High flow nasal oxygenation and videolaryngoscopy - the two saviours in a case of Prader-Willi syndrome: A case report

Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by hypothalamic-pituitary abnormalities, hypotonia, hyperphagia, and a high risk of morbid obesity, which complicates both general and regional anesthesia. Patients with PWS face additional challenges, including disturbances in thermoregulation, arrhythmias, cor pulmonale, and convulsions. These factors make anesthetic management particularly challenging, necessitating careful planning and monitoring. We report the anesthetic management of a 7-month-old infant with genetically confirmed PWS who underwent bilateral orchidopexy under general anesthesia. The patient’s preoperative evaluation revealed significant hypotonia and a predisposition to respiratory complications. During the procedure, advanced airway management techniques, including the use of C-MAC and high-flow nasal oxygenation (HFNO), were employed to alleviate the risk of difficult airway and respiratory failure. The anesthesia team meticulously monitored the patient's vital signs and thermoregulation throughout the surgery to prevent arrhythmias and other complications associated with PWS. The successful anesthetic management of a child with Prader-Willi syndrome requires a multidisciplinary approach, thorough preoperative planning, meticulous intraoperative monitoring, and vigilant postoperative management to minimize the risk of complications in this vulnerable patient population.

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Publication Details

Journal
Indian Journal of Clinical Anaesthesia
Published
2024-11-07
DOI
https://doi.org/10.18231/j.ijca.2024.109
Primary Topic
Genetic Syndromes and Imprinting
Type
article
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article

High flow nasal oxygenation and videolaryngoscopy - the two saviours in a case of Prader-Willi syndrome: A case report

Kush Sharma, Prachi Gaba, Lalit Gupta, A. U. Singh
Indian Journal of Clinical Anaesthesia
Genetic Syndromes and Imprinting
article

High flow nasal oxygenation and videolaryngoscopy - the two saviours in a case of Prader-Willi syndrome: A case report

Kush Sharma, Prachi Gaba, Lalit Gupta, A. U. Singh
article en

Abstract

Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by hypothalamic-pituitary abnormalities, hypotonia, hyperphagia, and a high risk of morbid obesity, which complicates both general and regional anesthesia. Patients with PWS face additional challenges, including disturbances in thermoregulation, arrhythmias, cor pulmonale, and convulsions. These factors make anesthetic management particularly challenging, necessitating careful planning and monitoring. We report the anesthetic management of a 7-month-old infant with genetically confirmed PWS who underwent bilateral orchidopexy under general anesthesia. The patient’s preoperative evaluation revealed significant hypotonia and a predisposition to respiratory complications. During the procedure, advanced airway management techniques, including the use of C-MAC and high-flow nasal oxygenation (HFNO), were employed to alleviate the risk of difficult airway and respiratory failure. The anesthesia team meticulously monitored the patient's vital signs and thermoregulation throughout the surgery to prevent arrhythmias and other complications associated with PWS. The successful anesthetic management of a child with Prader-Willi syndrome requires a multidisciplinary approach, thorough preoperative planning, meticulous intraoperative monitoring, and vigilant postoperative management to minimize the risk of complications in this vulnerable patient population.

Indian Journal of Clinical AnaesthesiaVol. 11(4)
Maulana Azad Medical College (IN)
Openalex Percentile: Top 77%
Genetic Syndromes and Imprinting
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