Restrictive myopathy

• Occurs in about 40%

• Due to fibrotic contracture

Elevation defect - most common Abduction defect - less common

Depression defect -uncommon Adduction defect - rare

Treatment:
 Correction of thyroid function abnormality
 Orbital decompression- to treat optic
neuropathy, orbital congestion, advanced
proptosis

 Orbital radiotherapy?
Orbital Inflammatory Disease
 Idiopathic tumor-like
inflammation made up
of pleomorphic
inflammatory cellular
response and
fibrovascular tissue
reaction
 Non-neoplastic and
non-infectious orbital
lesion
Idiopathic orbital inflammatory disease (IOID)
• Involves any or all soft-tissue components

• Abrupt, painful onset

• Usually unilateral
• Periorbital swelling and chemosis
• Proptosis
• Ophthalmoplegia
Clinical course and treatment of IOID
1. Early spontaneous remission without sequelae
Treatment - none
2. Prolonged intermittent activity with eventual remission
Treatment options - steroids, radiotherapy or cytotoxics

3. Severe prolonged activity causing a ‘frozen orbit’

Left involvement resulting in ophthalmoplegia and ptosis

ORBITAL TUMOURS
1. Vascular tumours
• Capillary hemangioma
• Cavernous hemangioma
2. Lacrimal gland tumours
• Pleomorphic adenoma
• Carcinoma
3. Neural tumours
• Optic nerve glioma
• Optic nerve sheath meningioma

4. Miscellaneous tumours
• Lymphoma
• Rhabdomyosarcoma
• Metastases
• Invasion from sinuses
 Capillary hemangioma
• Most common orbital tumour in children
• Presents - 30% at birth and 100% at 6 months

• Most commonly in superior anterior orbit

• May enlarge on coughing or straining
• Associated ‘strawberry’ nevus is common
Capillary Hemangioma
 Can cause
anisometropia,
strabismus, deprivation
amblyopia

 75% of lesions resolve

during the next 4 years of
life

 Treatment:
 Steroid
 radiotherapy
Cavernous Hemangioma
 Most common benign
neoplasm of the orbit

 Female preponderance-
70%

 Can cause retinal striae,

optic nerve compression,
increased IOP,
strabismus
Cavernous Hemangioma
 CT scan: homogenously enhanced, well
encapsulated mass
 Treatment: surgical excision
Classification of lacrimal gland tumours
()
 Pleomorphic Lacrimal Gland Adenoma
Presents - 4th to 5th decade

• Painless and very • Posterior extension • Smooth, encapsulated

slow-growing, smooth may cause proptosis outline
mass in lacrimal fossa and ophthalmoplegia
• Excavation of lacrimal
• Inferonasal globe gland fossa without
displacement destruction
 Lacrimal gland carcinoma
• Presents - 4th to 6th decades
• Very poor prognosis

• Painful, fast-growing mass in • Posterior extension may cause proptosis,

lacrimal fossa ophthalmoplegia & episcleral congestion
• Infero-nasal globe displacement • Trigeminal hypoaesthesia in 25%

Management
•Biopsy
• Radical surgery and radiotherapy
 Optic nerve glioma
• Typically affects young girls
• Associated neurofibromatosis -1 is common-25-50%
• Presents - end of first decade with gradual visual loss

Gradual painless, unilateral, axial, Optic atrophy, (+) RAPD

progressive proptosis
Optic Nerve Glioma: Treatment
 Observation- no growth, good vision
 Excision- rapid growth, poor vision,
increased ICP
 Radiotherapy- intracranial extension; non-
resectable tumors
 Chemotherapy-with actinomycin D &
vincristine; delays the need for
radiotherapy
Optic nerve sheath meningioma
Typically affects middle-aged women

Gradual visual loss due to optic Optociliary shunts in

Nerve compression 30%

Proptosis due to intraconal Thickening and

spread calcification on CT
Treatment
• Observation - slow-growing tumors
• Excision - aggressive tumors and poor vision
• Radiotherapy - slow-growing tumors and good vision
 Lymphoma
Presents - 6th to 8th decades

Affects any part of orbit & Anterior lesions are May be confined to
may be bilateral rubbery on palpitation lacrimal glands

Treatment
• Radiotherapy - localized lesions
• Chemotherapy - disseminated disease
 Rhabdomyosarcoma
• Most common primary childhood orbital malignancy
• Rapid onset in first decade ( average 7 yrs )

May involve any part of orbit Palpable mass and ptosis in about 30%

Treatment
• Radiotherapy and chemotherapy
• Exenteration for radio-resistant or recurrent tumours
 Childhood metastatic tumours
Neuroblastoma Chloroma

• Presents in early childhood • Presents at about age 7yrs

• May be bilateral • Rapid onset proptosis -may be bilateral

• Typically involves superior orbit • Subsequent systematic

dissemination
to full-blown leukaemia
 Adult metastatic tumours
Common primary sites - breast, bronchus, prostate, skin melanoma,
gastrointestinal tract and kidney
Presentations

Anterior orbital mass with Enophthalmos with

non-axial globe displacement schirrous tumors

Similar to orbital Cranial nerve involvement at orbital

pseudo-tumour apex and mild proptosis
 Orbital invasion by sinus tumors

Maxillary carcinoma Ethmoidal carcinoma

Upward globe displacement and Lateral globe displacement

epiphora
Orbital Fractures
Pathogenesis of orbital floor blow-out fracture
 Signs of orbital floor blow-out fracture

• Periocular ecchymosis • Ophthalmoplegia - • Enophthalmos & ptosis-

and edema typically in up- and orbital soft tissues
• Infraorbital nerve down-gaze prolapse into the
anaesthesia (double diplopia) maxillary sinus
Investigations of orbital floor
blow-out fracture Hess test
Coronal CT scan

• Restriction of right upgaze and downgaze

• Right blow-out fracture • Secondary overaction of left eye
with ‘tear-drop’ sign
Indications for surgery:
 Diplopia
with limitation of upgaze &/or
downgaze persistent even after 2 weeks

 Enophthalmos exceeding 2 mm that is

cosmetically unacceptable to the patient

 Large fractures involving at least half of

the orbital floor
 Medial wall blow-out fracture
Signs

Periorbital subcutaneous emphysema- Ophthalmoplegia - adduction and

if fracture extends into a sinus abduction
if medial rectus muscle is entrapped

Treatment
• Release of entrapped tissue
• Repair of bony defect