J Pediatr Surg

. 2022 Oct 14;S0022-3468(22)00634-0.

 doi: 10.1016/j.jpedsurg.2022.09.042. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/36333128/

Morbidity in children after fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: Results from a multidisciplinary clinic

Shelby R Sferra ¹, Melanie K Nies ², Jena L Miller ³, Alejandro V Garcia ¹, Erica I Hodgman ¹, Annalise B Penikis ¹, Abigail J Engwall-Gill ¹, Vera Joanna Burton ⁴, Jessica L Rice ⁵, Peter J Mogayzel ⁵, Ahmet A Baschat ³, Shaun M Kunisaki ⁶

Affiliations expand

• PMID: 36333128
• DOI: 10.1016/j.jpedsurg.2022.09.042

Abstract

Background: Although fetoscopic endoluminal tracheal occlusion (FETO) was recently shown to improve survival in a multicenter, randomized trial of severe congenital diaphragmatic hernia (CDH), morbidity outcomes remain essentially unknown. The purpose of this study was to assess long-term outcomes in children with severe CDH who underwent FETO.

Methods: We conducted a prospective study of severe CDH patients undergoing FETO at an experienced North American center from 2015-2021 (NCT02710968). This group was compared to a cohort of non-FETO CDH patients with severe disease as defined by liver herniation, large defect size, and/or ECMO use. Clinical data were collected through a multidisciplinary CDH clinic. Statistics were performed with t-tests and Chi-squared analyses (p≤0.05).

Results: There were 18 FETO and 17 non-FETO patients. ECMO utilization was 56% in the FETO cohort. Despite significantly lower median observed/expected lung-to-head ratio (O/E LHR) in the FETO group, [FETO: 23% (IQR:18-25) vs. non-FETO: 36% (IQR: 28-41), p<0.001], there were comparable survival rates at discharge (FETO: 78% vs. non-FETO: 59%, p = 0.23) and at 5-years (FETO: 67% vs. non-FETO: 59%, p = 0.53) between the two cohorts. At a median follow up of 5.8 years, metrics of pulmonary hypertension, pulmonary morbidity, and gastroesophageal reflux disease improved among patients after FETO. However, most FETO patients remained on bronchodilators/inhaled corticosteroids (58%) and were feeding tube dependent (67%).

Conclusions: These North American data show that prenatal tracheal occlusion, in conjunction with a long-term multidisciplinary CDH clinic, is associated with acceptable long-term survival and morbidity in children after FETO.

Level of evidence: Level III.

Keywords: Congenital diaphragmatic hernia; Fetal therapy; Fetoscopic endoluminal tracheal occlusion.

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