Conjunctiva and Subconjunctival Tissue
Transcription
Conjunctiva and Subconjunctival Tissue
Clinical Conjunctiva and Subconjunctival Tissue Venkatesh Prajna, Muralidhar Rajamani, Aravind Eye Hospital, Madurai Anatomy The conjunctiva is a thin, translucent, vascular mucous membrane that lines the inner surface of the eyelids and the anterior surface of the eyeball as far as the limbus. This tissue is arranged in a saclike fashion and is composed of a palpebral region (covering the inner aspects of the lids), a bulbar region (covering the surface of the sclera), a forniceal region, and a medial semilunar fold. While the palpebral conjunctiva shares its blood supply with the eyelids, the bulbar component is supplied by the anterior ciliary arteries. The nerve supply to the conjunctiva is through the lacrimal, supraorbital, supratrochlear, and infraorbital branches of the ophthalmic division of the trigeminal nerve. The conjunctival epithelium varies from 2 to 5 cells in thickness and is continuous with the corneal epithelium at the limbus and with the skin at the margin of the lids. While the bulbar conjunctiva is lined by a stratified non-keratinized squamous epithelium, the forniceal and tarsal regions are lined by columnar and cuboidal types, respectively. A key constituent of the cellular architecture is the presence of goblet cells, which accounts for 10% of the basal cells of the conjunctival epithelium. These cells are more prevalent in the medial forniceal and palpebral regions, and play a vital part in secreting the mucin component of the tear film. There is an increase in the number of these cells during chronic inflammation of conjunctiva, while conditions like pemphigoid and vitamin A deficiency causes a decrease. Other cell types which nestle within the epithelial layers include melanocytes, Langerhans' cells, and intraepithelial lymphocytes. Beneath the epithelium lies a loose structure called the substantia propria. This structure contains different cell types which mediate immune responses (mast cells, plasma cells, eosinophils, and lymphocytes) interspersed in a vascular network. This arrangement of immune cells, commonly referred to as conjunctivaassociated lymphoid tissue, existing in a vascular environment, is continuously exposed to potential external infective agents and allergens thus serving as a perfect setting for inflammation to set in. Beneath the conjunctiva lies a fibroelastic tissue, Tenon's capsule, which surrounds the eye ball from the corneoscleral junction to the optic nerve. Tenon's capsule is thicker in children and contains more fibroblasts. Hence surgeries like trabeculectomy performed in children, especially without adjuvant procedures like intraoperative use of antimetabolites, may fail due to the aggressive healing response induced by these fibroblasts.1 Conjunctiva in systemic disease A careful flashlight examination of the conjunctiva in a brightly lit environment often provides comprehensive information about a potential underlying systemic disorder. Information about color, lustre, abnormalities in vascularization, and pigmentation help to suspect an underlying ocular or a systemic cause. A slit-lamp evaluation can then be carried out to focus on the specific area of the pathology. Vitamin A deficiency This systemic condition affects organs throughout the body. The ocular manifestation is termed xerophthalmia and affected individuals present with night blindness, conjunctival xerosis, Bitot's spots, corneal xerosis, keratomalacia, and the "xerophthalmic" fundus. 8 In this condition, the conjunctival epithelium is transformed from the normal columnar to the stratified squamous type. There is an associated loss of goblet cells, formation of a granular cell layer, and keratinization of the surface. The conjunctiva loses its normal lustre and is altered into a dry or unwettable one (Fig. 1.1). It is almost always bilateral. A classic ocular sign is Bitot's spots, which is a superficial, scaly, gray area on the interpalpebral region of the bulbar conjunctiva (Fig.1.2). Corynebacterium xerosis can colonize these spots, and produce a foamy appearance because of the gas-forming nature of these organisms. If untreated, the condition involves the cornea, causing corneal xerosis and finally corneal melting, or keratomalacia. The diagnosis of xerophthalmia is often clinical and does not require any additional Fig. 1 Xerophthalmia. Dry, lustureless, inferior bulbar conjunctiva showing a wrinkled appearance. Fig.2 Bitot's spot. A superficial, scaly, foamy Bitot's spot of the bulbar conjunctiva AECS Illumination investigations. In doubtful cases, impression cytology of the superficial layers of the conjunctival epithelium may be helpful to show the loss of goblet cells and keratinization of epithelial cells. Oral administration of vitamin A is preferred because it is safe, cost-effective, and highly effective. In affected children above 12 months of age, retinol palmitate (110 mg) or retinol acetate (200 000 IU) are given orally immediately and the dose is repeated the following day. An additional dose should be given 2 weeks later to boost liver resources. Children between 6 and 11 months should receive only half the above-mentioned dose, and children less than 6 months one-quarter of the dose. Parenteral administration is indicated in those children with conditions such as persistent vomiting, severe stomatitis, and attendant difficulty in deglutition, severe diarrhea with malabsorption, and septic shock. Such children can be treated with intramuscular injection of 55 mg of water-miscible retinol palmitate (100 000 IU), which replaces the first oral dose. This is repeated the next day. Children of less than 1 year are treated with vitamin A in half the prescribed dosage. After the acute phase is over, dietary supplements with provitamin A-rich foods, should be provided. Xeroderma pigmentosa This condition is inherited as an autosomal recessive disorder. Symptoms appear in early childhood. Affected individuals present with extreme photophobia, photosensitivity, and typical dark pigmentary changes in the skin. They are at an increased risk for malignant lesions in sunexposed mucocutaneous and ocular structures (Fig. 3A). There is an impaired ability to repair ultraviolet light-induced DNA damage, which results in accumulation of the damaged DNA. This accumulation of abnormal DNA leads to chromosomal mutation and cell death and is thought to be responsible for neoplasms in these individuals. Vol. XVI, No.2, April - June 2016 A 9 B Fig. 3 Xeroderma pigmentosa. (A) The widespread skin pigmentation can be seen in this girl of Indian origin Fig.3 (B) The conjunctiva was affected by multifocal recurrent squamous cell carcinomas. Conjunctival involvement occurs mostly in the interpalpebral area in the form of xerosis, telangiectasia, chronic conjunctival congestion, pigmentation, pinguecula, and pterygium. Ocular surface neoplasms such as squamous cell carcinoma, basal cell carcinoma, and malignant melanoma may occur, with a predilection for the limbal area (Fig.3B). Corneal changes include exposure keratitis, band-shaped nodular keratopathy, scarring, ulceration, vascularization, and perforation. The posterior segment is usually spared. Elevated symptomatic conjunctival nodules and suspected neoplasms may require repeated excisions; otherwise the treatment is symptomatic. either autosomal dominant or X-linked in their inheritance pattern. A rare autosomal recessive form, lamellar icthyosis, occurs. In all these conditions, dry scaly lesions are present predominantly over the upper half of the body, mainly around the neck, mouth, and trunk. The conjunctiva may become inflamed primarily or secondarily due to lid anomalies like ectropion." A papillary reaction may develop (Fig.4). The treatment is to provide adequate lubrication and to correct the lid abnormali ties, if present. Sturge-Weber syndrome This is a congenital disorder with a classical triad of cutaneous facial angioma, leptomeningeal angioma, and ocular involvement. The facial angioma typically occurs in the distribution of the ophthalmic division of the trigeminal nerve. Dilated episcleral and conjunctival vessels with aneurysm formation in the limbal area are commonly seen. Glaucoma is a frequent accompaniment, especially in patients with severe onjunctival involvement. Icthyosis Ichthyosis is a heterogeneous family of at least 28 genetic skin disorders. Most pedigrees are Anemia Conjunctival pallor is a sensitive and commonly used sign to detect anemia in children. This examination should preferably be done in broad Fig.4 Icthyosis. (A) There is keratinization of the upper palpebral conjunctiva secondary to ectropion of the upper lids. (B) The classical 'stretched out" skin lesions are seen predominantly around the mouth and neck. 10 daylight and correlated with the other systemic indicators. An important causative factor, especially in underdeveloped economies, is systemic helminthiasis. Conjunctival pallor can be masked by conjunctival inflammation, notably trachoma. Leukemias Involvement of the conjunctiva is not a common feature of leukemia. It occurs in approximately 4% of all patients with this disease. However, it may be the initial sign of the disease or of a relapse. Herein lies the importance of early recognition. The affected individuals present initially with congestion of the bulbar (particularly the perilimbal area) or the palpebral conjunctiva. In some instances, the conjunctiva may be erythematous and chemotic. The lesions are firm and non-tender and often associated with subconjunctival hemorrhage. Histopathologically, the cells infiltrate all layers of the substantia propria. The infiltration may be diffuse or patchy and typically localize around the blood vessels. The conjunctival lesions usually respond rapidly to systemic chemotherapy. Measles keratoconjunctivitis Me a s l e s t y p i c a l l y p ro d u c e s a b i l a t e r a l keratoconjunctivitis. The characteristic Koplik's spots may be seen in the conjunctiva. The plica semilunaris may be swollen. Epithelial keratitis may supervene early in children and late in adults. The signs usually resolve without sequelae in the immunocompetent and well-nourished. Treatment is symptomatic and topical antiinflammatory therapy may provide relief. In children with protein/energy malnutrition, this disease can be particularly devastating. Vitamin A deficiency is also present and may present with rapid keratomalacia. Secondary bacterial infection is common in immunodeficient individuals. Alkaptonuria This is a rare autosomal recessive disorder in which an affected individual's urine turns a dark brown-blackish color when exposed to air. It is AECS Illumination linked to chromosome 3q21-q24, caused by a deficiency of homogentisic 1,2-dioxygenase5. This deficiency results in accumulation of homogentisic acid, which gets deposited in various tissues and organs. Systemic features include pigmentation over the face and nails, calcific and atherosclerotic heart disease, and arthritis. Ocular manifestations include a brown to black pigmentation of the nasal and temporal sclera especially in the area of the horizontal rectus muscle insertions. Pigmentation of the cornea has been reported. Ataxia telangiectasia (Louis-Bar syndrome) The condition is a rare autosomal recessive disorder characterized by early onset cerebellar ataxia, oculocutaneous telangiectasia, ocular motor apraxia (saccadic initiation failure), dysarthria and immuno deficiency. Of these, ataxia is the first sign and is progressive. Chromosomal fragility and increasing susceptibility to ionizing radiation result in a predilection to malignant disorders such as lymphomas and leukemias. Affected individuals tend to have high levels of alpha-fetoprotein in their blood. The most characteristic ocular involvement is the appearance of a conjunctival telangiectasia that appears around the first decade of life. This lesion is usually seen in the interpalpebral bulbar conjunctiva, but may extend to the fornices. It is caused by ultraviolet damage and can be prevented or minimized by early and consistent use of 100% UV filter lenses. Other associated disorders include hypometric saccades, horizontal ocular motor apraxia, deficient accommodative ability, strabismus, and nystagmus. Fabry's disease This X-linked disease is a disorder of lysosomal storage caused by a deficiency of alpha-galactosidase A which degrades glycosphingolipid components of the plasma membranes. This deficiency results in accumulation of glycosphingolipids, especially globotriaosylcerarnide." Conjunctival vascular tortuosity, telangiectasia and cornea verticellata are common manifestations. Vol. XVI, No.2, April - June 2016 11 Osler-Weber-Rendu Syndrome This is a rare autosomal dominant disorder of blood vessels that can cause excessive bleeding. It is characterized by vascular dilatations in a variety of organ systems. Systemic features include epistaxis, dyspnea on exertion, gastrointestinal bleeding, hemoptysis, and hematuria. The classical ocular abnormality detected is conjunctival telangiectasia. It may present with bloody tears or frank external hemorrhage. Retinal tel- angiectasia and arteriovenous malformations have been reported. These vessels are stable and can be distinguished from neovascular bundles by the absence of leakage on fundus fluorescein angiography. Sickle cell disease The conjunctival signs in this condition are fairly specific.Comma-shaped capillary and venular microaneurysms, which disappear under the heat of the examining lamp, are noted in the inferotemporal quadrant of an otherwise pale conjunctiva. These aneurysms reappear after application of a mild vasoconstrictor. These vascular anomalies are exaggerated during sickling crises. Conjunctival tumors Hamartomas Hamartomas are congenital overgrowths of a normal tissue at their naturally occurring site. Hemangiomas are the most common hamartomatous lesion. They are of two types: capillary and cavernous. The capillary type is more common and can be either an isolated conjunctival lesion or part of a lesion involving the orbit and lids as well. These lesions are detected shortly after birth as elevated, soft nodules (Fig. 5) and usually grow and become prominent in the first year of life after which a process of involution occurs. No active intervention is necessary in the vast majority of these cases. Rarely, the lids, periorbital tissue, conjunctiva, and deeper orbital tissues may also be involved. Extensive angiomatous lesions may preclude Fig.5 Subconjunctival capillary hemangioma in an otherwise normal child. Although it had regressed spontaneously from being quite large at birth, it was prone to repeated subconjunctival hemorrhages. On this occasion the hemorrhage is contained within the subconjunctival tissue but can be seen to be spreading anteriorly. visualization of the cornea. Lid involvement causes ptosis. A deeper orbital involvement may cause proptosis which increases with the Valsalva maneuver. Astigmatism can be present if the tumors press on the globe. Amblyopia may develop because of anisometropia or, rarely, stimulus deprivation. Optic nerve compression, strabismus, and exposure keratitis may be associated with some cases. lntralesional or systemic steroids or propranolol are used when necessary (almost always) for an orbital component; the response rate is 30-60%. Systemic interferon alpha-2a, previously reserved for life-threatening hemangiomas." is no longer used due to its side effects. Cavernous hemangioma is a much rarer and larger lesion than the capillary type. lt has a higher propensity to involve deeper structures. This tumor does not regress spontaneously and the management is by surgical excision. Choristomas Choristornas are congenital tumors comprising a proliferation of normal cells in an abnormal location. Dermoids and lipoderrnoids are the most common choristomas that present in the conjunctiva. Dermoids are firm lesions with a 12 Fig.6 Dermoid. A temporallimbal dermoid in a 5-year-old boy causing significant astigmatism. Hair growth is seen on the surface. predilection for the limbus. The surface can either be smooth, dome, shaped or keratinized with hair formation (Fig. 6). A dermoid contains all the tissues of the skin including the dermal adnexal structues such as sebaceous glands and hair. They can present either as an isolated lesion or as a part of goldenhar's syndrome (oculoauriculovertebral dysplasia), in which the structures that are derived from the first branchial arch are also affected. Dermoids have a tendency to grow during puberty. They are usually asymptomatic, but large dermoids can cause irritation, cosmetic disfigurement, or significant astigmatism. In these situations, surgical excision can be considered. As these tumour have a tendency to invade deeper structures, donor corneal scleral tissue should be available at the time of surgery10. Lipoderrnoids are softer, yellow-colored lesions often occur ring at the lateral canthal region. In most instances, the posterior extension cannot be demarcated clearly. Dermal adnexa structures are not seen histopathologically. They selden require any treatment. Surgery, if required, should be performed with extreme caution to avoid damage to other structures, especially the lacrimal gland, and because extensive excision predisposes to symblepharon. Epithelial tumors Squamous papillomas present as fleshy and often multiple pedunculated tumors in children. They AECS Illumination have a vascular stalk surrounded by acanthotic epithelium and are seen in the caruncle, fornix, and eyelid margin. They are caused by the human papilloma virus type 6. Viral shedding can cause large, confluent areas of ocular surface involvement. The surface of a papilloma flows out like branches of a tree with small vascular fronds on the surface. Topical mitomycin (0.02%) is useful in reducing the size of the tumor. It is applied twice a day for 15 days. Depending on the response, a second or third cycle can be repeated, with a drugfree interval of 15 days. Topical interferon therapy also seems to have some benefit. If the lesion does not respond to these medications, excision followed by cryotherapy can be performed. Keratoacanthomas are tumors characterized by pseudoepitheliomatous hyperplasia with a central keratin plug. This tumor presents as a painless, firm, grayish-white keratotic nodule which grows rapidly over a period of 3-4 weeks. This benign tumor is often confused with squamous cell carcinoma. Treatment is by complete surgical excision with application of cryotherapy to the base. Lymphangiectasia This condition is characterized by dilated lymphatic channels and may be diffuse or localized. The lesion usually occurs in the interpalpebral space and may accumulate blood episodically because of communication with a vein. This can cause surrounding conjunctival edema and a subconjunctival hemorrhage. Small lesions can be observed, but larger lesions require excision. Lymphangiomas These tumors are rare and are hamartomatous proliferations lined by endothelium. There is no known hereditary or systemic association. Clinically, in the conjunctiva, they present as clear vessels with variably sized clear fluid-filled cysts either alone or interspersed amongst blood-filled hemangioma vessels. They may increase in size on Valsalva maneuver. Hemorrhage into an orbital lymphangioma can produce rapidly increasing proptosis and present as an ophthalmic emergency Vol. XVI, No.2, April - June 2016 the presence of subconjunctival and buccal lymphangioma tissue may be a useful clinical sign of the underlying cause. Rhabdomyosarcoma This is the most common malignant orbital tumor in children. Conjunctiva is the source of the primary tumor in around 12% of patients with orbital rhabdomyosarcoma.11 The superior and superonasal areas are commonly involved. They present as light brown colored elevated lesions and may be confused for focal areas of inflammation, papillomas, capillary hemangiomas, or conjunctival cysts. Lack of response to medical management and rapid progression is a characteristic feature which should alert the clinician. Histopathologically, it comprises loosely coherent spindle cells in a myxoid stroma. MRI is needed to reveal orbital extension. Treatment is by radiotherapy and adjuvant chemotherapy. 13 Pigmented lesions of the conjunctiva Pigmentation of the conjunctiva is a common occurrence. The lesions are mostly benign in nature, but rarely can assume a malignant potential. A documented progression of these lesions is a clear indication for surgical removal. Conjunctival nevus This is one of the commonest pigmented lesions of the eye. Small, circumscribed lesions have little malignant potential and need not be surgically excised (Fig.7A). However, nevi that show progression, or show features suggestive of feeder vessel vascularization, should be excised (Fig. 7B). Nevi can be either junctional, compound, or subepithelial in type. Injunctional nevi, the melanocytes are restricted to the basilar Neurofibromas and neurilemmomas These tumors arise from the Schwarm cells which lie within the nerve. While neurilemmomas are discrete masses, neurofibromas may present as irregular tortuous lesions that may be local or diffuse. Localized symptomatic neurofibromas can be excised and the diffuse tumors can be managed by periodic partial excision. Juvenile xanthogranuloma Juvenile xanthogranuloma is an idiopathic cutaneous eruption of childhood in which conjunctival involvement is very rare. Most reported conjunctival cases have been in adults without skin lesions. They present as solitary, elevated conjunctival lesions near the corneoscleral limbus. They are usually round or yellow/fleshcolored masses. Histopathological examination reveals lipid histiocytes, chronic inflammatory cells and the characteristic Touton giant cell. Most conjunctival lesions have been excised in order to establish the diagnosis. However, they may spontaneously involute or respond to topical steroids. Fig.7 Conjunctival nevus. (A) Small circumscribed pigmentation of the conjunctiva which does not require active surgical intervention. (B) An aggressive looking nevus with suspicious feeder vessels in the superior aspect. This lesion warrants surgical excision and regular follow-up. 14 AECS Illumination epithelium. Compound nevi extend into the substantia propria, but maintain an intraepithelial component. They may develop cystic areas because of entrapment of goblet cells. The subepithelial type eventually loses its connection with the basilar epithelium and is confined within the substantia propria. Nevus of Ota In this condition, conjunctival or scleral pigmentation (slate-gray or bluish in color) is associated with pigmentation of the periocular tissue as well as the face. The nevi are present at birth. Patients with this condition are at increased risk for developing a uveal melanoma or glaucoma. Melanocytic nevus This is a rare form of congenital nevus that involves the eyelid skin, conjunctiva, and the face (Fig.8). The surface is jet black, hairy, and often nodular. It carries a high risk of malignant transformation, which is estimated to be as high as 45%. Radical excision and reconstructive surgery is the usual treatment. Malignant melanoma This condition is rare in children. Conditions such as dysplastic nevus syndrome, xeroderma pigmentosa, and neurofibromatosis put children at increased risk of developing a malignant melanoma. It is characterized by a rapid growth of pigmented lesions of the conjunctiva, and feeder vessels may be seen. Involvement of Tenon's capsule and the sclera may restrict the mobility of the tumor over the ocular surface. In suspected cases it is important to check the preauricular and submandibular node for enlargement. Miscellaneous disorders of conjunctiva Pyogenic granuloma An aberrant healing response following trauma or surgery for strabismus can result in an exaggerated fibrovascular response, erroneously referred to as pyogenic granuloma. However, this condition is neither an infection nor a typical granulomatous reaction, but a granulation tissue formation. Rapid growth may simulate a malignancy. A history of preceding trauma or surgery helps in making the diagnosis. The condition may resolve spontaneously, but usually some form of treatment is required. A short course of topical steroids may be given, but excision biopsy is the treatment of choice. Fig. 8 Giant melanocytic nevus. (A) Affecting the left half of the face along with pigmentation of the left eye. (B) A close-up view of the left eye showing dense pigmentation of the conjunctiva more on the nasal half. The surrounding skin of the eyelids also shows dark pigmentary changes. Subconjunctival hemorrhage A subconjunctival hemorrhage often occurs after a seemingly trivial blunt trauma (Fig. 9). It can also be seen in conditions which cause a rise in central venous pressure such as a seizure, violent coughing, or sudden straining. It may present in a dramatic manner and may cause considerable consternation. It usually resolves spontaneously Vol. XVI, No.2, April - June 2016 15 Fig. 10 An elevated nodular lesion seen in a 7-yearold girl on anti-tuberculous treatment. Fig.9 Subconjunctival hemorrhage following a fist injury to the eye. An associated ecchymosis of the lower lid is also seen. within 2 weeks and does not require any treatment. Subconjunctival hemorrhages are also seen in conditions such as leukemia and as a sequelae to some forms of conjunctivitis. In cases of suspected head injury, the presence of a subconjunctival hemorrhage with a poorly defined posterior margin is a matter of grave concern and has to be investigated radiologically. Conjunctival granulomas The presence of a variety of lymphoid cells in the substantia Propria serves as a perfect setting for the development of inflammatory granulomas following some systemic and local disorders, The common systemic diseases that can cause conjunctival granulomas include sarcoidois, tuberculosis, Parin-iud's oculoglandular syndrome, Wegener's granulomatosis, trematode-induced granulomas, and rhinosporidiosis. Sarcoid nodules manifest as small light brown colored nodules in the conjunctiva and show aggregates of epitheioid histiocytes on histopathological evaluation. Tuberculosis of the conjunctiva may cause granulomas, tarsal necrosis, conjunctival masses, and small miliary palperal conjunctival ulcers. Phlyctenular conjunctival response can also be seen in some cases (Fig.10). Coexisting anterior segment involvement may be present. Fig. 31.11 River water granuloma. Well-circumscribed smooth elevated nodular lesion seen in the bulbar conjunctiva. (Courtesy of Dr. Rathinam, DNB) Trematodes are known to cause conjunctival granulomas. They are acquired by children while swimming in fresh water ponds infested by these trematodes. The granuloma is usually seen as a smooth, enlarged nodular lesion in the bulbar conjunctiva (Fig.11). Coexistent anterior chamber granuloma and corneal inflammation may be seen in some cases. A specific inflammatory reaction known as Splendore-Hoeppli phenomenon has been described with helminthic infections, though the helminths themselves have rarely been isolated. The reaction comprises a central deposit of granular, acellular eosinophilic material surrounded by eosinophils, epithelioid cells, histiocytes. and lymphocytes. Small granulomas may be treated with topical corticosteroids. 16 AECS Illumination Fig.12 Rhinosporidiosis. Pedunculated irregular granuloma seen in the inferior palpebral conjunctiva. Note the white spherules over the surface of the granuloma. (Courtesy of Dr. Usha Kim, DNB.) Fig. 13 Cysticercosis ofthe conjunctiva. A subconjunctival translucent cyst in the bulbar conjunctiva caused by cysticercosis. Large lesions and those that fail conservative management should undergo an excisional biopsy. Rhinosporidiosis may present as pedunculated granulomas, often seen in the palpebral conjunctiva (Fig.12). The surface is granular and on careful examination reveals pearly white studded spores, which have a tendency to bleed on touch. Treatment is by complete excision followed by cryotherapy. This lesion has an extraordinary tendency to bleed during surgery. Ophthalmia nodosa is a granulomatous nodular conjunctivitis caused by irritation of the eye due to retained capillary hairs of caterpillars, spiders, or bees. Children presenting with this condition often give a history of a fall. Small nodules may be seen in the conjunctiva along with patterned corneal abrasions. These corneal abrasions are due to the hairs embedded in the upper tarsus and their sharp ends rubbing the cornea. subconjunctival in location with a thin translucent wall (Fig.13). Adjacent orbital structures and muscles may be involved. A chalky white area representing the scolex may be seen which clinches the diagnosis. Spontaneous expulsion of these cysts has been reported. A complete ophthalmic and systemic evaluation is needed to look for other involved areas. Treatment is by mechanical removal with supplementation of systemic antihelminthic treatment. Exuberant inflammatory responses may need a course of systemic steroid therapy. Parasitic infestation of the conjunctiva This condition is common in endemic areas and usually presents in the cyst form. The common parasitic cysts are cyst-icercus and hydatid. They may present with inflammation. These parasitic cysts have to be removed in toto along with cryotherapy of the base. Cysticercosis is caused by the larval form of Taenia solium known as cysticercosis cellulosae. The cysts are usually Ophthalmomyiasis This term refers to the infestation of the eye by the larval forms of the order Diptera. Implicated species include Oestrus ovis (transmitted from sheep and goats by gravid adult flies), Dermatolabia hominis. (transmitted from cattle and fowl by mosquitoes, such as Cuterebra, Hypoderma bovis, chng somyia and cordylobia). Conjunctival involvement causes irritation, foreign body sensation, redness, and chemosis. There may be pseudomembrane formation with petechial hemorrhages. Patients may experience perception of movements and slit-lamp examination shows the larvae. The aim of the treatment is to extract the worm mechanically. The worm may be caught with a forceps or pulled out with a suture passed through the larva. Vol. XVI, No.2, April - June 2016 17 Fig. 14 Subconjunctival worm. The contour of this worm, identified later as Filaria, is clearly seen. Fig.15 Subconjunctival foreign body. A small twig embedded in the conjunctiva and required mechanical removal. Occasionally, adult worms can be seen presenting in the subconjunctival space with the patient complaining of irritation and foreign body sensation. A slit-lamp evaluation will reveal the contours of the worm (Fig.14). The treatment by mechanical removal of the worm using a forceps. In some instances, the worm is entrapped beneath the conjunctiva in close proximity to the extraocular muscles. In these situations, care should be taken to isolate the muscle and extract the worm without injuring the muscle. anteriorly into the subconjunctival space and may present as a cystic lesion beneath the conjunctiva. Thi s condition, which may mimic a subconjunctival cyst, is referred to as a phacocele (Fig.16), which may also be a presenting sign of Ehlers-Danlos syndrome type 6. Conjunctival trauma and foreign bodies The conjunctiva is a common site for foreign bodies, most commonly upper tarsal. An examination after lid eversion is mandatory for any history of trauma and suspicion of foreign body. Various foreign bodies that have been described include insect wings, beetles, cilia, caterpillar hair, seed husk, pieces of wood, twigs (Fig.15), and natural and synthetic fibers. Any foreign body lodged in the conjunctiva evokes an acute inflammatory reaction with a copious outpouring of tears. However, if the foreign body has a large surface area, it may become embedded resulting in a chronic inflammatory response. This causes a granuloma containing epithelioid and foreign body giant cells. The symptoms rapidly abate after the removal of the foreign body. Very rarely, blunt injuries in the eye, especially in older children and young adults, can cause the lens to dislocate Symblepharon In this condition, there is an adhesion between the bulbar and palpebral conjunctiva. It commonly occurs in association with congenital lid coloboma. Other causes include a chronic dry eye, chemical burns, and Stevens-Johnson syndrome where the goblet cells are destroyed. Localized symblepharon, without compromise to the visual apparatus, need not be treated. Surgical release and forniceal reconstruction using amniotic membrane or mucous membrane grafts is indicated in severe cases. Fig.16 Phacocele. A traumatically anteriorly dislocated lens mimicking a subconjunctival cyst. 18 AECS Illumination References 1. Reynolds [D, Olitsky SE. Pediatric glaucoma. In: Wright KW, Spiegel PH, editors. Pediatric Ophthalmology and Strabismus. New York: Springer; 2002: 483-98. 2. WHO. Vitamin A deficiency and its consequences (accessed Iun 9 2011). Available from: http://www.who. int/nutrition/publications/ rnicronutrients/vitamin a deficieny/9241544783/en/index.html. 3. Sullivan T), J Clarke MP, Morin [JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus 1992; 29: 349-56. 4. Cruz AA, Menezes FA, Chaves R, et al. Eyelid abnormalities in lamellar ichthyoses. Ophthalmology 2000; 107: 1895-8. 5. Sharma V, Chong YV, Kosmin A. Alkaptonuria presenting with conjunctival lesion. Compr Ther 2007; 33: 71-2. 6. Farr AK, Shalev B, Crawford TO, et al. Ocular manifestations of ataxia-telangiectasia. Am J Ophthalmol 2002; 134: 891-6. 7. Mastropasqua L, Nubile M, Lanzini M, et al. Corneal and conjunctival manifestations in Fabry disease: in vivo confocal microscopy study. Am J Ophthalmol 2006; 141: 709-18. 8. Brant AM, Schachat AP, White Rl. Ocular manifestations in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Am 1 Ophthalmol 1989; 107: 642-6. 9. Haik BG, Karcioglu ZA, Gordon RA, Pechous BP. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol 1994; 38: 399-426. 10.Shen YO, Chen WL, Wang I), et al. Full-thickness central corneal grafts in lamellar keratoscleroplasty to treat limbal dermoids. Ophthalmology 2005; 112: 1955. 11.Shields CL, Shields lA, Honavar SG, Demirci H. Primary ophthalmic rhabdomyosarcoma in 33 patients. Trans Am Ophthalmol Sac 2001; 99: 133-42; discussion 142-3. 12.Sinha S, Cohen PJ), Schwartz RA. Nevus of Ota in children. Cutis 2008; 82: 25-9. 13.Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol 1978; 86: 648-55. 14.Rathinam S, Fritsche TR, Srinivasan M, et al. An outbreak of trematode-induced granulomas of the conjunctiva. Ophthalmology 2001; 108: 1223-9 15.Reidy Il. Sudesh S, Klafter AB, Olivia C. Infection of the conjunctiva by Rhinosporidium seeberi. Surv Ophthalmol 1997; 41: 409-13. 16.Nath K, Gogi R, Zaidi N, lohri A. Cystic lesions of conjunctiva (a clinicopathological study). Indian J Ophthalmol 1983; 31: 1-4. 17 Khurana S, Biswal M, Bhatti HS, et al. Ophthalmomyiasis: three cases from North India. Indian) J Med Microbial 2010; 28: 257-61.