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Year : 2008  |  Volume : 3  |  Issue : 2  |  Page : 169-171

Chiari III malformation with hypertelorism and microcephaly in a neonate: Case report and a review of the literature

Department of Neurosurgery, Sanjay Gandhi Postgraduate institute of Medical Sciences, Lucknow - 226 014, India

Correspondence Address:
Sanjay Behari
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226014
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.43652

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Chiari III malformation is an extremely rare anomaly characterized by low occipital and high cervical encephalocele with herniation of posterior fossa contents, that is, the cerebellum and/or the brainstem, occipital lobe, and fourth ventricle. We report the case of Chairi III malformation in a neonate with associated hypertelorism and microcephaly, discuss the etipopathogenetic and radiological features, and review the pertinent literature. The neonate, a two-day-old child, with Chairi-III malformation had a large low occipital and cervical osseous defect with nearly a nonexistent posterior fossa due to the herniation of the cerebellum and a part of the occipital lobe into the large encephalocele sac. The patient also had associated microcephaly and hypertelorism without hydrocephalus. The existence of significant, viable neural tissue within the encephalocele sac precluded any surgical intervention. In our patient, it is proposed that during early embryogenesis, incomplete distension of the telencephalic and the rhombencephalic ventricles resulted in Chiari III malformation (based upon unifying theory of embryogenesis of McLone and Knepper) with a large encephalocele containing major portions of cerebellum and occipital lobe, and left the chondrocranium without an adequate inductive force of the underlying neural mass. The skull was, therefore, microcephalic and the posterior fossa virtually nonexistent. Hypertelorism also resulted from failure of eyes to completely rotate forward during fetal life owing to the presence of this large posterior encephalocele and the absence of neural cues for the chondrocranial expansion. To the best of the authors' knowledge, the association of Chairi-III malformation with microcephaly and hypertelorism has not been previously reported.


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