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Table 3 Comparison of conditions associated with the simultaneous presence of microdontia and supernumery teeth along with taurodontism, microdontia, and dens invaginatus as well as distal symphalangism, hypoplastic carpal bones, microdontia, dental pulp stones, narrowed zygomatic arch.

From: Non-syndromic occurrence of true generalized microdontia with mandibular mesiodens - a rare case

Taurodontism, microdontia, and dens invaginatus Cleidocranial dysplasia Craniometadiaphyseal dysplasia Dermoodontodysplasia Hypodontia and nail dysgenesis Orofaciodigital syndrome type 3 Tricho-rhino-phalangeal syndrome type 1 Distal symphalangism, hypoplastic carpal bones, microdontia, dental pulp stones, narrowed zygomatic arch
Generalized microdontia Autosomal dominant Autosomal recessive Autosomal dominant Autosomal dominant Autosomal recessive Autosomal dominant Autosomal dominant
Taurodontism of first permanent molars Chromosome 6 Macrocephaly Dry skin Chromosome 4 Thin/hyperconvex/hypoplastic nails Autosomal recessive Absent/small nails
Multiple teeth with one or more dens invaginatus Arm p Frontal bossing Ichthyosis Arm p Prominent occiput Chromosome 8 Microdontia
X-linked recessive inheritance Normal height (with skeletal dysplasia) Large fontanelle Thin skin Dry skin Frontal bossing Arm q Other dental abnormality
  Short stature - postnatal Prominent eyes Pigmented naevi Fine hair Round face Normal height (with skeletal dysplasia) Abnormal clinical features of the limbs
  Absent/small nails Mandibular hyperostosis/sclerosis Abnormal hair texture Brittle hair/trichorrhexis nodosa/pili torti Hypertelorism Short stature - postnatal Brachydactyly
  Macrocephaly Optic nerve abnormality/atrophy Sparse/absent scalp hair - localised Sparse/absent scalp hair - generalised Down-slanting palpebral fissures Decreased body hair/hypotrichosis Irregularities of length/shape of fingers
  Flat occiput (brachycephaly) Microdontia Abnormal nails Absent/small nails Other orbital abnormality Decreased hair pigmentation - general Syndactyly of fingers
  Frontal bossing Abnormal tooth position/malocclusion/open bite Midface hypoplasia/flat/short midface Thin/hyperconvex/hypoplastic nails Paresis of ocular muscles/squint Decreased hair pigmentation - patchy Short foot (including brachydactyly)
  Wide sutures/delayed fusion of sutures Missing permanent teeth/retained deciduous teeth Micrognathia/agnathia/retrognathia Dysplastic/grooved/thick/discoloured nails Other eye movement disorder Fine hair Syndactyly (other than minimal 2nd and 3rd toes)
  Large fontanelle Anodontia/oligodontia Microdontia Depressed premaxillary region Broad/bulbous nasal tip Brittle hair/trichorrhexis nodosa/pili torti Irregular length or shape of toes
  Facies significantly abnormal Natal/neonatal teeth Anodontia/oligodontia Midface hypoplasia/flat/short midface Cleft soft palate/bifid uvula/submucous cleft Sparse/absent scalp hair - generalised Other skull abnormality
  Small face Supernumerary teeth Supernumerary teeth Micrognathia/agnathia/retrognathia Microdontia High hairline - front Absent/small/hypoplastic carpals
  Hypertelorism Dental caries Other dental abnormality Absent/decreased eyebrows/lateral thinning Abnormal tooth position/malocclusion/open bite Thin/hyperconvex/hypoplastic nails Symphalangism
  Prominent supraorbital ridges Low set ears Simian creases Absent/decreased lashes Supernumerary teeth Dysplastic/grooved/thick/discoloured nails Cone shaped epiphyses
  Depressed premaxillary region Scoliosis Dislocated hip Everted/protruding lips Cleft/notched tongue Broad/bifid nails Symphalangism
  Midface hypoplasia/flat/short midface Bowed limbs   Tooth crown shape abnormality Hamartoma/other tumours of the mouth Frontal bossing Cone-shaped epiphyses of middle phalanges
  Prognathism Mental retardation of any degree   Microdontia Other abnormality of tongue/gingivae/mucosa High forehead  
  Depressed nasal bridge Boney sclerosis of any type   Abnormal tooth position/malocclusion/open bite Low set ears Facies significantly abnormal  
  Paramedian/lateral cleft lip (uni/bilateral) Multiple fractures/increased boney fragility   Delayed eruption of teeth Tragus abnormal Long face  
  Cleft soft palate/bifid uvula/submucous cleft Enchondroma/radiolucencies - localized   Anodontia/oligodontia Pectus excavatum (funnel chest) Grooved/dimpled chin  
  High vaulted and narrow palate Lytic/lucent lesions of bone   Supernumerary teeth Abnormally placed nipples Micrognathia/agnathia/retrognathia  
  Microdontia Fibrous dysplasia of bone    Thoracolumbar general kyphosis Medial flare of eyebrows  
  Developmental defect of enamel Wide diaphyses (undertubulation)    Irregularities of length/shape of fingers Absent/decreased eyebrows/lateral thinning  
  Tooth discolouration Submetaphyseal undermodelling/expansion    Syndactyly of fingers Absent/decreased lashes  
  Delayed eruption of teeth Thin cortex of diaphyses    Polydactyly - postaxial (ulnar)/type unspecified Long/large nose  
  Missing permanent teeth/retained deciduous teeth Bowing of long bones    Abnormal palmar dermatoglyphics/skin creases Broad nasal bridge (see telecanthus)  
  Supernumerary teeth Cartilage tongues of metaphyses - localized    Polydactyly of feet - postaxial/type unspecified High nasal bridge  
  Dental cysts/tumours Hyperostosis/thickened/sclerotic calvarium    Syndactyly (other than minimal 2nd and 3rd toes) Broad/bulbous nasal tip  
  Deafness - conductive Absent/abnormal sinuses    Cranial nerve/nuclei Hypoplastic/small nostrils  
  Other hearing abnormality Wormian bones    Mental retardation - moderate/severe Abnormal columella  
  Narrow/sloping shoulder/hypermobile shoulders Sclerotic/hyperostotic facial bones    Hypotonia Thin lips  
  Pectus excavatum (funnel chest) Other skull abnormality    Movement disorder - dystonia/chorea/tremor/spasm Long philtrum  
  Bell-shaped chest Hyperostotic/wide clavicle    EEG abnormality Deeply grooved philtrum  
  Thoracolumbar general kyphosis Abnormal rib structure including fusion    Short sternum Microdontia  
  Gibbus/localised kyphosis Widened ribs     Abnormal tooth position/malocclusion/open bite  
  Scoliosis Irregular shape of pubic and ischial bones     Supernumerary teeth  
  Hyperextensible/hypermobile joints Absent/hypoplastic/short femur     Anteverted/prominent/bat ears  
  Small hand Femora short/deformed/bowed     Long/large ear  
  Brachydactyly Other abnormal femur     Pectus carinatum (pigeon chest)  
  Seizures of any type Bow legs - genu varum     Thoracolumbar general kyphosis  
  Hypotonia      Scoliosis  
  Imperforate anus/anal stenosis      Hyperextensible/hypermobile joints  
  Horseshoe/fused/ectopic kidneys      Small hand  
  Hypospadias/epispadias      Brachydactyly  
  Undescended/ectopic testes      Clinodactyly of 5th finger  
  Wilms tumour      Terminal hypoplasia fingers  
  Delayed skeletal maturation      Spindle shaped/tapered fingers  
  Poorly ossified calvarium/Soft skull      Ulnar deviation of fingers  
  Absent/abnormal sinuses      Other hand abnormality  
  Wormian bones      Mental retardation of any degree  
  Platybasia/basilar impression      Abnormal cardiovascular structure/function  
  Enlarged foramen magnum      Winged/other abnormal scapula (See Shoulder)  
  Small/absent scapula      Coxa vara  
  Winged/other abnormal scapula (See Shoulder)      Cone shaped epiphyses  
  Absent/hypoplastic clavicles      Small femoral head epiphyses  
  Pseudarthrosis of clavicle      Flat femoral head epiphyses  
  Short ribs (circumferential)      Deformed/irregular femoral head epiphyses  
  Under-/unossified sternum      Broad femoral neck  
  Hypoplastic/absent ribs      Cone-shaped epiphyses of proximal phalanges  
  Dorsal wedging of vertebral bodies      Some phalanges short and deformed  
  Narrow/trapezoid iliac wings (lack of flare)      Cone-shaped epiphyses of middle phalanges  
  Horizontal/flat acetabular roof      Cone-shaped epiphyses of distal phalanges  
  Delayed ossification of pubic and ischial bones       
  Open pubic symphysis in adults       
  Coxa valga       
  Coxa vara       
  Dislocated hip       
  Cone shaped epiphyses       
  Fibulae a-/hypoplastic/under-/unossified       
  Cone-shaped epiphyses of proximal phalanges       
  Cone-shaped epiphyses of middle phalanges       
  All middle phalanges short/deformed       
  Cone-shaped epiphyses of distal phalanges       
  All distal phalanges short/deformed