Cerebrovascular Disease in Children PDF

**OUTLINE** I. **Introduction** II. **Stroke Classification** a. Ischemic Stroke b. Hemorrhagic Stroke III. **Causes of Stroke in Children** a. Cardiac Condition b. Common Vasculopathies c. Hypercoagulable States d. CNS Infections IV. **Stroke** a. Idiopathic Stroke b. Signs and...

**OUTLINE** I. **Introduction** II. **Stroke Classification** a. Ischemic Stroke b. Hemorrhagic Stroke III. **Causes of Stroke in Children** a. Cardiac Condition b. Common Vasculopathies c. Hypercoagulable States d. CNS Infections IV. **Stroke** a. Idiopathic Stroke b. Signs and Symptoms c. Differential Diagnosis d. History e. Diagnostic Evaluation V. **Management** a. Medical management b. Surgical management c. Supportive management d. Recommendations for rehabilitation VI. **Outcomes** a. Neurologic Sequelae b. Stroke Recurrence VII. **Cerebral Sinovenous Thrombosis (CSVT)** a. Neuroimaging VIII. **Hemorrhagic Stroke** a. Spontaneous childhood hemorrhagic stroke (SICH) IX. **Aneurysm** a. Types b. Aneurysmal Hemorrhagic Stroke X. **Summary** XI. **Case Discussion (Plenary)** +-----------------------+-----------------------+-----------------------+ | **LEGEND** | | | +=======================+=======================+=======================+ | ⭐ | 🖊️ | 📖 | | | | | | Must | Lecture | Book | | | | | | Know | *\[lec\]* | *\[bk\]* | +-----------------------+-----------------------+-----------------------+ INTRODUCTION {#introduction.TransOutline} ============ Stroke occurs in children 2-8 / 100,000 children per year Comparable to pediatric brain tumors Significance Stroke is an important cause of mortality & morbidity in children CHILDREN ARE NOT LITTLE ADULTS Little folks have different strokes Brain of children aren't fully developed ∴ it can still adapt to counter the deficits Outcomes better than adults Less comorbidites compared to adults TERMINILOGY {#terminilogy.TransSub-subtopic2} ----------- Stroke in the young: Stroke in \< 45y/o Childhood stoke: Stroke in perinatal, neonatal, infants, and older children STROKE CLASSIFICATION {#stroke-classification.TransOutline} ===================== A. ISCHEMIC STROKE {#a.-ischemic-stroke.TransSubtopic1} ------------------ - Occlusion of blood flow to an area of the brain - There is a clot in the blood vessel occluding the blood supply to the brain - Specific risk factors sometimes suggest whether stroke has resulted from an embolus or thrombus - Cardiac diseases: Embolism \> thrombosis - Cerebral arteritis: \> Thrombosis - The anterior circulation is often more affected than the vertebrobasilar system - ⭐️ Basal ganglia & thalamus: Most frequent location for ischemic infarction (children) #### Figure 1. Ischemic stroke {#figure-1.-ischemic-stroke.TransSub-subtopic3} B. HEMORRHAGIC STROKE {#b.-hemorrhagic-stroke.TransSubtopic1} --------------------- Rupture of blood vessels with blood leaking to the brain tissue Structural vascular anomalies collectively constitute the largest cause of non-traumatic intraparenchymal & subarachnoid hemorrhage in children **Figure 2. Hemorrhagic stroke** ISCHEMIC STROKE {#ischemic-stroke.TransSub-subtopic2} --------------- - Can be arterial or venous - CLASSIFICATION OF ISCHEMIC STROKE ARTERIAL ISCHEMIC STROKE {#arterial-ischemic-stroke.TransSub-subtopic2} ------------------------ Old definition: An acute focal neurological deficit lasting \>24hrs with neuroimaging evidence of cerebral infarction  **Figure 3. Ischemic stroke; brain MRI (DWI Seq): LMCA Territory Infarction** New definition: Infarction in a focal arterial distribution resulting from occlusion of cerebral arteries There is no longer a time window for the duration of symptoms **Figure 4. Ischemic stroke** CAUSES OF STROKE IN CHILDREN {#causes-of-stroke-in-children.TransOutline} ============================ Risk for AIS: Cardiac conditions Vasculopathy / arteriopathy Hypercoagulable states CNS infections A. CARDIAC CONDITIONS {#a.-cardiac-conditions.TransSubtopic1} --------------------- Congenital heart disease: TOF (Tetralogy of Fallot) Cardiomyopathy Arrhythmia Endocarditis Acquired, such as patients with RHD Cardiac mass: Rhabdomyoma Paradoxical embolus: Patent Foramen Ovale (PFO) Cardiac procedure-related: Balloon atrial septostomy Cardiac masses associated with tuberous sclerosis \[2024\] All children with suspected AIS requires a thorough cardiovascular examination, an ECG + Echocardiogram  **Figure 5. 3 day old w/ cyanotic heart diseases & embolic strokes post BAS** The arrow points to the stroke on CT scan. The stroke is more evident on MRI on the 3^rd^ day of life. B. COMMON VASCULOPATHIES {#b.-common-vasculopathies.TransSubtopic1} ------------------------ ARTERIAL DISSECTION {#arterial-dissection.TransSub-subtopic2} ------------------- Results from bleeding into the vessel wall with blood spreading distally. Sub-intimal hematoma may narrow the lumen, producing ischemia by 2 possible mechanisms: Hemodynamic changes Embolism **Figure 6. 5y/o boy with arterial dissection** - The arrow points to the blood clot in the R vertebral artery to your left and on angiography to your right MOYA MOYA {#moya-moya.TransSub-subtopic2} --------- Japanese term for **"puff of smoke"** appearance seen on angiography Created by collateral vessels that develop secondary to a progressive, proximal, non-inflammatory, occlusive vasculopathy of the distal ICA. Moya moya is characterized by PROGRESSIVE OCCLUSION OF DISTAL INTERNAL CAROTID ARTERIES (ICAs) & BRANCHES OF THE CIRCLE OF WILLIS **Primary (Disease)** Disease = Idiopathic = Primary Familial in 7-10% with gene in chromosomes 3,16,17 Cause of childhood stoke in asians with mutations in the ring finger 213 gene (RNF 213) **Secondary (Syndrome)** Secondary to underlying conditions Neurofibromatosis Sickle cell disease Radiation therapy (Post-radiation vasculopathy) Down syndrome Congenital arteriopathies **Figure 7. 6 y/o boy with moya moya disease** - Yellow circle: Stenosis - Right: Puff of smoke appearance of the moya moya vessels on angiography - \[2024\] Puff of smoke appearance -- collateral vessels which are formed to compensate for the occlusion. The blood supply is insufficient, so smaller vessels are formed TRANSIENT CEREBRAL ANGIOGRAPATHY (TCA) {#transient-cerebral-angiograpathy-tca.TransSub-subtopic2} -------------------------------------- Monophasic, self-limiting large and medium vessel vasculitis. AKA "NON-PROGRESSIVE CNS VASCULITIS/POST VARICELLA ANGIOPATHY" If the patient had history of varicella infection within 12 months before stroke onset, we call it POST-VARICELLA ANGIOPATHY **Figure 8. 13y/o with primary CNS vasculitis** This is an example of a primary CNS vasculitis resulting in a left basal ganglia infarct as shown in the arrow on the left and the beaded appearance of the vasculitis in the angiography on the right image. Left MRI: Acute infarct, left basal ganglia Right Conventional angiogram: Beading in left distal ICA. Beading pattern in patients with CNS vasculitis C. HYPERCOAGULABLE STATES {#c.-hypercoagulable-states.TransSubtopic1} ------------------------- CONGENITAL {#congenital.TransSub-subtopic2} ---------- - Inherited deficiencies of coagulation inhibitors - Anti-thrombin - Protein C - Protein S - Inherited increased activity of coagulation proteins - Activated protein C resistance (APCR) - Prothrombin gene mutations ACQUIRED {#acquired.TransSub-subtopic2} -------- - As part of disease - SLE, Malignancy, Nephrotic syndrome - As part of treatment - Chemotherapy, radiotherapy, oral contraceptives, catheter insertion D. CNS INFECTIONS {#d.-cns-infections.TransSubtopic1} ----------------- - PH setting: CNS infection = major risk factor for stroke **Figure 9. CNS infection risk factor for stroke PH** STROKE {#stroke.TransOutline} ====== I. Interplay of multiple risk factors - Congenital thrombophilia & dehydration II. Multiple mechanisms - Prothrombotic state & vasculitis (e.g.SLE) - Coagulation & platelet abnormalities IDIOPATHIC STROKE {#idiopathic-stroke.TransSubtopic1} ----------------- III. In up to 30% of children after extensive investigations - 30% will have no risk factor, even after extensive investigation, and as such is called idiopathic stroke SIGNS AND SYMPTOMS {#signs-and-symptoms.TransSubtopic1} ------------------ IV. Clinical presentation of different signs & symptoms vary depending on AGE V. Neonates - Unexplained seizures - Altered mental status - Focal neurologic signs -- not common 1. Older infants & children - Sudden onset focal neurological deficits - \[2024\] Hemiparesis: common presenting deficit - Cranial nerve paresis - DIFFERENTIAL DIAGNOSIS - Mimics of childhood stroke: Characteristic of a prospective cohort by Shelhaas et al, 2006 - 143 patients suspected to have stroke over 1-year period - Out of 143 patients suspected of having stroke, 21% did not have stroke STROKE MIMICS {#stroke-mimics.TransSubtopic1} ------------- - A stroke mimics as mentioned including Epilepsy, Post-ictal paralysis, ADEM, Cerebelitis, or migraine. - Many disorders mimic stroke - History and clinical presentation often cannot distinguish benign and non-benign conditions - Timely investigations needed especially **[Brain MRI]** A list of medical information Description automatically generated with medium confidence **FIGURE 10. Stroke mimics (Shellhaas et al, 2006).** HISTORY {#history.TransSubtopic1} ------- - Directed at uncovering the potential risk factors HEAD AND NECK {#head-and-neck.TransSub-subtopic2} ------------- - Secondary stroke prevention for AIS - Injury or manipulation, neck pain - Why do you have to ask this in the history? Because neck pain is a harbinger of dissection. - Cranial irradiation - Radiation therapy can predispose the patient to Moyamoya. - Migraine INFECTION {#infection.TransSub-subtopic2} --------- - Varicella infection or vaccination - Other infections (otitis media, meningitis, etc) DRUGS {#drugs.TransSub-subtopic2} ----- - Oral contraceptive use - Amphetamine or cocain use - Chemotherapeutic agents SYSTEMIC DISEASE {#systemic-disease.TransSub-subtopic2} ---------------- - SLE, malignancy, cardiac disease HISTORY FOR NEONATAL AIS (ARTERIAL ISCHEMIC STROKE) {#history-for-neonatal-ais-arterial-ischemic-stroke.TransSub-subtopic2} --------------------------------------------------- - Birth and perinatal course - Manner of delivery - APGARs - Placenta - Maternal history - General health - Miscarriages FAMILY MEDICAL HISTORY {#family-medical-history.TransSub-subtopic2} ---------------------- - Early stroke - Early myocardial infarction - Lipid problems - Clotting problems - Fetal losses - Connective tissue diseases - Intracranial arteriopathies - Neurocutaneous syndromes - Migraine DIAGNOSTIC EVALUATION {#diagnostic-evaluation.TransSubtopic1} --------------------- - Confrim presence and type of stroke - Ischemic vs hemorrhagic - Arterial vs venous - Work-up for stroke risk factors NEUROIMAGING {#neuroimaging.TransSub-subtopic2} ------------ - Secondary stroke prevention for AIS - Brain parenchyma - CT Scan - It will distinguish hemorrhagic from ischemic lesions but may fail to identify smaller ischemic lesions. - MRI - The diffusion weighted imaging sequence (DWI) can detect ischemia a few minutes after the stroke and can also detect small lesions missed by a CT scan. - 🕮 **MRI** is superior to CT for **infarctions**, and **MRA** can show **abnormalities of the larger cerebral vessels**. Doing both studies at times provides complementary information - MRA is ideal for patients at additional risk from standard angiography or for those whose diagnosis is already suspected. - Blood Vessels (Arteries for AIS) - Conventional angiography - CT angiography (CTA) - MR angiography (MRA) +-----------------------------------+-----------------------------------+ | **Table 01. Cranial CT Scan** | | +===================================+===================================+ | **Advantages** | **Disadvantages** | +-----------------------------------+-----------------------------------+ | - Widely accessible | - If done too early, maybe | | | normal or | | - Convenient | | | | - If present, findings often | | - Less contraindications | subtle and usually missed | | | | | - Fast | - If seen, lesion not always | | | specific for stroke | | - Easily rules out hemorrhages | | | | - Radiation exposure | | - Less expensive MRI | | +-----------------------------------+-----------------------------------+ +-----------------------------------+-----------------------------------+ | **Table 02. Cranial MRI** | | +===================================+===================================+ | **Advantages** | **Disadvantages** | +-----------------------------------+-----------------------------------+ | - **Sensitive** in detecting | - Not widely accessible | | ischemia early (few minutes | | | after stroke onset), e.g. DWI | - **Contraindications** | | | (braces, pacemakers) | | - Can detect **small lesions** | | | missed by CT (Lesion is more | - Longer scanning time (\~30min | | visible on MRI) | -- 1hr) | | | | | | - May need general anesthesia | | | | | | - More expensive | +-----------------------------------+-----------------------------------+  **Figure 11. CT Scan vs. MRI (DWI). Note that the lesion (encircled in yellow) is more visible in MRI.** +-----------------------+-----------------------+-----------------------+ | **Table 3. CTA or MRA | | | | vs. Conventional | | | | Angiography** | | | +=======================+=======================+=======================+ | **Advantages** | **Disadvantages** | | +-----------------------+-----------------------+-----------------------+ | **CT Angiography | - Done to image the | - Lower resolution | | (CTA) or MR | cerebral blood | | | Angiography (MRA)** | vessels | (compared to | | | | Conventional | | | - Non-invasive | Angiography) | | | | | | | - Shorter hospital | - Especially if (1) | | | stay (can be done | appropriate | | | as an OPD) | region is not | | | | scanned; (2) | | | - No radiation for | technique is not | | | MRA | good, (3) | | | | Artifacts present | | | - MRA -ideal for | | | | patients at | | | | additional risk | | | | from standard | | | | angiography or | | | | those whose | | | | diagnosis is | | | | already suspected | | +-----------------------+-----------------------+-----------------------+ | **Conventional | - **Gold standard | - Invasive | | Angiography** | for imaging of | | | | blood vessels** | - Radiation | | | | exposure | | | - Better | | | | resolution; thus, | - Time-consuming | | | done if MRA/CTA | | | | quivocal | | +-----------------------+-----------------------+-----------------------+ STROKE WORK-UP {#stroke-work-up.TransSub-subtopic2} -------------- - Routine work-up includes - CBC - Thrombophilia work-up (Class IIa, Level C) - Anti-thrombin, protein c, protein s - Activated protein C resistance (APCR) - Lipoprotein A, homocysteine - Anti-cardiolipin antibodies, lupus anti-coagulant - Genetic mutations (prothrombin gene 20210A, MTHFR, Factor V Leiden) - Factors VII, IX, XI - Plasminogen, fibrinogen - Echocardiogram +/- bubble study - To look for patent foramen ovale - (to look for cardiac diseases) - Imaging brain parenchyma and blood vessels, prothrombotic workup, cardiac work up and all other tests mentioned above are required. These are routinely requested for a patient with stroke. - These are not part of the routine stroke work-up but if you **suspect vasculitis or a CNS infection** then you need to do these tests. - Vasculitis work-up: ANA, ANCA, RF etc. - Infectious work up: ESR, CRP, microbiologic (viral and/or bacterial) studies in CSF and blood MEDICAL MANAGEMENT {#medical-management.TransOutline} ================== - Medical - Surgical - Supportive - Rehabilitation therapy - Stroke in children management is **multi-modal.** **Figure 12. Definition of Classes and Levels of Evidence used in AHA Stroke Council Recommendation.** MEDICAL MANAGEMENT {#medical-management-1.TransSubtopic1} ------------------ - Secondary stroke prevention for AIS - Treat underlying risk factor/s - (Class I, Level of evidence C); So, if the patient has a CNS infection, then you must treat the CNS infection - Anti-thrombotic agents🖊️ you can use this for secondary stroke prophylaxis. - Tissue plasminogen activator (t-PA) - Heparin (UFH, LMWH) - Warfarin - Anti-platelet (aspirin, clopidogrel) TISSUE PLASMINOGEN ACTIVATOR (t-PA) {#tissue-plasminogen-activator-t-pa.TransSub-subtopic2} ----------------------------------- - Acute stroke if with no contraindications in selected centers abroad. 🖊️ However, it is not yet done routinely in the Philippines. - Not recommended for children with AIS outside a clinical trial - Class III, Level of evidence C - No consensus about the use of tPA in older adolescents meeting adult tPA eligibility criteria. - A.K.A. "Clot Busters" - Therapeutic time window: - IV tPA in adults: **4.5 hours;** 🖊️ Meaning, within 4.5 hours, You must administer IV tPA, beyond that, it\'s no longer possible. What will be given next is Intraarterial tPA (performed with Angiography). - IA tPA therapeutic time window: **6 hours** HEPARIN (UNFRACTIONATED OR LOW MOLECULAR WEIGHT) {#heparin-unfractionated-or-low-molecular-weight.TransSub-subtopic2} ------------------------------------------------ - May be given **up to 1 week after** an ischemic stroke pending further evaluation to determine the cause of the stroke - Class IIb, Level of evidence C - Two types of Heparins: - **Unfractionated**: Intravenous administration - Low molecular weight: Subcutaneous LONG-TERM ANTICOAGULATION (LMWH) {#long-term-anticoagulation-lmwh.TransSub-subtopic2} -------------------------------- - Secondary stroke prophylaxis - Cardioembolic strokes - Class I, Level of evidence C - Extracranial arterial dissection - Class IIa, Level of evidence C - Selected prothrombotic states - Class I, Level of evidence C - Recurrent strokes or TIA while on therapeutic aspirin - Given to avoid recurrence of stroke LONG-TERM ANTICOAGULATION (WARFARIN) {#long-term-anticoagulation-warfarin.TransSub-subtopic2} ------------------------------------ - Secondary stroke prevention - Class IIa, Level of evidence C - Cardioembolic strokes - Extracranial arterial dissection - Selected prothrombotic states - Warfarin is given orally. - Initiation of warfarin therapy is challenging, since the pharmacodynamic response is delayed and difficult to predict. (A review of warfarin dosing and monitoring, Kuruvilla et al.) {#section.TransSub-subtopic2} ANTI-PLATELET AGENTS {#anti-platelet-agents.TransSub-subtopic2} -------------------- - Secondary stroke prevention for AIS - 🖊️ For all those patients with stroke that are not due to the conditions mentioned for indications on LMWH and Warfarin use. - Not due to sickle disease or severe hypercoagulable disorder and - Those with low risk of recurrent embolism - Aspirin (Class IIa, level of evidence C) - Commonly used anti-platelet in children - Daily low-dose aspirin seems to be safe - 🖊️ Clopidogrel if unable to take aspirin, or aspirin failure. SURGICAL MANAGEMENT {#surgical-management.TransSubtopic1} ------------------- - Revascularization surgery for patients with Moyamoya - Decompressive hemicraniectomy as needed - Performed in patients with malignant brain infarction SUPPORTIVE MANAGEMENT {#supportive-management.TransSubtopic1} --------------------- I. 🖊️ Supportive management is just as important. CLASS I, LEVEL OF EVIDENCE C {#class-i-level-of-evidence-c.TransSub-subtopic2} ---------------------------- - Fever control - Normal oxygenation - Control of hypertension - Normalization of serum glucose CLASS IIA, LEVEL OF EVIDENCE C {#class-iia-level-of-evidence-c.TransSub-subtopic2} ------------------------------ - Treat dehydration and anemia CLASS III, LEVEL OF EVIDENCE C {#class-iii-level-of-evidence-c.TransSub-subtopic2} ------------------------------ - Supplemental oxygen NOT beneficial in absence of hypoxemia - Seizure prophylaxis NOT necessary - Unless the patient develops seizure. - Hypothermia should NOT be used. RECOMMENDATIONS FOR REHABILITATION {#recommendations-for-rehabilitation.TransSubtopic1} ---------------------------------- - Age-appropriate rehab - Class I, Level of evidence C - Psychological assessment to document cognitive and language deficits for planning therapy and educational programs. - Class I, Level of evidence C - 🖊️ Age-appropriate rehab and psychological assessment are all also important to plan therapy and educational programs to improve outcomes of patients with stroke. OUTCOMES {#outcomes.TransOutline} ======== A. NEUROLOGIC SEQUELAE {#a.-neurologic-sequelae.TransSubtopic1} ---------------------- - ⭐ Motor deficits -- most common - Language difficulties - Neuropsychiatric, cognitive, behavioral - Epilepsy -- 30 to 50% - Movement disorders - Headache B. STROKE RECURRENCE {#b.-stroke-recurrence.TransSubtopic1} -------------------- - 10 -- 30% of cases on the average - Usually within 6 months from initial stroke - Associated with (highest risk) - ⭐ Presence and severity of vasculopathy - ⭐ Prothrombic risk factors CEREBRAL SINOVENOUS THROMBOSIS (CSVT) {#cerebral-sinovenous-thrombosis-csvt.TransOutline} ===================================== - Thrombotic disruption of flow in the cerebral venous sinuses - May or may not result in venous infarct. - 📖 Majority of infarcts are hemorrhagic - 📖 Prothrombic states may be the sole risk factor for CSVT or in combination with other risk factors.  **Figure13. Overview of venous drainage** (2025) - Brain dysfunction occurs due to thrombotic occlusion of cerebral veins and/or dural venous sinuses. - Occurs more frequently in neonates and in males. - CSVT essentially results from a "hypercoagulable" state secondary to Virchow's triad. The role of coagulation cascade and thrombin-rich thrombosis predominates. - Clinical diagnosis is challenging due to the nonspecific nature of presenting neurologic symptoms and signs that can develop gradually over many hours, days, or even weeks. Abrupt onset can also occur. - CSVT must be ruled out in all children with suspected - Idiopathic Intracranial Hypertension (IIH) NEUROIMAGING FOR CSVT {#neuroimaging-for-csvt.TransSubtopic1} --------------------- MR VENOGRAM {#mr-venogram.TransSub-subtopic2} ----------- **Figure14.** **MR Venogram Flow artifacts mistaken as CVST (arrow)** - Advantages: - No radiation - N o contrast - Disadvantage: - Flow artifacts mistaken as CSVT (see arrow) - MRI is the diagnostic modality of choice for pediatric CSVT. CT VENOGRAM {#ct-venogram.TransSub-subtopic2} -----------  **Figure15. CT Venogram** - Advantage: - Better visualization (arrowhead) - Disadvantages: - Use of contrast - Radiation exposure - \(2025) Thrombus is represented by filling defect in the vein/sinus in which contrast through a narrowed channel or bypass via venous collaterals. - The empty triangle or "empty delta" sign is seen with occlusion of the larger dural sinuses like SSS and TS. It refers to the shape of the enhancing dura encompassing the non-enhancing intraluminal clot on axial CT slice. HEMORRHAGIC STROKE {#hemorrhagic-stroke.TransOutline} ================== - \(2025) Nice to know: - High Flow Lesions - Arteriovenous Malformations - Arteriovenous Fistulas - Vein of Galen Malformations - Low Flow Lesions - Cavernous Malformations - Aneurysm SPONTANEOUS CHILDHOOD HEMORRHAGIC STROKE (SICH) {#spontaneous-childhood-hemorrhagic-stroke-sich.TransSubtopic1} ----------------------------------------------- - When it comes to hemorrhagic stroke, it should be non-traumatic. Meaning there should be no accidents (e.g., not hit or fallen). - Annual incidence: 1.4/100,000 person-years - Median age at stroke onset: 12.2 years - Highest incidence in 0-4 years & 15-19 years - Male predominance - Hemorrhage patterns: - 50% pure intracerebral - 18% pure subarachnoid hemorrhage (SAH) - 32% combination of intracerebral and SAH CLINICAL PRESENTATION (N=116) {#clinical-presentation-n116.TransSub-subtopic2} ----------------------------- - ⭐ Headache -- 66 (57%) - most common presentation - Encephalopathy -- 55 (47%) - Seizure -- 34 (29%) - Focal motor deficit -- 27 (23%) ETIOLOGY- CALIFORNIA DATA (1993-2003) {#etiology--california-data-1993-2003.TransSub-subtopic2} ------------------------------------- **Figure 16. Etiology of SICH. California data.** - AVM -- 31% Ruptured AV malformation - Undetermined -- 25% - Cavernous malformation -- 15% - Aneurysm -- 13% - Brain tumors -- 2% PGH DATA (1994 -- 1999) {#pgh-data-1994-1999.TransSub-subtopic2} -----------------------  **Figure17. Etiology of SICH. PGH Data.** - 162 cases of childhood hemorrhagic stroke - Hematologic -- 32% - APCD -- 32% - AVM -- 16% - Amphetamine -- 10% - Liver -- 1% - Cardiac - Aneurysm - Brain tumor - HIE ANEURYSM {#aneurysm.TransOutline} ======== - Comes from Latin word, "aneurysma" meaning dilatation - 🖊️ One of the most common vascular anomalies of the central nervous system, but are far less common in children than in adults - 🖊️ Can cause bleeding, compression of adjacent structures, and concomitant loss of neurological function - 🖊️ Most aneurysms are asymptomatic and often are never detected - 🖊️ For those that are found because of symptoms: - Headache -- most common (80%) - Loss of consciousness (25%) - Seizure (20%) - Focal neurologic deficits (20%) - Visual changes (10%) - Sudden massive intracranial hemorrhage -- most common clinical manifestation in children - 🖊️ Children with SAH from aneurysm often present with less severe symptoms compared with adults **Figure 18.** **Cerebral Aneurysm**  **Figure 19. Pathogenesis of aneurysm** - Abnormal focal dilatation of the blood vessel wall usually arising from weakness of the elastic and muscular layers of the artery TYPES OF ANEURYSMS {#types-of-aneurysms.TransSubtopic1} ------------------ **Figure 20. Types of Aneurysms** (2025) - **Saccular** - Most often occur at the bifurcation of the internal carotid artery or one of its intermediate branches. Becomes symptomatic before 2 years old or after 10 years old. - **Fusiform** - Most often affects the MCA, ICA, and ACA. Segment of the vessel involved, delineation of normal borders, and involvement of perforators are important. - **Ruptured** - Ruptured aneurysm spills blood into the subarachnoid space →severe headache, vomiting, meningeal irritation, increased ICP → focal neurological deficits, seizures, impaired consciousness, retinal hemorrhages. A. ANEURYSMAL HEMORRHAGIC STROKE {#a.-aneurysmal-hemorrhagic-stroke.TransSubtopic1} -------------------------------- - Annual incidence: 0.18 per 100,000 person -- years - Highest incidence in late adolescence - Independent predictors of underlying aneurysm: - Late adolescent age (15 -- 19 years) - Pure subarachnoid hemorrhage (SAH) - 🖊️ If you have a patient presenting with a pure subarachnoid hemorrhage and the patient is between 15 to 19 years of age, you should highly suspect the presence of ruptured aneurysm.  **Figure 21.** **Annual incidence of aneurysmal hemorrhage stroke.** INTRACRANIAL ANEURYSMS IN CHILDREN ACROSS ALL AGE GROUPS {#intracranial-aneurysms-in-children-across-all-age-groups.TransSubtopic1} -------------------------------------------------------- - Male predominance - Higher frequency in adolescent age group - Mostly single lesion - ⭐ More commonly seen in anterior circulation particularly internal carotid artery (ICA) - Giant size (\>2.5 cm) aneurysms in 22--37% - Mycotic/infectious aneurysms not common - (lec) Remember, this data came from California. However, in the Philippines, we still cases of mycotic aneurysm especially in patients with infected endocarditis secondary to rheumatic heart disease - Mostly good outcome CLINICAL STUDY {#clinical-study.TransSub-subtopic2} -------------- **Figure 22.** **Disorders associated with pediatric intracranial aneurysms.** - This table above can show you that aneurysms and AV malformations can co-exist in one patient. ARTERIOVENOUS MALFORMATION (AVM) {#arteriovenous-malformation-avm.TransSubtopic1} -------------------------------- - Result from embryonic failure of capillary development between artery and vein  **Figure 23. Normal capillary bed versus AVM** - ⭐ "Bag of worms" appearance caused by: - Tangled mass of dilated veins - Enlarged and tortuous arteries feeding these venous channels - Interposed dilated vessels **Figure 24.** **AVM malformation.** LOCATION OF AVM {#location-of-avm.TransSub-subtopic2} --------------- - Cerebral hemispheres -- 67% - Cerebellum -- 13% - Brainstem -- 11% - Thalamus or basal ganglia -- 5% CLINICAL PRESENTATION {#clinical-presentation.TransSub-subtopic2} --------------------- - Intracranial hemorrhage in majority - Seizures - Headaches - Progressive neurologic deficits DIAGNOSTICS {#diagnostics.TransSub-subtopic2} ----------- - Cerebral angiography -- Gold standard  **Figure 25. Cerebral Angiography showing an AVM** MANAGEMENT {#management.TransSub-subtopic2} ---------- - General - ABC of resuscitation - Treat increased ICP - Control seizures if present - Neuroprotective measures - Specific/Definitive Management - Surgery +/- Embolization - Stereotactic radiosurgery/Gamma knife ACQUIRED PROTHROMBIN COMPLEX DEFICIENCY (APCD) {#acquired-prothrombin-complex-deficiency-apcd.TransSubtopic1} ---------------------------------------------- - Acquired bleeding disorder in infants between 1 to 12 months of age due to deficiency of Vitamin K dependent clotting factors - May present with bleeding (intracranial, GI, and/or skin) - Prevalent in breastfed infants - Predisposing factors: diarrhea, starvation, not given Vitamin K at birth - 🖊️ Thus, this is common in patients who are born at home - \(2025) That's why many cases of APCD (Acquired Vitamin K-Deficiency Bleeding) occur in the Philippines and Southeast Asia because traditional birth attendants do not administer Vitamin K to newborns during home deliveries. SUMMARY {#summary.TransOutline} ======= - Stroke can occur in children. - Risk factors are varied. - Management includes: - Identifying and treating underlying risk factors to prevent recurrence and improve outcome. - Secondary stroke prevention with use of anti-thrombotic agents in AIS - Regularly rehab to minimize long term disability. PLENARY DISCUSSION {#plenary-discussion.TransOutline} ================== GENERAL DATA {#general-data.TransSub-subtopic2} ------------ - AB, 7-year-old boy from Manila CHIEF COMPLAINT {#chief-complaint.TransSub-subtopic2} --------------- - Left arm and leg weakness HISTORY OF PRESENT ILLNESS {#history-of-present-illness.TransSub-subtopic2} -------------------------- - 1 hour PTC, the patient woke up with a note of left arm and leg weakness. He tried to stand up but was not able to do so, prompting consult at the ER. REVIEW OF SYSTEMS {#review-of-systems.TransSub-subtopic2} ----------------- - (+) Headache associated with weakness but no headaches in the past - No Cough/colds - No Fever - No Vomiting - No Seizure - No Diarrhea #### ANYTHING ELSE THAT YOU WOULD LIKE TO ASK FOR THE ROS? {#anything-else-that-you-would-like-to-ask-for-the-ros.TransSub-subtopic3} - 🖊️ You need to note all those things especially if you want to know if it\'s a red flag or it is something usual type or primary headache. - 🖊️ For this patient, he had a sudden diffuse type of headache experienced for the first time with the pain of 6/10. There were no provoking factors and there were no medications given for palliation. ANCILLARY HISTORY {#ancillary-history.TransSub-subtopic2} ----------------- - Past Medical History: No trauma, No hospitalization - Personal & Social History: Grade 1 average student - Nutritional History: No food preference - Developmental History: Normal - Immunization history: Care of local health center - Family history: (+) Hypertension in grandfather #### ANYTHING ELSE THAT YOU WOULD LIKE TO ASK FOR ANCILLARY HISTORY? {#anything-else-that-you-would-like-to-ask-for-ancillary-history.TransSub-subtopic3} - 🖊️ P&SH: Environmental factors - If the patient is exposed to smoking. Is there adequate ventilation in the house? - 🖊️ PMH: Are there any comorbidities present in the household? (Diabetes, Stroke, Hypertension etc.) - Relevant if the onset of stroke is \< 45 years old -- Early onset of stroke. - Yung mga apparently healthy with no hypertension, nonsmoker, no diabetes tapos nagkaroon ng stroke \ - 🖊️ NH: What specific diet does the patient have? - Baka obese naman pala tong pasyente na to or may hyperlipidemia so it is important to know what the patient eats. - Mali na nga yung kinakain sedentary pa yung lifestyle - 🖊️ PMH: Are there any history of seizures or previous infections that do not lead to hospitalizations? - Chicken pox infections may have stroke presentations - In relation to the patient who is 7 y/o, for example he had chickenpox infection at 2 y/o would it still be significant? - We will not totally disregard the information which is important but there would be decreased probability that this is the cause if the chickenpox infection happened 5 years ago. - Does the patient have any cardiac conditions? - It can be a potential risk factor for stroke in this patient. - 🖊️ Sleeping patterns - Can be compromised due to increased screen time of the child - It will also give an idea to the timing of medications kasi minsan diba iba yung oras ng tulog so yung oras ng inom ng gamot nilaif they are taking maintenance meds maiiba rin. - It depends on the diagnosis of the patient (e.g. Seizures) but it is not that necessary for this case. - 🖊️ Immunization history: What vaccines are given to the patient and if it is completed. - By this time the patient should have received vaccines like MMR, Polio, PCV, HiB etc. - It depends on what is available on the heath center because sometimes some vaccines are not available in in the center. - Ask the parents what specific vaccines do the patient have and have not received. - Sometimes there are cases that they do not remember the vaccines received by the patient, so you must be familiar with what is available in the health center. - Usual vaccines available in health centers: BCG, Hep B, PCV (in some centers), HiB, Polio, DPT, MMR. - 🖊️ Birth and Maternal History to note if there are any cardiovascular abnormalities such as heart failure defects or valvular abnormalities (child) - Important because sometimes what if the mother is gravidocardiac so pwede pa ring may problem potentially (nawala audio) yung mommy. PHYSICAL EXAM {#physical-exam.TransSub-subtopic2} ------------- - Vital Signs - HR 110 bpm - RR 20 cpm - Temp 36.8 C - Weight: 30 kg - Pink conjunctivae, anicteric sclerae - No neck mass, no cervical lymphadenopathy - Clear breath sounds, no murmur - Abdomen: globular, normoactive bowel sounds, non-tender - Skin: no lesions/rashes but with abrasions on the left arm from the fall - Extremities: pink nailbeds, no edema #### ANYTHING ELSE THAT YOU WOULD LIKE TO ASK FOR THE PHYSICAL EXAMINATION? {#anything-else-that-you-would-like-to-ask-for-the-physical-examination.TransSub-subtopic3} - 🖊️ Blood Pressure -- 90/60 mmHg - 🖊️ Pain Scale for Headache - 🖊️ Head lesions or traumas - Sometimes it is good that you ask because there may be cases of child abuse which the family will deny the trauma but when you look at the child there may be bruises or wounds. - 🖊️ Growth Charts - Some disease conditions like failure to thrive or stunting may be indicative that the patient may have hypopituitarism or other conditions neurologically that might have endocrine effects - 🖊️ BMI - Ask for height NEUROLOGIC EXAMS {#neurologic-exams.TransSub-subtopic2} ---------------- - Mental status: Awake, oriented, followed commands - Cranial Nerves - Visual acuity: 20/20 - Pupils 3 mm EBRTL, no papilledema - Full EOM movement - Good masseter tone - No facial asymmetry - Gross hearing intact - Weak left shoulder shrug - Tongue midline - Motors - Normal muscle bulk - Flaccid tone left arm and leg but normal tone right extremities - Normal tone right extremities - Dense hemiplegia of left arm and leg - DTRs: - Right extremities ++ (Normal reflex) - Left extremities +++ (Hyperreflexia) - Cerebellar: no dysmetria on right, not assessed on left - Sensory: intact to touch - Supple neck - Babinski and ankle clonus, left #### ANYTHING ELSE THAT YOU WOULD LIKE TO ASK FOR THE NEUROLOGICAL EXAMINATION? {#anything-else-that-you-would-like-to-ask-for-the-neurological-examination.TransSub-subtopic3} - 🖊️ Sensory Examination -- asses for whole sensory exam (position sense, vibration, etc.) - 🖊️ Is there a lesion? - Yes, Right Subcortical lesion at the Internal Capsule because even if the patient has headache, he is very much awake, has very dense hemiplegia (equal weakness of arms and legs) which points more towards a subcortical lesion. - In the motor homunculus, if the feet are weaker most likely it is in the ACA distribution or in the parasagittal distribution while if the hands are weaker, it is in the MCA distribution (can be in the frontal cortex). If this is presented with seizure or decreased sensorium with asymmetric weakness, then it might be cortical. QUESTIONS {#questions.TransSub-subtopic2} --------- #### WHAT ARE YOUR DIFFERENTIAL DIAGNOSIS? {#what-are-your-differential-diagnosis.TransSub-subtopic3} a\) Transient Ischemic attack b\) Post-ictal paralysis c\) Stroke **d) All of the above** - 🖊️By history, the patient woke up with one sided weakness and since we do not have MRI, MRA, or CT scan results then you cannot tell if it\'s stroke or TIA. - 🖊️It can be stroke or TIA because there are still no imaging results yet. - 🖊️Post-ictal paralysis /Todd's paralysis- cannot be ruled out at this point because he woke up with weakness already and you did not saw his sudden weakness without the seizure first. We must investigate further if he does have seizures, that is why when he woke up the other side was weaker than the other? +-----------------------------------------------------------------------+ | ***2025*** | | | | - It\'s challenging to differentiate between a stroke/TIA because | | there\'s no history indicating the resolution of weakness. | | | | - Why can\'t we rule out TIA vs. Stroke? | | | | - Even if the weakness resolved within an hour, if imaging | | studies reveal an occlusion/infarct (+), then it\'s | | considered a stroke.  Even if the weakness resolves, if | | there is imaging evidence of an infarct (+), it\'s classified | | as a stroke, not a TIA. | +-----------------------------------------------------------------------+ #### WHAT DIAGNOSTIC TESTS SHOULD YOU REQUEST? {#what-diagnostic-tests-should-you-request.TransSub-subtopic3} a\) Cranial CT scan b\) Cranial MRI **c) Cranial MRI/MRA** d\) Skeletal survey/X-ray - Many disorders mimic stroke - Cranial CT scan, if done too early and if the lesion is small, may not reveal infarct - 🖊️ May be done if there are no other rsources. - Cranial DWI (DWI Sequence) is sensitive in detecting ischemia early. - 🖊️ Kahit few minutes pa lang pwede na mapick up ng MRI kung maystrokie sya pero kelangan yung DWI sequence. - Di pwedeng MRI na walang DWI sequence kasi it will take time also for the lesion, if it's a stroke, to be reflected in other MRI sequences kung walang DWI. #### WHAT IS THE DIAGNOSIS? {#what-is-the-diagnosis.TransSub-subtopic3} **Figure X. (Left to Right) DWI sequence, ABC map, MRA** a\) Transient Ischemic Attack **b) Arterial Ischemic Stroke** c\) Hemorrhagic Stroke d\) Cerebral Sinovenous Thrombosis - Complete diagnosis: ACUTE ISCHEMIC STROKE, RIGHT MCA DISTRIBUTION +-----------------------------------------------------------------------+ | **2025** | | | | It follows the vascular distribution pattern of the middle cerebral | | artery (MCA), and additional evidence lies in the fact that the right | | side of the MCA appears interrupted, unlike in the case where blood | | flow is continuous. This indicates the presence of an occlusion, | | leading to the infarction in the right MCA territory. This is an | | acute ischemic stroke, as evidenced by the brightness on the DWI and | | the corresponding dark area on the ABC map. | +-----------------------------------------------------------------------+ #### {#section-1.TransSub-subtopic3} #### WHAT IS A POSSIBLE RISK FACTOR FOR THIS PATENT? {#what-is-a-possible-risk-factor-for-this-patent.TransSub-subtopic3} a\) Tetralogy of Fallot b\) TB meningitis c\) Moyamoya syndrome **d) Protein S deficiency** - 🖊️ Protein S Deficiency (Hypercoagulable states) -- acts as anticoagulant by directly inhibiting factor Va, Xa, and IXa (procoagulants). In the case of TIA, it can cause thrombus formation. - 🖊️ Not TB Meningitis (CNS Infections) because: - Patient does not have any fever or signs of infection - There was no familial history of TB - In PE there is no nuchal rigidity and by history the presentation is very acute because in TB meningitis they already have symptoms of fever, weight loss, on-and-off headaches for a few weeks etc. before they can have stroke. - But TB meningitis and other CNS infections can be a risk factor for stroke. It was just ruled out because the symptoms presented are very acute onset. - 🖊️ Not Moya-moya Syndrome (Vasculopathies) because: - MRA results showed an infarction rather than puff of smoke (not always present specially in early stages) and usually ICA is affected in moya-moya not the MCA. - MRA results of moya-moya usually show bilateral stenosis in terminal ICA-MCA junction and have collateral formation compared to those in AIS wherein the imaging showed only a right MCA. - There is no history of prior strokes. +-----------------------------------------------------------------------+ | **2025** | | | | Additionally, based on imaging (ABC map), the infarction appears too | | large. Typically, Moyamoya disease presents with small or lacunar | | infarctions in areas with limited blood supply, such as the basal | | ganglia or corona radiata. | +-----------------------------------------------------------------------+ - 🖊️ Not TOF (Cardiac Conditions) because: - Patient has normal vital signs - Patients with TOF will present with cyanosis and become tired quickly. - By this time, there is failure-to-thrive, they are thin and underweight and have cyanotic nailbeds with clubbing. - In our patient, although there are no signs of clubbing mentioned, he has pink nailbeds, no murmurs, and he has normal development. #### WHAT DRUG WILL YOU GIVE FOR SECONDARY STROKE PREVENTION? {#what-drug-will-you-give-for-secondary-stroke-prevention.TransSub-subtopic3} a\) Aspirin b\) Tissue Plasminogen Activator **c) Warfarin** d\) Ibuprofen - 🖊️ Not aspirin because: - It is an antiplatelet agent and if this patient has protein S deficiency there will be abnormal coagulation factors so the use of aspirin will not be effective because its target is platelet aggregation. - Warfarin - Indicated for cardioembolic strokes, extracranial arterial dissection, selected prothrombotic states. (2025) #### HOW WILL YOU MANAGE THIS PATIENT? {#how-will-you-manage-this-patient.TransSub-subtopic3} - Treat underlying risk factor - 🖊️ So, if it is protein S deficiency then address the deficiency but there's nothing you can do because it's usually a congenital condition. - 🖊️ If TB meningitis is the cause, then you can treat it. - Secondary risk prevention using anti-thrombotic agents - Aspirin - Heparin or Warfarin - Supportive management - 🖊️ If the patient has fever, then control the fever. If he has hypertension or hyperglycemia, you must correct those coexisting problems or complications. In cases of seizures, you give antiseizure medications. - Rehabilitation Therapy - 🖊️ Eventually because of the weakness, since there is dense hemiplegia, he can't walk or stand there is a need of rehab therapy so that he can be as normal as possible. Outcomes may vary because some patients go back to normal like nothing happened, but they are patients who have permanent residual disability (seizures, headache disorders, epilepsy, language and cognitive problems) depending on which part of the brain was affected by the stroke. REFERENCES {#references.TransOutline} ========== - 2025COM-Transcription - Marilyn Ta, MD. 2024. Child Stroke Lecture and Plenary Session.