Congenital Anomalies of the Upper Extremity | 2021

Madelung’s Deformity

 
 
 

Abstract


Madelung’s deformity is an uncommon congenital wrist condition characterized by premature closure of the volar-ulnar aspect of the distal radius physis, volar carpal subluxation, and distal ulna prominence. It is classified as a malformation of the radio-ulnar axis involving the entire upper limb, according to the Oberg-Manske-Tonkin Classification. It accounts for less than 2% of congenital upper extremity differences. Madelung’s deformity is most commonly idiopathic, but a Madelung-like deformity may result from trauma, infection, multiple hereditary exostoses (MHE), and Ollier’s disease. Additionally, it is associated with skeletal dysplasias involving mutations of the short stature homeobox (SHOX) gene. Madelung’s deformity predominantly affects females and becomes clinically apparent during adolescence. Affected individuals may present with wrist pain, restricted range of motion of the wrist and forearm, decreased grip strength, and function difficulties, as well as aesthetic concerns. Several surgical options have been described for children with Madelung’s deformity, depending on their age and degree of deformity, and include physiolysis, soft tissue release, and osteotomies, with promising outcomes.

Volume None
Pages None
DOI 10.1007/978-3-030-64159-7_25
Language English
Journal Congenital Anomalies of the Upper Extremity

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