Endovascular management of arteriovenous malformations and other intracranial arteriovenous shunts in neonates, infants, and children

Purpose

We discuss the management of cerebral arteriovenous shunts in neonates, infants, and children, with emphasis on our experience with pediatric cerebral arteriovenous malformations (AVMs). The management of vein of Galen malformations is discussed in a separate chapter.

Methods

An all-inclusive retrospective chart review of the endovascular surgery operative record database at the Hyman Newman Institute for Neurology and Neurosurgery at Roosevelt Hospital in NYC was conducted. All consecutive pediatric patients (newborn to 18 years of age) with intracranial arteriovenous shunts who presented from January 1, 2004 to June 16, 2009 were included.

Results

A total of 151 consecutive pediatric patients with intracranial arteriovenous shunts were evaluated from the period of January 1, 2004 to June 16, 2009. This included 56 patients with vein of Galen malformations, 48 cerebral AVMs, 11 patients with pial arteriovenous fistulae, six patients with dural arteriovenous malformations, and 30 patients with mixed intracranial vascular malformations. Forty-four patients underwent a total of 163 endovascular embolizations. The complications rate for endovascular embolizations was 6.7% (11 in 163), 5.5% with temporary complications and 1.2% with permanent complications. The mortality rate for the group of patients (excluding patients with vein of Galen malformations) that underwent endovascular embolizations was 0.0%.

Conclusions

Careful clinical observation and timely intervention are important in the management of pediatric patients with intracranial arteriovenous shunts. Trans-arterial endovascular embolization with liquid embolic agents is the treatment of choice for safe stabilization and/or improvement of symptoms in the group of pediatric patients with intracranial arteriovenous malformations.     

Vascular myelopathies-vascular malformations of the spinal cord: presentation and endovascular surgical management

Spinal cord vascular malformations are often a cause of spinal myelopathy. Endovascular surgical approaches to spinal cord vascular malformations have become an important adjunct and often the primary treatment of these disorders. Spinal cord vascular malformations may be divided into several categories based on their anatomy and location. They may present with a wide range of symptoms far removed from the vascular pathology. Early recognition and treatment can effect a better outcome and often reverse presenting neurologic deficits. Emerging magnetic resonance imaging techniques allow visualization of flow voids, some vascular anatomy, and intramedullary pathology and are a good screening modality. However, selective diagnostic spinal angiography remains the study of choice to diagnose and elaborate on the anatomy and potential treatment protocols. Rapidly evolving endovascular techniques and technology are revolutionizing the treatment of vascular pathology in the central nervous system, either as an adjunct to traditional neurosurgical techniques or as definitive therapy. We review the diagnosis and treatment options for spinal cord vascular malformations.     

Congestive cardiac manifestations from cerebrocranial arteriovenous shunts

Since 1984 we have been involved in the management of 30 children who had cardiac manifestations secondary to cerebrocranial arteriovenous shunts. Aneurysm malformation of the vein of Galen was the most common vascular lesion observed (73% of cases). In 77% of the patients the cardiac symptoms were the main presenting complaint. Medical treatment and/or endovascular therapy were indicated, depending on the age of the patients and the severity of the cardiac manifestations. Following embolization, the cardiac symptoms resolved (73%) or improved (18%) in 1 or 2 sessions. Mortality in the embolized group was 9%, and transient nonneurologic morbidity occurred in one case. Overall mortality, including four patients rejected for embolization, was 20%. These results compare favorably with medical and/or surgical management, alone or combined. The technique, challenges, indications and contraindications of endovascular therapy are discussed. Embolization represents an effective adjunct treatment to control, improve or cure the congestive cardiac manifestations caused by cerebrocranial arteriovenous shunts.Presented in part at the XVII Annual Meeting of the International Society for Pediatric Neurosurgery, Bombay 1989     

Delayed brain abscess after embolization of arterio-venous malformation: report of two cases and review of literature

Infections of central nervous system are rare complications of endovascular procedures. Review of literature revealed only four reported cases of brain abscesses after embolization of intracranial arteriovenous malformations (AVMs). We report two new cases of delayed brain abscess after embolization of AVM. In one of the patients, it was due to an unusual organism, Burkholderia caeci.     

Endovascular treatment of cerebrovascular disease

PURPOSE OF REVIEW: This review summarizes recent advances in endovascular therapy for cerebrovascular disease. RECENT FINDINGS: For ruptured aneurysms, the only large, randomized, controlled trial comparing surgical and endovascular treatment (the International Subarachnoid Aneurysm Trial) resulted in a significant reduction in death or dependency at 1 year, compared with surgery, providing sound evidence that coiling should be the treatment of first choice. Data from the International Study of Unruptured Intracranial Aneurysms demonstrated that treatment of unruptured anterior circulation aneurysms of under 7 mm with no history of subarachnoid hemorrhage is not justified. Embolization of arteriovenous malformations, as sole therapy, is curative only in a small percentage of cases, but can be part of a multimodal approach for reducing arteriovenous malformation size prior to surgery or radiotherapy. Partial treatment of complex arteriovenous malformations may be more dangerous than no treatment. Protection devices can reduce complication rates in carotid artery stenting, but scientific evidence is still lacking. Until the results of comparative trials are available, carotid artery stenting is indicated only in selected patients. Angioplasty and stenting of intracranial arterosclerotic disease is feasible but remains a high-risk procedure, indicated only in highly selected patients. In acute stroke therapy, new thrombolytics and clot-retrieval devices may result in better recanalization rates. SUMMARY: Advances in endovascular therapy have occurred in all areas of cerebrovascular disease. To obtain maximal patient benefit, endovascular treatment should be performed as an interdisciplinary approach in high-volume centers. Importantly, long-term follow-up review is necessary to clarify the overall role of endovascular treatment in the management of cerebrovascular disease.     

Endovascular treatment of a neonate with dural arteriovenous fistula and other features suggestive of cerebrofacial arteriovenous metameric syndromes

Introduction  Over the years, patients presented with cerebrofacial vascular malformations which occurred in clusters. The syndromic presentation suggested angio-architectural abnormality, which started to develop during embryogenesis. The segmentation of the neural tube could provide a rational explanation for such a neuropatholgical phenomenon. Based on this theory, cerebrofacial arteriovenous metameric syndrome (CAMS) was derived. This describes clinical association between arteriovenous malformations of the face, retina, and brain. In this case’s presentation, the syndromic presentation of the patient provided further evidence to the theory. Case report  The authors report a case of a male neonate presenting with heart failure at birth. He was found to have a left sigmoid dural arteriovenous fistula (dAVF), an upper lip arteriovenous malformation (AVM), and a capillary hemangioma over the nose. The neuropathological clustering of vascular lesions is suggestive of CAMS. The patient was successfully treated with endovascular treatment and showed normal development during our follow-up assessment. Conclusion  With the evolving evidence for the embryo-developmental theory for the clustering of angio-pathological lesions, further study and development of pathophysiology should be continued in following this track of theory. The success of endovascular intervention has warranted a favorable treatment option.     

Vertebro-epidural lumbosacral vascular malformations. An unusual cause of lumbo-sciatic pain

Sciatica is most commonly caused by nerve root compression secondary to herniated disk. Rarely, it can be due to a lumbosacral vascular malformation. We present five cases with such a malformation, presenting as a chronic lumboradiculagia. The patients were explored with computed tomography, MRI and selective spinal angiography. Polymorphic anatomic and hemodynamic aspect of these cases are reported: 1. One vertebral hemangioma with epidural extension; 2. Three purely epidural malformations: a) one epidural cavernous hemangioma, b) one epidural arteriovenous malformation, c) one epidural varix; 3. One paravertebral arteriovenous fistula with epidural venous drainage. Diagnosis of these rare malformations may be difficult. A multiplanar cross-sectional magnetic resonance and computed tomography scan with contrast enhancement can show characteristic abnormalities and may assist in recognition these malformations. Selective spinal angiography confirms the diagnosis, allows to classify the malformation and is required to evaluate endovascular therapeutic possibilities.     

Endovascular management of sigmoid sinus dural arteriovenous fistula associated with sinus stenosis in an infant

A 4-month-old female presented with a dural arteriovenous fistula (DAVF), which was successfully managed using endovascular techniques. There are very few case series reporting DAVF in infants younger than 12 months and, to our knowledge, only 60 pediatric patients with DAVF have been reported to date. Although most DAVF have a benign course, they can result in life-threatening hemorrhage. Endovascular therapies are usually indicated in the management of these neurosurgical vascular malformations. Endovascular therapy of DAVF in neonatal patients presents some major issues. Gaining arterial access may be problematic in femoral arteries too small for the introduction of a sizeable guiding catheter. The volumes of contrast and infused fluids must be carefully monitored to prevent fluid overload. Radiation exposure should be restricted as far as possible. This report contributes to the limited body of evidence on neonatal DAVF and its endovascular management.     

Central nervous system herpes simplex virus infection in afebrile children with seizures

Central nervous system herpes simplex virus infection is suspected in patients presenting with acute-onset seizures and lethargy. The potential neurologic sequelae from untreated herpes infection can prompt empirical acyclovir therapy, even in afebrile subjects. The objectives of this study were to determine the frequency of central nervous system herpes simplex virus infection in children presenting with afebrile seizures and to assess the need for empirical acyclovir therapy. Clinical and laboratory data of children with acute-onset afebrile seizures and children with central nervous system herpes simplex virus infection were compared. Polymerase chain reaction and viral cultures of the cerebrospinal fluid for herpes simplex virus infection were negative in all subjects with afebrile seizures; 32.7% of these subjects were empirically treated with acyclovir. In conclusion, central nervous system herpes simplex virus infection is uncommon in children presenting with afebrile seizures, and acyclovir therapy is rarely necessary in subjects with normal neurologic examination and cerebrospinal fluid analysis.     

脑脊髓血管造影4D成像技术及其在脑脊髓血管病中的应用

目的 探讨脑脊髓血管造影4D成像技术及其在脑脊髓血管病诊治中的应用价值。方法 回顾性分析2018年1月至2022年1月收治的57例脑脊髓血管病的临床资料。使用双平板DSA机行2D-DSA、3D-DSA、4D-DSA数据采集,再将原始成像数据传输到后处理工作站,利用成像软件重建动态全循环立体影像。结果 57例中,脑动静脉畸形37例,硬脑膜动静脉瘘10例,硬脊膜动静脉瘘5例,脊髓髓周动静脉瘘2例,脊髓动静脉畸形2例,硬脊膜外动静脉瘘1例。4D-DSA均做出正确诊断,指导显微手术或血管内治疗,取得满意效果。结论 4D-DSA对影像进行动态分析,提供更精准的影像,丰富了脑脊髓血管病诊断金标准（DSA含金量）,对脑脊髓血管病的精准治疗有指导价值。     

Arteriovenous malformations of the brain in children

A survey of 23 children treated for arteriovenous malformations of the brain is presented. Venous angiomas and aneurysms of the vein of Galen were excluded. Spontaneous hemorrhage was the first symptom in 83%. Angiographically occult arteriovenous malformations were found in 22% of cases. Contrast-enhanced CT failed to opacify abnormal vessels in most of these occult lesions. Fourteen patients underwent complete excision of their malformation with only 1 death (7.1% surgical mortality). Overall mortality for the group was 21.7%. Outcome was related to treatment modality and clinical condition on presentation. Four of 8 patients presenting in deep coma died, but 3 are normal and one slightly disabled. A spontaneous cerebral hemorrhage in a child is probably due to a vascular malformation, even when angiography and enhanced CT are negative. Optimal management consists of complete removal of the malformation; residual lesions tend to rebleed with fatal outcome. With aggressive treatment, complete recovery is possible even for children who present in coma.     

Endovascular treatment of spontaneous vertebral arteriovenous fistula in children: case report

Spontaneous cervical artery fistulas are rare arteriovenous malformations between the artery and veins of the neighborhood. We report a case of non traumatic vertebral arteriovenous fistula in a girl, aged 9 years, treated by endovascular approach. Under general anesthesia and with fluoroscopic guidance, using a endovascular technique, latex detachable balloons were used to successfully occlude the fistula. Complete clinical and angiographic recovery was achieved and no complications related to the embolization procedure occurred. The result obtained in this case corroborates that endovascular techniques, especially balloon occlusion embolization, has become the standard treatment for this vascular disorder.     

Intracranial arteriovenous malformations and radiosurgery with LINAC: review article

Among all vascular malformations, the intracranial arteriovenous malformations (AVM's) have the most powerful impact from the clinical point of view. The manifestations include hemorrhage, seizures, headacheh, but sometimes they are incidentally found during the diagnostic approach of patients with head trauma or chronic headache. There are three different types of treatments: microsurgery, endovascular treatment and radiosurgery. The actual role of the endovascular treatment is as an adjuvant therapy before microsurgery or radiosurgery just to diminish the nidus size. The goal of all treatments is complete nidus obliteration without causing a new neurological deficit. The overall obliteration index with LINAC based radiosurgery is about 80% and the result is dose, volume and time dependent. The mean dose reported in the literature fluctuates between 15 and 25 Gy, and the isodose coverage curve for the AVM with LINAC is generally the one of the 80%. There can be a treatment failure defined as the necessity to retreat the patient after three years from the first radiosurgical treatment in about 26% of the patients. There is a lack of evidence, principally from randomized trials, to point out the role of each of the modalities in the treatment of the AVM.     

Pros,cons, and current indications of open craniotomy versus gamma knife in the treatment of arteriovenous malformations and the role of endovascular embolization

The successful treatment of an intracranial arteriovenous malformation poses both technical and conceptual problems to the neurosurgeon. Treatment decisions are made in light of current understanding of the natural history of these lesions. It is important to understand the pros, cons and current indication of open craniotomy vs. gamma knife in the treatment of arteriovenous malformations and the role of endovascular embolization. Surgical removal of an arteriovenous malformation is indicated when the operative risk is less than the morbidity and mortality associated with its natural history. The treatment goal of complete angiographic obliteration of arteriovenous malformations is achieved most effectively by microneurosurgery in low-grade lesions. Large lesions frequently require a combination of embolization and microsurgery. Although recent advances in technology and medical management have allowed previously inoperable arteriovenous malformations to be surgically excised, there is still a small group of arteriovenous malformations that cannot be excised safely due to their size and location. Stereotactic radiosurgery is clearly an important adjunct in the multimodality treatment approach for large arteriovenous malformations. Endovascular embolization can potentially increase safety and efficacy in the treatment of arteriovenous malformations when applied to selective cases with well-defined treatment goals.     

Spinal vascular malformations--typical and atypical findings

Vascular malformations of the spinal cord and its meninges are rare diseases which comprise true inborn cavernomas and arteriovenous malformations (AVM), including perimedullary fistulae, glomerular and juvenile AVMs, and presumably acquired dural arteriovenous fistulae. This article gives an overview of the imaging features on magnetic resonance imaging (MRI) and digital subtraction angiography of both typical and atypical findings to describe the wide variety of possible pathological entities encountered. Clinical differential diagnoses, the neurological symptomatology and potential therapeutic approaches of these diseases, which may vary depending on the underlying pathology, are given. Although MRI constitutes the first choice diagnostic modality for suspected spinal vascular malformations, we conclude that the definite diagnosis of the disease and thus the choice of the appropriate therapeutic approach rests on selective spinal angiography which should be performed at a specialized center. Treatment in symptomatic patients offers an improvement in prognosis. Microsurgical treatment is recommended for symptomatic spinal cord cavernomas. Dural arteriovenous shunts can either be treated by microsurgical or endovascular approaches, the former being a simple, quick and secure approach to obliterate the fistula while the latter is technically demanding. In spinal arteriovenous malformations of both the fistulous and the glomerular type, the endovascular approach is the method of first choice; in selected cases, surgery or a combined therapy may be necessary.     

Galcn静脉动脉瘤样扩张——附8例报告

目的:报告8例Galen静脉动脉瘤样扩张(VGAD),并对其分型、血流动力学变化所致的临床症状及治疗进行分析和讨论.方法:自1986年5月～1996年5月,在4000例脑血管造影中检出VGAD8例,进一步检查:CT3例,MRI2例,全部行全脑血管造影.行栓塞治疗6例,栓塞加X-刀治疗1例,未治1例. 栓塞材料为IBCA或NBCA.结果:症状消失2例,好转4例,无效1例.结论:已证明此种栓塞技术对于VGAD的治疗是有效的,重要的是要了解VGAD的分型和有关血流动力学变化,常需多次栓塞.     

Aplasia cutis congenita and arteriovenous fistula. Case report and review

We describe a child with congenital aplasia cutis congenita of the scalp and an occult giant posterior fossa arteriovenous fistula. Previous case reports of central nervous system malformations associated with aplasia cutis congenita are reviewed. The exact incidence of such malformations is unknown. All patients with aplasia cutis congenita should undergo a neurologic evaluation, and their families should be examined for similar lesions. Early central nervous system imaging and other workup may be required, especially if plastic surgery in the head region is being planned.     

Arteriovenous malformations of the Galen vein: results of treatment

We performed retrospective and prospective analysis of surgical treatment of arteriovenous malformations of vein of Galen. Since 1987 till 2009 90 patients were operated in Burdenko Neurosurgical Institute. Age varied between 1 month and 38 years, 69 patients were children below 10. The paper describes main techniques for early diagnosis of the disease, its manifestation depending on age and type of malformation, treatment modalities, early postoperative and follow-up results. Endovascular treatment was applied in 75 (83.3%) patients. Complications with persistent neurological deficit after endovascular occlusion were observed in 10 (16%) patients, 2 patients died shortly after surgery. Conclusion: endovascular surgery is the treatment of choice in arteriovenous malformations of vein of Galen; optimal age for endovascular procedure is 4-5 months; observation in asymptomatic course is ineffective.     

Cerebral arteriovenous malformations in children

Over the past 10 years (1982–1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed (n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment (n = 56), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group (n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result.