Pustaka Ilmiah Universitas Padjadjaran

Abstrak

Crouzon’s Syndrome case report

Background: Crouzon’s syndrome incidence being calculated around 1 in 25.000 births. The mode of transmission is autosomal dominant. The syndrome is characterized by a high incidence of familial cases(44%). There are three major sign, craniosynostosis, shallow orbit that make proptosis and maxillary hypoplasia and some of them also accompamied with hydrocephalus. The management of the abnormal skull and associated hydrocephalus have to be considered by a pediatric neurosurgeon. Case Report: 20 month old male patient with asymmetric of the head with protusion of eye balls that he got since his birth. He was born from P2A2 mather with polyhydramnion and also sepsis with neonatal hyperbilirubinemia. His father had similar character of the face and skull. He got already a closed fontanel(craniosynostosis) and protuded of eye balls and also he got syndactily as other congenital anomaly. Radiological findings from skull x-ray shows impressio digitates and from CT Scan shows closed sutures, shallow orbits and ventricular enlargement. We plan to manage case considerate to the two main objective (1) to correct the cranial dispropotion and (@) to normalize the CSF flow and absorption and decreased a possibility that cause a further brain damage because of increased of ICP. How to solve this problem: (1.) Is it necessary to perform CSF shunting for the hydrocephalus? (2.) What kind of remodelling do we need for this patient? (3.) How long can we postpone the surgery?