Scorbutic beading of the ribs

American Journal of Diseases of Children VoL 19 MAï, ¡920 Nc. 5 SCORBUTIC BEADING OF THE RIBS ÅLFRED F. HESS, TI.D., AND LESTER J. UNGER, I\{.D NEYyYORK For many years beading of the ribs has been regarded as a sign characteristic of rickets. The "rachitic rosary" is sought for in order to establish the diagnosis, and its presence is considered pathognomonic of this disorder. In the course of a study of rickets begun about two years ago, rtre became especially interested in this sign, which was found to be one of the most valuable in follorving the course of this disease. It rvas soon appreciated that it varied greatly not only in degree, but also in kind, and that in order to make reliable use of this sign several factors always must be taken into consideration. As is well known, beading may be very slight, or the costochondral junctions may stand out, so as to be evident to even the casual observer. The clinical importance of this slight degree of beading, fourid so frequently on careful examination, is as yet undetermined. In order to follow the variations in the beading, six grades were formulated, which were designated in the records by plus minus 1-+) or plus (*l-) signs, + + f representing the maximal degree. It may seem that an empirical method of this kind is too inexact to furnish reliable clinical data. An experience of several hundred cases and more than one thousand examinations, has demonstrated, however, that with even moderate experience one can obtain surprisingly uniform results by this means. It has almost never happened that the physicians of the clinic differed in their appraisal of the beading to the extent of more than one degree; in other words, one observer might rate the beading at plus minus ( -r ) and another at one plus (*), but the difference of opinion was not greater. A large number of infants were followed in this way; the examinations tvere made every month by ope of us (,4.. F. H.), and a careful record was kept of the beading and other signs generally associated with rickets. As stated, beading differs not only in degree, but also in character. The costochondral junctions as a rule are rounded, smooth, knobby. There may be, however, what we have termed ,.angular beadingr" the junction taking on a steplike form, as if the abutting ends of the cartilage and of the bone were of unequal size, and not l,yell fitted to each other. At necropsy such is found frequently to be the case; the cartilage overtops the bone so that on palpating the joint there is a precipitous fall as \{,e run the finger outward from the surface of the Downloaded From: http://archpedijamanetwork.com/ by a Penn State Milton S Hershey Med Ctr User on 0512712015 332 AMERICAN JAURNAL OF DISEASES OF CHILDREN cartilage to that of the bone, or a sudden elevation on palpating the rib from without inward toward the sternum.l This angular beading is met with more often in infants more than 1 or lr/z years of age, and in many cases should be regarded as "residual beading," remaining after the 'rachitic process is no longer active. Cases which show residual beading are, therefore, not appropriate to test the value of therapeutic agents. In these instances cod liver oil will generally fail to diminish the beading, even though it be of rachitic origin. In the course of rickets, as is rryell known, the lower free border of the ribs may gradually become everted and project forward. There may occur what has been termecl "flanging of the ribs." Flanging also takes place usually in the later stages of the disorder, rvhen it shows a tendency to become stationary or to recede. This change in the bony structure naturally affects the beading, sometimes obliterating it, and in other instances causing it to become less evident externally. Whereever this eversion occurs, all attempts at following the course of the beading, and the progress of the disorder which gives rise to it, must be abandoned, and the case considered unsuitable for further study from this vielvpoint. As observed by many invesrigators, the beading of the ribs occurs most often in the middle tier, especially in the fifth, sixth and seventh ribs, and less often in the upper and in the floating ribs. Why such is the case is not thoroughly understood. Its explanation is involved in an understanding of the pathogenesis of beading. Some have thought it due to the fact that these .ribs grow most rapidly, and others that there is a greater degree of motility in the costochondral junctions at this level. A clinical test of the latter hypothesis proved inconclusive. In several cases we attempted to immobilize one half of the thorax by applying strips of adhesive plaster to the chest from the sternum to the spine. These were applied continuously for about two months, but did not reduce the rosary. On the other hand, in another group of cases the costochondral junctions rvere subjected to vibration daily, for ten minutes, during the same period of time, without having a decided effect in either an increase or a diminution of the size of the junctions. We do not wish, however, to discuss beading of the ribs from the point of view of rickets, but rather to draw attention in some detail l. Probably it is this condition which Barlow refers to in his article on Scurvy (Cyclopedia of the Diseases of Children, 1890, Philadelphia ZzZ72): "What had been taken during life for beads proved to be simply the ends of the costal cartilages abutting against ribs which ïrere so extremely wasted that their anterior ends by no ûreans came into comBlete apposition with the rvhole of the ends of the costal cartilages. There lvere no beads at the posterior surface. It was a wonder that the ribs had not separated from the costal cartilages or fr.actured beyond, they were so exceedingly llrittle. There was, in fact, nothing but a shell of bone containing a little soft red medutla. On the parietal pleura of both sides there were numerous petechiae corresponding wíth the ribs." Downloaded From: http://archpedijamanetwork.com/ by a Penn State Mitton S Hershey Med Ctr User on 05/2712015 HESS-ANGER_SCARBUTIC BEADING 333 to a fact rvhich we recently pointed out,e namely, that beading may be due to causes other than rickets, and more especially that there is true scorbutic beo.di,ng. The difficulty of concluding in a case.of scurvy that the beading is scorbutic is th¿t possibly conrplicating rachitic beading rnay likewise be present. We have had excçtional opportunity, however, to see cases where this explanation could not well be advanced, where beading developed in spite of the fact that infants had been receiving adequate amour¡ts of cod liver oil for long periods. In three instances, not only marked beading, but enlargement of the epiphyses as well, developed, although the babies had been given cod liver oil, three teaspoonfuls daily, for a preceding period of four months or more. This treatment had been b.gun when the babies we,re only 2 or 3 months of age. Further proof of the scorbutic character of these lesions was the fact that both the beading and the enlarged epiphyses were reduced markedly in size (Table 1) within six weeks after giving orange juice, three teaspoonfulls daily. Table 2 illustrates the fact that where cod liver oil has not been taken, ,rachitic beading may develop in the course of scur\.y and persist in spite of many months of antiscorbutic treatment. TABLE l.-Sconsuflc BBe¡¡Nc r¡¡ Two C¡sss or Scunvv (N. M. exo I. L.) This sign developed in spite of a dietary that included cod liver oil, and decreased rapidly when orange juice (15 c.c. daily) was added.* Beading Epiphysis Beading EpiÞl¡ysie Diet Àge' Ìlonths age, Months o 6 ô ++ +++ ++ + ++ ++ + + + I -|. :t 6 a 8 I ++ ++ + + Cod liver oll (rã gm. dally) 0range lulcc adtled (15 c.c. daily) " ¡! siguiles the least, aaal +++ the aax¡mum degr€e ot beading. Another group of cases significant in this connection is composed of infants who have scurvy, and who on receiving an antiscorbutic - one tablespoonful of orange juice or two tablespoonfuls of canned tomato - lost the beading of the ribs as well as the signs typical of scrrrvy. This also is illustrated in Table l, and by other cases which we have observed. There can be no question of having cured rickets instead of scurvy by means of this orange juice and tomato. An ex¡rerience of a year ago with a larger number of infants who were found to be suffering from acute or from latent sctlfvy was striking in this regard. The beading, t'hich was of most marked degree, nrelted arilay undef the influence of treatment with canned tomato. In passing, it may be suggestecl that this involvement of the costochondral junctions is the cause of the thoracic tenderness which is so evident when scorbutic infants are grasped by the thorax. 2. Hess, A, F., and ÏJnger, L. J.: J. A. M. A. 24:217 (Jan.) 1920. Downloaded From: http://archpedijamanetwork.com/ by a Penn State Milton S Hershey Med Ctr User on 0512712015 334 AMERICAN TOURNAL OF ÐISEASES,OF CHTLÐREN Tobler's I experience in a recent epidemic of scurvy in Vienna is illuminating in this particular as it concerns older children, between the ages of. 2 and, 15 years, who are far less subject to rickets. In more than 200 cases he noted the development of a rosary almost regularly. Tobler wâs uncertain, however, rutrether the swellinngs should be attributed to scurvy or whether they were entirely of rachitic origin. fn connection with this subject, a case described many years ago by Fraenkel a is convincing. A child, 7 years of age, developed acute scurvy. The thoracic beading was found clinically and was verified at the necropsy; its true scorbutic character was substantiated by mioroscopic examination. This would seem to constitute conclusive evidence of scorbutic beading from the pathologic standpoint TABLE 2.-R¡c¡urrc Benur¡¡c ¡N e Cese or Scunvy (S. ,4.., Acen l5 Mourns) Cod liver oil had not been included in the dietary. Beading wås not diminished by an antiscorbutic, atthough all the scorbutic signs disappeared. Aosary Eplphyeis Dlet 1917 April. June. +++ ++ ++ ++ +++ +++ ++ ++"+ ++'{- +++ ++t + + ++ .r? +++ ++ ++:! ++ Mâ¡t souÞ Can¡od tomato, 1F3oc.c. added Oanned toeato, 45 c.c. Canned to![ato, 1¿oc.c. JuIy... .... . Augngt..... September. October.,., November.- December........... 1918 January ++-þ There can be no question that scorbutic beading occurs in animals. This change has been noted by many who have produced experimental scurvy. It is one of the most vivid and striking phenomena on opening the thorax of the scorbutic guinea-pig, and is the counterpart of rvhat is found in infants. Frequently, it is most marked on the pleural aspect, it involves the middle tier of ribs, and is the site of subperiosteal hemorrhage. It can even be differentiated into an ¿ngular and a round variety. Although this alteration at the costochondral junctions has been observed frequently, it has, to our knowledge, not been brought forward as essentially scorbutic in nature. Some observers have passed it by with mere mention, others have termed it "pseudo-rachitic," whereas still others, notably Holst and Froelich,õ although showing its value in the study of guinea-pig scurvy, have not attempted to correlate it with the symptomatology of human scurvy. These authors showed that the microscopic lesions at the costochondral junctions are typical of scurvy - the zone of bone disintegration, the framework +'.1- +.{- + I 3. Tobter, W.: Ztschr. f. Kinderh. 18:63, 1918. 4. Fraenkel, E.: Fortschr. a. d. Geb. d. Roentgenstrahlen, supplement rB, lg0g. 5. Holst, 4., and Froelich, J. : Ztschr. f. Hygu. Infektionskiãnkh. TZzl',l9l?. Downloaded From: http://archpedijamanetwork.com/ by a Penn State Milton S Hershey Med Ctr User on 0512712015 ¡/.8.S.'_U¡/GER-.SCOR B U T I C B EAD I N G 335 mârro\r¡, the subperiosteal and medullary hemorrhages, are all present. The experience of others, as well as our own, serves merely to bear out their conclusions. It may be stated, therefore, that clinical signs of beading can be made out distinctlv in guinea-pigs du,ring life, that it is evident on gross examination after death, and that microscopic examination serves to verify its scorbutic nature. The work of Hart and Lessing on monkeys is corroborative in this respect; they described enlargement of the costochondral junctions which they term a "rachitic rosary," in spite of the fact that they found on microscopic examination lesions typical of scurvy. fn connection with the subject of beading, the recent work of Aschoff and Koch 6 is of interest. In their pathologic studies on scurvy among soldiers they frequently describe beading of the ribs, which they attribute to an inf,raction of the costochondral junctions. A convincing account of scorbutic beading is contained in an article by Boerich, which describes scurvy in Russian soldiers. He mentions the beading of the ribs, especially from the third to the seventh, which led, in many cases, to a diagnosis of pleurisy or pneumonia by the army surgeon. In adults the phenomenon can hardly be attributed to rickets. Beading is as characteristic a sign of infantile scurvy as it is of rickets - appearing and disappearing with the other signs and symptoms of the disorder. As mentioned in the article referred to above, it occt¡rs also in connection with beriberi, another so-called deficiency disease. Andrews ? reported that among eighteen necropsies made on infants dying of beriberi, he encountered three cases with beading of the ribs; in a personal communication he has stated that these were the only instances of "rickets" which he met with at necropsy in the Philippines. We have found that in some instances beading disappeared on adding 30 c.c. of autolyzed yeast to the diet. It is of interest to learn that beading occu.rs in association with pellagra. \Meston I makes this observation in his article on pellagra in children, and Agostini e states that "up to 19 years of age, one-fourth of these unfortunates shorv rickets." It is probable that in nrentioning these causes of beading rve have not exhausted the possibilities from an etiologic standpoint. It wiil be remembered, that until Barlow's rvriting, scu,rvy ü¡as confused with rickets, that even Moeller, who was one of the first to sense the distinction betu.een the two clinical conditions, considered infantile scuwy to be merely "acute rickets." For many years a lively discussion followed as to rvhether the so-called Barlow's disease was not merely a form of rickets. Hirschsprung contended stoutly for this 6. Aschofi, L., and Koch, 'W.: Skorbut. Gustav Fischer, Jena, 1919. 7. Andrews, V. L.: Phitippine J. Sc. 7: Sec. B, 67, 1912. 8. Weston, W.: Am. J. Dis. Child. T:124 (Feb.) 1914. 9. Harris, H. F.: Pellagra, Ifacmilan Co., New York, 1919. Downloaded From: http://archpedijamanetwork.com/ by a Penn State Milton S Hershey Med Ctr User on 0512712015 336 AMERICAN ]OURIíAL OF Ð/SEI.SË.' OF CHTLDRÐ| point of view, and many pathologists, for example Nauwerck, regarded the scurvy merely as "an episode in the course of rickets." 10 Others believed that infantile scurvy developed necessarily on a rachitic basis. In England, Cheadle held this viery. The fundamental basis for this protracted confusion between the trvo disorders was the fact that beading of the ribs was to be found in almost all cases of scurvy, and this sign was looked on as characteristic solely of rickets. There could be little misinterpretation of the other clinical signs and symptoms - the skin hemorrhages, the subperiosteal swelling, the marked tenderness and pain ,resembled in no respect the signs and symptoms of rickets. When we read in the excellent investigation of infantile scurvy carried out some years ago by the American Pediatric Society,ll that L52 of the 340 cases analyzed showed symptoms of rickets, the question arises as to whether the diagnosis of rickets was not in many cases based on the presence of beading. It is evident, therefore, that the fact that beading of the ribs may be likewise of scorbutic origin has clinical and diagnostic significance. Unless we are certain that a baby has received an adeguate quantity of antiscorbutic food, \Ã/e are not justified in considering the enlargement of the costochondral junctions of rachitic origin. The rosary may, indeed, be of twofold nature, as these two nutritional disorders may, and f'requently do, exist concomitantly. This does not imply, howev'er, that "very possibly the same defect in diet which produced the one produced the other also," for we know that a small quantity of the fruit juices will prevent scurvy, but not rickets, and, on the other hand, that cod liver oil will generally prevent the development of rickets, but has no prophylactic value for scurvy. CONCLUSIONS Beading of the ribs, the so-called rachitic rosary, should not be regarded as pathognonronic of rickets. It occurs very frequently in connection with infantile scurvy and is one of the typical signs of this disorder, developing in the course of the disease, and disappearing rapidly with the recession of the other symptoms when an antiscorbutic foodstuff is given. This view is borne out by clinical observations olr man and on animals, as well as by postmortem examinations. The rigid conception of this phenomenon as attributable solely to rickets led to a confusion of infantile scurvy and rickets in the past, and today is responsible for the misinterpretation of many cases of latent scurvy. 10. Schoedel, J., and Nauwerck, C.: Untersuchungen über die MoellerBarlowsche Krankheit, Gustav Fischer, Jena, 1900. 11. American Pediatric Society Investigation: Arch. Pediat. 1õ:481, 1898. Downloaded From: http://archpedijamanetwork.com/ by a Penn State Milton S llershey Med Ctr User on 0512712015