Developmental disorders Assoc Professor Jan Laco MD Ph

Developmental disorders Assoc. Professor Jan Laco, MD, Ph. D

Developmental disorders • 1. Teeth • 2. Jaws • 3. Clefts • 4. Oral soft tissue

1. Developmental disorders - teeth • 1 a. Abnormalities of size and form • 1 b. Abnormalities of number • 1 c. Defects of structure • 1 d. Disorders of eruption

1 a. Abnormalities of size and form • microdontia …teeth < normal – permanent teeth – absolute x relative – generalized x single (upper I 2, M 3) • macrodontia …teeth > normal – absolute x relative – generalized x single

1 a. Abnormalities of size and form • gemination (schizodontia, twinning) – incomplete division of tooth germ – 2 separated crowns on single root, upper I 1 • fusion (synodontia) • concrescence – fusion after development of roots (injury) – teeth united by cementum

1 a. Abnormalities of size and form • dilaceration – angulation at crown-root junction (injury) – difficult extraction • talon cusp – lingual or palatal aspect of permanent teeth • dens invaginatus (dens in dente) – invagination of enamel into pulp chamber – upper I 2, bilateral, caries

1 a. Abnormalities of size and form • taurodontism (bull tooth) – wide pulp chamber – molars • enamel pearl – displaced ameloblasts below amelocemental junction – mm nodule, upper M

1. Developmental disorders - teeth • 1 a. Abnormalities of size and form • 1 b. Abnormalities of number • 1 c. Defects of structure • 1 d. Disorders of eruption

1 b. Abnormalities of number • Isolated hypodontia – common – hereditary – M 3 + P 2 + I 2 – M 3 + P 2: NO effect – I 2: false eruption of C to I 1 • Anodontia – very rare

1 b. Abnormalities of number • Anhidrotic ectodermal dysplasia – X-linked inheritance – hypodontia + hypotrichosis + anhidrosis – failure of development of alveolar process • Down´ s syndrome – no M 3 (90% patients)

1 b. Abnormalities of number • Hyperdontia (additional teeth) – common – supernumerary: conical • incisor + molar region + midline (mesiodens) – supplemental: normal • excessive growth of l. dentalis • M 4 • Cleidocranial dysplasia • Gardner´s syndrome

1. Developmental disorders - teeth • 1 a. Abnormalities of size and form • 1 b. Abnormalities of number • 1 c. Defects of structure • 1 d. Disorders of eruption

1 c. Defects of structure • deciduous teeth – neonatal jaudice yellow – congenital porphyria red – Tetracycline • permanent teeth – periapical infection of d. predeccesor – Turner tooth – systemic diseases

1 c. Defects of structure – systemic diseases • genetic – amelogenesis imperfecta – dentinogenesis imperfecta – Ehlers-Danlos syndrome, Gardner´s syndrome • • infective – congenital syphilis metabolic – childhood infections, rickets drugs – tetracycline, cytotoxic chemotherapy fluorosis

1 c. Defects of structure – systemic diseases • Amelogenesis imperfecta – all teeth + whole enamel x dentine normal – Hereditary enamel hypoplasia • AD inheritance • defect of matrix formation – pits, grooves, hard – Hereditary enamel hypocalcification • AD + AR inheritance • normal matrix poorly calcified – soft, chalky

1 c. Defects of structure – systemic diseases • Chronological hypoplasia – transient impairment of enamel formation in case of severe illness – severe fever, gastroenteritis, measles in the past – horizontál bands of pits, grooves, defects • Molar-incisor hypomineralisation – all M 1 a I 2 – enamel - hard surface, but soft beneath – yellow brown discoloration, ↑ caries

1 c. Defects of structure – systemic diseases • Dentinogenesis imperfecta – – – defect of collagen/sialoprotein, AD normal teeth shape brownish, translucent enamel chip away obliteration of pulp dentine - few tubules + abnormal matrix + incomplete calcification – “shell teeth“ – thin mantle of dentine covers abnormal dentine, few odontoblasts, no roots Dentinal dysplasia – very short and conical roots, teeth are lost early Regional odontodysplasia (ghost teeth (radiolucent on RTG)) – hypoplastic / hypomineralised enamel + thin dentine

1 c. Defects of structure – systemic diseases • Ehlers - Danlos syndrome (floppy joints) – collagen disorder – hypermobile joints + loose skin – small teeth + short roots + pulp stones • Gardner´s syndrome – multiple osteomas (jaws) + skin tumors + colonic polyps malignant change – impacted teeth, supernumerary teeth

1 c. Defects of structure – systemic diseases • Congenital syphilis – Hutchinson (1858) – later fetal infection – dental follicle infection by T. pallidum – chronic inflammation fibrosis of tooth sac compression of developing tooth distortion of ameloblasts – permanent teeth • upper I 1 (Hutchinson´s incisors) – barrel-shaped, fissure on incisal edge • M 1 (Moon´s molars) – dome-shaped, pitted occlusal surface (“mulbery molar“)

1 c. Defects of structure – systemic diseases • Tetracycline (TTC) – taken up calcifying tissues – yellow fluorescence (UV light) – during teeth development – fetus x infancy – dentine – bright yellow brown grey – permanent teeth – Avoid TTC under 12 years

1 c. Defects of structure – systemic diseases • Fluorosis (mottled enamel) fluoride in drinking water, India during dental development paper-white patches brown permanent teeth hydroxyapatite calcium fluoroapatite patches of incomplete calcification with high organic and water content – enamel matrix usualy normal – – –

1. Developmental disorders - teeth • 1 a. Abnormalities of size and form • 1 b. Abnormalities of number • 1 c. Defects of structure • 1 d. Disorders of eruption

1 d. Disorders of eruption • deciduous teeth: 6 th – 30 th month – at birth: natal teeth – 1 st month: neonatal teeth (lower I 1) • permanent teeth: 6 th – 13 th y (M 3: 18 th – 30 th y) – loss of space, retention of a dec. predecessor – cretenism, rickets

Developmental disorders • 1. Teeth • 2. Jaws • 3. Clefts • 4. Oral soft tissue

2. Developmental disorders - jaws • • • micrognathia x macrognathia hereditary prognathism craniofacial anomalies cherubism Gardner´s syndrome osteogenesis imperfecta

Developmental disorders • 1. Teeth • 2. Jaws • 3. Clefts • 4. Oral soft tissue

3. Developmental disorders - clefts • • NOT clefts non-fusion !!! ? ethiology - genetic component (40%) cleft lip - males, inc. 1/1000 cleft palate - females, inc. 1/2000 lip : lip + palate : palate … 22% : 58% : 20% + Down´s syndrome (1 in 200 pts. ) van der Woude syndrome (AD, IRF 6 gene) – clefts + pits on lower lips + hypodontia

3. Developmental disorders - clefts • cleft lip – cheiloschisis – lateral: unilateral (left side) x bilateral – medial • • alveolar ridge cleft – gnathoschisis hard palate cleft – palatoschisis soft palate cleft - uranoschisis uvula cleft - staphyloschisis

3. Developmental disorders - clefts • complications – malformation or absence of teeth in defect – + other congenital defects – feeding disorder – breathing disorder – middle ear infections – speech defect – cosmetic defect

Developmental disorders • 1. Teeth • 2. Jaws • 3. Clefts • 4. Oral soft tissue

4. Developmental disorders – oral soft tissues • 4 a. oral mucosa • 4 b. gingiva and periodontium • 4 c. tongue

4 a. Developmental disorders – oral mucosa • white sponge nevus – AD inheritance – white, soft, thickened area of oral mucosa – Mi: acantosis + hyperkeratosis + intracellular edema • Fordyce´s granules – heterotopic sebaceous glands – 80% adults – soft yellowish spots or papules – symmetrical, buccal location

4 b. Developmental disorders – gingiva and periodontium • hereditary gingival fibromatosis – AD inheritance – grossly enlarged gingivae - preceding of teeth eruption – Mi: collagen bundles – gingivectomy after puberty • Ehlers-Danlos syndrome

4 c. Developmental disorders – tongue • macroglossia – – muscle hypertrophy congenital hemangioma or lymphangioma Down´s syndrome lingual thyroid • incidence 10% • nodule, foramen caecum • cleft tongue • ankyloglossia

4 c. Tongue Lingua plicata • • cerebelli-, cerebriformis, fissurata, gyrata, scrotalis fissured dorsal aspect of tongue children x adults asymptomatic x pain during eating, hygiene + geographical tongue + Cowden syndrome + Melkersson-Rosenthal syndrome

4 c. Jazyk Lingua geographica • • geographical tongue, glossitis migrans + lingua plicata; ± psoriasis asymptomatic x pain during eating irregular smooth red area healing (hours / days) another red area appears • centre – epithelium thinning; periphery – hyperplasia + keratinization; mild inflammation