Mullerian Anomaly – A Conundrum

Mullerian Anomaly – A Conundrum | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Mullerian Anomaly – A Conundrum Vaishnavi Rajaraman, Beena Kumary This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5628143/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background : A broad range of Müllerian anomalies have been documented, and multiple anomalies can coexist in a single individual. Accurate identification of the different components of an anomaly is crucial for determining an effective treatment plan. This report presents an extremely rare case of a Müllerian anomaly in a 34-year-old nulliparous woman. Case Presentation : A 34-year-old nulliparous woman presented to the outpatient department with a six-month history of dyspareunia, without any associated menstrual irregularities or disturbances. Perineal examination revealed a blind-ending vaginal pouch measuring 1 cm, along with a septum and a narrowed vaginal orifice. Ultrasound and pelvic MRI were critical in establishing the diagnosis of a Müllerian anomaly. Discussion : Although this is a rare presentation, it is essential to have a structured protocol for accurate diagnosis and management of Müllerian anomalies to ensure favorable outcomes. The patient underwent a complete septal resection, which successfully addressed her symptoms. This case emphasizes the importance of early and precise diagnosis using imaging modalities such as ultrasound and MRI for planning effective treatment and improving the patient's quality of life. Mullerian anomaly Septate uterus Double cervix Vaginal septum Figures Figure 1 Figure 2 Figure 3 SYNOPSIS This case report highlights the successful diagnosis and surgical management of a rare Müllerian anomaly, leading to significant symptomatic relief and improved sexual health, emphasizing the importance of advanced imaging modalities diagnosis and a multidisciplinary approach. INTRODUCTION The development of the female reproductive tract is a complex process closely linked to the formation of the male (Wolffian) reproductive tract and the renal anlage. A complete uterine septum, double cervix, along with both transverse and longitudinal vaginal septa, represents a rare and complex Müllerian malformation. It is categorized as Class U2b C2 V1 V3 according to the 2013 ESHRE/ESGE classification. ( 1 ) The prevalence of a septate uterus in the general population is approximately 1–2%, and it arises due to abnormal septal reabsorption in both cranial and caudal directions. ( 2 ) This condition is typically associated with adverse reproductive outcomes, such as infertility, recurrent miscarriages, preterm deliveries, and growth-restricted babies. ( 3 ) ( 4 ) The scarcity of reported studies in the literature, the lack of standardized diagnostic tools, and the low incidence of this condition, often result in misdiagnosis. Our report aims to underscore the complexity and rarity of such anomalies while highlighting the importance of 3D ultrasound and Magnetic resonance imaging (MRI) for precise diagnosis and the necessity of a multidisciplinary approach to management. CASE REPORT A 34-year-old married, nulliparous female presented with complaints of difficulty during coitus. She had attained menarche at the age of 12 and had regular menstrual cycles. There was no history of cyclical abdominal pain or distension. Upon abdominal examination, the abdomen was soft, non-tender, and no palpable mass was detected. Local examination revealed a blind-ending vaginal pouch measuring 1 cm with a septum and a narrowed vaginal orifice. Per rectal examination showed no abnormalities. Ultrasonography revealed two separate endometrial cavities continuous with the cervical canals, suggestive of complete uterine septae with two separate cervical canals. A transverse septum was also noted in the lower third of the vagina. (Fig. 1) The right ovary was enlarged, showing a bilocular cyst with thick internal echoes, while the left ovary appeared normal. A plastic surgeon was consulted, who advised higher imaging. MRI findings were consistent with a septate uterus, with an irregular transverse hyperintense band noted in the lower third of the vagina and a few small T2 hyperintense areas within it. A multidisciplinary approach was followed, after obtaining consent, the patient was taken up for surgical correction with the assistance of plastic surgeons. An 8 Fr Foley catheter was inserted through the right-sided opening and used as a guide. A transverse incision was made into the lower transverse vaginal septum, and upon deepening the incision, the left-sided cavity was accessed. A vertical muscular septum was identified, separating the two vaginal cavities. (Fig. 2 ) The septum was incised in the middle for about 7–8 cm, connecting both cavities. The raw areas were then closed by approximating the mucosa. (Fig. 3 ) Vaginal packing was applied to prevent strictures and promote healing. Serial vaginal dilation was advised postoperatively. DISCUSSION The formation of the female genital tract is a complex process, encompassing multiple stages of cellular differentiation, migration, fusion, and canalization. Failure of any of these stages of development can lead to congenital anomalies. Müllerian duct anomalies (MDA) are rare but represent a potentially treatable cause of infertility. Individuals with MDA are more likely to experience higher rates of infertility, recurrent first-trimester miscarriages, fetal growth restriction, abnormal fetal positioning, preterm labor, and retained placenta.( 5 ) Normal development of the Müllerian ducts depends on completing three phases: organogenesis, fusion, and septal resorption. When the final fibrous septum between the two Müllerian ducts fails to dissolve, it forms a uterus that is either fully or partially divided into two separate cavities, known as septate uterus. ( 6 ) Its etiology is often considered multifactorial, involving both genetic and environmental factors. Several genes, such as Pax, Lim1, Emx2, Wnt4, and Wnt9b, play a role in the development of the Müllerian ducts during morphogenesis. The vaginal septum can be either transverse or longitudinal in orientation and may be complete (causing obstruction) or incomplete (with fenestrations). ( 7 ) A transverse vaginal septum arises from a defect in the fusion of the downward-growing Müllerian duct and the upward-growing derivative of the urogenital sinus, and it is further classified as low, middle, or high based on its location. In contrast, longitudinal vaginal septum results from defective lateral fusion and incomplete resorption of the caudal portion of the Müllerian duct. ( 8 ) ( 9 ) Clinical presentations vary significantly depending on the type of anomaly. In cases of transverse vaginal septum (TVS), children may present with mucocolpos, adolescents with hematocolpos, while some individuals may remain asymptomatic, and others may present with primary amenorrhea A longitudinal vaginal septum, on the other hand, may present with dyspareunia, dystocia, or hygiene issues, though it is often asymptomatic.The use of diverse imaging modalities and clinical information offers valuable insights for diagnosing Müllerian duct anomalies (MDAs). The diagnostic imaging workup for MDAs typically includes 3D ultrasonography and MRI. ( 10 ) ( 11 ) Numerous studies have demonstrated the benefits of surgical correction as the primary treatment, leading to symptomatic improvement and enhanced reproductive outcomes While the septate uterus is particularly troublesome for reproductive outcomes, its treatment with hysteroscopic metroplasty is relatively straightforward and effective. ( 12 ) The vaginal septum should be treated with surgical resection and anastomosis of the proximal and distal vaginas. The choice of technique for TVS depends on the septum's location and thickness, which are assessed through physical examination, ultrasound, and MRI, and may involve either vaginal or laparoscopic approaches. Lower, thinner, or perforated Septa tend to have the best outcomes. The primary complications include stenosis, re-obstruction, dyspareunia, and psychological challenges. Postoperative vaginal dilation is generally recommended to enhance surgical outcomes. ( 13 ) Management in the literature emphasizes early diagnosis and surgical correction, as seen in our case, where timely septal resection led to successful symptom resolution, highlighting the importance of a tailored, multidisciplinary approach for optimal outcomes. At the 6-month post-operative follow-up, the patient showed a well-healed vagina with a length of approximately 7–8 cm. She experienced significant symptomatic relief and reported improved sexual health, indicating a successful surgical outcome and enhanced quality of life. The complexity of Müllerian anomalies is highlighted by their often-silent presentation and, in rare instances, their concurrent occurrence in the same individual, as seen in our case study. Our report also highlights the challenges involved in diagnosing and treating complex MDAs. The developmental abnormalities of the female genital tract, particularly septate uterus and vaginal septa, significantly impact the quality of sexual life & reproductive outcomes. Despite the rarity of these anomalies, awareness and a multidisciplinary approach are essential for timely diagnosis and effective management, as demonstrated in this case. CONCLUSION This case underscores the complexity and rarity of complex congenital Müllerian anomalies. Despite the uncommon presentation, multimodal imaging techniques such as 3D ultrasound and MRI are crucial for accurate diagnosis. Timely surgical intervention, involving a multidisciplinary team, can improve reproductive outcomes and enhance the overall quality of life for affected individuals. Declarations Funding Statement: No funding was obtained for our case study Clinical Trial Number – Not applicable Attestation Statement: • The subjects in this trial have not concomitantly been involved in other randomized trials – Not applicable • Data regarding any of the subjects in the study has not been previously published unless specified. • Data will be made available to the editors of the journal for review or query upon request. CARE CHECKLIST – This was followed while writing the report Ethics approval and consent to participate – Institutional ethics committee approval was obtained for publication. Consent for publication - A written informed consent from the patient was obtained for publication. Availability of data and material – The data is available if the corresponding author is approached Competing interests – None Funding – No funding was obtained Authors' contributions – VR - (First & Corresponding author) - Conceptualization, Data curation, validation, writing – original draft BK - (Second author) - Conceptualization, Data curation, validation, writing – Review & editing, supervision Acknowledgments - None References Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M et al. The ESHRE/ESGE Consensus on the Classification of Female Genital Tract Congenital Anomalies. Human Reproduction , vol. 28, no. 8, 14 June 2013, pp. 2032–2044, , https://doi.org/10.1093/humrep/det098 . Accessed 23 Dec. 2019. McBean JH, Brumsted JR. Septate Uterus with Cervical Duplication: A Rare Malformation. Fertility and Sterility , vol. 62, no. 2, 1 Aug. 1994, pp. 415–417, https://doi.org/10.1016/s0015-0282(16)56901-5 . Accessed 8 Aug. 2023. Chan YY et al. Sept. Reproductive Outcomes in Women with Congenital Uterine Anomalies: A Systematic Review. Ultrasound in Obstetrics & Gynecology , vol. 38, no. 4, 20 2011, pp. 371–382, obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.10056 , https://doi.org/10.1002/uog.10056 . Accessed 23 Dec. 2019. Grimbizis GF. Clinical Implications of Uterine Malformations and Hysteroscopic Treatment Results. Human Reproduction Update , vol. 7, no. 2, 1 Mar. 2001, pp. 161–174, academic.oup.com/humupd/article/7/2/161/638048 , https://doi.org/10.1093/humupd/7.2.161 Soc A, Fertil. The American Fertility Society Classification of Adnexal Adhesions, Distal Tubal Occlusion Secondary to Tubal Ligation, Tubal Pregnancies, Mullerian Anomalies and Intrauterine Adhesions. Fertility and Sterility , vol. 49, 1 Jan. 1988, pp. 944–955. Accessed 18 Sept. 2024. Chandler TM, et al. Müllerian Duct Anomalies: From Diagnosis to Intervention. Br J Radiol. Dec. 2009;82(984):1034–42. https://doi.org/10.1259/bjr/99354802 . . Sachita, Chapagain et al. Jan. An Unusual Case Report of Transverse Vaginal Septum Presenting as Primary Amenorrhea. International Journal of Surgery Case Reports , vol. 114, 1 2024, pp. 109132–109132, https://doi.org/10.1016/j.ijscr.2023.109132 . Accessed 18 Sept. 2024. Hoffman BL, et al. Williams Gynecology, 4e. New York, N.Y.: Mcgraw-Hill Education Llc; 2020. Joki-Erkkilä, Minna, Pentti KH. Presenting and Long-Term Clinical Implications and Fecundity in Females with Obstructing Vaginal Malformations. Journal of Pediatric and Adolescent Gynecology , vol. 16, no. 5, 1 Oct. 2003, pp. 307–312, https://doi.org/10.1016/s1083-3188(03)00157-8 Heinonen P, LONGITUDINAL, VAGINAL SEPTUM. Europ. J. Obstet. Gynec. Reprod. Biol , vol. 13, 1982, pp. 253–258. Accessed 18 Sept. 2024. Yoo R-E et al. A Systematic Approach to the Magnetic Resonance Imaging-Based Differential Diagnosis of Congenital Müllerian Duct Anomalies and Their Mimics. Abdominal Imaging , vol. 40, no. 1, 29 July 2014, pp. 192–206, https://doi.org/10.1007/s00261-014-0195-9 Rousseau L et al. June. Daily Practice Management of Septate Uterus: Reproductive Outcome after Septoplasty. Facts, Views and Vision in ObGyn , vol. 13, no. 3, 2021, pp. 257–263, https://doi.org/10.52054/fvvo.13.3.032 . Accessed 3 Oct. 2021. Williams CE et al. Aug. Transverse Vaginal Septae: Management and Long-Term Outcomes. BJOG: An International Journal of Obstetrics & Gynaecology , vol. 121, no. 13, 19 June 2014, pp. 1653–1658, https://doi.org/10.1111/1471-0528.12899 . Accessed 25 2019. Additional Declarations No competing interests reported. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-5628143","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":391710689,"identity":"cc48a183-3ec3-4bb5-af29-ac4d92419977","order_by":0,"name":"Vaishnavi Rajaraman","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA7UlEQVRIiWNgGAWjYNACA4YEEHWAoQJIMjM3kKLlDEgLIzFaGCBaGBjbwCR+LeazD7BJ/CioyzNnP2N4uHJebTR/O1DLj4ptOLXInEtgk+wxOFxs2ZNjcPDstuO5Mw4zNjD2nLmNU4sEDwOzAY/BgcQNB9ISDjZuO5bbANTCzNiGX4vhH4O6xA3nnwG1zDmWO58ILYyPeQyYEzfcSD5wsLGhJncDYS2MjY9lDA4DtTw+cLDh2IHcjUAtB/H7hfnAwTd/QA5LbP7YUFOXO+/84YMPflTg1oIeC4fB5AE86jFAHSmKR8EoGAWjYIQAACHEXmgN6jiOAAAAAElFTkSuQmCC","orcid":"","institution":"Government medical college kottayam","correspondingAuthor":true,"prefix":"","firstName":"Vaishnavi","middleName":"","lastName":"Rajaraman","suffix":""},{"id":391710690,"identity":"b6615837-1258-4baf-867e-c6a4001d0d63","order_by":1,"name":"Beena Kumary","email":"","orcid":"","institution":"Government medical college kottayam","correspondingAuthor":false,"prefix":"","firstName":"Beena","middleName":"","lastName":"Kumary","suffix":""}],"badges":[],"createdAt":"2024-12-12 04:23:07","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-5628143/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-5628143/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":72301197,"identity":"6cd118cf-3164-48b1-9798-8b3a03b9666f","added_by":"auto","created_at":"2024-12-25 01:29:48","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":231491,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003e3D Ultrasonogram of the pelvis showing two separate endometrial cavities, continuous with two cervical canals, suggestive of a complete septate uterus and double cervix.\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-5628143/v1/f41a0eaff070ecc7774feb91.png"},{"id":72301196,"identity":"538ebd6a-34d8-4a3c-a19b-991746346fb5","added_by":"auto","created_at":"2024-12-25 01:29:48","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":2120241,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eIntra-operative picture showing narrowed vaginal orifice (Left), through which an 8 Fr catheter is inserted as a guide wire. The image on the right shows two separate vaginal cavities divided by a longitudinal vaginal septum\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-5628143/v1/3c7270b279b32a7dece26b72.png"},{"id":72301374,"identity":"ffce5c96-ec56-455d-a613-f27221378804","added_by":"auto","created_at":"2024-12-25 01:37:48","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":1521300,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ePostoperative picture showing increased vaginal length after septal resection (Left). \u0026nbsp;The approximated vaginal wall post excision (Right).\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-5628143/v1/7d6a8b119dbfa652f3c037c4.png"},{"id":72301375,"identity":"83a7b56c-8e4c-4d8d-b874-d68cc40f9e51","added_by":"auto","created_at":"2024-12-25 01:37:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":4068376,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5628143/v1/05d1e451-8482-47fc-b2ab-cf895efe96dd.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eMullerian Anomaly – A Conundrum\u003c/p\u003e","fulltext":[{"header":"SYNOPSIS","content":"\u003cp\u003eThis case report highlights the successful diagnosis and surgical management of a rare M\u0026uuml;llerian anomaly, leading to significant symptomatic relief and improved sexual health, emphasizing the importance of advanced imaging modalities diagnosis and a multidisciplinary approach.\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"INTRODUCTION","content":"\u003cp\u003eThe development of the female reproductive tract is a complex process closely linked to the formation of the male (Wolffian) reproductive tract and the renal anlage. A complete uterine septum, double cervix, along with both transverse and longitudinal vaginal septa, represents a rare and complex M\u0026uuml;llerian malformation. It is categorized as Class U2b C2 V1 V3 according to the 2013 ESHRE/ESGE classification. (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e) The prevalence of a septate uterus in the general population is approximately 1\u0026ndash;2%, and it arises due to abnormal septal reabsorption in both cranial and caudal directions. (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e) This condition is typically associated with adverse reproductive outcomes, such as infertility, recurrent miscarriages, preterm deliveries, and growth-restricted babies. (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e) (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e) The scarcity of reported studies in the literature, the lack of standardized diagnostic tools, and the low incidence of this condition, often result in misdiagnosis. Our report aims to underscore the complexity and rarity of such anomalies while highlighting the importance of 3D ultrasound and Magnetic resonance imaging (MRI) for precise diagnosis and the necessity of a multidisciplinary approach to management.\u003c/p\u003e"},{"header":"CASE REPORT","content":"\u003cp\u003eA 34-year-old married, nulliparous female presented with complaints of difficulty during coitus. She had attained menarche at the age of 12 and had regular menstrual cycles. There was no history of cyclical abdominal pain or distension. Upon abdominal examination, the abdomen was soft, non-tender, and no palpable mass was detected. Local examination revealed a blind-ending vaginal pouch measuring 1 cm with a septum and a narrowed vaginal orifice. Per rectal examination showed no abnormalities.\u003c/p\u003e \u003cp\u003eUltrasonography revealed two separate endometrial cavities continuous with the cervical canals, suggestive of complete uterine septae with two separate cervical canals. A transverse septum was also noted in the lower third of the vagina. (Fig.\u0026nbsp;1) The right ovary was enlarged, showing a bilocular cyst with thick internal echoes, while the left ovary appeared normal. A plastic surgeon was consulted, who advised higher imaging. MRI findings were consistent with a septate uterus, with an irregular transverse hyperintense band noted in the lower third of the vagina and a few small T2 hyperintense areas within it.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eA multidisciplinary approach was followed, after obtaining consent, the patient was taken up for surgical correction with the assistance of plastic surgeons. An 8 Fr Foley catheter was inserted through the right-sided opening and used as a guide. A transverse incision was made into the lower transverse vaginal septum, and upon deepening the incision, the left-sided cavity was accessed. A vertical muscular septum was identified, separating the two vaginal cavities. (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e)\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe septum was incised in the middle for about 7\u0026ndash;8 cm, connecting both cavities. The raw areas were then closed by approximating the mucosa. (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e) Vaginal packing was applied to prevent strictures and promote healing. Serial vaginal dilation was advised postoperatively.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eThe formation of the female genital tract is a complex process, encompassing multiple stages of cellular differentiation, migration, fusion, and canalization. Failure of any of these stages of development can lead to congenital anomalies. M\u0026uuml;llerian duct anomalies (MDA) are rare but represent a potentially treatable cause of infertility. Individuals with MDA are more likely to experience higher rates of infertility, recurrent first-trimester miscarriages, fetal growth restriction, abnormal fetal positioning, preterm labor, and retained placenta.(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e)\u003c/p\u003e \u003cp\u003eNormal development of the M\u0026uuml;llerian ducts depends on completing three phases: organogenesis, fusion, and septal resorption. When the final fibrous septum between the two M\u0026uuml;llerian ducts fails to dissolve, it forms a uterus that is either fully or partially divided into two separate cavities, known as septate uterus. (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e) Its etiology is often considered multifactorial, involving both genetic and environmental factors. Several genes, such as\u003c/p\u003e \u003cp\u003ePax, Lim1, Emx2, Wnt4, and Wnt9b, play a role in the development of the M\u0026uuml;llerian ducts during morphogenesis.\u003c/p\u003e \u003cp\u003eThe vaginal septum can be either transverse or longitudinal in orientation and may be complete (causing obstruction) or incomplete (with fenestrations). (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e) A transverse vaginal septum arises from a defect in the fusion of the downward-growing M\u0026uuml;llerian duct and the upward-growing derivative of the urogenital sinus, and it is further classified as low, middle, or high based on its location. In contrast, longitudinal vaginal septum results from defective lateral fusion and incomplete resorption of the caudal portion of the M\u0026uuml;llerian duct. (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e) (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e)\u003c/p\u003e \u003cp\u003eClinical presentations vary significantly depending on the type of anomaly. In cases of transverse vaginal septum (TVS), children may present with mucocolpos, adolescents with hematocolpos, while some individuals may remain asymptomatic, and others may present with primary amenorrhea A longitudinal vaginal septum, on the other hand, may present with dyspareunia, dystocia, or hygiene issues, though it is often asymptomatic.The use of diverse imaging modalities and clinical information offers valuable insights for diagnosing M\u0026uuml;llerian duct anomalies (MDAs). The diagnostic imaging workup for MDAs typically includes 3D ultrasonography and MRI. (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e) (\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e)\u003c/p\u003e \u003cp\u003eNumerous studies have demonstrated the benefits of surgical correction as the primary treatment, leading to symptomatic improvement and enhanced reproductive outcomes While the septate uterus is particularly troublesome for reproductive outcomes, its treatment with hysteroscopic metroplasty is relatively straightforward and effective. (\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e)\u003c/p\u003e \u003cp\u003eThe vaginal septum should be treated with surgical resection and anastomosis of the proximal and distal vaginas. The choice of technique for TVS depends on the septum's location and thickness, which are assessed through physical examination, ultrasound, and MRI, and may involve either vaginal or laparoscopic approaches. Lower, thinner, or perforated Septa tend to have the best outcomes. The primary complications include stenosis, re-obstruction, dyspareunia, and psychological challenges. Postoperative vaginal dilation is generally recommended to enhance surgical outcomes. (\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e)\u003c/p\u003e \u003cp\u003eManagement in the literature emphasizes early diagnosis and surgical correction, as seen in our case, where timely septal resection led to successful symptom resolution, highlighting the importance of a tailored, multidisciplinary approach for optimal outcomes. At the 6-month post-operative follow-up, the patient showed a well-healed vagina with a length of approximately 7\u0026ndash;8 cm. She experienced significant symptomatic relief and reported improved sexual health, indicating a successful surgical outcome and enhanced quality of life.\u003c/p\u003e \u003cp\u003eThe complexity of M\u0026uuml;llerian anomalies is highlighted by their often-silent presentation and, in rare instances, their concurrent occurrence in the same individual, as seen in our case study. Our report also highlights the challenges involved in diagnosing and treating complex MDAs. The developmental abnormalities of the female genital tract, particularly septate uterus and vaginal septa, significantly impact the quality of sexual life \u0026amp; reproductive outcomes. Despite the rarity of these anomalies, awareness and a multidisciplinary approach are essential for timely diagnosis and effective management, as demonstrated in this case.\u003c/p\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003eThis case underscores the complexity and rarity of complex congenital M\u0026uuml;llerian anomalies. Despite the uncommon presentation, multimodal imaging techniques such as 3D ultrasound and MRI are crucial for accurate diagnosis. Timely surgical intervention, involving a multidisciplinary team, can improve reproductive outcomes and enhance the overall quality of life for affected individuals.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding Statement:\u0026nbsp;\u003c/strong\u003eNo funding was obtained for our case study\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinical Trial Number \u0026ndash;\u0026nbsp;\u003c/strong\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAttestation Statement:\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u0026bull; The subjects in this trial have not concomitantly been involved in other randomized\u003c/p\u003e\n\u003cp\u003etrials \u0026ndash; Not applicable\u003c/p\u003e\n\u003cp\u003e\u0026bull; Data regarding any of the subjects in the study has not been previously published unless\u003c/p\u003e\n\u003cp\u003especified.\u003c/p\u003e\n\u003cp\u003e\u0026bull; Data will be made available to the editors of the journal for review or query upon\u003c/p\u003e\n\u003cp\u003erequest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCARE CHECKLIST \u0026ndash;\u0026nbsp;\u003c/strong\u003eThis was followed while writing the report\u003c/p\u003e\n\u003cp\u003eEthics approval and consent to participate \u0026ndash; Institutional ethics committee approval was obtained for publication.\u003c/p\u003e\n\u003cp\u003eConsent for publication - A written informed consent from the patient was obtained for publication.\u003c/p\u003e\n\u003cp\u003eAvailability of data and material \u0026ndash; The data is available if the corresponding author is approached\u003c/p\u003e\n\u003cp\u003eCompeting interests \u0026ndash; None\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eFunding \u0026ndash; No funding was obtained\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAuthors\u0026apos; contributions \u0026ndash; VR - (First \u0026amp; Corresponding author) - Conceptualization, Data curation, validation, writing \u0026ndash; original draft\u003c/p\u003e\n\u003cp\u003eBK - (Second author) - Conceptualization, Data curation, validation, writing \u0026ndash; Review \u0026amp; editing, supervision\u003c/p\u003e\n\u003cp\u003eAcknowledgments - None\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eGrimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M et al. 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Dec. 2009;82(984):1034\u0026ndash;42. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1259/bjr/99354802\u003c/span\u003e\u003cspan address=\"10.1259/bjr/99354802\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e\u003c/span\u003e\u003cspan address=\"http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3473390/\" targettype=\"URL\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSachita, Chapagain et al. Jan. 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Daily Practice Management of Septate Uterus: Reproductive Outcome after Septoplasty. \u003cem\u003eFacts, Views and Vision in ObGyn\u003c/em\u003e, vol. 13, no. 3, 2021, pp. 257\u0026ndash;263, \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.52054/fvvo.13.3.032\u003c/span\u003e\u003cspan address=\"10.52054/fvvo.13.3.032\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. Accessed 3 Oct. 2021.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eWilliams CE et al. Aug. Transverse Vaginal Septae: Management and Long-Term Outcomes. \u003cem\u003eBJOG: An International Journal of Obstetrics \u0026amp; Gynaecology\u003c/em\u003e, vol. 121, no. 13, 19 June 2014, pp. 1653\u0026ndash;1658, \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1111/1471-0528.12899\u003c/span\u003e\u003cspan address=\"10.1111/1471-0528.12899\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. Accessed 25 2019.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Mullerian anomaly, Septate uterus, Double cervix, Vaginal septum","lastPublishedDoi":"10.21203/rs.3.rs-5628143/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5628143/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e: A broad range of Müllerian anomalies have been documented, and multiple anomalies can coexist in a single individual. Accurate identification of the different components of an anomaly is crucial for determining an effective treatment plan. This report presents an extremely rare case of a Müllerian anomaly in a 34-year-old nulliparous woman.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase Presentation\u003c/strong\u003e: A 34-year-old nulliparous woman presented to the outpatient department with a six-month history of dyspareunia, without any associated menstrual irregularities or disturbances. Perineal examination revealed a blind-ending vaginal pouch measuring 1 cm, along with a septum and a narrowed vaginal orifice. Ultrasound and pelvic MRI were critical in establishing the diagnosis of a Müllerian anomaly.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDiscussion\u003c/strong\u003e: Although this is a rare presentation, it is essential to have a structured protocol for accurate diagnosis and management of Müllerian anomalies to ensure favorable outcomes. The patient underwent a complete septal resection, which successfully addressed her symptoms. This case emphasizes the importance of early and precise diagnosis using imaging modalities such as ultrasound and MRI for planning effective treatment and improving the patient's quality of life.\u003c/p\u003e","manuscriptTitle":"Mullerian Anomaly – A Conundrum","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-12-25 01:29:44","doi":"10.21203/rs.3.rs-5628143/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"94a829fc-1b74-4af6-9f25-fcd987a33245","owner":[],"postedDate":"December 25th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-02-05T06:23:41+00:00","versionOfRecord":[],"versionCreatedAt":"2024-12-25 01:29:44","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5628143","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5628143","identity":"rs-5628143","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}