Hemorrhagic Descemet Membrane Detachment Following Syphilitic Interstitial Keratitis

CASE REPORT

Roland Höllhumer, MBChB, FC Ophth(SA), MMed,*† Aida Zairani MZ, MBChB, MS Ophthal (UKM),*‡ and Stephanie Watson, BSc(Med), MMBS, FRANZCO, PhD*§

CASE REPORT Purpose: fl Syphilitic interstitial keratitis is a stromal in ammatory A 65-year-old female presented to the ophthalmic emergency disease with characteristic secondary vascularization. This case department with a 2-day history of sudden painless vision loss in the illustrates a late complication of hemorrhagic Descemet right eye. There was no history of trauma or recent surgery. She was membrane detachment. a known type 2 diabetic on oral hypoglycemic medication and Methods: insulin injections. Case report. On examination her unaided visual acuity was counting fingers in the right improving to 6/45 with pinhole, and 6/12 in Results: The patient presented with painless sudden visual loss and the left improving to 6/7.5 with pinhole. On slit lamp examination, progressive shallowing of the anterior chamber caused by hemor- she had a right deep intrastromal corneal hemorrhage inferiorly, rhagic Descemet membrane detachment. She had corneal neo- which measured 6.0 mm (width) by 3.6 mm (height) (Fig. 1). A vascularization and a positive syphilis serology. Owing to the risk superior area of deep stromal bleeding was also noted. No infiltrate of pupil block glaucoma, the patient had surgical drainage of the was present, and the hemorrhage masked the presence of vessels in blood via an ab externo approach. the right eye. The anterior chamber was shallow and quiet, and there was no hyphema. The left cornea had ghost vessels superiorly, but Conclusions: This case illustrates a previously unreported com- was otherwise normal. The intraocular pressures were 9 mm Hg in plication of syphilitic interstitial keratitis. The patient recovered good the right and 14 mm Hg in the left eye. The posterior segments were visual acuity and had residual pigment deposits in the pre- normal bilaterally with no evidence of previous uveitis or Descemet interface. diabetic retinopathy. On systemic evaluation her blood pressure was 135/85 mm Key Words: syphilis, pre-Descemet hemorrhage, cornea, interstitial Hg and the random blood glucose was 6.5 mmol/L; there was no keratitis systemic evidence of congenital or acquired syphilis. – The patient was assessed as having had previous bilateral (Cornea 2016;35:1255 1256) interstitial keratitis, presenting now with a complication of intrastromal bleeding from the ghost vessels. Optical coherence tomography identified the blood to be between the stroma and nterstitial keratitis is a stromal inflammatory disease with Descemet membrane (Fig. 2). The patient was admitted for bed Icharacteristic secondary vascularization. Syphilis is a com- rest, workup, and monitoring. Owing to the increasing hemor- mon cause of interstitial keratitis, with an autoimmune rhage and concerns of a possible pupil block, the patient was inflammatory process developing 5 to 15 years after the taken to the operating room for evacuation of the blood. This was primary infection.1–3 Neovascularization develops in achieved by a clear corneal incision into the hematoma and response to inflammation, and subsequently, the vessels balanced salt solution washout of the interface. An anterior chamber air tamponade was kept to prevent further bleeding and become ghost vessels with quiescence. Rupture of these facilitate apposition of Descemet membrane to the stroma. There vessels has been described with elevated blood pressure, 1,4 was a small reaccumulation of blood in the postoperative period, trauma, or surgery. which subsided with conservative measures. The patient was kept on topical prednisolone acetate 1% chloramphenicol, and phen- ylephrine 2.5% in the postoperative period. Investigations re- Received for publication February 18, 2016; revision received March 28, vealed positive Treponema pallidum antibodies, and appropriate 2016; accepted March 31, 2016. Published online ahead of print May 25, systemic management for syphilis was implemented. Investiga- 2016. tions for sarcoidosis, tuberculosis, and a coagulopathy From the *Sydney Eye Hospital, Sydney, Australia; †University of the were negative. Witwatersrand, Johannesburg, South Africa; ‡Universiti Kebangsaan, In the last review 8 months after the hemorrhage, her visual Malaysia, Kuala Lumpur, Malaysia; and §Save Sight Institute, University acuity was 6/9 in the right eye and there was a small amount of of Sydney, Sydney, Australia. residual pigment from the hemoglobin breakdown at the interface. Clinical Professor Stephanie Watson is supported by a National Health and Medical Research Council (NHMRC) Career Development Fellowship (APP1050524). The authors have no other funding or conflicts of interest to disclose. DISCUSSION Reprints: Roland Höllhumer, MBChB, FC Ophth(SA), MMed, Sydney Eye Hospital Foundation, 8 Macquarie St, Sydney 2000, Australia (e-mail: This is a case of syphilitic interstitial keratitis that [email protected]). developed a spontaneous pre-Descemet hemorrhage. To our Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. knowledge, this is the first report of this rare complication of

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FIGURE 2. Optical coherence tomography depicting the location of the hemorrhage in the pre-Descemet plane. No pupil block is evident at this time. FIGURE 1. Paracentral intracorneal hemorrhage measuring 6.0 mm (width) by 3.6 mm (height). any interface fluid or blood. They report good results with 2 of their cases managed in this way. interstitial keratitis. The patient had a history of bilateral ocular Similarly, we performed ab externo drainage of the symptoms at the age of 10, bilateral superior ghost vessels, and blood and washed the interface with balanced salt solution. positive syphilis serology, suggesting a diagnosis of congenital An air fill was left in the anterior chamber at 100% for interstitial syphilitic keratitis. She had no cutaneous manifes- 10 minutes and then 40%. A small amount of blood tations or history to support a diagnosis of acquired syphilis. reaccumulated in the days after the surgery but resorbed The patient presented suddenly without any apparent spontaneously over months, while she was on topical steroid reason for the vessels to bleed at this time. There was no and vasoconstrictors. As shown by Sharma and Stone,6 it is history of trauma or recent surgery, and her blood pressure expected that the iron deposits will resorb with time and and coagulation screen were normal. Inadvertent rubbing become less evident. or minor trauma to the eye was presumed. This case illustrates a late complication of syphilitic Hemorrhage between the stroma and Descemet mem- interstitial keratitis with hemorrhagic Descemet membrane brane most commonly occurs in association with canaloplasty detachment. The patient was managed with an ab externo surgery, at an incidence of 1.1% to 3%. With prompt corneal drainage procedure and recovered good vision. The evacuation of the blood and tamponade with air in the anterior ab externo approach has the benefit of preserving the integrity 4 chamber these patients can have a good outcome. of Descemet membrane, thereby decreasing the likelihood of Hemorrhagic Descemet membrane detachment has further endothelial surgery. been reported to cause pupil block with severe elevation in intraocular pressure.5 To lower the intraocular pressure in this case, neodymium-doped yttrium aluminum garnet (ND- REFERENCES YAG) laser puncture of Descemet membrane and multiple 1. Wilhelmus KR. Syphilitic stromal keratitis. In: Krachmer JH, Mannis MJ, paracentesis was needed. Descemet stripping automated Holland EJ, ed. Cornea. United States: Elsevier Health Sciences; 2010: endothelial keratoplasty (DSAEK) was subsequently required 1043–1063. because of the inadvertent trauma to Descemet membrane. 2. Kiss S, Damico FM, Young LH. Ocular manifestations and treatment of Our patient had normal intraocular pressures; however, as the syphilis. Semin Ophthalmol. 2005;20:161–167. hemorrhage was increasing with the risk of pupil block, the 3. Aldave AJ, King JA, Cunningham ET. Ocular syphilis. Curr Opin Ophthalmol. 2001;12:433–441. hemorrhage was drained surgically. 4. Re˛kas M, Petz K, Wierzbowska J, et al. Evacuating a pre-Descemet 4 Rekas et al describe an ab externo technique for hematoma through a clear corneal incision during a canaloplasty pro- evacuation of the hemorrhage that preserves the integrity of cedure. J Cataract Refract Surg. 2014;40:1953–1957. Descemet membrane. They describe a clear corneal incision 5. Eide Macpherson JF, Slettedal JK, Drolsum L, et al. Spontaneous hemorrhagic Descemet membrane detachment causing pupillary block. into the site of the hematoma and evacuation with a spatula. Eur J Ophthalmol. 2012;15:819–822. The anterior chamber is left with a full air fill to facilitate 6. Sharma PS, Stone DU. Corneal blood staining secondary to Hemorrhagic apposition of the separated layers and allow further egress of Descemet membrane detachment. Cornea. 2007;26:1273–1274.