|Year : 1966 | Volume
| Issue : 5 | Page : 209-213
Thromboangeitis associated with retinal vasculitis
Minto Ophthalmic Hospital, Bangalore, India
|Date of Web Publication||17-Jan-2008|
S T Puttanna
Minto Ophthalmic Hospital, Bangalore
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Puttanna S T. Thromboangeitis associated with retinal vasculitis. Indian J Ophthalmol 1966;14:209-13
Thromboangeitis obliterans is a disease affecting mainly the limbs leading to progressive thrombosis and gangrene but vascular lesions may also involve the kidneys, heart, brain and has a protracted course with remissions and recrudescences. It is considered to be a distinct form of arteritis affecting males under 40 years (95%). Lower limbs are frequently involved and seriously affected than the arms. The vascular lesion is patchy and affects larger arteries and veins. The aetiology still remains obscure and smoking is considered to be a predisposing factor. We are reporting an unusual case of thromboangeitis obliterans associated with bilateral retinal vasculitis wherein both the retinal artery and vein are involved simultaneously; Retinal involvement is said to be rare as seen from the literature and hence the case is worth recording. In the reported cases retinal involvement is associated with an unusual feature of more generalised disease and could not be confirmed by pathological examinations. Bilateral partial or complete arterial occlusion, venous thrombosis and perivasculitis with or without vitreous hemorrhage have been reported by other workers in the field.
Leishman includes thromboangeitis obliterans and temporal arteritis under granulomatous arteritis. Lesion in Takayasu's disease also bears a close resemblance to that of temporal arteritis. Recently, various authors have doubted whether the lesion in thromboangeitis obliterans is a variant of atherosclerosis. Temporal arteritis is included under collagen diseases.
Histologically the lesion presents fibrosis of the media and adventitia, intimal thickening with or without thrombosis and cellular infiltration with formation of granular tissue and appearance of giant cells.
| Case Report|| |
N (Male), aged 47 years, was admitted on 3-8-64 for blurred vision of 5 years duration. He also complained of a wound in the left heel of 3 months duration.
Family History : Married. He had two sisters and three brothers all healthy. Father is alive and healthy. Mother died of heart attack three years ago.
Previous History : Five years ago he had experienced burning of the middle toe of right foot which was discharging pus. This toe had to be amputated and subsequently he underwent right lumbar sympathectomy. Three months after amputation he noticed sudden blurring of vision in both eyes at the same time. Again, in 1961 he was admitted to Krishnarajendra Hospital, for gangrene of the left fourth and fifth toes which were amputated. He smokes 20-25 beedies daily.
Present illness: As he developed an ulcer on the left heel after injury he got admitted again into the hospital. He noticed burning pain which aggravated with movements of the limbs and worse at night and in cold season. Intermittent claudication was present.
The limb was kept flexed at the hip and knee joints, the planta flexed at ankle joint. There was an ulcer on the left heel measuring ½ x 1", oval in shape. Margins were indurated and everted. Surface covered with slough with mucopurulent discharge. Base was fixed. Line of demarkation was diffuse. [Figure - 1]. The area was cold and tender. Surrounding area was edematous. Pulsation was absent in Dorsalis pedis, posterior tibial, popliteal, femoral arteries on the left side. On the right side pulsation was present but feeble. There was thickening of the arterial wall.
The 4th and 5th toes of the left foot and the 3rd toe of the right foot were missing [Figure - 1].
Pulse 78 per minute of low volume. Cardia normal. Blood pressure 170/ 100 in the left upper limb. Right arm 160/ 100; Red blood corpuscles 3.8 m/c mm. Bleeding time and coagulation time were within normal limits. Erythrocytic sedimentation rate 30 mm first hour. Vision on admission RE-CF at ½ m, LE-CF at 2½ m.
On 28-8-64, biopsy of right superficial temporal artery and on 25-9-64 that of the popliteal vessels were done. During biopsy there was no bleeding from the popliteal artery and there was slight oozing from the vein.
Biopsy report of superficial temporal artery : Specimen consists of cylindrical piece of tissue showing lumen 1 cm long x 2 mm in diameter. Intima in some places show vascularisation and is highly cellular. It shows thickening in some foci.
Biopsy report of popliteal vessels: (a) Specimen consists of pinkish white long piece of tissue about 1.5 cm. long, (b) Specimen consists of pinkish flat tissue (tubule) measuring l x 0.6 cm.
Occasional foci of intimal fibrosis is seen in the section.
Fundus in the Right Eye : Pale disc arteries obliterated, thread like without blood and their position represented by thin white lines. Veins very much reduced in calibre to the point of invisibility, with sheathing. Fibrous tissue was seen running vertically from the superior temporal vessels towards the macula. Pigmentary changes were seen at the macula with atrophy. Scattering of the pigment was seen here and there all over the fundus [Figure - 2].
Fundus in left eye: Very pale disc; arteries were not visible over the disc. Veins very much reduced in caliber and showed sheathing. Fibrous veil was seen above the disc spreading on to the surrounding retina. Pigmentary disturbances were seen with retinal atrophy [Figure - 3]. The fundus picture was suggestive of bilateral occlusion of central artery and vein with retinal atrophy and pigmentary disturbances with proliferation of fibrous tissue. Biopsy of superficial temperal artery on the right side and the popliteal artery and vein on the left side showed patchy intimal thickening here and there.
| Discussion|| |
Buerger (1908) described the vascular occlusive disease affecting the lower limbs which resulted in amputation for gangrene. He described an association between the arteritic lesion and phlebitis changes in the accompanying veins.
Marchesani (1935) has demonstrated narrowing and obliteration of vascular lumen histologically in the vessels of the retina, iris, and ciliary body in retinal periphlebitis in 3 patients with Buerger's disease. Mikuni (1936) Stauder (1934), Vyama (1937) and Highuchi (1940) have reported iritis, iridocyclitis and their sequelae, complicated cataract, phthysis bulbi due to Buerger's disease. Choroiditis, vitreous opacities and atrophy of the choroid in Buerger's disease were reported by Gresser, Marchesani, Mikuni, and Meyer (1948). White colouring of the retinal artery was reported by Marchesani (1936) and Geserick (1938). Periphlebitis retina- was reported by Marchesani and Stauder and Uyama (1937). White sheath formation on the retinal arteries was reported by Birmbaum (1934). Embolism of the central retinal arteries was reported by Schmalzer (1937), Lisch. K. (1937). Engorgement and spasm of the. central retinal arteries was reported by Forester and Guttmann (1933). Haemorrhages in the retina or vitreous body or both was reported by Marchesani, Marchesani and Stauder, Mikuni, Uyama, Geserick. Retinitis proliferans was reported by Marchesani, Marchesani and Stauder, Mikuni and retinal atrophy was reported by Gresser, Uyama, optic atrophy was reported by Marchesani, Gessrick and it was due to circulatory disturbances of the optic nerve.
In Schmellzer (1937) series of Buerger's disease none had signs or symptoms of vitreous hemorrhage. Schmid (1945) in his analysis of 85 patients with Buerger's disease of which 65 were subjected to arteriography found none with retinal periphlebitis and none had ocular disturbances. Mayer (1949) in his analysis of Eale's disease found none with signs or symptoms of Buerger's disease. Cross (1961) has reported Eale's disease being associated with thromboangeitis obiterans in a male aged 34 years.
Hirose and Baba (1963) experimentally by injecting fetal hair fragment suspension into the common carotid artery of rabbits produced ocular and cerebral changes which are clinically and histologically identical with the findings in Buerger's disease, and they concluded that, Takayasu-Ohnishi's disease is not a type of Buerger's disease but Buerger's disease can cause the Takayasu-Ohnishi syndrome as it can create stenosis or occlusion of the aortic arch and its major branches. It has also been observed that some cases of Takayasu-Ohnishi's disease lack pulse in the lower extremities. Cerebral signs are found in both diseases. Except for peripapillary coronal anastomosis of the central retinal artery and vein there are no specific ocular changes in Takayasu-Ohnishi's disease. Recently any change in the fundus seen in cases with an abnormal pulse in the arteries of upper extremities is indicative of Takayasu-Ohnishi's disease which makes some authors classify Takayasu-Ohnishi's disease as a variant of Buerger's disease.
Hirose and Kiya from their experimental studies concluded that stenosis or occlusion of the aortic arch and its major branches produces changes in the fundus specific of Takaysu-Ohnishi's disease depending on the location and extent of the stenosis or occlusion. Difficulty in diagnosis arises in cases where Berger's disease lacks spontaneous gangrene as well as pulsation in the carotid and other arteries of the uppear extremities.
The new concept of diffuse collagen diseases had given a new orientation to certain diseases whose etiology still remain obscure, and the number of conditions grouped under this rank are steadily growing. Occasionally the clinician is confronted with a patient presenting disseminated clinical patterns and groups those conditions as "undifferentiated collegen disease." The present case of retinal vasculitis associated with thromboangeitis obliterans may well be included under these undifferentiated collagen diseases as it presented varied clinical patterns which do not fit into definitive diagnosis. Further our knowledge regarding the ground substance, chemistry and reactivity to enzyme, hormones and noxious agents is still scanty and patchy. Even the biopsy material is not very conclusive as it showed patchy intimal thickening here and there. The other unusual feature of the present case is the bilateral involvement of both the central retinal artery and central vein simultaneously three months after amputation of toes.
We presume that the pathological changes that were operative at the limb level shoud be operative at the central retinal artery and vein producing an occulsive lesion in both.
| Summary|| |
1) An unusual case of thromboangeitis obliterans associated with retinal vasculitis simulating a picture of bilateral involvement of occulsion of central retinal vessels is reported.
2) Its relationship with Takayasu-Ohnish's disease is discussed.
3) Pertinent literature is reviewed.
4) It is suggested that the condition may be included under undifferentiated collagen diseases.
| Acknowledgment|| |
The author is thankful to Dr. K. V. Ghorpade, Professor of Pathology for the biopsy reports and Dr. M. Authikesavalu, Professor of Surgery, for the biopsy of superficial temporal artery and popliteal. vessels. He is also thankful to Wing Commander Dr. Jones of Aviation Medicine, for the fundus photographs.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3]