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

Neonatology. 2017;112(4):365-371. doi: 10.1159/000479145. Epub 2017 Sep 2.

Abstract

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.

Keywords: Congenital diaphragmatic hernia; Failure to thrive; Fetoscopic endoluminal tracheal occlusion; Gastro-oesophageal reflux; Long-term outcome; Pulmonary morbidity.

MeSH terms

  • Age Factors
  • Belgium
  • Cardiovascular Diseases / etiology
  • Child Development
  • Female
  • Fetoscopy* / adverse effects
  • Gastrointestinal Diseases / etiology
  • Hernias, Diaphragmatic, Congenital / complications
  • Hernias, Diaphragmatic, Congenital / diagnosis
  • Hernias, Diaphragmatic, Congenital / surgery*
  • Herniorrhaphy / adverse effects
  • Herniorrhaphy / methods*
  • Humans
  • Infant
  • Male
  • Nutritional Status
  • Pregnancy
  • Prospective Studies
  • Respiratory Tract Diseases / etiology
  • Risk Factors
  • Surveys and Questionnaires
  • Time Factors
  • Treatment Outcome