The Possible Role of Lymphaticovenous and Lymph Node to Vein Anastomosis for Improvement of Milroy Disease Related Congenital Chylothorax and Lower Extremity Lymphedema

Introduction: Primary lymphedema is characterized by lymphatic dysplasia in which one variant is Milroy’s disease. The association with congenital chylothorax is even rarer with poor outcome. This is the first report to utilize peripheral lymphovenous anastomosis (LVA) and lymph node to vein anastomosis (LNVA) for the management of such condition. Methods: Retrospective chart review of patients with Milroy’s disease with complication of chylothorax between 2019 till 2023 was performed. Clinical assessment and radiological investigations were reviewed. Results: Six patients with mean age of 12±3.9 years and disease duration of 10.5±2.8 years were reviewed. Three had ISL stage 3 while others had stage 2 late disease. All had bilateral lower extremity lymphedema and chylothorax with history of chest tube drainage. After LVA and LNVA, significant reduction in extremity volume was noted (p=0.028) along with near complete resolution of chylothorax was noted during the long-term follow-up (32 ±17.9 months). Conclusion: Milroy’s disease complicated with chylothorax remains to be challenging. This is the first report utilizing peripheral bypass (LVA and LNVA) which resulted in improvement of both lower extremity lymphedema and chylothorax. The utility of this approach represents a promising modality in the management of this devastating condition.