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

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.
Abdominal Pain ; Adolescent ; Child* ; Diagnosis ; Emergency Service, Hospital ; Female* ; Hematocolpos ; Humans ; Magnetic Resonance Imaging ; Mullerian Ducts ; Wolffian Ducts

Congenital Abnormalities ; pathology ; Endometrial Neoplasms ; pathology ; Female ; Humans ; Kidney ; abnormalities ; pathology ; Kidney Diseases ; congenital ; pathology ; Middle Aged ; Mullerian Ducts ; abnormalities

The Currarino triad is a unique complex of congenital caudal anomalies, including anorectal malformation, sacral bony defect and presacral mass. This triad may be associated with Mullerian duct anomalies, such as duplication of the vagina and uterus. Each of these diseases has a familial tendency and sometimes coexist within families. But, when coexisting in familial cases, nearly all reported cases revealed mutations of the motor neuron and pancreas homeobox 1 (MNX1) gene. Familial cases of Currarino triad combined with Mullerian duct anomaly without MNX1 gene mutation are very rare. Here we report cases of mother and daughter, who had Currarino triad and Mullerian duct anomaly without MNX1 gene mutation, along with a brief literature review.
Genes, Homeobox ; Humans ; Mothers* ; Motor Neurons ; Mullerian Ducts ; Nuclear Family* ; Pancreas ; Uterus ; Vagina

The diagnosis and treatment of a patient with Mullerian duct cyst torsion combined with bladder cancer were retrospectively analyzed. The patient received an open abdominal operation with right accessory resection plus pelvic adhesion release, and conducted microscopic examination for the bladder under general anesthesia. The results of histopathological examination confirmed the diagnosis of Mullerian duct cyst and papillary urothelial carcinoma at low level. After surgery, the patient received chemotherapy and follow-up observation, and all the indices for the patient were normal and no obvious discomfort. Although the final diagnosis of Mullerian duct cysts is based on histopathological examination, ultrasonography, CT scan and MRI can also detect it. Most of the Mullerian duct cysts are benign, and surgical excision is safe and effective.
Carcinoma, Papillary ; Cysts ; Humans ; Magnetic Resonance Imaging ; Mullerian Ducts ; Retrospective Studies ; Tomography, X-Ray Computed ; Urinary Bladder Neoplasms

Ultrasonography (US) is the most recent cross-sectional imaging modality to acquire three-dimensional (3D) capabilities. The reconstruction of volumetric US data for multiplanar display took a significantly longer time to develop in comparison with computed tomography and magnetic resonance imaging. The current equipment for 3D-US is capable of producing high-resolution images in three different planes, including real-time surface-rendered images. The use of 3D-US in gynaecology was accelerated through the development of the endovaginal volume transducer, which allows the automated acquisition of volumetric US data. Although initially considered an adjunct to two-dimensional US, 3D-US is now the imaging modality of choice for the assessment of Mullerian duct anomalies and the location of intrauterine devices.
Gynecology ; Imaging, Three-Dimensional ; Intrauterine Devices ; Magnetic Resonance Imaging ; Mullerian Ducts ; Transducers ; Ultrasonography*

OBJECTIVETo introduce and evaluate the technical feasibility and anatomical and functional outcomes of one-stage vaginoplasty with autologous buccal micromucosa combined with acellular allogenic dermis.

METHODSWe retrospectively reviewed our experiences with 17 patients with Mayer- Rokitansky-Kuster-Hauser syndrome treated with primary surgery from September 2010 to April 2013. All patients underwent vaginoplasty with autologous buccal micromucosa combined with acellular allogenic dermis. We describe the details of this technique, observe the time of epithelization and evaluate the long- term anatomical, functional, and sexual outcomes.

RESULTSThe time of epithelization was 13 d (range: 12-15 d). At a mean follow-up of 15 months (range: 12-24 months), the mean postoperative dependence on the vaginal stent was 11.7 ± 1.64 months (range: 9-15 months), the mean depth of the neovagina was (9.0 ± 0.94) cm (range: 7-11 cm), the mean circumference was (12.3 ± 1.36) cm (range: 10.0-14.5 cm) and the mean volume was (105 ± 10) ml (range 85-120 ml). The mean female sexual function index score of the 12 sexually active patients was 29.5 ± 2.6. No spouse reported discomfort during intercourse.

CONCLUSIONSVaginoplasty with autologous buccal micromucosa combined with acellular allogenic dermis is an effective and feasible approach for patients with Mayer-Rokitansky-Kuster-Hauser syndrome. The procedure has satisfactory long-term anatomical and functional results. The use of the acellular allogenic dermis is limited by the high price and the potential infection.

46, XX Disorders of Sex Development ; surgery ; Acellular Dermis ; Coitus ; Congenital Abnormalities ; surgery ; Feasibility Studies ; Female ; Humans ; Mouth Mucosa ; transplantation ; Mullerian Ducts ; abnormalities ; surgery ; Postoperative Period ; Reconstructive Surgical Procedures ; methods ; Retrospective Studies ; Vagina ; abnormalities ; surgery

Rectovestibular fistula is the most common type of anomaly found in a female newborn with anorectal malformation. However, when the baby is found to have two orifices in the introitus, rectovaginal fistula is much less common and suspected. The rare differential diagnosis of Müllerian agenesis, a condition in which the rectum shifts anteriorly and the vagina is absent, is seldom considered. In many cases, the diagnosis of Müllerian agenesis is made only during definitive anorectoplasty. In view of its impact on management, a proper examination under anaesthesia, imaging studies and a diagnostic laparoscopy may be required to confirm the presence or absence of Müllerian structures in such patients. We herein describe a patient with the rare coexistence of VACTERL association and Müllerian agenesis, and discuss the management of anorectal malformations in female patients with Müllerian agenesis.
Abnormalities, Multiple ; diagnosis ; Anal Canal ; abnormalities ; surgery ; Anorectal Malformations ; Anus, Imperforate ; complications ; diagnosis ; surgery ; Child ; Diagnosis, Differential ; Esophagus ; abnormalities ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Laparoscopy ; Limb Deformities, Congenital ; complications ; Mullerian Ducts ; abnormalities ; Rectal Fistula ; diagnosis ; Rectum ; abnormalities ; surgery ; Spine ; abnormalities ; Trachea ; abnormalities ; Vagina ; abnormalities

Carcinosarcomas of the uterine cervix are extremely rare. Cervical carcinosarcoma can be characterized by having two different origins: the Mullerian ducts and the mesonephric duct remnants. A 53-year-old Korean woman was admitted to the hospital because of pelvic mass detected on computed tomography scan done at private clinic. A Radical hysterectomy with bilateral salpingooophorectomy and pelvic lymphadenectomy was carried out upon a diagnosis of stage IB2 cervical sarcoma. Immunohistochemically, the epithelial component was positive for pancytokeratin and estrogen receptor, but negative for CD 10 and carletinin. The mesenchymal component was positive for vimentin. The histopathologic diagnosis was a carcinosarcoma of the uterine cervix arising from Mullerian ducts. She underwent chemotherapy. She developed systemic recurrence seven months after operation and died of disease. The origin of cervical carcinosarcoma needs to be verified and immunohistochemical studies using mesonephric marker (CD 10, carletinin, and estrogen receptor) is helpful.
Breast Neoplasms ; Carcinosarcoma* ; Cervix Uteri* ; Diagnosis ; Drug Therapy ; Estrogens ; Female ; Humans ; Hysterectomy ; Lymph Node Excision ; Middle Aged ; Mullerian Ducts* ; Recurrence ; Sarcoma ; Vimentin ; Wolffian Ducts

OBJECTIVE: To evaluate the feasibility and efficacy of transurethral electrotomy combined with seminal vesiculoscopy ejaculatory duct dilatation in treating patients with Müllerian duct cyst. 
 METHODS: We retrospectively analyzed 12 infertile men due to Müllerian duct cyst between 2009 and 2012. They were diagnosed by semen analysis (including quantity of semen, pH and fructose), digital rectal examination, transrectal ultrasonography, magnetic resonance images and aspiration biopsy when necessary. All patients were treated by transurethral electrotomy combined with seminal vesiculoscopy ejaculatory duct dilatation and were followed up for 12 months. 
 RESULTS: A significant improvement of semen quality was achieved after surgery and the sperms could be seen. The patients' semen was analyzed for 3 times in 12 months and the results were normal. Semen volume and pure berries of the patients were increased after the operation compared with those before the operation (P<0.05). Spontaneous pregnancies were achieved in 3 patients 9-12 months after surgery. Four patients' seminal vesicle became smaller obviously.
 CONCLUSION Transurethral electrotomy combined with seminal vesiculoscopy ejaculatory duct dilatation is effective and safe for the treatment of Müllerian duct cyst accompanied with ejaculatory duct obstruction.
Cysts ; surgery ; Dilatation ; Ejaculation ; Ejaculatory Ducts ; surgery ; Humans ; Infertility, Male ; pathology ; surgery ; Male ; Mullerian Ducts ; pathology ; surgery ; Retrospective Studies ; Semen ; Semen Analysis ; Seminal Vesicles ; surgery ; Spermatozoa