Exceptionally Extensive Trevor’s Disease Involving All Four Radial Carpals Presenting With Gross Wrist Deformity

TL;DR

A rare case of a developmental wrist disorder in a child is described, involving multiple bones and leading to deformity, with successful surgical treatment.

Contribution

This paper reports a rare case of extensive carpal involvement in Trevor’s disease with multilobulated lesions and successful surgical management.

Findings

A four-year-old girl presented with wrist deformity caused by multilobulated lesions involving four radial carpals.

Surgical excision and deformity correction led to functional recovery without recurrence after six months.

Histopathology confirmed the diagnosis of dysplasia epiphysealis hemimelica (DEH).

Abstract

Trevor’s disease, also known as dysplasia epiphysealis hemimelica (DEH), is an uncommon developmental disorder in which the epiphyseal cartilage grows asymmetrically, typically affecting the lower limbs. Carpal involvement is exceptionally uncommon. We report a four-year-old girl presenting with a progressively enlarging, firm swelling over the volar-radial aspect of the wrist, causing deformity and restricted motion. Imaging revealed multilobulated osteocartilaginous lesions involving the scaphoid, lunate, trapezium, and trapezoid, as well as an additional lesion within the abductor pollicis longus tendon sheath. Histopathology confirmed the diagnosis of DEH. Surgical excision with deformity correction resulted in good functional recovery without recurrence at six months. This rare case of extensive, multilobulated carpal DEH underscores the importance of recognizing its epiphyseal origin to differentiate it from osteochondroma and guide appropriate management.

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