gms | German Medical Science

Objectives/Interrogation: To introduce the hand deformity of congenital upper limb muscular hypertrophy and the treatment strategy.

Methods: From May 1992 to October 2012, 13 cases of congenital upper limb muscular hypertrophy were diagnosed by clinical characteristics, operative exploration and pathology result. The primary or other secondary macromelia were excluded. There were 8 males and 5 females with an average age of 6 (2 to 20 years). The presentation of the hand deformity, intraoperative exploration, the surgical treatment and outcomes were reviewed.

Results and Conclusions: The deformity of hand mainly includes excessive spreading in metacarpophalangeal(MP) joint, hyper-abduction of the thumb to radial and palmar side and hypertrophic web spaces, ulnar deviation and flexion deformity at the MP joints and cross finger deformity. Intraoperative findings include multiple aberrant intrinsic and extrinsic muscles with abnormal origin, insertion and orientation, and increased volume.The main surgical choice was proper resection of the aberrant muscles, abnormal musculature and skin to reduce volume of the thenar and hypothenar eminence, correction of hyper-abduction on thumb and little finger and correction ulnar deviation and flexion of the MP joint. Other treatment strategies included metacarpal osteotomy to correct cross-finger deformity, surgical release skin and soft-tissue to correct MP joint flexion, relocation and stabilization the central band to help correcting ulnar deviation and flexion of the MP joint. All the patients got different degrees of cosmetic and functional improvement with an average follow-up of 2.5 years.

The hand deformity of congenital upper limb muscular hypertrophy was mainly attributed to congenital aberrant intrinsic and extrinsic muscles with abnormal insertions and orientation,and increased volume. Surgical corrections can improve the appearance and function of the hand to different degrees.