei Y. Pankin, Robert V. Budny et al.  

E-print Network

Glenn Bateman,1 Alexei Y. Pankin,1 Robert V. Budny,2 and Douglas C. McCune2 1 Department of Physics, Arnold H. Kritz, Glenn Bateman, Alexei Y. Pankin, Robert V. Budny et al. Citation: Phys. Plasmas 15

Budny, Robert

368

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

369

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

370

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

NASA Astrophysics Data System (ADS)

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).

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

2012-09-01

371

ASSESSMENT OF ENVIRONMENTAL STRESSORS POTENTIALLY RESPONSIBLE FOR MALFORMATIONS IN NORTH AMERICAN ANURAN AMPHIBIANS  

EPA Science Inventory

A number of species of anuran amphibians from different regions across North America have recently exhibited an increased occurrence of, predominantly, hind limb malformations. Research concerning factors potentially responsible for these malformations has focused extensively on ...

372

Maternal diabetes and congenital malformations among live births in Hawaii  

Microsoft Academic Search

Summary  The frequency of congenital malformations, in 187,266 live births in Hawaii from 1956 through 1966, was found to be significantly\\u000a higher among offspring of diabetic mothers (0.0175) than in offspring of non-diabetic mothers (0.0086) or mothers with no\\u000a complications of pregnancy (0.0074). However, the frequency of malformations in offspring of diabetic mothers was not significantly\\u000a higher than in offspring of

Madeleine J. Goodman

1976-01-01

373

Multiple Complex Congenital Malformations in a Rabbit Kit (Oryctolagus cuniculi)  

PubMed Central

Congenital malformations may occur during early embryogenesis in cases of genetic abnormalities or various environmental factors. Affected subjects most often have only one or 2 abnormalities; subjects rarely have several unrelated congenital defects. Here we describe a case of a stillborn New Zealand white rabbit with multiple complex congenital malformations, including synophthalmia, holoprosencephaly, gastroschisis, and a supernumerary hindlimb, among other anomalies. There was no historical exposure to teratogens or other known environmental causes. Although not confirmed, this case was most likely a rare spontaneous genetic event. PMID:24209970

Booth, Jennifer L; Peng, Xuwen; Baccon, Jennifer; Cooper, Timothy K

2013-01-01

374

Prenatal diagnosis of congenital renal and urinary tract malformations  

PubMed Central

Congenital abnormalities of the kidneys and the urinary tract are the most common sonographically identified ­malformations in the prenatal period. Obstructive uropathies account for the majority of cases. The aim of prenatal diagnosis and management is to detect those anomalies having impact on the prognosis of the affected child and ­requiring early postnatal evaluation or treatment to minimize adverse outcomes. In this paper, we summarize the embryology of kidneys and urinary tract, the normal sonographic appearance through­out pregnancy and the prenatal diagnosis of their congenital malformations. PMID:24753862

Hindryckx, A.; De Catte, L.

2011-01-01

375

Imaging features of ductal plate malformations in adults.  

PubMed

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis. PMID:21840516

Venkatanarasimha, N; Thomas, R; Armstrong, E M; Shirley, J F; Fox, B M; Jackson, S A

2011-11-01

376

Pulmonary arteriovenous malformations presenting as refractory heart failure  

PubMed Central

A 22-year-old young man with a history of idiopathic dilated cardiomyopathy (IDC) was admitted to our hospital due to difficult-to-control heart failure. A thoracic X-ray showed multiple nodules at the both pulmonary hilus and upper lobe of the right lung. Computed tomography (CT) angiography of the thorax confirmed arteriovenous malformation (AVM). However, effective treatment was impossible due to the poor physical condition; he died a few days later. Here we reported on the case of pulmonary arteriovenous malformations (PAVMs) being misdiagnosed as refractory heart failure. PMID:25276390

Chen, Kai-Hong; Huang, Guo-Yong; Song, Wei

2014-01-01

377

Split cervical spinal cord malformation and vertebral dysgenesis.  

PubMed

We report a case of vertebral malformation associated with diplomyelia believed to be a type II split cord malformation. Cervicothoracic level cases are exceptional. This article reports the case of an 11-year-old boy with no neurological symptoms who had not undergone surgery. The diagnosis was made during pregnancy by prenatal screening with ultrasound and MRI. Several embryological theories have been offered to provide an explanation for this syndrome. Close follow-up is mandatory. Surgery must only be considered if neurological deterioration occurs. PMID:19837020

Andro, C; Pecquery, R; De Vries, P; Forlodou, P; Fenoll, B

2009-11-01

378

Diagnosis of the cavo-hepato-atrial pathway in Budd-Chiari syndrome by ultrasonography  

PubMed Central

The aim of this study was to investigate the ultrasonic features of the cavo-hepato-atrial pathway in Budd-Chiari syndrome (BCS), by which blood is drained from the occluded inferior vena cava (IVC) to the right atrium via hepatic veins. Ultrasonograms from 11 patients with BCS with cavo-hepato-atrial pathways were retrospectively studied. Doppler ultrasound was used to observe the direction of the flow and measure the velocity of the blood-draining vessels. Blood flow in the draining vessels and the collaterals was shown as blue, red or bicolored depending on whether the flow direction was away from the transducer, towards the transducer or both. For measurement, the Doppler angle between the axis of the Doppler beam and that of the vein examined was always <60°. Ultrasonography was performed 1–2 weeks prior to digital subtraction angiography (DSA). All patients were confirmed by DSA. Membranous and segmental occlusions of IVCs were observed in seven and four cases, respectively. Blood flow from the IVC reversed to the hepatic/accessory hepatic vein, continued through the dilated intrahepatic collaterals, onward to the other hepatic vein and finally to the right atrium. The majority of the inlets (8/11) of hepatic veins above the occlusion were narrow compared with the dilated distant parts of the lumens. Accelerated blood flow in the inlets was detected in all patients regardless of the luminal diameter. In conclusion, the results from the present study suggest that the unusual cavo-hepato-atrial pathway can be diagnosed reliably by ultrasonography, which may be useful for clinical management. PMID:25120601

GAI, YONG-HAO; CAI, SHI-FENG; FAN, HUI-LI; LIU, QING-WEI

2014-01-01

379

Spectral CT Imaging in Patients with Budd-Chiari Syndrome: Investigation of Image Quality.  

PubMed

To assess the image quality of monochromatic imaging from spectral CT in patients with Budd-Chiari syndrome (BCS), fifty patients with BCS underwent spectral CT to generate conventional 140 kVp polychromatic images (group A) and monochromatic images, with energy levels from 40 to 80, 40 + 70, and 50 + 70 keV fusion images (group B) during the portal venous phase (PVP) and the hepatic venous phase (HVP). Two-sample t tests compared vessel-to-liver contrast-to-noise ratio (CNR) and signal-to-noise ratio (SNR) for the portal vein (PV), hepatic vein (HV), inferior vena cava. Readers' subjective evaluations of the image quality were recorded. The highest SNR values in group B were distributed at 50 keV; the highest CNR values in group B were distributed at 40 keV. The higher CNR values and SNR values were obtained though PVP of PV (SNR 18.39 ± 6.13 vs. 10.56 ± 3.31, CNR 7.81 ± 3.40 vs. 3.58 ± 1.31) and HVP of HV (3.89 ± 2.08 vs. 1.27 ± 1.55) in the group B; the lower image noise for group B was at 70 keV and 50 + 70 keV (15.54 ± 8.39 vs. 18.40 ± 4.97, P = 0.0004 and 18.97 ± 7.61 vs. 18.40 ± 4.97, P = 0.0691); the results show that the 50 + 70 keV fusion image quality was better than that in group A. Monochromatic energy levels of 40-70, 40 + 70, and 50 + 70 keV fusion image can increase vascular contrast and that will be helpful for the diagnosis of BCS, we select the 50 + 70 keV fusion image to acquire the best BCS images. PMID:24833430

Su, Lei; Dong, Junqiang; Sun, Qiang; Liu, Jie; Lv, Peijie; Hu, Lili; Yan, Liangliang; Gao, Jianbo

2014-11-01

380

A Registry-Based Assessment of Cerebral Palsy and Cerebral Malformations  

Microsoft Academic Search

Cerebral malformations are 1 of the many possible causes of cerebral palsy. In this study, a population-based comprehensive cerebral palsy registry was used to identify children whose cerebral palsy could be attributed to a cerebral malformation. The clinical features of these children were then compared with other children with cerebral palsy. Children with cerebral palsy and cerebral malformation did not

Lauren Self; Michael I. Shevell

2010-01-01

381

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 that,…

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

382

Polymicrogyria: a common and heterogeneous malformation of cortical development.  

PubMed

Polymicrogyria (PMG) is one of the most common malformations of cortical development. It is characterized by overfolding of the cerebral cortex and abnormal cortical layering. It is a highly heterogeneous malformation with variable clinical and imaging features, pathological findings, and etiologies. It may occur as an isolated cortical malformation, or in association with other malformations within the brain or body as part of a multiple congenital anomaly syndrome. Polymicrogyria shows variable topographic patterns with the bilateral perisylvian pattern being most common. Schizencephaly is a subtype of PMG in which the overfolded cortex lines full-thickness clefts connecting the subarachnoid space with the cerebral ventricles. Both genetic and non-genetic causes of PMG have been identified. Non-genetic causes include congenital cytomegalovirus infection and in utero ischemia. Genetic causes include metabolic conditions such as peroxisomal disorders and the 22q11.2 and 1p36 continguous gene deletion syndromes. Mutations in over 30 genes have been found in association with PMG, especially mutations in the tubulin family of genes. Mutations in the (PI3K)-AKT pathway have been found in association PMG and megalencephaly. Despite recent genetic advances, the mechanisms by which polymicrogyric cortex forms and causes of the majority of cases remain unknown, making diagnostic and prenatal testing and genetic counseling challenging. This review summarizes the clinical, imaging, pathologic, and etiologic features of PMG, highlighting recent genetic advances. PMID:24888723

Stutterd, Chloe A; Leventer, Richard J

2014-06-01

383

Pulmonary arteriovenous malformation in the newborn: A familial case  

Microsoft Academic Search

Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in the newborn with nine previously reported cases. Typical signs at presentation include cyanosis, murmur, and congestive heart failure. Abnormality on chest x-ray is common with cardiomegaly, oligemia, and focal pulmonary density. There is a male predominance, unlike older children and adults with PAVM. Familial cases of PAVM have been

Steven W. Allen; Jonathan M. Whitfield; David R. Clarke; Eva Sujansky; James W. Wiggins

1993-01-01

384

Spontaneous Regression of Cerebral Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia  

Microsoft Academic Search

Summary: Cerebral arteriovenous malformations (AVMs) are associated with hereditary hemorrhagic telangiectasia (HHT). I report a case of a patient with HHT with four cerebral AVMs in whom one AVM spontaneously regressed during a 5-year period. The spontaneous regression of this AVM in this patient with HHT supports the theory that AVMs associated with this syndrome have a different nat- ural

Harry J. Cloft

385

Fusarium species associated with waterberry (Syzygium cordatum) floral malformation  

E-print Network

or in swampy spots. This evergreen tree has white, fluffy flowers that bloom from August to November. Its fruit is an edible berry that turns dark-purple when ripe. In rural areas the tree is used as a remedy large numbers of flowers. With time these malformed inflorescences dry out without ever bearing fruit

386

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

387

Failed transarterial embolization of subserosal uterine arteriovenous malformation.  

PubMed

Uterine arteriovenous malformation (AVM) is rare but potentially life-threatening from excessive vaginal bleeding. All uterine AVMs reported to date have been found in the endometrial or myometrial layers. Here we present a patient with a subserosal type AVM on the fundus of uterus, which spontaneously ruptured. PMID:24328024

Seo, Kyung Jin; Kim, Jin; Sohn, In Sook; Kwon, Han Sung; Park, Sang Woo; Hwang, Han Sung

2013-09-01

388

SHORT COMMUNICATION Fusarium mangiferae associated with mango malformation  

E-print Network

SHORT COMMUNICATION Fusarium mangiferae associated with mango malformation in the Sultanate of Oman in the beginning of 2005 in plantations at Sohar in the Sultanate of Oman. The affected inflorescences were factor 1 . -tubulin Mango (Mangifera indica) is an important perennial crop in the Sultanate of Oman

389

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

390

Valproic Acid and Congenital MalformationsA Case Report  

Microsoft Academic Search

An infant exposed in utero to valproic acid was born with multiple congenital malformations, including duodenal atresia, lumbar vertebral fusion, thoracolumbar scoliosis, renal abnormalities, diastasis recti abdominis, depigmentation of the eye brows, and a large hemangioma. The child had neonatal hyperbilirubinemia and postnatal growth deficiency. This case report is an addition to the literature im plicating valproic acid as a

Maj Eric W. Bantz

1984-01-01

391

Valproic acid and congenital malformations. A case report.  

PubMed

An infant exposed in utero to valproic acid was born with multiple congenital malformations, including duodenal atresia, lumbar vertebral fusion, thoracolumbar scoliosis, renal abnormalities, diastasis recti abdominis, depigmentation of the eyebrows, and a large hemangioma. The child had neonatal hyperbilirubinemia and postnatal growth deficiency. This case report is an addition to the literature implicating valproic acid as a human teratogen. PMID:6426840

Bantz, E W

1984-06-01

392

Proximity to Pollution Sources and Risk of Amphibian Limb Malformation  

PubMed Central

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.

2005-01-01

393

Coiling of a vulvar arterio-venous malformation  

PubMed Central

The authors report the case of a 13-year-old girl with a painful vulvar swelling and abnormal vaginal bleeding, increasing in size after trauma. With MRI (GE Signa HDx 1.5 Tesla), it is diagnosed as an arterio-venous malformation arising from the left superior femoral artery. It is treated by embolisation using a coil. PMID:22674935

Van der Woude, Daisy Adriana Annejan; Stegeman, Marjan; Seelen, Jan L

2011-01-01

394

Maternal smoking and congenital malformations: an epidemiological study  

Microsoft Academic Search

In a case-control study undertaken in several hospitals in Connecticut, it was found that women who reported smoking more than 20 cigarettes a day during pregnancy had a relative risk of about 1.6 for congenital malformations in the offspring of that pregnancy compared with women who said they had not smoked at all during pregnancy. However, there was no significant

J L Kelsey; T Dwyer; T R Holford; M B Bracken

1978-01-01

395

Development and malformations of the human pyramidal tract  

Microsoft Academic Search

The corticospinal tract develops over a rather long period of time, during which malformations involving this main central motor pathway may occur. In rodents, the spinal outgrowth of the corticospinal tract occurs entirely postnatally, but in primates largely prenatally. In mice, an increasing number of genes have been found to play a role during the development of the pyramidal tract.

H. J. ten Donkelaar; M. M. Y. Lammens; P. Wesseling; A. Hori; A. J. M. Keyser; J. J. Rotteveel

2004-01-01

396

Spitz nevus arising upon a congenital glomuvenous malformation.  

PubMed

There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM. PMID:22304367

Arica, Deniz A; Arica, Ibrahim E; Yayli, Savas; Cobanoglu, Umit; Akay, Bengu N; Anadolu, Rana; Bahadir, Sevgi

2013-01-01

397

A syndrome including thumb malformations, microcephaly, short stature, and hypogonadism  

Microsoft Academic Search

We report on eight patients from seven different families affected with a syndrome which includes thumb defects, short stature, microcephaly, and mental retardation. Most of the patients had additional malformations, in particular amenorrhoea and azoospermia in the adults. There were no haematological manifestations and the chromosomes were normal without evidence of breakage even after stimulation. In five of the cases

J Zlotogora; J Dagan; A Ganen; M Abu-Libdeh; Z Ben-Neriah; T Cohen

1997-01-01

398

LIMITED PCB ANTAGONISM OF TCDD-INDUCED MALFORMATIONS IN MICE  

EPA Science Inventory

C57BL/6N mice, used to model induction of cleft palate and kidney malformations in offspring following maternal treatment with TCDD, were dosed on gestation day (gd) 9 with ,2',4,4',5,5'-hexachloro-biphenyl (HCB) (62.5, 125, 250, 500. 1000 mg/kg) and/or gd 10 with 2,3,7,8-tetrach...

399

Neurodevelopmental outcomes in children with cerebellar malformations: a systematic review.  

PubMed

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 literature search of studies describing neurodevelopmental outcomes of cerebellar malformations between January 1997 and December 2007. Overall, the data suggested that children with isolated inferior vermis hypoplasia (IVH) and mega cisterna magna (MCM) have a good developmental outcome, whereas children with molar tooth sign/Joubert syndrome, vermis hypoplasia, pontocerebellar hypoplasia (PCH) type II, and cerebellar agenesis experience moderate to severe global developmental delays. Reports for Dandy-Walker malformation (DWM) were conflicting; however, the presence of a normally lobulated vermis and the absence of associated brain anomalies were associated with a more favourable outcome. Finally, children with isolated cerebellar hypoplasia experienced fewer impairments. Important methodological limitations highlighted include a lack of standardized outcome measure use in 79% of studies and the predominant use of retrospective study designs (85%), with 40% limited to case reports or case-series. In summary, rigorous outcome studies describing the spectrum of disabilities in survivors are urgently needed to accurately delineate the long-term neurodevelopmental consequences of cerebellar malformations. PMID:19191827

Bolduc, Marie-Eve; Limperopoulos, Catherine

2009-04-01

400

Stories and photographs of William A. Arnold (1904-2001), a pioneer of photosynthesis and a wonderful friend.  

PubMed

William A. Arnold discovered many phenomena in photosynthesis. In 1932, together with Robert Emerson, he provided the first experimental data that led to the concept of a large antenna and a few reaction centers (photosynthetic unit); in 1935, he obtained the minimum quantum requirement of 8-10 for the evolution of one O2 molecule; in 1951, together with Bernard L. Strehler, he discovered delayed fluorescence (also known as delayed light emission) in photosynthetic systems; and in 1956, together with Helen Sherwood, he discovered thermoluminescence in plants. He is also known for providing a solid-state picture of photosynthesis. Much has been written about him and his research, including many articles in a special issue of Photosynthesis Research (Govindjee et al. (eds.) 1996); and a biography of Arnold, by Govindjee and Srivastava (William Archibald Arnold (1904-2001), 2014), in the Biographical Memoirs of the US National Academy of Sciences, (Washington, DC). Our article here offers a glimpse into the everyday life, through stories and photographs, of this remarkable scientist. PMID:24861897

Choules, Lucinda; Govindjee

2014-10-01

401

Is a Swine Model of Arteriovenous Malformation Suitable for Human Extracranial Arteriovenous Malformation? A Preliminary Study  

SciTech Connect

Objective: A chronic arteriovenous malformation (AVM) model using the swine retia mirabilia (RMB) was developed and compared with the human extracranial AVM (EAVM) both in hemodynamics and pathology, to see if this brain AVM model can be used as an EAVM model. Methods: We created an arteriovenous fistula between the common carotid artery and the external jugular vein in eight animals by using end-to-end anastomosis. All animals were sacrificed 1 month after surgery, and the bilateral retia were obtained at autopsy and performed hematoxylin and eosin staining and immunohistochemistry. Pre- and postsurgical hemodynamic evaluations also were conducted. Then, the blood flow and histological changes of the animal model were compared with human EAVM. Results: The angiography after operation showed that the blood flow, like human EAVM, flowed from the feeding artery, via the nidus, drained to the draining vein. Microscopic examination showed dilated lumina and disrupted internal elastic lamina in both RMB of model and nidus of human EAVM, but the thickness of vessel wall had significant difference. Immunohistochemical reactivity for smooth muscle actin, angiopoietin 1, and angiopoietin 2 were similar in chronic model nidus microvessels and human EAVM, whereas vascular endothelial growth factor was significant difference between human EAVM and RMB of model. Conclusions: The AVM model described here is similar to human EAVM in hemodynamics and immunohistochemical features, but there are still some differences in anatomy and pathogenetic mechanism. Further study is needed to evaluate the applicability and efficacy of this model.

Lv, Ming-ming, E-mail: lvmingming001@163.com [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China); Fan, Xin-dong, E-mail: fanxindong@yahoo.com.cn [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China); Su, Li-xin, E-mail: sulixin1975@126.com [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)

2013-10-15

402

Epizootic of ovine congenital malformations associated with Schmallenberg virus infection.  

PubMed

Epizootic outbreaks of congenital malformations in sheep are rare and have, to the best of our knowledge, never been reported before in Europe. This paper describes relevant preliminary findings from the first epizootic outbreak of ovine congenital malformations in the Netherlands. Between 25 November and 20 December 2011, congenital malformations in newborn lambs on sheep farms throughout the country were reported to the Animal Health Service in Deventer. Subsequently, small ruminant veterinary specialists visited these farms and collected relevant information from farmers by means of questionnaires. The deformities varied from mild to severe, and ewes were reported to have given birth to both normal and deformed lambs; both male and female lambs were affected. Most of the affected lambs were delivered at term. Besides malformed and normal lambs, dummy lambs, unable to suckle, were born also on these farms. None of the ewes had shown clinical signs during gestation or at parturition. Dystocia was common, because of the lambs' deformities. Lambs were submitted for post-mortem examination, and samples of brain tissue were collected for virus detection. The main macroscopic findings included arthrogryposis, torticollis, scoliosis and kyphosis, brachygnathia inferior, and mild-to-marked hypoplasia of the cerebrum, cerebellum and spinal cord. Preliminary data from the first ten affected farms suggest that nutritional deficiencies, intoxication, and genetic factors are not likely to have caused the malformations. Preliminary diagnostic analyses of precolostral serum samples excluded border disease virus, bovine viral diarrhoea virus, and bluetongue virus. In December 2011, samples of brain tissue from 54 lambs were sent to the Central Veterinary Institute of Wageningen University Research, Lelystad. Real-time PCR detected the presence of a virus, provisionally named the Schmallenberg virus, in brain tissue from 22 of the 54 lambs, which originated from seven of eight farms that had submitted lambs for post-mortem examination. This Schmallenberg virus was first reported in Germany and seems to be related to the Shamonda, Aino, and Akabane viruses, all of which belong to the Simbu serogroup of the genus Orthobunyavirus of the family Bunyaviridae. These preliminary findings suggest that the Schmallenberg virus is the most likely cause of this epizootic of ovine congenital malformations, which is the first such outbreak reported in Europe. PMID:22393844

van den Brom, R; Luttikholt, S J M; Lievaart-Peterson, K; Peperkamp, N H M T; Mars, M H; van der Poel, W H M; Vellema, P

2012-02-01

403

Arteriovenous malformation of the mandible and parotid gland  

PubMed Central

Arteriovenous malformations (AVMs) of the jaws are relatively rare, with fewer than 200 cases reported in the literature. Their real importance lies in their potential to result in exsanguination, which usually follows an unrelated treatment, such as tooth extraction, surgical intervention, puncture wound or blunt injury in involved areas, with the dentist unaware of the existence of the AVM. The present case illustrates an AVM in an 18-year-old female with swelling on the right side of the face. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigation diagnosed it as high-flow vascular malformation. We ascertain the importance of dentists' awareness of the fatal outcome of these lesions and emphasize that, prior to performing any procedure, necessary investigations should always be done. PMID:22282511

Shailaja, S R; Manika; Manjula, M; Kumar, L V

2012-01-01

404

Congenital malformations of the vertebral column in ancient amphibians.  

PubMed

Temnospondyls, the largest group of Palaeozoic and Mesozoic amphibians, primitively possess rhachitomous vertebrae with multipartite centra (consisting of one horse-shoe-shaped inter- and paired pleurocentra). In a group of temnospondyls, the stereospondyls, the intercentra became pronounced and disc-like, whereas the pleurocentra were reduced. We report the presence of congenital vertebral malformations (hemi, wedge and block vertebrae) in Permian and Triassic temnospondyls, showing that defects of formation and segmentation in the tetrapod vertebral column represent a fundamental failure of somitogenesis that can be followed throughout tetrapod evolution. This is irrespective of the type of affected vertebra, that is, rhachitomous or stereospondylous, and all components of the vertebra can be involved (intercentrum, pleurocentrum and neural arch), either together or independently on their own. This is the oldest known occurrence of wedge vertebra and congenital block vertebra described in fossil tetrapods. The frequency of vertebral congenital malformations in amphibians appears unchanged from the Holocene. PMID:23551141

Witzmann, F; Rothschild, B M; Hampe, O; Sobral, G; Gubin, Y M; Asbach, P

2014-04-01

405

Resection of pontine cavernous malformation through the pontomedullary sulcus.  

PubMed

In this video, we illustrate a right far-lateral craniotomy for resection of a 13-mm cavernous malformation of the pons in a healthy 53-year-old female patient presenting with diplopia and right 6th nerve palsy. The cavernous malformation was surrounded by normal pons, but was within 1 mm of the pontomedullary sulcus. The lesion was exposed from below through a far lateral craniotomy and accessed through the vasoaccessory triangle, superior to olivary nucleus and 12th cranial nerve. The alternative retrosigmoid craniotomy would have involved significant transgression of the middle cerebellar peduncle. The patient had gross-total resection and some temporary increase in her abducens nerve palsy without any complication. The video can be found here: http://youtu.be/8nOnrnTk3Tg . PMID:24380516

Abla, Adib Adnan; Clark, Aaron J; Lawton, Michael L

2014-01-01

406

Cloacal malformation: embryology, anatomy, and prenatal imaging features.  

PubMed

Cloacal malformation is a rare but important anomaly. Prenatal diagnosis is possible with knowledge of the distinctive imaging features. The purpose of this case series is to illustrate characteristic prenatal sonographic and magnetic resonance imaging features of cloacal malformation using imaging from 6 cases seen at a single academic center to augment published data. The imaging feature common to all cases was a central cystic pelvic mass containing a characteristic fluid-fluid level. Additional anomalies include uterine and vaginal duplication, hydronephrosis, and lumbosacral anomalies. Prenatal magnetic resonance imaging showed the absence of the normal T1-hyperintense meconium-filled rectum in all cases. Prenatal diagnosis may affect immediate neonatal care (eg, immediate drainage of hydrocolpos) with an ultimate improved outcome. PMID:23091258

Winkler, Nicole S; Kennedy, Anne M; Woodward, Paula J

2012-11-01

407

[Facial venous malformation presented with an unusual course].  

PubMed

Venous malformations are constitutionally-dependent vascular anomalies. In contrast to haemangiomas, they show no spontaneous regression. We describe here the case of a 21-year-old woman with a very severe facial disfigurement caused by a large venous malformation. After intensive consultation, advice and planning, the patient decided to undergo therapy and was admitted to hospital for treatment by an interdisciplinary team, consisting of a radiologist and a plastic surgeon. Under general anaesthesia, percutaneous sclerosis was performed twice. This enabled thrombosing and subsequent extensive resection of the monstrously large tumour. The well-planned and efficiently performed surgery helped the patient to gain a significant improvement in her quality of life. PMID:19085823

Piza-Katzer, H; Waldenberger, P

2009-04-01

408

Juvenile Pilocytic Astrocytoma in Association with Arteriovenous Malformation  

PubMed Central

Summary Pilocytic astrocytomas are highly vascular, relatively common primary brain tumors in the pediatric population, but their association with a true arteriovenous malformation (AVM) is extremely rare. We describe an eight-year-old girl with a right supratentorial juvenile pilocytic astrocytoma (WHO grade I) with an angiographically documented AVM entangled in the tumor mass who presented with intracranial hemorrhage due to a ruptured anterior choroidal artery pseudoaneurysm encased in the lesion. The AVM nidus as well as the hemorrhage site was embolized with Onyx. A literature review revealed only one previous report of a true intermixture of these two lesions. We hypothesize whether the association of vascular malformations and primary brain tumors are merely coincidental or if they point to the existence of a distinct entity and/or a common etiologic factor. PMID:22681727

Soltanolkotabi, M.; Schoeneman, S.E.; Dipatri, A.J.; Hurley, M.C.; Ansari, S.A.; Rajaram, V.; Tomita, T.; Shaibani, A.

2012-01-01

409

Hepatocellular carcinoma in Budd-Chiari syndrome: enhancement patterns at dynamic gadolinium-enhanced T1-weighted MR imaging.  

PubMed

The objective of this study was to analyze the enhancement patterns at dynamic gadolinium-enhanced T1-weighted MR imaging in patients of Hepatocellular Carcinoma (HCC) with associated Budd-Chiari syndrome (BCS). The MR imaging findings in 10 patients of HCC with associated BCS were compared to those of 32 other patients of HCC without BCS. During the arterial phase, significantly more lesions with BCS were hyperintense than lesions without BCS; during the equilibrium phase, significantly more lesions with BCS were slightly hyperintense or isointense than lesions without BCS (P < 0.05 for both). For HCC, contrast enhancement on MRI shows different enhancement patterns between patients of HCC with associated BCS and those without BCS. PMID:24760630

Yang, Chun; Xu, Kai; Zheng, Junnian; Ma, Ping; Hu, Chunfeng; Li, Shaodong; Rong, Yutao; Lu, Xin; Zhang, Qingqiao; Zu, Maoheng; Hua, Rong; Zhang, Ling

2014-09-01

410

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

2013-10-01

411

Novel Image-Guided Management of a Uterine Arteriovenous Malformation  

SciTech Connect

The investigators present a novel image-guided embolization, not previously described, of a uterine arteriovenous malformation (AVM) resistant to endovascular management. The uterus was exposed surgically, and Histoacryl (Braun, Fulda, Germany) was injected directly into the nidus using ultrasound guidance and fluoroscopy. The patient had a successful full-term pregnancy after this procedure. This technique may be a useful alternative management strategy in patients with uterine AVM who fail traditional endovascular embolization and who still desire fertility.

Przybojewski, Stefan J., E-mail: drstefanp@hotmail.com; Sadler, David J. [University of Calgary, Diagnostic Imaging Department, Foothills Hospital (Canada)

2011-02-15

412

Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations  

Microsoft Academic Search

Purpose  Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 person-years. More than\\u000a half of children who have had a stroke have long-term neurological sequelae. The goal of this article is to review recent\\u000a literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations\\u000a as stroke

Lauren A. Beslow; Lori C. Jordan

2010-01-01

413

Arteriovenous Malformation of the Pancreas: Report of a Case  

Microsoft Academic Search

Arteriovenous malformation (AVM) of the pancreas (AVMP) is uncommon and generally asymptomatic; therefore, few cases have\\u000a so far been reported. The symptoms of AVMP include gastrointestinal bleeding, pain, and portal hypertension. Definitive diagnosis\\u000a is confirmed by angiographic study, and surgery is the only effective treatment. We report a case of AVMP confirmed by computed\\u000a tomography, magnetic resonance imaging, and angiographic

Jean M. Butte; Francisco Pacheco; Antonieta Solar; Fernando J. Crovari; Nicolás P. Jarufe

2007-01-01

414

Epidemiology, diagnostics and treatment of vascular tumours and malformations.  

PubMed

Vascular tumours and vascular malformations are common vasculose anomalies characteristic for dissimilar clinical course, specific biological as well as immune cytological and histological properties. Vascular lesions classification system and their detailed division into groups and subgroups were elaborated and implemented in Rome, in 1996, during meeting of the International Society for the Study of Vascular Anomalies (ISSVA). It was based on modification of an earlier going division by Mullikien and G?owacki from 1982. Infantile hemangiomas are the most numerous group of benign tumours of mesenchymal origin. Vascular malformations appear definitely less often. They are composed of normal endothelium lined displastic vessels which originate from vascular tissue abnormal morphogenesis. In contrast, in hemangiomas, at the proliferation stage, increased, multiplication of endothelial cells is observed as well as of fibroblasts, mastocytes and macrophages. Infantile hemangiomas are usually not present at the moment of birth and white chloasma with superficial teleangiectasis appears which increases within 3-4 weeks and gets bright red colour and reveal very characteristic clinical course basing on intensive growth period and involution long process. Vascular malformations are observed most often at the delivery moment or they may appear at an early childhood. They enlarge proportionally along with the child's growth and their sudden expansion may be triggered by an infection, hormonal changes or trauma. Contrary to hemangiomas, they do not subside spontaneously and their abrupt increase may result in impairment or deformation of important anatomical structures. Infantile hemangiomas and vascular malformations require different and individual treatments which are often multi-stage procedures carried on in specialistic centres of plastic surgery, vascular surgery or maxillofacial surgery. PMID:24979522

Wójcicki, Piotr; Wójcicka, Karolina

2014-01-01

415

Venous thoracic outlet syndrome caused by a congenital rib malformation  

PubMed Central

Summary Venous thoracic outlet syndrome (VTOS) represents a rare disorder. Hypertrophy of the anterior scalene musculature is the cause of the compression syndrome in most cases. To our knowledge, we describe the first reported case worldwide of a venous compression syndrome caused by a congenital malformation of the 1st and 2nd ribs. Treatment by transaxillary partial rib resection was necessary and a very good postoperative result was achieved. PMID:22544354

Kirschbaum, Andreas; Palade, Emanuel; Csatari, Zoltan; Passlick, Bernward

2012-01-01

416

Surgical treatment of symptomatic cavernous malformations of the brainstem  

Microsoft Academic Search

Summary  \\u000a Introduction and objectives. Cavernous malformations (CM) at the level of the brainstem, continue to present a challenge in therapeutic terms and are\\u000a an important source of controversy. Here we present our experience and the results obtained by adopting surgical treatment.\\u000a \\u000a \\u000a Materials and methods. The results of a consecutive series of 17 patients were studied. The surgical intervention was designed

R. G. Sola; P. Pulido; J. Pastor; M. Ochoa; J. Castedo

2007-01-01

417

Gated magnetic resonance imaging of congenital cardiac malformations  

SciTech Connect

Magnetic resonance (MR) images of a variety of cardiac malformations in 19 patients aged 1 week to 33 years were obtained using pulse plethysmographic- or ECG-gated spin echo pulse sequences. Coronal, axial, and sagittal images displaying intracardiac structures with excellent spatial and contrast resolution were acquired during systole or diastole. It is concluded that MR will be a valuable noninvasive method of diagnosing congenital heart disease.

Fletcher, B.D.; Jocobstein, M.D.; Nelson, A.D.; Riemenschneider, T.A.; Alfidi, R.J.

1984-01-01

418

Vascular Malformations of the Spine and Spinal Cord  

Microsoft Academic Search

Spinal vascular malformations are rare diseases with a wide variety of neurologic presentations. Their classification depends\\u000a on the differentiation of shunting versus nonshunting lesions, the latter being the spinal cord cavernomas. In the shunting\\u000a lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural\\u000a vascular shunts from the pial vascular

Timo Krings

2010-01-01

419

Cerebral arteriovenous malformation: prenatal and postnatal central blood flow dynamics  

Microsoft Academic Search

Using the Doppler technique, this study compared the prenatal and postnatal flow patterns of an infant with cerebral arteriovenous (AV) malformation. Fetal right ventricular end-diastolic dimension was 1.7 cm with right ventricular ejection equaling 66% of the combined cardiac output. Diastolic flow was reversed in the fetal aortic isthmus but forward-moving in the descending aorta, resulting in a watershed phenomenon.

D. J. Patton; J-C Fouron

1995-01-01

420

Volume measurement of cerebral arteriovenous malformations from angiography.  

PubMed

We designed software for measuring the volume of cerebral arteriovenous malformations from angiography and validated it against prescription volumes in radiosurgery. We aimed to create a model for the risk for complications as a function of volume, based on established outcome prediction models for Gamma Knife radiosurgery, but without the need for dose planning. We created an application for computing the volume of cerebral arteriovenous malformations from the intersection of two X-ray cones in stereotactic space. Volume measurements were compared with prescription volumes from dose planning, in phantoms and in patients treated with Gamma Knife radiosurgery for cerebral arteriovenous malformations. Previous studies of 1128 treated patients were used to calculate the risk for complication as a function of the nidus volume. In 63 patients volumes measured with either method correlated, R2 = 0.85. Volume as measured with the intersecting cone model (ICM) correlated with predicted Gamma Knife radiosurgery complication rate, R2 = 0.84. The ICM can thus be used for measurement of AVM volumes less than 10 cm3 from angiography. Outcome models from Gamma Knife radiosurgery may be applied, but with reduced exactness. Standardised AVM volume measurement is valuable for comparing outcome and for quantification of volume reduction after therapy, notably embolisation. Thus the optimal management plan may be selected in conjunction with diagnostic or therapeutic angiography. PMID:11071447

Söderman, M; Karlsson, B; Launnay, L; Thuresson, B; Ericson, K

2000-09-01

421

Environmental monitoring using malformed embryos of the amphipod Monoporeia affinis  

SciTech Connect

Reproduction variables of Monoporeia affinis, such as embryonic malformation were confirmed as the most sensitive variable, when soft bottom microcosms were exposed to metals such as cadmium and lead, arsenic, organic compounds such as 4,5,6 trichloroguaiacol, contaminated sediment from areas impacted by heavy metals and pulp mill effluents. The effects were demonstrated also in low concentrations that did not significantly affect the meiofauna community. The microcosm test-system with high ecological realism could offer a possibility to translate laboratory results to the natural environments. Field surveys outside different types of pulp mills and metal works on the coast of the Gulf of Bothnia have confirmed the laboratory results. Significantly higher levels of malformed embryos of Monoporeia affinis were demonstrated in the impacted areas in comparison with reference areas. The reproduction variables of Monoporeia affinis have been used in the national environmental monitoring program during two years and results indicated possibilities to distinguish between effects of xenobiotica and secondary eutrophication effects, such as unsaturated oxygen condition and occurrence of sulfides, which resulted in increased frequencies of dead eggs but not affected the frequencies of malformed eggs and embryos.

Sundelin, B.; Eriksson, A.K. [Stockholm Univ. (Sweden). Inst. of Applied Environmental Research

1995-12-31

422

Sonographic markers for early diagnosis of fetal malformations  

PubMed Central

Fetal malformations are very frequent in industrialized countries. Although advanced maternal age may affect pregnancy outcome adversely, 80%-90% of fetal malformations occur in the absence of a specific risk factor for parents. The only effective approach for prenatal screening is currently represented by an ultrasound scan. However, ultrasound methods present two important limitations: the substantial absence of quantitative parameters and the dependence on the sonographer experience. In recent years, together with the improvement in transducer technology, quantitative and objective sonographic markers highly predictive of fetal malformations have been developed. These markers can be detected at early gestation (11-14 wk) and generally are not pathological in themselves but have an increased incidence in abnormal fetuses. Thus, prenatal ultrasonography during the second trimester of gestation provides a “genetic sonogram”, including, for instance, nuchal translucency, short humeral length, echogenic bowel, echogenic intracardiac focus and choroid plexus cyst, that is used to identify morphological features of fetal Down’s syndrome with a potential sensitivity of more than 90%. Other specific and sensitive markers can be seen in the case of cardiac defects and skeletal anomalies. In the future, sonographic markers could limit even more the use of invasive and dangerous techniques of prenatal diagnosis (amniocentesis, etc.). PMID:24179631

Renna, Maria Daniela; Pisani, Paola; Conversano, Francesco; Perrone, Emanuele; Casciaro, Ernesto; Renzo, Gian Carlo Di; Paola, Marco Di; Perrone, Antonio; Casciaro, Sergio

2013-01-01

423

[Complications in the evolution of haemangiomas and vascular malformations].  

PubMed

The differentiation of haemangiomas and vascular malformations is histological, clinical and prognostic. Although the majority of haemangiomas evolve towards spontaneous resolution, as many as 10% of cases can develop complications with ulceration, pain and haemorrhaging. Besides, the localisation of haemangiomas in the head and neck, next to vital structures, can compromise their functions. Hence, compression of the airway might be a vital emergency. Periorbital haemangiomas can give rise to amblyopia due to sensory deprivation or due to a restrictive strabismus. Lumbosacral haemangiomas must be studied with Nuclear Magnetic Resonance because of their frequent association with alterations in the midline at the level of the spine, anus, genitals or kidneys. Amongst visceral haemangiomas, hepatic haemangiomas are the most serious due to their association with congestive cardiac insufficiency. The association of extensive facial haemangiomas with anomalies of the central nervous system, vascular, cardiac, ocular and sternal anomalies, is denominated PHACE syndrome and is frequently complicated by mental deficiency, convulsions or ictus. Vascular malformations of trigeminal localisation are associated in up to 15% of cases with glaucoma or choroidal or leptomeningeal haemangiomas (Sturge-Weber syndrome). Combined vascular malformations localised in the extremities can become complicated with thrombophlebitis, regional osteolysis and even distant thromboembolisms (Klippel-Treneaunay Syndrome). On the other hand, there is a coagulopathy due to consumption (Kassabach-Merrit Syndrome) that can complicate some vascular tumours such as the Kaposiform haemangioendothelioma and the tufted angioma. Finally, the complications of the treatments employed are reviewed. PMID:15148512

Belzunce, A; Casellas, M

2004-01-01

424

Congenital malformations of the central nervous system in spontaneous abortions.  

PubMed Central

A study of 2620 pregnancies ending in spontaneous abortion revealed a CNS defect in 3.6% of embryos and fetuses, and 3% of all complete conceptuses. The type of malformation observed varied with the gestational age at expulsion, encephaloceles being predominant in earlier specimens, while more typical anencephalus and spina bifida were more common among later abortions. Chromosome abnormalities were found in 40% of abortuses with CNS defects, but were almost entirely confined to those which were still at the embryonic stage of development. 53% of the latter were chromosomally abnormal, which is the same as the proportion found among embryos without a CNS malformation. Using published life-tables of recognized pregnancies it was estimated that the prevalence of anencephalus, spina bifida, or related malformation (other than hydrocephalus), without a chromosome anomaly, is 5.3 per thousand conceptuses at the beginning of the eighth week of gestation. By comparing this with the prevalence in total births, it was further estimated that only 24% of these are born alive, with 54% aborting spontaneously and 22% being stillborn. PMID:775092

Creasy, M R; Alberman, E D

1976-01-01

425

Guidelines for the treatment of head and neck venous malformations  

PubMed Central

Venous malformation is one of the most common benign vascular lesions, with approximately 40% of cases appearing in the head and neck. They can affect a patient’s appearance and functionality and even cause life-threatening bleeding or respiratory tract obstruction. The current methods of treatment include surgery, laser therapy, sclerotherapy, or a combined. The treatment of small and superficial venous malformations is relatively simple and effective; however, the treatment of deep and extensive lesions involving multiple anatomical sites remains a challenge for the physicians. For complex cases, the outcomes achieved with one single treatment approach are poor; therefore, individualized treatment modalities must be formulated based on the patient’s condition and the techniques available. Comprehensive multidisciplinary treatments have been adapted to achieve the most effective results. In this paper, based on the national and international literature, we formulated the treatment guidelines for head and neck venous malformations to standardize clinical practice. The guideline will be renewed and updated in a timely manner to reflect cutting-edge knowledge and to provide the best treatment modalities for patients. PMID:23724158

Zheng, Jia Wei; Mai, Hua Ming; Zhang, Ling; Wang, Yan An; Fan, Xin Dong; Su, Li Xin; Qin, Zhong Ping; Yang, Yao Wu; Jiang, Yin Hua; Zhao, Yi Fang; Suen, James Y

2013-01-01

426

OBESITY AND THE RISK AND DETECTION OF FETAL MALFORMATIONS  

PubMed Central

The incidence of obesity in pregnancy has increased over the past two decades, with nearly 50% of U.S. women aged 15–49 are classified as overweight or obese. Obesity (independent of diabetes) among gravidae poses unique risks which extend towards the fetus, with several large population-based analyses demonstrating independent increased risks for fetal malformations including neural tube defects, cardiac anomalies, and orofacial clefts as well as stillbirth and macrosomia. Unfortunately, several lines of evidence also suggest that the quality of the prenatal fetal anatomic survey and certain aspects of prenatal diagnostic screening programs are significantly limited. The net effect is that among obese gravidae, the increased risk of fetal anomalies is further offset by a concomitant diminished ability to sonographically detect such malformation in the prenatal interval. The purpose of this summary review is to systematically examine the evidence suggesting an increased risk of fetal malformations in obese gravidae, the contributing role of diabetes, and the limitations of prenatal diagnostic and sonographic screening among this at-risk population. PMID:22713503

RACUSIN, Diana; STEVENS, Blair; CAMPBELL, Genevieve; AAGAARD-TILLERY, Kjersti

2012-01-01

427

Rapid development of a hepatocellular carcinoma in isolated thrombosis of hepatic veins (classic Budd–Chiari syndrome): case report and review of literature  

PubMed Central

Budd–Chiari syndrome and membranous obstruction of the inferior vena cava frequently result in the development of mostly benign hepatic lesions. In cases of membranous obstruction of the inferior vena cava, which is prevalent mostly in the East, these lesions often progress to hepatocellular carcinoma. In contrast, malignant transformation has not yet been recognised in patients with isolated hepatic vein thrombosis. We report the case of a 37-year-old male Caucasian who presented with acute Budd–Chiari syndrome without involvement of the inferior vena cava. Despite porto-caval shunting, a hepatocellular carcinoma developed within several months. Three hepatic lesions were treated by radiofrequency thermal ablation until liver transplantation was performed. This report emphasises the possibility of malignant transformation of regenerative nodules in patients with disturbed hepatic perfusion in general. Physicians must be aware of this when assessing regenerative nodules, especially as no unambiguous predictors for the development of hepatocellular carcinoma have been identified so far. PMID:22125582

Walldorf, Jens; Tannapfel, Andrea; Holzhausen, Hans Jürgen; Wittekind, Christian; Seufferlein, Thomas; Settmacher, Utz; Fleig, Wolfgang E; Dollinger, Matthias M

2009-01-01

428

Myeloproliferative neoplasms and recurrent thrombotic events in patients undergoing liver transplantation for budd-Chiari syndrome: a single-center experience.  

PubMed

Background Budd-Chiari syndrome is a heterogeneous disease. The role of liver transplantation as a treatment option has been discussed since 1976. Many cases are related to underlying myeloproliferative neoplasms associated with prothrombotic propensity. The aim of this study was to evaluate the long-term clinical outcome after liver transplantation for Budd-Chiari syndrome at our center, with special emphasis on recurrent thrombosis and underlying myeloproliferative disorders. Material and Methods A medical records search revealed 25 patients transplanted at our center for Budd-Chiari syndrome between 2000 and 2009. Indications for transplantation were complications of end-stage liver disease or acute liver failure. Results Ten patients were men (40.0%). Median age of recipients at transplantation was 29.0 (17-51) years. Eighteen patients (72%) had evidence of myeloproliferation, 1 had paroxysmal nocturnal hemoglobinuria, and 6 had idiopathic disease. In 55.5% of cases eventually diagnosed with myeloproliferative neoplasms, Budd-Chiari syndrome was their initial presentation. All patients were maintained on long-term post-transplant anticoagulation protocol. The median follow-up time was 58.8 months. Four patients (16%) died during follow-up. Acute graft rejection occurred in 16% of cases. During the observation period, 5 patients had recurrent thrombotic events. The 5-year patient and graft survival rate was 84%. No case of transformation to acute leukemia was seen. Conclusions Our data show satisfactory long-term survival of patients and grafts in the study group. Occult course of myeloproliferative neoplasms is frequent in this population and exceeds 50%. We observed recurrent thrombosis in 20% of recipients. PMID:25394736

Oldakowska-Jedynak, Urszula; Ziarkiewicz, Mateusz; Ziarkiewicz-Wróblewska, Bogna; Dwilewicz-Trojaczek, Jadwiga; Górnicka, Barbara; Nyckowski, Pawe?; Paluszkiewicz, Rafa?; Wróblewski, Tadeusz; Zieniewicz, Krzysztof; Patkowski, Waldemar; P?czek, Leszek; Jedrzejczak, Wies?aw Wiktor; Krawczyk, Marek

2014-01-01

429

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

430

Bill malformations in double-crested cormorants with low exposure to organochlorines  

SciTech Connect

Eight of 20 newly hatched double-crested cormorants (Phalacrocorax auritus), captured at Dore Lake (Saskatchewan, Canada) and raised in captivity, developed malformed bills when they were 2 to 3 weeks old. Malformation was characterized by abnormal flexure and rotation of the maxilla and mandible, resulting in a crossed bill. By radiography, the premaxillary and dental bones were misshapen. Morphologically similar malformed bills in free-living comorants have been attributed to exposure to polyhalogenated aromatic hydrocarbons. However, the concentrations of total PCBs in the livers of these captive cormorants with malformed bills and in their diet were lower than have been previously associated with such malformations and were considered too low to have been the cause. The bill malformations may have been caused by deficiency of vitamin D{sub 3}, because the cormorants were kept indoors without exposure to ultraviolet light and were fed frozen fish that may have been deficient in this vitamin.

Kuiken, T.; Fox, G.A.; Danesik, K.L.

1999-12-01

431

Thoracolumbar spinal vascular malformation as a rare cause of isolated intraventricular hemorrhage.  

PubMed

Spinal vascular malformations are rare vascular lesions that most frequently present with back pain, radiculopathy, and/or myelopathy. Neurological decline is typically secondary to progressive radiculopathy, myelopathy, venous thrombosis, and stroke. Few case reports have described thoracolumbar spinal vascular malformations that present with both subarachnoid and intraventricular hemorrhage. This is the first reported case of a thoracolumbar spinal vascular malformation presenting with isolated intraventricular hemorrhage on initial imaging followed by acute and fatal rehemorrhage. PMID:24784978

Marlin, Evan S; Entwistle, John J; Arnold, Michael A; Pierson, Christopher R; Governale, Lance S

2014-07-01

432

Combined laparoscopic and cystoscopic injection sclerotherapy for bladder venous malformation: a novel technique.  

PubMed

Treatment of vascular malformations of the urinary bladder can be challenging. We report a case of bladder venous malformation treated with sodium tetradecyl sulphate (STS 3%) sclerotherapy, using a combined cystoscopic and percutaneous transperitoneal laparoscopy guided approach. When cystoscopic views are poor, the laparoscopic approach is a useful adjunct to aid sclerotherapy of bladder venous malformation. This technique has not been previously described. PMID:22841403

Sinha, C K; Barnacle, A; Mushtaq, I; Cherian, A

2013-02-01

433

Papillary endothelial hyperplasia in association with vascular malformation of the hand.  

PubMed

Vascular malformations are uncommonly encountered in the hand. This case report involves the unique case of a rapidly enlarging mass associated with a known vascular malformation. Final pathology revealed dense reactive fibrous tissue, with prominent blood vessels, consistent with a venous malformation and associated papillary endothelial hyperplasia. The intraoperative finding of two separately identifiable masses, one fibrotic and one vascular, has not previously been reported. PMID:24875342

Alves, Kristin; Bauer, Andrea; Jupiter, Jesse

2014-01-01

434

Aortic Runoff as a Sign of Intracranial Arteriovenous Malformation: Report of Two Cases  

PubMed Central

Background Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion. Case Presentation We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranial arteriovenous malformation and misdiagnosis of congenital heart disease. Conclusion Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation and had the major role in early diagnosis. PMID:23724190

Moradian, Maryam; Nokhostin-Davari, Paridokht; Merajie, Mahmood; Pouraliakbar, Hamid-Reza

2013-01-01

435

Emerging role of contrast-enhanced MRI in diagnosing vascular malformations.  

PubMed

Vascular malformations comprise a diverse and rare group of lesions which generally pose a formidable treatment challenge. Requisite for optimal surgical planning are imaging modalities capable of delineating involved anatomy and malformation flow characteristics. In this regard, we and others have purported the advantages of contrast-enhanced MRI. Here, we review the current body of literature regarding the emerging of role of contrast enhanced MRI for the management of vascular malformations. PMID:25301311

Turley, Ryan S; Lidsky, Michael E; Markovic, Jovan N; Shortell, Cynthia K

2014-07-01

436

Cervical spine malformation in cornelia de lange syndrome: a report of three patients.  

PubMed

Cornelia de Lange syndrome (CdLS) is a complex genetic disease with skeletal involvement mostly related to upper limb malformations. We report on three males with clinical and molecular diagnoses of CdLS. Besides typical CdLS features, all showed different cervical spine malformations. To the best of our knowledge, this is an unusual malformation in the CdLS phenotypic spectrum. PMID:24668777

Bettini, Laura Rachele; Locatelli, Laura; Mariani, Milena; Cianci, Paola; Giussani, Carlo; Canonico, Francesco; Cereda, Anna; Russo, Silvia; Gervasini, Cristina; Biondi, Andrea; Selicorni, Angelo

2014-06-01

437

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

438

A Case of Pulsatile Tinnitus from the Atherosclerosis and Atheroma in Superior Labial Artery and Facial Artery  

PubMed Central

Tinnitus is one of the most common symptoms in an audiologic field. It can be classified as either as subjective or objective; former referring to the sensation heard by both patient and examiner. Pulsatile tinnitus is perceived as sounds that vary in frequency, intensity and duration. The cause of pulsatile tinnitus include high jugular bulb, benign intracranial hypertension, glomus tumors, carotid artery stenosis, vascular lesions of the temporal bone, arteriovenous malformation, aneurysms, and Arnold-Chiari malformation. Vascular tinnitus is most common (7.6%). Recently, the authors experienced one case of the pulsatile tinnitus caused by atherosclerosis and atheroma in superior labial artery & facial artery. After surgery, the symptom had disappeared. We report a unique case regarding the objective tinnitus with the literature review. PMID:24653894

Kim, Jung Min; Kim, Choon Dong

2012-01-01

439

Clinical Features: Goldberg-Shprintzen megacolon syndrome (GOSHS, OMIM #609460) is a multiple malformation disorder  

E-print Network

malformation disorder characterized by Hirschsprung megacolon, microcephaly, hypertelorism, submucous cleft:185-189. 2. Hurst, JA., et al. Unknown syndrome: Hirschsprung's disease, microcephaly, and iris coloboma

Ober, Carole

440

Complex thoracic malformations: is there an association between adolescent idiopathic scoliosis and pectus excavatum?.  

E-print Network

??Study Design: This is a retrospective review of 220 patients with adolescent idiopathic scoliosis (AIS).Objectives: When combined thoracic malformations occur scoliosis and pectus excavatum (PE)… (more)

Berdan, Elizabeth Ann

2013-01-01

441

Trematode infection causes malformations and population effects in a declining New Zealand fish.  

PubMed

1. Animal malformations engender wide public and scientific concern because of associated environmental health risks. This is highlighted by increased incidence of limb malformations in amphibians associated with trematode infections and disturbance. Malformations may signal new emerging disease threats, but whether the phenomenon is broadly applicable across taxa, or has population-scale impacts, is unknown. 2. Malformations are widely reported in fish and, until now, have been attributed mainly to contaminants. We tested whether the trematode Telogaster opisthorchis caused severe malformations, leading to population effects, in Galaxias anomalus, a threatened New Zealand freshwater fish. 3. Experimental infection of larval fish caused increasing spinal malformation and mortality with infection intensity that closely matched field patterns. Field malformation frequency peaked in January (65%), before declining sharply in February (25%) and remaining low thereafter. 4. The peak occurred during a 'critical window' of larval development, with the decline coincident with a population crash, indicating that malformation was causing mortality in the field. 5. The occurrence of such critical developmental windows may explain why this mechanism of population impact has been overlooked. With global environmental stressors predicted to enhance trematode infections, our results show that parasite-induced malformation, and its population-scale impacts, could be more widespread than previously considered. PMID:19886894

Kelly, David W; Thomas, Harriet; Thieltges, David W; Poulin, Robert; Tompkins, Daniel M

2010-03-01

442

Single gene influences on radiologically-detectable malformations of the inner ear.  

PubMed

Inner ear malformations associated with hearing loss or vestibular dysfunction are discussed from the viewpoint of the etiologies of the malformation. Symptoms of classification of inner ear malformations are discussed. The significance of malformations of the cochlea and vestibular aqueduct to auditory function are discussed. Genetics features and characteristics of Branchio-oto-renal, Waardenburg's, Pendred's, DiGeorge's, Wildervanck, Fountain, and Treacher Collins syndromes are discussed in relation to ear abnormalities and hearing. Similar attention is given to genetic studies of nonsyndromic hearing loss. PMID:9777486

Smith, S D; Harker, L A

1998-01-01

443

Symptoms of Sleep Disordered Breathing in Children with Craniofacial Malformations  

PubMed Central

Study Objective: The purpose of this study was to investigate the frequency of sleep disordered breathing (SDB) symptoms in a clinical sample of children with congenital craniofacial malformations (CFM) followed at a tertiary medical center and non-selected for sleep problems. Methods: Cross-sectional study of 575 children aged 2-18 years followed at the Craniofacial Anomalies Program between March 2007 and May 2011. The Sleep-Related Breathing Disturbance scale of the Pediatric Sleep Questionnaire was used to screen for SDB, snoring, and sleepiness. A cutoff value ? 0.33 of the total answered questions identified children with positive screening for SDB symptoms. Results: Overall, 25% of children screened positive for SDB, 28% for snoring, and 20% for sleepiness. In children with non-syndromic CFM, those with Robin sequence had the highest frequency of SDB, snoring, and sleepiness (43%, 44%, and 38%, respectively). In children with syndromic CFM, velocardiofacial/ DiGeorge syndrome had the highest frequency of SDB and sleepiness (48% and 43%, respectively). Children with Treacher Collins had the highest frequency of snoring (83%). The presence of cleft palate was not associated with an increased frequency of SDB symptoms. Nevertheless, children with syndromic CFM, compared to those with non-syndromic CFM, had a higher SDB score (0.27 ± 0.21 vs.0.21 ± 0.19, p = 0.003) and were more likely to have sleepiness (26% vs. 18%, p = 0.05). Conclusions: Congenital craniofacial malformations in children are associated with high risk for SDB symptoms. Our findings should encourage a high index of suspicion for SDB in children with CFM, with a low threshold for further testing and close follow-up. Citation: Moraleda-Cibrián M; Edwards SP; Kasten SJ; Berger M; Buchman SR; O'Brien LM. Symptoms of sleep disordered breathing in children with craniofacial malformations. J Clin Sleep Med 2014;10(3):307-312. PMID:24634629

Moraleda-Cibrian, Marta; Edwards, Sean P.; Kasten, Steven J.; Berger, Mary; Buchman, Steven R.; O'Brien, Louise M.

2014-01-01

444

Treatment of vascular malformation of the gastrointestinal tract  

NASA Astrophysics Data System (ADS)

Vascular malformations of the gastrointestinal tract are rare phenomenon. They are generally manifested by upper or lower GI - bleeding and do not resolve spontaneously. Emergency intervention is necessary. This paper reports on 10 cases, treated in the Dept. of Pediatric surgery of the FU Berlin, recorded from 1981 to 1999. We use the Nd:YAG laser 1064 nm, Fibertom 5100, Dornier, Germany, with a 600nm barefiber. Reduction in size of the hemangiomas and stop of the GI-bleeding was achieved in all cases.

Waldschmidt, Juergen; Stroedter, L.; Doede, T.; Kischkel, A.

2000-06-01

445

Urothelial mucosal malformation: a rare cause for ureteropelvic junction obstruction.  

PubMed

Ureteropelvic junction obstruction (UPJO) constitutes a significant cause of morbidity in children and exists in a wide range of severity and clinical manifestations. The cause of UPJO remains largely unknown except for a small group, in which crossing vessels have been considered etiological. Herein we describe a unique case in which intraluminal occlusion was the result of mucosal malformation, characterized by invaginated and branching urothelial epithelium present in the lamina propria. We believe that the present case is the first such description of this type of alteration. PMID:16808629

Huang, Weei-Yuarn; Olumi, Aria F; Rosen, Seymour

2006-01-01

446

Management of palatal vascular malformation using absolute ethanol sclerotherapy  

PubMed Central

Arteriovenous malformation is treated by variety of techniques over the years. Sclerotherapy is considered an effective and conservative technique for the treatment of benign vascular lesions and replaced the traditional role of surgical therapy, especially for the venous lesions that are surgically difficult or at inaccessible areas. Absolute ethanol was adopted as a new sclerosant agent for this complex form of venous defects to improve overall treatment results with acceptable morbidity and recurrence rates. Sclerotherapy has the advantage of no external scaring, low cost, and few complications in comparison to the surgical treatment. PMID:24765386

El-Hakim, Ibrahim; Alyamani, Ahmed

2011-01-01

447

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

2011-12-01

448

Congenital malformations in offspring of women with hyperglycemia first detected during pregnancy  

Microsoft Academic Search

Objectives: Our aim was to determine risk factors for congenital malformations in offspring of women with hyperglycemia first detected during pregnancy (i.e., women with gestational diabetes). Study Design: A total of 3743 pregnancies complicated by gestational diabetes mellitus delivered at >20 weeks of gestation were reviewed for the presence of congenital malformations diagnosed before hospital discharge. Anomalies were categorized as

Ute M. Schaefer; Giulana Songster; Anny Xiang; Kathleen Berkowitz; Thomas A. Buchanan; Siri L. Kjos

1997-01-01

449

Screening for Systemic Manifestations of Vascular Malformations in Patients With Hereditary Haemorrhagic Telangiectasia (Osler Disease)  

Microsoft Academic Search

Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) is a disease characterized by systemic vascular malformations. Typical clinical manifestations are recurrent epistaxis and telangiectases of the skin and the mucous membranes. The syndrome is furthermore characterized by its hereditary aspect. The disease seems to be much more complicated than previously thought, mainly because of the accompanying vascular malformations in vital organs, like the

Ana Cerra Pohl; Jochen Alfred Werner; Benedikt Josef Folz

450

A review of contemporary options for medical management of hemangiomas, other vascular tumors, and vascular malformations.  

PubMed

Vascular anomalies include vascular tumors and vascular malformations. With growing pharmacologic options and parallels to cancer treatment and biology, the hematologist-oncologist has assumed a more prominent role in clinical care and research relating to these diagnoses. This also is a growing area for targeted therapies and drug repositioning. We performed a review of contemporary options for medical management of these lesions. PubMed was searched for "vascular anomaly", "hemangioma", "vascular malformation", "arteriovenous malformation", "capillary malformation", "cerebral cavernous malformation", "lymphatic malformation", and "venous malformation", each with "drug treatment" as a modifier. Manuscripts were reviewed to verify diagnoses, indications for treatment, dose-schedules, evidence of effectiveness, toxicities, and mechanisms of action. ClinicalTrials.gov also was reviewed for relevant trials. More than 20 agents were identified which have been used to treat vascular anomalies. Rigorous studies are lacking for many of these. The rarity of these tumors has limited development of medical approaches to treatment. Cooperative group trials will be needed to prove the effectiveness of drugs which have shown promise in cases and small series. The observant clinician remains a powerful tool for identifying potential new treatments for vascular tumors and malformations. PMID:23665062

Blatt, Julie; McLean, Thomas W; Castellino, Sharon M; Burkhart, Craig N

2013-09-01

451

Exceptional Multiplicity of Cerebral Arteriovenous Malformations Associated with Hereditary Hemorrhagic Telangiectasia (Osler Weber-Rendu Syndrome)  

Microsoft Academic Search

PURPOSE: To describe the clinical and imaging features of seven patients with hereditary hem- orrhagic telangiectasia and an exceptional number of cerebral arteriovenous malformations (AVMs). METHODS: One hundred thirty-six patients from a dedicated hereditary hemorrhagic telangiectasia clinic were screened systematically for cerebral AVMs by means of MR imaging. Thirty-one were found to have abnormalities suggestive of a vascular malformation. Eighteen

Christopher M. Putman; John C. Chaloupka; Robert K. Fulbright; Issam A. Awad; Robert I. White; Pierre B. Fayad

452

Congenital malformations after the use of inhaled budesonide in early pregnancy  

Microsoft Academic Search

Objective: To study possible teratogenic risks with the use of an inhaled glucocorticoid, budesonide, in early pregnancy.Methods: Using the Swedish Medical Birth Registry, congenital malformations were studied in 2014 infants whose mothers had used inhaled budesonide for asthma in early pregnancy. The presence of congenital malformations was checked further with auxilliary registries.Results: No increase in the general rate of congenital

Bengt Källén; Hakan Rydhstroem; Anders Åberg

1999-01-01

453

The Effect of Surgery for Split Spinal Cord Malformation on Neurologic and Urologic Function  

Microsoft Academic Search

The split spinal cord malformation (SSCM) is an occult spinal dysraphism which causes tethering of the spinal cord. We performed a retrospective analysis of 15 patients who had split cord malformations (without associated open neural tube defect) who underwent both pre- and postoperative urodynamic studies (UDS) in order to determine if a significant percentage of these patients, even in the

Mark R. Proctor; Stuart B. Bauer; R. Michael Scott

2000-01-01

454

Clinical Features Microcephaly-Capillary Malformation syndrome [MICCAP, OMIM#614261] is characterized by severe progressive  

E-print Network

4/13 Clinical Features Microcephaly-Capillary Malformation syndrome [MICCAP, OMIM#614261] is characterized by severe progressive microcephaly, early-onset refractory epilepsy, profound developmental delay Analysis for Microcephaly-Capillary Malformation Syndrome #12;4/13 References: 1. Carter MT, Geraghty MT

Das, Soma

455

Radionuclide blood pool scintigraphy in a child with intestinal arteriovenous malformation (juvenile angiodysplasia)  

Microsoft Academic Search

Arteriovenous (AV) malformation or angiodysplasia of the gastrointestinal tract is a very rare cause of bleeding in children. These lesions are congenital anomalies and thought to be of hamartomatous origin. We report on a 4-year-old child with an AV malformation of the distal ileum treated by local resection. A brief review of the relevant literature is included. The importance of

I. Garty; L. Siplovich; J. Horowitz; D. Miron; A. Verstandig; M. Dharan

1991-01-01

456

The cyclops and the mermaid: an epidemiological study of two types of rare malformation  

Microsoft Academic Search

Infants with cyclopia or sirenomelia are born at an approximate rate of 1 in 100,000 births. Eight malformation monitoring systems around the world jointly studied the epidemiology of these rare malformations: 102 infants with cyclopia, 96 with sirenomelia, and one with both conditions were identified among nearly 10.1 million births. Maternal age is somewhat increased for cyclopia, indicating the likely

B Källén; E E Castilla; P A Lancaster; O Mutchinick; L B Knudsen; M L Martínez-Frías; P Mastroiacovo; E Robert

1992-01-01

457

Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: Doppler sonographic screening in a large family  

Microsoft Academic Search

Background\\/Aims: The prevalence of hepatic vascular malformations in hereditary hemorrhagic telangiectasia has been estimated in the literature on clinical criteria, thus giving unreliable data. In our study the presence of hepatic vascular malformations in hereditary hemorrhagic telangiectasia was evaluated in a large Italian family by using Doppler sonography as screening technique. Doppler sonographic findings were compared to computed tomography and

Elisabetta Buscarini; Luigi Buscarini; Cesare Danesino; Mauro Piantanida; Giuseppe Civardi; Pietro Quaretti; Sandro Rossi; Michele Di Stasi; Matteo Silva

1997-01-01

458

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

459

Constructing masculinities under Thomas Arnold of Rugby (1828–1842): gender, educational policy and school life in an early?Victorian public school  

Microsoft Academic Search

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 using gender as

Fabrice Neddam

2004-01-01

460

Measurements of Ice Crystal Growth Rates in Air at -5C and -10C K. G. Libbrecht and H. M. Arnold  

E-print Network

Measurements of Ice Crystal Growth Rates in Air at -5C and -10C K. G. Libbrecht and H. M. Arnold to: kgl@caltech.edu Abstract. We present experiments investigating the growth of ice crystals from understand the surface molecular dynamics that determine crystal growth rates and morphologies. [The figures

Libbrecht, Kenneth G.

461

LATINO HEALTH DISPARITIES (TENURE-TRACK FACULTY POSITION) The University of South Carolina's Arnold School of Public Health invites applications for a  

E-print Network

LATINO HEALTH DISPARITIES (TENURE-TRACK FACULTY POSITION) The University of South Carolina's Arnold in Latino Health Disparities. The position will be in the Department of Health Promotion, Education-nation families, and communities. USC is committed to research on health inequalities and disparities. Candidates

Almor, Amit

462

The Institute for Advanced Study has announced the appointment of Arnold J. Levine as professor of molecular biology in the School of Natural Sciences. Profes-  

E-print Network

The Institute for Advanced Study has announced the appointment of Arnold J. Levine as professor of molecular biology in the School of Natural Sciences. Profes- sor Levine was formerly a visiting professor to both basic and applied biological research. Under Professor Levine's leadership, the Center for Systems

463

Clinical, Genetic and Environmental Factors Associated with Congenital Vertebral Malformations  

PubMed Central

Congenital vertebral malformations (CVM) pose a significant health problem because they can be associated with spinal deformities, such as congenital scoliosis and kyphosis, in addition to various syndromes and other congenital malformations. Additional information remains to be learned regarding the natural history of congenital scoliosis and related health problems. Although significant progress has been made in understanding the process of somite formation, which gives rise to vertebral bodies, there is a wide gap in our understanding of how genetic factors contribute to CVM development. Maternal diabetes during pregnancy most commonly contributes to the occurrence of CVM, followed by other factors such as hypoxia and anticonvulsant medications. This review highlights several emerging clinical issues related to CVM, including pulmonary and orthopedic outcome in congenital scoliosis. Recent breakthroughs in genetics related to gene and environment interactions associated with CVM development are discussed. The Klippel-Feil syndrome which is associated with cervical segmentation abnormalities is illustrated as an example in which animal models, such as the zebrafish, can be utilized to provide functional evidence of pathogenicity of identified mutations. PMID:23653580

Giampietro, P.F.; Raggio, C.L.; Blank, R.D.; McCarty, C.; Broeckel, U.; Pickart, M.A.

2013-01-01

464

Antenatal and postnatal management of congenital cystic adenomatoid malformation.  

PubMed

Congenital thoracic malformations (CTMs) are a heterogeneous group of rare disorders that may involve the airways or lung parenchyma. The authors have focused on the condition that causes the most controversy, namely, congenital cystic adenomatoid malformation (CCAM). The reported incidence is 3.5 and 0.94 per 10,000 live births for CTMs and CCAMs respectively. Ultrasound is the antenatal imaging modality of choice for screening for CCAMs whilst magnetic resonance imaging is complimentary for morphological and volumetric evaluation of the foetal lung. Most CCAMs are detected antenatally with only a small proportion presenting postnatally. Only a few CCAMs cause foetal problems, with foetal hydrops being the best predictor of death. Although many CCAMs regress during pregnancy, most remain detectable postnatally by CT scans. Surgical excision of symptomatic lesions is relatively straightforward, but management of asymptomatic lesions is controversial. Some surgeons adopt a "wait and see" approach operating only on those patients who develop symptoms, but others operate on asymptomatic patients usually within the first year of life. Due to the potential of malignant transformation, children should have long term follow up. There is an urgent need to delineate the natural history of antenatally detected CCAMs to guide future management. PMID:22726873

Kotecha, S; Barbato, A; Bush, A; Claus, F; Davenport, M; Delacourt, C; Deprest, J; Eber, E; Frenckner, B; Greenough, A; Nicholson, A G; Antón-Pacheco, J L; Midulla, F

2012-09-01

465

PDCD10 Gene Mutations in Multiple Cerebral Cavernous Malformations  

PubMed Central

Cerebral cavernous malformations (CCMs) are vascular abnormalities that may cause seizures, intracerebral haemorrhages, and focal neurological deficits. Familial form shows an autosomal dominant pattern of inheritance with incomplete penetrance and variable clinical expression. Three genes have been identified causing familial CCM: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3. Aim of this study is to report additional PDCD10/CCM3 families poorly described so far which account for 10-15% of hereditary cerebral cavernous malformations. Our group investigated 87 consecutive Italian affected individuals (i.e. positive Magnetic Resonance Imaging) with multiple/familial CCM through direct sequencing and Multiplex Ligation-Dependent Probe Amplification (MLPA) analysis. We identified mutations in over 97.7% of cases, and PDCD10/CCM3 accounts for 13.1%. PDCD10/CCM3 molecular screening revealed four already known mutations and four novel ones. The mutated patients show an earlier onset of clinical manifestations as compared to CCM1/CCM2 mutated patients. The study of further families carrying mutations in PDCD10/CCM3 may help define a possible correlation between genotype and phenotype; an accurate clinical follow up of the subjects would help define more precisely whether mutations in PDCD10/CCM3 lead to a characteristic phenotype. PMID:25354366

Cigoli, Maria Sole; Avemaria, Francesca; De Benedetti, Stefano; Gesu, Giovanni P.; Accorsi, Lucio Giordano; Parmigiani, Stefano; Corona, Maria Franca; Capra, Valeria; Mosca, Andrea; Giovannini, Simona; Notturno, Francesca; Ciccocioppo, Fausta; Volpi, Lilia; Estienne, Margherita; De Michele, Giuseppe; Antenora, Antonella; Bilo, Leda; Tavoni, Antonietta; Zamponi, Nelia; Alfei, Enrico; Baranello, Giovanni; Riva, Daria; Penco, Silvana

2014-01-01

466

A tortuous proximal urethra in urorectal septum malformation sequence?  

PubMed

We observed a newborn boy with urorectal septum malformation sequence. Anomalies of the genitalia and rectum were present. He expired on the first day of life, due to severe lung hypoplasia. Autopsy showed a colon that ended in a blind sac, an enlarged bladder with no grossly visible urethra, and dysplastic kidneys. A cone-shaped tissue at the usual site of the bladder outlet contained tortuous and slit-like lumina, suggesting an undeveloped proximal urethra. The urethral structure was lined by transitional epithelium with squamous metaplasia. Many small buds-lined with columnar epithelium-branched from the urethral structure. These ductal buds lined with columnar epithelium stained for prostatic acid phosphatase. Basal cells surrounding the ductal buds stained for p63 and high molecular weight cytokeratin-supporting an interpretation that the buds were early prostatic ducts with normal histology. To our knowledge, these are the first histological images of an undeveloped, obstructed urethra associated with the urorectal septum malformation sequence. PMID:24665006

Lin, Henry J; Lugo, Hector; Tran, Thu; Tovar, Jason P; Corral, Julia; Zork, Noelia M; Smith, Lynne M; French, Samuel W; Barajas, Luciano

2014-05-01

467

CHARGE association in Sweden: malformations and functional deficits.  

PubMed

CHARGE association (CA) consists of a non-random association of ocular coloboma (C), heart anomaly (H), atresia of choanae (A), retarded growth and/or development (R), genital hypoplasia (G), and ear anomalies and/or hearing impairment (E). A prospective multidisciplinary study of 31 Swedish patients with CA was undertaken in order to describe the associated malformations and functional deficits, find possible etiological factors and identify critical time periods for the maldevelopment. The clinical files were analyzed, the mothers answered a questionnaire on history of prenatal events, and a clinical evaluation of systemic findings, vision, hearing, balance, speech, oral and swallowing function, and neuro-psychiatric function, especially autism, was performed. The most frequent physical abnormalities affected ears (90%), eyes (90%), brain (61%), heart (52%), retarded growth (48%), genitals (38%), choanae (35%), and facial nerve (32%). Sixty-one percent of the patients were visually impaired or blind, and 74% had hearing loss or deafness. Problems in balance, speech, and eating were common. Forty percent of the patients had autism/atypical autism, and 82% had developmental delay. Three children were born following assisted fertilization and two mothers had diabetes. The mothers reported infections, bleedings, and drug use during pregnancy. Analysis of possible critical time periods suggested that most malformations were produced early in pregnancy, mainly during post conceptual weeks 4, 5, and 6. A multidisciplinary approach is essential in the assessment and management of CA. PMID:15633180

Strömland, Kerstin; Sjögreen, Lotta; Johansson, Maria; Ekman Joelsson, Britt-Marie; Miller, Marilyn; Danielsson, Susanna; Billstedt, Eva; Gillberg, Christopher; Jacobsson, Catharina; Norinder, Jan Andersson; Granström, Gösta

2005-03-15

468

The Alimentary Tract Malformations in the Rabbit Thalidomide Embryopathy  

PubMed Central

Among 906 thalidomide exposed young from 6 different rabbit strains, 141 major malformations involving the alimentary tract were found. All but 4 of the defects can confidently be regarded as a drug effect. The lesions occurred in the derivatives of the foregut, hindgut and umbilicus. Foregut anomalies (68 specimens) comprised hiatus hernia, megaoesophagus, prepyloric notching of the greater curvature of the stomach and deficient septum formation between oesophagus and trachea. In many rabbits all 4 lesions were present together. Hindgut malformations (61 specimens) were made up of rectal atresia and a small number of diverticula and/or saccular dilatations of the terminal colon, some in company with atresia. The 32 examples of atresia which were examined in detail had a coexistent fistula extending to the urethra or the anus. Omphalocoele (6 specimens in one strain) was the umbilical defect. It is suggested that some of the lesions result from impeded septum formation in the laryngotracheal grouve and the cloaca. ImagesFigs. 7-12Figs. 25-30Figs. 13-20Figs. 1-6Figs. 21-24 PMID:5429073

Vickers, T. H.

1970-01-01