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.

Evaluation of a Group-based Patient Education Program Promoting Self-management in Adults with Hirschsprung Disease and Anorectal Malformations.

INTRODUCTION: Adults with Hirschsprung disease (HD) and anorectal malformations (ARM) may experience persisting and new somatic and psychosocial problems. Patient education programs (PEPs) may improve self-management in patients with chronic illnesses. The aim of this study was to explore HD and ARM adults' experiences with and evaluation of a group-based PEP. We also looked at factors that might influence the attendance rate. METHOD: Non-intellectually impaired HD and ARM adults were invited to attend a diagnosis specific PEP at a pelvic floor interdisciplinary center. Eight health care professionals lectured. Aspects of the PEP were graded anonymously in a patient reported experience measure (PREM). Ethical approval was obtained. RESULTS: 17% (21/125) of invited adults (10HD, 11ARM) attended four PEPs. 19/21 (90%) PREMs were returned. Participants found meeting peers and sharing experiences especially valuable in addition to improved disease knowledge. Lectures by the pediatric and colorectal surgeons, stoma nurse, and sexologist were rated highest by the participants. The majority reported that the PEP would be helpful in managing everyday life. All participants recommended PEP in adolescence. Factors such as gender and travel distance did not affect attendance rate, but participants were older than non-participants, median 37 versus 24 years (p = 0.01). CONCLUSION: Attendance rates were low among HD and ARM adults invited to a PEP, but participants were overall highly satisfied. Peer support, mutual learning, and increased disease knowledge were seen as invaluable assets of the PEP. A web-based PEP was discouraged, while physical PEPs for both adults and adolescents were encouraged. LEVEL OF EVIDENCE: III.

A nordic multicenter study on contemporary outcomes of pediatric short bowel syndrome in 208 patients.

BACKGROUND & AIMS: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population. METHODS: Patients with SBS-IF treated during 2010-2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels. RESULTS: Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21-80%). After median follow up of 4.4 years (IQR 2.5-6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies. CONCLUSIONS: Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.

Nurses' Experiences With Anal Dilatations in Babies With Anorectal Malformations - A Focus Group Interview Study.

AIMS AND OBJECTIVES: To explore nurses' experiences with anal dilatations in babies with anorectal malformations. BACKGROUND: Most babies with anorectal malformations require repeated anal dilatations, either before and/or after reconstructive surgery. Anal dilatation is usually performed without sedation or pain medication. Nurses participate in anal dilatations by assisting doctors doing anal dilatation, doing anal dilatation themselves, and instructing parents how to do anal dilatations. No previous studies have explored how nurses experience being involved in anal dilatations. DESIGN: Qualitative study design utilizing focus group interviews. The COREQ guidelines were applied. METHODS: Nurses with either ≤2 or ≥10 years' working experience participated in two different focus group interviews. The focus group interviews were transcribed and analyzed with content analysis. RESULTS: Twelve nurses, two males, participated. Three main themes emerged from the focus group interviews. The first main theme, "Anal dilatation causes distress", describes the nurses' worries about causing physical and/or psychological harm when doing anal dilatations. The second main theme, "Need for guidelines and training", contains nurses' recommendations for more theoretical training in addition to written guidelines on anal dilatations. The third main theme, "Collegial support is vital", describes nurses' needs and strategies for coping with difficult situations related to anal dilatations. CONCLUSIONS: Anal dilatation causes distress in nurses, and collegial support is essential for coping. Guidelines and systematic training are recommended to improve current practice. LEVEL OF EVIDENCE: VI.

Perioperative and Long-Term Outcome in Patients Treated for Jejunoileal Atresia.

OBJECTIVES: Jejunoileal atresia is a common cause of neonatal intestinal obstruction. Results of long-term outcome are very limited. The aim of this study was to describe perioperative and postoperative outcome, and to evaluate long-term gastrointestinal quality of life (QoL) in patients treated for jejunoileal atresia. METHODS: We conducted a combined retrospective and cross-sectional observational study of patients treated for jejunoileal atresia during 2001-2019. Perioperative data were registered, and complications were classified according to the Clavien-Dindo classification. To evaluate long-term clinical status and QoL, the PedsQL Gastrointestinal Symptoms Scales questionnaire and a self-designed questionnaire were used. Approval from the Data Protection Office was obtained. RESULTS: Seventy patients were included in the retrospective part of the study. Of these, 70% got a primary anastomosis. Concomitant anomalies were registered in 46%. Median length of stay was 28 (5-140) days. Early (<28 days) complication rate was 66%. Early abdominal surgical reinterventions were performed in 11%, most frequently due to anastomotic leak. Late (>28 days) abdominal surgical reinterventions were performed in 21%, and most commonly for bowel obstruction. Overall mortality rate was 4%. Thirty-two patients with median 8 (2-19) years returned the questionnaires. Overall gastrointestinal QoL was good. However, concomitant gastrointestinal anomalies were associated with impaired outcome. Embarrassment of the scar was reported in 38%. Three (9%) patients used medication (laxatives, proton pump inhibitor) for gastrointestinal symptoms. CONCLUSIONS: Jejunoileal atresia is associated with significant morbidity during initial treatment. Despite this, the majority of the patients have excellent long-term outcomes.

Outcome a decade after laparoscopic and open Nissen fundoplication in children: results from a randomized controlled trial.

BACKGROUND: Randomized controlled trials (RCT) comparing long-term outcome after laparoscopic (LF) and open fundoplication (OF) in children are lacking. Here we report recurrence rates and time to recurrence, frequency of re-interventions, use of antisecretory drugs, gastrointestinal symptoms, and patient/parental satisfaction a decade after children were randomized to LF or OF. METHODS: Cross-sectional long-term follow-up study of a two-center RCT that included patients during 2003-2009. Patients/parents were interviewed and medical charts reviewed for any events that might be related to the fundoplication. If suspicion of recurrence, further diagnostics were performed. Informed consent and ethical approval were obtained. CLINICALTRIALS: gov: NCT01551134. RESULTS: Eighty-eight children, 56 (64%) boys, were randomized (LF 44, OF 44) at median 4.4 [interquartile range (IQR) 2.0-8.9] years. 46 (52%) had neurological impairment. Three were lost to follow-up before first scheduled control. Recurrence was significantly more frequent after LF (24/43, 56%) than after OF (13/42, 31%, p = 0.004). Median time to recurrence was 1.0 [IQR 0.3-2.2] and 5.1 [IQR 1.5-9.3] years after LF and OF, respectively. Eight (19%) underwent redo fundoplication after LF and three (7%) after OF (p = 0.094). Seventy patients/parents were interviewed median 11.9 [IQR 9.9-12.8] years postoperatively. Among these, use of anti-secretory drugs was significantly decreased from preoperatively after both LF (94% vs. 35%, p < 0.001) and OF (97% vs. 19%, p < 0.001). Regurgitation/vomiting were observed in 6% after LF and 3% after OF (p = 0.609), and heartburn in 14% after LF and 17% after OF (p = 1.000). Overall opinion of the surgical scars was good in both groups (LF: 95%, OF: 86%, p = 0.610). Patient/parental satisfaction with outcome was high, independent of surgical approach (LF: 81%, OF: 88%, p = 0.500). CONCLUSIONS: The recurrence rate was higher and recurrence occurred earlier after LF than after OF. Patient/parental satisfaction with outcome after both LF and OF was equally high.

Congenital Duodenal Obstruction: National Trends in Management and Outcomes during the Last Quarter of a Century in Norway.

INTRODUCTION: During the last quarter of a century, new surgical techniques in neonates have been introduced, and neonatal intensive care has developed. Few studies have explored the implementation of new techniques and if outcomes in neonates undergoing gastrointestinal surgery have improved in the last decades. Therefore, this study aimed to investigate possible changes in postoperative outcomes and surgical techniques in all neonates operated for congenital duodenal obstruction (CDO) 1995 to 2020 in Norway. MATERIAL AND METHODS: This is a national multicenter retrospective study of all neonates undergoing surgery for CDO in Norway from 1995 to 2020. Results from three periods (1995-2003, 2004-2012, and 2013-2020) were compared. The study was approved by the local data protection officers (2020/13386) and (2020/15125). RESULTS: We included 186 patients: 41 in period 1 (1995-2003), 83 in period 2 (2004-2012), and 62 in period 3 (2013-2020). Seventy (38%) neonates had Down syndrome and 104 (62%) had additional malformations/disorders. Birth weight, gender, frequency of Down syndrome, and other malformations/disorders did not differ between the three periods. We observed an increased rate of prenatal diagnosis throughout the study period (p < 0.001). The only change in surgical technique was the increased use of transanastomotic feeding tubes (p < 0.001). Length of stay, postoperative complication rate, days with parenteral nutrition, and 30-day mortality rate were stable over time. CONCLUSION: Perioperative treatment and postoperative outcomes in neonates with CDO have been surprisingly unchanged during the last quarter of a century. Only an increased rate of prenatal diagnosis and more frequent use of transanastomotic feeding tubes were observed.

Sexual function and lower urinary tract symptoms after minimally invasive endorectal pull-through in adolescent males with Hirschsprung disease.

BACKGROUND: Effects of colorectal resection on sexual function and the lower urinary tract are inadequately studied in patients with Hirschsprung disease (HD). This study aimed to increase existing knowledge on sexual function and lower urinary tract symptoms (LUTS) in HD male adolescents operated with minimally invasive endorectal pull-through (ERPT). METHODS: Non-syndromic male adolescents (12-18 years) operated with ERPT were invited to participate in this single-center cross-sectional study which included a semi-structured interview on sexual function and LUTS, a questionnaire recording LUTS, and a urodynamic study. Uroflowmetry curves were eligible for evaluation if the voided volume was >50% of expected bladder capacity. Ethical approval and informed consent were obtained. RESULTS: Of 37 eligible male adolescents, 35 (95%) with a median age of 14.9 (12.0 -18.3) years were included. 94% had rectosigmoid aganglionosis, and 97% underwent a minimally invasive ERPT. 34 (97%) visited the outpatient clinic. The ability to get erections and to ejaculate was assessed in 25/35 (71%) adolescents. 25/25 (100%) could get an erection, and of adolescents ≥15 years 14/15 (93%) could ejaculate. 32 (91%) returned the questionnaire and underwent urodynamic studies. 15/32 (47%) reported LUTS, but most had only sporadic LUTS. 31/32 (97%) were highly satisfied with their bladder function. Sporadic urinary incontinence was reported by 4/32 (13%), but none reported social problems due to this. Of the eligible 28/32 (88%) uroflowmetry curves, 15/28 (54%) were normal. CONCLUSIONS: Minimally invasive ERPT seems to preserve sexual function and does not induce lower urinary tract symptoms in adolescent HD males. LEVEL OF EVIDENCE: III.

Post-cholecystectomy syndrome in pediatric patients: Occurrence and spectrum of symptoms.

BACKGROUND: Post-cholecystectomy syndrome (PCS) refers to persistent or new abdominal symptoms after cholecystectomy. As there are very few reports on PCS in pediatric patients, we aimed to examine whether it was a frequent finding and which symptoms the affected children experienced. METHOD: This is a retrospective cross sectional study of pediatric patients, who underwent cholecystectomy during 2003-2019 at Oslo University Hospital. The PedsQL™ gastrointestinal symptoms scale questionnaire and a self-designed questionnaire exploring satisfaction after surgery and current medical conditions were mailed to all eligible patients. Patient/parental consent and approval from the local data protection officer (19/09054) were obtained. RESULTS: Questionnaires were sent to 82 patients of whom 44 (54%) answered. There were no significant demographic differences between the responders and the non responders. We identified 16 (36.7%) patients to have PCS. The most common symptoms were diarrhea (25%), bloating (16%), and heartburn/reflux (16%). Overweight was more common in patients with PCS (31%) than in patients without PCS (4%) (p = 0.014). Altogether 34/44 (77.3%) patients were satisfied with the result of the cholecystectomy; 92,6% of patients without PCS and 56.6% of those with PCS (p = 0.012). CONCLUSION: PCS is not uncommon in pediatric patients, and they report a wide range of gastrointestinal symptoms. We identified overweight as a potential risk factor for developing PCS. Nonetheless, most patients got total relief of abdominal pain and were satisfied with outcome after cholecystectomy. LEVEL OF EVIDENCE: Level 3.

Reduced complication rate after implementation of a detailed treatment protocol for percutaneous endoscopic gastrostomy with T-fastener fixation in pediatric patients: A prospective study.

BACKGROUND/AIMS: Percutaneous endoscopic gastrostomy with push technique (PEG-T) is increasingly used in pediatric patients. In a retrospective study of PEG-T (cohort 1) we reported frequent complications related to T-fasteners and tube dislodgment. The aim of this study was to assess complications after implementation of a strict treatment protocol, and to compare these with the previous retrospective study. MATERIALS AND METHODS: The study is a prospective study of PEG-T placement performed between 2017 and 2020 (cohort 2) in pediatric patients (0-18 years). Complications were recorded during hospital stay, fourteen days and three months postoperatively, graded according to the Clavien-Dindo classification and categorized as early (<30 days) or late (>30 days). RESULTS: In total 82 patients were included, of which 52 (60%) had neurologic impairments. Median age and weight were 2.0 years [6 months-18.1 years] and 13.4 kg [3.5-51.5 kg], respectively. There was a significant reduction in median operating time from 28 min [10-65 min] in cohort 1 to 15 min [6-35 min] in cohort 2 (p<0.001), number of patients with early tube dislodgement (cohort 1: 9 (10%) vs cohort 2: 1 (1%), p = 0.012), and number of patients with late migrated T-fasteners (cohort 1: 11 (13%) vs cohort 2: 1 (1%), p = 0.004). CONCLUSION: We experienced less migrated T-fasteners and tube dislodgment after implementation of strict treatment protocol. LEVEL OF EVIDENCE: Treatment study level III.

Comparison of clinical outcomes after total transanal and laparoscopic assisted endorectal pull-through in patients with rectosigmoid Hirschsprung disease.

BACKGROUND: Total transanal (TERPT) and laparoscopic endorectal pull-through (LERPT) are the most common procedures to treat rectosigmoid Hirschsprung's disease (HD). Since few studies have compared the two methods, we aimed to assess clinical outcomes after TERPT and LERPT in this cross-sectional study. METHODS AND PATIENTS: All patients with rectosigmoid HD operated with TERPT and LERPT between 2001 and 2018 were eligible. Peri-operative data were registered from patients' records, and bowel function was assessed according to the Krickenbeck classification. RESULTS: 91/97 (94%) patients were included; 46 operated with TERPT and 45 with LERPT. Bowel function was assessed in 80 patients at median seven (4-17) years. There was no difference in functional outcome between the procedures. Unplanned procedures under general anesthesia were frequent; 28% after TERPT and 49% after LERPT (p = 0.04). 11% of TERPT and 29% of LERPT patients got botulinum toxin injections (p = 0.03). In the TERPT group, patients operated in the neonatal period had poorer outcome (78%) than those operated later (24%) (p = 0.005). No difference in operative time, length of hospital stay, and rate of early and late complications was found between the procedures. CONCLUSION: There was no difference in long-term bowel function in patients with rectosigmoid HD operated with TERPT or LERPT. More LERPT patients had an unplanned procedure under general anesthesia, mostly due to obstructive symptoms. LEVEL OF EVIDENCE: III.

Short and long-term outcomes after pediatric redo fundoplication.

BACKGROUND: Redo fundoplication (RF) is the most common surgical treatment for recurrent gastroesophageal reflux disease (GERD) in children, but outcomes after RF are rarely reported. The aim of this study was to assess short- and long-term outcomes after RF in childhood. METHODS: The study is a follow-up study of patients undergoing RF from 2002 to 2020 at a teriary care center. Patients/parents were sent questionnaires recording symptoms of recurrent GERD, troublesome side-effects and satisfaction. Retrospective chart review was also performed. RESULTS: 24/28 (86%) patients were included median 9 (1.6 months-17.7 years) years after RF. 16 (67%) had neurologic impairment. Indications for RF was recurrence of GERD (n = 18), discomfort or dysphagia from a herniated wrap (n = 5) and dysphagia from a slipped fundoplication (n = 1). Median operating time was 128 (95-250) min. Six (25%) patients experienced early major complications, of which two were gastrostomy related. Five (21%) patients experienced recurrence after RF. Three of these were symptom free at follow-up with medical treatment or re-RF. The most common symptom at follow-up was stomach pain (37%) and excessive flatulence (38%). 18/22 (95%) patients/parents would choose RF again, and 21/22 would recommend RF to someone in a similar situation. CONCLUSIONS: RF is successful in treating recurrent GERD after primary fundoplication, and patient/parental satisfaction is high.

A Novel Standard for Systematic Reporting of Neuroblastoma Surgery: The International Neuroblastoma Surgical Report Form (INSRF): A Joint Initiative by the Pediatric Oncological Cooperative Groups SIOPEN∗, COG∗∗, and GPOH∗∗∗.

OBJECTIVE: To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA: NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS: After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS: The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION: The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.

Is postoperative transanastomotic feeding beneficial in neonates with congenital duodenal obstruction?

PURPOSE: We aimed to evaluate possible positive and negative effects of postoperative use of transanastomotic feeding tube (TAFT) in neonates operated for congenital duodenal obstruction (CDO). METHODS: This is a retrospective study reviewing medical records of neonates operated for CDO during 2003-2020 and comparing postoperative feeding outcomes and complications in patients with and without TAFT. Approval from the hospital's data protection officer was obtained. RESULTS: One hundred patients, 59% girls, were included, and 37% received TAFT. Mean birth weight and gestational age were 2628 (675.1) grams and 36.6 (2.4) weeks, respectively. Furthermore, 45% had no other malformations, and 36% had Down syndrome. Patient demographics were similar for TAFT and not-TAFT patients, except that not-TAFT neonates weighed median 335 g less (p = 0.013). The TAFT group got parenteral nutrition 2 days shorter (p < 0.001) and started enteral feeds 1.5 days earlier (p < 0.001) than the not-TAFT group. Fewer neonates with TAFT got a central venous catheter [65 vs 89%, (p = 0.008)]. In the TAFT group, 67% were breast fed at discharge compared to 49% in the not-TAFT group (p = 0.096). CONCLUSION: Neonates with TAFT had earlier first enteral feed, fewer days with parenteral nutrition and fewer placements of central venous catheters.

Reaching adulthood with Hirschsprung's disease: Patient experiences and recommendations for transitional care.

BACKGROUND/PURPOSE: The need for transitional care has gained increased focus in the treatment of patients with congenital colorectal disorders. We aimed to acquire in-depth knowledge about the experiences of adult patients with Hirschsprung's disease (HD) and their suggestions for transitional care. METHODS: Binational study applying gender equal focus group interviews (FGI). RESULTS: Seventeen (9 men) of 52 invited patients with median age 29 (19-43) years participated. Three themes evolved from the FGI. "Scarred body and soul" describes the somatic and psychosocial challenges the patients experienced and "limited health literacy on HD" refers to the patients' lack of HD knowledge. "Absent transition" depicts missing transitional care and the patients' inability to find adult HD specialists. The adult HD patients strongly recommended transitional care from early teens with focus on information about HD and establishment of a peer-to-peer program. They also emphasized the possibility of being referred to a pelvic floor center. CONCLUSIONS: HD negatively influences patients' somatic and psychosocial health in childhood, adolescence and adulthood. Adult HD patients strongly recommend transitional care from early teens and the possibility for referral to a center working with pelvic floor dysfunctions. LEVEL OF EVIDENCE: IV TYPE OF RESEARCH: Clinical.

Partial Biliary Diversion May Promote Long-Term Relief of Pruritus and Native Liver Survival in Children with Cholestatic Liver Diseases.

INTRODUCTION: Rare cholestatic liver diseases may cause debilitating pruritus in children. Partial biliary diversion (PBD) may relieve pruritus and postpone liver transplantation which is the only other alternative when conservative treatment fails. The aim was to report long-term outcome after PBD in a population of 26 million people during a 25-year period. MATERIALS AND METHODS: This is an international, multicenter retrospective study reviewing medical journals. Complications were graded according to the Clavien-Dindo classification system. RESULTS: Thirty-three patients, 14 males, underwent PBD at a median of 1.5 (0.3-13) years at four Nordic pediatric surgical centers. Progressive familial intrahepatic cholestasis was the most common underlying condition. Initially, all patients got external diversion, either cholecystojejunostomy (25 patients) or button placed in the gallbladder or a jejunal conduit. Early complications occurred in 14 (42%) patients, of which 3 were Clavien-Dindo grade 3. Long-term stoma-related complications were common (55%). Twenty secondary surgeries were performed due to stoma problems such as prolapse, stricture, and bleeding, or conversion to another form of PBD. Thirteen children have undergone liver transplantation, and two are listed for transplantation due to inefficient effect of PBD on pruritus. Serum levels of bile acids in the first week after PBD construction were significantly lower in patients with good relief of pruritus than in those with poor effect (13 [2-192] vs. 148 [5-383] µmol/L; p = 0.02). CONCLUSION: PBD may ensure long-term satisfactory effect on intolerable pruritus and native liver survival in children with cholestatic liver disease. However, stoma-related problems and reoperations are common.