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Craniopagus parasiticus is an extremely rare type of
parasitic twin A parasitic twin, also known as an asymmetrical or unequal conjoined twin, is the result of the processes that also produce vanishing twins and conjoined twins, and may represent a continuum between the two. Parasitic twins occur when a twin embr ...
ning occurring in about 2 to 3 of 5,000,000 births. In craniopagus parasiticus, a parasitic twin head with an undeveloped body is attached to the head of a developed twin. Fewer than a dozen cases of this type of
conjoined twin Conjoined twins – sometimes popularly referred to as Siamese twins – are twins joined ''in utero''. A very rare phenomenon, the occurrence is estimated to range from 1 in 49,000 births to 1 in 189,000 births, with a somewhat higher incidence ...
have been documented in literature.


Development

The exact development of craniopagus parasiticus is not well known. However, it is known that the underdeveloped twin is a
parasitic twin A parasitic twin, also known as an asymmetrical or unequal conjoined twin, is the result of the processes that also produce vanishing twins and conjoined twins, and may represent a continuum between the two. Parasitic twins occur when a twin embr ...
. Parasitic twins are known to occur ''in utero'' when
monozygotic twins Twins are two offspring produced by the same pregnancy.MedicineNet > Definition of TwinLast Editorial Review: 19 June 2000 Twins can be either ''monozygotic'' ('identical'), meaning that they develop from one zygote, which splits and forms two em ...
start to develop as an embryo, but the embryo fails to completely split. When this happens, one embryo will dominate development, while the other's development is severely altered. The key difference between a
parasitic twin A parasitic twin, also known as an asymmetrical or unequal conjoined twin, is the result of the processes that also produce vanishing twins and conjoined twins, and may represent a continuum between the two. Parasitic twins occur when a twin embr ...
and
conjoined twins Conjoined twins – sometimes popularly referred to as Siamese twins – are twins joined ''Uterus, in utero''. A very rare phenomenon, the occurrence is estimated to range from 1 in 49,000 births to 1 in 189,000 births, with a somewhat higher in ...
is that in parasitic twins, one twin, the
parasite Parasitism is a close relationship between species, where one organism, the parasite, lives on or inside another organism, the host, causing it some harm, and is adapted structurally to this way of life. The entomologist E. O. Wilson h ...
, stops development during
gestation Gestation is the period of development during the carrying of an embryo, and later fetus, inside viviparous animals (the embryo develops within the parent). It is typical for mammals, but also occurs for some non-mammals. Mammals during pr ...
, whereas the other twin, the autosite, develops completely. In normal monozygotic twin development, one egg is fertilized by a single
sperm Sperm is the male reproductive cell, or gamete, in anisogamous forms of sexual reproduction (forms in which there is a larger, female reproductive cell and a smaller, male one). Animals produce motile sperm with a tail known as a flagellum, ...
. The egg will then completely split into two, normally at the two-cell stage. If the egg splits in the early
blastocyst The blastocyst is a structure formed in the early embryonic development of mammals. It possesses an inner cell mass (ICM) also known as the ''embryoblast'' which subsequently forms the embryo, and an outer layer of trophoblast cells called th ...
stage, two inner cell masses will be present, eventually leading to the twins sharing the same
chorion The chorion is the outermost fetal membrane around the embryo in mammals, birds and reptiles (amniotes). It develops from an outer fold on the surface of the yolk sac, which lies outside the zona pellucida (in mammals), known as the vitell ...
and
placenta The placenta is a temporary embryonic and later fetal organ that begins developing from the blastocyst shortly after implantation. It plays critical roles in facilitating nutrient, gas and waste exchange between the physically separate mate ...
, but with separate
amnion The amnion is a membrane that closely covers the human and various other embryos when first formed. It fills with amniotic fluid, which causes the amnion to expand and become the amniotic sac that provides a protective environment for the develo ...
s. However, the egg can split into two, but still have one blastocyst. This will lead to one
inner cell mass The inner cell mass (ICM) or embryoblast (known as the pluriblast in marsupials) is a structure in the early development of an embryo. It is the mass of cells inside the blastocyst that will eventually give rise to the definitive structures of th ...
and one blastocyst. Then, as the twins develop, they will share the same
placenta The placenta is a temporary embryonic and later fetal organ that begins developing from the blastocyst shortly after implantation. It plays critical roles in facilitating nutrient, gas and waste exchange between the physically separate mate ...
,
chorion The chorion is the outermost fetal membrane around the embryo in mammals, birds and reptiles (amniotes). It develops from an outer fold on the surface of the yolk sac, which lies outside the zona pellucida (in mammals), known as the vitell ...
, and amnion. This is thought to be the most likely reason why
conjoined twins Conjoined twins – sometimes popularly referred to as Siamese twins – are twins joined ''Uterus, in utero''. A very rare phenomenon, the occurrence is estimated to range from 1 in 49,000 births to 1 in 189,000 births, with a somewhat higher in ...
occur, and could possibly play a role in the development of craniopagus parasiticus. One hypothesis is that craniopagus parasiticus starts with the development of two fetuses from a single zygote that fail to separate at the head region around the second week of gestation. Some believe, however, that it occurs later in development, around the fourth week of gestation, at which time the two embryos fuse together near the anterior open neuropore. Another hypothesis is that there is joining of the somatic and placental vascular system of the twins, as well as a degeneration of the umbilical cord of the parasitic twin. This suggests that craniopagus parasiticus develops due to the lack of blood supply to one of the twins.


Diagnosis


Related conditions

In addition to craniopagus parasiticus, a few other conditions involve a child's, or animal's, birth with two heads or faces.


Dicephalic parapagus

Dicephalic parapagus is a condition where two heads are side by side on a single torso, with varying degrees of twinning of organs and structures within the torso.


Diprosopus

Diprosopus Diprosopus ( el, διπρόσωπος, "two-faced", from , , "two" and , euter "face", "person"; with Latin ending), also known as craniofacial duplication (cranio- from Greek , "skull", the other parts Latin), is an extremely rare congenital di ...
occurs when a single body and neck are present, but there is a duplication of the structures of the face. This is different from craniopagus parasiticus in that there is only one head, although there is a duplication of the
craniofacial Craniofacial (''cranio-'' combining form meaning head or skull + ''-facial'' combining form referring to the facial structures grossly) is an adjective referring to the parts of the head enclosing the brain and the face. The term is typically used ...
features. Diprosopus can range from having two fully formed faces to just a duplication of the nose or eyes. Cats with the condition are referred to as ' Janus cats'. A cat named Frankenlouie was a famous example noted by the ''
Guinness Book of World Records ''Guinness World Records'', known from its inception in 1955 until 1999 as ''The Guinness Book of Records'' and in previous United States editions as ''The Guinness Book of World Records'', is a reference book published annually, listing world ...
'' in 2012 for being the longest surviving Janus cat.


Treatment

Few individuals survive until birth. For those who do, the only treatment available is to surgically remove the parasitic twin. Of the two documented attempts, however, one child died within hours and neither reached their second birthday. The problem with surgical intervention is that the arterial supplies of the head are so intertwined that it is very hard to control the bleeding, but it has been suggested that cutting off the parasitic twin's arterial supply might improve the odds of the developed twin's survival.


Prevalence

Only ten cases of craniopagus parasiticus have been documented, out of a total of at least eighty known cases of craniopagus in general. Only four cases have been documented by modern medicine to have survived birth: *An earlier case was the so-called "Two-Headed Boy of Bengal", who was born in 1783 at the village of Mandal Ghat in the New Jalpaiguri district of the Indian state of West Bengal and died of a cobra bite in 1787. His skull remains in the collection of the Hunterian Museum of the
Royal College of Surgeons of London The Royal College of Surgeons of England (RCS England) is an independent professional body and registered charity that promotes and advances standards of surgical care for patients, and regulates surgery and dentistry in England and Wales. ...
. *On December 10, 2003, Rebeca Martínez was born in the
Dominican Republic The Dominican Republic ( ; es, República Dominicana, ) is a country located on the island of Hispaniola in the Greater Antilles archipelago of the Caribbean region. It occupies the eastern five-eighths of the island, which it shares with ...
with this rare condition. She was the first baby born with the condition to undergo an operation to remove the second head. She died on February 7, 2004, after an 11-hour operation. *On March 30, 2004, Manar Maged was born with the rare condition. On February 19, 2005, 10-month-old Manar underwent a successful 13-hour surgery in
Egypt Egypt ( ar, مصر , ), officially the Arab Republic of Egypt, is a List of transcontinental countries, transcontinental country spanning the North Africa, northeast corner of Africa and Western Asia, southwest corner of Asia via a land bridg ...
. The underdeveloped conjoined twin, Islaam, was attached to Manar's head and was facing upward. Islaam could blink and even smile, but doctors determined she had to be removed, and that she could not survive on her own. Manar was featured on an episode of ''
The Oprah Winfrey Show ''The Oprah Winfrey Show'', often referred to as ''The Oprah Show'' or simply ''Oprah'', is an American daytime syndicated talk show that aired nationally for 25 seasons from September 8, 1986, to May 25, 2011, in Chicago, Illinois. Produced ...
'' and in the British documentary series ''
Body Shock ''Body Shock'' (also: Bodyshock) is a British medical documentary series about the conditions or lives of extraordinary people. It was originally produced by redback for Channel 4 in the UK, but in September 2006, it was taken over by ArkMedia. ...
''. Manar died on March 26, 2006, fourteen months after the surgery, just days before her second birthday, due to a severe infection in her brain. * On January 20, 2021, a baby was born with two heads, at the Elias Hospital in
Bucharest Bucharest ( , ; ro, București ) is the capital and largest city of Romania, as well as its cultural, industrial, and financial centre. It is located in the southeast of the country, on the banks of the Dâmbovița River, less than north o ...
,
Romania Romania ( ; ro, România ) is a country located at the crossroads of Central Europe, Central, Eastern Europe, Eastern, and Southeast Europe, Southeastern Europe. It borders Bulgaria to the south, Ukraine to the north, Hungary to the west, S ...
, but died some hours after being born.


History

Only ten cases of craniopagus parasiticus have been reported in the medical research literature. Of those cases, only four have survived birth. The first case on record is that of Everard Home's Two-Headed Boy of Bengal, whose skull is preserved at the Hunterian Museum at the Royal Society of Surgeons.


Terminology

In the past, the use of terminology when describing
parasitic twins A parasitic twin, also known as an asymmetrical or unequal conjoined twin, is the result of the processes that also produce vanishing twins and conjoined twins, and may represent a continuum between the two. Parasitic twins occur when a twin embry ...
has been somewhat inconsistent. By definition, a parasitic twin is joined to another twin in a certain anatomical location or position on the developed twin's body. The underdeveloped twin is termed the
parasite Parasitism is a close relationship between species, where one organism, the parasite, lives on or inside another organism, the host, causing it some harm, and is adapted structurally to this way of life. The entomologist E. O. Wilson h ...
, and the developed twin is termed the autosite. The autosite can have some abnormalities, as well. For the most part, however, it has developed enough that it can live on its own.


See also

*
Pasqual Piñón Pasqual Piñón (1889–1929), known as The Two-Headed Mexican, was a performer with the Sells-Floto Circus in the early 1900s. A railroad worker from Texas, Piñón was discovered by a sideshow promoter whose attention had been caught by a large ...
, a sideshow performer billed as the ''Two-headed Mexican'' *
Janus In ancient Roman religion and myth, Janus ( ; la, Ianvs ) is the god of beginnings, gates, transitions, time, duality, doorways, passages, frames, and endings. He is usually depicted as having two faces. The month of January is named for Jan ...
, ancient Roman god depicted with two faces * Edward Mordrake, an urban legend with a similar trait *
Futakuchi-onna A is a type of yōkai or Japanese monster. They are characterized by their two mouths – a normal one located on her face and a second one on the back of the head beneath the hair. There, the woman's skull splits apart, forming lips, teet ...
, Japanese yōkai with a similar trait *
Tomie Kawakami Tomie Kawakami, better known mononymously as Tomie, is a character from the Japanese horror manga and film series of the same name created by Junji Ito. Tomie was introduced in Ito's 1987 manga ''Tomie'', which was published in ''Monthly Halloween ...
, a titular character of
Tomie is a Japanese horror manga series written and illustrated by Junji Ito. ''Tomie'' was Ito's first published work he originally submitted to '' Monthly Halloween'', a ''shōjo'' magazine in 1987, which led to him winning the Kazuo Umezu ...
whom possess with the two-headed appearance


References


Bibliography

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External links


Image File of craniopagus parasiticus
{{Situs inversus and conjoined twins Congenital disorders Parasitic twin Rare diseases