The relationship of cloverleaf skull syndrome to hydrocephalus

Abstract

In 1960, Holtermüller and Wiedemann described chondrodystrophic hydrocephalus as Kleeblattschädel syndrome. In the present paper, we review 23 cases of this syndrome associated with hydrocephalus, including 2 cases of our own. Of these 23 cases, 8 involved communicating hydrocephalus and 15 non-communicating hydrocephalus. It has been speculated that the associated hydrocephalus results from basilar impression, compression of infratentorial structures, aqueductal stenosis in non-communicating hydrocephalus, and impairment of venous drainage and cerebral spinal fluid flow at the level of the constrictive cranial ring in communicating hydrocephalus. Our present cases also showed this cranial ring, as demonstrated by skull X-rays and computed tomography scans. Since cloverleaf skull syndrome often has other general anomalies, we consider the hydrocephalus in this syndrome to have causes associated with the osteocartilaginous system.