Scurvy Past and Present Part 11

=Hemorrhage.=--_Hemorrhage of the gums_ is one of the characteristic signs of scurvy. For a reason not clearly understood it involves first and foremost the tissues about the upper incisors. If, however, we fix our attention too narrowly to this region we may be led into error; in several instances we have first encountered hemorrhages about the molar or the canine teeth, which had been overlooked because the anterior part of the gums had been found normal. Where teeth are absent or not in the course of eruption hemorrhages do not appear. At the onset the gums may be merely deep red or bluish red, especially if they overlie upper incisors which are close to the surface. Hemorrhage is particularly apt to occur where the edges of the teeth have just broken through the mucous membrane. In this connection the question arises as to whether every hemorrhage of the gums in infants is to be considered a sign of scurvy. This is a matter of some diagnostic importance. We have seen hemorrhages of the gums at the site of erupting molar teeth where, as prolonged observation proved, not even latent scurvy existed. This sign should not, therefore, be regarded as pathognomonic. In two infants entirely free from scurvy we have noted slight hemorrhage of the gums overlying incisor teeth. It should be well understood that such an occurrence is most exceptional; it is to be attributed probably to bacterial invasion or to a const.i.tutional hemorrhagic condition. One of these cases was the following:

The infant was 8 months old. It had been nursed by the mother up to this time and was well nourished, but when first seen had some fever, probably due to a grippe infection. About ten days later distinct linear hemorrhages of the gums were noted over the two upper incisor teeth. No treatment was inst.i.tuted for this condition, and it healed within a week. There was no subsequent sign of similar hemorrhage or of other s...o...b..tic manifestation in the months that the baby was under observation.

The localization of the hemorrhage in the gums is due largely to trauma, occasioned by the sharp contact of the jaws or of the nursing-bottle.

Local infection plays almost no role in infants, although in the adult where there is caries of the teeth it frequently incites hemorrhage.

Dental caries and gingival infection may lead to local hemorrhage, even where the nutritional conditions are normal.

Subperiosteal hemorrhage is a sign distinctive of infantile scurvy, although it must be borne in mind that it may take place in the scurvy of adults. It involves most frequently the lower end of the femur and the tibia, but occurs in connection with the humerus, the mandible, the scapula and other bones.[47] The hemorrhage usually manifests itself as a swelling which appears suddenly at the lower end of the femur or femora. It is brought about by trauma, at times in the course of diapering, or by manipulation in testing for local tenderness. The swelling is very tender, and varies in size from an enlargement which is difficult to appreciate, to one which renders the leg fully twice its normal circ.u.mference (Fig. 18). It may involve merely a small part of the long bone or extend up or down the shaft for a long distance. As might be supposed from the nature of this lesion, the enlargement persists for weeks, frequently long after the gums and the general symptoms have disappeared. During this period it becomes harder and less tender, and may develop the consistency of bone; it is in this stage that such swellings have been diagnosed as new growths, and that incision or even amputation of the leg has been resorted to. In subacute cases the swelling--which must be regarded as hemorrhagic rather than s...o...b..tic--may be absorbed gradually in spite of the fact that no antis...o...b..tic food has been given. This has led to the mistaken conclusion that the scurvy has been cured without dietetic treatment.

[47] There seems to be some misconception as to the pathogenesis of the subperiosteal hemorrhage in scurvy. In most reports this lesion is described as if it resulted from a hemorrhage burrowing its way beneath the periosteum and raising it from the subjacent bone. In point of fact, such an event is impossible, as will be fully realized when one experiences the great difficulty in separating periosteum from normal bone. The s...o...b..tic process involves the periosteum so that it is no longer normal but becomes insecurely attached to the shaft of the bone, and is readily stripped off by hemorrhage.

Subperiosteal hemorrhage may be clearly seen by means of the fluoroscope or in X-ray photographs (Figs. 16 and 17). The shaft of the bone appears surmounted by an elongated blood-clot, which is more or less distinct according to its age and density. It may become calcified, as clearly seen in figures. More often the periosteum undergoes calcification or ossification, especially near the site of the separation of the epiphysis. This gives rise to a bizarre radiographic picture which may be difficult to interpret--the opaque strip or streamer being almost unrecognizable as periosteum (Fig. 17).

[Ill.u.s.tration: FIG. 16.--Infant 11 months old. Separation of lower epiphysis of femur. Fraying of end of femur and head of tibia.

Subperiosteal hemorrhage surrounding lower part of shaft of femur, with calcification of periosteum and of clot.]

[Ill.u.s.tration: FIG. 17.--Infant 11 months old. Separation of lower epiphysis of femur with marked subperiosteal hemorrhage. Typical periosteal "tags" or "streamers." The connection of these "streamers"

with the periosteal layer is evident.]

Hess and Unger observed that in several instances where subperiosteal hemorrhage had been diagnosed, X-ray examination disclosed that the swelling of the thigh was due mainly to infiltration of the muscles and subcutaneous tissue. It is surprising how an infiltration of serum gives rise to a swelling which resembles in appearance and consistency the cla.s.sical subperiosteal tumor.

The skin, mucous membranes and subcutaneous tissues are frequently the sites of hemorrhage. There is a difference of opinion as to how frequently petechial hemorrhages occur in scurvy, particularly as to whether they are encountered early in this disorder. Great variation in this regard may be noted in individuals and in groups of cases occurring at different times. In the cases reported in 1914 by Hess and Fish, petechial hemorrhages were frequently an early sign, to such an extent that they led to a study of the blood and blood-vessels in this disorder. The hemorrhages in this "scurvy epidemic" were the result of a complication of scurvy with an infectious disease. It is not necessary, however, for infection to exist to bring about a rupture of the small vessels. The idiosyncrasy of the individual has to be considered as well as the fact that infants have a tendency to develop minute skin hemorrhages, especially such as have an exudative diathesis. In the course of scurvy, petechiae may be found not only in the skin, but in the mucosa of the mouth, especially overlying the hard palate, and also in the palpebral conjunctiva, identical with the minute petechiae so significant of general sepsis. In addition to these minute hemorrhages larger ones are not infrequently found in various parts of the body, especially in the neighborhood of the joints. They appear as discolorations of various intensities and shades, and are often interpreted as being merely the result of bruises. These have been encountered most often about the knee-joint, on the forehead, or in the concha of the external ear, where they may best be seen by means of transmitted light.

A form of hemorrhage which must be especially mentioned, although it is very infrequent, is that taking place into the orbit, leading to a _proptosis of the eyeball_, usually the left (Still). This sign should be borne in mind, as it occurs occasionally before other symptoms have rendered the diagnosis clear, and may lead to a diagnosis of tumor.

As mentioned above, hemorrhages into the muscles or between the muscle planes are very common in adults, leading to hard swellings, the typical "scurvy sclerosis." Such effusions occur much less frequently in infants, due probably to their lack of activity. In addition to these hemorrhages there are serous effusions of the muscles similar to those which are found in the pleural and pericardial cavities. These effusions are very striking at necropsy, when one incises the muscles--for example, the muscles of the thigh. During life they are frequently mistaken for subperiosteal hemorrhages.

Less frequently there are _hemorrhages into the internal organs_. These, however, play a comparatively insignificant role in the symptomatology of this disease. At postmortem examination we find numerous hemorrhages of the pleura, pericardium and peritoneum, which rarely produce symptoms during life. Still records a case with marked abdominal pain and swelling, which he believed to have been due to hemorrhage into the wall of the intestine. As previously mentioned, O'Shea met with a case of hemorrhage into the caec.u.m which was mistakenly operated upon for appendicitis. Haemothorax and haeemopericardium occur, especially a.s.sociated with local inflammatory processes of tuberculous nature. The clinical aspect of hemorrhage of the gastro-intestinal and the genito-urinary tracts will be considered elsewhere.

In the scurvy of adults as well as that of infants, _the nails and the hair_ are altered by the nutritional condition. Mention has been made of the hyperkeratosis recently emphasized by Wiltshire as an early sign, occurring especially on the thighs and legs. The skin is frequently dry, the so-called "goose skin" that is seen in some poor nutritional states.

The nails are thin, brittle and lined; at times small hemorrhages will be noted beneath them. The hair also becomes thin and dry, and there is a tendency for petechial hemorrhages to develop at the roots.

In a paper on the therapeutic value of yeast and of wheat embryo the author called attention to the fact that _eczema_ may occur in connection with infantile scurvy, and be cured by means of orange juice.

We have met with eight cases of eczema in infantile scurvy, which, in almost every instance, have yielded promptly to an antis...o...b..tic, thus proving their s...o...b..tic nature. A case of this kind is the following:

M. L., seven months old, was getting "Molkenadaptierte" milk, and in addition autolyzed yeast. On May 25th it developed nasal diphtheria, but soon afterward did well. On June 9th it was gaining, but its pulse was 160 and respirations 80. A few days later it developed marked eczema about the neck and to a less extent on the back and b.u.t.tocks. The "capillary resistance test" was negative.

Cardiographic tracings showed merely a simple tachycardia. A few days later petechial spots appeared at the site of the eczema. On June 17th orange juice was given. The appet.i.te improved, the cardiorespiratory syndrome disappeared, and the child began to gain.

The eczema also cleared up rapidly without any local treatment.

We wish to draw particular attention to this skin condition, as it is generally not mentioned, or has been regarded merely as a chance occurrence. The report of the American Pediatric Society includes two cases of eczema as a complicating condition. This symptom is of special interest in view of the fact that a similar skin lesion const.i.tutes one of the typical signs of pellagra. In a case of infantile scurvy we have seen an eruption at the nape of the neck which was symmetrical and greatly resembled that of pellagra. Andrews refers to the occurrence of eczema in his description of infantile beriberi.

In a paper published a few years ago attention was drawn by Hess and Fish to the fact that infantile scurvy frequently is a.s.sociated with the exudative diathesis of Czerny, a pathological condition which predisposes to the development of exudations of the skin and the mucous membranes. Infants suffering from this condition--intertrigo, eczema, recurrent bronchitis--seem to be particularly susceptible to scurvy and to develop it more quickly than others.

As is well known, _edema_ const.i.tutes a not infrequent symptom of adult scurvy. It has not, however, been accorded any place in the symptomatology of infantile scurvy. We do not refer to the edema in connection with subperiosteal hemorrhage or separation of the epiphyses of the long bones, but a mild and peculiar form which is seen early in the disease. It involves most regularly the upper eyelids, and the legs--especially the skin covering the lower part of the tibiae. In the latter site it differs from edema as usually encountered, in that it does not pit on pressure; it is firm, tense, causing some glossiness of the overlying skin, which is rendered difficult to wrinkle or to pinch between the fingers. Not infrequently the skin is slightly reddened, a sign of interest, in view of a similar, although much more intense, hyperaemia seen in pellegra.

In addition to this very mild edema there may be marked swelling, resulting in what might be called, following the terminology of beriberi, "wet scurvy." The legs, body and even the face may be swollen.

This has been frequently described in adult scurvy, and occasionally in infantile scurvy. The first case of infantile scurvy described in America, that of Northrup, had marked edema of the s.c.r.o.t.u.m. Edema is frequently met with in "ship beriberi," a disorder considered by some writers to be a combination of beriberi and scurvy.

The symptom leading to the diagnosis of scurvy most often is _tenderness_ or swelling of one of the extremities, as the antecedent clinical signs, comprising latent scurvy, are generally overlooked.

These manifestations involve usually the distal end of the thigh or thighs. The tenderness is elicited most readily by pressure just above the knee, which causes the baby to wince, and to quickly flex the thigh, a reaction termed by Heubner "the jumping-jack phenomenon." As a result of pain and tenderness, the leg lies often immobile in a state of pseudo-paralysis (Fig. 18). There may be tenderness elsewhere than in the long bones. Kerley refers to two cases showing tenderness of the spine, and we have seen a similar case. Not infrequently there is tenderness of the chest wall, the earliest symptom noted by nurse or mother being unaccountable crying whenever the baby is lifted by the thorax. This is largely due to the sensitiveness of the ends of the cartilage and bone which are pressed together at their junction.

[Ill.u.s.tration: FIG. 18.--Infant with marked scurvy. Characteristic posture and swelling of right thigh.]

An early sign of infantile scurvy is _beading of the ribs_--the development of a "rosary" similar to that characteristic of rickets (Fig. 19). This has recently been described by Hess and Unger in an article devoted to this subject. That this rosary is truly s...o...b..tic and not rhachitic is proved by the fact that it recedes rapidly when antis...o...b..tic foodstuff is given, and that it remains uninfluenced by treatment with cod liver oil. A similar s...o...b..tic rosary occurs in guinea-pig scurvy, but has been termed "pseudo-rhachitic." It is important that this sign should be recognized, as it is probable that much of the confusion regarding the relationship and frequent a.s.sociation of these two diseases is due to considering the beading rhachitic. The interpretation of infantile scurvy as "acute rickets,"

the view held previous to the writings of Barlow, was based largely on the development of the rosary. To-day the error is made of regarding early scurvy as chronic rickets; the rickets supposed to be occasioned by a diet of condensed milk is probably more often scurvy. This beading differs generally from the round k.n.o.bby "rosary" usually encountered. It is more angular, the junction taking on a step-like form, as if the ab.u.t.ting ends of the cartilage and the bone were of unequal size, and not well fitted to each other. In the accompanying radiograph (Fig. 19) it will be noted that the "beads" present an irregular appearance.

[Ill.u.s.tration: FIG. 19.--Same infant as in figure 17. s...o...b..tic beading of the ribs (rosary). This developed on a diet which included cod liver oil, and decreased when an antis...o...b..tic was given. Note peculiar ragged appearance of "beads."]

In Figs. 6, 16 and 17 will be seen ill.u.s.trations of a _separation of the epiphyses_ of the head of the humerus, and of partial and of complete separation of the lower ends of the femora. This is a frequent lesion of fully developed scurvy in infants, children, and even in young adults.

It is most frequent at the lower end of the femur, the upper end of the tibia, the head of the humerus, and the costochondral junctions. It is to these epiphyseal separations that the term fracture or infraction usually refers. Union is remarkably perfect even where no splint has been employed, and nature has effected the cure (Fig. 7). Occasionally there is some deformity, as when c.o.xa vara develops. The callus is often remarkably large; an old callus sometimes undergoes destruction in the course of scurvy.

We have referred to use of rontgenograms in connection with separation of the epiphyses, subperiosteal hemorrhage, cardiac enlargement and beading of the ribs. In addition to its application in these connections, the X-ray may be of service to show a peculiar alteration of the ends of the long bones--_the white line of Fraenkel_. This is portrayed in Fig. 20. It is best seen at the lower ends of the radius and femur, and appears as a white, transverse, somewhat irregular band.

Its diagnostic value has been greatly exaggerated, as it is frequently not present when the disease is advanced (observe radiographs ill.u.s.trating separation of the epiphyses). This sign should therefore not be relied on for establishing the diagnosis. Furthermore, changes may be seen in connection with rickets (cases receiving antis...o...b..tic diet) which are very difficult to differentiate from the "white line."

It cannot be employed as a criterion of the progress of the case, as it may persist for months after all other signs and symptoms have disappeared.

[Ill.u.s.tration: FIG. 20.--Radiograph. Infant 14 months of age, showing "_white line_" at wrist some months after cure of scurvy.]

The joints may be involved in scurvy. In most instances, however, where swelling of the joints is diagnosed, the lesion is periarticular. An effusion of serum or of blood does occur occasionally into the joints and has been found at operation, at necropsy, and by puncture. If these effusions are allowed to go undisturbed, to be absorbed as a result of antis...o...b..tic treatment, they rarely suppurate. Czerny and Keller report the articular fluid as invariably sterile.

The cardiovascular system has been given but scant attention in connection with scurvy. Adults complain not infrequently of palpitation and pain over the pericardium, or rather of a tightness or oppression in the chest. Little information is given regarding the size of the heart.

Darling described enlargement of the heart, especially a right-sided hypertrophy, which he thought was pathognomonic of the Rand type of scurvy. The pulse is described in some cases as slow, and in others as rapid. In descriptions of infantile scurvy the entire subject is generally pa.s.sed over without mention--for example, in the excellent report of the American Pediatric Society nothing whatsoever is stated regarding the heart's action or the pulse. Barlow wrote: "There is nothing to note regarding the heart and lungs."

In a paper written a few years ago, it was pointed out by the author that there is frequently enlargement of the heart, and more especially of the right heart. This can be elicited at the bedside and has been substantiated in numerous cases by means of the Rontgen-ray, which demonstrates not only enlargement of the heart, but also a marked broadening at its base, at the site of the large vessels (Fig. 21).

These phenomena resemble closely the description of Reinhard in cases of beriberi.

[Ill.u.s.tration: FIG. 21.--Radiograph. s...o...b..tic infant 14 months of age, showing cardiac enlargement and broadening of shadow at base of heart.]

Necropsy protocols usually are incomplete and unsatisfactory in their descriptions of the heart. The excellent monograph of Schoedel and Nauwerk, however, which reports five careful necropsies, contains the following data regarding three:

1. Pericardial fluid somewhat increased, both ventricles moderately dilated, the right somewhat hypertrophic.

2. The heart showed a hypertrophy of the right and left ventricles, as well as dilatation of the right ventricle.

3. The right ventricle dilated and slightly hypertrophied, the muscles pale and tough.

In addition to this enlargement of the heart, or perhaps a.s.sociated with it, there is a combination of signs which has been termed "_the cardiorespiratory syndrome_" (Hess). It will be noted in the above description of a case of subacute scurvy, that the pulse- or heart-beat was frequently over 150, and the respiration 60. These phenomena were noted in several instances before their significance and intimate relationship to scurvy were realized. The heart-beat not infrequently is found to be 200 per minute, and to be characterized by marked lability--increasing to an astonishing degree as the result of slight exertion or excitement. A mild febrile disturbance causing a rise of temperature to little more than 100 F. will send the pulse-rate up 30 beats. It must not be thought that this refers to severe cases; the babies we have in mind are similar to the one cited as an instance of subacute scurvy. Apparently they are not ill, but show merely some tenderness of the thighs, pallor, and the other minor signs described.

The cardiographic tracings showed a simple tachycardia with an exceptionally tall T-wave in some tracings, such as is commonly seen in exophthalmic goitre (Fig. 22).

[Ill.u.s.tration: FIG. 22.--Electrocardiogram in case showing cardiorespiratory syndrome. Tachycardia with exceptionally tall T-wave.]

The rapidity of respirations is perhaps a more delicate indicator of this disturbance than the pulse and has been found to be markedly affected when the latter was merely slightly increased in rate. For example, in one instance the respirations were 64, 60 and 64 on three successive days, while the pulse was 124, 141 and 136; in other words, there was a 2:1 instead of the normal 4:1 pulse-respiration ratio. The accompanying chart (Fig. 15) ill.u.s.trates the phenomenon in all its details better than a verbal description. There is one point in connection with it, to which especial attention should be called. This is a reaction evident at a glance at the chart--the sharp drop in the pulse and in the respiratory rate when orange juice was given. It is the essence of the phenomenon; a therapeutic response which proves that the rapidity is s...o...b..tic in nature.

The main involvement of the _respiratory system_ in scurvy is the polypnoea just described in connection with the cardiorespiratory syndrome. There is no aphonia, a sign so typical of adult and of infantile beriberi, although at times the voice is abnormal and whining.

The lungs frequently show some dullness posteriorly, which may be due to engorgement or to the pressure of the enlarged heart. Pneumonia is a frequent complication and edema a terminal event. Hydrothorax a.s.sociated with hydropericardium is of frequent occurrence, and was noted in the early description of this disease in adults and in the first account of Barlow. These effusions rarely progress to what may be termed the clinical degree and under antis...o...b..tic treatment are rapidly absorbed.

It is commonly thought that scurvy does not involve _the nervous system_; that this is a feature which distinguishes it sharply from beriberi, another "deficiency disease." This view is incorrect, for the nervous system is probably affected in many cases of scurvy. The rapidity and lability of the pulse, combined with the rapid respirations, would seem to be due to a disturbance of the vagus mechanism. It is true that in beriberi the vagus is involved to a still greater extent, especially its recurrent laryngeal branch which brings about the characteristic aphonia. In scurvy the knee-reflexes are generally increased. Very rarely they are absent in infantile scurvy, as described in adults. It is impossible to judge whether the pain and tenderness in infants are due in part to a sensitiveness of the nerve trunks as well as of the periosteum. Careful studies in adult scurvy should furnish an answer to this question. No methodical examination for areas of anaesthesia or paraesthesia, signs which occur so frequently in connection with beriberi, has been carried out in scurvy. In certain epidemics, however, pains in the limbs have been prominent symptoms.

The optic discs are generally pale in both infants and in adults, with occasional signs of neuredema. Nyctalopia, so frequently encountered, must be regarded as a circulatory symptom rather than as one of nervous origin.