Document Detail

Anaesthetic management of a patient with Leigh's syndrome with central hypoventilation and obstructive sleep apnoea.
MedLine Citation:
PMID:  24356766     Owner:  NLM     Status:  In-Data-Review    
Leigh's syndrome, which is characterised by progressive neurodegeneration involving the brainstem and basal ganglia, belongs to a family of disorders classified as mitochondrial myopathies. It is most commonly transmitted by an autosomal recessive mode of inheritance, but can sometimes occur in a mitochondrial pattern. It typically presents during infancy with developmental delay and deterioration of brainstem function. Respiratory failure is the common cause of death and postoperative morbidity in patients with Leigh's disease. Herein, we report the case of a 17-year-old female patient with Leigh's syndrome who underwent general anaesthesia for a tracheostomy, which was performed in view of the patient's requirement for long-term ventilation and frequent toileting for secretions. Her respiratory complications included central hypoventilation secondary to brainstem involvement, and obstructive sleep apnoea due to obesity and muscle dystonia. She was hospitalised for acute respiratory decompensation secondary to hospital-acquired pneumonia. We review the anaesthetic implications of this disease and discuss its impact on preoperative, intraoperative and postoperative management.
A Tan; R Goy
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Singapore medical journal     Volume:  54     ISSN:  0037-5675     ISO Abbreviation:  Singapore Med J     Publication Date:  2013 Dec 
Date Detail:
Created Date:  2013-12-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0404516     Medline TA:  Singapore Med J     Country:  Singapore    
Other Details:
Languages:  eng     Pagination:  e250-3     Citation Subset:  IM    
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