CHAPTER XIV

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THE STATUS LYMPHATICUS.

Under the term _status lymphaticus_ has been described a condition which is of interest to the surgeon, as it includes not only minor complications, and even those which are serious, which follow surgical procedure, but it also has reference to the cases of sudden death during or after operation, some of which have been attributed to the anesthetic, while others have been considered absolutely unexplainable. The condition is so easily described or defined that it should be recognized during life, but it has often been discovered only after sudden death.

The essential feature of the condition is _enlargement of the lymphatic tissue and apparatus_, perhaps throughout the entire body, more frequently through its internal portions. It usually occurs in children. It is accompanied by much lowering of the power of resistance, and results in sudden death from cardiac failure, as the result of causes which seem disproportionately trivial; as, for instance, such deaths as occur not merely during anesthesia, but during bathing, convalescence from the exanthemas, etc. It stands in close relation to diphtheria, perhaps because of its peculiarly depressing toxins, and probably accounts for cases of sudden demise in that disease, even when mild.

The status lymphaticus is also designated by other names, as _lymphatism_, _lymphatic constitution_, and _status thymicus_, the latter because of the active participation of the thymus. Enlargement of this body has been noted at autopsy, but its relation to the other features of status lymphaticus were unknown until Rokitansky, in 1842, first recognized the condition in its entirety, but confused it with the tuberculosis of the lymphatics formerly called scrofula.

The death of a son of a German professor, a few years ago, shortly after an injection of antitoxin to protect him from diphtheria, and the subsequent discovery that the boy was the victim of this condition, caused a widespread interest in and a most careful study of the problems involved. They occur in the thymus more frequently than in any other organ of the body. Normally the thymus begins its involution within a few months after birth, and this process should be completed at puberty. In the status lymphaticus this involution does not occur, but enlargement persists or increases even into adult life, varying in different cases, the weight of the thymus varying from 20 to 135 grams.

While the thymus may for some purposes be grouped among the lymphatic tissues of the body, little is known as to its function. Its juice contains leukocytes, which find their way into the general circulation, and it is supposed to have an internal secretion correlated with that of other ductless glands.

Injections into dogs of thymus extract produce a fall in the blood pressure, with acceleration of the heart, and, in fatal doses, dyspnea and collapse. While enlargement of the thymus may cause death by pressure on important structures other than the trachea, it produces a type of asthma known as _thymic asthma_, in which death sometimes occurs unexpectedly and rapidly by strangulation.

An examination of the thymus rarely shows anything more abnormal than the enlargement of its natural structure, with perhaps acute hyperemia, while occasionally the cut surfaces will exude a milky fluid; there will be found, in addition to these changes in the thymus, a general hyperplasia of the lymphatic system, with enlargement of the superficial and deep nodes, especially the cervical and axillary, the inguinal and those in the abdomen. The spleen enlarges and the Malpighian bodies seem to be packed with lymphoid cells. There may be enlargement of the heart and increase in the thickness of the arterial walls. This is so marked that Virchow suggested the name _lymphatic chlorotic constitution_. It has been suggested that the narrowing of the aortic valve in these cases is due to this lymphoid infiltration.

_The relations between rickets and the status lymphaticus_ are so frequent and so conspicuous as to make one suspect a more than casual connection between them. Nearly all cases of lymphatism show the ordinary clinical evidences of rickets. By some such relation may be explained the benefit which accrues in rickets from the administration of the extract of thymus, as well as of the thyroid and the pituitary body.

Enlargement of the lymphoid tissue in the wall of the alimentary canal also occurs, and in those rings of adenoid tissue which mark the site of the embryonic canals. This tissue may be seen around the origin of the appendix, while its most conspicuous illustrations are seen about the pharynx, where not only the faucial, but the lingual and the pharyngeal tonsils are enlarged. In many of these cases there are the so-called “adenoids” of the throat specialists, while, of still greater interest to the surgeon, the deaths that have occurred from the status lymphaticus have happened repeatedly in operations for these growths within the nasopharynx. Furthermore, the yellow marrow of the bones seems to be replaced by red marrow, but whether this is due to the anemia which always accompanies the condition is not known.

Kaposi some years ago described under the name _lymphodermia perniciosa_ a rare condition characterized by a scaly and itching skin, exuding fluid, with later a diffuse and doughy condition of the affected parts, and then by nodules which sometimes ulcerate, lymph nodes and spleen being also enlarged, and the general health impaired. While some have held that this is a variety of mycosis fungoides, it is supposed that it is only another expression of lymphatism.

Another variety of this condition occurs in young people, in which coma comes on suddenly, followed by death in twelve to eighteen hours. Vomiting may occur during the coma, but it is convulsions and spasm of the glottis that cause the death of the patient.

_Thymic asthma_ has been called _laryngismus stridulus_. Whether the latter can ever occur without the former is not definitely known, but doubtless the asthma is very frequently the cause of the obstruction and the difficulty in breathing.

Medicolegal questions arise in this connection which are of interest. Death occurs, except under anesthesia, after a series of convulsions, yet it may happen almost instantly. Some claim that death may take place as the result of pressure of an enlarged thymus upon the vessels, and especially upon the nerves, while others claim it to be due to a sudden arrest of heart action by reflex activity.

Convulsions of any character in adolescent individuals and young children should raise a suspicion of this condition, and, of greater importance for the surgeon, all possibility of existence of the condition should be eliminated before operation is undertaken. Deaths occurring during anesthesia are often attributable to the anesthetist; nevertheless there are instances where he is absolutely blameless, and where death may occur as by a flash of lightning.

It does not follow that chloroform is the agent at fault in these cases, and opinion seems to trend in the direction of ascribing the censure to the status itself rather than to the anesthetic used. Deaths may occur at any stage of anesthesia, or some minutes after the anesthetic has been stopped. It is significant that the most conspicuous illustrations of the relations between the condition and sudden death have occurred during operations upon the throat and nose. This seems to show the role played by the adenoid tissue.

Another interesting question is why individuals with well-marked status lymphaticus should live, apparently comfortable for years, and then suddenly succumb from apparently trifling causes.

The relations between the thymus and the thyroid are unmistakable, yet obscure. In perhaps one-half of the cases where the thymus is enlarged the thyroid is also increased in size. When one is removed the other seems to undergo more or less compensatory enlargement. This would seem to indicate a species of interchangeable function. Much less has been ascertained between the relations of either of these bodies and the pituitary, while nothing has as yet appeared concerning any sympathetic involvement of the coccygeal body or Luschka’s gland.

=Diagnosis.=--Recognition of the status lymphaticus during life is somewhat difficult, nevertheless there are certain suggestive features which should arouse suspicion. Of these the close relation between the status lymphaticus and rickets, already alluded to, furnishes a hint, and, when recognized, a positive warning. Widespread enlargement of the lymph nodes may furnish another. Adenoid growths in the nasopharynx accompanied by enlargement of the spleen should be regarded as a suspicious combination; and when an area of dulness is discovered over the thymus, or when it can be detected by palpation, the diagnosis may be regarded as established. Moreover, children who are subject to this condition usually have a pasty complexion and an anxious facies. Besides showing evidences of rickets they are anemic, with liability to spasm of the glottis. The thyroid is often enlarged. In young adults the condition may simulate cretinism, in that they are retarded in growth and infantile in appearance, while sexual development is incomplete.

=Treatment.=--In well-marked instances of status lymphaticus there should be ordinarily no operative intervention; yet when the nose and pharynx are obstructed it is advisable to give free channels for breathing purposes.

Assuming that the result of experimental injection of thymic juice shows it to have a depressing and pressure-lowering effect, an effort should be made to ward off danger by the use of adrenalin, which should be given previous to the commencement of the anesthesia. These are cases where it is best to treat the surfaces to be operated with a spray of mild cocaine solution, in order to deaden liability to those impressions which may produce secondary and reflex cardiac disturbances if conveyed to the brain. When operation is necessary for glottic spasm or laryngismus stridulus it may be commenced with a tracheotomy, with the use of a long trachea tube. When operation is required for the relief of thymic enlargement, a preliminary tracheotomy should be made, with the use of a long tube. The improvement which results after the completion of the surgical treatment, for instance after removing adenoids from the nasopharynx, is gratifying.

The most reliable measures have proved to be adrenalin and artificial respiration, used as described in the chapters on Blood Pressure and Shock and Anesthetics.

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