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Introduction <ul><li>Includes: </li></ul><ul><ul><li>Cleft lip and palate </li></ul></ul><ul><ul><li>Developmental abnorma...
Incidence <ul><li>Cleft lip and palate is 1 in 600 live births. </li></ul><ul><li>Isolated cleft palate is 1 in 1000 live ...
Aetiology <ul><li>Genetic predisposition </li></ul><ul><ul><li>Fx of cleft lip and palate in 1 st  degree relatives increa...
Classification <ul><li>LAHSHAL system : describes site, size, extent and type of cleft. </li></ul><ul><li>eg. </li></ul><u...
Cleft Lip <ul><li>Anatomy: </li></ul><ul><li>Frontal view showing muscle chain of the face </li></ul><ul><ul><li>A) nasola...
Cleft lip <ul><li>Types: </li></ul><ul><li>Unilateral cleft lip: </li></ul><ul><ul><li>Nasolabial and bilabial muscle ring...
Cleft palate <ul><li>Embryologically: </li></ul><ul><ul><li>1ry palate : structures ant to incisive foramen (alveolus and ...
Soft Palate <ul><li>Muscle fibers of the soft palate are oriented  transversely  with no significant attachment to the har...
Hard Palate <ul><li>Anatomy: </li></ul><ul><ul><li>1-  Palatal fibromucosa:  lies directly below the floor of the nose. </...
Types   <ul><li>Incomplete:  when the cleft of the hard palate remains attached to the nasal septum and vomer. </li></ul><...
Primary management <ul><li>Antenatal diagnosis: </li></ul><ul><ul><li>cleft lip could be diagnosed by US after week 18 of ...
Cont.   <ul><li>Surgical techniques: </li></ul><ul><li>Cleft lip </li></ul><ul><ul><li>Principle : attach and reconnect th...
Cont. <ul><li>Surgical techniques: </li></ul><ul><li>Cleft palate </li></ul><ul><ul><li>Principle : mobilization and recon...
Cont. <ul><li>Delaire technique and sequence is one the regimens used for timing of primary cleft lip and palate procedure...
Secondary management <ul><li>Hearing  </li></ul><ul><ul><li>Higher incidence of sensorinueral hearing loss and conductive ...
Secondary surgery for cleft lip and palate <ul><li>Includes: </li></ul><ul><ul><li>Cleft lip revision </li></ul></ul><ul><...
Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve ...
Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve ...
Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve ...
Alveolar bone graft <ul><li>Done in patients with residual alveolar cleft ass with cleft lip and palate </li></ul><ul><li>...
Orthognathic surgery <ul><li>Impaired growth of maxilla is due to </li></ul><ul><ul><li>poor and traumatic 1ry surgery. </...
Developmental abnormalities of the jaw <ul><li>Disproportionate growth between the maxilla and the mandible resulting in d...
Developmental abnormalities of the teeth <ul><li>Divided into: </li></ul><ul><ul><li>Abnormality in number </li></ul></ul>...
Number <ul><li>Anodontia:  congenital absence of all teeth. Rare </li></ul><ul><li>Partial anodontia : failure of developm...
Defects of structure of the teeth <ul><li>Causes:  </li></ul><ul><li>Genetic disorders: </li></ul><ul><ul><li>Amelogenesis...
Disorders of eruption <ul><li>Delayed eruption of teeth may involve a single tooth or the entire dentition </li></ul><ul><...
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Congenital defects of the Face

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Congenital defects of the Face

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Transcript of "Congenital defects of the Face"

  1. 2. Introduction <ul><li>Includes: </li></ul><ul><ul><li>Cleft lip and palate </li></ul></ul><ul><ul><li>Developmental abnormalities of the jaw and teeth </li></ul></ul><ul><li>Cleft lip, alveolus,hard and soft palate are the most common congenital abnormalities of the orofacial structures. </li></ul>
  2. 3. Incidence <ul><li>Cleft lip and palate is 1 in 600 live births. </li></ul><ul><li>Isolated cleft palate is 1 in 1000 live births. </li></ul><ul><li>The typical distribution of cleft types is: </li></ul><ul><ul><li>Cleft lip alone 15% </li></ul></ul><ul><ul><li>Cleft lip and palate 45% </li></ul></ul><ul><ul><li>Isolated cleft palate 40% </li></ul></ul><ul><li>Cleft lip palate predominates in male </li></ul><ul><li>Cleft lip alone more in females </li></ul><ul><li>In unilateral cleft lip, the deformity affects left side in 60% of cases. </li></ul>
  3. 4. Aetiology <ul><li>Genetic predisposition </li></ul><ul><ul><li>Fx of cleft lip and palate in 1 st degree relatives increases the risk to 1 in 25 cases. </li></ul></ul><ul><li>Environmental factors: </li></ul><ul><ul><li>Maternal epilepsy </li></ul></ul><ul><ul><li>Drugs: steroids, phenytoin, diazepam </li></ul></ul><ul><li>Associated with syndromes ( mostly isolated cleft palate) </li></ul><ul><ul><li>Pierre Robin sequence (Isolated cleft palate, retrognathia, posteriorly displaced tongue) </li></ul></ul><ul><ul><li>Stickler syndrome ( ophthalmic & musculoskeletal abnormalities) </li></ul></ul><ul><ul><li>Downs syndrome </li></ul></ul>
  4. 5. Classification <ul><li>LAHSHAL system : describes site, size, extent and type of cleft. </li></ul><ul><li>eg. </li></ul><ul><ul><li>LAHSHAL = complete bilateral cleft lip and palate. </li></ul></ul><ul><ul><li>lahSh = incomplete right unilateral cleft lip and alveolus with complete cleft of the soft palate extending partially onto hard palate. </li></ul></ul>
  5. 6. Cleft Lip <ul><li>Anatomy: </li></ul><ul><li>Frontal view showing muscle chain of the face </li></ul><ul><ul><li>A) nasolabial muscles </li></ul></ul><ul><ul><li>1- transverse nasalis </li></ul></ul><ul><ul><li>2- levator labii superioris alaeque nasi </li></ul></ul><ul><ul><li>3-levator labii superioris </li></ul></ul><ul><ul><li>B) bilabial muscles orbicularis oris </li></ul></ul><ul><ul><li>4- oblique head </li></ul></ul><ul><ul><li>5- horizontal head </li></ul></ul><ul><ul><li>6- lower lip </li></ul></ul><ul><ul><li>C) labiomental muscles </li></ul></ul><ul><ul><li>7- depressor anguli oris </li></ul></ul><ul><ul><li>8- depressor labii inferioris </li></ul></ul><ul><ul><li>9- mantalis </li></ul></ul>
  6. 7. Cleft lip <ul><li>Types: </li></ul><ul><li>Unilateral cleft lip: </li></ul><ul><ul><li>Nasolabial and bilabial muscle rings are disrupted on one side </li></ul></ul><ul><ul><li>resulting in asymmetrical deformity involving external nasal cartilage, nasal septum, and maxilla </li></ul></ul><ul><ul><li>causing displacement of nasal skin onto the lip and retraction of labial skin. </li></ul></ul><ul><li>Bilateral cleft lip: </li></ul><ul><ul><li>Symmetrical deformity </li></ul></ul><ul><ul><li>Two muscular rings are disrupted on both sides. </li></ul></ul>
  7. 8. Cleft palate <ul><li>Embryologically: </li></ul><ul><ul><li>1ry palate : structures ant to incisive foramen (alveolus and upper lip) </li></ul></ul><ul><ul><li>2ry palate : structures post to incisive foramen (hard and soft palate) </li></ul></ul><ul><li>Cleft palate results from failure of fusion of the two palatine shelves </li></ul>
  8. 9. Soft Palate <ul><li>Muscle fibers of the soft palate are oriented transversely with no significant attachment to the hard palate. </li></ul><ul><li>In cleft soft palate, muscle fibers are orientated in an anteroposterior direction , inserting into the posterior edge of the hard palate </li></ul>
  9. 10. Hard Palate <ul><li>Anatomy: </li></ul><ul><ul><li>1- Palatal fibromucosa: lies directly below the floor of the nose. </li></ul></ul><ul><ul><li>2- Maxillary fibromucosa : contains the greater palatine neurovascular bundle. </li></ul></ul><ul><ul><li>3- Gingival fibromucosa : adjacent to the teeth. </li></ul></ul>
  10. 11. Types <ul><li>Incomplete: when the cleft of the hard palate remains attached to the nasal septum and vomer. </li></ul><ul><li>Complete : when the nasal septum and the vomer are completely separated from the palatine process </li></ul>
  11. 12. Primary management <ul><li>Antenatal diagnosis: </li></ul><ul><ul><li>cleft lip could be diagnosed by US after week 18 of gestation </li></ul></ul><ul><ul><li>Cleft palate cannot be diagnosed on antenatal scan </li></ul></ul><ul><li>Feeding: </li></ul><ul><ul><li>Soft teats and modified teats </li></ul></ul><ul><ul><li>Enlarging the hole in the teat. </li></ul></ul>
  12. 13. Cont. <ul><li>Surgical techniques: </li></ul><ul><li>Cleft lip </li></ul><ul><ul><li>Principle : attach and reconnect the muscles around nasal aperture and oral sphincter </li></ul></ul><ul><ul><li>Performed btw 3- 6 months </li></ul></ul><ul><ul><li>Skin incisions to restore displaced tissues including skin and cartilage to their normal position </li></ul></ul><ul><ul><li>Nasolabial muscles are anchored to the premaxilla </li></ul></ul><ul><ul><li>Oblique muscles of orbicularis oris are sutured to the base of the ant nasal spine </li></ul></ul><ul><ul><li>Closure is completed by suturing the horizontal fibers of orbicularis oris. </li></ul></ul>
  13. 14. Cont. <ul><li>Surgical techniques: </li></ul><ul><li>Cleft palate </li></ul><ul><ul><li>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 </li></ul></ul><ul><ul><li>Cleft palate closure can be achieved by one or two stage palatoplasty </li></ul></ul>
  14. 15. Cont. <ul><li>Delaire technique and sequence is one the regimens used for timing of primary cleft lip and palate procedures: </li></ul><ul><li>Cleft lip alone: </li></ul><ul><ul><li>Unilateral : one operation </li></ul></ul><ul><ul><ul><ul><ul><li>at 5-6 months </li></ul></ul></ul></ul></ul><ul><ul><li>Bilateral : one operation </li></ul></ul><ul><ul><ul><ul><ul><li>at 4-5 moths </li></ul></ul></ul></ul></ul><ul><li>Cleft palate only: </li></ul><ul><ul><li>Soft palate only : one operation, </li></ul></ul><ul><ul><ul><ul><ul><li>at 6month </li></ul></ul></ul></ul></ul><ul><ul><li>Soft and hard palate : two operations , </li></ul></ul><ul><ul><ul><ul><ul><li>soft at 6 months, </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>hard at 15- 18 months </li></ul></ul></ul></ul></ul><ul><li>Cleft lip and palate: </li></ul><ul><ul><li>Unilateral : two operations : </li></ul></ul><ul><ul><ul><ul><ul><li>cleft lip and soft palate at 5-6months </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Hard palate at 15- 18months </li></ul></ul></ul></ul></ul><ul><ul><li>Bilateral : two operations : </li></ul></ul><ul><ul><ul><ul><ul><li>cleft lip and soft palate at 4-5 months </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Hard palate at 15- 18 months </li></ul></ul></ul></ul></ul>
  15. 16. Secondary management <ul><li>Hearing </li></ul><ul><ul><li>Higher incidence of sensorinueral hearing loss and conductive hearing loss </li></ul></ul><ul><ul><li>Regular hearing tests should be performed before 12 months of age </li></ul></ul><ul><li>Speech: </li></ul><ul><ul><li>Velopharyngeal incompetence </li></ul></ul><ul><ul><li>Articulation problems </li></ul></ul><ul><ul><li>Speech problems </li></ul></ul><ul><li>Dental: </li></ul><ul><ul><li>Hypodontia, hyperdontia, delayed eruption of teeth are common problems </li></ul></ul><ul><ul><li>Management include dietary advice, fluoride supplements, and fissure sealants. </li></ul></ul>
  16. 17. Secondary surgery for cleft lip and palate <ul><li>Includes: </li></ul><ul><ul><li>Cleft lip revision </li></ul></ul><ul><ul><li>Alveolar bone graft </li></ul></ul><ul><ul><li>Simultaneous lip revision and alveolar bone graft </li></ul></ul><ul><ul><li>2ry palate procedures </li></ul></ul><ul><ul><li>Dentoalveolar procedure </li></ul></ul><ul><ul><li>Rhinoplasty </li></ul></ul><ul><ul><li>Orthognathic surgery </li></ul></ul>
  17. 18. Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve incomplete 1ry reconstruction </li></ul><ul><li>Indications: </li></ul><ul><ul><li>Lip deformities: </li></ul></ul><ul><ul><li>Misaligned vermilion </li></ul></ul><ul><ul><li>Asymmetrical cupid’s bow </li></ul></ul><ul><ul><li>Muscle discontinuity or misalignment </li></ul></ul><ul><ul><li>Nasal deformity : </li></ul></ul><ul><ul><li>Lateral drift of alar base </li></ul></ul><ul><ul><li>Poor nasal tip projection </li></ul></ul><ul><ul><li>Deviated nasal septum </li></ul></ul>
  18. 19. Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve incomplete 1ry reconstruction </li></ul><ul><li>Indications: </li></ul><ul><ul><li>Lip deformities: </li></ul></ul><ul><ul><li>Misaligned vermilion </li></ul></ul><ul><ul><li>Asymmetrical cupid’s bow </li></ul></ul><ul><ul><li>Muscle discontinuity or misalignment </li></ul></ul><ul><ul><li>Nasal deformity : </li></ul></ul><ul><ul><li>Lateral drift of alar base </li></ul></ul><ul><ul><li>Poor nasal tip projection </li></ul></ul><ul><ul><li>Deviated nasal septum </li></ul></ul>
  19. 20. Cleft lip revision <ul><li>Should be delayed for at least 2 years after the 1ry surgery </li></ul><ul><li>Aims to improve incomplete 1ry reconstruction </li></ul><ul><li>Indications: </li></ul><ul><ul><li>Lip deformities: </li></ul></ul><ul><ul><li>Misaligned vermilion </li></ul></ul><ul><ul><li>Asymmetrical cupid’s bow </li></ul></ul><ul><ul><li>Muscle discontinuity or misalignment </li></ul></ul><ul><ul><li>Nasal deformity : </li></ul></ul><ul><ul><li>Lateral drift of alar base </li></ul></ul><ul><ul><li>Poor nasal tip projection </li></ul></ul><ul><ul><li>Deviated nasal septum </li></ul></ul>
  20. 21. Alveolar bone graft <ul><li>Done in patients with residual alveolar cleft ass with cleft lip and palate </li></ul><ul><li>Aim: </li></ul><ul><ul><li>promotion of eruption of canine tooth into the cleft site </li></ul></ul><ul><ul><li>Eliminates oronasal fistula </li></ul></ul><ul><li>Best performed between 8- 11 years </li></ul>
  21. 22. Orthognathic surgery <ul><li>Impaired growth of maxilla is due to </li></ul><ul><ul><li>poor and traumatic 1ry surgery. </li></ul></ul><ul><ul><li>Genetic predisposition </li></ul></ul><ul><li>Principle of deformity: underdevelopment in both horizontal and vertical direction of maxilla leading to pseudoprognathism </li></ul><ul><li>(A projection of the mandible due to occlusal disharmonies that force the mandible forward; the mandibular condyles are forward of their expected functional position) </li></ul><ul><li>surgery is performed when facial growth is completed( female 16 years, male 19 years) </li></ul>
  22. 23. Developmental abnormalities of the jaw <ul><li>Disproportionate growth between the maxilla and the mandible resulting in derangement of the dental occlusion </li></ul><ul><li>Classification: </li></ul><ul><ul><li>Class I : theres normal relation between upper and lower incisors and molar dentition </li></ul></ul><ul><ul><li>Class II : the mandibular teeth are placed post to the maxillary teeth </li></ul></ul><ul><ul><li>Class III : the mandibular teeth are placed ant to the maxillary teeth. </li></ul></ul><ul><li>Management: orthognathic surgery </li></ul>
  23. 24. Developmental abnormalities of the teeth <ul><li>Divided into: </li></ul><ul><ul><li>Abnormality in number </li></ul></ul><ul><ul><li>Defects of structure and size </li></ul></ul><ul><ul><li>Disorders of eruption of teeth </li></ul></ul>
  24. 25. Number <ul><li>Anodontia: congenital absence of all teeth. Rare </li></ul><ul><li>Partial anodontia : failure of development of 1ry or more commonly 2ry dentition </li></ul><ul><li>Most frequent absent teeth: </li></ul><ul><ul><li>Third molar ( wisdom teeth) </li></ul></ul><ul><ul><li>2 nd premolars </li></ul></ul><ul><ul><li>Maxillary lateral incisor teeth </li></ul></ul><ul><li>Ass with: </li></ul><ul><ul><li>Ectodermal dysplasia </li></ul></ul><ul><ul><li>Down syndrome </li></ul></ul><ul><ul><li>Cleft lip and palate </li></ul></ul><ul><li>Management: prosthetic replacement of teeth </li></ul>
  25. 26. Defects of structure of the teeth <ul><li>Causes: </li></ul><ul><li>Genetic disorders: </li></ul><ul><ul><li>Amelogenesis imperfecta : changes in the structure (hypoplasia) or mineralization ( hypocalcification) </li></ul></ul><ul><ul><li>Dentinogenesis imperfecta : ass with soft dentine with short roots. Usually ass with osteogenesis imperfecta </li></ul></ul><ul><li>Systemic causes: </li></ul><ul><ul><li>Measels </li></ul></ul><ul><ul><li>Rickets </li></ul></ul><ul><ul><li>Hypoparathyroidism </li></ul></ul><ul><ul><li>Tetracycline </li></ul></ul>
  26. 27. Disorders of eruption <ul><li>Delayed eruption of teeth may involve a single tooth or the entire dentition </li></ul><ul><li>Local factors: </li></ul><ul><ul><li>Loss of space/ overcrowding </li></ul></ul><ul><ul><li>Additional teeth </li></ul></ul><ul><ul><li>Retention of deciduous tooth </li></ul></ul><ul><li>Systemic factors: </li></ul><ul><ul><li>Matabolic diseases: critinism and rickets </li></ul></ul><ul><ul><li>Osteodystrophies </li></ul></ul><ul><ul><li>Hereditary gingival fibromatosis </li></ul></ul><ul><li>Management: </li></ul><ul><ul><li>Remove any obstruction </li></ul></ul><ul><ul><li>Can be left alone </li></ul></ul>
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