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Unilateral Fused Kidney: A Report OF Five Cases and A Review of the Literature
UNILATERAL FUSED KIDNEY A
REPORT OF FIVE CASES AND A REVIEW OF THE LITERATURE
HARRY A. WILMER From the Department of Pathology, University of Minnesota
"Crossed ectopia with fusion," "crossed dystopia with fusion," and "unilateral fused kidney" have been used almost interchangeably in the literature to describe the same rare congenital renal anomaly where both kidneys are fused on one side of the midline. The term "unilateral fused kidney" is not strictly correct when the kidney is prevertebral in position, but it is accurate enough for practical purposes, and has the advantage of being readily understood. Unilateral fused kidney must not be confused with solitary congenital kidney (unilateral aplasia) in which only one kidney is present. When solitary congenital kidney occurs with complete ureteral duplication both ureters enter the bladder on the same side, while in the unilateral fused kidney each ureter enters the bladder on its corresponding side. A review of the statistics dealing with this anomaly shows that before the introduction of pyelography by Voelker and Von Lichtenberg in 1906 all cases were found either at autopsy or operation. Since the introduction of pyelography, however, a great many cases have been recognized clinically. Over 54 per cent of the 286 cases reviewed in this paper were reported since 1921. It appears that this anomaly is not as rare as indicated in earlier surveys based largely on autopsy findings. Crossed ectopia may occur without fusion. Pagel found 14.5 per cent unfused in a series of 55 cases of crossed ectopia. A classification of the fusion anomalies is presented here, in which the position of the ureters is the basis of differentiation. The four basic groups are given below, and further subdivisions of the unilateral fused kidney group will be given later. 1. Horseshoe kidney: Horseshoe kidneys are fused at their inferior poles by an isthmus of renal or connective tissue. In rare cases the superior poles are fused instead. They are generally prevertebral in position, and lie below the level of the origin of the inferior mesenteric artery. 2. "L" shaped kidney: This is a transitional form between typical 551
552
HARRY A. WILMER
horseshoe kidneys and unilateral fused kidney. When the ureter of the ectopic kidney crosses the midline the anomaly may be classified as unilateral fused kidney. When neither ureter crosses the midline, the "L" kidney is merely a variation of the horseshoe kidney. This is described further under subdivisions of unilateral fused kidney. 3. Unilateral fused kidney: The ureter of the ectopic kidney crosses the midline of the vertebral column. The entire renal mass is located largely on one side of the midline. The ectopic kidney is usually inferior to the orthotopic kidney, and the fusion is generally pole to pole, but a great many variations have been reported. 4. Miscellaneous: This includes all the cases that do not fall definitely in groups 1, 2, or 3. The lump kidney is usually in this class but may fall in group 3. When it is in group 4, it may be situated either prevertebral or in the pelvis, but neither ureter crosses the midline. Case 1 (fig. 1). The specimen was found in the dissecting room of the Department of Anatomy at the University of Minnesota. The body was that of a male 47 years of age. The clinical diagnosis of acute glomerulonephritis was verified by microscopic examination of the kidney by Dr. E. T. Bell. The right kidney was ectopic, being superimposed on the left orthotopic kidney. The ureters were unobstructed, and there was no evidence of hydronephrosis. The renal mass was situated chiefly on the left side, although the lower part was prevertebral. It extended from the top of the second to the lower part of the fifth lumbar vertebra, and measured in its longest dimensions 13.5 by 10.5 by 5 cm. The anterior surface showed fetal lobulations and the posterior surface was smooth. There was a clear line of demarcation between the kidneys on their anterior surfaces where the upper pole of the left kidney could be seen projecting above the right kidney. The posterior surface of the renal mass conformed to the psoas muscle, the left common iliac artery, and part of the vertebrae. The kidney weighed approximately 275 gm. The left adrenal was in the normal position above the kidney but not connected with it, while the right adrenal was situated normally though no kidney was below it. The right hilus was anterior, and the right ureter crossed the fifth lumbar vertebra to terminate at its normal position in the bladder. The left hilus was anterior and slightly lateral. The left ureter, likewise, terminated in the bladder normally.
The left or orthotopic kidney received 2 arteries directly from the left side of the aorta. The second of these two supplies both kidneys. The
UNILATERAL FUSED KIDNEY
553
left kidney also receives two more branches from the left common iliac .artery. One of the latter penetrates the middle of the posterior surface of the kidney directly above the left common iliac artery. The right kidney receives 2 arteries, one from the aorta, the other from the middle of the right common iliac artery. This branch passes under one of the
FIG. 1
fetal lobulations and penetrates the renal parenchyma near the right hilus. The left kidney is drained by 3 veins. The upper one begins at the left hilus and is joined by a small branch from the projecting part of the left kidney, and also by the left spermatic vein; this vein empties into the
554
HARRY A. WILMER
left side of the vena cava. The remaining 2 veins correspond to the 2 lower arteries from the left common iliac artery to the left kidney. The right kidney, is, likewise, drained by 3 veins. The first vein begins at the right hilus and empties into the left side of the vena cava. The second vein corresponds to the artery to the right hilus. It joins the vena cava slightly on the right side. The third vein arises from the lower part of the kidney below the hilus and empties into the left common iliac vein. There is no corresponding artery. Case 2 (From the Pathology Department, University of Minnesota): A female, aged 37, revealed a unilateral fused kidney at autopsy. Death was attributed to tuberculous enteritis. The left kidney was at the second lumbar vertebra in the midline. Its pelvis stretched from the center laterally down
FIG. 2
the midline to the right, and then coursed back to terminate in its normal position at the trigone. The right kidney is in approximately normal position, but its lower pole is fused with the left kidney. The kidney mass weighed 300 gm. (A 28-1714). Case 3 (By personal communication from Dr. Frederic E. B. Foley): A female 20 years of age (in 1926) complained of pain in the right epigastrium of 2 years' duration. The indigo-carmine test indicated normal renal function; cystoscopy revealed a normal bladder, and there was no record of hematuria. There was a palpable mass in the lower right quadrant, its upper limit being a little above the iliac crest, its mesial margin almost in the midline and extending as far down as the promontory. It was fixed and could not be forced up into the flank. There had been some polyuria for 2 or 3 months prior to consultation. In the traced pyelogram (fig. 2) it may be seen that the ureter of
UNILATERAL FUSED KIDNEY
555
the ectopic kidney is kinked and both pelves are dilated, A diagnosis of unilateral fused kidney was made from the pyelogram. Case 4 (By personal communication from Dr. Frederic E. B. Foley): A female 18 years of age, complained of pain in the left upper abdominal quadrant of 3 years' duration. There were no urinary symptoms, and no nausea,. The pyelogram showed both renal pelves to the right of the lumbar centra, and abnormally rotated (fig. 3). At operation the lower pole of the right kidney
FIG. 3
was found fused to the upper pole of the left crossed ectopic kidney (fig. The 2 kidneys were connected obliquely across the midline, the upper pole being well to the right lateral side of the vertebral column. The 2 kidneys were separated by a well-defined isthmus which measured about 1.5 thick x 4 cm. wide. The renal mass lay upon the vena cava, the right iliac artery, and the right iliac vein. The ureters appeared normal and there was no apparent obstruction. Dr. F. E. B. Foley performed a right nephropexy, dividing the
556
HARRY A. WILMER
isthmus and freeing the left kidney by blunt dissection. The inferior left kidney was pushed to the left side. There was an uneventful convalescence. A post-operative pyelogram at the time the patient was discharged from the hospital showed the right pelvis up in excellent position. Since leaving the hospital the patient has been seen a number of times in the out-patient department and reports complete relief of the abdominal pain present before operation. Case 5 (By personal communication from Dr. D. James Waterson, The Hospital for Sick Children, Great Ormond St., London): A unilateral fused kidney was found in a child, 5½ years of age, dead of diarrhea and acute bron-
FIG. 4
chopneumonia. The renal condition was not suspected during life. The autopsy revealed a right kidney situated normally, fused by its lower pole to the upper pole of the left kidney. The junction is marked on the anterior surface by a deep groove transmitting the right ureter. The left suprarenal capsule is separated from the kidney, which is lower than normal.
Source of cases reviewed. Renal anomalies have always been a fascinating subject. Aristotle mentioned a unilateral kidney defect. The :first case of unilateral fused kidney was reported by Pannorlus in 1654. In this paper 286 cases have been reviewed. They are. taken from the sources found at top- of following page: 1 1 We have found 29 cases reported in the literature as crossed ectopic kidneys, in which it is not stated whether there was fusion, or the original papers were inaccessible to us. Cases reported by Guillemin and Muller are listed in the 1937 cumulative index as crossed ectopic fused kidneys. Since the original papers are inaccessible to us these cases are not included.
557
UNILATERAL FUSED KIDNEY Year
Gerard .. .. ..................................... . . . . ....... . Papin and Palazolli .............. . .......... . . . . . . .. .. ...... Stein ... ..... ..... .. . ..... . .. .. . . .... .. ........ ... . .. .. .. .. Kretschmer. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pierson ... .. ............ .. . . .. . .... . ...... ... . . .... . . . .. ... . Thompson and Allen ..... ... . . . ... . .. . . .. ....... . . .. . . .. . .. . Townsend and Frumkin .. . . .. ... ............... . .. ... ... .... Beer and Ferber .................. .... ...... ... .. .. . . ........ This series ... . ............. . ... .. . . ...... . . .. .. ... .. . ...... Total.. . .... .. .. . ... ... . .. . . ...... ..... . .. .. ... . . . . . . . .
1905 1909 1916 1925 1932 1934 1937 1937 1938
Cases
11 60 11 28 16 20 32 14 94 286
For the purpose of this review, the cases collected from the literature and not given in previous compilations will be given in abstract form. Sex. Of 225 cases in which the sex was recorded, there were 125 males, and 100 females. These :figures suggest a predominance in the male; however, 116 cases out of the 286 were diagnosed at autopsy. It is the usual experience that more autopsies are performed on males than on females. Therefore, the sex difference is probably not significant. Side e.ffected. Of 239 cases in which the position of the kidney was mentioned, there were 142 on the right and 90 on the left. In seven cases the kidney mass was definitely prevertebral, and in one instance both ureters crossed the midline. Age. The age distribution graph (fig. 5) indicates by white columns the age at which the fused kidney was discovered clinically, and by black columns the age at which death occurred, in cases diagnosed at autopsy. The mode of the cases diagnosed in living patients is in the age group 21 to 30. The great majority of the cases recognized clinically were in patients under 50. In the autopsy series the cases are most frequent in the 2nd, 3rd, and 4th decades. It is interesting to note that death occurred at an early age in many of these cases. The great majority of the cases diagnosed at death were, likewise, under the age of SO. Associated renal lesions. The authors are often not clear as to the severity and the character of the coexisting renal lesions. In 137 cases in which there was a record of the renal pathology; infection of some kind was noted in 40 cases, and a diagnosis of nephritis was made in 14. Hydronephrosis was present in 26 cases and dilation of the ureter or pelvis was noted in 21. Calculi were found in 14 instances, nephroptosis was reported in 7, and tuberculosis in 7 cases. Miscellaneous lesions were described in the remaining. Unilateral fused kidneys seem predisposed to hydronephrosis and pyelonephritis, but not to other renal lesions.
558
HARRY A. WILMER TABLE
1
.!.ti i:.lS UOI
AUTHOR AND YEAR REPORTED
~~
DIAGNOSIS BY :
AGE
tl" .,14
.,""'.. "'"' "'"' ~:z;
><
- - - -"'"'
gi
<
RENAL
~
COMPLICATION
REMARKS
t'.l
i!l
"
< ~
ill ill - --
--
years Adler-Racz (1931)
?
?
Operation
L
Tuberculosis of lower kidney
Adler-Racz (1931)
25
F
Pyelography
L
Pyoncphrosis,
Adler-Racz (1930)
50
M Pyelography
L
Afanassiew (1933) Afanassjew (1933)
25 22
F F
Pyelography Pyelography
Afanassjew (1933)
45
F
Pyelography
X
?
X
X
L L
X right; tuberculosis, left Hydronephrosis, X left Nephroptosis X ? X
L
Bilateral pyelec- X
X
Pain of 5 years' duration Polyuria; pain
?
?
Death due to lung
?
?
?
? ?
? ?
Pain of 5 years' duration
tasis
Auge and Bonnet (1924)
?
F Autopsy
Babainantz (1934)
23
F
Pyelography
L
Barclay and Baird (1935) Barry and Garvey (1930)
?
?
?
28
F
Pyelography
L R
R
abscess; ''S'' kidney
Right
hydro-
nephrosis
Bessesen (1933)
Adult
M Pyelography L confirmed at
Braasch (1912)
X
calculus, upper
right kidney
operation
Borchardt (1932) Braasch (1912)
Bilateral hydro- ? nephrosis Pyonephrosis and X
54
F
?
?
and autopsy Pyelography L Pyelography L
?
?
Pyelography
L
due to post opera-
tive hemorrhage Hydronephrosis, left Hydronephrosis,
? ?
? ?
?
?
?
?
?
?
?
?
X
X
left Canelli (1918)
?
Canigiani (1936)
24
Carleton (1937)
Aged
Carraro (1914)
32
M Autopsy
?
Pyelography
L
R
M Dissecting room
L
F X-ray
R(?)
Pelvic lavage cleared up symptoms Nausea and vomiting; pain. Death
Hydronephrosis, right Hypoplasia, left
"L" kidney; lower left resected "L" kidney; lower left resected 325 gm.; 15.5 by 9 by 3.4 cm. Death due to accident
Dilation of both ureters; right ureteral orifice more posterior-central than left. Multiple bladder di verticula 14 cm. long kidney Polyuria; no macroscopic lesion seen
at operation; 15-17 cm. long Cifuentes and Ontanon (1933)
43
Craisin (1913)
?
F
?
Pyelography
Autopsy
L
?
X
?
X
?
Pain of one year dura-
tion; ectopic kidney superior N ephrectomy of entire kidney; death due to anuria
559
UNILATERAL FUSED KIDNEY TABLE
I-Continued
'!il
~" UOI
.!Pl AUTHOR AND YEAR REPORTED
oO
AGE
DIAGNOSIS BY:
tl ...
w"' A[j w ··
REMARKS
..
Ull,l
- - - -"'
~
[j ~
"''" ~:,;
><
!al
"'"'
RENAL
COMPLICATION
~ ~ - --
--
years
DiNatili (1934)
?
F
31
F
X-ray
R
Pyonephrosis
X
?
Mesia! fusion; pyonephrotic kidney
?
?
No renal symptoms;
resected
Dunavant (1919)
Egyedi (1935) Eisendrath (1934)
45
Elving (1926) Farcas and Revesz (1930)
22 46
Operation; L verified by pyelography M R M Probable au- R topsy F R M Pyelography R
Farcas and Revesz (1930)
60
M Pyelography
Genouville and Saquin (1924) Godard (1860)
?
?
Pyelography
?
?
Autopsy (?)
?
diagnosis
acci-
dental Perirenal abscess
N ephroptosis
R
? ?
? ?
Superior kidney ectopic
?
X
X
?
X
X
?
?
Nephropexy formed
?
?
Anomalies
Function test normal diagnosed Author horseshoe kidney; ureter crossed midline Indigo-carmin appeared from right 14 m in.; left 12 min.
?
Hydronephrosis
R
perof
the
genitalia Gottlieb (1927) Gruber (1924)
F F
Pyelography Autopsy
L
4
F
Autopsy
R
25
F
29
F
Laparatomy: R confirmed by pyelography Pyelography L
F
Pyelography Pyelography
30 3½
Gruber (1924)
? ?
? ?
Left ureter dilated
?
?
12.5 by 10 by 5.5 cm. "S" kidney; death due to post-operative bronchitis and
Hypoplasia, right
?
?
Heminephrectomy
X
?
R
X
X
L
X
?
Hematuria; nausea
Nephropexy; intense pain 4 lumbar months duration Heminephrectomy Pyelocystostomy. This appears to be a pelvie horseshoe kidney; both ureters cross the midline; i.e. both kidneys are crossed ectopic with their inferior poles fused
?
''Kuchenniere''
bronchiectasis
Guellemin (1937)
Guimpelson and nova (1935) Guimpelson and nova (1935) Guimpelson and nova (1935) Guimpelson and nova (1935) Hachez (1934) E. Hess (1929)
FatiaFatia-
32
Fatia-
35
F
Fatia-
42
M Pyelography
L
X
X
20 9
M Pyelography M
L X Both Extreme hydro- X nephrosis right
? ?
560
HARRY A. WILMER TABLE
!-Continued
6tl
i;ls
AUTHOR AND YEAR REPORTED
s: .. ""' oo DIAGNOSIS BY:
AGE
><
~
--- -
tl I;!
"'"'<
RENAL
.,~~.. "'"' "'"' ez
COMPLICATION
?
Hydronephrosis right ectopic kidney. Left ureter dilated
~
i!i
.:
"'... "' ...<.:
REMARKS
"' - --
years
Herman (1934)
10
Hodge (1870) 25
Jacobs (1933)
Kalberg (1932) Kundrat (1886) Kundrat (1886) Landois (1930)
55-60 ? ?
?
Pyelography
?
Autopsy
L
M Pyelography
R
M Autopsy
L R R L
?
? ?
Hydronephrosis right (lower)
?
?
Hematuria; dysuria; prevertebral, mostly on left
?
?
X
?
1 pelvis; 3 ureters; 2 arteries Severe pain of 3 months' duration; right kidney resected, left rotated to normal position
? ? ?
? ? ?
X
X
?
?
Extensive fusion
42
F F
Pyelography
Lembergh (1924)
1 mo .
F
Autopsy
Lembergh (1924) Lembergh (1924) Lembergh (1924) Lisa and Levine (1932)
4mo. 52 1 day ?
F Autopsy M ? M Autopsy M Autopsy
L L L R
? ? ? ?
? ? ? ?
Lisa and Levine (1932)
?
F
Autopsy
R
?
?
Maisels
25
M Pyelography
R
Hydronephrosis, X right; calculus, right pelvis, and left ureter
?
Pyelitis
X
X
?
?
?
?
Extensive anomalies of genitalia: ectopic kidney superior ? . No renal symptoms
Maisels
37
F
Pyelography
?
L
M Autopsy
R
30
M Laparatomy
L
Martius (1913)
45
F
?
L
?
Mattioli (1926)
?
F
Pyelography
R
?
McDonald (1885)
Mayer and (1911)
Nelkman
?
Autopsy report: acute nephritis, traumatic infarcts of kidney
Abdominal pain: function test norma! ''Kuchenniere'' 3 pelves, 2 ureters, 1 artery
Separate artery for
each kidney Separate artery for each kidney Recurrent attacks of right renal colic and hematuria; calculus in left ureter removed at operation. Other stone
?
passed later Recurrent attacks of pain for 3 years Death due to epileptic dementia kidney weight, 356 gm. Kidney ruptured by fall on a nail; death due to anuria; 400 gm. weight
561
UNILATERAL FUSED KIDNEY TABLE
!-Continued
'tl
~<>
"" A UTHOI< AND YEAR
REPORTED
AGE
DIAGNOSIS BY!
ii:04 oo tl'-<
r4f:1
.,A"'.. "'"' "'"' ~z
><
- - - -"'"' ...
35
Meyer (1908) 3 wks. Mintz and Stewart (1931) 17
....
~
-?
?
?
L L
? ?
? ?
L ? L(?) L R Hydronephrosis L Cystic, hydronephrotic, atrophied right kidney R(?)
? ? ? ? ? ?
? ? X ? ? ?
X
X
?
X
X
?
?
?
X
X
Pain of 2 years' duration; right kidney resected
?
?
2 arteries; 2 veins; 20
?
?
R
?
?
M Autopsy
Moure and Marcy (1937) Nauman (1897) Neuwirt (1924) Newman (1898) O'Conor (1934) Ogston (1879)
22 ? 28 26
? Autopsy M Operation; confirmed by pyelography F Pyelography ? Autopsy M Pyelography ? Autopsy M Pyelography F Autopsy
Pfitzer (1930)
45
M Pyelography
Pleschner (1929)
20
?
Pyelography
L
Ratner (1930)
35
F
?
L
Ratner (1930)
25
M Pyelography
R
Rumpel (1929)
26
F
Pyelography
R
Russel (1885)
55
M Autopsy
Ruthardt (1827)
40
M Autopsy
Sawyer (1934)
18
M Roentgeno-
?
REMARKS
f:l
.~ -
years
Meloy (1918)
"'"'< :,a
RENAL COMPLICATION
Hydronephrosis, both kidneys Pyelitis, left Pyelitis, right upper ureter dilated Hydronephrosis, right (which pressed on aorta)
R
?
Pyelectasis
;;'.;
gram
Schilling (1920) Schillings, Benez and Lapiece (1936) F. W. Smith, cited by Peterson (1919)
40 35
F Autopsy M Pyelography
L R
? X
? ?
24
M Pyelography
L
?
X
Sorrentino (1936) Surraco (1927)
26 38
M Pyelography ? Pyelography
R L
Tuberculosis N ephroptosis
X No X ?
285 gm.; 10 by 5 cm. 2 arteries; 2 veins '·L" kidney No symptoms of renal origin
Operation Cited by Kretschmer "'S" kidney
Left kidney resected No left adrenal; anomalies of genitalia Slight pain 3 years' right duration ; ureter divided in two at 4th lumbar vertebra; function test normal Right kidney resected Hematuria, subacute cystitis Dysuria
by 8.1 cm . Death due to psoas abscess; 5 arteries, 4 veins; 10 by 12.5 cm. Severe trauma to kidney Anomaly of genitalia
No renal symptoms. Function test norma! Vomiting
562
H ARRY A. WILMER T ABLE
!-Concluded Q !il
£lo AUTHOR AND YEAR
REPORTED
""' ~~
AGE
DIAGNOSIS BY:
tl'"' ,-f;j
.... "''"' "'"' "'~z'"
~
l:l
Schramm (1932)
46
M Laparotomy
R
Schramm (1932)
?
M Pyelography
L
Voutein (1910)
?
?
Autopsy
Zeiss and Boemingh aus (1934)
9
F
Pyelography
L
Zeiss and Boeminghaus (1934) Zeiss and Boemingh aus (1934) Zeiss and Boemingh aus (1934)
9
F
Pyelography
L
42
F
Pyelography
R
34
F
X-ray
R
Zeiss and Boeminghaus (1934) Zeiss and Boemingh aus (1934)
28
F
Pyelography
L
22
F
X-ray
L
Wilmer
47
M Dissection
R
Wilmer
37
F
Autopsy
Wilmer (Foley)
20
F
Wilmer (Foley)
18
F
Wilmer (Waterston)
5- 6mo. ?
?
:,i
REMARKS
['.l
.:"'
~ -
:;j
Bilateral pyelec- ? tasis; right kidney dilated Pyelectasis, ? right; hydronephrosis and infection left ?
?
c.>
-- years
"' "' <
RENAL COMPLICATION
.. ?
N ephrectomy of ec topic kidney
?
Death due to anuria following complet e nephrectomy "S" kidney
?
?
?
?
X
?
?
?
X
?
?
?
?
?
13.5 " 10.5 x 5 cm.
L
?
?
Pyelography
L
X
Pyelography
L
Bila teral pyelec- X tasis X
Autopsy
L
?
?
Death due to t uber culous enteritis P olyuria, left urete kinked P ain of 3 years' dura tion; isthmus wa divided and righ per nephropeJ
Pye!itis right; obstructed right ureter
Pyelectasis and infection right H ydronephrosis and calculus, left I nfection in both kidneys Infection in both kidneys; dilated right calyces; nephroptosis Acute glomerulonephritis
X
" S" kidney; no rena symptoms Pain in back of 3 years' duration Symptoms of 1 year duration
H ema turia symptom 9f 3 years' duration Symptoms of 4 years duration: nephro pexy a t 22; still sick at 37
Surgery. Hemi-nephrectomy was performed in 21 cases because of hydronephrosis, infection, tuberculosis, etc. N ephropexy was performed 13 times, lithotomy 9 times. The diagnosis was made at laparotomy 11 times. In two of these cases the failure to make a clinical
UNILATERAL FUSED KIDNEY
563
diagnosis was due to the fact that a unilateral pyelogram was made. Decapsulation was performed 3 times. In 8 cases death was attributed to anuria. In 5 of these cases the renal condition was not recognized preoperatively, and complete nephrectomy was performed. In 1 case an anomalous blood vessel was severed to relieve the symptoms. It is possible that the anomalous blood vessels may cause obstruction. In 2 obstetrical cases the position and size of the renal mass offered an obstruction that necessitated caesarian section. In one of these cases the woman had previously given birth to 6 children by normal confinements; in the other it was the first child. The frequent location of the fused kidney at the promontory or in the pelvis makes h a factor of importance '15 _AvEllEMfL
CONtJ/r/0N /l.EC06NIZEI>:
¥0
DOHi/AT/ON OIi X-RAY
35
t
10
;S
Z5
~
~
~
~
IAtt~sr
\
.J POINT SMOOTHING
\/
''
AGE
GROUPS
Fm. 5
in obstetrics. In many cases normal delivery with no dystocia is reported. Cause of death. Of 54 cases in which the cause of death was given, 18 were attributed to some pulmonary involvement. Of these, 7 were pulmonary tuberculosis, and 8 pneumonia. Three of the 18 cases were complicated by heart disease. In 7, death was due to nephritis, 3 of these were complicated by heart disease. Tuberculous enteritis was the cause of one death. Pulmonary and renal deaths together account for 25 of the 54 deaths. The remaining 28 contain 11 cases of post-operative death from peritonitis, hemorrhage, anuria following complete nephrectomy, etc.
564
HARRY A. WILMER
Incidence. The frequency of unilateral fused kidney is indicated by the following statistics: A ulhor
Cases
Stewart and Lodge. .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Morris .... .. . ... . .. . . . ...... . .... . . .. .. . . . .. . .. . . ... .. .. 1 Boody. .. . ... . ...... . ............ ... ... . ..... . . . .... .. .. 1 Nauman. . .. .. ...... ... .. . . ... .... ... . .. .. .. .. ... . .. .... . 1 University of Minnesota ..... . .... .. . . . . . . . .. . .. .... . ... .. 1 Rush Medical College ...... . ...... .. .. . . . ... ... . ... . .. ... . 7 Western Reserve University. ......... .. . ... .. .. ........ . .. 0 University of Michigan ........... . .. . . . ... . . ... ... . . . . . . . 0 University of Northwestern.. . . .... .. ... .. . ........... .. . . . 0 Total. .... .. ...... .. .. .. . . ... .. . ..... ... ....... . . ... 12 Average: one per 7,653 autopsies, approximately 1 per 7,500.
Autopsies
6, 500 15,908 500 5,088 25 ,000 30,0002 5, 770 3 1,0004 2,0725 91,838
Renal symptoms. Various lesions have been described and many symptoms have been attributed to the unilateral fused kidney, but in 17 cases there were definitely no symptoms of renal origin. Most of these silent cases were found accidently. Of the 20 cases reported by Thompson and Allen of the Mayo Clinic, 8 had no renal symptoms, 2 were found at autopsy. The most common symptom is pain. This may be due to 3 possible causes: (1), the anomalous kidney pressing on certain visceral structures; (2), renal lesions; and (3), lesions not associated with the kidney. Of 90 cases in which the symptoms were given, pain was described in 71 as being in: the lumbar region; the back; renal or iliac fossa; pelvic region; the side; or as radiating into the back or leg; or as merely a colicky pain. The pain is usually located in the abdominal quandrant corresponding to the position of the renal mass. In 40 cases a palpable mass was reported in the abdomen. Careful palpation should reveal the kidney in most cases, especially those in which an infection calls attention to the mass. There is no kidney palpable on the side of the ectopic kidney. In 36 cases there were urinary symptoms. Among these, 12 specified pyuria, 14 hematuria, 7 polyuria, and 3 dysuria. In 10 cases nausea was a symptom. In 13 cases 1 ureter crossed the other, and in 1 case the ureter was kinked. This as well as anomalous blood vessels may be an etiological 2 By personal communication from G. J. Rukstinat, M.D., Dept. of Pathology, Rush Medical College. 3 By personal communication from Alan R. Moritz, M.D., Dept. of Pathology, Western Reserve University. 4 By personal communication from Carl V. Weller, M.D., Professor of Pathology, University of Michigan. 5 By personal communication from J. P. Simon, M.D., Dept. of Pathology, Northwestern University Medical School.
UNILATERAL FUSED KIDNEY
565
factor in hydronephrosis and infection. Most of these were found at autopsy where there was no clinical record. In 1 case there was 1 pelvis and 3 ureters, and in another case 3 pelves and 2 ureters (i.e. 1 ureter bifurcated to form 2 pelves). Ureters were constricted in 2 cases, and obstructed in 6 cases. In 7 cases there were coexisting anomalies of the genitalia. The incidence is not very great since the kidneys fuse after the genital system is separated from the urinary system. In 5 cases unilateral absence of the adrenal was noted. Howden in 1887 pointed out that the relation of the suprarenal to the kidney was really one of accident, and when not molded to the top of the kidney the shape is somewhat rounded. Diagnosis. Of 244 cases in which the diagnosis was specified 112 were iiscovered at autopsy, 95 were found by roentgenography or pyelography, and 33 at operation. Four cases have been found in the dissecting room. At the present time, unilateral fused kidney is diagnosed chiefly by pyelography. If the ureteral pyelogram or the x-ray with opaque catheters in situ shows that both ureters pass to one side of the body, i.e. that one crosses the midline, and that the pelves are relatively close together at a point where there is a palpable mass, one can be fairly certain of unilateral fused kidney. The anomalous rotation of the pelves as shown by retrograde or intravenous pyelography is an almost constant manifestation of fusion. It may be called a "triangle" pyelogram, the base of which is formed by the bladder, and the two sides by the ureters running to the kidney at the apex. It resembles a right triangle, the hypotenuse of which is the ureter of the crossed ectopic kidney. Since the ureters enter the bladder normally (with the exception of 3 cases in which the position was slightly altered), cystoscopy is of no diagnostic value. Measurement. All the measurements here are taken only from cases past maturity. The average weight of 7 fused kidneys is 314 gm., which is approximately double the weight of a single kidney. The average length (17 cases) by width (14 cases) by thickness (7 cases) is 16.8 by 7.6 by 4.1 cm., in the longest measurements for each dimension. There is much variation in the relative vertical position of the fused renal mass. It may be found anywhere from the level of the lower thoracic vertebra to a position deep in the pelvis. Subdivisions of the unilateral fused kidney (fig. 6) are as follows: 1. Elongated kidney, pole to pole fusion: The ectopic kidney is always inferior, its superior pole being fused to the inferior pole of the
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HARRY A. WILMER
orthotopic or normal kidney. The hili are usually anterior, but they may be in any arrangement except that characteristic of the "S" kidney. This is the most common variety. 2. "S" kidney, sigmoid, pole to pole fusion: The superior hilus always faces mesially, and the hilus of the inferior ectopic kidney faces laterally. The convex borders of the two kidneys face in opposite directions so that it appears like the Latin "S". In this case both kidneys have completed their rotations, therefore, if the inferior ectopic kidney were transposed to its normal side, the hilus would face mesially. Type:s of
Fusion
Mu1al F\Jsio11
FIG. 6
3. Lump kidney, "kuchenniere," extensive fusion: The 2 kidneys are fused together over a large area so that together they form a lump or mass. Eack kidney loses its distinctiveness, and grossly they may be mistaken for a solitary kidney. The ureter of the ectopic kidney crosses the midline and in this way djstinguishes it from the lump kidneys which are not unilateral fused. 4. "L" kidney. The ectopic inferior kidney is fused to the superior orthotopic kidney so that its lon,g axis is perpendicular to it. The inferior hilus is usually anterior. The "L" kidney resembles the Latin "L", which, however, may be inverted or reversed. 5. Mesial border fusion: The kidneys lie side by side with their mesial
UNILATERAL FUSED KIDNEY
567
borders fused. LaRose reported an unique case in which the kidneys were fused in the form of a ring. 6. Superior kidney ectopic: Fusion may be of any nature but the crossed ectopic kidney is always superior. This is the rarest type. Embryology. In crossed ectopia fusion occurs at approximately the 10 mm. stage of the embryo. The ureteral bud arises from the Wolffian duct at 4.5 mm., penetrates the surrounding mesoderm and at 7 mm., it meets the blastema which then begins its ascent. Hill, Lewis and Papez and others explain the fusion by the fact that the bifurcation of the aorta into the umbilical arteries forms a crotch in which the kidneys may be forced together as they migrate upwards. The arteries acting as a mechanical obstruction tend to bring the right and left blastemas together so that fusion may readily take place. Pole to pole fusion according to Huntington, and Scheiner is the result of the ureteral buds arising at different vertical levels so that the caudal pole of one of the renal blastemas touches and fuses with the cephalic pole of the other. The cephalic kidney usually takes the lead and displaces the caudal organ across the midline. It probably ascends on the side of least resistance. Normally the renal pelves are anterior until migration, at which time the blastema rotates laterally and the pelves medially. The rotation is stopped at the time of fusion, and since the cephalic kidney has ascended farther at this time, it is usually more rotated. For this reason the hilus of the superior orthotopic kidney is frequently mesial, and the hilus of the inferior kidney anterior. Fusion brings about an irregular vascular supply. The kidneys migrate upwards through the mesonephric arteries. As the cephalic blood supply becomes sufficient to supply the ascending kidney, the caudal arteries separate off. Braasch, and Felix have compared the ascent through the mesonephric arteries to climbing a ladder. When the migration stops some blood vessels disappear, and the remaining blood vessels at that time become the permanent vessels. Therefore, anomalous vessels are a natural consequence of unilateral fused kidneys. The superior kidney usually receives one or two arteries from the aorta, and the inferior kidney receives one or two from the aorta or the common iliac depending on the location of the kidney. There is such a wide variation in the vascularization that no general statement can be made. Hyman pointed out that the congenital ectopic kidneys, because of the anomalies of the blood vessels and ureters, are prone to pathological lesions.
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HARRY A. WILMER
Acknowledgment. I am indebted to Dr. F. E . B. Foley for permission to use 2 clinical cases, to Dr. D. James Waterston to use 1 case, and especially to. Dr. E. T. Bell for indispensable advice and criticisms. SUMMARY
Five cases of unilateral fused kidney are reported here together with a series of 94 cases collected from the literature by the author, which brings the total number of reported cases of this anomaly to 286. In about 60 per cent of the cases the kidneys were found on the right side. The anomaly appears to be equally distributed between sexes. The great majority of cases either recognized clinically or at autopsy were in patients under 50 years of age. Unilateral fused kidneys seem predisposed to hydronephrosis and pyelonephritis, but not to other renal lesions. This anomaly falls into six classes: (1) elongated, (2) "S", (3) "L", (4) mesial border fusion, (5) lump, and (6) superior kidney ectopic kidney. · The unilateral fused kidney is found about once in 7,500 autopsies. The fusion of the kidneys is facilitated by a mechanical obstruction at the bifurcation of the aorta into umbilical arteries. These vessels form a crotch which may force together the ascending blastemas. Great variation exists in position, rotation, and vascular supply of the unilateral fused kidney. The most common symptom is pain. The renal mass is usually palpable, especially if involved in a lesion. Frequently there are urinary symptoms. The diagnosis can be easily and accurately made by a pyelogram which will show the ureter of the ectopic kidney crossing the midline to terminate in the bladder normally, presenting a "triangle" pyelogram. REFERENCES ADLER-RACZ, A. VON : (3 cases). Ztschr. f . urol. Chir., 31: 253, 1931. AFANASSJEW, A. N.: (3 cases). Ztschr. f . urol. Chir., 37: 143, 1933. AUGE, A., AND BONNET, A.: J. d'Urol., 18: 493, 1924. BABAINANTZ, M . L. : J. de Radio!. et d'Electr., 18: 561, 1934. BARCLAY AND BAIRD: Lancet, 2: 1169, (Nov.) 1935. BARRY, R . T., AND GARVEY, F . K.: J. A. M.A., 94: 1232, 1930. BEER, E ., AND FERBER, W. L. F.: (14 cases). J. Urol., 38: 541, 1937. BESSESEN, D. H. : Ann. Surg., 98: 314, 1933. BORCHARDT, M . : Med. Klin., 28: 439, 1932. BRAASCH, W. F.: Ann. Surg., 66: 726, 1912. CANELLI, A. F.: Riv. di din. pediat., 16: 577, 1918. CANIGIANI, T .: Rontgenpraxia, 8: 103, 1936. CARLETON, A.: J. Anat., pt. 2, 71: 292, 1937.
UNILATERAL FUSED KIDNEY CARRARO, N.: Morgagni, 56: 300, 1914. CIFUENTES, P., AND ONTANON, G.: J. d'Urol., 36: 343, 1933. CRAISIN: Arch. gen. de chir., 4: 455, 1913. DrNATILI, L.: Arch. Ital. di chir., 37: 104, 1935. DUNAVANT, B. N.: South. Med. J., 12: 689, 1919. ErSENDRATH, D. N., AND RoLNicK: Textbook on Urology, Phila., J. B. Lippincott & Co., 1934. ELVING, A.: Ztschr. f. urol. Chir., 19: 67, 1926. FARCAS, I., AND REVESZ, V.: (2 cases). Rontgenpraxis, 2: 759, 1930. GENOUVILLE AND SAQUIN: J. d'Urol., 18: 519, 1924. GERARD, G.: J. de l'anat. et de la physiol., 41: 241, 411, 1905. The following cases were collected by Gerard: Cathelin (1898). Reed (1845). Chambelent (1895). Sandifort (1793). Chassaignac (1832) Stoicesko (1883). Chassaignac (1840). Tanton (1901). Horne (1793). Tesson (1895). Hunter (1785). GODARD: Cited from HEINER, G. (TANDLER): Folia Urolog., 3: 186, 1909. GOTTLIEB, J. G.: J. d'Urol., 24: 139, 1927. GRUBER, G. B.: (2 cases). Wein. med. Wchnschr., 74: 2007, 1924. GuELLEllHN, A.: (Reported by Dossot). J. d'Urol., 43: 81, 1937. GUIMPELSON, E. I., AND FATIANOVA, L. N.: (4 cases). J. d'Urol., 40: 333, 1935. HACHEZ: Deutsche med. Wchnschr., 60: 1243, 1934. HESS, E.: J. d'Urol., 22: 667, 1929. HERMAN, L.: Atlantic M. J., 37: 1009, 1934. HODGE: Am. J. M. Sc. N. S., 6(): 455, 1870; cited from BALLOWITZ in: Virchow's Arch. f. path. Anat., 141: 309, 1895. HUNTINGTON, G. S.: (2 cases). Harvey Lectures, 1906-07, Genito-Urinary Tract: Variations. Phila. & London, J. B. Lippincott and Co., 1908, p. 222. JACOBS: Glasgow M. J., 1.20: 41, 1933. KALBERG, W.: Anat. Anzeiger., 74: 117, 1932. KRETSCHMER, H L.: Surg. Gynec. and Obst. 40: 360, 1925. The following cases were collected by Kretschmer: Bieberbach (1924). Hyman (1922). Kidd (1910). Bissel (1911). Boody (1899). Lowsley, Kingery, and Clarke (1924). Bugbee (1911). Lund (1919). Mennacher (1909). Bugbee (1911). Carrieu and DeRouville (1887). Meyer (1913-14). Day (1924). Pohlman (1904-5). Dennis (1904). Rathbun (1913). Felty (1918). Stewart and Lodge (1923). Gruner and Fraser (1911). Thompson (1913). Gruner and Fraser (1911). Wilcox (1921). Huntington (1906-7). Winternitz (1908). Huntington (1906-7). Wilhelmi (1920-21). Hyman (1922). Kretschmer (1925). KuNDRAT: (2 cases). Wein. med. Wchnschr., p. 109, 1886; cited from PAGEL. LMrnors, F.: Bruns' Beitr. z. klin. Chir., 149: 501, 1930. LEMBERGH, W.: (4 cases). Ztschr. f. urol. Chir., (Ref.), Hi: 66, 1924. LISA, J. R., AND LEVINE, J.: (2 cases). N. Y. State J. M., 32: 395, 1932. MAISEL, I.: (2 cases). British J. Urol., 9: 380, 1937. MARTIUS, K.: Frankfurt. Ztschr. f. Path., 12: 47, 1913. MATTIOLI: Gesellsch., Ztschr. f. urol. Chir., 19: 250, 1926. MAYER, A. J., AND NELKEN, A.: J. A. M.A., 57: 1262, 1911. MELOY: Grace Hosp. Bull., 3: 16, 1918. Cited from SEXTON. MEYER, 0.: Cited from DORLAND in: Surg. Gynec. Obst., 13: 303, 1911. McDONALD, P. W.: Lancet, 1: 979, 1885. MINTZ, E. R., AND STEWART, J. D.: New England Med. J., 206: 1282, 1931. MoURE, P., AND MARCY, J.: Mem. Acad. de chir. Paris, 63: 705, 1937. NAUMAN: Diss., Kiel, 1897; cited from KRETSCHMER. NEUWIRT, K.: ReL Ztschr. L uroL Chir., 15: 328, 1924.
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NEWMAN, D.: Cited from DORLAND in: Surg. Gynec. Obst., 13: 303, 1911. O'CoNOR, V. J. : The Anomalies of Kidneys, in: The Cyclopedia of Medicine, Vol. VII, Phila., F . A. Davis and Co., 1934, p. 741. OGSTON, F.: Brit. M. J., 1: 591, 1879 (also reported by Braxton-Hicks}. PAPIN AND PALAZOLLI: Ann. des. Mal. de Org. Genito-Urin., 27: 1681-1710, 1762-1786, ' 1842-1891; 1909. The following cases were collected by Papin and Palazolli: Albarran (1908). Laube (1722). Bachhammer (1879) . McMurrich (1898) . Bachhammer (1879). McNaughton (Jones) (1866). Bachhammer (1879). Morris (1901). Barth (1853). Palma (1891). Birmingham (1890). Pannorlus (1654). Bolintinean and Pastia (1907). Picard (1872). Portal (1804). Broesika (1884). Cholzow (1907). Poulalion (1890). Coupland (1877). Powell (1883). Rufz (1883). Dickenson (1895). DuBois (1907). Schmerber (1895). Duckworth (1869). Schwalb (1896). Duvivier (1730). Steiner (1901). Godard (1855). Stoquart (1879). Greenfield (1876). Stoquart (1893). Gronnerud (1907). Stolz Gruber (1887). Strube (1894). Hill (1906). Sutherland and Edington (1898) . Sutherland and Edington (1898). Hillier (1864). Tandler (Heiner) (1907). Houze! (1898). Huebschman (1908). Tully (Vaughan) (1897). Israel (1908). Turner (1886). Israel (1908) . Verocay (1907). Karltreu, B. von (1898). Wehn (1895). Kelly (1868). Weibel (1908) . Kruse (1890). Weisbach (1867). Kruse (1892). Wilcox (1880) . Kuster (1895). Zondek (1903). LaRoche (1895). Zondek (1903). PFITZER, H.: Ztschr. f. urol. Chir., 67: 772, 1930. PIERSON, L. E.: J. Urol., 28: 217, 1932. The following cases were collected by Pierson: Bieberbach (1924). Lavroff (1928). Caulk (1923). Makai (1926) . Donohue (1926). Pedroso (1930). Dourmashkin (1924). Pierson (1932). Hess (1926) . R athbun (1927). Ide! (1927) . Saikin (1926). LaRose (1924). Sexton (1925). Lazarus (1927). Spurr (1927). PLESCHNER, H. G.: Wein. klin. Wchnschr., 42: 1581, 1929. RATNER, S.: (2 cases). Ztschr. f. urol. Chir., 29: 531, 1930. RUMPEL, 0 . : Zentralbl. f . Chir., 69: 1290, 1932. RussEL, J . C. : J. Anat. and Physiol., 19: 1885. RUTHARDT: Arch. f . Anat. u. Physiol., p. 599, 1827. SAWYER, C. F.: Proc. Inst. Med. Chicago, 10: 134, 1934. SCHILLING, F. : Arch. f. Gynak., 114: 428, 1920. SCHILLINGS, M ., BRENEZ, J., AJ:
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STEIN, A. : Am. J. Obst., 73: 449, 1916. The following cases were collected by Stein: Colmer (1911) . Smith and Gammon (1901). Dumitreamu (1908). Stein (1916) . Geiss (1899). Strube (1894). Illyes (1908) . Winternitz Lalmohon (1876) . Zuckerkundl (1909). Pashkis (1910). SuRRAco, A.: J. d'Urol., 23: 411, 1927. THOMPSON, G. J., AND ALLEN, R. B.: (20 cases) . S. Clinics North America, 14: 729, 1934. TOWNSEND, J. M., AND FRUMKIN, J.: Urol. and Cutan. Rev., 41: 324, 1937. The following cases were collected by Townsend and Frumkin: Bethea and Peterson (1935). Lequeu (1930) . Bethea and Peterson (1935). Lindner (1935). Bethea and Peterson (1935). Masciottra (1935) . Bordas (1927). Oraison (1912). Bugbee and Losee (1919) . Pagel (1922). Bugbee and Losee (1919) . Pagel (1922) . Capua (1935). Pagel (1922). Capua (1935). Pagel (1922) . Carraro (1929). Palmer (1924). Chenault (1935) . Quinby (1925) . Fasiani (1926) . Ratner Glesne (1934) . Rejsek (1924). Hess (1933) . Stincer (1914). Homma (1930) . Strong (1900) . Iscenko (1933). Townsend and Frumkin (1937) . Iscenko (1933) . Willis (1931) . VouTEIN: Rev. de Chir., Paris, 42: 447, 1910. ZEISS, L., AND B0EMINGHAUS, H .: (6 cases). Ztschr. f . urol., 28: 577, 1934.
REPORT OF FIVE CASES AND A REVIEW OF THE LITERATURE
HARRY A. WILMER From the Department of Pathology, University of Minnesota
"Crossed ectopia with fusion," "crossed dystopia with fusion," and "unilateral fused kidney" have been used almost interchangeably in the literature to describe the same rare congenital renal anomaly where both kidneys are fused on one side of the midline. The term "unilateral fused kidney" is not strictly correct when the kidney is prevertebral in position, but it is accurate enough for practical purposes, and has the advantage of being readily understood. Unilateral fused kidney must not be confused with solitary congenital kidney (unilateral aplasia) in which only one kidney is present. When solitary congenital kidney occurs with complete ureteral duplication both ureters enter the bladder on the same side, while in the unilateral fused kidney each ureter enters the bladder on its corresponding side. A review of the statistics dealing with this anomaly shows that before the introduction of pyelography by Voelker and Von Lichtenberg in 1906 all cases were found either at autopsy or operation. Since the introduction of pyelography, however, a great many cases have been recognized clinically. Over 54 per cent of the 286 cases reviewed in this paper were reported since 1921. It appears that this anomaly is not as rare as indicated in earlier surveys based largely on autopsy findings. Crossed ectopia may occur without fusion. Pagel found 14.5 per cent unfused in a series of 55 cases of crossed ectopia. A classification of the fusion anomalies is presented here, in which the position of the ureters is the basis of differentiation. The four basic groups are given below, and further subdivisions of the unilateral fused kidney group will be given later. 1. Horseshoe kidney: Horseshoe kidneys are fused at their inferior poles by an isthmus of renal or connective tissue. In rare cases the superior poles are fused instead. They are generally prevertebral in position, and lie below the level of the origin of the inferior mesenteric artery. 2. "L" shaped kidney: This is a transitional form between typical 551
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HARRY A. WILMER
horseshoe kidneys and unilateral fused kidney. When the ureter of the ectopic kidney crosses the midline the anomaly may be classified as unilateral fused kidney. When neither ureter crosses the midline, the "L" kidney is merely a variation of the horseshoe kidney. This is described further under subdivisions of unilateral fused kidney. 3. Unilateral fused kidney: The ureter of the ectopic kidney crosses the midline of the vertebral column. The entire renal mass is located largely on one side of the midline. The ectopic kidney is usually inferior to the orthotopic kidney, and the fusion is generally pole to pole, but a great many variations have been reported. 4. Miscellaneous: This includes all the cases that do not fall definitely in groups 1, 2, or 3. The lump kidney is usually in this class but may fall in group 3. When it is in group 4, it may be situated either prevertebral or in the pelvis, but neither ureter crosses the midline. Case 1 (fig. 1). The specimen was found in the dissecting room of the Department of Anatomy at the University of Minnesota. The body was that of a male 47 years of age. The clinical diagnosis of acute glomerulonephritis was verified by microscopic examination of the kidney by Dr. E. T. Bell. The right kidney was ectopic, being superimposed on the left orthotopic kidney. The ureters were unobstructed, and there was no evidence of hydronephrosis. The renal mass was situated chiefly on the left side, although the lower part was prevertebral. It extended from the top of the second to the lower part of the fifth lumbar vertebra, and measured in its longest dimensions 13.5 by 10.5 by 5 cm. The anterior surface showed fetal lobulations and the posterior surface was smooth. There was a clear line of demarcation between the kidneys on their anterior surfaces where the upper pole of the left kidney could be seen projecting above the right kidney. The posterior surface of the renal mass conformed to the psoas muscle, the left common iliac artery, and part of the vertebrae. The kidney weighed approximately 275 gm. The left adrenal was in the normal position above the kidney but not connected with it, while the right adrenal was situated normally though no kidney was below it. The right hilus was anterior, and the right ureter crossed the fifth lumbar vertebra to terminate at its normal position in the bladder. The left hilus was anterior and slightly lateral. The left ureter, likewise, terminated in the bladder normally.
The left or orthotopic kidney received 2 arteries directly from the left side of the aorta. The second of these two supplies both kidneys. The
UNILATERAL FUSED KIDNEY
553
left kidney also receives two more branches from the left common iliac .artery. One of the latter penetrates the middle of the posterior surface of the kidney directly above the left common iliac artery. The right kidney receives 2 arteries, one from the aorta, the other from the middle of the right common iliac artery. This branch passes under one of the
FIG. 1
fetal lobulations and penetrates the renal parenchyma near the right hilus. The left kidney is drained by 3 veins. The upper one begins at the left hilus and is joined by a small branch from the projecting part of the left kidney, and also by the left spermatic vein; this vein empties into the
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HARRY A. WILMER
left side of the vena cava. The remaining 2 veins correspond to the 2 lower arteries from the left common iliac artery to the left kidney. The right kidney, is, likewise, drained by 3 veins. The first vein begins at the right hilus and empties into the left side of the vena cava. The second vein corresponds to the artery to the right hilus. It joins the vena cava slightly on the right side. The third vein arises from the lower part of the kidney below the hilus and empties into the left common iliac vein. There is no corresponding artery. Case 2 (From the Pathology Department, University of Minnesota): A female, aged 37, revealed a unilateral fused kidney at autopsy. Death was attributed to tuberculous enteritis. The left kidney was at the second lumbar vertebra in the midline. Its pelvis stretched from the center laterally down
FIG. 2
the midline to the right, and then coursed back to terminate in its normal position at the trigone. The right kidney is in approximately normal position, but its lower pole is fused with the left kidney. The kidney mass weighed 300 gm. (A 28-1714). Case 3 (By personal communication from Dr. Frederic E. B. Foley): A female 20 years of age (in 1926) complained of pain in the right epigastrium of 2 years' duration. The indigo-carmine test indicated normal renal function; cystoscopy revealed a normal bladder, and there was no record of hematuria. There was a palpable mass in the lower right quadrant, its upper limit being a little above the iliac crest, its mesial margin almost in the midline and extending as far down as the promontory. It was fixed and could not be forced up into the flank. There had been some polyuria for 2 or 3 months prior to consultation. In the traced pyelogram (fig. 2) it may be seen that the ureter of
UNILATERAL FUSED KIDNEY
555
the ectopic kidney is kinked and both pelves are dilated, A diagnosis of unilateral fused kidney was made from the pyelogram. Case 4 (By personal communication from Dr. Frederic E. B. Foley): A female 18 years of age, complained of pain in the left upper abdominal quadrant of 3 years' duration. There were no urinary symptoms, and no nausea,. The pyelogram showed both renal pelves to the right of the lumbar centra, and abnormally rotated (fig. 3). At operation the lower pole of the right kidney
FIG. 3
was found fused to the upper pole of the left crossed ectopic kidney (fig. The 2 kidneys were connected obliquely across the midline, the upper pole being well to the right lateral side of the vertebral column. The 2 kidneys were separated by a well-defined isthmus which measured about 1.5 thick x 4 cm. wide. The renal mass lay upon the vena cava, the right iliac artery, and the right iliac vein. The ureters appeared normal and there was no apparent obstruction. Dr. F. E. B. Foley performed a right nephropexy, dividing the
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HARRY A. WILMER
isthmus and freeing the left kidney by blunt dissection. The inferior left kidney was pushed to the left side. There was an uneventful convalescence. A post-operative pyelogram at the time the patient was discharged from the hospital showed the right pelvis up in excellent position. Since leaving the hospital the patient has been seen a number of times in the out-patient department and reports complete relief of the abdominal pain present before operation. Case 5 (By personal communication from Dr. D. James Waterson, The Hospital for Sick Children, Great Ormond St., London): A unilateral fused kidney was found in a child, 5½ years of age, dead of diarrhea and acute bron-
FIG. 4
chopneumonia. The renal condition was not suspected during life. The autopsy revealed a right kidney situated normally, fused by its lower pole to the upper pole of the left kidney. The junction is marked on the anterior surface by a deep groove transmitting the right ureter. The left suprarenal capsule is separated from the kidney, which is lower than normal.
Source of cases reviewed. Renal anomalies have always been a fascinating subject. Aristotle mentioned a unilateral kidney defect. The :first case of unilateral fused kidney was reported by Pannorlus in 1654. In this paper 286 cases have been reviewed. They are. taken from the sources found at top- of following page: 1 1 We have found 29 cases reported in the literature as crossed ectopic kidneys, in which it is not stated whether there was fusion, or the original papers were inaccessible to us. Cases reported by Guillemin and Muller are listed in the 1937 cumulative index as crossed ectopic fused kidneys. Since the original papers are inaccessible to us these cases are not included.
557
UNILATERAL FUSED KIDNEY Year
Gerard .. .. ..................................... . . . . ....... . Papin and Palazolli .............. . .......... . . . . . . .. .. ...... Stein ... ..... ..... .. . ..... . .. .. . . .... .. ........ ... . .. .. .. .. Kretschmer. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pierson ... .. ............ .. . . .. . .... . ...... ... . . .... . . . .. ... . Thompson and Allen ..... ... . . . ... . .. . . .. ....... . . .. . . .. . .. . Townsend and Frumkin .. . . .. ... ............... . .. ... ... .... Beer and Ferber .................. .... ...... ... .. .. . . ........ This series ... . ............. . ... .. . . ...... . . .. .. ... .. . ...... Total.. . .... .. .. . ... ... . .. . . ...... ..... . .. .. ... . . . . . . . .
1905 1909 1916 1925 1932 1934 1937 1937 1938
Cases
11 60 11 28 16 20 32 14 94 286
For the purpose of this review, the cases collected from the literature and not given in previous compilations will be given in abstract form. Sex. Of 225 cases in which the sex was recorded, there were 125 males, and 100 females. These :figures suggest a predominance in the male; however, 116 cases out of the 286 were diagnosed at autopsy. It is the usual experience that more autopsies are performed on males than on females. Therefore, the sex difference is probably not significant. Side e.ffected. Of 239 cases in which the position of the kidney was mentioned, there were 142 on the right and 90 on the left. In seven cases the kidney mass was definitely prevertebral, and in one instance both ureters crossed the midline. Age. The age distribution graph (fig. 5) indicates by white columns the age at which the fused kidney was discovered clinically, and by black columns the age at which death occurred, in cases diagnosed at autopsy. The mode of the cases diagnosed in living patients is in the age group 21 to 30. The great majority of the cases recognized clinically were in patients under 50. In the autopsy series the cases are most frequent in the 2nd, 3rd, and 4th decades. It is interesting to note that death occurred at an early age in many of these cases. The great majority of the cases diagnosed at death were, likewise, under the age of SO. Associated renal lesions. The authors are often not clear as to the severity and the character of the coexisting renal lesions. In 137 cases in which there was a record of the renal pathology; infection of some kind was noted in 40 cases, and a diagnosis of nephritis was made in 14. Hydronephrosis was present in 26 cases and dilation of the ureter or pelvis was noted in 21. Calculi were found in 14 instances, nephroptosis was reported in 7, and tuberculosis in 7 cases. Miscellaneous lesions were described in the remaining. Unilateral fused kidneys seem predisposed to hydronephrosis and pyelonephritis, but not to other renal lesions.
558
HARRY A. WILMER TABLE
1
.!.ti i:.lS UOI
AUTHOR AND YEAR REPORTED
~~
DIAGNOSIS BY :
AGE
tl" .,14
.,""'.. "'"' "'"' ~:z;
><
- - - -"'"'
gi
<
RENAL
~
COMPLICATION
REMARKS
t'.l
i!l
"
< ~
ill ill - --
--
years Adler-Racz (1931)
?
?
Operation
L
Tuberculosis of lower kidney
Adler-Racz (1931)
25
F
Pyelography
L
Pyoncphrosis,
Adler-Racz (1930)
50
M Pyelography
L
Afanassiew (1933) Afanassjew (1933)
25 22
F F
Pyelography Pyelography
Afanassjew (1933)
45
F
Pyelography
X
?
X
X
L L
X right; tuberculosis, left Hydronephrosis, X left Nephroptosis X ? X
L
Bilateral pyelec- X
X
Pain of 5 years' duration Polyuria; pain
?
?
Death due to lung
?
?
?
? ?
? ?
Pain of 5 years' duration
tasis
Auge and Bonnet (1924)
?
F Autopsy
Babainantz (1934)
23
F
Pyelography
L
Barclay and Baird (1935) Barry and Garvey (1930)
?
?
?
28
F
Pyelography
L R
R
abscess; ''S'' kidney
Right
hydro-
nephrosis
Bessesen (1933)
Adult
M Pyelography L confirmed at
Braasch (1912)
X
calculus, upper
right kidney
operation
Borchardt (1932) Braasch (1912)
Bilateral hydro- ? nephrosis Pyonephrosis and X
54
F
?
?
and autopsy Pyelography L Pyelography L
?
?
Pyelography
L
due to post opera-
tive hemorrhage Hydronephrosis, left Hydronephrosis,
? ?
? ?
?
?
?
?
?
?
?
?
X
X
left Canelli (1918)
?
Canigiani (1936)
24
Carleton (1937)
Aged
Carraro (1914)
32
M Autopsy
?
Pyelography
L
R
M Dissecting room
L
F X-ray
R(?)
Pelvic lavage cleared up symptoms Nausea and vomiting; pain. Death
Hydronephrosis, right Hypoplasia, left
"L" kidney; lower left resected "L" kidney; lower left resected 325 gm.; 15.5 by 9 by 3.4 cm. Death due to accident
Dilation of both ureters; right ureteral orifice more posterior-central than left. Multiple bladder di verticula 14 cm. long kidney Polyuria; no macroscopic lesion seen
at operation; 15-17 cm. long Cifuentes and Ontanon (1933)
43
Craisin (1913)
?
F
?
Pyelography
Autopsy
L
?
X
?
X
?
Pain of one year dura-
tion; ectopic kidney superior N ephrectomy of entire kidney; death due to anuria
559
UNILATERAL FUSED KIDNEY TABLE
I-Continued
'!il
~" UOI
.!Pl AUTHOR AND YEAR REPORTED
oO
AGE
DIAGNOSIS BY:
tl ...
w"' A[j w ··
REMARKS
..
Ull,l
- - - -"'
~
[j ~
"''" ~:,;
><
!al
"'"'
RENAL
COMPLICATION
~ ~ - --
--
years
DiNatili (1934)
?
F
31
F
X-ray
R
Pyonephrosis
X
?
Mesia! fusion; pyonephrotic kidney
?
?
No renal symptoms;
resected
Dunavant (1919)
Egyedi (1935) Eisendrath (1934)
45
Elving (1926) Farcas and Revesz (1930)
22 46
Operation; L verified by pyelography M R M Probable au- R topsy F R M Pyelography R
Farcas and Revesz (1930)
60
M Pyelography
Genouville and Saquin (1924) Godard (1860)
?
?
Pyelography
?
?
Autopsy (?)
?
diagnosis
acci-
dental Perirenal abscess
N ephroptosis
R
? ?
? ?
Superior kidney ectopic
?
X
X
?
X
X
?
?
Nephropexy formed
?
?
Anomalies
Function test normal diagnosed Author horseshoe kidney; ureter crossed midline Indigo-carmin appeared from right 14 m in.; left 12 min.
?
Hydronephrosis
R
perof
the
genitalia Gottlieb (1927) Gruber (1924)
F F
Pyelography Autopsy
L
4
F
Autopsy
R
25
F
29
F
Laparatomy: R confirmed by pyelography Pyelography L
F
Pyelography Pyelography
30 3½
Gruber (1924)
? ?
? ?
Left ureter dilated
?
?
12.5 by 10 by 5.5 cm. "S" kidney; death due to post-operative bronchitis and
Hypoplasia, right
?
?
Heminephrectomy
X
?
R
X
X
L
X
?
Hematuria; nausea
Nephropexy; intense pain 4 lumbar months duration Heminephrectomy Pyelocystostomy. This appears to be a pelvie horseshoe kidney; both ureters cross the midline; i.e. both kidneys are crossed ectopic with their inferior poles fused
?
''Kuchenniere''
bronchiectasis
Guellemin (1937)
Guimpelson and nova (1935) Guimpelson and nova (1935) Guimpelson and nova (1935) Guimpelson and nova (1935) Hachez (1934) E. Hess (1929)
FatiaFatia-
32
Fatia-
35
F
Fatia-
42
M Pyelography
L
X
X
20 9
M Pyelography M
L X Both Extreme hydro- X nephrosis right
? ?
560
HARRY A. WILMER TABLE
!-Continued
6tl
i;ls
AUTHOR AND YEAR REPORTED
s: .. ""' oo DIAGNOSIS BY:
AGE
><
~
--- -
tl I;!
"'"'<
RENAL
.,~~.. "'"' "'"' ez
COMPLICATION
?
Hydronephrosis right ectopic kidney. Left ureter dilated
~
i!i
.:
"'... "' ...<.:
REMARKS
"' - --
years
Herman (1934)
10
Hodge (1870) 25
Jacobs (1933)
Kalberg (1932) Kundrat (1886) Kundrat (1886) Landois (1930)
55-60 ? ?
?
Pyelography
?
Autopsy
L
M Pyelography
R
M Autopsy
L R R L
?
? ?
Hydronephrosis right (lower)
?
?
Hematuria; dysuria; prevertebral, mostly on left
?
?
X
?
1 pelvis; 3 ureters; 2 arteries Severe pain of 3 months' duration; right kidney resected, left rotated to normal position
? ? ?
? ? ?
X
X
?
?
Extensive fusion
42
F F
Pyelography
Lembergh (1924)
1 mo .
F
Autopsy
Lembergh (1924) Lembergh (1924) Lembergh (1924) Lisa and Levine (1932)
4mo. 52 1 day ?
F Autopsy M ? M Autopsy M Autopsy
L L L R
? ? ? ?
? ? ? ?
Lisa and Levine (1932)
?
F
Autopsy
R
?
?
Maisels
25
M Pyelography
R
Hydronephrosis, X right; calculus, right pelvis, and left ureter
?
Pyelitis
X
X
?
?
?
?
Extensive anomalies of genitalia: ectopic kidney superior ? . No renal symptoms
Maisels
37
F
Pyelography
?
L
M Autopsy
R
30
M Laparatomy
L
Martius (1913)
45
F
?
L
?
Mattioli (1926)
?
F
Pyelography
R
?
McDonald (1885)
Mayer and (1911)
Nelkman
?
Autopsy report: acute nephritis, traumatic infarcts of kidney
Abdominal pain: function test norma! ''Kuchenniere'' 3 pelves, 2 ureters, 1 artery
Separate artery for
each kidney Separate artery for each kidney Recurrent attacks of right renal colic and hematuria; calculus in left ureter removed at operation. Other stone
?
passed later Recurrent attacks of pain for 3 years Death due to epileptic dementia kidney weight, 356 gm. Kidney ruptured by fall on a nail; death due to anuria; 400 gm. weight
561
UNILATERAL FUSED KIDNEY TABLE
!-Continued
'tl
~<>
"" A UTHOI< AND YEAR
REPORTED
AGE
DIAGNOSIS BY!
ii:04 oo tl'-<
r4f:1
.,A"'.. "'"' "'"' ~z
><
- - - -"'"' ...
35
Meyer (1908) 3 wks. Mintz and Stewart (1931) 17
....
~
-?
?
?
L L
? ?
? ?
L ? L(?) L R Hydronephrosis L Cystic, hydronephrotic, atrophied right kidney R(?)
? ? ? ? ? ?
? ? X ? ? ?
X
X
?
X
X
?
?
?
X
X
Pain of 2 years' duration; right kidney resected
?
?
2 arteries; 2 veins; 20
?
?
R
?
?
M Autopsy
Moure and Marcy (1937) Nauman (1897) Neuwirt (1924) Newman (1898) O'Conor (1934) Ogston (1879)
22 ? 28 26
? Autopsy M Operation; confirmed by pyelography F Pyelography ? Autopsy M Pyelography ? Autopsy M Pyelography F Autopsy
Pfitzer (1930)
45
M Pyelography
Pleschner (1929)
20
?
Pyelography
L
Ratner (1930)
35
F
?
L
Ratner (1930)
25
M Pyelography
R
Rumpel (1929)
26
F
Pyelography
R
Russel (1885)
55
M Autopsy
Ruthardt (1827)
40
M Autopsy
Sawyer (1934)
18
M Roentgeno-
?
REMARKS
f:l
.~ -
years
Meloy (1918)
"'"'< :,a
RENAL COMPLICATION
Hydronephrosis, both kidneys Pyelitis, left Pyelitis, right upper ureter dilated Hydronephrosis, right (which pressed on aorta)
R
?
Pyelectasis
;;'.;
gram
Schilling (1920) Schillings, Benez and Lapiece (1936) F. W. Smith, cited by Peterson (1919)
40 35
F Autopsy M Pyelography
L R
? X
? ?
24
M Pyelography
L
?
X
Sorrentino (1936) Surraco (1927)
26 38
M Pyelography ? Pyelography
R L
Tuberculosis N ephroptosis
X No X ?
285 gm.; 10 by 5 cm. 2 arteries; 2 veins '·L" kidney No symptoms of renal origin
Operation Cited by Kretschmer "'S" kidney
Left kidney resected No left adrenal; anomalies of genitalia Slight pain 3 years' right duration ; ureter divided in two at 4th lumbar vertebra; function test normal Right kidney resected Hematuria, subacute cystitis Dysuria
by 8.1 cm . Death due to psoas abscess; 5 arteries, 4 veins; 10 by 12.5 cm. Severe trauma to kidney Anomaly of genitalia
No renal symptoms. Function test norma! Vomiting
562
H ARRY A. WILMER T ABLE
!-Concluded Q !il
£lo AUTHOR AND YEAR
REPORTED
""' ~~
AGE
DIAGNOSIS BY:
tl'"' ,-f;j
.... "''"' "'"' "'~z'"
~
l:l
Schramm (1932)
46
M Laparotomy
R
Schramm (1932)
?
M Pyelography
L
Voutein (1910)
?
?
Autopsy
Zeiss and Boemingh aus (1934)
9
F
Pyelography
L
Zeiss and Boeminghaus (1934) Zeiss and Boemingh aus (1934) Zeiss and Boemingh aus (1934)
9
F
Pyelography
L
42
F
Pyelography
R
34
F
X-ray
R
Zeiss and Boeminghaus (1934) Zeiss and Boemingh aus (1934)
28
F
Pyelography
L
22
F
X-ray
L
Wilmer
47
M Dissection
R
Wilmer
37
F
Autopsy
Wilmer (Foley)
20
F
Wilmer (Foley)
18
F
Wilmer (Waterston)
5- 6mo. ?
?
:,i
REMARKS
['.l
.:"'
~ -
:;j
Bilateral pyelec- ? tasis; right kidney dilated Pyelectasis, ? right; hydronephrosis and infection left ?
?
c.>
-- years
"' "' <
RENAL COMPLICATION
.. ?
N ephrectomy of ec topic kidney
?
Death due to anuria following complet e nephrectomy "S" kidney
?
?
?
?
X
?
?
?
X
?
?
?
?
?
13.5 " 10.5 x 5 cm.
L
?
?
Pyelography
L
X
Pyelography
L
Bila teral pyelec- X tasis X
Autopsy
L
?
?
Death due to t uber culous enteritis P olyuria, left urete kinked P ain of 3 years' dura tion; isthmus wa divided and righ per nephropeJ
Pye!itis right; obstructed right ureter
Pyelectasis and infection right H ydronephrosis and calculus, left I nfection in both kidneys Infection in both kidneys; dilated right calyces; nephroptosis Acute glomerulonephritis
X
" S" kidney; no rena symptoms Pain in back of 3 years' duration Symptoms of 1 year duration
H ema turia symptom 9f 3 years' duration Symptoms of 4 years duration: nephro pexy a t 22; still sick at 37
Surgery. Hemi-nephrectomy was performed in 21 cases because of hydronephrosis, infection, tuberculosis, etc. N ephropexy was performed 13 times, lithotomy 9 times. The diagnosis was made at laparotomy 11 times. In two of these cases the failure to make a clinical
UNILATERAL FUSED KIDNEY
563
diagnosis was due to the fact that a unilateral pyelogram was made. Decapsulation was performed 3 times. In 8 cases death was attributed to anuria. In 5 of these cases the renal condition was not recognized preoperatively, and complete nephrectomy was performed. In 1 case an anomalous blood vessel was severed to relieve the symptoms. It is possible that the anomalous blood vessels may cause obstruction. In 2 obstetrical cases the position and size of the renal mass offered an obstruction that necessitated caesarian section. In one of these cases the woman had previously given birth to 6 children by normal confinements; in the other it was the first child. The frequent location of the fused kidney at the promontory or in the pelvis makes h a factor of importance '15 _AvEllEMfL
CONtJ/r/0N /l.EC06NIZEI>:
¥0
DOHi/AT/ON OIi X-RAY
35
t
10
;S
Z5
~
~
~
~
IAtt~sr
\
.J POINT SMOOTHING
\/
''
AGE
GROUPS
Fm. 5
in obstetrics. In many cases normal delivery with no dystocia is reported. Cause of death. Of 54 cases in which the cause of death was given, 18 were attributed to some pulmonary involvement. Of these, 7 were pulmonary tuberculosis, and 8 pneumonia. Three of the 18 cases were complicated by heart disease. In 7, death was due to nephritis, 3 of these were complicated by heart disease. Tuberculous enteritis was the cause of one death. Pulmonary and renal deaths together account for 25 of the 54 deaths. The remaining 28 contain 11 cases of post-operative death from peritonitis, hemorrhage, anuria following complete nephrectomy, etc.
564
HARRY A. WILMER
Incidence. The frequency of unilateral fused kidney is indicated by the following statistics: A ulhor
Cases
Stewart and Lodge. .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Morris .... .. . ... . .. . . . ...... . .... . . .. .. . . . .. . .. . . ... .. .. 1 Boody. .. . ... . ...... . ............ ... ... . ..... . . . .... .. .. 1 Nauman. . .. .. ...... ... .. . . ... .... ... . .. .. .. .. ... . .. .... . 1 University of Minnesota ..... . .... .. . . . . . . . .. . .. .... . ... .. 1 Rush Medical College ...... . ...... .. .. . . . ... ... . ... . .. ... . 7 Western Reserve University. ......... .. . ... .. .. ........ . .. 0 University of Michigan ........... . .. . . . ... . . ... ... . . . . . . . 0 University of Northwestern.. . . .... .. ... .. . ........... .. . . . 0 Total. .... .. ...... .. .. .. . . ... .. . ..... ... ....... . . ... 12 Average: one per 7,653 autopsies, approximately 1 per 7,500.
Autopsies
6, 500 15,908 500 5,088 25 ,000 30,0002 5, 770 3 1,0004 2,0725 91,838
Renal symptoms. Various lesions have been described and many symptoms have been attributed to the unilateral fused kidney, but in 17 cases there were definitely no symptoms of renal origin. Most of these silent cases were found accidently. Of the 20 cases reported by Thompson and Allen of the Mayo Clinic, 8 had no renal symptoms, 2 were found at autopsy. The most common symptom is pain. This may be due to 3 possible causes: (1), the anomalous kidney pressing on certain visceral structures; (2), renal lesions; and (3), lesions not associated with the kidney. Of 90 cases in which the symptoms were given, pain was described in 71 as being in: the lumbar region; the back; renal or iliac fossa; pelvic region; the side; or as radiating into the back or leg; or as merely a colicky pain. The pain is usually located in the abdominal quandrant corresponding to the position of the renal mass. In 40 cases a palpable mass was reported in the abdomen. Careful palpation should reveal the kidney in most cases, especially those in which an infection calls attention to the mass. There is no kidney palpable on the side of the ectopic kidney. In 36 cases there were urinary symptoms. Among these, 12 specified pyuria, 14 hematuria, 7 polyuria, and 3 dysuria. In 10 cases nausea was a symptom. In 13 cases 1 ureter crossed the other, and in 1 case the ureter was kinked. This as well as anomalous blood vessels may be an etiological 2 By personal communication from G. J. Rukstinat, M.D., Dept. of Pathology, Rush Medical College. 3 By personal communication from Alan R. Moritz, M.D., Dept. of Pathology, Western Reserve University. 4 By personal communication from Carl V. Weller, M.D., Professor of Pathology, University of Michigan. 5 By personal communication from J. P. Simon, M.D., Dept. of Pathology, Northwestern University Medical School.
UNILATERAL FUSED KIDNEY
565
factor in hydronephrosis and infection. Most of these were found at autopsy where there was no clinical record. In 1 case there was 1 pelvis and 3 ureters, and in another case 3 pelves and 2 ureters (i.e. 1 ureter bifurcated to form 2 pelves). Ureters were constricted in 2 cases, and obstructed in 6 cases. In 7 cases there were coexisting anomalies of the genitalia. The incidence is not very great since the kidneys fuse after the genital system is separated from the urinary system. In 5 cases unilateral absence of the adrenal was noted. Howden in 1887 pointed out that the relation of the suprarenal to the kidney was really one of accident, and when not molded to the top of the kidney the shape is somewhat rounded. Diagnosis. Of 244 cases in which the diagnosis was specified 112 were iiscovered at autopsy, 95 were found by roentgenography or pyelography, and 33 at operation. Four cases have been found in the dissecting room. At the present time, unilateral fused kidney is diagnosed chiefly by pyelography. If the ureteral pyelogram or the x-ray with opaque catheters in situ shows that both ureters pass to one side of the body, i.e. that one crosses the midline, and that the pelves are relatively close together at a point where there is a palpable mass, one can be fairly certain of unilateral fused kidney. The anomalous rotation of the pelves as shown by retrograde or intravenous pyelography is an almost constant manifestation of fusion. It may be called a "triangle" pyelogram, the base of which is formed by the bladder, and the two sides by the ureters running to the kidney at the apex. It resembles a right triangle, the hypotenuse of which is the ureter of the crossed ectopic kidney. Since the ureters enter the bladder normally (with the exception of 3 cases in which the position was slightly altered), cystoscopy is of no diagnostic value. Measurement. All the measurements here are taken only from cases past maturity. The average weight of 7 fused kidneys is 314 gm., which is approximately double the weight of a single kidney. The average length (17 cases) by width (14 cases) by thickness (7 cases) is 16.8 by 7.6 by 4.1 cm., in the longest measurements for each dimension. There is much variation in the relative vertical position of the fused renal mass. It may be found anywhere from the level of the lower thoracic vertebra to a position deep in the pelvis. Subdivisions of the unilateral fused kidney (fig. 6) are as follows: 1. Elongated kidney, pole to pole fusion: The ectopic kidney is always inferior, its superior pole being fused to the inferior pole of the
566
HARRY A. WILMER
orthotopic or normal kidney. The hili are usually anterior, but they may be in any arrangement except that characteristic of the "S" kidney. This is the most common variety. 2. "S" kidney, sigmoid, pole to pole fusion: The superior hilus always faces mesially, and the hilus of the inferior ectopic kidney faces laterally. The convex borders of the two kidneys face in opposite directions so that it appears like the Latin "S". In this case both kidneys have completed their rotations, therefore, if the inferior ectopic kidney were transposed to its normal side, the hilus would face mesially. Type:s of
Fusion
Mu1al F\Jsio11
FIG. 6
3. Lump kidney, "kuchenniere," extensive fusion: The 2 kidneys are fused together over a large area so that together they form a lump or mass. Eack kidney loses its distinctiveness, and grossly they may be mistaken for a solitary kidney. The ureter of the ectopic kidney crosses the midline and in this way djstinguishes it from the lump kidneys which are not unilateral fused. 4. "L" kidney. The ectopic inferior kidney is fused to the superior orthotopic kidney so that its lon,g axis is perpendicular to it. The inferior hilus is usually anterior. The "L" kidney resembles the Latin "L", which, however, may be inverted or reversed. 5. Mesial border fusion: The kidneys lie side by side with their mesial
UNILATERAL FUSED KIDNEY
567
borders fused. LaRose reported an unique case in which the kidneys were fused in the form of a ring. 6. Superior kidney ectopic: Fusion may be of any nature but the crossed ectopic kidney is always superior. This is the rarest type. Embryology. In crossed ectopia fusion occurs at approximately the 10 mm. stage of the embryo. The ureteral bud arises from the Wolffian duct at 4.5 mm., penetrates the surrounding mesoderm and at 7 mm., it meets the blastema which then begins its ascent. Hill, Lewis and Papez and others explain the fusion by the fact that the bifurcation of the aorta into the umbilical arteries forms a crotch in which the kidneys may be forced together as they migrate upwards. The arteries acting as a mechanical obstruction tend to bring the right and left blastemas together so that fusion may readily take place. Pole to pole fusion according to Huntington, and Scheiner is the result of the ureteral buds arising at different vertical levels so that the caudal pole of one of the renal blastemas touches and fuses with the cephalic pole of the other. The cephalic kidney usually takes the lead and displaces the caudal organ across the midline. It probably ascends on the side of least resistance. Normally the renal pelves are anterior until migration, at which time the blastema rotates laterally and the pelves medially. The rotation is stopped at the time of fusion, and since the cephalic kidney has ascended farther at this time, it is usually more rotated. For this reason the hilus of the superior orthotopic kidney is frequently mesial, and the hilus of the inferior kidney anterior. Fusion brings about an irregular vascular supply. The kidneys migrate upwards through the mesonephric arteries. As the cephalic blood supply becomes sufficient to supply the ascending kidney, the caudal arteries separate off. Braasch, and Felix have compared the ascent through the mesonephric arteries to climbing a ladder. When the migration stops some blood vessels disappear, and the remaining blood vessels at that time become the permanent vessels. Therefore, anomalous vessels are a natural consequence of unilateral fused kidneys. The superior kidney usually receives one or two arteries from the aorta, and the inferior kidney receives one or two from the aorta or the common iliac depending on the location of the kidney. There is such a wide variation in the vascularization that no general statement can be made. Hyman pointed out that the congenital ectopic kidneys, because of the anomalies of the blood vessels and ureters, are prone to pathological lesions.
568
HARRY A. WILMER
Acknowledgment. I am indebted to Dr. F. E . B. Foley for permission to use 2 clinical cases, to Dr. D. James Waterston to use 1 case, and especially to. Dr. E. T. Bell for indispensable advice and criticisms. SUMMARY
Five cases of unilateral fused kidney are reported here together with a series of 94 cases collected from the literature by the author, which brings the total number of reported cases of this anomaly to 286. In about 60 per cent of the cases the kidneys were found on the right side. The anomaly appears to be equally distributed between sexes. The great majority of cases either recognized clinically or at autopsy were in patients under 50 years of age. Unilateral fused kidneys seem predisposed to hydronephrosis and pyelonephritis, but not to other renal lesions. This anomaly falls into six classes: (1) elongated, (2) "S", (3) "L", (4) mesial border fusion, (5) lump, and (6) superior kidney ectopic kidney. · The unilateral fused kidney is found about once in 7,500 autopsies. The fusion of the kidneys is facilitated by a mechanical obstruction at the bifurcation of the aorta into umbilical arteries. These vessels form a crotch which may force together the ascending blastemas. Great variation exists in position, rotation, and vascular supply of the unilateral fused kidney. The most common symptom is pain. The renal mass is usually palpable, especially if involved in a lesion. Frequently there are urinary symptoms. The diagnosis can be easily and accurately made by a pyelogram which will show the ureter of the ectopic kidney crossing the midline to terminate in the bladder normally, presenting a "triangle" pyelogram. REFERENCES ADLER-RACZ, A. VON : (3 cases). Ztschr. f . urol. Chir., 31: 253, 1931. AFANASSJEW, A. N.: (3 cases). Ztschr. f . urol. Chir., 37: 143, 1933. AUGE, A., AND BONNET, A.: J. d'Urol., 18: 493, 1924. BABAINANTZ, M . L. : J. de Radio!. et d'Electr., 18: 561, 1934. BARCLAY AND BAIRD: Lancet, 2: 1169, (Nov.) 1935. BARRY, R . T., AND GARVEY, F . K.: J. A. M.A., 94: 1232, 1930. BEER, E ., AND FERBER, W. L. F.: (14 cases). J. Urol., 38: 541, 1937. BESSESEN, D. H. : Ann. Surg., 98: 314, 1933. BORCHARDT, M . : Med. Klin., 28: 439, 1932. BRAASCH, W. F.: Ann. Surg., 66: 726, 1912. CANELLI, A. F.: Riv. di din. pediat., 16: 577, 1918. CANIGIANI, T .: Rontgenpraxia, 8: 103, 1936. CARLETON, A.: J. Anat., pt. 2, 71: 292, 1937.
UNILATERAL FUSED KIDNEY CARRARO, N.: Morgagni, 56: 300, 1914. CIFUENTES, P., AND ONTANON, G.: J. d'Urol., 36: 343, 1933. CRAISIN: Arch. gen. de chir., 4: 455, 1913. DrNATILI, L.: Arch. Ital. di chir., 37: 104, 1935. DUNAVANT, B. N.: South. Med. J., 12: 689, 1919. ErSENDRATH, D. N., AND RoLNicK: Textbook on Urology, Phila., J. B. Lippincott & Co., 1934. ELVING, A.: Ztschr. f. urol. Chir., 19: 67, 1926. FARCAS, I., AND REVESZ, V.: (2 cases). Rontgenpraxis, 2: 759, 1930. GENOUVILLE AND SAQUIN: J. d'Urol., 18: 519, 1924. GERARD, G.: J. de l'anat. et de la physiol., 41: 241, 411, 1905. The following cases were collected by Gerard: Cathelin (1898). Reed (1845). Chambelent (1895). Sandifort (1793). Chassaignac (1832) Stoicesko (1883). Chassaignac (1840). Tanton (1901). Horne (1793). Tesson (1895). Hunter (1785). GODARD: Cited from HEINER, G. (TANDLER): Folia Urolog., 3: 186, 1909. GOTTLIEB, J. G.: J. d'Urol., 24: 139, 1927. GRUBER, G. B.: (2 cases). Wein. med. Wchnschr., 74: 2007, 1924. GuELLEllHN, A.: (Reported by Dossot). J. d'Urol., 43: 81, 1937. GUIMPELSON, E. I., AND FATIANOVA, L. N.: (4 cases). J. d'Urol., 40: 333, 1935. HACHEZ: Deutsche med. Wchnschr., 60: 1243, 1934. HESS, E.: J. d'Urol., 22: 667, 1929. HERMAN, L.: Atlantic M. J., 37: 1009, 1934. HODGE: Am. J. M. Sc. N. S., 6(): 455, 1870; cited from BALLOWITZ in: Virchow's Arch. f. path. Anat., 141: 309, 1895. HUNTINGTON, G. S.: (2 cases). Harvey Lectures, 1906-07, Genito-Urinary Tract: Variations. Phila. & London, J. B. Lippincott and Co., 1908, p. 222. JACOBS: Glasgow M. J., 1.20: 41, 1933. KALBERG, W.: Anat. Anzeiger., 74: 117, 1932. KRETSCHMER, H L.: Surg. Gynec. and Obst. 40: 360, 1925. The following cases were collected by Kretschmer: Bieberbach (1924). Hyman (1922). Kidd (1910). Bissel (1911). Boody (1899). Lowsley, Kingery, and Clarke (1924). Bugbee (1911). Lund (1919). Mennacher (1909). Bugbee (1911). Carrieu and DeRouville (1887). Meyer (1913-14). Day (1924). Pohlman (1904-5). Dennis (1904). Rathbun (1913). Felty (1918). Stewart and Lodge (1923). Gruner and Fraser (1911). Thompson (1913). Gruner and Fraser (1911). Wilcox (1921). Huntington (1906-7). Winternitz (1908). Huntington (1906-7). Wilhelmi (1920-21). Hyman (1922). Kretschmer (1925). KuNDRAT: (2 cases). Wein. med. Wchnschr., p. 109, 1886; cited from PAGEL. LMrnors, F.: Bruns' Beitr. z. klin. Chir., 149: 501, 1930. LEMBERGH, W.: (4 cases). Ztschr. f. urol. Chir., (Ref.), Hi: 66, 1924. LISA, J. R., AND LEVINE, J.: (2 cases). N. Y. State J. M., 32: 395, 1932. MAISEL, I.: (2 cases). British J. Urol., 9: 380, 1937. MARTIUS, K.: Frankfurt. Ztschr. f. Path., 12: 47, 1913. MATTIOLI: Gesellsch., Ztschr. f. urol. Chir., 19: 250, 1926. MAYER, A. J., AND NELKEN, A.: J. A. M.A., 57: 1262, 1911. MELOY: Grace Hosp. Bull., 3: 16, 1918. Cited from SEXTON. MEYER, 0.: Cited from DORLAND in: Surg. Gynec. Obst., 13: 303, 1911. McDONALD, P. W.: Lancet, 1: 979, 1885. MINTZ, E. R., AND STEWART, J. D.: New England Med. J., 206: 1282, 1931. MoURE, P., AND MARCY, J.: Mem. Acad. de chir. Paris, 63: 705, 1937. NAUMAN: Diss., Kiel, 1897; cited from KRETSCHMER. NEUWIRT, K.: ReL Ztschr. L uroL Chir., 15: 328, 1924.
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NEWMAN, D.: Cited from DORLAND in: Surg. Gynec. Obst., 13: 303, 1911. O'CoNOR, V. J. : The Anomalies of Kidneys, in: The Cyclopedia of Medicine, Vol. VII, Phila., F . A. Davis and Co., 1934, p. 741. OGSTON, F.: Brit. M. J., 1: 591, 1879 (also reported by Braxton-Hicks}. PAPIN AND PALAZOLLI: Ann. des. Mal. de Org. Genito-Urin., 27: 1681-1710, 1762-1786, ' 1842-1891; 1909. The following cases were collected by Papin and Palazolli: Albarran (1908). Laube (1722). Bachhammer (1879) . McMurrich (1898) . Bachhammer (1879). McNaughton (Jones) (1866). Bachhammer (1879). Morris (1901). Barth (1853). Palma (1891). Birmingham (1890). Pannorlus (1654). Bolintinean and Pastia (1907). Picard (1872). Portal (1804). Broesika (1884). Cholzow (1907). Poulalion (1890). Coupland (1877). Powell (1883). Rufz (1883). Dickenson (1895). DuBois (1907). Schmerber (1895). Duckworth (1869). Schwalb (1896). Duvivier (1730). Steiner (1901). Godard (1855). Stoquart (1879). Greenfield (1876). Stoquart (1893). Gronnerud (1907). Stolz Gruber (1887). Strube (1894). Hill (1906). Sutherland and Edington (1898) . Sutherland and Edington (1898). Hillier (1864). Tandler (Heiner) (1907). Houze! (1898). Huebschman (1908). Tully (Vaughan) (1897). Israel (1908). Turner (1886). Israel (1908) . Verocay (1907). Karltreu, B. von (1898). Wehn (1895). Kelly (1868). Weibel (1908) . Kruse (1890). Weisbach (1867). Kruse (1892). Wilcox (1880) . Kuster (1895). Zondek (1903). LaRoche (1895). Zondek (1903). PFITZER, H.: Ztschr. f. urol. Chir., 67: 772, 1930. PIERSON, L. E.: J. Urol., 28: 217, 1932. The following cases were collected by Pierson: Bieberbach (1924). Lavroff (1928). Caulk (1923). Makai (1926) . Donohue (1926). Pedroso (1930). Dourmashkin (1924). Pierson (1932). Hess (1926) . R athbun (1927). Ide! (1927) . Saikin (1926). LaRose (1924). Sexton (1925). Lazarus (1927). Spurr (1927). PLESCHNER, H. G.: Wein. klin. Wchnschr., 42: 1581, 1929. RATNER, S.: (2 cases). Ztschr. f. urol. Chir., 29: 531, 1930. RUMPEL, 0 . : Zentralbl. f . Chir., 69: 1290, 1932. RussEL, J . C. : J. Anat. and Physiol., 19: 1885. RUTHARDT: Arch. f . Anat. u. Physiol., p. 599, 1827. SAWYER, C. F.: Proc. Inst. Med. Chicago, 10: 134, 1934. SCHILLING, F. : Arch. f. Gynak., 114: 428, 1920. SCHILLINGS, M ., BRENEZ, J., AJ:
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STEIN, A. : Am. J. Obst., 73: 449, 1916. The following cases were collected by Stein: Colmer (1911) . Smith and Gammon (1901). Dumitreamu (1908). Stein (1916) . Geiss (1899). Strube (1894). Illyes (1908) . Winternitz Lalmohon (1876) . Zuckerkundl (1909). Pashkis (1910). SuRRAco, A.: J. d'Urol., 23: 411, 1927. THOMPSON, G. J., AND ALLEN, R. B.: (20 cases) . S. Clinics North America, 14: 729, 1934. TOWNSEND, J. M., AND FRUMKIN, J.: Urol. and Cutan. Rev., 41: 324, 1937. The following cases were collected by Townsend and Frumkin: Bethea and Peterson (1935). Lequeu (1930) . Bethea and Peterson (1935). Lindner (1935). Bethea and Peterson (1935). Masciottra (1935) . Bordas (1927). Oraison (1912). Bugbee and Losee (1919) . Pagel (1922). Bugbee and Losee (1919) . Pagel (1922) . Capua (1935). Pagel (1922). Capua (1935). Pagel (1922) . Carraro (1929). Palmer (1924). Chenault (1935) . Quinby (1925) . Fasiani (1926) . Ratner Glesne (1934) . Rejsek (1924). Hess (1933) . Stincer (1914). Homma (1930) . Strong (1900) . Iscenko (1933). Townsend and Frumkin (1937) . Iscenko (1933) . Willis (1931) . VouTEIN: Rev. de Chir., Paris, 42: 447, 1910. ZEISS, L., AND B0EMINGHAUS, H .: (6 cases). Ztschr. f . urol., 28: 577, 1934.