Khe Hở Môi Và Vòm Miệng: Chẩn Đoán Và Quản Lý Điều Trị (Cleft Lip and Palate)

227
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_8, © Springer-Verlag Berlin Heidelberg 2013
8
8.1
Lip Pits
8.1.1 Pits of the Lower Lip in Cleft Lip
and/or Palate: Genetic
Considerations
Pits of the lower lip such as fi stulas of lower lip,
paramedian sinuses of lower lip, humps of lower
lip, or labial cysts are a very rare congenital mal-
formation, fi rst described by Demarquay in 1845
(Demarquay 1845 ) .
This minimally deforming anomaly is remark-
able chie fl y for its association with facial clefts.
The fact that clefts that occur with lip pits seem
to run stronger in families than clefts without lip
pits has attracted the attention of professionals
dealing with cleft patients (Figs. 8.1 , 8.2 , 8.3 ,
8.4 , and 8.5 ).
Lip Pits: Orthodontic Treatment,
Dentition, and Occlusion –
Associated Skeletal Structures
Samuel Berkowitz
S. Berkowitz, DDS, M.S., FICD
Adjunct Professor, Department of Orthodontics ,
College of Dentistry, University of Illinois ,
Chicago , IL , USA
Clinical Professor of Surgery and Pediatrics (Ret),
Director of Research (Ret),
South Florida Cleft Palate Clinic,
University of Miami School of Medicine ,
Miami , FL , USA
Consultant (Ret), Craniofacial Anomalies Program ,
Miami Children’s Hospital , Miami , FL , USA
e-mail: sberk3140@aol.com
a
b
Fig. 8.1 Lower-lip pits in a child with a bilateral cleft lip
and palate. ( a ) Before lip surgery. ( b ) After lip surgery at
6 months

228
S. Berkowitz
8.1.2 Frequency
No survey of lip pits has been carried out among
the general population; hence, the frequency of
this rare anomaly has been only roughly estimated
from its incidence in hospital records.
Assuming that 70 (Gorlin and Pindborg 1964 )
to 80 % (Van der Woude 1954 ) of patients with
pits of the lower lip have associated cleft lip and/
or palate and that the frequency of clefts is 1:650
(one in every 650 births), it can be estimated that
the frequency of lip pits among the general popu-
lation is about 1:75,000–1:100,000 (one in every
75,000–100,000 births).
8.1.3 Morphology
Fistulas of the lower lip usually appear as two
pits or humps on the vermilion portion of the
lower lip, generally equidistant from the midline.
Various kinds of asymmetry may be observed
with regard to the midline, or one pit may be
positioned more orally. Some pits are mere
depressions; others are channels 10–15 mm deep
with openings at the top of nipple-like elevations.
Some secrete small amounts of viscous saliva but
most are asymptomatic.
In exceptional cases, only one pit is pres-
ent, which may be located either centrally or on
one side or the other of the midline of the lower
lip. Some cases of single lip pit have occurred
in families with members with double lower-lip
pits. It can be assumed that a single pit is not a
distinct entity but rather an incomplete expres-
sion of the trait. On the other hand, the rarely
described fi stulas of the upper lip (Lannelongue
1879 ; Radcliff 1940 ) have not shown any inheri-
tance pattern.
Commissural or angular lip pits – small,
usually asymmetrical channels located at the lip
a
b
Fig. 8.2 ( a ) Pits in the upper and lower lips in a 20-year-old with a bilateral cleft lip and palate. ( b ) Close-up view
Fig. 8.3 Lip pits in the upper and lower lips in a bilateral
cleft lip and palate

229
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
angles – are also distinct entities with a much
higher incidence and different embryology
(Gorlin and Pindborg 1964 ; Lemke 1959 ; Everett
and Wescott 1961 ; Witkop 1964 ; Witkop and
Barros 1963 ; Schuermann et al. 1966 ) .
8.1.4 Association with Other
Malformations
In addition to their strikingly common association
with cleft lip and/or palate, pits of the lower lip
have been noted in association with other mal-
formations. Gorlin and Pindborg ( 1964 ) have
found cases of lip pits along with anomalies of
extremities, popliteal pterygia, and anomalies of
the genitourinary system. The association with
cleft lip and/or palate may well form a distinct
new syndrome.
A review of the literature suggests that a vari-
ety of other anomalies may be associated with lip
pits. Among them: syndactyly of the hands
together with cleft lip and palate (Lannelongue
1879 ; Bernauds 1906 ) ; mental retardation and
cleft, type not speci fi ed (Test and Falls 1947 ) ;
ankyloglossia and cleft lip and palate (Van der
Woude 1954 ) ; polythelia (Baxter 1939 ) ; sym-
blepharon and cleft lip and palate (Oberst 1910 ) ;
and ankyloblepharon, adhesion between the max-
illa and mandible, and cleft uvula (Neuman and
Shulman 1961 ) . In two cases of the orofacial
digital syndrome, pits were observed by Gorlin
and Psaume ( 1962 ) .
8.1.5 Inheritance
In most cases of lip pits described during the last
120 years, a marked hereditary pattern was
observed. Although all authors have excluded
Fig. 8.4 Radiograph of a
premaxilla showing two
central and one lateral
incisors ( left ) and twined
lateral incisors and one
malformed central incisor
( right )
a
b
Fig. 8.5 Missing and malformed anterior teeth. ( a ) A
very small premaxilla with one deciduous incisor but no
permanent tooth buds. ( b ) Malformed deciduous maxil-
lary anterior teeth and missing left deciduous anterior
teeth

230
S. Berkowitz
autosomal recessive inheritance or X-linked
inheritance, there is no uniform opinion concern-
ing whether or not the condition is due to a single
autosomal dominant pleiotropic gene.
Fogh-Andersen ( 1942, 1961 ) was the fi rst to
clearly point out that the inheritance of clefts in
the families with a history of lip pits is of a
different character than in families where no lip
pits occur. According to Fogh-Andersen ( 1942,
1961 ) , the role of genetic factors in families with
lip pits is much more pronounced, and both
genetically different types of clefts (cleft lip or
cleft lip and palate and isolated cleft palates)
commonly are found within a single family.
Fogh-Andersen also stated that in families in
which fi stulae of lower lip occur as a dominant
hereditary character, there are some cases of cleft
lip and cleft palate alone. Possibly, it may be
explained as the result of coupling of neighboring
genes.
Van der Woude ( 1954 ) , in a careful study of
fi ve pedigrees with clefts and lip pits, found that
the combination of pits and clefts is based on a
single dominant gene of variable expressivity.
She agreed with other authors (Test and Falls
1947 ) that a mildly affected individual can pass
the trait on in a very severe form and a severely
affected individual can pass the trait on in mild
form. The sex of the individual is not a factor in
passing on this anomaly. No sex limitation or
preference exists.
Patients with clefts but without lip pits (or
their parents) often ask for genetic advice. In
most of these cases, only a small risk of cleft lip
(less than 10 %) is indicated for the next child.
But the counseling situation is signi fi cantly
changed when associated lip pits are found. In
this instance, all clefts in the family are consid-
ered part of the syndrome, and risk fi gures for
clefts are remarkably higher.
8.1.6 Evidence of Heterogeneity
It is also clear that the risk of a cleft occurring
in a child is signi fi cantly higher when the par-
ent has lip pits and a cleft than when the parent
has lip pits only. Two alternative explanations
for this heterogeneity between families can be
considered: (1) The development of clefts in
persons carrying a “lip pit” major gene may be
in fl uenced by modifying genes at other loci, and
(2) in some families, a mutant allele may pro-
duce lip pits with only occasional clefts, whereas
in other families a different mutant allele (at the
same or a different locus) may frequently lead to
clefts in addition to lip pits. Thus far, efforts to
use the data to support one or the other of these
hypotheses have been unsuccessful. Cervenka
et al. ( 1967 ) reported data from 66 individuals
with lip pits in his study and 446 cases with lip
pits from the literature with known sex to estab-
lishing a 1:1 sex ratio. The frequency of the syn-
drome was estimated as 1:75,000–1:100,000 in
the white population.
Cervenka et al. ( 1967 ) further states that family
histories can be explained adequately on the basis
of autosomal dominant inheritance with variable
expressivity of the trait. Penetrance is high, esti-
mated at 80 %. Pits show up more frequently
than clefts, and there is a signi fi cant association
between the types of clefts in parents and their
children. Possibly, the development of clefts in
this syndrome is in fl uenced by modifying genes
or by different mutant alleles with a predilection
for the different types of cleft.
8.2
Orthodontic Treatment,
Dentition, and Occlusion
8.2.1 Crossbite Correction (Figs . 8.6 ,
8.7 , 8.8 , 8.9 , 8.10 , 8.11 , and 8.12 )
Bergland and Sidhu ( 1974 ) advocated postponing
orthodontic treatment until complete eruption of
the permanent anterior teeth. Segmental align-
ment can then be corrected with simultaneous
manipulation of the anterior teeth. We believe it
is best to start maxillary arch expansion when the
deciduous dentition is completely erupted
and when the children can be easily managed.
A three-phase treatment is followed: (1) Buccal
crossbite is corrected at 4–6 years, (2) anterior
teeth are aligned at 8–9 years, and (3) fi nal ortho-
dontics is utilized at 11-plus years.

231
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
Fig. 8.6 Palatal expansion increases nasal width. At
12 years of age, buccal crossbite correction of the cleft
segment was performed by palatal expansion. Crossbite
correction is orthopedic in that the cleft bony segment is
moved laterally, widening the nasal chamber on that side.
Top : Right buccal segment is in crossbite. Bottom : After
expansion: Palatal fi stulae are exposed with the separation
of the overlapped palatal segments
a
b
Fig. 8.7 ( a ) Fixed palatal
helix expander used in
deciduous dentition with or
without an anterior fi nger
spring. ( b ) Fixed “Arnold”
expander using a compressed
open coil spring to create an
expansion force
a
b
c
Fig. 8.8 ( a – c ) Crossbite correction for bilateral cleft lip.
( a ) Age 2 years 8 months. Note the bilateral crossbite.
( b ) Arnold expander in place. ( c ) Crossbite correction
after 3 months brought on by the outward movement of
the lateral palatal segments

232
S. Berkowitz
a
b
c
d
e
g
h
f
Fig. 8.9 ( a – h ) Correction of
anterior and posterior
crossbite in the deciduous and
mixed dentition in a severely
scarred palate. ( a ) Complete
unilateral cleft lip and palate,
unoperated. ( b ) Palatal
segments in contact. ( c )
Palatal cleft closed at
6 months of age: von
Langenbeck procedure was
used to close a very wide cleft
space. ( d ) Bilateral buccal and
anterior crossbite. ( e ) Arnold
expander is in place. ( f )
Crossbite is still present due to
excessive scar tissue. ( g )
Expander is fully extended in
the early mixed dentition. ( h )
Anterior crossbite was
corrected, but the buccal
crossbite is still present.
Comments: The severely
scarred palate prevents the
lateral movement of the
medially positioned palatal
segments. Scars can only be
stretched a slight amount, and
if that amount is exceeded, the
bony segments will not move,
and instead the teeth will
respond to the orthodontic
forces. Should the teeth be
tipped outwardly they will
have to be permanently
retained by a bridge or by
teeth splinting
Fig. 8.11 ( a ) Orthodontic correction of an anterior and
posterior crossbite in the permanent dentition. Anterior
dental crossbite does not necessarily mean that the maxilla
is anteroposteriorly de fi cient in size and requires a LeFort
I advancement. In this case, the maxillary dentition was
advanced orthodontically with the missing lateral incisor
space opened to achieve interarch congruency. The expansion
was maintained by complete arch splinting. ( b ) A severely
ventro fl exed premaxilla was uprighted during the deciduous
dentition. In order to maintain the correction after the decid-
uous anterior teeth are lost, a fi xed palatal retainer with an
acrylic button is placed on the premaxilla’s palatal incline

233
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
a
b
Fig. 8.10 ( a , b ) Anterior and buccal crossbite correction
the mixed dentition. ( a ) Anterior crossbite was due to
palatally displaced deciduous teeth with premaxillary seg-
ment and not to growth de fi ciency. The buccal teeth are in
crossbite as a result of the lesser palated segment being
palatally displaced. This picture has the maxillary anterior
teeth in crossbite; the lower anterior teeth are shown while
the upper teeth are hidden. ( b ) A fi xed palatal expander
with fi nger springs is used to expand the arch and advance
the anterior teeth. In most instances, it is unnecessary to
disocclude the anterior teeth to move them forward.
Buccal expansion depends on the ability of the lesser bony
segment to move outward. Retention of the bony correction
is essential
a
b

234
S. Berkowitz
a
b
Fig. 8.12 ( a , b ) Repaired bilateral cleft lip and palate.
An anterior openbite and retruded premaxilla resulting
from inadequate orthodontic and surgical planning. ( a ) A
slight anterior openbite was present at 8 years of age at
which time a secondary alveolar bone graft was per-
formed. The orthodontist attempted to close the lateral
incisor spaces by bringing the cuspids mesially, while
retracting the central incisors. This created a more severe
openbite and an anterior crossbite. ( b ) The orthodontic
mechanics were reversed. The lateral incisor spaces were
opened, and the central incisors were advanced into an
ideal overbite and overjet relationship. One of the bone
grafts had to be redone. Comments: When the buccal
occlusion is Class I, it is always better to keep the lateral
incisor space open in both bilateral and unilateral cleft
cases. There are occasions when a mandibular central
incisor will need to be extracted in order to avoid fl aring
the maxillary central incisors and to obtain a proper occlu-
sion. An anterior fi xed bridge will stabilize the arch and
replace the missing maxillary lateral incisors
Crossbite correction by moving palatal
segments laterally in the presence of extensive
palatal scarring is dif fi cult and often unsuccess-
ful. Extensive mucoperiosteal undermining,
leaving wide denuded palatal bone, was neces-
sary to close the wide palatal cleft at 6 months
of age. Uniting the lip moved the palatal seg-
ments together (molding action) into good arch
approximation. Palatal scar contracture moved
the buccal segments farther medially, placing the
buccal teeth in crossbite. The anterior dental
crossbite does not necessarily re fl ect dimin-
ished anteroposterior maxillary growth but,
rather, malposition of the premaxillary teeth of
the greater segment.
8.2.1.1 Unilateral Cleft Lip and Palate
Deciduous and Mixed Dentition : When the
smaller segment’s alveolar process is contained
within the premaxillary alveolar segment of the
larger segment, a dental crossbite occurs between
the maxillary and mandibular teeth. Dental dys-
plasia (eruption of a tooth out of position) may or
may not coexist with segmental dislocation.
A simple crossbite of one tooth may be due to its
malposition rather than to the palatal segment’s
collapse. The most frequent crossbite is brought
on by the mesioangular rotation of the lesser seg-
ment rather than by ectopic tooth eruption. Total
buccal crossbite is seen less frequently but is gen-
erally present when the palatal tissue is scarred.
In either case, the same helix type of palatal
expander can be utilized and correction achieved
within 2–4 months. Anterior movement of the
deciduous central incisors often requires lingual
undercuts either by orthodontic bands with lugs
or by direct lingual shelf bonding to stabilize the
anterior activated palatal fi nger springs.
A fi xed palatal retainer can hold the correc-
tion until the second stage of orthodontic therapy
is initiated. There is no way to predict the return
of a crossbite in the absence of permanent reten-
tion. Bone grafting across the alveolar cleft does
not guarantee retention of the corrected arch
form.
Permanent Dentition : In Class I and Class III
cases, it is usually preferable to open the missing
lateral incisor space with the expectation of uti-
lizing a fi xed bridge to stabilize the corrected
arch form and replace the missing tooth. In Class
II cases, it may be possible to encourage the cus-
pid to erupt through the alveolar bone graft in

235
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
the lateral incisor space, which eliminates the
need for extensive orthodontics and bridgework.
The cuspid may need to be splinted to the central
incisor in order to maintain the corrected arch
form.
8.2.1.2 Bilateral Cleft Lip and Palate
First Stage : Between the ages of 4 and 6 years,
the premaxilla is usually ventro fl exed and over-
laps one or both lateral palatal segments, which
may be in partial or complete buccal crossbite.
As in unilateral cases, treatment of bilateral cleft
lip/palate necessitates moving bony segments
into the surrounding muscle ring. If the premax-
illa is to be moved forward, bands with lingual
lugs are placed on the deciduous central incisors.
A fi xed helix-type palatal expander with anterior
activated fi nger springs is cemented to the sec-
ond deciduous molars. The fi nger springs are
positioned under the central incisor-banded lugs
for retention. The premaxilla is uprighted prior
to correcting the buccal crossbite. The anterior
and buccal crossbite correction can be completed
within 6 months. As the premaxilla and lateral
palatal segments are moved outward, the anterior
cleft space is uncovered. A fi xed palatal retainer
with an acrylic anterior extension to cover the
anterior cleft space is placed and kept in posi-
tion until the alveolar cleft is bone-grafted and
all fi stulas are surgically closed. Permanent com-
plete palatal retention is necessary, even after
alveolar bone grafting. A premaxillary retainer
must be placed when the deciduous anterior teeth
are lost.
Second Stage : At 7–8 years of age, when the
deciduous incisor teeth are replaced by permanent
incisors, the anterior crossbite may have to be
retreated. The incisors may be rotated and malposi-
tioned. Orthodontic brackets are placed in the upper
arch to support a labile arch wire, which will be uti-
lized to reposition the incisor teeth and reduce the
premaxillary overbite. A pontic tooth with band is
placed on the arch wire to achieve a more pleasing
aesthetic result. The premaxilla needs to be prop-
erly aligned with the lateral palatal segments prior
to alveolar bone grafting; this procedure may be
performed between 7 and 9 years of age. A lateral
incisor as well as a cuspid may erupt through the
area of new bone formation.
Third Stage : Children at 10+ years of age are
treated as any other child. The many malocclusion
possibilities render it impossible to develop treat-
ment plans for each contingency; instead, basic
treatment problems are discussed:
1. Class I malocclusion (with anterior and/or
buccal crossbite): The crossbite generally is
due to lingual bony displacement. The upper
arch should be advanced and expanded. If
there is suf fi cient arch length, the missing lat-
eral incisor area is left open. In arch shortage
cases, it may be preferable to move the cuspid
adjoining the space into the lateral incisor area
rather than extract the fi rst bicuspid on that
side. A number of variations of treatment exist
according to tooth size and location.
2. Class II malocclusion: The anterior overjet may
be corrected by retraction of the premaxillary
central incisors in bilateral cleft lip and/or cleft
palate cases. If one or both lateral incisors are
missing, it may be best to place the adjoining
cuspid in that space and extract either the oppo-
site lateral incisor or the fi rst bicuspid.
3. Class III malocclusion: An anterior crossbite
does not necessarily signify that a Class III mal-
occlusion exists. In some cases, the anterior teeth
can be advanced without excessively increas-
ing their axial inclination. A true depressed
midface with poor vertical and anteroposterior
development requires midfacial lengthening and
advancement. Segmental surgery may have to
be utilized to overcome palatal width problems
caused by excessive scarring.
8.2.1.3 Use of Orthopedic Forces
to Correct Midfacial Recession
Developing anterior crossbites in either mixed or
permanent dentition can be corrected using ortho-
pedic protraction forces. These forces must aver-
age 800 g/side and pull downward and forward
off hooks placed between the lateral incisor and
cuspid. The force needs to be applied 12 h/day.
With good cooperation, 5–10 mm of midfacial
advancement can be accomplished. The preferred
facial mask is the Delaire type.

236
S. Berkowitz
8.2.2 Supernumerary (Extra) Teeth,
Missing Teeth, and Aplasia
(Malformed Teeth)
This occurs more frequently in children with
cleft lip and/or palate than in other fi guration of
the nasal fl oor (Figs. 8.5 , 8.6 , 8.13 , and 8.14 ).
Bishara and coworkers ( 1985 ) studied untreated
adults in India who had clefts of the lip and alve-
olus only, unilateral cleft lip and palate, and
bilateral cleft lip and palate. They observed that
the maxilla and cranial base were not different
from a matched normal population but that the
relation of the maxilla and mandible to the cra-
nial base varied according to cleft type. Moss
( 1969 ) , Moss et al. ( 1968 ) , Blaine ( 1969 ) , Dahl
( 1970 ) , and Krogman et al. ( 1975 ) have stated
that the cranial base in cleft palate patients dif-
fered in both size and shape from noncleft
individuals.
The incidence reported in different articles has
varied because it is dif fi cult to distinguish
between variations rooted in congenital causes
and those related to surgery (Bohn 1963 ) .
Recently, it has been observed that supernumer-
ary teeth are more common in the deciduous
dentition. Moreover, the incidence of supernu-
merary teeth is greatest in cases of cleft lip only
and decreases as the extent of the cleft increases.
The relationship is the opposite in cases of apla-
sia; the incidence of aplasia is lowest for cleft
lip only and cleft palate only and increases in
proportion to the extent or complexity of the
cleft (Brook 1984 ; Garn et al. 1959, 1960, 1965 ;
Brabant 1967 ) .
In conditions of facial clefting, dental devel-
opment is, except for the third molars, delayed for
all teeth, both maxillary and mandibular (Brook
1984 ; Garn et al. 1959 ) . Asymmetrical develop-
ment of tooth pairs, with delayed development
on the cleft side, was recorded in approximately
half of a group of children with congenital lip
and/or palate clefts (Brabant 1967 ) . This sup-
ports other observations that eruption is delayed
in both dentitions (Garn et al. 1960, 1971, 1977a,
b ; Ranta 1971, 1972, 1973a, b ; Fanning 1961 ;
Dixon 1968 ; Falkner 1957 ; Delgado et al. 1975 ;
Demirjian 1986 ; Fishman 1970 ; Foster and
Lavelle 1971 ; Galili et al. 1969 ; Haring 1976 ;
Hatton 1955 ; Haavikko 1985 ; Haataja et al.
1972 ) .
Zilberman ( 1973 ) , from a study on clefts of
the lip and alveolar structures, and Mirsa and col-
leagues ( 1972 ) , after investigating clefts of the
lip and palate, reported that unilateral clefts are
more frequent on the left side and are more com-
mon in males than in females.
The incidence of dental malocclusion reported
in patients with cleft lip and/or palate varied
widely in studies by Huddart and Bodenham
( 1972 ) , Hellquist et al. ( 1979 ) , Dahl et al. ( 1981 ) ,
Norden and associates ( 1973 ) , Bergland and
Fig. 8.14 Tooth abnormalities. Coalescence of the right
deciduous central and lateral incisors in a right unilateral
cleft of the lip and alveolus
Fig. 8.13 The left central and lateral incisor in the line of
an alveolar cleft areas reduced in size

237
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
Sidhu ( 1974 ) , Nylen and coworkers ( 1974 ) , Ranta
and colleagues ( 1974a, b ) , and Hellquist and
Skoog ( 1976 ) . This may be because the patients
had varying types of clefts, and their cases were
recorded at different ages. Rehrman and coau-
thors ( 1973 ) found the incidence of malocclusion
in the mixed dentition to be twice that in the
deciduous dentition.
In cases of cleft palate only, Ranta and col-
leagues ( 1974a, b ) found only a slight increase
in anterior crossbite at the transition from the
deciduous to the mixed dentition. A noticeable
increase in the incidence of anterior crossbite in
the mixed dentition, in cases of complete unilat-
eral clefts of the lip and/or palate, was reported
by Bergland and Sidhu ( 1974 ) . This was irre-
spective of the arch con fi guration in the decidu-
ous dentition. They also reported that palatal
segments stabilized early after lip repair and that
further collapse was the exception. However,
contrary to the fi ndings just cited, Nylen and
coworkers (1974) found no increase in the fre-
quency of anterior crossbite in their mixed-den-
tition group.
8.2.3 Caries
Dahl et al. ( 1989 ) reported the incidence of car-
ies, gingivitis, and dental abnormalities in pre-
school children with cleft lip and/or palate in
Stockholm, Sweden. Oral health was studied in
49 children 5–6 years old with clefts of the lip
and/or palate (CL/P) and 49 healthy children
matched for sex and age. The results showed
a statistically signi fi cant increase in the preva-
lence and activity of caries among the CL/P
children. The average number of decayed and
fi lled tooth surfaces in the cleft group was 7.0
compared with 3.9 in the control group
( p < 0.05).
The most evident difference between the two
groups was found in the number of decayed prox-
imal surfaces. The mean number of decayed
proximal surfaces in the CL/P group was 2.5,
compared with 0.9 in the control group ( p < 0.001).
No signi fi cant differences were found in the prev-
alence and activity of caries among children with
isolated clefts of the lip or palate.
The children with cleft lips/palates also exhib-
ited a signi fi cant increase ( p > 0.01) in the num-
ber of gingival units with gingivitis. Other dental
abnormalities included increased enamel hypom-
ineralization ( p < 0.05), supernumerary teeth
( p < 0.001), unilateral crossbite ( p < 0.001), and
mesial terminal plane ( p < 0.01). These results
clearly show that children with CL/P as a group
must be considered to have an increased risk of
caries and gum disease and should therefore have
the bene fi t of additional preventive programs (see
Chap. 2 ).
8.3
The Relationship Between
the Clefting Process
and Contiguous Skeletal
Structures
Some studies have indicated that clefting is not
an isolated defect but may be a syndrome phe-
nomenon with rami fi cations in contiguous and
often remote structures.
In a study of Danish males, Dahl ( 1970 )
suggested that the presence of cleft palate, with
or without cleft lip, may have rami fi cations for
distant craniofacial structures and their develop-
ment. Farkas and Lindsay ( 1972 ) identi fi ed con-
sistent variations in facial morphology in the cleft
population and concluded that the cleft defect
was not an isolated condition. They reported that
what might otherwise be considered the normal
side of the face in cases of unilateral clefts was
not completely normal and that the anomaly
in fl uenced the development of the face equally
on both sides.
8.3.1 The Position of the Cleft Maxilla
Within the Cranium and the
Mandible
Berkowitz ( 1985 ) undertook a mixed cross-
sectional study of CUCLP and CBCLP cases to
determine whether the maxillary complex rela-
tive to the mandible is posteriorly positioned
within the face by studying the dental occlusion.
None of the cases had presurgical orthopedics,

238
S. Berkowitz
and the hard palate clefts were closed between 18
and 28 months of age using a modi fi ed von
Langenbeck procedure with a vomer fl ap. This
study was designed to test McNeil’s thesis that
the palatal segments, being detached from the
nasal septum, are not only reduced in mass but
also have not been brought forward with the
developing nasal septum. This failure would lead
to
retrusive
midface
with
a
Class
III
malocclusion.
Berkowitz ( 1985 ) found that the occlusal
relationships at 6 years of age did show Class I
and Class II occlusions, but none of the cases
had a Class III occlusion, which would have
been present if McNeil’s (McNeil 1950, 1954 )
hypothesis had been valid. Of the 29 bilateral
cases, fi ve cases had a crossbite on one side, one
case had a complete bilateral crossbite, and six
cases had no crossbites at all. It is quite evident
that a buccal crossbite is not, as stated by McNeil,
a predictable outcome of the presence of a pala-
tal cleft.
Semb’s ( 1991a, b ) and Ross’s ( 1987a, b ) stud-
ies and those already acknowledged elsewhere
established that in the cleft population, both the
maxilla and mandible are retropositioned within
the face (see Chap. 9 ). However, if McNeil’s
beliefs were accurate, the bilateral cleft palatal
segments would have been left behind in their
growth, and a greater proportion of the cases
would have shown a Class III malocclusion on
one or both sides.
Chierici and associates ( 1973 ) and Bishara
and Iversen ( 1974 ) , Bishara et al. ( 1985 ) found a
relative retrusion of the maxilla and mandible as
well as increased steepness of the mandibular
plane in various cleft types. Krogman and his col-
leagues ( 1975 ) reported signi fi cant differences in
the cleft population in the size of the cranial base,
its con fi guration, and direction of growth. They
concluded that the clefting process has growth
and/or development implications for the contigu-
ous cranial base and facial structures as well as
for the maxilla.
Bishara and Iversen ( 1974 ) reported that the
posterior positioning of the maxilla and mandible
relative to the anterior cranial base may result
from the cleft’s in fl uence on contiguous skeletal
structures and that clefting affects maxillary
development and facial morphology.
8.3.2
The Cranial Base
Hayashi and colleagues ( 1976 ) investigated cra-
nial growth of a large sample of subjects from 4
to 18 years of age with complete unilateral clefts.
The investigators found that the cranial base
angle was fl atter, the maxilla was more retruded,
and underdevelopment in both the maxilla and
the mandible was more pronounced in girls than
in boys. They speculated that upper face height in
patients of both genders was less than normal as
a result of cleft interference with nasal septal and
maxillary suture growth and changes in the
con fi guration of the nasal fl oor.
8.3.3
Relationship of the Nasal Cavity
to Arch Form (Figs. 8.15 and 8.16 )
Aduss and Pruzansky ( 1967 ) wrote that the ana-
tomic distortions common to all of their patients
with clefts included marked deviation of the nasal
septum toward the noncleft side; fl attening, par-
ticularly of the inferior turbinate on the cleft side;
and an anterolateral displacement of the noncleft
segment, with an outward and lateral rotation of
the premaxillary area adjoining the cleft.
These distortions are the result of unbridled
septal growth, deviant maxillary growth, and
aberrant vectors of muscle pull. Establishing a
continuous muscle band across the cleft, by lip
repair, can bring the palatal shelves closer
together and modify the con fi guration of the pal-
atal segments, as well as the con fi guration of the
internal nares.
Peyton and Ritchie ( 1936 ) , measuring the
displacement of the soft tissues of the nose in
complete unilateral cleft lip and palate, have
shown that deviation of the external part of the
nose toward the noncleft side extends for the
entire length of the nose, with the greatest dis-
placement at the tip. They further demonstrated
that growth of the nasal structures is the same in
noncleft children and children with complete
unilateral cleft and that the early cleft deformity
decreases with time. The natural tendency for
self-correction of the septal deviation was evi-
dent in the continual uprighting and medial
movement of the end of the septum observed in
all cases.

239
8 Lip Pits: Orthodontic Treatment, Dentition, and Occlusion – Associated Skeletal Structures
Nasal septum
Vomer
Cartilage of
nasal septum
Perpendicular plate
of ethmoid bone
a
b
Fig. 8.15 ( a ) Frontal view. The septum is straight dividing the nasal chamber in two equal parts. ( b ) Lateral view. The
components which make up the nasal septum: the ethmoid bone, the nasal cartilage, and the vomer
Fig. 8.16 Serial frontal cephalometric radiographs
illustrating geometric changes to the nasal chamber in a
CUCLP from birth to 4 years of age before and after pala-
tal expansion. Top : 0-2-8 At birth a widened nasal cham-
ber is evident. 0-5-22 After lip surgery the nasal chamber
has narrowed. Bottom : 1-8-3 The inferior turbinate on the
cleft side (R) makes contact with the vomer. 4-2-12 After
palatal expansion, the nasal width and the septum to infe-
rior turbinate distance has increased. Because the roof of
the mouth is also the fl oor of the nose, any disarrangement
in the architecture of the roof of the mouth is re fl ected in
the nasal chamber. Prior to lip repair, the nasal septum is
displaced to the noncleft side. After lip repair with the
medial movement of the cleft segment, the septum bows
toward the nasal chamber on the cleft side. After palate
repair, there is continual palatal movement with septal
uprighting and decreased septal bowing. The turbinates
on the cleft side are fl atter, and the buccal teeth on the
cleft side may be in crossbite. The nasal chamber on that
side is narrowed

240
S. Berkowitz
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Part III
Facial Growth in Cleft Palate Children

245
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_9, © Springer-Verlag Berlin Heidelberg 2013
9
9.1
Introduction
Congenital clefts of the lip and/or palate can
arise in isolation or together with other mal-
formations (syndromes) (Gorlin et al. 2001 ) .
This chapter deals solely with “nonsyndro-
mic” clefts. Both individuals with unoperated
and operated clefts have a face which differs
from those of unaffected individuals. Since the
introduction of roentgencephalometry more
than 70 years ago (Broadbent 1931 ) , hundreds
of cephalometric studies, including both unop-
erated and operated cleft individuals, have
suggested that some deviations are directly
caused by the primary anomaly, while others
are caused by the surgical interventions and the
following dysplastic and compensatory growth
of the facial bones (e.g., Graber 1949, 1954 ;
Slaughter and Brodie 1949 ; Ortiz-Monasterio
et al. 1959, 1966 ; Dahl 1970 ; Pruzansky 1971 ;
Bishara and Olin 1972 ; Friede and Pruzansky
1972a, b ; Bishara 1973 ; Friede and Johanson
1974 ; Bishara et al. 1976, 1985, 1986 ; Friede
and Morgan 1976 ; Friede 1977, 1978, 1998 ;
Friede et al. 1986 ; Smahel et al. 1987 ; Ehmann
1989 ; Mars and Houston 1990 ; da Silva Filho
et al. 1992b, 1998 ; Capelozza et al. 1993, 1996 ;
Tomanova and Müllerova 1994 ; Berkowitz
1995 ; Dahl and Kreiborg 1995 ; Semb and Shaw
1996 ; Sandham and Foong 1997 ; Friede and
Enemark 2001 ) . However, the relative impor-
tance of the intrinsic factors, the iatrogenic fac-
tors, and the functional or adaptive factors for
the facial development is still unclear. There
are probably several reasons for this. Firstly,
comprehensive knowledge of craniofacial mor-
phogenesis in cleft newborns or infants before
surgery, based on large, consecutive, well-con-
trolled samples, is very scarce. This situation
is not surprising since, in developed countries,
the cleft of the lip is surgically treated within
the fi rst couple of months after birth. Thus, the
possible period of examining the unoperated
state is short, and several methodological prob-
lems are involved. Secondly, the cephalometric
analyses are most often limited to the lateral
projection using simplistic cephalometric
analyses, typically based on 15–20 reference
points and almost invariably measuring maxil-
lary prognathism as the S-N-A angle or similar
measurements to the premaxilla, and the use
of infant cephalometry has been very limited.
Characteristics of Facial Morphology
and Growth in Infants with Clefts
Sven Kreiborg , Nuno V. Hermann ,
and Tron A. Darvann
S. Kreiborg , DDS, Ph.D., DrOdont ()
N. V. Hermann , DDS, Ph.D.
Department of Pediatric Dentistry and Clinical Genetics ,
School of Dentistry, University of Copenhagen ,
Copenhagen , Denmark
e-mail: skrei@sund.ku.dk; nuno@sund.ku.dk
T. A. Darvann , M.Sci., Ph.D.
3D Craniofacial Image Research Laboratory ,
School of Dentistry, University of Copenhagen ,
Copenhagen , Denmark
Department of Oral and Maxillofacial Surgery ,
Centre of Head and Orthopaedics,
Copenhagen University Hospital Rigshospitalet ,
Copenhagen , Denmark
e-mail: trd@sund.ku.dk

246
S. Kreiborg et al.
These authors are of the opinion that incom-
plete knowledge about the intrinsic factors
related to the cleft anomaly has automatically
lead to excessive emphasis on the importance
of iatrogenic and adaptive factors in facial
development of cleft children.
9.2
The Danish Experience
In the middle of the 1970s, we decided to take
advantage of the very favorable sampling con-
ditions in Denmark in an effort to contribute
to the question of the characteristics of facial
growth and development in children born with
clefts (Jensen et al. 1988 ) . In Denmark, for more
than 65 years, all newborns with facial clefts
have been recorded at the Institutes for Speech
Disorders in Copenhagen and Århus. Repeated
follow-up examinations have shown that the
registration of clefts in Denmark is highly reli-
able and nearly complete. The population is
homogeneous and stable, and only very few
children are lost to follow-up. Furthermore, all
primary cleft surgery is performed in one hos-
pital by one surgeon.
Inspired by Pruzansky and Lis ( 1958 ) , we
constructed a three-projection infant cephalome-
ter, which can obtain truly orthogonal lateral,
frontal, and axial cephalograms (Kreiborg et al.
1977 ) . A comprehensive cephalometric analysis
system was developed including all craniofacial
regions (calvaria, cranial base, orbits, maxilla,
mandible, airway, cervical spine, and soft tissue
pro fi le) (Kreiborg 1981 ; Heller et al. 1995 ;
Hermann et al. 2001a ) , and the method was
validated (Hermann et al. 2001a ) . Furthermore,
new methods of visualization of differences in
craniofacial morphology and growth between
different groups were developed using mean
plots (Kreiborg 1981 ; Hermann et al. 2001a ) ,
color-coded vector plots (Hermann et al. 2001a ) ,
and color-coded surfaces on a 3D CT-model
(Darvann et al. 1999 ) .
During the 6 years from 1976 to 1981, there
were 359,027 live births in Denmark. A total of
678 newborns of Northern European ancestry
with cleft lip, cleft palate, or both were regis-
tered in the period. Twenty-four infants died
before 22 months of age, and for practical rea-
sons, material uptake had to be omitted in some
patients with isolated cleft palate. Only nonsyn-
dromic clefts were included in the study, but
602 of the 678 children (about 90 %) were
examined by us (Jensen et al. 1988 ) and nearly
all at both 2 months of age (before any surgical
or orthopedic treatment) and at 22 months of
age (before closure of the posterior palate in the
children with clefts of the secondary palate).
All children were treated by the same surgeon
(Dr. Poul Fogh-Andersen), and in the children
with cleft of the primary palate, the cleft lip
was, in all cases, closed using a Tennison pro-
cedure. One-third of the children had isolated
cleft lip (CL), about 40 % had combined cleft
lip and palate (CLP), and about 27 % had iso-
lated cleft palate (CP). The clefts were
subclassi fi ed according to the method of Jensen
et al. ( 1988 ) .
In the 602 children included in the study,
cephalograms were obtained in the lateral,
frontal, and axial projections by three expe-
rienced orthodontists (Dr. Birgit Leth Jensen,
Dr. Erik Dahl, and Dr. Sven Kreiborg). In addi-
tion, impressions were made of the maxilla,
and anthropometric registrations (body height,
body length, and head circumference) were
carried out. The results of the cephalometric
analyses have been presented in a number of
publications (Dahl et al. 1982, 1989 ; Kreiborg
et al. 1985 ; Kreiborg and Cohen 1996 ; Darvann
et al. 2001 ; Hermann et al. 1999a, b, 2000,
2001a, b, 2002, 2003a, b, 2004 ; Kreiborg and
Hermann 2002 ) . So far, we have analyzed
infant craniofacial morphology and early cran-
iofacial growth in detail in three dimensions
in the following groups: unilateral incomplete
cleft lip (UICL), isolated cleft palate (ICP),
Robin sequence (RS), unilateral complete cleft
lip and palate (UCCLP) (Fig. 9.1a ), and bilat-
eral complete cleft lip and palate (BCCLP)
(Fig. 9.1b ). In the following, we shall sum-
marize our fi ndings, with emphasis on the

247
9 Characteristics of Facial Morphology and Growth in Infants with Clefts
Fig. 9.1 ( a ) The facial morphology in a 2-month-old
unoperated infant with unilateral complete cleft lip and palate
(UCCLP). ( b ) The facial morphology in a 2-month-old
unoperated infant with bilateral complete cleft lip and
palate (BCCLP)
unoperated infant to shed light on the intrin-
sic factors related to the cleft condition (see
Fig. 9.2 and Table 9.1 ), and compare them to
data in the literature on unoperated adolescents
and adults with clefts.
9.2.1 Cleft Lip (CL)
Isolated CL involves only structures of the
embryonic primary palate. The craniofacial
morphology in CL subjects has been shown to
be fairly normal except for the small region of
the cleft including the premaxilla and the inci-
sors. In unoperated bilateral complete CL, the
premaxilla may, however, protrude markedly.
In unilateral complete CL, the protrusion is less
pronounced but asymmetric. In subjects with
unoperated unilateral incomplete cleft lip
(UICL), the protrusion of the premaxilla is neg-
ligible (Hermann et al. 1999a ) . The interorbital
distance in CL subjects seems to be slightly
increased compared to the norm (Cohen 1997 ) .
The basal part of the maxilla has a normal prog-
nathism in relation to the anterior cranial base,
and the mandible is of normal size, shape, and
inclination (Dahl 1970 ; Hermann et al. 1999a ) .
Following lip surgery, the premaxilla is molded
into a normal position, and maxillary prog-
nathism measured to point A or ss ( subspinale )
is normal (Dahl 1970 ; Han et al. 1995 ; Hermann
et al. 1999a, b, 2000 ) . In conclusion, subjects
with UICL have a very close to normal cranio-
facial morphology from infancy to adult age,
and consequently, we have used our group of
infants with UICL as a control group in the
study of deviations in craniofacial morphology
and growth of infants and young children with
ICP, RS, UCCLP, and BCCLP since no actual
normative cephalometric data for Danish infants
and young children are available.
9.2.2 Cleft Palate (CP)
Isolated cleft palate (ICP) involves only structures
of the embryonic secondary palate. In Fig. 9.2a , the
mean facial diagrams of the ICP group are super-
imposed on the mean facial diagram of a group of
age-matched infants with UICL (control group).
The major deviations in the ICP group were:
reduced length and posterior height of the max-
illa, maxillary retrognathia, increased width of the
maxilla and the nasal cavity, and reduced length of
the mandible with mandibular retrognathia. Thus,
the ICP group revealed bimaxillary retrognathia.

248
S. Kreiborg et al.
ICP, 2m
UICL, 2m
RS, 2m
UICL, 2m
UCCLP, 2m
UICL, 2m
BCCLP, 2m
UICL, 2m
BCCLP, 2m
UICL, 2m
BCCLP, 2m
UICL, 2m
UCCLP, 2m
UICL, 2m
UCCLP, 2m
UICL, 2m
RS, 2m
UICL, 2m
RS, 2m
UICL, 2m
ICP, 2m
UICL, 2m
ICP, 2m
UICL, 2m
a
b
c
d
Fig. 9.2 ( a – d ) Mean plots in three projections (lateral,
frontal, and axial) of the four different cleft groups
superimposed on the control group with UICL. The lateral
mean plots are aligned on the n-s line and registered at s. The
frontal mean plots are aligned on the latero-orbital line and
registered at the center point of that line. The axial mean
plots are aligned on a line between the two tuber points and
registered at the center point of that line. Superimposition of
the mean plots for the 2-month-old ( a ) ICP and UICL
groups, ( b ) RS and UICL groups, ( c ) UCCLP and UICL
groups, and ( d ) BCCLP and UICL groups

249
9 Characteristics of Facial Morphology and Growth in Infants with Clefts
The sagittal jaw relationship was, however, nor-
mal. In addition, in the ICP group, the upper airway
dimensions were reduced. Bimaxillary retrognathia
and a short mandible were previously documented
in unoperated older children and adults with ICP
(Dahl 1970 ; Bishara 1972 ) .
9.2.3 Robin Sequence (RS)
Robin sequence (RS) is de fi ned as a triad of symp-
toms: isolated cleft palate, micrognathia, and
glossoptosis (Gorlin et al. 2001 ) . RS may be part
of several syndromes, e.g., Treacher-Collins syn-
drome (Kreiborg and Cohen 1996 ; Cohen 1997 ) .
In this chapter, only nonsyndromic cases of RS
will be discussed. We consider this group as a sub-
group of the ICP group (Hermann et al. 2003a ) . In
Fig. 9.2b , the mean facial diagram of the RS group
at 2 months of age is superimposed on the mean
facial diagram of the control group. The major
deviations in the RS group were decreased length
and posterior height of the maxilla, maxillary ret-
rognathia, increased width of the maxilla and nasal
cavity, and very short mandible with marked man-
dibular retrognathia. Thus, the RS group revealed
bimaxillary retrognathia ; the retrognathia was,
however, most marked for the mandible, and the
sagittal jaw relation was increased. In addition, the
RS group had a signi fi cantly smaller cranial base
angle ( n-s-ba ) resulting in a smaller depth of the
bony nasopharynx than the controls, and the upper
airway dimensions were markedly reduced. The
degree of maxillary retrognathia was similar in the
RS and the ICP group. However, the mandibular
retrognathia in the RS group was even more
marked than in the ICP subjects. It would seem
that RS subjects probably represent the extreme
part of the ICP population in terms of mandibular
retrognathia and upper airway constriction. As
mentioned above, we consider the RS group as a
special subgroup of the ICP group. Accordingly,
Table 9.1 Summary and comparison of the most important fi ndings in the primary anomaly in children with RS, ICP,
BCCLP, and UCCLP
Anomaly
RS
ICP
BCCLP
UCCLP
Maxilla
Decreased length measured to premaxilla (sp-pm) a
+ b
+
– c
–
Retrognathia measured to premaxilla (s-n-ss) d
+
+
– e
–
Decreased posterior length (ci-pm) f
+
+
+
+
Retrognathia measured to base of jaw (s-n-ci) g
+
+
+
+
Decreased posterior height
+
+
+
+
Increased width
+
+
++
++
Nasal cavity
Increased width
+
+
++
++
Mandible
Decreased length
+++
++
++
+
Retrognathia
+++
++
++
+
Pharyngeal airway
Reduced size
+++
++
+
+
RS Robin sequence, ICP isolated cleft palate, BCCLP bilateral complete cleft lip and palate, UCCLP unilateral complete
cleft lip and palate
a sp-pm: anterior nasal spine to point pterygomaxillare
b The deviation from the norm is shown as + or –, meaning, e.g., that decreased total length of the maxilla was observed
in the ICP and RS groups but not in the UCCLP group and that the length of the mandible is decreased in the UCCLP
group, very decreased in the ICP and BCCLP groups, and severely decreased in the RS group
c The total length was signi fi cantly increased
d s-n-ss: S-N-A
e The prognathism was increased measured to the premaxilla
f ci-pm: Point crista infrazygomatica to point pterygomaxillare
g s-n-ci: Maxillary prognathism measured to the infrazygomatic crest

250
S. Kreiborg et al.
we believe the bimaxillary retrognathia to be
intrinsically associated with the cleft of the
secondary palate.
9.2.4 Cleft Lip and Palate (CLP)
Combined clefts of the lip, alveolus, and palate
involve structures of both the embryonic primary
palate and secondary palate. In Fig. 9.2c , the
mean craniofacial morphology in 2-month-old
unoperated infants with unilateral complete cleft
lip and palate (UCCLP) was compared to the
control group (Hermann et al. 1999a ) . The major
deviations in the UCCLP group were decreased
posterior length and height of the maxilla; retrog-
nathia of the basal part of the maxilla with rela-
tive protrusion of the premaxilla; the width of the
maxilla and nasal cavity was markedly increased
and the premaxilla deviated to the noncleft side;
and the mandible was short and retrognathic.
Thus, the UCCLP group revealed bimaxillary
retrognathia combined with a relative protrusion
of the premaxilla, which deviated to the noncleft
side. In addition, in the UCCLP group, the upper
airway dimensions were reduced.
Increased width of the midface and nasal cavity
was previously reported in unoperated UCCLP
infants (Han et al. 1995 ) and in unoperated adults
with UCCLP (Motohashi et al. 1994 ) . Relative pro-
trusion and asymmetry of the premaxilla have also
been reported in unoperated UCCLP children,
adolescents, and adults (Ortiz-Monasterio et al.
1959, 1966 ; Bishara et al. 1976, 1985, 1986 ;
Capelozza et al. 1993 ) . The relative protrusion and
deviation are probably due to overgrowth in the
premaxillary-vomerine complex (Pruzansky 1971 ;
Friede and Morgan 1976 ; Friede 1978 ) and due to
the lack of structural integrity of the maxilla on one
side. This relative protrusion of the premaxilla
explains why we found the measurements s-n-ans
( S-N-ANS ) and s-n-ss ( S-N-A ) in the infant UCCLP
group to be comparable to the values in the control
group, despite the fact that the UCCLP group
showed signi fi cant maxillary retrognathia mea-
sured to the basal part of the maxilla.
Dahl et al. ( 1982 ) and Hermann et al. ( 2003a, b )
analyzed facial morphology in 2-month-old
infants with unoperated bilateral complete cleft
lip and palate from our sample. Fig. 9.2d illus-
trates the mean facial diagram of the BCCLP
group superimposed on the mean facial diagram
of the control group. The most obvious features
in the BCCLP group were protrusion of the pre-
maxilla both in relation to the anterior cranial
base and in relation to the basal part of the max-
illa; the length of the basal part of the maxilla and
posterior maxillary height were decreased; ret-
rognathia of the basal part of the maxilla; mark-
edly increased width of the maxilla and nasal
cavity; a short and retrognathic mandible. Thus,
the BCCLP group revealed bimaxillary retrog-
nathia with a truly protruding premaxilla. In
other words, the protruding premaxilla was situ-
ated in a totally retrognathic face with a fairly
normal sagittal jaw relationship. In addition, the
upper airway dimensions were reduced.
The extreme protrusion of the premaxilla is
probably the result of marked overgrowth in the
premaxillary-vomerine complex secondary to
total lack of structural integrity in the region.
For comparison, Mars and Houston ( 1990 )
and da Silva Filho et al. ( 1998 ) described groups
of adult unoperated patients with BCCLP and
found extreme protrusion of the premaxilla and a
very convex pro fi le measured as the ANB angle.
No measurements were performed to describe
the position of the body of the maxilla. Da Silva-
Filho et al. ( 1992a, 1998 ) also found the man-
dible to be short and retrognathic and discussed
whether this fi nding was related to the primary
anomaly or if it was caused by secondary func-
tional adaptations.
The retrognathia of the basal part of the max-
illa and the short and retrognathic mandible found
in our sample are, in our opinion, variations
intrinsically associated with the cleft of the sec-
ondary palate as discussed above.
9.3
Discussion and Conclusions
The Danish study of craniofacial morphology in
untreated cleft infants is the hitherto most com-
prehensive and well-controlled since it covers a
whole population, which is homogeneous and in

251
9 Characteristics of Facial Morphology and Growth in Infants with Clefts
which central registration of clefts has been car-
ried out for more than 65 years, a registration
which has been shown to be highly reliable and
nearly complete. Furthermore, all cleft infants
are surgically treated at one hospital by one sur-
geon using the same techniques. All infants were
examined with state-of-the-art three-projection
cephalometry using the hitherto most compre-
hensive cephalometric analysis covering all cran-
iofacial regions, and the methods were validated.
The study included more than 600 children, and
even after breakdown into subgroups, the sample
sizes were adequate for statistical testing (except
maybe for the RS group). Based on these facts,
the fi ndings related to the infant craniofacial mor-
phology at 2 months of age, prior to any surgical
or orthopedic treatment, must be considered to
represent the “true” malformation, primarily
caused by intrinsic factors.
In Table 9.1 , the most important fi ndings in
the primary anomaly in the Danish infants with
RS, ICP, BCCLP, and UCCLP are given, reveal-
ing a rather clear pattern. The fi ndings support
the suggestion of Dahl ( 1970 ) and others that
facial clefts should be classi fi ed based on the
embryonic facial development, i.e., into clefts
involving the primary palate only (CL), clefts
involving the secondary palate only (CP), and
clefts involving structures of both the primary
and the secondary palate (CLP). The postnatal
facial morphology in these groups differs greatly.
Infants with cleft of the secondary palate, with or
without cleft of the primary palate, shared a num-
ber of characteristic morphological traits when
compared to the norm: decreased posterior length
of the maxilla, maxillary retrognathia, decreased
posterior height of the maxilla, increased width
of the maxilla and the nasal cavity, decreased
length of the mandible, mandibular retrognathia,
and reduced size of the pharyngeal airway. As
seen from Table 9.1 and Fig. 9.3 , the mandibular
involvement was most pronounced in the RS
group followed by the ICP and BCCLP groups
and, fi nally, the UCCLP group. A similar pattern
was observed for the reduced size of the pharyn-
geal airway.
As for the maxilla, the increased width of the
maxilla and the nasal cavity was most pronounced
in the groups with clefts of both the secondary
and the primary palate, i.e., BCCLP and UCCLP.
None of these groups showed decreased total
length of the maxilla or retrognathia of the max-
illa when measured to the premaxilla; the reason
for this being a true and relative protrusion of the
premaxilla, respectively.
In conclusion, a short and retrognathic man-
dible was a constant fi nding in infants with cleft
of the secondary palate. The reduction in size
of the pharyngeal airway in infants with cleft of
the secondary palate was clearly related to the
short and retrognathic mandible, being most
severe in the RS group, which had the added
effect of a reduction in the cranial base angle.
But, in principle, all four groups had restricted
upper airways as part of the primary anomaly.
The increased width of the maxilla and nasal
cavity was most pronounced in the groups
which also had cleft of the primary palate
(UCCLP and BCCLP). The UCCLP group was
also characterized by relative protrusion of the
premaxilla which was positioned asymmetri-
cally, deviating to the noncleft side, whereas in
the BCCLP group, the premaxilla showed true
protrusion both in relation to the basal part of
the maxilla (the lateral segments) and to the
anterior cranial base. On average, the premax-
illa was found to be positioned in the midline in
this group, although most of the individual
cases showed some degree of asymmetry. The
protrusion of the premaxilla is suggested to be
secondary to the primary anomaly of clefting,
allowing for overgrowth in the premaxillary-
vomerine complex, due to partial or total lack
of anatomical integrity in the region.
2 Months
RS
ICP
UCCLP
UICL
BCCLP
Fig. 9.3 Mean plots of the mandible in the RS, ICP, BCCLP,
UCCLP, and UICL groups. Superimposition was made on
the mandibular line (ML) registered at pogonion (pg)

252
S. Kreiborg et al.
It has been the aim of this chapter to summa-
rize our fi ndings about the intrinsic variations in
facial morphology associated with the different
types of cleft malformations to form a basis for
valid estimations of the amount of surgical iatro-
genesis, especially to the maxillary development,
introduced by different surgical procedures and
regimes, including the timing of treatment. In
Fig. 9.4 , the growth changes of the craniofacial
skeleton from 2 to 22 months of age in the
UCCLP group have been compared to the UICL
group (control group) using color-coded surfaces
on a 3D CT-model. In both groups, the cleft lip
was surgically closed just after the examination
at 2 months of age using a Tennison procedure. In
the UCCLP group, the anterior part of the palate
was closed with a vomer fl ap at the same time.
The method of producing the illustrations will be
given below.
9.3.1 Intuitive Visualization of the
Location of Growth Differences
Cephalometric measurements in three projections
provided growth vectors at each of the 279 (230
skeletal and 49 soft tissue) anatomical landmarks.
The growth vectors, computed as the vector dif-
ference between corresponding landmark loca-
tions at the ages 2 and 22 months, respectively,
after alignment to a common coordinate system
(Hermann et al. 2000 ) , have been used to form
average growth patterns previously shown in
Hermann et al. ( 1999a, b ) (UICL, UCCLP) and
Hermann et al. ( 2004 ) (UICL, BCCLP). Results
of comparisons of growth between the UCCLP
and the BCCLP groups, respectively, and the
control group (UICL) have been shown as color-
coded average growth patterns in Hermann et al.
( 1999a, b ) (UCCLP vs. UICL) and Hermann
et al. ( 2004 ) (BCCLP vs. UICL). These color-
coded growth diagrams disclosed the locations
of signi fi cantly different growth (1, 5, and 10 %
levels) in the study group when compared to a
reference group, and the diagrams were shown
separately for each of the 3 projections (lateral,
frontal, and axial), as well as for the growth mag-
nitude and the two growth directions (x and y in
each of the projections, respectively). In order to
facilitate the effective comprehension of these
diagrams, the locations of signi fi cant difference
are color-coded onto the surface of a skull recon-
structed from a CT scan of a single (noncleft)
infant. As an example, Fig. 9.4 shows such color-
coded surfaces for the comparison of the UCCLP
with the UICL (control group). The color-coded
surfaces were created by landmarking the 3D
CT scan of the single noncleft infant at locations
corresponding to the 230 skeletal cephalometric
landmarks and color coding the surface in the
vicinity of each landmark by a color correspond-
ing to the signi fi cance of the growth difference.
The landmark locations are shown in Fig. 9.5 . A
color table was chosen such that colors signify
Student’s t -test p values smaller than 0.01. Blue
colors correspond to locations where the study
group exhibits larger growth than the control
group, while the opposite is the case at locations
colored red. Regions without any signi fi cant dif-
ferences between the two groups remained gray.
In the UICL and UCCLP groups, the frontal and
axial projection data were mirrored in order to
have all clefts on the left side. Accordingly, the
cleft is on the patient’s left side in Fig. 9.4 . The
spatial extent of colored surface area in the vicin-
ity of a landmark was governed by the distance
to its closest landmark, and a maximum extent
(spherically from landmark position) was chosen
as 40 mm. Color-coded skulls are shown for dif-
ferences in growth magnitude, as well as for each
of the three growth directions (sagittal, vertical,
and transverse). The colors for sagittal growth
differences were computed from the x-compo-
nent of the growth vectors in the lateral cepha-
lometric projection and the y-component of the
growth vectors in the axial projection. The colors
for vertical growth differences were computed
from the y-component of the growth vectors in
the lateral projection and the y-component of
the growth vectors in the frontal projection. The
colors for transverse growth differences were
computed from the x-component of the growth
vectors in the frontal projection and the x-compo-
nent of the growth vectors in the axial projection.
The method of color coding has previously been
described and applied for visualization of the

253
9 Characteristics of Facial Morphology and Growth in Infants with Clefts
UCCLP VERSUS UICL
Magnitude
Sagittal
a
b
c
d
Vertical
Transverse
Fig. 9.4 ( a – d ) 3D visualization of locations of growth
differences. Locations where UCCLP growth differs
signi fi cantly ( p < 0.01) from UICL growth (2–22 months
of age) are colored red (UCCLP < UICL) or blue
(UCCLP > UICL). The surface reconstruction shown is of
a noncleft subject of comparable age and is used solely for
illustration. Cleft side is on patient’s left in the fi gures.
Locations of differences in the ( a ) magnitude of growth,
( b ) sagittal, ( c ) vertical, and ( d ) transverse growth compo-
nents are shown

254
S. Kreiborg et al.
growth differences between UCCLP and UICL
in Darvann et al. ( 1999 ) .
Secondary to surgical closure of the lip at
2 months of age in the UCCLP group, we found
that the premaxilla was molded into place,
demasking the intrinsic maxillary retrognathia
and leading to a normal sagittal jaw relationship
at 22 months of age. Maxillary growth was,
besides the premaxillary molding, characterized
by smaller vertical growth on the cleft side and
reduced transverse development, which could
probably be related to the effects of surgery. The
amount of mandibular growth was similar in the
two groups. However, the direction of growth
was slightly more vertical in the UCCLP group.
This growth pattern was probably related to the
intrinsic pattern of mandibular development.
Otherwise, craniofacial growth seemed to be very
similar in the two groups.
We found that surgery to the lip and ante-
rior part of the hard palate at 2 months of age in
UCCLP subjects seemed to in fl uence the devel-
opment of the maxillary complex, as observed
at 22 months of age, in a number of bene fi cial
ways: the premaxilla was no longer relatively
protruding, and it was less asymmetric; the nasal
septum deviated less toward the noncleft side;
the width of the nasal cavity and the posterior
Fig. 9.5 3D landmark locations corresponding to the skeletal landmarks used in the three-projection cephalometric
analysis as well as for creating the color-coded surfaces in Fig. 9.4

255
9 Characteristics of Facial Morphology and Growth in Infants with Clefts
part of the maxilla became relatively more nor-
mal; and the transverse position of the lateral
maxillary segment on the noncleft side was
closer to normal. The posterior height of the
maxilla was, however, still reduced to the same
degree; the mandible was still short and retrog-
nathic to the same degree; and bimaxillary ret-
rognathia was still present. The only iatrogenic
effect observed was that the lateral maxillary
segment on the cleft side had become displaced
toward the midsagittal plane anteriorly, resulting
in a much too narrow dental arch at the level of
the deciduous canine (Hermann et al. 2000 ) . It is
noteworthy that several studies of older, unoper-
ated UCCLP children and adults fi nd the maxil-
lary prognathism to be within normal limits or
even increased when compared to normative
data (Ortiz-Monasterio et al. 1959, 1966 ; Mars
and Houston 1990 ; Capalozzo et al. 1996 ) . All
these studies, however, only measure maxillary
prognathism to the A-point or to the point
ANS, both located in the relatively protruding
premaxilla. Ortiz-Monasterio et al. ( 1959 ) con-
cluded based on their fi ndings in unoperated
adults with UCCLP that: “The embryonic factor
responsible for the facial cleft does not interfere
with maxillary growth. This evidence leads us to
believe that growth defects of the middle third of
the face so frequently seen are caused by early
or repeated and aggressive surgery.” We disagree
somewhat with this conclusion. Based on our
studies of infants with UCCLP, it would seem
that maxillary retrognathia in this group is part
of the intrinsic variations associated with the
cleft malformation of the secondary palate. In
the unoperated infant and the unoperated adult,
the maxillary retrognathia is, however, partly
masked by relative protrusion of the premaxilla,
secondary to overgrowth in the premaxillary-
vomerine suture. Surgical closure of the lip at
2 months of age molds the premaxilla back into
place, demasking the maxillary retrognathia.
Thus in the 22-month-old lip-operated UCCLP
group, it is our opinion that the bimaxillary ret-
rognathia illustrates the facial type characteristic
of the group rather than an iatrogenic effect of
cleft surgery (Hermann et al. 1999b, 2000 ) . Thus,
we do not consider the maxillary retrognathia
observed at 22 months of age as the result of
surgical iatrogenesis; rather, we believe it rep-
resents a normalization of the “intrinsic facial
type” characteristic of subjects with UCCLP;
and at 22 months of age, the face is still harmo-
nious with a normal sagittal jaw relationship. We
have, at this point in time, not reexamined the
sample at older ages and can, therefore, not com-
ment on facial growth and signs.
In conclusion, we are not arguing that cleft
surgery does not lead to disturbed maxillary
development during the growth period. But we
are suggesting that subjects with cleft of the sec-
ondary palate have a special “intrinsic” facial
type, primarily characterized by bimaxillary ret-
rognathia and increased maxillary width. We are
speculating that this facial type could be a “liabil-
ity factor” increasing the probability of CP or
CLP (Hermann et al. 1999a, b ) . Finally, we sug-
gest that when outcome of cleft surgery in CLP
subjects is evaluated at adolescence or adulthood,
comparisons should not be made to normal stan-
dards, but rather to the adolescent and adult mor-
phology seen in CP subjects.
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(1999a) A comparison of the craniofacial morphology
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36:38–41

259
S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_10, © Springer-Verlag Berlin Heidelberg 2013
10
10.1
Introduction and Background
The Sri Lankan Cleft Lip and Palate Project
(SLCLPP) was founded in 1984 and completed
data collection in 2009. Surgical visits were
undertaken in 1985, 1986 and 1990. More than
500 surgical patients have been followed up lon-
gitudinally for 25 years post-operatively, result-
ing in the creation of a unique multidisciplinary
archive. In total, 14 data collection visits were
made from 1984 to 2009 (Mars et al. 2008 ) .
Patients presented for surgery at all ages from
infancy to adulthood. Many had received no sur-
gery whatsoever; some had received lip surgery but
not palatal surgery and others lip and palate surgery
by local surgeons. They therefore provided a special
opportunity to study the nature and timing of
surgical intervention and its outcome on facial
growth up to adulthood (nature’s experiment).
This chapter is an analysis of the long-term
outcome of patients who were over 20 years of
age at their last data collection point.
10.1.1 Records Collected for Study
Standardised lateral skull radiographs and dental
study models were collected for all subjects as
part of this longitudinal study. Impressions were
taken in alginate material with the patients sitting
upright on a wooden chair.
10.2
Un-operated Unilateral
Cleft Lip and Palate
Adults with un-operated clefts of the lip and
palate provide the ideal group to study the natu-
ral progression of facial growth and to fully
assess the inherent growth potential in these
patients. Due to the ethical dif fi culties in with-
holding treatment for cleft patients, studies of
un-operated cleft subjects have been undertaken
in the developing world where surgery has not
been readily available. As a result, most studies
on such patients have lacked a suitably matched
local comparison group as most operated
patients presented from the developed world
(Mars 1993 ) .
A 25-Year Longitudinal Facial
Growth Study of Unilateral Cleft Lip
and Palate Subjects from the
Sri Lankan Cleft Lip and Palate Project
Brijesh Patel and Michael Mars
B. Patel , BDS (Hon), M.Sc., MFDS, M Orth, FDS (Orth)
North Thames Cleft Centre – Great Ormond Street
Hospital for Children , London , UK
St. Andrew’s Hospital , Chelmsford , UK
M. Mars , DSc (Hon), Ph.D., BDS, FDS, D.Orth,
FRCSLT (Hon), FSLCP (Hon) ()
North Thames Cleft Centre – Great Ormond Street
Hospital for Children , London , UK
Faculty of Medicine ,
Peradeniya , Sri Lanka
e-mail: michael-mars@msn.com

260
B. Patel and M. Mars
10.2.1 Clinical Features
The most striking feature in the un-operated
UCLP patient is the protrusion of the upper labial
segment (Fig. 10.1 ). These subjects present with
large overjets, proclined upper incisors, eversion
of the major segment, mild contraction of the
lesser segment in the anterior region and rarely,
buccal crossbites.
10.2.2 Cephalometry
Using the Sri Lankan growth archive, Liao and
Mars ( 2005a ) studied the long-term effects of
clefts on craniofacial morphology in patients
with UCLP. Employing a retrospective case–
control study design, they compared 30 un-
operated adult UCLP patients with 52 normal
(non-cleft) control subjects of the same eth-
nic
background.
Cephalometric
analysis
con fi rmed the presence of morphological dif-
ferences between UCLP and non-cleft patients
(Fig. 10.2 ). The adverse effects of clefting were
predominantly on the vertical development of
the maxilla both anteriorly and posteriorly and
to a lesser extent on the anteroposterior devel-
opment of the basal maxilla. In addition, there
were differences in the position and shape of
the mandible and the position of the maxillary
and mandibular incisors. However, the overall
anteroposterior dimensions of the maxilla were
not affected by clefting, and these patients did
not exhibit maxillary retrusion.
10.2.3 Study Model Analysis
Using the re fl ex microscope, McCance et al.
( 1990 ) studied the maxillary arch form of 41
adults with un-operated complete unilateral cleft
lip and palate and compared them to a control
group of 100 normal adults (Fig. 10.3 ).
The teeth in the cleft group were smaller than
their equivalents in the control group, the most
marked difference being found in the central and
lateral incisors. Arch widths of the cleft groups
were reduced, more anteriorly (5 mm in the
canine region) than posteriorly (1.6 mm in the
second molar region), resulting in more V-shaped
arches. No differences were found in the arch
length or chord lengths between the groups. There
was a higher prevalence of crossbites in the cleft
group, 19.5 %, compared to none of the controls,
and the overjet was greater in the cleft group
(mean 8.2 mm) than in the controls (3.7 mm).
A higher percentage of missing teeth, most com-
monly the lateral incisor teeth, was recorded
in the cleft group. There was no difference in
crowding between the two groups. Although the
reductions in tooth size and arch width would
suggest a small degree of primary hypoplasia,
the differences are small.
The GOSLON yardstick is a robust and repro-
ducible tool for categorising dental arch relation-
ships into fi ve distinct groups of increasing
deformity (Mars and Plint 1985 , Mars et al. 1987 ) .
It was applied to 51 un-operated UCLP cases, and
the results showed 98 % of the cases were in
groups 1 and 2 (excellent or very good arch rela-
tionships) and no cases in groups 4 or 5 (Fig. 10.4 ).
In contrast, only a small proportion of operated
patients were in group 1 when UK centres were
assessed as part of the CSAG study (Clinical
Standards Advisory Group 1998 ) (Fig. 10.5 ).
10.2.4 Summary
Studies using cephalometry and study mod-
els con fi rm that there is an intrinsic potential
for un-operated UCLP patients to grow rela-
tively normally with minor distortions around
the cleft site itself where the dentition is unre-
strained because of the disrupted musculature.
In addition, the lack of continuity of the arch
probably explains the transverse distortions
seen in the dental arch. There is a small degree
of hypoplasia as discussed above but these are
minor and do not account for the gross maxil-
lary retrusion frequently reported in surgically
repaired patients.

261
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
Fig. 10.1 ( a – j ) Illustrates a typical example of the facial appearance and dental study models of an un-operated
unilateral cleft lip and palate case

262
B. Patel and M. Mars
S
v
Cleft subjects
Non-cleft controls
v
Fig. 10.2 Dental and skeletal effects of lip surgery
(Liao and Mars 2005a )
Fig. 10.1 (continued)

263
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
Fig. 10.3 The digitised points, chord lengths and arch widths used in the re fl ex microscopic analysis of study models
Sri lankan UCLP 13+ M and F
Sri Lankan Surgeons − Wardill-Kilner
GOSLON Grouping
Totally unoperated (n = 51)
Lip surgery (n = 37)
Lip & palate surgery (n = 37)
0%
20%
40%
1+2
3
4+5
60%
80%
100%
Fig. 10.4 GOSLON grouping
of UCLP patients operated by
Sri-Lankan surgeons using
Wardill-Kilner (Mars and
Houston 1990 )

264
B. Patel and M. Mars
10.3
Effect of Primary Surgery
on Facial Growth
It is widely accepted that facial growth and mor-
phology in cleft lip and palate patients is abnor-
mal, with mid-face retrusion common in patients
who have had corrective surgery in infancy (Ross
1987 ; Semb 1991 ) .
Historically, the cause of this mid-face retru-
sion has been attributed to three possible causes:
an intrinsic developmental de fi ciency, functional
distortions affecting growth and iatrogenic fac-
tors due to surgical treatment. There has been
signi fi cant controversy on the extent and relative
contributory role of each of these possible caus-
ative factors. It was this unresolved con fl ict of
opinions on the aetiology of facial growth distor-
tion in operated cleft lip and palate patients that
led to the establishment of the SLCLPP.
A number of seminal papers on facial growth
in cleft patients have been published from the Sri
Lankan archives.
Mars and Houston ( 1990 ) reported the effects
of primary lip and palate surgery on craniofacial
growth in cleft patients using a cohort of Sri
Lankan male patients. The studied patients were
divided into three subgroups and compared to a
control group of healthy males using lateral
cephalometry and study model analysis. The three
subgroups analysed included: those who had
totally unrepaired cleft lip and palate, those who
received lip repair in infancy but not palatal repair
and those who had lip and palate repair in infancy.
From this preliminary study, it was clearly evident
that un-operated cleft patients had the potential to
grow normally. Furthermore, in patients who have
had a lip repair but no palate repair, the maxilla
appears to also grow relatively normally.
In addition to cephalometry, Mars and
Houston ( 1990 ) applied the GOSLON yardstick
to the same cohort of patients aged 13 years old
operated by Sri Lankan surgeons. Figure 10.4
shows that all the subjects in the totally un-oper-
ated subgroup had excellent dental arch relation-
ships (groups 1 and 2). In the lip only subgroup,
almost two thirds of the subject scored in groups
1 and 2, and only a small proportion scored in
groups 4 and 5. This contrasts markedly with the
lip and palate subgroup where two thirds of the
patients were in groups 4 and 5 (poor dental arch
relationship) and only a small proportion in
groups 1 and 2.
Using preliminary data from the SLCLPP, Mars
and Houston ( 1990 ) clearly showed the detrimental
effects of primary palatal surgery on facial growth.
We now have complete data for 198 UCLP
subjects who are greater than 20 years of age.
This chapter studies the GOSLON yardstick
analysis of all these patients and the cephalomet-
ric measures for 154 subjects.
10.3.1 Lip Surgery
Whilst studies on un-operated cleft patients have
demonstrated the iatrogenic effects of primary
35
Group 1
Group 2
Group 3
Group 4
GOSLON
UK CSAG study 1998
Group 5
30
25
20
%
15
10
5
0
Fig. 10.5 GOSLON for the
1998 CSAG (Clinical
Standards Advisory Group) for
the whole of the UK

265
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
cleft surgery on facial growth, there has been
controversy as to whether the lip or the palate
repair has the most detrimental effect on maxil-
lary growth (Muir 1986 ; Mars and Houston 1990 ;
Bardach et al. 1984 ) .
10.3.1.1 Cephalometry
Liao and Mars ( 2005b ) further clari fi ed the long-
term effects of lip surgery on craniofacial growth.
Using lateral cephalograms from the longitudinal
growth data obtained from the SLCLPP, they
studied 71 adult patients, 23 non-syndromic un-
operated UCLP patients and 48 non-sydromic
UCLP patients who had undergone lip repair only.
This study demonstrated that the major effect of
the lip repair was on the anteroposterior and verti-
cal position of the maxillary alveolus and the
maxillary incisors. Furthermore, there was a dif-
ferential in fl uence from the tip of the alveolus and
the incisal edge to the base of the alveolus and the
incisal apex. This was associated with uprighting
of the maxillary incisor and resulted in a decreased
overjet and increased overbite in the lip repair
only group (Fig. 10.6 ). The pressure of the lip
thus produces secondary bone resorption in the
base of the anterior maxillary alveolus.
10.3.1.2 Study Model Analysis
More recently, the authors carried out a review of
the SLCLPP archive using the GOSLON yard-
stick. All patients were operated within the proj-
ect and are now adults aged over 20 years and
have completed facial growth. The subjects were
divided into the same three subgroups as reported
by Mars and Houston ( 1990 ) and had had their
surgery at varying ages. Interestingly, the same
results were replicated. Figure 10.7 below is
almost a replica of Fig. 10.4 above.
Both GOSLON fi gures (Figs. 10.4 and 10.7 )
clearly demonstrate that surgery to the lip only has
Effects of lip surgery
Totally unoperated (n = 23)
Lip surgery (n = 48)
SNA
(P = 0.05)
SN-UI
(P < 0.0001)
SN-Pr
(P < 0.001)
Overjet
(P = 0.02)
120
80
40
0
Fig. 10.6 Effects of Lip
Surgery (Liao and Mars
2005b )
GOSLON scores at age 18 + Years
Totally unoperated (n = 51)
1
2
3
4
5
1+2
4+5
3
Lip surgery (n = 40)
Lip and Palate surgery (n = 155)
0%
20%
40%
60%
80%
100%
Fig. 10.7 The diagonal
line demonstrates the
progressively worsening
effect from no surgery to
lip surgery to lip and
palate surgery

266
B. Patel and M. Mars
some effect, but this is mainly dentoalveolar. The
most obvious difference is seen in the patients who
have undergone both lip and palate repair, clearly
suggesting that the palate repair has the most
signi fi cant in fl uence on future facial growth.
Muthusamy ( 1998 ) analysed study models of
26 patients pre and post lip repair using the re fl ex
microscope. These patients had not had any other
surgical interventions and were operated on by
British surgeons as part of the SLCLPP.
The patients were divided into two subgroups
– young being those who had the lip repair prepu-
bertally (15 patients) and mature being those who
had it post-pubertally (11 patients).
He found that in both groups, there was a
signi fi cant reduction in arch width with the great-
est reduction in the inter-canine distance.
Interestingly, in the younger group, there was a
reduction in arch width in all measures (anterior
and posterior), whereas in the mature group, there
was no signi fi cant reduction in the inter-molar
and inter-premolar.
After the lip repair, there was a reduction in
overjet and an increase in overbite in both groups
(Fig. 10.8 ).
10.3.1.3 Summary
Both the cephalometric and study model studies
con fi rm that that lip repair primarily produces a
localised bone-bending effect on the anterior
maxillary alveolus (alveolar moulding) and does
not have a signi fi cant effect on maxillary
growth.
10.3.2 Palate Surgery
Gillies and Fry ( 1921 ) were the fi rst to suggest
that the palate repair has a detrimental effect on
facial growth. Interestingly, they were also the
pioneers for the policy of delayed hard palate clo-
sure. The SLCLPP has been key in demonstrat-
ing the relative effects of lip and palate surgery
on future facial growth.
10.3.2.1 Cephalometry
Liao and Mars ( 2005c ) looked at the long-term
effects of palatal surgery on facial growth.
They compared non-syndromic UCLP Sri
Lankan adults who had had either a lip repair
only (48 patients) or a lip and palate repair (58
patients) using cephalometry. They concluded
that palate repair inhibits the forward displace-
ment of the basal maxilla and anteroposterior
development of the maxillary alveolus in patients
with UCLP. However, they found that the palate
repair did not have any detrimental effects on the
downward displacement of the basal maxilla or
on palatal remodelling in patients with a unilat-
eral cleft lip and palate. Contrary to expectations,
the axial inclination of the maxillary incisors is
not affected by the palate repair, though is a
major effect of the lip repair as discussed above.
10.3.2.2 Study Model Analysis
Both GOSLON charts (Figs. 10.4 and 10.7 ) above
show that the most obvious difference is seen in
the patients who have undergone both lip and pal-
ate repair, clearly suggesting that the palate repair
has the most signi fi cant in fl uence on future facial
growth.
10.4
Timing of Primary Surgery
and Its Effects on Facial Growth
Because patients presented at all ages for primary
surgery, it became possible to analyse the effects
of such surgery when performed at any age
between infancy and adulthood.
10.4.1 Timing of Lip Surgery
In another cephalometric study, Liao and Mars
( 2006 ) looked at the timing of lip repair and the rel-
evance of the operating surgeon. Although the sam-
ple size was small (23 in the early repair group and
25 in the mature repair group), they noted that early
lip repair was found to produce a greater bone
remodelling effect in the base of the anterior max-
illa. This is possibly related to the relatively greater
surgical trauma in a smaller individual or the early
onset on tension from the repaired lip or both. In
this study, dentofacial morphology was unrelated to
the surgeon who performed the lip repair.

267
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
Fig. 10.8 An adult patient who had a lip repair but no palate repair demonstrating the localised effects of lip surgery

268
B. Patel and M. Mars
In summary, patients with earlier lip repairs
display more localised bone remodelling than
those operated upon later. As the lip repair only
has a localised effect, it probably explains why
dentofacial morphology is unrelated to the sur-
geon carrying out the lip repair.
10.4.2 Timing of Palatal Surgery
As the SLCLPP archive has patients operated on
at different ages, it has provided an opportunity
to undertake longitudinal retrospective studies to
assess the question of the timing of hard palate
surgery and its effects on facial growth.
10.4.2.1 Cephalometry
Liao et al. (2006) analysed the longitudinal
records for 104 patients non-syndromic UCLP
patients who had had their hard palate repair
by the age of 13 years. A total of 290 lateral
cephalograms taken at different ages were
analysed using a linear regression model. The
timing of hard palate surgery had a signi fi cant
effect on the length and protrusion of the max-
illary alveolus and the anteroposterior jaw
relation measured at 20 years. The regression
model suggested a maxillary growth improve-
ment of 0.2mm in maxillary length and 0.4o in
both SNA and ANB for every year delay in hard
palate closure – Figs. 10.9 , 10.10 , 10.11 , and
10.12 . They concluded that the timing of hard
palate repair signi fi cantly affects the growth
of the maxilla, earlier palate repair has a more
adverse effect on the growth of the maxilla.
Following the most recent data collection
visit in 2009, the authors reviewed cephalo-
grams of a further 50 non-syndromic UCLP
adult patients, all aged over 20 years of age.
Cephalometric analysis to assess the effects of
the timing of palatal repair on facial growth
concurred with the earlier study (Figs. 10.13 ,
10.14 , and 10.15 ). Patients who underwent hard
palate surgery in infancy showed reduced max-
illary length and protrusion when compared to
patients who underwent surgery at a later age.
Patients who undergo surgery according to
accepted protocols in infancy exhibited more
class III malocclusions.
10.4.2.2 Study Model Analysis
The authors have recently reviewed all patients
treated in Sri Lanka who had records at age
greater than 20 years. One hundred and ninety-
eight patients were divided up according to the
age of palatal repair and the GOSLON yardstick
was used to assess the dental arch relationships.
The scores clearly show that the earlier the repair,
the more the detrimental affect it has on facial
growth in the long term (Figs. 10.16 and 10.17 ).
Timing of hard palate repair
The regression coefficient indicates the change in mean (mm or degrees)
of the dependant variable at 20 years of age per year increase at hard
palate repair.
Dependant veriable
PMP – A point (mm)
Maxillary length
0.2 (0.0,0.4)
0.05
<0.001
0.001
0.4 (0.2,0.7)
0.4 (0.2,0.6)
SNA (°)
Maxillary protrusion
ANB (°)
Relative maxillary/
Mandibular protrusion
Regression coefficient
(95% CI)
P Value
Fig. 10.9 Effect of timing
of hard palate repair on
maxillary growth (Liao et al.
2006 )

269
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
60
Early repair
Median repair
Late repair
55
50
45
10
12
Age (years)
PMP-A (mm)
14
16
18
20
Fig. 10.10 Effect of timing of
hard palate repair on palatal length
(Liao et al. 2006 )
Age (years)
10
5
010
12
14
16
18
20
Early repair
Median repair
Late repair
–5
ANB (degree)
Fig. 10.11 Effect of timing
of hard palate repair on
maxillary-mandibular
relationship (Liao et al. 2006 )

270
B. Patel and M. Mars
Age (years)
75
10
12
14
16
18
20
80
85
90
SNA (degree)
Early repair
Median repair
Late repair
Fig. 10.12 Effect of timing
of hard palate repair on
maxillary protrusion (Liao
et al. 2006 )
PMP-A point (mm)
49
50
51
52
53
54
55
56
2−13 years (n = 35)
0−2 years (n = 10)
13+ years (n = 5)
Fig. 10.13 Maxillary
length related to age at
primary surgery
SNA (degrees)
2−13 years (n = 35)
0−2 years (n = 10)
76
78
80
82
84
13+ years (n = 5)
Fig. 10.14 Maxillary
protrusion related to age at
primary surgery

271
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
2−13 years (n = 35)
1
0.5
0
–0.5
–1
0−2 years (n = 10)
13+ years (n = 5)
ANB (degrees)
Fig. 10.15 Relative
maxillary/mandibular
protrusion related to age at
primary surgery
0%
0−2 years (n = 23)
2−8 years (n = 64)
8−13 years (n = 15)
13−18 years (n = 15)
18 years (n = 40)
Lip surgery only (n= 41)
Timing of
palatal repair
20%
40%
1+2
3
1
2
3
GOSLON assessed at age 18+ years
4
5
4+5
60%
80%
100%
Fig. 10.16 Dental arch
relationships and timing of
palatal repair. The diagonal
line demonstrates the
progressively worsening
effect with earlier surgery

272
B. Patel and M. Mars
Fig. 10.17 Adjust subject who had lip and palate repair in early infancy demonstrating maxillary retrusion

273
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
10.5
Type of Primary Surgery
and Its Effect on Facial Growth
Studies from the SLCLPP data have clearly dem-
onstrated the effects lip and palate surgery on
facial growth. The data has been further analysed
to assess any correlations on the type of surgery
and its effects on long-term facial growth. The
results have to be taken in context as subdivision
of groups reduces numbers and the power of the
study. However, the trends noted can form the
basis for future randomised control trials.
10.5.1 Wardill-Kilner Versus
Von Langenbeck Repair
There is no agreement on the best surgical
approach. Both Pigott et al. ( 2002 ) and Johnston
et al. ( 2004 ) reported increased proportions of
poor or very poor GOSLON scores for a Veau-
Wardill-Kilner repair group compared to a von
Langenbeck group. The SLCLPP archive has
patients who had had either the Wardill-Kilner
or von Langenbeck procedures. The local Sri
Lankan surgeons and some of the earlier visit-
ing British surgeons favoured the Wardill-Kilner
procedure. The Scandinavian surgeons and some
of the later visiting British surgeons favoured the
von Langenbeck procedure. The GOSLON yard-
stick used to assess outcome clearly shows that
patients who undergo the Wardill-Kilner repair
(Fig. 10.4 ) have poorer outcomes when compared
to the von Langenbeck group (Fig. 10.18 ). These
results do not take the skill of the surgeon into
account, which clearly makes a difference.
10.5.2 Vomerine Flap
The use of a vomer fl ap has been another area
of controversy. It was popularised by the Oslo
team who have reported good long-term out-
comes with their protocol (Semb 1991 ) . Some
groups believe that the use of a vomer fl ap is
actually detrimental to future maxillary growth
(Friede and Lilja 1994 ) . Some surgeons within
the SLCLPP routinely performed vomer fl aps,
whilst others did not. It has therefore been pos-
sible to tease out the effects of the use of vom-
erine fl aps on dentoalveolar relationships in
the long term.
The authors looked at adult UCLP patients
who had only had a lip repair (31 patients) who
were subdivided into two groups – lip with
vomer fl ap and lip only. The GOSLON yardstick
was used to assess outcomes depending on what
age the lip surgery was carried out (Fig. 10.19 ).
In both groups, surgery after 2 years of age did
not have a marked effect on dentoalveolar rela-
tionships. In both groups, patients undergoing
surgery in infancy had poorer GOSLON scores
with more patients scoring 4 and 5 in the vomer
fl ap group. This suggests that the vomer fl ap
0%
20%
40%
60%
80%
100%
1
2
3
4
5
n = 44
n = 45
n = 81
Totally
unoperated
Lip surgery
Lip and palate
surgery
Lip repair vs lip and palate repair
GOSLON at >19 years age
British Surgeons − Von Langenbeck
Fig. 10.18 GOSLON scores
for adult patients operated by
British Surgeons using the
Von-Langenbeck procedure
(Mars et al. 2005 )

274
B. Patel and M. Mars
may have some detrimental effect on the growth
of the maxilla when assessed without the effects
of hard palate repair.
Similarly, the authors analysed adult UCLP
patients in the SLCLPP archive who had
undergone both lip and palate surgery and sub-
divided these into two groups: lip with vomer
fl ap (60 patients) and lip without vomer fl ap
(82 patients). All patients underwent a von
Langenbeck procedure to repair the palate. The
GOSLON yardstick was used to assess out-
comes depending on what age the lip surgery
was carried out. The fi gure below (Fig. 10.20 )
clearly shows that patients who had a vomer
Lip repair witrhout definitive palate repair
assessed using GOSLON at 18+ years
0%
20%
40%
60%
80%
100%
0%
20%
40%
60%
80%
100%
0−2 years
(n = 9)
0−2 years
(n = 6)
2−13 years
(n = 4)
2−13 years
(n = 3)
13+ years
(n = 5)
13+ years
(n = 4)
Lip with vomer flap
Lip only
Fig. 10.19 GOSLON at age 18+ years for patients who had lip repair without de fi nitive palate repair
0−2 years
(n = 7)
2−8 years
(n = 25)
8−13 years
(n = 3)
13 years
(n = 25)
0−2 years
(n = 8)
2−8 years
(n = 33)
8−13 years
(n = 13)
8−13 years
(n = 28)
Lip without vomer flap
Lip without vomer flap
0%
20%
40%
60%
80%
100%
0%
20%
40%
60%
80%
100%
Lip repair with definitive palate repair
assessed using GOSLON at 18+ years
Fig. 10.20 GOSLON at age 18+ years for patients who had lip repair with de fi nitive palate repair

275
10 A 25-Year Longitudinal Facial Growth Study of Unilateral Cleft Lip
fl ap repair did better and did not score 5 in any
age group. In both groups, patients who under-
went repair after the age of 2 did better than
the younger patients in the long term. The use
of a vomer fl ap provides ‘extra’ tissue to repair
the hard palate and limits the use of extensive
undermining of the soft tissues and relieving
incisions. As discussed above, the palate repair
has the most detrimental effect on long-term
facial growth, and the use of a vomer fl ap in
patients who undergo both lip and palate repair
appears to be bene fi cial.
10.6
Factors In fl uencing
Interpretation of Results
from the Sri Lankan Cleft Lip
and Palate Project
10.6.1 Malnutrition and Growth
and Delayed Onset of Puberty
It should be recognised that the above studies are
derived from subjects in the developing world.
Although Sri Lanka is a relatively advanced
developing country, there is nevertheless sig-
ni fi cant malnutrition and endemic infections,
for example, malaria. The failure of infants with
clefts to gain weight adequately has been docu-
mented by several authors (Avedian and Ruberg
1980 ; Ranalli and Mazaheri 1975 ) .
Malnutrition is a well-recognised form of
reversible growth hormone resistance, which can
be normalised with nutritional supplements. A
malnourished mother is likely to give birth to a
baby with low birth weight, while children with
protein-energy malnutrition do not grow as well
as others according to a recent report (Fernando
1990 ). This kind of malnutrition is an underlying
cause of almost one third of the deaths among
children under 5 years in Sri Lanka. Malnutrition
is still a serious problem in Sri Lanka (Rajapaksha
and Siriwardena 2002 ) . Food insecurity is one of
the major reasons for malnutrition in that country
according to the Department of Census and
Statistics. Poor fi nancial and physical access to
food is responsible for the malnutrition and food
insecurity. Drastic price increases of essential
food commodities and stagnating or deteriorating
incomes create poor fi nancial access to food. The
civil war from 1984 to 2010 in Sri Lanka has
exacerbated the provision of essential food and
has created fi nancial problems.
A recent survey of 16,000 Sri Lankan chil-
dren found that only one quarter were properly
nourished (Popham 2002 ) . More than one third
were suffering from third-degree malnutrition,
the level beyond which children exhibit dis-
tended stomachs and skinny frames. Supporting
evidence from the National Peace Council indi-
cated that only 4,863 children under 5 years out
of a random sample of 16,767 were within nor-
mal nutritional limits; 6,371 children had third-
degree malnutrition, 3,186 with second-degree
malnutrition and 2,347 with fi rst-degree malnu-
trition (National Peace Council of Sri Lanka
1998 ) . According to this report, diseases such as
malaria cause malnutrition fi rst, which is still
prevalent in Sri Lanka. Secondary causes of mal-
nutrition are by worm infestations and third by a
lack of food. Many of the subjects in this study
were social outcasts, who dropped out of school.
Females in particular were hidden away in their
houses, and only one female in the un-operated
population married. Children need a good emo-
tional climate to thrive. The mechanism of the
effects of emotional deprivation on growth is not
well documented but is linked to reduced growth
hormone secretion and its associated growth
failure.
It has been recognised in the context of growth
studies in general and facial growth studies in par-
ticular that many patients in the developing world
have delayed onset of puberty (Mars 1993 ) . Boys
may not attain maturity until after 20 years of age
and girls until after 18 years. This has important
implications for all studies in developing coun-
tries and failure to address this issue can seriously
confound the result of research. The large volume
of longitudinal data has enabled this problem to
be addressed (Liao and Mars 2006 ) .
10.6.2 Speech Implications
Whilst facial growth in the un-operated subject
presents without maxillary retrusion – unlike
many operated patients – the speech outcomes
for the same series of patients demonstrate almost
unintelligible speech for the whole sample.

276
B. Patel and M. Mars
Research on the Sri Lankan Cleft Lip and
Palate archive has demonstrated that surgery
when delayed beyond eight years of age and even
earlier results in permanent irremediable speech
disorders (Sell and Grunwell 1990 ; Sell 1991 ) .
This chapter is careful in not recommending
the delay of hard palate repair. Previous studies
have consistently demonstrated speech impair-
ment associated with delayed hard palate repair
(Bardach et al. 1984 ; Witzel et al. 1984 ; Noordhoff
et al. 1987 ; Rohrich et al. 1996 , Lohmander-
Agerskov 1988 ).
References
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cleft palate infants. Cleft Palate J 17:24–26
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MP, Dusdieker LB (1984) Lip pressure changes fol-
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lip and palate. Plast Reconstr Surg 74(4):476–481
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lip and/or palate. The Stationary Of fi ce, London
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(2004) A comparison of craniofacial form in Northern
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S. Berkowitz (ed.), Cleft Lip and Palate,
DOI 10.1007/978-3-642-30770-6_11, © Springer-Verlag Berlin Heidelberg 2013
11
Abbreviations
CNMC
Children’s National Medical Center
CPT
Current Procedure Terminology
ICD – 9 International Classi fi cations of
Diseases Version 9
LOS
Length of hospital stay
NPA
Nasopharyngeal airway
RS
Robin sequence
SPSS
Statistical Program for Social Sciences
TLA
Tongue-lip adhesion
11.1
Introduction
Robin sequence (RS), the clinical triad of
micrognathia (small jaw), glossoptosis (downwardly
displaced tongue), and upper airway obstruction,
affects approximately 1 in 8,500 births (Bush and
Williams 1983 ; Sadewitz 1992 ) . Cleft palate is also
noted in up to 90.4 % of patients (Caouette-Laberge
et al. 1994 ) . Infants may present with a wide pheno-
typic variability, ranging from infrequent episodes
of airway obstruction and/or feeding dif fi culty
to severe crises of asphyxia and failure to thrive
(Marques et al. 2001 ) . The latter group of patients
is at particular risk for hypoxic brain damage,
impaired mental development, pulmonary hyper-
tension, aspiration pneumonia, and failure to thrive
(Tomaski et al. 1995 ; Hoffman et al. 1965 ; Kapp-
Simon and Krueckeberg 2000 ) . Despite advances in
critical care medicine, mortality is not inconsequen-
tial and ranges from 0 to 13.6 % (Sadewitz 1992 ;
Marques et al. 2001 ; Caouette-Laberge et al. 1994 ;
Cruz et al. 1999 ; Dykes et al. 1985 ) . Such high
morbidity and mortality have been attributed to late
diagnosis, delayed airway protection, and multisys-
tem disorder. Furthermore, care of infants with RS
can require prolonged hospitalization, with aver-
ages ranging from 10 to 60 days and translate into
increased costs (Bull et al. 1990 ; Cruz et al. 1999 ;
Matsas et al. 2004 ; Wagener et al. 2003 ) .
Several modalities have been proposed to
address airway obstruction in infants with RS.
Options range from conservative management,
namely, prone positioning, nasopharyngeal air-
way (NPA) placement, and orthopedic devices to
Airway Management in Patients
with Robin Sequence
Kevin D. Han, Mitchel Seruya, Diego A. Preciado,
and Albert K. Oh
K. D. Han , M.D. • M. Seruya , M.D.
Department of Plastic Surgery, 1PHC ,
Georgetown University Hospital ,
3800 Reservoir Rd, NW , Washington , DC 20007 , USA
e-mail: kdh8@gunet.georgetown.edu; mseruya@cnmc.org
D. A. Preciado , M.D., Ph.D.
Department of Otolaryngology, Pediatrics,
and Integrative Systems Biology ,
The George Washington University School of Medicine ,
Washington , DC , USA
Division of Pediatric Otolaryngology, Department of
Otolaryngology , Children’s National Medical Center ,
111 Michigan Ave NW , Washington , DC 20010 , USA
e-mail: dpreciad@cnmc.org
A. K. Oh , M.D. ()
Department of Surgery and Pediatrics ,
The George Washington University School of Medicine ,
Washington , DC , USA
Department of Plastic and Reconstructive Surgery ,
Children’s National Medical Center ,
111 Michigan Ave NW 4th fl oor West Wing ,
Washington , DC 20010 , USA
e-mail: aoh@cnmc.org