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Authors present two cases. One of their patient was followed for 30 years. They discuss the pathological characters and diagnostic difficulties of the disease. They have observed that with radical operations of--according to necessity--serial surgical interventions the outcome of patients can be properly controlled against recent pessimistic opinions.
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Gorham's disease (disappearing bone disease, massive osteolysis, idiopathic osteolysis, essential osteolysis, progressive atrophy of bone, spontaneous absorption of bone, phantom bone, hemangiomatosis/lymphangiomatosis of bone, progressive osteolysis) is an extremely rare occurrence. There are fewer than 150 reported cases in the literature. This disorder can be characterized by spontaneous or posttraumatic progressive resorption of bone. The etiology is still very speculative, the prognosis unpredictable, and any effective therapy still unknown. This paper presents a review of the literature and two case reports of suspected Gorham's disease of the bones of the foot.
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The authors report a clinical and radiographic course of a 16 years old caucasian girl with a progressive deformation of her skeleton. The primary lesions occur in knees and wrists. This disease was at first considered as a rheumatoid arthritis, then as a dysplasia epiphysialis multiplex, and now as essential deformans osteolysis (with carpal lysis, shortening of the forearm bones, dislocation of the elbows, disparition of the humeral and femoral heads, contracture of hips and knees, posterior tarsal lysis, and kypho-scoliosis). The clinical (particularly ophtalmologic), biological (including inflammatory, phospho-calcic and nephrologic evaluation with mucopolysaccharidosis urinary excretion) and histological (skin, muscle and bone) check-up were normal. They review the different classification established on lesion topography, on association or not with a nephropathy, finally on an dominant or recessive autosomal inheritance. Then the authors think that their case is similar to the ones of Winchester and Hollister. They discuss the etiopathogenic factors, and do not consider their case as a new mucopolysaccharidosis, but rather as a generalized disease of the collagen of bones.
A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.
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A case of essential carpal osteolysis is reported. The morphological appearance of the hands is described. They showed ulnar deviation; they were short and pudgy, the carpus and metacarpus being almost absent. The fingers were normal, but the left thumb was atrophic. Radiographies of the left hand showed an almost complete disappearance of the carpus and severe irregularities of the ulna and radius. Aetiology, treatment and prognosis are briefly discussed.
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