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.
Affiliation
Department of Anesthesiology and ICM, Faculty of Medicine, Ankara University, Mesa Koza sitesi, 2.blok no:15, GOP, 06700, Ankara, Turkey, basakceyda@hotmail.com.
Journal Details
This article was published in the following journal.
Name: Journal of anesthesia
ISSN: 1438-8359
Pages:
Links
- 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
Prader-willi Syndrome
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)
Anesthesia, Closed-circuit
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.
Laryngeal Masks
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.
Recovery Room
Hospital unit providing continuous monitoring of the patient following anesthesia.
PubMed Articles
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Neurobehavioral phenotype in Prader-Willi syndrome.
The focus of this article is on the lifetime development of people with Prader-Willi syndrome (PWS) and specifically on the neurobehavioral phenotype. We consider studies of this aspect of the phenoty...
Loss of the Prader-Willi obesity syndrome protein necdin promotes adipogenesis.
We investigated the role of necdin during adipogenic differentiation. Necdin is one of several genes inactivated in children with Prader-Willi syndrome, who are predisposed to increased adiposity at t...
Exploring Patterns of Unwanted Behaviours in Adults with Prader-Willi Syndrome.
BACKGROUND: Obsessive-compulsive (O-C) traits, and excessive food intake are well known behavioural manifestations among individuals with Prader-Willi Syndrome (PWS). Other unwanted behaviours are als...
Clinical Trials
Gut Derived Hormones, Body Composition and Metabolism in Prader-Willi Syndrome
The purpose of this study is to investigate the effects of a GLP-1 agonist on satiety hormones in patients with Prader-Willi Syndrome (genetic defect causing obesity).
Cortisol Activity in Patients With Prader-Willi Syndrome and Healthy Controls
The purpose of the study is to find out if people with Prader-Willi syndrome have a difference in the protein which changes inactive cortisone to the active stress hormone cortisol.
Treatment of Self-Injurious Behavior in Individuals With Prader-Willi Syndrome
Prader-Willi syndrome (PWS) is a genetic disorder usually caused by the deletion of a specific gene. One of the symptoms of PWS is self-injurious behavior (SIB); a common form of SIB in P...
Exploring Stress and Coping Behaviors of the Major Carer Whose Children With Prader-Willi Syndrome
Ask the 4 carers of children with Prader-willi syndrome to disclose their experiences and difficulties for searching better management and intervention
Prader-Willi syndrome (PWS) is a genetic disorder characterized by childhood-onset obesity and endocrine dysfunction that leads to cardiovascular disability and early death within the firs...
