In twin-to-twin transfusion syndrome, which one of the following statements is true?

Patients initially will be identified by ultrasound examination by their primary provider. Consultation and subsequent ultrasound confirmation will be undertaken by our group. Patients meeting the inclusion criteria will undergo extensive verbal counseling regarding the clinical findings, prognosis, and management options. Those electing to proceed will sign written informed consent documents.

More specifically, after initial referral to our center for TTTS management, patients will undergo targeted ultrasound to confirm the diagnosis. If confirmed, patients will undergo extensive counseling regarding various management options. Those patients electing to proceed with selective laser photocoagulation (S-LPC) will undergo the informed consent process. The S-LPC will be performed in the Labor and Delivery (L&D), Operating Room (OR) at the Regional Medical Center, Regional One Health (RMC,ROH). In the majority of cases, maternal anesthesia will be an epidural. In those cases where the patient is unable to lay supine due to an enlarged uterus (resulting in maternal hypotension or respiratory insufficiency, or maternal anxiety), general anesthesia with intubation will be administered. In rare cases, intravenous sedation with infiltration of a local anesthetic into the skin, deep muscle, and fascia will be used. Following the administration of maternal anesthesia, ultrasound will be performed to assess fetal position, placentation, and select a site for insertion of the operative instruments. A small skin incision will be made following administration of local anesthetic to allow percutaneous access to the recipient gestational sac. An 18 gauge needle will be inserted through the maternal abdomen and uterus into the gestational sac. Once secured in place, the stylet will be removed and a J guide wire will be inserted through the needle. The needle will be removed and a 10-12 Fr (3-3.4 mm) trocar and cannula will be inserted into the sac over the guide wire. The trocar and guide wire will be removed and the fetoscopy instruments will be introduced through the cannula. The procedure is performed under continuous ultrasound guidance. After introduction of the fetoscope and operating sheath, the placenta is inspected by direct visualization for communicating vessels between the recipient and donor twin. A 400-600 micron laser fiber is introduced into the gestational sac via an instrument channel in the operating sheath. The fiber is directed to the communicating vessels, which are then ablated with thermal energy. An average of 7-15 sites will be ablated. At the completion of the procedure the amniotic fluid volume in the recipient sac will be reduced to a normal volume. Follow-up visits to track maternal and fetal progress after the procedure will be scheduled with our group.

Abstract

Background

In utero limb ischemia is a rare complication of the monochorionic twin pregnancies complicated with twin to twin transfusion syndrome (TTTS). The condition is more often seen in recipient twins. There are few theories of the pathogenesis including in utero venous thromboembolism, but the cause remains unclear. However, limb ischemia is thought to be unrelated with any prenatal intervention.

Case Presentation

We present a case of a monochorionic twin pregnancy complicated with TTTS admitted to the Clinic for selective fetoscopic laser photocoagulation. The invasive procedure failed due to poor visibility. In the following weeks of pregnancy, amnioreduction procedures were performed. At 28 weeks of gestation due to twin anemia-polycythemia sequence diagnosis the patient was qualified for cesarean section. Postnatally, the donor twin was diagnosed with lower right limb ischemic necrosis. The extremity was amputated 2 days later with an uncomplicated recovery. After speculations of the potential pathogeneses it was suggested that the ischemic limb occurred as a complication of the main condition – TTTS.

Conclusions

In literature, there have been no cases reported of TTTS stage I complicated with donor twin limb ischemia. The actual cause of the in utero limb ischemic necrosis in monochorionic twins remains unknown. Nevertheless, increased attention to the potential complication after failed invasive procedures or conservative treatment should be required.

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Background

Twin-to-twin transfusion syndrome (TTTS) is one of the complications of monochorionic twin pregnancies, occurring in approximately 10–15% [1, 2]. The classification of severity of the disease is based on the Quintero staging system [3, 4]. Untreated TTTS has a very high mortality rate and morbidity including neurologic and cardiovascular complications [2, 5, 6]. Although there are few treatment strategies for managing TTTS, selective laser photocoagulation (SLP) is thought to be the most cost-effective treatment [7, 8]. SLP improves perinatal outcomes by decreasing the risk of fetal demise and postnatal complications [9, 10].

One of the rare complications of TTTS is prenatal limb ischemia. In most cases, it affects the recipient twin [11]. There are few theories of the pathogenesis including in utero venous thromboembolism, but the cause remains unclear [12, 13]. However, limb ischemia is thought to be unrelated with any prenatal intervention [14].

Case Presentation

A 28-year-old multiparous patient was admitted to a tertiary referral center, 1st Department of Obstetrics and Gynecology, Medical University of Warsaw at 22 weeks and 0 days with Quintero stage I twin-to-twin transfusion syndrome. The recipient twin had the deepest vertical pocket of 10.3 cm, while the donor had anhydramnios. Doppler evidence of cardiac dysfunction was seen in both fetuses. After informed consent the patient underwent selective laser photocoagulation. A fetoscope was introduced into the recipient twin sac visualizing the intertwin membrane and the chorionic plate. Arteriovenous anastomoses were evaluated, but after the first coagulation procedure visibility had deteriorated significantly due to bleeding from the entry site. It was decided to stop the procedure due to safety reasons. The patient was monitored weekly after the procedure with ultrasound scans. Due to persistent polyhydramnios of the recipient twin, amnioreduction procedures were performed. The patient underwent four procedures of amnioreduction with an interval of 7 days (the amount of amniotic fluid withdrawn ranged from 1700 to 2500 ml). Noteworthy was the colour of amniotic fluid, that was not transparent but brown suggesting the high risk of bleeding while attempting more invasive procedures. No fetal anomaly was visualized on follow up ultrasound scans.

At 28 weeks and 6 days the patient was diagnosed with twin anemia-polycythemia sequence stage 1(TAPS). Due to the high risk of bleeding from the entry site the patient was disqualified from any invasive procedure as treatment of TAPS. The patient was admitted to the hospital and qualified for prenatal corticosteroids (Betamethasone). After the antenatal steroid therapy, the patient was qualified for a caesarean section.

Twin I (the recipient twin) birth’s weight was 1340 g with an Apgar score of 7/8 at 1 and 5 min. Twin II (the donor twin) birth’s weight was 1240 g with an Apgar score of 5/8 at 1 and 5 min. The right lower extremity of the donor twin was noted as ischemic at the thigh level (shown in Fig. 1 and 2). Both newborns demanded intubation and mechanical ventilation with surfactant treatment due to respiratory distress.

Fig. 1

Right lower extremity ischemic necrosis at delivery

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Fig. 2

Right lower extremity ischemic necrosis at delivery

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The recipient twin had a recorded haemoglobin of 15.8 g/dL. The donor twin had a recorded haemoglobin of 11.8 g/dL. Therefore, postnatally there was no evidence of twin anemia/polycythemia sequence. Few hours after birth the impaired function of the donor’s right kidney was diagnosed. Unilateral renal ischemia on the same side as limb necrosis suggested the embolic cause of the complication. The donor twin’s right lower extremity was amputated at the Department of Paediatric Surgery 2 days after delivery. The twin had an uncomplicated recovery after procedure. Both twins had complications typical for preterm delivery but remained stable at the Neonatal Intensive Care Unit.

Discussion and conclusions

Limb ischemic injury is a rare condition associated with twin-to-twin transfusion syndrome [12]. It is correlated with more conservative treatment including amnioreduction rather than successful SLP [14]. In most cases, vascular limb occlusion affects lower extremities (approximately 80%) of the recipient twins [15]. There are few theories suggesting the pathogenesis of the complication in recipient twins including polycythaemia/hyperviscosity syndrome, elevated level of vasoconstrictive hormones and venous thromboembolism [16].

In our case report, ischemic injury affected the donor twin lower extremity. The cause of the complication in donor twins is unknown. There are only a few cases described in the literature, but none of them explained the mechanism of in utero limb ischemia in donor twins [17]. Furthermore, most cases were related with advanced Quintero stage disease (in most of the reports stage III or IV) [14]. Therefore, the first hypothesis in our case (Quintero stage I; limb ischemia of the donor twin) was that the limb damage was related directly to SLP or due to the complication of the procedure- amniotic band disruption. After analysis of the case one of the thesis was disproved—SLP was stopped at the beginning of the procedure due to poor visibility. The second potential cause—amniotic band disruption was excluded due to the fact, that the fetuses where delivered via cesarean section in their amniotic sacs (En Caul Birth) with no evidence of the rupture of amniotic membranes. Based on that, it was suggested that the ischemic injury may be related to the main condition – TTTS. The coexisting impaired function of the right kidney and the length of the limb also suggested, that the ischemia of the lower extremity had occurred with no correlation with the invasive procedure, but as a result of thromboembolism.

In utero limb ischemia is a severe complication of TTTS. It occurs with the incidence rate of 0.5% [15]. Based on recent systematic review invasive procedures improve survival rates of twins in pregnancies complicated with TTTS Stage 1. Consequently, more cases are going to be qualified to SLP in early stages of TTTS [18]. Thus, neonates and obstetricians should be aware of the complication and before any treatment inform the patients about the risk of in utero limb injury in TTTS complicated twins. This case is unusual, because the ischemia of the lower extremity occurred in the donor twin in monochorionic twin pregnancy complicated with TTTS stage I. Therefore, it gives new insight into prenatal limb ischemic injury. Our case report supports the hypothesis that in utero limb ischemia is rather a complication of TTTS than SLP. However, further research is needed to reveal the mechanism of the ischemic limb injury in monochorionic twin pregnancies complicated with TTTS.

Availability of data and materials

All data generated or analysed during this study are included in this published article and its supplementary information files.

Abbreviations

TTTS:

Twin-to-twin transfusion syndrome

SLP:

Selective laser photocoagulation

References

Simpson LL, Society for Maternal-Fetal M. Twin-twin transfusion syndrome. Am J Obstet Gynecol. 2013;208(1):3–18.
Article PubMed Google Scholar
Bamberg C, Hecher K. Update on twin-to-twin transfusion syndrome. Best Pract Res Clin Obstet Gynaecol. 2019;58:55–65.
Article PubMed Google Scholar
Kontopoulos E, Chmait RH, Quintero RA. Twin-to-Twin Transfusion Syndrome: Definition, Staging, and Ultrasound Assessment. Twin Res Hum Genet. 2016;19(3):175–83.
Article PubMed Google Scholar
Quintero RA, Morales WJ, Allen MH, Bornick PW, Johnson PK, Kruger M. Staging of twin-twin transfusion syndrome. J Perinatol. 1999;19(8 Pt 1):550–5.
Article CAS PubMed Google Scholar
Hikino S, Ohga S, Kanda T, Nakashima T, Yamamoto J, Nakayama H, et al. Long-term outcome of infants with twin-to-twin transfusion syndrome. Fetal Diagn Ther. 2007;22(1):68–74.
Article PubMed Google Scholar
Merhar SL, Kline-Fath BM, Meinzen-Derr J, Schibler KR, Leach JL. Fetal and postnatal brain MRI in premature infants with twin-twin transfusion syndrome. J Perinatol. 2013;33(2):112–8.
Article CAS PubMed Google Scholar
Khalek N, Johnson MP, Bebbington MW. Fetoscopic laser therapy for twin-to-twin transfusion syndrome. Semin Pediatr Surg. 2013;22(1):18–23.
Article PubMed Google Scholar
Odibo AO, Caughey AB, Grobman W, Stamilio DM, Ville Y. Selective laser photocoagulation versus serial amniodrainage for the treatment of twin-twin transfusion syndrome: a cost-effectiveness analysis. J Perinatol. 2009;29(8):543–7.
Article CAS PubMed Google Scholar
Hecher K, Gardiner HM, Diemert A, Bartmann P. Long-term outcomes for monochorionic twins after laser therapy in twin-to-twin transfusion syndrome. Lancet Child Adolesc Health. 2018;2(7):525–35.
Article PubMed Google Scholar
Spruijt MS, Lopriore E, JS S, Slaghekke F, Van Klink JMM. Twin-twin transfusion syndrome in the era of fetoscopic laser surgery: antenatal management, neonatal outcome and beyond. Expert Rev Hematol. 2020;13(3):259–67.
Article CAS PubMed Google Scholar
Carr SR, Luks F, Tracy T, Plevyak M. Antenatal necrotic injury in severe twin-to-twin transfusion syndrome. A case and review. Fetal Diagn Ther. 2004;19(4):370–2.
Article PubMed Google Scholar
Lopriore E, Walther FJ, Oepkes D, Vandenbussche FP. On the pathogenesis of recipient twin limb ischemia. J Pediatr. 2007;151(1):e4 author reply e-5.
Article PubMed Google Scholar
Stranak Z, Korcek P, Hympanova L, Kyncl M, Krofta L. Prenatally Acquired Multiple Limb Ischemia in a Very Low Birth Weight Monochorionic Twin. Fetal Diagn Ther. 2017;41(3):237–8.
Article PubMed Google Scholar
Schrey S, Huber A, Hecher K, Quintero R, Alkazaleh F, Moise Jr KJ, et al. Vascular limb occlusion in twin-twin transfusion syndrome (TTTS): case series and literature review. Am J Obstet Gynecol. 2012;207(2):131 e1-10.
Article Google Scholar
Kilby M, Pounds R, Mannix P. Fetal Limb Ischaemia in Twin-to-Twin Transfusion Syndrome. Case Rep Pediatr. 2013;2013:278726.
PubMed PubMed Central Google Scholar
Broadbent RS. Recipient twin limb ischemia with postnatal onset. J Pediatr. 2007;150(2):207–9.
Article PubMed Google Scholar
Ballard HO, Shook L, Lain KY, Burns L, Crombleholme TM. An embolic complication in a donor twin with severe twin-twin transfusion syndrome after fetoscopic intervention. J Perinatol. 2009;29(3):250–1.
Article CAS PubMed Google Scholar
Di Mascio D, Khalil A, D’Amico A, Buca D, Benedetti Panici P, Flacco ME, et al. Outcome of twin-twin transfusion syndrome according to Quintero stage of disease: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2020;56(6):811–20.

Article PubMed Google Scholar

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Authors and Affiliations

1st Department of Obstetrics and Gynecology, Medical University of Warsaw, Warsaw, Poland
Agata Majewska, Robert Brawura-Biskupski-Samaha, Dorota Bomba-Opoń, Iwona Szymusik & Mirosław Wielgoś
The Institute of Mother and Child, Warsaw, Poland
Szymon Kozłowski
Students Scientific Association at the 1st Department of Obstetrics and Gynecology, Medical University of Warsaw, Warsaw, Poland
Olga Płaza

Authors

Agata Majewska
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Robert Brawura-Biskupski-Samaha
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Szymon Kozłowski
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Dorota Bomba-Opoń
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Iwona Szymusik
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Olga Płaza
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Mirosław Wielgoś
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AM, RBBS, SK, OP have coordinated drafting of the manuscript. DBO, IS and MW have coordinated revising of the manuscript. All authors listed have read and approved the final version of the manuscript.

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Correspondence to Robert Brawura-Biskupski-Samaha.

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Majewska, A., Brawura-Biskupski-Samaha, R., Kozłowski, S. et al. Twin-to-twin transfusion syndrome complicated with in utero limb ischemia of the donor twin – a case report. BMC Pregnancy Childbirth 22, 97 (2022). https://doi.org/10.1186/s12884-022-04429-0

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Received: 18 March 2021
Accepted: 26 January 2022
Published: 04 February 2022
DOI: https://doi.org/10.1186/s12884-022-04429-0

Keywords

Selective laser photocoagulation
Prenatal limb ischemia
Twin-to-twin transfusion syndrome

What happens in twin to twin transfusion syndrome?

One twin — the donor twin — gives away more blood than it receives in return and runs the risk of malnourishment and organ failure. The recipient twin receives too much blood and is susceptible to overwork of the heart and other cardiac complications.

What are the symptoms of twin to twin transfusion syndrome?

What are the signs of TTTS?.

A uterus that measures large for her stage of pregnancy..

Feeling like her abdomen is growing or expanding rapidly..

A sudden increase in body weight..

Increased abdominal pressure or pain..

Shortness of breath..

Uterine cramping or contractions..

Swelling of the hands and legs early in the pregnancy..

What causes twin to twin transfusion syndrome?

Causes. The exact cause of TTTS is not fully understood. However, it is known that abnormalities during division of the mother's egg after it has been fertilized lead to the placental abnormalities that can ultimately result in twin-twin transfusion syndrome.

What is twin to twin transfusion syndrome treatment?

Twin-twin transfusion syndrome can be treated with fetal laser photocoagulation (FLP), which selectively and sequentially ablates connections, depending on the type, to prevent the loss of one or both fetuses.