Assessment of quality of life outcomes using the pediatric quality of life inventory survey in prenatally diagnosed congenital diaphragmatic hernia patients

Introduction Congenital diaphragmatic hernia patients can have significant postnatal morbidity. To date, there has been no quality of life (QOL) study in a prenatally diagnosed CDH patient population, assessing prenatal disease severity and QOL. The purpose of this pilot study was to assess the QOL in a single institution fetal center CDH experience. Methods The study was a retrospective cohort study of CDH patients diagnosed by fetal ultrasound and MRI at a fetal center between March 2002-March 2014. Parents of children ≥ 2 years were contacted by telephone to participate in the validated Pediatric Quality of Life Inventory (PedsQL™, Version 4.0). Results Of 95 CDH survivors, 68 met inclusion criteria, of which 28 (42%) completed the survey (mean age, range: 5.5, 2.3-11.7 years). Based on prenatal markers of disease severity, there were no differences in performance between those with mild or severe forms of CDH. Overall, patients had minimal QOL limitations, but those with limitations had a higher risk for oxygen dependence at 30 days of life (71%v. 29%, OR 0.16, CI, 0.031-0.82, p = 0.02). ECMO was not associated with significantly worse QOL in physical or psychosocial functioning. Cronbach's alpha reliability coefficient yielded a correlation of 0.951 for the overall survey, 0.911 for physical functioning, and 0.901 for psychosocial functioning questions. Conclusions Patients that are prenatally diagnosed with severe forms of CDH have similar QOL outcomes across all categories of physical and psychosocial functioning. This pilot study is encouraging and may allow improved counseling for expectant parents of CDH patients.