Congenital pulmonary hernia secondary to absence of ribs

Congenital lung hernia is extremely rare with less than 50 reported cases.We report two cases of lung hernia, secondary to congenital absence of ribs – A 4?year?old girl without any antecedent history of chronic cough or chest trauma presenting with a left lower lobe hernia secondary to an absent left 9th rib; a 7 month?old girl with recurrent pneumonia presenting with severe respiratory distress, fever and severe malnourishment, found to have absent 6th-9th ribs on right side with associated liver and lung herniation. The older girl has been kept on observation without surgery but the infant expired within 48 hours of admission due to respiratory failure. The clinical scenario is a rarity and can be managed conservatively in most cases. Surgical treatment should be considered in symptomatic patients and in those with severe complications. Repair for cosmetic reasons is sometimes justified.
 

Keywords: Pulmonary hernia, Absent rib