Orbit Part2

Restrictive myopathy • Occurs in about 40% • Due to fibrotic contracture

Elevation defect - most common

Depression defect -uncommon

Abduction defect - less common

Adduction defect - rare

Treatment:  Correction

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

radiotherapy?

Orbital Inflammatory Disease 

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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

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75% of lesions resolve during the next 4 years of life

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Treatment:  

Steroid radiotherapy

Cavernous Hemangioma 

Most common benign neoplasm of the orbit

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Female preponderance70%

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Can cause retinal striae, optic nerve compression, increased IOP, strabismus

Cavernous Hemangioma  CT

scan: homogenously enhanced, well encapsulated mass  Treatment: surgical excision

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Classification of lacrimal gland tumours

Pleomorphic Lacrimal Gland Adenoma Presents - 4th to 5th decade

• Painless and very slow-growing, smooth mass in lacrimal fossa • Inferonasal globe displacement

• Posterior extension may cause proptosis and ophthalmoplegia

• Smooth, encapsulated outline • Excavation of lacrimal gland fossa without destruction

Lacrimal gland carcinoma • Presents - 4th to 6th decades • Very poor prognosis

• •

Painful, fast-growing mass in lacrimal fossa Infero-nasal globe displacement

• •

Posterior extension may cause proptosis, ophthalmoplegia & episcleral congestion 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, progressive proptosis

Optic atrophy, (+) RAPD

Optic Nerve Glioma: Treatment  Observation-

no growth, good vision  Excision- rapid growth, poor vision, increased ICP  Radiotherapy- intracranial extension; nonresectable 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 Nerve compression

Optociliary shunts in 30%

Proptosis due to intraconal spread

Thickening and

• • •

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 bilateral rubbery on palpitation

May be confined to 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

Presents in early childhood • May be bilateral • Typically involves superior orbit •

Chloroma

Presents at about age 7yrs • Rapid onset proptosis -may be bilateral • 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 non-axial globe displacement

Similar to orbital pseudo-tumour

Enophthalmos with schirrous tumors

Cranial nerve involvement at orbital apex and mild proptosis

Orbital invasion by sinus tumors Maxillary carcinoma

Upward globe displacement and epiphora

Ethmoidal carcinoma

Lateral globe displacement

Orbital Fractures

Pathogenesis of orbital floor blow-out fracture

Signs of orbital floor blow-out fracture

• Periocular ecchymosis • Ophthalmoplegia and edema typically in up- and down-gaze • Infraorbital nerve (double diplopia) anaesthesia

• Enophthalmos & ptosisorbital soft tissues prolapse into the maxillary sinus

Investigations of orbital floor blow-out fracture Hess test Coronal CT scan

• Right blow-out fracture with ‘tear-drop’ sign

• Restriction of right upgaze and downgaze • Secondary overaction of left eye

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

Treatment • Release of entrapped tissue • Repair of bony defect