Download Ptosis In Children Ptosis In Children

HEALTH CARE PROFESSIONALS
DELIVERING EYE CARE
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Rarely ptosis in the newborn can result from a third cranial nerve palsy or
damage to the sympathetic nerves going into the orbit. A third nerve palsy
may occur with birth trauma and generally presents with a complete ptosis
as well as an eye that is turned down and out. The pupil is also dilated.
Damage of the sympathetic nerve leads to Horner’s Syndrome (ptosis,
anhidrosis, miosis, iris heterochromatia and apparent enophthalmos).
Another neurogenic type of ptosis is referred to as “Jaw-wink ptosis” (or
synkinetic ptosis). In this situation, when the child sucks on a bottle, thumb
or chews food, the droopy lid f licks open. [Figure 2a and 2b] Essentially a
○
Neurogenic Ptosis
○
○
The commonest cause of ptosis in the child is due to a localized dystrophy
of the muscle tissue. [Figure 1] Histopathologic studies of the levator muscle
have demonstrated fibrous tissue where striated muscle would be expected.
The more fibrous tissue present, the poorer the levator function. The
process may be unilateral or bilateral. The lid may have good function or
poor function depending upon how much fibrous tissue is in the muscle.
○
Myogenic Ptosis
○
○
○
Aponeurotic disinsertion can occasionally occur in the child as a result of
trauma from forceps or trauma through the birth canal. The lid is low but
generally has excellent levator function as well as a defined lid crease.
○
○
○
○
○
○
○
○
○
Aponeurotic Ptosis
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Figure 1 – Ptosis blocking visual axis.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Congenital ptosis, may be divided into
Aponeurotic, Myogenic, Neurogenic,
Neuromyogenic and/or Mechanical causes
[Table 1]. The commonest cause of ptosis
in the adult is a result of a weakening or
dehiscence of the levator aponeurosis,
which connects the levator muscle to the
tarsal plate of the lid. Congenital ptosis on
the other hand is most commonly due to a
developmental problem within the muscle
itself (i.e. myogenic).
○
What Causes Congenital Ptosis?
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Ptosis (pronounced “toe-sis”) refers to a
drooping of the upper eyelid. The lid may
droop only slightly or it may droop enough
to partially or completely cover the pupil,
restricting or obscuring vision. [Figure 1]
Ptosis may be inherited, may affect one or
both eyelids, can be present at birth or
occur later in life. Ptosis which is present
at birth is called Congenital Ptosis. If the
ptosis develops with age it is referred to as
Acquired Ptosis. Although both congenital
and acquired ptosis may significantly affect
cosmesis and interfere with visual function,
congenital ptosis is of particular importance
as it may lead to serious problems with
visual system development and maturation,
proper ocular alignment as well as psychosocial development.
○
○
○
○
Professor of Ophthalmology
University of Ottawa
Ottawa, ON, Canada
○
M.D., F.R.C.S.(C), F.A.C.S.
○
by
David R. Jordan
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Ptosis In Children
○
Issue 4
○
Volume 6
○
○
○
○
○
○
○
In
A QUARTERLY REPORT FOR
1
· 3rd nerve palsy
· Horner’s syndrome
· Jaw-wink ptosis
Neuromyogenic
· myasthenia
Mechanical
· lid tumors
Pseudo-ptosis
·
·
·
·
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Figure 2a – Ptosis of childs left upper lid.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
¯
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
¯
○
○
○
○
Figure 2b – Child opens her mouth or chews and ptotic eyelid flicks open.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
microphthalmic eye, anophthalmos, phthisis bulbi
hypotropia
contralateral lid retraction
contralateral proptosis
○
Age at time of onset should be elicited from
the patient and/or the parents; old
photographs may be valuable for this
purpose. Di fferentiating congenital
(present at bir th) from acquired is
extremely important since management of
the two disorders can be quite different. A
neonatal medical history should include
information on prematurity, trauma at
○
○
Neurogenic
○
What Features Are Important
During History Taking?
○
○
○
○
○
○
○
○
○
· localized dystrophy (most common)
· other muscle diseases (myotonic dystrophy)
○
Myogenic
○
○
· disinsertion of aponeurosis
○
○
Aponeurotic
○
A distinction must also be made between
“pseudo-ptosis” and “tr ue ptosis” i n
determining the pathogenesis of abnormal
eyelid position in patients with suspected
congenital ptosis. Pseudo-ptosis, “an
apparent drooping of the upper eyelid
secondary to abnormalities involving the
globe or ocular adnexa” results from
ipsilateral enophthalmos (microphthalmic
eye), contralateral exopthalmos, hypotropia
(vertical strabismus), and contralateral eye
retraction.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Congenital Ptosis: Classification
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Pseudo-ptosis
○
○
○
○
○
○
Large upper lid lesions such as capillary
hemangiomas or neurofibromas may act as
a weight on the lid, causing it to droop.
○
○
Mechanical Ptosis
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
TABLE 1
○
○
birth, other congenital anomalies and, significant systemic illness. The
clinician should inquire about any prior associated ocular abnormalities
such as amblyopia, strabismus and the jaw-winking phenomenon when
eating, chewing, etc. A family history of congenital ptosis is also important
in making the diagnosis.
○
Myasthenia Gravis is a rare condition in
children that is a result of a problem at the
ner ve-muscle junc tion. The neurotransm itter (acet ylcholi ne) wh ich is
released from the ner ve terminal and
stimulates the muscle to contract is being
blocked from activating the muscle. The key
to this diagnosis is fatigue with time. That
is, the lid(s) appear at a normal height in
the morning but droops as the day goes on.
Treatment is nonsurgical and requires
medication to increase the level of
acetylcholine at the neuromuscular
junction.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Neuro-myogenic Ptosis
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
branch of the fifth cranial nerve going to
the pter ygoid muscles (used during
swal lowi ng, suck ing, etc.) has been
misdirected during embryologic development and travels to the lid where it
innervates the levator muscle. When these
sucking or chewing muscles are activated
the lid “f licks” open and gives the
appearance of a “winking” eyelid.
2
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Figure 3a – Ptosis of patients right upper lid pre-operative.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
¯
○
○
Children with ptosis will have a droop of
one or both lids and may appear sleepy
looking. They will often tilt their heads back
into a chin-up position to see, or raise their
eyebrows in an effort to raise their lids
above the visual axis. When the lid droop is
bilateral and severe, the developing infant
may bump into things as a result of the
droopy lids causing a visual restriction. This
may also lead to a delay in development of
the child.
○
○
○
○
○
○
○
○
What Are The Signs And
Symptoms Of Congenital Ptosis?
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
The most common surgical procedure for congenital ptosis involves an
advancement of the levator aponeurosis and/or levator muscle. [Figure 3a
and 3b]
○
○
○
How Is Congenital Ptosis Treated?
The treatment of congenital ptosis is surgical in the majority (98%) of
patients. Indication for surgery include visual axis occlusion by the ptotic
lid, an abnormal head position, developmental delay and an unsatisfactory
facial appearance. Surgery is generally performed during the pre-school
years (age 3-5) although earlier intervention may be required if visual
impairment (causing amblyopia) is significant. When deciding upon
surgery, the surgeon must consider many factors including the extent of
the patient’s ptosis, the maximum upper lid excision and the pathogenesis
of the disorder. For surgical cases it is the levator function that determines
what type of surgery is to be carried out.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Each child with ptosis requires a thorough
ophthalmic exam. A visual acuity check,
ocular motility assessment and refraction
The levator muscle function (downgaze to upgaze) is important to
determine during the exam as it will determine what type of surgery is
required.
○
○
Examination Of
The Child With Ptosis:
(looking for astigmatism) are important. Children should also be examined
for iris heterochromia (congenital Horner’s syndrome), Jaw-winking,
dilated pupil (3rd nerve) as well as looking at the lid anatomy for signs of
capillary hemangioma, neurofibroma or other congenital anomalies for
example the blepharophimosis syndrome (epicanthus inversus, telecanthus
and phimosis).
○
○
In addition, a number of children with
ptosis also have astigmatism on the same
side. If the astigmatism is not corrected, the
blurred image created can also lead to
amblyopia.
○
○
○
Ptosis can also hide a “misalignment or
crossing of the eyes” which can also cause
amblyopia. If the misalignment is not treated
early in childhood (<6 years) the child’s
vision will be permanently reduced as a
result.
Figure 3b – Post operative photo, equal lid heights.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
A child’s visual system develops and
matures throughout the first 6 years of life.
In the first few months of life the visual
system is particularly fragile. If the eyelid
covers part of the visual axis, the child’s
visual development can be turned off
resulting in poor vision and a condition
known as “amblyopia”. If the amblyopia is
not corrected early, the poor vision becomes
permanent and the vision will remain poor.
If the amblyopia is recognized early (i.e. less
than 6 years) it may be corrected with eye
patching and in some cases glasses. The
earlier the amblyopia is picked up, the easier
it is to correct. Similarly, the longer it is left
the harder it becomes to correct. Beyond 6
years the visual system is locked in and
amblyopia can not be corrected (by glasses,
laser, etc.).
○
○
○
○
What Problems Can Occur As A
Result Of Childhood Ptosis?
○
○
○
○
○
Figure 4b – Post frontalis sling surgery.
○
○
○
○
○
○
○
○
○
○
○
Bleeding and infection are possible but extremely rare. Under or over
correction of the lid height can occasionally occur. If the lid height anomaly
is obvious, a suture adjustment may be required in the first to two weeks. A
temporary inability to fully close the eye after ptosis surgery is common and
generally resolves in most over a few weeks. The eyelids do not remain “stuck
open”. During this time period when the lids are not fully closing it is
important to use lubricating drops and ointment to keep the cornea moist.
○
What Are The Risks Of Ptosis Surgery?
○
○
Summary
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Ptosis in children can be successfully treated with surgery to improve visual
function as well as improve cosmetic appearance. The drooping lid can affect
visual development in an infant and if the lid is blocking part of the visual
axis, an ophthalmic assessment is extremely important to avoid the
development of amblyopia.
○
The commonest type of ptosis in the child is due to a “muscle development
problem”, the muscle simply has more fibrous and fatty tissue than normal.
As a result it is weak. It does not improve with time. The only type of ptosis
that might improve with time is a ptosis secondary to birth canal trauma or
forceps. In these cases the lid may be bruised or swollen.
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
○
Figure 4a – Severe bilateral ptosis.
○
In those children with very poor levator
muscle function (less than 3 mm), there is
no point in advancing or resecting the
levator, the lid will remain low. In these
children the lid must be raised by attaching
a suture, silicone rod or facial strip
(autogenous or banked fascia) from the
eyelid to the frontalis muscle at the level of
the eyebrows. This procedure is termed a
“frontalis sling”. This procedure works very
well when the ptosis is bilateral. [Figure 4a
and 4b] When a poor function unilateral
ptosis is present a difficult question arises:
should one carry out a unilateral frontalis
sling, leaving one lid to be raised by the
normal levator muscle while the other
ptotic lid is being raised by a much
different mechanism, the frontalis
muscle? Or, should one carry out a
bilateral frontalis sling? The best surgical
approach to use in patients with severe
unilater al congenital ptosis remai ns
controversial. Some surgeons advocate
unilateral frontalis sling procedures to avoid
surger y on the anatom ically nor mal
contralateral upper lid. However, patients
undergoing unilateral frontalis suspension
may prefer to see with the normal nonptotic
eye; these patients may fail to use the
frontalis muscle that opens the ptotic eye,
thus negating the desired effect of surgery.
Additionally, unilateral frontalis sling
surgery may produce lagophthalmos and lid
lag on the operated side, with cosmetically
unacceptable palpebral fissure asymmetry
on downgaze. Consequently, many
surgeons, prefer an alternative approach:
bilateral frontalis suspension with
weakening of the normal levator muscle by
partial excision (basically converting the
normal eyelid to a poor function ptotic
eyelid). This procedure usually achieves
satisfactory eyelid elevation and palpebral
fissure symmetry in primary position and
downgaze. Bilateral frontalis suspension
may also be the procedure of choice in
patients with severe congenital ptosis and
the jaw-winking phenomenon. In these
patients, partial levator excision on the
ptotic winking side, levator excision on the
nor mal side, and bilateral frontalis
suspension are combined to alleviate
abnor mal upper lid movement with
masseter and pterygoid contraction.
If you have any questions regarding the topics of this newsletter, or requests for future topics of “InSight”, please contact Dr. David R. Jordan
by telephone (613) 563-3800, fax (613) 563-1576 or e-mail at [email protected] · Check our Web Site for previous issues of InSight – www.drjordan.on.ca