Venolymphatic malformation of the Vulva: A case report

Introduction

Venolymphatic malformations are benign, but rare congenital lesions that can be found throughout the body. They tend to grow in size along with the patient, and thus are especially rare in the pre-adolescent pediatric population. In this article, we present a case of a vulvar lymphatic/vascular malformation in a 3-year-old female.

Case Presentation

A 3-year-old, otherwise healthy, female presented with a labial/clitoral mass that had been growing over the past 2 years. A previous biopsy demonstrated benign vascular proliferation with stromal components, concerning for a vascular malformation versus hamartoma. Pelvic MRI was consistent with a lymphatic malformation. She was evaluated by both a pediatric endocrinologist and a genetic counselor who ruled out underlying hormonal abnormalities, such as central puberty or congenital adrenal hyperplasia, or genetic causes, such as neurofibromatosis 1. The 3.6 × 2.1 × 1.7cm mass was surgically excised with a multidisciplinary team with pediatric surgery, gynecologic oncology, and plastic and reconstructive surgery with final pathology demonstrating a lymphatic/vascular malformation. A rotational advancement flap was used to reconstruct the area. At 9 months, the sensation was preserved with good cosmesis.

Conclusion

Vulvar venolymphatic malformations in the pediatric population are exceedingly rare. This is a highly sensitive area and should thus be approached both methodically and cautiously with a multidisciplinary approach.