Background: There are surgical and non-surgical treatment options available to create a neo-vagina for women who have congenital vaginal agenesis or acquired vaginal stenosis. We report our success of vaginal dilator therapy in all suitable aetiologies for women who presented to our facility between January 2005 and June 2015. The therapy was deemed successful if the women were able to have sexual intercourse without pain or if they achieved a vaginal length of greater than 6cm and maximum width using the largest of the dilators throughout the vagina if they were not sexually active. Methods: All thirty-one women who presented to this service during the ten and half year period were included in this retrospective clinical audit, where the intervention was the use of Amielle comfort vaginal dilators. Of the presenting diagnoses, 17 had Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH), 2 had cloacal abnormalities, 1 had vaginal narrowing from previous neo-vagina creation surgery as a child, 2 had complete vaginal agenesis, 3 had partial vaginal agenesis, 1 had complete androgen insensitivity (CAIS), 1 had partial androgen insensitivity (PAIS), 1 had 5a reductase deficiency, 1 had hypogonadotrophic hypogonadism, 2 had congenital adrenal hyperplasia (CAH). Results: Out of these 31 women, 8 had to be excluded as they had not started the dilator therapy for reasons varying between being too young to need a vagina in the immediate period, or not being either psychologically or emotionally and physically ready to embark on what was expected to be an intense commitment. Of the remaining 23 women, 18 are satisfied with the results of their dilator therapy, 3 are having ongoing dilator therapy with good progress and 2 are unsatisfied with the results of their dilator therapy and are being considered for multidisciplinary surgery. These two latter patients, one was a patient with Disorder of sexual Differentiation, and is physically not entirely suited to total success with vaginal dilator therapy alone. The other patient had severe psychological barriers to using the dilator therapy despite extensive counselling by the treating specialists. Conclusion: Vaginal dilator therapy is an effective first line treatment for neo-vagina creation, with approximately 90% success to date in our cohort.

IntroductionRecently the EOS imaging system (EOS Imaging, Paris, France) has provided advancements in 3D spinal modeling. Advancements include low radiation as well as fast and accurate reconstructed measurements of spinal parameters. There is a paucity of studies analyzing the reproducibility of the EOS Imaging System and the sterEOS software in the production of 3D spinal models for children with adolescent idiopathic scoliosis (AIS). Objectives The purposes of the study were 1) to determine the intraclass correlation (ICC) for both the inter-observer and intra-observer in the measurements of Cobb angles in AP view as well as the Cobb angles in the lateral view; 2) to assess the ICC for inter-and intra-observer in the axial vertebral rotation (AVR) of the apex vertebra; 3) to compare differences of spinal parameters between two examiners and two trials; 4) to determine how long a 3D reconstruction of the spine takes. Methods Bilateral x-ray images of fifteen patients (age: 6 -15 years old, 5 males, 10 females) were retrospectively selected. These EOS images were uploaded into the sterEOS computer program. Within the software, spinal and pelvic parameters were identified manually to construct a 3D model of the spine. The sterEOS software calculates the Cobb angles, angles of lordosis, angles of kyphosis, and the AVRs of the apex vertebra. The 3D modeling was performed independently by two examiners. Each examiner modeled each patient's spine in two spaced out trials. The ICC between inter-and intra-observers were calculated and compared statistically. Results and discussionBoth the inter-and intra-observers showed excellent reproducibility for the Cobb angles in the proximal segment (ICC: 0.72 -0.91), kyphosis (ICC: 0.85-0.92), and lordosis (ICC: 0.82 -0.95). No significant differences were found between angle differences (0.35°to 2.4°). In contrast to the traditional radiography, the sterEOS provides a better high quality view within the sagittal plane. A moderate inter-observer ICC for the Cobb angle in the distal segment (ICC = 0.67) indicates the examiners have to carefully adjust the alignment and vertebrae in 3D rather than in 2D following the automatic computation from the EOS software. The interobserver ICC for the AVR in the lumbar region (0.80) is higher than the thoracic or thoracolumbar region (0.65), but with high differences of AVR (4.0°-6.3°). The average time that two examiners spent per subject ranged from 34.6 to 37.4 minutes. Conclusion and significance EOS provides significantly reliable and accurate spinal modeling in the measurement of children with AIS. Exposure to less radiation as compared to other radiographic modality allows EOS to offer acceptable quality view of the spine in the sagittal and transversal plane. sagittal balance and predictive equations to determine lumbopelvic compensatory patterns (LPCP). These equations are used to guide surgical decision making and technique selection. Although other lumbopelvic compensation equations are available, these have not been compared wi...

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Purpose: Spinal muscular atrophy (SMA) is a genetic disease resulting in orthopaedic problems that warrant intervention. Recent genetic therapies appear to improve the natural course of this disease, prompting care providers to rethink treatment strategies. Insufficient high-quality evidence exists to guide optimal intervention in this new landscape. To address this, the European Neuromuscular Centre (ENMC) tasked an orthopaedic subgroup to update previous recommendations. Materials and Methods: A consensus statement on orthopaedic treatment of patients with SMA undergoing genetic therapy was sought via Delphi method. After review of the orthopaedic literature, three iterative surveys were administered from 2015-2016 to worldwide experts in orthopaedic care of children with SMA. Surveys identified important topics for optimal management, solicited responses regarding management and evaluation of identified topics, and clarified decision-making strategies and indications for evaluation and management, respectively. In light of new evidence of the long-term effects of gene therapy on SMA patients, an additional review of literature was used to contextualize the consensus recommendations. Results: Thirteen experts on orthopaedic management of SMA identified treatment of spine deformity, hip instability, and joint contractures as issues of importance. Experts recommended instrumentation in spinal deformity for major curves greater than 50 degrees, while monitoring pain, respiratory function, kyphosis, pelvic obliquity, functional status, trunk imbalance, and rib deformity. Preferential use of magnetically controlled growth constructs and performance of final fusion with skipped levels to accommodate intrathecal access were also endorsed. Additionally, experts advocated for surgical correction of hip instability and/or contractures that cause pain or functional impairment. Conclusions: Practice guidelines for management of scoliosis, hip instability and joint contractures were created to aid clinical decision-making in the era of genetic therapy.

Introduction: Lesser tuberosity osteotomy (LTO) is an attractive option for subscapularis management during anatomic shoulder arthroplasty due to the biomechanical strength and reliable bone-to-bone healing. Patients with humeral head avascular necrosis (AVN) may have compromised bone healing, and the outcomes of LTO during AVN are unknown. Methods: A retrospective consecutive case series of 6 patients with Cruess grade 4 or 5 humeral head AVN who underwent anatomic shoulder arthroplasty with LTO from 2010 to 2016 was performed. Postoperative radiographic evaluation for LTO healing at 6 months was analyzed, and clinical outcomes at latest follow-up, including range of motion (ROM), strength, and pain were studied.> Results: Average age was 50.3 years. AVN was secondary to sickle cell in 1 patient, steroid use for systemic lupus erythematosus in 4, and chronic alcoholism in 1. By 6 months after arthroplasty, 100% had radiographically united and healed LTO. Patients averaged 140 AE 21 of active forward elevation and 42 AE 7 of active external rotation. Patients reported an improvement in visual analogue scale pain from 8.3 preoperatively to 3.8 postoperatively. All patients had a normal abdominal compression test. No patients required revision surgery. Conclusion: The use of LTO during anatomic shoulder arthroplasty for AVN has an excellent bony healing rate with improvements in pain, ROM, and strength. The diseases that cause humeral head AVN do not negatively influence LTO healing outcomes during anatomic shoulder replacement. Level of Evidence: IV Case Series