Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. RESULTS: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. TYPE OF STUDY: Clinical consensus statement. LEVEL OF EVIDENCE: 3a.

Push-PEG or Pull-PEG: Does the Technique Matter? A Prospective Study Comparing Outcomes After Gastrostomy Placement.

BACKGROUND: Push-PEG (percutaneous endoscopic gastrostomy) with T-fastener fixation (PEG-T) allows one-step insertion of a balloon tube or button, and avoids contamination of the stoma by oral bacteria. However, PEG-T is a technically more demanding procedure with a significant learning curve. The aim of the present study was to compare outcomes after PEG-T and pull-PEG in a setting where both procedures were well established. MATERIALS AND METHODS: The study is a prospective cohort study including all patients between 0 and 18 year undergoing PEG-T and pull-PEG between 2017 and 2020 at a combined local and tertiary referral center. Complications and parent reported outcomes were recorded during hospital stay, after 14 days and 3 months postoperatively. RESULTS: 82 (93%) of eligible PEG-T and 37 (86%) pull-PEG patients were included. The groups were not significantly different with regard to age or weight. Malignant disorders and heart conditions were more frequent in the pull-PEG group, whilst neurodevelopmental disorders were more frequent in the PEG-T group (p < 0.001). 54% in both groups had a complication within 2 weeks. Late complications (between 2 weeks and 3 months postoperatively) occurred in 63% PEG-T vs 62% pull-PEG patients (p = 0.896). More parents in the pull-PEG group (49%) reported that the gastrostomy tube restricted their child's activity, compared to PEG-T (24%) (p = 0.01). At 3 months follow-up, more pull-PEG patients (43%) reported discomfort from the gastrostomy compared to PEG-T (21%) (p = 0.03). CONCLUSION: Overall complication rates were approximately similar, but pull-PEG was associated with more discomfort and restriction of activity. LEVELS OF EVIDENCE: Treatment study level II.

Mental Health, Psychosocial Functioning, and Quality of Life in Adolescents With Hirschsprung Disease.

BACKGROUND: Studies of mental health in adolescents with Hirschsprung disease (HD) are scarce. This cross-sectional study investigates mental health, psychosocial functioning and quality of life in HD adolescents. METHODS: Adolescents (12-18 years) treated at the Department of pediatric surgery at Oslo University Hospital were invited for participation. Mental health was assessed by interview; Child Assessment Schedule (CAS) and questionnaires; parental Child Behavior Checklist (CBCL) and adolescent Youth Self-Report (YSR). Psychosocial functioning was rated by Child Global Assessment Scale (cGAS). Adolescent Quality of Life was assessed by Pediatric Quality of Life inventory (PedsQL) and chronic family difficulties (CFD) by interview. Medical records were reviewed for somatic history. RESULTS: Thirty-seven adolescents, 28 males, median age 14.3 years, participated. By CAS interview, 8 of 37 (44% of females and 14% of males) fulfilled criteria for psychiatric diagnosis all within emotional and related disorders. Twenty-seven percent had CBCL internalizing scores and 16% had YSR internalizing scores in clinical range indicating emotional problems. By interviewer rated cGAS, 27% were scored in clinical range. By PedsQL 16% reported reduced psychosocial health score. Increased CFD, lower psychosocial functioning and reduced QoL as well as less paternal education were significantly associated with psychiatric diagnosis. Twice as many (4/8) adolescents who either had a stoma or bowel management had a psychiatric diagnosis compared to those who had neither stoma nor bowel management (7/28). CONCLUSION: Nearly one in four adolescents with HD fulfilled criteria for psychiatric diagnosis. Mental health problems were associated with reduced psychosocial function and reduced QoL. LEVEL OF EVIDENCE: III.