Disparities in Healthcare Utilization: An Analysis of Disease Specific and Patient Level Factors in a Congenital Diaphragmatic Hernia Clinic.

OBJECTIVES: Our study examines if the disease severity profile of our Congenital Diaphragmatic Hernia (CDH) patient cohort adherent to long-term follow-up differs from patients lost to follow-up after discharge and examines factors associated with health care utilization. METHODS: Retrospective review identified CDH survivors born 2005-2019 with index repair at our institution. Primary outcome was long-term follow-up status: "active" or "inactive" according to clinic guidelines. Markers of CDH disease severity including CDH defect classification, oxygen use, tube feeds at discharge, and sociodemographic factors were examined as exposures. RESULTS: Of the 222 included patients, median age [IQR] was 10.2 years [6.7-14.3], 61% male, and 57 (26%) were insured by Medicaid. Sixty-three percent (139/222) of patients were adherent to follow-up. Seventy-six percent of patients discharged on tube feeds had active follow-up compared to 55% of patients who were not, with similar findings for oxygen at discharge (76% vs. 55%). Kaplan-Meier analysis showed patients with smaller defect size had earlier attrition compared to patients with larger defect size. Other race (Hispanic, Asian, Middle Eastern) patients had 2.87 higher odds of attrition compared to white patients (95% CI 1.18-7.0). Medicaid patients had 2.64 higher odds of attrition compared to private insurance (95% CI 1.23-5.66). CONCLUSION: Loss to follow-up was associated with race and insurance type. Disease severity was similar between the active and inactive clinic cohorts. Long-term CDH clinic publications should examine attrition to ensure reported outcomes reflect the discharged population. This study identified important factors to inform targeted interventions for follow-up adherence. LEVEL OF EVIDENCE: Level III.

Long-term follow-up of patients with congenital diaphragmatic hernia.

Neonates with congenital diaphragmatic hernia encounter a number of surgical and medical morbidities that persist into adulthood. As mortality improves for this population, these survivors warrant specialized follow-up for their unique disease-specific morbidities. Multidisciplinary congenital diaphragmatic hernia clinics are best positioned to address these complex long-term morbidities, provide long-term research outcomes, and help inform standardization of best practices in this cohort of patients. This review outlines long-term morbidities experienced by congenital diaphragmatic hernia survivors that can be addressed in a comprehensive follow-up clinic.

Exercise-Induced Pulmonary Hypertension in Long-Term Survivors of Congenital Diaphragmatic Hernia.

OBJECTIVE: To determine the prevalence of exercise-induced pulmonary hypertension (PH) among long-survivors of congenital diaphragmatic hernia repair. STUDY DESIGN: This is a single-center, retrospective cohort study of CDH survivors who underwent exercise stress echocardiography (ESE) at Boston Children's Hospital from January 2006 to June 2020. PH severity was assessed by echocardiogram at baseline and after exercise. Patients were categorized by right ventricular systolic pressure (RVSP) after exercise: Group 1 - no or mild PH; and Group 2 - moderate or severe PH (RVSP ≥ 60 mmHg or ≥ ½ systemic blood pressure). RESULTS: Eighty-four patients with CDH underwent 173 ESE with median age 8.1 (4.8 - 19.1) years at first ESE. Sixty-four patients were classified as Group 1, 11 as Group 2, and 9 had indeterminate RVSP with ESE. Moderate to severe PH after exercise was found in 8 (10%) patients with no or mild PH at rest. Exercise-induced PH was associated with larger CDH defect size, patch repair, use of ECMO, supplemental oxygen at discharge, and higher WHO functional class. Higher VE/VCO2 slope, lower peak oxygen saturation, and lower percent predicted FEV1, and FEV1/FVC ratio were associated with Group 2 classification. ESE changed management in 9/11 Group 2 patients. PH was confirmed in all 5 Group 2 patients undergoing cardiac catheterization after ESE. CONCLUSIONS: Among long-term CDH survivors, 10% had moderate-severe exercise-induced PH on ESE, indicating ongoing pulmonary vascular abnormalities. Further studies are needed to optimally define PH screening and treatment for patients with repaired CDH.

Risk Factors for Preoperative Pneumothorax in Neonates With Isolated Left-Sided Congenital Diaphragmatic Hernia: An International Cohort Study.

BACKGROUND: We aimed to investigate the clinical characteristics and outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH) who developed preoperative pneumothorax and determine its risk factors. METHODS: We performed an international cohort study of patients with CDH enrolled in the Congenital Diaphragmatic Hernia Study Group registry between January 2015 and December 2020. The main outcomes assessed included survival to hospital discharge and preoperative pneumothorax development. The cumulative incidence of pneumothorax was estimated by the Gray test. The Fine and Gray competing risk regression model was used to identify the risk factors for pneumothorax. RESULTS: Data for 2858 neonates with isolated left-sided CDH were extracted; 224 (7.8%) developed preoperative pneumothorax. Among patients with a large diaphragmatic defect, those with pneumothorax had a significantly lower rate of survival to discharge than did those without. The competing risks model demonstrated that a patent ductus arteriosus with a right-to-left shunt flow after birth (hazard ratio [HR]: 1.78; 95% confidence interval [CI]: 1.21-2.63; p = 0.003) and large defects (HR: 1.65; 95% CI: 1.13-2.42; p = 0.01) were associated with an increased risk of preoperative pneumothorax. Significant differences were observed in the cumulative incidence of pneumothorax depending on defect size and shunt direction (p < 0.001). CONCLUSIONS: Pneumothorax is a significant preoperative complication associated with increased mortality in neonates with CDH, particularly in cases with large defects. Large diaphragmatic defects and persistent pulmonary hypertension were found to be risk factors for preoperative pneumothorax development. LEVEL OF EVIDENCE: LEVEL Ⅲ Retrospective Comparative Study.

Long-Term Functional Outcomes at 1-Year After Hospital Discharge in Critically Ill Neonates With Congenital Diaphragmatic Hernia.

OBJECTIVES: Congenital diaphragmatic hernia (CDH) is a birth defect associated with long-term morbidity. Our objective was to examine longitudinal change in Functional Status Scale (FSS) after hospital discharge in CDH survivors. DESIGN: Single-center retrospective cohort study. SETTING: Center for comprehensive CDH management at a quaternary, free-standing children's hospital. PATIENTS: Infants with Bochdalek CDH were admitted to the ICU between January 2009 and December 2019 and survived until hospital discharge. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One hundred forty-two infants (58% male, mean birth weight 3.08 kg, 80% left-sided defects) met inclusion criteria. Relevant clinical data were extracted from the medical record to calculate FSS (primary outcome) at hospital discharge and three subsequent outpatient follow-up time points. The median (interquartile range [IQR]) FSS score at hospital discharge was 8.0 (7.0-9.0); 39 patients (27.5%) had at least moderate impairment (FSS ≥ 9). Median (IQR) FSS at 0- to 6-month ( n = 141), 6- to 12-month ( n = 141), and over 12-month ( n = 140) follow-up visits were 7.0 (7.0-8.0), 7.0 (6.0-8.0), and 6.0 (6.0-7.0), respectively. Twenty-one patients (15%) had at least moderate impairment at over 12-month follow-up; median composite FSS scores in the over 12-month time point decreased by 2.0 points from hospital discharge. Median feeding domain scores improved by 1.0 (1.0-2.0), whereas other domain scores remained without impairment. Multivariable analysis demonstrated right-sided, C- or D-size defects, extracorporeal membrane oxygenation use, cardiopulmonary resuscitation, and chromosomal anomalies were associated with impairment. CONCLUSIONS: The majority of CDH survivors at our center had mild functional status impairment (FSS ≤ 8) at discharge and 1-year follow-up; however, nearly 15% of patients had moderate impairment during this time period. The feeding domain had the highest level of functional impairment. We observed unchanged or improving functional status longitudinally over 1-year follow-up after hospital discharge. Longitudinal outcomes will guide interdisciplinary management strategies in CDH survivors.

A Tracheal Aspirate-derived Airway Basal Cell Model Reveals a Proinflammatory Epithelial Defect in Congenital Diaphragmatic Hernia.

Rationale: Congenital diaphragmatic hernia (CDH) is characterized by incomplete closure of the diaphragm and lung hypoplasia. The pathophysiology of lung defects in CDH is poorly understood. Objectives: To establish a translational model of human airway epithelium in CDH for pathogenic investigation and therapeutic testing. Methods: We developed a robust methodology of epithelial progenitor derivation from tracheal aspirates of newborns. Basal stem cells (BSCs) from patients with CDH and preterm and term non-CDH control subjects were derived and analyzed by bulk RNA sequencing, assay for transposase accessible chromatin with sequencing, and air-liquid interface differentiation. Lung sections from fetal human CDH samples and the nitrofen rat model of CDH were subjected to histological assessment of epithelial defects. Therapeutics to restore epithelial differentiation were evaluated in human epithelial cell culture and the nitrofen rat model of CDH. Measurements and Main Results: Transcriptomic and epigenetic profiling of CDH and control BSCs reveals a proinflammatory signature that is manifested by hyperactive nuclear factor kappa B and independent of severity and hernia size. In addition, CDH BSCs exhibit defective epithelial differentiation in vitro that recapitulates epithelial phenotypes found in fetal human CDH lung samples and fetal tracheas of the nitrofen rat model of CDH. Furthermore, blockade of nuclear factor kappa B hyperactivity normalizes epithelial differentiation phenotypes of human CDH BSCs in vitro and in nitrofen rat tracheas in vivo. Conclusions: Our findings have identified an underlying proinflammatory signature and BSC differentiation defects as a potential therapeutic target for airway epithelial defects in CDH.

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

BACKGROUND: The 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. METHODS: A 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. RESULTS: 102 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). CONCLUSION: We 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 EVIDENCE: Level III cohort study.

Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years.

OBJECTIVE: To determine if risk-adjusted survival of patients with CDH has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA: The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS: We divided registry data into 5-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS: 5203 patients from 23 centers with >22years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all P > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all P < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) ( P = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; P = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS: Risk-adjusted and observed-to-expected CDH mortality have improved over time.

Impact of bowel rotation and fixation on obstructive complications in congenital diaphragmatic hernia.

AIM OF THE STUDY: Small bowel obstruction (SBO) is a known complication after congenital diaphragmatic hernia (CDH) repair, which can require surgery and even extensive bowel resection causing short bowel syndrome (SBS). We investigate whether specific bowel rotation and fixation can be used as a predictor for SBO including volvulus. METHODS: A retrospective review of 256 CDH survivors following repair from 2003 to 2020 was performed. Operative notes and upper gastrointestinal series (UGI) were screened to determine the rotation and fixation of the bowel. Primary outcomes included SBO occurrence, SBO treated surgically, and volvulus. For statistical analysis Fisher's exact test was utilized. RESULTS: Twenty-two (9%) patients presented with SBO and majority, 19 (86%), required surgery. Adhesion were observed in 10 (45%), recurrence in 5 (23%), and extensive volvulus leading to SBS in 3 (14%). Both rotation and fixation were recorded in 117 (46%). Presence of left CDH with malrotation and nonfixation was a significant predictor for SBO requiring surgery (P<0.05 vs all other groups). All 3 patients with extensive volvulus had left CDH with nonfixed bowel (100%), however only 1 had malrotation (33%). CONCLUSIONS: Malrotation and nonfixation are associated with increased SBO in CDH. Normal rotation is not protective and patients are still at risk for volvulus resulting in SBS. SBO requiring surgical intervention is common in CDH. Bowel rotation and fixation are important determinants that, should be routinely documented and education about the risk of SBO should be included in family counseling. LEVEL OF EVIDENCE: Level IV - Case Series.

Diverting Ileostomy in Children With Functional Constipation: A Study Evaluating the Utility of Colon Manometry.

OBJECTIVES: Pediatric functional constipation (FC) may require invasive evaluations [like colon manometry (CM)] and surgical interventions [including diverting ostomy (DO)]. We evaluated the utility of CM in guiding surgery after DO. METHODS: Children with medically refractory FC undergoing an ostomy were included. Institutional Review Board approval was obtained for this retrospective study. Demographics and CM variables [high amplitude propagating contractions (HAPCs)] were recorded. Outcome measures: response to ostomy closure defined as successful if no need for further surgery after ostomy closure, and improvement on baseline CM after ostomy. A CM-guided ostomy closure algorithm was developed based on previous studies. We evaluated the association between response to ostomy closure and demographics, ostomy indication and CM improvement, and evaluated the role of CM predicting response using algorithm. RESULTS: A total of 60 children underwent ostomy for FC (median age: 7.1 years, range 0.15-23.6 years, 50% female). Ostomy was closed in 30 patients and deemed successful in 23 of 30. CM was performed in 42 of 60 patients before ostomy and in 29 of 30 before ostomy closure. We found no association between ostomy outcome and age, gender, weight, imaging studies, follow-up time, time with ostomy, HAPCs, and CM improvement. We found an association between failed response and ostomy indication of antegrade colonic enemas (ACE) failure ( P = 0.026) and successful response when ostomy closure was guided by algorithm ( P = 0.03). CONCLUSIONS: DO is a useful intervention in selected children with medically refractory FC, improving colon motility in most. CM can successfully guide the timing and type of ostomy closure. Larger studies are needed to further validate our findings.

Supraventricular Tachycardia in Infants With Congenital Diaphragmatic Hernia: Prevalence, Associations, and Outcomes.

OBJECTIVES: To characterize the prevalence, associations, management, and outcomes of supraventricular tachycardia (SVT) in neonates with congenital diaphragmatic hernia (CDH). DESIGN: Retrospective chart and cardiology code review within a cohort of patients with CDH was used to define a subpopulation with atrial arrhythmia. SVT mechanisms were confirmed by electrocardiogram analysis. Cox proportional hazard regression identified risk factors for SVT and association with clinical outcomes. SETTING: Medical Surgical ICU in a single, tertiary center, Boston Children's Hospital. PATIENTS: Eligible patients included neonates presenting with classic Bochdalek posterolateral CDH between 2005 and 2017, excluding newborns with Morgagni hernia or late diagnoses of CDH (>28 d). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: SVT arose in 25 of 232 neonates with CDH, (11%); 14 of 25 infants (56%) had recurrent SVT; atrioventricular node-dependent tachycardia was the most frequent mechanism (32%). The majority (71%) of SVT episodes received intervention. Nine patients (36%) received preventative antiarrhythmic medications. SVT was associated with lower Apgar score at 1 min, structural heart disease, larger defect size, extracorporeal membrane oxygenation (ECMO) support, and prostaglandin therapy for ductal patency as well as hospital stay greater than or equal to 8 weeks and use of supplemental oxygen at discharge. CONCLUSIONS: SVT can occur in neonates with CDH and frequently requires treatment. Odds of occurrence are increased with greater CDH disease severity, ECMO, and prostaglandin use. In unadjusted logistic regression analysis, SVT was associated with adverse hospital outcomes, underscoring the importance of recognition and management in this vulnerable population.

The in-utero diagnosis of choledochal cyst: can postnatal imaging predict benefit from early surgical intervention?

BACKGROUND: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications. METHODS: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed. RESULTS: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (n = 9), cyst resolution (n = 5), lack of IPU measurements (n = 3), or lack of follow-up (n = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n = 3; Asymptomatic: n = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n = 2; Asymptomatic: n = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation (p = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%). CONCLUSIONS: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.

High altitude simulation testing in patients with congenital diaphragmatic hernia.

AIM OF STUDY: Congenital Diaphragmatic Hernia (CDH) is associated with lung hypoplasia and pulmonary hypertension. Many patients receive care in specialty centers requiring air travel upon discharge and for subsequent follow-up care. Premature infants can experience significant hypoxia in flight, but this has not been studied in the CDH population. This report describes our center's experience with simulated altitude testing among CDH patients. METHODS: In a single center retrospective cohort study, CDH patients who underwent a High Altitude Simulation Test (HAST) from 2006 to 2019 were analyzed. HAST simulates increased altitude by reducing oxygen tension to an FIO2 of 0.15. Patients were tested only when flight was anticipated. Patients requiring oxygen were challenged on their baseline requirement. To pass, patients had to maintain oxygen saturation > 90%, and 94% if diagnosed with pulmonary hypertension. Supplemental oxygen was titrated as needed to achieve this goal. RESULTS: Of twenty patients tested, only six (30%) passed on their first attempt. Ten (50%) eventually passed, after an average of 3.2 additional attempts over 1.8 years. No patient passed initially who utilized ECMO support, diaphragmatic agenesis, or had elevated right ventricular pressure on echocardiogram. All patients achieved the targeted SpO2 with supplemental oxygen. CONCLUSION: CDH patients experience hypoxia when exposed to the simulated hypobaric nature of air travel and therefore may become hypoxic in flight, requiring oxygen supplementation. Disease severity seems to correlate with risk of in-flight hypoxia. This data suggests that CDH patients should be screened to assess their need for supplemental oxygen to ensure safe air travel. LEVEL OF EVIDENCE: Level 4 case series.

Novel Approach for Laparoscopically Placed Chronic Amniotic Fluid Catheters in Sheep.

INTRODUCTION: Several fetal therapies involve repeated amniotic fluid intervention. We hypothesize that a minimally invasive approach can be used to safely implant an intrauterine catheter infusion system in a fetal ovine model for chronic use during pregnancy. METHOD: Five pregnant sheep underwent operation between gestational days 110 and 115 (term 145 days). A Codman® implantable infusion pump was adapted for intrauterine use. The chamber was placed in the maternal flank and the tunneled catheter laparoscopically inserted into the amniotic cavity, secured with a pursestring. Three had an additional uterine anchoring suture. Ewes were sacrificed after natural delivery, and the uterus underwent gross and microscopic analyses. RESULTS: There were no maternal mortalities, abortions, or preterm labor. Pumps were accessed and remained functional throughout gestation. Four ewes delivered healthy term lambs; the other delivered twins with failure to progress and demise. On necropsy, catheters secured with an anchoring suture remained in place, while the other 2 dislodged during labor. There was no chorioamnionitis by culture or histology. CONCLUSION: Laparoscopically placed intra-amniotic infusion catheters were implanted safely and remained functional until delivery in an ovine model. This novel approach has promise in providing safe, durable amniotic fluid access for the potential treatment of fetal disease.

Prenatal Imaging Diagnosis of Suprarenal Lesions.

INTRODUCTION: Prenatal suprarenal lesions represent diverse pathologies. This study investigated prenatal imaging features and regression patterns associated with specific lesion diagnoses. METHODS: This is a multicenter retrospective review of fetuses with prenatally diagnosed suprarenal lesions between 2001 and 2019. Prenatal ultrasound and MRI characteristics, postnatal imaging, and clinical course were reviewed. Prenatal imaging findings were compared by the most common diagnoses and regression patterns. RESULTS: Forty-four fetuses were prenatally diagnosed with suprarenal lesions. Diagnoses included pulmonary sequestration (n = 12; 27.3%), adrenal hemorrhage (n = 12; 27.3%), upper quadrant cyst (including 2 duplication cysts, 1 splenic cyst, and 3 indeterminate cysts), neuroblastoma (n = 4), adrenal hyperplasia (n = 3), bilateral adrenal calcifications (n = 1), and indeterminate lesions (n = 6). Sequestrations were uniformly left-sided (100 vs. 50%; p = 0.014) and diagnosed earlier in gestation than adrenal hemorrhages (p = 0.025). Sequestrations were also significantly more likely to have a prenatal feeding vessel (p = 0.005), low T1 MRI signal (p = 0.015), and no MRI blood products (p = 0.018) compared to adrenal hemorrhages. When comparing all 44 patients, a prenatal feeding vessel and low T1 signal on prenatal MRI were significantly associated with lesion persistence (p = 0.003; p = 0.044). DISCUSSION/CONCLUSION: Imaging findings on prenatal ultrasound and MRI aid in the diagnosis of suprarenal lesions, including differentiating pulmonary sequestrations and adrenal hemorrhages.

Surgical Repair of Congenital Diaphragmatic Hernia After Extracorporeal Membrane Oxygenation Cannulation: Early Repair Improves Survival.

OBJECTIVE: To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation. SUMMARY BACKGROUND DATA: The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias. METHODS: This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017. Aim 1-Compare On versus After ECMO repair. Aim 2-Compare Early versus Late repair on ECMO. In order to minimize selection bias and account for non-repairs, subjects in each aim were stratified into study groups based on their treatment center's characteristics. In each aim, the study groups were matched based on propensity score (PS). The main outcomes included mortality rate and incidence of non-repair. RESULTS: In aim 1, 136 patients remained in each group after PS matching. Compared to the After ECMO group, patients in the On ECMO group demonstrated a lower mortality rate, hazard ratio (HR) 0.54 (0.38, 0.77) (P < 0.001), and lower incidence of non-repair, 5.9% versus 33.8% (P < 0.001). In aim 2, 77 patients remained in each group after PS matching. Compared to the Late group, Early repair of CDH on ECMO was associated with a lower mortality rate, HR 0.51 (0.33, 0.77) (P = 0.002), and lower incidence of non-repair, 9.1% versus 44.2% (P < 0.001). CONCLUSIONS: The approach of early repair after ECMO cannulation is associated with improved survival compared to delayed surgical correction.

Use of Cadaveric Skin Graft for Staged Gastroschisis Repair in a Premature Infant.

Gastroschisis is a congenital abdominal wall defect that, when simple, has excellent overall outcomes. However, morbidity increases with prematurity. A staged approach to closure is often needed until the infant can tolerate definitive repair. We demonstrate the novel use of cadaveric skin allograft as a defect patch, exploiting a tolerant neonatal immune system for long-term durable coverage. A 580-g, 26-week-gestation boy was born with gastroschisis. Primary closure was not possible, necessitating staged closure. After initial silo placement, neither the fascia nor the skin could be closed. Therefore, cadaveric skin was utilized for coverage: there was 100% take, no wound care needs, and no acute rejection. He was discharged at 4 months tolerating full feeds. At 6 months signs of rejection ultimately manifested, and he underwent uneventful elective graft excision and fascial closure. We offer this as a useful option for management of staged gastroschisis closure in an extremely premature infant.

Genetic diagnoses and associated anomalies in fetuses prenatally diagnosed with esophageal atresia.

Esophageal atresia (EA) is a congenital anomaly occurring in 2.3 per 10,000 live births. Due to advances in prenatal imaging, EA is more readily diagnosed, but data on the associated genetic diagnoses, other anomalies, and postnatal outcome for fetuses diagnosed prenatally with EA are scarce. We collected data from two academic medical centers (n = 61). Our data included fetuses with suspected EA on prenatal imaging that was confirmed postnatally and had at least one genetic test. In our cohort of 61 cases, 29 (49%) were born prematurely and 19% of those born alive died in the first 9 years of life. The most commonly associated birth defects were cardiac anomalies (67%) and spine anomalies (50%). A diagnosis was made in 61% of the cases; the most common diagnoses were vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula with esophageal atresia, radial or renal dysplasia, and limb anomalies association (43%, although 12% met only 2 of the criteria), trisomy 21 (5%), and CHARGE syndrome (5%). Our findings suggest that most fetuses with prenatally diagnosed EA have one or more additional major anomaly that warrants a more comprehensive clinical genetics evaluation. Fetuses diagnosed prenatally appear to represent a cohort with a worse outcome.