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Year : 2005  |  Volume : 15  |  Issue : 4  |  Page : 453-454
Osteopoikilosis : A case report


Parakh X-Ray & Diagnostic Centre, Kampoo, Gwalior 474009, MP, India

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Keywords: Osteopoikilosis, Osteosclerotic Dysplasias

How to cite this article:
Khot R, Sikarwar J S, Gupta R P, Sharma G L. Osteopoikilosis : A case report. Indian J Radiol Imaging 2005;15:453-4

How to cite this URL:
Khot R, Sikarwar J S, Gupta R P, Sharma G L. Osteopoikilosis : A case report. Indian J Radiol Imaging [serial online] 2005 [cited 2019 Jul 23];15:453-4. Available from: http://www.ijri.org/text.asp?2005/15/4/453/28771

   Introduction Top


Osteopoikilosis is an autosomal dominant condition characterized by multiple, discrete round or ovoid radio densities in cancellous bones [1]. These are asymptomatic and incidental findings. They histologically represent compact bone islands. They are occasionally associated with cutaneous lesions as well as other osteosclerotic skeletal disorders [2].


   Case Report Top


A twenty four years old man was referred to the radiology department for a radiograph of bilateral hip joint for evaluation of low back ache following history of a fall. The patient did not have any other complaints. Clinical examination was unremarkable. His laboratory values including erythrocyte sedimentation rate, rheumatoid factor, C-reactive protein, kidney and liver function tests, serum calcium, phosphorus, magnesium, alkaline phosphatase, sex hormone and parathyroid hormone levels were normal.

The radiograph of hip joint showed juxta-articular osteopenia and multiple small discrete, round, oval, dense lesions in symmetric distribution. Theses lesions were distributed more in the epiphysis and metaphysis of both the femoral bones at the proximal end extending down to the intertrochantric region. [Figure - 1]. Pelvic bones showed dense small discrete lesions along left sacroiliac joints, pubic symphysis and bilateral inferior pubic rami [Figure - 1]. Similar lesions were seen in the radiograph of shoulder; lesions were centered at the proximal epiphyseal and metaphyseal ends of both the humerus bone and acromion process. All findings were suggestive of osteopoikilosis. [Figure - 2]. The carpal and tarsal bones showed no dense lesions.

After the diagnosis, the patient was re-evaluated, and any overlooked pathology was searched. There was no pain or swelling of the joints, no muscle contractures, no soft tissue or skin changes. There was no significant family history.


   Discussion Top


Osteopoikilosis (osteopathia condensans disseminata, or "spotted bone disease") is an asymptomatic disorder characterized by an abnormality in the enchondral bone maturation process. It may either be inherited in an autosomal dominant fashion or occur sporadically. Familial clustering suggests a dominant inheritance. Men and Women are equally affected [1],[3].

Osteopoikilosis results in numerous round 2-10 mm densities of oval or rounded shape, that are symmetrically distributed within the metaphyses and epiphyses of the long bones, that appears in childhood and persists throughout life [2],[3],[5]. Sites of predilection include phalanges (100%), carpal bones (97.4%), metacarpals (92.5%), foot phalanges (87.2%), metatarsals (84.4%), tarsal bones (84.6%), pelvis (74.4%), femur (74.4%), radius (66.7%), ulna (66.7%), sacrum (58.9%), humerus (28.2%), tibia (20.5%), and fibula (2.8%) in one study of 4 families. The ribs, skull and vertebrae are typically spared. On radiography, small, well-defined, ovoid foci of increased radio density are clustered in periarticular osseous regions [1],[2],[4]. These little deposits of bone are essentially multifocal bone islands. On histology these foci are formed by dense trabeculae of spongious bone, sometimes forming a nidus without communication with bone marrow [2].

Some patients have connective tissue nevi called dermatofibrosis lenticularis disseminata. These, in addition to osteopoikilosis, comprise the  Buschke-Ollendorff syndrome More Details [6]. Osteopoikilosis have also been found to be associated with a tendency to keloid formation, dwarfism, spinal stenosis, dystocia, melorheostosis, tuberous sclerosis and scleroderma [3],[4],[7]. Possibly a relationship exists between this condition and other, especially osteopathia striata and melorheostosis, a combination of abnormalities that has been termed mixed sclerosing bone dystrophy [2].

Differential considerations include osteoblastic metastasis, tuberous sclerosis, mastocytosis and synovial chondromatosis [1],[3]. Its major clinical significance is that it should not be confused with metastatic osteoblastic lesions. The symmetrical distribution, the propensity for epiphyseal and metaphyseal involvement, and the uniform size of the foci are features that suggest osteopoikilosis, a diagnosis that is supported by normal alkaline and acid phosphatase and a bone scan of normal appearance [1],[2],[8].

The recognition of this asymptomatic condition by plain radiograph makes other expensive investigations unnecessary [1].

 
   References Top

1.Resnick D, Niwayama G. Enostosis, hyperostosis and periostitis. Reznick D, Niwayama G. Diagnosis of Bone and Joint Disorders. W.B Saunders Company Philadelphia 1988, pp.4084-4088.  Back to cited text no. 1    
2.Lagier R, Mbakop A, Bigler A. Osteopoikilosis:a radiological and pathological study. Skeletal Radiol 11(3):161-8, 1984.  Back to cited text no. 2    
3.Benli IT, Akalyn S, Baysan E et al. Epidemiological and radiological aspects of osteopoikilosis. J Bone Joint Surg Br 74(4):504-6, 1992.  Back to cited text no. 3    
4.Sarralde A, Garcia CD, Nazara Z. Osteopoikilosis: report of a familial case. Genet Couns 5(4):373-5, 1994.  Back to cited text no. 4    
5.Young LW, Gersman I, Simon PR. Radiological case of the month. Osteopoikilosis: familial documentation. Am J Dis Child 134(4):415-6, 1980.  Back to cited text no. 5    
6.Roberts NM, Langtry JA, Branfoot AC. Case report: Osteopoikilosis and the Buschke-Ollendorff syndrome. Br J Radiol 66(785):468-70, 1993.  Back to cited text no. 6    
7.Weisz GM. Lumbar spinal canal stenosis in osteopoikilosis. Clin Orthop 166:89-92, 1982.  Back to cited text no. 7    
8.Mungavan JA, Tung GA, Lambiase RE et al. Tc-99m MDP uptake in osteopoikilosis. Clin Nucl Med 19(1): 6-8, 1994.  Back to cited text no. 8    

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Correspondence Address:
R Khot
Room No.11, Senior Girls Hostel, GRMC & JA Hospital Campus, Gwalior, MP- 474009
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.28771

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    Figures

[Figure - 1], [Figure - 2]

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