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• W4288046997 endingPage "4062" @default.
• W4288046997 startingPage "4054" @default.
• W4288046997 abstract "Macrodactyly is a very rare congenital difference that affects hands and feet with significant developmental and psychological implications. Macrodactyly is attributed to a somatic mutation in PIK3CA, a component of the mTOR pathway-related overgrowth disorders. Other medical conditions have been associated with macrodactyly (e.g., neurofibromatosis and Proteus syndrome). A thorough investigation of the presence of these conditions should be undertaken by the multidisciplinary team. The aim of this study is to summarize the main clinical characteristics and associated conditions, with an emphasis on diagnosis and surgical treatment options.We present several clinical cases after a retrospective chart review of macrodactyly cases and a comprehensive literature review.The indications for surgery include peripheral compressive neuropathies (e.g., carpal tunnel syndrome), grotesque enlargement interfering with function, psychosocial distress due to the deformity, and macrodystrophic lipomatosis with proximal upper limb involvement. The main surgical treatment options are categorized as follows: digit reduction (e.g., soft tissue debulking, skeletal shortening/ terminalization, Barsky procedure, and Tsuge technique), limitation of growth (digital nerve stripping and epiphysiodesis), and correction of deviation (wedge or angulation osteotomy, arthrodesis, Millesi procedure for thumb macrodactyly correction, toe-to-hand transfer, ray resection, and combination of bony reduction and soft tissue debulking).Macrodactyly correction requires surgical experience and an individualized approach. Treatment is primarily surgical; however, efforts are being made to delineate the root cause of macrodactyly and provide nonoperative management." @default.
• W4288046997 created "2022-07-27" @default.
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• W4288046997 date "2022-11-01" @default.
• W4288046997 modified "2023-10-14" @default.
• W4288046997 title "Congenital difference of the hand and foot: Pediatric macrodactyly" @default.
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• W4288046997 doi "https://doi.org/10.1016/j.bjps.2022.06.059" @default.
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