Congenital pericardial agenesis, though exceedingly rare, should be considered in the differential diagnosis of exertional chest pain to prevent misdiagnosis.
Congenital absence of pericardium is an exceedingly rare condition with a prevalence of 0.002%-0.004%. Due to its rarity and absence of association with any specific clinical examination finding, the diagnosis may be challenging. Due to the absence of symptoms and clinical awareness, pericardial agenesis is commonly misdiagnosed. It is important to consider this as a differential diagnosis of exertional chest pains. We report the case of a 9-year-old boy who attended our institution for non-specific but frequent symptoms and was diagnosed with congenital complete absence of the pericardium. The rarity of the condition can hinder timely diagnosis, as a physician or radiologist may never encounter a single case in their lifetime.
Rayamajhi et al. (Fri,) studied this question.