Lympho-venous anastomosis for the treatment of congenital and acquired lesions of the central lymphatic system: a multidisciplinary treatment approach

Congenital and acquired lesions of the central lymphatic system represent rare entities with a significant morbidity. The optimal treatment strategy remains elusive given limited evidence and a variety of different treatment options. Based on the treatment of four patients with anomalies or lesions of central lymphatic system of different origin, we aimed to elaborate an interdisciplinary treatment algorithm. Between 2018 and 2020, two patients with a central conducting lymphatic anomaly (CCLA), a massively dilatated TD, and consecutive chylous reflux were treated. Conservative treatment comprising dietary restrictions and application of octreotide did not have any effect. Given the high risk to worsen the chylous reflux by interventional embolization, both patients underwent lymphovenous anastomosis (LVA) leading to full remission in one patient, and partial remission in the second. A patient with postoperative chylous fistula after neck dissection was treated similarly and also showed complete remission. In contrast, one patient was diagnosed with a spontaneous TD rupture which resolved spontaneously. Patients were followed until 2 years postoperatively including clinical presentation, abdominal sonography, thoracic x-ray, and magnetic resonance lymphangiography to exclude recurrence. Based on the presented case series in which conservative and interventional therapy had failed, we gained important clinical experience in microsurgical treatment of central lymphatic lesions and provide a new treatment algorithm. Radiologic interventions such as recanalization or embolization of the pathology should be evaluated as standard treatment. In case of remaining chylous leaks, chylous reflux with lymphedema, stenosis, or protein-loosing enteropathy, LVA of the thoracic duct represents an innovative microsurgical therapy. Level of Evidence: Level V, therapeutic study.