All anatomic
structures of the orbit can give rise to neoplasia.
Fortunately orbital tumors are very rare. There are over 1500
different tumors that can affect the orbit. The majority of
these tumors are benign. Occasionally, a malignant tumor may
involve the orbit primarily or through spread from an adjacent
or distant tumor. These lesions not only cause problems
because of their proximity to vital structures, but also the
risk of spread to adjacent and distant organs. Direct
extension from contiguous anatomical structures,
lymphoproliferative disorders, and hematogenous metastasis
result in orbital invasion.
Surgical anatomy:
The orbit in the broadest sense describes the cavity
containing structures essential for ocular function and the
bony architecture that encases them and resembles a pear, with
its widest aperture anterior and narrowing posteriorly. It is
an anatomically complex structure containing the globe,
extraocular muscles, fat, vascular, nerve, glandular, and
connective tissues.
The apex is formed by the optic canal and superior orbital
fissure. The roof is made up of the frontal bone. The maxilla
and zygoma form the floor. The lateral wall is made up of the
zygoma and the greater wing of the sphenoid. The maxilla, the
lacrimal bone, and the ethmoid bone contribute to the medial
wall.
The optic canal is about 10mm long, 5mm wide, and 5mm in
height. It extends anteroinferolaterally at an angle of about
40 degrees to the sagittal plane from the optic foramen. The
upper root of the lesser wing of the sphenoid forms the roof
of the canal. Medial border is by the sphenoid sinus and the
ethmoid air cells. The optic strut, a bone ridge joining the
lesser wing to the body of the sphenoid bone, forms the
inferior lateral border of the canal. The superior orbital
fissure is retort shaped with the broad end placed medially. A
fibrous ring, the annulus of Zinn, surrounds the optic canal
and the medial dilated part of the superior orbital fissure.
The intracranial archnoid continues as a discrete structure
through the optic canal and fuses with the pia at the globe.
At the orbital portion of the optic canal, the pia and the
archnoid are fused dorsomedially and ventrally with the dura
and the fibrous annulus of Zinn. The intracranial dura
continues through the canal as a dural-periosteal layer and
then separates into the dura of the optic nerve, and the
periorbita. At the apex, the six extraocular muscles oigin
from the annulus of Zinn. The levator muscle arises from the
upper medial margin of the annulus, and the superior rectus,
lying immediately beneath the levator, arises from the
superior portion of the annulus. Medial rectus is more medial
and inferior. The annulus loops widely around the nerve,
laterally and inferiorly, giving rise to the lateral rectus
which has two heads. The muscles broaden as they pass forward
to form a cone.
The annulus of Zinn envelops the optic foramen and the
medial aspect of the orbital fissure. The portion of the
orbital apex enclosed by the annulus is called the oculomotor
foramen. This foramen transmits the superior oculomotor, the
inferior oculomotor, the abducens, and the nasociliary nerves.
They remain inside the muscle cone. The trochlear, frontal,
and lacrimal branches of the 5th nerve and the
superior ophthalmic vein pass through the orbital fissure.
The ophthalmic artery, branches off from the ICA, just
above the cavernous sinus. It passes in the optic canal
lateral and inferior to the optic nerve. It provides the major
supply to the optic nerve. As it enters into the orbit, it
becomes more medial, and 8-15 mm behind the globe it gives off
the central retinal artery that penetrates into the medial
midportion of the optic nerve to supply the retina. The
primary venous drainage is through the superior and inferior
ophthalmic veins. The intraorbital optic nerve is about 30mm
long, 5mm longer than distance from the posterior margin of
the globe to the orbital apex.
On unroofing the orbit, the frontalis nerve is visible
through the periorbita. On opening the periorbita, the
frontalis nerve is seen overlying the levator and superior
rectus muscles. In the same plane, lies the trochlear nerve,
which crosses from lateral to medial above the optic nerve.
The nerve is approached medially between the dorasal superior
rectus and the medial rectus muscles. This obviates potential
trauma to the nerves passing through the oculomotor foramen.
In the orbital apex, the optic nerve is approached laterally
so as not to jeopardize its blood supply.
Clinical features:
Progressive proptosis is the most common symptom.
Apical tumors and those within the muscle cone push the
eyeball forwards (axial proptosis). Extraconal tumors displace
the eyeball in the opposite direction. A hyperostosing
sphenoidal meningioma produces prominence of the lateral wall.
Pulsatile exophthalmus in neurofibromatosis suggests a defect
in the sphenoid bone. The tumor may be palpated.
Diplopia with limitation of extraocular movements is
also common. Commonly, it is be due to mechanical factors.
Muscle infiltration or motor nerve palsies may also result in
diplopia. The eye can be rotated freely by the examiner in
nerve palsies after anesthetizing the conjunctiva.
Progressive visual loss may be the presenting symptom
in some. It may be transient and only in certain directions of
gaze. The unilateral visual loss may be detected by the
patient at a late stage. Testing for color vision may detect
an early visual defect.
Pain is not common and is a late manifestation in
malignancy. Painful paresis of one or more ocular nerves point
to a cavernous sinus lesion.
Chemosis suggest an inflammatory lesion or carotico-cavernous
fistula (CCF), and rarely in a malignant lesion. A bruit may
suggest a CCF. Intraorbital AV fistula and hemangioma can also
produce a bruit.
Pupillay abnormalities due to isolated sympathetic or
parasympathetic nerves as they pass through superior orbital
fissure are unusual.
Ophthalmoscopic examination will demonstrate either
papilledema or optic atrophy. Chronic compression of the
central retinal vein redirects retinal blood to the choroids
via a pre-existing system resulting in optociliary shunts. A
retroocular striae may suggest a lesion deforming the orbit.
Investigations:
MRI is the imaging of choice. It is particularly
valuable in assessing the orbital pathway because of the high
degree of sensitivity of fat tissue, changes of hydration
within the soft tissue, and lack of ionizing radiation.
Gadolinium MRI adds to better delineation.
A CT may help t o visualize the bony involvement
better. Plain x-rays have become obsolete.
Carotid angiography helps in evaluation of CCF and
orbital AV fistula.
Duplex ultrasound is useful to assess the
hemodynamic flow in the ophthalmic and retinal arteries.
Management:
The management of orbital tumors greatly depends on such
factors as tumor type, tumor location, patient age, and
vision. However, the following generalizations can be made.
Orbital inflammatory syndrome (orbital pseudotumor) may be
amenable to steroid treatment.
If the orbital lesion is discrete, then surgical excision
may be curative. Examples of discrete lesions include
cavernous hemangioma, unruptured dermoid cysts, neurofibromas
and schwannomas.
If the orbital lesion infiltrates the tissues, complete
excision may not be possible without harming the eye.
Examples of infiltrative lesions include lymphoma,
lymphangiomas, and orbital metastases.
Depending on the type of tumor, further surgery, radiation,
chemotherapy or a combination of the aforementioned treatments
may be required.
Vascular orbital tumors composed of large blood vessels are
difficult to address surgically because of their tendency to
bleed. Some vascular lesions of the orbit are amenable to
embolization with the aid of an interventional radiologist
Surgery:
Restoration of the proptosed eyeball to is
normal position with preservation of vision and ocular motor
function and cosmesis are the main goals of orbital surgery.
Surgical spaces in the orbit are used to
define the location of the lesion.
The three surgical spaces are the
subpersiosteal, peripheral surgical and central surgical
spaces.
a) The subperiosteal space potentially
exists between the orbital wall and the periorbita. Frontal
and ethmoidal sinus mucocoels, epithelial tumors arising from
sinuses, orbital abscesses, dermoid cysts and metastases begin
in the subperiosteal space before encroaching onto the deep
orbital structures. b) The peripheral surgical space exists
between the periorbita and the extraocular muscles. Lesions
involving the peripheral surgical space are lymphangioma,
hemangioma, dermoid cyst, lacrimal gland tumors, metastatic
lesions or orbital varices. C) The central surgical space is
seen posterior to the eye-ball, within the muscle cone. In the
central surgical space cavernous hemangioma,
hemangio-pericytoma, neurofibroma and pseudotumor are the
lesions commonly seen.
The position within the surgical spaces and
the character of the lesion determine the specific choice. The
surgeon must use clinical and radiographic information to
decide on the simplest and safest approach to the orbital
lesion. The approach is designed according to the location and
nature of the lesion. Those with intracranial extension or
involvement of the apex are primarily the responsibility of
the neurosurgeon. Those with paranasal extension require
skullbase approach ideally. Lesions not involving the apex and
wholly within the orbit may be managed by an ophthalmologist
or a neurosurgeon.
Three routes are used in orbitotomy:
anterior, lateral and superior (transcranial). The orbit may
be approached by any route or by a combination of these.
Anterior approach: The majority of
orbital procedures can be carried out through an anterior
incision in skin or conjunctiva. More commonly, ophthalmic
surgeons use this approach. This approach is useful for biopsy
of lesions anywhere in the orbit or to remove well-defined
anteriorly located tumors. Access can be through conjunctiva
or skin. When approached through skin, the dissection may
either extraperiosteal or more directly through the orbital
septum. The main incision sites are superior, inferior, in
quadrants, medial and lateral or directly over a palpable
lesion.
There are three anterior approaches:
transconjunctival, extraperiosteal and transeptal.
Transconjunctival approach:
Some anterior periocular and intraconal
lesions can be approached by direct conunctival incision and
dissection. A rectus muscle may be disinserted to enter the
intraconal space and the retractors placed between the muscle
and the globe. In addition, the optic nerve may accessed by
this route where it can be operated upon following
disinsertion of the medial rectus muscle, with lateral
rotation and anterior distraction of the globe. This is a
particularly useful approach to optic nerve sheath
decompression for chronic papilledema.
Exraperiosteal approach:
The anterior extraperiosteal approach is
most useful for lesions occurring in the peripheral surgical
space adjacent to periosteum or arising from and involving
bone. In particular, lesions such as dermoid cysts are readily
accessible by this approach. The skin incision is usually made
just at the orbital rim and carried down to the periosteum,
which can then be incised and elevated. The extraperiosteal
space can then be safety and extensively explored. An
alternative route of access inferiorly can be by means of
subsciliary incision through skin and orbicularis muscle with
dissection along the plane of the orbital septum and incision
of the periosteum at the orbital margin. The entire floor of
the orbit can be easily explored.
For the most part, anterior orbitotomies do
not require bony resection. But some large superior orbital
lesions can be more readily accessed by temporary removal of
the superior orbital margin. A clearer view of the entire
superior orbital space can be gained this way. It is not
necessary to transect the supraorbital nerve when operating on
large superior lesions. The nerve can be distracted after
unroofing the bony canal or incising the overlying ligament at
the time of superior orbital exploration.
Larger explorations through the
extraperiosteal space usually require postoperative drainage
with a Penrose drain. It should be cautioned that the
extraperiosteal approach should not be utilized in biopsy of
suspected malignant intraorbital lesions because the
periosteum provides a barrier to regress of malignancies.
Trans-septal approach:
Trans-septal route involves entry into the
orbit through the orbital septum leaving the periosteum
intact. This approach is indicated for biopsy of most
unresectable orbital malignancies. Anteriorly placed small
tumors can be removed through this route. Incision can be made
anywhere along the inferior orbit, but lacrimal sac must be
avoided medially. Superior incisions have to avoid the
supraorbital and supratrochlear nerves. Skin incision is made
over the preseptal orbicularis within the orbital rim. In the
lower lid a subciliary incision may be used in younger
patients. Orbicularis is opened and separated. Traction
sutures are put to promote exposure and hemostasis. After
identifying the orbital septum, gentle pressure is applied
over the upper lid which produces a forward displacement of
the orbital fat and septum. The septum is opened and extended
both medially and laterally. Orbital fat is displaced with a
malleable retractor to locate the lesion. The trans-septal
approach can be accessed through the relaxation lines around
the eye.
Lateral approach: In 1889, Krönlein
first described the lateral orbitotomy approach. This approach
is used less often these days. Lateral orbitotomy provides the
best access to reach the posterior lesions both within and
outside the muscle cone. Ideally, the lesions lateral to the
optic nerve and the apex are dealt with by this approach. The
amount of bony excision can be customized to include more or
the superolateral orbital rim, and even the zygomatic arch
when necessary, depending on the size and location of various
lesions. Most retrobulbar and parabulbar lesions can be
handled by an anterior or lateral orbitotomy alone or in
combination.
The patient is positioned with the head
slightly elevated and minimally rotated in the direction
opposite the operating side. Two types of incisions are
advocated to reach the lateral orbit. The Wright
incision (a curvilinear incision, extending from the lateral
half of the eyebrow to the zygomatic arch, anterior to the
hairline) allows a greater access to the lacrimal gland fossa
tumors and lesions in the superior and posterior quadrants.
The length of the incision can be adjusted depending on
location and extent of the orbital mass. Dissection is carried
down to expose the periosteum and temporalis muscle. The Berke
incision involves a 3 to 5 cms horizontal incision after a
complete lateral canthotomy. The upper and lower limbs of the
lateral canthal tendon are dissected completely from the
lateral orbital rim.
The periosteum is incised from the superior
aspect of the zygomatic arch to the zygomatioco-frontal
process. The periosteum and temporalis muscle are reflected
posteriorly. Stripping the temporalis muscle from the bony
fossa needs blunt dissection. Then the periorbita is elevated
from the inner orbital wall. Separation of periorbita from the
orbital rim to the apex must be done meticulously. After
protecting the globe with a malleable retractor, bony cuts are
made using a saw or chisel. Superior bone cut is above the
fronto-zygomatic suture and inferior cut is along the upper
margin of the zygomatic arch. The bony opening may be enlarged
posteriorly to the depth of temporalis fossa with a rongeur or
drill.
The periorbita is incised
antero-posteriorly and a vertical incision is made to form a
‘T’. Then the lateral rectus is identified and kept aside by
gentle traction suture or umbilical tape. On occasions, one
may have to do deeper intraconal dissections to expose a tumor
mass or to operate on the optic nerve.
For evaluation of the central surgical
space, especially if the lesion is small one, gentle traction
can be exerted on the suture placed into the stump of the
scleral insertion of the lateral rectus muscle. This maneuver
pulls the optic nerve into view without direct pressure by the
surgeon. The short ciliary arteries are readily seen and
should not be torn. It is important to remember that the
central retinal artery enters the optic nerve inferiorly about
10 to 15mm behind the globe. If the dural sheath is to be
opened, it is well to incise it on its anterolateral surface,
well away its vasculature.
Superior approach: The superior
approach is necessarily the domain of neurosurgeon. Dandy in
1921 laid the foundation for Neurosurgeon’s role in orbital
tumors with transcranial approach. The superior approach
indicated in compound trauma of the orbit and intracranial
cavity, decompression of the optic canal, or for removal of
apical or combined apical intracranial lesions. There are
three types of procedures used for this approach: Panoramic
orbitotomy (Fronto-orbital temporal approach), Frontal
approach, Supraorbital approach.
Fronto-orbital temporal (Panoramic)
approach:
This procedure allows for an en bloc
excision of the roof and lateral wall of the orbit and a wide
view of both the orbit and adjacent intracranial structure.
This approach consists of a coronal incision and removal of
the frontal flap in the usual manner followed by dissection of
the temporalis fossa and elevation of periorbita from the
adjacent bone. An incision is then made along the superomedial
wall of the orbit after distracting the frontal lobe from
above. The bony incision is extended along the roof of the
orbit to the lateral margin near the apex, whence the lateral
wall and frontozygomatic process are incised. With removal of
the bone a wide view of the roof and lateral orbital
structures is obtained. This approach is particularly useful
for excision of tumors at the apex of the orbit or for
combined intracranial orbital lesions such as tumor of the
optic nerve or sphenoid wing.
Frontal approach:
With the patient in the supine position a
bicoronal skin incision is made. A four burrhole frontal bone
flap is elevated after a subperiosteal dissection down to
orbital rim. If the tumour is confined to the orbit an
extradural approach is used. The dura is stripped from the
floor of the frontal fossa to expose the orbital roof. Orbital
unroofing is performed initially with a high-speed drill or a
chisel and then rongeurs are used. The optic canal, however,
is unroofed not with rongeurs, but only with a high speed
diamond drill. The orbitotomy extends medially to within 1.5cm
of the midline and laterally to within 1cm of the orbital
margin.
When orbital unroofing is complete, the
transparent periorbita displays the frontal branch of the
fifth nerve overlying the superior rectus and levator muscles.
The bony canal must be unroofed and annulus of Zinn incised
apically in the orbit. The best site for incision of the
annulus is medially between the superior oblique origin and
the levator palpebrae-superior rectus origins. The fourth
nerve can be seen coursing over these structures to its site
of insertion in posterior third of the superior oblique
muscle. It may be necessary to transect the fourth nerve in
order to deliver and optic nerve tumor. However, this can be
avoided by carefully dissecting the optic nerve within its
dural sheath from the annulus, transecting it intracranially,
and pulling it forward through the annulus into the orbit,
from which it can be removed following transection at the
globe and dissection from adjacent structures. Meningiomas or
optic gliomas are best approached by retracting the superior
rectus and levator muscles laterally. Dissection through the
fat is performed with small retractors and cottonoids. It is
possible to dissect the posterior ciliary nerves and vessels
within the orbit and avoid sectioning them while doing this
procedure. After excision of the tumor the orbital roof is
reconstructed with stainless steel mesh or bone taken from the
inner surface of the bone flap.
Supraorbital approach:
This approach ensures best exposure of the
apical portion of the orbit, with minimal or no retraction of
the frontal lobe. A bicoronal skin incision is used and the
scalp, including the periosteum, reflected anteriorly. At the
superior orbital ridge, the periorbita, which is continuous
with the periosteum, is separated from the surface of the
orbital roof. Two burrholes are placed, one in the midline at
the level of the orbital ridge and the other just behind the
arch of zygomatic process. The two burrholes are then
connected superiorly using a craniotomy to create the bone
flap. The bone flap so fashioned incorporates the superior
orbital rim and part of the orbital roof, thus allowing for an
excellent cosmetic closure. A further 3 to 4cms of the orbital
roof may then the removed using rongeurs. Minimally extradural
retraction of the frontal lobe allows easy access to the
superior and posterior orbit.
Combined approach: All of the
approaches defined above can be used in combinations or with
variations to obtain access to any of the surgical spaces of
the orbit. Widening bony incisions and even removing part or
all the sinus structures to expand the surgical space may
rarely be necessary.
Complications:
Transient complete or partial lateral and
superior rectus palsy occurs in almost all cases. Improvement
usually is seen within several days to 3 to 6 weeks and
recovery is complete by three months. Ptosis can be prevented
by gentle retraction of levator palpebrae superioris; if it
occurs, it usually resolves spontaneously. Visual loss can
result from injury to the optic nerve or due to central
retinal artery occlusion while dissecting medial to the optic
nerve in the apex. Perforation of the globe is another
potential risk. Postoperative hemorrhage will cause increasing
proptosis, ecchymosis, neuropraxia, and pain. The rapidity of
onset and development varies depending on the source of
bleeding. If the hemorrhage threatens ocular function (as
defined by decreasing vision with an afferent papillary
defect) or if it causes severe pain, prompt relief of orbital
pressure is necessary. CT scan or ultrasonography may help to
locate the blood pool. The decompression can be done through
the original incision and it may be enhanced if necessary by
means of alternate routed as for any decompression.
Orbital tumors in children:
The most common childhood tumors are benign and arise from
cystic orbital structures (dermoids) or abnormal blood vessels
within the orbit (hemangioma).
Capillary Hemangiomas: Capillary hemangiomas of
the orbit are benign vascular tumors, and are found almost
exclusively in children. They are the most common orbital
tumors found in children. Lined by vascular endothelium and
pericytes, these histologic benign lesions manifest at birth
or within the first 3 months of life, enlarge rapidly, and
begin to commence contracting around age 1 year. About 70% of
these tumors spontaneously decrease in size by seven years of
age. There are usually no other associated systemic
conditions. CT shows a large hyperdense, lobulated enhancing
mass. Total excision is impossible. The necessity of treatment
depends on whether there are ocular complications, such as the
development of amblyopia (lazy eye) or strabismus (crossed
eye). Most orbital capillary hemangiomas that cause secondary
ocular complications can be treated with steroids that are
either administered systemically, or injected into the tumor.
The prognosis is generally good.
Dermoid and Epidermoid Cysts: Dermoid and
epidermoid cysts are benign cystic structures which may be
present in the orbit, upper eyelid, or brow. They constitute
5% of the orbital tumors. They often progress very slowly. CT
shows a well circumscribed low density lesion. Most are
treated with surgical excision and the prognosis is good.
Total excision may be difficult.
Optic Nerve Glioma:
(discussed
elsewhere)
Rhabdomyosarcoma: Rhabdomyosarcoma, a
mesenchymal tumor, is the most common malignant orbital tumor
of childhood. These devastating lesions usually occur in
children younger than age 2 years or older than age 6 years,
and they have a predilection for the superior nasal orbit. The
average age of onset is about 6 years. This type of tumor
generally grow very rapidly, causing proptosis (forward
displacement) of the eye. It may destroy bone and enter
adjacent sinuses. CT reveals a solid homogeneously enhancing
tumor. A biopsy is required to confirm the diagnosis, but the
tumor cannot usually be removed surgically because of its
infiltrative nature. Once the diagnosis is made, the child is
treated promptly with radiation and chemotherapy.
Other malignant lesions include Burkitt lymphoma and
granulocytic sarcoma.
Metastatic tumors: Neuroblastomas, Ewing
sarcoma, Wilms tumor, and leukemias are the more common
metastatic orbital lesions afflicting children. Metastatic
neuroblastoma is an orbital tumor that may occur in children
with an adrenal gland tumor (neuroblastoma). In fact, 95% of
patients who present with this orbital tumor have a known
history of adrenal gland tumor. These children may present
with proptosis of one or both eyes and conjunctival or eyelid
hemorrhage. These tumors are usually treated with combined
radiation and chemotherapy.
Orbital tumors in adults:
| Cavernous
hemangiomas: They are the most common benign orbital
tumor. They are well capsulated. Histologically, large
blood-filled, endothelial-lined spaces with fibrous
interstitial tissue and smooth muscle are discerned. CT
reveals a well outlined hyperdense lesion with minimal
enhancement. Phleboliths (calcifications) may be seen. It
is isointense in ion with minimal enhancement. Phleboliths
(calcifications) may be seen.
It is isointense in T1 MRI and significantly
hyperintense to fat on T2. These lesions usually are well
tolerated by the patient and managed by conservative
therapy and reassurance, unless visual acuity or field
loss is found. Total excision is possible. Meningioma:
They arise from optic nerve dural sheath or periorbita.
Sheath meningiomas (5-6% of all orbital meningiomas)
arise from meningothelial cells present in the meninges
covering the optic nerve. Middle aged females are more
frequently involved. More commonly, they arise within the
intraorbital optic nerve sheath than within the canal.
Optic nerve sheath meningioma is the most common orbital
apical tumor and is the most common orbital tumor with
intracranial extension. (However, it is more common for an
intracranial meningioma to extend into orbit. Fibrous
dysplasia, giant aneurysms, and encephalocoeles can also
extend into the orbit). More often, they originate at the
cranial end of the optic canal between the optic nerve and
carotid artery and secondarily extend into the orbit. The
tumor may grow extradurally, resulting in early proptosis
or subdurally, resulting in early visual loss and
papilledema; proptosis occurs at a later stage. In
combined intra and extradural types there is progressive
visual loss with proptosis. Some of them are bilateral.
Lesions near the optic foramen or the optic canal produce
sclerosis of the bone or widening of the optic canal and
they may be seen in a plain x-ray. Sclerosis of the optic
foramen in a skull x-ray rules out a glioma. CT and MRI
delineate the tumor from the optic nerve.
The treatment is controversial as the tumor is slow
growing and the natural history is variable. Radical
excision will invariably cause loss of vision and is
better delayed until significant visual compromise.
Excision of the tumor involving the apex will also result
in a frozen eye. Subtotal excision and radiotherapy may be
an acceptable alternative. |
|
|
In the younger patients, these tumors are aggressive and
radical excision may be justified.
Periorbital meningiomas arise from the archnoid
cells in the superior orbital fissure and involve the upper
lateral quadrant of the orbit. Ectopic archnoidal cells within
the perineurium of a peripheral nerve have been postulated as
another source. Those involving the superior orbital fissure
can not be removed without damaging the nerves passing through
the fissure; subtotal excision and radiotherapy will be more
acceptable.
Lymphoma: It accounts for about 10% of all orbital
tumors, usually as a manifestation of systemic disease. It can
occur anywhere in the orbit and may mimic a pseudo tumor.
Circumscribed forms usually involve the lacrimal gland and the
diffuse form affects the muscle cone. There may be bone
destruction. CT shows variable morphology. On MRI it is hypo
intense with gadolinium enhancement.
Pseudotumor: It is a localized inflammatory disease
that mimics a tumor. There is no associated local or systemic
cause. The patient usually presents with rapidly progressing,
painful proptosis. They may be self limiting with or without
visual impairment. Recurrences are common. It is usually
unilateral. Bilateral ones must be differentiated from Grave’s
disease, or systemic collagen disease. CT reveals multifocal
involvement around tenon’s capsule, thickening of extraocular
muscles, and enlarged lacrimal gland. MRI shows nonspecific
widespread involvement. Occasionally, the diagnosis is by a
biopsy. 40-60 mg/day of prednisolone over weeks to months is
the usual treatment.
Hemangiopericytoma: It is from the pericyte of a
capillary and usually well encapsulated. Occasionally it is
infiltrative. The patient presents with slow painless
proptosis.
Lacrimal gland tumor: 5% of the orbital tumors are the
epithelial tumors of the lacrimal gland. Majority of them
are inflammatory. 50% of neoplastic ones are mixed ones
(painless proptosis) and the other 50% are carcinomas, mainly,
adenoid cystic carcinoma (painful proptosis). CT shows a mass
in the superotemporal quadrant. A period of conservative
therapy with anti-inflammatory drugs is indicated when
inflammatory swelling is suspected. Progressing lesion or the
presence of bony involvement warrants an en bloc excision.
Radical excision may be considered in malignancy.
Benign nerve sheath tumor: It could be neurofibroma,
schwannoma, or malignant sneurilemmoma. And may or may not be
associated with neurofibromatosis. They occur commonly in the
superolateral compartment and present with proptosis, more
marked in schwannomas than in neurofibromas. Total excision is
possible; however, total excision is impossible with plexiform
neurofibroma.
Mucocoeles: They are a common cause of proptosis. They
result from expansion of the sinuses secondary to blockage.
Pyococele results when infected. A smooth bulge in the bony
wall of the ethmoid or frontal sinus is characteristic.
Complete removal including the sinus mucosa and restoration of
drainage is essential.
Lymphangioma: It is slow growing tumor that
infiltrates the orbital tissue. Spontaneous intratumoral
hemorrhage can produce episodic proptosis. Total excision is
impossible.
Fibrous histiocytoma: This is the most common
mesenchymal orbital tumor. It is a benign, slow growing,
unencapsulated infiltrative tumor of the orbit, and may become
malignant. CT reveals a high density mass. Total excision is
advised.
Dermoid cyst, optic nerve glioma, granuloma, and
sarcomas are other benign orbital tumors in adults.
Metastatases: It is the most common malignant tumor of
the orbit. In adults, carcinomas of breast and the lung are
the most common source. Though proptosis is common, schirrous
carcinoma of the breast may produce retraction of the globe.
CT morphology is variable.
Adenocarcinoma, squamous cell carcinoma, lymphosarcoma, and
mixed malignant tumors are other common malignant orbital
tumors.