Herlyn Werner Wunderlich Syndrome is a rare congenital Mullerian anomaly that commonly presents with uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. Presented here is a rare variant with contralateral dysplastic kidney with ectopic ureteral insertion instead of renal agenesis. The patient initially presented as a case of recurrent pelvic inflammatory disease who eventually underwent excision of vaginal septum with drainage of pyocolpos. Postoperatively, the patient had urinary incontinence and was eventually referred to Urology for further surgical intervention. The wide range of symptomatology and the uncommon anatomic presentation of this case led to the delay in the diagnosis and the consequent gynecologic complications.
Uterine Anomalies

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12‑year‑old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.
Ureterocele ; Humans ; Femalec ; Uterine Anomalies ; Vagina ; Uterus

Muüllerian duct anomalies (MDAs) are congenital defects arising from probable teratogenic assault at 6–22 weeks of gestation.Uterine didelphys arises from complete lack of fusion of Muüllerian ducts, resulting in two entirely separate hemiuteri, cervices and vaginas.The incidence of MDA is <5% and the frequency of uterine didelphys is 1 in 1000–30,000 women and twin pregnancy in uterus didelphys is only 1 in a million. This is a case of a gravida 1 para 0, with uterine didelphys and unilateral renal agenesis diagnosed of single live intrauterine pregnancy in the left half of uterine didelphys who had a spontaneous passage of meaty material from the right hemiuterus which were histopathologically consistent with degenerating products of conception, hence confirming a dicavitary twin pregnancy. Management should be wholistic encompassing preconception, prenatal, intrapartum, and postpartum period as any complications may arise at any point.
Uterine Anomalies ; Urogenital Abnormalities ; Abortion, Spontaneous

BACKGROUND: Uterine arteriovenous malformation (AVM) is a web of arteries and veins lacking an intervening capillary network. Color flow Doppler is a popular method of diagnosis of uterine AVM. The definitive management is hysterectomy. However, for patients desirous of pregnancy, transarterial embolization is a safe and effective option. Although rare, uterine AVM can complicate pregnancy with torrential bleeding due to hormonal changes and significant remodeling of the myometrium.
CASE: We report a case of a term pregnancyin a 33 year old with a uterine AVM and a previous transarterial embolization procedure who developed a uterine AVM during multi-agent chemotherapy for gestational trophoblastic disease. She consulted for prenatal checkup. Due to the risk of massive bleeding during labor, she underwent elective cesarean section at term and delivered a baby with good outcome.
CONCLUSION: This case suggests that uterine AVM in pregnancy can be managed conservatively with serial ultrasound monitoring and close follow up.

Human ; Female ; Adult ; Pregnancy ; Myometrium ; Uterine Anomalies ; Uterus ; Urogenital Abnormalities ; Arteriovenous Malformations ; Gestational Trophoblastic Disease ; Hysterectomy ; Cesarean Section ; Arteries

Herlyn-Werner-Wunderlich Syndrome (HWWS) is a triad of uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis.
In the review of the locally published literature, there have been seven HWWS cases reported, none of whom were pregnant.
A 24-year-old was diagnosed with Herlyn-Werner-Wunderlich Syndrome during caesarean section of a term pregnancy, occupying the right hemiuterus with obstructed hemivagina. Ultrasound showed uterus didelphys with communicating endometrial cavities. MRI revealed uterus didelphys, two cervices and an obstructed right hemivagina. The patient refused excision of vaginal septum. Two years later, she delivered spontaneously to a live fetus, occupying the hemiuterus with the unobstructed hemivagina.
In pregnant women with HHWS, who did not undergo prior surgical intervention, the mode of delivery depends on the side of pregnancy. If it is located on the obstructed hemivagina, caesarean section is inevitable. If it is on the unobstructed side, there is hope for vaginal delivery.

Human ; Female ; Adult ; Pregnancy ; Hereditary Renal Agenesis ; Uterine Anomalies ; Uterus ; Urogenital Abnormalities ; Ultrasonography ; Cesarean Section ; Magnetic Resonance Imaging ; Fetus

OBJECTIVES: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare female genital tract malformation, and there are few large sample studies reported at home and abroad. The clinical manifestations of this syndrome are diverse, and insufficient understanding may delay the diagnosis and treatment of the patients. This study aims to analyze the clinical characteristics of different types of HWWS patients, and to improve the diagnosis and treatment of HWWS. METHODS: The clinical data of patients with HWWS who were hospitalized in the Department of Obstetrics and Gynecology, Third Xiangya Hospital of Central South University from October 1, 2009 to April 5, 2022 were retrospectively analyzed. The age, medical history, physical examination, imaging examination and treatment of the patients were collected for statistical analysis. The patients were divided into an imperforate oblique vaginal septum type, a perforate oblique vaginal septum type, and an imperforate oblique vaginal septum and cervical fistula type. The clinical characteristics of different types of HWWS patients were compared. RESULTS: A total of 102 HWWS patients were enrolled, with age of 10-46 years old, including 37 (36.27%) patients with type I, 50 (49.02%) type II, and 15 (14.71%) type III. All patients were diagnosed after menarche, with an average age of (20.5±7.4) years. There were significant differences in the age of diagnosis and course of disease among the 3 types of HWWS patients (both P<0.05). Patients with type I had the youngest age of diagnosis [(18.0±6.0) years] and the shortest course of disease (median course of 6 months), while patients with type III had the oldest age of diagnosis [(22.9±9.8) years] and the longest course of disease (median course of 48 months). The main clinical manifestation of type I was dysmenorrhea, and the main clinical manifestation of type II and type III was abnormal vaginal bleeding. Of the 102 patients, 67 (65.69%) patients had double uterus, 33 (32.35%) had septate uterus, and 2 (1.96%) had bicornuate uterus. The vast majority of patients had renal agenesis on the oblique septum, and only 1 patient had renal dysplasia on the oblique septum. The oblique septum located on the left side in 45 (44.12%) patients and on the right side in 57 (55.88%) patients. There were no significant differences in uterine morphology, urinary system malformation, pelvic mass, and oblique septum among the 3 types of HWWS patients (all P>0.05). Six (5.88%) patients had ovarian chocolate cyst, 4 (3.92%) patients had pelvic abscess, and 5 (4.90%) patients had hydrosalpinx. All patients underwent vaginal oblique septum resection. Among them, 42 patients underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen because they had no sexual life history, and the remaining 60 patients underwent traditional oblique vaginal septum resection. Among the 102 patients, 89 patients were followed up for 1 month to 12 years. The symptoms of vaginal oblique septum in 89 patients such as dysmenorrhea, abnormal vaginal bleeding and vaginal discharge were improved after operation. Among the 42 patients who underwent hysteroscopic incision of the oblique vaginal septum without destroying the intact hymen, 25 patients underwent hysteroscopies again 3 months after operation, and there was no obvious scar formation at the oblique septum incision site. CONCLUSIONS Different types of HWWS have different clinical manifestations, but all can be manifested as dysmenorrhea. The patient's uterine morphology can be manifested as double uterus, septate uterus, or bicornuate uterus. The possibility of HWWS should be considered if uterine malformation is combined with renal agenesis. Vaginal oblique septum resection is an effective treatment.
Pregnancy ; Humans ; Female ; Adolescent ; Young Adult ; Adult ; Child ; Middle Aged ; Male ; Dysmenorrhea ; Retrospective Studies ; Kidney Diseases ; Bicornuate Uterus ; Uterine Duplication Anomalies ; Uterine Hemorrhage