Secondary amenorrhea attributed to occlusion of perforate transverse vaginal septum

Abstract

OBJECTIVE: Transverse vaginal septum (TVS), the most common vaginal congenital anomaly, still remains a rare finding (1:30,000 to 1:84,000). A TVS can either be imperforate or perforate. Imperforate TVS may present during the neonatal period with hydrometrocolpos or with the onset of puberty with pelvic pain. Perforate TVS usually presents as an incidental finding, with either regular or irregular menses and dysmenorrhea. We present a patient with normal menses for 4 years followed by secondary amenorrhea due to an obstructed perforate TVS. DESIGN: Individual case report. MATERIALS AND METHODS: Not applicable. RESULTS: A 19 yo G0 patient presented to an REI specialist for irregular menses with a large hematometra on imaging. Her menarche began at age 14 with regular menses every 28-30 days lasting 5 days. At the age of 18 she began irregular menses with up to 6 months of amenorrhea. Eight months following the start of irregular menses the patient experienced bilateral lower abdominal pain. Ultrasound revealed an enlarged uterus measuring 17.8 x 6.9 x 3.7cm and an endometrial cavity and cervix distended with fluid and debris. An MRI study showed a large amount of fluid in the uterus with gravity-dependent shading. Physical exam revealed a 4cm vagina with thickened fibrotic tissue at the apex and no cervix. The patient underwent surgical evaluation and treatment. Laparoscopy showed a large hematocolpometra, with an otherwise normal uterus, bilateral fallopian tubes and ovaries. There was endometriosis on the surface of the ovaries, the bladder flap, and in the cul-de-sac. A stab incision was made in the bulging vaginal septum and 600ml of dark blood was evacuated. The incision in the vaginal septum was increased in a cruciate fashion, and excess tissue was excised with cautery and sutured. CONCLUSIONS: The upper 1/3 of the vagina is formed by cells from the mullerian ducts and the lower 2/3 from the urogenital sinus. A TVS is from the abnormal fusion between the mullerian ducts and the sinovaginal bulbs derived from the urogenital sinus. An unobstructed septum may be asymptomatic and appear incidentally. It may result in hypomenorrhea, dyspareunia, and dysmenorrhea. We present the first reported case of a patient with a perforate TVS with 4 years of regular menses and no pelvic pain, followed by an abrupt shift to irregular menses and eventually secondary amenorrhea with new-onset pain. This case demonstrates the possibility of total occlusion of a perforate TVS likely resulting from septal scarring from gradually retained blood and tissue.