FASHION GENERAL Presented by:- Ranjita Amanatya A rare case of a 39years old male with Atypical Orbital Growth. Presented by:- Ranjita Amanatya PG 3 rd year VIMSAR, BURLA “OUT OF SIGHT,OUT OF MIND”
Orbital pseudotumour is a rare benign condition of unknown cause, characterized by an encapsulated nasi -like aggregates of myofibroblastic spindle cells and inflammatory cells, including lymphocytes. A 39 year old male, who presented with slowly growing abnormal pedunculated growth of size 6cm*4cm*2cm with area of patchy necrosis in Left Eye. The symptoms initially started as mild discomfort but gradually intensified over time.The patient was already diagnosed as (L.E.) Rhabdomyosarcoma & eviscerated elsewhere for same. HOPI:- Past history:- There was no history of trauma or foreign body exposure. Not a k/c/o HTN, DIABETES, Thyroid disease, Sickle cell disease. He denied experiencing fever,chills or weight loss. No a/w upper respiratory tract infection symptoms, joint pain, morning stiffness. No other focal neurological deficits.
V.A. 6/9 PL (absent) I.O.P. 14mmHg Couldn’t be assessed Lids & Adnexa Normal Socket eye with growth Conjunctiva Normal Cornea Clear Couldn’t A.C WNL be Iris Normal assessed Pupil Circular/LR(+) Lens Clear OD OS Ocular examination
B.P- 130/90 mmHg RBS- 91 mg/dl Serum- Urea, Creatinine, Na+, K+, Ca2+ - WNL General physical examinations:- Chest:- B/L symmetrical VBS heard CVS :-S1 S2 no murmur CNS :- NAD SYSTEMIC EXAMINATION s:- LAB INVESTIGATION s:- Showed CBC WNL. Thyroid profile was WNL Viral markers were non-reactive
heterogeneously enhancing soft tissue density mass of size 24 x 42.6 x 22.8 mm noted in the left orbit involving both and region and extending till apex of the orbit causing enlargement of the optic canal with internal calcification. The superior sagittal sinus shows loss of N flow void (?thrombosis) (suggested MR for further evaluation)
Ill defined areas in the bilateral lobes- likely cytotoxic CECT(Brain and Orbit):- Exophytic heterogeneously enhancing soft tissue density mass of size 24 x 42.6 x 22.8 mm noted in the left orbit involving both extracanal and intracanal region and extending till apex of the orbit causing enlargement of the optic canal with internal calcification. The superior sagittal sinus shows loss of N flow void (?thrombosis) (suggested MR venogram for further evaluation)
Ill defined hyperintense areas in the bilateral patietal lobes- likely cytotoxic edema
Well defined exophytic lobulated left orbital soft tissue lesion with left voids within, causing proptosis of the globe, with stretching of the I/L optic nerve – Neoplastic etiology (Rhabdomyosarcoma) Bulky left extra ocular muscles with hypotense signal intensity Left optic nerve atrophy. His MRI report suggested features of noeplasticetiology and superior sagittal sinus thrombosis. MRI (Brain and Orbit): -
As the patient was very sick, we didn’t get neurosurgical clearance/ anaesthetic clearance. They suggested, there was association of venous sinus thrombosis. In this context,we planned to put the ligature with 3-0vicry suture at the root, allowing to autonecrosis . Treatment plan: -
POD :-01 POD:-05 POD:-03 POD:-10
FASHION GENERAL Evisceration done after autonecrosis of the growth
After 1month follow -up
HISTOPATHOLOGICAL EXAMINATION Finrocollagenous stroma with areas of haemorrhage & edema . Many congested blood vessels Numerous plasma cells in the stroma with some having Russell bodies. Variable number of histiocytes & polymorphs. Suggesting features of Plasma cell pseudotumour
DISCUSSION Orbital pseudotumour is a rare benign condition of unknown cause, characterized by an encapsulated nasi -like aggregates of myofibroblastic spindle cells and inflammatory cells, including lymphocytes. It is also known as Idiopathic Orbital Inflammation, which describes a spectrum of Idiopathic,non - neoplastic,non -infectious, space-occupying Orbital lesions without identifiable local or systemic cause. It was first described in 1905 by Birch –Hirschfeld who termed the condition Orbital Pseudotumour . It is the 3 rd most common ophthalmologic-disease of the orbit.
CONCLUSION Pre-operatively suspected mass as Rhabdomyosarcoma was later diagnosed as Orbital Pseudotumour by histopathological examination of of the mass. They have given the diagnosis as Pseudotumour according to MRI reports.But in my opinion it could be Granulomatous growth of orbit too. Physical examination & imaging studies were helpful in narrowing the differential diagnosis & proper treatment.