A Rare Case of Twiddler’s Syndrome in a Child with Epicardial Pacemaker Leads

Objectives: Twiddler’s syndrome is a rare complication after pacemaker implantation referring to pacemaker malfunction due to patient’s manipulation of the pulse generator resulting in coiling of the pacemaker leads with consequent lead dislodging and failure. It is a recognized condition in adults; however, in children it has only been occasionally described.

Methods: We present a case of Twiddler’s syndrome in a child with a pacemaker system with epicardial leads.

Result: A 7.5-year-old girl with Down’s syndrome was incidentally diagnosed with Twiddler’s syndrome during routine follow-up examination. She had a history of surgical correction of a complete atrioventricular septal defect at the age of 2.5 months with subsequent complete heart block and implantation of a dual-chamber pacemaker generator with bipolar epicardial atrial and ventricular leads (Medtronic Sensia SEDRO1, CapsureEPI 4968–25 cm; Medtronic, Meerbusch, Germany). The current lead parameters were good and had not changed since the previous interrogation. However, in the routine chest X-ray performed to screen for lead shortening due to patients’ growth, a 3.5-time axial rotation of the pacemaker generator located in an epigastric pocket was documented, having resulted in significant lead coiling. Due to safety reasons, revision of the leads was performed and surprisingly, a fracture of the ventricular lead insulation was noted, leaving approximately 4 mm of the lead noninsulated. After uncoiling of the leads, the insulation defect was repaired and the generator was exchanged since remaining estimated lifespan was only 22 months. To prevent recurring manipulation, it was positioned in a newly formed subrectus pocket and cautiously fixated with sutures.

Conclusion: Our presented case is remarkable for several reasons. First, reported cases in children are exceptionally rare with only a handful of published cases. Second, to our knowledge, this is the first report of Twiddler’s syndrome in a pacemaker system with epicardial leads. Also, most cases are reported to occur early within the first year after pacemaker implantation; however, as demonstrated by our case, it may also occur several years after implantation. Third, our case emphasizes the importance of pacemaker revision since lead defects may be present despite normal lead parameters. Unnoticed lead damages may result in acute and in pacemaker dependent patients potentially lethal malfunction.