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This study aimed at evaluating how transition of care is currently being organized in the European Reference Networks (ERNs) health care providers (HCPs) in pediatric areas and in the Anorectal Malformation Network (ARM-Net) Consortium hospitals. An online questionnaire was sent to a total of 80 surgeons, members of or affiliated members of three networks: ARM-Net Consortium, ERN eUROGEN, and ERN ERNICA. Complete information were obtained for 45 HCPs, most of which deal with transition and still see a few adult patients (ca. 10%). Gynecological, gastroenterological, urological, colorectal, and continence issues were the major problems described by adult patients to their physicians, and in line with these prevalent complaints, they are referred to the appropriate adult specialists. Forty percent of patients complain about sexual and fertility problems, but the percentage of andrologists and sexologists involved in the caring of adult patients with ARM/Hirschsprung's disease is low, just above 10.9%. Most hospitals deal with transition, but three basic criteria (i.e., presence of: [1] an official written transitional program, [2] a transitional coordinator, and [3] written information on transition to be handled to patients) are jointly met only by six HCPs. According to the responders, the most important issue requiring improvement is the lack of interest and of specific preparation by adult specialists. The overall results of this exploratory survey confirm the need for the development of comprehensive programs for transition in these rare and complex diseases, and identify the hospitals that, in collaboration with the networks, could share best practices in organizing structured transitional pathways and well follow-ups. [ABSTRACT FROM AUTHOR]

This article discusses the importance of standardized data collection in pediatric surgery. With advancements in technology, data collection has become easier, but healthcare professionals are facing increasing administrative burdens. Standardizing data sets and centralizing data collection can maximize the usability of data for research and quality control purposes. The article highlights the need for measurement standardization in research and clinical practice, the development of core outcome sets, the importance of standardized complication registration, the use of patient-reported outcome measures, the standardization of care, and the value of collective data collection through patient registries. The European Reference Network for Inherited and Congenital Anomalies is implementing the European Pediatric Surgical Audit as its official patient registry to reduce administrative burden and maximize data usability. [Extracted from the article]

Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%).Diagnosis: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77%), acetylcholinesterase (74%), calretinin (31%), S100 (2%), nicotinamide adenine dinucleotide-tetrazolium reductase (2%), succinate dehydrogenase (1%), and neuron-specific enolase (1%). A total of 85% respondents recognize entities including hypoganglionosis (69%), intestinal neuronal dysplasia (55%), and ultrashort segment HD (50%).Surgery: Pull-through (PT) is performed at diagnosis by 33% or delayed by 67% (4 months or > 5 kg). Awaiting definitive surgery, 77% perform rectal irrigations, 22% rectal dilatation/stimulations, and 33% perform a stoma. The preferred type of PT is the Soave approach (65%), performed with transanal technique by 70% respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76%; botulinum toxin = 27%), 30% would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4%), at 1 to 6 months (29%), 6 to 12 months (37%) or older (30%). If required, a stoma is sited in the ileum (31%), according to intraoperative biopsies (54%), macroscopic impression (13%), and radiology (2%). Duhamel PT is performed by 52%, Soave by 31%, and Swenson by 17%. Overall, 31% would perform a J-pouch. Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies. [ABSTRACT FROM AUTHOR]

Aim: This study prospectively evaluated neuropsychological functioning in 8-year-old patients with anorectal malformation (ARM) and Hirschsprung's disease (HD).Methods: School functioning and behaviour were assessed in a standardised interview. Intelligence, attention, self-esteem and quality of life were evaluated with validated tests and questionnaires. The following predictors were assessed: socio-economic status, number of episodes of general anaesthesia, laxative treatment and premature birth. Severely intellectually disabled patients were excluded.Results: In total, twelve of the 23 (52%) patients with ARM and 11 (55%) of the 20 patients with HD received special education or remedial teaching. The intelligence quotient was normal: mean (standard deviation or SD) was 98 (17) and 96 (17), respectively. However, sustained attention was below the norm: mean (SD) Z-score was -1.90 (1.94) and -1.43 (1.98) for ARM and HD patients; both p < 0.01. Self-esteem was normal: mean (SD) Z-score was 0.10 (1.29) and -0.20 (1.11) for ARM and HD patients. Quality of life was normal in ARM patients and slightly impaired in HD patients. No predictors for neuropsychological outcome were identified.Conclusion: Despite normal intelligence, more than half of these patients received special education or remedial teaching. In addition, problems with sustained attention were found. These findings are important for long-term care. [ABSTRACT FROM AUTHOR]

Introduction The aim of this article is to identify the ideal type and location of colostomy in children with colorectal disease. Patients and Methods A retrospective case study of children with an anorectal malformation who received a colostomy, born between January 1990 and July 2012. Furthermore, a systematic literature search on colostomies in neonates with an anorectal malformation or Hirschsprung disease. Colostomies were classified as loop or split colostomies in the transverse or sigmoid colon. Outcome measures were mortality and complications such as prolapse, technical difficulties with the reconstruction, urinary tract infections, and others. Results The mortality rate in the 180 children with anorectal malformation was 6%, and none of them were directly related to stoma formation or closure. The overall complication rate was 23% and the specific rates for the two types of procedures and the two locations of the colostomy did not differ (p = 0.389 and p = 0.667, respectively). All prolapses (n = 22) occurred in loop colostomies in the transverse colon. One colostomy required revision because of insufficient length for the reconstruction. Urinary tract infections were not documented. A total of eight studies were included in the systematic review (1982-2011; 2,954 patients). Mortality ranged between 0.1 and 11%. Loop colostomies had more complications than split colostomies (63 vs. 45%; p = 0.007), mainly prolapse (18 vs. 6%; p < 0.001). Overall complication rate differed between transverse en sigmoid colostomies (62 vs. 51%, p = 0.006), and prolapse occurred more often in the transverse colon (23 vs. 7%; p < 0.001). Revision because of insufficient length during the reconstruction was needed in 0 to 6%. Two studies reported on urinary tract infections which are as follows: One showed no difference between loop or split colostomies, whereas the other showed frequent episodes of urinary tract infections in 64% of the loop colostomies. Conclusions The complication to be avoided in transverse colostomies is prolapse and the surgical technique should be modified accordingly. The procedure of split sigmoid colostomy is meticulous, and the risk of insufficient length for the reconstruction remains. [ABSTRACT FROM AUTHOR]

Abstract: Background: It has been suggested that the outcome of transanal endorectal pull-through for classic Hirschprung's disease can be improved by laparoscopically mobilizing the colon before the pullthrough. Methods: Charts of 43 patients (2005–2009) with proven recto-sigmoid aganglionosis were retrospectively analyzed with respect to postoperative outcomes. Twenty-one had been treated with the transanal endorectal pull through (TERPT) and 22 with the laparoscopically assisted TERPT (LTERPT). Results: Gender ratio, congenital anomalies, preoperative enterostomy, and follow up did not differ between the groups. More colon was resected in the TERPT group: median 25cm vs. 15cm in the L-TERPT group (p<0.001). The TERPT-procedure took less time: median 153min. vs. L-TERPT 263min (p<0.001). Postoperatively, three patients showed colonic torsions after TERPT (p=0.07). The long-term clinical outcomes did not differ significantly between both groups. There was a significant association between length of resection and obstructive symptoms (OR=0.92, p=0.01). Conclusion: Postoperative and clinical outcomes are similar using the TERPT or L-TERPT to correct classic segment Hirschsprung's disease. Prevention of colonic torsion should be the prime concern during the TERPT procedure. L-TERPT requires laparoscopic equipment and takes more operation time, whereas TERPT leaves no visible scars. The positive relation between the larger length of resection and obstructive symptoms requires additional research. [Copyright &y& Elsevier]

Introduction Anorectal malformations ( ARMs) and Hirschsprung's disease ( HD) are congenital malformations requiring pelvic floor surgery in early childhood, with possible sequelae for psychosexual development. Aims To assess psychosexual well-being in adult ARM and HD patients related to health-related quality of life. Methods Eligible for this cross-sectional two-center study were all patients aged ≥18 years who had been operated for ARM or HD. Exclusion criteria were intellectual disability, comorbidity affecting sexual functioning, and cloacal malformation. Main Outcome Measures Participants completed the International Index of Erectile Functioning, Female Sexual Functioning Index, Female Sexual Distress Scale, Hirschsprung and Anorectal Malformation Quality of Life Questionnaire, and sexual education questionnaire. Results Response rates were 32% and 37% for ARM and HD patients, respectively. We studied 70 participating ARM and 36 HD patients (median age 26 years). We excluded 10 patients with sexual inactivity in the past 4 weeks. Six of 37 men with ARM (16%) reported moderate to severe erectile dysfunction, vs. two of 18 men with HD (11%). Thirteen and 10 of 26 women with ARM (50% and 38%) reported sexual dysfunction or sexual distress, respectively, vs. eight and three of 15 women with HD (53% and 20%). Quality of life and type of malformation or operation were not associated with self-reported psychosexual problems. Addressing sexuality with special interest to the congenital anomaly during medical care was reported to be insufficient by 42 ARM (60%) and 22 HD patients (61%). Conclusion Approximately 13% of male ARM and HD patients reported erectile dysfunction, while 50% female ARM and HD patients reported sexual dysfunction not related to quality of life or type of malformation. Both ARM and HD patients felt a need for better addressing sexual concerns during medical care. Further research is needed to optimize form and timing of this education. van den Hondel D, Sloots CEJ, Bolt JM, Wijnen RMH, de Blaauw I, and IJsselstijn H. Psychosexual well-being after childhood surgery for anorectal malformation or H irschsprung's disease. J Sex Med 2015;12:1616-1625. [ABSTRACT FROM AUTHOR]