Refractory Multisystem Gorham-Stout Syndrome: Robotic Approach, Vascular Microsurgery, and Immunological Therapy

Vollständiger Abstract

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Gorham-Stout syndrome (GSS) is a rare lymphatic malformation with fewer than 400 documented cases, characterized by progressive osteolysis and multisystem involvement. Its refractory management to conventional therapies represents a significant clinical challenge. This systematic review, following PRISMA 2020, synthesizes current evidence on pathophysiological mechanisms, diagnosis, and emerging therapeutic strategies for refractory multisystem GSS, with emphasis on robotic surgery, vascular microsurgery, and immunotherapy. PubMed, Scopus, Web of Science, and Orphanet were searched up to May 2026, including 74 studies. The results suggest that robotic surgery offers precision advantages for spinal stabilization and complex reconstructions; vascular microsurgery controls lymphatic leakage; and immunotherapy with interferon-alpha, sirolimus, and bisphosphonates stabilizes the disease in selected cases. Management requires a multidisciplinary approach, but the lack of clinical trials and heterogeneity limit high-quality evidence. Prospective registries and validated diagnostic criteria are needed.