Congenital Diaphragmatic Hernia

A hernia is an abnormal opening in any part of the abdomen or chest cavity. A diaphragmatic hernia is a congenital birth disorder where the foetal diaphragm does not form completely. This disorder occurs in 1 in every 5000 live births.

Cause of Congenital Diaphragmatic Hernia (CDH)

This birth defect occurs in the second trimester of pregnancy due to incomplete formation of the diaphragm. The organs in the stomach cavity crowd the chest cavity and impair lung development. The result is incomplete development of the lungs and respiratory distress after birth. The cause of CDH is unclear though it is associated with other birth defects like:

  • Smith-Lemli-Opitz syndrome
  • DiGeorge syndrome
  • Mutation in 13th,15th,18th and 21st Chromosome
  •  Fryns syndrome
  • Pallister-Killian

CDH has been classified into two types:

Bochdalek hernia: The organs in the abdominal cavity move to the chest cavity through a gap in the back of the diaphragm. CDH on the left side is more common and occurs in 80 per cent of all Bochdalek Hernia cases.


Morgagni hernia: This CDH is rare type is rare and occurs in 2 per cent of all CDH cases. The organs in the abdominal cavity move to the chest through a hole in the front of the diaphragm

Symptoms of CDH:

Excess volume of amniotic fluid in the expectant mother is an indication of CDH. This condition can be diagnosed with an ultrasound examination. If the paediatrician suspects CDH in a new born, he may confirm the diagnosis with a chest x ray.

  1. Severe respiratory disorders due to incomplete formation of the lungs
  2. Rapid breathing
  3. Fast heart beat rate
  4. Bluish tinge to the complexion due to impaired circulation
  5. Chest movements may be irregular
  6. The CDH may appear on either side of the chest cavity and breath sound may be difficult to detect on the affected side
  7. Upon palpation, the abdomen appears very soft and hollow.

Treatment:

The survival of a new born with CDH depends on the extent of lung development. Immediately upon delivery, the affected new born is put on respiratory support.


With advances in foetal surgery, surgeons often operate on a 24-28 week old foetus to place the organs in the correct position and to close up the diaphragm.


Even upon delivery, the child may be put on a heart-lung machine and operated upon in emergency surgery. A small gap can be closed surgically. A large gap may require the insertion of transplanted muscle flaps or a prosthetic flap.


Post -surgery steroid based medication to improve immunity levels and to help the lungs develop are given to help the child reach normal growth parameters.


Children diagnosed with CDH and renal or cardiac diseases have a low survival rate.
With advances in medical science, the survival rate of infants with CDH is more than 80 per cent.


This disorder requires long term follow up to prevent complications.



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