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.

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