Severe Carpal Tunnel Syndrome in a Pediatric Patient With a History of Congenital Adrenal Hyperplasia.

The patient is a 17-year-old right-hand-dominant girl with a history of virilizing congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase enzyme deficiency. Her CAH had been managed with supplemental exogenous steroids, but unfortunately, she had been noncompliant for many years. She subsequently presented with severe progressive numbness and tingling in the bilateral upper extremities that were refractory to conservative management. Electromyography/nerve conduction studies confirmed bilateral carpal tunnel syndrome (CTS) with the right being more severe than the left, and she underwent uncomplicated carpal tunnel releases that relieved her symptoms immediately and completely. Carpal tunnel syndrome secondary to CAH may be associated with the effects of elevated sex hormones within the CTS, leading to inflammation and median nerve entrapment. Moreover, hyperandrogenism is associated with elevated acute phase reactants and inflammatory cytokines, contributing to progressive median neuropathy. To the author's knowledge, there have been no reported cases of severe pediatric CTS with associated hyperandrogenism from CAH.