What are the risks of complications during surgery for patients with Prader-Willi syndrome?

Surgical Risks in Prader-Willi Syndrome

Patients with Prader-Willi syndrome face significantly elevated perioperative risks including respiratory complications, temperature dysregulation, increased anesthetic sensitivity, and high rates of postoperative complications—particularly respiratory failure, wound infections, and spinal cord injury in orthopedic procedures.

Critical Perioperative Risks

Respiratory Complications

• Obstructive sleep apnea is highly prevalent and increases risk of perioperative respiratory failure, requiring preoperative sleep evaluation 1
• Central control of ventilation is abnormal, leading to unpredictable responses to anesthetic agents and increased risk of postoperative apnea 2
• Patients require intensive postoperative monitoring with continuous oximetry for at least 24-48 hours, even after minor procedures 1

Anesthetic Sensitivity

• Markedly increased sensitivity to opioids necessitates dose reduction by approximately 50% or complete avoidance 1
• Intraoperative opioids should be minimized and replaced with non-opioid analgesics (acetaminophen, dexmedetomidine) to prevent respiratory depression 1
• General anesthesia carries higher risk of delayed emergence and prolonged respiratory depression 1, 2

Temperature Dysregulation

• Abnormal central temperature control increases risk of perioperative hypothermia or hyperthermia 2
• Temperature extremes must be actively prevented in the operating room 1

Orthopedic Surgery-Specific Risks

Scoliosis Surgery Complications

• Complication rates reach 75% in PWS patients undergoing spinal surgery, substantially higher than general population 3
• Major complications include proximal junctional kyphosis requiring revision (25%), deep infections (25%), and catastrophic spinal cord injury with permanent paraplegia 3, 4
• Four patients developed severe kyphosis above fusion requiring reoperation, with three resulting in permanent spinal cord injury 4

Fracture and Bone Concerns

• Osteopenia affects 57% and osteoporosis affects 29% of PWS patients, increasing fracture risk and surgical fixation challenges 5
• Diminished pain sensitivity masks fractures and complications, delaying diagnosis and treatment 1, 5
• Fracture management is associated with frequent minor complications due to poor bone quality 5

Behavioral and Psychiatric Risks

Perioperative Management Challenges

• 84% have Axis I psychiatric diagnoses including impulse control disorder, oppositional defiant disorder, and aggressive behaviors that complicate postoperative care 5
• Self-mutilating behaviors occur in 29% of patients, requiring specialized behavioral management 5
• Aggressive and violent behavior may emerge perioperatively, necessitating psychiatric consultation and behavioral protocols 2

Pain Assessment Difficulties

• High pain tolerance masks serious complications including intestinal necrosis after binge-eating, which can be fatal if unrecognized 1
• Absence of vomiting despite severe pathology is common; vomiting after binge-eating is an ominous sign requiring immediate evaluation 1

Metabolic and Endocrine Risks

• Glucose intolerance and diabetes increase infection risk and wound healing complications 1, 2
• Morbid obesity (common in PWS) independently increases surgical site infection risk and wound complications 1
• Hypogonadism and endocrine abnormalities may affect stress response to surgery 5, 2

Recommended Perioperative Protocol

Preoperative Optimization

• Mandatory sleep study evaluation for all patients, with treatment of sleep apnea before elective surgery 1
• Bone density assessment via DEXA scan to identify osteopenia/osteoporosis requiring perioperative management 5
• Psychiatric evaluation and behavioral management plan established preoperatively 5
• Optimize glucose control and screen for diabetes 1

Intraoperative Management

• Regional anesthesia should be strongly considered as primary technique when feasible to avoid general anesthesia complications 2
• If general anesthesia required: minimize or eliminate opioids, use short-acting agents, avoid nitrous oxide in patients with potential pulmonary hypertension 1
• Active temperature management throughout procedure 2
• For spinal surgery: multilevel pedicle screw fixation is preferred technique; avoid traditional growing rods due to high complication rates 3

Postoperative Management

• Admit all PWS patients to ICU or high-dependency unit for minimum 24-48 hours continuous cardiopulmonary monitoring, regardless of procedure complexity 1
• Non-opioid analgesia as primary pain management strategy 1
• If opioids necessary: use 50% reduced doses with extended monitoring periods 1
• Maintain high index of suspicion for masked complications due to diminished pain sensitivity 1, 5

Critical Pitfalls to Avoid

• Never discharge PWS patients to regular ward settings after any surgical procedure; extended monitoring is mandatory even for "minor" procedures 1
• Do not rely on patient pain reporting to detect complications; maintain systematic surveillance for infection, fracture, or other pathology 1, 5
• Avoid standard opioid dosing protocols; respiratory depression occurs at much lower doses than general population 1
• In spinal surgery, recognize that proximal junctional kyphosis and instrumentation failure rates are exceptionally high; plan fusion levels accordingly 3, 4
• Do not underestimate infection risk; deep wound infections occur frequently and may require hardware removal 3, 4