Various methods for vaginoplasty exist, but the choice depends on anatomy and body size. Conventional vaginoplasty, when performed with bowel segments rather than prosthetic materials, allows modification of canal length but lacks precise control of diameter, rendering it less suitable for pediatric patients and for those with small pelvic cavities. Moreover, the positional traction sometimes necessary under these conditions may compromise mesenteric perfusion and heighten the risk of ischemic complications. This report introduces a novel technique using the Yang-Monti tube principle with end-to-end anastomosis. A 12-year-old girl with type II Mayer-Rokitansky-Küster-Haüser syndrome (MRKH), left hydronephrosis, and hematometra was admitted with abdominal pain. Emergency drainage resolved her symptoms, and imaging revealed a unicornuate uterus, cervical atresia, and vaginal agenesis. A 7 cm sigmoid colon segment was used to create a vaginal canal, connecting the cervix and vaginal vestibular mucosa using the Yang-Monti method. The postoperative course was uneventful; the stent drain was removed after two weeks, and she was discharged on the 23rd day after learning self-care with a Nelaton catheter. Four years post-surgery, she remains healthy, with no complications such as infection or dysmenorrhea. This approach is a useful option for vaginal agenesis with cervical atresia in adolescents with a small pelvis.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome encompasses a range of Müllerian duct anomalies characterized by congenital absence of the uterus and the upper two-thirds of the vagina in young women who otherwise exhibit normal endocrine function and a 46,XX karyotype. MRKH syndrome can occur in an isolated form (type I) or in association with other congenital anomalies (type II or MURCS association), which may include renal, vertebral, auditory, and cardiac defects. It represents one of the most frequent causes of primary amenorrhea, affecting approximately 1 in every 4000-5000 women. MRKH syndrome often remains undiagnosed until a patient presents with primary amenorrhea, despite normal development of secondary sexual characteristics. Both genetic and non-genetic factors have been proposed as contributing to abnormal embryonic development, although the exact etiopathogenesis remains unclear. Imaging plays a key role in the evaluation of genital tract anomalies, allowing non-invasive and comprehensive assessment. Alongside physical examination and pelvic ultrasound, pelvic MRI is essential for identifying the presence of rudimentary uterine tissue. MRKH syndrome can have profound and lasting psychological impacts, making it essential for patients and their families to receive counseling both before and throughout treatment. A range of therapeutic options-both surgical and non-surgical-have been proposed for managing MRKH syndrome. Vaginal dilation remains the first-line treatment, as it offers high success rates with minimal risk of complications. Vaginoplasty is considered a second-line option for patients who do not respond to dilation therapy. Additionally, uterine transplantation and gestational surrogacy provide opportunities for women with MRKH syndrome to achieve biological motherhood. This review provides an updated overview of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, encompassing its etiological, clinical, diagnostic, psychological, therapeutic, and reproductive aspects. We also present a case involving a 19-year-old woman with MRKH syndrome who presented with primary amenorrhea, highlighting the crucial role and advantages of MRI in diagnosis, differential assessment, and treatment planning.

To describe the step-by-step simultaneous vaginal and laparoscopic approach for the creation and reconstruction of a neovagina using a skin graft in a woman affected by Mayer-Rokitansky-Küster-Hauser syndrome [1]. To date, no data are available in the literature comparing our technique with other validated approaches such as McIndoe and Davydov, as our process is a newly developed one. Tertiary-referral center. An eighteen-year-old woman affected by type 1 Mayer-Rokitansky-Küster-Hauser syndrome previously treated with vaginal dilators without success attracted our attention because of her desire to treat her vaginal agenesis. The patient report primary amenorrhea and appeared with normal secondary sexual characteristics. Blind vaginal pouch was confirmed via imaging [2]. After having an accurate counseling with the patient, using drawings on the thigh, informing her about the postoperative course, and showing aesthetic outcomes, an innovative surgery, resulting from a combination of vaginal and laparoscopic approaches, was proposed. The surgical procedure consisted of 2 phases. The vaginal step involved the creation of the lower part of the neovagina using the Fortunoff technique, whereas the laparoscopic step enabled the creation of the upper part under direct visual control. The neovagina was reconstructed using a skin graft harvested from the patient's thigh. The combination of techniques enabled the development of a functional vaginal canal with excellent anatomical and clinical outcomes. At three-month follow-up, the neovagina appeared fully epithelialized, with a total length of 8 cm and satisfactory anatomical and functional results [3-5]. In conclusion, this is the first reported case of a technique combining a vaginal approach for creating the lower canal of the neovagina with a laparoscopic approach for the upper part. We are committed to increasing our case series so that our surgical approach may be considered a viable option to offer surgeons in the future. VIDEO ABSTRACT.

Müllerian agenesis, also known as vaginal agenesis, Müllerian aplasia, or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a rare developmental disorder affecting one in 5000 female births, resulting from an error in Müllerian duct fusion during fetal development. We present a clinical case of a 46-year-old female patient, virgo intacta, with Müllerian agenesis and a leiomyoma arising from remnant uterine buds. The patient presented with primary amenorrhoea, continuous lower abdominal pain, and abdominal distension. We describe the diagnostic process and treatment strategy. MRKH syndrome is the leading cause of vaginal agenesis, often accompanied by uterine and cervical aplasia. It is categorized into Type 1, which involves symmetric underdevelopment of the Müllerian ducts, and Type 2, characterized by asymmetric malformations of the genital tract and other congenital anomalies. Leiomyomas arising from remnant uterine tissue in MRKH patients are extremely rare. Accurate diagnosis and a carefully planned treatment strategy are essential for managing patients with MRKH syndrome complicated by rare tumors. In this case, total tumor extirpation with bilateral adnexectomy was the preferred treatment, based on the patient's clinical presentation and diagnosis.

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital abnormality of sexual development characterized by agenesis of the uterus and vagina. It may be confined to the genital tract (MRKH type I) or be accompanied by abnormalities of the urinary or skeletal systems (MRKH type II). We report a case of an 8-year-old girl who was admitted to the hospital because of a "reducible mass in the left groin." Upon examination, a mass of about 3 × 2 × 2 cm3 in size was found in the left inguinal region on increasing abdominal pressure, and a left inguinal oblique hernia was diagnosed. The vulva developed normally, and the vaginal vestibule had only a urethral opening but no vaginal opening; abdominal ultrasound revealed that the right kidney was absent, and the left kidney seemed to be fused by two renal cavities; ultrasound of the uterine appendages showed no uterus, and the left and right ovaries were normal; thus, the patient was diagnosed with MRKH type II syndrome. Laparoscopic examination verified the above findings, and it was also found that no obvious fallopian tubes were found around both ovaries. High ligation of the left inguinal hernia was performed to treat the inguinal oblique hernia. When a patient with MRKH syndrome presents with an unexplained inguinal mass, an ovarian or uterine inguinal hernia should be suspected. Early detection of this anomaly will aid in reproductive outcome management and appropriate surgical management. A rare case of a young girl born without a uterus and vagina, with a hernia and kidney abnormalities This report describes a rare medical case in an 8-year-old girl who was born without a uterus and vagina, a condition known as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. She came to the hospital because of a soft, moveable lump in her left groin. Doctors found it was caused by a hernia, where tissue pushes through a weak spot in the belly muscles. Further examinations showed that while her outer genital area looked normal, she did not have a vaginal opening. Imaging tests also showed she had no uterus, her right kidney was missing, and her left kidney appeared unusual—likely formed by two kidney parts fused together. Her ovaries were normal, but doctors could not find her fallopian tubes. She was diagnosed with MRKH type II, which not only affects the reproductive system but also includes kidney and sometimes skeletal problems. Surgery was done to fix her hernia, and her condition will need long-term follow-up, especially as she grows older. This case shows how important it is to carefully check for reproductive and kidney issues in young girls with unusual groin masses or genital differences. Early diagnosis can help with treatment planning and support for future fertility and emotional well-being.