Abstract
A 34-year-old woman is presented who has recurrent, multiple, retinal arteriolar occlusions associated with a deficiency in protein C, a vitamin K-dependent anticoagulant. Protein C is a naturally occurring anticoagulant and if there is a deficiency, it can lead to episodes of thrombosis; the deficiency can be acquired or congenital. This is the first documented case of retinal arteriolar occlusion associated with a congenital deficiency of this protein. The mechanism for arteriolar occlusion in this patient is discussed, and whilst the role of protein C deficiency remains presumptive, it is strongly implicated.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Bertina RM, Broekmans AW, Linden IK van der, Mertens K (1982) Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemost 48:1–5
Bertina RM, Broekmans AW, Krommenhoek vas Es C, Wijngaarden A van (1984) The use of a functional and immunological assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency. Thromb Haemost 51:1–5
Brenner B, Shapira A, Bahari C, Haimovich L, Seligsohn U (1987) Hereditary protein C deficiency during pregnancy. Am J Obstet Gynecol 157:1160–1161
Broekmans AW, Veltkamp JJ, Bertina RM (1983) Congenital protein C deficiency and venous thromboembolism. New Engl J Med 1983 309:340–344
Brown GC, Magaral LE, Shields J, Goldberg RE, Walsh P (1981) Retinal arterial obstruction in children and young adults. Ophthalmology 88:18–25
Carroll D (1970) Retinal migraine. Headache 10:9–13
Clouse LH, Clomp PC (1986) The regulation of haemostasis: the protein C system. New Engl J Med 314:1298–1304
Coppeto JR, Lessell S, Sciarra R, Bear L (1986) Vascular retinopathy in migraine. Neurology 36:267–270
Esmon CT (1983) Protein C: biochemistry, physiology and clinical implications. Blood 62: 1155–1158
Estelles A, Garcia-Plaza I, Dasi A, Aznar J, Duart M, Sanz G, Perez-Requejo JL, Espana F, Jimenez C, Abeledo G (1984) Severe inherited homozygous protein C deficiency in a newborn infant. Thromb Haemost 52:53–56
Francis RB, McGehee WG (1985) Defibrination during warfarin therapy in a man with protein C deficiency. Thromb Haemost 53:249–251
Gitting JW (1985) Branch retinal artery occlusion in a young woman. Surv Ophthalmol 30: 52–58
Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C (1981) Deficiency of protein C in congenital thrombotic disease. J Clin Invest 68:1370–1373
Horellou MH, Conard J, Bertina RM, Samama M (1984) Congenital protein C deficiency and thrombotic disease in nine French families. Br Med J 289:1285–1287
Katz B (1985) Bilateral sequential migrainous ischaemic optic neuropathy. Am J Ophthalmol 99:489
Katz B (1986) Migrainous central retinal artery occlusion. J Clin Neuro-Ophthalmol 6: 69–71
Malm J, Laurell M, Dahlback B (1988) Changes in the plasma levels of vitamin K-dependent proteins C and S and of C4B-binding protein during pregnancy and oral contraception. Br J Haematol 68:437–443
Mammen EF, Thomas WR, Seegers WH (1960) Activation of purified prothrombin to autoprothrombin II (platelet co-factor II or autoprothrombin II-A). Thromb Diath Haemor 5:218–249
Mannucci PM, Owen WG (1987) Basic clinical aspects of proteins C and S. In: Bloom AL, Thomas DP (eds) Haemostasis and thrombosis, 2nd edn. Churchill Livingstone, Edinburgh Harlow New York, pp 452–464
Mannucci PM, Tripodi A (1987) Laboratory screening of inherited thrombotic syndromes. Thromb Haemost 57:247–251
Mannucci PM, Vigano S (1982) Deficiencies of protein C, an inhibitor of blood coagulation. Lancet II: 463–467
Mannucci PM, Vigano S, Bottasso B, Candotti G, Bozzetti P, Rossi E, Pardi G (1984) Protein C antigen during pregnancy, delivery and puerperium. Thromb Haemost 52:217
Marciniak E, Wilson HD, Marlar RA (1985) Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in the blood. Blood 65:15–20
Marlar RA (1985) Protein C in thromboembolic disease. Sem Thromb Haemost 11:387–393
Pabinger-Fasching I, Bertina RM, Lechner K, Niessner H, Korninger C (1983) Protein C deficiency in two Austrian families. Thromb Haemost 50: 810–813
Pulido JS, Lingua RM, Cristol S, Byrne SF (1987) Protein C deficiency associated with vitreous haemorrhage in a neonate. Am J Ophthalmol 104:546–547
Rubinstein K, Paton A (1968) Fluorescein angiography in oclusion of the retinal arteries. Trans Ophthalmol Soc UK 87:345–354
Russell RWR, Fytche TJ, Sanders MD (1966) A study of retinal vascular occlusions using fluorescein angiography Lancet II:821–825
Samama M, Horellou MH, Soria J, Conard J, Nicolas G (1984) Successful progressive anticoagulation in a severe protein C deficiency and previous skin necrosis at the initiation of oral anticoagulant therapy. Thromb Haemost 51:132–133
Seegers WH, Novoa E, Henry RL, Hassouna HI (1976) Relationship of new vitamin-K-dependent protein C and old autoprothrombin II-A. Thromb Res 8:543–552
Seligsohn E, Berger A, Abend M, Rubin L, Attias D, Zivelin A, Rapaport SI (1984) Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. New Engl J Med 310:559–562
Smith DB, Ens GE (1987) Protein C deficiency: a cause of amaurosis fugax. J Neurol Neurosurg Psychiatry 50:361–362
Stenflo J (1976) A new vitamin-K-dependent protein: purification from bovine plasma and preliminary characterisation. J Biol Chem 251:355–363
Troost BD (1986) Migraine. In: Duane TD (ed) Clinical ophthalmology, vol 2. Harper and Row, London, pp 1–22
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Nelson, M.E., Talbot, J.F. & Preston, F.E. Recurrent multiple-branch retinal arteriolar occlusions in a patient with protein C deficiency. Graefe's Arch Clin Exp Ophthalmol 227, 443–447 (1989). https://doi.org/10.1007/BF02172896
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02172896