The exact incidence of cervical cancer occurring in patients with Müllerian anomalies is unknown as both conditions are relatively rare and the literature is compromised principally of individual case reports or small series. Despite the rarity of this coexistence, the literature consistently highlights the significant diagnostic and therapeutic challenges it presents. This is particularly true when cervical duplication is present as the altered anatomy can hinder adequate cytologic sampling and delay the detection of malignant lesions. We report a case of unilateral invasive endocervical adenocarcinoma in a 43-year-old patient with complete septate uterus and longitudinal vaginal septum. The tumor was confined to the left cervix, staged as FIGO IB2 and underwent a type C1 radical hysterectomy with bilateral salpingo-oophorectomy and pelvic and para-aortic lymphadenectomy. Following the histologic confirmation of a well-differentiated adenocarcinoma with lymphovascular invasion and pelvic nodal metastasis the patient underwent chemoradiation and remains disease-free at five years. This case highlights how cervical duplication, particularly in the presence of a longitudinal vaginal septum may compromise routine screening and underscores the importance of careful anatomic assessment to ensure accurate diagnosis and individualized management in patients with uncommon Müllerian variants.

To evaluate quality of life in pediatric patients with Müllerian anomalies who underwent surgical intervention between 2018 and 2023. A retrospective descriptive case series was conducted including patients younger than 18 years who underwent surgical correction for Müllerian anomalies between 2018 and 2023. Quality of life was assessed using the validated Spanish version of the Pediatric Quality of Life Inventory (PedsQL 4.0®) through telephone follow-up at least six months after surgery. The most frequent anomalies were longitudinal vaginal septum and uterus didelphys (42.8%), while OHVIRA syndrome was least common (2.9%). Renal agenesis occurred in 28.6% of patients, predominantly right-sided (80%), and anorectal malformations (ARM) were associated in 57.1%. All underwent vaginoscopy and surgery, most commonly vaginal septum resection (40%). The PedsQL was applied by phone to all patients, with a mean follow-up of 48 months. Mean quality-of-life scores were good and comparable between parent-proxy and self-reports in the 13-18-year (73.34 vs. 77.51) and 8-12-year (68.75 vs. 72.82) groups. The highest scores were in the 2-4-year (80.43) and 5-7-year (79.71) groups. Emotional functioning consistently scored lowest, while physical and social functioning were highest. Currently, 25.7% of patients have initiated sexual activity, and two have had children-one by vaginal delivery and one by cesarean. Pediatric patients with Müllerian anomalies demonstrated good postoperative quality of life, highlighting the positive impact of timely surgical management on physical, social, and reproductive outcomes.

Congenital anomalies of the female genital tract, such as vaginal septa, arise from disruptions in Müllerian ducts and urogenital sinus development. Vaginal septa, including longitudinal and transverse types, are rare and often remain undiagnosed due to asymptomatic presentation. However, they can lead to significant complications such as dyspareunia, infertility, and obstructed labor. This narrative review aims to provide a detailed overview of the clinical presentation, diagnosis, and management of vaginal septum, with a particular focus on the challenges encountered during pregnancy and labor. A systematic search of PubMed, Web of Science, Scopus, and Embase identified 34 relevant articles, including 16 case reports detailing labor outcomes in women with vaginal septum. The review highlights that timely diagnosis through pelvic examination and imaging, such as magnetic resonance imaging or ultrasound, can prevent labor complications. Management options range from conservative observation in asymptomatic cases to surgical resection, which can be performed intrapartum or before delivery to facilitate vaginal birth. The presence of associated anomalies, including uterine malformations and renal agenesis, underscores the need for thorough evaluation. Postoperative outcomes are generally favorable, though careful surgical planning is essential to avoid complications such as restenosis or infection. Clinicians should remain highly vigilant for women with unexplained infertility, menstrual abnormalities, or labor obstruction. Early identification and individualized management of vaginal septum can optimize obstetric outcomes and preserve reproductive function. Future research should aim to improve early detection methods, refine surgical techniques, and investigate the genetic and embryological basis of these rare anomalies.

In this report we present a new anatomical stratification for vaginal obstruction (hydrocolpos) based on MRI findings while referring the level of obstruction to a fixed bony landmark (the pubic symphysis). This new approach can overcome the limitations of current classifications, which are prone to approximation errors during measurement and fail to account for variations in body mass across different age groups.Data of cases diagnosed with vaginal obstruction were retrospectively analyzed. Cases of cloaca with vaginal obstruction were excluded. MRI confirmed the diagnosis and enabled classification of the level of vaginal obstruction relative to the pubic symphysis in the mid-sagittal plane as low, intermediate, or high-opposite the lower, mid, or upper end of the pubic symphysis, respectively.A total of 13 girls presented with vaginal obstruction during the period 2010 through 2024. Their age ranged between 1 month and 14 years (mean: 54 months; median: 18 months). Three cases presented in the neonatal period with antenatal diagnosis of hydrocolpos, while six cases were referred later during infancy/childhood from other centers. Another group of adolescent girls (four cases) presented with a clinical picture of cryptomenorrhea. The cause of vaginal obstruction was imperforate hymen in one, vaginal atresia in six, persistent urogenital sinus (five cases), and one case of obstructed hemi-vagina. In this series, six cases (46%) had features related to genetic syndromes (Bardet Biedl/ McKusick-Kaufman spectrum). Chronic parenchymatous renal disease was present in three cases among other syndromic features of Bardet Biedl syndrome, in addition to another case with obstructed left hemi-vagina that had absent left kidney (Herlyn-Werner-Wunderlich syndrome). Surgical techniques included simple excision of distal obstructing membrane (four cases), abdominal assisted vaginoplasty (two cases), vaginal pull-through (four cases), simple introitoplasty (one case), urogenital sinus mobilization (one case), and division of longitudinal vaginal septum for a case of obstructed hemi-vagina. Vaginal stenosis or retraction occurred in three cases with intermediate to high-level obstruction-two following abdominal assisted vaginoplasty and one after vaginal pull-through.MRI-based stratification of vaginal obstruction using the pubic symphysis as a reference provides a practical and reproducible approach for surgical planning.This is a case series (level IV evidence).

We describe the first written description of chronic pelvic pain secondary to venous congestion isolated to a longitudinal vaginal septum. A 29-year-old nulliparous female with chronic pelvic pain was diagnosed with a complete longitudinal vaginal septum and complete septate uterus. The patient desired surgical management, and the vaginal septum was excised without complications. Vascular congestion of the septal tissue was noted on pathology, and postoperatively, the patient's pelvic pain improved significantly. Typically, patients with a longitudinal vaginal septum present with pain isolated to vaginal penetration. For atypical presentations like chronic pelvic pain, surgical resection is reasonable to consider with shared patient decision-making.