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Útero totalmente septado
ORPHA:180126CID-10 · Q51.2CID-11 · LB44.4DOENÇA RARA

A síndrome de Herlyn–Werner–Wunderlich (SHWW), também conhecida como OHVIRA é uma síndrome extremamente rara caracterizada por uma defectividade congênita dos órgãos abdominais inferiores e pélvicos. É um tipo de anormalidade dos ductos müllerianos.

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Introdução

O que você precisa saber de cara

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Malformação congênita uterina onde um septo completo divide a cavidade uterina em duas, podendo causar infertilidade, abortos recorrentes e complicações na gravidez.

Publicações científicas
82 artigos
Último publicado: 2026 Apr 14
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q51.2
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico82PubMed
Últimos 10 anos51publicações
Pico202411 papers
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2026Hoje · 2026📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

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Publicações mais relevantes

Timeline de publicações
52 papers (10 anos)
#1

Unilateral cervical carcinoma in a septate uterus with double cervix: A case report and literature review.

Gynecologic oncology reports2026 Apr

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.

#2

Chronic pelvic pain secondary to venous congestion of longitudinal vaginal septum: a case report.

AJOG global reports2025 Nov

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.

#3

Complete Septate Uterus With Cervical Duplication and Vaginal Septum (U2b C2 V1): A Rare Müllerian Malformation With Diagnostic and Management Challenges.

Cureus2025 Jul

Complex Müllerian anomalies, such as a complete septate uterus (U2b), cervical duplication (C2), and a non-obstructive longitudinal vaginal septum (V1), are rare malformations with intricate embryological origins. They often remain undiagnosed in the absence of specific clinical symptoms. This case highlights the diagnostic value of pelvic magnetic resonance imaging (MRI) and the therapeutic benefit of personalized surgical management. In our patient, surgical resection of the vaginal septum led to significant symptomatic improvement, including the restoration of a satisfactory sexual life.

#4

Laparoscopic transabdominal cerclage in a patient with a complete septate uterus and duplicated cervices.

Fertility and sterility2025 Nov 15

Cervical insufficiency is a significant cause of second-trimester loss and preterm birth. Transabdominal cerclage is indicated after failed transvaginal cerclage or anatomical factors that make a vaginal approach technically difficult-such as müllerian anomalies, prior cervical surgery, or cervical duplication. In patients with müllerian anomalies undergoing a cerclage placement, it is critical to properly identify the anatomy for surgical planning and patient counseling of the cerclage method. To describe transabdominal cerclage indications and surgical technique and to illustrate a needleless laparoscopic abdominal cerclage placement in a patient with a complete septate uterus and duplicated cervices and important related considerations. Case report. This case describes a 33-year-old G2P0201 female, with a history of undergoing a loop electrosurgical excision procedure and longitudinal vaginal septum resection, and who was previously misdiagnosed with a didelphic uterus and duplicated cervices. She had a failed history-indicated transvaginal cerclage placed around the cervix connected to the gravid hemicavity during her second pregnancy and was referred to our practice for transabdominal cerclage placement. A preoperative magnetic resonance imaging scan was performed. (The patient(s) included in this video gave consent for publication of the video and posting of the video online including social media, journal website, scientific literature websites [such as PubMed, ScienceDirect, Scopus, etc.] and other applicable sites.) INTERVENTION: Needleless laparoscopic transabdominal cerclage. Preoperative workup, imaging, and surgical technique. Magnetic resonance imaging scan revealed a complete septate uterus with duplicated cervices. A needleless laparoscopic transabdominal cerclage was placed after careful dissection of the uterine vessels at the level of the internal cervical os and creation of a tunnel medial to them. Nonabsorbable, braided polyester suture was used. Transabdominal cerclage is a feasible alternative in patients with congenital uterine anomalies, as a transvaginal approach might increase the risk of failure. Accurate diagnosis through advanced imaging, multidisciplinary consultation, and individualized surgical planning are key to optimizing reproductive outcomes.

#5

Surgical and reproductive outcomes of hysteroscopic metroplasty for complete septate uterus with or without associated cervical anomalies and/or vaginal septa.

European journal of obstetrics, gynecology, and reproductive biology2025 Aug

To evaluate surgical outcomes of hysteroscopic metroplasty for complete uterine septa with or without associated cervical anomalies and/or vaginal septa, and to compare reproductive outcomes to determine if the presence of these anomalies affects surgical success and fertility outcomes. multicentre retrospective observational study. 2 university affiliated hospitals in Italy. The study cohort comprised patients with history of primary infertility, recurrent pregnancy loss or severe preterm birth with complete uterine septum with or without cervical and/or vaginal septa anomalies diagnosed on 3D transvaginal ultrasound and hysteroscopy for which they had hysteroscopic metroplasty between January 2020 and May 2023 in either of the 2 participating centres. hysteroscopic metroplasty performed in the operating room under general or loco-regional anesthesia, using either miniaturized instruments (5Fr electrodes or 15Fr bipolar resectoscope) or 27Fr bipolar resectoscopes followed by application of auto-cross-linked hyaluronic acid gel for the prevention of post-operative adhesions. A total of 101 patients were included in our study. We collected perioperative outcomes including operative time, operative complications, ease of the procedure and need for a repeat resection for residual septum. We also explored reproductive outcomes including clinical pregnancy, live birth and miscarriage rates. All surgical procedures were successfully performed without any significant complications. Operative time was shorter and procedures were reported to be easier in patients without cervical anomalies (U2bC0V0). A second surgical resection was required in most cases regardless of the presence of cervical abnormalities. Reproductive outcomes were available for 66 (65.3 %) patients and there were no significant differences in reproductive outcomes between patients with and without cervical/vaginal anomalies. Hysteroscopic metroplasty for complete uterine septum, with or without cervical/vaginal abnormalities is effective and safe. The presence of cervical anomalies doesn't seem to affect the need for a second resection or reproductive outcomes.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC41 artigos no totalmostrando 50

2026

Unilateral cervical carcinoma in a septate uterus with double cervix: A case report and literature review.

Gynecologic oncology reports
2025

Chronic pelvic pain secondary to venous congestion of longitudinal vaginal septum: a case report.

AJOG global reports
2025

Complete Septate Uterus With Cervical Duplication and Vaginal Septum (U2b C2 V1): A Rare Müllerian Malformation With Diagnostic and Management Challenges.

Cureus
2025

Laparoscopic transabdominal cerclage in a patient with a complete septate uterus and duplicated cervices.

Fertility and sterility
2025

Surgical and reproductive outcomes of hysteroscopic metroplasty for complete septate uterus with or without associated cervical anomalies and/or vaginal septa.

European journal of obstetrics, gynecology, and reproductive biology
2025

One-step outpatient diagnosis and treatment of European Society of Human Reproduction and Embryology and European Society for Gynaecological Endoscopy class U1 and U2 congenital Müllerian anomalies.

Reproductive biomedicine online
2025

Cesarean Delivery of a Dichorionic Diamniotic Twin Pregnancy Complicated With a Complete Septate Uterus: A Case of Septum Perforation to Deliver the Trailing Twin.

Cureus
2025

HOXD10: A novel gene implicated in human Müllerian duct anomalies.

European journal of obstetrics, gynecology, and reproductive biology
2024

Hysteroscopic Management of Complete Septate Uterus With Septate Cervix and Longitudinal Vaginal Septum in a Third-Level Mexican Institution: A Case Report.

Cureus
2024

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

Biological research
2024

Assisted reproductive technology outcomes in women with congenital uterine anomalies: a systematic review.

Archives of gynecology and obstetrics
2025

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

Journal of pediatric and adolescent gynecology
2024

Clinical characteristics and treatment outcomes of patients with a septate uterus complicated by endometriosis.

Journal of gynecology obstetrics and human reproduction
2024

Natural conception and term pregnancy after hysteroscopic incision of complete septate uterus with septate cervix: A case report and literature review.

Taiwanese journal of obstetrics & gynecology
2024

Cervical Septum Incision Adversely Impacts Clinical Outcomes in Women With Complete Uterine Septum and Duplicated Cervix.

Journal of minimally invasive gynecology
2024

Hysteroscopic management of complete septate uterus with septate cervix, and longitudinal vaginal septum: a case report.

Journal of surgical case reports
2024

Effects of hysteroscopic septum incision versus expectant management on IVF outcomes in women with complete septate uterus: a retrospective study.

BMC women's health
2024

Splenogonadal fusion in a female: A case report of a rare congenital anomaly.

Radiology case reports
2024

Impact of congenital uterine anomalies on reproductive outcomes of IVF/ICSI-embryo transfer: a retrospective study.

European journal of medical research
2024

3D reconstruction of the uterus and automatic segmentation of the uterine cavity on 3D magnetic resonance imaging: A preliminary study.

Heliyon
2023

Aggressive Cervical Dilation Exploits Potential Weakness in the Septum of Duplicated Cervix during Hysteroscopic Cervix-preserving Metroplasty of Complete Septate Uterus: A Cohort Study.

Journal of minimally invasive gynecology
2023

Hysteroscopic Fenestration with Precise Incision of the Cavity Septum: A Novel Minimally Invasive Surgery of Complete Septate Uterus with Double Cervix.

Journal of minimally invasive gynecology
2023

A fetus of partial urorectal septum malformation sequence characterized by complete septate uterus: A case report.

Medicine
2023

Bilateral cervical cancer in a complete septate uterus with a double cervix and vagina: a case report.

The Journal of international medical research
2023

Reproductive outcomes after uterine septum resection in patients with recurrent miscarriage or infertility: a retrospective study in Chinese women.

Archives of gynecology and obstetrics
2022

Preliminary application of three-dimensional printing in congenital uterine anomalies based on three-dimensional transvaginal ultrasonographic data.

BMC women's health
2022

Incidence of congenital uterine abnormalities in polycystic ovarian syndrome (CONUTA Study).

European journal of obstetrics, gynecology, and reproductive biology
2022

A rare variant of obstructed hemivagina and ipsilateral renal agenesis and its improvement of classification.

The journal of obstetrics and gynaecology research
2021

Three degrees of separation: complete uterine and cervical septa.

Fertility and sterility
2021

Ultrasound at the intersection of art, science and technology: complete septate uterus with longitudinal vaginal septum.

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2021

Clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis.

The journal of obstetrics and gynaecology research
2020

Rare Case Report of an Endometrial Adenocarcinoma Arising in a Complete Septate Uterus With a Double Cervix and Vagina.

Cureus
2020

Longitudinal vaginal septum: a proposed classification and surgical management.

Fertility and sterility
2023

Analysis of postoperative pregnancy outcome in 180 women with congenital uterine malformation.

Archives of medical science : AMS
2020

Operative hysteroscopy-assisted pregnancy termination after failed surgical abortion in missed abortion of woman with complete septate uterus.

Obstetrics & gynecology science
2020

Reproductive outcome of a complete septate uterus after hysteroscopic metroplasty.

The Journal of international medical research
2018

An unusual case of Herlyn-Werner-Wunderlich syndrome with a complete septate uterus and complicated urinary tract with rare ectopic ureteral opening.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2018

Brachytherapy for cervical cancer in septate uterus: Dose-volume differences with tandem implant placement in right vs. left uterine canal: A case report.

Oncology letters
2018

New Ambulatory Hysteroscopic Septoplasty using Ballooning in a Woman with Complete Septate Uterus: A Case Report.

Development & reproduction
2018

Vaginal atresia and cervical agenesis combined with asymmetric septate uterus: A case report of a new genital malformation and literature review.

Medicine
2018

Hysteroscopic Corrections for Complete Septate and T-Shaped Uteri Have Similar Surgical and Reproductive Outcome.

Reproductive sciences (Thousand Oaks, Calif.)
2018

Small-Diameter Hysteroscopic Metroplasty for a Septate Uterus after Open-Assisted Laparoscopic Radical Trachelectomy.

Journal of minimally invasive gynecology
2017

Endometrial cancer with congenital uterine anomalies: 3 case reports and a literature review.

Cancer biology & therapy
2016

Intrapartal resection of the double cervix and longitudinal vaginal septum after hysteroscopic resection of the complete uterine septum, resulting in a term vaginal delivery: A case report.

Case reports in women's health
2016

Rare case of complicated congenital anomalies of female reproductive organs with bilateral undescended ovaries.

The journal of obstetrics and gynaecology research
2016

Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman-a rare variant of Herlyn-Werner-Wunderlich syndrome.

BJR case reports
2016

An Exceptional Case of Complete Septate Uterus With Unilateral Cervical Aplasia (Class U2bC3V0/ESHRE/ESGE Classification) and Isolated Mullerian Remnants: Combined Hysteroscopic and Laparoscopic Treatment.

Journal of minimally invasive gynecology
2015

Rare Müllerian Anomaly: Complete Septate Uterus with Simultaneous Longitudinal and Transverse Vaginal Septa.

Journal of pediatric and adolescent gynecology
2015

Pregnancy after hysteroscopic metroplasty under laparoscopy in a woman with complete septate uterus: a case report.

Journal of UOEH
2015

Endometrial carcinoma located in the right septate uterus cavity: a case report.

The Pan African medical journal

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Unilateral cervical carcinoma in a septate uterus with double cervix: A case report and literature review.
    Gynecologic oncology reports· 2026· PMID 41809419mais citado
  2. Chronic pelvic pain secondary to venous congestion of longitudinal vaginal septum: a case report.
    AJOG global reports· 2025· PMID 41321664mais citado
  3. Complete Septate Uterus With Cervical Duplication and Vaginal Septum (U2b C2 V1): A Rare Müllerian Malformation With Diagnostic and Management Challenges.
    Cureus· 2025· PMID 40861647mais citado
  4. Laparoscopic transabdominal cerclage in a patient with a complete septate uterus and duplicated cervices.
    Fertility and sterility· 2025· PMID 40618985mais citado
  5. Surgical and reproductive outcomes of hysteroscopic metroplasty for complete septate uterus with or without associated cervical anomalies and/or vaginal septa.
    European journal of obstetrics, gynecology, and reproductive biology· 2025· PMID 40466400mais citado
  6. Standardized hysteroscopic management of complete septate uterus with duplicated cervices and longitudinal vaginal septum: A single-center experience.
    Int J Gynaecol Obstet· 2026· PMID 41981807recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:180126(Orphanet)
  2. MONDO:0015840(MONDO)
  3. GARD:20181(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55785763(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Útero totalmente septado
Compêndio · Raras BR

Útero totalmente septado

ORPHA:180126 · MONDO:0015840
CID-10
Q51.2 · Outra duplicação do útero
CID-11
MedGen
UMLS
C2957116
EuropePMC
Wikidata
Papers 10a
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