Chronic granulomatous inflammation

Presenting complaint:
Swelling of Right eye since 2000.

 Patient was alright prior to 2000 when he started developing proptosis of the eye. He underwent R orbitotomy in 2001 and histopathology showed granulomatous inflammation. The swelling increased after the operation. He then underwent repeat orbitotomy in 2002 and it was once again diagnosed as granulomatous inflammation, most likely fungal. Patient was sent to radiology department who have referred him back. There is no history of tuberculosis or thyroid eye disease. Positive history of headaches.

local Exam

Investigations
BT 3:30 CT 4:35 ABF : 76mg/dL B/Urea : 36mg/dL S/Creatinine : 0.7mg/dL Calcium : 142 meq Potassium : 4.3 meq Hep B & C negative Urine exam - NAD

Hb : 12.0 mg/dL TLC :8600 cells/mm3 Lukocyte : 66% Lymphos : 30% Monocytes : 2% Eosinophils 0.2% Blood Group : A +ve

 C-ANCA ACE Myco dot

Histopathology Report 12-05-09

Histopathology report 18-04-09

Sections reveal chronic inflammation with granulmoma formation and giant cells, both lLanghan s and foreign body type of giant cells. Seen. PAS strain is positive for septate fi. Tuberculosis cannot be completely excluded. Conclusion:
Right eye PAS +ve Suggestive of fungal granulomas.

Histopathology Report 32-06-07

Specimen Orbital Grow Biopsy Gross Features:
Specimen received fixed in formalin and consists of soft tissue piece which is already cut, measuring 3.2 x 1cm

Microscopic features:
Histopathologic examination of the section shows a fibrocollagenous tissue with chronic inflammatory cell infiltrate and numerous granulomata. No malignancy seen.

Opinion / Comment:
Granulomatous inflammation

CT scan Brain and Orbit 15-04-05

Findings:
Soft tissue density mass is seen in the Left orbit retrobulbar region causing proptosis of the eyeball recurrent mass. Ethmoid and maxillary sinuses are clear No intracranial extension is seen. Left orbit normal No definite Bone erosion is seen.

Impression:
Diagnosis Retrobulbar orbit recurrent mass

CT scan Oribt
An irregular shaped soft tissue density lesion , retrobulbar in Right orbital cavity, with proptosis of Right eyeball. Lesion restricted to the orbital cavity with no extension intracranial or into the para-nasal sinuses.

Impression:
Morphological features are suggestive of inflammatory process.

CT Scan orbit 03-07-08

Soft tissue density enhancing mass seen posterior to the right eye globe. The mass cannot be separated form the right optic nerve. Inferior and anterior rectus muscles of right side are normal. The right globe is normal however it is pushed anteriorly due to the mass lesion. There is deviation of the nasal septum with convexity towards the left side. Both maxillary, ethmoid and frontal sinuses are normal. Left orbit and orbital structures are normal. Visualized brain parenchyma is normal. Comment:
Reterobulbar mass right orbit when compared with the previous lesion has reduced in size.

CT scan orbit 16-06-07

CT Scan Obit 10-10-07

Ill defined soft tissue density msass lesion with proptosis. Shadowing with mucosal thickening of ipsilateral sinus. Impression:
The appearance suggestive of pseudotumor rather than metastatic lesion.

CT scan Brain and orbit 02-06-03

Post operative follow-up scan. Large expanded soft tissue density lesion involving pper quadrant Right orbit with significant proptosis. No evidence ofo intracranial rxtension. Differential:
Pseudotumor.

Excisional biopsy - R orbital growth 25-10-2002

An irregular partly incised firm gray mass measuring 2.3.5x3x2cms. Slicing reveals firm gray yellow cut surface. Sections reveal fragment of fibro-cellular and fibro-collanegenous tissue with moderate inflammatory cell infiltrates comprising of lymphocytes, plasma cells, few eosinophils and macrophag3e. In addition there are many granulomatous collectrions composed of multiple multinucleated Langhan s and foreign body type of giant cells and collections of lymphocytes and macrophages. At the periphery fibro-fatty tissue, skeletal muscle fiber and multiple nerve bundles are seen. No epitheloid cells are seen. There is no evidence of malignancy. These appearances are of chronic granulomatous inflammation. Special stains for fungus reveal fungal hyphae.

Histopathology report 05-05-03

With large comments

CT scan 14-02

Bone Scan
No scintigraphic evidence of any bone pathology.

CT Scan Brain and Oribt 14-02-09

Soft tissue density mass lesion of right eyeball, retrobulbar area, is noted clearly in separate from posterior aspect of sclera, optic nerve and extraocular muscles, optic canal on ipsilateral side is normal. Significant proptosis of right eyeball is noted. Contralateral eyeball, both nasal cavities mastoid air cells, are appreciated to be normal, visualized brain parenchyma is normally appreciated, Bilateral frontal, ethmoidal, sphenoidal and maxillary sinuses along with nasal cavities appear normal. Impression:
Soft tissue density mass R Reterobulbar area (with above said extension)

 Histological sections of the sections reveals ocular tissue comprising of fibrocollagenous and fibrofatty tissue with dense infmlammatory cell infilt5rate comprising of lymphocytes. In addition there are granumuloms composed of multinucleated Langhan s and foreign body type o fgiant cells Granulomas are also seen in the peripheral adipose tissue and extraocular muscle. No caseation seen No vasculitis seen The features are strongly suggestive of chronic ranulomatous inflammation. Comments:
Most likely fungal granulomas.

Management

Plan
Enucleation radiotherapy

Spaces of the Retrobulbar Orbit

Cone:
Composed of the four rectus muscles and the thin intramuscular membrane which joins them and extends posteriorly to the insertion of the muscle tendons at the orbital apex.

Spaces of the Retrobulbar Orbit

Intraconal space:
Contains CN II, ophthalmic artery, superior division of CN III, nasociliary nerve (V1), inferior division of CN III, and CN VI.

Extraconal space:
Contains ophthalmic vein, lacrimal nerve (V1), CN IV and frontal nerve (V1).

Other Locations.

The lacrimal gland and lacrimal sac as well as the potential for multiple compartment involvement.

F: Frontal bone with associated suture and notch. <- Trochlea <- Optical Canal S: Superior orbital fissure I: Inferior orbital fissure L: Lacrimal bone E: Ethmoid bone.

Bony Orbit

Separation of Lesions by Anatomic Subsite

Evaluation - Review
Detailed recording of onset, duration and progression of the orbital disease. History of allergies, sinus infections, epistaxis, nasal discharge, and tearing to be reviewed to rule out sinonasal orgin. Review of systemic diseases (ex. thyroid, granulomatous and autoimmune) as well. PE: visual acuity, visual fields, pupillary responses, ocular motility, globe surface, exophthalmos, and direction of displacement. Complete head and neck evaluation. Lab/Imaging.

Cavernous Hemangioma Hamartomas contained

within a fibrous capsule with large vascular channels, but no definite feeding vessels. Most common benign tumor. Peak between 20-40 years. Slow growing, but easily enlarge with stress proptosis. CT sharp, well circuscribed, dense mass. Intra and extraconal.

Schwannoma
Arising from any nerve braches within the orbit most common V1. Account for 1-6% of all orbital masses. Slow growing, well circumscribed, ovid and homogenous. Antoni A (spindle shaped cells), Antoni B (foamy cells).

Lymphoma

After inflammation and hemangioma this is the third most common cause of proptosis. Usually insidious onset, typically presents with proptosis, ptosis, diplopia, motility disorders. Of all orbital sites, the lacrimal gland is the most common site involved. Commonly will mold itself along the globe margin rather than invade. Bilateral occurrence is common.

Rhabdomyosarcoma
Most common orbital tumor in children. 90% of cases occur before age 16. Rapidly progressive but painless exophthalmos, proptosis, and ptosis. Arises from primative mesenchymal elements into 4 different types: embryonal >> pleomorphic, alveolar (worst prognosis) >> differentiated (best prognosis).

Dermoid Cyst
Represent the most common congenital lesion of the orbit (1/3 of all childhood orbital tumors). Arise as a sequestration of ectoderm within the suture lines of the orbital bones. Commonly observed as a painless mass in the superiotemporal area at the lateral portion of the eyebrow. Classified into juxtasutural, sutural and soft tissue types.

Carotid Cavernous Fistula

Acute or delayed onset of post-traumatic diplopia with proptosis and chemosis. Venous flow reversal. Orbital presentation is secondary to prominent anterior venous drainage.

Orbital Varix

May be either congenital or acquired (thrombosis is common). Not neoplastic, but simple focal dilation that may be enlarged with increased venous pressure. May be associated with intraorbital/ intracranial AVM or simply result from wall weakness.

Capillary Hemangioma
One third are diagnosed at birth and over 90% are visible by 6 months of age. Bony erosion is not observed, although expansion of the walls is possible. Telangiectatic vessels typical create strawberry appearance. Frequently produce globe displacement and enlarge with crying.

Metastasis to the Orbit

Accounts for approximately 10% of all orbital neoplasms. (5% hematogenous, 5% from adjacent structures). What is the most common tumor to spread to the orbit?
Breast Cancer (42%). Lung Carcinoma (11%). Unknown Primary Cancer (11%). Prostate (8%). Melanoma (5%). Average survival after dx is a dismal 9 months.

In the pediatric population metastatic disease is far less common.

Metastatic neuroblastoma is second only to primary retinoblastoma as the most frequent malignant tumor in childhood.

Diagnosis
FNA is a minimally invasive technique that may be used for diagnosing orbital lesions. Differentiation between benign and malignant lesions reaches an accuracy of 95%. Coupled with clinical and radiological finding, proper diagnosis is made in 80% of cases. Disadvantages include poor cellular yield, difficulty in interpreting the specimen, and the possible need for another biopsy procedure. Open biopsy of an orbital tumor is the common method of obtaining tissue from the orbital lesion. It also may be necessary if FNA is inadequate. With open biopsy a histological diagnosis is commonly made because enough of a specimen usually is obtained.

Surgical Considerations
Some conditions (ex. Pseudotumor, capillary hemangioma) do not mandate surgical intervention. Surgical approach is tailored to the location of the lesion with minimization of infraorbital venous pressure. Orbitotomy can be performed in anterior, lateral, medial, cornal and even intracranial dissections can be made to gain inferior, supertemporal, posterior, or central access. Close intraoperative monitoring of the pupil size and administered medications in addition to post-operative evaluation with regards to vision, bleeding, and pain is essential is the treatment of these lesions. Finally, a thorough explanation of the procedure and the risks, benefits, and alternatives should be clearly explained and documented. The patient should be cognizant of the exact procedure and it is imperative that the patient understands the possibility of orbital exenteration if indicated.