ICNC Abstracts, ICNC 2018

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Hemimegalencephaly with congenital facial lipoma-A Case Report
Subramanian Velusamy, Subramanian Velusamy, Balaraman Krishnakumar

Last modified: 2018-09-09

Abstract


Introduction:Hemimegalencephaly is a rare congenital disorder of cortical malformation with hamartomatous overgrowth of all or a part of cerebral hemisphere.Association of hemimegalencephaly with congenital facial lipoma is very rare, and one such case is reported here.Case report:A 3 year 7 months old girl born for nonconsanguineous parents, presented with recurrent episodes of seizure since 6 months of age. Seizure semiology is in the form of deviation of eye and head to left side lasting  2 to 5 minutes without loss of consciousness. Her birth and developmental  history were normal. She had  facial mass  on left side and  lower incisor natal teeth.  On examination, head circumference  was 47 cm, and there was no neurocutaneous marker. A  mobile soft mass , 8cm in diameter,with smooth border was present on  left side of her face. Neurological examination was normal.MRI showed a diffuse lipoma in  left cheek and infratemporal fossa  and  hemimegalencephaly  involving left temporoparieto occipital region and cerebellum.Serology for Cytomegalovirus was negative.Discussion:Hemimegalencephaly may occur as isolated anomaly or in association with various neurocutaneous syndromes.Doneti et al  described the first case of hemimegalencephaly and ipsilateral congenital facial lipoma of face associated with cytomegalovirus verified by immunohistology of parotid gland.In our case, serological test for cytomegalovirus was negative.Only a very few cases of hemimegalencephaly associated with congenital facial lipoma were reported in the literature.Conclusion:The case is reported for its rarity.In any child with congenital facial lipoma,imaging of brain should be done to rule out associated malformations like hemimegalencephaly.


Keywords


hemimegalencephaly,congenital facial lipoma

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