Arteriovenous Malformations In Children Research Articles

Brain and lung arteriovenous malformation rescreening practices for children and adults with hereditary hemorrhagic telangiectasia

BackgroundPatients with hereditary hemorrhagic telangiectasia (HHT) are at risk for organ vascular malformations including arteriovenous malformations (AVMs) in the brain and lungs. North American HHT Centers of Excellence (CoEs) routinely screen for brain and lung AVMs, with the primary goal of detecting AVMs which can be treated before complications arise. Current international HHT guidelines provide recommendations for initial screening for brain and lung AVMs among children and adults with the disease, but rescreening recommendations are not comprehensively addressed and have not been reported. We determined current rescreening practices for brain and lung AVMs for children and adults with HHT among North American HHT CoEs.MethodsWe surveyed North American HHT CoEs regarding rescreening practices for new brain and lung AVMs in children and adults with initial negative screening.ResultsAll thirty CoEs responded; 28 regarding pediatric (93.3%) and 30 (100%) regarding adult HHT care. The median duration of practice experience in HHT was 11.5 (range 3–30) years for providers of pediatric HHT care and 11.5 (range 3–35) years for providers of adult HHT care. The median number of patients followed at each CoE was 60 for children (range 8–500) and 375 for adults (range 30–1500). 25/28 CoEs (89.3%) reported rescreening children for brain AVMs, most commonly with enhanced MRI (21/25, 84%). 25 CoEs rescreen children for lung AVMs, most commonly every 5 years (15/25). Only 4/30 CoEs (13.3%) rescreen adults for brain AVMs. 26/30 CoEs (86.7%) reported rescreening adults for lung AVMs, most commonly every 5 years (18/26, 69.2%).ConclusionsMost HHT CoEs routinely rescreen children for brain and lung AVMs and adults for lung AVMs when initial screening is negative, but adults are infrequently rescreened for brain AVMs. Long-term data regarding risk for new brain and lung AVMs are required to establish practice guidelines for rescreening.

Surgical Management of a Thalamic Arteriovenous Malformation in a Young Male in a Low Resource Setting: An Illustrative Case Report

Introduction: The natural history of patients with arteriovenous malformations (AVMs) suggests that 10-17% die due to hemorrhage and 40-50% experience serious physical deterioration of their working capacity in their 20-40-year period after presentation and treatment. Deep basal ganglia and thalamus lesions account for 3-12% of all AVMs and some literature suggests that these lesions have a more hostile clinical course. Case Presentation: 16-year-old male, a student with no known chronic illnesses came to our hospital as a referral from Lubaga hospital. He presented with fifteen days’ history of sudden onset of severe headache with no known relieving or exacerbating factors. This was associated with episodes of vomiting, altered mentation, right sided weakness, however there was no history of convulsions, blurring of vision or trauma. This was the first time for this kind of presentation. On admission: He was lethargic, moderately dehydrated, not pale nor jaundiced. BP was 109/52mmHg, HR of 60bpm, SPO2=96% on room air. Glasgow Coma Scale score of 14/15, V=4, PEAL, soft neck, normal muscle bulky with a power of 2/5 on the left side and 4/5 on the right side. Successful surgical resection of the AVM was done at Mbarara regional referral Hospital-Uganda. Currently the patient is back in the community and has returned to school. Conclusion: Children who present with intracranial hemorrhage from a previously undiagnosed AVM have a 12% chance of sudden death. Clinical triggers of hemorrhage are unpredictable, and all fatal cases were in locations with high risk of potential herniation. These data support a proactive, aggressive approach toward definitive treatment of AVMs in children and adults. Patients with diagnosed AVM can once again get a chance to survive like other children in the community in LMICs like Uganda by undergoing open surgery, although advanced opportunities are still limited.

Hypofractionated stereotactic radiotherapy in Spetzler Martin grades 4 and 5 arteriovenous malformations in the pediatric population: Is it a viable alternative?

To analyze the outcomes of hypofractionated stereotactic radiotherapy (HFSRT) for Spetzler Martin grades 4 and 5 arteriovenous malformations (AVMs) in a pediatric population. Fourteen patients with Spetzler Martin (SM) grades IV and V large AVMs who underwent HFSRT between January 2013 and July 2019 were retrospectively reviewed. All patients received HFSRT at a dose of 30-36Gy in 5 to 6 fractions. They were followed up annually with clinical and imaging assessments to evaluate obliteration rates. The median age at presentation was 15years (range 8-21years). Ten (71%) were SM grade 4 AVMs and the rest were SM grade 5 AVMs. The majority presented with headache (8 [57%]), and 3 (21%) presented with bleeding. The median nidus volume was 39.4cc (IQR, 31.4-52.4). Two (14%) patients had infratentorial AVMs. All of them had deep venous drainage. The median clinical follow-up duration was 75months (range 31-107months). There was complete obliteration of the nidus in 3 (21%) patients with a median time to obliteration of 39months. HFSRT resulted in a reduction of the AVM volume to 12cc or less in nearly 70% of patients. None of the patients experienced re-bleeding. 79% reported an improvement in their symptoms. HFSRT is a highly effective treatment for high-grade AVMs in children, which can result in either complete elimination or significant reduction of the nidus volume or make it suitable for additional treatment, such as single-session stereotactic radiosurgery (SRS).

Unruptured cerebral arteriovenous malformation in children: Outcome in treated and untreated patients

BackgroundThe management of unruptured cerebral arteriovenous malformation (URCAVM) is highly controversial; however, data regarding URCAVM in children are scarce. Material and methodsWe retrospectively reviewed consecutive children followed for URCAVM in our department between 2001 and 2021. ResultsOut of 36 patients, 12 were initially managed by observation, and 24 underwent first-line treatment: 8 by microsurgery, 10 by radiosurgery, 2 by embolization, and 4 by combined treatment. Mean follow-up of the whole group was 63months. Complete cure of the malformation was obtained in 14 patients (58%) in the treatment group: 8/8 in the microsurgery group, 5/10 in the radiosurgery group, 1/4 in the combined treatment group, and none in the embolization group. Two of the initially non-treated patients presented cerebral hemorrhage, with significant neurological consequences. In the treatment group, 5 patients presented new neurological deficits, only 1 of which, however, was functionally significant. Headache improved in 11 cases, mostly in the treatment group. Overall, 6 patients in the treatment group became asymptomatic, versus none in the observation group. ConclusionsThe treatment of URCAVM is a reasonable option in many pediatric cases, considering the cumulative risk of cerebral hemorrhage during the child's lifetime, as well as the symptoms specific to URCAVM. Microsurgery, when feasible, offers the best functional results and control of the AVM; however, the risk–benefit ratio should be weighed on a case-by-case basis. More studies will be needed to inform treatment decisions in pediatric URCAVM.

350 A Comparison of Incidental and Symptomatic Unruptured Brain Arteriovenous Malformations in Children

INTRODUCTION: Unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurologic deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. METHODS: We performed a retrospective analysis of patients presenting with brain AVMs from 1998 to 2021 at the University of California, San Francisco. Inclusion criteria included age ≤18 at time of presentation with angiographically-proven unruptured AVM diagnosed postnatally. RESULTS: Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurologic deficit. Ten (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared to symptomatic unruptured AVMs, incidental unruptured AVMs possessed smaller mean nidus maximum diameter (2.82 ± 1.1 vs. 3.98 ± 1.52 cm, p = 0.025) and deep venous drainage proportion (20% vs. 61%, p = 0.036). They also presented at earlier ages (10 ± 5.2 vs. 13.5 ± 4 years, p = 0.043) with longer durations to first treatment (541 ± 922 vs. 196 ± 448 days, p = 0.005). During the period of observation, one patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 centimeters or in deep location were treated with radiosurgery. Six other AVMs were treated with surgical resection with 2 receiving preoperative embolization. Seven AVMs (70%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurologic deficits following resection and 1 delayed seizure development following radiosurgery. The mean follow-up period was 5.1 ± 5.8 years without any hemorrhage episodes. CONCLUSIONS: A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into AVM natural history before symptom development or rupture.

A comparison of incidental and symptomatic unruptured brain arteriovenous malformations in children.

Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. Herein, the authors describe the largest series to date comparing the presentation, angioarchitecture, and management of incidental versus symptomatic unruptured AVMs in children. The authors performed a retrospective analysis of patients who presented with brain AVMs from 1998 to 2022 at the University of California, San Francisco. Inclusion criteria were age ≤ 18 years at the time of presentation and an angiographically proven unruptured AVM that had been diagnosed postnatally. Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurological deficit. Ten AVMs (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared with patients with symptomatic unruptured AVMs, patients with incidental unruptured AVMs had a smaller mean ± SD maximum nidus diameter (2.82 ± 1.1 vs 3.98 ± 1.52 cm, p = 0.025) and fewer had deep venous drainage (20% of patients vs 61%, p = 0.036). They also presented at an earlier age (10 ± 5.2 vs 13.5 ± 4 years, p = 0.043) and with longer duration to first treatment (541 ± 922 vs 196 ± 448 days, p = 0.005). During the observation period, 1 patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 cm in size or in a deep location were treated with radiosurgery. Six other AVMs were treated with resection, with 2 receiving preoperative embolization. Eight AVMs (80%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurological deficits after resection and 1 case of delayed seizure development after radiosurgery. The mean follow-up period was 5.7 ± 5.7 years without any hemorrhage episodes. A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into the natural history of AVM before symptom development or rupture.

Clinical Characteristics and Multimodality Therapy Outcomes in 304 Pediatric Patients with Cerebral Arteriovenous Malformations

Clinical follow-up data of pediatric patients with cerebral arteriovenous malformations (AVMs) are limited. This study investigated the characteristics of AVMs in children and analyzed the clinical outcomes of multimodality therapy in pediatric patients with AVMs at a single center. This retrospective study included consecutive patients diagnosed with AVMs at our institution between August 2008 and June 2018. Data on demographic characteristics, AVM features, and clinical outcomes were collected. Patients aged <18years at admission were defined as children. Overall, 1009 patients with AVMs were included, with 304 (30.1%) patients aged <18years. AVMs in pediatric patients were more likely to present with intracranial hemorrhage, mostly located in deep areas of the brain. A small nidus, exclusively deep drainage, and deep AVM location were associated with hemorrhage in children; Kaplan-Meier analysis revealed that patients with ruptured AVMs had a higher risk of developing a follow-up hemorrhage than those with unruptured AVMs. Among 290 children who were followed up, the multivariate regression analysis showed that a higher pretreatment modified Rankin Scale score, deep AVM location, and conservative treatment were significantly associated with unfavorable outcomes. In pediatric patients, AVMs were more likely to present with intracranial hemorrhage than that in adults. Hemorrhagic presentation in children was associated with a small nidus, exclusively deep drainage, and deep AVM location. Pediatric patients with ruptured AVMs had significantly higher risks of follow-up hemorrhage than those with unruptured AVMs. Our clinical results suggest that nonconservative treatment is better for pediatric patients with AVMs.

Effective treatment for symptomatic brain tumours and arteriovenous malformationin children by radiosurgery with a rotating gamma system

To assess the effectiveness of radiosurgery with a rotating gamma system (RGS) for paediatric brain tumours and arteriovenous malformation (AVM), this work was carried out on 123 patients with brain tumours or AVM received radiosurgery at Bach Mai hospital. The median dose was 13 Gy from a range of 8 to 20 Gy. Endpoints include the effects of increased intracranial pressure, seizures, hemiplegia, and tumour size on the syndrome. Results exhibited that the percentages of patients with complete response, partial response, and disease progression out of 59 patients with headache accounted for 11.9, 69.5, and 18.6%, respectively. Of the 20 patients with seizures, 30% had complete response, 65% partial response, and 5% stable disease. Regarding brain tumour size, 9 months after treatment, those with complete response, partial response, stable disease, and disease progression made up 18.7, 50.4, 14.6, and 16.3% of the total patients, respectively. There was no statistically significant effect of radiation dose on the results. This study showed a high percentage of children with brain tumours and AVM undergoing radiosurgery with a RGS had symptomatic responses after treatment.

Interventional Treatment Options in Children with Extracranial Vascular Malformations.

Extracranial vascular malformations vary greatly and belong to the complex field of orphan diseases and can involve all segments of the vascular tree: arteries, capillaries, and veins, and similarly the lymphatic system. The classification according to the International Society for the Study of Vascular Anomalies (ISSVA) represents an important guidance for selecting appropriate therapy. Although many of the principles of endovascular treatment, including image-guided sclerotherapy and embolization, are similar in adult and pediatric practice, there are some distinct differences regarding the treatment of vascular malformations of children. Thus, it is crucial to involve longer-term plan about managing these chronic diseases and their impact on a growing child. This review provides a detailed overview over the clinical presentation of venous, lymphatic, and arteriovenous malformations in children and emphasizes the specifics of their interventional treatment options, including distinct pediatric dose limitations and procedure-related side effects.

Curative Embolization for Pediatric Spetzler-Martin Grade III Cerebral Arteriovenous Malformations

Spetzler-Martin (SM) grade III arteriovenous malformations (AVMs) represent a gray zone due to their high variability in location, size, and angioarchitecture. In addition, there is a lack of information on curative embolization in the pediatric population, especially in this subgroup of lesions. Here we present our experience treating grade III AVMs by curative embolization in pediatric patients. Clinical and angiographic data from pediatric patients with grade III SM AVMs were retrospectively collected between 2011 and 2020 in a referral institution. We grouped the AVMs into subtypes according to size (S), venous drainage (V), and eloquence (E) and obtained subtypes: IIIA (S1V1E1), IIIB (S2V1E0), IIIC (S2V0E1), and IIID (S3V0E0). A total of 61 embolization sessions were performed in 35 pediatric patients. There were 25 females (64%), and the mean age was 12.2 years (range 5-18). Complete angiographic occlusion was achieved in 16 patients (47%). In 13 patients (37%), the AVM was occluded with a single embolization session and most (12/13) had small lesions (IIIA subtype). Among the 19 patients with incomplete occlusion, most (58%) had large lesions (IIIB, IIIC, and IIID). Large AVMs (IIIB, IIIC, and IIID) underwent 36 sessions; however, only 3 patients (21%) achieved complete occlusion in 11 sessions. Eight intraoperative complications (13% procedures) occurred mainly in ruptured AVMs (7/8) and eloquent zones (7/8). Curative embolization for SM grade III AVMs in children carries a high complication rate, especially in small, ruptured, and eloquent lesions. In addition, acceptable immediate complete angiographic occlusion rates were achieved, especially in small AVMs.

WITHDRAWN: Intralesional interstitial injection of bleomycin for management of extracranial arteriovenous malformations in children

Despite many advances in the treatment for extracranial arteriovenous malformations (AVMs), they still result in tedious dissection and potential unacceptable complications, particularly in children. Therefore, this study aimed to investigate the efficacy and safety of intralesional interstitial injection of bleomycin for the treatment of children with AVMs. A total of 10 children (6 boys and 4 girls) with AVMs were treated with serial interstitial bleomycin injections between May 2014 and January 2017. Maximum single doses of 15 U and 1 U/kg per session were administered for six sessions at a 1-month interval. Therapeutic effectiveness was evaluated and classified into four categories: complete response (CR), partial response (PR), no response, and worsening at 3 months after the last session. Further clinical follow-up outcomes were classified as improved, stable, or aggravated. Adverse events were recorded according to the Society of Interventional Radiology classification. All 10 children completed the sessions and follow-ups. CR occurred in 3 (30%) patients, PR in 6 (60%), and no response in 1 (20%). Minor complications (class A) included maculopapular rash, bulla, vomiting, and hyperpigmentation, whereas no major complications occurred. Intralesional interstitial injection of bleomycin is a feasible approach for the treatment of AVMs in children and provides safe and effective outcomes. This method may be an earlier treatment alternative in children to prevent potential destructive progression, considering the serious complications of currently available therapeutic methods.

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.

Intralesional Interstitial Injection of Bleomycin for Management of Extracranial Arteriovenous Malformations in Children

Despite many advances in the treatment for extracranial arteriovenous malformations (AVMs), they still result in tedious dissection and potential unacceptable complications, particularly in children. Therefore, this study aimed to investigate the efficacy and safety of intralesional interstitial injection of bleomycin for the treatment of children with AVMs. A total of 10 children (6 boys and 4 girls) with AVMs were treated with serial interstitial bleomycin injections between May 2014 and January 2017. Maximum single doses of 15 U and 1 U/kg per session were administered for six sessions at a 1-month interval. Therapeutic effectiveness was evaluated and classified into four categories: complete response (CR), partial response (PR), no response, and worsening at 3 months after the last session. Further clinical follow-up outcomes were classified as improved, stable, or aggravated. Adverse events were recorded according to the Society of Interventional Radiology classification. All 10 children completed the sessions and follow-ups. CR occurred in 3 (30%) patients, PR in 6 (60%), and no response in 1 (20%). Minor complications (class A) included maculopapular rash, bulla, vomiting, and hyperpigmentation, whereas no major complications occurred. Intralesional interstitial injection of bleomycin is a feasible approach for the treatment of AVMs in children and provides safe and effective outcomes. This method may be an earlier treatment alternative in children to prevent potential destructive progression, considering the serious complications of currently available therapeutic methods.

Spinal arteriovenous malformation in a pediatric patient with a history of congenital syphilis: a case report

BackgroundSpinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations.Case presentationA 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. MRI showed a lesion at T8-L1, angiogram was performed which confirmed the presence of a complex type IVc arteriovenous malformation and fistula from Artery of Adamkiewicz at L1-L2. It also showed peri medullary dilated veins and a pseudoaneurysm that compressed the spinal cord at T8-T10. Somatosensory evoked potentials and motor-evoked potentials were not recordable on the bilateral lower extremities prior to surgery. Once the patient was optimized for surgery, osteoplastic laminotomies from T6-T12 were performed. The dura was opened and the intradural, intramesenchymal hematoma was evacuated. There were two episodes of brisk arterial bleeding with hypotension during resection of the hematoma. The patient was taken to the angiography suite from the OR to successfully coil the large aneurysm. Intraoperative spinal cord monitoring remained undetectable in the bilateral lower extremities. The patient’s paraplegia remained unchanged from preoperative presentation.ConclusionCongenital syphilis may present with vascular changes that might impact surgical approaches and treatment outcomes in patients with spinal arteriovenous malformations. Preparation for massive transfusion and intraoperative monitoring are imperative in ensuring a safe perioperative experience.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

Background:Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs.Methods:In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included.Results:Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity.Conclusions:PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Brain Arteriovenous Malformation Recurrence After Apparent Microsurgical Cure: Increased Risk in Children Who Present With Arteriovenous Malformation Rupture.

Do children have an increased risk for brain arteriovenous malformation (AVM) recurrence compared with adults and does this risk vary depending on initial presentation with AVM rupture? We retrospectively studied 115 patients initially presenting with brain AVM under age 25 years who underwent complete surgical resection of the AVM as documented by digital subtraction angiography (DSA) and had delayed follow-up DSA to evaluate for AVM recurrence after apparent initial cure. The mean time from baseline DSA to follow-up DSA was 2.3 years, ranging from 0 to 15 years. Twelve patients (10.4% of the 115 patient cohort and 16.7% of 72 patients with hemorrhage at initial presentation) demonstrated AVM recurrence on follow-up DSA. All patients with recurrence initially presented with intracranial hemorrhage, and intracranial hemorrhage was a significant predictor of recurrence (log rank P=0.037). Among patients with initial hemorrhage, the 5-year recurrence rate was 17.8% (95% CI, 8.3%-35.7%). All recurrences occurred in patients who were children at the time of their initial presentation; the oldest was 15 years of age at the time of initial AVM surgery. The 5-year recurrence rate for children (0-18 years of age) with an initial presentation of hemorrhage was 21.4% (95% CI, 10.1%-41.9%). Using Cox regression, we found the risk of AVM recurrence decreased by 14% per each year increase in age at the time of initial surgical resection (hazard ratio=0.86 [95% CI, 0.75-0.99]; P=0.031). There is a high rate of recurrence of apparently cured brain AVMs in children who initially present with AVM rupture. Imaging follow-up is warranted to prevent re-rupture.

Treatment Strategies and Related Outcomes for Brain Arteriovenous Malformations in Children: A Systematic Review and Meta-Analysis.

OBJECTIVE. The objective of this study was to assess the available evidence in the literature regarding treatment outcomes for pediatric patients with brain arteriovenous malformation (bAVM) with the aim of providing practice guidelines for treatment decisions and highlighting research areas that need attention. MATERIALS AND METHODS. Keyword searches for studies published from January 1, 1981, to April 16, 2018, were performed in MEDLINE, Embase, and Web of Science. Predefined inclusion criteria were used to identify studies. Poisson regression analysis for associations between patient and bAVM characteristics and treatment outcomes. RESULTS. We identified 34 articles comprising 2158 children with bAVM who underwent treatment or observation. The mean age of the study cohort was 12.0 ± 1.6 (SD) years, and 48.1% of the patients were female; 64.3% of bAVMs were hemorrhagic at presentation. The mean follow-up was 50.6 ± 32.3 months. Overall, the meta-analysis of pooled data showed an obliteration rate of 69.8% (95% CI, 62.9-75.9%), recurrence rate of 2.2% (95% CI, 1.1-4.3%), and mortality rate of 2.4%. The pooled complication rate was 22.5% (95% CI, 15.7-31.1%) after surgery, 26.4% (95% CI, 15.2-41.9%) after embolization, and 27.1% (95% CI, 18.1-38.4%) after radiosurgery. Mortality was not associated with age, sex, or hemorrhage; however, recurrence after treatment was inversely associated with age. Complication and mortality rates were reduced for multimodal treatments. For patients with bAVM treated with observation only, complication and mortality rates were 35.9% and 23.5%, respectively. CONCLUSION. Multimodality treatments for pediatric bAVM had lower mortality and complication rates than individual treatments. However, there is a lack of evidence for long-term outcomes. The mortality rate was highest in conservatively managed patients (i.e., observation only). Further research directly comparing different treatment modalities for recurrence and complications is warranted. Gathering data prospectively through multiinstitutional registries will be key to provide strong evidence.

CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia.

OBJECTIVE. The purpose of this study was to evaluate the CT angiography (CTA) findings of pulmonary arteriovenous malformation (PAVMs) in patients with hereditary hemorrhagic telangiectasia and to correlate these findings with those of graded contrast-enhanced transthoracic echocardiography (CE-TTE). MATERIALS AND METHODS. A retrospective review was conducted of PAVMs visualized at CTA of patients with abnormal CE-TTE findings (3-point scale). Location, distribution, size, number, volume, grade, and relative attenuation (attenuation of PAVM divided by attenuation of aorta) of PAVMs were recorded. PAVMs were graded as follows on conventional and maximum-intensity-projection (MIP) images: 0, nodule, unlikely PAVM; 1, ground-glass opacity (GGO); 2, GGO with increased vascular network; 3, GGO or nodule with single vessel; 4, GGO or nodule with two or more vessels; 5, GGO or nodule with afferent and larger efferent vessels; 6, mature arteriovenous malformation. Correlation between PAVM grade and relative attenuation and between CTA variables and CE-TTE grades was assessed. RESULTS. Forty patients (median age, 14.9 years; range, 0.6-27.9 years) had 117 PAVMs at CTA: 107 peripheral, eight central, and two both peripheral and central. None of the PAVMs was diffuse. Median size and volume were 0.4 cm (range, 0.1-4.4 cm) and 0.031 mL (range, 0.0009-10.019 mL). At CTA, seven PAVMs were grade 1, five grade 2, 28 grade 3, 62 grade 4, two grade 5, and 13 grade 6. MIP images showed 39 of 117 PAVMs were higher grade. Statistically significant correlation was found between relative attenuation and PAVM grade (p < 0.001, r = 0.58) in 40 patients and between all CTA variables and CE-TTE (p < 0.05, strongest correlation with highest grades [p < 0.0001, r = 0.81]) in 32 patients. CONCLUSION. In children and young adults with hereditary hemorrhagic telangiectasia, grade 4 PAVMs were most common. Higher-grade PAVMs more often have right-to-left shunts.

Pulmonary arteriovenous malformations in children with palliated univentricular CHD: our limited understanding persists.

Pulmonary arteriovenous malformations in children with palliated univentricular CHD: our limited understanding persists.

Postoperative 30-day outcomes after craniotomy for supratentorial AVM resection in children

PurposeTo describe 30-day outcomes following craniotomy for arteriovenous malformation in children and identify risk factors for readmission, reoperation, and perioperative complication using the National Surgical Quality Improvement Program (NSQIP) Pediatric database. MethodsPatients aged 0–18 years who underwent surgery for arteriovenous malformations (years 2015–2016) were identified from the NSQIP Pediatric database. Descriptive statistics, uni-variate, and multi-variate regression analysis were performed using preoperative and perioperative data. The outcome of interest was postoperative adverse event including reoperation within 30 days, readmission within 30 days, discharge to rehab, and the complications wound infection/dehiscence, pneumonia, unplanned reintubation, pulmonary embolism (PE), renal insufficiency, urinary tract infection (UTI), stroke, venous thromboembolism (VT), and sepsis. Results167 patients were identified who met study criteria. 58% were male, and the majority had an ASA classification of 3 or greater (68%). 96 (57%) patients were found to have a preoperative comorbidity, with the most common comorbidity being seizure disorder (54 patients, 32%). 76 patients (46%) had documented perioperative events or complications. The incidence of wound infection/dehiscence was 4%; and of pneumonia, PE, unplanned reintubation, renal insufficiency, UTI, stroke, VT, sepsis were <1%. There were no deaths. The incidence of unplanned reoperation was 10% and unplanned readmission was 12%. Most (90%) were discharged to home. Operative time (p = .0001, OR = 9.53), emergent surgery status (p = .0001, OR = 8.19) and preoperative comorbidities (p = .007) were found to be significant predictors of poor outcome. ConclusionIn the NSQIP-P dataset, the incidence of perioperative complications and suboptimal outcomes among patients undergoing AVM resection were low.