Surgical Technique and Outcomes for Bilateral Humeral Lengthening for Achondroplasia: 26-year Experience

Background: Elongation in patients with achondroplasia provides better overall skeletal proportionality and significantly improves such individuals' access to their perineal region to self-manage personal hygiene. This paper describes our surgical technique and outcomes for bilateral humeral lengthening in achondroplasia patients over 26 years.

Methods: Ours was a retrospective study of 55 patients with achondroplasia-related short stature, in whom bilateral humeral lengthening was performed from 1990 to 2016. We describe the surgical technique and analyze mean gain in humeral length, days using an external fixator, mean percentage of lengthening, external fixation index, type of callus, and complications. Pre- and postoperative radiographic measurements were obtained. Patients also were contacted by telephone and asked about their ability to perform peri-anal self-hygiene and about their overall satisfaction.

Results: In total, 110 humeri were lengthened (28 males and 27 females) with medium elongation of 9.5 cm on the right and 9.6 cm on the left, while averaging 220 days in an external fixator. We observed 14 minor complications. There was no significant association between pin position and type of callus, and elongation most often external and in the presence of a straight callus. Before elongation, 77.1% of patients reported difficulties with perineal hygiene and 85.4% could not put their hands in their pockets. Upon completion of lengthening, 100% could perform both tasks and 94.5% were very satisfied.

Conclusions: Bilateral humeral elongation yields significant improvements in patient autonomy, with a relatively low complication rate and very high patient satisfaction.