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Abstract
Cleft lip and palate represent one of the great challenges of craniofacial surgery, with initial descriptions of the
condition and surgical repair dating back to ancient times. Despite many diagnostic and technical aspects remaining
unqualified, much progress has been achieved in understanding and treating this deformity. From more complex
genetic studies clarifying its etiology to less mutilating surgical techniques, these advances have helped improve
prevention and appropriate care. A protocol for the treatment of cleft patients should followed by the healthcare
providers, and coordination amongst them is a major contributor to success in cleft treatment.
INTRODUCTION History
Cleft lip with or without cleft palate [CL(P)] is the The first recorded operative treatment of a cleft
most common congenital malformation in the head and patient has been attributed to the period of the Chin
neck. The birth of a child with a cleft is a difficult and (Tsin) Dynasty (c390 AD). The repair was of a cleft lip
emotionally charged time for the child's family. [1] only, and no mention of cleft palate repair was made.
Evaluation and treatment of the child with cleft lip Palatal clefts were confused with the more common
and/or cleft palate requires a long-term comprehensive fistulas resulting from tertiary syphilis and were not
and multidisciplinary approach in terms of medical, addressed surgically because of this association. [3]The
surgical, dental, and psychological intervention best greater technical challenges of cleft palate repair no
accomplished in conduction with a cleft palate team.[2] doubt presented a barrier to surgical treatment as well.
Although the first known cleft palate repair was
performed in the early 19th century, the introduction of
anesthesia permitted a quantum leap in treatment as it
did for many diseases.[4]John Stephenson (1797–1842),
a physician who was born with incomplete cleft palate,
wrote the earliest recorded description of palatoplasty.
As a medical student in Edinburgh, he traveled to Paris
to observe the renowned surgeon, Philibert Roux
(1780–1854). While he was there, Roux noted his
abnormal speech pattern; he performed the primary
repair of his velum when Stephenson was 22 years old.
In his thesis, Stephenson wrote in remarkable detail of
his speech quality: I always pronounced /th/ like /s/. As
I grew up, the adjacent parts tended to close the defect,
and by speaking slowly I articulated better. [5]Thanks to
Fig-1: Orofacial Clefts the nasal quality of the language, I used to speak French
more clearly than English. Nature is kind and trying to
Embryologic Considerations and Classification The first phase involves proliferation of the
Normal embryologic development of the lip and mesoderm and ectoderm in the frontonasal process. The
palate can be considered to occur in two related phases: frontonasal process has three components: (a) an
the first phase (beginning at 4 to 5 weeks' gestation), anterior labial component, which forms the philtrum;
involving the development of the upper lip, nose, and (b) an anterior palatal component forming the alveolar
primary palate or premaxilla (the portion of the bony part of the premaxilla (with the central and lateral upper
palate anterior to the incisive foramen containing the incisors); and (c) a posterior palatal component forming
four upper incisors), and the second phase (beginning at the portion of the hard palate anterior to the incisive
8 to 9 weeks' gestation), involving the development of foramen. Laterally, proliferation of mesoderm with
the secondary palate (the hard and soft palate posterior overlying ectoderm occurs in the maxillary processes
to the incisive foramen).[14,16,17,18] that eventually form the lateral lip segments and nasal
alae.[1,2,13,14,16,18]
Embryonic formation of the frontonasal process begins septum).[18] A complete cleft palate refers to a cleft of
with differentiation of the olfactory placode epithelium both the primary and secondary palates and is nearly
.[2] Morphogenetic movement of the placode and always associated with a cleft lip. [18,19] The term
differential growth forms the characteristic curl of the incomplete cleft palate is synonymous with a cleft of
placode into the nasal alae.[1] The most recent theory of the secondary palate or can be used to describe a palatal
palatal formation involves contact, with subsequent cleft with an area of intact mucosa.[20] The classic
resorption of the contacting surface epithelial cells and submucous cleft palate (bifid uvula, midline diastasis of
adhesion of the contacting prominence. It is believed the levator muscles, and posterior hard palate notching
that this occurs in both primary and secondary palate caused by loss of the posterior nasal spine) is accurately
formation (i.e., contact, loss of surface epithelial cells, a microform expression of a cleft of the secondary
and mesodermal contact, with fusion and penetration palate.[21,22,23]
across the junction).[18] Differences in the gestational
time of development and fusion of the primary palate General Team Management Approach
(about 30 days) and secondary palate (about 50 days) Because the problems that confront affected children
are reflected in the separate genetic inheritance and their parents are complex, variable, and long-term,
patterns.[1, 2, 18] the facial surgeon should be allied with a cleft palate or
craniofacial team that meets regularly.[1,2][ Fig.5]This
Clefts of the lip are either unilateral right or left, or allows valuable consultation with colleagues in
bilateral (group I).[13-16] They can be complete (with pediatrics, plastic surgery, dentistry, orthodontics,
extension into the nasal floor) or incomplete (extending speech pathology, and audiology on a regular basis.
from a slight muscle diastasis at the vermilion to a small Other experts (e.g., neurosurgery, ophthalmology, and
bridge of tissue at the nasal sill). [13] A cleft involving prosthodontics clinicians) can be consulted, as needed,
only the lip can occur as an isolated entity, but a cleft of as well as oral surgeons, geneticists, nurses, and social
the alveolus is always associated with a cleft of the workers.[24] The team approach functions best in an
lip.[14,17,18] Following the birth of a child with a cleft, the parents
often react with disappointment and anger, followed by
Group III includes children with a cleft lip and depression, then guilt. Initial counseling is important
palate. Clefts of the palate can be divided into primary and should be scheduled as soon as possible with a
(involvement anterior to the incisive foramen, group healthcare professional skilled in counseling parents of
IV) or secondary (involvement posterior to the incisive children with craniofacial anomalies. [26] Care and
foramen, group II).[17,18] Palatal clefts also can be feeding in the first months of life should be
unilateral (the palatal process of one side is fused with demonstrated, and a general outline for the child's long-
the septum, resulting in communication of the oral and term care should be offered.[27,28] [Fig.6]
nasal cavities on one side only) or bilateral (no
connection between either palatal process and the
Cleft Palate
The deficiencies associated with cleft palate depend
on the location of the defect in the palate. In the normal
palate, the tensor veli palatini and levator palatini
muscles within the soft palate insert into an aponeurosis
at the midline raphe.[14,36] In the cleft palate, the muscle
fibers follow the medial margin of the cleft and insert
into the medial cleft edges and the posterior edge of the
lateral bony hard palate. Clefts involving the alveolus
Figure -7: Nasoalveolar molding (NAM) appliance. can disrupt normal dental development, eruption, and
Hard plate facilitates nipple compression. retention.[37,38]
traditionally treated by several specialists, are involved. and/or osseointegrated implants. It is our experience
In the oral cavity, the cleft affects not only the soft and that patients treated within the context of the
hard palate, but also the alveolus and dentition. [30,41] The multidisciplinary approach can obtain excellent
structural rehabilitation of these patients requires the outcomes related to speech, ideal occlusion, satisfactory
surgical correction of the soft- and hard-tissue defects lip aesthetics, and skeletal balance . [43]However, it is
as well asthe secondary effects of the cleft on maxillary the secondary cleft nasal deformity that still gives the
development, dental support, and dental–occlusal patient the “cleft stigmata.” [30,42] In recent years, new
alignment.[1,2,13,15] [Fig.8] The role of the orthodontist in orthodontic and surgical treatment modalities have
cleft management is essential as the orthodontist assists become available that may further improve outcomes in
the surgeon during all stages of reconstructive care: in patients with orofacial clefts. In infancy, this includes
the early stages, with presurgical nasal and maxillary the use of presurgical nasoalveolar molding techniques.
orthopedics; during the transitional dentition stage, with In the mixed dentition, novel orthodontic–orthopedic
alignment of the maxillary segments and dentition in approaches to correct maxillary hypoplasia are utilized;
preparation for secondary alveolar bone grafting; and and, in the permanent dentition, the use of new
during the permanent dentition and late adolescent appliances and dental materials to facilitate orthodontic
years, by obtaining satisfactory dental and occlusal treatment and the application of bone anchorage screws
relationships and also to prepare the dentition for (BAS) to facilitate orthodontic tooth movement are
prosthetic rehabilitation and orthognathic surgery, if employed.[43] In addition, the use of distraction
required. In addition, it has been the role of the osteogenesis to improve the position of the maxilla in
orthodontist to monitor craniofacial growth and dental those cases with severe maxillary hypoplasia has
development, as well as the treatment effects on these become a well-accepted procedures.[44] Finally the
patients through the use of roentgencephalometry. [18] availability of new diagnostic techniques such as digital
With this approach, the management of the cleft patient skull and dental models, three-dimensional (3D)
has evolved dramatically in recent years. [29,30,42]The photogrammetry, lower radiation computed tomography
reason for improved outcomes is based on refinements (CT) scans, cone beam CT (CBCT), and the
in primary and finishing surgical techniques, as well as development of 3D digital protocols to plan
timing and incorporation of other procedures such as orthognathic surgery are now at the forefront of current
presurgical orthopedics, orthodontics, and new orthodontic
prosthetic approaches utilizing resin-bonded prosthesis
and surgical approaches. The efforts towards Orthodontic treatment can be continued 8–12 weeks
improvement of orthodontic and surgical treatment after bone graft surgery. As soon as appropriate
strategies developed for noncleft patients will benefit maxillary arch and dental relations are achieved, the
orthodontic appliances are removed and the patient is
the challenging problems presented by cleft patients and
placed in retention until there is full permanent
are a welcome addition to the current treatment dentition. [1,2]Teeth that were severely rotated prior to
protocols.[28,30,45] [Fig.8] treatment need to be retained. [13]Absent teeth can be
@ 2021 | PUBLISHED BY GLOBAL JOURNAL OF RESEARCH PUBLICATION, INDIA 27
Global J Res Dent Sci. 2021; 1(1), 21-32
temporarily replaced with a removable prosthetic achievable goal to eliminate the need for artificial
appliance to improve aesthetics and limit the effects on replacement teeth .[1,2] In these cases in which space
speech production. [46] Patients treated with the protocol closure is not possible, the use of adhesive
outlined above complete the preparatory phase of bridgeworksor of implants in the grafted alveolar ridge
orthodontic treatment in the preteen or early teen years. has become a treatment of choice. A further possibility
Patients are followed every 6 months to determine their is the transplantation of a lower premolar to the upper
craniofacial growth and dental development, especially arch. [13-16] [Fig.12]
eruption of the maxillary lateral incisor and canine on
the cleft side. [15]Occasionally, the maxillary canine is The development of effective orthognathic surgical
impacted and requires surgical exposure and techniques in the 1970s and 1980s has provided
orthodontic incorporation into the arch as the child is in orthodontics with the means to complete treatment of
the full permanent dentition.[47] Impacted or severely almost all cleft patients.[1,2] The use of three-
malpositioned cleft-side maxillary lateral incisors are dimensional cephalometry, computed tomography, and
usually extracted .[47,48] scanned dental models, video imaging, and computer-
generated images have all contributed to the
Patients with CLP usually require an extensive and improvement of orthognathic surgery planning. [25-
30]
prolonged orthodontic treatment parallel to the surgical Although initially developed for non-cleft
treatment. Orthodontic treatment may be required: (a) in orthognathic surgery, the use of these applications in
infancy, before initial surgical repair of the lip, (b) cleft patients has been increased rapidly.[1,2,27,29,30]
during the primary dentition period, (b) during the
mixed dentition period, (c) during the permanent Finally, if it is determined that there is
dentition period and (d) in the late teens after anteroposterior skeletal disharmony, the reconstructive
completion of facial growth, in conjunction with team has to decide if it is convenient to do the bone
orthognathic surgery.[1,2,13,15] [Fig.9] grafting in the transitional dentition or if it should be
done in combination with future orthognathic surgical
The introduction of passive realignment of the hard procedures.[1,2] Patients in whom there is marked tissue
palate shelves has been introduced by McNeil and later deficiency, including maxillary hypoplasia and
by Burston[26,27]. This orthopedic approach makes CLP congenitally missing teeth, are likely candidates for
repair easier and may improve the aesthetic outcome of postponement of the traditional approach for secondary
primary CL nasal repair by repositioning the alar alveolar bone grafting and will be treated later on in the
base.[28] However, unless the appliances used are permanent dentition in combination with orthognathic
continued throughout the period of facial growth, their surgery.[13-16] If it is deemed important to preserve the
long-term influence on facial growth and dentition dentition adjacent to the alveolar cleft, orthodontics are
remains still a matter of discussion.[29,30] therefore indicated, even in the presence of a skeletal
disharmony[49]. The purpose of the orthodontic
Orthodontic intervention in the primary dentition treatment is then to prepare the dentition for the alveolar
has been recommended over the past 60 years, although bone graft and also to coordinate the maxillary arch to
less in recent years.[30]Suggested treatment at that time the mandibular arch for future orthognathic surgery that
ranged from full bandingto routine arch will be performed in the teen years. This approach
expansion[1`,2,29,30] [Fig.10] minimizes the required orthodontic treatment prior to
orthognathic surgery in the adolescent years.[47,50]
Numerous authors have described the beneficial [Fig.13]
effects on dental and skeletal growth development of
cleft patients through the elimination of functional and
structural problems at this developmental stage.
[1,2,16]
The most common procedures for this purpose
include: (a) maxillary expansion to correct the reduced
transverse dimension, (b) incisor alignment and
proclination to remove crowding, rotations, and anterior
crossbites, as well as (c) maxillary protraction to reduce
maxillary retrusion.[28-30] [Fig.11]
Orthodontic management following the growth patterns than those seen in noncleft
developmental approach outlined previously allows the individuals.[2]
clinician to take advantage of developmental and
growth changes and permits the patient and family to However, cleft patients have significant growth
recognize the need for distinct phases of orthodontic potential. If this potential is not negatively affected by
treatment which also allow for sufficient rest space the reconstructive procedures required by the patient, it
between stages[47]. This approach assures patient and is likely that a favorable outcome will be obtained.
[1]
family acceptance, compliance, and cooperation with Orthodontic treatment will be simplified if minimal
the treatment protocol.[1,2] growth disturbances affect the patient. [2]Simplification
and shortening of orthodontic treatment, which is
Since the orthodontist participates in the care of a usually the longest therapeutic intervention for many
cleft child from infancy into adulthood, it is imperative cleft patients, are desired as this will decrease the
to recognize that abnormal facial growth will present an burden of care (e.g., patient, family, provider, public
added challenge to the reconstructive team.[48] It is health system, society). [25,26] Cleft teams
understood that cleft patients do have different facial
Figure-10: (a) Occlusal pre-treatment and (b) with the hyrax appliance in place
• Lip taping or lip adhesion, an early preliminary 11. Goldwyn RM. (1968);Johann Friedrich
option for cleft lip, can be performed at 2 to 4 Dieffenbach (1794– 1847). Plast Reconstr
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12 months of age as long as the child is (2014) The Multifactorial Factors Influencing
gaining weight and growing in a normal Cleft Lip-Literature Review. International
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starts from birth and continues into adulthood, 1:510.doi:10.4172/ scientificreports.510
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Azzaldeen (2015) Human Genetic Factors in
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