Hydranencephaly

Case contributed by Dr. Fernando Frias Vizcarra

Presentation

Term newborn infant with arthrogryposis. Evident macrocephaly with diastasis of the sagittal suture on physical examination.

Patient Data

Age: 4 days
Gender: Male
ct

There are thin layers of rudimentary cerebral cortex towards frontotemporal regions. The brainstem and cerebellum are present and displaced inferiorly, as well as tentorium.

The large accumulation of fluid in the cranial vault is evident.

Case Discussion

The association of hydranencephaly and arthrogryposis is documented in the literature.
The prognosis is poor for function and reserved for short-term life.

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