Craniopagus parasiticus

Drawing of a skull of a child born in 1783 in Bengal, affected by craniopagus parasiticus.

Drawing of the Boy of Bengal, affected by craniopagus parasiticus.

Craniopagus parasiticus is an extremely rare type of parasitic twinning occurring in about 4 to 6 of 10,000,000 births.^[1] 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 have been documented in the literature. Most infants with this condition are stillborn, or die shortly after birth.

Terminology

In the past, the use of terminology when describing parasitic twins 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, 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 to where it can live on its own.^[2]

History

Only ten cases of craniopagus parasiticus have been reported in the medical research literature. Of those cases, only three have survived birth.^[3] The first case on record is that of Everard Home's Two-Headed Boy of Bengal, whose head is now preserved at the Hunterian Museum at the Royal Society of Surgeons.

Development

The exact development of craniopagus parasiticus is not well known. However, it is known that the underdeveloped twin is a parasitic twin. Parasitic twins are known to occur in utero when monozygotic twins 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 and conjoined twins is that in parasitic twins, one twin, the parasite, stops development during gestation, whereas the other twin, the autosite, develops completely.^[4]

In normal monozygotic twin development, one egg is fertilized by a single sperm. The egg will then completely split into two, normally at the two-cell stage. If the egg splits in the early blastocyst stage, two inner cell masses will be present, eventually leading to the twins sharing the same chorion and placenta, but with separate amnions. However, the egg can split into two, but still have one blastocyst. This will lead to one inner cell mass and one blastocyst. Then, as the twins develop, they will share the same placenta, chorion, and amnion.^[5] This is thought to be the most likely reason why conjoined twins occur,^[5] 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.^[1] 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.^[1]

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.^[6]

The condition also seems to be more predominant in males than females, with seven of the nine (78%) reported cases being male. With the number of cases being so small, it is hard to say whether this data has any significance. However, it might provide some insight into the possibility of a genetic predisposition to the condition.

Treatment

With so few individuals actually surviving until birth, the only treatment option is surgery to try to remove the parasitic twin. Surgery, however, is very dangerous and has been successful only once. 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, and it has been suggested that cutting off the parasitic twin's arterial supply might improve the odds of the developed twin's survival.^[1]

Cases

Only ten cases of craniopagus parasiticus have been documented, out of a total of at least eighty known cases of craniopagus in general.^[3]

Only three cases have been documented by modern medicine to have survived birth:

• On December 10, 2003, Rebeca Martínez was born in the Dominican Republic 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.^[7]
• On February 19, 2005, 10-month-old Manar Maged underwent a successful 13-hour surgery in Egypt. The underdeveloped conjoined twin, Islaam, was attached to Manar's head and was facing upward. Islaam could smile and even blink, but doctors determined she had to be removed, and that she could not survive on her own.^[8] Manar was featured on an episode of The Oprah Winfrey Show and in the British documentary series Body Shock. Manar died fourteen months after the surgery, just days before her second birthday, due to a severe infection in her brain.^[9]
• An earlier case was the so-called "Two-Headed Boy of Bengal", who was born in 1783 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.^[3]

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.^[10]

Diprosopus

Diprosopus 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 features. Diprosopus can range from having two fully formed faces to just a duplication of the nose or eyes.

See also

• Pasqual Pinon

References

Bibliography

External links

• Girl Suffering from craniopagus parasiticus dies 1 year after surgery
• Image File of craniopagus parasiticus