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