It is an autoimmune chronic inflammatory disease with
multi-system involvement. It typically occurs in
women between 40 and 50 years of age. Its main
feature is an aqueous deficiency dry eye — the
keratoconjunctivitis sicca (KCS). In primary Sjogren’s
syndrome patients present with sicca complex– a
combination of KCS and xerostomia (dryness of
mouth). In secondary Sjogren’s syndrome dry eye
and/or dry mouth are associated with an autoimmune
disease, commonly rheumatoid arthritis. Its
pathological features include focal accumulation and
infiltration by lymphocytes and plasma cells with
destruction of lacrimal and salivary glandular tissue.
Thursday, December 30, 2010
THE DRY EYE
The dry eye per se is not a disease entity, but a
symptom complex occurring as a sequelae to
deficiency or abnormalities of the tear film.
Etiology
1. Aqueous tear deficiency. It is also known as
keratoconjunctivitis sicca. It is seen in conditions
like congenital alacrimia, paralytic hyposecretion,
primary and secondary Sjogren’s syndrome, Riley Day
syndrome and idiopathic hyposecretion.
2. Mucin deficiency dry eye. It occurs when goblet
cells are damaged, as in hypovitaminosis A
(xerophthalmia) and conjunctival scarring diseases
such as Stevens-Johnson syndrome, trachoma,
chemical burns, radiations and ocular pemphigoid.
3. Lipid deficiency and abnormalities. Lipid
deficiency is extremely rare. It has only been
described in some cases of congenital anhydrotic
ectodermal dysplasia along with absence of
meibomian glands. However, lipid abnormalities are
quite common in patients with chronic blepharitis and
chronic meibomitis.
4. Impaired eyelid function. It is seen in patients
with Bell’s palsy, exposure keratitis, dellen,
symblepharon, pterygium, nocturnal lagophthalmos
and ectropion.
5. Epitheliopathies. Owing to the intimate
relationship between the corneal surface and tear film,
alterations in corneal epithelium affect the stability
of tear film.
Clinical features
Symptoms suggestive of dry eye include irritation,
foreign body (sandy) sensation, feeling of dryness,
itching, non-specific ocular discomfort and
chronically sore eyes not responding to a variety of
drops instilled earlier.
Signs of dry eye include: presence of stringy mucus
and particulate matter in the tear film, lustureless
ocular surface, conjunctival xerosis, reduced or absent
marginal tear strip and corneal changes in the form of
punctate epithelial erosions and filaments.
Tear film tests
These include tear film break-up time (BUT), Schirmer-
I test, vital staining with Rose Bengal, tear levels of
lysozyme and lactoferrin, tear osmolarity and
conjunctival impression cytology. Out of these BUT,
Schirmer-I test and Rose Bengal staining are most
important and when any two of these are positive,
diagnosis of dry eye syndrome is confirmed.
1. Tear film break-up (BUT). It is the interval between
a complete blink and appearance of first randomly
distributed dry spot on the cornea. It is noted after
instilling a drop of fluorescein and examining in a
cobalt-blue light of a slit-lamp. BUT is an indicator of
adequacy of mucin component of tears. Its normal
values range from 15 to 35 seconds. Values less than
10 seconds imply an unstable tear film.
2. Schirmer-I test. It measures total tear secretions. It
is performed with the help of a 5 × 35 mm strip of
Whatman-41 filter paper which is folded 5 mm from
one end and kept in the lower fornix at the junction of
lateral one-third and medial two-thirds. The patient is
asked to look up and not to blink or close the eyes
(Fig. 15.4). After 5 minutes wetting of the filter paper
strip from the bent end is measured. Normal values of
Schirmer-I test are more than 15 mm. Values of 5-10
mm are suggestive of moderate to mild
keratoconjunctivitis sicca (KCS) and less than 5 mm
of severe KCS.
3. Rose Bengal staining. It is a very useful test for
detecting even mild cases of KCS. Depending upon
the severity of KCS three staining patterns A, B and
C have been described: ‘C’ pattern represents mild or
early cases with fine punctate stains in the
interpalpebral area; ‘B’ the moderate cases with
extensive staining; and ‘A’ the severe cases with
confluent staining of conjunctiva and cornea.
Treatment
At present, there is no cure for dry eye. The following
treatment modalities have been tried with variable
results:
1. Supplementation with tear substitutes. Artificial
tears remains the mainstay in the treatment of dry
eye. These are available as drops, ointments and slowrelease
inserts. Mostly available artificial tear drops
contain either cellulose derivatives (e.g., 0.25 to 0.7%
methyl cellulose and 0.3% hypromellose) or polyvinyl
alcohol (1.4%).
2. Topical cyclosporine (0.05%, 0.1%) is reported to
be very effective drug for dry eye in many recent
studies. It helps by reducing the cell-mediated
inflammation of the lacrimal tissue.
3. Mucolytics, such as 5 percent acetylcystine used
4 times a day help by dispersing the mucus threads
and decreasing tear viscosity.
4. Topical retinoids have recently been reported to
be useful in reversing the cellular changes (squamous
metaplasia) occurring in the conjunctiva of dry eye
patients.
5. Preservation of existing tears by reducing
evaporation and decreasing drainage.
Evaporation can be reduced by decreasing room
temperature, use of moist chambers and protective
glasses.
Punctal occlusion to decrease drainage can be
carried out by collagen implants, cynoacrylate
tissue adhesives, electrocauterisation, argon laser
occlusion and surgical occlusion to decrease the
drainage of tears in patients with very severe
dry eye
symptom complex occurring as a sequelae to
deficiency or abnormalities of the tear film.
Etiology
1. Aqueous tear deficiency. It is also known as
keratoconjunctivitis sicca. It is seen in conditions
like congenital alacrimia, paralytic hyposecretion,
primary and secondary Sjogren’s syndrome, Riley Day
syndrome and idiopathic hyposecretion.
2. Mucin deficiency dry eye. It occurs when goblet
cells are damaged, as in hypovitaminosis A
(xerophthalmia) and conjunctival scarring diseases
such as Stevens-Johnson syndrome, trachoma,
chemical burns, radiations and ocular pemphigoid.
3. Lipid deficiency and abnormalities. Lipid
deficiency is extremely rare. It has only been
described in some cases of congenital anhydrotic
ectodermal dysplasia along with absence of
meibomian glands. However, lipid abnormalities are
quite common in patients with chronic blepharitis and
chronic meibomitis.
4. Impaired eyelid function. It is seen in patients
with Bell’s palsy, exposure keratitis, dellen,
symblepharon, pterygium, nocturnal lagophthalmos
and ectropion.
5. Epitheliopathies. Owing to the intimate
relationship between the corneal surface and tear film,
alterations in corneal epithelium affect the stability
of tear film.
Clinical features
Symptoms suggestive of dry eye include irritation,
foreign body (sandy) sensation, feeling of dryness,
itching, non-specific ocular discomfort and
chronically sore eyes not responding to a variety of
drops instilled earlier.
Signs of dry eye include: presence of stringy mucus
and particulate matter in the tear film, lustureless
ocular surface, conjunctival xerosis, reduced or absent
marginal tear strip and corneal changes in the form of
punctate epithelial erosions and filaments.
Tear film tests
These include tear film break-up time (BUT), Schirmer-
I test, vital staining with Rose Bengal, tear levels of
lysozyme and lactoferrin, tear osmolarity and
conjunctival impression cytology. Out of these BUT,
Schirmer-I test and Rose Bengal staining are most
important and when any two of these are positive,
diagnosis of dry eye syndrome is confirmed.
1. Tear film break-up (BUT). It is the interval between
a complete blink and appearance of first randomly
distributed dry spot on the cornea. It is noted after
instilling a drop of fluorescein and examining in a
cobalt-blue light of a slit-lamp. BUT is an indicator of
adequacy of mucin component of tears. Its normal
values range from 15 to 35 seconds. Values less than
10 seconds imply an unstable tear film.
2. Schirmer-I test. It measures total tear secretions. It
is performed with the help of a 5 × 35 mm strip of
Whatman-41 filter paper which is folded 5 mm from
one end and kept in the lower fornix at the junction of
lateral one-third and medial two-thirds. The patient is
asked to look up and not to blink or close the eyes
(Fig. 15.4). After 5 minutes wetting of the filter paper
strip from the bent end is measured. Normal values of
Schirmer-I test are more than 15 mm. Values of 5-10
mm are suggestive of moderate to mild
keratoconjunctivitis sicca (KCS) and less than 5 mm
of severe KCS.
3. Rose Bengal staining. It is a very useful test for
detecting even mild cases of KCS. Depending upon
the severity of KCS three staining patterns A, B and
C have been described: ‘C’ pattern represents mild or
early cases with fine punctate stains in the
interpalpebral area; ‘B’ the moderate cases with
extensive staining; and ‘A’ the severe cases with
confluent staining of conjunctiva and cornea.
Treatment
At present, there is no cure for dry eye. The following
treatment modalities have been tried with variable
results:
1. Supplementation with tear substitutes. Artificial
tears remains the mainstay in the treatment of dry
eye. These are available as drops, ointments and slowrelease
inserts. Mostly available artificial tear drops
contain either cellulose derivatives (e.g., 0.25 to 0.7%
methyl cellulose and 0.3% hypromellose) or polyvinyl
alcohol (1.4%).
2. Topical cyclosporine (0.05%, 0.1%) is reported to
be very effective drug for dry eye in many recent
studies. It helps by reducing the cell-mediated
inflammation of the lacrimal tissue.
3. Mucolytics, such as 5 percent acetylcystine used
4 times a day help by dispersing the mucus threads
and decreasing tear viscosity.
4. Topical retinoids have recently been reported to
be useful in reversing the cellular changes (squamous
metaplasia) occurring in the conjunctiva of dry eye
patients.
5. Preservation of existing tears by reducing
evaporation and decreasing drainage.
Evaporation can be reduced by decreasing room
temperature, use of moist chambers and protective
glasses.
Punctal occlusion to decrease drainage can be
carried out by collagen implants, cynoacrylate
tissue adhesives, electrocauterisation, argon laser
occlusion and surgical occlusion to decrease the
drainage of tears in patients with very severe
dry eye
Structure of tear film
Wolff was the first to describe the detailed structure
of the fluid covering the cornea and called it
precorneal film. He described this film to consist of
three layers, which from posterior to anterior are
mucus layer, aqueous layer and lipid or oily layer
(Fig. 15.2).
1. Mucus layer. It is the innermost and thinnest
stratum of the tear film. It consists of mucin secreted
by conjunctival goblet cells and glands of Manz. It
converts the hydrophobic corneal surface into
hydrophilic one.
2. Aqueous layer. The bulk of tear film is formed by
this intermediate layer which consists of tears
secreted by the main and accessory lacrimal glands.
The tears mainly comprise of water and small
quantities of solutes such as sodium chloride, sugar,
urea and proteins. Therefore, it is alkaline and salty
in taste. It also contains antibacterial substances like
lysozyme, betalysin and lactoferrin.
3. Lipid or oily layer. This is the outermost layer of
tear film formed at air-tear interface from the secretions
of Meibomian, Zeis, and Moll glands. This layer
prevents the overflow of tears, retards their
evaporation and lubricates the eyelids as they slide
over the surface of the globe.
Functions of tear film
1. Keeps the cornea and conjunctiva moist.
2. It provides oxygen to the corneal epithelium.
3. Washes away debris and noxious irritants.
4. Prevents infection due to presence of antibacterial
substances.
5. Facilitates movements of the lids over the globe.
Secretion of tears
Tears are continuously secreted throughtout the day
by accessory (basal secretion) and main (reflex
secretion) lacrimal glands. Reflex secretion is in
response to sensations from the cornea and
conjunctiva, probably produced by evaporation and
break-up of tear film. Hyperlacrimation occurs due to
irritative sensations from the cornea and conjunctiva.
Afferent pathway of this secretion is formed by fifth
nerve and efferent by parasympathetic (secretomotor)
supply of lacrimal gland.
Elimination of tears
Tears flow downward and medially across the surface
of eyeball to reach the lower fornix and then via lacus
lacrimalis in the inner canthus. From where they are
drained by lacrimal passages into the nasal cavity
(Fig. 15.3A). This is brought about by an active
lacrimal pump mechanism constituted by fibres of the
orbicularis (especially Horner’s muscle) which are
inserted on the lacrimal sac. When the eye lids close
during blink, contraction of these fibres distends the
fundus of the sac, creates therein a negative pressure
which syphons the tears through punctum and
canaliculi into the sac (Fig. 15.3B). When the eyelids
open, the Horner’s muscle relaxes, the lacrimal sac
collapses and a positive pressure is created which
forces the tears down the nasolacrimal duct into the
nose (Fig. 15.3C). Therefore, in atonia of sac, tears
are not drained through the lacrimal passages, in
spite of anatomical patency; resulting in epiphora.
of the fluid covering the cornea and called it
precorneal film. He described this film to consist of
three layers, which from posterior to anterior are
mucus layer, aqueous layer and lipid or oily layer
(Fig. 15.2).
1. Mucus layer. It is the innermost and thinnest
stratum of the tear film. It consists of mucin secreted
by conjunctival goblet cells and glands of Manz. It
converts the hydrophobic corneal surface into
hydrophilic one.
2. Aqueous layer. The bulk of tear film is formed by
this intermediate layer which consists of tears
secreted by the main and accessory lacrimal glands.
The tears mainly comprise of water and small
quantities of solutes such as sodium chloride, sugar,
urea and proteins. Therefore, it is alkaline and salty
in taste. It also contains antibacterial substances like
lysozyme, betalysin and lactoferrin.
3. Lipid or oily layer. This is the outermost layer of
tear film formed at air-tear interface from the secretions
of Meibomian, Zeis, and Moll glands. This layer
prevents the overflow of tears, retards their
evaporation and lubricates the eyelids as they slide
over the surface of the globe.
Functions of tear film
1. Keeps the cornea and conjunctiva moist.
2. It provides oxygen to the corneal epithelium.
3. Washes away debris and noxious irritants.
4. Prevents infection due to presence of antibacterial
substances.
5. Facilitates movements of the lids over the globe.
Secretion of tears
Tears are continuously secreted throughtout the day
by accessory (basal secretion) and main (reflex
secretion) lacrimal glands. Reflex secretion is in
response to sensations from the cornea and
conjunctiva, probably produced by evaporation and
break-up of tear film. Hyperlacrimation occurs due to
irritative sensations from the cornea and conjunctiva.
Afferent pathway of this secretion is formed by fifth
nerve and efferent by parasympathetic (secretomotor)
supply of lacrimal gland.
Elimination of tears
Tears flow downward and medially across the surface
of eyeball to reach the lower fornix and then via lacus
lacrimalis in the inner canthus. From where they are
drained by lacrimal passages into the nasal cavity
(Fig. 15.3A). This is brought about by an active
lacrimal pump mechanism constituted by fibres of the
orbicularis (especially Horner’s muscle) which are
inserted on the lacrimal sac. When the eye lids close
during blink, contraction of these fibres distends the
fundus of the sac, creates therein a negative pressure
which syphons the tears through punctum and
canaliculi into the sac (Fig. 15.3B). When the eyelids
open, the Horner’s muscle relaxes, the lacrimal sac
collapses and a positive pressure is created which
forces the tears down the nasolacrimal duct into the
nose (Fig. 15.3C). Therefore, in atonia of sac, tears
are not drained through the lacrimal passages, in
spite of anatomical patency; resulting in epiphora.
Lacrimal Apparatus Anatomy
The lacrimal apparatus comprises (1) Main lacrimal
gland, (2) Accessory lacrimal glands, and (3) Lacrimal
passages, which include: puncta, canaliculi, lacrimal
sac and nasolacrimal duct (NLD) (Fig. 15.1).
Main lacrimal gland
It consists of an upper orbital and a lower palpebral
part. (1) Orbital part is larger, about the size and
shape of a small almond, and is situated in the fossa
for lacrimal gland at the outer part of the orbital plate
of frontal bone. It has got two surfaces — superior
and inferior. The superior surface is convex and lies
in contact with the bone. The inferior surface is
concave and lies on the levator palpebrae superioris
muscle. (2) Palpebral part is small and consists of
only one or two lobules. It is situated upon the course
of the ducts of orbital part from which it is separated
by LPS muscle. Posteriorly, it is continuous with the
orbital part.
Ducts of lacrimal gland. Some 10-12 ducts pass
downward from the main gland to open in the lateral
part of superior fornix. One or two ducts also open in
the lateral part of inferior fornix.
Accessory lacrimal glands (Fig. 14.4)
1. Glands of Krause. These are microscopic glands
lying beneath the palpebral conjunctiva between
fornix and the edge of tarsus. These are about 42
in the upper fornix and 6-8 in the lower fornix.
2. Glands of Wolfring. These are present near the
upper border of the superior tarsal plate and
along the lower border of inferior tarsus.
Structure, blood supply and nerve supply
Structure. All lacrimal glands are serous acini, similar
in structure to the salivary glands. Microscopically
these consist of glandular tissue (acini and ducts),
connective tissue and puncta.
Blood supply. Main lacrimal gland is supplied by
lacrimal artery which is a branch of ophthalmic artery.
Nerve supply. (1) Sensory supply comes from lacrimal
nerve, a branch of the ophthalmic division of the fifth
nerve. (2) Sympathetic supply comes from the carotid
plexus of the cervical sympathetic chain. (3)
Secretomotor fibres are derived from the superior
salivary nucleus.
Lacrimal passages
1. Lacrimal puncta. These are two small, rounded or
oval openings on upper and lower lids, about 6 and
6.5 mm, respectively, temporal to the inner canthus.
Each punctum is situated upon a slight elevation
called lacrimal papilla which becomes prominent in
old age. Normally the puncta dip into the lacus
lacrimalis (collection of tear fluid in the inner canthus).
2. Lacrimal canaliculi. These join the puncta to the
lacrimal sac. Each canaliculus has two parts: vertical
(1-2 mm) and horizontal (6-8 mm) which lie at right
angle to each other. The horizontal part converges
towards inner canthus to open in the sac. The two
canaliculi may open separately or may join to form
common canaliculus which opens immediately into
the outer wall of lacrimal sac. A fold of mucosa at this
point forms the valve of Rosenmuller which prevents
reflux of tears.
3. Lacrimal sac. It lies in the lacrimal fossa located in
the anterior part of medial orbital wall. The lacrimal
fossa is formed by lacrimal bone and frontal process
of maxilla. It is bounded by anterior and posterior
lacrimal crests. When distended, lacrimal sac is about
15 mm in length and 5-6 mm in breadth. It has got
three parts: fundus (portion above the opening of
canaliculi), body (middle part) and the neck (lower
small part which is narrow and continuous with the
nasolacrimal duct).
4. Nasolacrimal duct (NLD). It extends from neck of
the lacrimal sac to inferior meatus of the nose. It is
about 15-18 mm long and lies in a bony canal formed
by the maxilla and the inferior turbinate. Direction of
the NLD is downwards, backwards and laterally.
Externally its location is represented by a line joining
inner canthus to the ala of nose. The upper end of the
NLD is the narrowest part.
There are numerous membranous valves in the
NLD, the most important is the valve of Hasner, which
is present at the lower end of the duct and prevents
reflux from the nose.
gland, (2) Accessory lacrimal glands, and (3) Lacrimal
passages, which include: puncta, canaliculi, lacrimal
sac and nasolacrimal duct (NLD) (Fig. 15.1).
Main lacrimal gland
It consists of an upper orbital and a lower palpebral
part. (1) Orbital part is larger, about the size and
shape of a small almond, and is situated in the fossa
for lacrimal gland at the outer part of the orbital plate
of frontal bone. It has got two surfaces — superior
and inferior. The superior surface is convex and lies
in contact with the bone. The inferior surface is
concave and lies on the levator palpebrae superioris
muscle. (2) Palpebral part is small and consists of
only one or two lobules. It is situated upon the course
of the ducts of orbital part from which it is separated
by LPS muscle. Posteriorly, it is continuous with the
orbital part.
Ducts of lacrimal gland. Some 10-12 ducts pass
downward from the main gland to open in the lateral
part of superior fornix. One or two ducts also open in
the lateral part of inferior fornix.
Accessory lacrimal glands (Fig. 14.4)
1. Glands of Krause. These are microscopic glands
lying beneath the palpebral conjunctiva between
fornix and the edge of tarsus. These are about 42
in the upper fornix and 6-8 in the lower fornix.
2. Glands of Wolfring. These are present near the
upper border of the superior tarsal plate and
along the lower border of inferior tarsus.
Structure, blood supply and nerve supply
Structure. All lacrimal glands are serous acini, similar
in structure to the salivary glands. Microscopically
these consist of glandular tissue (acini and ducts),
connective tissue and puncta.
Blood supply. Main lacrimal gland is supplied by
lacrimal artery which is a branch of ophthalmic artery.
Nerve supply. (1) Sensory supply comes from lacrimal
nerve, a branch of the ophthalmic division of the fifth
nerve. (2) Sympathetic supply comes from the carotid
plexus of the cervical sympathetic chain. (3)
Secretomotor fibres are derived from the superior
salivary nucleus.
Lacrimal passages
1. Lacrimal puncta. These are two small, rounded or
oval openings on upper and lower lids, about 6 and
6.5 mm, respectively, temporal to the inner canthus.
Each punctum is situated upon a slight elevation
called lacrimal papilla which becomes prominent in
old age. Normally the puncta dip into the lacus
lacrimalis (collection of tear fluid in the inner canthus).
2. Lacrimal canaliculi. These join the puncta to the
lacrimal sac. Each canaliculus has two parts: vertical
(1-2 mm) and horizontal (6-8 mm) which lie at right
angle to each other. The horizontal part converges
towards inner canthus to open in the sac. The two
canaliculi may open separately or may join to form
common canaliculus which opens immediately into
the outer wall of lacrimal sac. A fold of mucosa at this
point forms the valve of Rosenmuller which prevents
reflux of tears.
3. Lacrimal sac. It lies in the lacrimal fossa located in
the anterior part of medial orbital wall. The lacrimal
fossa is formed by lacrimal bone and frontal process
of maxilla. It is bounded by anterior and posterior
lacrimal crests. When distended, lacrimal sac is about
15 mm in length and 5-6 mm in breadth. It has got
three parts: fundus (portion above the opening of
canaliculi), body (middle part) and the neck (lower
small part which is narrow and continuous with the
nasolacrimal duct).
4. Nasolacrimal duct (NLD). It extends from neck of
the lacrimal sac to inferior meatus of the nose. It is
about 15-18 mm long and lies in a bony canal formed
by the maxilla and the inferior turbinate. Direction of
the NLD is downwards, backwards and laterally.
Externally its location is represented by a line joining
inner canthus to the ala of nose. The upper end of the
NLD is the narrowest part.
There are numerous membranous valves in the
NLD, the most important is the valve of Hasner, which
is present at the lower end of the duct and prevents
reflux from the nose.
Ptosis
Abnormal drooping of the upper eyelid is called ptosis.
Normally, upper lid covers about upper one-sixth of
the cornea, i.e., about 2 mm. Therefore, in ptosis it
covers more than 2 mm.
Types and etiology
I. Congenital ptosis
It is associated with congenital weakness
(maldevelopment) of the levator palpebrae superioris
(LPS). It may occur in the following forms:
1. Simple congenital ptosis (not associated with
any other anomaly) (Fig. 14.32A).
2. Congenital ptosis with associated weakness of
superior rectus muscle.
3. As a part of blepharophimosis syndrome, which
comprises congenital ptosis, blepharophimosis,
telecanthus and epicanthus inversus (Fig. 14.32B).
4. Congenital synkinetic ptosis (Marcus Gunn jawwinking
ptosis). In this condition there occurs
retraction of the ptotic lid with jaw movements
i.e., with stimulation of ipsilateral pterygoid muscle.
II. Acquired ptosis
Depending upon the cause it can be neurogenic,
myogenic, aponeurotic or mechanical.
1. Neurogenic ptosis. It is caused by innervational
defects such as third nerve palsy, Horner’s
syndrome, ophthalmoplegic migraine and multiple
sclerosis.
2. Myogenic ptosis. It occurs due to acquired
disorders of the LPS muscle or of the myoneural
junction. It may be seen in patients with
myasthenia gravis, dystrophia myotonica, ocular
myopathy, oculo-pharyngeal muscular dystrophy
and following trauma to the LPS muscle.
3. Aponeurotic ptosis. It develops due to defects of
the levator aponeurosis in the presence of a
normal functioning muscle. It includes involutional
(senile) ptosis, postoperative ptosis (which is
rarely observed after cataract and retinal
detachment surgery), ptosis due to aponeurotic
weakness associated with blepharochalasis, and
in traumatic dehiscence or disinsertion of the
aponeurosis.
4. Mechanical ptosis. It may result due to excessive
weight on the upper lid as seen in patients with
lid tumours, multiple chalazia and lid oedema. It
may also occur due to scarring (cicatricial ptosis)
as seen in patients with ocular pemphigoid and
trachoma.
Clinical evaluation
Following scheme may be adopted for work up of a
ptosis patient:
I. History. It should include age of onset, family
history, history of trauma, eye surgery and variability
in degree of the ptosis.
II. Examination
1. Exclude pseudoptosis (simulated ptosis) on
inspection. Its common causes are: microphthalmos,
anophthalmos, enophthalmos and phthisis bulbi.
2. Observe the following points in each case:
i. Whether ptosis is unilateral or bilateral.
ii. Function of orbicularis oculi muscle.
iii. Eyelid crease is present or absent.
iv. Jaw-winking phenomenon is present or not.
v. Associated weakness of any extraocular
muscle.
vi. Bell’s phenomenon (up and outrolling of the
eyeball during forceful closure) is present or
absent.
3. Measurement of amount (degree) of ptosis. In
unilateral cases, difference between the vertical height
of the palpebral fissures of the two sides indicates
the degree of ptosis (Fig. 14.33). In bilateral cases it
can be determined by measuring the amount of cornea
covered by the upper lid and then subtracting 2 mm.
Depending upon its amount the ptosis is graded as
Mild 2 mm
Moderate 3 mm
Severe 4 mm
4. Assessment of levator function. It is determined
by the lid excursion caused by LPS muscle (Burke’s
method). Patient is asked to look down, and thumb of
one hand is placed firmly against the eyebrow of the
patient (to block the action of frontalis muscle) by
the examiner. Then the patient is asked to look up and
the amount of upper lid excursion is measured with a
ruler (Fig. 14.34) held in the other hand by the examiner.
Levator function is graded as follows:
Normal 15 mm
Good 8 mm or more
Fair 5-7 mm
Poor 4 mm or less
5. Special investigations. Those required in patients
with acquired ptosis are as follows:
i. Tensilon test is performed when myasthenia is
suspected. There occurs improvement of ptosis
with intravenous injection of edrophonium
(Tensilon) in myasthenia.
ii. Phenylephrine test is carried out in patients
suspected of Horner’s syndrome.
iii. Neurological investigations may be required to
find out the cause in patient with neurogenic
ptosis.
6. Photographic record of the patient should be
maintained for comparison. Photographs should be
taken in primary position as well as in up and down
gazes.
Treatment
I. Congenital ptosis. It almost always needs surgical
correction. In severe ptosis, surgery should be
performed at the earliest to prevent stimulus
deprivation amblyopia. However, in mild and moderate
ptosis, surgery should be delayed until the age of 3-
4 years, when accurate measurements are possible.
Congenital ptosis can be treated by any of the
following operations:
1. Fasanella-Servat operation. It is performed in
cases having mild ptosis (1.5-2mm) and good levator
function. In it, upper lid is everted and the upper tarsal
border along with its attached Muller’s muscle and
conjunctiva are resected (Fig. 14.35).
2. Levator resection. It is a very commonly performed
operation for moderate and severe grades of ptosis.
It is contraindicated in patients having severe ptosis
with poor levator function.
Amount of levator resection required: Most of the
surgeons find it out by adjusting the lid margin in
relation to cornea during operation on the table in
individual case. However, a rough estimate in different
grades of ptosis is as follows:
Moderate ptosis
Level of LPS Amount of LPS to be
Function resected
Good 16-17 mm (minimal)
Fair18-22 mm (moderate)
Poor 23-24 mm (maximum)
Severe ptosis
Fair levator 23-24 mm (maximum
function LPS resected)
Techniques. Levator muscle may be resected by either
conjunctival or skin approach.
i. Conjunctival approach (Blaskowics’ operation):
This technique is comparatively easy but not
suitable for large amount of resection. In it LPS
muscle is exposed by an incision made through
the conjunctiva near the tarsal border, after the
upper lid is doubly everted over a Desmarre’s lid
retractor (Fig. 14.36).
ii. Skin approach (Everbusch’s operation): It is a
more frequently employed technique. It allows
comparatively better exposure of the LPS muscle
through a skin incision along the line of future lid
fold (Fig. 14.37).
3. Frontalis sling operation (Brow suspension):
This is performed in patients having severe ptosis
with no levator function. In this operation, lid is
anchored to the frontalis muscle via a sling (Fig.
14.38). Fascia lata or some non-absorbable material
(e.g., supramide suture) may be used as sling.
Normally, upper lid covers about upper one-sixth of
the cornea, i.e., about 2 mm. Therefore, in ptosis it
covers more than 2 mm.
Types and etiology
I. Congenital ptosis
It is associated with congenital weakness
(maldevelopment) of the levator palpebrae superioris
(LPS). It may occur in the following forms:
1. Simple congenital ptosis (not associated with
any other anomaly) (Fig. 14.32A).
2. Congenital ptosis with associated weakness of
superior rectus muscle.
3. As a part of blepharophimosis syndrome, which
comprises congenital ptosis, blepharophimosis,
telecanthus and epicanthus inversus (Fig. 14.32B).
4. Congenital synkinetic ptosis (Marcus Gunn jawwinking
ptosis). In this condition there occurs
retraction of the ptotic lid with jaw movements
i.e., with stimulation of ipsilateral pterygoid muscle.
II. Acquired ptosis
Depending upon the cause it can be neurogenic,
myogenic, aponeurotic or mechanical.
1. Neurogenic ptosis. It is caused by innervational
defects such as third nerve palsy, Horner’s
syndrome, ophthalmoplegic migraine and multiple
sclerosis.
2. Myogenic ptosis. It occurs due to acquired
disorders of the LPS muscle or of the myoneural
junction. It may be seen in patients with
myasthenia gravis, dystrophia myotonica, ocular
myopathy, oculo-pharyngeal muscular dystrophy
and following trauma to the LPS muscle.
3. Aponeurotic ptosis. It develops due to defects of
the levator aponeurosis in the presence of a
normal functioning muscle. It includes involutional
(senile) ptosis, postoperative ptosis (which is
rarely observed after cataract and retinal
detachment surgery), ptosis due to aponeurotic
weakness associated with blepharochalasis, and
in traumatic dehiscence or disinsertion of the
aponeurosis.
4. Mechanical ptosis. It may result due to excessive
weight on the upper lid as seen in patients with
lid tumours, multiple chalazia and lid oedema. It
may also occur due to scarring (cicatricial ptosis)
as seen in patients with ocular pemphigoid and
trachoma.
Clinical evaluation
Following scheme may be adopted for work up of a
ptosis patient:
I. History. It should include age of onset, family
history, history of trauma, eye surgery and variability
in degree of the ptosis.
II. Examination
1. Exclude pseudoptosis (simulated ptosis) on
inspection. Its common causes are: microphthalmos,
anophthalmos, enophthalmos and phthisis bulbi.
2. Observe the following points in each case:
i. Whether ptosis is unilateral or bilateral.
ii. Function of orbicularis oculi muscle.
iii. Eyelid crease is present or absent.
iv. Jaw-winking phenomenon is present or not.
v. Associated weakness of any extraocular
muscle.
vi. Bell’s phenomenon (up and outrolling of the
eyeball during forceful closure) is present or
absent.
3. Measurement of amount (degree) of ptosis. In
unilateral cases, difference between the vertical height
of the palpebral fissures of the two sides indicates
the degree of ptosis (Fig. 14.33). In bilateral cases it
can be determined by measuring the amount of cornea
covered by the upper lid and then subtracting 2 mm.
Depending upon its amount the ptosis is graded as
Mild 2 mm
Moderate 3 mm
Severe 4 mm
4. Assessment of levator function. It is determined
by the lid excursion caused by LPS muscle (Burke’s
method). Patient is asked to look down, and thumb of
one hand is placed firmly against the eyebrow of the
patient (to block the action of frontalis muscle) by
the examiner. Then the patient is asked to look up and
the amount of upper lid excursion is measured with a
ruler (Fig. 14.34) held in the other hand by the examiner.
Levator function is graded as follows:
Normal 15 mm
Good 8 mm or more
Fair 5-7 mm
Poor 4 mm or less
5. Special investigations. Those required in patients
with acquired ptosis are as follows:
i. Tensilon test is performed when myasthenia is
suspected. There occurs improvement of ptosis
with intravenous injection of edrophonium
(Tensilon) in myasthenia.
ii. Phenylephrine test is carried out in patients
suspected of Horner’s syndrome.
iii. Neurological investigations may be required to
find out the cause in patient with neurogenic
ptosis.
6. Photographic record of the patient should be
maintained for comparison. Photographs should be
taken in primary position as well as in up and down
gazes.
Treatment
I. Congenital ptosis. It almost always needs surgical
correction. In severe ptosis, surgery should be
performed at the earliest to prevent stimulus
deprivation amblyopia. However, in mild and moderate
ptosis, surgery should be delayed until the age of 3-
4 years, when accurate measurements are possible.
Congenital ptosis can be treated by any of the
following operations:
1. Fasanella-Servat operation. It is performed in
cases having mild ptosis (1.5-2mm) and good levator
function. In it, upper lid is everted and the upper tarsal
border along with its attached Muller’s muscle and
conjunctiva are resected (Fig. 14.35).
2. Levator resection. It is a very commonly performed
operation for moderate and severe grades of ptosis.
It is contraindicated in patients having severe ptosis
with poor levator function.
Amount of levator resection required: Most of the
surgeons find it out by adjusting the lid margin in
relation to cornea during operation on the table in
individual case. However, a rough estimate in different
grades of ptosis is as follows:
Moderate ptosis
Level of LPS Amount of LPS to be
Function resected
Good 16-17 mm (minimal)
Fair18-22 mm (moderate)
Poor 23-24 mm (maximum)
Severe ptosis
Fair levator 23-24 mm (maximum
function LPS resected)
Techniques. Levator muscle may be resected by either
conjunctival or skin approach.
i. Conjunctival approach (Blaskowics’ operation):
This technique is comparatively easy but not
suitable for large amount of resection. In it LPS
muscle is exposed by an incision made through
the conjunctiva near the tarsal border, after the
upper lid is doubly everted over a Desmarre’s lid
retractor (Fig. 14.36).
ii. Skin approach (Everbusch’s operation): It is a
more frequently employed technique. It allows
comparatively better exposure of the LPS muscle
through a skin incision along the line of future lid
fold (Fig. 14.37).
3. Frontalis sling operation (Brow suspension):
This is performed in patients having severe ptosis
with no levator function. In this operation, lid is
anchored to the frontalis muscle via a sling (Fig.
14.38). Fascia lata or some non-absorbable material
(e.g., supramide suture) may be used as sling.
BLEPHAROSPASM
It refers to the involuntary, sustained and forceful
closure of the eyelids.
Etiology. Blepharospasm occurs in two forms:
1. Essential (spontaneous) blepharospasm. It is a
rare idiopathic condition involving patients
between 45 and 65 years of age.
2. Reflex blepharospasm. It usually occurs due to
reflex sensory stimulation through branches of
fifth nerve, in conditions such as : phlyctenular
keratitis, interstitial keratitis, corneal foreign body,
corneal ulcers and iridocyclitis. It is also seen in
excessive stimulation of retina by dazzling light,
stimulation of facial nerve due to central causes
and in some hysterical patients.
Clinical features. Persistent epiphora may occur due
to spasmodic closure of the canaliculi which may lead
to eczema of the lower lid. Oedema of the lids is of
frequent occurrence. Spastic entropion (in elderly
people) and spastic ectropion (in children and young
adults) may develop in long-standing cases.
Blepharophimosis may result due to contraction of
the skin folds following eczema.
Treatment. In essential blepharospasm Botulinum
toxin, injected subcutaneously over the orbicularis
muscle, blocks the neuromuscular junction and
relieves the spasm. Facial denervation may be
required in severe cases. In reflex blepharospasm, the
causative disease should be treated to prevent
recurrences. Associated complications should also
be treated.
closure of the eyelids.
Etiology. Blepharospasm occurs in two forms:
1. Essential (spontaneous) blepharospasm. It is a
rare idiopathic condition involving patients
between 45 and 65 years of age.
2. Reflex blepharospasm. It usually occurs due to
reflex sensory stimulation through branches of
fifth nerve, in conditions such as : phlyctenular
keratitis, interstitial keratitis, corneal foreign body,
corneal ulcers and iridocyclitis. It is also seen in
excessive stimulation of retina by dazzling light,
stimulation of facial nerve due to central causes
and in some hysterical patients.
Clinical features. Persistent epiphora may occur due
to spasmodic closure of the canaliculi which may lead
to eczema of the lower lid. Oedema of the lids is of
frequent occurrence. Spastic entropion (in elderly
people) and spastic ectropion (in children and young
adults) may develop in long-standing cases.
Blepharophimosis may result due to contraction of
the skin folds following eczema.
Treatment. In essential blepharospasm Botulinum
toxin, injected subcutaneously over the orbicularis
muscle, blocks the neuromuscular junction and
relieves the spasm. Facial denervation may be
required in severe cases. In reflex blepharospasm, the
causative disease should be treated to prevent
recurrences. Associated complications should also
be treated.
TARSORRHAPHY
In this operation adhesions are created between a
part of the lid margins with the aim to narrow down or
almost close the palpebral aperture.
It is of two types: temporary and permanent.
1. Temporary tarsorrhaphy
Indications : (i) To protect the cornea when seventh
nerve palsy is expected to recover. (ii) To assist
healing of an indolent corneal ulcer. (iii) To assist in
healing of skin-grafts of the lids in the correct position.
Surgical techniques. This can be carried out as
median or paramedian tarsorrhaphy (Fig. 14.31).
1. Incision. For paramedian tarsorrhaphy, about 5
mm long incision site is marked on the
corresponding parts of the upper and lower lid
margins, 3-mm on either side of the midline. An
incision 2-mm deep is made in the grey line on the
marked site and the marginal epithelium is then
excised taking care not to damage the ciliary line
anteriorly and the sharp lid border posteriorly.
2. Suturing. The raw surfaces thus created on the
opposing parts of the lid margins are then sutured
with double-armed 6-0 silk sutures passed through
a rubber bolster.
2. Permanent tarsorrhaphy
Indications. (i) Established cases of VII nerve palsy
where there is no chance of recovery; and (ii)
established cases of neuroparalytic keratitis with
severe loss of corneal sensations.
Technique. It is performed at the lateral canthus to
create permanent adhesions. The eyelids are
overlapped after excising a triangular flap of skin and
orbicularis from the lower lid and corresponding
triangular tarso-conjunctival flap from the upper lid.
part of the lid margins with the aim to narrow down or
almost close the palpebral aperture.
It is of two types: temporary and permanent.
1. Temporary tarsorrhaphy
Indications : (i) To protect the cornea when seventh
nerve palsy is expected to recover. (ii) To assist
healing of an indolent corneal ulcer. (iii) To assist in
healing of skin-grafts of the lids in the correct position.
Surgical techniques. This can be carried out as
median or paramedian tarsorrhaphy (Fig. 14.31).
1. Incision. For paramedian tarsorrhaphy, about 5
mm long incision site is marked on the
corresponding parts of the upper and lower lid
margins, 3-mm on either side of the midline. An
incision 2-mm deep is made in the grey line on the
marked site and the marginal epithelium is then
excised taking care not to damage the ciliary line
anteriorly and the sharp lid border posteriorly.
2. Suturing. The raw surfaces thus created on the
opposing parts of the lid margins are then sutured
with double-armed 6-0 silk sutures passed through
a rubber bolster.
2. Permanent tarsorrhaphy
Indications. (i) Established cases of VII nerve palsy
where there is no chance of recovery; and (ii)
established cases of neuroparalytic keratitis with
severe loss of corneal sensations.
Technique. It is performed at the lateral canthus to
create permanent adhesions. The eyelids are
overlapped after excising a triangular flap of skin and
orbicularis from the lower lid and corresponding
triangular tarso-conjunctival flap from the upper lid.
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