Damle Nishikant Avinash, Patnecha Manish, Kumar Praveen, Gadodia Ankur, Subbarao Kiran, Bal Chandrasekhar
Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
Indian J Nucl Med. 2011 Jan;26(1):36-9. doi: 10.4103/0972-3919.84612.
Ribbing disease is a rare form of sclerosing dysplasia characterized by benign endosteal and periosteal bone growth confined to the diaphyses of the long bones, usually the tibiae and femora. It occurs after puberty and is more commonly seen in women. The most common presenting symptom is pain that is usually self-limited; however, progression is known. The etiology and optimal treatment for the disease are as yet undefined. We present here the case of a 31-year-old woman with clinical, radiological and bone scan manifestations of Ribbing disease corroborated by bone biopsy. Radiographs demonstrated cortical thickening of the diaphyses of both tibiae. 99mTc-methylene diphosphonate bone scan revealed intense irregular uptake in diaphyseal region of both tibiae. Magnetic resonance imaging showed cortical thickening with bone marrow edema in bilateral tibial diaphysis with minimal adjacent soft tissue edema. Bone biopsy revealed predominantly dense lamellar bone with irregular sized and spaced haversian systems. Serum and urine markers of bone metabolism were within normal limits. The patient was treated with analgesics, and had partial relief from pain. Medullary rimming is the next treatment option in case pain progresses. This report emphasizes the role of bone scan in the diagnosis of this rare condition.
里宾病是一种罕见的骨硬化发育异常形式，其特征为良性骨内膜和骨膜骨生长，局限于长骨骨干，通常为胫骨和股骨。它在青春期后发病，女性更为常见。最常见的症状是疼痛，通常为自限性；然而，已知会有进展。该病的病因和最佳治疗方法尚未明确。我们在此报告一例31岁女性病例，其临床、放射学及骨扫描表现符合里宾病，经骨活检证实。X线片显示双侧胫骨骨干皮质增厚。99m锝 - 亚甲基二膦酸盐骨扫描显示双侧胫骨骨干区域有强烈不规则摄取。磁共振成像显示双侧胫骨骨干皮质增厚伴骨髓水肿，相邻软组织水肿轻微。骨活检显示主要为致密板层骨，哈弗斯系统大小和间距不规则。骨代谢的血清和尿液标志物在正常范围内。患者接受了止痛治疗，疼痛部分缓解。如果疼痛进展，髓腔边缘切除是下一个治疗选择。本报告强调了骨扫描在诊断这种罕见疾病中的作用。
