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Pediatric Radiology
Published in: Pediatric Radiology 12/2009

Open Access 01-12-2009 | Case Report

Brainstem disconnection

Authors: Curtis Duffield, Jennifer Jocson, Sandra L. Wootton-Gorges

Published in: Pediatric Radiology | Issue 12/2009

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Abstract

Brainstem disconnection is a very rare neonatal abnormality, with only seven cases reported. We report a unique case of a neonate who presented at delivery with hypertonia, dysmorphic facial features, and respiratory distress, as well as numerous musculoskeletal and genitourinary abnormalities. MRI of the brain showed disconnection between the pons and medulla with cerebellar hypoplasia and absent cerebellar peduncles. It aided in the description of the neurological and vascular anomalies associated with this diagnosis.
Literature
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Poretti A, Boltshauser E, Plecko B (2007) Brainstem disconnection: case report and review of the literature. Neuropediatrics 38:210–212CrossRefPubMed
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McCann E, Pilling D, Hesseling M et al (2005) Pontomedullary disconnection: fetal and neonatal considerations. Pediatr Radiol 35:812–814CrossRefPubMed
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Mamourian A, Miller G (1994) Neonatal pontomedullary disconnection with aplasia or destruction of the lower brain stem: a case of pontoneocerebellar hypoplasia? AJNR 15:1483–1485PubMed
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Sarnat HB, Benjamin DR, Siebert JR et al (2002) Agenesis of the mesencephalon and metencephalon with cerebellar hypoplasia: putative mutation in the EN2 gene—report of 2 cases in early infancy. Pediatr Dev Pathol 5:54–68CrossRefPubMed
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Bednarek N, Scavarda D, Mesmin F et al (2005) Midbrain disconnection: an aetiology of severe central neonatal hypotonia. Eur J Paediatr Neurol 9:419–422CrossRefPubMed
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Metadata
Title
Brainstem disconnection
Authors
Curtis Duffield
Jennifer Jocson
Sandra L. Wootton-Gorges
Publication date
01-12-2009
Publisher
Springer-Verlag
Published in
Pediatric Radiology / Issue 12/2009
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-009-1378-3

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