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Diaphragmatic hernia, congenital

DA: 184




Developmental anomaly information

Definition: The diaphragm initially develops as a septum between the heart and liver, progresses posterolaterally, and closes at the left Bochdalek foramen at approximately 8-10 weeks' gestation.Recent studies show that the diaphragmatic defect occurs in the initial stages of diaphragm development, rather than in the later stages. The herniation of viscera in CDH usually occurs during the pseudoglandular stage of lung development. Lung compression results in pulmonary hypoplasia that is most severe on the ipsilateral side, although both lungs may be abnormal. CDH is characterized by a variable degree of pulmonary hypoplasia associated with a decrease in cross-sectional area of the pulmonary vasculature and dysfunction of the surfactant system. In very severe cases, left ventricular hypoplasia is observed. Pulmonary capillary blood flow is decreased because of the small cross-sectional area of the pulmonary vascular bed, and flow may be further decreased by abnormal pulmonary. The 3 basic types of CDH include the posterolateral Bochdalek hernia (occurring at approximately 6 weeks' gestation), the anterior Morgagni hernia, and the hiatus hernia. The left-sided Bochdalek hernia occurs in approximately 85% of cases. Left-sided hernias allow herniation of both the small and large bowel and intraabdominal solid organs into the thoracic cavity. In right-sided hernias (13% of cases), only the liver and a portion of the large bowel tend to herniate. Bilateral hernias are uncommon and usually fatal.


Synonyms:

  • CDH


Developmental anomaly parent: (empty)

Topology: Human body > Thorax > Mediastinum

System: Human body > Respiratory system

Developmental anomaly category: Malformation

Case records MDB56, MDB100, MDB122, MDB211, MDB277
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Observed in MDB cases
Observed in combinaisons (MDB)
Observed in malformative diseases (MDB)
  • () in case MDB
Associated genes in profile
Associated genes in malformative diseases
Associated chromosomal diseases
Associated signaling pathways
Associated developmental anomalies

Here is the list of developmental anomalies associated with this developmental anomaly, based on the cases' information :



Last modified: 2009-01-02 16:08:35 by farideh

Created: 2008-05-12 02:00:00 by phsaw



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