Right Bochdalek congenital diaphragmatic hernia: a tertiary center\u2019s experience over 13 years

Alshaima Alghamdi,Enaam Raboe

doi:10.1186/s43159-021-00081-z

Abstract

BackgroundRight Bochdalek congenital diaphragmatic hernia (RB-CDH) is far less common than left Bochdalek congenital diaphragmatic hernia, accounting for only 13% of cases. There are limited published data on the outcomes and survival rate of RB-CDH.We aimed at investigating the clinical characteristics and analyzing the risk factors of survival in neonates with RB-CDH treated in our center over a period of 13 years.ResultsFifteen infants with RB-CDH were identified. Most of the patients were full term (74%). The mean birth weight was 2.90± 0.72 kg. The ratio of male to female was 2:1. The mean APGAR score at 1 min was 5.31±2.34, and 7.30±1.59 at 5 min. Ten patients (67%) were imaged by antenatal ultrasound. Eleven patients (73.33%) survived to go for surgical repair. The hernia sac was found in 5 patients (45%). Most hernial defects were closed in a primary fashion. The mean age at the operative repair was 8.11±9.90 days. The average NICU stay for all patients was 40.47±50.38 days. The mean follow-up period was 20.45±9.34 months. Three patients had postoperative complications. The total survival rate in neonates with RB-CDH was 9/15 (60%). Nine out of 11 (82%) neonates survived after surgical repair. Four patients (27%) died before surgical repair. Ventilation-related bilateral pneumothorax was a contributing cause of death in three patients. Birth weight was found lower in the non-survivor’s group (P < 0.05). Moreover, the degree of pulmonary hypertension was more severe among non-survivors. No statistical significance was observed between other variables and mortality.ConclusionWe found that low birth weight and the presence of severe PHTN were risk factors for mortality in neonates with RB-CDH. These results are in line with previous studies on prognostic factors in CDH. Ventilator-related pneumothorax appears to be a significant contributing cause of death. Long-term follow-up studies of infants born with RB-CDH are needed as small number of cases limits large-volume RB-CDH studies.

Highlights

Right Bochdalek congenital diaphragmatic hernia (RB-Congenital diaphragmatic hernia (CDH)) is far less common than left Bochdalek congenital diaphragmatic hernia, accounting for only 13% of cases
The average Neonatal intensive care unit (NICU) stay for all patients was 40.47±50.38 days
Neonates with CDH are at risk for prematurity and low birth weight (LBW), which predisposes them to increased mortality [13, 14]

Summary

Introduction

Right Bochdalek congenital diaphragmatic hernia (RB-CDH) is far less common than left Bochdalek congenital diaphragmatic hernia, accounting for only 13% of cases. There are limited published data on the outcomes and survival rate of RB-CDH. We aimed at investigating the clinical characteristics and analyzing the risk factors of survival in neonates with RBCDH treated in our center over a period of 13 years. While a growing proportion of cases are diagnosed prenatally, some are diagnosed at birth due to immediate respiratory distress. The posterolateral (Bochdalek) diaphragmatic hernia accounts for 90% of all diaphragmatic hernia cases. The right Bochdaleck congenital diaphragmatic hernia (RB-CDH) is less common than the left Bochdaleck congenital diaphragmatic hernia (LBCDH) (13% vs 85%, respectively) [4]. Pulmonary hypertension (PHTN) occurs in up to 75% of infants with CDH [5].

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