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      Prenatal diagnosis of spontaneous twin anemia-polycythemia sequence and postnatal examination of placental vascular anastomoses

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          Abstract

          Twin anemia-polycythemia sequence (TAPS) is characterized by a wide discrepancy of hemoglobin between two monochorionic fetuses without sign of twin oligo-polyhydramnios sequence. A primiparous woman with monochorionic diamniotic twin transferred for preterm labor. Ultrasonographic evaluation at 32+3 weeks of gestation revealed increased middle cerebral artery-peak systolic velocity (77.4 cm/sec, 1.69 multiples of median) in donor and decreased in recipient twin (36.4 cm/sec, 0.79 multiples of median), the twin was diagnosed with TAPS. Repeated cesarean section was performed at 32+5 weeks of gestation following preeclampsia and preterm labor. After delivery, TAPS was confirmed through neonatal hematologic examination. There were no signs of acute hemorrhagic shock or brain injury. Placental evaluation via dye infusion and barium angiogram revealed one arterioarterial anastomoses with six arteriovenous anastomoses of placenta. We report a prenatally diagnosed case of spontaneous TAPS with arterioarterial and arteriovenous anastomoses and suggest careful monitoring of monochorionic twin and opinion on placenta vascular architecture.

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          Twin anemia-polycythemia sequence: diagnostic criteria, classification, perinatal management and outcome.

          Monochorionic twins share a single placenta with intertwin vascular anastomoses, allowing the transfer of blood from one fetus to the other and vice versa. These anastomoses are the essential anatomical substrate for the development of several complications, including twin-twin transfusion syndrome (TTTS) and twin anemia-polycythemia sequence (TAPS). TTTS and TAPS are both chronic forms of fetofetal transfusion. TTTS is characterized by the twin oligopolyhydramnios sequence, whereas TAPS is characterized by large intertwin hemoglobin differences in the absence of amniotic fluid discordances. TAPS may occur spontaneously in up to 5% of monochorionic twins and may also develop after incomplete laser treatment in TTTS cases. This review focuses on the pathogenesis, incidence, diagnostic criteria, management options and outcome in TAPS. In addition, we propose a classification system for antenatal and postnatal TAPS.
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            Twin anemia-polycythemia sequence in two monochorionic twin pairs without oligo-polyhydramnios sequence.

            Placental vascular anastomoses in monochorionic twins may lead to acute or chronic inter-twin transfusion. We report an uncommon form of chronic inter-twin transfusion, referred to as twin anemia-polycythemia sequence (TAPS), with severe anemia in one twin and polycythemia in the other, without the characteristically associated twin oligo-polyhydramnios sequence (TOPS) seen in the classical twin-to-twin transfusion syndrome (TTTS). The clinical course and placental characteristics of two pairs of monochorionic twins with TAPS born at, respectively, 33 and 34 weeks' gestation were reviewed. Serial fetal ultrasound examinations revealed no signs of TOPS. At birth, both donor twins were severely anemic requiring blood transfusion and both recipients were polycythemic, one requiring partial volume exchange transfusions. Inter-twin difference in reticulocyte counts was extremely high, suggesting a chronic form of inter-twin blood transfusion. Placental injection studies revealed a preponderance of very small (<1mm) arterio-venous anastomoses in one direction. In conclusion, chronic inter-twin transfusion may lead to an uncommon form of inter-twin transfusion, named TAPS, not associated with TOPS, resulting in severe fetal or neonatal hematological complications. We hypothesize that TAPS is mediated through minuscule unidirectional anastomoses. TAPS can be diagnosed antenatally with Doppler studies and postnatally by hemoglobin and reticulocyte measurements.
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              Severe cerebral injury in a recipient with twin anemia-polycythemia sequence.

              Twin anemia-polycythemia sequence (TAPS) results from slow intertwin blood transfusion through minuscule placental vascular anastomoses and is characterized by large intertwin hemoglobin differences in the absence of amniotic fluid discordance. The optimal management of TAPS is not clear. We report a case of TAPS detected antenatally by Doppler ultrasound examination at 15 + 6 weeks' gestation. After counseling, the parents opted for expectant management. Regular Doppler measurements were performed and these remained fairly stable. An emergency Cesarean section was performed at 34 + 5 weeks following signs of fetal distress. The donor twin was severely anemic while the recipient twin had severe polycythemia-hyperviscosity syndrome. On day 1, the recipient developed respiratory insufficiency and subclinical status epilepticus. Magnetic resonance imaging showed a total loss of gray-white matter differentiation as a sign of severe diffuse cerebral ischemia and bilateral intra- and extra-axial hemorrhages. There was almost complete lack of arterial and venous cerebral blood flow. On day 3 intensive care treatment was withdrawn in view of the severity of the brain injury. This case report demonstrates that TAPS may lead to severe cerebral injury and fatal outcome in the recipient twin, and highlights the importance of antenatal Doppler ultrasound monitoring and choice of management.
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                Author and article information

                Journal
                Obstet Gynecol Sci
                Obstet Gynecol Sci
                OGS
                Obstetrics & Gynecology Science
                Korean Society of Obstetrics and Gynecology; Korean Society of Contraception and Reproductive Health; Korean Society of Gynecologic Endocrinology; Korean Society of Gynecologic Endoscopy and Minimal Invasive Surgery; Korean Society of Maternal Fetal Medicine; Korean Society of Ultrasound in Obstetrics and Gynecology; Korean Urogynecologic Society
                2287-8572
                2287-8580
                November 2016
                15 November 2016
                : 59
                : 6
                : 539-543
                Affiliations
                Department of Obstetrics and Gynecology, Catholic University of Daegu School of Medicine, Daegu, Korea.
                Author notes
                Corresponding author: Seong Yeon Hong. Department of Obstetrics and Gynecology, Catholic University of Daegu School of Medicine, 33 Duryugongwon-ro 17-gil, Nam-gu, Daegu 42472, Korea. Tel: +82-53-650-4078, Fax: +82-53-650-4078, magu815@ 123456cu.ac.kr
                Article
                10.5468/ogs.2016.59.6.539
                5120076
                Copyright © 2016 Korean Society of Obstetrics and Gynecology

                Articles published in Obstet Gynecol Sci are open-access, distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report
                Maternal-Fetal Medicine

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