Usuario:Churubusco3737/Taller-VertebralDestructionSyndrome

Vertebral Destruction Syndrome

The vertebral destruction syndrome, described by Dr. Armando Alpizar Aguirre and his team in 2007, encompasses various pathologies that cause alterations in the structure of the vertebral column, resulting in deformities and neurological or mechanical impairments. This description arises from the need to have an efficient diagnostic strategy to achieve early and timely diagnosis of this pathology due to its diverse etiology. Among its causes are infectious, tumoral, and metabolic disorders.​The diagnosis, often inconclusive, follows a process that includes a topographic, syndromic, etiological, and differential diagnosis. This approach aims to systematize the management of these pathologies, allowing for faster and more precise treatment. Although they share characteristics, each disease has its own peculiarities, necessitating the use of specific imaging studies for each case. An accurate diagnosis is essential for improving the patient's prognosis and treatment.​

Etiology

Syndrome with multiple pathogenesis, due to three main causes that produce modifications in the structure of the spine, and defects in volume of the vertebral bodies.​

Among the most common causes of vertebral destruction are infectious ones, caused by pathogens such as bacteria (Staphylococcus aureus, Pseudomonas aeruginosa, Brucella), mycobacteria (Mycobacterium tuberculosis), and more recently, a relationship has been detected with fungal group (Candida and Aspergillus), with S. aureus being the most prevalent, reaching up to 50-60%.​

It has tumor etiology derived from lesions or metastases in the spine, the most common being multiple myeloma, plasmacytoma, osteoblastoma, osteoid osteoma, lymphoma, Ewing's sarcoma, hemangioma, giant cell tumor and neuroblastoma, producing dissemination by lymphatic or hematogenous route, even other types of neoplasm that are most frequently dispersed to the spine are lung,  breast, prostate, and kidney. ​

And it has a metabolic etiology derived from endocrine conditions such as osteoporosis, osteomalacia, Paget's disease and a less frequent but one that has been noticed in some patients, chronic kidney failure.​

Physiopathology

The destruction of the vertebral bodies due to the different causes, has its own biochemical and structural changes, leading to the loss of the shape of the disc due to a decrease in the synthesis of proteoglycans that gives a loss of water from the extracellular matrix in the nucleus pulposus (acquiring a fibrous consistency), giving an increase in the degradation of proteins that help the nutrition of the vertebrae (and can lead to apoptosis and cell death), and an alteration in collagen, which is the most abundant protein within the disc matrix (acquiring a thin shape).​

Infectious

Vertebral destruction by pathogens does not only encompass the underlying tissue, but involves the vertebral body, causing an inflammatory process that leads to an increase in volume.​

Dissemination occurs through three main routes, hematogenous, direct inoculation and contiguity dispersion within tissues.

• Hematogenous is the most common route, being different in adult and child populations, causing disc vascularization in children without any bone involvement, and in adults the spread occurs around the vertebral plate without any involvement of the disc or neighboring vertebrae. Various theories mention that the hematogenous pathway has two foci, one venous (related to the return of the pelvic and perivertebral venous plexuses) and an arterial one (arterial connections that are closest to the vertebral plate).

• The direct inoculation route is given more by techniques carried out for diagnosis, such as lumbar puncture or some type of infiltration.

• The route of dispersion due to contiguity to the tissues is the least frequent, but it can occur due to pathologies such as esophageal rupture, retropharyngeal abscesses or sacral eschar. ​

Tumoral

It is caused by metastases and primary tumors of the spine, or its vicinity, such as neural tissue, ribs, or paravertebral musculature.​

This occurs mostly by hematogenous dissemination, although it can also be done by the lymphatic route, it begins with the invasion of the arterial wall at the level of the tumor, causing some neoplastic cells to enter the bloodstream, when they enter they adhere, and are predisposed to cross the blood-brain barrier (covered by fenestrated endothelium and occlusion junctions that cover the cerebral microvasculature).  which has the function of limiting the passage of any harmful cell or macromolecule.

By conferring a breach of this barrier, it is impossible not to allow many microorganisms, drugs and neoplastic cells to enter. The breakdown of the barrier and the entry of cells occurs by two pathways, one transcellular, and one paracellular (by the destruction of endothelial junctions and expression of inflammatory mediators and proteases). ​

Once inside the microvasculature, they extravasate to vertebral tissues and bodies, taking into account that these can proliferate to nearby organs and tissues.​

Metabolic

Metabolic bone diseases that occur as a result of changes in calcium metabolism damage the spine due to a decrease in bone mass, and modifications in the microarchitecture of bone tissue, causing the cancellous bone to resist axial loads in isolation during posture or movement. The imbalance is mainly between bone resorption and bone formation. An example of this is osteoporosis.​

On the other hand, osteomalacia is due to a primary mineral deficiency of the bone matrix that makes the bone "soft" and can cause its deformation. Osteomalacia differs from osteoporosis, in which bone mass is deficient but bone mineralization is normal, and can have similar clinical manifestations. However, bone quality and serological data will differ significantly. ​

Finally, Paget's disease is a formative pathology of an excessive amount of bone content, however this will be of insufficient quality than necessary, causing symptoms due to compression of the dural sac and the nerve elements that accompany it.​

Clinical Features

As vertebral destruction syndrome is considered a condition of multiple etiologies, patients typically exhibit an insidious evolution, with common delays between symptom onset. The most frequent symptoms include pain and motor and sensory deficits, specific to the affected spinal segment.​​

Given its diverse pathologies, manifestations may include:

Infectious

In cases of bacterial infection, clinical presentation occurs in 90% of cases with cervical and/or dorsolumbar pain, with Staphylococcus aureus responsible for nearly 60% of spinal infections. Fungal infections may present with subacute pain.​

Tumoral

General symptoms are the initial clues for suspecting vertebral destruction syndrome secondary to malignant tumors. Lumbalgia is present in approximately 90% of patients, while systemic symptoms, such as weight loss, fatigue, and fever, should also be considered.​

Metabolic:

Clinical presentation is associated with loss of bone mass, decreased height, persistent dorsal or lumbar pain exacerbated by standing or intrathoracic pressure, and reduced during rest. ​

Diagnosis

Involvement of the vertebral column, encompassing various conditions leading to both mechanical and neurological instability in the back, necessitates an extensive array of diagnostic tests, ranging from laboratory analyses such as electrophoresis and immunological tests to imaging studies including X-rays, scintigraphy, computed tomography, and magnetic resonance imaging. While these procedures are essential for accurate evaluation, they can be prolonged and costly.​

During the initial assessment in the emergency department, a thorough screening is conducted to identify symptoms such as pain, fever, night sweats, weight loss, and consumption of unpasteurized dairy products that may suggest the presence of Brucella melitensis. Clinical presentation can be confusing; for instance, in a case where a patient was initially treated for lymphoma due to similar symptoms and radiological findings, but was ultimately diagnosed with vertebral osteomyelitis caused by Bartonella henselae.​

Treatment

To treat vertebral destruction syndrome, it's necessary to conduct a thorough medical history and pain assessment to identify the syndrome's etiology, whether it be infectious, metabolic, or tumoral.

Furthermore, imaging studies should be performed to locate the affected vertebrae, assess vertebral body fragmentation, identify disc invasion, and determine tumor location (if tumor is the etiology). Additionally, clinical studies should be conducted, including complete blood count, Western Blot, PCR, procalcitonin, Rose Bengal, Bence-Jones protein, biopsy, and bone densitometry.​

INFECTIOUS ETIOLOGY

It is important to think of vertebral osteomyelitis. If this is the case, it will depend on the access that the patient has, if he has the opportunity to perform surgery, it will be performed and treated with drugs, if surgery is not available, only pharmacological treatment will be used. If the blood culture is negative, a percutaneous vertebral biopsy will be performed.​

The criteria for surgery are:

• Neurological deficit​
• Persistent pain​
• Blood cultures, serology, and percutaneous biopsy were negative​
• Deformity in kyphosis of the cervical, thoracic, or thoracolumbar spine​
• Instability​

Brucellosis

• Blood culture positive for Brucella.​

Tuberculosis

• Staining or PCR positive for Mycobacterium Tuberculosis.
• Elevated globular sedimentation rate (SGV), suspected of tuberculosis.
• Be treated with anti-tuberculosis chemotherapy (QTAT).
• Initiate specific antibiotic therapy.

METABOLIC ETIOLOGY:

Both osteoporosis, osteomalacia, and Paget's disease are included in this syndrome, but osteoporosis is the most common.​

Osteoporosis is characterized by lower bone density, in addition to microarchitectural bone deterioration. They are associated with osteoporosis-related fracture risk: low bone mineral density, prior fractures due to frailty, advanced age, and a family history of osteoporosis.​

The first-line pharmacological treatment is:

• Bisphosphonates (alendronate, risedronate), raloxifene and calcitonin.
• Surgery should be performed when there is instability in the spine.

TUMOUR ETIOLOGY:

Some of the most common tumour etiologies affecting the spine are multiple myeloma, plasmacytoma, and metastases from the lung, prostate, breast, and kidney, which account for approximately 95% of all tumour lesions in the spine. It is important to note that chemotherapy has limited use in cases of spinal metastases due to its variable effectiveness and possible side effects.​

Neurological deficit and spinal instability are critical criteria for determining the need for surgery, as they indicate the urgency of intervention to prevent further damage.​

Therefore, the combination of radiation therapy with tumor resection surgery has established itself as one of the most effective therapeutic approaches today, with high success rates. This treatment seeks not only to eliminate the tumor, but also to preserve neurological function and stabilize the spine, which contributes significantly to improving patients' long-term quality of life.

Prognosis

Neurological recovery at the site of injury is fundamentally based on the regenerative capacity of partially damaged neurons and their potential to establish new synaptic connections.

In contrast, underlying neurologic dysfunction at the level of the lesion usually indicates a poor prognosis. However, the prospects for recovery fluctuate depending on the initial degree of involvement, categorized according to the American Spinal Injury Association (ASIA) scale.

In the case of lesions, studies indicate that approximately 31% of patients initially classified as ASIA grade B progress to grade D. This rate improves significantly in patients with grade C lesions, where about 65% achieve this improvement. During the acute post-injury phase, the most critical complications include pressure ulcers, particularly in the sacral area, this being the predominant complication.

The respiratory complications such as pneumonia and atelectasis represent the second most common cause of complications, affecting 13% of cases, while deep vein thrombosis occurs in 10%, with 3% of patients experiencing pulmonary thromboembolism.

The life expectancy for a 20-year-old patient diagnosed with "vertebral destruction syndrome" is approximately 33 years, increasing to 44 years in the absence of any of the aforementioned respiratory complications.​