Surgical correction of vaginal anomalies

Summary of accurate diagnosis and surgical management of vaginal anomalies. Imperforate hymen, transverse septa, and distal vaginal agenesis present similarly with pain and hematocolpos. Likewise, imperforate hymen, transverse septa, distal vaginal agenesis, and Müllerian aplasia may appear similarly on examination. Imaging should be used to better differentiate the anomaly. Although surgical correction is the mainstay of hymenal anomalies, septa, obstructed hemivaginas, and distal vaginal agenesis, first-line therapy for Müllerian aplasia is progressive dilation. Many surgical techniques are also described but no consensus exists as to the best one. Clinicians should be aware of the appropriate evaluation, differential diagnosis, and management options available for vaginal anomalies.     

女性生殖道畸形患者发生泌尿系统畸形的临床特点分析

目的 探讨生殖道畸形患者发生泌尿系统畸形的临床特点、诊断和治疗。方法对我院近4年来收治的生殖道畸形患者的临床资料进行回顾性分析。结果289例生殖道畸形患者,年龄在13—47岁,平均年龄(23.4±2.8)岁。根据生殖道畸形发生的情况分为对称性畸形及不对称性畸形两类。其中对称性生殖道畸形213例,包括子宫完全或不完全纵隔135例,处女膜或阴道闭锁20例,先天性无子宫无阴道58例。对称性生殖道畸形中只有1例合并同侧肾脏缺如,发生率为0.50％(1/213),占总生殖道畸形的0.35％(1/289)。不对称性生殖道畸形76例,包括阴道斜隔综合征59例,其中伴同侧肾脏缺如43例,盆腔异位单肾畸形2例,双侧马蹄肾1例,左侧马蹄肾伴右侧肾代偿性增大1例,双侧各有1对肾盂、肾盏,输尿管中下段合并1例;残角子宫13例及单角子宫4例,均合并同侧肾脏缺如。不对称性生殖道畸形合并泌尿系统畸形发生率为85.53％(65/76),占总生殖道畸形的22.49％(65/289)。结论生殖道畸形尤其是不对称性生殖道畸形合并肾脏畸形并非少见,但其临床表现也各有差异。患者就诊时已为成人期,且多以生殖系统异常症状就诊,因此及早发现生殖系统畸形有重要的临床意义。     

Long-Term Results of an Imperforate Hymen Procedure that Leaves the Hymen Intact

Purpose of the StudyThe aim of this study was to show the clinical results of postoperative evaluation of cases of imperforate hymen that presented at our center during a 21-year period.MethodsA Foley’s catheter was inserted in 74 patients of imperforate hymen who reported to the Department of Obstetrics and Gynecology, Meram Faculty of Medicine, Necmettin Erbakan University, between January 1, 1996, and December 31, 2016 with history of pelvic pain. In each case, the hymen was opened via a circular incision from the central of the distended. A Foley’s catheter was inserted, and estrogen cream was prescribed for application on the hymenal structure for 14 days. The catheter was removed after 14 days.ResultsThe mean age of the patients at the time of this study was 28.3 ± 2.6 years, and the mean age at diagnosis was 13.2 ± 2.5 years. Twenty-nine (96.6%) patients had experienced vaginal bleeding during their first sexual intercourse experience, and one patient (3.4%) had not. Fourteen out of the 30 married women had become pregnant, of whom nine had delivered vaginally and five had delivered via a cesarean section. After undergoing renal ultrasound, none of the patients had any apparent anomalies. Only one patient had a uterine anomaly, which was a bicornuate uterus.ConclusionA circular incision with insertion of Foley’s catheter prevents many social problems by preserving the hymen’s architecture and allowing vaginal bleeding to occur during the first sexual intercourse experience.     

Sexual Experience before Treatment for Vaginal Agenesis: A Retrospective Review of 137 Women

Study ObjectiveTo summarize the self-reported sexual experiences of women with vaginal agenesis before treatment and discuss the clinical implications.DesignA retrospective review of pretreatment baseline sexuality data and medical records of women with vaginal agenesis seeking vaginal construction.SettingA specialist multidisciplinary center for women with genital differences associated with diverse sex development.ParticipantsOne hundred thirty-seven women with untreated vaginal agenesis associated with Mayer-Rokitansky-Küster-Hauser Syndrome and complete androgen insensitivity syndrome aged 15 to 41 years (mean age, 20 years).InterventionsGynecological examination and completion of questionnaires.Main Outcome Measures(1) Sexual Experiences Questionnaire; (2) Multidimensional Sexuality Questionnaire; (3) Vaginal Self-Perceptions; and (4) vaginal length.ResultsA sizable proportion of women reported having had sexually intimate experiences before any medical intervention on the vagina. Vaginal length, which ranged from dimple to 7 cm and averaged 2.7 cm for the cohort, was unrelated to the range of sexual experiences. Most women perceived their vagina as being too small, but less than half believed that a sexual partner would notice this. Two-thirds of the cohort subsequently completed the dilation program, which was not predicted by pretreatment vaginal length or sexual experience.ConclusionContrary to the assumption that a vagina of certain dimensions is a prerequisite for women to “have sex,” many women with Mayer-Rokitansky-Küster-Hauser syndrome and complete androgen insensitivity syndrome reported having experienced genital and nongenital sexual activities with no medical interventions. It is recommended that treatment providers affirm women's capacity for sexual intimacy, relationships, and enjoyment before they introduce the topic of vaginal construction as a non-urgent choice.     

Types of hymen in the newborn infant

The incidence of various types of hymenal shape, its orifice and their interference with spontaneous vaginal discharge were investigated during the routine physical examination of 333 female infants within the first 24 h of life. A smooth hymen with a central orifice was observed in 53.5% of the female neonates, a folded hymen with a central orifice in 27.3%; folded hymen with eccentric orifice in 4.5%; an anterior opening of the hymen in 10.8%; posterior opening in 0.6%; hymenal band in 3%; almost imperforate hymen in 0.3% of the newborns. The presence of anterior opening, posterior opening, hymenal band or almost imperforate hymen may interfere with free vaginal discharge. A ruffled, irregular hymen and a hymenal band require a careful investigation for other genital malformations.     

Abnormalities of external and internal genitalia

The gynecologic examination of the female genitalia of children and young adults belongs in the hands of specialists and should be performed in the appropriate environment. The most important task of such an examination is the distinction between true developmental anomalies and anatomical findings mimicking such anomalies. Even newborn females can be successfully examined without any large effort expenditure. In most cases, the external inspection of the vestibule is enough to reach a diagnosis. In other case scenarios, vaginoscopy and gynecological speculum examination may be necessary. Additional imaging studies include abdominal sonography and magnetic resonance imaging. Normal variations of a child's hymenal membrane, fusion of the labia minora, hymenal polyps and hypertrophy of the preputium of the clitoris are the entities most likely to be mistaken for real developmental malformations. The most important asymptomatic developmental anomaly of the vagina and the uterus is the Mayer-Rokitansky-Kuster-Hauser syndrome (uterine and vaginal agenesis). Developmental malformations, such as hymenal atresia, vaginal septum formation and Millerian anomalies in general, tend to be symptomatic, presenting with hematometra, hematocolpos or dysmenorrhea. The treatment of genital developmental anomalies is generally not difficult but requires the appropriate clinical and surgical expertise.     

Partial Cervical Agenesis and Complete Vaginal Atresia

BackgroundThe objective of this study was to report 2 cases of the combined congenital anomalies of complete vaginal atresia and partial cervical agenesis, and highlight the limitations of magnetic resonance imaging for definitive initial diagnosis, and consequently the importance of early definitive management, to avoid life-threatening sepsis. Herein we provide a retrospective case audit of two patients with congenital abnormalities between 2005 and 2013 who were treated in a quaternary statewide pediatric and adolescent gynecology center.CasesTwo patients with the combined congenital anomalies of complete vaginal agenesis and partial cervical agenesis highlight the difficulties encountered with the limitations of magnetic resonance imaging in accuracy of diagnosis, as well as development of life-threatening sepsis that requires hysterectomy. Both patients were initially imaged as having distended endometrial cavities and cervical canals with what was thought to be an obstructive upper vaginal septum and absent lower vagina. Both required initial neovagina creation, however the cervices were never clinically or surgically visualized.Summary and ConclusionPartial cervical agenesis is a relatively rare form of Müllerian abnormality which, if not diagnosed and definitively treated early, can have significant morbidity and mortality. Although magnetic resonance imaging is the diagnostic imaging gold standard for Müllerian abnormalities, it is important to recognize the limitations of this modality, the potential sequelae of these limitations, and to appreciate the importance of early accurate diagnosis and treatment of this condition. Importantly, if the imaging diagnosis does not completely correlate with the clinical and surgical findings, then a high suspicion of complete or partial cervical agenesis is prudent, because the consequences of nondefinitive early treatment can be life-threatening and potentially fatal.     

Operative Korrektur genitaler Fehlbildungen

Operative treatment of genital developmental anomalies in children and young adults belongs in experienced hands. The most important task of the examination is the distinction between true developmental anomalies versus anatomical findings mimicking such anomalies. Even newborn females can be successfully examined without large effort. In most cases the external inspection of the vestibule is enough to reach a diagnosis. In other cases vaginoscopy and gynecological speculum examination may be necessary. Additional tests include abdominal sonography and magnetic resonance imaging. The entities most likely to be mistaken for real developmental malformations, include fusion of the labia minora, pseudo hymenal atresia, pseudo hypertrophie of the clitoris and the labia minora and pseudo tumors of the external genitalia (ex. urethral karunkle). The most important asymptomatic developmental anomaly of the vagina and the uterus is the Mayer-Rokitansky-Küster-Hauser-syndrome (uterine and vaginal agenesis). Included among the symptomatic obstructive developmental malformations are hymenal atresia, the vaginal septum, and the double uterus/vagina, where occasionally only one side is obstructed. They can lead to a blockage of menstrual blood, to hematocolpos and hematometra. The timing for operative correction is dependent on the diagnosis, the symptoms and the urgency of the operation.     

Quality of Life and Sexual Function of Patients Following Radical Hysterectomy and Vaginal Extension

IntroductionRadical hysterectomy (RH) has negative consequences on sexual function due to a shortened vagina, vaginal dryness, and dyspareunia. Peritoneovaginoplasty aims to extend vagina by vesical peritoneum and anterior rectal wall to improve postoperative sexual function.AimThe aim of this study was to investigate whether vaginal extension can improve sexual function and quality of life and the problem of sexual dysfunction in early-stage cervical cancer survivors (CCSs) in China.MethodsCase-control and questionnaire-based methods were employed. Thirty-one patients who had undergone vaginal extension following RH and 28 patients with matching factors after RH alone were enrolled in the study.Main Outcome MeasuresBoth groups were assessed retrospectively by questionnaires at least 6 months after treatment. The European Organization for Research and Treatment of Cancer Quality-of-Life questionnaire cervical cancer module and the Sexual Function Vaginal Changes Questionnaire are validated measurements for disease- and treatment-specific issues.ResultsVaginal length was 10.03 ± 1.26 cm and 5.92 ± 1.05 cm in study and control group, respectively (P< 0.05). In the study group, 67.7% patients and 64.3% of control group resumed sexual activity at the time of interview, averaging 6 months between treatment and sexual activity. While difficulty emptying bladder, incomplete emptying, and constipation were the most commonly reported symptoms, no significant difference was observed regarding pelvic floor symptoms. Reduced vagina size and shortened vagina was significantly more prominent in the control group, whereas both group presented with hypoactive sexual desire (88.1%), orgasm dysfunction (71.8%), and low enjoyment or relaxation after sex (51.3%).ConclusionShortened vagina was significantly less reported in study group, while no difference was observed in other sex-related dimensions. Vaginal extension does not worsen pelvic floor symptoms. Sexual rehabilitation interventions are of significance and should be paid more attention to the CCSs in China.Ye S, Yang J, Cao D, Zhu L, Lang J, Chuang LT, and Shen K. Quality of life and sexual function of patients following radical hysterectomy and vaginal extension. J Sex Med 2014;11:1334–1342.     

Laparoscopic Sacrocolpopexy With a Mesh for Sigmoid Neovaginal Prolapse: A Case Report

BackgroundCreating a functional neovagina is a practical therapeutic intervention for women with congenital vaginal agenesis and sexual needs. Although the incidence of neovaginal prolapse (NP) is low, it is inconvenient for patients and is a challenging problem for gynecologists.CaseA 32-year-old woman who had undergone transabdominal sigmoid vaginoplasty 10 years previously at another hospital visited our clinic for evaluation and treatment of NP. Gynecological examination showed exstrophy of the vaginal apex, 4 cm beyond the hymen. Laparoscopic sacrocolpopexy was performed using a mesh. The mesh was sutured at the anterior wall and apex of the neovagina and suspended in the anterior sacral region without blood vessels.Summary and ConclusionLaparoscopic sacrocolpopexy might be an effective and safe treatment for NP.     

Septate uterus with double cervices, unilaterally obstructed vaginal septum, and ipsilateral renal agenesis: a rare combination of müllerian and wolffian anomalies complicated by severe endometriosis in an adolescent

We present a previously unreported combination of müllerian and wolffian anomalies of a septate uterus with double cervices, unilaterally obstructed vaginal septum, and ipsilateral renal agenesis; this constellation of findings may offer clues that could modify classic embryologic explanations. In spite of the young age of our patient (15-years old), a chief complaint of malodorous vaginal discharge, and absence of dysmenorrhea or any other symptoms of endometriosis, laparoscopic examination revealed severe endometriosis with dense adhesions, probably as a result of abundant menstrual regurgitation. Laparoscopic resection of endometriotic lesions, adhesiolysis, and vaginoscopic septotomy were successfully performed while preserving hymenal integrity.     

Case Series: Vaginal Rupture Injuries after Sexual Assault in Children and Adolescents

BackgroundVaginal rupture after sexual assault is a rare but life-threatening occurrence requiring prompt recognition and treatment. Herein, we describe four such cases in children. Our purposes are to increase clinicians' awareness of the physical trauma that a sexual abuse victim can suffer and increase recognition that these victims require immediate trauma services.CasesEach patient had obvious hymenal and vaginal lacerations with a vaginal apical rupture injury and secondary acute blood loss. None of the four victims sustained infectious sequelae.Summary and ConclusionProviders should have a low threshold for managing sexual abuse victims as trauma cases when they have obvious hymenal and vaginal lacerations and genital bleeding, proceeding expeditiously to examination using general anesthesia when appropriate.     

Complexities of Müllerian Anatomy in 46XX Cloacal Exstrophy Patients

Study ObjectiveTo characterize Müllerian anatomy in 46,XX cloacal exstrophy patients.DesignRetrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database.SettingTertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland).ParticipantsWe included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy.InterventionsNone.Main Outcome MeasuresMüllerian structures, method of evaluation, management, and sexual activity.ResultsOf our patients, 12.9% (3/31) had no identified abnormalities. Vaginal anatomy was described for 30 patients; 3/30 had vaginal agenesis, 14/30 had a single vagina, and 13/30 had vaginal duplication. Of 14 patients with 1 vagina, 5 had atresia/hypoplasia, and 1 had a lateral displacement. One patient with 2 vaginas also had distal atresia. Of the cervices evaluated, 9/14 were duplicated (2/9 with a solitary vagina), and 19/27 of the uteri were duplicated (6/22 with 1 vagina, 1/22 with no vagina). Five patients required imaging to fully characterize their anatomy, and 7 patients had studies that failed to identify Müllerian structures seen in the operating room or on physical examination. Common reconstructive surgeries included vaginoplasties, incisions of vaginal septa, colporrhaphies, and hysterectomies. Sexual activity was confirmed for 3 patients, 2 of whom had conceived.ConclusionMost female cloacal exstrophy patients exhibit abnormalities of the Müllerian system. Axial imaging and ultrasound are helpful diagnostic adjuncts but do not replace careful physical examination and assessment in the operating room. Further studies of sexual activity and fertility are warranted.     

Coital Incontinence and Vaginal Symptoms and the Relationship to Pelvic Floor Muscle Function in Primiparous Women at 12 Months Postpartum: A Cross-Sectional Study

IntroductionSymptoms related to sexual dysfunction postpartum are scarcely addressed in the literature, and the relationship to pelvic floor muscle (PFM) function is largely unknown.AimsThe aim of this study was to investigate primiparous women 12 months postpartum and study: (i) prevalence and bother of coital incontinence, vaginal symptoms, and sexual matters; and (ii) whether coital incontinence and vaginal symptoms were associated with vaginal resting pressure (VRP), PFM strength, and endurance.MethodsInternational Consultation on Incontinence Modular Questionnaire (ICIQ) sexual matters module and ICIQ-Vaginal Symptoms Questionnaire were used for questions on coital incontinence, vaginal symptoms, and sexual matters, respectively. PFM function was assessed by manometer (Camtech AS, Sandvika, Norway).Main Outcome MeasuresCoital incontinence, vaginal symptoms, and PFM function were the main outcome measures.ResultsOne hundred seventy-seven primiparous women, mean age 28.7 (standard deviation [SD] 4.3) participated. Of the 94% of women having sexual intercourse, coital incontinence was found for 1.2% whereas 34.5% reported at least one vaginal symptom interfering with the sexual life of primiparous women. Of the symptoms investigated, “vagina feels dry,” “vagina feels sore,” and “vagina feels loose or lax” were most prevalent, but the overall impact on the woman's sexual life was minimally bothersome, mean 1.4 out of 10 (SD 2.5). Women reporting “vagina feels loose or lax” had lower VRP, PFM strength, and endurance when compared with women without the symptom.ConclusionsTwelve-month postpartum coital incontinence was rare, whereas the prevalence of vaginal symptoms interfering with sexual life was more common. The large majority of primiparous women in our study had sexual intercourse at 12 months postpartum and the reported overall bother on sexual life was low. Women reporting “vagina feels loose or lax” had lower VRP, PFM strength, and endurance when compared with women without the symptom. Tennfjord MK, Hilde G, Stær-Jensen J, Siafarikas F, Engh ME, and Bø K. Coital incontinence and vaginal symptoms and the relationship to pelvic floor muscle function in primiparous women at 12 months postpartum: A cross-sectional study. J Sex Med 2015;12:994–1003.     

Non-Obstructive Müllerian Anomalies

BackgroundThe nonobstructive group of anatomic variants involving the reproductive tract includes vaginal agenesis as well as the congenital anomalies of the vagina and uterus, occurring without pain during the pubertal years.ObjectiveThe objective is to discuss the non-obstructive morphologic variations in anatomy of the uterus and vagina.DesignSystematic review using the GRADE system.ResultsThese congenital anomalies are not associated with abnormalities of the external genitalia and therefore may be missed on routine physical examination. When these anomalies do cause symptoms they may be as minor as difficulty with menstrual hygiene or more significant such as primary amenorrhea, dyspareunia, recurrent pregnancy loss, and reproductive complications.ConclusionsWomen with non-obstructive reproductive tract anomalies present at various ages due to the asymptomatic nature or late symptom onset of certain conditions. An MRI is the gold standard in evaluation of such conditions to aid in confirming the müllerian variant. Each condition requires careful counseling because obstetric and gynecologic risks and consequences may differ. Treatment is individualized in cases of uterovaginal agenesis with both nonsurgical and surgical options available for neovagina creation. In cases of uterine or vaginal septae, the treatment timing may vary depending on patient history. Finally, in cases of non-obstructive communicating uterine horns, the risk of ectopic pregnancy is high in the remnant horn. Should a pregnancy occur in this small underdeveloped horn, therefore, excision is recommended.     

Delayed Diagnosis of Herlyn-Werner-Wunderlich Syndrome due to Microperforation and Pyocolpos in Obstructed Vaginal Canal

BackgroundTo present a rare anomaly consisting of uterus didelphys, longitudinal vaginal septum, obstructed hemivagina with pyocolpos, fistula to the open vaginal canal, and ipsilateral renal agenesis, referred as Herlyn-Werner-Wunderlich syndrome (HWWS).CaseA 14-year-old girl with recurring purulent vaginal discharge lasting for a few months. Preoperative examination revealed one vaginal canal with one cervical opening on the right side. There was a fistula leading from the obstructed vaginal canal to the left vagina. Intravaginal ultrasound examination demonstrated a longitudinal vaginal septum and a closed pyocolpos on the right side. The longitudinal vaginal septum was excised by way of electrocauterization under direct vision.ConclusionHWWS should be considered in the differential diagnosis in patients with uterus didelphys and unusual symptoms such as pyocolpos and vaginal discharge.     

Herlyn-Werner-Wunderlich Syndrome-Timely Diagnosis is Important to Preserve Fertility

BackgroundHerlyn-Werner-Wunderlich syndrome is an urogenital malformation with uterus didelphys and obstructed hemivagina with ipsilateral renal agenesis. Most of these patients present after the onset of menstruation. We describe two cases diagnosed too late to prevent the complications.CaseThe first patient presented with acute abdomen one year after the onset of menstruation and had salpingectomy due to pyosalpinx. The blind hemivagina was not recognized and she had severe endometriosis. She underwent hysterectomy 8 years later. The second patient presented with foul smelling vaginal discharge when she was 21 years old. She had a simple vaginal septum resection.Summary and ConclusionIn the presence of uterine cavities in a regularly menstruating girl with dysmenorrhea, the presence of both kidneys should be checked. When unilateral renal agenesis and uterus didelphys coexist the first thing that we should remember is to confirm or refute the presence of a blind vagina.     

Hymen Sparing Surgery for Imperforate Hymen: Case Reports and Review of Literature

BackgroundImperforate hymen, with an incidence between 0.1% and 0.05%, is the most common obstructive congenital abnormality of the female genital tract. 'Standard' surgical treatment of imperforate hymen involves hymenectomy after a cruciate, plus, or X-shaped hymenotomy incision.CasesTwo cases with imperforate hymen treated with a simple vertical incision are presented. A few oblique sutures were used to prevent refusion. Postoperative follow up was uneventful.Summary and ConclusionThe importance of the integrity of hymen changes in different cultures and religious groups. Option of a hymen sparing procedure is readily preferred by most of these patients and families. Also preservation of hymenal tissue, hence the perception of 'integrity' of female genitalia, might be an alternative treatment option.     

Unusual mullerian anomalies associated with distal extremity abnormalities

A 13-year-old girl was evaluated with nonfamilial mullerian anomalies consisting of bilateral blind uterine horns, a separate cervical remnant, and total vaginal agenesis. The observed musculoskeletal abnormalities of the distal extremities differed from those usually associated with both nonfamilial mullerian agenesis (Rokitansky-Küster-Hauser syndrome) and the familial syndromes associated with mullerian anomalies. The pattern of mullerian dysgenesis is unusual in that the entire vagina is absent and a cervical remnant separate from the two blind uterine horns is present in the midline in the normal course of the paramesonephric ducts.