Absent right iliac bone on Tc99m MDP bone scan in a patient with Gorham's vanishing bone disease.

Gorham's (vanishing bone) disease is an extremely rare condition of the bone. The diagnosis is usually made on the basis of the characteristic history of osteolysis and failure of bone healing in conjunction with the histological findings of marrow fibrosis and increased vascularity. When the disease is established, an X-ray and magnetic resonance imaging show complete loss of affected bone. There are very few reports found in literature on bone scan appearance of the disease. A bone scan of a 24-year-old female patient with known Gorham's disease revealed absence of tracer uptake in the right iliac bone, right sacroiliac joint, and part of the right ischial pubic rami, which matched the radiographic abnormalities. Consequently this disease should be added to the gamut of cold defects seen on bone scan.

INTRODUCTION

Gorham's (vanishing bone) disease is an extremely rare condition of the bone. The diagnosis is usually made on the basis of the characteristic history of osteolysis and failure of bone healing in conjunction with the histological findings of marrow fibrosis and increased vascularity. When the disease is established an X-ray and MRI show complete loss of affected bone. Very few reports are found in literature on bone scan appearance of the disease.

CASE REPORT

A bone scan was performed on a 24-year-old female patient with known Gorham's disease. This was correlated with a pelvic radiograph and corresponding MRI.

Images showed absence of tracer uptake in the right iliac bone, right sacroiliac joint, and part of the right ischial pubic rami, indicating absence of bone in the mentioned regions, which matched the absent bone tissue on the pelvic X-ray [Figure 1 and 2].

Figure 1

Tc99m MDP bone scan (anterior and posterior pelvic views) showing absence of tracer uptake in the right iliac bone, right sacroiliac joint, and part of the right ischial pubic rami (arrows)

Figure 2

Pelvic X-ray shows absent bone tissue in the right iliac bone, right sacroiliac joint, and part of the right ischial pubic rami (arrow).

DISCUSSION

Cold defects on bone scan have been reported in many disease conditions.[1] Gorham's disease should be included in this list. Gorham's disease, which has many other synonyms (Disappearing bone disease, Gorham's syndrome, Gorham-Stout syndrome, Vanishing bone disease, Phantom bone disease, Massive osteolysis, and others) is an extremely rare condition of the bone, with less than 200 cases in literature since the condition was originally reported. Few of the reported cases describe ragiological and scintigraphic findings in this disease condition.[2‐11] Consequently we consider that Gorham's disease should be added to the gamut of cold defects seen on bone scan.