Part 7

Miss M. E., a German, aged forty-three, was sent to me on June 14, 1899. For two years sight had been failing, until at this time vision was as follows: R. E. = 0; L. E = light perception. Tension was + 3. Both optic nerves showed marked cupping of the disk; the vessels were pushed to the nasal side. She stated that she had never had pain in the eyes, and had not consulted an ophthalmic surgeon.

I advised her to submit to an excision of the left superior cervical ganglion; she consented, and on June 15 the operation was performed by myself, assisted by Dr. E. C. Renaud, at St. Joseph’s Sanatorium, in the presence of Drs. J. C. Murphy, A. R. Kieffer, and S. A. Grantham. The operation was difficult, owing to the abnormal position of the vagus nerve. This was outside of and external to the carotid sheath, and was much smaller than normal; it was not larger in diameter than the head of a pin. It was identified by irritating it and watching the effect on the heart. The superior cervical ganglion was removed and one-half inch of the trunk of the sympathetic below. Shortly after the operation there were lachrymation, ocular congestion, and contraction of the pupil on the corresponding side. On the second day she counted fingers at 2½, and on the third at 3½ feet. Slight ptosis was present.

She left the hospital on the eighth day. At this time she counted fingers at four feet. There was only slight, if any, reduction of tension during the eight days she was in the hospital. In counting fingers she saw with the nasal side of the retina—temporal field. I did not see her again until June 30, and she was then counting fingers at five feet. Tension on that day was normal. She had light perception in the right eye.

On July 16 I excised the right superior cervical ganglion without difficulty, and on July 7 she counted fingers at seven feet with the left eye, and could see the hand at four inches with the right. I examined her on July 20, when vision remained the same, the tension of the right eye was + 1, and of the left + 2. She was well pleased to have the small amount of vision she possessed.

CASE III.—SYMPATHECTOMY FOR OPTIC-NERVE ATROPHY.

T. J., aged forty-six, an inmate of the St. Louis City Hospital, a laborer, was admitted on account of blindness. There was no history of syphilis, rheumatism, nor any systemic disease. The patient was of limited mentality. No history of his family could be obtained. He claimed to have had good health all his life, with the exception of an attack of malarial fever several years ago. The patient had been a moderate drinker of alcoholic beverages. In appearance he was robust, and he complained only of loss of vision which, in the left eye, had been failing for eleven months, in the right for seventeen weeks, according to his statement. Until seventeen weeks before this he could see enough with the right eye to get around. Since then vision had steadily declined until he had light perception only—and this only apparent when light was concentrated on the eye by the ophthalmoscopic mirror. Vision of the left eye = 0.

The pupils were widely dilated. The ophthalmoscope showed, in the right eye, a white disk, particularly on the temporal side; the arteries slightly reduced in caliber, veins normal. There was shallow, atrophic cupping of the nerve head. The retina and choroid were normal, the vitreous and lens clear. The left eye showed a disk of a dead white color throughout the whole area, arteries very small, atrophic excavation pronounced, veins reduced in caliber, and choroid normal. The macula was not visible in this eye, owing to the much-reduced blood-supply. The vitreous and lens were clear. Vision was as follows: R. E. = perception of concentrated light. L. E. = 0.

_Diagnosis._—R. E. = optic-nerve atrophy. L. E. = complete atrophy of optic nerve and retina.

_Treatment_: Resection of the right superior cervical ganglion of the sympathetic was done. The operation was followed by conjunctival congestion, lachrymation and contraction of the pupil, slight ptosis and hypotonia.

No appreciable change in the patient’s vision followed, and ophthalmoscopic examination made two weeks after operation showed no change in the appearance of the fundus, except that a cilioretinal artery in the upper part of the disk had doubled in caliber.

So far as I know, Case III. is the first instance in the history of medicine of an excision of the superior cervical ganglion, or of any part of the sympathetic system, for the relief of optic-nerve atrophy. Although the operation was not of benefit in this particular instance, yet I am not willing to concede that it will prove valueless in cases of non-inflammatory atrophy in which vision is not entirely lost. In truth, I expect it to prove beneficial in such cases, sufficiently often to justify the procedure.

I was led to make this experimental operation for several reasons: 1. The use of glonoin is often followed by an improvement in vision in cases of simple atrophy of the optic nerve. 2. Glonoin enlarges the retinal vessels, as has been proved by ophthalmoscopic examination. 3. There is no question that in glaucoma simplex—a disease in which there is an atrophy of the optic nerve—improvement in vision follows sympathectomy. 4. Excision of the cervical sympathetic is followed by an increase in the blood-supply of the orbital contents.

PATHOLOGIC CHANGES IN THE EXCISED GANGLIA.

The microscopic examination of three of the excised ganglia was made by my friend, Dr. Carl Fisch, of St. Louis. The specimens were those from Cases I., II., and III. Of the two ganglia removed from Case II. only the first one—the left—was examined.

Transverse and longitudinal sections of the three specimens were studied microscopically, by means of a great number of different staining methods. Owing to the method by which the ganglia had been preserved—weak formalin solution—the employment of the Golgi—Marchi—and the more delicate Nissl stains was rendered impossible. In general it may be said that the pathologic changes found were the same in the three cases, although a little less pronounced in No. 2 than in 1 and 3.

Most striking of all was a very marked hyperplasia of the connective tissue, which in some places resulted in dividing up the ganglion into small groups of nervous elements separated by broad bands of fibrous elements. The walls of the vascular structures showed decided sclerosis; the connective-tissue sheaths of the ganglionic cells were much increased in thickness. In Case I. small foci of round-cell infiltration were seen in this hyperplastic growth, of an inflammatory character. No plasma nor mast cells could be demonstrated.

The ganglionic cells were markedly pigmented. Together with a number of cells normal to all appearance there were great numbers showing different stages of degeneration. As a rule the nucleus, besides having lost part of its peculiar staining property, had assumed the parietal position; the nucleus was reduced in size or even missing in a large percentage of the cells. While in some cells the chromatic elements were well preserved, in others the process of chromatorhexis and chromatolysis could be followed up through all of its stages. Only comparatively few cells were seen showing the normal dendriform processes; very often the processes were short, ending bluntly, or they had even disappeared altogether. The general peripheral network of processes was much reduced in volume and compressed by the pressure of the connective-tissue formation. Only very few medullated fibers were seen. Unfortunately it was impossible to study their structure with the Marchi method.

The general pathologic aspect was that of a decided sclerosis, originating in inflammatory processes going on in, and starting out from, the walls of the vascular structures. The changes of the nervous elements were most likely not idiopathic, but due to pressure and inhibited nutrition.

The plates accompanying this paper have been made from drawings of sections of superior cervical ganglia.

TECHNIQUE OF THE OPERATION.

The ordinary precautions for surgical cleanliness are to be observed, and general anæsthesia employed. The incision should be made along the posterior border of the sterno-cleidomastoid muscle, starting at the mastoid process and running downward to within an inch of the clavicle. The sternomastoid is separated from the adjacent muscles, the spinal accessory nerve cut, and the carotid sheath reached. This dissection is made with the fingers. The carotid sheath should always be opened in order to locate the pneumogastric nerve. I consider this very important because: 1. The nerve is sometimes outside the sheath, as happened in my second case, in which the pneumogastric was much atrophied and was external to the sheath. 2. Differentiation of the cervical sympathetic from the vagus is sometimes difficult. Often, in operating on the cadaver, I have found both nerves inclosed in the same fascia. It is needless to say that excision of the vagus instead of the sympathetic would not only defeat the object of the operation, but would add a serious complication. Differentiation of these nerves after opening the carotid sheath is not usually difficult, for in working upward the operator comes upon the ganglionic expansion of the sympathetic. The ganglion is seized with forceps and stripped. Its branches are cut first, then the cord passing below is severed, and lastly the ganglion is cut above, as high as possible. It is best to use curved scissors and to have the finger under the ganglion while traction is made, thus cutting on the finger and avoiding injury to the underlying structures.

If the middle ganglion is to be removed, it will be best to excise it first and then work upward. If the entire chain of the sympathetic is to be removed, as is done for epilepsy, and as is now advised in exophthalmic goiter by Jonnesco, the operation is one of great difficulty, owing to the location of the inferior ganglion. This is situated near the neck of the first rib. One of my friends, who is a skillful surgeon, in removing this ganglion ruptured the vertebral artery near its origin and was obliged to tie the subclavian to check the hemorrhage. After the latter has ceased the wound is closed with superficial sutures. The hemorrhage in removal of the superior ganglion is usually trifling, only a few small vessels being cut. The external jugular vein was cut in my first case, but not in the others. The patient leaves the hospital on the eighth or ninth day.

Jonnesco’s method, according to his latest communication on the subject, is different. He always employs the premastoid route where only the superior ganglion is to be removed, reserving the postmastoid for the excision of the entire chain. The carotid sheath is split, the internal jugular vein and sternomastoid drawn outward by a retractor; a second retractor draws the vagus and internal carotid inward. In the space made the superior ganglion is found. The deep vertebral fascia is opened, all the branches of the ganglion isolated and cut by blunt, curved scissors; when this has been done the ganglion is attached only by nerve strands above, a strong pull is made, and the ganglion gives way. The excision is then completed by cutting the inferior strands. In closing the wound, he uses both deep and superficial sutures.

He mentions a transient dysphagia and pain in the cranio-mandibular joint as occurring after this operation.

EFFECTS OF EXCISION OF SUPERIOR CERVICAL GANGLION.

The effects of removal of this ganglion are immediate and remote: The immediate are relief of pain, lachrymation and conjunctival injection, together with a discharge from the corresponding nostril, unilateral sweating, and contraction of the pupil. Often there is an immediate reduction in intra-ocular tension. These effects are noted within five minutes after the excision.

The remote effects are ptosis, which appears on the third or fourth day, improvement of vision, and in some instances a tardy contraction of the pupil and a tardy reduction of the intra-ocular tension. To these there must also be added a slight sinking of the eyeball into the orbit, and a feeling of heaviness in the head. What I have just written applies particularly to cases of glaucoma.

In exophthalmic goiter, after the excision of the ganglia, the exophthalmus and tachycardia are said to improve almost immediately and a reduction of the goiter soon follows.

Although Jonnesco speaks of the immediate reduction of the intra-ocular tension, yet this does not always occur. In my second case, at the end of eight days the tension was + 2. On the sixteenth day the tension was normal. In my first case reduction of the tension was immediate. The relief from pain in the first case was immediate and lasting. This patient had not been free from pain for two months previously. The slight ptosis following sympathectomy is to be attributed to paralysis of Müller’s muscle. Sinking of the eyeball is no doubt due to paralysis of the unstriped peribulbar fibers found in Tenon’s capsule. Contraction of the pupil is usually an immediate result; it may, however, appear tardily. Thus in my first case the pupil was unchanged until the fourth day after the operation; and it did not become at any time as markedly contracted as in the other two patients. In the third case—that of optic-nerve atrophy—the pupil was markedly contracted within five minutes after the excision.

The lachrymation, conjunctival injection, and nasal moisture are transient symptoms which are usually absent after the first day.

In this connection it is interesting to note that Mr. Jonathan Hutchinson, as early as 1866, recognized many of the ocular symptoms of paralysis of the cervical sympathetic, and wrote a paper thereon.

HOW DOES EXCISION OF THE CERVICAL SYMPATHETIC REDUCE INTRA-OCULAR TENSION?

This is a question difficult to answer—difficult for the reason that we are not sufficiently acquainted with the physiology of the production of aqueous humor under normal surroundings. Panas and Duvigneaud have assumed rightfully that “If the nervous mechanism of intra-ocular secretion or, to speak without hypothesis, the action of the nervous system on intra-ocular tension can be known, the pathology of glaucoma will be cleared up, iridectomy will be explained, and perhaps a new and scientific basis for the treatment of glaucoma will be established.” Many observers have sought to solve the problem. Donders attributed the hypertension to a neuro-secretory cause and believed the trigeminus to be the agent of excessive secretion. He held that section of the trigeminus should relieve intra-ocular tension, while section of the cervical sympathetic could have no particular influence.

His views were overthrown by experiments made by Wegner in 1866, on rabbits. By means of manometers placed in the anterior chamber, he sought to record variations in the intraocular tension. He proved to his own satisfaction that the trigeminus takes no part, while section of the cervical sympathetic produces hypotonia, and irritation of its upper end and causes hypertonia. He held that section of the cervical sympathetic enlarges the blood vessels of the eye; the blood then flows under reduced pressure, and intra-ocular secretion is lessened. Almost identical results were obtained by Adamück—1866‒68—who experimented on cats.

Von Hippel and Gruenhagen believed that the cervical sympathetic contains vasoconstrictor fibers for the eye. Their experiments were made on cats and dogs. They found that irritation of the upper end of the cervical sympathetic causes in the cat hypertonia, while its extirpation increases intra-ocular tension. While, according to Wegner, the hypertonic action proceeds from the enlargement of vessels caused by cutting the cervical sympathetic, and the contraction of the blood vessels caused by the irritation of the nerve causes a hypertonic action, the contrary view is held by Adamück, Von Hippel, and Gruenhagen.

However this may be, there is no doubt that the trigeminus plays no great part in the production of ocular tension. Furthermore, the inefficiency of Bedal’s operation—stretching the nasal nerve—is explained by the fact that it is the cervical sympathetic, and not the trigeminus, which influences intra-ocular tension.

Jonnesco believes that the ocular sympathetic fibers from the brain and spinal cord pass through the superior cervical ganglion; permanent or intermittent irritation of these is accompanied by dilatation of the pupil, narrowing of the small intra-ocular arteries, contraction of the peribulbar muscular fibers, and probably an increased action of the elements which produce the aqueous humor. “As a matter of fact,” says Jonnesco, “any increase of the blood pressure will produce a permanent or intermittent narrowing of the arteries and cause the extravasation and increase in aqueous humor; then it is probable, although not definitely settled, that a permanent or intermittent irritation of the excito-secretory fibers is followed by an increase in the secretion of aqueous humor; the permanent or intermittent dilatation of the pupil pushes the iris into the iris-angle, closes the canals of the filtration zone, and hinders or prolongs the exit of aqueous humor from the eye; the permanent or intermittent contraction of the unstriped peribulbar muscular fibers closes the efferent veins of the eyeball, and hinders the venous circulation of the eye—hence the dilatation of the intra-ocular veins.”

He holds that excision of the superior cervical ganglion destroys all vasoconstrictor fibers of the eye. The arteries relax, the blood pressure is lowered, and extravasation is reduced. This operation destroys the excito-secretory fibers, thus limiting the amount of aqueous produced. The fibers which dilate the iris are destroyed, hence the contraction of the pupil reopens the iris-angle and removes the obstacle to the outflow of aqueous. The nerve-fibers supplying the unstriped muscular apparatus contained in Tenon’s capsule are destroyed, hence the pressure on the efferent veins is removed and ocular circulation is reestablished.

Jonnesco believes that the starting-point of the nervous derangement producing glaucoma is central: “When one removes the ganglion the point of origin of the influence will not be removed, but the communication between this center and the eyeball is destroyed.”

Regardless of the differing views of physiologists concerning the mechanism of the reduction of ocular tension, based on experiments made on the lower animals, there can be no difference of opinion concerning the effect of excision of the superior cervical ganglion in the human subject. The operations made by Jonnesco and others on the Continent, and by myself in America, prove that removal of the superior cervical ganglion causes a marked reduction of intra-ocular tension in glaucomatous cases. That the same effect occurs in eyes with normal tension is evident from my third operation—that done for optic-nerve atrophy.

EXTENT OF SYMPATHECTOMY IN DIFFERENT DISEASES.

Up to the present time excision of the cervical sympathetic has been performed for the following diseases: epilepsy, exophthalmic goiter, glaucoma, and optic-nerve atrophy. The question naturally arises: How extensive an operation is necessary in these affections? This I will attempt to answer:

In epilepsy it is necessary to excise the entire cervical chain on both sides for the reason that, according to Jonnesco’s theory, it is necessary to convert a state of cerebral anæmia—which he assumes is the condition in epilepsy—into one of cerebral hyperæmia. Since the carotid plexus is formed by branches from the superior ganglion, and the vertebral plexus arises from branches which have their origin in the inferior cervical ganglion, it is evident that the entire cervical sympathetic must be removed.

In exophthalmic goiter, although Jonnesco in his first operation excised only the superior and middle ganglia, he now believes it necessary to remove the inferior as well, for this reason: from the superior ganglion the ocular fibers arise; from the inferior the vasodilator, cardiac-accelerator, and, probably, the secretory nerves of the thyroid gland. If eye, thyroid, and cardiac symptoms are to be relieved the entire chain must be excised.

In glaucoma removal of the superior ganglion alone is necessary. All of the sympathetic fibers of the eye, with the exception of those which pass directly from the cerebrum by way of the trigeminus, are connected with the superior ganglion.

In optic-nerve atrophy, if it should be proved that noninflammatory atrophy of the optic nerve can be improved by sympathectomy, removal of the superior ganglion alone will be necessary, for reasons already given.

If the glaucoma is unilateral, it is necessary to remove only the corresponding ganglion.

HISTORY OF SYMPATHECTOMY.

In 1889 Alexander of Edinburgh resected the superior ganglion on both sides. In 1892 Jacksh resected the vertebral plexus and cut the cord connecting the middle and inferior ganglion. The third operator was Kummel, who excised the superior ganglion on one side only. In 1893 Bojdanik made a bilateral resection of the middle ganglion. In 1896 Jaboulay made a bilateral section of the sympathetic cord, above and below the middle ganglion. These operations were all made for epilepsy.

In regard to exophthalmic goiter, Jaboulay made a simple section of the sympathetic early in 1896. In September of the same year Jonnesco excised the superior and middle ganglia.

Jonnesco was the first, in 1896, to do a bilateral resection of all three cervical ganglia, though it is claimed by a Polish surgeon, Baracz, that he proposed the same in 1893. To Professor Jonnesco furthermore belongs the credit of having first excised the superior ganglion for glaucoma in September, 1897.

Ball of St. Louis was the first to remove the superior cervical ganglion for optic-nerve atrophy. The date of this operation was June 24, 1899.

Terrier, Guillemain, and Malherbe, in their “Chirurgie du Cou,” 1898, were among the first to give the surgery of the sympathetic a place in a text-book.

Among those who have operated on the cervical sympathetic for the relief either of glaucoma or exophthalmic goiter, or both, are Abadie, Réclus, Gerard-Marchant, Chauffand and Quénu, Jeunet, Bled, Ball, Renaud, and Bartlett.

Panas is opposed to sympathectomy in glaucoma. He reports seeing a patient in whom, three months after the operation, vision was still declining.

François-Frank, at a meeting of the Paris Academy of Medicine, held May 22, 1899, spoke of the effect of sympathectomy on the circulation of the thyroid gland, brain, and eyes, and on the heart. He believes that the operation can easily produce good results.

Doyon has described the trophic changes produced in the rabbit by excision of the cervical sympathetic.

CONCLUSIONS.

From a study of the cases of sympathectomy made by Jonnesco and others, and from the observation of my own cases, I offer these conclusions:

1. Excision of the superior cervical ganglion is a most valuable procedure in glaucoma.

2. It is of more value in glaucoma simplex than in inflammatory glaucoma.

3. In inflammatory glaucoma, on which iridectomy has been done without benefit, excision of the superior cervical ganglion should certainly be tried.

4. In cases of absolute glaucoma with pain, sympathectomy is to be tried before resorting to any operation on the eyeball.

5. In cases of simple optic-nerve atrophy, sympathectomy may possibly be beneficial if done before vision is entirely lost.

6. In cases of exophthalmic goiter, which do not improve under hygienic medicinal and electric treatment, excision of the cervical sympathetic on both sides is to be advised.