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Indian Pediatr 2010;47: 1051-1052

Frontonasal Encephalomyelocele


TM Ananda Kesavan* and Bejohn Johnson

Kunnath, Department of *Pediatrics and Pediatric Surgery, Government Medical College,
Thrissur, Kerala, India.
Email: [email protected]  
 


A seven year tribal male child presented with a painless swelling on the upper part of the nose since birth which had gradually increased to the present size (Fig.1). He has average intelligence and was otherwise asymptomatic. He was being worshipped as Lord Ganesha because of the typical appearance.

Fig. 1 A large mass of size 10×8×7 arising from the root of the nose.

Examination revealed a large, soft swelling of 10×8×7 cm in size arising from the right side of the root of the nose. Skin over the swelling was hyperpigmented. There was cough impulse but no trans-illumination A provisional diagnosis of frontal meningocele was made. Differential diagnoses included dermoid cyst, hemangioma, neuro-fibromatosis, rhabdomyosarcoma and encephalocele. Dermoid cyst, hemangioma, and neuro-fibromatosis are easily distinguished by their peculiar external appearance. Rhabdomyosarcoma is a rapidly progressing tumor; a relatively benign appearance of this tumor after a long period is against its diagnosis.

CT scan confirmed the diagnosis of right frontonasal encephalomyelocele with partial corpus callosal agenesis. He was referred to a higher center for surgical management.
 

 

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