Urokinase thrombolysis as a rescue treatment for midgut volvulus ischemia

Abstract Midgut volvulus in infants may lead to extreme short bowel syndrome. Strategies to avoid post-ischemia bowel loss have been proposed, involving thrombolysis prior to a definitive surgical treatment. Haemorrhagic risks in these patients may withhold from this approach. Herein we describe the use of urokinase systemic infusion after an unsatisfactory second look laparotomy, performed in a term baby with midgut volvulus. Continuous infusion of urokinase was given for seventeen hours prior to a third laparotomy. A total of 25 cm of small bowel were finally kept in place. Twelve months after the 1st laparotomy the patient was on oral feeding 125 calories/Kg/day. This case showed the possible rescue role of a fibrinolytic agent against persistent ischemia after the second look laparotomy for neonatal volvulus

VY anoplasty for ectropion of anal mucosa in an adult with anorectal malformation

Ectropion of anal mucosa (AME) is a possible complication following anorectal surgery and it is often cause of symptoms such as soiling, pain and bleeding affecting the patients' quality of life. Two-flaps anoplasty has been previously described for the correction of AME. We herein report a case of an adult patient who underwent a successful double V/Y-flaps for mucosal ectropion as consequence of repair of anorectal malformation (ARM).A 25 year-old woman with history of ARM presented with an extensive AME causing bleeding, pain, soiling, and dyspareunia. This condition prevented her from an effective rectal nursing and a satisfactory social life.The patient underwent the resection of ectopic mucosa and the creation of two V-shaped skin flaps to cover the skin gap. A minor dehiscence of one of the flaps was treated conservatively. At two years follow up the patient is symptoms free, has a good quality of life and can perform an effective bowel management.AME is a possible complication after anorectal surgery for ARM and the multiple V-Y plasty is a viable procedure for its treatment. A multidisciplinary approach in these cases is recommended.Level of Evidence V. Keywords: Anorectal malformation, Anoplasty, V-Y flaps, Mucosal ectropio

Robot-assisted splenectomy in a teenager with chronic autoimmune thrombocytopenia

The use of the Da Vinci Xi system is gaining popularity among all surgical disciplines. A splenectomy is a treatment option for patients with hematological disorders and splenic lesions. The laparoscopic approach is nowadays the standard of care. Despite the initial controversy, recently it has been demonstrated the superiority of robotic splenectomy performed in ''difficult'' cases. We report, to our knowledge, the first case of robot-assisted splenectomy following embolization of the splenic artery in a 15-year-old patient with chronic immune thrombocytopenia, worsened by a severe cerebral sinus thrombosis, while being treated with eltrombopag and mycophenolate. Due to the need for a rapid rise in platelet counts and failure of several medical treatments, splenectomy was advocated. To raise the platelet count pre-operatively and minimize intraoperative bleeding, the embolization of the spleen artery was performed before the planned splenectomy. The intervention was carried on without any complication and at 1 year follow up the patient is in good clinical condition and has improved his neurological condition. We propose a robotic splenectomy following embolization of the splenic artery as a feasible and safe procedure. The advantages of the Da Vinci Xi system are highlighted especially in complex cases, requiring maximum precision

Multidisciplinary expertise in the diagnosis of cecal lymphoma

This particular case highlights the importance of a multidisciplinary team expertise in the differential diagnostic process of acute abdominal pain. The case involves a 10-year old Chinese boy who presented with intermittent abdominal pain; the clinical and radiologic picture narrowed the differential diagnosis between an acute appendicitis and an intestinal lymphoma. Diagnosis of a high grade - B lymphoma was made by performing a colonoscopy; this procedure was deemed the best option to obtain a quick diagnosis with low invasiveness

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the ureteropelvic-junction obstruction

Not available

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia

Not available

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey

Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4–1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and “unclear anatomy” example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons’ scores (interobserver variation) was also calculated using Krippendorff’s alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in “poor” and “good” quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff’s alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

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.Peer reviewe

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

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.Peer reviewe