Heather Borders, MD
over 6 years ago
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Right-sided Congenital Diaphragmatic Hernia
Joseph Junewick, MD FACR
|Diagnostic Category: Developmental or Congenital
|Created: over 7 years ago
|Updated: over 7 years ago
3 month old with minor difficulty breathing.
Right-sided Congenital Diaphragmatic Hernia (delayed Morgagni type)
CR – Right-sided mediastinal mass with diminished hepatic shadow in right upper quadrant.
CT – Axial, sagittal and coronal images demonstrate herniation of the liver through an anterior and medial diaphragmatic defect.
Congenital diaphragmatic hernia (CDH) is sporadic and usually occurs by the 2nd trimester. Herniation of abdominal contents into the chest through a diaphragmatic defect results in pulmonary hypoplasia, lung immaturity, left heart hypoplasia and pulmonary hypertension. The diaphragmatic defect may be posterolateral (Bochdalek), anteromedial (Morgagni) or central tendon (septum transversum). Bochdalek defects are most common, accounting for 80% of hernias. Most hernias are left sided.
Delayed CDH is commonly seen on the right side (the liver temporarily blocks the extension of abdominal viscera into the chest). Delayed hernia has also been reported with group B beta-hemolytic streptococcal pneumonia.
Anomalies are associated with CDH in up to half of patients and include ventriculoseptal defect, vascular ring, aortic coarctation, genitourinary abnormalities, neural tube defects, esophageal atresia, omphalocele, cleft palate and chromosomal abnormalities (trisomy 12, 18 and 21). Early complications of CDH survivors include chylous effusion and bronchopulmonary dysplasia; late complications include chest wall deformities (pectus and scoliosis), bowel dysmotility, and gastroesophageal reflux.
Slovis TL and Bulas DI. Congenital and acquired (most causing respiratory distress) of the neonatal lung and thorax. Caffey’s Pediatric Diagnostic Imaging, 11th Ed. 2008.