An asymptomatic four year-old boy, weighing 13.8 kg, presented for reevaluation following construction of the Fontan circulation for double outlet right ventricle, atrioventricular septal defect with common atrioventricular junction, and pulmonary stenosis three years earlier. The operative report from a hospital abroad indicated that he had undergone a lateral tunnel procedure with an adjustable fenestration: “The fenestration hole had been surrounded by a 3-0 prolene suture and exited out the lateral atrial wall. The chest was then drained with a 20 Fr chest tube through a separate stab wound incision into the mediastinum.” The plan was “to close the fenestration prior to returning to his native country”, but it was unclear whether this had been done, or whether the suture material had been removed completely.

Echocardiography and catheterization confirmed complete occlusion of the inferior caval vein at its junction with the right atrium, superior to the hepatic veins. Additionally, there was a suppurating lesion on the right anterior chest wall (Fig. 1a) that shows the healed midline sternal incision (arrow) with the suppurative lesion to its right, from which Staphylococcus aureus was isolated. Figure 1b is an enlargement of the lesion, showing the orifice of the sinus. A sinugram in the left anterior oblique 90-degree projection showed a long serpentine tract, with extension to the pericardium deep within the mediastinum (Fig. 2). Dye passes into the mediastinum and to the pericardium (asterisk).

Figure 1.

Figure 2.

Following two weeks of treatment with antibiotics, the patient was converted to an extracardiac Fontan using a 16 mm bovine conduit (Contegra) without its valve, in order to reestablish patency of the inferior caval vein. The chronic granuloma surrounding the fistula and infiltrating the right atrium was resected, together with the Gore-tex patch of the intraatrial tunnel, which was encapsulated in a suppurating lake, cultures of which were sterile. The postoperative course was uneventful, and the patient remains well at a follow-up of ten months.

We assume that this unusual sinus was due to infection of the adjustable fenestration, as no foreign suture material was found at reoperation. The case also emphasises the necessity for complete and accurate documentation of management in patients who travel around the world and undergo complex procedures in a global healthcare system.