Overall Outcome of the Prenatally Diagnosed Left Isolated Congenital Diaphragmatic Hernia ― a Report from the Japanese CDH Study Group

PURPOSE:Owing to advances in prenatal diagnoses, the overall outcome of congenital diaphragmatic hernia (CDH) can be estimated; however, practical clinical information among CDH survivors for counselling families were lacking. The present study analyzed the overall outcome of prenatally diagnosed left isolated CDH, using a Japanese CDH Study Group (JCDHSG) database. METHODS:Prenatally diagnosed patients with left isolated CDH (n = 511), registered in our JCDHSG database from 2006 to 2021 were retrospectively reviewed. Prenatal diagnoses were divided into three categories depending on the observed-to-expected lung-to-head circumference ratio and liver herniation (extremely severe + severe [ES + S], n = 94; moderate [Mo], n = 156, and Mild [Mi], n = 261). The primary outcomes were the survival and intact discharge rates. Intact discharge was defined as CDH survivors without home-based medical care at discharge. The secondary outcomes included the operation timing, ventilation duration, and hospitalization duration. RESULTS:The overall survival rate at discharge was 86.9 % (444/511). Among survivors at discharge, the intact discharge rate was 80.6 % (358/444). The survival and intact discharge rates varied significantly by disease severity. Among CDH survivors, the median operation timing was consistent across severity grades, however, the median duration of ventilation (26, 20, and 10 days for ES + S, Mo, and Mi, respectively, p < 0.001) and length of hospitalization duration (83, 65, and 39 days for ES + S, Mo, and Mi, respectively, p < 0.001) differed significantly. CONCLUSIONS:Prenatal diagnoses, categorized by disease severity correlated well with the stratified clinical outcomes. These data support accurate prenatal family counselling with prognostic prediction in prenatally diagnosed left isolated CDH patients. TYPE OF STUDY:Original Research, Clinical Research. LEVELS OF EVIDENCE:LEVEL Ⅲ Retrospective Comparative Study.