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Hydranencephaly in Malawian children
1Neurosurgery Unit, Department of Surgery, College of Medicine, University of Malawi, Private Bag 360, Chichiri, Blantyre, Malawi.
Insights
Not all large heads in children indicate hydrocephalus. Hydranencephaly, a condition where the brain is replaced by fluid, can be mistaken for hydrocephalus, leading to unnecessary surgeries.
Area of Science:
- Pediatric Neurology
- Neuroimaging
- Neurosurgery
Background:
- Abnormal head enlargement in children is a common clinical presentation.
- Hydrocephalus, characterized by ventricular dilatation, is a frequent diagnosis.
- Distinguishing hydrocephalus from other causes of macrocephaly is crucial for appropriate management.
Purpose of the Study:
- To differentiate hydranencephaly from hydrocephalus in pediatric patients with enlarged heads.
- To highlight the importance of accurate diagnosis to prevent ineffective surgical interventions.
Main Methods:
- Prospective study of children presenting with suspected hydrocephalus at Queen Elizabeth Central Hospital, Blantyre.
- Diagnostic investigations included cranial ultrasonography, air ventriculography, and CT scans.
- Cerebral spinal fluid (CSF) aspiration was performed to assess fluid characteristics prior to considering shunting.
Main Results:
- Hydranencephaly presented as a large fluid-filled cavity replacing the brain, unlike hydrocephalus with ventricular dilatation.
- Ventriculo-peritoneal shunting was effective for hydrocephalus but not for hydranencephaly.
- Fifteen cases of hydranencephaly were identified during the study period.
Conclusions:
- Hydranencephaly is a significant differential diagnosis for pediatric macrocephaly, often confused with hydrocephalus.
- Accurate recognition of hydranencephaly is essential to avoid unnecessary and non-beneficial surgical procedures.
- This distinction is critical for guiding appropriate clinical management and improving patient outcomes.
Objective:
To show that not all big heads in children are due to hydrocephalus.
Setting:
The Queen Elizabeth Central Hospital (QECH), Blantyre, the teaching hospital of Malawi College of Medicine. It is the biggest and leading hospital in Malawi and the major referral centre for the whole country.
Design:
A prospective study was made of all children who presented at QECH, Blantyre with the diagnosis of hydrocephalus during the study period.
Subjects:
Fifteen cases of hydranencephaly were encountered.
Intervention:
All cases of abnormally large heads were investigated with cranial ultrasonography, a few children had air ventriculography and CT scan of the brain. All patients had ventricular aspiration of cerebral spinal fluid (CSF) to verify the colour and condition of the fluid before performing ventriculo-peritoneal shunting for which our locally made Malawi unishunt was used.
Results:
In hydranencephaly, the brain was found replaced by large fluid-filled cavity in contrast to hydrocephalus where brain was present and ventricular dilatation was encountered. Ventriculo-peritoneal shunting benefited children with hydrocephalus but not those with hydranencephaly.
Conclusion:
Hydranencephaly is one of the causes of abnormal head enlargement in children often confused with hydrocephalus. It is important to recognise hydranencephaly to avoid unnecessary operation which does not help the patient.
