NON-NEOPLASTIC ORBIT

CASE 1

CYST

KERATIN CONTENTS SKIN ADNEXA IN FIBROUS WALL

LINING JUST LIKE SKIN EPIDERMIS

DIAGNOSIS ?

DERMOID CYST Congenital Occur at point of bone sutures Epithelium-skin or conjunctival type Can rupture to give foreign body giant cell response to spilled keratin

CASE 2 Vertical diplopia

lymphocytes

Muscle fibres splayed apart by ACID MUCOPOLYSACCHARIDE matrix

DIAGNOSIS ?

Thyroid eye disease Non-granulomatous inflammation-lymphocytes Glycasaminoglycans-causes separation of skeletal muscle fibres. Eventually endomysial fibrosis. Muscle tendons and orbital fat spared by inflammation. Should be very familiar with this topic. Increased risk of orbital lymphoma.

CASE 3 Child-proptosis

Ectatic geographic shaped vascular elements

Attenuated endothelium lining Little smooth muscle in wall

Some channels contain Blood And possess thicker walls

Pink smooth muscle around these vessels

Complex vascular malformation Abnormal combination of lymphatic and conventional blood vessels (arteries and veins) elements. These are not strictly ‘omas’

CASE 4

History 45 y male Painful proptosis left eye, with limitation of ocular involvement for many months. Systemically well. Ophthalmologist-confirmed painful proptosis. Rest of eye examination-NAD. CT scan-orbital soft tissue mass. Orbital biopsy.

necrosis Giant cell

Diagnosis ?

Localised orbital Wegener’s granulomatosis.

Orbital Wegener’s Orbit affected by generalised and localised Wegener’s. In 7% of generalised and localised WG- proptosis is presenting feature.

Orbital Wegener’s Orbital localised WG-occurs from contiguous spread from localised sinus WG or primary WG in orbit. Common ocular features: proptosis, scleritis, episcleritis, cornea ulcers, optic nerve vasculitis, retinal artery occlusion, conjunctivitis and uveitis (inflammation of the iris, ciliary body and choroid).

Orbital Wegener’s ANCA negative in 37 % of localised and generalised WG. cANCA –ve cases may indicate early phase of disease. Usual histological triad-granulomatous inflammation, tissue necrosis and small vessel vasculitis. Triad only seen in 1/3 rd of orbital biopsies. Therefore, clinico-pathological approach essential.

Orbital Wegener’s biopsies Small vessel vasculitis Perivascular lymphocytes Necrosis (fat) Foamy macrophages Micro-abscesses Neutrophils, eosinophils, plasma cells FibroplasiaGranulomas Giant cells Often sclerotic areas with on-going active areas. Exclude infection. Affect any aspect of orbital soft tissue (inc lacrimal gland).

CASE 5

HISTORY 25 year-old Afro-Carribean woman Bilateral swelling of lacrimal glands Lacrimal gland biopsy

Non-caseating granuloma Naked-not surrounded by LYMPHOCYTES

Occasional giant cell

DIAGNOSIS ?

sarcoid Diagnosis of exclusion Exclude infective agent Afro-Carribean Female bias ACE, calcium levels, Chest X-ray, Gallium scan, biopsy Ocular features: Eyelids-lupus pernio or papular sarcoid Band keratopathy Corneal melt Dacryodenitis Conjunctival granulomas Episcleritis and scleritis Anterior uveitis Secondary cataracts and glaucoma Vitritis (classical sign) Choroiditis Retinal periphlebitis and candle wax exudates Retinal neovascularisation Pars planitis Optic nerve granulomas Disc oedema

CASE 6

TOUTON GIANT CELL-RING OF NUCLEI AROUND THE PERIPHERY OF THE CYTOPLASM, WITH A CLEAR CYTOPLASMIC RIM HISTIOCYTES IN BACKGROUND

EOSINOPHILS

DIAGNOSIS ?

JUVENILE XANTHOGRANULOMA Histiocytic disorder CD 68 positive cells (immunohistochemistry marker) Not S100 positive and lack Birbeck granules of Langerhan’s cell histiocytosis Sheets of histiocytes with bubbly cytoplasm, admixed with eosinophils and other inflammatory cells. Touton giant cells Can occur in iris, conjunctiva and eyelid.

Trendy diagnosis disease…

IgG4 sclerosing disease of the orbit