Intrathecal Baclofen Facilitated Postanesthetic Tracheal Extubation in a Dystonic Patient Associated with Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease)

Patients with Hallervorden-Spatz disease may be confronted by invasive procedure, like gastrostomy and thalamotomy for care of the status of extreme dystonia and rigidity. This rare disorder possesses potential perioperative risks, such as difficult airway management, aspiration pneumonia, hyperpyrexia, dehydration, acute renal failure, and postoperative pulmonary insufficiency. As patients were usually in the state of uncontrollable dystonia and rigidity, delayed endotracheal extubation had been suggested by several previous case reports. We report the anesthetic management of a 34-year-old Hallervorden-Spatz disease woman scheduled for intrathecal baclofen pump implantation under general anesthesia. Preoperatively she had suffered from severe dystonia, confusion and repeated attacks of respiratory insufficiency and pneumonia requiring mechanical ventilation and critical care. Anesthesia was induced uneventfully and the catheter of the pump reached the CSF via T2 interspace. Intraoperative intrathecal baclofen injection mildly relieved her severe dystonia and rigidity. Hemodynamics and arterial blood gas were improved than preoperative. She was extubated after consciousness was regained. Postanesthetic visit demonstrated a fair outcome. Whether this treatment applies to every case confronted by general anesthesia needs to be elucidated. Nevertheless, we suggest intrathecal baclofen may help postanesthetic pulmonary care by attenuating dystonia and rigidity and can be an adjuvant for anesthesia care in patients with Hallervorden-Spatz disease.