Anaesthetic Challenges in An Adolescent with Segawa Syndrome and Recurrent Aspiration Pneumonitis Scheduled For Laparoscopic Fundoplication. A Rare Presentation

Background: Segawa syndrome, or dopamine-responsive dystonia, is a rare genetic disorder that often presents with dystonia and gait disturbances. Literature on the anaesthetic management of these patients is generally limited, with especially minimal information on patients not treated with levodopa, particularly when multisystem complications are involved.
Case: A 16-year-old adolescent male with Segawa syndrome, characterised by dystonia of face and neck muscles with global developmental delay, seizures, recurrent aspiration pneumonitis, and gastroesophageal reflux disease, underwent laparoscopic fundoplication. Anaesthesia was administered using propofol for induction, video laryngoscopy was used for intubation, and sevoflurane was used for maintenance. Despite respiratory challenges, including lower lobe infiltrates and a history of aspiration, careful intraoperative management ensured a stable course.
Conclusions: This case highlights the need for a tailored anaesthetic approach in Segawa syndrome with multisystem involvement. It underscores the importance of vigilant respiratory management, cautious drug selection, and a multidisciplinary approach, particularly in patients not receiving levodopa therapy.