Feto-fetal transfusion syndrome

Feto-fetal transfusion syndrome
Feto-fetal transfusion syndrome
ICD-10	O 43.0 , P 02.3 , P 50.3
ICD-10-KM	, and
ICD-9	762.3 , 772.0
Diseasesdb	32064
Medlineplus
eMedicine	med / 3410
Mesh	D005330

Feto-fetal transfusion syndrome (SFTT), also known as feto-fetal transfusion syndrome ( English twin-to-twin transfusion syndrome, TTTS ; German. Fetofetale Transfusionssyndrom, FFTS ) is a serious complication of multiple fetal monochorial pregnancy , in which the blood flow of different fruits is significantly different. This syndrome is associated with a high risk of disability and mortality. In severe cases, fetal mortality ranges from 60 - 100%. ^[one]

Fetoscopy and laser ablation of anastomoses in SFTT

Content

History

SFTT was first described in 1882 by the German obstetrician Friedrich Schatz . The criteria used to make the diagnosis earlier - the difference in weight and hemoglobinemia of the fetus, are not the main ones today. Thus, it was proved that size imbalance is already a rather late sign, and the results of a series of cordocentesis confirmed the comparability of the hemoglobin level in the fetus even in severe cases of SFTT. ^[2]

Epidemiology

SFTT occurs in approximately 5.5 - 17.5% of all monochorial pregnancies. ^{[3] The} true occurrence of the syndrome is difficult to assess in connection with the so-called “hidden mortality” - spontaneous abortion often occurs before the time when it is possible to confirm the diagnosis of SFTT. ^[2]

Etiology and pathogenesis

Thanks to a common single placenta, the blood flow of the fetus can be combined. Despite the fact that everyone uses their part of the placenta, connective vessels ( anastomoses ) can form in it, blood through which can flow from one fetus to another, causing an imbalance in its volume. Depending on the direction of blood flow, one of the fruits becomes a " donor ", the second - " recipient ".

The outflow of blood from the donor reduces its total circulating volume, leads to tissue hypoxia, intrauterine growth retardation , decreased urine production, due to kidney damage and, as a result, oligohydramnios , which impedes normal lung tissue maturation. The blood volume of the “recipient” increases sharply, which increases the load on his heart and kidneys, leading to heart failure and polyhydramnios .

If SFTT occurs before 26 weeks, the fetuses usually die or are born with serious diseases. If the syndrome occurs after 26 weeks, most fruits survive and are more likely to avoid disability.

Treatment Options

Over the years, a variety of studies have been conducted on the effectiveness of certain treatments. None of the proposed therapeutic methods (bed rest, protein shakes, aspirin, etc.) gave statistically significant results. Therefore, this article deals exclusively with surgical options.

Lack of treatment

Expectant tactics, which are regarded as “zero interference,” lead to almost 100% mortality of one or all of the fetuses. An exception is the small group that is in the first stage of SFTT after 22 weeks of gestation (their mortality is slightly lower).

Multiple Amniodrainage

The procedure is the periodic removal of amniotic fluid. It is based on the assumption that acute polyhydramnios can cause premature birth, tissue damage, or antenatal fetal death. However, in the early stages, the procedure itself is associated with a risk of abortion. There are no clear criteria when and how much amniotic fluid should be removed, because its production system is very dynamic. The survival rate of at least one fetus is 66%, and the survivor will have a risk of cerebral palsy of about 15%, and the average delivery time will be 29 weeks of gestation.

Septostomy

Rupture of a twin separating membrane to combine amniotic sacs in order to equalize the volume of water and intrauterine pressure. There is no evidence of the effectiveness of this technique, moreover, it reduces the ability to monitor the progression of the syndrome and is associated with the risk of damage to the umbilical cord, and hence iatrogenic death of the fetus.

Umbilical cord occlusion

A procedure that stops the blood flow in one of the umbilical cords in order to change the pressure in the general system of the fetus and reduce transfusion. It is usually carried out in the early stages of pregnancy on one seriously suffering fetus in the interests of another. Using the method, about 85% of the remaining fetuses survive with a cerebral palsy risk of 5% and an average delivery term of 33–39 weeks.

Laser coagulation of blood vessels

The procedure is based on endoscopic (fetoscopic) technique. A thin conductor with optics (fetoscope) is inserted into the uterine cavity through the anterior abdominal wall under the control of ultrasound, through which a laser nozzle can be drawn. With the help of such a nozzle, coagulation (burning out) of anastomoses that connects the bloodstream of the fetus occurs. Own sites of the placenta and umbilical cord remain intact and functional. The success of the procedure directly depends on the experience of the surgeon and the technical support of the clinic.

PivotTable

A summary table of published data on the results of laser coagulation of anastamoses during TTTS. Only centers that performed more than 100 operations are taken into account.

Author	Period	Pregnancy, weeks	Delivery term, weeks	1 child survived,%	2 children survived,%	PPPROM ≤ 32	Re-TTTS,%	Fetoscope, mm
Tchirikov et al. (2011, 2018) Halle, Germany	2008-2018	20	35	98 ^[4]	79		2	one
Egawa et al. (2013) Tokyo, Japan	2003-2009	21.7	32.6 +/- 3.2	93	72	16,20		2
Martinez et al. (2003) Barcelona, Spain	1997-2001	20.7	33	88	49		one	2
Stirnemann et al. (2012) Paris, France	2004-2010	21		87	55		8.5	2
Baud et al. (2013) Toronto, Canada	1999-2012	21	31 +/- 5	87	57	63.70		2
Cincotta et al. (2009) Brisbane, Austria	2002-2007	21	31	85	66		0	2
Weingertner et al. (2011) Schiltigheim, France	2004-2010	20.6	32.6	85	52	17	7	2
Morris et al. (2010) Birmingheim, UK	2004-2009	20,4	33,2	85	3	17	11.50	2
Rossi et al. (2008) Milano, Italy	2000-2004	20.1	32,7	eight		38		2
Hecher et al. (2000) Hamburg, Germany	1995-1999	20.7	34.3	81	50			2
Rustico et al. (2012) Milano, Italy	2004-2009	20.5	30.5	74	41	28.70	11.30	2

As can be seen from the table, after laser coagulation performed in the best world clinics, one child survives in 80-93% of cases, two children in 60-70%. Using the latest ultra-thin fetoscope and trocar, child survival is increased, reaching 98% ^[4] for one child and 79% for both twins. A four-fold reduction in damage to the amniotic membranes, when using ultra-thin technique, gives a gain in an additional 3 weeks to the duration of pregnancy compared with the results when using the classic (old) fetoscopic technique. ^[four]

The leading European center for the treatment of feto-fetal transfusion syndrome is the clinic of obstetrics and perinatal medicine at the University of Halle (Saxony-Anhalt) ^[5] , Germany . The clinic's performance on fetal survival is by far the best in the world. ^[6]

De Wikkellkinderen (Children in Diapers), 1617 , author unknown. Possibly the first SFTT image.

Long flights by air do not cause a deterioration in survival rates after surgery for coagulation of anastomoses in feto-fetal transfusion syndrome. ^[7]

CFT in art

The painting, known as De Wikkellkinderen (Children in Diapers, 1617, author unknown), is considered the first image of the TFT. ^[8] The painting depicts twins, one of which is pale (possibly anemic), and the second is blush (possibly polycythemic). An analysis of the pedigree of the owners of the painting indicates the fact that the twins did not survive. However, there is no evidence that this was due to SFTT.

Notes

↑ Zach T, Ford SP. Twin-to-Twin Transfusion Syndrome. eMedicine.com. URL: http://www.emedicine.com/med/topic3410.htm . Accessed July 22, 2006.
↑ ¹ ² Johnson, A. & Moise, K., J. “Improving Survival in Twin-Twin Transfusion Syndrome.” Contemporary OB / GYN December, 2006. < Archived copy (unspecified) . Date of treatment November 25, 2008. Archived January 9, 2009.
↑ USF Fetal Care Center of Tampa Bay | USF Health Archived March 4, 2009.
↑ ¹ ² ³ M. Tchirikov, J. Mayer, U. Schneider, M. Entezami, M. Bergner. Anwendung der ultradünnen fetoskopischen Technik reduziert die Verletzung der amnialen Membran und verbessert das neonatale Outcome beim TTTS (ClinicalTrials.gov: NCT03151915) (German) // Geburtshilfe und Frauenheilkunde. - Georg Thieme Verlag KG, 2018/05. - Bd. 78 . - S. V 2 . - DOI : 10.1055 / s-0038-1645892 .
↑ [1] -Klinik und Poliklinik für Geburtshilfe und Frauenheilkunde
↑ Feto-fetal transfusion syndrome. Causes and methods of treatment (neopr.) . Date of treatment July 13, 2016.
↑ - PubMed
↑ Berger H., de Waard F., Molenaar Y. A case of twin-to-twin transfusion in 1617. (English) // The Lancet . - Elsevier , 2000. - Vol. 356 , no. 9232 . - P. 847-848 . - DOI : 10.1016 / S0140-6736 (00) 02665-9 . - PMID 11022944 .

Links

[1] Zach T, Ford SP. Twin-to-Twin Transfusion Syndrome. eMedicine.com. URL: http://www.emedicine.com/med/topic3410.htm . Accessed July 22, 2006.

[autogenerated1-2] ¹ ² Johnson, A. & Moise, K., J. “Improving Survival in Twin-Twin Transfusion Syndrome.” Contemporary OB / GYN December, 2006. < Archived copy (unspecified) . Date of treatment November 25, 2008. Archived January 9, 2009.

[3] USF Fetal Care Center of Tampa Bay | USF Health Archived March 4, 2009.

[:0-4] ¹ ² ³ M. Tchirikov, J. Mayer, U. Schneider, M. Entezami, M. Bergner. Anwendung der ultradünnen fetoskopischen Technik reduziert die Verletzung der amnialen Membran und verbessert das neonatale Outcome beim TTTS (ClinicalTrials.gov: NCT03151915) (German) // Geburtshilfe und Frauenheilkunde. - Georg Thieme Verlag KG, 2018/05. - Bd. 78 . - S. V 2 . - DOI : 10.1055 / s-0038-1645892 .

[5] [1] -Klinik und Poliklinik für Geburtshilfe und Frauenheilkunde

[6] Feto-fetal transfusion syndrome. Causes and methods of treatment (neopr.) . Date of treatment July 13, 2016.

[7] - PubMed

[8] Berger H., de Waard F., Molenaar Y. A case of twin-to-twin transfusion in 1617. (English) // The Lancet . - Elsevier , 2000. - Vol. 356 , no. 9232 . - P. 847-848 . - DOI : 10.1016 / S0140-6736 (00) 02665-9 . - PMID 11022944 .