DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS

Diffuse Idiopathic Skeletal Hyperostosis (DISH) is a kind of arthritis that primarily impacts the spine. Doctors diagnose the DISH condition when bony growths form along the spine or other damaged bones.

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Diffuse Idiopathic Skeletal Hyperostosis

Diffuse idiopathic skeletal hyperostosis (DISH) is a kind of arthritis that primarily impacts the spine. When bony growths form along the spine or other damaged bones, doctors often diagnose the DISH condition. On X-rays, bone spurs are easily seen. Furthermore, this condition is not associated with inflammation like other kinds of arthritis.

For example, it entails the hardening (calcification) of ligaments and connective tissues, most commonly in the spine. This results in “bony bridges” connecting the tiny bones of the back (called vertebrae).

This hardening of ligaments and connective tissue may also occur in the tissues surrounding the body’s other joints. For instance, the knees, elbows, and Achilles tendons. Where the tendons and ligaments join the bone, this condition causes bony growths.

DISH is not a new disease. It is confirmed throughout the archaeological record and has even been found in ancient Egyptian sites. However, many doctors are still unfamiliar with DISH because the symptoms differ from person to person. In addition, there is a lack of understanding of the disease.

The Disease Name

Diffuse Idiopathic Skeletal Hyperostosis, widely known as DISH, is a condition encompassing various medical terminologies that reflect different historical and medical insights. Most notably, Forestier’s Disease is a term often used. This name honors Dr. Jacques Forestier, the French physician who first described the disorder in 1950, initially calling it “senile ankylosing vertebral hyperostosis.”

In addition to Forestier’s Disease, some practitioners refer to the condition as Forestier’s Syndrome. This variation highlights the specific set of symptoms associated with the disease. Less frequently, it’s called Forestier-Rotes-Querol syndrome, named after both Dr. Forestier and his student Jaume Rotes-Querol, who collaborated in identifying and detailing this unique medical condition.

These labels, while interchangeable in some contexts, emphasize distinct aspects of historical and clinical significance associated with DISH. Each term provides a window into the evolving understanding and acknowledgment of contributions from key figures in the study of this disease.

QUESTIONS AND ANSWERS

What is Diffuse Idiopathic Skeletal Hyperostosis (DISH) in children, and how does it manifest in them?

Doctors consider diffuse Idiopathic Skeletal Hyperostosis (DISH) a rare condition where abnormal calcification and ossification (bone formation) of ligaments and tendons occurs, particularly along the spine and in other areas of the body. The manifestations in children can include stiffness and limited range of motion in the affected area, and in some cases, pain. The condition primarily occurs in the spine, leading to potential complications related to spinal cord compression or nerve impingement.

What are the common symptoms and signs of DISH in children?

In children, symptoms of DISH can vary but often include stiffness and reduced flexibility in the affected areas, particularly the spine. Children might experience pain, especially during movement or activities involving the affected area. In some cases, the child will experience abnormal bone growth or bony hardening along the spine or other affected ligaments and tendons.

When DISH affects the cervical spine, additional symptoms may emerge. Here’s what you might observe:

Neck Pain and Limited Mobility: Just like adults, children can suffer from neck pain and reduced neck mobility, affecting their daily activities and comfort.
Severe Complications: In some instances, osteophytes can grow extensively, compressing the esophagus or trachea. This can lead to difficulty swallowing (dysphagia) or breathing challenges due to this mechanical obstruction.
Vertebrae Fusion: The fusion of cervical vertebrae creates a long, immobile segment. This can increase the mechanical stress on the upper and lower intervertebral disc joints, accelerating degeneration that might already be present.
Degenerative Effects: The increased stress can lead to compression of the spinal cord or nerve roots, potentially causing symptoms like:
- Pain, numbness, or weakness in the arms and legs.
- Unsteady gait or difficulties with coordination.
- In severe cases, urinary incontinence.

By understanding these potential symptoms, parents and caregivers can better monitor and manage the condition, ensuring children receive the appropriate care and treatment.

How does a doctor diagnose and treat children with DISH?

“Diagnosis involves a combination of a thorough medical history, physical examination, and imaging studies such as X-rays, CT scans, or MRI to evaluate the spine and other affected areas. Treatment for DISH in children aims at managing symptoms and potentially slowing down the progression of the condition. Conservative treatments may include physical therapy, pain management, and activity modification. Regular monitoring and follow-up with a pediatric orthopedic specialist remain essential to manage the condition and adjust treatment as needed.

Imaging Techniques for DISH Diagnosis

A comprehensive diagnosis of diffuse idiopathic skeletal hyperostosis (DISH) primarily relies on imaging studies. X-rays are a crucial tool, providing good diagnostic sensitivity and specificity. They typically reveal characteristic changes in the spine, such as calcifications and bony outgrowths along the anterior longitudinal ligament. These changes are most commonly seen in the thoracic spine but can also occur in the cervical and lumbar regions. The hallmark X-ray findings, like flowing ossification along the spinal ligaments, are distinctive for DISH and help differentiate it from other spinal conditions.

However, X-rays have their limitations, particularly in visualizing certain spinal regions. This is where Computerized Tomography (CT) scans come into play. CT scans allow for much more precise visualization by providing a three-dimensional representation of the body. This enables accurate assessment of the shape, size, and location of osteophytes, surpassing the two-dimensional limitations of X-rays.

The Resnick and Niwayama criteria are traditionally used to confirm a diagnosis of DISH, requiring continuous calcification along four or more consecutive vertebrae and the absence of sacroiliac joint involvement. Recently, modifications have further refined the diagnostic process, particularly when using CT scans. These criteria are invaluable for healthcare professionals in distinguishing DISH from similar conditions that may mimic its radiographic findings.

If you want the very best in Children’s Health, make an appointment with one of our doctors at Medical City Children’s Orthopedics and Spine Specialists

Potential Causes of DISH Disease

The exact causes of diffuse idiopathic skeletal hyperostosis (DISH) remain largely enigmatic, signified by the term “idiopathic” in its name. However, several contributing factors have been suggested:

1. Genetic Influence:
Research has pinpointed several genetic elements that might play a role in the onset of DISH. Various genes, such as COL6A1 and BMP4, along with pathways like FGF2 and ALK2, have been identified as potential contributors to the disease’s development. These genetic markers suggest a hereditary component appears involved in its pathogenesis.

2. Age and Gender:
DISH predominantly affects older adults and is more frequently observed in men. A clear connection has been established between aging and the increased prevalence of DISH, with those over 70 being at greater risk. Interestingly, in some familial instances, individuals may develop the condition as early as their 30s.

Understanding these factors is crucial for better diagnosis and potential prevention strategies in the future.

How Is the Lumbar Spine Affected by DISH?

Diffuse Idiopathic Skeletal Hyperostosis (DISH) is a condition that can impact the lumbar spine, albeit less commonly than the thoracic or cervical regions. However, when DISH occurs in the lumbar spine, it manifests in specific ways.

Key Characteristics:

Ligament Changes: Similar to other spinal segments affected by DISH, the anterior longitudinal ligament in the lumbar spine undergoes calcification and ossification. This process is a trademark of the disease in this region.
Symmetric Osteophytes: Due to the lumbar spine’s proximity to the central position of the aorta, osteophytes—the bony projections that develop along the edges of bones—tend to form symmetrically in this area.
Bone Fusion: Additional bone growth can lead to the fusion of adjacent vertebrae. This fusion significantly reduces the flexibility of the lumbar spine, which is critical for its role as the body’s major load-bearing area.

Implications and Consequences:

Increased Stress: The inherent flexibility of the lumbar spine supports substantial body weight and movement. When this flexibility is compromised, the mechanical stress on the unaffected segments intensifies, accelerating their degeneration.
Visible Indicators: Radiographic indicators such as “bird beak” shaped osteophytes can often be observed, typically between lumbar vertebrae like L2 and L3.
Surgical Challenges: Patients with lumbar spine DISH may experience challenges requiring surgical interventions. However, surgeries may not always result in successful outcomes, as multiple unsuccessful operations on the lower lumbar spine highlight the complexities involved.

By understanding these effects of DISH on the lumbar spine, medical professionals can better diagnose, treat, and manage the condition, aiming to alleviate symptoms and improve the quality of life for affected individuals.

How is DISH Disease Differentiated from Similar Skeletal Conditions?

When it comes to differentiating Diffuse Idiopathic Skeletal Hyperostosis (DISH) from other skeletal ailments, understanding the unique characteristics of each condition is essential for precise diagnosis and treatment.

DISH vs. Ankylosing Spondylitis:

While both DISH and ankylosing spondylitis can lead to spontaneous spinal fusion, their underlying features are distinct:

Inflammation Absence: DISH does not involve inflammation, unlike ankylosing spondylitis, which is fundamentally inflammatory.
Calcification Patterns: In DISH, calcification occurs along the anterior longitudinal ligament without involving the sacroiliac joints (SI), although ligament calcification around these joints is frequent. In contrast, ankylosing spondylitis primarily targets the sacroiliac joints, leading to eventual spine fusion.
Spinal Deformity: The spinal deformation typical in ankylosing spondylitis, such as the bamboo spine appearance resulting from symmetric disc involvement, is generally not present in DISH.

DISH vs. Disc Degeneration:

Intervertebral disc degeneration, common with age, may appear similar to DISH, particularly due to osteophyte formation. However, they differ significantly:

Disc Space and Degeneration: DISH maintains the height of the disc space, unlike degenerative disc disease, which is marked by disc joint degeneration and narrowing.
Location of Osteophytes: Osteophytes from disc degeneration are typically found in the cervical and lumbar regions, whereas DISH often affects the thoracic spine. Moreover, osteophytes in disc degeneration are isolated to specific discs, while those in DISH cluster together.
Facet Joint Involvement: Facet joints are generally spared in DISH, as opposed to bearing additional load and hypertrophy due to degeneration.

Although DISH and degenerative spine diseases can co-occur in a patient, identifying DISH’s role as a possible trigger for lumbar degeneration is crucial. This differentiation impacts surgical outcomes and treatment decisions, emphasizing the importance of precise diagnosis.

Understanding the Impact of DISH on the Thoracic Spine

Diffuse Idiopathic Skeletal Hyperostosis (DISH) predominantly affects the thoracic spine by causing the formation of bony outgrowths, known as osteophytes, along the anterior portion of the spine. These bone spurs arise along the anterior longitudinal ligament, with the thoracic spine being particularly susceptible due to its limited mobility compared to other spinal regions.

Asymmetric Development:

Osteophytes primarily develop on the right side of the thoracic spine, especially in the lower segments (T5-T11). This asymmetry is possible because of the presence of the aorta on the left side, which might inhibit osteophyte formation there due to its pulsations.

Osteophyte Formation and Fusion:

In some parts of the thoracic spine, osteophytes may grow and eventually fuse into a single mass, forming a rigid “bone bridge.” This results in the fusion of several vertebral bodies into an immobile segment, which significantly reduces the overall motion range of that spinal segment. On X-rays or CT scans, this fusion can appear as a classic “wave-like” pattern.

In the upper thoracic area (T1-T5), osteophyte formation is less prevalent, and when it does occur, it tends to be symmetrical due to the absence of the aorta. In the thoracolumbar transition region, the aorta moves to a more central position, promoting symmetrical osteophyte growth.

Symptoms and Complications:

This condition leads to symptoms such as back pain, reduced spinal flexibility, and sometimes difficulty breathing due to the involvement of the rib cage. The spine’s reduced mobility is a major contributor to DISH-related morbidity and often goes unnoticed by patients, who may attribute it to aging.

Osteophytes might compress nearby structures like the sympathetic nerves, leading to autonomic-type pain, characterized by a burning sensation and other symptoms such as coldness or sweating. This nerve compression may cause dysfunction in internal organs, resulting in a range of symptoms including gastrointestinal issues and cardiac arrhythmia.

Long-Term Effects:

Long-term consequences include increased dynamic load on adjacent spinal segments, accelerating their degeneration. This compensatory mechanism can lead to conditions like disc herniations and spinal stenosis. Additionally, the fused rigid spine segment is more prone to fractures, even from minor impacts, akin to a long, fragile stick breaking under stress.

By understanding these specific effects of DISH on the thoracic spine, medical professionals can better diagnose and manage this condition, ultimately improving patient outcomes.

How Common is DISH?

Doctors consider DISH as the second most common form of arthritis after osteoarthritis. The exact prevalence and incidence of DISH are unknown as it is often undetected in the early stages of the disease. About 15-25% of North Americans over the age of 50 have DISH. The prevalence of DISH increases with each decade as the condition becomes better known and there are better tools to discover it. Interesting is the fact that it is diagnosed twice as often in men as in women.

Diffuse Idiopathic Skeletal Hyperostosis Warning Indicators

In the very early stages of the disease, many persons with DISH exhibit either minor symptoms or none at all. Initial DISH symptoms differ from person to person. It’s common to attribute “normal aging” to the minor symptoms that early DISH patients experience. In general, DISH patients frequently have more back stiffness and discomfort than the overall population. In the morning or after extended periods of inactivity, DISH symptoms include persistent or recurrent back discomfort and restricted range of motion. Mild exercise often helps to reduce the stiffness and back discomfort caused by DISH. As DISH worsens, the size and placement of the bony growths start to impact the surrounding tissues. However, this does not always happen.

DISH patients may have trouble swallowing due to esophageal tightness. Obstruction of the upper airway (more so in the pharynx and larynx than in the trachea) can result in aspiration (inhaling saliva into the lungs by accident), hoarseness, stridor, and sleep apnea (a sleep disorder in which breathing stops repeatedly during sleep). A higher risk of vertebral fractures may also exist in DISH patients. Due to the bony growths impacting those tendons and ligaments, individuals with DISH may also have recurrent episodes of discomfort in the area of joints in the arms and legs that feel like tendonitis. The pressure of bony growths on the spinal nerves can cause abnormal feelings in the arms or legs, as well as loss of muscular power. Despite being uncommon, a doctor should still evaluate the symptoms and check for bony growths via an X-ray.

How Quickly Can DISH Disease Progress?

DISH (Diffuse Idiopathic Skeletal Hyperostosis) disease exhibits a highly individual pace of progression. For a considerable number of individuals, the condition advances gradually. Symptoms often stabilize, remaining unchanged for extended periods. Some may find that the disease barely progresses beyond the initial onset, maintaining a consistent state.

However, there are instances where the disease incrementally deteriorates, impacting movement and daily activities. This variation may occur because of several factors, including age, overall health, and lifestyle choices.

Key Factors Affecting Progression:

Age: Older individuals may experience different progression rates compared to younger people.
Overall Health: Comorbidities can influence the disease’s trajectory.
Lifestyle: Activities and habits can play a role in either mitigating or exacerbating symptoms.

Given these variables, routine appointments with healthcare professionals are crucial. They offer a chance to track how the disease is developing and make necessary adjustments to treatment plans.

Understanding and managing expectations about the disease’s course can empower individuals to take an active role in their health care.

Diagnosing the DISH Condition

An X-ray serves as the main diagnostic tool for the DISH condition. On an X-ray, the bone growths brought on by DISH are typically easily seen to support the diagnosis. An X-ray is frequently the sole diagnostic tool required because many DISH patients exhibit no symptoms at first. If a patient has discomfort or wants a closer look at the growths, your doctor may occasionally request further imaging tests to confirm the condition and to search for other disorders that might also cause discomfort and pain. Your doctor may request a CT or MRI scan to get a better view. These examinations can reveal inflammation, deterioration, fractures, or further pain-related conditions.

Diffuse Idiopathic Skeletal Hyperostosis Risk Factors

What may raise the risk of the illness is something that doctors are aware of and consist of the following:

Sex. DISH is more prevalent in men.
Older age. DISH is more prevalent among the elderly, particularly those over 50.
Diabetes and other conditions. DISH appears more common in people with type 2 diabetes than in people without the disease.
Certain medications. Long-term usage of retinoids, which include isotretinoin (Amnesteem, Claravis, among others), a drug used to treat skin disorders including acne, can raise the risk.

What Genetic and Metabolic Factors May Contribute to DISH?

Diffuse Idiopathic Skeletal Hyperostosis (DISH) is a complex condition whose exact origins remain somewhat mysterious, given the “idiopathic” nature of its name. However, researchers have pinpointed several genetic and metabolic factors that might play a role in its development.

Genetic Influence

The genetic underpinnings of DISH have shown promise in identifying potential contributors to the condition. Specific genes have been explored, revealing possible pathways involved in DISH’s pathogenesis. These include:

COL6A1: A gene associated with collagen synthesis.
FGF2 & BMP4: Genes that regulate bone development and growth.
RSPO4 & LEMD3: Previously linked to bone and skeletal abnormalities.
ALK2: Plays a role in bone formation and remodeling.

These genetic components may interact in ways that predispose individuals to DISH, although further research is needed to solidify their exact roles.

Age and Gender

While not strictly genetic, age and gender significantly affect DISH’s occurrence. It predominantly affects older individuals, with a marked increase in cases reported among those over the age of 70. Additionally, men are more frequently diagnosed with DISH than women. Although age is a crucial factor, familial cases have shown early onset, sometimes as young as 30 years, suggesting a possible hereditary component.

By understanding these genetic markers and demographic trends, medical professionals aim to better predict and potentially mitigate the risks associated with DISH. The interplay of these factors highlights the complexity of the condition and emphasizes the need for ongoing research to uncover more definitive answers.

Complications

DISH patients run the risk of developing issues like:

Disability. Patients may not use an afflicted joint if there is a loss of range of motion. DISH, for instance, might make it challenging to use an arm.
Difficulty swallowing. Osteophytes in the neck can put pressure on the esophagus. Pressure from bone spurs can also cause hoarseness and sleep apnea. Rarely, does this become severe and require surgery to remove the osteophytes.
Spinal fracture. Osteophytes in the neck can put pressure on the esophagus. Pressure from bone spurs can also cause hoarseness and sleep apnea. Rarely, does this become severe and require surgery to remove the osteophytes.

What Emerging Treatments Are Available for DISH Disease?

Understanding of Diffuse Idiopathic Skeletal Hyperostosis (DISH) is expanding, leading to innovative treatment strategies that go beyond traditional symptom management. Patients have new options beyond the conventional focus on managing mobility and alleviating pain.

Targeted Therapies

Biologic Agents: Researchers are investigating biologic drugs that specifically target inflammation and abnormal bone growth associated with DISH. These medications aim to intervene more directly in the disease’s biological processes.
Gene Therapy: Although still in experimental stages, gene therapy offers the promise of altering the genetic landscape that contributes to the development and progression of DISH, potentially providing a long-term solution.

Surgical Innovations

Minimally Invasive Procedures: New surgical techniques are coming to the forefront, emphasizing minimally invasive methods that reduce recovery time and improve outcomes. These procedures aim to relieve pressure on the spine and restore more normal function without extensive surgery.

Advanced Pain Management

Nerve Block Injections: Emerging pain management methods include targeted nerve blocks that can alleviate discomfort in a precise and controlled manner, offering significant improvements in the quality of life for patients.

Rehabilitation and Lifestyle

Personalized Physical Therapy: Advances in rehabilitation focus on tailored physical therapy programs that address individual patient needs, enhancing mobility and overall well-being.

These emerging treatments represent a significant shift towards more personalized and effective management of DISH, providing hope and potential relief for sufferers around the globe. As research continues, expect further innovations and refinements in these areas, offering even greater promise for the future.

Non-surgical Treatments

Our doctor will work to reduce inflammation to stop any more calcification from happening. Ibuprofen (Advil, Motrin), naproxen (Aleve), and other non-steroid anti-inflammatory drugs can treat pain and lessen inflammation brought on by DISH. Additionally, treating the underlying illness can help limit the growth of DISH because of the link between DISH and endocrine disorders like diabetes. Physical therapy will help to alleviate stiffness once the inflammation has been brought under control.

Surgical Procedures/Treatments

Doctors may recommend Surgery if:

The spinal cord or nerve roots start to swell under the weight of bone spurs.
The DISH caused fractures that compressed the spinal cord or nerve roots causing pain.
DISH caused structural problems in the spine.

Resolving issues with the following solutions:

A surgery that removes a portion of the lamina, which is the bony ceiling of the spinal canal, to provide more room.
A treatment to alleviate aberrant pressure on the spinal cord by allowing the spinal cord and nerve roots more room.
A procedure where the entire or a portion of the vertebral body is removed, often to relieve pressure on the spinal cord and nerves
A treatment that involves the removal of aberrant disc material pressing against the spinal cord or a nerve root.
Spinal fusion. An operation to fuse two or more vertebrae to strengthen the spine.

Diffuse Idiopathic Skeletal Hyperostosis Treatment

To relieve stiffness, treatments typically include medication and physical therapy. Nonsurgical treatments are available and they are effective and capable of significantly reducing symptoms. The spinal cord and/or nerve roots may need an operation if DISH has compressed them. Our doctors at this point will develop a strategy for each person’s condition.

Physical Therapy

Physical therapy can loosen the stiffness brought on by DISH. Your child’s joints’ range of motion could improve with exercise. Ask your doctor about the exact workouts that are right for your child to perform. For more assistance, your doctor could suggest that you speak with a physical therapist.

Lifestyle and Home Remedies for Diffuse Idiopathic Skeletal Hyperostosis

Try these self-care techniques to assist in managing the pain and stiffness and slow the disease’s progression:

Exercise regularly. Regular cardiovascular activity, such as walking or swimming, can boost your child’s endurance, make the body more agile, and help your child deal with DISH. Before beginning a workout schedule, consult with your doctor or surgeon.
Achieve and maintain a desirable weight. Obesity is associated with DISH, so losing weight can help prevent disease progression and reduce the risk of complications.
Apply heat. Use a heating pad on the affected area of the body to relieve pain. Set the heating pad at low to reduce the risk of burns.

Diffuse Idiopathic Skeletal Hyperostosis Prevention

DISH can become quite common with age, but experts agree that most experience few if any, symptoms. Weight loss is an important preventive measure, especially as it can combat (and even prevent) problems that appear with DISH, such as diabetes and high blood pressure. Avoiding injury in patients with extensive DISH and an ankylosing (fused) spine is as easy as being careful not to fall. And watch out for back sprains and strains at all times. Use the right lifting and dragging techniques, especially for large things. Start your child’s workouts gently and with the correct safety measures (like yoga mats and the right sneakers).

What is the Prognosis for Patients with DISH Disease?

The outlook for individuals with Diffuse Idiopathic Skeletal Hyperostosis (DISH) is often positive, particularly when diagnosed at an early stage. Early detection allows for more effective management of the condition, significantly enhancing the patient’s quality of life.

However, it’s important to note that complications such as swallowing difficulties or compression of sympathetic nerves can affect this generally favorable prognosis. These complications may require additional attention and specialized care.

Key Factors Influencing Prognosis:

Timely Diagnosis: An early diagnosis is crucial in optimizing outcomes and managing the disease effectively.
Symptom Management: Regular monitoring and proactive management of symptoms play a vital role in sustaining a high quality of life.
Complications: Addressing any complications promptly is essential to prevent deterioration.

Patients are encouraged to actively participate in their healthcare plan, working closely with their healthcare provider to monitor any changes in their condition. This collaborative approach can help mitigate potential complications and maintain a stable prognosis.

Medical City Children’s Orthopedics and Spine Specialists

The Doctors, Shyam Kishan, MD, Kathryn Wiesman, MD, and Richard A. Hostin, MD, have successfully performed more than 6,000 surgeries at the Medical City Children’s Orthopedics and Spine Specialists medical practice. In doing so, they treat DISH patients with several objectives in mind. The initial step is to relieve the patient’s pain and/or any discomfort. Helping them maintain their range of motion and making an effort to regain any lost mobility is the second objective. The final objective is to stop the illness from getting worse. The Medical City Children’s Orthopedics and Spine Specialists provide the best up-to-date medical care possible.

For providing cutting-edge pediatric orthopedic care, the Medical City Children’s Orthopedics and Spine Specialists with offices in Arlington, Dallas, Flower Mound, Frisco, and McKinney, TX, has received yearly recognition. Families come from all over the world to consult with our skeletal dysplasia specialists. Even the most uncommon skeletal dysplasia, such as Diffuse Idiopathic Skeletal Hyperostosis, may be diagnosed through our genetics specialists thanks to their expertise. After determining your child’s health, our care teams collaborate to develop a specialized treatment strategy that is tailored to your child’s requirements.

Finally, our renowned pediatric orthopedic specialists will provide world-class treatments to children with DISH. Skeletal dysplasia may vary and evolve, and thus, the Medical City Children’s Orthopedics and Spine Specialists have developed one of the best pediatric imaging systems in the world to monitor and record changes in children’s bodies.

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Footnote:

Orthobullets: DISH

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