Anaesthetic management of a patient with Alport syndrome and tracheobronchial leiomyomatosis

Anaesthetic management of a patient with Alport syndrome and tracheobronchial leiomyomatosis

Published: 13 Jun 2018

© Anaesthesia Cases / 2018-0102 / ISSN 2396-8397

Authors

Dr Kasie Hiu Ying Ng [1]
Dr Jaclyn WM Wong [2]
  • [1] Medical Officer, Department of Anaesthesiology and Operating Theatre Services, Kwong Wah Hospita, Hong Kong, China
  • [2] Medical Officer, Department of Anaesthesiology and Operating Theatre Services, Kwong Wah Hospital, Hong Kong, China

Summary

Alport syndrome is a rare inherited disorder which can manifest with haemorrhagic nephritis, ocular lesions, sensorineural deafness, as well as leiomyomatosis of the respiratory and gastrointestinal tract. We describe a patient with Alport syndrome who underwent emergency surgery complicated by intra-operative respiratory compromise caused by suspected tracheobronchial leiomyomatosis. We suggest that pre-operative computer tomography or bronchoscopy should be considered in such patients in order to evaluate the extent of disease involvement and to aid the anaesthetic management plan.

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