gms | German Medical Science

Objectives: Bilateral short humerus in achondroplasia restricts function of upper extremity. We lengthen the humerus of achondroplasic patients for their difficulties about the personal care and lack of the hygiene.In our study we analysed functional and the clinical results of the humerus lengthening realised in achondroplasic patients.

Methods: 48 humerus of 24 patients with a mean age of 11 (range 30-7) are lengthened between 2001 and 2013 in bilateral fasion with unilateral external fixators. We analysed the external fixator index, DASH score, complications, bone and functional scores according to Paley et all. The mean follow up was 40 (104-12) months.

Results and Conclusion: The mean follow-up period was 40 (10 through 104) months; the mean lenghtening was 7.8 cm (6.5 through 10).The mean external fixator index was 24.1 day/cm (17.1 through 33.7) According to the Paley's complication classification 6 problems and 5 obstacles were detected. According to the Palay's functional scoring system, 30 segments was considered as very good, 18 segments as good; and according to the Paley's bone scoring system 40 segments were considered as very good and 8 segments as good. The mean DASH score was reported 42.2 pre-operatively and 9.4 post-operatively. This change was found to be statistically significant (p=0.04).

Humerus lengthening in achondroplasic patient is a safe and effective surgery with a mean of 60% (range 94-40) lengthening according to the preoperative humerus length. Great improvement in DASH score, perineal and perianal hygien; show that the dependance of achondroplasic patients to others decreases. Humerus lengthening of the achondroplasic patients is not just a cosmetic operation but also it is a functional necessity.