ARTERIAL PORENCEPHALY - keywords
arterial porencephaly
references to arterial porencephaly
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Abergel A, Lacalm A, Massoud M, Massardier J, des Portes V, Guibaud L (2017) Expanding Porencephalic Cysts: Prenatal Imaging and Differential Diagnosis. Fetal Diagn Ther, 41(3):226-233. doi: 10.1159/000447740.
Bordarier C, Robain O, Ponsot G. Bilateral porencephalic defect in a newborn after injection of benzol during pregnancy. Brain Dev 1991;13:126–129.
Cassart M, Bosson N, Garel C, Eurin D, Avni F (2008) Fetal intracranial tumors: a review of 27 cases. Eur Radiol, 18(10):2060-2066. doi: 10.1007/s00330-008-0999-5.
Clark R, Linell E. Case report: prenatal occlusion of the internal carotid artery. J Neurol Neurosurg Psych 1954;17:295.
Debus O, Koch HG, Kurlemann G, Sträter R, Vielhaber H, Weber P, Nowak-Göttl U. Factor V Leiden and genetic defects of thrombophilia in childhood porencephaly. Arch Dis Child Fet Neon Ed 1998;78:F121-F124.
Deasy NP, Jarosz JM et al. Congenital varicella syndrome: cranial MRI in a long-term survivor. Neuroradiol 1999;41:205-207.
Dominguez R, Villa-Coro AA, Slopis JM et al. Brain and ocular abnormalities in infants with in utero exposure to cocaine and other street drugs. Am J Dis Child 1991;145:688-695.
Govaert P. (2009) Prenatal stroke. Semin Fetal Neonatal Med 14:250-66.
Guzzetta F, Battaglia D, Di Rocco C, Caldarelli M. Symptomatic epilepsy in children with porencephalic cysts secondary to perinatal middle cerebral artery occlusion. Child’s Nerv Sys 2006;22:922-930.
Jung JH, Graham JM. Congenital hydranencephaly/porencephaly due to vascular disruption in monozygotic twins. Pediatrics 1984;73:467-469.
Larroche JC, Amiel C. Thrombose de l’artère Sylvienne à la période néonatale. Arch Franç Pédiatr 1966;23:257-264.
Lynch JK, Han CJ, Nee LE, Nelson KB. Prothrombotic factors in children with stroke or porencephaly. Pediatrics 2005;116(2):447-453.
Mittelbronn M, Beschorner R, Schittenhelm J, Capper D, Goeppert B, Meyermann R, Meyer-Wittkopf M, Mackensen-Haen S. Multiple thromboembolic events in fetofetal transfusion syndrome in triplets contributing to the understanding of pathogenesis of hydranencephaly in combinatin with polymicrogyria. Hum Pathol 2006;37:1503-1507.
Nixon GW, Johns RE, Myers GG. Congenital porencephaly. Pediatrics 1974;54:43-50.
Patten RM, Mack LA et al. Twin embolization syndrome: prenatal sonographic detection and significance. Radiology 1989;173:685-689.
Rolland M, Sevely A, Pradere M, Manelfe C, Regnier C. Occlusions artérielles cérébrales antenatales. Pédiatrie 1983;38:151-160.
Takada K, Shiota M, Ando M, Kimura M, Inoue K. Porencephaly and hydranencephaly: a neuropathological study of four autopsy cases. Brain Dev 1989;11:51-56.
Tardieu M, Evrard P, Lyon G. Progressive expanding congenital porencephalies: a treatable cause of progressive encephalopathy. Pediatrics 1981;68:198–202.
Tomas-Vila M, Garcia-Tamarit P, Garcia-Colino A, Torregrosa-Pascual P, Martinez-Salinas P. Schizencephaly associated with porencephaly in a girl with congenital cytomegalovirus infection. Rev Neurol 2000;31(10):952-5.
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typical images
Diverging descriptions of ‘porencephaly’ exist. Literally one would want to observe a cavity in brain substance with a connection ((porus) linking it to the lateral ventricle and/or the brain surface. Cavities without pore should be referred to as cysts.
Two major entities can be distinguished, depending on time of insult: (i) schizencephaly, a transmantle defect lined by heterotopic neurons because of onset before the end of neuronal migration; (ii) perinatal (clastic) porencephaly (> 24 w of gestation). Clear large artery infarcts of onset before 25 weeks of gestation may be bordered by polymicrogyria, when one enters a semantic discussion whether they are arterial porencephaly or schizencephaly (Takada et al. 1989).
When an insult occurred weeks before delivery, at any PMA an antenatal porencephalic defect can be detected with ultrasound on admission to the unit (surpriose finding). In presumed perinatal arterial ischaemic stroke, the porencephalic defect is recognized following clinical presentation with seizures or a hemisyndrome weeks to months after birth. In both the arterial template should be recognisable to refer to it as an old arterial infarct.
Epilepsy following perinatal porencephaly may become refractory to anti-epileptic drugs and surgical options, a.o. excision of the cortical areas forming the cyst “membrane”, should then be explored (Guzzetta et al. 2006).
neuropathology
presumed perinatal
Mechanisms and risk factors for intrauterine stroke (both for porencephaly and hydranencephaly) include:
- monozygous twinning (Rolland et al. 1983, Clark and Linell 1954, Jung and Graham 1984, Patten et al. 1989, Mittelbronn et al. 2006)
- maternal cardiac arrest or abdominal trauma
- cocaine abuse by the mother (Dominguez et al. 1991)
- injection of benzol to induce abortion (Bordarier et al. 1991)
- deficient protein C anticoagulant pathway (Debus et al. 1998, Lynch et al. 2005) and other prothrombotic conditions like factor II mutations and elevated lipoprotein A levels (Lynch et al. 2005)
- CMV fetopathy (Tomas-Vila et al. 2000)
- varicella fetopathy (Deasy et al 1999).
The prognosis of porencephaly depends on the size and location, as well as on other associated abnormalities.
arterial porencephaly
late neonatal death after MCA stroke: multiple cavities, not in contact with the lateral ventricle but reaching into the cortical areas
Friede 1989
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tissue loss——>
transmantle
Growth retarded preterm infant, gestational age 31 weeks: 7.5 MHz sections showing paraventricular arterial pial type of porencephaly; the image bottom left is parasagittal, the others coronal; the top image was taken on the first day of life, the others on day 53. In the initial pictures a cavity is seen with tissue remnants hanging on its outer wall; later on there is just a cavity, joined by an adjacent superficial arachnoid cyst where the cortex seems to be absent. This girl is now > 20 years old, has left-hand preference as the only sequel of this lesion.
Preterm infant with apnoea: coronal 7.5 MHz section illustrating a partial ‘basket brain’; the left ACA is still perfused and maintains a bridge of tissue around the remnants of a complete infarction in the left MCA; on the right there has been necrosis of the areas perfused by the ICA. The presumed cause of this event was the combination of maternal smoking and anticonceptive oestrogen use.
cerebral hemiatrophy: characteristic imaging
presumed perinatal arterial porencephaly
MRI at 7 months in hemiplegic infant with late discovery of perinatal MCA stroke (presumed perinatal arterial ischaemic stroke) in the presentation of arterial type of porencephaly
unclear whether this association is causal or incidental
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The transmantle variant of porencephaly that follows fetal arterial ischaemic stroke is described here. Clastic lesions form multiple or isolated cavities, some with irregular walls on account of the more mature late fetal character of tissue reaction. Borders of perinatal pial porencephalies are without polymicrogyria.
Most porencephalies follow prenatal periventicular venous white matter infarction and leave the cortical mantle intact, although extensive venous infarction may provoke ipsilateral arterial hypoperfusion that secondarily destroys overlying cortex.
Following occlusion of one of the major pial cerebral arteries or a large cortical branch, a cavity is formed after a few weeks, not always contacting the lateral ventricle but always opening into the arachnoid space because the (sub)cortex is destroyed by the injury. Parenchymal remnants stay visible in the cavity in the subacute stage.
Some arterial pial porencephalies communicate with the adjacent lateral ventricle and therefore become susceptible to distension following Laplace’s law, producing pressure porencephaly, documented with pneumencephalography (Nixon et al. 1974) and CT (Tardieu et al. 1981).
Early postmortem descriptions documented recanalization of affected arteries, explaining that occlusion of the involved artery is not permanent in most instances (Larroche and Amiel 1966). Often this cavity is found in the region of the MCA, but any major artery can be involved (Rolland et al. 1983). Similar to acute perinatal arterial ischaemic stroke the left side is most affected (Debus et al. 1989). In case of bilateral prenatal MCA infarction, the hippocampus, basal parts of the temporal lobes, occipital lobes and orbitofrontral cortex are maintained: this is referred to as a ‘basket brain’ because the residual mesial structures within ACA and PCA territory constitute a kind of handle around absent tissue. Given the ischaemic nature of any of these lesions it is possible to observe a combination of preterm white matter injury (leukomalacia) with stroke.
term infant succumbed in early infancy to brainstem haematoma following difficult delivery; associated richt partial posterior truncal MCA stroke in the end stage of porencephaly connected to the brain surface
arterial porencephaly: neuropathology
Longstanding neocortical defects of intrauterine origin affect distant neuronal structures like the nucleus of Meynert and the dentate nuclei (Takada et al. 1989).
Some of these lesions may partially destroy the corticospinal tract. Porencephalic cysts can even expand into the pericerebral CSF spaces (Abergel et al. 2017). Porencephalic cysts thus have to be differentiated from arachnoid cysts and rare tumors with mixed fluid and solid components. Arachnoid cysts are crisply demarcated extracerebral cystic abnormalities, which if large can cause mass effect. When this occurs, given that the arachnoid cyst is completely extra-axial, an intact cortical ribbon and underlying white matter can be identified in the brain parenchyma displaced or compressed by it. Tumors with a cystic component include teratoma and pilocytic astrocytoma (Cassart et al. 2008).
Although rare, focal arterial infarction of cerebellum exists and may occur in utero. In such instance the tissue defect will not be reported as porencephaly, but often as cerebellar dysplasia.
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