Oral and Maxillofacial surgery/Fifth year

‫ حسنين احمد‬.‫د‬

Cleft lip and palate

Cleft lip and palate (CLP) refers to a clinical spectrum of cleft lip with or
without associated cleft palate or isolated cleft palate.

Epidemiology
The estimated incidence of CLP ranges from 1:500 live births to 1: 2000
live births, the incidence is highest in Asians (1:500) followed by whites
(1:1000) while the lowest incidence is in African-Americans (1:2000), but
this racial variation is not observed in cases of isolated cleft palate (CP)
with reported incidence of 1:2000 live births.

 Isolated cleft lip (CL) is reported in 21%-25% of the cases.

 Isolated CP is reported in 33%-40% of the cases.
 CLP is reported in 35%-46%.
 Unilateral clefts are 9 times more common than bilateral clefts and
they are more frequent on the left side than on the right.
 Males are predominant in CLP population, whereas isolated CP occurs
more commonly in females.
 CLP can be associated with syndromes such as Van der Woude
syndrome, hemifacial microsomia, velocardiofacial syndrome or
DiGeorge syndrome, ectrodactyly-ectodermal dysplasia-clefting
syndrome, Stickler syndrome, trisomy 13 and trisomy 18.

Etiology
Clefts can occur as part of a syndrome or as isolated clefts, and they are
thought to be of a multifactorial etiology with a number of potential
environmental and genetic contributing factors.

The environmental factors during pregnancy that have been established

in the etiology of CLP include; smoking, alcohol, anticonvulsants,
steroids, folic acid deficiency, hypoxia, retinoids, radiation, viral
infections in addition to mechanical factors.

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Classification
Many classification systems were proposed for CLP starting in 1920s.
Clefts need to be described rather than categorized and the description of
CLP phenotypes should include:

1. The laterality (unilateral/bilateral/median) and severity (complete/

incomplete) of the labial defect.
2. Acknowledgment of an alveolar defect.
3. Morphological characterization of the palatal defect; one of the most
widely accepted classification of palatal defects is that of Victor Veau
introduced in 1931:
 Group 1; Cleft of soft palate only.
 Group 2; Cleft of hard and soft palate up to the incisive foramen,
involving secondary palate alone.
 Group 3; Complete unilateral cleft, extending from uvula to the
incisive foramen in midline, then deviating to one side and usually
extending through the alveolus at the position of the future lateral
incisor tooth.
 Group 4; Complete bilateral cleft, same as group 3 with two clefts
extending forward from the incisive foramen through the alveolus.

Prenatal diagnosis
Recent advances in ultrasound imaging have revolutionized prenatal
diagnosis of facial clefts, ultrasound images of clefts of the lip can be
visualized as early as 12-16 weeks, whereas diagnostic images of the
palate are more difficult to acquire.

Genetic counseling should be offered to parents especially in syndromic

cases. The risk of having a second child with CLP is 2-4%, this
percentage increases to about 10% when 2 previous children had CLP.
When one parent and one child had CLP the risk of having a second child
with CLP is 17%.

Clinical manifestations
Lip clefting may be microform, incomplete or complete (involving the full
vertical height of the lip). Complete cleft lips are often associated with an
alveolar cleft.

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In unilateral CL there is rotation and distortion of the vermillion with loss
of Cupid’s bow and philtral landmarks on the cleft side.

In bilateral CL, the premaxilla grows independently of the maxillae on

either side and may protrude considerably, particularly in complete
clefts.

The spectrum of CP ranges from a submucous cleft to complete clefting

of the primary and secondary palate. CP is primarily characterized by
disorientation of palatal muscles which lead to feeding difficulties,
velopharyngeal insufficiency, and speech problems.

Management
Successful management of the child born with CLP requires coordinated
care provided by a number of different specialties including oral and
maxillofacial surgery, plastic surgery, otolaryngology, genetics, speech
therapy, orthodontics, prosthodontics and others.

Specific goals of treatment include the following:

 Normalized esthetic appearance of the lip and nose

 Intact primary and secondary palate
 Normal speech, language, and hearing
 Nasal airway patency
 Class I occlusion with normal masticatory function
 Good dental and periodontal health
 Normal psychosocial development

The management of CLP patients can be divided into:

1. Preoperative management.
2. Primary operative management.
3. Secondary operative management.

Preoperative management
Feeding

One of the major concerns during this phase of management is feeding,

this concern is not very critical in children born with isolated CL, as they
can feed quite normally.

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 Infants with cleft palate, on the other hand, can have difficulty in feeding
due to the inability to form an adequate seal between the tongue and
palate for creation of sufficient negative pressure to suck fluid from a
bottle. Specialized nipples and bottles are necessary to improve feeding.

Presurgical orthopedics (PSO)

PSO entails using devices to mold the perioral structures of the infant
with a CLP to reposition the nasolabial and maxillary segments closer to
each other. It is mainly used in the first few weeks after birth and in the
months prior to palate repair. PSO treatment can be achieved by
intraoral or extraoral devices or appliances or a combination of the two,
examples of PSO are:

 Alveolar molding
 Lip strap/lip taping
 Nasoalveolar molding (NAM)

Primary operative management

Treatment planning and timing

The timing of CLP repair is controversial. The decision to surgically

manipulate the tissues of the growing child should take into account the
possible growth restriction that can occur with early surgery. Historically
the anesthetic risk-related data suggested that the safest time period for
surgery in this population of infants could be outlined simply by using
the “rule of 10’s.” This referred to the idea of delaying lip repair until the
child was at least 10 weeks old, 10 pounds in weight, and with a
minimum hemoglobin value of 10 g/dl.

Procedure Timing
CL repair After 10 weeks
CP repair 9-18 months
Pharyngeal flap or 3–5 years or later based on speech development
pharyngoplasty
Alveolar reconstruction 6–9 years based on dental development
Orthognathic surgery 14–16 years in girls, 16–18 years in boys
Rhinoplasty After age 5 years but preferably at skeletal maturity;
after orthognathic surgery when possible
Lip revision Anytime once initial remodeling and scar maturation is
complete but best performed after age 5 years

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Surgical procedures for CL
The basic premise of the repair is to create a three-layered closure of
skin, muscle, and mucosa that approximates normal tissue and excises
hypoplastic tissue at the cleft margins. Numerous techniques, as well as
modifications to popular techniques, have been extensively described in
the literature

 Lip adhesion

This procedure is carried out in an attempt to surgically approximate the

segments of the cleft lip prior to definitive lip repair to achieve a better
relationship of both the lip structures and the dental arches. It is usually
used in wide bilateral clefts. It is usually completed at 3 months of age
while definitive lip repair is 3-9 months later.

 Straight line repair

Straight line closure, or the Rose-Thompson closure is rarely used as

primary technique for cleft repair as it results in notching of the lip and
vertical scar contracture.

 Tennison-Randall triangular flap repair

It utilizes the interdigitation of triangular flaps (geometric design). The

concept underlying the technique can be similarly compared to a Z-
plasty reconstruction of the lip. This technique is based on careful
measurements of specific landmarks.

 Millard’s rotation-advancement flap repair

It is the most prevalently used technique in cleft lip repair. The technique
utilizes downward rotation of the superiorly displaced medial lip segment
with advancement of the lateral lip flap to correct the defect below the
nose. Many modifications for the original procedure were described.

Bilateral CL repair

Repair of the bilateral CL is often significantly more difficult than the

unilateral CL repair due to the presence of the premaxilla and prolabium.
Infant orthopedics, nasoalveolar molding, and taping of the lip can be
considered as an option with any of the techniques described earlier.

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Surgical procedures for CP
CP repair requires the mobilization of multilayered flaps to reconstruct
the defect in a layered fashion by first closing the nasal mucosa and then
the oral mucosa, the surgeon must also reconstruct the musculature of
the velopharyngeal mechanism. Therefore, the soft palate is closed in
three layers by approximating the nasal mucosa, velar musculature, and
the oral mucosa. The hard palate portion is closed in two layers using
nasal mucosa flaps and then oral mucosa flaps. Both the hard and soft
palate repairs must be done in a tension-free manner to avoid wound
breakdown and fistula formation.

Many techniques have been described in the literature with many

variations, each method having its theoretical advantages and limitations:

The von Langenbeck technique

It involves creating bipedicled mucoperiosteal flaps on both sides of the

cleft. The nasal side of the cleft is closed first, then the bipedicled flaps
are approximated to cover the oral surface of the cleft.

V-Y pushback technique

Many authors believed that the von Langenbeck procedure resulted in

insufficient length of the soft palate and advocated the V-Y pushback
technique. It has the advantage of lengthening the palate and
repositioning the levator muscle in a more favorable position. However,
this technique involves extensive dissection and the denuded palatal
bone from which the mucoperiosteal flaps are raised adversely affects
midfacial growth in cleft palate patients. This technique also has a higher
rate of fistula in complete cleft palate.

Two-flap technique

In this procedure a full-thickness mucoperiosteal flap is elevated on each

side of the cleft, which preserves the palatal neurovascular bundle.

The Furlow technique

It essentially consists of repairing palatal clefts using Z-plasties of the

oral and nasal mucosa. The theoretical advantage is that the soft palate
may be lengthened while preventing longitudinal scar contracture and
palatal shortening.

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Postoperative care

 Normal feeding can be resumed as soon as the infant is stable, with

the exception that active sucking is to be avoided for at least 2 weeks.
 Elbow immobilizers maybe used.
 Regular follow-up.

Complications

Immediate postoperative complications include breakdown of the repair

due to tension, palatal ischemia, secondary pressure, secondary trauma,
or bleeding. It may result in the formation of an oronasal fistula which
most commonly forms at the junction of the hard and soft palate.

Other long-term complications include midface growth deficiency,

velopharyngeal incompetence, recurrent fistula, and sleep apnea.

Secondary operative management

Alveolar bone grafting

Goals of alveolar bone reconstruction

1. To provide bone support and adequate attached gingival width for
teeth adjacent to the cleft.
2. To close the remaining oronasal fistula.
3. To improve support of the nasal alar base and lip on the affected
side(s).
4. To allow normal eruption of the permanent teeth in the cleft area and
providing sufficient bone for the placement of dental implants, where
needed.
5. To create an appropriate ridge form to allow for optimization of
orthodontic care and dental alignment.
6. To allow for stabilization of the premaxillary segment and to provide
continuity of the maxilla as a whole.
7. To improve nasal symmetry.
8. To provide support for the upper lip.

Timing of the alveolar bone reconstruction was one of the most

controversial issues. Generally it can be grouped according to timing
into:

Primary (early) grafting; which was defined as that performed

simultaneously with lip repair or as grafting performed before the palate
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is repaired at age younger than 2 years. This “early”-phase bone grafting
has been associated with reports of moderate to severe long-term
maxillary growth restriction.

Secondary (delayed) grafting; which can also be divided into:

 Early secondary; before the eruption of the permanent incisor teeth

(3-6 years). The literature does not support early secondary grafting.
 Secondary (mixed dentition); before the eruption of the maxillary
canine, (6-12 years), it can also be divided into early secondary (6-8
years) and late secondary (8-12 years). Ideally the patient is between
the ages of 8 and 12 years with a maxillary canine root that is one
half to two thirds developed, some authors advocated the early
secondary grafting (6-8 years) to preserve the lateral incisor especially
if present in the posterior segment.

Late grafting; after 12 or 13 years of age, after the eruption of the

permanent canine.

Common presenting problems

 Discharge and smell from the nose.

 Oral food/fluids leaking from the nose.
 Poor speech.
 Inability to suck up a straw or blow up balloons.
 Poor appearance of the incisor teeth.
 Missing or supernumerary teeth within the cleft area.
 Difficulty cleaning teeth in the cleft area.
 Poor facial appearance.

Assessment of the patient

It includes taking thorough history and clinical examination that should

note the presence of supplemental and supernumerary teeth and the
presence of dental caries in the remaining dentition. Meticulous dental
care addresses treatment of any periodontal disease and/or extraction of
any aberrant teeth to prepare the cleft site for bone grafting.

For radiographic examination panoramic (OPG), occlusal and periapical

films are essential for treatment planning. CBCT is becoming
increasingly important as this gives full visualization of the cleft defect at
a reasonable radiation dosage. Postoperatively, CBCT can be used to
accurately assess the volume of successfully grafted bone.

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Study models are obtained and, for the bilateral cleft cases, an
interocclusal acrylic wafer is constructed to the predetermined agreed
postoperative position of the premaxilla.

Orthodontic treatment may be required to align the maxillary arch in

preparation for the bone graft. This usually involves expansion of the
arch in a transverse diameter and results in opening up the alveolar cleft
to some degree.

Procedure

The procedure requires reflecting full-thickness mucoperiosteal buccal

and palatal flaps, the nasal mucosa is then reflected and sutured, then
the palatal defect is closed by first closing the palatal flaps, converting
the cleft palate into a single flap. The graft material is then placed into
the cleft and finally, the labial flaps can be advanced, and they are
sutured to each other and then to the palatal flap over the crest of the
ridge, it may be necessary to perform a small backcut or to release or
score the periosteum to obtain a tension-free closure. It is best to use a
resorbable monofilament suture.

Sources of bone graft materials

A variety of autologous, allogeneic, and xenogeneic bone materials have

been used for correcting alveolar cleft. Of these, fresh autologous
cancellous bone is the ideal bone graft source. Both cortical and
cancellous bones can be used for a bone graft, but cancellous bone is
known to be better because of the cell transfer and revascularization in
osteoinduction and osteoconduction.

 Iliac crest; is the most commonly used bone in bone grafting because
it is easy to harvest, it can provide a large amount of cancellous bone,
and cleft preparation can be performed at the same time. The
disadvantages of using this bone are possible scarring, postoperative
pain, delayed ambulation, and risk of cutaneous nerve injury.
 Cranium; it has little resorption, less postoperative pain, and a
concealed scar. However, a long operative time and serious side effects
such as hematoma, seroma, dural tear, dural exposure, and
cerebrospinal fluid leakage are possible.
 Tibia; it results in less bleeding, postoperative pain, operative time,
and scarring, as well as faster ambulation and a shorter hospital stay.
However, it yields relatively small amounts of bone. Moreover, in

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 children there is possibility of growth disturbance due to injury of the
epiphyseal cartilage.
 Mandibular symphysis; the mandible has the same embryonic origin
as the maxilla which may contribute to low resorption, surgery is in
the same operative field and postoperative discomfort is reduced.
However, there is a risk of roots and mental nerve damage, and the
amount that can be collected depends on the development of the
mandible.
 Bone graft substitutes; including demineralized freeze-dried bone
(DFDB), freeze-dried bone (FDB), freeze-dried matrix (FDM),
hydroxyapatite (HA) or tricalcium phosphate (TCP). Recently the
addition of growth factors such as platelet-rich plasma (PRP) and
platelet rich fibrin (PRF) with graft materials have used.

Postoperative care

Antibiotics usually start before the incision and continue postoperatively

for 7-10 days along with chlorhexidine mouth wash, excellent oral
hygiene and good nutrition are encouraged, patients and parents are
instructed to liquid and soft diet for 2-3 weeks and to avoid any trauma
to the operative site.

Complications

 Wound dehiscence which is managed by debridement and

antimicrobial mouth washes with or without systemic antibiotics.
 Infection which is managed by conservative debridement, daily
irrigation and packing with oral antibiotics.
 Persistent fistula which may require subsequent procedures.

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