Pott Disease

Background
Pott disease, also known as tuberculous spondylitis, is one of the oldest
demonstrated diseases of humankind, having been documented in spinal
remains from the Iron Age in Europe and in ancient mummies from Egypt and
the Pacific coast of South America. [1, 2] In 1779, Percivall Pott, for whom the
disease is named, presented the classic description of spinal tuberculosis.
Since the advent of antituberculous drugs and improved public health
measures, spinal tuberculosis has become rare in industrialized countries,
although it is still a significant cause of disease in developing nations.
Tuberculous involvement of the spine has the potential to cause serious
morbidity, including permanent neurologic deficits and severe deformities.
Medical treatment or combined medical and surgical strategies can control
the disease in most patients. [4, 5]

Patient education
Patients with Pott disease should be instructed on the importance of therapy
compliance

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. [6]
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.

Epidemiology
Occurrence in the United States
Although the incidence of tuberculosis increased in the late 1980s to early
1990s, the total number of cases has decreased in recent years. The
frequency of extrapulmonary tuberculosis has remained stable.
Bone and soft-tissue tuberculosis accounts for approximately 10-15% of
extrapulmonary tuberculosis cases and between 1% and 2% of total cases.
Tuberculous spondylitis is the most common manifestation of
musculoskeletal tuberculosis, accounting for approximately 40-50% of cases.
These figures are roughly similar for North American and international series.
[7, 8]
International occurrence
Approximately 1-2% of total tuberculosis cases are attributable to Pott
disease. In the Netherlands, between 1993 and 2001, tuberculosis of the
bone and joints accounted for 3.5% of all tuberculosis cases (0.2-1.1% in
patients of European origin, and 2.3-6.3% in patients of non-European
origin). [9]
Race-, sex-, and age-related demographics
Data from Los Angeles and New York show that musculoskeletal tuberculosis
affects primarily African Americans, Hispanic Americans, Asian Americans,
and foreign-born individuals.
As with other forms of tuberculosis, the frequency of Pott Disease is related
to socioeconomic factors and historical exposure to the infection.
Although some series have found that Pott disease does not have a sexual
predilection, the disease is more common in males (male-to-female ratio of
1.5-2:1).

In the United States and other developed countries, Pott disease occurs
primarily in adults. In countries with higher rates of Pott disease, involvement
in young adults and older children predominates.

Prognosis
Current treatment modalities are highly effective against Pott disease if the
disorder is not complicated by severe deformity or established neurologic
deficit.
Deformity and motor deficit are the most serious consequences of Pott
disease and continue to be a serious problem when diagnosis is delayed or
presentation of the patient is in advanced stages of the disease. [12]
Therapy compliance and drug resistance are additional factors that
significantly affect individual outcomes.
Paraplegia resulting from cord compression caused by the active disease
usually responds well to chemotherapy. However, paraplegia can manifest or
persist during healing because of permanent spinal cord damage.
Operative decompression can greatly increase the recovery rate, offering a
means of treatment when medical therapy does not bring rapid
improvement.
Careful long-term follow up is also recommended, since late-onset
complications can still occur (disease reactivation, late instability or
deformity). [13]

Morbidity
Pott disease is the most dangerous form of musculoskeletal tuberculosis
because it can cause bone destruction, deformity, and paraplegia.
Pott disease most commonly involves the thoracic and lumbosacral spine.
However, published series have shown some variation. [14, 15, 16, 17] The
lower thoracic vertebrae make up the most common area of involvement
(40-50%), followed closely by the lumbar spine (35-45%). In other series,
proportions are similar but favor lumbar spine involvement. [18]
Approximately 10% of Pott disease cases involve the cervical spine. History
The presentation of Pott disease depends on the following [19] :

Stage of disease
Affected site
Presence of complications such as neurologic deficits, abscesses, or
sinus tracts

Potential constitutional symptoms of Pott disease include fever and weight

loss. The reported average duration of symptoms at diagnosis is 4 months
[15] but can be considerably longer. [17, 20] This is due to the nonspecific
presentation of chronic back pain.
Back pain is the earliest and most common symptom of Pott disease, with
patients usually experiencing this problem for weeks before seeking
treatment. The pain caused by Pott disease can be spinal or radicular.
Neurologic abnormalities occur in 50% of cases and can include spinal cord
compression with paraplegia, paresis, impaired sensation, nerve root pain,
and/or cauda equina syndrome.
Cervical spine tuberculosis is a less common presentation but is potentially
more serious because severe neurologic complications are more likely. This
condition is characterized by pain and stiffness. Patients with lower cervical
spine disease can present with dysphagia or stridor. Symptoms can also
include torticollis, hoarseness, and neurologic deficits.
The clinical presentation of spinal tuberculosis in patients infected with the
human immunodeficiency virus (HIV) is similar to that of patients who are
HIV negative; however, spinal tuberculosis seems to be more common in
persons infected with HIV. [21]

Physical Examination
The physical examination in Pott disease should include the following:

Careful assessment of spinal alignment

Inspection of skin, with attention to detection of sinuses
Abdominal evaluation for subcutaneous flank mass
Meticulous neurologic examination

Although the thoracic and lumbar spinal segments are nearly equally
affected in persons with Pott disease, the thoracic spine is frequently
reported as the most common site of involvement. Together, these segments
make up 80-90% of spinal tuberculosis sites, with the remaining cases of Pott
disease occurring in the cervical spine. [16, 17, 19] Almost all patients with
Pott disease have some degree of spine deformity (kyphosis).
Examination should reveal local pain related to the affected area or radicular
pain. Muscle spasm and rigidity can also be associated.
Large, cold abscesses of paraspinal tissues or psoas muscle may protrude
under the inguinal ligament and may erode into the perineum or gluteal
area.

Neurologic deficits may occur early in the course of Pott disease. Signs of
such deficits depend on the level of spinal cord or nerve root compression.
Pott disease that involves the upper cervical spine can cause rapidly
progressive symptoms. Retropharyngeal abscesses occur in almost all cases
affecting this part of the spine. Neurologic manifestations occur early and
range from a single nerve palsy to hemiparesis or quadriplegia.
A large proportion of patients with Pott disease do not present with
extraskeletal disease. In reported series, only 10-38% of cases of Pott
disease are associated with extraskeletal tuberculosis.