Outcome of Open Hip Reduction, Pelvic Osteotomy, and Varus Derotational Osteotomy in Children With Cerebral Palsy: A Retrospective Study.

Introduction For spastic hip dislocations, a variety of operations are available with open hip reduction and varus derotational osteotomy of the proximal femur combined with pelvic osteotomy ± adductor release being a good option with favourable outcomes. This study aims to assess the outcome and complications of combined open hip reduction with pelvic osteotomy and varus derotational osteotomy. Methods In this study, 70 hips in 52 patients with spastic hip dislocation due to cerebral palsy were included. All included patients were treated surgically in our institute between January 2016 and December 2021. There were 31 males and 21 females. For each patient, information was collected about the age at the time of surgery and different radiological parameters at three different time intervals: pre-operatively, immediately post-operatively, and at the final follow-up. We also collected information about any complications arising from the surgery performed. Results The mean duration of follow-up was 19.58 months. The acetabular index decreased from an average of 35.01° to 17.18° with a mean difference of 17.83° (p<0.001). The central edge angle, which averaged -49.13° in the pre-operative period, increased to 26.34° and then marginally decreased to 25.47° at the final follow-up. The average migration index of 80.51% in the pre-operative period improved to 1.4% post-operatively with a mean difference of -79.11% (p<0.01). The migration index increased to 8.54% at the final follow-up. Similarly, the neck-shaft angle, which averaged 160.89° in the pre-operative period, decreased to 125.23° at the time of final follow-up with a percentage change of -22.16%. Conclusion Single-stage combined surgery in the form of combined open hip reduction and pelvic osteotomy with varus derotational osteotomy successfully treats the condition and shows good outcomes in patients with spastic hip dislocations. This treatment is associated with very few complications.

Young Patients' Satisfaction Following the Correction of Adolescent Idiopathic Scoliosis in Saudi Arabia: A Cross-Sectional Study.

INTRODUCTION: Posterior spinal instrumented fusion remains the mainstay treatment for adolescent idiopathic scoliosis (AIS) with acceptable post-operative patient satisfaction. However, in Saudi Arabia, patient satisfaction after surgical management for AIS has not been thoroughly studied. The purpose of this study was to determine patient satisfaction and quality of life using the Scoliosis Research Society-22r (SRS-22r, the most recent version) questionnaire after surgical correction of AIS in Saudi Arabia. METHODS: A retrospective study was conducted that included patients who underwent posterior spinal instrumented fusion for AIS from January 1995 through December 2015. We included 115 patients (both males and females) in our study. We used the Arabic version of the SRS-22r questionnaire that was completed through telephonic interviews. Data collected were then analyzed using SPSS Statistics, version 23. RESULTS: The mean age of our patients at the time of surgery was 15.0 ± 2.6 years and the average time from surgery to interview was 9.4 ± 4.7 years. A positive response was recorded in all domains including pain, function, mental health, and self-image. Furthermore, 76.5% of the patients were satisfied with their management outcome and 81.7% of the patients reported no complications. CONCLUSION: Surgical correction of AIS improved the quality of life of our patients that was assessed using the Arabic version of the SRS-22r questionnaire. Apart from overall patient satisfaction, positive responses were recorded in all four domains of the SRS-22r questionnaire.

Study of autosomal recessive osteogenesis imperfecta in Arabia reveals a novel locus defined by TMEM38B mutation.

BACKGROUND: Osteogenesis imperfecta (OI) is an hereditary bone disease in which increased bone fragility leads to frequent fractures and other complications, usually in an autosomal dominant fashion. An expanding list of genes that encode proteins related to collagen metabolism are now recognised as important causes of autosomal recessive (AR) OI. Our aim was to study the contribution of known genes to AR OI in order to identify novel loci in mutation-negative cases. METHODS: We enrolled multiplex consanguineous families and simplex cases (also consanguineous) in which mutations in COL1A1 and COL1A2 had been excluded. We used autozygome guided mutation analysis of AR OI (AR OI) genes followed by exome sequencing when such analysis failed to identify the causative mutation. RESULTS: Two simplex and 11 multiplex families were enrolled, encompassing 27 cases. In three multiplex families, autozygosity and linkage analysis revealed a novel recessive OI locus on chromosome 9q31.1-31.3, and a novel truncating deletion of exon 4 of TMEM38B was identified within that interval. In addition, gonadal or gonadal/somatic mosaic mutations in COL1A1 or COL1A2 and homozygous mutations in recently described AR OI genes were identified in all remaining families. CONCLUSIONS: TMEM38B is a novel candidate gene for AR OI. Future studies are needed to explore fully the contribution of this gene to AR OI in other populations.