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Diaphragmatic hernia

Fetal pregnancy complications


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Description

A congenital diaphragmatic hernia is a birth defect where a hole in the diaphragm allows the organs of the abdomen (stomach, small intestine, colon, spleen, liver) to move up into the thorax. This prevents optimal lung development and results in pulmonary hypoplasia, which can increase the severity of the defect.

Diaphragmatic hernias affect about 1 birth in every 3,000.

It occurs:

  • On the left side in 85% of cases
  • The right side in 10% of cases
  • On both sides (bilateral) in 5% of cases.

Causes

  • In a little more than half of the cases, the diaphragmatic hernia is isolated.
  • In nearly 25% of cases, it is associated with other defects.
  • In 30% of cases, a chromosome abnormality is found.
  • In 5% of cases, it is syndromic.

Tests and procedures

In more than 80% of cases, diaphragmatic hernias are diagnosed during routine pregnancy ultrasounds. When a diaphragmatic hernia is suspected, a consultation in a specialized centre such as CHU Sainte-Justine is recommended. Other diagnostic tests such as amniocentesis, fetal echocardiography, and an MRI may be useful in determining the prognosis of the abnormality.

The risk of pulmonary hypoplasia will determine prognosis.


Treatment and follow up care

Pregnancy monitoring and the delivery should be done in a specialized tertiary centre like CHU Sainte-Justine.

The care of the mother and the unborn baby is managed by a multidisciplinary team that includes an obstetrician specialized in fetal-maternal medicine, a geneticist, a pediatric surgeon, a neonatologist pediatrician, a radiologist, a nurse, and a respiratory therapist. Several other professionals such as a psychologist or a lactation consultant may also be part of the team depending on individual needs.

When the baby is born, the neonatal team takes immediate steps to stabilize respiratory function. At birth the newborn may, among other things, have difficulty breathing, cyanosis (blue skin), or rapid breathing.

Once breathing is stable, the baby can undergo surgery to properly reposition the organs in the abdomen and to close the hole in the diaphragm. The duration of hospitalization depends on pulmonary involvement and recovery of respiratory function.


Resources and useful links

Fiche par

Chantal Larcher, inf. clinicienne et Dre Lucie Morin

About this page
Updated on 12/19/2017
Created on 12/18/2017
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