intracerebral hemorrhage: Acute treatment and prognosis", section on 'Surgical approaches for selected patients'.) As children typically lack the baseline cerebral atrophy found in older adults that permits expansion of the hematoma without consequent compression of the surrounding parenchyma, it is biologically plausible that children may benefit from hematoma evacuation to reduce ICP. If a child is undergoing resection of an underlying vascular malformation that is at high risk for acute rebleeding, it may be optimal to concurrently evacuate the hematoma. Surgical evaluation also might be warranted if a child is comatose, has elevated intracranial pressure that is refractory to medical management, or a worsening neurological examination. As in adults, cerebellar hemorrhages >3 cm in diameter in a child who is deteriorating or in whom brainstem compression and/or hydrocephalus is developing due to compression on the ventricular system should also be considered for surgical evacuation. If a cerebellar hemorrhage is evacuated, suboccipital craniectomy is typically performed at the same time. While hemicraniectomy has not been studied in the setting of pediatric hemorrhagic stroke, there are some series in which hemicraniectomy was associated with improved function and survival in pediatric arterial ischemic stroke [74,75]. In a child who undergoes surgical hematoma evacuation due to a supratentorial hemorrhage causing elevated intracranial pressure that is refractory to medical management, the surgeon may elect to perform a concomitant hemicraniectomy, particularly if the intracranial pressure remains elevated after hematoma evacuation or if there is herniation out of the craniotomy defect. Identifying the etiology Obtaining dedicated cerebrovascular imaging in the acute setting is critical to guide appropriate interventions given the high rate of vascular malformations underlying hemorrhagic stroke in children. Cerebral angiography is a minimally invasive modality that may be used for diagnosis and treatment of vascular causes of ICH [76]. While conventional cerebral angiography remains the gold standard, many institutions opt to first use noninvasive modalities. One retrospective study found that a combination of magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and magnetic resonance venography (MRV) images accurately identified the cause of ICH in 66 percent of subjects, which was statistically equivalent to the diagnostic yield of conventional cerebral angiography alone (61 percent) [77]. However, another retrospective case series of children with nontraumatic ICH reported identification of the cause of bleeding in 97 percent of children who underwent conventional cerebral angiography compared with 80 percent of children who did not have angiography [7]. Another alternative is CT angiography (CTA), which can be rapidly obtained without the need for sedation in some children, is more widely available than MRA, and may offer superior https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 13/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate angioarchitectural visualization compared with MRA [78]. CTA also has a higher sensitivity for detecting aneurysms up to 2 mm in size. However, CTA exposes the child to both ionizing radiation and an iodinated contrast agent, requires a large bore intravenous line, and may be nondiagnostic if the child moves during the study. For children with hemorrhagic stroke and no identified cause despite a thorough evaluation, including appropriate noninvasive vascular imaging, we suggest conventional cerebral angiography. As an acute hemorrhage with large hematoma and significant cerebral edema can obscure visualization of an underlying vascular malformation, vascular studies that are initially nondiagnostic should be repeated weeks to months later once the clot has been reabsorbed if no other cause for the hemorrhage (eg, tumor, coagulopathy) is found [76]. The yield of an extensive evaluation for a bleeding diathesis in children with hemorrhagic stroke is not well-studied. A rational approach is to obtain the screening laboratory studies suggested above (see 'Laboratory studies' above) (ie, a complete blood count with platelets, coagulation studies, prothrombin time, international normalized ratio, and activated partial thromboplastin time) and to pursue additional testing if the screening studies are abnormal or if an underlying vascular lesion or tumor is not found. Higher-level studies may include factor VIII, IX, and XIII levels and von Willebrand disease studies and should be ordered in consultation with a hematologist. (See "Approach to the child with bleeding symptoms" and "Approach to the child with unexplained thrombocytopenia" and "Clinical manifestations and diagnosis of hemophilia" and "Clinical presentation and diagnosis of von Willebrand disease".) Treatment of vascular lesions Endovascular and/or surgical management of vascular malformations may be required in the acute setting depending on the location and vascular anatomy of the lesion in conjunction with the clinical status of the child. Multidisciplinary consultation with neurosurgery, interventional radiology, and neurology is advised to choose the optimal approach to treatment of these lesions [67]. Vascular malformations other than aneurysm typically have a low risk of acute rebleeding (although they may rebleed at later times) [79-81]. Therefore, many centers will await hematoma resolution prior to definitive treatment so that the full extent of the vascular malformation can be elucidated. However, if hematoma evacuation is needed, a vascular malformation may be addressed at the same time. Some arteriovenous malformations that cannot be treated with endovascular or surgical techniques may be amenable to gamma knife or proton beam therapy once the hematoma has retracted. Aneurysms have a higher rate of acute rebleeding [82]. Therefore, aneurysm repair typically occurs in the acute setting. AVMs with an aneurysmal component that may cause acute https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 14/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate rebleeding also may require earlier intervention. Follow-up imaging Due to the high risk of recurrence, we suggest follow-up imaging for most children with hemorrhagic stroke due to a vascular malformation. In addition, we suggest follow-up imaging in cases where a vascular cause is not found but is suspected. Even when complete resection of an arteriovenous malformation is achieved, there is a substantial risk of recurrence. In one retrospective report of 28 children who underwent surgical resection of arteriovenous malformations, 4 had recurrence leading to repeat resections [83]. Of note, two of the children in this cohort had arteriovenous malformations that were not detected until 17.7 and 25 months after the incident hemorrhage. While the frequency and modality of follow-up imaging is center-dependent, our suggested protocol is to obtain brain MRI with MRA at three to six months after the first hemorrhage, and/or a conventional angiogram between three and six months. Additional follow-up imaging at later points is typically necessary. In children with an unresected cavernous malformation, periodic imaging with MRI is suggested if the child is having frequent symptoms such as headaches or seizures. Genetic screening Genetic screening may be reasonable if multiple vascular malformations are found on imaging or if there is a suggestive family history [67]. The most common genetic cause of brain AVMs is hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant condition. Patients with HHT may have cerebral or spinal cord involvement with telangiectasias, brain AVMs, aneurysms, or cavernous malformations. (See "Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber- Rendu syndrome)", section on 'Genetic testing'.) Familial cases of cavernous malformation are associated with genetic variants of CCM1, CCM2, and CCM3. (See "Vascular malformations of the central nervous system", section on 'Cavernous malformations'.) PROGNOSIS The estimated mortality rate for children with hemorrhagic stroke ranges from 5 to 33 percent, and many studies (largely retrospective) report that neurologic outcomes are poor in approximately 25 to 57 percent of children, as discussed in the sections that follow. Mortality Older studies show that hemorrhagic stroke has a significantly higher mortality than arterial ischemic stroke in children [3,29,84,85] but lower mortality compared with that in https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 15/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate the adult population [86]. In a 2005 report, pooled data from multiple heterogeneous studies suggested an average mortality rate of 25 percent in children with hemorrhagic stroke [87]; later studies reported mortality rates ranging from 5 to 33 percent [8,88-90]. Neurologic outcome Neurologic outcome after hemorrhagic stroke has not been well studied in children. Most data are derived from small retrospective cohort studies or case series. Some data suggest neurologic deficits may persist in up to approximately 75 percent, and disability may be present in more than half of survivors [8,90-92]. As an example, a prospective cohort study of pediatric intracerebral hemorrhage (ICH) included 22 children from a single tertiary care center [8]. At follow-up (median 3.5 months), clinically significant disability (defined as moderate disability or worse, with patients unable to function normally and requiring additional care) was present in 57 percent, and neurologic deficits were present in 71 percent. Scholastic performance is frequently impaired in survivors of ICH [8]. In one cohort including 30 survivors of ICH (age 6 to 17 years), most returned to school within a year of onset, but less than one half were attending age-appropriate classes and the remainder required additional educational support [93]. In a retrospective study of 128 children with childhood stroke, of whom 82 had hemorrhagic stroke, 36 percent required special educational services at long-term follow up (median 43 months) [92]. Epilepsy at two years occurred in 13 percent of children in a prospective study of 53 children with ICH [59]. Elevated intracranial pressure that required urgent intervention during the acute hospitalization was a risk factor for a first remote symptomatic seizure and for developing epilepsy. Children with a diagnosis of epilepsy following stroke have worse parent-reported scores of health status than those without this diagnosis [94]. Outcome predictors In adult ICH, initial hematoma volume is the strongest predictor of mortality and functional outcome, and the level of consciousness at presentation is also an important prognostic factor. The 30-day mortality is approximately 90 percent if the size of the 3 hemorrhage exceeds 60 cm and the Glasgow coma scale (GCS) is <9 at presentation. This 3 compares with an estimated 19 percent mortality when the hemorrhage volume is <30 cm and the GCS is 9 [95]. (See "Spontaneous intracerebral hemorrhage: Acute treatment and prognosis", section on 'Risk factors for poor outcomes'.) Similarly, clinical and imaging features of the acute ICH associated with poor functional outcome in children include [8,90]: ICH volume Altered mental status Length of stay in an intensive care unit https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 16/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate Hemorrhage volume must be taken in the context of percentage of total brain volume (TBV) to account for the markedly varying brain sizes of children of different ages. In a retrospective report of 30 consecutive children, the strongest association with outcome was the intraparenchymal component of ICH expressed as a percentage of TBV; intraparenchymal hemorrhage 4 percent of TBV was independently associated with poor outcome, defined as severe disability or death (odds ratio [OR] 22.5, 95% CI 1.4-354) [56]. The odds of poor outcome 3 at 30 days increased significantly for every 10 cm of additional hemorrhage volume. Other predictors of poor outcome from retrospective studies include initial GCS 8, coagulopathy, and older age (11 to 18 years) [9,96]. Studies in adults suggest that posterior fossa hemorrhage and presence of intraventricular hemorrhage are predictors of poor outcome (see "Spontaneous intracerebral hemorrhage: Acute treatment and prognosis", section on 'Risk factors for poor outcomes'). However, data from small pediatric cohort studies have not confirmed that these factors predict poor outcome in children [8,56,89,97]. Prediction scores Pediatric ICH score The adult ICH score [98] is the most commonly used clinical grading scale for predicting mortality and functional outcome following adult ICH (see "Spontaneous intracerebral hemorrhage: Acute treatment and prognosis", section on 'Clinical prediction scores'). A similar pediatric ICH score was developed to assist with risk stratification in children following ICH. While the pediatric ICH score mirrors its adult counterpart, several components required alterations. Hemorrhage volume was expressed as a percent of TBV to account for the varying brain sizes of children of different ages. Due to the lack of availability of GCS scores in most children, the presence of herniation was used. Isolated intraventricular hemorrhage had not been predictive of outcome in previous studies and was present in about 40 percent of children, so this variable was replaced with hydrocephalus [99]. Thus, the pediatric ICH score is comprised of the following components: Intraparenchymal hemorrhage volume as percentage of TBV - - <2 percent = 0 points 2 to 3.99 percent = 1 point 4 percent = 2 points Hydrocephalus? - No = 0 points Yes = 1 point https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 17/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate Herniation? - No = 0 points Yes = 1 point Infratentorial location? - No = 0 points Yes = 1 point Therefore, the total pediatric ICH score ranges from 0 to 5 points. In one prospective cohort of 60 children with ICH, a pediatric ICH score 2 was sensitive for predicting severe disability or death and a score 1 was sensitive for predicting moderate disability or worse [99]. However, the pediatric ICH score has not been established as generally valid in independent populations. Modified pediatric ICH score The modified pediatric ICH (mPICH) score incorporated early altered mental status, a reported predictor of worse outcome following ICH [8], and intraventricular hemorrhage into the pediatric ICH score to improve prediction sensitivity for moderate or severe disability [100]. The modified pediatric ICH (mPICH) score (range, 0 to 13) is assigns points for presence of six variables as follows: Forebrain herniation, 4 points Altered mental status at initial presentation, 3 points Hydrocephalus, 2 points Infratentorial ICH, 2 points Intraventricular hemorrhage, 1 point ICH volume >2 percent of TBV, 1 point Using a retrospectively selected validation cohort of 43 children, an mPICH score of >4 was sensitive for predicting moderate disability or worse, a score >5 was sensitive for predicting severe disability or worse, and a score >6 was sensitive for predicting vegetative state or death [100]. Hemorrhagic stroke recurrence Data from pooled studies suggest that recurrence risk after hemorrhagic stroke in childhood is approximately 10 percent [87], but the length of follow-up in these studies was highly variable. Limited data suggest that the risk of recurrence depends mainly on etiology; children with untreated or incompletely treated vascular malformations and those with hematologic disorders appear to have the highest risk of recurrence [29,101]. In a population-based retrospective cohort study of 116 children with nontraumatic hemorrhagic stroke in northern California who were followed for a mean of 4.2 years, a recurrent https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 18/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate hemorrhagic stroke affected 11 children at a median of approximately three months (range 7 days to 5.7 years) [64]. The highest risk period was the first six months. The estimated five-year cumulative recurrence rate was 10 percent (95% CI 5-18 percent). Among the 11 recurrent hemorrhagic strokes, there were 5 due to cavernous malformations, 2 caused by to arteriovenous malformation, 2 attributed to tumor, 1 with hypertension, and 1 with idiopathic thrombocytopenia. Among the 9 children with a second hemorrhage and a structural cause (vascular malformation or tumor), the lesion was untreated in 6 and partially treated in 2 (partially resected tumor and second cavernous malformation which was not the cause of first hemorrhage). There were no recurrences among 29 children with idiopathic hemorrhagic stroke. Another study monitored adults and children with brain arteriovenous malformations (AVMs) for a total of 3620 person-years in the adult group and 996 person-years in the childhood group, starting from initial presentation [14]. The unadjusted rates of subsequent ICH were similar for children and adults (2.0 and 2.2 percent, respectively) However, compared with adults, children with AVMs were more likely to present with hemorrhage, and after adjusting for the higher proportion of hemorrhagic presentation in children, the risk of a subsequent ICH was lower for children (hazard ratio 0.1, 95% CI 0.01-0.86). These results suggest that cerebral AVMs in children do not need to be treated more aggressively than those in adults. However, although their annualized risk of hemorrhage is similar to adults, their cumulative risk is greater given their greater number of years left to live. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Stroke in children".) SUMMARY AND RECOMMENDATIONS Classification Hemorrhagic stroke encompasses spontaneous intracerebral hemorrhage (ICH), isolated intraventricular hemorrhage, and nontraumatic subarachnoid hemorrhage. ICH is defined by intraparenchymal hemorrhage with or without associated intraventricular hemorrhage. (See 'Classification' above.) Epidemiology Hemorrhagic stroke accounts for approximately one-half of all childhood strokes. The annual incidence rate is approximately 1 per 100,000 children. (See 'Epidemiology' above.) https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 19/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate Etiologies Hemorrhagic stroke in children living in developed countries is most commonly due to ruptured vascular malformations. Hematologic abnormalities, cancer, and hypertension are less common causes. Aneurysms are the most common cause of nontraumatic subarachnoid hemorrhage. (See 'Etiology and risk factors' above.) Clinical features The most common presenting symptom of hemorrhagic stroke in children is headache. Other common presenting symptoms include nausea and emesis, seizures, neck pain, focal neurologic deficits, and altered level of consciousness. (See 'Clinical features and presentation' above.) Diagnosis The diagnosis of hemorrhagic stroke requires confirmation by brain imaging with computed tomography (CT) or magnetic resonance imaging (MRI). (See 'Urgent neuroimaging' above.) Differential diagnosis and evaluation The differential diagnosis for hemorrhagic stroke includes a broad list of diagnoses that can mimic stroke syndromes ( table 1), with the most common being migraine syndromes and postictal (Todd) paralysis. (See 'Differential diagnosis' above.) Testing to identify underlying causes includes dedicated cerebrovascular imaging and screening laboratory studies. (See 'Identifying the etiology' above.) Management The goals of acute hemorrhagic stroke management include stabilization of the patient, treatment of elevated intracranial pressure (if present), and close monitoring for brain herniation. (See 'Management' above.) We suggest multidisciplinary consultation to choose the optimal endovascular and/or surgical approach of vascular malformations. (See 'Treatment of vascular lesions' above.) Follow-up imaging is warranted in cases where a vascular lesion is suspected but not found during the acute evaluation as well as for most children with hemorrhagic stroke due to a vascular malformation due to the risk of recurrence. (See 'Follow-up imaging' above.) Prognosis The estimated mortality rate for children with hemorrhagic stroke ranges from 5 to 33 percent. Neurologic deficits may persist in up to approximately 75 percent, and disability may be present in more than half of ICH survivors. (See 'Prognosis' above.) Use of UpToDate is subject to the Terms of Use. 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Radiology 2018; 286:651. 101. Nelson MD Jr, Maeder MA, Usner D, et al. Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia 1999; 5:306. Topic 107995 Version 14.0 https://www.uptodate.com/contents/hemorrhagic-stroke-in-children/print 27/33 7/5/23, 12:10 PM Hemorrhagic stroke in children - UpToDate GRAPHICS Hemorrhagic stroke subtypes Hemorrhagic stroke subtypes. (A) Axial head CT demonstrating large left temporal acute IPH (arrows) with surrounding edema and mass ef ventricle (arrowhead) with left to right midline shift. The underlying cause of hemorrhage in this patient was (B) Axial T2/FLAIR MRI sequence showing non-traumatic SAH visible as hyperintense signal within the cerebra right frontal lobe (arrows).