Mental Health Outcomes of Mothers of Children With Congenital Gastrointestinal Anomalies Are Similar to Control Mothers: A Longitudinal Retrospective Cohort Study.

PURPOSE: Mothers of infants born with congenital gastrointestinal surgical anomalies experience a unique caregiving role. Whether these challenges result in more mental health diagnoses than the general population is unknown. This study assessed mental health diagnoses in mothers of children born with congenital surgical anomalies (CSA) compared to mothers of children without CSA. METHODS: A total of 780 mothers of children with CSA (case-mothers) and 6994 control-mothers were included. Mental health diagnoses were obtained using International Classification of Diseases 9 codes from an administrative database. Multivariate hazard ratios (HR) of mental health diagnoses were determined for mothers following childbirth, controlling for socioeconomic status, mental health diagnoses prior to childbirth, and age at childbirth. Subgroup analyses were completed for anxiety, depression, and post-traumatic stress disorder (PTSD). The association between a woman's mental health history and becoming a case-mother was analyzed using risk ratios (RR). RESULTS: Case-mothers were not at increased risk of mental health disorders compared to controls (HR = 1.00; CI95 = 0.92-1.09). In aggregate analysis, no increased risk of anxiety, depression, or PTSD was found. Subgroup analyses revealed that congenital diaphragmatic hernia case-mothers had an increased risk of depression (HR = 1.43; CI95 = 1.08-1.88). No other case-mothers were at increased risk of mental health diagnoses, anxiety, depression, or PTSD. Only omphalocele was associated with mental health diagnoses before childbirth (RR = 1.89; CI95 = 1.58-2.26). CONCLUSIONS: Collectively, mothers of children with CSAs did not have a greater risk of a mental health disorder, anxiety, depression, or PTSD compared to control-mothers. Only mothers of children with congenital diaphragmatic hernia had an increased risk of depression. LEVEL OF EVIDENCE: II.

Prevalence of Mental Health Disorders in Individuals With Gastrointestinal Congenital Surgical Anomalies: A Systematic Review and Meta-analysis.

PURPOSE: Studies examining functional outcomes and health-related quality of life for patients with congenital surgical anomalies (CSA) are increasingly common. However, the prevalence of mental health disorders in this population has not been determined. The purpose of this review is to summarize the reported prevalence of mental health disorders in children born with gastrointestinal CSA. METHODS: A systematic review of the literature was conducted on Medline (OVID), EMBASE (OVID), CINAHL (EbscoHost), and Scopus to identify studies reporting mental health diagnoses in children with a gastrointestinal CSA. A meta-analysis of the prevalence of anxiety disorders and depressive disorders was completed. RESULTS: Of 2546 manuscripts, seven met the inclusion criteria. All included manuscripts looked at patients with anorectal malformation (ARM) and/or Hirschsprung disease (HD). No studies assessed other gastrointestinal CSAs. A total of 183 psychiatric diagnoses were reported in 1167 patients. A pooled analysis of ARM patients revealed a 14.0% prevalence of anxiety disorders (N = 208; CI95 = 4.4-23.4%) and an 18.75% prevalence of depressive disorders (N = 48; CI95 = 7.7-29.8%). A pooled analysis of HD patients revealed a 15.8% prevalence of anxiety disorders (N = 19; CI95 = 0.6-32.2%) and a 4.75% prevalence of depressive disorders (N = 758; CI95 = 3.23-6.26%). CONCLUSIONS: There is a paucity of evidence on mental health outcomes of individuals born with gastrointestinal CSA. However, the available evidence suggested a high prevalence of mental health diagnoses in individuals born with ARM or HD with 1 in 7 having an anxiety disorder. Additionally, 1 in 5 ARM patients and 1 in 20 HD patients were diagnosed with a depressive disorder. TYPE OF STUDY: Systematic review and meta-analysis. LEVEL OF EVIDENCE: IV.

Post-discharge follow-up of congenital duodenal obstruction patients: a systematic review.

PURPOSE: Long-term follow-up of congenital duodenal obstruction patients often falls on care providers with little experience of this condition. We performed a systematic review of the long-term outcomes of duodenal obstruction and provide a summary of sequelae care providers should anticipate. METHODS: In 2022, after registering with PROSPERA, Medline (Ovid), EMBASE, PSYCHINFO, CNAHL and SCOPUS databases were searched using the title keyword 'intestinal atresia'. Abstracts were filtered for inclusion if they included the duodenum. Papers of filtered abstracts were included if they reported post-discharge outcomes. Methodological Index for Non-Randomized Studies was used to grade the papers. RESULTS: Of the 1068 abstracts were screened, 32 papers were reviewed. Eleven studies were included. Thirty additional papers were included after reviewing references, for a total of 41 papers. The average MINORS was 7/16. CONCLUSION: There is good evidence that children with congenital duodenal obstruction do well in terms of survival, growth and general well-being. Associated cardiac, musculoskeletal and renal anomalies should be ruled-out. Care providers should be aware of anastomotic dysfunction, blind loop syndrome, bowel obstruction and reflux. Reflux may be asymptomatic. Laparoscopic repair does not change long-term outcomes, and associated Trisomy 21 worsens neurodevelopmental outcomes.

Preventing severe necrotizing enterocolitis: Propensity score analysis of interventions associated with surgical NEC or NEC-associated death.

PURPOSE: To determine what modifiable interventions used in the neonatal intensive care unit (NICU) are associated with severe necrotizing enterocolitis (NEC) requiring surgical intervention. METHODS: A retrospective review of patients treated for NEC at a tertiary hospital from 1991 to 2016 was performed. Patient characteristics were used to calculate propensity scores for likelihood of exposure to seven interventions: enteral feeds, use of glucocorticoids, nonsteroidal anti-inflammatory drugs (NSAIDs), antacids, antibiotics, or umbilical arterial (UAC) and venous catheters (UVC). Conditional logistic regression was used to compare the odds of having surgical NEC if exposed to each treatment. RESULTS: We included 195 NEC patients: 69 severe NEC managed with surgery and 126 non-severe NEC managed medically. After propensity score matching based on birth characteristics, exposure to glucocorticoids (OR 5.21, 95%CI: 1.62, 16.70), NSAIDs (OR 4.87, 95%CI: 1.67, 14.17), UVC (OR 2.53 (95%CI: 1.19, 5.73), and UAC (OR 4.91, 95%CI: 2.12, 11.37) were associated with surgical NEC in separate conditional logistic regression analyses. Including these treatments in a second round of propensity matching and conditional logistic regression revealed that glucocorticoids (OR 2.99, 95%CI: 1.01, 8.88), NSAIDs (OR 3.97, 95%CI: 1.41, 11.19), UVC (OR 3.07, 95%CI: 1.46, 6.48), and UAC (OR 5.10, 95%CI: 2.10, 12.36) were still associated with surgical NEC. CONCLUSION: After controlling for birth confounders and common NICU supportive interventions, use of glucocorticoids, NSAIDs and umbilical catheters independently increased the odds of developing severe NEC requiring surgical intervention. LEVEL-OF-EVIDENCE RATING: Case-control, Level III evidence.

Educational and mental health outcomes in school-aged children with anorectal malformations: A case-control cohort study.

PURPOSE: Outcome studies for patients with anorectal malformation (ARM) have focused on fecal incontinence and quality of life, but a comparison of educational outcomes between ARM cases and controls has not been reported. The purpose of this study was to assess real-world educational outcomes, neurodevelopmental disorders and mental health disorders in ARM patients and compare to an age-matched control group. METHODS: We performed a retrospective case-control study of children diagnosed with ARM from 1991 to 2017. We evaluated educational outcomes using an Early Developmental Instrument, Grades 3, 7, and 8 assessments, Grade 9 completion and performance, and high school graduation. Neurodevelopmental and mental health disorders were compared using International Classification of Diseases codes available from a population-based dataset. RESULTS: A total of 96 ARM cases and 960 controls were identified. Cases were at greater risk of failing to meet expectations on Grades 7 and 8 assessments. After entering high school, ARM patients were at no greater risk than their peers of failing to meet expectations. Cases were more likely to have a developmental or intellectual disability (OR 3.59, p < 0.001), anxiety (OR 1.86, p = 0.023), depression (OR 2.35, p = 0.022) or hyperactivity disorder (OR 2.01, p = 0.036). CONCLUSIONS: Our study demonstrated that ARM patients may be more likely to perform poorly in junior high school than controls and may be at greater risk of neurodevelopmental and mental health disorders. It is important for pediatric surgeons to anticipate these challenges and endorse psychosocial supports to optimize educational and mental health outcomes. LEVELS OF EVIDENCE: Level-Ⅲ.

Effect of trisomy 21 on long-term gastrointestinal outcomes in duodenal atresia.

PURPOSE: We aimed to determine if Trisomy 21 (T21) affected gastrointestinal outcomes for children with duodenal atresia (DA). METHODS: We identified children born with DA between 1991 and 2017. Cases were divided into DA with T21 and DA without T21. Ten healthy controls per case were included. Esophageal, ulcerative, obstructive and stomach complaints were assessed. Risk ratios (RR), rate ratios (RaR) and Cox models were constructed. Analyses were performed for cases versus controls, and for T21 cases versus non-T21 cases. RESULTS: DA cases totaled 52: 22 had T21 and 30 did not. There were 520 controls. DA cases had more gastrointestinal complaints than controls. T21 cases were at greater risk and frequency of esophageal disease than non-T21 cases (RR = 4.08, p = 0.002, RaR = 69.8, p < 0.001). T21 and non-T21 cases were equally likely to present with obstruction (RR = 0.91, p = 1), but T21 cases complained of obstructive symptoms less (RaR = 0.57, p = 0.003). T21 and non-T21 cases had the same risk of stomach diseases, but T21 cases complained more frequently (RaR = 6.20, p < 0.001). Cox models supported these observations. T21 did not affect ulcerative diseases. CONCLUSION: DA cases had more gastrointestinal problems than controls. T21 increased esophageal and gastric complaints in DA cases but did not affect ulcerative and obstructive complaints.

The impact of intestinal atresia on educational and mental health outcomes in school-aged children: A case-control cohort study.

PURPOSE: To assess real-world educational outcomes, neurodevelopmental disorders and mental health disorders in patients with intestinal atresia (IA) and compare these to outcomes to age-matched controls. METHODS: We performed a retrospective case-control study of children with IA born between 1991 and 2017. We evaluated educational outcomes using an Early Developmental Instrument, Grades 3, 7 and 8 assessments, Grade 9 completion and performance, high school graduation, and neurodevelopmental and mental health disorders using International Classification of Diseases codes available from a population-based dataset. RESULTS: We identified a total of 94 IA cases and 940 date-of-birth-matched controls. Cases with chromosomal anomalies were more likely to fail the Early Developmental Instrument (OR = 2.88, p = 0.037), Grade 8 reading (OR = 2.94, p = 0.043) and Grade 12 (OR = 3.25, p = 0.036); cases without chromosomal anomalies were at no greater risk than their peers of failing to meet expectations and did not have an increased risk of anxiety (OR = 0.48, p = 0.12), depression (OR = 0.74, p = 0.6) or hyperactivity disorder (OR = 1.92, p = 0.087). CONCLUSIONS: Our study demonstrated that IA patients without chromosomal anomalies are not at higher risk of neurodevelopmental and mental health disorders than controls. These results will offer more accurate outcome predictions for parents of children with IA.

Musculoskeletal deformities in children with congenital thoracic malformations: a population-based cohort study.

PURPOSE: It is unclear if musculoskeletal deformities observed in patients with congenital diaphragmatic hernia (CDH), congenital lung lesion (CLL) and esophageal atresia/tracheoesophageal fistula (EA/TEF) are associated with the anomaly or are a result of the surgery required to treat the anomaly. This study compared the prevalence of musculoskeletal deformities for: (1) children with congenital thoracic anomalies to controls; (2) CLL to EA/TEF both repaired via thoracotomy; and (3) CLL and EA/TEF to CDH repaired via laparotomy. METHODS: We performed a retrospective study of children with CLL, CDH or EA/TEF between 1990 and 2016. Date-of-birth-matched control groups were generated from a population-based dataset. International Classification of Disease codes were used to identify scoliosis and pectus anomalies. We determined Hazard ratios (HR) for cases versus controls. RESULTS: We included 167 cases (CDH n = 82; CLL n = 29; EA/TEF n = 56) and 1670 controls. EA/TEF had a greater risk of scoliosis (HR 5.52, 95%CI 1.49,13.73) and pectus deformities (HR 4.07, 95%CI 1.96,8.45). CDH showed more scoliosis (HR 5.03, 95%CI 1.99,12.74) but not pectus anomalies. Musculoskeletal deformities were not more common in CLL. CONCLUSION: Children born with CDH or EA/TEF, but not CLL, had more musculoskeletal deformities than controls. The inconsistent association between musculoskeletal deformities and the surgical approach suggested a congenital predisposition.

Respiratory outcomes in the first 10 years of life in children with gastroschisis: A retrospective cohort study.

BACKGROUND: Little attention has been given to the long-term respiratory outcomes of children with gastroschisis. The purpose of this study was to determine if gastroschisis survivors have more respiratory illnesses in their first 10 years of life compared with age-matched controls. METHODS: We performed a retrospective cohort study of all gastroschisis children born in Manitoba between 1991 and 2017. Gastroschisis cases were identified from a clinical database, and a date of birth-matched control cohort was constructed from a population-based data repository. International Classification of Disease codes were used to compare the risk and frequency of respiratory diagnoses for children with gastroschisis to date of birth-matched controls from 0-5 years of age and 5-10 years of age. RESULTS: The 0-5 years of age analysis included 117 gastroschisis cases and 1205 date of birth-matched controls; children with gastroschisis had a higher risk of asthma (relative risk [RR] = 1.46; 95% confidence interval [CI]: 1.03, 2.55; p = .029), acute bronchitis/bronchiolitis (RR = 1.61; 95% CI: 1.27, 2.03; p < .001), pneumonia (RR = 1.99; 95% CI: 1.45, 2.72; p < .001), viral pneumonia (RR = 5.15; 95% CI: 1.79, 14.81; p = .007), and pneumonia due to unspecified organism (RR = 2.06; 95% CI: 1.45, 2.92; p < .001). Gastroschisis children 0-5 years of age were also diagnosed more frequently with bronchitis/bronchiolitis (RR = 2.14; 95% CI: 1.79, 2.57; p < .001) and viral pneumonia (RR = 8.10; 95% CI: 3.79, 17.31; p < .001). The 5-10 years of age analysis included 73 cases and 738 controls; no difference in the risk of respiratory illness was found for gastroschisis cases and controls in this age group. However, gastroschisis cases were more frequently diagnosed with bacterial pneumonia (RR = 3.03; 95% CI: 1.67, 5.51; p < .001) and influenza (RR = 3.03; 95% CI: 1.67, 5.51; p < .001). CONCLUSION: Our study shows that children with gastroschisis have an increased risk of asthma and respiratory infections compared with children without gastroschisis, most noticeably in the first 5 years of life.

The prevalence of hearing loss in children with congenital diaphragmatic hernia: A longitudinal population-based study.

BACKGROUND: The true prevalence of hearing loss among children with congenital diaphragmatic hernia (CDH) is unknown, with some studies reporting rates up to 60%. PURPOSE: The purpose of this study was to determine the prevalence of hearing loss among children with CDH and compare it to age-matched controls. METHODS: We used population-based datasets to compare the number of hearing loss diagnoses in children younger than 10 years-of-age born between 1992 and 2009 with CDH to date-of-birth matched controls without CDH. Factors associated with CDH disease severity were analyzed to determine their effect on the prevalence of hearing loss. A sensitivity analysis was performed to determine if selection bias of improved care over the course of the study affected hearing loss in CDH patients. The prevalences of hearing loss were compared using Fisher's exact tests and statistical significance was defined as p < 0.05. RESULTS: A total of 529 children, 38 CDH cases and their 491 date-of-birth matched controls, met the inclusion criteria. Hearing loss was found in 7 children with CDH (18.4%) compared to 26 (5.3%) controls; the risk ratio (RR) of hearing loss was 3.48 (95%CI = 1.61-7.49, p = 0.006). There was no association between CDH disease severity and hearing loss. CONCLUSIONS: CDH is associated with hearing loss compared to the general population. Our results suggest that congenital factors may contribute to hearing loss in CDH more than perinatal exposures. LEVEL OF EVIDENCE: 3.

High-frequency vs. conventional ventilation at the time of CDH repair is not associated with higher mortality and oxygen dependency: a retrospective cohort study.

PURPOSE: The VICI-trial reported that in patients with congenital diaphragmatic hernia (CDH), mortality or bronchopulmonary dysplasia (BPD) were equivalent using conventional mechanical ventilation (CMV) and high-frequency oscillatory ventilation. The purpose of this study was to determine if the mode of ventilation at the time of CDH repair affected mortality or oxygen dependence at 28 days. METHODS: We performed a retrospective cohort study of infants born wih CDH from 1991 to 2015. A generalized linear model was applied to the data using a propensity score analysis. RESULTS: Eighty patients met the inclusion criteria; at the time of surgery 39 (48.8%) patients were on HFV and 41 (51.3%) patients were on CMV. In the HFV group, 16 (47.1%) patients remained oxygen dependent and there were 5 (12.8%) deaths at 28 days. In the CMV group, 5 (12.2%) patients remained oxygen dependent at 28 days but none had died. The base model demonstrated that the HFV group had increased rates of oxygen dependence [OR = 6.40 (2.13, 22.2), p = 0.002]. However, after propensity score analysis, we found no difference between HFV and CMV. CONCLUSION: Our study suggests that in infants with CDH, there is no significant difference between HFV and CMV in oxygen dependency or death.

Long-Term Health-Related Quality of Life in Survivors of Congenital Diaphragmatic Hernia.

The health-related quality of life (HRQoL) of survivors of congenital diaphragmatic hernia (CDH) is presently unknown. Literature prior to 2015 suggests that CDH survivors have worse HRQoL compared with the general population. However, due to changes in the diagnosis, management, and follow-up of CDH patients, this may no longer be true. The objective of this review was to determine what recent literature suggests regarding the HRQoL of CDH survivors. We reviewed all studies published between April 9, 2015, and April 6, 2020, that described the HRQoL of CDH survivors. Studies were identified using PubMed, and the references of the identified papers were searched for additional studies eligible for inclusion. Seven studies describing HRQoL in CDH survivors using validated measures of HRQoL for children, PedsQL (Pediatric Quality of Life Inventory), KIDSCREEN-52, and Comprehensive Quality of Life Scale - School Version were examined. The findings were disparate. One study suggested that CDH survivors had better than average HRQoL scores, three studies found patients and controls had equivalent scores, and two studies reported poor outcomes for CDH patients. The final study found no effect of disease severity on HRQoL, as determined by prenatal ultrasound. Evidence published between 2015 and 2020 suggests that CDH patients have an HRQoL equivalent to the general population.

Magnamosis for esophageal atresia is associated with anastomotic strictures requiring an increased number of dilatations.

BACKGROUND/PURPOSE: Magnamosis is a novel technique which utilizes high power magnets to anastomose the esophageal ends in children with esophageal atresia (EA) with or without a tracheoesophageal fistula (TEF), theoretically avoiding the need for thoracotomy. The objective of this study was to compare anastomotic stricture formation requiring dilatation after magnamosis versus after conventional anastomosis. METHODS: Our center treated the first 3 cases of EA ±â€¯TEF with magnamosis in Canada. One was unsuccessful and excluded from our study. The number of postintervention dilatations was compared to controls from our database, which includes all children with EA ±â€¯TEF treated between 1991 and 2015. The controls had EA ±â€¯TEF treated with pouch-to-end anastomosis or colonic interposition (n = 65). Mann-Whitney U tests were used with p < 0.05 being significant. RESULTS: The 2 magnamosis cases had a mean of 13.5 dilatations, compared to 2.6 for the controls. Those managed with pouch-to-end anastomosis or colonic interposition had a mean of 2.3 and 2.7 dilatations, respectively. We found that the cases required more dilatations than controls (p = 0.022) and pouch-to-end anastomosis (p = 0.021), but not than colonic interposition (p = 0.106). CONCLUSION: Our results indicate that magnamosis is associated with more postintervention dilatations than conventional anastomotic techniques, suggesting that magnamosis results in more frequent and/or more resilient anastomotic strictures. LEVEL OF EVIDENCE: 3.

Asthma Medication Use in Congenital Diaphragmatic Hernia Survivors: A Retrospective Population Level Data Analysis.

INTRODUCTION: The purpose of this study was to determine if congenital diaphragmatic hernia (CDH) survivors had worse long-term respiratory outcomes compared with age-matched controls, as measured by inhaled bronchodilator use, inhaled steroid use, and asthma-related physician visits. MATERIALS AND METHODS: We performed a retrospective case-control study of infants with isolated CDH from 1991 to 2013. The primary outcome measures were inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits between 0 and 5 years of age and between 5 and 10 years of age. Subgroup analysis compared the same outcomes for CDH patients grouped by: birth weight, gestational age, side of defect, defect size, liver herniation, hernia sac, and pulmonary hypertension. RESULTS: Fifty-six patients with CDH and 753 age-matched controls met the inclusion criteria for the 0 to 5 years of age analysis. Between 0 and 5 years of age, more CDH survivors were prescribed an inhaled bronchodilator (odds ratio [OR] = 2.47[1.38-4.48], p = 0.001) and inhaled steroid (OR = 2.03[1.07-3.74], p = 0.03), and had an asthma-related physician visit (OR = 1.92[1.00-3.56], p = 0.04). Thirty-eight cases and 491 controls met the inclusion criteria for the 5 to 10 years of age analysis. Between 5 and 10 years of age, CDH survivors were not more likely to be prescribed inhaled bronchodilators, inhaled steroids, or have an asthma-related physician visit. Among the CDH patients, we did not find a clinical characteristic associated with increased inhaled bronchodilator or steroid prescriptions at any age. CONCLUSION: A history of CDH is associated with higher rates of inhaled bronchodilator prescriptions, inhaled steroid prescriptions, and asthma-related physician visits from 0 to 5 years of age compared with age-matched controls. However, this difference resolves by 5 to 10 years of age.

The presence of a hernia sac in isolated congenital diaphragmatic hernia is associated with less disease severity: A retrospective cohort study.

INTRODUCTION: We aimed to determine if the presence of a hernia sac in neonates with isolated congenital diaphragmatic hernia (CDH) was associated with better clinical outcomes. METHODS: We performed a retrospective cohort study of infants with isolated CDH from 1991 to 2015. Primary outcome measures were oxygen-dependence and mortality at 28 days. Secondary measures were: inhaled nitric oxide use, vasoactive medication use, ventilator support, and recurrence rates. RESULTS: Seventy-one patients met the inclusion criteria: 14 patients (19.7%) had a hernia sac, and 57 patients (80.3%) did not. Mortality did not differ between the 2 groups [0 of 14 versus 3 of 57 (5.3%) (p = 1.000)]. Hernia sac patients had similar oxygen-dependence after 28 days [1 of 14 (7.1%) versus 14 of 57 (24.6%) (p = 0.273)]. Hernia sac children required less iNO (0.64 ±â€¯2.41 vs. 6.35 ±â€¯12.2 days, p = 0.002), vasoactive medications (2.79 ±â€¯3.07 vs. 5.36 ±â€¯5.52, p = 0.027), and time on ventilation (7.62 ±â€¯6.12 vs. 15.9 ±â€¯19.2, p = 0.010). Hernia sac children had similar recurrence rates within 2 years [0 of 14 versus 7 of 57 (12.3%) (p = 0.331)]. CONCLUSION: The presence of a hernia sac was not associated with lower rates of oxygen dependency or death at 28 days but was associated with decreased inhaled nitric oxide, vasoactive medication, and ventilator use. LEVEL OF EVIDENCE: III.

Identifying Information Needs for Hirschsprung Disease Through Caregiver Involvement via Social Media: A Prioritization Study and Literature Review.

BACKGROUND: Patient and public involvement in health research is important to produce relevant and impactful results. OBJECTIVE: This paper aimed to prioritize and summarize Hirschsprung disease (HD)-related information needs among caregivers of children with HD and pediatric surgeons through partnership with a parent-initiated social media campaign. METHODS: We conducted a Web-based survey with the 2 stakeholder groups to identify information needs. The caregiver survey was conducted through a global Web-based community, and the surgeon survey was distributed to members of the Canadian Association of Paediatric Surgeons (CAPS). We conducted a literature review to identify evidence on the prioritized topics. RESULTS: Our findings showed that 54.9% (89/162) of the individuals completed the caregiver survey and 23.8% (52/218 listed members) of the pediatric surgeons completed the survey distributed through CAPS. Only 20% (18/89) of the caregivers reported being very satisfied or satisfied with the current HD-related resources. A final prioritized list of information needs included bowel management, nutrition and growth, infection, perianal irritation, gastrointestinal pain, surgical diagnostics, and surgical complications. In total, 87 studies were included in the literature review, which included the following: 8 reviews, 2 randomized controlled trials, 74 cohort studies, and 3 practice guidelines. Two priority issues identified by caregivers had only a single study that met the inclusion criteria, whereas 1 topic had none. CONCLUSIONS: With caregiver and surgeon input, we identified 7 information priority areas related to HD. A review of the literature on the priorities found little evidence to support the development of high-quality guidelines. More research is necessary to meet the information needs related to HD as identified by stakeholders.

Applying vacuum to accomplish reduced wound infections in laparoscopic pediatric surgery.

INTRODUCTION: The prevention of surgical site infections has received little attention in pediatric surgery. Negative pressure wound therapy is used to treat complex wounds. We hypothesized that this principle could reduce wound infection rates following laparoscopic surgery. We tested this in a randomized controlled trial. MATERIALS AND METHODS: We randomized pediatric patients with an umbilical port site to a standard dressing or a vacuum dressing. The dressings were removed 48h after surgery. A nurse blinded for the treatment inspected the umbilical wound between post-operative days 7-10 for infection. Data comparison was performed using a Fisher exact test with p<0.05 defined as significant. RESULTS: We recruited 90 patients over 2 years and randomized 44 to the vacuum dressing arm and 42 to the control arm. We observed a 2.8% (n=1/35) infection rate in the vacuum dressing group and 3.3% (n=1/30) in the control group (p=1.0). DISCUSSION: We ended our study early when an interim analysis showed an impractical number of patients would be required to achieve sufficient power. We did not find a significant difference between the control and vacuum dressings in reducing post-operative wound infections. LEVEL OF EVIDENCE: 3.

Transplantation-associated thrombotic microangiopathy isolated to a congenital anomaly of the lung.

TA-TMA is a post-hematopoietic stem cell transplant complication with clinical features of hemolytic anemia and thrombocytopenia. A 26-month-old child who had had an allogeneic transplant for treatment of DBA developed severe TA-TMA with heavy red blood cell and platelet transfusion dependence. Incidentally, he was found to have a lung sequestration. TA-TMA resolved and transfusion dependence resolved after resection of the sequestration. The finding suggests the malformation vasculature was selectively vulnerable to the trigger of TA-TMA-raising perhaps a clue to basic pathophysiology of TA-TMA and/or vascular malformations.