Abstract
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.
Highlights
Hallervorden-Spatz disease (HSD) is a rare disorder characterized by movement abnormalities and dementia. [1] Its etiology is related to neurodegeneration with iron accumulation in the brain [1]
We report the anesthetic management of a 34-year-old Hallervorden-Spatz disease woman scheduled for intrathecal baclofen pump implantation under general anesthesia
She had suffered from severe dystonia, confusion and repeated attacks of respiratory insufficiency and pneumonia requiring mechanical ventilation and critical care
Summary
Hallervorden-Spatz disease (HSD) is a rare disorder characterized by movement abnormalities and dementia. [1] Its etiology is related to neurodegeneration with iron accumulation in the brain [1]. Rapid progression of the disease may lead to severe disability, life-threatening state, the status dystonicus, and several critical events such as hyperpyrexia, dehydration, respiratory insufficiency and acute renal failure secondary rhabomyolysis [2,3]. Patients requireing general anesthesia for these invasive procedures were usually in a status of uncontrolled dystonia and rigidity that threatened their lives. We report a case of HSD with late onset and rapid progression. She suffered from extreme dystonia and rigidity and repeated attacks of respiratory insufficiency and pneumonia. Intraoperative administration of baclofen helped relieve the dystonia and rigidity and tracheal extubation can be achieved earlier than usual. Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease) vorable pulmonary outcome
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