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Dexmedetomidine as an anaesthetic adjunct for total intravenous anaesthesia in patients with xeroderma pigmentosum.

Authors:

Bashu Dev Parajuli ,

Tribhuvan University Teaching Hospital, NP
About Bashu
Lecturer in Anaesthesiology
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Gentle Sunder Shrestha,

Tribhuvan University Teaching Hospital, NP
About Gentle
Lecturer in Anaesthesiology
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Bigen Man Shakya,

Tribhuvan University Teaching Hospital, NP
About Bigen
Lecturer in Anaesthesiology
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Anubhav Sharma,

Tribhuvan University Teaching Hospital, NP
About Anubhav
Resident, Anaesthesiology
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Pragya Acharya,

Tribhuvan University Teaching Hospital, NP
About Pragya
Resident, Anaesthesiology
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Santosh Acharya,

Tribhuvan University Teaching Hospital, NP
About Santosh
Resident, Anaesthesiology
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Sirish Maskey

Tribhuvan University Teaching Hospital, NP
About Sirish
Resident, Anaesthesiology
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Abstract

Xeroderma Pigmentosum (XP) is a rare autosomal re­cessive disease, caused by a mo­lecular defect in nucleotide excision repair (NER) genes. These patients present with many perioperative anaesthetic challenges like difficult venous cannulation, difficult intubation due to facial and oropharyngeal changes, difficult extubation due to epiglottis subsidence, prolonged action of neuromuscular blocking agents and increased sensitivity to opioids.Harmful effects of inhalational anaesthetic agents on nucleotide excision repair has been proposed. Planned airway management is necessary, short acting opioids with the use of multimodal analgesia is preferred and total intravenous anaesthesia (TIVA) is preferred to inhalational anesthetics. The novel anaesthetic agent Dexmedetomidine, alpha-2 agonist could be a valuable anaesthetic adjunct to TIVA. Besides, it also reduces the induction and maintenance dose of propofol, blunts the airway reflex during intubation and extubation, decreases the requirement of opioids and also helps to enhance the recovery.

 

We present 3 cases of XP, who underwent surgery using propofol and dexmedetomidine infusion, without the use of muscle relaxants and inhalational agents.

 

DOI: http://doi.org/10.4038/slja.v24i2.8144
How to Cite: Parajuli, B.D. et al., (2016). Dexmedetomidine as an anaesthetic adjunct for total intravenous anaesthesia in patients with xeroderma pigmentosum.. Sri Lankan Journal of Anaesthesiology. 24(2), pp.92–95. DOI: http://doi.org/10.4038/slja.v24i2.8144
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Published on 16 Jul 2016.
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