Malformations In Pediatric Patients Research Articles

Evaluation of auditory pathways and comorbid inner ear malformations in pediatric patients with Duane retraction syndrome.

This study aimed to investigate the presence, type, and severity of hearing losses in individuals with Duane Retraction Syndrome (DRS), and to ascertain if there are anomalies in the auditory pathways at the brainstem level in DRS, believed to arise from aberrant interaction between cranial nerves and brainstem nuclei. Cross-sectional observational study. Tertiary referral centre. The study group comprised 20 patients diagnosed with DRS, aged between 5 and 18 years, who underwent ophthalmological, otological and audiological follow-up at our clinic. The control group was established using data obtained from the previous work of Sanfins M.D., et al., 2022. Participants underwent tympanometry and pure tone audiometry after ophthalmological and otological examination. Patients with hearing loss underwent auditory brainstem response (ABR) testing, while patients with normal hearing underwent temporal CT and MRI to detect possible aetiology. Tympanograms, air and bone conduction pure tone averages, latencies of wave I, III and V and interwave latencies as well as radiological findings on CT and MRI were noted. Profound sensorineural hearing loss was detected in two of the 20 patients. One of the patients with hearing loss had left-sided exo-Duane, cochlear aplasia with dilated vestibule (CADV) anomaly in the right ear and profound sensorineural hearing loss in the right ear. The other patient had bilateral exo-Duane, incomplete partition type I (IP-I) malformation in the right ear and profound sensorineural hearing loss in the right ear. There was no significant difference observed in the ABR latencies of wave I, III, and V, as well as the inter-wave latencies, between the control group and the individuals diagnosed with DRS who had normal hearing. No statistically significant difference was found in auditory brainstem responses between the control group and patients with DRS without hearing loss. It can be speculated that Duane retraction syndrome and hearing loss do not share a common pathogenesis at the level of the brainstem. However, it should be noted that hearing loss may be associated with inner ear malformations in DRS patients.

Arnold-Chiari Malformations in Pediatric Patients After Fetal Surgery for Meningomyelocele.

(1) Background: Fetal surgery for meningomyelocele (MMC) should reduce the occurrence of Arnold-Chiari malformations, hydrocephalus, the associated need for craniocervical decompression, and the need for cerebrospinal fluid shunt insertion. Fetal surgery should improve ambulatory status. (2) Methods: We used retrospective analysis of the documentation and descriptive statistics to summarize the clinical data and measured MRI parameters. The neurosurgical results are presented as the frequency of findings in percentages and compared with the results of the Management of Myelomeningocele Study (MOMS). (3) Results: A total of eight patients who underwent prenatal correction of MMC between 2016 and 2020 participated. MRI detected Chiari II malformation in all patients during prenatal imaging and in 87.5% of the patients at the age of 12 months. Craniocervical decompression was used in 25% of the patients. Shunt-dependent hydrocephalus occurred in 50% of the cases. In 87.5% of the cases, the functional level exceeded the expected ambulatory status. (4) Conclusions: We present the clinical status of our patients. Analysis of the complete cohort confirmed that prenatal surgery is associated with a reduced occurrence of Chiari malformations and reduced associated occurrence of hydrocephalus. Specific lesion levels are not associated with the need for craniocervical decompression. The results of our study are valuable in prenatal counseling and important for treatment planning.

Vascular malformations in pediatric patients: 10-year experience of a vascular anomalies clinic.

Vascular malformations (VaMs) are caused by errors in vascular morphogenesis. Diagnosis and treatment can be complex. Few specialized centers care for these patients, and limited literature exists regarding their characteristics and clinical course. The vascular anomalies clinic (VAC) at the Instituto Nacional de Pediatría (National Institute for Pediatrics) is a multidisciplinary team and has been a reference center for patients with VaMs since 2012. We sought to describe the characteristics of patients cared for at the VAC, types of VaMs, treatments used, and clinical course. This was a descriptive, observational, retrospective, and cross-sectional study conducted from 2012 to 2022. We included 435 patients with VaMs; the median age of presentation was 1 month. The most frequent signs and symptoms were increased volume (97.2%), superficial color change (65.5%), and pain (43.3%). The most common VaMs were lymphatic (36.7%) and venolymphatic (18.3%). Sclerotherapy was the most frequent treatment (73.4%), followed by medical treatment with sirolimus (18.5%); response to both was excellent/good in > 85% of cases. In this retrospective study of children with VaMs, we found that low-flow malformations were the most common, and sclerotherapy and sirolimus were the most frequently used treatments. The therapeutic response was excellent/good in most cases.

Timing of Embolization, Radiosurgery, and Resection of Arteriovenous Malformations in Pediatric Patients: A Retrospective, Descriptive Study

ObjectiveCerebral arteriovenous malformations (AVMs) are a challenging pathology in pediatric patients, carrying a high risk of morbidity and mortality. Treatment modalities include resection, endovascular embolization and stereotactic radiosurgery (SRS). There is currently no consensus favoring one modality over another. Timing of multimodal therapy with embolization/SRS and resection is not well explored in the literature. Here we present a series of pediatric AVM patients, with special attention paid to the timing of treatment. MethodsElectronic medical records of all pediatric patients (<18 years at treatment) with AVMs treated at our institution were retrospectively reviewed after IRB approval. Demographic information, AVM characteristics, treatment variables and outcomes were recorded. Results27 total patients were included. 21 (77.8%) presented with a ruptured AVM. 6 patients (28.6%) had an GCS of 3 to 10, and underwent treatment within 24 hours of presentation. 10 patients (47.6 %) with a GCS of 12 to 15 were treated between 24 to 120 hours. 5 patients (23.8%) were treated 3 weeks to 14 months after AVM rupture. 96% of our cohort, regardless of rupture status, had mRS of 1-2 at most recent follow-up. ConclusionsWe present our institution’s experience with pediatric AVM’s, focusing on the timing of treatment. Based on our experience, early treatment of AVMs seems to be safe and effective regardless of rupture status.

1133 Biologically Effective Dose and Prediction of Obliteration of Arteriovenous Malformations in Pediatric Patients Treated by Gamma Knife Radiosurgery

INTRODUCTION: Stereotactic radiosurgery (SRS) represents an effective treatment for pediatric arteriovenous malformations (AVMs). Biologic effective dose (BED) is recognized as a predictive variable for outcomes in the adult population, but its role has never been studied in pediatric outcomes. METHODS: Retrospective data for = 18 years old patients treated with single-session SRS for AVM was collected from 1989 – 2019. BED calculations were performed using an a/b ratio of 2.47. Kaplan-Meier analysis was used to evaluate obliteration, new hemorrhage, and radiation-induced changes (RIC). Cox-regression analysis was used for obliteration prediction using two models (margin dose versus BED). RESULTS: One-hundred-ninety-seven patients [median age = 13.1 years, Interquartile range (IQR) = 5.2] were included; 72.6% presented initially with spontaneous hemorrhage. A median margin dose of 22 Gy (IQ = 4.0) with a median BED of 183.2Gy (IQR = 70.54) was used to treat AVMs with a median volume of 2.8 cm3 (IQR = 2.9). Following SRS, obliteration was confirmed in 115 patients (58.4%) using MRI and angiography at a median follow-up of 2.85 years (IQR = 2.26). The cumulative obliteration probability was 43.6% (95% CI = 36.1-50.3), 60.5% (95% CI+=2.2-67.4), 66.0% (95% CI= 56.0-73.7) at 3, 5 and 10 years respectively. In multivariate analysis, a BED &gt;180 Gy [HR = 2.11, 95% CI=1.30-3.40, p = 0.002] in model 1, and a margin dose &gt;20 Gy [HR = 1.90, 95% CI = 1.15-3.13, p = 0.019] in model 2, were associated with obliteration. An AVM nidus volume &gt;4 cm3 was associated with lower obliteration rates in both models. The probability of symptomatic RIC at 10 years was 8.6% (95% CI = 3.5-13.4). Neither BED nor margin dose were associated with RIC occurrence, with the only predictive factor being deep AVM location [HR = 3, 95% CI = 1-9.1, p = 0.048]. CONCLUSIONS: This study confirms BED as a predictor for pediatric AVM obliteration. Optimization of BED in pediatric AVM SRS planning may improve cumulative obliteration rates.

Biologically Effective Dose and Prediction of Obliteration of Arteriovenous Malformations in Pediatric Patients Treated by Gamma Knife Radiosurgery.

Stereotactic radiosurgery (SRS) represents an effective treatment for pediatric arteriovenous malformations (AVMs). Biological effective dose (BED) has shown promising results in 2 previous studies as a predictive variable for outcomes in adults, but its role has never been studied in pediatric outcomes. Retrospective data for patients 18 years or younger treated with a single-session SRS for AVMs were collected from 1989 to 2019. BED calculations were performed using an α/β ratio of 2.47. Kaplan-Meier analysis was used to evaluate obliteration, new hemorrhage, and radiation-induced changes (RIC). Cox-regression analysis was used for obliteration prediction using 2 models (margin dose vs BED). One hundred ninety-seven patients (median age = 13.1 years, IQR = 5.2) were included; 72.6% (143/197) of them presented initially with spontaneous hemorrhage. A median margin dose of 22 Gy (IQR = 4.0) with a median BED of 183.2 Gy (IQR = 70.54) was used to treat AVM with a median volume of 2.8 cm 3 (IQR = 2.9). After SRS, obliteration was confirmed in 115/197 patients (58.4%) using magnetic resonance imaging and angiography at a median follow-up of 2.85 years (IQR = 2.26). The cumulative obliteration probability was 43.6% (95% CI = 36.1-50.3), 60.5% (95% CI+ = 2.2-67.4), and 66.0% (95% CI = 56.0-73.7) at 3, 5, and 10 years, respectively. In Cox multivariate analysis, a BED >180 Gy (hazard ratio [HR] = 2.11, 95% CI = 1.30-3.40, P = .002) in model 1 and a margin dose >20 Gy (HR = 1.90, 95% CI = 1.15-3.13, P = .019) in model 2 were associated with obliteration. An AVM nidus volume >4 cm 3 was associated with lower obliteration rates in both models. The probability of symptomatic RIC at 10 years was 8.6% (95% CI = 3.5-13.4). Neither BED nor margin dose was associated with RIC occurrence, with the only predictive factor being deep AVM location (HR = 3, 95% CI = 1-9.1, P = .048). This study confirms BED as a predictor for pediatric AVM obliteration. Optimization of BED in pediatric AVM SRS planning may improve cumulative obliteration rates.

Endovascular Treatment of Brain Arteriovenous Malformations in Pediatric Patients: A Single Center Experience and Review of the Literature.

Brain arteriovenous malformations (bAVMs) are abnormal vascular connections with direct arteriovenous shunts, generally symptomatic in the adult life. However, a small number of bAVMs may manifest in pediatric patients, with higher bleeding risk and mortality rates when compared to adults. The purpose of this study is to review our experience with endovascular treatment of bAVMs in pediatric patients. This is a retrospective analysis of all bAVMs in pediatric patients (0-18 years) who underwent diagnostic digital subtraction angiography (DSA) at our institution from January 2010 to June 2021. Twenty-six patients met the inclusion criteria, of which 12 underwent endovascular treatment. Treated patients had a mean age of 10.25 years and 58% were females. Complete angiographic exclusion was achieved in five (42%) patients with endovascular treatment. Five patients with residual bAVM after embolization needed adjuvant therapy with surgery (n = 3) or stereotactic radiosurgery (SRS; n = 2). Two patients are still undergoing embolization sessions. Procedure-related complications occurred in two patients (17%) and included small vessel perforation and an occipital ischemic stroke. Two patients showed bAVM recurrence on follow-up (17%) and subsequently underwent SRS (n = 1) or surgery (n = 1), both resulting in complete bAVM exclusion. All patients had a modified Rankin scale (mRS) score of 0 to 2 on follow-up. Our experience supports the effectiveness and safety of endovascular treatment of bAVM in selected pediatric patients. A multidisciplinary approach combining surgery and SRS is warranted to achieve higher complete bAVM obliteration rates. Long-term follow-up is important as these lesions may show recurrence over time, especially in the pediatric population.

Recurrence of brain arteriovenous malformations in pediatric patients: a long-term follow-up study

BackgroundPreviously thought to be congenital, AVMs have shown evidence of de-novo formation and continued growth, thus shifting thoughts on their pathophysiology. Pediatric AVM patients have been reported to be more prone to develop AVM recurrence after a seemingly complete cure. Therefore, we assessed the risk of AVM treated in childhood to recur in adulthood after a long-term follow-up in our own cohort.MethodsControl DS-angiography was arranged during 2021–2022 as part of a new protocol for all AVM patients who were under 21 years of age at the time of their treatment and in whom the treatment had occurred at least five years earlier. Angiography was offered only to patients under 50 years of age at the time of the new protocol. The complete eradication of AVM after the primary treatment had been originally confirmed with DSA in every patient.ResultsA total of 42 patients participated in the late DSA control, and 41 of them were included in this analysis after excluding the patient diagnosed with HHT. The median age at the time of admission for AVM treatment was 14.6 (IQR 12–19, range 7–21 years) years. The median age at the time of the late follow-up DSA was 33.8 years (IQR 29.8–38.6, range 19.4–47.9 years). Two recurrent sporadic AVMs and one recurrent AVM in a patient with hereditary hemorrhagic telangiectasia (HHT) were detected. The recurrence rate was 4.9% for sporadic AVMs and 7.1% if HHT-AVM was included. All the recurrent AVMs had originally bled and been treated microsurgically. The patients with sporadic AVM recurrence had been smoking their whole adult lives.ConclusionsPediatric and adolescent patients are prone to develop recurrent AVMs, even after complete AVM obliteration verified by angiography. Therefore, imaging follow-up is recommended.

Targeted Diode Laser Therapy for Oral and Perioral Capillary-Venous Malformation in Pediatric Patients: A Prospective Study.

This study describes the management protocol for capillary-venous malformations in pediatric patients and reports the epidemiology of diagnosed and treated cases at the Unit of Odontostomatology of the Aldo Moro University of Bari from 2014 to 2022. The authors classified the intraoral and perioral capillary-venous malformations by superficial diameter (<1 cm, 1-3 cm, >3 cm) and ultrasonographical depth extension (≤5 mm, >5 mm). All patients underwent pulsed-mode diode laser transmucosal photocoagulation (8-12 W/cm2); those with malformations that were wide (>3 cm) and deep (>5 mm) received intralesional photocoagulation, too (13 W/cm2). The children received general anesthesia based on their compliance and lesions' extension. The follow-up lasted six months. A total of 22 females and 14 males (age range 4-18 years) presented 63 capillary-venous malformations. Five patients with Sturge-Weber syndrome, seven with hereditary hemorrhagic telangiectasia, and five with angiomatosis showed multiple malformations. The authors found no intraoperative or postoperative complications. Seventeen patients with lesions >1 cm and >5 mm deep required multiple laser sessions to heal. The results of the current study support diode laser photocoagulation as the gold standard for the treatment of intraoral and perioral capillary-venous malformations in pediatric patients.

Sociodemographic characteristics of pediatric patients with vascular malformations: Results of a single site study.

Vascular malformations, the abnormal development of blood vessels, are a rare set of congenital anomalies. The sociodemographic factors associated with vascular malformations in pediatric patients are poorly understood. This study examined sociodemographic factors of 352 patients presenting to a single vascular anomaly center from July 2019 to September 2022. Characteristics such as race, ethnicity, sex, age at presentation, degree of urbanization, and insurance status were recorded. This data was analyzed by comparing the different types of vascular malformations, including arteriovenous malformation, capillary malformation, venous malformation (VM), lymphatic malformation (LM), lymphedema, and overgrowth syndrome. Patients were primarily white, not Hispanic or Latino, female, had private health insurance, and were from the most urban setting. No differences in sociodemographic factors were found among the different vascular malformations except patients with VM presented at a later age than patients with LM or overgrowth syndrome. This study provides novel insight into the sociodemographic factors of pediatric patients presenting with vascular malformations and indicates a need for their improved recognition for the timely initiation of treatment.

Natural history and treatment of deep-seated brain arteriovenous malformations in pediatric patients.

Pediatric deep brain arteriovenous malformations (bAVMs) represent a unique management challenge given their higher cumulative risk of hemorrhage as well as a higher risk of treatment. Better understanding of hemorrhage risk in this patient population will lead to a better decision-making process for patient management. The authors retrospectively reviewed their institutional bAVM database from 1990 to 2019 and included patients younger than 21 years who had deep-seated bAVMs. They present the annual hemorrhage risk, during the natural history and after treatment, and functional outcomes. Thirty-one pediatric patients were included in this study (13 males and 18 females) with a mean age of 11.8 (SD 4.4) years. The most frequent presenting symptoms were headache (54.8%), weakness (38.7%), and seizure (22.6%). The mean follow-up duration was 13.14 (SD 12.5) years, during which 7 (22.6%) AVMs were obliterated, 10 (32.3%) individuals experienced hemorrhage, and the modified Rankin Scale score worsened in 8 (25.8%) patients. The annual natural history risk of hemorrhage was 3.24% per patient, and the overall annual hemorrhage risk after treatment was 1.98% per patient. In particular, the risk was reduced to 0.64% per patient in the stereotactic radiosurgery (SRS) group. Non-White race showed a trend of higher rupture at presentation (OR 5 [95% CI 0.84-41.68], p = 0.09). Female sex was associated with higher odds (OR 13.076 [95% CI 1.424-333.591], p = 0.048) and SRS was associated with lower odds (OR 0.122 [95% CI 0.011-0.862], p = 0.049) of follow-up hemorrhage. Given the substantial cumulative risk of lifelong hemorrhagic stroke in pediatric patients, timely definitive treatment is warranted. SRS may be beneficial when the risk-benefit profile is deemed acceptable.

Magnetic Resonance Imaging Findings and Accompanying Malformations in Pediatric Patients with Gray Matter Heterotopia

AbstractObjective: The aim of the study is to classify gray matter heterotopias detected in pediatric patients according to magnetic resonance imaging findings and to define the accompanying cerebral malformations.Material and Methods: Images of all pediatric patients who were detected to have heterotopia in brain magnetic resonance imaging between January 2012 and June 2020 were retrospectively evaluated. The type, location of heterotopia, and accompanying cerebral anomalies were analyzed.Results: A total of 42 patients, 22 male, and 20 female, with a mean age of 7.80±4.53 years (2-16 years) with gray matter heterotopia were included in the study. Of the patients 33 (78.6%) had subependymal, 7 (16.7%) had subcortical, and 2 (4.7%) had band heterotopia. Twenty-four patients had epilepsy.Conclusion: According to our study, subependymal heterotopia was most common in pediatric patients. The subependymal heterotopias were most commonly located in the trigon region of the lateral ventricles. All of the subcortical heterotopies were located in the frontal and unifocal. Band heterotopias were located symmetrically in the subcortical region of the bilateral cerebral hemispheres. The main accompanying anomalies were Chiari II malformation, and corpus callosum agenesis. Defining the type of heterotopia and associated anomalies in pediatric patients is important for patient management.

High-flow nasal cannula (HFNC) oxygenation for sclerotherapy of facial and upper airway vascular malformations in pediatric patients: Case series.

We report anesthesia management of sclerotherapy for vascular malformations (VMs) of the upper airway and face of pediatric patients conducted under sedation using a high-flow nasal cannula (HFNC) oxygen delivery system. Sclerotherapy procedures were carried out in six patients (five males, one female; age group: 5-12 years). The patients were sedated with midazolam, fentanyl, ketamine, and graded doses of propofol along with continuous oxygen delivery using HFNC. There were no episodes of oxygen desaturation, tongue fall or obstruction of the airway, interruption of procedure for assisted ventilation, and postoperative nausea and vomiting (PONV). Only two patients showed transient apnea for 10 and 15 s but did not require ventilatory assistance. HFNC provides effective oxygenation in pediatric patients undergoing sclerotherapy of VMs of the upper airway and face under sedation.

Bleomycin intralesional injections of maxillofacial venous malformations in pediatric patients

BackgroundMost venous malformations (VMs) present in the head and neck region. Many sclerosing agents have been documented in the literature, but Bleomycin is a new promising sclerosing agent of facial venous malformations. Objectivesthis study was conducted to evaluate the clinical outcome and safety of Bleomycin (BL) intralesional injections in management of maxillofacial venous malformation in pediatric patients. Methods15 patients of both sexes and of age up to 12 years having VMs in the maxillofacial region were randomly selected from the vascular anomalies clinic in Cairo University Specialized Pediatric Hospital. All patients were treated by BLM intralesional injections with the safe pediatric dose of 0.5 IU/kg, at 4 weeks intervals with maximum dose of 15 IU\\session. All cases were performed by the same operator, and patients were followed up over 12 months after last injection. ResultsTwelve patients had complete response (>90% improvement), and 3 patients had marked improvement response. Eleven patients were injected under topical local anaesthesia, and 4 patients had regional local anaesthesia. All patients had post-operative swelling that lasted for 3–5 days. There was no pain during or after injections, no ulceration, no weight loss, no hair loss and no signs of pulmonary fibrosis throughout the follow up time. Recurrence occurred only in 2 patients due to noncompliance with sessions, and they were re-injected with BLM. Conclusionthis study has shown that Bleomycin is safe, effective and an excellent therapy for treating VMs in the maxillofacial region. No systemic or local complications had occurred.

The Relationship Between the Third Window Abnormalities and Inner Ear Malformations in Children with Hearing Loss.

OBJECTIVE: To evaluate the relationship between the third window abnormalities and congenital inner ear malformations in pediatric patients with different types of hearing loss. If such a relationship should exist, it would be important to take it into account, in order to diagnose and treat pediatric hearing loss cases more accurately.Methods:Two hundred twenty-one children with hearing loss who had temporal bone computed tomography (CT) examination and were identified from 2013 to 2018 were retrospectively evaluated. The types of hearing loss were grouped as sensorineural hearing loss (SNHL), conductive hearing loss (CHL), and mixed hearing loss (MHL). Third window abnormalities included superior semicircular canal (SC) dehiscence, posterior SC dehiscence, enlarged vestibular aqueduct (EVA), X-linked stapes gusher, perilymph fistula, and bone dyscrasias. Congenital inner ear malformations included cochleovestibular, SC, and internal acoustic canal malformations. The relationships were analyzed with chi-square and Fisher’s exact tests.Results: In the study, 40 patients had unilateral hearing loss and 181 had bilateral hearing loss. In 402 ears, the rates of SNHL, CHL, and MHL were 88.5%, 6.9%, and 4.4%, respectively. EVA was the most common third window abnormality (41/402; 9.7%), and SC malformations were the most common inner ear malformations (53/402; 13.2%). In the SNHL group, superior and posterior SC dehiscence were associated with cochleovestibular malformations (P = .035 and.020, respectively). In the CHL group, there was a relationship between EVA and SC malformations (P = .041). No relationships were found in the MHL group. Conclusion: Third window abnormalities and congenital inner ear malformations may be encountered simultaneously in children with SNHL and CHL.

Cervical lymphatic malformations amenable to transhairline robotic surgical excision in children: A case series.

Lymphatic malformations are rare benign malformations that predominantly occur in the head and neck region. The advent of surgical robots in head and neck surgery may provide beneficial outcomes for pediatric patients. Here, we describe our experiences with transhairline incisions for robot-assisted surgical resection of cervical lymphatic malformations in pediatric patients.In this prospective longitudinal cohort study, we recruited consecutive patients under 18 years of age who were diagnosed with congenital cervical lymphatic malformations and scheduled for transhairline approach robotic surgery at a single medical center. We documented the docking times, console times, surgical results, complications, and postoperative follow-up outcomes.The studied patients included 2 with mixed-type lymphatic malformations and 2 with macrocystic-type lymphatic malformations. In all 4 patients, the incision was hidden in the hairline; the incision length was <5 cm in 3 patients but was extended to 6 cm in 1 patient. Elevating the skin flap and securely positioning it with Yang retractor took <1 hour in all cases. The mean docking time was 5.5 minutes, and the mean console time was 1 hour and 46 minutes. All 4 surgeries were completed endoscopically with the robot. The average total drainage volume in the postoperative period was 21.75 mL. No patients required tracheotomy or nasogastric feeding tubes. Neither were adverse surgery-associated neurovascular sequelae observed. All 4 patients were successfully treated for their lymphatic malformations, primarily with robotic surgical excisions.Cervical lymphatic malformations in pediatric patients could be accessed, properly visualized, and safely resected with transhairline-approach robotic surgery. Transhairline-approach robotic surgery is an innovative method for meeting clinical needs and addressing esthetic concerns.

Vein of Galen Aneurysmal Malformations in Pediatric Patients Submitted to Endovascular Treatment: Report of 3 Cases

Abstract Objective To report three cases of vein of Galen aneurysmal malformation (VGAM) in pediatric patients treated at the hemodynamics lab of Hospital Santa Isabel (HSI) in Blumenau, state of Santa Catarina, Brazil, from 2006 to 2020. Clinical presentation, endovascular treatment, and postprocedure evolution to date are included. Case description Three children aged 5 to 12 months with cardiac, respiratory, or neurological damage in the neonatal stage, were referred to the neurosurgery service and diagnosed with VGAM. The three patients underwent endovascular embolization of the malformation, with different clinical evolution throughout outpatient follow-up. Conclusion Vein of Galen aneurysmal malformations are uncommon vascular abnormalities that, until the advent of endovascular embolization, were associated with high morbidity and mortality rates. Its prognosis is linked with initial clinic, early diagnosis, and timely surgical correction.

Early Outcomes and Complications of Endovascular Treatment of Cerebral Arteriovenous Malformations in Pediatric Patients

Introduction: Arteriovenous malformations (AVMs) are the commonest cause of hemorrhagic stroke in children. Endovascular embolization is a feasible treatment modality, but cure rates are heterogenous from one series to another. We aimed to describe the immediate obliteration rates and periprocedural complications of embolization of pediatric AVMs. Methods: Between 2011 and 2019, participants below 18 years of age with AVMs treated by the same neurosurgeon at a single center were included. The clinical features, immediate angiographic results, and periprocedural complications were retrospectively collected from the clinical records. Results: Thirty-four embolization sessions were performed on 20 children (12 females with a mean age of 13). Intracranial hemorrhage was the most common presentation (75%), and the majority were frontal (30%) and basal ganglia (30%) lesions. An immediate complete angiographic obliteration was achieved in 9 patients (45%) with low-grade lesions (Spetzler-Martin grade I and II). NBCA was the most common embolic agent used (52.9%). Complications were reported in 3 (8.8%) out of 34 sessions. Two of them were intraoperative perforations with clinical consequences. A slight cortical hemorrhage during the procedure was observed in 1 patient without clinical repercussions. Discussion: This single-surgeon single-center experience suggests that endovascular treatment is a safe and efficient treatment for pediatric AVMs. Pediatric prognostic scores for a suitable selection of candidates are needed. Further studies are required to validate these results.

Cerebral Arteriovenous Malformations in Pediatric Patients with Hereditary Hemorrhagic Telangiectasia: Re-evaluating Appearance, Bleeding Risk, and Treatment Necessity in a Selective Meta-analysis

AbstractPediatric patients suffering from cerebral nidal arteriovenous malformations are a unique population due to the rare occurrence of the disease. Diagnosis of hereditary hemorrhagic telangiectasia (HHT) in children is a rare event and mainly restricted to coincidental detection from screening of possibly afflicted family members. In patients with HHT, it is well known that the incidence of cerebral vascular malformations (CVMs) is higher than expected when compared with the nonafflicted population. Even though CVMs comprise a variety of different distinct anatomical and radiographic entities (e.g., capillary telangiectasia, nidal arteriovenous malformation [AVM], cavernous malformation, dural or pial as well as mixed fistula, and vein of Galen malformation), they are mostly summarized and analyzed all in one category due to the low number of individual cases identified in single centers. Nevertheless, the best treatment algorithm will likely vary significantly between different CVMs as does the clinical presentation and the natural course of the CVM. It is therefore the objective of this article to focus on nidal AVMs in pediatric patients suffering from HHT. To this end, we performed a systematic selective literature research to estimate incidence, clinical and radiological appearance, as well as classification according to established grading system, and to evaluate the necessity of treatment of these lesions in light of their respective outcomes. Our line of arguments explains why we recommend to follow these lesions expectantly and suggest to keep pediatric patients under surveillance with sequential scans until they reach adulthood.

Letter: Clinical Outcomes of Stereotactic Radiosurgery for Cerebral Arteriovenous Malformations in Pediatric Patients: Systematic Review and Meta-Analysis.

Letter: Clinical Outcomes of Stereotactic Radiosurgery for Cerebral Arteriovenous Malformations in Pediatric Patients: Systematic Review and Meta-Analysis.