MANAGEMENT OF AN AESTHETICALLY DISABLING COMPLEX VULVAR VENOUS MALFORMATION IN A YOUNG WOMAN

Lenny Suryani Safri, Krishna Kumar, J. Tan, H. Lip, R. Zakaria, M. Idris, H. Harunarashid
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Abstract

Venous malformations (VM) of vulva, perineum and pelvis are uncommon condition which may present with cutaneous varices or aesthetically disabling swelling of external genitalia. Herein, we report a young woman who presented with a large left vulva bluish tinged swelling, progressively increasing in size since birth. Computed tomography of the pelvis and lower limbs confirmed the diagnosis of extensive VM of pelvis and perineum. She underwent selective angiogram which revealed venous malformations of left vulva and gluteal region with no arterial supply to the lesion. Surgical excision was performed but complicated with bleeding which necessitate multimodal hemostatic procedures and blood transfusion. There is no recurrent swelling after 5 years follow up. Surgical excision of vulvar venous malformation offer good cosmetic outcome. However, appropriate planning with axial scan, angiogram and operative technique including multimodal hemostatic measure are important steps to achieve good result with no recurrence. We discussed on challenges on management of this case in relation to the currently available literature.
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一位年轻女性复杂外阴静脉畸形的治疗
外阴、会阴和骨盆静脉畸形(VM)是一种罕见的疾病,可能会出现皮肤静脉曲张或外生殖器肿胀。在此,我们报告一位年轻女性,她表现为左侧外阴大的带蓝色的肿胀,自出生以来逐渐增大。骨盆及下肢计算机断层扫描证实骨盆及会阴广泛VM。她接受了选择性血管造影,发现左外阴和臀区静脉畸形,没有动脉供应病变。手术切除,但合并出血,需要多模式止血手术和输血。随访5年无复发性肿胀。手术切除外阴静脉畸形具有良好的美容效果。然而,适当规划轴位扫描、血管造影和包括多模态止血措施在内的手术技术是达到良好效果且无复发的重要步骤。我们讨论了与目前可用的文献有关的管理该病例的挑战。
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