Crohn's Disease and Retinal Vascular Disease Alan J. Ruby, M.D., and Lee M. Jampol, M.D. Patients with Crohn's disease may manifest extraintestinal findings including ocular involvement. We treated two patients with Crohn's disease who manifested retinal vascular disease that may have been related to the immune nature of the underlying disease or possibly to changes in the vasculature or coagulation system. One patient had a central retinal vein occlusion and the other had retinal vasculitis involving retinal arteries and veins with an apparent branch retinal artery occlusion. Both patients were in excellent health except for Crohn's disease, which was confirmed by a biopsy specimen. In one patient, the diagnosis of retinal vasculitis preceded the clinical diagnosis of Crohn's disease, whereas for the second patient the symptomatology of the intestinal disease preceded the ocular manifestations. We suggest that inflammatory bowel disease should be considered in the differential diagnosis of retinal vascular occlusive disease, especially in a young patient. refers to two clinically distinct entities: ulcerative colitis and Crohns disease. Ulcerative colitis is primarily a mucosal disease limited to the large intestine and characterized by inflammation and diffuse vascular congestion. Crohns disease is a transmural process that may affect any point along the digestive canal, most commonly the distal ileum and proximal colon. Histologically, Crohns disease is characterized by transmural inflammation with the formation of noncaseating granulomas.' Numerous extraintestinal complications of inflammatory bowel disease have been reported, which affect almost IDIOPATHIC INFLAMMATORY BOWEL DISEASE
Accepted for publication July 25, 1990. From the Department of Ophthalmology, Northwestern University Medical School, Chicago, Illinois. This study was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc. Reprint requests to Lee M. [arnpol, M.D., Northwestern University Medical School, 303 E. Chicago Ave., Chicago, IL 60611.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
110:349-353,
every organ system in the body.I" There have, however, only been a few reported cases of retinal vascular disease in conjunction with inflammatory bowel disease." We treated two patients with retinal vascular disease and Crohns disease.
Case Reports Case 1 A 33-year-old woman, in whom Crohns disease was diagnosed in 1972, had an eight-week history of blurred vision and micropsia in the left eye in October 1980. The patient was taking 50 mg of prednisone every other day and sulfasalazine four times a day as treatment for the inflammatory bowel disease. She had no previous ocular complaints and was otherwise in good health. Physical examination disclosed best-corrected visual acuity of R.E.: 20/25 and L.E.: 20/ 400. Results of external examination, pupillary examination, and confrontational visual fields were all normal. Results of slit-lamp examination were normal in both eyes, except for a few cells in the anterior chamber of the left eye. Intraocular pressure was R.E.: 17 mm Hg and L.E.: 15 mm Hg. Results of ophthalmoscopic examination of the right eye were normal, except for a choroidal nevus. Examination of the left eye (Fig. 1) disclosed marked disk edema with many peripapillary nerve fiber layer hemorrhages. The venous system was dilated with dot and blot hemorrhages throughout the peripheral retina. The macula was edematous with cystoid spaces. A fluorescein angiogram (Fig. 1) showed sluggish venous flow with rnicroaneurysms, capillary dilatation, and a late cystoid pattern. Disk leakage was present. Results of laboratory studies including protein electrophoresis, fluorescent treponema I antibody absorption test, rapid plasma reagin, antinuclear antibody, sedimentation rate, complete blood cell count, C3, and thyroid studies were all within normal limits. A diagnosis of central retinal vein occlusion OCTOBER,
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Fig. 1 (Ruby and Jampol). Case 1. Left, Left eye showing central retinal vein occlusion with marked disk edema and peripapillary nerve fiber layer hemorrhages. Right, Fluorescein angiogram of the left eye showing cystoid macular edema and late disk leakage.
was made. At the time of the most recent follow-up in April 1990, examination disclosed visual acuity of L.E.: counting fingers with macular pigment mottling and retinal fibrosis. No evidence of neovascularization was seen. The patient continues to have flare-ups of ileitis and continues to take varying doses of prednisone and sulfasalazine. Case 2 A 25-year-old woman, in whom bilateral iridocyclitis was diagnosed in 1983, was referred in May 1984 with vitreous floaters and blurred vision in the left eye for one week. The patient also complained of mouth ulcers that began after she took a sulfa antibiotic for an upper respiratory tract infection. She had not been treated for iritis since the initial episode. The patient's mother had Crohns disease. Bestcorrected visual acuity was R.E.: 20/20 and L.E.: 20/20 - 2. Results of color vision test, external examination, and motility were all normal. Slit-lamp examination showed a few residual posterior synechiae in the left eye with pigment deposition on the anterior lens capsule. Results of the remainder of the anterior segment examination were normal. Results of ophthalmoscopic examination of the right eye were unremarkable. Examination of the left eye (Fig. 2) disclosed mild vitreous cells, arteriovenous crossing changes with arteriolar attenuation, and venous dilatation with sheathing. There were scattered cotton-wool spots. Retinal vascular sheathing was seen (especially inferotemporally) involving the arteries and, to a lesser degree, the veins. The disk margins were blurred. Hemorrhages were scattered in the
distribution of the inferotemporal vein. A fluorescein angiogram showed markedly delayed filling of the vessels in the inferotemporal quadrant with a branch artery occlusion, disk leakage, and staining of the veins (Fig. 2). Results of laboratory investigations including chest x-ray, complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, fluorescent treponemal antibody absorption test, Toxoplasma titers, angiotensin-converting enzyme, and purified protein derivative (tuberculin) test were normal. She was given 60 mg of prednisone every day with a gradual taper. By November 1984, she had discontinued taking all corticosteroids and showed no evidence of active vasculitis. the patient had iritis in the right eye in February 1985 and was treated with topical corticosteroids with subsequent resolution. She was noted to have minimal peripheral venous sheathing in the right eye in March 1988. Crohn's disease was diagnosed after colonoscopy and intestinal biopsy in 1988. The symptoms of gastrointestinal disease had been present intermittently for many years. The patient has been followed up since that time without any flareups of the retinal vasculitis and visual acuity has remained 20/20 in both eyes. She is not being treated with corticosteroids for the bowel disease.
Discussion Crohns disease is a multisystem disease characterized by arthritis, skin manifestations,
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Fig. 2 (Ruby and Iampol), Case 2. Left, Left eye showing retinal vascular sheathing with multiple hemorrhages and cotton-wool spots. Right, Fluorescein angiogram of the left eye showing disk leakage and staining of the arteries and veins.
malabsorption, gallstones, kidney stones, osteoporosis, liver disease, peptic ulceration, corneal ulcers, keratitis, Sjogren's syndrome, blepharitis, scleritis, myositis, orbital and eyelid edema, retrobulbar neuritis, and neuroretlnitis.Pv" Many of the ocular conditions are believed to be secondary to the same phenomenon causing the underlying disease, namely immunologic disease that affects both the eyes and the bowel, possibly autoimmune in nature." Although many ocular manifestations of Crohn's disease have been reported, one patient manifesting primarily retinal vasculitis has been described. Duker, Brown, and Brooks? described a patient with Crohn's disease, which was confirmed by a biopsy specimen, who manifested severe, bilateral, asymmetric, obliterative retinal arteritis and phlebitis. Several cases that share some similarities with this case have also been reported. Macoul' described a patient with Crohns disease who showed evidence of vasculitis in the form of bilateral, severe neuroretinitis and hyalitis associated with recurrent conjunctivitis, bilateral iridocyclitis, and marginal corneal infiltrates and ulcers. Ellis and Gentry3 reported a case of retinal and disk edema associated with a hazy vitreous in a patient with Crohn's ileitis. Many conditions may predispose an individual to central or branch retinal vein occlusions, including alterations of blood flow, altered viscosity of the blood, and abnormalities of the coagulation system and of the vessel walls. Systemic diseases that can cause these changes include blood dyscrasias, dysproteinemias, vasculitis, and autoimmune diseases such as
rheumatoid arthritis and systemic lupus erythematosus." The association of inflammatory bowel disease with systemic vascular disease has been well documented. Thromboembolism is a recognized complication of inflammatory bowel disease that can involve the systemic vasculature as well as the cerebral and retinal vessels. Schneiderman, Sharpe, and Sutton" described five patients who had cerebral or retinal vascular occlusions in association with inflammatory bowel disease. One patient with Crohn's disease, which was confirmed by a biopsy specimen, had bilateral retinal branch artery occlusions. Fluorescein angiography showed no evidence of intrinsic retinal vascular disease. No evidence of embolic phenomena could be documented, since the patient had a normal carotid arteriogram and echocardiogram. Four other patients had cerebrovascular occlusions. An increased platelet count was identified in four of the five patients described. Neither of our patients demonstrated an increased platelet count. Talbot and associates" surveyed records of 7,199 patients with chronic ulcerative colitis or Crohn's disease over an II-year period. Thromboembolic complications developed in 92 (1.3%) of these patients (49 patients with Crohn's disease). Other authors have reported cases of central retinal artery occlusion associated with ulcerative colitiS. 12•13 These cases are similar to idiopathic retinal arteritis in which the patients, mostly young, manifest multiple retinal arterial occlusions." Alterations in the coagulation system that can occur with inflammatory bowel disease can also contribute to thrombosis. Coagulation
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abnormalities that have been reported include accelerated thromboplastin generation, and increased levels of factor V, factor VIII, fibrinogen, and low antithrombin III concentrations." Jong and associates" studied fibrinolytic activity in plasma and colonic mucosal biopsy specimens of 28 patients with inflammatory bowel diseases. In plasma of patients with inflammatory bowel disease, significantly decreased tissue-type plasminogen activator activity, increased plasminogen activator inhibition and fibrinogen, and prolonged thrombin and prothrombin times were found. Patients with high plasminogen activator inhibition concentrations are at risk for developing deep venous thrombosis." Increased plasma fibrinopeptide A levels and enhanced generation of monocyte tissue factor activity in patients with Crohns disease may contribute to the activation of blood coagulation." It has been proposed that only patients with active inflammatory bowel disease suffer from this hypercoagulable state and that stable patients are not at increased risk of thromboembolism." Neither of our patients had a history of previous thromboembolism and neither had coagulation studies performed. Retinal vasculitis as a separate clinical entity has been reported as an isolated finding or in association with such systemic conditions as systemic lupus erythematosus, Behcets disease, sarcoidosis, polyarteritis nodosum, Wegener's granulomatosis, tuberculosis, syphilis, multiple sclerosis, and ankylosing spondylitis." Most of these conditions have an underlying immunologic abnormality, which contributes to the disease process. Characteristics of retinal vasculitis may include diffuse capillary leakage, cotton-wool spots, vascular sheathing, vascular occlusion, vitreous cells, and retinal hemorrhages. Our second patient manifested most of these characteristics. Her retinal findings were similar to those seen in some patients with Behcets disease. Behcets disease, more common in males, is characterized by the classic triad of mouth ulcers, genital ulcers, and iritis. Diffuse capillary leakage, branch retinal vein or artery occlusions, infiltrates, and a terminal stage showing optic atrophy and macular pigmentary changes comprise the retinal findings. Gastrointestinal lesions simulating Crohns disease can often be found in patients with Behcet's disease, which cause diagnostic difficulties." A complete rheumatologic examination in Case 2 found no sys-
temic abnormalities to support a diagnosis of Behcets disease. This patient developed oral ulcers only after being treated with sulfa antibiotics for a respiratory infection. Additionally, aphthous stomatitis can be seen in up to 11 % of patients with Crohn's disease and represents the mouth lesion most often seen in these patients."
References 1. Goldner, F., and Kraft, S. D.: Internal Medicine, ed. 1. Boston, Little, Brown and Company, 1983, pp. 134-144. 2. Greenstein, A. J., Janowitz, H. D., and Sachar, D. B.: The extraintestinal complication of Crohn's disease and ulcerative colitis. A study of 700 patients. Medicine 55:401, 1976. 3. Ellis, P. P., and Gentry, J. H.: Ocular complications of ulcerative colitis. Am. J. Ophthalmol. 58:779, 1964. 4. Macoul, K. L.: Ocular changes in granulomatous ileocolitis. Arch. Ophthalmol. 84:95, 1970. 5. Crohn, B. B.: Ocular lesions complicating ulcerative colitis. Am. J. Med. Sci. 169:260, 1925. 6. Petrelli, E. A., McKinley, M., and Troncole, F. J.: Ocular manifestations of inflammatory bowel disease. Ann. Ophthalmol. 14:356, 1982. 7. Duker, J. S., Brown, G. c.. and Brooks, L.: Retinal vasculitis in Crohn's disease. Am. J. Ophthalmol. 103:664,1987. 8. Korelitz, B. J., and Coles, R. S.: Uveitis (iritis) associated with ulcerative/granulomatous colitis. Gastroenterology 52:78, 1967. 9. Gutman, F. A.: Evaluation of a patient with central retinal vein occlusion. Ophthalmology 90:481,1983. 10. Schneiderman, J. H., Sharpe, J. A., and Sutton, D. M.: Cerebral and retinal vascular complications of inflammatory bowel disease. Ann. Neurol. 5:331, 1979. 11. Talbot, R. W., Heppell, J., Dozois, R., and Beart. R. W.: Vascular complications of inflammatory bowel disease. Mayo Clin. Proc. 61:140, 1986. 12. Kehoe, E. L., and Newcomber, K. L.: Thromboembolic phenomena in ulcerative colitis. Arch. Intern. Med. 113:711, 1964. 13. Mayeux, R., and Fahn, S.: Strokes and ulcerative colitis. Neurology 28:571, 1978. 14. [ampol, L. M., Isenberg, S. J., and Goldberg, M. F.: Occlusive retinal arteriolitis with neovascularization. Am. J. Ophthalmol. 81:583, 1976. 15. Lam, A., Borda, 1. T., Inwood, M. J., and Thompson, S.: Coagulation studies in ulcerative colitis and Crohns disease. Gastroenterology 68:245, 1975.
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16. Jong, E., Porte, R., Knot, E., Verheijen, J., and Dees, J.: Disturbed fibrinolysis in patients with inflammatory bowel disease. A study in blood plasma, colon mucosa, and faeces. Gut 30:188,1989. 17. Juhan-Vague, I., Valadier, J., Alessi, M. c.. Aillaud, M. F., Ansaldi, J., Philip-Ioet, c., Holvoet, P., Serradimigni, A., and Collen, D.: Deficient t-PA release and elevated PA inhibitor levels in patients with spontaneous or recurrent deep venous thrombosis. Thromb. Haemost. 1:67, 1987. 18. Edwards, R. 1., Levine, J. 1., Green, R., Duffy, J., Mathews, E., Brande, W., and Rickles, F. R.: Activa-
353
tion of blood coagulation in Crohn's disease. Gastroenterology 92:329,1987. 19. Zegarra, H., Gutman, F. A., and Conforte, J.: The natural course of central retinal vein occlusion. Ophthalmology 86:1931, 1979. 20. Sanders, M. D., and Graham, E. M.: Retinal vasculitis. Postgrad. Med. J. 64:488, 1988. 21. Shimuzo, T., and Ogino, T.: Clinico-pathological studies on the intestinal lesions in Behcet's disease with special reference to entero-Behcets syndrome. Stomach Intestine 10:1593, 1975.
OPHTHALMIC MINIATURE
Some men never injure themselves by being ridiculous .... A pamphlet has been received containing the names of "upwards of nine hundred persons operated on for the cure of strabismus or squinting by T.]. Crossman, M.D., with introductory remarks, notices of the public press, and correspondence with patients." Well may the doctor exclaim, "eyes right," after having pocketed, as acknowledged on the 9th page, $45,000 .... Here is a young man, whose name rarely, if ever, appears in places where we are accustomed to find a catalogue of distinguished benefactors of the human race, who has secured a larger fortune within two or three years, according to his own account, by the practice of a trivial operation, than some of the most distinguished surgeons on the Continent acquire in half a century. Editorial note: Boston Med. Surg. J. 29:123-124, 1843.
Accepted for publication July 25, 1990. From the Department of Ophthalmology, Northwestern University Medical School, Chicago, Illinois. This study was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc. Reprint requests to Lee M. [arnpol, M.D., Northwestern University Medical School, 303 E. Chicago Ave., Chicago, IL 60611.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
110:349-353,
every organ system in the body.I" There have, however, only been a few reported cases of retinal vascular disease in conjunction with inflammatory bowel disease." We treated two patients with retinal vascular disease and Crohns disease.
Case Reports Case 1 A 33-year-old woman, in whom Crohns disease was diagnosed in 1972, had an eight-week history of blurred vision and micropsia in the left eye in October 1980. The patient was taking 50 mg of prednisone every other day and sulfasalazine four times a day as treatment for the inflammatory bowel disease. She had no previous ocular complaints and was otherwise in good health. Physical examination disclosed best-corrected visual acuity of R.E.: 20/25 and L.E.: 20/ 400. Results of external examination, pupillary examination, and confrontational visual fields were all normal. Results of slit-lamp examination were normal in both eyes, except for a few cells in the anterior chamber of the left eye. Intraocular pressure was R.E.: 17 mm Hg and L.E.: 15 mm Hg. Results of ophthalmoscopic examination of the right eye were normal, except for a choroidal nevus. Examination of the left eye (Fig. 1) disclosed marked disk edema with many peripapillary nerve fiber layer hemorrhages. The venous system was dilated with dot and blot hemorrhages throughout the peripheral retina. The macula was edematous with cystoid spaces. A fluorescein angiogram (Fig. 1) showed sluggish venous flow with rnicroaneurysms, capillary dilatation, and a late cystoid pattern. Disk leakage was present. Results of laboratory studies including protein electrophoresis, fluorescent treponema I antibody absorption test, rapid plasma reagin, antinuclear antibody, sedimentation rate, complete blood cell count, C3, and thyroid studies were all within normal limits. A diagnosis of central retinal vein occlusion OCTOBER,
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Fig. 1 (Ruby and Jampol). Case 1. Left, Left eye showing central retinal vein occlusion with marked disk edema and peripapillary nerve fiber layer hemorrhages. Right, Fluorescein angiogram of the left eye showing cystoid macular edema and late disk leakage.
was made. At the time of the most recent follow-up in April 1990, examination disclosed visual acuity of L.E.: counting fingers with macular pigment mottling and retinal fibrosis. No evidence of neovascularization was seen. The patient continues to have flare-ups of ileitis and continues to take varying doses of prednisone and sulfasalazine. Case 2 A 25-year-old woman, in whom bilateral iridocyclitis was diagnosed in 1983, was referred in May 1984 with vitreous floaters and blurred vision in the left eye for one week. The patient also complained of mouth ulcers that began after she took a sulfa antibiotic for an upper respiratory tract infection. She had not been treated for iritis since the initial episode. The patient's mother had Crohns disease. Bestcorrected visual acuity was R.E.: 20/20 and L.E.: 20/20 - 2. Results of color vision test, external examination, and motility were all normal. Slit-lamp examination showed a few residual posterior synechiae in the left eye with pigment deposition on the anterior lens capsule. Results of the remainder of the anterior segment examination were normal. Results of ophthalmoscopic examination of the right eye were unremarkable. Examination of the left eye (Fig. 2) disclosed mild vitreous cells, arteriovenous crossing changes with arteriolar attenuation, and venous dilatation with sheathing. There were scattered cotton-wool spots. Retinal vascular sheathing was seen (especially inferotemporally) involving the arteries and, to a lesser degree, the veins. The disk margins were blurred. Hemorrhages were scattered in the
distribution of the inferotemporal vein. A fluorescein angiogram showed markedly delayed filling of the vessels in the inferotemporal quadrant with a branch artery occlusion, disk leakage, and staining of the veins (Fig. 2). Results of laboratory investigations including chest x-ray, complete blood cell count, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, fluorescent treponemal antibody absorption test, Toxoplasma titers, angiotensin-converting enzyme, and purified protein derivative (tuberculin) test were normal. She was given 60 mg of prednisone every day with a gradual taper. By November 1984, she had discontinued taking all corticosteroids and showed no evidence of active vasculitis. the patient had iritis in the right eye in February 1985 and was treated with topical corticosteroids with subsequent resolution. She was noted to have minimal peripheral venous sheathing in the right eye in March 1988. Crohn's disease was diagnosed after colonoscopy and intestinal biopsy in 1988. The symptoms of gastrointestinal disease had been present intermittently for many years. The patient has been followed up since that time without any flareups of the retinal vasculitis and visual acuity has remained 20/20 in both eyes. She is not being treated with corticosteroids for the bowel disease.
Discussion Crohns disease is a multisystem disease characterized by arthritis, skin manifestations,
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351
Fig. 2 (Ruby and Iampol), Case 2. Left, Left eye showing retinal vascular sheathing with multiple hemorrhages and cotton-wool spots. Right, Fluorescein angiogram of the left eye showing disk leakage and staining of the arteries and veins.
malabsorption, gallstones, kidney stones, osteoporosis, liver disease, peptic ulceration, corneal ulcers, keratitis, Sjogren's syndrome, blepharitis, scleritis, myositis, orbital and eyelid edema, retrobulbar neuritis, and neuroretlnitis.Pv" Many of the ocular conditions are believed to be secondary to the same phenomenon causing the underlying disease, namely immunologic disease that affects both the eyes and the bowel, possibly autoimmune in nature." Although many ocular manifestations of Crohn's disease have been reported, one patient manifesting primarily retinal vasculitis has been described. Duker, Brown, and Brooks? described a patient with Crohn's disease, which was confirmed by a biopsy specimen, who manifested severe, bilateral, asymmetric, obliterative retinal arteritis and phlebitis. Several cases that share some similarities with this case have also been reported. Macoul' described a patient with Crohns disease who showed evidence of vasculitis in the form of bilateral, severe neuroretinitis and hyalitis associated with recurrent conjunctivitis, bilateral iridocyclitis, and marginal corneal infiltrates and ulcers. Ellis and Gentry3 reported a case of retinal and disk edema associated with a hazy vitreous in a patient with Crohn's ileitis. Many conditions may predispose an individual to central or branch retinal vein occlusions, including alterations of blood flow, altered viscosity of the blood, and abnormalities of the coagulation system and of the vessel walls. Systemic diseases that can cause these changes include blood dyscrasias, dysproteinemias, vasculitis, and autoimmune diseases such as
rheumatoid arthritis and systemic lupus erythematosus." The association of inflammatory bowel disease with systemic vascular disease has been well documented. Thromboembolism is a recognized complication of inflammatory bowel disease that can involve the systemic vasculature as well as the cerebral and retinal vessels. Schneiderman, Sharpe, and Sutton" described five patients who had cerebral or retinal vascular occlusions in association with inflammatory bowel disease. One patient with Crohn's disease, which was confirmed by a biopsy specimen, had bilateral retinal branch artery occlusions. Fluorescein angiography showed no evidence of intrinsic retinal vascular disease. No evidence of embolic phenomena could be documented, since the patient had a normal carotid arteriogram and echocardiogram. Four other patients had cerebrovascular occlusions. An increased platelet count was identified in four of the five patients described. Neither of our patients demonstrated an increased platelet count. Talbot and associates" surveyed records of 7,199 patients with chronic ulcerative colitis or Crohn's disease over an II-year period. Thromboembolic complications developed in 92 (1.3%) of these patients (49 patients with Crohn's disease). Other authors have reported cases of central retinal artery occlusion associated with ulcerative colitiS. 12•13 These cases are similar to idiopathic retinal arteritis in which the patients, mostly young, manifest multiple retinal arterial occlusions." Alterations in the coagulation system that can occur with inflammatory bowel disease can also contribute to thrombosis. Coagulation
352
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abnormalities that have been reported include accelerated thromboplastin generation, and increased levels of factor V, factor VIII, fibrinogen, and low antithrombin III concentrations." Jong and associates" studied fibrinolytic activity in plasma and colonic mucosal biopsy specimens of 28 patients with inflammatory bowel diseases. In plasma of patients with inflammatory bowel disease, significantly decreased tissue-type plasminogen activator activity, increased plasminogen activator inhibition and fibrinogen, and prolonged thrombin and prothrombin times were found. Patients with high plasminogen activator inhibition concentrations are at risk for developing deep venous thrombosis." Increased plasma fibrinopeptide A levels and enhanced generation of monocyte tissue factor activity in patients with Crohns disease may contribute to the activation of blood coagulation." It has been proposed that only patients with active inflammatory bowel disease suffer from this hypercoagulable state and that stable patients are not at increased risk of thromboembolism." Neither of our patients had a history of previous thromboembolism and neither had coagulation studies performed. Retinal vasculitis as a separate clinical entity has been reported as an isolated finding or in association with such systemic conditions as systemic lupus erythematosus, Behcets disease, sarcoidosis, polyarteritis nodosum, Wegener's granulomatosis, tuberculosis, syphilis, multiple sclerosis, and ankylosing spondylitis." Most of these conditions have an underlying immunologic abnormality, which contributes to the disease process. Characteristics of retinal vasculitis may include diffuse capillary leakage, cotton-wool spots, vascular sheathing, vascular occlusion, vitreous cells, and retinal hemorrhages. Our second patient manifested most of these characteristics. Her retinal findings were similar to those seen in some patients with Behcets disease. Behcets disease, more common in males, is characterized by the classic triad of mouth ulcers, genital ulcers, and iritis. Diffuse capillary leakage, branch retinal vein or artery occlusions, infiltrates, and a terminal stage showing optic atrophy and macular pigmentary changes comprise the retinal findings. Gastrointestinal lesions simulating Crohns disease can often be found in patients with Behcet's disease, which cause diagnostic difficulties." A complete rheumatologic examination in Case 2 found no sys-
temic abnormalities to support a diagnosis of Behcets disease. This patient developed oral ulcers only after being treated with sulfa antibiotics for a respiratory infection. Additionally, aphthous stomatitis can be seen in up to 11 % of patients with Crohn's disease and represents the mouth lesion most often seen in these patients."
References 1. Goldner, F., and Kraft, S. D.: Internal Medicine, ed. 1. Boston, Little, Brown and Company, 1983, pp. 134-144. 2. Greenstein, A. J., Janowitz, H. D., and Sachar, D. B.: The extraintestinal complication of Crohn's disease and ulcerative colitis. A study of 700 patients. Medicine 55:401, 1976. 3. Ellis, P. P., and Gentry, J. H.: Ocular complications of ulcerative colitis. Am. J. Ophthalmol. 58:779, 1964. 4. Macoul, K. L.: Ocular changes in granulomatous ileocolitis. Arch. Ophthalmol. 84:95, 1970. 5. Crohn, B. B.: Ocular lesions complicating ulcerative colitis. Am. J. Med. Sci. 169:260, 1925. 6. Petrelli, E. A., McKinley, M., and Troncole, F. J.: Ocular manifestations of inflammatory bowel disease. Ann. Ophthalmol. 14:356, 1982. 7. Duker, J. S., Brown, G. c.. and Brooks, L.: Retinal vasculitis in Crohn's disease. Am. J. Ophthalmol. 103:664,1987. 8. Korelitz, B. J., and Coles, R. S.: Uveitis (iritis) associated with ulcerative/granulomatous colitis. Gastroenterology 52:78, 1967. 9. Gutman, F. A.: Evaluation of a patient with central retinal vein occlusion. Ophthalmology 90:481,1983. 10. Schneiderman, J. H., Sharpe, J. A., and Sutton, D. M.: Cerebral and retinal vascular complications of inflammatory bowel disease. Ann. Neurol. 5:331, 1979. 11. Talbot, R. W., Heppell, J., Dozois, R., and Beart. R. W.: Vascular complications of inflammatory bowel disease. Mayo Clin. Proc. 61:140, 1986. 12. Kehoe, E. L., and Newcomber, K. L.: Thromboembolic phenomena in ulcerative colitis. Arch. Intern. Med. 113:711, 1964. 13. Mayeux, R., and Fahn, S.: Strokes and ulcerative colitis. Neurology 28:571, 1978. 14. [ampol, L. M., Isenberg, S. J., and Goldberg, M. F.: Occlusive retinal arteriolitis with neovascularization. Am. J. Ophthalmol. 81:583, 1976. 15. Lam, A., Borda, 1. T., Inwood, M. J., and Thompson, S.: Coagulation studies in ulcerative colitis and Crohns disease. Gastroenterology 68:245, 1975.
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16. Jong, E., Porte, R., Knot, E., Verheijen, J., and Dees, J.: Disturbed fibrinolysis in patients with inflammatory bowel disease. A study in blood plasma, colon mucosa, and faeces. Gut 30:188,1989. 17. Juhan-Vague, I., Valadier, J., Alessi, M. c.. Aillaud, M. F., Ansaldi, J., Philip-Ioet, c., Holvoet, P., Serradimigni, A., and Collen, D.: Deficient t-PA release and elevated PA inhibitor levels in patients with spontaneous or recurrent deep venous thrombosis. Thromb. Haemost. 1:67, 1987. 18. Edwards, R. 1., Levine, J. 1., Green, R., Duffy, J., Mathews, E., Brande, W., and Rickles, F. R.: Activa-
353
tion of blood coagulation in Crohn's disease. Gastroenterology 92:329,1987. 19. Zegarra, H., Gutman, F. A., and Conforte, J.: The natural course of central retinal vein occlusion. Ophthalmology 86:1931, 1979. 20. Sanders, M. D., and Graham, E. M.: Retinal vasculitis. Postgrad. Med. J. 64:488, 1988. 21. Shimuzo, T., and Ogino, T.: Clinico-pathological studies on the intestinal lesions in Behcet's disease with special reference to entero-Behcets syndrome. Stomach Intestine 10:1593, 1975.
OPHTHALMIC MINIATURE
Some men never injure themselves by being ridiculous .... A pamphlet has been received containing the names of "upwards of nine hundred persons operated on for the cure of strabismus or squinting by T.]. Crossman, M.D., with introductory remarks, notices of the public press, and correspondence with patients." Well may the doctor exclaim, "eyes right," after having pocketed, as acknowledged on the 9th page, $45,000 .... Here is a young man, whose name rarely, if ever, appears in places where we are accustomed to find a catalogue of distinguished benefactors of the human race, who has secured a larger fortune within two or three years, according to his own account, by the practice of a trivial operation, than some of the most distinguished surgeons on the Continent acquire in half a century. Editorial note: Boston Med. Surg. J. 29:123-124, 1843.
