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Bilateral congenital diaphragmatic hernia
Holly L Neville1, Tom Jaksic, Jay M Wilson
1Department of Surgery, University of Texas-Houston Medical School, Houston, TX 77030, USA.
Insights
Bilateral congenital diaphragmatic hernia (CDH) is rare and associated with high mortality. Infants with bilateral CDH often have other anomalies, complicating management and requiring prompt evaluation.
Area of Science:
- Neonatal surgery
- Pediatric surgery
- Congenital abnormalities
Background:
- Congenital diaphragmatic hernia (CDH) affects approximately 1 in 2,450 live births.
- Bilateral CDH is an extremely rare form of CDH, presenting unique management challenges for neonatologists and surgeons.
Purpose of the Study:
- To detail the management and outcomes of infants diagnosed with bilateral congenital diaphragmatic hernia.
- To analyze the characteristics and survival rates of bilateral CDH cases.
Main Methods:
- Retrospective review of liveborn patients with CDH between 1995 and 2001 from an 83-hospital database.
- Analysis of data from 17 patients with bilateral CDH, including demographics, associated anomalies, and treatment outcomes.
- Statistical analysis using the Chi-squared test to compare outcomes.
Main Results:
- Bilateral CDH represented 0.9% of CDH cases (17 out of 1833).
- A high mortality rate of 65% was observed in bilateral CDH cases, significantly higher than the 33% in unilateral CDH (P=0.01).
- Seventy percent of bilateral CDH patients had other major anomalies, including cardiac and chromosomal abnormalities.
Conclusions:
- Infants with bilateral congenital diaphragmatic hernia face significant management difficulties and a high mortality rate.
- The frequent association of bilateral CDH with other anomalies necessitates thorough evaluation for additional abnormalities.
Background/Purpose:
CDH occurs in approximately 1 in 2,450 live births. Bilateral CDH, previously identified through a limited number of case reports, is extremely rare. The care of CDH patients is a challenge for neonatologists and surgeons. This report details the management and outcome of patients with bilateral CDH.
Methods:
Records of all liveborn patients with CDH between 1995 and 2001 in 83 hospitals were entered into the CDH database. Those with bilateral CDH were reviewed retrospectively. Data were analyzed using the Chi;(2) test.
Results:
A total of 1833 patients were entered in the database, 17 of these had bilateral CDH (0.9%). Eleven were boys. The average birth weight was 2.6 kg. The average gestational age was 36.8 weeks. Sixteen patients experienced early distress requiring intubation (12 immediately), and 4 were placed on extracorporeal membrane oxygenation (ECMO). Seven patients were diagnosed prenatally. Twelve patients (70%) were found to have other anomalies, 3 had chromosomal abnormalities and 7 had cardiac anomalies. These included tetralogy of Fallot (TOF), VSD, absence of the pericardium, coarctation of the aorta (2), accessory SVC with aortic coarctation, and ASD with TOF. Only 9 of 17 (53%) patients underwent surgical repair (6 primary, 3 patch). Mortality rate was 65% compared with 33% of patients with unilateral CDH (P = 0.01). Seven patients died within 48 hours of birth. There was no significant difference in survival based on gender, weight, gestational age, presence of anomalies, or prenatal diagnosis.
Conclusions:
The management of infants with bilateral congenital diaphragmatic hernia remains a difficult problem with a significant mortality. Bilateral congenital diaphragmatic hernia is associated more frequently with other major anomalies than unilateral congenital diaphragmatic hernia and should prompt an evaluation for further anomalies.
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