Eyelid and conjunctival tumors are generally more evident than intraocular or orbital tumors, prompting an early visit to a physician. Most tumors in the eyelid or conjunctival region have characteristic features, so an accurate diagnosis can generally be made with inspection. However, additional diagnostic studies of optical coherence tomography (OCT) or magnetic resonance imaging (MRI) can be helpful.

Intraocular tumors are often hidden until the patient is visually symptomatic. Young children often do not complain of visual loss and visual acuity can be difficult to assess due to cooperation. However, several features should alert the pediatrician to consider the possibility of an intraocular tumor and prompt a timely referral, particularly the presence of leukocoria (white pupil), strabismus (crossed eye), or lack of visual response.

Leukocoria There are several causes of leukocoria in children (2,4,5,6,7) (Fig. 19.1). The more common causes include congenital cataract, retinal detachment from retinopathy of prematurity, persistent fetal vasculature (persistent hyperplastic primary vitreous), and Coats’s disease. Retinoblastoma is likely the most serious condition to cause leukocoria in children. Any child with leukocoria should be referred promptly to an ocular oncologist for diagnostic evaluation and management.

Strabismus Most children with strabismus do not have an intraocular tumor. However, about 30% of patients with retinoblastoma present initially with either esotropia or exotropia, due to the tumor location in the macular area which disrupts central fixation. It is important that a complete retinal examination with indirect ophthalmoscopy be performed to exclude an underlying tumor in these cases.

Visual Impairment An older child with an intraocular tumor might complain of visual impairment or could be found to have decreased vision on visual screening in school. This can occur from reduction of central retinal function by a tumor or by the presence of vitreous hemorrhage, hyphema, or secondary cataract formation.

Unlike tumors of the eyelid and conjunctiva, orbital tumors are not directly visualized by the physician. Therefore, these tumors can attain a large size before becoming clinically evident. These patients generally present with proptosis or displacement of the eye (3). Pain, diplopia, and conjunctival edema can also be early clinical features of an orbital tumor. Computed tomography (CT) and MRI have revolutionized the diagnosis and treatment of orbital tumors (8). Caution is issued for use of CT in children to reduce radiation exposure (9).

Most eyelid and conjunctival tumors are recognized by their classic clinical features. Diagnostic studies can provide additional help. Smaller suspicious tumors can be removed by excisional biopsy and larger tumors are best diagnosed by incisional biopsy and definitive treatment withheld until a definite diagnosis is established.

Concerning intraocular tumors, lesions of the iris can often be recognized with external ocular examination or slit-lamp biomicroscopy. Tumors of the retina and choroid can be visualized with ophthalmoscopy, which can reveal typical features depending on the type of tumor. Many small tumors are difficult to visualize and may only be detected by an experienced ophthalmologist using binocular indirect ophthalmoscopy. Ancillary studies such as fundus photography, autofluorescence, OCT, fluorescein angiography, indocyanine green angiography, ocular ultrasonography, and occasionally CT or MRI are of supplemental value in establishing the diagnosis. OCT is a relatively newer fundus scanning method using a rapid, noncontact, comfortable technique providing cross-sectional imaging of the retina to 4 to 5 µm resolution in a few minutes. Children comfortably tolerate this technique (10). OCT can provide in vivo, high-resolution information of the retina to the 10 µm level. Fine needle aspiration biopsy under general anesthesia has recently been employed in selected intraocular tumors of children (11).

Some orbital tumors occur in an anterior location and can be recognized by their extension into the conjunctiva and eyelid area. This is particularly true of childhood vascular tumors such as capillary hemangioma and lymphangioma. Other tumors reside in the deeper orbital tissues and are less accessible. Orbital ultrasonography can be performed in the office, but often more formal imaging with CT or MRI is necessary.

Neoplasms of the eyelid and conjunctiva can be removed surgically by a qualified ophthalmologist or ocular oncologist. Inflammatory lesions that simulate neoplasia can be managed by antibiotics or corticosteroids, depending on the diagnosis. Some malignant neoplasms such as leukemias and lymphomas are best managed with a limited diagnostic biopsy followed by irradiation and/or chemotherapy.

The management of intraocular tumors is more complex. Certain benign intraocular tumors that are asymptomatic are usually managed by serial observation. Some symptomatic benign tumors can be treated with laser or cryotherapy, depending on the mechanism of visual impairment. Malignant tumors, such as retinoblastoma, are managed with chemotherapy (intravenous chemotherapy, intra-arterial chemotherapy [IAC], sub-Tenon chemotherapy, or intravitreal chemotherapy approaches), radiotherapy (external beam or plaque radiotherapy), laser photocoagulation, thermotherapy, cryotherapy, or enucleation of the eye (2,4,12,13). More recently, there is a strong trend toward using methods of chemotherapy for retinoblastoma control (13,14).

The treatment of an orbital tumor varies greatly with the clinical signs of histopathologic diagnosis. Benign vascular tumors, such as capillary hemangioma and lymphangioma, can be managed by serial observation or amblyopia patching of the opposite eye to decrease the severity of associated amblyopia. Circumscribed tumors in the anterior orbit may be managed by excisional biopsy. Many malignant tumors, such as rhabdomyosarcoma and orbital leukemia, require limited biopsy to establish the diagnosis, followed by irradiation or chemotherapy (3,5).

A variety of tumors can be present at birth or become clinically apparent shortly after birth. Most of the lesions are choristomas, consisting of displaced tissue elements normally not found in these areas. A simple choristoma is comprised of one tissue element such as epithelium, whereas a complex choristoma represents variable combinations of ectopic tissues like bone, cartilage, and lacrimal gland.

Dermoid Conjunctival dermoid is a congenital wellcircumscribed yellow-white solid mass that involves the bulbar or limbal conjunctiva (25,26). It characteristically occurs inferotemporally and often this tumor has fine white hairs (Fig. 19.6). In rare cases, it can extend to the central cornea or be located in other quadrants on the bulbar surface. Most often dermoid straddles the limbus, but in rare cases it can be extensive and involves the full-thickness cornea, anterior chamber, and iris stroma. The more severe dermoids occur earlier in embryogenesis.

The conjunctival dermoid can occur as a solitary lesion or can be associated with Goldenhar’s syndrome. The patient should be evaluated for ipsilateral or bilateral preauricular skin appendages, hearing loss, eyelid coloboma, orbitoconjunctival dermolipoma, and cervical vertebral anomalies.

Histopathologically, the conjunctival dermoid is a simple choristomatous malformation that consists of dense fibrous tissue lined by conjunctival epithelium with deeper dermal elements including hair follicles and sebaceous glands. The management of an epibulbar dermoid includes observation if the lesion is small and visually asymptomatic. Anterior segment OCT can assist in judging depth of involvement. It is possible to excise the lesion for cosmetic reasons, but the remaining corneal scar can be cosmetically unacceptable. Larger or symptomatic dermoids can produce visual loss from astigmatism. These can be approached by lamellar keratosclerectomy with primary closure of overlying tissue if the defect is superficial, or closure using corneal graft if the defect is deep or of full thickness. The cosmetic appearance might improve, but the refractive and astigmatic error and visual acuity might not change. When the lesion involves the central cornea, a lamellar or penetrating keratoplasty is necessary and long-term amblyopia should be anticipated.

Dermolipoma Dermolipoma is believed to be congenital, but it classically remains asymptomatic for years and might not be detected until adulthood. This tumor tends to occur in the conjunctival fornix superotemporally and appears as a yellow, soft, fluctuant mass with fine white hairs on its surface (Fig. 19.7). It can extend into the orbital fat and onto the bulbar conjunctiva, sometimes reaching the limbus.

Dermolipoma has features similar to orbital fat on CT and MRI scans. Histopathologically, it is lined by conjunctival epithelium on its surface and the subepithelial tissue has variable quantities of collagenous connective tissue and adipose tissue. Pilosebaceous units and lacrimal gland tissue might be
present. The majority of dermolipomas require no treatment, but larger ones or those that are cosmetically unappealing can be managed by excision of the entire orbitoconjunctival lesion through a conjunctival forniceal approach or by removing the anterior portion of the lesion in a manner similar to that used to remove prolapsed orbital fat. Amniotic membrane graft might be necessary to repair the defect.

Epibulbar Osseous Choristoma Epibulbar osseous choristoma is a tumor comprised of mature bone, usually located in the bulbar conjunctiva superotemporally (3,27) (Fig. 19.8). It is believed to be congenital and typically remains undetected until palpated by the older patient. On ultrasonography or CT scanning, the mass demonstrates calcium. This tumor is usually managed by observation. Occasionally, a foreign body sensation necessitates excision of the mass using a conjunctival forniceal incision followed by dissection of the tumor to bare sclera.

Lacrimal Gland Choristoma Lacrimal gland choristoma is a congenital lesion, discovered in young children as an asymptomatic pink stromal mass, typically in the superotemporal or temporal portion of the conjunctiva. It is speculated that this lesion represents small sequestrations of the embryonic evagination of the lacrimal gland from the conjunctiva. The lacrimal gland choristoma can masquerade as a focus of inflammation due to its pink color. Rarely, this mass can be cystic due to ongoing secretions if there is no connection to the conjunctival surface. Excisional biopsy is usually performed to confirm the diagnosis.

Complex Choristoma The conjunctival dermoid and epibulbar osseous choristoma are simple choristomas as they contain one tissue type such as skin or bone. A complex choristoma contains a greater variety of tissue derived from two germ layers such as lacrimal tissue and cartilage. It is variable in its clinical appearance and can cover much of the epibulbar surface, or it may form a circumferential growth pattern around the limbus.

The complex choristoma has an association with the linear nevus sebaceous of Jadassohn (26) (Fig. 19.9). The nevus sebaceous of Jadassohn includes cutaneous features with sebaceous nevus in the facial region and neurologic features including seizures, mental retardation, arachnoidal cyst, and cerebral atrophy. The ophthalmic features of this syndrome include epibulbar complex choristoma and posterior scleral cartilage. The management of complex choristoma depends upon the extent of the lesion. Observation or wide local excision followed by mucous membrane graft reconstruction are options.

There are several benign and malignant tumors that can arise from the squamous epithelium of the conjunctiva.

Papilloma Squamous papilloma is a benign tumor, documented to be associated with human papillomavirus infection of the conjunctiva (28,29). This tumor can occur in both children and adults. It is speculated that the virus is acquired through transfer from the mother’s vagina to the newborn’s conjunctiva as the child passes through the mother’s birth canal. Papillomas appear as a pink fibrovascular frond of tissue arranged in a sessile or pedunculated configuration (Fig. 19.10). The numerous fine vascular channels ramify through the stroma beneath the epithelial surface of the lesion. In children, the lesion is usually small, multiple, and located in the inferior fornix. Histopathologically, the lesion shows numerous vascularized papillary fronds lined by acanthotic epithelium.

There are several treatment options for small sessile papillomas. Sometimes observation allows for slow spontaneous resolution of this tumor. Larger or more pedunculated
tumors can lead to foreign body sensation, chronic mucous production, hemorrhagic tears, incomplete eyelid closure, and poor cosmetic appearance, requiring therapeutic intervention. Complete removal of the mass without direction manipulation of the tumor (no touch technique) is advisable to avoid spreading of the virus (30). Double freeze-thaw cryotherapy is applied to the remaining conjunctiva around the excised lesion in order to prevent tumor recurrence. In some instances, the pedunculated tumor is frozen alone and then excised while frozen. Topical or injection interferon and Mitomycin C have been employed for resistant or multiply recurrent conjunctival papillomas (31,32). For difficult recurrent lesions, oral cimetidine for several months following surgical resection can minimize recurrence by boosting the patient’s immune system and suppressing the virally stimulated mass (33).

Hereditary Benign Intraepithelial Dyskeratosis Hereditary benign intraepithelial dyskeratosis (HBID) is a rare, benign condition seen in an inbred isolate of Caucasian, African American, and Native Americans (Haliwa Indians), initially identified in North Carolina. It is an autosomal dominant disorder characterized by bilateral elevated fleshy plaques on the nasal or temporal perilimbal conjunctiva and on the buccal mucosa. It can remain asymptomatic or can cause redness and foreign body sensation. It is characterized histopathologically by acanthosis, dyskeratosis on the epithelial surface and deep within the epithelium, and prominent chronic inflammatory cells. HBID does not usually require aggressive treatment. Smaller, lesssymptomatic lesions can be treated with ocular lubricants and topical corticosteroids. Larger symptomatic lesions can be managed by local resection with mucous membrane grafting if necessary.

Squamous Cell Carcinoma/Conjunctival Intraepithelial Neoplasia Squamous cell carcinoma and conjunctival intraepithelial neoplasia (CIN) are malignancies of the surface epithelial cells. Intraepithelial neoplasia displays anaplastic cells within the epithelium, whereas squamous cell carcinoma displays extension of anaplastic cells through the basement membrane into the conjunctival stroma. Clinically, invasive squamous cell carcinoma is usually larger and more elevated than CIN. Leukoplakia can be seen with either condition.

Patients who are medically immunosuppressed from organ transplantation, those with HIV, or those with underlying DNA repair abnormalities like xeroderma pigmentosum are at particular risk to develop conjunctival squamous cell carcinoma and malignant melanoma (34). In these cases, the risk for life-threatening metastatic disease is greater.

The management of conjunctival squamous cell carcinoma varies with the extent of the lesion. Tumors in the limbal area require alcohol epitheliectomy for the corneal component and partial lamellar scleroconjunctivectomy with wide margins for the conjunctival component followed by freeze-thaw cryotherapy to the remaining adjacent bulbar conjunctiva. Extensive tumors or those tumors that are recurrent, especially with extensive corneal component, are treated with adjuvant topical Interferon, Mitomycin C, or 5-Fluorouracil (31,32,34,35).

There are several lesions that arise from the melanocytes of the conjunctiva and episclera. The most important ones include nevus, racial melanosis, primary acquired melanosis (PAM), and malignant melanoma (Table 19.3). Ocular melanocytosis should be included in this section as its scleral pigmentation can masquerade as conjunctival pigmentation.

Ocular Melanocytosis

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