Right congenital diaphragmatic hernia: prenatal assessment and outcome

• Published in: Journal of pediatric surgery 2004-03, Vol.39 (3), p.319-323
• Main Authors: Hedrick, Holly L, Crombleholme, Timothy M, Flake, Alan W, Nance, Michael L, von Allmen, Daniel, Howell, Lori J, Johnson, Mark P, Wilson, R.Douglas, Adzick, N.Scott
• Format: Article
• Language: English
• Subjects: Extracorporeal Membrane Oxygenation; Female; Fetus - abnormalities; Gestational Age; Hernia, Diaphragmatic - diagnosis; Hernia, Diaphragmatic - mortality; Hernia, Diaphragmatic - therapy; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Liver - abnormalities; outcome; Pregnancy; Pregnancy Complications; Prenatal Diagnosis; Retrospective Studies; Right congenital diaphragmatic hernia; Survival Rate; Treatment Outcome
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2003.11.006

To understand the natural history of right congenital diaphragmatic hernia (CDH), the authors retrospectively reviewed 27 cases of right CDH that presented for prenatal evaluation or postnatal treatment. Between 1995 and September 2002, a total of 194 cases of fetal CDH were evaluated and included 22 right-sided defects. The authors reviewed prenatal diagnostic studies (ultrasound scan, magnetic resonance imaging [MRI] echocardiography) and pre- and postnatal outcomes in these 22 cases of right CDH. Five additional cases of right CDH without a prenatal diagnosis were reviewed. The mean gestational age at evaluation was 26.1 weeks. The lung area to head circumference ratio (LHR) ranged from 0.32 to 2.5. In all cases, the fetal liver was herniated into the right chest. Associated anomalies were common. There were no karyotype abnormalities (17 of 22 tested). There were 4 terminations. Nine of the 18 (50%) continuing pregnancies had polyhydramnios, premature rupture of membranes, or preterm labor. The mean gestational age at birth was 36.8 weeks. One patient underwent tracheal occlusion at 27 weeks, and 2 patients died before postnatal repair. Overall survival rate (22 prenatal plus 5 postnatal diagnoses) was 19 of 27 (70%). Postnatal survival rate was 19 of 23 (83%). A Gore-tex (W. L. Gore and Associates, Flagstaff, AZ) patch was utilized in 14 of 21 neonates undergoing surgery. Twelve of 23 (52%) required extracorporeal membrane oxygenation (ECMO) with a 75% survival rate. Significant morbidity occurred in 10 of 19 survivors and included neurologic sequelae in 6 of 19 (32%). MRI was helpful in the determination of liver position and confirmation of diagnosis. The high incidence of preterm complications, frequent need for ECMO, and high prevalence of comorbidities are indicative of the severity of this CDH population and warrant close prenatal surveillance and delivery at a tertiary care center with ECMO capability.