OWL: OCOSH Classification/Bone Diseases/Bone Resorption/Osteolysis/Essential Osteolysis

Diffuse Cystic Angiomatosis of bone OMIM

Not Available — 2008-03-22 00:08:27 GMT

Alternative titles; GORHAM-STOUT DISEASE; GSD OSTEOLYSIS, MASSIVE
Reid et al. (1989) described a family in which 12 persons in 4 generations had diffuse cystic angiomatosis of bone. The affected individuals were asymptomatic. Roentgenographically, the lesions occurred throughout the length of long bones and were osteolytic, with a thin sclerotic rim. The cortex of the bone was rarely involved and showed no periosteal reaction. Growth plate closure and remodeling were unaffected. With age, increasing sclerosis occurred, resulting in complete obliteration of the cyst with irregular reactive trabeculations. Five of the affected individuals were female. There were several examples of male-to-male transmission. Cystic angiomatosis of bone includes both hemangiomatosis and lymphangiomatosis. The disorder is different from monocentric massive osteolysis, which goes by the name of Gorham (Gorham and Stout, 1955) and appears to be nonmendelian.

Gorham Vanishing Bone Disease Bibliography

Myles Clough — 2003-02-11 00:08:27 GMT

PubMed search using the terms Essential Osteolysis AND Gorhams. There are some other types of Essential Osteolysis so this search only pulls out those cases specifically referred to as Gorhams. If you delete the "AND Gorham" part of the search string you will get a more comprehensive search

Gorham's Vanishing Bone Disease ORCID

Myles Clough — 2008-03-30 00:08:27 GMT

Attempt to collect cases of this condition. Orthopaedic Rare Conditions Internet Database (ORCID)
Synonyms (MeSH term Essential Osteolysis)
Gorham's Disease
Disappearing Bone Disease
Gorham's Syndrome
Gorham-Stout Syndrome
Idiopathic Massive Osteolysis
Massive Gorham Osteolysis
Massive Osteolysis
Morbus Gorham-Stout Disease
Progressive Massive Osteolysis
Vanishing Bone Disease
Phantom Bone Disease

Gorhams Disease or Massive Osteolysis

Patel — 2008-03-22 00:08:27 GMT

Gorham�s disease is a rare disorder characterized by proliferation of vascular channels that results in destruction and resorption of osseous matrix. Since the initial description of the disease by Gorham and colleagues (1954) and by Gorham and Stout (1955), fifty years have elapsed but still the precise etiology of Gorham�s disease remains poorly understood and largely unknown. There is no evidence of a malignant, neuropathic, or infectious component involved in the causation of this disorder. The mechanism of bone resorption is unclear.
Full Text. Gorham�s Disease or Massive Osteolysis Dipak V. Patel, MD Clinical Medicine & Research Volume 3, Number 2 : 65 -74 2005

Hemangioma

Danielle Katz — 2005-11-05 00:08:27 GMT

Intramuscular hemangiomas A significant number of intramuscular hemangiomas are associated with relatively mild symptoms, such as intermittent aching or discomfort with exercise. These may require no treatment and may have no significant sequelae. Unfortunately, those symptomatic enough to indicate treatment are those most likely to be incompletely excised, and thus, they recur. Recurrence rates following surgery range from 18-50%. Synovial hemangiomas The outcomes for diffuse synovial hemangiomas are similar to those of intramuscular hemangiomas. Patients with localized synovial hemangiomas tend to have excellent results following surgical excision. Hemangiomas of bone Many osseous hemangiomas remain asymptomatic, require no treatment, and have no significant sequelae. Hemangiomatosis Hemangiomatosis often becomes symptomatic during childhood, and yet is nearly impossible to excise. Therefore, treatment with chemotherapy has been tried with variable success. Gorham disease The rarity of Gorham disease prohibits a clear assessment of its prognosis. Patients have had variable results from treatment with steroids and radiation therapy.

Hereditory Multicentric Osteolysis OMIM

Not Available — 2008-03-22 00:08:27 GMT

Among the offspring of double second cousins, Torg et al. (1969) described a new skeletal disorder to which they gave the above designation. In addition to collapse and resorption of the carpal and tarsal bones, there was osteoporosis, cortical thinning, and increased caliber of the tubular and long bones. Clinically, the disorder was characterized by fusiform enlargement of the digits and flexion contractures of the knees, hip, and elbows.

Hereditory Osteolysis of carpal bones with nephropathy OMIM

Not Available — 2008-03-22 00:08:27 GMT

Osteolysis of the carpal bones led to disappearance of these bones in older subjects. Deformity of the hands suggesting arthritis also occurred in severe cases. Hypertension and renal failure resulting from arteriolar thickening were internal complications.

Management of a Fracture in a Known Case of Gorham's Vanishing Bone Disease

Clough, Donaldson & McNeely — 2008-03-30 00:08:27 GMT

We present the case of a right handed construction worker who has sustained two fractures in his left forearm. In 1990 he sustained a wrist fracture which failed to heal. The bone of the distal radius, ulna and carpus was re-absorbed. In 1995 he had radiotherapy and a free vascularized fibular graft from proximal ulna to 2nd metacarpal resulting in fusion of his left wrist and forearm but resolution of the injury. The history and a bone biopsy showing intense neovascularity together made the diagnosis of Gorham's Disease. He injured himself again in 1998 sustaining a fracture of the left olecranon process. A bone biopsy at the time of internal fixation confirmed that the bone was affected by his condition. Aggressive adjunctive therapy was initiated immediately following the surgery and included electrical stimulation, osteoclast inhibitory therapy and radiotherapy. At three months post injury the patient had painless function of his elbow and returned to work against advice.
The Xray showed evidence of healing. This case will be discussed and the literature reviewed with emphasis on the pathological process. Since this involves haemangiomatous replacement of normal bone we suggest that the new anti- neovascularization drugs be tried in this condition.
Clough, Donaldson & McNeely 1998 INABIS Poster

Osteolysis induced by AV-fistula in idiopathic carpotarsal osteolysis

Muyshondt et a; — 2008-03-22 00:08:27 GMT

Account of Idiopathic Carpotarsal Osteolysis in Ilse Muyshondt, Luc Lateur, Guy Van Roost and Bart Maes1 Nephrol Dial Transplant (2003) 18: 2185-2188

Primary Idiopathic osteolysis

Naranjo et al — 2008-03-22 00:08:27 GMT

A clinical, analytical, and radiological study was carried out on three members of the same family with multicentric idiopathic osteolysis. Transmission appeared to be via the dominant autosome present in the mother and two daughters. In the daughters osteolysis was seen in the carpal and tarsal bones, whereas in the mother radiology showed it to be in the phalanges of the hands and feet.
Full text available: Ann Rheum Dis. 1992 September; 51(9): 1074�1078. Primary idiopathic osteolysis: description of a family. A Naranjo, M A Muniain, J Mart�n, J V�zquez, and J N��ez

spontaneous osteolysis

Kahn — 2002-01-31 00:08:27 GMT

spontaneous osteolysis several syndromes of "vanishing bones": (Cf: rapid demineralization) idiopathic hereditary osteolysis autosomal dominant, manifest in early childhood carpals + tarsals involved first autosomal-recessive carpal and tarsal osteolysis idiopathic osteolysis with nephropathy carpals, tarsals, adjacent tubular bones involved azotemia --> death in early adulthood massive osteolysis of Gorham angiomatous destruction

Treatment of Gorham's Disease

Yip et al — 2002-08-26 00:08:27 GMT

Case report of a patient with Gorham's disease treated with pamidronate. Archive Copy 2004

Vanishing Bone Disease

Inamadar et al — 2005-08-29 00:08:27 GMT

Case report from dermatological viewpoint Indian Journal of Dermatology, Venereology and Leprology 70(3)