Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome

A 27-year-old nulliparous woman, with menarche at age 12 and normal menses. The patient did not report having dysmenorrhea; however, intermittent dyspareunia, chronic pelvic pain and abundant vaginal purulent discharge episodes were present in the past. Her medical history was significant for a congenitally absent right kidney, diagnosed at age 15, and infertility history for over 6 years. The infertility causes of the couple were investigated, and the magnetic resonance imaging (MRI) identified a uterus didelphys with partial longitudinal vaginal septum, moderated hematocolpos in the right hemi-uterus, ipsilateral renal agenesis and right ureteral ectasia with ectopic vesical insertion, suggestive of renal scar regression ( Fig. 1 ). The patient had not had any treatment at that point, as she ceased to attend infertility appointments. Coronal abdomino-pelvic MRI – uterus didelphys, ipsilateral renal agenesis, and right ureteral ectasia with ectopic vesical insertion. She achieved a spontaneous pregnancy and was referred to our prenatal care department, in first trimester, due to the uterine anomaly and its associated risks, as well as complaints of pelvic pain and vaginal purulent discharge. The general physical examination was unremarkable. Copious quantities of yellowish discharge from the left-side cervix were found on the speculum exam. A vaginal swab was collected, and cultures of Streptococcus oralis and mitis stains were isolated. The laboratory evaluations, including leukogram and c-reactive protein (CRP), were within normal limits. The patient was started on cefuroxime according to an antibiotic susceptibility test, and continued daily antibiotic prophylaxis with 1 g Amoxicillin throughout pregnancy. The pelvic ultrasound scan revealed a single fetus on the left hemi-uterus, and the right hemi-uterus was filled with fluid with altered echogenicity. Two separate cervices were documented, the left anterior and right posterior ( Figs. 2 and 3 ). Pelvic ultrasound scan – fetus on the left hemi-uterus and right hemi-uterus with altered echogenicity fluid (*). Pelvic ultrasound – two separate cervices, left anterior (32.3 mm) and right posterior (29 mm). The patient attended a Maternal-Fetal Medicine consultation, and serial evaluations of inflammatory parameters and cervical length were performed throughout the pregnancy, and they remained within normal limits. The fetus showed appropriate growth monitored in a serial ultrasound every four weeks. The patient experienced spontaneous labor at 39 weeks. The partial septum was not an impediment to the vaginal birth, and a healthy female newborn weighing 2,645 g was delivered. The postpartum period progressed uneventfully. She was discharged within 48 hours postpartum ( Fig. 4 ). Postpartum pelvic ultrasound. In the absence of any signs and symptoms, the routine assessment of the maternal health was undertaken six weeks after delivery, and the partial vaginal septum was not found.

Herlyn-Werner-Wunderlich syndrome is extremely rare. To our knowledge, there are five cases of pregnancies associated to HWWS, 12 13 14 15 seven cases of HWWS with pyocolpos, 16 17 18 19 20 21 22 one case of pregnancy in HWWS with pyocolpos, 23 and two cases of pregnancy with pyocolpos in a uterus didelphys. 24 25 According to the European Society of Human Reproduction and Embryology (ESHRE) and the European Society for Gynecological Endoscopy (ESGE) 26 consensus on the Classification of Female Genital tract congenital anomalies, HWWS appears to be included in Class U3B uterine anomaly, class C2 cervix anomaly, and class V3 vaginal anomaly. Herlyn-Werner-Wunderlich syndrome is commonly diagnosed at the time of menarche. The vaginal septum is generally longitudinal, and can have a variable thickness. Delay in diagnosis is common especially due to: the communication between the two cavities, from the incomplete hemivaginal obstruction; septum elasticity; and the use of drugs, such as oral contraceptives and non-steroidal anti-inflammatory drugs, which might minimize symptoms and, therefore, further delay the recognition of the condition. A high index of suspicion is required to diagnose these cases at an early stage, avoiding complications, such as retrograde tubal reflux and consequent endometriosis and infertility. 27 28 When clinical signs and symptoms are present, the ultrasonography is usually the initial image exam performed, but it is highly dependent on the expertise of the operator. Magnetic resonance imaging is considered the gold standard for diagnosis and preoperative planning for the treatment of HWWS. 29 30 31 Treatment should be individualized depending on complaints, and the main goal is to relieve the obstruction by remodeling the vagina. Some vaginal septa can be easily displaced to the side, and others may be thick enough to cause symptoms, and therefore require surgical excision. Our patient was essentially asymptomatic, which explains the late diagnosis of this condition, during an infertility investigation. The presence of a partial vaginal septum can lead to late diagnosis due to the lack of exuberant symptoms, as menses can outflow throughout that communication. Congenital developmental anomalies of the Müllerian ducts are a significant etiological factor of infertility, and are associated with an increment in obstetrical complication. A recent meta-analysis 32 revealed that uterus didelphys was associated with increased rates of preterm delivery and higher incidence of fetal breech presentation in comparison with women with a normal uterus. Other studies 30 33 revealed other obstetric complications, namely miscarriage, premature rupture of membranes, and intrauterine growth restriction among patients with uterus didelphys. Therefore, more frequent and rigorous obstetric monitoring is required to identify potential obstetric complications. In our case, we performed serial transvaginal ultrasounds, from the twentieth week forward, for preterm birth screening. Cervical incompetence is not usually associated with uterus didelphys, and therefore cerclage is not routinely used. In order to screen intrauterine growth restriction, an increased frequency of surveillance was performed, with serial ultrasound estimation of fetal weight, along with an umbilical artery Doppler study. Drainage of pus with a urinary catheter or dilatation of the cervix was not considered due to the abundant outflow of discharge. The pyometra was treated with antibiotics, according to culture and sensitivity. However, every time the antibiotic was discontinued, yellowish vaginal discharge and intermittent pelvic pain complaints worsened, which justified the maintenance of the antibiotic throughout the pregnancy. We presume that a partial vaginal septum might have been critical to the success of the pregnancy, preventing the ascending infection of the pregnant uterus. A uterus didelphys is not an indication for cesarean delivery, and thus vaginal delivery should be considered first, if there is no obstruction of the birth canal. 34 35 36 The absence of similar cases in the literature proved to be a challenge in the management of this clinical case. Careful attention is necessary for early recognition and appropriate management of this condition for a successful outcome.

Mullerian duct anomalies (MDAs) are congenital defects of the female genital system that arise from the abnormal embryological development of the Mullerian ducts. 1 2 These abnormalities include a wide range of developmental anomalies, resulting from failure of development, defective fusion or defects in regression of the septum during fetal development. A review of the prevalence of different types of uterine malformations revealed that uterus didelphys was found to be the second least common of all MDAs. 3 Herlyn-Werner-Wunderlich syndrome (HWWS), also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome was first described in 1971–1976. 4 5 It is a rare congenital anomaly of the female genital tract characterized by uterus didelphys, ipsilateral renal agenesis and blind hemivagina. Its prevalence is difficult to ascertain, but it is estimated to be 0.1–3.8%. 1 In the literature, the syndrome often appears as a single case report or as a small series. 3 6 7 8 9 Patients with this disorder usually present, shortly after menarche, with intermittent dysmenorrhea, irregular vaginal hemorrhage, mucopurulent discharge, acute pelvic pain or palpable mass due to the associated hematocolpos or hematometra, which result from retained long standing menstrual flow in the obstructed hemivagina. The rarity of this condition may contribute to diagnostic delay. Early diagnosis with appropriate surgical intervention with vaginoplasty and vaginal septostomy decreases the long-term morbidity of these women. 10 11 The surgical outcome is excellent, and it is associated with a successful reproductive performance in the future. In this case report we discuss a rare case of a HWWS patient who kept a chronic purulent discharge throughout all pregnancy, but carried her pregnancy to term and delivered vaginally without complications.