CONGENITAL DIAPHRAGMATIC HERNIA

Kathy Wilson, RN BSN BA RNA CDIS 03/2019

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Presentation on the Following Aspects of CDH

Definition of Congenital Diaphragmatic Hernia [CDH]Clinical Presentation of CDHSurgical Repair of CDHLifelong Sequelae of CDH

CDI Considerations for CDH

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What Is A Congenital Diaphragmatic Hernia?(CDH)

A congenital diaphragmatic hernia (CDH) occurs when the diaphragm muscle — the muscle that separates the chest from the abdomen — fails to close during prenatal development, and the contents from the abdomen (stomach, intestines and/or liver) migrate into the chest through this hole.

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KW1KW2

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KW1 This picture is from CHOP website. The actual herniated diaphragm in represented in the Left picutre. The normal diaphragm is represented on he Right.Kathy Wilson, 1/30/2019

KW2 Kathy Wilson, 1/30/2019

TYPES of CDH

CDH can occur on the left side, right side or, very rarely, on both sides and vary in severity

A Bochdalek hernia is a hole in the back of the diaphragm. Ninety percent of Congenital Diaphragmatic Hernias are this type

AMorgagni hernia involves a hole in the front of the diaphragm

Very large or incomplete diaphragmatic hernias often requireECMO immediately after delivery 5

Fetal Surgical Repair of CDH [For severe cases of CDH]

Fetoscopic endoluminal tracheal occlusion (FETO) is a fetal surgery procedure that may improve outcomes in babies with the most severe cases of CDH.  It is performed while infant is still in utero.

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Postnatal Surgical Repair for Small

CDH Defects

An incision is made just below the baby’s rib cage, the organs in the chest are guided back down into the abdomen and the hole in the diaphragm is sewn closed. The space created in the chest allows the lungs to continue to grow.  [see following slides]

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Postnatal Surgical Repair for Large CDH Defects

For babies with large defects or completely lacking a diaphragm, the hole is closed with a GORE‐TEX® patch or muscle flap.

Sometimes the abdominal wall cannot be closed during surgery. In these cases, temporary placement of a silo, mesh or Vacuum Assisted Closure® (VAC) device may be recommended.

As the child grows, the condition of the patch will be regularly monitored by doctors to ensure that it remains intact. [see next slide] 10

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When the abdominal organs are in the chest, there is limited room for the lungs to grow. 

The lungs are prevented from developing normally, resulting in pulmonary hypoplasia (underdeveloped lungs).

This pulmonary hypoplasia causes reduced blood flow to the lungs resulting inpulmonary hypertension (high blood pressure in the pulmonary circulation)

In a very short time, the pulmonary hypertension leads tocardiac dysfunction

HOW CDH AFFECTS OTHER ORGAN DEVELOPMENT

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PULMONARY HYPOPLASIA

Pulmonary hypoplasia is a condition characterized by small, underdeveloped lungs.

These underdeveloped lungs have a profound effect on the development of the rest of the body, such as:

• Breathing• Heart function• The ability to feed• Overall physical and mental development

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PULMONARY HYPERTENSION

Pulmonary hypertension is high blood pressure in the arteries of the lungs(the pulmonary arteries)

• Over time, this pressure causes the pulmonary arteries to narrow, making the right side of the heart work harder as it forces blood through the narrowed arteries.

• Chronic pulmonary hypertension can lead to right ventricular dilatationand diastolic dysfunction

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CARDIAC DYSFUNCTIONCardiac dysfunction is an important consequence of pulmonary hypertension in congenital 

diaphragmatic hernia and a determinant of disease severity

Increased afterload leads to right ventricular dilatation and diastolic dysfunction. 

Septal displacement and dysfunction impair left ventricular function, which may also cause fetal cardiac hypoplasia

Biventricular failure contributes to systemic hypotension and hypoperfusion16

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CDI Guidelines for CDH

For each post‐repair admission, review for documentation of these    diagnoses which are lifelong sequelae of CDH

Pulmonary Hypoplasia/Hypoplastic Lung

Pulmonary Hypertension

Cardiac Dysfunction

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QUERY OPPORTUNITIES FOR SEQUALAE OF CDH

Review for clinical indicators of, or documentation related to any pulmonary issues that would indicate lung dysfunction and query for pulmonary hypoplasia

Review for clinical indicators of, or documentation related to hypertension and query for clarification of pulmonary hypertension

Review for clinical indicators of, or documentation related to any type of heart defect and query for the specific cardiac dysfunction

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“CODING CONUNDRUM”

Should Congenital Diaphragmatic Hernias be considered a lifelong diagnosis and therefore captured by the Q790 code on every subsequent admission?

This Photo by Unknown Author is licensed under CC BY-ND

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