title: Posterior spinal instrumented fusion for idiopathic scoliosis in patients with multisystemic neurodegenerative disorder: a report of two cases.
abstract: Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) syndrome is a progressive multisystemic neurodegenerative disorder. MELAS syndrome impairs oxidative phosphorylation and predisposes patients to lactic acidosis, particularly under metabolic stress. We report 2 siblings with MELAS-associated idiopathic scoliosis who underwent posterior spinal instrumented fusion with measures taken to minimise anaesthetic and surgical stress, blood loss, and operating time.
authors: Department of Orthopaedic Surgery, University of Malaya, Kuala Lumpur, Malaysia.
authors: K W
authors: KW
authors: Loh
authors: Department of Orthopaedic Surgery, University of Malaya, Kuala Lumpur, Malaysia.
authors: C Yw
authors: CY
authors: Chan
authors: Department of Orthopaedic Surgery, University of Malaya, Kuala Lumpur, Malaysia.
authors: C K
authors: CK
authors: Chiu
authors: Department of Anaesthesiology, University of Malaya, Kuala Lumpur, Malaysia.
authors: M S
authors: MS
authors: Bin Hasan
authors: Department of Orthopaedic Surgery, University of Malaya, Kuala Lumpur, Malaysia.
authors: M K
authors: MK
authors: Kwan
mesh_terms: D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications*; D008297:Male; D019636:Neurodegenerative Diseases / Q000150:complications; D012600:Scoliosis / Q000209:etiology / Q000601:surgery*; D035781:Siblings; D013123:Spinal Fusion / Q000295:instrumentation / Q000379:methods*
keywords: MELAS syndrome; mitochondrial diseases; scoliosis