1/25. Acropathia ulcero-mutilans acquisita of the upper limbs.We report an 80-year-old woman with a bilateral mutilating acro-osteolysis of the fingers progressing over a period of 3 years. As the patient refused nerve or muscle biopsies, our diagnosis of acropathia ulcero-mutilans acquisita or Bureau-Barriere syndrome, characterized by osteolysis, remains hypothetical. Sporadic sensory mutilating acropathy of the upper limbs is rare, and we discuss the differential diagnosis.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/25. Winchester syndrome.Winchester syndrome was first described in 1969 and since then nine patients have been reported in the literature. The syndrome is characterized by short stature, coarse face, corneal opacities, generalized osteolysis and progressive painful arthropathy with joint stiffness and contractures of distal phalanges in combination with skin changes. The etiology is unknown. Parental consanguinity supports autosomal inheritance. The diagnosis is based on clinical and radiological manifestations. We describe a case in a 7-year-old Pakistani boy.- - - - - - - - - - ranking = 3.2800702906732keywords = chest (Clic here for more details about this article) |
3/25. Massive osteolysis of the maxillo-facial bones: case report and review of the literature.An unusual case of massive osteolysis destroying the left side of the mandible, the maxilla, the orbit, the cranium bones and the upper cervical spine is reported. The evolution of this disease was observed over a period of 4 years. The literature was reviewed, only 39 cases have been found involving the maxillo-facial bones. In our case, attempts at surgery was without success. However, further radiotherapy with 35 Gy controlled the progression of this osteolysis.- - - - - - - - - - ranking = 0.2keywords = upper (Clic here for more details about this article) |
4/25. 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.- - - - - - - - - - ranking = 0.4keywords = upper (Clic here for more details about this article) |
5/25. Mediastinal lymphangioma and chylothorax: thoracic involvement of Gorham's disease.We report a case of mediastinal lymphangioma associated with Gorham's disease in a 38-year-old man who had suffered recurrent clavicular fractures during a seven-year period. Mediastinal widening associated with osteolysis of the clavicles and the sternal manubrium was revealed by chest radiography, while computed tomography demonstrated a cystic anterior mediastinal mass infiltrating mediastinal fat and associated with osseous destruction of the clavicles and manubrium. chylothorax recurred during the course of the disease.- - - - - - - - - - ranking = 0.65601405813464keywords = chest (Clic here for more details about this article) |
6/25. Talo-patello-scaphoid osteolysis, synovitis, and short fourth metacarpals in sisters: a new syndrome?Osteolysis syndromes are characterized by resorption of affected bones with associated swelling and pain. Various forms of multicentric osteolysis syndromes including autosomal dominant and recessive carpal-tarsal osteolysis, Torg, Francois, Whyte-Hemingway, Hajdu-Cheney, Winchester, and other forms have been described. Most present in pre-school years with extensive involvement and destruction of multiple bones. We present a sister-pair, both of whom presented in early teenage, i.e., 13 and 15.5 years, respectively, with bilateral ankle, knee, and later, wrist pain. Radiological examination revealed bilateral osteolysis of tali, scaphoids, and patellae, and short fourth metacarpals in both sisters. Further investigation revealed absence of renal involvement, a normal excretion of amino acids, mucopolysaccharides and oligosaccharides, and presence of chronic synovitis in both sisters. Both parents and a younger brother were without radiographic or clinical evidence of the disease and there was no history of consanguinity. Thus, our sister-pair presented with the same carpal and tarsal bone involvement at a much later age, with evidence of chronic synovitis, along with short fourth metacarpals (brachydactyly type E changes) and without renal disease, suggesting a new syndrome with probable autosomal recessive inheritance.- - - - - - - - - - ranking = 0.65601405813464keywords = chest (Clic here for more details about this article) |
7/25. Winchester syndrome. A case report and literature review.The mucopolysaccharidoses are a group of inherited lysosomal storage diseases that are caused by a deficiency of specific enzymes. The acid mucopolysaccharides are stored in tissue and excreted in large quantities in the urine. The storage of this material leads to effects on a wide variety of tissues and to remarkable changes in morphologic features. Winchester syndrome is a rare disorder in the group of mucopolysaccharidoses. This article is a report of a case with classic clinical, radiologic, and biochemical characteristics of the Winchester syndrome.- - - - - - - - - - ranking = 3.9360843488079keywords = chest (Clic here for more details about this article) |
8/25. radiation therapy in the treatment of Gorham disease.An adolescent male with noonan syndrome presented with a nonhealing fracture of the proximal right humerus. Over a 7-month period there was progressive loss of bone in this region, resulting in a flail arm at 9 months. Radiographic review was consistent with Gorham disease. In addition, there was significant bleeding in the soft tissues and pain. radiation was delivered. Seven years passed, until the patient re-presented with right hemithorax near collapse secondary to chylothorax. A chest tube was placed with temporary relief, but significant effusion remained. radiation was again administered, and by the end of therapy the chest tube was removed. The effusion has not recollected at last follow-up, which is now 6 months. The use of radiation in the treatment of Gorham disease has been demonstrated to have excellent palliative ability.- - - - - - - - - - ranking = 1.3120281162693keywords = chest (Clic here for more details about this article) |
9/25. Gorham-Stout disease--stabilization during bisphosphonate treatment.A 45-year-old woman presented with recent onset of left-sided chest pain. On clinical examination, these symptoms seemed to be strictly localized to a region that was marked by a long-standing cutaneous erythematous lesion. Laboratory results showed no gross abnormalities. Radiological imaging including conventional X-ray, MRI scans, and 3D CT reconstruction of the rib cage revealed circumscript destruction of the left lateral ribs 9-11. Histological analysis of a rib biopsy showed angiomatous hypervascularization and intracortical fibrosis. In keeping with these findings, the patient's condition was diagnosed as Gorham-Stout disease, a rare condition with localized, often unilateral, bone destruction. Monotherapy with bisphosphonates (pamidronate 30 mg i.v. every 3 months) was initiated, leading to rapid disappearance of local pain. Follow-up over 24 months documented a stable clinical and radiological picture without evidence of progressive bone destruction.- - - - - - - - - - ranking = 0.65601405813464keywords = chest (Clic here for more details about this article) |
10/25. 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 = 1.9680421744039keywords = chest (Clic here for more details about this article) |
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