Limb lengthening in Turner syndrome

Area of Science:

• Orthopedics
• Genetics
• Pediatric Endocrinology

Background:

• Turner syndrome is a genetic condition often associated with disproportionate short stature.
• Limb lengthening surgery is a potential treatment option for short stature.

Purpose of the Study:

• To evaluate the outcomes and complications of bilateral tibial lengthening in patients with Turner syndrome.
• To assess the efficacy and risks of distraction osteogenesis for treating short stature in this population.

Main Methods:

• Retrospective review of eight consecutive patients with Turner syndrome undergoing bilateral tibial lengthening.
• Distraction osteogenesis using monolateral external fixation was the primary surgical technique.
• Data collected included lengthening achieved, treatment duration, and complication rates.

Main Results:

• Average tibial lengthening was 9.2 cm (33% of original length) over a mean of 268 days.
• A high overall complication rate of 169% per tibia was observed, with an average of 1.7 additional procedures per segment.
• Common complications included Achilles tendon contractures (44%), angulation requiring osteotomy (56%), and distraction site nonunion (12.5%).

Conclusions:

• Tibial lengthening using distraction osteogenesis is technically feasible for improving height in Turner syndrome.
• The significant complication rate raises concerns about whether the cosmetic height increase justifies the risks and extensive treatment.
• Further investigation into the psychosocial and functional benefits is crucial for determining the true cost-benefit ratio of this procedure.