Radiological Diagnosis of Hydrancephaly in a Specialist Hospital in Port Harcourt, A Case Report

Chidinma Wekhe, Enighe Ugboma, Chukwuemeka Agu

Abstract


BACKGROUND

Hydrancephaly a rare disorder, is an extreme form of porencephaly occurring in less than 1 in 10,000 live births and about 0.2% of infantile autopsies. It may present with delayed milestones in children and need a high index of suspicion to diagnose. The aim of this case report is to improve awareness on the use of imaging in the evaluation of children with poor development and the diagnosis of intracranial abnormalities.

METHODS

We describe a case of hydrancephaly in the computed tomography (CT) of an infant male with delayed developmental milestone referred to us for CT brain in Braithwaithe Memorial Specialist Hospital Port Harcourt and also present a review of the literature.

RESULTS

Hydrancephalyis characterized by varying degrees of absence of cerebral hemispheres and basal ganglia. The falx may or may not be absent depending on the severity. However the thalami, pons, cerebral peduncles and cerebellum are usually present. The remaining cranial cavity is thus filled with cerebrospinal fluid. The condition is usually and typically congenital, due to in utero vascular insults to the brain. Presentation varies in different patients but with the increasing use of different advanced imaging modalities more cases are being diagnosed.

CONCLUSION

Brain imaging should be recommended by paediatricians and other doctors in the investigation of children with poor development as this may lead to the diagnosis of rare and unsuspected intracranial abnormalities.


Keywords


Hydrancephaly; Congenital Abnormality; Port Harcourt; Computed axial tomography

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ISSN: 1597-4292

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