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
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 ...
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
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 ...
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
Further reading
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External links
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Heart diseases
Syndromes