Congenital Diaphragmatic Hernia

Your baby has a problem called "Congenital Diaphragmatic Hernia" or CDH. Congenital means "born with" and a hernia is a problem where something goes through a hole it is not supposed to. Initial theories about the pathophysiology of this condition centered on the presence of the herniated viscera within the chest and the need for its prompt removal In Congenital Diaphragmatic Hernia, abdominal organs push into the chest cavity through the defect (or herniate), compressing the developing lungs. The specific defect is an opening or whole in the diaphragm (the muscle that separates the chest cavity from the abdominal cavity). After birth the infant will have difficulty breathing if the lungs are not developed enough. This compresses the fetal lung nearest the hernia, preventing full development, and thus interferes with breathing after the infant is born.

During the last 10 years of the study, infants who were of low birth weight, had a syndrome, or were prenatally diagnosed were more likely to die than other infants with congenital diaphragmatic hernia. Pulmonary capillary blood flow is decreased because of the small cross-sectional area of the pulmonary vascular bed, and flow may be further decreased by abnormal pulmonary vasoconstriction. The intestines can also push on the other lung and keep it from growing fully and can sometimes keep the heart from growing normally. Many babies with CDH do fine after leaving the hospital, but some have problems that need to be followed. This is a picture of how things look in a baby with a left-sided diaphragmatic hernia.

Causes of Congenital Diaphragmatic Hernia

The common causes of Congenital Diaphragmatic Hernia :

• The diaphragm initially develops as a septum between the heart and liver, progresses posterolaterally, and closes at the left Bochdalek foramen at approximately 8-10 weeks' gestation.
• More than 10% of infants with CDH have an underlying syndromic diagnosis, although few gene mutations are currently recognized.
• In a Bochdalek hernia, the diaphragm may not develop properly, or the intestine may become trapped in the chest cavity as the diaphragm is forming.
• The intestines then enter the pleural cavity and cause poor lung development leading to pulmonary hypoplasia (a reduced number of alveoli per area of lung tissue).
• This defect is most commonly seen on the left side of the body but may also occur on the right side or the central portion of the diaphragm.
• Recently, chromosome deletions on chromosomes 1q, 8p, and 15q have been reported in association with CDH. Deletions of chromosomes 8p and 15q appear to be associated with heart malformations.

Symptoms of Congenital Diaphragmatic Hernia

Some common symptoms of Congenital Diaphragmatic Hernia :

• Abnormal chest development, with one side being larger than the other
• Cyanosis (blue color of the skin )
• fast breathing (tachypnea) asymmetry of the chest wall
• severe breathing difficulty
• bluish coloration of the skin due to lack of oxygen
• abdomen that appears caved in (concave)
• fast heart rate (tachycardia)
• Chest movements that do not coordinate with your baby's breathing rhythm

Treatment of Congenital Diaphragmatic Hernia

• Infants with CDH are critically ill and require meticulous attention to detail for subsequent medical care, including continuous monitoring of oxygenation, blood pressure, and perfusion.
• In the delivery room, if the infant is known or suspected to have CDH, immediately place a vented orogastric tube and connect it to continuous suction to prevent bowel distension and further lung compression.
• Dlood gases normalize with no significant changes between preductal and postductal samples,
• Echocardiogram demonstrate reduce pulmonary pressure and pulmonary peripheral resistance.
• Some infants are placed on ECMO (extracorporeal membrane oxygenation) which is a heart/lung bypass machine which gives the lungs a chance to recover and expand after surgery.
• your baby's tolerance for specific medications, procedures, or therapies
• ECMO does the job that the heart and lungs would be doing: putting oxygen in the bloodstream and pumping blood to the body.
• your baby's overall health and medical history

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