CONGENITAL DIAPHRAGMATIC hernia Kathy Wilson, RN BSN BA RNA CDIS 03/2019

Presentation on the Following Aspects of CDH Definition of Congenital Diaphragmatic Hernia [CDH] Clinical Presentation of CDH Surgical Repair of CDH Lifelong Sequelae of CDH CDI Considerations for CDH

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. https://www.chop.edu/conditions-diseases/congenital-diaphragmatic-hernia-cdh

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 A Morgagni hernia involves a hole in the front of the diaphragm Very large or incomplete diaphragmatic hernias often require ECMO immediately after delivery This Photo by Unknown Author is licensed under CC BY-SA

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

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] This Photo by Unknown Author is licensed under CC BY-SA

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]

How CDH Affects Other Organ Development 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 in pulmonary hypertension (high blood pressure in the pulmonary circulation) In a very short time, the pulmonary hypertension leads to cardiac dysfunction

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

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 dilatation and diastolic dysfunction

CARDIAC DYSFUNCTION Cardiac 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 hypoperfusion Semin Pediatr Surg. 2017 Jun;26(3):154-158.doi:10.1053/j.sempedsurg.2017.04.001. Epub 2017 Apr 25

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

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

“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