IntroductionIncludes:

Cleft lip and palateDevelopmental abnormalities of the jaw

and teethCleft lip, alveolus,hard and soft palate

are the most common congenital abnormalities of the orofacial structures.

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 maleCleft lip alone more in femalesIn unilateral cleft lip, the deformity

affects left side in 60% of cases.

Aetiology Genetic predisposition

Fx of cleft lip and palate in 1st 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

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.

Cleft LipAnatomy: Frontal view showing

muscle chain of the face A) nasolabial muscles1- transverse nasalis2- levator labii superioris

alaeque nasi3-levator labii superioris B) bilabial muscles

orbicularis oris4- oblique head5- horizontal head6- lower lip C) labiomental muscles7- depressor anguli oris8- depressor labii inferioris9- mantalis

Cleft lipTypes: 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.

Cleft palateEmbryologically:

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

Soft PalateMuscle 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

Hard PalateAnatomy:

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.

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

Primary managementAntenatal 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 teatsEnlarging the hole in the teat.

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.

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

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

Secondary managementHearing

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.

Secondary surgery for cleft lip and palateIncludes:

Cleft lip revisionAlveolar bone graftSimultaneous lip revision and alveolar

bone graft2ry palate proceduresDentoalveolar procedureRhinoplastyOrthognathic surgery

Cleft lip revisionShould be delayed for at least 2 years after

the 1ry surgeryAims to improve incomplete 1ry

reconstructionIndications:

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

Cleft lip revisionShould 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

Cleft lip revisionShould 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

Alveolar bone graftDone 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

Orthognathic surgeryImpaired 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)

Developmental abnormalities of the jawDisproportionate 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

Developmental abnormalities of the teethDivided into:

Abnormality in number

Defects of structure and size

Disorders of eruption of teeth

NumberAnodontia: congenital absence of all

teeth. RarePartial anodontia: failure of

development of 1ry or more commonly 2ry dentition

Most frequent absent teeth: Third molar ( wisdom teeth) 2nd premolars Maxillary lateral incisor teeth

Ass with: Ectodermal dysplasia Down syndrome Cleft lip and palate

Management: prosthetic replacement of teeth

Defects of structure of the teethCauses: 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

Disorders of eruptionDelayed eruption of teeth may involve a

single tooth or the entire dentitionLocal 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