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Scientific Articles   |    
Precocious Appearance of the Capital Femoral Ossific Nucleus in Larsen Syndrome
Adriana De La Rocha, MS1; John G. Birch, MD, FRCS(C)1; Jonathan R. Schiller, MD1
1 Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219. E-mail address for J.G. Birch: john.birch@tsrh.org
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Investigation performed at the Texas Scottish Rite Hospital for Children, Dallas, Texas



Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. One or more of the authors, or his or her institution, has had a financial relationship, in the thirty-six months prior to submission of this work, with an entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, one or more of the authors has had another relationship, or has engaged in another activity, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.

Copyright © 2012 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2012 May 02;94(9):e55 1-4. doi: 10.2106/JBJS.K.00865
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Abstract

Background: 

Larsen syndrome is associated with multiple complications, including spinal deformities and recalcitrant joint dislocations. We noted capital femoral ossific nuclei on ultrasonographic images that were made for two infants with Larsen syndrome who were less than two weeks of age. We sought to confirm that this finding is common in patients with Larsen syndrome and unusual in patients with normal hips or idiopathic developmental dysplasia of the hip.

Methods: 

We identified eight patients with Larsen syndrome who had undergone ultrasonographic or radiographic evaluation of the hips before the age of three months. We compared the findings for these eight patients with those for forty consecutive patients from a prospective study of infants with developmental dysplasia of the hip who had ultrasonographic evaluation of the hips at approximately three months of age, including twenty patients who had normal clinical and ultrasonographic findings and twenty who had clinical instability of one or both hips.

Results: 

All eight patients with Larsen syndrome had radiographic or ultrasonographic evidence of an ossific nucleus at an average age of forty days (range, six to 115 days); four of these patients had evidence of an ossific nucleus at six to ten days of age. In comparison, only two of twenty normal infants (three of forty hips) and one of twenty infants (two of forty hips) with developmental dysplasia of the hip had ultrasonographic evidence of an ossific nucleus at an average age of eighty-four days (range, seventy-six to ninety-four days) (p < 0.0001, Fisher exact test).

Conclusions: 

In this small group of patients with clinically documented Larsen syndrome, the capital femoral ossific nucleus was evident on ultrasonography of the hip as early as six days of age and was unusual in normal patients or those with idiopathic developmental dysplasia of the hip. The detection of precocious development of a capital femoral ossific nucleus in infants being screened for skeletal anomalies may warrant further evaluation for the possibility of the presence of Larsen syndrome.

Level of Evidence: 

Diagnostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

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    References

    Accreditation Statement
    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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