Acrorenal Mandibular Syndrome: A Case Report

Acral (limb) and renal anomalies occur together as a single developmental field defect (DFC)1 or as components of several malformation syndromes. The embryogenesis of that defect was studied experimentally by Lash2 and the developmental relationship between kidneys and limbs has been reviewed by Buchta3 and Gilbert4. In 1980, Halal et al. first described the association of bilateral split foot malformation, severe mandibular hypoplasia and bilateral renal malformation in female sibs born to consanguineous French- Canadian parents. Bilateral renal agenesis was found in one sib and bilateral polycystic kidneys in the other5.These authors proposed the name acro-renal-mandibular syndrome (ARMS) for the condition and considered it most likely that the mechanism of inheritance was autosomal recessive.