Background: Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims: This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods: Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results: Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion: In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.

This study revealed musculoskeletal disorders in a majority of laparoscopists. The laparoscopic technique often requires static and tiring work positions, sometimes extreme, which can explain musculoskeletal disorders among general surgeons and gynecologist.

Level II.

Hematoma and bruising (sugillation) are frequent problems after operations for primary breast cancer. In the present study we evaluated the influence of various methods of perioperative thromboembolic prophylaxis on the postoperative incidence of hematoma and suggilation. From June 1994 through August 1996, a series of 425 patients consecutively operated on for primary breast cancer were included. Thromboembolic prophylaxis was low-molecular-weight heparin (LMWH) in 310 patients and thigh-long graded compression (TED) stockings in 102 patients. Postoperative complications including deep vein thrombosis, pulmonary embolism, wound hematoma, and sugillation were recorded, and 17 variables with a potential influence on complications were analyzed by logistic regression analysis. Heparin prophylaxis compared to prophylaxis with TED stockings was significantly and independently associated with postoperative hematoma [odds ratio (OR) 3, 13; 95% confidence interval (CI) 1.38-7.13] or sugillation (OR 3.34; 95% CI 1.93-5.78). No clinically overt thromboembolic complications were diagnosed. After operations for breast cancer we found that LMWH was significantly associated with postoperative hematoma and sugillation compared to TED stockings for perioperative thromboembolic prophylaxis.

Background. Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear. Methods. Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices. Results. During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n=119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p=0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p=0.005) and with fusiform CMs (56% vs. 25%, p=0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p<0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported. Conclusions. CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.