Inconvenience and adaptation in Japanese adult achondroplasia and hypochondroplasia: A cross-sectional study.

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Abstract

Conflict of interest statement: The authors declare no conflicts of interest in connection with the submitted article. 2. Indian J Orthop. 2016 Jul-Aug;50(4):397-405. doi: 10.4103/0019-5413.185604. Limb lengthening in achondroplasia. Chilbule SK(1), Dutt V(1), Madhuri V(1). Author information: (1)Pediatric Orthopedics Unit, Christian Medical College, Vellore, Tamil Nadu, India. BACKGROUND: Stature lengthening in skeletal dysplasia is a contentious issue. Specific guidelines regarding the age and sequence of surgery, methods and extent of lengthening at each stage are not uniform around the world. Despite the need for multiple surgeries, with their attendant complications, parents demanding stature lengthening are not rare, due to the social bias and psychological effects experienced by these patients. This study describes the outcome and complications of extensive stature lengthening performed at our center. MATERIALS AND METHODS: Eight achondroplasic and one hypochondroplasic patient underwent bilateral transverse lengthening for tibiae, humeri and femora. Tibia lengthening was carried out using a ring fixator and bifocal corticotomy, while a monolateral pediatric limb reconstruction system with unifocal corticotomy was used for the femur and humerus. Lengthening of each bone segment, height gain, healing index and complications were assessed. Subgroup analysis was carried out to assess the effect of age and bone segment on the healing index. RESULTS: Nine patients aged five to 25 years (mean age 10.2 years) underwent limb lengthening procedures for 18 tibiae, 10 femora and 8 humeri. Four patients underwent bilateral lengthening of all three segments. The mean length gain for the tibia, femur and humerus was 15.4 cm (100.7%), 9.9 cm (52.8%) and 9.6 cm (77.9%), respectively. Healing index was 25.7, 25.6 and 20.6 days/cm, respectively, for the tibia, femur and humerus. An average of 33.3% height gain was attained. Lengthening of both tibia and femur added to projected height achieved as the 3(rd) percentile of standard height in three out of four patients. In all, 33 complications were encountered (0.9 complications per segment). Healing index was not affected by age or bone segment. CONCLUSION: Extensive limb lengthening (more than 50% over initial length) carries significant risk and should be undertaken only after due consideration. DOI: 10.4103/0019-5413.185604 PMCID: PMC4964773