Neuropediatrics 2008; 39(2): 106-112
DOI: 10.1055/s-2008-1081460
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Cerebellar Cleft: A Form of Prenatal Cerebellar Disruption

A. Poretti 1 , R. J. Leventer 2 , F. M. Cowan 3 , M. A. Rutherford 3 , M. Steinlin 4 , A. Klein 1 , I. Scheer 5 , T. A. G. M. Huisman 5 , 6 , E. Boltshauser 1
  • 1Department of Neurology, University Children's Hospital of Zurich, Zurich, Switzerland
  • 2Children's Neuroscience Centre, Royal Children's Hospital and Murdoch Children's Research Institute, Melbourne, Australia
  • 3Department of Paediatrics and Imaging Sciences Department, MRC Clinical Sciences Centre, Hammersmith Campus, Imperial College, London, UK
  • 4Department of Neurology, University Children's Hospital of Berne, Berne, Switzerland
  • 5Department of Diagnostic Imaging, University Children's Hospital of Zurich, Zurich, Switzerland
  • 6Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
Further Information

Publication History

received 23.01.2008

accepted 10.06.2008

Publication Date:
31 July 2008 (online)

Abstract

In contrast to malformations, cerebellar disruptions have attracted little interest in the literature. We draw attention for the first time to the hypothesis that cerebellar clefts are residual changes following a prenatal cerebellar insult, and represent disruptions. We reviewed the clinical records and MR findings of six patients with a cerebellar cleft, two of whom also had prenatal MRI at 24 weeks of gestation. The clefts were located in the left cerebellar hemisphere in five cases, in the right in one patient. Other typical findings included disorderly alignment of the cerebellar folia and fissures, irregular gray/white matter junction, and abnormal arborization of the white matter in all patients. The cerebellar cleft extended into the fourth ventricle in three cases, and in two children cystic cortical lesions were seen. Supratentorial schizencephaly was found in two patients. In two patients there was a documented fetal cerebellar hemorrhage at 24 weeks of gestation. We conclude that cerebellar clefts are residual changes resulting from a prenatal cerebellar insult and consequently represent disruptions rather than primary malformations. The supratentorial findings are also in agreement with an acquired lesion. The outcome in these children was variable, mainly depending of the presence of supratentorial lesions.

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Correspondence

E. Boltshauser

Department of Pediatric Neurology

University Children's of Zurich

Steinwiesstraße 75

8032 Zurich

Switzerland

Phone: +41/44/266 73 30

Fax: +41/44/266 71 63

Email: Eugen.Boltshauser@kispi.uzh.ch

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