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.

Psychosocial effects of repetitive surgeries in children with early-onset scoliosis: are we putting them at risk?

BACKGROUND: The use of growing instrumentation in children with early-onset scoliosis (EOS) has created interest in determining if these repetitive procedures are prompting the development of lasting psychosocial problems. Given the increasing role of this treatment modality in the management of EOS, this study aimed to assess the psychological status of this patient population and to determine factors associated with worse scores in various psychosocial domains. METHODS: A cross-sectional assessment of 34 EOS patients was performed utilizing 2 well-established, caregiver-completed psychiatric instruments: the Child Behavior Checklist (CBCL) and the Strength and Difficulties Questionnaire. Scores were calculated for 15 CBCL and 6 Strength and Difficulties Questionnaire domains and subdomains and grouped as "Normal" or "Abnormal" according to published normative values. The prevalence of abnormal scores was within each instrument subdomain and was compared with the national norms. Domain scores were also correlated with age at first scoliosis surgery, total number of operative procedures, and total number of growing instrumentation surgeries. RESULTS: A higher prevalence of Abnormal scores were found in multiple psychosocial domains in our cohort as compared with national normative data. Children with Abnormal CBCL "Total Problems" domain scores were younger at the time of first scoliosis surgery (2.50 vs. 5.52 y). Normal and Abnormal scoring groups showed significant differences in the number of (1) total surgeries; (2) total scoliosis surgeries; and (3) growing instrumentation surgeries in multiple domains on both instruments. Aggression, Rule-breaking, and Conduct were positively correlated with total number of surgeries. CONCLUSIONS: Our findings showed a higher prevalence of Abnormal psychosocial scores in multiple domains in multioperated EOS patients as compared with national norms. Our findings suggest that EOS patients with abnormal psychosocial scores were younger at the time of their initial scoliosis surgery. The number of repetitive surgeries also correlated positively with 3 behavioral problem scores. Although healthier scores were seen in 1 positive behavioral domain in more operated children suggesting the potential for "posttraumatic growth," the other findings of this study are concerning given the increasing use of this treatment modality and indicate a need for ongoing screening and mental health care in this high-risk population. LEVEL OF EVIDENCE: Level III--case-control.

Influence of the social context on use of surgical-lengthening and group-empowering coping strategies among people with dwarfism.

OBJECTIVE: To assess the role that social contextual factors exert on the way people with disproportionate short stature (dwarfism) cope with the negative consequences of discrimination. METHOD: Using multigroup structural equation modeling, we compare the coping process of people with dwarfism from Spain (N = 63) and the USA (N = 145), two countries that differ in the role played by organizations offering support to people with dwarfism. RESULTS: In Spain, where organizational support is recent, a coping approach aimed at achieving integration with the majority group through limb-lengthening surgery prevails; in the USA, where the long-standing organization of people with dwarfism encourages pride in being a "little person" and positive intragroup contact, a coping strategy based on empowering the minority group dominates. CONCLUSIONS: Both strategies, each in its own context, are effective at protecting psychological well-being from the negative consequences of stigmatization; however, they exert their positive effects through different processes.

Distraction osteogenesis in congenital limb length discrepancy: a review.

This paper reviews the technique of callotasis used to correct limb length discrepancy due to congenital causes. Many more lower than upper limb lengthening procedures have been reported. Despite the low complication rate reported by the originators of the technique, patients undergoing limb lengthening because of congenital problems are at significant risk of pin tract sepsis, joint contracture, nerve palsies, angular deformities and fracture through the regenerate bone. A minority of studies focus specifically on limb lengthening for congenital defects. Most series simply include some congenital patients, but it is difficult to retrieve the data, and to generalize from them. The amount of lengthening with an acceptable complication rate should not exceed 25%; of the initial bone length. Even using circular frames with small pins, practically all patients may be expected to develop at least one complication each, ranging from pin tract infection to the necessity of carrying out additional unplanned operative procedures either during or after the treatment period. The prevalence of major complications seems to be correlated with the complexity and the duration of the treatment. The functional outcome and the psychological problems associated with a lengthy procedure, which may require long periods of repeated hospitalization, have only rarely been studied. However, it appears that prolonged strength loss is frequent, and that significant psychological morbidity is experienced. Patients and their families should be counselled before and during the procedure on these lesser known aspects of callotasis lengthening.

Leg lengthening: patient selection and management in achondroplasia.

Current concepts in limb lengthening in short stature are discussed together with management protocol and a review of 32 patients undergoing surgery using the three surgical techniques of lengthening. Limb lengthening in achondroplasia requires careful planning. Decisions must be reached regarding the suitability of the patient, timing of surgery, and the amount of lengthening.

Operative lengthening of the lower extremity and associated psychological aspects: the Children's Hospital experience.

Twenty-two patients underwent 23 femoral and tibial lengthenings by the Wagner technique from 1977 to 1987. Average length gained was 5.8 and 5.2 cm, respectively. Long hospitalization in addition to multiple operative procedures and a high complication rate created a significant psychological impact. Fourteen of 22 patients experienced psychological problems, primarily adjustment disorders, all of which resolved without long-term sequelae. Parental/family and nursing staff support were identified as the most important during the lengthening process. Advances in limb lengthening techniques may lessen the overall impact of this procedure, but preoperative psychological preparation is important.

Psychologic, vascular, and physiologic aspects of lower limb lengthening in achondroplastics.

Achondroplastic patients having lengthening surgery of the lower limbs were preoperatively evaluated from psychologic, functional, vascular, and endocrinometabolic points of view. Long-term research is required to determine effects of surgical correction of achondroplasia from both psychologic and various physical points of view.