title: Anaesthetic management of a child with panthothenate kinase-associated neurodegeneration.
abstract: Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy. The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation. The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency. We report successful anaesthetic management of a 9-year-old intellectually disabled male child with PKAN, scheduled for ophthalmic surgery under general anaesthesia.
authors: Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India.
authors: Renu
authors: R
authors: Sinha
authors: Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India.
authors: Ghansham
authors: G
authors: Biyani
authors: Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India.
authors: Sulagna
authors: S
authors: Bhattacharjee
keywords: Aspiration pneumonia; difficult airway; dystonia; muscle spasm; panthothenate kinase-associated neurodegeneration; respiratory failure