'Vanishing carpal bones' in a patient with congenital adrenal hyperplasia - A diagnostic dilemma

Abstract

The vanishing bone disease, also known as Gorhams disease usually involves long bones. Isolated carpal bone involvement is uncommon. A 14 year old male presented with pain in the right wrist joint since 4 months. He was a known case of Congenital Adrenal Hyperplasia on oral steroids for 3 years. X-rays showed deformed arthritic scaphoid, lunate and capitate. A proximal row carpectomy was performed to provide functional wrist mobility to the patient. Histopathological examination was consistent with Gorham's disease. In a patient with systemic disease receiving steroid therapy Gorhams disease poses a diagnostic challenge.

Keywords: Gorham's disease; Vanishing carpal bones; Wrist.

Fig. 1

Radiographs of the wrist showing osteolysis with deformed scaphoid, lunate and triquetrum.

Fig. 2

Radiographs of the opposite wrist showing normal carpal bones.

Fig. 3

Computed Tomography scan of the involved wrist showing similar features.

Fig. 4

Through a dorsal approach to the wrist a proximal row carpectomy was performed and the deformed bones sent for histo-pathological examination.

Fig. 5

Post-operative radiographs of the wrist.

Fig. 6

Histopathological examination of the scaphoid, lunate and triquetrum showing evidence of Gorham-Stout disease.

Fig. 7

Two years follow up x rays showing no evidence of recurrence.

Fig. 8

Full painless ROM at wrist at two years follow up.