Society for Maternal-Fetal Medicine Consult Series #72: Twin-twin transfusion syndrome and twin anemia-polycythemia sequence.

30% of spontaneously occurring twins are monozygotic, of which two-thirds are monochorionic, possessing a single placenta. A common placental mass with shared inter-twin placental circulation is key to the development and management of complications unique to monochorionic gestations. In this Consult, we review general considerations and a contemporary approach to twin-twin transfusion syndrome and twin anemia polycythemia sequence, providing management recommendations based on the available evidence. The following are Society for Maternal-Fetal Medicine recommendations: (1) we recommend routine first trimester sonographic determination of chorionicity and amnionicity (GRADE 1B); (2) we recommend that ultrasound surveillance for twin-twin transfusion syndrome begin at 16 weeks of gestation for all monochorionic-diamniotic twin pregnancies and continue at least every 2 weeks until delivery, with more frequent monitoring indicated with clinical concern (GRADE 1C); (3) we recommend that routine sonographic surveillance for twin-twin transfusion syndrome minimally include assessment of amniotic fluid volumes on both sides of the inter-twin membrane and evaluation for the presence or absence of urine-filled fetal bladders, and ideally incorporate Doppler study of the umbilical arteries (GRADE 1C); (4) we recommend fetoscopic laser surgery as the standard treatment for stage II through stage IV twin-twin transfusion syndrome presenting between 16 and 26 weeks of gestation (GRADE 1A); (5) we recommend expectant management with at least weekly fetal surveillance for asymptomatic patients continuing pregnancies complicated by stage I twin-twin transfusion syndrome, and consideration for fetoscopic laser surgery for stage I twin-twin transfusion syndrome presentations between 16 and 26 weeks of gestation complicated by additional factors such as maternal polyhydramnios-associated symptomatology (GRADE 1B); (6) we recommend an individualized approach to laser surgery for early- and late-presenting twin-twin transfusion syndrome (GRADE 1C); (7) we recommend that all patients with twin-twin transfusion syndrome qualifying for laser therapy be referred to a fetal intervention center for further evaluation, consultation, and care (Best Practice); (8) after laser therapy, we suggest weekly surveillance for 6 weeks followed by resumption of every-other-week surveillance thereafter, unless concern exists for post-laser twin-twin transfusion syndrome, post-laser twin anemia polycythemia sequence, or fetal growth restriction (GRADE 2C); (9) following the resolution of twin-twin transfusion syndrome after fetoscopic laser surgery, and without other indications for earlier delivery, we recommend delivery of dual-surviving monochorionic-diamniotic twins at 34 to 36 weeks of gestation (GRADE 1C); (10) in twin-twin transfusion syndrome pregnancies complicated by post-treatment single fetal demise, we recommend full-term delivery (39 weeks) of the surviving co-twin to avoid complications of prematurity unless indications for earlier delivery exist (GRADE 1C); (11) we recommend that fetoscopic laser surgery should not influence the mode of delivery (Best Practice); (12) we recommend that prenatal diagnosis of twin anemia polycythemia sequence minimally requires either middle cerebral artery Doppler peak systolic velocity values >1.5 multiples of the median and <1.0 multiples of the median in donor and recipient twins, respectively, or an inter-twin Δ middle cerebral artery peak systolic velocity >0.5 multiples of the median (GRADE 1C); (13) we recommend that providers consider incorporating middle cerebral artery Doppler peak systolic velocity determinations into all monochorionic twin ultrasound surveillance beginning at 16 weeks of gestation (GRADE 1C); (14) consultation with a specialized fetal care center is recommended when twin anemia polycythemia sequence progresses to a more advanced disease stage (≥ stage II) prior to 32 weeks of gestation or when concern arises for co-existing complications such as twin-twin transfusion syndrome (Best Practice).

HEP® (Homeostasis-Enrichment-Plasticity) Approach Changes Sensory-Motor Development Trajectory and Improves Parental Goals: A Single Subject Study of an Infant with Hemiparetic Cerebral Palsy and Twin Anemia Polycythemia Sequence (TAPS).

BACKGROUND: Early intervention (EI) for infants identified as being at high risk for cerebral palsy (CP), or who have been diagnosed with it, is critical for promotion of postnatal brain organization. The aim of this study was to explore the effectiveness of the Homeostasis-Enrichment-Plasticity (HEP) Approach, which is a contemporary EI model that applies the key principles of enriched environment paradigms and neuronal plasticity from experimental animal studies to ecological theories of human development on the motor development, sensory functions, and parental goals of an infant with twin anemia polycythemia sequence (TAPS) and CP. METHODS: An AB phase with follow-up single case study design which consisted of multiple baseline assessments with the Peabody Developmental Motor Scales-2 (PDMS-2) and the Test of Sensory Functions in Infants (TSFI) was used. Non-overlapping confidence intervals analysis was used for pre-post PDMS-2 scores. The measurement of progress toward goals and objectives was conducted using the Goal Attainment Scale (GAS). The HEP Approach intervention consisted of 12 one-hour sessions implemented over a period of 3 months, where a physical therapist provided weekly clinic-based parental coaching. RESULTS: Results found a stable baseline during Phase A and improvement in response to the HEP Approach intervention during Phase B in both the PDMS-2 and TSFI according to 2SD Band analysis. The confidence intervals for the PDMS-2 scores also indicated a significant improvement after HEP intervention. The scores for both the PDMS-2 and the TSFI were consistent or showed improvement throughout the Follow-Up phase. A GAS t-score of 77.14 indicated that the infant exceeded intervention goal expectations. CONCLUSIONS: Although our findings suggest that the HEP Approach intervention has promise in enhancing sensory functions, motor skill outcomes, and parental goals in an infant with TAPS and CP, further research is required to validate and apply these results more broadly.

Monochorionic Twin Complications and Fetoscopic Interventions.

Monochorionic twins are at risk for complications due to the presence of placental vascular anastomoses, including twin-twin transfusion syndrome, twin anemia-polycythemia sequence, selective fetal growth restriction, and twin reversed arterial perfusion sequence. While ultrasound is the primary modality to screen for the development of these complications, MRI plays an important role in assessing monochorionic twin pregnancies for the development of other complications, such as neurologic injury. In this article, the authors review the ultrasound imaging findings associated with monochorionic twin complications, management options, and the role for MRI in these pregnancies.

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

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.

A Case of Spontaneous Twin Anemia-Polycythemia Sequence

Twin anemia-polycythemia sequence (TAPS) is characterized by the transfer of blood via intertwin vascular anastomoses in a single placenta. It differs from twin-twin transfusion syndrome (TTTS) in that amniotic fluid levels remain normal during pregnancy. Since the concept of TAPS was first introduced in 2007, some cases have been reported abroad, but no such a case has been reported in Korea. Here, we report the first case of spontaneous TAPS in Korea.

Sequência anemia-policitemia em gêmeos: definição, classificação e tratamento / Twin anemia-polycythemia sequence: definition, classification and treatment

A presença de anastomoses vasculares placentárias nas gestações gemelares monocoriônicas favorece o surgimento de complicações graves como a Síndrome de Transfusão Feto-Fetal (STFF) e a Sequência Anemia-Policitemia em Gêmeos (TAPS). STFF e TAPS são ambas formas crônicas de transfusão feto-fetal. TAPS, descrita em 2007, é caracterizada por anemia crônica no gêmeo doador e policitemia no gêmeo receptor, na ausência de discordância no volume de líquido amniótico, característica da STFF clássica. O diagnóstico pode ser feito pré- e pós-natal. O critério diagnóstico pré-natal é baseado na discordância intergemelar da medida do Doppler do pico de velocidade sistólica da artéria cerebral média (PSV-ACM). O diagnóstico pós-natal é baseado na grande diferença da hemoglobina intergemelar, ao nascimento, e reticulocitose no gêmeo doador.(AU)

The presence of placental vascular anastomoses in monochorionic twins favors the development of several complications like Twin-Twin Transfusion Syndrome (TTTS) and Twin Anemia-Polycythemia Sequence (TAPS). TTTS and TAPS are both chronic forms of fetofetal transfusion. TAPS, described on 2007, is characterized by chronic anemia in the donor twin and polycythemia in the recipient twin, in the absence of amniotic fluid volume discordances, characteristic of classical TTTS. The diagnostic can be made pree posnatally. The prenatal diagnostic criteria is based on the inter-twin discordance in Doppler measurement of peak systolic velocity in the middle cerebral artery (MCA-PSV). The postnatal diagnosis is based on large intertwin hemoglobin difference at birth and retoculocytosis in the donor twin.(AU)

Complicaciones tardías de la terapia láser como tratamiento de la transfusión feto-fetal: caso clínico / Late complications of laser therapy as a treatment for feto-fetal transfusion: clinical case

Presentamos la descripción del diagnóstico y manejo de una secuencia anemia-policitemia (SAP) que se presenta como complicación de una terapia láser exitosa en un embarazo gemelar monocorial cursando una transfusión feto-fetal (TFF) severa. Describimos la manifestación de esta complicación tardía de la terapia láser de la TFF severa y realizamos una revisión de la literatura internacional al respecto. A pesar del éxito de la introducción de la terapia láser en cuanto a la sobrevida y secuelas neonatales, recientemente se han descrito una serie de complicaciones de presentación tempranas o tardías. Entre las tardías, destacan la muerte de uno o ambos gemelos, recidiva de la TFF, y aparición de una SAP. Varios autores han descrito que la SAP sería secundaria a la presencia, o persistencia, de comunicaciones vasculares extremadamente pequeñas de flujo lento, las cuales llevan a una discordancia en los niveles de hemoglobina entre ambos gemelos, sin diferencias en sus volúmenes sanguíneos.

We describe the diagnosis and management of twin anemia-polycythemia sequence (TAPS), which occurs as a late complication of successful laser therapy in twin monochorionic pregnancies developing severetwin to twin transfusion syndrome (TTTS). We offer a description of this late complication of laser therapy in this condition and a review of the related medical literature. Despite the successful introduction of laser therapy on the survival and neonatal sequelae, various early and late complications related to this procedure have been recently described. Among the late, stands out the death of one or both twins, recurrence of TTTS, and the appearance of TAPS. With regards TAPS, several authors have reported that it would be secondary to the presence, o persistence, of extremely small slow flow vascular communications, which lead to discre-pancies in the hemoglobin levéis between the twins, with no differences in blood volume.