Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:
Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5526_Библиотеки_им_академика_М_И_Перельмана.pdf
Скачиваний:
0
Добавлен:
31.08.2026
Размер:
29 Мб
Скачать
180
C. M. Brito et al.

18.5 Clinical Presentation

Headache attributed to AVMs can manifest in various forms, making diagnosis chal­lenging. Presentations include hemorrhage (50% of cases), seizures (30% of cases), and headache (5–14% of cases) [1]. Some common characteristics of the headache include: The headache is usually ipsilateral to the AVM, although it may be bilateral in some cases. In some studies, headache was found to be ipsilateral to the AVM in all cases of migraine [11]. One study found a strong positive correlation (88.8%) between the side of the AVM and the side of pain [6].
The headache may be described as throbbing, pulsatile, or dull. The intensity of the headache can vary from mild to severe. Other neurological symptoms, such as seizures, focal neurological decits, and visual changes, may accompany the head­ache. Patients with occipital AVMs frequently present with visual symptoms, such as scintillating scotomas and hemianopsia [12]. It is important to note that head­aches attributed to AVMs can mimic primary headaches, such as migraine, making differential diagnosis essential. Headache associated with AVMs may present char­acteristics similar to those of migraine, including pulsatile quality and the presence of autonomic symptoms. Some studies have observed that a signicant proportion of patients with AVMs had a history of migraine [13]. A history of previous migraine may complicate the diagnosis, necessitating consideration of the possibility of sec­ondary headache due to AVM.
In a recent study, the risk factors associated with headaches in 946 patients with brain AVMs include female sex, supply artery dilation, and occipital lobe topog­raphy [3].

18.6 Diagnosis

The diagnosis of headache attributed to AVMs requires a systematic approach. It should follow the criteria established by the International Classication of Headache Disorders, 3rd edition (ICHD-3) (Headache Classication Committee of the International Headache Society, 2018) [14]:
A detailed history of the headache, including its characteristics, frequency, dura­tion, and associated symptoms, is essential. A complete neurological examination should be performed to identify any neurological decits.
Diagnostic criteria: According to ICHD-3, the diagnostic criteria for headache attributed to AVMs (code 6.3.2) are:
A. Any new headache fullling criterion C B. An arteriovenous malformation (AVM) has been diagnosed C. Evidence of causation demonstrated by at least two of the following:
1. The headache has developed in close temporal relation to other symptoms
and/or clinical signs of AVM, or has led to its discovery
18 Headache Attributed toArteriovenous Malformation
2. Either or both of the following:
(a) The headache has signicantly worsened in parallel with the worsening
of the AVM
(b) The headache has signicantly improved or resolved after effective
treatment of the AVM
3. The headache is localized to the site of the AVM
D. Not better accounted for by another ICHD-3 diagnosis
Thomsen etal. (2021) proposed criteria for “symptomatic migraine,” requiring evidence of a causal relationship between the underlying cause and migraine symptoms.
ICHD-3 emphasizes the importance of observing the evolution of headache over time, especially in relation to the diagnosis and treatment of AVM. Although not part of the formal diagnostic criteria, ICHD-3 notes that headache attributed to AVM is often described as pulsating and may be accompanied by other focal neuro­logical signs. ICHD-3 recognizes that, in rare cases, AVM may cause cluster head­ache, emphasizing the importance of considering secondary causes even in typical presentations of primary headaches.
181

18.7 Differential Diagnosis

It is essential to consider other causes of headache, including primary headaches such as migraine, which may coexist or be mimicked by headache attributed to AVM.Neuroimaging is crucial for conrming the presence of an AVM and exclud­ing other causes of headache. CT of the head is performed mainly when intracranial bleeding is suspected, which can be followed by CT angiography to diagnose the underlying arteriovenous malformation. Magnetic resonance imaging (MRI) with magnetic resonance angiography (MRA) is generally the preferred initial imaging examination. Catheter cerebral angiography is the gold standard for evaluating AVM anatomy and planning treatment. Cerebral angiography remains essential for surgical planning and detailed evaluation of the AVM [15].

18.8 Treatment

The treatment of headache attributed to arteriovenous malformations (AVMs) focuses on both alleviating pain and preventing serious complications, such as intra­cranial hemorrhage. Pain relief can be achieved through the use of analgesics, including nonsteroidal anti-inammatory drugs (NSAIDs) and, when necessary, opioids—though the latter should be used cautiously due to the risk of dependency. In some instances, prophylactic medications such as beta-blockers, tricyclic
182
C. M. Brito et al.
antidepressants, or anticonvulsants may be prescribed to reduce the frequency and severity of headache episodes. Management of the underlying AVM is guided by a careful assessment of the hemorrhagic risk versus the procedural risks associated with intervention [1]. Treatment strategies may include microsurgical resection, ste­reotactic radiosurgery (SRS), endovascular embolization, or a combination of these approaches. The selection of the most appropriate modality depends on multiple factors, including the AVM’s size, location, and vascular architecture, as well as the patient’s age and overall health condition.
Microsurgery is generally indicated for supercial, small- to medium-sized AVMs in non-eloquent areas of the brain [16].
Advantages Complete and immediate removal of the AVM, high cure rate (95–99%
in selected cases); immediate elimination of hemorrhage risk; relief of intracranial pressure in cases of associated hematomas.
Disadvantages Possibility of postoperative neurological decits. In cases of giant
occipital AVMs with frequent migraine-type headaches, surgical removal of the AVM may lead to headache resolution, allowing the patient to return to a normal life without the need for assistance [17]. A recent review and meta-analysis of 21 stud­ies comprising 5452 patients with unruptured arteriovenous malformations showed the highest rate of obliteration with microsurgery [18]. Another review and meta­analysis about morbidity and mortality pointed out that studies with more than 50% of arteriovenous malformations Spetzler-Martin grades 1–2 exhibited signicantly lower mortality rates compared to those with less than 50% cases of Spetzler-Martin grade 1–2.
Radiosurgery is often used for small (<3cm), deep, or AVMs in eloquent areas of the brain [19].
Advantages Non-invasive treatment and lower risk of immediate complications. It
is effective for AVMs in surgically difcult-to-access locations.
Disadvantages
Gradual obliteration of the AVM (2–3 years for complete effect)
and persistent risk of hemorrhage until complete obliteration, as well as the possi­bility of late radiation side effects. Limited efcacy for AVMs larger than 3cm. Endovascular embolization has proven to be an effective technique for reducing AVM size and decreasing the risk of hemorrhage [20]. Can be used as an adjuvant treatment before microsurgery or radiosurgery to reduce AVM size and facilitate denitive treatment.
Multimodal treatment, combining embolization, microsurgery, and radiosurgery, has gained popularity in recent years, especially for complex AVMs [21].
Advantages: Allows treatment of complex and large AVMs, reduces risks associ­ated with each modality, and can improve complete obliteration rates. Typical approach: Initial embolization to reduce AVM size and ow, followed by microsur­gery for removal of the majority of the lesion; radiosurgery to treat any residual remnant.
18 Headache Attributed toArteriovenous Malformation
183

18.9 Conclusion

Headache attributed to AVMs is a complex condition that requires a careful diag­nostic and therapeutic approach. Although a headache can be debilitating, success­ful treatment of the AVM can lead to the resolution or signicant improvement of the headache. Healthcare professionals must be aware of this entity to ensure proper diagnosis and treatment. The choice of treatment modality should be individualized, considering the characteristics of the AVM, clinical presentation, treatment team experience, and patient preferences. Multidisciplinary management, involving neu­rologists, neurosurgeons, and interventional radiologists, is crucial for optimizing clinical outcomes.

References

1. Abecassis IJ, Xu DS, Batjer HH, Bendok BR.Natural history of brain arteriovenous malfor­mations: a systematic review. Neurosurg Focus. 2014;37(3):E7.
2. Dasenbrock HH, Du R. Presentation of cerebral arteriovenous malformations. World Neurosurg. 2015.
3. Zhang H, Han H, Ma L, Li R, Li Z, Li A, Yuan K, Zhu Q, Wang C, Zhang Y, Zhang H, Gao D, Guo G, Kang S, Ye X, Li Y, Sun S, Wang H, Hao Q, Chen Y, Wang R, Chen X, Zhao Y; Registry of Multimodality Treatment for Brain Arteriovenous Malformation in Mainland China (MATCH). A comprehensive analysis of patients with cerebral arteriovenous malforma­tion with headache: assessment of risk factors and treatment effectiveness. J Headache Pain. 2024;25(1):72. PMC11075233
4. Laakso A, Hernesniemi J.Arteriovenous malformations: epidemiology and clinical presenta­tion. Neurosurg Clin N Am. 2012;23(1):1–6.
5. Hofmeister C, etal. Demographic, morphological, and clinical characteristics of 1289 patients with brain arteriovenous malformation. Stroke. 2000;31:1307–10.
6. Pereira Monteiro JM, Rosas MJ, Correia AP, Vaz AR.Migraine and intracranial vascular mal­formations. Headache. 1993;33:563–5.
7. Ellis JA, etal. Arteriovenous malformations and headache. J Clin Neurosci. 2016;23:38–43.
8. Thomsen AV, Sørensen MT, Ashina M, Hougaard A. Symptomatic migraine: a systematic review to establish a clinically important diagnostic entity. Headache. 2021:1–14.
9. Pereira L, Costa EC, Nunes T, Saraiva P, Ferreira J, Cruz P, Rodrigues M.Dynamics of a haemodynamic headache: a case report and literature review of headache secondary to ow inversion of the internal jugular vein. Cephalalgia. 2016;36(14):1370–8.
10. Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986;65(4):476–83. https://doi.org/10.3171/jns.1986.65.4.0476. PMID: 3760956.
11. Galletti F, et al. Occipital arteriovenous malformations and migraine. Cephalalgia. 2011;31:1320–4.
12. Kupersmith MJ, etal. Occipital arteriovenous malformations: visual disturbances and presen­tation. Neurology. 1996;46:953–7.
13. Waltimo O, Hokkanen E, Pirskanen R.Intracranial arteriovenous malformations and head­ache. Headache. 1975;15:133–5.
14. Headache Classication Committee of the International Headache Society (IHS). The International Classication of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211.
https://doi.org/10.1186/s10194- 024- 01774- 7. PMID: 38714978; PMCID:
184
15. Lawton MT, Rutledge WC, Kim H, Stapf C, Whitehead KJ, Li DY, Krings T, terBrugge K, Kondziolka D, Morgan MK, Moon K, Spetzler RF. Brain arteriovenous malformations. Nat Rev Dis Primers. 2015;1:15008. https://doi.org/10.1038/nrdp.2015.8. PMID: 27188382.
16. Bradac O, Charvat F, Benes V. Treatment for brain arteriovenous malformation in the 1998-2011 period and review of the literature. Acta Neurochir. 2014;155(2):199–209.
17. Nagata S, Morioka T, Natori Y, Sasaki T.Surgical treatment of non-ruptured Giant occipital arteriovenous malformations with frequent migraine-like headache—two case reports. Neurol Med Chir. 2006;46(9):441–5.
18. Liu R, Zhan Y, Piao J, Yang Z, Wei Y, Liu P, Chen X, Jiang Y.Treatments of unruptured brain arteriovenous malformations: a systematic review and meta-analysis. Medicine (Baltimore). 2021;100(25):e26352. https://doi.org/10.1097/MD.0000000000026352. PMID: 34160402; PMCID: PMC8238300.
19. Ding D, Yen CP, Xu Z, Starke RM, Sheehan JP.Radiosurgery for patients with unruptured intracranial arteriovenous malformations. J Neurosurg. 2013;118(5):958–66.
20. Weber W, Kis B, Ruefenacht D, Dietrich PY.Endovascular management of brain arteriovenous malformations: experience of 15 years. J Neurol. 2008;255(8):1179–89.
21. Javadpour M, Al-Mahfoudh R, Mitchell PS, Kirollos R.Outcome of microsurgical excision of unruptured brain arteriovenous malformations in Aruba-eligible patients. Br J Neurosurg. 2012;26(6):925–7.
C. M. Brito et al.
Chapter 19
Dural Arteriovenous Fistula (DAVF) Attributed Headache
EceYanık andDoğaVurallı

19.1 Introduction

Dural arteriovenous stulas (DAVFs) are rare vascular malformations characterized by pathological anastomoses between dural arteries and dural venous sinuses or cortical veins. These stulas often arise in the dura surrounding a venous sinus, characteristically at the transverse-sigmoid junction. However, they can also occur in the cavernous sinus, superior sagittal sinus, anterior fossa, tentorium, and other locations [1]. DAVFs may be completely asymptomatic. Symptoms may range from mild symptoms to fatal hemorrhage. One of the most common clinical symptoms is headache. Headache may be the rst or only presenting complaint [2].
In this chapter, we aim to dene headache phenotypes and develop early diagno­sis and treatment algorithms for DAVFs-related headache for future research by understanding the relationship between headache and DAVFs and establishing a high index of suspicion.
E. Yanık Department of Neurology and Algology, Neuropsychiatry Center, Gazi University Faculty of Medicine, Ankara, Türkiye
D. Vurallı ( Department of Neurology and Algology, Neuroscience and Neurotechnology Center of Excellence (NÖROM), Neuropsychiatry Center, Gazi University Faculty of Medicine, Ankara, Türkiye
Switzerland AG 2026 D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_19
*)
185© The Author(s), under exclusive license to Springer Nature
186
E. Ya n ık and D. Vurallı

19.2 Pathophysiology

Many mechanisms are involved in the pathophysiology of headache associated with DAVFs. These include venous hypertension, changes in intracranial pressure, and cortical and dural inammation [3].
Venous Hypertension: Impaired venous drainage leads to intracranial hyperten-
sion, causing diffuse or pressure-like headaches.
Cortical Venous Reux: When arterialized blood ows retrograde into cortical
veins, it can cause seizures, focal neurological decits, or hemorrhage, often
preceded by headaches.
Dural and Perivascular Inammation: Inammatory changes near the dura may
activate pain-sensitive structures, contributing to headache syndromes.
As a result, stimulation of pain-related structures in the dura, increased intracra­nial pressure, venous ischemia, or hemorrhage may result in severe thunderclap headache. Venous drainage is a critical determinant of the clinical presentation and risk prole.

19.3 Case Presentation

A 64-year-old male patient presented with a history of new-onset headache. The headache was unilateral, consistently localized to the right side, and progressively worsened over several months. He had no prior history of headaches with similar characteristics or severity. The visual analog scale (VAS) score for pain intensity was 8/10. The headache was throbbing, sometimes pulsatile, predominantly occipi­tal, and worsened at night. Additionally, the patient reported unilateral pulsatile tin­nitus on the right side for over a year.
Blood tests, as well as neurological and general clinical examinations, were unremarkable. Given the new-onset headache, accompanying symptoms, and the patient’s elderly age, further imaging was necessary. A cranial computed tomogra­phy (CT) scan, cranial magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) were performed that revealed diffuse, tubular, dilated venous vascular structures in the frontobasal and middle cranial fossa, extending toward the sinuses within the cisternal region of the posterior fossa. These ndings raised the suspicion of an arteriovenous stula, and digital subtraction angiography (DSA) was recommended for further evaluation.
Cerebral DSA conrmed the presence of a right-sided dural arteriovenous s­tula, characterized by early arterial phase blood supply from all branches of the external carotid artery and early venous drainage into the sigmoid sinus and jugular vein. Imaging obtained during the early venous and late arterial phases is presented in Fig. 19.1a–c. The patient underwent endovascular embolization, resulting in complete occlusion of the stula. At the two-month follow-up, he had no headache after the DAVF embolization.
19 Dural Arteriovenous Fistula (DAVF) Attributed Headache
187
a
b
c
Fig. 19.1 (a and b) Fistulized vessels in the late arterial phase. (c) Fistulized vessels in the early venous phase

19.4 Case Discussion

This case highlights the importance of thorough evaluation in elderly patients pre­senting with new-onset, progressive headaches, as such symptoms may indicate underlying vascular pathologies [1]. Recognizing red ag signs in headache history is crucial for early diagnosis. Pulsatile tinnitus, while nonspecic, is a frequently reported symptom in patients with DAVFs [4]. This case underscores the signi­cance of early identication and comprehensive management of DAVFs to prevent potential complications.
188
E. Ya n ık and D. Vurallı
Dural arteriovenous stula (DAVF)-related headaches are most commonly observed in middle-aged adults, with the median age of onset occurring in the fth to sixth decades of life [5]. These headaches are often part of a more aggressive clinical presentation, which may include the sudden onset of severe headache, sei­zures, and various forms of intracranial hemorrhage, such as intraparenchymal, sub­arachnoid, or subdural bleeding. The headaches associated with DAVFs are typically described as extremely severe and may present with a variety of patterns [6]. They are often localized to the occipital or frontal regions but can also be diffuse. A thun­derclap headache, characterized by a sudden and intense onset, may mimic sub­arachnoid hemorrhage and can signal cortical venous reux—necessitating urgent evaluation. Pulsating headaches are also common and may be accompanied by an audible bruit, reecting turbulent blood ow within the stula. Unilateral orbital or retroorbital headache is frequently associated with cavernous sinus DAVFs and is often accompanied by ocular signs, such as proptosis, conjunctival injection, or cranial nerve palsies. In some patients, headaches may worsen with changes in head position, indicating a disruption in venous drainage. Additionally, headaches associ­ated with pulsatile tinnitus—especially in cases involving the transverse or sigmoid sinuses—are a hallmark of DAVFs. Some cases may mimic chronic tension-type or migraine-like headaches, which can lead to misdiagnosis and delays in appropriate treatment. Notably, ocular symptoms in the context of nonmigrainous headache may suggest a carotid-cavernous stula, whereas migrainous features may point toward other DAVF locations. Pulsatile tinnitus and symptoms of increased dural sinus ow are particularly characteristic of stulas located in the transverse and sigmoid sinuses [7, 8].

19.5 Diagnostic Approach

The most common symptoms of DAVFs, including headache and tinnitus, are non­specic, making the diagnosis challenging [8]. Red ags that may suggest DAVFs­related headache are: New onset or progressive headaches in middle-aged or older adults, headache with cranial nerve palsies or other focal neurologic ndings, head­ache associated with pulsatile tinnitus, headache aggravated by Valsalva manoeu­vers or changes in head position [4].
The International Headache Society (IHS) has established criteria for classifying the headache attributed to DAVF (6.3.3), considering the following factors: (i) tem­poral relation to other symptoms/signs of DAVF; (ii) parallel prognosis between DAVF and headache (headache worsening concurrently with other symptoms or clinical/radiological signs of the DAVF and improving or resolving after the effec­tive treatment of the DAVF); (iii) association with other signs/symptoms (such as pulsatile tinnitus and ophthalmoplegia) and its clinical course (progressive, worse in the morning or worse due to a Valsalva’s maneuver, during coughing and/or bending over); (iv) exact localization of headache and site of DAVF [9]. Intracerebral
19 Dural Arteriovenous Fistula (DAVF) Attributed Headache
189
hemorrhage and cerebral venous thrombosis should be excluded before the diagno­sis of headache attributed to DAVF [9]. Additional imaging is essential. Unexplained subarachnoid or lobar hemorrhages should prompt consideration of a DAVF in the differential diagnosis [10].
Magnetic resonance imaging (MRI) helps dene the anatomy of DAVF.It can show dural thickening, thrombosis, stenosed venous sinuses, and hypertrophic arteries. In addition, complications such as diffuse white matter edema, dilated pial veins, infarction, and hemorrhage due to venous congestion are also seen [2]. Typical ndings include cortical or deep venous dilatation, cerebral edema, and stula-related microbleeds. Contrast-enhanced T1-weighted images may show dilated leptomeningeal and medullary vessels, venous ectasia, parenchymal enhancement, and venous sinus occlusion or thrombosis [1].
Magnetic resonance angiography (MRA), including time-of-ight (TOF) and contrast-enhanced techniques, is commonly used for noninvasive evaluation of vas­cular structures. TOF MRA is capable of visualizing arterialized veins, while dynamic contrast-enhanced MRA can reveal arteriovenous shunting and venous drainage patterns [11]. However, a notable limitation of MRA is its relatively lower spatial resolution compared to conventional angiography [12]. Computed tomogra­phy (CT), on the other hand, plays a key role in identifying complications such as intracranial or subarachnoid hemorrhage, hydrocephalus, and vasogenic edema [1]. CT may also reveal nonspecic ndings such as asymmetry in the dural venous sinuses or mild dilatation of the cavernous sinus or superior ophthalmic vein; how­ever, it is generally insufcient for detecting intracranial dural arteriovenous stulas (DAVFs) on its own [12]. Computed tomography angiography (CTA) is a more effective modality for detecting DAVFs, as it can demonstrate arterialized veins, abnormal venous drainage patterns, and dilated venous sinuses [13]. Despite CTA’s improved spatial resolution over MRA, digital subtraction angiography (DSA) remains the gold standard for denitive diagnosis and classication. DSA provides comprehensive information about the presence, location, and hemodynamic fea­tures of DAVFs, including identication of arterial feeders, venous drainage direc­tion, venous ectasia, stenosis, arteriovenous shunts, cortical venous collaterals, calcications, and involvement of pial and dural branches [2].

19.6 Management

Management of DAVF-related headache depends on the severity of symptoms, the presence of cortical venous reux, and associated comorbidities [3, 8, 10].
– Conservative management: Asymptomatic or low-risk DAVFs can be monitored
with regular imaging and symptomatic headache management.
– Endovascular treatment: The primary treatment modality for symptomatic or
high-risk DAVFs is endovascular treatment using transarterial or transvenous
embolization.