Transvenous retrograde thoracic duct embolization for effective treatment of recurrent plastic bronchitis after Fontan palliation

Abstract

Plastic bronchitis (PB) is a rare but potentially detrimental complication of the Fontan circulation. Retrograde transvenous thoracic duct embolization (TDE) has been proposed as a treatment strategy for lymphatic dysfunction. A 5.5-year-old patient (16 kg/105 cm) was referred to our institution for severe PB refractory to conservative treatment 3 months after completion of Fontan palliation. CT-scan showed multiple bilateral areas of patchy consolidation and ground-glass opacity with obstruction of the right main and right lower bronchi. Bi-inguinal transnodal fluoroscopy-guided lymphangiogram confirmed the diffuse and abnormal lymphatic leak originating from the thoracic duct into the chest, predominantly right-sided, and showed the TD ostium near the left venous angle. There was no opacification of any target central lymphatic vessel for direct transabdominal puncture. Retrograde approach from the femoral vein was adopted to catheterize the thoracic duct and selectively embolize its distal portion using intercalation of microcoils and liquid embolic adhesive. Recurrence of symptoms after 2 months indicated a redo catheterization that revealed the persistence of dilated peribronchial lymphatic network with a minimal residual leak across the previously embolized caudal section of the TD. Complete occlusion of the TD over its entire length was performed using the same technique. The procedure was technically successful and the patient was uneventfully discharged after 2 days. There was no cast recurrence or procedure-related complications and sustained clinical improvement at 18-month of postoperative follow-up. Fluoroscopy-guided lymphangiography was essential to understand the anatomy and flow pattern of the lymphatic system and to plan the lymphatic intervention. End-to-end TDE is an effective minimally invasive treatment in Fontan patients with PB and can be necessary in case of relapse after selectively embolizing the TD distal section.