Cloverleaf skull--when should one operate?

Cloverleaf skull or kleeblattschadel usually occurs as a result of premature closure of the cranial sutures, with accompanying hydrocephalus, proptosis, cranial base and midface hypoplasia. The incidence of this abnormality is rare, with less than 130 reported cases in the literature, and is associated with various congenital syndromes. Conventional surgical management advocates shunting prior to definitive craniotomy and bone remodeling. We report an unusual case of Pfeiffer syndrome with associated cloverleaf skull deformity, in which early surgery was vital for decompression of raised intracranial pressure and shunt independence for the first six months. A secondary craniofacial procedure was performed at nine months of age to improve cosmesis. In such cases, good outcome can be achieved if aggressive surgery is combined with good paediatric anaesthesia and intensive care.