Single-Stage Uterovaginal Anastomosis: A Surgical Solution for Primary Amenorrhea Due to Vaginal Agenesis: A Case Series.

This study, conducted between 2022 and 2024, involved three patients with functional uteri and vaginal agenesis referred to three university hospitals affiliated with Shiraz University of Medical Sciences (SUMS), Shiraz, Iran. The first author performed all surgeries. Vaginal agenesis/atresia often sees delayed diagnosis due to: low awareness among medical professionals, misdiagnosis as other conditions (appendicitis, ovarian cysts, IBS, dysmenorrhea), and ineffective surgeries. Timely recognition by gynecologists (primary amenorrhea + cyclic pelvic pain in adolescents) and early referral to specialized centers for reconstructive surgery are crucial to prevent long‐term issues such as severe endometriosis, infertility, and pelvic adhesive disease. All our patients presented with primary amenorrhea and worsening cyclic abdominopelvic pain. Physical examination revealed varying degrees of vaginal agenesis/atresia. Transrectal ultrasound and MRI confirmed hematometra, with or without hematocolpos, in all patients. Differential diagnoses for primary amenorrhea with cyclic abdominopelvic pain and suspected vaginal agenesis include imperforate hymen, transverse vaginal septum, müllerian agenesis (Mayer–Rokitansky–Küster–Hauser syndrome) with functioning endometrium variations, cervical agenesis or dysgenesis, vaginal atresia/segmental vaginal agenesis, obstructive uterovaginal anomalies (e.g., noncommunicating uterine horn), hematocolpos/hematometra due to acquired obstruction, androgen insensitivity syndrome (typically absent uterus), and rare outflow obstruction from severe vaginal/vestibular trauma or infection. Key diagnostic tests include pelvic exam, pelvic ultrasound (including transrectal), MRI, and, when indicated, karyotype/hormonal profile.

Elham Askary: data curation, investigation, project administration, supervision, writing – original draft, writing – review and editing. Zahra Parsaiyan: data curation, investigation, writing – review and editing. Nahideh Afshar Zakaria: data curation, investigation, writing – review and editing. Saeed Alborzi: data curation, writing – review and editing. Tahereh Poordast: data curation, writing – review and editing. Shaghayegh Moradi Alamdarlo: data curation, writing – review and editing.

Informed written consent, data on surgery, and medical history were collected prospectively at the time of inclusion. The protocol of the study was according to the Declaration of Helsinki and was approved by the Ethics Committee of Shiraz University of medical sciences, Shiraz, Iran (IR.SUMS.REC.1404.235).

The authors have nothing to report.

Detailed informed consent was obtained from patients and parents, outlining the disease, treatment options, and potential complications such as bladder and rectal injuries, fistula formation, vaginal stenosis, re‐operations, and possible hysterectomy. Patients were followed for 1 year post‐surgery. Under general anesthesia and in the low lithotomy position, following prophylactic antibiotic administration and sterile urinary catheterization, the recto‐vesical space was dissected to create a neovagina. Diagnostic laparoscopy from the umbilical site evaluated the uterine contour, adnexa, and released adhesions from retrograde menstruation. This was followed by mini‐laparotomy. Subsequently, the bladder serosa was mobilized, a small Kehr incision was made on the lower uterine segment, and hematometra and hematocolpos were drained. A Hegar dilator (No. 9) was passed from the uterus into the neovagina. Upon meeting the dilator, a pull‐through vaginovaginal/vaginointroitus anastomosis was performed. A 30 French Nelaton rectal tube was secured with Vicryl No. 1 to the uterine cavity, placed through the vagina, and surrounded by an amnion graft, remaining in situ until spontaneous expulsion. Finally, a Jackson‐Pratt drain was placed in the posterior cul‐de‐sac (Figures  1 and 2 ). Illustration of side view of female pelvis. Before operation‐ agenesis of vaginal mid part: Hematometra and hematocolpos are obvious. Illustration of side view of female pelvis. After operation‐anastomosis was done vaginally and rectal tube coated by amnion graft was inserted and fixed to endometrial line and vagina. Patients were discharged after 3–7 days, depending on their condition. Ambulation began once stable, and the urinary catheter was removed on the first post‐operative morning. Diet resumed after 8 h. Oral cefixime (400 mg daily) was administered until rectal tube expulsion. Vaginal molds were then constructed, and patients were instructed on their continuous use for 3 months, followed by nightly use for an additional 6 months. Follow‐up appointments were scheduled weekly for 4 weeks, then biweekly for 4 weeks, monthly for 6 months, and finally at 6 months post‐last monthly follow‐up. During follow‐up, patients were assessed for dysmenorrhea, menstrual cycle patterns, spotting, and abnormal vaginal discharges. Vaginal examination and ultrasound were performed to detect any vaginal stenosis (Table  1 ). Patients' demographics data.

The mean age at operation was 11.7 years (range 11–12 years). The average BMI was 19.2 kg/m 2 (range 19–19.5). The average duration from symptom onset to operation was 3 months (range 3–5 months). Patients exhibited 2/3 distal agenesis; all had a functional uterus with a cervix (Table  2 ). Intraoperative and postoperative findings.

Vaginal agenesis, a rare congenital anomaly, necessitates individualized surgical intervention to restore anatomical and functional integrity. Our study evaluated seven patients with varying degrees of vaginal agenesis who underwent single‐stage uterovaginal/vaginovaginal anastomosis using a rectal tube and amnion graft. This approach effectively restored menstrual function and preserved fertility with minimal complications. A key challenge in managing vaginal agenesis with a functional uterus is preventing retrograde menstruation and its associated complications (hematometra, hematocolpos, and endometriosis). Previous studies often considered hysterectomy a definitive solution, or suggested uterine aspiration and continuous oral contraceptives, which could lead to failed menstruation and irreversible fertility loss [ 8 ]. In contrast, our technique aimed to preserve reproductive potential while ensuring a functional neovagina. The overall success rate in our cohort was high, with all of patients achieving regular, pain‐free menstruation without abnormal discharge during follow‐up. This highlights the critical importance of strict adherence to post‐operative vaginal dilation protocols [ 9 ]. While other reconstructive techniques like the McIndoe procedure, Vecchietti technique, and bowel segment interposition have shown success, they often involve multiple surgical stages, extended recovery times, or complications such as scarring, neovaginal contraction, and uncertain fertility outcomes [ 10 ]. Our single‐stage approach offers an effective alternative, utilizing the natural epithelialization potential of the amnion graft, which reduces fibrosis and promotes epithelial healing [ 11 ]. Endometriosis is another important consideration in these patients, well‐documented in Müllerian anomalies with retrograde menstruation as a hypothesized cause [ 12 ]. By ensuring adequate drainage and anatomical restoration, our approach may mitigate this risk, though long‐term studies are needed for confirmation. Intraoperatively, our results demonstrated low morbidity, with no significant blood loss or organ injury, unlike prior reports of bladder and rectal injuries. Meticulous dissection and careful rectal tube placement were crucial in preventing such complications [ 13 ]. Despite promising outcomes, limitations include a small sample size, limiting generalizability, and a follow‐up period insufficient to evaluate long‐term reproductive outcomes, such as future pregnancies or potential cervical insufficiency. Future studies should include larger cohorts and extended follow‐up to assess reproductive potential and sexual function.

Written informed consent was obtained from all patients for publication of clinical details and images. A copy of the written consent is available for review by the editor of the journal.

Vaginal agenesis affects approximately 1 in 4000–10,000 females. While most cases involve cervical or uterine agenesis, a smaller percentage (7%–10%) exhibit varying degrees of Müllerian development [ 1 , 2 ]. In 2%–7% of cases, individuals may have functional endometrial tissue despite an absent vaginal canal. This obstructive disorder can lead to complications such as hematocolpos, hematometra, hematosalpinx, and endometriosis. Patients typically present with worsening cyclic abdominopelvic pain, amenorrhea, and sometimes a pelvic mass. Accurate mapping of Müllerian duct anomalies is crucial to avoid unnecessary or incorrect surgeries that can lead to extensive scar tissue, complicating future reconstructive procedures [ 3 , 4 ]. Age, religious, and cultural factors are important considerations for diagnosis and patient counseling, especially for adolescents undergoing vaginal surgeries who may have limited compliance and understanding of their condition. Comprehensive information about postoperative instructions is essential for optimal outcomes. Managing vaginal atresia requires a multidisciplinary approach addressing physical, psychological, and emotional well‐being. Improved surgical techniques and a deeper understanding of the condition's underlying biology can enhance outcomes and quality of life. Previously, hysterectomy was performed to prevent complications from retrograde menstruation and obstruction. However, this raises ethical and emotional concerns due to the irreversible loss of fertility [ 5 ]. The preferred surgical approach is single‐stage uterovaginal anastomosis, aiming to relieve symptoms, restore normal anatomy, and preserve fertility [ 6 ]. Various lining materials, such as skin grafts (e.g., pudendal thigh grafts), amnion, peritoneum, allogenic epidermal sheets, and Interceed, can be used for vaginoplasty [ 7 ]. This report presents the follow‐up of three cases of mid‐vaginal atresia treated with single‐stage uterovaginal anastomosis, utilizing rectal tube insertion in the uterus for drainage and to maintain the anastomosis, alongside an amnion graft for vaginoplasty.

The authors declare no conflicts of interest.