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Year : 2009  |  Volume : 19  |  Issue : 2  |  Page : 158-159
Images - Extra (too many) carpal bones in Larsen's syndrome


1 Department of Radiology, Sahyadri Speciality Hospital, Plot No. 30-C, Erandawane, Pune - 411 004, Maharashtra, India
2 Department of Orthopaedics, Sahyadri Speciality Hospital, Plot No. 30-C, Erandawane, Pune - 411 004, Maharashtra, India

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   Abstract 

Multiple carpal bones may be seen in different syndromes, especially Larsen's syndrome. A case of Larsen's syndrome with many typical features and "too many" carpal bones, is described.

Keywords: Larsen Syndrome, extra carpal bones

How to cite this article:
Rahalkar MD, Rahalkar AM, Patwardhan SA. Images - Extra (too many) carpal bones in Larsen's syndrome. Indian J Radiol Imaging 2009;19:158-9

How to cite this URL:
Rahalkar MD, Rahalkar AM, Patwardhan SA. Images - Extra (too many) carpal bones in Larsen's syndrome. Indian J Radiol Imaging [serial online] 2009 [cited 2020 Aug 4];19:158-9. Available from: http://www.ijri.org/text.asp?2009/19/2/158/50837
An 11-year-old girl was seen by an orthopedic surgeon for treatment of knee deformities. She had kyphoscoliosis, contractures, genu valgus, hypermobility of the ankles, frontal bossing, hypertelorism, and a flat bridge of the nose. She was a dwarf. Her brother and a male cousin had similar clinical features.

Apart from other radiographs, a frontal radiograph of both hands was also obtained [Figure 1A],[Figure 1B],[Figure 1C],[Figure 1D], which showed multiple (extra) carpal bones-15 on the left and 13 on the right-with abnormal lie. Additionally, negative ulnar variance was seen.


   Discussion Top


Extra (accessory or supernumerary ossicles) bones of the wrist have been described in syndromes such as brachydactyly A1, ulnar dimelia, hand-foot-uterus syndrome, Holt-Oram syndrome, oto-palato-digital syndrome, chondroectodermal dysplasia, diastrophic dysplasia, Gorlin's syndrome, and Larsen's syndrome. [1] As many as 20 to 25 carpal bones have been described in some cases. The other features, as described above, were typical of Larsen's syndrome.

Arthrogryposis is a descriptive term used to describe conditions with deformities due to contractures and dislocations and includes Larsen's syndrome.[2] The changes on a hand radiograph in Larsen's syndrome are typical. These include a broad or bifid thumb, polydactyly, radio-ulnar synostosis, short metacarpals, long proximal phalanges, short distal phalanges with enlarged epiphyses, and extra carpal bones. [3],[4],[5] The number of carpal bones present may vary. The patient in the present case showed 15 carpal bones on the left and 13 on the right.

In this case, the presence of extra carpal bones was a nonspecific finding, but when combined with the other characteristic features, it was suggestive of Larsen's syndrome.

 
   References Top

1.Scanderbeg AC, Dallapicola B. Abnormal skeletal phenotypes: From simple signs to complex diagnoses. Springer Publication: 2005. p. 432-3.  Back to cited text no. 1    
2.Houstan CS, Reed MH, Desautels JE. Separating Larsen's syndrome from the "arthrogryposis basket". J Can Assoc Radiol 1981;32:206-14.  Back to cited text no. 2    
3.Latta RJ, Graham CB, Aase J, Scham SM, Smith DW. Larsen's syndrome: A skeletal dysplasia with mutliple joint dislocations and unusual facies. J Pediatr 1971;78:291-8.  Back to cited text no. 3    
4.Steel HS, Kohl EJ. Multiple congenital dislocations associated with other skeletal anomalies (Larsen's syndrome) in three siblings. J Bone Joint Surg Am 1972;54:75-82.  Back to cited text no. 4    
5.Shah HR, Patwa PC, Tannk AV, Jain P, Sadhu N, Desai NR. Radiological quiz: Musculoskeletal. Indian J Radiol Imaging 2000;10:183-4.  Back to cited text no. 5    Medknow Journal

Top
Correspondence Address:
Mukund D Rahalkar
Department of Radiology, Sahyadri Speciality Hospital, Plot No. 30-C, Erandawane, Pune - 411 004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.50837

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    Figures

  [Figure 1A], [Figure 1B], [Figure 1C], [Figure 1D]

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[Pubmed]



 

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