Homonymous Hemianopia Secondary to A Long Fusiform Aneurysm of Posterior Cerebral Artery in A Patient with Connective Tissue Disease
Downloads
Highlight:
- Fusiform aneurysms are uncommon, accounting for only 1% of total intracranial aneurysms, and posterior circulation involvement is only 3-13% of cases of intracranial aneurysms.
- Connective tissue diseases are very rare as the cause of this vasculopathy.
- Homonymous hemianopia due to the mass effect or dilated blood vessels compressing the left optic tract.
ABSTRACT
Introduction: Fusiform aneurysms are uncommon, accounting for 1% of all intracranial aneurysms. Dissection and atherosclerosis are the main causes of this vasculopathy, but connective tissue disease is a very uncommon cause. Ehlers-Danlos Syndrome is the most common connective tissue disease, accounting for 11% of all cases. Symptoms depend on the location and size of the aneurysm, including headaches, blurred or double vision, and focal neurological deficits. Case: A 36-year-old man suddenly experienced blurred vision in both eyes on the right, starting with a chronic left-sided headache and no history of cardiovascular disease. In the confrontation test, Humphrey gave the right homonymous hemianopia. A head CT scan showed a lobulated lesion which showed enhancement in the left suprasellar region, and cerebral digital subtraction angiography (DSA) gave the impression of a long fusiform aneurysm L PCA. Clinically, the patient's skin on the left side of his face was darker than on the right, his skin was more elastic, and his blood vessels were wider and more prominent on the side of the fusiform aneurysm. Conclusion: Posterior circulation involvement is only 3-13% of cases of intracranial aneurysms. Many cases of intracranial aneurysms are not detected before rupture, resulting in delays in treatment. Surgical or endovascular surgery can be performed if the size is >10 mm and causes clinical symptoms. Symptoms of ischemia are managed with antiplatelets or anticoagulants. Incidentally detected unruptured aneurysms are generally managed conservatively because of the highly friable nature of the blood vessels in patients with connective tissue diseases.
Barletta E, Ricci R, Silva RG, Gaspar RML, Araújo JM, Neves MF, et al. Fusiform aneurysms: A review from its pathogenesis to treatment options. Surg Neurol Int. 2018;9(1):189.
Brown RD, Broderick JP. Unruptured intracranial aneurysms: epidemiology, natural history, management options, and familial screening. Lancet Neurol. 2014;13(4):393–404.
Kim S, Brinjikji W, Kallmes D. Prevalence of intracranial aneurysms in patients with connective tissue diseases: A retrospective study. Am J Neuroradiol. 2016;37(8):1422–6.
Kobkitsuksakul C, Somboonnitiphol K, Apirakkan M, Lueangapapong P, Chanthanaphak E. Dolichoectasia of the internal carotid artery terminus, posterior communicating artery, and posterior cerebral artery: The embryonic caudal ramus internal carotid segmental vulnerability legacy. Interv Neuroradiol. 2020;26(2):124–30.
Onofrj V, Cortes M, Tampieri D. The insidious appearance of the dissecting aneurysm: Imaging findings and related pathophysiology. A report of two cases. Interv Neuroradiol. 2016 Dec 20;22(6):638–42.
Serrone JC, Gozal YM, Grossman AW, Andaluz N, Abruzzo T, Zuccarello M, et al. Vertebrobasilar fusiform aneurysms. Neurosurg Clin N Am. 2014;25(3):471–84.
Ropper AH, Samuels MA, Klein JP. Cerebrovascular disease. In: Adams and Victor's Priciples of Neurology. 10th ed. McGraw Hill; 2014.
Kim ST, Brinjikji W, Lanzino G, Kallmes DF. Neurovascular manifestations of connective-tissue diseases: A review. Interv Neuroradiol. 2016;22(6):624–37.
Eder J, Laccone F, Rohrbach M, Giunta C, Aumayr K, Reichel C, et al. A new COL3A1 mutation in Ehlers-Danlos syndrome type IV. Exp Dermatol. 2013;22(3):231–4.
Debette S, Germain DP. Neurologic manifestations of inherited disorders of connective tissue. In: Handbook of Clinical Neurology. 2014. p. 565–76.
Campbell WW. The optic nerve. In: DeJong's The Neurologic Examination. 7th ed. Lippincott Williams & Wilkins, a Wolters Kluwer; 2013.
D'Arco F, D'Amico A, Caranci F, Di Paolo N, Melis D, Brunetti A. Cerebrovascular stenosis in neurofibromatosis type 1 and utility of magnetic resonance angiography: our experience and literature review. Radiol Med. 2013;119(6):415–21.
Lv X, Yang H, Liu P, Li Y. Flow-diverter devices in the treatment of intracranial aneurysms: A meta-analysis and systematic review. Neuroradiol J. 2016;29(1):66–71.
Awad AJ, Mascitelli JR, Haroun RR, De Leacy RA, Fifi JT, Mocco J. Endovascular management of fusiform aneurysms in the posterior circulation: The era of flow diversion. Neurosurg Focus. 2017;42(6):E14.
Bhogal P, Pérez MA, Ganslandt O, Bäzner H, Henkes H, Fischer S. Treatment of posterior circulation non-saccular aneurysms with flow diverters: A single-center experience and review of 56 patients. J Neurointerv Surg. 2017;9(5):471–81.
Matsumura H, Kato N, Fujiwara Y, Hosoo H, Yamazaki T, Yasuda S, et al. Endovascular treatments for posterior cerebral artery aneurysms and vascular insufficiency of fetal-type circulation after parent artery occlusion. J Clin Neurosci. 2016;32:41–6.
Briganti F, Leone G, Marseglia M, Mariniello G, Caranci F, Brunetti A, et al. Endovascular treatment of cerebral aneurysms using flow-diverter devices: A systematic review. Neuroradiol J. 2015;28(4):365–75.
Zhang Y, Tian Z, Sui B, Wang Y, Liu J, Li M, et al. Endovascular treatment of spontaneous intracranial fusiform and dissecting aneurysms: Outcomes related to imaging classification of 309 cases. World Neurosurg. 2017;98:444–55.
Copyright (c) 2022 Pinto Desti Ramadhoni, Asep Riswandi
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.