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.

[proteus syndrome]

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 .