LOCALIZED GIGANTISM: A CLINICAL STUDY OF 20 CASES

Abstract

BACKGROUND: Localized gigantism is uncommon condition encountered in clinical practice. Enlargement & thickening of a limb or digit may be due to haemangioma,

lymphangioma, lipoma,or tumour mass. In these situations, only a defined element (vessels,

subcutaneous fat, bone etc) is affected. Congenital localized gigantism strictly speaking, refers to the rare malformation characterised by enlargement of all structures of a limb,digit or its phalanges, subcutaneous fat, nerve, vessel, skin, nail etc. Localized gigantism has also been described under many names such as macrodactyly,megalodactyly, dactylomegaly, macrosomia,macrodystrophia lipomatosa(MDL).

MATERIAL AND METHODS: The Study Of 20 cases of Localized form of gigantism in different congenital conditions was carried out in Mmcri Mysore, Kims Hubli and Esic  medical college Kalaburagi during the last 30 years. Here patients were mainly seeking medical advice for cosmetic reasons & for disability certificates. We took clinical photographs , plain radiographs of affected limb ,USG(Gray scale & Dopple) & biopsy of tissue.

RESULTS: In all the above cases there was abundance of fibrofatty tissue along with dense fibrous tissue, in few cases especially upper limb ,nerves were  enlarged & thickened. It was confined to Megafoot- 5 Cases since childhood, stretched shiny scaly dry skin ,double the girth of opposite limb, Syndactyly(2 & 4^th) Toe ,Rudimentary(1^st & 5^th Toe Variation),Unilateral Giant Lower Limb -5 cases, Giant Upper Limb- 3 Cases ,Radial Mega Hand Mega Thumb-4 cases ,Macrodactaly Of Index Finger-3cases. All were compatible with normal life.

CONCLUSION: We conclude that it is a rare type of disease. No genetic involvement, any theories cannot be formed or dimissed.  Few were associated with Neurofibromatosis. HPE: Excessive Proliferation & Accumulation of fat is the basic lesion. Surgical correction is the treatment of choice than observation, taking into account possible future complications in the absence of surgery and the beneficial outcomes of surgical procedures.