Persistent left superior vena cava discovered during central line insertion in a patient with Joubert syndrome: a...

Persistent left superior vena cava is the most common thoracic venous anomaly. It is usually asymptomatic and discovered incidentally during diagnostic or therapeutic interventions. It can complicate central line insertion, causing arrythmias and thromboembolic complications. Joubert syndrome and related disorders are a group of rare congenital disorders associated with developmental abnormalities and multiple organ system involvement including renal, hepatic, and neurological manifestations.

We report a case of a 12-year-old Kuwaiti Arab male child with Joubert syndrome, who was diagnosed with renal and hepatic failure and required central line insertion to initiate total parenteral nutrition. The central line was inserted through the left internal jugular vein because the patient had a dialysis catheter inserted in the right subclavian vein. After the line insertion, a chest X-ray revealed that the catheter appeared to descend directly into the left mediastinum rather than crossing to the right mediastinum through the innominate vein. Blood gas sample and subsequent echocardiography confirmed the presence of the catheter in a persistent left superior vena cava. The catheter was kept in place and used without complication for total parenteral nutrition and resuscitation for 18 days.

Although persistent left superior vena cava is a rare anomaly, knowledge of its presence is important for the clinician in order to avoid complications associated with utilizing it for venous access, and to avoid unnecessary replacement for an uncomplicated catheter inserted in it. This is especially important for patients who would be expected to require invasive interventions, such as patients with Joubert syndrome.