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Prosthetic patches (patch) and muscle flaps (flap) are techniques used for repair of congenital diaphragmatic hernia (CDH) with a large defect unamenable to primary closure. We hypothesized that the flap technique for CDH repair while on extra-corporeal membrane oxygenation (on-ECMO) would have decreased bleeding complications compared to patch due to the hemostatic advantage of native tissue. A single-center retrospective comparative study of patients who underwent on-ECMO CDH repair between 2008 and 2022 was performed. Fifty-two patients met inclusion criteria: 18 patch (34.6%) and 34 flap (65.4%). There was no difference in CDH severity between groups. On univariate analysis, reoperation for surgical bleeding was lower following flap repair compared to patch (23.5% vs 55.6%, respectively; p = 0.045), 48-h postoperative blood product transfusion was lower after flap repair (132 mL/kg vs 273.5 mL/kg patch; p = 0.006), and two-year survival was increased in the flap repair group compared to patch (53.1% vs 17.7%, respectively; p = 0.036). On multivariate analysis adjusting for CDH side, day on ECMO repaired, and day of life CDH repaired, flap repair was significantly associated with lower five-day postoperative packed red blood cell transfusion amount, improved survival to hospital discharge, and improved two-year survival. Our experience suggests that the muscle flap technique for on-ECMO CDH repair is associated with reduced bleeding complications compared to prosthetic patch repair, which may in part be responsible for the improved survival seen in the flap repair group. These results support the flap repair technique as a favored method for on-ECMO CDH repair. Level III. • The abdominal wall muscle flap technique can be used to repair large CDH defects and is feasible to perform on-ECMO. • The muscle flap technique is safe to perform on-ECMO and is associated with decreased postoperative bleeding complications, potentially making it the favored technique for large CDH defects which require on-ECMO repair. [ABSTRACT FROM AUTHOR]

Objective: To determine how blood gas exchange is altered during the transition in the first hour of life in infants with congenital diaphragmatic hernia (CDH).Study Design: This was a prospective observational cohort study evaluating arterial blood gas (ABG) samples and ventilator support in 34 infants with CDH in the first hour of life. Infants were stratified into mild, moderate, and severe CDH. The first ABG was compared with the umbilical cord ABGs and response to intervention evaluated on subsequent ABGs among infants with different CDH severities.Results: Infants were intubated at a median of 120 seconds (range 50-240 seconds) and ABGs obtained at a median of 6 minutes (IQR 4, 8 minutes), 16 minutes (IQR 13.5, 22.5 minutes), and 60 minutes (IQR 56, 64 minutes). Compared with the cord ABG, first ABG mean partial pressure of carbon dioxide (PaCO2) increased from 49.8 mm Hg to 82.1 mm Hg, mean base deficit decreased from -2.2 to -7.3, and mean pH from 7.298 to 7.060. With ventilator adjustments, second mean PaCO2 decreased to 76.7 mm Hg and third mean PaCO2 48.5 mm Hg. When stratified, with all CDH severities PaCO2 increased abruptly, remained elevated in moderate and severe CDH, and improved in all severities by 60 minutes after delivery.Conclusions: Gas exchange is markedly altered in the first hour of life in infants with CDH with abrupt onset of acidemia and a mixed respiratory and metabolic acidosis. Early implementation of adequate cardiopulmonary support may contribute to more timely stabilization of gas exchange. [ABSTRACT FROM AUTHOR]

Background: For infants with severe congenital diaphragmatic hernia (CDH) stabilized with extracorporeal membrane oxygenation (ECMO), early repair on ECMO improves outcome; however when compared to operative repair after ECMO, repair on ECMO is associated with increase bleeding risk and need for blood product transfusions. Methods: A retrospective review of 54 patients with CDH placed on ECMO prior to CDH repair was performed. For the subset of patients repaired on ECMO, analysis comparing those repaired early (within 48 h of cannulation) and late (beyond 48 h) on ECMO was performed. Outcomes of interest included survival to discharge, days on ECMO, and postoperative blood product utilization. Results: When compared to those patients repaired prior to 48 h of ECMO initiation, 57.7% of patients survived versus 40.9% of late repair patients. For those repaired early, blood product utilization was significantly less. Early repair patients received a median of 72 mL/kg packed red blood cells (PRBC) and 75 mL/kg platelets compared to 151.9 mL/kg and 98.7 mL/kg, respectively (p < 0.05 respectively). There was no difference in median days on ECMO (p = 0.38). Conclusion: Our data supports prior reports of improved outcome with repair with 48 h of ECMO initiation and suggests early repair on ECMO is associated with less bleeding and decreased blood product requirement in the postoperative period. [ABSTRACT FROM AUTHOR]

To identify factors associated with gastrostomy tube (GT) placement in infants with congenital diaphragmatic hernia (CDH). Retrospective 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. GT 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. Identification 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 II (Retrospective Study) [ABSTRACT FROM AUTHOR]

Objective: To determine whether postnatal echocardiographic indices of left ventricular (LV) size and function are associated with atrial-level shunt direction and investigate whether a relationship exists between LV function and LV size in patients with congenital diaphragmatic hernia (CDH).Study Design: This was a single-center retrospective study of 51 neonates with CDH evaluated at Children's Hospital Colorado. Initial postnatal echocardiograms were analyzed for cardiac dimensions, valvar integrity, cardiac time intervals, and biventricular function. Comparisons were made between neonates with left-to-right vs right-to-left atrial-level shunting, as well as between those with decreased (ejection fraction <55%) vs normal (ejection fraction ≥55%) LV function.Results: The majority (82.4%) of infants with CDH had a left-to-right atrial-level shunt, and 84.3% had systemic or suprasystemic pulmonary artery pressure. Decreased LV function was demonstrated in 37.2% and was associated with smaller LV volumes and worse outcomes, including the need for extracorporeal membrane oxygenation (ECMO) and survival.Conclusions: Left-to-right atrial-level shunting is common in neonates with severe CDH, even in the presence of suprasystemic pulmonary artery pressure. LV dysfunction correlates with decreased LV volumes and is associated with adverse neonatal outcomes, including increased need for ECMO and decreased survival. [ABSTRACT FROM AUTHOR]

Accurate, real-time technology is needed to predict which newborns with congenital diaphragmatic hernia (CDH) will require ECMO. The Compensatory Reserve Index (CRI) is a noninvasive monitoring technology that continuously trends an individual's capacity to compensate from normovolemia (CRI = 1) to decompensation (CRI = 0). We hypothesized that postnatal CRI values would be lower in CDH newborns that required ECMO than those who did not require ECMO. Newborns with a CDH were prospectively monitored with a CipherOx® CRI M1 device. We compared CRI values from delivery to ECMO (ECMO group) versus delivery to clinical stabilization (non-ECMO group). Postnatal CRI values were available from 26 newborns. Eight underwent ECMO within 33 h of delivery, and median CRI prior to ECMO was 0.068 (IQR: 0.057, 0.078). Eighteen did not require ECMO. Median CRI from birth to 48 h was 0.112 (IQR: 0.082, 0.15). CRI values were significantly lower in newborns that required ECMO versus those who did not (p = 0.0035). Postnatal CRI had the highest AUC (0.85) compared to other prenatal prognostic measures. Humans from newborns to adults share elemental features of the pulsatile waveform that are associated with progression to decompensation. CRI may be helpful when deciding when to initiate ECMO. Level III. Diagnostic test. [ABSTRACT FROM AUTHOR]

Congenital diaphragmatic hernia (CDH) repair is technically challenging with different approaches for repair. The aim of this study was to compare outcomes between patch and flap repair. CDH repairs performed at a quaternary care children's hospital between 2008 and 2018 were reviewed. Seventy CDH repairs were analyzed after primary repairs were excluded (n = 52). Analysis included proportions or median with interquartile range and chi-square or Wilcoxon rank-sum test. Comparing patch and flap repairs, demographic characteristics and hernia parameters were similar. Examining outcomes, length of stay, 30-day mortality, and 1-year mortality were all similar between groups, but total/symptomatic recurrence was higher in the patch group (10%/7% vs. 3%/0%), and the median time to recurrence was 3 months. A sub-analysis comparing those who recurred to those who did not demonstrated no significant difference between the two groups. Patients who undergo flap repair have a lower risk of recurrence compared to those who undergo patch repair, and this may be due to the ability of the flap to grow over time with the child. Treatment Study. III [ABSTRACT FROM AUTHOR]

Congenital diaphragmatic hernia (CDH) is a severe birth anomaly where a defect in the diaphragm allows abdominal organs to herniate into the chest with compression of the intrathoracic structures, specifically the lungs and heart. Pulmonary and left ventricular hypoplasia result in respiratory insufficiency after birth with disordered transition and persistent pulmonary hypertension of the newborn (PPHN). As a result, infants need immediate intervention after birth to support the transition. Delayed cord clamping (DCC) is recommended for all healthy newborns and improves outcomes in infants born preterm and in infants with congenital heart disease; however, DCC may not be feasible in newborns needing immediate intervention after birth. Recent studies have explored resuscitation with intact umbilical cords, to determine feasibility, safety, and efficacy in infants with CDH, with promising results. In this report we discuss the physiologic basis for intact cord resuscitation in infants with CDH and review the above reports with a view to determining optimal timing of umbilical cord clamping in infants with CDH. [ABSTRACT FROM AUTHOR]

Congenital diaphragmatic hernia (CDH) is characterized by a diaphragmatic defect with herniation of abdominal organs into the thoracic cavity. Outcomes are largely dependent on the degree of pulmonary hypoplasia and pulmonary hypertension, with the most severe cases associated with approximately 50% mortality. Long term pulmonary complications are well known, however, development of lobar emphysema leading to hemodynamic instability is exceedingly rare. We report the case of a patient with severe left-sided CDH who underwent successful diaphragmatic hernia repair but subsequently developed emphysematous bullous disease of the left lung with hemodynamic compromise, requiring emergent lung volume reduction surgery. [ABSTRACT FROM AUTHOR]

Objective: To determine whether prominent intrapulmonary anastomotic vessels (IPAVs) or bronchopulmonary "shunt" vessels can be identified in lungs from infants with fatal congenital diaphragmatic hernia (CDH).Study Design: We performed histology with immunostaining for CD31 (endothelium) and D2-40 (lymphatics), along with high-precision 3-dimensional (3D) reconstruction on lung tissue from 9 patients who died with CDH.Results: Each patient with CDH required mechanical ventilation, cardiotonic support, and pulmonary hypertension (PH)-targeted drug therapy. All patients were diagnosed with severe PH by echocardiography, and 5 received extracorporeal membrane oxygenation therapy. Death occurred at a median age of 24 days (range, 10-150 days) from refractory hypoxemia with severe PH, pneumonia, or tension pneumothorax. Histology showed decreased alveolarization with pulmonary vascular disease. In each patient, prominent IPAVs were identified as engorged, thin-walled vessels that connected pulmonary veins with microvessels surrounding pulmonary arteries and airways in lungs ipsilateral and contralateral to the CDH. Prominent anastomoses between pulmonary arteries and bronchial arteries were noted as well. The 3D reconstruction studies demonstrated that IPAVs connect pulmonary vasculature to systemic (bronchial) vessels both at the arterial and venous side.Conclusion: Histology and 3D reconstruction identified prominent bronchopulmonary vascular anastamoses in the lungs of infants who died with severe CDH. We speculate that IPAVs connecting pulmonary and bronchial arteries contribute to refractory hypoxemia in severe CDH. [ABSTRACT FROM AUTHOR]

Abstract: Traumatic airway perforation during endotracheal intubation is an uncommon but life-threatening complication in preterm infants. Death usually occurs at the time of the injury, but in rare cases where the infant survives the initial resuscitation, therapeutic options include conservative versus surgical management. We describe three cases of airway perforation treated successfully with surgical intervention and without lung resection, utilizing novel graft material and cardiopulmonary bypass to facilitate repair. In preterm infants who survive the initial injury we advocate for early identification and surgical management with cardiopulmonary bypass when feasible. [Copyright &y& Elsevier]

Objective: To predict incident bloodstream infection and urinary tract infection (UTI) in infants with congenital diaphragmatic hernia (CDH).Study Design: We conducted a retrospective analysis using the Children's Hospital Neonatal Database during 2010-2016. Infants with CDH admitted at 22 participating regional neonatal intensive care units were included; patients repaired or discharged to home prior to admission/referral were excluded. The primary outcome was death or the occurrence of bloodstream infection or UTI prior to discharge. Factors associated with this outcome were used to develop a multivariable equation using 80% of the cohort. Validation was performed in the remaining 20% of infants.Results: Median gestation and postnatal age at referral in this cohort (n = 1085) were 38 weeks and 3.1 hours, respectively. The primary outcome occurred in 395 patients (36%); and was associated with low birth weight, low Apgar, low admission pH, renal and associated anomalies, patch repair, and extracorporeal membrane oxygenation (P < .001 for all; area under receiver operating curve = 0.824; goodness of fit χ2 = 0.52). After omitting death from the outcome measure, admission pH, patch repair of CDH, and duration of central line placement were significantly associated with incident bloodstream infection or UTI.Conclusions: Infants with CDH are at high risk of infection which was predicted by clinical factors. Early identification and low threshold for sepsis evaluations in high-risk infants may attenuate acquisition and the consequences of these infections. [ABSTRACT FROM AUTHOR]

Objective: To determine whether endotracheal tube (ETT) insertion depth should be modified in infants with congenital diaphragmatic hernia (CDH) to reduce the risk of main-stem intubation.Study Design: The distance from the thoracic inlet to the carina was measured antenatally by fetal magnetic resonance imaging (MRI) between 20-28 weeks' (early) and 30-34 weeks' (late) gestation in 30 infants with CDH and compared with 12 early and 36 late MRIs in control infants without CDH. Postnatal tube position was assessed by chest radiograph in the same 30 infants with CDH and compared with 20 control infants with postnatal birth depression.Results: The carina position was displaced upward in fetuses and newborns with CDH. Distance from the thoracic inlet to the carina compared with controls was 1.04 ± 0.1 cm vs 1.42 ± 0.07 cm on early MRI (P < .05), 1.43 ± 0.14 cm vs 1.9 ± 0.04 cm on late MRI (P < .01), and 2.36 ± 0.07 cm vs 3.28 ± 0.05 cm on postnatal radiographs (P < .01). Adjusting the ETT depth by 1 cm resulted in a median distance of 1.27 cm from the tip of the ETT to the carina.Conclusion: Cephalad displacement of the carina in infants with CDH may predispose them to right main-stem intubation and subsequent development of pneumothorax. We speculate that modifying the ETT insertion depth to 5.5 cm + weight in newborns born at term may prevent pneumothoraces and improve outcomes for infants with CDH. [ABSTRACT FROM AUTHOR]

Objective: To assess the impact of specific echocardiographic criteria for timing of congenital diaphragmatic hernia repair on the incidence of acute postoperative clinical decompensation from pulmonary hypertensive crisis and/or acute respiratory decompensation, with secondary outcomes including survival to discharge, duration of ventilator support, and length of hospitalization.Study Design: The multidisciplinary congenital diaphragmatic hernia management team instituted a protocol in 2012 requiring the specific criterion of echocardiogram-estimated pulmonary artery pressure ≤80% systemic blood pressure before repairing congenital diaphragmatic hernias. A retrospective review of 77 neonatal patients with Bochdalek hernias repaired between 2008 and 2015 were reviewed: group 1 included patients repaired before protocol implementation (n = 25) and group 2 included patients repaired after implementation (n = 52).Results: The groups had similar baseline characteristics. Postoperative decompensation occurred less often in group 2 compared with group 1 (17% vs 48%, P = .01). Adjusted analysis accounting for repair type, liver herniation, and prematurity yielded similar results (15% vs 37%, P = .04). Group 2 displayed a trend toward improved survival to 30 days postoperatively, though this did not reach statistical significance (94% vs 80%, P = .06). Patient survival to discharge, duration of ventilator support, and length of hospitalization were not different between groups.Conclusions: The implementation of a protocol requiring echocardiogram-estimated pulmonary arterial pressure ≤80% of systemic pressure before congenital diaphragmatic hernia repair may reduce the incidence of acute postoperative decompensation, although there was no difference in longer-term secondary outcomes, including survival to discharge. [ABSTRACT FROM AUTHOR]

Objective: To assess the ability of vasopressin to stabilize hemodynamics in infants with systemic hypotension secondary to congenital diaphragmatic hernia (CDH). Study design: A retrospective chart review was performed to identify 13 patients with CDH treated with vasopressin for refractory hypotension to assess the effect of vasopressin on pulmonary and systemic hemodynamics and gas exchange in this setting. Data collected included demographics, respiratory support, inotropic agents, pulmonary and systemic hemodynamics, urine output, and serum and urine sodium levels during vasopressin therapy. Results: Vasopressin therapy increased mean arterial pressure and decreased pulmonary/systemic pressure ratio, heart rate, and fraction of inspired oxygen. In 6 of 13 patients, extracorporeal membrane oxygenation therapy was no longer indicated after treatment with vasopressin. Improvement in left ventricular function and oxygenation index after vasopressin initiation was associated with a decreased need for extracorporeal membrane oxygenation therapy. Prolonged vasopressin treatment was associated with hyponatremia, increased urine output, and increased urine sodium. Conclusions: Vasopressin stabilized systemic hemodynamics without adverse effects on pulmonary hemodynamics in a subset of infants with CDH. Our results suggest a potential role for vasopressin therapy in patients with CDH with catecholamine-resistant refractory hypotension. [Copyright &y& Elsevier]