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.The study has financial support from the Norwegian DAM foundation (2019/FO249474).publishedVersio

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.</p

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

Laparoscopic gastrostomy placement in children has few major, but many minor early complications

Abstract Introduction Laparoscopic gastrostomy (LAPG) is an increasingly popular alternative to more traditional gastrostomy techniques. This study evaluates early postoperative complications following LAPG and investigates risk factors for gastrostomy complications. Materials and Methods Retrospective study of patients &lt;16 years undergoing LAPG from 2005 to 2018. Early postoperative complications (&lt;30 days) were grouped as gastrostomy-related or general and graded according to the Clavien-Dindo classification for surgical complications. Results A total of 104 patients, of which 54 (52%) had neurological impairment (NI), were included. Median age and weight were 1.2 years (1 day–15.2 years) and 8.9 kg (3.4–36), respectively. Operating time was median 37 minutes (19–86) and shorter in the second half of the patients (46 vs. 35 minutes, p = 0.04). A total of 40 (38%) patients experienced 53 gastrostomy-related complications. Of these, seven complications needed surgical treatment; severe leakage (2), too short gastrostomy button (1), feeding difficulties (1), gastric outlet obstruction (1), omentum trapped in umbilical port sutures (1), and suspected fascial defect (1). Stoma infection and granulation tissue were reported in 13 and 12%, respectively. Tube dislodgement occurred in six patients and was managed with bedside reinsertion in all. Gastrostomy-related complications were less frequent in NI patients (46 vs 22%, p = 0.01). Conclusion LAPG is a safe procedure with few major complications, but a high rate of minor complications. Operating time declined during the study period, and NI patients had fewer gastrostomy-related complications

Experiences and needs of parents of babies treated for congenital duodenal obstruction – A qualitative study

Background: Knowledge about experiences and needs of parents of babies operated for congenital gastrointestinal anomalies in the neonatal period is very limited. Therefore, we performed this qualitative study to explore the experiences and needs in this group of parents. Methods: Parents of babies operated for congenital duodenal obstruction participated in a focus group discussion. The focus group discussion was analyzed with hermeneutical interpretation, and themes representing important areas to the parents were identified. Results: Six parents of five babies participated. Four themes evolved from the focus group discussion. “Grief over loss of initial parent-child bonding” describes the feeling of being separated from the baby during initial treatment. “Let me take care of my baby” refers to the importance for the parents to be involved in caring for their baby. “Concerns about breastfeeding” reflects the mothers’ worries about and need for encouragement and guidance regarding pumping and breastfeeding. “Trusting health care providers” implies that the parents were confident that their baby was well taken care of, despite an emotionally stressful situation. Conclusions: This qualitative study provides new and important knowledge regarding the experiences and needs of parents of babies operated for congenital duodenal obstruction. The results may be generalizable to other gastrointestinal anomalies needing surgery. This new knowledge may help health care providers to better meet the needs in parents of a baby having undergone repair of gastrointestinal anomaly. Level of evidence: I

Bowel function after transanal endorectal pull-through for Hirschsprung disease – does outcome improve over time?

Background The reported functional outcome in patients operated with transanal endorectal pull-through (ERPT) for Hirschsprung disease (HD) varies greatly. Some studies suggest better functional outcome in older than in younger HD patients, but there are almost no longitudinal studies that have demonstrated such improvement. Therefore, we aimed to compare functional outcome in a cohort of HD patients over time to assess whether bowel function improves with increasing age. Methods Functional outcome in HD patients operated with ERPT from 1998 to 2007 was recorded by standardized interviews by an independent investigator during 2008–2011 and again in 2017/2018. Bowel function was assessed using the Krickenbeck questionnaire. Clinical data were collected prospectively. Results 50 of the original 62 patients responded for a second interview. Median age at the two interviews was 8,1 (3,4-16,6) and 15,4 (9,9–25) years respectively. There was no difference in the rate of soiling at first (52%) and second (52%) follow-up. Constipation was reported in 20% of the patients at first, and in 24% at second follow-up. Bowel management was used by 30% and 32% at first and second interview respectively. Conclusion Soiling and constipation are common in HD patients several years after surgery, and no improvement of bowel function with increasing age could be demonstrated

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

Abstract 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  &lt; 0.001) and started enteral feeds 1.5 days earlier ( p  &lt; 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

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

Bowel symptoms in children with anorectal malformation : A follow-up with a gender and age perspective.

Abstract Background Gender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling. Purpose To assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age. Method All children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8years, was analyzed. Results Among those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p=0.005) whereas constipation occurred in 62% of the females and 35% of the males (p0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p=0.013). Bowel symptoms did not change with age among the females. Conclusion Gender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes

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. Keywords Hirschsprung disease Endorectal pull-through Minimally invasive surgery Sexual function Lower urinary tract symptoms Adolescence Abbreviations CAKUTcongenital anomalies of the kidney and urinary tract DAN-PSSdanish prostatic symptom score ERPTendorectal pull-through HDHirschsprung disease LUTSlower urinary tract symptoms MCUGmicturating cystourethrogra