It results in ptosis (drooping upper eyelid), miosis (constricted pupil), and occasionally enophthalmos (the impression that the eye is sunk in) and anhidrosis (decreased sweating) on one side of the face.

In children Horner's syndrome sometimes leads to a difference in eye color between the two eyes (heterochromia). This happens because a lack of sympathetic stimulation in childhood interferes with melanin pigmentation of the melanocytes in the superficial stroma of the iris.

History

It is named after Dr Johann Friedrich Horner (1831-1886), the Swiss ophthalmologist who first described the syndrome in 1869. Several others had previously described cases, but "Horner's syndrome" is most prevalent. In France, Claude Bernard is also eponymised with the condition being called "syndrome Bernard-Horner".

Causes

Horner's syndrome is usually acquired but may also be congenital. Although most causes are relatively benign, Horner's syndrome may reflect serious pathology in the neck or chest (such as a Pancoast tumor) and hence requires workup.

Horner's Syndrome is due to a deficiency of sympathetic activity. The site of lesion to the sympathetic outflow is on the ipsilateral side that the symptoms are on. The following are examples of conditions that cause the clinical appearance of Horner's Syndrome:

First-order neuron disorder: Central lesions that involve the hypothalamospinal pathway (e.g. transection of the cervical spinal cord).
Second-order neuron disorder: Preganglionic lesions (e.g. compression of the sympathetic chain by a lung tumor).
Third-order neuron disorder: Postganglionic lesions at the level of the internal carotid artery (e.g. a tumor in the cavernous sinus).

Diagnosis

Three tests are useful in confirming the presence and severity of Horner's syndrome:

Cocaine test - Cocaine blocks the reuptake of norepinephrine resulting in the dilation of a normal pupil. The pupil will fail to dilate in Horner's syndrome.
Paredrine test
Dilation lag test

Definition

Sympathectomy is a surgical procedure that destroys nerves in the sympathetic nervous system. The procedure is done to increase blood flow and decrease long-term pain in certain diseases that cause narrowed blood vessels. It can also be used to decrease excessive sweating. This surgical procedure cuts or destroys the sympathetic ganglia, collections of nerve cell bodies in clusters along the thoracic or lumbar spinal cord.

Purpose

The autonomic nervous system that controls unwilled (involuntary) body functions, such as breathing, sweating, and blood pressure, are divided into the sympathetic and the parasympathetic nervous systems. The sympathetic nervous system speeds the heart rate, narrows (constricts) blood vessels, and raises blood pressure. Blood pressure is controlled by means of nerve cells that run through sheaths around the arteries. The sympathetic nervous system can be described as the "fight or flight" system because it allows us to respond to danger by fighting off an attacker or by running away. When danger threatens, the sympathetic nervous system increases heart and respiratory rate, increases blood flow to muscles, and decreases blood flow to other areas, such as skin, digestive tract, and limb veins. The net effect is an increase in blood pressure.

Sympathectomy is performed to relieve intermittent constricting of blood vessels (ischemia) when the fingers, toes, ears, or nose are exposed to cold (Raynaud's phenomenon). In Raynaud's phenomenon, the affected extremities turn white, then blue, and red as the blood supply is cut off. The color changes are accompanied by numbness, tingling, burning, and pain. Normal color and feeling are restored when heat is applied. The condition sometimes occurs without direct cause but it is more often caused by an underlying medical condition, such as rheumatoid arthritis. Sympathectomy is usually less effective when Raynaud's is caused by an underlying medical condition. Narrowed blood vessels in the legs that cause painful cramping (claudication) are also treated with sympathectomy.

Sympathectomy may be helpful in treating reflex sympathetic dystrophy (RSD), a condition that sometimes develops after injury. In RSD, the affected limb is painful (causalgia) and swollen. The color, temperature, and texture of the skin change. These symptoms are related to prolonged and excessive activity of the sympathetic nervous system.

Because sweating is controlled by the sympathetic nervous system, sympathectomy is also effective in treating excessive sweating (hyperhidrosis) of the palms, armpits, or face.

Precautions

To determine whether sympathectomy is needed, a reversible block of the affected nerve cell (ganglion) should be done. A reversible ganglion block interrupts nerve impulses by means of steroid and anesthetic injected into it. If the block has a positive effect on pain and blood flow in the affected area, the sympathectomy will probably be helpful. The surgical procedure should be performed only if conservative treatment has not worked. Conservative treatment includes avoiding exposure to stress and cold, physical therapy, and medications.

Sympathectomy is most likely to be effective in relieving the pain of reflex sympathetic dystrophy if it is done soon after the injury occurs. However, increased benefit from early surgery should be balanced against time needed to promote spontaneous recovery and response to conservative treatment.

Description

Sympathectomy was traditionally done as an inpatient surgical procedure under general anesthesia. An incision was made on the mid-back, exposing the ganglia to be cut. Recent techniques are less invasive and may be done under local anesthesia and as outpatient surgery. If only one arm or leg is affected, it may be treated with a percutaneous radiofrequency technique. In this technique, the surgeon locates the ganglia by a combination of x ray and electrical stimulation. The ganglia are destroyed by applying radio waves through electrodes on the skin.

Sympathectomy for hyperhidrosis can be done by making a small incision under the armpit and introducing air into the chest cavity. The surgeon inserts a fiber optic tube (endoscope) that projects an image of the operation on a video screen. The ganglia can then be cut with fine scissors attached to the endoscope. Laser beams can also be used to destroy the ganglia.

Preparation

As with any surgery, patients should discuss expected results and possible risks with their surgeons. They should tell their surgeons all medications they are taking and all their medical problems, and they should be in good general health. To improve general health, the patient may be asked to lose weight, give up smoking or alcohol, and get the proper sleep, diet, and exercise. Immediately before the surgery, patients will not be permitted to eat or drink, and the surgical site will be cleaned and scrubbed.

Aftercare

The surgeon will inform the patient about specific aftercare needed for the technique used. Doppler ultrasonography, a test using sound waves to measure blood flow, can help to determine whether sympathectomy has had a positive result.

Risks

Side effects of sympathectomy may include decreased blood pressure while standing, which may cause fainting spells. After sympathectomy in men, semen is sometimes ejaculated into the bladder, which may impair fertility. After a sympathectomy done by inserting an endoscope in the chest cavity, patients may experience chest pain with deep breathing. This problem usually disappears within two weeks. They may also experience pneumothorax (air in the chest cavity).

In 30% of cases, surgery for hyperhidrosis may cause increased sweating on the chest. In 2% of cases, this surgery causes increased sweating in other areas, including increased facial sweating while eating. Other complications occur less frequently. These complications include Horner's syndrome, a condition of the nervous system that causes the pupil of the eye to close, the eyelid to droop, and sweating to decrease on one side of the face. Other rare complications are nasal blockage and pain of the nerves supplying the skin between the ribs.

Normal results

Some studies report that sympathectomy relieves causalgia in as many as 75% of cases. The studies also show that it relieves hyperhidrosis in more than 90% of cases. The less invasive procedures cause very little scarring. Most patients stay in hospital for less than one day and return to work within the week.

Key Terms

Causalgia: A severe burning sensation sometimes accompanied by redness and inflammation of the skin. Causalgia is caused by injury to a nerve outside the spinal cord.
Claudication: Cramping or pain in a leg caused by poor blood circulation. This condition is frequently caused by hardening of the arteries (atherosclerosis). Intermittent claudication occurs only at certain times, usually after exercise, and is relieved by rest.
Fiberoptics: In medicine, fiberoptics uses glass or plastic fibers to transmit light through a specially designed tube. The tube is inserted into organs or body cavities where it transmits a magnified image of the internal body structures.
Hyperhidrosis: Excessive sweating. Hyperhidrosis can be caused by heat, overactive thyroid glands, strong emotion, menopause, or infection.
Parasympathetic nervous system: The division of the autonomic (involuntary or unwilled) nervous system that slows heart rate, increases digestive and gland activity, and relaxes the sphincter muscles that close off body organs.
Percutaneous: Performed through the skin, from the Latin , meaning through and , meaning skin.
Pneumothorax: A collection of air or gas in the chest cavity that causes a lung to collapse. Pneumothorax may be caused by an open chest wound that admits air.

For Your Information

Periodicals

Lai, Y. T., et al. "Complications in Patients with Palmar Hyperhidrosis Treated with Transthoracic Endoscopic Sympathectomy." Neurosurgery 41 (1997): 110-113.
Lee, K.H, and P. Y. Hwang. "Video Endoscopic Sympathectomy for Palmar Hyperhidrosis." Journal of Neurosurgery 84 (1996): 484-486.
Schwartzman, R. J., et al. "Long-Term Outcome Following Sympathectomy for Complex Regional Pain Syndrome Type 1 (RSD)." Journal of the Neurological Sciences 150 (1997): 149-152.
Wilkinson, H. A. "Percutaneous Radiofrequency Upper Thoracic Sympathectomy." Neurosurgery 38 (1996): 715-725.
Other
The Center for Hyperhidrosis, Inc. http:/www.handsweat.com

Gale Encyclopedia of Medicine. Gale Research, 1999.

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