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- Grace Il Tan, David Cy Low, Lee Ping Ng, Wan Tew Seow, and Sharon Yy Low.
- National Neuroscience Institute, Department of Neurosurgery, National Neuroscience Institute, Singapore.
- World Neurosurg. 2018 Oct 1; 118: 301-303.
BackgroundChiari III malformation is an extremely rare congenital anomaly. At present, its primary and associated pathognomonic factors remain poorly understood. The authors report a case of a male neonate born with an occipital encephalocoele with herniation of posterior fossa contents associated with bilateral postaxial polydactyly.Case DescriptionThe patient is a dichorionic, diamniotic twin conceived via assisted reproductive methods; his twin sister has no congenital anomaly. Neurosurgical management included successful repair of the encephalocoele and subsequent cerebrospinal fluid diversion via a ventriculoperitoneal shunt.ConclusionsOwing to the uniqueness of this patient's presentation, the concurrent diagnoses of Chiari III malformation and polydactyly are discussed in concordance with updated literature.Copyright © 2018 Elsevier Inc. All rights reserved.
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