Discussion
Twiddler’s syndrome is a rare complication of cardiac implantable electronic devices resulting in device malfunction due to lead migration and dislodgement that was first described by Bayliss in 19681 Other variants of “idiopathic lead migration syndrome”3 have been described including “reel syndrome” (in which the lead is retracted along the generator, similar to a fishing reel), and “ratchet syndrome” (where the suture sleeve acts as a unidirectional “brake”).” The incidence of twiddler’s syndrome reported in the literature range from 0.07% to 2.69%.4, 5 The single most important risk factor for twiddler’s syndrome is manual manipulation of the device by the patient3 although “iatrogenic” twiddler’s syndrome has also been reported6. Advanced and younger ages, redundant skin, obesity, oversized pocket, and a history of a psychiatric disorder (especially obsessive-compulsive disorder) are considered other possible risk factors. 2, 3, 7 A left pectoral implant and silicone-insulated electrodes are also more commonly seen in patients developing twiddler’s syndrome.3
Although many cases of twiddler’s syndrome with transvenous pacemakers or ICDs have been reported, S-ICD-related lead migration has only previously been reported in one pediatric case.8Compared to TV-ICD, the S-ICD has been shown to have a lower rate of early lead displacement within 30 days of device implantation (1% vs 2.7%), and lead malfunction (0% vs. 6.2%).9Additionally, complication rates of S-ICD implants rapidly decline after the proceduralist’s first four implants and stabilize after 13 implant procedures.10
Device malfunction as a result of twiddler’s syndrome can be potentially fatal due to undetected or unsuccessful therapy for ventricular tachyarrhythmia. Routine workup for suspected cases of twiddler’s syndrome includes electrocardiogram, posterior-anterior and lateral chest X-ray, as well as device interrogation.3Replacement of a new lead and repositioning of pulse generator are often required to fully restore device function.
Studies have shown that certain surgical techniques can reduce the risk of twiddler’s syndrome in TV-ICD placement, such as anchoring sutures to pectoralis fascia, antimicrobial pouches, and woven Dacron pouches.11-13 It is also reasonable to implant the device sub-muscularly in patients with a high risk of loose subcutaneous tissue, especially patients of advanced age, and those who are obese or female. Patient education can be helpful but not completely effective, given manipulation of the device may be subconscious and in some cases device migration may be spontaneous. 3 Considering that the mechanisms for twiddler’s syndrome in TV-ICD can also be relevant for S-ICD leads, it is reasonable to infer that the aforementioned strategies would also reduce the risk of S-ICD twiddler’s syndrome.