What is the treatment for Twiddler's syndrome?

Treatment for Twiddler's Syndrome

The definitive treatment for Twiddler's syndrome is surgical revision with device repositioning and securing the pulse generator to the underlying fascia to prevent recurrence. 1, 2

Understanding Twiddler's Syndrome

Twiddler's syndrome is a complication of cardiac implantable electronic device (CIED) implantation characterized by:

• Deliberate or unconscious manipulation of the device by the patient
• Rotation of the pulse generator within its subcutaneous pocket
• Dislodgement and retraction of the leads toward the pocket
• Subsequent device malfunction

Diagnostic Approach

When Twiddler's syndrome is suspected:

1. Device interrogation: Will show lead dislodgement, inappropriate shocks, or pacing failure
2. Chest radiography: Will demonstrate lead dislodgement and rotation of the pulse generator
3. Patient assessment: May reveal unawareness of device manipulation in many cases 2

Treatment Options

Several surgical approaches have proven effective:

1. Complete System Revision

• Removal of the existing device and leads
• Implantation of a new system with:
  • Creation of a tighter pocket
  • Securing the generator to the fascia with non-absorbable sutures
  • Consideration of active fixation leads 1, 3

2. Submuscular Pocket Placement

• Disconnection of the pulse generator
• Creation of a submuscular pocket
• Anchoring the device to the pectoralis major muscle
• Reconnection of existing leads if viable 1

3. Leadless Device Implantation

• Complete removal of the traditional system
• Implantation of a leadless pacemaker (e.g., Medtronic Micra VR) when appropriate for the patient's pacing needs 1

Prevention Strategies

For high-risk patients (elderly, obese, or those with loose subcutaneous tissue):

• Active fixation leads: Preferred over passive fixation to prevent lead dislodgement 3
• Smaller device pocket: Create a pocket that closely fits the device
• Secure anchoring: Suture the generator to the underlying fascia
• Patient education: Clear instructions against manipulating the generator
• Device selection: Consider device shape, as certain elongated designs may be more prone to spontaneous rotation 4

Follow-up Recommendations

After surgical revision:

• Regular device checks at 1 week, 1 month, and then every 3-6 months
• Chest radiography at follow-up visits to confirm proper device position
• Reinforcement of patient education regarding device manipulation

Special Considerations

Twiddler's syndrome can occur with various implantable devices beyond cardiac devices, including:

• Deep brain stimulators
• Intrathecal drug delivery devices
• Sacral neuromodulation devices 5

The syndrome can present very early (within 48 hours of implantation) or much later, so vigilance is required throughout follow-up 3.