Percutaneous osteoplasty in treatment of bone lymphangiomatosis
We present a case of a 34 year old woman who was diagnosed as bone lymphangiomatosis in the femur and tibia. The patient had undergone surgery for the lower limb discrepancy. Epiphysiodesis distal of the right femur was done at the age of 12 years and left femoral lengthening at the age of 14 years. Since, the age of 21 years the patient has had symptoms of lymphangitis, with an acute inflammatory process in the left thigh and leg as well as signs and symptoms of phlogosis, pain, edema, functional impairment and a fever of 39.5°C. Laboratory tests showed leukocytosis with left shift. Throughout the years, she suffered an additional thirteen episodes of lymphangitis, which was bacterial in some cases.\nAt the age of 29, the patient underwent curettage and filling of the femoral and tibial cavities with freeze-dried bone and autogenous cancellous bone from the iliac crest. As a result of these procedures, it was observed that this was a particular case of dysplasia as there was an accumulation of lymph in the interior of the cavity as well, which suggested lymphatic dysplasia or bone lymphangiomatosis. Reabsorption of the bone grafts was later observed, which coincided with two additional episodes of lymphangitis.\nThe differential diagnosis included neoplastic and proliferative bone marrow disorders, especially histiocytosis; as well as benign lesions such as solitary bone cysts, fibrous dysplasia, fibrous bone defects, cystic fibrous osteitis resulting from hyperparathyroidism and congenital generalized fibromatosis. She was diagnosed by histopathological study and she was treated by percutaneous osteoplasty and was followed for 2 years and 10 months in the femur and 2 years and 3 months in tibia.\nThe radiographic imaging preosteoplasty showed large areas of medullary resorption and cortical thinning of distal femoral and proximal tibial epiphysio metaphysial region [], CT scans revealed decreased trabeculation and endosteal scalloping with cortical thinning []. Magnetic resonance imaging (MRI) scans revealed diffuse lymphangiomatosis of bones and soft-tissues [].\nHistological sections stained with H and E, which revealed dense fibro-connective tissue with an abundance of large-caliber vascular channels []. Said channels were lined by flat monostratified epithelium, which tested positive for D2-40 (antibody that specifically stains lymphatic endothelial cells) through a specific immunohistochemical technique []. The postoperative images show cavities filled with acrylic cement in tibia (December 2010) [] and femur (May 2010) []. After over 2 years of followup (2 years and 10 months in the femur and 2 years and 3 months in tibia) there are no signs of disease progression at this level in the radiographic images [].