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Background/Purpose: Macrodactyly of the hand is a rare group of congenital disorders that present as overgrowth of one or more digits.  The etiology of non-syndromic hand macrodactyly is unknown.  Patients have variable growth patterns in the affected digits, some degree of nerve involvement, and impairment of extremity function.  Most patients are treated by serial debulking procedures or amputation, but outcome studies are not available.  In this study, we characterized the surgical treatment and outcomes of a large series of pediatric patients with hand macrodactyly.

Methods: We queried our institution’s pathology database for hand macrodactyly samples submitted between 1996 and 2011.  We excluded patients with diagnoses of other known overgrowth syndromes, neurofibromatosis, vascular anomalies, or otherwise uncharacterized syndromic presentations of macrodactyly.  We obtained the following data from medical records: demographics, clinical presentation, procedure and treatment details, complications, and follow-up if recorded. 

Results: We identified 22 patients with hand macrodactyly.  Average age at presentation was 5.3 years (range 0.12 – 21.8). Twenty-one patients had unilateral involvement with the right hand being the most commonly affected side (n=14).  Thirteen patients had at least 2 affected digits.   Indications for surgery included substantial overgrowth, impaired function, and/or pain. Patients underwent an average of 4.2 corrective debulking procedures (range 1 – 16). Surgical treatment also included: osteotomy (n=13), amputation (n=5), index to long metacarpal transfer (n=2), and toe transfer (n=1).  One patient had an associated syndactyly between affected digits that was corrected during debulking surgery.  Patients experienced minimal complications. The most frequently reported complication was hypertrophic scarring (n=4). There were no records of major or minor infections or other complications over a mean follow-up period of 2.9 years (range 0 – 9.7). 

Conclusions: Congenital hand macrodactyly ranges in severity and the rate of growth. Macrodactyly can be treated effectively with staged debulking procedures.  Attempts should be made to salvage affected digits when possible. Although multiple procedures may be required, complication rates are low.  Patients should be followed over the course of growth and development to assess the need for subsequent procedures to improve appearance and function.