Vanishing Bone Disease Research Articles

Gorham-Stout Syndrome with Chylothorax in a Six-Year-Old Boy

Gorham-Stout syndrome, also called "disappearing bone disease, diffuse cystic angiomatosis of bone, disseminated lymphangiomatosis, Gorham's vanishing bone disease, phantom bone disease or idiopathic massive osteolysis, is a rare disease of unknown etiology and pathogenesis. It is characterized by rapidly progressive localized massive osteolysis associated with proliferation of vascular structures of benign origin in which the absence of new bone formation is representative. When it is complicated by chylothorax, the prognosis is poor. The authors report a 6-year-old boy with Gorham-Stout syndrome who presented with pleural effusion showing features of chylothorax, who responsed poorly to currently available therapeutic modalities.

Vanishing Bone Disease: A Review

Vanishing Bone Disease: A Review

Chronic recurrent Gorham-Stout syndrome with cutaneous involvement

Type IV osteolysis or Gorham-Stout syndrome is a rare condition characterized by recurrent vascular tumors that disrupt normal anatomical architecture. Gorham-Stout syndrome is most commonly associated with the skeletal system with resulting replacement of bone with scar tissue following tumor regression. The loss of entire bones has given Gorham-Stout syndrome the moniker vanishing bone disease. Natural progression of Gorham-Stout syndrome is characterized by spontaneous disease resolution. However, rare variants of recurrent, progressive, and/or systemic disease have been reported. We present a patient with a history of recurrent Gorham- Stout disease refractory to all treatment options considered. In addition to skeletal disease, our patient had soft tissue and cutaneous involvement, thus reflecting the more aggressive disease variant. Previous surgical attempts to control disease had been ineffective and the patient was referred to us for radiation therapy. Treatment with external beam radiation therapy resulted in good local control and symptom palliation, but full disease resolution was never accomplished. In addition to presentation of this patient, a review of the literature on etiological hypotheses and past/future treatment options was conducted and is included.

Chylotamponade

Gorham-Stout syndrome is a rare disorder that leads to extensive lymphangiomatosis in bones with resorption of adjacent bone matrix. As a result, the disease is also known as massive osteolysis or “vanishing bone disease.” Although the disease primarily occurs in bone, it may also involve adjacent soft tissues. We report a case of Gorham-Stout syndrome presented with chylotamponade as a result of extensive intrathoracic lymphatic hyperproliferation. A 50-year-old woman, who is known to have Gorham-Stout syndrome, presented with progressively worsening dyspnea for 1 week. She was hemodynamically stable and her cardiorespiratory examination was unremarkable except for elevated jugular venous pressure. Chest radiography revealed an enlarged cardiac silhouette and a small left pleural effusion. Transthoracic echocardiography confirmed a large pericardial effusion with evidence of tamponade. Urgent pericardiocentesis was performed, and ≈1.2 L of milky fluid, identified a s chyle on biochemistry, was drained. She became asymptomatic soon after pericardiocentesis; however, pericardial fluid reaccumulated over the next few days. She had been diagnosed as having Gorham-Stout syndrome from the age of 12 years. Massive osteolysis of the mandible developed, which required reconstruction. Osteolysis subsequently recurred in the reconstructed mandible. Also, bone involvement in the skull base had …

Commentary: massive osteolysis or Gorham-Stout disease

In this issue of the Journal, three interesting case reports, one in the print version 1 Tong A.C.K. Leung T.M. Cheung P.T. Management of massive osteolysis of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010; 109: 239-242 Google Scholar and two online, 2 Pedroletti F. Rangarajan S. McCain J.P. Velez I. The conservative treatment of a pathologic fracture in a patient with Gorham-Stout disease. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010; 109: e49-e52 Google Scholar , 3 Gondivkar S.M. Gadbail A. Gorham-Stout syndrome—a rare clinical entity and a review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2010; 109: e41-e48 Google Scholar discuss a rare condition characterized by a massive and progressive loss of bone. Although rare, this disease is recognized by several names, including disappearing bone disease (phantom bone), Gorham syndrome, Gorham-Stout syndrome, Idiopathic massive osteolysis, massive Gorham osteolysis, massive osteolysis, Morbus Gorham-Stout disease, progressive massive osteolysis, vanishing bone disease, and essential osteolysis. This bone resorption abnormality can affect different sites of the skeleton and in some instances the head and neck region, as described in these 3 cases. Gorham-Stout disease (GSD) is characterized by massive bone loss, which can affect different skeletal sites, and excessive intraosseous proliferation of small blood or lymphatic vessels, resulting in resorption and destruction of bone. The case reports and literature review update the reader in the various aspects that involve this rare condition, including the fact that the condition is often diagnosed by exclusion and late.

Vanished humeral head in a patient with rheumatoid arthritis: a case of Gorham’s disease

We report an 81-year-old female with rheumatoid arthritis who suVered from “vanishing bone disease or Gorham’s disease” involving the right humeral head. In 2003, she was diagnosed of elderly onset rheumatoid arthritis with high titer of rheumatoid factor, which was treated with leXunomide, hydroxychloroquine, and lowdose prednisolone. In October 2008, she presented with pain and swelling of the right shoulder which had persisted for 1 month. She had been doing farm work, but denied having experienced any major trauma. Around that time, her arthritis was controlled pretty well. On laboratory test, serum C-reactive protein level was 1.43 mg/dL (normal range, <0.3 mg/dL). Unexpectedly, humeral head was not visible (Fig. 1, short arrow) and soft tissue swelling was present on plain radiograph of the right shoulder. In addition, multiple calciWc materials were found adjacent to the proximal metaphysis of the right humerus (Fig. 1, long arrow). Ultrasonography revealed marked distension of the subacromial bursa and 65 ml of bloody Xuid was aspirated. On Xuid analysis, white blood cell count was 190/mm and red blood cell count 18,000/mm. Because she had been followed up in our rheumatology clinic, we had checked regular chest radiograph in June 2008, 3 months before her right shoulder pain developed. Notably, right humeral head was visible on that chest radiograph (Fig. 2, arrow). Based on the disappeared humeral head and multiple calciWc materials, rapid osteolysis of the humeral head seemed to have occurred during the 3-month period and the vanishing bone disease or Gorham’s disease was diagnosed. Because she was unwilling to undergo further treatment such as shoulder joint arthroplasty, conservative care was done. Vanishing bone disease, called Gorham’s disease, is a rare disease characterized by massive idiopathic osteolysis [1]. It can occur spontaneously or after trauma; however, precise etiology is still not known. In our patient, minor trauma seemed to be the most probable cause based on the hemorrhagic nature of the subacromial bursal Xuid. It was reported that excessive vascular proliferation and increased osteoclastic activity are thought to be the cause of the bone matrix destruction in Gorham’s disease and bone matrix is replaced by thinwalled vessels and Wbrous tissue [2]. Gorham’s disease can involve various bones such as ribs, vertebra, humerus, hip, foot, and even facial bones [3–8]. It can be progressive and spread to adjacent bones. The prognosis varies depending on which bones are involved. In most cases it is rarely fatal, but extension to spinal cord and/or internal organs can be life threatening when vertebra, scapula, rib or sternum are involved. As for the treatment, radiation therapy, chemotherapy, antiresorptive medications, and surgical intervention such as grafting have all been tried with diVerent degrees of success, but eVective therapy is not established yet [1]. So far, only H.-J. Ko · K.-S. Park (&) · C.-S. Cho · H.-Y. Kim Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea College of Medicine, 505 Banpo-dong, Seocho-gu, Seoul 137-701, South Korea e-mail: pkyungsu@catholic.ac.kr

Gorham's massive osteolysis of the mandible – a progressive radiographic presentation

Massive osteolysis is well known under the synonyms Gorham's disease, vanishing bone disease and phantom bone disease. It is a rare condition of unknown aetiology and results in the progressive destruction of bony structures. Only a few cases have been reported in the jaws. Considering the rarity of the condition, we report here an interesting and unique case of massive osteolysis of the lower jaw that initially affected the mandibular basal and alveolar bone, and subsequently involved the rami and the condyles. The clinical and radiological findings were consistent with the criteria for the diagnosis of Gorham's massive osteolysis.

Gorham's Disease

Gorham's disease, also known as massive osteolysis, Gorham-Stout disease, vanishing bone disease, or, phantom bone disease is a rare disorder of the musculoskeletal system. The disease is characterized by osteolysis in bony segments, with localized proliferation of lymphatic channels. The presence of abundant, leaky systemic lymphatic vessels is often accompanied by chylous ascites. There is no standardized treatment available for Gorham's disease, and its molecular mechanisms remain unclear. Future strategies for understanding Gorham's disease should emphasize its apparent identity as a disease of disordered lymphangiogenesis. Breakthroughs in lymphatic research have identified several lymphangiogenic pathways that may play a relevant role in Gorham's disease.

Multicentric Gorham's Disease in the Oral and Maxillofacial Region: Report of a Case and Review of the Literature

Gorham's disease, also known as massive osteolysis, Gorham-Stout syndrome, vanishing bone disease, and phantom bone disease, is a rare disease characterized by progressive osteolysis and proliferation of fibrous connective tissue similar to that of vascular malformations.1 It was first described by Jackson in 18382 and characterized pathologically by Gorham and Stout in 1955.3 It is more common in young adults with no significant gender difference and affects mainly the pelvis, humerus, and axial skeleton.

Gorham Disease: A Rare Cause of Massive Bone Destruction

Background. Gorham disease is a very rare condition that causes severe bone destruction. Also referred to as massive osteolysis and vanishing bone disease, it may progress from 1 to several bones, replacing them with fibrous tissue. Fewer than 40 cases of Gorham disease of the maxillofacial skeleton have been reported.1 Patients are often asymptomatic or present only with mild pain or swelling. Correct diagnosis is difficult and often delayed, because other, more common, diagnoses are considered first.

Vanishing Bone Disease: Review and Case Reports

Vanishing bone disease is a rare idiopathic disease, leading to extensive loss of bony matrix, replaced by proliferating thin-walled vascular channels and fibrous tissue. There are >191 cases reported in the English literature. Gorham and Stout made the first overview of the disease in 1955 and they first presented 24 cases known at that time. The etiology remains speculative, the prognosis unpredictable, and effective therapy still unknown. The disease can be monostotic or polyostotic although multicentric involvement is exceptional. We report 2 cases of a histologically studied vanishing bone disease involving the humerus and the femoral head. The patients' past history was noncontributory. The radiographic study revealed a destructive lesion of the left humerus in the first case, and complete disappearance of the femoral head in the second case. Laboratory findings including hormonological tests revealed no evidence of metabolic, immunologic, neoplastic, or infection etiology. Histopathological findings of the 2 cases revealed thickened bone of lamellar structure without marrow cavities next to fibrous tissue, with few fibroblasts and a small number of newly formed vascular channels. The prognosis varies from slight disability to death by involvement of vital skeletal structures. The treatment of vanishing bone disease is controversial. Several treatment modalities have been proposed. Surgical intervention has been suggested as a method of choice by many authors and concerns local resection of the affected bone, with or without replacement prosthesis or bone grafts.

Skeletal angiomatosis - rare cause of bone destruction: A case report with review of literature

Classification of skeletal angiomatosis into aggressive and nonaggressive types is on the basis of their clinical behavior and pattern of skeletal involvement (regional and disseminated). Gorham's disease (massive osteolysis) is an aggressive form of skeletal angiomatosis that shows regional involvement, frequently involving the shoulder and hip areas. Cystic angiomatosis is a nonaggressive form of skeletal angiomatosis with multifocal involvement, predominantly affecting the trunk bones. The imaging modalities gave the diagnosis of cystic angiomatosis of humerus showing multicystic lytic areas. The histopathological differential diagnosis was cystic angiomatosis and Gorham's disease, as microscopically both are indistinguishable from each other. Both represent a complex network of dilated thin-walled capillaries growing in the marrow space associated with the destruction of bone and infiltration into the adjacent soft tissues. The case is presented because of its extreme rarity and due to the diagnostic dilemma, whether to label it as Gorham's disease or as cystic angiomatosis. Considering the site involved and its aggressiveness, the diagnosis is in favor of Gorham's vanishing bone disease as cystic angiomatosis is multicentric and nonaggressive, involving mostly vertebrae and skull with multicystic lytic lesions.

Vanishing Bone Disease of foot

Vanishing bone disease, also known as idiopathic osteolysis and Gorham Stout disease is a rare clinicopathological entity characterized by proliferation of intra osseous capillaries and lymphatics thereby leading to bony destruction. All age groups can be affected. The most common sites for predilection are the shoulder and pelvis. The authors report a rare and interesting case of idiopathic osteolysis of right foot in a 50 year old female who presented with progressive regression of 2nd and 3rd toes.

A Case of Gorham's Disease with Chylothorax Treated Curatively with Radiation Therapy

Gorham's disease, also known as vanishing bone disease or massive osteolysis, is a rare disorder of uncertain etiology. It is characterized by uncontrolled proliferation of vascular or lymphatic capillaries within bone, leading to resorption and replacement with angiomatous tissue. It can be complicated by chylous pericardial and pleural effusions, which can be life threatening. Patients are also at risk of mortality or serious morbidity due to bone destruction, especially when the disease involves the spine. We report the case of a 31-year-old female with Gorham's disease involving several bones along with chylous pericardial and pleural effusions. She was effectively treated with definitive radiation therapy.

Vanishing bone disease involving the pelvis

Vanishing bone disease is a rare condition characterized by progressive osteolysis of the bony structures by vascular tissue and their replacement by fibrous, vascular connective tissue. A 38-year-old lady who had a previous history of angioma of the left iliac bone presented with vague symptoms of a limping gait and pain during walking since 2 years duration. The radiologic findings were suggestive of degenerative changes. A possibility of metastatic disease was also considered. However the biopsy and Positron emission tomography (PET) scan ruled out any active disease. Like in most other cases this was possibly a self-limited disease where bone resorption had spontaneously arrested.

Total scapular replacement: Medium-term follow-up

Gorham's disease, or massive osteolysis (disappearing or vanishing bone disease, idiopathic osteolysis, progressive atrophy of bone), is an extremely unusual condition with less than 180 cases reported in the literature. In 1955 Gorham and Stout 5 Gorham L.W Stout A.P Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone). J Bone Joint Surg Am. 1955; 37: 985-1004 PubMed Google Scholar defined a specific disease entity from a review of 24 cases in the literature. The condition had, however, been previously reported by Jackson 8 Jackson J.B.S A boneless arm. Boston Med Surg J. 1838; 18: 368-369 Crossref Google Scholar , 9 Jackson J.B.S Absorption of the humerus after fracture. Boston Med Surg J. 1872; 10: 245-247 Google Scholar in 1838 and 1872.

Chylotamponade: an unusual presentation of gorham’s syndrome

Gorham’s Syndrome, also known as massive osteolysis or “vanishing bone disease” results from lymphangiomatosis with adjacent bone resorption. Chylothorax is a common complication in cases of mediastinal involvement. We report a case of Gorham’s Syndrome presenting as chylotamponade successfully treated with pericardial drainage, early parenteral nutritional support, bilateral pleurodesis for chylous effusions, and adjuvant external beam radiation.

Vanishing bone disease in a five year old: report of a case and review of the literature

Vanishing bone disease is a rare condition of unknown aetiology. It can affect almost any bone, including those of the maxillofacial region. It is most commonly seen in the second and third decades of life. To the author's knowledge, this is the second case reported in the maxillofacial region of a child within the first decade of life, and the first who survived.

Gorham disease of the orbit

PURPOSE: To report a case of Gorham disease, also known as vanishing bone disease, of the orbit. DESIGN: Interventional case report. METHODS: A 43-year-old Caucasian woman developed a depression in her left temple, pulsation in her left upper eyelid and temple, and bulging of the left upper eyelid and headache. Computerized tomography revealed a lytic lesion in the roof of the left orbit. RESULTS: Intraoperative examination of the lesion revealed a bony defect in the left orbital roof. Surgical intervention resulted in improvement of her symptoms, and pathology failed to reveal evidence of malignancy or other benign neoplastic process. CONCLUSION: Gorham disease should be considered in the differential diagnosis of lytic bony lesions of the orbit.

Gorham's Disease Involving the Shoulder Joint: A Case Report

Gorham's disease is a rare vanishing bone disease characterized by massive osteolysis and replacement with numerous wide engorged capillaries. The exact nature and effective treatment modalities for this condition are as yet unclear. A fifty-years old female, who had unexplained destruction of the left shoulder joint, was diagnosed as having Gorham's disease according to histopathological (capillary tissue aggregation) and immunopathological (immunoreactions with IL-1and IL-6) studies. Although we treated her with antiresorptive medication and radiotherapy, which are current treatment modalities, the destructive process progressed. We report upon this case and provide a review of its literature.