A Unique Support System for Teens Diagnosed With MRKH-Mayer Rokitansky Kuster Hauser Syndrome and Their Families

Abstract

There was no clitoromegaly and the remaining genital structures were normal. Translabial ultrasound identified an anechoic labial cyst with thin septations measuring 8.7 X 3.5 X 4.8cm. MRI confirmed these sonographic findings. Percutaneous drainage was performed with a 4 French centesis needle using ultrasound guidance (see figure 2). Following a fluoroscopic contrast study and aspiration of the contrast, doxycycline sclerotherapy was performed using 30cc of concentrated doxycycline (20 mg/cc). The sclerosant was aspirated after 20 minutes of dwell time. Subsequent delayed labioplasty was performed with excellent cosmetic results (see figure 3,4). Comment: Doxycycline functions as an inhibitor of angiogenesis by interfering with cell proliferation and migration via inhibition of matrix metalloproteinase and suppression of vascular endothelial growth factor induced angiogensis and lymphangiogenisis. It causes a fibrocytic cellular reaction with deposition of fibrin and collagen resulting in cystic regression. While surgical treatment remains the standard of care, sclerotherapy may provide a less invasive approach to treatment of large lymphatic malformations of the labia. This case suggests that doxycycline percutaneous sclerotherapy is a safe and effective treatment for labial lymphatic malformations.