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Twin reversed arterial perfusion sequence—also called TRAP sequence, TRAPS, or acardiac twinning—is a rare complication of monochorionic twin pregnancies. It is a severe variant of twin-to-twin transfusion syndrome (TTTS). The twins' blood systems are connected instead of independent. One twin, called the "acardiac twin" or "TRAP fetus", is severely malformed. The heart is missing or deformed, hence the name acardiac, as are the upper structures of the body . The legs may be partially present or missing, and internal structures of the torso are often poorly formed. The other twin is usually normal in appearance. The normal twin, called the "pump twin", drives blood through both fetuses. It is called "reversed arterial perfusion" because in the acardiac twin the blood flows in a reversed direction.
TRAP sequence occurs in 1% of monochorionic twin pregnancies and in 1 in 35,000 pregnancies overall.
The acardiac twin is a parasitic twin that fails to properly develop a heart, and therefore generally does not develop the upper structures of the body. The parasitic twin, little more than a torso with or without legs, receives its blood supply from the host twin by means of an umbilical cord-like structure (which often only has 2 blood vessels, instead of 3), much like a fetus in fetu, except the acardiac twin is outside the host twin's body. Although the reason is not fully understood, it is apparent that deoxygenated blood from the pump twin is perfused to the acardiac twin. The acardiac twin grows along with the pump twin, but due to inadequate oxygenation it is unable to develop the structures necessary for life, and presents with dramatic deformities.
Although no two acardiac twins are alike, twins with this disorder are grouped into 4 classes: Acephalus, anceps, acormus, and amorphus.
- Acephalus – The most common type, lacking a head, though it may have arms. Thoracic organs are generally absent, and disorganized & unidentifiable tissues take their place.
- Anceps – The acardius has most body parts, including a head with face and incomplete brain. Organs, though present, are crudely formed.
- Acormus – This type has no apparent body and the umbilical cord is seemingly attached to the neck, but x-rays or dissection reveal thoracic structures in the apparent head. One had a leg apparently attached to the head. This may be due to embryopathy degenerating a once normal embryo.
- Amorphus – This extreme form not only lacks a head and limbs, but also any internal organs, and consists of tissues with blood vessels branching from the umbilical cord. Some may only be stem cell tumors.
The acardiac twin may also be described as a "hemiacardius", which has an incompletely formed heart, or a "holoacardius", in which the heart is not present at all.
Twin-to-twin transfusion syndrome (TTTS), also known as feto-fetal transfusion syndrome (FFTS) and twin oligohydramnios-polyhydramnios sequence (TOPS) is a complication of disproportionate blood supply, resulting in high morbidity and mortality. It can affect monochorionic multiples, that is, multiple pregnancies where two or more fetuses share a chorion and hence a single placenta. Severe TTTS has a 60–100% mortality rate.
A vanishing twin, also known as fetal resorption, is a fetus in a multi-gestation pregnancy which dies in utero and is then partially or completely reabsorbed. In some instances, the dead twin will be compressed into a flattened, parchment-like state known as "fetus papyraceus".
Vanishing twins occur in up to one out of every eight multifetus pregnancies and may not even be known in most cases. "High resorption rates, which cannot be explained on the basis of the expected abortion rate...suggest intense fetal competition for space, nutrition, or other factors during early gestation, with frequent loss or resorption of the other twin(s)."
In pregnancies achieved by IVF, "it frequently happens that more than one amniotic sac can be seen in early pregnancy, whereas a few weeks later there is only one to be seen and the other has 'vanished'."
A breech birth occurs when a baby is born bottom first instead of head first. Around 3-5% of pregnant women at term (37–40 weeks pregnant) will have a breech baby.
Most babies in the breech position are born by a caesarean section because it is seen as safer than being born vaginally.
As most breech babies are delivered by caesarean section in developed countries, doctors and midwives may lose the skills required to safely assist women giving birth to a breech baby vaginally. Delivering all breech babies by caesarean section in developing countries may be very difficult to implement or even impossible as there are not always resources available to provide this service.
A staging system proposed by fetal surgeon Dr. Ruben Quintero is commonly used to classify the severity of TTTS.
Stage I: A small amount of amniotic fluid (oligohydramnios) is found around the donor twin and a large amount of amniotic fluid (polyhydramnios) is found around the recipient twin.
Stage II: In addition to the description above, the ultrasound is not able to identify the bladder in the donor twin.
Stage III: In addition to the characteristics of Stages I and II, there is abnormal blood flow in the umbilical cords of the twins.
Stage IV: In addition to all of the above findings, the recipient twin has swelling under the skin and appears to be experiencing heart failure (fetal hydrops).
Stage V: In addition to all of the above findings, one of the twins has died. This can happen to either twin. The risk to either the donor or the recipient is roughly equal & is quite high in Stage II or higher TTTS.
The Quintero staging does not provide information about prognosis, and other staging systems have been proposed.
Types of breech depend on how the baby’s legs are lying.
- A frank breech (otherwise known as an extended breech) is where the baby’s legs are up next to its abdomen, with its knees straight and its feet next to its ears. This is the most common type of breech.
- A complete breech (flexed) breech is when the baby appears as though it is sitting crossed-legged with its legs bent at the hips and knees.
- A footling breech is when one or both of the baby’s feet are born first instead of the pelvis. This is more common in babies born prematurely or before their due date.
In addition to the above, breech births in which the sacrum is the fetal denominator can be classified by the position of a fetus. Thus sacro-anterior, sacro-transverse and sacro-posterior positions all exist, but left sacro-anterior is the most common presentation. Sacro-anterior indicates an easier delivery compared to other forms.
Craniopagus parasiticus is an extremely rare type of parasitic twinning occurring in about 4 to 6 of 10,000,000 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 have been documented in the literature. Most infants with this condition are stillborn, or die shortly after birth.
Locations can include:
- subcutaneous tissue/scalp
- pleura (pleural effusion)
- pericardium (pericardial effusion)
- abdomen (ascites)
The edema is usually seen in the fetal subcutaneous tissue, sometimes leading to spontaneous abortion. It is a prenatal form of heart failure, in which the heart is unable to satisfy its demand for a high amount of blood flow.
Locked twins is a rare complication of multiple pregnancy where two fetuses become interlocked during presentation before birth. It occurs in roughly 1 in 1,000 twin deliveries and 1 in 90,000 deliveries overall. Most often, locked twins are delivered via Caesarean section, given that the condition has been diagnosed early enough. The fetal mortality rate is high for the twin that presents first, with over 50% being stillborn.
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 that it can live on its own.
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 embryo begins developing in utero, but the pair does not fully separate, and one embryo maintains dominant development at the expense of its twin. Unlike conjoined twins, one ceases development during gestation and is vestigial to a mostly fully formed, otherwise healthy individual twin. The undeveloped twin is defined as parasitic, rather than conjoined, because it is incompletely formed or wholly dependent on the body functions of the complete fetus.
The independent twin is called the autosite.
Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively.
There are two types of locked twins: breech/vertex and vertex/vertex. In breech/vertex presentations, which are much more common, the first twin is in the breech position, presenting feet-first, and the second is in the cephalic (vertex) position, presenting in the normal head-first manner. In these cases, the chin of the first twin locks behind the chin of the second twin while in the uterus or birth canal, preventing vaginal delivery. In vertex/vertex presentations, where both twins are positioned for head-first delivery, the two heads become locked at the pelvic brim, preventing either fetus from passing through the pelvic inlet in a vaginal delivery.
Contributing factors to the interlocking of twins include uterine hypertonicity, small fetal size, and reduced levels of amniotic fluid following rupture of the membranes. It is more likely to occur in women with large pelvises, young primigravidae (young women in their first pregnancy), and pregnancies with monoamniotic twins.
Umbilical cord prolapse occurs when the umbilical cord comes out of the uterus with or before the presenting part of the fetus. It is a relatively rare condition and occurs in fewer than 1% of pregnancies. Cord prolapse is more common in women who have had rupture of their amniotic sac. Other risk factors include maternal or fetal factors that prevent the fetus from occupying a normal position in the maternal pelvis, such as abnormal fetal lie, too much amniotic fluid, or a premature or small fetus. The concern with cord prolapse is that pressure on the cord from the fetus will cause cord compression that compromises blood flow to the fetus. Whenever there is a sudden decrease in fetal heart rate or abnormal fetal heart tracing, umbilical cord prolapse should be considered. Due to the possibility for fetal death and other complications, umbilical cord prolapse is considered an obstetric emergency during pregnancy or labor. Current management guidelines focus on quick delivery, which usually entails a cesarean section. With appropriate management, the majority of cases have good neonatal outcomes.
The first sign of umbilical cord prolapse is usually a sudden decrease in fetal heart rate that is severe and does not immediately resolve. On a fetal heart tracing, this would usually look like moderate to severe variable decelerations. Occasionally, the cord can be seen or felt on vaginal examination, particularly with overt cord prolapse.
Fetus in fetu (or foetus in foetu) is a developmental abnormality in which a mass of tissue resembling a fetus forms inside the body. An early example of the phenomenon was described in 1808 by George William Young.
There are two theories of origin concerning "fetus in fetu". One theory is that the mass begins as a normal fetus but becomes enveloped inside its twin. The other theory is that the mass is a highly developed teratoma. "Fetus in fetu" is estimated to occur in 1 in 500,000 live births.
Twin anemia-polycythemia sequence, abbreviated as TAPS, is a form of chronic inter-twin transfusion.
There are two main theories about the development of fetus in fetu.
Usually the cerebellum and brain stem are formed normally, although in some cases the cerebellum may also be absent. An infant with hydranencephaly may appear normal at birth or may have some distortion of the skull and upper facial features due to fluid pressure inside the skull. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal, depending on the severity of the condition. However, after a few weeks the infant sometimes becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop, if they did not exist at birth. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits.
Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), limited thermoregulation abilities, and respiratory problems.
Still other infants display no obvious symptoms at birth, going many months without a confirmed diagnosis of hydranencephaly. In some cases a severe hydrocephalus, or other cephalic condition, is misdiagnosed.
Germinal matrix hemorrhage is a bleeding into the subependymal germinal matrix with or without subsequent rupture into the lateral ventricle. Such intraventricular hemorrhage can occur due to perinatal asphyxia in preterm neonates.
The condition is typically seen in premature infants, and the timing of its onset is generally inversely proportional to the gestational age of the baby at birth (i.e. the earlier a baby is born, the later signs of NEC are typically seen). Initial symptoms include feeding intolerance, increased gastric residuals, abdominal distension and bloody stools. Symptoms may progress rapidly to abdominal discoloration with intestinal perforation and peritonitis and systemic hypotension requiring intensive medical support.
Hydranencephaly or hydrancephaly is a condition in which the brain's cerebral hemispheres are absent to varying degrees and the remaining cranial cavity is filled with cerebrospinal fluid.
Hydranencephaly (or hydrancephaly) is a type of cephalic disorder.
These disorders are congenital conditions that derive from either damage to, or abnormal development of, the fetal nervous system in the earliest stages of development in utero. Cephalic is the medical term for “head” or “head end of body.” These conditions do not have any definitive identifiable cause factor; instead generally attributed to a variety of hereditary or genetic conditions, but also by environmental factors such as maternal infection, pharmaceutical intake, or even exposure to high levels of radiation.
This should not be confused with hydrocephalus, which is an accumulation of excess cerebrospinal fluid in the ventricles of the brain.
In hemihydranencephaly, only half of the cranial cavity is filled with fluid.
Signs and symptoms of Eisenmenger syndrome include the following:
- Cyanosis (a blue tinge to the skin resulting from lack of oxygen)
- High red blood cell count
- Swollen or clubbed finger tips (clubbing)
- Fainting (also known as syncope)
- Heart failure
- Abnormal heart rhythms
- Bleeding disorders
- Coughing up blood
- Iron deficiency
- Infections (endocarditis and pneumonia)
- Kidney problems
- Stroke
- Gout (rarely) due to increased uric acid resorption and production with impaired excretion
- Gallstones
This may lead to various neurological sequelae including presentation with cerebral palsy, mental retardation and seizures.