Introduction.

Conjoined twins are two babies that are born physically connected to each
other. A rare phenomenon, the occurrence is estimated to range from 1 in
50,000 births to 1 in 200,000 births, with a somewhat higher incidence in
Southwest Asia and Africa. The condition is more frequently found among
females, with a ratio of 3:1.
Conjoined twins develop when an early embryo partially separates to form
two individuals. Although two fetuses will develop from this embryo, they
will remain physically connected most often at the chest, pelvis or
buttocks. Conjoined twins may also share one or more internal organs.
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 that also
Most conjoined twins are stillborn or die shortly after birth. Some surviving
conjoined twins can be surgically separated. The success of surgery to
separate conjoined twins depends on where the twins are joined and how
many and which organs are shared, as well as on the experience and skill
of the surgical team.
The most famous pair of conjoined twins was Chang and Eng
Bunker (Thai: - , In-Chan) (18111874), Thaibrothers born in Siam,
now Thailand. They travelled with P.T. Barnum's circus for many years and
were billed as the Siamese Twins. Chang and Eng were joined by a band
of flesh, cartilage, and their fused livers at the torso. 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.

CAUSE
Conjoined twins begin as a single fertilized egg. Usually a single fertilized
egg develops into a single baby. Sometimes a single egg divides in half

during the first one to two weeks after its fertilized. This creates a set of
identical twins.
The exact cause of conjoined twinning is not known. There are two
theories. One is that the egg divides late and does not divide completely.
Eight to 12 days after conception, the embryonic layers that will split to form
monozygotic twins begin to develop into specific organs and structures. It's
believed that when the embryo splits later than this usually between 13
and 15 days after conception separation stops before the process is
complete, and the resulting twins are conjoined.
If the inner mass of the cell is split into equal halves, each capable of
forming a normal individual. But complete separation of the inner cell mass
within the chronic vesicle does not occur and non-separated parts of the
otherwise normal twins remain fused throughout development.
The second and more generally accepted theory is fusion, in which a
fertilized egg completely separates, but stem cells (which search for similar
cells) find like-stem cells on the other twin and fuse the twins together

The connection between the twins bodies may be fairly simple. They may
share only a small amount of tissue, and both children may have all the
organs and other structures they need. For example, the twins may be
joined at the belly with a bridge that connects their livers.
Usually the connection is more complex, and sometimes it is very complex.
The children may share:
Vital organs, like one heart
Many structures, like several parts of their digestive, genital and urinary
systems
A large segment of their body, like all of their lower body
Part of the brain and skull
SYMPTOMS
There aren't any specific signs and symptoms that indicate a woman is
carrying conjoined twins. As with other twin pregnancies, the uterus may
grow more rapidly than expected, and mothers of twins may also have
more fatigue, nausea and vomiting early in the pregnancy.

How twins are joined

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):[7] 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.[8]
Thoracopagus (18.5%):[7] Two bodies fused from the upper thorax to
lower belly. The heart is always involved in these cases.[8]
Omphalopagus (10%):[7] Two bodies fused at the lower chest. Unlike
thoracopagus, the heart is never involved in these cases; however, the
twins often share aliver, digestive system, diaphragm and other
organs.[8]
Parasitic twins (10%):[7] Twins that are asymmetrically conjoined,
resulting in one twin that is small, less formed, and dependent on the
larger twin for survival.
Craniopagus (6%):[7] 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.[8]

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
god Janus) or syncephalus.[8]
Syncephalus: One head with a single face but four ears, and two
bodies.[8]
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.[8][9]
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.[8] 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; two, three or
four legs; and typically one external set of genitalia and anus.[8]
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.[8]
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 are dicephalic, and
have two (dibrachius), three (tribrachius), or four (tetrabrachius) arms.[8]
Craniopagus parasiticus: Like craniopagus, but with a second
bodiless head attached to the dominant head.
Pygopagus (Iliopagus): Two bodies joined at the pelvis.[8]
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 [10]

TEST AND DIAGNOSIS (During pregnancy)

In most cases, doctors see that twins are conjoined during a
routine ultrasound late in the first trimester of pregnancy or in the second
trimester. Once your doctor can see by ultrasound where the twins are
joined, the doctor will have some idea of which structures the twins are
likely to share. Other imaging studies can also take pictures of the twins
before birth. They include echocardiography and prenatal MRI (magnetic
resonance imaging). These may give your doctor more details.
After birth, conjoined twins need many other imaging studies and tests to
learn about their health and the connections between them. Your doctor will
want to find out more about their anatomy, or structure, and their function,
or how well their bodies work. It will be important to check all of their
systems.
The exact studies and tests your babies need will depend on where they
are joined and how their health seems. Heart (echocardiography and
electrocardiography) and lung (pulmonary) tests are a few of the common
tests for conjoined twins.

TREATMENT (Delivery)
Treatment of conjoined twins depends on their unique circumstances
their health, where they're joined, and whether they share organs or other
vital structures.
If you're carrying conjoined twins, you will be very closely monitored
throughout your pregnancy. You will be best served by a team of doctors
who work to learn as much as possible about your twins' anatomy,
functional capabilities and prognosis after birth. Having this information can
help your doctors form a treatment plan for your twins.
A surgical delivery (C-section) is planned ahead of time, often two to four
weeks before the due date.
After your conjoined twins are born, you and your doctors must decide
whether separation surgery should be attempted. An emergency separation
may be needed if one of the twins dies, develops a life-threatening
condition or threatens the survival of the other twin. More often, however,
separation surgery is an elective procedure done two to four months after
birth. Recent advances in prenatal imaging, critical care and anesthetic
care have improved outcomes in separation surgery.

Separating Twins: No Easy Matter

The surgical separation of conjoined twins is a delicate and risky
procedure, requiring extreme precision and care. Therefore, the decision to
separate twins is a serious one.
Mortality rates for twins who undergo separation vary, depending on their
type of connection, and the organs they share. For example, twins joined at
the sacrum at the base of the spine have a 68 percent chance of
successful separation, whereas, in cases of twins with conjoined hearts at
the ventricular (pumping chamber) level, there are no known survivors.
Although success rates have improved over the years, surgical separation
is still rare. Since 1950, at least one twin has survived separation about 75
percent of the time.

It is only after twins are born that doctors can use magnetic resonance
imaging, ultrasound and angiography to find out what organs the twins
share. In order to determine the feasibility of separation, doctors must
carefully assess how the twins' shared organs function.
After separation, most twins need intensive rehabilitation because of the
malformation and position of their spines. The muscles in their backs are
constantly being flexed and they often have a difficult time bending their
backs forward and backwards and sitting up straight.

Success rate for surgery of conjoined twins

Improvement in survival rate for surgery of conjoined twins is due to
advances in diagnostic techniques, especially computed tomography and
magnetic resonance imaging, meticulous anaesthetic management with
careful monitoring of fluid replacement intraoperatively, improved surgical
techniques with special emphasis on restricting blood loss, such as the use
of ultrasonic separation of fused livers, achieving body-wall closure, and,
most importantly, the value of previous experience, and postoperative
intensive care with accurate attention to potentially labile cardiovascular
status.
There are four opportunities for intervention in the management of
conjoined twins. Prenatally, the diagnosis of conjoined twins can be made
as early as 12 weeks gestational age, with accurate anatomical detail
achievable at 20 weeks. Elective termination would be considered in the
event of complex cardiac (thoracopagus) or neural (craniopagus) fusion.
Alternatively, elective caesarean section delivery should be planned at 38
weeks' gestation. At birth, separation may be declined because of cardiac
or neural fusion or when the extent of deformity after separation would be
so extensive as to be unacceptable to the parents. Emergency separation
is undertaken when one twin is already dead or dying and threatens the
survival of the other, or if a baby has a correctable associated congenital
abnormality present which, if untreated, would be fatal, such as intestinal
obstruction, midgut volvulus, or ruptured exomphalos. In all other cases,
elective separation is planned at around age 3 months. Full investigations
can be carried out before surgery to define accurately the anatomy of the
union1 and to apply methods such as tissue expansion to achieve primary
closure.2 From 1985 to 2000, 17 sets of conjoined twins were managed by
a single surgical team. Their treatment and outcome is shown in the table.

Since five of the 14 infants involved in emergency separation were already

dead (two) or unsalvageable (three), the true survival rate for this group
should be 44%.
The importance of previous experience in dealing with conjoined twins
cannot be overemphasised. The operative approach is unusual, the
anatomical configuration can be highly complex, and during the operative
procedure anatomical variants might alter the planned course of action.A
wide range of specialties need to be involved in the procedure such as
cardiologists and cardiac surgeons in thoracopagus, urologists in
ischiopagus and pygopagus, and orthopaedic and plastic surgeons.
Nephrological expertise might be required for dialysis in the event of
postoperative renal failure.
Success in the management of conjoined twins requires an experienced
team functioning in the tertiary referral centre with the full range of medical
and surgical specialties.

SOME AMAZING CONJOINED TWIN SEPARATIONS.

Angelica and Angelina Sabuco

Angelica and Angelina Sabuco, twins who were born conjoined at the
chest and abdomen, are now separate little girls. The 2-year-olds
were separated Nov. 1 2011 in a 10-hour surgery at Lucile Packard

Children's Hospital. The operation was the culmination of several

months of complex planning involving specialists from nearly every
part of the hospital. The riskiest portion of the procedure, dividing the
girls' fused livers, went slowly but smoothly. Hemorrhage had been a
concern because one quarter of the body's blood supply passes
through the liver each minute. But thanks to the state-of-the-art
equipment the team used to divide the tissue and cauterize the girls'
blood vessels, virtually no blood loss occurred during that part of
the procedure, said Hartman, clinical professor of pediatric surgery at
the School of Medicine.

The surgery was the second separation of conjoined twins performed

at Packard Children's. The first pair was successfully separated in
2007 by a team that Hartman led. The Sabucos' operation was less
complex because they shared fewer organs

Rital and Ritaj Gaboura

In September 2011, twin girls born joined at the head have been
successfully separated by British doctors. Rital and Ritag Gaboura,
who are 11 months old, survived at odds of one-in-10 million. They
had four complex operations at London's Great Ormond Street
Children's Hospital. The sisters, who were born in Khartoum, Sudan,
were brought to Britain for the procedures by their parents
Abdelmajeed Gaboura, 31, and, Enas, 27, who are both doctors. By
the time they arrived, Ritag's heart was already failing.
Rital and Ritag were born with one of the most serious forms of the
condition as they shared blood vessels, and there was significant
blood flow between their brains. Ritag supplied half her sister's brain
with blood and drained most of it back into her own body to reoxygenate meaning her heart was doing most of the work for both
of them. Any significant drop in blood pressure during surgery could
have caused brain damage. While both girls are as alert as they were
before the operation, it could be years before their parents and
doctors discover if they have suffered any long-term problems.