1/11. chylothorax complicating Gorham's disease.Gorham's disease is a rare disorder characterized by a proliferation of thin-walled lymphatic vessels (lymphangiectasia) resulting in an osteolysis. A chylothorax is present in about one-fifth of the patients and carries a poor prognosis. In this circumstance, surgery including thoracic duct ligation, pleurodesis, and excision of involved tissue is probably the treatment of choice. It is facilitated by a precise radiological assessment using a computed tomographic scanning coupled with a lymphography. We report such a case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/11. Massive osteolysis of skull bones secondary to lymphangioma of paranasal sinuses and mastoid sinus: report of two cases in children.Massive osteolysis (MO) is a rare condition in which progressive localized bone tissue resorption is associated with proliferating thin-walled vessels in the absence of inflammation. Rare cases have been reported to occur in the skull. This paper describes two patients with MO who presented with massive assymetric swelling of the skull. This was associated with extensive enlargement of the paranasal sinuses (frontal, ethmoidal, and sphenoidal in one and the mastoid air cells in the other). The second patient developed subcutaneous emphysema on several occasions and the valsalva maneuver increased the swelling, indicating transmission of the air from the nasopharynx to the mastoid cells and from there to the subcutaneous tissue. In the first patient, the sinus mucosa was shown to be involved by an extensive lymphangioma, and a similar change was seen in the mastoid air cells (patient 2). We are proposing that MO of these two patients resulted from bone resorption due to progressive extension of sinus mucous lymphangiomata.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/11. Total mandibular reconstruction for massive osteolysis of the mandible (Gorham-Stout syndrome).Massive osteolysis (Gorham-Stout syndrome) is a rare condition of unknown aetiology that is thought to result from a localised endothelial proliferation of lymphatic vessels resulting in destruction and absorption of bone. The diagnosis of Gorham-Stout syndrome can be made only after first excluding osteolysis from infection, inflammation, endocrine disease, and cancer. The syndrome is rarely seen in the facial skeleton and has a variable prognosis. Many treatments have been advocated but only surgery and radiotherapy have had some success. We present a case of massive osteolysis of the entire mandible, which was reconstructed with a free vascularised fibular graft and bilateral prosthetic replacements of the temporomandibular (TMJ) joints. Four years later the fibular graft had been absorbed requiring further reconstruction with another fibula graft.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/11. Remission induced by interferon alfa in a patient with massive osteolysis and extension of lymph-hemangiomatosis: a severe case of Gorham-Stout syndrome.The treatment of massive osteolysis with lymphangioma and/or hemangioma (Gorham-Stout syndrome) has been controversial. The authors report on a patient with multiple massive osteolyses and extensive lymph-hemangiomatosis whose lesions were reduced by interferon alfa therapy. A 2-year-old girl had complained of left chylothorax. thoracoscopy showed an increase in small lymphatic vessels in the chest wall. The chylothorax was improved by coagulation of the lymphatic vessels. Later, multiple massive osteolyses appeared in the left 11th and 12th ribs, the TH10-L3 vertebrae, and the right femur. There were also hemangiomas in the liver and spleen, a tumor lesion in the left lower chest wall, and hemangiomatous change on the skin surface of the left back. The left lung had only a minimal air content. After OK-432 was injected into the femur and chest wall lesions, the femur lesion disappeared. Then, as right chylothorax appeared, OK-432 was injected into the right pulmonary cavity. The chylothorax disappeared, but pericardial effusion appeared. After steroid pulse therapy, pericardial effusion disappeared. During these treatments, the 7th to 10th ribs disappeared from the x-ray and scoliosis developed. One month later, a cloudy fluid collection in the right lung was found on computed tomography. Interferon alfa and steroid pulse therapy were started. Interferon alfa (1,500,000 units) was subcutaneously administered daily for 2 months and was gradually reduced and maintained at 1,500,000 unit/wk. steroids were also reduced and maintained at 5 mg/d of predonine. Later, the progress of osteolysis and the extension of lymph-hemangiomatosis stopped. Ten months later, hemangioma in the back disappeared, and the 7th to 10th ribs, which had disappeared, reappeared. The interferon alfa therapy was stopped 14 months after it was administered. The patient's condition has been stable for 10 months since then. At this time, computed tomography shows regression of the hemangiomatous lesion in the back. The authors clinically diagnosed the patient as having Gorham-Stout syndrome with extension of lymph-hemangiomatosis. Interferon alfa with or without steroid therapy should be a choice for patients with extension lesions.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/11. Massive Gorham-Stout syndrome of the pelvis.Gorham-Stout disease is defined as a spontaneous, massive, and nonfamilial idiopathic osteolysis. The diagnosis is based essentially on radiological and histological findings. biopsy reviews always reveal excessive intraosseous nonmalignant proliferation of small vessels, which results in bone resorption and may extend to adjacent bones and soft tissues. These lesions are progressively replaced by extensive fibrosis. Since its first description in 1955, there is still controversy about its prognosis, etiology, and treatment. A case of Gorham-Stout disease, located on the right pelvis, is reported with 50 years of clinical and radiographic follow-up, in a man who has never been treated. To date, this is the longest documented case report of the disease and its rare natural history. It demonstrates that after a variable time of evolution, the massive osteolysis is able to undergo spontaneous arrest and that the lesions may remain stable during several decades. Besides, no reossification was observed, even after 37 years of disease quiescence. Based on a large review of the literature, the authors then discuss the prognosis, etiopathology, and different therapeutic options available to halt the progression of the osteolysis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/11. Gorham disease: an intraoperative case study.Gorham disease is a rare chronic disorder that is characterized by the abnormal proliferation of thin-walled capillaries and small lymphatic vessels that results in the massive osteolysis of adjacent bone. Clinical manifestations are determined by the area of involvement, which may include the chest and ribs. The case presented involves a 47-year-old man with Gorham disease complicated by unilateral chylothorax who was treated with thoracic duct ligation. The anesthetic implications associated with Gorham disease are discussed, and nonsurgical primary and adjunctive treatments for chylothorax are summarized.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/11. Gorham's disease complicated by chyloma of the chest wall.Gorham's disease is a rare disorder characterized by a non-malignant proliferation of thin-walled lymphatic vessels that result in progressive bony destruction and often extend into the surrounding soft tissues. chylothorax is a common complication in cases of mediastinal involvement. Here we report a case of a 22-year-old male with Gorham's syndrome complicated by chyloma of the chest wall, but without chylothorax. To our knowledge, this is the first report demonstrating an abnormality of the parietal pleura prior to the clinical development of the chylothorax.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/11. Massive osteolysis of the mandible.This report deals with a massive osteolysis, which is a rare disease characterized by the progressive dissolution of contiguous osseous structure, occurring in the mandible of a 46-year-old Japanese female. Histopathologic examination of the amputation material revealed that the mandibular body, ramus, condylar and coronoid processes were completely replaced by fibrous tissue, but inferior alveolar nerve and artery in the lesion were intact. A few small islands of osteoid tissue or immature fibrous bone were seen in the lesion. In the transitional area between the lesion and normal bone, active absorption of bone trabeculae was in progress and intertrabecular spaces were occupied by densely packed fibroblastic cells, however, angiomatous proliferation of vessels was not observed. A few bone trabeculae were surrounded by osteoclasts, but osteoclasts might not play a primary etiological role in massive osteolysis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/11. Disappearing bone disease: a case report and review of the literature.Disappearing bone disease is a rare condition manifested by massive osteolysis. Its etiology is unknown. Histologically, involved bones show a nonmalignant proliferation of thin-walled vessels. The mechanism of bone absorption remains unclear. No treatment has proven effective in arresting the disease. Cases in which there is extraosseous involvement have a much poorer prognosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/11. Nephropathy of idiopathic multicentric osteolysis.Progressive osteolysis with disappearance of the carpal and tarsal bones occurs as a rare syndrome associated with a serious nephropathy. The characteristic skeletal changes have an onset in early childhood and precede renal failure by 10--15 years. The present case, the ninth reported in the world literature, was a 22-year-old, white female who presented with carpal and tarsal swelling at age 2. Severe hypertension and azotemia were present when the patient died of pneumonia and extracellular fluid volume depletion. At necropsy, the kidneys weighed 55 g each. microscopy showed proliferation of vascular intima and medial hypertrophy of renal vessels of all sizes. Tubulointerstitial and glomerular changes are probably secondary to the severe vascular disease. Although similar skeletal lesions can be seen in kindred with autosomal dominant and recessive inheritance, all reported cases of nephropathy including the present one are sporadic.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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