Delayed tooth eruption
Tooth eruption
Tooth eruption is the process by
which developing teeth emerge through
the soft tissue of the jaws and the
overlying mucosa to enter the oral cavity,
contact the teeth of the opposing arch,
and function in mastication
Phases of tooth eruption
• Preeruptive phase: All movements of primary and permanent
tooth germs (crowns) from time of their early initiation and
formation to the time of crown completion (ends with early initiation
of root formation)
• Eruptive phase: Starts with initiation of root formation and made
by teeth to move from its position within bone of the jaw to its
functional position in occlusion. Has an intraosseous and extraosseous
compartments. 4 stages: root formation, movement,
penetration and occlusal contact
• Posteruptive phase: Takes place after the teeth are functioning to
maintain the position of the erupted tooth in occlusion while the
jaws are continuing to grow and compensate for occlusal and proximal
tooth wear
Delay tooth eruption
A tooth is defined to have delayed
eruption when the concurrent tooth
in the adjacent quadrant has erupted
or when there is a delay of over 6
months after the defined normal
eruption time
Causes of delayed eruption
• Local Conditions
• Systemic Conditions
• Genetic conditions
Local Conditions associated with DTE
• Mucosal barriers-scar tissue: trauma/surgery
• Odontogenic tumors (eg, adenomatoid
odontogenic Tumors, odontomas)
• Ankylosis of deciduous teeth
• Radiation damage
• Oral clefts
Mucosal barriers-scar tissue:
trauma/surgery
• failure of the follicle of an erupting tooth to
unite with the mucosa
• Gingival hyperplasia resulting from various
causes might cause an abundance of dense
connective
• tissue or acellular collagen that can be an
impediment to tooth eruption
Odontogenic tumors
ODONTOMA
• calcified mass with
radiodensity of tooth
structure or tooth like
structure surrounded by a
narrow radiolucent zone
• maxilla > mandible
• asymptomatic
• combined with impacted
teeth lead to impaction of
the involved tooth
ADENOMATOID ODONTOGENIC
TUMOR
• 2/3 case 10 to 19 years of
age
• Females > males
• maxilla > mandible
• Radiographic 75% of cases,
as a circumscribed,
unilocular radiolucency
involves unerupted tooth
crown, most often a canine
• may appear completely
radiolucent or fine
calcifications (snowflake)
Ankylosis of deciduous teeth
• fusion of the cementum or dentin with the
alveolar bone
• most common local cause
• occurs commonly in the deciduous dentition,
• usually affecting the molars
• mandible > maxilla
• infraocclusion
Radiation damage
• Radiation therapy involves the head and neck
affects on the developing permanent teeth in
jawbone. These affects include delayed
eruption , fusion of the deciduous teeth in the
alveolar bone preventing the permanent teeth
from erupting.
• Abnormal development in humans has been
observed with a total dose as low as 400 cGy
Oral clefts
• CL ± CP occurs in 1 of every 700 to 1000
births.
• Frequently in Asian populations (1.5 times
higher than in whites)
• more common in males than in females
• May associated to Genetic disorder or not
Oral cleft
• GENETIC FACTORS
• Gene Locus
• SKI/MTHFR 1p36
• TGFB2 1q41
• TGFA 2p13
• MSX1 4p16, 4q31, 6p23
• PVRL1 1 1q23
• TGFB3 14q24
• GABRB3 15q11
• RARA 17q21
• BCL3 19q13
ENVIRONMENTAL FACTORS
● Maternal alcohol consumption
● Maternal cigarette smoking
● Folic acid defi ciency
● Corticosteroid use
● Anticonvulsant therapy
Nonsyndromic Orofacial Clefts
Systemic Conditions associated with
DTE
• Nutrition
• Vitamin D-resistant rickets
• Endocrine disorders (Hypothyroidism
,Hypopitutarism, Hypoparathyroidism,
pseudohypoparathyroidism)
• HIV infection
• Cerebral palsy
• Celiac disease
• Ichthyosis
Malnutrition
• Early malnutrition in childhood affects skeletal
growth and results in decreased height.
Similarly, poor nutrition affects tooth eruption
and results in delayed emergence of the
deciduous teeth.
Vitamin D-resistant rickets
• a group of metabolic disorders characterized b
y renal tubular defects in phosphate transport
and bone abnormalities resulting in
hypophosphatemic ricket
• teeth may not erupt until after one year.
When decido re they erupt, they may be
smaller than normal.
Endocrine disorders
• Disturbance of the endocrine glands usually
has a profound effect on the entire body,
including the dentition. Hypothyroidism,
hypopituitarism, hypoparathyroidism, and
pseudohypoparathyroidism are the most
common endocrine disorders associated with
DTE
Hypothyroidism (CRETINISM , MYXEDEMA)
• decreased levels of thyroid hormone
• may be classifi ed as either primary or
secondary
• In primary hypothyroidism > the thyroid
abnormal
• in secondary hypothyroidism > the pituitary
gland does not produce thyroid-stimulating
hormone (TSH) > thyroid gland not release
thyroid hormone.
Hypothyroidism
• lethargy, dry and coarse skin
• swelling of the face and extremities
• huskiness of the voice, constipation,
weakness, and fatigue
• Bradycardia
• Hypothermia
Hypothyroidism
• Oral & Dental manifestations
– Delayed eruption
– Enamel hypoplasia in both dentitions (being less
intense in the permanent dentition)
– Anterior open bite
– Macroglossia
– Micrognathia
– Thick lips
– Dysgeusia
– Mouth breathing
Hypopituitarism (PITUITARY DWARFISM)
• Affected patients are typically much shorter
than normal
• maxilla and mandible smaller than normal
• teeth show a delayed pattern of eruption,
delay ranges from 1 to 3 years for teeth that
normally erup
• lack of 3rd molars
Hypoparathyroidism
• Hypoparathyroidism is a metabolic disorder
characterized by hypocalcemia and
hypophosphatemia due to a deficiency or
absence of parathyroid hormone secretion
Hypoparathyroidism
• Dental manifestations
- Enamel hypoplasia in horizontal lines
- Poorly calcified dentin
- Widened pulp chambers
- Dental pulp calcifications
- Shortened roots
- Hypodontia
- Delay or cessation of dental development
Hypoparathyroidism
• Mandibular tori
• Chronic candidiasis
• Paresthesia of the tongue or lips
• Alteration in facial muscles
Pseudohypoparathyroidism
• Pseudohypoparathyroidism (PHP) is a
heterogeneous group of disorders characterized
by hypocalcemia, hyperphosphatemia, increased
serum concentration of parathyroid hormone
(PTH)
• Insensitivity to the biological activity of PTH
• may be considered an inborn error of
metabolism.
• inability of the body to respond appropriately to
parathyroid hormone (PTH)
Pseudohypoparathyroidism
• Dental manifestations
– enamel hypoplasia
– retarded root development with short blunted
roots
– larger root canals and distorted and thick dentin
at the apex
– Delayed eruption
HIV infection
• Hauk et al ,2001 found a correlation between
progression of HIV infection to pediatric AIDS
and delayed dental eruption and this delay is
most closely linked to severity of symptom not
CD4 depletion
Cerebral palsy
• Cerebral palsy is a disorder of movement and posture
caused by nonprogressive abnormality of the immature
brain that originates during the prenatal or perinatal
period or first few years of life. > significant impairment
of functional mobility.
• 4 major subtypes are
• Spastic (most common)
• dyskinetic/athetoid
• ataxic
• mixed cerebral palsy
Cerebral palsy
• Clinical Manifestations
– Intellectual disability (60% of patients)
– Seizure disorder (30-50% of patients)
– Delayed motor development
– Limb spasticity
– Persistent primitive reflexes
– Involuntary movements, and ataxia
Cerebral palsy
• Oral Manifestations
– risk for dental caries and periodontal disease
– Enamel hypoplasia
– Dental erosion
– Delayed eruption of permanent teeth
– Dilantin hyperplasia
– Increased incidence of Class II Div I malocclusion
– Increased risk for oral trauma and injury
Celiac disease
Celiac disease is a permanent intolerance to
gluten that results in damage to the small
intestinal mucosa caused by an autoimmune
mechanism in those who are genetically
susceptible to the disease. The villous atrophy
that ensues can lead to malabsorption of a
variety of macro- and micronutrients including
iron, calcium, folate and fat-soluble vitamins.
Celiac disease
• Oral and dental Manifestations
– Enamel defects
– Delayed eruption
– Recurrent aphthous ulcers
– Cheilosis
– Oral lichen planus
– Atrophic glossitis
Ichthyosis
• Ichthyosis is a heterogeneous family of
hereditary disorders mostly characterized by
the accumulation of hyperkeratotic scales on
the skin surface
Ichthyosis
• Oral and dental manifestation
– Periodontal disease
– Enamel hypoplasia
– high caries incidence
– delayed deciduous and permanent eruption
– bruxism
– bifid teeth
– irregular morphology of teeth
– hyperkeratotic plaques on the tongue.
Genetic conditions
• Amelogenesis imperfect
• Cherubism
• Cleidocranial dysplasia
• Cornelia de Lange Syndrome
• Down s yndrome
• Hurler syndrome
• Gorlin syndrome
• Osteopetrosis (marble bone disease)
Amelogenesis imperfect
• Amelogenesis imperfecta (AI) is a genetically
and clinically heterogeneous group of
inherited dental enamel defects.
• hypoplastic enamel
• Hypomineralised
• Eruption of permanent teeth is often delayed
and sometimes completely impaired
Cherubism
• autosomal dominant
disorder and with 100%
penetrance in males
and 50 to 75%
penetrance in females,
with 2:1 male
predominance
• Cystic, fibrous jaw
lesions
• Bilateral symmetric jaw
expansion
• Exposure of sclera
below the iris
• Aplasia of teeth
Cleidocranial dysplasia
• Caused by a defect in
the CBFA1 gene
• Large head with
prominent frontal
bones
• Wide sutures and
fontanelles
• Small face
• Aplasia/hypoplasia
of clavicles
• Supernumerary
teeth
• Delayed eruption
tooth
Cornelia de Lange Syndrome
• General features:
– microbrachycephaly
– synophrys/long eyelashes
– hand and limb anomalies
– delayed bone age
– abnormal speech pattern
– frequently do not express facial
expression
– low hairline on forehead and
neck R short, thick neck R
pigmented nevi on ski
• Oral manifestation :
– micrognathia
– down turned, thin lips
– cleft palate in 20%
– delayed tooth eruption and widely spaced teeth
– microdontia
– often severe dental behavior problem
Down s yndrome
• Incidence 1 in 600-700.
• Most cases of trisomy 21 are caused by non disjunction, resulting in
an extra chromosome
• General feature :
– brachycephaly/flat occiput
– upward slanting palpebral fissures/epicanthal folds
– brachydactyly/clinodactyly/simian crease
• Note: Aatlantoaxial instability (10-20%) and
• occipitoatlantal instability is seen in these
• patients. These features are important
• particularly in relation to cervical extension
• during OR procedures or during full restraint
• procedures.
Down s yndrome
• Oral manifestration
– Enamel hypoplasia (primary dentition)
– Periodontal disease (90%) and NUG (30%)
– Narrow and short palate
– Irregular and delayed dental eruption
– missing
– teeth (3rd M, 2nd M, Lat. Incisors) decreased
– calculus formation
• caries
• rate is low hypodontia
• taurodontism
• Malocclusion (crossbites and openbites common)
• Parotid salivary flow rate is decreased and increase Na+, Ca 2+, HCO -, and
uric acid
Down s yndrome
• caries
• rate is low hypodontia
• taurodontism
• Malocclusion (crossbites
and openbites common)
• Parotid salivary flow rate
is decreased and
• increase Na+, Ca 2+, HCO
-, and uric acid
Down s yndrome
• cardiac
• defect may be present requiring SBE
• prophylaxis
• lingual
• papillae are large protruding,
• large and fissured tongue
• Lips are broad, irregular fissured, and dry
Open mouth
Hurler syndrome
• Autosomal Recessiv (MPS I)
• (Mucopolysaccharidosis I-H)
• General features:
– macrocephaly/frontal
bossing
– course facies/thick ear lobes
and lips
– hepatosplenomegaly
– chest and spine deformities
• Oral manifastration:
– flattened philtrum
– Lip patulous
– widely spaced teeth
– lip and tongue enlargement after 5 years old -
incisors somewhat conical
– anterior open bite due to macroglossia - ectopic
molars in mandibular rami
Oral manifastration : (con)
– delayed eruption
– TMJ may exhibit limited motion
– dentigerous cysts
– airway obstruction
– sleep apnea reported
– mandible short and broad
– wide bigonial distance
Gorlin syndrome
• Gorlin syndrome is inherited as an autosomal
dominant trait with a high degree ofpenetrance
and variable expressivity.
• The disorder has a population prevalence of 1 per
57,000 people
• Male < female 3:1
• multiple odontogenic keratocysts
• mandibular prognathism
• and/or cleft lip and palate
• Delayed tooth eruprion
Osteopetrosis (marble bone disease)
• Rare hereditary bone disorder characterized
by increase in bone density due to defect in
bone remodeling caused by failure of normal
osteoclast function.
• Oral manifestations:
– Facial deformity leading to hypertelorism,
– snub nose, frontal bossing etc.
– Delayed tooth eruption and osteomyelitis of jaws.
– Sclerosis of skull bones leads to narrowing of foramina
which causes compression of various cranial nerves –
blindness, deafness, facial paralysis etc.
– The medullary spaces of the jaws are reduced.
– Fracture of jaws during extraction procedure can
occur without undue force, due to fragility of the bone

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delay tooth eruption

  • 2. Tooth eruption Tooth eruption is the process by which developing teeth emerge through the soft tissue of the jaws and the overlying mucosa to enter the oral cavity, contact the teeth of the opposing arch, and function in mastication
  • 3. Phases of tooth eruption • Preeruptive phase: All movements of primary and permanent tooth germs (crowns) from time of their early initiation and formation to the time of crown completion (ends with early initiation of root formation) • Eruptive phase: Starts with initiation of root formation and made by teeth to move from its position within bone of the jaw to its functional position in occlusion. Has an intraosseous and extraosseous compartments. 4 stages: root formation, movement, penetration and occlusal contact • Posteruptive phase: Takes place after the teeth are functioning to maintain the position of the erupted tooth in occlusion while the jaws are continuing to grow and compensate for occlusal and proximal tooth wear
  • 4. Delay tooth eruption A tooth is defined to have delayed eruption when the concurrent tooth in the adjacent quadrant has erupted or when there is a delay of over 6 months after the defined normal eruption time
  • 5. Causes of delayed eruption • Local Conditions • Systemic Conditions • Genetic conditions
  • 6. Local Conditions associated with DTE • Mucosal barriers-scar tissue: trauma/surgery • Odontogenic tumors (eg, adenomatoid odontogenic Tumors, odontomas) • Ankylosis of deciduous teeth • Radiation damage • Oral clefts
  • 7. Mucosal barriers-scar tissue: trauma/surgery • failure of the follicle of an erupting tooth to unite with the mucosa • Gingival hyperplasia resulting from various causes might cause an abundance of dense connective • tissue or acellular collagen that can be an impediment to tooth eruption
  • 8. Odontogenic tumors ODONTOMA • calcified mass with radiodensity of tooth structure or tooth like structure surrounded by a narrow radiolucent zone • maxilla > mandible • asymptomatic • combined with impacted teeth lead to impaction of the involved tooth
  • 9. ADENOMATOID ODONTOGENIC TUMOR • 2/3 case 10 to 19 years of age • Females > males • maxilla > mandible • Radiographic 75% of cases, as a circumscribed, unilocular radiolucency involves unerupted tooth crown, most often a canine • may appear completely radiolucent or fine calcifications (snowflake)
  • 10. Ankylosis of deciduous teeth • fusion of the cementum or dentin with the alveolar bone • most common local cause • occurs commonly in the deciduous dentition, • usually affecting the molars • mandible > maxilla • infraocclusion
  • 11. Radiation damage • Radiation therapy involves the head and neck affects on the developing permanent teeth in jawbone. These affects include delayed eruption , fusion of the deciduous teeth in the alveolar bone preventing the permanent teeth from erupting. • Abnormal development in humans has been observed with a total dose as low as 400 cGy
  • 12. Oral clefts • CL ± CP occurs in 1 of every 700 to 1000 births. • Frequently in Asian populations (1.5 times higher than in whites) • more common in males than in females • May associated to Genetic disorder or not
  • 13. Oral cleft • GENETIC FACTORS • Gene Locus • SKI/MTHFR 1p36 • TGFB2 1q41 • TGFA 2p13 • MSX1 4p16, 4q31, 6p23 • PVRL1 1 1q23 • TGFB3 14q24 • GABRB3 15q11 • RARA 17q21 • BCL3 19q13 ENVIRONMENTAL FACTORS ● Maternal alcohol consumption ● Maternal cigarette smoking ● Folic acid defi ciency ● Corticosteroid use ● Anticonvulsant therapy Nonsyndromic Orofacial Clefts
  • 14. Systemic Conditions associated with DTE • Nutrition • Vitamin D-resistant rickets • Endocrine disorders (Hypothyroidism ,Hypopitutarism, Hypoparathyroidism, pseudohypoparathyroidism) • HIV infection • Cerebral palsy • Celiac disease • Ichthyosis
  • 15. Malnutrition • Early malnutrition in childhood affects skeletal growth and results in decreased height. Similarly, poor nutrition affects tooth eruption and results in delayed emergence of the deciduous teeth.
  • 16. Vitamin D-resistant rickets • a group of metabolic disorders characterized b y renal tubular defects in phosphate transport and bone abnormalities resulting in hypophosphatemic ricket • teeth may not erupt until after one year. When decido re they erupt, they may be smaller than normal.
  • 17. Endocrine disorders • Disturbance of the endocrine glands usually has a profound effect on the entire body, including the dentition. Hypothyroidism, hypopituitarism, hypoparathyroidism, and pseudohypoparathyroidism are the most common endocrine disorders associated with DTE
  • 18. Hypothyroidism (CRETINISM , MYXEDEMA) • decreased levels of thyroid hormone • may be classifi ed as either primary or secondary • In primary hypothyroidism > the thyroid abnormal • in secondary hypothyroidism > the pituitary gland does not produce thyroid-stimulating hormone (TSH) > thyroid gland not release thyroid hormone.
  • 19. Hypothyroidism • lethargy, dry and coarse skin • swelling of the face and extremities • huskiness of the voice, constipation, weakness, and fatigue • Bradycardia • Hypothermia
  • 20. Hypothyroidism • Oral & Dental manifestations – Delayed eruption – Enamel hypoplasia in both dentitions (being less intense in the permanent dentition) – Anterior open bite – Macroglossia – Micrognathia – Thick lips – Dysgeusia – Mouth breathing
  • 21. Hypopituitarism (PITUITARY DWARFISM) • Affected patients are typically much shorter than normal • maxilla and mandible smaller than normal • teeth show a delayed pattern of eruption, delay ranges from 1 to 3 years for teeth that normally erup • lack of 3rd molars
  • 22. Hypoparathyroidism • Hypoparathyroidism is a metabolic disorder characterized by hypocalcemia and hypophosphatemia due to a deficiency or absence of parathyroid hormone secretion
  • 23. Hypoparathyroidism • Dental manifestations - Enamel hypoplasia in horizontal lines - Poorly calcified dentin - Widened pulp chambers - Dental pulp calcifications - Shortened roots - Hypodontia - Delay or cessation of dental development
  • 24. Hypoparathyroidism • Mandibular tori • Chronic candidiasis • Paresthesia of the tongue or lips • Alteration in facial muscles
  • 25. Pseudohypoparathyroidism • Pseudohypoparathyroidism (PHP) is a heterogeneous group of disorders characterized by hypocalcemia, hyperphosphatemia, increased serum concentration of parathyroid hormone (PTH) • Insensitivity to the biological activity of PTH • may be considered an inborn error of metabolism. • inability of the body to respond appropriately to parathyroid hormone (PTH)
  • 26. Pseudohypoparathyroidism • Dental manifestations – enamel hypoplasia – retarded root development with short blunted roots – larger root canals and distorted and thick dentin at the apex – Delayed eruption
  • 27. HIV infection • Hauk et al ,2001 found a correlation between progression of HIV infection to pediatric AIDS and delayed dental eruption and this delay is most closely linked to severity of symptom not CD4 depletion
  • 28. Cerebral palsy • Cerebral palsy is a disorder of movement and posture caused by nonprogressive abnormality of the immature brain that originates during the prenatal or perinatal period or first few years of life. > significant impairment of functional mobility. • 4 major subtypes are • Spastic (most common) • dyskinetic/athetoid • ataxic • mixed cerebral palsy
  • 29. Cerebral palsy • Clinical Manifestations – Intellectual disability (60% of patients) – Seizure disorder (30-50% of patients) – Delayed motor development – Limb spasticity – Persistent primitive reflexes – Involuntary movements, and ataxia
  • 30. Cerebral palsy • Oral Manifestations – risk for dental caries and periodontal disease – Enamel hypoplasia – Dental erosion – Delayed eruption of permanent teeth – Dilantin hyperplasia – Increased incidence of Class II Div I malocclusion – Increased risk for oral trauma and injury
  • 31. Celiac disease Celiac disease is a permanent intolerance to gluten that results in damage to the small intestinal mucosa caused by an autoimmune mechanism in those who are genetically susceptible to the disease. The villous atrophy that ensues can lead to malabsorption of a variety of macro- and micronutrients including iron, calcium, folate and fat-soluble vitamins.
  • 32. Celiac disease • Oral and dental Manifestations – Enamel defects – Delayed eruption – Recurrent aphthous ulcers – Cheilosis – Oral lichen planus – Atrophic glossitis
  • 33. Ichthyosis • Ichthyosis is a heterogeneous family of hereditary disorders mostly characterized by the accumulation of hyperkeratotic scales on the skin surface
  • 34. Ichthyosis • Oral and dental manifestation – Periodontal disease – Enamel hypoplasia – high caries incidence – delayed deciduous and permanent eruption – bruxism – bifid teeth – irregular morphology of teeth – hyperkeratotic plaques on the tongue.
  • 35. Genetic conditions • Amelogenesis imperfect • Cherubism • Cleidocranial dysplasia • Cornelia de Lange Syndrome • Down s yndrome • Hurler syndrome • Gorlin syndrome • Osteopetrosis (marble bone disease)
  • 36. Amelogenesis imperfect • Amelogenesis imperfecta (AI) is a genetically and clinically heterogeneous group of inherited dental enamel defects. • hypoplastic enamel • Hypomineralised • Eruption of permanent teeth is often delayed and sometimes completely impaired
  • 37. Cherubism • autosomal dominant disorder and with 100% penetrance in males and 50 to 75% penetrance in females, with 2:1 male predominance • Cystic, fibrous jaw lesions • Bilateral symmetric jaw expansion • Exposure of sclera below the iris • Aplasia of teeth
  • 38. Cleidocranial dysplasia • Caused by a defect in the CBFA1 gene • Large head with prominent frontal bones • Wide sutures and fontanelles • Small face • Aplasia/hypoplasia of clavicles • Supernumerary teeth • Delayed eruption tooth
  • 39. Cornelia de Lange Syndrome • General features: – microbrachycephaly – synophrys/long eyelashes – hand and limb anomalies – delayed bone age – abnormal speech pattern – frequently do not express facial expression – low hairline on forehead and neck R short, thick neck R pigmented nevi on ski
  • 40. • Oral manifestation : – micrognathia – down turned, thin lips – cleft palate in 20% – delayed tooth eruption and widely spaced teeth – microdontia – often severe dental behavior problem
  • 41. Down s yndrome • Incidence 1 in 600-700. • Most cases of trisomy 21 are caused by non disjunction, resulting in an extra chromosome • General feature : – brachycephaly/flat occiput – upward slanting palpebral fissures/epicanthal folds – brachydactyly/clinodactyly/simian crease • Note: Aatlantoaxial instability (10-20%) and • occipitoatlantal instability is seen in these • patients. These features are important • particularly in relation to cervical extension • during OR procedures or during full restraint • procedures.
  • 42. Down s yndrome • Oral manifestration – Enamel hypoplasia (primary dentition) – Periodontal disease (90%) and NUG (30%) – Narrow and short palate – Irregular and delayed dental eruption – missing – teeth (3rd M, 2nd M, Lat. Incisors) decreased – calculus formation • caries • rate is low hypodontia • taurodontism • Malocclusion (crossbites and openbites common) • Parotid salivary flow rate is decreased and increase Na+, Ca 2+, HCO -, and uric acid
  • 43. Down s yndrome • caries • rate is low hypodontia • taurodontism • Malocclusion (crossbites and openbites common) • Parotid salivary flow rate is decreased and • increase Na+, Ca 2+, HCO -, and uric acid
  • 44. Down s yndrome • cardiac • defect may be present requiring SBE • prophylaxis • lingual • papillae are large protruding, • large and fissured tongue • Lips are broad, irregular fissured, and dry Open mouth
  • 45. Hurler syndrome • Autosomal Recessiv (MPS I) • (Mucopolysaccharidosis I-H) • General features: – macrocephaly/frontal bossing – course facies/thick ear lobes and lips – hepatosplenomegaly – chest and spine deformities
  • 46. • Oral manifastration: – flattened philtrum – Lip patulous – widely spaced teeth – lip and tongue enlargement after 5 years old - incisors somewhat conical – anterior open bite due to macroglossia - ectopic molars in mandibular rami
  • 47. Oral manifastration : (con) – delayed eruption – TMJ may exhibit limited motion – dentigerous cysts – airway obstruction – sleep apnea reported – mandible short and broad – wide bigonial distance
  • 48. Gorlin syndrome • Gorlin syndrome is inherited as an autosomal dominant trait with a high degree ofpenetrance and variable expressivity. • The disorder has a population prevalence of 1 per 57,000 people • Male < female 3:1 • multiple odontogenic keratocysts • mandibular prognathism • and/or cleft lip and palate • Delayed tooth eruprion
  • 49. Osteopetrosis (marble bone disease) • Rare hereditary bone disorder characterized by increase in bone density due to defect in bone remodeling caused by failure of normal osteoclast function.
  • 50. • Oral manifestations: – Facial deformity leading to hypertelorism, – snub nose, frontal bossing etc. – Delayed tooth eruption and osteomyelitis of jaws. – Sclerosis of skull bones leads to narrowing of foramina which causes compression of various cranial nerves – blindness, deafness, facial paralysis etc. – The medullary spaces of the jaws are reduced. – Fracture of jaws during extraction procedure can occur without undue force, due to fragility of the bone

Editor's Notes

  • #8: Gingival hyperplasia resulting from various causes (hormonal or hereditary causes, drugs such as phenytoin)
  • #11: Lack of resorption of deciduous tooth
  • #29: dyskinetic/athetoid (slow, writhing involuntary muscle movement), ataxic (low muscle tone and poor coordination), and mixed cerebral palsy, with spastic forms being the most common
  • #31: Others: Tongue thrust, mouth breathing, hyperactive or hypoactive gag reflex, dysphagia, oral hypersensitivity (overreaction to touch, taste, or smell), prolonged and exaggerated bite reflexes, bruxism, sialorrhea, poor oral hygiene, and food pouching ifestations