Presenter: Trenton E Burgess, MD
Trenton E Burgess, MD; Lena Perger, MD; University of New Mexico
A 9-month-old male was prenatally diagnosed with left sided congenital pulmonary airway malformation (CPAM). Outpatient workup included a CT angiogram of the chest at age 7 months which demonstrated a triangular lesion in the region of the left upper lobe. He presented for elective thoracoscopic resection of left CPAM at 9 months. Inspection of the left thorax upon entry revealed complete absence of the parietal pericardium, with the heart freely exposed to the pleural cavity. An extralobar pulmonary sequestration was diagnosed and resected. The procedure was well tolerated, and the patient discharged postoperative day 1.
Complete (bilateral) agenesis is the rarest form of pericardial agenesis, and has the best prognosis without concern for herniation. Left hemiagenesis is most common, accounting for 70-80% of cases. Smaller left sided defects are the most dangerous, and should be closely evaluated for strangulation and involvement of coronary arteries prior to proceeding with pericardioplasty.