CLEFT LIP I: PRIMARY DEFORMITIES
Marcello Pantaloni MD and H Steve Byrd MD
HISTORY
The following historical notes on the surgery of
cleft lip (CL) are excerpted from the chapter by Still
and Georgiade1 in Symposium on Management
of Cleft Lip and Palate and Associated Deformi-
ties, from 1974. Still and Georgiade1 illustrate vari-
ous methods of unilateral cleft lip repair (Fig 1).
The first cleft lip repair was reportedly performed
by an unidentified Chinese physician in about 390
AD. The Flemish surgeon Yperman (1295-1350) is
credited with the original description of a proce-
dure that cut the cleft edges and sutured the mar-
gins with needle and twisted waxed thread, rein-
forcing the closure with harelip needles secured
by a figure-of-8 tie.
Fig 1. Various methods of cleft lip repair. (Reprinted with permission from Still JM Jr, Georgiade NG: Historical Review of Management
of Cleft Lip and Palate. In: Georgiade NG (ed), Symposium on Management of Cleft Lip and Palate and Associated Deformities.
St Louis, Mosby, 1974. Vol 8, Ch 3.)
Although the term lagocheilos is ascribed to
Galen, later popular use of the term harelip derives
from Johnson’s translation in 1649 of Ambroise
Pare’s writings about bec de lievre (lip of the hare),
around 1575.
Early techniques of CL repair involved a straight-
line closure, such as in the operations proposed by
Rose2 and Thompson.3
The concept of closure of the cleft lip by local
flaps was introduced by Malgaigne4 in 1843. The
next year Mirault5 modified Malgaigne’s method
by bringing the lateral flap across the cleft. Mirault’s
variation filled the medial deficit with a lateral flap;
all subsequent methods of lip closure are essen-
tially based on this principle.
SRPS Volume 9, Number 21

In 1884 Hagedorn6 applied the Z-plasty tech-

nique to cleft lip closure while popularizing the use
of a rectangular flap in the repair.
The first half of the 20th century was devoted to
the established straight-line closure. In the 1930s
and ’40s, however, the Blair-Brown7 and Brown-
McDowell8 repairs—modifications of Mirault’s origi-
nal procedure—dominated the field of cleft lip sur-
gery. Their techniques are based on a triangular
flap brought into the lower portion of the lip.
LeMesurier 9 and Tennison 10 independently
modified the technique of lateral flap tissue trans-
ferred into the lower portion of the lip.
LeMesurier’s9 innovation consisted of a quadrilat-
eral flap whereas Tennison’s10 involved a triangu-
lar flap; both introduced tissue into the lower part
of the lip and shared the advantage of producing
a pouting of the tubercle. Subsequently Wynn11
and later Davies12 described variations of triangu-
lar flaps introduced into the upper lip. These
repairs enjoyed a great deal of popularity in the
Fig 2. Formation of the neural tube in the developing embryo.
1950s and early ’60s. (A) Beginning involution of the neural ectoderm. (B) Neural
In 1955 Millard13 developed the concept of crest cells differentiate into ectomesenchyme.
lateral flap advancement into the upper portion of
the lip combined with downward rotation of the As the neural tube takes shape, neural crest cells
medial segment. His technique preserves both differentiate from the ectoderm and effectively sepa-
the Cupid’s bow and the philtral dimple and places rate the neuroectoderm of the neural tube from
the tension of the closure under the alar base, the covering cutaneous ectoderm. These special
thereby reducing flare and promoting better mold- neural crest cells, although of ectodermal origin,
ing of the underlying alveolar process. Millard’s exhibit most of the properties associated with mes-
repair has withstood the test of time and remains enchyme, so that the tissue they form is termed
a most popular method for closure of the unilat- ectomesenchyme.
eral cleft lip. The ectomesenchyme migrates along the natu-
Skoog14 and Trauner and Trauner15 indepen- ral cleavage planes between the mesoderm, ecto-
dently described procedures involving a combina- derm, and endoderm. In migration from their site
tion of two simultaneous flaps in both the upper of formation, the neural crest cells have no prede-
and lower portions of the lip. termined pattern of travel; their complex yet highly
organized migratory behavior is largely directed by
local factors in their environment.16 The migration
EMBRYOLOGY of this ectomesenchyme over and around the head
The mesoderm involved in facial development is essential to the development of the facial pro-
is believed to be a separate tissue akin to the pri- cesses17 (Fig 3). Failures in the migratory pattern
mary germ cell types and of ectodermal origin. and path of this ectomesenchymal tissue are
This specialized tissue begins to differentiate on or responsible for clefting in the face, lip, and palate.
about day 21 of gestation as the ectoderm in the In the normal sequence, the frontonasal pro-
vicinity of the neural plate folds on itself to form the cess evolves over the developing brain. Maxillary
neural tube (Fig 2). At this point the embryo is and mandibular processes evolve around the sto-
approximately 3 mm long. modeum and the branchial arches18 (Fig 4).

2

Fig 3. Migratory pattern of ectomesenchyme to form the facial
processes. (Reprinted with permission from Millard DR JR: Cleft
Craft—The Evolution of Its Surgery. I. The Unilateral Deformity.
Boston, Little Brown, 1976.)
Fig 4. Some features of the human embryo at 5 to 6 weeks of
gestation. (Copyright 1976, CIBA Pharmaceutical Co, a division
of CIBA-Geigy Corporation. Reprinted with permission from
Clinical Symposia, illustrated by Frank H Netter, MD. All rights
reserved. Legends adapted.)
The primary palate forms by fusion of the maxil-
lary processes with the frontonasal process. Olfac-
tory placodes split the frontonasal process into cen-
tral and lateral processes. The central portion of
the frontonasal process is buried by progressive
ingrowth of the maxillary process after it has joined
with both the nasomedial and nasolateral processes,
effectively separating the mouth from the nose.
The primary palate closes between the 4th and 8th
weeks of intrauterine life.
SRPS Volume 9, Number 21
Two theories are typically mentioned when dis-
cussing the embryogenesis of facial clefts. The
classical theory, espoused by Durscy19 and His,20
assumes closure of the facial processes by fusion
of the ectodermal and mesodermal elements in a
manner not unlike the healing of wound edges.
According to the classical theory, the nasomedial
process is the key structure in the development of
clefts of the lip. A cleft lip results from failure of
fusion of the medial nasal prominence with the
maxillary prominence(s).
The mesodermal penetration theory, introduced
by Pohlmann,21 modified by Veau,22 and popular-
ized by Stark,23 postulates mesodermal migratory
routes with closure between the processes depend-
ing on mesodermal reinforcement at key points
along these routes. According to this theory, clefts
result from lack of reinforcement between the two
epithelial layers and consequent epithelial break-
down and separation.
Most researchers agree that the sequence
explained by the classical theory, that is, fusion of
the margins of the processes, is pertinent to clo-
sure of the secondary palate, whereas closure of
the primary palate involves some form of meso-
dermal penetration.
Schendel, Pearl, and De’Armond24 hypothesized
that some of the morphologic deformities associ-
ated with cleft lip may cause a failure of mesenchy-
mal reinforcement of the facial processes at a criti-
cal time in development. The authors obtained 66
muscle biopsies from cleft lip infants at the time of
lip repair and noted a non-neurogenic muscle atro-
phy that varied in severity, with muscle fibers near
the cleft being most atrophic and disorganized. They
concluded that the abnormal muscles in cleft lip
deformities reflected either myopathy in the facial
mesenchymal mitochondrion or at least a delay in
maturation.
Raposio and colleagues25 studied the ultrastruc-
ture of orbicularis oris muscle in unilateral cleft lip
patients and detected structural alterations such as
variation in fiber size, increased number of mito-
chondria, and abnormal glycogen deposits in all
specimens. The authors speculate that these
changes are the result of increased oxidative
metabolism and an undefined genetic inflamma-
tory condition of the muscle.
In a separate study, the same authors26 found
increased concentration of the enzymes lactate
3
SRPS Volume 9, Number 21
dehydrogenase and creatine phosphokinase in the
amniotic fluid of pregnant women whose fetuses
had cleft lip/palate. They conclude that local meta-
bolic impairment can be involved in the pathogen-
esis of cleft lip and palate.
CLASSIFICATION
In order to standardize reporting of cleft lip and
palate cases, the Nomenclature Committee of the
American Association for Cleft Palate Rehabilita-
tion published a comprehensive classification sys-
tem in 1962 that was later adopted by the Cleft
Palate Association.27 The complexity of the sys-
tem, however, mitigated against its widespread
acceptance.
A few years earlier Kernahan and Stark28 had
suggested a classification scheme that was simple
and straightforward, if somewhat lacking in detail.
Then in 1971 Kernahan29,30 introduced a simplified
symbolic classification scheme with logo, the “striped
Y” method, that uses the incisive foramen as its
focal point (Fig 5).
Fig 5. Suggested manner of recording some of the more unusual
clefts using the ‘striped Y’ method. (Reprinted with permission
from Kernahan DA: On cleft lip and palate classification. Plast
Reconstr Surg 51:578, 1973.)
4
This system has become quite popular, particu-
larly after Millard adopted it in his Cleft Craft vol-
umes. Since then the hard palate has been
restricted to position 7, the soft palate is identified
as 8, and submucous clefts as 9; otherwise the
classification remains the same.
Schwartz and colleagues31 suggest a modifica-
tion of Kernahan’s striped-Y system that uses a 3-
digit number for recording the anatomic sites of
clefting. The proposed method
— assigns the number 1 to the first (right) limb of
the Y (Kernahan’s positions 1, 2, and 3);
— the number 2 to the left side of the lip (positions
4, 5, and 6); and
— the number 3 to represent the base of the Y (7,
8, and 9).
This modification facilitates computer data entry
while including all appropriate descriptions of each
anatomic cleft variant.
EPIDEMIOLOGY
Racial Incidence. Burdi32 and Habib33 review
the epidemiology of oral clefts. Cleft lip and palate
(CLP) shows evidence of racial heterogeneity. The
mean incidence in Asians is approximately 2.1 in
1000 live births; in whites it is 1 in 1000; and in
New World blacks it is 0.41 in 1000.34-36 There
appears to be no such racial heterogeneity in iso-
lated cleft palate (CP), which has a constant inci-
dence of 0.5 in 1000 births among the races.
Relative Incidence. The frequency of the vari-
ous types of cleft relative to each other is harder to
determine. In their survey Fraser and Calnan37
found 21% of cases had isolated cleft lip, 46% had
cleft lip and palate, and 33% had isolated cleft pal-
ate. As for site of occurrence, left-sided clefts were
twice as frequent as right-sided clefts and 6X more
frequent than bilateral clefts, for a 6:3:1 ratio.38 This
frequency is exaggerated in boys. Bilateral CL is
associated with CP in 86% of cases.33
In a review of 2471 cleft cases, Ranta and
Rintala39 found that 2.7% had separate clefts of the
lip and palate. On the basis of epidemiologic stud-
ies of the families and of physical findings related to
the clefting, the authors concluded that patients
with separate clefts of the lip and palate belong to
the same etiologic class as cleft lip-palate cases.

Parental Age. The incidence of CL/P probably
does rise with increasing parental age. The father’s
age is apparently more significant than the mother’s,
but the risk certainly is higher when both parents
are over 30 years of age.40
Seasonal Incidence. A higher incidence of oral
clefts in January and February has been reported,
but subsequent studies have not confirmed this
seasonal variation.
Birth Order. The birth order of children with
oral clefts is not significantly different from that of
normal children.
Social Class. Some association has been made
between a high incidence of oral clefts and low
socioeconomic status, presumably related to poorer
nutrition at the lower end of the economic scale.
Head Topography of Parents and Siblings. Par-
ents of children with CL/P generally show under-
developed maxillae with flattened anterior surfaces.
Their faces are more trapezoidal or rectangular
than the average and their upper lips are typically
thin.
Suzuki and others 41 compared the dento-
craniofacial morphology of parents of children with
CL/P with that of parents of children without clefts.
The parents of CL/P children showed a distinct
craniofacial morphology consisting of increased
interorbital and intercoronoid process distance,
wider nasal cavity, and increased length of the
anterior cranial base and total cranial base.
Associated Defects. Central nervous system
malformations, club foot, and cardiac abnormalities
are most commonly associated with CL/P. The
overall incidence of associated anomalies in all cleft
cases is 29%; the highest association is with iso-
lated cleft palate. This phenomenon may be due
to the inclusion of Pierre Robin cases as associated
abnormalities in CP, although the syndrome is now
regarded as part of the total picture of cleft palate
rather than a concomitant anomaly.42
Hagberg and others43 studied the incidence of
different types of clefts, sex, birth weight, mother’s
age, and length of pregnancy in a Swedish popula-
tion of 251 patients with cleft lip/palate born in
Stockholm between 1991 and 1995. They found
SRPS Volume 9, Number 21
additional malformations in approximately every
sixth newborn with a cleft when children with Robin
sequence were excluded. Newborns with bilateral
clefts showed a tendency for additional malforma-
tions. Children with clefts and additional malforma-
tions had lower birth weight and were born earlier
than children with clefts only. The authors recom-
mend that preterm cleft children with low birth
weight be screened for the presence of other birth
defects.
ETIOLOGY
In a review of the etiology of facial clefting,
Jones44 traces the epidemiologic history of cleft lip
and palate to the work of Fogh-Anderson45 in the
1940s, which indicated that CL +/– CP is distinct
from isolated CP but both tend to cluster in fami-
lies. Fogh-Anderson45 found the disorder to be
familial twice as often in CL/P as in CP alone, and
Jones’s44 series confirms these numbers. Overall,
approximately 33%42 to 36%44 of cases of CL/P or
CP have a positive family history, while in the other
67% the etiology is presumably environmental.42
Genetic Factors
Aylsworth46 reviews the genetic considerations
in cleft lip and cleft palate. The author stresses the
value of a history and clinical examination to appro-
priately categorize the cleft deformity as a malfor-
mation, a disruption, or a deformation. A malfor-
mation is a morphologic defect of an organ, part of
an organ, or larger region of the body stemming
from an intrinsic error of morphogenesis. A dis-
ruption is a morphologic defect of an organ, part
of an organ, or a larger region of the body result-
ing from the extrinsic breakdown of, or interfer-
ence with, an originally normal developmental pro-
cess. This concept implies a disruption of an intrin-
sically normal developmental process by something
in the environment such as mechanical forces or
an infectious agent. A deformation is an abnormal
form, shape, or position of a part of the body caused
by mechanical forces.
Cleft deformities are either syndromic or
nonsyndromic. A cleft is syndromic if there is more
than one malformation involving more than one
developmental field. A cleft is nonsyndromic if
there is only one defect or multiple anomalies that
5
SRPS Volume 9, Number 21
are the result of a single initiating event or primary
malformation. Jones’s44 statistics derive from data
on 963 consecutive patients grouped by cleft type
and recognizable pattern (Table 1). The propor-
tion of facial clefts in this database was 59.6% CL/P,
34.1% CP, 4.8% velopharyngeal insufficiency (VPI),
and 1.5% atypical.
TABLE 1
Frequency of Multiple Malformation
Syndromes For Each Cleft Group
(Reprinted with permission from Jones MC: Facial clefting.
Etiology and developmental pathogenesis. Clin Plast Surg
20(4):599, 1993.)
A child was considered to have a syndrome if in
addition to the cleft there were two or more major
malformations or three or more minor malforma-
tions not explained by the family background.
Clefting disorders associated with early death were
excluded. Data analysis showed that syndromes
were clearly more common in isolated CP than in
CL/P: of 79 syndromic CL/P patients, recogniz-
able patterns of malformation were identified in
57% (Table 2).
By far the most common recognized disorder in
the CP alone population is the Stickler syndrome,
which accounts for 17.5% of syndromic clefts in
the Children’s Hospital series.44 This autosomal
dominant condition is caused by a mutation in the
gene for Type 2 collagen. When parents are not
affected the diagnosis is made on the basis of oph-
thalmologic manifestations, including severe myo-
pia, retinal detachment, and glaucoma.44
Pashayan47 offers a set of ten tables to serve as
guidelines in the diagnosis of children with cleft lip
or cleft palate and other structural anomalies. Of
6
TABLE 2
Cleft Lip/Palate: 79 Patients With Multiple
Malformation Syndromes
(Reprinted with permission from Jones MC: Facial clefting.
Etiology and developmental pathogenesis. Clin Plast Surg
20(4):599, 1993.)
note is Van der Woude’s syndrome, an autosomal,
dominantly inherited disorder characterized by
bilateral lower lip pits, cleft lip with or without pal-
ate, or isolated cleft palate, and occasional con-
genital absence of the second molars.48,49 Genetic
penetrance of this syndrome is 70% to 100%48,49
and expressivity is variable. The risk for children of
affected parents is 50%.
In 1996 Gorlin and others50 coined the term
blepharo-cheilo-dontic syndrome for a distinct con-
dition characterized by abnormalities of the eyelids
(euryblepharon, ectropion of the lower eyelids,
distichiasis of the upper eyelids, lagophthalmia),
teeth (oligodontia and conical crown form), and
cleft lip and palate. This entity is believed to have
autosomal dominant inheritance with variable
expressivity.
Several investigators51-53 note a potential major
gene on 6p23-24 and a modifying role for 2p13, an
area harboring the TGFA gene, on the etiology of
nonsyndromic cleft lip with or without cleft palate
(NSCLP). Christensen and Olsen,54 on the other
hand, found no association between the TGFA locus
and either cleft lip/palate or cleft palate. Retinoic
acid receptor alpha (RAR-a) on 17q, MSX1 on 4p,

4q, BCL3 on 19q, and a susceptibility locus on the
X chromosome have all been linked to NSCLP in
certain families, supporting the hypothesis that sev-
eral interacting major genes participate in the etiol-
ogy of NSCLP.55
Evidence is mounting that major genes predis-
pose to nonsyndromic clefting in certain individuals
and families. A recent meta-analysis by Carinci and
others56 summarizes our knowledge as follows:
It is well established that nonsyndromic cleft of
the lip with or without the palate (CL+/–P) and cleft
palate only (CPO) are separate entities. Genetic
heterogeneity has been observed in CL+/–P, which
involves different chromosome regions, mainly
6p23 (OFC1), 2q13 (OFC2), and 19q13.2 (OFC3),
as well as other loci, such as 4q25-4q31.3 and
17q21. Furthermore, an interaction between
different genes has been suggested in the oligo-
genic model. . . . The mode of inheritance of CPO
is compatible with a recessive single major gene
model, while an association with a candidate gene,
mapping on the chromosome region 2q13/TGFA,
remains to be confirmed.
Carinci et al (2000)
In an epidemiologic study of 196 index cases
and their families, Steinwachs and colleagues57
found no association between nonsyndromic cleft
lip and palate and cancer or other birth defects.
Environmental Agents
Numerous studies have implicated chemical
agents in the pathogenesis of CL/P and CP. As
Jones44 points out, most of these studies involved
experimental animals, and it is well recognized that
teratogens are species-specific in their effect, that
is, teratogenicity in animals does not imply terato-
genicity in humans. As noted by Jones, although
CL/P and CP alone may represent one feature of a
number of recognized patterns of malformations
attributable to environmental agents—alcohol,
anticonvulsants, 13-cis-retinoic acid—isolated clefting
without associated structural and functional prob-
lems has yet to be convincingly associated with
prenatal exposure to a single substance.
A study by Hassler and Moran58 on the effects
of ethanol on the cytoskeleton of migrating and
differentiating neural crest cells suggests a possible
role for alcohol in teratogenesis. Their review
chronicles the effects of ethanol on the neural crest
cells, which participate in the formation of cranio-
SRPS Volume 9, Number 21
facial structures that are malformed in the fetal al-
cohol syndrome.
Shaw and Lammer 59 performed a large
(N=48,844) population-based controlled study of
maternal alcohol consumption and clefting. They
concluded that there is no increased risk of cleft
with relatively low quantities of maternal alcohol
consumption. However, there is increased risk of
clefting with higher quantities of alcohol consump-
tion. Women who consumed five or more drinks
per week showed increased risk of having a child
with isolated cleft lip/palate (odds ratio = 3.4, 95%
CI, 1.1:9.7).
Johnston and Millicovsky60 review the normal
and abnormal development of the lip and palate.50
The authors emphasize the role of neural crest-cell
migration in the formation of all facial epithelium
and mesenchyme, and trace human embryonic
development from completion of crest-cell migra-
tions to fusion of the facial prominences. Failure of
fusion of these facial prominences—lateral and
median nasal, maxillary, mandibular, and hyoid—
leads to cleft lip, whereas failure of fusion of the
palatal shelves—medial extensions of maxillary
prominences—leads to cleft palate. Regarding the
secondary palate, Johnston and Millicovsky60 con-
clude from scant human data that the mechanism
by which most cases of human CP arise is some
sort of interference with shelf elevation and pre-
sumably contact. In the primary palate a number of
environmental factors can interfere with fusion of
the facial prominences. For instance, children of
epileptic women on anticonvulsant (Dilantin)
therapy have an incidence of cleft lip that is 10X
higher than normal.
The role of maternal smoking during pregnancy
in the pathogenesis of oral clefts remains contro-
versial.61 In the largest study to date on the asso-
ciation between maternal cigarette smoking and
cleft lip/palate, Chung and coworkers62 collected
maternal demographic and medical data on 2207
newborns with cleft lip/palate from 46 American
states. They found a significant association between
maternal cigarette use during pregnancy and birth
of a child with cleft lip/palate [odds ratio 1.55,
P<0.001]. A dose-response relationship was noted,
with increased cigarette smoking during pregnancy
resulting in increased risk of having a child with
cleft lip/palate—odds ratio 1.78 for consumption of
21 cigarettes or more per day.
7
SRPS Volume 9, Number 21
Some investigators63,64 report a lower-than-ex-
pected incidence of CL/P in future siblings of CL/P
children when the mother’s diet is supplemented
with folic acid and vitamin B6 during pregnancy.
Subsequent studies have both refuted and con-
firmed the benefits of folic acid as a preventive
pharmacologic agent in facial clefting.44
Tolarova and Harris65 evaluated the effect of
periconceptional multivitamin folic acid supplemen-
tation. The study group consisted of 221 pregnan-
cies in women at risk of a child with cleft lip/cleft
palate. Supplementation was begun 2 months
before planned conception and continued for 3
months thereafter. A comparison group compris-
ing 1901 women at risk of a child with CL/P who
received no supplementation and who gave birth
within the same period as the study group served
as controls. The supplemented group had 65.4%
decreased incidence of CL/P births (observed vs
expected). No efficacy of folic acid supplementa-
tion was observed in female probands with bilat-
eral cleft lip/cleft palate. Generally, efficacy was
greater for subgroups with unilateral rather than
bilateral clefts and for male over female probands.
A case-control pair analysis by Czeizel and col-
leagues 66 demonstrated significant protection
against cardiovascular defects, neural tube defects,
cleft lip with or without cleft palate, and posterior
cleft palate with folic acid supplementation during
the critical period of embryogenesis.
Shaw and associates67 found increased risk of
clefting for infants with the A2 TGFA genotype
(homozygous or heterozygous) whose mothers
did not use vitamins containing folic acid compared
with infants of the same genotype whose mothers
used multivitamins, suggesting a gene–nutrient
interaction in the risk of cleft.
Willhite and colleagues68 discuss isotretinoin-
induced cranial malformations in humans and ham-
sters. Oral administration of Accutane during the
first month of human pregnancy can induce severe
congenital malformations. The Accutane dysmor-
phic syndrome includes rudimentary external ears,
absent or imperforate auditory canals, a triangular
microcephalic skull, cleft palate, depressed midface,
and anomalies of the brain, jaw, and heart.
Carmichael and Shaw69 conducted a population-
based case control study that suggested a possible
association between corticosteroid use and cleft lip
with or without cleft palate (odds ratio 4.3, 95% CI)
and isolated cleft palate (odds ratio 5.3, 95% CI).
8
Multifactorial Model. Jones44 states that
In the late 1960s the multifactorial/threshold
model was advanced to explain the mode of
inheritance for a variety of structural defects such as
CL(P) and CP, which clustered in families but
whose inheritance did not conform to mendelian
laws. The model involved the concept of genetic
liability or susceptibility to a given characteristic,
governed by many different genes, and a threshold,
determined by both genetic and environmental
factors. Individuals who lay beyond the threshold
exhibited the phenotype, whereas those who did
not were phenotypically normal. The multifactorial/
threshold model makes several predictions, specifi-
cally
(1) The defect in question will cluster in
families.
(2) The risk for first-degree relatives of affected
individuals (parents, siblings, and offspring) will
approximate the square root of the population risk.
(3) The risk for second-degree relatives (uncles,
aunts, half-siblings) will be sharply lower than the
risk for first-degree relatives.
(4) The more severe the malformation, the
greater the risk for recurrence.
(5) The greater the number of affected family
members, the greater the risk for recurrence.
(6) The risk for recurrence will be increased for
relatives of the less affected sex, if sex differences
are noted.
(7) Consanguinity will increase the risk.
Jones (1993)
When cleft populations were judged against this
model, CLP conformed to predictions while CP
alone rarely conformed.44
A recent study70 evaluated genotype–environ-
ment interactions for nonsyndromic cleft lip and
palate. The authors found significantly elevated
risk for CP in infants of mothers who smoked more
than 10 cigarettes per day and for CLP in infants
whose mothers drank more than 4 alcoholic drinks
per month.
Altitude. Castilla and colleagues71 compared the
birth prevalence of specific types of congenital
anomalies at low and high altitudes in South
America. The study included 1,668,722 consecu-
tive births and showed a significantly (P <0.01)
higher relative risk for cleft lip in the highlands than
in the lowlands (RR=1.57, 95% CI). The study
showed a higher relative risk also for microtia,

preauricular tag, branchial arch anomaly complex,
constriction band, and anal atresia. The authors
hypothesize the teratologic effect of altitude by
itself can be related to a mechanism of chronic
hypobaric hypoxia during embryologic and fetal
development.
GENETIC COUNSELING
Table 3 from Fraser72 summarizes the standard
risks for cleft lip with and without cleft palate.
The risk to relatives also increases with severity
of the cleft. While subsequent siblings of a child
with unilateral CL have a risk of clefting deformity
of about 2.5%, when the affected child has unilat-
eral CL/P the risk to siblings becomes 4.2%, and
with bilateral CL/P, it is 5.7%.40
PRENATAL DIAGNOSIS
A scanning ultrasound is usually performed in
the late first trimester or early second trimester of
pregnancy. Cleft lip/palate can be first diagnosed
at that time. The fetal lip and nose can be visual-
ized at 15 weeks of gestation with a 3.5-MHz scan-
ner.73 A 6.5-MHz transvaginal scanner can show
TABLE 4
Risks of Cleft Lip and Cleft Lip/Palate Given Several Family Situations
(Reprinted with permission from Fraser FC: Etiology of Cleft Lip and Palate. In: Grabb WC, Rosenstein SW, Bzoch KR (eds), Cleft
Lip and Palate—Surgical, Dental, and Speech Aspects. Boston, Little Brown, 1971.)
SRPS Volume 9, Number 21
the face and palate of the fetus at 12 weeks of
gestation.74
In 2086 fetuses karyotyped after an abnormal
ultrasound, Nicolaides75 found 146 (7%) had facial
abnormalities. Among these, 64 (44%) had cleft lip
and palate. Of these, eight fetuses (13%) had iso-
lated cleft lip/palate, 39% had cleft lip/palate associ-
ated with other anomalies but with normal chromo-
somes, and 48% had chromosomal abnormalities.
At birth, all fetuses with ultrasonographic diagnosis
of isolated clefts and with normal chromosomes
were confirmed to have cleft lip/palate without
associated anomalies. On the basis of these find-
ings the authors recommend karyotype studies when
the prenatal diagnosis of cleft lip/palate is made.
The sensitivity of the screening ultrasound in the
diagnosis of cleft is unknown and appears to relate
to whether the face is specifically studied and
whether there is isolated cleft palate. Hegge76
showed that only 10% of clefts were diagnosed
prenatally by scanning ultrasound. In another study,
Hegge77estimated that one cleft lip/palate is identi-
fied out of every five occurrences. Two of the four
missed diagnoses are isolated cleft palates. At no
time was isolated cleft lip identified, and no false
positives were reported.
9
SRPS Volume 9, Number 21
The capability to diagnose cleft lip/palate in utero
raises a number of controversial issues. In 1996
Bronshtein et al78 detected 15 cases of cleft lip by
transvaginal ultrasonography in a population from
northern Israel. Three years later Blumenfeld and
coworkers79 increased these numbers to 24 cases of
cleft lip, and the diagnosis was confirmed in 22. Of
note, 23 of the 24 women chose to terminate the
pregnancy. The only couple who did not terminate
the pregnancy subsequently stated that they would
choose abortion in the future if again they had a fetus
who was diagnosed prenatally with a cleft lip.
In contrast, Matthews and others80 note that the
New Jersey families interviewed felt that diagnosis
of cleft before birth was desirable in order to be
emotionally prepared and educated. None of the
nine couples would consider abortion for isolated
cleft. Jones’s81 cleft team reports that of eight
women from Southern California who received a
prenatal diagnosis of isolated cleft lip/palate and
wrestled with a termination decision, six decided to
continue the pregnancy and two did not.
Clearly the results of these studies vary among
countries, reflecting socioeconomic, religious, politi-
cal, and cultural differences in the populations. Nev-
ertheless, they all underscore the importance of pre-
natal counseling and support by the whole cleft team.
ANATOMY
The following descriptions of anatomical ele-
ments in unilateral cleft lip have been adapted from
Millard.17
Cleft (Lateral) Side:
• The premaxilla is outwardly rotated and project-
ing, and the lateral maxillary element is
retropositioned.
• The inferior edge of the septum is dislocated
out of the vomer groove and presents with the
nasal spine in the floor of the normal nostril.
• There is unilateral shortness in the vertical height
of the columella, varying from three-fourths to
even one-half that of the normal side.
• The lower lateral cartilage is attenuated, its medial
crus lower in the columella and its dome sepa-
rated from the opposite alar cartilage to rest below
it. The lateral segment is flattened and spread
across the cleft at an obtuse angle. The alar crease
10
has no alar cartilage bulge to give way to and
consequently continues obliquely across the tip
just lateral to the joint of the columella and through
the rim of the ala. This is often responsible for an
actual kink in the alar margin itself.
• The alar base is invariably rotated outwardly in a
flare.
• The alar rim is invariably distorted by a skin cur-
tain (without cartilage) that droops over the alar
rim like a web and further reduces the apparent
height of the columella.
• The vestibular lining is deficient on the cleft side.
• The orbicularis oris muscle in the lateral lip ele-
ment ends upward at the margin of the cleft to
insert into the alar wing. In cases of incomplete
clefting the muscle does not as a rule cross the
cleft unless the bridge is at least one-third the
height of the lip.82
• The philtrum is short.
Non-Cleft (Medial) Side:
• There is shortened philtral height and
• A shortened columella.
• Two-thirds of the cupid’s bow, one philtral col-
umn, and a dimple hollow are preserved.83
• The musculature between the philtral midline
and the cleft is hypoplastic.
Park and colleagues84 showed that the lateral crus
of the lower lateral cartilage on the cleft side is not
hypoplastic. During primary rhinoplasty of 55 unilat-
eral cleft lip nose patients, the authors measured the
lateral crus of the lower lateral cartilage on both sides.
On the cleft side, the midportion of the lateral crus
appeared to be thicker and wider in comparison with
the noncleft side. No histologic differences were
found between the lateral crura of the normal and the
cleft side. They concluded that the cleft lip nasal
deformity is caused by external factors rather than
intrinsic factors. Interestingly, using a proliferating cell
and nuclear antigen technique, they proved that the
lateral crus growth had ceased after 17 years of age.
Fisher and associates85 studied the geometry of
primary cleft lip nasal deformity before surgical cor-
rection. The study involved 3-dimensional comput-
erized tomography in a group of 3-month-old babies
with complete unilateral cleft lip and palate. Six

patients had clefts narrower than 10 mm and 6
patients had clefts wider than 10 mm. The S-N-ANS
angle was greater in the group with the wider cleft,
evidence of a relationship between severity of the
cleft and premaxillary protrusion. Four consistent
findings were observed in all patients in order of
decreasing magnitude (Fig 6):
• the columella base was deviated to the noncleft
side
• the cleft side alar base was more posterior than
the noncleft side alar base
• the noncleft side alar base was farther from the
midline than the cleft side alar base
• the cleft side piriform margin was more poste-
rior than the noncleft side piriform margin
Fig 6. Above, soft-tissue landmarks of the cleft nasal deformity
based on CT analysis. Below, with the head tilted 30 degrees,
a 3-D effect is obtained. (Reprined with permission from Fisher
DM, Lo LJ, Chen YR, Noordhoff MS: Three-dimensional com-
puted tomographic analysis of the primary nasal deformity in 3-
month-old infants with complete unilateral cleft lip and palate.
Plast Reconstr Surg 103(7):1826, 1999.)
This study underscores the asymmetry of both
bony and soft-tissue landmarks at the nasal base
and stresses the bilaterality of the unilateral cleft lip
nasal deformity.
The pathologic anatomy of the bilateral cleft is
essentially the same as that of the unilateral cleft.
Typically there is more shortening of the columella,
SRPS Volume 9, Number 21
and the prolabium is unique in that there is absence
of philtral remnants and muscle elements.
Details of the muscular anatomy of unilateral
and bilateral cleft lip are found in the article by
Fara.82 A pictorial summary of these is found in Fig
7 from Millard.17,86
Fig 7. The soft tissue deformity in (A) unilateral and (B) bilateral
cleft lip. (Reprinted with permission from Millard DR Jr: Cleft
Craft—The Evolution of Its Surgery. I. The Unilateral Deformity.
Boston, Little Brown, 1976; and Cleft Craft—The Evolution of Its
Surgery. II. Bilateral and Rare Deformities. Boston, Little Brown,
1977.)
Dado and Kernahan87 review the anatomic and
histologic findings in clinical cleft lip specimens and
describe differences between theirs and Fara’s82
observations. Specifically, the authors note that
the muscle bulge in complete and incomplete cleft
lips consists of a haphazard arrangement of muscle
fibers running transversely, obliquely, and anteri-
orly to posteriorly. In contrast to Fara’s82 report,
Dado and Kernahan87 found no distinct muscle
11
SRPS Volume 9, Number 21

bundles paralleling the cleft margin and inserting

into the alar base and columella.
The modern trend toward radical muscle mobili-
zation in the surgical repair of unilateral cleft lip88-91
is based on evidence of the importance of the
muscular anatomy in this deformity. Nicolau89 dis-
tinguishes two well-defined parts of the orbicularis
oris muscle, as follows:
The deep component functions in catching food
with a general sphincteric activity, and works in
association with the other muscular loops of the
oropharynx . . . The deep orbicularis oris originates
from the modiolus on each side. It is horizontal
with continuous fibers passing from one commis-
sure to the other across the midline, and lies close
to the inner mucosal surface. Its lower border curls
upon itself, forming the vermilion by everting the
mucous membrane. At the commissure, the curled
border of the upper lip muscle divides, and the
curled border of the lower lip muscle inserts
between these slips. When the fibers shorten to
close the lips, the margins flatten out and the
interdigitation provides a scissor-like motion which
seals the angles of the mouth.
Nicolau (1983)
The superficial orbicularis oris muscle functions
in facial expression and provides the very precise
movements of the lips needed in speech (Fig 8).
The orbicularis oris originates
. . . from the muscles of facial expression and
consists of an upper and lower bundle. The lower
or nasolabial bundle derives its fibers from the
depressor anguli oris muscle on each side. They Fig 8. Anatomy of the lip musculature.
insert in the skin forming the philtral ridges...The
upper or nasal bundle represents the common
insertion of the fibers of the zygomaticus major and abnormal insertion on the cleft side and a partially
minor, levator labii superioris, levator labii superioris distorted insertion on the non-cleft side . . . On the
alaeque nasi, and transversus nasi. [These bundles] cleft side, the lateral part of the nasolabial bundle is
insert into the anterior nasal spine, the septo- normal and passes from below upwards from the
premaxillary ligament, and the nostril sill, passing modiolus towards the midline. However, it
deep to the alar base. changes in direction at a point below the displaced
Nicolau (1983) alar base, running almost vertically, becoming
Nicolau’s work on clefts suggests that the deep attached to the nostril and the periosteum of the
sphincteric part of the muscle does not reach the piriform aperture. Contraction therefore results in a
marked lateral bulge. Stimulation of the nasal
extremity of the interrupted vermilion. The muscle
bundle on the cleft side could be obtained only
fibers are not distorted by the cleft, but simply inter-
after pushing the needle electrode almost down to
rupted. They end on either side of the cleft at the the bone on the lateral aspect of the ala nasi and in
point where, in classical descriptions, the skin/ver- the nasolabial fold. Its pull laterally and upwards
milion ridge becomes thin. appears to be the main cause of the typical nostril
The superficial muscle, which normally inserts on deformities . . . On the non-cleft side, stimulation of
either side of the midline, is misdirected by the the most medial part of the nasolabial bundle
cleft, and its two components find a new and reveals the formation of an almost normal Cupid’s

12

bow. It appears that the cleft is disrupting the
normal bilateral insertion and that the fibers
crossing through the preserved philtral ridge insert
into the cleft edge almost perpendicular to it. . . .
The nasal bundle appears normal and is not
affected by the cleft, as its most medial insertion
point is the nasal spine. Nevertheless, its unop-
posed lateral and upward pull might contribute to
the anterior septal deformity.
Fara (1968)
Development of the orbicularis oris muscle in
the normal and cleft lip/palate human fetus is usu-
ally three-dimensional. Computer reconstruction
was undertaken by Mooney et al,92 who investi-
gated 29 fetuses ranging in age from 18 to 21
postgestational weeks. The orbicularis oris muscle
in the normal fetal sample with discernible lip fibers
increased symmetrically in both fiber density and
complexity from 12 to 21 weeks. In contrast, the
unilateral CL/P fetal specimens with discernible lip
fibers exhibited a 3.5-week delay in overall muscle
development, asymmetrical fiber distribution, and
abnormal fiber insertions. Qualitatively, however,
there were no significant differences noted in
orbicularis oris muscle thickness or volume between
the normal and CL/P specimens through 21 weeks.
These findings suggest that the orbicularis muscle
deficiency noted clinically in CL/P neonates may
be a result of perinatal function dysmorphogenesis
rather than congenital mesenchymal reduction or
deficiency. Millard17 lists the normal dimensions of
the lip and nose according to age, sex, and race in
Cleft Craft I.
De Mey93 and associates studied the anatomy
of the orbicularis oris muscle in cleft lip. In incom-
plete clefts the intrinsic part of the orbicularis, located
in the vermilion, is simply interrupted without dis-
tortion. The extrinsic part, lying higher in the lip,
crosses the cleft but is distorted vertically accord-
ing to the degree of nasal deformity. In complete
clefts the intrinsic bundle ends in the submucosa of
the vermilion, as in incomplete clefts. The extrinsic
bundle deviates toward the ala nasi on the lateral
side; on the medial side the fibers are rarer and
more horizontal.
Mulliken94 details the gross and microscopic
anatomy of the skin-mucosal junction (“white roll”
of Gillies) at the Cupid’s bow of infants with normal
and cleft lips. In the normal upper lip, the anterior
projection of the pars marginalis of the orbicularis
oris muscle gives rise to the cutaneous-vermilion
SRPS Volume 9, Number 21
junction. The normal vermilion is widest at the
peaks of Cupid’s bow. On sagittal section begin-
ning anteriorly at the white roll and proceeding
orally, the vermilion mucosa exhibits progressively
increasing epidermal thickness and size of rete
ridges, decreasing melanin, more superficial capil-
laries, and an abrupt transition from keratinized to
nonkeratinized squamous epithelium (“red line” of
Noordhoff).
In cleft lip specimens the white roll is absent,
there is hypoplasia and disorientation of the under-
lying pars marginalis component of the orbicularis
oris muscle, decreased vermilion width on the
medial side of the cleft, and normal to slightly
increased width of vermilion laterally (the entire
prolabial vermilion component of bilateral cleft lip
specimens is hypoplastic.) On the basis of these
observations the authors94 endorse Noordhoff’s95
recommendation of a lateral vermilion flap to aug-
ment the deficient medial vermilion in cleft lip repair.
Slaughter and coworkers96 detail the blood sup-
ply of unilateral and bilateral cleft lip. Clefting inter-
rupts the normal anastomoses occurring between
the superior labial artery, anterior ethmoidal artery,
posterior septal artery, and greater palatine artery
to various degrees. In complete bilateral cleft lip
the dominant blood supply to the prolabium is via
the posterior septal artery, without other collaterals.
Despite this alteration in blood supply, ischemia
from surgical repair is seldom seen. The prolabium
may be quartered and lifted to the nasal spine with-
out significant compromise to its circulation.
FEEDING
Feeding is an important part of care of the cleft
lip/palate patient both preoperatively and postop-
eratively. Lazarus97 studied 640 children with cleft
lip/palate and determined that 30.5% were under-
weight for age. By comparison, only 13.7% of a
similar group of noncleft controls were underweight
for age. The difference between the two groups
was highly significant (P <0.01). Children with cleft
palate with or without cleft lip were found to be
more underweight for age than those with isolated
unilateral cleft lip (P <0.008). Children who under-
went sugery after 1 year of age were 1.5X more
likely to be underweight than children who had
surgery before age 1 year (P <0.01). There was
also a tendency toward a higher fistula rate in chil-
13
SRPS Volume 9, Number 21
dren with isolated cleft palate who were under-
weight for age in comparison with children of nor-
mal weight.
Shaw98 compared the growth of cleft lip/palate
babies fed with a squeezable bottle with that of CL/
P babies fed with a rigid bottle. The two groups
were different in weight and head circumference
at 12 months, indicating increased growth in the
squeezable-bottle babies. Squeezable bottles are
recommended because they are easier to use and
have a beneficial impact on the babies’ growth.
Traditional measures for protecting surgically
repaired clefts have included the use of cups and
catheters for feeding, avoiding nippling and any
solid substances. A Logan’s bow has also been
used to reduce lip tension and protect the repair.
These old-fashioned attitudes have been challenged
by Weatherley-White and colleagues,99 who com-
pared 49 infants operated on during the first 3
weeks of life with another group of 51 children
whose clefts were repaired at an older age. There
was no significant difference in complications
between the early and later operative groups. A
third subgroup of 26 infants who were operated
on at 1 week of age or earlier had significantly
fewer complications than the other two groups.
There were no apparent differences in operative
results as defined by whether or not the child
needed subsequent revision. On the basis of these
findings the authors conclude that early operation
had a lower complication rate and equally favor-
able operative results.
With the cooperation of 60 mothers, the effect
of breast feeding immediately following operation
was investigated.99 The babies were sorted into
three groups: 16 were breast fed for a minimum
of 6 weeks, 22 were fed by means of a cup or
syringe, and 22 started breast feeding but converted
to a bottle within 6 weeks. There were no compli-
cations attributable to breast feeding. These babies
had the highest weight gain among the groups,
and hospital stay was shortened by 1+ day com-
pared with those fed by cup. The authors advo-
cate “early repair and breast feeding in the full-term
baby as the optimum method of management of
newborns with cleft lip.”99
Additional support for breast feeding is offered
by Darzi and others100 in a prospective, random-
ized trial of 40 infants, 20 of whom were breast fed
and the others fed by spoon. The breast-fed infants
14
showed more weight gain after surgery than the
spoon fed infants. No difference in wound healing
was noted between the two groups.
Skinner and others101 compared the impact of
nipple feeding versus cup feeding on the postop-
erative healing of cleft lip infants. Group I had no
nipple feeding for 6 weeks; Group 2 had no nipple
feeding for 3 weeks; and Group 3 had no restric-
tions on nipple feeding. The authors found no
difference in healing between the groups and con-
cluded that the duration of the alternate feeding
program does not make any difference.
SURGICAL MANAGEMENT
Koch102 reviews the surgical management of cleft
lip and discusses alternatives in primary lip repair,
nasal tip adjustment, secondary lip repair, manage-
ment of alveolar clefts, hard and soft palate clefts,
and secondary procedures for velopharyngeal
incompetence.
Stal and associates103 describe the approach to
cleft lip and palate care at the Texas Children’s
Hospital in algorithmic form. Their review encom-
passes unilateral and bilateral cleft lip/palate, the
cleft nose deformity, secondary deformities of the
lip, velopharyngeal incompetence, alveolar bone
grafting, and orthognathic surgery.
Cohen and coworkers104 looked at the cumula-
tive operative procedures in patients aged 14 years
and older who were born with CLP. Lip and palate
repair was carried out in all patients. The average
number of operations per patient was 6.12 for uni-
lateral CLP and 8.04 for bilateral CLP. Lip adhe-
sions were done in 29% and 62%, pharyngoplasties
in 39% and 38%, alveolar bone grafts in 82% and
79%, Abbe flaps in 0 and 10%, and orthognathic
surgery in 10.5% and 13.8%, respectively.
Mackay and others105 reviewed retrospectively
the medical charts of 374 cleft lip/palate patients
treated at the Lancaster Cleft Palate Clinic between
1965 and 1977, with particular attention to the num-
ber of operative procedures needed to obtain the
best possible result. This information was to be
disseminated to insurance carriers and politicians
in hopes of improving claim reimbursement. An
average of 3.3 reconstructive procedures and 1.2
otolaryngologic procedures were done per patient.
The 51 patients with incomplete unilateral cleft lip
had a mean 1.5 reconstructive procedures, 29%

secondary lip revisions, and 10% rhinoplasties. The
19 patients with complete unilateral cleft lip had 2.5
mean reconstructive procedures, 37% secondary
lip revisions, and 47% rhinoplasties. Of the 110
patients with complete unilateral cleft lip and pal-
ate, each had a mean 3.9 reconstructive proce-
dures, 36% secondary lip repairs, and 45% rhino-
plasties. Two-stage palate repair was done in 72%,
alveolar bone grafts in 37%, pharyngeal flaps in
42%, 16% had fistula closure, and 8% had
orthognathic surgery. The 51 children with bilat-
eral cleft lip and palate had a mean 6.8 reconstruc-
tive procedures, 84% had secondary lip revision,
73% had rhinoplasty, 84% had two-stage palate
repair, 61% had alveolar bone graft, 57% had pha-
ryngeal flap, 18% had fistula, and 14% had
orthognathic surgery.
An overview of the implications of intrauterine
repair of cleft lip is presented by Dado, Kernahan,
and Gianopoulos.106 Interest in intrauterine repair
of cleft lip has been stimulated by the experimental
findings in fetal surgery where wounds created in
the ectoderm of the fetus in utero heal clinically
and histologically without scar formation. This phe-
nomenon is not clearly understood but is believed
to be related to the absence of cellular inflamma-
tory response by the fetus, no collagen deposition,
no wound contraction, and healing by what appears
to be regeneration.
Hedrick and associates107 studied delayed in-
utero repair of surgically created cleft lip and palate
in the fetal lamb model. Incisional or excisional
unilateral cleft lips were created early in gestation
and later repaired. The incisional and excisional
clefts were made completely through the lip and
alveolus. Two weeks later, the wound edges were
freshened and repaired in all but one lamb in each
group. Early in the third trimester the fetuses were
harvested and studied. The incisional clefts healed
spontaneously with replacement of the native
reticular collagen pattern and regeneration of the
skin appendages. In contrast, the excisional clefts
did not autorepair, and when repaired surgically
healed without a collagen scar and no skin append-
ages.
While the implications of these findings applied
to fetal cleft lip repair are obviously attractive, the
risk of fetal loss is high. Preterm labor is a major
complication and one that is directly related to the
large hysterotomy required for fetal exposure.108
SRPS Volume 9, Number 21
As of this writing, intrauterine fetal surgery is still
largely reserved for severe malformations that can-
not be helped significantly by postnatal interven-
tion.
Endoscopic surgical manipulation through small
uterine ports promises eventual prenatal correc-
tion of non-life-threatening malformations in the
human fetus. Oberg and colleagues109 compared
clipped and sutured intrauterine endoscopic repairs
of surgically induced bilateral cleft lips in fetal lambs.
The repair with clips was completed faster and
incited less inflammation than the sutured repair.
Levine and others110 investigated the possibility
of intrauterine manipulation of the shape and size
of the nose by placing hypertonic sponges inside
the right nostril of fetal lambs during the second
trimester. The expanded nose had increased sep-
tal length, a doubled nostril area, and a more than
doubled intranasal volume.
Stelnicki and coworkers111 analyzed the long-
term functional and esthetic outcome of in-utero
versus neonatal cleft lip repair in lambs. There was
no evidence of maxillary growth impairment in the
lambs repaired in utero. In addition, the clefts
repaired in utero were scarless. Both the in-utero
and neonatally repaired lambs had lips that were
considerably shorter vertically on the repaired side
than on the normal side. This was thought to be a
function of the straight-line closure, and points to
the need for similar comparisons in this ovine model
using a Millard-type rotation advancement tech-
nique.
Druschel and colleagues112 used the New York
State Congenital Malformations Registry to study
mortality during the first year of life in infants with
oral clefts. The adjusted risk for children without
additional malformations who had cleft palate was
1.2; for cleft lip, it was 1.1. However, 35% of chil-
dren with oral clefts had associated malformations
and experienced a much higher mortality.
Stephens113 looked at anesthetic complications
of neonatal cleft lip repair. The retrospective analy-
sis included 50 cases of neonatal cleft lip, including
11 premature infants. There were no deaths, one
perioperative hypoxemia, one postoperative laryn-
gospasm requiring reintubation, and one transient
apnea. The authors conclude that neonatal cleft lip
repair is safe provided that
• the babies are gestationally mature with no in-
tercurrent illness
15
SRPS Volume 9, Number 21
• no opioid analgesia is given during the surgical
procedure
• experienced nurses care for the infants postop-
eratively
• the infants are monitored with oximetry and
apnea detectors.
Millard114 describes primary surgical and dental
techniques for the staged treatment of cleft lip/
palate. These measures are based on biologic prin-
ciples and are designed to facilitate continued
migration of the tissues toward a normal end-point.
Precise placement of alveolar segments, dissection
of mucoperiosteum out of the cleft, and union of
mucoperiosteum across the alveolar and anterior
hard palate cleft create a periosteal tunnel across
the bony gap and set up a condition conducive to
bone formation and eventual tooth eruption in the
cleft area. Lip closure by adhesion reduces tension
on the primary lip closure and allows gentle mold-
ing until the arch solidifies, converting a complete
cleft into an incomplete one.115
Once there is a balanced and stable maxillary
platform, definitive lip and nose correction can be
carried out in the unilateral cleft lip at 8 months of
age. In the bilateral cleft lip, definitive cleft lip repair
is done at 18 months, together with banking of
forked flaps, and at 3-4 years of age the nasal cor-
rection is performed. These planned measures
bypass a persistent cleft, fistulas, raw areas, and
malposition of alveolar segments, and decrease
the necessity for later bone grafting.
Santiago and colleagues116 reviewed a series of
20 unilateral and bilateral alveolar clefts treated with
presurgical orthopedics and gingivoperiosteoplasty.
They report that 60% of patients did not need
alveolar bone graft. Lukash and others,117 in their
cephalometric analysis of patients treated with max-
illary orthopedics and early periosteoplasty, con-
firmed no midfacial growth retardation in short fol-
low-up (at age 6 to 10).
MICROFORM CLEFTS
The microform (forme fruste) cleft lip deformity
has three components, all of which need not be
present for the diagnosis to be made. These ele-
ments are as follows: (1) a small notch within the
borders of the vermilion; (2) a band of fibrous
tissue running from the edge of the red lip to the
nostril floor; and (3) a deformity of the ala on the
16
side of the notch or band. The objectives of surgi-
cal repair are to eliminate any notch of the vermil-
ion, correct the drooping or flattened ala, and
restore muscle continuity with a minimal amount
of scarring.
Thomson and Delpero118 analyze their experi-
ence with surgical correction of microform clefts in
24 patients. Repair was by rotation-advancement,
low triangular flap, and modified straight-line clo-
sure. No distinct advantage of one method over
another was noted, and the results in all but four
patients justified surgery.
Onizuka and colleagues119 review the preferred
operative methods for repair of microform clefts.
The authors define cleft severity according to the
degree of downward depression of the nostril rim,
skin striae of the upper lip, notching of Cupid’s
bow, and deformity of the vermilion border. They
suggest that first-degree microform clefts should
be repaired by rhinoplasty, whereas second-degree
deformities should be repaired by rhinoplasty and
either a Z-plasty or other small operation on the lip
without whole lip skin incision. Third-degree defor-
mities are viewed as incomplete clefts and should
be managed by full-thickness lip repair.
In a short discussion of this paper, Millard120 notes
that “an attempt at creating a philtral column by
undermining skin and gathering it in a fold with
sutures tied over stents usually does not maintain
great improvement after removal of the sutures.”
He also believes that “V-Y procedures along the
alar rim may give an unnatural effect [postopera-
tively] and probably do not correct the fundamen-
tal problem.”
UNILATERAL CLEFT LIP
Clifford and Pool121 give an excellent account
of the anatomy of unilateral cleft lip and of the Z-
plasty principle in its surgical correction. Besides
establishing the normal vertical height of lips
according to age, the authors categorize the vari-
ous surgical repairs in terms of the Z-plasty method
(Fig 9).
Straight-line Repair
Modifications of the straight-line repair introduced
by Thompson3 continue to enjoy limited use in
present-day plastic surgery for the correction of

Fig 9. The Z-plasty principle in unilateral cleft lip repair.
the minimal cleft lip deformity. The technique has
widest application in correcting notch deformities
at the vermilion.
Nakajima and associates122 recently showed nice
results in the repair of incomplete unilateral cleft lip
using a modification of the straight-line technique.
Triangular-flap Repair
The Tennison triangular flap repair, with
Randall’s123 suggested revisions, is still popular today.
In their long and distinguished experience, Brauer
and Cronin124-126 obtained excellent results with the
Tennison lip repair, stressing the value of geomet-
ric planning as proposed by Randall,123 but noting
that the repaired side should be designed 1 mm
shorter than the noncleft side to avoid excessive
vertical height of the lip.
Saunders and colleagues127 reviewed the vari-
ous viewpoints on lip growth after repair by the
triangular flap technique. They evaluated the
surgical results in 50 children who had triangular
flap repair of unilateral cleft lip, with follow-up
ranging from 5 to 14 years. During the first 5
years, lips were designed to allow for future
SRPS Volume 9, Number 21
growth and the expected growth did not occur.
During the second 5 years, lip length was de-
signed equal to the normal side in the belief that
unequal growth does not occur. Deviations from
the correct lip length were equally divided be-
tween too long and too short. No patient had a
lip that started too short and became equal or
that started equal and became too long. The
authors conclude that a repaired unilateral cleft
lip retains the configuration and length given at
the initial repair.
The above findings stand in sharp contrast to the
recommendations of Brauer and Cronin,126 who
suggest that the repair side be made 1 mm shorter
than the noncleft side to accommodate future
growth.
Another modification of the Tennison repair calls
for an offset in the incision immediately above the
vermilion line to avoid an oblique scar crossing the
vermilion. A third modification calls for an aware-
ness of the too-long lateral lip segment, which
requires vertical shortening of the lateral lip ele-
ment. These points are reaffirmed by Brauer,126
who documents excellent results with the triangu-
lar flap repair.
17
SRPS Volume 9, Number 21
Bardach and colleagues128 propose a modifica-
tion of the triangular flap technique of cleft lip
repair that results in less lip pressure than repairs
involving wide undermining of the soft tissues.
They believe a minimum of pressure is desirable
based on animal studies indicating that the pres-
ence of increased lip pressure results in greater
degrees of facial growth disturbance and
dysmorphia.
Goulian and associates129 offer further refine-
ments in the design of a triangular flap repair.
They compared the original markings proposed
by Randall123 with their own recommended mark-
ings, giving step-by-step suggestions for deter-
mining the location of points 8, 9, and 10 which
are the crucial points of the triangular flap. The
authors support the construction of a lip that is
neither too long nor too short in vertical dimen-
sion.
Thomson130 offers a long-term appraisal of one
surgeon’s experience with repair of the complete
unilateral cleft lip (although some patients with
Simonart’s bands* were included if the alveolus
was completely cleft). The clefts were classified
into one of four groups depending on the severity
of the nasal deformity, width of the soft-tissue de-
fect, and acuteness of the columella-frenulum angle.
The study encompassed 85 patients whose clefts
were repaired by the low triangular flap technique.
Anthropometric measurements were taken and the
results expressed as a ratio of the cleft side to the
normal side. The outcome of the repair was also
judged subjectively by a panel of observers, who
individually rated 10 components of the operated
cleft lip and nose (modified Williams technique).
As expected, patients with mild clefts received the
highest ratings; those with severe clefts, the low-
est. The author concludes that “it is worthwhile
evaluating surgical results relative to the severity of
the preoperative deformity.” In addition, “surgical
correction of the cleft nose can be one of the most
challenging and frustrating aspects of cleft lip sur-
gery.”130
Indications for cleft lip repair by the triangular
flap method are impossible to define. Some believe
it is never appropriate because the resultant scar
crosses the projected line for the philtrum.17 Oth-
ers reserve the technique for use primarily in
18
extremely wide clefts, while still others use it rou-
tinely.
Rotation-Advancement Repair
The rotation-advancement repair consists of an
upper triangular flap inset into the rotation defect
of the medial segment. The technique allows the
scar to follow the projected line of the philtral col-
umn except in its uppermost portion, where it arcs
beneath the columella. Millard131 furnishes techni-
cal details of the procedure.
Later refinements of the rotation-advancement
principle can be found in Millard’s17 Cleft Craft I,
and consist of broader use of mucosal parings in
correcting the associated nasal deformity. This
repair, with its more recent modifications, is one of
the most popular among American cleft lip sur-
geons today.
Criticisms of the rotation-advancement method
relate to its technical difficulty in wide clefts, the
necessity for wide soft-tissue undermining, tension
across the nostril sill, and a tendency toward a con-
stricted nostril on the side of repair.
Millard 132 emphasizes the importance of
presurgical orthopedics, lip adhesion and nasal cor-
rection as part of the rotation-advancement proce-
dure, the use of an L-flap to increase vestibular
lining, and proper use of a C-flap to increase col-
umellar length on the cleft side. Alar base advance-
ment, alar cartilage lift, and medial and lateral alar
cartilage freeing are discussed in terms of timing
and anticipated result.
Trier133 offers a comprehensive overview of the
rotation-advancement repair technique. The author
reviews the pertinent surgical anatomy, evaluation
and presurgical treatment, treatment plan and
options such as lip adhesion, technical details of
the repair, including considerations for the nose
and muscle repair with vermilion reconstruction,
and postoperative care and follow-up.
Honigmann134 reviews his experience with the
rotation-advancement procedure. He varies the
cut on the medial segment to raise a tongue-shaped
flap at the base of the philtrum. This flap is rotated
downward with the medial lip stump, filling the defect
beneath the columellar base normally filled by the
tip of the advancement flap in Millard’s repair. Pre-
sumably the scar then lies parallel with the philtral
column, avoiding the oblique rotation component

in the upper portion of the philtrum inherent in the
Millard rotation-advancement technique. Honig-
mann’s modification, however, is only applicable
to minimal clefts.
Outcome Studies
In 1971 Musgrave surveyed the time-honored
135
methods of lip repair and compared their advan-
tages and disadvantages in concise table form.
Many of the repairs have since fallen out of favor
and others have been modified to ameliorate their
troublesome features. A case in point involves the
rotation-advancement repair, which was criticized
for producing a small nostril when simultaneous
with nasal correction.
Repairs involving minimal undermining and
detachment of the alar base leave fibrous bands
between the alar cartilage and the maxilla and aber-
rant muscle fibers from the orbicularis and the leva-
tor labii superioris alaeque nasi. These bands and
muscles inevitably keep the alar cartilage tethered
laterally and contribute to subsequent drift of the
base of the ala laterally and superiorly. Overcor-
recting the position of the alar base does not pro-
duce a constricted nostril in these cases.
When these bands and muscles are released
during rotation-advancement repairs, overcorrec-
tion should be avoided, as there is no tendency for
lateral drift of the ala.
Holtmann and Wray136 compared the triangular
flap and rotation-advancement techniques for the
repair of unilateral cleft lip. Unfortunately, the ran-
dom selection yielded dissimilar study groups, with
a greater proportion of complete clefts for the tri-
angular flap patients than for the rotation-advance-
ment group. Accordingly, the observations regard-
ing postoperative nasal deformity and need for
revisions in triangular flap repairs may simply reflect
the fact that the preoperative nasal deformity was
more severe in these cases.
An apparent meaningful difference between the
groups may have been due to the presence of
hypertrophic scars in the rotation-advancement
repair group. No significant difference in vertical
lip length was noted, although both groups con-
tained lips that were either too short or too long
postoperatively. The overall score of results favored
slightly the rotation-advancement repair, but no sta-
tistically significant difference was observed
SRPS Volume 9, Number 21
between the two groups. The authors did not
investigate what effect, if any, the type of repair
used had on facial growth.
The Vermilion
Noordhoff95 discusses the anatomy of the ver-
milion and the proper orientation of the white skin
roll and mucosa-vermilion border (red line). The
vermilion is widest at the base of the philtral col-
umn and there is an ever-present deficiency of ver-
milion on the cleft side. The author used a lateral
vermilion flap to augment the deficient vermilion
beneath the white skin roll on the cleft side of the
Cupid’s bow, with very attractive results.
Aligning the Orbicularis Oris
Kernahan and Bauer91 emphasize the need for a
functional orbicularis reconstruction and realign-
ment of similar parts. Skin closure typically involves
a Z-plasty at the nostril floor and a second Z-plasty
approximately 1 mm above the mucocutaneous
junction. The purpose of the cutaneous Z-plasties
is to gain the needed length and symmetry.
Muller137 advocates differential reconstruction of
the orbicularis oris muscle in unilateral cleft lip,
believing that the most important step in correc-
tion is rearrangement of the muscle components
to reset their insertion and reorient the muscle
fibers. Each of the three components of the
orbicularis oris muscle should be repaired inde-
pendently to allow them to function separately.
The repair consists of inserting the nasal bundle
into the anterior nasal spine, correcting the misdi-
rected nasolabial bundle, and coapting the deep
fibers of the vermilion end to end.
Park and Ha138 stress accurate repair of the
orbicularis oris muscle. The superficial component
of the orbicularis muscle serves as a retractor, while
the deep component serves as a constrictor of the
lip. Because of these antagonistic actions, the bal-
ance and dynamics of the repaired lip will be ham-
pered if the muscle is not properly and anatomi-
cally aligned at the time of surgery.
Effect of the Repair on Facial Growth
Joos139 evaluated the effect of muscle recon-
struction on skeletal growth. Of 110 children with
19
SRPS Volume 9, Number 21
unilateral cleft lip and palate, 50 were treated with
musculoperiosteal reconstruction at age 3 months
and velar muscle reconstruction at 9 to 12 months.
The babies received neither pre- nor postoperative
orthopedic care. A second group of 60 children
had dentofacial treatment preoperatively, lip repair
by the Millard technique at 6 months, postopera-
tive orthopedic treatment, and palatal surgery by
the Campbell-Widmaier technique at age 2½ years.
A comparison of long-term results showed that
skeletal development in the first group was better
than in the second group despite the lack of ortho-
pedic growth stimulation. The author concludes
that if the primary and secondary growth centers
are not joined at the first operation and the midfacial
muscles are not reconstructed, even orthopedic
growth stimulation will not compensate for the
defect.
Delaire, Precious, and Gordeef140 recommend
wide subperiosteal undermining in the primary sur-
gical correction of cleft lip. The authors believe
that it not only enhances the osteogenic activity of
the periosteum, but also adds suppleness to the
overlying muscles. (This concept has been advo-
cated in the past by both Veau141 and Skoog.142)
Delaire et al140 report improved maxillary growth
when a subperiosteal approach is used. The
undermining must extend to or even beyond the
muscle insertions. It is not clear from their paper
how the aberrantly inserted muscles around the
alar base and cleft margin are managed.
Joos 143 emphasizes reconstruction of the
perinasal muscles. MRI studies demonstrate that
the lateral nasal muscles, particularly at the spina
nasalis, run dorsally in a stepwise manner through
the nasal floor to insert into the nasal septum. This
explains why in a cleft lip and palate the nasal sep-
tum always deviates to the healthy side, where the
muscles insert, and why on the side of the cleft the
muscles pull upward. Joos concludes that the
paranasal muscles should be identified and
advanced as far as the midline during the cleft lip
repair.
BILATERAL CLEFT LIP
Anatomy
As excerpted from Millard,86 the following ana-
tomical features are altered in bilateral cleft lip:
20
• an intact alveolar arch with teeth in occlusion
• a lined upper labial sulcus
• an uninterrupted orbicularis oris muscle with
fibers running in a horizontal direction
• a central philtral dimple
• symmetrical philtral columns
• a Cupid’s bow
• a midline vermilion tubercle
• a white skin roll or lighter ridge topping the
mucocutaneous junction of the upper lip
• an upper lip short enough vertically to expose
the lower third of the central incisors at rest,
and more teeth with smiling and laughing
• width of the philtrum less than one-fourth the
width of the lip from commissure to commis-
sure
• an upper lip in normal relation (anteriorly
everted) to the lower lip
• an elevated, slender nasal tip
• a graceful, relatively elongated columella set at
a natural nasolabial angle of 90 to 120 degrees
• symmetrical alar rims
• unflared alar bases
• patent nasal airways bilaterally
Surgical Repair
Surgical correction of the bilateral cleft lip has
been much less satisfying than for the unilateral
deformity. Early procedures excised the prolabium
or mistakenly assumed it to be a displaced col-
umella,86 producing a lip that was grossly deficient
horizontally. Later the prolabium was used to form
the upper central lip, its vertical height supple-
mented with excess tissue from the lateral lip,144
resulting in a lip that was too long and too narrow.
These early repairs reflect the failure to recognize
the potential of the prolabium to grow in width and
height when attached to the dynamic lateral lip
elements.
Adams and Adams145 reviewed the results of
these early techniques. Their findings, along with
the developmental studies of Stark and Ehrmann,146
showed the prolabium to be a part of the upper

central lip and led to the evolution of more recent
methods of repair.
Straight-line Repair
Modern techniques concentrate on using the
prolabium for the entire central portion of the lip.
These repairs are grouped into straight-line clo-
sures and closures involving the Z-plasty principle.
Among proponents of the straight-line closure are
Veau, 141 Axhausen, 147 Brown, 148 Schultz, 149
Vaughan,150 Cronin,151 Manchester,152 and Broad-
bent and Woolf.153
Z-plasty
The Z-plasties may be in the lower lip, the upper
lip, or in both the upper and lower lip. Advocates
of the lower lip Z-plasty include Berkeley154 and
Bauer et al.155 Proponents of the upper lip Z-plasty
are Millard156 and Wynn.157 Skoog142,158 prefers a
combined upper and lower lip Z-plasty.
A discussion of the specifics of the various repairs
is beyond the scope of this overview. Fig 10 illus-
trates several preoperative designs and final result-
ing scars.
Fig 10. The Z-plasty principle in bilateral cleft lip repair.
SRPS Volume 9, Number 21
Millard Repair
Millard’s technique is detailed in his 1971 paper159
and in Cleft Craft II.86 Mulliken160 and Noordhoff161
recount their separate experiences with Millard’s
bilateral lip repair, bringing lateral vermilion flaps
beneath the prolabium and reconstructing the
orbicularis muscle beneath the elevated prolabium
(Fig 11).
Mulliken160 advocates a very narrow, concave
prolabial paring and documents prolabial widening
after a Millard repair to as much as 2.5X the normal
dimension over a 3-year period. Noordhoff161
emphasizes vermilion and Cupid’s bow reconstruc-
tion with lateral vermilion flaps.
Modified Manchester Repair
Broadbent and Woolf153 described a modifica-
tion of the Manchester one-stage primary repair
that can be carried out without preoperative orth-
odontic or orthopedic treatment of the alveolar
segments. The modified incisions on the prolabium
and lateral lip elements bring the repair out of the
nostril floor, narrow the Cupid’s bow, and place
the repair in a gently curved pattern on the philtral
edge. Closure of the lateral flaps beneath the
21
SRPS Volume 9, Number 21

Fig 11. Details of the Millard repair of complete bilateral cleft

lip. A turndown flap of vermilion from the prolabium is placed
posteriorly to prevent a whistling deformity. Note the banked
flaps of alar base that will be used later to lengthen the columella.
(Reprinted with permission from Millard DR Jr: Closure of
bilateral cleft lip and elongation of columella by two operations
in infancy. Plast Reconstr Surg (4):324, 1971.)

unfurled prolabial mucosa provides lip lining and a

normal buccolabial sulcus. The unfurled prolabium
is brought down to complete the central mucosal
tubercle (Fig 12).
The Millard technique86,159 and that described by
Broadbent and Woolf153 appear to give the most
acceptable final lip appearance. They have in com-
mon a reduction of the prolabium to a smaller
philtral dimension at the time of closure.
Fig 12. Modified Manchester one-stage repair of bilateral
complete cleft lip. (Reprinted with permission from
Broadbent TR, Woolf RM: Bilateral cleft lip: one-stage
primary repair. (Reprinted with permission from Still JM Jr,
Georgiade NG: Historical Review of Management of Cleft
Lip and Palate. In: Georgiade NG (ed), Symposium on
Management of Cleft Lip and Palate and Associated Defor-
mities. St Louis, Mosby, 1974. Vol 8.)

22

Lengthening the Columella
The modified Manchester repair keeps the
prolabial white roll and vermilion and discards the
lateral prolabial paring. The Millard repair brings in
a new white roll from the lateral lip elements and
the prolabial parings are banked for future columellar
lengthening (Fig 11). Conceivably the Broadbent
and Woolf repair could be further modified to bank
the prolabial parings for future columellar length-
ening, in Millard fashion.
Tolhurst162 recommends an early lip adhesion
with primary forked flap advancement for columel-
lar lengthening. Definitive lip repair with anchor-
age of the muscle beneath the prolabium and nar-
rowing of the prolabium to a proper philtral dimen-
sion is carried out after molding of the premaxilla
and lateral maxillary arches has occurred.
Schultz149 initially proposed restoration of orbicu-
laris oris muscle continuity in bilateral cleft lip repair.
This concept was later emphasized by Duffy163 and
championed by Randall.164
Mulliken165 describes a personal experience that
evolved from staged to synchronous surgical cor-
rection of bilateral complete cleft lip and nasal
deformity, as follows: (1) two-stage lip and nose
repair with banked forked flap and subsequent
intranasal transposition of the tines; (2) two-stage
lip and nose repair with later transection of the
banked tines; and (3) one-stage lip and nose repair
without a forked flap and with closure of alveolar
clefts. Anthropometric analysis of Mulliken’s
patients showed good anatomic results, better alar
and columellar width, and more reproducible out-
come with the single-stage repair. Mulliken165
believes that
the columella is concealed in the nose of an
infant with bilateral complete cleft lip. There is no
deficiency of columellar skin and no need to recruit
tissue from the lip or sills. The surgical stratagem is
symmetrical labial repair and synchronous anatomic
positioning of the alar cartilages with sculpturing/
draping of the nasal soft tissues.
Mulliken (1995)
Cutting and Grayson166 describe a prolabial
unwinding flap for one-stage repair of the bilateral
cleft lip, nose, and alveolus: “The columella of the
nose and the central lip are produced by ‘unwind-
ing’ the columellar and labial sections of the pro-
labium around a small central tab, which is used to
center the junction between the lip and columella.”
SRPS Volume 9, Number 21
This produces two scars running vertically along
each philtral column. The technique depends on
accurate preoperative orthopedics without lip
adhesion.
In a later update, Cutting and Grayson167,168 rec-
ommend presurgical orthopedic molding of the
lip, alveolus, and nose combined with a one-stage
lip, alveolus, and nose repair. Presurgical orthope-
dics aligns the protruding premaxilla with the maxil-
lary arch and expands the tissues of the nasal col-
umella and nasal lining with acrylic stents.169
Zheng and colleagues170 describe a method of
bilateral cleft lip repair characterized by orbicularis
oris muscle reconstruction and prolabial self-elon-
gation. The technique consists of bilateral horizon-
tal lateral-to-medial backcuts starting at the most
superior point of the prolabium paring cut. The
result is an elongated prolabial flap pedicle on the
columellar base.
The bilateral complete cleft lip is functionally ster-
ile,141 ie, the prolabium contains primarily immature
fibroblastic tissue and fine collagen, but no mature
muscle.171 The incomplete bilateral cleft lip, on the
other hand, typically has near-normal muscle pen-
etration into the prolabium. Muscle bundles are
cylindrically bunched in the remaining lip bridge,
then fan out into the prolabium.171
In summary, the applications of the prolabium
are as follows:
• to lengthen the central lip
• to lengthen the columella as needed (lateral
prolabial parings)
• to establish a continuous oral sphincter early in
the repair sequence.
The latter is done by direct suture of the lateral
muscle elements behind the prolabium as initially
described by Schultz149 and later by Glover and
Newcomb.172 Randall164 emphasizes the impor-
tance of sphincter continuity in both primary and
secondary bilateral cleft lip repairs.
When the white roll and vermilion of the pro-
labium are of good quality, a modified Manchester
repair as performed by Broadbent and Woolf153
appears to be the method of choice. The lateral
prolabial parings may be banked for future col-
umellar lengthening, and the orbicularis oris muscle
should be reconstituted behind the prolabium. If
the white roll and vermilion of the prolabium are of
poor quality, a Millard repair159 that borrows these
23
SRPS Volume 9, Number 21
parts from the lateral lip element appears to be the
better method.
LIP ADHESION
Preliminary lip adhesion in the unilateral com-
plete cleft lip, advocated as early as 1954 by
Johanson and Ohlsson,173 was mainly popularized
by Randall174 in the late ’60s. Seibert175 discusses
the principles of lip adhesion and the possible ben-
efits of the procedure. Preliminary adhesion
decreases the tension of lip closure through its
effect on the maxillary segments. Seibert175 rec-
ommends minimal soft-tissue undermining of the
lateral maxillary segment.
Randall174 suggests short broad triangular flaps
interdigitated and sutured to the mucosa, muscu-
laris, and skin. Millard,17 on the other hand, pro-
poses a high adhesion that avoids scar in the area
of the repair and that introduces lateral lip parings
into the lateral nasal vestibule. Millard132 advocates
lip adhesion as a substitute for presurgical orthope-
dics and incorporates some nasal correction in the
adhesion.
Furnas176 discusses the historical evolution of
the straight-line repair, which he proposes as a first
step before rotation-advancement closure in unilat-
eral clefts. In essence, Furnas performs a prelimi-
nary adhesion consisting of straight-line closure,
leaving the lip vertically short across the closure.
The significance of this adhesion is that lateral lip
parings are introduced into the piriform aperture
of the nose, releasing the tethering in this area and
providing muscle closure across the alveolar cleft.
Because closure is done in the neonatal period,
there is rapid molding, and subsequent rotation-
advancement can proceed under little tension. At
the time of definitive repair there is minimal nasal
deformity.
Indications for lip adhesion in the unilateral cleft
lip deformity are emotionally debated. In general,
its main application is in wide unilateral complete
clefts and in complete clefts with poorly aligned
maxillary segments. Opponents of the lip adhe-
sion principle argue that the scar introduced by the
adhesion interferes with the results of subsequent
cheiloplasty.
Because of the observed advantages of early
closure over the projecting premaxilla, the con-
cept of preliminary lip adhesion seems a logical
24
one in the bilateral cleft deformity. However,
Randall and Graham 177 reviewed their 2-year
experience with preliminary lip adhesion in chil-
dren with bilateral cleft lip, and concluded that the
operation has little to offer in complete clefts when
(1) the lip elements are not markedly separated
or distorted, (2) the alveolar segments are not
badly displaced, or (3) the lip margins of the cleft
can be brought together without undue tension.
The main disadvantage of a preliminary adhesion
is undesirable scar tissue in the area of definitive
lip repair.
Millard86 also expresses dissatisfaction with pre-
liminary lip adhesion in the double cleft deformity.
He believes primary repair with muscle-to-muscle
closure is far more effective than preliminary adhe-
sion in bringing the premaxilla into alignment, and
also avoids some scarring and loss of tissue. A
qualified exception is made for the “diminutive”
prolabium, where preliminary adhesion stretches
the prolabium to a more manageable size, creates
a philtrum of normal dimension, and the excess
tissue may be used in columellar reconstruction.
The same increase in size is seen following a pri-
mary repair, with subsequent secondary reshaping
of the philtrum and simultaneous columellar length-
ening, posing minimal risk to the prolabial tissues.
Preliminary adhesion also requires two additional
operations unless one risks elevating the prolabium
for muscle reconstruction at the time forked flaps
are advanced.
THE PROJECTING PREMAXILLA
The position of the premaxilla is cardinal to facial
morphology. The premaxillary segment consists
of paired premaxillary bones joined in the midline
at the interpremaxillary suture (a component of the
midpalatal suture). Each premaxilla articulates pos-
teriorly with the vomer and extends a short dis-
tance around it. The premaxillary vomeral suture
forms a tongue-in-groove joint (the single median
vomer represents the “tongue” and the paired pre-
maxillary bones form the “groove”).
The size of the premaxilla varies widely among
cleft patients, from a solid premaxilla containing 4
permanent incisors to a small mobile fragment
containing only two poorly formed central inci-
sors with a thin covering of bone.178 The small
isolated premaxillary segment may present an

almost insurmountable protrusion often associ-
ated with deviation.
Excision of the premaxilla to accommodate lip
closure was practiced very early by notable sur-
geons such as Pierre Franco in 1556 and Guillaume
Dupuytren.86 This gross lack of respect for the
premaxilla invariably results in a tight lip, dish-face
deformity of the mid-third of the face, and relative
mandibular prognathia.
Almost as soon as resection of the premaxilla
was abandoned, other surgeons began to employ
surgical means of retropositioning, or setting back,
the projecting premaxilla in infancy. As early as
1833 Gensoul in Paris described a compression
fracture technique to set back the projecting pre-
maxilla.86 In 1865 the basic principle of surgical
retropositioning of the premaxilla was described
by Adolf von Bardeleben, 86 whose technique
involved subperiosteal section of the vomer.
Cronin and Penoff 179 subsequently recom-
mended avoiding the suture between the vomer,
prevomerine bone, and growing septal cartilage,
and later modifications by Burston and Kernahan
are illustrated by Millard.86
Vargervik180 studied 63 patients with complete
bilateral cleft lip and palate. One group had no
surgical setback or early bone grafting procedures,
while the second group had early surgical proce-
dures to reduce a prominent premaxilla. In the first
group the premaxilla remained protrusive until age
12 and thereafter began to recede, so that by the
end of the growth period it was not excessively
protrusive. Patients in the second group showed
early midfacial retrusion that became severe as the
children grew. Vargervik concluded that, in patients
with bilateral CL/P, a protrusive premaxilla conferred
an advantage during most of the growth period
because the premaxilla grows at a far slower rate
than the mandible.
In 1947 Brown, McDowell, and Byars181 wrote:
“Briefly, the problem of the premaxilla is that it is
nearly always too far forward in the newborn baby,
but only with considerable effort can it be kept
from being retruded too far backward in the adult.”
This comment stemmed from the characteristic
long-term appearance of a patient with a total double
cleft following traditional surgical setback, ie, a hori-
zontally tight lip and inadequate projection of the
anterior maxillary arch. When other methods for
gaining control of the premaxillary segment have
SRPS Volume 9, Number 21
failed and surgical setback is deemed necessary, a
procedure less intrusive than the classical technique
is indicated.
Following dissatisfaction with primary excision,
ingenious methods of external compression were
devised. In 1768 a French surgeon named Louis
explored the concept of joining the cleft lip by
means of external compression, and advocated a
“uniting bandage to accustom the patient to the
inconvenience”.182 Griswold and Sage182 later
exercised the principle of extraoral traction in the
management of cleft lip deformities, with surpris-
ingly good results. Traction was applied from the
hour of birth for as long as 6 weeks to 3 months.
Compression was by means of a headcap made
out of a woman’s Playtex girdle. The average
amount of traction was approximately 8 oz. An
intraoral expansion device to hold the maxillary
segments out had to be applied at frequent inter-
vals.
The same principle is at work in Georgiade and
Latham’s183 use of the pinned coaxial screw appli-
ance, a controlled, fixed external traction device.
The purpose is to offer complete three-dimensional
control over the cleft segments. Controlled pres-
sure through a spring gauge exerts traction on
both the lateral maxillary segments and the pre-
maxilla. The premaxilla in this series was retracted
rapidly in 7 to 10 days, allowing early lip closure. At
the time of lip closure, gingivoperiosteoplasty was
also carried out in hopes of avoiding cross-bite and
the long-term use of intraoral retention appliances.
Georgiade184 later reemphasized preoperative
positioning of the protruding premaxilla in the bilat-
eral cleft lip patient using one of two pinned intraoral
appliances. The appliances functionally expand the
palatal shelves while retracting the premaxillary seg-
ment. The traction appliance is maintained for 10
to 14 days before the premaxillary segment is
repositioned sufficiently to allow one-step bilateral
cleft lip repair. Patients were evaluated at age 15
years to determine the long-term effects of pre-
maxillary retrusion, but no definitive conclusions
could be drawn from a comparison with normal
controls without appliances.184
Glover and Newcomb172 point out that patients
treated with methods that preserve the growth
potential of the premaxilla exhibit distinctly better
results. They advocate a bilateral lip repair that
quickly establishes continuity of the orbicularis oris
25
SRPS Volume 9, Number 21
muscle in front of the premaxilla. The authors pos-
tulate that this type of repair favors forward growth
of the deficient lateral alveolar processes and aids
in achieving normal facial contour.
Bitter185 offers his surgical protocol for manag-
ing premaxillary protrusion in bilateral cleft lip and
palate, which he believes establishes the functional
matrix as early as possible. The treatment sequence
involves insertion of a Latham’s appliance at
approximately 2 months of age. The appliance
relocates the segments over 3 to 4 weeks, and
removal of the appliance is immediately followed
by functional surgery. The first operation consists
of an intravelar veloplasty, closure of the alveolar
cleft with a gingivoperiosteoplasty, lip adhesion,
and insertion of ear tubes. The results in 41 patients
with complete bilateral clefts of the lip and palate
are encouraging, but the short observation time
(longest follow-up was 3 years) precludes defini-
tive conclusions regarding the merits of this
approach.
The following guidelines for management of the
premaxilla appear to be justified:
• If the premaxilla is well in the arch, mucoperi-
osteal flaps can be turned at the time of lip
closure to effect fibrous union of the arches.
• If the premaxilla is projecting, extraoral traction
beginning at the time of birth is indicated. In the
event of poor response to this form of therapy,
the controlled fixed-pin external traction device
of Georgiade’s186 may be needed.
• The goal of orthodontics is to achieve retrodis-
placement of the premaxilla in line with the max-
illary arches, sufficient to allow lip closure with
approximation of the lateral muscle segments
across the premaxilla. Undercorrection of the
premaxilla is certainly acceptable.
• For the overly projecting premaxilla that fails to
respond to these orthodontics, surgical setback
in an undercorrected position may be indicated,
as per Cronin and Penoff179 or Burston and
Kernahan.86 These are unusual cases.
In older patients the degree of premaxillary pro-
trusion can justify repositioning with osteotomy.
Padwa and coworkers187 state that a protrusive pre-
maxilla can be repositioned after age 6 to 8 years
without deleterious effect on midfacial growth. In
their words,
26
Premaxillary positioning in a child of 11 years or
older is unlikely to impact on midfacial growth.
However, waiting until adolescence to correct the
protruding premaxilla has a negative impact on the
nasolabial size and shape (i.e., the protruding
premaxilla stretches and elongates the upper lip
and widens the nose). In addition, postponing
premaxillary correction compromises eruption of
the maxillary incisors and proper inclination and
stabilization of the dental arch. . . . The benefits of
early surgical osteotomy and positioning of the
premaxilla include release of the nasolabial soft
tissue, closure of oronasal fistulas and alveolar clefts,
[and] alignment and stabilization of the dental arch.
. . . There is no reason to wait . . . until age 10 to
12 years to properly position the premaxilla.
Padwa et al (1999)
Iino and others188 propose surgical reposition-
ing of the premaxilla together with a staged alveo-
lar bone graft in bilateral cleft lip and palate. The
premaxillary osteotomy is performed at 8 to 14
years of age with simultaneous one-sided alveolar
bone graft. Six months later the contralateral side
is bone grafted. This technique ensures adequate
blood supply to the premaxilla and shows good
bone formation on computed tomography 1 year
after the second bone graft. The study is limited by
a short follow-up and unclear indications for this
protocol.
MEDIAN FACIAL DYSPLASIA IN CLEFT LIP
Noordhoff189 describes a subgroup of patients
with median facial dysplasia in the presence of a
unilateral or bilateral cleft lip with or without cleft
palate and without clinically detectable brain anoma-
lies. Intelligence tests show a normal distribution.
These children comprise 2% of CL/P patients.
The midline facial deficiencies in median facial
dysplasia are characterized by a poorly defined
Cupid’s bow; absence of the labial frenulum and
anaterior nasal spine; deficient columella; and
poorly developed septal cartilage and premaxilla.
One of the central incisors may be absent or
rudimentary. There are no definable gross abnor-
malities of the brain. . . . [The median facial
dysplasia patients exhibit] an inherent potential for
poor midfacial growth. Deficiencies of soft-tissue
such as lack of the Cupid’s bow make it difficult to
reconstruct the lip and nose satisfactorily.
Noordhoff, Huang, Lo (1993)

Moffat et al190 report the results of CT appraisal
of 18 children with frontonasal dysplasia and 12
children with craniofrontonasal dysplasia. The analy-
ses were compared with those of age-matched
controls. In the frontonasal dysplasia group, the
mean anterior interorbital and mid-interorbital dis-
tances were significantly increased at 148% and
118% of normal. In patients with craniofrontonasal
dysplasia, these values were 177% and 140% of
normal. Other findings in both groups included
excessive medial orbital wall protrusion, shortened
zygomatic arch lengths, and reduced cephalic
lengths. An expanded interzygomatic buttress dis-
tance was noted only in the craniofrontonasal dys-
plasia group.
Apesos and Anigian191 reviewed the etiology
and epidemiology of median cleft lip. Two major
categories of dysplasia of the frontonasal process
have been described in association with median
cleft lip: (1) frontonasal deformity associated with
hypotelorism and (2) median facial cleft syndrome
associated with hypertelorism.
FACIAL GROWTH AND MORPHOLOGY
Ortiz-Monasterio and colleagues192 analyzed
cephalometrograms of 14 untreated adults with
complete cleft lip/palate and concluded that growth
of the maxilla is normal.
More recently da Silva Filho and coworkers193
compared the craniofacial morphology of 28
untreated CL/P patients aged 15 to 40 years with a
matched noncleft group. The cleft subjects showed
smaller dimensions of the cranial base but no dif-
ference in cranial base angulation. The maxilla was
extremely prominent, which produced great facial
convexity. The mandible appeared smaller and
had a vertical growth pattern, which led to an obtuse
gonial angle and a long anterior lower face height.
The prominent premaxilla and the smaller mandible
created extreme imbalance between the jaws. The
posterior facial height was reduced and there was
a tendency toward retroclination of incisor teeth in
both jaws.
In a separate study, da Silva Filho194 compared
the upper dental arch morphology of adult
untreated complete bilateral cleft lip and palate sub-
jects to a noncleft, sex-matched population. The
authors found that while sex significantly influences
the size of the maxillary arch in normal subjects,
SRPS Volume 9, Number 21
this size differential was not present in cleft adults.
Rather, the morphologic characteristics of the max-
illary arch appeared to be influenced more by the
presence of the cleft, and consisted of premaxillary
prognathism and constriction of the maxillary den-
tal arch progressing anteriorly. The significantly
increased anteroposterior dimension was attributed
to the projecting premaxilla.
Bardach128,195-200 has investigated extensively the
effects of lip and palatal surgery on facial growth
and morphology. Using both rabbit and dog mod-
els, the author has demonstrated that unrepaired,
surgically created clefts of the lip and palate have
less lip pressure than normal, whereas repaired,
surgically created clefts have more lip pressure than
normal. Bardach showed significant inhibition of
anterior-posterior (AP) facial growth that increased
when lip repair was added. When isolated two-flap
palatoplasty was done on the control model, there
was no significant difference in the altered AP
growth disturbance previously mentioned. In addi-
tion, assessment of transverse maxillary width
showed no significant difference between the
operated and unoperated groups. When the lip
and palate were repaired simultaneously, inhibition
of AP and transverse maxillary growth was demon-
strated.
Bardach’s studies198-200 cast doubt on reports sug-
gesting various operations on the palate are
responsible for facial growth inhibition. Instead,
they tend to substantiate Sarnat’s observation of
no significant inhibition of facial growth in rhesus
monkeys after excision of a strip of mucoperios-
teum and after unilateral resection of the hard pal-
ate.
A subsequent comparison of facial growth after
cleft-lip repair with and without soft-tissue under-
mining128 showed that repairs involving sulcus inci-
sion and soft-tissue undermining were associated
with more mid-facial growth inhibition and
dysmorphia than lip repair only. Significantly higher
lip pressures were seen after repairs with soft-tis-
sue undermining.
Leipziger and associates201 used an animal model
to answer the following questions: 1) Does
aggressive bilateral soft-tissue undermining of the
nasomaxillary complex inhibit growth significantly
in an immature animal without an iatrogenically pro-
duced cleft lip? 2) If yes, is the early timing of this
undermining crucial to the inhibitory effect? The
27
SRPS Volume 9, Number 21
study involved five groups of 10 rabbits each, one
consisting of unoperated controls (Group I) and
others whose soft tissues were undermined at 3 to
4 days (Group II), 7 to 10 days (Group III), 18 to 21
days (Group IV), and 50 to 56 days (Group V).
The animals were sacrificed at 6 months of age and
osteometric values compared among the groups.
Analysis of the data showed that “a significantly
retruded, constricted, and vertically shortened max-
illa was produced as a direct result of bilateral
nasomaxillary soft-tissue undermining alone regard-
less of the timing.”
Kapucu and colleagues202 reviewed 10 adult
patients with unilateral cleft lip and palate who had
had only lip repair in childhood, no palatal repair
(Group 1). These individuals were compared with
30 adult patients with unilateral cleft lip and palate
who had had both lip and palate repair in child-
hood (Group 2). The controls were 24 noncleft
adults. Both Group 1 and 2 patients showed sig-
nificant degrees of maxillary retrusion compared
with the normal subjects. The magnitude of maxil-
lary retrusion was not increased by cleft palate
repair, and none of the cephalometric measure-
ments were significantly different between the two
repaired groups.
The effects of soft-tissue undermining on subse-
quent lip growth lend credence to Collito and
Walker’s203 admonition against it in lip repair, and
argue against recent modifications of the Millard
rotation-advancement cheiloplasty that detach the
aberrantly oriented orbicularis muscles and free
the alar complex and sulcus incision with under-
mining, particularly in the lateral segment. Never-
theless, the excellent clinical results that Millard has
demonstrated, with follow-ups ranging into adult-
hood, remain the standard against which other
repairs must be compared.
In a series of articles appearing in the Cleft
Palate Journal, Ross204-210 took an in-depth look
at the variables influencing facial growth in com-
plete unilateral cleft lip and palate, including pre-
cise timing and technique of lip and palate repair
as well as specific management of the velum,
secondary palate, and anterior primary palate.
His analysis of the effects of cleft lip repair on
facial growth showed insignificant differences
between the commonly used surgical tech-
28
niques. 207 While intrinsic factors account for
some differences in cleft and noncleft compari-
sons, the author suggests that it is probably true
that all surgical treatment for unilateral CL/P
inhibits or distorts facial growth to some extent.204
In contrast, presurgical orthopedics in the neo-
natal period has no apparent long-term effect on
facial growth.205
Surgical repair of the alveolus seems to result in
deficiency in vertical growth of the anterior max-
illa.206 If an additional bone graft or periosteoplasty
is used, the vertical growth deficiency is slightly
worse.206 Ross concludes that repair of the alveo-
lus in infancy, especially using bone grafts, has
undesirable effects.210 The best results appeared
to follow cleft repair at age 4 to 5 months without
closure of the alveolus.
The least interference with growth following
hard palate repair occurred when the repair was
deferred until the late teens.208 The growth data
for palate repair at early ages was mixed, and
there was no difference between a one-stage and
a two-stage hard and soft palate repair.209 Varia-
tions in the timing and technique of hard palate
repair within the first decade do not affect the
results appreciably. Repair of the hard palate
appears to be the major influence on maxillary
forward translation and development of the den-
toalveolar process.
Extreme differences were noted among the sur-
gical centers that were compared in Ross’s review,
underscoring the degree to which surgical tech-
nique and operating surgeon can influence the
results. Circumstantial evidence suggests that the
surgeon is the most important variable.
Dahl and coworkers211 stress that infants with
CP or CL/P have distinguishable craniofacial mor-
phologic alterations at age 2 to 3 months, before
any surgical intervention. Children with isolated
CP show a short anterior cranial base, a short
maxilla, reduced posterior maxillary height, re-
duced dimensions of the mandible, and a narrow
nasal and oropharyngeal airway; those with CL/P
have increased intraorbital width and symmetry
deviations in the anterior part of the maxilla.
Support for these observations is found in the
study by Jain and Krogman.212 The authors fol-
lowed the craniofacial growth of 64 children with
unilateral CL/P, 32 children with bilateral CL/P, and

78 children with CP only from the age of 1 month
to 10 years, and found that the cleft type differ-
ences were mainly restricted to the baseline width,
intraorbital width, optic foramen width, basal maxil-
lary width, intraorbital height, optic foramen height,
and gonial height.
Joos213 notes that the results following functional
cleft lip and palate muscle reconstruction vary even
when the same treatment is applied to similar
patients of similar ages. The cause of this discrep-
ancy is found in the individual growth process and
the degree to which it is affected by functional
stimuli.
The ethmoid region and the alar-orbital-temporalis
region of the cartilaginously preformed skull base
play a special role in the development of the face.
Joos213 set out to establish a relationship between
the conditions at the skull base in childhood and
the adult dentoalveolar status. Radiographic com-
parisons revealed that a tendency at the skull base
to a class I in most cases led to a slight dentoalveo-
lar angle class III. In contrast, a tendency at the
skull base to class II usually resulted in an angle
class I or II relationship. A tendency at the cranial
base to class III always resulted in a severe dentoal-
veolar angle class III relationship. The author con-
cludes that predisposing factors in the skull base
have a substantial influence in the result and man-
agement of CL/P.
Satoh and others214 used lateral cephalograms
to evaluate growth of the bony nasopharynx from
early childhood to puberty in patients with com-
plete unilateral cleft lip and palate with complete
velopharyngeal closure. All patients underwent
Tennison lip repair at about 6 months of age and
pushback palatal repair at about 18 months. Com-
pared with noncleft controls, the study patients’
maxillae were more posterosuperior but there were
no differences in cranial base or cervical vertebra
measurements—evidence of independent growth
of these structures. The nasopharyngeal angle con-
necting three points of the cranial base, posterior
maxillary points and atlas showed harmonious
growth in both subjects and controls.
From a review of the evidence it seems clear
that the craniofacial morphology of children and
adults with operated clefts is the result of both
intrinsic and environmental factors operating upon
it, and cannot invariably be considered a conse-
quence of the surgery.
SRPS Volume 9, Number 21
ORTHODONTICS AND BONE GRAFTING
Vig and Turvey215 record the orthodontic and
surgical interaction in CL and CL/P patients begin-
ning in the neonatal period and extending through
the phases of mixed and permanent dentition. The
orthodontic and surgical options that relate to spe-
cific skeletal findings in the different age groups are
reviewed.
Peat 216 looked at the long-term effects of
presurgical oral orthopedics on children with bilat-
eral complete cleft lip and palate. The treated and
untreated group were identical in terms of the type
and timing of surgery. The premaxillary segments
were affected in both the deciduous and mixed
stages; little permanent effect was seen in the buc-
cal segments. On cephalometrics, there was no
significant difference in any of the craniofacial angles
between the treated and untreated groups. The
vomerine flap used in the hard palate repair appar-
ently created scar tissue that collapsed the buccal
segment, accounting for the lack of long-term effect
by presurgical orthopedics.
Kramer and colleagues217 studied early (from
birth to age 3 months) palatal development in vari-
ous complete and incomplete forms of cleft lip and
palate by means of dental casts. The authors used
reproducible reference points to plot palatal shape
and dimensions in 128 affected children and 68
normal children who served as controls. There
was substantial normal palatal growth in the first 3
months of life in all subjects, and the authors con-
cluded that preoperative maxillary orthopedics does
not stimulate palatal growth, but rather restricts it.
In a retrospective analysis of 29 patients with
early maxillary orthopedics, Gnoinski218 found all
patients who had a “balanced” skeletal relationship
at age 15 had had lip surgery after 5½ months of
age and palatal surgery after age 3. Lip surgery
before 5 months of age and palatal surgery before
2 years regularly resulted in an unbalanced or defi-
nitely critical skeletal situation at age 15. While the
author stressed the role of timing of surgery in
these deleterious effects, it should be recognized
that the type of repair is equally important.
Rygh and Tindlund219 detail the appropriate orth-
odontic treatment plan beginning in the deciduous
dentition phase. Conventional correction of the
maxilla was mainly by rotation and expansion of
the lateral segments, with some lateral movement
of the maxillary bones and labial tipping of the lin-
29
SRPS Volume 9, Number 21
gually positioned upper incisor teeth. These meth-
ods failed to yield any appreciable forward move-
ment of the maxillary dentoalveolar arch basal parts.
The authors, following the method of Delaire, sug-
gest application of a protraction face-mask tech-
nique to promote an increase in vertical height and
sagittal length of the maxilla. Because sutural growth
in the upper jaw is most active at age 6 to 7 years
and then declines until the pubertal spurt, maxillary
protraction therapy must be instituted before the
age of 8 years if significant effect is to be obtained.
Age 6 seems the ideal time to begin therapy, since
eruption of the permanent maxillary incisors takes
place during the treatment period.
The orthodontic plan for the adolescent cleft
child with a relative class III relationship secondary
to a retruded maxilla must be carefully scrutinized.
While the orthodontist may be able to narrow the
upper and lower arches through extraction of first
molar or bicuspid teeth and improve occlusal rela-
tionships, this scheme may well limit the appropri-
ate orthognathic correction based on the soft tis-
sue relationships in children so affected. These
children generally benefit from an orthodontic plan
that allows the maximum orthognathic advance-
ment of their Le Fort I segment.
ALVEOLAR BONE GRAFTS
The appropriateness of bone grafts in closure of
alveolar clefts remains controversial. The technique
of maxillary alveolar cleft repair with a cortico-
cancellous block graft of contoured iliac crest bone
and marrow packing is illustrated by Demas and
Sotereanos220 (Fig 13).
In a retrospective analysis, Walle and Forbes221
studied the effect of alveolar cleft size on bone
graft success. Other factors such as surgical tech-
nique, patient age at surgery, and preoperative level
of interproximal bone can affect the outcome of
bone grafting. In this study the area of defect and
the surgeon influenced the degree of bony sup-
port and percent bone fill that was attained. Bilat-
eral clefts had a lower percentage of bone forma-
tion than unilateral clefts, and more surgeries were
needed to achieve a satisfactory result.
Sadove and coauthors222 compared iliac bone
and cranial bone harvested by two different tech-
niques in the closure of alveolar clefts. Both iliac
bone and calvarial bone dust resulted in high rates
30
of osseous continuity between maxillary segments
and closure of the alveolar defect. Calvarial bone
obtained with a craniotome had a statistically lower
success rate than the other two groups.
McCanny and coworkers223 compared the tre-
phine technique with the open hip technique for
harvesting cancellous bone grafts. The open tech-
nique resulted in greater postoperative morbidity,
such as a limp and an infected hematoma, and the
authors conclude that trephine is the preferred har-
vesting technique for alveolar bone grafts.
Boustred and associates224 described a minimally
invasive harvesting technique for cancellous bone
grafts from the ilium. Through a small step incision
5–8 mm in length, the midportion of the iliac crest
is raised. With a curette, a core of fresh cartilage is
removed and cancellous bone is scraped from
between the inner and outer cortices. The amount
of bone obtained is sufficient for narrow as well as
wide defects. The technique is quick and needs no
special equipment. There is minimal donor site
morbidity and the patient walks comfortably from
the first postoperative day.
Friede and Johanson225 reviewed the adolescent
facial morphology of children who had undergone
early bone grafting of their alveolar segment. Sub-
ject age ranged from 15 to 20 years and included
19 bilateral and 42 unilateral cleft patients. Cepha-
lometric data indicated maxillary retrognathia in both
cleft categories as well as deficient vertical descent
of the maxilla, especially in the anterior part. In
about 40% of the bilateral and 50% of the unilat-
eral cleft patients, mid-facial growth attenuation had
reached such magnitude that surgical advancement
of the maxilla was necessary. Fusion of the suture
between the premaxilla and vomer was thought to
be the cause of the most pronounced growth
impairment. The authors concluded that early bone
grafting of the alveolus should be avoided.
An 11-year follow-up of the effects of early bone
grafting in infants born with complete clefts of the
lip and palate is presented by Robertson and
Jolleys.226 In this report matched pairs of similar
cleft types were treated according to the same
treatment protocol, except that only one of each
pair received an autogenous rib bone graft at the
age of 15 months. The overall impression was that
early bone grafting is not beneficial to the patient.
Rintala and Ranta227 agree with Robertson and
Jolleys226 on the basis of their review of 90 patients
 SRPS Volume 9, Number 21

Fig 13. Technique of alveolar cleft repair with bone grafts.

(Reprinted with permission from Demas PN, Sotereanos GC:
Closure of alveolar clefts with corticocancellous block grafts and
marrow: A retrospective study. J Oral Maxillofac Surg 46:682,
1988.)

31
SRPS Volume 9, Number 21
segregated according to closure with maxillary pe-
riosteal flaps (n=67) and pretibial periosteal grafts
(n=23). Definite bone bridge occurred in 64% and
85% of patients respectively, but more than 70%
required secondary bone grafting regardless of the
type of procedure used. Lateral cross-bite was
seen in all patients, and anterior cross-bite occurred
in 80% of both groups. The use of periosteum
failed to prevent maxillary collapse or lessen the
need for secondary bone grafting. A dissenting
opinion is expressed by Rosenstein et al228 and
Monroe et al.229
Rosenstein and coworkers230 report the results
of early bone grafting and infant maxillary ortho-
pedics in 20 patients who had complete unilateral
clefts of the lip, alveolus, and palate, and 17 patients
who had complete bilateral clefts. Anteroposte-
rior and vertical facial growth was evaluated by
cephalometric analysis. Compared with the origi-
nal series,228 these patients showed no adverse
growth restraints, and the authors conclude that
their protocol—consisting of early primary bone
grafting and orthodontics—”leads to teeth in bet-
ter overall occlusion than if it had not been under-
taken.”
Trotman and colleagues231 reported on a col-
laborative study from two cleft palate centers in
which the post-treatment craniofacial morphology
of patients with complete cleft lip and palate was
assessed. Patients from the Chicago clinic received
primary alveolar bone grafting with definitive lip
repair at age 4 to 6 months and hard- and soft-
palate repair at age 6 to 12 months. Patients from
the Lancaster clinic underwent definitive triangular
flap lip repair at age 3 months followed by staged
surgeries of the hard- and soft-palates, both com-
pleted by age 18 months, but without primary
alveolar bone grafting. Outcome analysis showed
that
. . . the grafted Chicago group had faces that
were on average less maxillary protrusive compared
with the nongrafted Lancaster sample. It appeared,
however, that the mandible compensated for the
maxillary position by downward and backward
rotation. As a result, a similar maxillomandibular
relationship was noted in both groups, although in
the Chicago group the lower anterior facial height
increased.
Trotman et al (1996)
32
Abyholm and colleagues232 offer an excellent
discussion of the appropriate timing for secondary
bone grafting of alveolar clefts. The surgical goal is
cancellous bone graft to the residual cleft of the
primary palate with subsequent orthodontic move-
ment of teeth into the former cleft area. A review
of their cases suggests that optimal results were
obtained when bone grafting was performed before
full eruption of the cleft-side canine. In this situa-
tion the known potential of an erupting tooth to
induce alveolar bone generation proved to be of
great advantage. By deliberately guiding the erupt-
ing canine through the grafted area close to the
incisor, a nearly normal intraalveolar septum was
formed and the gap in the dental arch was closed
orthodontically in 23 out of 26 clefts. Additional
advantages of their procedure include stability of
the maxillary segments, closure of oronasal fistulas,
and support for the receded alar base. Complica-
tions in this series of 89 clefts consisted of two
bone graft losses secondary to infection.
Kalaaji and associates233 compared the outcome
of secondary and late secondary bone grafting in
46 patients with complete unilateral cleft lip and
palate. The dehiscence rate was 23% and total
failure rate was 4%. Alveolar bone height was
>75% of normal in 81% of patients. The cleft space
was closed by orthodontic means in 49%. The
best results in this study were obtained when bone
grafting was performed before the canines erupted.
The surgeon’s experience was also a factor.
Paulin and colleagues234 compared the maxillary
dimensions in two groups of patients undergoing
alveolar bone grafts, one group before the erup-
tion of the canine tooth and the second group
after the eruption of the canine tooth. Initial heal-
ing was more favorable in the group that was
grafted prior to eruption of the canines.
The long-term results of secondary bone graft-
ing of alveolar clefts was evaluated by Enemark
and associates.235 Three groups of patients (224
clefts) were studied: one group had alveolar bone
grafts before eruption of the canines; a second
group had alveolar bone grafts after eruption of
the canines; and the third group had alveolar bone
grafting after 16 years of age. In the younger
groups, the marginal bone level was found to be
significantly higher among unilateral CL/P patients
than those with bilateral deformities. Analysis indi-
cated significantly better results with secondary

bone grafting when treatment was performed
before eruption of the canines.
Honma and others236 used computed tomogra-
phy to evaluate the fate of secondary cancellous
bone graft of alveolar clefts. They noted decreas-
ing volume of new bone in the cleft site from 3
months to 1 year after grafting. This trend was
reversed in two patients who had tooth eruption
into the grafted site, suggesting that a functional
load in this area, be it a functioning tooth or
endosseous implant, might prevent bone resorp-
tion.
Bone grafts to the alveolar cleft are the standard
method of bridging the bony gap, followed by orth-
odontic tooth movement and alignment of adja-
cent teeth into the grafted cleft. Children with mixed
dentitions usually are not candidates for tooth
movement to optimally close the dental arch over
the cleft. The lateral incisor and sometimes the
central incisors and canines erupt into the osseous
cleft and are lost. Boyne237 established the suitabil-
ity of particulate autogenous osteogenic marrow
and cancellous bone grafts in reconstructing the
dental arch. Working with macaque monkeys,
Boyne noted complete graft union and replace-
ment with new bone within 6 months. The grafted
bone is remodeled and recontoured to form a
normal trabecular pattern and alveolar cortical sur-
faces of lamellated bone. The developing tooth
buds on either side of the grafted areas are undis-
turbed by the surgery.
Hamamoto and coworkers238 looked into the
ideal timing for autotransplantation of teeth into
bone-grafted alveolar clefts in humans. At 6
months the bone graft was still undergoing
remodeling and replacement with newly formed
bone. The active remodeling was completed by
12 months. The authors conclude that tooth trans-
plantation should be performed soon after the
formation of a bone bridge is confirmed, when
bone remodeling is still underway, to provide an
occlusal load to the graft and prevent resorption.
Orthodontic treatment of the transplanted teeth
can be started 3 months after transplantation when
regeneration of the periodontal space and lamina
dura is confirmed on dental radiographs. Thus
the “transplanted teeth can be moved ortho-
dontically, and the grafted alveolar bone support-
ing the teeth grows in harmony with the adjacent
normal alveolar bone.”
SRPS Volume 9, Number 21
The problem of edentulousness in the cleft area
of a patient with cleft lip and palate is usually ap-
proached by construction of a fixed bridge. This
solution is not always optimal. Lilja and associ-
ates239 in Sweden and Jansma and others240 in the
Netherlands describe their experience with titanium
implants for dental rehabilitation of patients with
cleft lip and palate.
Gingivoperiosteoplasty
Smahel and Mullerova241 looked at the facial
growth of unilateral cleft lip and palate patients after
10 years of primary periosteoplasty. Children
treated by primary periosteoplasty (n=35) were
compared with a matched series treated by pri-
mary osteoplasty (n=32) and another group surgi-
cally repaired without a bone graft or periosteal
flap (n=30). Jaw development was best after pri-
mary periosteoplasty and worst after bone graft.
Facial changes after periosteoplasty consisted of
mild retrusion of the upper jaw, maintenance of
overjet, and satisfactory prominence of the upper
lip.
Wood, Grayson, and Cutting242 report the effect
of gingivoperiosteoplasty on growth of the midface
skeleton 6 years after primary surgical repair. All
patients received preoperative orthopedics with
passive molding appliances followed by repair of
the lip, alveolus, and nose in a single operation at
age 3 months. Repair was by rotation-advance-
ment technique. The only difference between treat-
ment groups was whether or not gingivo-
periosteoplasty was performed. After relatively
short follow-up, no significant difference in mean
position of ANS-PNS was found between the
groups. The authors conclude that “gingivo-
periosteoplasty results in a more uniform position
of the hard palate” without apparent impairment of
maxillary growth.
Lehman and coworkers243 report an experience
with one-stage closure of the entire primary palate
at the approximate age of 3 months in 61 patients.
A single procedure is performed that closes the lip,
anterior hard palate, and alveolus. Mucosal turn-
over flaps from the vomer, along with lateral nasal
mucosal flaps, provide the nasal lining, and a buccal
sulcus flap completes the oral repair. The second-
ary palate is repaired by 12 months of age. The
authors report complete and stable closure in 95%
33
SRPS Volume 9, Number 21
of patients. The incidence of fistula was 3 of 61. A
longer follow-up will show if this protocol averts
the need for bone grafting. If the prevomerine
suture is not disturbed during surgery, these authors
and others feel that early surgery is no more del-
eterious to maxillary growth than surgery later in
life.
Smith, Markus, and Delaire244 describe primary
closure of the cleft alveolus as a two-stage tech-
nique. Meticulous nasolabial muscle reconstruc-
tion is performed at age 6 months in patients with
unilateral clefts and at age 4–5 months in patients
with bilateral clefts. The inferior surface of the
reconstituted nasal floor is covered with the recon-
structed transverse nasalis muscle and, where nec-
essary, a modified mirror flap. Permanent closure
of the lip and floor of the nose is carried out in
cases of complete cleft at the same time as closure
of the soft palate to encourage narrowing of the
alveolar and hard palatal defect and good maxillary
arch alignment without presurgical orthopedics. At
about 14 months of age the second stage of pri-
mary closure is carried out by means of gingivo-
periosteoplasty. Mucoperiosteal flaps are mobi-
lized and advanced across the alveolar defect on
the labial aspect by wide subperiosteal undermin-
ing. The integrity of all sutures is preserved to
maximize postoperative growth. The authors state:
The median septal system is an important
structure influencing premaxillary development.
The nasolabial muscles, in particular transverse
nasalis, superficial and deep elevators of the upper
lip including alaeque nasi and orbicularis of the
upper lip converge on each side toward the anterior
nasal spine and insert directly and indirectly onto
the antero-inferior aspect of the septal cartilage,
thereby forming a ‘musculo-periosteal tent.’ . . .
Primary surgeru must aim to reinsert the nasolabial
muscles into the nasal septum and anterior nasal
spine, preserving all the elements of the median
septal [system].
Smith, Markus, Delaire (1995)
In a study of maxillary asymmetry in children
with clefts of the lip and palate treated at three
different cleft palate centers in Europe, Molsted et
al 245 determined that children who receive
presurgical orthopedics and a primary bone graft-
ing procedure at the time of lip closure—at age 4 to
6 months—achieve a more symmetrical dentoal-
veolar development than if bone grafting is not
34
performed at an early age. However, maxillary
development in these children is retarded, not only
in the sagittal plane but also in the transverse plane.
In contrast, children who are treated by early lip
closure and vomerplasty with no involvement of
the alveolar process exhibit asymmetrical develop-
ment, with a tilting premaxilla and deviating inclina-
tion of the central incisors. This study once again
confirms the negative effect of vomerplasty on
midfacial growth.
PRIMARY CORRECTION OF THE
CLEFT NASAL DEFORMITY
Traditional methods of lip repair did little to ad-
dress the associated cleft nasal deformity. While
alar base repositioning was common, release,
repositioning, and dissection of the nasal complex
was avoided because of the general feeling that
growth disturbances would ensue. The literature
now suggests that many cleft surgeons consider
correction of the associated cleft alar deformity to
be part of the lip repair.
Nasal growth in complete bilateral cleft lip/pal-
ate was evaluated by Ishii and Vargervik.246 Their
findings suggest that
• the nose of subjects with bilateral cleft lip and
cleft palate grows more downward than for-
ward
• the growth spurt occurs between age 12 and
16 years
• forward growth of the nose is almost always
seen, whereas the maxilla becomes more retru-
sive
McComb247,248 and Salyer249 report long-term
follow-up on the nasal deformity corrected at the
time of lip repair. In general, the authors247-249 sug-
gest that correction of the nasal deformity at the
time of lip repair endures, does not disturb growth,
and is beneficial to nasal morphology. They advo-
cate mobilization of the lower lateral cartilage from
the overlying nasal skin, simultaneously releasing it
on the cleft side from attachments along the piri-
form aperture and involving the medial crus in the
septal area. The method creates a flap of the lining
mucosa and cartilage, with the lateral portion of
the lower lateral cartilage advanced medially and
cephalad and the medial crus advanced toward the
tip. Suture-fixation or bolster-suturing techniques

are used to hold the cartilage in position. The
excellent results depicted in these articles should
certainly encourage the cleft surgeon to consider
correction of the cleft nasal deformity at the time
of lip repair.
Takato and coworkers250 described early cor-
rection of the nose through an open method in
unilateral cleft lip patients. The cleft nasal deformity
was corrected during the preschool years, and fol-
low-up evaluation was done when the patients were
between 15 and 19 years of age. Although early
results appeared satisfactory, as the patients
approached their adolescent growth spurt at ap-
proximate age 15, undesirable features became
obvious. Patients showed strikingly large noses
with large amounts of subcutaneous fat, thick skin,
and a wide nasal tip. These features were consid-
ered unique to patients undergoing early cleft nasal
repair by the open method. Consequently, the
authors discontinued open rhinoplasty in pre-
schoolers and now delay secondary correction until
age 9 to 12 years.
Cussons et al251 assessed the results of early
versus no nasal correction of the cleft lip nose by
means of a panel of “blind” observers who were
asked to rank standardized patient photographs.
Scores were separately given for three features, 1)
symmetry of the upper nasal perimeter, 2) symme-
try of the nostril outline, and 3) overall esthetic
appearance. A final rank was calculated by adding
the scores and then ranking the totals. Each fea-
ture was compared with itself and with other fea-
tures across treatment groups. Analysis of the rank-
ing data showed a significant difference between
groups, with normal controls ranking consistently
highest, followed by corrected noses, which
showed better symmetry than if no nasal surgery
had been done.
Cutting and Bardach252 made a comparative
study of the skin envelope of the unilateral cleft lip
nose subsequent to rotation-advancement and tri-
angular flap lip repairs. Both groups had similar
vertical asymmetries of the nasal skin envelope—
that is, the alar dome on the cleft side was
depressed, the columella was short on the cleft
side, and there was hooding of the nostril apex.
The principal difference between the two lip repairs
was observed in the horizontal dimension in the
nasal skin envelope. Specifically, the position of
the alar base was more normal following the Millard
SRPS Volume 9, Number 21
repair, while the triangular flap repair left the alar
base laterally displaced.
McComb253 reviews his 15-year experience with
columellar reconstruction in the primary repair of
bilateral cleft lip employing forked flaps. The author
describes three unfavorable features of this proce-
dure, namely (1) the columella may grow too long
and the nostrils too large; (2) often the nasal tip
remains too broad; and (3) the columellar base
tends to drift and a scar courses through the lip-
columellar angle. On the basis of these long-term
unfavorable consequences, McComb253 advocates
a new treatment plan involving a two-stage repair
fully described in his article.
Van der Meulen254 describes a technique of col-
umellar elongation in bilateral cleft lip repair that
consists of three-dimensional Z-plasty on the alar
rim to lengthen the columella and achieve forward
projection of the tip. Technical details are illus-
trated in the article. Indications for the procedure
are to correct flattening of the nasal tip and short-
ness of the columella while avoiding undesirable
scars on the surface of the nose or lip. Early results
in 15 patients are encouraging.
Nakajima, Yoshimura, and Kami255 review a
largely successful experience with the Tajima repair,
which employs a reverse-U incision along the alar
margin with a back-cut along the vestibular lining at
the junction of the piriform aperture to release the
plica vestibularis.
An objective evaluation of the Tajima secondary
cleft lip nose correction is offered by Coghlan and
Boorman.256 The early and late results of surgery
wer objectively measured by computer on 24 uni-
lateral complete cleft lip and palate patents. Late
results ranged from 1 to 4.2 years. In the late
follow-up group the deformity recurred and the
nasal shape could not be satisfactorily and statisti-
cally separated from the preoperative appearance.
In two separate articles, Trott and Mohan257,258
report the preliminary results of open-tip rhinoplasty
at the time of lip repair in unilateral and bilateral
cleft lip and palate. The method consists of simulta-
neous lip closure and open-tip rhinoplasty involv-
ing nostril and columellar rim incisions. The surgi-
cal emphasis is on alar cartilage manipulation, with
the skin being adjusted secondarily. The tip of the
nose is reconstructed from alar dome reposition-
ing and fixation under direct vision, construction of
a subcutaneous soft-tissue pad, and caudal advance-
35
SRPS Volume 9, Number 21
ment of dorsal nasal skin. Wide dissection of nasal
mucosa from the medial wall of the maxilla and
piriform margin allows correct positioning of the
alar base and prevents intranasal stenosis. Wide
dissection and advancement of cheek soft tissues
supports the alar base and allows for a tension-free
closure of the lip.
Matsuo and Hirose 259 propose a rotational
method of bilateral cleft lip nose repair that length-
ens the columella from above by rotating and
advancing the lateral crus into the domal zone.
Figi’s “flying bird” incision is extended to the col-
umellar base along the nostril margin. Through
this incision the lower one-third of the nose,
including the alae and nostril floors, is undermined
widely. The nostrils are freed from the surround-
ing tissues except in the region of the columella
and septum, and are then rotated superiorly and
medially toward each other. The operative details
and clinical results of the operation are illustrated
in the article.
Nonsurgical molding of the cleft alar deformity
in the early neonatal period is advocated by Matsuo
and coworkers.260 The nose is stented at the time
of surgery, which is performed between days 2
and 7 of life. The mold or retainer in the affected
nostril is kept in place for 3 months. Except for a
single nasal infection, there were no complications
of the procedure, and nasal shape and symmetry
were considered superior to those conventionally
operated on at about 3 months of age.
Cutting, Grayson, and others 167-169 advocate
presurgical orthopedics and nasal molding as part
of the treatment plan. In the unilateral deformity,
they use a molding plate with nasal stent. The nasal
stent elongates the columella on the affected side
and repositions the lower lateral cartilage. The oral
portion of the molding plate corrects the alveolar
deformity. For the bilateral deformity, they use an
oral molding plate with bilateral nasal stents. The
nasal stent elongates the columella and reshapes
the lower lateral cartilage. The appliance also has a
horizontal prolabial band attached to the nasal stent
that depresses the columella at the junction with
the prolabium. The prolabium is inferiorly stretched
with steri-strips.
The benefits of a dynamic nostril splint in sur-
gery of the nasal tip are emphasized by Cenzi and
Guarda.261 Their experience “has shown that the
36
application of a dynamic nasal splint has contrib-
uted efficiently to maintaining the surgical results
by opposing healing contraction.”
Nakajima et al262 also subscribe to the policy of
postsurgical splinting for 3 to 4 months to retain
the corrected contour of the cleft lip nose, and
recommend moldable silicone rubber retainers to
add volume to ready-made nostril splints. This
method achieves and maintains precise alignment
of tissues and slight overcorrection of the defor-
mity.
Yeow and coworkers263 noted a tendency of
the lower lateral cartilage to return to its preopera-
tive malformed state despite primary surgical cor-
rection of the cleft lip nasal deformity. They now fit
patients with a nostril retainer that is to be worn for
at least 6 months to maintain the correction, and in
a recent paper they reported good results with
nasal splinting. The children who had postopera-
tive nasal splinting showed significantly better
esthetics than those who were not splinted, and
the authors recommend the use of nostril retainers
for at least 6 months postoperatively.
* Editor’s Note: The tissue on the nostril floor in an
incomplete cleft lip is commonly called a Simonart’s
band. Millard states that “even the most minor
Simonart’s band acting as a restrainer in utero
greatly reduces the extent of maxillary and nasal
distortion.” The history of the eponym he recounts
as follows:
While with Gillies at Rooksdown I worked
with Holdsworth who referred to the residual
congenital skin bridges with or without muscle that
occasionally span the upper part of a lip cleft as
‘Simonart’s’ or ‘Simonartz’ bands. Since then I have
been guilty of using this term loosely. In 1976
intellectual Tom Gibson, intrigued by these terms,
started an intensive search for their origin and
found that Gustav Simon in his 1868 book had
presented the above operation for bilateral clefts.
Gibson then deduced that someone subsequently
must have written about repositioning of the
premaxilla by creating the transverse bands of
‘Simon Arzt in Rostock’ which is an operative band
and not a congenital one. This no doubt solves the
name origin of the fictitious Simonart’s band which
I propose could now be designated Gibson’s bridge.
Millard, Cleft Craft II, p 175-6

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261. Cenzi R, Guarda L: A dynamic nostril splint in the surgery
of the nasal tip: technical innovation. J Craniomaxillofac
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262. Nakajima T, Yoshimura Y, Sakakibara A: Augmentation
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SRPS Volume 9, Number 21

RECOMMENDED READING

Jones MC: Facial clefting: etiology and developmental pathogenesis. Clin Plast Surg 20(4):599, 1993.
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Noordhoff MS: Reconstruction of vermilion in unilateral and bilateral cleft lips. Plast Reconstr Surg
73:52, 1984.
Millard DR Jr, Latham RA: Improved primary surgical and dental treatment of clefts. Plast Reconstr Surg
86:856, 1990.
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Plast Reconstr Surg 80:29, 1987.
Joos U: Skeletal growth after muscular reconstruction for cleft lip, alveolus, and palate. Br J Oral
Maxillofac Surg 33:139, 1995.
Cutting C, Grayson B: The prolabial unwinding flap method for one-stage repair of bilateral cleft lip,
nose, and alveolus. Plast Reconstr Surg 91:37, 1993.
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a second report. Plast Reconstr Surg 87:644, 1991.
Smith WP, Markus AF, Delaire J: Primary closure of the cleft alveolus: a functional approach. Br J Oral
Maxillofac Surg 33:156, 1995.
McComb H: Primary repair of the bilateral cleft lip nose: a 15-year review and a new treatment plan.
Plast Reconstr Surg 86:882, 1990.
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Cleft Palate Craniofac J 35:40, 1998.
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making process. Cleft Palate Craniofac J 36:105, 1999.
Millard DR, Latham R, Huifen X, et al: Cleft lip and palate treated by presurgical orthopedics,
gingivoperiosteoplasty, and lip adhesion (POPLA) compared with previous lip adhesion method:
a preliminary study of serial dental casts. Plast Reconstr Surg 103:1630, 1999.
Santiago PE, Grayson BH, Cutting CB, et al: Reduced need for alveolar bone grafting by presurgical
orthopedics and primary gingivoperiosteoplasty. Cleft Palate Craniofac J 35:77, 1998.
Nakajima T, Yoshimura Y, Yoneda K, Nakanishi Y: Primary repair of an incomplete unilateral cleft lip:
avoiding an elongated lip and achieving a straight suture line. Br J Plast Surg 51:511, 1998.
Cutting C, Grayson B, Brecht L, et al: Presurgical columellar elongation and primary retrograde nasal
reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg 101:630, 1998.
Padwa BL, Sonis A, Bagheri S, Mulliken JB: Children with repaired bilateral cleft lip/palate: effect of age
at premaxillary osteotomy on facial growth. Plast Reconstr Surg 104:1261, 1999.
Satoh K, Wada T, Tachimura T, et al: A cephalometric study by multivariate analysis of growth of the
bony nasopharynx in patients with clefts and non-cleft controls. J Craniomaxillofac Surg 26:394, 1998.

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