Document Detail


Vanishing cerebellum in myelomeningocoele.
MedLine Citation:
PMID:  11995957     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Reduced hindbrain herniation observed after intrauterine myelomeningocoele repair suggests that posterior fossa changes in myelomeningocoele are secondary results of prolonged prenatal spinal cerebrospinal fluid leak. Exceptionally, this transforaminal herniation results in 'degeneration' of cerebellar tissue, presumably due to mechanically induced ischaemia. This phenomenon was called 'vanishing cerebellum in Chiari II malformation'. We report three similar cases of this apparently rare finding. Pregnancies were normal. Cerebellar hypoplasia was already recognized in one instance by prenatal ultrasound at gestational week 25. Postnatal imaging was similar in all three patients showing small posterior fossa, beaked midbrain tectum, small brainstem without pontine prominence, reduced cerebellar tissue with virtual absence of one hemisphere and supratentorial hydrocephalus. Our series is too small to draw firm conclusions about predisposing risk factors for and consequences of vanishing cerebellum. Cerebellar damage can interfere with cognitive development, as shown in children with cerebellar agenesis/ hypoplasia, congenital ataxia and small cerebellum following prematurity. A final conclusion on the cognitive consequence of vanishing cerebellum cannot be drawn on the available literature and our limited observations, as one of our patients died at 3 months and another is still too young for appropriate testing. However, the third (aged 15 years) is very severely retarded.
Authors:
Eugen Boltshauser; Jacques Schneider; Spyros Kollias; Peter Waibel; Markus Weissert
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society     Volume:  6     ISSN:  1090-3798     ISO Abbreviation:  Eur. J. Paediatr. Neurol.     Publication Date:  2002  
Date Detail:
Created Date:  2002-05-08     Completed Date:  2002-10-01     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9715169     Medline TA:  Eur J Paediatr Neurol     Country:  England    
Other Details:
Languages:  eng     Pagination:  109-13     Citation Subset:  IM    
Affiliation:
University Children's Hospital, Zurich, Switzerland. eugen.boltshauser@kispi.unizh.ch
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Arnold-Chiari Malformation / diagnosis*
Atrophy
Brain Damage, Chronic / diagnosis
Cerebellum / pathology*
Child
Child, Preschool
Encephalocele / diagnosis*
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging
Male
Meningomyelocele / diagnosis*
Mental Retardation / diagnosis
Pregnancy
Prenatal Diagnosis

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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