Perioperative management of surgical orthodontic treatment in a patient with glucose transporter 1 deficiency: report of a case and review of the literature

• Published in: Perioperative medicine (London) Vol. 11; no. 1; p. 55
• Main Authors: Kyoko, Nishiyama, Masakazu, Hamada, Shin, Nabatame, Hidetaka, Shimizu, Narikazu, Uzawa
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 16.12.2022; BioMed Central; BMC
• Subjects: Ataxia; Care and treatment; Case Study; Convulsions & seizures; Dextrose; Diarrhea; Epilepsy; General anesthesia; Glucose; Glucose transporter type 1 (GLUT1) deficiency; Health aspects; Infections; Intellectual disabilities; Jaw deformity; Ketogenic diet; Metabolism; Nervous system; Orthodontics; Orthognathic surgery; Paralysis; Perioperative management; Seizures (Medicine); Surgery; Vomiting; Orthognathic surgery; Perioperative management; Glucose transporter type 1 (GLUT1) deficiency; Jaw deformity; Ketogenic diet
• ISSN: 2047-0525; 2047-0525
• DOI: 10.1186/s13741-022-00287-8

Glucose transporter 1 (GLUT1) deficiency is a rare cerebral metabolic disorder caused by the shortage of glucose supply to the brain. For this disease, ketogenic diet therapy is essential. In addition, perioperative management requires not only the continuation of ketogenic diet therapy but also the management of nausea/vomiting, diarrhea, seizures, and infection. However, there have been few reports regarding oral and maxillofacial surgery. We describe a patient with GLUT1 deficiency who underwent orthognathic surgery. An 18-year-old man was referred to our hospital with the chief complaint of mandibular regression. Surgical tolerance was assessed by a fasting test and tooth extraction under general anesthesia, and orthognathic surgery was then performed. For orthognathic surgery, the mandibular dentition had scissor-like occlusion, and it was difficult to arrange the mandible. Therefore, we decided to perform maxillary osteotomy first. After the mandibular dentition was arranged by maxillary osteotomy, sagittal split ramus osteotomy (SSRO) was performed. Intermaxillary fixation (IMF) was necessary for SSRO, and caution was needed to prevent suffocation. The orthognathic surgery was successful, although complications, such as vomiting, diarrhea, and seizures, developed. Surgical orthodontic treatment in GLUT1 deficiency can be performed relatively safely by maintaining the diet, taking measures against epilepsy and vomiting, and using antimicrobial agents in close collaboration with pediatricians, anesthesiologists, pharmacists, and nutritionists.