A CLOVERLEAF SKULL SYNDROME PROBABLY OF BEARE-STEVENSON TYPE ASSOCIATED WITH CHIARI MALFORMATION

A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported, The patient developed congenital hydrocephalus, upper airwa y obstruction and breath holding spells, Ventriculo-peritoneal shunt i mproved the hydrocephalus, but the patient progressively developed apn eic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervica l vertebrae and craniectomy at the occipital bone were performed to re lieve the brain stem compression, Postoperatively, the respiratory sym ptoms improved slightly, but 8 weeks later the patient suddenly died, Literature survey revealed that this is the second case report of CLS associated with Chiari malformation, It was assumed that the respirato ry problems in our patient are ascribable to the Chiari malformation a s well as the upper airway obstruction, The patient here reported seem s to be a case of Beare-Stevenson cutis gyrata syndrome, in view of an association of multiple characteristic dysmorphic features, in additi on to CLS.