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Limb lengthening in children with Russell–Silver syndrome: a comparison to other etiologies

  • Original Clinical Article
  • Published:
Journal of Children's Orthopaedics

Abstract

Introduction/background

Russell–Silver syndrome (RSS) is the combination of intrauterine growth retardation, difficulty feeding, and postnatal growth retardation. Leg length discrepancy (LLD) is one of four major diagnostic criteria of RSS and is present in most cases. We aimed to ascertain whether pediatric RSS patients will adequately consolidate bony regenerate following leg lengthening.

Materials and methods

We retrospectively reviewed pediatric RSS patients who underwent limb lengthening and compared them to a similar group of patients with LLD resulting from tumor, trauma, or congenital etiology. The primary outcome measurement was the bone healing index (BHI).

Results

The RSS group included seven lengthened segments in five patients; the comparison group included 21 segments in 19 patients. The groups had similar lengthening amounts (3.3 vs. 3.9 cm, p = 0.507). The RSS group healed significantly faster (lower BHI) than the control group (BHI 29 vs. 43 days/cm, p = 0.028). Secondary analysis showed no difference between RSS and trauma patients in terms of the BHI (29 vs. 31); however, the BHI of the RSS group was significantly lower than both of the other congenital etiologies (29 vs. 41, p = 0.032) and tumor patients (29 vs. 66, p = 0.019). The RSS patients had fewer and less significant complications than the controls.

Discussion

The limb lengthening regenerate healing of RSS patients is faster than the healing of patients with other congenital etiologies and tumor patients, and is as fast as the regenerate healing of patients with posttraumatic LLD. Although all RSS patients were treated with human growth hormone (hGH), we are unable to isolate the hGH contribution to the regenerate bone healing. We conclude that RSS patients can have safe limb lengthening.

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Acknowledgments

We would like to thank Dr. Stephen Lyman for his assistance with the statistical issues in this study. This study was not financially supported by any source.

Conflict of interest

None.

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Authors and Affiliations

  1. Limb Lengthening and Complex Reconstructions Service, Hospital for Special Surgery, Weill Cornell Medical College, 535 East 70th Street, New York, NY, 10021, USA

    V. Goldman, T. H. McCoy, A. T. Fragomen & S. R. Rozbruch

  2. Pediatric Endocrinology Service, Mount Sinai Hospital, New York, NY, USA

    M. D. Harbison

  3. Orthopedic Surgery Department, Hadassah Medical Center, Jerusalem, Israel

    V. Goldman

Authors
  1. V. Goldman
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  2. T. H. McCoy
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  3. M. D. Harbison
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  4. A. T. Fragomen
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  5. S. R. Rozbruch
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Corresponding author

Correspondence to S. R. Rozbruch.

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Goldman, V., McCoy, T.H., Harbison, M.D. et al. Limb lengthening in children with Russell–Silver syndrome: a comparison to other etiologies. J Child Orthop 7, 151–156 (2013). https://doi.org/10.1007/s11832-012-0474-3

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  • DOI: https://doi.org/10.1007/s11832-012-0474-3

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