IPEG 2020 TOP ABSTRACT: Pericardial Agenesis

A 9-month-old boy was diagnosed intraoperatively with pericardial agenesis during thoracoscopic resection of a CPAM (congenital pulmonary airway malformation).

CT scan at 7 months demonstrated a lesion in the region of the left upper lobe.

Postoperative imaging review revealed slight leftward cardiac positioning and no appreciable lung in the pre-aortic recess, which is the described finding in pericardial agenesis.

The lesion was not aerated or connected to an airway, leading to diagnosis of pulmonary sequestration with arterial supply from the pulmonary artery and venous drainage into the innominate vein.

Complete or bilateral pericardial agenesis was diagnosed during surgical exploration.

The location of the phrenic nerve was unable to be identified during the procedure.

A very short arterial pedicle was thought to be more risky for initial dissection, prompting dissection of the venous pedicle first.

Initial dissection of the venous pedicle allowed identification of the left vagus nerve.

The vein was ligated using bipolar electrocautery.

Great care was taken to avoid injury to the left pulmonary artery during dissection.

Once the artery had been skeletonized, it was doubly clipped using hemalock clips.

An arteriotomy was created to verify hemostasis prior to complete transection.

There are fewer than 400 reported cases of pericardial agenesis in the literature.

Bilateral pericardial agenesis comprises 9% of all pericardial agenesis cases.

Complete pericardial agenesis carries a favorable prognosis without risk of the feared complication of cardiac herniation.