The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review.
OBJECTIVE
To review the recent Japanese experience with prenatally diagnosed congenital diaphragmatic hernia (CDH) based on a multi-institutional survey.


METHODS
A multicenter, retrospective cohort study was conducted on 117 patients born between 2002 and 2007 with isolated prenatally diagnosed CDH. All patients were managed by maternal transport, planned delivery, immediate resuscitation and gentle ventilation. The primary outcome measurements were the 90-day survival and intact discharge. The examined prenatal factors included gestational age (GA) at diagnosis, lung-to-head ratio (LHR), lung-to-thorax transverse area ratio (L/T) and liver position. Physical growth and motor/speech development were evaluated at 1.5 and 3 years of age. Data were expressed as the median (range).


RESULTS
The mean GA at diagnosis was 29 (17-40) weeks. The LHR and L/T were 1.56 (0.37-4.23) and 0.11 (0.04-0.25), respectively. There were 48 patients with liver up. The mean GA at birth was 38 (28-42) weeks. The 90-day survival rate and intact discharge rate were 79 and 63%, respectively. Twelve patients had major morbidity at discharge, and 71% of these patients had physical growth or developmental retardation at 3 years of age.


CONCLUSIONS
This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe CDH.
Department of Pediatric Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan. okuyama@hyo-med.ac.jp
Hiroomi
H
Okuyama
Yoshihiro
Y
Kitano
Mari
M
Saito
Noriaki
N
Usui
Nobuyuki
N
Morikawa
Kouji
K
Masumoto
Hajime
H
Takayasu
Tomoo
T
Nakamura
Hiroshi
H
Ishikawa
Motoyoshi
M
Kawataki
Satoshi
S
Hayashi
Noboru
N
Inamura
Keisuke
K
Nose
Haruhiko
H
Sago
D016009:Chi-Square Distribution; D002675:Child, Preschool; D005260:Female; D006548:Hernia, Diaphragmatic / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D008297:Male; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D015996:Survival Rate