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 Table of Contents  
TEACHING IMAGES
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 263

Maffucci syndrome revisited


Department of Radiology and Imaging, Yenepoya Medical College, Nithyananda Nagara, Deralakatte, Mangalore, Karnataka, India

Date of Web Publication11-Nov-2014

Correspondence Address:
Sandhya K Hemraj
Department of Radiology and Imaging, Yenepoya Medical College, Mangalore 575 018, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.144369

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How to cite this article:
Hemraj SK, Acharya DK, Ravichandra G. Maffucci syndrome revisited . Arch Med Health Sci 2014;2:263

How to cite this URL:
Hemraj SK, Acharya DK, Ravichandra G. Maffucci syndrome revisited . Arch Med Health Sci [serial online] 2014 [cited 2019 Oct 17];2:263. Available from: http://www.amhsjournal.org/text.asp?2014/2/2/263/144369


  Case Report Top


A 49 year old lady presented with pain, restricted movements of right shoulder, and soft tissue swellings in right wrist. Radiographs revealed multiple osseous enchondromas and soft tissue phleboliths in the hand suggesting hemangiomas. Skeletal survey revealed similar lesions in other bones. A diagnosis of Maffucci's syndrome was made.

This is a rare, congenital disorder manifested by enchondromatosis and multiple soft tissue hemangiomas. [1] The pathognomonic radiological findings include multiple radiolucent well demarcated osseous lesions causing expansile remodeling; some showing matrix mineralization suggesting chondroid origin [Figure 1]. [2] There are multiple soft tissue hemangiomas [Figure 2]. Bone scintigraphy is useful to detect chondrosarcomatous changes. [3]
Figure 1: Radiograph of right shoulder showing a large, osteolytic, expansile, multiseptated lesion involving the proximal metadiaphysis of right Humerus; with focal areas of cortical breech suggesting extraosseous extension and increased surrounding soft tissue density. There is another lesion in the scapula showing arc and rings calcification suggesting chondroid origin

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Figure 2: Radiograph of right forearm AP and Lateral views show a multiloculated, mildly expansile, osteolytic lesion involving shaft of right ulna causing cortical thinning and bony remodeling. There are multiple, rounded calcific densities in the soft tissues of right wrist in lateral view, suggesting phleboliths associated with hemangiomas

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  References Top

1.Subhash Desai, Kubeyinje, Belagavi, Desai S. Maffucci's Syndrome. Ann. Saudi Med 1997;17: 451-3.  Back to cited text no. 1
    
2.Zwenneke Flach H, Ginai A Z, Wolter Oosterhuis J. Maffucci Syndrome: Radiologic and pathologic findings. Radiographics 2001;21:1311-6.  Back to cited text no. 2
    
3.Murphey MD, Walker, Wilson AJ, Kransdorf MJ, Gannon FH. Imaging of primary chondrosarcoma: Radiologic-pathologic correlation. Radiographics, 2003; 23:1245-78.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]


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