Vertebrobasilar Dolichoectasia, Hypoplastic Third Ventricle, and Related Biventricular Hydrocephalus: Case Report and Review of the Literature

Abstract

Background Vertebrobasilar dolichoectasia (VBDE) is defined as a symptomatic dilatation and tortuosity of the vertebrobasilar arteries. The risk of hydrocephalus development is due to direct compression of the third ventricle outflow or brainstem compression and related aqueduct stenosis. We present an uncommon case of a patient with symptomatic VBDE with the uniqueness of a hypoplastic third ventricle associated with biventricular hydrocephalus. A literature review concerning diagnosis and management of patients affected by biventricular hydrocephalus caused by VBDE was also performed.

Case Illustration We report a case of a 54-year-old man who presented with headache, ideomotor apraxia, and gait disorder. A head computed tomography (CT) scan showed a biventricular hydrocephalus and a subsequent CT angiography documented the presence of a VBDE compressing the anterior part of the third ventricle that also appeared hypoplastic. The patient also presented a clinical history of arterial hypertension for which he was given a proper pharmacologic treatment with symptom relief. A surgical treatment of ventriculoperitoneal shunt along with endoscopic septostomy was proposed, but the patient refused, probably due to the slightly positive response to medical treatment.

Conclusions The natural clinical history of patients affected by VBDE is unfavorable with 7.8 years of median survival. The therapeutic strategy is usually conservative and the role of antiplatelets or oral anticoagulants is still debated. In selected patients, ventriculoperitoneal shunt to resolve intracranial hypertension caused by biventricular hydrocephalus is the most effective treatment. In our opinion, chronic third ventricle compression could lead to anatomic–pathologic alterations like the third ventricle hypoplasia documented in our report.

Keywords

hydrocephalus - biventricular - vertebrobasilar artery - dolichoectasia - third ventricle

Patient Consent

An informed written consent was obtained from the patient.

Publikationsverlauf

Eingereicht: 16. Juni 2020

Angenommen: 02. November 2020

Artikel online veröffentlicht:
02. Juni 2021

• References

• 1 Gutierrez J, Sacco RL, Wright CB. Dolichoectasia-an evolving arterial disease. Nat Rev Neurol 2011; 7 (01) 41-50
• 2 Adams Jr HP, Hachinski VC, Norris JW. Ischemic Cerebrovascular Disease. Oxford: Oxford University Press; 2001
• 3 Yuh SJ, Alkherayf F, Lesiuk H. Dolichoectasia of the vertebral basilar and internal carotid arteries: a case report and literature review. Surg Neurol Int 2013; 4: 153
• 4 Giang DW, Perlin SJ, Monajati A, Kido DJ, Hollander J. Vertebrobasilar dolichoectasia: assessment using MR. Neuroradiology 1988; 30 (06) 518-523
• 5 Smoker WR, Corbett JJ, Gentry LR, Keyes WD, Price MJ, McKusker S. High-resolution computed tomography of the basilar artery: 2. Vertebrobasilar dolichoectasia: clinical-pathologic correlation and review. AJNR Am J Neuroradiol 1986; 7: 61-72
• 6 Ubogu EE, Zaidat OO. Vertebrobasilar dolichoectasia diagnosed by magnetic resonance angiography and risk of stroke and death: a cohort study. J Neurol Neurosurg Psychiatry 2004; 75 (01) 22-26
• 7 Flemming KD, Wiebers DO, Brown Jr RD. et al. The natural history of radiographically defined vertebrobasilar nonsaccular intracranial aneurysms. Cerebrovasc Dis 2005; 20 (04) 270-279
• 8 Passero SG, Rossi S. Natural history of vertebrobasilar dolichoectasia. Neurology 2008; 70 (01) 66-72
• 9 Yuan YJ, Xu K, Luo Q, Yu JL. Research progress on vertebrobasilar dolichoectasia. Int J Med Sci 2014; 11 (10) 1039-1048
• 10 Ikeda K, Nakamura Y, Hirayama T. et al. Cardiovascular risk and neuroradiological profiles in asymptomatic vertebrobasilar dolichoectasia. Cerebrovasc Dis 2010; 30 (01) 23-28
• 11 Lou M, Caplan LR. Vertebrobasilar dilatative arteriopathy (dolichoectasia). Ann N Y Acad Sci 2010; 1184: 121-133
• 12 Passero S, Rossi S, Giannini F, Nuti D. Brain-stem compression in vertebrobasilar dolichoectasia. A multimodal electrophysiological study. Clin Neurophysiol 2001; 112 (08) 1531-1539
• 13 Pereira-Filho A, Faria M, Bleil C, Kraemer JL. Brainstem compression syndrome caused by vertebrobasilar dolichoectasia: microvascular repositioning technique. Arq Neuropsiquiatr 2008; 66 (2B): 408-411
• 14 El-Ghandour NM. Microvascular decompression in the treatment of trigeminal neuralgia caused by vertebrobasilar ectasia. Neurosurgery 2010; 67 (02) 330-337
• 15 Yang XS, Li ST, Zhong J. et al. Microvascular decompression on patients with trigeminal neuralgia caused by ectatic vertebrobasilar artery complex: technique notes. Acta Neurochir (Wien) 2012; 154 (05) 793-797 , discussion 797
• 16 Ebrahimzadeh K, Bakhtevari MH, Shafizad M, Rezaei O. Hydrocephalus as a rare compilation of vertebrobasilar dolichoectasia: a case report and review of the literature. Surg Neurol Int 2017; 8: 256
• 17 Huang L, Yu C-Y, Wang B-N, Zhang H-M, Li L-Y, Wang Y. Vertebrobasilar dolichoectasia causing a presentation resembling basilar-type migraine. Clin Neurol Neurosurg 2013; 115 (06) 784-786
• 18 Moreira I, Mendonça T, Monteiro JP, Santos E. Hypnic headache and basilar artery dolichoectasia. Neurologist 2015; 20 (06) 106-107
• 19 Breig A, Ekbom K, Greitz T, Kugelberg E. Hydrocephalus due to elongated basilar artery. A new clinicoradiological syndrome. Lancet 1967; 1 (7495): 874-875
• 20 Ekbom K, Greitz T, Kugelberg E. Hydrocephalus due to ectasia of the basilar artery. J Neurol Sci 1969; 8 (03) 465-477
• 21 Healy JF, Wells MV, Rosenkrantz H. Computed tomographic demonstration of enlarged, ectatic basilar artery associated with obstruction of the anterior third ventricle. Comput Tomogr 1981; 5 (04) 239-245
• 22 Ricci G, Lenzi J, Esposito V. Hydrocephalus caused by dolichoectatic basilar artery. Case report. J Neurosurg Sci 2000; 44 (03) 155-158
• 23 Weber MA, Steiner T, Fiebach J. Unusual cause of acute impairment of consciousness. Decompensated hydrocephalus caused by blockage of the foramini Monroi due to enlarged basilar artery. Radiologe 2002; 42 (10) 833-836
• 24 Thiex R, Mull M. Basilar megadolicho trunk causing obstructive hydrocephalus at the foramina of Monro. Surg Neurol 2006; 65 (02) 199-201
• 25 Seshadri R, Sadashiva N, Shukla D. et al. Vertebrobasilar dolichoectasia presenting as symptomatic obstructive hydrocephalus: a case report with review of literature. Indian J Neurosurg 2012; 1 (02) 165
• 26 Zisimopoulou V, Ntouniadaki A, Aggelidakis P, Siatouni A, Gatzonis S, Tavernarakis A. Vertebrobasilar dolichoectasia induced hydrocephalus: the waterhammer effect. Clin Pract 2015; 5 (02) 749
• 27 Mohammed K, Iqbal J, Kamel H, Mathew J, Al-Sulaiti G. Obstructive hydrocephalus and facial nerve palsy secondary to vertebrobasilar dolichoectasia: case report. Surg Neurol Int 2018; 9: 60
• 28 Oishi Y, Ueda R, Kuranari Y, Imanishi T. Vertebrobasilar dolichoectasia causing symptomatic hydrocephalus due to third ventricular compression. J Stroke Cerebrovasc Dis 2018; 27 (12) 3503-3504
• 29 Zhang DP, Peng YF, Ma QK, Zhao M, Zhang HL, Yin S. Why does my patient's basilar artery continue to grow? A four-year case study of a patient with symptoms of vertebrobasilar dolichoectasia. BMC Neurol 2018; 18 (01) 45
• 30 Schulz R, Fegbeutel C, Althoff A, Traupe H, Grimminger F, Seeger W. Central sleep apnoea and unilateral diaphragmatic paralysis associated with vertebral artery compression of the medulla oblongata. J Neurol 2003; 250 (04) 503-505
• 31 Passero S, Filosomi G. Posterior circulation infarcts in patients with vertebrobasilar dolichoectasia. Stroke 1998; 29 (03) 653-659
• 32 Marinescu M, Remy A, Dufour H, Derome P, Cambon H. A peculiar mechanism of hydrocephalus: the “water-hammering” effect. Neurochirurgie 1998; 44 (02) 117-120