Vulvar vascular malformations

Background: Vascular malformations are congenital structural abnormalities of the blood vessels that may present at any age. In the vulvovaginal region, these lesions are uncommon and frequently misdiagnosed because their clinical appearance overlaps with common gynecologic conditions, particularly Bartholin’s gland cyst or abscess. Inappropriate surgical intervention without prior vascular evaluation may result in hemorrhage, incomplete treatment, and recurrence. Methods: A structured narrative review of the literature was performed using PubMed/MEDLINE and EMBASE databases (January 2000–April 2024) to summarize the classification, pathophysiology, clinical presentation, imaging characteristics, differential diagnosis, and management of vulvovaginal vascular malformations. Publications addressing vascular anomalies in other anatomical locations were also included when clinically relevant. A representative clinical case confirmed by histopathologic and molecular analysis is presented to illustrate the diagnostic pitfalls. Results: Vulvovaginal vascular malformations are predominantly low-flow venous lesions but may include high-flow arteriovenous malformations. A clinical examination alone is insufficient for diagnosis. Doppler ultrasonography is the recommended initial imaging modality, followed by magnetic resonance imaging to define the lesion extent and flow characteristics. Misdiagnosis most commonly occurs when lesions are treated as Bartholin’s gland pathology without prior imaging. Low-flow lesions are generally managed with sclerotherapy or planned surgical excision, whereas high-flow lesions require embolization and multidisciplinary care. Hormonal and hemodynamic changes, including pregnancy, may precipitate enlargement or thrombosis. Conclusions: Vascular malformations should be considered in the differential diagnosis of atypical vulvar masses. Preoperative imaging is essential in order to avoid inappropriate surgical procedures. A structured diagnostic approach combining clinical assessment and imaging enables correct classification and guides treatment. The presented case demonstrates a typical diagnostic pitfall and emphasizes the importance of recognizing vascular lesions in gynecologic practice.