2023 FSA Podium and Poster Abstracts
P064: DELAYED RECOVERY FROM OPIOIDS AND BENZODIAZEPINES IN SMITH-MAGENIS SYNDROME: A CASE REPORT.
Sae-In Kay, DO; Langely Chaparro, MD; Luis Rodriguez, MD; University of Miami / Jackson Health System
Introduction: Smith-Magenis syndrome (SMS) is a rare genetic disorder from a chromosomal microdeletion (17p11.2), associated with craniofacial, cardiac and renal abnormalities, neurocognitive deficits, peripheral neuropathy and pain insensitivity, which can pose a high risk for anesthetic complications. There is little literature about anesthetic considerations for patients with SMS, especially regarding perioperative use of opioids and anxiolytics. Here we report a case of a 16-years-old male with SMS and history of delayed recovery from fentanyl, now exhibiting delayed recovery from midazolam premedication.
Methods: A 16-year-old 81.7kg male with SMS, history of delayed recovery from fentanyl 15 years ago (respiratory distress in PACU, reversed with naloxone), obstructive sleep apnea, asthma, developmental delay, and exotropia, presented for a complete ophthalmological examination. Preoperative chest radiograph and vitals were unremarkable. He was premedicated with midazolam 20 mg PO. Uneventful inhalational induction with sevoflurane followed by insertion of supraglottic airway took place. Within an hour into the procedure, the patient desaturated to low 90s with persistent bradypnea to 6 breaths/min. FiO2 was increased to 100% and flumazenil 0.3 mg was given.
Results: Shortly after administration of flumazenil, SpO2 improved and respiratory depression resolved. Throughout this event, the patient remained hemodynamically stable and SpO2 remained above 90%. Nasopharyngeal airway was placed before the supraglottic airway was removed. The patient had no further complications during intraoperative and recovery period, and was discharged home the same day.
Discussion/Conclusion: SMS is a multiple congenital anomaly syndrome associated with the microdeletion of chromosome 17. The craniofacial abnormalities, cardiac and renal defects, along with the associated behavioral disorders are the principal causes of anesthetic complications in patients with SMS. The preoperative assessment should include a detailed history of developmental milestones and abnormal behavior patterns, detection of any cardiac and renal defects, and the severity and extent of peripheral neuropathy.
As demonstrated by this case, the intraoperative course may be complicated by hypersensitivity to narcotics and benzodiazepines. Although the exact cause of increased sensitivity to narcotics in patients with SMS is unknown, this delayed recovery from narcotics or anxiolytics may be explained by decreased sensitivity to pain in SMS. SMS patients have bilateral decrease of gray matter concentration in insulo-lenticular regions, which may contribute to behavioral phenotypes of SMS including pain insensitivity, self-injury, aggressive behavior and hyperactivity. Decreased pain sensitivity can manifest with broken limbs without complaint, onychotillomania, and self-injurious behavior. This decreased pain perception likely results in increased sensitivity to narcotics and delayed recovery from anesthetics. Thus, in SMS patients, it is advisable that a decreased amount of narcotics should be used with caution and close monitoring for any signs of prolonged respiratory depression is essential. Parental presence in the operating room during induction may reduce anxiety of the patients and minimize the perioperative anxiolytic use. Lastly, due to increased potential risk of anesthetic complications in SMS patients, it is prudent to provide care in tertiary centers where subspecialties in pediatric anesthesia are readily available.