Outcomes in Early Versus Late Presentation of Focal Fibrocartilaginous Dysplasia Affecting the Upper Extremity: A Review of 4 Cases.

Abstract

Focal fibrocartilaginous dysplasia (FFCD) is a rare disorder of the upper and lower extremities. In the distal ulna, a ligamentous tether emerging from the metaphysis crosses the physis and restricts growth, leading to deformity. Lesion excision before radiocapitellar subluxation has been shown to restore growth and allow remodeling. We review the outcomes of 4 patients with FFCD of the distal ulna after the occurrence of radiocapitellar incongruity. This retrospective review examined 4 patients diagnosed with FFCD of the distal ulna from 2007 to 2015. Diagnosis was based on radiographic parameters and pathology when available. The radiographic and functional outcomes are reported. Three males and 1 female patient presented at an average of 37.5 months (range, 22 to 48 mo) with ulnar FFCD and radiocapitellar joint incongruity. The first patient had progressive radiocapitellar dislocation, poor motion, pain, and deformity. The second patient presented for a third opinion after previous surgery secondary to deformity progression and radial head dislocation. These patients required salvage procedures with creation of a 1-bone forearm. Patient 3 had frank dislocation of the radiocapitellar joint, yet maintained functional motion. This family elected for continued observation. Patient 4 had a 50% subluxation of the radiocapitellar joint and underwent tether excision and ulnar lengthening with an external fixator. Both joint congruity and deformity improved with functional forearm and elbow motion. Delayed treatment of ulnar FFCD may require salvage procedures to maximize function and provide pain relief.