Herein, polyorchidism possessing of more than the usual number of testicles, which was found in two patients, is reported. Both patients had three testes, and one presented with painful swelling of the right scrotum caused by torsion of the testis and the other revealed a non-palpable testis in the right hemiscrotum. Both patients were managed surgically with orchiopexy and an orchiectomy, respectively, followed by the insertion of an artificial testis.
Humans ; Orchiectomy ; Orchiopexy ; Scrotum ; Testis ; Urogenital Abnormalities

Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct anomaly. This case series shows a wide spectrum of the condition, one presenting in an adolescent, managed conservatively and the other in the perimenopausal age group given a more definitive management. The first case is an 18-year- old nulligravid who manifested with progressive dysmenorrhea and foul smelling vaginal discharge a few years after menarche. She subsequently underwent vaginal septotomy followed by diagnostic hysteroscopy. On the other hand, the second case is a 46- year-old nulligravid whose chief complaint is a foul smelling vaginal discharge and consequently went through a total abdominal hysterectomy with salpingo-oophorectomy. To our knowledge, the second case is the only patient diagnosed in the perimenopausal stage and underwent a total hysterectomy. Ultrasound is the first-line imaging modality used in both cases and its merits are highlighted to prove its importance and diagnostic value in the workup of this condition.
Mullerian aplasia ; Mullerian Ducts ; Urogenital Abnormalities

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

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

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

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Excision of the obstructed vaginal septum is the treatment of choice for symptom relief and the preservation of reproductive capability. A 14-yr-old girl complained of persistent vaginal spotting following each menstruation. Pelvic magnetic resonance imaging revealed a uterus didelphys with left hematocolpos and ipsilateral renal agenesis. Instead of conventional transvaginal excision of the vaginal septum, we used hysteroscopic excision under transabdominal ultrasonographic guidance to preserve the integrity of the hymen. The postoperative course was uneventful, and clinical symptoms were completely resolved after this intervention. Resectoscopic excision of the vaginal septum was found to be easy, safe, effective, and appropriate for young women as it preserved hymen integrity. We believe that this is the first Korean report on the use of a hysteroscopy for vaginal septum resection in a patient with uterus didelphys with obstructed hemivagina.
Adolescent ; Female ; Humans ; Hysteroscopy/*methods ; Kidney/abnormalities ; Urogenital Abnormalities/surgery ; Uterus/*abnormalities ; Vagina/*abnormalities/*surgery/ultrasonography

Uterine torsion, which most often occurs during pregnancy, is rare. Torsion of a nongravid uterus is extremely rare and is associated with pelvic adhesions, uterine leiomyomas, or uterine anomalies. We experienced a case of uterine torsion, with a huge subserosal leiomyoma and multiple intramural leiomyomas, in a postmenopausal woman, one that showed the most extreme degree of rotation among the known cases of uterine torsion. Necrosis of the uterus and both adnexae was observed. A total abdominal hysterectomy with a bilateral salpingo-oophorectomy was performed. We present this case with a brief review of the relevant literature.
Female ; Humans ; Hysterectomy ; Leiomyoma ; Necrosis ; Postmenopause ; Pregnancy ; Urogenital Abnormalities ; Uterus

Cloacal malformations are characterized by the confluence of the lower urinary tract, the female reproductive tract, and the rectum to create a common channel with a single opening on the perineum. The presence of a cloaca is a normal phase of early human embryological development. Between the 4^th and 7^th weeks of gestation, the cloaca undergoes subdivision to form the hindgut and urogenital sinus. Failure of this process results in the congenital anomaly termed persistent cloaca (PC). The term urorectal septum malformation sequence (URSMS) is also used to describe this anomaly. The classic description of this process which is still cited in many standard textbooks dates from the 19^th century. However, this has been increasingly called into question by the findings of studies using modern scientific methodology. Urogenital sinus anomalies are defined by the confluence of the urethra and vagina to form a common channel of varying length with a single perineal opening. In this condition, the anorectal canal opens separately on the perineum. The presence of a urogenital sinus represents a transient phase of the normal development of the lower genital tract in the female fetus. However, the form of urogenital sinus most commonly encountered in the developed world is a feature of disordered sexual differentiation and does not arise simply from the persistence of the anatomical structure which is a feature of normal fetal development.
Cloaca/embryology* ; Female ; Humans ; Urogenital Abnormalities/embryology* ; Vagina/embryology*

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

Blepharophimosis ; diagnosis ; genetics ; Humans ; Male ; Middle Aged ; Pedigree ; Phenotype ; Skin Abnormalities ; diagnosis ; genetics ; Urogenital Abnormalities