Ocular Manifestations of Buerger’s Disease – A Review of Current Knowledge

Figures & data

Table 1 Review of 9 Articles Concerning Ocular Manifestations of Buerger’s Disease

Authors, Year of Publication	Sex and Age of the Patient on Admission, the Affected eye	Extra-Ophthalmologic Signs and Symptoms and Past History	Symptoms, Signs and Basic Examination on Admission	Results of the Ophthalmic Examination of the Affected eye	Final Diagnosis	Treatment	Outcome
Boeke W. and Duncker G, 1983^Citation24	Male, 30, right and left eye	Thrombophlebitis of the deep leg and pelvic veins (recurrences of the thrombophlebitis in the lower extremities were accompanied by deterioration in the eye condition)	-	Slit lamp of OU: features of anterior uveitis, opacification of the vitreous body, complicated cataract Fundoscopy of OU: white retinal foci, retinal hemorrhages, retinal edema, constriction of the retinal vessels	Bilateral uveitis, retinitis and papillitis (recurrent)	Azathioprine (150 mg/d) Fluocortolone (15 mg every other day) Phenprocumarol	Visual loss Optic atrophy VA OD = 1/15 VA OS = 0.1 Concentric constrictions in the visual field
Dimopoulos I.S. et al., 2020^Citation25	Male, 48, right eye	Recurrent ulcers at the tip of the fingers (duration: 3 years) Macrocytosis (probably secondary to the alcohol intake) Protein C and antithrombin III deficiency Abnormal photoplethysmography in the upper limb arterial duplex scan - small vessel occlusive disease in the upper extremity and a mild degree of reverse flow in the left ulnar artery Digital pitting, finger nail bed changes	Sudden onset of vision loss VA OD = 20/500 VA OS = 20/20	Fundoscopy of OD: ischemic retina, cotton wool spots Fundoscopy of OS: normal	Occlusive retinal vasculitis and periphlebitis	Aspirin 81 mg/day po	-
	One year later - left eye	-	Sudden onset of severe vision loss VA OD = 20/600 VA OS = cf at 1m	Slit lamp of OU: mild nuclear sclerotic cataract Fundoscopy of OU: retinal thinning in the macular region, vascular loops with collateralization, dot-blot and flame-shaped hemorrhages, arteriolar narrowing with venular dilation and sheathing Additionally in the fundus of OD: a few nerve fiber layer infarcts FA: early phase vessel wall staining involving veins and collateral arteries and mid/ late phase leakage, the arm-retinal and arteriovenous transit times for the left eye was equal to 16.8 s and 11.3 s respectively, the foveal avascular zone (FAZ) was enlarged more in the right eye compared to left OCT of OU: areas of inner retinal atrophy with paracentral cystoid degeneration and edema		Apixaban 5 mg/day po	-
Eris E. et al., 2017^Citation26	Male, 64, left eye	After left leg amputation	Sudden vision loss VA OD = 16/20 VA OS = hand motion RAPD+	Fundoscopy: area of interpapillomacular retinal ischemia with whitish edges and a red spot in the macula OCT: increased reflectivity of the inner retinal layers, central macular thickness = 650 uM FA: no perfusion in the macular area with late staining of small veins and arterioles and leakage	Central retinal artery occlusion (CRAO)	Hyperbaric oxygen therapy (20 sessions)	VA OS = 10/200 OCT: macular atrophy
Koban Y. et al., 2014^Citation27	Male, 48, right and left eye	After bilateral below-the-knee amputations for ischemic ulcers of the lower limbs Diabetes mellitus (controlled by oral antidiabetics) Left carotid artery stenosis in bilateral carotid artery color doppler ultrasound	Vision loss of OS BCVA OD = 20/20 BCVA OS = 20/32 Ishihara plates OD = 10/10 Ishihara plates OS = 6/10 RAPD+ in OS IOP OD (applanation) = 18 mm Hg IOP OS (applanation) = 16 mm Hg	Gonioscopy OU: open iridocorneal angles to the ciliary body bands for 360 degrees Slit lamp OU: mild posterior subcapsular cataract Fundoscopy OD: arteriolar constriction, minimal pallor of the temporal rim, c/d = 0.5 Fundoscopy OS: relatively superior entrance to the central retinal artery, temporal peripapillary atrophy, arteriolar constriction, retinal edema in the inferopapillary area, splinter hemorrhages, soft exudate, c/d = 0.8 Visual field test: peripheral constriction, decreased retinal sensitivity in the Bjerrum area of the right eye, and a small temporal island FA: delay in inferonasal branch retinal artery filling in the left eye	Acute inferonasal branch retinal artery occlusion in the left eye and bilateral normal tension glaucoma (NTG)	-	-
Korkmaz A et. al, 2018^Citation28	Male, 43, left eye	Infrapopliteal artery occlusive disease Occlusion in the right posterior tibial artery Phlebitis migrans	Sudden decrease of VA of OS (duration: 5 days) BCVA OD = 10/10 BCVA OS = 2/10 Ishihara plates OD = 21/21 Ishihara plates OS = 8/21 RAPD+	Fundoscopy: blurring, hemorrhagic swelling of optic disc Humphrey visual field: inferior altitudinal defect FA: late phase - leakage of optic disc	Non-arteritic anterior ischemic optic neuropathy (NAION)	Acetyl salicylic acid (330 mg/d) + Prednisone 1mg/kg/d for 14 days)	After 4 days of treatment: BCVA: 4/10 Ishihara plates: 14/21 Fundoscopy: partially improved disc edema
Marques A. et al., 2015^Citation29	Male, 64, right and left eye	After amputation of some of the fingers and toes	Progressive bilateral visual acuity decrease and nyctalopia BCVA OD = cf BCVA OS = 0.7	Slit lamp OU: phacosclerosis Fundoscopy OU: extensive confluent areas of chorioretinal atrophy with involvement of the posterior pole (except in the perifoveal area of OS), diffuse arteriolar narrowing FA: delay in the arm-to-retina time (27 seconds), areas of staining; OD – evident exposure of the sclera in the foveal area SD-OCT: reduction in the thickness of the internal and external layers of the retina (except in the fovea of OS) Visual field test: bilateral generalized retinal sensitivity reduction, small central island of vision in OS Flash ERG, scotopic ERG and photopic ERG: extremely diminished amplitudes of both a and b waves	Chorioretinal atrophy	Acetylsalicylic acid 150mg/d	After a year: Maintenance of the VA of OU
Ohguro I. et al, 2006^Citation30	Male, 66, left eye	After lumbar sympathectomy After an acute myocardial infarction (1995) Ankle-brachial pressure index: 0.69 (norm: 0.9–1,3)	Progressive visual field disturbance in the left eye VA OD = 1.2 VA OS = 1.5 IOP OU = 13 mm Hg	Slit lamp OU: slight nuclear sclerosis Fundoscopy of OU: retinal vessel tortuosity, arteriosclerosis and glaucomatous cupping c/d of OD = 0.8 c/d of OS = 0.6 Additionally in the fundoscopy of OS: plaques and occlusion in the inferotemporal retinal artery, retinal edema in the inferotemporal area of the macula Humphrey Field Analyzer: decreased retinal sensitivities in the Bjerrum area of OU, retinal artery occlusion area of OS FA: retardation of arm-to-retina circulation time and filling defect of the inferotemporal retinal artery in the left eye	Branch retinal artery occlusion and normal tension glaucoma	-	-
Reche Sainz JA et al., 2018^Citation31	Male, 27, right eye	Chronic distal ischemia of the left lower limb (durations: years)	Sudden visual loss VA OD = 0.9 VA OS = no information	Fundoscopy: congestive papilla, cotton wool spots, venous tortuosity, scattered retinal hemorrhages	Papillophlebitis	Acetylic acid (100 mg/d)	VA OD = 1.0 The papilledema and most of retinal hemorrhages have resolved
Zaoui K. et al., 2020^Citation32	Male, 30, right eye	Paresthesia of the lower right extremity with intermittent coldness of the feet Necrosis of the right little toe during hospitalization which lead to the amputation	Rapidly progressing visual acuity drop of the right eye and blurred vision of the left eye VA OD = 5/10 VA OS = 10/10	Slit lamp: mixed bilateral granulomatous keratic precipitates with “mutton fat” at the bottom Fundoscopy of OU: multiple, yellow-white spots, greyish pigmented lesions (choroidal lesions) FA: delay in filling the choriocapillary, progressive impregnation of the lesions with slight diffusion per area	Bilateral panuveitis	–	–

Abbreviations: OD, oculus dexter; OS, oculus sinister; OU, oculus uterque (both eyes); VA, visual acuity; BCVA, best corrected visual acuity; FA, fluorescein angiography; OCT, optical coherence tomography; ERG, electroretinogram; SD-OCT, spectral domain optical coherence tomography; IOP, intraocular pressure; CDR, cup to disc ratio; RAPD, relative afferent pupillary defect; cf, counting fingers.

Table 2 The Percentage of Different Types of Ophthalmological Involvement in Patients with Buerger’s Disease

Diagnosis	Number	Percentage (Out of 44)
Non-arteritic ischemic optic neuropathy	2	4.55%
Occlusive retinal vasculitis and periphlebitis	1	2.27%
Papillophlebitis	1	2.27%
Central retinal artery occlusion	1	2.27%
Branch retinal artery occlusion	2	4.55%
Normal tension glaucoma	2	4.55%
Uveitis	2	4.55%
Chorioretinal atrophy	1	2.27%
Retinitis	1	2.27%
Papillitis	1	2.27%
Optic atrophy	2	4.55%
Changes typical for atherosclerotic hypertensive retinopathy	22	50.00%
Abnormalities in electroretinography	10	22.73%

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