Congenital diaphragmatic hernia (CDH) occurs when abdominal organs protrude into the chest cavity due to a defect in the diaphragm. It has an incidence of about 1 in 2500 births. CDH causes pulmonary hypoplasia and hypertension due to compression of the lungs. Initial treatment focuses on stabilizing the infant medically to improve oxygenation and reverse pulmonary hypertension before repairing the diaphragmatic defect via surgery. Prenatal diagnosis allows for fetal stabilization attempts. Prognosis depends on the degree of pulmonary hypoplasia and hypertension present.

1. Congenital Diaphragmatic Hernia
Eyayalem M.

2. Introduction of CDH
• Incidence: about 1/2500
• Definition
– Failure of the pleural and peritoneal canal to
close at about GA 8 wk
– Herniation of the abdominal viscera into the
thoracic cavity and pulmonary hypoplasia
from compression by the viscera on the
developing lungs
• Cause: Unknown

3. Pathophysiology
• Diaphragmatic defect
• Failure of closure of pleuro-peritoneal canal
• Most common area is a postero-lateral defect
( Bochdalek )
• Left side more common

4. Pathophysiology
• Simple pathophysiology after herniation:
– Abdominal viscera fill the chest cavity
– Abdomen small & poorly developed
– Compressionpulmonary hypoplasia
– Dysfunction of the surfactant system
– Increased muscularization of the intra-acinar
pulmonary arteriespulmonary hypertension
– In very severe cases, left ventricular hypoplasia is
observed.

6. Pulmonary hypoplasia

7. Pathology of CDH
Following delivery
• Bowels fill with air
• Compression of ipsilateral lung
• Mediastinal shift
• Compression of contralateral lung(mechanical
compression of lung)

10. Lung development in CDH
• No. of bronchial branches – greatly reduced
• Alveolar development severely affected
• Increased muscle mass in the conducting
airways
• Seen in contra lateral lung too

11. Pulmonary vasculature in CDH
• Reduction in the total no. of branches
• Both in ipsilateral and contra lateral lungs
Significant adventitial and medial wall
thickening
• Increased susceptibility to
– hypoxia, acidosis, hypothermia, stress

14. Persistent fetal circulation
pulmonary
artery
resistance
pulmonary
artery
pressures
pulmonary
vascular flow
Right to left shunting
Hypoxia &
Progressive
desaturation
Respiratory
failure

15. Diagnosis
• CXR diagnostic
• Absence of diaphragm
• Scaphoid abdomen
• Bowel loops in chest
• Mediastinal shift

16. Prenatal Diagnosis
• Prenatal ultrasound: 59% detection rate, at
average GA 24.2 wk
• Predictors of outcome by prenatal ultrasound:
Polyhydramnios, Intrathoracic stomach or
liver, Abd circumference, lung-to-head
ratio(LHR), lung/transverse thorax ratio
• New predictors: PA diameter and LV mass (by
fetal echocardiography), MRI lung volumetry
• 1/3 P’t have associated malformations: CV, GU,
GI, CNS, chromosome anomalies

17. Chest X - Ray

18. Problems
Hypoxia
Respiratory distress
Metabolic
acidosis
Hypercarbia

20. Treatment
• Initial management Approach should be first medical
stabilization of the pt for improving respiratory and
general status and then proceeding for surgery after
stabilization
• GOALS
1. Reverse persistent pulm HTN to decrease rt to lt shunting.
2. Improvement in ventilation & oxygenation
3. Correction of acidosis
4. Correction of systemic hypotension
» Time taken to stabilize varies from 24-48 hrs to 7-10 days,
and upto 3 wks in some neonates
» Repair of hernia in emergency when the contents are
incarcerated

21. Perinatal Treatment
Fetal stabilization:
• Naso- or Oro-gastric tube: placed as soon as
possibledecompression
• ICU monitor: cross-match, CBC, electrolyte,
blood gas, CXR, head ultrasound(for
IVHECMO), echocardiogram(for previously
undetected cardiac anomalies, pulmonary
hypertension)

22. Perinatal Treatment
• Ventilation:
– Permissive hypercapnea: gentle ventilation
– Avoidance of hyperventilation and
barotrauma
– HFOV: controversy
• Surfactant administration: controversy
• Surgical correction