Pediatric intracranial aneurysms in a Southern Brazilian population.

INTRODUCTION: Intracranial aneurysms are rare in the pediatric population, and their diagnosis can be challenging. They differ from their adult counterparts in several aspects, and hemorrhage is the most common presentation. OBJECTIVE: To evaluate clinical data, aneurysm characteristics, and therapeutic results in a series of patients younger than 19 years of age with intracranial aneurysms. METHOD: A retrospective cross-sectional observational study design analyzed medical records and imaging studies. Variables included age, sex, clinical presentation, comorbidities, aneurysmal characteristics, treatment modality, and clinical outcomes. RESULTS: We identified 15 intracranial aneurysms in 11 patients (6 male), with ages ranging from 3 months to 15 years (mean age 5.2 years). Five patients had associated medical conditions, and hemorrhage was the most frequent clinical presentation (45%). Three patients (27%) had multiple aneurysms, and seven aneurysms were fusiform or dysplastic. The internal carotid artery was the most affected site, involved in 47% of cases. Aneurysm size ranged from 2 to 60 mm (mean 16.8 mm), with giant aneurysms in 27%. Seven patients were treated with endovascular procedures, while three aneurysms were clipped. Symptomatic vasospasm requiring angioplasty occurred in two patients and led to worse outcomes. One patient died due to severe aspiration pneumonia and sepsis that precluded treatment. Good functional outcome (modified Rankin scale - mRS ≤ 2) was achieved in all treated patients (91%). CONCLUSION: The patients with aneurysms in this series were mostly male, presented mostly hemorrhagic syndromes, and mainly had internal carotid artery involvement. The outcome of treated patients was favorable, regardless of treatment modality.

Solitaire AB Stent Deployment for Treatment of Basilar Apex Aneurysm via the Posterior Communicating Artery.

AIM: To report the retrograde technique of horizontal stenting through the PCoA using a Solitaire AB stent. MATERIAL AND METHODS: A self-expandable stent was deployed from one posterior cerebral artery to the opposite, across the neck of a ruptured wide-neck basilar apex aneurysm. RESULTS: The technique allowed successful aneurysm embolization with coils in a 53-year-old woman. CONCLUSION: Differently from clipping, where the fetal posterior communicating arteries may represent an obstacle, in endovascular treatment it provides an alternative way to the aneurysm. The Solitaire AB stent is easy to navigate, fully retrievable and repositionable, which enables accurate deployment.

Chemical Angioplasty with Nitroglycerin for Vasospasm after Subarachnoid Hemorrhage: Case Series and Review

Introduction Vasospasm is a common and potentially devastating complication in patients with subarachnoid hemorrhage, causing high morbidity and mortality. There is no effective and consistent way to prevent or treat cerebral vasospasm capable of altering the morbidity and mortality of this complication. Animal and human studies have attempted to show improvement in aneurysmal vasospasm. Some sought their prevention; others, the treatment of already installed vasospasm. Some achieved only angiographic improvement without clinical correlation, others achieved both, but with ephemeral duration or at the expense of very harmful associated effects. Endovascular techniques allow immediate and aggressive treatment of cerebral vasospasm and include methods such as mechanical and chemical angioplasty. These methods have risks and benefits. Objectives To analyze the results of chemical angioplasty using nitroglycerin (GTN). In addition, to performa comprehensive review and analysis of aneurysmal vasospasm. Methods We describe our series of 77 patients treated for 8 years with angioplasty for vasospasm, either mechanical (with balloon), chemical (with GTN) or both. Results Eleven patients received only balloon; 37 received only GTN; 29 received both. Forty-four patients (70.1%) evolved with delayed cerebral ischemia and 19 died (mortality of 24.7%). Two deaths were causally related to the rupture of the vessel by the balloon. The only predictors of poor outcome were the need for external ventricular drainage in the first hours of admission, and isolated mechanical angioplasty. Conclusions Balloon angioplasty has excellent results, but it is restricted to proximal vessels and is not without complications. Chemical angioplasty using nitroglycerin has reasonable but short-lived results and further research is needed about it. It is restricted to vasospasm angioplasties only in hospitals, like ours, where better and more potent vasodilator agents are not available.

Moyamoya disease and syndrome: a review.

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Moyamoya disease and syndrome: a review / Doença e síndrome de moyamoya: uma revisão

Abstract Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

RESUMO A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Endovascular Treatment in the Subclavian Steal Syndrome: Series of 29 Patients

Subclavian steal syndrome is a group of symptoms resulting fromretrograde flow in the vertebral artery, "stealing" blood from the posterior intracranial circulation and other territories, caused by stenosis or occlusion of the subclavian artery proximal to the origin of the same vertebral artery, or even of the brachiocephalic trunk. Most of the time, it is an incidental finding in patients with other conditions or cerebrovascular risk factors. We report a series of 29 patients with an angiographic diagnosis, in which 7 received treatment (all endovascular), all with symptoms directly related to this condition. Advanced age, systemic arterial hypertension, diabetes mellitus, smoking and stroke were comorbidities frequently related. Six patients improved completely after the procedure and one remained with vertigo.

Matas test revisited: carotid compression for embolization of high-flow pediatric pial arteriovenous fistulas.

OBJECTIVE: Cerebral pial arteriovenous fistula (AVF) is a rare vascular malformation and may cause hemorrhage and neurological deficit. The presence of high-flow shunts constitutes a challenge when performing the endovascular technique, due to risk of distal embolization. The authors report a simple maneuver, adapted from the Matas test, that was successfully applied to treat a child with two pial AVFs. METHODS: An 8-year-old boy presented with headache and vomiting due to two single-channel high-flow intracerebral pial AVFs. He was treated with an endovascular approach using brief, gentle compression of the ipsilateral cervical carotid artery. The temporary flow arrest ensured proper placement of the first coil, allowing definitive obliteration of the shunt. RESULTS: There were no complications with the procedure, and the patient recovered uneventfully. Throughout the 9-month follow-up, the patient experienced a stable neurological condition, with both fistulas occluded and improvement of local circulation. CONCLUSIONS: This easy-to-perform maneuver allows precise positioning of embolic material into high-flow shunts to facilitate treatment of pial AVF.

Pediatric Cervical Aneurysmal Bone Cyst Treated by Endovascular Approach.

INTRODUCTION: Aneurysmal bone cyst (ABC) is a rare, benign, and expansible bone lesion, occurring mainly in childhood. Although most lesions are located at the metaphysis of long bones, they can also be found in flat bones and spine. CASE PRESENTATION: We report a case of a 16-year-old boy with cervical ABC treated by endovascular embolization. The afferents were occluded as a preoperative preparation for surgery; however, as the patient became asymptomatic after the procedure, the family refused surgery. Image at 1-year follow-up showed reduction and ossification of the lesion. CONCLUSION: Endovascular treatment decreases surgical morbidity by reducing blood loss, which is particularly important in the pediatric population. This therapeutic option may also halt ABC growth in selected cases.

Complications of Endovascular Treatment in Fibromuscular Dysplasia.

BACKGROUND: Fibromuscular dysplasia affects generally renal artery, causing renovascular hypertension. The most classical angiographic pattern, string-of-beads, can be found in cervical and more rarely in other arteries. With the advance of endovascular procedures techniques, the number of open surgeries is decreasing, and complications related to the selective catheterization of diseased vessels are increasing. CASE REPORT: A 37-year-old man presenting with subarachnoid hemorrhage was submitted to angioplasty for dissecting aneurysms of vertebral artery with a good outcome. Several arteries were angiographically diagnosed with the dysplasia (renal, carotid, femoral), and some complications like stenosis, dissection, arteriovenous fistula, and dissecting aneurysm occurred in sequence. CONCLUSIONS: FMD of cervical arteries is usually asymptomatic. There are no guidelines or protocols to cervical FMD treatment, being indicated only for the complications. Because of the vessels fragility, a several spontaneous or post endovascular procedure complications can be disastrous.

Prenatal Diagnosis of Vein of Galen Aneurysmal Malformation Allows Early Transumbilical Endovascular Treatment

Neonates with vein of Galen aneurysmal malformation (VGAM) presenting with severe cardiac failure and pulmonary hypertension represent a challenge for endovascular therapy.When early treatment is required, the small femoral arteries in this population are usually difficult to cannulate. Alternatively, the umbilical vessels offer a natural pathway to reach the lesion. Therefore, prenatal diagnosis of VGAM allows for delivery planning, perinatal management, and embolization through umbilical approach, thus leading to better outcomes.

Dural Arteriovenous Fistula Associated with Dental Implant.

BACKGROUND: Dural arteriovenous fistulas (DAVFs) are characterized by pathologic shunts between meningeal arteries and venous sinuses, without nidus. Although many lesions are considered idiopathic, there may be an association with inflammatory processes, including sinus thrombosis, venous hypertension, craniotomy, trauma, and infections. CASE DESCRIPTION: A 50-year-old woman with an infectious complication of a dental implant evolved with an occipital DAVF. To our knowledge, this is the first case of a dental implant-related DAVF reported in the medical literature. CONCLUSIONS: Physicians should be aware of the possibility of DAVF formation after craniofacial infections. Better understanding of the etiopathogenesis of this type of lesion is required to avoid and treat potential complications.

Cerebellar mutism after embolization of vermian arteriovenous malformation.

BACKGROUND: Cerebellar mutism is usually associated with posterior fossa tumor surgery. CASE REPORT: We report a case of a 17-year-old female, presented with headache and tremor after hemorrhage from a vermian arteriovenous malformation. She was successfully treated by embolization; however, on immediate postoperative, she developed persistent mutism. To the best authors' knowledge, this is the first case of cerebellar mutism after endovascular treatment reported in the medical literature. CONCLUSION: The endovascular approach may have the same potential of complication of conventional surgery; therefore, more study is necessary to clarify the role and limits of this technique to treat cerebellar arteriovenous malformation.

Ondine's curse: myth meets reality.

Ondine's curse is one of the most enchanting mythical tales in the field of Medicine. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. In one of the versions of the tale, when she caught her husband sleeping with another woman, she cursed him to remain awake in order to control his own breathing. During the 19th century, the rare syndrome characterized by loss of autonomic breath control, while voluntary respiration remains intact, was cleverly named "Ondine's curse". Nowadays, the term Ondine's curse is usually associated with congenital central hypoventilation syndrome; however, in medical literature, it also designates several respiratory disorders. Here, we present a review of the myth focused on history, arts and medicine.

Origin of the Posterior Inferior Cerebellar Artery over the C1 Posterior Arch.

A artéria cerebelar inferoposterior geralmente nasce do segmento intracraniano (V4) da artéria vertebral. Apesar de ter diâmetro médio de 2 mm, usualmente irriga áreas eloquentes do encéfalo. Quando ocluída, seja por trauma ou cirurgia, pode causar infarto no tronco encefálico e no cerebelo. Apresentamos um caso de artéria cerebelar inferoposterior com origem anômala no segmento cervical (V3) da artéria vertebral, demonstrado por angiografia. Os resultados foram registrados e comparados com os de publicações anteriores. Foram fornecidas breves explicações sobre a anatomia, anomalias vasculares e embriologia. A revisão da literatura mostrou que os ramos anômalos do segmento cervical da artéria vertebral são infrequentes e devem ser conhecidos. Uma melhor compreensão da anatomia e suas variações permite fazer um diagnóstico topográfico preciso, bem como planejar a abordagem e a terapia cirúrgicas ideais. O conhecimento dessa variação anatômica é essencial, pois, se confundida com um ramo muscular e coagulada, pode causar isquemia com sequelas incapacitantes.

Origin of the Posterior Inferior Cerebellar Artery over the C1 Posterior Arch / Origem da artéria cerebelar inferoposterior sobre o arco posterior de C1

Resumo The posterior inferior cerebellar artery usually arises from the intracranial segment (V4) of the vertebral artery. Despite its mean diameter of 2 mm, it usually irrigates important areas of the brain. When occluded, whether due to trauma or surgery, it may cause infarction in the brain stem and cerebellum. The present report describes a case of incidental finding of a posterior inferior cerebellar artery arising from the cervical segment (V3) of the vertebral artery, demonstrated by angiography. The findings were recorded and compared to those of earlier publications. Brief explanations regarding anatomy, vascular anomalies and embryology were provided. A literature review showed that anomalous branches of the cervical segment of the vertebral artery are infrequent andmust be known. A better understanding of anatomy and its variations enables an accurate topographic diagnosis, as well as the planning of the optimal surgical approach and therapy. Knowledge of this anatomical variation is essential because, if it is mistaken for a muscle branch and coagulated, this can cause ischemia and disabling sequelae.

Resumo A artéria cerebelar inferoposterior geralmente nasce do segmento intracraniano (V4) da artéria vertebral. Apesar de ter diâmetro médio de 2 mm, usualmente irriga áreas eloquentes do encéfalo. Quando ocluída, seja por trauma ou cirurgia, pode causar infarto no tronco encefálico e no cerebelo. Apresentamos um caso de artéria cerebelar inferoposterior com origem anômala no segmento cervical (V3) da artéria vertebral, demonstrado por angiografia. Os resultados foram registrados e comparados com os de publicações anteriores. Foram fornecidas breves explicações sobre a anatomia, anomalias vasculares e embriologia. A revisão da literatura mostrou que os ramos anômalos do segmento cervical da artéria vertebral são infrequentes e devem ser conhecidos. Uma melhor compreensão da anatomia e suas variações permite fazer um diagnóstico topográfico preciso, bem como planejar a abordagem e a terapia cirúrgicas ideais. O conhecimento dessa variação anatômica é essencial, pois, se confundida com um ramo muscular e coagulada, pode causar isquemia com sequelas incapacitantes.