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 quality improvement (QI) 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. These approaches have not been approved by APSA.

General Guidelines

At 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.

Resources:

• _{UT Houston Protocol for CDH Management (From Birth to Surgery)}
• UT Houston Methodology for the Development of the CDH Management Guidelines

Stakeholders: MFM, neonatology, pediatric surgery, cardiology, pharmacy.

Published data:

• Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update [1].
• Management of Congenital Diaphragmatic Hernia: A systematic review from the APSA Outcomes and Evidence Based Practice Commmittee [2] .
• Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline [3]
• Video: Protocols for the Management of CDH

• Submitted by: Matt Harding

Additional implementers:Tim Jancelewicz, MD, MA, Yigit Guner, MDyguner@uci.edu

The initial medical management of CDH and pulmonary hypertension were protocoled at Children’s Hospital of Los Angeles.

Challenges and Solutions: The major challenge was in communication among a 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.

Resources:

• CHLA Congenital Diaphragmatic Hernia Consensus Guidelines

Stakeholders: neonatologists, pediatric surgeons, anesthesiologists, ECMO providers, nurses.

Submitted by: Juan Carlos Pelayo

Additional implementers: Neonatologists: Rachel Chapman, Phillipe Fredrick; Pediatric Surgeons: David Bliss, Chris Gayer

Children’s Hospital of Philadelphia

Congenital Diaphragmatic Hernia Optimal Care Guidelines: Early Management, Pulmonary Hypertension, ECMO, Surgical Repair, Discharge and Follow up.

Resources:

• CHOP CDH Optimal Care Guidelines

Submitted by: Holly Hedrick

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 a 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 were significantly streamlined.

Resources:

• NYU Langone High Risk CDH Delivery Plan

Stakeholders: MFM, Neonatology/NICU Nursing, Pediatric Surgery, ECMO/Perfusion, OR Staffing, Pharmacy

Published data:

• Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia? [4]
• Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors?[5]

Submitted by: Jason C. Fisher, MD

Surgical Strategies

Children’s Hospital Los Angeles developed a QI project for standardization in the management of CDH given that the surgeons offering CDH repair during ECMO had different thresholds and time ranges before an operation was offered. They decided to divide their group into early versus delayed repair cohorts and apply differential protocols for these groups.

CDH patients were therefore classified into 4 groups:

Group 1: Low risk, no ECMO

Group 2a: ECMO weaned, then repaired

Group 2b: ECMO unweanable, delayed repair up to 2 weeks

Group 3: ECMO early repair within 24-48hrs. Surgeons were assigned to either delayed repair (Group 2) or early repair (Group 3).

Resources:

• CHLA CDH Surgical Management: Description of QI Project
• CHLA CDH Surgical Management Protocol

Stakeholders: neonatologists, pediatric surgeons, anesthesiologists, ECMO providers, nurses.

Submitted by: Juan Carlos Pelayo, MD

Additional implementers: Neonatologists: Rachel Chapman, Phillipe Fredrick; Pediatric Surgeons: David Bliss, Chris Gayer

References