Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion.
OBJECTIVE
Fetuses with congenital diaphragmatic hernia (CDH) who have a "poor prognosis" with postnatal treatment now can be identified on the basis of liver herniation, early diagnosis (before 25 weeks' gestation) and a low lung-to-head ratio (LHR). Because complete in utero repair proved unsuccessful for this group, the strategy of temporary tracheal occlusion was developed to gradually enlarge the hypoplastic fetal lung. The purpose of this study is to compare the outcome of patients in the poor-prognosis group treated by one of three methods: (1) standard postnatal care, (2) fetal tracheal occlusion via open hysterotomy, and (3) the recently developed video-fetoscopic (Fetendo) technique of tracheal occlusion without hysterotomy.


METHODS
In the past 3 years, 34 of 86 fetuses with an isolated left CDH met criteria for the poor-prognosis group. Thirteen families chose postnatal treatment at an extracorporeal membrane oxygenation (ECMO) center, 13 underwent open fetal tracheal occlusion, and eight underwent fetoscopic tracheal occlusion.


RESULTS
The survival rate was 38% in the group treated by standard postnatal therapy, 15% in the open tracheal occlusion group, and 75% in the Fetendo group. There were less postoperative pulmonary complications noted in mothers who underwent the Fetendo procedure versus the open tracheal occlusion. All but one Fetendo clip patient had a striking physiological response demonstrated by sonographic enlargement of the small left lung that was documented postnatally by plain radiographs and its subjective appearance during repair of the CDH. In contrast, only 5 of the 13 open tracheal occlusion patients demonstrated lung growth.


CONCLUSIONS
Fetuses with a left CDH who have liver herniation and a low LHR are at high risk of neonatal demise and appear to benefit from temporary tracheal occlusion when performed fetoscopically, but not when performed by open fetal surgery.
The Fetal Treatment Center and the Department of Surgery, University of California, San Francisco 94143-0570, USA.
M R
MR
Harrison
G B
GB
Mychaliska
C T
CT
Albanese
R W
RW
Jennings
J A
JA
Farrell
S
S
Hawgood
P
P
Sandberg
A H
AH
Levine
E
E
Lobo
R A
RA
Filly
D000328:Adult; D016009:Chi-Square Distribution; D004724:Endoscopy; D005260:Female; D005315:Fetal Diseases / Q000401:mortality / Q000601:surgery*; D005325:Fetal Organ Maturity; D005332:Fetoscopy; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000401:mortality / Q000601:surgery*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D008107:Liver Diseases / Q000150:complications; D008168:Lung / Q000196:embryology*; D011247:Pregnancy; D018566:Pregnancy, High-Risk; D011379:Prognosis; D019736:Prostheses and Implants; D015996:Survival Rate; D014132:Trachea