Faculty Collaboration Database

Giant posterior fossa cavernous malformations in 2 infants with familial cerebral cavernomatosis: the case for early screening. Neurosurg Focus 2010 Sep;29(3):E18

Date

09/03/2010

Pubmed ID

20809759

DOI

10.3171/2010.5.FOCUS10119

Scopus ID

2-s2.0-77956195360 (requires institutional sign-in at Scopus site)   14 Citations

Abstract

The author reports the details in 2 cases of infants with familial cerebral cavernomatosis who presented in dire condition from hemorrhagic posterior fossa cavernous malformations. In Case 1, a 4-month-old boy presented with opisthotonos, gaze palsy, and lethargy. Magnetic resonance imaging revealed a multilobulated cavernous malformation in the fourth ventricle with evidence of bleeding and obstructive hydrocephalus. In Case 2, a 7-month-old girl presented with lethargy, followed by rapid neurological decline. Imaging demonstrated a large lesion involving both the brainstem and cerebellum, with obstructive hydrocephalus. Both patients required immediate surgical intervention, and external ventricular drainage and posterior fossa craniotomies were performed. Both patients made excellent recoveries. These cases suggest that infants in families with suspected or confirmed familial cerebral cavernomatosis should be screened at an early age.

Author List

Lew SM

Author

Sean Lew MD Chief, Professor in the Neurosurgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Brain Stem Neoplasms
Cerebral Hemorrhage
Cranial Fossa, Posterior
Early Detection of Cancer
Female
Genetic Testing
Hemangioma, Cavernous, Central Nervous System
Humans
Hydrocephalus
Infant
Magnetic Resonance Imaging
Male
Tomography, X-Ray Computed