Prenatally Acquired Multiple Limb Ischemia in a Very Low Birth Weight Monochorionic Twin

Straňák, Z; Korček, Peter; Hympánová, Lucie; Kyncl, Martin; Krofta, Ladislav

doi:10.1159/000434644

Cited by 10 publications

(4 citation statements)

References 5 publications

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“…Hematological complications are commonly seen in TAPS donors and recipients, requiring blood transfusion or PET, respectively. TAPS recipients may develop polycythemia hyperviscosity syndrome, which may possibly lead to necrosis of the skin and multiple limb ischemia (Robyr et al, 2006; Stranak et al, 2015). In addition, recipients are at increased risk of thrombocytopenia, probably due to impaired production secondary to tissue hypoxia and slow spleen blood flow (Lopriore et al, 2010; Sarkar & Rosenkrantz, 2008).…”

Section: Neonatal and Pediatric Outcomementioning

confidence: 99%

Twin Anemia Polycythemia Sequence: Current Views on Pathogenesis, Diagnostic Criteria, Perinatal Management, and Outcome

et al. 2016

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Monochorionic twins share a single placenta and are connected with each other through vascular anastomoses. Unbalanced inter-twin blood transfusion may lead to various complications, including twin-to-twin transfusion syndrome (TTTS) and twin anemia polycythemia sequence (TAPS). TAPS was first described less than a decade ago, and the pathogenesis of TAPS results from slow blood transfusion from donor to recipient through a few minuscule vascular anastomoses. This gradually leads to anemia in the donor and polycythemia in the recipient, in the absence of twin oligo-polyhydramnios sequence (TOPS). TAPS may occur spontaneously in 3-5% of monochorionic twins or after laser surgery for TTTS. The prevalence of post-laser TAPS varies from 2% to 16% of TTTS cases, depending on the rate of residual anastomoses. Pre-natal diagnosis of TAPS is currently based on discordant measurements of the middle cerebral artery peak systolic velocity (MCA-PSV; >1.5 multiples of the median [MoM] in donors and <1.0 in recipients). Post-natal diagnosis is based on large inter-twin hemoglobin (Hb) difference (>8 g/dL), and at least one of the following: reticulocyte count ratio >1.7 or minuscule placental anastomoses. Management includes expectant management, and intra-uterine blood transfusion (IUT) with or without partial exchange transfusion (PET) or fetoscopic laser surgery. Post-laser TAPS can be prevented by using the Solomon laser surgery technique. Short-term neonatal outcome ranges from isolated inter-twin Hb differences to severe neonatal morbidity and neonatal death. Long-term neonatal outcome in post-laser TAPS is comparable with long-term outcome after treated TTTS. This review summarizes the current knowledge after 10 years of research on the pathogenesis, diagnosis, management, and outcome in TAPS.

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Section: Neonatal and Pediatric Outcomementioning

confidence: 99%

Twin Anemia Polycythemia Sequence: Current Views on Pathogenesis, Diagnostic Criteria, Perinatal Management, and Outcome

et al. 2016

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“…Further work is also required to elucidate the benefits of intrauterine treatment. While grave outcomes such as neonatal death, ischemic loss of fetal limbs, and cerebellar disruption have been described, it is unclear whether a treatment protocol based on MCA-PSV values can prevent these outcomes [1,[74][75][76].…”

Section: Discussionmentioning

confidence: 99%

Middle Cerebral Artery Doppler Velocimetry for the Diagnosis of Twin Anemia Polycythemia Sequence: A Systematic Review

Brock

Bergh

Moise

et al. 2020

JCM

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Twin anemia polycythemia sequence (TAPS) is a rare complication of monochorionic diamniotic (MCDA) twins. Middle cerebral artery peak systolic velocity (MCA-PSV) measurements are used to screen for TAPS while fetal or neonatal hemoglobin levels are required for definitive diagnosis. We sought to perform a systematic review of the efficacy of MCA-PSV in diagnosing TAPS. Search criteria were developed using relevant terms to query the Pubmed, Embase, and SCOPUS electronic databases. Publications reporting diagnostic characteristics of MCA-PSV measurements (i.e., sensitivity, specificity or receiver operator curves) were included. Each article was assessed for bias using the Quality Assessment of Diagnostic Accuracy Studies II (QUADAS II) tool. Results were assessed for uniformity to determine whether meta-analysis was feasible. Data were presented in tabular form. Among publications, five met the inclusion criteria. QUADAS II analysis revealed that four of the publications were highly likely to have bias in multiple areas. Meta-analysis was precluded by non-uniformity between definitions of TAPS by MCA-PSV and neonatal or fetal hemoglobin levels. High-quality prospective studies with consistent definitions and ultrasound surveillance protocols are still required to determine the efficacy of MCA-PSV in diagnosing TAPS. Other ultrasound findings (e.g., placenta echogenicity discordance) may augment Doppler studies.

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“…In contrast, recipients may be severely polycythemic and require PET in 40-71% of cases [36][37][38][39]. Severe hyperviscosity in recipients may lead to necrosis of the skin and multiple limb ischemia or severe cerebral injury [23,40]. Prompt detection and treatment at birth with PET is of paramount importance.…”

Section: Hematological Disorders In Tapsmentioning

confidence: 99%

Hematological disorders at birth in complicated monochorionic twins

Verbeek

Slaghekke

Sueters

et al. 2017

Expert Review of Hematology

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Monochorionic twins are at risk of severe complications including twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS) and acute peripartum TTTS. The pathophysiology is based on inter-twin blood transfusion through placental vascular anastomoses. Areas covered: This review focuses on the incidence, management and outcome of neonatal hematological complications at birth in TTTS, TAPS and acute peripartum TTTS. Expert commentary: Hematological disorders are often present at birth in monochorionic twins and include acute or chronic anemia, polycythemia and thrombocytopenia. Routine measurement of complete blood counts in all complicated monochorionic twins is strongly recommended. Increased awareness on these disorders and correct diagnostic tests will lead to prompt and adequate management at birth.

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Prenatally Acquired Multiple Limb Ischemia in a Very Low Birth Weight Monochorionic Twin

Cited by 10 publications

References 5 publications

Twin Anemia Polycythemia Sequence: Current Views on Pathogenesis, Diagnostic Criteria, Perinatal Management, and Outcome

Twin Anemia Polycythemia Sequence: Current Views on Pathogenesis, Diagnostic Criteria, Perinatal Management, and Outcome

Middle Cerebral Artery Doppler Velocimetry for the Diagnosis of Twin Anemia Polycythemia Sequence: A Systematic Review

Hematological disorders at birth in complicated monochorionic twins

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