Ｆｒｅｅｍａｎ−Ｓｈｅｌｄｏｎ症候群患児の小口症に対する手術経験の１例

Abstract

This case report describes the surgical plastic reconstruction of the lips and an enlarged aperture of the mouth in an 8-year-old Japanese girl who was given a diagnosis of Freeman-Sheldon syndrome.In this patient, it was difficult to decide where to make the incision in the myopathic orbicularis oris muscle and there by positively affect the future morphology and function of the lips. The operation was performed to obtain sufficient intercommissural distance of the lips, and care was taken to prevent ptosis of both commissures of the lips.At present, 6 years after the operation, the outcome is generally satisfactory with respect to the function of mouth opening. However, the patient still lacks a complete or sufficient lip shield, and the upper incisors have gradually become exposed.Lateral cephalogram analysis conducted 6 years postoperatively indicated that the upper incisiors had inclined labially and the lips had moved forward. These changes due to plastic correction apparently led to reduced lip pressure against the upper incisors.