VII.TUMORS OF THE CONJUNCTIVA

Tumors of the conjunctiva may develop from epithielial cells, melanocytes (see above), lymphoid and mesenchymal tissues. Epithelial tumors are very rare in children.

Capillary hemangioma

Conjunctival capillary hemangioma is very rare in children. Usually they are associated with lid, cutaneous or orbital hemangiomas which are very common in infancy and small children. It is hamartomatous proliferation of primitive mesenchymal tissues and is composed of endothelial-lined, anastomosing, immature blood vessels filled with blood and separated by fibrous septa. Conjunctival lesions involve the palpebral and fornical conjunctiva and are very rarely seen in bulbar area. Often there are continous with orbital or lid or even intracranial lesions. They are small and do not cover visual axis. Usually capillary hemangiomas appear in the age of 2-5 months and increase in size during first year of life and afterwards they regress spontaneously. Treatment of conjunctival lesions is not necessary but sometimes it is indicated to treat associated lid or orbital lesions (with intralesional steroid injections, systemic steroids, radiotherapy, systemic interferon, laser or surgical excision) if their size interfere with the function of the eye. Removal is often followed by recurrences. (32)

Lymphangioma

As capillary hemangioma also lymphangioma is very rare in children. It is also hamartomatous proliferation of vascular tissue and is composed of lymph-like channels lined of endothelial cells. Usually they appear in the first decade of life. They manifest as white, round, elevated, subconjunctival masses with clear fluid filled cystic spaces.(32, 35) Sometimes blood can be seen within the lymphatic spaces. Lymphangiomas can regress spontaneously. They also respond to corticosteroid therapy. Other form of therapy are cryotherapy or external beam radiation. Surgical excision is rarely indicated.

Lymphangioma should be differentiated with dilated lymphatics - lymphangiectasia. (36) It occurs usually in the interpalpebral area and is caused by the obstruction of lymphatic drainage vessels by conjunctival anomalies like scars, tumors (neurofibromatosis) and in Turner syndrome. (36) They are seen as translucent dilated channels under the conjunctiva and they can be easily overlooked because of intensive white color of underlying sclera. (Fig. 32) Diffuse form was also described clinically resembling chemosis.(36) Because lymphatics have a connection with blood vessels they can be filled (usually intermittently) with blood. (Fig. 33)

Fig. 32 Subconjunctival lymphangiectasias in patients with neurofibromatosis

Fig. 33 Subconjunctival lymphangiectasias with lymph vessels filled with blood

Leukemic infiltration of the conjunctiva

Leukemic infiltrations are usually located in the fornices as a pale or pink subepithelial unilateral masses. Less frequently they can be bilateral or seen as a diffuse infiltrations. In most cases they are late signs of leukemia but rarely they can be the first manifestation of these diseases. Conjuntival leukemic infiltrations regress after chemotherapy. Radiation therapy is rarely indicated, usually in chemotherapy-resistant cases. (37)

Choristomas

They are common congenital lesions with little growth potential composed of dermal and epithelial elements that are not normally found in the conjunctiva. Conjunctival choristomas can be divided to solid limbal dermoids, dermolipomas and complex choristomas.

Solid limbal dermoids are dense, round, elevated, smooth, yellow, unilateral tumors located at the inferotemporal limbus. Most of them are superficial but some can penetrate deeply into the sclera or cornea. Inside dermoids contain thick, gelatinous masses with hair, sweat glands, fat, sebaceous glands and sometimes teeth. They can be associated with eyelid colobomas. Surgical excision is usually performed because of cosmetic reasons. In deep lesions complete excision may require peripheral keratoplasty. (31)

Dermolipomas are smooth, yellow, elevated tumors occurring typically on the temporal bulbar conjunctiva. (Fig. 34) They may be isolated lesions and are usually unilateral or may be part of Goldenhar’s syndrome (oculoauriculovertebral dysplasia) (Fig. 35) where they are often bilateral. Dermolipomas can extend posteriorly to the orbit between superior and lateral rectus muscles. Surgical excision is indicated because of cosmetic reasons or when the tumor restricts eye movements. Usually anterior part of the tumor is excised because deep penetration into the orbit may damage rectus muscles, lacrimal gland or levator palpebrae muscle.

Fig. 34 Dermolipoma penetrating into the orbit

Fig. 35 Dermolipoma in a child with Goldenhar syndrome

Complex choristomas clinically resemble dermoids or dermolipomas but often have more red and vascularized appearance. They consists of ectopic tissues such as acinar glands, cartilages, adipose tissue and smooth muscles. They can penetrate deeply into sclera or cornea. Some growth of the tumor may be seen during puberty. Surgery is usually not indicated because of deep penetration into the globe. (31)

  1. OTHER CONJUNCTIVAL LESIONS

Pingueculum and pterygium

Pingueculae and pterygia are rare in children. They can found in children living in hot and dry countries. More frequent are pseudopterygia developing after corneal and conjunctival inflammatory diseases or excision of corneal lesions (dermoid).

Surgery is these lesions is rarely necessary in children. It is usually performed because of cosmetic reasons.

Papilloma

Conjunctival papillomas is common in children. They are caused by human papillomavirus. (38) The infection usualy manifests as follicular conjunctivitis but occasionally tumor-like lesions with papillomatous surface and typical vascular pattern (hypervascularity) may develop. (Fig. 36, 37) The lesions be removed surgically, they tend to be recurrent in children. Cryotherapy, CO2 laser, diathermy and interferon therapy have been tried to prevent recurrences. (39)

Fig. 36 Inner canthus conjunctival papilloma

Fig. 37 Exuberant papilloma involving whole bulbar conjunctiva

Xeroderma pigmentosum

Xeroderma pigmentosum is a group of autosomal recessive diseases in which light induced damage to nuclear DNA cannot be repaired through the normal endonuclease-initiated multistep process. It causes excessive skin sensitivity to sunlight (most noticeably in exposed areas) resulting in widespread skin pigmentation, telangiectasis, keratosis, and development of basal cell and squamous cell carcinomas, malignant melanomas and other tumors. In 20% of patients may have neurological abnormalities including mental retardation, microcephaly, spasticity, ataxia, cerebellar atrophy and deafness. The most common conjunctival complications are conjunctivitis, conjunctival melanosis and dry eye syndrome. Less frequent lesions include squamous cell carcinoma, melanoma and angiosarcoma of the conjunctiva.

Management of xeroderma pigmentosum consist of protection from ultraviolet light and early excision of suspicious tumors. Survival is reduced because of possibility of malignant neoplasms development. (31, 32)

Epithelial cysts

Epithelial inclusion cysts may appear in older children and can be found both in bulbar and palpebral conjunctiva. They are lined with nonkeratinized, squamous epithelium and filled with clear fluid. Usually they are symptom free and may disappear spontaneously. Treatment consists of surgical excision and is performed mainly because of concern of parents. Sometimes cysts can recur after removal.

Avitaminosis A

The disease is caused by vitamin A deficiency as well as a more generalized malnutrition. The disease is even nowadays one of the leading causes of childhood blindness worldwide.Main ocular manifestations include nyctalopia (night blindness), keratinization of corneal epithelium (xerophthalmia), pannus and progressive corneal opacification. In severe cases corneal ulcers and scars with keratomalacia (corneal necrosis) can develop. Typical conjunctival signs of avitaminosis A are Bitot’s spots. They are foamy, elevated patches of the temporal, exposed areas of the conjunctiva. They consists of inflammatory cells and corynebacterium xerosis. Other conjunctival changes include keratinization and thickening of the conjunctival epithelium and dry eye syndrome.

The treatment is protein, caloric and vitamin A replacement. (40)

  1. CONJUNCTIVAL MANIFESTATIONS OF SYSTEMIC DISEASES

Comparing with other parts of the eye conjunctiva is rarely involved in systemic diseases. Conjunctival manifestations of systemic diseases are grouped in table XI

TABLE XI. CONJUNCTIVAL MANIFESTATIONS OF SYSTEMIC DISEASES

Disorder / Conjuntival manifestation / Disorder / Conjuntival manifestation
Alkaptonuria / “Oil droplet” epithelial limbal opacities, pigmentation near horizontal rectus muscles, pigmented pingueculae, pigmented granules in episclera / Leptospirosis / Conjunctivitis
Amyloidosis / Yellow-white, waxy, flat, nontender, subconjunctival masses, usually in the inferior fornix / Measles / Purulent conjunctivits and keratitis
Ataxia telangiectasia (Louis-Bar syndrome) / Tortuous and telangiectatic conjunctival vessels / Mucolipidosis I / Conjunctival vessels tortuosity
Avitaminosis A / Thickening and keratinization of conjunctival epithelium, dry eye syndrome, Bitot’s spots, pannus / Riley-Day syndrome / Tear deficiency
Cat scratch disease
(Parinaud’s oculoglandular syndrome) / Unilateral follicular conjunctivitis / Reiter syndrome / Conjunctivitis
Cystynosis / Conjunctival cystine crystal depositions (needle-shaped, refractile, polychromatic) / Richnert-Hanhart syndrome / Conjunctival thickening
Erythema multiforne, Stevens-Johnson syndrome and TEN / Mucopurulent conjunctivitis with papillary reaction of the tarsal conjunctiva, erythematous and edematous macules and papules, vesicles, bullae, pseudomembranes and membranes formation, conjunctival scarring See above / Ring D chromosome / Epicanthal folds
Fabry disease / Conjunctival vessels tortuosity / Sarcoidosis / Conjunctivitis, conjunctival granulomas, symblepharon
Gaucher disease / Prominent pingueculae / Sturge-Weber syndrome / Tortuous, dilated conjunctival and episcleral vessels
Goldenberg syndrome / Conjunctival teleangiectasie / Trisomia 18 / Epicanthal folds
Herpetic infections (HSV 1 and 2, VZV) / Conjunctivitis with corneal involvement. See above / Turner syndrome / Epicanthal folds
Hyperlipoptoteinemia / Conjunctival xanthomata / 13 q deletion / Epicanthal folds
Kawasaki syndrome / Conjunctivitis / 18 q deletion / Epicanthal folds
Klippel-Trenaunay-Weber syndrome / Conjunctival angiomas / 18 r deletion / Epicanthal folds

Literature

  1. Taylor P, Talbot EM: Perilimbal vascular anomaly associated with ipsilateral en coup de sabre morphea. Br J Ophthalmol, 195, 69: 60-62.
  2. Rapoza PA, Quinn TC, Kiessling LA, Taylor HR: Epidemiology of neonatal conjunctivitis. Ophthalmology, 1986, 93: 456-461.
  3. Isenberg SJ, Apt L, Wood M: A controlled trial of povidone-iodine as prophylaxis against ophthamia neonatorum. N Eng J Med, 1995, 332: 562-566.
  4. Bell TA, Grayston, JT, Krohn MA: Randomized trial of silver nitrate, erythromycin, and no eye prophylaxis for the prevention of conjunctivitis of among newborns not at risk for gonococcal ophthalmitis. Pediatrics, 1993, 92: 755-760.
  5. O’Hara, MA: Ophthalmia neonatorum. Pediatr Ophthalmol, 1993, 40: 715-725.
  6. AmericanAcademy of Ophthalmology, Conjunctivitis, Preferred Practice Pattern, AmericanAcademy of Ophthalmology, 2003
  1. Whitley RJ, Nahmias AJ, Visitine AM et al: The natural history of herpes virus infection of mother and newborn. Pediatrics, 1980, 66: 489-494.
  2. Rubestein JB, Jick SL: Disorders of the conjunctiva and limbus. In: Yanoff M, Duker J.S: Ophthalmology, St.Louis, 2004.
  3. Gigliotti F, Williams WT, Hatden FG,et al.: Etiology of acute conjunctivitis in children. J Pediatrics, 1981, 98:531-536.
  4. Prost M, Semczuk K.: Antibiotic resistance of conjunctival bacterial flora in children.Klin Oczna 2005, 107: 418-420.
  5. Petricek I, Prost M, Popova A:The differential diagnosis of red eye: A survey of medical practitioners from Eastern Europe and the Middle East. Ophthalmologica 206, 220: 229-237.
  6. Weiss A, Brinsor J.H, Nazar-Stewart V: Acute conjunctivitis in childhood. J Pediatr, 1993; 122: 10-14.
  7. Wagner RS, DeRespinis PA: Pediatric conjunctivitis. Clinical Signs in Ophthalmology, 1996,17: 2-11.
  8. Weiss A. Acute conjunctivitis in childhood: Current Problems in Pediatrics, 1994, 24: 4-11.
  9. Haimovici R, Russel, TJ: Treatment of gonococcal conjunctivitis with single dose intermuscular Ceftriaxone. Am J Ophthalmol, 1989, 107: 511-514.
  10. Lichtenstein SJ, Dorfman M, Kennedy R, Stroman D: Controlling contagious bacterial conjunctivitis. J Ped Ophthalmol Strab, 2006, 43: 19-26.
  11. Block SL, Hedrick J, Tyler R et al: Increasing bacterial resistance in pediatric acute conjunctivitis (1997-1998). Antimicrob Agents Chemother, 2000, 44: 16650-1654.
  12. Sundmacher R.: Infektiöse keratokonjunktivitis. Wie lange muss ich krankschreiben. Medical Tribune, 1980, 39, 68.
  13. Hammer, lh, Perry HD, Donnenfeld ED et al: Symblepharon formation in epidemic keratoconjunctivitis. Corneaz, 1990, 9: 338-340.
  14. Yin-Murphy M: Viruses of acute hemorrhagic conjunctivitis. Lancet, 1973, 1: 545-546.
  15. Hoh HB, Hurley C, Claoue C, Viswalingham M, Easty DL, Goldschmidt P, Collum LM: Randomised trial of ganciclovir and acyclovir in the treatment of herpes simplex dendritic keratitis: a multicentre study. Brit J Ophthalmol, 1996, 80: 140-143.
  16. Thylefors, B, Dawson CR, Jones BR et al.: Simple system for the assessment of trachoma and its complications. Bull World Health Organ, 1987, 65:477-483.
  17. Dawson CR, JonesBR, Tarizzo M: Guide to trachoma control. Geneva: World Health Organization, 1980, p. 56.
  18. Dawson CR, Juster R, Marx R et al: Limbal disease in trachoma and other ocular chlamydial infections: risk factors for corneal neovascularization. Eye, 1989, 3: 204-209.
  19. Schachter J, WestSK, Mabey MD: Azithromycin in control of trachoma. Lancet, 1999, 354: 630-635.
  20. Chen S, Wishart M, Hiscott P: Ligneous conjunctivitis: a local manifestation of systemic disorder? J AAPOS, 2000, 4:313 – 315.
  21. WattsP, SureshP, MezerE, et al.: Effective treatment of ligneous conjunctivitis with topical plasminogen. Am J Ophthalmol. 2002, 133: 451-455.
  22. Yetiv, JZ, Biachine JR, Owen JA: Etiologic factors of the Stevens-Johnson syndrome. South Med J, 1980:, 73: 599-602.
  23. Goyal R, Jones SM, M, Espinosa, Green V, Nishal K: Amniotic membrane transplantation in children with symblepharon and massive pannus. Arch Ophthalmol, 2006, 124: 1435-1440.
  24. Tsubota Shimmura S, Shinozaki N et al: Clinical applications of living-related conjunctival-limbal allografts. Am J Ophthalmol, 2002, 133: 134-135.
  25. Augsburger JJ, Schneider S: Tumors of conjunctiva and cornea. In: Yanoff M, Duker J.S: Ophthalmology, St.Louis, 2004.
  26. Calvo, EA, Haik BG, Karcioglu, ZA, Ainbinder DJ: Tumors of the conjunctiva. In: Wright KW: Pediatric ophthalmology and strabismus. Mosby, St Louis, 1995.
  27. Gloor P, Alexandrakis G: Clinical characterization of primary acquired melanosis. Invest Ophthalmol Vis Sci, 1995, 36: 1721-1729.
  28. McDonnel JM, Carpenter JD, Jacobs P et al: Conjunctival melanocytic lesions in children. Ophthalmology, 1989, 96: 986-993.
  29. Iliff, WJ, Green WR: Orbital lymphangiomas. Ophthalmology, 1979, 86: 914-929.
  30. Yanoff M. Fine B: Conjunctival lymphangiectasias. In Ocular pathology. Harper & Row, Hagerstown, 1975.
  31. Kincaid MC, Green WR: Ocular and orbital involvement in leukemia. Surv Ophthalmol, 1983, 27: 211-232.
  32. Lass, JH, GroveAS, Papale JJ, et al: Detection of human papillomavirus DNA sequences in conjunctival papillomas. Am J Ophthalmol, 1983, 96: 670-671.
  33. Lass, JH, Foster CS, GroveAS et al: Interferon-alpha therapy in recurrent conjunctival papillomas. Am J Ophthalmol, 1987, 103: 294-301.
  34. Gujral S, Abbi R, Giopaldas T: Xerophthalmia, vitamin A supplementation and morbidity in children. J Tropic Pediat, 1993, 39: 89-92.

Flow chart I. Stepwise differential diagnosis of “red eye”