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Cleft lip and palate

Cleft lip and cleft palate are the second most


frequently occurring of the major congenital
anomalies they occur in 1:750-1:1000 ,club foot
being the most common.
Racial and ethnic variations exist, with clefting
occurring more commonly in Asians and less
frequently in Africans, while whites are
intermediate in occurrence.
Assay
1-Medication: e.g. phyenytoin, steroid, diazepam, retinoic
acid
2-Smoking.
3-Parental age especially father age, or both mother and
father age over 30 years.
4-Family history: the risk increase with increase number
of family who had cleft lip. Most cases are sporadic
(multifactorial), but may be X-linked, or autosomal
dominant e.g. Van der moude syndrome which is
autosomal dominant disorder with lower lip pits in
association with different degree of lip and palatal
clefting
Or associated with syndrome e.g. Down syndrome,
there are more than 150 syndromes described in
which the clefting may be a feature.
5-Folic acid and B6: intake during pregnancy may reduce
cleft lip and cleft palate.

6- low socioeconomic status : this is possibly related


. to
inadequate nutrition.
• The critical developmental period of lip and primary palate
occur during 4-6 weeks of gestation.
‫مهههم‬
• Unilateral cleft lip result from failure of fusion of medial nasal
prominence and maxillary prominence on one side.
• Bilateral cleft lip result from failure of fusion of medial nasal
prominence with maxillary prominence on either site. Male to
female ratio 2:1, left side more common than right side.
Intrauterine life screening
- to identify associated anomalies ( most important )
- to prepare surgical team or decide to do abortion
Unilateral cleft lip Bilateral cleft lip and palate
1-according to extent of cleft lip: ‫كلش مهمة هاي التقسيمات‬

Microform cleft lip: furrow or scar through the vertical length of lip.
Incomplete cleft lip: vertical separation of lip (skin, orbicularis muscle
and mucosa) with intact nasal sill.
Complete cleft lip: vertical separation of the lip, nasal sill and alveolus.
Microform
Incomplete unilateral Complete unilateral
2-according to location of the cleft:
– Unilateral cleft lip.
– Bilateral cleft lip.

For both of above may be complete or incomplete or microform.

Bilateral complete Bilateral complete


‫مهمة‬
Cleft lip may be associated with nasal deformity, which could be mild,
moderate or severe nasal deformity.
1-Topographic landmark.

Columella

Philter columns
Cupid’s bow

Vermillion roll

Wet vermillion

Dry vermillion
2-muscles:
– Orbicularis oris: function as sphincter (deep fibers) and for speech (superficial fibers).
– Levator labii superoris: elevate the upper lip.
– Nasalis or depressor septi nasi muscle: depress the columella down and elevate the
upper lip.
3-Arterial blood supply: by labial artery bilaterally.
4-Sensory innervation: by maxillary branch of trigeminal nerve.
5-Motor innervation: by zygomatic and buccal branches of facial nerve.
1-Disruption of continuity, orientation and quality
of muscles.
2-Cupid bow and lip rotated upward on both
the lateral –cleft side-as well as medial side.
• 3-The alveolus and nostril floor are open in
complete cleft lip.
• 4-The premaxilla is rotated and protruding
especially in bilateral cleft lip.
• 5-Associated cleft lip nasal deformity e.g.
flatten alar dome on affected side, shortened
columella especially bilateral cases.

Deficient columella
MTD approach including ENT, speech therapist, maxilofacial surgeon

• The parents should be reassured, and the


newborn should evaluate for associated
anomalies, and the parents should inform about
the stages and operation that expected throughout
the child lifetime.
• Time of repair: according to rules of ten: ‫مهمة كلش‬

• Should be 10 weeks old.


• Should be 10 pounds (4.5kg).
• Should be 10gm/dl hemoglobin level.
1-Elastic head cap: used in first week of life
especially for projected premaxilla in bilateral cleft
lip.
• 2-Maxillary orthopedic: for collapsed maxillary
arches at (1-2) weeks of age.
Notes: ‫مهمةة‬

1-Initial lip procedure at (10-12 weeks) of age.


2-Columellar lengthening at (18 months) of age.
3-Time of revision should be complete at age (5-6
years) i.e. preschool age.
4-Final nasal deformity revision in adolescent.
Preoperative investigation: chest X-ray to exclude any
chest infection, bleeding profile to exclude any bleeding
tendencies and Hb level.
• Principal of repair:
1-Produce functional continuity of muscles.All the layers should be repaired

2-Recreate symmetry.

3-Reconstruction Cupid bow. Very important

4-Minimize scarring.

5-Treated the associated nasal deformity.

6-Should repair all layers of lip (skin, muscles, and


mucos).
Methods of repair:
These 3 are old surgeries
1-Straight line repair.

2-Lateral quadrilateral flap technique.

3-Lateral triangular flap technique.


4-Rotational advancement technique(Millard):most
commonly used repair in which the medial lip is rotated
downward to fill the cleft defect, and lateral lip is
advanced to fill the defect that occur in place of medial
lip ,with small pennant shaped C-flap used either to
create nasal sill or lengthened the columella.
Repair of bilateral cleft lip

complete incomplete
• Avoid using nipple of
Or bottle for feeding instead
used
Use spoon for feeding.

• Keep using tape in place for support after


suture removal (usually suture removed under
general anesthesia after 5 days).
It could occur as separated deformity or in combination
with cleft lip deformity. It may be unilateral or bilateral
deformity.
• Cleft of the primary palate which located anterior to
incisive foramen is result from failure of the
mesenchymal masses in the lateral palatine process
to fuse with those in median palatine process.
• cleft of the secondary palate which is located
posterior to incisive foramen is result from
failure of the mesenchymal masses in the lateral
palatine process to fuse with each other and
with nasal septum , it is believed that delay in
elevation of the palatal shelves from vertical to
horizontal is part of the underlying mechanism.
1-Hard palate: it is the bony part consists of primary and secondary
palate which separated by incisive foramen. It consist of :
– Premaxilla, extended to incisive foramen.
– Paired maxilla.
– Palatine bone.
• 2-soft palate(velum): contains muscle involved in velopharyngeal
closure which:

1-extrinsic muscles:
– Levator palate muscle.
– Tensor veli palatini muscle.
– Palatopharyngeus
– Palatoglossus
– Salpingopharyngeus
– Superior constictor

2-intrinsic muscle:
-musculus uvulae

‫مهمم كلش‬
All are supply by vagus and glossopgaryngeal nerve except the tensor veli palatini
which supply by trigeminal nerve.
• Vascular and nerve supply of hard palate is through the greater palatine
artery and nerves through the greater palatine foramen.

• Secondary blood supply through the lesser palatine artery and nerve through
lesser palatine foramen
• -The prepalatal structures (primary palate): structure anterior to
incisive foramen (alveolus, lip, nasal floor, and alar cartilage).

• -The palatal structures (secondary palate): those posterior to


incisive foramen.
Cleft palate anatomy:
Classification of cleft palate:
Veau described a classification divided into four groups:

group 1: cleft of the soft palate.


Group 2: cleft of the hard and soft palate up to incisive foramen involving the
secondary palate.
‫كلش مهم التقسيم‬

Group 3: complete unilateral cleft, extending from uvula to the incisive foramen in
midline , then reflecting to one side and extending through alvelous at the position
of future lateral incisor tooth
Group 4: complete bilateral cleft, resembling group 3 with two cleft
projecting forward from incisive foramen through alveolus; the small
anterior segment of palate, the premaxilla, remains suspended from
the nasal septum.
other rare type of cleft palate include:

Bifid uvula:
‫مهم كلش‬
Submucous cleft: bifid uvula plus dehiscence of levator muscle plus
short palate plus notch of posterior third of bony palate. the majority of
the patients with submucous cleft palate are asymptomatic, although
approximately 15% will develop velopharyngeal insufficiency (VPI)
VPI causes speech problems
1-Feeding: since the baby with cleft palate is unable to create adequate suction
so that the feeding should be done with nipple with large holes, and baby
should hold in 45° degree to decrease regurgitation into the nose, and
feeding should take longer time.

2-Maintenance airway by prone position during sleeping.

3-Patient usually has otitis media because of Eustachian tube abnormalities the
child need careful fallow up with otolaryngologist and audiologist.

4-Associated deformity occurs in about 29% of children with cleft palate.


‫مهمم‬
Time usually between ages of 9-11 months.orthopaedic appliances should be
wear by patient before surgery.
Goals of reconstruction:

1- Recreate a continuous hard palate with palatal tissue that allow


no communications between oral and nasal cavities.

2- Reconstruct the muscular anatomy to that of normal to allow


the action of the levator veli palatini muscle to elevate the
mobile soft palate and separate the nose.

These goals should be accomplished without creating significant


scarring of maxilla which increase the incidence of midface
retrusion.
Surgical procedures are:
1-Bilateral bipedicled mucoperiosteal flap (Von Longenbeck
repair): the flaps are elevated and then closed at midline,
nasal mucosa first and oral mucosa last. This technique is not
involving elongation of palate.
2- Two flap palatoplasty (Baradach): its modification of
Langenbeck techniqe, where the incision extend along the
alveolar margins to edge of cleft.making the flap entirely
dependent on circulation from greater palatine artery. This
technique is not involving elongation of palate.
3-V-Y elongation technique (Veau-Wardil-kilner). This
technique is involving elongation of palate.
4-Furlow technique: soft palate elongation with
double reversing Z-plasties
Postoperative care:

-Elbow restrain are worn continually for 4-6 weeks.

- The airway is observed and the child placed on oxygen


monitor.

- Patient are given liquid only diet for 3 week and child
is transitioned to soft diet for an additional 3 weeks.
Surgical complication of cleft palate surgery:
1-Fistula: most common in wide bilateral cleft
palate.
2-Airway obstruction may occur secondary to postoperative bleeding.
3-Midfacial growth problem.
1-Speech problem: velopharyngeal incompetence will need speech
therapy.
2-Secondary palatal procedure:
Treatment of palatal fistula.
Treatment of velopharyngeal incompetence.
3-Alveolar reconstruction at 7-10 : years of age with initial
orthodontic alignment, then bone graft.
Velopharyngeal incompetence: ‫كلش مهم‬
Incomplete closure of soft palate against the posterior
pharyngeal wall during speech , this lead to escape the
air from oropharynx up through nasopharynx which
lead to hypernasal speech.
Management:
1-Preoperative increasing the pharyngeal muscles strength
by asking the baby to blow.
2-Using the procedure which elongate the soft palate e.g. V-
Y advancement and double opposite Z- plasty.
3-Using mymucosal flap from posterior pharyngeal wall that
suture to posterior soft plate.

4- posterior pharyngeal wall augmentation using autologus


tissues or alloplastic materials to reduce the size of
velopharyngeal orifice.
Thank you

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