Primary localized conjunctival amyloidosis presenting with recurrence of subconjunctival hemorrhage

Primary localized conjunctival amyloidosis presenting with recurrence of subconjunctival hemorrhage

tube insertion is an alternative technique for adult patients, especially those known (or potential) keloid-former cases, or those preferring not to u...

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tube insertion is an alternative technique for adult patients, especially those known (or potential) keloid-former cases, or those preferring not to undergo laser treatment and wanting to avoid an external facial scar. (Am J Ophthalmol 2000;129:244 –245. © 2000 by Elsevier Science Inc. All rights reserved.)

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HE

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PERFORMING

CONJUNCTIVO-

dacryocystorhinostomy (CDCR), a skin approach, has remained essentially unchanged.1–3 We present a conjunctival approach to CDCR with Jones tube insertion, thereby avoiding external skin scarring. The technique was performed on 13 adult patients; all candidates had complete lacrimal obstruction at the common canaliculus and excessive tearing. All patients were known (or potential) keloid-formers, and suitable candidates for a CDCR with Jones tube insertion. Also, all 13 patients wanted to avoid an external facial scar. One patient refused laser treatment to open severe bicanalicular scarring, although she was amenable to CDCR. Exclusion criteria included patients with facial trauma, history of ocular or sinus malignancy, conjunctival disease, and children. The nose is packed with vasoconstrictive–analgesic medications. Lidocaine 2% with epinephrine is injected subcutaneously in the medial canthus, and subconjunctivally from around the caruncle to the midportion of the lower fornix. Malleable retractors and rakes can aid exposure. The lower eyelid is retracted and incised from the caruncle to the inferomedial fornix. The caruncle is excised. The soft tissue incision is made posterior to the lacrimal sac. The periosteum is incised and reflected (Figure 1, left). The Freer periosteal elevator is used to make an opening in the thin bone posterior to the lacrimal fossa. A Kerrison punch is employed to enlarge the osteotomy to about 10 ⫻ 12 mm. The lacrimal sac is not incised nor anastomosed. Because of the relatively restricted size of the surgical field, maneuvers are somewhat limited. A probe is used to estimate the proper length of the tube needed and aids in the insertion. The tube is positioned in the inferomedial fornix and anchored to the eyelid margin with a 6 – 0 nylon suture (Figure 1, right). The periosteal and muscle layers are closed with 5– 0 vicryl, and the conjunctiva is closed with 6 – 0 chromic suture. Operative time averages 30 minutes (range, 25– 40 minutes). This technique was applied throughout an 8-year period (between 1990 and 1998) on nine females and four males, aged 33 to 45 years. All patients except one female had unilateral procedures. General anesthesia was used in four of 13 patients, and local anesthesia with intravenous sedation was used in nine of 13 patients. Postoperatively, one patient underwent a tube exchange; another complained about occasional tearing; otherwise, all 13 patients had surgically successful outcomes without external scarring. Follow-up ranged from 9 months to 7 years, median, 4.5 VOL. 129, NO. 2

years. All tubes were in proper position and functioning at last follow-up. Although no further complications have occurred to date, possibilities include globe, eyelid, or medial canthal tendon injury. The literature is scant regarding surgical approaches for CDCR.1–3 In such cases as canalicular scarring, in which laser may be used, or in situations in which endoscopic techniques may be employed, the associated lower success rates and higher costs are practical considerations. Moreover, those techniques tend to be more time-consuming and equipment may not always be available. One main advantage in the studied conjunctival incision approach to CDCR is the yield of excellent results without external scarring. The studied technique offers an alternative approach to primary CDCR, although, in experienced hands, successful results can be obtained regardless of which technique is used. REFERENCES

1. Jones LT. Conjunctivodacryocystorhinostomy. Am J Ophthalmol 1965;59:773–783. 2. Jones LT, Wobig JL. Surgery of the eyelids and lacrimal system. Birmingham: Aesculapius, 1976:194 –233. 3. Steinsapir KD, Glatt HJ, Putterman AM. A 16-year study of conjunctival dacryocystorhinostomy. Am J Ophthalmol 1990; 109:387–393.

Primary Localized Conjunctival Amyloidosis Presenting With Recurrence of Subconjunctival Hemorrhage Hung-Ming Lee, MBBS, FRCS(Edin), Joel Naor, MD, Dan DeAngelis, MD, FRCSC, and David S. Rootman, MD, FRCSC PURPOSE:

To report the ocular presentation and histopathology of a patient with primary localized conjunctival amyloidosis. METHODS: A 38-year-old woman presented with a recurrence of episodes of severe bilateral subconjunctival hemorrhage. Ocular examination revealed yellowish, marked folding and redundancy of the conjunctiva in the inferior cul-de-sac of each eye. RESULTS: After two initial conjunctival biopsies that showed only chronic inflammation, a third biopsy revealed the presence of amyloid in the substantia propria of the conjunctiva.

Accepted for publication Sept 1, 1999. From the Toronto Western Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada. Inquiries to David S. Rootman, MD, FRCSC, Toronto Hospital, Western Division, 399 Bathurst St, EC7-009, Toronto, Ontario, M5T 2S8 Canada; fax: (416) 603-1993; e-mail: [email protected]

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CONCLUSION:

Primary localized conjunctival amyloidosis is rare and usually diagnosed histologically instead of clinically. Recurrence of subconjunctival hemorrhage may be the initial presentation. Evaluation for systemic diseases is advised, though the results of the examination are almost always negative. (Am J Ophthalmol 2000; 129:245–247. © 2000 by Elsevier Science Inc. All rights reserved.)

A

MYLOIDOSIS IS A GROUP OF DISORDERS OF DIVERSE

origin and pathogenesis defined by deposition of a particular group of eosinophilic hyaline protein in tissue. A useful classification of amyloidosis considers four types: (1) primary localized amyloidosis, (2) primary systemic amyloidosis, (3) secondary localized amyloidosis, and (4) secondary systemic amyloidosis.1 Ocular amyloidosis is also classified in the same manner. Ocular amyloid made local to the conjunctiva is relatively rare and usually not considered to be a common differential diagnosis of isolated eyelid-conjunctival tumor. Most conjunctival amyloid lesions are located in the palpebral-conjunctival or fornix-conjunctival regions.2 We describe a rare case of bilateral conjunctival amyloidosis that presented with a recurrence of episodes of severe subconjunctival hemorrhage. A 38-year-old woman presented with her first episode of left subconjunctival hemorrhage in 1992 after the delivery of her second child. Since then, she had recurrent episodes of left subconjunctival hemorrhage (Figure 1), almost on a monthly basis; soon, the right eye was involved as well. No ocular pain or visual deterioration occurred. Also, no progression of antecedent inflammatory or degenerative disease of the conjunctiva occurred. Gradually, she started to have dryness and itchiness in both eyes. Visual acuity was 20/25 in each eye. Ocular examination revealed follicular conjunctivitis associated with yellowish, marked folding and redundancy of the conjunctiva in the inferior cul-de-sac of each eye (Figure 2, top). There was also a thickened yellowish tissue in the upper tarsal conjunctiva of each eye that prolapsed on eyelid eversion. Her corneas were clear, and ocular examination revealed no other noteworthy changes. Two conjunctival biopsies performed on separate occasions showed chronic inflammatory cell infiltrates and subconjunctival fibrosis. A third conjunctival biopsy finally revealed homogenous, eosinophilic, and hyaline material in the connective tissue (Figure 2, bottom), which showed the following staining characteristics: metachromasia with crystal violet dye, positive Congo-red birefringence, and dichroism with Congo red under polarized light. There were also mild chronic inflammatory cells with a predominance of plasma cells in the infiltrate. Electron microscopic analysis of the deposit revealed a fibrillary structure consistent with amyloid. The patient was managed conservatively with tear lubricants, and over 246

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FIGURE 1. Left eye, severe subconjunctival hemorrhage.

FIGURE 2. (Top) Yellowish marked folding and redundancy of conjunctiva in the inferior cul-de-sac. (Bottom) Conjunctival stroma with homogenous, eosinophilic, and hyaline material in the hematoxylin and eosin stain section, ⴛ75 before 55% reduction.

the year, her conjunctival amyloid had remained stable without much progression, although she still had recurrent episodes of subconjunctival hemorrhage. She was also examined extensively for systemic diseases, which were all negative. OF

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The diagnosis of localized amyloidosis is based on the demonstration of amyloid material in tissue in the absence of clinical evidence of systemic amyloid diseases. Though amyloidosis may be suspected on clinical grounds, the diagnosis is usually made histologically instead of clinically. Conjunctival amyloidosis often presents as a painless, nodular thickening or swelling of the eyelid/conjunctiva or blepharoptosis.2 To our knowledge, this is the first case of conjunctival amyloid that presented initially with recurrent subconjunctival hemorrhage. This is probably correlated with amyloid in the walls of the blood vessels, because purpura is also a known common manifestation of primary systemic amyloidosis. The mere recognition of amyloid in an ocular structure should not be considered a complete diagnosis. The possibility of amyloidosis in extraocular sites associated with underlying diseases or immunoglobulin abnormalities must be considered.3 Evaluation for systemic diseases is advised for such patients, though the results of the investigations are almost always negative.4 REFERENCES

1. Brownstein MD, Elliott R, Helwig EB. Ophthalmologic aspects of amyloidosis. Am J Ophthalmol 1970;69:423– 430. 2. Smith MH, Zimmermann LE. Amyloidosis of the eyelid and conjunctiva. Arch Ophthalmol 1975;75:42–50. 3. Blodi FC, Apple DJ. Localized conjunctival amyloidosis. Am J Ophthalmol 1979;88:346 –350. 4. Sugar A. Corneal and conjunctival degenerations. In: Kaufman HE, Barron BA, McDonald MB, editors. The cornea. New York: Butterworth-Heinemann, 1998:477– 496.

Localized Infection by Serratia marcescens Simulating a Conjunctival Neoplasm Jerry A. Shields, MD, Carol L. Shields, MD, Ralph C. Eagle, Jr, MD, and Marlene Moster, MD PURPOSE: To report a Serratia marcescens infection that clinically simulated a conjunctival neoplasm. METHOD: Case report. RESULTS: A healthy 80-year-old man without contact lenses presented with a pink–yellow conjunctival mass that resembled a solid neoplasm. Stains and cultures of Accepted for publication Sept 1, 1999. From the Oncology Service (J.A.S., C.L.S.), the Department of Pathology (R.C.E.), and the Glaucoma Service (M.M.), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. This work was supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (J.A.S., C.L.S.), by the Award of Merit in Retina Research, Houston, Texas (J.A.S.), the Macula Foundation, New York, New York (C.L.S.), and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital (R.C.E.). Inquiries to Jerry A. Shields, MD, Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.

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material that exuded from the mass during surgery revealed S marcescens. Histopathology disclosed an epithelial-lined cyst with macrophages containing S marcescens. CONCLUSION: Although S marcescens usually affects the eye as a keratoconjunctivitis in patients with contact lenses, it can also present as a mass simulating a neoplasm in a patient who does not wear contact lenses. (Am J Ophthalmol 2000;129:247–248. © 2000 by Elsevier Science Inc. All rights reserved.)

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ERRATIA MARCESCENS IS AN OPPORTUNISTIC GRAM-

negative bacillus once considered to be nonpathogenic in humans. Human infections with this organism were first recognized in neonates, in whom it produced meningitis, pneumonia, urinary infections, and conjunctivitis.1,2 Rarely, S marcescens has caused endophthalmitis.3 It is now best known to cause keratoconjunctivitis, particularly in patients who wear soft contact lenses,4,5 and in immunosuppressed patients (Dr. Marguerite McDonald, personal communication, June 1999). To our knowledge, S marcescens has not been reported to cause a localized conjunctival infection simulating a neoplasm. We managed a patient without contact lenses who developed a solidappearing epibulbar mass that proved to be a conjunctival cyst infected with S marcescens. A healthy 80-year-old man with bilateral pseudophakia and open-angle glaucoma developed a painless, subconjunctival mass in the right eye. He was being treated with topical latanoprost and timolol in standard doses. He denied trauma, contact lens use, and immunosuppression. Visual acuity was 6/6, and intraocular pressure was 21 mm Hg in each eye. A pink–yellow, solid-appearing mass in the bulbar conjunctiva inferonasally measured 12 ⫻ 7 ⫻ 5 mm (Figure 1). No discharge or inflammatory signs existed. Our differential diagnosis included lymphoma and amelanotic melanoma, lipoma, and fibrous histiocytoma. We believed an infectious process was unlikely. Excisional biopsy was planned. However, during dissection of the mass from the sclera, the lesion ruptured and purulent material was liberated. Cultures and sensitivities were submitted, and the residual lesion was removed. The area was irrigated, and the patient was treated with ciprofloxin drops every 2 hours. Gram stains disclosed intracellular gram-negative bacilli, and cultures were positive for S marcescens, sensitive to ciprofloxin. Ten days later, the area had healed and ciprofloxin was discontinued. Histopathologic examination disclosed a collapsed cyst lined by nonkeratinizing stratified epithelium. The lumen of the cyst contained desquamated epithelial cells and macrophages with numerous intracytoplasmic gramnegative rods, compatible with S marcescens (Figure 2). The patient has no recurrence after 1 year. Ocular involvement by S marcescens usually occurs as keratoconjunctivitis, particularly in patients who wear soft

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