|Year : 1975 | Volume
| Issue : 1 | Page : 36-38
Ocular manifestations of pulseless disease
Nehru Institute of Ophthalmology and Research, Eye Hospital, Sitapur, India
A K Paul
Nehru Institute of Ophthalmology and Research, Eye Hospital, Sitapur
|How to cite this article:|
Paul A K. Ocular manifestations of pulseless disease. Indian J Ophthalmol 1975;23:36-8
Pulseless disease is a form of chronic circulatory insufficiency characterised by progressive obliteration of the great vessels arising from the arch of the aorta leading to arterial hypotension and a diminution or absence of pulsation of the vessels in the neck and arms and is associated with hypertension in the lower extremities. ,,,
In India few cases have been reported so far ,,,,,
Two types of the disease occur. The first, is common in females between the age 20 and 30 years, frequently seen in. Japanese and is mainly inflammatory in origin known as Takayasu's disease. The main characteristic changes are diffuse and non-specific arteritis, in vascular wall particularly of adventitia with inflammatory reaction containing giant cells leading to thrombosis  It is associated with raised sedimentation rate, leucocytosis and occasional fever. The second type, is found in patients with degenerative in nature and also known as Takayasu's syndrome or aortic arch syndrome. It occurs in older patients of either sex without racial predilection usually during 5th or 6th decades of life. The etiology of occlusion is principally the result of syphilitic aortitis and aneurysm  and atherosclerosis with thrombosis and atheroma to be common basis of the syndrome  .
The most common ocular findings described are  : 1. Atrophy of ciliary muscle and processes, 2. Cataractous change in the lens 3. diminished central retinal arterial blood flow 4. Retinal arteriovenous anastomoses and new vessels formation in nerve fibre layers. 5. Clumping of rods and cones. 6. Decrease in the number of ganglion cells and 7. Demyelinization of optic nerve.
| Case Report|| |
A Hindu female aged 30 years was admitted with history of progressively severe episodes of transient loss of vision, headache, fainting and weakness with tingling sensation in limbs since one year. The fainting was precipitated on standing from lying down position or on looking up. She had also noticed that if she put her hand under her head, or pillow while resting, the fingers would get numb.
A young moderately built woman, married 12 years back, has three children and all are healthy. The heart was slightly enlarged but sounds were normal. radial, brachial and temporal arteries were not palpable and were without pulsation. Blood pressure could not be recorded in the upper limb but in the lower limb (Popliteal artery) it was 210/100 mm of Hg. Her speech was slow and face looked exhausted. Routine laboratory tests were within normal limits, except that the haemoglobin percentage was slightly low. The fasting blood sugar was 90 mg%.
Vision in right eye was finger counting one meter and in left eye it was only perception of light. The conjunctiva and cornea showed no abnormalities. Both the pupils were dilated and reacting sluggishly to light and convergence. There were patches of atrophy in the iris. Fine vessels could be seen on the surface e€ the iris mostly in the ciliary region. The lens showed posterior subcapsular opacity in both the eyes.
The intra ocular pressure in right eye was 17.3 mm. and in the left eye was 14.2 mm. (Schiotz). The ophthalmodynamometric pressure of the central retinal artery was found to be very low.
The most significant abnormalities were seen in the retina and pictures were almost same in both eyes. The discs were pale and covered with dilated and tortuous blood vessels. Arteries were almost untraceable from the discs. The veins were tortuous, irregular with dilatations and showed segmentation of the moving blood columns. There were peripapillary arteriovenous communications. There were superficial haemorrhages around the disc and few exudative spots were seen in the para macular area.
Fluorescein angiography showed delayed arm to retina circulation time (28 seconds) [Figure - 1] The arteries were seen occluded about 2 disc diameters from the disc. The veins were also similarly obliterated. There were dilated arteriovenous communications [Figure - 2]. The fine blood vessels on the disc were dilated tortuous and were showing aneurysmal dilatations. The dye [Figure - 3]was freely leaking from these vessels.
The essential ocular lesions described relate to persistent chronic ischaemia as a result of stenosis or occlusion of the great vessels originating from the arch of the aorta. This case presents a unique picture of vaso-occlusive disease. The extensive occlusion of the large branches of the central retinal blood vessels a small distance away from the disc seems to be a part of the primary disease process. The arteriovenous communications, and other micro-vascular anomalies could be due to general state of chronic anoxemia.
| Summary|| |
A case of pulseless disease showing extensive retinal vascular occlusion with consequent vascular anomalies on and around the disc has been presented with fluorescein retinal photographs.
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[Figure - 1], [Figure - 2], [Figure - 3]