Spinal Epidural Lipomatosis

Spinal Epidural Lipomatosis

Article
Brain & Nerve
Diseases & Conditions
+2
Contributed byMaulik P. Purohit MD MPHJul 18, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • SEL (Spinal Epidural Lipomatosis)
  • Steroid-Induced SEL
  • Steroid-Induced Spinal Epidural Lipomatosis

What is Spinal Epidural Lipomatosis? (Definition/Background Information)

  • Lipomatosis is a benign condition of fatty tissue overgrowth, when abnormal levels of fat are spread over a wide area in the body with an uneven distribution pattern. The fatty tissues can even infiltrate the underlying or adjacent muscle layers
  • Spinal Epidural Lipomatosis (SEL) is a rare benign disorder that occurs against a background of abnormal hormone levels or steroids in the body. It causes spinal cord compression in the lumbar or thoracic region, with accompanying neurological signs and symptoms
  • The steroids may be externally administered to treat an underlying disorder, or the body produces abnormal hormones due to an adverse health condition. However, not all cases of Spinal Epidural Lipomatosis have been recognized as being caused by a body hormonal imbalance (these are termed idiopathic SELs)
  • Generally, middle-aged and older adults are observed with Spinal Epidural Lipomatosis. The signs and symptoms may include back pain, numbness, tingling sensation in the affected region, and weakness in the lower extremities
  • Both conservative and surgical treatment measures may be considered to address Spinal Epidural Lipomatosis based upon the severity of the condition and the extent of spinal stenosis (narrowing of the spine due to compression)
  • The prognosis of Spinal Epidural Lipomatosis depends upon many factors including the severity of the signs and symptoms, the underlying cause of the condition, and response to treatment

Who gets Spinal Epidural Lipomatosis? (Age and Sex Distribution)

  • Spinal Epidural Lipomatosis is seen in both children (as young as 6 years) and adults; the average age of presentation of SEL is over 40 years
  • Both males and females are affected, though some studies show a higher prevalence of SEL in males
  • Generally, there is no known geographical, racial, or ethnic preference observed for the condition

What are the Risk Factors for Spinal Epidural Lipomatosis? (Predisposing Factors)

The following risk factors have been identified for Spinal Epidural Lipomatosis:

  • Steroid therapy:
    • Individuals on long-term hormone or steroid therapy for various health conditions (such as systemic lupus erythematosus); the duration may be between 5-11 years
    • In general, the higher the dosage and longer the duration of steroid medication administration, the greater may be the risk
    • However, individuals on short-term (few months) steroidal hormone therapy and with lower drug dosage are also at risk
    • Oral administration of steroids is noted to frequently play a role in the development of SEL; though, inhaled or injected drugs can also cause the condition
  • Individuals with increased adrenocortical steroid production, such as due to Cushing’s disease
  • Hypothyroidism
  • Pituitary prolactinoma: A tumor of the pituitary gland that produces excess amounts of hormone called prolactin
  • Morbid obesity
  • In some cases, no risk factor for SEL is apparent or identified

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Spinal Epidural Lipomatosis? (Etiology)

The exact cause and mechanism of Spinal Epidural Lipomatosis formation is unknown. However, the condition is associated with the following:

  • Chronic steroid therapy that may be administered for various illnesses is known to be the largest contributory factor (in as many as 3 out of 4 cases, according to a study)
  • Increased production of hormones in the body resulting in hormonal imbalances. This occurs due to conditions including:
    • Cushing syndrome and Cushing disease
    • Obesity
    • Hypothyroidism
    • Pituitary prolactinoma
  • In many cases, steroidal use or the involvement of hormonal imbalance is not evidently noted

What are the Signs and Symptoms of Spinal Epidural Lipomatosis?

Individuals with Spinal Epidural Lipomatosis have excessive, but non-uniform fat deposition in the epidural space (the space that lies outermost within the spinal canal), causing compression of the spinal cord. Commonly, the thoracic region (in about 60% of the cases) or lumbar region (in about 40% of the cases) is involved.

This gives rise to the associated neurological signs and symptoms, depending on the specific region along the spine that is affected.

  • Pain (especially back pain) and numbness
  • Weakness in the legs
  • Sensation of pinpricks or tingling sensation
  • Altered reflexes
  • Damage to the spinal cord that can be progressive
  • In rare cases, the function of the urinary bladder or bowel is affected
  • Additionally, the signs and symptoms of the underlying condition may be seen

How is Spinal Epidural Lipomatosis Diagnosed?

A diagnosis of Spinal Epidural Lipomatosis is made using the following tools:

  • Complete physical examination with thorough evaluation of the individual’s medical history (including history of steroidal medication use)
  • Endocrinological evaluation
  • X-ray of the affected portion of the spine may help exclude other causes of the condition
  • Myelography; a type of radiological exam using a contrast to detect cord compression
  • CT and MRI scan of the affected region: It can show the extent of fat accumulation in the body
  • Nerve conduction studies, if the nervous system is affected and neurological symptoms are noted
  • Additional tests with respect to the underlying condition or disorder may be required
  • Tissue biopsy of the fatty tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Note: A differential diagnosis to eliminate encapsulated spinal lipoma is necessary, since many of the signs and symptoms of the condition closely resemble that of SEL.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Spinal Epidural Lipomatosis?

The complications from Spinal Epidural Lipomatosis could include:

  • Overall quality of life is affected; emotional stress
  • Permanent injury to the spinal roots
  • Severe disability, in some cases
  • Severe neurogenic atrophy (wasting away of the muscles due to injury to nerves)
  • Loss of bladder/bowel function (incontinence)
  • Surgical complications: Damage to the nerve during decompression surgery and also to remove the tumor
  • Recurrence after surgery at the same location

How is Spinal Epidural Lipomatosis Treated?

The treatment measures for Spinal Epidural Lipomatosis may include the following:

  • Steroid lipomatosis can be treated through suitable medication or by lowering steroid dose levels in the body, which may reduce the buildup of fat in the epidural space. However, this treatment option also depends on the reason for initially administering steroid therapy (vis-à-vis the underlying condition)
  • In case of obesity, weight loss is recommended and is found to be an effective treatment tool
  • Addressing the cause of overproduction of corticosteroids in the body
  • Surgical excision with removal of the abnormal fatty tumor can be attempted through a decompressive laminectomy. This intervention tool can help relieve pressure on the spinal cord, improving the symptoms. It is found to be very effective in individuals without an identified SEL cause (idiopathic and not linked to steroids), but is only performed when severe symptoms are noted
  • Physical therapy exercises to strengthen and improve flexibility of the affected spinal cord region may help reduce pain and discomfort
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important and encouraged

How can Spinal Epidural Lipomatosis be Prevented?

Current medical research has not established a method of preventing Spinal Epidural Lipomatosis. However, some of the risk factors for the condition may be reduced or addressed:

  • Thoroughly evaluating the health and medical history of an individual prior to administering any hormonal therapy
  • Individuals who are overweight or obese can bring about lifestyle and dietary modifications and undertake regular exercising to reduce their weight
  • Undertaking appropriate and early treatment of any underlying disorder or hormonal condition

What is the Prognosis of Spinal Epidural Lipomatosis? (Outcomes/Resolutions)

  • The prognosis of Spinal Epidural Lipomatosis (benign tumor) depends upon several factors including:
    • The underlying cause of the condition
    • Severity of the signs and symptoms
    • Overall health of the individual
    • Response to treatment
  • Spinal Epidural Lipomatosis may be treated through conservative techniques for optimum results, if the signs and symptoms are mild or moderate. However, the treatment effectiveness is dependent upon the severity of the underlying (hormonal) condition/disorder
  • Studies have shown that surgical intervention techniques, for severe cord compression, may be beneficial in individuals with idiopathic SEL. Sometimes, multiple surgeries may be necessary to remove fat that has accumulated again at the same location
  • However, a surgery has to be weighed against complications from the invasive procedure, long-term medical issues, and the presence of comorbidities. Moreover, studies have not indicated that a rapid resolution of SEL is observed with decompressive laminectomy surgery
  • Long-term vigilance and follow-up is recommended

Additional and Relevant Useful Information for Spinal Epidural Lipomatosis:

  • Lipomatosis of Nerve is an infrequent benign tumor occurring on the surface of a nerve due to the growth of fibrous and fatty tissue

The following link will help you understand lipomatosis of nerve:

http://www.dovemed.com/diseases-conditions/lipomatosis-nerve/

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Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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