[Benign aggressive vascular anomalies in children]

Area of Science:

• Vascular biology and pathology
• Dermatology
• Pediatrics

Background:

• Superficial vascular anomalies encompass tumors (e.g., infantile hemangioma) and malformations (VMs) of various vessel types.
• The International Society for the Study of Vascular Anomalies (ISSVA) classification distinguishes tumors (hyperplasia) from malformations (dysplastic vessels).
• While infantile hemangiomas are common and treated with propranolol, rarer conditions like congenital hemangiomas with Kasabach-Merritt phenomenon are managed with sirolimus.

Purpose of the Study:

• To review the classification, pathogenesis, and current management strategies for superficial vascular anomalies.
• To highlight the evolving role of medical therapies, particularly sirolimus, in treating complex vascular malformations.
• To emphasize the importance of individualized, multidisciplinary approaches to patient care.

Main Methods:

• Review of existing literature and clinical guidelines on vascular anomalies.
• Discussion of pathogenic differences between vascular tumors and malformations.
• Analysis of treatment outcomes for various vascular anomalies, including pharmacological interventions.

Main Results:

• Vascular tumors like infantile hemangioma are treated with propranolol; Kasabach-Merritt phenomenon is managed with sirolimus.
• Low-flow VMs (capillary, venous, lymphatic) and high-flow VMs (arteriovenous) have distinct characteristics and management needs.
• Interventional treatments (surgery, sclerotherapy) offer partial efficacy; medical drugs are increasingly central to VM management.
• Low molecular weight heparin is first-line for venous malformation coagulation disorders; sirolimus shows efficacy in refractory hemorrhagic complications and painful lymphatic malformations.

Conclusions:

• Superficial vascular anomalies require precise classification and tailored treatment strategies.
• Sirolimus is emerging as a key therapeutic agent for complex and refractory vascular malformations.
• Multidisciplinary consultation and individualized case management are crucial for optimizing outcomes in patients with vascular anomalies.