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Pyramidal molars-abnormal upper lip syndrome

Disease definition

A rare multiple congenital anomalies/dysmorphic syndrome characterized by a variable combination of dental, cutaneous, ocular, and bone abnormalities, including pyramidal and fused molar roots, taurodontism, an abnormal upper lip without a cupid's bow and thickened and wide philtrum, juvenile glaucoma, syndactyly, and clinodactyly. There have been no further descriptions in the literature since 1973.


Classification level: Disorder
  • Synonym(s):
    • Ackerman fused molar roots syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: K00.2
  • OMIM: 200970
  • UMLS: C1860167
  • MeSH: C538170
  • GARD: 469
  • MedDRA: -
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