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Indian Pediatr 2015;52: 265 |
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Localised Gigantism
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* Narendra Bagri, Sriparna
Basu and Ashok Kumar
Department of Pediatrics, Institute of Medical Sciences,BHU, Varanasi, UP, India.
Email: [email protected]
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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).
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Fig. 1. Localized
asymmetric hypertrophy of digits.
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Fig. 2 Hypertrophy of bones
and soft tissues of involved digits.
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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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