Developmental disorders of orofacial structures dental oral pathology

1. Developmental disorders of
orofacial structures

2. Orofacial Clefts
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Cleft Lip (CL)
Cleft Palate ( CP )
Cleft Lip with Cleft Palate (CL + CP)
Lateral Facial Cleft
Oblique Facial Cleft
Median Cleft of upper lip
Median Maxillary anterior alveolar cleft.

3. Etiology
• Genetic abnormalities
• Inherited
• Spontaneous mutation
• Environmental factors
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Nutritional Deficiency
Cigarette smoking
Drugs, radiation.
Amniotic bands

4. Cleft Lip and Palate
• Most common among the facial clefts.
• Cleft is a division or separation of parts of
the lip or palate that is formed during the
early months of development of the fetus.
• Clefts may be unilateral or bilateral.
• They can vary in severity.
• 3-8% of clefts are associated with
syndromes

5. Unilateral incomplete cleft lip

6. Unilateral Complete Cleft lip

7. Bilateral Complete Cleft Lip

8. Frequency of Occurrence
• In whites,CL ± CP occur in 1 of every 700-1000
births
• In Asians,prevalence is 1.5 times higher.
• In African Americans,less than 0.4 in 1000.
• in Native Americans fequency is 3.6per 1000birth
• Incidence of clefts is highest in Native American
population and lowest in African Americans

9. Frequency of occurrence
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Isolated Cleft Lip :
25%
Isolated Cleft Palate :
30%
Cleft Lip + Cleft Palate : 45%
CL ± CP : more common in males.
For isolated CL , M : F = 1.5 : 1
For CL + CP, M : F = 2 : 1
Isolated CP is more common in females.

10. Location
• Cleft Lip is more commonly unilateral
(80%)
• 70% of cleft lips on left side.
• Complete CL extends upward into nostril.
• CP may involve hard and soft palate or soft
palate alone.
• Cleft or Bifid Uvula is more common.

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Bifid Uvula:
Whites: 1 in 80
Asian and Native American: 1 in 10
African Americans: 1 in 250
Submucous palatal cleft : surface mucosa is
intact but underlying musculature of soft
palate is defective.

12. Complete cleft palate

13. Bifid uvula

14. Submucous palatal cleft

15. Pierre Robin Syndrome
• Triad of :
• Mandibular micrognathia
• Glossoptosis
• Cleft Palate.

16. Bird facies

17. Problems associated with clefts
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Esthetic disfigurement
Difficulty in breathing ,feeding and speech
Malocclusion.
Psychosocial problems.
Recurrent upper respiratory tract infections.

18. Treatment
• Multidisciplinary approach
• Plastic surgery,
Dentistry,Otolaryngology,Audiology,speec
h pathology,Genetics and Pediatrics
• SURGICAL REPAIR.

19. Cleft lip repair
• Objective: Closure of cleft to create a
pleasing face that will develop normally
with minimum scarring
• Performed by plastic surgeon when baby is
approx. 3 months of age and weighs atleast
10 lbs.

20. • Surgery under GA( takes approx 2-3 hours)
• Special considerations in positioning and
feeding.
• Takes approx. 3 weeks for the wound to
gain enough strength.
• The lip scar is initially red and swollen but
begins to mature and improve in appearance
in 6-12 months.
• In Bilateral cleft lip , repair in stages.
• In alveolar cleft, bone graft required ( 5-10
yrs)

21. Cleft palate repair
• Objective: To close the palate to restore
normal function of eating and drinking and
to enhance the development of normal
speech.
• Best accomplished in one surgical
procedure before the child reaches 12-14
months of age.

22. Pierre Robin syndrome
• Infant placed in prone position until
adequate growth of jaws occur.
• Tongue- Lip adhesion/Tracheostomy
• Mandibular bone expansion- Distraction
Osteogenesis with an expansion device.

23. Distraction osteogenesis

24. After removal of the expansion device

25. Late cleft treatment
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Cleft palate surgery
Intensive speech therapy regimen
Orthognathic surgery.
Orthodontic correction.

26. Genetic counseling
• In Non syndromic cases, risk of
Cleft development in a sibling or
offspring is 3-5% if no other first
degree relative is affected. It
increases to 10 – 20 % if another
first degree relative is affected

27. Developmental disorders of Lips
• Lip Pits:
• Para median Lip Pits
• Commissural Lip Pits
• Double Lip

28. Paramedian Lip Pits
• Rare
• Autosomal dominant
inheritance
• Persistence of lateral
sulci on embryonic
mandibular arch
• Bilateral symmetric
fistulas on either side
of the midline

29. Paramedian Lip Pits
• Appearance varies from
subtle depressions to
prominent humps.
• Pits can extend to a depth
of 1.5 cm and may express
salivary secretions
• Seen in Van der Woude
syndrome with CL±CP
• Surgical excision for
cosmetic reasons

30. Commissural Lip Pit
• Small mucosal
invaginations at
corners of the mouth
on vermilion border.
• Failure in the normal
fusion of maxillary
and mandibular
processes during
development

31. Commissural Lip Pit
• Seen in 12-20 % of
adult population.
• Males > Females
• Unilateral / Bilateral
• May be associated
with preauricular pits
• No treatment required

32. Double Lip
• Rare
• Redundant fold of
tissue on the mucosal
side of the lip.
• May be Congenital or
acquired( from trauma
or oral habits such as
lip sucking)

33. Double lip
• More common in
upper lip
• Sometimes both
lips affected
• Seen on smiling.
• Feature of Ascher’s
syndrome

34. Ascher’s syndrome
• Triad of :
• Double lip
• Blepharochalasis
• Nontoxic enlargement of thyroid gland

35. Developmental disorders of tongue
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Macroglossia
Microglossia
Ankyloglossia
Lingual Thyroid
Median Rhomboid glossitis
Benign Migratory glossitis
Hairy Tongue
Bifid tongue
Fissured tongue
Lingual Varices

36. Macroglossia

37. Causes
• Congenital:
• Acquired:
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Haemangioma
Lymphangioma
Cretinism
Down syndrome
Beckwith Weidemann
Syndrome
• Neurofibromatosis
• Hemi hyperplasia
Amyloidosis
Myxoedema
Acromegaly
Angioedema
Carcinoma and other
tumours
• Edentulous state

38. Differentiating features
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Cretinism and Beckwith Weidemann Syn:
Smooth, diffuse generalized enlargement
Lymphangioma:
Pebbly appearance with multiple vesicle
like blebs
• Amyloidosis, Neurofibromatosis:
• Multinodular appearance

39. Differentiating features
• Down Syndrome:
• Papillary fissured surface
• Hemifacial Hyperplasia,
Neurofibromatosis:
• Unilateral enlargement
• Edentulous:
• Lateral spreading of tongue.

40. Microglossia
• Oromandibular- limb hypogenisis syndrome
• Frequently Seen along with hypoplasia of mandible

41. Ankyloglossia
Anomaly characterized
by short thick lingual
frenum resulting in
restriction in tongue
movement.
Sever case tongue fused
to floor of mouth
Frenum some time
attached to tip of the
tongue

42. Ankyloglossia
• Seen in 2-5 % of the
population
• Males > Females
• May be partial or
complete
• May cause speech
defects.
• Surgery if the
anomaly is severe

43. Lingual Thyroid-origin
• Failure of primitive
thyroid gland to
descend into the neck
during development.
• About 10 % of both
men and women may
show thyroid tissue if
biopsy is taken from
posterior part of
tongue.

44. Lingual Thyroid-Clinical
features
• Symptomatic and
clinically evident
lingual thyroid seen
less commonly.
• 4 - 7 times more
common in females
• May cause dyspnoea,
dysphonia, dysphagia

45. Lingual Thyroid-Diagnosis
• In 70% of cases this
ectopic gland is the
patient’s only thyroid
tissue.
• Thyroid scans using
Iodine isotopes or
technetium 99m
• CT and MRI to know
the size and extent

46. Lingual Thyroid-Treatment
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Asymptomatic patients:
No treatment, Periodic follow up
Symptomatic patients:
Suppressive therapy with thyroid supplements
In case of airway obstruction,surgery with auto
transplantation to another body site.
• In males over 30 years ,prophylactic surgical
excision as there is a risk of malignancy.

47. Median Rhomboid glossitis

48. Benign Migratory glossitis

49. Hairy Tongue
• Accumulation of keratin on the filiform papillae
may be due to increased production of keratin or
decrease in the normal keratin desquamation

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Etiology
Antibiotic therapy
Poor oral hygiene
General debilitation
Radiation therapy
Use of oxidizing mouth wash or antacid
Overgrowth of fungus or bacterial organism
Histopathology- it is characterized by
marked elongation and hyper keratosis of
filiform papillae

51. Fissured Tongue
• Multiple grooves and furrows on the surface of the tongue
• Strong association noted between geographic tongue
• Seen some time in Melkersson Rosenthal syndrome

52. Lingual Varices
• Abnormally dilated
and tortuous veins
• Seen in 2/3rds of
people older than 60
years of age(indicating
age related
degeneration )
• Asymptomatic

53. Developmental disorders of the jaws
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Exostoses and Tori
Hemifacial atrophy
Hemifacial hypertrophy
Condylar hypertrophy
Coronoid hypertrophy
Mandibular aplasia

54. Mandibular Torus

55. Palatine Torus

56. Hemifacial Atrophy

57. Hemifacial hypertrophy

58. Condylar hypertrophy

59. Coronoid hypertrophy

60. Mandibular aplasia

61. Thank You