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

AimsTo 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.MethodsThe 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**.ResultsSurvivors (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.ConclusionBoth 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 studyPrognostic Study.Level of evidenceLevel III.     

Requirement and Duration of Tube Feed Supplementation among Congenital Diaphragmatic Hernia Patients

PurposeOral feeds pose a challenge for congenital diaphragmatic hernia (CDH) infants. Tube feed (TF) supplementation may be required to support the achievement of normal growth. The aim of this study was to determine the duration and factors associated with TF use in CDH infants at our institution.MethodsA single centre retrospective chart review was performed for CDH-born infants who underwent repair between 2000 to 2013 (REB #1000053124). Patient demographics, perinatal management, and feeding status of infants with at least 1-year follow-up were reviewed.ResultsOf 160 CDH infants, 32 (20%) were discharged on partial or complete TF, and an additional 5 (3.1%) patients started TF post discharge. CDH infants with TF were more likely to have initial arterial blood pH < 7.25, patch repair, ECMO support, and prolonged ICU stay (p < 0.05). Time to TF discontinuation did not differ significantly between those partially or fully TF at discharge. Twelve patients (33.3%) remained TF at their last known follow-up.ConclusionHigh risk CDH patients are likely to require TF to support their nutritional intake. Parents and caregivers need to be informed and properly supported. Long-term monitoring of CDH patient oral intake, growth, and development will be required.Level of Evidence/Type of StudyLevel III Retrospective Study.     

The Timing of Congenital Diaphragmatic Hernia Repair on Extracorporeal Membrane Oxygenation Impacts Surgical Bleeding Risk

BackgroundThe optimal timing of surgical repair for infants with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO) support remains controversial. The risk of surgical bleeding is considered by many centers as a primary factor in determining the preferred timing of CDH repair for infants requiring ECMO support. This study compares surgical bleeding following CDH repair on ECMO in early versus delayed fashion.MethodsA retrospective review of 146 infants who underwent CDH repair while on ECMO support from 1995 to 2021. Early repair occurred during the first 48 h after ECMO cannulation (ER) and delayed repair after 48 h (DR). Surgical bleeding was defined by the requirement of reoperative intervention for hemostasis or decompression.Results102 infants had ER and 44 infants DR. Surgical bleeding was more frequent in the DR group (36% vs 5%, p < 0.001) with an odds ratio of 11.7 (95% CI: 3.48–39.3, p < 0.001). Blood urea nitrogen level on the day of repair was significantly elevated among those who bled (median 63 mg/dL, IQR 20–85) vs. those who did not (median 9 mg/dL, IQR 7–13) (p < 0.0001). Duration of ECMO support was shorter in the ER group (median 13 vs 18 days, p = 0.005). Survival was not statistically different between the two groups (ER 60% vs. DR 57%, p = 0.737).ConclusionWe demonstrate a significantly lower incidence of bleeding and shorter duration of ECMO with early CDH repair. Azotemia was a strong risk factor for surgical bleeding associated with delayed CDH repair on ECMO.Level of evidenceLevel III cohort study.     

Evaluation of Lung Injury in Infants with Congenital Diaphragmatic Hernia

Background/PurposeThe presence of lung injury and the factors that contribute to it in infants with congenital diaphragmatic hernia (CDH) have not been objectively measured during their clinical course. In adults with acute respiratory distress syndrome, higher serum levels of surfactant protein D (SP-D) are linked to lung injury and worse outcomes. We hypothesized that serum SP-D levels would be elevated in CDH infants and that the levels would correlate to the amount of lung injury present.MethodsIn this retrospective cohort study, serum SP-D levels were analyzed in 37 CDH infants and 5 control infants using a commercially available enzyme-linked immunosorbent assay kit.ResultsInfants with more severe CDH had a statistically significant increase (p < 0.001) in serum SP-D over their first month of life. SP-D levels in CDH infants were similar to control infants while on extracorporeal membrane oxygenation (ECMO) but were 2.5-fold higher (p = 0.03) than controls following ECMO termination. SP-D levels increased 1.6-fold following surgical repair of the diaphragm and were significantly higher in the second week following surgery when compared to pre-operative levels (p < 0.03).ConclusionsThese results demonstrate that CDH infants experience lung injury during the first week of life, around the time of surgery, and at the time of ECMO termination.Level II prognosis study.     

Potential survival benefit with repair of congenital diaphragmatic hernia (CDH) after extracorporeal membrane oxygenation (ECMO) in select patients: Study by ELSO CDH Interest Group

PurposeStudying the timing of repair in CDH is prone to confounding factors, including variability in disease severity and management. We hypothesized that delaying repair until post-ECMO would confer a survival benefit.MethodsNeonates who underwent CDH repair were identified within the ELSO Registry. Patients were then divided into on-ECMO versus post-ECMO repair. Patients were 1:1 matched for severity based on pre-ECMO covariates using the propensity score (PS) for the timing of repair. Outcomes examined included mortality and severe neurologic injury (SNI).ResultsAfter matching, 2,224 infants were included. On-ECMO repair was associated with greater than 3-fold higher odds of mortality (OR 3.41, 95% CI: 2.84–4.09, p < 0.01). The odds of SNI was also higher for on-ECMO repair (OR 1.49, 95% CI: 1.13–1.96, p < 0.01). A sensitivity analysis was performed by including the length of ECMO as an additional matching variable. On-ECMO repair was still associated with higher odds of mortality (OR 2.38, 95% CI: 1.96–2.89, p < 0.01). Results for SNI were similar but were no longer statistically significant (OR 1.33, 95% CI: 0.99–1.79, p = 0.06).ConclusionsOf the infants who can be liberated from ECMO and undergo CDH repair, there is a potential survival benefit for delaying CDH repair until after decannulation.Type of StudyTreatment StudyLevel of EvidenceIII     

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

IntroductionWe aimed to determine if the presence of a hernia sac in neonates with isolated congenital diaphragmatic hernia (CDH) was associated with better clinical outcomes.MethodsWe 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.ResultsSeventy-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 ± 2.41 vs. 6.35 ± 12.2 days, p = 0.002), vasoactive medications (2.79 ± 3.07 vs. 5.36 ± 5.52, p = 0.027), and time on ventilation (7.62 ± 6.12 vs. 15.9 ± 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)].ConclusionThe 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 evidenceIII     

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study

IntroductionCongenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular morbidity.Material and methodsThis was a nationwide population-based prospective case-control study within a cohort of Swedish children with CDH, born 1982–2015. Five controls for each patient were randomly sampled from the population. The outcomes were the corresponding International Statistical Classification of Disease (ICD) codes for cardiovascular diagnoses according to ICD 9 and 10.ResultsThere was an overrepresentation of cardiovascular diagnoses in the CDH group after one year of age compared to the control group, 8.0vs 0.5% (n = 53 versus n = 16). The risk of having a cardiovascular diagnosis in this CDH group was 15 times higher compared to the control group (HR 15.8, 95% CI: 9–27.6, p < 0.005). The diagnoses of cardiac arrhythmias and systemic hypertension were less common in the CDH group before the age of one year compared to the CDH group beyond the age of one year. Arrhythmia 3.7 vs 15.1%, systemic hypertension 3.7 vs 7.5%.ConclusionCDH survivors have increased cardiovascular morbidity during childhood and young adulthood. This implies that structured follow up programs, covering cardiovascular morbidity, needs to be developed and should be offered in pediatric and adult care. Being born with CDH seems to be a risk factor for future cardiovascular diagnoses.Level of evidenceLevel 3: Case-Control Study     

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

BackgroundThe true prevalence of hearing loss among children with congenital diaphragmatic hernia (CDH) is unknown, with some studies reporting rates up to 60%.PurposeThe purpose of this study was to determine the prevalence of hearing loss among children with CDH and compare it to age-matched controls.MethodsWe 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.ResultsA 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.ConclusionsCDH 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 evidence3.     

Necrotizing Enterocolitis Associated with Congenital Heart Disease: a Different Entity?

BackgroundNecrotizing enterocolitis (NEC) predominantly occurs in preterm infants (PT-NEC). In term neonates, NEC occurs more frequently when a congenital heart disease is present (CHDNEC). Our aim was to evaluate differences and similarities in disease characteristics of PT-NEC versus CHD-NEC.MethodsIn this retrospective case–control study we identified all CHD infants who developed NEC Bell's stage ≥ 2 in our center from 2004 to 2014. We randomly selected (1:2 ratio) PT-NEC infants from the same period. Biochemical and clinical variables were retrieved from patient files.ResultsWe found 18 CHD-NEC infants and selected 36 PT-NEC infants (gestational age 28.3 [25–35.6] weeks vs. 38.6 [31.7–40.7] weeks). Postnatal age at onset was significantly lower in CHD-NEC patients (4 [2–24] vs. 11 [4–41] days, p < 0.001). Lowest pH levels were lower (7.21 [7.01–7.47] vs. 7.27 [6.68–7.39], p = 0.02), and highest CRP levels were higher (112.5 mg/L [5.0–425.0] vs. 66.0 [5.2–189.0], p = 0.05) in PT-NEC vs. CHD-NEC. Anatomic localisation of the disease differed: the colon was significantly more often involved in CHD-NEC versus PT-NEC (86% vs. 33%, p = 0.03). Mortality caused by NEC was not different (22% vs. 11%, p = 0.47).ConclusionWhile outcome of NEC in both groups is similar, the predominant NEC localisation differed between CHD-NEC and PT-NEC patients. This suggests that both variants of the disease have a different underlying pathophysiological mechanism that predisposes different intestinal regions to develop NEC.Type of StudyRetrospective Case–Control Study.Level of evidenceLevel III.     

Necrotizing enterocolitis in term neonates: A different disease process?

IntroductionNecrotizing enterocolitis (NEC) has been a long-recognized complication of prematurity, but there is a paucity of studies on term infants. We sought to characterize the clinical presentation and outcomes of full term (FT) infants with NEC and compare these to our experience with preterm (PT) neonates.MethodsWe conducted a chart review of infants admitted to the NICU at University of Michigan with a diagnosis of NEC for over a 10-year period with a Modified Bell stage of 2 or greater. We compared the outcomes and comorbidities of PT against those of FT, defined as gestational age at birth below and above 37 weeks, respectively.ResultsOut of 170 infants, 28(17%) were FT. FT neonates were more likely to have undergone cardiac surgery for a congenital defect, excluding PDA ligation (64% vs. 8%)*. When compared to FT infants, PT infants were more likely to require surgical intervention (18% vs. 59%)*, have Bell stage 3 disease (82% vs. 43%)*, require vasopressor support (21% vs. 42%)⁺, and require ventilatory support (43% vs 75%)*. *p < 0.01,⁺ p < 0.05.ConclusionFT neonates present with different patterns of disease and have different outcomes, suggesting that this may be a different clinical entity than NEC in preterm infants.Type of studyRetrospective reviewLevel of evidenceLevel III     

Impact of a standardized management guideline for infants with CDH: A single-center experience

BackgroundInfants with congenital diaphragmatic hernia (CDH) are at high risk of death, even despite extracorporeal membrane oxygenation (ECMO) support. In January 2012 we implemented a standardized clinical practice guideline (CPG) to manage infants with CDH. We hypothesized that infants with CDH managed with CPG had better clinical outcomes, less ECMO utilization, and increased survival to discharge.MethodsWe conducted a retrospective pre-post study of infants with CDH admitted between January 2007 and July 2021 (n = 133). Patients were divided into Cohort 1, pre-CPG (January 2007 to December 2011, n = 54), and Cohort 2, post-CPG (January 2012 to July 2021, n = 79).ResultsMore patients in Cohort 1 were small for gestational age than in Cohort 2. No other patient demographics were different between cohorts. Cohort 2 had significantly lower ECMO utilization as compared to Cohort 1 (18% vs 50%, p<0.001). Cohort 2 had significantly higher survival to discharge compared to Cohort 1 (85% vs 57%, p<0.001). Survival for ECMO-treated patients in Cohort 2 was significantly higher than in Cohort 1 (71% vs 26%, p = 0.005). In Cohort 1, 70% of the non-survivors were repaired, of which 81% were repaired on ECMO. In Cohort 2, 8% of the non-survivors were repaired, none on ECMO. Only 3% in Cohort 2 were discharged with pulmonary hypertension medication.ConclusionsA standardized CPG to manage patients with CDH decreased ECMO utilization and improved clinical outcomes including survival to discharge. Refinement of management strategies, implementation of new interventions, and meticulous care can improve outcomes in patients with CDH.     

Primary contributors to gastrostomy tube placement in infants with Congenital Diaphragmatic Hernia

ObjectiveTo identify factors associated with gastrostomy tube (GT) placement in infants with congenital diaphragmatic hernia (CDH).MethodsRetrospective cohort study of 114 surviving infants with CDH at a single tertiary care neonatal intensive care unit from 2010–2019. Prenatal, perinatal and postnatal characteristics were compared between patients who were discharged home with and without a GT. Prenatal imaging was available for 50.9% of the cohort. Logistic regression was used to assess the association between GT placement and pertinent clinical factors. ROC curves were generated, and Youden's J statistic was used to determine optimal predictive cutoffs for continuous variables. Elastic net regularized regression was used to identify variables associated with GT placement in multivariable analysis.ResultsGT was placed in 43.9% of surviving infants with CDH. Prenatal variables predictive of GT placement were percent predicted lung volume (PPLV) <21%, total lung volume (TLV) <30 ml, lung-head ratio (LHR) <1.2 or observed to expected LHR (O/E LHR) <55%. Infants who required a GT were diagnosed earlier prenatally (23.6 ± 3.4 vs. 26.4 ± 5.6 weeks). Patients whose stomach was above the diaphragm on prenatal ultrasound (up) had a higher odds of GT placement compared to those with stomachs below the diaphragm (down) position by a factor of 2.9 (95% CI: 1.25, 7.1); p = 0.0154. Postnatally, infants with GT had lower Apgar scores at 1 and 5 min, longer lengths of stay and higher proportion of flap closures. Infants with a type C or D defect and extracorporeal membrane oxygenation (ECMO) were associated with increased odds of needing a GT. Postnatal association included being NPO for >12 days, need for transpyloric (TP) feeds for >10 days, >14 days to transition to a 30 min bolus feed, presence of gastro-esophageal reflux (GER), chronic lung disease and pulmonary hypertension. In multivariable analysis, duration of NPO, time to TP feeds, transition to 30 min bolus feeds remained significantly associated with GT placement after adjusting for severity of pulmonary hypertension (PH), GER diagnosis and sildenafil treatment.ConclusionIdentification of risk factors associated with need for long-term feeding access may improve timing of GT placement and prevent prolonged hospitalization related to feeding issues.Level of Evidence RatingLevel II (Retrospective Study)     

Impaired somatic growth and delayed lung development in infants with congenital diaphragmatic hernia—evidence from a 10-year, single center prospective follow-up study

Purpose

In infants with congenital diaphragmatic hernia (CDH), somatic growth and pulmonary development are key issues beyond the time of intensive care treatment. The aim of the study was to investigate the somatic growth and pulmonary function after discharge and to compare CDH patients with a group of matched controls.

Methods

Anthropometric measurements and lung function tests were performed in 26 infants after surgical repair of CDH and 26 non-CDH intensive care patients, matched for gestational age and birth weight. Spontaneously breathing infants were tested at a mean of 44 weeks postconceptional age (range, 36-58 weeks). Body weight, body length, respiratory rate (RR), tidal volume (V_T), functional residual capacity by body plethysmography (FRC_pleth), respiratory compliance (C_rs), and respiratory resistance (R_rs) were measured.

Results

The mean (SD) weight gain per week in the CDH infants was significantly lower compared to non-CDH infants (89 [39] g vs 141 [49] g; P = .002). The breathing pattern between both groups differed considerably. In CDH infants, V_T was significantly lower (P < .001) and RR significantly higher (P = .005). The respiratory compliance was also significantly (P < .001) reduced, whereas R_rs did not differ significantly. No statistically significant differences were seen in FRC_pleth related to the body weight between CDH and non-CDH infants (20.3 [4.4] mL/kg vs 21.5 [4.9] mL/kg).

Conclusion

Despite apparently well-inflated lungs after surgery, evidence of early and significantly reduced weight gain and impaired lung function in CHD patients should prompt careful dietary monitoring and regular lung function testing.     

Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic

BackgroundAlthough fetoscopic endoluminal tracheal occlusion (FETO) was recently shown to improve survival in a multicenter, randomized trial of severe congenital diaphragmatic hernia (CDH), morbidity outcomes remain essentially unknown. The purpose of this study was to assess long-term outcomes in children with severe CDH who underwent FETO.MethodsWe conducted a prospective study of severe CDH patients undergoing FETO at an experienced North American center from 2015-2021 (NCT02710968). This group was compared to a cohort of non-FETO CDH patients with severe disease as defined by liver herniation, large defect size, and/or ECMO use. Clinical data were collected through a multidisciplinary CDH clinic. Statistics were performed with t-tests and Chi-squared analyses (p≤0.05).ResultsThere were 18 FETO and 17 non-FETO patients. ECMO utilization was 56% in the FETO cohort. Despite significantly lower median observed/expected lung-to-head ratio (O/E LHR) in the FETO group, [FETO: 23% (IQR:18-25) vs. non-FETO: 36% (IQR: 28-41), p<0.001], there were comparable survival rates at discharge (FETO: 78% vs. non-FETO: 59%, p = 0.23) and at 5-years (FETO: 67% vs. non-FETO: 59%, p = 0.53) between the two cohorts. At a median follow up of 5.8 years, metrics of pulmonary hypertension, pulmonary morbidity, and gastroesophageal reflux disease improved among patients after FETO. However, most FETO patients remained on bronchodilators/inhaled corticosteroids (58%) and were feeding tube dependent (67%).ConclusionsThese North American data show that prenatal tracheal occlusion, in conjunction with a long-term multidisciplinary CDH clinic, is associated with acceptable long-term survival and morbidity in children after FETO.Level of EvidenceLevel III     

Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia

Background

Use of exogenous surfactant in congenital diaphragmatic hernia (CDH) patients is routine in many centers. The authors sought to determine the impact of surfactant use in the premature infant with CDH.

Methods

Data on liveborn infants with CDH from participating institutions were collected prospectively. Surfactant use and timing and outcome data were analyzed retrospectively. The authors evaluated the prenatal diagnosis patients as well. The outcome variable was survival to discharge. Odds ratios with confidence intervals were calculated.

Results

Five hundred ten infants less than 37 weeks’ gestation were entered in the CDH registry. Infants with severe anomalies (n = 80) were excluded. Information on surfactant use was available for 424 patients. Infants receiving surfactant (n = 209) had a greater odds of death than infants not receiving surfactant (n = 215, odds ratio, 2.17, 95% CI: 1.5 to 3.2; P < .01). In prenatally diagnosed infants with immediate distress, there was a trend toward worse survival rates among those receiving surfactant at 1 hour (52 patients) versus those that did not (93 patients; odds ratio, 1.93, 95% CI: 0.96 to 3.9; P < .07).

Conclusions

Surfactant, as currently used, is associated with a lower survival rate in preterm infants with CDH. The use of surfactant replacement in premature infants with CDH can be recommended only within the context of a randomized clinical trial.     

Use of trans-anastomotic tubes in congenital duodenal obstruction

AimDespite data to suggest benefit of trans- anastomotic tube (TAT) feeding in infants following repair of congenital duodenal obstruction (CDO), TAT usage is limited. We aimed to report a large series of infants with CDO treated with or without TAT in order to improve the evidence underlying this simple intervention.MethodSingle centre retrospective review of all infants CDO over a 20-year period (January 1999 – November 2020, inclusive). Important outcomes were compared between infants treated with or without TAT. Data are median [IQR].ResultsNinety-six infants were included. A TAT was placed in 54 infants (56%). Median time to full enteral feed was significantly shorter in the TAT group (6 [5–8] days vs 10 [7.5–12], p <0.001). Time to first feed was shorter in the TAT group (2 [2–2.8] days vs 3 [2–5], p<0.001). Significantly fewer infants with a TAT placed received a central venous catheter (CVC, 15% vs 76%, p <0.001). Infants without a TAT received parenteral nutrition (PN) for longer (0 [0–0] vs 7 [0–11] days, p <0.001). There was no change in length of stay between TAT and no TAT group (16 [13–21.8] vs 15 [12–21.8] days, p = 0.722). Eight infants (15%) in the TAT group required a CVC and PN. One infant in the TAT group developed a perforation that required surgical management and nine infants in the non-TAT group had complications related to the CVC (21%), including one infant that required general anaesthetic for tunnelled central line placement (2.3%).ConclusionIn infants with CDO, TAT use was associated with earlier establishment of full enteral feeds, reduced need for CVC and PN and reduced complications. Further research should focus on the barriers to wider use of TAT by surgeons and neonatologists in infants with CDO.Level III evidence     

Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices

Purpose

Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH. We aimed at identifying the relationship between McGoon index (MGI) and pulmonary artery index (PAI) scores and disease severity among infants with CDH.

Methods

We retrospectively reviewed the medical records of 19 high-risk patients with CDH born between January 2006 and December 2007. McGoon index and PAI scores were determined on admission. We evaluated statistically the relationship between these scores and variables representing severity as follows: number of vasodilators, use of inhaled nitric oxide (iNO), closed method of diaphragm, duration of intubation, duration of hospitalization, and use of home oxygen therapy. Statistical significance was P < .05.

Results

Overall median MGI and PAI scores were 1.40 and 108, respectively; scores for nonsurvivors were significantly (P < .05 and P < .01, respectively) lower than those for survivors. Among survivors, PAI scores were significantly (P < .05) lower in infants requiring iNO than in infants not requiring iNO and patch repair. The PAI scores were significantly correlated with the number of vasodilators (r = −0.789; P < .01) and duration of intubation (r = −0.610; P < .05).

Conclusions

McGoon index (cutoff value, 1.31) and PAI (cutoff value, 90) are reliable indices for predicting mortality in CDH. Pulmonary artery index appears to be more useful than MGI for predicting disease severity among survivors.     

One-year outcomes of congenital diaphragmatic hernia repair: Factors associated with recurrence and complications

PurposeCongenital diaphragmatic hernia (CDH) is a congenital anomaly associated with lifelong multisystem morbidity. This study sought to identify factors contributing to hospital readmission after CDH repair.MethodsThe Nationwide Readmissions Database from 2010 to 2014 was used to identify patients with CDH who underwent surgical repair. Primary outcomes included all cause readmission at 30-days and 1 year and readmission for hernia recurrence. Patient and hospital factors were compared using chi-squared analysis.ResultsFive hundred eleven patients were identified with neonatal CDH. All repairs were performed at teaching hospitals via laparotomy in 59% (n = 303), thoracotomy in 36% (n = 183), and minimally invasive (MIS) repair in 5% (n = 25). The readmission rate within 30-days was 32% (n = 163), and 97% (n = 495) within 1 year. The most common conditions surrounding readmission were for gastroesophageal reflux (20%), CDH recurrence (17%), and surgery for gastrostomy tube and/or fundoplication (16%). Recurrence was significantly higher after MIS repair (48%) compared to those with open repair via either approach (16%), p < 0.001.ConclusionsThis is the first study to evaluate nationwide readmissions in newborns with CDH. Readmission is commonly due to CDH recurrence and reflux-associated complications. The recurrence rate is higher than previously reported and is more common after MIS and repair via thoracotomy.Level of evidenceLevel III treatment study.     

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia – Side,stage, and outcome

Aim

To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH.

Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice

IntroductionGastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication.(3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair;MethodsThis prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion.ResultsOf 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0–5.0).(1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p < 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not.(2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p < 0.0001), patch repair (p < 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis.(3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03).ConclusionsChildren undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children.Level of evidenceII – Prospective Study.