Bilateral Compression of the Median Nerve by Supracondylar Spurs

Mutilating hand syndrome in an infant with familial carpal tunnel syndrome

“…8 Children may present with manifestations of weakness or unusual patterns of hand use in the absence of more typical CTS symptoms such as pain or paresthesia. Well-documented causes of childhood median nerve entrapment at the wrist include storage disorders such as the mucopolysaccharidoses and mucolipidoses 13,27,33,37 ; compression of the median nerve via congenitally aberrant constriction bands, bony spurs, or structural anomalies of muscles, tendons, or bones 2,18,26,31 ; hemophilia (bleeding into a restricted space) 25 ; hemangiomas, hamartomas, tumors, or abscesses 5,12,32 ; traumatic injuries, burns, or strenuous overuse (i.e., wheelchair propulsion) 4,11,16 ; systemic diseases such as disseminated angiomatosis, 28 lipoid proteinosis, 15 and scleroderma 8 ; skeletal dysplasia (spondyloepiphyseal dysplasia tarda) 31 and Weill-Marchesani syndrome 7 ; melorheostosis 3 ; sustained wrist flexion in dystonia or cerebral palsy 1 ; or hypertrophy of the transverse carpal ligament. 10 We report clinical observations and electromyographic (EMG) studies of an infant who presented with hand mutilation as the primary manifestation of CTS, in conjunction with features consistent with congenital insensitivity to pain.…”