|IMAGES IN ACADEMIC MEDICINE
|Year : 2019 | Volume
| Issue : 2 | Page : 124-126
Ganesh Singh Dharmshaktu1, Tanuja Pangtey2
1 Department of Orthopaedics, Government Medical College, Haldwani, Uttarakhand, India
2 Department of Pathology, Government Medical College, Haldwani, Uttarakhand, India
|Date of Submission||16-Jun-2018|
|Date of Decision||08-Jul-2018|
|Date of Acceptance||13-Jul-2018|
|Date of Web Publication||29-Aug-2019|
Dr. Ganesh Singh Dharmshaktu
Department of Orthopaedics, Government Medical College, Haldwani - 263 139, Uttarakhand
Source of Support: None, Conflict of Interest: None
Ribbing disease is an uncommon cause of localized pain and presents as a region of diaphyseal sclerosis sparing the epiphyseal area. Clinical features are to be correlated with advanced imaging modalities to rule out other differentials. Knowledge of this lesion is important to avoid inappropriate management and a case of Ribbing disease affecting tibia of a 52-year- old male is presented.
The following core competencies are addressed in this article: Medical Knowledge, Patient care and procedural skills.
Keywords: Diaphysis, Ribbing disease, tibia
|How to cite this article:|
Dharmshaktu GS, Pangtey T. Ribbing disease. Int J Acad Med 2019;5:124-6
Ribbing disease is a rare skeletal dysplasia affecting long tubular bones of the body and causing unexplained medullary and cortical sclerosis. The disorder is also known as hereditary multiple diaphyseal sclerosis or diaphyseal sclerosis. The diaphyseal region of tibia and femur is commonly involved with sparing of the epiphysis. The disease is common in females and pubertal age. It usually is unilateral deformity but can be bilateral with asymmetric involvement.
A 52-year-old male patient presented to us with mildly increase in the right shin size with mild pain on activity for the past 8 months [Figure 1]. There was no history suggestive of any “red flag” signs for the infective or neoplastic disorder. The complaint was limited to the right leg and no history of the similar incident was present in the family. The local temperature was normal and mildly tender enlargement of a palpable portion of shin was noted while the distal and upper leg had no symptoms. Initially, he was managed as stress-related pain in the leg with rest and pain medications. The radiograph showed enlargement of the diaphysis of the tibia with sclerosis and thickening in the intramedullary region. There was no soft-tissue swelling or signs of fracture noted [Figure 1]. The patient was advised magnetic resonance imaging (MRI) for further evaluation and it revealed altered signal intensities over a segment of the diaphysis [Figure 2]. The lesion was hypo-intense in T1 and hyperintense on T2 and fat-suppression images. The fibula was normal along with soft tissues, muscles, and neurovascular structures [Figure 3]. The core biopsy study revealed nonspecific sclerosis with medullary fibrosis. On the basis of clinic-radiological basis, diagnosis of ribbing disease was made.
|Figure 1: Clinical image and radiograph of the right leg showing thickening of the diaphyseal region. Radiographs showing hypertrophy, sclerosis of the cortical and intramedullary area seen. No periosteal reaction or soft-tissue swelling is noted|
Click here to view
|Figure 2: The magnetic resonance imaging sagittal view showing cortical and medullary sclerosis of diaphyseal segment while ends of the bone are unaffected. The lesion is hypo-intense in T1 images (a). The lesion appears hyper-intensities in T2 images (b) with sclerosis involving medullary cavity, and no soft-tissue extension or collection is seen|
Click here to view
|Figure 3: The wavy, irregular thickening in the diaphysis (a) and transverse cuts showing filling of the medullary cavity in T2 (b) and fat-suppressed images (c)|
Click here to view
The radiographic findings in cases of ribbing disease include cortical and intramedullary osteosclerosis in the diaphyseal region with no periosteal or soft-tissue signs of the infective or neoplastic lesion. Computerized tomogram scan and MRI are other useful aids with MRI delineates the extent of the lesion and confirms the presence of sclerosis and bone edema as well as rules out infective, neoplastic, or mimicking disorders. Some of the disorders that require exclusion are Camurati–Engelmann disease (progressive diaphyseal dysplasia) which also presents with diffuse diaphyseal dysplasia but commonly involves children and is bilateral. Van buchem's disease and Erdheim–Chester disease are another disorders with similar features, but in contrast, both may involve epiphysis, and some other characteristic sites of involvement such as jaw and cardio-respiratory tissues, respectively, may help in differentiation., Other mimics like melorheostosis, bone forming neoplasms, metastasis, and osteoid osteoma can be excluded on basis of characteristic features pertaining to them. Knowledge of disorders with similar clinico-radiological features is crucial to better diagnosis and avoiding irrelevant investigations.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
Ethical conduct of research
The authors followed the applicable EQUATOR Network (http://www.equator-network.org/) guidelines during the preparation of this work.
| References|| |
Ribbing S. Hereditary, multiple, diaphyseal sclerosis. Acta radiol 1949;31:522-36.
Mukkada PJ, Franklin T, Rajeswaran R, Joseph S. Ribbing disease. Indian J Radiol Imaging 2010;20:47-9.
] [Full text]
Janssens K, Vanhoenacker F, Bonduelle M, Verbruggen L, Van Maldergem L, Ralston S, et al.
Camurati-Engelmann disease: Review of the clinical, radiological, and molecular data of 24 families and implications for diagnosis and treatment. J Med Genet 2006;43:1-1.
Dixon JM, Cull RE, Gamble P. Two cases of Van Buchem's disease. J Neurol Neurosurg Psychiatry 1982;45:913-8.
Resnick D, Greenway G, Genant H, Brower A, Haghighi P, Emmett M. Erdheim-Chester disease. Radiology 1982;142:289-95.
[Figure 1], [Figure 2], [Figure 3]