Oropalatal dysplasia: A case report with an innovative sequence of reconstruction

Abstract

A rare syndromic cleft palate child with microstomia and ankyloglossia is presented. The child presented at 1-month of age with microstomia, complete cleft of the secondary palate, ankyloglossia with dorsal groove of the tongue. There was no family history of oropalatal anomaly. This is a case of Bettex Graf Syndrome. The karyotype was normal 46XY. The reconstructive surgery was delayed to facilitate anesthesia and surgery. The reconstruction was carried out in one stage with an innovative sequence. Microstomia was released using Y-V plasty; palate repair was carried out with Bardach′s two flap palatoplasty and ankyloglossia was released with V-Y technique. The child has been followed-up for more than 6 months. Mouth opening is satisfactory, though there is recurrence of microstomia. Child is able to protrude the tongue well, however, it remains grooved.

Keywords

• ankyloglossia
• cleft palate
• dysplasia
• microstomia
• syndromic cleft palate
• tongue tie