Long-Term Outcomes of Congenital Diaphragmatic Hernia: Report of a Multicenter Study in Japan

Abstract

Background: Treatment modalities for neonates with congenital diaphragmatic hernia (CDH) have greatly improved in recent years, with a concomitant increase in survival. However, long-term outcomes restrict the identification of optimal care pathways for CDH survivors in adolescence and adulthood. Therefore, we evaluated the long-term outcomes within the Japanese CDH Study Group (JCDHSG). Methods: Participants were born with CDH between 2006 and 2018 according to the JCDHSG. Participants were enrolled in the database at 1.5, 3, 6, and 12 years old. Follow-up items included long-term complications, operations for long-term complication, and home medical care. Results: A total of 747 patients were included in this study, with 626 survivors (83.8%) and 121 non-survivors (16.2%). At 1.5, 3, 6, and 12 years old, 45.4%, 36.5%, 34.8%, and 43.6% developed complications, and 20.1%, 14.7%, 11.5%, and 5.1% of participants required home care, respectively. Recurrence, pneumonia, pneumothorax, gastroesophageal reflux disease, and intestinal obstruction decreased with age, and thoracic deformity increased with age. Conclusions: As CDH survival rates improve, there is a need for continued research and fine-tuning of long-term care to optimize appropriate surveillance and long-term follow-up.

Association of Fetal Lung Development Disorders with Adult Diseases: A Comprehensive Review

“…FEY 1 -forced expiratory volume in one second, FEF 25-75 -forced expiratory flow between 25 and 75 percent of vital capacity, V max50 -maximal expiratory flow at 50 percent of vital capacity, FRC-functional residual capacity, TLC-total lung capacity, RV/TLC-the ratio of residual volume to total lung capacity, D LCO -diffusing capacity for carbon monoxide [107]. (F) Association between CDH in infancy and its long-term impact on children's health (the study was carried out in Japan) [127].…”

“…CDH survivors require ongoing medical and surgical care, including long-term medication, respiratory support, and multiple surgeries; these needs persist from infancy through adulthood [151,152]. After hospital discharge, CDH survivors face issues like respiratory problems, reflux, growth failure, developmental delays, hearing loss, hernia recurrence, and orthopedic issues (Figure 4F) [127,[153][154][155]. These complications stem from underlying lung issues and surgeries.…”

Radiomics-Assisted Computed Tomography-Based Analysis to Evaluate Lung Morphology Characteristics after Congenital Diaphragmatic Hernia

“…Some of the most commonly encountered forms of morbidity in survivors of congenital diaphragmatic hernia (CDH) encompass bilateral lung hypoplasia, ventilator-induced lung injury and hyperinflation, diaphragmatic dysfunctions, as well as anatomical alterations of the spine and thoracic wall [9]. The persistence of pulmonary hypertension is noted, with an incidence ranging between 3% and 12%, and it has been demonstrated to resolve within weeks up to months following surgery, with some reports suggesting persistence into adolescence [10]. The vast majority of survivors of CDH tend to develop gastroesophageal reflux disease (GERD) due to the intricate interplay of mechanical and developmental factors, such as a short abdominal esophagus, developmental impairment of sphincter function, or an excess of abdominal pressure across the hiatus in the postoperative period [11].…”

“…Complications such as pneumonia, asthma, or pneumothorax arise in a substantial percentage of cases, ranging from 10% to 50% [10]. Notably, chronic pneumonia, manifesting recurrently throughout the year, stands out as the most prevalent concern, affecting up to 8.8% of survivors who may necessitate hospitalization by the time they reach 1.5 years [10]. Some patients were also shown to develop pneumothorax [10].…”

“…Notably, chronic pneumonia, manifesting recurrently throughout the year, stands out as the most prevalent concern, affecting up to 8.8% of survivors who may necessitate hospitalization by the time they reach 1.5 years [10]. Some patients were also shown to develop pneumothorax [10].…”