金台診所醫學美容的部落格

By Prof Dr.Gehad Elnahri

Conjunctival masses to remember

1-Pinguecula
2-Inflamed pinguecula
3-Pterygium
4-Pseudopterygium
5,6-Conjunctival amyloid
7-Pyogenic granuloma
8-Suture granuloma
9-Malignant melanoma
10-Sarcoid
11,12-Lymphoma
13-Papilloma
14-Hereditary benign intraepithelial dyskeratosis
15-CIN
16-Squamous carcinoma
17-Sebaceous carcinoma
18-Racial pigment
19-Nevus
20-Dermoid

1-Pinguecula:
A relatively common non-malignant, raised yellow-white lesion of the interpalpebral bulbar conjunctiva that does not involve the cornea and represents elastoic degeneration of subepithelial collagen with hyalinized connective tissue. These fleshy lesions are typically found bilaterally and adjacent to the limbus of the nasal bulbar conjunctiva although they can be present temporally as well.

Pingueculae are thought to arise as a result of the effects of environmental irritants such as wind and dust and are associated with UV-light exposure and aging

Treatment: Lubrication with artificial tears and ointment can help with ocular surface irritation. Excision is indicated only when pingueculae are cosmetically unacceptable or when they become chronically inflamed or interfere with successful contact lens wear. Long-term use of topical steroid therapy should be discouraged due to adverse side-effects but can but used judiciously in patients with inflamed pingeuculae termed “pingueculitis”. Topical indomethicin has also been demonstrated at reducing symptoms of inflammation.
Laser photocoagulation and surgical excision of pingueculae have both been used successfully with similar cosmetic outcomes.

2-Inflamed pinguecula:
Long-term use of topical steroid therapy should be discouraged due to adverse side-effects but can but used judiciously in patients with inflamed pingeuculae termed “pingueculitis”. Topical indomethicin has also been demonstrated at reducing symptoms of inflammation.

3-Pterygium:
is a common ocular surface lesion originating in the limbal conjunctiva within the palpebral fissure with progressive involvement of the cornea. The lesion occurs more frequently at the nasal limbus than the temporal with a characteristic wing-like appearance.

The etiology is unknown. An increased incidence is noted in latitudes nearer the equator and in individuals with a history of increased UV exposure. Some studies have shown a slightly higher incidence in males than females, which may only reflect a higher rate of UV radiation.

Treatment:
 

• Inflamed pterygia may cause irritation, foreign body sensation, and tearing which, in many cases, can be alleviated with over the counter vasoconstrictor drops, lubricating drops and ointments.
• Initially, the corneal extension of the pterygium should be measured and followed every 1 to 2 years to determine the rate of growth toward the visual axis.
• Surgical removal

4-Pseudopterygium:
Pseudopterygium is sometimes referred to as cicatricial pterygium. A true pterygium has edges that can be elevated with forceps or under which a probe can be passed A true pterygium aries from a pinguecula. A pseudopterygium arises from destruction of the marginal, corneal epithelium through trauma, e.g. caustics, burns or inflammation.The adjacent conjunctiva migrates to the injured area and becomes fixed to it. A pseudopterygium does not show any tendency to progress. It is able to develop at any point of the corneal circumference.

5,6-Conjunctival amyloid:
Conjunctival amyloidosis usually appears as confluent fusiform lesions or polypoidal papules that have a salmon-colored or yellow-pink color. Any conjunctival surface may be involved, but the superior fornix and tarsal conjunctiva are the most frequently affected. This deposition may result in ptosis. Patients may also present with recurrent subconjunctival hemorrhage from the accumulation of amyloid in the walls of the blood vessels. The eyelid, extraocular muscles, and lacrimal gland may also be affected by amyloidosis.

Amyloidosis is caused by the extracellular tissue deposition of amyloid fibrillar proteins. Amyloid fibrils are insoluble polymers comprised of low molecular weight subunit proteins, which adopt a beta-pleated sheet configuration. The deposition of these insoluble proteins can lead to tissue toxicity and disease.

Treatment:Management modalities include observation, judicious use of artificial tears, excision, or liquid nitrogen cryotherapy for localized conjunctival amyloidosis. For patients with systemic manifestations, stem cell transplant, chemotherapy, and steroids may be considered depending on the extent of the disease. Surgical debulking remains the standard treatment, but because complete excision is not always possible, the disease may recur. Demirci and Leibovitch report a recurrence rate of 21-27% after surgical debulking of localized orbital amyloidosis. Newer therapies such as cryotherapy may further decrease recurrence rate by decreasing blood supply to the surrounding tissue, but more research is needed. Radiotherapy for localized amyloidosis has also been reported but is not considered the standard of care. 



7-Pyogenic granuloma:
Benign vascular proliferation of immature capillaries that is neither purulent nor granulomatous and is also called lobular capillary hemangioma. PG presents as a lobulated raised lesion within the conjunctiva.

Although there are several predisposing factors, pyogenic granuloma is most commonly seen at a traumatic wound site or near a suture line after surgery for chalazion, pterygium, strabismus, retinopexy, enucleation, or other ocular insults. Rare cases have been reported of PG formation secondary to ocular acne rosacea and soft contact lens wear.

Treatment:Most PGs will resolve once the primary inciting cause such as a chalazion is treated. Lubrication with artificial tears and occasionally topical steroid eye drops can help with symptom relief. If the PG does not regress within weeks or is causing symptoms, simple excision can be curative. Shaving, followed by cautery and cryotherapy, is the most common practice, but recurrence is not rare Low-dose plaque brachytherapy can be used if the PG has multiple recurrences[

8-Suture granuloma:
See Pyogenic granuloma

9-Malignant melanoma:
a rare but potentially fatal ocular surface tumor affecting about 0.2 persons per million and representing approximately 2 percent of the malignancies of the eye.

Conjunctival melanoma originates de novo in about 5 percent of all cases, from preexisting conjunctival primary acquired melanosis (PAM) in 75 percent, and from nevi in 20 percent. It is an aggressive tumor, and recurrence rates may be as high as 51 percent over 10 years, especially if the tumor is not located at the limbus and has not been completely excised at the time of surgery. Metastatic disease, involving primarily the parotid, preauricular and submandibular lymph nodes, develops in 26 percent of cases with a mortality rate of 13 percent over 10 years; that rate is true even in referral centers. Recent studies have shown an increasing incidence of conjunctival melanoma in the United States. Correct diagnosis is crucial for early excision, which is associated with a lower risk of metastatic disease and tumor-associated mortality.

10-Sarcoid:
is a granulomatous disease that affects multiple organ systems. As a multi-system disease, it can present with a variety of symptoms which often makes diagnosis challenging. Ocular involvement occurs in approximately 25-38% of cases of sarcoidosis and is the first sign of disease in about 20% of cases. Typical ocular signs of sarcoidosis include granulomatous iridocyclitis, retinal periphlebitis, and/or chorioretinitis5. However, other diseases may present with similar findings, including tuberculosis, syphilis, and primary intraocular lymphoma.

Performance of a thorough slit lamp examination can identify conjunctival nodules in 7-17% of patients with ocular sarcoidosis. These conjunctival findings appear as small, yellow or tan nodules primarily in the lower cul-de-sac. They may also appear on the lacrimal gland. Classically, the lesions are described as having a size and appearance similar to that of a millet seed.

11,12-Lymphoma:
Ocular adnexal lymphomas account for 2 percent of all non-Hodgkin lymphomas, and the conjunctiva is the primary area of involvement in 30 to 40 percent of cases. Conjunctival lymphoma constitutes about 1.5 percent of conjunctival tumors and is reportedly the most common acquired subepithelial lesion.

These lesions are usually primary extranodal B-cell neoplasms, although 10 to 30 percent are secondary tumors in patients with disseminated lymphoma. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-type lymphoma) is the most prevalent subtype, followed by follicular and diffuse large B-cell lymphoma.

The incidence of MALT ocular lymphomas is on the rise, with an annual increase of 6 percent. The masses are bilateral in about 20 to 38 percent of patients. Conjunctival lymphomas typically have an indolent course, and isolated involvement of the conjunctiva portends a better prognosis. However, 20 percent of patients with an initially localized conjunctival lymphoma will eventually develop disseminated disease. Systemic involvement is more common in bilateral (47 percent) than in unilateral lymphoid infiltrates (17 percent).



13-Papilloma:
papilloma is a histopathological term describing tumors with specific morphology. They take on a classic finger like or cauliflower like appearance. Papillomatous lesions often are lobulated with a central vascular core.

Conjunctival papillomas are categorized into infectious (viral), squamous cell, limbal, and inverted (histological description) based on appearance, location, patient's age, propensity to recur after excision, and histopathology. They demonstrate an exophytic growth pattern. Interestingly, inverted papillomas exhibit exophytic and endophytic growth patterns.

Conjunctival papilloma also can be classified based on gross clinical appearance, as either pedunculated or sessile. The pedunculated type is synonymous with infectious conjunctival papilloma and squamous cell papilloma. The limbal conjunctival papilloma often is referred to as noninfectious conjunctival papilloma because it is believed that limbal papillomas arise from UV radiation exposure. Because of its gross appearance, limbal papillomas are typed as sessile. Although rare, inverted conjunctival papillomas sometimes are referred to as mucoepidermoid papillomas because these lesions possess both a mucous component and an epidermoid component.

A strong association exists between Human Papilloma Virus (HPV) types 6 and 11 and the development of conjunctival papillomas. Infectious conjunctival papillomas also are known as squamous cell papillomas. This term arises from its histopathological appearance (i.e., the lesion is confined to the epithelial layer, which is acanthotic). 

14-Hereditary benign intraepithelial dyskeratosis:
The disease process involves acanthosis, dyskeratosis, and parakeratosis of the stratified squamous epithelium of the cornea and oral mucosa. However, the etiology of these cellular changes has not been explicated.

Hereditary benign intraepithelial dyskeratosis has a distinct clinical picture. Diagnosis can be made by slit lamp biomicroscopy alone. Affected patients may have ocular involvement, oral involvement, or both. Oral manifestations of the disease include white, spongy plaques of the buccal mucosa, tongue, or lips. Ocular manifestation of the disease is characterized by bilateral, conjunctival injection with whitish-gray, elevated, gelatinous corneal plaques located in the perilimbal area, most often nasally or temporally. The corneal plaques may become visually significant with extension into the central visual axis, disruption the normal ocular surface, or induction of astigmatism. Corneal neovascularization can occur around areas of plaque formation. Most commonly, neovascularization develops superficially, but involvement of the mid to deep stroma has been reported.

Although originally thought to be congenital, HBID is not present at birth. Symptoms begin in early childhood and follow a waxing and waning pattern throughout life. Few reports have suggested that plaques spontaneously shed, however there has never been photographic documentation of this phenomenon. Excision of plaques leads to recurrence and further exacerbation in most cases.

15-CIN ( Conjunctival intraepithelial neoplasia ):
is non- invasive by definition; the basement membrane remains intact and the underlying substantia propria is spared. It is a slow- growing tumor that arises from a single mutated cell on the ocular surface.1CIN is known by other names including Bowen’s disease, conjunctival squamous dysplasia, intraepithelial epithelioma, and epithelial dyskeratosis.

There are three major clinical variants of CIN: papilliform, gelatinous, or leukoplakic.These categories are not mutually exclusive and may overlap. Leukoplakia refers to whitening and thickening of the tumor’s surface as a result of surface hyperkeratinization. However, most CINs present as gelatinous with hairpin configuration of the associated conjunctival vessels. This configuration is in contrast to the “red- dot” or “strawberry” pattern seen in squamous papillomas.There may also be associated pigment within the lesion, resulting in an erroneous diagnosis of melanoma especially in individuals with darker complexions.

16-Squamous carcinoma:
a malignant lesion in which the dysplastic epithelial cells have penetrated the corneal basement membrane, gaining metastatic potential.
CIN is often the precursor to conjunctival SCC. Due to a barrier provided by Bowman’s membrane, subepithelial cellular invasion is almost exclusively limited to the conjunctiva.Histologically, there is replacement of normal epithelium with disorganized, dyskeratotic, and acanthotic cells with penetration of the basement membrane.

SCC presents similarly to CIN; however, the conjunctival lesion tends to be immobile and more raised in appearance. The presence of a large feeder vessel is suggestive of epithelial basement membrane violation. Rarely, SCC may present as bilateral lesions, which are typically keratinized and papillary in appearance.1

17-Sebaceous carcinoma:

• Eyelid tumors often involve conjunctiva
• May be due to regression of primary meibomian gland carcinoma
• Rarely is a primary conjunctival lesion
• Intraepithelial lesions may not become invasive for many years
• May be associated with Muir-Torre syndrome
• Large anaplastic cells with finely vacuolated cytoplasm
• Open vesicular nuclei and prominent nucleoli
• Tumor spreads via infiltrating lobules, nests, and cords, as well as pagetoid patterns within epithelium

18-Racial pigment:
Complexion-Associated Melanosis ( CAM ), also known as racial melanosis, is a benign lesion found among darkly pigmented individuals.
It is typically observed around the limbus. On examination, the pigmentation appears flat and noncystic. It can cover the conjunctiva extensively and increase in size with age; contrary to nevi and PAM, it is usually bilateral.

Treatment: Although CAM has not been shown to progress to a melanoma, darkly pigmented individuals can develop melanomas, so yearly observation is recommended.



19-Nevus:
Nevi represent more than 50 percent of conjunctival lesions and are the most benign of the pigmented lesions. Nevi usually appear in childhood. Conjunctival nevi are very similar to those of the skin and are categorized as junctional, compound, or subepithelial. They are typically located on the interpalpebral conjunctiva, near the limbus, and they remain relatively stationary throughout life.

Conjunctival nevi can first appear as a nonpigmented sessile mass and become darker later on, especially with puberty or pregnancy. This increase in pigmentation may be misleading, as it may give a false impression that the lesion is growing. On slit-lamp examination, most nevi are well circumscribed with a cystic appearance.

Traetment: Nevi should be monitored regularly. When they are stable, this is usually yearly, but examinations may occur more frequently if warranted. A slit-lamp photo should be taken on the first visit for baseline purposes. If the nevus enlarges, or if there is increased vascularity, prompt evaluation is recommended. A lesion should be removed if there is a change in color or increase in size. Nevi may change with pregnancy or puberty but otherwise should remain stable.

20-Dermoid:
Limbal dermoids are benign congenital tumors that contain choristomatous tissue (tissue not found normally at that site). They appear most frequently at the inferior temporal quadrant of the corneal limbus. However, they may occasionally present entirely within the cornea or may be confined to the conjunctiva. They may contain a variety of histologically aberrant tissues, including epidermal appendages, connective tissue, skin, fat, sweat gland, lacrimal gland, muscle, teeth, cartilage, bone, vascular structures, and neurologic tissue, including the brain. Malignant degeneration is extremely rare.