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.