Congenital Diaphragmatic Hernia

Introduction

Welcome to the APSA Quality and Safety committee Congenital Diaphragmatic Hernia (CDH)Toolkit. This page is intended to help anyone who is interested in implementing quality improvement (QI) initiatives for the management of CDH.

Available toolkit projects are listed below. These approaches utilize varying degrees of evidence-based approaches and some represent a hybrid blend of clinical practice guidelines and QI initiatives rather than pure QI alone. These approaches have not been approved by APSA and may not represent processes subjected to external peer review.

General Guidelines

Children’s Memorial Hermann Hospital / University of Texas Health Sciences Center at Houston

The Neonatology and Pediatric Surgery departments developed an evidence-based guideline focused on the initial stabilization and management of CDH.

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Children’s Hospital of Los Angeles

The initial medical management of CDH and pulmonary hypertension were protocoled.

Challenges and Solutions: The major challenge is in communication among large group of providers. The neonatologists selected a small group of staff based on experience and interest to manage patients before ECMO and during ECMO in order to improve communication and standardize management of pulmonary hypertension.

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Children’s Hospital of Philadelphia

Newborn / Infant Intensive Care Unit optimal care guidelines for CDH

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ECLS and High Risk Algorithms

Hassenfeld Children’s Hospital at NYU Langone

The Fetal Diagnosis and Treatment program at Hassenfeld Children’s developed a high-level workflow algorithm to coordinate the multiple teams and decision-making that occurs during an expected high-risk CDH delivery with ECMO immediately available.

Challenges and Solutions: EXIT-to-ECMO for high-risk CDH has largely been abandoned due to lack of significant benefit over an ECMO-on-standby approach. Institutions may accomplish a C-section with ECMO-standby approach in a variety of different ways, driven by physical-plant, clinical service-line, and staffing considerations. After several cases where this approach was deployed, there was a broad desire to create a codified approach to this multi-disciplinary, high-risk plan effectively involving separate maternal and infant treatment teams co-localized to a single location. The provided workflow outlines our institution’s approach to providing a rapid assessment in the OR of the newborn, and making determination as to whether they should be cannulated or transported to the NICU for further care. Following implementation, team satisfaction and coordination across services was significantly streamlined.

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Surgical Strategies

Children’s Hospital Los Angeles

Surgeons that offer CDH repair during ECMO at our institution had different thresholds and time ranges before an operation was offered. We decided to divide our group into early repair versus delayed repair cohorts and apply differential protocols for these groups. Half of our group defined early repair as < 48hr on ECMO and late repair >48hr on ECMO. CDH patients were therefore classified into 4 groups: Group 1) Low risk no ECMO, 2A) ECMO weaned then repaired, 2B) ECMO unweanable delayed repair up to 2 weeks. 3) ECMO early repair within 24-48hrs. Surgeons were assigned to either delayed repair (group 2) or Early repair (group 3).

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