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)
VIII. 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, CO 2 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)
IX. 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
Conjuntival
Disorder
Conjuntival
Disorder
manifestation
Alkaptonuria
Amyloidosis
Ataxia telangiectasia
(Louis-Bar syndrome)
manifestation
“Oil droplet” epithelial
limbal opacities,
pigmentation near
Leptospirosis
horizontal rectus
muscles, pigmented
pingueculae, pigmented
granules in episclera
Yellow-white, waxy,
flat, nontender,
Measles
subconjunctival
masses, usually in the
inferior fornix
Tortuous and
telangiectatic
conjunctival vessels
Conjunctivitis
Purulent conjunctivits
and keratitis
Conjunctival vessels
Mucolipidosis I
tortuosity
Avitaminosis A
Thickening and
keratinization of
conjunctival
epithelium, dry eye
syndrome, Bitot’s
spots, pannus
Riley-Day syndrome
Tear deficiency
Reiter syndrome
Conjunctivitis
Cat scratch disease
(Parinaud’s
Unilateral follicular
conjunctivitis
oculoglandular syndrome)
Cystynosis
Erythema multiforne,
Stevens-Johnson
syndrome and TEN
Conjunctival cystine
Richnert-Hanhart
crystal depositions
(needle-shaped,
syndrome
refractile,
polychromatic)
Mucopurulent
conjunctivitis with
papillary reaction of the
tarsal conjunctiva,
erythematous and
edematous macules
Ring D chromosome
and papules, vesicles,
bullae,
pseudomembranes and
membranes formation,
conjunctival scarring
See above
Conjunctival
thickening
Epicanthal folds
Conjunctivitis,
Conjunctival vessels
Fabry disease
conjunctival
Sarcoidosis
tortuosity
granulomas,
symblepharon
Tortuous, dilated
Sturge-Weber
Gaucher disease
Prominent pingueculae
conjunctival and
syndrome
episcleral vessels
Conjunctival
Goldenberg syndrome
Trisomia 18
Epicanthal folds
Turner syndrome
Epicanthal folds
teleangiectasie
Herpetic infections
(HSV 1 and 2, VZV)
Conjunctivitis with
corneal involvement.
See above
Conjunctival
13 q deletion
Epicanthal folds
18 q deletion
Epicanthal folds
Conjunctival angiomas 18 r deletion
Epicanthal folds
Hyperlipoptoteinemia
xanthomata
Kawasaki syndrome
Conjunctivitis
Klippel-TrenaunayWeber syndrome
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Flow chart I. Stepwise differential diagnosis of “red eye”
EYE ITCHING
YES
NO
ALLERGIC
CONJ.
EYE BURNING
YES
S
DRY EYE SYNDR.
NO
PURULENT STICKY DISCHARGE
NO
YES
BACTERIAL CONJUNCTIVITIS
VISION BLURRING
NO
YES
KERATITIS OR UVEITIS
TREAT AS APPRIOPRIATE
MECHANICAL OR CHEMICAL TRAUMA IN ANAMNESIS
YES
NO
TREAT AS APPRIOPRIATE
DIURATION MORE THAN 3 WEEKS
AND RECURRENT
NO
YES
CHLAMYDIAL CONJ
TREAT AS APPRIOPRIATE
ASSOCIATED UPPER RESPIRATORY
INFECTION, ENLARGED LYMPH HODES
NO
YES
VIRAL CONJUNCTIVITIS
TREAT AS APPRIOPRIATE
USED EYE MEDICATIONS
YES
TOXIC REACTION
WITHDRAW MEDICATIONS
NO
PALPEBRAL CHANGES E.G.
BLEPHARITIS, TRICHIASIS
YES
TREAT AS APPRIOPRIATE