Mri findings of gorham s disease are nonspecific and variable16 and advanced imaging studies may fail to reveal the presumed vascular nature of this condition. It is characterized by uncontrolled proliferation of nonneoplastic vascular or lymphatic tissues. If the spine and ribs are affected, the subsequent kyphosis and chest wall deformity may cause severe. Treatment for gorhams disease involves surgical removal and reconstruction of affected areas and radiation therapy. A case of gorhams disease disappearing bone disease of the clavicle with bilateral pleural effusions was described by us in chest. A case of gorhams disease disappearing bone disease of the clavicle with bilateral pleural. Gorham disease is a rare condition affecting bones of skeletal system with unknown cause. Gorhamstout syndrome is a disease that presents idiopathic osteolysis of a bone or closely contiguous area.
Gorhamstout disease mimicking spondyloarthritis the. Symtoms may include pain, swelling, and increased risk of fracture. Gorhams disease was first reported in 1838, again in 1872, and was finally defined in 1955 by gorham and stout as a specific disease entity. Gorham syndrome with postoperative respiratory failure and.
Till date, less than 200 cases have been reported in the international literature with about 51 cases involving the maxillofacial site. Gorham s disease is a rare bone disorder characterized by bone loss osteolysis, often associated abnormal blood vessel growth angiomatous proliferation. Massive osteolysis known as gorham stout disease is a rare idiopathic disorder typically affecting long bones in a unifocal pattern. A 31yearold man presented with increasingly serious abdominal distention and back pain. The lymphatic anomaly of diffuse or multicentric proliferative lesions as an aggressive disease of the lymphatic system. Mar 01, 2017 gorhams disease is very rare, and some cases may be misdiagnosed or undiagnosed.
Vanishing bone disease gorham stout syndrome is a rare entity of unknown etiology, characterized by destruction of osseous matrix and proliferation of vascular structures, resulting in destruction and absorption of bone. We report a case of an adolescent boy with massive osteolysis of the mandible in whom the diagnosis of gorhams disease was made. Gorhams goramz disease is a very rare skeletal condition of uncertain etiology, characterized by the uncontrolled proliferation of distended, thinwalled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas andor fibrosis. Till date very few cases have been reported in the literature. Gorhams disease is a rare condition characterized by progressive osteolysis of bone with ultimate total disappearance of bone. Idiopathic osteolysis was first described by jackson in 1838. Vanishing bone disease gorhamstout syndrome is a rare entity of unknown etiology, characterized by destruction of osseous matrix and proliferation of vascular structures. Gorham s disease gd is a rare form of lymphangiomatosis associated with profound osteolysis. Small limb lesions can cause fractures, while spinal involvement or chylothorax associated with rib lesions can be life threatening. Gorham stout disease gsd is a rare disorder characterized by the proliferation of nonneoplastic thinwalled vascular tissue associated with progressive osteolysis1. Weather angiomatosis is the cause or the result of osteolysis is subject of intense discussion kawasaki et al. It is a rare condition that is difficult to diagnose, and. Gorhams disease is a rare osteolytic disorder characterized by progressive resorption of bone and replacement of osseous matrix by a proliferative nonneoplastic vascular or lymphatic tissue.
Ab we report a case of gorham s disease of the chest wall in a 29yearold man. Pdf gorhamstout disease phantom bone of the shoulder girdle. R, china 2 department of radiology, west china hospital, sichuan university, 37 guo xue xiang, wu hou district, chengdu 610 041, p. If you have problems viewing pdf files, download the latest version of adobe reader. Chylothorax as a debut form of gorhamstout disease. The natural history of gorham disease is poorly defined. The gorham times is a free volunteerrun community newspaper distributed every other thursday to more than 100 pickup sites throughout gorham.
Gorham syndrome is a rare disease that presents as progressive osteolysis, and may affect any part of the skeleton. Gorhamstout disease gsd is a rare disorder characterized by the proliferation of nonneoplastic thinwalled vascular tissue associated with progressive osteolysis1. Gorham stout disease is a rare disease of unknown etiology. The pathologic process involves the replacement of. The disease is most often seen in children and young adults. In 1955, gorham and stout established its clinical and. Gorham disease is characterized by proliferation of the vasculature of the bone in the early stage, followed by gradual osteolysis and fibrous replacement. Progressive destruction and resorption slowly or rapidly. It is a rare condition that is difficult to diagnose, and its treatment is controversial. We report a case of an adolescent boy with massive osteolysis of the mandible in whom the diagnosis of gorhams.
Gorhams disease of the spine is very rare and usually associated with poor prognosis. The most conspicuous element of gorhams disease is its radiographic features, wherein massive disappearance of the mandible is observed. International journal of oral and maxillofacial surgery. Gorham body of endangered whale hauled historical society. Although described long ago, this rare and potentially catastrophic disease remains understudied, thus, poorly understood. Lgda 19919 villa lante place boca raton, fl 33434 telefono. Gorhams disease a syndrome that is characterized by bone loss osteolysis which is often associated with swelling or abnormal blood vessel growth angiomatous proliferation. For language access assistance, contact the ncats public information officer. Gorham disease or vanishing bone disease is a poorly understood rare skeletal condition which manifests with massive progressive osteolysis along with a proliferation of thin walled vascular channels. Gorhams disease also called as vanishing bone disease, acute spontaneous absorption of bone, hemangiomatosis, lymphangiomatosis, is a rare disease.
The gorham times is a free volunteerrun community newspaper. It is characterized by spontaneous excessive replacement of bone by proliferative nonneoplastic thinwalled lymphatic andor. Treatment for patients with gorham s disease includes surgical removal, reconstruction of affected areas, and radiation therapy. Angiomatosis is strongly connected to the osteolysis. Gorham s disease pronounced goramz, also known as gorham vanishing bone disease and phantom bone disease, is a very rare skeletal condition of unknown cause, characterized by the uncontrolled proliferation of distended, thinwalled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas and. Successful treatment of gorhamstout syndrome in the spine b. Pdf gorhamstout disease phantom bone of the shoulder. May 14, 2016 gorham disease is a rare condition affecting bones of skeletal system with unknown cause. The syndrome can affect one or multiple bones of the patient, including the skull, the upper and lower extremities, the spine and pelvis. Gorhams disease pronounced goramz, also known as gorham vanishing bone disease and phantom bone disease, is a very rare skeletal condition of unknown cause, characterized by the uncontrolled proliferation of distended, thinwalled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas andor fibrosis. To our knowledge the number of identified cases worldwide ranges around 300. The disease starts in one bone but may spread to involve adjacent bony and soft tissue structures.
Treatment for gorhams disease stanford health care. Gorham disease of mandible treated with postoperative. Gorhams disease, also known by the name of disappearing bone disease, is an extremely uncommon medical condition of the skeletal system which does not have a specific etiology in which there is uncontrolled proliferation of lymphatic channels within the bone causing resorption and replacement of bone fibrosis. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. A standardized treatment protocol has not yet been defined due to the unpredictable natural history of the disease and variable clinical presentations. Gorhams disease pronounced goramz, also known as gorham vanishing bone disease and phantom bone disease, is a very rare skeletal condition of unknown cause, characterized by the. Malignant disease must be ruled out histologically, although lack of a. Gorham s disease of the calvarium ji zhang 1, jin li 1, liu ling 2, yue kang zhang 1 1 department of neurosurgery, west china hospital, sichuan university, 37 guo xue xiang, wu hou district, chengdu 610 041, p. A 37yearold woman with a 7year history of chronic bilateral pelvic pain was evaluated for aggravated left hip pain.
Jul 01, 2014 gorham s disease is a rare disorder of uncertain etiology characterized by spontaneous and progressive osteolysis of one or more skeletal bones. Gorhamstout disease phantom bone of the shoulder girdle. There may also be significant osteoclast activation. This change occurs at the cellular level and is an ongoing process throughout the life of an individual.
Gorham s disease a syndrome that is characterized by bone loss osteolysis which is often associated with swelling or abnormal blood vessel growth angiomatous proliferation. As of now, etiology of gorham s disease or disappearing bone disease is not known and is a matter of ongoing research. Because of the rarity, the disorder goes unrecognized. It may affect any bone of the body, although there is predilection for the pelvis, humerus, axial skeleton, and mandible. To the best of our knowledge, new bone formation has not been reported in previous cases of gorham s disease. A histologic specimen obtained by incisional biopsy from the left third rib revealed proliferation of lymphangiomatous tissue in the bone marrow. Gorhams disease, a congenital disorder characterized by massive bone loss disambiguation page providing links to topics that could be referred to by the same search term this. Gorham stout syndrome is a disease that presents idiopathic osteolysis of a bone or closely contiguous area. Mar 27, 2017 the most conspicuous element of gorhams disease is its radiographic features, wherein massive disappearance of the mandible is observed. Gorham and stout were the first to define this disease back in 1954 and 1955. Complex single step skull reconstruction in gorhams.
Generalized lymphatic anomaly and gorhamstout disease. Gorhamstout disease is a rare disease of unknown etiology. Gorhams disease is a rare bone disorder characterized by bone loss osteolysis, often associated abnormal blood vessel growth angiomatous proliferation. Various treatment modalities are undertaken with very. Gorhams disease gsd is a rare osteolytic disease with unclear etiology, and no known prevention or effective treatment. Extensive loss of calcium from a single bone so that it cannot be seen on xray. Complex single step skull reconstruction in gorhams disease. Gorhams disease of the calvarium ji zhang 1, jin li 1, liu ling 2, yue kang zhang 1 1 department of neurosurgery, west china hospital, sichuan university, 37 guo xue xiang, wu hou district.
Was on sirolimus for 2 yrs, which stabilized condition, but his pain continues to be very debilitating. Gorhams disease of the clavicle with bilateral pleural. She was diagnosed with spondyloarthritis spa 4 years prior and was receiving medical treatment. Gorham disease also known as gorhamstout disease, disappearing bone disease, or essential osteolysis is a rare disorder characterized by relatively painless bone. No standard treatment is currently available for gorham.
Bone loss can occur in just one bone or spread to soft tissue and adjacent bones. Mri findings of gorhams disease are nonspecific and variable16 and advanced imaging studies may fail to reveal the presumed vascular nature of this condition. Gorhams disease of the clavicle with bilateral pleural effusions eight years later to the editor. A diagnosis of gorham s disease was made based on these clinical, radiologic, and histologic findings. The pathologic process involves the replacement of normal bone by aggressively expanding but nonneoplastic vascular tissue, resulting in massive osteolysis of the adjacent bone. To our knowledge the number of identified cases worldwide ranges around. Gorhams disease of femur a response sir, we read with great interest the manuscript entitled gorhams disease of femur, by baba et al. Nov 16, 2015 gorham stout syndrome is a disease that presents idiopathic osteolysis of a bone or closely contiguous area. Report of a rare case of gorhamstout disease of both. Gorham disease is a rare osteolytic disorder with only around 200 cases reported in english literature. Gorham disease also known as gorham stout disease, disappearing bone disease, or essential osteolysis is a rare disorder characterized by relatively painless bone destruction with massive osteolysis due to progression of lymphangiomatous tissue. Gorhams disease is a rare and potentially disabling osteolytic disorder. Gorhams disease gd is a rare form of lymphangiomatosis associated with profound osteolysis.
The syndrome can affect one or multiple bones of the patient, including the skull, the upper and lower. Gorhamstout disease vanishing or disappearing bone disease, phantom bone is a rare disease of unknown aetiology that is characterized by massive osteolysis and excessive intraosseous proliferation of small blood or lymphatic vessels, resulting in progressive resorption of bone. Any bone can be involved but the upper arm, shoulder, and jaw are most frequent. The topic gorhams syndrome you are seeking is a synonym, or alternative name, or is closely related to the medical condition gorhamstout disease. It is characterized by spontaneous excessive replacement of bone by proliferative nonneoplastic thinwalled lymphatic andor blood vessels. Successful treatment of gorhamstout syndrome in the spine. A case of gorham s disease disappearing bone disease of the clavicle with bilateral pleural effusions was described by us in chest.
Gorham s disease, a congenital disorder characterized by massive bone loss disambiguation page providing links to topics that could be referred to by the same search term this disambiguation page lists articles associated with the title gorham. Bone loss can occur in just one bone, or spread to soft tissue and adjacent bones. Massive osteolysis known as gorhamstout disease is a rare idiopathic disorder typically affecting long bones in a unifocal pattern. S ir, gorhamstout disease is a rare disease of unknown aetiology, characterized by massive osteolysis and excessive intraosseous proliferation of small blood or lymphatic. To sum up, gorhamstout disease is a rare musculoskeletal disease that should be considered in the presence of chylothorax even as a first sign. Gorhams disease of the clavicle with bilateral pleural effusions. Aug 20, 2019 gorhams disease, also known by the name of disappearing bone disease, is an extremely uncommon medical condition of the skeletal system which does not have a specific etiology in which there is uncontrolled proliferation of lymphatic channels within the bone causing resorption and replacement of bone fibrosis. Gorhams disease genetic and rare diseases information. Gorhams disease of the calvarium zhang j, li j, ling l. Gorham stout syndrome, gorhams disease, vanishing bone disease, massive osteolysis.
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