5.7  Oculoplastic Differential Diagnoses

Contents

Acute Orbital Inflammation

Vascular

  1. Lymphangioma
  2. CCF

Endocrine

Thyroid Eye Disease

Infective / Inflammatory

  1. Orbital cellulitis
    1. Bacterial infections cause profound acute inflammation
    2. Fungal infections can present more insidiously. Always exclude mucormycosis in diabetic patients with orbital cellulitis
    3. Masquerading infections (TB, syphilis)
  2. Orbital inflammatory diseases
    1. Sarcoid
    2. GPA / Wegeners or other vasculitis disorders
    3. IgG4-related disease

Neoplasia

  1. Aggressive neoplasms can cause or mimic acute inflammation
  2. Lymphoma often presents as subacute or chronic inflammation

Other

  1. Ruptured dermoid cyst
  2. Medication-related
  3. Bisphosphonates

Enophthalmos (Ipsilateral)

Increased Orbital Volume

  1. Orbital floor fracture
  2. Bony remodelling (silent sinus syndrome)
  3. Absence of greater wing of sphenoid (Neurofibromatosis type 1)

Orbital Tissue Atrophy

  1. Age related involutional changes
  2. Scirrhous metastasis (breast cancer)
  3. Post-Surgical (fracture repair, blepharoplasty, enucleation)
  4. Lid Scleroderma
  5. Hemifacial atrophy
  6. Prostaglandin Associated Periorbitopathy

Contralateral Exophthalmos

Globe Retraction

  1. Duane’s Syndrome
  2. Convergence-Retraction nystagmus (Parinaud’s syndrome)

Extraocular Muscle Swelling

Tendon Sparing

Thyroid Eye Disease

Tendon-Involving

  1. Idiopathic Orbital Inflammatory Disease
  2. Carotid-Cavernous Fistula
  3. Neoplasia (lymphoma, metastasis)

Eyelid Retraction

Neurogenic

  1. MGJW
  2. Aberrant CN III Palsy
  3. Duane’s Syndrome
  4. Parinaud’s Syndrome
  5. CN VII Palsy
  6. Phenylepherine

Myogenic

Thyroid Eye Disease

Mechanical

  1. Any causes of proptosis
  2. Buphthalmos
  3. Overcorrection following ptosis repair

Hertogh Sign (Lack of Outer 3rd of Eyebrows)

  1. Hypogonadism
  2. Hypothyroidism
  3. Scleroderma

Lacrimal Gland Mass and an S-Shaped Lid Deformity

Dacryoadenitis

  1. Infective
    1. Viral (mumps, EBV, influenza, VZV)
    2. Bacterial
  2. Inflammatory
    1. IOID
    2. Sarcoid (Mikulicz syndrome)
    3. Tuberculosis
    4. Granulomatous polyangiitis (Wegener’s)
    5. Polyarteritis nodosa

Neoplastic

  1. Benign
    1. Pleomorphic adenoma
    2. Plexiform Neurofibroma
  2. Malignant
    1. Pleomorphic adenocarcinoma
    2. Adenoid cystic carcinoma

Others

  1. Dermoid Cyst
  2. Lipodermoid

Madarosis

Local

  1. Blepharitis
  2. Tumour
  3. Radiation Therapy

Dermatological

  1. Alopecia
  2. Psoriasis
  3. Severe Rosacea

Systemic

  1. Hypothyroidism
  2. SLE
  3. Syphilis
  4. Leprosy

Others

  1. Iatrogenic
  2. Trichotilomania

Painful Ophthalmoplegia

“MINT”

  • M igraine
  • I nflammatory and I nfective
    • Inflammtory: Tolosa hunt, IOID, GCA
    • Infective: Orbital Abscess, mucormycosis, post-herpetic neuralgia
  • N eoplastic
  • T rauma (orbital fracture)

Proptosis

“VEIN-O”

  • M igraine
  • I nflammatory and I nfective
    • Inflammtory: Tolosa hunt, IOID, GCA
    • Infective: Orbital Abscess, mucormycosis, post-herpetic neuralgia
  • N eoplastic
  • T rauma (orbital fracture)

V ascular

  1. Orbital Varix
  2. Carotid Cavernous Fistula (CCF)

E ndocrine

Thyroid Ophthalmopathy

I nfective / Inflammatory

  1. Idiopathic Orbital Inflmmatory Disease (IOID)
  2. Orbital Cellulitis

N eoplastic

  1. Cavernous haemangioma
  2. Lacrimal gland (pleomorphic adenoma, Ca)
  3. Optic Nerve (glioma)
  4. Meningioma
  5. Neurofibroma
  6. Lymphoma
  7. Sphenoid wing meningioma
  8. Metastases
  9. Invasion by sinus tumours (nasopharyngeal and maxillary cancer)

O ther

  1. Dermoid Cyst
  2. Sinus mucocele

When approaching proptosis, these categories will help differentiate the various pathologies:

  1. Axial vs non-Axial proptosis
  2. Time course (acute vs subacute vs chronic vs intermittent)
  3. Adult or Childhood onset
Figure 5.7.1 Proptosis Differential Diagnosis

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Figure 5.7.1
Proptosis Differential Diagnosis

Figure 5.7.2 Thyroid Eye Disease

Figure 5.7.2
Thyroid Eye Disease

Figure 5.7.3 Left Orbital Cellulitis

Figure 5.7.3
Left Orbital Cellulitis

Pseudo-Proptosis

The appearance of proptosis (not true proptosis):

  1. Ipsilateral upper eyelid retraction or contralateral ptosis
  2. Contralateral enophthalmos
  3. Enlarged globe (e.g. high myopia, buphthalmos)
  4. Asymmetrical orbits (craniofacial anomalies)

Pulsatile Exophthalmos

  1. Carotid-cavernous fistula
  2. Sphenoid wing dysplasia (NF-1)
  3. AV malformation
  4. Trauma (Orbital Roof fracture)

Ptosis

Congenital

Acquired

Idiopathic

Congenital

Isolated congenital ptosis

Mechanical

Congenital

  1. BPES
  2. Eyelidmasses (Neuroblastoma; Plexiform neurofibroma; SWS; lymphoma; Rhabdo; Leukemia)

Acquired

  1. Aponeurotic (involutional, post-operative / trauma)
  2. Tumours (BCC, SCC, Melanoma) / Upper eyelid oedema / Anterior orbital lesions / Trauma / Giant Papillary conjunctivitis

Myogenic

Congenital

  1. CFEOM
  2. CPEO / KSS
  3. Congenital Myasthenic Syndromes
  4. Myotonic Dystrophy

Acquired

  1. Myasthenia Gravis
  2. Myotonic Dystrophy
  3. CPEO / KSS
  4. Oculo-pharyngeal dystrophy

Neurogenic

Congenital

  1. Marcus-Gunn Jaw winking
  2. Congenital CN III
  3. Congenital Horner’s

Acquired

  1. CN III palsy
  2. Aberrent Regeneration of CN III
  3. Horner’s

Pseudo-Ptosis

  1. Dermatochalasis
  2. Brow ptosis
  3. Globe:
    1. Small (microphthalmos, phthisis bulbi, implant)
    2. Hypotropia (e.g. Monocular elevation deficiency)
    3. Enophthalmos (e.g. Duane syndrome, PESS)
  4. Contralateral lid retraction (e.g. Thyroid Eye Disease)
  5. Apraxia of lid opening (Parkinson’s disease, Progressive supranuclear palsy)
  6. Blepharospasm, hemifacial spasm

Complete Ptosis

  1. CNIII palsy
  2. Aponeurotic trauma
  3. Myasthenia gravis
  4. Botulinum toxin

Bilateral Ptosis and Ocular Motility Disturbance

  1. Peripheral
    1. Myasthenia Gravis
    2. CPEO / Kearns-Sayre
    3. Miller-Fisher syndrome
    4. Myotonic Dystrophy
    5. CFEOM
  2. Central
    1. Bilateral CNIII palsies (midbrain lesion):
      1. Basilar artery CVA
      2. Pituitary apoplexy
      3. Cavernous sinus pathology


          

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