Lecture 3 orbit 1 evaluation of orbital disease

Bệnh nhãn giáp là biểu hiện ở mắt của các bệnh liên quan tuyến giáp. Bệnh lý này có nhiều tên gọi khác nhau như bệnh Graves, bệnh nhãn giáp, bệnh lý hốc mắt của Graves hay bệnh mắt liên quan tuyến giáp.

Evaluation of Orbital Disease

Symptoms, Signs and Imaging

Topics in Orbital Disease

1 Evaluation of Patients with Proptosis and other

signs or symptoms of Orbital Disease

2 Orbital Inflammatory Disorders

3 Orbital Surgery Orbital Tumors

Soft Tissues

1 Extra Ocular Muscles

2 Globe and Optic Nerve

3 Superior Ophthalmic Vein

4 Orbital Fat

5 Lacrimal Gland

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Pre-septal Cellulitis

Red Swollen Eyelids

Signs Suggesting Orbital Disease

• Proptosis, and other

Lid Mass or Fullness, UL sulcus defect (asymmetry)

Sometimes: Ptosis, Lid Retraction, Ectropion and Entropion

• Loss of Vision

• Fundus Findings: Disc edema, Vessel Congestion

Left hypoglobus

Lid deformity in orbital NF

Example: Orbital Cellulitis

Eyelid Edema- Erythema, but with;

Proptosis, Chemosis, Limitations in

motility as well

The Six “P’s” of Orbital Disease

PAINFUL CONDITIONS

• Infectious or other inflammatory

Orbital Cellulitis, Abscess

malignant lacrimal gland tumors

neural tumors at times – neurofibroma, neurilemmoma

SuB-periosteal Abscess

http://www.nature.com/eye/journal/v20/n10/fig

Retrobulbar hemorrhage

Not usually Painful

Forward displacement of the eye due to increased orbital contents

Pseudo-proptosis – simulation of

abnormal prominence of the eye

Enlarged Globe - Glaucoma, Myopia

Contralateral Enophthalmos Asymmetry of Bony Orbits Lid Retraction

18.5 mm 24.7 mm

African American Women

Forward Displacement of the Eye

1 Graves Orbitopathy (#1 Cause)

2 Orbital Cellulitis

3 Orbital Pseudotumor

4 Other Inflammatory - Wegener’s, Sarcoidosis, see also Orbital Inflammation

5 Orbital Tumors, Sinus Tumors

6 Orbital Hemorrhage- Trauma, Lymphangioma

Subperiosteal Hemorrhage

7 Orbital Trauma and Compartment Syndrome

8 Vascular Abnormality – e.g Varix, Carotid Cavernous Fistula

9 Bony Orbital Malformation – e.g congenital

• Superior Displacement

Maxillary Sinus Tumors

Infiltrating Skin Tumors

Large Mucocele

• Inferior Displacement

Down and Medial:

Dermoid Cysts

Lacrimal Gland Tumors

Down and Lateral

Sinus Carcinoma

Frontal and Ethmoidal Mucoceles

Orbital Dermoid

Orbital Mucocele

Valsalva Maneuver

Orbital Varices

Orbital Varix Causing Enophthalmos

Hong Kong Med J 2009;15:299-300

• Orbital Blow-out Fracture

• Congenital – asymmetry in bony orbits

• Bony defects in orbit due to tumor, chronic infection, vascular malformations (e.g

varix), radiation, Sinus Infection, Gorham’s Disease, NF

• Neoplastic –e.g Metastatic Scirrhous CA (e.g Breast)

• Orbital Fat Atrophy

- Parry- Romberg Syndrome (Idiopathic Hemifacial Atrophy, Sympathetic Input?)

- Past inflammation, Past Trauma, Linear Scleroderma

- Orbital tumor removal – tumor caused pressure atrophy of the fat

• Pseudo-Enophthalmos

Microophthalmos, Phthisis, Contralateral Exophthalmos,

Ptosis, Isolated Superior Sulcus Deformity

Posteriorly Displaced Globe often with superior sulcus deformity

Enophthalmos OD

Note Pseudo-Ptosis

As wellEnophthalmos

Silent Sinus Syndrome

“implosion of maxillary antrum”

Lacrimal Gland Prolapse, Tumor or

Lymphoma, Pseudotumor (Dacryoadenitis)

Masses in Nasal and Superior Nasal Quadrant

What is the mass in superior nasal quadrant

Bulging nasal fat pad

Swelling in the Superior Temporal

Quadrant

Mass in Superior Temporal Orbit

• Dermoid Cyst

• Neurofibromas

• Mucocele

• Lacrimal Gland Problem

Benign and Malignant Tumors

Mass in Superior Temporal Orbit

Dermoid Cyst

Neurofibromas

Prolapsed Orbital Fat

Dermatolipoma

Lacrimal Gland Problem

Benign and Malignant Tumors

Benign Mixed Tumor, Lacrimal Gland CA

Prolapse of Lacrimal Gland

Dacryops – Lacrimal duct cyst

Masses in Nasal and Superior Nasal Quadrant

Dermoid Cysts Meningocele and Encephalocele Sinus Mucocele

Lacrimal Sac: Tumor, Dacryocystocele, Dacryocystitis Neurofibromas

Bulging Nasal Fat Pad

Visible and Palpable Masses

in and around the Orbit

Apparent Mass under Lower Eyelid

Orbital Tumors yes, but also need to consider:

Festoons - Prolapsed Orbital Fat and and/or Redundant folds of skin Inflammatory Disease – Orbital Cellulitis, Graves Disease

Lymphedema (See list #37), Allergic Reactions Lymphoproliferative Disorders - Lymphoma, Orbital Pseudotumor, etc.

• Pulsation without bruits

Orbital bone wall/roof defects Neurofibromatoisis

Meningoencephalocelees Trauma

• Pulsation with bruits

Cavernous Carotid Fistulas

Dural AV Fistulas

Orbital AV Fistulas

Carotid Cavernous Fistula

Periorbital Signs

Lid retraction and Lid Lag on downgaze

What is one of the first conditions you think of?

Ptosis Can be a presenting sign of orbital disease

Periorbital Signs

Injected Conjunctival or Episcleral Vessels

- Carotid Cavernous Fistula

- Obstruction of Venous Outflow

Orbital Tumor or Inflammation

Black Necrotic Areas

• Suggest Mucormycosis

in immunocompromised patient

Chemosis

• Orbital Disease

Inflammatory – Orbital Cellulitis

Orbital Pseudotumor Graves Ophthalmopathy Orbital Trauma, Surgery

Vascular Congestion – Apex process, CC fistula

Tumor

• Conjunctivitis – local irritant – chemical, insect sting,

allergic, infectious, HSV, nearby corneal inflamm.

• Blocked Orbital Lymphatics – surgery, radiation, lymphedema

• Systemic – R Heart Failure, Nephrotic syndrome ( Plasma protein)

Prolapsing Orbital Fat

or

Pleomorphic Lipoma of the Orbit

OPH 2003;110:101

Signs of Orbital InflammationDifferential Diagnosis

Vasculitis (e.g GCA), CC Fistula, Cavernous Sinus or Orbital Vein Thrombosis

9 Inflammatory -Mimicking Orbital Tumors

Rhabdomyosarcoma, Lacrimal Gland Carcinoma, Lymphangioma

10 Orbital Hemorrhage

Need to R/O Orbital Cellulitis / Malignancy

CT Imaging of the Orbits

Indications for CT of the Orbits

1 Orbital and Ocular Trauma

2 Proptosis

3 Masses in Anterior Orbit and Eyelids that are not clinically obvious

4 Signs of Orbital Inflammation

Suspect Cellulitis or Oorbital Pseudotumor

(Chemosis, Loss of Motility, Pupil defect, and/or Eyelid Edema)

5 Suspected Orbital and Ocular Tumors

6 When MRI is contraindicated: Ferromagnetic FB, Pacemaker, recent

cardiac stent, metallic implants, incompatible aneurysm clips, cochlear implants, Claustrophobia

Orbital Soft Tissue Structures

SR /Levator and SOV

Superior Oblique

Medial Rectus

Soft Tissues

• Optic Nerve and Lacrimal Gland

Four Rectus Muscles

Note how LR can be rather small

and hard to see

Medial and Lateral Rectus Muscles

Superior Rectus and Levator Muscles

Inferior Rectus Muscles

Superior Ophthalmic Vein

(SOV)

Lacrimal Gland

Lacrimal Gland Anatomy

Orbital and Palpebral Lobes

Anteriorly, it is divided into an upper (orbital) and lower

(palpebral) lobe by the lateral horn of the levator aponeurosis

Palpebral Lobe

Orbital Pseudotumor (Idiopathic)

Acute- post viral, mononucleosis, bacterial

Chronic – TB, Sarcoidosis, Syphilis

Autoimmune Disease: e.g Sjogren's

Graves Disease

Other Things to Consider that give clinical appearance of

enlarged LG:

Prolapse of Lacrimal Gland

Dacryops – Cyst of Lacrimal gland ducts

Adjacent Lipoma or Dermatolipoma in region of LG

JAMA Ophthalmol 2013;131(7):929-932

doi:10.1001/jamaophthalmol.2013.1885.

Dacryops

Graves Ophthalmopathy

Orbital Pseudotumor

CT Orbits

What view?, What Window? What’s Wrong???

OH Sheath meningioma

Calcifications

Left orbital phlebolithes from a varix

Sarcoma of the bone

Metastatic Disease to the Bone 

Paget’s Disease

Calcifications

Orbital Varix (Phleboliths)

Optic Nerve Sheath Meningioma Ocular Tumor – e.g RB Optic Nerve Head Drusen

Enlarged Extra Ocular Muscle(s)

Graves Ophthalmopathy (IR ,MR >SR most common)

Orbital Pseudotumor (Myositis) Post- Viral Myositis (Children)

Lymphoma Metastatic Disease Primary Tumor

Orbital Tumors to Consider

Age Category

1 Children

2 Adults

RhabdomyosarcomaNeuroblastoma, LymphangiomaCapillary Hemangioma

Glioma of Optic NerveDermoid Cyst

Neurofibromas (NF1 and NF2)

Cavernous HemangiomaLymphoma or other WBC tumor Optic Nerve Sheath MeningiomaLacrimal Gland CA and other tumorsNeural and Fibrous Tumors

Adjacent Invasive Sinus TumorMetastasis

Granulomatous Inflammation

Wegener’s Granulomatosis, Sjogrens , TB, Sarcoidosis, Syphilis, IgG4-ROD

8 Medications: e.g Biphosphonates

9 Vascular etiologies

Vasculitis (e.g GCA), CC Fistula, Cavernous Sinus

or Superior Orbital Vein Thrombosis (Sepsis, Coagulopathies, etc)

10 Inflammatory Mimicking Orbital Tumors

Rhabdomyosarcoma, Lacrimal Gland Carcinoma, Lymphangioma

11 Orbital Hemorrhage

Need to R/O Orbital Cellulitis / Malignancy Imaging of the Orbits

Signs of Orbital Inflammation

Proptosis, Chemosis, Eyelid

Edema, etc.

Work-up could include - CT orbits +

TED: TFTs, TSI IOIS: CBC (Eosinophilia), ANA, IgG4, Also: ANCA, ESR, Glucose, UA, CXR, RF Testing for TB / Sarcoid + possible Orbital Biopsy

Orbital Cellulitis

infection - exceptions – e.g MRSA

• Serial CT orbits to look for abscess development and possible need for surgical intervention

Orbital Pseudotumor

(IOIS = Idiopathic Orbital Inflammatory Syndrome)

• Non- Infectious –

part of the spectrum of lymphoproliferative disorders

• Frequently involves Lacrimal Gland or EOM(s)

• Frequently responds to corticosteroids

(sometimes after treatment with antibiotics)

Graves Ophthalmopathy

(TED= Thyroid Eye Disease)

• In addition to typical orbital

inflammatory signs – look

for :

• Lid Retraction, Lid Lag on

downgaze, Specific motility

problems such as

infraduction or abduction

deficits and ET or HoT

Carotid Cavernous Fistula

(CCF)

• In addition to typical orbital

inflammatory signs – look

and listen for :

• Pulsations, Bruits, Venous

Congestion

I tell you the truth, unless a man is born again, he

cannot see the kingdom of God

John 3:3