Download Grand Rounds Case Report I. Case History 64 year old male

Grand Rounds Case Report
I. Case History
 64 year old male complains of decreased vision at near for the past year with current
glasses
 No history of trauma, injury, or surgery to eyes
 Medical History:
 Family history of malignant neoplasm of prostate
 Hypertension
 Alcohol Abuse
 Arthralgia of knee
 Current medications:
 IBUprofen 800mg
 Lisinopril 40mg
 Atenolol 25 mg
 HCTZ 25 mg
 Amlodipine Besylate 10 mg
II. Pertinent findings
 Best Corrected Visual Acuity
 Distance OD 20/20-2
OS 20/25
 Anterior Segment Findings:
 Pupils: Equal, round, reactive to light
 2+ Afferent Pupillary Defect OS
 Lens OD: 1+ Nuclear Sclerosis
OS: 1+ Nuclear Sclerosis
 Tonometry OD: 13mmHg
OS: 14mmHg
 Color Vision: (Ishihara Plates) OD: 11/14 OS: 7/14
 Red Cap Comparison OD 100% saturation OS 60% saturation
 Posterior Segment Pertinent Findings
 OD: .4/.4 round healthy rim tissue
 OS: .5/.5 round with temporal pallor and possible diffuse pallor 360

OCT- Results Repeatable
o OD: normal
o OS: thinning superior, inferior, temporal
o OU: correlates with nerve appearance

Humprey Visual Field 24-2
o OD: reliable field 0/11 Fixation losses; Cluster defect present superior temporal ,
edge point depression nasally
o OS: reliable, 2/12 Fixation losses; defect inferior nasal; defect corresponds to
location of the optic nerve pallor and compression within the optic tracts

Imaging: MRI of orbits
o There is marked dolichoectasia of the basilar artery which loops superiorly
displacing the optic tracts immediately posterior to the optic chiasm. The right
optic tract is superiorly displaced and compressed by the tortuous artery whereas
the left optic tract is superiorly displaced.
o There is associated atrophy of the optic nerves, left greater than right. The left
nerve has a thickened nerve sheath surrounding it compared to the right nerve.
III. Differential diagnosis
 Optic neuropathy
 Optic nerve and chiasmal compressive lesions
 pituitary adenoma, meningioma, glioma, thyroid ophthalmopathy
 Infiltrative lesions
 sarcoidosis, lymphomas, leukemias
 Normal tension glaucoma
 Prior trauma
IV. Diagnosis and discussion
 Dolichoectasia-Optic Atrophy due to displacement of the optic tracts by the vetebral
basilar arteries
 Monitor patient 4-6 months with visual field. Currently there is no treatment for
dolioectasia.
 Optic nerve is vulnerable to compression in the areas surrounded by bone leaving it
immobile causing a slowly progressive visual and field loss with accompanying afferent
pupillary defect
 Incidence is 4 cases per 100,000 per year.2 Most cases are due to thyroid
ophthalmopathy.
 Visual field defects2:
 Compression of the optic nerve anterior to the chiasm include enlarged blind spot,
relative central scotoma, and constriction.
 Compression of the optic chiasm, a bitemporal hemianopia usually is found,
although unilateral field loss or homonymous hemianopia may occur if the lesion
is prefixed or postfixed.
 Pathophysiology: elongation and dilation (>4.5mm)9 of the vertebrobasilar artery or
internal carotid arteries 6
 Can lead to compression or ischemia of adjacent structures or cause thrombotic
occlusions
 Cranial neuropathy can present as multiple or isolated (26%) 6 nerve palsies on
one or both sides
 Cranial Nerves most commonly affected6: V and VII; affected less
commonly: III,IV, VI, and VIII
 Theory of Mechanism 5:
 Upper cervical spinal/neck trauma or severe systemic hypertension with
consequential arterial dissection
 Vertebrobasilar dolichoectasia (VBD) induced aneurysmal formation and rupture
 Blood flow within the dilated arteries is orthograde and retrograde leading
to the risk of thrombus formation 7
 Brainstem compression due to worsening vessel enlargement and tortousity
 Tortous, ectatic basilar artery can cause elevation and deformity of the
hypothalamus, chiasm and third ventricle compression, distortion of cerebellar
tonsils and cranio-cervical junction 6
 Incidence of Intracranial Dolichoectasia7: .06-5.8%
 Risk factors: older age, male, hypertension, history of myocardial infarction or lacuna
infarction 7
 Ocular Signs:
 Monocular Impaired color vision
 Visual field defects: none, congruent or incongruent homonymous
hemianopia, or bitemporal hemianopia8
 Vertical Diplopia (rare)
 Cranial nerve palsy (V and VII – most common; III, IV, VI, VII – rare)6
 Decreased brightness perception in affected eye
 Optic nerve head pallor
 Ocular Symptoms:
 Transient ischemic attack (50%)6
 Gradual decrease in vision
 Systemic Signs:
 Hemifacial Spasm
 Paresis
 Trigeminal neuralgia
 Clinical Conditions associated4:
 Isolated or combined brainstem/cranial nerve syndromes
 Cervicomedullary junction compression
 Transient or permanent motor deficits
 Cerebellar dysfunction
 Central sleep apnea
 Hydrocephalus
 Ischemic stroke
 Outcome
 A small case study found the survival rate in vertebrobasilar dolichoectasia after
3 years follow up was found to be 60%.5
 Another study stated that long-term prognosis depended mainly on the severity of
the condition at the time of diagnosis and its evolutionary characteristics.11
V. Conclusion
 Very limited data is available regarding basilar artery dolichoectasia and compression of
the optic tract or optic chiasm
 Evidence of cranial nerve palsies involving III, IV, V, VI, VII, and VII have been shown
in case reports
 Visual field defects vary from none, scattered, bitemporal hemianopia, or homonymous
hemianopia
 Prognosis uncertain with risk of stroke
VI. Bibliography, literature review encouraged
1. Pakrou, N, Craig, J. Idiopathic Sclerochoroidal Calcification in a 79-year-old woman.
Clinical & Experimental Ophthalmology 2006 Jan;34(1):76-78
2. Gandhi R. Optic Atrophy. Updated: Sep 28, 2009.
http://emedicine.medscape.com/article/1217760-overview
3. Pituitary tumors. 1998-2009 Mayo Foundation for Medical Education and Research .
http://www.mayoclinic.com/health/medical/IM02722
4. Adams HP Jr. Secondary prevention of atherothrombotic events after ischemic stroke.
Mayo Clin Proc. 2009;84(1):43-51.
5. Zaidat O, Ubogu E. Vertebrobasilar dolichoectasia diagnosed by magnetic resonance
angiography and risk of stroke and death: a cohort study. J Neurol Neurosurg Psychiatry
2004;75:22–26.
6. Kawasaki A, Purvin, V. Isolated Ivth (Trochlear) Nerve Palsy due to Basilar Artery
Dolichoectasia. Klin Monatsbl Augenheilkd 2006;223:459-461.
7. Titlic M, Tonkic A, Jukic I, Kolic K, Dolic K. Clinical Manifestations of vertebrobasilar
dolichoectasia. Bratisl Lek Listy 2008;109(11):528-530.
8. Guirgis M, Lam B, Falcone S. Optic Tract Compression from Dolichoectatic Basilar
Artery. Am J Oph 2001 August;132(2):283-286.
9. Yu-Shih Lin J, Lin S-Y, Wu J, Wang I. Optic Neruopathy and Sixth Cranial Nerve Palsy
Caused by Compression From a Dolichoectatic Basilar Artery. J Neuro-Ophth
2006;26:190-191.
10. Beers, M. H. & Berkow, R. (ed). Cerebrovascular disease [electronic version]. The
Merck Manual of Diagnosis and Therapy, 17th Edition. Merck Research Laboratories,
NJ, 1999. www.merck.com/mrkshared/mmanual/section14/chapter174/174a.jsp.
11. Passero S, Rossi S. Natural history of vertebrobasilar dolichoectasia. Neurology 2008
Jan;70:66-72.