Diaphragmatic Hernia CDH

Overview

Congenital diaphragmatic hernia (CDH) is a condition in which a hole in the diaphragm allows the passage of the abdominal organs into the chest, which limits the development of lungs.

Congenital diaphragmatic hernia (CDH) is a birth defect that affects about one in every 2,500 live births. Characterized by development at very early stages of pregnancy, an orifice in the diaphragm, a muscle involved in breathing and separates the chest abdomen. It is more common to find the hole on the left side.

As a result, the intestines and other abdominal organs can travel to the chest and compressing the developing lungs. This prevents the lungs grow and develop normally, and can decrease blood flow to the lungs and cause pulmonary hypertension (high blood pressure in the pulmonary circulation).

If left untreated, could endanger life.

Diagnosis

This exploration of ultrafast MRI evidence for certain liver herniation into the chest.  As with other birth defects, most congenital diaphragmatic hernia (CDH) are usually detected on routine ultrasound studies between 16 and 18 weeks of gestation. Families who are referred to the Centre for Foetal Diagnosis and Treatment at The Children’s Hospital undergoing a comprehensive evaluation of a day, which includes:

High Definition Ultrasound Level II

To evaluate and determine congenital lung-head circumference ratio (CPC) of the foetus, a measure that can help predict the severity of pulmonary problems associated with the CDH.

Ultrafast foetal MRI

An advanced technique imaging that is performed controls the position of the abdominal organs, especially the liver, and measures its protrusion into the chest.

Studies of foetal chromosomes

To detect the presence of genetic disorders.

Foetal echocardiography

A test that uses sound waves to create an image of the heart to detect congenital heart disease, a disorder that can occur simultaneously with the CDH.

The severity of congenital diaphragmatic hernia (CDH) is determined largely by the position of the liver and the quotient CPC. Best results are usually obtained in cases where the liver remains in the lower abdomen and the CPC is higher ratio. The overall survival rate is 70 percent.

Monitoring and treatment

After assessment, families gather with our multidisciplinary team. The purpose of this meeting is to discuss the results of the studies confirm the diagnosis, explain treatment options and possible outcomes, and answer any questions the family may have. The team develops a treatment plan specifically designed according to the specific needs of the mother and foetus.

In most cases, the pregnancy will be carefully monitored throughout its development, with more frequent checks in the third quarter. Surgical repair of CDH is done after birth.

In severe cases in which most of the liver has been displaced toward the chest and cause significant lung growth restriction, foetal therapy may be suggested.

Delivery

Scheduled deliveries of babies with CDH are made in the Special Deliveries Unit allowing immediate attention at the highest level for the new-born and obstetric experts to the mother, all of which, in a paediatric hospital first level. Special Deliveries Unit is the first delivery room in the world that has been exclusively designed for pregnancies complicated by birth defects, and is a key element of the capacity that our team to provide comprehensive care.

Most babies with CDH born vaginally. The neonatal team is present during delivery, because the baby will need immediate stabilization and, in many cases, support ventilation (respiratory) specialist. The neonatology team has extensive experience in ventilation techniques for infants with CDH.

Hospital stay

After stabilization, the baby was transferred to the Neonatal Intensive Care Unit / Pediatric, where the team continues to evaluate and careful monitoring of the newborn state. Babies with very compromised lungs or fragile may require ECMO (extracorporeal membrane oxygenation), a technique of temporary deviation used to oxygenate the blood and allow the lungs to rest for several days or even weeks.

Surgical repair of the CDH depends on the development of the baby in the days after birth, and can be made from the three days of life. The surgery is performed in the Neonatal Intensive Care Unit / Pediatric, and thus avoiding the risks of transfer to the operating room. An incision is made just below the rib cage of the baby, the bodies have been shifted to the Chest rehomed in the abdomen and close the hole in the diaphragm. The space created in the chest allows the lungs continue to grow, in children, there may be a compensatory lung growth until 8 or 9 years old.

For infants with larger defects, or when the diaphragm has not been developed directly on one side, the hole is closed with a patch of soft tissue. As the child grows, the status of the patch will be checked regularly by doctors to ensure it has not deteriorated.

The residence time in the Neonatal Intensive Care Unit / Pediatric may vary considerably. Some children need mechanical ventilation for a short time, is undergoing surgical repair and can go home in a relatively short period. Others may require ventilation or extracorporeal membrane oxygenation for prolonged periods, and may need to remain hospitalized.

Care Monitoring

All patients with CDH require continuous follow-up care. Depending on the severity of the initial defect and the type of repair is made, monitoring can be done by the primary care paediatrician or child may require more specialized care. Many children with CDH have developed pulmonary hypoplasia, a condition in which the lungs are small and underdeveloped, and can affect not only breathing but also cardiac function, the ability to feed and general development. The Children’s Hospital of Philadelphia has created a Pulmonary Hypoplasia Program provides unique and interdisciplinary care, specifically focused on this disorder so delicate. Through the program, it keeps track of children throughout childhood, and even for several years with the school.