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Indian Pediatr 2015;52: 265

Localised Gigantism


*Narendra Bagri, Sriparna Basu and Ashok Kumar

Department of Pediatrics, Institute of Medical Sciences,BHU, Varanasi, UP, India.
Email: drnarendrabagri@yahoo.co.in



A 2-year-old girl presented with enlargement of bilateral index and middle fingers since birth (Fig. 1). Rest of the systemic examination was unremarkable. Roentenogram of both hands revealed bony and soft tissue hypertrophy of the involved digits (Fig. 2).

Fig. 1. Localized asymmetric hypertrophy of digits.

Fig. 2 Hypertrophy of bones and soft tissues of involved digits.

Localized gigantism is a rare non-hereditary congenital malformation presenting with localized, usually asymmetric gigantism involving single or multiple digits, entire limb, or hemihypertrophy of the whole body. It results from hypertrophy of bones and soft tissues of the affected part, and leads to cosmetic and functional impairment. The growth of the involved part continues till puberty. The exact etiopathogenesis is unknown; a novel mutation confined to the affected tissue (mosaicism) has been reported. Management includes multistage reconstructive surgery. Differential diagnosis includes variants of neurocutaneous syndromes, Maffucci syndrome, Proteus syndrome, fibrolipomatous hamartoma and lymphangiomatosis.


  

 

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