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Management of Developm ental

symmetrical Facial Growth

Birte Prahl Andersen an d Clara E. Fischer

Several aspects of the management of developmental asymmetrical facial

growth are addressed, The abnormality is further defined, Methods of

exam ination and assess ment of records are discussed, A complicating factor

has been the adoption of too many classification systems. With three-

dimensional imaging techniques computed tomog raph y scan and stereopho-

tography) great advancement has been made in efforts at describing the

range of variation, Hemifacial microsomia patients are best treated in

multidisciplinary centers by com pete nt specialists with t he necessary exper-

tise and skills, The procedure followed in the craniofacial center in Rotter-

dam is described and discussed in relation to current treatment strategies,

The success of the tre atm ent of the asymm etrical facial growth depends on

the original abnormality, on secondary abnormal development, and on

orthodontic and surgical intervention, International cooperation is necessary

to compile sufficient statistical data for a scientific evaluation of treatment

results and to improve the effectiveness and the efficiency of treatment,

Semin Orthod 1996;2:64-83.) Co p y r i g h t © 19 96 b y W B Sa u n d e r s Com p a n y

n u n d e r s t a n d i n g o f c r a n i o fa c i a l g ro wt h h a s

a lwa ys p o s e d p ro b l e m s , a n d d e v e l o p m e n t a l

a b n o rm a l i t i e s fu r t h e r c o m p l i c a t e s t h e u n d e r -

s t a n d i n g o f t h e s e g ro wt h p ro c e ss e s . T h e g ro wt h

p ro c e s s e s a r e n o t a s i m p l e c o o rd i n a t e d wh o l e ,

b u t r a t h e r i t s e e m s a s if m a n y c r a n i a l a n d f a c ia l

s t r u c t u re s i n t e r a c t a n d u n d e r g o d i f f e r e n t ia l

g ro wt h e i t h e r s y m m e t r i c a l ly o r a s y m m e t ri c a l ly .

T h e r e is n o d o u b t t h a t t h e c e ll s g e n o m e

c o n t a i n s s p e c i f i c i n s t ru c t i o n s t h a t d i c t a t e t h e

p a t t e r n o f g ro wt h, b u t c e l l s a r e a l s o r e s p o n s i v e t o

e p i g e n e t i c a n d e n v i ro n m e n t a l f a c t o r s . T h e p ro -

p o r t i o n o f g ro wt h t h a t is g e n e t i c a l l y d e t e rm i n e d

o r e n v i ro n m e n t a l l y c o n t ro l l e d i s n o t k n o wn , b u t

i t i s t h e i n t e rp l a y t h a t a c c o u n t s fo r n o rm a l

v a r ia t io n s a n d f o r a b n o r m a l d e v e l o p m e n t .

Ve ry f e w c ra ni o fa c i a l m a l fo rm a t i o n s c a n b e

p r e v e n t e d b a s e d o n k n o w l e d g e o f t h e i r e t i o lo g y

From the Academic Centre of Dentistry Amsterdam Department

of Orthodontics; and the Orthodontic Department University Hospi-

tal Rotterdam The Netherlands.

Address correspondence to B. Prahl-Andersen PhD DD S Aca-

demic Centre of Dentistry Amsterdam Department o f Orthodontics

Louwesweg 1 1066 EA Amsterdam The Netherlands.

Copyright © 1996 by W.B. Saunders Company

1073-8746/96/0202-000555. 00/0

o r p a t h o g e n e s i s . On t h e o t h e r h a n d , t h e i d en t if i -

c a t i o n o f c au s a l m e c h a n i s m s o f c r a n i o fa c ia l m a l-

fo rm a t i o n s m a y p l a y a ro l e in t h e fu t u re i n t h e

c l in i ca l m a n a g e m e n t o f m a l f o r m a t io n s .

W h e n c o n s i d e r i n g a s y m m e t r i e s a r i s i n g i n

c ra n i o fa c i a l d e v e l o p m e n t , t h e m o s t s i g n i f i c a n t

a s y m m e t r i c a l m a l fo rm a t i o n i s h e m i fa c i a l m i c ro -

s o m i a o r o t o m a n d i b u l a r d y so s to s is , a l so r e f e r r e d

t o a s t h e F i r s t a n d S e c o n d B ra n c h i a l Arc h S y n -

d r o m e . T h i s n o m e n c l a t u r e i n c l ud e s a n u m b e r o f

c o n d i t i o n s ; h o w e v e r , a l l s h o w a s y m m e t r i c a l

g r o w t h o f t h e m a n d i b l e . D u r i n g d e v e l o p m e n t it

c a n b e d i f f i c u l t t o d i s c r i m i n a t e b e t we e n e i t h e r

h y p e rp l a s i a o r h y p o p l a s i a o f o n e s i de o f t h e

m a n d i b l e . I t i s i m p o r t a n t t o c o n s i d e r t h e d i f f e r -

e n t i a l d i a gn o s i s o f h e m i fa c i a l m i c ro s o m i a f ro m

o t h e r f a c i al a s y m m e t r i c a l a b n o rm a l i t i e s , b e c a u s e

t h e t r e a t m e n t w i l l i n m o s t c a s e s , b e d i f f e r e n t .

Ap p ro p r i a t e e x a m i n a t i o n a n d o b s e rv a t i o n s h o u l d

a l l o w fo r t h e c o r r e c t d i a g n o s i s . T h e fo l l o wi n g

m o s t c o m m o n c o n d i t i o n s a r e il l u st r a te d : h e m i fa -

c ia l microsomia (F ig 1 ) , p lag iocephaly (F ig 2 ) ,

h y p e rp l a s i a o f o n e c o n d y l e (F ig 3 ), a n d P a r ry -

R o m b e rg s s y n d ro m e , p ro g re s s i ve h e m i fa c i a l a t -

ro p h y (F ig 4 ) .

I n o n e f o r m o r a n o t h e r a s y m m e tr i c al g r o w th

64 Seminar s in Orthodontics, Vol 2, No 2 June ), 1 996: pp 64 -83

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SNA : 79 ° (83 ° )

' l/ J 9 -: -

Figure 1. (A) Illustrat ion of a patient with hemifacial microsomia. Note the asymmetrical position of the lower

jaw and the devia ted chin to the affected left side. (B) Profile view of this patient. Note the deformed ear on the

affected left side. (C) Profile headfilm o f the patient (1A and B). (D) Tracing of the profile headfilm of the

patient (1A-C). All measurements are smaller than normal measurements (shown in brackets).

of the mandi bular occurs in one of 3,000 births.

The etiology of hemifacial microsomia is un-

clear. A theory of mesodermal deficiency has

been suggested, as has a theory of a vascular

defect or circula tory deficiency. Poswillo 1 sug-

gested that the theory of a vascular defect was

plausible. The varying involvement of the differ-

ent structures suggests that the causal factor

varies in intensity and that it is active at several

periods during prenatal development. The

anomaly often extends beyond the mandible and

both primary and derived defects are observed

in the form o f deviation in the outer and middle

ear, affected malar region, orbit, frontal region,

maxilla, and many of the associated bony and

soft tissues, such as cleft palate. Th e g eneral ter m

craniofacial microsom ia has been propos ed by

Mun ro 2 to describe the severe forms. Th e final

clinical appearance of a patient who has not

undergone surgery with hemifacial microsomia

depends on the initial defect and the subsequent

secondary abnormal development.

M e t h o d s o f Ex a m in a t io n an d A s s e s s m e n t

of Records

To assure and improve quality care of chil dren

with asymmetrical facial developme nt, these chil-

dren are best treated in multidisciplinary cranio-

facial centers. In these centers a sufficient num-

ber of abnorm al children should be observed

and treated to ensure that competent specialists

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66

Prahl Andersen a nd F ischer

Figure 2. A) Illustration of a patien t with a plagiocephaly. Uneq ual g rowth of the right and left sides has the

effect of produc ing u pp er face deviation to the righ t the side of the stenosis) and low er face deviation to the left .

B) Illustration of the dentition in occlusion. Note the crossbite on the right side. C) Three-dim ensional

reconstruction of the right and left side of the skull of the patient. Note the insufficient posterior growth

transla t ion of the r ight tem porom andibu lar ar t icula tion. D) Three-dimensional recons truct ion of the skullbase

of the patient.

h a v e t h e n e c e s s a r y t r a i n i n g , k n o w l e d g e , a n d

s ki ll s t o m a n a g e t h e s e c o m p l e x p r o b l e m s . E v a l u-

a t i o n , d i a g n o s i s , a n d t r e a t m e n t p r o t o c o l s s h o u l d

be ag ree d u po n by a l l spec ial i s ts i nvolved in the

t r e a t m e n t .

A c c o r d i n g t o R o s s 3 t h e r e s u lt s o f d y s m o r p h i c

g r o w t h c a n b e t h r e e f o l d , t h e s i t u a t i o n c a n i m -

p r o v e , b e c o m e w o r s e , o r s t a y t h e s a m e . T h i s

m e a n s t h a t r e g u l a r c h e c k - u p s a n d l o n g - t e r m

fol low-up of pa t i en t s a re neces sa ry . Th e fo l low-

i n g a g e s a r e p r o p o s e d a n d u s e d f o r f o l l o w - u p o f

p a t i e n t s i n t h e c r a n i o f a c i a l c e n t e r i n R o t t e r d a m :

4 , 6, 9 , 12, 15 , and 18 yea rs o f age . The ra t ion a le

f o r s e l e c t in g t h e s e t i m e s o f o b s e r v a t i o n i s b a s e d

o n g r o w t h , d e n t a l d e v e l o p m e n t , a n d p s y c h o s o -

c i a l p rogres s .

T h e f o l l o w i n g r e c o r d s s h o u l d b e c o l l e c t e d o n

a rou t ine bas i s : l a t e ra l sku l l r ad iograph t aken in

n a t u r a l h e a d p o s i t i o n , o r t h o p a n t o m o g r a p h

O P G ) , d e n t a l c a s t s , n o r m a l a n d s t e r e o - p h o t o -

g r a p h s F i g 5 ) . O b l i q u e h e a d f i l m s o r a n t e r o -

p o s t e r i o r h e a d f il m s m a y a ls o b e r e c o m m e n d e d .

P r e f e r a b l y , b e f o r e a n d a f t e r s u r g i c a l i n t e r v e n -

t i o n, C T s c a n s a n d t h r e e - d i m e n s i o n a l 3 -D ) i m a g -

i n g s h o u l d b e u s e d f o r t h e q u a n t i f i c a t i o n o f t h e

b o n e a n d m u s c l e t i s s u e p r e s e n t i n a n i n d i v i d u a l

c h i l d F i g 6 ) . F i n al l y a n E M G o f t h e m a s t i c a t o r y

musc les i s a va luab le record ing , re l a t ive to func -

t ion Fig 7) .

T h e s t a n d a r d m e t h o d o f t a k i n g a l a t e ra l sk u ll

r a d i o g r a p h u s i n g a c e p h a l o s t a t h a s l i m i t e d v a lu e

i n a s s e s s m e n t o f f a c ia l a s y m m e t r i e s f o r p a t i e n t s

w i t h c r a n i o f a c i al a b n o r m a l i t i e s . I t is r e c o m -

m e n d e d w h e n t a k i n g a l a t e r a l - s k u l l r a d i o g r a p h

f o r t h e p a t i e n t t o b e i n n a t u r a l h e a d p o s i t i o n . T o

a c h i e v e t h is , s p ec i f ic e q u i p m e n t i s r e q u i r e d t o

p o s i t i o n t h e p a t i e n t f o r t h e l a t e r a l h e a d p l a t e

Fig 8) .

A p r e r e q u i s i t e f o r t h e u s e o f t r a d i t i o n a l c e p h a -

l o m e t r i c r a d i o g r a p h s is a p p r o p r i a t e r e f e r e n c e

d a t a . L o n g i t u d i n a l g r o w t h c h a r t s o f d i f f e r e n t

r e l e v a n t c r a n i o f a c i a l m e a s u r e m e n t s a r e n e c e s -

s a ry for boys and g i rl s . Such da ta a re ava i l ab le

f r o m t h e N i j m e g e n g r o w t h s t u d y 4 a n d c a n b e

u s e d a s r e f e r e n c e d a t a f o r D u t c h c h i l d r e n l o n g i-

tud ina l ly F ig 9) .

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Figure 3. A) Illustration of a patient with hyperplasia of the right condyle. Note the deviation of the chin towards

the left, unaffected side. B) Illustration of the dentition in occlusion of the patient. Note the open bite on the

right side caused by ankylosis of the teeth on this side. C) Frontal section of a bone scintigraphy of the patient

shows more growth activity of the right condyle than of the left condyle. D) Illustration of the dentition in

protrusion. Abnormal positioning of the tongue and ankylosis of the teeth on the right side cause an unexpected

midline deviation.

Although the OPG cannot be used for mea-

surements, it is a useful recording because the

dental development can be easily followed and

the severity of the deformity can be approxi-

matel y assessed Fig 10). Th e den tal casts are

traditional records and can be used to monitor

the dental development and to record asymme-

tries in the dentition and occlusion. Photo-

graphs are important for the recording of exter-

nal deformities and should be taken in as

standar dized a man ner as is possible. However, if

reliably measurable data are wanted, stereopho-

tography is a sophisticated me tho d for the quan-

tification of the asymm etry of the face 5 Fig 5).

Ras et al 5 con clu ded fro m their study that indi-

viduals without craniofacial anomalies show a

facial left-sided dominance in the transverse

direction, and a right-sided dominance in the

sagittal direction. All faces are asymmetrical, the

level at which an asymmetry is unacceptable

depe nds on psychological factors. However, little

is known about the degree of asymmetry that is

acceptabl e to society at large.

Three-dimensional imaging constructed on

the basis of comp ute d tomogra phy CT) scans

gives an invaluable 3-D impression of the abnor-

mality. The latter is very important for the

surgical planning. An additional advantage is

also the ability to quantify the bony malforma-

tion as well as the effect of the treatment. This

method also provides valuable information rela-

tive to the volume of the muscles and as such is a

valuable research tool Figs 11-13). The treat-

ment of the bony deficiency will no t repair the

functional deficiency of the abnormal muscle

condi tion Fig 14).

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Prahl Andersen and F ischer

The Range and Variation

Developmental asymmetry of the face is charac-

terized by varying degrees of underd evel opme nt

of the craniofacial structures. As mention ed, the

majo r deficiency effects the mandible. Several

classifications have been proposed in the litera-

ture: Pruzansky, 6 Har vol d et al, 7 David et al, s

Munr o, 2 and Vento et al. 9 Munr o 2 divided pa-

tients according to the skeletal deformity into 5

types, a purely surgical-anatomic classification.

Pruzansky6 had earlier i ntr oduc ed a 3-point scale

of severity of the m andi bula r deficiency (Fig 10).

It seems that most of these commonly used

classifications are approximate because a spec-

trum o f other abnormalities are not taken into

Figure 4. (A) Illustrat ion of a patient with the Parry-

Romberg s syndrome, progressive hemifacial atrophy

of the right side. Note the slight deviation of the chin

to the right, affected side. (B) Illustration of the

denti tion in occlusion. Premature loss of the lower

first deciduous molar on the right side causes an

unexpected midline deviation. (C and D) Three-

dimensional reconstruct ion of the r ight and left sides

of the skull of this patient with progressive hemifacial

atrophy. (E) Illustration of the denti tion of the patient

on slight mouth opening showing no deviation of the

lower jaw.

account. Other bony anatomical structures in-

volved can be the zygoma, zygomatic arch, orbit,

frontal bone, pterygoid processes of the sphe-

noid bone, mastoid and tympanic process of the

temporal bone and the maxilla. For the abnor-

mality of the ears a separate scale has also been

propos ed by Pruzansky. 6 Photog raphs are used

to record this abnormality (Fig 15).

Nonbony structures affected can be the facial

nerve (obvious when the child is smiling), the

mouth, eyes, facial muscles, muscles of mastica-

tion, tongue, and parotid gland (Fig 16). It is

considered that with the more sophisticated

met hod of CT scans and 3-D reconstructions, a

better measurement of the total bony deficiency

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F i gur e 5 . A andB ) E x ampl e o f a pa i r o f s t e r eoph o t o -

g r aphs i nc l ud i ng t he r e f e r ence f r ames and po i n t s and

pr o j ec t i on o f a g ri d on t o t he f ace. C ) E qu i p men t f o r

s t e r eopho t ogr aphy cons i s t i ng o f t w o synchr on i zed

camer as f i xed on t o a f r ame w i t h a d i s tance o f 50 cm

be t w een t he camer as , and p os i t i oned conve r ge n t l y a t

an angle of 15 degrees . A f lash spot i s fas tened

be t w een t he camer as .

c a n b e o b t a i n e d . S t r u c t u r e s s u c h a s t h e p t e r y -

g o i d p r o c e s s a r e o f t e n a f f e c t e d t o g e t h e r w i t h a

h y p o p l a s t i c e x t e r n a l o r i n te r n a l p t e r y g o i d

mu sc l e , a0

I n r e v i e w i n g t h e s e r i e s o f p a t i e n t s w i t h h e m i f a -

c i al m i c r o s o m i a t r e a t e d i n t h e c r a n i o f a c i a l c e n -

t e r i n R o t t e r d a m n = 8 4 ) t h e a b s e n c e o f a n y

a p p a r e n t c o r r e l a t i o n b e t w e e n e x t e r n a l d e f o r m i -

t ie s, h y p o p la s t i c m u s c l e s a n d a c c o m p a n y i n g m a n -

d i b u l a r m a l f o r m a t i o n w a s s t r i k i n g . T h i s w a s a l s o

r e c o g n i z e d b y C o n v e r s e e t a l. n U n f o r t u n a t e l y

d a t a w a s n o t y e t a v a i l a b le a n d t h e r e f o r e a s tu d y

w a s u n d e r t a k e n t o o b t a i n d a t a . I n t hi s s t u d y t h e

v o l u m e a n d p o s i t i o n o f t h e i n v o l v e d m a s t i c a t o r y

m u s c l e s w e re m e a s u r e d i n p a t i e n t s w i th h e m i f a -

c i a l m i c r o s o m i a a n d i l l u s t r a t e d w i t h 3- D r e c o n -

s t r u c t e d C T s c a n s F i gs 1 1 -1 3 ). D i f f e r e n c e s b e -

t w e e n l e f t a n d r i g h t m a s t i c a t o r y m u s c l e s w e r e

i d e n t i f i e d a n d r e l a t e d t o t h e b o n y g r a d e o f

d e s t r u c t i o n . T h e r e a p p e a r e d t o b e n o l i n e a r

r e l a t i o n s h i p b e t w e e n t h e b o n y a n d t h e m u s c u l a r

u n d e r d e v e l o p m e n t . E v e n a m i n i m a l h y p o p l a s i a

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Prahl Andersen a nd F ischer

\4

Figure 6. (A) Horizon tal CT scan section of a pat ient with hemifacial microsomia. (B) Tracing o f the horizontal

CT scan sect ion. N ote the difference in s ize of the left and r igh t masseter muscles.

o f t h e m a n d i b u l a r r a m u s c a n b e a s s o c i a te d w i th

e x te n si v e u n d e r d e v e l o p m e n t o f t he m a s s e t er

m u s c l e .

F o r t h e o r t h o d o n t i s t t h e p r o b l e m s i n h e m i f a -

c i a l m i c r o s o m i a p a t i e n t s i n c l u d e d i s t a l o c c l u s i o n

c a u s e d b y l ac k o f m a n d i b u l a r g r o w t h , a s y m m e t r i -

c a l p o s i t i o n o f t h e l o w e r j a w , c h i n d e v i a t e d t o t h e

a f f e c t e d s id e , f l a t t e n e d b o d y o f t h e m a n d i b l e o n

t h e u n a f f e c t e d s i d e , t i l t ed o c c l u s a l p l a n e ( l o w e r

o n t h e u n a f f e c t e d s i d e a n d h i g h e r o n t h e a f -

f e c t e d s i d e ) , c r o s s - b i t e , c r o w d i n g ( a n d o t h e r

p r o b l e m s f o u n d i n p a t i e n t s w i t h i n d i c a t i o n s f o r

o r t h o d o n t i c t r e a t m e n t ) , i m p a i r e d f u n c t i o n o f

t h e e x t e r n a l p t e r y g o i d m u s c l e , a n d l i m i t a t i o n o f

R M S m a s s e t e r

R L

10 2

0.84

0.56

0.28

0.00

50%

10

I1

R M S t e m p o r a l

~ a L

lO 2

0.82

0.55

0.27

0 00

50%

lO

Figure 7. (A) EMG plots of the r ight and left masseter muscles of a pat ient with hemifacia l microsomia rec orded

at different bi te force levels ( percentage of maximal bi te force) . Note the difference in activity of the r ight an d left

masseter muscle especia l ly a t 50 of max imum bite force . The r ig ht masseter muscle (affected s ide) prod uces

considerable less act ivi ty than the left one. (B) EM G plots of the r ight a nd left temporal is muscle o f the same

patient. Th e right temp oralis musc le (a ffected side) prod uce s less activity than the left one.

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Figure 8. Without any f ixat ion of the pat ient s head, a

lateral skull x-ray is taken using a la serbeam projected

on the facia l midl ine and a mirror to control head

position.

p r o t r us i v e m o v e m e n t a n d l a te r al m o v e m e n t s

toward the a f fec t ed s ide (F ig 1) .

Growth Management and Treatment

oncepts

T h e g o a l o f t h e t r e a t m e n t i s t o i m p r o v e f a c i a l

s y m m e t r y a n d m a n d i b u l a r f u n c t i o n w h e n t h e

c r a n i o f a c ia l g r o w t h i s c o m p l e t e d , b u t i t i s i m p o r -

t a n t t o r e c o g n i z e t h a t t h e p s y c h o s o c i a l d e v e l o p -

m e n t o f a n i n d i v i d u a l c h i l d m a y s u ff e r f r o m a n

o b v i o u s a b n o r m a l i t y . T h e d e c i s i o n t o p e r f o r m

s u r g e r y o r n o t s h o u l d o n l y b e d i c t a t e d b y t h e

pos s ib i l it y o f an op t ima l long - t e r m resu l t . Pos t -

p o n e m e n t o f su r g e r y r e q u ir e s t h a t t h e p a t i e nt s

and the i r f am i l i e s a re ca re fu l ly and c l ea r ly in -

f o r m e d . B e c a u s e t h e o r t h o d o n t i s t i s u s u a ll y t h e

c o o r d i n a t o r a n d c o n t r o l s t h e c o l l e c t io n o f r e -

cords , a s we l l a s the reca l l sys t em, he or she

s h o u l d b e a b l e t o k e e p p a t i e n t s i n f o r m e d . I n

c a se s o f se v e r e p r o b l e m s t h e p s y c h o l o g i s t o f t h e

t e a m s h o u l d b e i n v o l v e d . T o o o f t e n s p e c i a l i s t s

a r e f o r c e d i n t o a c t i v e t r e a t m e n t w h e r e d e l a y

w o u l d h a ve b e e n t h e a p p r o p r i a t e m a n a g e m e n t .

T h e p u r p o s e o f th e t r e a t m e n t o f p at i e n ts w it h

h e m i f a c i a l m i c r o s o m i a i s t o i m p r o v e s y m m e t r y

o f t h e m a n d i b l e a n d a l so im p r o v e t h e m a x i l l a r y

de f i c i ency , t o re s to re the den ta l occ lus ion , t o

e x p a n d s o ft t is su e a n d t o l e n g t h e n t h e m a n d i b u -

l a r ramu s e i th e r surg ica l ly o r g radu a l ly (d i s t rac -

t i o n ) , o r g r a d u a l l y w i t h f u n c t i o n a l a p p l i a n c e s .

T h e r e is d e b a t e r e g a r d i n g t h e o p t i m a l t r e a t m e n t

r e g i m e n f o r p a t i e n t s w i t h h e m i f a c i a l m i c r o s o -

m i a . T h e r e a r e v a r y i n g o p i n i o n s r e g a r d i n g t r e a t -

d e g re e s

85

84

8 3 .

8 2 -

81

80

79

78

77

9

G: girls, sd = 4.6

B: boys, sd = 3.8

I .H. 15 10 75

G~ G ~ G~-'---'B--G - -

~ '- G -~ ._ .~ . . . B /~ e - - e /~

B

d e g re e s

7 8

7 7

76

75

7 4 '

7 3

7 2 -

71

70

I .H. 15 10 75

G: glr]s, sd = 4.3

B: bo ys, sd = 3.4

G G~

.B.~B~G...._B~G~ ~- ~B : --B~

~ o q

/o /

I

u ; u n I I ; I i ;

10 11 12 13 14 15 10 11 12 13 14

years years

5

Figure 9. (A) A longitudinal plot of the measu rem ents of the SNA angle of a patien t with hemifacial microso mia

comp ared with no rmal d ata from the N i jmegen Growth Study. (B) A longitudinal plot of the measu rements of the

SNB angle of the patie nt (A) com par ed with nor mal data.

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: , t- J,. a >> Hom e I TO C

72

Prahl Andersen and Fischer

O M.B. 4.3 years

14 6 91

M.A. 10.11 years

6 7 84

M.K. 4.6 years

6 3 84

Figure 10. A) Ortho pantomogram showing a Pruzansky I affected mandible with only slight hypoplasia of the

left condyle. B) Tracing of the orthopant omogram with Pruzansky I affected mandible. C) Orthopanto mogram

showing a Pruzansky II affected mandible with severe hypoplasia of the condyle and mild hypoplasia of the ramns

at the left side. D) Tracing of the ortho pantomogr am C) with Pruzansky II affected mandible. E)

Orthopan tomogram showing a Pruzansky III affected mandible with severe hypoplasia of the ramus and absence

of the condyle and the coronoid process on the right side. F) Tracing of the orthopant omogr am E) with

Pruzansky III affected mandible.

ment strategies with varying claims regarding

optimal treatme nt results.

The controversy centers arou nd timing and

mo de of inte rven tion in relation to age, severity,

and psychosocial considerations. The lack of

agreement can only be reduced by intercenter

studies or even better with a mnlticenter ra ndom-

ized clinical trial. This means international coop-

eration is needed because no single center has a

sufficient pool, finance, or facilities to carr y out a

trial individually. This orphan abnormality needs

international funding to be able to solve the

probl em o f arriving at the optimal treatme nt

strategy.

The controversy seems to be conce ntrate d

aro und the question of timing of surgery and

whether or not it is possible to stimulate growth

of the affected mandible with functional appli-

ances. Bone-lengthening by gradual distraction

is the latest proposal which is still in the experi-

mental stage, but it could be an excellent method

of correcti ng asymmetries of the mandible Fig

17). Some clinicians claim success with this new

methodology, but if appropriat e evaluation of

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/ ,V J,- ?:- >>


H o m e I T O C

7 3

F i gur e 11 . ( A ) P a t i en t w i t h a P r uzansky I a f f ec t ed man d i b l e ( r i gh t ) . ( B ) T hr ee - d i mens i o na l r econs t r uc t i on o f

t he sku l l o f the pa t i en t . ( C ) T h r ee - d i me ns i ona l r econ s t r uc t i on o f t he man d i b l e , t he m asse t e r musc l e s and t he

temp oral i s muscles , l a tera l r ight , and lef t view. Note s l ight hypo plas ia of these muscles on the r ight s ide .

( D ) T hr ee - d i mens i on a l r econs t r uc t i on o f t he mand i b l e , t he med i a l p t e r ygo i d musc l e s and t he l a t e r a l p t e r ygo i d

musc l es , an t e r i o r and pos t e r i o r v iew. N ot e s l i gh t hypop l a s i a o f t hese musc l e s on t he r i gh t s i de .

t h e m e t h o d o l o g y is n o t c a r r i e d o u t , i t w il l r e m a i n

e x p e r i m e n t a l a s d o s o m a n y o t h e r m e t h o d s o f

m a n a g e m e n t .

M u n r o z s t a te s t h a t t h e r u l e s h o u l d b e f i r s t

t h e b o n e , t h e n t h e s o ft t i s s u e . H e p r e f e r s e a r ly

i n t e r v e n t i o n a t 5 to 6 y e a r s o f a g e . H e f o u n d t h a t

u s i n g c o s t o c h o n d r a l g r a f t s r e s u l t e d i n le s s a s y m -

m e t r y a n d l e ss o c c l u s a l ti lt . I n s o m e c a s e s t h e r i b

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: , t- J,. a >> Hom e I TO C

74


Figure 12. A) Patient with a Pruzansky II affected mandible right). B) Three-dimensional reconstructi on of

the skull of the patient. C) Three-dimensi onal reconstructi on of the mandible, the masseter muscles and the

temporalis muscles, lateral right and left view. Note severe hypoplasia of the massete r muscle on the right side and

slight hypoplasia of the temporalis muscle on the right side. D) Three-dime nsional reconstructi on of the

mandible, the medial pterygoid muscles and the lateral pterygoid muscles, anterior a nd posterior view. Note

slight hypoplasia of these muscles on the r ight side.

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~,t- .t,. a >> Ho me I TO C


75

F i gur e 13 . A ) P a t i en t w i th a P r uzansky I I I a f f ec t ed mand i b l e r i gh t ) . B ) T hr ee - d i mens i o na l r econs t r uc t i on o f

t he sku l l o f t he pa t i en t . C ) T hr ee - d i men s i ona l r econs t r uc t i on o f t he mand i b l e , t he masse t e r musc l e s and t he

t empor a l i s musc l es , l a t e r a l f i gh t and l e f t v iew. N ot e cons i de r ab l e hypop l a s i a o f t he masse t e r musc l e a nd seve re

hypop l a s i a o f the t empor a l i s musc l e on t he f i gh t si de . D ) T hr ee - d i me ns i ona l r econs t r uc t i on o f the mand i b l e ,

t he med i a l p t e r ygo i d musc l e s and t he l a t e r a l p t e r ygo i d musc l e s , an t e r i o r and pos t e r i o r v iew. N ot e cons i de r ab l e

hypop l a s i a o f t he me d i a l p t e r ygo i d musc l e and seve re bypop l a s i a o f t he l a t e r a l p t e r ygo i d musc l e on t he r i gh t s i de .

o v e r g ro w s a n d d e f o r m s t h e f a ce i n th e o p p o s i t e

d i r e c t i o n . F o r t h e o r t h o d o n t i s t , t h e p r o b l e m i s a s

i t is i n c o n v e n t i o n a l o r t h o d o n t i c s . V a r g e r v i k 12

s t at e s t h a t w i t h o r t h o d o n t i c f u n c t i o n a l a p p l i -

a n c e s i t i s p o s s i b l e t o o b t a i n i n c r e a s e d l e n g t h o f

t h e m a n d i b l e a n d a s s o c i a te d m u s c l e s a n d o t h e r

s o f t t i ss u e s b e f o r e s u r g i c a l i n t e r v e n t i o n . T h e

t h e o r e t i c a l b a s i s f o r t h i s s t a t e m e n t i s s t i m u l a t i o n

o f b o n e g r o w t h b y i m i ta t i n g t h e f u n c t i o n o f t h e

p t e r y g o i d m u s c l e . T h i s t r e a t m e n t s t r a t eg y c an

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; , t- . t ,. a >> Hom e I TO C

76


Figure 14. A) Illustration of a surgically treated hem ifacial micro somia patient. The bon y deficiency has been

recons tructed. B) Pat ient wi th open mou th af ter surgery. Note that t reatm ent of the bony deficiency has not

correc ted the fu nctional deficiency.

a l so b e a p p l i e d a f t e r s u r g i c a l r e p o s i t i o n i n g o f t h e

m a n d i b l e . T h e a d v a n t a g e o f th i s s t r a t eg y is t h a t

s u r g e r y c a n b e p o s t p o n e d u n t i l 8 t o 9 y e a r s o f

age . A t th i s age the d en t i t i on i s i n the t ran s i t iona l

p h a s e w it h e n o u g h p e r m a n e n t t e e t h f o r f i x at i o n

a n d s u r g e r y is p e r f o r m e d i n a d e v e l o p m e n t a l

s t ag e w i th m i n i m u m g r o w t h o f t h e m a n d i b l e F ig

18).

T h e t wo p re v i ou s l y m e n t i o n e d t r e a t m e n t p h i -

losoph ies have ye t t o be t e s t ed a s to which re su l t s

a r e u l t i m a t e l y o p t i m a l . I n s e v e r e c a s e s a s e c o n d

o p e r a t i o n i s o f t e n n e c e s s a r y , p r e f e r a b l y a f t e r

g r o w t h h a s c e a s e d .

I t c o u l d b e a s O s b o r n e 13 h a s s u g g e s t e d t h a t

s u r g e r y b e f o r e 6 y e a rs o f a g e g i v e s t h e m a x i l l a a

c h a n c e t o d e v e l o p a f t e r th e r e l e a s e o f t h e u p -

w a r d p r e s s u r e e x c e r t e d o n t h e a f f e c t e d s i d e b y

t h e h y p o p l a s t i c m a n d i b l e . H o w e v e r , i t h a s t o b e

d e m o n s t r a t e d t h a t th e m a n d i b l e d o e s e x e r t p r e s-

s u r e o n t h e m a x i l l a . T h e m a x i l l a m a y b e d e f i -

c i e n t f r o m b i r t h a n d b y c r e a t i n g a n o c c l u s al g a p

a t t h e a f f e c t e d s i de , o v e r - e r u p t i o n o f t h e t e e t h i n

t h e m a x i l l a m a y fo ll ow . T h e t r a n s i t io n a l p e r i o d

a f t e r th e e a r l y m i x e d d e n t i o n s t a g e i s p r e f e r r e d

b y m a n y s u r g e o n s f o r t h e f i rs t c o r r e c t i v e s u r g e r y

b e c a u s e t h e r e a r e e n o u g h p e r m a n e n t t e e t h f o r

i n t e r m a x i l l a r y f i x a t io n , l e ss c h a n c e o f d a m a g i n g

t o o t h b u d s , a n d t h e o p e r a t i o n i s p e r f o r m e d i n a

d e v e l o p m e n t a l s t a g e w i t h m i n i m a l g r o w t h o f t h e

m a n d i b l e .

T h e t r e a t m e n t m e t h o d f o l l o w ed in R o t t e r d a m

h a s b e e n i n s p i r e d b y t h e m e t h o d o f H a r v o l d e t

a l, 70 bw eg ese r , a4 and Gnoinsk i . 15 At 4 yea rs o f

a g e t h e p r e v i o u s l y d e s c r i b e d r e c o r d s o f t h e

p a t i e n t a r e c o l l e c t ed . I f c o o p e r a t i o n c a n b e

o b t a i n e d , t h e p o s i t i o n o f t h e m a n d i b l e i s n o r m a l -

i z ed , w i t h r e g a r d t o t h e m i d l i n e a n d t h e h o r i z o n -

t a l p o s i t i o n . W i t h t h e f u n c t i o n a l a p p l i a n c e t h e

mandib le i s kep t i n a s l igh t ly fo rward pos i t ion .

T h e p u r p o s e i s t o o b t a i n a m o r e s y m m e t r i c a l

musc le func t ion and the pos s ib i l it y , by ad jus t ing

the ac t iva tor on the a f fec t ed s ide , t o a l low for

p a ss i ve e r u p t i o n o f t h e m a x i l l a r y b u c c a l t e e t h . A t

the ag e of 9 yea rs a dec i s ion i s ma de e i the r to

o p e r a t e o r t o w a i t u n t i l t h e e n d o f t h e g r o w t h

p e r i o d . A t t h i s a g e m e a s u r e m e n t s a r e m a d e a t

th ree d i f fe ren t t imes , and usua l ly a l so a s e t o f CT

s c a n s a r e m a d e . O n t h e b a s is o f t h is i n f o r m a t i o n

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: d-'J~- ?:- >>


H o m e I T O C

Figure 15. A) Ear abnormality, gradat ion I : s l ight hypoplas ia and bow led-form of oute r s t ructure of ear.

B) Gradat ion II : aplasia of external acoust ic tube and variable hypoplas ia of outer s t ructure . C) Grada t ion III :

dislocation o f ear-lobe, aplasia of oute r structu re, ear-lobe small, an d pl aced a nterior ly in most cases.

D) Gradat ion W: complete aplas ia of outer s t ruc ture and earlobe.

a n d d i s c u s s i o n s w i t h t h e p a t i e n t a n d t h e p a r e n t s

a n a g r e e d u p o n t r e a t m e n t g o a l is r e a c h e d .

I f s u r g e r y i s p l a n n e d , f i x e d o r t h o d o n t i c a p p li -

a n c e s a r e u s e d a s i n t e r m a x i l l a r y f i x a t i o n . A f t e r

s u r g e r y a n d t h e r e t e n t i o n p e r i o d t h e p a t ie n t s a r e

k e p t i n r e t e n t i o n w i t h a n a c t i v a t o r , a l l o w i n g f o r

f u r t h e r d e n t a l d e v e l o p m e n t . T h e d e f i n i t i v e o r t h -

o d o n t i c t r e a t m e n t is c a r r i e d o u t w it h f i x ed o r t h -

o d o n t i c a p p l i a n c e t h e r a p y a n d i f t h e s u r g e r y h a s

b e e n s u c c es s f u l, t h e r e t e n t i o n a p p l i a n c e m a y b e

a n a c t iv a t o r o r b o n d e d r e t a i n e r s F i gs 1 9 a n d 2 0 ) .

I n a f e w i n s t a n c e s t h e p a t i e n t s h a v e d e c l i n e d

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At- .t~ e >> Ho me I TO C

8


Figure 16. Illustration of different anatomical abnormalities in patients with hemifacial microsomia. A) Aplasia

of the facial nerv e on the right side. g) Macrostomia on the left side. C) Abnorm al and small eye, the hypoplasia

of the muscles of masticati on on the right side. D) Small eye with epi bulb ar der moi d and hypoplasia of the

muscles of mastication and the man dible on the right side Goldenha r syndrome).

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: - j - j , . ? .. >>


Home I TOC

9

Figure 17. (A) Schematic drawing of surgical opt ions f or pat ients with developm ental asymmetrical lower jaw.

Interpos i t ional bo ne graft in case of a funct ion ing join t or pseudo joint . (B) Ribgraft. (C and D) Dis tract ion

therapy, mode l surgery, i llus tra ted o n a 3-D reconstruct ion of the skul l of a pat ient with hemifacia l microsomia.

s u r g e r y f o l l o w i n g t r e a t m e n t w i t h f u n c t i o n a l a p p l i -

a n c e s ( F i g 2 1 ) . I n t h e s e s i tu a t i o n s t h e o c c l u s i o n

i s c o r r e c t e d w i t h fi x e d a p p l i a n c e s , a d a p t i n g t h e

o c c l u s i o n t o t h e s l ig h t a s y m m e t r y o f t h e l o w e r

j aw . T h e r e s u l t o f f u n c t i o n a l a p p l i a n c e t r e a t m e n t

104_

I 100.

96.

92

116_ _3.2

< - -X - - . 2 .4

12

: = =__x===v: -'x ; ¥. - -~ - . ,,

} 1 8

0 8 ~

0

0 8

years

Figure

18. Growth curves and velocity curves for the

mand ible, mea sur ed by the distanc e Ar-Gn. B, boys; G,

girls; x, girls velocity; y, boys velocity.

is o f t e n a t e m p o r a r y o p e n b i t o n t h e n o n a f f e c t e d

s ide (F ig 21D ) .

i s cuss i on

T h e s u c ce s s o f t h e t r e a t m e n t o f as y m m e t r i c a l

f a c ia l g r o w t h d e p e n d s o n t h e s e v e ri t y o f t h e

o r i g i n a l a b n o r m a l i t y . I m p o r t a n t f a c t o r s a r e t h e

l a c k o f s o f t t i ss u es , t h e i n v o l v e m e n t o f t h e m a x -

i l l a , t h e f r o n t a l b o n e a n d t h e o r b i t . T h e s e v e r e

a b n o r m a l i t i e s r e q u i r e e x t e n s i v e c r a n i o f a c i a l s u r -

g e r y . I f t h e s t r u c t u r e s a r e l e f t a l o n e a n d o n l y t h e

m a n d i b l e i s c o r r e c t e d , r e s u l t s i l l u s t r a te d i n F i g

2 2 A a n d C c a n b e e x p e c t e d . I n c o m p a r i s o n w i t h

t h e l a t t e r a le s s s e v e r e c a s e w i t h o u t o r b i t a l a n d

f r o n t a l i n v o l v e m e n t i s p r e s e n t e d i n F i g u r e 2 2 B

a n d D . T h e d e f i c i e n c y o f t h e m a x i l l a i n t h i s c a se

is c o m p e n s a t e d b y e x t r u s i o n o f m a x i l l a r y t e e t h

o n t h e a f f e c t e d s id e a n d n o r e c o n s t r u c t i o n o f t h e

s o f t t i s s u e d e f i c i e n c y h a s b e e n a t t e m p t e d .

T h e s ki ll o f t h e s u r g e o n a n d t h e o t h e r p r o f e s-

s i o n a ls i n v o l v e d in t h e t r e a t m e n t o f t h e s e p a -

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: , l - 'J~£ ~-- >> H o m e I T O

~ ~

Figure 19 . Show s the seq uence o f t rea tm ent in a g i r l w i th hem i fac ia l m icrosom ia: ear ly deve lopm ent func t iona l

appl ian ce used before surgery f ixed appl ian ce used after surgery and the end result .

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: , t- J,. a >> Hom e I TO C

F i g u r e 2 0 . A ) O P G o f t h e p a t i e n t s h o w n i n F i g 1 9 b e f o r e s u r g e r y . B ) O P G o f t h e p a t i e n t s h o w n i n F i g 1 9 a f t e r

surgery .

F i g u r e 2 1 . A ) I l lu s t r a t i o n o f a h e m i f a c i a l m i c r o s o m i a p a t i e n t w h o d e c l i n e d s u r g e r y , a t t h e a g e o f 4 y e a rs .

B ) P a t i e n t a t 6 y e a r s o f a g e . C ) P a t i e n t a t 9 y e a r s o f a g e a f t e r t r e a t m e n t w i t h a f u n c t i o n a l a p p l i a n c e . D )

D e n t i t i o n o f t h e p a t i e n t a t t h e a g e o f 9 y e a rs . N o t e t h e o p e n b i t e o n t h e n o n a f f e c t e d r i g h t s i d e as a r e s u l t o f t h e

t r e a t m e n t w i t h t h e f u n c t i o n a l a p p l i a n c e ,

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: , t- J,. a >> Hom e I TO C

8

Prahl Ander sen and Fischer

Figure 22. A) A patient with hemifacial microsomia. Note the cant of the mout h a nd the involvement of the

orbital region. B) A patient with hemifacial microsomia. Note that the c ant of the mouth and the involvement of

the orbital region is less when compa red with the pat ient A). C) Illustrat ion of the tr eat ment result of the

pati ent A). Only the mandib le was correct ed an d the orbital involve ment was left alone. D) Illustrat ion of the

treatme nt result of the patient B). Only the mandi ble was affected and corrected.

t ients are of para moun t i mpor tance. This means

a sufficient caseload, expertise, and interest in

craniofacia l problems concen tra te d in a mul t id is -

ciplina ry team. An efficient appr oach to the

treatment of craniofacia l malformat ions , on an

int erna tio nal basis , is lacking. No consens us on

classif ication, for ex ample, hemifacial microso-

mia, has been reached. This makes discussions

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At- .t~ e >> Ho me I TO C


8

on treatment indications and comparisons of

treatment results difficult. International coopera

tion is necessary to compile sufficient statistical

data for a scientific evaluation of treatment

results. This is the only way the quality of treat

ment results can be improved.

References

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Birth Defects 1975;11:61-81.

2. Munro IR. Treatment of craniofacial microsomia. Clin

Plast Surg 1987;14:177-186.

3. Ross RB. Lateral facial dysplasia first and seco nd bran-

chial arch syndrome, hemifacial microsomia). Birth

Defects 1975;11:51-59.

4. Prahl -Andersen B, Kowalski CJ, Heijdenda el P. A Mixed-

longitudinal Interdisciplinary Study of Growth an d Devel-

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dominance in cleft lip and palate: Three dimension

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dimens ional imagi ng in craniofacial surgery. World J

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11. Converse JM, Coccaro PJ, Becker M, et al. On hemifacial

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12. Vargerv ik K, Ous ter hou t DK, Farias M. Factors affecti ng

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ing ramus. BrJ Plast Surg 1964;17:376.

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oto-mandibular dysostosis. J Maxillofac Surg 1974;2:

73-92.

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sis and its conse quen ces for therapy. Trans Eur Orth od

Soc 1974:77-84.

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