Br J Ophthalmol: first published as 10.1136/bjo.72.5.377 on 1 May 1988. Downloaded from British Journal of Ophthalmology, 1988, 72, 377-379 Ophthalmic complications with disseminated intravascular coagulation RICHARD B PATCHETT, W BRUCE WILSON, AND PHILIP P ELLIS From the Department ofOphthalmology, University of Colorado School ofMedicine, Denver, Colorado, USA. SUMMARY Massive lid oedema, ecchymosis, proptosis with a total restriction of extraocular movement, markedly raised intraocular pressure, and occlusion of the central retinal artery developed acutely in the right eye of a 26-year-old woman with a past history of disseminated intravascular coagulation. She had been admitted to hospital for symptoms of abdominal pain and bleeding from multiple sites a-few hours earlier. Five days previously she had some proptosis of the other eye and had been treated with antibiotics for suspected orbital cellulitis at another hospital. The oedema and proptosis resolved on high-dose intravenous corticosteroid therapy. Despite attempts to relieve the orbital oedema and raised intraocular pressure with a lateral canthotomy and antiglaucoma medications, the patient lost all perception of light in the right eye and has subsequently developed an optic nerve atrophy. Disseminated intravascular coagulation (DIC) is a and massive lid oedema with proptosis were present. syndrome involving widespread intravascular coagu- Five days prior to this admission the patient had http://bjo.bmj.com/ lation, primarily in small vessels. Associated dis- noted fever, proptosis, and lid oedema of her left eye orders have included head trauma, sepsis, surgery, and was admitted to another hospital. Although burns, obstetrical complications, liver disease, malig- there was no evidence of sinusitis or a history of nancy, and inflammatory bowel disease.' 2 Common penetrating injury, she was treated with oral and to these is the incitement of the coagulation cascade, intravenous antibiotics for orbital cellulitis. Orbital with resultant thrombosis. Consumption of clotting symptoms and signs progressed slowly despite treat- elements eventually leads to hypocoagulability and ment. On the day of transfer to the University on September 30, 2021 by guest. Protected copyright. haemorrhage.' Reported ocular complications Hospital the unaffected right eye became acutely include thrombosis of choroidal and retinal vessels, proptotic. as well as bleeding in the optic nerve sheath, vitreous, She had a history of colitis and DIC. The first choroid, and retina.-5 Similar problems are seen episode of DIC occurred two and one-half years in the brain, heart, kidney, gastrointestinal and previously during an active period of colitis when the genitourinary tracts, as well as involvement of other patient was five months pregnant. Generalised organs."5 oedema, including facial swelling, was present at that time. The patient improved on prednisone and Case report sulphasalazine therapy. Six months before the present admission she had been in hospital again with A 26-year-old woman presented to the University DIC. She improved with the addition of prednisone Hospital with severe abdominal pain, headache, and to the maintenance sulphasalazine therapy. bleeding from the urinary tract, rectum, vagina and Ophthalmic complications were not present during nose. She had ecchymosis of the knees and buttocks either episode. and petechiae of the hard palate. Generalised Our examination revealed a blind right eye, and oedema, most marked in the head and neck regions, uncorrected near vision of 20/200 in the left eye. Correspondence to Dr Philip P Ellis, Box B-204, Department of Pronounced lid oedema, proptosis, and haemorr- Ophthalmology, University of Colorado Health Sciences Center, hagic conjunctival chemosis were present (Fig. 1). 4200 East 9th Avenue, Denver, Colorado 80262, USA. Intraocular pressure by Schi0tz tonometry was 377 Br J Ophthalmol: first published as 10.1136/bjo.72.5.377 on 1 May 1988. Downloaded from 378 Richard B Patchett, WBruce Wilson, and Philip P Ellis Fig. 1 Pronouncedproptosis, lidandfacialecchymosis, Fig. 2 Computed tomographicscan shows bilateral and oedema, conjunctival chemosis, andhaemorrhage are proptosis, greater on the rightside, thickening ofextraocular evident on each side. muscles, and increased radiodensity ofoptic nerve suggestive ofhaemorrhages within thesheaths. greater than 80 mmHg in the right eye and 11 mmHg the right eye fell to 29 mmHg. Vision has remained in the left eye. There was no extraocular motility of no light perception in the right eye but improved to the right eye; there was approximately 50% of 20/40 in the left eye over 12 hours and to 20/20 over normal abduction and adduction of the left eye. several days. Orbital oedema and proptosis gradually Ophthalmoscopic examination revealed narrowed resolved, but the right eye slowly developed optic retinal arterioles and diffuse retinal oedema with a atrophy. cherry red macula in the right eye; the fundus of the left eye appeared normal. There was no evidence of Discussion http://bjo.bmj.com/ intraocular inflammation in either eye. Laboratory data from the referring hospital Among the multiple extraintestinal manifestations of included a platelet count of 160x 109/l, a prothrombin inflammatory bowel disease is hypercoagulability time (PT) of 25 seconds, a partial thromboplastin resulting from abnormal coagulation factors and time (PIT) of 100 seconds, a fibrinogen level of 0.29 platelets.6 This may have precipitated the episodes of g/l, and a fibrin split product (FSP) titre of 1:2560. DIC in our patient. Talbot et al.2 observed DIC in Repeat laboratory studies at the time of admission three patients with inflammatory bowel disease, one showed the platelet count had decreased to 69x 109/l; of whom also had sepsis. Endo et al.7 described DIC on September 30, 2021 by guest. Protected copyright. the erythrocyte sedimentation rate was 7 mm/h in two patients with ulcerative colitis; however, in (Westergren). Serological studies for antinuclear both patients the DIC followed a surgical procedure. antibody titre, rheumatoid factor, and complement This patient's coagulopathy probably involved the levels gave normal results. A pregnancy test was ophthalmic, maxillary, and episcleral vessels, result- negative. Blood and conjunctival cultures were ing in ischaemia, haemorrhage, and massive oedema. negative. Computerised tomographic examination of These factors may have led to an increased episcleral the orbits showed bilateral proptosis, thickening of venous pressure, to raised intraocular pressure, and the muscle outlines, and increased radiodensity of eventually to central retinal artery occlusion. In the optic nerves (Fig. 2). carotid-cavernous fistula the arterialisation of all Initial treatment included intravenous administra- orbital vessels may lead to an increased intraocular tion of 125 mg methylprednisolone followed by pressure.8 Presumably this occurs secondary to a intravenous dexamethasone 25 mg every 6 hours for raised episcleral venous pressure. Alternatively, four doses and then 20 mg every 6 hours for four optic nerve ischaemia or thrombus formation in the doses. A right lateral canthotomy with division of the central retinal artery may have contributed to visual canthal tendon was performed. No significant blood loss. was drained by this manoeuvre, though the tenseness The extraocular muscle dysfunction presumably of the orbital tissues was noticeably relieved. In the resulted from massive oedema, though ischaemia to subsequent seven hours the intraocular pressure in the muscles themselves is a possibility. No restriction Br J Ophthalmol: first published as 10.1136/bjo.72.5.377 on 1 May 1988. Downloaded from Ophthalmic complications with disseminated intravascularcoagulation 379 was present on forced duction testing. Periorbital References oedema has been described in a patient with throm- 1 Hamilton PJ, Stalker AL, Douglas AS. Disseminated intra- botic thrombocytopenic purpura.9 We can only vascular coagulation: a review. J Clin Pathol 1978; 31: 609-19. speculate as to whether the mechanisms may have 2 Talbot RW, Heppell J, Dozois RR, Beart RW Jr. Vascular been the same. complications of inflammatory bowel disease. Mayo Clin Proc 1986; 61: 140-5. Many arterial and venous complications have been 3 Ortiz JM, Yanoff M, Cameron JD, Schaffer D. Disseminated reported as sequelae of inflammatory bowel disease intravascular coagulation in infancy and in the neonate. Arch with or without DIC, including central retinal arterial Ophthalmol 1982; 100: 1413-5. occlusion.2 I'l Proptosis and lid oedema have been 4 Azar P, Smith RS, Greenberg MH. Ocular findings in dissemi- nated intravascular coagulation. Am J Ophthalmol 1974; 78: reported in a patient with orbital pseudotumour and 493-6. Crohn's disease; we believe our patient's course to be 5 Cogan DG. Ocular involvement in disseminated intravascular too rapid and the oedema too massive to be explained coagulopathy. Arch Ophthalmol 1975; 93: 1-8. on such a basis."3 Since our patient did not show any 6 Lam A, Borda IT, Inwood MJ, Thomson S. Coagulation studies in ulcerative colitis and Crohn's disease. Gastroenterology 1975; ocular signs of inflammation, we do not believe the 68: 245-51. inflammation associated with Crohn's disease (that 7 Endo K, Miura N, Sato T, et al. Two cases of ulcerative colitis is, uveitis or optic neuritis) was a likely contributing complicating disseminated intravascular coagulation. Jpn J cause of visual loss.'0 14 Gastroenterol