Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins

doi:10.1016/0887-8994(95)00219-7

Pediatric Neurology

Volume 13, Issue 4, November 1995, Pages 279-285

https://doi.org/10.1016/0887-8994(95)00219-7 Get rights and content

Abstract

The prenatal histories, clinical courses, and neuroradiographic studies of 8 infants who had survived the in utero demise of a homozygous co-twin were reviewed. Three distinct modes of clinical presentation were found: (1) severe neonatal encephalopathy with seizures; (2) a more benign neonatal course with onset of seizures and profound developmental disabilities within the first 6 months of age; (3) late infantile presentation with seizures. Only the third group had milder outcomes. Neuroradiographic studies demonstrated two pathologic patterns: varying degrees of periventricular white matter infarction with migrational abnormalities observed with earlier demise of the co-twin, and multicystic encephalomalacia observed when the demise occurred at or near term. Pathophysiology is uncertain and most likely multifactorial. Exsanguination injury to the survivor can occur acutely following co-twin demise, so urgent delivery may be appropriate at or near term.

References (23)

C.M. Moore et al.
Intrauterine disseminated intravascular coagulation: A syndrome of multiple pregnancy with a dead fetus
J Pediatr
(1969)
J. Aicardi et al.
Multicystic encephalomalacia of infants and its relation to abnormal gestation and hydranencephaly
J Neurol Sci
(1972)
M. Melnick
Brain damage in survivor after in-utero demise of monozygous twin (letter)
Lancet
(1977)
H. Yoshioka et al.
Multicystic encephalomalacia in liveborn twin with a stillborn macerated co-twin
J Pediatr
(1979)
D.S. Wagner et al.
Placental emboli from a fetus papyraceous
J Pediatr Surg
(1990)
H.J. Landy et al.
The “vanishing twin”: Ultrasonographic assessment of fetal disappearance in the first trimester
Am J Obstet Gynecol
(1986)
R. Bejar et al.
Antenatal origin of neurologic damage in newborn infants: II. Multiple gestations
Am J Obstet Gynecol
(1990)
R. Gonen
The origin of brain lesions in survivors of twin gestations complicated by fetal death (letter)
Am J Obstet Gynecol
(1991)
L.E. Reisman et al.
Bilateral renal cortical necrosis in the newborn
Am J Dis Child
(1996)
H.E. Hoyme et al.
Vascular etiology of disruptive structural defects in monozygotic twins
Pediatrics
(1981)

J.H. Jung et al.

Congenital hydranencephaly/porencephaly due to vascular disruption in monozygotic twins

Pediatrics

(1984)

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Citation Excerpt :
It was therefore hypothesised that the acute fall in blood pressure in the dying twin allows shunting of blood from the survivor, causing acute hypovolaemia, hypotension, hypoxia, acidosis and anaemia in that twin. Anaemia has been reported in the surviving twin at birth113,129,138 and the acute blood loss has been considered to be the main cause of perinatal death129 and/or brain damage.113,168,169 There is some evidence for acute anaemia in the surviving twin on fetal blood sampling, when the haematocrit may halve acutely from 35 to 40% to less than 20 in the surviving twin.168
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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Perinatal stroke has become increasingly recognized with the increased application of neuroimaging for neonatal seizures and encephalopathy. The frequency of perinatal stroke is estimated at 1 in 5000 births. Perinatal arterial ischemic stroke (AIS) accounts for 80% of neonatal stroke, while cerebral sinovenous thrombosis (CSVT) accounts for 20%. This chapter will summarize the neuropathology, pathophysiology, clinical presentation, management, and prognosis of perinatal AIS and CSVT. These conditions differ in their risk factors, presentation, management, and prognosis emphasizing the importance of their accurate diagnosis in the newborn infant.
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Widespread selective neuronal cell death (in deep grey matter, brainstem and cord) may coexist on the basis of global forebrain ischaemia.59,60 Monozygous twinning predisposes to this type of injury,55,59,61 often afflicting the donor (Figs. 1 and 7). A similar global insult occurs in some types of birth asphyxia.62
The main focus of this chapter is the comprehensive description of the neuropathology, the imaging correlates and underlying mechanisms of prenatal stroke. We describe established prenatal stroke in subgroups similar to postnatal stroke: arterial (forebrain or hindbrain) infarction, venous thrombosis, primary lobar haemorrhage. This longitudinal classification should facilitate the study of risk factors and mechanisms. Forebrain lesions of arterial type present as porencephaly, (hemi)hydranencephaly, multicystic encephalopathy or schizencephaly. Venous prenatal forebrain stroke presents as simple porencephaly (in some of genetic nature) and sinus thrombosis. A list of rare porencephaly-like conditions is added for differentiation from arterial and venous porencephaly. Hindbrain infarctions (so far the only reported variants seem to be of arterial nature) present as brainstem disconnection, focal brainstem destruction, uni- or bilateral cerebellar destruction and focal spinal cord ischaemia. Prenatal intracranial haemorrhage and congenital brain infection should be considered in the differential diagnosis of prenatal stroke.
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Citation Excerpt :
Monochorionic twins appear to be primarily at risk.20,39,44,46-54 At all gestational ages, the risk of cerebral palsy is higher in like-sex surviving cotwins.55 Abnormalities in surviving cotwins are usually attributed to the complex vascular anastomoses that occur in monochorionic gestations.
Any discussion of multiple pregnancy figures prominently in the consideration of the medicolegal aspects of placental pathology. Multiple gestations are common and becoming more so with assisted reproductive techniques, and multiples are associated with a disproportionate share of complications that may result in disputes over quality of care. Higher rates of intrauterine growth retardation, prematurity, stillbirth, morbidity, mortality, cerebral palsy, anomalous development, and malformation as compared with singletons are well documented in multiple pregnancy and should be anticipated. Monochorionic placentation and complications of vascular anastomosis are important factors contributing to poor outcome. Other factors, although occurring in all gestations, are relevant because they are more common in multiple gestations.
Risk of Cerebral Palsy in Multiple Pregnancies
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Perinatal and neonatal ischaemic stroke: A review
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Original articlePatterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins

Abstract

J Pediatr

J Neurol Sci

Lancet

J Pediatr

J Pediatr Surg

Am J Obstet Gynecol

Am J Obstet Gynecol

Am J Obstet Gynecol

Bilateral renal cortical necrosis in the newborn

Am J Dis Child

Vascular etiology of disruptive structural defects in monozygotic twins

Pediatrics

Congenital hydranencephaly/porencephaly due to vascular disruption in monozygotic twins

Pediatrics

Original article
Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins