Bilateral Scaphotrapezium Coalition with Bilateral Scaphoid Nonunion in a Patient with Klippel–Feil Syndrome: A Case Report

Abstract

The purpose of this case report is to discuss the evaluation and management of scaphoid nonunion in patients with congenital musculoskeletal syndromes. Klippel–Feil syndrome (KFS) is a rare disorder characterized by the presence of congenital synostosis of some or all cervical vertebrae [26] caused by a failure in the normal segmentation of the cervical vertebrae during the third to the eighth week of gestation [25]. The classic clinical triad of KFS consists of a short neck, low posterior hairline, and limited neck range of motion due to cervical synostosis [26]. Abnormalities of the hand have been associated with KFS [2, 4, 8, 15, 25] and are predominantly preaxial [2, 4, 15, 25]. One instance of a postaxial anomaly in a patient with KFS has been reported [2]. A prior identification of capitohamate coalition in a patient with Klippel–Feil syndrome was suggested to be a manifestation of a new clinical syndrome [14]. Additional carpal coalitions have not been reported nor have scaphoid abnormalities secondary to the carpal coalitions been described that are associated with Klippel–Feil syndrome.