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Pott Spine/ Pott Disease!

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 and in ancient mummies from Egypt and the Pacic coast of South America. 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 signicant cause of disease in developing nations. Tuberculous involvement of the spine has the potential to cause serious morbidity, including permanent neurologic decits and severe deformities. Medical treatment or combined medical and surgical strategies can control the disease in most patients.!

! Meaning and Denition! !

Pott's disease is spinal infection, which begins in a vertebral body and often spreads to adjacent vertebrae, with narrowing of the disk space between them. Untreated, the vertebrae may collapse, possibly impinging on the spinal cord. Symptoms include progressive or constant pain in involved bones and chronic or subacute arthritis (usually monoarticular). In Pott's disease, spinal cord compression produces neurologic decits, including paraplegia; paravertebral swelling may result from an abscess.!

Denition/Description! Potts Disease, also known as tuberculosis spondylitis, is a rare infectious disease of the spine which is typically caused by an extraspinal infection. Potts Disease is a combination of osteomyelitis and arthritis which involves multiple vertebrae.[1] The typical site of involvement is the anterior aspect of the vertebral body adjacent to the subchondral plate and occurs most frequently in the lower thoracic vertebrae. A possible effect of this disease is vertebral collapse and when this occurs anteriorly, anterior wedging results, leading to kyphotic deformity of the spine.[1] [2][3] Other possible effects can include compression fractures, spinal deformities and neurological insults, including paraplegia. [1][4]!

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Prevalence! Incidence! In 2005, there were 8.8 million new patients with tuberculosis (TB) all over the world, and of these, 7.4 million were in Asia and sub-Saharan Africa.[5] Involvement of the spine reportedly occurs in less than 1-2% of patients who contract TB. Although the incidence of tuberculosis increased in the

late 1980s to early 1990s, the total number of cases has decreased in recent years. In the United States, bone and soft tissue tuberculosis accounts for approximately 10% of extrapulmonary TB cases and between 1% and 2% of total cases. Of these cases, Potts disease is the most common manifestation of musculoskeletal TB, accounting for approximately 40-50%. Internationally, approximately 1-2% of total tuberculosis cases are attributable to Potts disease. [1]!

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Ethnicity! Data from the United States show that musculoskeletal tuberculosis primarily affects African Americans, Hispanic Americans, Asian Americans, and foreign-born individuals.[1] The number of patients with TB spondylitis in Japan also declined to 233 in 2005 from 734 in 1978 and 276 in 2001.[5]! Gender! Although some studies have found that Potts disease does not have sexual predilection, the disease is more common in males. The male to female ratio is reportedly 1.5-2:1.!

Age! In the United States and other developed countries, Potts disease occurs primarily in adults. In underdeveloped countries which have higher rates of Potts disease, involvement in young adults and older children predominates.[1]! 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.[3]! 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 decits.! 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.!

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Characteristics/Clinical Presentation! Spinal Involvement! Lower thoracic vertebrae is the most common area of involvement (40-50%), followed by the Lumbar spine (35-45%)! Approximately 10% of Pott's disease cases involve the cervical spine.[1]! The thoracic spine is involved in about 65% of cases, and the lumbar, cervical and thoracolumbar spine in about 20%, 10% and 5%, respectively! The atlanto-axial region may also be involved in less than 1% of cases[2]! Physical Findings!

Localized Tenderness! Muscle Spasms! Restricted Spinal Motion! Spinal Deformity!

Neurological Decits! Back Pain!

Back pain is the earliest and most common symptom. Patients with Potts disease usually experience back pain for weeks before seeking treatment and the pain caused by spinal TB can present as spinal or radicular. Although both the thoracic and lumbar spinal segments are nearly equally affected, the thoracic spine is frequently reported as the most common site of involvement. Together, thoracic and lumbar involvement comprise of 80-90% of spinal TB sites.[1]!

! Neurological Signs! ! !

Neurologic abnormalities occur in 50% of cases and can include spinal cord compression with the following: ! Paraplegia! Paresis! Impaired sensation! Nerve root pain! Cauda equina syndrome[1]! Spinal Deformities!

Almost all patients with Potts disease have some degree of spine deformity with thoracic kyphosis being the most common.[1]!

! Constitutional Symptoms! !
Fever! Night sweats! Weight loss ! Malaise[6][7][3][8]! Cervical Spinal TB!

Cervical spine TB is a less common presentation occurring in approximately 10% of cases, but is potentially more serious because severe neurological complications are more likely. This condition is characterized by cervical pain and stiffness and symptoms can also include torticollis, hoarseness, and neurological decits. Upper cervical spine involvement can cause rapidly progressive symptoms and neurologic manifestations occur early, ranging from a single nerve palsy to hemiparesis or quadriplegia. Retropharyngeal abscesses occur in almost all cases. In lower cervical spine insults, the patient can present with dysphagia or stridor.[1]!

! Presentation in People Infected with HIV! ! ! Asymptomatic Presentation! ! !

The clinical presentation of spinal tuberculosis in patients infected with the human immunodeciency virus (HIV) is similar to that of patients who are HIV negative; however, spinal TB seems to be more common in persons infected with HIV.[1]!

62-90% of patients with Pott's disease are reported to have no evidence of extraspinal tuberculosis, further complicating a timely diagnosis.[1]! Associated Co-morbidities! Immunosuppressive Disorders! HIV/AIDS! TB! Gastrectomy!

Peptic Ulcer! Drug Addiction! Alcoholism! Malnourishment! Low Socioeconomic Status! Medications! The duration of treatment is somewhat controversial. Although some studies favor 6 to 9 month course, traditional courses range from 9 months to longer than 1 year. The duration of therapy should be individualized and based on the resolution of active symptoms and the clinical stability of the patient.[1]!

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The main drug class consists of agents that inhibit growth and proliferation of the causative bacteria. Isoniazid and rifampin should be administered during the whole course of therapy. Additional drugs are administered during the rst two months of therapy and these are generally chosen among the rst-line drugs which include pyrazinamide, ethambutol, and streptomycin. The use of second-line drugs is indicated in cases of drug resistance.[1]!

Isoniazid (Laniazid, Nydrazid)! View full drug information: http://reference.medscape.com/drug/isoniazid-342564! Highly active against Mycobacterium tuberculosis. Has good GI absorption and penetrates well into all body uids and cavities.!

Rifampin (Rifadin, Rimactane)! View full drug information: http://reference.medscape.com/drug/rifadin-rimactane-rifampin-342570! For use in combination with at least one other antituberculous drug; inhibits DNA-dependent bacterial but not mammalian RNA polymerase. Cross-resistance may occur.!

Pyrazinamide! View full drug information: http://reference.medscape.com/drug/pyrazinamide-342678! Bactericidal against M tuberculosis in an acid environment (macrophages). Has good absorption from the GI tract and penetrates well into most tissues, including CSF.!

Ethambutol (Myambutol)! View full drug information: http://reference.medscape.com/drug/myambutol-ethambutol-342677! Has bacteriostatic activity against M tuberculosis. Has good GI absorption. CSF concentrations remain low, even in the presence of meningeal inammation.!

Streptomycin! View full drug information: http://reference.medscape.com/drug/streptomycin-342682! Bactericidal in an alkaline environment. Because it is not absorbed from the GI tract, must be administered parenterally. Exerts action mainly on extracellular tubercle bacilli. Only about 10% of the drug penetrates cells that harbor organisms. Enters the CSF only in the presence of meningeal inammation. Excretion is almost entirely renal. (3)!

! Diagnostic Tests/Lab Tests/Lab Values! !

The Mantoux Test (Tuberculin Skin Test)! Injection of a puried protein derivative (PPD). Results are positive in 84-95% of patients with Potts disease who are not infected with HIV.[1][8]!

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Erythrocyte Sedimentation Rate (ESR)! ESR may be markedly elevated (>100 mm/h)! Microbiology Studies! Microbiology studies are used to conrm diagnosis. Bone tissue or abscess samples are obtained to stain for acid-fast bacilli (AFB), and organisms are isolated for culture and susceptibility. CTguided procedures can be used to guide percutaneous sampling of affected bone or soft tissue structures; however, these study ndings are positive in only about 50% of the cases.[1]! Radiography! Radiographic changes associated with Potts disease present relatively late. The following are radiographic changes characteristics of spinal tuberculosis on plain radiography:! Lytic destruction of anterior portion of vertebral body! Increased anterior wedging! Collapse of vertebral body! Reactive sclerosis on a progressive lytic process! Enlarged psoas shadow with or without calcication! Vertebral end plates may be osteoporotic! Intervertebral disks may be shrunk or destroyed! Vertebral bodies show variable degrees of destruction! Fusiform paravertebral shadows suggest abscess formation! Bone lesions may occur at more than one level[1]! CT Scanning! CT scanning provides much better bony detail of irregular lytic lesions, sclerosis, disk collapse, and disruption of bone circumference. Low contrast resolution provides a better assessment of soft tissue, particularly in epidural and paraspinal areas. CT scanning reveals early lesions and is more effective for dening the shape and calcication of soft tissue abscesses which is common in TB lesions.[1]!

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Biopsy! Use of a percutaneous CT-guided needle biopsy of bone lesions can be used to obtain tissue samples. This is a safe procedure that also allows therapeutic drainage of large paraspinal abscesses.[1]! Polymerase Chain Reaction (PCR)! PCR techniques amplify species-specic DNA sequences which is able to rapidly detect and diagnose several strains of mycobacterium without the need for prolonged culture. They have also been used to identify discrete genetic mutations in DNA sequences associated with drug resistance.[10] ! Etiology/Causes! The four primary patterns of involvment in adults are as follows:!

! 1. Paradiscal! ! !

Most common, comprising 50% of all cases! Primary focus of infection in the vertebral metaphysis! The granuloma erodes the cartilaginous endplate and narrows the disc space! 2. Anterior Granuloma! Granulomas develop underneath the anterior longitudinal ligament! Less bony destruction but increased bone devascularization!

Further development of abscess, necrosis and deformity! 3. Central Lesions!

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Involves entire vertebral body! 2-3 vertebrae are often affected! Results in signicant deformities and pathological fractures! 4. Appendiceal Type Lesions! Lamina, pedicles, articular facets and spinous processes! Initial expansion followed by rupture and failure[8]! The organism that has been identied as causing Potts disease is mycobacterium tuberculosis. The primary mode of transmission the bacteria travels to the spine is hematogenously from an extraspinal site of infection. It is common to travel from the lungs in adults but the primary site of infection is often unknown in children.[7][11] The infection has also been found to spread through the lymphatic system.[12] Once being spread, the infection can target vertebrae, intervertebral discs, the epidural or intradural space within the spinal canal and adjacent soft tissue.[6] When the infection is developing, it can spread up and down the vertebral column, stripping the anterior and posterior longitudinal ligaments and the periosteum from the front and sides of the vertebral bodies. This results in loss of the periosteal blood supply and distraction of the anterolateral surface of the vertebrae.[3]!

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If a single vertebra is affected, the surrounding intervertebral discs will remain normal. However, if two adjacent vertebrae are affected, the intervertebral disc between them will also collapse and become avascular.[7] Due to the vascularity of intervertebral discs in children, the discs can become a primary site of infection rather than spreading from the vertebrae.[1]! Spinal cord compression in Potts disease is usually caused by paravertebral abscesses which can also develop calcications or sequestra within them.[2] If the infection reaches adjacent ligaments and soft tissues, a cold abscess can also form. Abscesses in the lumbar region may descend down the sheath of the psoas to the femoral trigone region and eventually erode into the skin.[1] Other causes of neurological involvement include dural invasion from granulation tissue, sequestrated bone, intervertebral disc collapse or a dislocated vertebra.[2][1][7] Neurological symptoms can occur at any point, including years later as a result of stretching of the spinal cord within the vertebral foramen of the deformed spine.[2]!

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Medical Management (current best evidence)! Treatment goals! Conrm Diagnosis! Eradicate Infection! Identify and Remove Causative Pathogen! Recover/Maintain Neurological Function! Recover/Maintain Mechanical Spine Stability! Correct or Prevent Spinal Deformity and Possible Sequelae ! Functional Return to Activities of Daily Living[3][8]! Treatment Techniques!

Anti-Tuberculosis Chemotherapy! Surgical Drainage of Abscess! Surgical Spinal Cord Decompression! Surgical Spinal Fusion! Spinal Immobilization! Predictors of Good Prognosis!

Partial Cord Compression!

Short Duration of Neural Complications! Early Onset Cord Involvement with Delayed Neural Complications! Young Age! Good General Condition[6]! Effective chemotherapy for Potts disease is the gold standard and must be started at the early stages of the disease.[6] Radical ventral debridement, fusion and reconstruction of the vertebral column remains the gold standard of surgical treatment for tuberculosis spondylitis.[8]!

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Multiple surgical approaches have been conducted to correct the spinal deformity seen in Pott's disease with varying results. Laminectomy failed to address the anterior component of the disease process and spinal instability. Posterior fusion has been successful at reducing kyphosis but preoperative infection and high levels of kyphosis have resulted in many fusion failures. An anterior approach, used by Hodgson and Stock, has also been used with great success.[6]! Various surgical techniques are utilized based on which area of the spine is affected. In the upper cervical spine, a transoral or extreme lateral approach is taken which typically requires concurrent occipito-cervical fusion to prevent collapse, instability and delayed deformity. Midcervical lesions are often treated with standard anterior cervical approaches and achieve excellent results. Transsternal, transmanubrial, or lateral extracavitary approaches are conducted in patients with involvement of the lower cervical/upper thoracic spine. In the thoracic spine surgeons make use of transthoracic, extraplural anterolateral or extended posterolateral approaches. The posterolateral method is more often utilized in severe cases of kyphosis due to the nature of the spinal deformity and ease of access to the spine. However, surgical correction of a severe kyphotic deformity (>30 degrees) will often require a posterior technique that is complex and technically demanding. Surgical morbidity and mortality can be signicant for these technically demanding procedures with an 8-10% incidence post correction neurological complications. Surgical procedures in the lumbar spine are typically performed through a lateral retroperitoneal approach which is the preferred method compared to an anterior or retroperitoneal procedure.[8]!

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Surgery done during the active course of the disease is much safer with a faster and better response. Moreover, the importance of early diagnosis, start of appropriate treatment and its continuation for adequate duration along with the proper counseling of the patient and family members with the timely surgical intervention is the key for the success in achieving a good outcome.[6]! Physical Therapy Management (current best evidence)! Patients with Pott's disease often undergo spinal fusion or spinal decompression surgeries to correct their structural deformity and prevent further neurological complications. There are no established guidelines which dictate treatments that will yield positive outcomes in such patients. However, treatment regimens should address each patient individually, focusing on any impairments, functional limitations and/or disabilities with which they present. !

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