RSD Puzzle #067

H. Hooshmand, M. D.

DIPLOMATE AMERICAN BOARD OF PSYCHIATRY AND NEUROLOGY

BOARD CERTIFIED IN ELECTROENCEPHOLOGRAPHY

BOARD CERTIFIED IN ELECTROMYOGRAPHY

BOARD CERTIFIED IN AMERICAN BOARD OF ELECTODIAGNOSTIC MEDICINE

INTRACTABLE NEUROLOGY

EPILEPSY, PAIN, MS

An International Referral Center dedicated to Treatment, Education and Research

RSD PUZZLE #67
WHAT IS WRONG WITH OPERATING FOR CARPAL TUNNEL SYNDROME,
TARSAL TUNNEL SYNDROME OR THORACIC OUTLET SYNDROME
IN RSD?

RSD is considered a regional disease. The new terminology for it is Complex Regional Pain Syndrome or CRPS. The reason it is called regional pain syndrome is because it is not limited to one nerve or one finger or one toe.

The difference between the somatic system and the sympathetic system is that the sympathetic nerves follow the path of the arteries and arterioles. On the other hand, the somatic nerves and nerve roots follow specific path of the trunk of the nerves such as for example the 7th cervical (C7) nerve root, the 5th lumbar nerve root (L5), or the 1st sacral (S1) nerve root. If the patient has a circumscribed lumbosacral disc herniation, then the S1 nerve root selectively is impinged and causes a specific nerve root distribution type of pain and sensory loss which would be mainly in the posterior aspect of the leg all the way down to the 4th and 5th toes of the foot. On the other hand, when the sympathetic nerve is injured then it does not follow a specific nerve root but it follows the distribution of the blood vessel. The sensory loss and the pain are in the distribution of for example brachial artery showing a more brachial plexus type of distribution of sensory loss and pain, or the femoral artery showing more of an arterial distribution of the femoral artery and its branches. This anatomical fact causes a major confusion in regards to the diagnosis and treatment of the sympathetic nerve dysfunction. The following is a true story of a patient who had minor injury that resulted in 5 operations before the proper diagnosis was made and further surgery was prevented.

Whereas in exceptional cases such as a torn ligament in the knee-an RSD patient may need surgery under the protection of simultaneous nerve block, in other nerve involvement's at the wrist or ankle, surgery leads to devastating aggravation of RSD. The following is an example.

"T.B" was a 50 year old air condition repairman who was injured while carrying an air conditioning compressor. Accidentally, the compressor fell injuring the medial aspect of his right ankle causing burning pain and swelling over the medial aspect of the right ankle and foot. This was followed by sharp pain spreading up to the right groin. On the basis of the severe pain over the right groin region, an exploration was done in search of inguinal hernia. None was found and after the surgical procedure the patient's pain became more intense.

He started having pain and spasm involving the distribution of the femoral artery. A month later the left inguinal region over the left groin was explored in the search of hernia or impingement of any nerve. Again, no abnormality was found and the area was sutured. By this time the patient had the spread of the disease to both lower extremities in the distribution of both femoral arteries (see RSD Puzzle #18: spread of RSD).

Two months later the patient started having burning pain and swelling, inflammation, and tenderness over the palmar aspect of the right wrist. A nerve conduction time and EMG were done. The EMG was extremely painful because of the fact that the patient's hand was swollen and extremely tender with burning pain. The EMG was normal. The NCV showed no delay in nerve conduction times- regardless, the patient was diagnosed clinically as suffering from carpal tunnel syndrome. Carpal tunnel surgery was done over the right hand. The operation resulted in the development of tremor and weakness of the muscles of the right hand as well as difficulty with extending his fingers. He started developing flexion deformity of the fingers of the right hand.

He also developed radiation of the pain and inflammation all the way up to the right shoulder. He could not move his right shoulder. He had severe pain over the right shoulder. Five weeks later, because of the fact that the patient had a frozen shoulder and flexion of the right elbow and right wrist and because of the fact that he had swelling, tenderness and hypersensitivity to touch over the right shoulder, an MRI of the right shoulder was done. MRI was diagnosed as mild "rotator cuff tear". While awaiting surgery for this condition, the patient started having similar symptoms over the left hand and wrist with similar pain, tenderness and swelling of the left hand and wrist. Within one week the patient underwent carpal tunnel surgery for the left hand. After the surgery, the patient started having pain radiating to the left shoulder as well.

Because the patient had pain, tenderness and black and blue spots over the anterior aspect of the right elbow, he underwent a "supinator release surgery" over the anterior aspect of the right elbow. Needless to say, the right elbow surgery did not help the patient at all.

Since then the patient has had bouts of waking up in the morning having black and blue spots and swelling over the entire right upper extremity or entire right lower extremity for no obvious reasons. In addition, he has had tendency for neurodermatitis and swelling over the left hand of intermittent and spontaneous nature.

After the 5 operations, the patient was scheduled to have surgery for the right rotator cuff tear but because the patient was getting quite frustrated with side effects of operations he demanded another opinion. The last doctor who saw him proceeded with thermography which confirmed the diagnosis of the temperature changes involving the upper and lower extremities which were not in the distribution of any specific nerve roots but more in the distribution of blood vessels in the femoral and brachial arteries. The right upper extremity below the elbow where the patient had already had two operations, showed hyperthermia with 2 centigrade temperature higher than proximal portion of the right upper extremity. The left upper extremity and the lower extremities showed 1 to 3 centigrade temperature reduction in the distal portions of the extremities compared to the proximal portions.

The patient was diagnosed as suffering from RSD. He was given 3 epidural and 3 stellate ganglion nerve blocks on the right side which were quite painful and did not provide pain relief. By then he was advised that he needed to have rotator cuff surgical repair or sympathectomy.

Obviously the surgical procedures were canceled and the patient was treated conservatively for RSD.

The lessons to be learned from this case are the facts that the patient had injury to the right ankle yet he has had multiple operations in the areas away from the site of the injury.

The patient never had direct injury to his shoulder yet he showed rotator cuff tear. The reason for the rotator cuff tear is the fact that inflammation of RSD causes swelling and extravasation in the soft tissues of the extremity. Extravasation refers to the fact that RSD causes not only inflammation but also defective membrane function in the small blood vessels causing leakage of the blood and plasma outside the blood vessel. Eventually this swelling and inflammation becomes severe enough to cause separation of the rotator cuff tendon fibers and causes typical picture of rotator cuff tear. The same phenomenon causes the typical pictures of carpal tunnel syndrome, tarsal tunnel syndrome, tardy ulnar palsy or thoracic outlet syndrome.

Surgery in such areas of inflammation is only going to cause more trauma, more damage and more dysfunction of the sympathetic nervous system and it is going to spread the disease further into adjacent or remote regions of the extremities.

The diagram in RSD puzzle #18 shows the vertical and horizontal connections of the sympathetic ganglia around the vertebrae and explains the reason for vertical or across the midline spread of RSD. In the same RSD puzzle #18, there are 15 references that explain clearly the nature of the spread of RSD to other extremities.

The clue to RSD nerve entrapment rather than standard carpal tunnel or tarsal tunnel syndrome is the fact that the area of nerve entrapment has not undergone any kind of direct trauma. As is the case with Mr. T.B. whose history is outlined above, the original area of nerve damage is remote, and only as a regional spread such pictures of entrapment of nerves due to inflammation develop.

In RSD there are four main features. First of all the hyperpathic and allodynic pain which are typical characteristic pains of RSD manifested by elicitation of pain with simple touch or breeze (allodynia) and an out of proportion pain which spreads to the adjacent region (hyperpathia). The second feature of RSD is muscle spasm, muscle weakness, spasm in the wall of blood vessels (causing cold extremity) and tremor. The third manifestation of RSD is disturbance of immune system and secondary inflammation which becomes markedly aggravated by either inactivity or repeated operations. The fourth manifestation is disturbance of limbic system causing emotional disturbance, poor memory, poor concentration, irritability, agitation and depression. The case of Mr. T.B. manifests all of these criteria.

The answer to such horrible spread of RSD due to unnecessary operations is to avoid surgery when there has been no direct trauma at the area that is being operated on; to treat the patient with medications that reduce the edema and swelling and inflammation (such as ACTH or IV Mannitol treatment). Treatment with physical therapy and exercise will help reduce the swelling and inflammation. The use of Epsom salt and warm water also helps correct this condition.

More importantly, to enable the patient to recover and heal his own body, it is necessary to provide proper and effective pain relief with the help of treatment with Naloxon, reversible antidepressants such as Trazodone, and treatment with non-addicting strong pain medications such as Tramadol (Ultram) and Stadol.

Heat, massage, ultrasound and exercise are essential. Avoidance of especially ice (which causes further damage to the sensory nerve fibers and spreads the disease), and avoidance of the use of brace and especially casts, is essential. Dr. Cardoso and Dr. Jankovic have shown that 10 out of 11 patients who developed Parkinsonian type of tremor after application of the cast, suffered from RSD[1]. If the sympathetic nerve blocks are not effective because of the long duration of the disease and because of the fact that treatments with multiple operations has already exhausted the sympathetic system (as noted above in the case of Mr. T.B., whose right upper extremity was warm rather than cold), then instead of doing sympathetic ganglion nerve blocks the patient should receive epidural and paravertebral nerve blocks. The most important preventive measures are encouragement of activity, prevention of pain, avoidance of the use of ice and avoidance of unnecessary surgical procedures and immobilization (e.g. with cast or brace).

H. Hooshmand, M.D.

1. Cardoso F, Jankovic J: Peripherally induced tremor and parkinsonism: Arch Neurol 1995;52:263-70.

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