congenitaldiaphragmatichernia.pptx

1. Pathogenesis of CDH
and
anatomic findings
Chaojie Zhang

2. What is CDH ?
Failure of diaphragmatic fusion
Hernitation of abdominal contents into
thoracic cavity
Pulmonary hypoplasia

3. Types of congenital diaphragmatic hernia
Bochdalek hernia: A left-sided CDH
(present in 85~90% of babies)
Morgagni hernia: right-sided CDH
(present in 23~28% of babies)
The hiatus hernia

4. Bochdalek
hernia

5. Morgagni
hernia

6. left vs. right CDH
In right CDH, the liver is more likely to move up into the chest
where the baby’s lungs should be growing.
The timing of surgery to repair the CDH was later in babies with
right-sided defects.
Babies with right CDH are more likely to need treatment with
nitric oxide.
Right CDH babies are also more likely to need medications to keep
their lungs relaxed after discharge.
In right CDH, there is a greater likelihood of needing
supplemental oxygen at the time of discharge, and more of a
chance of needing a tracheostomy for long-term breathing
support.
There was no difference in survival, total length of
hospitalization, or need for ECMO support between
the two groups.
----Children’s Hospital of Philadelphia

7. Causes
Genetics
Harmful environmental exposure
during pregnancy
Fetal malnutrition

8. Ginekologia Polska 2017; 88, 12: 24–30

9. Ginekologia Polska 2017; 88, 12: 24–30

10. Ginekologia Polska 2017; 88, 12: 24–30

11. Pathophysiology of CDH
Mechanical
compression
PP
HN
Lung
hypoplasia

12. Mechanical
compresion
Bowels fill with air
Compression of ipsilateral lung
Mediastinal shift
Compression of contralateral lung

13. Lung development in
CDH
Bronchial branches greatly reduced
Alveolar development severely affected
Reduction in the total branches
Both in ipsilateral and contra lateral lungs

14. Pulmonary vasculature in
CDH
Significant adventitial and medial wall thickening
Increased muscle mass in the conducting airways
Seen in contra lateral lung too
Increased susceptibility to PPHN hypoxia, acidosis,
hypothermia, stress

15. Summary
Congenital diaphragmatic hernia(CDH) is a developmental defect in the diaphragm with
deficient lung development.
CDH can occur on the left or right side. Left side more common (8:1). Most common area is a
postero-lateral defect ( Bochdalek ).
The cause of CDH is unknown. CDH are often associated with chromosomal abnormalities
and structural malformations in other major organ systems.
Pulmonary hypoplasia and pulmonary vasculature can result in life-threatening persistent
pulmonary hypertension.