Ilyas Bader, Naeemuz Zafar Khan.
Severe form of Frontonasal Dysplasia (FND) with bilateral Anophthalmia - a case report with review of literature.
Pak J Med Sci Jan ;21(1):82-4.

A two days old female neonate of consanguineous marriage with no family history of frontonasal dysplasia presented to us with multiple birth anomalies. On examination she was found to have grotesque midline cranio-facial defects from absence of premaxilla to central nervous system anomalies. The craniofacial anomalies included midline defects with absent prolabium and bilateral cleft lips, bilateral cleft palate, and complete nasal coloboma with absence of columella and upper part of nasal septum. Inferior part of nasal septum and inferior turbinates were visible on gross examination. There was hypertelorism with bilateral anophthalmia. Bony orbits were shallow and had bulging skin covered cystic swellings filled with transilluminant fluid. There were no noticeable ocular movements. Because of inability to do postmortem we were unable to look for detailed structural anomalies of optic discs and eyes. There were bilateral frontal encephaloceles and hydrocephalus. The patient had syndactyly of the first three toes of the left foot. Ultra-sound examination of the brain revealed absence of corpus callosum, single frontal lobe and single ventricle. Because of inadequate diagnostic and treatment facilities and poor prognosis, the child was not offered any form of surgical treatment. Parents were counseled and they decided to take the child home. Baby died after a fortnight.

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