Haitham's Ophthalmology Board Exam Revision- part 1

Haitham’s ophthalmology
board exam revision– Part 1
Dr Haitham Al-Mahrouqi
Oman Medical Specialty Board
2020

Preface
• All the best for those sitting the ophthalmology board exam.
• This revision has most of the common cases. It is organized as (with
few exceptions):
• 1st slide: a photo for description
• 2nd slide: Important points about the case to know

References (+ pictures)
• AAO books 2015-2016
• Kanski
• Yanoff
• Google pictures

Table of contents
• Oculoplasty………………………………………………………………………………p.4
• Vitreoretinal……..……………………………………………………………………p.80
• Uveitis .…………………………………………………………………………………p.173
• Glaucoma .……………………………………………………………………………p.251

Hemangioma
• Vascular hamartoma
• Most common in children (M: F = 3:1). Grows in first 12 months then regresses.
30% by 3 years, 50% by 5 years, 70% by 7 years.
• With large and multiple Kasabach–Merritt syndrome (thrombocytopenia, heart
failure) and PHACEs.
• Treatment indication:
• Amblyopia
• Astigmatism
• Cosmesis.
• Tx:
• Propranolol in the growth phase.
• Steroids in the plataue and shrinkage phase OR for fast resolution as in amblyopia
(intralesional or systemic). 1-2mls of 40/1ml injections. May use topical steroids.
• May need surgical excision.

Orbital lymphangioma
• Vascular hamartoma, from precursors of lymphatic and venous tissue.
• Can occur in the orbit, conjunctiva, eyelid…etc
• Enlarge during URTI and may present with spontaneous hmg with abrupt
proptosis.
• MRI may demonstrate pathognomonic features (multiple grapelike cystic
lesions with fluid-fluid layering of the serum and red blood cells).
• Management:
• For acute bleeding, steroids and percutaneous drainage.
• For macrocysts: slidanafil
• For large vascular component: propranolol
• For microcysts: sclerosing agents like bleomycin.

• Port-wine stain (nevus flemmus)
• 10% associated with sturge weber which has CNS leptomeningeal
involvement and ipsilateral glaucoma (due to high episcleral venous
pressure due to hemangioma). Can have choroidal hemangioma and
iris heterochromaia.
• 50% associated with glaucoma.
• Sharply demarcated dermatomal. Do not blanch
• Tx:
• Screen for glaucoma from infant:
• Skin hypertrophy associated with it may be treated with laser.

• Xeroderma pigmentosa
• Bird-like faces
• Impaired DNA repair mechanism.
• AR
• Prone to get BCC, SCC and melanomas.
• Dry eyes, corneal neovascularization and retinal atrophy also.

A B
C D
Fig. 1.19 Clinical appearance of basal cell carcinoma. (A) Early nodular lesion; (B) larger nodular tumour; (C) rodent ulcer;
(D) large rodent ulcer;
Continued

BCC
• BCC (90% of all eyelid tumors)
• Called rodent ulcer. Pearly edges and ulcered base.
• Histology: peripheral palisading cells.
• Mostly lower eyelid (sun-damaged), followed by medical canthus, upper
eyelid and then lateral (same order as TED muscle involvement).
• Types:
• Nodular
• Noduloulcerative: Rodent ulcer
• Morpheophorm BCC (above pic)
• Systemic association with multiple BCC: Gorlin Goltz syndrome
(predisposed to breast ca and lymphoma).

SCC
• SCC more commonly occurs in the lower eyelid (AAO).
• Can arise from acitinc keratosis or de novo
• Much more aggressive, metastasizes to lymph nodes
• Can present like cutaneous horn or keratoacanthoma.
• Keratin on surface (histology keratin pearls).
• Tx: is surgery.
• Prognosis is worse for medial canthal lesions.
Diagnosis
A pink dome-shaped hyperkeratotic lesion develops, often on the
lower lid (Fig. 1.22B), and may double or treble in size within
weeks (Fig. 1.22C). Growth then ceases for 2–3 months, after which
spontaneous involution occurs, when a keratin-filled crater may
develop (Fig. 1.22D). Complete involution may take up to a year
and usually leaves an unsightly scar.
Treatment
Treatment generally involves complete surgical excision with a
margin of at least 3 mm, or utilizing Mohs surgery; radiotherapy,
cryotherapy or local chemotherapy are sometimes used. Observa-
tion is now regarded as inappropriate.
Keratoacanthoma
Introduction
Keratoacanthoma is a rare, rapidly growing but subsequently
regressing tumour that usually occurs in fair-skinned individuals
with a history of chronic sun exposure. Immunosuppressive
therapy is also a predisposing factor. It is regarded as falling within
the spectrum of SCC, and although invasion and metastasis are
rare, definitive treatment is usually indicated. Histopathologically,
irregular thickened epidermis is surrounded by acanthotic squa-
mous epithelium; a sharp transition from the thickened involved
area to normal adjacent epidermis is referred to as shoulder for-
mation (Fig. 1.22A); a keratin-filled crater may be seen.
Fig. 1.21 Squamous cell carcinoma. (A) Histopathology shows acanthotic squamous epithelium and eosinophilic (pink)
islands of dysplastic squamous epithelium within the dermis; (B) nodular tumour with surface keratosis; (C) ulcerating
tumour; (D) cutaneous horn
(Courtesy of L Horton – fig. A; A Singh, from Clinical Ophthalmic Oncology, Saunders 2007 – fig. B; H Frank – fig. C; S Farley, T Cole and L Rimmer – fig. D)
C D

Keratoacanthoma
• Keratoacanthoma, is a less invasive variant of SCC
• It can regress
• Crater with keratin filled center.
• Grows rapidly
• Tx: Surgery with 3-4mm margin. Use mohs micrographic surgery.

20 Malignant Tumours
lid melanomas are non-pigmented and this may give rise to
diagnostic difficulty. Features suggestive of melanoma include
recent onset of a pigmented lesion, change in an existing pig-
mented lesion, irregular margins, asymmetrical shape, colour
change or presence of multiple colours, and diameter greater than
6 mm.
Lentigo maligna
Lentigo maligna (melanoma in situ, intraepidermal melanoma,
Hutchinson freckle) is an uncommon condition that develops in
sun-damaged skin in elderly individuals. Malignant change may
occur, with infiltration of the dermis. Histopathology shows
intraepidermal proliferation of spindle-shaped atypical melano-
cytes replacing the basal layer of the epidermis (Fig. 1.24A). Clini-
cally lentigo maligna presents as a slowly expanding pigmented
macule with an irregular border (Fig. 1.24B). Treatment is usually
by excision. Nodular thickening and areas of irregular pig-
mentation are highly suggestive of malignant transformation
(Fig. 1.24C).
Melanoma
Histopathology shows large atypical melanocytes invading the
dermis (Fig. 1.25A). Superficial spreading melanoma is character-
ized by a plaque with an irregular outline and variable pigmenta-
tion (Fig. 1.25B). Nodular melanoma is typically a blue – black
nodule surrounded by normal skin (Fig. 1.25C). Treatment is
usually by wide excision and may include local lymph node
removal. Radiotherapy, chemotherapy, biological and ‘targeted’
therapy may also be used, generally as adjuvants.
Merkel cell carcinoma
Merkel cells are a form of sensory receptor concerned with light
touch. Merkel cell carcinoma is a rapidly growing, highly malig-
nant tumour that typically affects older adults. Its rarity may lead
to difficulty in diagnosis and delay in treatment, and 50% of
patients have metastatic spread by presentation. A violaceous,
well-demarcated nodule with intact overlying skin is seen, most
A
B
Melanoma
Histopathology shows large atypical melanocytes invadin
dermis (Fig. 1.25A). Superficial spreading melanoma is char
ized by a plaque with an irregular outline and variable pigm
tion (Fig. 1.25B). Nodular melanoma is typically a blue –
nodule surrounded by normal skin (Fig. 1.25C). Treatme
usually by wide excision and may include local lymph
removal. Radiotherapy, chemotherapy, biological and ‘targ
therapy may also be used, generally as adjuvants.
Merkel cell carcinoma
Merkel cells are a form of sensory receptor concerned with
touch. Merkel cell carcinoma is a rapidly growing, highly m
nant tumour that typically affects older adults. Its rarity may
to difficulty in diagnosis and delay in treatment, and 50
patients have metastatic spread by presentation. A violac
well-demarcated nodule with intact overlying skin is seen,
frequently involving the upper eyelid (Fig. 1.26). Treatment
excision, often with adjuvant therapy.
Kaposi sarcoma
Kaposi sarcoma is a vascular tumour that typically affects pa
with AIDS. Many patients have advanced systemic disease alth
in some instances the tumour may be the only clinical mani
tion of human immunodeficiency virus (HIV) infection. His
thology shows proliferating spindle cells, vascular channel
inflammatory cells within the dermis (Fig. 1.27A). Clinic
pink, red-violet to brown lesion (Fig. 1.27B) develops, which
be mistaken for a haematoma or naevus. Treatment is by r
therapy or excision, and by optimal control of AIDS w
relevant.
Fig. 1.23 Sebaceous gland carcinoma. (A) Histopathology
shows cells with large hyperchromatic nuclei and
vacuolated cytoplasm; (B) nodular tumour; (C) spreading
tumour
(Courtesy of A Garner – fig. A; A Singh, from Clinical Ophthalmic Oncology,
Saunders 2007 – fig. B; S Tuft – fig. C)
B
C

Sebaceous cell carcinoma
• Arise from Holocrine glands: Sebaceous and meibomian glands or caruncle.
• More likely in the upper eyelid (higher number of meibomian glands)
• Missed diagnosis as chalazion or chronic blepharitis
• Loss of eyelashes, widening of tarsal plate, loss of the normal Meibomian
gland orifices
• Tx
• Surgery: Spreads to local lymph nodes rather than blood. Sentinal node biopsy can
be used for frozen sections. Intra-epidermal spread (pagetoid) over the palpebral and
bulbar conj with skip areas, therefore need for map biopsy of conj
• Radio-resistent

A
B
C
A
B
Fig. 1.24 Lentigo maligna of the eyelid. (A) Histopathology
shows melanoma cells proliferating within the basal layers
of the epidermis; (B) early lentigo maligna; (C) melanoma
arising from lentigo maligna
(Courtesy of L Horton – ﬁg. A; S Delva – ﬁg. C)
A
B
C
A
B
C

• Melanoma can be in situ (called lentigo maligna as the first picture) or
invasive melanoma (second pic).
• Uncommon but lethal if in eyelid.
• Tx:
• Surgery and sentinel mode bioposy.
• Chemo and radio may be needed.

Cavernous hemangioma
• Most common benign orbital tumor, hamartoma.
• Middle aged women
• Encapsulated
• Needs surgical resection if optic nerve compromise or severe
proptosis.

104 Lacrimal Gland Tumours
Fig. 3.35 Pleomorphic lacrimal gland adenoma. (A) Histology
A B
C D
104 Lacrimal Gland Tumours
Fig. 3.35 Pleomorphic lacrimal gland adenoma. (A) Histology
shows glandular tissue and squamous differentiation with
keratin formation; (B) inferonasal dystopia due to a tumour
arising from the orbital lobe; (C) eyelid swelling without
dystopia; (D) eversion of the upper eyelid reveals the
tumour; (E) coronal CT showing an orbital lobe lesion
(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology,
Butterworth-Heinemann 2001 – ﬁg. A; A Pearson – ﬁgs B and E)
A B
C D
E

Pleomorphic adenoma (benign mixed tumor)
• Both epithelial (keratin) and mesenchymal cells (cartilage and glands)
• Non painful. Most common lacrimal gland tumor
• Orbital lobe more common (needs lateral orbitotomy), presents with
more than 12 months mass and dystopia.
• Palpebral lobe less common (anterior orbitotomy), presents with
mass.
• Imaging shows well circumscribed lesion with indentation of bone. No
dustruction.
• En-block resection otherwise recurrence and malignant
transformation.

Fig. 3.37 Lacrimal gland carcinoma. (A) Histology of adenoid
cystic carcinoma shows nests of basaloid cells with solid
A
B
C
Treatment
Treatment involves excision of the tumour and adjacent tissues.
Extensive tumours may require orbital exenteration or
midfacial resection, but the prognosis for life is frequently poor.
Radiotherapy combined with local resection may prolong life and
reduce pain. Adjuvant intra-arterial chemotherapy and/or brachy-
therapy may be utilized in some cases.
NEURAL TUMOURS
Optic nerve glioma
Introduction
Optic nerve glioma is a slowly growing, pilocytic astrocytoma that
typically affects children (median age 6.5 years); histopathology
shows spindle-shaped pilocytic (hair-like) astrocytes and glial ﬁla-
ments (Fig. 3.38A). The prognosis is variable; some have an indo-
lentcoursewithlittlegrowth,whileothersmayextendintracranially
and threaten life. Approximately 30% of patients have associated
neuroﬁbromatosis type I (NF1 – see Ch. 19) and in these patients
the prognosis is generally superior. Malignant glioma (glioblast-
oma) is rare, has a very poor prognosis, and usually occurs in adult
males.
Diagnosis
• Symptoms
○ 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.
• Signs
○ Proptosis is often non-axial, with temporal or inferior
dystopia (Fig. 3.38B).
○ The optic nerve head, initially swollen, subsequently
becomes atrophic.
○ Opticociliary collaterals (see Fig. 3.6C) and other fundus
signs such as central retinal vein occlusion are
occasionally seen.
○ Intracranial spread to the chiasm and hypothalamus may

Adenoid cystic carcinoma (cylindroma)
• Rare. But very mortal.
• Can arise from previous pleomorphic adenoma
• Painful (perineural spread)
• Swiss cheese pattern.
• Erodes bone with calcifications.
• Biopsy and then assess with imaging for Perineural spread.
• Tx:
• Exenteration with adjuvant radiochemotherapy.

ON glioma
• Children. 50% have NF1 and tend to extend to the brain as opposed to
those who are not NF1.
• Arises from astrocytes, spindle shaped on histology.
• Fusiform enlargement. with kinking in NF1 especially.
• Treatment:
• Observation
• Chemotherapy especially in children as radiotherapy can affect the pituitary function
in CNS disease and thus result in growth retardation.
• Radiotherapy especially if CNS involvement
• Surgery if no vision only or rapid growth.
• Malignant counterpart glioblastoma occurs in young males.

Carotid cavernous fistula
• Types as per schedual. Arteriography definitely diffrenciate between
the two or MRA.
• Direct due to trauma in 70% of patients.
• Signs: pulsating proptosis, bruit, tortous conjunctival vessels, high IOP,
dilated SOV on CT scan. Orbital color Doppler US shows reversed,
arterialized flow in superior ophthalmic vein.
• Tx:. Treat the proptosis with lubrication
• Treat the glaucoma with aqueous suppressants.
• Low flow (indirect) normally close on their own
• High flow: Refer to neurosurgery for embolization or coiling.

ON sheath meningioma
• Middle aged women
• Associated with NF 2
• 2/3 extends from a primary intracranial meningioma.
• Histology: psamomma bodies.
• Radiologically: Tram-track appearance with calcifications.
• The classic (Hoyt–Spencer) triad consists of progressive visual loss,
optic atrophy and opticociliary shunt
• Tx: Observation, radiotherapy for vision sparing, surgery if no vision.

pathology shows widely dilated blood-filled spaces separated by fibrous septa; (B) and
gression of port-wine stain over time, with associated underlying soft tissue hypertrophy
Fig. 1.16 Plexiform neurofibroma – characteristic S-shaped
upper lid
(Courtesy of J Harry)

Plexiform neurofibroma
• Neural hamartoma
• Presents in childhood
• Bag of worms
• Pathgnomonic for NF1
• May become malignant
• High chance of glaucoma if plexiform
• Resection is difficult due to bleeding and orbital extension.

○ Swelling and redness of overlying skin develop but the
skin is not warm (see Fig. 3.41A).
○ Diplopia is frequent, but pain is less common.
• Investigation
○ MRI shows a poorly defined mass (Fig. 3.41C).
○ CT shows a poorly defined mass of homogeneous
density, often with adjacent bony destruction (Fig. 3.41D).
○ 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
Commonly used guidelines for staging and a corresponding treat-
ment protocol were produced by the Intergroup Rhabdomyosar-
coma Study Group (IRSG); treatment encompasses a combination
of radiotherapy, chemotherapy and sometimes surgical debulking.
The prognosis for patients with disease confined to the orbit is
good.
METASTATIC TUMOURS
Adult metastatic tumours
Introduction
Orbital metastases are an infrequent cause of proptosis, and are
much less common than metastases to the choroid. If the orbit is
the site of initial manifestation of the tumour, the ophthalmologist
may be the first specialist to see the patient. In approximate order
of frequency the most common primary sites are breast (up to
70%), bronchus, prostate, skin (melanoma), gastrointestinal tract
and kidney.
Diagnosis
• Signs. Associated with the range of tumours that can spread
to the orbit, presentation can take a variety of forms.
○ Dystopia and proptosis (Fig. 3.42A) are the most
common features.
○ Infiltration of orbital tissues characterized by ptosis,
diplopia, brawny indurated periorbital skin and a firm
orbit, with resistance to manual retropulsion of the globe.
○ Enophthalmos with scirrhous tumours.
○ Chronic inflammation.
○ Primarily with cranial nerve involvement (II, III, IV, V,
VI) and only mild proptosis with orbital apex lesions.
A
B
C

• Orbital lymphoma is Non-hodgkins and B-cell in origin.
• Can be primary or secondary (if elsewhere site is identified as
primary).
• lymphoma of eyelids (67% have systemic involvement) > orbit (35%) >
conjunctiva (20%); 90% 5-year survival..
• Treatment: Radiotherapy for localized lesions, chemo for systemic
involvement.

Rhabdomyosarcoma
• Most common orbital malignancy in
children. Arises from mesenchymal
tissue.
• Histology: Cross-striations like actin
and myocin.
• Average age 7
• Superionasal
• 4 types
• Needs biopsy: systemic workup,
common sites include lungs and bone.
• Tx: Radiochemotherapy, sometimes
surgical debulking.
Type characteristics
Pleomorphic Best prognosis.
Embryonal Most common (everyone). 85% of cases.
Superionasal
Alveolar Inferior. Majority of remaining.
Botyroid Variant of embryonal. Grape like. Extends
from sinuses.
Figure 31.3. Axial computed tomography showing superonasal orbital
mass.
Figure 3
Coronal C
best app
biopsy w
Figure 31.5. Histopathology showing malignant strap cells with cross-
striations and some larger round cells with abundant extracellular
matrix. (Hematoxylin-eosin 200.)
Figure 31
in the aff

Neuroblastoma
• Most common metastatic tumor of childhood
• Originates from adrenal or sympathetic trunk
• Associated with sudden proptosis (with bone destruction), OU ecchymosis
and Opsoclonus (saccadomania; random, rapid eye movements in all
directions that disappear during sleep), horner syndrome.
• Tx:
• Chemoradiotherapy
• Prognosis is good if <1 year.
• Ocular flutter and opsoclonus: the defoveating movement is a saccade.
Flutter is purely horizontal, opsoclonus is multi-directionaly. Causes
including viral encaphlitis,

2 year old with skin and iris lesion

Juvenile xanthogranuloma
• Childhood disease < 5 years old
• Presents with granulomatous orange lesions in the skin and iris.
• The iris lesion is always UNILATERAL
• Regresses after 5 years.
• The risk of spontaneous hyphaema is large and thus warrents
prophylactic treatment:
• Tapering frequent steroids
• Radiotherapy
• Excision

5 year old child with pain and reduced vision

Medulloepithellioma
• Childhood disease
• May present with pain, cataract, ectopia lentis, reduced vision, glaucoma.
• Originates from non-pigmented epithelium
• Histologically: Ribbon like structure.
• Nonteratoid or simple: pure proliferation of embryonic nonpigmented ciliary epithelium
• Teratoid: contains heterotopic elements such as cartilage, brain tissue
• Tx:
• Surgery with enucleation
• Prognosis:
• Good except if local invasion.
• Rarely metastasizes

2 year old child with left superiotemporal
puffiness and erythema not responding to VI
antibiotics.

Orbital eosinophilic granuloma (spectrum of
histiocytosis X or langerhan cell histiocytosis)
• Childhood disease (although reported in adults)
• Can be localized like this disease or disseminated to involve all hematopoetically active bones (like
superiotemporal orbit).
• Produces proptosis and lytic lesions because the clones or the histiocytes produce IL-1 which
causes bone destruction.
• Hand-Schuller-Christian disease (multifocal lytic lesions) and Letterer-Siwe disease (fatal
disseminated disease).
• Tx:
• Must exclude disseminated disease by bone marrow biopsy and exclude diabeted insipidus (thought to occur
due to involvement of the pituitary gland).
• Biopsy with curretage
• Local treatment include intralesional steroids and low dose radiotherapy.
• Systemic steroids and chemo.
• Prognosis:
Poor if < 2 years old and in disseminated disease.

3 year old. No other limbal abnormalities

Craniocynostosis
• Most common disease is Crouzon’s syndrome
• AD
• Does not have any other limbal abnormalities
• Results in proptosis, hypertelorism and hydrocephalus only.
• Normal intelligence.
• Complications of craniocynostosis:
• Corneal exposure
• Optic nerve compromise
• Subluxated globe (leads to corneal exposure and reduced blood supply to the globe; reposition with thumb
and finger). Lateral tarsorrhaphy may be done if recurrent.
• V-pattern exotropia
• NLDO
Abnormalities in the development of the cranium and brancial arches result in:
• Goldenhar: skin tags, dermoids, dermolipomas, upper eyelid coloboma, duane retraction syndrome.

Mucormycosis
• Acquired from inhalation of spores
• Causes septic necrosis due to occlusive vasculitis (hyphae invade
blood vessels).
• Occurs in immunocompromised and diabetic ketoacidosis patients.
• black eschar that may develop on the palate, turbinates, nasal
septum, skin and eyelids
• Treat with IV amphotericin B (liposomal amphotericin B which is less
toxic) and surgical debridment + hyperbaric oxygen

• Trauma with cricket ball
864 Orbital Trauma
ORBITAL TRAUMA
Orbital floor fracture
Introduction
A blow-out fracture of the orbital floor is typically caused by a
sudden increase in the orbital pressure from an impacting object
that is greater in diameter than the orbital aperture (about 5 cm),
such as a fist or tennis ball, so that the eyeball itself is displaced
and transmits rather than absorbs the impact (Fig. 21.5). Since the
bones of the lateral wall and the roof are usually able to withstand
such trauma, the fracture most frequently involves the floor of the
orbit along the thin bone covering the infraorbital canal. Occa-
sionally, the medial orbital wall may also be fractured; fractures of
the orbital rim and adjacent facial bones require appropriately
tailored management. Clinical features vary with the severity of
trauma and the interval between injury and examination. Care
should be taken to ensure that a full evaluation for head and sys-
temic injury has been performed, and any necessary interspecialty
referrals initiated.
Diagnosis
• Visual function, especially acuity, should be recorded and
monitored as necessary, particularly in the acute situation.
• Periocular signs include variable ecchymosis, oedema (Fig.
21.6A) and occasionally subcutaneous emphysema (a
crackling sensation on palpation due to air in the
subcutaneous tissues).
• Infraorbital nerve anaesthesia involving the lower lid,
cheek, side of nose, upper lip, upper teeth and gums is very
common as the fracture frequently involves the infraorbital
canal.
• Diplopia may be caused by one of the following
mechanisms:
○ Haemorrhage and oedema in the orbit may causing the
septa connecting the inferior rectus and inferior oblique Fig. 21.6 Right orbital floor blow-out fracture. (A) Marked
periocular ecchymosis, oedema and subconjunctival
haemorrhage; (B) restricted elevation; (C) mild right
enophthalmos
(Courtesy of S Chen – fig. A)
A
B
C

• Approach to orbital trauma:
• Ensure life not at risk (intracranial bleed) + no oculocardiac reflex.
• Globe and ON intact
• Exclude Orbital wall #
• Blow-out fracture: Injury which is bigger than entrance of orbit which increase the
intraorbital pressure so bones fracture.
• Tx: Not to blow nose, decongestants, cold compressors, oral Abx.
• Surgery indicated (after 2 weeks to reduce oedema. If left for long time, there might be
fibrosis) if:
• Trapped muscle with diplopia
• Enopthalmos > 2mm
• Large fracture (usually occurs along the inferorbital fissure).
• Early surgery indicated if :
• Significant oculocardiac reflex
• Trap-door fracture seen in young individuals.
Fig. 21.5 Mechanism of an orbital ﬂoor blow-out fracture

• Indirect # of medial wall involve the posteriomedial part.
• Direct # are 3 types:
• type I being a central fragment of bone attached to canthal tendon,
• type II having comminuted fracture of the central fragment, and
• type III having a comminuted tendon attachment or avulsed tendon
• Patients present with orbital emphysema and possible
telecanthus in direct injuries if type III.
• Tx: Miniplate for the #, and trans-nasal wiring for the tendon.
• Orbital roof #: Occurs in children before pneumatization of the
frontal sinus which acts as a crumple zone in adults preventing #.
CHA PTER 6: Orbital Trauma •
Type I
Type II
Type Ill
Figure 6-4 Nasa-orbital-ethmoidal fractu res result in traumatic telecanthus w ith roundin
the medial canthus. Types I-III are described depending on the severity of the injury. !Illust
by Chrisrine Gralapp.)

• Mechanism and Treatment of indirect optic neuropathy
• Mechanism of optic nerve avlusion

• Frontal bone injury, acceleration of the nerve at the optic
canal where it is tethered to the dural sheath, thought to
rupture the microvascular supply), secondary vasospasm,
oedema and transmission of a shock wave through the
orbit.
• Tx:
• Observation
• Medical (IV steroids loading 30mg/kg and then 15mg/kg qid).
Must be started in the first 8 hours.
• Decompression of the optic canal medial wall, must be
performed in the first 5 days.
• CRASH study (acute brain injury), found patient receiving
steroids were more likely to die!
• Optic nerve avlusion associated with rotational injuries or
anterior displacement of the globe.
of brain dam
sequent hypo
ischaemia.
• Presentat
vomiting,
gastroente
injury is w
• Systemic
ranging fr
and subar
survivors
rib and lo
examinati
• Ocular fe
○ Retina
the mo
involve
retinal
pole, b
○ Periocu
○ Poor v
○ Visual
result o
Penetrat
Introductio
Penetrating in
females, and
15–34). The m
pational accid
could be prev
The extent of
Abusive head trauma (shaken baby syndrome) is a form of physi-
cal abuse occurring typically in children under the age of 2 years.
Mortality is more than 25%, and it is responsible for up to
50% of deaths from child abuse. It is caused principally by violent
shaking, often in association with impact injury to the head,
and should be considered in conjunction with a specialist paedia-
trician whenever characteristic ophthalmic features are identiﬁed.
The pattern of injury results from rotational acceleration and
deceleration of the head, in contrast to the linear forces generated
by falls. It is thought that direct trauma is not the main mechanism
Fig. 21.22 Optic nerve avulsion
(Courtesy of J Donald M Gass, from Stereoscopic Atlas of Macular Diseases,
Mosby 1997)

L1
204
muscle creates less pull on the skin so the crease is less dis-
tinct or “weak.” Read this paragraph again.
The vital signs will give you the information that you need
to know to classify and treat ptosis. You will see the follow-
ing concepts repeated several times in this chapter:
Simple congenital ptosis is associated with decreased
levator function
Involutional ptosis is associated with normal levator
function
If levator function is normal, a shortening of the
aponeurosis works well to lift the upper lid (a levator
aponeurosis advancement operation)
If the levator function is poor, the eyelid is surgically
“connected” to the brow (a frontalis sling operation).
The action of the frontalis muscle lifts the upper eyelid
Notice how important the levator function is. As you become
experienced in taking care of patients with ptosis, you will
see that asking “What is the levator function?” becomes the
critical question (Box 8-1). We will discuss this further in the
next section.
Checkpoint
Name the retractors of the upper eyelid
What are the “eyelid vital signs”? What are the normal
measurements? Remember these findings indicate the
“health” of the upper eyelid
What are the two most common types of ptosis?
Which of the vital signs determines the treatment of
ptosis?
Classification of ptosis
A simplified system
There are many types of ptosis and many systems that c
sify the types (Box 8-2). From a practical point of view, m
patients you will see have either simple congenital ptosi
involutional ptosis. Most of the children you will see h
simple congenital ptosis. Most of the older adults you
see have an involutional ptosis. I group the other
common types into the category unusual ptosis. This is
oversimplification, but it works well.
The levator function: the relationship
to classification
For this approach to work, you must learn the comm
types of ptosis well. Most of our medical training is sp
learning the details of diseases that we will rarely see,
example being ptosis secondary to myasthenia gravis.
now, I will be emphasizing the two common types of pto
After you know all about these two types, you will be a
to easily identify an unusual type of ptosis when you
something that doesn’t fit with the two common types.
Box 8-2
Types of Ptosis: A Simplified System

• First exclude pseudoptosis
• Causes:
• Neurological: CN III palsy, Horner
• NMJ: MG
• Aponurotic
• Myogenic
• Mechanical or traumatic
• In history: Ask about time since symptom, diplopia, periodicity, trauma,
pain.
• Vital signs of the eyelid!
• What is pre-tarsal show and skin fold??
Simple congenital ptosis is associated with reduced
levator function
Involutional ptosis is associated with normal or near
normal levator function
Unusual types of ptosis are usually associated with
reduced levator function, but levator function also can
be normal
When I see a patient with ptosis, I classify the ptosis into
one of these three categories. Most patients will have invo-
lutional or simple congenital ptosis. Congenital ptosis is
much less common than the acquired forms of ptosis; con-
sequently, the majority of patients will have involutional
ptosis.
Tips for measuring the levator function
Figure 8-6 Measurement of the levator function. Note the position of the thumb on the brow, preventing any eyelid elevation by the frontalis muscle.
(A) Eyelid in extreme downgaze. (B) Eyelid in extreme upgaze. The excursion of the eyelid, or levator function, is 15 mm in this case.
A B
Box 8-3
Levator Function
Levator function is the basis of both classification and treatment
of ptosis. The extremes are:
Seen in involutional ptosis
The levator aponeurosis advancement operation is the
treatment
Seen in simple congenital ptosis
The frontalis sling operation (for patients with poor levator
function) is the treatment
Classification of ptosis
Evaluation and treatment of the patient with ptosis8
Figure 8-5 The frontalis muscle is a weak elevator of the eyelid.
We have already mentioned the MRD as a simple way to
measure the height of the upper lid. The most practical
measure of the strength of the levator muscle is the levator
function. The levator function is defined as the excursion of
the upper lid from extreme downgaze to extreme upgaze
measured in millimeters. This movement is normally 15 mm.
You will recall that the upper eyelid skin crease is created by
the pull of the levator aponeurosis on the skin. The skin
crease height is the distance from the lid margin to the crease.
This height varies among individuals, but averages 6–8 mm
for men and 8–10 mm for women at the highest point. The
Box 8-1
The “Eyelid Vital Signs”
The status and health of the upper lid are measured by the
“eyelid vital signs:”
The MRD describes the eyelid position:
1 measures the distance from the central light reflex
to the upper lid margin
Levator function is an indicator of the strength of the
levator muscle:
eyelid from downgaze to upgaze
The upper lid skin crease is formed by the pulling of the
levator aponeurosis from beneath skin:
recognizing the amount of levator function present and the
type of ptosis
there is reduced pull
for symmetry and an optimal surgical result

• Treatment is based on lavator
function.
• Congenital ptosis: frontalis sling
(fox pentagon)/lavator resection
but not routine as the muscle is
fibrotic.
• Senile aponeurotic: mullerectomy
(if < 2mm and responds to PE test
and good lavator action) or lavator
advancement/re-
inserion/resection.
L1
220
tively. With this technique, the levator aponeurosis is
advanced a certain amount, depending on the preoperative
MRD. There is no adjustment based on levator function. The
amount of resection is determined preoperatively. For mild ptosis
(MRD1 of 3 mm), the resection would be 10–13 mm. For
severe ptosis (MRD1 of 0 mm), the amount of resection
would be 23 mm or more (Box 8-10).
Although these techniques seem quite different, they are
similar in principle because the degree of ptosis is usually
related to the amount of levator function. I rely mainly on
the levator function technique, but combine the estimates (part
of the “art” of ptosis surgery). If a patient has moderate
ptosis with an MRD1 of 2 mm and the levator function is
6 mm, I start with a resection of about 16 mm. If the intra-
operative height is lower than where I want it postopera-
tively, I will resect a little more. If the intraoperative lid
height is higher than where I want it to be, I will back off a
few millimeters. You can see that the amount to resect is an
educated guess. A good rule to follow is that, if you don’t think
the lid is high enough intraoperatively, resect more. It is difﬁcult
to overcorrect congenital ptosis.
You should notice that the least amount of resection is 10 mm.
Whitnall’s ligament is usually at about 13 mm in a child so
Box 8-10
Estimating the Amount of Resection for Simple
Congenital Ptosis
Levator function technique (based on Berke, 1959)
Levator function Intraoperative lid height
At upper limbus
2 mm overlap
5 mm overlap
MRD technique (based on Beard, 1981)
Preoperative MRD1 Amount of resection
>23 mm
Surgical correction of eyelid ptosis
D
E

• Other types of ptosis
• Marcus Gunn jaw winking: Mis-wiring between agenetic 3rd nerve and 5th.
Treatment is by lavator disinsertion and lavator sling.
• Blepharophimosis syndrome: AD disease. Tx is by frontalis sling and then
aesthetic surgery.
• Horner:
• MG: Fatigability test (look up for 60 seconds), ice-pack test (2 mins of cold
pack, ptosis improves due to slowing of the Ach esterase in the NMJ) tenislon
(edrophonium), single fibre EMG and anti-ACH receptor antibodies (only
present in 70% of ocular MG and 90% in systemic disease). Tx is by
physostigamine, steroids and other immunomodulators or IG plasmaphoresis
+ Thymomectomy.

Ectropion
• Most common is involutional and lower eyelid.
• Congenital: Vertical insufficiency of the anterior lamella. Associated
with blepharophimosis syndrome, down syndrome. Treated as
cicatricial with lateral strip.
• Acquired:
• Involutional (due to lax lower eyelid retractors treated with lateral tarsal
strip),
• Cicatricial (fibrosis causing shortening of the anterior lamella and treated with
full thickness skin graft)
• Neurogenic (Facial nerve palsy)
• Mechanical.

Major points
Ectropion of the eyelid is said to occur when the eyelid
margin is everted off the eyeball.
Three types of ectropion occur:
Involutional ectropion is the most commonly seen form of
ectropion. Lower lid laxity is the cause of the involutional
ectropion. Lower lid laxity often accompanies cicatricial and
paralytic ectropion as well.
Cicatricial ectropion is caused by shortening of the anterior
lamella.
Paralytic ectropion is caused by a loss of orbicularis muscle
tone associated with facial nerve paralysis.
The history and physical examination will determine the
etiology and best treatment of the ectropion.
Involutional ectropion is treated with lower lid tightening
using the lateral tarsal strip operation.
operation that you can learn in oculoplastic surgery
the lateral tarsal strip operation for correcting punctal
eversion
Cicatricial ectropion is treated with full-thickness skin grafting,
often in conjunction with the lateral tarsal strip procedure.
Paralytic ectropion is treated with a lateral tarsal strip
operation.
exposure and brow ptosis, are necessary in the treatment
of facial nerve palsy
The eversion of the upper eyelid that occurs in
ﬂoppy eyelid syndrome is caused by extreme upper eyelid
laxity.
syndrome
the lateral upper eyelid
Suggested reading

include lubricants, taping, soft bandage contact lenses and
orbicularis chemodenervation with botulinum toxin injection.
ectropion.
l sling –
er lid is passed
the upper lid
as of Ophthalmic Plastic
Preseptal over-riding
A

• Most common is cicatricial upper eyelid.
• Congenital: Insufficiency of the posterior lamella
• Acquired:
• Involutional: Either due to horizontal eyelid laxity or disinsertion of the lower eyelid retractors or
over-riding of the pre-septal orbicularis muscle .
• Cicatricial: Fibrosis of the posterior lamella causes shortening. It can be only marginal entropion
which is the most common form of cicatricial entropion normally causes by trochoma.
• Spasmic: Due to irritation of the globe or neurological. Lax eyelids is a contributing factor. As with
lax eyelids, causes irritation of the globe and then spasms.
• Mechanical
• Tx:
• Tarsal fracture
• Lateral tarsal strip
• Re-insertion of the lower eyelid retractors
• Everting sutures or taping.

The diagnosis and treatment of entropion4
Major points
Entropion of the eyelid occurs when the lid margin inverts or
turns against the eyeball. There are four main types of
entropion:
Factors contributing to lower lid involutional entropion
include:
Shortening of the posterior lamella causes cicatricial
entropion.
Identiﬁcation of the cause of the entropion is the key to
deﬁning treatment.
Treatment of lower lid involutional entropion is:
Treatment of mild cicatricial entropion uses incisions with
sutures to stabilize the lid while healing occurs, restoring the
length of the posterior lamella:
operation
operation (some surgeons prefer the tarsal fracture
procedure; if you use this, be sure to bury the sutures to
protect the cornea. A bandage contact lens may be
required for a few weeks)
For mild to moderate upper eyelid entropion, excision of the
anterior lamella containing the eyelash follicles is an
alternative to rotation procedures. This procedure shortens
the anterior lamella to match the posterior lamella length and
removes all eyelashes.
Treatment of moderate or severe cicatricial entropion
requires lysis of the scar tissue and mucous membrane grafts
to lengthen the posterior lamella.

Trichiasis (mis-directed eyelashes)
trauma
3. The entire length of the lower lid with marginal
entropion
4. The entire length of the upper lid with marginal
entropion
5. A child with epiblepharon and photophobia
5. Lower lid blepharoplasty
6. Lid splitting with posterior lamellar cryotherapy
Cicatricial and involutional entropion also cause the lashes
to rub against the eye but are considered speciﬁc entities,
rather than causes of trichiasis.
Major points
The normal landmarks on the lid margin include:
Any misdirection of the eyelashes is known as trichiasis.
The most common cause of trichiasis in adults is marginal
entropion.
The signs of marginal entropion include:
Trauma may cause trichiasis with or without lid margin
irregularities.
parallel
The treatment options for trichiasis include:
reconstruction
Two childhood conditions cause the eyelashes to rub the
cornea:
lamellar cryotherapy
Suggested reading 8. Kersten RC, Kleiner FP, Kulwin DR: Tarsotomy for the

Eyelid retraction
• Causes:
• TED
• Trauma
• Post surgery correction of ptosis
• Parinaud syndrome
• Tx:
• Muller muscle or lavator recession

Blepharophimosis syndrome
• Consentallation of: Epicanthus inversus, telecanthus, ectropion ptosis
and phimosis.
• AD (may or may not be associated with ovarian failure).
• Inheritance is usually autosomal dominant; both BPES type I (with
premature ovarian failure) and BPES type II (without premature
ovarian failure) are caused by mutations in the FOXL2 gene on
chromosome 3
• Tx:
Treat epicanthus inversus with V-Y or Z plasty and then telecanthus and
last frontalis suspension.

45 year old lady with FB sensation in the eyes

Thyroid eye disease (TED)
• 50% of graves disease patients will develop some TED
• 90% of TED have graves disease
• TED may preceed, coincide or proceed graves. TED patients develop graves hyperthyroidism 25% in 1 year
and 50% in 5 years.
• Pathogenesis: orbital fibroblasts secreting GAG and also diffrentiating to adipocytes and secret fat (more in
young).
• Signs?
• CT: fusiform enlargement of (in order) inferior, medial, superior and lateral.
• Tx:
• Stop smoking, restore euthyroid state, lubrication
• Self limiting disorder lasting 1 year in non-smokers and 3 years smokers, followed by quiescent phase.
• If moderate to severe disease: steroids 1mg/kg (Tapered over 4-6 weeks) OR IV methylpred 500mg once weekly for 6 weeks,
then 250mg weekly for 6 weeks; total 4.5 g
• Vision threatening condition require, IV methylpred or radiotherapy or decompression.
• Patients younger than 40 years demonstrate enlargement of the orbital fat compartment, whereas those older than 40
typically show more significant extraocular muscle enlargement. This difference determines the effectiveness of bone versus
fat decompression surgery.
• Orbital decompression involves medial wall followed by inferior and then lateral.
• After quiescence order of surgery: orbital decompression, strabismus and last eyelid.

40 year old with discharging lower punctum

Canaliculitis
• The most common pathogen is a filamentous gram-positive rod,
Actinomyces israelii
• Tx:
• Canaliculotomy and removal of the concretions
• Irrigating with penicillin and bovidone
• Broad spectrum antibiotics until sensitivity.

1) Difference of RD from
retinoschisis
2) Difference between
acute and chronic RD

• Degenerative retinoschisis is associated with hyperopia whereas RD
associated with myopia. The typical form is thought to be a
progression of cystoid degeneration in the OPL and thus will have
absolute scotoma and this is rarely associated with RD. The reticular
form occurs in the RNFL and may have holes in both leaflets thus RD
may occur.
• Chronic vs acute RD:
• Demarcation line
• Pigmentation
• PVR
• Smooth surface

• DR vascular abnormalities:
• Arterioles: flame shaped hmgs in RNFL
• Capillaries: Microaneurysms are saccular
or from fusion of 2 looped capillaries.
They can leak exudate or thrombose thus
block perfusion and thus are found mostly
near capillary non-perfusion areas.
Capillary or microaneurysm chronic
leakage and absorption of fluid leads to
hard exudates (lipoproteins and
macrophage filled with lipid and if large
cholestrol), found in OPL. Extensive
capillary leakage leads to DME.
• Venules: Dot and blot hmgs in deeper
retinal layers. Dark hmgs are hemorrhagic
retinal infarcts.
524 Diabetic Retinopathy
oedematous. With central accumulation of fluid the fovea assumes○ Chronic leakage leads to enlargement and the deposition
Fig. 13.4 Retinal haemorrhages. (A) Histology shows blood lying diffusely in the retinal nerve fibre and ganglion cell layers
and as globules in the outer layers; (B) retinal nerve fibre layer (flame) haemorrhages; (C) dot and blot haemorrhages;
(D) deep dark haemorrhages
(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A)
A B
C D

• Indications for scleral buckle, over vitrectomy over pneumatic
retinopexy?

Retinal detachment
• Indications for:
• Vitrectomy: Giant retinal tear, PVR, RRD, posterior breaks or break not
visualized.
• Scleral buckle: Inferior break, young patient (no PVD), simple RD, break can be
visualized
• Pneumatic: Superior break within 8 clock hours, no PVR, all breaks identified
and close to each other, patient can tolerate positioning.

Important percentages (higher ends; up to)
Lattice degeneration in general population: 10%
• Lattice degeneration in RD: 30%
• Cilioretinal artery in general population: 30%
• Retinal tears in symptomatic PVD: 10%
• Smokestack FFA in CSR: 10%
• Common canaliculus: Present in 90%
• Steroid response in general population: 5%

• What are the stages of DR? and when to follow-up?
• What is CSME?
• What is high risk PDR?
• What is ischaemic maculopathy?
• Treatment of DR?
• Lifestyle
• Medical
• Surgical

Diabetic papillopathy
• is uncommon.
• The telengectatic vessels may be mistaken for NVD.
• Diffuse ischaemia of the ONH from ? Blocked posterior ciliary arteries.
• Variant of NA-AION
• Disc swelling although is more commonly bilateral and need to exclude
high ICP.
• Need to exclude from RVOs.
• The swelling is more pronounced than other causes of disc swelling.
• Treatment: treat underlying DR, some people try Anti-VEGF.
• Prognosis: Will lead to optic disc atrophy.

CRVO
• BRVO and CRVO occur due to atherosclerosis at the AV junction or retrolaminal area due to turbulent blood flow.
• Risk factors are similar: ocular (glaucoma, vasculitis, ONH drusen or crowding) or systemic (DM, HTN, Hyperlipidaemia, smoking) +
hypercoagulable states..
• Tx:
• Oedema anti-VEGF OR IVI steroids.
• Neovascularization: sectoral or scatter PRP
• VH: surgery
• F/U monthly for 6 months. Initially OCT, then at 1 month do FFA and then at 3 months look for NV.
Photocoagulation Intravitreal Anti-VEGF Intravitreal steroid
BRVO BVOS
(photocoagulation is
helpful in NV and
macular oedema)
BRAVO (Anti-VEGF
helpful in macular
oedema)
Score-BRVO (steroids
helpful in macular
oedema)
CRVO CVOS
(photocoagulation is
helpful in NV but NOT
in macular oedema)
Cruise trial (Anti-VEGF
helpful in macular
oedema)
Score-CRVO

hthalmologist
by a specialist
pically within
are following
x; the risk of
er a transient
rillation is of
ation may be
ar risk factors
ry
ication,
nd existing
tremely
hthalmic
,particularly
bsence of a
ease.
Fig. 13.36 Retinal emboli. (A) Hollenhorst plaque; (B) fibrin-
platelet emboli; (C) calcific embolus at the disc
(Courtesy of L Merin – fig. A; S Chen – fig. B; C Barry – fig. C)
B
C
554 Retinal Arterial Occlusive Disease
A B

CROA
• Never miss GCA in CRAO or AION
• CRAO is MAINLY caused by atherosclerosis-related thrombosis occurring at the level of
the lamina cribrosa or large embolus e.g. calcium plaque. However, BRAO is often
caused by embolization (25%).
• Three main varieties of emboli are recognized:
• cholesterol emboli (Hollenhorst plaques) arising in the carotid arteries (Fig 6-18):
Middle sized and obstructs at a major bifurcation.
• platelet-fibrin emboli associated with large- vessel arteriosclerosis or cardiac: The smallest
and obstructs a distal vessel.
• calcific emboli arising from diseased cardiac valves: The largest and lodges at the optic nerve.
• Signs: Retinal oedema, cherry red spot, embolus (if BRAO at bifurcation) and box-carring.
• Tx is managing the underlying cause including GCA. Acute management include ocular
massaging, Diamox, ac paracentesis.
• F/U 1 week and then 1 month (when NV develops).

• Old man with gradual reduction in vision and found to have NVI and
low IOP.

Ocular ischaemic syndrome
• OIS, along with non-ischaemic CRVO, is sometimes termed ‘venous stasis retinopathy’
but it may be prudent to avoid this term.
• Symptoms of gradual reduction of vision along with episodes of amourosis fugax, along
with ocular angina.
• patients may notice unusually persistent after-images, or worsening of vision with
sudden exposure to bright light (‘bright light amaurosis fugax’), with slow adaptation.
• Signs include low IOP, anterior segment ischemia (corneal oedema, ac reaction, iris
atrophy and NVI) and posterior dilated non-turtous veins and narrowed arteries,
equatorial proliferative retinopathy with cotton wool spots +/- macular oedema.
Spontaneous arterial pulsation (due to low diastolic pressure)
• FFA show marked delay in both choroidal and retinal circulation.
• Tx:
• Control risk factors
• Anterior segment: steroids
• NV: PRP
• Oedema: Anti-VEGF
• And endarterectomy if >70% stenosis
is ge
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Color Doppler imaging is an excellent noninvasive means by which to
assess the velocity of blood flow in the retrobulbar circulation. Diminution
of blood flow velocities in the central retinal artery, choroidal vessels, and
ophthalmic artery is typical. Reversal of flow in the ophthalmic artery is
common also. Color Doppler imaging may be used to assess the carotid
arteries simultaneously.
Carotid arteriography discloses generally a 90% or greater obstruction
of the ipsilateral carotid artery in patients who have OIS.5
Approximately
50% of eyes are associated with a 100% ipsilateral carotid artery stenosis,
whereas in 10% there is a bilateral 100% carotid stenosis. If noninvasive
carotid artery evaluation is unremarkable in an eye that shows signs sug-
gestive of ocular ischemia, conventional carotid arteriography or digital
subtraction angiography may be required to demonstrate possible chronic
Fig. 6.23.5 Staining of Retinal Arteries, Ocular Ischemic Syndrome. Prominent
staining of retinal arteries, rather than venules, can help to differentiate an eye that
has ocular ischemic syndrome from an eye that is affected by a nonischemic central
retinal vein occlusion (which shows more prominent staining of the venules).
Clinical signs and fluorescein angiography that help differentiate ocular ischemic syndrome
from nonischemic central retinal vein occlusions or diabetic retinopathy.
TABLE 6.23.1 Features That Distinguish Ocular Ischemic Syndrome
Feature
Ocular Ischemic
Syndrome
Nonischemic Central
Retinal Vein Occlusion
Diabetic
Retinopathy
Laterality 80% unilateral Unilateral Bilateral
Age (years) 50–80 50–80 Variable
Fundus Signs
Veins Dilated, nontortuous Dilated, tortuous Dilated, beaded
Optic disc Normal Swollen Normal
Retinal artery
perfusion pressure
Decreased Normal Normal
Retinal hemorrhages Mild Mild to severe Mild to moderate
Microaneurysms Midperiphery Variable Posterior pole
Hard exudates Absent unless in
association with
diabetes
Rare Common
Fluorescein Angiography
Choroidal filling Delayed, patchy Normal Normal
Arteriovenous transit
time
Prolonged Prolonged Normal
Retinal vessel
staining
Prominent arterial
staining
Prominent venous
staining
Absent (usually)

• Old man with no
known medical
problems. Type A
personality and is a
broker.
Fig. 13.43 Hypertensive retinopathy. (A) Generalized arteriolar attenuation;(B) focal arteriolar attenuation;(C) red-free
photograph showing arteriovenous nipping;(D) ‘copper wiring’;(E) grade 3 retinopathy with macular star;(F) grade 4
A B
C D
E
F

Hypertensive retinopathy
• Can be asymptomatic.
• Stages as above
• Chronic hypertension can have tortuous blood vessels as in
impending CRVO.
• Tx: Treat underlying HTN and risk factors

Hypertensive choroidopathy
• Seen in acute hypertensive elevations.
• Signs:
• Elschnig spots: focal infacts
• Siegrist streaks: necrosis of the vessels with overlying retinal thinning and
loss.
• Exudative retinal detachment

Sickle cell retinopathy
• More common with SC and S-thal because:
• SS causes relative anaemia and thus less viscosity
• SS causes more anoxia rather than hypoxia and thus less anti-vegf
• Retinopathy: SS 3%, S-Thal 14%, SC 33%
• Sickle cell trait have the same complications of hyphema as SS. However, do not develop retinopathy.
• Sickle cell retinopathy can be classified into:
• Non-proliferative: Comma conjunctival sign, salmon patch, sun-burst
• Proliferative:
• Tx:
• Observation for non-proliferative
• PRP, cryo for proliferative
• Vitrectomy for VH and RD (do not buckle otherwise AS ischaemia).
an arborization of the neovascular complex occu
monly are observed in patients who have SC dis
and are rare in the other hemoglobinopathies.18,22
F
demonstrates massive leakage of dye into the vit
seafans represent a progressive proliferative retin
the patient to the risks of vitreous hemorrhage a
Seafans may spontaneously involute, resulting in
fibrovascular tissues that often have residual per
their base. About 40%–50% of seafans may underg
infarction during their course.5
Vitreous hemorrhage is a common complicatio
mation. Patients with limited vitreous hemorrha
whereas those with dense vitreous hemorrhage ha
loss. These hemorrhages may clear spontaneously w
to give ochre-colored vitreous membranes.
Retinal seafans may induce fibrovascular tissu
PSR was classified into five stages by Goldberg18,22
:
Stage I: Peripheral arteriolar occlusions.
Stage II: Arteriolar–venular anastomoses.
Stage III: Neovascular proliferation.
Stage IV: Vitreous hemorrhage.
Stage V: Retinal detachment.
This progression of PSR typically occurs in the third or fourth decade of
life, but the youngest case of PSR was reported in an 8-year-old patient with
SC disease.23
Peripheral retinal arteriolar occlusion can leave large areas of
anterior retinal capillary nonperfusion, which is highlighted well by fluo-
rescein angiography. Curiously, retinal venous occlusion is uncommon in
patients who have SCD. Occluded arterioles initially appear dark red but
subsequently evolve into “silver wire” vessels. Peripheral arteriolar–venular
anastomoses evolve to shunt retinal arterial blood into retinal venules.
These anastomoses can be best seen with fluorescein angiography at the
junction of perfused and nonperfused retina, typically just peripheral to
Fig. 6.24.6 Peripheral Retina in a Sickle SC Patient. Partially regressed peripheral
retinal neovascularization at the junction of the perfused retina (to the right) and
the nonperfused retina (to the far left).
B
Fig. 6.24.7 Fluorescein Angiography. (A) Wide-field fluo
proliferative sickle retinopathy (PSR) demonstrating exten
the vitreous cavity from the sites of neovascularization. (B
fluorescein angiography of PSR demonstrating peripheral
(to the far right), retinal vascular remodeling, arteriovenou
and leakage of fluorescein dye into the vitreous cavity fro
neovascularization. (Courtesy Alok Bansal, MD.)

ROP
• Screening?
• Staging? Location? Extent?
• Types?
• Treatment?
• F/U?
• Complications?

Macroaneurysms
• Develops in old hypertensive ladies
• At arteriovenous crossing from arterial origin.
• Tx:
• Observation
• Laser
• Anti-VEGF
• Surgery if VH

• Child 5 years old.
RetinalVascularDisease
MedicalRetina
Fig. 56.3 Clinical images of various stages of Coats disease, including 3A1, 3A2, and 4. (A) Color fundus photograph of a patient with stage 3A1
Coats disease. Note the temporal extrafoveal exudation. (B) Recirculation-phase ﬂuorescein angiogram of the same patient as in (A). Note the
temporal vascular abnormalities, areas of capillary nonperfusion, and perivascular dye leakage. (C) Color fundus photograph of a patient with
A B
C D
E F

• Coats disease is an idiopathic condition characterized by telangiectatic and
aneurysmal retinal vessels with intraretinal and subretinal lipid exudation and
fluid. Vessels can be sheathed.
• Thought to be same spectrum as norrie and FEVR.
• Leber military aneurysms is a milder form.
• Occurs in young boys. Mostly unilateral (80-90%).
• Xanthocoria.
• Complications: ERM,RD, NVG, cataract
• Tx:
• Need to diffrenciate from retinoblastoma
• Laser is first choice
• Cryo if there is subretinal fluid and laser spots are not effective.
• Surgery

• Young Indian with reduction in vision and
AC reaction.

Eales disease
• Peripheral bilateral idiopathic occlusive perivasculitis (veins affected).
• Occurs in young Indian males.
• Stages: inflammatory, occlusive and then neovascularization
• Recurrent VH from neovascularization. May present with floaters.
• Associated with tuberculin hypersensitivity.
• Tx:
• Anti-tubercular treatment
• Inflammatory: periocular steroids and topical
• Neovascular: PRP, anti-VEGF and surgery (if VH)

Norrie disease
• X-linked recessive
• Bilateral disease with leukocoria
• Spectrum of FEVR and Coats with vasculopathic changes. Usually
presents with a ball of dysplastic retina similar to the pic showed
called ”pseudoglioma”, results in rearly RD, cataract and glaucoma.
• Involves congenitive and sensorineural hearing loss.

Incontinentia pigmenti
• X-linked dominant, therefore lethal to boys
• Signs includes cataract and peripheral vasculopathy with non-
dichotomous branching of retina and avascular retina, resulting in
neovascularization. No exudation normally.
• DDx FEVR, coats.
Aicardi syndrome and Alport (also X-linked dominant).

• Severely ill patient in ICU
that are not associated with trauma (Purtscher retinopathy by definition occ
trauma). Causes include acute pancreatitis, malignant hypertension, collagen
diseases (e.g., systemic lupus erythematosis, scleroderma, dermatomyositis,
syndrome), thrombotic thrombocytopenic purpura (TTP), chronic renal failu
amniotic fluid embolism, retrobulbar anesthesia, orbital steroid injection, al
and long bone fractures.
Figure 3.20.1. Purtscher retinopathy.
• Central retinal vein occlusion: Unilateral, multiple hemorrhages and cotton w

Purtchers retinopathy
• Bilateral disease
• Multiple cotton wool spots and white opacification of the retina +
hmgs peripapillary.
• Though to be due to fat embolization or leukoembolization.
• If no trauma, elevated complement 5a is diagnostic.
• Tx: Manage underlying condition.

AMD
• AMD
• Dry:
• Drusen (small <63um, medium 63-125um, large >125um), basal laminar and basal linear.
• Pigmentation
• Geographical atrophy
• Wet: CNV, fibrovascular PED, RAP and PCV
• Risk factors: Old white smoking lady
• AERDS: for many medium sized drusen, OR at least 1 large OR advanced AMD in the other eye.
• AREDs2: substituted b-caroteine due to risk of lung cancer in smokers. No benefit of omega 3.
• PED:
• Drusenoid: observation only, no tx
• Serous: may progress to CNV, requires monitoring.
• Fibrovascular and hemorrhagic: Forms of CNV. If no drusen may be due to PCV. Therefore treat as CNV.
• CNV:
• Type 1: sub-RPE
• Type 2: sub-retinal
• CNV by FFA “macular photocoagulation study”:
• Classic
• Predominantly classic
• occult
• Recommended daily supplementation based on AREDS2:
○ Vitamin E (400 IU).
○ Vitamin C (500 mg).
○ Lutein (10 mg).
○ Zeaxanthin (2 mg).
○ Zinc (25–80 mg; the lower dose may be equally effective).
○ Copper (2 mg; this may not be required with the lower
zinc dose).
Other considerations
• A liberal green leafy vegetable intake confers a lower risk of
AMD, and for individuals with a strong family history of
AMD and those with early AMD who do not meet the
AREDS criteria, this may be a prudent lifestyle choice.
• Cessation of smoking should be advised.
• Protective measures against exposure to excessive sunlight
should be considered.
• Some authorities consider that evidence still supports the
regular consumption of oily fish.
Non-exudative (dry, non-neovascular)
AMD
Diagnosis
• Symptoms consist of gradual impairment of vision over
months or years. Both eyes are usually affected, but often
asymmetrically. Vision may fluctuate, and is often better in
bright light.
• Signs in approximately chronological order:
○ Numerous intermediate–large soft drusen; may become
confluent.
○ Focal hyper- and/or hypopigmentation of the RPE (Fig.
•
Ma
•
•
•
•
Tx:
• Stop smoking
• Anti-oxidents and good diet
• Amsler grid for dry AMD
• Low vision aids

tion
a
ma
as
ssion
the
alous
s are still visible;diffuse tissue staining is also present;(D) late
oidal vessels and gradual fading of diffuse hyperﬂuorescence
Fig. 14.33 ICGA image showing hyperﬂuorescence due to
polyps and leakage in polypoidal choroidal vasculopathy
(Courtesy of S Chen)

PCV
• Varient of CNV.
• Think about it when hmgs, serous PED and orange lesions in the
absence of drusen (DDx RAP)
• Seen in Africans and Asians ladies
• Both PCV and RAP are bilateral diseases.
• Tx: PDT + Anti-VEGF

ERM
• Fibrocellular membrane
• Better seen with red-free (as the photo shows) + distortion of the
retinal vessels.
• Primary: Post PVD (always look for a break)
• Secondary: Post RD (most common), break, vascular occlusions,
inflammation, trauma.
• Symptoms of metomorphobsia
• FFA sometimes is done to look for previous vascular occlusions.
• Surgery if worse than 6/15 vision and metomorphobsia.

ular Interface Disorders
OCT features
macular hole (IVTS: vitreomacular adhesion
) was a term proposed originally to denote the
nding of oblique foveal vitreoretinal traction
he appearance of clinical changes.
: ‘Impending’ macular hole (IVTS: vitreomacular
n – VMT) appears as flattening of the foveal
ion with an underlying yellow spot. Pathologically,
er retinal layers detach from the underlying
eceptor layer, often with the formation of a
e schisis cavity. The differential diagnosis of a
ellow spot includes adult vitelliform macular
hy, solar and laser pointer retinopathy, and CMO.
b: Occult macular hole (IVTS: vitreomacular
n – VMT) is seen as a yellow ring (Fig. 14.53A).
ss of structural support, the photoreceptor
mmonly undergoes centrifugal displacement
.54B).
Small full-thickness hole (IVTS: small or medium
with VMT) consists of a full-thickness hole less
0 μm in diameter (Fig. 14.53B and see Fig. 14.56B)
arrowest point; the defect may be central, slightly
ic or crescent-shaped. A dehiscence is present in
er retina with persistent vitreofoveolar adhesion
.54C).
Full-size macular hole (IVTS: medium or large
with VMT). A full-thickness hole greater than
in diameter, with a red base in which yellow–
ots may be seen. A surrounding grey cuff of
nal fluid is usually present (Figs 14.53C and
A
OCT finding of oblique foveal vitreoretinal traction
before the appearance of clinical changes.
○ Stage 1a: ‘Impending’ macular hole (IVTS: vitreomacular
traction – VMT) appears as flattening of the foveal
depression with an underlying yellow spot. Pathologically,
the inner retinal layers detach from the underlying
photoreceptor layer, often with the formation of a
cyst-like schisis cavity. The differential diagnosis of a
foveal yellow spot includes adult vitelliform macular
dystrophy, solar and laser pointer retinopathy, and CMO.
○ Stage 1b: Occult macular hole (IVTS: vitreomacular
traction – VMT) is seen as a yellow ring (Fig. 14.53A).
With loss of structural support, the photoreceptor
layer commonly undergoes centrifugal displacement
(Fig. 14.54B).
○ Stage 2: Small full-thickness hole (IVTS: small or medium
FTMH with VMT) consists of a full-thickness hole less
than 400 μm in diameter (Fig. 14.53B and see Fig. 14.56B)
at its narrowest point; the defect may be central, slightly
eccentric or crescent-shaped. A dehiscence is present in
the inner retina with persistent vitreofoveolar adhesion
(Fig. 14.54C).
○ Stage 3: Full-size macular hole (IVTS: medium or large
FTMH with VMT). A full-thickness hole greater than
400 μm in diameter, with a red base in which yellow–
white dots may be seen. A surrounding grey cuff of
subretinal fluid is usually present (Figs 14.53C and
14.54D), and an overlying retinal operculum (sometimes
called a pseudo-operculum) may be visible. Visual acuity
is commonly reduced to 6/60, but is occasionally better,
particularly with eccentric fixation. Opercula (Fig. 14.54E)
consist primarily of glial tissue and condensed vitreous
cortex, though 40% contain photoreceptor elements. By
definition, there is persistent parafoveal attachment of the
vitreous cortex.
○ Stage 4: Full-size macular hole with complete PVD
(IVTS: small, medium or large FTMH without VMT).
The clinical appearance is indistinguishable from stage 3.
The posterior vitreous is completely detached, often
suggested (but not confirmed) by the presence of a Weiss
ring. A significant proportion of idiopathic macular holes
have an associated ERM.
○ Spontaneously resolved macular hole. Macular holes may
heal spontaneously, often resuming a near-normal or
even normal clinical and OCT appearance. A tiny outer
A
B

Macular hole
• Theories: VMT VS vitreoschesis
• Based on biomicroscopic features, Stages?
• Stage 1: schesis cavity with yellow dot (1a) and yellow ring (1b)
• Stage 2: Full thickness hole, Posterior hyaloid still attached to fovea.
• Operculum is seen in stage 3 or 4 (depending on if PVD is complete from the ONH).
• 400um is measured at the narrowest point.
• Tx:
• Observation for holes stage 1
• Chemical vitreolysis (ocriplasmin) for stages 1-2 without ERM
• For holes stages 2-3 (or +/- fresh stage 4): PPV, vitrectomy + (ERM removal post trypan blue staining) + ILM
peeling (brilliant blue staining) and endogas tamponade.
• NOTEs:
• partial thickness macular hole is normally a sequalae of CME, however can be due to anomalous PVD.
• Vitreomacular adhesion: Broad >1500um, focal <1500 when foveal contour is not distorted (see pic)

CSR
• Risk factors: Stress, steroids, cushings
• FFA: Ink blot (expansile dot) 85%, smokestack 10%
• For previous CSR, do FAF, it will show.
• Must exclude optic disc pit.
• Tx:
• Observation. Eighty percent to 90% of the cases of central serous retinopathy (CSR)
will spontaneously resolve within 3-6. months, but 40% to50% will recur
• PDT
• Laser for non-foveal lesions, or micropulse laser to target the leaking points.
• ? Anti-VEGF
• Mineralocorticoid receptor antagonists (e.g., eplerenone)

628 Idiopathic Macular Telangiectasia
A
B
C
D

Idiopathic Macular (juxtafoveal) telengectasia
• Two types:
1. Aneurysmal telengectasia (type 1): Unilateral
disease. A spectrum of coats disease, known
previously as leber miliary aneurysms. Presents
as lipid exudate and aneurysms.
2. Perifoveal telengectasia (type 2, pic): Bilateral
disease. More common than type 1. Temporal
to fovea, telengectasia and crystalline deposits.
Right angles venules which can give rise to
CNV. OCT shows cysts.
3. Type III occlusive telengectasia.
• Tx:
• Laser to aneurysms if small no. but can be
difficult due to close proximity to fovea
• Anti-VEGF if CNV

Angioid streaks
• Associations PEPSI in 50% of cases only. 50% no identifiable cause.
• Calcified bruchs membrane. In Sickle cell and thalassemia it is the iron which makes the
bruchs membrane brittle.
• PXE: plucked chicken appearance in neck and axilla. Pau d’orange appearance of the
temporal macula (pitted orange).
• ONH drusen seen in 25%.
• NEVER do scleral depression (otherwise choroidal rupture).
• Complications:
• Foveal involvement by streak
• CNV
• Choroidal rupture.
• Treatment:
• Avoid contact sports and wear protective glasses
• Anti-VEGF for CNV

Choroidal folds
• HORIZONTAL parallel lines
• Seen in tumors, hypotony (<6mmhg) and some rare cases of
hypermetropia.
• Hypotonic maculopathy seen in post filtring surgery, chronic uveitis,
cyclodialysis cleft, RD

Risk for choroidal melanoma
• To Find Small Ocular Melanoma Using Helpful Hints Daily
This stands for thickness greater than 2 mm, subretinal fluid, symptoms,
orange pigment present, margin within 3 mm of the optic
disc, ultrasonographic hollowness (versus solid/flat), absence
of halo and absence of drusen.6 (A halo refers to a pigmented choroidal
nevus surrounded by a circular band of depigmentation.)
• COMS trial:

Photic toxicity
• Photochemical injury (sub-coagulation injury)
• Foveal cyst with photoreceptor-RPE damage. Sometimes manifest as
hyperreflective leasion as in pic (welding arc)
• Good prognosis.
• Laser pointers should be class 3A or less
vitreo
as init
with r
intrao
which is replaced over time by RPE irregularity or lamellar hole. No eﬀec-
tive therapy exists, and vision usually improves with time, with permanent
vision eﬀects being rare.
Phototoxicity has also been described following brief incidental light
BA
Fig. 6.10.2 Optical Coherence Tomography of Welding Arc Retinopathy That Occurred Afte
BA
Fig. 6.10.3 Acute Retinal Phototoxicity, 2 Weeks After Cataract Surgery. (A) Perifoveal fluore
and retinal pigment mottling in the late phase. Visual acuity is 20/60. (Courtesy Gordon A. Byrne

Retinal dystrophies
• Degeneration: Acquired tissue changes characterised by deposition
of acelluar material and loss of tissue. They are often unilateral or
bilateral asymmetrical, peripherally located and may be associated
with post-inflammatory states.
• Dystrophies: Inherited tissue changes characterised by being
bilateral, symmetrical, slowly progressive, and not associated by post-
inflammatory states.

• 20 year old with high myopia
and large retinal breaks

Stickler syndrome
• Arthro-ophthalmopathy
• Most common inherited cause of rhegmatogenous RD.
• AD disease COL2A1 mutations
• In Stickler’s syndrome, defects in specific genes have been associated with particular phenotypes, thus enabling the classification
of patients with Stickler’s syndrome into four subgroups (only type III does not have eye involvement; type 4 is only eyes).
• Patients in the subgroups with vitreous abnormalities are found to have defects in the genes coding for type II procollagen and
type V/XI procollagen.
• Signs:
• high myopia (> -8)
• Optically empty vitreous with free strands
• Large retinal tears and RD
• At risk of cataract and glaucoma as well
• Systemic: submucosal cleft palate (can be examined with gloved finger), bifid uvela, joint problems, mitral valve prolapse and sensorineural
hearing loss.
• Tx:
• Vitrectomy better than buckle
• Prophylactic 360* retinopexy

• 6 year old child with reduced
vision

X-linked retinoschesis
• RS1 gene mutation
• Affects foveal RNFL
• DDx of non-leaky CME:
• X-linked retinoschisis
• Goldman fevr
• RP associated CME
• Nicotinic acid
• The average visual acuity is 20/60 (6/18) at age 20 years and 20/200 (6/60) at age 60 years.
• In contrast to the histological studies showing anterior layer splitting, spectral domain optical
coherence tomography (OCT) shows widespread cystic spaces in both inner and outer macular
retina.
• Tx:
• Gene therapy
• RD surgery

RD in children think about:
• ROP
• FEVR
• Stickler
• Tumors

ROP FEVR
History
Pre-maturity, low birth weight and NICU admission Family history of retinopathy (AD), genes include
NR2E3 and LRP5, EVR
Examination
ROP stages, no exudates, no hmgs Straightening of vessels, fibrovascular stalk similar to
PHPV, brush border vessels at the periphery, exudates,
hmgs

• Patient with night
blindness

Enhanced S-cone syndrome
• Type of RP with prominent retinal features
• NR2E3 gene mutation (similar to also Goldman Fevr)
• Signs:
• Ring pigmentation around macula
• +/- foveoschesis
• ERG shows depressed rod function, L and M cones (photopic similar
to scotopic). However enhanced S-cone function

Choroideremia
• X-linked recessive
• CHM gene
• Leads to night blindness due to loss of PR, RPE and CC
• Macular sparing especially in female carriers
• Late disease also affects the large choroidal vessels and sclera is seen
posteriorly
• Both EOG and ERG are affected.
• DDx albinism: In albinism there is nystagmus, hair albinimis, iris
tranillumination defects and foveal hypoplasia + normal ERG.
• Tx:
• Gene therapy
• Treat early cataract

Gyrate atrophy
• Discrete areas of chorioretinal atrophy, scalloped edges
• AR, ornithine aminotransferase enzyme deficiency, and increase
ornithine in urine.
• Symptoms of nyctalopia during second or third decade of life
• Tx:
• ornithine aminotransferase enzyme is dependent on Vit-B6 therefore needs
supplementation
• Avoid argenine (because the end product is ornithine as well)

• 20 year old with reduced VA

Stargardt disease (fundus flavimaculatus)
• Most common macular dystrophy
• Presents at 2-3 decades of life
• AR, ABCA4 gene
• Signs:
• Pisciform flecks in macula (lipofusin), sparing ONH (best seen in autoflouresecne)
• Bull’s eye maculopathy
• If flecks all over the retina called “fundus flavimaculatus”
• FFA shows hyperflouresecnt spots (window defects at sight of flecks due to
degenerated RPE) and Silent choroid
• Tx:
• Patient normally have fair vision
• Gene therapy

• 30 year old with retinal lesion

Best’s disease
• AD, VMD gene mutation, leads to accumulation of lipofusin.
• Juvenile and adult forms ( most common type of pattern dystrophy, RDS mutation, presents 4-6th
decade of life, lesions much smaller and EOG is normal).
• Leads to central mild reduction in vision only except in late stages
• Stages:
• Pre-vitteliform
• Vitteliform
• Psuedohypopon
• Scrambled egg
• Atrophic
• CNVM
• Arden ratio < 1.6, Normal ERG
• Tx:
• Treat complications including cataract and CNV
• Gene therapy

• 40 year old with retinal
pathology

Malattia leventinase
• Familial drusen
• AD
• All are due to a single mutation from an arginine to a tryptophan at
amino acid 345 in the EFEMP1 gene.
• Signs: Drusen extends outside macula, leading to RPE atrophy and
CNV
• Tx:
• Treat CNV

Typical retinitis pigmentosa
• Rod-cone dystrophy
• Can be AD, AR or X-linked. X-linked is rare but most severe.
• Nyctalopia, later with photophobia
• Signs:
• Waxy pale disc
• Bone specules
• Attenuated vessels
• Others: CME, drusens, myopia
• Tx:
• Treat complications
• Low vision aids

• Child with searching eye movements
and oculodigital reflex.

Leber congenital amaurosis
• Atypical RP, early onset
• AR
• Associated with cataract, strabismus and
hypermetropia
• Extinguished ERG
• Other atypical RP:
• Sector retinitis pigmentosa
• Retinitis punctate albescens
• Sectoral RP
• Sine pigmento

• Obese child with reduced
vision.

Pigmentary retinopathies
• Systemic diseases associated with retinal pigmentation.
• Associated with obesity, diabetes: Bardet beidel (can have bulls eye
maculopathy)
• Associated with deafness: Usher, Refsum
• Associated with ophthalmoplegia: Kearn Sayer, CPEO
• ================
Three treatable inherited chorioretinal dystrophies:
1) Gyrate atrophy: Limit arginine and give B6
2) Refsum: Limit phytanic acid
3) Bassen–Kornzweig syndrome or abetalipoproteinaemia: Vitamin
supplementation.

• 20 year old with colour blindness and reduced vision + photophobia

Achromatopsia
• Autosomal recessive.
• photoreceptor disorders with subsequent loss of the whole cone. It is defined as an absence
of color discrimination, or achromatopsia, means that any spectral color can be matched
with any other solely by intensity adjustments. Patients present with nystagmus. See the
world as grey. Reduced visual acuity, hameralopia (day blindness) and nystagmus. CNGB and
CNGA genes.
• There are two forms
• Complete (also called rod monochromatism): autosomal recessive
• Incomplete (also called S-cone monochromatism): x-linked. S-cone function and rods are preserved.
• Signs/investigations:
• Foveal granularity
• OCT shows optically empty cavity in the photoreceptor zones.
• Abnormal multifocal ERG
• Tx:
• Gene therapy
• Tinted glasses

• 20 year old nyctalopia. The fundus
changes colour (to normal) after dark
adaptation.

CSNB
• DDx of pathological nystagmus in childhood
• CSNB
• Achromatopsia
• Albinism
• X-linked retinoschisis
• LCA
• Can be autosomal recessive or dominant (rhodopsin
gene) or x-linked. X linked is most common and most
severe.
• Electronegative ERG
• Normal fundus apart from: Oguchi disease (pics showing
Mizuo Nikamura phenomenon; Mutations in RHOK
[rhodopsin kinase] and SAG [arrestin]), fundus
albipunctatus (Mutations in retinol dehydrogenase
;RDH5) and Enhanced S-cone syndrome.

• 20 year old with nystagmus since
birth

Albinism
• In the embryo, the activation of melanogenesis by tyrosinases is concomitant with the formation
of the optic cup. Melanin production seems to play a role in the development of the retina.
• People with oculocutaneous albinism can be tyrosinase +ve or –ve (beware of Hermansky–Pudlak
syndrome [platelet dysfunction] and Chediak–Higashi syndrome [white blood cell lysosomal
dysfunction] in tyrosinase +ve) and are autosomal recessive (OCT1A –ve, OCT1B and OCTB are
tryosinase +ve).
• This syndrome is different from ocular albinism alone or cutaneous albinism alone. Ocular
albinism is x-linked with reduced melanosomes. Oculocutaenous albinism is autosomal
recessive with reduced melanin.
• Chiasm: about 90% crossed fibres (normally only 53%) resulting in loss of stereopsis and strabismus, this can be
demonstrated in VEP.
• Regardless of this, ocular manifestations transform to either (can occur in all):
• VA affected with foveal hypoplasia and pendular nystagmus (true albinism)
• VA and fovea not affected (called albinoidism).
• Investigations:
• CBC, hair bulb

406 Uveitis in Spondyloarthropathies
arthritis. Around 75% of patients are positive for HLA-B27. A
range of infective agents can trigger the syndrome, which develops
in 1–3% of men after non-specific urethritis, and around 4% of
individuals after enteric infections caused by a range of organisms
including Shigella, Salmonella and Campylobacter. Chlamydia
pneumoniae respiratory infection and others may also precede
ReA.
Systemic features
• Presentation is with the acute onset of malaise, with fever
and dysuria 1–4 weeks after a linked infection in a patient
aged between 20 and 40, with arthritis that may be preceded
by conjunctivitis. A variety of other features may be present,
though not always the defining triad.
• Peripheral oligoarthritis is acute, asymmetrical and
migratory; 2–4 joints tend to be involved, most commonly
the knees, ankles and toes.
• Spondyloarthritis affects about 50% of patients, manifesting
with low back pain. This sometimes becomes chronic.
• Enthesitis manifests with plantar fasciitis, Achilles
tenosynovitis, bursitis and calcaneal periostitis; reactive bone
formation in the latter may result in a calcaneal spur.
• Mucocutaneous lesions include painless mouth ulceration,
circinate balanitis and keratoderma blennorrhagica – skin
lesions resembling psoriasis – involving the palms and soles
(Fig. 11.11).
• Genitourinary involvement includes cervicitis, prostatitis
and epididymitis.
• Aortitis occurs in 1–2%.
Ocular features
The eye is involved in 50% of cases with a urogenital inciting
infection and 75% of enteric ReA syndrome.
• Conjunctivitis is very common; it classically follows
urethritis but precedes arthritis. The inflammation is usually
Fig. 11.10 Ankylosing spondylitis. (A) Fixed flexion deformity
of the spine; (B) sclerosis and bony obliteration of the
sacroiliac joints
(Courtesy of MA Mir, from Atlas of Clinical Diagnosis, Saunders 2003 – fig. A)
A
B

HLA-B27 related AAU
• Ankylosing spondalitis is the most common. 25% of patients are affected.
• Occurs in young males.
• Bilateral disease but eyes are affected at different times, not simultaneous.
• Leads to bamboo spine and sacroiliac joint obliteration.
• AAU occurs in 20% of reactive arthritis (shigella, cambylobacter diarrohea)
and 7% of psoriatic arthritis patients and 5% of inflammatory bowl disease
(mostly ulcerative colitis).
• Reactive arthritis occur in male 90%
• Spondylarthropathies also leads to scleritis and episcleritis.

Fuch heterochromic iridocyclitis
• Thought to be due to breakdown in blood aqueous barrier rather
than inflammation. Inflammation does not respond to steroids.
• Rubella virus is implicated.
• Bilateral disease associated with glaucoma, cataract (complicated by
amsler sign and weak zonules and dilatation).
• KP: diffuse stellate
• Moth eaten iris leading to heterochromia.
• Glaucoma treated with valve filtration surgery

• Child 2 years
pre
gen
Sys
mo
exa
rev
Mi
pat
Inf
the
the
for
clo
Sel
pat
the
uve
sho
exa
dif
scr
• Differ
Partic
Idi
pat
un
lasts for several years and responds poorly to treatment.
The presence of complications at initial examination
appears to be an important risk factor for the
development of subsequent complications, regardless
of therapy.
Fig. 11.14 Band keratopathy, posterior synechiae and mature
cataract in chronic anterior uveitis associated with juvenile
idiopathic arthritis

JIA
• Mean is 6 years, Girls, ANA +ve, RF –ve, pauciarticular (<4 joints,
migratory).
• 20-30% of above will develop uveitis.
• Typically white eye (no injection despite severe inflammation). Therefore,
cases are missed, hence need for screening despite absence of symptoms.
• Flare > cells
• Bilateral disease which waxes and wanes.
• Complications include: Band keratopathy, posterior synechiae, glaucoma,
cataract, cyclitic membrane, CME.
• Tx: steroids (topical, periocular and systemic) + cycloplegia +
immunomodulators including methotrexate, infliximab and adalimumab.

Intermediate uveitis
• Called pars planitis if idiopathic
• 85-90% idiopathic
• Onset is with blurred vision and floaters. Eye is typically white.
• Sarcoidosis will have more AC reaction than MS. MS has periphlebitis
more.
• Glaucoma, cataract and CME is common
• Tx:
• Underlying cause
• Pars planitis: periocular steroids, intravitreal steroids, immunomodulators, par
plana photocoagulation (cryo is associated with RD) and vitrectomy.

auditory manifestations tends to resolve but skin, lash a
hair changes usually persist.
Investigation
Systemic manifestations should be investigated and manag
an appropriate specialist.
• Lumbar puncture if diagnosis uncertain; CSF shows a
transient lymphocytic pleocytosis, and melanin-contain
macrophages.
• FAF demonstrates areas of serous detachment (Fig. 11.21
• OCT (Fig. 11.21B) allows quantification of subretinal flu
• Ultrasonography shows diffuse choroidal thickening an
excludes posterior scleritis; UBM can be used to demon
ciliary effusions.
• FA of the acute phase shows multifocal hyperfluorescen
dots at the level of the retinal pigment epithelium (RPE
Fig. 11.21C) followed by subretinal pooling (Fig. 11.21D).
chronic phase shows RPE window defects.
• ICGA during the acute phase of the disease shows regul
distributed hypofluorescent spots, most of which remai
hypofluorescent during the late phase, when diffuse
hyperfluorescence over the posterior pole is also shown
fundus appearance (‘sunset glow’ fundus – Fig. 11.20) and
depigmented limbal lesions (Sugiura sign) in pigmented,
especially Japanese, patients.
• Chronic recurrent phase is characterized by smouldering
anterior uveitis with exacerbations. Recurrent posterior
uveitis is much less common.
• Diagnostic criteria for VKH are set out in Table 11.8; in
complete VKH, criteria 1–5 must be present, in incomplete
VKH, criteria 1–3 and either 4 or 5 must be present, and in
probable VKH (isolated ocular disease), criteria 1–3 must be
present.
• Ocular complications include choroidal neovascularization,
subretinal fibrosis, preretinal and disc new vessels and
vitreous haemorrhage, cataract and glaucoma.
• Prognosis is very variable, and is partly dependent on
aggressive control in the early stages. Neurological and
Fig. 11.19 Vitiligo and poliosis in Vogt–Koyanagi–Harada
1. Absence of a history of penetrating ocular trauma
2. Absence of other ocular disease entities
3. Bilateral uveitis
4. Neurological and auditory manifestations
5. Integumentary findings, not preceding onset of cen
nervous system or ocular disease, such as alopec
poliosis and vitiligo

VKH
• Classification:
• Complete: All 5 criteria
• Incomplete: 1-3 + 4 OR 5
• Probable: 1-3 only (Harada)
• Must have papillitis
• 4 phases:
• Prodromal
• Acute uveitis: Multiple serous RD
• Convalescent: sun-set fundus, alopecia, vitiligo and poliosis.
• Chronic recurrent: anterior uveitis mostly
• Investigations: LP (pleocytosis, melanin containing macrophages), OCT, FFA
• Treatment: prednisolone 3 days 1g/day, then 1mg/day tapering. May need
immunomodulators.
416 Sympathetic Ophthalmitis
auditory manifestations tends to resolve but skin, lash and
hair changes usually persist.
Investigation
Systemic manifestations should be investigated and managed by
an appropriate specialist.
• Lumbar puncture if diagnosis uncertain; CSF shows a
transient lymphocytic pleocytosis, and melanin-containing
macrophages.
• FAF demonstrates areas of serous detachment (Fig. 11.21A).
• OCT (Fig. 11.21B) allows quantification of subretinal fluid.
• Ultrasonography shows diffuse choroidal thickening and
excludes posterior scleritis; UBM can be used to demonstrate
ciliary effusions.
• FA of the acute phase shows multifocal hyperfluorescent
• Convalescent phase follows several weeks later: localized
alopecia, poliosis and vitiligo (Fig. 11.19); depigmented
fundus appearance (‘sunset glow’ fundus – Fig. 11.20) and
depigmented limbal lesions (Sugiura sign) in pigmented,
especially Japanese, patients.
• Chronic recurrent phase is characterized by smouldering
anterior uveitis with exacerbations. Recurrent posterior
uveitis is much less common.
• Diagnostic criteria for VKH are set out in Table 11.8; in
complete VKH, criteria 1–5 must be present, in incomplete
VKH, criteria 1–3 and either 4 or 5 must be present, and in
probable VKH (isolated ocular disease), criteria 1–3 must be
present.
• Ocular complications include choroidal neovascularization,
subretinal fibrosis, preretinal and disc new vessels and
vitreous haemorrhage, cataract and glaucoma.
• Prognosis is very variable, and is partly dependent on
aggressive control in the early stages. Neurological and
Table 11.8 Modified diagnostic criteria for Vogt–
Koyanagi–Harada syndrome
1. Absence of a history of penetrating ocular trauma
2. Absence of other ocular disease entities
3. Bilateral uveitis
4. Neurological and auditory manifestations
5. Integumentary findings, not preceding onset of central
nervous system or ocular disease, such as alopecia,
poliosis and vitiligo

Sarcoidosis
• Occurs in middle aged African female origin.
• Ocular involvement in 70% of cases
• Bilateral disease mostly granulomatous anterior uveitis.
• Conjunctival follicles and Posteriorly, choroidal granulomas, periphlebitis (non-occlusive,
arteries not involved), candlewax drippings, snowballs and banks. Lacrimal gland
infiltration.
• Systemic includes erythema nodosa, arthlagia.
• Investigations:
• Negative tuberculin skin test
• Diagnosis may require bronchial biopsy. CT chest is more diagnostic.
• LFT, Ca, ACE, lysozyme, urinelysis (calcinuria).
• Tx:
• Steroids
• Immunomodulators.

Behcet disease
• Young middle eastern men
• Ocular involvement in 70% of cases
• Bilateral posterior uveitis characterized with occlusive vasculitis (both arteries and veins) and
necrotizing retinitis.
• Anterior uveitis is typically white eyed with mobile hypopyon.
• Latest criteria in 2013 allocates points. Skin lesions include pseudofolliculitis, erythema nodosum,
dermatographia).
• HLA-B51 is of little diagnostic value.
• Tx:
• Steroids + azathioprine/cyclosporin. Immunomodulation is important to start to prevent CNS
vascultitis/aneurysms and coronary arteritis.

Toxoplasmosis
• Cats are the definitive hosts. Humans are the intermediate hosts.
• Oocysts and ingested by cats. It divides and shed in faces where it becomes sporozoyte (a motile form), if
ingested by humans transform into tachyzoites (infective form) and if fought by the immune system (becomes
dormant) transform into bradyzoyte (tissue cyst). In uncooked meat, the bradyzoyte can become tachyzoyte in
the human.
• Infection of humans can be congenital (tachyzoyte phase and is more likely to occur during the third trimester
where blood is more likely to mix) or acquired.
• Majority of cases are congenital: With retinochoroiditis, intracranial calcification and hydrocephalus.
• Retinochoroiditis often occur in the macula in the congenital form. Rare manifestion is outer macular
punctate lesions.
• Diagnosis is clinical often
• Investigations:
• Serology: IgM can stay for up to 1 year on new acquired infection
• Goldman witmer coefficient (around 75% sensitive): Ratio of IgG of aqueous to serum (denotes local production of IgG).
• Tx:
• Only if involving macula, ONH, major arteries, bilateral in immuncompromised, or severe
• Triple therapy: Pyramethamine, sulfadiazine and prednisolone. Folinic acid (5mg alternate days) to reduce leukopenia and
thrombocytopenia from pyrimethamine. Pyramethamine is avoided in AIDS patients
• Alternative is clindamycin OR co-trimoxazole.
• Spiromycin in pregnancy

Toxocarisis
• Toxocara Canis: Nematehelminth. Cause retinal lesions similar to retinoblastoma. Lives in
dogs or cats. Can also cause endophthalmitis.
• Toxocara canis and Toxocara cati
• Causes two distinct disease, visceral larva migrans (visceral toxocariasis) and ocular toxocariasis
• Infects children mostly < 4 years old.
• Humans get infested as they eat the oocyst or the larvae form normally from soil.
• The eosinophilic granuloma formed comprise the larva enveloped by a central core of eosinophils
surrounded by mononuclear cells, histiocytes, epithelioid cells and giant cells
• Presents with white eye with leukocoria, strabismus with anterior and posterior chamber
reactions.
• Oesinophilia may be found systematic or aqueous.
• Can cause granuloma (most common), endophthalmitis or atypical lesions.
• Peripheral granulomas have a fibrocellular band extending to the optic nerve or macula.
• Endophthalmitis presents with white eye
• Treatment is topical, periocular and systemic steroids during active inflammation. Visceral larva
migrans is treated with albendazole, however, may not kill the intraocular larvae (and can
exacerbate the ocular inflammation). Laser photocoagulation may be used for peripheral lesions.

Onchocerciasis
• Nematyhelminth.
• River night blindness. Transmitted by black flies
• Sclerosing keratitis, anterior uveitis with microfilaria seen in A/C,
Chrioretinitis and papillitis causing optic nerve atrophy.
• Treatment with ivermectin (or newer mexidectin): may need yearly
injections also.

Cysticercosis
• Caused by platyhelminth (tape worm= Tenia solium).
• Can cause cysts in the A/C or retina. Scolex can be seen in the cyst.
• Treatment is surgical removal of the cysts (AC wash or vitrectomy).
subretinal cysticerci can be removed by the transvitreous or trans-
scleral route. Anti-helminths can exacerbate the inflammation.

Diffuse unilateral subacute
neuroretinitis (DUSN)
• DUSN is a clinical syndrome due to the presence of a single motile
subretinal nematode such as Toxocara canis, Baylisascaris procyonis
and Ancylostoma caninum
• Mis-diagnosed as multifocal choroiditis.
• Acute disease. Crops of grey–white outer retinal lesions, vitritis,
papillitis and retinal vasculitis.End-stage disease. Optic atrophy,
retinal vascular attenuation and diffuse RPE degeneration
• Tx:
• Laser photocoagulation if larvae seen.
• Systemic albendazole and prednisolone.

• Young man with no morbidity.

HIV microangiopathy
• Most common feature.
• Cotton wool spots disappear.
• AIDS develops in about half of HIV-infected patients within 10 years and is
defined as HIV infection with either a CD4+ T count <200 cells/μl or the
development of one or more
• AIDS-defining conditions
• Particular opportunistic infections such as respiratory or oesophageal candidiasis
• Pneumocystis jirovecii pneumonia,
• Cryptosporidiosis
• cytomegalovirus retinitis
• Specific tumours including Kaposi sarcoma (Fig. 11.49A) and certain lymphomas
• progressive multifocal leukoencephalopathy.

CMV retinitis
• 50% bilateral, especially if unilateral not treated.
• AIDs defining condition. CD4 <50
• Three clinical pictures:
• Fulminant
• Indolent (Granular)
• Frosted branch angiitis
• Tx:
• Valganciclovir 900mg bid then od (to prevent other eye involvement), oral
• If very fulminant disease, intravitreal ganciclovir twice weekly.
• HAART to be initiated 1-2 weeks post therapy due to immune recovery uveitis
• Ganciclovir cause bone marrow suppression, cidofovir and foscarnet are nephrotoxic.
• Slow release ganciclovir implant is available.
• F/U:
• Ganciclovir is virostatic, therefore, relapse may happen when decreased immunity.
• Tx may be stopped when CD4 > 100 for at least 6 weeks.
• During F/U look for retinal breaks, will need barricade.

• AIDs patient with bilateral reduction
in vision

PORN
• Occurs in immunocompromised
• Varicella
• Affects macula first, may look like cherry red spot
• Diagnosis using PCR of aqueous or vitreous sample
• Tx:
• Similar to CMV
• Although varicella zoster is susceptible to valaciclovir, valganciclovir is given as
it covers all HSV, varicella and CMV.

ARN
• Panuveitis syndrome with peripheral well demarcated retinal
necrosis, arteriolitis (less likely phlebitis). Very painful.
• Herpes simplex virus 1 in young, Varicella in older.
• Tx:
• Intravitreal ganciclovir twice weekly
• Systemic valcyclovir 1g tid (preferred and more compliance) or acyclovir
800mg 5/day for 3-4 months. Oral as good as IV as long as IVI are given.
• Cycloplegia, topical prednisolone. Oral prednisolone can be started after 24-
48 hours.
• Later necrotic retina can be barricaded.

• Red, painful eye with ciliary
congestion

HSV uveitis
• Patchy iris thinning in HSV, sectoral in VZ.
• Treat with predforte, cycloplegia and oral acyclovir 400mg 5/day or
valacyclovir 500mg tid.

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