Four hundred consecutive cases of jaundice

FOUR HUNDRED

CONSECUTIVE

CARL A. BACHHUBER

M.D.

AND

Los Angeles,

T

CASES OF JAUNDICE*

ALFRED

E.

GILBERT

M.D.

California

evaIuation of a good history and physica examination. CertainIy the daiIy observation of the stoo1 as to coIor and the observation of urine wiI1 immediateIy give one a clue as to the type of jaundice with which the patient is afhicted. It is sureIy quite disconcerting to be caIIed to see a patient with jaundice and see no daiIy observations recorded regarding the stoo1 and urine. The increase of the amount of biIe in the urine or the changing coIor of the stoo1 wiI1 afford pertinent information as to the advancement or retrogression of the etioIogic factor producing jaundice. After the icteric changes of the skin have advanced to a certain point visua1 observation will give no further information as to the amount of bile pigment present in the circuIation. And it is here that the icteric index or the serum bilirubinate determination is of some vaIue. NevertheIess, even at this point, the observation of the stoo1 and urine wiI1 stiI1 be of considerabIe diagnostic vaIue. The assumption that the icteric index continues to rise to a given IeveI as Iong as the stooIs are acholic is essentiaIIy correct. The changing coIor of the stoo1 wouId dehniteIy point to a fluctuating icteric index and wouId favor a diagnosis of Iithogenic disease in place of pancreatic neoplasm. The persistence of an acholic stoo1 is significant of obstructive jaundice, be it intra- or extrahepatic in type. The reIease of the obstructive Iesion wouId immediateIy manifest itseIf by a change in character of the urine and coIor of the stool, and wouId favor a diagnosis of Iithogenic, toxic or infectious type of jaundice in preference to a neopIastic type. The direct or indirect van den Bergh test has but Iittle practica1 appIication if the

ODAY there are two cIassifications of jaundice which have received widespread consideration, nameIy, those by Rich’ and McNee.2 Rich in his cIassihcation divides jaundice into the regurgitation and retention types whiIe McNee, whose cIassification is the more uses three divisions, wideIy accepted, namely, obstructive, toxic and infectious and hemoIytic. The Iatter has been criticized by Rich who states that the cIassihcation of McNee is based upon both cIinica1 and etioIogic factors using two standards within one His objection is rightIy cIassification. stated, but unti1 Rich’s classification receives more generaIized usage the one of McNee’s wiI1 remain. In accord with Rich’s classification is the one offered by Yater, in that he divides jaundice into the obstructive and hemoIytic groups. He further subdivides the obstructive group and _ into the intrahepatic extrahepatic groups thereby pIacing toxic and infectious iaundice into the intrahepatic obstructive group in which they rightfuhy beIong. This is aIso in accord with the teaching of Rich. The accumuIation of biIe pigments within the circuIation producing an icteric coIor of the scIera and skin may have various etioIogic factors. VoIumes have been written, numerous tests devised and stiI1 with the exception of a few basic accepted principIes the entire question is far from being soIved. On the differentia1 diagnosis of jaundice much also has been written and many tests have been devised, a11 of which have IittIe significant vaIue if the proper cIinica1 observation has been made. There are stiI1 no tests which wiI1 repIace the proper

* From the SurgicaI Service of the Los Angeles County General Hospital and Department Medical Evangelists, Los AngeIes, Calif. I44

of Surgery, ColIege of

VOL. LXXVI,

No. z

Bachhuber,

GiIbert-Jaundice

aforementioned observations have been diIigentIy carried out; for the achoIic stoo1 with choIuria and a cholic stoo1 with achoIuria wouId immediately place the icterus in one of the two main divisions. There are many other tests and whiIe a few are of some vaIue as confirmatory evidence, they all are usuaIIy found Iacking when one seeks aid in the finer differentia1 points. Since a11 the tests used for jaundice dea1 with Iiver function, the vaIue is greatIy depreciated by the fact that each test evaIuates but one hepatic function and since the Iiver possesses many functions the impairment of any one function is not necessariIy indicative of the function of the organ as a whole. Furthermore, the liver has marked regenerative powers and there must be considerabIe impairment of function present before it reflects itself in the Iaboratory anaIysis. One must also appreciate the fact that it is seIdom he is deaIing with one form of jaundice alone; for with the hemolytic type sooner or Iater hepatic impairment wiI1 become evident thereby compIicating what once was a hemoIytic jaundice with an intrahepatic obstructive or regurgitant type of jaundice. AIso an intrahepatic or obstructive type of jaundice wiI1 soon produce Iiver changes thereby compIicating the picture with a retention type of jaundice. Jaundice has also been cIassified into the painIess and painful type, the painfu1 group suggesting an inff ammatory or Iithogenic basis whiIe the painIess type presumes a neopIastic or toxic basis. WhiIe the presence or absence of pain is of some vaIue in differentia1 diagnosis, nevertheIess a study of patients suffering from icterus reveaIs a surprising number who die from painIess obstruction of the common duct due to a stone or, converseIy, those who because of pain were subjected to surgery in consequence of a diagnosis or a common duct stone onIy to find a maIignant Iesion in the pancreas. It is not unusua1 to find at surgery or at autopsy a common duct stone as a cause of jaundice which had previousIy been

American

Journal

of Surgery

‘45

diagnosed carcinoma of the head of the converseIy, a so-caIIed common pancreas; duct stone frequentIy proves to be a carcinoma of the head of the pancreas. Since this error is of frequent occurrence, it behooves one to perform an exploratory laparotomy in an effort to definiteIy determine the etioIogic factor of the jaundice and to ameIiorate or remove the fundamenta1 cause of the affection if possible. In this particuIar study we have reviewed the records of 400 consecutive patients suffering from jaundice as taken from the fiIes of the Los Angeles County Genera1 HospitaI. WhiIe a review of this type wiI1 produce statistics which are of some vaIue in drawing concIusions, one must nevertheless be aware of the fact that due to the numerous uncontroIIabIe variabIes encountered, nameIy, the failure to defmiteIy estabIish a11 diagnosis through surgery or necropsy, we must be somewhat hesitant to accept the concIusions as being absoIuteIy correct. We must also recognize that diagnostic abiIity, even that of our best diagnostic institutions, is far from what might be desired. Furthermore, the Iarge group of patients who have recovered and been discharged as having had a certain type of jaundice is no assurance that the diagnosis was correct. However, we are safe in assuming that patients so discharged as cured or improved were in their proper category as we11 as those who were dismissed in an unimproved condition and who showed a steady downhi course. But it must aIso be remembered that a11 patients with jaundice are not hospitaIized. In certain groups, nameIy, the Iithogenic and neopIastic of the pancreas, sufficient patients were subjected to surgery or necropsy to correctIy estabIish the diagnosis in a very high percentage of cases. In presenting the foIIowing tabIes we have divided the four hundred patients into seven groups. (TabIe I.) WhiIe we reaIize that the cIass&cation is far from idea1 and certain rearrangement might be advisabIe, nevertheIess, it at Ieast presents

146

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JournaI

of Surgery

Bachhuber,

GiIbert-Jaundice

a working basis for presentation of the statistics. In reviewing the group as a whoIe it immediateIy caIIs forth the observation that an individua1 who becomes jaundiced has approximateIy three and one-haIf of TABLE

Awusr,

1948

mova1 of the gaIIbIadder with its contained stones before the onset of maIignancy wouId spare a high percentage of these patients. It is the onIy procedure which wouId give permanent reIief for a11 other surgica1 procedures are onIy paIIiative in character.

I

TABLE II MALIGNANCY

-

Malignancy. Common duct stone Inflammatory. HemoIytic PortaI cirrhosis. Unknown. . Stricture. .

Total

Per Cent

x75* 86

43.75 21.50 16.00

Total

64 38 20 II 6 400

9.50 5.00 2.75 I.50

100.00

* IncIudes two patients with Hodgkin’s disease.

ten chances to recover. For as we appraise the mortahty of the various groups it is quite apparent that the inffammatory group, which incIudes catarrha1 jaundice, offers the patient the best opportunity to recover but in the remaining groups the mortaIity graduaIIy increases, reaching a IOO per cent in the maIignant group. Mulignancy. In this group we have one hundred seventy-five patients who suffered from maIignancy, or 43.75 per cent of the tota1. This immediateIy brings the patient’s chance of recovery to a 5.5 to 4.5 basis, or a IittIe better than an equa1 break. (TabIe II.) Carcinoma of the head of the pancreas Ieads the Iist as a cause of jaundice and accounts for approximateIy 25 per cent of cases, or about one in four. CoupIed with the percentage of common duct stones, which is second in the Iist as a cause of jaundice with a tota of 21.50 per cent, these two groups are responsibIe for approximateIy one-haIf of the jaundiced patients. (TabIe III.) Neither medicine nor surgery has much to offer the malignant group. The presence of gaIIstones in a high percentage of maIignant gaIIbIadders offers a surgica1 incentive for reduction of the mortaIity for the re-

Carcinoma of head of the pantreas. . . . . . . . . Carcinoma of biIiary tract. GaIlbIadder ducts and papilla of Vater.. .. . . Metatastic Carcinoma to liver Pressure on duct (two Hodgkin’s disease). . . Carcinoma of duodenum.

Per Per Zent of Cent Maligof nancy TotaI

94

53.7’

36 32

20.57 18.29

9.00 8.00

I2

6.86

3.00

I

.57 --

.25 ---

100.00

43.75

--

175

23.50

i TABLE

III

Per Cent of TotaI

Common duct stone..

..

21.50

In tweIve instances in the maIignant group jaundice was ascribed to pressure on the biIiary ducts by a maIignant Iesion incIuding two cases of Hodgkin’s disease. WhiIe this group represents the tota in which extrahepatic ducta pressure produced jaundice, we must recognize the fact that the remaining number represent intrahepatic ducta pressure. It is generaIIy conceded today that maIignancy neither primary nor secondary wiI1 produce jaundice without pressure on the intra-or extrahepatic ducts. AI1 of us no doubt have seen necropsies at which aImost the entire Iiver was repIaced by carcinomatous tissue and stiI1 no jaundice ensued. Carcinoma of the head of the pancreas if present can expect IittIe but paIIiative care. WhiIe it is true that in recent years surgica1

VOL.LXXVI, No. 2

Bachhuber,

GiIbert-Jaundice

procedures have been devised for the remova1 of pancreatic malignancies, &II the outcome at present, at least, is very disappointing. However, it is beIieved that with improvement of diagnostic means and surgica1 technic the outIook for these patients may become more hopefu1. Common Duct Stone. In the second group we are deaIing with common duct stone. This group accounts for more than one-fifth of the entire series. From a medica and surgica1 standpoint it is groups two, three, four and six which offer the patient a IittIe more than paIIiation, but in the inflammatory group, be it Iithogenie or nonIithogenic, and the hemoIytic group the patient can derive medica or surgica1 reIief. However, surgery for common duct stone in the presence of jaundice carries a high mortaIity. These patients usuaIIy are in the eIderIy age groups, frequentIy suffering from some concomitant disease, who have done nothing over the period of years to aIIeviate their suffering from gaIIbIadder disease unti1 jaundice appeared. FrequentIy these patients have been toId, even when aware of the presence of stones, that surgica1 remova is not indicated unIess they are subject to recurrent attacks of colic or inff ammation. There is no doubt that gafIstones shouId be removed when first discovered. This appIies equaIIy to the gaIIbIadder with the solitary or so-caIIed siIent stone as we11 as to the gaIIbIadder f3Ied with numerous pigmented or choIestero1 stones with recurrent attacks of acute ChoIeIithiasis and choIecystitis. WhiIe it is true that the soIitary type of stone usuaIIy is evidence against a common duct stone, nevertheIess this is a vicious type of stone which undoubtedIy is responsible for many of the serious cases of jaundice due to ascending choIangitis and, incidentaIIy, to many perforations. InfEammatory UndoubtedIy the most hopefu1 group in this series is the group suffering from catarrha1 jaundice for here the recoverv Y is IOO A Der cent. (TabIe IV.‘, . ~I

AmericanJournaIof Surgery

I47

Since there were no deaths and no surgeries, diagnosis rests soIeIy on cIinica1 observation and obviousIy upon the fact that the patient recovered. This disease is seIdom found in the patient past thirty; it is usuaIIy in the twenty to thirty year TABLE w

No.

Per Cent

Per Cent of TotaI

48.44

7.75

25.00

4.00 3.00

ICatarrhal jaundice. Cholaneitis. :. non-calculous. calculous. Weil’s disease. Inflammatory edema. Chronic pancreatitis. Lues .

3I 28 16 I2 2

I I I

18.75 3.13 1.56 1.56 1.56

.5o .25 .2$ .25

group. One must be rather reIuctant to make this diagnosis in an individua1 past fifty years of age. AI1 patients in this group but two were beIow thirty-two years of age. The diagnosis of this type of jaundice is usuaIIy readiIy estabIished with a fair dewithout surgica1 or gree of certainty, autopsy findings. The type of onset, age group, rapid course, miId gastrointestina1 symptoms and the earIy recovery of the patient is the picture ascribed to catarrha1 jaundice. FrequentIy time pIays an essentia1 part in estabIishing diagnosis. ChoIangitis, which is usuaIIy secondary to an inflammatory Iesion of the extrahepatic biIiary system, either caIcuIous or non-caIcuIous, comprised 43.75 per cent of this group or 7 per cent of the tota1. It is in this group, of course, that surgery is at its best, offering the patient a cure with but a Iow surgica1 mortality. Its presence can usuaIIy be diagnosed with a fair degree of accuracy because of the history of gaIIbIadder disease or repeated attacks of biIiary coIic accompanied with a tender, upper right quadrant, temperature, chiIIs and Ieukocytosis. If the patient is cIoseIy watched and surgery instituted at the proper time, Iow mortaIity wiI1 be encountered. The best prophyIaxis, however, is remova of the gaIIbIadder and its con-

4

Bachhuber,

American Journal of Surgery

Gilbert-Jaundice

tained stones at the proper time when the diagnosis is first estabhshed. Two patients who suffered from WeiI’s disease and who recovered, one with inAammatory edema in which necropsy estabhshed the diagnosis, one suffering TABLE TOXIC AND

-

/ No.

Hemolytic ....... : ...... acquired. ............. congenita1. ........... Toxic ..................

arsphenamine......... phosphates. .......... sulfonamides .......... Transfusion ............. Massive pulmonary hemorrhage ...............

and dosage. It is quite IikeIy that with but few exceptions this type of jaundice wiI1 become Iess frequent. However, the question of permanent Iiver damage due to an overdose of an arsenical is always a possibility.

v

HEMOLYTIC

23

I0

AUGUST, 1948

TABLE VI PORTAL CIRRHOSIS

ICTERUS

Per Cent

Per Cent of TotaI

60.53

9.50

Portal cirrhosis..

NO.

Per Cent

20

$00

of Total

2.50

13 12

31.58

3.25 3.00

4 I 7

2 1

5.26

.5o

2.63

.25

L

from chronic pancreatitis in which the diagnosis was estabhshed surgicaIIy and one patient with Iuetic jaundice comprise the remainder of this group. Toxic and Hemolytic Icterus. Toxic and hemolytic icterus composed thirty-eight of the tota1, or 9.50 per cent. (TabIe v.) This probabIy represents the next most hopefu1 groups because a high percentage of these patients can be given medica or surgica1 reIief or cure. It is we11 known that in congenita1 hemoIytic icterus spIenectomy wiI1 stem the progress of the disease. In the acquired type of hemoIytic icterus the etioIogic factor wiI1 determine the patient’s possibiIity of receiving either medical or surgica1 aid. In approximateIy 50 per cent of these patients the diagnosis was confirmed by autopsy or surgery. With the advent of suIphonamides, one must always be on the Iookout for a toxic reaction for the possibiIity of the icterus being on a toxic basis does exist. But the uItimate outcome, if the drug is promptIy discontinued and medica measures instituted, is quite satisfactory. Arsphenamine reactions, as far as icterus is concerned, are seen Iess and Iess frequentIy because of improvement of technic

In spite of the improvement of technic an occasiona reaction from bIood transfusions wiI1 occur. But with the advent of a wider knowIedge of the Rh factor and proper grouping of the bIood and cross matching, it is quite IikeIy the icterus resuiting from transfusion wiI1 rareIy occur. As a whoIe the outIook for a patient suffering from the toxic or hemoIytic type of jaundice is quite encouraging. Portal Cirrhosis. PortaI cirrhosis represents 5 per cent of the tota of the four hundred case histories reviewed. (TabIe VI.) Just why some patients suffering from this disease shouId become jaundiced and others shouId not remains a mystery. There is aIso some question as to the type of jaundice porta cirrhosis represents for the etioIogic factor being unknown it is quite IikeIy that it may be a mixed type of jaundice when present. The most accepted theory is that it is a nutritiona deficiency secondary to aIcoho1 or some unknown toxin. However, there are patients suffering from porta cirrhosis who have never used aIcohoIic beverages in any form; consequentIy, some other toxin beside aIcoho1 must be considered as an etioIogic factor. Suffice to say that the outIook for the group is poor and it is a question of only a few years when a11 wiI1 succumb to the disease. In the unknown group there Unknown. were eIeven, or 2.75 per cent of the tota1. (TabIe VII.) In reaIity this should be a

VOL. LXXVI, No. 2

Bachhuber,

Giibert-Jaundice

much Iarger group for frequentIy diagnosis was estabhshed for statistica purposes when a diagnosis of unknown etioIogy wouId have been more feasibIe. NaturaIIy the patients that recover care IittIe if the proper diagnosis has been estabIished.

__

149

TABLE VIII STRICTURES

TABLE VII

..

~~~~~~~ of surgery

it behooves one to retrograde manner, proceed with caution or stiI1 better remove the gaIIbIadder from the fundus to the duct. As Iong as the bIood suppIy to the viscus has not been interrupted it may stiI1 be

UNKNOWN

Unknown..

American

No.

Per Cent of TotaI

II

2.75

On the other hand, autopsies in a11 deaths and surgica1 expIoration if indicated, if the patient is wiIIing, wouId probabIy bring many of these cases within their proper classrfication. Strictures. The fina group, nameIy, those of stricture again represent rather a hopeIess picture. (TabIe VIII.) NaturaIIy a congenita1 stricture of the newborn means a mortality of IOO per cent. As to the acquired type the surgeon wiI1 have to assume the entire responsibiIity for the mortaIity in this group. The presence of a stricture which is not promptI? repaired wiI1 Iead to cirrhotic changes rn the liver which definiteIy contributes to the mortality. Th e patients are usuaIIy poor surgica1 risks; the secondary surgica1 procedures consume considerabIe time, Ieaving the patient in an extremeIy precarious condition postoperatively. This type of compIication usuaIIy can be avoided. The proper exposure of an anatomic structure and careful Iigating or severing of same wiI1 avoid most of these accidents. Since most accidents occur in removing the gaIIbIadder in a

No.

Congenital. Acquired.

I 5

Per Cent of Total

0.25 1.25

used for an anastomotic procedure, but with a prehminary Iigation of the cystic artery the viscus must be removed for necrosis and gangrene are sure to foIIow. SUMMARY

A patient who becomes jaundiced, regardless of age, has approximateIy three and one-haIf chances of ten to recover. If the jaundice occurs in an individua1 who has reached middIe age, his possibIe percentage of recovery is stiI1 smaIIer. RemovaI of a gaIIbIadder and its contained stones at the proper time after diagnosis has been established wiI1 reduce the number of deaths due to common duct stones, malignancy of the gaIIbIadder and the inflammatory group due to caIcuIous disease of the viscus. REFERENCES I. KICH, A. R. The pathogenesis of the forms of jaundice. Bull. Jobns Hopkins Hosp., 47: 338, 1930. 2. MCNEE, J. W. Quart. J. Med., 16: 390, 1923. 3. YATER, W. M. Fundamentals of Internal Medicine. 2nd ed., p. 398. New York, 1944. D. AppIetonCentury Company.