Twin anemia-polycythemia sequence | |
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Other names | TAPS |
Photo of twins legs with twin anemia-polycythemia sequence color difference | |
Specialty | Obstetrics |
Twin anemia-polycythemia sequence (TAPS) is a chronic type of unbalanced fetal transfusion in monochorionic twins that results in polycythemia in the TAPS recipient and anemia in the TAPS donor due to tiny placental anastomoses. [1] Post-laser TAPS and spontaneous TAPS are the two forms of TAPS. Unlike twin-to-twin transfusion syndrome, which arises when twin oligohydramnios polyhydramnios sequence (TOPS) is present, TAPS develops in its absence. [2]
A pale, anemic donor and a plethoric, polycythemic recipient make up the traditional clinical picture of TAPS. [3]
In 29% of spontaneous TAPS twins and 23% of post-laser TAPS twins, severe fetal growth restriction is present. [4] [5]
The cause of TAPS is slow and persistent unbalanced feto-fetal transfusion through tiny placental anastomoses, which progressively results in highly discordant hemoglobin levels. This causes the recipient twin to become polycythemic and the donor twin to become anemic. [1]
There are very few, tiny arteriovenous vascular anastomoses present in TAPS placentas. This distinct angiography is the foundation of the pathogenesis of TAPS. A slow transfusion of blood from the donor to the recipient is made possible by the few tiny anastomoses, which eventually cause very disparate hemoglobin levels. It's unclear if hormonal dysfunction may also contribute to the onset of TAPS. [6]
Doppler ultrasound abnormalities demonstrating an increased peak systolic velocity in the middle cerebral arteries (MCAPSV) in the donor twin and a decreased MCA-PSV in the recipient twin can be used to make an antenatal diagnosis of TAPS. The presence of polycythemia in the recipient and (chronic) anemia in the donor, along with characteristic placental angioarchitecture as determined by injection with colored dye, are the basis for the postnatal diagnosis of TAPS. [6]
Antenatal stage | Results of a Doppler ultrasound scan |
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Stage 1 | MCA-PSV donor >1.5 MoM and MCA-PSV recipient <1.0 MoM, without other signs of fetal compromise |
Stage 2 | MCA-PSV donor >1.7 MoM and MCA-PSV recipient <0.8 MoM, without other signs of fetal compromise |
Stage 3 | As stage 1 or 2, with cardiac compromise of donor, defined as critically abnormal flow. [note 1] |
Stage 4 | Hydrops of donor. |
Stage 5 | Intrauterine demise of one or both fetuses preceded by TAPS |
Postnatal stage | Intertwin hemoglobin difference, g/dl |
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Stage 1 | >8.0 |
Stage 2 | >11.0 |
Stage 3 | >14.0 |
Stage 4 | >17.0 |
Stage 5 | >20.0 |
The rate of residual anastomoses can be decreased to prevent postlaser TAPS. The "Solomon technique," an alternate method of laser surgery, may help lower the possibility of omitting a tiny anastomosis during the procedure. [6]
Weekly ultrasound monitoring, which includes a full staging Doppler examination with the MCA-PSV, is part of the expectant management protocol. The recent onset of ultrasound abnormalities may require an increase in the surveillance frequency. When TAPS patients present in the first or early second trimester, this approach is preferred because many of these patients may resolve or remain clinically stable. Expectant care can be continued with the aim of achieving a late preterm delivery in stable cases that do not advance past stage 2. It is necessary to take into account alternate management options if TAPS is accelerating. [7]
Treatments that are temporary include intrauterine blood transfusion of the anemic donor twin or exchange transfusions, which remove blood from the recipient twin and then transfusion of the donor. [8]
Treating TAPS with fetal laser coagulation of vascular anastomoses is the only potentially effective modality. [9]
In the TAPS Registry cohort, 11% of post-laser TAPS twins and 5% of spontaneous TAPS twins experienced spontaneous fetal death. [5] [4]
Postlaser TAPS can occur in 2–13% of cases, depending on the definitions and criteria applied. [10] [11] The range of incidence for spontaneous TAPS is 3–5%. [6]
A vanishing twin, also known as twin resorption, is a fetus in a multigestation pregnancy that dies in utero and is then partially or completely reabsorbed. In some instances, the dead twin is compressed into a flattened, parchment-like state known as fetus papyraceus.
Twin-to-twin transfusion syndrome (TTTS), also known as feto-fetal transfusion syndrome (FFTS), twin oligohydramnios-polyhydramnios sequence (TOPS) and stuck twin syndrome, is a complication of monochorionic multiple pregnancies in which there is disproportionate blood supply between the fetuses. This leads to unequal levels of amniotic fluid between each fetus and usually leads to death of the undersupplied twin and, without treatment, usually death or a range of birth defects or disabilities for a surviving twin, such as underdeveloped, damaged or missing limbs, digits or organs, especially cerebral palsy.
Hemolytic disease of the newborn, also known as hemolytic disease of the fetus and newborn, HDN, HDFN, or erythroblastosis fetalis, is an alloimmune condition that develops in a fetus at or around birth, when the IgG molecules produced by the mother pass through the placenta. Among these antibodies are some which attack antigens on the red blood cells in the fetal circulation, breaking down and destroying the cells. The fetus can develop reticulocytosis and anemia. The intensity of this fetal disease ranges from mild to very severe, and fetal death from heart failure can occur. When the disease is moderate or severe, many erythroblasts are present in the fetal blood, earning these forms of the disease the name erythroblastosis fetalis.
Hydrops fetalis or hydrops foetalis 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.
A nuchal cord is when the umbilical cord becomes wrapped around the fetus's neck. Symptoms present in the baby shortly after birth from a prior nuchal cord may include duskiness of face, facial petechia, and bleeding in the whites of the eye. Complications can include meconium, respiratory distress, anemia, and stillbirth. Multiple wraps are associated with greater risk.
Placenta accreta occurs when all or part of the placenta attaches abnormally to the myometrium. Three grades of abnormal placental attachment are defined according to the depth of attachment and invasion into the muscular layers of the uterus:
In ABO hemolytic disease of the newborn maternal IgG antibodies with specificity for the ABO blood group system pass through the placenta to the fetal circulation where they can cause hemolysis of fetal red blood cells which can lead to fetal anemia and HDN. In contrast to Rh disease, about half of the cases of ABO HDN occur in a firstborn baby and ABO HDN does not become more severe after further pregnancies.
Hemolytic disease of the newborn (anti-Kell1) is the second most common cause of severe hemolytic disease of the newborn (HDN) after Rh disease. Anti-Kell1 is becoming relatively more important as prevention of Rh disease is also becoming more effective.
Hemolytic disease of the newborn (anti-Rhc) can range from a mild to a severe disease. It is the third most common cause of severe HDN. Rh disease is the most common and hemolytic disease of the newborn (anti-Kell) is the second most common cause of severe HDN. It occurs more commonly in women who are Rh D negative.
Placental insufficiency or utero-placental insufficiency is the failure of the placenta to deliver sufficient nutrients to the fetus during pregnancy, and is often a result of insufficient blood flow to the placenta. The term is also sometimes used to designate late decelerations of fetal heart rate as measured by cardiotocography or an NST, even if there is no other evidence of reduced blood flow to the placenta, normal uterine blood flow rate being 600mL/min.
Kyprianos "Kypros" Nicolaides is a Greek Cypriot physician of British citizenship, Professor of Fetal Medicine at King's College Hospital, London. He is one of the pioneers of fetal medicine and his discoveries have revolutionised the field. He was elected to the US National Academy of Medicine in 2020 for 'improving the care of pregnant women worldwide with pioneering rigorous and creative approaches, and making seminal contributions to prenatal diagnosis and every major obstetrical disorder'. This is considered to be one of the highest honours in the fields of health and medicine and recognises individuals who have demonstrated outstanding professional achievement and commitment to service.
Neonatal alloimmune thrombocytopenia is a disease that affects babies in which the platelet count is decreased because the mother's immune system attacks her fetus' or newborn's platelets. A low platelet count increases the risk of bleeding in the fetus and newborn. If the bleeding occurs in the brain, there may be long-term effects.
Mirror syndrome, triple edema or Ballantyne syndrome is a rare disorder affecting pregnant women. It describes the unusual association of fetal and placental hydrops with maternal preeclampsia.
Hemolytic disease of the newborn (anti-RhE) is caused by the anti-RhE antibody of the Rh blood group system. The anti-RhE antibody can be naturally occurring, or arise following immune sensitization after a blood transfusion or pregnancy.
Percutaneous umbilical cord blood sampling (PUBS), also called cordocentesis, fetal blood sampling, or umbilical vein sampling is a diagnostic genetic test that examines blood from the fetal umbilical cord to detect fetal abnormalities. Fetal and maternal blood supply are typically connected in utero with one vein and two arteries to the fetus. The umbilical vein is responsible for delivering oxygen rich blood to the fetus from the mother; the umbilical arteries are responsible for removing oxygen poor blood from the fetus. This allows for the fetus’ tissues to properly perfuse. PUBS provides a means of rapid chromosome analysis and is useful when information cannot be obtained through amniocentesis, chorionic villus sampling, or ultrasound ; this test carries a significant risk of complication and is typically reserved for pregnancies determined to be at high risk for genetic defect. It has been used with mothers with immune thrombocytopenic purpura.
Velamentous cord insertion is a complication of pregnancy where the umbilical cord is inserted in the fetal membranes. It is a major cause of antepartum hemorrhage that leads to loss of fetal blood and associated with high perinatal mortality. In normal pregnancies, the umbilical cord inserts into the middle of the placental mass and is completely encased by the amniotic sac. The vessels are hence normally protected by Wharton's jelly, which prevents rupture during pregnancy and labor. In velamentous cord insertion, the vessels of the umbilical cord are improperly inserted in the chorioamniotic membrane, and hence the vessels traverse between the amnion and the chorion towards the placenta. Without Wharton's jelly protecting the vessels, the exposed vessels are susceptible to compression and rupture.
Monoamniotic twins are identical or semi-identical twins that share the same amniotic sac within their mother's uterus. Monoamniotic twins are always monochorionic and are usually termed Monoamniotic-Monochorionic twins. They share the placenta, but have two separate umbilical cords. Monoamniotic twins develop when an embryo does not split until after formation of the amniotic sac, at about 9–13 days after fertilization. Monoamniotic triplets or other monoamniotic multiples are possible, but extremely rare. Other obscure possibilities include multiples sets where monoamniotic twins are part of a larger gestation such as triplets, quadruplets, or more.
Monochorionic twins are monozygotic (identical) twins that share the same placenta. If the placenta is shared by more than two twins, these are monochorionic multiples. Monochorionic twins occur in 0.3% of all pregnancies. Seventy-five percent of monozygotic twin pregnancies are monochorionic; the remaining 25% are dichorionic diamniotic. If the placenta divides, this takes place before the third day after fertilization.
An Intrauterine transfusion (IUT) is a procedure that provides blood to a fetus, most commonly through the umbilical cord. It is used in cases of severe fetal anemia, such as when fetal red blood cells are being destroyed by maternal antibodies. IUTs are performed by perinatologists at hospitals or specialized centers.
Beryl Rice Benacerraf was an American radiologist and professor of obstetrics, gynecology and reproductive biology and radiology at Harvard Medical School. She was a pioneer in the use of prenatal ultrasound to diagnose fetal abnormalities, including Down syndrome. In 2021, she was recognized as a "Giant in Obstetrics and Gynecology" by the American Journal of Obstetrics & Gynecology.