Perinatal Outcomes in Fetuses Prenatally Diagnosed with Congenital Diaphragmatic Hernia and Concomitant Lung Lesions: A 10-Year Review.

AIM: To describe perinatal outcomes of fetuses with a prenatal diagnosis of a concomitant lung lesion in the setting of congenital diaphragmatic hernia (CDH) and to compare outcomes with an isolated CDH control group without a lung lesion, matched by ultrasound-based prognostic markers including presence of liver herniation and lung measurements.

MATERIAL AND METHODS: This was a retrospective case-control study, wherein all pregnancies diagnosed with CDH and concomitant lung lesions were identified between July 1, 2008, and December 31, 2018. For each case, 2 controls with isolated CDH from the same study period were selected after matching for the presence of liver herniation into the thoracic cavity and ultrasound-based lung measurements either observed over expected lung-to-head ratio (LHR) or absolute LHR with their corresponding gestational age. The outcomes analyzed in the 2 groups included survival to hospital discharge, neonatal intensive care unit (NICU) length of stay (LOS), extracorporeal membrane oxygenation (ECMO) requirement and need for supplemental oxygen (O2) at day 30 of life.

RESULTS: A total of 21 pregnancies were identified with CDH and a concomitant lung lesion in the study period. All the lung lesions were stratified into a "low-risk category" with a congenital cystic adenomatoid malformation volume ratio of less than 1.0 at the time of presentation. None of these fetuses developed hydrops or required in utero intervention. Overall survival in the group was 80.7% (17/21) and rate of ECMO was 38.1%. Causes of mortality included pulmonary insufficiency, sepsis, renal failure, and bowel infarction. Upon comparison between the cases and controls, the 2 groups were similar with respect to pregnancy demographics. There were no fetal demises in either group. Outcomes including survival rate, NICU LOS, ECMO requirements and need for supplemental O2 at day 30 of life, were comparable among the 2 groups.

CONCLUSIONS: In our descriptive series, the presence of a concomitant, low-risk lung lesion in the setting of fetal CDH did not have a significant impact on the natural course of the disease, nor was it associated with a worse prognosis.