uterus didelphys). She had secondary amenorrhea, and an ovarian mass and uterus didelphys were discovered during examination. After right salpingo-oophorectomy, the tumor was confirmed as dysgerminoma, and a chromosome study revealed a normal female karyotype (46, XX). The patient completely responded to 6 cycles of chemotherapy. To our knowledge, this is the first reported case of dysgerminoma with uterus didelphys. Although gynecologic malignancies in patients with Müllerian anomalies are very rare, clinicians should be aware of the coexistence of gynecologic malignancies and uterine malformations.]]>
Adult ; Amenorrhea ; Congenital Abnormalities ; Drug Therapy ; Dysgerminoma ; Female ; Humans ; Karyotype ; Uterus

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.
Abdominal Pain ; Breast ; Congenital Abnormalities ; Dysmenorrhea ; Endometriosis ; Female ; Follow-Up Studies ; Gonadotropin-Releasing Hormone ; Humans ; Infertility ; Kidney ; Magnetic Resonance Imaging ; Mass Screening ; Menstruation ; Parturition ; Puberty, Precocious ; Ultrasonography ; Uterus ; Vaginal Discharge

Adult ; Female ; Humans ; Pregnancy ; Pregnancy Complications ; diagnosis ; therapy ; Uterus ; abnormalities

BACKGROUNDThe pregnancy outcome of the unicornuate uterus is associated with an increased risk of miscarriage, cervical incompetence, and a number of obstetric complications. However, at present, there is no accepted treatment method for women with unicornuate uterus, other than expectant measures. The aim of this study was to evaluate the reproductive outcome of transcervical uterine incision (TCUI) in patients with unicornuate uterus.

METHODSThirty-three patients with unicornuate uterus presented to our tertiary center for infertility or miscarriage. All 33 patients underwent TCUI and were followed up for 10-52 months. The pregnancy outcomes ( first-trimester miscarriage, second-trimester miscarriage, preterm, term, intrauterine death, ongoing pregnancy, and live birth) before and after TCUI were compared by t- test.

RESULTSAmong 31 patients who attempted to conceive after TCUI, twenty conceived including one termination of pregnancy, one second-trimester miscarriage, one ectopic pregnancy, five preterm deliveries, 11 term delivery, and one ongoing pregnancy. There were 16 live births in total. There was significant reduction in the first-trimester miscarriage rate (t = 4.890; P< 0.001), increase in term delivery (t = -3.288; P = 0.002), and live birth rates (t = -4.073; P< 0.001) after TCUI.

CONCLUSIONTCUI appeared to improve the pregnancy outcome in women with unicornuate uterus presenting with infertility or miscarriage.

Adult ; Female ; Humans ; Hysteroscopy ; Pregnancy ; Pregnancy Complications ; surgery ; Pregnancy Outcome ; Urogenital Abnormalities ; surgery ; Uterus ; abnormalities ; surgery

BACKGROUNDFemale genital malformations represent miscellaneous deviations from normal anatomy. This study aimed to explore the clinical characteristics of patients who underwent surgery for genital tract malformations at Peking Union Medical College Hospital (PUMCH) during a 31-year period.

METHODSWe retrospectively reviewed surgical cases of congenital malformation of the female genital tract at PUMCH for a 31-year period, analyzed the clinical characteristics of 1634 hospitalized patients, and investigated their general condition, diagnosis, and treatment process.

RESULTSThe average patient age was 27.6 ± 9.9 years. The average ages of patients who underwent surgery for uterine malformation and vaginal malformation were 31.9 ± 8.8 years and 24.7 ± 9.0 years, respectively; these ages differed significantly (P < 0.01). Among patients with genital tract malformation, the percentages of vaginal malformation, uterine malformation, vulva malformation, cervical malformation, and other malformations were 43.9%, 43.5%, 7.4%, 2.3%, and 2.8%, respectively. Among patients with uterine malformation, 34.5% underwent surgery for the genital tract malformation, whereas in patients with vaginal malformation, the proportion is 70.6%; the difference between the two groups was statistically significant (P < 0.01). The percentage of complications of the urinary system in patients with vaginal malformations was 10.2%, which was statistically significantly higher than that (5.3%) in patients with uterine malformations (P < 0.01).

CONCLUSIONSCompared to patients with uterine malformations, patients with vaginal malformations displayed more severe clinical symptoms, a younger surgical age, and a greater need for attention, early diagnosis, and treatment. Patients with genital tract malformations, particularly vaginal malformations, tend to have more complications of the urinary system and other malformations than patients with uterine malformations.

Abnormalities, Multiple ; surgery ; Adolescent ; Adult ; China ; Female ; Genitalia, Female ; surgery ; Hospitals ; Humans ; Male ; Retrospective Studies ; Urogenital Abnormalities ; surgery ; Uterus ; abnormalities ; surgery ; Vagina ; surgery ; Young Adult

BACKGROUNDUterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as Herlyn-Werner-Wunderlich syndrome (HWWS). In the literature, the syndrome often appears as a single case report or as a small series. In our study, we reviewed the characteristics of all HWWS patients at Peking Union Medical College Hospital (PUMCH) and suggested a new classification for this syndrome because the clinical characteristics differed significantly between the completely and incompletely obstructed vaginal septum. This new classification allows for earlier diagnosis and treatment.

METHODSFrom January 1986 to March 2013, all diagnosed cases of HWWS at PUMCH were reviewed. A retrospective long-term follow-up study of the clinical presentation, surgical prognosis, and pregnancy outcomes was performed. Statistical analyses were performed using SPSS, version 15.0 (IBM, Armonk, NY, USA). Between-group comparisons were performed using the χ2 test, Fisher's exact test, and the t-test. The significance level for all analyses was set at P < 0.05.

RESULTSThe clinical data from 79 patients with HWWS were analyzed until March 31, 2013. According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or incomplete obstruction of the hemivagina as follows: Classification 1, a completely obstructed hemivagina and Classification 2, an incompletely obstructed hemivagina. The clinical details associated with these two types are distinctly different.

CONCLUSIONSHWWS patients should be differentiated according to these two classifications. The two classifications could be generalized by gynecologists world-wide.

Adolescent ; Child ; Congenital Abnormalities ; classification ; diagnosis ; Female ; Humans ; Male ; Retrospective Studies ; Urogenital Abnormalities ; classification ; diagnosis ; Uterus ; abnormalities ; Vagina ; abnormalities

Ultrasonography in obstetrics is increasingly used for the screening of chromosomal abnormalities as well as for prenatal diagnosis of congenital abnormalities with safety and technological advancements. In the first trimester, it is important to confirm normal intrauterine pregnancy with viability, detect the abnormalities of uterus and adnexa, determine the number of fetuses and assess chorionicity and amnionicity in case of multiple pregnancy. After establishment of gestational age accurately by crown-rump length, thickened fetal nuchal translucency, absence of nasal bone, tricuspid regurgitation, reverse a wave of ductus venosus and cystic hygroma can be markers for screening of chromosomal abnormalities. In addition, the scan also offers an opportunity to detect gross structural abnormalities, which could help improve the prognosis by early prenatal intervention. In the second trimester, aneuploidy (trisomy 21, 18, 13, Turner syndrome) and genetic syndromes could be detected by major structural defects and soft markers. It is important to consider that many malformations may not be detected prenatally even by qualified practitioners and appropriate equipment, and to counsel patients about the potential for false-positive or false-negative results.
Amnion ; Aneuploidy ; Chorion ; Chromosome Aberrations ; Congenital Abnormalities ; Crown-Rump Length ; Female ; Fetus ; Gestational Age ; Humans ; Lymphangioma, Cystic ; Mass Screening* ; Nasal Bone ; Nuchal Translucency Measurement ; Obstetrics ; Pregnancy Trimester, First ; Pregnancy Trimester, Second ; Pregnancy* ; Pregnancy, Multiple ; Prenatal Diagnosis ; Prognosis ; Tricuspid Valve Insufficiency ; Trisomy ; Ultrasonography* ; Ultrasonography, Prenatal ; Uterus

Female ; Humans ; Male ; Urogenital Abnormalities ; classification ; diagnosis ; Uterus ; abnormalities

Female ; Humans ; Male ; Urogenital Abnormalities ; classification ; diagnosis ; Uterus ; abnormalities

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