Clinical Course of Extracranial Arteriovenous Malformations.

Purpose: Despite many advances in the knowledge of vascular malformations, extracranial arteriovenous malformations (AVMs) remain an enigma and are usually misdiagnosed and mismanaged due to their associated rare morbidity. This study aimed to describe the clinical course and emphasize the progressive nature of AVMs through a retrospective study of 446 patients.

Methods: Patients with cutaneous and soft-tissue AVMs presenting to our Vascular Anomalies Center between March 2011 and March 2017 were reviewed. Medical records were examined for disease course, age at first presentation at our institution, distributions and locations of lesions, clinical staging, progression, and previous treatments. Progression was defined as advancement to a higher Schobinger stage from a lower stage.

Results: A total of 446 patients (mean age, 25.6 ± 14.0 years) were enrolled in this study, including 232 (52.0%) males (gender ratio, 1.08:1). Arteriovenous malformations lesions in 76.7% (342/446) of the patients were located in the head and neck. Children with Stage I AVMs had a 41.9% risk of progression before adolescence and an 80.0% risk of progression before adulthood. Nearly all patients (96.2%) showed progression in adulthood. Diffuse lesions were more likely to progress than localized lesions (P < 0.05) in childhood and adolescence. Lesions in the head and neck regions were less likely to progress than those in other regions in childhood (P = 0.005). A total of 216 (48.4%) patients had undergone previous treatments. Among these patients, bleomycin showed an unintentional positive effect in the treatment of AVMs.

Conclusions: Extracranial AVMs have a continuously progressive nature. A full understanding regarding the progressive course of AVMs can lead patients and physicians to attach importance to early diagnosis and management. Meanwhile exploring innovative treatments should be focused in the future to prevent potential destructive progression.