A Case Report of a Bloch-sulzberger Syndrome (Incontinentia Pigmenti) Patient on Whom We Performed Orthognathic Surgery under General Anesthesia

Abstract

Bloch-Sulzberger syndrome (Incontinentia pigmenti) is a genetic disease that displays multiple forms of symptoms including those of the skin, teeth and eyes. Here, we report the case of a patient with Bloch-Sulzberger syndrome on whom we performed orthognathic surgery under general anesthesia. The patient was a 21-year-old female. Oral cavity findings were congenital defects in the right upper jaw 7542, left upper jaw 12578, right lower jaw 754, and left lower jaw 125. Late-stage residual E in the left upper jaw, and abnormal crown morphology of tooth 23 in the right lower jaw and tooth 3 in the left upper jaw were observed. Right cephalometric radiographic analysis revealed marked skeletal mandibular protrusion at SNA: 77.6°, SNB: 89.1°, and ANB: −11.5°. Mandibular protrusion with anomaly in the number of teeth was diagnosed; sagittal split ramus osteotomy and Le Fort 1 osteotomy were performed under general anesthesia. At 18 months postsurgery, the patient is wearing the final prosthesis and occlusion has been confirmed. The present status of occlusion is good and the patient is also satisfied.