Acquired Chiari type I malformation managed by supratentorial cranial enlargement

Abstract

Introduction: Acquired Chiari type-I malformation in hydrocephalic patients who have undergone surgical treatment was initially thought to depend on a craniocephalic disproportion induced by the cerebrospinal (CSF) shunt. However, most of the reports in the literature deal with children with lumbo-peritoneal shunts and emphasize the pathogenic role of the cranio-spinal pressure differential across the foramen magnum brought about by this type of shunt.

Method: In the present report, the authors describe two further cases of symptomatic acquired Chiari type-I malformation observed in two adolescents operated on for correction of pseudotumor cerebri in one (lumbo-peritoneal shunt) and of a suprasellar arachnoid cyst (cysto-ventriculo-peritoneal shunt) in the other.

Results: In both subjects, both the clinical manifestations and the cerebellar tonsillar herniation regressed after supratentorial cranial expansion, without the need for any manipulation of the shunt devices implanted earlier.

Discussion: These results, together with the observation of the concomitant upward and downward herniation of the cerebellum in both patients, indicate that secondary craniocephalic disproportion plays a relevant role in the genesis of acquired Chiari type-I malformation in children bearing extrathecal CSF shunts.