Cleft lip and palate /certified fixed orthodontic courses by Indian dental academy

INDIAN DENTAL ACADEMY
Leader in continuing dental education
www.indiandentalacademy.com


INTRODUCTION

Cleft lip and palate is one of the most common congenital
anomalies afflicting humans. The potential problems that may beset
the unfortunate victims of the condition include such severe
handicaps as impaired suckling and resultant failure to thrive,
speech impediment, deafness, malocclusion, gross facial deformity,
and severe psychological problems.


The clefting of lip and/or palate occurs at such a strategic
place in the orofacial region and at such a crucial time (before
birth) that it becomes a complex congenital deformity and a
major therapeutic challenge incapable of resolution by any
individual clinician and it warrants the coordinated skills of
many modern specialists for its correction.
Plastic
surgeons,
pediatricians,
orthodontists,
prosthodontists,
speech
pathologists,
otolaryngologists,
anesthesiologists, and audiologists form a less than
comprehensive list of those who may be required to provide a
high degree of interdisciplinary communication and
understanding in the treatment of cleft lip and palate deformities.

HISTORY
In ancient times many congenital deformities including cleft
lip and palate, were considered to be an evidence of evil spirit in the
afflicted child. These children were often removed from the tribe or
cultural unit and left to die in the surrounding wilderness.
The repair of cleft lip is noted in an ancient monograph
during the Chin Dynasty (255-206 BC) and is the earliest report of
such surgery any where in the world.
Jehan Yipperman, a Flemish surgeon (1295-1351),
LeMonnier, a French dentist (1753) described in detail the repair of
a cleft lip and palate patient. In the United States, Josiah Flagg,
surgeon- dentist, advertised in a handbill in Boston in 1786, ……….
that among the many other things he does, he also sews up hare lip.


Stevens performed the first cleft palate repair in the United
States in 1827.
Fauchard, in 1786, made valuable contributions and
innovation in prosthodontics, particularly in the area of obturators
for cleft lip and palate patients.
Kingsley who shares with others the claim to be the Father of
Orthodontia has written over 100 articles on cleft palate
rehabilitation, the inadequacies of cleft palate surgery, obturators,
orthodontic diagnosis and orthodontic appliances bear his name.
His first book, a treatise on Oral Deformities, as a Branch of
Mechanical Surgery, in 1880, featured the orthodontic appliances of
the period, including jack screws, retainers and arches with
ligatures.

Calvin Case published a Prosthetic Treatise on the
Techniques and Principles of Dental Orthopedia and Prosthetic
Correction of the Cleft Palate in 1921. The appliances described
were all of the fixed type.
It was only a few years ago that orthodontists demonstrated
that palatal surgery interfered with maxillary growth. Surgeons
were asked not to repair the palate until maxillary growth was
complete so as to avoid midfacial deformity.
At the same time speech and language pathologists were
requesting for early operation on palate to prevent the development
of compensatory mechanisms of articulations.


Taking note of the suggestions made by both
orthodontists and speech pathologists the surgeons have modified
their techniques, the timing of surgery, and the number of palatal
procedures that were performed.
Along with surgical refinements, advances have
been made in orthodontics and other fields concerned with
rehabilitation of cleft lip and palate to such an extent that, the
treatment of children with clefts have resulted in a cleft
population that is distinctly different from the facially crippled
stereo-types of previous generations.


INCIDENCE OF CLEFT LIP AND PALATE

Ø     In India, Incidence of major malformations in total births
is  estimated to be 14.82 per 1000 births.
Ø     The incidence of Cleft lip with or without cleft palate was
1.25 and that of cleft palate alone was 0.46 per 1000 births.
Ø      In other parts of the world, the incidence of this anomaly is
reported to be the highest in Afghans as 4.9 and lowest in
the Negroid population as 0.4 per 1000 live births.


EMBRYOLOGY OF CLEFT LIP AND PALATE

Ø     Ralph Millard Jr. once said, “Though various theories have
been put forth to enlighten the panorama of the embryological
basis, no theory enjoys universal acceptance as no one has been
an eye witness to the entire 'in utero show' as yet and thus it is
impossible to propose and prove what exactly is happening.”

Ø      Wilhelm His popularized the theory that separate facial
processes fuse to form the mid portion of the face.
Ø     It was Richard B. Stark of St. Luke Hospital, New York, in
1954, who wrote extensively explaining mesodermal migration.

There are five principal stages in craniofacial development:
Ø     Stage – I Germ layer formation and initial organization
of craniofacial structures.
Ø     Stage – II Neural tube formation and initial formation of
the oropharynx.
Ø   Stage – II Origins, migration and interactions of cell
populations, especially neural crest cells and their
derivatives.
Ø    Stage – IV formation of organ systems, especially the
pharyngeal arches and the primary and secondary
palates, and
Ø     Stage – V Final differentiation of tissues (skeletal,
muscular, and nervous elements).

EMBRYOLOGY OF FACE, NOSE AND PALATE

Ø  After the formation of the head fold, the developing brain
and the pericardium form two prominent bulging on the
ventral aspect of the embryo.
Ø   These bulging are separated by the stomatodaeum.
Ø
The floor of the stomatodaeum is formed by the
    buccopharyngeal membrane, which separates it from the
foregut.
Ø Soon, mesoderm covering the developing forebrain
proliferates, and forms a downward projection frontonasal
process that overlaps the upper part of the stomatodaeum.


Ø      The pharyngeal arches are laid down in the lateral and
ventral walls of the cranial most part of the foregut. These
are also, therefore, in very close relationship to the
stomatodaeum.
Ø

It will now be readily appreciated that the face is derived
from the following structures that lie around the
stomatodaeum:
(a)   The frontonasal process and
(b)   The first pharyngeal (or mandibular) arch of each side.

Fig.1-Head end of the embryo just before formation of the frontonasal
process

     At this stage each mandibular arch forms the lateral wall of th
stomatodaeum.
      This arch gives off a bud from its dorsal end called th
maxillary process.
      It grows ventro-medially cranial to the main part of the arc
which is now called the mandibular process.

      The ectoderm overlaying the frontonasal process soon shows
bilateral localized thickenings that are situated a little above the
stomatodaeum called the nasal placodes. These placodes soon sink
below the surface to form nasal pits.
     The pits are continuous below with the stomatodaeum.
     The edges of each pit are raised above the surface; the medial
raised edge is called the medial nasal process and the lateral edge is
called the lateral nasal process.


Development of the face
We are now in a position to study the formation of the various parts
of the face.


Lower lip
      The mandibular processes of the two sides grow towards
each      other and fuse in the midline.
     They now form the lower margin of the stomatodaeum.
     The mouth develops from the stomatodaeum
      The fused mandibular processes give rise to the lower lip,
and    the lower jaw.


Upper lip
     Each maxillary process now grows medially and fuses, first
with the lateral nasal process, and then with the medial
nasal     process.
     The medial and lateral nasal processes also fuse with each
     other.
      In this way the nasal pits (now called anterior nares) are
cut      off from the stomatodaeum.
     The maxillary processes undergo considerable growth.
     The frontonasal process becomes much narrower from side
     to side, so that the two anterior nares come closer together.


     The stomatodaeum is now bounded above by the upper
lip which is derived as follows:

(i)    The mesodermal basis of the lateral part of the lip is
formed  the maxillary process.
(ii)    The overlaying skin is derived from ectoderm covering
this process.
(iii)   The mesodermal basis of the lip (called philtrum) is
formed
from the frontonasal process.
(iv) The ectoderm of the maxillary process, however,
overgrows this mesoderm to meet that of the opposite
maxillary process in the midline.
(v)     As a result, the skin of the entire upper lip is
innervated by
the maxillary nerves.

 The muscles of the face (including the lips) are derived from
mesoderm of the second branchial arch and are, therefore,
supplied by the facial nerve.


Nose
      The anterior nares are formed when the nasal pits are cut
off from the stomatodaeum by the fusion of the maxillary
process with the medial nasal process.
      The anterior nares gradually approach each other.
     This is a result of the fact that the frontonasal process
becomes progressively narrower and its deeper part
ultimately forms the nasal septum.
     Mesoderm becomes heaped up in the median plane to form
the prominence of the nose. Simultaneously, a groove
appears between the region of the nose and the bulging
forebrain (which may now be called forehead).
    As the nose becomes prominent the anterior nares come to
open downwards instead of forwards. The external form of
the nose is thus established.

Cheeks
   After formation of the upper and lower lips the
stomatodaeum (which can now be called the mouth) is very
broad.
      In its lateral part, it is bound above by the maxillary
process
and below by the mandibular process.
     These processes undergo progressive fusion with each other
to form the cheeks.
      During the formation of upper lip the maxillary process
fuses with the lateral nasal process.


This fusion occurs, not only in the region of the lip, but also
extends from the stomatodaeum to the medial angle of the
developing eye.
      For some time this line of fusion is marked by a groove
called the naso-optic furrow or nasolacrimal sulcus.
    A strip of ectoderm becomes buried along this furrow and
gives rise to the nasolacrimal duct.



Development anomalies of the face
It has been that the formation of various parts of the face
involves fusion of diverse components. This fusion is occasionally
incomplete and gives rise to various anomalies.


Harelip

The upper lip of the hare normally has a cleft. Hence the
term hare-lip is used for clefts of the lips.
 When one or both maxillary processes do not fuse with the
medial nasal process, this gives rise to defects in the upper lip.
These may vary in degree and may be unilateral or bilateral.
 Defective development of the lowermost part of the
frontonasal process may give rise to a midline defect of the
upper lip.
 When the two mandibular processes do not fuse with each
other the lower lip shows a defect in the midline. The defect
usually extends into the jaw.


Oblique facial cleft
 Non-fusion of the maxillary and lateral nasal processes
gives rise to cleft running from the medial angle of the eye to
the mouth.
 The nasolacrimal duct is not formed.


INTRODUCTION
Cleft lip and palate is one of the most common congenital
anomalies afflicting humans. The potential problems that may
beast the unfortunate victims of the condition include such
severe handicaps as impaired suckling and resultant failure to
thrive, speech impediment, deafness, malocclusion, gross facial
deformity, and severe psychological problems.


The clefting of lip and/or palate occurs at such a strategic place
in the orofacial region and at such a crucial time (before birth)
that it becomes a complex congenital deformity and a major
therapeutic challenge incapable of resolution by any individual
clinician and it warrants the co-ordinated skills of many modern
specialists for its correction. Plastic surgeons, pediatricians,
orthodontists,
prosthodontists,
speech
pathologists,
otolaryngologists, anesthesiologists, and audiologists form a less
than comprehensive list of those who may be required to provide
a high degree of interdisciplinary communication and
understanding in the treatment of cleft lip and palate
deformities.

The abundance of published material, however, indicates a
remarkable lack of consensus in the treatment of these
deformities. Each multidisciplinary team develops its own
protocol for management of children with cleft lip and palate
and those ideas are held tenaciously. What is the effect of
surgery as a factor in facial growth. As contrasted with the effect
of the inherent growth disturbance associated with the
deformity? What is the optimal timing for surgical procedures?
By what standards can results be judged? Is there value in using
different methods of investigation? These questions have been
much discussed over the years and still await resolution.


One reason for lack of consensus is the apparent inability to
design and complete adequate prospective studies that produce
unequivocal results and enable comparison with the work of
others. The reasons for this failure are apparent. Adequate
studies take years- almost the professional lifetime of any one
clinician. Often patients move and are lost to follow-up.
Professionals move, and others take their place and introduce
changes in the original protocol. When a research design must
be applied to a complex biological problem, the number of
variables may take meaningful standardization almost
impossible.


HISTORY
In ancient times many congenital deformities including cleft
lip and palate, were considered to be an evidence of evil spirit in
the afflicted child. These children were often removed from the
tribe or cultural unit and left to die in the surrounding
wilderness.


The repair of cleft lip is noted in an ancient monograph during
the Chin Dynasty (255-206 BC) and is the earliest report of such
surgery any where in the world. Jehan Yipperman, a Flemish
surgeon (1295-1351), LeMonnier, a French dentist (1753)
described in detail the repair of a cleft lip and palate patient. In
the United States, Josiah Flagg, surgeon- dentist, advertised in a
handbill in Boston in 1786, ………. that among the many other
things he does, he also sews up hare lip.
The first cleft palate repair in the United States was performed
by Stevens in 1827. Fauchard, in 1786, made valuable
contributions and innovation in prosthodontics, particularly in
the area of obturators for cleft lip and palate patients.

Two men stand out for their early contribution to the
orthodontic and prosthodontic treatment of these deformities.
Kingsley who shares with others the claim to be the Father of
Orthodontia has written over 100 articles on cleft palate
rehabilitation, the inadequacies of cleft palate surgery,
obturators, orthodontic diagnosis and orthodontic appliances
bear his name. His first book, A treatise on Oral Deformities, as
a Branch of Mechanical Surgery, in 1880, featured the
orthodontic appliances of the period, including jack screws,
retainers and arches with ligatures. Calvin Case published A
Prosthetic Treatise on the Techniques and Principles of Dental
Orthopedia and Prosthetic Correction of the Cleft Palate in
1921. The appliances described were all of the fixed type.

It was only a few years ago that orthodontists demonstrated
that palatal surgery interfered with maxillary growth. Surgeons
were asked not to repair the palate until maxillary growth was
complete so as to avoid midfacial deformity. At the same time
speech and language pathologists were requesting for early
operation on palate to prevent the development of compensatory
mechanisms of articulations.


TTaking note of the suggestions made by both orthodontists and
speech pathologists the surgeons have modified their techniques,
the timing of surgery, and the number of palatal procedures that
were performed. Along with surgical refinements, advances have
been made in orthodontics and other fields concerned with
rehabilitation of cleft lip and palate to such an extent that, the
treatment of children with clefts have resulted in a cleft
population that is distinctly different from the facially crippled
stereo-types of previous generations.


INCIDENCE OF CLEFT LIP AND PALATE
Incidence of major malformations in total births at 26 large
hospitals from big cities and towns from different parts of the
country is estimated to be 14.82 per 1000 births (2695 total
malformations from amongst 1,81,798 births). Of these
malformations, the incidence of CL+P was 1.25 and that of cleft
palate alone was 0.46 per 1000 births. In other parts of the
world, the incidence of this anomaly is reported to be the highest
in Afghans as 4.9 and lowest in the Negroid population as 0.4 per
1000 live births.


EMBRYOLOGY OF CLEFT LIP AND
PALATE
Ralph Millard Jr. once said Though various theories have
been put forth to enlighten the panorama of the embryological
basis, no theory enjoys universal acceptance as no one has been
an eye witness to the entire 'in utero show' as yet and thus it is
impossible to propose and prove what exactly is happening.


The theory that separate facial processes fuse to form the mid
portion of the face was popularized by Wilthelm His. It was
Richard B Stark of St. Luke Hospital, New York, in 1954, who
wrote extensively explaining mesodermal migration.


There are five principal stages in craniofacial development:Stage – I
Germ layer formation and initial organization of craniofacial
structures.
Stage – II
Neural tube formation and initial formation of the
oropharynx.


Stage – III
Origins, migration and interactions of cell populations,
especially neural crest cells and their derivatives.
Stage – IV
Formation of organ systems, especially the pharyngeal
arches and the primary and secondary palates, and
Stage – V
Final differentiation of tissues (skeletal, muscular, and
nervous elements).


EMBRYOLOGY OF FACE, NOSE AND
PALATE
After the formation of the head fold, the developing brain
and the pericardium form two prominent bulgings on the
ventral aspect of the embryo. These bulgings are separated by
the stometodaeum. The floor of the stomatodaeum is formed by
the buccopharyngeal membrane, which separates it from the
foregut. Soon, mesoderm covering the developing forebrain
proliferates, and forms a downward projection that overlaps the
upper part of the stomatodaeum.


This downward projection is called the frontonasal process. The
pharyngeal arches are laid down in the lateral and ventral walls
of the cranialmost part of the foregut. These are also, therefore,
in very close relationship to the stomatodaeum. It will now be
readily appreciated that the face is derived from the following
structures that lie around the stomatodaeum:
 the frontonasal process and
 the first pharyngeal (or mandibular) arch of each side.


At this stage each mandibular arch forms the lateral wall of the
stomatodaeum. This arch gives off a bud from its dorsal end.
This bud is called the maxillary process. It grows ventromedially cranial to the main part of the arch which is now called
the mandibular process.


The ectoderm overlaying the frontonasal process soon shows
bilateral localized thickenings, that are situated a little above the
stomatadaeum. These are called the nasal placodes. These
placodes soon sink below the surface to form nasal pits. The pits
are continuous below with the stomatodaeum. The edges of each
pit are raised above the surface; the medial raised edge is called
the medial nasal process and the lateral edge is called the lateral
nasal process.
We are now in a position to study the formation of the various
parts of the face.


Lower lip
The mandibular processes of the two sides grow towards
each other and fuse in the midline. They now form the lower
margin of the stomatodaeum. If it is remembered that the mouth
develops from the stomatodaeum. It will be readily understood
that the fused mandibular processes give rise to the lower lip,
and the lower jaw.


Upper lip
 Each maxillary process now grows medially and fuses,
first with the lateral nasal process, and then with the
medial nasal process. The medial and lateral nasal
processes also fuse with each other. In this way the nasal
pits (now called anterior nares) are cut off from the
stomatodaeum.
 The maxillary processes undergo considerable growth. At
the same time the frontonasal process becomes much
narrower from side to side, so that the two anterior nares
come closer together.

 The stomayodaeum is now bounded above by the upper
lip which is derived as follows:
♦

The mesodermal basis of the lateral part of the lip
is formed the maxillary process. The overlaying
skin is
derived from ectoderm covering this
process.
♦

The mesodermal basis of the lip (called philtrum)
is formed from the frontonasal process. The
ectoderm of the maxillary process, however, overgrows
this mesoderm to meet that of the opposite maxillary
process
in the midline. As a result, the skin of the

TThe muscles of the face (including the lips) are derived from
mesoderm of the second branchial arch and are, therefore,
supplied by the facial nerve.


Nose
We have seen that the anterior nares are formed when the
nasal pits are cut off from the stomatodaeum by the fusion of the
maxillary process with the medial nasal process. We have also
noted that the anterior nares gradually approach each other.
This is a result of the fact that the frontonasal process becomes
progressively narrower and its deeper part ultimately forms the
nasal septum. Mesoderm becomes heaped up in the median
plane to form the prominence of the nose. Simultaneously, a
groove appears between the region of the nose and the bulging
forebrain (which may now be called forehead). As the nose
becomes prominent the anterior nares come to open downwards
instead of forwards. The external form of the nose is thus
established.

Cheeks
After formation of the upper and lower lips the
stomatodaeum (which can now be called the mouth) is very
broad. In its lateral part, it is bound above by the maxillary
process and below by the mandibular process. These processes
undergo progressive fusion with each other to form the cheeks.


We have already seen that (during the formation of upper lip)
the maxillary process fuses with the lateral nasal process. This
fusion occurs, not only in the region of the lip, but also extends
from the stomatodaeum to the medial angle of the developing
eye. For some time this line of fusion is marked by a groove
called the naso-optic furrow or nasolacrimal sulcus. A strip of
ectoderm becomes buried along this furrow and gives rise to the
nasolacrimal duct.


Development anomalies of the face
It has been that the formation of various parts of the face
involves fusion of diverse components. This fusion is
occasionally incomplete and gives rise to various anomalies.


Hare-lip:
The upper lip of the hare normally has a
cleft. Hence the term hare-lip is used for clefts of the lips.
♦
When one or both maxillary processes do not fuse
with the medial nasal process, this gives rise to defects in
the upper lip. These may vary in degree and may be
unilateral or bilateral.
♦
Detective development of the lowermost part of
the frontonasal process may give rise to a midline defect
of the upper lip.
♦
When the two mandibular processes do not fuse
with each other the lower lip shows a defect in the
midline.
The defect usually extends into the jaw.



Oblique facial cleft: Non-fusion of the maxillary
and lateral nasal processes gives rise to cleft
running from the medial angle of the eye to the mouth.
The nasolacrimal duct is not formed.


DEVELOPMENT OF NASAL CAVITIES
The nasal cavities are formed by extension of the nasal pits.
We have seen that these pits are at first in open communication
with the stomatodaeum. Soon the medial and lateral nasal
processes fuse, and from a partition between the pit and the
stomatodaeum. This is called the primitive palate, and is derived
from the frontonasal process.


The nasal pits now deepen to from the nasal sacs which expand
both dorsally and caudally. The dorsal part of the sac is, at first,
separated from the stomatodaeum by a thin membrane called
the bucconasal membrane (or nasal fin). This soon breaks down.
The nasal sac now has a ventral orifice that opens on the face
(anterior nares), and a dorsal orifice that opens into the
stomatodaeum (primitive posterior nares).


The two nasal sacs are at first widely separated form one
another by the frontonasal process. We have seen, however, that
the frontonasal process becomes progressively narrower. This
narrowing of the frontonasal process, and the enlargement of
the nasal cavities themselves, brings them closer together. The
intervening tissue becomes much thinned to from the nasal
septum. The ventral part of the nasal septum is attached below
to the primitive palate. More posteriorly, the septum is at first
attached to the bucconasal membrane, but on disappearance of
this membrane it has a free lower edge. The nasal cavities are
separated from the mouth by the development of the palate, as
described below.

The lateral wall of the nose is derived on each side from the
lateral nasal process. The nasal conchae appear as elevations on
the lateral wall of each nasal cavity. The original olfactory
placodes form the olfactory epithelium that lies in the roof and
adjoining parts of the walls, of the nasal cavity.


Development of the palate
To understand the development of the palate let us have
another look at the maxillary processes. From ------- it will be
seen that these processes not only from the upper lip but also
extend backwards on either side of the stomatodaeum. They can,
therefore, be diagrammatically illustrated as in fig…. If we cut a
coronal section through the region (along the line X-Y) the
maxillary processes will be seen as in……… Finally, if we now
correlate …. with …… , the relationship of the maxillary
processes to, the developing nasal cavity and mouth is easily
understood.

From each maxillary process a palate like shelf grows medially.
This is called the palatal process. We now have three
components from which the palate will be formed. These are: (a)
the two palatal processes and (b) the primitive palate formed
from the frontonasal process. The definitive palate is formed by
the fusion of these three parts as follows: (i) Each palatal process
fuses with the posterior margin of the primitive palate. (ii) The
two palatal processes fuse with each other in midline. Their
fusion begins anteriorly and proceeds backwards. (iii) The
medial edges of the palatal processes fuse with the free lower
edge of the nasal septum, thus separating the two nasal cavities
from each other, and from the mouth.

At a later stage the mesoderm in the palate undergoes
intramembranous ossification to form the hard palate. However,
ossification does not extend into the posterior-most portion,
which remains as the soft palate. The part of the palate derived
from the frontonasal process the premaxilla, which carries the
incisor teeth.


Cleft palate
Defective fusion of the various components of the palate
gives rise to clefts in the palate. These vary considerably in
degree as illustrated. Clefts on the palate that extend to its
anterior end are associated with cleft-lip, as both the upper lip
and the palate are formed by fusion of the maxillary processes
with the frontonasal process. Clefts of the palate result in
communications between the mouth and the nose. These may be
unilateral, or bilateral.


ETIOLOGICAL FACTORS IN CLEFT LIP
AND PALATE
Following are the probable etiological factors causing cleft lip
and palate:








Monogenic or single gene disorder
Polygenic or multifactorial inheritance
Teratogenic
Chromosomal abnormalities
Familial
Heterogeneity
Sex predominance

Monogenic or Single Gene Disorders
Approximately half of the recognized syndromes associated with
cleft lip and palate are due to single gene disorders with equal
distribution autosomal dominant and autosomal recessive.
Single gene defects may give rise to Mendelian patterns of
inheritance, either of isolated cleft lip (palate) or in multiple
malformations associated with cleft lip with or without cleft
palate.


Teratogenic Drugs
Anticonvulsants (a) Diphenylhydantoin: CL (CP) is part of
the fetal hydantoin syndrome related to the drug and its
analogues.
The association between diazepam and cleft lip (palate) has
also been reported.
Material use of alcohol is potentially teratogenic.
Rubella in the first few weeks of gestation is associated
with clefting.

Chromosomal Abnormalities
Chromosomal abnormalities account for 18% of the clefting
syndromes and would invariably be associated with other
malformations. Chromosomal abnormalities notably trisomy D
and also less frequently trisomy E, may cause multiple
malformations including CL (P). There are multiple deletions
and translocations where individual case reports included
clefting. Clefting syndromes are three times more frequent in
Down's syndrome.


Familial
Familial aggregation has been observed in CL (P). Thus it is
inferred that someone with a relative affected by one of these
disorders is more likely to be affected than if that person had no
affected relatives.
The relatives of the index patient shares genes in common in
direct proportion to the closeness of their relationship.
According to the multifactorial model, the relatives will share
some of the disease predisposing genes and hence will be shifted
towards the threshold for disease.

Analysis of family studies indicate degrees of susceptibility.
Fogh-Anderson's family studies shows that siblings of patients
with cleft lip had increased frequency of cleft lip and cleft palate,
but no increased frequency of cleft palate alone. Siblings of
patients with cleft palate had increased frequency of cleft palate,
but not CL and CP.
A cleft uvula, submucous cleft of the palate, and velopharyngeal
insufficiency (microforms of clefts) when noted in a parent, is a
guideline in discerning autosomal dominant varieties of
genetically heterogenous clefting syndromes.


Of first degree relatives, the incidence in the sibling of index
cases of CL (P) is approximately 40 times the incidence in the
general population. The incidence in 2nd degree relatives is
approximately seven times and that of the 3rd degree relatives is
three times that of general.


Heterogeneity
Genetic heterogeneity means what appears first to be one
disease turns out to be many diseases with different genetic and
non-genetic etiologics. Thus differing genotypes and/or
environmental causes may produce a single or similar
phenotypes.


Sex Predominance
More males are born with cleft lip and cleft palate than
females and more females than males have cleft palate alone.


CLASSIFICATION
Any disease or anomaly has to be defined and classified for
the sake of understanding and treating the anomaly.
Classification also as a language for communicating with other
professionals colleagues.
Symbolic representations of cleft lip and palate anomalies
serve the clinician as a convenient short hand method for
recording conditions. Surgeons, in particular, find it appealing
to see at a visual summary of anatomic malformations,
particularly congenital anomalies.

Numerous classifications have been proposed by various
authors. Each has its own advantages and disadvantages. Only
those classification systems which have found wide clinical
acceptance will be described. They are:






Davis and Ritchie classification (1922)
Veau's classification (1931)
Keranhan's striped 'Y' classification (1971)
Spina's classification (1974)
Millard's classification (1977).


Davis & Ritchie Classification (1922)
They classified the congenital clefts into three groups
according to the position of the cleft in relation to the alveolar
process.
Group – I
Prealveolar clefts:
♦
Unilateral
♦
Median
♦
Bilateral

Group – II
Postalveolar clefts:
♦
Soft palate only
♦
Soft & hard palate
♦
Submucous cleft
Group – III
Alveolar clefts:
♦
Unilateral
♦
Bilateral
♦
Median.


Veau's Classification (1931)
He divided clefts into four groups:
Group – I
Cleft of soft palate only.
Group – II
Cleft of the hard and soft palate extending no further than
the incisive foramen, thus involving the secondary palate alone.


Group – III
Complete unilateral cleft, extending from the uvula to
incisive foramen in the midline, then deviating to one side and
usually extending through the alveolus at the position of the
future lateral incisor tooth.


Group – IV
Complete bilateral cleft, resembling group –III, with two
clefts extending forward from the incisive foramen through the
alveolus. When both clefts involve the alveolus, the small
anterior element of the palate commonly referred to as the
premaxilla, remains suspended from the nasal septum.


Kernahan's Striped 'Y' Classification (1971)
In this classification incisive foramen is the reference point
and it is based on embryology rather than morphology.
The two arms of the stripes Y Logo are each divided into
three sections, representing the lip, the alveolus, and the hard
palate as far back as the incisive foramen. The stem of Y is also
divided into three parts, representing varying degrees of clefting
of the hard and soft palates. Over clefting is represented by
stippling and submucous clefting by cross-hatching.

The advantage of the diagrammatic representation of clefting is
that it gives as immediate and readily recognized account of the
original extent of the cleft at any time in the long progress of the
patient's treatment.


Spina's Classification (1974)
He divided clefts into four groups:
Group – I
Preincisive foramen clefts (clefts lying anterior to the incisive
foramen). Clefts of the lip with or without an alveolar cleft.


 Unilateral:
♦ Right or left: Total when they reach the alveolar
arcade or partial.
 Bilateral:
♦ Total:
(On one or both sides).
♦ Partial
 Median:
♦ Total
♦ Partial


Group – II
Transincisive foramen clefts. (clefts of the lip, alveolus and
palate).
Unilateral :
 Bilateral

Right or Left.


Group – III
Postinisive foramen clefts
 Total
 Partial
Group – IV
Rare facial clefts.


Millard's Classification (1977)
Millard's symbolic representation is basically a modification
of Kernahan's striped 'Y' classification. He has added inverted
triangles atop the upright triangular segment which in turn
stand on the 'Y' proposed by Kernahan. The inverted triangles
represent the nasal arch and the upright triangles represent the
nasal floor.


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