Anesthesia in an 8-year old child with familial dysautonomia (Reilly-day syndrome)

AUTHORS

Parisa Golfam 1 , * , Farzaneh Golfam 2 , Mitra Yari 1

1 Dept. of Anesthesia, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran

2 Dept. of Surgery, School of Medicine, Shahed University of Medical Sciences, Kermanshah, Iran

How to Cite: Golfam P , Golfam F, Yari M. Anesthesia in an 8-year old child with familial dysautonomia (Reilly-day syndrome) , J Kermanshah Univ Med Sci. 2012 ; 16(3):e78804.

ARTICLE INFORMATION

Journal of Kermanshah University of Medical Sciences: 16 (3); e78804
Published Online: September 29, 2012
Article Type: Case Report
Received: September 03, 2011
Accepted: February 21, 2012

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Abstract

Flowchart: Display: Case ReportBackground: Familial dysautonomia is a rare genetic disorder with autosomal recessive inheritance that has been mainly reported in the Ashkenazi Jewish population. Autonomic dysfunction, tendency to severe hyperpyrexia, vomiting and recurrent pulmonary aspiration, and pain conception disorder, make a patient an interesting candidate for anesthesia. 

Case Report: The patient was an 8-year old girl underwent surgery due to osteomyelitis and foot ulceration. Minimal drug, including sufentanil and nitrous oxide were used. Vital signs remained stable in the patient during the entire procedure.

Conclusion: Careful planning of the anesthetic management, preoperative hydration, understanding the physiological consequences, and being able to titrate the medications utilized are keys to decreasing complications encountered in these kinds of patients.

Keywords

familial dysautonomia anesthesia complications

© 2012, Journal of Kermanshah University of Medical Sciences. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

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