The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations

Area of Science:

• Vascular Biology
• Rare Diseases
• Medical Genetics

Background:

• Lymphatic malformations (LMs) are congenital vascular anomalies of lymphatic vessels.
• While histologically benign, LMs can cause severe complications like airway compression, disfigurement, psychological distress, recurrent infections, and chronic wounds.
• Existing management guidelines for LMs are limited, often focusing on specific scenarios, and clear indications for treatment and follow-up remain undefined.

Purpose of the Study:

• To develop a standardized diagnostic and management pathway for lymphatic malformations.
• To establish consensus-based strategies for approaching patients with LMs, addressing current gaps in clinical guidance.
• To provide a practical, algorithmic approach for healthcare professionals managing LMs.

Main Methods:

• Utilized the Nominal Group Technique (NGT), a structured consensus-building method.
• Conducted two face-to-face meetings and multiple virtual meetings, supplemented by mail communication.
• Involved experts and patient representatives from the Vascular Anomalies Working Group (VASCA-WG) of VASCERN.

Main Results:

• Developed a comprehensive diagnostic and management pathway for LMs.
• Generated an opinion statement reflecting expert and patient consensus on LM management strategies.
• Created an algorithmic overview for practical patient management.

Conclusions:

• The developed pathway offers a structured approach to LM diagnosis and management.
• This consensus-driven strategy aims to improve the care and outcomes for patients with lymphatic malformations.
• The pathway addresses the need for clearer guidelines in managing these complex vascular anomalies.