Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

Fig. 9.7: Ocular melanocytosis.

skin discolouration, in V1 and V2 trigeminal distribution.

c.Patients have an increased risk of developing malignant choroidal melanoma and, rarely, primary orbital melanoma, so arrange yearly dilated fundus examination.

■Vascular tumours:

1.Pyogenic granuloma:

a.Benign fibrovascular tumour.

b.Neither an infection nor a granuloma.

c.Fleshy, thickened, red/pink-coloured lesions occurring near previous surgical sites.

d. May respond to topical steroid therapy but may require local surgical excision with careful wound closure. An example is shown in Figure 1.27; (p. 38).

2.Capillary haemangioma: a. Benign.

b. Circumscribed, red lesion found in infants.

c.May enlarge over time, but usually undergoes spontaneous involution.

d. Typically managed by observation, but local excision or intralesional steroid treatment may be used.

e.For orbital and skin involvement, see pages 34 and 92.

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Conjunctival tumours

3.Kaposi’s sarcoma: a. Malignant.

b. Bright red, solitary or multifocal mass associated with haemorrhage.

c.May involve the eyelid skin. An example is shown in Figure 1.36.

d. A characteristic lesion in AIDS.

e.Treatment options include observation, low-dose radiotherapy, and excision. Intralesional interferon

alpha2a chemotherapy is described. Generalized disease requires chemotherapy ± radiotherapy.

4.Orbital varix and lymphangioma. See pages 93 and 94.

■Lymphoid tumours:

1.Lymphoma (Fig. 9.8):

a.Malignant.

b. Fleshy lesions with deep pink ‘salmon patch’ appearance.

c.Most are extranodal marginal zone B-cell lymphomas of MALT type that develop de novo in conjunctiva and in which bilateral presentation and orbital involvement are common. Some develop in association with systemic lymphoma.

d. Diagnosed by incisional biopsy followed by systemic evaluation.

e.Disease localized to the conjunctiva and orbit is usually treated by external beam radiotherapy and the prognosis for the eye, for vision, and for life is generally good.

Investigations Baseline and serial photographs are useful to detect or monitor change. Excisional or incisional biopsy is performed to make a histological diagnosis. Orbital CT may be needed to determine tumour extension.

Treatment

■Surgical excision : may be part of the initial biopsy. Generally, wide surgical margins are preferred with primary closure of large defects. Techniques such as absolute alcohol epithelial debridement and lamellar corneoscleral–conjunctival resection may be used.

■Surgical debulking : is needed when, in established cases, tumour overgrowth limits the use of adjuvant treatments such as topical agents or cryotherapy, and for symptomatic relief.

■Cryotherapy : with a double or triple freeze-thaw technique is an important tool for local tumour control after biopsy.

■Adjuvant treatments : such as plaque radiotherapy, topical anticancer agents (interferon alpha or mitomycin C), and external beam radiotherapy may be used in selected cases.

■Orbital exenteration : is reserved for extensive invasion by malignant tumours such as SCC or MM.

Follow–up After diagnosis and treatment, premalignant and malignant conjunctival tumours require close and thorough followup every 3–4 months to exclude progression or recurrence.

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Iris melanoma

Iris Melanoma

Background Iris melanomas account for 5–10% of all uveal tract melanomas. They are usually well differentiated and rarely metastasize. The mortality rate is around 5%, much lower than for ciliary body or choroidal melanomas.

Symptoms Visual changes occur with larger tumours that grow into the visual axis or induce a central cataract. There may be pain from elevated IOP associated with tumour pigment shedding, direct angle invasion, and anterior segment neovascularization. Tumours may also be asymptomatic.

Signs Iris melanomas are raised, replace the iris stroma, have variable pigmentation (though classically dark brown), and have visible intrinsic tumour vessels (Fig. 9.9). They usually arise from the pupillary margin, causing a peaked pupil or uveal ectropion. Focal cataract may be seen. Focal conjunctival and episcleral vascular dilation (sentinel vessels) are seen in the same quadrant as larger, more chronic malignant iris masses. Extraocular extension of a pigmented tumour through the sclera may occur.

History and examination Ask about visual symptoms, ocular surgery, cancer, or constitutional symptoms. Detailed ocular examination includes IOP, pre-and postdilation gonioscopy to determine angle and/or anterior ciliary body involvement, and dilated fundal examination. Transpupillary transillumination is used to determine the posterior extension of an iris tumour into the ciliary body.

Fig. 9.10: Iris naevus.

Differential diagnosis

■Hyperpigmented lesions: 1. Iris naevus (Fig. 9.10): a. Asymptomatic.

b. Generally flat (<1 mm height) and pigmented.

c.Sporadic, often present after the second decade, and rarely grow.

d. Iris infiltration, sectorial cataract, or pupil distortion increase the suspicion of malignancy.

e.Small, focal iris freckles may be associated with neurofibromatosis type I and the iridocorneal– endothelial (ICE or ‘Cogan-Reese’) syndrome. Review stable lesions yearly, but more frequently if suspicious.

■Hypopigmented lesions:

1.Iris metastasis:

a.Pinkor yellow-coloured, rapidly growing, unior multifocal lesion.

b.Associated with pseudohypopyon, hyphaema, and elevated IOP.

c.Many patients have a history of cancer (e.g. breast, lung), risk factors (e.g. smoking history, family

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Iris melanoma

d. Treat with chemotherapy for systemic tumour and external beam radiotherapy for the iris tumour.

2.Iris cyst:

a. Cysts have an absence of internal reflectivity on ultrasound.

3.Inflammatory nodules:

a. Koeppe (peripupillary) and Busacca (mid-iris) nodules are small grey/yellow nodules seen in granulomatous uveitis along with other signs of inflammation.

Investigations Colour photography for documentation and follow-up. Ultrasonography (A- and B-scan) to document size and internal reflectivity (usually low to moderate). Rarely, fluorescein angiography is needed. Incisional iris biopsy or fine needle biopsy may confirm the diagnosis prior to definitive treatment.

Treatment Treat elevated IOP but refractory glaucoma may indicate extensive ring involvement of the drainage angle by tumour that is better treated by enucleation. Occasionally, lesions may be observed if very small or if atypical/benign appearance. Local treatments such as tumour resection by sector iridectomy or combined iridocyclectomy for combined iris and localized ciliary body tumours may be performed with tumour-free margins. Primary radiation therapy with plaque (typically with the radioisotope ruthenium106 in the UK) can be used for larger tumours unsuitable for local resection. Enucleation is reserved for:

■Large iris masses infiltrating the angle and causing secondary glaucoma.

■Diffuse (‘ring’) iris melanomas.

■After failed local therapy.

■Blind, painful eye from melanoma-induced glaucoma.

Follow–up Examine 4–6 monthly for tumour recurrence, including gonioscopy. Generally, there is a low metastatic potential.

Differential Diagnosis of

Fundus Tumours

Choroidal naevus Typically asymptomatic, flat (<2 mm height), variably pigmented lesions with regular margins. Risk factors for transformation into a malignant choroidal melanoma include documented growth, height >2.0 mm, presence of orange pigment (lipofuscin), posterior location, and/or visual symptoms from macular involvement or associated serous retinal detachment (Fig. 9.11). Naevi with a low risk of malignant transformation are typically small (less than 6 mm basal dimension), flat (less than

1 mm height), and associated with drusen or RPE hyperpigmentation. Intermediate-risk naevi may be >2.0 mm in height and contain lipofuscin pigment granules. High-risk naevi have multiple risk factors, particularly documented growth and visual symptoms.

Photograph low-risk naevi for comparison and arrange annual review by an ophthalmologist or optometrist. Photograph intermediate-risk naevi and review every 4–6 months by an ophthalmologist for at least 1 year, then yearly. Promptly refer progressing, intermediateand high-risk naevi to an ocular oncology centre.

Choroidal melanoma See page 404.

Congenital hypertrophy of the RPE

(CHRPE) Asymptomatic, flat, dark black lesions with regular margins (Fig. 9.12). Usually unilateral, unifocal, and located in the peripheral retina. Solitary peripheral lesions require no follow-up. Central CHRPE may be followed serially to confirm that there is no growth, then discharged. Patients with adenomatous intestinal polyposis commonly have multiple bilateral CHRPE, so exclude a family history of bowel disease.

Subretinal haemorrhage Peripheral, domed, disciform, subretinal haemorrhage may resemble choroidal melanoma due to the variable pigmentation of blood elements (Fig. 9.13). Commonly caused by subretinal choroidal neovascularization (agerelated, high myopia, inflammatory, post-traumatic) or retinal artery macroaneurysms. Subretinal haemorrhage resolves over time with reduction in the height of the lesion, unlike malignant tumours.

Choroidal detachment Particularly in hypotonous eyes or less commonly due to scleritis, carotid cavernous fistula, lymphoma, or uveal effusion syndrome (see Fig. 11.10). B-scan ultrasound is helpful if the diagnosis is uncertain.

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Differential diagnosis of fundus tumours

A

B

Fig. 9.11: High-risk (A) and low-risk (B) choroidal naevi.

Differential diagnosis of fundus tumours

Fig. 9.14: Retinal capillary haemangioma.