title: Ribbing disease: radiographic and biochemical characterization, lack of response to pamidronate.
abstract: 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. The onset is usually after puberty and the most common presentation is pain that is usually self-limited, but may progress. The etiology and optimal treatment for the disease are unknown. We present the case of a 39-year-old Hispanic man with clinical and radiological manifestations of Ribbing disease. Radiographs and CT imaging demonstrated typical cortical thickening in the mid-diaphyses of the tibiae bilaterally that correlated with intense tracer uptake on (99m)Tc-MDP bone scans. MRI demonstrated cortical thickening and abnormal marrow signal consistent with marrow edema. Bone marrow edema may explain the pain frequently associated with the disease. Multiple serum and urine markers of bone metabolism were within normal limits. In an effort to ameliorate pain, the patient was treated with the bisphosphonate, pamidronate. In spite of treatment, pain increased, requiring additional and larger doses of analgesics. Serial radiographs, CT, bone scans, and MRI all demonstrated disease progression with pamidronate treatment. In this report we present for the first time the finding of bone marrow edema with MRI as well as disease progression during intravenous pamidronate treatment.
authors.affiliation: Department of Orthopedic Surgery, University of Rochester, Rochester, New York, USA.
authors.forename: Navid
authors.initials: N
authors.lastname: Ziran
authors.forename: Suvimol
authors.initials: S
authors.lastname: Hill
authors.forename: Mary E
authors.initials: ME
authors.lastname: Wright
authors.forename: Joseph
authors.initials: J
authors.lastname: Kovacs
authors.forename: Pamela Gehron
authors.initials: PG
authors.lastname: Robey
authors.forename: Shlomo
authors.initials: S
authors.lastname: Wientroub
authors.forename: Michael T
authors.initials: MT
authors.lastname: Collins
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D003966:Camurati-Engelmann Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D004164:Diphosphonates / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D000077268:Pamidronate; D019275:Radiopharmaceuticals; D013669:Technetium Tc 99m Medronate; D014057:Tomography, X-Ray Computed
chemical_list: D000893:Anti-Inflammatory Agents; D004164:Diphosphonates; D019275:Radiopharmaceuticals; D000077268:Pamidronate; D013669:Technetium Tc 99m Medronate