Clinical Case Report: Recurrent Twiddler’s Syndrome

Bayliss et al were the first to describe Twiddler’s syndrome.¹ In the paper, the authors presented the main characteristics of Twiddler’s syndrome — namely, the twisting or coiling of the lead around the pacing device, followed by lead retraction or lead fracture. They also noted that loss of capture and sensing are typically seen.

Lead retraction is frequently associated with extra cardiac pacing, as the tip of the lead first passes by the phrenic nerve, then the brachial plexus, as it is pulled back toward the pocket. This may result in diaphragmatic stimulation followed by arm twitching. Bayliss reported this “twiddling” in a patient with a thoracic, subcutaneous, transvenous pacing device.¹

The phenomenon has subsequently been described in implantable cardioverter defibrillator (ICD) patients² with abdominal implants and endocardial (transvenous) ICD leads as well as those with thoracic devices and transvenous leads.³ Twiddler’s syndrome has also been described with chemotherapy infusion devices⁴ and deep brain stimulators.⁵

In our clinical practice, we have encountered 12 cases of suspected or confirmed Twiddler’s syndrome among 2,357 implanted devices between 2002 and 2010 (incidence of 0.5%). Several patients twiddled multiple times. There was no difference in the frequency of twiddling seen, whether or not a device suture was used to anchor the pulse generator to the pectoral fasica. Twiddling was seen in both pacemaker and ICD patients.

Six patients underwent device revision with extra anchor sleeves and standard treatment. Of these, 4 (67%) went on to twiddle again, and therefore, required subsequent procedures. This is consistent with other case series, which have shown that a substantial percentage of patients will repeatedly dislodge their leads.

Six patients in our series received the AIGISrx Antibacterial Envelope (TYRX, Inc., Monmouth Junction, NJ) as treatment for Twiddler’s syndrome. These patients required no further procedures. The following case report examines one of the patients from that series.

Case Report

A 63-year-old male underwent implantation of a primary prevention ICD on December 18, 2009. He has a history of coronary artery bypass grafting, ischemic cardiomyopathy (ejection fraction 25–30% by echo), pulmonary embolism with subsequent IVC filter, chronic kidney disease, and chronic systolic congestive heart failure with a narrow QRS complex. As a result, a single lead system was chosen.

The lead was a Durata 7120Q (St. Jude Medical, St. Paul, MN), and the implantable defibrillator a Current Plus VR CD1211-36Q (St. Jude Medical). The implant procedure, using cephalic vein cutdown and a subcutaneous pocket, was unremarkable, with excellent initial fluoroscopic lead appearance and electrical parameters:

• R-wave — 9.7 mV;
• impedance — 460 ohms;
• pacing threshold — 0.75 V at 0.5 milliseconds; and
• defibrillation — threshold 15 joules. 

On the post-procedural X-ray on December 18, the lead position was acceptable. However, loss of capture and sensing were noted on ICD testing the next day (December 19). The patient was brought back to the electrophysiology (EP) lab, where fluoroscopy revealed that the tip of the ICD lead lay in the right atrium. A single loop of lead was wrapped around the ICD device. The lead was repositioned with excellent electrical parameters, and fluoroscopic positioning was obtained. The pocket size was acceptable, and there was no excessive mobility of the device in the pocket. Due to suspicion of Twiddler’s syndrome, the lead was secured to the pectoralis fascia with two lead-anchor sleeves at right angles to each other.

During office follow-up at 1 week, normal parameters were obtained (a chest radiograph was not done). One-month routine device check again showed failure to pace or sense, and the patient was scheduled for lead revision or possible extraction with reimplantation.

On February 10, 2010, the patient was brought back to the EP lab for revision. Fluoroscopy demonstrated the lead was wrapped multiple times around the ICD with the proximal coil contained entirely within the ICD pocket. In addition, the distal lead tip lay in the left innominate vein. No lead slack was seen. The lead anchor sleeves were well visualized at fluoroscopy and lay lateral to the ICD can (they had initially been medial), which was consistent with the anchors being pulled around the ICD can with the lead.

The lead was found to be damaged — the active fixation screw retracted, but would not re-extend — and was therefore explanted and replaced. Both anchor sleeves were still attached to the lead, but their sutures had been pulled through their pectoral muscle attachments. Given the evidence for Twiddler’s syndrome, a bacteriostatic pouch (AIGISrx Antibacterial Envelope) was used to secure the device and lead in the pocket.

Upon questioning the patient after the second procedure, he adamantly denied ever touching the ICD pocket. However, his wife and two adult children pointed out that he was “constantly” fidgeting with his ICD site. In more than two years of follow-up since his second lead revision procedure and placement of the AIGISrx Antibacterial Envelope, no further lead dislodgement issues have been encountered.

Discussion

The best approach to Twiddler’s syndrome is prevention. Careful matching of the size of the device pocket to the implanted device is essential for patient comfort as well as to limit the available room in the pocket for pulse-generator rotation. In addition, carefully anchoring the transvenous lead to the pectoral muscle is essential. It is reasonable to consider submuscular device implantation in patients with loose subcutaneous tissue, such as elderly, obese females.

When Twiddler’s syndrome has been confirmed, the leads are typically re-anchored to the pectoral muscle with multiple anchoring sleeves. In addition, the device is often aggressively anchored to the pectoral muscle as well. Woven Dacron pouches have been available for many years and have been shown to efficaciously prevent device migration and flipping.⁶ Despite these interventions, repeat lead dislodgement is a serious clinical issue.

The AIGISrx Antibacterial Envelope is an open-weave, knitted polypropylene mesh coated with a polymer that carries rifampin and minocycline. The pouch is typically used in patients at high risk of device infection at the time of ICD implantation or generator change. However, it is also indicated for improving device stability in the pocket by reducing device migration and rotation.

The mechanism by which the AIGISrx Antibacterial Envelope may reduce subsequent twiddling is unknown. The initial cause of twiddling is likely an overly capacious pocket. Conversely, the pocket may be expanded by the forced rotation of an asymmetric ICD or pacemaker can.

The AIGISrx Antibacterial Envelope most likely prevents further device rotation by taking up this extra pocket volume and reducing room for rotation. Additionally, the pouch itself is somewhat coarse and rough. This may provide increased friction at what is normally a lubricous interface between the pacing device and the tissue surrounding it.

Conclusion

While twiddling is certainly uncommon (incidence of 0.07–1.1% in published reports^7,8) it remains a significant clinical problem, as damage to or dislodgement of leads will result in loss of pacing with possible injury or death in pacing-dependant patients. Furthermore, in ICD patients, particularly in patients in whom the device was placed for primary prevention, dislodgement of the ICD lead is frequently asymptomatic as these patients nearly always have the ICD programmed to minimize ventricular pacing. As a result, the only symptom in such patients may be failure to detect or treat potentially lethal ventricular arrhythmias.

References

1. Bayliss CE, Beanlands DS, Baird RJ. The pacemaker-twiddler’s syndrome: a new complication of implantable transvenous pacemakers. Can Med Assoc J 1968;99(8):371–373.
2. Boyle NG, Anselme F, Monahan KM, et al. Twiddler’s syndrome variants in ICD patients. Pacing Clin Electrophysiol 1998;21(12):2685–2687.
3. Higgins SL, Suh BD, Stein JB, et al. Recurrent Twiddler’s syndrome in a nonthoracotomy ICD system despite a Dacron pouch. Pacing Clin Electrophysiol 1998;21(1):130–133.
4. Rodan BA, Martyak SN. Twiddler’s syndrome: another twist. South Med J 1988;81(3):418–419.
5. Penn DL, Wu C, Skidmore C, et al. Twiddler’s syndrome in a patient with epilepsy treated with deep brain stimulation. 3 May 2012 (epub ahead of print).
6. Shandling AH, Ellestad MH, Castellanet MJ, Messenger JC. Dacron-woven pacemaker pouch. Influence on long-term pacemaker mobility. Chest 1991;99(3):660–662.
7. Fahraeus T, Höijer CJ. Early pacemaker twiddler syndrome. Europace 2003;5(3):279–281.
8. Hill PE. Complications of permanent transvenous cardiac pacing: a 14-year review of all transvenous pacemakers inserted at one community hospital. Pacing Clin Electrophysiol 1987;10(3 Pt 1):564–570.