C. M. Miller1, K. P. Lally1, M. T. Harting1 1McGovern Medical School at UTHealth and Children’s Memorial Hermann Hospital,Pediatric Surgery,Houston, TX, USA
Introduction: As advances in care re-define survivorship in congenital diaphragmatic hernia (CDH), particularly among infants with severe CDH, the onus is shifting to long-term management. CDH survivors are frequently challenged by pulmonary (PULM), gastrointestinal (GI), neurologic (NEURO), and orthopedic (ORTHO) morbidities. Our objective was to characterize long-term morbidity in the modern era of care and the association with neonatal risk factors as defined by CDH study group (SG) Stage (A-D).
Methods: A single center, retrospective cohort study of survivors born 2011-2017 was performed. Patients with CDH clinic visits between 1-2 and 4-5 years of age were included. Patient demographics, prenatal, and neonatal characteristics were reviewed. The primary outcomes were morbidities at two and five year (±12 months) follow-up. Morbidities included PULM, GI, NEURO, and ORTHO.
Results: A total of 37 patients were included in the study cohort. There were 27 patients at 2 years and 10 patients at 5 years of age. Overall morbidity was 88.9% and 90% at 2 and 5 years, respectively. At two years of age, 11(41%) had PULM, 13(48%) GI, 9(33%) NEURO, and/or 9(33%) ORTHO morbidities. Of the 11 patients with PULM morbidity at two years, the majority were taking a pulmonary medication (n=8, 72.7%). GI morbidity included gastroesophageal reflux (n=4, 30.8%), supplemental feeds (n=3, 23.7), constipation (n=3, 23.1%), and GI medication (n=5, 38.5%). NEURO morbidity was dominated by neurodevelopmental delay (n=8, 88.9%). ORTHO morbidity included pectus excavatum (n=4, 44.4%), rib abnormalities (n=4, 44.4%), and spine abnormalities (n=1, 11.1%). At five years of age, 8(80%) had PULM, 2(20%) NEURO, 3(30%) GI, and/or 4(40%) ORTHO morbidities. A large portion of pulmonary morbidity at 5 years included taking a pulmonary disease related medication (n=6, 75%), asthma (n=3, 37.5%), and sleep disordered breathing (n=3, 37.5%). Only GI medication occurred more than once at 5 years (n=2, 66.6%). NEURO morbidity was confined to developmental delay (n=2, 100%). ORTHO morbidity included pectus excavatum (n=2, 50%), rib abnormalities (n=1, 25%), and spine abnormalities (n=2, 25%). Morbidity for both 2 and 5 years relative to CDHSG Stage is shown (Table). Analysis by χ2 indicated no difference in observed incidence of morbidity by CDHSG Stage.
Conclusion: Survivors with CDH continue to face significant morbidity at two and five years. At two years the spectrum of morbidity resembles that of discharge, with a predilection toward gastrointestinal morbidity. Alternatively, at five years, the primary source of morbidity is pulmonary. These preliminary data will inform multi-center collaboration in long-term CDH data collection.
