Potts Disease

Dexter Jay B. Flores

BSN 3 UNEP

Potts disease
This entity was first described by Percivall Pott. He noted this as a painful kyphotic deformity of the spine associated with paraplegia. Tuberculosis of the spine is one of the oldest diseases afflicting humans. Evidences of spinal tuberculosis have been found in Egyptian mummies dating back to 3400 BC

 One fifth of TB population is in India. Three percent are suffering from skeletal TB, 50% of these suffer from spinal lesion and almost 50% are from pediatric group. An estimated 2 million or more patients have active spinal tuberculosis. Every day 1000 die of tuberculosis in India.

Regional Distribution
1 2 3 4 5 6 Cervical cervicodorsal Dorsal Dorsolumbar Lumbar Lumbosacral 12% 5% 42% 12% 26% 3%

Pathophysiology
Pott disease is usually secondary to an extraspinal source of infection. The basic lesion is a combination of osteomyelitis and arthritis. The area usually affected is the anterior aspect of the vertebral body adjacent to the subchondral plate. 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. This leads to spinal cord compression and neurologic deficits. Kyphotic deformity occurs as a consequence of collapse in the anterior spine. Lesions in the thoracic spine have a greater tendency for kyphosis than those in the lumbar spine. The collapse is minimal in cervical spine because most of the body weight is borne through the articular processes. Healing takes place by gradual fibrosis and calcification of the granulmatous tuberculous tissue. Eventually the fibrous tissue is ossified, with resulting bony ankylosis of the collapsed vertebrae.

 Paravertebral abscess formation occurs in almost every case. With collapse of the vertebral body, tuberculous granulation tissue, caseous matter, and necrotic bone and bone marrow are extruded through the bony cortex and accumulate beneath the anterior longitudinal ligament. These cold abscesses gravitate along the fascial planes and present externally at some distance from the site of the original lesion. In the lumbar region the abscess gravitates along the psoas fascial sheath and usually points into the groin just below the inguinal ligament. In the thoracic region, the longitudinal ligaments limit the abscess, which is seen in the radiogram as a fusiform radiopaque shadow at or just below the level of the involved vertebra. Thoracic abscess may reach the anterior chest wall in the parasternal area by tracking via the intercostal vessels.

The lesion could be:

Florid - invasive and destructive lesion. Non destructive - lesion suspected clinically but identifiable by modern investigations like CT scan or M.R.I. Encysted disease Carries sicca Hypertrophied Periosteal lesion.

 Recently, two distinct patterns of spinal TB can be identified, the classic form, called spondylodiscitis (SPD) a atypical form characterized by spondylitis without disk involvement (SPwD). SPwD seems to be the most common pattern of spinal TB.

Anatomically the lesion could be

1. Paradiscal - destruction of adjacent end plates and diminution of disc space. 2. Appendeceal (Posterior) involvement of pedicles, laminae, spinous process. 3. Central - Cystic or lytic, concertina collapse. 4. Anterior longitudinal lig, Aneurysmal phenomenon 5. Synovitis in post facet

History
Presentation depends on the following:
Stage of disease Site Presence of complications such as neurologic deficits, abscesses, or sinus tracts

The reported average duration of symptoms at the time of diagnosis is 3-4 months. Back pain is the earliest and most common symptom.
Patients have usually had back pain for weeks prior to presentation. Pain can be spinal or radicular.

Constitutional symptoms 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, or cauda equina syndrome. Cervical spine tuberculosis is a less common presentation 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, the relative proportion of individuals who are HIV positive seems to be higher.

Natural course of disease

53% died within 10 yrs of onset Early stage of healing focus surrounded by sclerotic bone Ivory vertebra Early radiological sign of healing sharpening of fuzzy paradiscal margins & reappearance and minrralization of tuberculae Several vertebrae destroyed fibrous tissue Disc space destroyed bony ankylosis/bone block formation

Lab Studies
Tuberculin skin test (purified protein derivative [PPD]) demonstrates a positive finding in 84-95% of patients who are nonHIV-positive. Erythrocyte sedimentation rate (ESR) may be markedly elevated . The enzyme-linked immunosorbent assay (ELISA) has a reported sensitivity of 60 to 80 per cent The polymerase chain reaction A brucella complement fixation test

 IFN- Release Assays (IGRAs) Recently, two in vitro assays that measure T cell release of IFN- in response to stimulation with the highly tuberculosisspecific antigens ESAT-6 and CFP-10 have become commercially available.

 Microbiology studies to confirm diagnosis: Obtain bone tissue or abscess samples to stain for acid-fast bacilli (AFB), and isolate organisms for culture and susceptibility. CT-guided procedures can be used to guide percutaneous sampling of affected bone or soft tissue structures. These study findings may be positive in only about 50% of the cases.

X Ray appearances
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 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.

X Ray appearances
Discovertebral lesions, detected in 93% of patients, Localized fluffy osseous destruction with surrounding osteoporosis is the earliest signs. concentric collapse and may look like A.V.N. Local lytic lesion may cause problem of diagnosis from neoplasic lesion. destruction of adjacent vertebrae, Konstram (K) angle appears and shows the progress on follow up. Skipped lesion (10% cases) can be diagnosed on suspicion and in correct size film.

X-ray of the thoracolumbar spine (Lateral view) showing wedge collapse of L1 and L2 vertebral bodies.

X-ray of the spine in a child showing complete destruction of D12 and L1 vertebral bodies leaving only the pedicles.

Kumars clinico-radiological Classification

stage PreI II
destructive Earlydestructive Mild kyphos Moderate kyphos Severe kyphos

features Straightening, spasm, hyperemia in scinti Diminished space paradiscal erosion Knuckle <10 2-3 verte k:10-30 >3 verte K:30-60 >3 verte K:>60

Usual duration

<3 mo 2-4 mo

III IV V

3-9 mo 6-24 mo >2 years

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. It detects early lesions and is more effective for defining the shape and calcification of soft tissue abscesses. In contrast to pyogenic disease, calcification is common in tuberculous lesions.

MRI
MRI is the criterion standard for evaluating disk space infection and osteomyelitis of the spine MRI findings useful to differentiate tuberculous spondylitis from pyogenic spondylitis include thin and smooth enhancement of the abscess wall and well-defined paraspinal abnormal signal, whereas thick and irregular enhancement of abscess wall and ill-defined paraspinal abnormal signal are suggestive of pyogenic spondylitis. contrast-enhanced MRI appears to be important in the differentiation of these two types of spondylitis. most effective for demonstrating neural compression.

Myelography
Spinal tumor syndrome Multiple vertebral lesions Patients not recovered after decompression 1. Block present : second decompression 2. Block not present : intrinsic damage 1.Ischemic infarction 2.Interstitial gliosis 3.atrophy 4. tuberculous myelitis 5.Myelomalacia

1. Pyogenic infections 2. Typhoid spine 3. Brucella Spondylitis 4. Mycotic Spondylitis 5. Syphilitic 6. Tumorous condition 7. Primary malignant tumor 8. Multiple Myeloma 9. Lymphomas 10.Secondary 11.Histocytosis-X 12.Spinal Osteochondrosis 13.Spondylolisthesis 14.Hydatid disease

Differentials

Complications of tuberculosis
1. 2. 3. 4. 5. 6. Paraplegia Cold abscess Sinuses Secondary infection Amyloid disease Fatality

Tb spine with PARAPLEGIA

INCIDENCE 10-30% Dorsal spine (MC) Motor functions affected before /greater than sensory Sense of position & vibration last to disappear

Patho of Tuberculoses Paraplegia

1. Inflammatory Edema vascular stasis,toxin 2. Extradural Mass Tuberculous ostetis+ abscess 3. Bony Disorder Sequestra, Internal Gibbus 4. Meningeal changes dura as rule not involved Extradural grnulation -- contract, cicatrization peridural fibrosis paraplegia

5. Infarction of spinal cord

- Ant spinal artery Endarteritis,Periarteritis,Thrombosis 6. Changes in Spinal cordMyelomalacic,Syringomyelic change,Atrophy upto 50%dec in dia-good functions

Seddons Classification:

GROUP A_-Early onset - This comes up in active stage of the disease within first 2 years. Compressive Agents are inflammatory edema, granulation, abscess, casseous material, sequestra and rarely ischaemic lesion. GROUP B -Late onset- Usually after 2 years of onset of the disease.
due to recurrence or by mechanical pressure. This can be better divided into paraplegia with active disease and with healed disease.

Active disease - Caseous material, debris, sequestrated disc or bone, internal gibbus, stenosis and deformity can cause compression. Healed disease - Usually internal gibbus and acute kyphotic deformity can also give late onset paraplegia. Usually there is a continuous traction, compression leading to paraplegia.

Kumars classification of tuberculous paraplegia

stage

1 2 3

Negligible

Clinical features Unaware of neural deficit, Plantar extensor/ Ankle clonus Walk with support

Mild

Moderate Nonambulatory,

Paralysis in extention,sensory loss <50% 4 Severe 3+ paralysis in flexion/sensory loss>50%/ Sphinters involved

Evolution of treatment
Pre-antitubercular era Artificial abscess- Pott in 1779 Laminectomy & laminotomy : chipault(1896 ) Costo-transversectomy: Menard in 1896 Posterior mediastinotomy Calves operation 1917 Lateral rhachiotomy of carpener 1933 Anterlateral decompression of Dott& Alexander:1947

Early diagnosis Expeditious medical treatment Aggressive surgical approach Prevent deformity Expect good outcome

BASIC PRINCIPLES OF MANAGEMENT

 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 a 1994 recommendation by the US Centers for Disease Control and Prevention, this is the treatment of choice.

What is Middle path regime?

Admission rest in bed Chemotherapy X-ray & ESR once in 3 months MRI/ CT at 6 months interval for 2 years Craniovertebral ,cervicodorsal, lumbosacral& sacroiliac joints Gradual mobilization 3-9 weeks- back extention exercise 5-10 min 3-4 times Spinal brace--- 18 months-2 years

Abcesses aspirate near surface Instille 1gm Streptomycin +/- INH in sol Sinus heals 6-12 weeks Neural complications if responds 3-4 weeks :surgery unnecssary Excisional surgery for posterior spinal disease Operative debridement for patients if no arrest after 3-6 months- spinal arthrodesis (recommended) Post op--Spinal brace--- 18 months-2 years

Drugs in middle path

phase duration drug INH Rifampicin 300ofloxacin400-600mg / 400mg streptomycin -do 3-4mth Pyrazinamide 1500mg 4-5mth Rifampicin Ethambutol 1200mg Intensive 5-6 months Continua 7-8 tion months Prophyla 4-5 ctic months

-do

Surgical indications
1. No sign of Neurological recovery after trial of 3-4 weeks therapy 2. Neurological complication during treatment 3. Neuro deficit becoming worse 4. Recurrence of neuro complication 5. Prevertebral cervical abscesses,neurological signs& difficulty in deglutition& respiration 6. Advanced casesSphincter involvement, flaccid paralysis, Severe flexor spasms

Other indications
Recurrent paraplegia Painful paraplegia d/t root compression,etc Posterior spinal disease--involving the post elements of vertb Spinal tumor syndrome resulting in cord compression Rapid onset paraplegia due to thrombosis,trauma etc Severe paraplegia Secondary to cervical disease and cauda equina paralysis

1 2

Decompression +/- fusion Debridement+/fusion Debridement +/DECOMP+/fusion Debridement+/fusion Anterior transpostion Laminectomy

Failed response,Too advanced Failed response after 3-6 months,Doubtful diagnosis,Instability Recrudescence of disease

4 5 6

Prevent severe Kyphosis Severe Kyphosis +neural deficit STS,secondary stenosis, posterior disease

APPROACH
1. Cervical spine Anterior retropharyngeal (smith-Robinsons) Anterior approach Anterior/Medial border of sternocleidomastoid 2. Dorsal spine (D1 to L1) 1 Transthoraccic transpleural 2 Anterolateral decompression(D2 L1) 3. Lumbar spine Anterolateral(Lumbovertebrotomy) Extraperitoneal Ant. approach

Tulis recommended approch

Cervical spine T1 Anterior approch Dorsal spine DL junction Antrolateral approch Lumbar spine &Lumboscral junction Extraperitoneal Transverse Vertebrotomy

Surgical technique
Costotransversectomy in tense paravertebral abscess remove transverse process rib 2 inchs

Anterolateral decompression
Posterior part of rib Transverse process Pedicle Part of the vertebral body Griffith Seddon Roaf -- prone position

Tuli --right lateral position Advantage:- 1 avoid venous congestion 2 avoid excessive bleeding 3 permits freer respiration 4 better look at site

Posterior Spinal Arthrodesis

By Albee & Hibbs Albee tibial graft inserted longitudinally in to the split spinous vertebral process Hibbs overlapping numerous small osseous flap from contiguous laminae spinous process & articular facets Indications 1 mechanical instability 2 to stabilise craniovertebral region 3 as part of panvertebral operation

DYNAMIC CAGE :Govender&Prabhoo

SYNTHES PLATE WITH SAPCER

Yilmaz C, Selek HY, et al. Anterior instrumentation for the Treatment of Spinal Tuberculosis. J Bone and Joint Surg 1999; 81-A : 1261-67

we feel that every attempt should be made to minimize this deformity with some form of instrumentation wherever indicated, and preferably anteriorly.

Thank

you Thank you