Congenital diaphragmatic hernia size predicts infant's morbidity at discharge

Last Updated: 2016-10-28

By Marilynn Larkin

NEW YORK (Reuters Health) - In infants with congenital diaphragmatic hernia (CDH), the size of the defect is a reliable indicator of hospital course and disease burden at discharge, researchers report.

Survival for such infants has improved, "yet substantial burden of disease remains," write Dr. Matthew Harting of the University of Texas Health Science Center at Houston and colleagues in Pediatrics, online October 27.

"Although larger CDH defect sizes increase mortality, the association between defect size and morbidity has not been reported," they add.

To evaluate the association of defect size with pulmonary, neurologic, and gastrointestinal morbidity at the time of discharge from the hospital, the team reviewed data from 2007 to 2014 from the international Congenital Diaphragmatic Hernia Study Group. A total of 3,665 patients were included in the study.

Overall survival was 70.9% during the study period, and 84% of those survivors were discharged from the hospital (16% transferred).

The average age at discharge was 38 days, ranging from 22 days for the smallest ("A") defects to 89 days for the largest ("D") defects (p<0.001). Among those discharged, 61.7% had gastrointestinal morbidities, 30.2% had pulmonary morbidities, and 20.4% neurologic morbidities. While the majority of patients had gastrointestinal morbidities regardless of defect size, the frequency of this morbidity "increased significantly" with larger sizes, according to the authors.

Subsequent analyses revealed that the size of the defect "was consistently the greatest predictor of overall morbidity, hospital length of stay, and duration of ventilation," they concluded.

Dr. Harting told Reuters Health, "I think the key point for neonatologists and pediatric surgeons is that among patients with CDH, the defect size is a strong predictor of the patient's discharge morbidity (or ongoing burden of disease)."

"Further, particularly for pediatricians and providers that manage CDH patients after discharge, we outline the frequency of the most common morbidities, which will aid parent counseling, focus follow-up evaluation, and allow early recognition/management of these morbidities," he said by email.

He added, "We hope this work elucidates the significant ongoing morbidity these patients face, enabling clinicians to better recognize and manage these challenges, which, in turn, reduce the burden for patients and families."

"Finally," Dr. Harting concluded, "these data will focus the morbidity targets and avenues of investigation for clinical scientists."

Dr. Danelle Fisher, chair of pediatrics at Providence Saint John's Health Center in Santa Monica, California, told Reuters Health the study can help "guide expectant management for how children with CDH will do beyond the hospital stay."

"These are extremely sick infants who spend weeks to months in the hospital from birth. When they come out into the community, they will need general pediatric care, which includes coordinating care with specialists," she explained by email.

"These families need to know how CDH, including the severity and/or size of the defect, will influence their child's medical conditions in the next years of his or her life," she said. "The goal is to deal with all of the ongoing medical issues and keep the child with CDH growing and thriving."

SOURCE: http://bit.ly/2eVmqzW

Pediatrics 2016.