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Ординатура / Офтальмология / Английские материалы / Imaging of Orbital and Visual Pathway Pathology_Muller-Forell_2005

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346

W. Müller-Forell

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Fig. 7.4a–d. A 17 year old girl with complete blindness (and deafness) since childhood and a known neurofibromatosis. Diagnosis: astrocytoma WHO II. MRI: a Axial, T1-weighted, contrast-enhanced view at the level of the chiasm, showing the solid tumor in the entire suprasellar cistern, growing into the right optic nerve and canal with intraorbital expansion. Note a cystic tumor with a small, solid, contrast-enhancing tumor knot in the left temporal lobe. b Axial proton-weighted view, demonstrating the hypothalamic and bilateral thalamic infiltration, apparently arising from both optic tracts. c Sagittal, T1-weighted, contrastenhanced view, in which not only the expansion into the sella with compression of the pituitary gland and hypothalamic infiltration is apparent, but another tumor part in the pulvinar of the thalamus is seen. d Coronal, T1-weighted, contrastenhanced view showing the huge bilateral tumor expansion

Intracranial Pathology of the Visual Pathway

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Fig. 7.5a–c. A 5-month-old girl with persisting vertical nystagmus, intermittent strabismus, and increased intracranial pressure. Diagnosis: astrocytoma (WHO II) of the chiasm with infiltration of the left optic nerve. MRI: a Axial T2weighted view with a very large, space-occupying, isointense lesion located in a widened suprasellar cistern, depressing and spreading the basal vessels. b Coronal T1-weighted native view demonstrating pressure exertion on the widened third ventricle by the central hypointense (necrotic) tumor. c Midsagittal, T1-weighted, contrast-enhanced view with demarcation of the entire enhancing tumor, compressing and displacing the brainstem, and extending into the posterior fossa. Note widening of the entrance of the otherwise normally configured sella (arrow). (With permission of Dr. Klusemann, Radiologische Gemeinschaftspraxis, Bad Homburg)

348

W. Müller-Forell

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Fig. 7.6a–e. A 3-month-old girl with bilateral nystagmus and pap-

 

illedema of the left eye. Diagnosis: juvenile pilocytic astrocy-

 

toma (WHO I–II) of the chiasm. CT: a Axial contrast-enhanced

 

image, showing an extensive tumor with central necrosis in the

 

suprasellar cistern. MRI: b Corresponding T1-weighted, contrast-

 

enhanced view. c Axial, T1-weighted, contrast-enhanced view at

 

the level of the cerebral peduncles with visualization of mixed

 

intraand extraparenchymal tumor, extending from the inferior

 

third ventricle with infiltration of the right mesial temporal lobe

 

and the ipsilateral cerebral peduncle. d Right paramedian, sag-

 

ittal, T1-weighted, contrast-enhanced plane demonstrating the

 

presence of hypothalamic infiltration, infiltration of the optic

 

tract and fibers of the fornix (arrows), in addition to intracranial,

 

CSF-induced metastasis at the craniocervical junction. e Coronal,

 

T1-weighted, contrast-enhanced view with more distinct demon-

 

stration of infiltration of the optic tract (large arrow) extending

 

towards the right lateral geniculate nucleus, as well as infiltration

c

of the right fornix (small arrow) and the metastatic tumor at the

right craniocervical junction

Intracranial Pathology of the Visual Pathway

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Fig. 7.7a–d. A 9-year-old boy with pubertas prae-

 

cox.A bilateral optic nerve atrophy and visual def-

 

icit (right: 0.4, left: 0.6) were unknown, but found

 

during ophthalmological examination. Diagno-

 

sis: optic glioma with hypothalamic involvement.

 

MRI: a Axial T2-weighted view with right hypo-

 

thalamic (posterior of the anterior commissure)

 

and temporomesial tumor infiltration. Note the

 

slight signal enhancement of the left optic tract

 

(arrow), indicating an additional contralateral

 

involvement. b Corresponding T1-weighted, con-

 

trast-enhanced view with BBB disruption of all

 

tumor regions. c Coronal, T1-weighted, contrast-

 

enhanced view, best demonstrating the basal gan-

 

glia and hypothalamic tumor spread with prefer-

 

ence to the right side. d Midsagittal, T1-weighted,

 

contrast-enhanced image without any delineation

d

of the chiasm

350

W. Müller-Forell

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Fig. 7.8a–f. A 70-year-old woman with a visual deficit progressing to complete loss of vision in the right eye within 2 weeks, and subsequent loss of vision (0.1) in the left eye. Diagnosis: glioblastoma of the chiasm, intracranial optic nerve, and proximal optic tract. MRI: a Midsagittal T1-weighted native image with diffuse enlargement of the chiasm and hypothalamus. b Corresponding T1-weighted, contrast-enhanced image with brighter signal enhancement of the enlarged chiasm than of the pituitary stalk. c Right paramedian sagittal, T1-weighted, contrast-enhanced image showing tumor expansion along the proximal optic tract. d Axial, T1weighted, contrast-enhanced view, demonstrating lateralization of the tumor growth to the right, as well as some necrosis at the center of the tumor. e Coronal, T1-weighted, contrast-enhanced image visualizing exclusive infiltration of the chiasm. f Coronal, T1-weighted, contrast-enhanced view, several millimeters anterior to e with tumor infiltration of the intracranial right optic nerve

Intracranial Pathology of the Visual Pathway

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(Walsh 1985). Visual impairment is dependent on the size and location of the mass, thus a significantly higher percentage of visual loss is seen in children with involvement of the postchiasmal visual pathway (Balcer et al. 2001).

Imaging should discriminate from non-neoplastic tumor-like lesions as in the patient with recurrent retro-bulbar neuritis (RBN) of unknown origin, that present with additional involvement and thickening of the chiasm (Fig. 7.10). If the chiasmal lesion demonstrates signs of malignancy like a necrotic area (Fig. 7.8), spread into the nearby brain parenchyma, or meningeal implants (Fig. 7.9) (Shapiro et al. 1982), the diagnosis of malignant glioma of the chiasm is likely.

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Fig. 7.9a–c. A 54-year-old woman with recurrent, exacerbating headache, N VI-paresis, and acute visual deficit in the right eye. Diagnosis: astrocytoma (WHO III). T1-weighted, contrastenhanced MRI: a Axial image showing multilobulated, contrastenhancing lesions at the chiasm (slightly asymmetric to the right), hypothalamus, and proximal part of the right optic tract (arrow), as well as at the quadrigeminal plate, with greater accentuation to the left side. Note widening of the temporal horns of the lateral ventricles as a sign of increased intracranial pressure exerted by an occlusive hydrocephalus, due to midbrain invasion with consecutive stenosis of the aqueduct. b Coronal view demonstrating invasion of both the chiasm and the right thalamus. Note periventricular signal diminution by transependymal CSF leakage (white stars) associated with the identified occlusive hydrocephalus. c Right paramedian sagittal image, showing complete tumor invasion of the chiasm, hypothalamus, rostral and posterior nuclei of the thalamus, the quadrigeminal plate, and the aqueduct, extending to the floor (rhombencephalon) and the roof of the fourth ventricle

7.2.1.2

Pituitary Lesions

7.2.1.2.1

Pituitary Adenoma

Although pituitary adenomas represent 10%–15% of all intracranial tumors (Kovacs et al. 1985; Okazaki 1989), the systematic of tumors of the “nervous system” (Kleihues and Cavanee 2000) consistently lacks the definition of pituitary adenomas, which most frequently manifest in adults. These benign, slowly growing, and dislocating tumors composed of cells of the adenohypophysis normally have a pseudocapsule, which enables good

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Fig. 7.10a–c. A 44-year-old man presenting with recurrent, alternating signs of bilateral retro-bulbar neuritis (RBN) with visual field defects and retardation on VEP without an afference defect; no pathologic findings on CSF and humoral immunology. Diagnosis: chiasmal edema of unknown origin. T2-weighted MRI: axial (a), sagittal (b), and coronal (c) (FLAIR) views demonstrating a thickening and high signal intensity of the entire chiasm with preference to the right. No systemic disorder could be found, but cortisone therapy diminished the subjective symptoms

demarcation from the neighboring structures. They frequently present as degenerative cysts with small necrotic areas and hemorrhages, sometimes leading to acute degeneration, i.e., adenoma apoplecticum (Figs. 7.11–7.13). This entity occurs as a result of sudden infarction or hemorrhage in the course of tumor growth. However, the clinical symptomatology of insufficiency of the adenohypophysis, presenting with headache, nausea, and vomiting, is not as frequent as presumed (Bonneville et al. 1986; Zülch 1986; Kucharczyk et al. 1996). In medical

clinical symptomatology, pituitary adenomas present predominantly in the form of endocrinological disorders, depending on the activity of the pathological cells, which is why endocrinological active pituitary adenomas are most frequently found in the group of microadenomas (diameter <10 mm). Conversely, hormone-inactive tumors (belonging to our subject) fulfill the criteria of macroadenomas (diameter >10 mm) as they present with the characteristic clinical symptom of bitemporal visual field deficit, due to inferior compression of the

Intracranial Pathology of the Visual Pathway

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Fig. 7.11a–c. A 47-year-old man who presented in pituitary coma. After substitution, he complained of headache and variable visual deficits. Hemianopia to the left, visual deficit (right: 0.6, left: 0.2) was found, together with an inflammatory clinical constellation with fever and meningism. Diagnosis: abscess-forming pituitary adenoma with adjacent bacterial leptomeningitis. T1-weighted MRI: a Coronal native view with intraand suprasellar lesion and inferior chiasmal compression. Note the slightly hypointense signal in the sphenoid sinus. b Corresponding contrast-enhanced view with inhomogeneous contrast enhancement of the intra-/suprasellar, apparently encapsulated lesion,but homogeneous enhancement in the sphenoid sinus.CORR = Sponding to sinus inflammation, note the small leptomeningeal enhancement at the base of the left frontal lobe (arrow). c Axial contrastenhanced view with necrotizing, encapsulated tumor and leptomeningeal enhancement of the basal frontal sulci (arrows)

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Fig. 7.12a–d. A 62-year-old man with slowly progressing visual deficit of the left eye persisting for 2 years. Diagnosis: pituitary adenoma with small colloidal cysts (proven by histology), not clearly differentiated from older hemorrhage (methemoglobin). MRI: a Axial T2-weighted view identifying different signals with high (left) and apparently low signal intensity (right) of the tumor of the suprasellar region, spreading to the proximal optic tracts (white arrows). b Coronal T1-weighted native image in which both intrasellar and suprasellar expansion with inferior impression of the chiasm is demonstrated. c Midsagittal, T1weighted, contrast-enhanced view identifying slight signal enhancement of the solid tumor parts invading the sphenoid sinus. Note the hyperintense lesion superior to the posterior knee of the corpus callosum, representing a small lipoma. After 5 days, the patient developed acute, nearly complete loss of vision on the left side as well as severe, ipsilateral N VI paresis, together with an adynamic state: d axial native emergency CT shows acute hemorrhage into the pituitary adenoma, expanding superiorly, necessitating immediate decompression of the chiasm

Intracranial Pathology of the Visual Pathway

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13.17

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rest of 13.11

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Fig. 7.13a–d. A 21-year-old man with hypogonadism and hypophyseal-hypothalamic insufficiency in the absence of ophthalmologic deficits. Diagnosis: prolactinoma with acute hemorrhage. MRI: a Axial T2-weighted view with irregular hyperintensity (methemoglobin) in the sphenoid sinus and enlarged sella. b Right paramedian, sagittal, T1-weighted native view demonstrating suprasellar tumor expansion, compression of the slightly hyperintense pituitary stalk (small arrow), and tumor extension to the right prechiasmal optic nerve (large arrow). c Coronal, T1-weighted, contrast-enhanced view with dislocation of the enhanced pituitary stalk (arrow) to the right. Note the thin layer on the left side of the tumor, representing the remaining pituitary gland (arrowheads). d Corresponding diagram: 3.9 = round foramen, 12.2 = chiasm, 13.11 = pituitary gland, 13.12 = pituitary stalk13.17 = ventricles, 14.2 = ICA

chiasm (Fig. 7.14) (Kucharczyk et al. 1996; Majos et al. 1998), but sometimes even only with unspecific visual deficits (Fig. 7.15).

Imaging Characteristics. MRI as the method of choice generally shows a lengthening of both T1 and T2 relaxation times. In macroadenomas, MRI enables a high anatomic resolution and definition of the neighboring tissue, i.e., intracranial optic nerves, chiasm, and cavernous sinus. In most cases, native, non-contrast-enhanced images allow accurate and conclusive differentiation of the tumor and deformed, compressed, flattened visual structures of the optic

nerves, chiasm, and/or optic tracts (Figs. 7.11, 7.12, 7.15–7.19). Due to the lack of a solid barrier between the pituitary gland and the cavernous sinus in the presence of only a loose circumferential fibrous bed (Dietemann et al. 1998), even a careful analysis of the preand post-contrast images does not routinely enable the definite prediction of tumor invasion of the cavernous sinus. Only a carotid artery encasement or an extension lateral of the cavernous sinus towards the temporal lobe is a reliable indicator of cavernous sinus invasion (Scotti et al. 1988; Kucharczyk et al. 1996). In pre-contrast images, most adenomas show a homogeneous, isointense signal and a similarly

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