Affiliation:
1. City Clinical Hospital No.1,
Pirogov Russian National Research Medical University
2. Pirogov Russian National Research Medical University
Abstract
Melorheostosis is a rare mesenchymal dysplasia of bone manifesting as regions of sclerosing and thickening of bone tissue. This disease may involve the adjacent soft tissues and lead to joint pain, limitation of joint motion, stiffness resulting from abnormal ossification and soft-tissue contractures due to periarticular fibrosis. The paper describes a clinical case of a patient who presented with pain and stiffness in the proximal part of the left hip which patient suffered for the last 10 years. At first the patient had intermittent pain in the lower back and left hip during and after walking. Radiographs revealed dense sclerotic and wavy cortex and hyperostosis involving the left iliac crest, the acetabulum, and the femur. CT angiography with contrast was performed for preoperative planning. During the procedure the authors performed total hip arthroplasty of the left hip with excision of fibro-ossifications in the left ilioinguinal area. Severe periarticular fibrosis of the soft tissue was observed intraoperatively and cartilage-like formation was visible around the joint. A sample of the ossification bone was resected for histologic confirmation of the diagnosis; extensive cortical sclerosis with varying thickness typical of melorheostosis was found. Early postoperative period went without complications following routine post-THR protocol. Postoperative X-rays at 6, 12, 24 months did not reveal any complications or new ossifications. Full ROM and pain-free function were achieved in the left hip and lower back of the patient. The present clinical case of total hip arthroplasty with excision of fibroossifications provided good clinical outcome for melorheostosis of the left hip.
Reference15 articles.
1. Moulder E., Marsh C. Soft tissue knee contracture of the knee due to melorheostosis, treated by Total knee arthroplasty. Knee. 2006;13(5):395-396. doi: 10.1016/j.knee.2006.05.006.
2. Chernov A.P., Cherkashin S.S., Kudashev D.S. [Melorheostosis of the Upper Flaring Portion of the Ilium (case report)]. Vestnik travmatologii i ortopedii im. N.N. Priorova [N.N. Priorov Journal of Traumatology and Oorthopedics]. 2006;(1);89-90. (In Russian).
3. Mumm S., Wenkert D., Zanng X., McAlister W.H., Mier R.J., Whyte M.P. Deactivating germline mutations in LE MD3 cause osteopoikilosis and Buschke-Ollendorff syndrome, but not sporadic melorheostosis. J Bone Mineral Res. 2007;22(2);243-250. doi: 10.1359/jbmr.061102.
4. Léri A., Joanny M. Une affection non décrite des os: Hyperostose ‘en coulée’ sur toute la longueur d’un membre ou ‘mélorhéostose’. Bull Mem Soc Med Hop (Paris).1922;46;1141-1145. [in French].
5. Morris J.M., Samilson R.L., Corley C.L. Melorheostosis. Review of the literature and report of an interesting case with a nineteen-year follow-up. J Bone Joint Surg Am. 1963;45:1191-206.