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Year : 1995  |  Volume : 43  |  Issue : 2  |  Page : 79-81

Ophthalmic manifestations of pulseless disease: A case report

From Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Atul Kumar
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029
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Source of Support: None, Conflict of Interest: None

PMID: 8818318

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How to cite this article:
Kumar G, Kumar A, Menon V. Ophthalmic manifestations of pulseless disease: A case report. Indian J Ophthalmol 1995;43:79-81

How to cite this URL:
Kumar G, Kumar A, Menon V. Ophthalmic manifestations of pulseless disease: A case report. Indian J Ophthalmol [serial online] 1995 [cited 2023 Nov 28];43:79-81. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1995/43/2/79/25265

The aortic arch syndrome represents a chronic disorder of the great arteries that branch from the aortic arch and manifests in diminished calibre and insufficient blood supply to the areas supplied by these vessels. Pulseless disease is one of the disorders of the aortic arch syndrome and is characterized by diminished or absent pulsations in the arteries of the head, neck and upper extremities. Takayasu[1] reported a case which had peculiar, wreath-like anastomosis of the central artery and vein of the retina around the optic disc. Ohnishi mentioned the association of this fundus picture with pulselessness of the radial artery. These two abnormalities may be considered the essential criteria for the diagnosis of this vascular disorder known as Takayasu-Ohnishi's disease or pulseless disease.[2]

Although the ocular manifestations of the disease are well known,[2][3][4][5][6][7] studies by Currier et al[8] indicate that the ocular manifestations of the condition represent its most common and constant symptomology and, is worthy of re-emphasis. We report a case of pulseless disease with its classical ophthalmological features.

  Case report Top

A 65-year-old male presented with complaints of painless progressive diminution of vision in the right eye of one year duration. He gave a history of transient obscuration of vision in both eyes, which lasted for few minutes followed by spontaneous recovery. The attacks were not heralded by flashes of light or any other entoptic phenomenon, nor were they related to activity or to the time of day. He also reported redness of both eyes, not associated with any pain, watering or discharge. He gave a history of episodes of giddiness associated with loss of consciousness. He had no relevant previous medical history.

His visual acuity was perception of light with accurate projection in the right eye and 6/24 in the left eye. On ocular examination, he had a right divergent squint and the ocular motility was normal. There were dilated conjunctival and episcleral vessels in both eyes. The iris of both eyes showed florid neovascularization and gonioscopy revealed open angles with new vessels. There was anisocoria; the right pupil was larger than the left. The pupillary reactions were not elicitable in the right eye while the left eye showed normal direct and consensual reactions. The right lens was opaque and the left lens had early posterior subcapsular opacities. The intraocular pressure was 10 mm Hg in both the eyes. Fundus examination in the left eye revealed a mild temporal pallor of the optic disc with a few anastomotic loops on the surface of the optic nerve head. There was cattle trucking of the blood column in the large arterial branches with venous dilatation and tortuosity. There were no microaneurysms, haemorrhages, exudates or any area of neovascularization. The anterior segment fluorescein angiography showed areas of patchy nonperfusion on the iris surface with extensive dye leakage from the irideal new vessels [Figure - 1]. Fluorescein angiography of the fundus revealed a prolonged arm to retina time, with delayed choroidal filling and patchy hypofluorescence of the optic disc. There was evidence of early cystoid macular oedema. No area of neovascularization was identified [Figure - 2]. Goldmann perimetry showed a mild constriction of the visual field of the left eye.

On systemic examination, all peripheral pulses in the upper extremity were not palpable. He had a normal blood pressure in the lower limb and the rest of his evaluation was unremarkable. All routine investigations done were normal with blood tests for syphilis being negative. The X-ray evaluation of the chest showed a calcification of the aortic arch.

  Discussion Top

Pulseless disease is a suitable descriptive term for the condition where reduced perfusion to the upper part of the body leads to diminished or absent peripheral pulses. Classically, pulseless disease consists of three cardinal signs - absence of palpable arterial pulsations in the upper extremity, arteriovenous anastomosis surrounding the optic nerve and a hypersensitive carotid sinus frequently resulting in attacks of syncope and vertigo.

The ocular features include changes in the retina, choroid and anterior segment, together with a lowering of the intraocular pressure. The retinal changes have been well documented by Uyama and Asayama.[9] Hirose[6] has described the fundus changes occurring early during the course of the disease. The chronic nature of the disease produces progressive dilatation of the capillaries leading to the formation of microaneurysms. Exudates and haemorrhages are rare since the intraluminal pressure is very low. Progressive retinal hypoxia causes arteriovenous anastomosis to form, which characteristically surrounds the disc in a whorl-like arrangement.[1] Ocular hypoxia also results in changes in the anterior segment. There is dilatation and tortuosity of the conjunctival and episcleral vessels and eventually the eye becomes painful, with corneal clouding, rubeosis iridis, cataract and an ischemic uveitis.

The case reported here had the unusual presentation of early fundus changes associated with severe anterior segment ischemia with florid iris neovascularization and cataract. It would appear that ocular manifestations of pulseless disease include features of both anterior and posterior segment hypoperfusion with either of them being the predominant presentation. The fact that ocular manifestations are the disease's most common and constant symptomology, it is possible that ocular findings may be the only apparent evidence of the underlying disorder.

  References Top

Takeaways M. Case report of a peculiar abnormality of the retinal central vessels. Acta Soc Ophthalmol Jap 12:554, 1908.  Back to cited text no. 1
Caccamise WC, Okuda K. Takayasu's or pulseless disease. Am J Ophthalmol 37:784-786, 1954.  Back to cited text no. 2
Pinkham RA. The ocular manifestations of the pulseless syndrome. Acta XVII Concilium Ophth 1:348-368, 1954.  Back to cited text no. 3
Ostler HB. Pulseless disease (Takayasu's disease). Am J Ophthalmol 43:583-589, 1957.  Back to cited text no. 4
Tour RL, Hoyt WF. The syndrome of the aortic arch: Ocular manifestations of "pulseless disease" and a report of a surgically treated case. Am J Ophthalmol 47:35-48, 1959.  Back to cited text no. 5
Hirose K. A study of fundus changes in the early stages of Takayasu-Ohnishi's (pulseless) Disease. Am J Ophthalmol 55:295-301, 1963.  Back to cited text no. 6
Hedges TR. The aortic arch syndromes. Arch Ophthalmol 71:28-34, 1964.  Back to cited text no. 7
Currier RD, De Jong RN, Bole GG. Pulseless disease: Central nervous system manifestations. Neurology 4:818-830, 1954.  Back to cited text no. 8
Uyama M, Asayama K. Retinal vascular changes in Takayasu's disease. Doc Ophthalmol 9:549-553, 1976.  Back to cited text no. 9


  [Figure - 1], [Figure - 2]


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