Conjoined Twins

Conjoined twins are identical twins joined in utero. An extremely 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 in Southwest Asia and Africa. Approximately half are stillborn, and an additional one-third die within 24 hours. Most live births are female, with a ratio of 3:1.

Two contradicting theories exist to explain the origins of conjoined twins. The more generally accepted theory is fission, in which the fertilized egg splits partially. The other theory, no longer believed to be the basis of conjoined twinning, is fusion, in which a fertilized egg completely separates, but stem cells (which search for similar cells) find similar stem cells on the other twin and fuse the twins together. Conjoined twins share a single common chorion, placenta, and amniotic sac, although these characteristics are not exclusive to conjoined twins, as there are some monozygotic but non-conjoined twins who also share these structures in utero.

The most famous pair of conjoined twins was Chang and Eng Bunker (Thai: อิน-จัน, In-Chan) (1811–1874), Thai brothers born in Siam, now Thailand. They traveled with P.T. Barnum’s circus for many years and were labeled as the Siamese twins. Chang and Eng were joined at the torso by a band of flesh, cartilage, and their fused livers. In modern times, they could have been easily separated. Due to the brothers’ fame and the rarity of the condition, the term “Siamese twins” came to be used as a synonym for conjoined twins.

Causes
There are two theories about the development of conjoined twins. The first is that a single fertilized egg does not fully split during the process of forming identical twins. The second theory is that a fusion of two fertilized eggs occurs earlier in development. Although conjoined twinning has not been linked to any environmental or genetic cause, they occur so rarely it has not been possible to draw firm conclusions.

Management
Separation
Surgery to separate conjoined twins may range from very easy to very difficult depending on the point of attachment and the internal parts that are shared. Most cases of separation are extremely risky and life-threatening. In many cases, the surgery results in the death of one or both of the twins, particularly if they are joined at the head or share a vital organ. This makes the ethics of surgical separation, where the twins can survive if not separated, contentious. Alice Dreger of Northwestern University found the quality of life of twins who remain conjoined to be higher than is commonly supposed. Lori and George Schappell and Abby and Brittany Hensel are notable examples.

The first record of separating conjoined twins took place in the Byzantine Empire in the 900s. One of the conjoined twins had already died, so surgeons attempted to separate the dead twin from the surviving twin. The result was partly successful as the remaining twin lived for three days after separation. The next case of separating conjoined twins was recorded in 1689 in Germany several centuries later. The first recorded successful separation of conjoined twins was performed in 1689 by Johannes Fatio. In 1955, neurosurgeon Harold Voris (1902-1980) and his team at Mercy Hospital in Chicago performed the first successful operation to separate craniopagus twins (conjoined at the head), which resulted in long-term survival for both. The larger girl was reported in 1963 as developing normally, but the smaller was permanently impaired.

In 1957, Bertram Katz and his surgical team made international medical history performing the world’s first successful separation of conjoined twins sharing a vital organ. Omphalopagus twins John Nelson and James Edward Freeman (Johnny and Jimmy) were born in Youngstown, Ohio, on April 27, 1956. The boys shared a liver but had separate hearts and were successfully separated at North Side Hospital in Youngstown, Ohio, by Bertram Katz. The operation was funded by the Ohio Crippled Children’s Service Society.

Recent successful separations of conjoined twins include that of the separation of Ganga and Jamuna Shreshta in 2001, who were born in Kathmandu, Nepal, in 2000. The 197-hour surgery on the pair of craniopagus twins was a landmark one which took place in Singapore; the team was led by neurosurgeons Chumpon Chan and Keith Goh. The surgery left Ganga with brain damage and Jamuna unable to walk. Seven years later, Ganga Shrestha died at the Model Hospital in Kathmandu in July 2009, at the age of 8, three days after being admitted for treatment of a severe chest infection.

Infants Rose and Grace (“Mary” and “Jodie”) Attard, conjoined twins from Malta, were separated in Great Britain by court order Re A (Children) (Conjoined Twins: Surgical Separation) over the religious objections of their parents, Michaelangelo and Rina Attard. The twins were attached at the lower abdomen and spine. The surgery took place in November, 2000, at St Mary’s Hospital in Manchester. The operation was controversial because Rose, the weaker twin, would die as a result of the procedure as her heart and lungs were dependent upon Grace’s. However, if the operation had not taken place, it was certain that both twins would die. Grace survived to enjoy a normal childhood.

In 2003, two 29-year-old women from Iran, Ladan and Laleh Bijani, who were joined at the head but had separate brains (craniopagus) were surgically separated in Singapore, despite surgeons’ warnings that the operation could be fatal to one or both. Their complex case was accepted only because technologically advanced graphical imagery and modelling would allow the medical team to plan the risky surgery. Unfortunately, an undetected major vein hidden from the scans was discovered during the operation. The separation was completed but both women died while still in surgery.

Types
Conjoined twins are typically classified by the point at which their bodies are joined. The most common types of conjoined twins are:

Thoraco-omphalopagus (28% of cases): Two bodies fused from the upper chest to the lower chest. These twins usually share a heart, and may also share the liver or part of the digestive system.
Thoracopagus (18.5%): Two bodies fused from the upper thorax to lower belly. The heart is always involved in these cases. As of 2015, separation of a genuinely shared heart has not offered survival to two twins; a designated twin may survive if allotted the heart, sacrificing the other twin.
Omphalopagus (10%): Two bodies fused at the lower abdomen. Unlike thoracopagus, the heart is never involved in these cases; however, the twins often share a liver, digestive system, diaphragm and other organs.
Parasitic twins (10%): Twins that are asymmetrically conjoined, resulting in one twin that is small, less formed, and dependent on the larger twin for survival.
Craniopagus (6%):Fused skulls, but separate bodies. These twins can be conjoined at the back of the head, the front of the head, or the side of the head, but not on the face or the base of the skull.
Other, less common types of conjoined twins include:

Cephalopagus: Two faces on opposite sides of a single, conjoined head; the upper portion of the body is fused while the bottom portions are separate. These twins generally cannot survive due to severe malformations of the brain. Also known as janiceps (after the two-faced Roman deity Janus) or syncephalus.
Syncephalus: One head with a single face but four ears, and two bodies.
Cephalothoracopagus: Bodies fused in the head and thorax. In this type of twins, there are two faces facing in opposite directions, or sometimes a single face and an enlarged skull.
Xiphopagus: Two bodies fused in the xiphoid cartilage, which is approximately from the navel to the lower breastbone. These twins almost never share any vital organs, with the exception of the liver. A famous example is Chang and Eng Bunker.
Ischiopagus: Fused lower half of the two bodies, with spines conjoined end-to-end at a 180° angle. These twins have four arms; one, two, three or four legs; and typically one external set of genitalia and anus.
Omphalo-Ischiopagus: Fused in a similar fashion as ischiopagus twins, but facing each other with a joined abdomen akin to omphalopagus. These twins have four arms, and two, three, or four legs.
Parapagus: Fused side-by-side with a shared pelvis. Twins that are dithoracic parapagus are fused at the abdomen and pelvis, but not the thorax. Twins that are diprosopic parapagus have one trunk and two faces. Twins that are dicephalic parapagus have one trunk and two heads, and have two (dibrachius), three (tribrachius), or four (tetrabrachius) arms.
Craniopagus parasiticus: Like craniopagus, but with a second bodiless head attached to the dominant head.
Pygopagus (Iliopagus): Two bodies joined at the pelvis.
Rachipagus: Twins joined along the dorsal aspect (back) of their bodies, with fusion of the vertebral arches and the soft tissue from the head to the buttocks.

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