Cloverleaf skull syndrome with occipital bone cristae

Abstract

Two cases of cloverleaf skull syndrome are reported. The patients were born with trilobed deformity of the skull associated with synostosis of the lambdoidal, coronal, and sagittal sutures in Case 1 and of the lambdoidal suture in Case 2. Both patients had hydrocephalus, low-set ears, and ocular hypertelorism. Ventriculoperitoneal shunting had been performed for hydrocephalus in both cases in early infancy. Thereafter, the skull anomaly caused mental and physical retardation and elevated intracranial pressure. In both cases, surgery was successful in terms of cosmetic and neurological improvement. Both cases involved prominent midline crista formation of the skull over the posterior fossa, which steadily progressed during the first year of life and produced hydrocephalus, apparently by compressing or obstructing the aqueduct and/or the ostia of the fourth ventricle. Twenty-four cases of surgery for cloverleaf skull syndrome have been reported to date. Only Partington's types II and III were treated surgically, and patients with type III had more favorable outcomes than those with type II. However, these reports provide no clear-cut data that surgery significantly influences the outcome. The experience with the two cases reported here suggests the benefits of early shunting followed later by radical surgery. © 1989, The Japan Neurosurgical Society. All rights reserved.