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Show JOl/ rnal of Clil/ ical Nfllro- Ol'/, tllalm% gy 12( 3): 167- 170, 1992. Interstitial Keratitis and Iridoschisis in Congenital Syphilis A. J. E. Foss, F. R. C. S., F. C. Ophth., P. G. Hykin, F. R. C. S., F. C. Ophth., and L. Benjamin, F. R. C. S., F. C. Ophth. " j 19Y2 Raven Press, Ltd., New York Bilateral interstitial keratitis and iridoschisis are reported in four cases with extraocular stigmata of congenital syphilis and positive syphilis serology. The iridoschisis was extensive in two cases giving the iris an unusual ragged appearance, while it was slight in one case. Iridoschisis may suggest the diagnosis of congenital syphilis, especially when interstitial keratitis is mild. Chronic open- angle glaucoma should be excluded in all patients with interstitial keratitis and iridoschisis. Key Words: Congenital syphilis- Interstitial keratitislridoschisis. From the Oxford Eye Hospital, Walton Street, Oxford OX2 6AN, England. Address correspondence and reprint requests to Mr. P. G. Hykin, Institute of Ophthalmology, 17/ 25 Cayton Street, London ECIV 9AT, England. 167 Acquired syphilis remains an important cause of neuro- ophthalmic disease, with more than 48,000 cases reported in the United States in 1990 ( I), of which it is estimated that 10% of untreated cases develop neurological disease ( 2). Less emphasized in recent years are the difficulties that elderly patients experience from the stigmata of congenital syphilis. While interstitial keratitis and chorioretinal scarring are well recognized and may cause reduced visual acuity and visual field defects ( 3), other features, including recurrent anterior uveitis ( 3), steroid- induced uveitis ( 4), cataract ( 3,5), band keratopathy ( 3,5), keratoconus, and chronic openangle glaucoma ( 5,6), have been reported. Several isolated cases of iridoschisis have also been described ( 7), but only recently has an association between interstitial keratitis and iridoschisis in congenital syphilis been proposed ( 8,9). We report a further four such cases, and emphasize the apparent high incidence of chronic open- angle glaucoma in these patients. CASE REPORTS Case 1 A 51- year- old woman gave a history of poor visual acuity as a child, attending a school for the partially sighted from the age of 16 years. Her right eye was profoundly amblyopic, and a large- angle right esotropia was corrected when she was 8 years old, and a secondary right exotropia was corrected when she was 43. She had normal facies but characteristic Hutchinson's teeth. Right visual acuity was CF at 1 m and left 20/ 200. There was severe scarring of the left cornea with both patent and ghost vessels secondary to interstitial keratitis, which was mild in the right eye. Both anterior chambers were quiet, and there were no keratoprecipitates. There was atrophy of the stroma of the anterior stromal leaf of 168 A. J. E. FOSS ET AL. both irides with preservation of radial fibers ( Fig. 1). The posterior iris stroma was well preserved ( Fig. 2), and there was no iris retroillumination. Right intraocular pressure was 15 mmHg and left 16 mmHg, and goniscopy showed grade 2 open anterior chamber angles in both eyes, with moderate pigment deposition but no peripheral anterior synechiae. Pupil responses were normal, and there was no relative afferent pupillary defect. There was bilateral chorioretinal scarring and optic atrophy. Treponema pallidum hemagglutination ( TPHA) and fluorescent treponemal antibody absorption ( FTA- Abs) were positive ( Table 1). Case 2 A 70- year- old woman complained of reduced vision in both eyes for 6 months. At age 20 she had presented with bilateral interstitial keratitis, which resolved spontaneously, but profound sensorineural deafness developed 10 years later. A Wasserman reaction was positive, and she was treated with intramuscular penicillin. Chronic open- angle glaucoma had been diagnosed at age 60, but intraocular pressures were normalized, and visual field loss and pathological disk cupping halted on topical antiglaucoma therapy. On examination, there was maxillary hypoplasia and an absent nasal bridge, and she was profoundly deaf. Right visual acuity was 20/ 80 and left 20/ 60. Slit lamp examination showed diffuse bilateral interstitial keratitis with central corneal thinning and prominent ghost vessels. Both anterior chambers were quiet and there was no iris retroil- FIG. 2. High power view ot the ngnt iriS or Case I showing loss of the substance of the anterior stromal leaf with preserJation of radial stromal fibers ( arrow). lumination. The substance of the anterior stromal leaf of the iris was lost in the right eye with preservation of radial fibers extending from the iris root to the pupillary margin. There was mild iridoschisis in the left eye. Right intraocular pressure was 18 mmHg, left 18 mmHg, and gonioscopy showed a limited view of an open- angle with moderate pigment deposition and no peripheral anterior synechiae. Pupil responses were normal and there was no relative afferent pupillary defect. Fundal e~ aminati? n showed bilateral glaucomatous optic d~ sk CUppIng, ~ ut normal retinas. Despite reduced VIsual acUIty, Visual field examination showed constriction of both visual fields. TPHA and FTA- Abs were positive ( Table 1). Case 3 A. 73- year- old woman complained of poor visual a~ Ulty. On entering military service 48 years preVIOusly, best corrected visual acuity was right and left 20/ 60. A year later she experienced an acute episode of bilateral interstitial keratitis, after which her visual acuity further deteriorated. She had no systemic stigmata of congenital syphilis. FIG. 1. Gene~ alized iridoschisis ( arrows) involving the , ",' v · ~ " f '_ · 8se 1 Interstitial keratitis was slight. Case VORL 1 ve 2 - ve 3 weak + ve 4 - ve TABLE 1. TPHA + ve + ve + ve +/- ve FTA + ve + ve + ve + ve Serum 19M vs Trep. P. - ve - ve - ve ve SYPHILITIC KERATITIS AND IRIDOSCHISIS 169 Right visual acuity was 20/ 80 and left 20/ 200. There was marked interstitial keratitis with prominent ghost vessels in both eyes ( Fig. 3). The right iris showed patchy loss of the anterior stromal leaf with relative preservation of radial fibers ( Fig. 4). The left iris showed similar changes confined to the lower half of the iris. Right and left intraocular pressures were 16 mm Hg. Both pupils were irregular, but there was no relative afferent pupillary defect. There was nuclear sclerosis in the left eye, bilateral chorioretinal scarring, and slight pallor of both optic nerve heads. A left penetrating keratoplasty with extracapsular cataract extraction was performed without complication. At 6 months, left visual acuity had improved to 201120. Syphilis serology was positive ( Table 1). Case 4 A 75- year- old woman with congenital deafness gave a 24- year history of bilateral chronic openangle glaucoma. Intraocular pressures were normalized with topical antiglaucoma medication for 10 years, before intraocular pressures rose to above 50 mmHg on full topical therapy and systemic acetazolamide. Uncomplicated trabeculectomy was performed in the right and then the left eye. Intraocular pressures remained normal on no additional medical therapy for 14 years. Right extracapsular cataract extraction and penetrating keratoplasty were performed 10 years after the original surgery when marked nuclear sclerosis developed in the right eye. Right visual acuity was hard movements ( HM) and left 20/ 200. A clear, penetrating keratoplasty was noted in the pseudophakic right eye. Interstitial keratitis and old keratoprecipitates were present in the left eye. The right iris was normal, but the left eye showed mild loss of the anterior stromal leaf in the ciliary portion of the inferior iris. Right intraocular pressure was 16 mmHg, and left 5 mmHg, and gonioscopy showed grade 2 open anterior chamber angles with mild pigment deposition. Pupil responses were normal, and there was no relative afferent pupillary defect. There was a small right Nd: Yag posterior capsulotomy and moderate nuclear sclerosis in the left eye. There was a right macular scar. The inferior rim of the right optic disk was notched and corresponded to a large right superior arcuate scotoma. Right and left cup to disk ratio was 0.5. Bilateral inferionasal chorioretinal scarring was present. TPHA and FTA- Abs were positive ( Table 1). DISCUSSION We report four cases of congenital syphilis, three of which had well- recognized extraocular stigmata and all of whom had positive syphilis serology ( 10). Interstitial keratitis was present in all cases; prominent ghost vessels were present in two cases, and one case had significant stromal thinning. Presentation of two patients with interstitial keratitis at ages 20 and 26 was typical and common when congenital syphilis was more frequently encountered ( 5). Iridoschisis involving predominantly the inferior half of the iris was present in all cases ( 7). Preservation of anterior stromal leaf radial fibers and deep stromal iris tissue without transillumination were all characteristic findings. FIG. 3. Marked interstitial keratitis in the right eye of Case 3. I Clin Nel4To- ophtha/ mol, Vol. 12. No. 3, 1992 170 A. J. E. FOSS ET AL. FIG. 4. Iridoschisis in the right eye of Case 3 ( arrows). Correctopiae and ectropion uveae were absent ( 7). Despite difficulties with gonioscopy, no peripheral anterior synechiae or fibrovascular membranes were seen to account for the open- angle glaucoma that developed in Cases 2 and 4. This was unusual, in that a narrow- angle and peripheral anterior synechiae are well reported in large series of cases of interstitial keratitis ( 5), although they presumably represent more severe cases, and it is possible that at least two of our cases were mild, in that the patients could not remember the clinical episode. The association of interstitial keratitis and iridoschisis has only been reported in individual cases before. While interstitial keratitis is a common manifestation of congenital syphilis, iridoschisis has rarely been reported, although difficulty in visualizing the iris in cases with interstitial keratitis may in part explain this. The fact that we have identified four cases suggests the association may be more common than is generally realized. Neither interstitial keratitis nor iridoschisis have a suppurative component, and both are presumably immunologic in their pathogenesis. Changing levels of antigen in patients with congenital syphilis may explain the frequent delayed onset of interstitial keratitis. Some cases of interstitial keratitis are reported after the onset of treatment, suggesting that dead tissue antigen or protein may suddenly be released from the corneal stroma into the limbal vascular arcades and playa significant role in pre- , """ 1/", 1",,, 1, Vol. 12, No. 3, 1992 cipitating the disease. The fact that the int~ ct blood- aqueous barrier prevents many protems from coming into contact with iris stroma perhaps explains the less common incidence of iridoschisis. Two of our cases had chronic open- angle glaucoma, eventually requiring trabeculectomy. In both cases the anterior chamber angle was grossly normal with no peripheral anterior synechiae or visible fibrovascular membrane. Chronic openangle glaucoma with isolated iridoschisis in the absence of peripheral anterior synechiae or apparent angle pathology is well reported and the etiology remains obscure. It is presumed that an as yet unrecognizable abnormality exists within the trabecular meshwork, although this has not been confirmed histologically. There may be an association between interstitial keratitis and iridoschisis in congenital syphilis. This is an important association to be aware of because, in cases in which interstitial keratitis is mild, iridoschisis may suggest the diagnosis of congenital syphilis. Chronic open- angle glaucoma would appear to be a sequela of this association due to a presumed abnormality of the trabecular meshwork. We emphasize the need for glaucoma to be excluded in cases of interstitial keratitis and iridoschisis. Acknowledgments: We would like to thank Mr. Awdry and Mr. Cheng for allowing us to report their patients. REFERENCES 1. Centers for Disease Control. Cases of specified notifiable diseases, United States. MMWR 1991; 39: 994. 2. Merrit HH, Adams RA, Solomon He. Neurosyphilis. New York: Oxford University Press, 1946: 443. 3. Duane TD, ed. Bacterial uveitis. In: Clinical ophthalmology. Vol 4. Philadelphia: Lippincott, 1988: Ch 44. 4. Shin DH, Kass MA, Kolker AE, Becker B, et al. Positive FTA- Abs tests in subjects with corticosteroid- induced uveitis. Am JOphthalmol 1976; 81: 259~ 0. 5. Duke- Elder S. Diseases of the anterior segment. In: DukeElder S, ed. System of Ophthalmology, St. Louis: CV Mosby, 1966: 815- 30, 6. Ritch R, ed. Glaucoma associated with uveitis. The glaucomas, St. Louis: CV Mosby, 1989: Ch 67. 7. Duke- Elder S. Disease of the uveal tract. In: Duke- Elder S, ed. System of Ophthalmology. St. Louis: CV Mosby, 1966: 9,694- 98. 8. Pearson PA, Amrien JM, Baldwin B, Smith TJ. lridoschisis associated with syphilitic interstitial keratitis. Am JOphthalmol 1989; 107: 88- 90. 9. Lowenstein A, Forster J, Sledge SK. lridoschisis with multiple rupture of stromal threads. Br JOphthalmoI1945' 29: 15- ~. ' 10. Paris- Harmelin A. Syphilis serology in 1991. J Clin NeuroophthalmoI1991; 1l( 3): 144- 51. |
