Intracranial Hemorrhage In Children Research Articles

P.125 Cerebral AVM recurrence post gamma knife obliteration: a 20 year single centre retrospective analysis and review of literature

Background: Pediatric arteriovenous malformations (AVMs) are rare, but represent the leading cause of intracranial hemorrhage in children. These are traditionally understood to be congenital lesions, however AVMs recurrence within previously unaffected tissue challenges this understanding. Recurrence after microsurgery and endovascular treatment have been studied in greater detail, but little literature exists surrounding recurrence after Gamma Knife Radiosurgery (GKRS). Methods: We performed a retrospective chart review of all pediatric AVMs treated with GKRS at our centre. Charts were assessed by two reviewers to identify cases of AVM recurrence after angiographically confirmed obliteration. To contextualize our institutional patterns, we also performed a structured literature review of published data reporting pediatric AVM recurrence after GKRS. Results: Our institutional review revealed two cases of AVM recurrence after angiographically proven cure, and our review of literature identified nine retrospective reviews and three case reports, which in total reported 22 individual cases of recurrence. The recurrence rate in the retrospective reviews ranged from 0 to 18%. Conclusions: The current work illustrates that while AVM recurrence is rare, it is a possible complication of GKRS. There was also a qualitative suggestion that embolization prior to CT increased risk of recurrence. Both these facts should be included in decision-making and patient counselling.

Natural history and clinical manifestation of Pediatric Brain Arteriovenous Malformations.

Brain arteriovenous malformations (bAVMs) are aberrant arteriovenous shunts through a vascular nidus with no intervening capillary beds. They are one of the commonest causes of spontaneous intracranial haemorrhage in children and may be associated with significant morbidity and mortality in cases of rupture. Treatment strategies include microsurgical resection, endovascular embolisation, stereotactic radiosurgery, multimodality treatment with a combination thereof, and particularly in high-grade bAVMs, conservative management. Clinicians involved in treating bAVMs need to have familiarity with the natural history pertaining to bAVMs in terms of risk of rupture, risk factors elevating rupture risk as well as understanding the clinical manifestations of bAVMs. This invited review serves to provide a synthesis on natural history and clinical presentation of bAVMs with particular focus in children to inform decision-making pertaining to management.

Computed tomography of intracranial hemorrhages in injured infants and little children aged from 0 months till 3 years

Introduction. The most common reason for young children to seek medical aid in hospitals is head injuries caused by falls from a small height. Currently, computed tomography (CT) of the head is a preferred method for rapid detection of bone fractures and brain injuries in children.Purpose. To investigate specific features of CT signs of intracranial hemorrhages in children with TBI under three years of age.Material and methods. 1334 children aged less than one month to 3 years with isolated TBI were examined at CT scanning. 128-slice scanner "Ingenuity CT" (Philips) was used for the examination. Scanning of the area of interest (head + cervical spine) was performed at the lowest possible values to reduce radiation exposure, including the O-MAR program, with step 0.75 mm at slice thickness 0.75 mm; reconstruction interval was 2 mm. The voltage applied to an X-ray tube during scanning (kV), current strength and time (mAS) were selected depending on patient's weight and age. The effective dose range was from 1.27 mSv to 1.91 mSv.Results. In 510 out of 1334 injured children (38.2%), there were traumatic injuries of various degree, from uncomplicated cephalohematomas and linear fractures to massive intracranial hematomas and total cerebral edema; the rest 61.8% (n=824) had concussion. The performed CT scanning revealed that 87.84% (448/510) children had skull fractures, of which only 18.3% (82/448) had “isolated skull fractures”; the others (366 = 81.7%) had accompanying intracranial injuries.Discussion. Pathological changes in children, aged 0 mon-3 years, after TBI are significantly different of those in children of other age groups. CT is the basic primary diagnostic instrument and should be used in all children with TBI no later than the first three hours. Radiation diagnostics play a key role in putting a correct diagnosis, if physicians use the information obtained at CT and know TBI mechanism in infants and little children.Conclusion. CT is an imaging method of choice for acute TBI in little children to accurately identify and therefore treat intracranial lesions. In addition, CT is an effective diagnostic tool in detecting secondary traumatic injuries.

Microsurgical resection of a ruptured intraventricular arteriovenous malformation in a neonate: considerations in management. Illustrative case.

Arteriovenous malformations (AVMs) are the most common cause of intracranial hemorrhage in children, although they are rarer in neonates. Age, location, lesion architecture, and rupture status define treatment options. Sparse literature exists to guide the management of clinically symptomatic intraventricular AVM rupture in neonates. We highlight the case of a neonate with a ruptured intraventricular AVM to showcase considerations in treatment, discuss surgical technique, and help guide management. An 18-day-old female presented with lethargy in extremis and was found to have new intraventricular hemorrhage. Angiogram revealed a Spetzler-Martin grade 2 AVM with a right posterior choroidal feeder and deep venous drainage within the ventricle. Her age limited radiosurgical and endovascular interventions. She underwent an interhemispheric, transcollosal, intraventricular approach for complete AVM resection. Perioperative care was managed by a multidisciplinary team, successfully mitigating the patient's high risk of hemovascular collapse. Stereotactic radiosurgery, endovascular embolization, and microsurgery are options for AVM obliteration, and multimodal therapy must be tailored to the lesion and patient. Conservative management can also be considered. Each intervention carries risks and varying likelihoods of success. Balancing these outcomes is challenging without definitive, high-quality, evidence-based guidance. The best treatment maximizes the chance of AVM obliteration while minimizing morbidity.

Twenty years' experience of immune thrombocytopenia and intracranial haemorrhage in the Nordic countries-A NOPHO study.

Twenty years' experience of immune thrombocytopenia and intracranial haemorrhage in the Nordic countries-A NOPHO study.

Intracranial hemorrhage in children with hemophilia

Aim: Hemophilias are inherited bleeding disorders, in which the patients generally present with clinical complaints of hemarthrosis. Intracranial hemorrhage (ICH) is one of the severe bleeding types with the highest mortality and morbidity throughout childhood, as well as in patients with a diagnosis of hemophilia. Herein, a single-center experience of intracranial hemorrhage in children with hemophilia is presented.
 Materials and Methods: The files and hospital records of the patients with the diagnosis of hemophilia who were followed up by the Pediatric Hematology and Oncology Department of Erciyes University between the years 1993-2022 were evaluated retrospectively.
 Results: A total of 81 patients with hemophilia were evaluated. Among them, 9 patients developed ICH. All patients had severe diseases. The mean age of incidence ICH was 2,6 months (4 days- 8,7 months). All the ICH episodes were observed within the first year of life. Four patients were diagnosed with the ICH episode initially. None of the patients had primary prophylaxis. The majority of them were admitted with neurological signs and symptoms. There was no ICH-related mortality in our study and all of the patients are being followed up in our department.
 Conclusion: Intracranial hemorrhage remains important in patients with congenital bleeding disorders, especially in hemophilia; with difficulties in diagnosis, management, and treatment.

739: DECOMPRESSIVE CRANIECTOMY IN CHILDREN WITH TRAUMATIC BRAIN INJURY IN THE U.S. FROM 2000 TO 2019

Introduction: The role of decompressive craniectomy (DC) in children with TBI and intracranial hemorrhage (ICH) or refractory raised intracranial pressure (ICP) is undetermined. This study aimed to describe the prevalence of DC and outcomes associated with DC and ICH in children with TBI. Methods: A retrospective analysis of Kids’ Inpatient Database from 2000 to 2019 was performed. Hospitalized children from 28 days to 20 years were selected. ICD 9 and ICD 10 codes were used to identify diagnosis and procedure variables. Data were compared between early DC (procedure day = 0) and late DC (procedure day >0) using chi-square and Mann-Whitney U tests. Multivariable analysis was performed to determine the adjusted odds of mortality after early vs late DC and various types of ICH and raised ICP. Linear trends in the prevalence of DC were analyzed using the Extended Mantel-Haenszel test. Results: Among 23,804,204 hospitalized children, 193,456 children had TBI (8.1 per 1000 discharges). There was a decreasing trend in TBI hospitalization (p< 0.001) and associated mortality (p < 0.001). DC was documented in 9,887 (5.1%). DC procedure day was documented in 8,480 with early DC in 67.1% and late DC in 32.9%. In patients with TBI and DC, epidural hemorrhage (EH) was present in 19.6%, subdural hemorrhage (SH) in 11.4%, subarachnoid hemorrhage (SAH) in 5.1%, multiple hemorrhage in 51.8%, any hemorrhage in 83.5%, and raised ICP (presence of cerebral edema or EVD) in 6.6%. Early DC was more common in children with EH (OR=1.5; 95%CI:1.4-1.7) and late DC was common in SH (OR=0.7; 95%CI:0.6-0.8), SAH (OR=0.6; 95%CI:0.5-0.7) and raised ICP (OR=0.54; 95%CI:0.48-0.61). The overall mortality and morbidity associated with TBI children with DC was 36.6% and was higher with late DC (OR:1.59; 95%CI: 1.45-1.74). On multivariable regression analysis, morbidity-mortality rate was higher in raised ICP (aOR=6.5; 95%CI:5.5-7.6), SAH (aOR=3.0; 95%CI: 3.3-3.8), SH (aOR=1.9; 95%CI:1.6-2.2), and lower with early DC (aOR= 0.8; 95%CI:0.7-0.9), and EH (aOR=0.27; 95%CI:0.22-0.31). Conclusions: In our national study, in children with TBI, 5% underwent DC. ICH was present in over 80% of children who had DC. The morbidity-mortality was lower with early DC and EH, and higher with raised ICP, SAH, SH and late DC.

Short-term intermittent prophylaxis post-intracranial hemorrhage in children with hemophilia

Background Intracranial hemorrhage (ICH) is one of the major bleeding events causing mortality and long-term morbidity in children with hemophilia, especially those who receive on-demand therapy.&#x0D; Objective To evaluate the outcome of children with hemophilia after ICH receiving short-term intermittent prophylactic treatment.&#x0D; Methods This retrospective study was conducted in the Department of Child Health, Dr. Cipto Mangunkusumo Hospital, Jakarta. Children £18 years of age with hemophilia presenting with ICH between 2015-2020 were included. We recorded patients’ demographics, type and severity of hemophilia, the presence of factor VIII (FVIII) inhibitor, brain CT scan, treatment, and outcomes of these patients. Patients who received short-term intermittent prophylaxis using clotting factor concentrate (CFC) post-ICH episodes were observed for ICH recurrence.&#x0D; Results There were 19 episodes of ICH experienced by 18 patients, consisting of 16 patients with hemophilia A and 2 with hemophilia B. Patients’ median age was 4 years (range 0-16 years). Hemophilia was classified as severe in 13 patients, moderate in 4 patients, and mild in 1 patient. Thirteen episodes were preceded by head trauma. The most common clinical manifestation was seizures (13.2%). The most common type of ICH was subdural hematoma. Two patients died and 2 patients had neurological sequelae during hospitalization. The median dose of short-term intermittent prophylaxis using CFC (n=16) was 20 IU/kg of FVIII twice a week and 30 IU/kg of FIX twice a week, for a median duration of 8 weeks (range 5-12 weeks). One patient who did not adhere to the prophylaxis regimen had recurrent ICH at a similar location 6 months after the first episode. &#x0D; Conclusion Our findings suggest that short-term intermittent prophylaxis is important to prevent the recurrence of ICH in children with hemophilia.

Pediatric hepatobiliary disease: Genetic and clinical manifestations

In children, hepatobiliary diseases are rarely and mainly due to congenital defects during formation, development of the liver and biliary tract or due to disorders of metabolism. The liver and biliary tract of infant have the incomplete development of physiology during the perinatal until the childhood period. In the process of complete development of the child's liver and biliary tract, there are important changes and affected by genetic and environmental factors. Therefore, the liver and biliary tract are very vulnerable leading to hepatobiliary diseases in children. Disorders in the formation of bile ducts, bile secretion, hepatocellular metabolism, disturbances of metabolism all lead to the formation associated with hepatobiliary diseases. Based on pathophysiology, the hepatobiliary diseases in children can be divided into two groups: hepatobiliary diseases due to the incomplete development of the structure and function of the liver and biliary tract, and hepatobiliary diseases due to the disruption of metabolic processes in liver cells. The secondary effects of hepatobiliary disease can threaten a child's life, and is the cause of metabolic disorders such as hypoglycemia, secondary coagulation disorder due to low concentration of vitamin K-dependent factors leading to intracranial hemorrhage in children, infections caused by immunodeficiency, malnutrition, increased portal venous pressure leading to severe gastrointestinal bleeding... Therefore, the hepatobiliary disease in children should be detected and treated early to avoid adverse complications. In the context of this paper, we focus on hepatobiliary diseases with genetic causes in children. Genetic factors and research situation in the World and Vietnam will be also mentioned. The information about genetic and clinical manifestations will be aggregated to contribute to the general understanding of hepatobiliary diseases in children and to orientate the accurate and effective treatment for patients.

Premedication with Hydrocortisone Increases Risk of Adverse Drug Events and Return to Medical Care Among Pediatric Patients with ITP Treated with IVIG

Immune Thrombocytopenia (ITP) is one of the most common acquired bleeding disorders affecting children. While 75-80% of these children will develop only mild bleeding symptoms, others can experience more severe bleeding, including rare intracranial hemorrhage (ICH), and may require platelet-directed therapy. Intravenous immunoglobulin (IVIG), a commonly utilized and effective first-line therapy, may be associated with adverse drug events (ADEs). The most commonly reported ADEs include headache (approximately 30% of treated patients) and nausea/vomiting, however the exact rates of these events and relationship to premedication strategies are poorly classified. These ADEs often result in emergent medical evaluation and additional diagnostic testing, including computed tomography (CT) of the head, to evaluate for ICH in children with severe thrombocytopenia.In order to identify rates of IVIG-associated ADEs and subsequent emergent medical evaluations in pediatric patients with ITP, we completed an retrospective observational cohort study of all pediatric patients with ITP who were treated with one-time IVIG infusion utilizing Gamunex-C ® 10% at a dose of 1g/kg across 4 large pediatric centers from 2010 to 2019. Included patients were between the ages of 0 and 21 years.Among the 563 patients receiving single IVIG infusions across all 4 centers, patients reported headache, nausea/vomiting, or both following 203 infusions (36%). Excluding those patients who remained hospitalized at the time of symptom development, 16% (84 of 513) of all patients returned for urgent medical evaluation following IVIG infusion. Nine percent of all patients receiving a single IVIG infusion underwent head CT to rule out ICH. All 51 CTs were negative for ICH.In order to determine if specific patient characteristics were associated with the development of IVIG-associated ADEs and return to medical care, univariable logistic regression was performed. Multiple logistic regression was used to determine whether the associations identified in the univariable analysis persisted after adjusting for other significant factors. Older age at administration (Adjusted Odds Ratio [adj OR] 1.07 for each additional year of age; 95% Confidence Interval [CI] 1.03, 1.11; p<0.001) and premedication with hydrocortisone (adj OR 2.36; 95% CI 1.38, 4.07; p=0.002) were associated with increased odds of IVIG-associated ADEs. Patients who received premedication with hydrocortisone were more likely to re-present for medical evaluation than patients who did not receive hydrocortisone (OR 1.90; 95% CI 0.98, 3.54; p=0.049). No associations were identified between ADEs and gender, race/ethnicity, premedication with diphenhydramine or ondansetron, pre-treatment serum IgG levels, or concurrent glucocorticoid administration.In summary, the rate of ADEs among pediatric patients with ITP treated with IVIG is remarkably high. Thirty-six percent of patients in our multi-institutional cohort reported headache, nausea/vomiting, or both following single IVIG infusion, consistent with our previous single institution experience. This again highlights the need for proper balance of risks and benefits when considering treatment for children with ITP. Patients who received premedication with acetaminophen were less likely to develop IVIG-associated ADEs, but those who received premedication with diphenhydramine or concurrent glucocorticoid therapy were no more or less likely to develop ADEs. Interestingly, however, patients who received premedication with hydrocortisone were more likely to develop IVIG-associated ADEs. Despite only a small subset of our cohort being premedicated with hydrocortisone (12%, n=66), this premedication strategy was significantly associated with an increased rate of return to medical care following IVIG infusion. Patients returning for emergent medical evaluation frequently underwent repeat laboratory studies or CT imaging, resulting in unnecessary radiation exposure and increased healthcare-related costs. These findings will help to inform IVIG premedication strategies in an attempt to reduce both ADEs and re-presentation to medical care among pediatric patients with ITP receiving IVIG therapy. [Display omitted] DisclosuresKirk: Biomarin: Honoraria. Grace: Novartis: Research Funding; Principia: Membership on an entity's Board of Directors or advisory committees; Dova: Membership on an entity's Board of Directors or advisory committees, Research Funding; Agios: Research Funding. Neunert: Novartis: Research Funding. Lambert: Bayer: Consultancy; ClinGen, ISTH, ASH, GW University: Honoraria; Novartis: Consultancy, Honoraria, Research Funding; Astra Zeneca: Research Funding; Argenx: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Principia: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Dova: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; Shionogi: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Rigel: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding; PDSA: Research Funding; Sysmex: Research Funding; Octapharma: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Despotovic: UpToDate: Patents & Royalties: Royalties; Apellis: Consultancy; Novartis: Consultancy, Research Funding; Agios: Consultancy.

Intracranial hemorrhages in children with immune thrombocytopenia

Primary immune thrombocytopenia is a benign and self-limiting process in the majority of children. Severe life-threatening hemorrhages, including intracranial, develop rarely. Risk factors predisposing for development of severe hemorrhagic complications have not been determined. In order to decrease the severity of neurological consequences and mortality in intracranial hemorrhages, timely combined urgent therapy is neсessary. There are four clinical cases of intracranial hemorrhage in immune thrombocytopenia in children with different outcomes in this article. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.

Endovascular embolization for cases of 'hidden' pediatric cerebral arteriovenous malformations: A diagnostic & therapeutic challenge.

BackgroundRuptured arteriovenous malformations (AVM) hold a larger proportion as the cause of spontaneous intracranial hemorrhage in children compared to those in adults. Although surgical excision still remains as the gold standard therapy for arteriovenous malformations, some smaller ones are reported to resolve from embolization alone. However, difficulty arises when small arteriovenous malformations are not detected on certain diagnostic modalities such as Computed Tomography Angiography (CTA), giving rise to false negatives, which may compromise appropriate management of patients. Endovascular embolization can be used as alternative options as diagnostic and therapy for invisible arteriovenous malformation in children. We report two cases of ruptured paediatrics arteriovenous malformations with a complication of hydrocephalus, managed with endovascular embolization and a cerebrospinal fluid diversionary procedure.Case descriptionWe report 2 case in from Dr. Soetomo academic general hospital in 2021, the first case was a fully conscious 6-year-old-female child with sudden left-sided weakness and severe headache in January, and the second case a 9-year-old female came with decreased consciousness in May. Both had evidence of intracerebral hemorrhage, intraventricular hemorrhage, and hydrocephalus on head radiological examination, but no visible vascular malformations on Computed Tomography Angiography. The first patient was treated with extra ventricular drainage initially, while the second case was not. Transfemoral cerebral angiography revealed small arteriovenous malformations in both patients, and both had successful endovascular embolization afterwards. The first case was shunt-free, while the second case had her drainage switched to ventriculoperitoneal shunt right after the embolization procedure. Both patients recovered fully without complications and sequelae, and were discharged afterwards.DiscussionBoth patients did not undergo surgical resection of the arteriovenous malformations; the first case only underwent endovascular embolization, while the second case underwent embolization and ventriculoperitoneal shunting. The cases described here help highlight the irreplaceable role of Transfemoral Cerebral Angiography as a gold standard for cases for arteriovenous malformations compared to other modalities, such as Computed Tomography Angiography (CTA). Smaller arteriovenous malformations in paediatrics are reported to achieve complete radiological resolution, and cerebrospinal fluid diversion in hydrocephalic cases are not always performed. Several factors to be considered include initial consciousness and severity of neurological deficit, which were taken into account in the management of our patients.ConclusionEmbolization procedures may be beneficial in some pediatric arteriovenous malformations, preferably in smaller ones that undetectable by angiography. Several factors such as the consciousness and neurological deficit upon initial presentation may help in the decision making of these cases.

Intracranial Hemorrhage in Children with Inherited Bleeding Disorders: A Single Center Study in China

Introduction: Intracranial hemorrhage (ICH) is a life threatening condition in children. Inherited bleeding disorders may(IBD) cause ICHAim: This single center study aim to identify the incidence, risk factors, and neurological outcome in children with IBDMethod: From 2005 to 2016, 240 children with inherited bleeding disorders hospitalized in Nanfang hospital, department of pediatrics, were evaluated. The ICH episodes were identified by medical history, general physical examination, detailed neurological examination, and CT or MRI exam. The risk factors, location of ICH, management strategies, surgical intervention and outcome were noted.Results: ICH was confirmed in 54/240 children with IBD. The overall risk of ICH among children with IBD was 22.5% (95% CI: 17.2, 27.8%). 52/54 (96.2%) (95% CI: 91.1, 99.9%) was hemophilic patients. The mean age of patients was 53 months and 18/54 (33.3%) (95% CI: 20.3, 46.3%) had an ICH in the first year of life. 6/52(11.5%) (95% CI: 2.6, 20.5%) hemophilic children had multiple episodes of ICH in which 4 were inhibitor positive.9/52 (17.3%) (95% CI: 6.7, 27.9%) hemophilic children were inhibitor positive while in 24/52(46.1%) (95% CI: 32.1, 60.2%) children the inhibitor was not assessed. 22(36%) (95% CI: 23.7, 48.5%) out of 61 ICH episodes were caused by trauma and 39(63.9%) (95% CI: 51.5, 76.3%) were non-trauma related. Subdural hematoma was most frequently observed. Mortality risk from ICH in children with IBD was 5/54(9.2%) (95% CI: 1.3, 17.2%). 11(22.4%) (95% CI: 10.3, 34.6%) of 49 survivors had known neurological sequelae while 38(77.5%) (95% CI: 65.4, 89.7%) had no documented evidence of neurological impairment.Conclusion: Hemophilia is the most common primary disease of ICH in children in our center. Risk and consequences of ICH in IBD were high during the first year of life while in older children better outcome may be expected. The optimal management of ICH depends on immediate recognition and prompt replacement therapy. DisclosuresNo relevant conflicts of interest to declare.

Intracranial hemorrhage in primary immune thrombocytopenia (ITP): 20years' experience in pediatrics.

Incidence of intracranial hemorrhage (ICH) among children with primary immune thrombocytopenia (ITP) varies among different studies. We published data during the period of 1997-2007 of ICH in children with primary ITP, addressing risk factors and outcome. The aim of this study is to assess changes in incidence, risk factors, and outcome of ICH in children with ITP from last decade and to report the overall 20years' experience. We compared 2008-2018 with the decade before it. Data of children with ITP and ICH during study period and ITP control cases were analyzed. Neurosurgical intervention and outcome were also reported. A total of 4340 children with primary ITP were evaluated. Twenty-five (0.63%) ICH events were reported over 2 decades. Head trauma, hematuria, and platelet counts < 10 × 109/L were the risk factors mostly associated with ICH. Overall mortality was 24%, and a further 28% had neurologic sequelae. Neurosurgical intervention was done in 12% of cases with good outcome.Conclusion: Persistent platelet counts < 10 × 109/L were a significant risk factor for ICH in both time periods, while head trauma and hematuria were more reported in the period of 2008-2018 as significant risk factors for ICH. Outcome was comparable in both periods. What is Known: • ICH is a rare complication of ITP; however, early recognition of risk factors and aggressive treatment might lead to complete recovery without sequalae. Platelet counts less than < 10 × 109/L are the main risk factor for ICH. Few studies reported other significant risk factors. What is New: • Hematuria and head trauma are significant risk factors for ICH in ITP, in addition to having a persistently low platelet count < 10 × 109/L. (more than 90days in chronic ITP, 45days in persistent and 21days in acute ITP) • Combined treatment with IVIG and HDMP followed by platelet transfusion was associated with complete recovery without sequelae in almost 50% of patients.

Risk Factors of Venoarterial Extracorporeal Membrane Oxygenation-Related Intracranial Hemorrhage in Children with Congenital Heart Disease.

To analyze the risk factors for intracranial hemorrhage (ICH) in congenital heart disease (CHD) patients supported with venoarterial extracorporeal membrane oxygenation (VA-ECMO). We performed a retrospective analysis of VA ECMO patients after open heart surgery in Shanghai Children's Medical Center from February 2017 to December 2018, with a total of 50 patients: 14 patients in the ICH group and 36 patients in the control group. Clinical data were analyzed and compared between groups to search for risk factors for ICH. The overall incidence of ICH was 28% (14/50). The in-hospital mortality rate of the ICH group was 57.1% (8/14) vs. 58.3% (21/36) in the control group. The proportion of neonates in the ICH group was 64.3% (9/14) vs. 25% in the control group (9/36) (p = 0.009), and the ICH incidence in extracorporeal cardiopulmonary resuscitation (ECPR) patients was 64.3% (9/14) vs. 13.9% (5/36) (p = 0.000). The percentage of selective cerebral perfusion in the ICH group was 64.3% (9/14) vs. 16.7% (6/36) (p = 0.001) in the control group, and the maximum procalcitonin (PCT) was 43.70 ± 30.48 ng/ml in the ICH group versus 26.92 ± 23.28 ng/ml (p = 0.050) in the control group. Multivariate analysis showed that neonates (odds ratio [OR] = 6.47 [1.09-38.46]), ECPR use (OR = 7.48 [1.26-44.41]), and maximum PCT values (OR = 1.04 [1.001-1.070]) were independent risk factors for ICH. The probability of ICH remains high in children supported with VA-ECMO after cardiac surgery. Neonatal patients, ECPR use, and PCT peak values are independent risk factors for ICH.

The CTSC-RAB38 Fusion Transcript Is Associated With the Risk of Hemorrhage in Brain Arteriovenous Malformations.

Brain arteriovenous malformations (bAVMs) are congenital anomalies of blood vessels that cause intracranial hemorrhage in children and young adults. Chromosomal rearrangements and fusion genes play an important role in tumor pathogenesis, though the role of fusion genes in bAVM pathophysiological processes is unclear. The aim of this study was to identify fusion transcripts in bAVMs and analyze their effects. To identify fusion transcripts associated with bAVM, RNA sequencing was performed on 73 samples, including 66 bAVM and 7 normal cerebrovascular samples, followed by STAR-Fusion analysis. Reverse transcription polymerase chain reaction and Sanger sequencing were applied to verify fusion transcripts. Functional pathway analysis was performed to identify potential effects of different fusion types. A total of 21 fusion transcripts were detected. Cathepsin C (CTSC)-Ras-Related Protein Rab-38 (RAB38) was the most common fusion and was detected in 10 of 66 (15%) bAVM samples. In CTSC-RAB38 fusion-positive samples, CTSC and RAB38 expression was significantly increased and activated immune/inflammatory signaling. Clinically, CTSC-RAB38 fusion bAVM cases had a higher hemorrhage rate than non-CTSC-RAB38 bAVM cases (p < 0.05). Our study identified recurrent CTSC-RAB38 fusion transcripts in bAVMs, which may be associated with bAVM hemorrhage by promoting immune/inflammatory signaling.

Risk of traumatic intracranial haemorrhage in children with bleeding disorders.

To assess computerised tomography (CT) use and the risk of intracranial haemorrhage (ICH) in children with bleeding disorders following a head trauma. Design: Multicentre prospective observational study. 10 paediatric emergency departments (ED) in Australia and New Zealand. Children <18 years with and without bleeding disorders assessed in ED following head trauma between April 2011 and November 2014. Data collection of patient characteristics, management and outcomes. Rate of CT use and frequency of ICH on CT. Of 20 137 patients overall, 103 (0.5%) had a congenital or acquired bleeding disorder. CT use was higher in these patients compared with children without bleeding disorders (30.1 vs. 10.4%; rate ratio 2.91 95% CI 2.16-3.91). Only one of 31 (3.2%) children who underwent CT in the ED had an ICH. This patient rapidly deteriorated in the ED on arrival and required neurosurgery. None of the patients with bleeding disorders who did not have a CT obtained in the ED or had an initial negative CT had evidence of ICH on follow up. Although children with a bleeding disorder and a head trauma more often received a CT scan in the ED, their risk of ICH seemed low and appeared associated with post-traumatic clinical findings. Selective CT use combined with observation may be cautiously considered in these children based on clinical presentation and severity of bleeding disorder.

Pediatric intracranial arteriovenous malformations: a single-center experience

OBJECTIVEIntracranial arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children. Exploring outcomes of treatment and factors that predict recurrence may help guide the management of pediatric AVMs. In this study, the authors review a single-center experience with pediatric AVMs, aiming to identify factors contributing to recurrence and areas for improvement in treatment.METHODSA single-center retrospective review of patients ages 0–18 years with AVMs in the period from 2005 to 2018 was conducted. Demographic, clinical, and radiographic data were collected and examined to compare the clinical and functional outcomes of patients managed with and those managed without open microsurgical resection.RESULTSOne hundred five patients were diagnosed and treated during the study period. Fifty (47.6%) had been treated surgically and 35 (33.3%) nonsurgically, and 20 (19%) had been treated primarily at an outside institution and thus were excluded from further analysis. Of the 50 treated surgically, 92% had complete obliteration on postoperative imaging, with the remaining patients having residual AVM and undergoing re-resection or SRS, ultimately resulting in a 96% radiographic cure rate. The surgically treated patients were followed up for an average of 3.34 years. Eighty-two percent had a modified Rankin Scale (mRS) score of 0–2. There was a recurrence rate of 12%, with the time to recurrence averaging 327.86 days (range 85–596 days). Of the 35 patients managed without open surgery, 77% underwent serial imaging surveillance, 20% underwent endovascular embolization, and 3% underwent stereotactic radiosurgery. Radiographic cure was seen in 6% of those treated nonsurgically, and all had undergone endovascular treatment alone. The nonsurgically treated patients were followed up for an average of 2.14 years. Eighty percent had an mRS score of 0–2.CONCLUSIONSThe data revealed that patients with lower-grade AVMs treated surgically and those with higher-grade AVMs managed without surgery have good functional and clinical outcomes. A high rate of recurrence was noted, and the possible contributing factors were explored. The authors encourage further study into mechanisms to reduce AVM recurrence in pediatric patients and look forward to advances in the medical management of lesions that are considered unresectable.

Surgical strategies for management of pediatric arteriovenous malformation rupture: the role of initial decompressive craniectomy.

Arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children, which can result in elevated intracranial pressure (ICP) and cerebral edema. We sought to explore the role of initial decompressive craniectomy at time of rupture, followed by interval surgical AVM resection, compared to treatment with initial resection, in clinical outcomes and recovery in children. A retrospective chart review was conducted examining patients age 0-18 with AVM rupture between 2005 and 2018 who underwent resection for ruptured AVM either initially at presentation or underwent initial decompressive craniectomy followed by interval AVM resection. Clinical, radiographic, surgical, and outcome data were examined. Primary outcomes measured included functional status, AVM obliteration rate, AVM recurrence/residual, and re-hemorrhage. Thirty-six cases were included; 28 (77.8%) underwent initial AVM resection, and 7 (19.4%) underwent initial decompressive craniectomy with interval resection. The mean time between craniectomy and resection was 66.9days (SD 59.3). Patients undergoing initial decompressive craniectomy with interval resection were younger (mean age 6.1 vs. 9.8years, p = 0.05) and had a higher mean hematoma volume (52.9 vs. 22.2mL, p = 0.01), mean midline shift (5.1 vs. 2.1mm, p = 0.01), and presence of cisternal effacement (p = 0.01). There were no statistically significant associations between surgical strategy and postoperative outcomes, including complications, radiographic outcomes, complete resection, residual, recurrence, and functional outcomes. Those treated by initial craniectomy followed by interval resection were associated with undergoing additional procedures. Children presenting with AVM rupture who require emergent decompression may safely undergo emergent craniectomy with interval AVM resection and cranioplasty without additional risk of morbidity or mortality. This is reasonable in those with elevated intracranial pressure. This strategy may provide time for initial recovery and allow for natural degradation of the hematoma enhancing the plane for interval AVM resection, perhaps improving outcomes.

Intracranial Hemorrhage in Egyptian Children with Primary Immune Thrombocytopenia (ITP): Report of 24 Cases in 20 Years

Background: 35 million children &lt; 18 years; approximately 1800 new cases of ITP were diagnosed annually in Egypt. Intracranial hemorrhage (ICH) is a rare devastating complication of childhood immune thrombocytopenia (ITP). Incidence of ICH among children with ITP varies markedly in different studies from 0.2 up to 1.0 %. Intracranial hemorrhage after head trauma in children with ITP leads to significant morbidity and mortality. We published data during 1997 - 2007 (10 ICH) in children with primary ITP; risk factors and outcome. Aim &amp; Methodology: A follow-up study to assess any change in outcome of ICH from last decade; whether new therapies might change the landscape of ICH. Centers treating &gt; 25 child with prim ITP /year and offered a complete data of &gt;150 children with ITP over 2 decades were enrolled. We compared 2008 - 2018 with the decade before it; variation of ICH reporting from center to center and outcome in relation to therapy. All children with ITP and ICH during study period had been treated, within &lt; 24 hours and referred to neurosurgical hospital complex facility for consultation and intervention. Time elapsed till receiving platelet enhancing therapy and neurosurgical intervention was assessed, Outcome whether a complete recovery, permanent sequalae or death was reported. Results: Four thousand, three-hundred and forty primary ITP were evaluated, ( 380 were excluded due to incomplete data ) Twenty-four (0.6%) ICH were reported over 2 decades (14 in this decade with 20% increase incidence) and 48 matched ITP control subjects were evaluated. Platelet counts were less than 10 x 10(9)/L in 90% of children with ICH. Four (16.2%) children developed ICH within 14 days of diagnosis of ITP; one of these, was the presenting feature of ITP. four were from 3-12 months and sixteen (66.6%) of children had chronic ITP. Centers treating &gt; 50 case/year had a higher frequency of reporting 0.8 % compared to 0.2 % in centers &lt; 50 cases/year. Outcome is better on early intervention as well as aggressive platelet enhancing therapy with 70% complete recovery compared with 30% complete recovery on delayed intervention. Head trauma and hematuria and PC &lt; 10 were the mostly associated with ICH, identified in 33%, 25% and 90% respectively of the patients with ICH and in 1, none and 50% of the controls (P &lt; .001). Bleeding beyond petechiae and ecchymoses was also linked to ICH. Mortality was 25%; a further 25% had neurologic sequelae. Neurosurgical intervention was done in 25% of cases with good outcome. Reporting was more in this decade with better outcome in bigger centers. Conclusion: A rise in the incidence of ICH in Children with severe thrombocytopenia over last decade; high risk for ICH among those with PC&lt; 10,000 plus head trauma and/or hematuria. Platelet enhancing agents whether HDMP or IVIG or TPO-RAs could not prevent ICH. However they had a good impact on survival and lessen sequalae if used in combination. Strategies by which high-risk children could be identified and well managed in small centers. Disclosures No relevant conflicts of interest to declare.