Open AccessAnaesthetic management of a child with panthothenate kinase-associated neurodegeneration
Author(s) -
Renu Saxena,
Ghansham Biyani,
Sulagna Bhattacharjee
Publication year - 2015
Publication title -
indian journal of anaesthesia/indian journal of anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.645
H-Index - 30
eISSN - 0976-2817
pISSN - 0019-5049
DOI - 10.4103/0019-5049.149449
Subject(s) - medicine , botulism , aspiration pneumonia , neurodegeneration , pediatrics , mitochondrial disease , disease , anesthesia , pneumonia , pathology , genetics , gene , mitochondrial dna , biology
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.