Orbital Pathology, EJR 2004

European Journal of Radiology 49 (2004) 105142
Orbital pathology
W. Mller-Forell a, , S. Pitz b
b a Institute of Neuroradiology, Medical School University of Mainz, Langenbeckstrasse 1, D-55101 Mainz, Germany Department of Ophthalmology, Medical School University of Mainz, Langenbeckstrasse 1, D-55101 Mainz, Germany
Received 7 October 2003; received in revised form 8 October 2003; accepted 10 October 2003
Abstract This overview of orbital pathology deals with different kinds of tumors, inammatory, vascular, and traumatic diseases, which may involve the orbit. Depending on the respective orbital compartment of the globe, the intrakonal, extrakonal and optic nerve the most important and most frequent lesions are presented with their specic clinical symptoms. Their specic presentation on CT- and MR-imaging is discussed in detail, including the most important differential diagnosis. 2003 Elsevier Ireland Ltd. All rights reserved.
Keywords: Idiopathic orbital inammation; Retinoblastoma; Rhabdomyosarcoma; Metastasis; CT; MRI
1. Orbital pathology Although more than 100 different clinical pathologies may affect the orbit [1] only some require diagnostic imaging. The knowledge of clinical signs and symptoms plays a crucial role in determining the appropriate investigation. The age of the patient may lead to the diagnosis, as in children the most frequent orbital diseases constitute in inammatory processes, trauma or malignant tumors (e.g. rhabdomyosarcoma or neuroblastoma), while in the group of elderly patients neoplasms represent the main pathologies. As already mentioned in the anatomical section, the consideration of the four compartments of the orbit (the globe, the intrakonal and the extrakonal space and the optic nerve itself with its menigeal layers) may help in differential diagnosis of the different diseases. In this short overview we will present the most frequent inammatory and tumorous diseases and some characteristic traumatic lesions of each of these compartments.
case of leucocoria or cataract MR-imaging (MRI) may be required. Its high resolution is able to visualize not only the pathology itself but accompanying retinal detachment and/or effusion. 2.1. Inammatory disorders Although rare, the posterior scleritis represents the local form of the idiopathic orbital inammation, predominantly affecting females [2]. Clinical presentation is characterized by a slowly progressing, painful proptosis accompanied by decreased vision, due to thickening of the sclera and choroidal folds. Histologically the inammation presents diffuse or localized, necrotic or granulomatous, and scleral sequesters are found with leakage of proteinaceous edema uid into the interstice of the uvea and Tenons capsule [3,2]. CT- and MR-imaging of scleritis is characterized by diffuse scleral thickening of different extent and inltration of the adjacent tissue with contrast enhancement, depending on the extent of the inammatory process (Fig. 1ac). Nodular scleritis does not show contrast enhancement [2,4], an important criterion in differential diagnosis to uveal melanoma. 2.2. Tumors The neoplastic diseases of the globe may arise from different layers, the most signicant diagnosis are the retinoblastoma in childhood and the uveal melanoma in the adult patients.
2. Globe The majority of the diseases of the globe are assessed by ophthalmologic methods and do not need imaging, but in
Tel.: +49-6131-17-7139; fax: +49-6131-17-6643. E-mail address: mueller-forell@neuroradio.klinik.uni-mainz.de (W. Mller-Forell).
0720-048X/$ see front matter 2003 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrad.2003.10.011
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W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142
Fig. 1. MR of a 48-year-old man with right hemicrania, orbital pain and edema of the right eyelid. Diagnosis: posterior scleritis of the right eye. (a) Axial T2w view demonstrating the retrobulbar inammation (plus edema) of the right side. (b) Corresponding T1w native view with isointense signal of the retrobulbar inltration. (c) Corresponding T1w contrast-enhanced (fat suppressed: FS) view with bright signal enhancement (white arrow).
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Fig. 2. A 2-year-old boy with leukokoria of the left eye. Diagnosis: retinoblastoma. (a) The axial CT demonstrates not only spots of calcication but slight hyperdensity of the left vitreous body. (b) Corresponding proton density (PDw) MR, where the tumor itself (white star) looks isointense, while bilateral retinal detachment show bright signal (white arrows). (c) Corresponding T1w contrast-enhanced (FS) view. Note the intensive enhancement of the tumor, but intermediate signal of the subretinal uid.
Fig. 3. A 2-year-old boy presenting with leukokoria and complete retinal detachment of the left eye. Diagnosis: Coats disease. (a) Axial CT with only slight hyperdensity of the entire left globe, which also demonstrates a diminution, compared to the right. (b) Corresponding IR view, where the characteristic V-shaped, bilateral retinal detachment is seen. The hyperintensity represent some hemorrhage, the arrow indicates the remaining vitreous body. (c) The corresponding T1w contrast-enhanced (FS) image demonstrates only slight enhancement of the detached retina, and lack of enhancement of the remaining vitreous body behind the lens (white arrow) (with permission of Mller-Forell [15]).
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2.2.1. Retinoblastoma Retinoblastoma, a highly malignant tumor arising from neuroectodermal cells of the retina, represents the most common intraocular tumor in childhood, striking more than 90% children younger than 5 years of age, at an equal sex contribution [5,6]. There are non-heritable and inherited forms, the latter are autosomal dominant. A chromosome 13q14 band mutation is seen in all patients with bilateral occurrence and in 15% with unilateral disease [79]. Leukokoria is the most common clinical sign, found in 60% of the patients, followed by strabismus, and (rather rare) pain caused by secondary glaucoma [5]. Imaging should be used in the diagnosis of retinoblastoma, although ophthalmoscopy may conrm tumors as small as 0.002 mm. Especially MR is able to dene tumor extension in all aspects of the globe, an eventually extraocular extension, intracranial metastasis, and/or a second tumor. Bilateral retinoblas-
toma with or without pinealoma (trilateral retinoblastoma) are found in about 2535% of the cases [10]. Consequently MRI is the method of choice as it has not only the diagnostic potential concerning small intraocular lesions (especially when surface coils are used), but the ability of three dimensional examination, combined with the high resolution of potential intracranial pathology, especially after injection of paramagnetic gadolinium [1113], leaving CT-examination with its demonstration of calcication (Fig. 2a) only for cases of differential diagnostic problems. MR is able to dene the different growth patterns of endophytic, exophytic of diffuse retinoblastoma [14,12]. On T1w and proton density images retinoblastoma appears slightly hyperintense compared to normal vitreous, the latter enabling a better visualisation of accompanying retinal detachment, while on T2w images the tumor is seen as an area of low signal intensity. After injection of contrast medium
Fig. 4. MR of a 74-year-old man with progressive visual decit of the left eye. Diagnosis: Choroidal melanoma. (a) Axial T2w view with hypointense tumor of the medial circumference of the left globe. Note bilateral retinal detachment (white arrows). (b) Corresponding T1w native view where the tumor demonstrates a hyperintense signal. (c) Corresponding T1w contrast-enhanced view with bright signal of the enhancing tumor.
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retinoblastoma exhibit a moderate to marked enhancement, especially on fat-suppressed sequences (Fig. 2ac) [6,13]. The most important differential diagnosis of retinoblastoma are lesions also presenting with leucocoria like persistent hyperplastic primary vitreous (PHPV), and Coats disease (Fig. 3) [15] while endophthalmitis, retinal detachment, toxocaria and choroidal hemangioma are rather rare [12,16,17]. 2.2.2. Malignant uveal melanoma The incidence of malignant uveal melanoma, the most common primary intraocular tumor in adults, increases with age and ranges from 5.2 to 7.5 cases per million per year at a mean age of 53 years [6,18]. Usually diagnosis of uveal melanoma is made by ophthalmoscopy, uorescein
angiography and ultrasound, but imaging is required in case of opaque lens or extensive subretinal effusion [2,6]. Uveal melanoma may arise from benign choroidal nevi, not demanding imaging, but regular ophthalmoscopic control. The most common starting-point is the choroid (85%) (Fig. 4), while the ciliary body (9%), and the iris (6%) are rather rare locations (6). Uveal melanoma present with different growth patterns: the most common is circumscribed nodular, a diffuse growth is seen rather rare. In case of a mushroom-like appearance the tumor progression into the orbit is caused by the rupture of Bruchs membrane [6,19], a nding to direct ones attention especially in early stages (Fig. 5), as the therapy is different from that of exclusively intraocular melanoma. As in retinoblastoma, MR is the imaging of choice, although the elevated, primarily
Fig. 5. MR of a 60-year-old female with visual decit of the right eye. Diagnosis: choroidal melanoma with initial destruction of Bruchs membrane. (a) Axial T2w view of the inferior orbit, where the tumor of the right globe demonstrates a hypointense signal. (b) Corresponding T1w native view. (c) Corresponding T1w contrast-enhanced (FS) view of the inferior globe, where the melanoma demonstrates a characteristic bright enhancement. (d) Coronal T1w contrast-enhanced (FS) view, where a slight irregularity of the inferior circumference (white arrow) indicates the extraocular growth of the tumor (conrmed by histology).
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hyperdense, sharply circumscribed enhancing tumors are seen on CT. Melanin produces stable free radicals, which cause a paramagnetic proton relaxation enhancement, that shortens both T1- and T2-relaxation times, and resulting in a moderately high signal in T1w and PDw images, while signal reduction is seen on T2w images (Figs. 4 and 5) [20]. The well dened tumor present in up to 95% hyperintense (with respect to the vitreous) in T1w, and hypointense in T2w images, and demonstrate a bright signal enhancement after gadolinium injection, especially in fat-suppressed images, which enables the detection of small tumors as well as an additional differentiation to retinal detachment (Fig. 4) [3,12,19,21]. Differential diagnosis should include choroidal hemangioma, choroidal nevi, choroidal detachment, neurobroma,
disciform degeneration of the macula, and metastasis [2]. 2.2.3. Different retinal and choroidal lesions Choroidal hemangioma is a congenital vascular hamartoma seen in middle-aged to elderly patients with neurocutaneous syndromes, which may present solitary or diffuse [2,22]. On CT/MR they present as lenticular, hyperdense/hyperintense (in both T1- and T2w-images) well circumscribed structure in the juxtapapillary or macular region with a marked/intense contrast enhancement [2,6,15]. It should not be mistaken with the capillary hemangioma in Hippel-Lindau syndrome, where imaging is indicated in order to conrm/rule out cerebellar or spinal cord hemangioma, as diagnosis of retinal hemangioma is
Fig. 6. A 19-year-old male car accident victim (no seat belts worn at the time of the accident!). At the follow-up (6 months after the accident), the patient presented with suspicion of endophtahlmitis. Diagnosis: right globe rupture with vitreous body hemorrhage, multiple orbital wall fractures, developing posttraumatic phthisis. (a) Axial CT, showing multiple fractures of all orbital walls, with penetrating fragment into the globe, vitreous hemorrhage, and post-bulbar hematoma (black arrow). (b) Corresponding T1w native view 6 months later with shrunken right bulb in the inferior orbit, and dislocation of the medial external muscle and fat into the fractured ethmoidal sinus. (c) Corresponding contrast enhanced (FS) view with enhancement of the brous scar tissue as intense as the enhancement of the external muscles (with permission of Mller-Forell [15]).
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Fig. 7. Axial CT of a 78-year-old woman, who fell in the course of a basal ganglia infarction. Diagnosis: acute vitreous hemorrhage. Note the hyperdensity of the left vitreous body compared to the right, combined with a slight swelling of the left eyelid.
based on its typical ophthalmoscopic appearance [23,24]. The rather rare choroidal osteoma is typically seen in young females before completion of the third decade of life. This benign ossifying tumor contains marrow, mast cells, loose brovascular elements, and mesenchymal cells. On imaging it presents as a sharply demarcated, lentiform calcication on CT, on MR with a high signal on non-enhanced T1w and low signal on T2w images, and a marked signal enhancement after gadolinium injection, simulating both retinoblastoma and choroidal melanoma [2,6,15,25]. 2.3. Traumatic lesions Although the globe, its neurovascular structures and muscles are protected by the bony orbit and the shock-absorbing fat the ocular bulb as the most anterior structure of the visual system is extremely vulnerable. In case of impaired ophthalmoscopy (e.g. extreme swelling of the eyelid) CT can visualize rapidly and accurately the full extent of the injury. On the other hand, after a trauma, the external integrity of the globe and orbit should never lead to the assumption of intact retroglobal structures, since innocuous orbital trauma may produce devastating functional decits. Injury of the globe may occur as a component of major facial trauma (Fig. 6) or as an isolated event (Fig. 7). Orbital injuries may be caused by blunt and penetrating trauma [26,27]. While the former is due to an impact with low-velocity forces, the latter is a result of impact at proportionally high energy with penetrating of the object (Fig. 8). In case of orbital trauma, CT is the method of choice, as, beside the readily availability it enables both accurate visibility of bony and soft-tissue lesions and exact localization of (mostly ferromagnetic) foreign bodies without risk of dislocation. The scope of ocular injuries includes a great
variety of lesions, ranging from rupture of the globe to dislocation of the lens, from vitreous (Fig. 7), subretinal, retrohyaloid hematoma to retroocular hemorrhages. In the sequel of severe ocular trauma, phthisis bulbi refers to a scarred, shrunken globe, possibly associated with dystrophic calcication, as a type of developmental microphthalmia (Fig. 6) [4].
3. Konal/intrakonal area 3.1. Inammatory lesions With more than 60% inammation is the far most common disease of the orbit, the majority of which are related to thyroid orbitopathy [1]. 3.1.1. Idiopathic orbital inammation Since the commentary of ROOTMAN [28] the term orbital pseudotumor, routinely used for a diversity of disorders, which represent the most frequent pathologic process of the orbit, should be discontinued. As this term has contributed to diagnostic confusion both clinically and pathologically, it should be replaced by a more specic denition regarding the underlying pathophysiological and clinical patterns. Idiopathic orbital inammation is one of the four major intraconal disorders, the other include inltrative, mass effect and vascular disorders. They all present with different but specic clinical symptoms, requiring the individual diagnosis in an individual case, following consideration of specic nding on imaging, the age of the patient, systemic evaluation and the result of biopsy [28]. Though, systemic steroid therapy may be regarded as a pragmatic therapeutic strategy in the majority of inammatory cases, but histological conrmation of the underlying
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Fig. 8. CT of a 35-year-old male with foreign body accident 3 days before, presenting awake but with hemiparesis of the left side. Diagnosis: perforation of the right globe by a nail, with intracranial penetration and basal ganglia hemorrhage. (a) Axial midorbital view, showing the shrunken right globe, and some air bubbles in the right orbit. (b) Axial view of the brain at the level of the basal ganglia, where the hemorrhage of the lateral basal ganglia is seen, in combination with some small air inclusions. The metallic artifacts are caused by the nail in the occipital lobe. (c) Lateral scout, demonstrating the intracranial position of the nail.
pathologic process should be mandatory, since some tumors, especially lymphomas are characterized by similar presentation and therapeutic answer [29]. Histological cellular components of idiopathic orbital inammation present extremely variable, consisting of mature lymphocytes, lymphoid follicles, plasma cells, neutrophil and eosinophil granulocytes, histocytes and macrophages [2931]. Bi-
lateral involvement is considered to be a manifestation of chronic progressive immune-mediated generalized brosis, observed in cases with additional retroperitoneal brosis (M. Ormond) or in Erdheim-Chester disease [3234]. A classication into non-specic infections and infestations, and specic non-infectious inammation [1] is
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Fig. 9. Axial CT of a 14-year-old girl with painful, progressive exophthalmos of the right eye. Diagnosis: diffuse idiopathic orbital inammation. There is a diffuse scleral thickening of the entire circumference, distinct, hazy hyperdensity of the retrobulbar fat, diffuse swelling of the external rectus muscles, including the tendinous insertion.
helpful on pathological purposes, but in clinical practice a morphologic classication of idiopathic orbital inammation according to a (1) diffuse and (2) local form seems to be useful [4,6,35]. In diffuse idiopathic orbital inammation every orbital structure may be involved (Fig. 9), while in the localized form only one of the different parts of the orbit is affected (Fig. 10). CT- and MR-imaging characteristics are unspecic, including contrast enhancement, due to high vascularity of the inammatory process, inltration of the fat, proptosis, and an isolated or general, uni- or bilateral extraocular muscle enlargement [3]. Differential diagnosis to true orbital tumors may be difcult, but clinical characteristics with painful acute to subacute, mostly unilateral proptosis may help, especially in the acute form. If only one muscle is affected, diagnosis of myositis, the local presentation of an idiopathic orbital inammation is most likely (Fig. 10). Beside the mainly single muscle involvement, another characteristic sign, helping in differential diagnosis of myositis versus Graves disease, is the involvement of the tendon into the inammatory process, demonstrating on imaging as additional (or only) swelling and enlargement (Fig. 11). 3.1.2. Graves disease (syn. thyroid orbitopathy, M. Basedow) Dysthyroid endocrine orbitopathy is the most frequent cause of uni- or bilateral proptosis in adults, mainly associated with the autoimmune hyperthyroidism (synonym: M. Basedow or Graves disease) [3638]. As in the retroorbital
tissue, especially in extraocular muscles, TSH-receptors and thyrotropin receptor antibodies (TRAb) are found, these compartments seem to be the main target of this inammatory process [39]. Lymphocyte inammation, mucopolysaccharid and plasma cell inltration lead to tissue edema of all orbital structures, demonstrating not only enlargement of extraocular muscles, but corresponding increase of retrobulbar fat volume [40], while in more chronic stages, collagen deposition in the effected muscles result in brosis, and may be associated with fat deposition [41]. Apart from the classical clinical and ophthalmological symptoms, and endocrinological ndings, where elevated thyroid hormone levels were found even in clinical absence of hypderthyroidism [39] imaging is indicated for primary diagnosis as well as for control examination in the course of the disease. Spindle shaped spreading of typically more than one of the extraocular muscles (>4 mm) without involvement of the corresponding tendon (Fig. 12ac) [42], and the preferential affection of the inferior and medial rectus muscles, followed by the superior and lateral extraocular muscles, and the compression of the optic nerve in the orbital apex (crowded orbital apex syndrome [43]) are the most important morphological diagnostic criteria of endocrine orbitopathy. Although the morphological accuracy of CT is similar to MR, the latter is the most effective tool not only in establishing the initial diagnosis but also in the course of endocrine orbitopathy. MR enables the calculation of T2-relaxation time (Fig. 12h) [44,45], thus allowing the differentiation between an acute and chronic, brosing form, which is of greatest interest with regard to
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Fig. 10. MR of a 20-year-old female presenting with acute, painful proptosis of the left eye. Diagnosis: myositis of the left lateral rectus muscle. (a) Axial T1w midorbital view, where a swelling of the entire lateral rectus muscle is seen. (b) Corresponding T2w view with bright signal enhancement of the affected muscle. (c) The coronal T2 view shows an additional involvement of the superior rectus muscle and levator palpebrae complex (note the bright signal enhancement of both maxillary sinus mucosa as a sign of acute sinusitis).
the decision whether the patient may or may not benet from immunosuppressive and/or radiation therapy [46,47]. Surgical decompression with removal of different orbital walls is indicated in case of optic neuropathy and provides space for the enlarged muscles as well (Fig. 12f and g) [48]. Differential diagnosis of Graves orbitopathy includes lymphoma, metastasis, diffuse or focal idiopathic orbital inammation with mass effect, and vascular diseases like carotid-sinus cavernosus-stula (CCF) [49]. As already mentioned, the most important differential diagnosis is myositis, especially in rare cases of unilateral involvement of thyroid orbitopathy. 3.2. Solid tumors Peripheral nerve tumors account for about 4% of all orbital tumors with a preference to (plexiform) neuro-
bromas and schwannomas as the orbit is host of a great number of nerves including the motor branches of the cranial nerves III, IV and VI, and the sensorial branches of cranial nerve V, as well as sympathetic and parasympathetic nerves [50]. On imaging schwannomas demonstrate a well-dened intra- or extrakonal ovoid mass with varying degree of contrast enhancement [51], while (plexiform) neurobroma present with an irregular, often inltrating mass with marked enhancement, due to the high vascular component [52]. 3.2.1. Lymphoma Orbital lymphoma accounts for up to 55% of all malignant orbital tumors [53], including a heterogeneous group of neoplasms of the lymphoproliterative system with distinct entities dened by clinical, histological, immunological, molecular, and genetic characteristics [54].
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Fig. 11. MR of a 53-year-old female, suffering from painful double vision of the right eye. Diagnosis: myositis of the right superior oblique muscle. (a) Coronal PDw view with hyperintensity of the tendon of the right superior oblique muscle (black arrow), adjacent to the trochlea. (b) Corresponding T1w view, demonstrating the slight swelling of the right tendon (black arrow), compared to the left. (c) Axial T1w, conrming the thickening of the affected tendon (black arrow).
They include benign, atypical and malignant lesions, and most of those involving the orbit are B-cell lymphoma, although orbital lymphoma primarily belong to low-grade non-Hodgkin-lymphoma (NHL) or extranodal mucosa-associated lymphoid tissue (MALT lymphoma) [55]. The patients, mainly older than 50 years, present with painless, mostly unilateral proptosis and with or without motility disturbances or decrease of visual acuity, but displacement of the globe. The methods for the assessment of orbital lymphoma include ultrasound, CT and MR. As any orbital compartment may be involved (with predilection for the lacrimal gland), there is a wide spectrum of imaging ndings from well dened, round or lobulated, but not encapsulated mass to less dened inltrative process (Fig. 13) [55].
3.3. Vascular lesions Vascular lesions are an important and substantial component of orbital anomalies, but their classication, whether they are vascular tumors or vascular malformation is discussed controversially [56,57]. 3.3.1. Hemangioma Hemangiomas are the most frequent vascular tumors of the orbit. Capillary hemangioma, which consist of plump, rapidly dividing endothelial cells with lumina of varying size, pericytes and multi-laminated basement membranes, are seen in young children [56]. Present at birth they show a tendency of spontaneous involution, sometimes with complete disappearance within the rst 5 years of life [58].
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In case of extended orbital involvement there is a high risk of visual acuity decits as well as distortion of the orbital contents, eventually demanding more aggressive therapy [57], as surgery, laser treatment and/or administration of intralesional steroid or interferon therapy. On imaging they present as lobulated, irregularly marginated parenchymal mass, demonstrating a bright contrast enhancement (Fig. 14), especially on fat suppressed T1w MR images. Cavernous hemangiomas of the adult, predominantly arising in the intrakonal space, consist of enlarged, ectatic (cavernous) venous spaces, surrounded by a capsule of brous tissue [59]. The leading clinical symptoms are slowly pro-
gressing, painless, axial exophthalmos, occasionally with mild visual decits, due to optic nerve compression or extension by stretching; choroidal folds or papilledema are found in some 25% of patients. Imaging characteristics consist of a well outlined, round or oval, mainly intrakonal mass, that usually (but not always) spares the orbital apex (Fig. 15). Calcication, due to phlebolits are well detected by CT, on MR cavernoma present iso- to hypointense on T1w images with a generally extensive, but delayed contrast enhancement, while on T2w series the lesions exhibit a hyperintense homogeneous at times irregular signal. The main differential diagnosis is an orbital varix, which may also demonstrate a well-delineated mass, but may present with an intermittent
Fig. 12. A 61-year-old female presenting with bilateral axial proptosis and chemosis. Diagnosis: Graves disease. (a) Axial CT of the midorbital region, demonstrating the bilateral extraorbital location of both globes, and the lacrimal glands. Note the spindle-shaped enlargement of the medial and lateral external rectus muscles, but slim tendons. (b) Corresponding PDw view with moderate signal enhancement of the medial rectus muscles, corresponding to intramuscular edema. (c) Corresponding T1w view. (d) Coronal CT, where the involvement of all external muscles is apparent, including the superior oblique muscle. (e) Corresponding T1w MR. (f) Axial CT after surgical decompression. Note the increased size of the muscles, invaginating the entire former ethmoidal sinus. (g) Corresponding coronal view. (h) T2-time calculation in coronal view with bright signal of the external rectus muscles, indicating a recurrent inammation with edema (corresponding to the clinical presentation).
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Fig. 12. (Continued ).
proptosis on Valsalva maneuver. Hemangiopericytoma, brous histiocytoma and neurinoma should also be considered [60]. 3.3.2. Orbital venous anomaly The nomenclature of orbital varix is discussed controversially, since it is thought to be the same underlying abnormality as the venous lymphatic malformation, proposing the term orbital venous anomaly [61]. As orbital veins do not have valves, these abnormally dilated veins enlarge during systemic pressure changes, as coughing, forced expiration, or bending forward, resulting in variable proptosis, the main clinical symptom. On imaging, orbital venous malformation present as intrakonal, well-dened, triangular congured mass, tapering toward the apex, with highly intensive, homogeneous contrast enhancement (Fig. 16) [57].
3.3.3. Venous lymphatic malformation Occurring in the head and neck, predominantly in children and young adults, the venous lymphatic malformation (until recently called lymphangioma) is considered to be a vascular anomaly of unknown origin [57]. It contains abortive vessels, which spread among normal structures and present as an unencapsulated, primarily thin-walled mass with numerous cystic spaces of different size. It shows a tendency to spontaneous hemorrhages, resulting in a sudden onset of proptosis combined with periorbital swelling and reduced eye motility, at times leading to optic nerve compression [62], thus indicating prompt imaging as clinical symptoms may be similar to rhabdomyosarcoma. On imaging they present as an inltrative, multilobulated mass with poor encapsulation, found as well intra- as extraconal, harbouring sometimes calcication, seen on CT. Due to slowly growth, an asymmetric
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Fig. 13. CT of an 82-year-old woman with slowly progressing painless, unilateral, extra-axial proptosis of the left eye. Diagnosis: lymphoma: (a) axial and (b) coronal view with a clearly dened retro-and supra-bulbar mass, presenting slight enhancement. No separation of the external muscles is seen, but adaptation to the contour of the globe; the latter nding excludes the presence of hemangioma as a specic diagnostic criterion (with permission of Mller-Forell [15]).
Fig. 14. CT old, a 3-month-old boy with progressive exophthalmos developing in the second and third month of life, accentuated when screaming. Diagnosis: juvenile capillary hemangioma. (a) Axial contrast-enhanced view, where a homogeneous irregular mass is seen in the retrobulbar compartment of right orbit, involving the external muscles, but sparing the apex region. (b) The coronal view delineates the mainly inferior and medial location.
enlargement of the effected orbit may be seen (Fig. 17). The most important differential diagnostic criterion, demarcating it from cavernous hemangioma and/or idiopathic orbital inammation is the lack of contrast enhancement [63]. 3.4. Miscellaneous Thrombosis of orbital veins is characterized by an intravasal mass in the dilated (mostly) superior ophthalmic
vein. Depending on the imaging technique used, on CT, a primarily hyperdense, dilated vessel is seen without any contrast enhancement, while on MR, the appearance depends on the age of the thrombosis and the paramagnetic properties of haemoglobin [6]. Carotid-cavernous stula (CCF) represents an arteriovenous shunt of the internal carotid artery in its cavernous segment to the superior ophthalmic vein (respectively arterialized). The resulting venous congestion of the orbit causes hypervolemia, clinically manifesting as pulsatile exophthalmos, combined with dilated episcleral vessels, chemosis of different extent, secondary glaucoma, eventually papilledema, ocular pain, and ophthalmoplegia [64], which may lead to vision loss as a result of increased intraorbital
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Fig. 15. MR of a 53-year-old man with slowly progressing proptosis of the right eye, and a retrobulbar mass seen on ultrasound. Diagnosis: cavernoma. (a) Axial T2w midorbital view, demonstrating a hyperintense, solid, well dened mass in the right lateral intrakonal compartment. (b) Corresponding T1w native view, where a slight impression of the globe is seen, responsible for the development of choroidal folds. (c) Corresponding T1w contrast-enhanced view, where the impression of the posterior circumference of the globe is apparent (white arrow). (d) Midsagittal image of the right orbit, presenting the intraconal location, inferior to the optic nerve.
pressure. The etiology may be traumatic or spontaneous, the latter occurring primarily in diabetic older women. Pathophysiological classication of CCF into high-ow (type A) and low-ow (type B) stula (Fig. 18) is crucial in determining the denite therapy [65]. Color-coded ultrasound can denitely demonstrate reversal blood ow. The mentioned clinical symptoms with (mostly) unilateral proptosis, combined with imaging features of enlarged external muscles and widening of the superior ophthalmic vein is indicative for CCF. Although on both, CT- and MR-angiography a diagnosis can be done, digital subtraction angiography (DSA) remains the examination of choice, especially since in
type-A-stula an interventional therapy can be performed. Differential diagnosis of a dilated superior ophthalmic vein should include cerebral arteriovenous malformation (AVM) with atypical venous drainage [66]. 4. Extrakonal area 4.1. Inammatory lesions 4.1.1. Orbital infections Orbital infections, relatively common in children as a complication of paranasal sinusitis, are classied as
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Fig. 16. T1w contrast-enhanced (FS) MR of a 42-year-old man with recurrent exophthalmos. Diagnosis: orbital venous anomaly. A sharply dened, hyperintense lobulated mass is seen medial and lateral to the optic nerve, with some extension into the orbital apex (black arrow).
Fig. 17. CT of a 2-year-old boy, who suffered from an acute hemorrhagic exophthalmos of the right eye, suspicious for rhabdomyosarcoma. Diagnosis: venous lymphatic malformation. (a) Axial view of the midorbital region. A multilobulated mass is seen mainly in the medial superior orbit, with inferior dislocation of the globe and some calcication (not shown). Note the enlargement of the right orbit, compared to the left, due to long lasting intraorbital mass. (b) Corresponding contrast-enhanced view, where the choroid, basilar and carotid artery, and the cavernous sinus are seen hyperdense, but not the mass itself.
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Fig. 18. A 84-year-old diabetic woman presented with progressive exophthalmos and vision loss of the right eye. Diagnosis: low-ow carotis-sinus cavernosus stula (CCF). (a) Axial T1w contrast-enhanced (FS) midorbital view, where the protrusion of the right globe is apparent, as well as the thickening of the external muscles, and additional retinal detachment (white arrow), all due to venous congestion. (b) Axial T1w native view of the upper orbit, demonstrating the massive enlargement of the right superior ophthalmic vein (black arrow) compared to the left (white arrow). (c) Coronal PDw view with high signal intensity of the dilated right superior ophthalmic vein (black arrow). (d) Lateral view of the right internal carotid artery (ICA) (arterial phase) with digital subtraction angiography (DSA), with lling of the inferior part of the cavernous sinus (black arrow). (e) Corresponding early venous phase with the dilated superior ophthalmic vein, normally not seen.
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Fig. 19. Axial contrast-enhanced CT of a 2.5-year-old boy with febrile state and reddish swollen left eye and face. Diagnosis: preseptal cellulitis. The soft tissue inammation respects the orbital septum (small white arrow).
Fig. 20. Contrast-enhanced CT of a 4-year-old girl, suffering from chronic nasal sinusitis, acute presenting with febrile state and suspicion of infected mucocele. Diagnosis: subperiostal abscess of the left orbit. (a) Axial midorbital view, demonstrating the necrotic abscess between the lamina papyracea and the dislocated, swollen periorbita. Note the normal hypodensity of extraconal fat (white arrow) and the infection of the ispilateral ethmoid sinus. (b) The coronal view shows the infection of the entire ipsilateral sinuses, but no muco- or pyocele.
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Fig. 21. Contrast-enhanced CT of a 7-year-old boy with chronic sinusitis, actual presenting with progressive loss of consciousness and a febrile state. Diagnosis: subperiostal orbital and epidural intracranial abscess as orbital complication of nasal sinusitis. (a) Coronal view of the orbit, showing the irregular enhancing intraorbital extraconal soft tissue mass, and abscess of the left frontal sinus. (b) Axial view of the brain demonstrating an extraparenchymal uid with enhancing capsule (dura), with typical biconvex conguration of epidural mass. Some intracranial air conrms the connection with the extracranial space.
bacterial preseptal cellulitis, subperiostal abscess, orbital abscess, and cavernous sinus thrombosis [67]. Preseptal cellulitis, a bacterial infection of the skin and subcutaneous tissue, clinically presents as an erythema with severe pain, lid edema, chemosis, and pseudo-proptosis (Fig. 19). The orbital septum as a reection of the periorbita provides an
effective barrier against intraorbital infection, as does the periorbita at the lamina papyracea [67]. In an advanced stage postseptal soft tissue inltration along the orbital wall, and replacement of the fat and subperiostal space may lead to orbital phlegmone. Subperiostal abscess is mainly due to the spread of infection through the congenital dehiscences
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Fig. 22. A 25-year-old man presenting with unspecied pressure of the left orbit persisting for several months and a recent onset of double vision. Diagnosis: mucocele of the left ethmoid sinus. MR. (a) Axial PDw view with a hyperintense, well delineated, cystic structure of the left middle ethmoid region. (b) Corresponding T1w contrast-enhanced image, demonstrating pressure exerted on the periorbita and an attenuated, impressed, and dislocated medial rectus muscle. Note the hyperintense line of the wall of the mucocele in combination with the periorbita (small black arrow). (c) Coronal T1w view with intraorbital expansion and attening of the medial rectus muscle (black arrows), and dislocation of the superior oblique muscle (white arrow). CT: (d) corresponding coronal CT (bone window) with characteristic biconvex conguration of the ethmoid cell and thinned bony cortex (white arrows) ((b) and (d) with permission of Mller-Forell [15]).
and foramina of the thin orbital bones (Fig. 20) [67]. On contrast-enhanced CT, a sharply dened, extrakonal, space occupying mass with marginal ring-enhancement is seen. On MR, the subperiostal abscess is characterized by an intermediate signal on T1-weighted and proton density images, with marked rim enhancement on the corresponding contrast enhanced views. Intracranial extension may lead to severe complications as, e.g., epidural or subdural abscess (Fig. 21), purulent meningitis, or cavernous sinus thrombosis. 4.1.2. Muco-/pyocele Mucoceles are inammatory lesions of the nasal sinuses, most commonly resulting from inammatory obstruction of
the ostium of an affected sinus (primary mucocele), or as a result of posttraumatic, postoperative or neoplastic obstruction (secondary mucocele). They act as space occupying lesions, containing a mucous sac lined by mucous membrane. In case of superinfection the mucous retention is referred to as pyocele, presenting with a marginal contrast enhancement [4]. The characteristic presentation of a mucocele on imaging is the crescent-shaped, sharp and thinned remodeling of the bony wall of the affected sinus, combined with the mass effect to the extrakonal space (Fig. 22). The appearance of mucocele on MR may present in different ways, depending on the extent of hydration of the sinus secretion. A high signal of the sinus on T2w images (corresponding low signal on T1w) may be due to high free water content.
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Fig. 23. MR of a 14-year-old boy with initial complaint of double vision, followed by painless protrusion of the left eye after 1 week. Diagnosis: rhabdomyosarcoma. (a) Axial PDw image of the inferior orbit, showing slight hyperintensity of the tumor lateral to the left inferior rectus muscle. (b) Coronal T1w native view with only slight hypointensity of the tumor, compared to the inferior rectus muscle (black arrow).
Fig. 24. CT of a 60-year-old man with extra-axial proptosis of the right eye. Diagnosis: hemangiopericytoma. Spherical tumor of the right extraconal space, dislocating, but not inltrating the medial rectus muscle up to the optic nerve. Medially extension into the ethmoid sinus with bony destruction.
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Fig. 25. A 65-year-old woman, presenting with slight proptosis of the right eye, diffuse pressure in the orbital region, and difculty in nasal breathing for a period of several months. Diagnosis: esthesioneuroblastoma. (a) Axial contrast-enhanced CT, showing a large tumor occupying predominantly the right ethmoid and sphenoid sinus, and crossing the midline. Although the medial orbital wall is destroyed (black star), and intraorbital extraconal tumor expansion with medial dislocation of the normal sized medial rectus muscle is visualized, the presence of a small fat border (white arrow) indicates sparing of the periorbita. (b) Corresponding T1w native MR demonstrating the thin periorbita (small white arrow) and the extraconal fat (white arrow). Note the expansion to the bony optic canal. (c) Coronal contrast-enhanced CT, where the intracranial expansion is apparent, because of destruction of the frontal skull base. (d) T1w contrast-enhanced (FS) view demonstrating the entire cranio-caudal extension of the tumor not only with solid tumor part, reaching the endocranium, but inferior necrotic area in the nasal cavity. Note the hypointensity of the right maxillary sinus, simulating air in the sinus, but compared with the corresponding CT it contains inspisated mucous retention ((b) with permission of Mller-Forell [15]).
With increasing dehydration a high signal is seen on both T1w and T2w images, while in case of inspisated secretion the signal may decrease in both weightings, simulating normal air content (Fig. 25). In order to avoid false negative ndings, CT should be performed in clinically suspected mucocele [4]. 4.2. Tumors The extrakonal space is the rst orbital compartment that primary (e.g. osteoma, hemangiopericytoma) or secondary
(nasal/paranasal) orbital tumors invade on their way to deeper orbital or even intracranial structures. 4.2.1. Rhabdomyosarcoma Rhabdomyosarcoma (RMS) is the most common, highly malignant, soft-tissue tumor in childhood with up to 48% of all children less than 15 years of age, and a slight predilection for boys (5:3) [68,22]. Arising from primitive mesenchymal cells, four different subgroups of rhabdomyosarcoma can be divided histopathologically: (a) the embryonal rms, mainly arising in childhood, and
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Fig. 26. A 78-year-old man presenting with epistaxis and numbness of the left check. Diagnosis: carcinoma of the left maxillary sinus. (a) Axial native CT showing soft tissue in the inferior left orbit with poor distinction from the lateral rectus muscle. (b) Corresponding T2w MR, where necrotic tumor parts present with high signal. (c) Coronal T2w view. Although destruction of the orbital oor (including infraorbital canal (V2), responsible for the numbness of the left cheek) is apparent, the inferior rectus muscle (black arrow) is only dislocated but not inltrated. Note the big maxillary cyst of the right side. (d) Corresponding T1w native view identifying additional pronounced destruction of the maxillary sinus oor (white arrow) ((a), (c) and (d), with permission of Mller-Forell [15]).
representing the most malignant variant, (b) the pleomorphic rms, the tumor type of the adult, (c) the alveolar type of young adults, and (d) the botryoide rhabdomyosarcoma [69]. The characteristic clinical presentation is an extremely rapidly progressing exophthalmos in an otherwise healthy child, with dislocation of the eye, disturbance of motility, sometimes combined with unilateral ptosis and/or subcutaneous hematoma. On imaging the usually well-dened tumor is isodense/isointense to the orbital external muscles on CT/T1w images, demonstrating a marked enhancement, due to its high vascularisation, more prominent on fat-suppressed MR (Fig. 23) [6,35]. Rapid histopathological conrmation is mandatory, as modern chemotherapy was demonstrated to lead to successful remission and a
survival rate of 93% [70]. Differential diagnosis should include venous lymphatic malformation, lymphoma, and intraorbital neuroblastoma as well as idiopathic orbital inammation. 4.2.2. Various tumors (hemangiopericytoma, olfactory neuroblastoma, sinunasal malignancies, Langerhans-cell histiocytosis) Hemangiopericytoma, consisting of numerous sinusoidal vascular spaces, lined by endothelial cells, and surrounded by proliferating pericytes are very rare and uncommon orbital tumors [57], which recommend complete surgical excision, because of frequent recurrence [71]. Imaging demonstrates a circumscribed, generally
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Fig. 27. CT of a 74-year-old man with known lung-carcinoma presented with progressing exophthalmos of the right side. Diagnosis: metastasis of lung carcinoma. (a) Axial, contrast enhanced midorbital view, demonstrating the medial protrusion of the right globe due to a soft tissue mass in the lateral extra- and intraconal compartment with poor distinction of the lateral rectus muscle and/or lacrimal gland. The enhancing mass extends into the ispilateral temporal fossa. (b) The coronal view shows the inferior dislocation of the right globe, the tumor invades the endocranium with destruction of the orbital roof including the frontal bone. (c) The coronal view in bone window of the posterior orbital region exhibits the extended tumor inltration of the zygomatic, sphenoid, and fronto-temporal bone.
encapsulated markedly enhancing mass, in case of inltration of adjacent tissue, bone erosion is best seen on CT (Fig. 24). Olfactory neuroblastoma (syn. esthesioneuroblastoma) represent an uncommon malignant ectodermal tumor originating from bipolar olfactory receptor cells high in the mucosa of the nasal cavity, affecting both sexes with an approximately equal frequency at any age [72]. After long standing symptoms of nasal obstruction and anosmia, recurrent epistaxis leads to imaging, where the involvement of the orbit, and sometimes the endocranium represents an already advanced stage (Fig. 25). The orbit may be involved secondarily by any sinus lesion that is inammatory or neoplastic. Especially carcinoma, the
most common sinunasal neoplasms may not only inltrate the orbit, but extend to the endocranium (Fig. 26). Metastasis involvement of the orbit is known to be the rst manifestation of an occult primary malignancy, accounting for 1.58% of all orbital tumors [1], with preference of breast, lung (Fig. 27), and prostate carcinoma. Langerhans-cell histiocytosis (LCH) is a disease of unknown origin with variable clinical manifestation, ranging from non-progressive solitary eosinophilic granuloma to progressive and aggressive multisystemic involvement [73]. Solitary or monostotic eosinophilic granuloma is the most common presentation of LCH in children. Typically affecting the skull vault and presenting as well dened circumscribed mass, the lesion presents isodense/isointense to
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Fig. 28. MR of a 6-year-old boy who presented with slight proptosis and a rapidly growing mass of the left temporal region. Diagnosis: Langerhans-cell histiocytosis (LCH). (a) Axial T2w image with a hypo- to isointense mass in the temporal fossa and adjacent bone. (b) Corresponding T1w native view, where the inltration of the cortex of the sphenoid and temporal bone and intracranial, but extracerebral extension of the lesion is apparent (black arrow). Note the normal size of the left lateral rectus muscle. (c) Corresponding contrast-enhanced view with marked enhancement of the tumor. Note that parts of the periorbita (black arrow) are respected. (d) Coronal T1w native view, demonstrating the inltration of the temporal muscle (white star), explaining the suspected rst diagnosis of a rhabdomyosarcoma.
cortical gray matter on CT/T1w MR, with a marked contrast enhancement, while on T2w images it appears with a hypointense signal (Fig. 28) [22]. 4.3. Lacrimal gland Lacrimal gland lesions mainly present as unspecic enlargement of the gland, and can be divided into epithelial and non-epithelial lesions, the former including largely neoplastic disorders, while the latter consist of mostly congenital, inammatory, but also neoplastic disorders. 4.3.1. Congenital lesions The lacrimal gland represents the only organ of the extrakonal compartment, giving rise to different pathologic
processes, and demanding imaging only in some case of tumors, rarely in case of inammatory diseases. The most common congenital lesion is the dermoid cyst, arising from epithelial remnants of embryonal epithelial tissue, characterized by a slowly, painless growth. Containing fat, skin appendices (hair) or sebaceus gland [74], they present on imaging as well-circumscribed, round to ovoid encapsulated mass in the upper lateral orbit (Fig. 29). On CT the fatty components result in hypodensity, calcication may be seen. Sometimes a shallow impression of the bone, adjacent to the frontozygomatic suture, is apparent, where in some (rare) cases a small portion may extent into the temporal fossa, causing a so-called dumbbell-shaped orbital dermoid. On MR, the signicant content of fat results in a hyperintense signal both on T1w and T2w images, and distinct
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Fig. 29. CT of a 32-year-old woman with slight painless protrusion of the right eye. Diagnosis: dermoid of the right lacrimal gland. Slight enlargement of the right lacrimal gland, compared to the left side, with hypodense (fat) area (white arrow) in the anterior part.
Fig. 30. A 86-year-old woman with a long history of extra-axial proptosis of the left eye. Diagnosis: pleomorphic adenoma of the left lacrimal gland. (a) Axial contrast-enhanced CT with a predominantly solid, partly calcied encapsulated tumor of the left upper extraconal space, with signicant depression and attening of the anterior dislocated globe. (b) Corresponding T1w contrast-enhanced view with more distinct differentiation of the capsule and the cystic tumor parts (with permission of Mller-Forell [15]).
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Fig. 31. MR of a 35-year-old woman, who presented with painful protrusion of the right eye. Diagnosis: adenoidcystic carcinoma. (a) Axial T2w image of the upper orbit, demonstrating a hyperintense signal of the slightly enlarged right lacrimal gland with moderate impression of the upper globe. (b) Corresponding T1w native view, where an initial destruction of the fronto-zygomatic suture is seen (white arrow). (c) The coronal T1w, contrast-enhanced (FS) view demonstrates the marked contrast enhancement of the affected gland, compared to the left side. Note the impression of the globe (white arrow).
signal enhancement on diffusion weighted imaging (DWI) [75]. 4.3.2. Tumors The most common benign tumor of the lacrimal gland, producing an inferior and medially dislocation of the globe is the pleomorphic adenoma, affecting mainly middle-aged to old patients. This benign mixed, slowly growing tumor, originates mainly from the inner lobe of the lacrimal gland. Imaging features represent the histological conditions, as the tumor presents as a well circumscribed, encapsulated mass with heterogeneous densities/signals, due to myxoid, chondroid, or mucinous areas [76] (Fig. 30). The adenoidcystic carcinoma as the most malignant tumor of the lacrimal gland, account for 29% of all epithelial
lacrimal gland tumors [77], affecting patients of any age with a peak in the fourth decade and patients of either sex. They present with a hard mass in the upper lid, often associated with persistent pain, due to inltrative perineural growth, even in small tumors [1]. On CT or MR, a less circumscribed, nodular mass in the lacrimal fossa with additional bone erosion suggests adenoidcystic carcinoma (Fig. 31). The lacrimal gland is the at most touched intraorbital structure affected by the wide spectrum of lymphomatous lesions that range from reactive lymphoid hyperplasia, low-grade mucosa associated lymphoid tissue (MALT) lymphoma, to malignant lymphoma of various types, including the malignant NHL [78]. Mainly older patients present with a painless, slowly growing mass in the upper lid, leading to an inferior displacement of the globe.
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Fig. 32. CT of a 86-year-old woman with chronic blepharo-conjunctivitis of the left eye, known NHL for the past 9 years, actually presenting with progressing exophthalmos of the left eye. Diagnosis: non-Hodgkin-lymphoma (NHL). (a) Axial native midorbital view with diffuse involvement of the extraconal space, but no distinction of the lateral rectus muscle. (b) Axial contrast-enhanced view of the upper orbit, showing the involvement of the entire upper orbit, laterally and medially to the compressed superior rectus muscle, which presents slightly hypodense.
In some cases combined with conjunctival redness and/or visual impairment [76], a palpable smooth mass is seen in 50% of the patients, appearing as a pink, salmon-colored lesion. Lymphoma present on imaging as an inltrative, homogeneous mass of the lacrimal gland (Fig. 32), moulding adjacent structures without causing indentation. On MR, they show a moderate to marked hypointensity on T2w images, while on T1w views hyperintense signal compared to extraocular muscles is demonstrated with a marked contrast enhancement, especially in fat-suppressed images [6,54]. Inltration of the adjacent bones and intracranial extension is seen in malignant variants [6,76]. 4.4. Traumatic lesions Traumatic orbital lesions are manifold, reaching from small ssures, presenting only with orbital emphysema, to
severe osseous destructions. Certain orbital fractures occur with greater frequency: the most common are fractures of the orbital oor and medial walls (lamina papyracea) (Fig. 33), while those of the orbital roof (about 5%) (Fig. 34), lateral wall (Fig. 35) or complex orbital rim fractures are rather rare [79]. As already mentioned biplanar high-resolution/multislice CT with secondary reconstructions is the imaging modality of choice in orbital trauma, while MR is required in evaluating vessel injuries or in the detection of non-metallic foreign bodies [79]. In blow-out fractures (isolated orbital wall fractures without involvement of the orbital rim) a sudden increase in intraorbital pressure from a traumatic impact to the orbital soft tissue result in expansion and outward displacement of the thin orbital oor and/or fragile lamina papyracea. Restricted eye motility is due to incarceration of parts the ne network of brous septa that functionally unites the periosteum
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Fig. 33. CT of a 69-year-old man, who was involved in a free ght, presented with monocular hematoma and severe exophthalmos of the right eye. Diagnosis: orbital and subconjunctival emphysema, due to fracture of the lamina papyracea. (a) Axial view of the midorbital region demonstrates the subconjunctival emphysema. (b) Axial view of the upper orbit, where the orbital structures are fading over by the extreme intraorbital emphysema. (c) The coronal view shows the site of the fracture of the lamina papyracea (white arrow), as source of the connection of the nasal/paranasal cavity with the orbit.
of the orbital oor, the inferior brofatty tissue, and the sheaths of the inferior and oblique rectus muscle (Fig. 36). The so-called hanging drop represents a small local hematoma or brofatty displacement at the fracture site (Fig. 36).
5. Optic nerve 5.1. Inammatory lesions The term optic neuritis includes a variety of idiopathic, infectious or demyelinating diseases, clinically presenting (among others) with an acute uni- or bilateral loss of central visual acuity, central scotoma, loss of colour vision,
and an afferent pupillary defect with or without associated retroocular pain on eye movement and pathologic visual evoked potential (VEP) [80]. The most common underlying pathologies are multiple sclerosis, with optic neuritis as the rst symptom, and acute disseminating encephalomyelitis (ADEM). Infections, caused by various agents (e.g. cytomegalovirus, varicella zoster, mycosis or tuberculosis), and different autoimmune diseases (e.g. lupus erythematosus, autoimmune vasculitis) may also be responsible for optic neuritis [81]. Again, the imaging method of choice in optic neuritis as an intrinsic lesion is MR, as it demonstrates on one side the localization of the affected area, with preference to the intraorbital and intracanalicular portions of the optic nerve (Fig. 37), on the other hand, MR is able
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Fig. 34. CT of a 22-year-old man with state after a free ght and thrust with a knife-injury. Diagnosis: traumatic frontal encephalocele. (a) Axial view of the upper orbit, where a soft tissue mass is seen in the medial upper orbit, and defect (black arrow) with bony fragment of the medial orbital roof. (b) Coronal view, where the drop-like mass of the encephalocele (white arrow) dislocates the medial rectus muscle. (c) Corresponding bone window, demonstrating the wide defect of the skull base. (d) Axial view of the basal brain, showing a small intraparenchymal hematoma in the frontal lobe.
to dene the underlying cerebral pathology. In addition to increased signal intensity of the affected area on T2wand FLAIR-images, focal contrast enhancement is seen in the majority of patients after gadolinium not only in acute, but in even persistent neuritis, as an expression of blood-optic-nerve-barrier (BOB) disruption [81].
5.2. Tumors As the optic nerve is part of the central nervous system, two different types of optic nerve tumors should be differentiated, the intrinsic optic nerve glioma, and the extrinsic optic nerve sheath meningeoma.
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Fig. 35. CT of a 24-year-old man with state after a car accident. He presented with extreme swelling and hematoma of the right eyelid. Diagnosis: fracture of the lateral orbital wall. (a) Axial view of the midorbital region with severe swelling of the eyelid and subcutaneous area of the temporal fossa, but no sign of retrobulbar hematoma. (b) Axial view of the upper orbit, showing several bony fragments in the lateral extraconal space, and hematoma in the anterior part. (c) The coronal view in bone window demonstrates the intraorbital dislocation of the zygomatic bone fragments in the upper extraconal space.
5.2.1. Optic nerve glioma Histopathologically proven as (juvenile) pilocytic astrocytoma, this benign, slow-growing tumor of low radiation sensibility, may affect the visual system at different locations in different extension: on the optic disc, intraorbital, and intracranial, the latter with or without involvement of the chiasm. Optic nerve glioma account for 66% of all primary optic nerve tumors, and 4% of all orbital lesions [82], the preferred affected group are children and young adults, without any sex predilection [83]. The incidence in patients suffering from NF1 is rather high, as about 50% of patients with NF1 harbor optic nerve glioma, a bilateral disease considered to be pathognomonic [6]. Visual dysfunction is found in about 47% of the patients
[84], but the incidence of symptomatic optic pathway tumors is less than 20% [22]. Although CT may dene asymmetric enlargement of the affected optic nerve, MR should be the method of choice, as it is able to dene the lesion with superior contrast resolution and clear morphology. The question, whether the second optic nerve, and/or the chiasm and optic tract are involved additionally is answered best by MR. On T2w images, the effected nerve demonstrates homogeneous high signal compared to the contralateral unaffected nerve, due to the intraneural growth pattern with expansion of brovascular trabeculae by intra-axial astrocytic proliferation, combined with cystic degeneration (Fig. 38a). Another hyperintense portion, surrounding the isointense compressed nerve should not be
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Fig. 36. CT of a 20 year-old man with double vision and acute proptosis of the right eye after kick-box training. Diagnosis: blow-out fracture of the orbital oor. (a) Axial view of the midorbital region, showing some small retrobulbar hematoma, without mass effect. (b) The coronal view demonstrates the piercing of the inferior rectus muscle by a bony fragment (white arrow), lateral to a prolapsus of orbital fat into the maxillary sinus (so-called hanging drop). Note the hyperdensities of small intraconal hematoma, compared to the left side. (c) Corresponding bone window, where the additional hematosinus (with uid level) is apparent.
Fig. 37. MR of a 21-year-old male with recurrent visual decit of both eyes. Diagnosis: left optic nerve neuritis of unknown origin. (a) Axial T1w native view. (b) Corresponding contrast-enhanced (FS) view with bright signal enhancement of the left pre-canalicular and canalicular optic nerve compartment (white arrow).
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Fig. 38. MR of a 4-year-old girl with known NF1, presenting with progressive proptosis of the left eye. Diagnosis: optic nerve glioma. (a) Axial T2w midorbital view with enlargement of the intraorbital compartment of the left optic nerve. The hyperintensity of the optic nerve sheath complex represents a combination of subarachnoid space and arachnoidal gliomatosis. (b) Corresponding T1w native view. (c) Corresponding T1w contrast-enhanced (FS) image, demonstrating the tumor expansion into the subarachnoid space. Note the impression of the optic disc (white arrow), visible expression of papilledema. (d) Coronal T1w native view with enlargement of the left optic nerve complex, compared to the right side.
mixed up with a widened subarachnoid space, as it represents the perineural arachnoidal gliomatosis suggestive for patients with NF1 [85] (Fig. 38a and c). T1w pre- and postcontrast images, the latter with fats-suppression technique (Fig. 38c), delineate best subtle blood-brain-barrier (BBB) disruption in the optic nerve itself and/or in the course of the visual pathway (chiasm, optic tract, and hypothalamus). Additional intrinsic cerebral lesions, seen in NF1-patients with optic nerve glioma are astrocytoma, with preference to the brainstem [86]. Differential diagnosis of optic nerve glioma should include demyelinating optic neuritis, idiopathic inammation of the optic nerve, optic nerve sheath meningeoma, sarcoidosis, and even metastasis.
5.2.2. Meningeoma of the optic nerve sheath Unspecic clinical symptoms of slowly progressing, painless visual loss and unilateral proptosis or transient visual obscuration in middle-aged adults (mean 40 years), with a female predominance, are seen in patients with optic nerve sheath meningeoma. This primarily benign, slowly growing neoplasm arises from arachnoid cap cells within the optic nerve sheath complex, or from some intraorbital portion of sphenoid wing meningeoma [87] (Fig. 39). Optic nerve sheath meningeoma present in three different patterns [6]: (1) diffuse, with thickening of the optic nerve sheath complex (Fig. 39), sometimes with tubular calcication of the nerve sheath (the so-called tram-track-sign),
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Fig. 39. MR of a 81-year-old woman with headache since years, known visual loss of the right eye, but progressive visual decit of the left eye. Diagnosis: optic nerve sheath and sphenoid wing meningeoma of the right side with compression of the chiasm. (a) Axial T1w native view of the midorbital region with delineation of an enlargement of the right optic nerve sheath complex. (b) Corresponding contrast-enhanced (FS) image, demonstrating not only the signal enhancement of the optic nerve sheath but an enlargement and enhancement of the ispilateral cavernous sinus too (white arrow). (c) Coronal T1w native view of the midorbital region, showing the different diameter of both optic nerves. (d) Coronal T1w contrast-enhanced (FS) view of the region of the optic canal. Note the enhancement of the right optic nerve in the canal (long white arrow), compared to the left (short black arrow), and the dural enhancement of the sphenoid plane (black star). (e) Coronal T1w contrast-enhanced view at the level of the optic chiasm, demonstrating not only the compressive dislocation of the chiasm to the left side (white arrow), but the tumor involvement of the right cavernous sinus and skull base.
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Fig. 40. MR of a 73-year-old woman with progressive visual decit of the right eye. Diagnosis: tubular optic nerve sheath meningeoma. (a) Axial T1w contrast-enhanced (FS) view of the midorbital region, demonstrating the tubular growth of the meningeoma (so-called tram-track-sign) of the right optic nerve sheath. (b) Corresponding coronal view, where the compressed optic nerve is seen as a centrally located hypointense area (white arrow) (with permission of Radiologen am Brand, Mainz).
Fig. 41. MR of a 55-year-old woman with slowly progressing axial proptosis of the right eye, examined for possible endocrine orbitopathy. Diagnosis: eccentric meningeoma of the optic nerve sheath. (a) Axial PDw midorbital view with a well-dened intraconal mass of isointense signal. (b) Corresponding T1w native view, demonstrating a homogeneous, apparently encapsulated mass. (c) Corresponding contrast-enhanced (FS) view with homogeneous, but intermediate signal enhancement. (d) Coronal T1w contrast-enhanced image demonstrating the encapsulation of the nearly normal sized optic nerve (with permission of Mller-Forell [15]).
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best, but not exclusively seen on CT (Fig. 40), (2) fusiform, with circumscribed swelling of the sheath complex or (3) as an eccentric tumor (Fig. 41). The imaging method of choice again is MR, as it provides not only a high sensitivity and specicity (despite its lower sensitivity for calcication) but any intracranial extension via the optic canal can be proved or ruled out, even in small and en-plaque growing tumor matrix. On T2w images, the tumor presents with iso- to slightly hyperintense signal, on T1w images it is isointense compared to the external muscles. Due to high vascularity and the absence of a BBB, the demonstration of a homogeneous, moderate to marked signal enhancement of the tumor, especially in fat-suppressed sequences is signicant, clearly delineating the tumor from the compressed, isointense optic nerve (Fig. 41d). The subtle analysis of imaging criteria concerning the relation to adjacent/involved structures may help in sometimes difcult differential diagnosis of cavernous hemangioma and eccentric optic nerve sheath meningeoma. 5.3. Trauma Single traumatic lesions of the optic nerve are rare, as in traumatic disorders of the face and orbit, the optic nerve is only one of multiple structures involved.
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European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

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W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

W. Mller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105142

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