Dislocation of the crystalline lens has been described in association with numerous systemic disorders and as a complication of ocular trauma. We present a case of bilateral lens dislocation in a patient with presumed syphilitic uveitis. The mechanism of lens dislocation is related to humoral and cellular effects of inflammation, although the role of trauma cannot be ignored. Persistent uveitis should be considered as a sign of active infection

Bogorad, David D.

Rapkin, Jeffrey S.

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Henry Ford Hospital Medical Journal Volume 34 Number 3 Article 15 9-1986 Bilateral Dislocation of the Crystalline Lens in a Patient with Presumed Syphilitic Uveitis Jeffrey S. Rapkin David D. Bogorad Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Rapkin, Jeffrey S. and Bogorad, David D. (1986) "Bilateral Dislocation of the Crystalline Lens in a Patient with Presumed Syphilitic Uveitis," Henry Ford Hospital Medical Journal : Vol. 34 : No. 3 , 207-210. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol34/iss3/15 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Bilateral Dislocation of the Crystalline Lens in a Patient with Presumed Syphilitic Uveitis Jeffrey S. Rapkin, MD,* and David D. Bogorad, MD^ Dislocation ofthe crystalline lens has been described in association with numerous systemic disorders and as a complication of ocular trauma. We present a case of bilateral lens dislocation in a patient with presumed syphilitic uveitis. The mechanism of lens dislocation is related to humoral and cellular effects of inflammation, although the role of trauma cannot be ignored. Persistent uveitis should be considered as a sign of active infection. (Henry Ford Hosp MedJ 1986:34:207-10) Dislocation of the crystalline lens is a well-recognized com-plication of ocular trauma (1-3). In the absence of trauma this condition can occur in association with several recognized systemic disorders (1). With the exception of syphilis and syph-ilis-induced uveitis, dislocation of the crystalline lens has not been reported as a complication of uveitis. This case report pre-sents a patient with a long history of nongranulomatous uveitis who developed bilateral dislocation of the crystalline lens with secondary acute glaucoma over a short period of time and in the absence of admitted trauma. Case Report The patient is a 44-year-old woman, known to this clinic with a past history of uveitis. Her past medical history is significant for alcohol abuse and medical noncompliance. At the time of presentation her chief complaint was vision loss in the right eye. On examination, the best vision in the right eye was 20/300 with an aphakic correction. The intraocular tension (IOT) was 12 mm Hg, and evidence of nongranulomatous uveitis without formation of synechiae was present. The crystalline lens was dislocated inferioriy (Fig I), There was a hazy view of the fundus secondary to the inflammation. Laboratory studies revealed normal electrolytes and urinalysis. The hemoglobin was Il.O g/dL. The white blood cell count was 4,800, and the differential count was unremarkable. The Westergren erythrocyte sedimentation rate was 43 mm/hr. The chest X ray revealed no findings suggestive of sarcoidosis. The antinuclear antibody was negative, and HLA typing was negative for HLA B-27. The VDRL was negative. The treponeme hemagglutination was reactive. The patient's uveitis was treated with topical steroids and cycloplegic agents. An infectious disease consultation was obtained. The patient was known to their clinic, since she had been treated for secondary syphilis in May 1983. At that time, she had received treatment with 18 million units of parenteral penicillin daily for two days and 12 g of par-enteral ampicillin daily for six days. Against medical advice, she left the hospital before completing the prescribed ten-day regimen. At the time of this consultation, it was felt that the patient's syphilis had been adequately treated, and no further intervention was indicated. Three weeks after presentation to our clinic, the patient returned complaining of a painful right eye. The visual acuity in the right eye was counting fingers, and the IOT was 56 mm Hg. The lens had become dislocated into the anterior chamber (Fig 2). Intravenous mannitol was used to reduce the IOT. An intracapsular lens extraction and anterior vitrectomy were performed. A peripheral iridectomy was performed at the time of surgery. Postoperatively, the best vision in the right eye was 20/200. The IOT was 10 mm Hg, and there was persistence of a smoldering nongranulomatous uveitis. The vision was limited primarily by the presence of chronic cystoid macular edema with macular hole formation. On initial presentation the vision in the left eye was 20/200. A mini-mal posterior subcapsular cataract was present; this had been noted pre-viously and was unchanged from previous description. There was no evidence of dislocation of the lens nor of phakodenesis. Three months after presentation, during the patient's routine postoperative visit for the right eye, the left lens appeared minimally subluxed posteriorly along the superior border (Fig 3). There was minimal phakodenesis present. As in the right eye, the left eye showed evidence of nongranulomatous uveitis. The funduscopic examination revealed evidence of chronic macular edema. One week later, the patient retumed with "sudden vision loss" in the left eye. The best vision in the left eye was 20/70 with aphakic correc-tion. The lens was significandy dislocated posterioriy, remaining fixed at the 6:00 position. There was hinge-like motion of the lens, whose locafion varied with head position. The location of the lens ranged from nearly normal to nearly horizontal. Consideration was given to elective lens extraction. However, because of the technical difficulty of such a procedure it was felt that a safer course would be to follow the condition without surgery. Dislocation of the lens into the vitreous cavity was considered the likeliest course of events, and this condition is well toler-ated in the presence of an intact lens capsule. Submitled for publication: June 27, 1986. Accepted for publication; September 5, 1986. •Formerly Department of Ophthalmology, Henry Ford Hospital. Currenlly Departmenl of Ophthalmology, Dreyer Medical Clinic, Aurora, IL. tDepartment of Ophthalmology, Henry Ford Hospital; University of Michigan, School of Medicine, Ann Arbor, Ml. Address correspondence lo Dr Rapkin, Departmenl of Ophthalmology, Dreyer Medical Clinic, 1870 W Galena Blvd, Aurora, IL 60506. Henry Ford Hosp Med J—Vol 34, No 3, 1986 Bilateral Dislocation—Rapkin & Bogorad 207 One week later, the patient presented with a painful left eye. The vi-sion was counting fingers, and the IOT was 42 mm Hg. The lens had become dislocated into the anterior chamber (Fig 4). The patient was treated with intravenous mannitol to reduce the IOT. An intracapsular lens extraction with anterior vitrectomy and peripheral iridectomy was perfonned. Postoperatively, the best vision in the left eye was 20/100. Evidence of persistent smoldering nongranulomatous uveitis as well as chronic cystoid macular edema was present. Eig 1—Inferior dislocation of the crystalline lens in the right eye upon presentation. Discussion The association of dislocation of the crystalline lens with a number of systemic disorders is well recognized (I). The Table lists a number of the known systemic diseases that have been described in association with this condition. Marfan's syndrome is an inheritable abnormality of collagen biosynthesis. This syn-drome includes skeletal and cardiovascular abnormalities as well as ectopia lentis. Homocystinuria is an inborn enor of metabolism of the sulfur containing amino acids. The Weill-Marchesani syndrome is a rare familial condition. Both homo-cystinuria and the Weill-Marchesani syndrome have associated skeletal manifestations, as well as the ocular findings. With the exception of syphilis, the other disorders listed in the Table are either less common or more rarely associated with ectopia lentis. The associadon of lens dislocation with syphilis has been ret-rospective (1,4-7). In patients with lens subluxation in whom other etiologies were mled out. Smith et al (4) reported that 75% had positive serology for syphilis. However, in a controlled study, Rosenbaum and Podos (3) showed no significant dif-ference in serology between patients with a subluxed lens and those in the control group. They suggest that this association is somewhat less common than is implied in other reviews. The etiologic factors contributing to dislocation of the lens in pa-tients with syphilis is also unclear It has been postulated that the humoral and cellular effects of uveitis weaken the zonules (7). The role of trauma, though, is not clearly defined. The high inci-dence of alcohol abuse in patients with positive serology is well known. These patients, therefore, are at higher risk for trauma. Histologic evidence of angle recession has been described in pathologic studies of eyes with dislocated lenses, which sup-ports this theory (2). Fig 2—Dislocation of the lens (right eye) into the anterior chamber 208 Henry Ford Hosp Med J—Vol 34. No 3, 1986 Bilateral Dislocation—Rapkin & Bogorad The findings of ocular syphilis have been reported (8-14), The presence of spirochetes in aqueous humor has been identified by various authors (4-7,15,16). Their presence has also been noted in patients felt to be adequately treated for syphilis (4,15) and in patients whose serology (including the FTA-ABS) was negative (4,6). This finding is considered evidence of active syphilitic in-fection. However, the sensitivity of this test has not proven to be high when examined in a prospective study (5). Immunofluores-cent staining techniques failed to distinguish Treponema pal-lidum from other spirochetes, including theBorrelia species, which was felt to be a contaminant in the study by Ryan et al (5). Other, smaller spiral forms identified in this study were thought to be either glass shavings or species of Vibrio or Spirilla rather than tme spirochetes. This test was not performed on our pa-tient, since the decreased volume of aqueous humor present with Tabie Systemic Conditions Associated with Ectopia Lentis Marian's syndrome Homocystinuria Weill-Marchesani syndrome Hyperlysinemia Sulfite oxidase deficiency Syphilis Sturge-Weber syndrome Ehlers-Danlos syndrome Crouzon's disease Sclerodemia Mandibulofacial dysostosis Ref sum "s disea.se an anterior dislocation of the lens allows too small a sample size and greatly increases the risk of corneal endothelial damage sec-ondary to the maneuver Immediately following lens extraction, dilution of the aqueous humor would greatly reduce the validity ofthe test. Also, as indicated by Ryan et al (5), significant false-positive results are often reported in laboratories where the test is not frequently pertormed. Eig 3—Minimal posterior subluxation of the superior border of the left crystalline lens noted three months after initial presentation. Fig 4—Dislocation ofthe lens (left eye) into the anterior chamber Henry Ford Hosp Med J—Vol 34. No 3. 1986 Bilateral Dislocation—Rapkin & Bogorad 209 We have presented a case of bilateral dislocation of the crystalline lens in a patient with syphUitic uveitis. The two events occuned over a short period of time and in the absence of admitted trauma. The presence of uveitis in a patient with sup-portive historical findings should raise the clinician's suspicion of active syphilitic infection. References 1. Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982; 27:143-60. 2. Rosman HI. Chronic open-angle glaucoma associated with traumatic dis-location ofthe lens. Arch Ophthalmol 1963;69:445-54. 3. Rosenbaum LJ, Podos SM. Traumatic ectopia lentis. Am J Ophthalmol 1967;64:1095-8. 4. Smith JL, Singer JA, Moore MB, YobsAR. Seronegative ocular and neu-rosyphilis. Am J Ophthalmol 1965;59:753-65. 5. Ryan S J, Nell EE, Hardy PH. A study of aqueous humor for the presence of spirochetes. Am J Ophthalmol 1972;73:250-7. 6. Christman EH, Hamilton RW. Heaton CL, Hoffmeyer EM. Intraocular treponemes. Arch Ophthalmol 1968;80:303-7. 7. Golden B, Thompson HS. Implications of spiral forms in the eye. Surv Ophthalmol 1969;14:179-83. 8. Belin MW, Baltch AL, Hay PB. Secondary syphilitic uveitis. Am J Ophthalmol 1981;92:210-4. 9. DeLuise VF, Clark SW III , Smith JL, CoUart P Syphilitic retinal detach-ment and uveal effusion. Am J Ophthalmol 1982:94:757-61. 10. Kranias G, Schneider D, Raymond LA. A ease of syphilitic uveitis. Am J Ophthalmol 198I;9I;26I-3. 11. McCarton MJ, Albert DM. Iridocyclitis and an iris mass associated with secondary syphilis. Ophthalmology 1984;91:1264-8. 12. Ross WH, Sutton HFS. Acquired syphilitic uveitis. Arch Ophthalmol 1980;98:496-8. 13. Schlaegel TF Jr, Kao SFA review (1970-1980) of 28 presumptive cases of syphilitic uveitis. Am J Ophthalmol l982;93;412-4. 14. Schlaegel TF, O'Connor GR. Tuberculosis and syphilis. Arch Oph-thalmol 1981;99:2206-7. 15. Smith JL, Israel CW. Spirochetes in the aqueous humor of seronegative ocular syphiUs. Arch Ophthalmol 1967:77:474-7. 16. Goldman JN, Girard KF Fluorescent antibody staining of treponemes in uveitis. Arch Ophthalmol 1968;79:716-22. 210 Henry Ford Hosp Med J—Vol 34, No 3, 1986 Bilateral Dislocation—Rapkin & Bogorad 

Bilateral Dislocation of the Crystalline Lens in a Patient with Presumed Syphilitic Uveitis

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