Congenital malrotation of atlas with unilateral hypertrophy of atlanto-ocipital joint – A rare anomaly of cranio-vertebral junction and its management: A case report.

Congenital malrotation of atlas with unilateral hypertrophy of atlanto-ocipital joint – A rare anomaly of cranio-vertebral junction and its management: A case report.

World Neurosurg. 2015 Dec 24;

Authors: Nath PC, Mishra SS, Deo RC, Mahanta I

Abstract
Congenital anomalies are commonly encountered in cranio-vertebral junction (CVJ) due to its unique embryological development. CVJ usually comprises occiput, atlas and axis. However malrotation of atlas (C1) in between occiput (C0) and axis (C2) with Chiari -1 malformation as well as hypertrophy of unilateral atlanto-ocipital joint and lateral mass manifesting features of high cervical myelopathy is hardly reported anomaly. Here we report a case of successfully managed 22 years old female who presented to us with high cervical compressive myelopathy. Imaging revealed rotation of C1 vertebra around 20 degrees towards left side with right atlanto-ocipital joint dislocation and hypertrophy. It also revealed hypertrophy of right lateral mass of C1, Chiari -1 malformation and right atlanto axial dislocation exposing right C2 superior facet. She underwent right extreme far lateral approach comprising right C1 posterior arch excision and medialization of right vertebral artery with excision of hypertrophied atlanto -occipital joint extradurally and posteromedial excision of superior articular facet of C1. The patient's compressive myelopathy features improved post operatively. We report the management of this rare congenital anomaly with review of literatures.

PMID: 26724634 [PubMed – as supplied by publisher]

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