Ординатура / Офтальмология / Английские материалы / Clinical Ophthalmology A Systematic Approach 7th Edition_Kanski, Bowling_2011

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Fig. 12.44 Retinal capillary haemangioma. (A) Histology shows capillary-like vascular channels between large foamy cells; (B) early tumour; (C) more advanced tumour associated with vascular dilatation and tortuosity; (D) optic nerve head lesion; (E) sessile lesion

(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A; B Damato – fig. C; J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. D; P Saine – fig. E)

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Fig. 12.45 FA of retinal capillary haemangioma. (A) Early filling; (B) late leakage

(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997)

Fig. 12.46 Severe exudation associated with a juxtapapillary capillary haemangioma

Treatment

1Observation is advised for asymptomatic juxtapapillary haemangiomas without exudation, because these may remain inactive for many years and because of the high risk of iatrogenic visual loss. Early peripheral lesions are not usually left untreated because they are relatively easy to ablate.

2Laser photocoagulation of small lesions. After closing the feeder vessels, the tumour is treated with low-energy, long duration burns. Multiple sessions may be needed.

3Cryotherapy for larger peripheral lesions or those with exudative retinal detachment. Vigorous treatment of a large lesion may cause a temporary but extensive exudative retinal detachment.

4 Brachytherapy for lesions too large for cryotherapy.

5Vitreoretinal surgery may be required for non-absorbing vitreous haemorrhage, epiretinal fibrosis or tractional retinal detachment. If appropriate, the tumour may be destroyed by endolaser photocoagulation or surgical removal.

6Other modalities include PDT, which avoids damage to adjacent tissues, and anti-vascular endothelial growth factor (VEGF) agents. These are worth considering with juxtapapillary tumours, which are otherwise virtually untreatable without visual loss.

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Von Hippel–Lindau syndrome

1Inheritance is AD condition caused by a mutation of the VHL gene on chromosome 3p26-p25.

2Clinical features

•CNS haemangioma involving the cerebellum (Fig. 12.47A), spinal cord, medulla or pons affects about 25% of patients with retinal tumours.

•Phaeochromocytoma.

•Renal carcinoma (Fig. 12.47B) and pancreatic islet cell carcinoma.

•Cysts of the testes, kidneys, ovaries, lungs, liver and pancreas.

•Polycythaemia which may be the result of factors released by a cerebellar or renal tumour.

2Screening is vital because it is impossible to predict which patients with retinal haemangiomas will harbour systemic lesions. The ophthalmologist must therefore refer all such patients for systemic and neurological evaluation. Relatives should also be screened because of the dominant inheritance pattern of the disease. The following screening protocol should be regularly performed in patients with established VHL and relatives at risk.

aAnnual screening

•Physical examination.

•Annual ophthalmoscopy from age 5 years, increased to 6-monthly from 10 to 30 years.

•Renal ultrasonography from age 16 years.

•Twenty-four hour urine collection for estimation of vanillyl mandelic acid and catecholamine levels from age 10 years to detect phaeochromocytoma.

bScreening every 2 years involves abdominal and brain MR from the age of 15 years.

cGenetic tests are indicated in all patients with suspected disease and in first and second degree relatives. With modern techniques the sensitivity is almost 100%.

Fig. 12.47 Tumours in von Hippel–Lindau syndrome. (A) Axial MRshows a cerebellar haemangioma; (B) axial CTof the abdomen shows a renal carcinoma

(Courtesy of CD Forbes and WF Jackson, from Atlas and Text of Clinical Medicine, Mosby 2003 – fig. B)

Cavernous haemangioma

Cavernous haemangioma of the retina and optic nerve head is a rare, unilateral, congenital hamartoma. It is usually sporadic but

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occasionally can be inherited as AD with incomplete penetrance, in combination with lesions of the skin and CNS (‘neurooculocutaneous phacomatosis’ or ‘cavernoma multiplex’).

1Histology shows multiple thin-walled dilated channels with surface gliosis.

2Presentation may be with vitreous haemorrhage or, more frequently, as a chance finding.

3Signs

•Sessile clusters of saccular aneurysms resembling a ‘bunch of grapes’ in the peripheral retina (Fig. 12.48A and B).

•Because of sluggish flow of blood, the red cells may sediment and separate from plasma, giving rise to ‘menisci’ or fluid levels within the lesion.

•The lesion occasionally involves the optic nerve head (Fig. 12.48C).

4 FA highlights the sedimentation of erythrocytes and shows delayed filling in the venous phase and lack of leakage (Fig. 12.48D).

5Complications, which are uncommon, include haemorrhage and epiretinal membrane formation.

6Treatment. Rarely vitrectomy may be necessary for non-absorbing vitreous haemorrhage but photocoagulation should be avoided as it may precipitate haemorrhage and enlargement of the tumour.

Fig. 12.48 Cavernous haemangioma. (A) Very small peripheral lesion; (B) larger peripheral lesion; (C) optic nerve involvement; (D) FA shows fluid levels due to separation of red cells (hypofluorescent) fromplasma (hyperfluorescent)

(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. D)

Racemose haemangioma

Racemose haemangioma (also known as arteriovenous malformation) of the retina and optic nerve head is a rare, sporadic, usually unilateral, congenital malformation involving direct communication between the arteries and veins without an intervening capillary bed. Some patients have similar ipsilateral lesions involving the midbrain, basofrontal region and posterior fossa (an association referred to as Wyburn –Mason syndrome). Brain involvement may lead to spontaneous haemorrhage or epilepsy. Occasionally, malformations may involve the maxilla and mandible, predisposing the patient to haemorrhage after dental treatment. Facial skin lesions have also been reported.

1Presentation is usually as a chance finding.

2Signs

•Enlarged, tortuous blood vessels which are often more numerous than normal with the vein and artery appearing similar (Fig. 12.49A).

•With time the vessels become more dilated and tortuous, and may become sclerotic (Fig. 12.49B).

3 FA shows hyperfluorescence but absence of leakage (Fig. 12.49C). 4 Treatment is not required.

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Fig. 12.49 Racemose haemangioma. (A) Vascular dilatation and tortuosity; (B) more severe lesion in which some vessels show sclerosis; (C) FA shows hyperfluorescence but absence of leakage

(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. B)

Vasoproliferative tumour

Retinal vasoproliferative tumour is a rare gliovascular lesion which can be primary or secondary to conditions such as intermediate uveitis, ocular trauma and retinitis pigmentosa. Secondary lesions may be multiple and occasionally bilateral depending on the underlying aetiology.

1Histology shows glial cells and a network of fine capillaries with some larger dilated vessels.

2 Presentation is usually in the 3rd–5th decades with blurring of vision due to macular exudation.

3Signs

•A reddish-yellow globular vascular mass, most frequently located in the inferotemporal periphery (Fig. 12.50).

•Retinal vessels may be seen entering the lesion posteriorly.

4Complications include subretinal exudation, exudative retinal detachment, macular oedema and fibrosis, and haemorrhage.

5Treatment with cryotherapy or brachytherapy induces regression of the tumour and exudation but the visual prognosis is guarded if there is maculopathy.

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Fig. 12.50 Vasoproliferative tumour with retinal detachment

(Courtesy of B Damato)

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Primary intraocular lymphoma

Overview

Lymphoma is a group of conditions characterized by neoplastic proliferation of cells of the immune system typified by lymphadenopathy, constitutional symptoms, and occasionally CNS involvement. The main classification and ocular manifestations are as follows:

1Hodgkin disease may cause anterior uveitis, vitritis, and multifocal fundus lesions resembling chorioretinitis.

2 Non-Hodgkin lymphoma may cause conjunctival involvement, orbital involvement, Mikulicz syndrome and uveal infiltration.

3CNS B-cell lymphoma may be associated with intermediate uveitis and sub-RPE infiltrates.

4Primary intraocular lymphoma (PIOL) represents a subset of primary central nervous system lymphoma (PCNSL), which is a variant of extranodal non-Hodgkin lymphoma. The lymphoma cells are large, pleomorphic B lymphocytes with large multilobular nuclei, prominent nucleoli and scanty cytoplasm (Fig. 12.51A). The tumour arises from within the brain, spinal cord and leptomeninges, and has a very poor prognosis. About 20% of patients with PCNSL have ocular manifestations, which can precede or follow neurological involvement. Most patients with PIOL develop CNS symptoms after a mean delay of 29 months.

Fig. 12.51 Primary intraocular lymphoma. (A) Vitreous biopsy shows cells with irregular large nuclei and scanty cytoplasm; (B) multifocal subretinal infiltrates; (C) coalescent subretinal infiltrates; (D) shallow retinal detachment

(Courtesy of P Smith – fig. A; B Damato – figs B and C; A Turno-Krecicka – fig. D)

Ocular features

1Presentation is in the 6th–7th decades with unilateral floaters, blurred vision, red eye or photophobia, which frequently becomes bilateral after a variable interval.

2Signs

•Mild anterior uveitis with cells, flare and keratic precipitates.

•Vitritis may impede visualization of the fundus.

•Large solid multifocal subretinal infiltrates (Fig. 12.51B).

•Occasionally coalescence of sub-RPE deposits may form a ring encircling the equator (Fig. 12.51C).

•Other features include retinal vasculitis, vascular occlusion, exudative retinal detachment (Fig. 12.51D) and optic atrophy.

•Lack of CMO is an important diagnostic clue, since in true uveitis significant vitritis is almost always accompanied by CMO.

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Neurological features

•An intracranial mass may cause headache, nausea, personality change, focal deficit and seizures.

•Leptomeningeal disease may cause neuropathy.

•Spinal cord involvement may cause bilateral motor and sensory deficits.

•Abnormal clinical neurological examination, such as cranial nerve palsies, hemiparesis and ataxia.

•MR of head and spine with gadolinium, which can detect one or more intracranial tumours, diffuse meningeal or periventricular lesions, and/or localized intradural spinal masses.

•Lumbar puncture, which can demonstrate malignant cells in CSF in a minority of patients with abnormal MRI. A positive result avoids the need for brain or eye biopsy.

Investigations

1FA shows blockage with a granular characteristic, due to the presence of sub-RPE accumulation of lymphomatous cells (leopard skin spots).

2 US may show vitreous debris, elevated subretinal lesions, retinal detachment and thickening of the optic nerves.

3Cytology of vitreous samples or subretinal nodules.

4Immunohistochemistry based on cell-surface markers allows identification of the lymphocytic proliferation, which is of a B-cell type in most patients.

5CNS screening by regular MR scans is indicated.

Treatment

1Radiotherapy has long been the first-line treatment for PIOL, but recurrence is common and complications such as radiation retinopathy and cataract can occur.

2Intravitreal methotrexate is useful for recurrent disease, but close monitoring is needed to detect ocular complications and any recurrence.

3Systemic chemotherapy with a variety of regimes including methotrexate can prolong survival in patients with CNS disease. This can be given in combination with whole brain irradiation but neurotoxicity is a problem. A variety of methods have been developed to overcome the blood–brain barrier. Systemic treatment is usually effective for ocular disease and this is preferred to ocular radiotherapy in some centres because in addition to avoiding radiation-induced complications it may improve survival. Monotherapy for PIOL with ifosfamide or trofosfamide has also been successful.

4Biologic agents involving specific anti-B cell monoclonal antibodies (such as rituximab), may represent a useful alternative, but probably need to be given locally because of poor penetration of the blood-brain barrier.

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Tumours of the retinal pigment epithelium

Typical congenital hypertrophy of the RPE

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a common benign lesion which may be (a) typical, either solitary or grouped, or (b) atypical. It is important to differentiate between the two types because the latter may have important systemic implications.

1Solitary CHRPE

•A flat, dark-grey or black, round or oval lesion with well-defined margins usually located near the equator (Fig. 12.52A).

•Depigmented lacunae are common (Fig. 12.52B).

•Some lesions may become virtually totally depigmented (Fig. 12.52C).

•Juxtapapillary lesions are uncommon (Fig. 12.52D).

2Grouped CHRPE

•Multiple lesions organized in a pattern simulating animal footprints (‘bear-track’ pigmentation) often confined to one sector or quadrant of the fundus with the smaller spots usually located more centrally (Fig. 12.53A).

•Rarely the lesions may be depigmented (‘polar bear tracks’) (Fig. 12.53B).

Fig. 12.52 Solitary CHRPE. (A) Completely pigmented lesion; (B) partly depigmented lesion; (C) largely depigmented lesion; (D) juxtapapillary lesion

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Fig. 12.53 Grouped CHRPE. (A) ‘Bear-track’ lesions; (B) ‘polar bear track’ lesions

(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 –fig. B)

Atypical congenital hypertrophy of the RPE

Signs

•Bilateral, multiple, widely separated, frequently oval or spindle-shaped lesions of variable size associated with hypopigmentation at one margin (Fig. 12.54A and B).

•The lesions have a haphazard distribution and may be pigmented, depigmented or heterogeneous.

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