Twiddler’s Syndrome is a rare complication that affects individuals with implanted medical devices. It involves manipulation of an implanted device, leading to malfunction. Understanding it is important for patients and caregivers due to its impact on device function and patient well-being. First described in 1968, it highlighted an unusual interaction between a patient and their life-supporting implant.
How Twiddler’s Syndrome Happens
Twiddler’s Syndrome occurs when a patient, either consciously or subconsciously, manipulates the implanted device within its subcutaneous pocket, causing it to rotate on its axis. As the device rotates, the electrical leads connected to it become coiled, twisted, or retracted. This physical stress can lead to the dislodgement of the leads from their intended position, or in some cases, cause damage like fracture or insulation leakage. For instance, in pacemakers, leads may retract from the heart, ceasing to deliver necessary electrical impulses. While most commonly associated with pacemakers, Twiddler’s Syndrome has also been reported in patients with implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy devices.
Who Is at Risk
Patient characteristics and surgical factors increase the likelihood of Twiddler’s Syndrome. Older age is a risk factor, partly due to increased subcutaneous tissue laxity, allowing easier device movement. Cognitive impairment, such as dementia, and psychiatric conditions also elevate risk, as these can lead to impaired judgment or restless behaviors that result in device manipulation. Obesity is another factor, as excess soft tissue can provide a looser environment for the device, making it more prone to rotation. Additionally, the size of the device pocket relative to the device itself plays a role; a larger or poorly secured pocket offers more room for the device to move and rotate. Female gender and pediatric age groups are also at increased risk.
Recognizing the Signs
Signs of Twiddler’s Syndrome include symptoms of device malfunction and physical changes at the implant site. If the device is a cardiac implant, such as a pacemaker or ICD, patients may experience symptoms like dizziness, fainting spells (syncope), palpitations, or shortness of breath due to the device failing to regulate heart rhythm effectively. Inappropriate or painful shocks can occur in ICD patients if the device misinterprets signals due to lead displacement. Physical signs include visible displacement or rotation of the device, skin erosion over the implant, or localized pain and swelling. A distinctive sign can be the appearance of “bunching” or coiling of the lead wires under the skin, or rhythmic muscle twitching in the arm or abdomen if leads stimulate nearby nerves like the brachial plexus or phrenic nerve.
Managing and Preventing Twiddler’s Syndrome
Diagnosis
Diagnosis involves clinical suspicion, physical examination, and imaging. A chest X-ray is often the most effective diagnostic tool, providing clear images of lead coiling or device rotation. Device interrogation, using specialized programming equipment, can confirm malfunction by revealing abnormal lead parameters, such as increased pacing thresholds or loss of capture.
Treatment
Treatment often requires surgical intervention. This may involve repositioning the displaced device and leads, re-implanting the system, or replacing damaged leads. The pulse generator is then securely sutured to the underlying fascia to prevent future rotation, and a smaller, tighter device pocket may be created. In some instances, a leadless pacing system might be considered.
Prevention
Prevention focuses on careful surgical techniques and patient education. During initial implantation, measures such as creating a snug device pocket and securely suturing the device and leads can minimize movement. Patient and family education is important, emphasizing avoidance of implant site manipulation. For individuals at higher risk, such as those with cognitive impairments, ongoing counseling and vigilance are beneficial.