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Polycystic Kidney Disease1
POLYCYSTIC KIDNEY DISEASE 1 R. E. OUJ\1JHIXG Detro1:t, Mfrhigan
Investigation :ctnd therapy dealing with vital organs already handicapped by developmental defects, at once arm the clinician with wariness, and intrigue him with possibilities for the benefit of a certainly hopeless infirmity. In patients with polycystic kidneys it may be said "he is as old as his cyst-development." It seems definitely proven that when the cyst development (or cystic degeneration) pursues its course to a certain point, Jife as dependent upon kidney function, is no longer possible, One might speak of polycystic kidneys as growing ripe, referring to the stage, at ·which oftentimes the disease is first recognized, the beginning of a fatal decline, manifested by symptoms comparable to nephritic downward progress, renal dysfunction, uremia, anuria, death. As can be readily presupposed, the margin of safety with polycystic kidney disease, is narrow to a degree parallel with the advancement of degeneration, and always narrower than in kidney pathology engrafted upon congenitally healthy organs. With no presupposition of this disease in a given case, one can readily err both in diagnostic methods and prognostic acumen. Post-mortem examinations shmv that in many instances> the polycystic kidney is not suspected in life, death being due to other causes, ·while the kidneys have carried on satisfactorily. But this fact should not disarm one, since age is no criterion of the cystic formation stage, as completely advanced instances of the disease are found at birth and in extreme old age. Certain diagnostic and inYestigati-ve procedures familiar to the urologist, serve not only to pro Ye the presence of the disease we 1 Read at. the annual meeting of the J .. mcrican Urological Association, Baltimore. May, 1927.
140 'J'HE JOPRX.AL OF rJ--tOI,OGY, VOL. XIX, >fO.
?
150
R. E. CUMMING
are discussing, but as means of improving the function of the failing kidneys, thereby possibly delaying the consummation of the disease and ultimate death. We refer particularly to cystoscopy and kidney pelvis drainage, pyelography for diagnosis, and in rare instance nephrotomy for drainage of grossly infected cyst areas. All of these maneuvers require the utmost care and good judgment; simultaneous catheterization of both ureters being dangerous and bilateral pyelography prohibitive. Such radical steps as nephrectomy, removal of the more advanced and (presumably) the pyonephrotic kidney, are likely never of value. Differential diagnosis becomes of more importance, the further one advances in a consideration of the proper care of the polycystic kidney types; the latter word is used deliberately, for although we have outlined generalizations to which all cases conform, certain occasions arise wherein with acute illness intervening, a newly-discovered lateral abdominal mass may demand accurate study with no definite lead to renal involvement, or to false surgical measures, sufficient in themselves to prove of lethal consequence. In the suggested dilemma, medical attendant and surgeon alike, quite naturally call upon the urologist for aid and depend upon him for accurate information relative to retroperitoneal pathology. Naturally too, a mass in this position, with none on the opposite side, the fellow kidney being less advanced cystically, may as readily prove to be a new growth, an abscess, or more commonly a huge hydronephrotic kidney. The physical examination may be entirely ineffective, due to tenderness and muscular defense. Furthermore the ureteral catheter may, by meeting obstruction, fail to enter such a kidney mass, so that the contents cannot be studied, and pyelography is impossible. Ordinarily, however, roentgenographic studies readily differentiate tumor, hydronephrosis and polycystic disease. Unless one is thoroughly familiar with the gross appearance of polycystic kidneys, he will occasionally be perplexed, meeting at the operating table, that large cystic hydronephrotic organ which in the gross has the size and outline of true cystic disease, and is in a sense, a polycystic kidney although the simple result of obstruction and infection which has advanced to complete de-
POLYCYSTIC KIDXEY DISEASE
151
struction of the organ, leaving no functioning renal tissue and filled with stale urine. In such a predicament it is well to remember that the pelvis of a polycystic kidney is rarely dilated; it is usually narrowed and lengthened, ,vhile that of a hydronephrotic kidney is most often dilated. Again, the cysts of polycystic kidneys are closed while
FIG.
L
Bn.ATJ
PY],JLOGRAM,
CASE
7,
TYPICAL OF PoLYCYSTIC DISEASE,
PYI0LOGJUPHY PRJ"CIPI'l'ATED RAPID DECLINE AND DEATH
See case history
the pseudo-cysts of hydronephrotic kidneys are in communication with one another and the renal pelvis. Aspiration will readily differentiate the two conditions. Pyelographic evidence follows the same rule, the media :filling the cyst areas in one and partially surrounding them in the other. The foregoing paragraphs suggest a further consideration of the pathology invoh·ed in polycystic kidney disease, and we find
152
R. E. CUMMING
divergent oprn10ns in the literature and in the utterances of present-day pathologists. It is conceded that of all renal anomalies, the polycystic kidney is the most important. The fact as already intimated, that one organ may show no gross evidence of the disease, does not disprove the theory that the condition is always bilateral. In many instances, Davis has found in cases presenting on the one side advanced cystic degeneration and on the other a grossly normal kidney (as to cyst formation), that this other organ presented under the microscope, unmistakable evidence of early cysts. Other observers have proven this same contention, and, like Davis, have pointed out the tendency to deformities in the skeleton and in other organs, notably the liver. In one of our own cases, the patient was acromegalic and presented a definite deformity of the sternum, in two other cases the spleen was cystic, in many there were liver cysts. Various other congenital deformities were noted. Carefully reviewing the literature one also finds varying views on the anatomic development of polycystic kidneys, and similarly divergent expressions of opinion, concerning etiology. In explanation of this, Davis blames the scarcity of material and the failure of a coordination of ideas, and undertakes, by means of a laborious study of many specimens, to reach a final stage of knowledge relating to the etiological tendencies. Following his work, we are convinced his conclusions will go far to explain logically and satisfactorily, this pathological problem. As to others' theories, a brief resume of those most seriously considered follows: 1. Virchow 's theory was that the cysts are due to inflammation of papillae with closure of their ducts. This idea was abandoned when it was proven that obstruction of the papillary ducts was followed by atrophy of the tubules rather than cystic changes. 2. Brigidi and Severi described "epithelial nests" which degenerated into cysts. This theory of neoplastic development has also been discarded. 3. Hildebrandt's conclusions were based on our knowledge of two separate embryological structures which unite to form the complete kidney; the cortex and major part of the medulla, con-
POLYCYSTIC Krm,,EY DISEASE
153
taining the convoluted tubules and loops of Henle, arising from the nephrogemc tissue of the Wolffian body while the collectiug tubules and peh is are a separate formation from the 'vVolffian duct. Hildebrandt believed that failure of union on the part of these separate strnctures produced cysts at the time secretory actiYity of the glomerulus and tubule should normally begin. ,VhiJe this is the generally accepted theory Yon Mutach and later Bunting, haYe shovm that a direct continuity can be traced between cysts and tubules and Berner found carmine in cysts :1fter injection of the uretero Greene, isolating tubules, found direct continuity between cysts and collecting tubules and so proYes that retention alone does not explain cyst formation. Earlier ,vriters state that the congenital polycystic kidney occurs in all domestic animals, "its origin accredited to cessation of grnwth, with or without union of convoluted and collecting tubules. n Davis' own conclusions would tend to the assumption that human polycystic kidneys result from a partial cessation of development at the mesonephric stage, degenerative changes coming later to complete the picture of interference ,vith the norrnal kidney function. He compares the human polycystic kidney to the normal kidney of the codfish (and other lower forms) and findt' distinct similarities which are most significant since in these lower animals, the full-grown kidney is of the mesonephric type. In DaYis' series of fourteen completely studied cases, he found universal evidence of progressive characteristics and of more importance to the clinical worker, a ''marked tendency to hemorrhage and infection . " This was especially noticed in the adult specimens. The gross material shmved the predominating posit-ion of the majority of the well-formed cysts to be in the cortical and subcortica1 zones, and the oldest and largest formations near the fusion line of the lobules. In all cases of advanced cyst forrna-· ti.on, the lessened renal tissue was accompanied by a ma10sive increase in kidney bulk. Case 24 of this series is an exception to this ruleo Other well recognized gross changes include the irregular surface contour, serrations of concaTe and convex areas correspond0
154
R. E. ClT'.\E'vfI~G
ing to the underlying cysts, the walls of many being almost transparent. The fatty capsule is scarcely recognized, blending with an adherent, toughened and stretched out fibrous capsule. The fluid contained in the cysts varies considerably, but is more serous in type than the retained urine of hydronephrosis. It may have a large solid content derived from the urine salts, blood, tissue degeneration and the coincident chemical reactions upon these elements. We found the fluid in all polycystic kidneys of adult life, to contain blood and pus, evidence of infection and hemorrhage, and Braasch, reviewing those cases observed at the Mayo Clinic, stated that in over 40 per cent of the cases, hematuria was a definite symptom. This latter is commonly overlooked in our consideration of hematuria even in obscure clinical problems. It is perhaps due to the rather uniform gross and microscopic picture, overshadowed with secondary changes (hemorrhage and infection), which the adult kidney presents, that an earlier accurate estimate of etiological possibilities has not been satisfactorily made. Davis hit upon the plan of studying young specimens, especially in early stages (in a fetus) in order to escape the changes mentioned. It is particularly from his study of a serially-sectioned polycystic kidney of a full-term fetus that he has made his admirable and probably correct analogy between polycystic types and the embryological mesonephros. Some significant features of the evidence of developmental halting are the facts that the renal cortex and medulla are never fully demarcated, the latter being recognized in early specimens only by slight indentures of the calyces. Davis states that "the regular assembling of the pyramids with the intervening columns of convoluted loops is never complete in congenital polycystic kidney." In young specimens we found glomeruli in the capsular area, with cysts of Bowman's capsules, as if individual glomeruli had merely dropped out. This disproves the old contention that cysts are confined to the tubular zones. It is not the purpose of this article to deal further with the microscopic anatomy of our subject, itself an intricate and as yet unravelled problem. We do feel however, that one must con-
POLYCYS'I'IC KIDNlsW DISEASE
stantly -investigate the causative factors of any entity met in the clinical sense in order to have a foundation for logical conclusions. Our own conclusions are that the etiologic factors in congenital polycystic kidneys are: L Inherited protoplasmic insufficiency specifically expressed in the differentiation of the m.ost complicated tubular organic structures (kidney and liver), CHART'
L
HEirnDIT'Y RECORD OF CASE
'7
il·faternal gran clmother Died at age 53 Polycystic kidney disease
I I
r--
- - - - - \ - - - - - - - - - - -1 I ! 1 Mother Aunt Uncle Died at age 40--50 Die,l at age 45 Died at age 48 Large bilateral poly., Polyc::•.rstic Polyc:vstic kidney disease kidney disease cystic kidneys
i I
Case?' Died a,t age 32 Polyc:ystic kidney disease See also pyelogrnms (fig. 1), and photograph of specimens (fig. 1'.2). In this family there have been five additional instances of polycystic kidney diseasn, incompietely 1·eccirdeu.
2, This insufficiency is manifested by delayed differentiation of cellular unit-structures. 3, The morphologic eYidence of this structural delay is identi-cal at all ages 1 and is strikingly illustrated in sections s1mwing undifferentiated stroma (embryonic type) containing aggregations of cells which at one le,·el have a loose arrangement, at another form a solid mass or column> at :still another show a dis-ti.net lumen and finally merge into a cyst. The hereditary aspect of polycystic kidney disease haB been widely commented upon; this to such an extent that a familial history 1s eonsidered of the greatest diagnostic value. As many
156
R. E. CUMMING
as fourteen instances in one family are reported, and in our own series, ten individuals of close blood relationship proved to be CHART
2.
HEREDITY RECORD OF CASE
24
Paternal grandfather Died of old age
Paternal grandmother Died of old age
Maternal grandfather Died of old age
I
I
I
L _ _ T _ _ _J
I Father Drowned at age 44
'---T I
Maternal grandmother Died at age ? Polycystic kidney disease I
Mother Died at age 42 Had several operations for renal tumors including a nephrectomy after which she lived one year
L--------------~
r
I
Brother Died at age 43 Bilateral renal tumors
Sister Living at age 38 Has kidney trouble similar to case 24 associated with hypertension
Case 24 Died at age 36 Bilateral polycystic kidneys (Left kidney atrophic and polycystic) (Right kidney polycystic) See also diagnostic pyelogram (fig. 2), post-mortem pyelo-uretero-cystogram (fig. 3), post-mortem specimens (fig. 10), and dra,ving (fig. 11), depicting complete pathology.
victims of the disease. Whether the congenitality indicates direct and true hereditary transmission and whether both male and female progenitors are likely to be followed by the polycystic
POLYCYSTIC KIDKE1' DISEASE
., r,::1.l,)
I
kidney tendency, are questions we can ooly mention to leave for further study.
FIG. 2. PYELOGRAM lW RIGHT KIDNEY, CASE
N·ote hydronephrosis.
24,
l\il.ADJ<; AT DIAGNOSTIC STUDY,
Left u_reter did not connect ·,Nith bladder"
1925
See
figure 11 .
The recognition of several instances of polycystic kidney disease in one family is largely responsible for the term. "cofr genital, n now properly applied in view of the frequency with
158
R. E. CUMMING
which it is found in feti and the newborn. Coupled with this, it seems incongruous to observe the same tissue changes in the organs of a fetus and of an individual attaining old age; but in the one case the degenerative processes rapidly replaced tissue capable of functioning as human kidney whereas in the other, a
Fm. 3.
POST-MORTEM PYELO-URETJmO-CYSTOGRAM, CASE
24
~ote filling of large cysts in right kidney, accomplished by extreme pressure from syringe in pelvis. At autopsy right pelvis and ureter were enormously distended with blood clot.
single collection of tubules show both functionating parts and fully-formed cysts. Tubules develop to the stage of functioning properly while contiguous areas in varying speeds present the typical cystic changes. Returning to the clinical aspect of our study let us consider very briefly the diagnostic signs and discuss the methods of treatment.
159
'.!:'OLYCYSTIC KIDNEY DISEASE
Symptoms: Pain, hematuria, albuminuria, These are common but inconstant and only apply to those cases clinically recognized; in addition one may find any symptom associated with renal disease.
Fm . 4 Frn. 4.
Bn,A'l'ERAL PYELOGRAM,
Fm. 5 CAS:E:
31.
CONDITION NOT PEVIOUSLY SUSPECTED. TIAL NEPHRITIS.
Fm, 5.
LEFT
EXCELLENT RENAL Ft>NCTION PRESERVlm
PYELOGRAM,
ATYPICAL REPRESENTA'!.'ION, TO COB.TEX,
BILATERAL PoLYCYSTIC KIDN:8YS. FINDINGS OF CnrwNIC INTE;RSTI-
CA:sE
29.
BILATERAL
PROBABLY CYSTS ARE
PoLYcvsnc
KIDNEYS,
CONFINl
PATIENT HAS SYMPTOMS OF CHRONIC INTEH.STITIAL .°\JcPHRI'l'IS
Physical signs: Palpable turn.or, unilateral or bilateral. Fluctuation is absent. In patients past fifty, arteriosclerotic changes, hypertension, with their attendant signs, are frequently observed. Diagnostic aids: Pyelography usually typifies the condition, the pelvis is elongated, ,;vithout much dilatation. Many of the
160
R. E. CUMMING
calyces are stretched over the cysts, and converted into convex slits. The urine is abundant and of low specific gravity. vVhen
FIG. 7
FIG. 6 FIG.
6.
RIGHT
PYELOGRAM,
CASE
29.
HYDRONEPHROSIS
DEMONSTRATED,
BILATERAL POLYCYSTIC KIDNEYS
See figure 5. FIG. 7.
RIGHT PYELOGRAM, CASE
(Courtesy Dr. Ormond) 28.
BILATERAL POLYCYSTIC KIDNEY DISEASE.
PYELOGRAM NOT DIAGNOSTIC, INDICATING ONLY A LARGE KIDNEY. OTHER SIGNS TYPICAL OF PROGRESSIVE DISEASE
(Courtesy Dr. MacArthur)
POLYCYSTIC KID:N"EY DISEASE
161
renal insufficiency becomes marked, any or all symptoms of uremia may be presento The several clinical types usually described are but transitions of one another; the slowly advancing case parallels chronic inter., stitial nephritis and may continue for years 1 while at any period
FIG.
.FIG. 8.
PYELOGRAM,
.FIG. 9
8 RIGHT
KIDNEY.
BILATERAL I'OLYCYS'l'IC KIDNEYS.
OUTLINKS MANY VARYING SIZED CYSTS.
lVlEDJA
TYPICAL Ni,RROWED p,~LVIS.
lJRI
(Courtesy Dr. Lowsley)
J:<'IG. 9.
PYELOGRAM, RIGHT KrnNEY.
~vl01rn
BILATERAL PoLYCYSTIC .KIDNEY DISEASE,
ADVANCED ON RIGHT SIDE,.
SuGG8STS HYDRONEPHR0SIS
(Courtesy Dr. Lowsley)
it may, on the other hand merge into the uremic type, with fatal outcome in a few days, or, as is particularly noted by us, any given case may become surgical with pain, hematuria and pyuria. ·with these latter symptoms, according to the treatment and results, a given case returns to the chronic form or gradually
162
R. E. CUMMING
presents grave uremia, frequently having several periods of infection. Treatment: This is largely medical; conservatism is ideal. Dietetic advice and caution as to exercise, physical or mental excesses) exposure, are all important. Repeated and regularly TABLE 1
Collected data indicating wide variations of renal function and suggesting progressive nature a/disease Functional test variations CASE ~UMBER
DYE
INTERVAL
per cent
16 18 19 20 24
DYE
INTERVAL
per cent
61 0 0 5 0
90 5 5 4 8
days days days days days
65 0 0 20 0
DYE
per cent
3 days
0
11 days 2 days
43 0
Total non-protein nitrogen variations CASE NUMBER
N2
INTERVAL
mym.
16 18 19 20 24
29 32 .4 54.5 53 120
N,
INTERVAL
mgm.
I
6 8 5 4 8
months days days days days
31.4 60.3 94 38 172
N2 mgm.
11 days 2 days
29 122
Late data on case 20 February 15 , 1927 { Total non-protein nitrogen ... _................. 54 mgm. Total phenolsulphonephthalem ................ 25 per cent April 25, 1927
{ Total non-protein nitrogen .................... 43 mgm. Total phenolsulphonephthalein ................ 38 per cent
timed examinations, are wise and for increasing gravity of symptoms, surgical procedures must be considered. Brin reported nephrectomy in 17 cases and condemned it. N ephrectomy apparently hastens cystic degeneration in the opposite kidney. In 22 cases, nephrotomy was performed with 31 per cent early mortality and only two patients alive after two
163
POL YCYSTIC KID~EY DISEASE
years. Rovsing advised evacuation of cysts; this has been of value in at least one reported case, its basis depending upon lessened intrarenal pressure with consequent gain in function. Combined with this operation, one might readily drain an infected pelvis and possibly establish a nephrostomy. 17Vhile theoreticaJly) ff\'acuation of as many cysts as can be reached by surgical exposure of one, or both kidneys, seems of advantage, a study of many gross specimens shows how futile such operative efforts TABLE 2
Record of cliagnoslic and therapeutic cysto,,copy CJ,_SE NUI\JBER
7
CYS'rOSCOPIES
INCIDENTAL TRE .-'.._Tl\lE::-.IT
3
Inlying catheter
2
Opern,tion (explorntio,1); transfusion
l5 16 ]7 l8
HI 20 22 23 24,
2
26
1
27
l
')
•J
Operation for stone ~ ephrotomy (death) Transfusion
29
Nephrotomy
30 31
Total cystoscopies ... Diagnostic cystoscopies.. . . . . . .. Therapeutic cystoscopies ....
26 15 11
would prove, since the cysts occupy the entire bulk of the kidney" As late writers advise the so-called Rovsing operation, we would plead a more accurate record of results obtained, Further discussion of nephrectomy is scarcely necessary; our own case reports are typical of this phase of the subject; when one kidney is removed an extra, load on the opposite organ again causes what we may term, a physiological crisis, forcing any differentiation from normal to be more eYident, with resulting
164
R. E. CUMMING
diminution of function. Other involved organs, such as the liver, are simultaneously weakened, tending to dysfunction. All malformed tissue displays a greater tendency to succumb to the vicissitudes of life, so one may say that the potentiality of protoplasm varies in proportion to its defects. Repeated pelvic drainage and lavage have often been of value, and in our hands have relieved acute symptoms repeatedly. Catheter drainage and the cautious use of weak solutions of silver nitrate, have invariably checked a stubborn hematuria, and this drug may be of additional value since in all cases there has been coincident infection. Other considerations of importance along with the surgical manipulations, which are of distinctive value if properly chosen are: 1. Absolute rest in bed 2. Restriction of diet, allowing free fluids 3. Intensive accessory elinination (this type of patient tends to sluggishness of intestinal tract and poor sweating) 4. Selective use of drugs 5. Avoidance of general anesthetics; polycystic kidney disease affords an ideal indication for local or regional anesthesia, where surgery is necessary
Our clinical observations are based upon a total of 31 cases; of these I have studied the surgical or autopsy material in 27 cases. For data upon the remaining 4 cases I am indebted to Dr. 0. S. Lowsley. It is my purpose here to give in briefest outline, a clinical record of selected cases, with a summary relative to age incidence, multiple physical defects and possible surgical results. Case 7. Adult male, aged thirty-two years. Bilateral polycystic kidney, acromegaly, deformed sternum, cystic liver. Kidneys obtained at autopsy; right kidney 20.5 cm. in length, weight 760 grams. Left kidney 23 cm. in length, weight 1140 grams. There is a history in his generation of four relatives with congenital cystic kidney, and a total of IO cases in three generations. Renal disease recognized in childhood (albuminuria). Under medical supervision since fourteen years of age; active with no serious illness until ten weeks prior to his death. At
POLYCYSTIC KIDNEY DISEASE
165
that time severe pain began in left kidney. Bilateral pyelography and complete renal studies made. Extremely active colon bacillus infection with pyuria found involving both kidneys. Prior to pyelography, fair renal function; rapid decrease afterward without an increase in infection which was controlled by catheter drainage and pelvic lavage. Death due to uremia. Repeated urine studies showed a constant marked albuminuria, inconstant casts, low specific gravity and urea nitrogen output, many pus cells and motile bacteria. Case 18. Female child, aged fourteen. Bilateral polycystic kidneys, cystic spleen. Patient under-sized; treated for nephritis since six years of age. Onset of acute symptoms nine weeks prior to death. Pain in left kidney area for eight months preceding last illness. During that period polyuria, urine showing albumin constantly, with low specific gravity. Admitted to hospital she -;ly after onset of acute symptoms of fever, malaise, pain in left kidney area. Examination : Extreme anemia, bilateral large palpabie kidneys, left very tender. Marked pyuria, polyuria, albuminuria. Treatment: Blood transfusion, kidney pelvic lavage and drainage (col~n bacillus infection found in both kidneys). Repeated drainage at four day intervals. Exploratory operation five days later, left kidney, due to extreme pain, leucocytosis, pyrexia, and an increasing tendency to urinary suppression. Operative findings: Polycystic left kidney and spleen. Several large renal cysts on aspiration contained bloody fluid . Postoperative course : death, occurring after five days with uremia, coma, urinary suppression, constant emes1s. In case 18, family history was negative as obtained. Case 20. Female adult, afted thirty-four. Polycystic kidney disease recognized at age of twenty-five, during pregnancy. Latter successful. Kidneys described at operation for appendicitis at age thirty. No knowledge of kidney dysfunction until onset of hematuria sixteen days prior to admission to hospital. Hematuria and associated pain and infection in left kidney relieved by repeated pelvic lavage and use of silver nitrate. Patient under observation one and one-half years. Father died of Bright's disease at sixty-seven. Case 19. Male adult, aged forty-seven. Polycystic kidney disease. Left renal calculi. Onset gastric ulcer symptoms, 1922; gastroenterostomy, 1925. At this time surgeon referred to "large hydronephrosis," THE JOURNAL OF UROLOGY, VOL. XIX, NO.
2
166
R. E. CUMMING
left kidney. Twenty years ago passed a stone from bladder. One year ago severe left renal colic. X-ray showed two large (?) calculi in left kidney; these later passed from bladder. Recent return of pain in left kidney area, and left groin, urination scant and painful. Referred for urological study with diagnosis of urinary stone. Examination: Both kidneys large, readily palpable, left tender. Roentgenogram negative for calculus, pyelogram typical of cystic kidney (left). At time of admission, non-protein nitrogen 95 mg. per 100 cc. blood. Phthalein output, nil in two hours. Urine from left kidney contained many pus
FIG. 10.
PHOTOGRA)'H OF SPECIMENS, CASE
24.
KIDNEYS BISECTED
~ote practical absence of left kidney with large pelvis. Cysts in right kidney filled with blood. Weight: Right kidney, 1650 grams; left kidney, 80 grams. Measurements: Right kidney, 24.5 by 11 cm.; left kidney, 9 by 3.5 cm.
cells and colon bacilli. Pelvic drainage relieved pain. Retained nitrogen decreased by hospital treatment. Discharged improved. Under observation nine months. Case 27. Adult male, aged thirty-one. Diagnosis, polycystic kidney disease, bilateral; six years previously diagnosed chronic interstitial nephritis, basis being persistent albuminuria. Admitted to hospital for left lumbar pain, recurring for two and one-half years. No hematuria. Examination: Function of both kidneys normal. Gross phthalein 65 per cent. Blood pressure 168/132. Pelvic drainage relieved pain in left kidney, the urine from which proved to be infected, contain-
POLYCYSTIC KIDKEY DISEASE
mg many pus cells. Under observation eight months. Albumi.nuria continues markedly.
Fm. 11.
DRAWING
m·
CoMPUJTE
AUTOPSY
CoNGE"."lTAL POLYCY8TIC KIDNEYS. DROURETER.
"""c.rn1'°""
CASE
24.
I:\n,ATERAL
BILATERAL HYDRONEPHROSIS AND HY--
BLIND LEFT URETER, THE LOWER END
5.5
CM. JN DIAlHE1,EH,
It is conjectural as to whether left kidney ever functioned in life. Di.verticulum of bladder, the opening corresponding in position to the location of the left ureter orifice. Diverticulum anterior to blind ureter, and its wall identical with bladder wall. Persistent urachus. See case history
168
R. E. CUMMING
Case 15. Adult female, aged forty-seven. Bilateral polycystic kidneys. Exploratory laparotomy, 1924. Right kidney reported as ten times normal size, left kidney as four times normal. Present illness: Hematuria, backache, nausea, flatulence, vertigo. Examination: Large palpable masses in both flanks. Cystoscopy: hematuria, right kidney. X-ray, right polycystic kidney. Blood chemistry normal.
Fm. 12.
PHOTOGRAPH OF SPECIMENS, CASE
7.
BILATERAL PoLYCYSTIC
KIDNEY DISEASE
Weight: Right kidney, 760 grams; left kidney, 1040 grams. kidney, 20.5 cm.; left kidney, 21cm. See chart 1 and figure 1.
Length: Right
Urine from right kidney showed, blood, pus, colon bacilli. Three treatments at weekly intervals. Cessation of bleeding. Mild persistent albuminuria. No recurrence of bleeding. Under observation seventeen months. Case 24. Male, aged thirty-six. Admission complaints, March 30, 1927, pain in right side and hematuria. Sixteen years ago patient developed an abscess in the region of the right kidney; this was drained
POL YCYSTIC KIDNEY DISEASE
surgically. At that time, right kidney was larger than normaL In not feeling was examined and found to have a large right (diagnosis by pyelography), hypertension and albuminu-., ria. No evidence of a left kidney could be found; there was a
FIG.
13.
PHOTOGRAPH OF SPECIMENS, CAsE
SYMPTO:VIS UWl'IL
Two
30.
AGED FoRTY·EIGHT.
\VEEKS PRIOR TO DEATH.
No
CONDITION SINCE CHILDHOOD, AND AN EXTREMELY ACTIVE PHYSICIAN.
OF CASE
SER.i.ous
PATIENT COGNIZANT UNCLE
.
See micro photographs. Weight: Right kidney, 3062 grams; left 2948 grams. Measurements: Right kidney, 35 by 18.1 by 10 cm.; left kidney, 32.5 by 20 by 9 cm.
opening in the bladder situated at the normal site of the left uretera1 orifice; this proved to be a small diverticulum. Blood nitrogen renal function low. Advised as to gravity of conditions and prolonged rest ordered. The patient rested for three months, then returned to work (street car conductor); has worked since, regularly, until a few
170
R. E. CUMMING
days prior to this admission to the hospital, at which time present symptoms began. Hereditary record: See accompanying figure. At this examination, patient found anemic (hemoglobin, 50 per cent, red blood cells, 2,300,000). Skin yellowish, suggesting jaundice, sternum prominent (pigeon breast type). Breath ammoniacal. Large abdominal mass on right side, tender, readily visible. No tenderness in left abdominal area, or left flank. Urine scant, very bloody. Blood
FIG.
14.
PHOTOGRAPH SPECIMENS.
DISEASE.
RIGHT
PYONEPHROSIS.
KIDNEY
CASE
(LARGER
16.
BILATERAL POLYCYSTIC KIDNEY
SPECIMEN)
REMOVED
FOR
DEATH FIFTEEN HOURS LATER WITH ANDRIA.
AGNOSIS NoT SUSPECTED.
SUPPOSED TRUE DI-
No PRELIMINARY UROLOGICAL STUDY
pressure 140/90. Blood Wassermann negative. Non-protein nitrogen 172 mgm. Treatment: (1) Catheterization of right ureter; at cystoscopy, absenceof left ureter again noted. Free bleeding from right kidney. Large catheter left in kidney pelvis twenty-four hours. Pelvis lavaged frequently with sterile water. (2) Daily colonic irrigations ancl hot packs. (3) Increasing fluid intake. (4) Blood transfusion. Thel hematuria was checked for twenty-four hours, then returned and was not improved by transfusion which was done eight days after admission. I
POLYCYSTIC KIDNEY DISEASE
FIG.
1,5, PHOTOGRAPHS OF SPECIMEN, SHOWING ADVANCED HYDRONEPHROSIS 1, Snv1ULATING PoLYCYSTIC KIDNEY "CYsTs" CoMMUNlCA'rE FREELY \'{ITH PELVIS WHICH WAS SMALL
TJreteral obstruction
pyelography
172
R. E. CUMMING
Fm. 16.
PHOTOMICROGRAPH FROM SPECIMEN, CASE
30
Note compressed glomeruli projecting into cyst, simulating papillomata. This was basis for new-growth theory of origin. Note also unassembled cells simulating round-cell infiltration.
Fm. 17.
PHOTOMICROGRAPH, CASE
30
Note dilated tubules, compressed glomeruli, nests of undifferentiated tubular cells, and an extensive area of tubular segments, part of which are solid cell masses. Further along these segments one finds cysts.
POLYCYS'rIC K.IQNEY DISEASE
173
Clinical course: Continued renal bleeding, the bladder repeatedly filling with blood clots. For three days, no apparent secretion of urine. Death April 10, 1927, The right kidney had increased in size and continued painful, requiring opiates, Autopsy findings: Large, right polycystic kidney, with dilated pelvis and ureter. These latter were filled with blood clots. Very atrophic left polycystic kidney with enormously dilated ureter, the lower end of which ,vas blind, having only fascial attachments to bladder. Anterior to the lower ureter there was a small diverticulum, thick-walled,. lined with bladder mucosa, and opening into bladder at upper left angle of trigone. Persistent urachus. Multiple small cysts in liver.
FJG. 18.
PHOTOMICROGRAPH,
CYl3T CoN~.'AINING
CASE
30.
SECTION OF LARGE) CYST WALL; EARLY
GLOMElRULUS; CoMPRElSSED UNDIFFElRENTIATED TUBULAR AREAS
Summary of findings in thirty-one cases Age variation: 5·-months fetus to age 66. One, 5-months fetus,. One, 7½-months fetus. Three, full-term feti. One, one.day-old child. One, age, 14, (Total, feti and under 15 years, 7.) One, age 22, One, age 27. One, age 28. (Total, second decade, 3.) Two, age 31. One, age 32. One,. age 34, One, age 36. (Total, third decade, 5.) Two, age 40, One, age 43. Three, age 45. Two, age 47. One, age 48. (Total, fourth decade, 9.) One, age 50. Two, age 52. Two, age 54. One, age 55. (Total, fifth decade, 6.) One, age 65. (Total, sixth decade, l.)
174
R. E. CUMMING
Sex incidence: Female, 18. Male, 13. Cases coming io aiitopsy: 16. Cases subjected to major surgical procedures: 9. (Of these only two lived longer than one month following operation.) Cases subjected to pyelography and pelvic lavage ancl drainage. 14. Pyelography apparently instrumental in fatal outcome in two cases. Drainage of definite value in 12 cases. (N.B. The majority of cases
Fm. 19.
CYsT WITH
A LARGE MAss
OF
Pus
CELL DEBRIS
subjected to surgical procedures, nephrectomy, etc., had no urological study). History of familial polycystic kidney disease: Positive in 11 cases or 36 per cent; negative in 6 cases or 19 per cent; undetermined in 14 cases or 45 per cent. Hematuria noted: 7 cases or 22 per cent. Blood and pus in cysts: 19 cases or 61 per cent. Evidence of nephritis: (clinical) 13 cases or 41 per cent.
175
POLYCYS'l'IC KIDNEY DISEASE
Tendency to predominance of cyst location: Young cases, capsular zones, cortex. Less involvement in medulla. Young adult cases, and old adult cases, cysts diffuse. Associatecl diseases: Nephritis, 13 cases. Lithiasis, 2 cases. Syphilis, 1 case (questionable).
Fm. 20 . .1,
MARKED
OBLITERATION;
CONNECTIVE TrssuE
3,
INCREASE;
2,
CoLLECTING TuBULE
CELL I~LAR DEBRIS lN DILATED TUBULE SEGMENTS
GENERAL CONCLUSIONS
L It is logical to assume that "congenital polycystic kidneys are caused by defective protoplasm, which may be inherited or congenital in obedience with the same biologic laws governing the recessive characteristics illustrated in phylogenetic changes and in other congenital deformities" (Davis).
176
R. E. CUMMING
2. Davis compares the human polycystic kidney to the normal kidney of the codfish and finds distinct similarities; we can therefore assume that the generally accepted theory of cyst-formation as due to developmental defects is amplified, since there is halting at the mesonephric stage.
FIG. 21. 1,
NESTS OF COMMA-SHAPED Tt:BULE SEGMENTS;
AT Tor OF SECTION;
3,
2,
LARGE CYST BORDER
EMBRYONIC INTERSTITIAL CONNECTIVE TISSUE
3. While theoretically, evacuation of cysts to lessen intrarenal pressure, and nephrotomy for drainage of associated pyonephrosis, seem of value, their practical application is not satisfactory. Simpler measures, namely, pelvic lavage and catheter drainage, are of decided value, especially adapted to control hemorrhage and infection.
POLYCYS'.rIC KIDNEY DISEASE
177
4. R ephrectomy for polycystic disease is rarely of ,alue and usually fatal; it is often performed in ignorance of the actual pathology. Careful urological study is indicated in all cases presenting more than a simple nephritic syndrome, and caution is necessary to prevent an increased renal loss of function. Simultaneous ureteral catheterization is dangerous and bilateral pye-· lography impossible. I wish to express my appreciation for the use of their clinical data, to Dr. 0. S. Lowsley, New York, and Drs. H. L. Morris, R. A. MacArthur and J.E. Davis, Detroit. Dr. Eugene Spoehr, Detroit, has n1ade most exhaustiYe abstracts of the literature, eovering hundreds of treatises and reports; only a few referenceR are noted below because of their pertinence to this paper . REFEREXCES ALVAREZ, J. R..: Internal. Jour. Surg., 1911, xxiv, 1-9 . .ASCHOFF, L . : Pathologische Anatomie, 1921 (Eng. Translation Davis, J. E. Ostrander, J\1'.L). BARNETT, C. E.: Polycystic kidney. Surg., Gynec. and Obst., 19,753, December, 1914. BRAASCH, W. F., AND SCHOLL, A. J.: Pathologic complications with duplication of the renal pelvis and ureter (double kidney). Surg., Gynec, and Obst., 1922, xxxv, 401-417. BUGBEE, H. G., AND WoLLSTEn,, M.: Surgical pa,thology of the urinary tract in infants. Jour. Amer. Med. Assoc., 1924, lxxxiii, no. 24, 1887. BUNTING, C. H.: Congenita.l cystic kidney and liver with family tendency. ,four. Exper. Med., 1906, viii, 157-167. BEAUN, L.: Polycystic kidney of atypical character. Amer. Jour. Obst. and Gynec., 1921, i, 868. CRAWFORD, R. H.: Polycystic kidneys. Surg., Gynec. and Obst., 1923, xxxvi" 183-188. DAVIS, JAMES E.: Neoplasia of the kidney. Amer .•lour. Obst. and Gynec., 1922, iii, no. 5. DAVIS, JAMES E.: Congenital polycystic kidneys. Amer. Jour. Obst. a.nd Gynec., ix, no. 6, lune, 1925. D~.Y, R. U.: Some rnre anomalies of the kidney a.nd ureter, with case reports. Surg., Gynec. and Obst., 1924, xxxviii, 51. EISENDRATH 1 D. N.: Polycystic kidney and liver. Ann. Surg., 1921, lxxi1i, 62-64. EISENDEATH, D. N.: Congenital solitary kidney. Ann. Surg., 1924, lxxix, 206-228. Fox, HERBERT: Disease in Captive Vnld Animals and Birds. J. B. Lippincott Company, p . 267.
178
R. E. CUMMING
FRISCHER, JuLius: Polycystic kidney. Jour. Oklahoma State Med. Assoc., June, 1926. GREENE, C.H.: Bilateral hypoplastic cystic kidneys. Amer. Jour. Dis. of Child., 1922, xxiv, 1. GRUBER, G. B., AND BING, L.: Malformations of the kidneys with bibliography. Ztschr. f. Urol. Chir., 1921, vii, 251. HARPSTER, C. M.: Solitary unilateral large serous cysts of the kidneys with report of two cases and review of the literature. Jour. Urol., 1924, ii, 157-165. HER'\IAN, L.: Renal anomalogy. Surg. Clinics of No. Amer., 1924, iv, no. 1, 265-288. HINMAN, F., AND MoRISON, D. M.: Comparative study of circulatory changes in hydronephrosis, cases cavernous tuberculosis, and polycystic kidney. Jour. Urol., 1924, ii, 131-141. IL;,.MPMEIER, 0.: A hitherto unrecognized mode of origin of congenital renal cysts. Surg., Gynec. and Obst., 1923, xxxvi, 208-216. KATZ, G., AND MuHE, E.: Two cases of congenital cysts of the kidney with hypertonia and other complications. Ztschr. f. Urol., 1924, xvii, 453-461. KEYES, E. L.: Urology. Appleton, 1923. KRETSCHMER, H. L.: Solitary cysts of kidney. Jour. Urol., 1920, iv, 567. KRETSCHMER, H. L.: Supernumerary kidney. Jour. Amer. Med. Assoc., 1915, lxiv, 1447. LEE-BROWN, R. K.: Circulatory changes in progressive hydronephrosis. Jour. Urol., 1924, xii. LowSLEY, 0. S., KINGERY, L.B., AND CLARK, H. C.: The surgical significance of certain abnormalities of the urinary tract. Jour. Urol., 1924, xi, 293. LowsLEY, 0. S., AND KrnwIN, T. J.: A Textbook of Urology, 1926. Lea and Febiger. LUND, P. M.: Case of fused kidneys. Amer. Jour. Roent., New York, 1919, N.S., vi, 504. MERTZ, H. 0.: A st-udy of the pelves of double kidneys. Jour. Urol., 1924, ii, 259-291. MORRISEY, J. H., AND SMITH, F. W.: Symptomatology and diagnosis of polycystic kidney. Med. Jour. and Record, 199: 494, May 21, 1924. MoscHOWITZ, E., Amer. Jour. Med. Sc., 1906, no. 131, 674-697. ORMOND, J. K.: Pyelography. Ann. Clin. Med., v, no. 7, January, 1927. PYE, W.: Observations on the development and structure of the kidney. Jour. Anat. and Phys., London, 1875, ix. RrscHBIETH AND DECRESPINGY, C. P. C.: Polycystic disease of kidneys. Lancet, 1913, i, 450. RovsING: Amer. Jour. Urol., 1912, viii, 120. RUMPEL, 0.: Congenital atrophy of one kidney. Arch. f. Kin. Chir., 1921, cxviii, 173-188. ScHOLL, A. J., AND JUDD, E. S.: Hydronephrosis and pyonephroses. Surg. Clin. of No. Amer., 1924, iv, no. 2. SIEBER: Deutsch. Zeitscher. f. Chir., 1905, xxix. SINGER, H. A., AND BRAMS, J.: Congenital cystic kidney in the newborn. Surg., Gynec. Obst., 38: 768, June, 1924.
POLYCYSTIC KIDNEY DISEASE Sl';UTH, SMITH,
E. R, AND K H.: Two cases of maJformation of the kidney in infancy Lancet, London, 1922, i, 737. G. G.; Solitary cysts of the kidney. Boston Med. and Surg. ,Jour., 1924,
693 G ..J.: Duplication of the left renal pelvis and ureter. JouL UroL, 1924, ii, 10.5-110. Tow, A.· Polycystic disease of the kidneys (report of a case in an infant), Amer, Jour. Dis, Child., 1923, xxv, 222. G.: Congenital absence of one kidney. ZtschL f. Uroi. Chir, 1921, no. 3--4, p. 162. WnHEu1.,, C. M,: A case of double ureter in man with failure of development of the kidney about the aberrant ureter. Anat. Rec., 1920, xviii-xix, THOMAS,
179. R. E.; Congenital polycystic kidney with a report of four ca.ses occurring in children of the same mother. Surg., Gynec. and 1918, pp. 423--42.5,
Investigation :ctnd therapy dealing with vital organs already handicapped by developmental defects, at once arm the clinician with wariness, and intrigue him with possibilities for the benefit of a certainly hopeless infirmity. In patients with polycystic kidneys it may be said "he is as old as his cyst-development." It seems definitely proven that when the cyst development (or cystic degeneration) pursues its course to a certain point, Jife as dependent upon kidney function, is no longer possible, One might speak of polycystic kidneys as growing ripe, referring to the stage, at ·which oftentimes the disease is first recognized, the beginning of a fatal decline, manifested by symptoms comparable to nephritic downward progress, renal dysfunction, uremia, anuria, death. As can be readily presupposed, the margin of safety with polycystic kidney disease, is narrow to a degree parallel with the advancement of degeneration, and always narrower than in kidney pathology engrafted upon congenitally healthy organs. With no presupposition of this disease in a given case, one can readily err both in diagnostic methods and prognostic acumen. Post-mortem examinations shmv that in many instances> the polycystic kidney is not suspected in life, death being due to other causes, ·while the kidneys have carried on satisfactorily. But this fact should not disarm one, since age is no criterion of the cystic formation stage, as completely advanced instances of the disease are found at birth and in extreme old age. Certain diagnostic and inYestigati-ve procedures familiar to the urologist, serve not only to pro Ye the presence of the disease we 1 Read at. the annual meeting of the J .. mcrican Urological Association, Baltimore. May, 1927.
140 'J'HE JOPRX.AL OF rJ--tOI,OGY, VOL. XIX, >fO.
?
150
R. E. CUMMING
are discussing, but as means of improving the function of the failing kidneys, thereby possibly delaying the consummation of the disease and ultimate death. We refer particularly to cystoscopy and kidney pelvis drainage, pyelography for diagnosis, and in rare instance nephrotomy for drainage of grossly infected cyst areas. All of these maneuvers require the utmost care and good judgment; simultaneous catheterization of both ureters being dangerous and bilateral pyelography prohibitive. Such radical steps as nephrectomy, removal of the more advanced and (presumably) the pyonephrotic kidney, are likely never of value. Differential diagnosis becomes of more importance, the further one advances in a consideration of the proper care of the polycystic kidney types; the latter word is used deliberately, for although we have outlined generalizations to which all cases conform, certain occasions arise wherein with acute illness intervening, a newly-discovered lateral abdominal mass may demand accurate study with no definite lead to renal involvement, or to false surgical measures, sufficient in themselves to prove of lethal consequence. In the suggested dilemma, medical attendant and surgeon alike, quite naturally call upon the urologist for aid and depend upon him for accurate information relative to retroperitoneal pathology. Naturally too, a mass in this position, with none on the opposite side, the fellow kidney being less advanced cystically, may as readily prove to be a new growth, an abscess, or more commonly a huge hydronephrotic kidney. The physical examination may be entirely ineffective, due to tenderness and muscular defense. Furthermore the ureteral catheter may, by meeting obstruction, fail to enter such a kidney mass, so that the contents cannot be studied, and pyelography is impossible. Ordinarily, however, roentgenographic studies readily differentiate tumor, hydronephrosis and polycystic disease. Unless one is thoroughly familiar with the gross appearance of polycystic kidneys, he will occasionally be perplexed, meeting at the operating table, that large cystic hydronephrotic organ which in the gross has the size and outline of true cystic disease, and is in a sense, a polycystic kidney although the simple result of obstruction and infection which has advanced to complete de-
POLYCYSTIC KIDXEY DISEASE
151
struction of the organ, leaving no functioning renal tissue and filled with stale urine. In such a predicament it is well to remember that the pelvis of a polycystic kidney is rarely dilated; it is usually narrowed and lengthened, ,vhile that of a hydronephrotic kidney is most often dilated. Again, the cysts of polycystic kidneys are closed while
FIG.
L
Bn.ATJ
PY],JLOGRAM,
CASE
7,
TYPICAL OF PoLYCYSTIC DISEASE,
PYI0LOGJUPHY PRJ"CIPI'l'ATED RAPID DECLINE AND DEATH
See case history
the pseudo-cysts of hydronephrotic kidneys are in communication with one another and the renal pelvis. Aspiration will readily differentiate the two conditions. Pyelographic evidence follows the same rule, the media :filling the cyst areas in one and partially surrounding them in the other. The foregoing paragraphs suggest a further consideration of the pathology invoh·ed in polycystic kidney disease, and we find
152
R. E. CUMMING
divergent oprn10ns in the literature and in the utterances of present-day pathologists. It is conceded that of all renal anomalies, the polycystic kidney is the most important. The fact as already intimated, that one organ may show no gross evidence of the disease, does not disprove the theory that the condition is always bilateral. In many instances, Davis has found in cases presenting on the one side advanced cystic degeneration and on the other a grossly normal kidney (as to cyst formation), that this other organ presented under the microscope, unmistakable evidence of early cysts. Other observers have proven this same contention, and, like Davis, have pointed out the tendency to deformities in the skeleton and in other organs, notably the liver. In one of our own cases, the patient was acromegalic and presented a definite deformity of the sternum, in two other cases the spleen was cystic, in many there were liver cysts. Various other congenital deformities were noted. Carefully reviewing the literature one also finds varying views on the anatomic development of polycystic kidneys, and similarly divergent expressions of opinion, concerning etiology. In explanation of this, Davis blames the scarcity of material and the failure of a coordination of ideas, and undertakes, by means of a laborious study of many specimens, to reach a final stage of knowledge relating to the etiological tendencies. Following his work, we are convinced his conclusions will go far to explain logically and satisfactorily, this pathological problem. As to others' theories, a brief resume of those most seriously considered follows: 1. Virchow 's theory was that the cysts are due to inflammation of papillae with closure of their ducts. This idea was abandoned when it was proven that obstruction of the papillary ducts was followed by atrophy of the tubules rather than cystic changes. 2. Brigidi and Severi described "epithelial nests" which degenerated into cysts. This theory of neoplastic development has also been discarded. 3. Hildebrandt's conclusions were based on our knowledge of two separate embryological structures which unite to form the complete kidney; the cortex and major part of the medulla, con-
POLYCYSTIC Krm,,EY DISEASE
153
taining the convoluted tubules and loops of Henle, arising from the nephrogemc tissue of the Wolffian body while the collectiug tubules and peh is are a separate formation from the 'vVolffian duct. Hildebrandt believed that failure of union on the part of these separate strnctures produced cysts at the time secretory actiYity of the glomerulus and tubule should normally begin. ,VhiJe this is the generally accepted theory Yon Mutach and later Bunting, haYe shovm that a direct continuity can be traced between cysts and tubules and Berner found carmine in cysts :1fter injection of the uretero Greene, isolating tubules, found direct continuity between cysts and collecting tubules and so proYes that retention alone does not explain cyst formation. Earlier ,vriters state that the congenital polycystic kidney occurs in all domestic animals, "its origin accredited to cessation of grnwth, with or without union of convoluted and collecting tubules. n Davis' own conclusions would tend to the assumption that human polycystic kidneys result from a partial cessation of development at the mesonephric stage, degenerative changes coming later to complete the picture of interference ,vith the norrnal kidney function. He compares the human polycystic kidney to the normal kidney of the codfish (and other lower forms) and findt' distinct similarities which are most significant since in these lower animals, the full-grown kidney is of the mesonephric type. In DaYis' series of fourteen completely studied cases, he found universal evidence of progressive characteristics and of more importance to the clinical worker, a ''marked tendency to hemorrhage and infection . " This was especially noticed in the adult specimens. The gross material shmved the predominating posit-ion of the majority of the well-formed cysts to be in the cortical and subcortica1 zones, and the oldest and largest formations near the fusion line of the lobules. In all cases of advanced cyst forrna-· ti.on, the lessened renal tissue was accompanied by a ma10sive increase in kidney bulk. Case 24 of this series is an exception to this ruleo Other well recognized gross changes include the irregular surface contour, serrations of concaTe and convex areas correspond0
154
R. E. ClT'.\E'vfI~G
ing to the underlying cysts, the walls of many being almost transparent. The fatty capsule is scarcely recognized, blending with an adherent, toughened and stretched out fibrous capsule. The fluid contained in the cysts varies considerably, but is more serous in type than the retained urine of hydronephrosis. It may have a large solid content derived from the urine salts, blood, tissue degeneration and the coincident chemical reactions upon these elements. We found the fluid in all polycystic kidneys of adult life, to contain blood and pus, evidence of infection and hemorrhage, and Braasch, reviewing those cases observed at the Mayo Clinic, stated that in over 40 per cent of the cases, hematuria was a definite symptom. This latter is commonly overlooked in our consideration of hematuria even in obscure clinical problems. It is perhaps due to the rather uniform gross and microscopic picture, overshadowed with secondary changes (hemorrhage and infection), which the adult kidney presents, that an earlier accurate estimate of etiological possibilities has not been satisfactorily made. Davis hit upon the plan of studying young specimens, especially in early stages (in a fetus) in order to escape the changes mentioned. It is particularly from his study of a serially-sectioned polycystic kidney of a full-term fetus that he has made his admirable and probably correct analogy between polycystic types and the embryological mesonephros. Some significant features of the evidence of developmental halting are the facts that the renal cortex and medulla are never fully demarcated, the latter being recognized in early specimens only by slight indentures of the calyces. Davis states that "the regular assembling of the pyramids with the intervening columns of convoluted loops is never complete in congenital polycystic kidney." In young specimens we found glomeruli in the capsular area, with cysts of Bowman's capsules, as if individual glomeruli had merely dropped out. This disproves the old contention that cysts are confined to the tubular zones. It is not the purpose of this article to deal further with the microscopic anatomy of our subject, itself an intricate and as yet unravelled problem. We do feel however, that one must con-
POLYCYS'I'IC KIDNlsW DISEASE
stantly -investigate the causative factors of any entity met in the clinical sense in order to have a foundation for logical conclusions. Our own conclusions are that the etiologic factors in congenital polycystic kidneys are: L Inherited protoplasmic insufficiency specifically expressed in the differentiation of the m.ost complicated tubular organic structures (kidney and liver), CHART'
L
HEirnDIT'Y RECORD OF CASE
'7
il·faternal gran clmother Died at age 53 Polycystic kidney disease
I I
r--
- - - - - \ - - - - - - - - - - -1 I ! 1 Mother Aunt Uncle Died at age 40--50 Die,l at age 45 Died at age 48 Large bilateral poly., Polyc::•.rstic Polyc:vstic kidney disease kidney disease cystic kidneys
i I
Case?' Died a,t age 32 Polyc:ystic kidney disease See also pyelogrnms (fig. 1), and photograph of specimens (fig. 1'.2). In this family there have been five additional instances of polycystic kidney diseasn, incompietely 1·eccirdeu.
2, This insufficiency is manifested by delayed differentiation of cellular unit-structures. 3, The morphologic eYidence of this structural delay is identi-cal at all ages 1 and is strikingly illustrated in sections s1mwing undifferentiated stroma (embryonic type) containing aggregations of cells which at one le,·el have a loose arrangement, at another form a solid mass or column> at :still another show a dis-ti.net lumen and finally merge into a cyst. The hereditary aspect of polycystic kidney disease haB been widely commented upon; this to such an extent that a familial history 1s eonsidered of the greatest diagnostic value. As many
156
R. E. CUMMING
as fourteen instances in one family are reported, and in our own series, ten individuals of close blood relationship proved to be CHART
2.
HEREDITY RECORD OF CASE
24
Paternal grandfather Died of old age
Paternal grandmother Died of old age
Maternal grandfather Died of old age
I
I
I
L _ _ T _ _ _J
I Father Drowned at age 44
'---T I
Maternal grandmother Died at age ? Polycystic kidney disease I
Mother Died at age 42 Had several operations for renal tumors including a nephrectomy after which she lived one year
L--------------~
r
I
Brother Died at age 43 Bilateral renal tumors
Sister Living at age 38 Has kidney trouble similar to case 24 associated with hypertension
Case 24 Died at age 36 Bilateral polycystic kidneys (Left kidney atrophic and polycystic) (Right kidney polycystic) See also diagnostic pyelogram (fig. 2), post-mortem pyelo-uretero-cystogram (fig. 3), post-mortem specimens (fig. 10), and dra,ving (fig. 11), depicting complete pathology.
victims of the disease. Whether the congenitality indicates direct and true hereditary transmission and whether both male and female progenitors are likely to be followed by the polycystic
POLYCYSTIC KIDKE1' DISEASE
., r,::1.l,)
I
kidney tendency, are questions we can ooly mention to leave for further study.
FIG. 2. PYELOGRAM lW RIGHT KIDNEY, CASE
N·ote hydronephrosis.
24,
l\il.ADJ<; AT DIAGNOSTIC STUDY,
Left u_reter did not connect ·,Nith bladder"
1925
See
figure 11 .
The recognition of several instances of polycystic kidney disease in one family is largely responsible for the term. "cofr genital, n now properly applied in view of the frequency with
158
R. E. CUMMING
which it is found in feti and the newborn. Coupled with this, it seems incongruous to observe the same tissue changes in the organs of a fetus and of an individual attaining old age; but in the one case the degenerative processes rapidly replaced tissue capable of functioning as human kidney whereas in the other, a
Fm. 3.
POST-MORTEM PYELO-URETJmO-CYSTOGRAM, CASE
24
~ote filling of large cysts in right kidney, accomplished by extreme pressure from syringe in pelvis. At autopsy right pelvis and ureter were enormously distended with blood clot.
single collection of tubules show both functionating parts and fully-formed cysts. Tubules develop to the stage of functioning properly while contiguous areas in varying speeds present the typical cystic changes. Returning to the clinical aspect of our study let us consider very briefly the diagnostic signs and discuss the methods of treatment.
159
'.!:'OLYCYSTIC KIDNEY DISEASE
Symptoms: Pain, hematuria, albuminuria, These are common but inconstant and only apply to those cases clinically recognized; in addition one may find any symptom associated with renal disease.
Fm . 4 Frn. 4.
Bn,A'l'ERAL PYELOGRAM,
Fm. 5 CAS:E:
31.
CONDITION NOT PEVIOUSLY SUSPECTED. TIAL NEPHRITIS.
Fm, 5.
LEFT
EXCELLENT RENAL Ft>NCTION PRESERVlm
PYELOGRAM,
ATYPICAL REPRESENTA'!.'ION, TO COB.TEX,
BILATERAL PoLYCYSTIC KIDN:8YS. FINDINGS OF CnrwNIC INTE;RSTI-
CA:sE
29.
BILATERAL
PROBABLY CYSTS ARE
PoLYcvsnc
KIDNEYS,
CONFINl
PATIENT HAS SYMPTOMS OF CHRONIC INTEH.STITIAL .°\JcPHRI'l'IS
Physical signs: Palpable turn.or, unilateral or bilateral. Fluctuation is absent. In patients past fifty, arteriosclerotic changes, hypertension, with their attendant signs, are frequently observed. Diagnostic aids: Pyelography usually typifies the condition, the pelvis is elongated, ,;vithout much dilatation. Many of the
160
R. E. CUMMING
calyces are stretched over the cysts, and converted into convex slits. The urine is abundant and of low specific gravity. vVhen
FIG. 7
FIG. 6 FIG.
6.
RIGHT
PYELOGRAM,
CASE
29.
HYDRONEPHROSIS
DEMONSTRATED,
BILATERAL POLYCYSTIC KIDNEYS
See figure 5. FIG. 7.
RIGHT PYELOGRAM, CASE
(Courtesy Dr. Ormond) 28.
BILATERAL POLYCYSTIC KIDNEY DISEASE.
PYELOGRAM NOT DIAGNOSTIC, INDICATING ONLY A LARGE KIDNEY. OTHER SIGNS TYPICAL OF PROGRESSIVE DISEASE
(Courtesy Dr. MacArthur)
POLYCYSTIC KID:N"EY DISEASE
161
renal insufficiency becomes marked, any or all symptoms of uremia may be presento The several clinical types usually described are but transitions of one another; the slowly advancing case parallels chronic inter., stitial nephritis and may continue for years 1 while at any period
FIG.
.FIG. 8.
PYELOGRAM,
.FIG. 9
8 RIGHT
KIDNEY.
BILATERAL I'OLYCYS'l'IC KIDNEYS.
OUTLINKS MANY VARYING SIZED CYSTS.
lVlEDJA
TYPICAL Ni,RROWED p,~LVIS.
lJRI
(Courtesy Dr. Lowsley)
J:<'IG. 9.
PYELOGRAM, RIGHT KrnNEY.
~vl01rn
BILATERAL PoLYCYSTIC .KIDNEY DISEASE,
ADVANCED ON RIGHT SIDE,.
SuGG8STS HYDRONEPHR0SIS
(Courtesy Dr. Lowsley)
it may, on the other hand merge into the uremic type, with fatal outcome in a few days, or, as is particularly noted by us, any given case may become surgical with pain, hematuria and pyuria. ·with these latter symptoms, according to the treatment and results, a given case returns to the chronic form or gradually
162
R. E. CUMMING
presents grave uremia, frequently having several periods of infection. Treatment: This is largely medical; conservatism is ideal. Dietetic advice and caution as to exercise, physical or mental excesses) exposure, are all important. Repeated and regularly TABLE 1
Collected data indicating wide variations of renal function and suggesting progressive nature a/disease Functional test variations CASE ~UMBER
DYE
INTERVAL
per cent
16 18 19 20 24
DYE
INTERVAL
per cent
61 0 0 5 0
90 5 5 4 8
days days days days days
65 0 0 20 0
DYE
per cent
3 days
0
11 days 2 days
43 0
Total non-protein nitrogen variations CASE NUMBER
N2
INTERVAL
mym.
16 18 19 20 24
29 32 .4 54.5 53 120
N,
INTERVAL
mgm.
I
6 8 5 4 8
months days days days days
31.4 60.3 94 38 172
N2 mgm.
11 days 2 days
29 122
Late data on case 20 February 15 , 1927 { Total non-protein nitrogen ... _................. 54 mgm. Total phenolsulphonephthalem ................ 25 per cent April 25, 1927
{ Total non-protein nitrogen .................... 43 mgm. Total phenolsulphonephthalein ................ 38 per cent
timed examinations, are wise and for increasing gravity of symptoms, surgical procedures must be considered. Brin reported nephrectomy in 17 cases and condemned it. N ephrectomy apparently hastens cystic degeneration in the opposite kidney. In 22 cases, nephrotomy was performed with 31 per cent early mortality and only two patients alive after two
163
POL YCYSTIC KID~EY DISEASE
years. Rovsing advised evacuation of cysts; this has been of value in at least one reported case, its basis depending upon lessened intrarenal pressure with consequent gain in function. Combined with this operation, one might readily drain an infected pelvis and possibly establish a nephrostomy. 17Vhile theoreticaJly) ff\'acuation of as many cysts as can be reached by surgical exposure of one, or both kidneys, seems of advantage, a study of many gross specimens shows how futile such operative efforts TABLE 2
Record of cliagnoslic and therapeutic cysto,,copy CJ,_SE NUI\JBER
7
CYS'rOSCOPIES
INCIDENTAL TRE .-'.._Tl\lE::-.IT
3
Inlying catheter
2
Opern,tion (explorntio,1); transfusion
l5 16 ]7 l8
HI 20 22 23 24,
2
26
1
27
l
')
•J
Operation for stone ~ ephrotomy (death) Transfusion
29
Nephrotomy
30 31
Total cystoscopies ... Diagnostic cystoscopies.. . . . . . .. Therapeutic cystoscopies ....
26 15 11
would prove, since the cysts occupy the entire bulk of the kidney" As late writers advise the so-called Rovsing operation, we would plead a more accurate record of results obtained, Further discussion of nephrectomy is scarcely necessary; our own case reports are typical of this phase of the subject; when one kidney is removed an extra, load on the opposite organ again causes what we may term, a physiological crisis, forcing any differentiation from normal to be more eYident, with resulting
164
R. E. CUMMING
diminution of function. Other involved organs, such as the liver, are simultaneously weakened, tending to dysfunction. All malformed tissue displays a greater tendency to succumb to the vicissitudes of life, so one may say that the potentiality of protoplasm varies in proportion to its defects. Repeated pelvic drainage and lavage have often been of value, and in our hands have relieved acute symptoms repeatedly. Catheter drainage and the cautious use of weak solutions of silver nitrate, have invariably checked a stubborn hematuria, and this drug may be of additional value since in all cases there has been coincident infection. Other considerations of importance along with the surgical manipulations, which are of distinctive value if properly chosen are: 1. Absolute rest in bed 2. Restriction of diet, allowing free fluids 3. Intensive accessory elinination (this type of patient tends to sluggishness of intestinal tract and poor sweating) 4. Selective use of drugs 5. Avoidance of general anesthetics; polycystic kidney disease affords an ideal indication for local or regional anesthesia, where surgery is necessary
Our clinical observations are based upon a total of 31 cases; of these I have studied the surgical or autopsy material in 27 cases. For data upon the remaining 4 cases I am indebted to Dr. 0. S. Lowsley. It is my purpose here to give in briefest outline, a clinical record of selected cases, with a summary relative to age incidence, multiple physical defects and possible surgical results. Case 7. Adult male, aged thirty-two years. Bilateral polycystic kidney, acromegaly, deformed sternum, cystic liver. Kidneys obtained at autopsy; right kidney 20.5 cm. in length, weight 760 grams. Left kidney 23 cm. in length, weight 1140 grams. There is a history in his generation of four relatives with congenital cystic kidney, and a total of IO cases in three generations. Renal disease recognized in childhood (albuminuria). Under medical supervision since fourteen years of age; active with no serious illness until ten weeks prior to his death. At
POLYCYSTIC KIDNEY DISEASE
165
that time severe pain began in left kidney. Bilateral pyelography and complete renal studies made. Extremely active colon bacillus infection with pyuria found involving both kidneys. Prior to pyelography, fair renal function; rapid decrease afterward without an increase in infection which was controlled by catheter drainage and pelvic lavage. Death due to uremia. Repeated urine studies showed a constant marked albuminuria, inconstant casts, low specific gravity and urea nitrogen output, many pus cells and motile bacteria. Case 18. Female child, aged fourteen. Bilateral polycystic kidneys, cystic spleen. Patient under-sized; treated for nephritis since six years of age. Onset of acute symptoms nine weeks prior to death. Pain in left kidney area for eight months preceding last illness. During that period polyuria, urine showing albumin constantly, with low specific gravity. Admitted to hospital she -;ly after onset of acute symptoms of fever, malaise, pain in left kidney area. Examination : Extreme anemia, bilateral large palpabie kidneys, left very tender. Marked pyuria, polyuria, albuminuria. Treatment: Blood transfusion, kidney pelvic lavage and drainage (col~n bacillus infection found in both kidneys). Repeated drainage at four day intervals. Exploratory operation five days later, left kidney, due to extreme pain, leucocytosis, pyrexia, and an increasing tendency to urinary suppression. Operative findings: Polycystic left kidney and spleen. Several large renal cysts on aspiration contained bloody fluid . Postoperative course : death, occurring after five days with uremia, coma, urinary suppression, constant emes1s. In case 18, family history was negative as obtained. Case 20. Female adult, afted thirty-four. Polycystic kidney disease recognized at age of twenty-five, during pregnancy. Latter successful. Kidneys described at operation for appendicitis at age thirty. No knowledge of kidney dysfunction until onset of hematuria sixteen days prior to admission to hospital. Hematuria and associated pain and infection in left kidney relieved by repeated pelvic lavage and use of silver nitrate. Patient under observation one and one-half years. Father died of Bright's disease at sixty-seven. Case 19. Male adult, aged forty-seven. Polycystic kidney disease. Left renal calculi. Onset gastric ulcer symptoms, 1922; gastroenterostomy, 1925. At this time surgeon referred to "large hydronephrosis," THE JOURNAL OF UROLOGY, VOL. XIX, NO.
2
166
R. E. CUMMING
left kidney. Twenty years ago passed a stone from bladder. One year ago severe left renal colic. X-ray showed two large (?) calculi in left kidney; these later passed from bladder. Recent return of pain in left kidney area, and left groin, urination scant and painful. Referred for urological study with diagnosis of urinary stone. Examination: Both kidneys large, readily palpable, left tender. Roentgenogram negative for calculus, pyelogram typical of cystic kidney (left). At time of admission, non-protein nitrogen 95 mg. per 100 cc. blood. Phthalein output, nil in two hours. Urine from left kidney contained many pus
FIG. 10.
PHOTOGRA)'H OF SPECIMENS, CASE
24.
KIDNEYS BISECTED
~ote practical absence of left kidney with large pelvis. Cysts in right kidney filled with blood. Weight: Right kidney, 1650 grams; left kidney, 80 grams. Measurements: Right kidney, 24.5 by 11 cm.; left kidney, 9 by 3.5 cm.
cells and colon bacilli. Pelvic drainage relieved pain. Retained nitrogen decreased by hospital treatment. Discharged improved. Under observation nine months. Case 27. Adult male, aged thirty-one. Diagnosis, polycystic kidney disease, bilateral; six years previously diagnosed chronic interstitial nephritis, basis being persistent albuminuria. Admitted to hospital for left lumbar pain, recurring for two and one-half years. No hematuria. Examination: Function of both kidneys normal. Gross phthalein 65 per cent. Blood pressure 168/132. Pelvic drainage relieved pain in left kidney, the urine from which proved to be infected, contain-
POLYCYSTIC KIDKEY DISEASE
mg many pus cells. Under observation eight months. Albumi.nuria continues markedly.
Fm. 11.
DRAWING
m·
CoMPUJTE
AUTOPSY
CoNGE"."lTAL POLYCY8TIC KIDNEYS. DROURETER.
"""c.rn1'°""
CASE
24.
I:\n,ATERAL
BILATERAL HYDRONEPHROSIS AND HY--
BLIND LEFT URETER, THE LOWER END
5.5
CM. JN DIAlHE1,EH,
It is conjectural as to whether left kidney ever functioned in life. Di.verticulum of bladder, the opening corresponding in position to the location of the left ureter orifice. Diverticulum anterior to blind ureter, and its wall identical with bladder wall. Persistent urachus. See case history
168
R. E. CUMMING
Case 15. Adult female, aged forty-seven. Bilateral polycystic kidneys. Exploratory laparotomy, 1924. Right kidney reported as ten times normal size, left kidney as four times normal. Present illness: Hematuria, backache, nausea, flatulence, vertigo. Examination: Large palpable masses in both flanks. Cystoscopy: hematuria, right kidney. X-ray, right polycystic kidney. Blood chemistry normal.
Fm. 12.
PHOTOGRAPH OF SPECIMENS, CASE
7.
BILATERAL PoLYCYSTIC
KIDNEY DISEASE
Weight: Right kidney, 760 grams; left kidney, 1040 grams. kidney, 20.5 cm.; left kidney, 21cm. See chart 1 and figure 1.
Length: Right
Urine from right kidney showed, blood, pus, colon bacilli. Three treatments at weekly intervals. Cessation of bleeding. Mild persistent albuminuria. No recurrence of bleeding. Under observation seventeen months. Case 24. Male, aged thirty-six. Admission complaints, March 30, 1927, pain in right side and hematuria. Sixteen years ago patient developed an abscess in the region of the right kidney; this was drained
POL YCYSTIC KIDNEY DISEASE
surgically. At that time, right kidney was larger than normaL In not feeling was examined and found to have a large right (diagnosis by pyelography), hypertension and albuminu-., ria. No evidence of a left kidney could be found; there was a
FIG.
13.
PHOTOGRAPH OF SPECIMENS, CAsE
SYMPTO:VIS UWl'IL
Two
30.
AGED FoRTY·EIGHT.
\VEEKS PRIOR TO DEATH.
No
CONDITION SINCE CHILDHOOD, AND AN EXTREMELY ACTIVE PHYSICIAN.
OF CASE
SER.i.ous
PATIENT COGNIZANT UNCLE
.
See micro photographs. Weight: Right kidney, 3062 grams; left 2948 grams. Measurements: Right kidney, 35 by 18.1 by 10 cm.; left kidney, 32.5 by 20 by 9 cm.
opening in the bladder situated at the normal site of the left uretera1 orifice; this proved to be a small diverticulum. Blood nitrogen renal function low. Advised as to gravity of conditions and prolonged rest ordered. The patient rested for three months, then returned to work (street car conductor); has worked since, regularly, until a few
170
R. E. CUMMING
days prior to this admission to the hospital, at which time present symptoms began. Hereditary record: See accompanying figure. At this examination, patient found anemic (hemoglobin, 50 per cent, red blood cells, 2,300,000). Skin yellowish, suggesting jaundice, sternum prominent (pigeon breast type). Breath ammoniacal. Large abdominal mass on right side, tender, readily visible. No tenderness in left abdominal area, or left flank. Urine scant, very bloody. Blood
FIG.
14.
PHOTOGRAPH SPECIMENS.
DISEASE.
RIGHT
PYONEPHROSIS.
KIDNEY
CASE
(LARGER
16.
BILATERAL POLYCYSTIC KIDNEY
SPECIMEN)
REMOVED
FOR
DEATH FIFTEEN HOURS LATER WITH ANDRIA.
AGNOSIS NoT SUSPECTED.
SUPPOSED TRUE DI-
No PRELIMINARY UROLOGICAL STUDY
pressure 140/90. Blood Wassermann negative. Non-protein nitrogen 172 mgm. Treatment: (1) Catheterization of right ureter; at cystoscopy, absenceof left ureter again noted. Free bleeding from right kidney. Large catheter left in kidney pelvis twenty-four hours. Pelvis lavaged frequently with sterile water. (2) Daily colonic irrigations ancl hot packs. (3) Increasing fluid intake. (4) Blood transfusion. Thel hematuria was checked for twenty-four hours, then returned and was not improved by transfusion which was done eight days after admission. I
POLYCYSTIC KIDNEY DISEASE
FIG.
1,5, PHOTOGRAPHS OF SPECIMEN, SHOWING ADVANCED HYDRONEPHROSIS 1, Snv1ULATING PoLYCYSTIC KIDNEY "CYsTs" CoMMUNlCA'rE FREELY \'{ITH PELVIS WHICH WAS SMALL
TJreteral obstruction
pyelography
172
R. E. CUMMING
Fm. 16.
PHOTOMICROGRAPH FROM SPECIMEN, CASE
30
Note compressed glomeruli projecting into cyst, simulating papillomata. This was basis for new-growth theory of origin. Note also unassembled cells simulating round-cell infiltration.
Fm. 17.
PHOTOMICROGRAPH, CASE
30
Note dilated tubules, compressed glomeruli, nests of undifferentiated tubular cells, and an extensive area of tubular segments, part of which are solid cell masses. Further along these segments one finds cysts.
POLYCYS'rIC K.IQNEY DISEASE
173
Clinical course: Continued renal bleeding, the bladder repeatedly filling with blood clots. For three days, no apparent secretion of urine. Death April 10, 1927, The right kidney had increased in size and continued painful, requiring opiates, Autopsy findings: Large, right polycystic kidney, with dilated pelvis and ureter. These latter were filled with blood clots. Very atrophic left polycystic kidney with enormously dilated ureter, the lower end of which ,vas blind, having only fascial attachments to bladder. Anterior to the lower ureter there was a small diverticulum, thick-walled,. lined with bladder mucosa, and opening into bladder at upper left angle of trigone. Persistent urachus. Multiple small cysts in liver.
FJG. 18.
PHOTOMICROGRAPH,
CYl3T CoN~.'AINING
CASE
30.
SECTION OF LARGE) CYST WALL; EARLY
GLOMElRULUS; CoMPRElSSED UNDIFFElRENTIATED TUBULAR AREAS
Summary of findings in thirty-one cases Age variation: 5·-months fetus to age 66. One, 5-months fetus,. One, 7½-months fetus. Three, full-term feti. One, one.day-old child. One, age, 14, (Total, feti and under 15 years, 7.) One, age 22, One, age 27. One, age 28. (Total, second decade, 3.) Two, age 31. One, age 32. One,. age 34, One, age 36. (Total, third decade, 5.) Two, age 40, One, age 43. Three, age 45. Two, age 47. One, age 48. (Total, fourth decade, 9.) One, age 50. Two, age 52. Two, age 54. One, age 55. (Total, fifth decade, 6.) One, age 65. (Total, sixth decade, l.)
174
R. E. CUMMING
Sex incidence: Female, 18. Male, 13. Cases coming io aiitopsy: 16. Cases subjected to major surgical procedures: 9. (Of these only two lived longer than one month following operation.) Cases subjected to pyelography and pelvic lavage ancl drainage. 14. Pyelography apparently instrumental in fatal outcome in two cases. Drainage of definite value in 12 cases. (N.B. The majority of cases
Fm. 19.
CYsT WITH
A LARGE MAss
OF
Pus
CELL DEBRIS
subjected to surgical procedures, nephrectomy, etc., had no urological study). History of familial polycystic kidney disease: Positive in 11 cases or 36 per cent; negative in 6 cases or 19 per cent; undetermined in 14 cases or 45 per cent. Hematuria noted: 7 cases or 22 per cent. Blood and pus in cysts: 19 cases or 61 per cent. Evidence of nephritis: (clinical) 13 cases or 41 per cent.
175
POLYCYS'l'IC KIDNEY DISEASE
Tendency to predominance of cyst location: Young cases, capsular zones, cortex. Less involvement in medulla. Young adult cases, and old adult cases, cysts diffuse. Associatecl diseases: Nephritis, 13 cases. Lithiasis, 2 cases. Syphilis, 1 case (questionable).
Fm. 20 . .1,
MARKED
OBLITERATION;
CONNECTIVE TrssuE
3,
INCREASE;
2,
CoLLECTING TuBULE
CELL I~LAR DEBRIS lN DILATED TUBULE SEGMENTS
GENERAL CONCLUSIONS
L It is logical to assume that "congenital polycystic kidneys are caused by defective protoplasm, which may be inherited or congenital in obedience with the same biologic laws governing the recessive characteristics illustrated in phylogenetic changes and in other congenital deformities" (Davis).
176
R. E. CUMMING
2. Davis compares the human polycystic kidney to the normal kidney of the codfish and finds distinct similarities; we can therefore assume that the generally accepted theory of cyst-formation as due to developmental defects is amplified, since there is halting at the mesonephric stage.
FIG. 21. 1,
NESTS OF COMMA-SHAPED Tt:BULE SEGMENTS;
AT Tor OF SECTION;
3,
2,
LARGE CYST BORDER
EMBRYONIC INTERSTITIAL CONNECTIVE TISSUE
3. While theoretically, evacuation of cysts to lessen intrarenal pressure, and nephrotomy for drainage of associated pyonephrosis, seem of value, their practical application is not satisfactory. Simpler measures, namely, pelvic lavage and catheter drainage, are of decided value, especially adapted to control hemorrhage and infection.
POLYCYS'.rIC KIDNEY DISEASE
177
4. R ephrectomy for polycystic disease is rarely of ,alue and usually fatal; it is often performed in ignorance of the actual pathology. Careful urological study is indicated in all cases presenting more than a simple nephritic syndrome, and caution is necessary to prevent an increased renal loss of function. Simultaneous ureteral catheterization is dangerous and bilateral pye-· lography impossible. I wish to express my appreciation for the use of their clinical data, to Dr. 0. S. Lowsley, New York, and Drs. H. L. Morris, R. A. MacArthur and J.E. Davis, Detroit. Dr. Eugene Spoehr, Detroit, has n1ade most exhaustiYe abstracts of the literature, eovering hundreds of treatises and reports; only a few referenceR are noted below because of their pertinence to this paper . REFEREXCES ALVAREZ, J. R..: Internal. Jour. Surg., 1911, xxiv, 1-9 . .ASCHOFF, L . : Pathologische Anatomie, 1921 (Eng. Translation Davis, J. E. Ostrander, J\1'.L). BARNETT, C. E.: Polycystic kidney. Surg., Gynec. and Obst., 19,753, December, 1914. BRAASCH, W. F., AND SCHOLL, A. J.: Pathologic complications with duplication of the renal pelvis and ureter (double kidney). Surg., Gynec, and Obst., 1922, xxxv, 401-417. BUGBEE, H. G., AND WoLLSTEn,, M.: Surgical pa,thology of the urinary tract in infants. Jour. Amer. Med. Assoc., 1924, lxxxiii, no. 24, 1887. BUNTING, C. H.: Congenita.l cystic kidney and liver with family tendency. ,four. Exper. Med., 1906, viii, 157-167. BEAUN, L.: Polycystic kidney of atypical character. Amer. Jour. Obst. and Gynec., 1921, i, 868. CRAWFORD, R. H.: Polycystic kidneys. Surg., Gynec. and Obst., 1923, xxxvi" 183-188. DAVIS, JAMES E.: Neoplasia of the kidney. Amer .•lour. Obst. and Gynec., 1922, iii, no. 5. DAVIS, JAMES E.: Congenital polycystic kidneys. Amer. Jour. Obst. a.nd Gynec., ix, no. 6, lune, 1925. D~.Y, R. U.: Some rnre anomalies of the kidney a.nd ureter, with case reports. Surg., Gynec. and Obst., 1924, xxxviii, 51. EISENDRATH 1 D. N.: Polycystic kidney and liver. Ann. Surg., 1921, lxxi1i, 62-64. EISENDEATH, D. N.: Congenital solitary kidney. Ann. Surg., 1924, lxxix, 206-228. Fox, HERBERT: Disease in Captive Vnld Animals and Birds. J. B. Lippincott Company, p . 267.
178
R. E. CUMMING
FRISCHER, JuLius: Polycystic kidney. Jour. Oklahoma State Med. Assoc., June, 1926. GREENE, C.H.: Bilateral hypoplastic cystic kidneys. Amer. Jour. Dis. of Child., 1922, xxiv, 1. GRUBER, G. B., AND BING, L.: Malformations of the kidneys with bibliography. Ztschr. f. Urol. Chir., 1921, vii, 251. HARPSTER, C. M.: Solitary unilateral large serous cysts of the kidneys with report of two cases and review of the literature. Jour. Urol., 1924, ii, 157-165. HER'\IAN, L.: Renal anomalogy. Surg. Clinics of No. Amer., 1924, iv, no. 1, 265-288. HINMAN, F., AND MoRISON, D. M.: Comparative study of circulatory changes in hydronephrosis, cases cavernous tuberculosis, and polycystic kidney. Jour. Urol., 1924, ii, 131-141. IL;,.MPMEIER, 0.: A hitherto unrecognized mode of origin of congenital renal cysts. Surg., Gynec. and Obst., 1923, xxxvi, 208-216. KATZ, G., AND MuHE, E.: Two cases of congenital cysts of the kidney with hypertonia and other complications. Ztschr. f. Urol., 1924, xvii, 453-461. KEYES, E. L.: Urology. Appleton, 1923. KRETSCHMER, H. L.: Solitary cysts of kidney. Jour. Urol., 1920, iv, 567. KRETSCHMER, H. L.: Supernumerary kidney. Jour. Amer. Med. Assoc., 1915, lxiv, 1447. LEE-BROWN, R. K.: Circulatory changes in progressive hydronephrosis. Jour. Urol., 1924, xii. LowSLEY, 0. S., KINGERY, L.B., AND CLARK, H. C.: The surgical significance of certain abnormalities of the urinary tract. Jour. Urol., 1924, xi, 293. LowsLEY, 0. S., AND KrnwIN, T. J.: A Textbook of Urology, 1926. Lea and Febiger. LUND, P. M.: Case of fused kidneys. Amer. Jour. Roent., New York, 1919, N.S., vi, 504. MERTZ, H. 0.: A st-udy of the pelves of double kidneys. Jour. Urol., 1924, ii, 259-291. MORRISEY, J. H., AND SMITH, F. W.: Symptomatology and diagnosis of polycystic kidney. Med. Jour. and Record, 199: 494, May 21, 1924. MoscHOWITZ, E., Amer. Jour. Med. Sc., 1906, no. 131, 674-697. ORMOND, J. K.: Pyelography. Ann. Clin. Med., v, no. 7, January, 1927. PYE, W.: Observations on the development and structure of the kidney. Jour. Anat. and Phys., London, 1875, ix. RrscHBIETH AND DECRESPINGY, C. P. C.: Polycystic disease of kidneys. Lancet, 1913, i, 450. RovsING: Amer. Jour. Urol., 1912, viii, 120. RUMPEL, 0.: Congenital atrophy of one kidney. Arch. f. Kin. Chir., 1921, cxviii, 173-188. ScHOLL, A. J., AND JUDD, E. S.: Hydronephrosis and pyonephroses. Surg. Clin. of No. Amer., 1924, iv, no. 2. SIEBER: Deutsch. Zeitscher. f. Chir., 1905, xxix. SINGER, H. A., AND BRAMS, J.: Congenital cystic kidney in the newborn. Surg., Gynec. Obst., 38: 768, June, 1924.
POLYCYSTIC KIDNEY DISEASE Sl';UTH, SMITH,
E. R, AND K H.: Two cases of maJformation of the kidney in infancy Lancet, London, 1922, i, 737. G. G.; Solitary cysts of the kidney. Boston Med. and Surg. ,Jour., 1924,
693 G ..J.: Duplication of the left renal pelvis and ureter. JouL UroL, 1924, ii, 10.5-110. Tow, A.· Polycystic disease of the kidneys (report of a case in an infant), Amer, Jour. Dis, Child., 1923, xxv, 222. G.: Congenital absence of one kidney. ZtschL f. Uroi. Chir, 1921, no. 3--4, p. 162. WnHEu1.,, C. M,: A case of double ureter in man with failure of development of the kidney about the aberrant ureter. Anat. Rec., 1920, xviii-xix, THOMAS,
179. R. E.; Congenital polycystic kidney with a report of four ca.ses occurring in children of the same mother. Surg., Gynec. and 1918, pp. 423--42.5,