Operative Technique in Plastic Surgery – Kevin C.Chung

critical. The ear template facilitates this process.
Costal
cartilage
harvesting
■ Harvesting rib cartilage while leaving the perichondrium behind is
safer than harvesting cartilage with perichondrium. This helps to
prevent pneumothorax and to minimize chest wall deformities.
Bolster
sutures
■ A very gentle tie is the key. Even with a gentle tie, one will see
tightness at the time of maximum swelling (48–72 h). Check
underneath the bolster suture with bright LED light for pressure sores,
especially by 2 or 3 days postoperatively.
■ The authors recommend not shampooing for 2 weeks postoperatively.
Keeping the bolster dressing clean and dry helps to prevent infection.
POSTOPERATIVE CARE
■ Periodic wound check for 2 weeks is strongly recommended to avoid
complications.
■ It is recommended to keep the wound dry for 2 weeks to prevent infection.
Shampoo may cause redness of the wound and make it difficult to differentiate
from infection.
■ The bolster sutures will be removed in 2 weeks. Until then, periodic checks for
pressure sores or tightness of the bolster sutures are important to prevent skin
necrosis.
OUTCOMES
■ If a balanced augmentation of the skin envelope and cartilage is achieved to
expand the helix, a stable outcome is likely (FIG 3).

FIG 3 • Left (A) and right (B) ears are shown 3 months
after surgery.
COMPLICATIONS
■ Incomplete correction of the helix deformities
■ Infection
■ Hematoma: Careful bolster suture helps to prevent hematoma and to stabilize
ear shape during the early wound healing process.
REFERENCES
1. Tanzer RC. The constricted (cup and lop) ear. Plast Reconstr Surg.
1975;55:406-415.
2. Farkas LG, Posnick JC, Hreczko TM. Anthropometric growth study of the ear.
Cleft Palate Craniofac J. 1992;29(4):324-329.
3. Harada T, Yamada A, Sato A, Tomita I. Analysis of the characteristics of
external ears’ curves and the development of the templates. Bull JSSD (Jpn)
2011;57(5):21-26.
4. Musgrave RH. A variation on the correction of the congenital lop ear. Plast
Reconstr Surg. 1966;37:394-398.
5. Park C. A new corrective method for the Tanzer’s group IIB constricted ear:
helical expansion using a free-floating costal cartilage. Plast Reconstr Surg.
2009;123(4):1209-1219.
6. Nagata S. Alternative surgical methods of treatment for the constricted ear.

Clin Plast Surg. 2002;29:301-315.
7. Grotting JK. Otoplasty for congenital cupped protruding ears using a
postauricular flap. Plast Reconstr Surg. 1958;22:164-167.

36
CHAPTERCryptotia
Akira Yamada and Arun K. Gosain
DEFINITION/ANATOMY
■ Cryptotia is a relatively rare congenital ear deformity. The upper portion of the
auricular cartilage is partially buried beneath the skin on the side of the head,
hiding the upper portion of the ear. Cryptotia is often associated with helix-
antihelix adhesion that results in a narrower helical arc in the upper pole.
Cryptotia is also associated with upper helix deficiency that is a manifestation
of constricted ear. Cryptotia is more frequent in Asian countries. In Japan, it
has been reported in as many as 1 in 500 births.
PATHOGENESIS
■ The cause of cryptotia is unknown. One theory is that cryptotia is the result of
an anomaly of the intrinsic transverse and oblique auricular muscles.1
PATIENT HISTORY AND PHYSICAL FINDINGS
■ The upper portion of the helix is buried underneath the skin (FIG 1). If one
pulls the existing helix, the upper portion of the helix comes out easily. On
releasing the helix, the upper helix returns to its initial position buried beneath
the temporal skin.

FIG 1 • Preoperative view of cryptotia; the upper pole of
the helix is buried underneath the scalp.
DIFFERENTIAL DIAGNOSIS
■ Helix-antihelix adhesion
■ Constricted ear
■ Microtia
NONOPERATIVE MANAGEMENT
■ If the patient presents before 1 month of age, nonsurgical correction of the
deformity with ear molding techniques may obviate the need for surgery.2
■ Ear molding techniques are most effective during the first month of life and
are unlikely to work after the 2nd month of life.
■ Helix-antihelix adhesion associated with cryptotia or helix deficiency
associated with constricted ear is less likely to resolve with molding
techniques.
SURGICAL MANAGEMENT
Preoperative Planning
■ It is important to check if the cryptotia is associated with other congenital
anomalies, such as helix-antihelix adhesion or constricted ear. Pure cryptotia
surgery will augment the skin deficiency but will not resolve helix-antihelix
adhesion or cartilage defect of the helix associated with constricted ear.
■ If cryptotia is not associated with cartilage deformities, surgery can be done in
early childhood. However, if cryptotia is associated with helix deformities, the
authors recommend delaying surgery until at least age 4 to 6 years because of

the possible need to harvest rib cartilage.
Positioning
■ Supine position with both ears prepped, including the temporal hair near the
ears
T E C H N I Q U E S
■ Marking of Z-plasty
■ Z-plasty flaps are designed along the bandlike contracture above the auricle,
while pulling outward away from head (TECH FIG 1A). The length of
triangular flap depends on the degree of buried position of helix, but in this
particular case, the tip of the triangular flap is located along the hairline
margin (TECH FIG 1B,C).
■ Usually, the length of Z-plasty limb is 1.5 to 2 cm3. The anterior limb of the
Z (A–C) is usually placed 5 to 10 mm from the ascending margin of the
helix. The posterior limb of Z-plasty (B–D) has a more obtuse angle than
does the anterior limb, and the continuous extension of the skin incision is
marked beyond the retroauricular sulcus (D–E). If more mobilization of the
skin flap is needed, D–E can be extended further down, and a back cut may
facilitate movement of the flap toward the skin deficiency behind the upper
helix (TECH FIG 2).
TECH FIG 1 • A. Triangular flap is designed along the
bandlike contracture above the auricle, while pulling
outward from head. B. The tip of the triangular flap is

located along the hairline margin. C. If more
mobilization of the skin flap is needed, the skin
incision can be extended further down. A back cut
may facilitate flap movement toward the skin
deficiency behind the upper helix.
TECH FIG 2 • The diagram shows the basic design of
the skin flaps, and the view after the wound closure.
A. Preoperative view of cryptotia; upper helix is buried
underneath the skin. B. The design of the skin incision
is marked while buries portion of the upper helix is
pulled out. C. AB lies along the line of traction. Point C
must be located slightly anterior to the ascending
margin of helix. D. CABD is a Z-plasty. The incision is
extended further (as far as point E) in accordance with
the requirement of cartilage plasty and the extent of
skin advancement. ‘de‘ curve is located posterior than
intended retroauricular sulcus. E. Triangular skin flap
‘cab’ and skin flap ‘bde’ is raised. F. Upper ear
cartilage is exposed with supra-perichondrial skin flap

dissection. G. Triangular skin flap is transposed
posteriorly, and the wound is closed primarily: this
maneuver eliminates the space for buried upper helix.
H. Upper auricle is reflected anteriorly to demonstrate
the completion of wound closure: Z-plasty plus
advancement flap creates enough skin envelope for
new helix.
■ Skin Flap Dissection and Ear Cartilage Exposure
■ Dissection of the skin flap is performed just above perichondrium to see if
any cartilage modification is needed. The temporal and mastoid skin is
widely undermined to facilitate skin transposition so as to augment the
posterior skin defect of the upper helix.
■ Skin Flap Transposition
■ After skin flap preparation and careful hemostasis, the skin flaps are
transposed toward the skin deficiency at the back of superior helix, filling
residual defects.3
■ Wound Closure
■ The skin is closed in single layer. In most cases, there is less skin tension
after transposition of skin flaps to facilitate closure.
■ Bolster Sutures
■ Bolster sutures (TECH FIG 3) are classic techniques, popularized by Tanzer
more than 50 years ago. The bolster sutures have two purposes: (a)
preventing hematoma formation and (b) to form an adhesion between the flap
and cartilage. Nagata uses bolster sutures for almost all ear reconstruction.
The key is to (a) use soft materials (eg, Xeroform gauze with additional
soaking with ample Vaseline-based ointment) and (b) tie very gently to avoid
any strain to the skin. The authors use 4-0 Prolene on a double-armed, SH

needle (Ethicon Inc., Somerville, NJ). Periodic check, especially 2 days post-
op when the swelling is maximum, is mandatory to prevent a pressure sore
underneath the bolster sutures.
TECH FIG 3 • The view after the bolster sutures are
placed.
PEARLS AND PITFALLS
Timing of
surgery
■ If you are unsure about helix deformities, delay the surgery until
4–6 years of age when a floating rib graft becomes an option.
Associated
ear
anomalies
■ Look for helix-antihelix adhesion, and helix deficiencies
associated with a constricted ear.
Design of
skin flaps
■ Mark the hairline before shaving so as not to obscure the margin of
the hairless posterior auricular skin.
Bolster
sutures
■ Tie very gently. Use soft materials for a gauze pillow such as
Xeroform soaked in Vaseline-based ointment.
Skin flap
dissection
■ Skin dissection should be at uniform thickness in a plane just
above perichondrium.

POSTOPERATIVE CARE
■ Patients/parents should be instructed to keep the wound dry for 2 weeks. Early
shampoo prior to 2 weeks may cause redness of the wound. Although this may
not indicate an infection, redness is confusing for both physicians and patients.
If bolster sutures are used, periodic check underneath the bolster is mandatory
to prevent pressure sore.
OUTCOMES
■ FIG 2 shows the immediate outcome of surgery. If surgery is performed in
early childhood, it is important to explain to the family that the child/family
may notice recurrent deformities of the helix later in childhood. Families
should understand that a second surgery may be considered should helix
deficiencies recur. Mild helix deformities can be addressed with the Musgrave
procedure. For more severe helix deformities, floating rib graft (Park) may be
indicated (see chapter on Constricted Ear).
FIG 2 • The view immediately after wound closure.
COMPLICATIONS
■ Infection: Infection can be caused by a break in proper sterile surgical
technique or dehiscence secondary to excessive tension during closure. It can
also be an untoward sequela of prior hematoma evacuation. A history of atopic
dermatitis (eczema) is a warning sign. The ear canal could also serve as a
reservoir of bacteria, especially in swimmers. Careful preparation of hairs near
the surgical site is important to prevent infection.
■ Postoperative hematoma: Hematoma is heralded by the acute onset of severe,

persistent, and often unilateral pain. If there is evidence of ongoing bleeding,
reoperation and exploration are mandatory. Meticulous postoperative dressing,
including bolster sutures, helps to prevent hematoma.
■ Skin necrosis: When raising skin flap, it is important to raise the skin flap in
the same plane and thickness to avoid irregular thickness of the skin flap.
Small blunt straight scissors may be better than popular curved scissors in this
respect. The proper plane of elevation for prominent ear is just above the
perichondrium. A thinner dissection plane may result in skin necrosis. When
using bolster sutures, it is mandatory to check for possible pressure sore
beneath the bolster regularly until sutures removal 2 weeks postoperatively.
■ Chondritis: Chondritis is a surgical emergency, which, if left untreated, can
result in permanent deformity. Therefore, prompt debridement of devitalized
tissue is necessary.
■ Late sequelae of helix deformities of ear: These are mainly caused by
unrecognized helix-antihelix adhesion or helix deficiencies associated with
cryptotia. In order to avoid these complications, it is important to recognize the
associated congenital ear deformities associated with cryptotia.
REFERENCES
1. Hirose T, Tomono T, Matsuo K, et al. Cryptotia: our classification and
treatment. Br J Plast Surg. 1985;38(3):352-360.
2. Matsuo K, Hirose T, Tomono T, et al. Nonsurgical correction of congenital
auricular deformities in the early neonate: a preliminary report. Plast Reconstr
Surg. 1984;73(1):38-51.
3. Yanai A, Tange I, Bandoh Y, et al. Our method of correcting cryptotia. Plast
Reconstr Surg. 1988;82(6):965-972.

37
CHAPTERProminent Ear
Akira Yamada and Arun K. Gosain
DEFINITION
■ Prominent ears are a common abnormality, affecting about 5% of children.
When observing other people’s faces, the first thing we pay attention to is the
facial triangle, eye–mouth–eye. The auricle is usually not the primary area of
attention. However, if the auricle is projected more than 25 mm from the head,
it tends to catch the attention of human eyes as “prominent.”
ANATOMY
■ The main components of the prominent ear are (1) loss of antihelical fold, (2)
concha scapha angle greater than 90 degrees, and (3) conchal cartilage excess
(FIG 1).
■ Understanding ear shape anatomy, aesthetics of the ear, ear shape dimensions,
and proper anatomical location of the ear is essential to achieving a natural
result for the correction of prominent ear deformity.1,2
PATHOGENESIS
■ The most common etiology of the prominent ear is effacement of the
antithetical fold. Also a deep conchal bowl (greater than 1.5 cm) frequently
contributes to the overall pathogenesis. Combination of these two is very
common.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Five parameters should be assessed systematically to ensure that all
components of the prominent ear deformity are recognized: (1)
auriculocephalic angle, (2) scapha-conchal angle, (3) relationship of the
antihelix to the helix, (4) horizontal distance from the most lateral aspect of the

helix to a point perpendicular to the skull, and (5) depth of the conchal bowl.
DIFFERENTIAL DIAGNOSIS
■ Constricted ear
■ Stahl ear
■ Microtia
FIG 1 • Preoperative view of unilateral prominent ear
(Case 2).
NONOPERATIVE MANAGEMENT
■ If the patient presents in infancy, preferably less than 1 month of age, ear
molding techniques may be indicated. Success of ear molding progressively
diminishes after the first month of life, and after age 2 months, surgery is the
only reliable option to correct the prominent ear.
SURGICAL MANAGEMENT
■ If the distance from the head to the top of helix at any point is over 25 mm, it
is considered to be a prominent ear.
■ Because the prominent ear could be the target of bullying and ridicule during
school age, the most popular timing of surgery is before the start of primary
school.
■ There seems to be a slightly different prominent ear concept among different
cultures. Generally speaking, Asian countries are more tolerant to prominent
ear, because the “big ear” is a good sign of luck and wealth.
Preoperative Planning

■ Preoperative anthropometric measurement of the auricle is extremely
important to achieve symmetry of the projection of the auricle postoperatively.
The direct measurements also help to assess the nature of prominence. For
example, if the patient has more prominence in the lower pole (25 to 30 mm), it
is likely that conchal hypertrophy exists. To assess the degree of projection, the
authors recommend measuring at least three points (eg, upper, middle, and
lower) from the head to the top of helix. An otoplasty grid provides objective
data of projection from the top to the tail of the helix (FIG 2). In case of
unilateral prominent ear, otoplasty grid taken from normal data can be used as
an intraoperative template to achieve symmetry (FIG 3).
FIG 2 • Preoperative assessment of projection (Case 1).
Prediction of the Projection Reduction
■ 20 mm distance from the mastoid to the peak of helix is acceptable projection,
especially in Asian countries. In the United States, somewhere between 15 to
20 mm is the reasonable goal to achieve. The trend of ear projection in the
patient’s family is a good indicator for the targeted height of projection.

FIG 3 • Otoplasty grid is used to assess the postop
helix-mastoid distance on the (A) left and (B) on the
right.
Preoperative Quantification of Prominence by Otoplasty Grid
■ After determining the goal of projection, the authors create a customized
otoplasty grid for the patient, with a planned projection from the mastoid to the
peak of the helix (15 mm in FIG 2). Upon placing the grid at the back of the
auricle, one can visualize the amount of reduction needed for surgery.
Positioning
■ Supine position with bilateral auricle preparation is recommended, even for
the unilateral prominent ear, so as to check for symmetry (FIGS 4 and 5). One
should also prepare the hair adjacent to the ears.
Approach
■ Most cases need a combined approach to correct the deformities. Among
numerous techniques reported in the literatures, Mustardé mattress sutures are
the most commonly used method to create the antihelical fold.3
■ Stenstrom-type anterior scoring4 can be applied to more rigid cartilage in older
children and adults to facilitate folding of the antihelix. One must be aware that
adult cartilage is also fragile, and overly aggressive scoring can cause
secondary deformities with irregularity of the antihelix, which are aesthetically
unfavorable.
■ To prevent recurrence of the prominent ear deformity, posterior skin excision
is an effective adjunct that can be combined with the Mustardé techniques (FIG

6). The postauricular sulcus should not be markedly decreased; thus, the
amount of skin excision should be conservative. Excessive skin excision can
cause tension in skin closure that may lead to hypertrophic scarring, especially
in the dark-skinned population.
FIG 4 • The patient is placed in supine position to be
able to assess bilateral projection.
FIG 5 • Immediate postoperative view from the front.
Symmetrical set back is achieved.
■ If the patient has projection over 25 mm in the lower pole, conchal
hypertrophy may exist and resection of conchal cartilage and/or conchal
setback may be necessary. Conchal hypertrophy can cause two unwanted
effects: (i) recurrence of projection, and (ii) unnatural bending with sharp
angulation of the antihelix at the level of the antitragus.

■ A prominent lobule not corrected by finger pressure on the helical rim will
require an additional procedure.5 Fishtail-type skin excision and a suture from
the dermis to the scalp periosteum, identifying the point of control,6 are
techniques to address the prominent lobule (FIG 7).
FIG 6 • Mustardé’s radiated suture techniques are
illustrated.
FIG 7 • Preoperative frontal view of Case 1 (bilateral

prominent ears).
T E C H N I Q U E S
■ Preoperative Marking After Intubation
■ Visualizing the precise location to placing sutures: While using fingers to
create ideal setback of the helix, the authors draw two curvilinear lines where
the needle is planned to pass through the cartilage. Radiating direction of
suture placement is also drawn (TECH FIG 1).
TECH FIG 1 • After intubation, curvilinear line (red)
where sutures piercing cartilage and the direction of
suture placement (green) are drawn on the anterior
surface.
Deciding the Amount of Skin Incision
■ Skin incision should be in the middle of posterior aspect of the auricle, so
both helix and mastoid are accessible. The amount of skin excision should be
conservative to preserve the postauricular sulcus. The skin closure should be
tension free to prevent hypertrophic scarring (TECH FIG 2).

TECH FIG 2 • The amount of predicted skin excision is
marked prior to preparation.
■ Local Injection
■ 4 to 5 cc of half and half formula (mixture of 1% lidocaine with epinephrine,
and 0.25% bupivacaine) is injected along the posterior skin incision, with
pinpoint injection anteriorly where sutures are planned to penetrate cartilage.
Injection is performed prior to skin prep to allow time for vasoconstriction.
■ Skin Flap Dissection
■ The skin flap is dissected above the perichondrium. It is important to raise
the skin flap in the same plane to create a skin flap of uniform thickness. The
entire posterior cartilage surface can be exposed through this skin incision.
■ Identify the Two Curvilinear Lines and Draw
Them on the Posterior Surface of the Auricle
■ 25G needles are placed from the anterior surface through the posterior
surface of the cartilage. These points are tattooed with blue ink (methylene
blue). This maneuver is done for each point of insertion of the 25G needles,
and the tattooed marks connected to create a curvilinear line on the posterior
cartilage surface. Two curvilinear lines are drawn on the posterior surface.

■ Mattress Sutures Placement
■ The authors use 4-0 double-armed needles (SH needle) for 4 points. A round
needle is recommended because it is less likely to tear the cartilage. All four
sutures are placed, but not tied, and held with mosquito forceps. The authors
prefer to hand tie the sutures to visualize setback of the cartilage in creating
the antihelix. The otoplasty grid helps to check the height of the helix from
the skull (TECH FIG 3A). Sutures are tied sequentially from the top of the
helix to the bottom. The lowest suture should be at the level of antitragus to
avoid too much angulation or curve distortion of the antihelix due to the
sutures. Care is taken to give a gentle curve to antihelix (TECH FIG 3B).
TECH FIG 3 • A. The view of projection immediately
postoperatively on the left. B. The view of the anterior
surface immediately after Mustardé suture placement.
■ Would Closure
■ Two-layer skin closure is performed after confirmation of hemostasis and
copious saline irrigation. The authors recommend lifting the helix very gently
when closing the skin to minimize pull on the reshaped ear.
Bolster Sutures
■ Use of bolster sutures (TECH FIG 4) is a classic technique popularized by
Tanzer more than 50 years ago. Nagata also uses bolster sutures for most ear

reconstructions. The key is to (1) use soft materials (eg, Xeroform gauze)
amply soaked in vaseline-based ointment and (2) tie very gently to avoid
excess pressure on the skin. The authors use 4-0 Prolene, double-armed, SH
needles for the bolster sutures. Periodic checks, especially 2 days
postoperatively when the swelling is maximum, are mandatory to prevent
pressure sores underneath the bolster sutures.
TECH FIG 4 • Bolster sutures are placed at the end.
PEARLS AND PITFALLS
Mustardé
mattress
sutures
■ Try to keep the natural curve of the antihelix. Do not destroy
the curve by sutures.
Two curvilinear
lines
■ Draw two curvilinear lines for suture placement on the
posterior surface of the auricle before placing sutures.
Anterior
cartilage
surface scoring
■ Do not abrade the cartilage too aggressively, as this may cause
late cartilage deformities.
Measurement of
the projection
■ Check scalp-helix distance in at least three points bilaterally
before and after surgery; the otoplasty grid can be used as a
template.
Conchal
■ If the angle of the antihelix in the vicinity of the antitragus is

hypertrophy
too acute, consider excising a small amount of concha.
FIG 8 • Preoperative posterior left side view of Case 1
(bilateral prominent ears).
POSTOPERATIVE CARE
■ The authors ask patients/parents to keep the wound dry for 2 weeks. Early
shampoo prior to 2 weeks may cause redness; this does not necessarily indicate
infection, but it may confuse both the physician and the patient. If one uses
bolster sutures, periodic check underneath the bolster is mandatory to prevent
pressure sores.
OUTCOMES
■ Achieving symmetry is one of the key elements for the success of prominent
ear correction (Case 1: FIGS 8–10). Measurement of the distance from
mastoid to helix is a helpful guide. The authors use the otoplasty grid to help
achieve symmetry. FIG 11 shows the outcome of unilateral prominent ear
correction (Case 2), for which the otoplasty grid was used as a guide to achieve
symmetry.
COMPLICATIONS
■ Recurrence of prominent ear: Combined approaches instead of a single
technique are better to prevent recurrence of the prominent ear. The use of
resorbable sutures for the Mustardé techniques can contribute to recurrence.
■ Infection: Infection can be caused by a break in proper sterile surgical

technique or dehiscence secondary to excessive tension during skin closure.
Infection can also be an untoward sequela of prior hematoma evacuation.
History of eczema, such as atopic dermatitis, is a warning sign. The external
auditory canal could serve as a reservoir for bacteria, particularly in swimmers.
Prepping the hair near the surgical site is important to prevent infection.
FIG 9 • Postoperative frontal view of Case 1 (bilateral
prominent ears).
FIG 10 • Postoperative posterior view of Case 1
(bilateral prominent ears).
■ Postoperative hematoma: Hematoma is a dreaded immediate postoperative
complication. It is heralded by the acute onset of severe, persistent, and often
unilateral pain. If there is evidence of ongoing bleeding, reoperation and

exploration are mandatory. Meticulous postoperative dressing is vital important
to prevent hematoma. Compression dressings or bolster sutures are effective
dressings and vary with surgeon preference.
■ Skin necrosis: It is important to raise the skin flap in the same plane and
thickness to avoid irregularity of the skin flap. Small blunt straight scissors
may be better than curved scissors in this respect. The proper plane of
elevation for prominent ear is just above the perichondrium. A thinner skin flap
is prone to skin necrosis. When using bolster sutures, it is mandatory to check
for pressure sores beneath the bolster on a regular basis until the bolster sutures
are removed 2 weeks postoperatively.
■ Chondritis: Chondritis is a surgical emergency. If left untreated, it can result in
permanent deformity. Therefore, prompt debridement of devitalized tissue is
necessary.
■ Dysesthesia: Dysesthesia is an uncommon, or less recognized, complication.
The best way to avoid this complication is to use a single skin incision rather
than multiple skin incisions.
■ Suture granuloma and extrusion: This is more common with permanent
braided sutures.
■ Late sequelae of irregularities and deformities of the ear: These are mainly
caused by too aggressive surgical techniques to modify the ear cartilage. In
order to avoid these complications, less traumatic techniques and gentle
manipulation of the ear cartilages are recommended.
FIG 11 • Postoperative frontal view of Case 2 (unilateral
prominent ear).

REFERENCES
1. Tolleth H. Artistic anatomy, dimensions, and proportions of the external ear.
Clin Plast Surg. 1978;5:337.
2. Tolleth H. A hierarchy of values in the design and construction of the ear. Clin
Plast Surg. 1990;17:193.
3. Mustardé JC. The correction of prominent ear susing simple mattress sutures.
Br J Plast Surg. 1963;16:170.
4. Stenstrom SJ. A “natural” technique for correction of congenitally prominent
ears. Plast Reconstr Surg. 1963;32:509.
5. Janis JE, Rohrich RJ, Gutowski KA. Otoplasty. Plast Reconstr Surg.
2005;115(4):60e-72e.
6. Gosain AK, Recinos RF. A novel approach to correction of the prominent
lobule during otoplasty. Plast Reconstr Surg. 2003;112(2):575-583.

38
CHAPTER
Section IX: Eyelid
Reconstruction
Eyelid Coloboma
Adam R. Sweeney and Christopher B. Chambers
DEFINITION
■ Eyelid coloboma (plural: colobomata) is a congenital anomaly arising from
developmental interruption of the eyelid folds causing an eyelid cleft in either
the upper or lower eyelid.
■ The defect may be isolated and incidental but is more commonly associated
with craniofacial syndromes, ocular or periocular colobomata, or other facial
clefts.1,2
ANATOMY
■ The classic description of an eyelid coloboma is a full-thickness defect of a
segment or an entire eyelid including absence of the conjunctiva, tarsus,
orbicularis muscle, and skin.
■ The term “pseudocoloboma” may be used to refer to a partial-thickness defect
of the lid, usually involving the deeper structures with intact superficial layers.
■ There is considerable variability in involvement including only a slight lid
indentation to complete lid absence with underlying ocular abnormalities.
■ The majority are found in the medial third of the upper lid.
■ Approximately 75% of cases are unilateral.2,3
PATHOGENESIS
■ The etiology of eyelid colobomata is not fully understood and likely varies.
■ Variability in the formation of the eyelid folds early in embryogenesis vs later
disruption of eyelid fusion/fissure may implicate the location of a coloboma

defect and any associated facial clefts.2,4
■ Attributed etiologies include intrauterine amniotic bands, abnormalities in
vitamin A metabolism, intrauterine inflammation, decreased placental
circulation, hamartomas, abnormal fetal vasculature, failure of eyelid fold
fusion, or abnormal fetal migration of the neural crest cells.5–7
■ Colobomata may occur in conjunction with Goldenhar syndrome
(oculoauriculovertebral dysplasia),1,8 Treacher Collins syndrome
(mandibulofacial dysostosis),3 frontonasal dysplasia, and Delleman syndrome.9
Goldenhar syndrome is associated with unilateral upper lid coloboma, whereas
Treacher Collins is associated with lower eyelid coloboma or
pseudocoloboma.2
NATURAL HISTORY
■ Untreated, an eyelid coloboma may lead to neonatal exposure keratopathy
with corneal ulceration and perforation.
■ A small coloboma associated with the appropriate amount of superior rotation
of the eye upon eye closure, known as a Bell reflex, may be asymptomatic
requiring only cosmetic surgery later in life.
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Careful physical examination should be performed to assess the thickness and
extent of eyelid loss in both upper and lower eyelids of both eyes. Attention
should be given to the degree of skin laxity for anticipated donor lids, integrity
of anchor sites on the involved lid, and skin color/thickness.10
■ Fornices of eyelids should be meticulously examined for adhesions
(symblepharon) that may hint at underlying diagnosis of cryptophthalmos.
■ Full ophthalmic examination with cycloplegic refraction, eye motility
examination, external examination, slit-lamp examination and dilated fundus
examination should be performed to identify other regions of adverse
development.
■ Both eyes should be closely examined.
■ The most common finding is an upper eyelid medial full-thickness
abnormality. The defect is typically a triangular or quadrangular lesion with the
base toward the eyelid margin (FIG 1).1,6,11
■ The defect may vary in severity from a small indentation to the full eyelid
length.
■ If present in the lower eyelid, partial-thickness defects are more common.

FIG 1 • Left upper eyelid coloboma with loss of lashes,
eyelid notch, and irregular upper eyelid contour.
DIFFERENTIAL DIAGNOSIS
■ Associated syndromes:
■ Goldenhar syndrome (epibulbar dermoids, preauricular appendages, blind
pretragal fistulae, hemifacial atrophy, vertebral anomalies)1,8
■ Treacher Collins syndrome (lower eyelash irregularities, absence of lower
puncta, iris coloboma, microphthalmos, midface hypoplasia, lateral downward
slant of the palpebral fissures)3
■ Delleman syndrome (orbital cysts, intracranial cysts, skin appendages)9
■ Other facial clefting syndromes affecting the eyelids; as described by Tessier,
facial clefts
■ Eyelid trauma
■ Entropion
■ Cryptophthalmos
NONOPERATIVE MANAGEMENT
■ A small eyelid coloboma with mild corneal exposure may be treated with
lubrication, bandage contact lenses, or moisture chambers with surgery
deferred until 6 months of age or later when pediatric eyelid tension has

relaxed.3
SURGICAL MANAGEMENT
■ Surgical correction is the preferred method of treatment.
■ Surgery is required and urgently indicated in cases where exposure
keratopathy is present or imminent.
Preoperative Planning
■ General anesthesia is recommended for pediatric cases.
■ Review the facial photos and examination including size, location, and shape
of the lid defect to help determine the appropriate technique.
Positioning
■ The patient is positioned supine on a surgical table with an adjustable headrest
lowered to give the surgeon the best access. The patient should be prepped and
draped leaving the full face exposed.
Approach
■ Upper or lower eyelid lesions involving ¼ of the eyelid or less may be
amenable to direct closure.
■ Upper or lower eyelid lesions involving a quarter to half the eyelid are treated
with Tenzel semicircular flap.
■ Large lesions with significant tissue loss involving ½ or more of the eyelid
may be managed via a one-stage graft technique involving a tarsomarginal
graft from the ipsilateral lower intact lid or from the contralateral intact upper
eyelid.12
■ For lesions with greater than ½ eyelid tissue loss and not amenable to a one-
stage closure, a two-stage lower lid rotation to the upper lid via a Cutler-Beard
technique may be employed.13
■ The vast majority of large colobomata involving greater than ½ of the eyelid
involve the upper eyelid; thus, techniques specifically addressing upper eyelid
repair are described below. One-stage and two-stage grafting for lower eyelid
closures are routinely performed and described for other conditions; however,
these are out of the scope of this chapter.
T E C H N I Q U E S

■ Direct Closure
■ Place topical tetracaine on the conjunctival surface.
■ Inject 1 to 2 cc 2% lidocaine with 1:100 000 epinephrine into the
subcutaneous space around the lesion and the lateral canthus.
■ Unscroll the underlying tissue of the eyelid margin to explore the full depth
of the defect.
TECH FIG 1 • A,B. Upper eyelid coloboma with
position of proposed canthotomy outlined in the dotted
line.
■ Using a no. 15 blade, trim the borders of the defect to prepare for a full-
thickness, aligned graft site.
■ Pull the defect edges together; if there is significant tension, consider a
canthotomy and cantholysis (TECH FIG 1).
■ Create a horizontal incision along the lid crease or intended lid crease from
the border of the medial defect laterally along the lid crease. The extent of the
lid crease incision should be lengthened to create the amount of laxity needed
to approximate the lateral border of the defect to the medial border of the

defect without tension.
■ Using a 6-0 Vicryl suture, make a single pass at the eyelid margin from
tarsus to tarsus spanning the defect. Ensure equal depth (2 mm deep to the
margin) and horizontal distance from the defect (2 mm from the defect
border) to create a perfectly reapproximated closure. Apply tension to the
ends of the suture to assess the alignment of the closure, but do not tie a knot.
■ Using 6-0 Vicryl suture, reapproximate the deep tarsus using a buried
interrupted pass. This pass should be repeated for every 2 mm of vertical
tarsus absent. Do not tie the knot.
■ Using another 6-0 Vicryl suture, reapproximate the eyelash line using a
single pass.
■ Using another 6-0 Vicryl suture, make a third eyelid margin pass
reapproximating the gray line (TECH FIG 2A). Ensure attention is paid to
the same techniques as used to reapproximate the tarsus at the margin.
■ Tighten all the sutures. The eyelid margin should realign with a slight
eversion. If the eyelid has perfect reapproximation, tie all the knots with a 3-
1-1 knot. Leave the suture tails 3 to 5 mm long (TECH FIG 2B).
■ Close the superficial skin using 7-0 fast gut interrupted sutures. Tie the knots
of the sutures with the tails of the Vicryl suture under the knot to keep the
eyelid margin suture tails from rubbing against the cornea (TECH FIG 2C).
■ Close the relaxation incision with 7-0 fast gut sutures in an interrupted
fashion.
■ Apply erythromycin ointment over the wound.

TECH FIG 2 • Margin involving defect closure. A.
Simple closure of a small eyelid defect including three
eyelid margin passes: one through the tarsus, one
through the gray line, and one through the lash line. B.
Deep nonmarginal tarsus passes are closed first in
buried fashion. Tying of margin involving knots is then
performed leaving suture tails 3 to 5 cm long. C. Close
the superficial skin incorporating the marginal suture
tails in the skin closure knots to detract the tails from
the cornea.
■ Tenzel Semicircular Flap
■ Place topical tetracaine on the conjunctival surface.
■ Using a sterile marking pen, draw a semicircle at the lateral canthus

inferiorly oriented for upper lid repair or superiorly oriented for lower lid
repair (TECH FIG 3A).
■ Inject 2% lidocaine with 1:100 000 epinephrine into the subcutaneous space
around the defect extending to the frontozygomatic suture.
■ Unscroll the underlying tissue of the eyelid margin to explore the full depth
of the defect.
■ Using a no. 15 blade, incise the skin and orbicularis tissue along the marked
semicircle beginning at the lateral canthus.
■ Undermine the musculocutaneous semicircular flap laterally using blunt
dissection to create lid laxity (TECH FIG 3B).
■ Complete a full-thickness canthotomy using Westcott scissors.
TECH FIG 3 • A,B. Diagram depicting inferiorly
oriented semicircular orientation for large upper lid
coloboma repair.
■ Grasp the lateral aspect of the involved eyelid and gently apply anterior
traction to expose the canthal tendon. For an upper eyelid coloboma, perform
a superior arm cantholysis; and for lower eyelid coloboma, perform an
inferior arm cantholysis (TECH FIG 4A,B).
■ The cantholysis of the respective arm of the canthal tendon is performed by
strumming the multiple attachments of the respective arm with closed
scissors and then cutting each attachment, ensuring further laxity is felt with
each cut (TECH FIG 4B). Avoid cutting conjunctiva by maintaining
adequate visualization and hemostasis with handheld cautery.
■ Full cantholysis is achieved when the respective eyelid can be distracted to
reach the superior orbital rim for an inferior cantholysis or the inferior orbital
rim for a superior cantholysis (TECH FIG 4C).

■ Using a no. 15 blade, trim the borders of the defect to prepare a full-
thickness, aligned graft site. This should be attempted to create a rectangle-
shaped border to ensure easier closure.
TECH FIG 4 • Lateral canthotomy and cantholysis. A.
Following canthotomy, a lateral cantholysis is
performed by grasping the eyelid margin and brought
anteriorly to expose the multiple canthal tendon arms.
For upper lid repairs, a superior cantholysis is
performed; for lower lid repairs, an inferior cantholysis
is performed (B). C. Full lower lid cantholysis is
achieved when the lower lid can be distracted
superiorly to the superior orbital rim. Similarly, an
upper lid cantholysis is achieved when the upper lid
can be distracted to the inferior orbital rim.
■ Using forceps, distract the loose eyelid margin to bring together the edges of
the wound (TECH FIG 5A). Mark the temporal aspect of the distracted
eyelid that aligns with the lateral canthus of the uninvolved/unaffected eyelid.

■ Close the eyelid defect wound using the steps described in the “direct
closure” technique above (TECH FIG 3).
■ Using a single interrupted 5-0 Vicryl suture, make a pass through the deep
tissues along the repaired eyelid where previously marked at the intended site
of lateral canthus reconstitution. Secure this suture by passing it through the
periosteum of the underlying lateral orbital rim exiting 1 to 2 mm superior to
the entrance of the pass (TECH FIG 5B).
■ Secure the lateral aspect of the flap using 5-0 Vicryl suture by making a
single pass through the deep tissues of the flap, into the underlying
prezygomatic fascia, and then out the deep tissue of the opposite side of the
flap to create a buried suture adherent to the underlying fascia.
■ Repeat this step with interrupted sutures to fully close the length of the flap.
■ Reconstitute the lateral canthus by suturing the upper lid gray line to the
lower lid gray line approximately 2 mm from the termination of the lateral
gray line using 6-0 Vicryl suture.
■ Close the superficial skin of the flap using 7-0 fast gut suture in an
interrupted fashion.
■ Apply erythromycin ointment over the wound.
TECH FIG 5 • A. Align the tissue with minimal tension
to the lateral canthus of the opposite eyelid. B. Secure
the tissue with a 5.0 vicryl suture through the
obicularis muscle to the orbital rim periosteum.
■ One-Stage Graft (Tarsomarginal Graft)
■ Place topical tetracaine on the conjunctival surface.
■ Using a sterile marking pen, draw an inferiorly oriented semicircle at the

lateral canthus.
■ Inject 2% lidocaine with 1:100 000 epinephrine into the subcutaneous space
around the defect and the contralateral upper eyelid.
■ Unscroll the underlying tissue of the eyelid margin to explore the full depth
of the defect.
■ Using a no. 15 blade, trim the borders of the defect for a full-thickness,
aligned graft site.
■ Using calibers, measure the dimensions of the missing underlying tarsus.
■ Mark the contralateral (donor) upper eyelid with the calipers per the
dimensions measured for the graft upper lid using an inferior border that is
superior to the eyelid margin (TECH FIG 6).
TECH FIG 6 • Scheme depicting one-stage
contralateral upper eyelid graft using donor
conjunctival/tarsus to host eyelid. Host upper eyelid
anterior lamella is placed over the graft after
undermining superiorly and laterally.
■ On the contralateral (donor) upper eyelid, use Westcott scissors to dissect a
plane at the level of the orbicularis oculi, extending to the marked borders
between the posterior lamella (conjunctiva and tarsus) and the anterior
lamella (orbicularis muscle and skin).
■ Using Westcott scissors, amputate the graft of tarsus and conjunctiva from
the contralateral donor upper eyelid within the borders marked, sparing the
eyelid margin.
■ Bring the tarsomarginal graft into the donor site and suture the grafted tarsus
to the host tarsus with two interrupted 6-0 Vicryl sutures per side leaving the
eyelid margin free of suture. Ensure the knots are buried deep, avoiding
potential conjunctival or corneal abrasion from posterior oriented knots.

■ Using Westcott scissors, reconstruct the donor upper lid anterior lamella by
undermining deep to the patient’s adjacent orbicularis muscle using Westcott
scissors. Advance the adjacent anterior lamella over the graft tissue ensuring
no vertical tension is created. Close this tissue with interrupted 7-0 gut
passes.
■ Close the posterior lamella of the donor eyelid via the “direct closure” of
deep horizontal eyelid lesion technique described above (TECH FIG 3).
■ Apply erythromycin ointment over both eyelids.
■ Two-Stage Graft (Cutler-Beard)
■ Place topical tetracaine on the conjunctival surface.
■ Inject 2% lidocaine with 1:100 000 epinephrine into the subcutaneous space
of the upper and lower eyelids.
■ Unscroll the underlying tissue of the eyelid margin to explore the full depth
of the defect.
■ Using a no. 15 blade, trim the borders of the defect for a full-thickness,
aligned graft site. This should be attempted to create a rectangle-shaped
border to ensure easier closure.
■ Using a sterile marking pen, make two vertical markings on the lower lid to
outline the borders of the horizontal defect of the upper lid. Connect the
vertical markings of the lower lid horizontal mark placed 4 mm inferior to the
eyelid margin.
■ Pass a 4-0 silk traction suture into the lower eyelid margin ensuring a tarsal
bite is achieved.
■ Using a no. 15 blade, incise the skin and orbicularis over the horizontal
marking (TECH FIG 7A).
■ Holding the silk suture by the tails, evert the lower lid over an eyelid
retractor and make a horizontal conjunctival incision. This incision should be
deep to the horizontal marking previously placed on the lower eyelid skin.
■ Using Westcott scissors, incise through the conjunctival incision full
thickness to connect to the anterior lamella incision.
■ Using Westcott scissors, make deep orbicularis cuts through the vertical
markings of the lower lid (TECH FIG 7B).
■ With toothed forceps, grasp the superior edge of the flap created by the full-
thickness horizontal incision already created. Bring this flap under the lower

eyelid margin (through the blepharotomy) and place it into the region of the
upper eyelid defect (TECH FIG 7C).
■ Suture the lower lid grafted tarsus to the tarsus at the borders of the upper lid
coloboma using 5-0 Vicryl suture with at least two passes per side ensuring
anteriorly oriented knots to avoid conjunctival abrasion (TECH FIG 7D).
■ Undermine around the lid defect until the anterior lamella is lax enough to
close the anterior lamella directly and without tension using 5-0 Vicryl
sutures (TECH FIG 7E).
■ Close the superficial skin of the flap using 7-0 fast gut interrupted passes.
■ Apply erythromycin ointment over the wound.
■ The second stage is performed by releasing the flap with Westcott scissors at
the intended eyelid margin. Excess conjunctiva and skin can be removed
from the upper eyelid (TECH FIG 8).

TECH FIG 7 • First stage of Cutler-Beard procedure.
A. Incision is made through skin and orbicularis over
the horizontal marking. B. Incision through the vertical
markings is made revealing full tarsoconjunctival flap.
This is dissected from the anterior lamellae. C. The
flap is grasped and sutured into the graft position
allowing laxity of tissue. D. The graft is sutured into
position using 5-0 Vicryl suture ensuring anteriorly
oriented knots. E. The anterior lamellae host tissue is

then sutured into position using 7-0 fast gut.
TECH FIG 8 • Second stage of Cutler-Beard
procedure. A. Incise the graft tissue at the desired
upper lid margin using a metal backing to protect the
underlying cornea. B. Marginal and graft are then
sutured ensuring no knots exposed to the cornea.
■ The remaining conjunctiva is sutured into place to the anterior edge of the
eyelid margin using 7-0 fast gut sutures with the knot placed away from the
cornea (TECH FIG 9).
■ In children between birth and 14 years old, the second stage is performed at
2 weeks, rather than the recommended 4 to 6 weeks, to minimize the risk of
deprivation amblyopia.

TECH FIG 9 • Suture the excess conjunctiva over the
anterior edge of the eyelid margin with a fast gut
suture.
PEARLS AND PITFALLS
Choosing
a
technique
■ Two-stage repairs for large colobomata incur a period of visual
deprivation that can lead to amblyopia. Full consideration of one-stage
technique, when applicable, is recommended.
■ Skin laxity and eyelid graft development must be considered when
choosing a graft. There is tendency to underestimate the tension and
required modifications to adequately close pediatric eyelid defects.
Timing of
surgery
■ Children less than 3–6 months of age have less skin laxity, and when
not contraindicated, postponement of surgery may achieve better
outcomes. When early surgery is indicated, the lateral canthotomy and
cantholysis described in the “direct closure” technique offers improved
eyelid relaxation, decreasing horizontal tension.
POSTOPERATIVE CARE
■ Prescribe topical application of antibiotic ointment over the wounds 3 times a

day for 1 to 2 weeks.
■ Recommend sun tan lotion to improve scar results.
■ Comanagement with pediatric ophthalmologist is recommended for regular
refraction and monitoring for development of primary or secondary strabismus
or corneal scarring.
OUTCOMES
■ Surgical success is dependent on the degree of the coloboma and the procedure
type selected.
■ Irregular postoperative eyelid contour may occur if the lid defect is closed
under too much tension.3
COMPLICATIONS
■ Exposure keratopathy from unintended vertical traction
■ Loss of lashes from the graft or trichiasis
■ Entropion or ectropion
■ Ptosis secondary to poor levator function or postoperative horizontal traction
■ Poor appearance
REFERENCES
1. Tawfik HA, Abdulhafez MH, Fouad YA. Congenital upper eyelid coloboma:
embryologic, nomenclatorial, nosologic, etiologic, pathogenetic,
epidemiologic, clinical, and management perspectives. Ophthal Plast Reconstr
Surg. 2015;31(1):1-12.
2. Smith HB, Verity DH, Collin JR. The incidence, embryology, and oculofacial
abnormalities associated with eyelid colobomas. Eye (Lond). 2015;29(4):492-
498.
3. Grover AK, Chaudhuri Z, Malik S, et al. Congenital eyelid colobomas in 51
patients. J Pediatr Ophthalmol Strabismus. 2009;46(3):151-159.
4. Guercio JR, Martyn LJ. Congenital malformations of the eye and orbit.
Otolaryngol Clin North Am. 2007;40(1):113-140, vii.
5. Roper-Hall MJ. Congenital colobomata of the lids. Trans Ophthalmol Soc U
K. 1969;88:557-566.
6. Seah LL, Choo CT, Fong KS. Congenital upper lid colobomas: management
and visual outcome. Ophthal Plast Reconstr Surg. 2002;18(3):190-195.
7. Miller MT, Deutsch TA, Cronin C, Keys CL. Amniotic bands as a cause of
ocular anomalies. Am J Ophthalmol. 1987;104(3):

270-279.
8. Gorlin RJ, Jue KL, Jacobsen U, Goldschmidt E. Oculoauriculovertebral
dysplasia. J Pediatr. 1963;63:991-999.
9. Delleman JW, Winkelman JE. [The significance of atypical colobomata and
defects of the iris for the diagnosis of the hereditary aniridia syndrome
(author’s transl)]. Klin Monbl Augenheilkd. 1973;163(5):528-542.
10. Collin JR. Congenital upper lid coloboma. Aust N Z J Ophthalmol.
1986;14(4):313-317.
11. Nouby G. Congenital upper eyelid coloboma and cryptophthalmos. Ophthal
Plast Reconstr Surg. 2002;18(5):373-377.
12. Hoyama E, Limawararut V, Malhotra R, et al. Tarsomarginal graft in upper
eyelid coloboma repair. J AAPOS. 2007;11(5):499-501.
13. Cutler NL, Beard C. A method for partial and total upper lid reconstruction.
Am J Ophthalmol. 1955;39(1):1-7.

39
CHAPTERCongenital Ptosis
Adam R. Sweeney and Christopher B. Chambers
DEFINITION
■ Blepharoptosis, commonly referred to as ptosis, is eyelid positioning inferior
to normal posture. Often, ptosis is defined as asymmetry of 1 mm or more
between the two upper eyelids or a marginal reflex distance of less than 2.5
mm.1
■ Congenital ptosis refers to cases presenting within the first year of life, as an
isolated finding or as a manifestation of ophthalmic or systemic disorders.
■ Typically, congenital ptosis results from fibrofatty degeneration of the levator
complex.
■ Congenital ptosis affects an estimated 1 in 842 births in the United States.1
ANATOMY
■ The anterior lamella is made up of the skin and the preseptal orbicularis (FIG
1).
■ The posterior lamella is made up of the tarsus and the palpebral conjunctiva.
■ The tarsus is a fibrous plate located in the eyelid. It is 9 to 10 mm in height in
the upper eyelid and 4 mm in height in the lower lid. The length of the tarsus is
approximately 22 to 25 mm.
■ Posterior to the orbital septum lies the preaponeurotic fat pad, an important
surgical landmark.
■ The levator aponeurosis is the primary retractor of the upper eyelid and is
located just posterior to the preaponeurotic fat.
■ The levator palpebrae originates above the annulus of Zinn and attaches to
Whitnall ligament, a transverse facial suspensory structure. This attachment
allows the levator muscle’s horizontal vector to be redirected to the vertical

vector created from its anterior extension, the levator aponeurosis, which
terminates at the anterior tarsus.
■ Muller muscle arises from the posterior layer of the levator muscle and inserts
into the upper border of the tarsal plate.
■ The lid crease is formed from levator aponeurosis fibers inserting into the
fascicular bundles of the pretarsal orbicularis.
■ In patients not of Asian descent, the lid crease is located between the upper
border of the tarsus and the insertion of the orbital septum, which is
approximately 3 to 5 mm superior to the upper border of the tarsus. The lid
crease is approximately 9 to 11 mm and 8 to 9 mm superior to the eyelid
margin in females and males, respectively.
■ In Asians, the lid crease is much lower secondary to a more anterior
preaponeurotic fat pad and low/absent attachment of the aponeurosis fibers to
the fascicular bundles of the pretarsal orbicularis.

FIG 1 • Anatomy of the upper eyelid.
■ The levator muscle is a striated muscle and is innervated by cranial nerve (CN)
III, whereas Muller muscle is a smooth muscle innervated by the sympathetic
chain.
■ The frontalis muscle inserts into the orbicularis oculi at the inferior brow.
PATHOGENESIS
■ There are multiple different etiologies of congenital ptosis.

■ Approximately 80% of congenital ptosis arises from dysgenesis or fibrosis of
the levator muscle with remaining integrity of the levator aponeurosis, termed
myogenic ptosis or simple congenital ptosis.1,2
■ Myogenic ptosis is more common unilaterally than bilaterally.
■ Neurogenic etiologies are much less common and arise from innervation
abnormalities during development such as abnormal motor nerve connections
of the levator muscle and branches of the trigeminal nerve innervating the
external pterygoid muscle (Marcus Gunn jaw winking), interrupted
sympathetic nervous chain (Horner syndrome), congenital cranial nerve III
palsy, or congenital cranial dysinnervation disorders such as Duane retraction
syndrome.
■ Aponeurotic ptosis is caused by a defect in the tendon’s ability to redirect the
action supplied by the levator muscle. This is classically different from
disinsertion of the aponeurosis from the levator muscle, as is commonly seen in
advancing age or from trauma including childbirth.
■ Mechanical ptosis is caused by a forced inferior excursion of the eyelid such
as seen in tumor, eyelid inflammation/edema, cicatricial processes, or foreign
body.
■ Traumatic ptosis usually can be found to be inclusive in one of the above
categories and may arise in utero or from trauma, such as during childbirth.
NATURAL HISTORY
■ Congenital ptosis may be mild and benign with no impact on vision or
function of the child.
■ Severe congenital ptosis may cause amblyopia due to deprivation or
astigmatism.
■ Twenty to thirty percent with congenital ptosis have concurrent strabismus or
astigmatism.3,4
■ Twenty-five percent of patients with congenital ptosis develop amblyopia of
the involved eye.2
PATIENT HISTORY AND PHYSICAL FINDINGS
■ Hallmark findings distinguishing congenital myogenic ptosis from acquired
ptosis: absence or irregular upper eyelid crease, decreased levator function
(particularly prominent in upward gaze), eyelid retraction in downward gaze,
and presence of lagophthalmos (a common finding in congenital ptosis).
■ Marcus Gunn jaw-winking ptosis involves elevations of the eyelid with

pterygoid muscle movement usually evident with sucking or chewing.
Monitoring for association of lid height in various gazes and facial movements
including jaw movement is important to rule out this condition.
■ Horner syndrome classically manifests with ptosis, meiosis, and anhidrosis
ipsilateral to the neurological defect. Ipsilateral lower lid inverse ptosis may
also be seen. Heterochromia characterizes the congenital form from acquired
Horner syndrome and necessitates systemic workup.
■ Margin reflex distance (MRD) of the upper lid and vertical palpebral fissure
height should be routinely assessed, when possible.
■ MRD1 in down gaze, evaluating chin, head, and brow position should be noted
as compensatory mechanisms.
■ Strength of Bell phenomenon (eye elevation and abduction on attempted lid
closure) may indicate whether dysgenesis of the levator muscle includes the
superior rectus muscle.
■ Perform a pupil exam monitoring for symmetry.
■ Test extraocular muscle movements assessing for partial CN III palsy or
assessing for hypotropia-related pseudoptosis.
■ Test the frontalis contribution by immobilizing the brow in primary position as
children often will attempt to correct ptosis by recruiting the frontalis muscle.
■ Assess corneal exposure issues with fluorescein staining.
■ The phenylephrine test is performed by instilling one eye drop of 2.5%
phenylephrine to the inferior cul-de-sac. This elevates the eyelids within 10
minutes via the sympathetic response on smooth muscles of the eyelid,
principally Muller muscle. The MRD1 should be assessed before and 10
minutes after instillation. Demonstration of adequate improvement of the ptosis
after phenylephrine test may guide the surgical technique required, such as a
Muller muscle resection. Unilateral phenylephrine dosing may reveal a
contralateral ptosis masked by Hering law of equal bilateral innervation.
■ Attaining visual acuity and fixation preferences may help detect amblyopia.
Evaluation of these patients with a pediatric ophthalmologist is important.
IMAGING
■ Quality full facial photographs should be obtained for preoperative planning
and intraoperative reference.
■ In older children or adults with congenital ptosis, visual field testing with
taped and untaped eyelids should be performed.

■ An A-scan may be performed if one is concerned about pseudoptosis, a term
describing ptosislike appearance from pathology independent of the eyelid,
such as microphthalmia or advanced hyperopia.
■ Cycloplegic refraction or autorefraction may be performed to detect ptosis-
induced astigmatism that may be amblyogenic.
■ Congenital Horner syndrome should be evaluated with a tumor workup that
may necessitate MRI investigation.
DIFFERENTIAL DIAGNOSIS
■ Myogenic: myasthenia gravis, chronic progressive external ophthalmoplegia,
muscular dystrophy, and oculopharyngeal dystrophy
■ Myogenic etiology also seen in blepharophimosis-ptosis-epicanthus inversus
syndrome (BPES), which also may have euryblepharon, telecanthus, ear
deformities, and hypoplasia of the superior orbital rims and nasal bridge
■ Neurogenic: Marcus Gunn jaw-winking ptosis, Horner syndrome, aberrant
regeneration of the oculomotor nerve, Duane retraction syndrome
■ Aponeurotic ptosis refers to slowly progressive ptosis from involutional
changes in the levator aponeurosis with gradual stretching or dehiscence of this
structure.
■ Mechanical ptosis from excessive upper eyelid weight such as secondary to an
eyelid mass, foreign body, or eyelid edema
■ Pseudoptosis: Microphthalmia (underdevelopment of the orbit, lids, and
socket) or advanced hyperopia with smaller anteroposterior globe length
NONOPERATIVE MANAGEMENT
■ If ptosis is not cosmetically bothersome and does not appear to affect visual
axis or lead to astigmatism, observation may be appropriate.
SURGICAL MANAGEMENT
■ When potentially amblyogenic, prompt ptosis surgery is indicated.
■ The timing of surgical correction in the absence of visually threatening ptosis
is debated with many advocating for correction at approximately age 4 when
structures are more defined and the child is more cooperative5; however, others
suggest earlier treatment advocating ease of development and postoperative
care.
■ Consideration of the type of surgery should reflect the degree of ptosis, levator
function, and emphasis on cosmetic outcomes.