Congenital Deficiency of the Pericardium

Abstract

A case report, plus a brief review of the salient features of congenital deficiency of the pericardium, is offered because of the rarity of the condition. Diagnosis, if one is aware of the entity, can be made from plain x-ray films of the chest. Case Report A 10-year-old boy was seen in the Outpatient Service with symptoms of acute pneumonia. A chest film revealed pulmonary infiltrate in the left lower lobe, primarily the superior segment, and some pleural effusion. In addition, the unusual cardiac silhouette seen in Figure 1 was noted, and a left upper mediastinal mass discovered. Fluoroscopic examination was subsequently performed. The aortic knob could be seen through the shadow of the mass and appeared normal. The pulmonary artery pulsation was unduly prominent, but left and right branches did not pulsate abnormally. No pulmonary plethora could be suspected. The relative levoposition of the heart made it appear enlarged on the postero-anterior film, but the cardiac chambers and total volume were normal fluoroscopically. The mediastinal mass did not pulsate and was thought probably to be a bronchogenic cyst. On the basis of the plain films alone, Dr. Benjamin Felson suggested the possibility of congenital absence of the pericardium, the diagnostic features being levoposition of a normal-sized heart and the prominent pulmonary artery shadow with air density of lung outlining the notch between it and the aorta. The fluoroscopic finding of pronounced pulsation of an otherwise normal pulmonary artery afforded further confirmation. Diagnostic pneumothorax was considered but was not carried out, since operation for the mass was planned. The child was admitted for thoracotomy after uneventful clearing of the pneumonia. There was no history of previous illness except for temporary anemia at the age of five. No previous chest films had been made. The father had been retired for arrested pulmonary tuberculosis. The mother, five brothers, and four sisters were all reported living and well with negative skin tests for tuberculosis. The patient was the “most fragile” of the 10 children but was not considered ill. He could stand on his head without noticeable eflect. The physical examination was unremarkable. Pertinent negative findings included a normal heart examination, with no murmurs. The point of maximal impulse was 1 cm. to the left of the mid-clavicular line. There was no venous distention. The patient was 54 inches tall and weighed 64 pounds. Urinalysis was negative, and hemoglobin and hematocrit were normal. At surgery, on Jan. 5, 1961, a small cystic mass was removed from the left upper mediastinum. Microscopically, it was identified as representing pulmonary sequestration, pathologically quite similar to that in the case of Rusby and Sellers (3). This aspect is to be reported in another paper by Lt. Col. Hu Blake.