Congenital Diaphragmatic Hernia

Sunita Goel
Associated Conditions Seen With Congenital Diaphragmatic Hernia
Malrotation- 90%
CNS defects (spina bifida, hydrocephalus, cerebral dysgenesis)- 30%
Cardiovascular defects (ASD, VSD, coarctation of aorta, TOF)- 20%
Chromosomal disorders (Trisomy 18 and 21)- 5 - 15%

Diaphragm is composed of several components:
- Septum transversum: forms the central tendon
- Pleuroperitoneal membrane: forms the dorsolateral portion
- Intercostal muscle group: forms the posterior lateral portion
- Esophageal mesentry: forms the dorsal crura
In 7th week of gestation, peritoneal folds extend medially to fuse with central tendon. If the fusion is incomplete, the defect is well established by 10 to 12 weeks of gestation. Size of the defect varies from 2 cm to complete agenesis of the diaphragm

Development of the Respiratory System
Normal Development:
- 4 weeks: Ventral out pouching of foregut
- 16 weeks: Successive branching of bronchi increase in alveolar air spaces
- 24 weeks to 8 years of age: development of alveolus
In CDH, there is interruption of bronchial airway branching at 10 weeks gestation. The ipsilateral lung development is retarded at 12th - 14th generation. Contralateral lung development is retarded at 16th - 18th generation. Development of Type 1 alveolar epithelium is arrested.

Factors Involved in Abnormal Lung Development (Table 1)

Factors Involved in Abnormal Lung Development

Seen in 1 out of 5000 newborns. Posterolateral (Foramen of Bochdalek) hernia is more common with incidence of almost 80% (L > R). Anteromedial (Foramen of Morgagni), Esophageal hernia and Bilateral hernias are less common. Overall mortality (with modern treatment) is 50% and morbidity remains high.

Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia 12/31/2001
Congenital Diaphragmatic Hernia - Presentation >>
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