Online ISSN: 2515-8260

A RARE CASE RECURRENT ALOBARHOLOPROSENCEPHALY

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DR.GEETHAKRISHNAMOORTHY, DR.YAGNASWETHA.U

Abstract

Holoprosencephaly (HPE), a congenital induction disorder, occurs due to failed segmentation of neural tube and subsequent incomplete separation of the prosencephalon. Because of the defect in the ventral induction, HPE is also associated with multiple facial abnormalities. Mortality correlates with the severity of brain malformation and facial phenotype. Frequent causes of death include respiratory infections, dehydration due to uncontrolled diabetes insipidus, intractable seizures, and brainstem malfunction. In AH, there is a failure of cleavage of the prosencephalon sagittally into cerebral hemispheres, transversely into telencephalon and diencephalon, and horizontally into olfactory tracts and bulbs. As a result, a small monoventricular cerebrum lacks interhemispheric division. The thalami and the corpora striata are fused across the midline. Olfactorytracts and bulbs, corpus callosum is absent. Alobar holoprosencephaly (AH) usually has various facial dysmorphisms which have higher incidence as 80% in such patients and may be nondiagnostic in 20% of the patients.There are very few reported cases of AH with normal facies in the literature. We are reporting a female with reccurent pregnancies with Alobar holoprosencephaly.

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