What are the components of treatment for Prader-Willi syndrome (PWS)?

Updated: Oct 10, 2018
  • Author: Ann Scheimann, MD, MBA; Chief Editor: Luis O Rohena, MD, FAAP, FACMG  more...
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Answer

Patients with Prader-Willi syndrome frequently require medical care for the following [5] :

  • Initial management of hypotonia or poor feeding
  • Evaluation for hypogonadism or hypopituitarism
  • Management of obesity
  • Monitoring for scoliosis
  • Therapy for behavioral issues

On June 20, 2000, the US Food and Drug Administration (FDA) approved the use of growth hormone in children with genetically confirmed Prader-Willi syndrome and evidence of growth failure. [8, 9, 10]

Patients with Prader-Willi syndrome may require surgical care for treatment of complications of obesity, treatment of cryptorchidism, and scoliosis intervention. They may require urgent surgical attention for abdominal issues. Because of a high pain tolerance and decreased ability to vomit, they may present late with symptoms of cholecystitis, appendicitis, or acute gastric dilation with risk for progression to necrosis. [11, 12]

Tonsillectomy, adenoidectomy, or tracheostomy placement may be required in patients with obstructive sleep apnea.


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