Fibromuscular dysplasia of cervico-cephalic arteries with multiple dissections and a carotid-cavernous fistula. A pathological study.

Abstract

A rare case of widespread fibromuscular dysplasia (F.M.D.) is reported, involving the cervico-cephalic arteries associated with multiple dissections, saccular aneurysms and a carotid-cavernous fistula. A detailed post-mortem examination revealed FMD involvement of the intracranial vessels, not demonstrated by arteriography.

Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions

“…Multiple cervical dissections may occur simultaneously or within a short period of time. [67][68][69][70][71] Persistent headache severe enough to interfere with the quality of life may occur in up to 17% of patients after a cervical artery dissection. 72,73 In a study of 200 patients who developed a spontaneous cervical artery dissection, recurrent dissection occurred in 8 patients (2%) within a month after the first dissection and between 1.4 and 8.6 years later in 12 patients (1%/y).…”

Internal carotid artery dissection as a cause of severe ischemic stroke with lethal outcome

“…Это свидетельствует о том, что обнаруженные изменения артериальной стенки не были следстви-ем диссекции, а предшествовали ей, отражая нали-чие артериопатии, которая и предрасполагала к дис-секции. В целом изменения ВЭМ (истончение, рас-щепление, отсутствие на отдельных участках) и ме-дии (участки фиброза, очевидно, на месте погибших миоцитов, нарушение ориентации миоцитов с обра-зование «клубков», снижение числа эластических волокон, нарушение их ориентации на некоторых участках, гиперэластоз) аналогичны таковым при фиброзно-мышечной дисплазии (ФМД) [20][21][22]. Ранее мы уже отмечали это в проведенном клинико-морфологическом исследовании при диссекции [10].…”

Combined Arterial and Venous Approaches for Cure of Carotid-Cavernous Sinus Fistula in a Patient with Fibromuscular Dysplasia

“…On (2), the inferior petrosal sinus (3), and the patent pterygoid venous plexus (4) that courses inferiorly through the skull base. B: Right internal carotid angiography performed shortly before embolization, after the onset of severe headache and worsening of ocular signs of venous hypertension.…”

“…Shortly thereafter, the catheter position was lost and further attempts to regain it were unsuccessful. Although CCFs are often effectively treated by sinus occlusion by transarterial delivery of a detachable balloon device,7-9 the high risk of inadvertent balloon detachment [2], inferior petrosal sinus [3], pterygoid plexus [4] SKULL BASE SURGERYNOLUME 4, NUMBER 2 APRIL 1994 related to the observed proximal carotid artery stenoses prevented its use for fistula closure in this case.…”

“…When dissection involves the intracavernous segment of the ICA, a fistula may develop between the artery and the cavernous sinus (carotid-cavernous fistula [CCF]). [1][2][3] FMD may predispose to the development of bilateral fistulas, which present added therapeutic difficulties. 4,5 The following case study illustrates some of the difficulties involved in treating such patients and how transvascular embolotherapy must incorporate a flexible approach for access, selection of embolic materials, and clinical care.…”