KERATOKONJUNGTIVITIS LIMBIK SUPERIOR
(KLS)
PENDAHULUAN
Epitel keratitis dan hipertrofi papil konjungtiva tarsal superior sering
mengacaukan karakteristik dari keratokonjungtivitis limbus superior yang
berhubungan erat dengan limbus superior.
Pada tahun 1963 Thygeson dan Kimura mendeskripsikan
keratokonjungtivitis limbus superior sebagai konjungtivitis filamen yang
kronis dan terlokalisir. Pada saat ini oleh Theodore penyakit ini diberi
nama yang lebih dikenal dengan istilah keratokonjungtivitis limbus
superior. Lima tahun yang lalu Tenzel dan Corwin melaporkan bahwa
adanya kaitan tiroid yang abnormal dengan keratokonjungtivitis limbus
superior.(5)
ANATOMI KONJUNGTIVA
Konjungtiva merupakan membran yang menutupi sklera dan
kelopak bagian belakang. Bermacam-macam obat mata dapat diserap
melalui konjungtiva ini. Konjungtiva mengandung kelenjar musin yang
dihasilkan oleh sel goblet. Musin bersifat membasahi bola mata terutama
kornea.
Konjungtiva terdiri atas tiga bagian, yaitu :
- Konjungtiva tarsal yang menutupi tarsus, konjungtiva tarsal sukar
digerakkan dari tarsus.
- Konjungtiva bulbi menutupi sklera dan mudah digerakkan dari
sklera dibawahnya.
- Konjungtiva fornises atau forniks konjungtiva yang merupakan
tempat peralihan konjungtiva tarsal dengan konjungtiva bulbi.
Konjungtiva bulbi dan forniks berhubungan dengan sangat longgar
dengan jaringan dibawahnya sehingga bola mata mudah bergeerak.(8)
DEFINISI
Keratokonjungtivitis limbus superior adalah suatu peradangan
konjungtiva bulbi dan konjungtiva tarsus superior yang tidak diketahui
sebabnya, disertai kelainan-kelainan pada limbus bagian atas.(8,10)
EPIDEMIOLOGI
Penyakit ini biasanya bilateral, simetris, terletak pada limbus sekitar
jam 12.. dapat juga unilateral. Lebih sering terdapat pada wanita. Dapat
mengenai penderita berusia antara 4 – 81 tahun. Kelainan ini bersifat
menahun, disertai remisi dan eksaserbasi dan diduga ada hubungannya
dengan hipertiroid.(8,10)
ETIOLOGI
Tidak diketahui penyebabnya. Dianggap merupakan infeksi viral
dan gangguan kelenjar thyroid.(8,9,10)
PATOFISIOLOGI
Baru-baru ini ada pendapat yang mengemukakan bahwa
keratokonjungtivitis limbik superior terjadi karena longgarnya konjungtiva
bulbi superior, yang menyebabkan terjadinya peradangan dari kerusakan
jaringan oleh mikrotrauma. Pengaturan toleransi fisiologi dari
penghancuran jaringan permukaan mata yang sangat halus, peradangan
yang kronis akan mengakibatkan menebalnya konjungtiva dan timbulnya
keratinisasi, yang kemudian akan menjadi inflamasi. Peristiwa ini (5)SLK is
believed to be present secondary to superior bulbar conjunctiva laxity, which induces inflammatory
changes from mechanical soft tissue microtrauma.4 In settings where the physiological tolerance of
mechanical forces on the delicate ocular surface is exceeded, chronic inflammation results in
thickening of the conjunctiva and keratinization, which is then cyclical in perpetuating the
inflammation. Eventually, a filamentary response may be induced on the affected cornea. Factors
inducing conjunctiva laxity include thyroid eye disease, tight upper eyelids, and prominent globes.
Immunochemical histopathologic examination of the abnormal conjunctiva in SLK lends credence to
microtrauma being of most significance to the development of SLK.
HISTOPATOLOGIK
Secara histopatologik kerokan epitel konjungtiva tarsus
memperlihatkan epitel normal dan kadang-kadang ditemukan sel
polimorfonuklear. Tidak pernah ditemukan sel eosinofil ataupun inklusi sel
epitel. Dengan pewarnaan Giemsa ditemukan kreatinisasi sel-sel epitel
yang ditandai dengan pengerutan dan hialinisasi sitoplasma dan nukleus
yang berdegenerasi dan piknotik. Pada biopsi konjungtiva bulbi,
ditemukan kreatinisasi dan akantosis, sel epitel membengkak dan
berdegenerasi. Terdapat degenerasi balon pada nukleus sel-sel radang
neutrofil, sel limfosit dan sel plasma.(8,10)
GAMBARAN KLINIS
Gejala subjektif:
Pada keadaan yang ringan terdapat rasa tidak enak pada mata,
sedangkan pada keadaan yang berat dapat sampai terjadi blefarospasme
dan rasa seperti ada benda asing, lakrimasi dan fotofobia.(8,9,10)
Gejala objektif :
Pada keadaan yang ringan ditemukan peradangan kapiler dan
hipertrofi papil pada bagian tengah konjungtiva tarsus superior.
Konjungtiva tarsus inferior tak ada kelainan. Injeksi konjungtiva pada
episklera ditemukan pada konjungtiva bulbi.
Pada konjungtiva bulbi yang terkena terdapat bendungan,
penebalan dan hipertrofi daerah limbus. Pada keadaan yang berat terlihat
seolah-olah ada pembentukan lengkung limbus yang baru. Dapat dijumpai
pewarnaan pungtata kornea pada pemeriksaan dengan zat warna dan
dapat ditemukan filamen-filamen pada kornea (1/3 bagian atas). Dapat
terjadi remisi spontan dan keadaan patologik yang terjadi dapat
menghilang hanya dalam satu hari.
Kebanyakan penderita mengalami rekurens dan remisi tanpa
menimbulkan parut, lamanya bervariasi 1 sampai 10 tahun. Gejala satu
mata lebih berat dari yang lain. Jarang yang unilateral.(8,10)
DIAGNOSIS BANDING
Conjunctivitis allergic
Conjunctivitis bacterial
Conjunctivitis Giant Papillary
Conjunctivitis viral
Dry eye syndrome
Episcleritis
Floppy eyelid syndrome
Keratoconjunctivitis epidemic
Keratoconjunctivitis sicca
Red eye evaluation
Sebaceous gland carcinoma
Thyroid ophthalmopathy
Trachoma(5)
TERAPI
MEDIKAMENTOSA
Pengobatan yang tepat belum ada, karena penyebabnya belum
jelas. Dapat diberikan pengobatan secara simtomatik berupa tetes mata
dekongestan, zinc sulfat, metil selulosa, polivinil alkohol, kortikosteroid
atau antibiotik.
Pada waktu akut dapat juga diberikan AgNO3 0,5%-1% yang
diusapkan pada konjungtiva tarsus superior, pemberian tekanan, dan
memperbesar diameter pemakaian lensa kontak, retinoic acid topical
0,1% dan siklosporin. Injeksi triamsinolon pada tarsal superior dapat
mengurangi gejala dan tanda dari konjungtivitis limbus superior dan
merupakan teerapi adjuvant. Lebih dari 50% penderita
keratokonjungtivitis limbus superior bisa menjadi keratokonjungtivitis sika
dan terdapat masa yang padat di pungtum bagian atas setelah
pengobatan keratokonjungtivitis limbus superior.
PEMBEDAHAN
Reseksi limbus atau reseksi konjungtiva bulbi bagian atas dengan
menggunakan zat warna rose bengal, kauterisasi konjungtiva bulbi bagian
atas.
KONSULTASI
Diagnostik yang tepat dari fungsi tiroid melibatkan ahli
endokrinologi sebagai ahli konsultasi.(5,8,9,10,)
PROGNOSIS
Prognosis umumnya baik dan pada kasus-kasus yang telah sembuh,
biasanya tidak dijumpai gangguan penglihatan dan gejala sisa.(8,10)
Definition: SLK is a bilateral chronic recurrent inflammatory lesion
located in the superior bulbar and tarsal conjunctiva.
Etiology: SLK is often associated with thyroid dysfunction. One theory is
that lid retraction results in rubbing of the tarsus and bulbar conjunctiva
producing inflammation.
Epidemiology: Most common in adult women 20-70 years old.
Clinical: A bilateral and localized process confined to the superior limbal
area and tarsus from the 10-2 o'clock position. Thickening of the
conjunctiva accompanies dilated blood vessels and erythema of the
conjunctiva. Both Rose Bengal and fluorescein stain in this region.
Clinically these cases are often associated with dysthyroid eye disease
that features lid retraction. In general thyroid function tests are
recommended in patients that present with these findings.
Histopathology: Click to enlarge photo.
Superior limbic keratoconjunctivitis has been
described as having keratinization of the epithelium (see the
keratohyaline granules and anucleate squamous cells here) , acanthosis
(notice the thickening to the far right), and cellular infiltration with
lymphocytes, plasma cells and ballooning degeneration (see central cystic
space with degeneration of epithelial cells and exocytosis of
lymphocytes). In addition in this photo there is a chronic perivascular
inflammatory infiltrate.
Treatment: SLK often spontaneously resolves but may recur for 10 years.
Some treatments that have been used include bandage contact lenses,
punctal occlusion, topical cyclosporin A (0.5%), topical application of
autologous serum, thermo and chemical cauterization of the area affected
, and resection of the conjunctiva.(1)
GAMBAR(2)
GAMBAR(3)
The conjunctiva
The conjunctiva is a mucous membrane that performs the task of
attaching the eyeball to the orbit and lids. It permits a certain degree of
rotation of the eyeball in the orbit (the hole in the skull intended for the
eyeball). The conjunctiva lines the lids and also covers the surface of the
eyeball. The part of the conjuctiva that lines the lids is called the palpebral
portion and the part that covers the white of the eyeball is called the
bulbar conjunctiva.
The other parts of the conjuctiva include two loose recesses, known as the
upper and lower fornices. They are also known as conjunctival sacs and
are located between the bulbar and the palpebral conjunctiva. It is
possible for the eyelids and eyeballs to move only because of the
looseness of the conjunctiva at these points. If you’ve been to an
ophthalmologist or had the experience of having eye drops put in your
eyes. The eye drops are put inside the lower fornice. This is done by
pulling the outer lid away from the globe. The drops are retained in this
cavity for a short while, which is enough for the liquid to diffuse through
the cornea and act on the internal structure of the eye.(3)
The typical patient with superior limbic keratoconjunctivitis (SLK) is a woman aged between
20 and 60 years of age with chronic red and irritable eyes.1 Although both eyes are usually
affected, the condition maybe asymmetrical.1 After episodes of exacerbation and remission it
usually resolves. The patient may also have abnormal thyroid function.2
SLK has been treated with silver nitrate or thermal cauterisation of the superior bulbar
conjunctiva, pressure patching, and large diameter bandage contact lenses (BCL), topical
trans-retinoic acid 0.1%, and recession or resection of the superior bulbar conjunctiva.1, 3 Over
50% of patients with SLK are said to have keratoconjunctivitis sicca4 and recently upper
punctal plugs have been used to treat SLK.5
We report two cases in which a unilateral BCL wear ameliorated the symptoms of bilateral
SLK and a possible explanation is discussed.(4)
Differential Diagnoses
Conjunctivitis, Allergic Keratoconjunctivitis, Epidemic
Conjunctivitis, Bacterial Keratoconjunctivitis, Sicca
Conjunctivitis, Giant
Papillary
Red Eye Evaluation
Conjunctivitis, Viral Sebaceous Gland Carcinoma
Dry Eye Syndrome Thyroid Ophthalmopathy
Episcleritis Trachoma
Floppy Eyelid Syndrome
Other Problems to Be Considered
Filamentary keratopathy
Workup
Laboratory Studies
Thyroid evaluation - Thyroid-stimulating hormone, free thyroxine (T4), thyroid-
stimulating immunoglobulin, or thyroid-stimulating hormone–binding inhibitory
immunoglobulin
Schirmer test, measurement of tear lake, and tear breakup time evaluating for dry eye
syndrome, which is often present with SLK
Histologic Findings
Surgical specimens taken from patients with SLK who had not received treatment with silver
nitrate demonstrate abnormal limbic epithelium with keratinized epithelial cells with
dyskeratosis and acanthosis and balloon degeneration of some nuclei. The intracellular
accumulation of glycogen in the epithelial cells of tissue sections of the bulbar conjunctiva
has been documented. The conjunctival stroma demonstrates edema without significant
inflammatory cellular infiltrate. In specimens obtained after silver nitrate treatment,
significant numbers of inflammatory cells, including plasma cells, neutrophils, and
lymphocytes, also are found in the epithelium and stroma.
Immunohistochemical pathologic examination of the abnormal conjunctiva in SLK
demonstrates a lack of the typical mosaic pattern of the epithelium in the resulting keratinized
cells before undergoing treatment and up-regulation of transforming growth factor-beta 2 and
tenascin. In separate studies, increased expression of proliferating cell nuclear antigens and
altered expression of cytokines, as well as the presence of involucrin, were shown.(5A)
Treatment
Medical Care
Several approaches are used by practitioners to speed the recovery of patients toward the
resolution of symptoms. Pressure patching, placement of a bandage contact lens (primarily or
as an adjunct), silver nitrate solution application, mast cell stabilizers, vitamin A preparations,
and cyclosporine A6 have been used with moderate success. Supratarsal triamcinolone
injection has had reported success in mitigating signs and symptoms and may be helpful as an
adjunctive therapy.7 As these approaches usually offer only temporary mitigation of
symptoms, more definitive treatments often are required.
Surgical Care
Surgical resection of the involved conjunctiva as delineated intraoperatively by the use of
rose bengal staining removes the affected tissue. Folds of superfluous conjunctiva are
eliminated, adhesions with underlying Tenon capsule and episclera develop, which may be
augmented by transplantation of cryopreserved amniotic membrane with fibrin glue,8 and
keratinized epithelium is replaced by normal ingrowth.9,10 Thermocautery accomplishes 2 of
these treatment objectives. Autologous serum application has been shown to be beneficial as
an alternative therapy in a small case series.11 Superior lacrimal punctal occlusion and
bandage contact lens application have been advocated but are not widely used.
Consultations
Appropriate investigations into thyroid function may involve an endocrinologist consultation.
Medication
Both mast cell stabilizers and vitamin A preparations have been used with moderate success.
However, these approaches usually offer only temporary mitigation of symptoms, and more
definitive treatments often are required. Preservative-free artificial tears also may be helpful.
Recently, topical cyclosporine A has been shown to provide symptom relief and to improve
the signs of SLK; however, maintenance therapy is required for continued benefit.12
Mast cell stabilizers
Long-term inhibition of inflammation. Inhibits type 1 immediate hypersensitivity reaction.(5B)
Background
This disorder is characterized as an inflammation of the superior bulbar conjunctiva with
predominant involvement of the superior limbus, an adjacent epithelial keratitis, and a
papillary hypertrophy of the upper tarsal conjunctiva.
In 1963, Thygeson and Kimura described it as a chronic, localized, filamentary
conjunctivitis.1 It was given its name, superior limbic keratoconjunctivitis (SLK), by
Theodore, contemporaneously. Five years later, Tenzel and Corwin reported an association
with thyroid abnormalities and SLK.2,3 A mimicking disorder has been encountered in soft
contact lens wearers, typically with exposure to thimerosal-preserved solutions.
Pathophysiology
SLK is believed to be present secondary to superior bulbar conjunctiva laxity, which induces
inflammatory changes from mechanical soft tissue microtrauma.4 In settings where the
physiological tolerance of mechanical forces on the delicate ocular surface is exceeded,
chronic inflammation results in thickening of the conjunctiva and keratinization, which is
then cyclical in perpetuating the inflammation. Eventually, a filamentary response may be
induced on the affected cornea. Factors inducing conjunctiva laxity include thyroid eye
disease, tight upper eyelids, and prominent globes. Immunochemical histopathologic
examination of the abnormal conjunctiva in SLK lends credence to microtrauma being of
most significance to the development of SLK.
Frequency
United States
The frequency of SLK has been found to be 3% in a cohort of Graves ophthalmopathy
patients, but it is much lower in the general population.
International
The international frequency is unknown.
Mortality/Morbidity
The natural history of the disorder is remission and eventual total resolution but only after a
prolonged clinical course.
Race
No racial predilection exists.
Sex
Women are predominantly affected.
Age
Typically, middle-aged people are affected; however, this entity has been reported to occur in
patients aged 4-81 years.
Clinical
History
Patients present with complaints of burning and irritation of the affected eye.
o Some patients may present with redness. Upgaze may elicit these symptoms.
o Typically, usage of moisturizing medications only provides minimal relief.
o Symptoms remit and exacerbate and are variable in degree, but no diurnal pattern
to the worsening of symptoms exists.
In most cases, the condition is present bilaterally, although one eye may be more
symptomatic.
Patients with filaments are usually extremely symptomatic.
Commonly, a history of thyroid dysfunction is elicited upon questioning. The natural history
of SLK is prolonged, with gradual clearing.
Patients often have numerous eye specialists for their symptoms. Unless the doctors have
specifically examined the upper bulbar conjunctivae or everted the upper eyelids, they may
have missed the diagnosis.
Physical
Marked inflammation of the upper lid tarsal conjunctiva, adjacent inflammation of the upper
bulbar conjunctiva, and punctate rose bengal staining of the cornea at the upper limbus are
signs of SLK.
The conjunctiva extending from the upper limbus to the insertion of the superior rectus
muscle also demonstrates thickening, hyperemia, and typical rose bengal staining. It stands
out in stark contrast to the normal appearance of the inferior conjunctiva and cornea.
Approximately one third of patients present with filaments on the upper cornea or along the
superior limbus.
Causes
The cause of SLK is unknown, but inflammatory changes from mechanical soft tissue
microtrauma are the final common pathway.
SLK is associated with thyroid dysfunction.
SLK has also developed in association with scarring of the palpebral conjunctiva in euthyroid
patients.
Prolonged eyelid closure with associated hypoxia or reduced tear volume may be a risk
factor for SLK development.
Morphological or functional changes in superior conjunctival apposition to the globe
following upper eyelid procedures may induce SLK.(5C)
Signs and Symptoms
Individuals presenting with SLK typically report symptoms of ocular discomfort, including burning, foreign-
body sensation, or non-descript pain. Additionally, patients may complain of photophobia and excessive tearing.
Gross clinical signs often include mild lid swelling and pseudoptosis as well as blepharospasm. Visual acuity is
usually not affected.
Inspection of the ocular surface in SLK reveals a sectoral inflammation and injection of the superior bulbar
conjunctiva. The limbal margin of the cornea may be inflamed as well. Eversion of the upper lid reveals a
uniform papillary hypertrophy along the tarsus, which may be mild to marked. Vital dye staining is standard in
SLK, with patients displaying punctate epithelial disruption of the affected region; this is evident with both
sodium fluorescein dye as well as rose bengal or lissamine green solutions. Filaments are encountered within the
precorneal tear film in roughly half of all patients with SLK. The condition is typically bilateral but often
asymmetric. In most instances, the diagnosis of SLK is based solely upon the characteristic presentation. The
only known laboratory confirmation is the presence of keratinized epithelial cells from scrapings of the affected
superior bulbar conjunctivae.
Pathophysiology
The exact etiology and pathogenesis of SLK remains unclear. Infectious agents such as bacteria, viruses, fungi,
and other intracellular parasites appear to be unrelated to this condition. An autoimmune etiology has been
considered, based upon the pattern of the disorder (i.e., exacerbations and remissions), the female
predominance, and an association with thyroid disease and other autoimmune diseases.
The most widely accepted theory regarding the pathogenesis of SLK is that it results from mechanical irritation
of the superior limbal region, as loose conjunctival tissue rubs against the limbus during blinking. Factors such
as tight lids, prominent globes, and thyroid disease have been offered as potential instigators of this reaction.
A newer theory regarding the etiology of SLK implicates a local tear deficiency to the superior
keratoconjunctiva. Researchers have proposed that this deficiency results in significantly reduced levels of vital
tear-based nutrients to the affected region, as well as increased mechanical friction from the superior lid.
Management
SLK is a chronic, recurrent and sometimes recalcitrant disorder. While no treatment has yet been shown to be
100 percent effective, many modalities have been employed successfully. The treatment of choice for most
practitioners has been 0.5 to 1.0% silver nitrate solution, applied topically to the superior bulbar and tarsal
conjunctivae. This treatment chemically cauterizes the irregular tissue, promoting regrowth of new, healthy
epithelium. Unfortunately, recurrences have been known to occur after using silver nitrate, and retreatments are
common.
Pressure patching has been employed for severely symptomatic cases of SLK, as well as the use of subsequent
bandage hydrogel lenses to alleviate the mechanical irritation. Thermal cauterization as well as surgical
recession or resection of the superior bulbar conjunctiva has also been employed as treatment modalities for
SLK. The use of topical preparations, including vitamin A eyedrops, 4% cromolyn sodium solution, and
Alomide (0.1% lodoxamide tromethamine solution, Alcon) have also been somewhat effective in managing
SLK. Most recently, lacrimal punctal occlusion therapy has been advocated for this disorder.
Clinical Pearls
In managing this disorder, topical agents should be employed in the early stages of all mild and
moderate presentations; thermocautery, chemocautery, and surgical resection should be employed only
when less invasive means have failed.
Lacrimal occlusion therapy may prove to be a viable option for SLK; while additional research is
needed in this area, recent studies have shown great potential.
SLK of Theodore should not be confused with contact lens-induced SLK (CL-SLK), a condition that is
occasionally observed in young, otherwise healthy hydrogel lens wearers. An association with
thimerosal-preserved solutions has been seen in some of these patients. The typical presentation of CL-
SLK consists of increasing contact lens intolerance, superior tarsal and bulbar injection, and significant
superior corneal staining with stromal hazing. Corneal involvement may be noted as far inferiorly as
the superior pupillary margin. Treatment for CL-SLK consists of temporarily discontinuing contact
lens wear, along with the liberal use of preservative-free ocular lubricants. Upon resolution, contact
lens wear may be resumed with a fresh pair of lenses, however all thimerosal-preserved solutions
should be terminated. In more severe or recurrent cases, patients may need to be refit with RGP
materials.
Refer all patients presenting with SLK for a systemic workup, including a serologic thyroid panel. A
1995 study demonstrated a 65 percent correlation between SLK and systemic thyroid disease. Other
disorders such as rheumatoid arthritis and Sjögren’s syndrome may also have similar associations.
Other reports in this section
Allergic Conjunctivitis &Vernal Keratoconjunctivitis
Viral Conjunctivitis
Bacterial Conjunctivitis
Chlamydial & Gonococcal Conjunctivitis
Conjunctival Laceration
Episcleritis
Scleritis
Pingueculitis
Pterygium
Superior Limbic Keratoconjunctivitis (SLK of Theodore)
Toxic Conjunctivitis
Conjunctivitis with Pseudomembrane
Giant Papillary Conjunctivitis (6)
Superior limbic keratoconjunctivitis is an ocular surface disease characterized by
episodes of recurrent inflammation of the superior cornea and limbus, as well as of the
superior tarsal and bulbar conjunctiva.
Even though the pathophysiology remains unclear, it is thought that mechanical
trauma from tight upper lids or loose redundant conjunctiva could lead to the
disruption of normal epithelium. This mechanical hypothesis is supported by the
increased lid apposition of exophthalmic thyroid patients, who are known to have an
increased incidence of superior limbic keratoconjunctivitis.
Patients present with red eye, burning, tearing, foreign body sensation, mild
photophobia. Inflammation and thickening of the conjunctiva is observed, espedially
at the limbus.
Lubrication is an effective treatment for this pathology.(7)
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