Cleft lip and cleft palate pdf - د. احمد خلف

Baghdad College of Medicine / 5

grade

Student’s Name :

Dr. Ahmed Khalaf
Lec. 1

Cleft lip & Cleft palate

Wed. 28 / 9 / 2016

DONE BY : Ali Kareem

مكتب اشو

ر لالستنساخ

2016 – 2017

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

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.

Risk factors :

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.

Cleft lip :

o The critical developmental period of lip and primary palate occur during 4-

6 weeks of gestation.

o Unilateral cleft lip result from failure of fusion of medial nasal prominence

and maxillary prominence on one side.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

o 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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Classification of cleft lip:

1- according to extent of cleft lip:

o Microform cleft lip : furrow or scar through the vertical length of lip.
o Incomplete cleft lip : vertical separation of lip (skin, orbicularis

muscle and mucosa) with intact nasal sill.

o Complete cleft lip : vertical separation of the lip, nasal sill and

alveolus.

Unilateral cleft lip

Bilateral cleft lip and palate

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

2- according to location of the cleft :

o Unilateral cleft lip.
o Bilateral cleft lip.

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

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Cleft lip may be associated with nasal deformity, which could be mild, moderate or
severe nasal deformity.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Normal anatomy of lip :

1- Topographic landmark.

Columella

Philter columns

Cupid’s bow

Vermillion roll

Wet vermillion

Dry vermillion

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

2- muscles:

o Orbicularis oris: function as sphincter (deep fibers) and for speech

(superficial fibers).

o Levator labii superoris: elevate the upper lip.
o 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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

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.

Management :

o 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.

o Time of repair: according to rules of ten:

o Should be 10 weeks old.

o Should be 10 pounds (4.5kg).

o Should be 10gm/dl hemoglobin level.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Preoperative measurement :

1- Elastic head cap: used in first week of

life especially for projected premaxill
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- 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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

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-  Produce functional continuity of muscles.
2-  Recreate symmetry.
3-  Reconstruction Cupid bow.
4-  Minimize scarring.
5-  Treated the associated nasal deformity.
6-  Should repair all layers of lip (skin, muscles, and mucos).

Methods of repair:

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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Repair of bilateral cleft lip

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

Postoperative care

o Avoid using nipple of bottle for feeding instead used spoon for feeding.
o Keep using tape in place for support after suture removal (usually suture

removed under general anesthesia after 5 days).

Cleft palate

o It could occur as separated deformity or in combination with cleft lip

deformity. It may be unilateral or bilateral deformity.

Embryology

o 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 Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

o 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.

Anatomy :

1- Hard palate: it is the bony part consists of primary and secondary palate

which separated by incisive foramen. It consist of :

o  Premaxilla, extended to incisive foramen.
o  Paired maxilla.
o  Palatine bone.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

2- soft palate(velum): contains muscle involved in velopharyngeal closure

which:

1- extrinsic muscles:

o  Levator palate muscle.
o  Tensor veli palatini muscle.
o  Palatopharyngeus
o  Palatoglossus
o  Salpingopharyngeus
o  Superior constictor

2- intrinsic muscle:

o musculus uvulae

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

o The prepalatal structures (primary palate):

structure anterior to incisive foramen
(alveolus, lip, nasal floor, and alar cartilage).

o The palatal structures (secondary palate):

those posterior to incisive foramen.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

©Ali Kareem 2016-2017

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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

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 :

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

o 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)

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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

Surgical correction :

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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

4- Furlow technique: soft palate elongation with double reversing Z-plasties

Postoperative care:

o  Elbow restrain are worn continually for 4-6 weeks.
o  The airway is observed and the child placed on oxygen monitor.
o  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.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

Outcome :

1- Speech problem: velopharyngeal incompetence will need speech therapy.

2- Secondary palatal procedure:

o Treatment of palatal fistula.
o Treatment of velopharyngeal

incompetence.

3- Alveolar reconstruction at 7-10 : years of age with initial orthodontic

alignment, then bone graft.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

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 hypernasal speech.

Cleft Lip and Palate Dr. Ahmed Khalaf

28-9-2016

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.

#END of this Lecture …