Direct EBUS-guided transtracheal lymphosclerosis for plastic bronchitis after Fontan

Area of Science:

• Cardiology
• Interventional Radiology
• Thoracic Surgery

Background:

• Plastic bronchitis (PB) is a rare but serious complication following Fontan repair.
• Current treatment options for PB are limited, often involving medical management with variable success.

Observation:

• High-resolution contrast-enhanced spiral CT revealed enlarged paratracheal lymph nodes.
• Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography (DCMRL) demonstrated abnormal lymphatic flow and leakage into the bronchial tree.

Findings:

• A new technique utilized endobronchial ultrasound to access and puncture enlarged paratracheal, subcarinal, and hilar lymph nodes.
• Direct transtracheal mediastinal lymphatic access allowed for targeted occlusion of aberrant lymph vessels using lipiodol/NBCA (Histoacryl).
• Successful occlusion resulted in total remission of plastic bronchitis, with sustained results at 10 months follow-up.

Implications:

• This innovative procedure offers a potential new therapeutic strategy for plastic bronchitis after Fontan repair.
• Minimally invasive lymphatic embolization may provide a more effective treatment for this challenging condition.
• Further research is warranted to evaluate long-term efficacy and applicability in a broader patient population.