J Pediatr Surg

. 2020 Aug 15;S0022-3468(20)30588-1. doi: 10.1016/j.jpedsurg.2020.08.010. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/32951888/

Predicting lethal pulmonary hypoplasia in congenital diaphragmatic hernia (CDH): Institutional experience combined with CDH registry outcomes

Aimee G Kim ¹, Rodrigo Mon ², Monita Karmakar ¹, Pamela A Lally ³, Ronald B Hirschl ¹, George B Mychaliska ¹, Erin E Perrone ⁴, Congenital Diaphragmatic Hernia Study GroupAffiliations expand

• PMID: 32951888
• DOI: 10.1016/j.jpedsurg.2020.08.010

Abstract

Background: The Severe Pulmonary Hypoplasia and Evaluation for Resuscitative Efforts (SPHERE) protocol was developed to attempt to identify CDH patients with likely lethal pulmonary hypoplasia. We present our experience with this protocol and utilize the CDH Registry to critically assess the protocol.

Methods: SPHERE patients identified based on prenatal imaging (10/2009-1/2018) were offered ECMO if meeting postnatal physiologic criteria, while others received comfort measures. Within the CDH Registry, patients with suspected severe CDH were identified and separated into “passed” (lowest pCO2 ≤100) versus “failed” (lowest pCO2 >100) groups.

Results: Of 23 SPHERE patients, 57% (13/23) passed criteria for ECMO and survival was 46% (6/13) in that cohort. Of 4912 patients in the CDH Registry, 265 met criteria. There was no difference in survival rates between those that “passed” (122/227; 54%) versus “failed” (18/38; 47%). However, the latter had longer ECMO runs and more required ventilator/ECMO support at 30 days. Amongst survivors, the “failed” group had longer hospital stays and more frequently required tube feeds at discharge.

Conclusions: The SPHERE protocol did not predict mortality in the CDH Registry. However, our data suggest resource utilization is significant when unable to reach pCO2 ≤100 despite resuscitation. Morbidity remains high in this group.

Level of evidence: Level III ANNOTATION OF CHANGES: Institutional Review Board Approval at University of Michigan (HUM00031524 and HUM00044010) TYPE OF STUDY: Retrospective Review.

Keywords: Congenital diaphragmatic hernia; Prenatal diagnosis; Pulmonary hypertension.

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