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Syndromes /certified fixed orthodontic courses by Indian dental academy


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Syndromes /certified fixed orthodontic courses by Indian dental academy

  1. 1. Malocclusions associated with syndromes INDIAN DENTAL ACADEMY Leader in continuing dental education
  2. 2. What is a syndrome? An anamolad is a malformation together with its subsequently derived structural changes,the primary defect setting off a series of secondary or even tertiary events resulting in multiple anamolies. Syndrome is defined as a set of signs or a series of events occuring together that often point to a single disease or condition as a
  3. 3. Genetic syndromes: A relatively small number of orthodontic patients are affected by known genetic syndromes that affect oral structures. By far, the most common orthodontic problem that may be a part of a genetic syndrome is cleft lip and palate.However there are many causes for facial clefting conditions and not all of the causes can be traced to
  4. 4. The greatest value in knowing that a patient has a particular syndrome is that it allows much better prediction of future development in the individual who will not grow in the normal pattern. Sometimes recognizing a syndrome is made more difficult by incomplete expression of the gene or genes involved – called partial penetrance.
  5. 5. If a genetic syndrome is suspected the orthodontist should have the patient evaluated through a clinic that specializes in such matters. A patient who has anamolous teeth should be examined carefully for ear, hand or other deformities that may indicate a genetic
  6. 6. Genetic influences: It is widely acknowledged that malocclusions have a genetic component.Because of the polygenic inheritance of craniofacial and dental characters,it is unlikely that simple methods would reveal the genetic component of most malocclusions. Studies of twins and of triplets have shown a high concordance of dentofacial traits in monozygotic individuals. Skeletal variations seemed to be more influenced my heredity and dental variations seemed to be influenced by the environment.
  7. 7. Syndromes occurring commonly with malocclusions are classified as: (Cohen,Proffit,Bell,White ) •Malformation syndromes associated with mandibular deficiency. •Malformation syndromes associated with mandibular prognathism. •Malformation syndromes associated with problems of facial height. •Malformation syndromes associated with facial
  8. 8. Malformation syndromes associated with mandibular deficiency: Robin complex: Etiology: Heterogenous ,considered a sporadic or non genetic condition with a very low recurrence risk in the family. Striking features: Micrognathia,cleft palate and glossoptosis. Ensuing hypoplasia of mandible producing the characteristic bird
  9. 9. Pierre Robin syndrome can occur solely or in combination with other syndromes like the Stickler syndrome, cerebrocostomandibular syndrome,the camptomelic syndrome and the persistent left superior vena cava syndrome.
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  11. 11. Treacher Collins syndrome: ( Mandibulo facial dysostosis; Franceschetti syndrome ) Etiology: The Treacher Collins syndrome encompasses a group of closely related defects of the head and face , often hereditary or familial in pattern following an irregular form of dominant transmission. ( Autosomal dominant ). Striking features: Symmetrically hypoplastic low set ears, down slanting palpebral fissures, micrognathia,sometimes cleft palate.
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  13. 13. Hypoplasia of facial bones especially the malar bones and mandible , macrostomia,blind fistulas between the angles of the mouth and the angles of the ears are certain features. The syndrome is thought to result from a retardation or failure of differentiation of maxillary mesoderm at and after the 50 mm stage of the embryo.
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  16. 16. Nager acrofacial dysostosis: Etiology: Autosomal recessive trait – hereditary. Striking features: Symmetrically hypoplastic ears , down slanting palpebral fissures, micrognathia, cleft lip and palate in some cases,pre axial upper limb deficiency.
  17. 17. Wilder vanck Smith syndrome: Etiology – Unknown; all cases to date sporadic. Striking features: Symmetrically hypoplastic ears , down slanting palpebral fissures, micrognathia, cleft lip and palate in some cases; limb reduction defects of upper and lower
  18. 18. Hemifacial microsomia ( Goldenhar syndrome ). Etiology: Most cases are sporadic,few familial instances in which pedigrees are compatible with autosomal dominant or recessive inheritance. Striking features: Unilateral or bilateral asymmetrically hypoplastic ears and mandibular ramus;ear tags and/or pits, micrognathia, variably cleft lip or palate;equibulbar dermoids; vertebral anamolies;cardiac defects;renal anamolies;
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  21. 21. Mobius syndrome: Etiology: Unknown ;almost all cases sporadic; rare familial instances. Striking features: Bilateral sixth and seventh nerve palsy and variably other cranial nerve involvement;high broad nasal bridge;epicanthic folds;micrognathia ; talipes equinovarus ; limb reduction defects; mental
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  23. 23. Mobius syndrome is otherwise called as congenital facial diplegia.It is usually manifested in infancy during the first few days of life by failure to close the eyes during sleep.Because of partial or complete paralysis the infant exhibits no change in facial expression even while crying or laughing. The prominent lips are often everted and the mouth may remain partially opened.
  24. 24. Hallermann – Streiff syndrome Etiology: Unknown , all cases to dte sporadic. Striking features:- Dyscephaly; hypotrichosis,congenital cataracts,beaked nose, micrognathia, anteriorly placed mandibular condyles,natal teeth ; oligodontia; short stature
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  26. 26. Malformation syndromes associatred with mandibular prognathism. Basal cell nevus syndrome ( Gorlin Goltz syndrome). Etiology: Autosomal dominant – hereditary. Striking features – Macrocephaly ; frontal and biparietal bossing;dystopia canthorum or ocular hypertelorism; mild mandibular prognathism;jaw cysts ; multiple basal cell carcinomas; bifid ribs; spina bifida occulta ; short fourth
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  29. 29. Klinefelter syndrome: Etiology: Commonly xxy karyotype, but xxxy and xxxxy also occur. Striking features: Skeletal disproportion; small testes; increased urinary gonadotropins ; gynaecomastia and mental deficiency in some cases, mandibular prognathism.Prognathism increases as number of x chromosomes increases.
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  32. 32. Marfan syndrome: Etiology: Autosomal dominant. Defect of fibrillin. Striking features: Marfanoid habitus; dolichostenomedia; arachnodactyly; ectopia lentis ; fusiform and dissecting aneurysms of aorta ; mandibular prognathism.
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  34. 34. Osteogenesis imperfecta; Etiology:Autosomal dominant ( common type ) Etiologically heterogenous. Striking features: Fragile bones;blue sclerae;deafness;loose ligaments;dentinogenesis imperfecta like tooth condition ; mandibular prognathism.
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  37. 37. Waardenburg syndrome: Etiology: Autosomal dominant ( common type ); etiologically heterogenous with three other types known. Striking features: White forelock ; dystopia canthorum ; heterochromia irides , synophyrs , mild hypoplasia of alar cartilages ; mild mandibular prognathism; sensorineural deafness.
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  39. 39. Malformation syndromes associated with problems of facial height. Amelogenesis imperfecta: Etiology: heterogenous ; several modes of inheritance depending upon type of enamel defect and family history.
  40. 40. Striking features: Discolored teeth; hypomaturation, hypoplasia or hypocalcification of enamel ; anterior open bite.
  41. 41. Beckwith Weidemann syndrome: Etiology: Unknown; most cases sporadic ; few familial instances. Striking features: Macroglossia ; anterior open bite ; mandibular prognathism ; earlobe grooves; depressions on posterior rim of the ear;omphalocoele or umbilical hernia ; neonatal hypoglycemia; visceromegaly ; postsomatic gigantism; other
  42. 42. Malformation associated with facial asymmetry: Hemifacial microsomia ( goldenhar syndrome ) Hemifacial hypertrophy: Etilogy: Unknown , sporadic occurrence. Striking features: Hemihypertrophy involving head , limbs and body;exoression variable;hypertrophy involves both bone and soft tissue;tongue,mandible and teeth may be unilaterally enlarged,ocassionally associated with Wilms tumour,adrenocortical carcinoma or hepatoblastoma.
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  44. 44. Neurofibromatosis:( von recklinghausens disease) Etiology: Autosomal dominant. Striking features: Café au lait spots, neurofibromas,other hamartomas and neoplasms; skeletal defects ; endocrine disturbances; some patients have macrocephaly ,craniofacial asymmetry or hypertrophy of facial soft tissue.
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  46. 46. Romberg syndrome: Etiology: Unknown Striking features: Hemiatrophy involving muscle, bone and cartilage ( progressive ). Involves lip and tongue ; left side predilection;epilepsy in some cases;migraine in some cases;small percentage have body involvement.
  47. 47. Crouzons syndrome: The craniosynostosis syndromes constitute a group of conditions each characterized by premature craniosynostosis occuring in association with a variety of other abnormalities.These may or may not occur with syndactyly,anamolies of the hands and feet. The most common of the craniosynostotic syndromes occuring without syndactyly is Crouzon syndrome.
  48. 48. Majority of cases have followed an autosomal dominant trait. Clinical features: The signs are all basically due to synostosis of the sutures.The patients show a protruberant frontal region with an anteroposterior ridge overhanging the frontal eminence and often passing to the root of the nose.Hypoplasia of the maxillae and mandibular prognathism. Eye changes noted – hypertelorism,exopthalmos with divergent strabismus.
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  52. 52. Aperts syndrome: This is also a type of craniofacial dysostosis syndrome.Characteristic difference between Aperts and Crouzons syndrome is that Aperts syndrome involves syndactyly. Otherwise the features are the same as Crouzons.
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  57. 57. Cleido cranial dysostosis: Etiology: Sporadic. Clinical features: Characterized by abnormalities of the skull,teeth,jaws and shoulder girdle as well as by occasional stunting of the long bones. Patient with cleido cranial dysostosis characteristically exhibit a high narrow arched palate and actual cleft appears common.Underdeveloped maxilla.
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  59. 59. Downs syndrome: Trisomy 21 syndrome;Mongolism Downs syndrome is a disease associated with subnormal mentality in which an extremely wide variety of anomalies and functional disturbances may occur. Etiological factors: Advanced maternal age and uterine and placental abnormalities. Chromosomal aberration. – trisomy 21
  60. 60. Clinical features: Patients with Downs syndrome are characterised by flat face, a large anterior fontanel,open sutures,small slanting eyes with epicanthal folds,open mouth, frequent prognathism of mandible , sexual underdevelopment and cardiac abnormalities,hypermobility of joints. Oral findings: Macroglossia,fissured tongue,microdontia and enamel hypoplasia,destructive periodontal disease.
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  62. 62. Pfeiffer syndrome: Acrocephalosyndactyly.
  63. 63. Etiology: Genetic defect,mutation in fibroblast growth factor receptor 1 gene. Clinical features: flattened face with maxillary hypoplasia relative mandibular prognathism low set ears hypertelorism depressed nasal bridge high arched palate Short broad thumbs which are often malformed syndactyly of soft tissue of digits.
  64. 64. Conclusion: Malocclusions associated with syndromes should be treated in combination with various faculty departments involving surgeons, speech therapists, paediatricians, prosthetists and orthodontists. It is important for us to recognize the common syndromes enumerated above in order to bring about effective treatment which would benefit the society.
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