A case of 17 year-old nulligravid who initially presented with a 3 year history of cyclic hypogastric pain with gradual abdominal enlargement. She was assessed with Imperforate Hymen and underwent hymenectomy and ultrasound-guided aspiration of blood clots per vagina. No vaginal canal was noted. Patient was subsequently lost to follow up.

Patient returned due to recurrence of hypogastric pain and enlargement of abdomen. Transrectal ultrasound revealed hematocolpos with hematometra. Assessment was Cervical and Vaginal Agenesis. Patient underwent exploratory laparotomy, total abdominal hysterectomy with bilateral salpingectomy.

According to American Fertility Society, there is no classification for a complex mullerian malformation such as mixed vaginal and cervical Agenesis. There is no reported case of mixed vaginal and cervical agenesis in the Philippines.

The best surgical management is still controversial. Conservative surgical method should be considered. A rare case of mixed vaginal and cervical agenesis is being presented where the management is a dilemma.

Human ; Female ; Adolescent ; Cervix Uteri-abnormalities ; Vagina-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

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

Non-surgical vaginal dilation is a safe and effective method for the creation of neovagina in the patient with vaginal agenesis. Compared to surgical methods, non-surgical vaginal dilation has the advantage of low morbidity, the creation of a more physiologic vaginal milieu, and no surgical scarring. To overcome some technical limitations of original Frank's method, in 1981 Ingram proposed a modification of the technique that used dilators of gradually increasing size mounted on a bicycle seat stool. Although several studies have shown satisfactory outcomes using Ingram's method, there are some practical difficulties in making and handling the bicycle seat stool. This article reports a case of a 24-year-old woman with Mayer-Rokitansky-Kster-Hauser syndrome whose vaginal agenesis is successfully treated with a simplified version of Ingram's method. This method uses dilators of gradually increasing size mounted on an ordinary chair instead of a bicycle seat stool. When necessary, the patient may use a fulcrum under the dilator.
Vagina/*abnormalities/pathology ; Humans ; Gynecology/instrumentation/methods ; Female ; Adult ; Abnormalities/therapy

Non-surgical vaginal dilation is a safe and effective method for the creation of neovagina in the patient with vaginal agenesis. Compared to surgical methods, non-surgical vaginal dilation has the advantage of low morbidity, the creation of a more physiologic vaginal milieu, and no surgical scarring. To overcome some technical limitations of original Frank's method, in 1981 Ingram proposed a modification of the technique that used dilators of gradually increasing size mounted on a bicycle seat stool. Although several studies have shown satisfactory outcomes using Ingram's method, there are some practical difficulties in making and handling the bicycle seat stool. This article reports a case of a 24-year-old woman with Mayer-Rokitansky-Kster-Hauser syndrome whose vaginal agenesis is successfully treated with a simplified version of Ingram's method. This method uses dilators of gradually increasing size mounted on an ordinary chair instead of a bicycle seat stool. When necessary, the patient may use a fulcrum under the dilator.
Vagina/*abnormalities/pathology ; Humans ; Gynecology/instrumentation/methods ; Female ; Adult ; Abnormalities/therapy

OBJECTIVETo describe the magnetic resonance (MR) imaging manifestations in vaginal anomalies, and to discuss the utility and limit of MR imaging in diagnosis of this abnormality.

METHODSNine female patients with congenital vaginal anomalies who required surgical intervention were studied. MR imaging and ultrasound of the pelvic was performed before surgery. MR imaging features were retrospectively analyzed correlating with surgery.

RESULTSThe vaginal anomalies were categorized into three groups: (1) congenital absence of the vagina and uterus, i.e. Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome) (n=3); (2) vaginal atresia (n=2); and (3) transverse vaginal septum (n=2). There was also one case of absence of vaginal with complicated urinogenital abnormality, and 1 case of obstructed hymen.

CONCLUSIONSMRI is a reliable method for evaluating vaginal anomalies. An accurate MRI examination can be helpful for surgical management.

Abnormalities, Multiple ; diagnosis ; Adolescent ; Adult ; Female ; Humans ; Hymen ; abnormalities ; Magnetic Resonance Imaging ; Retrospective Studies ; Urogenital Abnormalities ; diagnosis ; Uterus ; abnormalities ; Vagina ; abnormalities

Abnormalities, Multiple ; surgery ; Adolescent ; Child ; Female ; Humans ; Kidney ; abnormalities ; Kidney Diseases ; Retrospective Studies ; Uterus ; abnormalities ; surgery ; Vagina ; abnormalities ; surgery ; Young Adult

We present a case of vaginal ectopic ureter with ipsilateral partial duplication of the upper ureter (Y-type ureter), ipsilateral hypoplastic pelvic kidney and bicornuate uterus in a 20-year-old woman who presented with mild urinary incontinence since infancy. Ultrasonography, computed tomography and intravenous pyelography examination showed a left kidney with no evidence of a right kidney. Cystourethroscopy showed absence of the right hemitrigone. Magnetic resonance (MR) urography demonstrated the presence of a bicornuate uterus, an ectopic dysplastic right kidney in the pelvic cavity, and a right ureter that terminates in the vaginae fornix. The patient underwent right nephroureterectomy and urinary continence was restored completely. Although congenital malformations of the urinary tract are frequently associated with genital tract abnormalities, to best our knowledge, this is the first report of the coexistence of all of these anomalies in an individual. Our report also highlights the importance of MR urography in the diagnosis of such rare and complex anomalies.
Female ; Humans ; Kidney ; abnormalities ; pathology ; Kidney Diseases ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Ureter ; abnormalities ; pathology ; Urinary Incontinence ; etiology ; Uterus ; abnormalities ; pathology ; Vagina ; abnormalities ; pathology ; Young Adult

OBJECTIVETo evaluate a pudendal-thigh island flap for vaginal reconstruction.

METHODSForty-seven patients with congenital absence of vagina were undergoing the treatment. Based on the pedicle including the posterior labial neurovascular bundle, a pudendal-thigh island flap was designed and raised in the groin crease just lateral to the labia majora under the deep fascia. It was then transferred to the tunnel between the urethra and the anus for reconstruction of the vagina.

RESULTSFrom May of 1993 to July of 2001, 47 patients were successfully treated for the vaginal reconstruction with the flap. The results were satisfactory without complications.

CONCLUSIONThe above mentioned technique could be a safe and effect method for vaginal reconstruction with the advantages of reliable blood supply, good sensation and few complications. The areaes with the bilateral pudendal-thigh could be large enongh for the vaginal reconstruction without problem of the donor closure.

Fasciotomy ; Female ; Groin ; Gynecologic Surgical Procedures ; methods ; Humans ; Male ; Surgical Flaps ; Thigh ; Urethra ; Vagina ; abnormalities ; surgery ; Vulva ; abnormalities ; surgery