ABSTRACT
Extracranial congenital arteriovenous malformations (AVMs) are rare clinical entities that can be progressive in nature. The influence of pregnancy on lesion progression has been discussed in the past. This report presents an unusual case of 23-year-old primigravida woman who presented at 36 weeks' gestation with complicated necrotic ulceration and hemorrhage of the right gluteal region. A hyperpigmented mark with varicosities was initially noted at birth, but during pregnancy it showed remarkable progression and was first identified as an AVM. After hemorrhage control and induced delivery, the lesion was successfully treated with several embolizations. Complete wound healing was achieved, but because of partial recurrence at 3 years, repeat embolization was performed, with satisfactory clinical improvement and residual 25% arteriovenous shunting on transarterial lung perfusion scintigraphy study. AVM complications during pregnancy are uncommon, and this case supports the prior opinion that pregnancy can stimulate lesion progression. Especially in undiagnosed and previously untreated cases, this can lead to life-threatening complications for the mother and fetus. Long-term lesion management usually requires combined endovascular and surgical treatment.