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Major review

Congenital ptosis

Jeffrey R. SooHoo, MDa, Brett W. Davies, MDa, Felicia D. Allard, MDb,

Vikram D. Durairaj, MD, FACSa,c,*
a
Department of Ophthalmology, University of Colorado, Aurora, CO
b
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
c
Texas Oculoplastic Consultants, Austin, TX

article info abstract

Article history: Congenital blepharoptosis presents within the first year of life either in isolation or as
Received 9 November 2013 a part of many different ocular or systemic disorders. Surgical repair is challenging, and
Received in revised form recurrence necessitating more than one operation is not uncommon. Not all patients with
22 January 2014 congenital ptosis require surgery, but children with amblyopia due to astigmatic aniso-
Accepted 28 January 2014 metropia or deprivation may benefit from early surgical correction. A variety of surgical
Available online xxx procedures to correct congenital ptosis have been described. The choice of procedure de-
pends on a number of patient-specific factors, such as degree of ptosis and levator func-
Keywords: tion, as well as surgeon preference and resource availability. We review the genetics,
blepharoptosis associated syndromes, and surgical treatments of congenital ptosis.
congenital ptosis ª 2014 Elsevier Inc. All rights reserved.
ptosis
frontalis sling
eyelid surgery

1. Introduction The eyelids are primarily elevated by the contraction of the

Blepharoptosis, often abbreviated as ptosis, refers to an upper
eyelid that is positioned lower than normal, which narrows
the vertical dimension of the palpebral fissure. If present
within the first year of life, it is considered congenital (Fig. 1). It
may be either unilateral or bilateral and seen either in isola-
tion or in conjunction with other ocular or systemic condi-
tions.70 In general, congenital ptosis is non-progressive, but it
may be associated with abnormalities of visual development
and function, including amblyopia. These complications may
be minimized or avoided with early surgical correction.70
levator palpebrae superioris (LPS) muscle, innervated by the
superior branch of the third cranial nerve (CN3). The superior
branch of CN3 also innervates the superior rectus muscle.
Both LPS muscles receive innervation from a single, midline
subnucleus in the rostral CN3 nucleus. Therefore, damage to
the central subnucleus results in bilateral ptosis.
Epidemiological data regarding congenital ptosis are not
widely available. One of the largest reports comes from China,
published by Hu in 1987.44 A population study of more than
seven million people from multiple Chinese provinces, Hu’s
report provides information about a variety of genetic eye

* Corresponding author: Vikram D. Durairaj, MD, FACS, Texas Oculoplastic Consultants, Austin Medical Plaza, 3705 Medical Parkway,
Suite 120, Austin, Texas 78705.
E-mail addresses: vdurairaj@tocaustin.com, vikram.durairaj@ucdenver.edu (V.D. Durairaj).
0039-6257/$ e see front matter ª 2014 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.survophthal.2014.01.005
2 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
Fig. 1 e Congenital ptosis of the left upper eyelid.
diseases, including congenital ptosis. In that study, the prev-
alence of congenital ptosis was 0.18%. Pedigree analysis
showed that the majority of cases were sporadic, although
18.4% were inherited in an autosomal dominant fashion and
14.5% in an autosomal recessive pattern. These findings
cannot be extrapolated to other ethnic groups. In a more
recent study from a tertiary hospital in Egypt, a retrospective
review over a nine-year period found a total of 336 children
with ptosis, 69% congenital.28 Ptosis was unilateral in 65% of
cases and the left side was more commonly affected (74%).
Griepentrog et al reviewed all cases of childhood ptosis over a
40-year time period in Olmsted County, Minnesota. They
identified 107 cases of ptosis, with an incidence of 7.9 per
100,000. Of these patients, 89.7% had congenital ptosis,
although only 12% had a positive family history. The rate of
congenital ptosis was 1 in 842 births. Only 3% were bilateral,
and there was a slight predominance of left ptosis (55%).36
Many theories have been proposed regarding the patho-
genesis of congenital ptosis. Historically, congenital ptosis has
been thought of as a disorder of muscle development, but
newer theories focus on disordered muscle innervation. His-
topathologic studies have demonstrated a primary defect in
the LPS muscle with fibrosis and decreased numbers of skel-
etal muscle fibers.12,48 The exact mechanism of levator
dysgenesis is poorly understood and could vary by condition.
There are many other possible causes of congenital ptosis,
including levator disinsertion secondary to birth trauma.34 We
shall review the etiology of congenital ptosis and options for
surgical repair.
2. Genetics
In 1990, Vestal et al conducted a literature review investi-
gating the concordance of congenital ptosis in monozygotic
twins and found a heritability index of 0.75, indicating that
75% of the phenotype is attributable to genetic factors.73
These data support a transmissible genetic defect contrib-
uting to this disorder. There are a variety of genes implicated
in this process, with new loci being investigated and identified
each year.
The first gene to be identified as a locus for isolated
congenital ptosis was the PTOS1 gene.29 Engle et al studied the
DNA from 42 members of a family in which 20 members had
isolated congenital ptosis in at least one eye. Fluorescent
in-situ hybridization was performed and identified a 3 centi-
Morgan (cM) region of chromosome 1 as being responsible for
ptosis. For this gene, inheritance is autosomal dominant with
incomplete penetrance.
In 2002, McMullan et al described an X-linked dominant
form of isolated, bilateral congenital ptosis.53 The family
studied was noted to have a dominant inheritance pattern
without male-to-male transmission. Genetic linkage studies
identified the locus of interest as Xq24-q27.1.
The ZFH-4 gene was identified in 2002 after DNA analysis
of a child with bilateral congenital ptosis who was found to
have a balanced translocation of chromosomes 8 and 10.54
The mutation in chromosome 8 was found to disrupt the
ZFH-4 gene located at 8q21.12. This gene encodes a protein
with a zinc-finger homeodomain that acts as a transcription
factor. This protein has been shown to be prominently
expressed in developing muscles and nerves.47 The same gene
product is also expressed in the developing midbrain and
could affect the structure and function of the oculomotor
cranial nerve nuclei.38,57
3. Associated syndromes
Normal extraocular muscle functioning depends upon normal
innervation, which in turn requires normal cranial nerve
development. Many well-characterized congenital syndromes
display abnormal extraocular muscle innervation. Collec-
tively, these syndromes are known as congenital cranial
dysinnervation disorders (CCDDs), congenital disorders
resulting from aberrant innervation of the ocular and facial
musculature.38 Many of these syndromes have associated
ptosis. Ptosis can also be seen in association with dysfunction
of the sympathetic nervous system, as in Horner syndrome, or
with other forms of strabismus including congenital esotropia
or exotropia.39
3.1. Duane retraction syndrome
Duane retraction syndrome (DRS) is the most common of the
CCDDs. Type 1 DRS presents with limited abduction of the
affected eye as well as contraction of both the medial and
lateral rectus muscles upon attempted adduction, although
adduction is intact or only slightly limited.27 In type 2 DRS,
abduction is usually intact but adduction is impaired in the
affected eye. Type 3 DRS presents with limitations in both
abduction and adduction. The contraction of the horizontal
recti upon attempted adduction causes globe retraction,
which leads to enophthalmos and resultant ptosis. Abnormal
development of the motor neurons of the sixth cranial nerve
leads to abnormal innervation of the lateral rectus.43,57
Although the exact sequence and etiology is poorly under-
stood, other cranial nerves can aberrantly innervate the
lateral rectus.43 This condition is usually sporadic and no
single gene has been identified for DRS; however, various
candidate genes have been mapped to chromosomes
2q31 and 8q13.17,32
 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0 3

3.2. Blepharophimosis ptosis epicanthus inversus a transcription factor that has been shown to be essential to
syndrome the development of the oculomotor and trochlear nuclei in
mice and zebrafish.37 It is hypothesized that CFEOM2 results
Blepharophimosis ptosis epicanthus inversus syndrome from hypoplasia of these cranial nerve nuclei in humans.
(BPES) is an autosomal dominant disorder characterized by CFEOM3 is an autosomal dominant condition with incomplete
blepharophimosis, blepharoptosis, epicanthus inversus, and penetrance that can lead to a heterogeneous phenotype.72 In
telecanthus (Fig. 2). One can also see cicatricial ectropion of general, affected individuals display eye movement disorders
the lower eyelids. The disorder is bilateral and is divided into characterized by variable ptosis as well as restrictive oph-
two subtypes. Type 1 BPES is associated with premature thalmoplegia. The causative mutation has been mapped to
ovarian failure in female patients. BPES is due to a mutation in chromosome 16q24.2-q24.3, and mutations in KIF21 A can also
the transcription factor FOXL2, leading to production of result in CFEOM3.41,49
truncated proteins in the mesenchyme of developing eyelid
structures and maturing ovarian follicles.22 Type 2 BPES has
3.4. Marcus Gunn jaw-winking syndrome and
the same facial features without associated premature
monocular elevation deficiency
ovarian failure.
The incidence of blepharophimosis varies across studies.
Marcus Gunn jaw-winking syndrome was first described in
In a review of 336 Egyptian children with ptosis, El Essawy et al
1883. Classically, this phenomenon presents as ptosis that is
found that blepharophimosis accounted for 17%.28 A review of
increased by moving the jaw towards the affected side;
155 children from the United Kingdom found an incidence of
conversely, lid retraction occurs on jaw movement to the
4.5%,13 whereas a review of 107 children from Minnesota
contralateral side (Figs. 3 and 4). The synkinesis is the result
found only three cases of blepharophimosis (2.8%).36 These
of an aberrant connection between the motor branches of the
latter two studies more closely represent the experience of the
trigeminal nerve innervating the external pterygoid muscle
authors.
and the fibers of the superior division of the oculomotor
nerve that innervate the LPS.25 Current theory suggests that
3.3. Congenital fibrosis of the extraocular muscles injury to the involved cranial nerves in utero leads to sec-
ondary degeneration of the involved cranial nerve nuclei and
Congenital fibrosis of the extraocular muscles (CFEOM) is a the reappearance of phylogenetically primitive synkinetic
group of conditions characterized by congenital paralytic stra- movement.25,65
bismus secondary to restrictive ophthalmoplegia, often with Monocular elevation deficiency, also known as double
accompanying ptosis. CFEOM1, a bilateral, non-progressive elevator palsy, may be associated with congenital ptosis as
ptosis inherited in an autosomal dominant fashion, has been well as Marcus Gunn jaw-winking syndrome.79 One must be
mapped to chromosome 12p11.2-q12, which codes for the careful to differentiate true ptosis from a pseudoptosis due to
kinesin KIF21 A, a protein highly expressed in neurons.50 the associated hypotropia. In one review, 25% of patients with
Neurons use kinesin and dynein microtubule transport pro- Marcus Gunn jaw-winking syndrome had concurrent monoc-
teins to move essential cellular components along the length of ular elevation deficiency.66
axons and dendrites. Lack of this motor protein is associated
with absence of the superior division of the oculomotor nerve
and corresponding midbrain motor neurons, leading to pro-
found atrophy of the LPS and superior rectus.30 This loss of
innervation leads to a fixed downward gaze with the head
classically held in a chin-up position. In addition, horizontal
strabismus is common as well as convergent or divergent gaze
with attempted upgaze.72 CFEOM2 is autosomal recessive and
affected individuals have bilateral ptosis and restrictive oph-
thalmoplegia with exotropia. CFEOM2 has been mapped to the
ARIX/PHOX2A gene located on chromosome 11q13.1.74 ARIX is

Fig. 2 e Blepharophimosis syndrome demonstrating Fig. 3 e Marcus Gunn jaw winking syndrome. Note ptosis
ptosis, telecanthus, and epicanthus inversus. of right eyelid with mouth closed.
4 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
Fig. 4 e Marcus Gunn jaw winking syndrome. Note
elevation of right eyelid with jaw movement.
3.5. Horner syndrome
Horner syndrome results from a disruption in the sympathetic
nervous system, producing the classic triad of ipsilateral
ptosis, miosis, and anhidrosis. The ptosis seen in Horner
syndrome is mild, typically on the order of 1e2 mm due to
dysfunction of the sympathetically innvervated Müller’s
muscle. Congenital Horner syndrome can also lead to heter-
ochromia with a lighter colored iris on the ipsilateral side.58
Congenital Horner syndrome is most commonly idiopathic
or related to brachial plexus injury during delivery, but one
must also rule out a more serious etiology such as primary
neuroblastoma.80
4. Surgical correction of congenital ptosis
Surgical repair of congenital ptosis is indicated when the
upper eyelid interferes with the visual axis causing stimulus
deprivation or induces astigmatism that is amblyogenic.
Amblyopia may also result from associated strabismus. Ptosis
can induce psychological distress, and surgery to improve
cosmesis may be beneficial in these cases. The type of surgery
to perform depends upon the amount of ptosis and levator
function, and the optimal timing of surgery varies from case to
case. Some advocate waiting until the patient is about 4 years
of age or older so that the child can more reliably cooperate
with the physical exam and thereby improve the surgical
outcome and so that use of autogenous fascia lata is an op-
tion.39 When amblyopia is present, early surgical correction is
suggested.9,62 Consultation with a pediatric ophthalmologist
is invaluable to identify amblyopia and discuss treatment
options. The various methods of surgical correction are dis-
cussed in this section.
4.1. Frontalis sling
One common surgical technique to repair congenital ptosis
with poor levator function (<3 mm of eyelid elevation) is the
frontalis sling.28 This procedure aims to form a connection
between the frontalis muscle and the tarsus of the upper
eyelid. After sling placement, contraction of the frontalis
muscle elevates the upper eyelid, bypassing the poorly func-
tioning LPS (Figs. 5 and 6).11 Generally, this procedure is
considered safe, effective, and technically straightforward.
There are disadvantages, however, most importantly the risk
of lagophthalmos. One must also be cognizant of the cosmetic
outcome, as the procedure can lead to scarring, loss of the
eyelid crease, and poor eyelid apposition to the globe. The risk
of these complications is often related to the surgical tech-
nique and the sling material used.11
When performing a frontalis sling, one can use a single
loop or a double pentagon sling. No difference in recurrence,
function, or cosmetic result has been reported between these
two techniques.11 The incision can be made through the su-
perior eyelid crease or via a supraciliary stab incision. The
eyelid crease incision may result in better eyelid contour and
symmetry.77
Over time, various modifications to the frontalis sling have
been suggested. One modification utilizes a broader area of
fascia fixation. The theory is that the increased surface area of
connection will improve the strength of the attachment. This
procedure, described by DeMartelaere et al,26 requires the
harvesting of four autogenous fascia strips, each measuring
3e4 mm wide and 10e12 cm long. These strips are then placed
between the levator and an incision above the brow. This
technique is thought to result in good maintenance of the
eyelid crease and uniform eyelid contour.26 Another modifi-
cation involves suturing the sling to the tarsal plate. Buttanri
et al reviewed 80 surgical procedures and found a statistically
significant difference in surgical success (defined as eyelid
position maintained within 1 mm of normal eyelid position)
when suturing the silicone rod to the tarsal plate.16 Although
there was no statistical difference when comparing the type of
suture used, they did find that suturing with braided polyester
was slightly more effective than suturing with monofilament
polypropylene.
A number of materials have been used to create the sling
between the frontalis muscle and the tarsus, including autog-
enous or banked fascia lata and alloplastic materials that
include chromic gut, collagen, polypropylene, silicone,
Fig. 5 e Intraoperative photo of frontalis sling with silicone
rods being externalized just superior to the eyebrow.
 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
Fig. 6 e Intraoperative photo of silicone rods being
tightened at the central eyebrow incision.
stainless steel, silk, nylon monofilament, polyester, and poly-
tetrafluoroethylene (PTFE).11 Some suggest that autogenous
fascia lata is the material of choice for the creation of a frontalis
sling.70,75 The Crawford technique, originally described in 1956
and still popular today, uses fascia lata for the sling material.21
Autogenous fascia lata slings have the lowest incidence of
infection and extrusion, although resorption of the grafts may
limit long-term success.75 The child must be at least 3 years of
age in order to have adequate leg length to provide suitable
fascia lata. When autogenous fascia lata is not available (e.g., in
children younger than 3 years of age), banked fascia lata is also
an option, although not as effective.75 Silicone rods and slings
have often been used as suspension materials because of their
combination of strength (allowing for eyelid elevation) and
elasticity (allowing for full eyelid closure).20,35 Implant removal
may be required because of extrusion or infection regardless of
the type of sling material used.
Mersilene (Ethicon, Blue Ash, Ohio) suture is a non-
absorbable polyester fiber mesh knitted suture material that
is both flexible as well as porous, which allows for tissue
ingrowth. One study of 32 patients, however, reported a sig-
nificant re-operation rate of 12.5% and soft tissue complica-
tions in 20%.55 Porous PTFE (Gore-Tex; Gore Medical, Flagstaff,
Arizona) is a synthetic porous fluoropolymer that is best
known for its use in the manufacture of water-resistant
outerwear. The material has also seen application in a vari-
ety of medical specialties, however, including use as suture
material when performing a frontalis sling. A large review of
164 frontalis slings reported a trend towards a low incidence
of recurrent ptosis when using the Gore-Tex suture, although
the results did not reach statistical significance.11 Nylon
monofilament is readily available and easy to use, but it has a
high rate of recurrent ptosis.75
The dynamic frontalis muscle flap, a modified procedure
that is based on similar principles as the traditional frontalis
sling, requires the creation and elevation of a frontalis muscle
flap, which is then draped over a pulley created near the
insertion of the orbital septum at the superior orbital rim and
attached to the tarsus with permanent sutures. This redirects
the action of the frontalis muscle to the tarsus, leading to
improved eyelid elevation with frontalis muscle contraction
and eliminating the need for the alloplastic or autologous
5
materials used in a traditional frontalis sling.69 It also leads to
less ptosis with upgaze and less lid lag with downgaze, elim-
inates the brow incision, and preserves eyelid contour. Com-
plications of this procedure include transient postoperative
forehead anesthesia, overcorrection, and lagophthalmos.
An additional modification to the frontalis flap procedure
was introduced in an attempt to decrease the incidence of
early postoperative lagophthalmos: the forked frontalis mus-
cle aponeurosis (FFMA).81 This involves making a vertical
incision about 5e8 mm in length in the frontalis muscle
aponeurosis to form a forked flap of aponeurosis. Then, the
central area of the upper rim of tarsus is sutured to the upper
point of the longitudinal incision on the FFMA and the lateral
edges of the tarsus are sutured to the two forked ends of the
aponeurosis. This technique is thought to provide at least
three key advantages when compared with the original
frontalis flap procedure: elimination of the skin incision in
the lower rim of the eyebrow, elimination of the incision in the
frontalis muscle, and elimination of dissection under the
frontalis muscle.81
Borman et al described a similar technique to the FFMA,
also in an attempt to decrease postoperative lagophthalmos:
double-breasted orbicularis oculi muscle flaps.14 Two orbicu-
laris oculi muscle flaps are created, one superiorly and one
inferiorly. The superior flap is then elevated and sutured to
the tarsal plate and the lower flap is sutured over the upper
flap in order to achieve a secure closure.
Because frontalis suspension is often achieved with allo-
plastic materials, this operation has a unique set of compli-
cations. Implantation of a foreign material may lead to
inflammation, infection, and extrusion. In their review of 110
eyelids undergoing frontalis sling with silicone rods, Morris
et al reported complications in 11 (9%).59 Included in this list
were two cases of silicone rod abscess (1.8%). Infections have
been reported due to normal skin flora, atypical mycobacteria,
and Candida species.24 Treatment usually involves systemic
antibiotics, and occasionally sling explantation is required.
4.2. Levator resection and advancement
Resection and advancement of the levator aponeurosis
through an external incision, or external levator advancement
(ELA), can be used for correction of ptosis with good levator
function (5 mm). Exposure is obtained through a superior
eyelid crease incision and the dissection is carried down to the
levator aponeurosis. The levator aponeurosis is then folded or
excised and reattached to the anterior surface of the tarsus
with non-absorbable sutures (Figs. 7 and 8). This approach has
many advantages, including the ability to customize the
amount of eyelid elevation intraoperatively and, if the sur-
geon desires, preservation of Müller’s muscle and Whitnall’s
ligament.45 The use of a smaller skin incision and therefore
less local anesthetic can lead to good cosmetic results with
less surgical time and rapid recovery.6 Levator resection may
also reduce the need for additional surgery. Skaat et al
reviewed reports of 162 patients who underwent surgical
correction for congenital ptosis. The procedures reviewed
were levator resection (47%), frontalis suspension (46%), and
Fasanella-Servat procedure (7%). Re-operation rates were
10.4%, 29.3%, and 20%, respectively.71
6 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
Fig. 7 e Intraoperative photo of an external levator
advancement demonstrating the suture being passed
through the anterior surface of the tarsus.
Park et al performed a retrospective review of 130 patients
with poor levator function who underwent either levator
resection or direct frontalis muscle transfer without autoge-
nous fascia lata.64 All patients had been diagnosed with
congenital ptosis and levator function in all patients was be-
tween 2 mm and 4 mm. Although there was a trend towards
less residual ptosis with the frontalis transfer, the results did
not reach statistical significance. Whitehouse et al reported
satisfactory results using this technique in 30 surgeries for
congenital ptosis in which the patient had a minimum of
4 mm of levator function.76 They noted a 16.7% under-
correction or recurrence rate, requiring reoperation in 6.7% of
cases. Patients who underwent levator resection had a lower
risk of developing amblyopia, although this is likely in part
due to the lesser degree of preoperative ptosis as well as the
Fig. 8 e Intraoperative photo of an external levator
advancement demonstrating the suture securing the
levator aponeurosis.
success of the procedure itself. Press et al have reported good
results using maximal levator resection in patients with le-
vator function less than 2 mm.67 The authors suggest that the
primary advantage to this is the lack of an implant, which may
reduce the risk of infection.
When performing a levator resection for congenital ptosis,
there are several methods the surgeon can use to determine
the amount of levator to resect. The first involves placing the
eyelid at the desired position, then removing the redundant
tissue.40 Although straightforward, results may be unpre-
dictable as a result of the effect of anesthesia on muscle tone
and gaze position. Another technique involves preoperative
determination based on the severity of ptosis and amount of
levator function. Complex algorithms have been published by
Beard8 and Finsterer,34 but simple formulas have also been
described, such as the recommendation to resect 4 mm of
levator for every 1 mm of ptosis.19
Even in the setting of poor levator function and severe
unilateral ptosis, some surgeons will perform a super-
maximum levator resection instead of a frontalis suspension
procedure. Epstein and Putterman described the procedure for
patients with unilateral congenital ptosis, which involves
removing 30 mm or more of levator with or without a tarsec-
tomy.31 They found cosmetically acceptable results in 75% of
cases, compared with a rate of 50% using frontalis suspension.
4.3. Whitnall sling
The Whitnall sling procedure is a technique that can be used
for correction of moderate to severe ptosis (3e5 mm levator
function). Whitnall’s ligament converts the action of the LPS
from a horizontal to vertical plane and provides support for
the upper eyelid.1 The Whitnall sling procedure requires
resection of the levator aponeurosis up to the point of Whit-
nall’s ligament. Whitnall’s ligament and the underlying leva-
tor muscle are subsequently sutured to the superior portion of
the tarsal plate using either absorbable or non-absorbable
sutures.3 This procedure can be combined with superior tar-
sectomy to provide additional eyelid elevation.42 Care must be
taken to avoid mistaking the lower-positioned transverse
ligament (LPTL) for Whitnall’s ligament. Accidental fixation of
the LPTL to the tarsus results in insufficient eyelid elevation.46
One must also be sure not to advance Whitnall’s ligament
too far down the tarsal plate and cause eyelid eversion.
Undercorrection is a common complication that increases in
frequency over time postoperatively. One case series review-
ing 69 Whitnall slings reported a reoperation rate for recurrent
ptosis of 30.7%.3 The high incidence of late undercorrection
seen in cases corrected with Whitnall sling alone have led to
the suggestion that the procedure should often be combined
with tarsectomy.42 A 5 mm superior tarsectomy may offer
1e1.5 mm of additional eyelid elevation.
4.4. Fasanella-Servat procedure
Fasanella and Servat first introduced their procedure for
correction of mild ptosis in 1961.33 As described, the technique
involves removal of up to 3 mm of superior tarsus, conjunc-
tiva, Müller’s muscle, and levator. Over the years numerous
variations in surgical technique have been described, but it is
 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
generally agreed that it should be reserved for cases with good
levator function and less than 3 mm of ptosis. The Fasanella-
Servat technique has only been used sparingly in the correc-
tion of congenital ptosis. There are several explanations for
this. First, congenital ptosis often results in poor levator
function, which limits the role for this procedure. Second,
many surgeons prefer to avoid removing healthy tarsus given
its functional role in eyelid stability and, when necessary, its
value in eyelid reconstruction.
Several studies have looked at the success of the Fasanella-
Servat procedure in congenital ptosis. Berry-Brincat et al
reviewed 155 cases of congenital ptosis.13 Ten percent of the
cases were repaired using the Fasanella-Servat technique. The
overall success rate for all surgical techniques was 71% with a
20% reoperation rate. In the Fasanella-Servat group specif-
ically, there was a poor outcome in 3 of the 15 cases and a 20%
reoperation rate. No child in the Fasanella-Servat group
developed amblyopia. Pang et al reviewed 169 cases of ptosis
repair using Fasanella-Servat.63 Of these cases, 18 were clas-
sified as congenital ptosis. The overall success rate was 89.5%,
but congenital cases had the lowest success rate of all studied
subgroups (76.4%). Complications of the Fasanella-Servat
procedure include dermatochalasis, duplicate eyelid creases,
eyelid contour abnormalities, hematoma, undercorrection,
overcorrection, wound dehiscence, pyogenic granulomas, and
bleeding.63
4.5. Müller’s muscle-conjunctival resection
Müller’s muscle is an involuntary, sympathetically innervated
smooth muscle that originates from underneath the levator
aponeurosis just distal to Whitnall’s ligament. It attaches to
the superior tarsal border and is responsible for an estimated
2e3 mm of eyelid elevation.56 Müller’s muscle-conjunctival
resection (MMCR) is traditionally recommended for patients
who display resolution of ptosis after instillation of topical
phenylephrine, the sympathomimetic properties of which
stimulate the contraction of Müller’s muscle. Recent studies
suggest that even in patients that do not respond to phenyl-
ephrine, MMCR can still be successful with a low complication
rate.5 As originally described, MMCR involves removing con-
junctiva and Müller’s muscle just superior to the tarsus using a
posterior eyelid approach.68 The advantage of this technique
over the Fasanella-Servat surgery is that the tarsus is spared.
Typically, 6.5e9.5 mm of conjunctiva and muscle are resected,
depending on the clinical response to phenylephrine. A unique
potential complication is the risk of corneal abrasion or ul-
ceration from exposed suture on the palpebral conjunctiva.
The tarsal fixation sutures should be of partial thickness to
avoid this undesirable outcome. Some critics also argue that
the removal of normal adjacent structures such as goblet cells
and accessory lacrimal glands of Krause and Wolfring could
lead to increasing dryness of the ocular surface.4 Although this
raises a legitimate concern, it has never been objectively
proven. In fact, Dailey et al showed that there was no signifi-
cant decrease in tear production after MMCR.23
Unlike other techniques, one cannot adjust eyelid height
during MMCR. The amount of tissue resection is decided
preoperatively. Various ratios of muscle resection to eyelid
elevation are described. Mercandetti reports a ratio of 1 mm of
7
resection for each 0.32 mm of desired eyelid elevation.56
Because this procedure’s success is dependent upon the
function of both the levator and Müller’s muscle, it should
only be used in patients with mild ptosis. Although offering
the advantage of no external scarring, it provides only
1e2 mm of eyelid elevation and as such is rarely appropriate
for correction of congenital ptosis. One review of eight pa-
tients who underwent MMCR for congenital ptosis showed
good outcomes, although all patients had levator function of
at least 12 mm. The upper eyelid marginal reflex distance
improved an average of 2.38 mm, which resulted in adequate
correction of ptosis.51 MMCR is a reasonable consideration for
patients with mild ptosis, good levator function, and who
demonstrate an appropriate response to topical phenyleph-
rine during the preoperative evaluation.
4.6. Special considerations
Surgical repair of blepharophimosis often involves several
surgical procedures, including ptosis repair and medial epi-
canthoplasty. The type of ptosis repair depends on amount of
ptosis and levator function, and options for the medial can-
thoplasty include Y-V flaps, the Mustardé technique,60 and a
5-flap technique.2 The repair of these eyelids is complex, and
the timing of surgical repair is a source of debate. These pro-
cedures can be staged or combined into a single operation.61
This decision depends on a number of patient-specific fac-
tors and should be addressed on an individual basis.
A controversial approach proposed by Beard in 1965 for the
treatment of severe unilateral ptosis involves performing le-
vator ablation on the normal eyelid, creating severe bilateral
ptosis.7 Both sides are then repaired with frontalis suspen-
sion. The goal is to create more symmetry between the two
eyelids. This technique has been heavily criticized, however,
for taking a normally functioning eyelid and damaging it for
the sake of symmetry. A similar approach is advocated for
correction of ptosis in patients with Marcus Gunn jaw wink.15
The same authors recommend a levator resection for cases
with mild winking and ptosis, but when the ptosis or winking
is more severe, they argue for bilateral levator disinsertion
followed by frontalis suspension.
After repair of unilateral ptosis, the contralateral eyelid will
occasionally become ptotic secondary to Hering’s law. Her-
ing’s law of equal innervation postulates that ocular yoke
muscles receive equal innervation. More specifically, when
one eyelid is ptotic, the brain increases innervation to both
LPS muscles in an attempt to clear the visual axis. The
increased innervation to the contralateral eyelid can result in
pseudoretraction. After repair of unilateral ptosis, the inner-
vation to the LPS is decreased and one may observe post-
operative lowering of the contralateral eyelid.6
5. Complications
In addition to the specific complications mentioned earlier for
each procedure, there are a number common to all surgical
treatments for ptosis. Patients and families should be coun-
seled to expect temporary lagophthalmos after surgery. The
length of time depends on the type of correction, typically
8 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
lasting longest after frontalis suspension and shortest after
MMCR. It is important to stress aggressive lubrication of the
cornea during this time. The most common complication is
overcorrection or undercorrection of ptosis. Undercorrection
results in residual ptosis and, depending on the position of the
upper eyelid, may require reoperation. Overcorrection can
lead to permanent lagophthalmos, resulting in exposure ker-
atopathy. Again, treatment involves aggressive lubrication,
and may require reoperation to lower the eyelid. Other out-
comes that should be discussed with the patient and family
include eyelid asymmetry, late recurrence of ptosis, infection,
and scar formation.
Reported success rates of ptosis repair vary between
studies. In a study of 239 patients undergoing frontalis sus-
pension with autologous fascia lata, Yoon and Lee found a
functional success rate of 94% with a minimum of 6 months
follow-up.78 Cosmetic outcomes were not quite as favorable,
with good outcomes of 85.4%, 65.7%, and 66.9% for eyelid
contour, symmetry, and eyelid crease, respectively. In their
retrospective review of frontalis suspension using silicone
rods, Morris et al experienced 11 complications in 110 cases
(9%).59 These complications included four cases of exposure
keratopathy, two abscesses, one corneal ulcer, and four cases
of early reoperation. They also reported a long-term reopera-
tion rate of 9%, all for undercorrection.
In one of the largest studies looking at outcomes of levator
advancement, McCulley et al reviewed 828 patients over a
9-year period.52 Although this study looked only at adult
patients, the results are worth noting. They found a reoper-
ation rate of 8.7%, but noted that an additional 14% of pa-
tients had results that did not have the desired outcome but
declined reoperation. They also found that patients who
presented with bilateral ptosis or severe ptosis, defined as
greater than 4 mm of ptosis, had a statistically significant
increased risk of undercorrection. This is an important
observation, as congenital ptosis can often be bilateral and/or
severe. Their rate of reoperation after levator advancement
was comparable to that found by Skaat et al, who reviewed
the procedure in congenital ptosis.71 Their reoperation rate
after levator advancement was 10.4%, which was better than
both frontalis suspension (29.3%) and the Fasanella-Servat
procedure (20%).
In a retrospective comparison between external levator
advancement and MMCR, Ben Simon et al reviewed the out-
comes for 159 adult patients.10 They found a reoperation rate
of 18% in the ELA group but only 3% in the MMCR group. They
also noted that patients undergoing ELA had significantly
more ptosis preoperatively, which may contribute to the
higher reoperation rate. Other complications included
lagophthalmos (3.6%), overcorrection (1.4%), and pyogenic
granuloma (<1%).
6. Current recommendations
The choice of surgical procedure depends upon a number of
factors, including surgeon preference and experience as well
as resource availability. The ideal ptosis repair combines
longevity with good functional and cosmetic outcome while
minimizing complication and revision rates. The main
objective criteria that should be used when deciding upon
surgery is the degree of ptosis and levator function. Correction
of severe unilateral ptosis with poor levator function (<3 mm
eyelid elevation) is certainly a challenge. The surgeon must
balance the desired amount of eyelid elevation with the pre-
vention of lagophthalmos. Other considerations include
eyelid crease location, symmetry, and eyelid contour. Some
authors suggest bilateral operations for severe unilateral
ptosis to achieve more symmetric results.18 Ptosis with poor
levator function is best treated with a frontalis sling, Whitnall
sling (with or without superior tarsectomy), or dynamic
frontalis muscle flap.
With good levator function (>5 mm eyelid elevation), the
surgeon has more options. In addition to the procedures listed
here, one can consider ELA, or rarely, MMCR, if the amount of
ptosis is mild and only requires 1e2 mm of eyelid elevation.
Regardless of which procedure is used, it is important to
explain the risks of surgery and possible postoperative com-
plications to the patient and their family.
7. Conclusion
Congenital ptosis, either as an isolated condition or found in
association with other ocular and systemic abnormalities, can
cause profound functional and social impairment if not cor-
rected. Many genes and proteins have been implicated in the
etiology of congenital ptosis and more are likely to be identi-
fied. Surgical repair is indicated when the upper eyelid ob-
structs the visual axis or induces significant astigmatism,
which can result in amblyopia. A variety of techniques and
materials can be used to achieve the goal of eyelid elevation
and clearance of the visual axis. The choice of repair is
dependent upon the degree of ptosis, the degree of levator
function, and surgeon experience and preference. The most
common procedure, the frontalis sling, can be performed
using a variety of materials. More than one operation is often
required to correct congenital ptosis. Despite the many op-
tions available, repair of congenital ptosis is often challenging
for even the most experienced surgeons.
8. Literature search
The literature search was performed using a Medline search
from 1946 through September 2013 for the term congenital
ptosis. EMBASE was searched using the same search term.
Articles were reviewed and included if the information
therein was pertinent to the epidemiology, pathogenesis, or
treatment and surgical correction of this condition. Most pa-
pers reviewed were written in English, although select English
abstracts for articles written in another language were
reviewed and included if appropriate. Reference lists for arti-
cles were also reviewed for other publications of significance.
Articles were excluded if their discussion was beyond the
scope of the present review or did not add significant new
information. Original articles, review articles, and case series
were included.
 s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 4 ) 1 e1 0
9. Disclosure
The authors have no conflicts of interest to report.
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