Vaginal agenesis

OBJECTIVE: To describe the incidence and management of vaginal stricture after uterus transplants (UTx) in the United States, to propose a grading system to classify stricture severity, and to identify risk factors for stricture formation. DESIGN: Prospective cohort study. SUBJECTS: Recipients undergoing UTx from 2016 to 2023 at Baylor University Medical Center at Dallas, Cleveland Clinic, University of Pennsylvania, and University of Alabama at Birmingham were monitored postoperatively with regular pelvic exams...

Mayer-Rokitansky-Kuster-Hauser syndrome also known as mullerian agenesis is a rare congenital condition in which there is absence of uterus along with upper vagina. Patient usually presents with primary amenorrhea with or without cyclical lower abdominal pain but have normal secondary sexual characters. Modified McIndoe Vaginoplasty with amnion graft is the commonest surgery performed worldwide. A 23 year old girl with normal secondary sexual characters presented with primary amenorrhea with cyclical lower abdominal pain; on examination blind vagina was present...

BACKGROUND: Müllerian agenesis, known as Mayer-Rokitansky-Küster-Hauser syndrome, is characterized by an absent uterus, cervix, and two-thirds proximal vagina (1). To allow sexual intercourse, dilatators-based conservative approaches and Vecchietti vaginoplasty generate progressive traction on the vaginal stump until adequate vaginal size is achieved. Other approaches create the neovagina using mucous/cutaneous, peritoneal, or ileal/sigmoid grafts or cutaneous flaps from the genitalia to fulfill a newly developed space between the bladder and rectum...

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome is a rare complex congenital disorder, presenting with obstructed vagina, uterus didelphys and ipsilateral renal agenesis. Hemivaginal obstruction firstly asymptomatic, leads to symptoms after menarche such as dysmenorrhea, pelvic pain or infertility. CASE PRESENTATION: A 15-year-old patient presenting with few symptoms, transvaginal ultrasound reveals an hematocolpos, we report also typical findings of this disorder on magnetic resonance imaging...

STUDY OBJECTIVE: The diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is often a lengthy process that typically occurs during late adolescence. To support optimized and patient-centered care, this study aimed to investigate how women with MRKH syndrome experience the diagnostic process. METHODS: From January 2021 to March 2021, we conducted in-depth interviews with 18 Danish women (≥25 years) diagnosed with MRKH syndrome. The interviews lasted a median of 92 minutes (range: 67-117)...

Management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome patients is by creating functional neovagina through surgical or nonsurgical route. Surgical repair using minimally invasive technique is a favorable option in creating neovagina. In this study, the patients underwent neovaginoplasty. Clinical follow-ups were done at 3, 6, and 12 months postoperatively. The primary outcomes were anatomic and functional successes; anatomical success was defined as a ≥6 cm-long neovagina that allows for easy introduction of two fingers, and functional success was defined with Female Sexual Function Index FSFI-6 questionnaire score above 19...

Primary amenorrhoea due to Müllerian malformations is rare, with 1 in 4500 cases and 2%-8% of cases presenting as infertility. Obstructive Müllerian anomalies present as hematometra and hematocolpos during puberty. Timely surgical intervention is required to relieve acute pelvic pain and restore functional anatomy. A 15-year-old girl presented to OPD with complaints of severe pain in her lower abdomen and lower back for the last 2-3 weeks, not relieving on medication. She has not attained menarche and has been having cyclical pain and low backache for 7-8 days every month for the last year...

Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage)...

The Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract. It is characterized by a combination of didelphys uterus, unilateral vaginal obstruction, and ipsilateral renal agenesis. MRI imaging is usually used for diagnosis; however, the authors present a case of HWWS diagnosed by ultrasonography (HSG) and hysterosalpingography (HSG) in a 22-year-old lady who has undergone an imaging workup of infertility.

OBJECTIVE: To demonstrate the surgical approach for Müllerian agenesis with bilateral uterine remnants containing functional endometrium. DESIGN: Stepwise demonstration of the technique with narrated video footage. SETTING: Reproductive surgery unit of a tertiary university hospital. PATIENT: An 18-year-old adolescent was admitted to a tertiary university hospital with complaints of primary amenorrhea and cyclic pelvic pain...

UNLABELLED: Herlyn-Werner-Wunderlich syndrome is a rare congenital malformation of the Mullerian ducts characterized by uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis. Commonly, such patients present with pelvic pain, dysmenorrhea following menarche, and an abdominal mass secondary to hematometrocolpos. In this report, a case of a 14-year-old female presented with abdominal pain, back pain and acute urinary retention. She attained menarche at the age of 10 years; however, symptoms of dysmenorrhea only appeared 4 years later...

OBJECTIVE: To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome. STUDY DESIGN: We conducted a retrospective analysis of prospectively collected data. Women affected by Rokitansky syndrome, who underwent neovaginal biopsy after vaginoplasty (McIndoe surgery, intestinal vaginoplasty, Vecchietti's surgery and Davydov surgery) were included. Macroscopic mucosal features were assessed through clinical examination and Shilling test...

INTRODUCTION: OHVIRA syndrome is a rare congenital anomaly of Müllerian duct development characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The primary treatment is surgical excision of the obstructed hemivaginal septum and hematometrial drainage. In recent years, minimally invasive approaches such as hysteroscopic or vaginoscopic septum resection have been reported. Furthermore, we originally developed some novel pneumovaginoscopic gynecologic surgeries for years using a device that consists of a cylinder that fits into the vagina and a lid that mounts multiple ports, allowing the vagina to be dilated with carbon dioxide gas, similar to a single-port laparoscope...

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare and complex congenital anomaly of the genitourinary system characterized by uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. It is the result of the maldevelopment of both the Mullerian and Wolffian ducts. Clinical manifestations of dysmenorrhea, dysuria, and urinary retention appear after menarche due to the accumulation of menstrual blood and distention of the obstructed hemivagina. Diagnosis of this anomaly is often delayed, and the obstructive nature of the condition is missed due to cyclic menstruation from the one canalized hemivagina...

BACKGROUND: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. METHODS: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review...

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis...

PURPOSE: The bowel remains the favored tissue for neo-vaginoplasty in pediatric patients with vaginal agenesis. In 2001, the first description of neo-vaginoplasty using the Yang-Monti technique with a sigmoid double tubular flap was published. We present our experience of neo-vaginoplasty with a single Yang-Monti tube flap and report on the use of different segments of colon. MATERIALS AND METHODS: We conducted a retrospective review of patients who underwent neo-vaginoplasty using bowel single Yang-Monti tube between 2009 and 2021...

OBJECTIVE: To describe and present the results of the use of urogenital mobilization extended by two flaps from the redundant distal part of the urethra in the correction of a severe form of distal vaginal agenesis. METHODS: During the past 4 years (from December 2018 to March 2023), we have performed 2 vaginoplasties using the described procedure. The age of the patients at surgery was 13 years. Two girls presented with primary amenorrhea associated with cyclic pelvic pain evolving over several months...

We present here a case of complex uterine anomaly-obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome...

Congenital vaginal atresia is a rare anomaly of the female genital tract. Many vaginoplasty procedures have been described, but the postoperative risk of vaginal stenosis remains a challenge. We report a case of isolated distal vaginal agenesis in a patient with neurological impairment where the use of an "alternative" dilator was needed. An 11-year-old girl with Down syndrome was admitted to the Emergency Department complaining of pelvic pain. The clinical evaluation showed a hard and painful pelvic mass associated with an imperforate hymen...