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Congenital Diaphragmatic

Hernias (CDHs)

What are they?

CDHs are a cause of respiratory distress or failure after an infant's birth.  The under development of the foetal diaphragm, during the eighth week of gestation, is the cause of this debilitating condition.  Because the liver is located on the right side of the abdomen, eighty percent of CDHs occur on the left side.  The undeveloped hole in the abdomen allows the abdominal organs to enter the chest cavity.  These organ occupy the space in which the lungs are supposed to grow, and prevents the lungs from fully developing.  The herniated diaphragm often affects both lungs and this insufficient lung development is referred to as pulmonary hypoplasia.  Sixty percent of CDH foetuses do not survive after birth, as their bodies cannot intake the necessary oxygen.

Above are healthy infant lungs.
        On the right are CHD lungs, with the
        abdominal organs inside the lung cavity


An ultrasound examination allows for the detection of a CDH.  The location of the foetus' liver is the initial indicator, as well as the size of the lungs.  The foetal lung size is an estimate generated by the doctor from the ultrasound images.  The liver of CDH foetuses rises into the chest cavity and therefore is the key indicator in diagnosing CDH.  The liver is the first intestinal organ to rise into the chest cavity in CDH babies, inferring that liver observation can allow for prompt diagnosis.

     On the right and left are initial
ultrasound pictures diagnosing
                                                                  a CDH.

Tracheal occlusion using a fetoscope is the method of intervention used for the restoration and accelerated development of the foetus' lungs.  The surgery is done by making a one-inch incision into the womb, allowing for the insertion of a fetoscope.  The fetoscope acts as the doctor's eyes for the operation.  A small opening is cut through the neck of the foetus and a stitch is used to seal the trachea, preventing fluid from escaping or entering the lungs.  Five years of research were devoted to discovering a successful method of accelerating lung development.  Found was a fluid produced in the lungs of the foetus which was circulated out of the chest cavity, through the trachea, and into the womb for dilution by the amniotic fluid.  This fluid was determined to be responsible for lung development.  A tracheal occlusion prevents this fluid from circulating out of the lungs, and its high concentration within the chest cavity accelerates lung development and allows for a foetus to have functioning lungs at birth.  The pressure created by the fluid development guides the abdominal organs back into the abdomen and also promotes the completed development of the diaphragm. 
A C-section delivery is required for CDH babies.  The stitch sealing the trachea is removed promptly after birth and a breathing tube is inserted down the child's trachea to ensure that oxygen is quickly circulated through the body.  After the infant is stable, the tube may be removed and the child can breathe on his or her own.  Further surgeries may be required to ensure that the diaphragm is properly sealed and that the lungs are properly functioning.

This picture shows the fetoscope inside the womb.


Additional Information
Recent research has shown that CDH may be an autosomal recessive disorder.  It occurs approximately once in every three thousand foetuses, and there is a two percent chance that a second child suffering from the condition will be produce from the same parents.  Only twenty percent of CDH foetuses are female are the condition is often associated with Fryns syndrome.  Currently, the Fetal Treatment Center in California is the only institution to offer foetal surgeries for congenital diaphragmatic hernias.

The Anatomy and Development of the Foetus

Medical Conditions Affecting the Foetus from initial diagnosis to treatment:
Congenital Diaphragmatic Hernia (CDH)
Congenital Cystic Adenomatoid Malformation of the Lung (CCAM)
Sacrococcygeal Teratoma (SCT)
Urinary Tract Obstruction

Related Technologies:
Radiofrequency Ablation (RFA)

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