Affiliation:
1. Faculty of Medicine and Pharmacy, University Mohammed Premier, Oujda, Morocco
2. Department of Neurosurgery, Mohammed VI University Hospital Center, Oujda, Morocco
Abstract
Abstract
Background:
Hydranencephaly is a rare congenital disorder of the central nervous system, in which the cerebrum is replaced by a membranous sac of cerebrospinal fluid.
Clinical Description:
A 2-month-old baby girl presented with abnormal body movements. She was born to a mother with gestational diabetes and had been diagnosed with hydrocephalus antenatally. The baby had features of facial dysmorphism such as bilateral megalocornea, bilateral cleft lip, and cleft palate. A magnetic resonance imaging (MRI) brain revealed the absence of cerebral hemispheres with remnants of parenchyma in the orbitofrontal area that had been replaced by cerebrospinal fluid, diagnostic of hydranencephaly. Karyotyping and echocardiography findings were normal.
Management and Outcome:
The prognosis was explained to parents, who opted for no therapeutic intervention. The thus patient kept on supportive care and she succumbed at the age of 6 months.
Conclusion:
Our case creates awareness regarding a possible association between gestational diabetes and hydranencephaly. Furthermore, the hydranencephaly in this case was unique due to its association with bilateral megalocornea and cleft lip. The case reiterates the fact that a hydrocephalus diagnosed antenatally may actually be a hydranencephaly, which is confirmed in MRI brain imaging postnatally.