Fetal stroke and cerebrovascular disease: Advances in understanding from lenticulostriate and venous imaging,alloimmune thrombocytopaenia and monochorionic twins |
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Authors: | Fenella J. Kirkham Dimitrios Zafeiriou David Howe Philippa Czarpran Ashley Harris Roxanna Gunny Brigitte Vollmer |
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Affiliation: | 1. Developmental Neurosciences Section and Biomedical Research Centre, UCL Great Ormond Street Institute of Child Health, London, United Kingdom;2. Departments of Child Health, Obstetrics and Gynaecology and Radiology, University Hospital Southampton, United Kingdom;3. Clinical and Experimental Sciences, University of Southampton, United Kingdom;4. 1st Department of Pediatrics, “Hippokratio’ General Hospital, Aristotle University, Thessaloniki, Greece;5. Department of Radiology, St George''s hospital, London, United Kingdom |
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Abstract: | Fetal stroke is an important cause of cerebral palsy but is difficult to diagnose unless imaging is undertaken in pregnancies at risk because of known maternal or fetal disorders. Fetal ultrasound or magnetic resonance imaging may show haemorrhage or ischaemic lesions including multicystic encephalomalacia and focal porencephaly. Serial imaging has shown the development of malformations including schizencephaly and polymicrogyra after ischaemic and haemorrhagic stroke. Recognised causes of haemorrhagic fetal stroke include alloimmune and autoimmune thrombocytopaenia, maternal and fetal clotting disorders and trauma but these are relatively rare. It is likely that a significant proportion of periventricular and intraventricular haemorrhages are of venous origin. Recent evidence highlights the importance of arterial endothelial dysfunction, rather than thrombocytopaenia, in the intraparenchymal haemorrhage of alloimmune thrombocytopaenia. In the context of placental anastomoses, monochorionic diamniotic twins are at risk of twin twin transfusion syndrome (TTTS), or partial forms including Twin Oligohydramnios Polyhydramnios Sequence (TOPS), differences in estimated weight (selective Intrauterine growth Retardation; sIUGR), or in fetal haemoglobin (Twin Anaemia Polycythaemia Sequence; TAPS). There is a very wide range of ischaemic and haemorrhagic injury in a focal as well as a global distribution. Acute twin twin transfusion may account for intraventricular haemorrhage in recipients and periventricular leukomalacia in donors but there are additional risk factors for focal embolism and cerebrovascular disease. The recipient has circulatory overload, with effects on systemic and pulmonary circulations which probably lead to systemic and pulmonary hypertension and even right ventricular outflow tract obstruction as well as the polycythaemia which is a risk factor for thrombosis and vasculopathy. The donor is hypovolaemic and has a reticulocytosis in response to the anaemia while maternal hypertension and diabetes may influence stroke risk. Understanding of the mechanisms, including the role of vasculopathy, in well studied conditions such as alloimmune thrombocytopaenia and monochorionic diamniotic twinning may lead to reduction of the burden of antenatally sustained cerebral palsy. |
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Keywords: | Fetus Stroke Focal ischemia Intraparenchymal hemorrhage Posterior fossa hemorrhage Subarachnoid hemorrhage Periventricular hemorrhage Intraventricular hemorrhage Periventricular leukomalacia Encephalomalcia Porencephaly Polymicrogyria Schizencephaly Hydrancephaly Focal cortical dysplasia Vascular disruption Monochorionic diamniotic twins Parabiotic Placental anastomoses Twin twin transfusion syndrome Twin oligohydramnios polyhydramnios syndrome Selective intrauterine growth retardation Twin anemia polycythemia syndrome Twin reverse arterial perfusion TTTS TOPS sIUGR TAPS TRAP Fetal and neonatal alloimmune thrombocytopenia FNAIT Endothelial dysfunction Venous sinus thrombosis Dural venous ectasia with thrombosis Fetal periventricular venous infarction Lenticulostriate vasculopathy Middle cerebral artery peak systolic velocity Vein of Galen malformation Hypovolemia Anemia Polycythemia Embolus Pulmonary hypertension Right ventricular outflow tract obstruction Cytomegalovirus Zika Parvovirus B19 Thermolabile methylene tetrahydrofolate reductase polymorphism Factor V leiden Prothrombotic COL4A1 COL4A2 Trauma Maternal warfarin Maternal cocaine |
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