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Hydrocephalic holoprosencephaly: An oxymoron? Insights into etiology and management
Anuj Kumar Tripathi1, Deepak Agrawal, Gopal Sedain
1Department of Neurosurgery, Neurosciences and Gamma-knife Center, All India Institute of Medical Sciences, New Delhi-110 029, India.
Abstract:
Holoprosencephaly is usually associated with microcephaly, although macrocephaly is not uncommonly seen. However, the cause of hydrocephalus in holoprosencephaly remains ill-defined. Here, the authors report a case of CSF ascites following ventriculoperitoneal shunt placement in a five month-old child with alobar holoprosencephaly, and hypothesize that the excessive CSF production which occurs in this condition may be responsible for the formation of CSF ascites. Further research is required to assess whether the gene responsible for holoprosencephaly is also responsible for upregulating CSF production in patients with concomitant hydrocephalus.
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