POTTS DISEASE Pott's disease is a presentation of extrapulmonary tuberculosis that affects the spine, a kind of tuberculous arthritis of the

intervertebral joints. It is named after Percivall Pott (17141788), a London surgeon who trained at St Bartholomew's Hospital, London. The lower thoracic and upper lumbar vertebrae are the areas of the spine most often affected. Scientifically, it is called tuberculous spondylitis and it is most commonly localized in the thoracic portion of the spine. Potts disease results from haematogenous spread of tuberculosis from other sites, often pulmonary. The infection then spreads from two adjacent vertebrae into the adjoining intervertebral disc space. If only one vertebra is affected, the disc is normal, but if two are involved, the disc, which is avascular, cannot receive nutrients and collapses. The disc tissue dies and is broken down by caseation, leading to vertebral narrowing and eventually to vertebral collapse and spinal damage. A dry soft tissue mass often forms and superinfection is rare. 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 Peru. In 1779, Percivall Pott, for whom Pott 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 developed countries, although it is still a significant cause of disease in developing countries. 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. Cultural references The fictional Hunchback of Notre Dame had a gibbous deformity (humpback) that is thought to have been caused by tuberculosis. In Henrik Ibsen's play "A Doll's House," Dr. Rank suffers from "consumption of the spine." Furthermore, Jocelin, the Dean who wanted a spire on his cathedral in William Golding's "The Spire" probably suffered and died as a result of this disease. English poets Alexander Pope and William Ernest Henley both suffered from Pott's disease. Anna Roosevelt Cowles, sister of president Theodore Roosevelt, suffered from Pott's Disease. Chick Webb, swing era drummer and band leader, was afflicted with tuberculosis of the spine as a child, which left him hunchbacked. The Sicilian mafia boss Luciano Leggio had Pott's disease and wore a brace. Morton, the railroad magnate in Once Upon a Time in the West, suffers from the disease and needs crutches to walk. Writer Max Blecher also had Pott's Disease. Marxist thinker and Communist leader Antonio Gramsci suffered from Pott's disease, probably due to the bad conditions of his incarceration in fascist Italy during the 1930s. Italian writer, poet and phylosopher Giacomo Leopardi suffered too of this disease. CAUSES Pott's disease, which is also known as Potts caries, David's disease, and Pott's curvature, is a medical condition of the spine. Individuals suffering from Pott's disease typically experience back pain, night sweats, fever, weight loss, and anorexia. They may also develop a spinal mass,

which results in tingling, numbness, or a general feeling of weakness in the leg muscles. Often, the pain associated with Pott's disease causes the sufferer to walk in an upright and stiff position. Potts disease is caused when the vertebrae become soft and collapse as the result of caries or osteitis. Typically, this is caused by mycobacterium tuberculosis. As a result, a person with Pott's disease often develops kyphosis, which results in a hunchback. This is often referred to as Potts curvature. In some cases, a person with Pott's disease may also develop paralysis, referred to as Potts paraplegia, when the spinal nerves become affected by the curvature. A person with Pott's disease may experience additional complications as a result of the curvature. For example, an infection can more easily spread from the paravertebral tissue, which can cause abscesses to occur. Regardless of the complications that may occur, Pott's disease is typically slow spreading and can last for several months or years. INCIDENCE Epidemiology Frequency United States
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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% 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.

International 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). Mortality/Morbidity
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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 show some variation. Lower thoracic vertebrae is 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. Approximately 10% of Pott disease cases involve the cervical spine.

Race
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Data from Los Angeles and New York show that musculoskeletal tuberculosis primarily affects 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.

Sex 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). Age
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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

RISK FACTORS Tuberculosis remains one of the most life-threatening infections and it is believed that up to onethird of the worlds population has latent TB. A large proportion of these people will never experience an active infection but the risk increases substantially with certain diseases and living conditions. Risk factors for tuberculosis includes :
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Weakened immune system HIV/AIDS, poorly controlled diabetes mellitus, end-stage renal failure, malnutrition, old age, immune suppressing drugs in the treatment of cancer and autoimmune diseases, medication to prevent transplant rejection. Substance abuse including alcoholism, prescription drugs and illicit substances. IV drug users have a greater risk. Poverty which may contribute to poor nutrition, a lack of sanitation, living in crowded settings and a lack of adequate medical care. Health care facilities and nursing homes workers, long stay patients and residents of these facilities are at a greater risk of contracting TB.

These risk factors apply to tuberculosis as a whole and pulmonary tuberculosis in particular. The specific risk factors for secondary infections like Potts disease is having pulmonary TB in HIV/AIDS patients, not seeking medical treatment or discontinuing drug therapy prematurely.

CLINICAL PRESENTATION History

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The presentation of Pott disease depends on the following: o Stage of disease o Affected site o Presence of complications such as neurologic deficits, abscesses, or sinus tracts The reported average duration of symptoms at diagnosis is 4 months but can be considerably longer, even in most recent series. This is due to the nonspecific presentation of chronic back pain. Back pain is the earliest and most common symptom. o Patients with Pott disease usually experience back pain for weeks before seeking treatment. o The pain caused by Pott disease can be spinal or radicular. Potential constitutional symptoms of Pott disease include fever and weight loss. 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. o This condition is characterized by pain and stiffness. o Patients with lower cervical spine disease can present with dysphagia or stridor. o 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.

Physical
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The examination should include the following: o Careful assessment of spinal alignment o Inspection of skin, with attention to detection of sinuses o Abdominal evaluation for subcutaneous flank mass o Meticulous neurologic examination Although both 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, they comprise 80-90% of spinal tuberculosis sites. The remaining cases correspond to the cervical spine. Almost all patients with Pott disease have some degree of spine deformity (kyphosis). 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.

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Retropharyngeal abscesses occur in almost all cases. Neurologic manifestations occur early and range from a single nerve palsy to hemiparesis or quadriplegia. Many persons with Pott disease (62-90% of patients in reported series) have no evidence of extraspinal tuberculosis, further complicating a timely diagnosis. Information from imaging studies, microbiology, and anatomic pathology should help establish the diagnosis.
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Signs and symptoms

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back pain fever night sweating anorexia Spinal mass, sometimes associated with numbness, paraesthesia, or muscle weakness of the legs

ANATOMY and PHYSIOLOGY

The vertebral column provides structural support for the trunk and surrounds and protects the spinal cord. The vertebral column also provides attachment points for the muscles of the back and ribs. The vertebral disks serve as shock absorbers during activities such as walking, running, and jumping. They also allow the spine to flex and extend The spinal cord is the largest nerve in the body, and it is comprised of the nerves which act as the communicat ion system for the body. The nerve fibers within the spinal cord carry messages to and from the brain to other parts of the bo d y. T he s p i n a l c o r d is s u r r o u nd e d b y p r o t e ct i ve bo ne s e g me nt s , c a l l e d t he vertebral column. The vertebral column is comprised of seven cervical vertebrae, twelve thoracic vertebrae, five lumbar vertebrae and five sacral vertebrae. The vertebral column also provides attachment points for muscles of the back and r i bs . T he ve r t e br a l d i s k s s e r v e a s s h o c k a b s o r be r s d ur i ng a c t i v it ie s s u c h a s walking, running and jumping, they also allow the spine to flex and extend. PATHOPHYSIOLOGY Pott disease is usually secondary to an extraspinal source of infection. The basic lesion involved in Pott disease is a combination of osteomyelitis and arthritis that usually involves more than one vertebra. The anterior aspect of the vertebral body adjacent to the subchondral plate is area 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, because the disk is vascularized, it can be a primary site. 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. Diagnostic and Laboratory Tests y y y y y y y blood tests elevated erythrocyte sedimentation rate tuberculin skin test radiographs of the spine bone scan CT of the spine bone biopsy MRI

Imaging Studies
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Radiography o Radiographic changes associated with Pott disease present relatively late. The following are radiographic changes characteristic 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 calcification o Additional radiographic findings may include the following:  Vertebral end plates are 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. CT scanning o CT scanning provides much better bony detail of irregular lytic lesions, sclerosis, disk collapse, and disruption of bone circumference. o Low-contrast resolution provides a better assessment of soft tissue, particularly in epidural and paraspinal areas. o CT scanning reveals early lesions and is more effective for defining the shape and calcification of soft-tissue abscesses. o In contrast to pyogenic disease, calcification is common in tuberculous lesions. MRI o MRI is the criterion standard for evaluating disk-space infection and osteomyelitis of the spine and is most effective for demonstrating the extension of disease into soft tissues and the spread of tuberculous debris under the anterior and posterior longitudinal ligaments. MRI is also the most effective imaging study for demonstrating neural compression.

MRI findings useful to differentiate tuberculous spondylitis from pyogenic spondylitis include thin and smooth enhancement of the abscess wall and welldefined paraspinal abnormal signal, whereas thick and irregular enhancement of abscess wall and ill-defined paraspinal abnormal signal suggest pyogenic spondylitis. Thus, contrast-enhanced MRI appears to be important in the differentiation of these two types of spondylitis. o The images below are studies of a 31-year-old man with tuberculosis of the spine.
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MRI of a 31-year-old man with tuberculosis of the spine. Images show the thoracic spine before and after an infusion of intravenous gadolinium contrast. The abscess and subsequent destruction of the T11-T12 disc interspace is marked with arrowheads. Vertebral body alignment is normal. Courtesy of Mark C. Diamond, MD, and J. Antonio Bouffard, MD, Detroit, Mich.

MRI of the T11 in a 31-year-old man with tuberculosis of the spine. Extensive bone destruction consistent with tuberculous osteomyelitis is evident. The spinal cord has normal caliber and signal. No evidence of spinal cord compression or significant spinal stenosis is distinguishable. Laboratory Studies
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Tuberculin skin test (purified protein derivative [PPD]) results are positive in 84-95% of patients with Pott disease who are not infected with HIV. The erythrocyte sedimentation rate (ESR) may be markedly elevated (>100 mm/h). Microbiology studies are used to confirm diagnosis. Bone tissue or abscess samples are obtained to stain for acid-fast bacilli (AFB), and organisms are isolated for culture and susceptibility. CT-guided procedures can be used to guide percutaneous sampling of affected bone or soft-tissue structures. These study findings are positive in only about 50% of the cases.

Other Tests
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Radionuclide scanning findings are not specific for Pott disease. Gallium and Tc-bone scans yield high false-negative rates (70% and up to 35%, respectively)

Procedures
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Use a percutaneous CT-guided needle biopsy of bone lesions to obtain tissue samples. o This is a safe procedure that also allows therapeutic drainage of large paraspinal abscesses. o Obtain a tissue sample for microbiology and pathology studies to confirm diagnosis and to isolate organisms for culture and susceptibility. Some cases of Pott disease are diagnosed following an open drainage procedure (eg, following presentation with acute neurologic deterioration).

Histologic Findings Because microbiologic studies may be nondiagnostic of Pott disease, anatomic pathology can be significant. Gross pathologic findings include exudative granulation tissue with interspersed abscesses. Coalescence of abscesses results in areas of caseating necrosis. MANAGEMENT Prevention Controlling the spread of tuberculosis infection can prevent tuberculous spondylitis and arthritis. Patients who have a positive PPD test (but not active tuberculosis) may decrease their risk by properly taking medicines to prevent tuberculosis. To effectively treat tuberculosis, it is crucial that patients take their medications exactly as prescribed. Therapy
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non-operative antituberculous drugs Chiropractic treatments analgesics immobilization of the spine region by rod (Hull) Surgery may be necessary, especially to drain spinal abscesses or to stabilize the spine Richards intramedullary hip screw facilitating for bone healing Kuntcher Nail intramedullary rod Austin Moore intrameduallary rod (for Hemiarthroplasty) Thoracic spinal fusion as a last resort

Medical Care

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Before the advent of effective antituberculosis chemotherapy, Pott disease was treated with immobilization using prolonged bed rest or a body cast. At the time, Pott disease carried a mortality rate of 20%, and relapse was common (30%). The duration of treatment, surgical indications, and inpatient care have since evolved. Studies performed by the British Medical Research Council indicate that tuberculous spondylitis of the thoracolumbar spine should be treated with combination chemotherapy for 6-9 months. According to the most recent recommendations issued in 2003 by the US Centers for Disease Control and Prevention, the Infectious Diseases Society of America, and the American Thoracic Society, a 4-drug regimen should be used empirically to treat Pott disease. Isoniazid and rifampin should be administered during the whole course of therapy. Additional drugs are administered during the first 2 months of therapy. These are generally chosen among the first-line drugs, which include pyrazinamide, ethambutol, and streptomycin. The use of second-line drugs is indicated in cases of drug resistance. Regarding the duration of therapy, the British Medical Research Council studies did not include patients with multiple vertebral involvement, cervical lesions, or major neurologic involvement. Because of these limitations, many experts still recommend chemotherapy for 9-12 months. Opinions differ regarding whether the treatment of choice should be conservative chemotherapy or a combination of chemotherapy and surgery. The treatment decision should be individualized for each patient. Routine surgery does not to seem to be indicated. Most common indications for surgical procedures are discussed below.

Surgical Care
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Indications for surgical treatment of Pott disease generally include the following: o Neurologic deficit (acute neurologic deterioration, paraparesis, paraplegia) o Spinal deformity with instability or pain o No response to medical therapy (continuing progression of kyphosis or instability) o Large paraspinal abscess o Nondiagnostic percutaneous needle biopsy sample Resources and experience are key factors in the decision to use a surgical approach. The lesion site, extent of vertebral destruction, and presence of cord compression or spinal deformity determine the specific operative approach (kyphosis, paraplegia, tuberculous abscess). Vertebral damage is considered significant if more than 50% of the vertebral body is collapsed or destroyed or a spinal deformity of more than 5 exists. The most conventional approaches include anterior radical focal debridement and posterior stabilization with instrumentation. In Pott disease that involves the cervical spine, the following factors justify early surgical intervention: o High frequency and severity of neurologic deficits o Severe abscess compression that may induce dysphagia or asphyxia o Instability of the cervical spine

Contraindications: Vertebral collapse of a lesser magnitude is not considered an indication for surgery because, with appropriate treatment and therapy compliance, it is less likely to progress to a severe deformity.

Activity
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Despite questionable efficacy, prolonged recumbence and the use of frames, plaster beds, plaster jackets, and braces are still used. Cast or brace immobilization was a traditional form of treatment but has generally been discarded. Patients with Pott disease should be treated with external bracing.

Medication Summary
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A 4-drug regimen should be used empirically to treat Pott disease. Treatment can be adjusted when susceptibility information becomes available. Isoniazid and rifampin should be administered during the whole course of therapy. Additional drugs are administered during the first 2 months of therapy. These are generally chosen among the first-line drugs, which include pyrazinamide, ethambutol, and streptomycin. A 3-drug regimen usually includes isoniazid, rifampin, and pyrazinamide. The use of second-line drugs is indicated in cases of drug resistance. The duration of treatment is somewhat controversial. Although some studies favor a 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.

Patient Education
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Patients with Pott disease should be instructed on the importance of therapy compliance. For excellent patient education resources, visit eMedicine's Bacterial and Viral Infections Center. Also, see eMedicine's patient education article Tuberculosis.

Prognosis
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Current treatment modalities are highly effective if not complicated by severe deformity or established neurologic deficit. Therapy compliance and drug resistance are additional factors that significantly affect individual outcomes. Paraplegia resulting from the active disease causing cord compression usually responds well to chemotherapy. If medical therapy does not result in rapid improvement, operative decompression will greatly increase the recovery rate. Paraplegia can manifest or persist during healing because of permanent spinal cord damage.

Late complications
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Vertebral collapse resulting in kyphosis Spinal cord compression sinus formation paraplegia (so called Pott's paraplegia)

REFERENCES: http://www.healthhype.com/bone-tuberculosis-and-back-tb-potts-disease.html http://en.wikipedia.org/wiki/Pott_disease http://emedicine.medscape.com/article/226141-overview http://www.slideshare.net/prammy4u/pott-disease-presentation http://www.wisegeek.com/what-causes-potts-disease.htm

CASE STUDY

POTTS DISEASE

Presented by:

Kniazeff, Divine A. Loro, Ailyn J.

Presented to:

Mr. Joey Servan, RN, MSN Clinical Preceptor