The patient came 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.

MRI 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 for evaluation.

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.

Smooth postoperative 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 upward.

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 consistent with diffuse low-grade lymphoma of B-cell origin.

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 approach.

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.