What is the role of surgery in the treatment of 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

See the list below:

  • 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 the 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.

  • Biliopancreatic diversion and gastric bypass surgery have been ineffective for long-term weight reduction. [56, 57] Significant disruption in the enterohepatic circulation of bile acids may result in deficiencies of fat-soluble vitamins and steatorrhea with anal pruritus due to bile acids. Anal pruritus may exacerbate rectal-picking compulsions. Deficiencies of fat-soluble vitamins may exacerbate the following:

    • Osteoporosis (vitamin D)

    • Hypochromic anemia (vitamin E)

    • Hyporeflexia (vitamin E)

    • Spinocerebellar ataxia (vitamin E)

    • Coagulopathy (vitamin K)

    • Night blindness (vitamin A)

    • Enhanced susceptibility to infections (vitamin A)


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