Surgical Management of Macrodystrophia Lipomatosa, a rare case report of the left hand middle finger macrodactyly

Abstract

IntroductionMacrodystrophia Lipomatosa is a rare congenital non-hereditary disorder, characterized by the presence of gigantism of a small part of the limb or the whole limb due to an overgrowth and disproportionate growth of fibroadipose tissue, causing macrodactyly.Case presentationOne case of the 14-year-old girl presented with an enlarged middle finger of her left hand since birth, discomfort during the last 6 months, and resistance to flex, was underwent surgery by performing debulking procedure and a yellow cord-like mass was obtained from the digital nerve covered and enlarged by fibrofatty tissue to the palm area, could be cut off completely. Imaging and histopathological examination revealed to macrodystrophia lipomatosa. We follow up the patient for the finger's range of motion, the neurological disturbance and re-enlargement of the tumor.DiscussionSince there were compression of the nerves, functional impairment due to enlarge fingers and cosmesis problems, the surgery was indicated. Types of surgery may include debulking of soft tissue, especially adipose tissue. The mass size was decrease, motor function and movement of the middle finger were normal post operatively, but sensory deficits persisted according to the distribution of the digital nerves. Three months and six years after surgery, no enlargement of the middle finger, normal motor function, normal finger movements but sensory deficit still persisted according to the distribution of the digital nerves.ConclusionSurgical management in macrodactyly due to macrodystrophia lipomatosa of the finger give the satisfactory result.