Cleft Lectures

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2/2/2024

Cleft Lip, Cleft Palate and


Alveolar Cleft

 A cleft lip or palate is an orofacial birth defects in which the


tissues of the mouth or lip don't form properly during fetal
development.

 A cleft lip may be just a small notch in the lip. It may also be
a complete split in the lip that goes all the way to the base of
the nose.

 The incidence of cleft is :

- Cleft lip and palate 1-700

- Cleft lip only 1-2000

- Cleft palate only 1-2000

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 Cleft lip and palate M:F = 2:1

 Cleft palate F:M = 2:1

 Cleft lip M:F = 2:1

 Cleft lip and palate - Cleft palate - Cleft lip

46% 33% 21%

Embryology

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Facial Development:
- From 5th – 9th week and extend to 12th week for
secondary palate formation.
- The face is formed by 5 processes:
- Forehead which composed of median nasal process
& lateral nasal process.
- Two maxillary process

- Two mandibular process

- The lateral nasal process forming the ala of the nose


- The median nasal process forming:
1- the middle portion of the nose
2- the middle portion of the upper lip
3- the middle portion of the maxilla
4- the entire primary palate
In general
( frontal head, nose, philtrum, central of upper lip &
primary palate)
If there is failure of fusion cleft lip &-or palate &-or
alveolar cleft

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- The two maxillary processes formed:

Upper part of cheek, maxilla, lateral part of upper lip,


and palatine process of maxillary bone.

- The two mandibular processes formed:

Lower lip, lower part of cheek, floor of the mouth and


chin.

Common Orofacial Clefts

Partial or complete fissure of the upper lip.


Cleft
It can be unilateral or bilateral, and can be
Lip Only
associated with a cleft of the alveolar bone.

Cleft of the upper lip extending through the


primary and secondary palates, with or
Cleft Lip
without extension through the soft palate.
With Cleft Palate
Cleft lip and palate is the most common
orofacial cleft.

Cleft Palate Only


Fissure of the palate, which can affect the
soft and hard palate, or only the soft palate.

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• Anatomical Landmarks
of the Lip and Nose:

•• Philtral ridges or columns


are raised areas ascending
from the height of the peak
of the Cupid’s bow point on
each side toward the base
of the columella.

•• White roll is a rolled-up area of skin 1 to 2 mm in


height, and it extends from one oral commissure to the
other in normal persons.

•• Vermillion is the portion of the lip below the white roll.


It is made up of a superior dry area and inferior wet area
of mucosa. There is a red line at the junction of these
two areas.

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• Nose
•• Columella is the central column extending from the
base to the nasal tip in the midline.

•• Nasal sill is the raised fold on either side extending


from the lateral part of the columella to the alar base.

•• Alar bases are at the lateral most part of the nose.

•• The two ala arch symmetrically on each side from the


nasal tip.

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Problems that may be present


because of a cleft lip or palate are:
• Failure to gain weight
• Feeding problems
• Flow of milk through nasal passages during
feeding
• Misaligned teeth
• Poor growth
• Recurrent ear infections
• Speech difficulties

Pathophysiology

 Cleft lip:
◦ Caused by incomplete fusion of the nasomedial or
intermaxillary process during the 2nd month of
embryonic development

◦ Cleft causes structures of mouth and face to develop


without the normal lip muscles

◦ May affect external nose, nasal cartilages, nasal septum,


and alveolar processes also

◦ Usually just beneath the center of one nostril

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◦ Can occur bilaterally, symmetrically or asymmetrically

◦ More complete the cleft lip, the greater the chance that
teeth in the line of the cleft will be missing or malformed
 Complete cleft= entire thickness of the lip
 Incomplete cleft= only a portion of the lip is involved

Pathophysiology
 Cleft Palate:
◦ Often associated with cleft lip, but may occur without it.
◦ Fissure may affect only the uvula and soft palate
(secondary palate, formed ~ 9 weeks), or may extend
forward to the nostril and involve the hard palate and the
maxillary alveolar ridge (primary palate, formed ~ 4-5
weeks )
◦ Complete= involves the primary and secondary palate
◦ Incomplete= involves the secondary palate only
◦ Unilateral= on one side the palatal process of the maxilla
is fused with the nasal septum
◦ Bilateral= not attached to the nasal septum, and the
septum is visible through the cleft.

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Signs and Symptoms


 Split in lip or palate
 Symptoms related to location of cleft:
◦ Hard & soft palate: Facial defect
◦ Soft palate: leaking of ingested liquids from the nose
◦ Hard palate: weak, dysfunctional suck, trouble
swallowing, gagging, choking.
 Submucous Cleft- only in muscles of soft palate and hidden
by lining of mouth
◦ May not be diagnosed until later in life
◦ Indicated by: trouble feeding, nasal drainage, nasal
sounding voice.

Diagnosis

• Physical examination at birth

• Ultrasound: always visible

• Genetic testing for parents to determine risk of


having additional children with cleft lip/palate

• Echocardiography

• Skeletal X-ray

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Risk Factors
 Family History: Cleft lip more likely to be inherited than
cleft palate
 Race: More common in Native American, Hispanic & Asian
patients
 Sex: Males 2x as likely to have cleft lip; Females 2x as likely
to have cleft palate
 Environmental factors: exposure of fetus to alcohol,
cigarette smoke, or drugs
 Maternal Nutrition Deficiency: especially lack of folic acid
◦ Encourage use of prenatal vitamins.

Diagnosis
Cleft lip only is usually easily recognized on physical examination after delivery

Direct visualization of the pharynx after delivery is needed to detect a cleft palate

When visualization is difficult, palpation of palate with a finger may be useful

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Treatment and Interventions


 Treatment of the child with CL is surgical and usually
involves no long-term interventions other than possible
scar revision
 However, management of CP involves the care of a
multidisciplinary health care team to provide optimum
results
◦ includes pediatrics, plastic surgery, orthodontics,
otolaryngology, speech pathology, audiology, nursing and
social work
 Management of both is directed towards closure of the
cleft, prevention of complications and facilitation of normal
growth and development of the child.

Timing of Surgery

- Role of 10 ( 10 pound, 10 Hg, 10 weeks)


- 9-18 m. lip & palate
- 2-4 y. Speech obturator
- 4-6 y. Revision
- 7-11y. Alveolar graft
- 14-16y. Orthogenetic

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Anatomy of the Lip

Nasal sill
Philtrum
Vermillion
Cupid’s bow

Photo courtesy of Alina Flores

Orofacial Clefts | 25

Cleft Palate: Heterogeneous Phenotype

Bifid Uvula Cleft hard palate Cleft hard palate Cleft hard palate

Cleft hard palate Cleft hard palate Cleft hard palate Cleft hard palate
with cleft lip, with bilateral cleft
specified as lip
unilateral

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When art and science meet

The better example is repair of a cleft lip

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Classification of cleft
- Anatomically:

A- Cleft of primary palate

( Lip, Alveolus & anterior palate)

Unilateral or Bilateral ( Complete or Incomplete)

B- Cleft of Secondary palate

( posterior hard palate)

Unilateral or Bilateral Soft & hard palate

C- Combination cleft of primary & secondary

Classification of cleft
- Stripped Y

A- 1 & 4 Lip

B- 2 & 5 Alveolus

C- 3 & 6 Hard palate

D- O Incisive foramen

E- 7 & 8 Hard palate

F- 9 Soft palate

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- Veau Classification

A- Cleft of soft palate

B- Cleft of soft & hard palate

C- Unilateral cleft of lip & palate

D- Bilateral cleft of lip & palate

Surgical Anatomy of the Lip and Nose


• Lip
The orbicularis oris muscle has a superficial part
originating from bone (maxilla, mandible, and also the
nasal septum) and a deep part arising from the facial
muscles like the buccinator.
In an incomplete unilateral cleft lip, the muscle fibers
cross across the lip if there is at least half of the lip
that is not cleft.
In more severe cases, there is no crossover of the
muscle bundles.

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• Nose
- The hemi columella is shorter on the cleft side as
compared to the noncleft side.

- The ala on the cleft side is depressed and buckled


manifest as a wide nostril.

- The anterior nasal spine is displaced toward the


noncleft side and the nasal septal cartilage is also
deviated to the noncleft side anteriorly.

Millard’s Rotation
Advancement Procedure

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Goals of Treatment

The ultimate aim in the repair of a unilateral


cleft lip is to create a functional and
aesthetically acceptable lip looking as close to
normal as is humanly possible.

Principles of Cleft Lip Repair

• Achieving accurate skin, muscle, and mucous


membrane union.

• Reconstruction of symmetrical nostril floor.

• Symmetrical vermilion border.

• Slight eversion of the lip.

• Minimal scar.

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Postoperative Care:
- A tongue stitch placed deep and
adequately posteriorly on the tongue helps
to pull out the tongue in case of obstruction
from the tongue falling back in a baby
emerging from the effects of anesthesia.
- A nasal pack is used with paraffin gauze for
its tamponade effect in the immediate
postoperative period.

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The major objectives of a cleft palate operation


are:

• To produce anatomical closure of the defect.

• To development and production of normal speech.

• To minimize the maxillary growth disturbances.

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The basic principles of cleft palate repair are:


• Closure of the defect.
• Repositioning of the abnormal position of the
muscles of the soft palate.
• Reconstruction of the muscle sling.
• Favorable retro positioning of the soft palate
and uvula.
• Last and most important is tension free suturing.

Methods of Cleft Palate Repair Methods of repair

• Von Langenbeck’s bipedicle flap technique.


• Furlow double opposing Z-Plasty.
• Two-stage palatal repair.
• Palatoplasty.
• Alveolar extension palatoplasty (AEP).
• Primary pharyngeal flap.
• Vomer flap.
• Buccal myomucosal flap

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Complications Immédiate complications.


• Hemorrhage.
• Respiratory obstruction.
• Hanging palate.
• Dehiscence of the repair.
• Oronasal fistula formation. Late complications.
• Bifid uvula.
• Velopharyngeal incompetence.
• Abnormal speech.
• Maxillary hypoplasia.
• Dental malpositioning and malalignment.
• Otitis media

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