Diagnosis and treatment of Wunderlich syndrome

Abstract

Wunderlich syndrome is a rare Mullerian anomaly consisting of uterine didelphy, cervical cyst, and ipsilateral renal agenesis [1]. It is likely that the malformations occur from embryonic arrest at 8 weeks of gestation, at which point the adjacent Mullerian and metanephric ducts are simultaneously affected. This anomaly inhibits the outflow of menstruation and causes menstrual blood to pool in the uterus; however, it is often asymptomatic anddifficult to diagnose [2]. A healthy 15-year-old female who had experienced normal pubertal events underwent menarche. Three months after menarche, she complained of lower abdominal pain, especially after menstruation. She was of moderate stature, with normal external genitalia, and a regular menstrual cycle. She was diagnosed by magnetic resonance imaging (MRI) as having a double uterus with a right cervical cyst (Fig. 1). Intravenous pyelography demonstrated right renal agenesis with normal function and contour of the left renal collecting system (Fig. 1). The left cervix was intact and the right cystic wall was resected using an ultrasonic cutting and coagulating surgical device (Harmonic scalpel; Johnson & Johnson, New Brunswick, NJ, USA). She was diagnosed with Wunderlich syndrome since the resected tissue demonstrated cervical glands. The postoperative course was uneventful. A healthy 18-year-old female complained of intermittent purulent vaginal discharge since menarche. She underwent menarche at the age of 12 years and experienced normal pubertal events. She had a history of regular menses with increasing lower abdominal pain lasting 2–4 days during each menstrual cycle. She was ofmoderate stature with normal external genitalia. Abdominal ultrasonography demonstrated a small pelvic cystic mass; MRI demonstrated uterus didelphys with a left cervical cyst containing homogeneous fluid compatible with old blood. Intravenous pyelography demonstrated left renal agenesis. The left cystic wall was resectedusinganultrasonic cutting andcoagulating surgical device, andWunderlich syndromewas diagnosed. The postoperative period was uneventful. Hematocervix of Wunderlich syndrome is diagnosed when the ipsilateral hemiuterus and vagina are not connected. Differential diagnosis includes Herlyn–Werner syndrome, which consists of a renal agenesis and an ipsilateral blind hemivagina [3]. Gartner's cysts of Herlyn–Werner syndrome can be pathologically differentiated from cervical cysts of Wunderlich syndrome. A Gartner's cyst is a cystic remnant of the mesonephric ducts in the vaginal wall, while a cervical cyst demonstrates cervical glands. Common clinical presentations of both Herlyn–Werner syndrome and Wunderlich syndrome are pelvic pain and/or dysmenorrhea shortly after menarche, in association with a vaginal/pelvic mass. When the cyst and vagina are connected, symptoms of abnormal vaginal discharge and bleeding are common. Abdominal and pelvic MRI studies are necessary to confirm and characterize thedetails of uterinemalformation and renal anomaly. Treatment is to resect as much of the obstructing vaginal septum as possible. Errors in surgical management can occur when the diagnosis is incorrect, and laparotomy is performed to explore and resect the intra-abdominal mass. Although fertility is not compromised, spontaneous abortion rate is high. However, patients who carry pregnancy to term often have no obstetric difficulties. Prompt and accurate diagnosis, and subsequent excision of the obstructing vaginal septum, is required in patients with ⁎ Corresponding author. Ehime University School of Medicine, Shitsukawa, Toon, Ehime 791 0295, Japan. Tel.: +81 89 960 5379; fax: +81 89 960 5381. E-mail address: takeyu@m.ehime-u.ac.jp (Y. Matsubara).