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RECONSTRUCTIVE

Experience with Developmental Facial Paralysis:


Part II. Outcomes of Reconstruction
Julia K. Terzis, M.D., Ph.D.
Katerina Anesti, M.D.
Long Island City, N.Y.

Background: The purpose of this study was to document the 30-year experience
of the authors center in the management of developmental facial paralysis and
to analyze the outcomes of microsurgical reconstruction.
Methods: Forty-two cases of developmental facial paralysis were identified in a
retrospective clinical review (1980 to 2010); 34 (80.95 percent) were children
(age, 8 6 years) and eight (19.05 percent) were adults (age, 27 12 years).
Comparisons between preoperative and postoperative results were performed
with electrophysiologic studies and video evaluations by three independent
observers.
Results: Mean follow-up was 8 6.3 years (range, 1 to 23 years). Overall,
outcome scores improved in all of the patients, as was evident from the observers
mean scores (preoperatively, 2.44; 2 years postoperatively, 3.66; final, 4.11; p
0.001, Kruskal-Wallis test) and the electrophysiologic data (p 0.0001). The
improvement in eye closure, smile, and depressor function was greater in
children as compared with adults (p 0.005, Mann-Whitney test).
Conclusions: Early targeted screening and diagnosis, with prompt specialized
treatment, improves the physical and emotional development of children with
developmental facial paralysis and reduces the prevalence of aesthetic and
functional sequelae of the condition, thus facilitating reintegration among their
peers. The experience of this center should serve as a framework for the
establishment of accurate and reliable guidelines that will facilitate early diagnosis and management of developmental facial paralysis and provide support
and counseling to the family. (Plast. Reconstr. Surg. 129: 66e, 2012.)

he critical and unique role of the face in


human development and social interaction
is revealed in case studies of individuals with
developmental and acquired facial deformities.1,2
Facially disfigured people can experience significant psychological problems, commonly related to
difficulties in social interaction.3,4
The importance of early recognition and treatment of neonatal facial paralysis has been stressed
by many authors.5 8 The main argument behind
early versus late treatment is that alterations in
facial appearance may interfere with attachment
formation between the child and primary caretakers and may influence the childs perception of
himself or herself. Later, the manner in which
peers and other adults react to the facial deformity
From the International Institute of Reconstructive Microsurgery and the Department of Plastic and Reconstructive Surgery, New York University Medical Center.
Received for publication April 4, 2011; accepted June 16,
2011.
Copyright 2011 by the American Society of Plastic Surgeons
DOI: 10.1097/PRS.0b013e3182362122

66e

may be particularly stigmatizing. In contrast, differentiating developmental from traumatic facial


paralysis noted at birth is important not only for
determining prognosis but also for medicolegal
reasons.9,10
Congenital facial paralysis refers to conditions
that are acquired during or at birth (e.g., from
trauma), whereas developmental facial paralysis is
the result of developmental mishaps during fetal
development.11 Developmental facial paralysis can
present in isolation or as part of a recognized
syndrome (e.g., Mobius, Goldenhar, CHARGE).
The purpose of this study was to document the
30-year experience of our center in the management of developmental facial paralysis and
to analyze the outcomes of microsurgical reconstruction. This study has been approved by the

Disclosure: Neither of the authors has a financial


interest in any of the products or devices mentioned
in this article.

www.PRSJournal.com

Volume 129, Number 1 Developmental Facial Paralysis


Institutional Review Board at Eastern Virginia
Medical School.

ETIOLOGY
In general, the anatomical presentation of the
various anomalies of the facial nerve in developmental facial paralysis can be summarized into
four categories:
1. Aplasia or hypoplasia of cranial nerve nuclei.
2. Nuclear agenesis.
3. Peripheral nerve abnormalities (aplasia or
hypoplasia, bifurcation, anomalies in the
course of the facial nerve).
4. Primary myopathy.12
There appear to be two main pathways through
which cranial nerve dysfunction occurs. The first
involves failure of the cranial nerve nuclei to develop
normally and their motoneuron pools to differentiate, aggregate, and establish proper neuronal
connections. Genetic factors, vascular events, and
teratogenic insults have been implicated. The second mechanism involves genetic defects that
lead to abnormal axonal transport of molecules
necessary for normal muscle function and
development.13 The timing and exact location of
errors may have different effects, accounting in
part for the diversity of clinical presentations14 and
various stigmata or associated anomalies.4
The initial optimal assessment of the neonate born with unilateral facial paralysis should
be performed as soon after birth as possible with
the goal of distinguishing between a congenital
and a developmental cause.15 A thorough diagnostic workup that includes complete neurologic examination; ophthalmologic evaluation;
and ear, nose, and throat/hearing evaluation is
imperative for proceeding with the appropriate
treatment plan.4

PATIENTS AND METHODS


The medical records of 42 patients were reviewed thoroughly for age at presentation, sex,
surgical details, and outcomes. Preoperative evaluation of all patients was performed with detailed
physical examination, electroneurographic evaluation, needle electromyography, nerve conduction studies, ophthalmologic assessment to exclude amblyopia, and occasional otolaryngologic
consultation. Imaging studies included computed
tomographic scans of bilateral temporal bones, or
polytomograms in the earlier years. Evidence of
various stigmata and/or associated anomalies
(i.e., hypoplastic facial canal, aberrant vertical segment of the facial nerve, amblyopia, nasal alar

atresia, acne, and possible ear deformities) were


documented in all patients.4
All operations were performed by the senior
author (J.K.T.) except for the unroofing of the
mastoid, which was performed by an ear, nose, and
throat colleague. Each procedure was documented preoperatively, intraoperatively, and postoperatively by videos and detailed drawings of the
staged reconstruction. Facial reanimation procedures including cross-facial nerve grafting, free
muscle transplantation, and revision surgery were
performed as described previously,16 19 through a
multistage approach.
The experience of our unit in the evaluation,
classification, and treatment of Mobius patients
has been reported previously.20,21 Comparisons between preoperative and postoperative results have
been assessed with electromyography and video
evaluations. A panel of three independent observers used the Terzis Functional and Aesthetic Grading System19,22 to grade separately patients videos
at three time points: preoperatively, 2 years after
the free muscle transfer, and at the final follow-up
visit. The intraclass correlation coefficient (Spearman rs) was 0.5793.
Population Dynamics
Forty-two cases of developmental facial paralysis were identified; 34 (80.95 percent) were children and eight (19.05 percent) were adults. Of
these, 24 (57.14 percent) were female patients and
18 (42.86 percent) were male patients. Demographic characteristics are listed in Tables 1
through 3.
Table 1. Demographics of Our Study Population
Characteristic
Total no. of patients
Sex
Female
Male
Age group
Children
Adults
Mean age SD, yr
Children
Adults
Side
Left facial paralysis
Right facial paralysis
Follow-up, yr
Children
Mean
Range
Adults
Mean
Range

Value
42
24
18
34
8
86
27 12
18
24
8
123
7.3
120

67e

68e

13

15
13

12

11

1
10

10

16

10
4

11

4
5

10

11

12

13
14

15

16

17

18
19

20

21

Patient
1

F
M

F
F

M
F

R
L

L
L

L
L

Age
(yr) Sex Side
2
F
R

Malocclusion

Hypothyroidism

Epileptic

Cleft lipcleft palate,


external ear,
aberrant subclavian
artery, left XI,
bilateral VIII

PDA, TOF, choanal


atresia, external
ear, undescended
testes, right VI, X,
bilateral VIII

PDA, plagiocephaly,
hypognathia, left
VIII, left XII, left
microtia

Other Anomalies/
Cranial Nerves
Involved

Left pectoralis
minor
Right pectoralis
minor

Left gracilis

CFNG 3
CFNG 2

CFNG 2

No

No

No
No

CFNG 3

CFNG 3

CFNG 3
CFNG 4

CFNG 3

CFNG 4

CFNG 3

CFNG 2
Fascia sling

CFNG 2
CFNG 3

CFNG 3

CFNG 2

CFNG 3

CFNG 3

No
CLP repair,
CFNG, ear
reconstruction

No

No

No

No

No

No

Right platysma to
right depressor
plus revisions

Mini-temporalis to
right lip

Stage III
Revisions

Revisions

Additional Stages
Left pectoralis minor
plus revisions

Right frontalis to
left eye
Right pectoralis Left digastric plus
minor
revisions
Left gracilis
Revisions
Right pectoralis Right frontalis
minor
Left pectoralis
Right digastric
Mini-temporalis plus
minor
plus revisions
revisions
Left pectoralis
Mini-temporalis plus PL minitendon to
minor
left platysma to
lower eyelid plus
lower lip
revisions

Left pectoralis
minor
Right serratus

Mini-temporalis plus
STFF
Left pectoralis
Mini-temporalis plus
minor
revisions
Right pectoralis Revisions
Revisions
minor
Right pectoralis
minor
Left pectoralis
Revisions
minor
Left digastric
Revisions
Right pectoralis Right frontalis to
Left digastric plus
minor
left eye
revisions

Left pectoralis
minor
Left pectoralis
minor
Left gracilis

CFNG 3
CFNG 3

Stage II
Left frontalis

Stage I
CFNG 2

No
CFNG 2
Ear reconstruction CFNG 4

No

No

Previous Surgery
No

Table 2. Demographic and Intraoperative Variables of the Pediatric Population

(Continued)

22

17

7
2

23

10

5
6

15

3
1

Follow-Up
(yr)
19

Plastic and Reconstructive Surgery January 2012

1
2

3
8

15

15
11

14

23

24
25

26

27
28

29

30
31

32

33

34

F
M

M
F

M
F

R
R

R
R

L
R

No
No

No

No

Previous Surgery

Left pectoralis
minor

CFNG 3

TOF, hypognathia,
TOF repair,
Bilateral digastric
external left ear
feeding
to depressors
anomaly, and testes,
gastrostomy
plus mini- XII
aberrant subclavian
artery, left V, VI,
bilateral I, VIII, XI,
and XII
Plagiocephaly,
Correction of
CFNG 4 plus
cerebral palsy, left
plagiocephaly
mini-XII
V, VI, and VIII
plus left VI palsy

Right platysma
to left eye

CFNG 3 plus
revisions

Left gracilis

Left gracilis
Left pectoralis
minor

Left gracilis

Left gracilis
Left gracilis

Left digastric
Left gracilis

CFNG 2
CFNG 2

CFNG 3

Left pectoralis
minor
Left gracilis

Stage II

CFNG 2

Stage I

No
CFNG 4
CFNG plus left VII CFNG 3, XI to
to right VII
VII
Benign heart murmur No
CFNG 2, miniXII
No
CFNG 3
Hypognathia,
Tarsorrhaphy,
CFNG 3
external ear,
right ear
agenesis right
reconstruction,
kidney, bilateral I,
otoplasty
VIII, IX, and X
No
CFNG 3

Benign heart murmur No

Other Anomalies/
Cranial Nerves
Involved

Right pectoralis
minor

Left platysma for


excess pull
Right gracilis

Left platysma plus


revisions

Mini-temporalis

STFF right cheek


STFF right cheek

Mini-temporalis to
right eye
Left platysma to
right eye

PL minitendon
graft
PL minitendon
graft

Stage III

Sternocleidomastoid
to left depressor
plus revisions

Mini-temporalis plus
PCFF plus
revisions
Revisions
Mini-temporalis plus
revisions
STFF right cheek
plus revisions

Additional Stages

15

10

12
7

11
6

16

5
4

12

Follow-Up
(yr)

F, female; M, male; R, right; L, left; CFNG, cross-facial nerve grafting; PDA, patent ductus arteriosus; TOF, tetralogy of Fallot; CLP, cleft lip cleft palate; STFF, superficial temporal fascia
flap; PL, palmaris langus; PCFF, posterior calf vascular fascia flap.

22

Patient

Age
(yr) Sex Side

Table 2. (Continued)

Volume 129, Number 1 Developmental Facial Paralysis

69e

70e

30

28

51

25

19

19

23

18

Patient

Sex

Side

Hemifacial
microsomia left VI,
VII, VIII, XII
tongue tie
retrognathia
Right VI

Other Anomalies/
Cranial Nerves
Involved

Bilateral medial
rectus resection

Muscle graft 3,
blepharoplasty,
TFL sling,
bilateral
otoplasty
Bilateral medial
rectus resection
bilateral
mandibular split

Intraoral masseter
transposition
plus brow lift
No

Face lift

No

No

Previous
Surgery
Stage II

Left gracilis

Mini- temporalis
plus STFF right
cheek plus
revisions

CFNG 3 Left gracilis

Revisions

Revisions

Mini-temporalis
with PL sling

Stage III

CFNG 4 Right frontalis


to left eye

CFNG 2 Right digastric


to right
depressor
CFNG 4 Right gracilis

CFNG 1 Left digastric to


left depressor
CFNG 3 Left pectoralis
minor
CFNG 3 Left gracilis

CFNG 3 Left gracilis

Stage I

DN left tongue plus


eye spring plus
revisions

Revisions

Revisions

Additional
Stages

12

17

20

Follow-Up
(yr)

F, female; M, male; R, right; L, left; CFNG, cross-facial nerve grafting; PL, palmaris langus; TFL, tensor fasciae latae; DN, direct neurotization; STFF, superficial temporal fascia flap.

Age
(yr)

Table 3. Demographic and Intraoperative Variables of the Adult Population

Plastic and Reconstructive Surgery January 2012

Volume 129, Number 1 Developmental Facial Paralysis


The right facial nerve was involved in 24 cases
(57.14 percent) and the left facial nerve was involved in 18 cases (42.86 percent). The average
age at presentation was 8 years (range, 1 month to
16 years) in children and 27 years (range, 18 to 55
years) in adults.
Thirty-one patients had simple developmental
paralysis and two patients had developmental unilateral lower lip palsy. There were nine patients
with associated anomalies or craniofacial syndromes. Five of these patients had multiple cranial
nerve deficits.
Statistical Analysis
Data analysis was performed with PC software
(GraphPad Instat; GraphPad Software, Inc., La
Jolla, Calif.). Unpaired t test and one-way analysis
of variance test followed by post hoc test if indicated were computed for comparison of two or
more groups managed with different techniques.
Mann-Whitney and Kruskal-Wallis nonparametric
tests were used if normality or equal variance testing failed.
To measure the effect of surgical procedures,
postoperative final gain scores (difference in median scores) were computed using a percentage
gain score and the associated confidence interval
and p value. The level of significance was set at
p 0.05. An association between two variables was
tested with Spearmans correlation.

RESULTS
Our reconstructive approach in these patients
is summarized below and in Table 4.

Upper Face
Fourteen patients in this series (33.3 percent)
required no eye intervention. Ten cases (23.8 percent) had a silent orbicularis oculi muscle on needle electromyography and underwent orbicularis
oculi muscle substitution. Orbicularis oculi muscle substitution consisted of pedicled frontalis
transposition in five cases (Fig. 1) and pedicled
temporalis in one. Two patients received a free
contralateral platysma transfer, and in another
two patients the upper slip of a free pectoralis
minor was used (Fig. 6).
In 11 patients (26.1 percent), needle electromyography yielded fibrillations of the orbicularis
oculi muscle; direct neurotization (nine cases),
seventh nervetoseventh nerve transfer (one
case) and minitwelfth nervetoseventh nerve
transfer (one case) was carried out to strengthen
the eye sphincter. For the direct neurotization of
the orbicularis oculi muscle, the upper cross-facial
nerve graft that carried eye fibers was used. Ten
of these 21 patients with inadequate outcomes
were complemented with ancillary procedures:
gold weight (n 3); gold weight and minitendon
(n 1); eye spring (n 2); minitendon and
wedge excision (n 1); minitendon (n 5);
minitendon, gold weight, and eye spring (n 1);
and wedge excision (n 2).
Finally, four patients were managed with static
procedures (minitendon and wedge excision (n
1) or minitendon (n 3). In addition, lateral
canthoplasty was needed in four patients, and medial canthoplasty was needed in seven cases. Overall, patients presenting with inadequate eye closure required from two to four procedures,

Table 4. Main Reconstructive, Ancillary, and Aesthetic Procedures


Reconstructive Options
Target: upper face
Pedicled frontalis transposition
Free platysma transfer
Free split pectoralis
Pedicled temporalis transposition
Direct neurotization
VIIVII transfer
XIIVII transfer
Target: middle face
Free pectoralis
Free gracilis
Free serratus anterior
Target: lower face
Digastric transfer
Platysma transfer
Sternocleidomastoid transfer
Direct neurotization

No.
5
2
2
1
9
1
1

Ancillary Procedures

No.

Aesthetic Revisions

Gold weight
Eye spring
Minitendon to lower eyelid

5
3
12

Webbing correction
Brow lift
Blepharoplasty

20
16
1

Mini-temporalis to augment smile


Platysma transfer for excess pull
Plication of levators
Conchal cartilage graft to nasal alar
V-Y alar base

10
2
3
9
15

Vascularized fascia flap


Debulk cheek/free muscle
Face lift
Rhinoplasty
Otoplasty

10
2
1
3

Selective myectomy contralateral depressor

Defat neck
Dermal graft to chin
Chin implant

No.
3
5
7

6
17
4
3
4
3
3
1

71e

Plastic and Reconstructive Surgery January 2012

Fig. 1. Example of pedicled frontalis for eye sphincter augmentation.


This 10-year-old girl presented with left developmental facial paralysis along with multiple developmental defects: cleft lip cleft palate,
bilateral hearing loss and external ear deformities, aberrant right subclavian artery, left spinal accessory nerve deficit with weak shoulder,
and neck webbing. She underwent her first stage of facial reanimation when she was 10 years old with three cross-facial nerve
grafts. The upper cross-facial nerve graft was coapted to the upper
zygomatic branch of the right facial nerve. Fifteen months later, in
the second stage, a right free pectoralis minor was transferred to
the left cheek. Seventeen months later, she had a right pedicled
frontalis transfer for augmentation of her left eye sphincter. The
distal end of the upper cross-facial nerve graft was coapted to the
nerve of the right frontalis. (Above) The patient is shown attempting
eye closure at her first office visit. (Below) The patient demonstrates eye
closure during her final visit. The panel graded her preoperative eye closure as 3 (moderate) and 4 (good) at her final visit (Table 2, patient 14).

(median, two). The aesthetic procedures most


commonly performed in the upper face were brow
lift (n 5), blepharoplasty (n 7), and webbing
correction (n 3).
Midface
Primary Dynamic Procedures
The main strategy for smile restoration consisted of cross-facial nerve grafting followed by
a free-muscle transfer in a second-stage [37 pa-

72e

tients (88 percent)]. Gracilis muscle was used in


16 patients (38 percent) and the pectoralis minor was used in 20 patients (47.6 percent) (Figs.
2 and 3). Excessive free-muscle contraction was
observed in three patients. One patient was
managed with selective myectomies and two patients underwent platysma transposition to the
contralateral commissure to strengthen the oral
sphincter and offset powerful overcontraction
of the muscle.

Volume 129, Number 1 Developmental Facial Paralysis

Fig. 2. Example of free pectoralis minor for smile restoration.


This 2-year-old girl presented with right developmental facial paralysis (appearance at her first office visit). At the first stage of
facial reanimation, when she was 3 years old, she underwent two
cross-facial nerve grafts, and at the second stage, 14 months later,
a pedicled left frontalis was transferred to her right upper and
lower eyelid, to augment her eye sphincter. Subsequently, the
left pectoralis minor was transferred to her right cheek. She had
multiple revisions, including right upper blepharoplasty and canthoplasty, free conchal cartilage graft to the right ala, V-Y advancement of the right alar base, myectomy to the left depressor,
and bilateral otoplasty.

Secondary Refinements
Asymmetry caused by excessive cheek volume
required debulking of the muscle graft in two
cases or defatting of the cheek in 15 cases. The
senior author never excises the buccal fad pad or
subcutaneous fat routinely. Instead, in developmental facial paralysis, a relatively thick flap of skin
and subcutaneous tissue at the level of the superficial musculoaponeurotic system is raised. If debulking is needed, the buccal fat pad and/or subcutaneous fat is removed during secondary
revision as needed.
Cheiloplasty for correction of the upper lip
inversion deformity was performed in six cases.
Contour deficits and problems of skin tethering
were treated with a pedicled superficial vascularized temporal fascia flap6 or a free posterior calf
vascularized fascia flap.1 Nasal deformity and alar
collapse were addressed with free conchal cartilage grafts in nine cases, whereas 15 V-Y advancement flaps were used for repositioning of the alar
base in cases with minor deformity.
Lower Face
Although 21 of 42 patients (50 percent) had
an effective depressor mechanism, 13 (30.9 percent) required muscle substitution by an anterior
digastric (n 10), ipsilateral pedicled platysma

Fig. 3. Same patient as shown in Figure 2. The patient is shown in repose (left) and during
smiling (right) at her last office visit, 18 years after the onset of her reanimation operations.
The panel graded her result as 1 (poor) preoperatively and 4 (good) at her last office visit
(Table 2, patient 1).

73e

Plastic and Reconstructive Surgery January 2012


(n 2), or sternocleidomastoid pedicled transfer
(n 1) (Fig. 4).
Function was augmented by means of direct
neurotization in three patients, with increased
electrical activity on electromyography but inadequate lower lip excursion. One of the platysma
transfer cases had inadequate outcome, and symmetry was achieved with contralateral myectomy
of the depressor.

Five patients were managed with contralateral


myectomy only.
All of the patients had a mean follow-up of
8 6.3 years, with a range of 1 to 23 years. Overall,
all of the patients improved their outcome scores,
as is demonstrated in Tables 5 through 7. There
was also statistically significant improvement in
the long-term electromyographic scores as compared with the preoperative scores in children and
in adults (Table 8). The results of the final assessment were better compared with the 2-year assessment, but statistically significant improvement was
evident only in the long-term results of the pediatric population (Table 9).
For each type of reconstruction, a percentage
gain score was calculated comparing the preoperative and the final follow-up scores (Table 6).
The pediatric population achieved better scores
compared with the adults (Table 7). It is generally
accepted that the complexity of these multistage
reanimation procedures requires multiple revisions and refinements. This was also true in our
series.
There was a free flap loss in an adult caused by
venous thrombosis. A new free gracilis flap was
performed 1 year later. Final outcome was satisfactory. Representative clinical examples are demonstrated in Figures 5 through 8.

DISCUSSION

Fig. 4. Example of ipsilateral pedicle sternocleidomastoid transfer for depressor restoration. A 4-year-old girl with left developmental facial paralysis (left greater than right), multiple birth
defects, including cerebral palsy, left enophthalmos, left plagiocephaly, and involvement of multiple cranial nerves: left fifth
(weak masseter), sixth (absence of lateral gaze), and left eighth
(hearing loss). She underwent four cross-facial nerve grafts and,
11 months later, had a right free platysma microneurovascular
transfer to her left eye and, 17 months later, a right free pectoralis
minor for smile. Her depressor function was restored with a pedicled left sternocleidomastoid transfer (neurotized by the lowest
cross-facial nerve graft) as, on exploration, both the left platysma
and digastric muscles were identified as hypoplastic and did not
respond to electrical stimulation. Appearance of her lower lip depressor is shown at the first office visit (above) and at her final
follow-up, 15 years later (below). The panel graded her lower lip
depression as 1 (poor) preoperatively and 5 (excellent) at her last
office visit (Table 2, patient 34).

74e

Studies of the management of pediatric facial


paralysis have been limited,11,23,24 and the lack of
universal assessment and evaluation tools makes
the comparisons of outcomes very difficult. Ysunza
et al.25 reported excellent results in the treatment
of facial paralysis in a group of 18 patients with
hemifacial microsomia with cross-facial nerve
grafting and direct neurotization of the facial muscles. They documented that patients younger than
1 year had the best outcomes and they recommended early surgical intervention to prevent further facial muscle degeneration.
The question of when facial muscles have
reached the state of degeneration or atrophy at
which adequate movement does not return after
reinnervation has historically been approached
from two different directions. Some feel that time
delay factors prevail, whereas others, including
our center, are more concerned about the detailed preoperative needle electromyographic and
nerve conduction findings, regardless of the time
since facial involvement.
The decision on how to treat a patient with
facial paralysis is therefore based on an accurate

Volume 129, Number 1 Developmental Facial Paralysis


Table 5. Averaged and Median Scores of Outcomes Given by the Three Assessors

Case
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25
26
27
28
29
30
31
32
33
34
35
36
37
38
39
40
41
42

Preoperative
Mean

Preoperative
Median

1.33
2.22
3
4
2.66
2.55
1.55
2.77
3
1.166
2
3.88
3
2.22
2.22
1.44
2
1.88
3.11
2
2.88
2.88
1.77
3.55
1.33
1.77
2.11
2.77
3.55
3.33
2.44
1.88
3.66
1.67
3.66
3.55
2.44
2.55
3.55
2.11
1.44
1

1
1
2.50
4
1.50
1
1
3
2
1
1
4
3
3
1
1
1
1
3
1
4
3
1
5
1
1
2
2
4
4
1
1
4
1
4
5
1
2
5
3
1
1

2-Year
Postoperative
Mean

2-Year
Postoperative
Median

Final
Postoperative
Mean

3.55
3.55

4
3

4.44

3.88
2.44
3.77
4
3.66

4
2
4
4
4

4.66
4.66
3.22

5
5
4

2.88
3.44
3.44
4.66
3.33
3.88
4
3.55
4
3.66
3.55
3.33
3.66
4.16
4.44
3.55
3.22
4.44
3.22
4.44

3
4
4
5
4
4
4
4
5
3
4
3
4
4
4
4
3
5
3
5

4.55
3.66

5
5

3.44
3.33

4
3

3.88
3.33
4.33
4.333
4.66
4
3.88
5
4.11
4.11
4
4.44
4.66
3.16
4
3.77
4.11
4
4.22
3.66
4.44
4.33
4.33
4.50
4.22
4.11
4.11
4.33
4.66
4.77
3.77
4.11
4.44
3.67
4.44
3.44
5
4
4
3.83
3.33
3

Final
Postoperative
Median
4
4
4
5
4
4
4
5
5
5
4
4
3
5
4
4.50
4
5
5
5
5
4
5
4
5
5
5
4
4
5
4
5
5
5
5
5
4
3
3

Table 6. Gain Percentage with Different Target and Procedures


Target

Group Size (no.)

% Gain Score (CI) Preoperative/Final

5
1
2
2
8

36.6 (13.959.3)
70 (57197)
53.3 (4110)
50
26.9 (12.541.2)

0.0058*
0.0903
0.0572
0.3440
0.0015*

16
20
1

48 (37.658.4)
54 (45.662.4)
33.3 (4.662)

0.0001*
0.0001*
0.0377*

10
2
4
5

30.8 (457.7)
33.4
24.4 (0.649.5)
44.6 (29.459.9)

0.0275*
0.1242
0.0524*
0.0007*

Procedures for eye


Pedicled frontalis transposition
Pedicled temporalis transposition
Free platysma transfer
Free split pectoralis transfer
Direct neurotization or VIIVII transfer
Procedures for smile
Free gracilis muscle transfer
Free pectoralis minor muscle transfer
Free serratus anterior muscle
Procedures for depressor
Pedicled digastric transposition
Pedicled platysma transposition
Direct neurotization or VIIVII transfer
Contralateral myectomy

CI, confidence interval.


*Statistically significant result, p 0.05 (Mann-Whitney test).
Nonsignificant (p 0.05).

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Plastic and Reconstructive Surgery January 2012


Table 7. Gain Percentage with Different Targets in Children versus Adults
Children

Eye closure
Smile
Depressor

Adults

Median Score
Difference (% Gain)

Confidence
Interval

Median Score
Difference (% Gain)

Confidence
Interval

1.03 (19.4)
2.45 (49)
1.63 (32.6)

0.461.60
2.072.82
1.182.08

0.003*
0.0001*
0.0001*

0.67 (13.3)
2 (40)
1.48 (29.5)

0.531.86
0.483.51
0.042.91

0.2475
0.0141*
0.0443*

*Statistically significant result, p 0.05 (Mann-Whitney test).


Nonsignificant (p 0.05).

Table 8. Overall Electromyographic Outcomes in Children Compared with Adults


Preoperatively/2 Yr
Postoperatively

Children
Adults
All patients

Preoperatively/Final
Postoperatively

2 Yr Postoperatively/Final
Postoperatively

% Gain Score (CI)

% Gain Score (CI)

% Gain Score (CI)

27.5 (1936.5)
18.5 (3.337.4)
26.1 (18.333.8)

0.001*
0.05
0.001*

31.1 (21.940.3)
20.5 (0.6740.4)
29.1 (2137.3)

0.001*
0.05
0.001*

3.33 (6.413)
2 (18.522.5)
3.1 (5.511.7)

0.05
0.05
0.05

CI, confidence interval.


*Statistically significant result, p 0.05, Kruskal-Wallis test (nonparametric analysis of variance).
Nonsignificant (p 0.05).

Table 9. Overall Video Evaluation Outcomes in Children and Adults


Preoperatively/2 Yr
Postoperatively

Children
Adults
All patients

Preoperatively/Final
Postoperatively

2 Yr Postoperatively/Final
Postoperatively

% Gain Score (CI)

% Gain Score (CI)

% Gain Score (CI)

37.3 (25.948.7)
28 (9.765.7)
35.4 (24.246.6)

0.001*
0.05
0.001*

47.4 (3658.7)
31.4 (2.965.8)
44.6 (33.655.5)

0.001*
0.05
0.001*

10 (1.6221.7)
3.4 (34.2841.14)
9.2 (2.320.6)

0.05
0.05
0.05

CI, confidence interval.


*Statistically significant result, p 0.05, Kruskal-Wallis test (nonparametric analysis of variance).
Nonsignificant (p 0.05).

diagnosis of facial muscle viability and a realistic


assessment of anticipated functional facial movement after reinnervation. Our experience with
direct muscle neurotization in the adult and pediatric facial paralysis population has been published before,26,27 and we agree that there is a role
for this procedure in developmental facial paralysis in certain cases.
In addition, we recommend the babysitter
procedure (mini-hypoglossaltofacial nerve transfer) that was introduced by Terzis in 1984, as a
technique of rapidly providing strong neuronal
input to the denervated muscles while the contralateral facial nerve fibers are regenerating
through the cross-facial nerve grafts. The wisdom
of proceeding with the babysitter procedure, in
these cases, is that there is a possibility that some
function might return from the powerful ipsilateral donors that will make secondary surgery more
successful.28 Although it is difficult to compare outcomes of the different techniques used because of

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the small sample sizes, some important points


should be highlighted.
Upper Face
Despite previous reports of synkinesis with the
use of split-muscle techniques for eye-smile
reanimation,29,30 two patients with a free split pectoralis muscle in this series had excellent results;
however, the upper slip of the pectoralis in both
cases was neurotized separately by the upper crossfacial nerve graft carrying eye fibers from the
contralateral facial nerve. Evidence of successful
reanimation of the eye sphincter with free platysma or direct neurotization has also been documented in this series.
Middle Face
Free pectoralis muscle for smile reanimation
achieved higher percentage gain scores compared
with the free gracilis, but the difference was not

Volume 129, Number 1 Developmental Facial Paralysis

Fig. 5. A 13-year-old girl presented with left developmental facial paralysis. (Left) Appearance and smile at her first office visit are shown. She underwent three cross-facial nerve
grafts, and in the second stage, 12 months later, the right pectoralis minor was transferred
to her left cheek. A third stage was performed 13 months later, when she had transfer of a
mini-temporalis to the left commissure for smile augmentation, debulking of her left cheek,
and myectomy of the right lower lip depressor. Final revisions were performed 13 months
later and included repositioning of the left alar base with a V-Y advancement flap, liposculpturing of her left cheek, and revision of the preauricular scars. The patient is shown at her
last office visit displaying a full dental smile (right). The panel graded her smile as 2.5 (fair)
preoperatively and 4 (good) at her last office visit (Table 2, patient 3).

significant. The fact that the free pectoralis was


used in children and the free gracilis muscle was
mainly used in adults might have also influenced
the outcomes.
Lower Face
Although our preferred method of depressor
restoration is the pedicled platysma transfer, when
feasible, it appears that the pedicled digastric
transposition and the direct neurotization techniques can achieve equally satisfactory outcomes
in selected cases. In summary, the critical assessment of developmental facial paralysis and associated craniomaxillofacial defects is essential for
an adequate approach to reconstruction. The superior results of free muscle transfer in children
with facial paralysis and comparison with those
involving adults are demonstrated, as has been
previously reported.3,5,22,23
The remarkable improvement in facial aesthetics and function following reanimation procedures, especially in the pediatric group, has

been evidenced. However, patients with developmental facial paralysis should be offered an
individualized approach and tailor-made
treatment strategies based on the cause and the
presence of various stigmata and associated
anomalies with functional sequelae. The different aspects of facial paralysis are interrelated
and have to be treated almost simultaneously
because the treatment modalities reinforce each
other.4,19
Another important concept in evaluating
these procedures in the pediatric population is the
expanding skeleton of the face.22 In our population, the children demonstrated stable outcomes
at the long-term follow-up, without any aesthetic
or functional downgrading attributable to skeletal
changes with growth.22
In a previous study22 that had a follow-up of
nearly 20 years, it was shown that the relation
between transferred muscle and recipient cheek
remained unchanged, despite an expanding skeleton. With the advent of magnetic resonance imaging and the discovery of genes directing brain-

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Plastic and Reconstructive Surgery January 2012

Fig. 6. Example of a split pectoralis minor transfer for eye sphincter substitution and smile restoration in a
3-year-old girl with left developmental facial paralysis. Appearance at her first office visit while attempting to
smile (left) and close her eyes (above, right) is shown. She underwent three-cross facial nerve grafts, and in the
second stage, 12 months later, she had a free right pectoralis minor transfer for eye closure and smile restoration.
In this case, the upper slip of the pectoralis minor muscle was used for eye sphincter substitution and was
innervated by the upper cross-facial nerve graft that was coapted to upper zygomatic fibers for the eye of the
right facial nerve, and the lower slips were innervated by the middle cross-facial nerve graft that was coapted
to smile fibers of the right facial nerve. A third stage of revisions was performed 13 months later, when she had
transfer of the anterior belly of the digastric muscle to the lower lip for restoration of depressor function,
debulking of the left cheek, repositioning of the left alar base with a V-Y advancement flap, and left medial
canthoplasty. The patient is shown here 5 years after the split pectoralis minor muscle. Note improved closure
of left eye sphincter achieved from the upper slip of the free pectoralis minor muscle that is innervated by the
upper cross-facial nerve graft, thus yielding coordinated eye closure and blink (below, right) (Table 2, patient 17).

stem formation, a more coherent clinical picture


of the developmental disorders is emerging. The
clinicians will be able to approach them with a
framework of proper evaluation, management,
and counseling.31
As has been previously stated, there is a need
to provide a more comprehensive service for people with disfigurements over and beyond that of
surgical intervention. Because of the vulnerability
of facial paralysis pediatric patients and the inequality of the physician-patient relationship, care
should be taken to use this power in positive
ways.1,4 Physicians should demonstrate interest,
sensibility, and acceptance of the individual and

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recommend attention to all important concerns


and issues.4

CONCLUSIONS
As children are quite capable of central reorganization and establishment of substitution
patterns,3234 early targeted screening and diagnosis followed by prompt treatment of developmental facial paralysis promises further benefits that
will diminish the sequelae of associated stigmata
and allow for a smooth emotional and social
development and reintegration among their
peers.1,4,35,36 The experience of this center should
serve as a framework for the establishment of ac-

Volume 129, Number 1 Developmental Facial Paralysis

Fig. 7. Same patient as shown in Figure 6. At her last office visit, 23 years later, her appearance at rest (left) and during smiling (right) is shown. The panel graded her preoperatively
as 2 (fair) and 5 (excellent) at her last office visit (Table 2, patient 17).

Fig. 8. A 51-year-old woman presented with right developmental facial paralysis (left,
smile at her first office visit). She underwent three cross-facial nerve grafts and, 18 months
later, in the second stage, a left free pectoralis minor muscle was transferred to her right
cheek. She had subsequent revision operations that included debulking of the right
cheek, repositioning of the right alar base, bilateral blepharoplasties, right medial canthoplasty, right eyebrow lift, and bilateral miniface lifts. (Right) The patient is shown
smiling at her last office visit. The panel graded her preoperative appearance as 1 (poor)
and her final postoperative appearance as 5 (excellent) (Table 3, patient 3).

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Plastic and Reconstructive Surgery January 2012


curate and reliable guidelines that will facilitate
early diagnosis and management of developmental facial paralysis while providing support and
counseling to the family.
Julia K. Terzis, M.D., Ph.D.
International Institute of Reconstructive Microsurgery
27-28 Thomson Avenue, Suite 620
Long Island City, N.Y. 11101-2942
mrc@jkterzis.com

PATIENT CONSENT

Patients or parents or guardians provided written


consent for the use of the patient images.
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