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Twiddle Me This! A Look at Twiddler’s Syndrome

2015; Elsevier BV; Volume: 66; Issue: 1 Linguagem: Inglês

10.1016/j.annemergmed.2015.05.018

1097-6760

Jan Shoenberger, Paul Jhun, Aaron Bright, Mel Herbert,

Cardiovascular Syncope and Autonomic Disorders

SEE RELATED ARTICLE, P. 19. “A 53-year-old female 3 days status post pacemaker/defibrillator placement presented to the emergency department (ED) after a syncopal episode. The pacemaker was placed for intermittent complete heart block leading to recurrent syncope. Since discharge, she had complained of incisional pain but denied fever, erythema, or exudate from her incision. On arrival an ECG showed an intermittently paced regular rhythm. The patient became symptomatic with light-headedness when her pulse rate decreased to the rage of 38-42 beats/min. The physical examination result was unremarkable. However, the patient was noted by nursing to be frequently touching her incision.”1Frizell A.W. MacVane C.Z. Woman With Syncope.Ann Emerg Med. 2015; 66: 19Abstract Full Text Full Text PDF Scopus (2) Google Scholar Riddle me this: have you heard of twiddler’s syndrome? Does it have to do with Twizzlers? Tiddlywinks? Twitter? None of those? Troubleshooting a dysfunctional pacemaker can be a challenge, and arriving at a diagnosis that you’ve never heard of can be even more difficult. Fortunately, a simple chest radiograph can help you rule out this problem in a matter of seconds! The first implantable pacemaker was placed in the United States in 1960.2Beck H. Boden W.E. Patibandia S. et al.50th Anniversary of the first successful permanent pacemaker implantation in the United States: historical review and future directions.Am J Cardiol. 2010; 106: 810-818Abstract Full Text Full Text PDF PubMed Scopus (65) Google Scholar It was only a matter of time before problems with pacemakers started to arise, including battery failure, lead fracture and displacement, failure to pace, oversensing, and a host of other issues. It took 8 years for the medical literature to publish the first case of a pacer lead being displaced as a result of the pulse generator rotating in its implanted site.3Bayliss C.E. Beanlands D.S. Baird R.J. The pacemaker-twiddler’s syndrome: a new complication of implantable transvenous pacemakers.CMAJ. 1968; 99: 371-373PubMed Google Scholar This rotation typically occurs accidentally because the subcutaneous pocket, in which the pulse generator is placed, is generously sized. This allows the generator enough room to rotate on its own or, more likely, with the help of a patient who fiddles with or “twiddles” it intentionally or subconsciously, causing it to rotate. That being said, perhaps we shouldn’t be so quick to judge because the rotation may also occur as a result of arm movement.4Bracke F. van Gelder B. Dijkman B. et al.Lead system causing twiddler’s syndrome in patients with an implantable cardioverter-defibrillator.J Thorac Cardiovasc Surg. 2005; 129: 231-232Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar Regardless, given that the leads are attached to the generator, twisting of the generator may cause the leads to also twist and coil up like a fishing reel line, displacing the pacer lead from its intended site. Voilà! You’ve now got pacer dysfunction and a diagnosis of twiddler’s syndrome. It doesn’t take a genius to realize that if the pacer leads aren’t where they are supposed to be, the pacemaker might not work right. But it might take a genius to make this diagnosis from some of the more subtle ways that it can present! For example, a patient who presents with 2 days of right upper quadrant “spasms” wouldn’t initially have a suspected pacemaker cause of those spasms. But what if on physical examination you noted infrequent, palpable spasms to the right side of the abdomen? Holy cannoli! Could it be the pacemaker? Indeed it could. In fact, in one case, the right ventricular pacemaker lead had dislodged, with the lead resting in the superior vena cava and stimulating the phrenic nerve, causing contractions of the right hemidiaphragm, which manifested as visible, palpable spasms. The chest radiograph and ECG made the diagnosis.5Close M.D. Genes N. An unusual presentation of twiddler’s syndrome.J Emerg Med. 2012; 43: 55-56Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Other unusual clinical presentations have been described, including displaced leads causing hiccups and dysphonia from phrenic nerve stimulation,6Gasparini M. Regoli F. Ceriotti C. et al.Images in cardiovascular medicine. Hiccups and dysphonic metallic voice: a unique presentation of twiddler syndrome.Circulation. 2006; 114: e534-535Crossref PubMed Scopus (18) Google Scholar rhythmic arm twitching from brachial plexus stimulation,7Nicholson W.J. Tuohy K.A. Tilkemeier P. Twiddler’s syndrome.N Engl J Med. 2003; 348: 1726-1727Crossref PubMed Scopus (64) Google Scholar and even right-sided chest pain.8Liang J.J. Fenstad E.R. Twiddler’s syndrome.Lancet. 2013; 382: 47Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Twiddler’s syndrome is thought to occur more frequently in obese patients, who have more subcutaneous fatty tissue and thus a larger pocket in which the generator can twist.9Newland G.M. Janz T.G. Pacemaker-twiddler’s syndrome: a rare cause of lead displacement and pacemaker malfunction.Ann Emerg Med. 1994; 23: 136-138Abstract Full Text PDF PubMed Scopus (21) Google Scholar Elderly patients, who also may lack muscle in the area of implantation, may also be at higher risk. Patients with dementia or obsessive-compulsive disorder tend to twiddle more often.10Castillo R. Cavusoglu E. Twiddler’s syndrome: an interesting cause of pacemaker failure.Cardiology. 2006; 105: 119-121Crossref PubMed Scopus (22) Google Scholar, 11Jaafari N. Bachollet M.S. Paillot C. et al.Obsessive compulsive disorder in a patient with twiddler’s syndrome.Pacing Clin Electrophysiol. 2009; 32: 399-402Crossref PubMed Scopus (7) Google Scholar Twiddler’s syndrome has also been described in children and in patients with developmental delay, so it can happen to almost anyone.12Trout A.T. Larson D.B. Mangano F.T. et al.Twiddler syndrome with a twist: a cause of vagal nerve stimulator lead fracture.Pediatr Radiol. 2013; 43: 1647-1651Crossref PubMed Scopus (9) Google Scholar The evaluation of the dysfunctional pacemaker will certainly include the all-important ECG, but it also must include a chest radiograph. Evaluating the pacemaker on chest radiograph is important because it allows a complete evaluation of the physical integrity of the pacemaker or implanted cardioverter-defibrillator (ICD). When evaluating a pacemaker on chest radiograph, look at all of the elements. The 2 main components to examine are the pulse generator and the leads. These should get your full attention, and you should follow those leads through their entire course. There are 3 basic types of pacemakers. A single-chamber pacemaker will have 1 single lead that will typically go to the right atrium or right ventricle. A dual-chamber pacemaker will have 2 leads. One will go to the right atrium and one to the right ventricle. A biventricular pacemaker (also called a triple-chambered pacemaker) will typically have 1 right and 1 left ventricular lead (through the coronary sinus and the posterior cardiac vein). It may also have a right atrial lead if it is a triple-chambered pacemaker. An ICD usually has a single lead with 2 shock coils, which appear as thick metallic bands on the lead itself. The coils are typically positioned in the region of the junction of the superior vena cava and the right ventricle. Sometimes a pacemaker and ICD are combined. Usually, this is a biventricular pacemaker combined with an ICD, resulting in 3 leads: one in the right atrium, one traversing to the left ventricle, and a combined pacemaker-ICD lead in the right ventricle.13Aguilera A.L. Volokhina Y.V. Fisher K.L. Radiography of cardiac conduction devices: a comprehensive review.Radiographics. 2011; 31: 1669-1682Crossref PubMed Scopus (28) Google Scholar If the pulse generator has twirled around in its subcutaneous pocket, the leads will twist around it. This can be observed on chest radiograph as long as you are looking for it! If this has happened, chances are that the leads have retracted from their intended position or have fractured, causing problems as described above. Yes, reverse twiddler’s. What if the pulse generator rotates, but in the opposite direction? This can cause the leads to advance farther into the heart, rather than retract, from their intended positions.14Vlay S. Reverse twiddler’s syndrome.Pacing Clin Electrophysiol. 2009; 32: 146Crossref PubMed Scopus (4) Google Scholar In the case of a pacemaker with multiple leads, you can even have a combination of classic and reverse twiddler’s, with one lead retracting and the other advancing, depending on the placement and rotation. Bummer.15Ahmed F.Z. Luckie M. Goode G.K. An unusual case of combined classic and reverse twiddler’s syndrome.Can J Cardiol. 2013; 29: 1015PubMed Google Scholar With the increasing use of implantable medical devices, you can imagine that this phenomenon can easily happen to any device placed into a subcutaneous pocket. In fact, there are case reports of this happening in deep brain stimulators,16Astradsson A. Schweder P.M. Joint C. et al.Twiddler’s syndrome in a patient with a deep brain stimulation device for generalized dystonia.J Clin Neurosci. 2011; 18: 970-972Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar vagal nerve stimulators,12Trout A.T. Larson D.B. Mangano F.T. et al.Twiddler syndrome with a twist: a cause of vagal nerve stimulator lead fracture.Pediatr Radiol. 2013; 43: 1647-1651Crossref PubMed Scopus (9) Google Scholar phrenic nerve stimulators,17Fitzgerald D. Davis G.M. Gottesman R. et al.Diaphragmatic pacemaker failure in congenital central hypoventilation syndrome: a tale of two twiddlers.Pediatr Pulmonol. 1996; 22: 319-321Crossref PubMed Scopus (15) Google Scholar chemotherapy infusion ports,18Gebarski S.S. Gebarski K.S. Chemotherapy port “twiddler’s syndrome.” A need for preinjection radiography.Cancer. 1984; 54: 38-39Crossref PubMed Scopus (32) Google Scholar and intrathecal devices.19Moens M. De Smedt A. Brouns R. Opioid withdrawal due to twiddler syndrome.Neurology. 2011; 77: 86Crossref PubMed Scopus (4) Google Scholar Yowzers. Moral of the story: malfunctioning implanted devices must undergo an imaging study to evaluate the entire device and leads. This 53-year old patient, who was only 3 days status postpacemaker/defibrillator placement, presented with syncope. The pacer had originally been placed for recurrent syncope because of heart block. Could it be a malfunctioning pacemaker? Yes! The nurse also noted that the patient was frequently touching her incision. Chest radiograph was performed and demonstrated lead migration and coiling around the pulse generator. The patient underwent lead revision and was discharged without complication. At the end of the day, your mom was right when she told you not to pick at stuff. And you’re welcome for our not making a joke about Twiddler on the Roof. Woman With SyncopeAnnals of Emergency MedicineVol. 66Issue 1PreviewA 53-year-old woman 3 days status postpacemaker/defibrillator placement presented to the emergency department (ED) after a syncopal episode. The pacemaker was placed for intermittent complete heart block leading to recurrent syncope. Since discharge, she had complained of incisional pain but denied fever, erythema, or exudate from her incision. On her arrival, an ECG showed an intermittently paced regular rhythm. The patient became symptomatic with light-headedness when her pulse rate decreased to the range of 38-42 beats/min.The physical examination result was unremarkable. Full-Text PDF