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Anesthetic management in a pediatric patient with infantile phase Prader-Willi Syndrome: A case report
Jae Young Kim1, Ji Hyang Lee1, Eun Joo Kim1
1Department of Anesthesiology and Pain Medicine, Daegu Fatima Hospital, Daegu, Korea. lovehan3@yahoo.co.kr.
Insights
Prader-Willi syndrome (PWS) patients require cautious anesthetic management due to infantile hypotonia. This case study highlights successful general anesthesia without muscle relaxants in a 5-month-old infant with PWS.
Area of Science:
- Genetics
- Pediatrics
- Anesthesiology
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder.
- PWS presents with infantile hypotonia, obesity, developmental delay, and hypogonadism.
- The syndrome has distinct infantile hypotonic and childhood obese phases.
Purpose of the Study:
- To describe the anesthetic management of a pediatric patient with Prader-Willi syndrome.
- To emphasize the importance of avoiding muscle relaxants in infants with PWS.
Main Methods:
- Case report of a 5-month-old female infant with PWS.
- General anesthesia administered for excision of BCG lymphadenitis and a preauricular mass.
- Anesthetic plan specifically excluded the use of muscle relaxants.
Main Results:
- Successful anesthetic management was achieved without the use of muscle relaxants.
- The patient tolerated the procedure well under general anesthesia.
- No adverse postoperative events related to anesthesia were noted.
Conclusions:
- Anesthetic management in infants with Prader-Willi syndrome requires careful consideration of hypotonia.
- Avoiding muscle relaxants can be a safe and effective strategy in this patient population.
- This case supports cautious anesthetic planning for PWS patients.
Abstract:
Prader-Willi syndrome is a genetic disorder characterized by infantile hypotonia, childhood obesity, characteristic facial appearance, mental retardation, hypogonadism and short stature. It is described as a 2-stage disorder with an infantile hypotonic phase, followed by a childhood obese phase. The first phase, during the newborn and infancy period, is characterized by marked hypotonia, poor sucking, swallowing, coughing, crying, and episodes of asphyxia. Since these signs of poor strength cause poor reversal in the postoperative period, muscle relaxants should be used cautiously, especially in patients in the first phase of the syndrome. We experienced the anesthetic management of a 5-month-old female patient with Prader-Willi syndrome under general anesthesia, without muscle relaxant for excision of BCG lymphadenitis and a preauricular mass.
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