A case report of anesthetic management of a patient with Hallervorden–Spatz disease posted for incision and drainage of rectus sheath hematoma

Abstract

Pantothenate kinase-associated neurodegeneration (PKAN), also known as Hallervorden–Spatz disease, is a neurodegenerative disorder presenting with dystonia, cognitive impairment, rigidity, and mental deterioration. It has an autosomal recessive mode of inheritance, which usually presents in late childhood or early adolescence. Rigidity poses a challenge to an anesthesiologist in airway management. In this case report, we present the case of a 23-year-old male patient, a diagnosed case of Hallervorden–Spatz disease scheduled for incision and drainage of rectus sheath hematoma which occurred due to blunt trauma to the abdomen. Patients with PKAN who require general anesthesia may present with symptoms that influence perioperative anesthetic management as well as postoperative care.