ABSTRACT
Introduction: Hirschsprung's Disease (HD) is a motor disorder of the gut caused by the failure of neural crest cells to migrate craniocaudally into the bowel during intestinal development, resulting in a functional obstruction. The majority of patients with HD are diagnosed in the neonatal period when they present with symptoms of distal intestinal obstruction. Aim: This study aims to identify the clinic-pathological characteristic of HD patients in our institution in KSA and comparing it with local and international data. Materials and Methods: This retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC), a tertiary care center in Riyadh, Kingdome of Saudi Arabia (KSA). Results: A total of 54 patients (72% male) were diagnosed with HD. Forty-eight patients (89%) were born at term, and 6 were pre-term. Sixty-three percent of the patients presented in the neonatal period. Twenty-two patients (41%) underwent one-stage endorectal pull-through procedure, 23 patients (43%) two-stage endorectal pull-through, and 9 patients (16%) had three-stage endorectal pull-through. Five out of 54 patients had ganglion cells seen on FS but were absent in the permanent section. Therefore, the concordance rate was 90.8%. Conclusion: FS biopsy is a necessary method to determine the level of aganglionosis intraoperatively in HD, but the definitive diagnosis should be with permanent section. Also, the choice of surgical operation type (single-stage or multi-stage pull-through) depends on the patient's clinical condition.
ABSTRACT
BACKGROUND: Developmental dysplasia of the hip (DDH) is one of the prevalent musculoskeletal conditions in young adults and is a leading cause of hip osteoarthrosis in this age group. The optimum age for surgical intervention when needed is not well established in the literature and the lack of management guidelines and standard practice of DDH leads to different practices worldwide. This study aims to investigate the current practice of paediatric orthopaedic surgeons in diagnosing and treating DDH worldwide and identify points of agreement and disagreement. METHODS: A cross-sectional study utilizing an online questionnaire was designed to examine the different points of view and current practice of paediatric orthopaedic surgeons worldwide regarding DDH diagnosis and treatment. RESULTS: Ninety-one surgeons responded, with an overall response of 45.5%. The vast majority of respondents use ultrasonography in children less than 3 months of age and pelvic radiography in over 3 months to diagnose DDH. Pavlik harness is the most popular DDH treatment for children younger than 6 months. For older children, closed reduction with hip spica cast is the most preferable treatment. The maximum duration of first-line treatment has a broad range. The treatment of bilateral DDH varies widely among surgeons. CONCLUSION: This study shows clearly that paediatric orthopaedic surgeons do not agree on the diagnosis and treatment protocol of DDH, hence different approaches to this common disease are practised. The lack of an international guideline should motivate paediatric orthopaedic surgeons to discuss and formulate a uniform and evidence-based protocol for the diagnosis and treatment of DDH.
