Anorectal malformation and Hirschsprung's disease: a cross-sectional multicentre comparison of quality of life and bowel function to a healthy population.

PURPOSE: Children with anorectal malformation (ARM) and Hirschsprung's disease (HD) often experience bowel symptoms into adulthood, despite definitive surgery. This study evaluates the quality of life (QOL) and bowel functional outcome of children treated for ARM and HD in comparison to healthy controls. METHODS: Between December 2020 and February 2023, we recruited patients with ARM and HD aged 3-17 years at four tertiary referral centres, who had primary corrective surgery done >12 months prior. Healthy controls were age-matched and sex-matched. All participants completed the Pediatric Quality of Life Inventory Generic Core Scales 4.0, General Well-Being (GWB) Scale 3.0 and Family Impact (FI) Module 2.0 Questionnaires. Bowel Function Score (BFS) Questionnaires were also administered. We also performed subgroup analysis according to age categories. Appropriate statistical analysis was performed with p<0.05 significance. Ethical approval was obtained. RESULTS: There were 306 participants: 101 ARM, 87 HD, 118 controls. Patients with ARM and HD had significantly worse Core and FI Scores compared with controls overall and in all age categories. In the GWB Scale, only ARM and HD adolescents (13-17 years) had worse scores than controls. ARM and HD had significantly worse BFSs compared with controls overall and in all age categories. There was significant positive correlation between BFS and Core Scores, GWB Scores and FI Scores. CONCLUSION: Patients with ARM and HD had worse QOL than controls. Lower GWB Scores in adolescents suggests targeted interventions are necessary. Bowel function influences QOL, indicating the need for continuous support into adulthood.

Parent-Reported Health-Related Quality of Life Outcomes and Impact on Family of Congenital Diaphragmatic Hernia Survivors After Repair: A Cross-Sectional Comparison to Healthy Controls.

BACKGROUND: Congenital diaphragmatic hernia (CDH) survivors often experience long-term CDH-associated morbidities, including musculoskeletal, gastrointestinal and respiratory issues. This study evaluates parent-reported health-related quality of life (HRQOL) and family impact of the disease. METHODS: Electronic medical records (EMR) were reviewed and phone surveys performed with parents of CDH survivors who underwent repair at our institution from 2010 to 2019. They completed the following Pediatric Quality of Life Inventory™ (PedsQL™) questionnaires: Generic Core Scales 4.0 (parent-proxy report) and Family Impact (FI) Module 2.0. Age-matched and gender-matched healthy controls from an existing database were used for comparison. Subgroup analysis of CDH patients alone was also performed. Appropriate statistical analysis was used with p < 0.05 significance. Data are reported as median (range). Ethical approval was obtained. RESULTS: Of 76 CDH survivors, 45 parents (59 %) consented for study participation. Patients and controls were aged 6 (3-12) years; 51 % were male. In Core Scales, there were significant differences between the groups in the overall scores (p = 0.003) and the psychosocial health sub-scores (p = 0.004), but no difference in the physical health domain scores. In the Family Impact Module, there were no significant differences between the groups in overall scores, in parent HRQOL and in family functioning sub-scores. However, CDH patients with learning disabilities had significantly worse Family Impact Module overall scores compared to CDH patients without learning disabilities (p = 0.04). CONCLUSION: The overall HRQOL in children with CDH survivors is worse compared to controls, but impact on family appears similar. We highlight the need for long-term follow-up of CDH patients, especially those with learning disabilities. Healthcare providers should formulate tailored care plans to address patient and caregiver needs. TYPE OF STUDY: Prospective Study. LEVEL OF EVIDENCE: Level III.

Sociodemographic factors affecting paediatric surgical training in Malaysia: Gender matters.

BACKGROUND: A shortage of specialist paediatric surgeons in Malaysia led to establishment of a national postgraduate training programme in 2004. We aimed to identify sociodemographic factors impacting training experience, and to define pressure points during training to inform targeted trainee support strategies. METHODS: We conducted an anonymized online survey in June 2021 on all programme graduates. Participants were asked for sociodemographic data, both current and during training. Likert scale responses were required for questions regarding adequacy of support received for family, health and personal issues during training. A free text response question soliciting suggestions for programme improvement was included. Data are reported as median (range). Chi-square/Fisher's exact tests for categorical variables and Mann-Whitney U tests for continuous variables were used, with p<0.05 significance. RESULTS: Of 53 eligible participants, 52 (98%) responded, 24 (46%) were female. Marital status was similar between genders at entry, but female trainees were more likely to be unmarried on exit (p = 0.001), and less likely to have children while training (p = 0.017). Of the 6 female and 18 male trainees who had children while training, women were more likely to take parental leave (p = 0.01). The majority felt advice given regarding parental leave and managing training while having children were poor. In thematic analysis of free text answers, lack of hands-on experience was the most common concern. CONCLUSION: Factors related to marriage and parenthood significantly associate with gender amongst trainees in Malaysia despite both genders being well represented. Concerns regarding adequacy of hands-on training highlight the need for educational innovations such as simulation models. LEVEL OF STUDY: Level III.

Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery.

INTRODUCTION: Anorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function. METHODOLOGY: This was a prospective observational study. Convenience sampling method was used to recruit all HD patients who had definitive pullthrough from January 2019 to December2020 in our institution. High-resolution anorectal manometry (HRAM) was used to record anal resting pressure (ARP), length of high-pressure zone (HPZ), and presence/absence of recto-anal inhibitory reflex (RAIR). The Paediatric Incontinence/Constipation Scoring System (PICSS) was scored for all participants. PICSS is a validated questionnaire with scores mapped to an age-specific normogram to denote constipation, incontinence, and their combinations. Non-parametric and chi-square tests at significance p<0.05 were conducted to examine the relationship between PICSS categories and manometry findings. Ethical approval was obtained. RESULTS: There were 32 participants (30 boys). Median age at participation was 26.5 months (range: 13.8-156). Twenty-four (75%) had transanal pullthrough, 8(25%) underwent Duhamel procedure. PICSS scored 10(31.3%) as normal, 8(25%) as constipation, 10(31.3%) as incontinent, and 4(12.5%) as mixed. RAIR was present in 12 patients (37.5%). HPZ, maximum ARP, mean ARP were comparable across all PICSS groups without statistically significant differences. Presence of RAIR was not significantly associated with any PICSS groups (p = 0.13). CONCLUSION: Bowel function does not appear to be significantly associated with HRAM findings after definitive pullthrough for HD, but our study is limited by small sample size. RAIR was present in 37.5% patients after pullthrough. LEVEL OF EVIDENCE: Level II.

Outcomes of neonatal congenital diaphragmatic hernia in a non-ECMO center in a middle-income country: a retrospective cohort study.

BACKGROUND: Most studies examining survival of neonates with congenital diaphragmatic hernia (CDH) are in high-income countries. We aimed to describe the management, survival to hospital discharge rate, and factors associated with survival of neonates with unilateral CDH in a middle-income country. METHODS: We retrospectively reviewed the medical notes of neonates with unilateral CDH admitted to a pediatric intensive care unit (PICU) in a tertiary referral center over a 15-year period, from 2003-2017. We described the newborns' respiratory care pathways and then compared baseline demographic, hemodynamic, and respiratory indicators between survivors and non-survivors. The primary outcome measure was survival to hospital discharge. RESULTS: Altogether, 120 neonates were included with 43.3% (52/120) diagnosed antenatally. Stabilization occurred in 38.3% (46/120) with conventional ventilation, 13.3% (16/120) with high-frequency intermittent positive-pressure ventilation, and 22.5% (27/120) with high frequency oscillatory ventilation. Surgical repair was possible in 75.0% (90/120). The overall 30-day survival was 70.8% (85/120) and survival to hospital discharge was 66.7% (80/120). Survival to hospital discharge tended to improve over time (p > 0.05), from 56.0% to 69.5% before and after, respectively, a service reorganization. For those neonates who could be stabilized and operated on, 90.9% (80/88) survived to hospital discharge. The commonest post-operative complication was infection, occurring in 43.3%. The median survivor length of stay was 32.5 (interquartile range 18.8-58.0) days. Multiple logistic regression modelling showed vaginal delivery (odds ratio [OR] = 4.8; 95% confidence interval [CI] [1.1-21.67]; p = 0.041), Apgar score [Formula: see text] 7 at 5 min (OR = 6.7; 95% CI [1.2-36.3]; p = 0.028), and fraction of inspired oxygen (FiO2) < 50% at 24 h (OR = 89.6; 95% CI [10.6-758.6]; p < 0.001) were significantly associated with improved survival to hospital discharge. CONCLUSIONS: We report a survival to hospital discharge rate of 66.7%. Survival tended to improve over time, reflecting a greater critical volume of cases and multi-disciplinary care with early involvement of the respiratory team resulting in improved transitioning from PICU. Vaginal delivery, Apgar score [Formula: see text] 7 at 5 min, and FiO2 < 50% at 24 h increased the likelihood of survival to hospital discharge.

Utility of postoperative anorectal manometry in children with anorectal malformation: a systematic review.

Children with anorectal malformation (ARM) often continue to have disturbances in bowel function long after reconstructive surgery. Anorectal manometry may be utilized to evaluate bowel function in these children. We aimed to describe the reported protocols and manometric findings in children with ARM post-reconstructive surgery and to investigate the correlation between manometric evaluation and bowel functional outcome. PubMed, EMBASE, and Google Scholar databases were searched from 1980 to 2021. Data were reviewed and extracted independently by two authors, in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses. Included studies were English articles reporting postoperative assessment of children (≤ 18 years) with ARM using anorectal manometry. From 128 articles obtained in the initial search, five retrospective cohort studies and one prospective study fulfilled inclusion criteria. The rectoanal inhibitory reflex and mean anal resting pressure were parameters most often reported to correlate with postoperative bowel function. The least reported parameters among the studies were high-pressure zone, rectal volume, and rectal sensation. Anorectal manometry could be an objective method providing important information for personalized management of postoperative ARM patients with bowel function issues, but lack of standardized protocols limits a comprehensive analysis of their utility.

Intra-abdominal hypertension in neonates following congenital diaphragmatic hernia repair: Correlation with early postoperative respiratory and gastrointestinal outcomes.

BACKGROUND: Increased intra-abdominal pressure (IAP) is seen in patients after congenital diaphragmatic hernia (CDH) repair owing to reduction of thoracic contents into the relatively smaller abdominal cavity. In infants, IAP ≥11 mmHg is considered intra-abdominal hypertension (IAH). We aim to determine the incidence of IAH and its relationship with duration of ventilatory support, and gastrointestinal function post CDH repair. METHODS: We prospectively recruited all neonates who had CDH repair in four hospitals in Malaysia from June 2018 to October 2020. Intra vesical pressure was used as a proxy for IAP and was measured for 5 consecutive days post surgery. The daily median value was used for analysis. We categorized IAP as <11 mmHg (no IAH), 11-15 mmHg (IAH), and >15 mmHg (severe IAH). Incidence of IAH, its effects on the duration of ventilatory support, and gastrointestinal function were studied. RESULTS: There were 24 neonates included in this study. They were operated between day 1 and 6 of life (median: 4 days old). IAH was detected within the first 3 days post surgery, with 83% occurring on day one. Those requiring ventilatory support for more than 3 days contributed the largest proportion of IAH (n = 17, 71%). There was strong correlation between days of IAH and duration of ventilation (p < 0.001, r = 0.70). There was moderate correlation between days of IAH and duration taken to achieve full enteral feeding (p < 0.005, r = 0.70). CONCLUSION: IAP measurement is a safe and useful adjunct in post CDH monitoring and in predicting ventilatory support requirements and the time needed to establish feeding.

Effectiveness of table salt versus copper sulphate in treating umbilical granuloma: A pilot randomized controlled trial.

BACKGROUND: Umbilical granuloma is a common infantile condition which usually responds to non operative management. Copper sulphate (CuSO4) is often used as chemical cauterization but can cause superficial skin burns. An alternative is table salt (NaCl), which osmotically dehydrates wet granulation tissue causing necrosis. We aimed to compare the effectiveness of NaCl versus CuSO4 in treating umbilical granuloma. METHOD: We performed a multi centerrandomized controlled trial involving three regional pediatricsurgical units. We included children who presented with umbilical granuloma from December 2018 to May 2020. Children who received treatment prior to index visit were excluded. They were randomly allocated to receive NaCl (twice/day application for 5 days by caregiver) or CuSO4 (single application by clinician). Demographic data, compliance in the NaCl group by pill counting method, treatment outcomes, and complications were recorded. Treatment success was defined as complete lesion resolution. Partial or no response was considered treatment failure. Subsequent treatment then reverted to the respective center's routine management. RESULT: We recruited 70 participants with 6 dropouts (2 defaulters, 1 vitellointestinal duct, 3 urachal remnants), leaving 64 subjects for final analysis: 31 NaCl, 33 CuSO4. Compliance rate of 77.4% was recorded for NaCl, with 6 (20%) 'poor compliance' participants stopping therapy before completion owing to complete resolution. NaCl group had a significantly higher complete resolution rate (90.4%) compared to CuSO4 (69.7%), p = 0.040. No NaCl participant developed complications versus 9% (n = 3) in CuSO4 (periumbilical superficial skin burn). CONCLUSION: Table salt is an ideal treatment choice for umbilical granuloma as it is effective, safe, and readily available. LEVEL OF EVIDENCE: II.

Assess, adapt and act: a paediatric surgery division's initial approach in a rapidly evolving pandemic.

PURPOSE: The COVID-19 pandemic has placed an unprecedented test on the delivery and management of healthcare services globally. This study describes the adaptive measures taken and evolving roles of the members of the paediatric surgery division in a developing country during this period. METHODS: We adopted multiple adaptive strategies including changes to stratification of surgeries, out-patient services by urgency and hospital alert status, policy writing involving multidisciplinary teams, and redeployment of manpower. Modifications were made to teaching activities and skills training to observe social distancing and mitigate reduced operative learning opportunities. Roles of academic staff were expanded to include non-surgical duties. RESULTS: The planned strategies and changes to pre COVID-19 practices were successful in ensuring minimal disruption to the delivery of essential paediatric surgical services and training. Despite the lack of established guidelines and literature outlining strategies to address the impact of this pandemic on surgical services, most of the initial measures employed were consistent with that of other surgical centres. CONCLUSION: Changes to delivery of surgical services and surgical training warrant a holistic approach and a constant re-evaluation of practices with emergence of new experiences and guidelines.