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22-APRIL-2010 HUSSEIN ABDEL-FATAH HAMDAN
57 YEARS INTRAORBITAL RETRO-BULBAR TUMOR OF THE RIGHT ORBIT.
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Preoperative MRI and CT-scan demonstrating the mass which is causing severe
proptosis, pushing the eyeball anteriorly, laterally and downward.
Check CT-scan the next day after surgery showing practically symmetrical
position of the eyeballs.
to the clinic 19-April-2010 complaining of
right exophthalmus for 9 years. The patient was
in prednisolone 20 mg and was under
control. The patient was by some advice, stopped
prednisolone after what the exophthalmus became
more severe with deterioration of vision
and progression of conjunctivitis.
of the brain done 17-April-2010 showing huge mass in
the right orbit 4 times bigger than the eye bulb,
pushing it down and medially. It was so big that the
optic nerve canal was wide and the superior orbital
fissure also and the adipose tissue was noted only
near the medial orbital wall. It was extending
outward reaching the lacrimal gland, which was
pushed outside the orbit. It was difficult to
establish the histological verification by MRI and
CT-scan done 20-April-2010 ruled out bony
destruction of the superior and lateral walls of the
right orbit and rabdomyosarcoma was suspected.
On examination: the
patient has decreased vision of the right eye
and conjunctivitis with non-painful severe proptosis
and exophthalmus of the
right eye. It was possible to palpate the extruded
abnormal lacrimal gland. It was hard to evaluate the
status of the muscles of the eye, but there was
function of all muscles with some limitation due to
proposed severe exophthalmus. Optic sheath
meningioma was also considered.
The patient was advised to
resume immediately prednisolone and was admitted
Tarsorrhaphy of the right eye was
performed after induction of aneasthesia.
Considering that his poor hair in the frontal region
bifrontal approach with reflection of the skin to
the face was achieved. Monofrontal osteoplastic
craniotomy was done with reflection of the
bone flap to the right ear. During that the frontal
sinuses were violated to make the approach flush
with the anterior fossa and prevent traction injury
to the frontal lobe. The dura was detached
from the the anterior fossa down parallel to the
trajectory of the olfactory bulb and tract to avoid
traction injury to the olfactory function and the
separation was proceeded posteriorly to the middle
fossa and the anterior clinoid. The superior orbital
bone was looking healthy and drilling of the
superior wall was achieved, so that 1 cm width of
the anterior orbital roof was left intact and
drilling was extended posteriorly until the right
anterior clinoid and the lateral was of the orbit
was also drilled down until reaching the inferior
wall margin. The superior orbital fissure was also
exposed. Palpation of the periorbital fascia
revealed that all the area was rubbery in
consistency and the was no meningiomatous matrix.
The fascia was incised longitudinally and it was
noted that there is no adipose tissue inside.
Separation of the rubbery lateral rectus muscle and
the superior oblique muscle and dissection between
the muscular structures did not reveal any separable
tumor mass. Instead, the muscles were diffusely
enlarged and with rubbery consistency and invaded by
the tumor. Frozen section studies confirmed the
presence of lymphoma. Using Inomed ISIS
HighLine 32 channel IOM, electrophysiological
studies of the of the involved tissues was carried
on. Using DNS stimulation, only the superior rectus
muscle was looking healthy and functioning well. All
other structures were severely involved in the
process. After meticulous study of any involved
structure, it was possible to resect parts of the
entirely non-functioning parts of the lateral rectus
muscle and the superior oblique muscle and pats of
the rubbery transformed submuscular
degenerated fat to achieve some decompression.
The lacrimal gland was not violated, nor the running
nerves. It was impossible to achieve satisfactory
acceptable decompression. The periorbital fascia was
left open intentionally and artificial dura was put
over the exposed parts of the right orbit, because
the original dura was tiny thin.
Routine closure of the wound.
recovery, and the patient sent to the ICU.
The patient the next day
is alert and cooperative and dressing done showing
regression of the proptosis, for what the
tarsorrhaphy construct was removed. The
patient claim that the visual function is better and
can move the eye to all direction except that he
cannot elevate the upper eyelid and cannot see
The final diagnosis was
that of fibroskeletal tissues and few nerve bundles
diffusely infiltrated by small lymphoid cells. The
latter are positive for CD20 but negative for CD3.
The proliferation index was about 4%. These data
diffuse low-grade lymphoma of B-cell
The patient has this
pathology for 9 years, which means that
malignancy is rarely expected. But malignant
transformation must be kept in mind.
The incidence of
intraorbital retrobulbar lymphoma is very
rare event. It seems that surgery cannot achieve
satisfactory resection of the tumor, but wide
decompression of the superior and lateral wall
and the superior orbital fissure could regain
some cosmetic results..
Wide exposure of the orbit
could make possible to perform intraoperative
studies of the involved structures and
excision of the the non-functioning parts and
preserving the acceptable functioning
structures. In this case 200 degrees around the
orbit was exposed and studied accordingly. This
could be done only through transcranial
Orbital pseudotumor is a
benign intraorbital process confined to the
orbit but extra orbital involvement can occur.
It is among the 3 rd most common orbital
diseases along with thyroid orbitopathy and
lymphoproliferative disorder and accounts for
5-10% of orbital processes. Clinically, orbital
pseudotumor has been categorized as myositis,
dacryoadenitis, anterior, apical and diffuse
process. Patients may present with diplopia,
conjunctival chemosis, proptosis or abnormal
computed tomography scan (CT-scan) findings.
Patients may also have associated optic
neuropathy. Diagnosis is based on careful
history, ultrasonography (U/S), CT-scan and
magnetic resonance imaging (MRI) studies which
may also provide prognostic information.
Treatment consists of systemic corticosteroids
in the form of oral or intravenous
administration. Confirmation is made by orbital
biopsy. In addition to radiation, cytotoxic
agents, immunosuppressant, IV immunoglobulin,
biological therapy, TNF-alpha inhibitor
monoclonal antibody and Mycophenolate Moftil
have been found to be useful in the management
of refractory orbital pseudotumor.
Understanding of the clinical features of
patients with orbital pseudotumor,
differentiating it from other orbital processes
by use of imaging techniques and timely
implementation of available treatment strategies
may help prevent visual loss and associated
morbidity from this condition. Our case has
long-standing process with lymphoid infiltrate,
which is confirmed by histology. For more
information please refer to
Imtiaz A Chaudhry et al.
Notice: Not all operative activities
can be recorded due to lack of time.
Notice: Head injuries and very urgent surgeries are also
escaped from the plan .