Cleft lip and cleft 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.
Cleft lip:
The critical developmental period of lip and primary palate occur during 4-6
weeks of gestation.
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.
Risk factors:
1. Medication: e.g. phyenytoin, steroid, diazepam.
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 e Xlinked, or autosomal dominant e.g. Van der moude syndrome. Or
familial i.e. associated with syndrome e.g. Down syndrome.
5. Folic acid and B6 intake during pregnancy may reduce left lip and
cleft palate.
Classification of cleft lip:
1. according to extent of cleft lip:
 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.
1
 Complete cleft lip: vertical separation of the lip, nasal sill and
alveolus.
2. according to location of the cleft:
 Unilateral cleft lip.
 Bilateral cleft lip.
For both of above may be complete or incomplete or microform.
Cleft lip may be associated with nasal deformity, which could be mild,
moderate or severe nasal deformity.
Normal anatomy of lip:
1. Topographic landmark.
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.
Cleft lip anatomy:
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.
2
Management:
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:
1. Should be 10 weeks old.
2. Should be 10 pounds (4.5kg).
3. Should be 10gm/dl hemoglobin level.
Preoperative measurement:
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:
1. Initial lip procedure at (10-12 weeks) of age.
2. Time of revision should be complete at age (5-6 years) i.e. preschool
age.
3. Final nasal deformity revision in adolescent.
Surgical treatment:
Preoperative investigation: chest X-ray to exclude any chest infection,
bleeding profile to exclude any bleeding tendencies and Hb level.
Principal of repair:
1.
2.
3.
4.
Produce functional continuity of muscles.
Recreate symmetry.
Reconstruction Cupid bow.
Minimize scarring.
3
5. Treated the associated nasal deformity.
6. Should repair all layers of lip (skin, muscles, and mucos).
Methods of repair:
1.
2.
3.
4.
Straight line repair.
Lateral quadrilateral flap technique.
Lateral triangular flap technique.
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.
Postoperative care;
1. Avoid using nipple of bottle for feeding instead used spoon for
feeding.
2. Keep using tape in place for support after suture removal (usually
suture removed under general anesthesia after 5 days).
Cleft palate;
It could occur as separated deformity or in combination with cleft lip
deformity. It may be unilateral or bilateral deformity.
Embryology;
Failure of fusion of palatal shelves in the midline. Fusion occurs from
anterior to posterior.
Anatomy:
The palate composed of:
1. Hard palate: it is the bony part consists of primary and secondary
palate which separated by incisive foramen. It consist of :
4
 Premaxilla, extended to incisive foramen.
 Paired maxilla.
 Palatine bone.
2. soft palate(velum): contains muscle involved in velopharyngeal
closure which:
 Levator palate muscle.
 Tensor palate muscle.
Vascular and nerve supply through the greater palatine artery and nerves
through the greater palatine foramen.
Secondary blood supply through the lesser palatine artery and nerve through
incisive 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.
Classification of cleft palate:
1. Prepalatal cleft (cleft of primary palate): could be unilateral or
bilateral.
2. Palatal cleft (cleft of secondary palate): could be complete or
incomplete.
3. Bifid uvula.
4. Submucous cleft: bifid uvula plus dehiscence of levator muscle plus
short palate plus submucosa cleft of posterior third of bony palate.
Management:
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.
5
Surgical correction:
Time usually between ages of 9-11 months.orthopaedic appliances should be
wear by patient before surgery.
Surgical procedures are:
1. Bilateral bipedicled mucoperiosteal flap (Von Longenbeck
repair): the flaps are closed at midline, nasal mucosa first and oral
mucosa last. This technique is not involving elongation of palate.
2. V-Y elongation technique (Veau-Wardil-kilner).
3. Furlow technique: soft palate elongation with double reversing Zplasties.
Surgical complication of cleft palate surgery:
1. Fistula: most common in wide bilateral cleft palate.
2. Midfacial growth problem.
3. Airway obstruction may occur secondary to postoperative bleeding.
Outcome:
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:
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 hyponasal speech.
6
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.
7