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Year : 2018  |  Volume : 25  |  Issue : 3  |  Page : 191-194

Delleman syndrome: A case report from West Africa - features and the challenges of management

1 Department of Ophthalmology, University College Hospital, Ibadan, Nigeria
2 Department of Radiology, University College Hospital, Ibadan, Nigeria

Correspondence Address:
Bolutife Olusanya
Department of Ophthalmology, University College Hospital, University of Ibadan, Ibadan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/npmj.npmj_75_18

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Delleman syndrome is a rare congenital disorder. We report an 8-month old female with a history of a fleshy mass covering the left eye since birth. Examination revealed poor vision in the left eye, an upper lid coloboma and an epibulbar dermoid with a large area of alopecia and scalp hypoplasia involving the left frontoparietal region. Transfontanelle ultrasound scan did not reveal any intracranial cyst. Cranial computerised tomography subsequently revealed characteristic cerebral malformations of Delleman syndrome. This report demonstrates clinical and computed tomographic features of a case of Delleman syndrome with emphasis on the usefulness of transfontanelle ultrasonography in assessing for life-threatening intracerebral cysts or hydrocephalus, particularly in resource-limited settings, where neuroimaging is not readily available or affordable.

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