Lymphaticovenous and Lymph Node-to-Vein Anastomosis to Improve Milroy Disease-Related Chylothorax and Lymphedema.

Background: Primary lymphedema is characterized by lymphatic dysplasia in which one variant is Milroy disease. The association with congenital chylothorax is even rarer, with poor outcome. This is the first report to use peripheral lymphovenous anastomosis (LVA) and lymph node-to-vein anastomosis (LNVA) for the management of such condition.

Methods: A retrospective chart review of patients with Milroy disease with complication of chylothorax between 2019 until 2023 was performed. Clinical assessment and radiologic investigations were reviewed.

Results: Six patients with a mean age of 12 ± 3.9 years and disease duration of 10.5 ± 2.8 years were reviewed. Three had International Society of Lymphology stage 3 disease, and the 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 ( P = 0.028) along with nearly complete resolution of chylothorax were noted during the long-term follow-up (32 ± 17.9 months).

Conclusions: Milroy disease complicated with chylothorax remains challenging. This is the first report using 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.

Clinical question/level of evidence: Therapeutic, IV.