Search

Abstract: Purpose: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency. Methods: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30. Results: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p =0.02), but not OELHR (p =0.12) or PPLV (p =0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p =0.03, p =0.02, p =0.01), ECMO (p <0.01, p <0.01, p =0.03), and ventilator dependency (p <0.01, p <0.01, p =0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p =0.04), ECMO (p =0.03), and ventilator dependency (p =0.02), while LHR was associated with ECMO (p =0.01) and ventilator dependency (p =0.02) but not mortality (p =0.06). Conclusion: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes. [Copyright &y& Elsevier]

Abstract: Purpose: Patients with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS) are at increased risk for acute kidney injury (AKI). We hypothesized that AKI would be associated with increased mortality. We further hypothesized that vasopressor requirement, nephrotoxic medications, and infections would be associated with AKI. Methods: We performed a retrospective chart review in all patients with CDH requiring ECLS from 1999 to 2009 (n = 68). Patient variables that could potentiate renal failure were collected. We used a rise in creatinine from baseline by the RIFLE (risk, 1.5×; injury, 2×; failure, 3×; loss; and end-stage renal disease) criteria to define AKI. Statistical analysis was performed via SPSS (SPSS, Chicago, IL) using Student t test and χ 2 analysis, with P < .05 being considered significant. Results: Survival to hospital discharge was 37 (54.4%) of 68. Acute kidney injury was identified in 48 (71%) of 68 patients, with 15 (22% of all patients) qualifying as injury and 33 (49% of all patients) qualifying as failure by the RIFLE criteria. Patients who qualified as failure by the RIFLE criteria had a significant decrease in survival (27.3% with failure vs 80% without failure; P = .001). Patients who qualified as failure also had increased length of ECLS (314 ± 145 vs 197 ± 115 hours; P = .001) and decreased ventilator-free days in the first 60 days (1.39 ± 5.3 vs 20.17 ± 17.4 days; P = .001). There was no significant difference in survival when patients qualified as risk or injury. Conclusions: This is the first report using a systematic definition of AKI in patients with CDH on ECLS. There is a high incidence of AKI in these patients, and when it progresses to failure, it is associated with higher mortality, increased ECLS duration, and increased ventilator days. This highlights the importance of recognizing AKI in patients with CDH requiring ECLS and the potential benefit of preventing progression of AKI or early intervention. [Copyright &y& Elsevier]

Abstract: Purpose: Chylothorax after congenital diaphragmatic hernia (CDH) repair contributes significantly to morbidity. Our aim was to identify factors contributing to chylothorax and effective treatment strategies. Methods: We reviewed 171 patients with CDH from 1997 to 2008 and analyzed hernia characteristics, extracorporeal membrane oxygenation (ECMO) use, operative details, and treatment approaches for chylothorax. Results: Ten (7%) patients developed chylothorax; all were left sided. Using univariate analysis, prenatal diagnosis, ECMO use, and patch repair were associated with development of chylothorax. Logistic regression analysis showed that patch repair was the only variable predictive of chylothorax (P = .028; confidence interval, 0.032-0.823). Although survival was not affected, patients with chylothorax had a significant increase in ventilator days and length of stay (t = 3.57; P = .000; t = 2.74; P = .007). All received thoracostomy and total parenteral nutrition. Six patients received octreotide, 5 of whom required pleurectomy because of failed medical management; the remaining patient died of overwhelming sepsis. Conclusions: The incidence of chylothorax at our institution was relatively low. Patch repair was associated with the formation of chylothorax. Morbidity was substantial, but survival was not significantly affected. Total parenteral nutrition and thoracostomy were appropriate initial treatments. Octreotide was not an effective adjunct. Refractory cases were successfully treated with pleurectomy. [Copyright &y& Elsevier]

Abstract: Purpose: Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy. Methods: We examined deidentified data from the CDH study group (CDHSG) registry from 1995 to 2005 on patients who underwent repair and ECMO therapy (n = 636). We used Cox regression analysis to assess differences in survival between those who underwent repair during and after ECMO. Results: Five covariates were significantly associated with mortality as follows: timing of repair relative to ECMO (P = .03), defect side (P = .01), ECMO run length (P < .01), need for patch repair (P = .03), birth weight (P < .01), and Apgar score at 5 minutes (P = .03). Birth year, inborn vs transfer status, diaphragmatic agenesis, age at repair, and presence of cardiac or chromosomal abnormalities were not associated with survival. Repair after ECMO therapy was associated with increased survival relative to repair on ECMO (hazard ratio, 1.407; P = .03). Conclusion: These data suggest that CDH repair after ECMO therapy is associated with improved survival compared to repair on ECMO, despite controlling for factors associated with the severity of CDH. [Copyright &y& Elsevier]

Abstract: Purpose: The purpose of the study was to assess the treatment strategies and outcome of right-sided congenital diaphragmatic hernia (R-CDH), particularly extracorporeal membrane oxygenation (ECMO). Methods: We reviewed the cases of 42 patients treated for R-CDH at our institution from 1991 to 2006. We gathered demographic information, documented ECMO use and the type of surgical repair, and compared outcomes with predicted survival as calculated by the CDH Study Group''s equation. Results: Of the 35 patients included in our statistical analysis (7 were excluded), 12 (34%) were born at our institution, all of whom were prenatally diagnosed with R-CDH. Nineteen patients (54%) required ECMO therapy. Extracorporeal membrane oxygenation was initiated after repair of the R-CDH in 2 patients (11%). Of those patients who went on ECMO before repair, 4 patients (21%) were repaired on ECMO, 9 patients (47%) underwent repair after ECMO, and 4 patients (21%) underwent ECMO but died before their R-CDH could be repaired. Primary repair of the diaphragm was possible in 15 cases (56%), and primary closure of the abdominal incision was possible in 15 of the 23 open repairs (65%). The mean predicted survival for all 35 patients was 63%, whereas 28 (80%) actually survived. Logistic regression showed a significant association between the presence of cardiac defects and mortality (odds ratio = 0.008, P = .014). Conclusions: Our data suggest that patients with R-CDH have high ECMO utilization and may experience greater relative benefit from ECMO as evidenced by their higher-than-expected overall survival. Extracorporeal membrane oxygenation may be found to have a distinctive role in managing R-CDH. More high-powered series are needed to elucidate differences between R-CDH and left-sided CDH that may dictate alternate forms of management. [Copyright &y& Elsevier]

Abstract: Background: Infants with severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) have a high morbidity and mortality. We hypothesized that placement of an abdominal wall silo and staged abdominal wall closure may reduce problems associated with decreased abdominal domain in CDH. Methods: We performed a retrospective review and identified 7 CDH patients requiring ECMO who had a silastic abdominal wall silo between 2003 and 2006. Variables analyzed included survival, ECMO duration, duration of silo, time to discharge, and long-term outcome. Results: Predicted mean survival for the entire cohort using the published CDH Study Group equation was 47% (range, 9%-86%). All 7 patients (100%) survived. Extracorporeal membrane oxygenation duration averaged 15 days (range, 5-19 days). Four of the patients (58%) were repaired with a silo on ECMO, and 3 (42%) had their repair after ECMO. The abdominal wall defect was closed at a mean of 21 days (range, 4-41 days). Hospital stay after silo placement averaged 54 days (range, 20-170 days) with no infections or wound complications. Conclusions: Abdominal wall silo placement in infants with CDH requiring ECMO appears to be an effective strategy for decreased abdominal domain. Further studies are warranted to determine the efficacy of such a strategy for these high-risk CDH patients. [Copyright &y& Elsevier]