Longitudinal Follow-Up With Radiologic Screening for Recurrence and Secondary Hiatal Hernia in Neonates With Open Repair of Congenital Diaphragmatic Hernia-A Large Prospective, Observational Cohort Study at One Referral Center.

Katrin B. Zahn,Thomas Schaible,Lucas Wessel,Neysan Rafat,Christel Weiss,Meike Weis

doi:10.3389/fped.2021.796478

Abstract

Objective: After neonatal repair of congenital diaphragmatic hernia (CDH) recurrence is the most severe surgical complication and reported in up to 50% after patch implantation. Previous studies are difficult to compare due to differences in surgical techniques and retrospective study design and lack of standardized follow-up or radiologic imaging. The aim was to reliably detect complication rates by radiologic screening during longitudinal follow-up after neonatal open repair of CDH and to determine possible risk factors.Methods: At our referral center with standardized treatment algorithm and follow-up program, consecutive neonates were screened for recurrence by radiologic imaging at defined intervals during a 12-year period.Results: 326 neonates with open CDH repair completed follow-up of a minimum of 2 years. 68 patients (21%) received a primary repair, 251 (77%) a broad cone-shaped patch, and 7 a flat patch (2%). Recurrence occurred in 3 patients (0.7%) until discharge and diaphragmatic complications in 28 (8.6%) thereafter. Overall, 38 recurrences and/or secondary hiatal hernias were diagnosed (9% after primary repair, 12.7% after cone-shaped patch; p = 0.53). Diaphragmatic complications were significantly associated with initial defect size (r = 0.26). In multivariate analysis left-sided CDH, an abdominal wall patch and age below 4 years were identified as independent risk factors. Accordingly, relative risks (RRs) were significantly increased [left-sided CDH: 8.5 (p = 0.03); abdominal wall patch: 3.2 (p < 0.001); age ≤4 years: 6.5 (p < 0.002)]. 97% of patients with diaphragmatic complications showed no or nonspecific symptoms and 45% occurred beyond 1 year of age.Conclusions: The long-term complication rate after CDH repair highly depends on surgical technique: a comparatively low recurrence rate seems to be achievable in large defects by implantation of a broad cone-shaped, non-absorbable patch. Longitudinal follow-up with regular radiologic imaging until adolescence is essential to reliably detecting recurrence to prevent acute incarceration and chronic gastrointestinal morbidity with their impact on prognosis. Based on our findings and literature review, a risk-stratified approach to diaphragmatic complications is proposed.

Highlights

Congenital diaphragmatic hernia (CDH) is a rare malformation, and surgical repair is still an intervention with a remarkable complication rate
We examined long-term rates of diaphragmatic complications after neonatal open CDH repair and aimed at identifying possible risk factors
In 508 neonates with CDH born in the study period, survival to discharge was 81% (n = 410): 37 patients (7%) died without surgery due to prematurity, fatal syndrome or associated malformations, severe lung hypoplasia, or contraindication to extracorporeal membrane oxygenation (ECMO) therapy; 29 of the ECMO patients (14%) died without CDH repair; and 26 (13%) died after CDH repair

Summary

Introduction

Congenital diaphragmatic hernia (CDH) is a rare malformation, and surgical repair is still an intervention with a remarkable complication rate. It has been shown that these are more likely to require postnatal extracorporeal membrane oxygenation (ECMO) therapy and diaphragmatic reconstruction with a patch and that they are at risk of early mortality and long-term morbidity [4]. These fetuses should be transferred to a high-volume center for optimized treatment and follow-up [5]. The importance of longitudinal follow-up for CDH survivors due to their numerous comorbidities and complex needs has been emphasized and a schedule for a risk-stratified multi-specialty follow-up has been proposed [16]

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Conclusion