European Journal of Radiology 49 (2004) 105–142

Orbital pathology
W. Müller-Forell a,∗ , S. Pitz b
a Institute of Neuroradiology, Medical School University of Mainz, Langenbeckstrasse 1, D-55101 Mainz, Germany
b 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, inflammatory, 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 specific clinical symptoms. Their specific 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 inflammation; Retinoblastoma; Rhabdomyosarcoma; Metastasis; CT; MRI

1. Orbital pathology case of leucocoria or cataract MR-imaging (MRI) may be

Although more than 100 different clinical pathologies
may affect the orbit [1] only some require diagnostic imag-
ing. 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
inflammatory processes, trauma or malignant tumors (e.g.
rhabdomyosarcoma or neuroblastoma), while in the group
of elderly patients neoplasms represent the main patholo-
gies. 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 inflammatory and tumorous
diseases and some characteristic traumatic lesions of each
of these compartments.
2. Globe
required. Its high resolution is able to visualize not only the
pathology itself but accompanying retinal detachment and/or
effusion.
2.1. Inflammatory disorders
Although rare, the posterior scleritis represents the local
form of the idiopathic orbital inflammation, 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 inflammation presents
diffuse or localized, necrotic or granulomatous, and scleral
sequesters are found with leakage of proteinaceous edema
fluid into the interstice of the uvea and Tenon’s capsule [3,2].
CT- and MR-imaging of scleritis is characterized by dif-
fuse scleral thickening of different extent and infiltration of
the adjacent tissue with contrast enhancement, depending on
the extent of the inflammatory process (Fig. 1a–c). Nodular
scleritis does not show contrast enhancement [2,4], an im-
portant criterion in differential diagnosis to uveal melanoma.

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. Müller-Forell).
2.2. Tumors
The neoplastic diseases of the globe may arise from
different layers, the most significant diagnosis are the
retinoblastoma in childhood and the uveal melanoma in the
adult patients.

0720-048X/$ – see front matter © 2003 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ejrad.2003.10.011
106 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

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 inflammation (plus edema) of the right side. (b) Corresponding T1w native view with isointense signal
of the retrobulbar infiltration. (c) Corresponding T1w contrast-enhanced (fat suppressed: FS) view with bright signal enhancement (white arrow).
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142
Fig. 2. A 2-year-old boy with leukokoria of the left eye. Diagnosis:
retinoblastoma. (a) The axial CT demonstrates not only spots of calcifica-
tion but slight hyperdensity of the left vitreous body. (b) Corresponding
proton density (PDw) MR, where the tumor itself (white star) looks isoin-
tense, 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 fluid.
107
Fig. 3. A 2-year-old boy presenting with leukokoria and complete reti-
nal detachment of the left eye. Diagnosis: Coat’s disease. (a) Axial CT
with only slight hyperdensity of the entire left globe, which also demon-
strates 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 Müller-Forell [15]).
108 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

2.2.1. Retinoblastoma toma with or without pinealoma (trilateral retinoblastoma)

Retinoblastoma, a highly malignant tumor arising from
neuroectodermal cells of the retina, represents the most com-
mon intraocular tumor in childhood, striking more than 90%
children younger than 5 years of age, at an equal sex contri-
bution [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 [7–9].
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 ophthal-
moscopy may confirm tumors as small as 0.002 mm. Espe-
cially MR is able to define tumor extension in all aspects
of the globe, an eventually extraocular extension, intracra-
nial metastasis, and/or a second tumor. Bilateral retinoblas-
are found in about 25–35% 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 abil-
ity of three dimensional examination, combined with the
high resolution of potential intracranial pathology, especially
after injection of paramagnetic gadolinium [11–13], leav-
ing CT-examination with its demonstration of calcification
(Fig. 2a) only for cases of differential diagnostic problems.
MR is able to define the different growth patterns of endo-
phytic, exophytic of diffuse retinoblastoma [14,12]. On T1w
and proton density images retinoblastoma appears slightly
hyperintense compared to normal vitreous, the latter en-
abling a better visualisation of accompanying retinal detach-
ment, 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 deficit 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.
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 109

retinoblastoma exhibit a moderate to marked enhancement,
especially on fat-suppressed sequences (Fig. 2a–c) [6,13].
The most important differential diagnosis of retinoblas-
toma are lesions also presenting with leucocoria like per-
sistent hyperplastic primary vitreous (PHPV), and Coat’s
disease (Fig. 3) [15] while endophthalmitis, retinal detach-
ment, 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, fluorescein
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 con-
trol. 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 dif-
ferent 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 Bruch’s membrane
[6,19], a finding to direct one’s attention especially in early
stages (Fig. 5), as the therapy is different from that of ex-
clusively 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 deficit of the right eye. Diagnosis: choroidal melanoma with initial destruction of Bruch’s 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 (confirmed by histology).
110 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

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 defined 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 im-
ages, 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 heman-
gioma, choroidal nevi, choroidal detachment, neurofibroma,
disciform degeneration of the macula, and metastasis
[2].
2.2.3. Different retinal and choroidal lesions
Choroidal hemangioma is a congenital vascular hamar-
toma seen in middle-aged to elderly patients with neu-
rocutaneous syndromes, which may present solitary or
diffuse [2,22]. On CT/MR they present as lenticular, hy-
perdense/hyperintense (in both T1- and T2w-images) well
circumscribed structure in the juxtapapillary or macu-
lar region with a marked/intense contrast enhancement
[2,6,15]. It should not be mistaken with the capillary he-
mangioma in Hippel-Lindau syndrome, where imaging is
indicated in order to confirm/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 fibrous
scar tissue as intense as the enhancement of the external muscles (with permission of Müller-Forell [15]).
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142
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 fibrovascular elements, and mesenchymal
cells. On imaging it presents as a sharply demarcated,
lentiform calcification 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 mus-
cles 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 ophthal-
moscopy (e.g. extreme swelling of the eyelid) CT can visu-
alize 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 deficits. 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 penetrat-
ing 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 localiza-
tion of (mostly ferromagnetic) foreign bodies without risk
of dislocation. The scope of ocular injuries includes a great
111
variety of lesions, ranging from rupture of the globe to dis-
location of the lens, from vitreous (Fig. 7), subretinal, retro-
hyaloid hematoma to retroocular hemorrhages. In the sequel
of severe ocular trauma, phthisis bulbi refers to a scarred,
shrunken globe, possibly associated with dystrophic calcifi-
cation, as a type of developmental microphthalmia (Fig. 6)
[4].
3. Konal/intrakonal area
3.1. Inflammatory lesions
With more than 60% inflammation is the far most common
disease of the orbit, the majority of which are related to
thyroid orbitopathy [1].
3.1.1. Idiopathic orbital inflammation
Since the commentary of ROOTMAN [28] the term
orbital pseudotumor, routinely used for a diversity of dis-
orders, which represent the most frequent pathologic pro-
cess 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 specific
definition regarding the underlying pathophysiological and
clinical patterns. Idiopathic orbital inflammation is one of
the four major intraconal disorders, the other include infil-
trative, mass effect and vascular disorders. They all present
with different but specific clinical symptoms, requiring the
individual diagnosis in an individual case, following con-
sideration of specific finding 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 inflamma-
tory cases, but histological confirmation 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 tu-
mors, especially lymphomas are characterized by similar
presentation and therapeutic answer [29]. Histological cel-
lular components of idiopathic orbital inflammation present
extremely variable, consisting of mature lymphocytes, lym-
phoid follicles, plasma cells, neutrophil and eosinophil
granulocytes, histocytes and macrophages [29–31]. Bi-
lateral involvement is considered to be a manifestation
of chronic progressive immune-mediated generalized fi-
brosis, observed in cases with additional retroperitoneal
fibrosis (M. Ormond) or in Erdheim-Chester disease
[32–34].
A classification into non-specific infections and infes-
tations, and specific non-infectious inflammation [1] is
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142
Fig. 9. Axial CT of a 14-year-old girl with painful, progressive exophthalmos of the right eye. Diagnosis: diffuse idiopathic orbital inflammation. 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 classification of idiopathic orbital inflamma-
tion according to a (1) diffuse and (2) local form seems to
be useful [4,6,35]. In diffuse idiopathic orbital inflamma-
tion 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 unspecific, in-
cluding contrast enhancement, due to high vascularity of
the inflammatory process, infiltration of the fat, proptosis,
and an isolated or general, uni- or bilateral extraocular
muscle enlargement [3]. Differential diagnosis to true or-
bital tumors may be difficult, but clinical characteristics
with painful acute to subacute, mostly unilateral propto-
sis may help, especially in the acute form. If only one
muscle is affected, diagnosis of myositis, the local presen-
tation of an idiopathic orbital inflammation 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 inflammatory 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 associ-
ated with the autoimmune hyperthyroidism (synonym: M.
Basedow or Graves’ disease) [36–38]. As in the retroorbital
113
tissue, especially in extraocular muscles, TSH-receptors
and thyrotropin receptor antibodies (TRAb) are found,
these compartments seem to be the main target of this
inflammatory process [39]. Lymphocyte inflammation, mu-
copolysaccharid and plasma cell infiltration lead to tissue
edema of all orbital structures, demonstrating not only
enlargement of extraocular muscles, but corresponding in-
crease of retrobulbar fat volume [40], while in more chronic
stages, collagen deposition in the effected muscles result
in fibrosis, and may be associated with fat deposition [41].
Apart from the classical clinical and ophthalmological
symptoms, and endocrinological findings, 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 in-
volvement of the corresponding tendon (Fig. 12a–c) [42],
and the preferential affection of the inferior and medial
rectus muscles, followed by the superior and lateral ex-
traocular 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 accu-
racy 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,
fibrosing 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 benefit
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
inflammation with mass effect, and vascular diseases like
carotid-sinus cavernosus-fistula (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) neurofi-
bromas and schwannomas as the orbit is host of a great
number of nerves including the motor branches of the cra-
nial nerves III, IV and VI, and the sensorial branches of
cranial nerve V, as well as sympathetic and parasympa-
thetic nerves [50]. On imaging schwannomas demonstrate
a well-defined intra- or extrakonal ovoid mass with vary-
ing degree of contrast enhancement [51], while (plexiform)
neurofibroma present with an irregular, often infiltrating
mass with marked enhancement, due to the high vascular
component [52].
3.2.1. Lymphoma
Orbital lymphoma accounts for up to 55% of all ma-
lignant orbital tumors [53], including a heterogeneous
group of neoplasms of the lymphoproliterative system
with distinct entities defined by clinical, histological, im-
munological, molecular, and genetic characteristics [54].
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 115

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, confirming 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 lym-
phoma, 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 dis-
placement 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
findings from well defined, round or lobulated, but not en-
capsulated mass to less defined infiltrative process (Fig. 13)
[55].
3.3. Vascular lesions
Vascular lesions are an important and substantial compo-
nent of orbital anomalies, but their classification, whether
they are vascular tumors or vascular malformation is dis-
cussed 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 com-
plete disappearance within the first 5 years of life [58].
116 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

In case of extended orbital involvement there is a high
risk of visual acuity deficits as well as distortion of the
orbital contents, eventually demanding more aggressive
therapy [57], as surgery, laser treatment and/or adminis-
tration of intralesional steroid or interferon therapy. On
imaging they present as lobulated, irregularly marginated
parenchymal mass, demonstrating a bright contrast en-
hancement (Fig. 14), especially on fat suppressed T1w MR
images.
Cavernous hemangiomas of the adult, predominantly aris-
ing in the intrakonal space, consist of enlarged, ectatic (cav-
ernous) venous spaces, surrounded by a capsule of fibrous
tissue [59]. The leading clinical symptoms are slowly pro-
gressing, painless, axial exophthalmos, occasionally with
mild visual deficits, due to optic nerve compression or exten-
sion 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).
Calcification, 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 enhance-
ment, 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 inflammation with edema (corresponding to the clinical presentation).
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 117

Fig. 12. (Continued ).

proptosis on Valsalva maneuver. Hemangiopericytoma, fi- 3.3.3. Venous lymphatic malformation

brous histiocytoma and neurinoma should also be consid-
ered [60].
3.3.2. Orbital venous anomaly
The nomenclature of “orbital varix” is discussed con-
troversially, since it is thought to be the same underlying
abnormality as the venous lymphatic malformation, propos-
ing 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 ex-
piration, or bending forward, resulting in variable proptosis,
the main clinical symptom. On imaging, orbital venous
malformation present as intrakonal, well-defined, triangu-
lar configured mass, tapering toward the apex, with highly
intensive, homogeneous contrast enhancement (Fig. 16)
[57].
Occurring in the head and neck, predominantly in chil-
dren 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 perior-
bital swelling and reduced eye motility, at times leading
to optic nerve compression [62], thus indicating prompt
imaging as clinical symptoms may be similar to rhab-
domyosarcoma. On imaging they present as an infiltrative,
multilobulated mass with poor encapsulation, found as
well intra- as extraconal, harbouring sometimes calcifica-
tion, seen on CT. Due to slowly growth, an asymmetric
118 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142
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 defined retro-and supra-bulbar
mass, presenting slight enhancement. No separation of the external mus-
cles is seen, but adaptation to the contour of the globe; the latter finding
excludes the presence of hemangioma as a specific diagnostic criterion
(with permission of Müller-Forell [15]).
enlargement of the effected orbit may be seen (Fig. 17).
The most important differential diagnostic criterion, de-
marcating it from cavernous hemangioma and/or idiopathic
orbital inflammation is the lack of contrast enhancement
[63].
3.4. Miscellaneous
Thrombosis of orbital veins is characterized by an in-
travasal mass in the dilated (mostly) superior ophthalmic
Fig. 14. CT old, a 3-month-old boy with progressive exophthalmos devel-
oping 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.
vein. Depending on the imaging technique used, on CT, a
primarily hyperdense, dilated vessel is seen without any con-
trast enhancement, while on MR, the appearance depends on
the age of the thrombosis and the paramagnetic properties
of haemoglobin [6].
Carotid-cavernous fistula (CCF) represents an arteriove-
nous shunt of the internal carotid artery in its cavernous
segment to the superior ophthalmic vein (respectively arteri-
alized). The resulting venous congestion of the orbit causes
hypervolemia, clinically manifesting as pulsatile exophthal-
mos, combined with dilated episcleral vessels, chemosis
of different extent, secondary glaucoma, eventually pa-
pilledema, ocular pain, and ophthalmoplegia [64], which
may lead to vision loss as a result of increased intraorbital
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 119

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 defined 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. Patho-
physiological classification of CCF into high-flow (type A)
and low-flow (type B) fistula (Fig. 18) is crucial in determin-
ing the definite therapy [65]. Color-coded ultrasound can
definitely demonstrate reversal blood flow. The mentioned
clinical symptoms with (mostly) unilateral proptosis, com-
bined 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 di-
agnosis can be done, digital subtraction angiography (DSA)
remains the examination of choice, especially since in
type-A-fistula 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. Inflammatory lesions
4.1.1. Orbital infections
Orbital infections, relatively common in children as
a complication of paranasal sinusitis, are classified 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 defined,
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 calcification (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.
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 121

Fig. 18. A 84-year-old diabetic woman presented with progressive exophthalmos and vision loss of the right eye. Diagnosis: low-flow carotis-sinus
cavernosus fistula (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 filling 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 inflammation 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.
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 123

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 fluid with enhancing capsule (dura), with typical biconvex configuration of epidural mass. Some intracranial air confirms 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 reflection 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 infiltration 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
124 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

Fig. 22. A 25-year-old man presenting with unspecified 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 flattening 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 configuration of the ethmoid cell and thinned bony cortex (white arrows)
((b) and (d) with permission of Müller-Forell [15]).

and foramina of the thin orbital bones (Fig. 20) [67]. On
contrast-enhanced CT, a sharply defined, 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 correspond-
ing 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 inflammatory lesions of the nasal sinuses,
most commonly resulting from inflammatory obstruction of
the ostium of an affected sinus (primary mucocele), or as a
result of posttraumatic, postoperative or neoplastic obstruc-
tion (secondary mucocele). They act as space occupying le-
sions, 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 enhance-
ment [4]. The characteristic presentation of a mucocele on
imaging is the crescent-shaped, sharp and thinned remod-
eling of the bony wall of the affected sinus, combined with
the mass effect to the extrakonal space (Fig. 22). The ap-
pearance 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.
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 125

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 infiltrating the medial rectus muscle up to the optic nerve. Medially extension into the ethmoid sinus with bony destruction.
126 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

Fig. 25. A 65-year-old woman, presenting with slight proptosis of the right eye, diffuse pressure in the orbital region, and difficulty 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 Müller-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 nor-
mal air content (Fig. 25). In order to avoid false negative
findings, CT should be performed in clinically suspected
mucocele [4].
4.2. Tumors
The extrakonal space is the first 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
4–8% 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
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 127

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 floor (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 infiltrated. Note the big maxillary cyst of the
right side. (d) Corresponding T1w native view identifying additional pronounced destruction of the maxillary sinus floor (white arrow) ((a), (c) and (d),
with permission of Müller-Forell [15]).

representing the most malignant variant, (b) the pleomor-
phic 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 subcu-
taneous hematoma. On imaging the usually well-defined
tumor is isodense/isointense to the orbital external muscles
on CT/T1w images, demonstrating a marked enhance-
ment, due to its high vascularisation, more prominent on
fat-suppressed MR (Fig. 23) [6,35]. Rapid histopatholog-
ical confirmation 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
inflammation.
4.2.2. Various tumors (hemangiopericytoma, olfactory
neuroblastoma, sinunasal malignancies, Langerhans-cell
histiocytosis)
Hemangiopericytoma, consisting of numerous sinu-
soidal vascular spaces, lined by endothelial cells, and
surrounded by proliferating pericytes are very rare and
uncommon orbital tumors [57], which recommend com-
plete 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 infiltration of the
zygomatic, sphenoid, and fronto-temporal bone.

encapsulated markedly enhancing mass, in case of infiltra-
tion of adjacent tissue, bone erosion is best seen on CT
(Fig. 24).
Olfactory neuroblastoma (syn. esthesioneuroblastoma)
represent an uncommon malignant ectodermal tumor orig-
inating 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, re-
current 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 inflammatory or neoplastic. Especially carcinoma, the
most common sinunasal neoplasms may not only infiltrate
the orbit, but extend to the endocranium (Fig. 26). Metastasis
involvement of the orbit is known to be the first manifestation
of an occult primary malignancy, accounting for 1.5–8%
of all orbital tumors [1], with preference of breast, lung
(Fig. 27), and prostate carcinoma.
Langerhans-cell histiocytosis (LCH) is a disease of un-
known origin with variable clinical manifestation, rang-
ing 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 defined cir-
cumscribed mass, the lesion presents isodense/isointense to
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 129

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 infiltration 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 infiltration of the temporal muscle (white star), explaining
the suspected first diagnosis of a rhabdomyosarcoma.

cortical gray matter on CT/T1w MR, with a marked con-
trast enhancement, while on T2w images it appears with a
hypointense signal (Fig. 28) [22].
4.3. Lacrimal gland
Lacrimal gland lesions mainly present as unspecific en-
largement of the gland, and can be divided into epithelial and
non-epithelial lesions, the former including largely neoplas-
tic disorders, while the latter consist of mostly congenital,
inflammatory, 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 inflammatory diseases. The most
common congenital lesion is the dermoid cyst, arising from
epithelial remnants of embryonal epithelial tissue, charac-
terized by a slowly, painless growth. Containing fat, skin
appendices (hair) or sebaceus gland [74], they present on
imaging as well-circumscribed, round to ovoid encapsu-
lated mass in the upper lateral orbit (Fig. 29). On CT the
fatty components result in hypodensity, calcification 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 significant content of fat results in a hyper-
intense 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 calcified encapsulated tumor of the left upper extraconal space, with significant depression
and flattening 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 Müller-Forell [15]).
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 131

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 tu-
mor, originates mainly from the inner lobe of the lacrimal
gland. Imaging features represent the histological con-
ditions, 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 tu-
mor 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 as-
sociated with persistent pain, due to infiltrative 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, in-
cluding the malignant NHL [78]. Mainly older patients
present with a painless, slowly growing mass in the up-
per 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 infiltrative, ho-
mogeneous mass of the lacrimal gland (Fig. 32), moulding
adjacent structures without causing indentation. On MR,
they show a moderate to marked hypointensity on T2w im-
ages, while on T1w views hyperintense signal compared to
extraocular muscles is demonstrated with a marked contrast
enhancement, especially in fat-suppressed images [6,54].
Infiltration 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 fissures, presenting only with orbital emphysema, to
severe osseous destructions. Certain orbital fractures occur
with greater frequency: the most common are fractures
of the orbital floor 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 frac-
tures are rather rare [79]. As already mentioned biplanar
high-resolution/multislice CT with secondary reconstruc-
tions 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 involve-
ment 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 floor and/or fragile lamina papyracea. Restricted eye
motility is due to incarceration of parts the fine network
of fibrous septa that functionally unites the periosteum
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 133

Fig. 33. CT of a 69-year-old man, who was involved in a free fight, 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 floor, the inferior fibrofatty tissue, and the
sheaths of the inferior and oblique rectus muscle (Fig. 36).
The so-called “hanging drop” represents a small local
hematoma or fibrofatty displacement at the fracture site
(Fig. 36).
5. Optic nerve
5.1. Inflammatory 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 cen-
tral 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
first symptom, and acute disseminating encephalomyelitis
(ADEM). Infections, caused by various agents (e.g. cy-
tomegalovirus, varicella zoster, mycosis or tuberculosis),
and different autoimmune diseases (e.g. lupus erythemato-
sus, autoimmune vasculitis) may also be responsible for
optic neuritis [81].
Again, the imaging method of choice in optic neuri-
tis as an intrinsic lesion is MR, as it demonstrates on
one side the localization of the affected area, with pref-
erence 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 fight 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 define the underlying cerebral pathology. In addition 5.2. Tumors

to increased signal intensity of the affected area on T2w-
and 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].
As the optic nerve is part of the central nervous system,
two different types of optic nerve tumors should be differ-
entiated, 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 astro-
cytoma, this benign, slow-growing tumor of low radiation
sensibility, may affect the visual system at different loca-
tions 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]. Vi-
sual dysfunction is found in about 47% of the patients
[84], but the incidence of symptomatic optic pathway tu-
mors is less than 20% [22]. Although CT may define
asymmetric enlargement of the affected optic nerve, MR
should be the method of choice, as it is able to define
the lesion with superior contrast resolution and clear mor-
phology. 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 fibrovascular trabeculae
by intra-axial astrocytic proliferation, combined with cys-
tic degeneration (Fig. 38a). Another hyperintense portion,
surrounding the isointense compressed nerve should not be
136 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

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 floor. (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 fluid level) is apparent.

Fig. 37. MR of a 21-year-old male with recurrent visual deficit 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).
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 137

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) Ax-
ial 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 ar-
row), 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 rep-
resents the perineural arachnoidal gliomatosis suggestive
for patients with NF1 [85] (Fig. 38a and c). T1w pre- and
postcontrast images, the latter with fats-suppression tech-
nique (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 hy-
pothalamus). Additional intrinsic cerebral lesions, seen
in NF1-patients with optic nerve glioma are astrocytoma,
with preference to the brainstem [86]. Differential diag-
nosis of optic nerve glioma should include demyelinating
optic neuritis, idiopathic inflammation of the optic nerve,
optic nerve sheath meningeoma, sarcoidosis, and even
metastasis.
5.2.2. Meningeoma of the optic nerve sheath
Unspecific 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 calcifica-
tion of the nerve sheath (the so-called “tram-track-sign”),
138 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142

Fig. 39. MR of a 81-year-old woman with headache since years, known visual loss of the right eye, but progressive visual deficit 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.
 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142 139

Fig. 40. MR of a 73-year-old woman with progressive visual deficit 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-defined 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 Müller-Forell [15]).
140 W. Müller-Forell, S. Pitz / European Journal of Radiology 49 (2004) 105–142
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
specificity (despite its lower sensitivity for calcification) 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 homo-
geneous, moderate to marked signal enhancement of the tu-
mor, especially in fat-suppressed sequences is significant,
clearly delineating the tumor from the compressed, isoin-
tense optic nerve (Fig. 41d). The subtle analysis of imaging
criteria concerning the relation to adjacent/involved struc-
tures may help in sometimes difficult 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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