Early Craniofacial Morphology and Growth in Children With Bilateral

Complete Cleft Lip and Palate

N.V. HERMANN, D.D.S., PH.D.
T.A. DARVANN, M.SC., PH.D.
B.L. JENSEN, D.D.S., DR.ODONT., PH.D.
E. DAHL, D.D.S., DR.ODONT.
S. BOLUND, M.D.
S. KREIBORG, D.D.S., DR.ODONT., PH.D.
Purpose: Analysis of craniofacial morphology and growth in children with
bilateral complete cleft lip and palate (BCCLP), compared with a control group
with unilateral incomplete cleft lip (UICL), before any treatment as well as 20
months after lip closure.
Material: The children were drawn from a group representing all Danish children with cleft born 1976 to 1981. Sixty-four children were included in the study
(19 BCCLP and 45 UICL). The ages were 2 and 22 months at examinations 1
and 2, respectively.
Method: The method of investigation was infant cephalometry in three projections. The craniofacial morphology was analyzed using linear, angular, and
area variables. Growth was defined as the displacement vector from the coordinate of the corresponding landmark in the x-ray at examination 1 to its
coordinate at examination 2, corrected for x-ray magnification. The growth of
an anatomical region in a patient was assessed by investigating the growth
pattern formed by a collection of individual growth vectors in that region.
Results: The BCCLP group differed significantly from the UICL group. The
most striking findings in BCCLP were an extremely protruding premaxilla;
markedly increased posterior maxillary width; increased width of the nasal cavity; short maxilla with reduced posterior height; short mandible; bimaxillary
retrognathia; severe reduction in the size of the pharyngeal airway; and a more
vertical facial growth pattern.
Conclusion: Our findings indicate that a facial type including a wide and
posterior short maxilla, short mandible, and bimaxillary retrognathia might be
a liability factor that increases the probability of developing cleft lip and palate.
KEY WORDS: bilateral complete cleft lip and palate, early craniofacial morphology and growth, roentgencephalometry, three orthogonal
projections, unilateral incomplete cleft lip

Among the different types of cleft lip and palate (CLP),

bilateral complete cleft lip and palate (BCCLP) without any
soft tissue bridges (Simonarts band) crossing the clefts rep-

resents the most extensive anomaly and the anomaly that is

the most complicated to handle clinically. BCCLP divides the
maxilla into two lateral segments that have developed without
any bony connection with the nasal septum and a premaxilla
that, at birth, is most often markedly protruded in relation to
the two lateral segments (Dahl, 1970). Studies including individuals with and without surgically-repaired CLP have
shown that some deviations are directly caused by the primary
anomaly, whereas others are caused by surgical interventions
and the subsequent dysplastic and compensatory growth of the
facial bones (Dahl, 1970; Ehmann, 1989; Mars and Houston,
1990; Dahl and Kreiborg, 1995; Sandham and Foong, 1997;
Hermann et al., 1999a, b, 2000; Kreiborg and Hermann, 2002).
Semb and Shaw (1996) suggested several factors that may be
potential sources of interference with the normal craniofacial
growth pattern in individuals with clefts, including variations
intrinsically associated with the cleft malformation, other var-

Dr. Hermann, Dr. Jensen, and Dr. Kreiborg are with the Department of Pediatric Dentistry and Clinical Genetics, School of Dentistry, Copenhagen University Hospital. Dr. Hermann, Dr. Darvann, and Dr. Kreiborg are with the 3DLaboratory, Informatics and Mathematical Modelling, Technical University of
Denmark, and Dr. Dahl is retired from the Department of Orthodontics, School
of Dentistry, University of Copenhagen, Copenhagen, Denmark. Dr. Bolund is
with the Department of Plastic and Reconstructive Surgery, and Dr. Kreiborg
is with the Department of Clinical Genetics, Juliane Marie Centre, Copenhagen
University Hospital, Copenhagen, Denmark.
Submitted April 2003; Accepted August 2003.
Address correspondence to: N. V. Hermann, D.D.S., Ph.D., Assistant Professor, Department of Pediatric Dentistry and Clinical Genetics, School of Dentistry, University of Copenhagen, Nrre Alle 20, DK-2200 Copenhagen N,
Denmark. E-mail nh@lab3d.odont.ku.dk.
424

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

TABLE 1 Sex Distribution in the Two Groups*

Group

BCCLP

UICL

Total

Girls
Boys

8
11

16
29

24
40

Total

19

45

64

clusion in the study group were as follows: (1) the infant was
of Danish (Caucasian) origin, (2) the child was healthy except
for its single anomaly, and (3) in the UICL group, the surgical
procedure (lip closure) was carried out by a Tennison procedure at about 2 months of age. In the BCCLP group, the lip
closure was done stepwise because of the bilateral clefting. At
about 2 months of age, the lip was, in principle, closed on the
side on which the cleft was the widest. However, no marked
difference in cleft width was observed in a number of cases,
although it appeared that in 83% of the cases the cleft on the
left side was closed first. Two months later, at 4 months of
age, the nonrepaired cleft lip was then closed. All surgical
procedures were performed by the same surgeon.
The longitudinal samples included in this study consisted of
19 children with BCCLP and 45 with UICL (Table 1) who
had all been examined at both 2 and 22 months of age, and
both populations are believed to be representative of the Danish BCCLP and UICL populations. The longitudinal UICL
sample has previously been described by Hermann et al.
(1999a, 1999b, 2000). For the UICL group, the age at examination 1 was between 50 and 100 days and at examination 2
between 654 and 750 days. For the BCCLP group, the age at
examination 1 was between 52 and 72 days and at examination
2 between 611 and 691 days. At examination 1, all children
were examined just prior to surgical closure of the cleft lip (in
the BCCLP group, the first closure of the cleft lip). The mean
age at examination 1 was 60 days. At the second examination,
all children with BCCLP were examined just prior to surgical
closure of the cleft palate (the examination of the children with
UICL as matched to this age). The mean age was 662 days
(Table 2). The sex distribution male/female for both groups
was 2:1. The sex and diagnosis of the longitudinal samples are
given in Table 1.

* BCCLP 5 bilateral complete cleft lip and palate; UICL 5 unilateral incomplete cleft lip.

iations intrinsically associated with the cleft, functional adaptations, and surgical iatrogenesis. Improved insight into the
variations intrinsically associated with the cleft malformation
requires extensive studies of the craniofacial morphology at
birth before carrying out any treatment and determination of
the functional adaptations considered to be of relatively low
magnitude.
Several studies of the BCCLP infant maxillary morphology
have been carried out based on plaster casts (Huddart, 1970;
Wada et al., 1984; Berkowitz, 1996; Heidbuchel et al., 1998),
whereas only very few studies have analyzed infant craniofacial morphology from cephalometry (Friede and Pruzansky,
1972; Dahl et al., 1989). The few available cephalometric studies of infants with unoperated BCCLP have focused on the
morphology of the facial skeleton and cranial base as seen in
the lateral cephalometric films. No comprehensive, three-dimensional analysis of craniofacial dysmorphology in infants
with unoperated BCCLP has been carried out to date. The
purpose of the present study was to carry out such an analysis.
In addition, the study examined how early surgical closure of
the clefts in the lip will influence early facial development.
MATERIAL
The present study is part of a major study of infant craniofacial morphology and early growth in all Danish children born
with a cleft in the period 1976 to 1981. The objective of this
investigation was to describe in detail the preoperative facial
morphology and the variability in the cleft condition and to
study craniofacial growth from 2 to 22 months of age. A total
of 678 children with clefts were born in Denmark from 1976
to 1981 (Jensen et al., 1988), and we examined 602 using
three-projection cephalometry. For the present study, longitudinal data (examination at both 2 and 22 months of age) of
children with BCCLP and children with unilateral incomplete
cleft lip (UICL) (lip grades 1 or 2; control group) were selected.
Except from the criterion of diagnosis, the criteria for in-

METHOD
Anthropometric Data
Body length and weight were obtained for all children at
birth and at 2 and 22 months of age.
Cephalometric Data
Acquisition of Three Projection X-Rays
The infant cephalometric unit used for the current investigation was developed and described in detail by Kreiborg et
al. (1977).

TABLE 2 Age Distribution (in Days) at the Time of the First and Second Examinations*
BCCLP
Examination 1

UICL
Examination 2

Examination 1

Examination 2

Group

Mean

Min

Max

Mean

Min

Max

Mean

Min

Max

Mean

Min

Max

Girls
Boys
Combined

58.3
60.8
59.6

52
53
52

69
72
72

650.5
653.3
651.9

618
611
611

691
677
691

73.4
71.3
72.0

50
55
50

91
100
100

711.5
688.3
696.6

654
657
654

750
749
750

* BCCLP 5 bilateral complete cleft lip and palate; UICL 5 unilateral incomplete cleft lip.

425

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

TABLE 3 Mean Body Length (cm) According to Diagnosis and Sex at Birth and at 2 and 22 Months of Age*
BCCLP
Birth

UICL

Examination 1

Examination 2

Birth

Examination 1

Examination 2

Group

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Girls
Boys
Combined

50.75
52.09
51.53

2.25
2.81
2.61

58.69
58.18
58.39

2.58
3.19
2.88

83.29
82.60
82.88

2.93
3.44
3.16

51.31
52.11
51.82

2.96
2.21
2.50

59.41
60.47
60.09

2.52
2.97
2.84

85.19
86.45
85.99

3.71
3.42
3.54

* No significant differences were found in body length between the BCCLP and the UICL group at birth and at 2 months of age, however, at 22 months of age the BCCLP group was significantly
shorter than the UICL group (p , .01). BCCLP 5 bilateral complete cleft lip and palate; UICL 5 unilateral incomplete cleft lip.
Significant difference between the two groups at the 1% level.

Landmark Definition and Digitization

BCCLP had a significantly lower body weight than the UICL

group, and at 22 months of age, the children with BCCLP had
both a significantly lower mean body weight and shorter mean
body length than the UICL group.

The x-rays were analyzed by a modification of the method

developed by Kreiborg (1981). The method has previously
been described by Heller et al. (1995), and Hermann et al.
(2001). The procedure included identification of a total of 279
landmarks, 142 on the lateral projection, 77 on the frontal, and
60 on the axial projection. Using these landmarks, 356 variables were calculated describing all craniofacial regions.

Cephalometric Findings
When comparing the craniofacial morphology of the two
groups, the boys were pooled with the girls. In the UICL
group, the frontal and axial cephalometric films for the patients
with right-sided cleft were mirrored to have all the clefts on
the left side, allowing all children with UICL (n 5 45) to be
compared with all the children with BCCLP (n 5 19). The
craniofacial morphology in the BCCLP and UICL groups was
fairly similar in some regions, but in other regions the two
groups differed significantly. Therefore, craniofacial morphology will be discussed region by region in the following section.

Statistical Methods
Students t test (Hald, 1952) was used to test the significance
of the differences between the means in the BCCLP and the
UICL groups. Growth statistics for a sample population were
carried out as previously described by Hermann et al. (1999a,
2001, 2002, 2003b). The level of significance was set to 1%.
Error of the Method

Craniofacial Morphology at 2 and 22 Months of Age

The error of the landmark digitization ranged from 0.27 to
1.94 mm for linear variables and from 0.36 to 2.97 degrees
for angular variables (Hermann et al., 2001) and was judged
to be acceptable.

Skeletal Morphology
Calvaria. No significant differences in size and shape of the
calvaria were observed between the two groups at 2 or at 22
months of age (Fig. 1).
Cranial base. No significant differences in the size (length
and width) of the cranial base were observed between the two
groups at 2 or at 22 months of age. The cranial base angle
was significantly increased in the BCCLP group at 2 months
of age (Fig. 1A); at 22 months of age, this angle was, however,
found to be similar in the two groups (Fig. 1B).
Orbital region. At 2 months of age the orbital axis, the

RESULTS
Anthropometric Findings
The comparison of body length (Table 3) and body weight
(Table 4) of the children showed no significant differences in
the means between the total BCCLP group and the total UICL
group at birth. However, at 2 months of age, the children with

TABLE 4 Mean Body Weight (kg) According to Diagnosis and Sex at Birth and at 2 and 22 Months of Age*
BCCLP
Birth

UICL

Examination 1

Examination 2

Birth

Examination 1

Examination 2

Group

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Mean

SD

Girls
Boys
Combined

3.28
3.51
3.41

0.61
0.57
0.59

4.39
4.90
4.69

0.44
0.58
0.58

11.24
11.57
11.43

1.66
1.01
1.29

3.42
3.40
3.41

0.66
0.47
0.54

5.16
5.60
5.45

0.68
0.66
0.69

11.98
12.76
12.48

1.48
1.44
1.49

* At birth no significant difference was found between the two groups; however, at 2 and 22 months of age, the BCCLP group had a significantly lower weight than the UICL group (p , .01).
BCCLP 5 bilateral complete cleft lip and palate; UICL 5 unilateral incomplete cleft lip.
Significant difference between the two groups at the 1% level.

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

427

FIGURE 1 A. Two months of age. The lateral mean drawing of the total unilateral incomplete cleft lip (UICL) group (n 545) superimposed on the total
bilateral complete cleft lip and palate (BCCLP) group (n 519). B. Twenty-two months of age. The lateral mean drawing of the total UICL group superimposed on the total BCCLP group.

distance from the sella point to the inferior orbital margin, and
the orbital floor were significantly reduced in length in the
BCCLP group, whereas the length of the orbital roof was similar in the two groups. The lateral orbital margin was significantly retruded in the BCCLP group. Both the outer and the
inner interorbital distances were similar in the two groups (Fig.
2A). At 22 months of age, the length of the orbital axis, the
distance from the sella point to the inferior orbital margin, and
the length of the orbital floor and the orbital roof were somewhat, but not significantly, reduced in the BCCLP group. The
lateral orbital margin was, however, still significantly retruded

in the BCCLP group. The interorbital distances were still similar in the two groups (Fig. 2B).
Nasal bone. The length of the nasal bone was similar in the
two groups at both 2 and 22 months of age. The inclination
of the nasal bone in relation to the anterior cranial base was
decreased in the BCCLP group; the difference was, however,
only significant at 22 months of age (Fig. 1).
Maxilla. At 2 months of age, the total length of the maxilla
from the anterior nasal spine to the pterygomaxillary fissure
was markedly and highly significantly increased in the BCCLP
group. In contrast, the length of the maxilla measured from

FIGURE 2 A. Two months of age. Enlargement of the maxillary region of the frontal mean drawing of the total unilateral incomplete cleft lip (UICL)
group (n 545) superimposed on the mean drawing of the total bilateral complete cleft lip and palate (BCCLP) group (n 519). B. Twenty-two months of
age. Enlargement of the maxillary region of the frontal mean drawing of the total UICL group superimposed on the mean drawing of the total BCCLP
group.

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

FIGURE 3 A. Two months of age. Enlargement of the maxillary region in the axial mean drawing of the total unilateral incomplete cleft lip (UICL) group
(n 545) superimposed on the total bilateral complete cleft lip and palate (BCCLP) group (n 519). Superimposition was carried out according to posterior
part of the maxilla. B. Twenty-two months of age. Enlargement of the maxillary region in the axial mean drawing of the total UICL group superimposed
on the total BCCLP group. Superimposition was carried out according to posterior part of the maxilla.

the infrazygomatic crest to the pterygomaxillary fissure was

markedly and highly significantly decreased in the BCCLP
group. The posterior maxillary height was also significantly
smaller in the BCCLP group than the UICL group (Fig. 1A).
The posterior maxillary width was significantly increased in
the BCCLP group (Fig. 3A).
In the sagittal plane, the maxillary prognathism measured to
the premaxilla was markedly increased in relation to the anterior cranial base in the 2-month-old BCCLP group. The
mean difference between the two groups was about 110%
(Fig. 1A). In contrast, maxillary prognathism measured to the
basal part of the maxilla was markedly and significantly decreased in the BCCLP group. The mean difference was about
27% (Fig. 1A). The width of the nasal cavity was significantly
increased in the BCCLP group. The nasal septum was located
in the midline, and the maxillary complex was symmetric in
all three planes of space (Figs. 2A and 3A).
In the sagittal plane, the deciduous central incisors were
positioned significantly more anteriorly in the BCCLP group
than in the UICL group at 2 months of age. The inclination
of the deciduous central incisors was slightly retroclined in the
BCCLP group, although the difference between the groups was
not significant (Fig. 1A). In the transverse plane, the central
incisors were symmetrically positioned with respect to the
midline in the BCCLP group, whereas the central incisors were
somewhat displaced toward the noncleft side in the UICL
group (Fig. 3A). In contrast to the position of the deciduous
central incisors (located in the premaxilla), the deciduous canines and molars (located in the lateral segments) were positioned significantly more distally in the BCCLP group than in
the UICL group at 2 months of age (Fig. 1A).
The width of the maxillary dental arch in the 2-month-old
BCCLP group was significantly increased at all sagittal levels
(deciduous canines, first and second deciduous molars, and
first permanent molar). The increase was most pronounced distally and least at the level of the canines. The total length of
the dental arch was significantly increased in the BCCLP

group, whereas the lengths of the dental arches in the lateral

segments of the maxilla were similar in the two groups (Fig.
3A).
At 22 months of age, the total length of the maxilla from
the anterior nasal spine to the pterygomaxillary fissure was
slightly but significantly increased in the BCCLP group. The
length of the maxilla measured from the infrazygomatic crest
to the pterygomaxillary fissure was still markedly and significantly reduced in the BCCLP group. The posterior maxillary
height was also still significantly smaller in the BCCLP group
than in the UICL group (Fig. 1B). The posterior maxillary
width (Fig. 2B) was still significantly increased, whereas the
distance (Fig. 3B) was somewhat, but not significantly, increased in the BCCLP group.
In the sagittal plane, the maxillary prognathism measured to
the premaxilla was similar in the two groups at 22 months of
age, whereas the prognathism measured to the basal part of
the maxilla was still marked and significantly decreased in the
BCCLP group. The mean difference was about 28 degrees
(Fig. 1B).
The width of the nasal cavity was still significantly increased in the BCCLP group (Fig. 2B); the posterior part of
the nasal septum was located in the midline, whereas the anterior part deviated somewhat from the UICL group. In the
frontal and axial planes, the premaxilla deviated to the left side
and the lateral maxillary segment on the left side was somewhat closer to the midsagittal plane on the right side (Figs. 2B
and 3B).
In the sagittal plane, the deciduous central incisors were
positioned significantly more distally in the BCCLP group than
in the UICL group at 22 months of age, and the deciduous
central incisors in the BCCLP group were markedly posteriorly
inclined (Fig. 1B). In the transverse plane, the central incisors
were symmetrically positioned with respect to the midline in
the UICL group, whereas the central incisors in the BCCLP
group were displaced to the left side (Fig. 2B). The deciduous

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

canines and molars were positioned significantly more distally

in the BCCLP group than in the UICL group (Fig. 1B).
The width of the maxillary dental arch in the 22-month-old
BCCLP group was still significantly increased at the level of
the first permanent molar, whereas it had become significantly
decreased at the level of the deciduous canine and fist deciduous molar (Fig. 3B). The total length of the dental arch was
significantly decreased in the BCCLP group, whereas the
lengths of the dental arches in the lateral segments of the maxilla were still similar in the two groups.
Mandible. The BCCLP mandible was markedly and highly
significantly reduced in length at both 2 and 22 months of age
(Fig. 1), whereas mandibular height and width were similar in
the two groups. No significant difference was observed in the
gonial angle between the two groups. The mandible was markedly and highly significantly retrognathic in relation to the anterior cranial base in the BCCLP group at both 2 and 22
months of age (Fig. 1). The inclination of the mandible in
relation to the anterior cranial base was increased in the
BCCLP group; the difference between the two groups was,
however, significant only at 22 months of age (Fig. 1B).
Jaw relations. At 2 months of age, the sagittal jaw relations
were extremely and significantly increased (about 117 degrees) in the BCCLP group when measured to the premaxilla.
However, if the sagittal jaw relations were measured to the
basal part of the maxilla, the mean value for the 2-month-old
BCCLP group was only slightly (1.6 degrees), and not significantly, increased. The vertical jaw relation was significantly
decreased in the BCCLP group (see Fig. 1A). At 22 months
of age, the sagittal jaw relations were still increased in the
BCCLP group when measured to the premaxilla. The mean
difference was, however, only about one third (5 degrees) the
size of the difference observed at 2 months of age; the difference was, though, significant. If the sagittal jaw relations were
measured to the basal part of the maxilla, the value for the
BCCLP group was still only slightly increased. The vertical
jaw relation was still somewhat, but not significantly, decreased in the BCCLP group (see Fig. 1B).
Facial height. At 2 months of age, the total anterior facial
height was similar in the two groups, whereas the posterior
facial height was significantly decreased in the BCCLP group
(Fig. 1A). At 22 months of age, the total anterior and posterior
facial heights were both similar in the BCCLP and UICL
groups (Fig. 1B).
Pharynx. At 2 months of age, the nasopharyngeal height
was significantly decreased, whereas the width of the bony
nasopharynx was significantly increased in the BCCLP group
(Figs. 1A and 3A). Furthermore, the hyoid bone was positioned significantly closer to the cranial base and somewhat,
but not significantly, closer to the cervical column in the
BCCLP group than in the control group (Fig. 1A). At 22
months the nasopharyngeal height was still significantly decreased in the BCCLP group (Fig. 1B), and the width of the
bony nasopharynx was somewhat, but not significantly, increased in the BCCLP group (Fig. 3B). The hyoid bone was
positioned at a normal distance to the cranial base and some-

429

what, but not significantly, closer to the cervical column in the

BCCLP group than in the control group (Fig. 1B).
Cervical column. In the 2-month-old BCCLP group, the
height of the cervical column from c2 to c4 was significantly
decreased. The angle between the cervical column and the anterior cranial base was similar in the two groups, indicating a
normal head-to-neck relation in the BCCLP group (Fig. 1A).
At 22 months the height of the cervical column from c2 to c4
was somewhat, but not significantly, decreased in the BCCLP
group. The angle between the cervical column and the anterior
cranial base was significantly increased in the BCCLP group,
indicating an extension of the head in relation to the cervical
column (Fig. 1B).
Soft Tissue Morphology
Nose. At 2 months of age, the height and length of the nose
were significantly reduced in the BCCLP group, and the shape
of the BCCLP nose was significantly flatter. The inclination of
the dorsum of the nose to the facial plane and to the anterior
cranial base was significantly decreased in the BCCLP group
at 2 months of age. At 22 months the height and length of the
nose was still significantly reduced, and the nose was still significantly flatter in the BCCLP group than in the control group.
The inclination of the dorsum of the nose was still significantly
decreased in the BCCLP group.
Upper and lower lips. At 2 months of age, the height of the
upper lip was significantly shorter in the BCCLP group than
in the UICL group.
The thickness of the upper lip was similar in the two groups,
but the lip in the BCCLP group was significantly protruded in
relation to the anterior cranial base. The nasolabial angle was
significantly decreased in the BCCLP group, compared with
the UICL group. At 22 months the height of the upper lip was
somewhat, but not significantly, longer in the BCCLP group
than in the UICL group. The thickness of the upper lip was
still similar in the two groups, but the lip in the BCCLP group
was significantly retruded in relation to the anterior cranial
base. The nasolabial angle was markedly and highly significantly increased in the BCCLP group, compared with the
UICL group. There were no significant differences in the size
or shape of the lower lip when comparing the BCCLP group
with the UICL group at 2 months and at 22 months of age.
Chin. There were no significant differences in the shape of
the chin between the groups at 2 months and at 22 months of
age. However, in the BCCLP group, the chin was significantly
retruded in relation to the anterior cranial base, and the thickness of the soft tissue chin was increased both at 2 and 22
months of age (Fig. 1).
Sagittal facial relations. At 2 months of age, the sagittal
facial relations showed significantly increased mean values in
the BCCLP group, compared with the UICL group. At 22
months, the sagittal facial relations were somewhat, but not
significantly, increased in the BCCLP group, compared with
the UICL group.
Facial height. The upper and lower facial heights were sig-

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

nificantly reduced in the BCCLP group at 2 months of age. At

22 months of age, the upper facial height was significantly
increased and the lower facial height was somewhat, but not
significantly, increased in the BCCLP group.
Pharynx. Both the depth and height of the pharyngeal airway were significantly decreased in the 2-month-old BCCLP
group. The mean pharyngeal area (Fig. 1A) was significantly
reduced (by about 26%) in the BCCLP group when compared
with the UICL group. At 22 months of age, the depth of the
pharyngeal airway was still significantly decreased in the
BCCLP group, whereas the height of the pharyngeal airway
was similar in the two groups. The mean pharyngeal area (Fig.
1B) was still significantly reduced (by about 28%) in the
BCCLP group when compared with the UICL group at 22
months of age.
Eye. At 2 months of age, the globe of the eye was significantly protruded in relation to the orbital opening, whereas the
globe to sella distance was similar in the BCCLP and UICL
group. At 22 months the globe of the eye was somewhat, but
not significantly, protruded in relation to the orbital opening,
whereas the globe to sella distance was still similar in the
BCCLP and UICL groups (Fig. 1).
Ear. At 2 months of age, the mean height and the vertical
position as well as the angulation of the ear showed no significant differences between the two groups. At 22 months the
mean height was slightly but significantly increased, and the
angulation of the ear was significantly decreased. However, in
the sagittal plane, the ears were positioned similarly in the
BCCLP and UICL group at 22 months of age (Fig. 1).
Growth
Quantitative Analysis
The average craniofacial growth from 2 to 22 months of age
in the BCCLP group and the UICL group was analyzed from
superimposed mean drawings representing the lateral, frontal,
and axial projections. The mean length, mean x- and y- components of the length, and direction (in relation to the line of
superimposition) of each growth vector were calculated in both
groups. The differences between the means in the two groups
were tested using Students t test. The p values were color
coded onto the vector representation of the growth in the
BCCLP group as shown in Figures 4 through 7. Figures 4A,
5A, and 6A illustrate the significance of the differences in
mean length of the vectors in the two groups in terms of p(t).
Figures 4B, 4C, 5B, 5C, 6B, and 6C illustrate the significance
of the differences in the mean direction of the vectors in the
two groups in terms of p(t)(x) and p(t)(y). Figure 7 shows the
differences in direction of growth p(t)(dir) for the face (maxilla
and mandible) and soft tissue profile. In the following, only
differences significant at the 1% level (red vectors) will be
considered.

Lateral Projection
As seen in Figure 4A, the vast majority of the growth vectors were longer in the BCCLP group than in the control group
except in the region of the nose and the premaxilla. However,
the mean length of only 33 of 141 lateral vectors differed
significantly between the BCCLP and UICL groups (Fig. 4A).
Twenty-six of the vectors were significantly longer and seven
vectors were significantly shorter in the BCCLP group. Four
vectors were related to the BCCLP maxilla; one vector was
increased in length and three reduced vectors were related to
the premaxilla. Six increased vectors were related to the mandible in the BCCLP group. Three decreased vectors were related to the soft tissue nose or nasal tip, whereas two vectors
belonging to the upper lip, four to the lower lip, and four to
the soft tissue chin all were increased in length in the BCCLP
group. Furthermore, three increased vectors were related to the
cervical spine and three to the calvaria. One increased vector
was related to the hyoid bone, one to the orbit, one to the ears,
and a single decreased vector was related to the soft tissue
profile in the BCCLP group.
Looking at differences in the amount of growth in the sagittal direction (x-direction), 42 vectors differed significantly
between the BCCLP and UICL groups (Fig. 4B). Nine vectors
were significantly longer and 33 vectors were significantly
shorter in the BCCLP group. Eleven decreased vectors were
related to the maxilla in the BCCLP group, four were related
to the mandible; five to the soft tissue nose, three to the upper
lip, and six to the soft tissue chin. Three increased vectors were
related to the cervical spine, one increased vector to the cranial
base, and one decreased vector to the soft tissue profile. In the
pharynx two vectors were decreased and one was increased in
length. Furthermore, three vectors related to the calvaria were
increased in length.
In the vertical direction (y-direction; Fig. 4C), 35 vectors
showed a significantly different length in the BCCLP group
than in the control group, 34 of the vectors were longer, and
one was significantly shorter in the BCCLP group. Three increased vectors were related to the maxillary complex, 10 to
the mandible, four vectors to the soft tissue nose, three vectors
to the upper lip, three vectors to the lower lip, five vectors to
the soft tissue chin, three vectors to the cervical spine, a single
vector to the orbital complex and the hyoid bone in the BCCLP
group. The soft tissue profile in the BCCLP showed one increased and one reduced vector.
Frontal Projection
For the frontal projection, 7 of the 74 mean length vectors
showed significantly different lengths in the BCCLP group,
compared with the UICL group (Fig. 5A); two vectors were
longer, and five vectors were shorter in the BCCLP group.
Four vectors related to the nasal cavity were reduced in length;
so was a single vector related to the orbital complex. Two
vectors related to the incisors in the mandible were increased
in length.

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

431

FIGURE 4 The significance of the differences in the lateral projection in the mean direction of the vectors in the two groups in terms of the total length
p(t) (A), the length in the x-direction p(t)(x) (B), and the length in the y-direction p(t)(y) (C). Vectors with filled arrowheads indicate a larger growth in the
bilateral complete cleft lip and palate (BCCLP) group than in the control group (unilateral incomplete cleft lip), whereas vectors with unfilled arrowheads
indicate a smaller growth in the BCCLP group than in the control group.

In the horizontal direction (x-direction), seven vectors were

significantly different in the BCCLP group, compared with the
UICL group (Fig. 5B), five vectors were longer, and two were
shorter in the BCCLP group. Two increased vectors were related to the maxillary complex and three to the mandible in
the BCCLP group. One reduced vector was related to the nasal
cavity and one to the orbital complex.
In the vertical direction (y-direction), 10 vectors were significantly different in the BCCLP group, compared with the
UICL group (Fig. 5C), three vectors were longer, and seven
were shorter in the BCCLP group. Two increased vectors were
related to the mandible and one to the orbital complex. Five
decreased vectors were related to the nasal cavity, one to the
cranial base, and one to the orbital complex.

Axial Projection
For the axial projection, 23 of the 61 vectors mean lengths
vectors showed significantly different lengths in the BCCLP
group, compared with the UICL group (Fig. 6A shows zoomed
plots of the maxilla); 20 of the vectors were longer, and three
vectors were shorter in the BCCLP group. Six of the vectors
belonged to the maxilla, three were increased in length, and
three vectors were decreased in length in the BCCLP group.
Ten vectors that were all increased in size belonged to the
mandible in the BCCLP group. Also, six vectors in the cranial
base and one in the calvaria were found to be increased in the
BCCLP group.
Looking at the amount of growth in the horizontal direction

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

FIGURE 5 The significance of the differences in the frontal projection in the mean direction of the vectors in the two groups in terms of the total length
p(t) (A), the length in the x-direction p(t)(x) (B), and the length in the y-direction p(t)(y) (C). Vectors with filled arrowheads indicate a larger growth in the
bilateral complete cleft lip and palate (BCCLP) group than in the control group (unilateral incomplete cleft lip), whereas vectors with unfilled arrowheads
indicate a smaller growth in the BCCLP group than in the control group.

(x-direction), 14 vectors in the BCCLP group were significantly different in the BCCLP group, compared with UICL
(6B); seven vectors were longer, and seven vectors were shorter in the BCCLP group. Seven of the vectors belonged to the
maxilla; two of these were increased in length and five vectors
were decreased in length. Three vectors belonged to the mandible: a single vector was increased in length and two vectors
were decreased in length. Three increased vectors were related
to the cranial base and one to the calvaria. In the sagittal direction (y-direction), 25 vectors were significantly different in
the two groups: 22 vectors were longer and three vectors were
shorter in the BCCLP than in the UICL group (Fig. 6C). Seven
vectors belonged to the maxilla: four vectors were increased
in length and three vectors were decreased in length. Thirteen

vectors belonged to the mandible. Furthermore, five increased

vectors belonged to the cranial base.

Growth Direction of the Face

Looking at the growth direction of the face and soft tissue

profile in the lateral cephalometric projection in the BCCLP
group, the growth pattern differed most in the maxilla and
mandible (Fig. 7). Both jaws showed a more vertical growth
pattern in the BCCLP group than in the control group with
UICL. Also, the nonsignificant vectors in the region showed a
similar tendency.

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

DISCUSSION
Anthropometric Findings
The normal body weight and length at birth, decrease in
body weight at 2 months of age, and decrease in body weight
and length at 22 months of age in the BCCLP group might be
explained in part by the somewhat younger mean age in this
group than in the control group at both examinations and partly
by early feeding problems and recurrent infections of the upper
airways in young children with BCCLP (Jensen et al., 1988).
It is noteworthy that Dahl (1970) found the adult stature in his
sample of subjects with bilateral cleft lip and palate to be significantly reduced, compared with a control group. Dahl
(1970) did not discuss the cause of the reduced body height,
but it could probably be explained by early feeding problems,
respiratory distress, and recurrent infections of the upper airways.
Cephalometric Findings
The statistical analysis revealed that the average craniofacial
morphology in the children with BCCLP deviated significantly
from the morphology in the children with UICL. In the following the craniofacial morphology will be discussed region
by region, and the growth will be discussed in terms of the
amount and direction of growth.
Skeletal Morphology
Calvaria. The present study showed that the primary size
and shape of the calvaria in BCCLP was within normal limits.
In contrast, Dahl (1970) found in individuals with adult bilateral cleft lip and palate that the calvaria was reduced in length
and height. This difference may be related to the fact that
Dahls adult sample had a reduced body height as mentioned
above.
Cranial base. To our knowledge, no previous studies of the
cranial base in infants with BCCLP have made comparison
with UICL or normal infants. Our findings are in accordance
with the findings of Smahel (1984), who found a normal cranial base in adults with surgically-repaired BCCLP. In contrast,
Dahl (1970) found the cranial base to be diminished in length
and the cranial base angle to be flatter in adults with surgicallyrepaired bilateral cleft lip and palate than in noncleft individuals, and da Silva Filho et al. (1998a) found smaller cranial
base dimensions but a normal cranial base angle in adults with
unoperated BCCLP.
Orbital region. A similar interorbital distance in infants with
unoperated BCCLP and UICL has to our knowledge not been
described before. Previous studies have reported increased interorbital distances in adults with surgically-repaired BCCLP,
compared with individuals without clefts (Dahl, 1970; Smahel,
1984). However, several investigators (Dixon, 1966; Ishiguro
et al., 1976; Friede et al., 1986) have reported that there is a
tendency to skeletal hypertelorism in individuals with cleft of

433

the primary palate, which may be the explanation for our findings (i.e., both the BCCLP and UICL groups could have a
somewhat increased interorbital distance, compared with normal values; this was, however, not examined in the present
study). Our finding of a retruded lateral orbital margin in children with BCCLP is probably related to the retrusion of the
maxillary and zygomatic complex.
Maxilla. The most obvious abnormal facial trait in infants
with unoperated BCCLP is the extreme protrusion of the premaxilla. The dramatic facial dysmorphology is caused by a
combination of a premaxilla, which is truly protruding in relation to the anterior cranial base, and the lateral segments (the
basal parts of the maxilla), which are markedly retrognathic in
relation to the anterior cranial base. A protruding premaxilla
in BCCLP has repeatedly been reported in the literature in
infants and children with and without surgical repair (e.g.,
Huddart, 1970; Pruzansky, 1971; Friede and Pruzansky, 1972;
Dahl et al., 1989; Trotman and Ross, 1993; Berkowitz, 1996)
as well as in adults with unoperated clefts (da Silva Filho et
al., 1998a). The cause of the protrusion has been shown to be
overgrowth in the vomeropremaxillary suture (Pruzansky,
1971; Friede and Morgan, 1976) as a consequence of lack of
restraint from a united maxilla (Latham, 1973; Atherton, 1974;
Friede, 1977).
Retrognathia of the basal parts of the maxilla in infants with
BCCLP has previously been reported only by Dahl et al.
(1989) in a study that partly included subjects from the same
population as the present study. However, several of our own
previous studies have documented that infants born with a cleft
of the secondary palate, with or without a cleft of the primary
palate (unilateral complete cleft lip and palate [UCCLP], isolated cleft palate [ICP], and Robin sequence [RS]), have an
intrinsic maxillary retrognathia (Hermann et al., 1999b, 2002,
2003a, 2003b; Kreiborg and Hermann, 2002). Trotman and
Ross (1993) found the posterior segments of the maxilla to be
hypoplastic in 6-year-old children, adolescents, and adults with
BCCLP.
Prior to surgical closure of the clefts in the lip, at about 2
and 4 months of age, respectively, the position of the premaxilla deviated to the right or the left side in a number of cases,
but on average the premaxilla was positioned symmetrically
around the midsagittal plane. In principle, the side on which
the cleft lip was the widest was closed first (around 2 months
of age), and the other side was closed later (around 4 months
of age). However, the widths of the clefts on the two sides did,
in general, not vary much, but in reality the cleft lip on the
left side was closed first in the majority of cases (83%), probably because cleft lip in general is much more common on the
left side than on the right side (2:1), which means that the
surgeon has the most experience with closure of left-sided
clefts and therefore, intuitively, chooses to close this side first
in bilateral cases. At 22 months of age, the effect of lip surgery
on maxillary development could be evaluated. The force from
the united upper lip produces a backward pressure, primarily,
on the premaxilla, which results in a restraint of forward
growth in the vomeropremaxillary suture leading to a relative-

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

FIGURE 6 The significance of the differences in the axial projection in the mean direction of the vectors in the two groups in terms of the total length
p(t) (A), the length in the x-direction p(t)(x) (B), and the length in the y-direction p(t)(y) (C). Vectors with filled arrowheads indicate a larger growth in the
bilateral complete cleft lip and palate (BCCLP) group than in the control group (unilateral incomplete cleft lip), whereas vectors with unfilled arrowheads
indicate a smaller growth in the BCCLP group than in the control group.

ly more normal sagittal position of the premaxilla in relation

to the anterior cranial base at 22 months of age. In fact, on
average, the premaxilla in the BCCLP group was in the same
sagittal position at 22 months of age as in the UICL group.
This position is, however, hardly normal in relation to the basal
part of the maxilla in the BCCLP group, which is intrinsically
retrognathic.
It was noteworthy that the total length of the maxilla measured to the anterior nasal spine was still significantly increased in the BCCLP group at 22 months of age. On average,
the relative retrusion of the premaxilla was combined with a
distal tilt and a lateral shift to the left. These movements prob-

ably occurred at the level of the vomeropremaxillary suture

and can be explained by lack of bony union between the premaxilla and the lateral segments. The average shift of the premaxilla to the left side can be explained by the fact that the
cleft lip on the left side was closed first in 83% of the cases.
The first lip closure probably led to a rather immediate shift
of the premaxilla to the operated side because of soft tissue
traction, and it is suggested that this shift was partly maintained even after surgical closure of the other side of the lip
at around 4 months of age. It would be conceivable that the
early iatrogenic result of the two-stage lip closure would lead
to a long-lasting maxillary asymmetry.

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

435

FIGURE 7 The significance of the differences in the direction of the mean facial growth pattern in bilateral complete cleft lip and palate (BCCLP) and
unilateral incomplete cleft lip (UICL; lateral projection). Vectors with filled arrowheads indicate a larger angle of the vector in relation to nasion-sella line,
meaning a more vertical growth relation in the BCCLP group than in the control group (UICL), whereas vectors with unfilled arrowheads indicate a smaller
angle of the vector in relation to nasion-sella line, meaning less vertical growth in the BCCLP group than in the control group.

It should, however, be mentioned that since about 1990 the

lip surgery protocol for BCCLP in Denmark has been changed
to an early bilateral lip-adhesion procedure followed by a later
bilateral complete closure.
Reduced posterior height of the maxilla in infants with unoperated BCCLP has previously been described only by Dahl et
al. (1989) in a study that partly included subjects from the same
population as the present study. A similar finding has been reported in older children, adolescents, and adults (Dahl, 1970;
Trotman and Ross, 1993). A reduced posterior height of the maxilla was previously reported in subjects with unoperated UCCLP
(Hermann et al., 1999b). It would seem that the finding is related
to the cleft malformation of the secondary palate. The deviation
persisted relatively unaltered from 2 months of age to 22 months
of age in the present study and is thus not influenced by the
growth change induced by lip surgery.
Increased width of the posterior maxilla and the nasal cavity
in infants with unoperated BCCLP are probably the result of
lack of restraint from a unified maxilla. Similar findings have
been observed in both infants with UCCLP and those with
ICP (Hermann et al., 1999a, 1999b, 2000; Kreiborg and Hermann, 2002). The increase in width was most pronounced posteriorly and least at the level of the deciduous canines. The lip
surgery would seem to influence the transverse maxillary development. Both lateral segments exhibited some degree of

collapse anteriorly, more so on the left side than on the right

side. The side difference was probably related to, on average,
the earlier surgery on the left side than on the right side. At
22 months of age, the transverse distance at the deciduous
canines and first molars had become significantly decreased in
the BCCLP group, whereas the maxilla remained wider at the
level of the second deciduous molar, first permanent molar,
and the tuberosities. The transverse collapse of the lateral segments, thus, seems to occur as a rotation with the center of
rotation located at the maxillary tuberosities. The width of the
nasal cavity remained increased at 22 months of age, indicating
that this measurement in the frontal radiograph probably represents the posterior part of the nasal cavity.
The position of the maxillary primary teeth followed the
changes of the bony structures resulting in, in general, a distal
tilt and a lateral tilt of the incisors and a medial shift of the
canines and first molars; which may explain some of the malocclusion traits typically observed later in childhood and adolescence (e.g., Berkowitz, 1996; Lidral and Vig, 2002).
In conclusion, it seems likely that an extremely protruding
premaxilla, an increased maxillary width, a decreased basal
length, and posterior maxillary height as well as maxillary
retrognathia in relation to the anterior cranial base are all part
of the primary anomaly in BCCLP. Lip surgery would seem
to influence early maxillary development in both a beneficial

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

as well as an iatrogenic manner. The beneficial effect is the

relative retrusion of the extremely protruding premaxilla. The
iatrogenic effects are the tilt of the premaxilla in the sagittal
and transverse planes and probably the medial collapse of the
lateral segments of the maxilla. It is, however, noteworthy that
da Silva Filho et al. (1998b) found collapse of the lateral maxillary segments in adults with unoperated BCCLP. This finding
would seem to indicate that collapse found in the present sample could partly be explained as the result of growth and function and partly by surgery.
Mandible. In the infants with unoperated BCCLP, the mandible was found to be short and retrognathic. Dahl et al. (1989)
reported similar findings, but that study partly included subjects from the same population as the present study. Mandibular retrognathia in American infants with unoperated BCCLP
was reported by Friede and Pruzansky (1972).
It has repeatedly been reported in the literature that older
children with BCCLP (Trotman and Ross, 1993) and adults
with surgically-repaired (Dahl, 1970; Smahel, 1984) and unoperated (da Silva Filho et al., 1998a) BCCLP have mandibular retrognathia. None of these studies have, however, been
able to reveal whether this finding can be primarily related to
the cleft malformation or whether it is caused by secondary
factors such as growth and functional adaptations.
We previously suggested that a short and retrognathic mandible is part of the primary anomaly in infants born with a
cleft of the secondary palate; with or without clefting of the
primary palate (Hermann et al., 1999b, 2002, 2003a, 2003b;
Kreiborg and Hermann, 2002). Such a correlation has previously been shown to exist in animal experiments (Diewert,
1979; Diewert and Pratt, 1979).
The inclination of the mandible in relation to the anterior
cranial base was normal in the infants with unoperated BCCLP
in the present study, but the inclination increased with time.
An increased inclination of the mandible in relation to the
anterior cranial base has previously been observed in older
children with surgically-repaired BCCLP (Trotman and Ross,
1993) and in adults with surgically-repaired bilateral cleft lip
and palate (Dahl, 1970) and in adults with unoperated bilateral
cleft lip and palate (da Silva Filho et al., 1998a).
Based on the findings in the present study, it is suggested
that an increased mandibular plane angle is not part of the
primary anomaly in BCCLP but that it is rather the result of
growth and functional adaptations.
Jaw relations. The extreme increase in the sagittal jaw relations in the infants with unoperated BCCLP was partly
caused by the markedly protruding premaxilla and partly by
mandibular retrognathia. This finding is in agreement with the
observations of markedly increased facial convexity in infants
with unoperated BCCLP by Friede and Pruzansky (1972) and
Friede (1977). In the present study, it was noteworthy that the
sagittal jaw relations were fairly normal in the BCCLP group
when measured to the basal part of the maxilla. This finding
shows that bimaxillary retrognathia is actually characteristic of
the group and that the degree of retrognathia in the two jaws
is fairly similar. Furthermore, these findings support the hy-

pothesis that bimaxillary retrognathia is characteristic of infants born with a cleft of the secondary palate; with or without
clefting of the primary palate (Hermann et al. 1999b, 2002,
2003a, 2003b; Kreiborg and Hermann, 2002).
At 22 months of age, the marked reduction of the sagittal
jaw relations, when measured to the premaxilla, can be explained by the relative retrusion of the premaxilla secondary
to lip surgery at about 2 months of age (see above).
The fact that the sagittal jaw relations measured to the premaxilla are still significantly increased at 22 months of age in
the BCCLP group would seem to support the idea that the
premaxilla is still relative protrusive at this age, although it is
at the same sagittal level as in the UICL group. It was noteworthy that the sagittal jaw relations measured to the basal
part of the maxilla did not change much from 2 to 22 months
of age in the BCCLP group or in the UICL group.
The vertical jaw relation was significantly decreased in the
BCCLP group at 2 months of age. This finding can be explained by the backward inclination of the nasal floor in this
group, a deviation that is probably related to the primary
anomaly. At 22 months of age, the vertical jaw relation in the
BCCLP group had reached an almost average value, not because of a normalization of the inclination of the nasal floor
but because of a more backward inclination of the mandible
in relation to the anterior cranial base and to the nasal floor.
Pharynx. The decreased height of the bony nasopharynx in the
BCCLP group at both 2 and 22 months of age can be explained
by the reduced posterior height of the maxilla. The reduced height
and increased width of the nasopharynx in the BCCLP group are
both related to the primary maxillary anomaly.
The hyoid bone was positioned higher and closer to the
cervical column in the BCCLP group at 2 months of age.
These findings can be explained by the reduced posterior face
height and the mandibular retrognathia found in this group.
At 22 months of age, the vertical position of the hyoid bone
had normalized and so had the size of the posterior facial
height. In contrast, the sagittal position of the hyoid bone remained closer to the cervical column reflecting the persistent
mandibular retrognathia in the BCCLP group. The head at this
age was found to be in an extended position in relation to the
cervical column probably as a compensatory function to protect the airway.
Soft Tissue Morphology
Nose. At 2 months of age, the flatter BCCLP nose with a
decreased inclination of dorsum nasi in relation to the anterior
cranial base is probably related to the basal maxillary retrognathia observed in the group. At 22 months of age, the nose
had become even flatter, probably secondary to the surgical
closure of the lip creating tension in the soft tissues.
Upper lip. The protrusion of the upper lip and the decreased
nasolabial angle found in the BCCLP group at 2 months of
age can be explained by the extremely protruding premaxilla.
However, about 2 years of age, at 18 to 20 months after lip
closure, the backward forces from the united upper lip had led

Hermann et al., BILATERAL COMPLETE CLEFT LIP AND PALATE

to a restraint of forward growth in the vomeropremaxillary

suture and to a distal tilt of the premaxilla, which could explain
the development of retrusion of the upper lip and the markedly
increased nasolabial angle.
Chin. The retrusion of the chin in the BCCLP group at both
2 and 22 months of age reflects the mandibular retrognathia
observed in the group, although the thickness of the soft tissue
chin was increased in the BCCLP group.
Pharynx. The primary cause for the observed size reduction
of the BCCLP pharynx was the shorter and more retrognathic
mandible leading to a more retruded position of the tongue.
We have previously shown that infants with cleft of the secondary palate with or without clefting of the primary palate
have a reduced size of the pharyngeal airway (Hermann et al.
1999b, 2002, 2003a, 2003b; Kreiborg and Hermann, 2002). It
would seem that the distance from the dorsum of the tongue
to the posterior pharyngeal wall is especially significant in relation to breathing problems as seen in Robin sequence (Hermann et al., 2003b). Kreiborg and Cohen (1996) also described
how the combination of isolated cleft palate, a short mandible
with a retruded tongue, and cranial base kyphosis can lead to
respiratory distress (RS) in extreme cases of Treacher Collins
syndrome.
Does the Facial Growth in BCCLP Differ From the Norm?
The present study showed that the average amount of
growth from examination 1 to examination 2, in general,
would seem to be somewhat greater in the BCCLP group than
in the UICL group. The explanation for this finding could be
the slightly younger mean age of the BCCLP group at the first
examination. The most significant differences were related to
the growth vectors of the mandible; in particular, the vertical
component was increased. This finding could be related to a
more vertical mandibular growth pattern in the BCCLP group
with a backward growth rotation of the mandible. A possible
difference in the mean amount of mouth opening at examination 2 between the groups could also influence this finding.
However, Pruzansky and Lis (1958) found that the mandible
assumes its rest position when the infant is placed in a supine
position and that this is stable and reproducible.
The forward growth of the nose and premaxilla was reduced, whereas the vertical growth of this region was generally
increased. These findings can be related to the restraining force
from the surgically united lip producing a backward pressure
on the premaxilla and a downward pull in the soft tissue nose.
The change in the morphology of the upper lip can be explained in a similar manner.
The BCCLP group revealed a more vertical growth pattern
of the face than the UICL group; however, the lowering of the
nasal floor, as seen in the frontal projection, was significantly
reduced in the BCCLP group. A similar reduced lowering of
the nasal floor was previously reported on the cleft side in
young children with UCCLP (Hermann, 1999a). An increased
vertical development of the face has previously been reported
in older children and adults with surgically-repaired BCCLP

437

and with unoperated BCCLP (Dahl, 1970; Trotman and Ross,

1993; da Silva Filho, 1998a).
It is therefore suggested that the increased vertical development
of the BCCLP face is a functional growth adaptation that can be
related to the decreased size of the airway and the resultant extended head posture observed in the present study and also in
previous studies on CLP (Sandham and Foong, 1997).
The relative decrease in the width of the maxilla during
growth could partly be explained by the surgical closure of
the lip and partly by functional factors (e.g., lowering of the
tongue).
It might therefore be concluded that the facial growth pattern in the BCCLP group was more vertical than in the control
group with UICL. A similar tendency has previously been reported in children with UCCLP (Hermann, 1999a), and it is
suggested that the growth pattern is related to the intrinsic
bimaxillary retrognathia, which is characteristic of both infants
with BCCLP and infants with UCCLP.
CONCLUSION
The present longitudinal study showed that the facial morphology in 2- and 22-month-old children with BCCLP differed
significantly from that of a control group with UICL with a
similar age. In both groups, surgical closure of the lip was
carried out at about 2 months of age. The craniofacial morphology and growth in the control group with UICL has previously been analyzed (Hermann et al., 1999a, 1999b, 2000).
The most pronounced deviations in the craniofacial morphology in the BCCLP group at 2 months of age were found
in the maxillary complex, the mandible, and the pharynx. The
most striking findings were an extremely protruding premaxilla; a markedly increased posterior maxillary width; an increased width of the nasal cavity; a short maxilla with reduced
posterior height; a short mandible; bimaxillary retrognathia;
and severe reduction in the size of the pharyngeal airway.
These findings are suggested to be intrinsic variations related
to the cleft condition.
Lip surgery would seem to influence early facial development in a generally beneficial manner, (i.e., it causes a relative
retrusion of the premaxilla that leads to a decrease in extreme
facial convexity observed prior to surgery). However, lip surgery has some iatrogenic effects on maxillary development
(i.e., the relative premaxillary retrusion and distal tilt of the
premaxilla occurs in an uncontrolled manner in all three planes
of space). Furthermore, lip surgery contributes to an excessive
transverse collapse in the anterior part of the lateral segments.
Furthermore, it seems conceivable that the early iatrogenic result of the two-stage lip closure might lead to a long-lasting
maxillary asymmetry.
We have, in previous studies, described the craniofacial
morphology of young children with unoperated UCCLP, ICP,
and nonsyndromic RS and have found that these groups had
only local deviations in the facial morphology, and their common most striking deviations were a wide and posteriorly short
maxilla, a short mandible, and bimaxillary retrognathia. Also,

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Cleft PalateCraniofacial Journal, July 2004, Vol. 41 No. 4

these groups all showed a vertical facial growth pattern that

might be more or less pronounced. Our findings indicate that
the facial type described above might be a liability factor
(Fraser 1976) that increases the probability of developing cleft
lip and palate.
REFERENCES
Atherton JD. The natural history of the bilateral cleft. Angle Orthod. 1974;44:
269278.
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