76. Hematocolpos caused by Vulvovaginal Graft-Versus-Host Disease

Abstract

<h3>Background</h3> One of the mainstays of treatment of Diamond-Blackfan anemia after failure of initial supportive care is allogeneic transplantation. Of the many sequelae of allogeneic hematopoetic stem cell transplantation in female survivors, graft-vs-host disease of the vulva and vagina remains one of the most common and destructive to quality of life. Labial fusion and adhesions, urethral obstruction, vaginal scarring and more uncommon - hematocolpos - are some of the presentations of severe disease. In this case report, we will discuss the treatment hematocolpos as a sequelae of GVHD following allogeneic transplantation. <h3>Case</h3> 16 year old with Diamond-Blackfan anemia status post HLA-matched unrelated donor bone marrow transplant presented 7 months post-transplant with 2 days of low abdominal pain. Pain was 8/10, intermittent, nonradiating, worse with movement and completion of urination and improved with heat packs and oral pain medication. Menarche was at age 15, and she experienced one period prior to transplant which lasted 4 days with moderate flow and no pain. Pretransplant, she had received Depot Lupron and then maintained on norethindrone acetate 5mg daily which was increased after she developed breakthrough bleeding following transplant and then stopped taking 4 months after transplant. Following transplant, she developed secondary amenorrhea consistent with hypothalamic suppression. On presentation, she had GVHD with skin, oral and ocular involvement, normal abdominal exam and a pelvic exam with Tanner stage IV development, labia majora without posterior fusion, rugation, or hyperpigmentation, and labia minora without adhesions or lesions. The distal vagina was present with patent hymen, and a Q-tip was unable to be passed beyond 2cm into the vagina. Transvaginal ultrasound was notable for a complex cystic lesion continuous with the uterine edometrium and upper vagina, demonstrating an air-fluid level measuring 5.9 × 5.9 × 5.4cm, consistent with moderate hematocolpos. Left and right ovaries unremarkable. MRI corroborated these findings. She has undergone 3 separate exams under anesthesia with lysis of adhesions but remains nonadherent to topical steroid or estrogen therapy. She takes continuous combined pills for menstrual suppression. <h3>Comments</h3> This patient highlights an uncommon instance of stenotic vaginal, without vulvar, GVHD. With an absence of external symptoms of GVHD on her genitals, diagnosis may have been delayed. The patient's discomfort with topical therapy necessitates the use of alternative treatment options, which are limited. Menstrual suppression addresses the hematocolpos, but does not address the adhesions, which may impair both future sexual intimacy and overall quality of life.