Congenital Diaphragmatic Hernia

What does Congenital Diaphragmatic Hernia mean?

Congenital diaphragmatic hernia (CDH) is a birth condition affecting prenatal lung development. The diaphragm is a muscle mass that separates the chest from the abdomen and which fails to form during fetal development in CDH. This allows the contents from the stomach or the abdominal organs to flow into the chest region, crowding the heart and lungs and affecting their average growth.

What Are The Different Types of CDHs?

Different types of CDHs reported over the years are:

  • Bochdalek hernia- Opening in diaphragm from side or back. 80-90% of fetuses have this type of hernia.
  • Morgnani hernia- Frontal opening in the diaphragm and is rarely observed. 2% of fetuses have this type with no symptoms at birth.
  • Cases with the complete absence of a diaphragm or a thin membrane in its place have also been reported.

What are the signs of CDH?

Congenital diaphragmatic hernia majorly affects the lungs. The underdeveloped lungs lead to breathing difficulties which are sometimes apparent from birth and, in some cases, appear later in life. In addition, in some cases, there is sharp abdominal pain from the protrusion of abdominal organs into the chest cavity.

What are the causes of CDH?

The diaphragm is usually formed by ten weeks of pregnancy. In cases of CDH, the diagram formation is not complete or is disrupted.

  • Chromosomal abnormalities which affect several genes or a single gene simultaneously lead to the disorder of many body systems in which CDH occurs.
  • 25% of individuals affected by CDH have more than one abnormality in various body systems. These developmental abnormalities have some common underlying disruption that is not clear.
  • Around 50-60% CDHs are isolated, meaning that the individual has no other significant malformations
  • Environmental factors, in some cases, have been reported to cause developmental issues in fetuses.  

How is CDH Diagnosed?

Diagnosis of CDH usually happens at one of the regular ultrasounds suggesting the presence of contents of the abdomen in fetal chest activity. Before confirming CDH, your doctor will perform different tests like

  • Ultrafast fetal MRI
  • High-resolution fetal ultrasound
  • Fetal echocardiogram
  • Genetic testing
  • Lung size measurement

The severity prediction in CDH is dependent on the placement of the liver. In cases where the liver is pressed against the chest, it causes pulmonary hypoplasia. It is diagnosed by measuring the size of the lungs during ultrasound or MRI. Also, this condition is majorly observed in individuals showing defects on the diaphragm's left side, giving rise to the stomach, intestines, and liver herniating in the chest region. In some cases, fetuses have defects on the diaphragm's right side.

How to Manage CDH During Pregnancy?

Once accurately diagnosed, treatment and potential outcomes will be discussed with you and your family by a pediatric surgeon or a fetal medicine specialist. Typically, a follow-up will be taken every three weeks until close to your term, when your doctor will see you more frequently. During these follow-ups, lung measurements with ultrasound will be monitored. Also, the levels of amniotic fluid around the fetus are measured. After completion of 34 weeks, fetal health will be monitored weekly.

How are Babies Affected by CDH Delivered?

Before birth, the placenta performs all the primary organ functions in the fetus. However, after birth, the baby depends upon the proper functioning of organs for survival. Thus, the health of the newly born fetus can change drastically unless specialized care has been given. Once delivered, the fetus will be immediately placed with a breathing tube connected to a ventilator.

A breathing machine will help the infant to breathe correctly. Also, IV lines will be set to provide fluids and medications. During all times, blood levels will be monitored unless the infant is stabilized in N/IICU.

What Are the Treatment Options for CDH?

The treatment plan in babies with CDH is to heal the lung damage from lung compression during pregnancy. Different procedures help decrease the amount of lung damage, such as:

  • Fetoscopic tracheal occlusion (FETO): The lungs of the fetus produce fluid that leaves through the baby's mouth. Blockage of this outflow leads to swelling and infection in the lungs. FETO relieves this blockage by barricading the fetal windpipe with a small balloon.
  • Fetal monitoring and delivery planning: Specialized care with a comprehensive treatment plan prevents the fetus' deterioration and adverse effects of CDH.

Request an appointment at Apollo Cradle, Amritsar - Abadi Court Road. Call 1860-500-1066 to book an appointment.

1. Is CDH inherited through the family?

The exact cause of CDH is unknown. However, there is a substantial genetic contribution. CDH can reoccur within families as a part of chromosomal disorders.

2. Can CDH correct itself?

No, the only treatment available for CDH is surgery of the diaphragm. However, it is essential for the child to be stabilized first and has overall good health before conducting any procedure.

3. How long do CDH babies required to stay in the hospital?

Typically, a two to three-month stay is required depending upon the infant's condition. However, babies with ECMO or persistent pulmonary issues might need a more extended stay.

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