ophthalmological subject

1. Prepared By:
Dr. Niwar Ameen Othman
Resident Ophthalmologist
Duhok Eye Hospital
2018
PROPTOSIS
CLINICAL APPROACH

2. CONTENTS
• Introduction
• Causes
• Clinical approach
• Investigations
• Causes review
• References

3. INTRODUCTION
• Orbital anatomy
- Seven bones form the boundaries of the orbit, which are frontal,
zygomatic, palatine, lacrimal, sphenoid, ethmoid and maxillary
bones.
- Orbital cavity is pear shaped and has a volume of 30 cc.
- Structures and tissues occupying the cavity are the globes,
muscles, tendons, fat, fascia, vessels, nerves, sympathetic
ganglia, and cartilaginous trochlea.

4. • Terminology
- Proptosis denotes a forward displacement or bulging of a body
part and is commonly used to refer to protrusion of the eye.
- Exophthalmos specifically means proptosis of the eye and is
sometimes used to describe the bulging of the eye associated
with TED.
- Exorbitism refers to an angle between the lateral orbital walls
that is greater than 90º
- Hyperglobus or hypoglobus are used to describe the eye
when is displaced vertically or horizontally.
- Enophthalmos refers to retrodisplacement of the eye into the
orbit.

5. EXORBITISM

6. CAUSES
• Pseudoproptosis
• 1- enlarged globe
• 2- contralateral enophthalmos
• 3- asymmetric orbital size
• 4- asymmetric palpebral fissure

7. The causes of the proptosis can be classified according to the
underlying pathology as follow:
1- Inflammatory
2- Infectious
3- Trauma
4- Vascular
5- Cyst
6- Tumor

8. Inflammatory
1. Thyroid-related ophthalmopathy
2. Idiopathic orbital inflammatory disease (pseudotumor)
3. Sarcoidosis
4. Wegener’s granulomatosis
5. Systemic amyloidosis
6. Histiocytosis

9. Infectious
1. Orbital cellulitis
2. Sub periosteal abscess
3. Gumma
4. Mucormycosis
Trauma
1. Orbital blow-out fracture
2. Retrobulbar hemorrhage
3. Carotid-cavernous fistula
4. Retained orbital FB

10. Vascular
1. Orbital varices
2. Aneurysms
Cysts
1. Dermoid cyst
2. Mucocele
3. Hydatid cyst
4. Cysticercus

11.  Tumors
1. Capillary hemangioma
2. Cavernous hemangioma
3. Rhabdomyosarcoma
4. Pleomorphic lacrimal gland adenoma
5. Lacrimal gland carcinoma
6. Lymphangioma
7. Optic nerve glioma
8. Optic nerve sheath meningioma
9. Retinoblastoma
10. Osteoma
11. Neurofibroma
12. Leukemia
13. Lymphoma
14. Metastatic tumors

12. CLINICAL APPROACH
History
Examination
Investigation

13. • Age of onset
• Nature of onset
- acute (hrs to wks)
- subacute (1-4 wks)
- chronic
• Progression or intermittent
• Associated symptoms
- vision loss
- pain
- diplopia
Past ophthalmic Hx (e.g. trauma,
surgery)
• Past medical Hx
- Thyroid disease
- Hx of Malignancy ( breast, lung,
bowel, nasopharyngeal ca. )
- smoking Hx ( exacerbate TED )
• Drug Hx
- long-term IS with steroids or other
agents may mask inflam. disease.
- anticoagulants may cause bleeding
from vascular lesions
• Family Hx of ( familial idiopathic
proptosis, telecanthus)
HISTORY
Oxford Specialty Training, 2nd, 2016

14. EXAMINATION
 Local evaluation
• Inspection
- Uni or bilateral
- Direction of displacement
- periorbital changes for example salmon patch in lymphoma
- Eyelid abnormalities such as lid lag, lid retraction … etc.
- Ocular movements limitations
• Palpation (orbital rims and regional L.N)
• Auscultation (over globe or mastoid bone to check for any bruits as in cases of
carotid-cavernous fistula)
• Measurement of proptosis

15. • Measurement of proptosis is very important and it should be done
in all 3 direction, anteroposterior (axial), horizontal and vertical.
 Naphzeiger’s test

16.  Another simple bedside test to detect mild proptosis is with the help of a scale. Ask
the patient to gently close the eyes, and keep a scale across the eye in contact with
his forehead and cheek. Normally there will be space between the scale and the
closed lid. In the presence of proptosis this space is obliterated (Figures 2.33A and
B).

17.  Exophthalmometer
Commonly, a binocular exophthalmometer (e.g. Hertel) is
employed, using visualization of the corneal apices to determine
the degree of ocular protrusion from a scale (Fig. 3.4C).
Measurements can be taken both relaxed and with the Valsalva
maneuver. Readings greater than 20 mm are indicative of proptosis
and a difference of 2–3 mm or more between the two eyes is
suspicious regardless of the absolute values. The dimensions of
the palpebral apertures and any lagophthalmos should also be
noted.

19.  Ophthalmic examination
• Visual acuity
• IOP measurement in different gaze, to differentiate paralytic from restrictive
by change of IOP by more than 5-6 mmHg
• RAPD
• Color vision
• Ocular motility if limited FDT
• Fundus examination (optic disc swelling --- compressive ON, optic atrophy)

20. General examination
• All the systems including vital signs
• PR in TED
• Temp. in orbital cellulitis
• Otolaryngological examination

21. INVESTIGATIONS
• Primary studies:
1. CT-scan
• Is the most valuable technique for delineating the shape, location, extent and
character of the lesion in orbit.
• Usually obtained in 3-mm sections as opposed to the thicker 5-mm, and
sometimes 1.5-mm section is required
2. MRI
3. Ultrasonography
• Two-dimensional B-scan US
• One dimensional standardized A-scan US

23. • Secondary studies are performed for specific rare cases and include
1. Venography in diagnosis and management of orbital varices and study of
cavernous sinus
2. Arteriography for diagnosis of arterial lesions such as aneurysm or AV
malformations
3. CT and MR angiography for aneurysms, AV malformations and fistulas

24. • Laboratory studies
1. CBC, CRP, ESR
2. TFT includes T3, T4 and TSH, also TSH receptor Abs
3. ANCA --- Wegener's granulomatosis
4. ACE --- sarcoidosis

25. CAUSES REVIEW

26. THYROID EYE DISEASES
• It is the most common cause of both bi and unilateral proptosis in adult.
• Thyrotoxicosis is an excess of thyroid hormone secretion
• Graves disease is most common of hyperthyroidism, it is autoimmune.
• More common in female in 4th to 5th decade present.
• Features; weight loss, ↑ appetite, ↑ bowel frequency, irritability, nervousness,
sweating, heat intolerance, palpitation, weakness and fatigue.
• Risk factors are smoking, women and radioactive iodine used to Rx
hyperthyroidism ↑ TED.

27. Pathogenesis of Ophthalmopathy
• Organ specific autoimmune reaction AB reacts against TG cells and
ocular fibroblasts inflammation of EOMs, interstitial tissues, orbital
fat and lacrimal glands pleomorphic cellular infiltration, associated
with secretion of glycosaminoglycans and osmotic imbibition of water.
• Subsequent in the volume of orbital contents.
• Particularly EOMs, which can swell up to 8 times of their normal size.
• Secondary elevation of intraorbital pressure and optic nerve compression.
• Eventually degeneration of muscle fibers lead to fibrosis and resulting
restrictive myopathy and diplopia.

28. Clinical features
1. Soft tissue involvement
- Grittiness, red eyes, lacrimation, photophobia, puffy lids and retrobulbar discomfort.
- Epibulbar hyperaemia, Periorbital swelling, Tear insufficiency and instability is common.
2. Lid retraction
- Retraction of upper and lower lid in 50%, due to over stimulation of Muller muscle and
fibrotic changes.
- Signs; - Scleral show upper eyelid above superior limbus
- The Dalrymple sign is lid retraction in primary gaze
- The Kocher sign describes a staring and frightened appearance
- The von Graefe sign signifies retarded descent of the upper lid on downgaze

29. 3. Proptosis
- It is axial, unilateral or bilateral, symmetrical or asymmetrical, and frequently
permanent.
- Severe proptosis may compromise lid closure and along with lid retraction
and tear dysfunction can lead to exposure keratopathy, corneal ulceration
and infection.
4. Restrictive myopathy
- Between 30% and 50% of patients with TED develop ophthalmoplegia and
this may be permanent.
- Ocular motility is restricted initially by inflammatory oedema, and later by
fibrosis.
- Diplopia and discomfort in some position of gaze.
- EOMs involvement IMSL
5. Optic neuropathy
• Optic neuropathy is a fairly common (up to 6%) serious complication caused
by compression of the optic nerve or its blood supply at the orbital apex by
the congested and enlarged recti and swollen orbital tissue.

34. Management
• History
• Examination (VA, VF, RAPD and Hess chart)
• Investigations (TFT, CT-orbit)
• Treatment:
A. General measurements
- 1- lubricants
- 2- taping the eyelids
- 3- cold compresses
B. Systemic steroids
- 1- oral prednisolone 60-80 mg/day tapering over 2-8 wks
- 2- IV methyleprednisolone 0.5g/200 ml saline over 30 min
C. Radiotherapy (alone or with CS)
D. Combined (radiation + IS + CS)
E. Surgical decompression (OSE)

36. ORBITAL CELLULITIS
• It is a serious infection of soft tissue behind the orbital septum
• Can be sight and life threatening infection
• More common in children
• Causative M.O Strept. pneumoniae, Staph. aureus, Strept. pyogenes and
Haemophilus influenzae are common
• Origin of infection from typical from paranasal sinus (ethmoid) and also
preseptal cellulitis, dacryocystitis, midfacial skin or dental infection and follow
trauma, including any form of ocular surgery. Blood-borne spread from
infection elsewhere in the body may occur.

37.  Clinical features
Symptoms
• Unilateral most of time
• Pain of rapid onset, exacerbated by eye movements
• Swelling of the eye
• Malaise
• Frequently visual impairment and Diplopia
Signs
• Pyrexia is marked
• Tender firm erythematous eye
• Periocular and conj edema with injection and subconj hemorrhage
• Proptosis is common may be cover by swollen eyelids
• Fundus --- choroidal fold and optic disc swelling may be present

38.  Management
• History; recent history of nasal, sinus or respiratory symptoms.
• Examination VA, RAPD, Temp
• Investigations CBC, culture of nasal discharge, blood culture and CT-orbit,
sinuses and brain.
• Treatment
- Hospital admission
- IV antibiotics according to sensitivity, till patient is fever free
- Oral continued for 1-3 wks
- Optic nerve function monitoring
- Surgical drainage of orbital abscess

40. IDIOPATHIC ORBITAL INFLAMMATORY
DISEASE
• Idiopathic orbital inflammatory disease, also non-specific orbital inflammation
or orbital pseudotumor)
• It is an uncommon disorder characterized by non-neoplastic, non-infective,
space occupying orbital infiltration with inflammatory features.
• The process may preferentially involve any or all of the orbital soft tissues.
• Histopathological analysis reveals pleomorphic inflammatory cellular
infiltration followed by reactive fibrosis.
• Unilateral disease is typical in adults, although in children bilateral
involvement may occur.
• Intracranial extension is rare; simultaneous orbital and sinus involvement is
also rare, and may be a distinct entity.

41.  Presentation
Symptoms
o typically consist of unilateral acute or subacute ocular and periocular
redness, swelling and pain, 3rd - 6th decade. Systemic symptoms are
common in children.
Signs
o Pyrexia is present in up to 50% of children, but is rare in adults.
o Congestive proptosis.
o Mild to severe ophthalmoplegia may occur.
o Fundus may shows optic disc swelling and Choroidal folds.
Course
The natural history of the inflammatory process is very variable.
o Spontaneous remission
o Intermittent episodes
o Severe prolonged inflammation

42.  Management
• History
• Examination
• Investigations CT-orbit and biopsy to rule out neoplasia and other systemic
inflammatory diseases.
• Treatment
• - observation
• - oral NSAIDs
• - systemic steroids (oral prednisolone 1-1.5 mg/kg/day)
• - radiotherapy
• - other options such as cytotoxic agents (methotrexate, azathioptine)
• - surgical resection of inflammatory focus

44. CAROTID–CAVERNOUS FISTULA
• Arteriovenous fistula between carotid artery and cavernous
sinus.
• Raise in venous pressure in sinus and structure draining to it.
• Blood stasis in venous and arterial supply around the eye and
orbit
• Increase in episecleral venous pressure and decrease of blood
flow to the CN within the cavernous sinus.
• CCF can direct (high flow shunt) or indirect (dural shunt)

45.  Direct CCF
• 70-90% of CCF
• The communication is between intracavernous ICA and cavernous sinus
• Causes
1. Spontaneous
- Middle aged hypertensive woman aggravated by pregnancy
- atherosclerosis
- aneurysm
2. Head trauma
3. Iatrogenic
 Indirect CCF
• The communication is between cavernous sinus and meningeal branches of ICA
and/or ECA
• Causes
- Spontaneous
- Congenital AV malformation

46.  Clinical features
• Days to wks after head trauma
• History of systemic or surgical factors
• Presentation
- Pulsatile proptosis and bruit
- Chemosis and epibulbar injection
- Decreased vision
- Diplopia
- Ptosis
- Elevated IOP
- Anterior segment ischemia

47. • Fundus examination may show optic disc swelling, venous dilatation and
intraretinal haemorrhages from venous stasis and impaired retinal blood flow.
• Investigation: CT, MRI angiography, Doppler US.
• Complications
- Exposure keratopathy
- Secondary glaucoma
- Ischemic ON
- CRVO
• Treatment
- Medical toward the complications
- Surgical if spontaneous closure not occur

49. CAVERNOUS HAEMANGIOMA
• Cavernous haemangioma occurs in middle-aged adults
• Female preponderance of 70%; growth may be accelerated by pregnancy.
• It is the most common orbital tumour in adults
• It is vascular malformation rather than a neoplastic lesion.
• Although it may develop anywhere in the orbit, it most frequently occurs
within the lateral part of the muscle cone just behind the globe, and behaves
like a low-flow arteriovenous malformation.
Features
• Slowly progressive unilateral proptosis; bilateral cases are very rare.
• Decreased VA, Diplopia and gaze evoked transient blurring vision
• Choroidal fold and optic disc edema
Investigation. CT and MRI show a well circumscribed oval lesion, usually within
the muscle cone. Ultrasound is also useful.
Treatment is surgical excision for symptomatic lesions

51. PLEOMORPHIC LACRIMAL GLAND ADENOMA
• Pleomorphic adenoma (benign mixed-cell tumour) is the most common
epithelial tumour of the lacrimal gland and is derived from the ducts and
secretory elements including myoepithelial cells.
• Painless slowly progressive proptosis or swelling in the superolateral eyelid
• Old photograph may reveal abnormality
• Orbital lobe tumor can cause inferonasal dystopia, or proptosis,
ophthalmoplegia choroidal fold may present in case of posterior extension.
• Palpebral lobe tumour is less common and tends to grow anteriorly causing
upper lid swelling without dystopia, it may be visible to inspection.
• Investigation ---- CT-orbit and biopsy is wise to be avoided not to seed tumor
cells.
• Treatment ---- surgical excision.

53. OPTIC NERVE GLIOMA
• Optic nerve glioma is a slowly growing, pilocytic astrocytoma
• It typically affects children (median age 6.5 years)
• The prognosis is variable; some have an indolent course with little growth, while others
may extend intracranially and threaten life.
• Approximately 30% of patients have associated neurofibromatosis type I.
• Slowly progressive visual loss, followed later by proptosis, although this sequence may
occasionally be reversed.
• Acute loss of vision due to haemorrhage into the tumour can occur, but is uncommon.
• Proptosis is often non-axial, with temporal or inferior dystopia.
• The optic nerve head, initially swollen, subsequently becomes atrophic.
• Opticociliary collaterals and other fundus signs such as central retinal vein occlusion are
occasionally seen.
• Intracranial spread to the chiasm and hypothalamus may develop.
• Investigations --- MRI , CT in patient with NF1
• Treatment --- observation … surgical excision for growing tumor… radi and chemotherapy
for extension.

55. LYMPHOMA
• Lymphomas of the ocular adnexa constitute approximately 8% of all extranodal lymphomas.
• The majority of orbital lymphomas are non-Hodgkin, and most of these (80%) are of B-cell origin.
• Those affected are typically older individuals.
• The condition may be primary, involving one or both orbits only, or secondary if there are one or more
identical foci elsewhere in the body
• The onset is characteristically insidious.
Symptoms
• An absence of symptoms is common, but may include discomfort, double vision, a bulging eye or a
visible mass.
Signs
• Any part of the orbit may be affected anterior lesions may be palpated, and generally have a rubbery
consistency
• Occasionally the lymphoma may be confined to the conjunctiva or lacrimal glands, sparing the orbit.
• Local lymph nodes should be palpated, but systemic evaluation by an appropriate specialist is required.

56. Investigation
• ○ Orbital imaging, usually with MRI.
• ○ Biopsy is usually performed to establish the diagnosis.
• ○ Systemic investigation to establish the extent of disease.
Treatment
• Radiotherapy is used for localized lesions
• Chemotherapy for disseminated disease and some subtypes.
• Immunotherapy (e.g. rituximab) is a newer modality that may assume a
dominant role in the future.
• Occasionally a well-defined orbital lesion may be resected.

58. RHABDOMYOSARCOMA
• Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood: 40% develop
in the head and neck
• It is the most common primary orbital malignancy in children but s still a rare condition; 90%
occur in children under 16 and the average age of onset is 7 years.
• The tumour is derived from undifferentiated mesenchymal cells that have the potential to
differentiate into striated muscle.
• Various genetic predispositions have been identified, including variants of the RB1 gene
responsible for retinoblastoma.
• Four subtypes are recognized; Embryonal, Alveolar, Botyroid, and Pleomorphic.
Symptoms
• Rapidly progressive unilateral proptosis is usual, and may mimic an inflammatory condition such
as orbital cellulitis.
Signs
• The tumour is most commonly superonasal or superior, but may arise anywhere in the orbit,
including inferiorly. It can also arise in other tissues, such as conjunctiva and uvea.
• Swelling and redness of overlying skin develop but the skin is not warm.
• Diplopia is frequent, but pain is less common.

59. Investigation
• MRI shows a poorly defined mass.
• CT shows a poorly defined mass of homogeneous density, often with adjacent bony
destruction
• Incisional biopsy is performed to confirm the diagnosis and establish the
histopathological subtype and cytogenetic characteristics.
• Systemic investigation for metastasis should be performed; the most common sites
are lung and bone.
Treatment
• Treatment encompasses a combination of radiotherapy, chemotherapy and
sometimes surgical debulking.
• The prognosis for patients with disease confined to the orbit is good.

61. REFERENCES
• American Academy of Ophthalmology, orbit, eyelids and
lacrimal system BSCS, 2014-2015
• Brad Bowling, Kanski’s Clinical Ophthalmology, 8th edition,
2016
• Subrahmanyam Mallajosyula MS, DO,. Surgical Atlas of
Orbital Diseases, 1st edition, 2009

62. THANK YOU