Recommendations of Yamashita et al for Anesthetic Management of a Patient with the Cri du Chat (Partial Deletion 5p) Syndrome

Description

A patient with the cri du chat syndrome (partial deletion of the 5p chromosome) may have a number of findings that can affect anesthetic management. Most patients can be managed safely without complications if suitable adjustments are made. the authors are from the University of Hirosaki in Japan.

Potential Risk Factor	Implication
anomalies of the upper airway	difficult intubation
generalized muscular hypotonia	unpredictable effects of muscle relaxants
congenital heart disease	cyanosis, require monitoring
feeding difficulties	recurrent aspiration with chronic pneumonia; protection of airway
mental retardation	limited ability to follow commands

Anomalies of the airway affecting intubation:

(1) laryngeal: (a) small, (b) narrow and diamond shaped, (c) laryngomalacia, (d) vocal cord paralysis

(2) epiglottis: (a) hypotonic, (b) hypoplastic, (c) small and flaccid <?= mixed hypotonia and hypoplasia>, (d) long, curved and floppy

(3) retrognathia

(4) high palatal vault

Intubation should be done by a person with experience who has assistance and the proper equipment readily available.

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Recommendations of Yamashita et al for Anesthetic Management of a Patient with the Cri du Chat (Partial Deletion 5p) Syndrome

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