CONGENITAL ANOMALIES OF THE FACIAL REGION OF THE HEAD. CLEFT LIP.
CLEFT PALATE.
Embryology of the Lip and Palate
The “primary palate,” which includes the nostril sill, upper lip, alveolus, and hard palate
anterior to the incisive foramen, forms from fusion between the medial nasal and
maxillary prominences during weeks 4 through 7 of gestation.
Development of the hard palate posterior to the incisive foramen and the soft palate,
which are collectively known as the “secondary palate,” occurs during weeks 6 through
12 of gestation.
The normal palate functions primarily as a speech organ, but it is also intimately
involved in feeding, swallowing, and breathing. The soft palate, or velum, together with
lateral and posterior pharyngeal walls, can be conceptualized as a valve that regulates
the passage of air through the nasopharynx.
Based on anatomy, etiology, and current treatment principles, most craniofacial
anomalies can be classified into one of four categories:
1. Clefts
2. Synostoses: The term “craniosynostosis” refers to premature fusion of one or more
calvarial sutures.
3. Atrophy–hypoplasia
4. Hypertrophy–hyperplasia–neoplasia
Cleft Lip And Cleft Palate
Development of Face
Face develops from median nasal process, lateral nasal process, maxillary process,
mandibular arch, globular arch, olfactory pit and eye. Any change in the development or
fusion of these arches leads to formation of different types of cleft lip or cleft palate.
Aetiology
 Familial—more common in cleft lip or combined cleft lip and palate (Risk is 1:25
live births).
 Protein and vitamin deficiency.
 Rubella infection.
 Radiation.
 Chromosomal abnormalities.
 Maternal epilepsy and drug intake during pregnancy (steroids/ eptoin/diazepam).
Classification
I. Cleft lip alone: Unilateral, Bilateral, Median.
II. Cleft of primary palate (in front of incisive foramen) only:
a. Complete—means absence of pre-maxilla.
b. Incomplete—means rudimentary pre-maxilla: Unilateral, Bilateral, Median.
III. Cleft of secondary palate (behind the incisive foramen) only:
a. Complete—nasal septum and vomer are separated from palatine process.
b. Incomplete.
c. Submucous: It can be - Cleft with soft palate involvement. - Cleft without soft palate
involvement.
IV. Cleft of both primary and secondary palates.
V. Cleft lip and cleft palate together.
The most clinically useful system to describe cleft palate morphology is the Veau
classification.
 A Veau I cleft is midline and limited to the soft palate alone, whereas a Veau II cleft
may extend further anteriorly to involve the midline of the posterior hard palate
(the “secondary palate”).
 A Veau III cleft is a complete unilateral cleft of primary and secondary palates, in
which the cleft extends through the lip, the alveolus, the entire length of the nasal
floor on the cleft side, and the midline of the soft palate.
 Veau IV clefts are bilateral complete clefts of the primary palate that converge at the
incisive foramen and continue posteriorly through the entire secondary palate.
 Not included in the Veau classification is the submucous cleft palate, which occurs
when there is clefting of the soft palate musculature beneath intact mucosa.
Submucous cleft palate classically presents as the triad of a bifid uvula, a midline
translucency called the “zona pellucida” and a palpable notch of the posterior hard
palate.
Cleft Lip
 Central—rare. In upper lip. Between two median nasal processes. (Hare lip)
 Lateral—maxillary and median nasal process, commonest; can be unilateral or
bilateral
 Incomplete cleft lip does not extend into nose
 Complete cleft lip extends into nasal floor
 Simple cleft lip is only cleft in the lip
 Compound cleft lip is cleft lip with cleft of alveolus.
LAHS classification of cleft disorders
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‘L’ for lip, ‘A’ for alveolus, ‘H’ for hard palate, ‘S’ for soft palate
Capital ‘LAHS’ for ‘complete type'
Small letters ‘lahs’ for ‘incomplete type’
Asterisks ‘lahs’ for microclefts
‘LAHSHAL’ for bilateral clefts
Problems in Cleft Disorders
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Difficulty in sucking and swallowing. This is more commonly observed in cleft
palate than in cleft lip.
Speech is defective especially in cleft palate, mainly to phonate B, D, K, P, T and G.
Altered dentition or supernumerary teeth.
Recurrent upper respiratory tract infection.
Respiratory obstruction (in Pierre-Robin syndrome)
Chronic otitis media, middle ear problems.
Cosmetic problems.
Hypoplasia of the maxilla.
Problems due to other associated disorders.
Treatment for Cleft Lip
Millard criteria are used to undertake surgery for cleft lip.
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Millard cleft lip repair by rotating the local nasolabial flaps.
Management of associated primary or secondary cleft palate deformity.
Proper postoperative management like control of infection, training for sucking,
swallowing and speech.
Tenninson’s ‘Z’ plasty (Tenninson-Randall triangular flap).
Millard citeria (rule of 10)
 10 pound in weight
 10 weeks old
 10 gm % haemoglobin
Note:
Delaire timing of the cleft surgery – Unilateral/bilateral
cleft lip alone, in one stage operation done in 4–6
months. For cleft palate alone involving only soft palate, in one stage, surgery is done in
6 months.
For cleft palate alone but involving both soft and hard palates—soft palate in 6 months;
hard palate in 18 months. In combined cleft lip and palate, unilateral or bilateral, in two
stages—cleft lip and soft palate in 6 months; hard palate in 18 months.
Cleft Palate
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It is due to failure of fusion of the two palatine processes.
Defect in fusion of lines between premaxilla (developed from median nasal process)
and palatine processes of maxilla one on each side.
When premaxilla and both palatine processes do not fuse, it leads into complete
cleft palate (Type I cleft palate).
Incomplete fusion of these three components can cause incomplete cleft palate
beginning from uvula towards posteriorly at various lengths. So it could be Type II
a–bifid uvula, Type II b–bifid soft palate (entire length) or Type II c –bifid soft palate
and posterior part of hard palate (but anterior part of hard palate is normal).
Small maxilla with crowded teeth, absent/poorly developed upper lateral incisors.
Bacterial contamination of upper respiratory tract with recurrent infection is
common.
Chronic otitis media with deafness may occur.
Swallowing difficulties to certain extent and speech problems can occur.
Cosmetic problems can occur.
Principles of cleft lip repair
1. “Rule of 10’ should be fulfilled
2. Before 6 months, it should be operated
3. Infection should not be present
4. Millard advancement flap is commonly used for unilateral cleft lip repair
5. Bilateral cleft lip repair can be done either in a single or two stages (with 6 months
gap between each stage)
6. One stage bilateral cleft lip repair is done using Veau III method/ Millard’s single
stage/Black method
7. Proper markings are made prior to surgery and incision should be over full
thickness lip
8. Often 1:2,00,000 adrenaline injection is used to achieve haemostasis
9. Three-layer lip repair should be done (mucosa, muscle and skin)
10. Cupid’s bow should be horizontal
Treatment for Cleft Palate
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Cleft palate is usually repaired in 12–18 months. Early repair causes retarded
maxillary growth (probably due to trauma to growth center and periosteum of the
maxilla during surgery if done early). Late repair causes speech defect.
Both soft and hard palates are repaired.
Abnormal insertion of tensor palati is released. Mucoperiosteal flaps are raised in
the palate which is sewed together. If maxillary hypoplasia is present, then
osteotomy of the maxilla is done. With orthodontic help teeth extraction and
alignment of dentition is done.
Regular examination of ear, nose and throat during follow up period.
Postoperative speech therapy.
Whenever complicated problems are present, staged surgical procedure is done.
Wardill- Kilner push back operation—by raising mucoperiosteum flaps based on
greater palatine vessels.
Secondary management:
 Hearing support is given using hearing aids if defect is present; control of otitis
media.
 Speech problems occur due to velopharyngeal incompetence; articulation
problems also can occur – speech therapy is given. It is corrected by
pharyngoplasty, veloplasty, speech devices.
 Dental problems like uneruption, unalignments are common. They should be
corrected by proper dentist opinion, and reconstructive surgery.
 Orthodontic management with alveolar bone graft, maxillary osteotomy—done
in 8–11 years of age.
 Veloplasty, dental implants, rhinoplasty, orthognathic surgeries, etc.
Principles of palatoplasty
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Timing is between 10–18 months
Mucoperiosteum flap is raised
Palatal defect is closed using 3 layers—nasal, muscle and oral layers
Hook of pterygoid hamulus is fractured to relax tensor palate muscle to relieve
tension on suture line.