Congenital diaphragmatic hernia (CDH) is a potentially fatal condition found in neonates where embryological defects in the diaphragm negatively impact fetal maturation and growth. The defect allows contents below the diaphragm to potentially migrate into the thoracic cavity during development, which could lead to secondary complication including pulmonary hypertension and left ventricular hypoplasia. CDH tends to have a high neonate mortality rate in congruence with the severity of the condition. Several risk factors for CDH include accompanying chromosomal abnormalities and the anatomical positions of organs in the fetus. Diagnosis is typically found with an ultrasound (US) in utero. There have been several studies in order to better understand the pathology of the disease and new techniques to try and alleviate the cases prenatally, however the risks involved with these procedures may outweigh the benefits. The standard practice for neonates that qualify for postnatal treatment is the use of extracorporeal membrane oxygenation (ECMO) postnatally, to facilitate oxygenated blood to the fetus via a bio-mechanical device. Recent treatment techniques that have revolutionized care for CDH include a delayed surgical intervention in order to reduce the risk of developing a pulmonary ailment such as pulmonary hypertension and/or lung hypoplasia. Interventions with inhaled nitric oxide have also been shown to relegate a similar outcome to those with ECMO intervention. Despite the advancements in knowledge, treatment, and technology, the mortality rate for CDH still hovers around 50% on average, yet that percentage can increase or decrease depending on the severity of the condition and any genetic abnormalities associated with it. Overall, while there have been great strides in treatment and understanding of CDH, additional research is necessary in order to provide the utmost care for future generations of CDH patients.
Identifer | oai:union.ndltd.org:bu.edu/oai:open.bu.edu:2144/23760 |
Date | 12 July 2017 |
Creators | Bovino, Scott Anthony |
Source Sets | Boston University |
Language | en_US |
Detected Language | English |
Type | Thesis/Dissertation |
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