Pyramidal molars-abnormal upper lip syndrome

Alternate names

Juvenile glaucoma with unusual upper lip and dental roots; Ackerman fused molar roots syndrome

Definition

A rare syndrome characterized by pyramidal molar roots and taurodontism, associated with variable anomalies. It has been described in two generations of one family. Both parents and their six sibs had pyramidal, taurodont or fused molar roots.Some of the patients also had hypotrichosis, an abnormal upper lip, thickened and wide philtrum, and/or juvenile glaucoma. Other features included entropion of the eyelid, syndactyly and clinodactyly of the fifth fingers.

NIH genetic and rare disease info

• NIH info on Pyramidal molars-abnormal upper lip syndrome

Pyramidal molars-abnormal upper lip syndrome is a rare disease.

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