Anesthesia for a 16-month-old patient with Prader-Willi syndrome.
Summary of "Anesthesia for a 16-month-old patient with Prader-Willi syndrome."
Prader-Willi syndrome (PWS) is a rare disorder of chromosome abnormalities in which the paternal genes in chromosome 15 are lacking. The clinical course is characterized by hypotonia, hyperphagia, and morbid obesity. Both general and regional anesthesia in these patients is difficult due to morbid obesity and hypotonia. We report our anesthetic management in a patient with PWS with a body mass index (BMI) of 29.43 kg/m(2) who underwent orchiopexy and hypospadias repair. The clinical course of the patient was uneventful during the procedure and postoperative period. However, arrangements with a pediatric intensive care setting for the postoperative period are recommended for patients with PWS undergoing surgery.
Department of Anesthesiology and ICM, Faculty of Medicine, Ankara University, Mesa Koza sitesi, 2.blok no:15, GOP, 06700, Ankara, Turkey, firstname.lastname@example.org.
This article was published in the following journal.
Name: Journal of anesthesia
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20711615
- DOI: http://dx.doi.org/10.1007/s00540-010-1005-3
Medical and Biotech [MESH] Definitions
An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229)
Inhalation anesthesia where the gases exhaled by the patient are rebreathed as some carbon dioxide is simultaneously removed and anesthetic gas and oxygen are added so that no anesthetic escapes into the room. Closed-circuit anesthesia is used especially with explosive anesthetics to prevent fires where electrical sparking from instruments is possible.
A type of oropharyngeal airway that provides an alternative to endotracheal intubation and standard mask anesthesia in certain patients. It is introduced into the hypopharynx to form a seal around the larynx thus permitting spontaneous or positive pressure ventilation without penetration of the larynx or esophagus. It is used in place of a facemask in routine anesthesia. The advantages over standard mask anesthesia are better airway control, minimal anesthetic gas leakage, a secure airway during patient transport to the recovery area, and minimal postoperative problems.
Delayed Emergence From Anesthesia
Abnormally slow pace of regaining CONSCIOUSNESS after general anesthesia (ANESTHESIA, GENERAL) usually given during surgical procedures. This condition is characterized by persistent somnolence.
Hospital unit providing continuous monitoring of the patient following anesthesia.
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