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Objective Vaginal or uterine outlet obstruction leads to hematocolpos or hematometra. Detection of the etiology of this entity is important to guide adequate surgical management and thereby avoid complications and to preserve fertility. The aim of this study was to evaluate obstructing vaginal malformations in adolescent girls presenting with hematocolpos or hematometra with MR imaging. Materials and methods A hospital based prospective study was conducted in a tertiary care centre from September 2015 to October 2016. The study included 17 adolescent females who were evaluated with MRI. Result Of 17 adolescent female with vaginal or uterine outflow obstructive anomalies with hematocolpos or hematometra, where 6 patients (35.3%) had HWWS, 6 patients (35.3%) had imperforate hymen, 2 patients (11.8%) had transverse vaginal septum, 1 patient each (5.9%) had cervico-vaginal atresia, unicornuate uterus and communicating rudimentary Uterine horn. MRI revealed hematocolpos in 15 patients (88.2%), hematometra in 13 patients (76.5%), endometriotic ovarian cysts in 6 patients (35.3%) and hematosalpnix in 3 patients (17.6%). Conclusion Early radiological diagnosis of the cause of vaginal or uterine outflow obstruction is important to guide adequate surgical management which if undertaken promptly helps to avoid complications due to reflux from vaginal or uterine outflow obstruction. [ABSTRACT FROM AUTHOR]

Children with anorectal malformations (ARM) constitute a significant group within a pediatric surgery practice. It is important with female cases of anorectal malformations to consider the association of gynecologic anomalies, especially at the time of the definitive repair. However, it is critical to consider the association of such gynecologic anomalies when caring for patients with a cloacal anomaly. If not recognized, an opportunity to diagnose and treat such anomalies may be missed with the possibility of negative implications for future reproductive capacity. With the knowledge of the associated anomalies and long-term sequelae, surgeons can provide better care for girls and important counseling for parents. Knowledge of reproductive related issues in females with cloaca allows the pediatric surgeon an opportunity both, to provide optimal surgical management in infancy, childhood, and into young adulthood and to collaborate medically and surgically with an experienced gynecologist in patients with more complex anatomic variations. Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility. [ABSTRACT FROM AUTHOR]

Transverse vaginal septum found in 1/2100 dan 1/72.000 women. The classical management for transvers vaginal septum was septum excision, with risk for vaginal narrowing and vagina shortening after surgery. We reported four cases underwent interdigitating "Y" flap with no vaginal narrowing or shortening after procedure as alternative surgical technique for better result. The transverse vaginal septums were right behind hymen to 5 cm proximal from hymen (low to mid location). The post-operative evaluation up to eleven months after surgery found no vaginal narrowing or vaginal shortening. A Case with haematosalphing was re-evaluated one month after surgery and found that the haematosalphing has been resolved and no recurrency on 10 months after surgery. Another case with bigger haematosalphing underwent laparotomy salphingostomy concomitantly with "Y" flap procedure and on 11 months evaluation found no recurrent haematosalphing. As the septum was relatively thick and composed of 2 embryonic origins, dividing them into distal flaps and proximal flaps was possible. Interdigitating "Y" flap technique offer better preservation in vaginal length and less constricture as the technique spread the tissue tension evenly. Cases with haematosalphings implies that adequate drainage through vagina help evacuate the haematosalphing and prevent recurrency. However longer-term evaluation shall be performed. Interdigitating "Y" Flap offers satisfying outcome besides short hospital stay (less intra operative bleeding, no need for postoperative vaginal dilatation, preserved vaginal length and vaginal caliber). [ABSTRACT FROM AUTHOR]

Study Objective: To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina.Design: Stepwise demonstration of the technique with narrated video footage.Setting: In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis.Interventions: A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen.Conclusion: Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction. [ABSTRACT FROM AUTHOR]

Abstract: Study Objective: To assess the fertility and obstetric outcome after surgical treatment of complete uterine and vaginal septum. Design: Retrospective study (Canadian Task Force Classification II-2). Setting: Teaching hospital in France. Patients: Twenty-two women who have experienced infertility, pregnancy losses, dyspareunia, or dysmenorrhea. Intervention: Hysteroscopic section of complete uterine septum and resection of longitudinal vaginal septum. Measurements and Main Results: Improvement of dyspareunia or dysmenorrhea and obstetric outcome, focusing on the miscarriage rate, obstetric complications, and the gestational age at delivery were assessed. Overall, 20 women had conceived a total of 37 pregnancies, with 10 and 8 deliveries before and after metroplasty, respectively. Median gestational age at delivery was not significantly different in both groups (36.5 [33–39.5] vs 38.0 weeks’ gestation [35–40], respectively). Preterm delivery occurred in 4 cases (25%) before the surgery and in 3 cases (14%) after (p = .44). The live birth rate was also not significantly different before and after surgery (62.5% and 38%, respectively) (p = .19). There was a decrease of caesarean section and significantly fewer breech deliveries after metroplasty (p = .01). A decrease in the prevalence of dyspareunia or dysmenorrhea was observed after metroplasty in the 19 patients originally displaying these symptoms. No perioperative complications were observed in this series. Conclusion: Resection of vaginal septum and hysteroscopic metroplasty for complete uterine septum with resection of the cervical septum is a safe procedure that may improve dyspareunia and dysmenorrhea when present. Reproductive and obstetric outcomes after this procedure do not appear to be compromised, even though a relatively high miscarriage rate remains after metroplasty, questioning its systematic practice in symptom-free women without any previous obstetric history. [Copyright &y& Elsevier]

Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an imperforate anus with a rectovestibular fistula, may seem straightforward, treated with a posterior sagittal anorectoplasty; however, it is vital to recognize the association with gynecologic anomalies. Girls with the most complex anorectal defect, cloacal malformation, have significant associated urological and gynecologic anomalies, which should be recognized and treated. An opportunity to diagnose and treat such anomalies may be missed in the newborn period or at the definitive surgery, unless the pediatric surgeon is aware of this association. With the knowledge of the associated anomalies and long-term sequelae, surgeons can provide better care for girls and important counseling for parents. Important gynecologic issues to consider may be divided into several categories, such as infancy, puberty, sexual intimacy, and obstetrical concerns. Knowledge of reproductive-related issues in females with ARMs allows the pediatric surgeon and their gynecology colleagues to provide optimal surgical management throughout infancy, childhood, and into young adulthood. Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility. [Copyright &y& Elsevier]

Abstract: The impact of common congenital anomalies of the female genital tract is hugely variable. Some anomalies are asymptomatic chance findings requiring no intervention. Others have a major impact on the potential for sexual activity and fertility. A good knowledge of basic embryology is important to understand the pathogenesis and clinical features of these anomalies. All gynaecologists should be aware of these conditions and possible clinical presentations. Whilst some conditions, such as imperforate hymen require a simple surgical intervention, other more complex anomalies need careful assessment and accurate pre-operative assessment to optimise the long-term outcomes. The contribution of uterine anomalies to subfertility is poorly understood and the role of uterine surgery needs further research. [Copyright &y& Elsevier]

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation. To present 10 patients with OHVIRA treated at the clinical center. To perform a systematic review of OHVIRA case series related to the prevalence of anatomical variants, surgical interventions and endometriosis, and to compare them with our case series. Medical records from 10 OHVIRA patients treated between 2016 and 2020 were retrospectively reviewed. For the systematic review, PubMed and Web of Science were used to search for relevant studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were strictly followed. The most common anatomical variant includes left obstructed hemivagina (50.7%) with isolated hematocolpos or hydrocolpos (55.9%), uterus didelphys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemihysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some subjects required salpingectomy (3.3%) or oophorectomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolution of hydrocolpos. Endometriosis was fortuitously found in 13.6% of the selected cases who underwent laparoscopy or laparotomy. The most common variant of OHVIRA includes isolated hematocolpos and a thick vaginal septum between adjacent hemivaginas. Endometriosis was present in approximately 14% of OHVIRA patients, but this number is probably underestimated. Routine laparoscopy is not required. However, all patients need further monitoring due to a higher risk of endometriosis. Based on the analyzed studies and our case series, vaginal septectomy is a sufficient surgical technique to relieve symptoms and prevent possible complications in most OHVIRA patients. [ABSTRACT FROM AUTHOR]

Abstract: Two patients had a rare uterine anomaly characterized by longitudinal vaginal septum, double cervix, and incomplete septum of the uterus with normal fundus. This report is, to our knowledge, the third in the literature. There are some other works describing a similar anomaly, with a complete uterine septum. These cases challenge the classic unidirectional theory of müllerian development and support an alternative bidirectional hypothesis. After definitive diagnosis, the vaginal and uterine septa were successfully resected. Both patients conceived spontaneously and had an uneventful obstetric outcome. Surgical treatment might be an alternative treatment in the case of infertility or recurrent pregnancy loss in patients with such a rare müllerian anomaly. The hysteroscopic uterine reunification should be the preferable surgical method and can improve the poor reproductive outcome. [Copyright &y& Elsevier]

Anorectal agenesis with rectovaginal fistula are extremely rare type of anorectal malformations (ARM) characterized by the absence of the anus and abnormal connection of the rectum to the vagina. Uterus didelphys and longitudinal vaginal septum are varieties of mullerian anomalies. In this article, we report a case of ARM with rectovaginal fistula associated with uterus diphedus and longitudinal vaginal septum with the surgical management. The clinical and radiological findings and the surgical management were discussed. • Anorectal agenesis with rectovaginal fistula is extremely rare type of ARM, Uterus didelphys and longitudinal vaginal septum are varieties of Mullerian anomalies. • Association of these tow pathologies are a unique case. • The laparoscopic approach seems to be useful in these cases. • Awareness of the genitourinary anomalies in cases of ARM, could eliminate serious future complications during pregnancy and delivery. [ABSTRACT FROM AUTHOR]