Skeletal Radiology

, Volume 31, Issue 12, pp 714–719

Ribbing disease: radiographic and biochemical characterization, lack of response to pamidronate

  • Navid Ziran
  • Suvimol Hill
  • Mary E. Wright
  • Joseph Kovacs
  • Pamela Robey
  • Shlomo Wientroub
  • Michael T. Collins
Case Report

DOI: 10.1007/s00256-002-0552-6

Cite this article as:
Ziran, N., Hill, S., Wright, M.E. et al. Skeletal Radiol (2002) 31: 714. doi:10.1007/s00256-002-0552-6

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 99mTc-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.

Diaphyseal dysplasia Tibiae Bisphosphonates Radiographs CT MRI

Copyright information

© ISS 2002

Authors and Affiliations

  • Navid Ziran
    • 1
  • Suvimol Hill
    • 2
  • Mary E. Wright
    • 3
  • Joseph Kovacs
    • 3
  • Pamela Robey
    • 4
  • Shlomo Wientroub
    • 6
  • Michael T. Collins
    • 4
  1. 1.Department of Orthopedic Surgery, University of Rochester, Rochester, New York, USA
  2. 2.Department of Radiology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland, USA
  3. 3.Department of Critical Care Medicine, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland, USA
  4. 4.Craniofacial and Skeletal Diseases Branch, National Institutes of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland, USA
  5. 5.CSDB/NIDCR/NIH, Building 30 Room 228, MSC 4320, Bethesda, MD 20892-4320, USA
  6. 6.Department of Pediatric Orthopedic Surgery, Dana Children's Hospital, Tel-Aviv Medical Center, Tel-Aviv, Israel