Ординатура / Офтальмология / Английские материалы / Ocular Pathology_6th edition_Yanoff, Sassani_2009

1.Fibroblastic and angiomatous types of meningiomas rarely occur primarily in the orbit.

“Angioblastic type” of meningioma, once thought to be of meningeal origin, is now generally accepted to be a hemangiopericytoma of the CNS. However, a case of primary orbital angiomatous meningioma, not arising from the optic nerve, has been reported.

2.Frequently, meningiomas extend extradurally to invade the orbital tissue.

3.Uncommonly, they invade the optic nerve and sclera and may even invade into the choroid and retina.

Malignant meningioma may be diagnosed if the tumor

shows either, or both, unequivocal anaplasia or invasion of brain parenchyma (<10% of intracranial meningiomas are malignant).

IV. Melanocytoma (see p. 721 in Chapter 17)

V.Hemangioma is usually associated with the phakomatoses

(see p. 30 in Chapter 2).

Hemangiomas, usually cavernous, rarely capillary, infrequently may occur as a primary optic nerve tumor unassociated with the phakomatoses.

VI. Medulloepithelioma may rarely arise from the distal end of the optic nerve (see p. 686 in Chapter 17).

VII. Giant drusen of the anterior portion of the optic nerve are astrocytic hamartomas usually associated with tuberous sclerosis (see pp. 34–35 in Chapter 2).

VIII. Ordinary drusen of the anterior portion of the optic nerve (Fig. 13.20)

A.Ordinary optic disc drusen occur in 3.4 to 24 per 1000 population and are bilateral in about 75%.

The drusen tend to increase in size with age because of increased calcium deposition.

B.These may present as pseudopapilledema.

Ordinary drusen of the optic nerve may occur in retinitis pigmentosa or pseudoxanthoma elasticum.

Drusen of the optic nerve occur 20 to 50 times more often in pseudoxanthoma elasticum than in the general, healthy population.

C.Although field defects are common (87% in one series), very rarely does a patient lose central vision.

D.Hemorrhage of the optic disc is a rare complication.

1.The hemorrhage may extend into the vitreous or under the surrounding retina.

2.Peripapillary subretinal neovascularization may occur.

E.Histologically, basophilic, calcareous, laminated acellular bodies of di erent sizes and shapes are located in the substance of the optic disc anterior to the scleral lamina cribrosa.

Drusen seem to start intracellularly, but then enlarge and become extracellular. Alterations in axoplasmic transport (flow) may play a role in the formation of these drusen.

IX. Drusen of the adjacent RPE may protrude into the lateral aspect of the retinal layer of the optic nerve head.

Although drusen of the RPE and of the optic nerve have the same name, they are quite different. RPE drusen are basement membrane secretions of the RPE (Fig. 13.21), whereas optic nerve drusen are structurally as described previously and may be degenerative products of optic nerve glial cells, presumably astrocytes anterior to the scleral lamina cribrosa.

X.Corpora amylacea—these are intracellular, basophilic, periodic acid–Schi -positive structures often observed in the white matter of the brain, including optic nerve and neural retina (mainly nerve fiber layer).

A.The structures are composed of a glycoprotein–acid mucopolysaccharide complex produced within neuronal axons, and probably represent products of axonal degeneration.

B.They have no clinical significance and are considered an aging phenomenon.

Corpora amylacea in the retinal ganglion cell layer may decrease in number with advancing glaucoma.

XI. Corpora arenacea (psammoma bodies)

A.These are laminated, basophilic bodies produced by the arachnoid meningothelial cells.

B.They are of no clinical significance and are an aging phenomenon.

Morphologically identical structures, psammoma bodies, may be found in meningiomas and in a variety of papillary carcinomas.

XII. Cysts of the optic nerve may be congenital, arise de novo, or occur secondary to conditions such as optic nerve juvenile pilocytic astrocytomas (gliomas), NF, empty sella syndrome, or hemangioma of leptomeningeal origin.

Optociliary veins (shunt vessels) may be found in the presence of optic nerve cysts.

XIII. Choristoma

XIV. Buscaiano bodies resemble corpora amylacea but are fixation artifacts.

Secondary

I. Retinoblastoma

II. Malignant melanoma of choroid

III. Pseudotumor of RPE

IV. Intracranial meningioma

V.Metastatic carcinoma

A.Metastatic to parenchyma

B.Metastatic to meninges

VI. Glioblastoma multiforme of brain VII. Lymphoma or leukemia (Fig. 13.22)

VIII. Macroscopically, artifacts may simulate secondary (or primary) optic nerve tumors (e.g., myelin).

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NORMAL ANATOMY

I.The orbital volume is approximately 30 ml, and the orbital depth (anterior to posterior) is approximately 4.5 cm (Figs

14.1 to 14.5).

A.The medial orbital wall is quite thin (<0.5 mm) and transparent.

B.The roof of the orbit, composed mainly of the orbital plate of the frontal bone, is thinnest anteriorly, where it is adjacent to the frontal sinus, and separates the orbit from the frontal lobes of the brain.

C.The orbital floor is composed of the orbital plates of the maxilla and zygomatic bones and a small contribution from the palatine bone posteriorly.

D.The floor is also thin, 0.5 to 1 mm, and thus is easily fractured, especially medial to the infraorbital canal.

E.Conversely, the lateral orbital wall is thick, composed anteriorly by the zygomatic bone and posteriorly by the greater wing of the sphenoid.

II.In addition to the bony walls, the eye, and the optic nerve, the orbit contains many soft-tissue structures such as fat, muscle (striated and nonstriated), cartilage, bone, fibrous tissue, nerves, and blood vessels.

A.Other than the epithelia within the eyeball, the lacrimal gland is the only epithelial structure in the orbit.

B.All orbital structures may be involved in disease processes.

C.Orbital disease, whatever its cause, tends to increase the bulk of the orbit, so the main presenting sign is exophthalmos.

EXOPHTHALMOS

I.The main clinical manifestation of orbital disease is exophthalmos (ocular proptosis), the extent and direction of which depend on a number of factors*: (1) size of lesion;

(2)character of lesion (expansile versus infiltrative

growth, rapid versus slow growth); (3) location of the lesion in the orbit (small lesion in muscle cone causes more exophthalmos than lesion of same size outside the muscle cone; lesions anterior to septum orbitale do not produce exophthalmos unless they also grow posteriorly); and (4) lesion’s e ect on the extraocular muscles (complete paralysis of all muscles by itself can cause 2 mm of exophthalmos)

II.Exophthalmos may be simulated by many conditions: lid retraction due to any cause (most commonly Graves’ disease); unilateral enlargement of the globe; high myopia; buphthalmos; sagging lower lid; relaxation of the rectus muscle(s); enophthalmos or microphthalmos of the oppo-

*Exophthalmos refers specifically to protrusion of the eyes, whereas proptosis refers to protrusion of any part of the body.