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Limb lengthening in children with Russell-Silver syndrome: a comparison to other etiologies
V Goldman1, T H McCoy2, M D Harbison3
1Limb Lengthening and Complex Reconstructions Service, Hospital for Special Surgery, Weill Cornell Medical College, 535 East 70th Street, New York, NY 10021 USA ; Orthopedic Surgery Department, Hadassah Medical Center, Jerusalem, Israel.
Insights
Pediatric patients with Russell-Silver syndrome (RSS) demonstrate faster bone healing after leg lengthening compared to other congenital conditions. This suggests safe and effective limb lengthening is achievable for RSS patients.
Area of Science:
- Orthopedics and Regenerative Medicine
- Pediatric Endocrinology
- Skeletal Dysplasias
Background:
- Russell-Silver syndrome (RSS) is characterized by intrauterine and postnatal growth retardation, often presenting with leg length discrepancy (LLD).
- LLD is a major diagnostic criterion for RSS, necessitating evaluation of bone consolidation following limb lengthening procedures.
Purpose of the Study:
- To evaluate the bone healing capacity of pediatric patients with RSS undergoing limb lengthening.
- To compare the bone healing index (BHI) in RSS patients with a control group of pediatric patients with LLD from other etiologies.
Main Methods:
- Retrospective review of pediatric RSS patients who underwent limb lengthening.
- Comparison of bone healing index (BHI) between RSS patients and a control group (LLD due to tumor, trauma, or congenital causes).
Main Results:
- RSS patients exhibited significantly faster bone healing (lower BHI) compared to controls (29 vs. 43 days/cm).
- RSS patients showed comparable healing to trauma patients but faster healing than other congenital etiologies and tumor patients.
- RSS patients experienced fewer complications.
Conclusions:
- Limb lengthening regenerate healing in RSS patients is faster than in other congenital etiologies and tumor patients, and comparable to posttraumatic LLD.
- While human growth hormone (hGH) was used, its specific contribution to bone healing could not be isolated.
- Limb lengthening can be safely performed in pediatric RSS patients.
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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