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Anesthetic Management and Bispectral Index in a Child with Miller-Dieker Syndrome: A Case Report
Sang Jin Park1, Jongyoon Baek1, Suyoun Chun1
1Department of Anesthesiology and Pain Medicine, Yeungnam University College of Medicine, Daegu 42415, Republic of Korea.
Abstract:
Miller-Dieker syndrome (MDS) is a genetic disorder characterized by classic lissencephaly, distinctive facial features, intellectual disability, seizures, and early death. 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. Herein, we report a case of anesthetic management in a child with MDS and describe relevant clinical features in a perioperative anesthetic setting. This case highlights the importance of difficult airway manipulation using a videolaryngoscope, seizure management with regard to anesthetics use, and the low validity of BIS monitoring in patients with MDS.
Insights
Anesthetic management for Miller-Dieker syndrome (MDS) requires careful airway handling and seizure control. Videolaryngoscopy is vital for intubation, and BIS monitoring may be unreliable in these patients.
Area of Science:
- Medical Genetics
- Neurology
- Anesthesiology
Background:
- Miller-Dieker syndrome (MDS) is a rare genetic disorder.
- MDS presents with lissencephaly, facial dysmorphia, intellectual disability, and seizures.
- Patients with MDS face significant anesthetic risks, including difficult intubation and potential seizures.
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