Orbital tumors

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 to inspection, palpation, and biopsy. Orbital ultrasonography can be performed quickly in many ophthalmologists’ offices and can sometimes provide useful diagnostic information in cases of anterior orbital tumors. As mentioned earlier, CT and MRI have revolutionized orbital tumor diagnosis in children and have greatly improved the management of such cases.

Therapeutic approaches

The treatment of an ocular tumor in a child also depends on the location of the tumor and the size of the lesion.

Eyelid and conjunctiva

using chemoreduction to reduce the tumor(s) to a small size so that enucleation and irradiation can be avoided.13

Orbital tumors

The treatment of an orbital tumor varies greatly with the clinical or histopathologic diagnosis. Benign vascular tumors, such as capillary hemangioma and lymphangioma, can be managed by serial observation or patching treatment 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, may require limited biopsy to establish the diagnosis, followed by irradiation or chemotherapy.3,5

Eyelid tumors

There are many pediatric cutaneous tumors that can affect the skin of the eyelids.3,14 Only the

more important tumors will be considered here.

272

272 Cutaneous hemangioma in twin #2 on the hand.

273

273 Extensive capillary hemangioma of the eyelid,facial skin,and orbit before treatment with systemic corticosteroids.

274

274 Large capillary hemangioma of the eyelid obstructing vision,before treatment.

275

275 The capillary hemangioma in 264 after

2 months of oral corticosteroids.Note the tumor reduction and facial weight gain.

Facial nevus flammeus

Facial nevus flammeus is a congenital cutaneous vascular lesion that occurs in the distribution of the fifth cranial nerve (276). It may be an isolated entity or it may occur with variations of the SW syndrome. Infants with this lesion have a higher incidence of ipsilateral glaucoma, diffuse choroidal hemangioma, and secondary retinal detachment. Affected infants should be referred to an ophthalmologist as early as possible in order to diagnosis and treat these serious ocular conditions. Management of the cutaneous lesion includes observation, cosmetic make-up, or laser treatment.

Kaposi’s sarcoma

Opportunistic neoplasms such as Kaposi’s sarcoma can be found in immunosuppressed children, particularly those with acquired immune deficiency syndrome (AIDS). Although the affected patient can display red cutaneous lesions elsewhere, the eyelid can occasionally be the initial site of involvement. The lesion appears as a reddish-blue subcutaneous mass near the eyelid margin. It generally responds best to chemotherapy and radiotherapy.

Basal cell carcinoma

in younger patients, particularly if there is a family history of the basal cell carcinoma syndrome. It generally occurs on the lower eyelid as a slowly progressive mass that frequently develops a central ulcer (rodent ulcer). Lesions near the eyelid margin often develop loss of eyelashes in the area of involvement. Treatment is local excision using frozen section control and eyelid reconstruction.

Taylor et al. reviewed 39 patients with basal cell carcinoma syndrome (Gorlin–Goltz syndrome) and found the age of presentation to be between 5 and 72 years.16 The presenting clinical features included odontogenic keratocyst (n=17 patients), basal cell carcinoma (n=13), and congenital malformations (n=2). Seventeen of the 39 patients confirmed a family history of the syndrome in a parent. Basal cell carcinoma developed in 18 of 28

(64%) patients before the age of 30 years. Thus this syndrome should be explored in any child with basal cell carcinoma.

Melanocytic nevus

A melanocytic nevus is a tumor composed of benign melanocytes. It can occur on the eyelid as a variably-pigmented well-circumscribed lesion, identical to those that occur elsewhere on the skin. It does not usually cause loss of cilia. In some instances, the nevus is congenital and large and involves both the upper and lower eyelids and is termed ‘kissing nevus’ or ‘divided nevus’. There is some evidence that early intervention with curettage of the lesion within the first 3–4 weeks after birth can successfully remove the lesion without the need for extensive grafting. The nevus at an early stage is superficial and can be scraped off the superficial skin, whereas its involvement deepens into the subcutaneous tissue over time, making surgical removal more difficult. Following curettage, the affected infant is treated with topical antibiotic ointment and the skin heals by granulation.

The blue nevus is often apparent at birth, whereas the junctional or compound nevus may not become clinically apparent until puberty. Transformation into malignant melanoma is rare and usually occurs later in life. Although most eyelid nevi in children can be safely observed, they are occasionally excised because of cosmetic considerations or because of fear of malignant transformation.

Neurofibroma

A neurofibroma can occur on the eyelid as a diffuse or plexiform lesion that is often associated with von Recklinghausen’s neurofibromatosis. In the earliest stages the lesion produces a characteristic S-shaped curve to the upper lid. Larger lesions produce thickening of the eyelid with secondary blepharoptosis (277). Since these diffuse tumors are often difficult or impossible to completely excise, they should be managed by periodic observation or surgical debulking if they cause a major cosmetic problem.17