Ординатура / Офтальмология / Английские материалы / Imaging of Orbital and Visual Pathway Pathology_Muller-Forell_2005
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Fig. 6.75a,b. A 73-year-old woman with bilateral exophthalmos. Diagnosis: Graves’ disease. CT: a Axial view with severe thickening of all external rectus muscles, excluding the tendinous insertion. Note marked compression of both optic nerves at the apex and pressure exertion on the lamina papyracea responding to slight spontaneous decompression. b Coronal view. (With permission of Müller-Forell and Lieb 1995b)
which may lead to slowly progressing or acute visual deficit or loss. This finding, in addition to the presence of corneal ulcers due to massive proptosis, is a conclusive indicator for surgical intervention, while it is discretionary in the case of cosmetically disfiguring exophthalmos. Surgical decompression of the optic nerve with removal of different orbital walls is a therapy that provides space for the enlarged muscles and alleviates optic nerve compression (Fig. 6.79). In these cases, CT may be the method of choice, especially because it is capable of presenting the bony structures,and since postoperative complication with acute hemorrhage demands an emergency examination (Fig. 6.80).
The differential diagnosis of endocrine orbitopathy includes lymphoma, metastasis, diffuse or focal idiopathic orbital inflammation with mass effect (Fig.6.64), any tumor of the nasal cavity and sinuses (Förster and Kahaly 1998), and vascular diseases like carotid
sinus cavernous fistula (Ahmadi et al. 1983). The most important differential diagnosis of endocrine orbitopathy is myositis, the local form of idiopathic orbital inflammation (see Sect. 6.2.3.1), especially in unilateral involvement (Fig. 6.81). In addition to the different clinical presentations with painful, unilateral proptosis combined with double vision and diffuse orbital swelling, the most important differential imaging criterion, visualized by both CT and MRI, is the enlargement of the muscle, including the tendon (Figs. 6.71, 6.72).This pattern is characteristic of the inflammation of a single muscle, especially since the isolated idiopathic orbital inflammation shows no preference for a muscle group,i.e.,the inferior or medial rectus muscles (Harnsberger 1990; Casper et al. 1993). Rare conditions such as unspecific, benign lymphoid hyperplasia may mimic the clinical findings of Graves disease, but present with different symptomatology and imaging morphology, as shown in Fig. 6.167.
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Fig. 6.76a–f. A 48-year-old man with acute, bilateral, symmet- |
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ric proptosis, chemosis, reduced mobility of both eyes, and |
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hyperthyreosis. Diagnosis: endocrine orbitopathy (Graves’ dis- |
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ease). a Portrait of the patient, demonstrating chemosis and |
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severe bilateral lid edema. b Axial CT, demonstrating bilateral |
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proptosis (Hertel 25 mm), thickening of the medial and lateral |
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rectus muscles, and slight pressure on the right lamina papy- |
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racea. Note thickening of both conjunctivas. c Corresponding |
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T2-weighted (FS) MRI with signal enhancement not only of |
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the enlarged rectus muscles, but also of the edema of the con- |
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junctiva of both eyes. d Coronal CT with marked involvement |
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of all external muscles, including both oblique and levator pal- |
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pebrae muscles. e Coronal T1-weighted MRI. f Corresponding |
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coronal T2-weighted image with the edema being primarily |
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detectable in the inferior, superior, and oblique rectus muscles. |
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Additional T2-time measurements (not shown) identified a |
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difference of more than 100 ms in comparison with the non- |
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inflammatory involved lateral muscles |
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Fig. 6.77. A 49-year-old woman with persistent Graves’ disease. Axial CT shows severe thickening of the medial rectus muscles, more pronounced on the right side, and marked bilateral pressure on the lamina papyracea. Note substantial hypertrophy of the orbital fat, responsible for extreme exophthalmos and stretching of both optic nerves. (With permission of
Müller-Forell and Lieb 1995b)
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Fig. 6.78a–c. A 46-year-old woman with |
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symptoms of recurrence of known |
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Graves’ disease. Diagnosis: acute Graves’ |
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disease. MRI: a Axial T1-weighted view |
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without apparent evidence of enlarged |
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rectus muscles. b Coronal T1-weighted |
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view demonstrating moderate enlarge- |
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ment of all rectus muscles, but slight |
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thickening of the left inferior rectus |
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muscle. c Axial view of octreotide/MR- |
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matching,demonstrating coating of both |
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entire orbits with octreotide. Above: |
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octreotide scan, middle: corresponding |
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MR (3D) slices, below: matched slices. |
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(c with the permission of Dr. Förster, |
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Institute of Nuclear Medicine, Medical |
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School, University of Mainz) |
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Fig. 6.79a–f. A 50-year-old woman with bilateral proptosis in the course of Graves’ disease. Diagnosis: Graves’ disease. CT: a Preoperative view with bilateral symmetric enlargement of the medial and (less marked) lateral rectus muscles. Note slight impression of the medial orbital wall as some spontaneous decompression. b Corresponding postoperative view with protrusion of the medial rectus muscles in the former ethmoidal cell area. MRI: c axial T1-weighted view, corresponding to a. d Corresponding postoperative image. Note that the enlargement of the muscles themselves remains unaffected. e Coronal T1weighted preoperative view. f Corresponding postoperative image
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Fig. 6.80a–d. A 51-year-old woman with known Graves disease. One day after operative fat resection of the left eye, the patient developed acute deterioration of the severe proptosis (right: 29 mm, left 33 mm), but no visual deficit. Diagnosis: acute retrobulbar hemorrhage (as an operative complication). CT: a Axial, native view of the medial orbit, where demonstrating bilateral severe proptosis and inferior dislocation of the left globe. b Axial view of the upper orbit with hyperdense mass in the region of the left superior muscle complex. c Coronal view of the region of the posterior globe where the hyperdense hemorrhage is seen, apparently involving the superior rectus muscle. d Coronal view of the anterior orbit, demonstrating best an inferior dislocation of the protruded left globe by the space-occupying hemorrhage
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Fig. 6.81a,b. A 45-year-old woman with known hyperthyroidism, painless, left-sided proptosis, and incomplete abduction paralysis on the left side. Diagnosis: unilateral Graves’ disease. MRI: a Axial T1-weighted view with enlargement of the left medial external muscle (although the tendon appears to be involved, Graves’ disease was confirmed based on clinical symptoms and endocrinological findings; the patient profited from radiation therapy). b Coronal T2-weighted view with massive signal enhancement of the swollen inferior and medial left rectus muscles, representing intramuscular edema
6.2.4
Miscellaneous (Amyloidoma, Metastasis, Varia)
6.2.4.1 Amyloidoma
Amyloidoma of the orbit is a rare lesion (see Sect. 6.3.4.2.4) and may resemble multiple differential diagnoses, most frequently idiopathic orbital inflammation, but also inflammatory or tumorous lesions of other etiology (Mafee et al.1999b).Clinical symptoms of painless proptosis and lack of other inflammatory signs, combined with an inhomogeneous intraorbital mass without significant signal enhancement after i.v. contrast administration, but calcification on CT, should enable an accurate diagnosis (Fig. 6.82).
6.2.4.2 Metastasis
Metastases represent the most common malignancy of the orbit and may thus involve any orbital area, with preference for the extraconal area, whereas substantial involvement of the intraconal space is rare. Enophthalmos may present as the first symptom in remote carcinoma. Solid or diffuse infiltration of the
intraconal area, leading to secondary rectus muscle shortening and contraction, and in some instances to bone destruction, is seen in cirrhotic breast carcinoma metastasis (Fig. 6.83) (Reifler and Davison 1986; Ewald et al. 1994; Lagreze et al. 1997). Traumatic/posttraumatic conditions represent another, more common etiology of enophthalmos (Fig. 6.156).
An extremely rare condition is the intraorbital metastasis of a small-intestinal carcinoid (Fig. 6.84). The finding of an intramuscular mass may be of differential diagnostic value if additional specific examination with a positive octreotide scan is not available (Shields et al. 1987b; Guthoff et al. 2000).
6.2.4.3 Varia
Although located in the extraconal area, sphenoid bone dysplasia may exert a secondary influence on intraconal tissue (muscles). Exposure of the fanshaped fibrillation of muscle fibers occurring as a secondary process to prolonged irregular strain on the rectus muscles may lead to deformity in persisting, thus far unknown disorders (Fig. 6.85), rendering, e.g., a strabismus operation impossible.
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Fig. 6.82a–f. A 71-year-old man with persistent, slowly progressing, right-sided proptosis, but an acute history of ipsilateral painful chemosis. Diagnosis: orbital amyloidoma. CT: a Axial native view where medial of the orbit, in addition to exophthalmos of the right eye and extraconal location of the slightly enlarged lacrimal gland, an irregular contour of the lateral rectus muscle and adjacent bulbar fat can be identified. b Axial native view of the superior orbit which is characterized by an irregular, partly calcified mass. c Coronal view of the middle orbit, demonstrating the partly calcified intraconal mass between the lateral and superior rectus muscle. MRI: d Axial T1-weighted native view, corresponding to b. Note the small, normal superior ophthalmic vein in both images. e Corresponding contrast-enhanced (FS) view without marked contrast enhancement of the mass. f Coronal T1-weighted view (corresponding to c) with suspicion of additional extraconal involvement
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Fig. 6.83a–f. A 57-year-old woman with known NF 1, and enophthalmos for the past 6 weeks, combined with increasing double vision for all directions of the left eye. Diagnosis: (estrogen-receptor positive) metastasis of a cirrhotic breast carcinoma as initial symptom of the disease. MRI: a Axial proton density view of the orbit, demonstrating enophthalmos of the left eye, pathologic signal intensity of the retrobulbar space and widening of the CSF space of the left optic nerve. b Corresponding T1-weighted native view, distinguishing only little intraand extraconal (medially) fat (arrows). c Corresponding T1-weighted, contrast-enhanced (FS) view, showing enhancement of the entire orbital tissue (compare with corresponding right side). d Coronal T1-weighted native view with similar signals of the rectus muscles and of the primarily intrabut also extraconal tumor. Remnants of orbital fat are seen as small hyperintense structures in the inferior left orbit. e Corresponding T1-weighted, contrast-enhanced (FS) view with complete tumor enhancement. f Parasagittal T1-weighted IR view, demonstrating compression of the optic nerve and widening of the CSF space in the optic nerve sheath (compare to a)
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Fig. 6.84a–e. A 68-year-old woman with known enteric carci- |
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noid for the past 3 years, presenting with acute onset of double |
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vision. Diagnosis: Suspected metastasis of an enteric carcinoid |
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(no histology, patient refused therapy). CT: a Axial, contrast- |
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enhanced view with belly-like enlargement of the right medial |
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rectus muscle due to a slightly ring-shaped enhancing intra- |
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muscular mass. b Corresponding coronal view. Coronal MRT: |
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c T2-weighted (FLAIR) view where the target-like mass pres- |
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ents definitely intramuscularly. d Corresponding T1-weighted |
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native view. e Corresponding T1-weighted, contrast-enhanced |
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(FS) view with extensive signal enhancement of the outer rim |
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of the lesion. Note the slight enlargement of the ipsilateral |
inferior rectus muscle |
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Fig. 6.85a–e. A 36-year-old man undergoing imaging prior to planned surgical correction of persistent divergent strabismus. Diagnosis: NF 1 with sphenoid wing dysplasia and subsequent muscle degeneration. CT: a Axial image with deformed left orbital content: deformed globe and lateral rectus muscle as well as bony pathology are more conclusively visualized in corresponding view in bone window (b). Impression of the lamina papyracea, thickening of the large wing of the sphenoid, and widening of the orbital fissure (normal optic canal – not shown), apparently due to the absence of the small wing of the sphenoid, findings leading to suspected NF 1. MRI: c Corresponding axial native T1weighted view. Note the deformed left globe. d Corresponding contrast-enhanced (FS) image demonstrating the spread of single muscle fibers of the lateral rectus muscle, expanding intracranially and impinging on the temporal pole. e Coronal T1-weighted view at the level of the optic canal (white star: right, white arrow: left), showing extensive intracranial
expansion of intraorbital tissue
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