What is the treatment for Twiddler's syndrome?

Treatment of Twiddler's Syndrome

The definitive treatment for Twiddler's syndrome is surgical revision of the device pocket with active fixation of the leads and securing the pulse generator to the fascia to prevent recurrence.

Definition and Mechanism

Twiddler's syndrome is a rare but potentially dangerous complication of implantable cardiac devices (pacemakers or implantable cardioverter-defibrillators) characterized by:

• Malfunction of the device due to lead dislodgement
• Caused by rotation of the pulse generator in its subcutaneous pocket
• May be deliberate, subconscious, or in some cases "spontaneous"

Risk Factors

Patients at higher risk include:

• Elderly individuals 1
• Obese patients with loose subcutaneous tissue 1
• Patients with psychiatric conditions, particularly obsessive-compulsive disorder 2
• Implantation of devices with specific shapes that may be prone to rotation 3

Diagnosis

Diagnosis is typically made when a patient presents with:

• Device malfunction (inappropriate shocks, failure to pace)
• Radiographic evidence of lead dislodgement
• Visible or palpable rotation of the device in the pocket
• Some patients may be asymptomatic despite device malfunction 1

Treatment Algorithm

1. Immediate Management:

   • Assess for hemodynamic stability
   • Obtain chest radiography to confirm lead dislodgement
   • Device interrogation to evaluate extent of malfunction

2. Definitive Treatment:

   • Surgical revision of the device pocket 4, 1
   • Replacement of dislodged leads if damaged
   • Active fixation of leads (critical for prevention of recurrence) 4
   • Secure pulse generator to the fascia with non-absorbable sutures
   • Consider creating a smaller, tighter pocket to limit device movement

3. Post-Surgical Management:

   • Device interrogation to confirm proper function
   • Patient education regarding avoidance of manipulation
   • Consider psychiatric evaluation if manipulation was deliberate 2

Prevention Strategies

• Pre-implant psychiatric assessment for patients with risk factors 2
• Patient education about avoiding manipulation of the device
• Active fixation of leads rather than passive fixation 4
• Secure anchoring of the pulse generator to the fascia
• Creation of appropriately sized device pockets
• Consider device shape when selecting implantable devices 3

Special Considerations

• Twiddler's syndrome can occur very early (within 48 hours) or years after implantation 4
• Some cases may be "spontaneous" due to device shape rather than patient manipulation 3
• The syndrome has been reported with other implantable devices including sacral neuromodulators 5
• Psychiatric intervention may be necessary to prevent recurrence in patients with compulsive behaviors 2

Monitoring After Treatment

• Regular device checks to ensure proper function
• Radiographic evaluation if any suspicion of recurrence
• Long-term follow-up to monitor for potential recurrence