Role of NIRS in a Child with Moyamoya Disease for Encephaloduroarteriomyosynangiosis (EDAMS): A Case Report

Background: Moyamoya, “puff of smoke” disease (MMD), is a rare cerebrovascular disease with progressive stenosis of intracranial blood vessels. Anesthetic goal of revascularization surgery is to maintain the cerebral oxygen supply and demand. We wanted to report a case of NIRS-based anesthetic management of encephaloduroarteriomyosynangiosis (EDAMS).

Case Description: An 8-year-old boy weighing 24 kg presented with two episodes of transient ischemic attacks (TIAs), diagnosed with MMD, planned for EDAMS. Overnight intravenous fluid (IVF) was given to prevent dehydration and diazepam premedication was given for anxiolysis. In the operating room, with ECG, EtCO2, SPO2, NIRS, and NIBP monitoring were attached; induction and intubation were performed using propofol, fentanyl, and atracurium. Postintubation, arterial BP, temperature, and urine output monitoring was established. Regional scalp block with 0.2% ropivacaine was given for analgesia. Anesthesia was maintained using air, oxygen, and sevoflurane. Depth of anesthesia, brain oxygenation, and hemoglobin trends were monitored using the patient state index, NIRS, and noninvasive hemoglobin analyzer using SedLine, Masimo. MAP, CO2, temperature, and depth of anesthesia were tightly controlled to keep the cerebral oxygenation close to the baseline. Repeat regional scalp block, fentanyl infusion was given for postoperative analgesia. He was discharged on the POD 4 without neurological deficit.

Conclusion: MMD has limited cerebrovascular reserve with propensity to develop cerebral ischemia during the perioperative period. In our case, the balance between the cerebral oxygen supply and demand was strictly maintained by monitoring the cerebral oxygenation using NIRS. Various factors, such as MAP, EtCO2, temperature, depth of anesthesia, and intraoperative hematocrit were controlled within normal limit to maintain the cerebral oxygenation close to baseline throughout surgery which aided for the successful outcome.

Publication History

Article published online:
25 March 2020