{{about|a type of uterine malformation in humans|the mammalian bicornuate uterus and other forms|Uterus}} {{Infobox medical condition (new) | name = Bicornuate uterus | synonyms = bicornate uterus | image = Uterus bicornis 1.png | caption = Two types of a human bicornuate uterus | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}
A '''bicornuate uterus''', or '''bicornate uterus''' (from the Latin ''cornū'', meaning "horn"), is a type of Müllerian anomaly in the human uterus, where there is a deep indentation at the fundus (top) of the uterus.
==Pathophysiology== A bicornuate uterus develops during embryogenesis. It occurs when the proximal (upper) portions of the paramesonephric ducts do not fuse, but the distal portions that develops into the lower uterine segment, cervix, and upper vagina fuse normally.<ref name=":0">{{Cite book|title=CURRENT Diagnosis & Treatment: Obstetrics & Gynecology|last=Bauman|first=D.|publisher=McGraw-Hill|year=2013|chapter=Pediatric & Adolescent Gynecology}}</ref>
== Diagnosis == [[File:Bicornuate uterus with pregnancy.jpg|thumb|Transvaginal ultrasonography showing a cross-section of a bicornuate uterus, with two cavities (or "horns") to the left and right, respectively. The one to the right contains a gestational sac.]] Diagnosis of bicornuate uterus typically involves imaging of the uterus with 2D or 3D ultrasound, hysterosalpingography, or magnetic resonance imaging (MRI). On imaging, a bicornuate uterus can be distinguished from a septate uterus by the angle between the cornua (intercornual angle): less than 75 degrees in a septate uterus, and greater than 105 degrees in a bicornuate uterus. Measuring the depth of the cleft between the cornua (fundal cleft) may also assist in diagnosis; a cleft of over {{Convert|1|cm|inch}} is indicative of bicornuate uterus.<ref name=":1" />
===Classification=== Bicornuate uterus is typically classified based on whether or not the division extends to the external cervical os. Bicornuate uteri with a division above the os are called ''bicornuate unicollis'' and those with a divided os are called ''bicornuate bicollis.''<ref name=":1">{{Cite book|title=Williams Obstetrics|publisher=McGraw-Hill|veditors=Cunningham F, Leveno KJ, Bloom SL, Dashe JS, Hoffman BL, Casey BM, Spong CY|edition=25|chapter=Congenital Genitourinary Abnormalities}}</ref> There is a continuous range of the degree and location of the fusion of the paramesonephric ducts, and existence of a spectrum, rather than a fixed number of types corresponding to strict medical definitions. Two processes that occur during the embryonic development of the paramesonephric ducts — fusion and reabsorption — can be affected to different degrees.<ref>{{cite journal |vauthors=Acién P, Acién M, Sánchez-Ferrer ML |title=Müllerian anomalies "without a classification": from the didelphys-unicollis uterus to the bicervical uterus with or without septate vagina |journal=Fertil. Steril. |volume= 91|issue= 6|pages= 2369–75|year=2008 |pmid=18367185 |doi=10.1016/j.fertnstert.2008.01.079 |doi-access=free }}</ref>
There is also a ''hybrid bicornuate uterus:'' External fundal depressions of variable depths associated with a septate uterus can be seen by laparoscopy, indicating the coexistence of the two anomalies. These cases are candidates for hysteroscopic metroplasty under appropriate sonographic and/or laparoscopic monitoring.<ref>{{cite journal | vauthors = El Saman AM, Shahin AY, Nasr A, Tawfik RM, Saadeldeen HS, Othman ER, Habib DM, Abdel-Aleem MA | date = Nov 2012 | title = Hybrid septate uterus, coexistence of bicornuate and septate varieties: a genuine report | journal = Journal of Obstetrics and Gynaecology Research | volume = 38 | issue = 11| pages = 1308–14 | doi = 10.1111/j.1447-0756.2012.01866.x | pmid = 22612567 | s2cid = 6177612 }}</ref>
An ''obstructed bicornuate uterus'' showing uni or bilateral obstruction might also be possible. The unilateral obstruction is more difficult to diagnose than the bilateral obstructive. A delay in the diagnosis can be problematic and compromise the reproductive abilities of those cases.<ref>{{cite journal | vauthors = El Saman AM, Nasr A, Tawfik RM, Saadeldeen HS | date = Aug 2011 | title = Müllerian duct anomalies: successful endoscopic management of a hybrid bicornuate/septate variety | journal = J Pediatr Adolesc Gynecol | volume = 24 | issue = 4| pages = e89–92 | doi = 10.1016/j.jpag.2011.02.013 | pmid = 21514191 }}</ref>
== Treatment == Bicornuate uterus typically requires no treatment.<ref name=":0" /> In those who do need treatment, metroplasty is the surgical correction of choice.<ref name=":1" /> Women who have recurrent miscarriage with no other explanation may benefit from surgery.<ref>{{Cite book|title=Williams Gynecology|veditors=Hoffman BL, Schorge JO, Bradshaw KD, Halvorson LM, Schaffer JI, Corton MM|edition=3|chapter=Anatomic Disorders}}</ref>
==Epidemiology== The occurrence of all types of paramesonephric duct abnormalities in women is estimated around 0.4%.<ref>{{cite journal |vauthors=Byrne J, Nussbaum-Blask A, Taylor WS |title=Prevalence of Müllerian duct anomalies detected at ultrasound |journal=Am. J. Med. Genet. |volume=94 |issue=1 |pages=9–12 |year=2000 |pmid=10982475 |doi=10.1002/1096-8628(20000904)94:1<9::AID-AJMG3>3.0.CO;2-H |display-authors=etal}}</ref> A bicornuate uterus occur in 0.4% of the general population.<ref>{{cite journal |last1=Chan |first1=Y.Y. |last2=Jayaprakasan |first2=K. |last3=Zamora |first3=J. |last4=Thornton |first4=J.G. |last5=Raine-Fenning |first5=N. |last6=Coomarasamy |first6=A. |title=The prevalence of congenital uterine anomalies in unselected and high-risk populations: a systematic review |journal=Human Reproduction Update |date=November 2011 |volume=17 |issue=6 |pages=761–771 |doi=10.1093/humupd/dmr028 |pmid=21705770 |pmc=3191936 }}</ref><ref>{{cite book |last1=Kaur |first1=Parneet |last2=Panneerselvam |first2=Deepan |title=StatPearls |date=2024 |publisher=StatPearls Publishing |chapter-url=https://www.ncbi.nlm.nih.gov/books/NBK560859/ |chapter=Bicornuate Uterus |pmid=32809694 }}</ref>
==In pregnancy== A bicornuate uterus is an indication for increased surveillance of a pregnancy, though most women with a bicornuate uterus are able to have healthy pregnancies.<ref name=":0" /> Women with a bicornuate uterus are at an increased risk of recurrent miscarriage,<ref name=":1" /><ref>{{cite journal |vauthors=Rackow BW, Arici A |title=Reproductive performance of women with müllerian anomalies |journal=Curr. Opin. Obstet. Gynecol. |volume=19 |issue=3 |pages=229–37 |year=2007 |pmid=17495638 |doi=10.1097/GCO.0b013e32814b0649 |s2cid=5476966 }}</ref> preterm birth,<ref name=":1" /><ref name=":2">{{cite journal|vauthors=Airoldi J, Berghella V, Sehdev H, Ludmir J|year=2005|title=Transvaginal ultrasonography of the cervix to predict preterm birth in women with uterine anomalies|journal=Obstet Gynecol|volume=106|issue=3|pages=553–6|doi=10.1097/01.AOG.0000173987.59595.e2|pmid=16135586|s2cid=22903707|doi-access=}}</ref> malpresentation,<ref name=":1" /><ref name=":3">{{cite journal|vauthors=Heinonen PK, Saarikoski S, Pystynen P|year=1982|title=Reproductive performance of women with uterine anomalies. An evaluation of 182 cases|journal=Acta Obstet Gynecol Scand|volume=61|issue=2|pages=157–62|doi=10.3109/00016348209156548|pmid=7113692|s2cid=72723061}}</ref> disruptions to fetal growth,<ref>{{Cite journal | url=http://pediatrics.aappublications.org/content/101/4/e10.short | doi=10.1542/peds.101.4.e10| title=Congenital Anomalies in the Offspring of Mothers with a Bicornuate Uterus| journal=Pediatrics| volume=101| issue=4| pages=e10| year=1998| last1=Martı́Nez-Frı́As| first1=María Luisa| last2=Bermejo| first2=Eva| last3=Rodrı́Guez-Pinilla| first3=Elvira| last4=Frı́As| first4=Jaime Luis| pmid=9521976| doi-access=| url-access=subscription}}</ref> premature rupture of membranes, placenta previa and retained placenta (which can lead to postpartum hemorrhage).<ref name=":4" /> This is due to the distortion of the normal shape of the uterus, distortion of the cervix leading to cervical insufficiency, or under-vascularization of the endometrium as the pregnancy requires more blood supply. In some cases, the nonpregnant horn can rupture during labor, necessitating emergency surgery.<ref name=":4">{{Cite book|title=Oxorn-Foote Human Labor & Birth, 6e|last=Pascali|first=Dante|year=2014|chapter=Uterus and Vagina}}</ref>
Fetuses developing in bicornuate uteri are more likely to present breech or transverse, with the fetal head in one horn and the feet in the other. This will often necessitate cesarean delivery. If the fetus is vertex (head down), the two horns may not contract in coordination, or the horn that does not contain the pregnancy may interfere with contractions and descent of the fetus, causing obstructed labor.<ref name=":4" />
==Effect on intrauterine device usage== Usage of intrauterine device (IUD) with copper requires one IUD in each horn to be effective in case of bicornuate uterus. The same practice is generally applied when using IUD with progestogen due to lack of evidence of efficacy with only one IUD.<ref name="Amies OelschlagerDebiec2013">{{cite journal|last1=Amies Oelschlager|first1=Anne-Marie|last2=Debiec|first2=Kate|last3=Micks|first3=Elizabeth|last4=Prager|first4=Sarah|title=Use of the Levonorgestrel Intrauterine System in Adolescents With Known Uterine Didelphys or Unicornuate Uterus|journal=Journal of Pediatric and Adolescent Gynecology|volume=26|issue=2|year=2013|pages=e58|issn=1083-3188|doi=10.1016/j.jpag.2013.01.029|doi-access=free}}</ref> Evidence is lacking regarding progestogen IUD usage for menorrhagia in bicornuate uterus, but a case report showed good effect with a single IUD.<ref>{{cite journal |vauthors=Acharya GP, Mills AM |title=Successful management of intractable menorrhagia with a levonorgestrel-releasing intrauterine device, in a woman with a bicornuate uterus |journal=J Obstet Gynaecol |volume=18 |issue=4 |pages=392–3 |date=July 1998 |pmid=15512123 |doi=10.1080/01443619867263 }}</ref>
== Uterus bicornis in mammals == {{See also|Uterus#Other animals}} thumb| A double uterus is the normal female anatomy in many mammals. In insectivores, certain species of primates i.e. lemurs, furthermore in carnivores, and in whales the uterus bicornis a part of their healthy morphological structures.<ref>Rüdiger Wehner, Walter Gehring: Zoologie. Georg Thieme Verlag Stuttgart/New York, 1990, S. 744-746</ref>
== Health issues == [[File:Bicornuate Uterus.jpg|thumb|Preparation of a uterus bicornis after removal of the left ovarian tumour and later hysterectomy.]]
The risk of cancer is ''not'' increased in the case of uterus bicornis. Endometrial carcinomas are very rare. If the presence of a uterus bicornis is not yet known and there is clinical suspicion of a carcinoma, which cannot be confirmed by the histological findings, sonography and magnetic resonance imaging provide diagnostic possibilities, as the cause may lie in a second uterine horn.<ref>{{cite journal | doi=10.3802/jgo.2009.20.3.195 | title=A single horn endometrial carcinoma of a uterus bicornis unicollis | year=2009 | last1=Dane | first1=Cem | last2=Tatar | first2=Zeynep | last3=Dane | first3=Banu | last4=Erqinbas | first4=Murat | last5=Cetin | first5=Ahmet | journal=Journal of Gynecologic Oncology | volume=20 | issue=3 | pages=195–197 | pmid=19809556 | pmc=2757567 }}</ref><ref>{{cite journal | doi=10.1016/j.jnci.2018.04.003 | title=Endometrial carcinoma in a single horn of a bicornuate uterus: A case report | year=2018 | last1=Gaballa | first1=Khaled | last2=Cicero | first2=Carla | last3=Gallotta | first3=Valerio | last4=Zannoni | first4=Gianfranco | last5=Scambia | first5=Giovanni | journal=Journal of the Egyptian National Cancer Institute | volume=30 | issue=2 | pages=81–83 | pmid=29779939 | s2cid=29167968 | doi-access=free }}</ref><ref>{{cite journal | doi=10.1080/15384047.2017.1281495 | title=Endometrial cancer with congenital uterine anomalies: 3 case reports and a literature review | year=2017 | last1=Gao | first1=Jinping | last2=Zhang | first2=Jintian | last3=Tian | first3=Wenyan | last4=Teng | first4=Fei | last5=Zhang | first5=Huiying | last6=Zhang | first6=Xuhong | last7=Wang | first7=Yingmei | last8=Xue | first8=Fengxia | journal=Cancer Biology & Therapy | volume=18 | issue=3 | pages=123–131 | pmid=28118070 | pmc=5389419 }}</ref><ref>{{cite journal | doi=10.4103/0973-1482.73322 | title=Endometrial adenocarcinoma involving both horns of a bicornuate uterus | year=2010 | last1=Shirley | first1=Sundersingh | last2=Devi | first2=Velusamisri | last3=Krishnamurthy | first3=Radha | last4=Nabhi | first4=Murtyvr | last5=Majhi | first5=Urmila | last6=Selvaluxmy | first6=Ganesharajah | journal=Journal of Cancer Research and Therapeutics | volume=6 | issue=3 | pages=304–306 | pmid=21119258 | doi-access=free }}</ref><ref>{{cite journal | doi=10.5468/ogs.2014.57.5.401 | title=Endometrial carcinoma arising in a bicornuate uterus | year=2014 | last1=Munkhdelger | first1=Jijgee | last2=Mia-Jan | first2=Khalilullah | last3=Cha | first3=Dong Soo | last4=Eom | first4=Minseob | journal=Obstetrics & Gynecology Science | volume=57 | issue=5 | pages=401–404 | pmid=25264532 | pmc=4175602 }}</ref> Approximately 15% of all ovarian tumours and 8% to 10% of epithelial ovarian tumours are mucinous cyst adenomas, which can also arise in a normally developed uterus.<ref>V. More, H. Warke, N. M. Mayadeo, M. N. Satia: ''[http://www.jpgo.org/2015/04/large-bilateral-mucinous-cystadenoma-of.html Large Bilateral Mucinous Cystadenoma Of Ovary]''. In: ''Journal of Postgraduate Gynecology & Obstetrics''. Volume 2, Issue 4, April 2015.</ref>
==References== {{Reflist}}
== External links == {{Medical resources | DiseasesDB = 33376 | ICD10 = Q51.3 | ICD9 = {{ICD9|752.34}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = }} {{Congenital malformations of genital organs and urinary system}}
{{DEFAULTSORT:Bicornuate Uterus}} Category:Congenital malformations of uterus and cervix