Ribbing disease (multiple diaphyseal sclerosis): imaging and differential diagnosis

• Published in: American journal of roentgenology (1976) Vol. 167; no. 3; pp. 689 - 694
• Main Authors: Seeger, L L, Hewel, K C, Yao, L, Gold, R H, Mirra, J M, Chandnani, V P, Eckardt, J J
• Format: Journal Article
• Language: English
• Published: United States 01.09.1996
• Subjects: Adult; Camurati-Engelmann Syndrome - diagnosis; Diagnosis, Differential; Diaphyses - pathology; Female; Femur - diagnostic imaging; Femur - pathology; Humans; Osteosclerosis - diagnosis; Radionuclide Imaging; Technetium Tc 99m Medronate; Tibia - diagnostic imaging; Tibia - pathology; Tomography, X-Ray Computed
• ISSN: 0361-803X
• DOI: 10.2214/ajr.167.3.8751682

This study describes the clinical presentation and the course of Ribbing disease in six patients and illustrates imaging features on plain radiography, conventional and computed tomography, and 99mTc-methylene diphosphonate bone scans. Between 1982 and 1990, six female patients presented with painful bony lesions that were believed to be Ribbing disease. Ten bones were affected: both tibiae in three patients, a unilateral tibia in one, both femora in one, and a unilateral femur in one. Plain radiographs and either conventional or computed tomography were available for all patients and 99mTc-methylene diphosphonate bone scans, for five patients. All patients underwent open biopsy and/or surgical decompression. The diagnosis was reached in all patients through a combination of clinical findings (lack of systemic signs of infection or laboratory values suggesting metabolic bone disease), imaging, histologic evaluation, and specimen cultures. Radiographs and tomographic studies showed benign-appearing endosteal and periosteal cortical thickening. Intense uptake of radionuclide tracer was confined to the shaft of all involved bones. All pathologic specimens revealed nonspecific changes that included a slow increase in the mass of cortical and endosteal bone. These specimens also assisted in excluding neoplastic or infectious causes for the new bone formation. Ribbing disease is a rare disorder that, on imaging studies, may simulate stress fracture, chronic infection, bone-forming neoplasia, or a systemic metabolic or endocrine disorder. Clinical and imaging features may suggest the correct diagnosis.