Journal of Neurosciences in Rural Practice · 2014 · 11 citations · 0 references
MedicineNeurosurgeryPediatricsCongenital NeurosurgeryCraniofacial SurgeryPediatric Head SurgeryChiari MalformationCongenital Cranial AnomaliesCongenital Craniofacial AnomaliesCongenital Skull DefectsCraniofacial AnomaliesCraniosynostosis SurgeryChiari Malformation Type IClinical SymptomsCongenital Cranial MalformationsCranial Vault DefectsPediatric Chiari MalformationPosterior Fossa DecompressionSyndromic Craniosynostosis ManagementSyndromic Craniosynostosis Surgery
Chiari malformation Type I (CM-I) related to syndromic craniosynostosis in pediatric patients has been well-studied. The surgical management consists of cranial vault remodeling with or without posterior fossa decompression. There were also cases, in whom CM-I was diagnosed prior to the craniosynostosis in early childhood. We present a 16-year-old boy who admitted with symptoms related to CM-I. With careful examination and further genetic investigations, a diagnosis of Crouzon syndrome was made, of which the patient and his family was unaware before. The patient underwent surgery for posterior fossa decompression and followed-up for Crouzon's syndrome. To our knowledge, this is the only case report indicating a late adolescent diagnosis of Crouzon syndrome through clinical symptoms of an associated CM-I.