Download - Congenital defects of the Face
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