Pediatric cerebrovascular disease epidemiology

Pediatric cerebrovascular disease epidemiology

The incidence of pediatric stroke is 1 in 5000, and if hemiplegic cerebral palsy due to vaso-occlusive stroke is included, the number could be as high as 1 in 3000. Additionally, cerebrovascular disease is 1 of the top 10 causes of death in infants younger than 1 year. Finally, 20% to 30% of children with arterial ischemic stroke will have recurrent strokes, even with treatment. Stroke in children differs from stroke in adults. Not only is it rare, but its presentation is subtle—particularly in infants—and even with a focal hemiplegia there is a wide differential diagnosis. Coagulation mechanisms, the arteries, and the neurological systems are all different in children, and each of these plays a large role in stroke. The causes of pediatric stroke do not include atherosclerosis, so a myriad of other risk factors and associations exist and are unique for each age group. The causes of pediatric stroke are poorly understood, and although this is a fertile area of research, clinical trials in the field are lacking. Currently, any treatment guidelines or tools being used to treat children with stroke either come from the field of adult stroke or are based on empirical information.

More than 95% of children with ischemic stroke have an underlying thrombus occluding an artery or a vein, and our understanding of clot pathogenesis in children is increasing. Whereas in adults, platelet clots predominantly form secondary to atherosclerosis, in children and infants there is likely a higher fibrin composition, which may require a different treatment strategy. Although anticoagulation is typically used, it is not known whether anticoagulation is more effective than aspirin. There are also major clinical challenges, the most significant of which is that the diagnosis is not made and the stroke is missed entirely or that the diagnosis is severely delayed and by the time the diagnosis is made, the infarct is much larger 1).


In 1978 A 10-year review of the Mayo Clinic experience with childhood cerebrovascular disease unrelated to birth, intracranial infection, or trauma identified 69 patients (38 with ischemic stroke, and 31 with subarachnoid or intracerebral hemorrhage). Although children with cerebral infarction had better survival, they experienced more residual disability than children with cerebral hemorrhage. The medical records-linkage system for Rochester, Minnesota residents made it possible for the first time to study cerebrovascular disease in a well-defined childhood population. Records from all medical facilities serving this population (average of 15,834 resident children) showed four strokes over 10 years (average annual incidence rate of 2.52 cases per 100,000 per year) 2).


In 2018 a study reported the period prevalence, incidence, and risk factors of pediatric stroke in Taiwan.

All Taiwan inhabitants aged 1 month to 18 years registered in the National Health Insurance Research Database between 2010 and 2011 were enrolled in this study. Factors including age, sex, location, and household income levels were collected. Incidence, period prevalence, mortality rate, and the possible risks were completely evaluated. Outcomes and results: Hemorrhagic stroke has a significantly higher mortality rate than ischemic stroke (27.6% vs. 10.2%, P<0.05). Risk factors or underlying diseases for stroke were identified in 77.8% of the patients and 16.2% had more than one risk factor. The most common risk factors were vascular diseases (26.3%), infection (14.0%), and cardiac disorders (9.1%).

Infants younger than 2 years, boys, and children in lower socioeconomic status have a significantly higher risk of stroke. Hemorrhagic stroke has a significantly higher mortality rate than ischemic stroke. More than half of the children with stroke had underlying diseases and the causes of hemorrhagic stroke are significantly different from ischemic stroke 3).


In 2019 Surmava et al. sought to evaluate in -Ontario, the incidence and characteristics of pediatric stroke and TIA including care gaps and the predictive value of International Classification of Diseases (ICD) codes.

A retrospective chart review was conducted at 147 Ontario pediatric and adult acute care hospitals. Pediatric stroke and TIA cases (age < 18 years) were identified using ICD-10 code searches in the 2010/11 Canadian Institute for Health Information’s Discharge Abstract Database (CIHI-DAD) and National Ambulatory Care Reporting System (NACRS) databases in the Ontario Stroke Audit.

Among 478 potential pediatric strokes and TIA cases identified in the CIHI-DAD and NACRS databases, 163 were confirmed as cases of stroke and TIA during the 1-year study period. The Ontario stroke and TIA incidence rate was 5.9 per 100,000 children (3.3 ischemic, 1.8 hemorrhagic, and 0.8 TIA). The mean age was 6.4 years (16% neonate). Nearly half were not imaged within 24 h of arrival in emergency and only 56% were given antithrombotic treatment. At discharge, 83 out of 121 (69%) required health care services post-discharge. Overall positive predictive value (PPV) of ICD-10 stroke and TIA codes was 31% (range 5-74%) and yield ranged from 2.4 to 29% for acute stroke or TIA event; code I63 achieved maximal PPV and yield.

This population-based study yielded a higher incidence rate than prior North-American studies. Important care gaps exist including delayed diagnosis, lack of expert care, and departure from published treatment guidelines. Variability in ICD PPV and yield underlines the need for prospective data collection and for improving the pediatric stroke and TIA coding processes 4).


It is believed that the incidence in the Hospital Universitario “Dr. Jose Eleuterio Gonzalez,” Universidad Autonoma de Nuevo Leon, Monterrey, Nuevo Leon, Mexico is higher than it appears.

A study by Garza-Alatorre et al. aimed to assess the incidence and characteristics of pediatric stroke in this university hospital. Likewise, this study seeks to evaluate if a longer symptoms-to-diagnosis time is associated with mortality in patients with ischemic stroke.

Methods: A retrospective study including children with stroke admitted to the UANL University Hospital from January 2013 to December 2016.

Results: A total of 41 patients and 46 stroke episodes were admitted. About 45.7% had an ischemic stroke and 54.3% had a hemorrhagic stroke. Mortality of 24.4% and morbidity of 60.9% were recorded. Regarding ischemic and hemorrhagic stroke, and increased symptoms-to-diagnosis time and a higher mortality were obtained with a relative risk of 2.667 (95% confidence interval [CI]: 1.09-6.524, p = 0.013) and 8.0 (95% CI: 2.18-29.24, p = < 0.0001), respectively. A continuous increase in the incidence rate, ranging from 4.57 to 13.21 per 1,000 admissions comparing the first period (2013) versus the last period (2016), p = 0.02, was found in our center.

Pediatric stroke is a rare disease; however, its incidence shows a continuous increase. More awareness toward pediatric stroke is needed 5).


1)

Bowers KJ, Deveber GA, Ferriero DM, Roach ES, Vexler ZS, Maria BL. Cerebrovascular disease in children: recent advances in diagnosis and management. J Child Neurol. 2011 Sep;26(9):1074-100. doi: 10.1177/0883073811413585. Epub 2011 Jul 21. PMID: 21778188; PMCID: PMC5289387.
2)

Schoenberg BS, Mellinger JF, Schoenberg DG. Cerebrovascular disease in infants and children: a study of incidence, clinical features, and survival. Neurology. 1978 Aug;28(8):763-8. doi: 10.1212/wnl.28.8.763. PMID: 567292.
3)

Chiang KL, Cheng CY. Epidemiology, risk factors and characteristics of pediatric stroke: a nationwide population-based study. QJM. 2018 Jul 1;111(7):445-454. doi: 10.1093/qjmed/hcy066. PMID: 29648667.
4)

Surmava AM, Maclagan LC, Khan F, Kapral MK, Hall RE, deVeber G. Incidence and Current Treatment Gaps in Pediatric Stroke and TIA: An Ontario-Wide Population-Based Study. Neuroepidemiology. 2019;52(3-4):119-127. doi: 10.1159/000493140. Epub 2019 Jan 17. PMID: 30654369.
5)

Garza-Alatorre G, Carrion-Garcia AL, Falcon-Delgado A, Garza-Davila EC, Martinez-Ponce de Leon AR, Botello-Hernandez E. Characteristics of Pediatric Stroke and Association of Delayed Diagnosis with Mortality in a Mexican Tertiary Care Hospital. Neuropediatrics. 2021 Jul 14. doi: 10.1055/s-0041-1731802. Epub ahead of print. PMID: 34261144.

Double origin of the posterior inferior cerebellar artery

Double origin of the posterior inferior cerebellar artery

The posterior inferior cerebellar artery (PICA) frequently arises from the fenestrated segment of the intracranial vertebral artery (VA), and this common variation can be misinterpreted as or confused with a PICA of double origin. Rarely, a PICA of true double origin occurs when two branches of the PICA arise separately from the intracranial VA and fuse to form an arterial ring.

Uchino et al. discovered this rare variation incidentally while interpreting images of magnetic resonance angiography. This is the first report of MR angiographic findings of this rare variation 1).


For Lesley et al. double origin of the PICA is seen in 4.1% of patients with intracranial aneurysms and on 1.45% of catheter angiograms. The double origin of the PICA has an increased association with intracranial aneurysmal disease and may represent a risk factor for subsequent development of an intracranial aneurysm 2).


Posterior fossa arteriovenous malformations (AVMs), especially cerebellar arteriovenous malformations, are also not common. Consequently, the association of a Double origin of the posterior inferior cerebellar artery (DOPICA) with a cerebellar AVM is even rare.

Rodriguez-Calienes et al. presented a rare case of a pediatric cerebellar AVM supplied by a branch of a DOPICA which was treated endovascularly with NBCA. Total obliteration was achieved in the immediate controls and at 1-year follow-up.

Navigation through tortuous and long branches from a DOPICA is technically feasible. Although NBCA cure rates are relatively low, when the microcatheter can no longer navigate through the feeding artery, a correct dilution of NBCA with lipiodol can provide adequate penetration of this embolic agent, to obliterate the AVM nidus completely 3).


A cadaveric specimen was prepared for dissection. A far lateral craniotomy was performed on the right side. While exploring the right cerebellomedullary cistern, two separate origins of PICA were found from the vertebral artery (VA) as the caudal and rostral trunks that joined to form the distal PICA trunk at the tonsillomedullary segment. Microscopic and endoscopic illustrations are provided.

To the best of the authors’ knowledge, this is the first anatomic report on the DOPICA. Cadaveric illustration of this variant helps with understanding its anatomical relationship with adjacent neurovascular structures of the cerebellomedullary cistern including the perforating arteries and the lower cranial nerves 4).


Cho et al. demonstrated the first case of double origin of the posterior inferior cerebellar artery (PICA) with juxta-proximal fenestration of the caudal component, which was misunderstood as triple origin, combined with an unruptured middle cerebral artery aneurysm. The caudal component of the PICA originated from the atlanto-occipital portion and it was fenestrated shortly after origin. The cranial component originated from the intracranial vertebral artery and converged with the superior branch of the caudal component, and then met the inferior branch of the caudal component distally 5).


Two cases of anatomical variation of the PICA that they have called its double origin, one of which gave rise to an aneurysm. The first patient was a 36-year-old man who presented with a subarachnoid hemorrhage related to the rupture of a PICA aneurysm. The aneurysm was treated by the endovascular route. Selective and super selective studies showed that the PICA origin was low on the fourth segment of the vertebral artery (VA). The aneurysm was located on an anastomosis between the PICA and a small upper arterial branch originating from the VA. Embolization was performed through the small branch with no problem, but a lateral medullary infarct followed, probably due to occlusion of the perforating vessels. The same anatomical variation was incidentally discovered in the second patient. To the authors’ knowledge, neither this anatomical variation of the PICA nor the aneurysm’s topography has been previously described angiographically. This highlights the role of angiography in pretreatment evaluation of aneurysms especially when perforating vessels or small accessory branches that are poorly visualized on angiographic studies are concerned, as in the territory of the PICA. Anatomy is sometimes unpredictable, and the surgeon must be very careful when confronted with these variations because they are potentially dangerous for endovascular treatment 6).


1)

Uchino A, Saito N, Ishihara S. Double Origin of the Posterior Inferior Cerebellar Artery Diagnosed by MR Angiography: A Report of Two Cases. Neuroradiol J. 2015 Apr;28(2):187-9. doi: 10.1177/1971400915576659. Epub 2015 Apr 13. PMID: 25923681; PMCID: PMC4757150.
2)

Lesley WS, Rajab MH, Case RS. Double origin of the posterior inferior cerebellar artery: association with intracranial aneurysm on catheter angiography. AJR Am J Roentgenol. 2007 Oct;189(4):893-7. doi: 10.2214/AJR.07.2453. PMID: 17885063.
3)

Rodriguez-Calienes A, Saal-Zapata G, De la Cruz J. Endovascular Treatment of an Arteriovenous Malformation Associated with a Double Origin of the Posterior Inferior Cerebellar Artery. Pediatr Neurosurg. 2021 Jul 8:1-5. doi: 10.1159/000517248. Epub ahead of print. PMID: 34237747.
4)

Meybodi AT, Moreira LB, Zhao X, Lawton MT, Preul MC. Double Origin of the Posterior Inferior Cerebellar Artery: Anatomic Case Report. World Neurosurg. 2019 Jan 3:S1878-8750(18)32950-4. doi: 10.1016/j.wneu.2018.12.127. Epub ahead of print. PMID: 30611945.
5)

Cho YD, Han MH, Lee JY. Double origin of the posterior inferior cerebellar artery with juxta-proximal fenestration of caudal component. Surg Radiol Anat. 2011 Apr;33(3):271-3. doi: 10.1007/s00276-010-0747-9. Epub 2010 Nov 24. PMID: 21107570.
6)

Pasco A, Thouveny F, Papon X, Tanguy JY, Mercier P, Caron-Poitreau C, Herbreteau D. Ruptured aneurysm on a double origin of the posterior inferior cerebellar artery: a pathological entity in an anatomical variation. Report of two cases and review of the literature. J Neurosurg. 2002 Jan;96(1):127-31. doi: 10.3171/jns.2002.96.1.0127. PMID: 11794593.

Chronic Cerebral Ischemia Treatment

Chronic Cerebral Ischemia Treatment

see also Acute Ischemic Stroke Treatment.


Carotid artery stenting

Revascularization for Chronic Cerebral Ischemia Treatment.

The role of cellular transplantation to promote functional recovery after stroke has been evaluated over the last two decades. Preclinical studies first established the potential for cultured neuronal cells derived from a teratocarcinoma cell line to be tested for safety and efficacy in the treatment of human stroke. In animal models of stroke that caused reproducible learning and motor deficits, injection of neuronal cells resulted in a return of learning behavior, retention time, and motor function. Clinical trials followed. Additional work with cells derived from a bone marrow neuroprogenitor line, fetal cortical stem cells, and other cell sources showed promise in preclinical studies and then these cells were tested in clinical studies 1).

prospective randomized trial (NCT03745092) enrolled 50 cases of CCI patients, which were divided into NBO (8 L/min of oxygen supplement) group and control group (room air) randomly, and also enrolled 21 healthy volunteers. Two times of 30-min EEG recordings with the interval of 45min of NBO or room air were analyzed quantitatively.

The CCI-mediated EEG presented with two patterns of electrical activities: high-power oscillations (high-power EEG, n = 26) and paroxysmal slow activities under the normal-power background (normal-power EEG, n = 24). The fronto-central absolute power (AP) of the beta, alpha, theta, and delta in the high-power EEG was higher than that in healthy EEG (p < 0.05). The fronto-central theta/alpha, delta/alpha and (delta + theta)/(alpha + beta) ratios in the normal-power EEG were higher than those in healthy EEG (p < 0.05). The high-power EEG in NBO group had higher fronto-central AP reduction rates than those in control group (p < 0.05). NBO remarkably reduced the fronto-central theta/alpha, delta/alpha, and (delta + theta)/(alpha + beta) ratios in the normal-power EEG (p < 0.05).

NBO rapidly ameliorates CCI-mediated EEG anomalies, including attenuation of the abnormal high-power oscillations and the paroxysmal slow activities associated with CCI 2).


1)

Kondziolka D. Stem Cell Treatment for Ischemic Stroke Recovery. Semin Neurol. 2021 Jan 27. doi: 10.1055/s-0040-1722640. Epub ahead of print. PMID: 33506475.
2)

Ding JY, Liu Y, Rajah GB, Chen ZY, Zhang SY, Ding YC, Ji XM, Meng R. Normobaric oxygen may correct chronic cerebral ischemia-mediated EEG anomalies. CNS Neurosci Ther. 2021 Jul 9. doi: 10.1111/cns.13703. Epub ahead of print. PMID: 34242498.
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