A rare case of congenital midface underdevelopment (dysgenesis cerebrofacialis mediana)

A case of defective development of the midface with missing median segment of the upper lip and premaxilla is described. Such a deformity is interesting because it represents a rarely observed stage of development of the midface, i.e. the intermediate stage between fully developed arhinencephalia and a bilateral penetrating cleft of the upper lip. The case described may serve as evidence for the assumption of Brucker that arhinencephaly is a type of bilateral cleft of the upper lip rather than a median cleft. In these deformities underdevelopment of certain parts of the central nervous system should be expected. It therefore follows that it is necessary to pay attention to the psychic and neurologic condition of the child affected.