Alobar holoprosencephaly

Case contributed by Dr Hani Salam

Presentation

Neurologically abnormal and enlarged head.

Patient Data

Age: Neonate
Gender: Male
CT

CT brain

Non-contrast CT demonstrates a thin rim of cerebral tissue anteriorly, without midline cleft. There is a large CSF density space posteriorly. There is no evidence of falx cerebri. The cerebellum is malformed with abnormal appearance of the fourth ventricle. Brainstem is visualized.

In addition there are a number of other midline abnormalities:

  1. bony defect in the occipital bone with an occipital meningiocele
  2. cleft lip and palate
  3. under-developed nose and hypoteloresim

Case Discussion

This case demonstrates the typical appearances of alobar holoprosencephaly, incompatible with long term survival. 

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Case information

rID: 8329
Case created: 24th Jan 2010
Last edited: 24th Aug 2017
Inclusion in quiz mode: Included

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