- Email: [email protected]
Progressive outer retinal necrosis syndrome in a lymphoma patient with good visual outcome
T
HE INCIDENCE OF INFECTIOUS ENDOPHTHALMITIS AF-
ter penetrating trauma is a approximately 7%1 but may be as high as 30% after rural penetrating trauma.2 Posttraumatic endophthalmitis is associated with a poor visual prognosis (approximately 30% with visual acuity better than 20/400).1,3 The most common organisms associated with posttraumatic endophthalmitis are Staphylococcus epidermidis, Bacillus spp., gram-negative rods, and Clostridium perfringes. We are unaware of previous reports of endophthalmitis involving a combination of Bacillus spp and Clostridium tetani, and could find none in a computerized search using MEDLINE. A 25-year-old healthy man, employed as a bricklayer, sustained a traumatic rupture of his right globe after he was struck in the eye by a concrete reinforcement bar. Initial examination revealed an uncorrected visual acuity of RE: counting fingers at 12 inches and LE: 20/20. The right eye had a corneoscleral laceration extending 5 to 6 mm. There was a flat anterior chamber with prolapsed iris tissue, and a cataract was noted with no view of the fundus. No gross foreign bodies were seen. The left eye was within normal limits. The patient was given a tetanus toxoid booster at the emergency department, and the corneoscleral laceration was primarily repaired. The following day, the patient had no light perception in the right eye. Slit-lamp examination revealed purulent discharge, corneal infiltrates, a hypopyon, and a dense cataract. He underwent pars plana lensectomy and vitrectomy, with aspiration of vitreous fluid and a conjunctival swab for cultures. Intravitreal injections of ceftazidime 2 mg/0.1 ml and clindamycin 1 mg/0.1 ml and periocular injections of ceftazidime 50 mg/0.5 ml and clindamycin 150 mg/1 ml were given. The vitreous cavity was noted to be completely occupied by a thick fibrin purulent-type reaction with no discernible retinal detail. The patient was postoperatively placed on gentamicin 80 mg intravenously every 8 hours and ceftazidime 1000 mg intravenously every 8 hours, as well as fortified tobramycin and ceftazidime eye drops. Cultures from the conjunctival swab were negative for organisms. Gram stain of the vitreous aspirate revealed many gram-positive bacilli with moderate Bacillus spp. on culture. Anaerobic cultures of the vitreous aspirate were available 72 hours after vitrectomy and revealed many C. tetani. Following the recommendations of an infectious disease consultant, he was given a standard dose of tetanus immune globulin and switched to doxycycline 100 mg intravenously every 12 hours. By the third postoperative day when cultures became available, the patient developed severe eye pain associated with marked orbital congestion, increased swelling and erythema of the lids, marked injection and chemosis of the conjunctiva, and opacification of the cornea. Panophthalmitis was suspected, and after an informed consent was obtained, the patient underwent evisceraVOL. 132, NO. 1
tion. The intraocular contents consisted of mucopurulent material with clotted blood. The contents were culture negative. The inflammation resolved after the evisceration of the right eye, and he was discharged to home on doxycycline 100 mg orally two times daily for 10 days. The role of Bacillus spp. in endophthalmitis has been well documented.1 Clostridium tetani is a spore-forming gram-positive anaerobe that is ubiquitous in soil. Clostridium tetani produces two toxins: an oxygen-labile hemolysin (tetanolysin) and a plasmid-encoded neurotoxin (tetanospasmin). Tetanospasmin blocks inhibitory mediators in the central nervous system and causes the characteristic symptoms of tetanus, including violent muscle spasms, trismus, and exaggerated reflexes. The clinical significance of tetanolysin is unknown, because it is inhibited by oxygen and serum cholesterol.4 The finding of C. tetani intravitreally underscores the importance of determining tetanus immunization status and considering early intervention with tetanus toxoid booster (or diptheria, tetanus, pertussis for children under 7 years of age) and/or tetanus immune globulin in patients with penetrating eye injury and cultures positive for C. tetani. The current recommendations are tetanus toxoid booster if the patient has completed the primary series of three doses of tetanus toxoid but it has been more than 5 years since the last dose, or tetanus toxoid booster plus tetanus immune globulin if the patient has an unknown history of immunization or has not completed the primary series.5 REFERENCES
1. Kresloff MS, Castellarin AA, Zarbin MA. Endophthalmitis. Surv Ophthalmol 1998;43:193–224. 2. Boldt HC, Pulido JS, Blodi CF, Folk JC, Weingeist TA. Rural endophthalmitis. Ophthalmology 1989;96:1722–1726. 3. Thompson WS, Rubsamen PE, Flynn HW Jr, Schiffman J, Cousins SW. Endophthalmitis after penetrating trauma. Risk factors and visual acuity outcomes. Ophthalmology 1995;102: 1696 –1701. 4. Clostridium. Murray PR, Rosenthal KS, Kobayashi G, Pfaller MA, editors. Med Micro Biol, 3rd ed. St. Louis: Mosby-Year Book, 1998:299 –302. 5. Update on adult immunization. Recommendations of the immunization practices advisory committee (ACIP). Morbidity Mortal Wkly Rep 1991;40:1–52.
Progressive Outer Retinal Necrosis Syndrome in a Lymphoma Patient With Good Visual Outcome Robert E. Foster, MD, Michael R. Petersen, MD, PhD, Michael N. Neuss, MD, and Robert H. Osher, MD
BRIEF REPORTS
117
PURPOSE:
To report an HIV-negative lymphoma patient who developed progressive outer retinal necrosis syndrome and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. METHODS: Case report. RESULTS: A 43-year-old man with small lymphocytic lymphoma was diagnosed with progressive outer retinal necrosis in his left eye. Treatment was initiated with intravenous foscarnet and ganciclovir as well as intravenous gammaglobulin at a dose of 0.5 gm/kg per day for 5 days. On the second hospital day he was started on decadron 4 mg orally four times daily. No further posterior retinitis progression was observed despite severe immunosuppression. Visual acuity remained stable at 20/30 with 10 months’ follow-up. CONCLUSIONS: The benefit of using gammaglobulin in progressive outer retinal necrosis is unknown. Given the rapid improvement seen in this patient’s retinitis, it may be reasonable to consider the use of gammaglobulin in other cases of infectious retinitis in immunocompromised patients. (Am J Ophthalmol 2001;132:117–120. © 2001 by Elsevier Science Inc. All rights reserved.)
T
blurred vision in his left eye. Examination revealed a best-corrected visual acuity of RE: 20/20 and LE: 20/30. A left relative afferent pupillary defect was observed. Examination of the right eye was normal. Slit-lamp biomicroscopy of the left eye revealed mild anterior chamber cell and flare. Dilated fundus examination revealed mild vitritis and multifocal areas of outer retinal yellow–white opacification throughout the midperiphery in all quadrants (Figure 1). There was no evidence for retinal vasculitis or occlusion. Based on these findings, he was diagnosed with progressive outer retinal necrosis. He was immediately hospitalized and treated with intravenous foscarnet and ganciclovir as well as intravenous gammaglobulin at a dose of 0.5 gm/kg per day for 5 days. The chlorambucil maintenance chemotherapy was held. On the second hospital day he was started on decadron 4 mg orally four times daily. No further posterior retinitis progression was observed. The retinitis consolidated with the development of characteristic lucent zones highlighting retinal venules (Figure 2). Within 2 weeks, the retinitis began to resolve and extensive peripheral retinal pigment epithelial changes subsequently developed. There was never any progression of retinitis despite severe immunosuppression, including pretreatment for an autologous stem cell transplant. Gradually, in the left eye, a posterior subcapsular cataract developed that reduced visual acuity to 20/200 by 4 months later. He underwent uneventful phacoemulsification with placement of a posterior chamber intraocular lens in the capsular bag. By January of 1998, a subclinical rhegmatogenous retinal detachment was observed superotemporally, which was surrounded with laser. When last examined, visual acuity remained stable at 20/30. There was no active intraocular inflammation and no extension of the peripheral retinal detachment. In June 1998, lymphoma recurred and the patient died. Progressive outer retinal necrosis has been reported in other myeloproliferative diseases.2 Our case demonstrates that progressive outer retinal necrosis can also develop in a patient without HIV with chronic lymphocytic lymphoma. Importantly, lymphoma has been reported as a masquerading condition of progressive outer retinal necrosis. Topilow and associates3 described a 73-year-old patient with no previous lymphoma history who had slowly progressive retinal opacification over a 6-month period despite antiviral therapy and was eventually diagnosed with lymphoma. In contrast, our case involved a younger person with a known history of lymphoma who developed rapidly progressive retinal opacification, which quickly resolved with a combination of two antiviral drugs and intravenous gammaglobulin. Combined (two-drug) antiviral therapy has been reported to be more effective in preserving vision than monotherapy in progressive outer retinal necrosis.4 The benefit of using gammaglobulin in progressive outer retinal necrosis is unknown. However, gammaglobulin is used to promote humoral immunity in patients with chronic lym-
HE PROGRESSIVE OUTER RETINAL NECROSIS SYNDROME
is a rapidly progressive infectious retinitis associated with varicella zoster virus. Typically, the retinitis starts as a multifocal outer retinal inflammation that rapidly becomes more confluent. As the retinitis consolidates, a characteristic “venular lucency” can be observed within areas of opaque retina.1 Visual acuity outcomes have been poor in most reported cases. Although progressive outer retinal necrosis was initially described in HIV-infected patients, recent reports have described progressive outer retinal necrosis in HIV-negative patients as well.2 We report a patient with HIV-negative lymphoma who developed progressive outer retinal necrosis and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. At the age of 43, a man was diagnosed as having small lymphocytic lymphoma by lymph node biopsy in May 1993. He was treated with a variety of chemotherapy agents, including mitoxantrone and fludarabine, intermittent chlorambucil and prednisone, cladribine as a single agent, and high-dose pulse corticosteroids. In 1997, chemotherapy consisted only of corticosteroids and alkylating agent therapy, including chlorambucil and cyclophosphamide. In July, perianal herpes zoster was treated with famciclovir and steroids. In August of 1997, he was evaluated for progressive Accepted for publication Dec 5, 2000. From the Cincinnati Eye Institute (R.E.F., M.R.P., R.H.O), Cincinnati, Ohio, and the Oncology-Hematology Care/University Internal Medicine Associates (M.N.N.), Cincinnati, Ohio. Inquires to Robert E. Foster, MD, Cincinnati Eye Institute, 10494 Montgomery Rd, Cincinnati, OH 45242; e-mail: [email protected]
118
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OPHTHALMOLOGY
JULY 2001
FIGURE 1. (Left) Wide-angle fundus photograph showing the multifocal outer retinitis temporal to the macula in the left eye. (Right) Inferiorly, more consolidated retinitis was observed. Note the perivascular hemorrhages inferiorly.
FIGURE 2. Two weeks later, wide-angle fundus photograph of inferior retina in the left eye showing same area as in Figure 1, right image. Note the partial resolution of the retinitis and the perivenular “lucency” or clearing, a characteristic finding in progressive outer retinal necrosis.
phoproliferative disease and other hypoglobulinemic states.5 Its utility in viral disease, specifically cytomegalovirus pulmonary disease in severely immunocompromised patients who have had stem cell transplants, is also established.6 Given the rapid improvement seen in this patient’s retinitis, it may be reasonable to consider the use of gammaglobulin in other cases of infectious retinitis in immunocompromised patients.
2.
3. 4.
REFERENCES
5.
1. Engstrom RE, Holland GA, Margolis TP, et al. The progressive outer retinal necrosis syndrome: a variant of necrotizing
6.
VOL. 132, NO. 1
herpetic retinopathy in patients with AIDS. Ophthalmology 1994;101:1488 –1502. Lewis JM, Nagae Y, Tano Y. Progressive outer retinal necrosis after bone marrow transplantation. Am J Ophthalmol 1996; 122:892– 895. Topilow HW, Ackerman AL, Friedman A. Progressive outer retinal necrosis [letter]. Ophthalmology 1995;102:1737–1738. Morley MG, Duker J, Zacks C. Successful treatment of rapidly progressive outer retinal necrosis in the acquired immunodeficiency syndrome. Am J Ophthalmol 1994;117:264 –265. Rozman C and Montserrat E. Current concepts: chronic lymphocytic leukemia. N Engl J Med 1995;333:1052–1057. Messori A, Rampazzo R, Scroccaro G, Martini N. Efficacy of hyperimmune anti-cytomegalovirus immunoglobulins for the
BRIEF REPORTS
119
● CASE 1:
prevention of cytomegalovirus infection in recipients of allogeneic bone marrow transplantation: a meta analysis. Bone Marrow Transplant 1994;13:163–167.
A 25-year-old woman was referred approximately 2 months after a scleral buckling procedure using a cryopexy and vitrectomy in the right eye done elsewhere. She had the procedure for an inferior detachment with a preoperative visual acuity of 20/20. After her operative procedure, her acuity was 20/400. In the right eye, there were multiple, irregularly rounded, well-circumscribed, yellow– orange, bleb-like subretinal lesions in the posterior pole (Figure 1, top). One month later, she developed some subretinal precipitates in the bleb-like lesions. Fluorescein angiography demonstrated the lesions to be slightly hyperfluorescent with no leakage or staining. Indocyanine green angiography did not show any leakage of either the retinal or choroidal vessels. Optical coherence tomography demonstrated that the lesions were small retinal detachments (Figure 1, bottom). During the first 4 months, the bleb-like lesions changed in their grouping slightly, but the fovea remained involved. By 6 months after her surgery, most of the bleb-like lesions resorbed although the fovea remained involved. Her visual acuity improved to 20/60.
Optical Coherence Tomography of Bleb-like Subretinal Lesions After Retinal Reattachment Surgery Tatsushi Kaga, MD, Roberto A. Fonseca, MD, Marcos A. Dantas, MD, Lawrence A. Yannuzzi, MD, and Richard F. Spaide, MD PURPOSE:
To report the optical coherence tomographic characteristics of persistent bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy. METHODS: Case reports. RESULTS: In two eyes of two patients yellow to yellowishorange bleb-like lesions were found in the posterior pole after retinal reattachment surgery. The common aspects of the surgery were scleral buckling procedures and cryopexy. There were subretinal precipitates in some of the lesions. The lesions showed no associated leakage during fluorescein or indocyanine green angiography. Optical coherence tomography demonstrated that the bleb-like lesions were tiny retinal detachments. CONCLUSION: Optical coherence tomography examination of bleb-like subretinal lesions showed that they were actually retinal detachments. Our patients and previously reported patients developed these lesions after scleral buckling and cryopexy. These lesions may represent persistent retinal detachment because of the presence of protein in the subretinal fluid. (Am J Ophthalmol 2001;132:120 –121. © 2001 by Elsevier Science Inc. All rights reserved.)
B
● CASE 2: A 56-year-old woman was referred after a scleral buckling procedure with cryopexy, left eye, after her visual acuity failed to improve completely. Her left visual acuity was 20/50. There was a round, well-demarcated, slightly yellow, bleb-like subretinal lesion beneath the fovea in her left eye. There were some subretinal precipitates in the lesion (Figure 2, top). Fluorescein angiography demonstrated the lesions to be slightly hyperfluorescent, but there was no leakage or staining. Optical coherence tomography demonstrated that the lesion was a retinal detachment (Figure 2, bottom). Her findings remained stable for 3 months. We found small bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy, in two patients, and these lesions looked like previously reported lesions.1–3 We showed that the lesions were actually small retinal detachments using optical coherence tomography. Machemer,4 who examined experimental retinal detachments in owl monkeys, thought that similarly appearing lesions might have been retinal detachments, but his fluorescein images showed early intense hyperfluorescence. In both our cases and in all other cases in humans, the lesions were demonstrated to be only minimally hyperfluorescent with no leakage or staining. The bleb-like subretinal lesions in our cases and those previously reported have occurred temporal to the optic nerve, with a predilection for the posterior pole. The lesions occurred in patients treated with scleral buckling and cryopexy for rhegmatogenous retinal detachments. Our first case also had a concurrent vitrectomy. We found that the lesions represented small retinal detachments. It is not known, however, why the detachments persist. We found that there was no leakage during fluorescein or
LEB-LIKE SUBRETINAL LESIONS AFTER SCLERAL BUCK-
ling procedures were reported as lesions simulating pigment epithelial detachments.1–3 Although the lesions were well circumscribed, it is thought that they represent persistent, isolated pockets of subretinal fluid, because they showed no increasing hyperfluorescence throughout the fluorescein angiography. Using optical coherence tomography, we found that the lesions appeared to be small retinal detachments.
Accepted for publication Dec 6, 2000. From the LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York, and the VitreousRetina-Macula Consultants of New York, New York, New York. Inquiries to Richard F. Spaide, MD, 519 East 72nd St, Suite 203, New York, New York 10021; fax number: (212) 628-0698; e-mail: VRMNY@ aol.com
120
AMERICAN JOURNAL
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OPHTHALMOLOGY
JULY 2001
HE INCIDENCE OF INFECTIOUS ENDOPHTHALMITIS AF-
ter penetrating trauma is a approximately 7%1 but may be as high as 30% after rural penetrating trauma.2 Posttraumatic endophthalmitis is associated with a poor visual prognosis (approximately 30% with visual acuity better than 20/400).1,3 The most common organisms associated with posttraumatic endophthalmitis are Staphylococcus epidermidis, Bacillus spp., gram-negative rods, and Clostridium perfringes. We are unaware of previous reports of endophthalmitis involving a combination of Bacillus spp and Clostridium tetani, and could find none in a computerized search using MEDLINE. A 25-year-old healthy man, employed as a bricklayer, sustained a traumatic rupture of his right globe after he was struck in the eye by a concrete reinforcement bar. Initial examination revealed an uncorrected visual acuity of RE: counting fingers at 12 inches and LE: 20/20. The right eye had a corneoscleral laceration extending 5 to 6 mm. There was a flat anterior chamber with prolapsed iris tissue, and a cataract was noted with no view of the fundus. No gross foreign bodies were seen. The left eye was within normal limits. The patient was given a tetanus toxoid booster at the emergency department, and the corneoscleral laceration was primarily repaired. The following day, the patient had no light perception in the right eye. Slit-lamp examination revealed purulent discharge, corneal infiltrates, a hypopyon, and a dense cataract. He underwent pars plana lensectomy and vitrectomy, with aspiration of vitreous fluid and a conjunctival swab for cultures. Intravitreal injections of ceftazidime 2 mg/0.1 ml and clindamycin 1 mg/0.1 ml and periocular injections of ceftazidime 50 mg/0.5 ml and clindamycin 150 mg/1 ml were given. The vitreous cavity was noted to be completely occupied by a thick fibrin purulent-type reaction with no discernible retinal detail. The patient was postoperatively placed on gentamicin 80 mg intravenously every 8 hours and ceftazidime 1000 mg intravenously every 8 hours, as well as fortified tobramycin and ceftazidime eye drops. Cultures from the conjunctival swab were negative for organisms. Gram stain of the vitreous aspirate revealed many gram-positive bacilli with moderate Bacillus spp. on culture. Anaerobic cultures of the vitreous aspirate were available 72 hours after vitrectomy and revealed many C. tetani. Following the recommendations of an infectious disease consultant, he was given a standard dose of tetanus immune globulin and switched to doxycycline 100 mg intravenously every 12 hours. By the third postoperative day when cultures became available, the patient developed severe eye pain associated with marked orbital congestion, increased swelling and erythema of the lids, marked injection and chemosis of the conjunctiva, and opacification of the cornea. Panophthalmitis was suspected, and after an informed consent was obtained, the patient underwent evisceraVOL. 132, NO. 1
tion. The intraocular contents consisted of mucopurulent material with clotted blood. The contents were culture negative. The inflammation resolved after the evisceration of the right eye, and he was discharged to home on doxycycline 100 mg orally two times daily for 10 days. The role of Bacillus spp. in endophthalmitis has been well documented.1 Clostridium tetani is a spore-forming gram-positive anaerobe that is ubiquitous in soil. Clostridium tetani produces two toxins: an oxygen-labile hemolysin (tetanolysin) and a plasmid-encoded neurotoxin (tetanospasmin). Tetanospasmin blocks inhibitory mediators in the central nervous system and causes the characteristic symptoms of tetanus, including violent muscle spasms, trismus, and exaggerated reflexes. The clinical significance of tetanolysin is unknown, because it is inhibited by oxygen and serum cholesterol.4 The finding of C. tetani intravitreally underscores the importance of determining tetanus immunization status and considering early intervention with tetanus toxoid booster (or diptheria, tetanus, pertussis for children under 7 years of age) and/or tetanus immune globulin in patients with penetrating eye injury and cultures positive for C. tetani. The current recommendations are tetanus toxoid booster if the patient has completed the primary series of three doses of tetanus toxoid but it has been more than 5 years since the last dose, or tetanus toxoid booster plus tetanus immune globulin if the patient has an unknown history of immunization or has not completed the primary series.5 REFERENCES
1. Kresloff MS, Castellarin AA, Zarbin MA. Endophthalmitis. Surv Ophthalmol 1998;43:193–224. 2. Boldt HC, Pulido JS, Blodi CF, Folk JC, Weingeist TA. Rural endophthalmitis. Ophthalmology 1989;96:1722–1726. 3. Thompson WS, Rubsamen PE, Flynn HW Jr, Schiffman J, Cousins SW. Endophthalmitis after penetrating trauma. Risk factors and visual acuity outcomes. Ophthalmology 1995;102: 1696 –1701. 4. Clostridium. Murray PR, Rosenthal KS, Kobayashi G, Pfaller MA, editors. Med Micro Biol, 3rd ed. St. Louis: Mosby-Year Book, 1998:299 –302. 5. Update on adult immunization. Recommendations of the immunization practices advisory committee (ACIP). Morbidity Mortal Wkly Rep 1991;40:1–52.
Progressive Outer Retinal Necrosis Syndrome in a Lymphoma Patient With Good Visual Outcome Robert E. Foster, MD, Michael R. Petersen, MD, PhD, Michael N. Neuss, MD, and Robert H. Osher, MD
BRIEF REPORTS
117
PURPOSE:
To report an HIV-negative lymphoma patient who developed progressive outer retinal necrosis syndrome and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. METHODS: Case report. RESULTS: A 43-year-old man with small lymphocytic lymphoma was diagnosed with progressive outer retinal necrosis in his left eye. Treatment was initiated with intravenous foscarnet and ganciclovir as well as intravenous gammaglobulin at a dose of 0.5 gm/kg per day for 5 days. On the second hospital day he was started on decadron 4 mg orally four times daily. No further posterior retinitis progression was observed despite severe immunosuppression. Visual acuity remained stable at 20/30 with 10 months’ follow-up. CONCLUSIONS: The benefit of using gammaglobulin in progressive outer retinal necrosis is unknown. Given the rapid improvement seen in this patient’s retinitis, it may be reasonable to consider the use of gammaglobulin in other cases of infectious retinitis in immunocompromised patients. (Am J Ophthalmol 2001;132:117–120. © 2001 by Elsevier Science Inc. All rights reserved.)
T
blurred vision in his left eye. Examination revealed a best-corrected visual acuity of RE: 20/20 and LE: 20/30. A left relative afferent pupillary defect was observed. Examination of the right eye was normal. Slit-lamp biomicroscopy of the left eye revealed mild anterior chamber cell and flare. Dilated fundus examination revealed mild vitritis and multifocal areas of outer retinal yellow–white opacification throughout the midperiphery in all quadrants (Figure 1). There was no evidence for retinal vasculitis or occlusion. Based on these findings, he was diagnosed with progressive outer retinal necrosis. He was immediately hospitalized and treated with intravenous foscarnet and ganciclovir as well as intravenous gammaglobulin at a dose of 0.5 gm/kg per day for 5 days. The chlorambucil maintenance chemotherapy was held. On the second hospital day he was started on decadron 4 mg orally four times daily. No further posterior retinitis progression was observed. The retinitis consolidated with the development of characteristic lucent zones highlighting retinal venules (Figure 2). Within 2 weeks, the retinitis began to resolve and extensive peripheral retinal pigment epithelial changes subsequently developed. There was never any progression of retinitis despite severe immunosuppression, including pretreatment for an autologous stem cell transplant. Gradually, in the left eye, a posterior subcapsular cataract developed that reduced visual acuity to 20/200 by 4 months later. He underwent uneventful phacoemulsification with placement of a posterior chamber intraocular lens in the capsular bag. By January of 1998, a subclinical rhegmatogenous retinal detachment was observed superotemporally, which was surrounded with laser. When last examined, visual acuity remained stable at 20/30. There was no active intraocular inflammation and no extension of the peripheral retinal detachment. In June 1998, lymphoma recurred and the patient died. Progressive outer retinal necrosis has been reported in other myeloproliferative diseases.2 Our case demonstrates that progressive outer retinal necrosis can also develop in a patient without HIV with chronic lymphocytic lymphoma. Importantly, lymphoma has been reported as a masquerading condition of progressive outer retinal necrosis. Topilow and associates3 described a 73-year-old patient with no previous lymphoma history who had slowly progressive retinal opacification over a 6-month period despite antiviral therapy and was eventually diagnosed with lymphoma. In contrast, our case involved a younger person with a known history of lymphoma who developed rapidly progressive retinal opacification, which quickly resolved with a combination of two antiviral drugs and intravenous gammaglobulin. Combined (two-drug) antiviral therapy has been reported to be more effective in preserving vision than monotherapy in progressive outer retinal necrosis.4 The benefit of using gammaglobulin in progressive outer retinal necrosis is unknown. However, gammaglobulin is used to promote humoral immunity in patients with chronic lym-
HE PROGRESSIVE OUTER RETINAL NECROSIS SYNDROME
is a rapidly progressive infectious retinitis associated with varicella zoster virus. Typically, the retinitis starts as a multifocal outer retinal inflammation that rapidly becomes more confluent. As the retinitis consolidates, a characteristic “venular lucency” can be observed within areas of opaque retina.1 Visual acuity outcomes have been poor in most reported cases. Although progressive outer retinal necrosis was initially described in HIV-infected patients, recent reports have described progressive outer retinal necrosis in HIV-negative patients as well.2 We report a patient with HIV-negative lymphoma who developed progressive outer retinal necrosis and who had a good visual outcome after treatment with two-drug antiviral therapy and intravenous immunoglobulin. At the age of 43, a man was diagnosed as having small lymphocytic lymphoma by lymph node biopsy in May 1993. He was treated with a variety of chemotherapy agents, including mitoxantrone and fludarabine, intermittent chlorambucil and prednisone, cladribine as a single agent, and high-dose pulse corticosteroids. In 1997, chemotherapy consisted only of corticosteroids and alkylating agent therapy, including chlorambucil and cyclophosphamide. In July, perianal herpes zoster was treated with famciclovir and steroids. In August of 1997, he was evaluated for progressive Accepted for publication Dec 5, 2000. From the Cincinnati Eye Institute (R.E.F., M.R.P., R.H.O), Cincinnati, Ohio, and the Oncology-Hematology Care/University Internal Medicine Associates (M.N.N.), Cincinnati, Ohio. Inquires to Robert E. Foster, MD, Cincinnati Eye Institute, 10494 Montgomery Rd, Cincinnati, OH 45242; e-mail: [email protected]
118
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OPHTHALMOLOGY
JULY 2001
FIGURE 1. (Left) Wide-angle fundus photograph showing the multifocal outer retinitis temporal to the macula in the left eye. (Right) Inferiorly, more consolidated retinitis was observed. Note the perivascular hemorrhages inferiorly.
FIGURE 2. Two weeks later, wide-angle fundus photograph of inferior retina in the left eye showing same area as in Figure 1, right image. Note the partial resolution of the retinitis and the perivenular “lucency” or clearing, a characteristic finding in progressive outer retinal necrosis.
phoproliferative disease and other hypoglobulinemic states.5 Its utility in viral disease, specifically cytomegalovirus pulmonary disease in severely immunocompromised patients who have had stem cell transplants, is also established.6 Given the rapid improvement seen in this patient’s retinitis, it may be reasonable to consider the use of gammaglobulin in other cases of infectious retinitis in immunocompromised patients.
2.
3. 4.
REFERENCES
5.
1. Engstrom RE, Holland GA, Margolis TP, et al. The progressive outer retinal necrosis syndrome: a variant of necrotizing
6.
VOL. 132, NO. 1
herpetic retinopathy in patients with AIDS. Ophthalmology 1994;101:1488 –1502. Lewis JM, Nagae Y, Tano Y. Progressive outer retinal necrosis after bone marrow transplantation. Am J Ophthalmol 1996; 122:892– 895. Topilow HW, Ackerman AL, Friedman A. Progressive outer retinal necrosis [letter]. Ophthalmology 1995;102:1737–1738. Morley MG, Duker J, Zacks C. Successful treatment of rapidly progressive outer retinal necrosis in the acquired immunodeficiency syndrome. Am J Ophthalmol 1994;117:264 –265. Rozman C and Montserrat E. Current concepts: chronic lymphocytic leukemia. N Engl J Med 1995;333:1052–1057. Messori A, Rampazzo R, Scroccaro G, Martini N. Efficacy of hyperimmune anti-cytomegalovirus immunoglobulins for the
BRIEF REPORTS
119
● CASE 1:
prevention of cytomegalovirus infection in recipients of allogeneic bone marrow transplantation: a meta analysis. Bone Marrow Transplant 1994;13:163–167.
A 25-year-old woman was referred approximately 2 months after a scleral buckling procedure using a cryopexy and vitrectomy in the right eye done elsewhere. She had the procedure for an inferior detachment with a preoperative visual acuity of 20/20. After her operative procedure, her acuity was 20/400. In the right eye, there were multiple, irregularly rounded, well-circumscribed, yellow– orange, bleb-like subretinal lesions in the posterior pole (Figure 1, top). One month later, she developed some subretinal precipitates in the bleb-like lesions. Fluorescein angiography demonstrated the lesions to be slightly hyperfluorescent with no leakage or staining. Indocyanine green angiography did not show any leakage of either the retinal or choroidal vessels. Optical coherence tomography demonstrated that the lesions were small retinal detachments (Figure 1, bottom). During the first 4 months, the bleb-like lesions changed in their grouping slightly, but the fovea remained involved. By 6 months after her surgery, most of the bleb-like lesions resorbed although the fovea remained involved. Her visual acuity improved to 20/60.
Optical Coherence Tomography of Bleb-like Subretinal Lesions After Retinal Reattachment Surgery Tatsushi Kaga, MD, Roberto A. Fonseca, MD, Marcos A. Dantas, MD, Lawrence A. Yannuzzi, MD, and Richard F. Spaide, MD PURPOSE:
To report the optical coherence tomographic characteristics of persistent bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy. METHODS: Case reports. RESULTS: In two eyes of two patients yellow to yellowishorange bleb-like lesions were found in the posterior pole after retinal reattachment surgery. The common aspects of the surgery were scleral buckling procedures and cryopexy. There were subretinal precipitates in some of the lesions. The lesions showed no associated leakage during fluorescein or indocyanine green angiography. Optical coherence tomography demonstrated that the bleb-like lesions were tiny retinal detachments. CONCLUSION: Optical coherence tomography examination of bleb-like subretinal lesions showed that they were actually retinal detachments. Our patients and previously reported patients developed these lesions after scleral buckling and cryopexy. These lesions may represent persistent retinal detachment because of the presence of protein in the subretinal fluid. (Am J Ophthalmol 2001;132:120 –121. © 2001 by Elsevier Science Inc. All rights reserved.)
B
● CASE 2: A 56-year-old woman was referred after a scleral buckling procedure with cryopexy, left eye, after her visual acuity failed to improve completely. Her left visual acuity was 20/50. There was a round, well-demarcated, slightly yellow, bleb-like subretinal lesion beneath the fovea in her left eye. There were some subretinal precipitates in the lesion (Figure 2, top). Fluorescein angiography demonstrated the lesions to be slightly hyperfluorescent, but there was no leakage or staining. Optical coherence tomography demonstrated that the lesion was a retinal detachment (Figure 2, bottom). Her findings remained stable for 3 months. We found small bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy, in two patients, and these lesions looked like previously reported lesions.1–3 We showed that the lesions were actually small retinal detachments using optical coherence tomography. Machemer,4 who examined experimental retinal detachments in owl monkeys, thought that similarly appearing lesions might have been retinal detachments, but his fluorescein images showed early intense hyperfluorescence. In both our cases and in all other cases in humans, the lesions were demonstrated to be only minimally hyperfluorescent with no leakage or staining. The bleb-like subretinal lesions in our cases and those previously reported have occurred temporal to the optic nerve, with a predilection for the posterior pole. The lesions occurred in patients treated with scleral buckling and cryopexy for rhegmatogenous retinal detachments. Our first case also had a concurrent vitrectomy. We found that the lesions represented small retinal detachments. It is not known, however, why the detachments persist. We found that there was no leakage during fluorescein or
LEB-LIKE SUBRETINAL LESIONS AFTER SCLERAL BUCK-
ling procedures were reported as lesions simulating pigment epithelial detachments.1–3 Although the lesions were well circumscribed, it is thought that they represent persistent, isolated pockets of subretinal fluid, because they showed no increasing hyperfluorescence throughout the fluorescein angiography. Using optical coherence tomography, we found that the lesions appeared to be small retinal detachments.
Accepted for publication Dec 6, 2000. From the LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York, and the VitreousRetina-Macula Consultants of New York, New York, New York. Inquiries to Richard F. Spaide, MD, 519 East 72nd St, Suite 203, New York, New York 10021; fax number: (212) 628-0698; e-mail: VRMNY@ aol.com
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AMERICAN JOURNAL
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