Kerala Journal of Ophthalmology

CASE REPORT
Year
: 2016  |  Volume : 28  |  Issue : 2  |  Page : 139--141

Vertebrobasilar dolichoectasia


V Babitha, C Prasannakumari, KV Raju 
 Department of Ophthalmology, Govt Medical College, Kozhikode, Kerala, India

Correspondence Address:
V Babitha
Department of Ophthalmology, Govt Medical College, Kozhikode - 673 008, Kerala
India

Abstract

Dolichoectasia is the increase in the length and diameter of vessels. It is a rare disease effectin g major vessels. For an ophthalmologist awareness of this entity is important to avoid unwanted investigation since this disease present as external ophthalmoplegia.



How to cite this article:
Babitha V, Prasannakumari C, Raju K V. Vertebrobasilar dolichoectasia.Kerala J Ophthalmol 2016;28:139-141


How to cite this URL:
Babitha V, Prasannakumari C, Raju K V. Vertebrobasilar dolichoectasia. Kerala J Ophthalmol [serial online] 2016 [cited 2023 Feb 8 ];28:139-141
Available from: http://www.kjophthal.com/text.asp?2016/28/2/139/202478


Full Text



 Introduction



Vertebrobasilar dolichoectasia is the increase in the length and diameter of vertebrobasilar arteries.[1],[2],[3] The incidence is 0.06–5.8%.[4] Associated internal carotid artery and aortic enlargement can also be seen. If this disease presents with ocular motor nerves palsy, ophthalmologist is the first person to evaluate the clinical signs of this rare entity. Even though it may rarely be fatal due to stroke and intracranial bleeding, it can be managed conservatively.[5] Awareness about this rare entity can avoid unwanted investigation. A case of a 65-year-old hypertensive male patient with vertebrobasilar dolichoectasia is presented here.

 Case Report



A 65-year-old hypertensive male patient presented with diplopia of 2-month duration. He was pseudophakic both eyes (right 8 years and left 2 months). No other ocular or systemic disease or trauma in the past were reported.

Examination showed alternate esotropia of 15° [Figure 1]. He had bilateral abduction restriction, right more than left, and all other extraocular movements were within normal limits. Both pupils were irregular and reacting. Best corrected visual acuity was 20/20 for distance and 0.5 Snellen for near for both eyes. Fundus examination was normal. System examination including central nervous system was within normal limits except for bilateral abducent nerves paralysis. Magnetic resonance imaging showed vertebrobasilar dolichoectasia causing indentation over cisternal segment of the right abducent nerve [Figure 2]. All other investigations were within normal limits. Patient was managed conservatively with systemic antihypertensives and improved. Patient was followed up for 6 months.{Figure 1}{Figure 2}

 Discussion



Vertebrobasilar dolichoectasia is also known as megalodolichoectasia, fusiform aneurysm of vertebrobasilar artery, or tortuous vertebrobasilar system. Dysfunction of metalloproteinases [6] in the vessel wall and irregular turbulent flow in the vessel leads to irreversible disruption of internal elastic lamina of tunica intima and tunica media occasionally.[2],[7] This leads to weakening of vessel wall and elongation and distension of vessel. Early fragmentation of internal elastic lamina, intimal hyperplasia, and intramural hemorrhage can be seen in vessel wall. Blood flow in arteries in both orthograde and retrograde direction leads to significant obstacle to circulation and thrombus formation. Multiple other risk factors such as congenital factors, infections, immune status, and degenerative diseases are involved in the development of vertebrobasilar dolichoectasia.[3]

Most of the patients are asymptomatic; only <10% patients present with neurological symptoms caused by compression of adjacent structures, and involvement of one of the ocular motor nerves is rare. According to Titlic et al.,[4] the descending order of cranial nerve involvement is facial nerve, trigeminal nerve, vestibulo cochlear nerve, and abducent nerve. Ischemic symptoms because of effect on the brainstem and cerebellar hemisphere and features of obstructive hydrocephalus [8] may be present. Rarely, compression of optic tract and progressive visual loss, trigeminal neuralgia,[9] hemifacial spasm, tic douloureux, pulsating tinnitus, and recurrent vertigo may be present.[4],[10] There is an increased risk of posterior circulation dysfunction and intracranial haemorrhage.[4]

The important risk factors are hypertension, hyperlipidemia, increasing age, male sex, previous history of myocardial infarction, and lacunar infarct.[2],[4] Patients with autosomal dominant polycystic kidney disease has an association with vertebrobasilar dolichoectasia.[11]

Conventional intraarterial digital subtraction angiography is the gold standard method of imaging of vertebral artery.[4] MRI and MRA are more sensitive and specific whereas CTA can be used for screening.[12] Diagnosis of posterior circulation dolichoectasia is based on Smoker's criteria [2] [Table 1].{Table 1}

Treatment is conservative with lifestyle modification including dietary changes, weight loss, regular exercise, and antihypertensives such as thiazides, betablockers, and ACE inhibitors.[7]

 Conclusion



Dolichoectasia in our case is different because of its presentation with bilateral abducent nerve palsy, which is the only manifestation of this rare, unilateral condition in this patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Pico F, Labreuche J, Cohen A, Touboul PJ, Amarenco P; GENIC investigators. Intracranial arterial dolichoectasia is associated with enlarged descending thoracic aorta. GENIC investigators. Neurology 2004;63:2016-21.
2Gutierrez J, Sacco RL, Wright CB. Dolichoectasia-an evolving arterial disease. Nat Rev Neurol 2011;7:41-50.
3Yuan YJ, Xu K, Luo Q, Yu JL. Research progress on vertebrobasilar dolichoectasia. Int J Med Sci 2014;11:1039-48.
4Titlic M, Tonkic A, Jukic I, Kolic K, Dolic K. Clinical manifestations of vertebrobasilar dolichoectasia. Bratisl Lek Listy 2008;109:528-30.
5Debette S, Compter A, Labeyrie MA, Uyttenboogaart M, Metso TM, Majersik JJ, et al. Epidemiology, pathophysiology, diagnosis, and management of intracranial artery dissection. Lancet Neurol 2015;14:640-54.
6Pico F, Jacob MP, Labreuche J, Soufir N, Touboul PJ, Benessiano J, et al. Matrix metalloproteinase-3 and intracranial arterial dolichoectasia. Ann Neurol 2010;67:508-15.
7Pico F, Labreuche J, Amarenco P. Pathophysiology, presentation, prognosis, and management of intracranial arterial dolichoectasia. Lancet Neurol 2015;14:833-45.
8Zisimopoulou V, Ntouniadaki A, Aggelidakis P, Siatouni A, Gatzonis S, Tavernarakis A. Vertebrobasilar Dolichoectasia Induced Hydrocephalus: The Water-Hammer Effect. Clin Pract 2015;5:749.
9Alcalá-Cerra G, Gutiérrez-Paternina JJ, Niño-Hernández LM, Moscote-Salazar LR, Polo Torres C, Sabogal Barrios R. Brain stem compression preceded by trigeminal neuralgia in a patient with vertebro-basilar and bilateral carotid dolichoectasia. Bol Asoc Med P R 2011;103:34-7.
10Baquero M, Yayá-Huamán R. Vertebrobasilar dolichoectasia. Rev Neurol 1998;26:143-8.
11Graf S, Schischma A, Eberhardt KE, Istel R, Stiasny B, Schulze BD. Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant 2002;17:819-23.
12Akgun V, Battal B, Bozkurt Y, Oz O, Hamcan S, Sari S, Akgun H. Normal Anatomical Features and Variations of the Vertebrobasilar Circulation and Its Branches: An Analysis with 64-Detector Row CT and 3T MR Angiographies. ScientificWorldJournal 2013;2013:620162.