/Ackerman syndrome

Ackerman syndrome

Rare Disease

MONDO:0008706

Also Known As

Ackerman fused molar rooth syndromeAckerman syndromepyramidal molar-glaucoma-upper abnormal lip syndromeglaucoma, juvenile, with unusual upper lip and dental rootsjuvenile glaucoma with unusual upper lip and dental rootsmolar roots, pyramidal, with juvenile glaucoma and unusual upper lippyramidal molars, glaucoma, abnormal upper lip

Definition

Ackerman syndrome is 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.