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Case of the Week 26 2015

*3 day-old infant with respiratory distress

What is the most likely diagnosis?

1-Congenital cystic adenomatoid malformation
2-Pulmonary sequestration
3-Hybrid lesion
4-Diaphragmatic hernia

Answer

Answer: Congenital diaphragmatic hernia

Case Discussion:
Babygram determined herniation of multiple air-filled loops of bowel into right thoracic cavity through diaphragm with displacement of right lung and mediastinum towards left. Barium study showed barium-filled loops of bowel into right thoracic cavity. Gasless abdomen is also noted. Surgery was performed and diagnosis of congenital diaphragmatic hernia was confirmed.
Diaphragmatic hernias1 are divided into two main categories: congenital diaphragmatic defects and acquired diaphragmatic defects.
Congenital (rare)
Congenital diaphragmatic hernias occur due to embryologic defect in the diaphragm. There are two main kinds of them.
• Bochdalek hernia: most common, located posteriorly and typically presents earlier (in infancy)
• Morgagni hernia: less common, smaller, located anteriorly and presents later
Acquired (common)
•traumatic diaphragmatic rupture
•hiatus hernia
•iatrogenic

Chest radiograph
 indistinct diaphragm with opacification of the hemithorax
 scaphoid abdomen
 deviation of lines 2 (endotracheal tube, nasogastic tube, umbilical arterial-venous catheter)

References:
1. Mullins ME, Stein J, Saini SS et-al. Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am J Roentgenol. 2001;177 (2): 363-6.
2. Sakurai M, Donnelly LF, Klosterman LA et-al. Congenital diaphragmatic hernia in neonates: variations in umbilical catheter and enteric tube position. Radiology. 2000;216 (1): 112-6.