Vulvar AV Malformation in a 16-Year-Old Girl
Congenital arteriovenous malformations (AVMs) are infrequent but represent a serious medical challenge because of their unpredictable progression and high hemodynamic activity.

Published in the Journal of Pediatric and Adolescent Gynaecology. The authors report on the treatment of a voluminous vulvar AVM in a 16-year-old girl. After failure of medical therapy, a radical surgical resection was performed with preliminary embolization and flap reconstruction.

A 16-year-old girl reported with a congenital AVM of the right labia majora (97 × 140 × 30 mm). She had previously undergone 2 selective embolizations at 2 and 9 years of age. The lesion did not progress for 2 years after the last treatment but then suddenly increased in volume when the patient was 11 to become painful, inflamed, ulcerated, and turgid with a nodular aspect. The size of the lesion and the labia asymmetry caused discomfort and was esthetically displeasing.

The patient was treated with a mammalian target of rapamycin (mTOR) inhibitor as targeted therapy combined with prophylactic antibiotic therapy. The aspect of the lesion improved within a month of treatment: the skin color returned to normal, the ulceration healed, and the volume and pain decreased.

Two years later (9 months after her first menstruation) the patient experienced cutaneous erosions along with severe episodic bleeding, lesion enlargement, and pain. The mTOR inhibitor was stopped and a β-blocker treatment was introduced to control the bleeding. Because this deterioration was thought to be hormonal-based, antiandrogen medication (cyproterone acetate) was initiated. This resulted in a short transient improvement before ulceration, bleeding, and pain returned.

Because medical therapy was no longer effective, we decided to perform surgery. The patient was hospitalized in a pediatric ward because of her young age. MRI revealed a lesion of 97 × 140 × 30 mm affecting the right labia majora and the pubis with a high-flow vascularization stemming from the right external iliac artery, the right common femoral artery, and both internal and external pudendal arteries. The lesion was drained using the femoral and right pudendal veins, forming an arteriovenous anastomosis in the nidus.

Continue reading about the case here: