61 Menses Suppression in an Adolescent with Vaginal Agenesis.

Abstract

Background: Mayer-Rokitansky-Kuster-Hauser Anomaly (MRKHA) exists within a spectrum ranging from the complete absence of all müllerian structures to the presence of a normal uterus but vaginal agenesis. Patients with this disorder have normal ovarian function and normal pubertal development, but primary amenorrhea. In girls with a uterus and an anatomic outflow obstruction, endometrial build up can lead to significantly increased pressure in the genital tract with retrograde flow of menstrual blood, resulting in an increased risk of endometriosis and abdominal pain. The optimal clinical management of such patients is unknown. Objective: We describe a case of MRKHA with a particularly severe initial presentation. Current treatment options for menses suppression in this setting are discussed. Results: The patient was a previously healthy 12 year old female who presented with an acute abdomen. Laparoscopy revealed hematosalpinx and absence of a vagina. The right fallopian tube was severely distended and removed. MRI and vaginoscopy confirmed the diagnosis of MRKHA. As the family opted to defer vaginoplasty until the patient was older, menses suppression was sought in order to prevent future episodes of severe abdominal pain and endometrial proliferation. Three potential therapeutic approaches were identified; GnRHa suppression, depot medroxyprogesterone acetate and combination estrogen/progesterone. However, minimal data on comparable safety and efficacy of these preparations for the purpose of suppressing menses in a healthy adolescent girl were found. Due to theoretical concerns regarding adverse effects on bone mineral density with the other two choices, the decision was made to initiate treatment with the estrogen-progesterone patch. However after several months on treatment, she was 12 hours late in changing her patch and presented to the emergency room with abdominal pain. Pelvic ultrasound showed uterine and pelvic blood. She was then changed to a 30μg estrogen with progesterone pill for improved continuous estrogen suppression of the endometrium. No further episodes of bleeding have been noted over the past 8 months. Conclusions: Complete suppression of menses in an otherwise normal child is an infrequent goal of clinical management. This case emphasizes the difficulty of choosing the ideal pharmacologic strategy and highlights the lack of long term therapeutic outcome data in similar patients. Prospective controlled studies are needed to investigate safety and efficacy of different therapeutic modalities for menses suppression in those who choose to delay surgical correction of a non-patent outflow tract.