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Lee - 1999 - Primary Localized Conjunctival Amyloidosis Presenting With Recurrence of Subconjunctival Hemorrhage

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tube insertion is an alternative technique for adult patients, years.

All tubes were in proper position and functioning at


especially those known (or potential) keloid-former cases, last follow-up. Although no further complications have
or those preferring not to undergo laser treatment and occurred to date, possibilities include globe, eyelid, or
wanting to avoid an external facial scar. (Am J Ophthal-
mol 2000;129:244 –245. © 2000 by Elsevier Science Inc. medial canthal tendon injury.
All rights reserved.) 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
T HE TECHNIQUE OF PERFORMING CONJUNCTIVO-
dacryocystorhinostomy (CDCR), a skin approach, has
remained essentially unchanged.1–3 We present a conjunc-
techniques may be employed, the associated lower success
rates and higher costs are practical considerations. More-
tival approach to CDCR with Jones tube insertion, thereby over, those techniques tend to be more time-consuming
avoiding external skin scarring. and equipment may not always be available. One main
The technique was performed on 13 adult patients; all advantage in the studied conjunctival incision approach to
candidates had complete lacrimal obstruction at the com- CDCR is the yield of excellent results without external
mon canaliculus and excessive tearing. All patients were scarring. The studied technique offers an alternative ap-
known (or potential) keloid-formers, and suitable candi- proach to primary CDCR, although, in experienced hands,
dates for a CDCR with Jones tube insertion. Also, all 13 successful results can be obtained regardless of which
patients wanted to avoid an external facial scar. One patient technique is used.
refused laser treatment to open severe bicanalicular scarring,
REFERENCES
although she was amenable to CDCR. Exclusion criteria
included patients with facial trauma, history of ocular or sinus 1. Jones LT. Conjunctivodacryocystorhinostomy. Am J Oph-
malignancy, conjunctival disease, and children. thalmol 1965;59:773–783.
The nose is packed with vasoconstrictive–analgesic 2. Jones LT, Wobig JL. Surgery of the eyelids and lacrimal
medications. Lidocaine 2% with epinephrine is injected system. Birmingham: Aesculapius, 1976:194 –233.
3. Steinsapir KD, Glatt HJ, Putterman AM. A 16-year study of
subcutaneously in the medial canthus, and subconjuncti- conjunctival dacryocystorhinostomy. Am J Ophthalmol 1990;
vally from around the caruncle to the midportion of the 109:387–393.
lower fornix. Malleable retractors and rakes can aid expo-
sure. The lower eyelid is retracted and incised from the
caruncle to the inferomedial fornix. The caruncle is Primary Localized Conjunctival
excised. The soft tissue incision is made posterior to the
lacrimal sac. The periosteum is incised and reflected
Amyloidosis Presenting
(Figure 1, left). The Freer periosteal elevator is used to With Recurrence
make an opening in the thin bone posterior to the lacrimal of Subconjunctival Hemorrhage
fossa. A Kerrison punch is employed to enlarge the
Hung-Ming Lee, MBBS, FRCS(Edin),
osteotomy to about 10 ⫻ 12 mm. The lacrimal sac is not
Joel Naor, MD, Dan DeAngelis, MD, FRCSC,
incised nor anastomosed. Because of the relatively re-
stricted size of the surgical field, maneuvers are somewhat and David S. Rootman, MD, FRCSC
limited. A probe is used to estimate the proper length of
the tube needed and aids in the insertion. The tube is PURPOSE: To report the ocular presentation and histopa-
positioned in the inferomedial fornix and anchored to the thology of a patient with primary localized conjunctival
eyelid margin with a 6 – 0 nylon suture (Figure 1, right). amyloidosis.
The periosteal and muscle layers are closed with 5– 0 METHODS: A 38-year-old woman presented with a recur-
vicryl, and the conjunctiva is closed with 6 – 0 chromic rence of episodes of severe bilateral subconjunctival
suture. Operative time averages 30 minutes (range, 25– 40 hemorrhage. Ocular examination revealed yellowish,
minutes). marked folding and redundancy of the conjunctiva in the
This technique was applied throughout an 8-year period inferior cul-de-sac of each eye.
RESULTS: After two initial conjunctival biopsies that
(between 1990 and 1998) on nine females and four males,
aged 33 to 45 years. All patients except one female had showed only chronic inflammation, a third biopsy re-
unilateral procedures. General anesthesia was used in four vealed the presence of amyloid in the substantia propria
of 13 patients, and local anesthesia with intravenous of the conjunctiva.
sedation was used in nine of 13 patients. Postoperatively,
one patient underwent a tube exchange; another com- Accepted for publication Sept 1, 1999.
plained about occasional tearing; otherwise, all 13 patients From the Toronto Western Hospital, University Health Network,
had surgically successful outcomes without external scar- University of Toronto, Toronto, Ontario, Canada.
Inquiries to David S. Rootman, MD, FRCSC, Toronto Hospital,
ring. Western Division, 399 Bathurst St, EC7-009, Toronto, Ontario, M5T
Follow-up ranged from 9 months to 7 years, median, 4.5 2S8 Canada; fax: (416) 603-1993; e-mail: d.rootman@utoronto.ca

VOL. 129, NO. 2 BRIEF REPORTS 245


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 amy- FIGURE 1. Left eye, severe subconjunctival hemorrhage.
loidosis, (3) secondary localized amyloidosis, and (4) sec-
ondary systemic amyloidosis.1 Ocular amyloidosis is also
classified in the same manner.
Ocular amyloid made local to the conjunctiva is rela-
tively 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 occa-
sions showed chronic inflammatory cell infiltrates and
FIGURE 2. (Top) Yellowish marked folding and redundancy
subconjunctival fibrosis. A third conjunctival biopsy fi- of conjunctiva in the inferior cul-de-sac. (Bottom) Conjuncti-
nally revealed homogenous, eosinophilic, and hyaline val stroma with homogenous, eosinophilic, and hyaline material
material in the connective tissue (Figure 2, bottom), which in the hematoxylin and eosin stain section, ⴛ75 before 55%
showed the following staining characteristics: metachro- reduction.
masia with crystal violet dye, positive Congo-red birefrin-
gence, and dichroism with Congo red under polarized
light. There were also mild chronic inflammatory cells the year, her conjunctival amyloid had remained stable
with a predominance of plasma cells in the infiltrate. without much progression, although she still had recurrent
Electron microscopic analysis of the deposit revealed a episodes of subconjunctival hemorrhage. She was also
fibrillary structure consistent with amyloid. The patient examined extensively for systemic diseases, which were all
was managed conservatively with tear lubricants, and over negative.

246 AMERICAN JOURNAL OF OPHTHALMOLOGY FEBRUARY 2000


The diagnosis of localized amyloidosis is based on the material that exuded from the mass during surgery
demonstration of amyloid material in tissue in the absence revealed S marcescens. Histopathology disclosed an
of clinical evidence of systemic amyloid diseases. Though epithelial-lined cyst with macrophages containing S
amyloidosis may be suspected on clinical grounds, the marcescens.
diagnosis is usually made histologically instead of clini- CONCLUSION: Although S marcescens usually affects the
cally. eye as a keratoconjunctivitis in patients with contact
Conjunctival amyloidosis often presents as a painless, lenses, it can also present as a mass simulating a neoplasm
nodular thickening or swelling of the eyelid/conjunctiva or in a patient who does not wear contact lenses. (Am J
blepharoptosis.2 To our knowledge, this is the first case of Ophthalmol 2000;129:247–248. © 2000 by Elsevier
conjunctival amyloid that presented initially with recur- Science Inc. All rights reserved.)
rent subconjunctival hemorrhage. This is probably corre-
lated with amyloid in the walls of the blood vessels,
because purpura is also a known common manifestation of
primary systemic amyloidosis.
S ERRATIA MARCESCENS IS AN OPPORTUNISTIC GRAM-
negative bacillus once considered to be nonpathogenic
in humans. Human infections with this organism were first
The mere recognition of amyloid in an ocular structure recognized in neonates, in whom it produced meningitis,
should not be considered a complete diagnosis. The pos- pneumonia, urinary infections, and conjunctivitis.1,2
sibility of amyloidosis in extraocular sites associated with Rarely, S marcescens has caused endophthalmitis.3 It is now
underlying diseases or immunoglobulin abnormalities must best known to cause keratoconjunctivitis, particularly in
be considered.3 Evaluation for systemic diseases is advised patients who wear soft contact lenses,4,5 and in immuno-
for such patients, though the results of the investigations
suppressed patients (Dr. Marguerite McDonald, personal
are almost always negative.4
communication, June 1999). To our knowledge, S marc-
escens has not been reported to cause a localized conjunc-
REFERENCES
tival infection simulating a neoplasm. We managed a
1. Brownstein MD, Elliott R, Helwig EB. Ophthalmologic as- patient without contact lenses who developed a solid-
pects of amyloidosis. Am J Ophthalmol 1970;69:423– 430. appearing epibulbar mass that proved to be a conjunctival
2. Smith MH, Zimmermann LE. Amyloidosis of the eyelid and cyst infected with S marcescens.
conjunctiva. Arch Ophthalmol 1975;75:42–50.
A healthy 80-year-old man with bilateral pseudophakia
3. Blodi FC, Apple DJ. Localized conjunctival amyloidosis. Am J
Ophthalmol 1979;88:346 –350. and open-angle glaucoma developed a painless, subcon-
4. Sugar A. Corneal and conjunctival degenerations. In: Kauf- junctival mass in the right eye. He was being treated with
man HE, Barron BA, McDonald MB, editors. The cornea. topical latanoprost and timolol in standard doses. He
New York: Butterworth-Heinemann, 1998:477– 496. 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
Localized Infection bulbar conjunctiva inferonasally measured 12 ⫻ 7 ⫻ 5 mm
by Serratia marcescens Simulating (Figure 1). No discharge or inflammatory signs existed. Our
a Conjunctival Neoplasm differential diagnosis included lymphoma and amelanotic
melanoma, lipoma, and fibrous histiocytoma. We believed an
Jerry A. Shields, MD, Carol L. Shields, MD, infectious process was unlikely.
Ralph C. Eagle, Jr, MD, and Marlene Moster, MD Excisional biopsy was planned. However, during dissec-
tion of the mass from the sclera, the lesion ruptured and
PURPOSE: To report a Serratia marcescens infection that purulent material was liberated. Cultures and sensitivities
clinically simulated a conjunctival neoplasm. were submitted, and the residual lesion was removed. The
METHOD: Case report. area was irrigated, and the patient was treated with
RESULTS: A healthy 80-year-old man without contact ciprofloxin drops every 2 hours. Gram stains disclosed
lenses presented with a pink–yellow conjunctival mass intracellular gram-negative bacilli, and cultures were pos-
that resembled a solid neoplasm. Stains and cultures of itive for S marcescens, sensitive to ciprofloxin. Ten days
later, the area had healed and ciprofloxin was discontin-
Accepted for publication Sept 1, 1999.
From the Oncology Service (J.A.S., C.L.S.), the Department of
ued. Histopathologic examination disclosed a collapsed
Pathology (R.C.E.), and the Glaucoma Service (M.M.), Wills Eye cyst lined by nonkeratinizing stratified epithelium. The
Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. This lumen of the cyst contained desquamated epithelial cells
work was supported by the Eye Tumor Research Foundation, Philadel-
phia, Pennsylvania (J.A.S., C.L.S.), by the Award of Merit in Retina and macrophages with numerous intracytoplasmic gram-
Research, Houston, Texas (J.A.S.), the Macula Foundation, New York, negative rods, compatible with S marcescens (Figure 2).
New York (C.L.S.), and the Noel T. and Sara L. Simmonds Endowment The patient has no recurrence after 1 year.
for Ophthalmic Pathology, Wills Eye Hospital (R.C.E.).
Inquiries to Jerry A. Shields, MD, Oncology Service, Wills Eye Ocular involvement by S marcescens usually occurs as
Hospital, 900 Walnut St, Philadelphia, PA 19107. keratoconjunctivitis, particularly in patients who wear soft

VOL. 129, NO. 2 BRIEF REPORTS 247

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