Systemic Diseases and the Eye Various systemic diseases affect the eye and it's functioning. Here we present those common systemic diseases that are responsible to cause effects in the eye. What are the diseases that affect eye? Eye is affected by the following diseases: Systemic Hypertension (Increased blood pressure of the body) Diabetes Melitus (Increased blood sugar level) Systemic Lupus Erythromatosus (SLE) AIDS and other Venereal Diseases like Syphilis Sickle Cell Anemia, Eales Disease and many more. Look at the slides.

1. Systemic Diseases andSystemic Diseases and
the eyethe eye
Rabindra AdhikaryRabindra Adhikary
ravinems@iom.edu.npravinems@iom.edu.np

2. Common systemic diseasesCommon systemic diseases
affecting the eyeaffecting the eye
InfectiousInfectious
ToxoplasmosisToxoplasmosis
ToxocariasisToxocariasis
TBTB
SyphilisSyphilis
LeprosyLeprosy
HIVHIV
CMVCMV
Non-infectiousNon-infectious
Endocrine – diabetes,Endocrine – diabetes,
thyroidthyroid
Connective tissueConnective tissue
disease – RA/ SLE/disease – RA/ SLE/
Wegeners/ PAN/Wegeners/ PAN/
Systemic sclerosisSystemic sclerosis
Vasculitides (GCA)Vasculitides (GCA)
SarcoidosisSarcoidosis
Behcet’s DiseaseBehcet’s Disease
Vogt Koyanagi HaradaVogt Koyanagi Harada
syndromesyndrome
PhakomatosesPhakomatoses

3. 1. Soft tissue involvement
• Periorbital and lid swelling
• Conjunctival hyperaemia
• Chemosis
• Superior limbic keratoconjunctivitis
2. Eyelid retraction
3. Proptosis
4. Optic neuropathy
5. Restrictive myopathy
THYROID EYE DISEASE

4. Soft tissue involvement
Periorbital and lid swelling
Chemosis
Conjunctival hyperaemia
Superior limbic
keratoconjunctivitis

5. Signs of eyelid retraction
Occurs in about 50%
• Bilateral lid retraction
• No associated proptosis
• Bilateral lid retraction
• Bilateral proptosis
• Lid lag in downgaze• Unilateral lid retraction
• Unilateral proptosis

6. Proptosis
Treatment options
• Systemic steroids
• Radiotherapy
• Surgical decompression
• Occurs in about 50%
• Uninfluenced by treatment of hyperthyroidism
Axial and permanent in about 70% May be associated with choroidal folds

7. Optic neuropathy
• Occurs in about 5%
• Early defective colour vision
• Usually normal disc appearance
Caused by optic nerve
compression at
orbital apex by enlarged recti
Often occurs in absence of significant
proptosis

8. • Occurs in about 40%
• Due to fibrotic contracture
Restrictive myopathy
Elevation defect - most common Abduction defect - less common
Depression defect - uncommon Adduction defect - rare

9. SARCOIDOSISSARCOIDOSIS
Idiopathic multisystem disorderIdiopathic multisystem disorder
Characterised by non-caseatingCharacterised by non-caseating
granulomatagranulomata
More common in women 20-50 yrsMore common in women 20-50 yrs
More common in blacks and AsiansMore common in blacks and Asians
? Related to mycobacteria? Related to mycobacteria

10. SARCOIDOSISSARCOIDOSIS
Systemic InvolvementSystemic Involvement
Lung lesions – 95%Lung lesions – 95%
Thoracic lymph nodesThoracic lymph nodes
– 50%– 50%
Skin lesions – 30%Skin lesions – 30% →→
Eyes – 30%Eyes – 30%

11. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
Anterior segmentAnterior segment
lesions (30%)lesions (30%)

Conjunctival granulomaConjunctival granuloma

Lacrimal glandLacrimal gland
involvement/dry eyeinvolvement/dry eye

Acute or chronic uveitisAcute or chronic uveitis
→→

KPs described asKPs described as
‘mutton fat’ because they‘mutton fat’ because they
are large and greasyare large and greasy

12. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
Posterior segmentPosterior segment
lesions (20%)lesions (20%)

Patchy venous sheathingPatchy venous sheathing

Cellular infiltrate aroundCellular infiltrate around
vesselsvessels

ChorioretinalChorioretinal
granulonmasgranulonmas

Vasculitis includingVasculitis including
occlusive causing:-occlusive causing:-

NeovascularisationNeovascularisation

Infiltrate in vitreousInfiltrate in vitreous
(vitritis) including cell(vitritis) including cell
clumps (snowballs)clumps (snowballs)

13. SARCOIDOSISSARCOIDOSIS
Ocular InvolvementOcular Involvement
Sheathing of theSheathing of the
retinal veinsretinal veins
FluoresceinFluorescein
angiography showingangiography showing
leakage and stainingleakage and staining
at sites of sheathingat sites of sheathing

14. SARCOIDOSISSARCOIDOSIS
Granuloma in FundusGranuloma in Fundus
Retinal and pre-retinalRetinal and pre-retinal
ChoroidalChoroidal

15. SARCOIDOSISSARCOIDOSIS
Granuloma in FundusGranuloma in Fundus
Optic nerve headOptic nerve head
granulomagranuloma
Normal optic nerveNormal optic nerve
headhead

16. SARCOIDOSISSARCOIDOSIS
Systemic SignsSystemic Signs
Lupus pernio affectingLupus pernio affecting
the nose – a chronicthe nose – a chronic
progressiveprogressive
cutaneous sarcoidcutaneous sarcoid
that most commonlythat most commonly
affects face and earsaffects face and ears

17. SARCOIDOSISSARCOIDOSIS
Systemic signsSystemic signs
Facial palsyFacial palsy
Salivary glandSalivary gland
enlargementenlargement

18. SARCOIDOSISSARCOIDOSIS
Systemic signsSystemic signs
Hilar adenopathy onHilar adenopathy on
chest x-raychest x-ray
Lung infiltrateLung infiltrate
Erythema nodosumErythema nodosum
ArthritisArthritis

19. SARCOIDOSISSARCOIDOSIS
Investigations (1)Investigations (1)
CXR – to detectCXR – to detect
pulmonary signspulmonary signs
Bilateral hilar lymph-Bilateral hilar lymph-
adenopathyadenopathy
Pulmonary mottlingPulmonary mottling

20. SARCOIDOSISSARCOIDOSIS
Investigations (2)Investigations (2)
Serum angiotensin-converting enzymeSerum angiotensin-converting enzyme
(ACE) – elevated in active sarcoidosis(ACE) – elevated in active sarcoidosis
Mantoux test – caution in patients whoMantoux test – caution in patients who
have had BCG vaccination. Test may behave had BCG vaccination. Test may be
negativenegative
Lung function testsLung function tests

21. SARCOIDOSISSARCOIDOSIS
Investigations (3)Investigations (3)
Gallium scan showingGallium scan showing
increased uptake inincreased uptake in
the lacrimal andthe lacrimal and
parotid glands andparotid glands and
pulmonary regions inpulmonary regions in
a patient with activea patient with active
sarcoidosissarcoidosis

22. SARCOIDOSISSARCOIDOSIS
TreatmentTreatment
Systemic steroids may be necessary inSystemic steroids may be necessary in
patients with posterior segment diseasepatients with posterior segment disease
where vision is threatened, especially ifwhere vision is threatened, especially if
optic nerve is involvedoptic nerve is involved

23. PHACOMATOSES
1. Neurofibromatosis
2. Tuberous sclerosis (Bourneville disease)
3. von-Hippel-Lindau syndrome
4. Sturge-Weber syndrome
• Type I (NF-1) - von Recklinghausen disease
• Type II (NF-2) - bilateral acoustic neuromas

24. Neurofibromatosis type-1 - (NF-1)
Appear during first year of life
Café-au-lait spots
• Most common phacomatosis
Increase in size and number
throughout childhood
• Affects 1:4000 individuals
• Presents in childhood
• Gene localized to chromosome 17q11

25. Fibroma molluscum in NF-1
• Appear at puberty
• Pedunculated, flabby nodules consisting of
neurofibromas or schwannomas
• Increase in number
throughout life
• Usually widely distributed

26. Plexiform neurofibroma in NF-1
• May be associated with
overgrowth of skin
• Appear during childhood
• Large and ill-defined

27. Skeletal defects in NF-1
• Mild head enlargement - uncommon
• Other - scoliosis, short stature, thinning
of long bones
• Facial hemiatrophy

28. Orbital lesions in NF-1
Spheno-orbital encephaloceleOptic nerve glioma in 15%
• Sagittal MRI scan of optic nerve
glioma invading hypothalamus
• Glioma may be uni or bilateral
• Axial CT scan of congenital absence
of left greater wing of sphenoid bone
• Pulsating proptosis without bruit!

29. Eyelid neurofibromas in NF-1
Nodular Plexiform
May cause mechanical ptosis May be associated with glaucoma

30. Intraocular lesions in NF-1
Lisch nodules
Very common - eventually
present in 95% of cases
Congenital ectropion uveae
Uncommon - may be
associated with glaucoma
Retinal astrocytomas
Rare - identical to those seen
In tuberous sclerosis
Choroidal naevi
Common - may be
Multifocal and bilateral

31. Ocular features of NF-2
Common - combined hamartomas of
RPE and retina
Very common -presenile cataract

32. Tuberous sclerosis (Bourneville disease)
• Diffuse thickening over
lumbar region
• Present in 40%
Shagreen patches
• Autosomal dominant
• Triad - mental handicap, epilepsy, adenoma sebaceum
Adenoma sebaceum
• Around nose and
cheeks
• Appear after age 1
and slowly enlarge
Ash leaf spots
• Hypopigmented skin patches
• In infants best detected using
ultraviolet light (Wood’s lamp)

33. Systemic hamartomas in tuberous sclerosis
Astrocytic cerebral hamartomas
• Slow-growing periventricular tumours
• May cause hydrocephalus, epilepsy and
mental retardation
• Usually asymptomatic and
innocuous
• Kidneys (angiomyolipoma),
heart (rhabdomyoma)
Visceral and subungual hamartomas

34. Retinal astrocytomas in tuberous scleritis
Dense white tumour Mulberry-like tumour
Early
• Innocuous tumour present in 50% of patients
• May be multiple and bilateral
Semitranslucent nodule White plaque
Advanced

35. Systemic features of v-H-L syndrome
Autosomal dominant
• Tumours - renal
carcinoma and
phaeochromocytoma
• Cysts - kidneys, liver,
pancreas, epididymis,
ovary and lungs
• Polycythaemia
CNS Haemangioblastoma
MRI-spinal cord tumour
Angiogram of cerebellar
tumour
Visceral tumours

36. Retinal capillary haemangioma
in v-H-L syndrome
Round orange-red mass
Early
• Vision-threatening tumour present in 50% of patients
• May be multiple and bilateral
Tiny lesion between
arteriole and venuole
Small red nodule
Associated dilatation and
tortuosity of feeder vessels
Advanced

37. Systemic features of Sturge-Weber syndrome
• Congenital, does not
blanche with pressure
• Associated with ipsilateral
glaucoma in 30% of cases
Naevus flammeus
• CT scan showing left
parietal haemangioma
• Complications –mental retard,
epilepsy and hemiparesis
Meningeal haemangioma

38. Ocular features of Sturge-Weber syndrome
Normal eye
Buphthalmos in 60%May be associated with
episcleral haemangioma
Affected eye
Diffuse choroidal haemangioma
Glaucoma

39. Peripheral corneal involvement in
rheumatoid arthritis
• Chronic and asymptomatic
• Circumferential thinning with intact
epithelium (‘contact lens cornea’)
• Acute and painful
• Circumferential ulceration and
infiltration
Treatment - systemic steroids and/or cytotoxic drugs
Without inflammation With inflammation

40. Peripheral corneal involvement in
Wegener granulomatosis and polyarteritis nodos
Circumferential and central
ulceration similar to Mooren ulcer
Unlike Mooren ulcer sclera may also
become involved
Treatment - systemic steroids and cyclophosphamide

41. Ocular manifestations ofOcular manifestations of
HIV infectionHIV infection

42. IntroductionIntroduction
AIDS is an infectious disease caused by the gradualAIDS is an infectious disease caused by the gradual
decrease indecrease in CD4+ T lymphocytesCD4+ T lymphocytes causingcausing
subsequent opportunistic infections and neoplasia. Itsubsequent opportunistic infections and neoplasia. It
is a blood borne and sexually transmitted infectionis a blood borne and sexually transmitted infection
caused by the HIV (Human Immunodeficiency Virus)caused by the HIV (Human Immunodeficiency Virus)
Approximately 36 million persons around the worldApproximately 36 million persons around the world
are infected. Up to 70% of patients infected with HIVare infected. Up to 70% of patients infected with HIV
will develop some form of ocular involvement, ie:will develop some form of ocular involvement, ie:
direct infection by HIV,opportunistic infections anddirect infection by HIV,opportunistic infections and
neoplasia.neoplasia.
HIV infection progresses thoughHIV infection progresses though different phasesdifferent phases

44. Ophthalmic Manifestations of HIV InfectionOphthalmic Manifestations of HIV Infection
AROUND THE EYEAROUND THE EYE

Molluscum ContagiosumMolluscum Contagiosum

Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus

Kaposi’s SarcomaKaposi’s Sarcoma

Conjunctival Squamous Cell CarcinomaConjunctival Squamous Cell Carcinoma

TrichomegalyTrichomegaly
FRONT OF THE EYEFRONT OF THE EYE

Dry EyeDry Eye

Anterior UveitisAnterior Uveitis
BACK OF THE EYEBACK OF THE EYE

Retinal MicrovasculopathyRetinal Microvasculopathy

CMV RetinitisCMV Retinitis

Acute Retinal NecrosisAcute Retinal Necrosis

Progressive Outer Retinal NecroProgressive Outer Retinal Necro

Toxoplasmosis RetinochoroiditisToxoplasmosis Retinochoroiditis

Syphilis RetinitisSyphilis Retinitis

Candida albicans endophthalmitiCandida albicans endophthalmiti
NEURO-OPHTHALMICNEURO-OPHTHALMIC

45. Molluscum ContagiosumMolluscum Contagiosum
Molluscum contagiosum is aMolluscum contagiosum is a
viral infection of the skin.viral infection of the skin.
Affects up to 20% ofAffects up to 20% of
symptomatic HIV infectedsymptomatic HIV infected
patients.patients.
Clinically appears like painless,Clinically appears like painless,
small, umbilicated nodules,small, umbilicated nodules,
which produce a waxywhich produce a waxy
discharge when pressured.discharge when pressured.
Treatment consists on excisionTreatment consists on excision
of the lesion, curettage orof the lesion, curettage or
cryotherapycryotherapy

46. Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
Due to the reactivation of a latent infection by VaricellaDue to the reactivation of a latent infection by Varicella
Zoster Virus in the dorsal root of trigeminal nerveZoster Virus in the dorsal root of trigeminal nerve
ganglion.ganglion.
It manifests with a maculo-papulo-vesicular rash whichIt manifests with a maculo-papulo-vesicular rash which
often is preceded by pain. Usually involves the upper lidoften is preceded by pain. Usually involves the upper lid
and does not cross the midlineand does not cross the midline
Treatment consists on oral Aciclovir 800mg 5 timesTreatment consists on oral Aciclovir 800mg 5 times
/day. In immunocompromised patients Aciclovir is given/day. In immunocompromised patients Aciclovir is given
intravenously for two weeks. Ocular manifestationsintravenously for two weeks. Ocular manifestations
such as anterior uveitis, are treated with topical steroidssuch as anterior uveitis, are treated with topical steroids
and mydriatics.and mydriatics.

48. Kaposi’s SarcomaKaposi’s Sarcoma
Kaposi’s sarcoma is a vascular neoplasm which is almostKaposi’s sarcoma is a vascular neoplasm which is almost
exclusively seen in patients with AIDS.exclusively seen in patients with AIDS.
KS is the commonest anterior segment lesion seen in AIDS;KS is the commonest anterior segment lesion seen in AIDS;
appears as a violaceous non-tender nodule on the eyelid orappears as a violaceous non-tender nodule on the eyelid or
conjunctiva.conjunctiva.
Typically KS involves only the skin but when there is aTypically KS involves only the skin but when there is a
reduced CD4 count it can progress rapidly to other sitesreduced CD4 count it can progress rapidly to other sites
such as the gastrointestinal tract and CNSsuch as the gastrointestinal tract and CNS
Treatment of ocular adnexal KS may be necessary forTreatment of ocular adnexal KS may be necessary for
cosmesis and to relieve functional difficulties. The mainstaycosmesis and to relieve functional difficulties. The mainstay
of treatment is radiotherapy. Other options includeof treatment is radiotherapy. Other options include
cryotherapy or chemotherapy.cryotherapy or chemotherapy.

50. Conjunctival Squamous Cell CarcinomaConjunctival Squamous Cell Carcinoma
Squamous cell carcinoma (SCC) is the third mostSquamous cell carcinoma (SCC) is the third most
common neoplasm associated to HIV infection.common neoplasm associated to HIV infection.
This may be due to an interaction between HIV, sunlightThis may be due to an interaction between HIV, sunlight
and Human Papilloma Virus infection.and Human Papilloma Virus infection.
SCC appears as a pink, gelatinous growth, usually in theSCC appears as a pink, gelatinous growth, usually in the
interpalpebral area. Often an engorgedinterpalpebral area. Often an engorged blood vesselblood vessel
feeding the tumour is seen.feeding the tumour is seen.
It may extend onto the cornea, but deep invasion andIt may extend onto the cornea, but deep invasion and
metastasis are rare.metastasis are rare.
The treatment of choice is local excision and cryotherapyThe treatment of choice is local excision and cryotherapy
but the presence of orbital invasion is an indication ofbut the presence of orbital invasion is an indication of
exenterationexenteration

52. TrichomegalyTrichomegaly
Trichomegaly orTrichomegaly or
hypertrichosis is anhypertrichosis is an
exaggerated growth ofexaggerated growth of
the eye lashes found inthe eye lashes found in
the later stages of thethe later stages of the
diseasedisease
The cause is not knownThe cause is not known
When symptomatic or forWhen symptomatic or for
cosmetic reasons thecosmetic reasons the
eyelashes can beeyelashes can be
trimmed or pluckedtrimmed or plucked

53. Dry EyeDry Eye
Sicca syndrome isSicca syndrome is
common with HIVcommon with HIV
infectioninfection
Patients complain ofPatients complain of
burning uncomfortableburning uncomfortable
red eyes.red eyes.
There are severalThere are several
causes of dry eye incauses of dry eye in
HIV infection fromHIV infection from
blepharitis toblepharitis to
destruction of thedestruction of the
lacrimal glands.lacrimal glands.
Treatment is with tearTreatment is with tear
supplementssupplements

54. Anterior UveitisAnterior Uveitis
HIV related anteriorHIV related anterior uveitis can be:uveitis can be:

Direct manifestation of the HIVDirect manifestation of the HIV
infectioninfection

autoimmnune in originautoimmnune in origin

drug induced ie: rifabutin,drug induced ie: rifabutin,
secondary to direct toxic effect uponsecondary to direct toxic effect upon
the non-pigmented epithelium of thethe non-pigmented epithelium of the
ciliary body.ciliary body.

Any of the different infectionsAny of the different infections
associated with AIDS :-associated with AIDS :-
Herpes Zoster Virus,Herpes Zoster Virus,
Herpes Simplex Virus,Herpes Simplex Virus,
Cytomegalovirus,Cytomegalovirus,
Toxoplasma gondiiToxoplasma gondii
SyphilisSyphilis

55. Rifabutin induced anterior uveitisRifabutin induced anterior uveitis

56. Retinal microvasculitisRetinal microvasculitis
Retinal microvasculopathy occurs in more than half of theRetinal microvasculopathy occurs in more than half of the
patients with HIVpatients with HIV
It is seen as transient cotton wool spots (CWS), intra-retinalIt is seen as transient cotton wool spots (CWS), intra-retinal
haemorrhages and microaneurysm,haemorrhages and microaneurysm,
Occurs in 50-70% of patients. It is usually asymptomatic.Occurs in 50-70% of patients. It is usually asymptomatic.
Unclear pathogenesis,but thought to be HIV infection of retinalUnclear pathogenesis,but thought to be HIV infection of retinal
vascular cells.vascular cells.
Serological test for HIV will confirm the diagnosis.Serological test for HIV will confirm the diagnosis.
Treatment is based in delaying the progression of the diseaseTreatment is based in delaying the progression of the disease
associated with HIV.associated with HIV.

57. Cotton Wool SpotsCotton Wool Spots

58. CMV RetinitisCMV Retinitis
IntroductionIntroduction

CMV Retinitis is the commonest intraocular ocular opportunistic infectionCMV Retinitis is the commonest intraocular ocular opportunistic infection
seen in patients with AIDSseen in patients with AIDS

Antibodies are found in almost 95% of adults, causing a trivial illness inAntibodies are found in almost 95% of adults, causing a trivial illness in
immunocompetent adults, however severe immunosuppression causesimmunocompetent adults, however severe immunosuppression causes
viral reactivation and tissue invasive diseaseviral reactivation and tissue invasive disease
PathogenesisPathogenesis

Reactivation from extraocular sites leads to seeding in other sites suchReactivation from extraocular sites leads to seeding in other sites such
as the retinaas the retina
EpidemiologyEpidemiology

The number of newly diagnosed cases of CMVR has decreased sinceThe number of newly diagnosed cases of CMVR has decreased since
the introduction of the HAARTthe introduction of the HAART
Highly Active Antiretroviral TherapyHighly Active Antiretroviral Therapy

59. CMV RetinitisCMV Retinitis
Clinical manifestationsClinical manifestations

Patients may complain of minor visual symptoms such as floaters,Patients may complain of minor visual symptoms such as floaters,
flashing lights or mild blurred vision, or be totally asymptomatic.flashing lights or mild blurred vision, or be totally asymptomatic.

It presents with a wide range of clinical appearances. From cotton woolIt presents with a wide range of clinical appearances. From cotton wool
spots which may look like HIV Retinopathy to confluent areas of fullspots which may look like HIV Retinopathy to confluent areas of full
thickness retinal necrosis and vasculitis. CMVR can progress in athickness retinal necrosis and vasculitis. CMVR can progress in a
“brushfire” pattern from the active edge of an active lesion. The retinal“brushfire” pattern from the active edge of an active lesion. The retinal
vessels in an affected area show attenuation, becoming ghost vesselsvessels in an affected area show attenuation, becoming ghost vessels
eventually.eventually.
TreatmentTreatment

The treatment of CMVR in patients with AIDS requires the use of specificThe treatment of CMVR in patients with AIDS requires the use of specific
antiviral agents, ganciclovir, foscarnet or cidovir in conjunction withantiviral agents, ganciclovir, foscarnet or cidovir in conjunction with
HAART.HAART.

These treatments can be administered orally, intravenously orThese treatments can be administered orally, intravenously or
intravitreally. Systemic treatment has the advantage of treating infectionintravitreally. Systemic treatment has the advantage of treating infection
elsewhere in the body as well as the other eye but has theelsewhere in the body as well as the other eye but has the
disadvantages of systemic side effects.disadvantages of systemic side effects.

60. CMV RetinitisCMV Retinitis

61. Acute Retinal NecrosisAcute Retinal Necrosis
ARN is a confluent peripheral whitening of the retina withARN is a confluent peripheral whitening of the retina with
marked vitritis and blood vessel closure. Optic neuritismarked vitritis and blood vessel closure. Optic neuritis
and retinal detachment are frequent complications.and retinal detachment are frequent complications.
ARN is usually due to Varicella-Zoster infection, but it canARN is usually due to Varicella-Zoster infection, but it can
also be caused by Herpes Simplex virus oralso be caused by Herpes Simplex virus or
Cytomegalovirus.Cytomegalovirus.
Initially described in the immunocompetent, it has alsoInitially described in the immunocompetent, it has also
been described in the immunosuppressed.been described in the immunosuppressed.
The diagnosis is mainly clinical and is confirmed by PCRThe diagnosis is mainly clinical and is confirmed by PCR
assays on vitreous samples.assays on vitreous samples.
Patients are treated with high doses of intravenousPatients are treated with high doses of intravenous
aciclovir or famciclovir, combined with laser treatment toaciclovir or famciclovir, combined with laser treatment to
prevent retinal detachment.prevent retinal detachment.

62. Acute Retinal NecrosisAcute Retinal Necrosis

63. Progressive Outer Retinal NecrosisProgressive Outer Retinal Necrosis
(Varicella-Zoster Retinitis)(Varicella-Zoster Retinitis)
PORN is a devastating viral retinitis caused by Varicella-ZosterPORN is a devastating viral retinitis caused by Varicella-Zoster
virus, without vitritis or retinal vasculitis.virus, without vitritis or retinal vasculitis.
The retinitis can be located anywhere but it is common for theThe retinitis can be located anywhere but it is common for the
lesions to coalesce and spread posteriorly in a rapid fashion.lesions to coalesce and spread posteriorly in a rapid fashion.
The main symptom is rapid loss of vision.The retina showsThe main symptom is rapid loss of vision.The retina shows
typically a white lesion with no haemorrhages or exudates.typically a white lesion with no haemorrhages or exudates.
Treatment is often unsatisfactory (Ganciclovir and Aciclovir).Treatment is often unsatisfactory (Ganciclovir and Aciclovir).
The prognosis is very poor and retinal detachment is common.The prognosis is very poor and retinal detachment is common.
Resolution may leave a white plaque with the appearance ofResolution may leave a white plaque with the appearance of
“cracked mud”.“cracked mud”.

65. Toxoplasma RetinochoroiditisToxoplasma Retinochoroiditis
Toxoplasmosis retinochoroiditis is an uncommonToxoplasmosis retinochoroiditis is an uncommon
infection of the eye in AIDS.infection of the eye in AIDS.
Ocular toxoplasmosis in HIV positive patients is differentOcular toxoplasmosis in HIV positive patients is different
in appearance from immunocompetent patients.in appearance from immunocompetent patients.
HIV infected patients often have bilateral and multifocalHIV infected patients often have bilateral and multifocal
disease associated with anterior uveitis and vitritisdisease associated with anterior uveitis and vitritis
No pigmented scars adjacent to the areas of retinalNo pigmented scars adjacent to the areas of retinal
necrosis. (unlike in immunocompetent patients)necrosis. (unlike in immunocompetent patients)
Retinochoroiditis is not self-limiting as it is inRetinochoroiditis is not self-limiting as it is in
imunocompetent patients.imunocompetent patients.

66. Toxoplasma RetinochoroiditisToxoplasma Retinochoroiditis
When testing patients for antibodies to toxoplasmosisWhen testing patients for antibodies to toxoplasmosis
both IgG and IgM levels may be raised, but inboth IgG and IgM levels may be raised, but in
immunocompromised patients these tests may beimmunocompromised patients these tests may be
negative.negative.
Often associated with toxoplasma lesions in the CentralOften associated with toxoplasma lesions in the Central
Nervous System.Nervous System.
Treatment in immunocompromised patientsTreatment in immunocompromised patients
sulphadiazine or clindamycin +/- pyrimethamine andsulphadiazine or clindamycin +/- pyrimethamine and
folinic acid (triple therapy).folinic acid (triple therapy).
Long term maintenance to prevent relapses.Long term maintenance to prevent relapses.

67. MRI T1 showing an uniformly
enhancing lesion in the
midbrain
One week later, the lesion
showing ring enhancement

68. ImmunocompetentImmunocompetent ImmunocompromisedImmunocompromised

69. Syphilis RetinitisSyphilis Retinitis
There is a strong association between syphilis andThere is a strong association between syphilis and
HIV infection.HIV infection.
It can manifest as a retinitis with dense vitritis,It can manifest as a retinitis with dense vitritis,
retinal vasculitis, serous retinal detachment orretinal vasculitis, serous retinal detachment or
neuroretinitis, conjunctivitis, anterior uveitis, cranialneuroretinitis, conjunctivitis, anterior uveitis, cranial
nerve palsies and optic neuritis.nerve palsies and optic neuritis.
Treatment high dose of intravenous Penicillin for 2Treatment high dose of intravenous Penicillin for 2
weeks.weeks.

71. Candida albicansCandida albicans
endophthalmitisendophthalmitis
Infection with candida albicans is rare. Candida albicansInfection with candida albicans is rare. Candida albicans
is the commonest cause of fungal endophthalmitisis the commonest cause of fungal endophthalmitis
Affected patients usually have a history of drug abuseAffected patients usually have a history of drug abuse
or indwelling central lines or immuno-compromised.or indwelling central lines or immuno-compromised.
In the initial stages, floaters are the main symptom. AsIn the initial stages, floaters are the main symptom. As
the condition progresses, whitish “puff-balls” andthe condition progresses, whitish “puff-balls” and
vitreous strands develop (‘string of pearls’)vitreous strands develop (‘string of pearls’)
The treatment depends on the severity of the ocularThe treatment depends on the severity of the ocular
involvement and systemic disease. The original fociinvolvement and systemic disease. The original foci
should be removed. The drugs of choice areshould be removed. The drugs of choice are
Amphotericin B and FluconazoleAmphotericin B and Fluconazole

72. Candida albicansCandida albicans
endophthalmitisendophthalmitis

73. GlossaryGlossary
CD4CD4: Director of the immune response. When activated it: Director of the immune response. When activated it
releasesreleases cytokines which in turn will activate the immunecytokines which in turn will activate the immune
systemsystem
Cotton Wool SpotsCotton Wool Spots: Light-coloured deposits in the retina: Light-coloured deposits in the retina
secondarysecondary to infarcts of the nerve fibre layerto infarcts of the nerve fibre layer
HAARTHAART: Highly Active Antiretroviral Therapy: Highly Active Antiretroviral Therapy
ImmunoblogulinImmunoblogulin: Protein in charge of fighting foreign: Protein in charge of fighting foreign
substances insubstances in our body.our body. IgGIgG is the commonest type ofis the commonest type of
immunoglobulin andimmunoglobulin and IgMIgM is the earliest classis the earliest class
of immunoglobulin.of immunoglobulin.
PCRPCR: Polymerase Chain Reaction is a technique used to make: Polymerase Chain Reaction is a technique used to make
numerous copies of an specific portion of DNAnumerous copies of an specific portion of DNA
VDRLVDRL: Venereal Disease Research Laboratory. The test: Venereal Disease Research Laboratory. The test
becomesbecomes negative after successful treatment of the disease.negative after successful treatment of the disease.

74. Special ThanksSpecial Thanks
Prof Jeevan K ShresthaProf Jeevan K Shrestha
Lecturer Jyoti Baba ShresthaLecturer Jyoti Baba Shrestha
Instructor Robin MishraInstructor Robin Mishra