Twiddler's syndrome is a malfunction of a

pacemaker A pacemaker, also known as an artificial cardiac pacemaker, is an implanted medical device that generates electrical pulses delivered by electrodes to one or more of the chambers of the heart. Each pulse causes the targeted chamber(s) to co ...

due to manipulation of the device and the consequent dislodging of the leads from their intended location. As the leads move, they stop pacing the heart and can cause strange symptoms such as

phrenic nerve The phrenic nerve is a mixed nerve that originates from the C3–C5 spinal nerves in the neck. The nerve is important for breathing because it provides exclusive motor control of the diaphragm, the primary muscle of respiration. In humans, t ...

stimulation resulting in abdominal pulsing or

brachial plexus The brachial plexus is a network of nerves (nerve plexus) formed by the anterior rami of the lower four Spinal nerve#Cervical nerves, cervical nerves and first Spinal nerve#Thoracic nerves, thoracic nerve (cervical spinal nerve 5, C5, Cervical spi ...

stimulation resulting in rhythmic arm twitching. Twiddler's syndrome in patients with an implanted defibrilator may lead to inadequate, painful defibrillation-shocks.

Signs and symptoms

Twiddler's syndrome presents with a range of symptoms, contingent on the extent of entanglement, the electrode's subsequent retraction, and the final location of the dislodged lead. Leads that become more dislodged up can activate the ipsilateral phrenic nerves, which can result in

hiccups A hiccup (scientific name singultus, from Latin for "sob, hiccup"; also spelled hiccough) is an involuntary contraction ( myoclonic jerk) of the diaphragm that may repeat several times per minute. The hiccup is an involuntary action involvi ...

, involuntary breathing spasms, or diaphragmatic contractions. The

is stimulated by additional coiling and withdrawal of the lead, which causes regular arm twitches.

Causes

Patient "twiddling," or manipulation, of the pulse generator within its skin pocket results in the device's painless dislodgment; lead dislodgement follows, eventually leading to

malfunction. There have also been reports of variations of this phenomenon resulting in the failure of implanted cardioverter- defibrillators and

cardiac resynchronization therapy Cardiac resynchronisation therapy (CRT or CRT-P) is the insertion of electrodes in the left and right ventricles of the heart, as well as on occasion the right atrium, to treat heart failure by coordinating the function of the left and right ...

, which can be fatal.

Risk factors

The condition's risk factors include being a woman, being

obese Obesity is a medical condition, considered by multiple organizations to be a disease, in which excess body fat has accumulated to such an extent that it can potentially have negative effects on health. People are classified as obese when ...

, belonging to an older age group, having

cognitive impairments Cognition is the "mental action or process of acquiring knowledge and understanding through thought, experience, and the senses". It encompasses all aspects of intellectual functions and processes such as: perception, attention, thought, i ...

, and having an implanted device that is smaller than its pocket. A related increase in subcutaneous tissue laxity, especially in older patients, makes it easier for the device to become dislodged. Furthermore, the newer devices' smaller sizes make it easy for them to rotate inside the skin pocket.

Diagnosis

Given its speed and ability to provide a clear image of the lead coiling and device rotation, the

chest X-ray A chest radiograph, chest X-ray (CXR), or chest film is a Projectional radiography, projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common fi ...

is the most straightforward and important diagnostic tool for Twiddler's syndrome.

Treatment

Repositioning the pulse generator, implanting a new lead, and uncoiling the lead are among the treatments for diagnosed cases.

References

External links

{{Medical resources , ICD11 = {{ICD11, NE82.21 , ICD10 = {{ICD10, T82.1 , ICD10CM = {{ICD10CM, T82.198 , ICD9 = , ICDO = , OMIM = , MeshID = , DiseasesDB = , SNOMED CT = 129575004 , Curlie = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , PatientUK = , NCI = , GeneReviewsNBK = , GeneReviewsName = , NORD = , GARDNum = , GARDName = , RP = 37046 , AO = , WO = , OrthoInfo = , Orphanet = , Scholia = Q25326602 , OB = Heart diseases Syndromes