Chronic Granulomatous Inflammation Cpc2

8/6/2019 Chronic Granulomatous Inflammation Cpc2

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Chronic granulomatous

inflammation


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Presenting complaint:

Swelling of Right eye since 2000.


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Patient was alright prior to 2000 when he starteddeveloping proptosis of the eye. He underwent Rorbitotomy in 2001 and histopathology showed

granulomatous inflammation. The swelling increasedafter the operation. He then underwent repeatorbitotomy in 2002 and it was once again diagnosed asgranulomatous inflammation, most likely fungal.Patient was sent to radiology department who have

referred him back. There is no history of tuberculosis orthyroid eye disease.

Positive history of headaches.


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local Exam


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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


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Hb : 12.0 mg/dL

TLC :8600 cells/mm3

Lukocyte : 66%

Lymphos : 30%

Monocytes : 2%

Eosinophils 0.2% Blood Group : A +ve


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C-ANCA

ACE

Myco dot


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Histopathology Report 12-05-09


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Histopathology report 18-04-09

Sections reveal chronic inflammation with

granulmoma formation and giant cells, both

lLanghans 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.


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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 afibrocollagenous tissue with chronic inflammatory cellinfiltrate and numerous granulomata. No malignancy seen.

Opinion / Comment:

Granulomatous inflammation


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CT scan Brain and Orbit 15-04-05

Findings:

Soft tissue density mass is seen in the Left orbitretrobulbar 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


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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 noextension intracranial or into the para-nasal

sinuses.

I

mpression: Morphological features are suggestive of

inflammatory process.


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CT Scan orbit 03-07-08

Soft tissue density enhancing mass seen posterior to the right eyeglobe. The mass cannot be separated form the right optic nerve.

Inferior and anterior rectus muscles of right side are normal. Theright globe is normal however it is pushed anteriorly due to themass lesion.

There is deviation of the nasal septum with convexity towards theleft 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 lesionhas reduced in size.


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CT scan orbit 16-06-07


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CT Scan Obit 10-10-07

Ill defined soft tissue density msass lesion

with proptosis.

Shadowing with mucosal thickening ofipsilateral sinus.

Impression:

The appearance suggestive of pseudotumor rather

than metastatic lesion.


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CT scan Brain and orbit 02-06-03

Post operative follow-up scan.

Large expanded soft tissue density lesion

involving pper quadrant Right orbit withsignificant proptosis.

No evidence ofo intracranial rxtension.

Differential: Pseudotumor.


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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-collanegenoustissue with moderate inflammatory cell infiltrates comprising oflymphocytes, plasma cells, few eosinophils and macrophag3e. In

addition there are many granulomatous collectrions composed ofmultiple multinucleated Langhans and foreign body type of giantcells and collections of lymphocytes and macrophages. At theperiphery fibro-fatty tissue, skeletal muscle fiber and multiple nervebundles are seen. No epitheloid cells are seen.

There is no evidence of malignancy. These appearances are of

chronic granulomatous inflammation. Special stains for fungusreveal fungal hyphae.


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Histopathology report 05-05-03

With large comments


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CT scan 14-02


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Bone Scan

No scintigraphic evidence of any bone

pathology.


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CT Scan Brain and Oribt 14-02-09

Soft tissue density mass lesion of right eyeball,retrobulbar area, is noted clearly in separate fromposterior aspect of sclera, optic nerve and extraocularmuscles, optic canal on ipsilateral side is normal.

Significant proptosis of right eyeball is noted.Contralateral eyeball, both nasal cavities mastoid aircells, are appreciated to be normal, visualized brainparenchyma 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 abovesaid extension)


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Histological sections of the sections reveals ocular tissue comprisingof fibrocollagenous and fibrofatty tissue with dense infmlammatorycell infilt5rate comprising of lymphocytes.

In addition there are granumuloms composed of multinucleatedLanghans and foreign body type o fgiant cells

Granulomas are also seen in the peripheral adipose tissue andextraocular muscle.

No caseation seen

No vasculitis seen

The features are strongly suggestive of chronic ranulomatous

inflammation. Comments:

Most likely fungal granulomas.


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Management


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Plan

Enucleation

radiotherapy


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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.


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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).


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Bony Orbit F: Frontal bone with

associated suture andnotch.


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Separation of Lesions by Anatomic

Subsite


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Evaluation - Review

Detailed recording of onset, duration and progression ofthe orbital disease.

History of allergies, sinus infections, epistaxis, nasaldischarge, 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, ocularmotility, globe surface, exophthalmos, and direction of

displacement. Complete head and neck evaluation.

Lab/Imaging.


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Cavernous Hemangioma Hamartomas contained

within a fibrous capsule withlarge 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.


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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).


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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.


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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).


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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.


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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.


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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.


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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.


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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.

Metastatic neuroblastoma is second only to primary retinoblastoma as the

most frequent malignant tumor in childhood.

In the pediatric population metastatic disease is far less common.


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Diagnosis

FNA is a minimally invasive technique that may be used fordiagnosing orbital lesions.

Differentiation between benign and malignant lesions reaches anaccuracy of 95%.

Coupled with clinical and radiological finding, proper diagnosis ismade in 80% of cases.

Disadvantages include poor cellular yield, difficulty in interpretingthe specimen, and the possible need for another biopsy procedure.

Open biopsyof an orbital tumor is the common method ofobtaining tissue from the orbital lesion. It also may be necessary ifFNA is inadequate.

With open biopsy a histological diagnosis is commonly madebecause enough of a specimen usually is obtained.


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Surgical Considerations

Some conditions (ex. Pseudotumor, capillary hemangioma) do notmandate surgical intervention.

Surgical approach is tailored to the location of the lesion withminimization of infraorbital venous pressure.

Orbitotomy can be performed in anterior, lateral, medial, cornaland even intracranial dissections can be made to gain inferior,supertemporal, posterior, or central access.

Close intraoperative monitoring of the pupil size and administeredmedications in addition to post-operative evaluation with regards tovision, bleeding, and pain is essential is the treatment of theselesions.

Finally, a thorough explanation of the procedure and the risks,benefits, and alternatives should be clearly explained anddocumented. The patient should be cognizant of the exactprocedure and it is imperative that the patient understands the

possibility of orbital exenteration if indicated.


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Chronic Granulomatous Inflammation Cpc2