Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 1986

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

MUSCULOSKELETAL RADIOLOGY Table of Contents   
Year : 2010  |  Volume : 20  |  Issue : 1  |  Page : 47-49
Ribbing disease


Department of Radiology and Imaging Sciences, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai - 600 116, India

Click here for correspondence address and email

Date of Web Publication6-Feb-2010
 

   Abstract 

Ribbing disease is a rare sclerosing dysplasia that involves long tubular bones, especially the tibia and femur. It occurs after puberty and is reported to be more common in women. In this article we describe how Ribbing disease can be differentiated from diseases like Engelmann-Camurati disease, van Buchem disease, Erdheim-Chester disease, osteoid osteoma, chronic osteomyelitis, stress fracture, etc.

Keywords: Ribbing disease; imaging; sclerosing

How to cite this article:
Mukkada PJ, Franklin T, Rajeswaran R, Joseph S. Ribbing disease. Indian J Radiol Imaging 2010;20:47-9

How to cite this URL:
Mukkada PJ, Franklin T, Rajeswaran R, Joseph S. Ribbing disease. Indian J Radiol Imaging [serial online] 2010 [cited 2019 Nov 18];20:47-9. Available from: http://www.ijri.org/text.asp?2010/20/1/47/59754

   Introduction Top


Ribbing disease is a rare sclerosing dysplasia involving the long tubular bones, especially the tibia and femur. In order to avoid unnecessary treatment, it is important to differentiate this entity from other sclerosing dysplasia like Engelmann-Camurati disease, Van Buchem disease,  Erdheim-Chester disease More Details, osteoid osteoma, chronic osteomyelitis, stress fracture and rarely, neoplasms. [1] There are very few case reports, so far, which have described the findings in all the imaging modalities in a single patient. [2],[3] We would like to report a case of Ribbing disease, with emphasis on how it can be differentiated from its close mimics.


   Case Report Top


A 37-year-old woman presented with pain in the right leg of six months' duration and pain in the left leg of four weeks' duration. While the pain in the left leg was mild, that in the right leg had increased progressively. Based on the radiographic features, she was treated with antibiotics elsewhere as a case of low-grade osteomyelitis and was referred to our institution for further management as there was no improvement in the symptoms. There was no history of similar illness in her family. Her general examination was unremarkable. Local examination did not reveal any significant findings except for mild tenderness. Her routine blood investigations were unremarkable.

A frontal radiograph of both legs demonstrated sclerosis with cortical thickening involving the diaphyses of both tibias, which was more pronounced on the right side [Figure 1]. A CT scan showed circumferential cortical thickening with almost complete replacement of the medullary cavity [Figure 2]. MRI revealed similar findings; in addition, it revealed bone marrow edema and minimal adjacent soft tissue inflammation [Figure 3]a and b. Whole body Tc-99 m methylene diphosphonate (MDP) bone scan showed focal areas of increased tracer uptake in both tibial diaphyses, which were more on the right side [Figure 4]. No focus of increased tracer uptake was seen elsewhere in the skeleton. Biopsy from the right tibial diaphysis showed cortical thickening with packed trabeculae, suggestive of Ribbing disease.


   Discussion Top


Ribbing disease is a nonhereditary sclerosing dysplasia of unknown etiology affecting the diaphyses of tubular bones with sparing of epiphyses; it especially affects the tibia and femur. It was first described in 1949 by Ribbing. [4] It is a disease of adults and the incidence is reported to be more in women, [4] our patient also being a woman in her thirties. The typical presenting symptom is pain, which may be progressive in nature and associated with physical activity. Usually a self-limiting disorder, it may progress in some patients.

Radiographically, it is characterized by cortical and medullary sclerosis confined to the diaphysis, with sparing of the epiphysis. CT and MRI can delineate the extent of disease, reveal asymmetricity if there is bilateral disease, and also help in ruling out any associated cortical break, periosteal reaction, or soft tissue component. These findings help in differentiating Ribbing disease from other conditions like osteosarcoma, lymphoma, and metastasis. MRI shows low-signal-intensity areas in the medullary cavity, corresponding to the sclerosis, on all pulse sequences. Associated increased signal on short tau inversion recovery (STIR) and proton-density fat-saturation sequences may be seen; this represents bone marrow edema, which is believed to be the cause of pain in these patients. [5] Cross-sectional imaging also helps in differentiating Ribbing disease from osteoid osteoma, where the characteristic nidus can be seen. Bone scintigraphy shows increased uptake of Tc-99 MDP in the involved diaphysis and helps in ruling out abnormal tracer concentration elsewhere in the skeleton. [6]

The close mimic of Ribbing disease is Engelmann-Camurati disease, a hereditary sclerosing dysplasia with autosomal dominant inheritance that affects children. Both diseases involve the diaphyses of long tubular bones with sparing of the epiphyses, but Engelmann-Camurati disease is bilaterally symmetrical, whereas Ribbing disease is either unilateral or, if bilateral, always asymmetric. The presence of skull bone involvement, gait and neurologic abnormalities, and anemia with extramedullary hemopoiesis favors Engelmann-Camurati over Ribbing disease. [1],[6],[7] Van Buchem disease (hyperostosis corticalis generalisata familiaris) is a hereditary sclerosing dysplasia that affects the long bones; the diaphysis is predominantly affected but the epiphysis can also be involved. There is conspicuous involvement of the jaws. [8] Other sclerosing dysplasia like melorheostosis and osteopetrosis can be differentiated from Ribbing disease by their characteristic pattern. Bilateral involvement of tubular bones in Ribbing disease helps in excluding chronic sclerosing osteomyelitis, which is usually unilateral.

Erdheim-Chester disease is a systemic histiocytic disorder with sclerosis of long bones, but the clinical features and the involvement of bone epiphyses as well as of other systems/tissues like pericardium, lungs, and retroperitoneum helps in ruling out this rare disorder. [9] Stress reaction can mimic Ribbing disease radiologically, but the history and the relatively discrete involvement seen in the former may help in diagnosis. Neoplasms like lymphoma, metastasis, and multicentric osteosarcoma can easily be differentiated from Ribbing disease by the clinical history and imaging.


   Conclusion Top


Ribbing disease is mainly a diagnosis of exclusion. The important diagnostic features of Ribbing disease are adult onset, female predominance, absence of similar disease in family members, pain, normal serum markers; sclerosis of the diaphyses of long tubular bones, with sparing of epiphyses (especially affecting the tibia); the bilateral asymmetric involvement of bone, without any cortical break, periosteal reaction, and soft tissue component; and the absence of involvement of the skull base, the jaw, or other systems.

 
   References Top

1.Seeger LL, Hewel KC, Yao L, Gold RH, Mirra JM, Chandnani VP, et al. Ribbing disease: Imaging and differential diagnosis. AJR A J Roentegol 1996;167:689-94.  Back to cited text no. 1      
2.Abdul-Karim FW, Carter JR, Makley JT, Morrison SC, Helper SD, Joyce MJ, et al. Intramedullary osteosclerosis: Report of the clinicopathologic features of five cases. Orthopedics 1988;11:1667-75.  Back to cited text no. 2      
3.Chanchairujira K, Chung CB, Lai YM, Haghighi P, Resnick D. Intramedullary Osteosclerosis: Imaging Features in Nine Patients. Radiology 2001;220:25-230.  Back to cited text no. 3      
4.Ribbing S. Hereditary multiple diaphyseal sclerosis. Acta Radiol 1949;31:522-36.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Ziran N, Hill S, Wright ME, Kovacs J, Robey PG, Wientroub S, et al. Ribbing Disease: Radiographic and biochemical characterization, lack of response to pamidronate. Skeletal Radiol 2002;31:714-9.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Shier CK, Krasicky GA, Ellis BI, Kottamasu SR. Ribbing disease: Radiographic -scintigraphic correlation and comparative analysis with Engelman disease. J Nucl Med 1987;28:244-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Byanyima RK, Nabawesi JB. Camurati-Engelmann's disease: A case report. Afr Health Sci 2002;2:118-20.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Dixon JM, Cull RE, Gamble P. Two cases of Van Buchem's disease. J Neurol Neurosurg Psychiatry 1982;45:913-8.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Resnick D, Greenway GD, Genant HK, Brower AC, Haghighi P, Emme M. Erdheim Chester disease. Radiology 1982;142:289-95.  Back to cited text no. 9      

Top
Correspondence Address:
Philson J Mukkada
Department of Radiology and Imaging Sciences, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai - 600 116
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.59754

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Orthopaedic Case of the Month: Lower Leg Pain in a 41-year-old Woman
Seungcheol Kang,Ilkyu Han,Seung Han Shin,Han-Soo Kim
Clinical Orthopaedics and Related Research®. 2012; 470(1): 321
[Pubmed] | [DOI]
2 Ribbing disease: A case report and literature review
Öztürkmen, Y., Karamehmetoǧlu, M.
Öztürkmen, Y., Karamehmetoǧlu, M.. 2011; 45(1): 58-65
[Pubmed]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed3406    
    Printed185    
    Emailed3    
    PDF Downloaded503    
    Comments [Add]    
    Cited by others 2    

Recommend this journal