POT Syndrome (POTS, Postural

Orthostatic Tachycardia
Syndrome)
Postural orthostatic tachycardia syndrome (POTS) is one
of a group of disorders that have orthostatic intolerance
(OI) as their primary symptom. OI describes a condition
in which an excessively reduced volume of blood returns
to the heart after an individual stands up from a lying
down
position.
The
primary
symptom
of
OI
is lightheadedness or fainting.
In
POTS,
the
lightheadedness or fainting is also accompanied by a
rapid increase in heartbeat of more than 30 beats per
minute, or a heart rate that exceeds 120 beats per
minute, within 10 minutes of rising. The faintness or
lightheadedness of POTS are relieved by lying down
again. Anyone at any age can develop POTS, but the
majority of individuals affected (between 75 and 80
percent) are women between the ages of 15 to 50 years
of age. Some women report an increase in episodes of
POTS right before their menstrual periods. POTS often
begins after a pregnancy, major surgery, trauma, or a
viral illness. It may make individuals unable
to exercise because the activity brings on fainting spells
or dizziness.

What Causes POTS?

POTS is a heterogeneous (meaning it has many causes) group of disorders with similar
clinical manifestations.1,4 POTS itself is not a disease; it is simply a cluster of symptoms
that are frequently seen together. This is why the 'S' in POTS stands for "Syndrome."
Since POTS is not a disease, it is fair to say that POTS is caused by something else.
However, figuring out what is causing the symptoms of POTS in each patient can be
very difficult, and in many cases, patients and their doctors will not be able to determine
the precise underlying cause. When doctors cannot pinpoint the underlying cause of a
patient's POTS, it may be called Primary or Idiopathic POTS. 1 Idiopathic simply means
"of an unknown origin."
While researchers are still working to identify the root causes and pathology of POTS,

there are several underlying diseases and conditions that are known to cause or be
associated with POTS or POTS like symptoms in some patients. This is a partial list:
-Amyloidosis;1
-Autoimmune Diseases such as Autoimmune Autonomic Ganglionopathy, Sjogren's
Syndrome, Lupus, Sarcoidosis, Antiphospholipid Syndrome; 1,3, 22
-Chiari Malformation19
-Deconditioning;4
-Delta Storage Pool Deficiency13
-Diabetes and pre-diabetes
-Ehlers Danlos Syndrome - a collagen protein disorder than can lead to joint
hypermobility and "stretchy" veins;3,12
-Genetic Disorders/Abnormalities;3
-Infections such as Mononucleosis, Epstein Barr Virus, Lyme Disease, extra-pulmonary
Mycoplasma pneumonia and Hepatitis C;1,2,3,4,10,11
-Multiple Sclerosis;14
-Mitochondrial Diseases;15
-Mast Cell Activation Disorders;3
-Paraneoplastic Syndrome - rare small tumors of the lung, ovary, breast and pancreas
that produce antibodies;1
-Toxicity from alcoholism, chemotherapy and heavy metal poisoning. 1
-Traumas, pregnancy or surgery;1,2,3
-Vaccinations;18
-Vitamin Deficiencies/Anemia;16,17

. Clinical manifestations:
-back pain
-body malaise
-fever and chills
-anorexia
-loss of appetite
-weight loss
-night sweating
-headache
-change in the shape of the back
-spinal mass, sometimes it goes along with numbness, tingling sensation or muscle weakness of

legs.

What is the prognosis?

POTS may follow a relapsing-remitting course, in which symptoms come and go, for
years. In most cases (approximately 80 percent), an individual with POTS improves and
becomes functional, although some residual symptoms are common.
Treatment
Each patient is different, thus consulting with a physician who has experience in treating
autonomic disorders is important. The most common treatments for POTS include
increasing fluid intake to 2-3 liters per day; increasing salt consumption to 3,000 mg to
10,000 mg per day; wearing compression stockings; raising the head of the bed (to
conserve blood volume); reclined exercises such as rowing, recumbent bicycling and
swimming; a healthy diet; avoiding substances and situations that worsen orthostatic
symptoms; and finally, the addition of medications meant to improve symptoms. 1,3 Many
different medications are used to treat POTS, such as Fludrocortisone, Beta Blockers,
Midodrine, Clonidine, Pyridostigmine, Benzodiazepines, SSRIs, SNRIs, Erythropoietin
and Octreotide.1,3 If an underlying cause of the POTS symptoms can be identified,
treating the underlying cause is very important as well.

Pathophysiology
Pott disease is usually secondary to an extraspinal source of infection. Pott disease
manifests as a combination of osteomyelitis and arthritis that usually involves more than
1 vertebra. The anterior aspect of the vertebral body adjacent to the subchondral plate
is usually affected. Tuberculosis may spread from that area to adjacent intervertebral
disks. In adults, disk disease is secondary to the spread of infection from the vertebral
body. In children, the disk, because it is vascularized, can be the primary site. [4]
Progressive bone destruction leads to vertebral collapse and kyphosis. The spinal canal
can be narrowed by abscesses, granulation tissue, or direct dural invasion, leading to
spinal cord compression and neurologic deficits.

The kyphotic deformity is caused by collapse in the anterior spine. Lesions in the
thoracic spine are more likely to lead to kyphosis than those in the lumbar spine. A cold
abscess can occur if the infection extends to adjacent ligaments and soft tissues.
Abscesses in the lumbar region may descend down the sheath of the psoas to the
femoral trigone region and eventually erode into the skin.

Medical Management:
-immobilization must be used using prolonged bed rest body cast.
-tuberbulous spondylitis of the thoraco- lumbar spine should be treated with combination chemotherapy
for 6-9 months.
-isoniazid and rifampin should be administered during the therapy is the first line anti-tuberculous
medication used in the revention and treatment of ruberculosis. It has no use except in the treatment of
mycobacterium infection.
-Rifampicin- is typically used to treat mycobacterium infection including tuberculosis and leprosy
Surgical Management:
1. Anterior decompression and spinal fusion- through a standard thorocotomy the abscess is
evacuated and debridement done. Fusion is accomplished through the use of bone parts, bone
chips taken from the iliac crest, wire of acrylic glue.
2. Costo- transversectomy- in this operation, the posterior ends of one or two ribs and the
corresponding transverse processes of the vertebrae are excised and the cold abscess evacuated.
3. Antero- lateral decompression- in this operation, in addition to costotransversectomy, the pedicles
and part of the vertebral bodies are excised to achieve decompression.
4. Spinal fusion- to reduce pain.
Postprodedure:
-restrain the patient flat on bed for 1week
-teach the patient on log rolling technique
-protect the patient from infection
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