Management of general anesthesia in a child with Miller–Dieker syndrome: a case report

Abstract

Miller–Dieker syndrome (MDS) is a rare disorder characterized by type I lissencephaly and a distinctive facial appearance that may include prominent forehead, bitemporal hollowing, and micrognathia. MDS is associated with epilepsy. We here report an 18-month-old girl with MDS who required general anesthesia. The child had an extremely low Bispectral Index (BIS) value prior to undergoing general anesthesia. Her perioperative course was uneventful. This case highlights some of the important anesthetic concerns in patients with MDS, which include potentially difficult airways and extremely low BIS values.

Anesthetic Management and Bispectral Index in a Child with Miller–Dieker Syndrome: A Case Report

“…An anesthesiologists must pay close attention to the above-mentioned issues. Wakiguchi et al reported a case of airway management in an 18-month-old girl with MDS underwent laryngotracheal separation surgery [ 2 ]. In that case, a direct laryngoscope with a Macintosh blade was used.…”

“…Additionally, Wachiguchi et al reported a case of MDS in an 18-month-old child. The child had BIS values of 16–21 before anesthetic induction, 15–34 during sevoflurane maintenance, and 26–33 after anesthetic administration [ 2 ]. In our case, the BIS values were 37, 17–26, and 51 before, during, and after the anesthesia, respectively.…”

“…The anesthetic management of patients with MDS should focus on airway manipulation with the risk of potentially difficult intubation, seizure control due to lissencephaly, and any other clinical complications. Previously, Wakiguchi et al described the comprehensive anesthetic management in an 18-month-old girl with MDS [ 2 ]. Herein, however, we report a case of general anesthesia in a child with MDS, which specifically focuses on careful airway management using a videolaryngoscope, seizure management with regard to anesthetics use, and the validity of BIS monitoring.…”