A five-year statewide experience with congenital diaphragmatic hernia Katharine D. Wenstrom, MD: Carl P. Weiner, MD: and James W. Hanson, MD b Iowa City, Iowa Antenatal in utero surgery for congenital diaphragmatic hernia has been justified by reported perinatal mortality rates of 77% to 80%. Such rates may have been subject to bias of ascertainment and may include fetuses with additional severe malformations who would not be surgical candidates. We used the Iowa Birth Defects Registry to conduct a complete population survey to determine the incidence of congenital diaphragmatic hernia, the frequency of associated severe malformations, and the morbidity and mortality of infants with isolated congenital diaphragmatic hernia who were not subjected to antenatal surgery. The incidence of congenital diaphragmatic hernia was 1 in 3715. Twenty-eight percent of affected fetuses had associated severe malformations that were potentially identifiable prenatally and that would have precluded antenatal surgery. Of those with isolated congenital diaphragmatic hernia, 55% survived in spite of delivery (88.5%) and/or surgical repair (44%) in a level I or II hospital. Any decision for in utero surgery to repair congenital diaphragmatic hernia must be based on this or similarly obtained information. (AM J OBSTET GVNECOL 1991;165:838-42.)
Key words: Congenital diaphragmatic hernia, in utero surgery
As techniques of fetal diagnosis and treatment have evolved, considerable attention has focused on the possibility of antenatal surgical therapy for select malformations. One potential malformation amenable to prenatal surgical correction is congenital diaphragmatic hernia. 1,2 This surgery entails risks to both mother and child, with a reported fetal mortality rate of 75% (6/8) early in the development of this procedure and with associated maternal morbidity that includes chorioamnionitis, preterm labor and delivery, and the creation of an unstable uterine scar dictating repeat cesarean sections for future pregnancies. I, 2 This still-experimental surgery has been justified by the reportedly high mortality rate (77% to 80%) of fetuses with congenital diaphragmatic hernia who are not treated until after birth. These rates were derived from physician surveys,' the experience of single tertiary care centers,1-6 and infant death registries. 7 Such data, however, may be biased by referral patterns 3 -6 and incomplete ascertainment. 7 Before fetal surgery for congenital diaphragmatic hernia can be considered an appropriate
From the Department of Obstetrics and Gynecologya and the Department of PediatTics,' University of Iowa Hospitals and Clinics_ This research was supported by a cooperative agreement between the Centers for Disease Control and the University of Iowa (No, U50CCU70J035-05). Presented at the Eleventh Annual Meeting of the Society of Perinatal Obstetricians, San Francisco, California, January 28-Febr-uary 2, 1991, Reprint requests: Katharine D, Wenstrom, MD, Department of Obstetrics and Gynecology, University of 10wa Hospitals and Clinics, 10wa City, 1A 52242_ 6/6/30900
838
option, the natural history of this malformation must be determined as accurately as possible. We used the Iowa Birth Defects Registry to conduct a complete population survey from which we obtained information concerning the incidence and associated morbidity and mortality of congenital diaphragmatic hernia among fetuses delivered with this defect.
Material and methods All fetuses with congenital diaphragmatic hernia who were born to Iowa residents between 1983 and 1988 were identified through the Iowa Birth Defects Registry. The staff of this registry, which is a cooperative effort involving the University of Iowa, the Iowa Department of Public Health, and the Centers for Disease Control, United State Public Health Service, confidentially investigates all infants with congenital malformations who are born to Iowa residents at >20 weeks' gestation or who weigh >500 gm. Fetuses or infants with birth defects recorded on birth certificates and / or death certificates are identified, and all hospital records (prenatal, labor and delivery, neonatal, and subsequent hospitalizations) are thoroughly reviewed on site by specially trained field representatives. Only fetuses or infants with confirmed diagnoses of congenital diaphragmatic hernia (Bochdalek or Morgagni hernias or congenital absence of the diaphragm) were included in this study. Information obtained from each case study included maternal history, family history, prenatal course, labor and delivery events, level of the delivery hospital, level of the hospital that provided postnatal care, diagnostic
Congenital diaphragmatic hernia 839
Volume 165 Number 4, Part 1
Table I. Outcome of infants with congenital diaphragmatic hernia and additional severe malformations Malformation
No.
Outcome
Trisomy 18 Trisomy 13 Neural tube defect Hypoplastic left heart Hypoplastic right heart Multiple malformations Cornelia de Lange syndrome Pentalogy of Cantrell Aortic malformation Cystic hygroma, hydrops Potter syndrome Microcephaly
3 1 3
Stillbirths at 32.5 and 33 wk; death on 1st day of life Stillbirth at 36.5 wk Stillbirths at 28.5, 32, and 35.5 wk Stillbirths at 36 and 40 wk Stillbirth at 31.5 wk Stillbirth at 35.5 wk Death on 1st day of life Stillbirth at 28 wk Death on 1st day of life Stillbirth at 22.5 wk Stillbirth at 39 wk Alive at 28 mo (seizure disorder, severe developmental delay)
2
I I 2 1 1 1 1 1
tests, surgical therapy, postoperative course, autopsy findings, and follow-up. Information obtained from the registry was supplemented with direct chart review. Statistical analysis was by X2 and Mann-Whitney U test where appropriate. A p value of
Key words: Congenital diaphragmatic hernia, in utero surgery
As techniques of fetal diagnosis and treatment have evolved, considerable attention has focused on the possibility of antenatal surgical therapy for select malformations. One potential malformation amenable to prenatal surgical correction is congenital diaphragmatic hernia. 1,2 This surgery entails risks to both mother and child, with a reported fetal mortality rate of 75% (6/8) early in the development of this procedure and with associated maternal morbidity that includes chorioamnionitis, preterm labor and delivery, and the creation of an unstable uterine scar dictating repeat cesarean sections for future pregnancies. I, 2 This still-experimental surgery has been justified by the reportedly high mortality rate (77% to 80%) of fetuses with congenital diaphragmatic hernia who are not treated until after birth. These rates were derived from physician surveys,' the experience of single tertiary care centers,1-6 and infant death registries. 7 Such data, however, may be biased by referral patterns 3 -6 and incomplete ascertainment. 7 Before fetal surgery for congenital diaphragmatic hernia can be considered an appropriate
From the Department of Obstetrics and Gynecologya and the Department of PediatTics,' University of Iowa Hospitals and Clinics_ This research was supported by a cooperative agreement between the Centers for Disease Control and the University of Iowa (No, U50CCU70J035-05). Presented at the Eleventh Annual Meeting of the Society of Perinatal Obstetricians, San Francisco, California, January 28-Febr-uary 2, 1991, Reprint requests: Katharine D, Wenstrom, MD, Department of Obstetrics and Gynecology, University of 10wa Hospitals and Clinics, 10wa City, 1A 52242_ 6/6/30900
838
option, the natural history of this malformation must be determined as accurately as possible. We used the Iowa Birth Defects Registry to conduct a complete population survey from which we obtained information concerning the incidence and associated morbidity and mortality of congenital diaphragmatic hernia among fetuses delivered with this defect.
Material and methods All fetuses with congenital diaphragmatic hernia who were born to Iowa residents between 1983 and 1988 were identified through the Iowa Birth Defects Registry. The staff of this registry, which is a cooperative effort involving the University of Iowa, the Iowa Department of Public Health, and the Centers for Disease Control, United State Public Health Service, confidentially investigates all infants with congenital malformations who are born to Iowa residents at >20 weeks' gestation or who weigh >500 gm. Fetuses or infants with birth defects recorded on birth certificates and / or death certificates are identified, and all hospital records (prenatal, labor and delivery, neonatal, and subsequent hospitalizations) are thoroughly reviewed on site by specially trained field representatives. Only fetuses or infants with confirmed diagnoses of congenital diaphragmatic hernia (Bochdalek or Morgagni hernias or congenital absence of the diaphragm) were included in this study. Information obtained from each case study included maternal history, family history, prenatal course, labor and delivery events, level of the delivery hospital, level of the hospital that provided postnatal care, diagnostic
Congenital diaphragmatic hernia 839
Volume 165 Number 4, Part 1
Table I. Outcome of infants with congenital diaphragmatic hernia and additional severe malformations Malformation
No.
Outcome
Trisomy 18 Trisomy 13 Neural tube defect Hypoplastic left heart Hypoplastic right heart Multiple malformations Cornelia de Lange syndrome Pentalogy of Cantrell Aortic malformation Cystic hygroma, hydrops Potter syndrome Microcephaly
3 1 3
Stillbirths at 32.5 and 33 wk; death on 1st day of life Stillbirth at 36.5 wk Stillbirths at 28.5, 32, and 35.5 wk Stillbirths at 36 and 40 wk Stillbirth at 31.5 wk Stillbirth at 35.5 wk Death on 1st day of life Stillbirth at 28 wk Death on 1st day of life Stillbirth at 22.5 wk Stillbirth at 39 wk Alive at 28 mo (seizure disorder, severe developmental delay)
2
I I 2 1 1 1 1 1
tests, surgical therapy, postoperative course, autopsy findings, and follow-up. Information obtained from the registry was supplemented with direct chart review. Statistical analysis was by X2 and Mann-Whitney U test where appropriate. A p value of
