Thyroid ophthalmopathy:
Thyroid eye disease (TED), also known as thyroid-associated orbitopathy and
Graves ophthalmopathy, is a very common orbital disorder, and is the most
common cause of both bilateral and unilateral proptosis in an adult. TED
typically proceeds through a congestive (inflammatory) stage in which the
eyes are red and painful; this tends to remit within 1–3 years and only about
10% of patients develop serious longterm ocular problems. A fibrotic
(quiescent) stage follows in which the eyes are white, although a painless
motility defect may be present. Clinical features broadly can be categorized
into (i) soft tissue involvement, (ii) lid retraction, (iii)proptosis, (iv) optic
neuropathy and (v) restrictive myopathy. The ocular signs of thyroid
diseases arise from 2 sources:
1: sympathetic over activity that causing many of eyelid signs
2: increase volume of orbital contents
Signs:
a- Lid lag and lid retraction
b- Orbital congestion
c- Exophthalmos which is either unilateral or bilateral
d- Extra ocular muscles involvement which include the followings:
- Enlargement of muscles due to increase mucopolysaccharide content,
(the size of the muscles may reach 6 times than the normal)
-contracture of the muscles involving the inferior rectus, the medial
rectus result in limitation of ocular movement (restrictive myopathy)
Eye and systemic
diseases
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e- Corneal involvement which include exposure keratitis and ocular irritation
as a result of rapid dryness of tear film.
F-Optic nerve involvement: including papillitis and papilloedema.
Parathyroid gland diseases:
Hypercalcemia: Ocular findings: - Ca
++
crystals deposition in cornea and
conjunctiva ,Band shape keratopathy( in palpebral fissure)
Hypocalcaemia: leading to cataract
Disease of the blood
1-sickle cell disease:
**Anterior segment:
• Conjunctiva: Dark red corkscrew- or comma-shaped vessels
that are
typically transient.
• Iris: Patches of ischaemic atrophy and occasionally
rubeosis.
• Hyphaema: may be spontaneous or follow minor trauma.
Careful
IOP control (avoiding CAI) is critical to reduce the
risk of RVO in the
presence of a hyphaema.
**Non-proliferative retinopathy:
• Venous changes. Tortuosity is very common and is thought to be due to
peripheral arteriovenous shunting.
• Arteriolar changes. Occlusions can involve branch, central or macular
vessels. ‘Silver wiring’ of arterioles in the peripheral retina signifies
previously occluded vessels. Corkscrewing of peripheral vessels may be seen.
• Optic disc sign of sickling’. Dark red blots on the disc surface due to
small vessel occlusion.
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• ‘Salmon patches: Orange–red mid-peripheral superficial intraretinal
haemorrhages that may break through to become preretinal or subretinal.
• Black sunbursts. Patches of peripheral RPE hyperplasia and chorioretinal
atrophy that evolve from some salmon patches.
**Proliferative retinopathy
Diagnosis:The development of proliferative retinopathy is usually insidious,
with no symptoms unless vitreous haemorrhage or retinal detachment
occurs.
• Stage 1. Peripheral arteriolar occlusion.
• Stage 2. Peripheral arteriovenous anastomosis proximal to non-perfused
areas.
• Stage 3. ‘Sea fan’ neovascularization develops at
the edge of perfused
retina, usually with a single supplying
arteriole and a single draining venule.
• Stage 4. Vitreous haemorrhage from the new vessels.
• Stage 5. Rhegmatogenous retinal detachment caused by a retinal break
associated with extensive fibrovascular proliferation. Tractional detachment
may also occur.
Treatment:
• Observation if vitreous haemorrhage has not occurred, particularly in
middle-aged and older patients. Many neovascular complexes involute
spontaneously as a result of auto-infarction or fibrotic strangulation,
subsequently appearing as greyish fibrovascular lesions.
• Laser or cryotherapy ablation of peripheral non-perfused retina is
probably the optimal approach, though ablation of neovascularization may also
be used.
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• Vitreoretinal surgery may be required for tractional retinal detachment
and/or persistent vitreous haemorrhage.
2- Polycythaemia:
is caused by the neoplastic proliferation of
erythrocytes with increased bone marrow activity and hyperviscosity. The
ocular findings related to blood hyper viscosity resulting in dilatation of
conjunctival and retinal blood vessels, and central retinal vein occlusion.
3-leukaemia:
Ocular involvement is more commonly seen in the acute
than the chronic forms and virtually any ocular structure may be involved.
Primary leukaemic infiltration is fairly rare. Secondary changes are more
common and include intraocular bleeding, infection and vascular occlusion.
• Fundus: Retinal haemorrhages and cotton wool spots are common. Roth
spots are retinal haemorrhages with white centres , the whitish element
thought to be composed of coagulated fibrin in most cases. They occur in
acute leukaemias. Peripheral retinal neovascularization is an occasional
feature of chronic myeloid leukaemia . Retinal and choroidal infiltrates may
occur and may masquerade as posterior uveitides; In chronic leukaemia they
may give rise to a ‘leopard skin’ appearance. Optic nerve infiltration may
cause swelling and visual loss.
• Other features: Orbital involvement, particularly in children. Iris
thickening, iritis and pseudohypopyon. Spontaneous subconjunctival
haemorrhage and hyphaema.
Connective tissue disorders
A-
Marfan syndrome
:
AD inheritance.
Musculoskeletal features include a tall, thin stature, long fingers and toes
(arachnodactyly), a narrow high-arched palate, Kyphoscoliosis, sternal
abnormalities, mild joint laxity ,muscular underdevelopment and
predisposition to hernias .
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Cardiovascular lesions include dilatation of the aortic root, mitral valve
prolapse and aortic aneurysm formation.
Bilateral ectopia lentis (80%); subluxation is most frequently
superotemporal. The zonule is frequently intact so that accommodation is
retained ,although rarely the lens may dislocate into the anterior chamber
or Vitreous.
Other ocular features: angle anomaly may lead to glaucoma, retinal
detachment; there may be hypoplasia of the dilator pupillae,
microspherophakia, and strabismus.
B-
Sjogren syndrome
:
90% are females, the patient presented with
xerostomia, chronic arthritis and dry eye (kerato conjunctivitis sicca)
C- rheumatoid arthritis:
Ocular features: Iritis, scleritis may
lead to sclera melting (sclera malacia performance) and dry eye.
D- Juvenile rheumatoid arthritis:
Ocular findings are: Iritis (bilateral), cataract and band shape keratopathy.
E- ankylosing spondylitis
:
It is inflammatory disease of the hip,
sacro iliac joints, shoulder joints and spine, HLA B27.
F- S.L.E.:
the patient presented with cotton wool spots in the retina,
superficial and deep retinal hemorrhages, retinal arterial and venous
occlusions.
g- Myotonia dystrophica
:
it is an autosomal dominant disease
characterized by muscle wasting of extremities ,frontal baldness, testicular
atrophy, atrophy of muscles of mastication
Ocular findings: Cataract is the most common ocular findings and ptosis
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h- Myasthenia gravis:
Myasthenia gravis (MG) is an autoimmune disease in which antibodies
mediate damage and destruction of acetylcholine receptors in striated
muscle. The resultant impairment of neuromuscularconduction causes
weakness and fatigability of skeletal musculature, but not of cardiac and
involuntary muscles. The disease affects females twice as commonly as
males. MG may be ocular, bulbar (affecting the cranial nerves arising from
the lower brainstem) or generalized.
Ocular myasthenia
: Ocular involvement occurs in 90% of cases and is
the presenting feature in 60%. Two-thirds of patients have both ptosis and
diplopia.
• Ptosis is insidious, bilateral and frequently asymmetrical. Typically worse
at the end of the day. Worse on prolonged (60 second) upgaze due to
fatigue.
• Diplopia is frequently vertical, although any or all of the extraocular
muscles may be affected. Patients with stable deviations may benefit from
muscle surgery, botulinum toxin injection or a combination of both.
• Nystagmoid movements :MG should be considered in the differential
diagnosis of any ocular motility disorder that does not fit with a recognized
pattern.
Investigations
• Ice pack test: This tests for an improvement after an ice pack is placed
on the ptotic eyelid (or other affected muscle) for 2 minutes, as cold
inhibits the breakdown of acetylcholine by acetylcholinesterase.
• Antibody testing supports a diagnosis of MG and predicts the likelihood
of thymoma.
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• Edrophonium (Tensilon) test Edrophonium is a short-acting
anticholinesterase that confers a transient improvement of weakness in MG.
• Electromyography shows characteristic features.
• Muscle biopsy.
• Thoracic imaging (MR, CT) to detect thymoma, present in 10%.
Treatment:
An anticholinesterase agent such as pyridostigmine may be
used
alone in mild disease, but is usually combined with steroids and
other
immunosuppressive treatment (e.g. azathioprine). Plasmapheresis
and
intravenous immunoglobulins are shorter-term
measures to address acute
illness; emergency respiratory support
is rarely required. Thymectomy is
performed if a thymoma is
present.
i-
Steven Johnson syndrome (erythema multiformae):
The basic pathology is vasculitis; it is associated with infectious agents or
drugs . Ocular findings: Conjunctivitis, Iritis, dry eye, trichiasis and
symplepharon
.
A IDS
It is fatal multi systemic disease of cell mediated immune system defined
by the presence of opportunistic infections and uncommon neoplasm in
individual infected with H.I.V who has no other known cause of immune
deficiency . Common ocular manifestations:
a- Retinal cotton wool spots 27%-53%
b- C.M.V retinitis 4%-34%
c- Retinal hemorrhages 6%-40%
d- KAPOSI sarcoma of the conjunctiva 10%
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Rare ocular manifestations: H.Z.O, toxoplasmosis, fungal infections, acute
retinal necrosis syndrome due to Herpes Simplex retinitis.
Sy philis and T.B
** SYPHILIS
:
Congenital syphilis: the patient presented with interstitial keratitis,
pigmentary retinopathy.
Acquired syphilis: the patient presented with patchy neuroretinitis,
papillitis, vitritis and uveitis.
** T.B
The patient presented with: Chronic granulomatus irido cyclitis; Iris
nodules, Peri-phlebitis of retinal blood vessels, retinal hemorrhages Focal or
diffuse choroiditis
Hy pertensive retinopathy
Retinopathy:
The primary response of the retinal arterioles to systemic hypertension
is
vasoconstriction. Arteriolosclerosis
refers to hardening and loss of
elasticity of small vessel
walls, manifested most obviously by arteriovenous
(AV) nipping
(nicking) at crossing points; its presence makes it probable that
hypertension has been present for many years, even if the BP
is currently
controlled. In sustained hypertension the inner
blood–retinal barrier is
disrupted, increased vascular permeability
leading to flame-shaped retinal
haemorrhages and oedema.
• Grade 1. Mild generalized retinal arteriolar narrowing.
• Grade 2. Focal arteriolar narrowing and arteriovenous nipping. A ‘copper
wiring’ opacified appearance of arteriolar walls may be seen.
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• Grade 3. Grade 2 plus retinal haemorrhages (dot, blot, flame), exudates
‘macular star’ and cotton wool spots.
• Grade 4. Severe grade 3 plus optic disc swelling; this is a marker of
malignant hypertension.
Choroidopathy:
Hypertensive choroidopathy is rare but may occur as the result of an
acute hypertensive crisis (accelerated hypertension) in young adults.
Exudative retinal detachment:
sometimes bilateral, may occur in acute severe hypertension such as that
associated with toxaemia of pregnancy.
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M.B.ch.B F.A.B.Ophth. F.I.C.O V.R.S
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