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CASE REPORT
Year : 2012  |  Volume : 1  |  Issue : 3  |  Page : 152-154

A severe and rapidly progressive case of proteus syndrome in a neonate who presented with unilateral hydrocephalus apart from other typical features of the proteus syndrome


Department of Pediatrics, Katihar Medical College, Katihar, Bihar, India

Correspondence Address:
Rakesh Kumar
Department of Pediatrics, Katihar Medical College, Katihar, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4847.101702

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Proteus syndrome is a rare hamartomatous disorder affecting multiple tissues and manifesting itself in a variety of ways. The understanding of the complete spectrum of clinical features, the natural clinical course of the disease and the proper management of such a rare but highly variable syndrome depend heavily on experiences gathered by previously reported cases. We present an unusually severely affected and rapidly progressive case of proteus syndrome in a neonate who presented with craniofacial hemihypertrophy, subcutaneous masses, capillary hemangioma, varicose veins, epidermal nevi and macrodactyly. The cranial ultrasonogram revealed unilateral hydrocephalus with partial obstruction of the foramen of monro.


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