Crouzon Syndrome with Hydrocephalus

Abstract

Background: Hydrocephalous in the presence of primary craniosynostosis is a unique condition with respect to pathogenesis, clinical significance, and morphological appearance. It is rarely observed in nonsyndromic craniosynostosis, and in these cases usually attributable to coincidental disorders. Conversely, it is a common feature of syndromic craniosynostosis. The management of CS requires a multidisciplinary approach, and early diagnosis is vital in order to avoid complications such as blindness and raised intracranial pressure.Case presentation: A 7-years-old boy presented to the Neurosurgery OPD complaining of facial disfigurement, headache, vomiting and one episode of fit. X-Ray Skull films showed typical features of impressions of sulci and gyri demonstrating raised ICP due to fused sutures. Computed tomography scan showed hydrocephalus and prominent markings on the skull’s inner table with shallow orbits. Based on all these findings the patient was diagnosed to have the Crouzon syndrome.Conclusion: In true hydrocephalus ventriculo-peritoneal shunting is currently the single promising mode of treatment in case of craniosynostosis case.

Keywords

• crouzon syndrome
• hydrocephalous
• craniosynostosis