Congenital defects of the Face

Congenital defects of the Face

2. Introduction
Includes:
Cleft lip and palate
Developmental abnormalities of the jaw and teeth
Cleft lip, alveolus,hard and soft palate are the
most common congenital abnormalities of the
orofacial structures.

3. Incidence
Cleft lip and palate is 1 in 600 live births.
Isolated cleft palate is 1 in 1000 live births.
The typical distribution of cleft types is:
Cleft lip alone 15%
Cleft lip and palate 45%
Isolated cleft palate 40%
Cleft lip palate predominates in male
Cleft lip alone more in females
In unilateral cleft lip, the deformity affects
left side in 60% of cases.

4. Aetiology
Genetic predisposition
Fx of cleft lip and palate in 1
st
degree relatives increases the risk to 1
in 25 cases.
Environmental factors:
Maternal epilepsy
Drugs: steroids, phenytoin, diazepam
Associated with syndromes ( mostly isolated cleft palate)
Pierre Robin sequence (Isolated cleft palate, retrognathia,
posteriorly displaced tongue)
Stickler syndrome ( ophthalmic & musculoskeletal abnormalities)
Downs syndrome

5. Classification
LAHSHAL system: describes site, size, extent and type of cleft.
eg.
 LAHSHAL = complete bilateral cleft lip and palate.
lahSh = incomplete right unilateral cleft lip and alveolus with complete cleft
of the soft palate extending partially onto hard palate.

6. Cleft Lip
Anatomy:
Frontal view showing muscle
chain of the face
A) nasolabial muscles
1- transverse nasalis
2- levator labii superioris
alaeque nasi
3-levator labii superioris
B) bilabial muscles orbicularis
oris
4- oblique head
5- horizontal head
6- lower lip
C) labiomental muscles
7- depressor anguli oris
8- depressor labii inferioris
9- mantalis

7. Cleft lip
Types:
Unilateral cleft lip:
Nasolabial and bilabial muscle rings are
disrupted on one side
 resulting in asymmetrical deformity
involving external nasal cartilage, nasal
septum, and maxilla
causing displacement of nasal skin onto
the lip and retraction of labial skin.
Bilateral cleft lip:
Symmetrical deformity
Two muscular rings are disrupted on both
sides.

8. Cleft palate
Embryologically:
1ry palate: structures ant to
incisive foramen (alveolus
and upper lip)
2ry palate: structures post to
incisive foramen (hard and
soft palate)
Cleft palate results from
failure of fusion of the two
palatine shelves

9. Soft Palate
Muscle fibers of the soft
palate are oriented
transversely with no
significant attachment to the
hard palate.
In cleft soft palate, muscle
fibers are orientated in an
anteroposterior direction,
inserting into the posterior
edge of the hard palate

10. Hard Palate
Anatomy:
1- Palatal fibromucosa:
lies directly below the
floor of the nose.
2- Maxillary fibromucosa:
contains the greater
palatine neurovascular
bundle.
3- Gingival fibromucosa:
adjacent to the teeth.

11. Types
Incomplete: when the cleft
of the hard palate remains
attached to the nasal
septum and vomer.
Complete: when the nasal
septum and the vomer are
completely separated from
the palatine process

12. Primary management
Antenatal diagnosis:
 cleft lip could be diagnosed by US after week 18 of
gestation
Cleft palate cannot be diagnosed on antenatal scan
Feeding:
Soft teats and modified teats
Enlarging the hole in the teat.

13. Cont.
Surgical techniques:
Cleft lip
Principle: attach and reconnect the muscles around nasal
aperture and oral sphincter
Performed btw 3- 6 months
Skin incisions to restore displaced tissues including skin and
cartilage to their normal position
Nasolabial muscles are anchored to the premaxilla
Oblique muscles of orbicularis oris are sutured to the base of
the ant nasal spine
Closure is completed by suturing the horizontal fibers of
orbicularis oris.

14. Cont.
Surgical techniques:
Cleft palate
Principle: mobilization and reconstruction of the abbarent
soft palate muscles, together with closure of the residual
hard palate cleft by minimal dissection and subsequent
scarring
Cleft palate closure can be achieved by one or two stage
palatoplasty

15. Cont.
Delaire technique and sequence is one the regimens used for timing of
primary cleft lip and palate procedures:
Cleft lip alone:
Unilateral: one operation
at 5-6 months
Bilateral: one operation
at 4-5 moths
Cleft palate only:
Soft palate only: one operation,
 at 6month
Soft and hard palate: two operations,
soft at 6 months,
hard at 15- 18 months
Cleft lip and palate:
Unilateral: two operations:
cleft lip and soft palate at 5-6months
Hard palate at 15- 18months
Bilateral: two operations:
 cleft lip and soft palate at 4-5 months
Hard palate at 15- 18 months

16. Secondary management
Hearing
Higher incidence of sensorinueral hearing loss and conductive
hearing loss
Regular hearing tests should be performed before 12 months of age
Speech:
 Velopharyngeal incompetence
Articulation problems
Speech problems
Dental:
Hypodontia, hyperdontia, delayed eruption of teeth are common
problems
Management include dietary advice, fluoride supplements, and
fissure sealants.

17. Secondary surgery for cleft lip and palate
Includes:
Cleft lip revision
Alveolar bone graft
Simultaneous lip revision and alveolar bone graft
2ry palate procedures
Dentoalveolar procedure
Rhinoplasty
Orthognathic surgery

18. Cleft lip revision
Should be delayed for at least 2 years after the 1ry
surgery
Aims to improve incomplete 1ry reconstruction
Indications:
Lip deformities:
Misaligned vermilion
Asymmetrical cupid’s bow
Muscle discontinuity or misalignment
Nasal deformity:
Lateral drift of alar base
Poor nasal tip projection
Deviated nasal septum

19. Cleft lip revision
Should be delayed for at least 2
years after the 1ry surgery
Aims to improve incomplete 1ry
reconstruction
Indications:
Lip deformities:
Misaligned vermilion
Asymmetrical cupid’s bow
Muscle discontinuity or
misalignment
Nasal deformity:
Lateral drift of alar base
Poor nasal tip projection
Deviated nasal septum


20. Cleft lip revision
Should be delayed for at least 2
years after the 1ry surgery
Aims to improve incomplete 1ry
reconstruction
Indications:
Lip deformities:
Misaligned vermilion
Asymmetrical cupid’s bow
Muscle discontinuity or
misalignment
Nasal deformity:
Lateral drift of alar base
Poor nasal tip projection
Deviated nasal septum

21. Alveolar bone graft
Done in patients with residual alveolar
cleft ass with cleft lip and palate
Aim:
promotion of eruption of canine tooth
into the cleft site
Eliminates oronasal fistula
Best performed between 8- 11 years

22. Orthognathic surgery
Impaired growth of maxilla is due to
 poor and traumatic 1ry surgery.
Genetic predisposition
Principle of deformity:
underdevelopment in both horizontal
and vertical direction of maxilla
leading to pseudoprognathism
(A projection of the mandible due to
occlusal disharmonies that force the
mandible forward; the mandibular
condyles are forward of their expected
functional position)
 surgery is performed when facial
growth is completed( female 16 years,
male 19 years)

23. Developmental abnormalities of the jaw
Disproportionate growth between
the maxilla and the mandible
resulting in derangement of the
dental occlusion
Classification:
Class I: theres normal relation
between upper and lower incisors
and molar dentition
Class II: the mandibular teeth are
placed post to the maxillary teeth
Class III: the mandibular teeth are
placed ant to the maxillary teeth.
Management: orthognathic surgery

24. Developmental abnormalities of the teeth
Divided into:
Abnormality in number
Defects of structure and size
Disorders of eruption of teeth

25. Number
Anodontia: congenital absence of all teeth. Rare
Partial anodontia: failure of development of 1ry
or more commonly 2ry dentition
Most frequent absent teeth:
Third molar ( wisdom teeth)
2
nd
premolars
Maxillary lateral incisor teeth
Ass with:
Ectodermal dysplasia
Down syndrome
Cleft lip and palate
Management: prosthetic replacement of teeth

26. Defects of structure of the teeth
Causes:
Genetic disorders:
Amelogenesis imperfecta: changes in the structure
(hypoplasia) or mineralization ( hypocalcification)
Dentinogenesis imperfecta: ass with soft dentine with short
roots. Usually ass with osteogenesis imperfecta
Systemic causes:
Measels
Rickets
Hypoparathyroidism
Tetracycline

27. Disorders of eruption
Delayed eruption of teeth may involve a single tooth
or the entire dentition
Local factors:
Loss of space/ overcrowding
Additional teeth
Retention of deciduous tooth
Systemic factors:
Matabolic diseases: critinism and rickets
Osteodystrophies
Hereditary gingival fibromatosis
Management:
Remove any obstruction
Can be left alone