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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.
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Monosomy 1p36 deletion is a rare syndrome that consists of developmental delay, intellectual disability, seizures, hearing and vision defects, brain anomalies, orofacial clefting, congenital heart defects, cardiomyopathy, renal anomalies, and scoliosis. We report the case of an eight-year-old boy who presented to the orthopedic clinic with spinal deformity with a background of 1p36 deletion syndrome. The treatment modalities at this age include growing rods, vertical expandable prosthetic titanium rib (VEPTR), or posterior spinal fusion. Keeping in view the challenges in this case due to multi-organ involvement and severe intellectual disability, we decided to manage this patient with a VEPTR device to prevent the progression of scoliosis and allow spinal growth. Vertical expandable prosthetic titanium rib (VEPTR) instrumentation for progressive scoliosis in p36 deletion syndrome is an effective mode of treatment and leads to favorable outcomes.
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Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) association is a rare syndrome. This unique condition consists of Müllerian duct aplasia, cervicothoracic somite dysplasia, and renal aplasia, and skeletal abnormalities manifesting in childhood. We report the case of a 14-year-old girl who presented to the orthopedic clinic with spinal deformity and Sprengel's shoulder complicated by a background of MURCS association. The treatment modalities of scoliosis include posterior spinal fusion and the vertical expandable prosthetic titanium rib. On the other hand, Sprengel's deformity is surgically managed by Woodward's procedure. The management plan for our patient involved correcting scoliosis by the posterior spinal fusion procedure and performing Woodward's procedure to correct Sprengel's deformity simultaneously. Simultaneous scoliosis correction with posterior spinal instrumented fusion and Sprengel's deformity correction with modified Woodward's procedure is a promising surgical technique that can lead to favorable outcomes.
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Introduction For Sprengel deformity, a variety of operations are available, with Woodward's procedure being a favorable option with good outcomes. This study aims to assess the outcomes of Woodward's procedure with brachial plexus monitoring to prevent the possible complications of nerve injury and consequent deficits. Methods In our study, we included 18 patients with Sprengel deformity treated with Woodward's procedure using intraoperative neuromonitoring for the brachial plexus from 2013 to 2019 at our institute. For each patient, we collected information about age, gender, follow-up duration, affected shoulder side, and presence of an omovertebral bar. Also, preoperative and postoperative degrees of shoulder abduction, Cavendish grade of cosmetic appearance, Rigaults grade, and difference in scapular elevation along with postoperative complications were all measured to evaluate the outcomes. Results The mean duration of follow-up was 12 months. The average preoperative Cavendish grade was 3.1, which decreased to 1.3 on the final follow-up. The average preoperative Rigault grade was 2.5, which has decreased to an average of 1.8. The average increase in the degree of shoulder abduction postoperatively was 48.3°. The average preoperative difference in scapular height (mm) was 26.9, which decreased to an average of 12.2. Furthermore, the final outcome was not impacted by the absence or the existence of the omovertebral bar. Conclusion Woodward's procedure using intraoperative neuromonitoring without clavicle osteotomy for Sprengel's deformity successfully corrects the deformity and decreases the risk of iatrogenic brachial plexus injury.
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INTRODUCTION: In congenital anomalies of the thoracic spine, fusion in situ and hemi-epiphysiodesis are unsuitable surgical options, because three-dimensional thoracic deformity and insufficiency are uncorrectable. We aimed to evaluate the radiological outcome of vertical expandable titanium rib (VEPTR) application after follow-up in children with congenital scoliosis with or without rib fusion. METHODS: In our study, we included 58 patients with congenital scoliosis with or without fused ribs; all treated with VEPTR from 2005 to 2015 at our institute. There were 19 males and 39 females. For each patient, we collected information about age at the index surgery (VEPTR application) and the total number of VEPTR lengthening procedures. Also, Cobb angle, kyphotic angle, thoracic height, and spinal height were measured on preoperative radiographs, immediately post-operative, two years post-operative, and at final follow-up. RESULTS: The mean duration of follow-up was five years (range, 2-12 years). Twenty-eight patients had rib-to-pelvis type VEPTR, 20 patients had rib-to-rib type VEPTR, and 10 patients had a rib to pedicle/lamina type of VEPTR implant. Post-VEPTR, 63.8% of our patients reported one or more complications. The immediate post-VEPTR application showed that the mean Cobb angle decreased to 43.56° with a percentage change of 22.8% (p<0.001). The mean increase in thoracic height between VEPTR application surgery and final follow-up was 32 mm with a 19.3% increase (p<0.001). Similarly, the mean increase in the spinal height between the VEPTR application surgery and final follow-up was 46.6 mm, with a 23% increase (p<0.001). CONCLUSIONS: VEPTR instrumentation for congenital scoliosis, with or without rib fusion, successfully corrects the coronal Cobb angle in the majority of patients. It also allows the thoracic (T1-T12) and spinal (T1-S1) growth to approach normal for a particular age.