SYSTEMIC DISEASES AND THE EYE

Presented by Dr.Hussien Zienab

Damascus - Teshreen hospital

ENDOCRINE DISORDERSAND THE EYE

FOREWORD

• The eye is a mirror which reflect the health of other systems in the human

body.

• The human eye, as an organ, can offer critical clues to the

diagnosis of various systemic illnesses.

• Ocular changes are common in various endocrine disorders such as diabetes

mellitus and Graves’ disease.

• Awareness of the associations between the ocular manifestations and

endocrine disorders is the first step in the diagnosis and

management of these complex patients.

PITUITARY GLAND

PITUITARY TUMORS

Foreword:

• Pituitary tumors are benign growths of cells in the pituitary gland .

• Small tumors of the pituitary are very common and usually without symptoms,

unless the tumor produces a hormone.

• However if the tumor enlarges,:

it can cause hormonal deficiencies by pressing on the normal pituitary cells.

If it grows even larger (> 1 cm), it can cause visual symptoms.

Ocular symptoms:

Depending on the size

of a pituitary tumor,

visual symptoms may

include:

• Blurred vision (in one or both

eyes)

• Loss of peripheral vision

• Double vision

A pituitary tumor can cause visual

symptoms arising from :

Pressure effects exerted on the optic

nerves, chiasma, or tracts.

Extension of the tumor into the

cavernous sinus can lead to paresis

of the third, fourth, or sixth nerves,

causing disorders of extraocular

movement.

Double vision :

Can occur when the normal eye

movements are affected.

Visual field defect in pituitary

tumor:

• are caused by tumor compression

on the optic nerve or chiasm.

• Depending on the size and

location of the tumor.

• The severity and symmetry of the

visual field defect may vary as well

as the anatomical relationship of the

chiasm to the pituitary stalk.

TYPES OF VISUAL FIELD DEFECT

Monocular Visual Field Deficit.

Chiasmal Field Deficit.

Junctional Field Deficits.

Monocular Visual Field Deficit:

• Asymmetric tumors may involve

one side of the chiasm or an optic

nerve, and most commonly presents

as a supertemporal quadrantanopsia.

Chiasmal Field Deficit:

• lesions at the level of the optic

chiasm produce a bitemporal hemianopia.

• Pituitary adenomas, which grow

upward from the pituitary stalk,

compress the chiasm from below, which

preferentially involves the inferior, nasal,

and macular nerve fibers. This corresponds

to superior, bitemporal, and central vision loss.

Junctional Field Deficits:

• central scotoma in one eye with

temporal visual field loss in the other eye .

• It caused by compression to an anterior

loop to the decussating nasal fibers

within the posterior optic nerve

"Wilbrand's knee,"

Diagnosis:

The doctor will examine:

• Visual acuity

• Color vision

• Peripheral vision .

• Eye movements

• The appearance of the

retina and optic nerve .

Papilledema is a rare finding in pituitary tumors.

because of the slow –growing nature of these tumors which

cause secondary optic atrophy before the tumor enlarges

sufficiently to increase ICP.

Treatment:

may include:

Surgery.

Medications to shrink the

tumor (depending on the type

of tumor cells).

Radiation treatment

Treatments for double vision

include:

• Blocking the vision from one eye.

• Prisms in eyeglasses.

• Surgery on eye muscles to correct

the alignment of the eyes.

Prognosis:• Visual loss :

often improve after a pituitary tumor is treated.

Or may be permanent if it has been present for a long time or is severe.

• Visual field:

The pattern of recovery after decompression suggests at least three

phases of improvement.

The three phases of improvement :

The early fast phase (surgery to 1 week) may lead to normalization in

some individuals.

The early slow phase (1-4 months) is the period of most notable

improvement .

A late phase (6 months to 3 years) of mild improvement doesn't appear

significant over all but maybe marked in some individuals.

the most common Hypothalmic –pituitary

syndromes

Septo-optic dysplasia (SOD).

Kallman's syndrome.

Empty sella syndrome.

Oliver Mcfarlane syndrome.

Septo-optic dysplasia (SOD):

It is a rare congenital anomaly

The classical triad of SOD includes:

(i) optic nerve hypoplasia .

(ii) pituitary hormone abnormalities.

(iii) midline brain defects.

Diagnosis of SOD can be made clinically

when two or more features of the triad are present.

Ocular manifestations:

• varying degrees of visual impairment.

• microphthalmia or anophthalmia.

• optic nerve dysplasia, or hypoplasia (wherein the optic nerve

appears small and pale).

Note...The presence of strabismus or nystagmus in a child at

birth with multiple congenital abnormalities should alert an

ophthalmologist to seek the opinion of an endocrinologist.

Kallman's syndrome:

A rare genetic disorder.

It consists of :

defective gonadotropin-releasing hormone synthesis.

olfactory nerve agenesis or hypoplasia.

Ocular manifestations: • optic atrophy.

• color blindness.

• oculomotor abnormalities.

Empty sella syndrome:

Is defined as an intrasellar herniation of the suprasellar space with

compression of the pituitary gland.

Is classified as :

Primary… caused by combination of:

• Incomplete diaphragma sella.

• An increased CSF fluid pressure.

Secondary…when it discovers following pituitary radiation or

pituitary surgery.

Ocular manifestations:

• Diminished visual acuity .

• Visual field defects such as peripheral field constriction,

bitemporal hemianopia, or quadrantanopia.

Note…... Patients with secondary empty sella predominantly

present with visual abnormality occurring due to arachnoidal

adhesions and traction on the optic chiasma.

Oliver Mcfarlane syndrome:

It is an extremely rare condition associated with :

chorioretinal degeneration, patients usually present with marked

decrease in vision.

Dwarfism with growth hormone deficiency.

Hair abnormalities.

Cerebellar dysfunction.

THYROID EYE DISEASES

Foreword:

An autoimmune condition, which means that the body’s immune

system mistakenly targets its own tissues.

It occurs with :

an overactive thyroid in :

Grave’s disease.

Toxic nodular goitre.

Hypothyroidism, for example

with Hashimoto’s disease.

GRAVES’ DISEASE

Foreword:

• Autoimmune disease, in which immunoglobulins are directed

against the TSH receptors on the thyroid cellular membrane.

• Most common form of thyrotoxicosis.

• May occur at any age but mostly from 20-40.

• Is a condition that predominantly affects females.

Clinical features:

I. Eye features.

II. Goitre.

III. Thyroid dermopathy (pretibial myxedema).

IV. Heat intolerance.

V. Cardiovascular.

VI. Gastrointestinal.

VII. Reproductive.

VIII. Bone.

IX. Neuromuscular.

X. Skin.

Eye features:

Classes 0-6, mnemonic “NO SPECS”

• Class 0: No signs or symptoms.

• Class 1: Only signs (lid retraction, stare, lid lag), no symptoms.

• Class 2: Soft tissue involvement (periorbital edema, congestion

or redness of the conjunctiva, and chemosis).

• Class 3: Proptosis .

• Class 4: Extraocular muscle involvement.

• Class 5: Corneal involvement.

• Class 6: Sight loss (optic nerve involvement).

Lid lag in downgaz lid retraction

CONJUNCTIVAL HYPERAEMIA PERIORBITAL AND LID SWELLING

proptosis chemosis

Diagnosis:

Low TSH, High FT4 and/or FT3.

If eye signs are present, the diagnosis of Graves’ disease can be made without

further tests.

If eye signs are absent and the patient is hyperthyroid with or without a goitre, we

need other tests for diagnosis (radioiodine, ………).

Symptoms and signs influence the management

strategy so we should assesse:

The degree of exophthalmos (exophthalmometer).

the intraocular pressure.

Extraocular muscle thickness

(CT, ultrasonography).

The degree of optic nerve compression

(electroretinogram, cortical visual evoked

potentials, and color contrast sensitivity ).

Treatment:

• Medical therapy.

• Surgical therapy.

• Radioactive iodine therapy.

Management of opthalmopathy:

Management involves cooperation between the endocrinologist and

the opthalmologist.

Keep head elevated at night to diminish periorbital edema.

If the cornea is exposed, it is important to prescribe artificial tears as

a means of corneal lubrication.

For more severely affected eyes, immunosuppressive therapy

with glucocorticoids benefit approximately 60% of patients with

thyroid-associated opthalmopathy.

If steroid therapy is not effective external x-ray therapy to the

retrobulbar area may be helpful.

If vision is threatened orbital decompression ( surgical) can be

used.

Hashimoto’s thyroiditis

• Is a common cause of hypothyroidism and goitre especially in children and young

adults.

• It is an autoimmune disease .

• Hypothyroidism usually has an insidious onset : patients present with complaints

of (lethargy, weight gain, dry and thickened skin ………………).

• Ophthalmologic features:

periorbital swelling (part of the generalized nonpitting skin edema of

myxedema).

characteristic loss of the outer third of the eyebrow.

open-angle glaucoma (deposition of a mucopolysaccharide within the

trabecular meshwork).

periorbital swelling loss of the outer third of the eyebrow.

PARATHYROID EYE DISEASE

There are four parathyroid

glands, which are located

behind the thyroid.

Hyperparathyroidism:

Hyperparathyroidism may be subdivided into primary,

secondary, tertiary, and pseudohyperparathyroidism.

Hyperparathyroidism causes hypercalcemia can lead to

ocular manifestations :

calcification of the conjunctiva.

calcified nodules of the eyelids.

band keratopathy.

Band keratopathy

calcification of the conjunctiva.

Hypoparathyroidism:

Is usually the result of the accidental removal of the parathyroid

glands during thyroidectomy, although it may be idiopathic in origin.

The lack of parathyroid hormone produces a clinical state of

hypocalcemia and hyperphosphatemia.

The ocular response to hypocalcemia is:

cataractogenesis:

At presentation the lens develops subcapsular cataract.

Which with progression, involves the lenticular cortex.

chronic keratoconjunctivitis (especially in children with idiopathic

Hypoparathyroidism ).

subcapsular cataractchronic keratoconjunctivitis

Adrenal eye disease

Cushing's Syndrome:

Excessive production of adrenocortical products.

Ocular involvement is poorly defined and may include :

Cataract (posterior subcapsular type) due to prolonged administration of

steroids not a feature of endogenous steroid overproduction.

elevation of intraocular pressure.

hypertensive retinopathy.

proptosis.

Addison's Disease:

Caused by insufficiency of the

adrenal cortex.

ocular manifestations:

pigmentation involving the

eyelids and conjunctiva.

Papilledema caused by

increased

intracranial pressure.

Neuroblastoma:

Neuroblastomas arise from primitive neuroectodermal elements.

Patients may present with an abdominal mass and, because most of these

tumors secrete catecholamines.

ocular manifestation :

orbital metastatic can present with:

proptosis.

subconjunctival hemorrhage .

ecchymosis of the eyelids.

Horner's syndrome, usually associated with heterochromia iridis (less common

).

ecchymosis of the eyelids heterochromia iridis

Pheochromocytoma:

This rare catecholamine-secreting

tumor originates in chromaffin cells.

The major ophthalmic feature

of the condition is hypertensive

retinopathy with:

flame-shaped hemorrhages.

cotton-wool spots.

narrowed arteries.

swollen optic discs.

Gonadal Disorders:

Turner syndrome:

Turner syndrome is a condition in which there is an absence or structural

abnormality of one X chromosome in phenotypic females.

Ocular manifestations:

strabismus

ptosis

hypertelorism.

epicanthus.

red–green color deficiency.

Ocular hypertension and glaucoma.

Klinefelter's syndrome:

Is the most frequent form of sex chromosome aneuploidy.

Ocular manifestations include:

colobomas of the iris,

choroid and optic nerve.

microphthalmia.

strabismus.

Thanks