H. Sriraman1, M. Verla1, C. Style1, A. Mehollin-Ray2, C. Fernandes3, A. Vogel1, T. Lee1, S. Keswani1, O. Oluyinka1 1Baylor College Of Medicine, Michael E. DeBakey Department of Surgery,Texas Children’s Fetal Center,Houston, TX, USA 2Texas Children’s Hospital – Fetal Center,Department Of Radiology,Houston, TX, USA 3Texas Children’s Hospital,Departments Of Pediatrics – Newborn Section,Houston, TX, USA
Introduction: Bilateral congenital diaphragmatic hernia (CDH) is a rare variant of CDH with a mortality rate as high as 74%. However, we hypothesize that not all variants of bilateral CDH have a poor prognosis. The aim of our study was to evaluate our institution’s postnatal outcomes of neonates with bilateral congenital diaphragmatic hernia to determine which elements may portend a better prognosis.
Methods: Following IRB approval, a single center, retrospective review of all patients with bilateral CDH evaluated from January 2004 to December 2017 was performed. Demographics, associated congenital abnormalities, type of CDH defect, operative repair and approach, ECMO use and survival were collected. Descriptive statistics were used to analyze the data.
Results: Over the 14-year study period, 282 patients with CDH were identified and 7 had a bilateral intrapleural defect. Six of the seven neonates with bilateral CDH were diagnosed prenatally. Four neonates had posterior-lateral defects, while the other three neonates had anterior CDH defects. Three neonates had at least one concomitant major congenital anomaly, but none had a genetic anomaly (Table 1). The median gestational age at birth was 38 weeks (IQR: 37, 39) and birthweight was 3020 grams (IQR: 2288, 3525). The median Apgar scores at 1 and 5 minutes were 6 (IQR: 3, 7) and 8 (IQR: 7, 8), respectively. None of the seven patients required ECMO and the overall cohort survival was 57% (Table 1). Median age at follow-up was 2 years (IQR: 0.4, 7). All the patients with anterior defects received a primary repair, while two of the four neonates with posterior-lateral defects had some form of patch repair.
Conclusion: Prognosis from bilateral intrapleural CDH may not be as grim as initially reported. Compared to posterior-lateral defects, patients with large bilateral anterior defects have a relatively benign course. This case series indicates good outcomes for patients with bilateral anterior CDH defects which suggests that not all patients with bilateral CDH will have a dismal outcome. Better prenatal determination of anterior versus posterior bilateral CDH may be helpful when counseling about clinical outcomes.
