Ormond Disease - Causes, Symptoms, Diagnosis, Management, and Complications

What Is Ormond Disease?

Ormond disease is also known as idiopathic retroperitoneal fibrosis and occurs rarely. The space behind the abdomen comprises numerous organs and is known as the retroperitoneal space. The perirenal space is the largest part of the retroperitoneum and comprises the kidneys, their tubules, and the adrenal glands. When this space is occupied by abnormal fiber-like tissues, the condition is known as retroperitoneal fibrosis. When the cause of the condition is unknown, and it occurs spontaneously, it is known as idiopathic retroperitoneal fibrosis. As kidneys are one of the members of the retroperitoneal space, these fibers or tissues can grow and spread to the kidneys and ureters (the tubes that carry the urine from the kidneys to the bladder). Sometimes, these tubes get blocked and restrict the flow of urine. The patient must undergo treatment at the earliest to avoid complications.

What Are the Causes of Ormond Disease?

Ormond disease or retroperitoneal fibrosis is a rare condition and has been reported to occur in only one percent of the patients. Males between 40 to 60 years are affected more compared to females. Retroperitoneal fibrosis can be primary or secondary, depending upon the causes. Primary or idiopathic retroperitoneal fibrosis, also known as Ormond disease, does not have a specific cause. However, a few causes listed below have been associated with the occurrence of Ormond disease:

1. Medications - The medications like Methysergide, Ergotamines, Methyldopa, and Hydralazine have been associated with the occurrence of Ormond disease in 12 percent of the patients.

2. Cancer - Cancer occurs when the cells present in a particular organ show uncontrolled growth and spread to the other organs. If cancer spreads to the retroperitoneal space, radiotherapy is done. Ormond disease can occur as a consequence of radiotherapy at a later stage.

3. Immunoglobulin (IgG4)- Related Disease - It is a condition in which the tumor-like lesions are formed in the various organs of the body due to an immune-mediated fibroinflammatory response. The condition usually occurs when the antibodies destroy the healthy cells of the body. However, the exact cause of the condition is still unknown.

4. Damaged Tissues - The tissues present in the pelvic region might get damaged after an injury or surgery. If these tissues are allowed to remain for long in the body, they might be the cause of Ormond disease.

5. Abdominal Aortic Aneurysm - It is a condition in which the blood vessel (aorta) that supplies the majority of the body organs becomes enlarged. The problem arises when it ruptures, causing severe bleeding from the stomach and the surrounding organs.

What Are the Signs and Symptoms of Ormond Disease?

The patient presents with several symptoms of Ormond disease or retroperitoneal fibrosis. Some of them are directly due to the disease, while some are linked to other health conditions. The signs and symptoms of Ormond disease are listed below:

1. Dull and vague pain in the abdomen increases slowly as the time passes.

2. Fever, vomiting, and nausea are commonly seen.

3. The person might lose weight as he does not feel like eating anything due to the pain.

4. Swelling of a leg occurs if the blood supply is affected.

5. Pain and discoloration of the leg due to a reduced supply of blood.

6. Pain and burning sensation while urinating are commonly seen.

7. Urinary incontinence or leakage of urine can occur in some patients.

8. The person feels thirsty all the time.

9. Hematuria, or the presence of blood in the urine, is a common finding.

10. Bleeding in the stomach and the intestines are seen at a later stage.

What Methods Are Used to Diagnose Ormond Diseases?

The diagnosis of Ormond disease is based on medical history, physical examination, laboratory, and imaging tests. Medical history and physical examination provide an idea about the general health of the patient. Laboratory and imaging tests help to confirm the diagnosis and evaluate the condition of the internal body organs. The diagnostic methods have been described below:

• Medical History - The medical history gives an idea about the symptoms of the condition, the time of their onset, if the patient has undergone any surgery in the past, drug history, and family history. Medical history provides a better understanding of the patient and his disease.

• Physical Examination - The doctor palpates the abdomen of the patient to feel the mass or the lump formed due to fibrous tissues. The blood pressure is also measured as the patients suffering from Ormond disease are usually hypertensive (show high blood pressure).

• Laboratory Tests - The following laboratory tests are recommended:

  • Blood Tests - The doctor recommends the patient undergo a blood test to measure the levels of urea, red blood cells, and C-reactive protein (CRP). The red blood cells are present in fewer numbers while the levels of urea and C-reactive protein are high. Urea is a waste product that gets eliminated from the body by the kidneys. C-reactive protein is formed by the liver in response to inflammation. C-reactive protein levels are usually high in the presence of inflammation.

  • Urine Tests - The patient collects the urine in a container or a sterile bag provided by the hospital. It is then sent to the laboratory for examination under a microscope. The urine tests help to check the urine output, the presence of blood, and bacteria in the urine.

• Imaging Tests - The following imaging tests are usually recommended:

  • Ultrasound - It is an imaging technique in which images of the abdomen and the urinary system are obtained, including the retroperitoneal space and the kidneys. Ultrasound is the most preferred method because it helps to identify the problems associated with Ormond disease. In this procedure, sound waves are sent to the body with the help of a device known as the transducer. The images are obtained on the computer screen. These images help to detect the disease and provide an idea about the condition of the kidneys.

  • Computerized Tomography (CT) Scans - In this procedure, the patient is laid on a table that slides into a tunnel-like device where the X-rays are taken. This procedure provides three-dimensional images of the internal organs, so it helps to confirm the Ormond disease. Hydronephrosis or the swelling of the kidneys can also be detected through a computerized tomography (CT) scan.

  • Magnetic Resonance Imaging (MRI) Technique - This technique uses a combination of magnetic fields and radio waves to obtain the images of the internal organs of the body. It is one of the most effective methods of diagnosing problems related to the abdomen.

  • Biopsy - It is the gold standard for diagnosing Ormond disease. A mass of tissue is taken from the retroperitoneal space to check the presence of fiber-like tissues. The presence of Ormond disease can only be confirmed after a biopsy report.

How Is Ormond Disease Managed?

The treatment of Ormond disease depends upon the severity, the degree of damage caused due to complications, and the underlying cause. The treatment options have been described below:

1. Antibiotics - Certain drugs have shown positive results in the treatment of Ormond disease. The drugs have been listed below:

   1. Glucocorticoids - Prednisolone.

   2. Immunosuppressants - Azathioprine.

   3. Estrogen Receptor Antagonists - Tamoxifen.

2. Surgery - Surgical intervention is required to free the organ that has got constricted due to fibrous tissues. It is also used to treat the problems of the urinary tract due to Ormond disease. The surgical options have been described below:

   1. Ureterolysis - It is a procedure in which the tissues that block or put pressure on the ureters are removed surgically. The ureters mainly transport the urine from the kidneys to the urinary bladder. If they are blocked, the urine flow gets restricted. In ureterolysis, the tissue compressing the ureters is removed, and they are moved away from the site of obstruction. The ureters are then stitched to the new position to allow the drainage of urine.

   2. Ureteral Stenting - Ureters become narrow when the fibrous tissues spread from the retroperitoneal space. In such a situation, a tube known as a ureteral stent is inserted into the ureters through the urethra. This tube helps to widen the ureters and facilitates the drainage of urine.

   3. Laparoscopic Surgery - In this procedure, small incisions are made in the abdomen, and a laparoscope is inserted. It is a telescope-like instrument that helps to visualize and treat the problems of the abdomen.

   4. Endourologic Procedures - Endourologic procedures are minimally invasive and do not require large incisions to be made. They are used to treat kidney stones, cancer, and blockages of the urinary tract. Small incisions are made to insert the instrument, and the area to be treated is visualized with the help of a camera. If the stones are present, they are removed directly or broken into fragments to allow them to pass through the urine.

What Are the Complications of Ormond Disease?

Retroperitoneal fibrosis or Ormond disease can block the other organs of the body and cause severe complications if left untreated for long. The complications of Ormond disease have been described below:

• Obstructive Uropathy - It is a condition in which the urine does not flow through the body as the ureters or other organs of the urinary tract are blocked. The patient might not be able to pass urine at all, resulting in anuria. It is a life-threatening condition and requires intensive medical care and attention. The fibrous tissue growth in the Ormond disease can block the ureters.

• Hydronephrosis - It is one of the most severe complications of Ormond disease. If the ureters are blocked, the urine flows back to the kidneys. As a result, the kidneys swell due to the accumulation of urine, resulting in hydronephrosis.

• Jaundice - It is a condition in which the skin and the eyes appear yellow due to the deposition of a pigment known as bilirubin. Normally, this pigment is metabolized by the liver, but jaundice occurs if the liver fails to function. The fibrous tissues get deposited in the liver and obstruct them.

• Hypertension - Hypertension or high blood pressure is one of the complications of Ormond disease. It is because the fibrous tissues formed in the retroperitoneal space obstruct the blood vessels. As a result, the blood flows under pressure, resulting in hypertension.

Conclusion:

Ormond disease or idiopathic retroperitoneal fibrosis is characterized by the presence of fibrous tissues in the space behind the abdomen. The disease occurs spontaneously as the exact cause is unknown. However, there are numerous factors associated with the occurrence of Ormond disease. The patient presents with abdominal pain, urinary tract problems, and swelling of the legs due to the obstruction of the organs caused by the fibrous tissues. Ormond disease is not a life-threatening condition but requires treatment at the earliest. Obstructive uropathy, jaundice, and hydronephrosis are some of the complications of Ormond disease. The patient need not worry as the disease is not incurable, but he must report to the doctor immediately for diagnosis and treatment.