Fetofetal transfusion syndrome

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Classification according to ICD-10
O43.0 (mother) Transplacental transfusion syndromes
P02.3 (twins) Damage to the fetus from transplacental transfusion syndromes
ICD-10 online (WHO version 2019)
"Rose red and snow white". Newborn twins with TTTS. Both acceptor (left) and donor (right) survived.

The fetofetal transfusion syndrome (abbreviated FFTS , synonymous twin syndrome ; English twin-to-twin transfusion syndrome , abbreviated TTTS ) is rare, but its effects are usually a very serious circulatory and nutritional disorder of identical twins . In these “monochorionic” pregnancies , the two fetuses share a placenta . This syndrome is based on extraordinary vascular connections in the child's blood circulation on the placenta, which cause the unborn children to receive unequal supply. Extreme cases of this developmental disorder are usually successfully treated endoscopically with laser ablation .

causes

A fetofetal transfusion syndrome is only observed in twin pregnancies in which both fetuses share a placenta, the so-called monochorionic twin pregnancies. In 85% to 95% of these cases, blood vessel anastomoses form in the placenta ; therefore one speaks of a placenta with communicating blood vessels. Such connections are dangerous when arterial branches open into veins (= arteriovenous) before the arteries can pass through the chorionic villi . However, connections between arteries (= arterioarterial) or between veins (= venovenous) are also disadvantageous.

A reciprocal blood transfusion takes place through the anastomoses . In the majority of cases, this blood exchange is not dangerous and therefore of little medical relevance. Whether or not it occurs frequently, blood exchange is to be regarded as normal as long as it is balanced between the two fetuses. However, if the transfusion via the placental vessels is unbalanced, a twin will lose more blood to its sibling than it receives. The donor twin must be distinguished from the usually larger recipient twin (acceptor). The fetofetal transfusion syndrome is caused by an imbalance in the two blood circuits.

frequency

In 10 to 15 out of 100 pregnancies with identical twins on a common placenta (monochorionic twin pregnancies), a medically relevant imbalance develops between the two fetal blood circulation systems.

A publication from the Netherlands reports 0.4% of monochorionic twin pregnancies, based on the total population. A large, population-based Canadian study counted only 48 FFTS cases in a total of 142,715 births.

diagnosis

Since an FFTS almost never develops when two placenta are present, it must be ensured that the pregnancy is a monochorionic twin pregnancy, i.e. only one placenta for care , in order to assess the risk of developing an FFTS and for a possible subsequent diagnosis of the children. The best way to check the chorale is between the ninth and twelfth week of pregnancy. After that, the chorale is much more difficult, sometimes even impossible to determine. If a twin pregnancy is considered monochorionic, it must be monitored with ultrasound ( sonography ) at least every three weeks .

The differential diagnosis of placental insufficiency (achievement weakness of the placenta) must be clarified. The twins would also be of different sizes, although the larger child does not show any unusually strong increase in amniotic fluid (polyhydramnios) (unless this is due to other causes).

When caring for the expectant mother , ICD-10 code O43.0 (Pathological conditions of the placenta - transplacental transfusion syndromes) is given for diagnoses .

Symptoms

In TTTS there is usually a permanently unbalanced blood exchange between the twins. Typical symptoms are changes in the ultrasound. Symptoms are usually very unspecific (e.g. in the case of polyhydramnios) and only appear in advanced TTTS. An early examination in specialized centers is therefore essential if TTTS is suspected.

Staging according to Quintero

The degree of severity of TTTS is generally classified using the Quintero staging. Above all, sonographic criteria are included in the classification.

stage Diagnostic criteria
I. There is a marked mismatch in the amount of amniotic fluid between the twins. The largest measurable pool of amniotic fluid in the ultrasound is <2 cm for the donor twin and> 8 cm for the recipient twin.
II In addition to stage I, the urinary bladder of the donor twin cannot be visualized on ultrasound.
III In addition to stage II, there are abnormalities in the Doppler ultrasound of the fetal vessels: Missing or negative end-diastolic blood flow in the umbilical artery, negative A-wave in the ductus venosus.
IV In addition to stage III, fetal hydrops as a sign of decompensation of the heart can be detected on ultrasound.
V Intrauterine death of a twin or both fetuses.

Symptoms in the acceptor (recipient twin)

The recipient twin is usually significantly larger than the donor twin. Due to increased urine excretion ( diuresis ), it forms more amniotic fluid. This so-called polyhydramnios occurs as a result of a hormone called atrial natriuretic peptide (ANP), which is increasingly secreted by cells in the atrium. And the increased ANP level increases the diuresis in the donor. The research results give hope that in the future the fetus will be treated directly with an ANP blocker.

The excessive amniotic fluid overstretched the uterus and, if left untreated, leads to premature labor , premature rupture of the amniotic sac (rupture) and, accordingly, miscarriage or premature birth . Due to the permanently increased blood volume, the recipient twin suffers from heart failure ; as a sign of the decompensation of the heart, a hydrops fetalis , a generalized edema formation, develops . If left untreated, heart failure can lead to prenatal death. After birth, the acceptor has an unusually large amount of blood ( plethora ).

Symptoms in the donor (donor twin)

The donor twin is significantly smaller than the receiving twin due to growth retardation. Due to reduced or even intermittent urine excretion, its fruit cavity contains little amniotic fluid ( oligohydramnios ). In severe cases there is no amniotic fluid ( anhydramnios , “stuck twin”) and accordingly the amniotic sac can not be visualized with ultrasound . By anemia ( anemia ) and general lack supply at too small a proportion of the placenta prenatal death may also occur when the donor. After the birth, the donor twin always shows anemia with a difference in hemoglobin concentration compared to the recipient twin.

Intrauterine fetal death (IUFT)

Both the donor and the acceptor can lead to death in the womb in the case of untreated TTTS due to the above. Changes are coming. If a twin dies, in 26% of such cases the actually viable twin bled to death via the superficial anastomoses of the dead one. The risk of thrombosis is significantly increased in such situations. In 25% IUFT, the survivor has a necrotizing lesion in the white matter of the brain, kidneys, and other organs. In up to 33% of IUFT cases, the surviving twin dies in the first year of life.

therapy

To date, there is no treatment method that guarantees the survival of the twins with certainty and guarantees that the children will not suffer any permanent impairment as a result of the effects of the TTTS or the prenatal and postnatal treatment.

If only minor symptoms are pronounced, one can wait and see. However, the course of the pregnancy must be closely monitored in specialized centers or by specialized examiners in order to initiate therapy in the event of deterioration. In the past, amniocentesis was used as a symptom-related therapy. Many studies now show laser ablation to be more beneficial than amniocentesis. Laser ablation is therefore the therapy of choice for TTTS in need of treatment.

Amniotic fluid discharge puncture

In the amniotic fluid relief puncture is amniotic sac of the recipient twin punctured and deflated amniotic fluid. This relief reduces the risk of rupture of the amniotic sac as well as the occurrence of premature labor. The relief puncture is a so-called symptomatic treatment, which means that it does not eliminate the cause of the disease, but only part of its consequences. The intervention does not change anything in the circulatory situation. Since polyhydramnios usually occur again after a discharge puncture, several punctures must be performed during pregnancy.

Laser ablation

FFTS laser therapy, schematic: arterio-venous anastomoses separated. NS  umbilical cord; U uterus; =  Laser cut

According to current knowledge, the severing of the vascular anastomoses by laser is the method of choice for pronounced TTTS. A minimally invasive reflection of the uterus is carried out for laser ablation : In such a fetoscopy , the vascular connections on the inner placenta surface are sought. First, connections between the arteries of the donor twin and the veins of the recipient twin must be sealed with laser pulses. Then connections between the arteries of the recipient and the veins of the donor must be lasered. The recommended sequence allows a blood transfusion during the operation from the (previous) recipient to the undersupplied donor.

If the wrong connections could be closed, the laser ablation was successful: it created two separate fetal circuits and eliminated the cause of the TTTS. As part of laser ablation, amniotic fluid is also removed from the amniotic sac of the recipient twin. The combined treatment achieves higher survival rates than with amniotic fluid relief alone.

Laser treatment should generally only be carried out in specialized centers by experienced surgeons.

Complications

Possible complications are caused by the special formation of the placenta and the vascular connections on the placenta that are unfavorably located for the operation. If one of the fetuses does not have enough placenta left after the laser treatment to be cared for, it will die in the womb. The other twin usually remains undamaged. If the complication depends on the formation of the placenta, the surgeon cannot influence it. If anastomoses have been overlooked, a new laser treatment must be carried out, which significantly increases the risk of pregnancy. The frequency of this complication depends on the surgeon's experience and varies significantly when comparing the centers (see table below).

Damage to the intestines is known to be: venom obstruction , ileum obstruction , breakthrough at the site of a necrotizing intestinal inflammation. The affected intestinal sections were removed after birth and continuous intestinal passages were made. The infants subsequently did not suffer from serious digestive disorders.

Other complications include premature rupture of the bladder as a result of the fetoscopy and bleeding from the procedure. The supply of this z. Complications, some of which are severe, also depend on the surgeon's experience and the type of fetoscopy. For example, the introduction of a modified fetoscopy trocar with ultra-thin, flexible optics at the University Clinic in Halle reduced the damage to the egg membrane from 11.27 mm² to 2.65 mm², thereby significantly reducing the complication rate.

Table: Comparison of specialized centers with> 100 published cases. Child survival rate after laser ablation, rate of necessary re-laser surgery (ReTTTS) and fetoscope optics used

Author / center Report period at least 1 child survived 2 children survive Re-TTTS Feto-scope
Tchirikov et al. (2011, 2013) Halle / Saale, Germany 2008-2013 97.0% 79.0% 2% 1 mm
Egawa et al. (2013) Tokyo, Japan 2003-2009 93.2% 71.6% k. A. 2 mm
Quintero et al. (2007) Tampa, USA 2003-2005 89.6% 68.9% k. A. 2 mm
Martinez et al. (2003) Barcelona, ​​Spain 1997-2001 88.2% 49.1% 1 % 2 mm
Paek et al. (2009) 2003-2009 87.0% 63.0% k. A. 2 mm
Stirnemann et al. (2012) Paris, France 2004-2010 87.0% 55.0% 8.50% 2 mm
Baud et al. (2013) Toronto, Canada 1999-2012 86.9% 56.6% k. A. 2 mm
Cincotta et al. (2009) Brisbane, Australia 2002-2007 85.0% 66.0% 0% 2 mm
Weingertner et al. (2011) Schiltigheim, France 2004-2010 85.0% 52.0% 7% 3 mm
Morris et al. (2010) Birmingham, UK 2004-2009 85.0% 38.4% 11.50% 2 mm
Huber et al. (2006) Hamburg, Germany 1999-2003 83.5% 59.5% k. A. 2 mm
Hecher et al. (2018) Hamburg, Germany 1995-2018 > 90% 70.0% k. A. 2 mm
Rustico et al. (2012) Milano, Italy 2004-2009 74.0% 40.7% 11.30% 2 mm

The TTTS can be ended by a premature birth. Depending on the week of pregnancy (gestational age), the method of choice can be to induce childbirth before the calculated delivery date so that the twins are no longer exposed to the TTTS. However, there are risks associated with premature birth (increased mortality, permanent damage), which must be weighed against the risks arising from the consequences of the TTTS.

In order to avoid complications in addition to the TTTS, it helps to constantly practice surgical skill. Every laser operation must be carefully planned and simulated. This clinical purpose is served by a computer platform that generates a three-dimensional model of the uterus from findings from magnetic resonance imaging and ultrasound.

forecast

Without treatment, TTTS poses high risks for the twins, namely mortality before or during birth. Surviving TTTS babies can suffer from a variety of illnesses, particularly central nervous system developmental disorders . In a small number of 64 untreated TTTS pregnancies, in 12 cases (19%) both twins survived the birth, in 11 cases (17%) only one twin survived, but there was no survivor in 41 cases (64%).

With amniotic fluid aspiration, survival rates of 60% to 80% have been reported for all FFTS children. But after endoscopic laser treatment, the chance that at least one twin will survive is between 80% and 100%, depending on the treating center (see table above).

An early examination reported 92 pregnancies in which one twin died in the mother. The recipient twin was mostly affected by intrauterine death because anastomoses of arteries in veins play a disadvantageously decisive role.

Long-term consequences

After birth, some twins affected by TTTS have organ-specific disorders. For example, kidney failure and low blood pressure ( hypotension ) requiring treatment are increasing . However, these disturbances usually pass.

Surviving children who were treated with amniotic fluid discharge punctures suffer a high frequency of about 15% permanent brain damage, infantile cerebral palsy . In addition, another 8% are found with general developmental delay. In contrast, infantile cerebral palsy was reported in children after laser vascular transection only about 5%. Acute kidney damage is also a known postpartum TTTS complication.

literature

  • Lennart Van Der Veeken, I Couck, J Van Der Merwe, Luc De Catte, R Devlieger, Jan Deprest, Liesbeth Lewi: Laser for twin-to-twin transfusion syndrome: A guide for endoscopic surgeons. In: Facts Views Vis Obgyn 2019, 11, 3, 2019: 197–205. PDF; Nice Pictures.
  • Hitoshi Yoda: Fetal and neonatal circulatory disorders in twin to twin transfusion syndrome (The secondary publication). In: J Nippon Med Sch 86, 4, 2019: 192-200. PDF; Nice Pictures.

Individual evidence

  1. ^ A b T. Zach, RM Ramus: Twin-to-Twin Transfusion Syndrome. on Medscape.com (Retrieved February 16, 2012)
  2. ^ Julian E De Lia, Randall S Kuhlmann: Twin-to-twin transfusion syndrome: 30 years at the front. In: Am J Perinatol 31, 2014: S7-S12. Abstract.
  3. ↑ Christian Bamberg, Kurt Hecher: Update on twin-to-twin transfusion syndrome. In: Best Pract Res Clin Obstet Gynaecol 58, 2019: 55-65. Abstract.
  4. ↑ SF de Villiers, DP Zhao, D Cohen, EW van Zwet, T Duan, D Oepkes, E Lopriore:  Correlation between veno-venous anastomoses, TTTS and perinatal mortality in monochorionic twin pregnancies. In: Placenta 36, 5, 2015: 603–606.
  5. ↑ Enrico Lopriore, Liesbeth Lewi, Asma Khalil: Monochorionic twins: A delicate balance. In: J Clin Med 8, 10, 2019: 1711. PDF.
  6. ↑ S. Lutfi et al .: Twin-Twin Transfusion Syndrome: A Population-Based Study. In: Obstetrics & Gynecology 104, 2004: 1289-1297. PMID 15572492 .
  7. ^ Rubén A Quintero, Walter J Morales, Mary H Allen, Patricia W Bornick, Patricia K Johnson, Michael Kruger: Staging of twin-twin transfusion syndrome. In: J Perinatol 19, 1999: 550-555. PDF.
  8. ↑ Rekha Bajoria, Stuart Ward, SR Sooranna: Atrial natriuretic peptide mediated polyuria: pathogenesis of polyhydramnios in the recipient twin of twin-twin transfusion syndrome. In: Placenta 22, 8-9, 2001: 716-724. doi: 10.1053 / plac.2001.0715.
  9. ↑ R Bajoria et al .: Outcome of twin pregnancies complicated by single intrauterine death in relation to vascular anatomy of the monochorionic placenta. In: Hum Reprod 14, 1999: 2124-2130.
  10. ↑ KR Duncan, ML Denbow, NM Fisk: The aetiology and management of twin-twin transfusion syndrome. In: Prenatal Diagnosis 17, 1997: 1227-1236.
  11. ↑ a b D. Roberts: Interventions for twin – twin transfusion syndrome: a Cochrane review. In: Ultrasound Obstet Gynecol 31, 2008: 701-711.
  12. ↑ Ramen H Chmait, Eftichia V Kontopoulos, Rubén A Quintero: Sequential laser surgery for twin – twin transfusion syndrome. In: Am J Perinatol 31, Suppl 1, 2014: S13-S18. Abstract.
  13. ↑ UMM: Twin Transfusion Syndrome: University Hospital Mannheim. Retrieved August 8, 2018 .
  14. ↑ Alba Sánchez-Galán, JL Encinas, E Antolín, et al: Complicaciones intestinales en el síndrome de trasfusión feto fetal (STFF) [Intestinal complications in twin-to-twin transfusion syndrome (TTTS) treated by laser coagulation (LC)]. In: Cir Pediatr 30, 2017: 33-38.
  15. ↑ Michael Tchirikov, Viktor Oshovskyy, Joscha Steetskamp, ​​Andreas Falkert, Georgine Huber, Michael Entezami: Neonatal outcome using ultrathin fetoscope for laser coagulation in twin-to-twin-transfusion syndrome. In: J Perinat Med 39, 2011: 725-730. PDF.
  16. ↑ Michael Tchirikov, Carola Arnold, Viktor Oshovskyy, Ulf-Rüdiger Heinrich, Volker Thäle: Three years' experience of using a 29-gauge atraumatic needle for amniocentesis. In: J Perinat Med 40, 4, 2012: 413-417. PDF.
  17. ↑ Florian Steger, Simone Ehm, Michael Tchirikov (Hg): Prenatal diagnostics and therapy in ethics, medicine and law. Springer Verlag, 2014. Table of contents.
  18. ↑ Kurt Hecher, Helena M Gardiner, Anke Diemert, Peter Bartmann: Long-term outcomes for monochorionic twins after laser therapy in twin-to-twin transfusion syndrome. In: Lancet Child Adolesc Health 2, 7, 2018: 525-535. Summary.
  19. ↑ Jordina Torrents-Barrena, Rocío López-Velazco, Narcís Masoller, Brenda Valenzuela-Alcaraz, Eduard Gratacós, Elisenda Eixarch, Mario Ceresa, Miguel Ángel González Ballester: TTTS-GPS: Patient-specific preoperative planning and simulation platform for twin-to- twin transfusion syndrome fetal surgery. In: Computer Methods Programs Biomed 179, 2019: 104993. Abstract.
  20. ↑ a b R B Cincotta, PH Gray, G Phythian, YM Rogers, FY Chan: Long term outcome of twin-twin transfusion syndrome. In: Arch Dis Child Fetal Neonatal Ed 83, 2000: F171-F176. PDF.
  21. ↑ Elizabeth B Sasso, Macy T Hardley, Isabela Bottura, Lisa M Korst, Joseph G Ouzounian, Ramen H Chmait: Characteristics of referred patients with twin-twin transfusion syndrome who did not undergo fetal therapy. In: Prenat Diagn 39, 4, 2019: 280–286.
  22. ↑ Rekha Bajoria, Ling Y Wee, Shaheen Anwar, Stuart Ward: Outcome of twin pregnancies complicated by single intrauterine death in relation to vascular anatomy of the monochorionic placenta. In: Hum Reprod 14, 8, 1999: 2124-2130. doi: 10.1093 / humrep / 14.8.2124.
  23. ↑ Lisanne SA Tollenaar, Femke Slaghekke, Johanna M Middeldorp, Frans J Klumper, Monique C Haak, Dick Oepkes, Enrico Lopriore: Twin anemia polycythemia sequence: Current views on pathogenesis, diagnostic criteria, perinatal management, and outcome. In: Twin Res Hum Genet 19, 3, 2016: 222-233. Abstract; further FFTS topics.
  24. ^ Nabil Ziad Melhem, Sarah Ledermann, Lesley Rees: Chronic kidney disease following twin-to-twin transfusion syndrome: long-term outcomes. In: Pediatr Nephrol 34, 5, 2019: 883-888. Abstract.

See also

Web links
This version was added to the list of articles worth reading on June 23, 2006 .