Timing Of Congenital Diaphragmatic Hernia Repair Research Articles

Long-Term Evaluation of Gastroesophageal Reflux in Neonates with and without Preventive Anti-reflux Surgery at the Time of Congenital Diaphragmatic Hernia Repair.

One potential comorbidity after congenital diaphragmatic hernia (CDH) is gastroesophageal reflux (GER), which can have a substantial effect on patients’ quality of life, thriving, and complications later in life. Efforts have been made to reduce gastroesophageal reflux with a preventive anti-reflux procedure at the time of CDH repair. In this follow-up study of neonates participating in a primary RCT study on preventive anti-reflux surgery, symptoms of GER were assessed longitudinally. Long-term data with a median follow-up time of ten years was available in 66 patients. Thirty-one neonates received an initial fundoplication. Secondary anti-reflux surgery was necessary in 18% and only in patients with large defects. It was required significantly more often in patients with intrathoracic herniation of liver (p = 0.015) and stomach (p = 0.019) and patch repair (p = 0.03). Liver herniation was the only independent risk factor identified in multivariate regression analysis. Primary fundopexy and hemifundoplication did not reveal a protective effect regarding the occurrence of GER symptoms, the need for secondary antireflux surgery or the gain of body weight regardless of defect size neither in the short nor in the long term. Symptoms of GER must be assessed carefully especially in children with large defects, as these are prone to require secondary anti-reflux surgery in the long-term. Routine evaluation of GER including endoscopy and impedance measurement should be recommended especially for high-risk patients.

Variation across centers in standardized mortality ratios for congenital diaphragmatic hernia receiving extracorporeal life support

BackgroundWe sought to elucidate the degree of variation across centers by calculating center-specific standardized mortality ratios (SMRs) for infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS). MethodsThe Extracorporeal Life Support Organization (ELSO) registry data (2000–2019) were used to estimate SMRs. Center-specific SMRs and their 95% confidence intervals (CIs) were used to identify centers with mortality as significantly worse (SW), significantly better (SB), or not different (ND) relative to the median standardized mortality rate. ResultsWe identified 4,223 neonates with CDH from 109 centers. SMRs were risk-adjusted for pre-ECLS case-mix (birthweight, sex, race, 5 min Apgar, blood gases, gestational age, hernia side, prenatal diagnosis, pre-ECLS arrest, and comorbidities). Observed (unadjusted) mortality rates across centers varied substantially (range: 14.3%–90.9%; interquartile range [IQR]: 42.9%–62.1%). Thirteen centers (11.9%) had SB SMRs< 1 (SMR 0.52 to 0.84), 7 centers (6.4%) had SW SMRs>1 (SMR 1.25 to 1.43), and 89 centers (81.7%) had SMRs ND relative to the median SMR rate across all centers (i.e., SMR not different from one). Descriptive analyses demonstrated that SB centers had a lower proportion of cases with renal complications, infectious complications and discontinuation of ECLS owing to complications, as well as differences in pre-ECLS treatments and timing of CDH repair, compared to SW and ND centers. ConclusionThis study specifically identified ECLS centers with higher and lower survival for patients with CDH, which may serve as a benchmark for institutional quality improvement. Future studies are needed to identify those specific processes at those centers that leads to favorable outcomes with the goal of improving care globally. Level of evidenceLevel III.

Appropriate timing of surgery for neonates with congenital diaphragmatic hernia: early or delayed repair?

This study was aimed to evaluate the influence of timing of surgery on patient outcomes, and to clarify appropriate timing of surgery in neonates with congenital diaphragmatic hernia (CDH). A total of 477 neonates with isolated CDH were included. Patients were classified into two groups by timing of surgery: early repair (ER) (≤48h) and delayed repair (DR) (>48h). The primary outcome was 90-day survival, with treatment duration (ventilation, oxygen, and hospitalization) being a secondary outcome. To adjust for disease severity, patients were stratified into three severities by Apgar score 1min ("mild" 8-10, "moderate" 4-7, and "severe" 0-3), and outcomes were compared between ER and DR within each severity. Although 90-day survival was significantly different among the three severities ("mild" 97%, "moderate" 89%, and "severe" 76%, p=0.002), there were no differences in 90-day survival between DR and ER within each severity. In "mild", there were no differences in treatment duration between ER and DR. In "moderate", treatment duration was shorter in ER than DR (ventilation 11 vs. 16days, oxygen 15 vs. 20days, and hospitalization 34 vs. 48days). In "severe", treatment duration was shorter in ER than DR, while the best OI was higher in DR than ER. Timing of CDH repair seems to have no influence on 90-day survival regardless of disease severity. Patients with moderate severity may benefit from the early repair by reducing treatment duration.

A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: Does timing of operation matter?

Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH. Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated. Patients were stratified by timing of repair: Day of life (DOL) 0-3 (group 1), 4-7 (group 2), or >8 (group 3), and the effect of surgical timing on mortality was determined by logistic regression and risk-adjusted for severity of illness. The unadjusted odds ratio (OR) for mortality increased significantly with delayed repair (group 2, 1.73 [95% CI, 1.00-2.98; group 3, 3.42 [95% CI, 1.97-5.96]). However, when adjusted for severity of illness, delay in repair did not predict increased mortality (group 2, 1.2 [95% CI, 0.7-2.2]; group 3, 1.4 [95% CI, 0.8-2.6]), nor did it portend an increased need for postoperative ECMO (group 2, 1.1 [95% CI, 0.5-2.4]; group 3, 0.5 [95% CI, 0.2-1.4]). After adjustment for known risk factors, the timing of CDH repair in low-risk infants does not seem to influence mortality. However, specific clinical parameters guiding timing of elective CDH repair remain unknown.

Abdominal wall closure in neonates after congenital diaphragmatic hernia repair

PurposeRepair of Congenital Diaphragmatic Hernia (CDH) abruptly increases intra-abdominal pressure. This study sought to characterize the incidence and significance of ACS and delayed fascial closure (DFC) after CDH repair. MethodsWe reviewed the CAPSNet database from 2006 to 2011, identifying the subset of patients that developed ACS or required DFC. Prenatal and demographic information, operative and physiologic details, and outcomes were investigated. ResultsOf 349 patients with CDH, 3 (0.8%) were diagnosed with ACS, while 43 patients (12%) had DFC at the time of CDH repair. Patients more often had right-sided defects (26% vs 13%, p=0.04) and trended toward requiring a patch repair (41% vs 31.2%, p=0.23) and having a liver lobe above the diaphragmatic rim (47% vs 32.7, p=0.09).Patients with ACS or DFC had increased length of stay (47.5 vs 33.9, p=0.01), days fasting (8.2 vs 5.8, p=0.01), days on parenteral nutrition (23.6 vs 15.5, p=0.003), and days on mechanical ventilation (16.3 vs 9.0, p=0.001). ConclusionsWhile ACS in neonates after CDH repair is rare (<1%), DFC is required relatively commonly (>10%) and is associated with right-sided diaphragmatic hernias. Inability to close abdominal fascia is associated with increased morbidity. Clinicians caring for neonates with CDH should be facile with strategies to manage delayed abdominal fascia closure.

The impact of chylothorax on neonates with repaired congenital diaphragmatic hernia

PurposeChylothorax is a known complication in neonates after congenital diaphragmatic hernia (CDH) repair. This report uses a large international registry to evaluate risk factors, treatment, morbidity, and survival associated with chylothorax in a prospective cohort of neonates after CDH repair. MethodsFrom January 2007 to January 2010, live-born neonates with repaired, unilateral CDHs were evaluated from a prospective database for chylothorax development. Chylothorax was diagnosed based on pleural fluid examination. Study variables included patient characteristics, CDH defect and disease severity characteristics, chylothorax treatment, and survival. In addition, the temporal relationship between timing of CDH repair and extracorporeal membrane oxygenation (ECMO) therapy was evaluated as a risk factor for chylothorax. Univariate and multivariate regression analyses were utilized. ResultsAmong the 1383 patients evaluated, chylothorax was diagnosed in 4.6% of the cohort. Patch repair and ECMO were statistically significant risk factors for chylothorax. The odds of developing a chylothorax were significantly increased in patients with CDH repair on ECMO (aOR 2.6; 95% CI: 1.3–4.9) or after ECMO (aOR 3.1; 95% CI: 1.7–5.8). Most chylothoraces (83.1%) were successfully treated without surgery. Chylothorax patients had significant morbidity including increased oxygen use at 30days and longer length of stay. Survival was not significantly affected by chylothorax. ConclusionsChylothorax is a known but uncommon complication of neonatal CDH repair. In this very large series of chylothorax in association with CDH, major risk factors appear to be related to increased disease severity with the highest risk in patients repaired on or after ECMO. Chylothoraces usually improve with conservative therapy and lead to significant morbidity but not increased mortality.

Antireflux surgery after congenital diaphragmatic hernia repair: a plea for a tailored approach

Preventive antireflux surgery (ARS) at the moment of congenital diaphragmatic hernia (CDH) repair has been suggested by some authors, particularly in subgroups with a liver herniated in the chest or patch requirement. We evaluated the incidence and associated factors of gastro-oesophageal reflux disease (GERD) and the need for subsequent ARS in our CDH patients. We retrospectively reviewed our CDH database. Demographics, prenatal assessment of severity, prenatal treatment, type of repair, intraoperative findings and incidences of gastro-oesophageal reflux and ARS were recorded. CDH repair was performed in 77 infants between July 1993 and November 2009. Eight died after repair. Seven were lost to follow-up. The median follow-up was 4.0 (0.16-14.88) years. Fourteen of these 62 patients were prenatally treated with fetoscopic endoluminal tracheal occlusion (FETO) because of severe pulmonary hypoplasia. After CDH repair, GERD was diagnosed in 31 patients. In all of them, medical antireflux treatment was started. Thirteen (42%) patients needed ARS at a median age of 64 (37-264) days. One year after starting medical treatment, 14 (45%) patients were completely off antireflux medication. In CDH subgroups with patch repair, liver herniated in the chest or previous FETO, the incidences of gastro-oesophageal reflux and ARS were 61 and 32%, 73 and 38% and 71 and 43%, respectively. Univariable analysis of associated potentially predisposing factors shows that patch repair, liver herniated in the chest, pulmonary hypertension, high-frequency oscillatory ventilation and FETO are associated with subsequent ARS. On multivariable analysis, liver herniated in the chest was the only independent predictor for both gastro-oesophageal reflux and ARS. Of all CDH patients, 50% developed gastro-oesophageal reflux and 21% required ARS. For both, liver in the chest was the only independent predictor. Routine ARS in certain subgroups at the time of CDH repair seems not to be justified. Foetal endoluminal tracheal occlusion creates a new cohort of survivors with an increased risk for undergoing ARS. The surgical group, in particular, reflects a more complex gastro-oesophageal reflux physiopathology.

Preventive antireflux surgery in neonates with congenital diaphragmatic hernia: a single-blinded prospective study

Objective Congenital diaphragmatic hernia (CDH) is known to be a predisposing factor in gastroesophageal reflux (GER) leading to pulmonary and nutritional problems. The aim of this prospective, randomized, patient-blinded study was to evaluate the benefit of antireflux surgery at the time of CDH repair. Methods From 2003 to 2009, 79 neonates with left-sided CDH were included. Forty-three had regular hernia closure. Thirty-six patients additionally had fundoplication at hernia repair. Follow-up was at 6, 12, and 24 months after birth with a standardized questionnaire and a thorax radiograph. Patients with clinical signs for GER were evaluated with upper gastrointestinal series and 24-hour pH-metry. Results Seventy-nine of 263 patients participated in this prospective trial. Survival rate was 88.61%. The GER symptoms were almost significantly more frequent in the group without concomitant fundoplication at the age of 6 months. At 24 months, the difference between both groups was not significant anymore. Development of body weight in the first 2 years of life was similar in both groups. No complications related to initial antireflux surgery were noted. Conclusion Patients profit from fundoplication at CDH repair only within the first year of life. At the present point of this study, simultaneous fundoplication at the time of primary CDH repair cannot be recommended as a standard procedure in all patients with left-sided CDH.

Anesthesia Literature Review

Anesthesia Literature Review

Anterior fundoplication at the time of congenital diaphragmatic hernia repair

PurposeThe loss of normal anatomic barriers in neonates with congenital diaphragmatic hernia (CDH) can predispose children to gastroesophageal reflux (GER). In an attempt to improve post-operative feeding, we have added a modified anterior fundoplication to restore natural gastric and esophageal positioning.MethodsThe institutional review board of both participating centers approved this study. Between 1997 and 2008, 13 neonates with high-risk anatomy underwent repair of CDH combined with an anterior fundoplication (Boix-Ochoa). The anatomic indications for concomitant fundoplication were absence of an intra-abdominal esophagus, an obtuse angle of His, and a small, vertically oriented stomach.ResultsTen patients survived to discharge and eight were on full oral nourishment. One required partial gastrostomy feedings for an improving oral aversion and quickly progressed to full oral feedings. One patient with chromosomal anomalies and swallowing dysfunction remained on long-term bolus gastrostomy feedings. Two with progressive symptoms of GER and failure to thrive required conversion to a 360° wrap after 18 months of medical management. This was performed in conjunction with a planned, staged muscle flap reconstruction in one patient. There were no complications related to the fundoplication.ConclusionAnatomic predictors of severe GER can be efficiently countered at the time of CDH repair. A modified fundoplication should be considered in the operative management of high-risk infants.

Predicting the need for fundoplication at the time of congenital diaphragmatic hernia repair

Purpose This study was conducted to examine the preoperative factors predictive of subsequent intervention for gastroesophageal reflux (GER) in children with congenital diaphragmatic hernia (CDH). Methods We conducted a retrospective cohort study on children who underwent repair of a CDH between January 1, 1995, and December 31, 2002 with follow-up continuing to September 1, 2005. Excluded in the study were children who died during their first admission, or who underwent fundoplication at the time of CDH repair. Univariate and multivariate logistic regressions were performed to examine preoperative factors predictive of subsequent intervention (fundoplication or gastrojejunal tube placement). Results Of 86 children, 13 underwent intervention (fundoplication, 10; gastrojejunal tube, 3) for GER. Univariate predictors included the following: right-sided CDH, use of nonconventional ventilation, liver within the chest, and patch closure of the CDH. However, only liver within the chest and patch closure of the CDH were significant predictors in a multiple variable analysis. The positive and negative predictive values of the multivariate model were 69.2% and 87.7%, respectively. Conclusions Infants with CDH who have liver within the chest or require patch closure of their hernia are at increased risk for subsequent intervention for GER. These children may represent a subpopulation that would benefit from fundoplication at the time of CDH repair.