A nonmalignant, but sometimes fatal, syndrome of massive osteolysis complicated by lymphangiomatosis. Bone is replaced by fibrovascular tissue with capillary proliferation.A mass of proliferating, thin-walled vascular and lymphatic channels extend into adjacent bones, viscera, and soft tissues.The process often begins after minor trauma. It may result from enhanced osteoclastic activity, and interleukin-6 may have a role (Devlin et al 1996). It occurs predominantly in young people. Surgery may be required for ligation of the thoracic duct. Although grafting can be performed, resorption of the grafted bone may occur.
1. Osteolysis; areas most commonly affected are the shoulder, upper arm, pelvis, jaw, thorax, o and spine. Pain and discomfort may result from bone involvement and deformity, neural damage or and chest problems may occur. a
2. Pathological, thin-walled vascular or sy lymphatic channels develop in association with the osteolysis.These extend into bones, viscera, 3 and soft tissues.
3. Pleural effusions or chylothorax may occur, and when the latter develops, it is associated with a high mortality. Malnutrition, lymphopenia and sepsis result from massive fluid and protein losses into the pleural cavity.
4. Treatment includes radiotherapy, bleomycin, ligation of thoracic duct, and bone grafts.
5. Deaths have occurred from chest wall involvement, spinal cord transection, sepsis, and pulmonary aspiration (Choma et al 1987).
1. Problems of reduction in lung function in the presence of an effusion or a chylothorax (McNeil et al 1996).
2. Complications associated with malnutrition from loss of protein from the chylothorax; one patient was draining 1500 ml day-1 (Riantawan et al 1996). Anaesthesia was reported for revision of pleurosubclavian shunt (Mangar et al 1994). Another patient developed bilateral pleural effusions following spinal decompression (Szabo & Habre 2000).
3. Vertebral destruction, vertebral collapse, and spinal cord compromise (Aoki et al 1996).
4. Mandibular and maxillary involvement (Ohya et al 1990).The disease can present with a pathological fracture after only minor trauma (Fisher & Pogrel 1990). In one patient, massive mandibular osteolysis resulted in obstructive sleep apnoea syndrome (Kayada et al 1995). Bone grafts may subsequently undergo resorption.
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