Intracranial Arteriovenous Malformations

Intracranial arteriovenous malformations (AVMs) are a type of intracranial vascular malformation that consists of an abnormal connection of arteries and veins without intervening capillary beds. AVMs may come to medical attention because of seizure, intracranial hemorrhage, or incidental radiographic findings in a patient undergoing brain imaging for a different indication. This chapter focuses on the epidemiology, natural history, diagnosis, and management of intracranial AVMs.

A dural arteriovenous fistula associated with an encephalocele presenting as otitis media with effusion

ObjectiveThis case report illustrates an unusual case of a dural arteriovenous fistula and an associated encephalocele presenting as otitis media with effusion.Case reportA 53-year-old man presented with right-sided hearing loss and aural fullness of 2 years’ duration. Examination revealed ipsilateral post-auricular pulsatile tenderness. Computed tomography showed transcalvarial channels suggestive of dural arteriovenous fistula. Further magnetic resonance imaging demonstrated the presence of a temporal encephalocele herniating through the tegmen tympani defect, as well as the abnormal vascularity. Angiography confirmed a Cognard type I dural arteriovenous fistula, which is being managed conservatively. Surgical repair of the encephalocele was recommended but declined by the patient.ConclusionDural arteriovenous fistula is an uncommon intracranial vascular malformation rarely seen by otolaryngologists, with pulsatile tinnitus being the usual presentation. To our knowledge, this is the first reported case of dural arteriovenous fistula presenting with conductive hearing loss and otalgia.

Vein of Galen Malformation

Vein of Galen malformation (VGM) is a high-flow intracranial vascular malformation, presenting in utero or after birth with a range of initial symptoms. The natural history of untreated VGM is grave, with progressive permanent neurological dysfunction, developmental delay, seizures, intracranial hemorrhage, and death. Presentation varies with age, with more severe manifestations resulting in earlier presentation and poorer outcome. The mainstay of treatment is medical stabilization followed by endovascular embolization. The goal is to reduce flow through the malformation such that normal development can proceed without neurological deficit. The past 20 years have seen great advances both in the technical treatment of the vascular lesion and in the overall prognosis of patients treated for this condition. Key to a good outcome is appropriate selection of patients and treatment by physicians familiar with this pathophysiology.

Vein of Galen aneurysmal malformation—clinical and angiographic spectrum with management perspective: an institutional experience

Background and purposeVein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically heterogeneous set of VGAM patients.MethodsWe retrospectively analyzed cases of VGAM from our departmental archive collected between 1988 and January 2015. Demographic, clinical, therapeutic, and follow-up details were obtained for each patient from the available records.ResultsWe identified 36 patients with VGAM including 6 neonates, 18 infants, 7 children aged 2–10 years, and 5 adults. Macrocrania was the commonest presenting feature. Type of fistulae was mural in 14 and choroidal in 18 patients while 4 had a thrombosed sac at presentation. In 3 cases the dilated venous sac had connection with the deep venous system. Bilateral jugular atresia and stenosis were seen in 9 and 6 patients, respectively. Giant venous sac (>4 cm) was significantly correlated with mural type (p=0.0001). Dural arterial recruitment was seen in 4 patients including 3 adults. Among the 23 patients treated by endovascular means, 14 had a good outcome, 5 had a poor outcome, and 4 died. A significant correlation was noted between jugular atresia and poor outcome (p=0.003).ConclusionsWe encountered a wide range of demographic, clinical, and angiographic features in VGAM. Mural type malformations were associated with giant venous sacs. Good outcome after embolization was seen in selected neonates and in most of the infants, children, and adults. Jugular atresia was significantly associated with poor outcome.

Vertex scalp mass as presenting sign of a complex intracranial vascular malformation

Scalp masses are not infrequently encountered in daily clinical practice. They are represented by a wide spectrum of different clinical entities and are usually managed by an excision or by simple observation. Although it happens rarely, head lumps may hide an underlying cranioencephalic malformation that has to be preoperatively diagnosed to perform an appropriate treatment. Cerebral arteriovenous malformations (AVMs) are not included among the intracranial malformations connected with a scalp mass. The authors report on the unusual case of a child harboring a complex intracranial AVM that initially presented as a small scalp mass. Actually, this young boy came to the authors' attention just for a small, soft, pulsatile, and reducible mass of the vertex that produced a circumscribed bone erosion. The presence of macrocranium and venous engorgement of the face, however, suggested the presence of an intracranial “mass.” The neuroimaging investigations pointed out a temporal AVM causing dilation of the intracranial sinuses and ectasia of the vein of the scalp; one of the veins was appreciable as a lump on the vertex.