Uterine Anomalies Research Articles

Vaginal Synechiae in a Pregnant Woman with Past Surgery of Low Septum Resection: A Case Report

Introduction: Transverse vaginal septum is a rare mullerian anomaly. Patients with a complete transverse vaginal septum are diagnosed during menarche with symptoms of abdominal pain and amenorrhea. Here, we present a patient who underwent septal resection during puberty and was referred in pregnancy due to vaginal synechiae. Case presentation: The patient was a 40-year-old primigravid referred to the maternity department at 39 weeks of gestational age with labor pain and inability to perform a vaginal examination. She had a past surgical history of low vaginal septum at 13 years old. The vagina was completely obstructed. A thorough resection of the synechiae was performed along with a cesarean section to create a drainage path. Conclusion: It is important to consider a pregnant woman's history of Müllerian abnormalities. In these patients, due to the low frequency of intercourse and the conditions of progesterone dominance, there is a possibility of re-stenosis and the imposition of cesarean section. Paying attention to this possible complication and preventing it by using a dilator can be helpful.

Postpartum Ultrasound: An Indispensable Tool in the Labor Ward.

Postpartum hemorrhage can occur because of different conditions. Apart from placental remnants and uterine atony, less common causes include uterine rupture, placental polyps, arteriovenous malformations, and pseudoaneurysms. Ultrasound plays a key role in the precise definition of the underlying pathology, which is necessary to initiate the appropriate therapy. It also helps to depict intraabdominal hematomas and uterine scar dehiscence, 2 conditions that do not necessarily lead to postpartum hemorrhage but are associated with increased maternal morbidity. This article presents 52 ultrasound images together with clinical and histopathologic images to give an overview of both frequent and rare pathologies in the postpartum period.

Surgical management of 12-year-old with complete septate uterus with duplicated cervices and obstructed left hemivagina: A case report

Introduction: When a patient with an obstructed hemivagina presents in childhood, either hormonal suppression or a staged procedure is proposed for management of the obstruction. Case Report: This case report is a 12-year-old patient presenting for evaluation of hematocolpos with a complete septate uterus, duplicate cervices, bilateral kidneys, and obstructed left hemivagina who was surgically managed with a complete vaginal septum resection and hysteroscopic metroplasty in one procedure shortly after diagnosis. Conclusion: This anomaly is not only rare, and the first presented in the literature, but it also importantly demonstrates that if desired by the family after a consideration of benefits and risks, resection of both vaginal and uterine septa can be safely performed in a young post-menarchal patient without the need for a staged procedure later in life.

8577 Gonadal function throughout life in XX and XY patients with a germline WT1 variant - lessons from a cohort of 80 patients

Abstract Disclosure: M. Carré Lecoindre: None. D. Mallet: None. C. Dossier: None. M. Glenisson: None. M. Grapin: None. A. Brac de la Perriere: None. Z. Chakhtoura: None. C. Bouvattier: None. M. Houang: None. C. Pienkowski: None. N. Zaegel: None. R. Rayneau,MD and PhD: None. T. Blanc: None. L. Martinerie: None. The WT1 gene is involved in tissue homeostasis, tumorigenesis, as well as embryonic development of kidneys, bipotential gonads and ovarian determinism. Germline WT1 variants are known to generate kidney and gonadal developmental diseases associated with differences in genital development (DSD). However, the state of gonadal function, its evolution over time, and the impact of WT1 variants on pubertal development and fertility potential in patients have never been studied. A French national, retrospective, multi-center, observational study evaluated gonadal function over time in patients with a germline variant of the WT1 gene, according to XX or XY karyotype and genotype. Among the 80 patients included - the largest cohort to date - 30% had a XX karyotype, 95% of whom were assigned female at birth. Importantly, 40% of them had uterine malformation, which was not known to be associated with WT1 anomalies. Patients with a XY karyotype presented with DSD (59%), typical female genitalia (15%) or typical male genitalia with or without bilateral cryptorchidism (27%). Thirty percent of XX and 87% of XY patients had gonadal dysgenesis defined as gonads with a dysplastic and/or dysmorphic appearance on histology, surgery and/or imaging or biological hormonal markers compatible with gonadal insufficiency. However only 3 patients (4%) developed gonadal tumors to date. Spontaneous puberty was observed in 94% of XX and 60% of XY patients. However, 67% had impaired gonadal function - defined as AMH and/or inhibin B below the threshold for age and/or elevated FSH and/or LH above 10 UI/L - at the age of 12.6 years and 83% at the age of 16.2 years, considering all karyotypes and genotypes. Hormonal treatment had to be initiated in 57% of XY patients with a median age at 14.4 years old. No difference was seen between patients with or without renal failure (p-value = 0,37), nor regarding the timing of renal insufficiency. In addition, increased FSH, LH and decreased AMH or inhibin B plasma levels were observed independently from gonadotoxic treatment exposure in most patients. None of the patient has had children to date.In conclusion, most XX and XY patients with a germline variant of WT1 gene are affected by early gonadal insufficiency of variable severity with potential impact on puberty and fertility. Even though dialysis, immunosuppressors, gonadotoxic chemotherapies, local radiotherapy and gonadal surgery are known to impair gonadal function, the gonadal disease linked to WT1 variants is the principal factor of gonadal impairment. For these reasons, regular follow-up by an endocrinologist appears essential for both XY and XX patients, providing clear information about gonadal function and fertility. Presentation: 6/1/2024

Investigating the complications of frequent pregnancy loss in patients referred to obstetrics and gynecology clinics at King Khalid University Hospital

ABSTRACT Background: Recurrent pregnancy loss (RPL) is a common problem affecting many women. This study aimed to investigate the baseline characteristics of 400 women with RPL and to examine any differences in baseline characteristics of the recruited women with RPL throughout the years. Methods: This study used retrospective data, which included the records of patients with RPL who visited King Khalid University Hospital’s obstetric clinic between 2019 and 2022. The records of 400 patients who met the inclusion criteria were reviewed and analyzed. Results: The mean age of enrolled women was 36.5 ± 5.4 years, and the mean RPL was 3.8 ± 2.5. The study also revealed that there were significant statistical differences in obesity and vitamin D deficiency among women with RPL based on year (P = 0.013 and P = 0.036, respectively), whereas no significant statistical differences were found between women with RPL in terms of age, parity, diabetes, hypothyroidism, hyperprolactinemia, antiphospholipid antibodies (APLs), uterine malformation, or consanguinity. Conclusion: Advanced maternal age, number of previous miscarriages, parity, diabetes, hypothyroidism, APLs, uterine malformations, first-degree consanguinity, and vitamin D deficiency were found to be significant risk factors associated with RPL among women in Saudi Arabia.

Uterus Didelphys with Hematocervix in the Unilateral Obstructed Hemivagina and Ipsilateral Renal Agenesis: A Case Report of Herlyn–Werner–Wunderlich Syndrome

Background: Mullerian duct anomalies include a broad spectrum of genital tract defects that arise from developmental abnormalities of the genital tract. Herlyn–Werner–Wunderlich syndrome (HWWS) refers to the combination of uterus didelphys, blind hemivagina and ipsilateral renal agenesis. In the literature, the syndrome often appears as a few sporadic case reports. Case: We report a case of symptomatic Herlyn–Werner–Wunderlich syndrome diagnosed by transavaginal scan and MRI and successfully treated with a laparoscopic-assisted hysteroscopic vaginal septum resection. The postoperative course was without complications, and clinical symptoms completely resolved the surgery. Summary and Conclusions: Combination of laparoscopy and hysteroscopy was found to be an easy, safe, effective and appropriate approach for patients with HWWS.

Developmental uterovaginal anomalies and histologic findings in transgender patients receiving gender-affirming hysterectomies: A large case series.

To describe the incidence of uterovaginal anomalies and histologic findings in transgender and nonbinary (TGNB) patients seeking hysterectomies. All patients receiving gender-affirming hysterectomies between 2013 and 2023 were retrospectively reviewed. Primary outcomes included uterovaginal anomalies and histological findings. Multivariable logistic regressions were performed to evaluate relationships between variables of interest and whether they predict findings of uterovaginal anomalies, inactive endometrium, adenomyosis, leiomyoma, endometriosis, and cervical atrophy. 278 patients received hysterectomies at an average age of 29.2 ± 8.3 years. Seven patients (2.5%) were found to have a developmental anomaly, including two bicornuate uterus (0.7%), two unicornuate uterus (0.7%), one septate uterus (0.4%), and two vaginal septum (0.7%). 60 patients (21.6%) were found to have inactive endometrium and 26 patients (9.4%) had cervical atrophy. Although 262 patients (94.2%) were on testosterone therapy, hormone duration was not a significant predictor of any uterine findings. This study describes uterovaginal anomalies in a large cohort of patients receiving gender-affirming hysterectomies. Although long-term testosterone use is commonly believed to be associated with endometrial and cervical atrophy, this study shows no such association.

Congenital uterine arteriovenous malformation treated by hysterectomy: a description of two cases.

Congenital uterine arteriovenous malformation treated by hysterectomy: a description of two cases.

A comprehensive diagnostic approach to differentiate intrauterine arteriovenous malformation in cases of enhanced myometrial vascularity.

The differentiation between conditions such as uterine arteriovenous malformation, pseudoaneurysm, gestational trophoblastic disease, and retained trophoblastic tissue can be challenging. Ultrasound imaging and Doppler interrogation are the primary diagnostic tools to assess cases of enhanced myometrial vascularity and differentiate intrauterine vascular anomalies. However, some cases remain of difficult differentiation. This study aims to analyze suspected cases and describe their diagnostic management and outcomes. We reviewed post-abortion cases that underwent pelvic transvaginal U/S imaging and Doppler examinations due to suspected uterine vascular anomalies. CT scans were performed in cases in which ultrasound did not reach a diagnosis. Simple follow-up, medical or surgical therapy, or embolization of uterine arteries were performed according to the final diagnosis. From 2015 to 2022, we retrieved from electronic ultrasound records 22 cases of suspected vascular malformations. In eight cases, first-line U/S at admission excluded the suspected anomaly.In Five ofthe remaining 14 patients, uterine vascular anomalies were excluded upon a second-level U/S based on angio-Doppler imaging and Doppler peak velocity interrogation. Nine cases underwent CT scan, and a digital angiography and embolization were performed in eight of these cases, of whom only two had a documented uterine arteriovenous malformation. Our triage proved that only two out of 22 suspected cases had a uterine arteriovenous malformation. This diagnosis is frequently misused in clinical practice. Our data confirm that enhanced myometrial vascularity should be used to encompass the spectrum of possible differential diagnosis. A precise step-by-step diagnostic method is of paramount importance to prevent unnecessary interventions.

Identification and functional characteristics of CHD1L gene variants implicated in human Müllerian duct anomalies

BackgroundMüllerian duct anomalies (MDAs) are congenital developmental disorders that present as a series of abnormalities within the reproductive tracts of females. Genetic factors are linked to MDAs and recent advancements in whole-exome sequencing (WES) provide innovative perspectives in this field. However, relevant mechanism has only been investigated in a restricted manner without clear elucidation of respective observations.MethodsOur previous study reported that 2 of 12 patients with MDAs harbored the CHD1L variant c.348-1G>C. Subsequently, an additional 85 MDAs patients were recruited. Variants in CHD1L were screened through the in-house database of WES performed in the cohort and two cases were identified. One presented with partial septate uterus with left renal agenesis and the other with complete septate uterus, duplicated cervices and longitudinal vaginal septum. The pathogenicity of the discovered variants was further assessed by molecular dynamics simulation and various functional assays.ResultsUltimately, two novel heterozygous CHD1L variants, including a missense variant c.956G>A (p.R319Q) and a nonsense variant c.1831C>T (p.R611*) were observed. The variants were absent in 100 controls. Altogether, the contribution yield of CHD1L to MDAs was calculated as 4.12% (4/97). All three variants were assessed as pathogenic through various functional analysis. The splice-site variant c.348-1G>C resulted in a 11 bp sequence skipping in exon 4 of CHD1L and led to nonsense mediated decay of its transcripts. Unlike WT CHD1L, the truncated R611* protein mislocalized to the cytoplasm, abolish the ability of CHD1L to promote cell migration and failed to interact with PARP1 owing to the loss of macro domain. The R319Q variant exhibited conformational disparities and showed abnormal protein recruitment behavior through laser microirradiation comparing with the WT CHD1L. All these variants impaired the CHD1L function in DNA damage repair, thus participating in MDAs.ConclusionsThe current study not only expands the mutational spectrum of CHD1L in MDAs but determines three variants as pathogenic according to ACMG guidelines with reliable functional evidence. Additionally, the impairment in DNA damage repair is an underlying mechanism involved in MDAs.

Uterine arteriovenous malformation: a case report with proposal for pathogenesis

Uterine arteriovenous malformation (UAM) is a rare medical condition. Even though UAM can cause abnormal and life-threatening uterine hemorrhage, there is no consensus on the clinical guideline on its treatments, most likely due to its low incidence, unknown etiology, and distinct fertility demands by different patients. Here, we present one elderly woman with uncommon UAM. We first discuss our experience regarding the diagnoses and treatments of UAM and then propose a hypothesis for the pathogenesis of UAM.

A rare case of uterine arteriovenous malformation masquerading as retained products of conceptus: a case report

An Arteriovenous malformation (AVM) is essentially an abnormal vascular structural anomalous connection between arteries and veins that bypass the capillaries.1 While they can be found anywhere in the body, uterine AVMs are relatively rare with only about 100 cases having been reported in literature to date. Herein, we describe an atypical presentation and subsequent management of a uterine AVM which initially appeared to resemble products of conceptus. It is important to differentiate between the two conditions since the management of the former differs widely from the latter and involves a specialized unit.

Vaginal Agenesis: Diagnosis on Ultrasound and Management of a Case at the Gabriel Toure University Hospital in Bamako (Mali)

Vaginal malformations can be diagnosed by ultrasound in a large number of cases. The prevalence of uterovaginal aplasia usually reported in the literature is 1/4000 to 1/10,000. Objectives: We tell you about the difficulties of diagnosis with ultrasound, the difficulties of managing our case of vaginal aplasia. Observation: This was a 20-year-old patient saleswoman, single. History of the disease: was marked by cyclic pelvic headache for 6 months with primary amenorrhea. She has no particular medical-surgical history. On physical examination: Secondary sexual characteristics were developed. On inspection of the vulva, normal pubic hair was noted. Examination of the vulva showed the labia majora and labia minora present and normal-looking. A hymen could not be identified and there was no visible vaginal opening. Faced with this clinical and paraclinical symptomatology, we made the diagnosis of vaginal agenesis. The ultrasound showed a uterus measuring about 92x71x62 mm, its parenchyma is homogeneous, its contours are regular. The presence in the uterine cavity at the corporeofundic level of a homogeneous echogenic collection of about 66.5 cc (69x35 mm). Note the congenital malformation of the cervix with agenesis). The Surgical Procedure Performed is vaginoplasty. Conclusion: Ultrasound of uterine malformation is difficult and requires some experience from the sonographer.

Predictors of preterm birth following emergency and ultrasound-indicated cervical cerclage: A retrospective study on 136 singleton pregnancies

ObjectiveWe aimed to retrospectively evaluate obstetric and neonatal outcomes in patients who underwent ultrasound-indicated and rescue cervical cerclage and to identify predictors for cerclage failure and consequent preterm birth (PTB). Materials and methodsWe conducted a retrospective analysis on singleton pregnancies between 16 and 27 weeks of gestation who presented with a transvaginal sonographic cervical length (TVS-CL) <25 mm and a previous PTB/second-trimester miscarriage or prolapsed amniotic membranes and/or a TVS-CL <15 mm and underwent cervical cerclage at Mangiagalli Center, Milan, between September 2011 and December 2021. Univariate and multivariate logistic regression analyses were used to identify possible predictive factors of cerclage failure. ResultsDuring the study period, a total of 136 singletons met the inclusion criteria. Overall, 3 % of pregnancies did not reach fetal viability, mostly due to post-cerclage preterm premature rupture of membranes and/or chorioamnionitis. The mean gestational age at delivery was 35.9 ± 4.5 weeks. Neonates were delivered beyond 37 weeks in 63.2 % of cases, between 28 and 37 weeks in 26.5 %, and less than 28 gestational weeks in 10.3 %. At the multivariate analysis, independent risk factors for miscarriage or PTB were cervical length <10 mm (odds ratio, OR 3.44), advanced cervical dilatation (OR 4.76), and in vitro fertilization (OR 4.66). A history of previous miscarriage, premature delivery, and uterine malformations did not increase the risk of post-cerclage delivery before 37 weeks. In the preterm delivery group, 14 % of patients experienced preterm premature rupture of membranes (pPROM) and 10 % had chorioamnionitis, while no case was registered in the term delivery group. A positive vaginal swab at the time of cervical cerclage was not significantly associated with PTB at the multivariate analysis but it emerged as a significant risk factor for both chorioamnionitis (OR 11.03) and pPROM (OR 5.28). ConclusionsUltrasound-indicated and rescue cervical cerclage were effective in prolonging pregnancy, even when placed beyond 24 weeks of gestation. Preoperative cervical length of less than 10 mm, advanced dilatation, and in vitro fertilization are associated with an increased risk of cerclage failure. A positive vaginal swab before cerclage is associated with increased rates of intrauterine infectious-inflammatory processes.

Critical postpartum bleeding caused by an acquired uterine arteriovenous malformation: A Case Report

Introduction Postpartum uterine bleeding is a common occurrence in the field of Obstetrics and varies in severity from harmless to life- threatening. The majority of the cases are due to atony, retained tissue or lesions in the birth canal, making up more than 99% of the cases. However, rare causes, such as structural abnormalities and coagulopathies, are important to keep in mind during diagnostics, when the common causes of postpartum bleeding are ruled out, particularly when the symptoms present in an unconventional manner. Methods A case report of a healthy woman presenting with acute life-threatening postpartum bleeding due to an acquired arteriovenous malformation of the uterus. Results A 43-year-old healthy woman presented with acute life-threatening gynecological bleeding 16 days after acute caesarean section. Ultrasound raised the suspicion of arteriovenous malformation. However, due to uncertainty of the diagnosis no initial treatment was preformed until the patient presented with heavy bleeding once more, resulting in a life-saving hysterectomy. Conclusion Arteriovenous malformation is an important diagnosis to consider, as early and effective diagnostics brings the opportunity for a safe and fertility-preserving treatment in the form of radiological coiling embolization. Advances in ultrasound skills of clinicians has made fast and bedside diagnostics possible, therefore the number of hysterectomies should decrease in favor of fertility-preserving treatment.

A rare case report of urogenital anomaly in a teenage girl: Herlyn–Werner–Wunderlich syndrome/OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly)

BackgroundHerlyn–Werner–Wunderlich syndrome also known as OHVIRA syndrome is a rare complex congenital developmental anomaly characterized by the triad of uterine didelphys, obstructed hemivagina causing hematometrocolpos and ipsilateral renal anomaly.Case presentationHere we report a case of a 14-year-old girl who presented with acute onset lower abdominal pain. Ultrasound and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina and right renal agenesis. Patient underwent hematocolpos drainage.ConclusionsOHVIRA syndrome is an uncommon congenital anomaly. Imaging plays a major role in diagnosis. Surgery is the treatment of choice to resect the septum and relieve the obstruction. An early correct diagnosis is the goal to relieve the symptoms and prevent complications, caused by retrograde menstruation which may result in endometriosis and, also, preserve sexual and conception abilities.

A Challenging Diagnosis - Large Leiomyomas on Rudimentary Uterus in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

A 40-year-old woman diagnosed with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome at 16 years of age presented with a large abdominal mass protruding to the right subcostal margin, equivalent to 30 weeks gestation. She didn’t have comorbidities of hypertension or diabetes. The vitals were normal with BMI 30. She was asymptomatic except for occasional vague abdominal discomfort. Further imaging studies were conducted to assess the extent and nature of the findings. Pelvic and transvaginal ultrasound demonstrated the presence of multiple large fibroids extending beyond the level of the umbilicus. This was corroborated by pelvic MRI, which further confirmed the massively enlarged multi-fibroid uterus. Additionally, intravenous contrast-enhanced imaging revealed large bilateral ovarian collaterals and prominent blood vessels extending to the broad ligament. Triple-phase CT angiogram of the abdomen/pelvis with pre-contrast revealed an 8mm aneurysm on the anterior aspect of the abdominal aorta and unusual vascular anatomy, raising the possibility of an atypical or neoplastic process in the right iliac fossa. In view of these findings, an opinion by an Oncologist was sought, who suspected the possible presence of an abnormal nidus of vessels and a fluid attenuation area in the right iliac fossa. Additionally, the Risk of Ovarian Malignancy Algorithm (ROMA) test was performed, revealing a slightly elevated value. To reach the final diagnosis, the decision for the exploratory laparotomy was undertaken. Intraoperative findings revealed the presence of a rudimentary uterus with normal ovaries (Figure 1 and Figure 2), as well as the presence of bilateral broad fibroids (Figure 3). The broad ligament areas around the uterus were occupied with large circumscribed masses, identified as fibroids. The right-side fibroid measuring 17 x 15cm (Figure 4) and the left-side fibroid measuring 10 x 10cm (Figure 5) were excised, respectively, while preserving the left ovary and rudimentary uterus. Peritoneal fluid samples were obtained to ascertain the nature of malignancy. No evidence of infiltration or adhesions was detected. Postoperatively, the patient experienced no complications. Histological examination of the masses growing from bilateral uterine remnants confirmed the presence of leiomyomas, with no glandular epithelium identified. Peritoneal fluid analysis revealed the absence of malignant cells.

Role of Hysteroscopy in the Management of Uterine Vascular Malformations with a Focus on Enhanced Myometrial Vascularity – A Review of Diagnosis and Treatment with the Suggested Algorithm

Abstract Postpregnancy bleeding is common after failed pregnancy, termination of pregnancy, and postnatally after both vaginal and cesarean delivery. Pelvic ultrasound (US) is usually the initial imaging modality of choice to ascertain the cause when the bleeding is heavy or prolonged. When used in combination with Doppler studies, US can help differentiate retained products of conception (RPOC) from rarer uterine vascular malformations (UVM), including true arterial vascular malformations and areas of enhanced endometrial vascularity (EMV), which may themselves be associated with any RPOC present. While the management of RPOC is well established and has evolved from an almost universal surgical approach to increasingly more medical or expectant alternatives, clinical decisions over the management of a concurrent UVM are less clear and treatment options need to be individualized depending on features of the lesion and the clinical situation with hysteroscopy featured as an important modality, especially when dealing with EMV’s. In this review, we discuss the role of hysteroscopy in the treatment of enhanced myometrial vascularity with and without associated RPOC and propose a management algorithm.

The effect of unicornuate uterus on reproductive outcomes in infertile patients

ObjectiveUnicornuate uterus, which is a rare congenital anomaly, has been associated with obstetric and perinatal complications. The aim of this study is to evaluate the reproductive outcomes of patients with unicornuate uterus, and also to compare them with outcomes of infertile patients without a Müllerian anomaly. MethodsRetrospective analyses of the data of 18–40 year old infertile patients with unicornuate uterus diagnosed in between January 2012 and December 2022, and a control group with the same number of patients, with age and infertility durations matched were performed. Demographic data, cycle parameters and reproductive outcomes of the unicornuate uterus and control groups were compared. ResultsA total of 75 patients with unicornuate uterus and a control group of 75 infertile patients without a Müllerian anomaly were included in the study. Totally, 116 and 91 pregnancies were achieved at unicornuate uterus and the control groups, respectively. Ectopic pregnancy rates (10.3 % vs 2.2 %, OR = 5.53, 95 %CI [1.17–26.21]) and malpresentation rates (29 % vs 0 %, OR = 1.40, 95 %CI [1.12–1.76]) were significantly higher and newborn birth weights in singleton pregnancies were significantly lower in the unicornuate group (3000 g vs 3455 g, p = 0.005). No significant difference was found in other obstetric parameters. ConclusionUnicornuate uterus constitutes a small portion of all congenital uterine anomalies. Although increased ectopic pregnancy and malpresentation rates with diminished birth weights were found in the unicornuate group in our study, live birth rates and preterm deliveries did not differ significantly between the two groups. However, patients should be counseled on this issue both during the reproductive treatment process and during pregnancy, and should be followed closely.

Hysteroscopic Management of Complete Vaginal and Uterine Septum with Double Cervix: Tips & Tricks for a Safe Surgery

The septate uterus is the most common uterine abnormality, comprising 35% of all identified uterine malformations. According to the ESHRE/ESGE 2013 classification, the class U2bC2V1 is a rare congenital malformation characterized by a complete septate uterus with double cervix and nonobstructive longitudinal vaginal septum. to share the decision-making process, the preoperative ultrasonographic assessment and our step-by-step hysteroscopic surgery technique to manage this complex malformation: including how to access the septate uterine cavity, the resection of the septum and its post-operative management. The goal of the surgical treatment is to restore the regular morphology of the vaginal canal and the uterine cavity with the right amount of myometrium. we present video footage of a hysteroscopic approach at a tertiary care academic medical center for managing a 21-year-old patient with a symptomatic uterine septum with double cervix and non-obstructive longitudinal vaginal septum. Both the Intraoperative and postoperative periods were uneventful: a complete resolution of the vaginal septum and creation of a single uterine cavity were obtained. Due to the significant anatomic variability of this condition, this type of surgery can be challenging. The goal of this video is also educational, aiming to illustrate a reproducible technique that can be utilized by younger or less experienced surgeons. In our experience, this hysteroscopic technique appears to be a valid option for patients with symptomatic Mullerian malformation or those with a history of recurrent miscarriages or unexplained infertility.