Experiences of youth growing up with anorectal malformation or Hirschsprung's disease: a multicenter qualitative in-depth interview study.

PURPOSE: Patients with anorectal malformation (ARM) and Hirschsprung's disease (HD) live with long-term impact of these diseases even into adulthood. We aimed to explore the physical, social and emotional impact of these diseases in adolescents and young adults to develop best practices for transition care. METHODS: We conducted one-on-one in-depth interviews with ARM and HD patients aged ≥ 11 years who had undergone surgery at four tertiary referral centers. All interviews were audio-recorded and transcribed verbatim. We analyzed transcripts for recurring themes, and data were collected until data saturation was reached. Three researchers independently coded the transcripts for major themes using thematic analysis approach. RESULTS: We interviewed 16 participants (11 males) between October 2022 and April 2023. Ages ranged from 11 to 26 years. Five major themes emerged: (1) personal impact (subthemes: physical, emotional and mental health, social, school), (2) impact on family, (3) perceptions of their future (subthemes: relationships, career, state of health), (4) sources of support (subthemes: family, peers, partner), and (5) transition care (subthemes: concerns, expectations). Only females expressed concerns regarding future fertility. CONCLUSION: This study highlights the evolving problems faced by adolescents and young adults with ARM and HD, especially gender-specific concerns. Our findings can inform efforts to provide individualized care.

Treatment of megarectum in anorectal malformation with emphasis on preventive aspects: 17 years experience.

PURPOSE: Megarectum in anorectal malformation (ARM) causes severe morbidity. To compare conservative management (CM) of megarectum with excision (EX), to propose a new classification and to analyse management strategies. METHODS: Between 2000-2016, we reviewed all ARM to identify megarectum, defined by radiological recto-pelvic ratio > 0.61. A new classification was proposed: primary megarectum (PM) pre-anorectoplasty, and secondary megarectum (SM) post-anorectoplasty, with sub-types. Complications and Krickenbeck bowel function were compared between CM and EX. RESULTS: Of 124 ARM, 22 (18%) developed megarectum; of these, 7 underwent EX. There was no difference in functional outcomes when comparing CM vs EX-voluntary bowel movement (both 86%), soiling (40% vs. 57%) and constipation (both 86%). However, EX was associated with major complications (43%) and the requirement for invasive bowel management, compared to CM (85% vs. 27%, P = 0.02). 6/7 EX needed antegrade continence enema (ACE), one of these has a permanent ileostomy. With strategic changes, incidence of megarectum reduced from 20/77 (26%) to 2/47 (4%) after 2013 (P = 0.002). CONCLUSION: EX did not confer benefit in the functional outcome but carried a high risk of complications, often needing ACE or stoma. By adhering to strategies discussed, we reduced the incidence of megarectum and have avoided EX since 2013.

Duhamel operation for children with distal colonic dysmotility.

PURPOSE: To report outcomes of children with constipation refractory to medical management and manometrically proven distal colonic dysmotility, managed with rectosigmoidectomy followed by Duhamel operation (Duhamel). METHODS: Children who underwent a Duhamel from 2009 onwards for intractable constipation and left colonic dysmotility were retrospectively reviewed. The primary end point was resolution of constipation, and secondary end point was postoperative complications. Continuous data were median (range). RESULTS: 11 patients (4 males) had Duhamel at 11 years (5-16) with constipation started from 2 years (1-8). Hirschsprung's disease was excluded. All Duhamels were performed with a covering ileostomy: 9 following a Hartmann procedure, one following a previously failed reversal of Hartmann, and one Duhamel performed with a pre-existing ileostomy. All ileostomies were subsequently closed. Median resection length was 22 cm (11-31). Length of stay was 8 days (5-23). Follow-up was 5 years (0.5-7). Age at final review was 15 years (10-18). Resolution of constipation occurred in nine patients (4 required antegrade continence enemas (ACE), 5 with laxative); two had persistent constipation and faecal incontinence despite ACE, ultimately requiring an ileostomy. Two postoperative small bowel obstructions required laparotomy. CONCLUSION: Duhamel performed in children with manometrically proven distal colonic dysmotility yielded 82% resolution of refractory constipation; half of them subsequently needed ACE.

Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis.

OBJECTIVE: To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy. STUDY DESIGN: PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic. RESULTS: From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Postoperative mortality after Ladd procedure mainly was associated with cardiac insufficiency, and overall it was significantly greater in the emergency group compared with the prophylactic group (18% vs 5.6%). The complication rate also was greater in case of emergency vs prophylactic abdominal surgery (27% vs 16%); adhesional small bowel obstruction was the most common complication overall (6%). CONCLUSION: The screen-detected incidences of IRA and acute midgut volvulus were significantly greater in heterotaxy than the normal population. Prophylactic Ladd procedure was associated with less morbidity and mortality compared with emergency surgery. A long-term prospective randomized trial is needed to define the indication for screening and prophylactic treatment of IRA in heterotaxy.

Coagulation Gene Expression Profiling in Infants With Necrotizing Enterocolitis.

OBJECTIVES: Coagulopathy and mesenteric thrombosis are common in premature neonates with necrotizing enterocolitis (NEC). This pilot study aimed to investigate the hypothesis that there are changes in the gene expression related to the coagulation and anticoagulation systems in NEC. METHODS: Consecutive neonates (n = 11) with NEC (Bell stages 2-3) were recruited. Two comparison groups, matched for birth weight and corrected gestational age, were selected based on the absence of inflammation and coagulopathy (healthy control, n = 10), or the presence of a confirmed blood infection (sepsis control, n = 12). A pathway-specific quantitative polymerase chain reaction array was used to determine the expression of 94 genes involved in human blood coagulation and anticoagulation cascade. RESULTS: Twelve genes of the coagulation and anticoagulation systems were significantly altered in the patients with NEC compared with healthy controls. In particular, neutrophil elastase, CD63, PROS1, HGF, and F12 were significantly upregulated (mean fold changes [FCs] +2.74, P < 0.05) with an overall procoagulant effect; MFGE8, factor II (thrombin) receptor-like 1 (F2RL1), FGL2, PLAT, PROCR, SERPIND1, and HNF4A were significantly downregulated (mean FCs -2.45, P < 0.05) with a reduction in fibrinolysis and endothelial regeneration. In the comparison between NEC and sepsis, we did observe a significant difference in expression of F2RL1 (FC -2.50, P = 0.01). CONCLUSIONS: We have identified potential biomarkers associated with coagulopathy and disease progression in NEC. In particular, the overall procoagulant status, at the transcriptional level, should be further investigated to unveil molecular mechanisms leading to intestinal necrosis, multiorgan failure, and death.

Extensive Necrotising Enterocolitis: Objective Evaluation of the Role of Second-Look Laparotomy in Bowel Salvage and Survival.

AIM: We investigated the role and outcome of a planned second-look laparotomy (SLL) in preserving bowel in extensive necrotizing enterocolitis (NEC). METHODS: Extensive NECs managed surgically in a tertiary centre in 2006-2009 were retrospectively studied to include patients planned for an SLL. End points were bowel salvage rate and survival outcomes. Results were median (ranges), and statistical significance was P < 0.05. MAIN RESULTS: In 4 years, 34 NECs required a laparotomy, and 9 extensive NECs who required an SLL were included. The gestation at birth was 27 (24-38) weeks, birth weight was 1120 (580-2835) g, and first laparotomy performed on day 34 (2-77) of life, with SLL performed 2 (1-3) days after initial laparotomy. Commonest indications for SLL were doubtful bowel viability and physiological instability. 3 died before SLL. Patients who survived to have an SLL (n = 6) had remaining small bowel length of 41 (25-70) cm, overall small bowel salvage rate 51 % (0-100 %), and 30-day survival 5/6 (83 %). Four patients survived for 1 year, their length of NICU stay was 114 (76-120) postoperative days, time on PN was 84 postoperative days (71 days-17 months), including one patient with short bowel syndrome who achieved enteral autonomy at 17 months; one late mortality had short bowel syndrome after further bowel resection for bowel obstruction, developed intestinal failure associated liver disease, and died before 1 year of life following liver transplant. CONCLUSION: SLL is a viable approach for extensive NEC. It offered bowel salvage rate of 51 % and long-term PN-free survival of 44 %, in the patient group who would have had significant risk of mortality and major morbidity.

Identifying Risk Factors for Surgical Site Infection After Stoma Closure in Infants for Targeted Implementation of Surgical Site Infection Reduction Bundle.

Background: To determine risk factors for surgical site infection (SSI) in infants after stoma closure, to identify at-risk patients, plan timing of surgery, and implement SSI-reduction strategies. Patients and Methods: A single center retrospective comparison study of all children less than one year of age who underwent enterostomy closure (2018-2020) with SSI diagnosed through a prospective surveillance program, using criteria from Public Health England (PHE). Demographics and risk factors, types of SSI, systemic sepsis, mortality and length of stay were compared between SSI and non-SSI. Significant factors associated with SSI were analyzed in a multivariate binomial logistic regression model. Results: Eighty-nine stoma closures were performed, most commonly for necrotizing enterocolitis (NEC) and anorectal malformation. Fourteen had SSI (16%): 12 superficial and two deep; three developed systemic sepsis, but no 30-day mortality. Surgical site infection was associated with NEC (12/14 vs. 32/75; p = 0.003), younger age (median 76 vs. 89 days; p = 0.014), lower corrected gestation (cutoff: 39 weeks gestation; 11/14 vs. 27/75; p = 0.004) and lower weight (cutoff: 2.2 kg; 7/14 vs. 16/75; p = 0.032), compared with non-SSI. After correcting for age, gestation, and weight, logistic regression showed NEC was an independent predictor for SSI (odds ratio [OR], 12; 95% confidence interval [CI],1.2-125). The at-risk cohort (n = 56; 63%) had seven-fold increased risk of SSI and four-fold longer hospital stay, which may be the target for SSI-reduction strategies. Conclusions: Necrotizing enterocolitis-related stoma closure is at increased risk for SSI. Considerations for delaying stoma closure until achieving 39 weeks gestation or 2.2 kg in weight may further reduce SSI. Targeting SSI-reduction strategies using these criteria may improve resource-rationalization.

Comparing Consistency and Usability of Common Bowel Function Scoring Systems in Anorectal Malformation Patients.

BACKGROUND: Assessment of postoperative bowel function in anorectal malformation (ARM) patients is crucial for benchmarking outcomes. We compared existing bowel function scoring systems in various aspects in patients with ARM. METHODS: With ethical approval, this was a cross-sectional study involving 5 paediatric surgery referral centres in Malaysia, comparing the Kelly, Japanese Study Group of Anorectal Anomalies (JSGA), Holschneider and Krickenbeck bowel function questionnaires. We recruited patients aged 4-17 years, who had completed definitive surgery & stoma closure (where relevant) > 12 months prior to participation. We standardised outcomes of each scoring system into categories ('good', 'fair', 'poor' and 'very poor') to facilitate comparison. Parents & patients were surveyed and asked to rate the ease of understanding of each questionnaire. The difference in protocol scores rated between parents and patients were compared. Association of each bowel function scoring protocol with type of anomaly was assessed. Statistical significance was p < 0.05. RESULTS: Thirty-nine parents (21 mothers, 18 fathers) and 23 patients were included in this study. Fair agreement was found between Kelly and Krickenbeck protocols (κ = 0.343; p < 0.001), between JSGA constipation and Holschneider protocols (κ = 0.276; p = 0.002); JSGA constipation and Krickenbeck protocols (κ = 0.256; p = 0.004); and between Holschneider and Krickenbeck protocols (κ = 0.273; p = 0.003). Only the Kelly protocol showed significant correlation between parents and patients' answers (ρ = 0.459, p = 0.028). Krickenbeck demonstrated the best negative correlation of patients' scores with ARM types (ρ = -0.401, p = 0.001). The Kelly protocol ranked highest when comparing ease of understanding. CONCLUSION: All the questionnaires appeared comparable in assessing postoperative faecal continence in ARM patients. The Kelly questionnaire performed best in 3 key areas of assessment. LEVEL OF EVIDENCE: Level III Cross-Sectional Study.

Transitional Care in Anorectal Malformation and Hirschsprung's Disease: A Systematic Review of Challenges and Solutions.

BACKGROUND: The literature on transitional care in anorectal malformation (ARM) and Hirschsprung's disease (HD) is diverse and heterogeneous. There is a lack of standards and guidelines specific to transitional care in these conditions. We aim to establish and systematically categorize challenges and solutions related to colorectal transition care. METHODS: Systematic review of qualitative studies from MEDLINE, EMBASE, PubMed and Scopus databases (2008-2022) was conducted to identify the challenges and solutions of healthcare transition specific to ARM and HD. Thematic analyses are reported with reference to patient, healthcare provider and healthcare system. RESULTS: Sixteen studies from 234 unique articles were included. Fourteen themes related to challenges and solutions, each, are identified. Most challenges identified are patient related. The key challenges pertain to: (1) patient's lack of understanding of their disorder, resulting in over-reliance on the pediatric surgical team and reluctance towards transitioning to adult services; (2) a lack of education and awareness among adult colorectal surgeons in caring for pediatric colorectal conditions and inadequate communication between pediatric and adult teams; and (3) a lack of structured transition program and joint-clinic to meet the needs of the transitioning patients. The key solutions are: (1) fostering young adult patient's autonomy and independence; (2) conducting joint pediatric-adult transition clinics; and (3) ensuring a structured and coordinated transition program is available using a standardized guideline. CONCLUSION: A comprehensive framework related to barriers and solutions for pediatric colorectal transition is established to help benchmark care quality of transitional care services. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Systematic review without meta-analysis.

Exomphalos with intestinal fistulation: Case series and systematic review for clinical characterization, management and embryopathogenesis.

INTRODUCTION: Exomphalos with intestinal fistulation (EIF) is a rare variant of exomphalos with intestine opening to the surface of an intact sac, and may result in a diagnostic challenge. We report 3 new cases and conducted a systematic review of the literature, to characterize its association with the type of exomphalos and vitellointestinal duct (VI) as well as to evaluate its management and outcomes. METHODS: A literature search from PubMed using keywords pertaining to exomphalos and fistulation was used to identify all unique cases reported between 1950 and 2020, in addition to the case series reported here, to establish the clinical presentation, histological findings, management and outcomes. RESULTS: We found a total of 28 cases of EIF, of which 25 had been reported in 70 years from 19 reports. There was a male predominance (4-to-1 ratio). The majority presented as an exomphalos minor (n = 23, 82%) with a prolapsing patent VI duct (n = 16, 57%), most had evidence of Meckel's diverticulum (n = 25, 89%). All but one case were managed by fistula excision with or without ileal resection and anastomosis, followed by primary closure of the abdominal wall defect. All patients, except one with Trisomy 13 who received only palliative care, underwent surgery. Post-operative complications occurred in 7 patients (25%). Congenital anomalies were present in 12 (43%) and none had Beckwith-Wiedemann syndrome. Mortality occurred in 4 patients (14%) between 3 and 17 days. One EIF with exomphalos major failed early conservative treatment due to sac disintegration and sepsis, requiring staged closure, but had a good outcome. DISCUSSION: EIF is a rare entity usually associated with exomphalos minor and vitelline duct involvement. EIF presentation is variable but primary surgery is the mainstay of treatment with generally good outcomes. Common features of EIF suggest a different embryopathogenesis to other forms of exomphalos.

Vanishing enteric duplication cyst presenting with melaena.

A toddler presented with melaena on a background of a possible enteric duplication cyst, diagnosed in the neonatal period which 'vanished'. What was later confirmed to be a small bowel duplication cyst was not seen on follow-up ultrasounds and thus the patient was managed expectantly until presentation as an emergency. Our case highlights the challenges faced in managing cystic abdominal structures in infancy and the potential consequences of expectant management. We discuss whether cross-sectional imaging or diagnostic laparoscopy should be advocated when duplications cysts are considered amongst the differentials of a lesion on ultrasound.

Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes.

INTRODUCTION: Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its definition remains unclear and its implication on HD-related core outcomes has not been fully reported. METHODS: A single-center 20-year series was reviewed to include HD with follow-up of 1 year or more post pull-through (PT) and aged 5 years or older. We investigated six core outcomes derived from NETS1HD study by comparing the groups dichotomized by four time points using age at diagnosis (44-week gestation, 6 months, 1 year, and 3 years). Following establishment of definition of LDHD, the outcomes and complications were compared with timely diagnosis of HD (TDHD). RESULTS: Forty-nine out of eighty-six HD were included. The definition of LDHD was found to be HD diagnosed at 1 year of age or later because 3/6 core outcomes were significantly worse than TDHD. Nine patients (18%) had LDHD-median age at diagnosis 42 months (12-89) and PT performed at 57 months (12-103), mostly Soave (73%); a covering stoma was performed in 7/9, significantly higher than TDHD in 10/40 (p = 0.001). LDHD was associated with increased unplanned surgery (78% vs. 30%, p = 0.019), fecal incontinence (100% vs. 62%, p = 0.01), and permanent stoma (33% vs. 5%, p = 0.037). Major complications (56% vs. 20%, p = 0.043) and redo PT (33% vs. 5%, p = 0.037) were also higher in LDHD. CONCLUSION: LDHD could be defined as HD diagnosis at or over 1 year of age. LDHD was associated with more preoperative stoma, major postoperative complications, unplanned reoperation, and worse HD-related core outcomes.

A Critical Analysis of Rectal Biopsy to Exclude Hirschsprung's Disease.

INTRODUCTION: Most Hirschsprung's disease (HD) are diagnosed in young children with increased risk ("red flag"). Older children (>6 months) require open rectal biopsy (ORB) with its own impact on risk and resources. We investigated if "red flag", age, and sex used in combination could exclude HD. MATERIALS AND METHODS: "Red flags" are risk factors associated with HD, including neonatal bowel obstruction, genetic association, failure of passage of meconium in <48 hours, infantile constipation, distension with vomiting, or family history. All rectal biopsies (2015-2018) were reviewed for indications, methods, and histopathological findings. Logistic regression analysis was adopted to assess predictive value of "red flag," age, and sex (p < 0.05* was significant). RESULTS: A total of 187 children underwent 84 suction rectal biopsies and 113 ORBs (n = 197 in total). Final histopathological diagnoses were non-HD (n = 154) and HD (n = 43). Total 78% of rectal biopsies were non-HD, of which 63% by ORB. Non-HD was associated with absence of "red flag" (49 vs. 16%*), increased age at biopsy (22 months vs. 28 days*), >6 months old (62 vs. 30%*), and female gender (54 vs. 16%*), compared with HD. In the absence of "red flag," 7/82 (9%) had HD (negative predictive value = 91%). Logistic regression analysis found absent "red flag" predicted non-HD biopsy with odds ratio 4.77 (1.38, 16.47), corrected for age and sex. CONCLUSION: Negative rectal biopsy rate for HD is very high. The majority required ORB. Although "red flag" and gender, but not age, have strong predictive values, it is inadequate for excluding HD. This study supports the need for alternative strategies in excluding HD.

Long-term urology outcomes of anorectal malformation.

INTRODUCTION: Urological problems are a recognised feature of anorectal malformation (ARM). Previous assumptions of favourable long-term urinary outcomes are being challenged. OBJECTIVE: We hypothesised that urinary tract problems are common in ARM and frequently persist into adulthood. We retrospectively reviewed long-term renal and bladder outcomes in ARM patients. STUDY DESIGN: Patients with ARM born between 1984-2005 were identified from electronic hospital databases. Their case notes were reviewed. Renal outcomes included serum creatinine and the need for renal replacement therapy. Bladder outcomes included symptom review, bladder medication, need for intermittent catheterisation, videourodynamics and whether the patient had undergone augmentation cystoplasty. RESULT (TABLE 1): The case notes of 50 patients were reviewed. The median age at last follow up was 18 years (range 12-34 years). The level of fistula was noted to be high in 17 patients, intermediate in eight, and low in 10. Four had cloaca. Congenital urological abnormalities were present in 25 (50%). An abnormal spinal cord was present in 22 (44%) patients. VACTERL association occurred in 27 (54%). Chronic kidney disease stage II or above was found in 14 (28%) patients, of whom four required a renal transplant. Abnormal bladder outcomes were found in 39 (78%) patients. Augmentation cystoplasty with Mitrofanoff had been performed in 12. Of those who had not undergone cystoplasty, 17 had urinary symptoms, including urinary incontinence in 12. Of the 39 patients with abnormal bladder outcome, 19 (49%) did not have a spinal cord abnormality. There was no significant statistical association between level of ARM and abnormal renal outcome or presence of bladder abnormality. DISCUSSION: Adverse renal and bladder outcomes are common in our cohort of young people with ARM with a significantly higher incidence compared with current literature. We did not demonstrate an association between level of ARM or presence of spinal cord anomaly with persistent bladder problems. Congenital urological anomalies are more common in those who later have an abnormal renal outcome. Although this difference is statistically significant, one fifth of patients born with anatomically normal upper tracts develop reduced renal function, implying an important acquired component. CONCLUSION: Bladder problems and reduced renal function affect a significant proportion of young adults with ARM. Neither adverse outcome is reliably predicted from ARM level, congenital urological anomaly or spinal cord anomaly. We advise continued long-term bladder and kidney follow-up for all patients with ARM.

Optimal management of gastrojejunal tube in the ENFit era - Interventions that changed practice.

BACKGROUND: We experienced a high incidence of jejunal tube (JEJ) displacement in children who underwent percutaneous endoscopic transgastric jejunostomy (PEGJ), ever since the introduction of ENFit connector (2017). METHODS: Two interventions were introduced in 2018 - fixative suture to PEGJ ENFit connector, and conversion to balloon transgastric-jejunal feeding device (Balloon GJ) whenever possible. Children receiving PEGJ and Balloon GJ in 2.8 years were categorized into 3 eras: 2016 (pre-ENFit), 2017 (ENFit) and 2018 (interventional), for comparison of complications and sequelae. Kaplan-Meier survival curves with log-rank test (P < 0.05) were applied. RESULTS: 100 children underwent 323 JEJ insertions - PEGJ (n = 237), Balloon GJ (n = 86). Complications occurred in 188 JEJs (58%), more frequently with PEGJ than Balloon GJ (69% vs. 29%, P < 0.0005). PEGJ had higher complication/1000-tube-days (6 vs. 0, P < 0.0005). In 2018, complication rate reduced from 76% to 30% (P < 0.0005) owing to effectiveness of PEGJ connector suture application (P = 0.019), and increased utilization of Balloon GJ (16% to 44%, P = 0.005). Balloon GJ showed better JEJ survival (P = 0.019), less morbidity (emergency attendance, X-ray) and greater cost-effectiveness than PEGJ. CONCLUSIONS: Balloon GJ had better overall outcomes than PEGJ. Suture application to connector successfully reduced JEJ internal displacement in PEGJ; however, conversion to Balloon GJ should be strongly considered. LEVEL OF EVIDENCE: II.

Carpal scaphoid fracture in the skeletally immature: a single centre one-year prospective study.

We report up-to-date one-year prospective data on the incidence of scaphoid fracture in skeletally immature patients managed in a Fracture Clinic of a Level I Trauma Centre in a University Hospital in the UK. All scaphoid fractures were immobilised until union was achieved. One hundred twenty one skeletally immature patients were referred for a suspected fracture of the scaphoid. Fourteen patients (11.5%) did have a scaphoid fracture, an incidence of 15 per 100,000 (0.55% of all paediatric injuries referred). Mean delay in being seen was 2.6 days, and mean follow-up time was 52.3 days. Only plain radiography was used to diagnose and follow up scaphoid fractures. Fractures of the scaphoid in skeletally immature individuals are uncommon, are usually undisplaced, occur more commonly in the distal portion of the bone, and carry a good prognosis.

Prognostic value of the oxygenation index to predict survival and timing of surgery in infants with congenital diaphragmatic hernia.

AIMS: To compare the mean oxygenation index on day1 (MOId1) with the best OI on day1 (BOId1) in predicting 30-day mortality in congenital diaphragmatic hernia (CDH). In addition, to determine whether serial OIs in the first 48 h after birth and preoperative OI (PreOp-OI), were associated with optimal timing of surgery, ventilation requirement and hospital stay in infants with CDH. METHODS: The medical records of infants with antenatally diagnosed CDH during 2009-2015 were retrospectively reviewed. Receiver operator characteristic curves were constructed to identify MOId1 and BOId1 cutoff levels to predict 30-day mortality. In those who underwent surgery, the mean OI on each of the first two days (MOId1, MOId2) and PreOp-OI were correlated using Spearman (rs) with the age at surgery, the duration of ventilation and length of stay. Statistical significance was P < 0.05* and < 0.001**. RESULTS: Survivors (n = 44) and nonsurvivors (n = 24) were comparable in gestational age, birth weight and defect laterality. Nonsurvivors had higher median BOId1 (15.4 vs 2.9; P < 0.01) and MOId1 (48 vs 7.5; p < 0.01) than survivors. Mortality was best predicted by two cutoffs [BOId1 > 6 (sensitivity 92%, specificity 89%); and MOId1 > 17 (sensitivity 96%, specificity 96%)]. Forty-four infants underwent surgery at a median postnatal age of five (range 2-19) days. MOId1 and MOId2 both correlated significantly with the age at surgery (rs = 0.4**, rs 0.5**) but not ventilation period and length of stay. PreOp-OI correlated significantly with age at surgery, duration of ventilation and length of stay (rs = 0.32*, rs = 0.47**, rs = 0.37*). A PreOp-OI <3 was predictive of optimal timing for surgery with improved duration of ventilation (8 vs 22, P = 0.001) and length of stay (26 vs 47, P = 0.004). However, 11/44 (25%) patients would not achieve a PreOp-OI < 3 by day 7+ of life and might still require surgery, one of them died. CONCLUSION: Both MOId1 and BOId1 are highly predictive of mortality in CDH. Oxygenation indices in the first 48 h poorly predicted the timing of surgery. PreOp-OI <3 may be a cutoff for optimal timing for surgery in infants for CDH repair. TYPE OF STUDY: Prognostic Study. LEVEL OF EVIDENCE: Level III.

Morgagni hernia repair in children over two decades: Institutional experience, systematic review, and meta-analysis of 296 patients.

BACKGROUND/PURPOSE: Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch. METHODS: We retrospectively reviewed all cases of MH who underwent first-time repair in 2012-2017 in our institution to determine recurrence and complication rate. A MEDLINE search related to minimally invasive surgery (MIS) and patch repair of MH was conducted for systematic review. Eligible articles published from 1997-2017 with follow-up data available were included. Primary outcomes measured were recurrence and complication. Meta-analysis to compare open versus MIS and primary versus patch repair in the MIS group were performed in comparative cohorts. Continuous data were presented as median (range), and statistical significance was P<0.05. RESULTS: In our institution, 12 consecutive patients aged 17-month-old (22 days-7 years), underwent laparoscopic patch repair of MH, with one conversion to laparotomy. No recurrence or significant complication occurred over a follow-up period of 8 months (1-48 months). Thirty-six articles were included from literature review and were combined with the current series. All were retrospective case reports or series, of which 6 were comparative cohorts with both MIS and open repairs. A total of 296 patients from 37 series were ultimately used for analysis: 80 had open repair (4 patch) and 216 had MIS repair (32 patch), with a patch rate of 12%. There were 13 recurrences (4%): no difference between open and MIS repairs (4/80 vs 9/216, p=0.75); recurrence rate following primary repair was 13/260 (5%), but no recurrence occurred with 36 patch repairs. Meta-analysis showed no difference in recurrence between open and MIS repair (p=0.83), whereas patch repair was associated with 14% less recurrence compared with primary repair, although it did not reach statistical significance (p=0.12). There were 13 complications (5%): no difference between open and MIS repairs (5/80 vs 8/216, p=0.35). One small bowel obstruction occurred in a patient who had laparoscopic patch repair. CONCLUSION: In MH, recurrence and complication rates are comparable between MIS and open repairs. Use of patch appeared to confer additional benefit in reducing recurrence. TYPE OF STUDY: Systematic review LEVEL OF EVIDENCE: 3A.

The management of boys under 3 months of age with an inguinal hernia and ipsilateral palpable undescended testis.

AIMS: The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies. METHODOLOGY: We undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005-2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p<0.05 was considered significant. RESULTS: Survey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic. Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36-65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred. CONCLUSION: This study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age.

Using serial oxygenation index as an objective predictor of survival for antenatally diagnosed congenital diaphragmatic hernia.

INTRODUCTION: Best oxygenation index on day 1 (BOId1) had been shown to predict survival in congenital diaphragmatic hernia (CDH). Serial oxygenation index (OI) may enable better assessment of response to cardiorespiratory support than BOId1. METHODS: All antenatally diagnosed CDH from one tertiary neonatal unit were retrospectively reviewed. Oxygenation index at 6, 12, 24, and 48 hours from birth, as well as BOId1, were compared between survivors and nonsurvivors. The area under the curve and receiver operating characteristic (ROC) curves were used to compare serial OI within the first 24 hours and BOId1 between survivors and nonsurvivors. Statistical significance was set at P < .05. RESULTS: Twenty-four patients with CDH (13 survivors, 11 nonsurvivors) were included. Both groups were comparable in demographics and variables that could affect outcome. In terms of nonsurvival, ROC curve analysis demonstrated a sensitivity of 78% for serial OI greater than 252 and 56% for BOId1 greater than 8.5, both having a specificity of 100%. The area under the ROC curve for serial OI and BOId1 were 0.96 and 0.85, respectively. The positive predictive value of serial OI (>252) and BOId1 (>11) for nonsurvival were both 100%, with an negative predictive value of 87% and 76%, respectively. CONCLUSIONS: Our preliminary study showed that serial OI in the first 24 hours of life is a good predictor of survival. It is simple to use and has the added advantage of assessing response to medical support in CDH. The results support the need for a large prospective study exploring the potential of serial OI to guide management and prognosis.