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Case Reports
. 2024 Aug 10;16(8):e66612.
doi: 10.7759/cureus.66612. eCollection 2024 Aug.

Remimazolam Anesthesia for a Pediatric Patient With Glutaric Aciduria Type I: A Case Report

Tomoko Tsuruno  1 , Hiroki Tateiwa  1 , Yuki Hashimoto  1 , Yoshifumi Katsumata  1 , Takashi Kawano  1
Affiliations
Case Reports

Remimazolam Anesthesia for a Pediatric Patient With Glutaric Aciduria Type I: A Case Report

Tomoko Tsuruno et al. Cureus. .

Abstract

Glutaric aciduria type I (GA-1) is a rare metabolic disorder caused by an autosomal, recessive, inherited deficiency of glutaryl-CoA dehydrogenase. Reports on the anesthetic management of patients with GA-1 are limited. It has been suggested that inhalation anesthesia is safer than propofol due to the mitochondrial dysfunction inherent in GA-1. However, inhalation anesthesia poses a risk, albeit rare, of malignant hyperthermia, which can result in severe neurological damage in GA-1 patients. Therefore, we considered that management using remimazolam might be effective and, provided a successful general anesthesia using it for a pediatric patient with GA-1. We report a case of a four-year-old girl with GA-1 who underwent a laparoscopic gastrostomy under general anesthesia. Remimazolam was used for both induction and maintenance of anesthesia. Our perioperative management also included measures to prevent a hypercatabolic condition such as adequate hydration and blood glucose control. The patient had an uneventful perioperative course and was discharged on postoperative day 7. Thus, remimazolam is proposed as a new option for anesthetic management in patients with GA-1. Additionally, tailored perioperative management that addresses the unique characteristics of GA-1 is crucial for favorable outcomes.

Keywords: general anesthesia; glutaric aciduria type 1; mitochondrial disorder; perioperative management; remimazolam.

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Conflict of interest statement

Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. MRI features of the patient at four years old (before surgery)
Axial T2-weighted MR images (A-D), a diffusion-weighted image (E), and an apparent diffusion coefficient (ADC) map (F) are shown. Enlarged anterior temporal fossa subarachnoid spaces (white arrows in A) and dilation of the Sylvian fissures (outlined arrows in B) were observed. Diffuse high-intensity signals in T2-weighted images (C, D), corresponding diffusion restriction on the diffusion-weighted image (E), and a drop in the ADC map (F) were observed in the bilateral frontal and temporal white matter. These findings showed almost no change from the MR images taken at birth.
Figure 2
Figure 2. The anesthesia record
Cross marks: the beginning and end of anesthesia, T: intubation, E: extubation, Double circles: the beginning and end of surgery. sBP: systolic blood pressure, dBP: diastolic blood pressure, HR: heart rate, PSi: patient state index (their scales are on the left side), BT: body temperature (its scale is on the right side), BS: blood sugar
See this image and copyright information in PMC

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