Reel Versus Twiddler Syndrome in a Patient With a Pacemaker: A Case Report of Iatrogenic Manipulation.

Twiddler syndrome is a rare entity in which patient manipulation causes lead dislocation with lead retraction. Reel syndrome, on the other hand, is observed after external manipulation, intentional or unintentional, in which the leads move along their transverse axis and is considered a variant of Twiddler syndrome. We present the clinical case of a 91-year-old female who, after pacemaker implantation, presented with dizziness and chest discomfort following constant manipulation of the pacemaker, resulting in complete retraction of the lead tip into the pouch, which is unusual in the literature to date.

An unusual cause of inappropriate shocks delivered by an implantable cardioverter defibrillator.

INTRODUCTION: Cardiac implantable electronic device (CIED) complications present significant challenges in clinical practice, especially in elderly patients with multiple comorbidities. Common adverse events include infection, lead malfunction, and device migration. Twiddler's Syndrome, a rare but serious CIED complication characterised by patient manipulation causing lead displacement and device malfunction, is often underreported. The literature consists mainly of case reports and small series, providing limited guidance on prevention and management. As CIEDs are critical for managing cardiac arrhythmias and heart failure, understanding and addressing Twiddler's Syndrome is essential. This case report aims to contribute to the literature by detailing a case of Twiddler's Syndrome, emphasising the importance of a multidisciplinary approach for optimal management. CASE PRESENTATION: A 59-year-old male presented with discomfort around his implantable cardioverter defibrillator (ICD) site and the sternal area over the past two days. He denied pain, dyspnoea, or dizziness. Clinical examination revealed a normal heart rhythm and no peripheral pulse deficit. Ultrasound revealed a reduced left ventricular ejection fraction. The atrial lead was not visible, and the shock coil was misplaced. ICD interrogation showed inappropriate shocks due to sensing artifacts and exit block in both leads, with no arrhythmias detected. An X-ray confirmed lead dislodgement and significant entanglement in the pocket. The patient was diagnosed with Twiddler's Syndrome and scheduled for surgical revision. DISCUSSION/CONCLUSIONS: Dilated cardiomyopathy (DCM), characterised by left ventricular dilatation and dysfunction, accounts for a significant proportion of systolic heart failure cases. Despite advancements in heart failure management, DCM patients remain at high risk for sudden cardiac death (SCD), making ICD implantation crucial. However, CIED placement carries risks of complications, including Twiddler's Syndrome. This condition can lead to lead dislodgement and device malfunction, resulting in inappropriate shocks and potential patient harm. In this case, a single-session extraction and re-implantation were successfully performed using a multidisciplinary approach, emphasising the importance of comprehensive management strategies to address such complications effectively. Regular follow-up showed no adverse events, highlighting the procedure's success and the potential benefits of using advanced antimicrobial adjuncts to prevent infections. This case underscores the need for awareness and standardised protocols for managing Twiddler's Syndrome to improve patient outcomes in the growing population of CIED recipients.

Twiddler's Syndrome.

Teaching point: Twiddler's syndrome is a very rare but potentially disastrous complication after implantation of a neurostimulator or pacemaker, caused by twisting of the generator within the subcutaneous pocket, resulting in dislodgement and/or interruption of the electrodes that should be reported on x-ray.

Twiddler's Syndrome Causing Lead Fracture in Sacral Neuromodulation: A Case Report.

Twiddler's syndrome is the voluntary or involuntary manipulation of an implanted device, most described in cardiac literature. Lead coiling may result in device malfunction due to lead migration or, less commonly, lead fracture. There are few but increasing reports of Twiddler's syndrome resulting in lead migration in sacral neuromodulation, but lead fracture has not yet been described. A 57-year-old Latina female presented with fecal incontinence and refractory overactive bladder. She underwent successful implantation of a sacral neuromodulation device with the resolution of symptoms. Following significant weight loss and two falls, she developed a recurrence of symptoms and was found to have lead migration on pelvic radiographs. At the time of surgical intervention, radiographs demonstrated worsened Twiddler's syndrome with complete lead fracture despite no further trauma. She subsequently underwent partial lead removal and replacement with additional measures to prevent Twiddler's syndrome and its sequelae. Twiddler's syndrome resulting in lead fracture can occur in sacral neuromodulation. Preventive techniques may be applied for patients with known risk factors for Twiddler's syndrome, especially generator anchoring and lead replacement.

Asymptomatic cable twisting in a patient with impending Twiddler syndrome detected during deep brain stimulation surgery for Parkinson's disease: A case report.

Background: Deep brain stimulation (DBS) has consistently demonstrated high efficacy and safety in patients with Parkinson's disease. Twiddler's syndrome is a rare occurrence of hardware failure in patients undergoing neuromodulation. We report here a case of subclinical cable twisting jeopardizing Twiddler's syndrome in a patient with Parkinson's disease who underwent DBS surgery targeting the globus pallidus internus (GPI). Case Description: A 70-year-old woman with a 7-year history of Parkinson's disease refractory to medication was referred to our department for treatment of involuntary movements of the left hand and leg. She underwent right GPI DBS implantation. Left GPI DBS implantation was subsequently planned to manage resting tremors that developed in the right leg after the first surgery at around one year after the first surgery. During a routine check-up before the second surgery, we incidentally detected Twiddler's syndrome. The patient showed no neurological deficits in the left extremities, the same as before right GPI DBS. We performed left GPI DBS concomitantly with the revision of the implantable pulse generator and extension wire. Conclusion: Twiddler's syndrome is a rare complication of DBS. Subclinical risk of cable twisting jeopardizing Twiddler's syndrome is rarely detected without clinical indications of hardware failure. Neurosurgeons should be cognizant of and regularly monitor the implanted device in case serious complications occur.

Cardiovascular images: pacemaker-lead fracture and excessive coiling in a dog.

A seven-year-old male castrated Labrador Retriever presented emergently due to concern for pacemaker malfunction five years after successful transvenous pacemaker implantation to treat partial atrial standstill. On presentation, the dog's pulse rate was 30-50 beats per minute. An electrocardiogram showed no spontaneous atrial activity or paced ventricular activity. Pacemaker interrogation revealed an increased impedance of 7557 ohms, indicating a lead malfunction. Thoracic radiographs confirmed the lead was fractured and had excessive coiling. The transvenous pacing system was turned off, left in place, and an epicardial pacing system was implanted the following day. The dog was discharged with no perioperative complications. The dog eventually required escalated medical therapy for progressive cardiac disease and was euthanized two years after implantation of the replacement pacemaker. This manuscript illustrates a complete lead fracture and excessive lead coiling, which has not previously been detailed in veterinary medicine.

A case series of the twiddler syndrome.

Background: Twiddler syndrome (TS) is a complication of cardiac implantable electronic device (CIED) implantation, caused by the deliberate or unconscious manipulation of the device by the patient himself, which results in dislocation of the leads by retraction towards the subcutaneous pocket. Case summary: This report describes two clinical cases that occurred in our centre, for which two different solutions were successfully implemented. In the first case, a complete removal of the stimulation system was performed, and a leadless pacemaker (PM; Medtronic Micra VR) was implanted. In the second case, the patient underwent a revision procedure. The PM was disconnected, and the electrodes were debrided, a submuscular pocket for the PM was created, and at the end of the procedure, the PM was anchored to the pectoralis major. Discussion: Twiddler syndrome is a not so rare and serious complication of CIED implantation, leading to device malfunctioning and higher risk of infection of the pocket due to multiple re-interventions. In these two cases, different surgical solutions were performed, both resulting to be effective to solve the effects of TS.

Twiddler's syndrome after dorsal root ganglion stimulation: A case report.

Dorsal root ganglion stimulation (DRG-S) is a promising therapy for chronic neuropathic pain, but complications of this therapy are poorly understood. Twiddler's syndrome, a rare complication characterized by lead displacement and coiling of wires, has been reported in other neuromodulation devices, but has not been described in the context of DRG-S. Here, we present a first-of-a-kind case report of Twiddler's syndrome occurring after 8 months of DRG-S. This case report highlights the importance of considering Twiddler's syndrome as a potential complication in patients undergoing DRG-S, especially in those with significant weight loss history.

Hematoma-induced Twiddler-like phenomenon as a presentation of DBS hardware failure: Case report.

Deep brain stimulators (DBS) may fail for a multitude of reasons. We present a 79-year-old Parkinson's disease patient who suffered a DBS failure following impulse generator (IPG) replacement surgery due to the IPG flipping within an expanded capsular pocket. This creation of the pocket was unintentional, and the pocket formed around an undiagnosed postoperative hemorrhage. The syndrome could be considered "Twiddler-like" because it resulted in device flipping. There were, however, many characteristic differences between our case and classical Twiddler's syndrome. DBS neurostimulator failure due to hematoma induced device flipping should be suspected when device interrogation is impossible or there are abnormally high impedances across multiple DBS lead contacts. A plain film X-ray series should be ordered and can be useful in providing radiological evidence of device flipping. In cases like ours the extensive braiding encountered in Twiddler's syndrome may be absent. Anchoring the IPG to a deep fascial layer as well as the use of an antimicrobial pouch are two methods that may be employed to prevent or to treat this complication.

Involuntary arm movements post-pacemaker insertion - real or Reel syndrome?

BACKGROUND: Twiddler syndrome, and the variant Reel syndrome, are rare but important complications of pacemaker implantation. CASE SUMMARY: We describe a rare complication of conventional permanent pacemaker implantation of rhythmic arm twitching secondary to brachial plexus stimulation from a displaced pacing lead caused by Reel syndrome. DISCUSSION: Twiddler syndrome and its variants are rare but important complications of pacemaker insertion. Holistic planning of cardiac procedures in elderly patients should identify those at risk to allow for targeted education and post-procedural care.

Prevalence and predictors of Twiddler's syndrome.

BACKGROUND: Twiddler's syndrome is a poorly understood clinical phenomenon when patients either consciously or subconsciously rotate their cardiac device resulting in lead dislodgement. We aimed to determine the true prevalence and risk factors associated with Twiddler's syndrome in a real-world population. METHODS: A retrospective chart review was performed on all patients who underwent cardiac device implantation from January 1st 2017 until Jan 1st 2022. We specifically searched for the terms "Twiddler" or "Twiddler's" imbedded within the text of the medical chart. Demographic and clinical variables were collected from the electronic medical record system. We utilized multivariable logistic regression analysis as well as Kaplan-Meier prediction models to determine independent clinical predictors of Twiddler's syndrome as well as associated mortality, respectively. RESULTS: Twenty one out of 1793 patients (1.2%) were identified as having Twiddler's syndrome after chart review. Independent variables associated with Twiddler's syndrome were female sex (OR 3.76; 95% CI 1.29-10.95), antidepressant medications (OR 3.58; 95% CI 1.07-11.99), and BMI (OR 1.08; 95% CI 1.03-1.31). There was no increased six-month mortality via Kaplan-Meier analysis. CONCLUSION: Our study shows that 1.2% of patients in our real-world population had evidence of Twiddler's syndrome. Independent predictors of Twiddler's syndrome include female sex, antidepressant medications as well as BMI.

A Rare Case of Rectus Muscle Twitching Due to Abandoned Uncapped Pacemaker Leads.

Extra-cardiac stimulation after cardiac pacemaker implantation is seldom seen in the cardiac field. However, this case report demonstrates an unusual symptom of persistent abdominal twitching in a 42-year-old male patient who underwent pacemaker replacement, lasting for 15 years. Initially, it was attributed to diaphragmatic pacing by the new pacemaker. Despite several attempts to replace the endocardial leads, the patient's symptoms did not improve. Finally, he was referred to our hospital, where our team conducted further investigations and discovered that the old pacemaker lead was exposed, leading to excitation of the rectus muscle.

An unusual cause of pacemaker malfunction: A case report of an association of twiddler and reel syndrome.

Introduction and importance: Lead dislodgement syndromes (Twiddler, Ratchet or Reel syndromes) are rare causes of cardiac stimulation device malfunction that can occur most commonly early after device implantation. Each one of them associated with a unique pattern of lead coiling and dysfunction. Our clinical case reports an unusual association and shed the light on the available diagnostic modalities. Case presentation: A 62-year-old woman who was referred to our hospital for a symptomatic high degree AV block, she underwent dual chamber pacemaker implantation. She experienced 3 weeks following implantation a rhythmic twitching of the right arm without syncope. The device interrogation revealed an increase in both leads pacing impedance and chest X-ray showed leads had pulled out of the heart and were tangling and wrapped repeatedly around the pulse generator. Revision procedure was performed to reposition the leads. Clinical discussion: Recognizing this complication early can prevent life threatening complication and is then of the utmost importance. Twiddler's syndrome is due to rotation of the device along its long axis. Reel syndrome is produced by device rotation along the transverse axis. In most cases, lead replacement or reposition is needed. Preventive measures such as patient education and use of a smaller pocket will reduce the risk of developing the syndrome. Conclusion: Our case highlights the available diagnostic modalities for early detection of twiddler's syndrome. The unique nature of this case increases the importance of considering device lead dislodgement as the cause for patients presenting with extra-cardiac symptoms.

An Atypical Presentation of Twiddler's Syndrome: A Case Report.

The prevalence of implanted pacemaker/defibrillator devices continues to rise. Automatic implantable cardioverter-defibrillator (AICD) lead displacement (Twiddler's syndrome) is an uncommon form of the device malfunction, usually presenting with cardiac symptoms. We present a case of Twiddler's syndrome with an atypical presentation, accompanied by critical alkalosis on arterial blood gas. Considering Twiddler's syndrome as part of the differential diagnosis in patients with implanted devices and utilizing remote ICD interrogation may improve the care of patients presenting with device malfunction.

Twiddler's syndrome: Between mechanical recoil and behavioral troubles.

A 72-year-old male with severe dilated cardiomyopathy, benefited of implantable cardiac defibrillator implementation. Device control shows high impedance. On X-ray, electrodes were completely twisted in the generator pocket, they were replaced and the generator was fixed to pectoralis-major fascia. Nurses report patient abnormal movements, scratching implantation area. This was identified as probably the trigger of the complication.

Reel syndrome: a rare presentation of a rare cause of cardiac resynchronization malfunction.

Abstract: Reel syndrome is a rare cause of pacemaker lead displacement. This case report shows a rare presentation of Reel syndrome highlighting the importance of an early diagnosis and discussing the underlying mechanism, management and prevention.