Clinical Outcome for Congenital Diaphragmatic Hernia at the Age of 1 Year in the Era of Fetal Intervention.

BACKGROUND

Congenital diaphragmatic hernia (CDH) is an abnormal development of the diaphragm leading to high neonatal mortality and morbidity. Beyond the neonatal period, prospective data on overall long-term outcome in CDH survivors is scarce. In particular, for those treated with fetoscopic endoluminal tracheal occlusion (FETO), a promising new technique to increase survival chances for severe cases, the outcomes are even less documented.

AIM

To prospectively document the clinical outcome of CDH at 1 year including FETO-treated infants in relation to ante- and postnatal variables.

METHODS

A single-centre prospective clinical follow-up at 1 year of age included a standardised respiratory questionnaire.

RESULTS

Thirty-four CDH survivors were included, 10 of which were FETO-treated infants. At 1 year, the median weight z score was -1.50 (interquartile range [IQR]: -2.45 to -0.88) and 10/34 were "failure to thrive" (FTT) cases. Gastro-intestinal (GI) problems at 1 year were mainly related to gastro-oesophageal reflux (16/34) with a high need for tube feeding in the first year (9/34). Tachypnoea was present in 10/29 (34%) and 8/34 (24%) were on chronic inhaled medication. The median total respiratory symptom score was 20 (IQR: 13-32) and correlated with the number of neonatal ventilation days (p = 0.048). Hospital re-admission occurred in 19/34 infants (56%), and was for respiratory problems in almost half of them (8/19).

CONCLUSION

In this CDH cohort, morbidity at 1 year was determined more by GI problems and FTT than respiratory morbidity. The respiratory questionnaire may, however, have underestimated the respiratory morbidity since respondents had a 1-in-4 chance to have persistent tachypnoea and be on chronic inhalation therapy.