View source for Cyclopia

{{Short description|Extreme form of holoprosencephaly}}
{{Use dmy dates|date=January 2025}}{{about|the congenital disorder}}
{{Infobox medical condition (new)
| name = Cyclopia
| synonyms = Cyclocephaly, synophthalmia, monophthalmia
| image = Cyclopia fetuses.jpg
| caption = Fetuses with cyclopia
| pronounce = 
| field = [[Medical genetics]]
| symptoms = 
| complications = 
| onset = During embryonic development
| duration = Lifelong
| types = 
| causes = 
| risks = 
| diagnosis = 
| differential = 
| prevention = 
| treatment = 
| medication = 
| prognosis = Invariably fatal
| frequency = 1 in 100,000 births
| deaths = 
}}
'''Cyclopia''' (named after the Greek mythology characters [[cyclopes]]), also known as '''alobar holoprosencephaly''', is the most extreme form of [[holoprosencephaly]] and is a [[congenital disorder]] (birth defect) characterized by the failure of the embryonic [[prosencephalon]] to properly divide the [[Orbit (anatomy)|orbits]] of the eye into two cavities. Its incidence is 1 in 16,000 in born animals and 1 in 200 in [[miscarriage|miscarried fetuses]].<ref>''Taber's Cyclopedic Medical Dictionary'', {{ISBN|0-8036-0654-0}}</ref><ref>[[Armand Marie Leroi|Leroi, Armand Marie]] ''Mutants: On Genetic Variety and the Human Body'', p.73. {{ISBN|978-0142004821}}</ref>

== Signs and symptoms ==
Typically, the nose is either missing or not functional. This deformity (called [[Proboscis (anomaly)|proboscis]]) forms above the center eye and is characteristic of a form of cyclopia called rhinencephaly or rhinocephaly.<ref>Dark, Graham (2007). [http://cancerweb.ncl.ac.uk/cgi-bin/omd?rhinocephaly Rhinocephaly]. In [http://cancerweb.ncl.ac.uk/cgi-bin/omd?query=&action=Home ''Online Medical Dictionary'']. Retrieved July 23, 2008.</ref> Most such embryos are either naturally [[miscarriage|miscarried]] or are [[stillbirth|stillborn]] upon [[childbirth|delivery]].

Although cyclopia is rare, several cyclopic human babies are preserved in medical museums (e.g. The [[University of Amsterdam|Vrolik Museum]], Amsterdam, Trivandrum Medical College).<ref>{{cite web|url=http://www.amc.uva.nl/index.cfm?pid=2668|title=Vrolik Museum, Department of Anatomy And Embryology, University of Amsterdam|url-status=dead|archive-url=https://web.archive.org/web/20071208132537/http://www.amc.uva.nl/index.cfm?pid=2668|archive-date=2007-12-08}}</ref>

Some extreme cases of cyclopia have been documented in farm animals (horses, sheep, pigs, goats, and sometimes chickens). In such cases, the nose and mouth fail to form, or the nose grows from the roof of the mouth, obstructing airflow and resulting in suffocation shortly after birth.<ref name="freakyface">{{cite web|url=http://www.messybeast.com/freak-face.htm |title=Feline Medical Curiosities: Facial Deformities |publisher=Messybeast.com |year=2007 |access-date=2008-11-29}}</ref>

== Causes ==
Genetic defects or toxins can misdirect the embryonic forebrain-dividing process.<ref name="medterms">{{cite web|url=http://www.medterms.com/script/main/art.asp?articlekey=15530|title=Cyclopia definition|work=Medical Dictionary|publisher=MedTerms|access-date=2008-11-29|archive-date=2013-11-02|archive-url=https://web.archive.org/web/20131102213304/http://www.medterms.com/script/main/art.asp?articlekey=15530|url-status=dead}}</ref> One highly [[teratogenic]] alkaloid toxin that can cause cyclopia is [[cyclopamine]] or 2-deoxyjervine, found in the plant ''[[Veratrum californicum]]'' (also known as corn lily or false hellebore). Grazing animals are most likely to ingest this plant and induce cyclopia in offspring. People sometimes accidentally ingest false hellebore while pregnant thinking it is [[hellebore]], which has been suggested as a "natural" treatment for [[vomiting]], [[cramp]]s, and poor circulation – three conditions which may be present in the early stages of pregnancy.<ref>{{cite web|url=http://www.teratology.org/members/JMFpresentation/tsld011.htm|title=Teratology Society|access-date=2007-09-24|archive-url=https://web.archive.org/web/20071218093222/http://www.teratology.org/members/JMFpresentation/tsld011.htm|archive-date=2007-12-18|url-status=dead}}</ref> Cyclopia occurs  when [[Holoprosencephaly#Genetics|certain proteins are inappropriately expressed]],<ref>{{Cite journal|title=Mutations in the C-terminal domain of Sonic Hedgehog cause holoprosencephaly|journal=Human Molecular Genetics|volume=6|issue=11|pages=1847–1853|author1=Erich Roessler |author2=Elena Belloni |author3= Karin Gaudenz |author4= Fernando Vargas |author5=Stephen W. Scherer |author6= Lap-Chee Tsui |author7=Maximilian Muenke |name-list-style=amp |year=1997|doi=10.1093/hmg/6.11.1847|pmid=9302262|doi-access=free}}</ref> causing the brain to stay whole, rather than developing two distinct hemispheres. This leads to the fetus having one [[Midbrain#Corpora quadrigemina|optic lobe]] and one [[Olfactory bulb|olfactory lobe]], resulting in the eye and nose malformations of cyclopia.<ref>{{cite journal|title=Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function|journal=Nature|author1=Chin Chiang |author2=Ying Litingtung |author3=Eric Lee |author4=Keith E. Young |author5=Jeffrey L Corden |author6=Heiner Westphal |author7=Philip A. Beachy | doi=10.1038/383407a0 | volume=383 |issue=6599|pmid=8837770 | pages=407–413|year=1996|bibcode=1996Natur.383..407C|s2cid=4339131}}</ref>

The [[Sonic hedgehog protein]] (SHH) is the gene regulator involved in the separation of the single eye field into two bilateral fields.<ref>{{cite book |last= Carlson |first= Bruce |year= 2014 |title= Human Embryology and Developmental Biology |edition = 5th |publisher= Elsevier |page= 309 |isbn= 978-1-4557-2794-0}}</ref> 
Although not proven, it is thought that SHH emitted from the prechordal plate suppresses [[Pax6]], which causes the eye field to divide into two. If the SHH gene is mutated, the result is cyclopia, a single eye in the center of the face (Gilbert, 2000).

== Notable cases ==
* A [[United Kingdom|British]] description from 1665 of a [[Colt (horse)|colt]] that appeared to have cyclopia reads:{{Blockquote|text=''First'', That it had no sign of any ''Nose'' in the usual place, nor had it any, in any other place of the Head, unless the double Bagg CC that grew out of the midst of the forehead, were some rudiment of it. ''Next'', That the ''two Eyes'' were united into one ''Double Eye'' which was placed just in the middle of the Brow.<ref>{{cite journal |date = 3 July 1665 |title = Observables upon a Monstrous Head | journal = Philosophical Transactions of the Royal Society |volume = 1 |issue = 5 |pages = 85–86 |jstor=101436 |doi=10.1098/rstl.1665.0037 |bibcode = 1665RSPT....1...85. |s2cid = 186213738 }}{{gutenberg|28758 |Volume 1 – 1666}}</ref>}}
* In October 1766, an infant in France was born with cyclopia, living for only a few hours. Reports of the case were made in the ''[[Mercure de France]]'' and an illustration of the infant was made by [[Marie Marguerite Bihéron|Marie Bihéron]]. The case was also mentioned in Volume IV of [[Georges-Louis Leclerc, Comte de Buffon|Buffon]]'s ''L'Histoire Naturelle''.
[[File:Cyclopia1793.png|thumb|A Swedish description from 1793 of a newborn with cyclopia]]
* On 1 March 1793, a 46-year-old woman in Boalts Torp, [[Glimåkra]], [[Sweden]] gave birth to a child with cyclopia that died after two hours. The child was 35&nbsp;cm long, its face without nose and nostrils, and its lidless eye with no eyebrow sat raised on the middle of its forehead like a large blueberry. The wrists were somewhat crooked as well as the right foot which was completely crooked and bent inwards. It was not clear whether it was a boy or a girl, but it was believed to be the former.<ref>{{cite book |author= <!--Staff writer(s); no by-line.--> |title= Kyrkbok |location= [[Glimåkra]] |volume= CI:3 |series= 1752–1800 |chapter= Födde |pages= 174–175 |year= 1793 |trans-chapter=Birth |trans-title=[[Parish Register]] |language= sv }}</ref>
* In November 1889, reports emerged from [[Umatilla County, Oregon]], of a [[Nez Perce]] child with one eye in the center of the forehead. The child was reportedly healthy and "able to see with ease, and ran about with as much freedom as any of his companions."<ref>{{Cite news |last=Humanities |first=National Endowment for the |date=1889-11-22 |title=The state rights democrat. [volume] (Albany, Linn County, Oregon) 1865-1900, November 22, 1889, Image 3 |url=https://chroniclingamerica.loc.gov/lccn/sn84022644/1889-11-22/ed-1/seq-3/ |access-date=2023-04-30 |issn=2470-9085}}</ref><ref>{{Cite news |last=Humanities |first=National Endowment for the |date=1889-11-22 |title=The Corvallis gazette. [volume] (Corvallis, Or.) 1862-1899, November 22, 1889, Image 4 |url=https://chroniclingamerica.loc.gov/lccn/sn84022650/1889-11-22/ed-1/seq-4/ |access-date=2023-04-30 |issn=2472-4416}}</ref><ref>{{Cite news |last=Humanities |first=National Endowment for the |date=1889-11-26 |title=The Anaconda standard. [volume] (Anaconda, Mont.) 1889-1970, November 26, 1889, Morning, Image 8 |pages=8 |url=https://chroniclingamerica.loc.gov/lccn/sn84036012/1889-11-26/ed-1/seq-8/ |access-date=2023-04-30 |issn=2163-4483}}</ref> Several individuals tried to locate the child to be put on display, but the tribe had left to hunt in the mountains by the time they arrived.<ref>{{Cite news |last=Humanities |first=National Endowment for the |date=1889-11-28 |title=The Yakima herald. [volume] (North Yakima, W.T. [Wash.]) 1889-1914, November 28, 1889, Image 1 |url=https://chroniclingamerica.loc.gov/lccn/sn88085523/1889-11-28/ed-1/seq-1/ |access-date=2023-04-30 |issn=2158-4745}}</ref> It is unknown what became of the child.
* On December 28, 2005, a kitten with cyclopia, "Cy", was born in [[Redmond, Oregon]], [[United States]] and died about one day after birth.<ref name=cy>{{cite web |last = Petty |first = Terrence |title = Not a Hoax, One-Eyed Kitten Had Bizarre Condition |work=Animaldomain |publisher=LiveScience.com |date=January 11, 2006 |url=http://www.livescience.com/animalworld/060111_ap_cyclops_cat.html |archive-url=https://web.archive.org/web/20060203010920/http://www.livescience.com/animalworld/060111_ap_cyclops_cat.html |archive-date = 2006-02-03 |access-date = 2014-03-07 }}</ref>
* In 2006, a baby girl in India with cyclopia was born. Her only eye was in the center of her forehead. She did not have a nose and her brain did not separate into two separate hemispheres ([[holoprosencephaly]]).<ref name="Cyclops Baby">{{cite web |title=Cyclops Baby |url=http://www.oddee.com/item_93761.aspxb |publisher=Oddee |access-date=1 October 2012 |date=2008-02-28}}</ref> The child died one day after her birth.<ref name="Cyclops Baby Born in India Only Survives 1 Day">{{cite web |title=Cyclops Baby Born in India Only Survives 1 Day |url=http://www.aolnews.com/2011/03/24/baby-with-1-eye-and-no-nose-born-in-india-survives-1-day/ |publisher=AOL News |access-date=1 October 2012 |url-status=dead |archive-url=https://web.archive.org/web/20120919173046/http://www.aolnews.com/2011/03/24/baby-with-1-eye-and-no-nose-born-in-india-survives-1-day/ |archive-date=19 September 2012}}</ref>
* In 2011, an [[Albinism|albino]] cyclops shark fetus was discovered in the body of a caught shark in Mexico, with no discernible nose and one giant eye. The unborn fetus was turned over for medical studies.<ref>{{cite web|url=http://news.nationalgeographic.com/news/2011/10/pictures/111013-shark-albino-one-eyed-fetus/ |archive-url=https://archive.today/20140307095739/http://news.nationalgeographic.com/news/2011/10/pictures/111013-shark-albino-one-eyed-fetus/ |url-status=dead |archive-date=March 7, 2014 |title=Pictures: Rare "Cyclops" Shark Found |publisher=News.nationalgeographic.com |date=2011-10-13 |access-date=2014-03-07}}</ref>
* On October 10, 2012, a small kitten was born. Its eye was in the center of the forehead and there was no developed nose to be found. The small cat died shortly after it was born. It was nicknamed Cleyed the Cyclops.<ref name=cleyed>{{cite web |title=Viewers pitied Cleyed, the one-eyed kitten |url=http://doubtfulnews.com/2012/10/viewers-pitied-cleyed-the-one-eyed-kitten |access-date=2012-10-17 |archive-url=https://web.archive.org/web/20121212015103/http://doubtfulnews.com/2012/10/viewers-pitied-cleyed-the-one-eyed-kitten/ |archive-date=2012-12-12 |url-status=dead }}</ref>
* On May 10, 2017, in [[Assam, India]], a black goat was born with one eye and other cyclopia-related facial abnormalities. It was reported to still be alive over a week later, which is unusual for this condition.<ref>{{cite web | url=https://www.expressen.se/tv/omtalat/geten-har-ett-stort-jatteoga--markliga-cyklopen-vordas-som-helig/ | title=Geten har ett stort jätteöga- märkliga cyklopen vördas som helig | date=28 May 2017 }}</ref>
*On September 13, 2018, in [[Mandailing Natal Regency|Mandailing Natal]], [[North Sumatra]], [[Indonesia]], a baby with cyclopia was born without a nose and one eye with the weight of 2.4kg (5.3lb) and heart rate under 100 bpm. The child died seven hours after birth.<ref>{{cite web | url=https://regional.kompas.com/read/2018/09/14/07150041/bertahan-hidup-7-jam-bayi-cyclopia-hembuskan-nafas-terakhir | title=Bertahan Hidup 7 Jam, Bayi "Cyclopia" Hembuskan Nafas Terakhir| date=2018-09-14}}</ref>
*On March 16, 2022, a boy was born in [[Al Bayda, Yemen]], with cyclopia. He died 7 hours after birth. He was seen in photos taken by Yemeni journalist Karim Zarai.<ref>{{Cite web |date=2022-03-19 |title=One-eyed baby born in Yemen dies 7 hours later |url=https://gulfnews.com/world/gulf/yemen/one-eyed-baby-born-in-yemen-dies-7-hours-later-1.86570881 |access-date=2023-08-17 |website=gulfnews.com |language=en}}</ref>
*In March  2024, a pig was born in [[Ilocos Sur]], [[Philippines]], with cyclopia.<ref>{{cite web |title=Cyclops pig, isinilang sa Ilocos Sur |url=https://www.gmanetwork.com/news/balitambayan/umg/901009/cyclops-pig-isinilang-sa-ilocos-sur/story/}}</ref>
*In December 2024, the Korean Society of Perinatology documented a female infant with cyclopia who survived for 8 months on palliative care, which is the longest known to date. The baby girl was born as part of a twin birth, with the other twin being reportedly normal. <ref>{{Cite journal |last1=Lee |first1=Yejun |last2=Yang |first2=Misun |last3=Ahn |first3=So Yoon |last4=Sung |first4=Se In |last5=Chang |first5=Yun Sil |date=2024-12-31 |title=A Case Report of Alobar Holoprosencephaly with Cyclopia and Arrhinia: The Longest-Surviving Infant without Life-Sustaining Interventions|url=https://e-kjp.org/DOIx.php?id=10.14734/PN.2024.35.4.146 |access-date=2025-08-24 |journal=Perinatology |volume=35 |issue=4 |page=146 |doi=10.14734/PN.2024.35.4.146 |language=en|doi-access=free }}</ref>

== Cultural significance ==

The [[Islamic State]] used photos of babies born with cyclopia in its recruitment campaign. ISIS claimed the photos depicted [[Masih ad-Dajjal]], who according to the Hadith, would have only one eye. Mainstream Islamic scholars consider the prophecy as referring to a one-eyed man, not a cyclops.<ref name="isil">{{cite web|url=https://www.ibtimes.co.uk/isis-use-picture-cyclops-baby-recruit-fighters-apocalyptic-battle-1465323|title=Isis Use Picture of 'Cyclops Baby' to Recruit Fighters for Apocalyptic Battle|date=13 September 2014 }}</ref> One infant whose image was circulated in 2014 was claimed to be [[Israel|Israeli]], but was actually [[Bolivia|Bolivian]] and from 2008. The baby girl from India born in 2006 (see notable cases above) also had her image used.<ref>{{Cite web |last=Porter |first=Tom |date=2014-09-13 |title=Isis Use Picture of 'Cyclops Baby' to Recruit Fighters for Apocalyptic Battle |url=https://www.ibtimes.co.uk/isis-use-picture-cyclops-baby-recruit-fighters-apocalyptic-battle-1465323 |access-date=2023-08-17 |website=International Business Times UK |language=en}}</ref>

== Gallery ==
=== Humans ===
<gallery>
Janus head or two-faced man Wellcome L0032588.jpg|1616, [[Italy]]
Licetus, De monstrorum caussis,...1634 Wellcome L0007741.jpg|1634, Italy
An infant with one central eye. Engraving. Wellcome V0007405.jpg|1766, [[France]]
B. C. Hirst & G. A. Piersol, Human monstrosities. Wellcome L0027960.jpg|1893, [[United States]]
Pediatrics. (1900) (14578802990).jpg|1900, United States
Roland Cyclopie 2.jpg|1909, France
Cyclopia 1914.jpg|1912, [[United Kingdom]]
Cyclopia front.jpg|1920, United States
Cyclopia.JPG|1926, [[Egypt]]
Cephalothoracopagus Janiceps 1.jpg|1950, [[India]]
Cyclopia 1.jpg|1968, United States
Patauface.PNG|[[Patau syndrome]]
</gallery>

=== Animals ===
<gallery>
Les écarts de la nature page20.jpg|[[Cat]], 1775
Les écarts de la nature page10.jpg|[[Horse]], 1775
Les écarts de la nature page42.jpg|[[Pig]] from [[Martinique]], 1775 
ABNORMALITIES; Tabulae ad illustrandam embry Wellcome L0032361.jpg|[[Goat]], 1849
Dog with congenital defects. Lithograph. Wellcome V0022910EBL.jpg|[[Dog]]
Head of a donkey with a congenitally deformed head. Lithogra Wellcome V0022905EL.jpg|[[Donkey]]
Cyclopelamb2.jpg|[[Sheep]] with ''in utero'' [[cyclopamine]] exposure
BLW Cyclopian Pig.jpg|Pig in [[Hunterian Museum]]
Chat cyclope - anatomie comparé mnhn Paris.JPG|Cat in [[National Museum of Natural History, France|National Museum of Natural History]]
Cyclopic Lissachatina immaculata.JPG|''[[Lissachatina immaculata]]''
</gallery>

== See also ==
* [[Anophthalmia]]
* [[Hedgehog pathway]]

== References ==
{{reflist}}

== External links ==
{{Commonscatinline}}
{{Medical resources
|  DiseasesDB =
|  ICD10 = {{ICD10|Q|87|0|q|80}}
|  ICD9 = {{ICD9|759.89}}
|  ICDO =
|  OMIM =
|  MedlinePlus =
|  eMedicineSubj =
|  eMedicineTopic =
|  MeshID =
}}
{{Phakomatoses and other congenital malformations not elsewhere classified}}

[[Category:Congenital disorders]]
[[Category:Absent body parts]]
[[Category:Congenital disorders of eyes]]