Orthotic management with a customized humeral brace for Gorham-Stout disease of the humerus: A case report

@#Gorham-Stout disease is characterized by massive osteolysis or "vanishing bone" on radiograph. Due to its rarity, no standard Physical Medicine and Rehabilitation (PM&R) management has been published. With this comes the dilemma of managing another case of vanishing right humerus in a 13 year-old male, right handed student, with normal growth and development. To date, this could be the third documented case in the Philippines, but the first with humeral involvement, and the first to manage using a customized humeral brace. The absence of the right humerus affects the bimanual overhead and tabletop activities of the patient, for which a custom-made humeral orthosis was provided to manage the limited activities. There were improvements in activities such as writing, card turning, stacking, and lifting objects of variable weights, as well as with hand dexterity as evidenced by the standardized hand function tests done prior and post brace fitting. Being a rare bone disease with no standard management and unpredictable course, cases are managed symptomatically. For this case of an absent humerus significantly affecting upper extremity function, orthotic management is one aspect that could be recommended to achieve positive functional outcomes.

Doença de Gorham da escápula e clavícula: relato de caso e evolução de dois anos de uma afecção rara / Gorham's disease of scapula and clavicle: case report and two-year follow-up of a rare disorder

A doença de Gorham é uma rara desordem osteolítica, de etiologia controversa, que pode afetar qualquer osso. O substrato histopatológico é a substituição óssea por uma formação expansiva de natureza vascular agressiva, não neoplásica. Descrevemos os achados radiográficos, tomográficos e de ressonância magnética de um caso desta afecção acometendo a escápula esquerda e, dois anos depois, a clavícula ipsilateral.

Gorham's disease is a rare osteolytic disorder of still controversial etiology that may affect any bone. The histopathological substrate for such a condition is the replacement of normal bone by aggressive non-neoplastic expansile vascular tissue. The authors describe radiographic, computed tomography and magnetic resonance imaging findings in a case of this entity initially affecting the left scapula and, two years later, the ipsilateral clavicle.

Vanishing bone disease (Gorham's disease) - A rare occurrence of unknown etiology.

A 20-year-old male patient presented with painful swelling around left elbow joint. Radiographic examination revealed osteolytic lesion with pathological fracture of lower end of humerus and upper radius. Upper end of ulna was completely absent along with bony erosion. Histopathology of the bony tissue revealed hemangioma-like lesion composed of vascular channels lined by benign endothelium replacing bone. The diagnosis of Gorham's massive osteolysis was made. Gorham's disease is a benign self-limiting condition affecting any age, may involve any part of the skeleton and is characterized by replacement of bone by hemangiomatous tissue resulting in formation of lesions exhibiting massive osteolysis, which may be to the extent of disappearance of the affected bone in radiograph. This nonhereditary case was not associated with nephropathy, which is often a coexistent condition. The case is being reported for its rarity.

The Preliminary Study of Trace Elements in the Hair of Patient With Massive Osteolysis / 环境与健康杂志

Objective To have knowledge of the exact cause of massive osteolysis. Methods Hair of patients from Xinjiang province was collected and 14 trace elements were determined by inductively coupled plasma mass spectrometryICP-MS. Results Trace elements imbalance in the body of patients was disturbed. Chromium and zinc which are benefit to the growth of the bones were only 0.5 ?g/g and 40 ?g/g respectively that were much lower compared with the healthy persons cadmium was much higher than the limit level in healthy person. Moreover the quantity and ratio of potassium and sodium in the patients were obvious abnormal. Conclusion According to the result of the present paper may be the environmental and dietary factors play an important role in pathogenesis of this disease.

Gorham's Syndrom: A Case Report / 대한정형외과학회잡지

Gorham's syndrome (Gorham's disease, Massive osteolysis, Disappearing bone disease, phantom bone) is a rare disorder characterized by histologically benign proliferation of thin-walled vascular channels originating in bone associated with extensive lysis of regional bone matrix. It starts in bone, but it may secondarily involve soft tissues and adjacent bones. Although the osteolysis usually arrests spontaneously, its biologic behavior is unpredictable. The etiolgy is still in dispute and the treatment presents many problems. The author's case was a 20-year old girl, who had pathologic fracture in neck and supracondylar area of left femur with osteolysis of left pelvis, femur, and proximal fibula and tibia. She also had lymphangioma involving bones and adjacent soft tissues. To our knowledge, there is no report on Gorham's syndrome in Korea. The authors report a case of Gorham's syndrome with review of literatures.