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Resection of a Unilateral Fused Kidney with Case Report1
RESECTION OF A UNILATERAL FUSED KIDNEY WITH CASE REPORT1 W. G. SEXTON Marshfield Clinic, Marshfield, Wisconsin Received for publication February 1, 1925
Anomalies of the urinary organs have been reported since the time of Aristotle, but it has only been in recent years that the frequency of their occurrence has been noted and that emphasis has been laid upon their clinical importance. Bugbee and Wollstein (1) in 4900 autopsies in the Babies Hospital found anomalies in 3 per cent of children. From the clinical side Braasch (2) reports that in a series of 649 renal operations one anomaly was found in every 26 cases. The studies of Eisendrath (3) have shown us the variety and frequency of accessory renal vessels. Mertz (4), Buerger (5), Hepburn (6), Dorland (7), and Eisendrath (8) have demonstrated numerous malformations of the ureters, ·including duplications, abnomal insertions and strictures. Chas. Mayo (9) and Rathburn (10) have reported cases of solitary kidneys. A. Mueller (11) and Kretschmer (12) have produced splendid articles on accessory kidneys. Double kidneys have been studied by Pilcher (13), Hamer and Mertz (14, 15), Braasch (2) and especially by Eisendrath (16). The most unusual renal anomaly is the unilateral fused kidney which is also called crossed ectopia or crossed dystopia of the kidney. Since, according to Hufman (17), one may have a crossed dystopia with or without fusion, it seems that unilateral fused kidney is a more exact term. 1
Read before the Chicago Urological Society, Chicago, Ill., January 22, 1925. 399 THE JOURNAL OF U ROLOGY, V OL. X IJT, N O. 4
400
W. G. SEXTON
The fused kidneys may be classified as follows: A. Horseshoe kidney (Hufeisenniere)
B. Clump kidney (Kuchenniere) C. Long kidney (Langniere) D. S-shaped kidney or sigmoid kidney
The horseshoe kidney has its fusion by an isthmus connecting either the uppe.r or lower poles with the kidney on either side of the spine. The clump kidney is a fusion in a very irregular shape, usually on one side. The long kidney is a fusion of the kidneys end to end. The sigmoid kidney is an end to end fusion with great variations in the amount of rotation of the two kidneys. These fusion types are all embryological developmental defects, and can be explained upon these grounds. So many articles have appeared bearing upon the embryology of the urinary organs, that one wishing to know the details of this may refer to Keibel and Mall (18) or the articles by Bugbee and Losee (19) or Chas. H. Mayo (9). It is sufficient at this time to merely recall that the adult kidney is the final stage in development of three primitive kidneys, pronephros, metanephros, and mesonephros. "The first two degenerate while the third remains as the permanent kidney. The second begins to develop while the first degenerates and the third while the second degenerates. A constructive and destructive process at the same time." Their arterial supply comes from many sources and changes as the _kidney ascends from the pelvis to its final resting place. The ureter and pelvis develop as outgrowths of Wolffian duct near the cloaca while the secreting portion comes from mesothelial cells. Thus the kidneys begin their development low down and ascend out of the pelvis. It is during this early stage that the fusion takes place and the dystopic kidney is dragged to the opposite side to form a unilateral fused kidney. The best article on unilateral fused kidney is that by Papin and Palizolli (20), and I am greatly indebted to Dr. Eisendrath for a complete translation of this study. They made a
RESECTION OF UNILATERAL FUSED KIDNEY
401
critical survey of the 70 cases in literature up to 1909. I have been able to find 30 cases reported since then-as shown in table 1. TABLE 1 REFERENCE
AUTHOR
Cases diagnosed by operative or cystoscopic methods Joseph Rejsek.. ... . . ............... J . d'Urol Med. Et. Chir: Paris, October 1924, xviii, 317 Bugbee and Losee (3 cases)..... . . . . Surg. Gyn. Obst., February, 1919, xxviii 97 Victor G. LaRose. . . . . . . . . . . . . . . . . . Jour. Urol., xii, 127 A. Hymen (2 cases)... .. . . ....... ... Zeitsch. f. Urol. Chir., ix, 157 Stein .. . .. ...... . .. . ....... ...... . . . Amer. Jour. Obst., March, 1916, lxxiii, 449 Rathburn. ........ .. .... .. . . ... . .. . Urol. and Cut. Rev., 1919, xxiii, 77 Dunavant...... .. . ... .. .. .. ... ... .. South Med. Jour., 1919, xii, 689 Lowsley and Kingery and Clark. ... Jour. Urol., February, 1923, xi, 293 Bieberback.............. . . . . . . . . . . . . Urol. and Cut. Rev., January, 1924, xxviii~ 35 Dourmashkin.. .......... . .... . . . .. . Jour. Amer. Med. Assoc., June 21, I924, lxxxii, no. 2, 25 Braasch (3 cases)... .. ..... . . ....... Ann. Surg., November, 1912, lxvi, 726 Buerger. ..... . .. . .... .. ..... . ..... . Surg. Gyn. Obst., xxviii, 183 Caulk, John R ... . ..... ... ... .. .... Ann. Surg., July, 1923, lxxvii, 65 Cases found at autopsy Mayer and Nelken. . . . ........ . ... .. Jour. Amer. Med. Assoc., October 14, 1911 lvii, 1262 Schilling. . . . . . . . . . . . . . . . . . . . . . . . . . . Arch. Gynack, 1921, cxiv, 428 Craisin.... . ............. . .. . ....... Arch. Gen. de Chir., 1913, iv, 455 Vautrin ...... ......... ... .. .. .. . . .. Rev. de Chir., 1910, xlii, 447 Bugbee-Wollstein (3 cases) ..... . .... Jour. Amer. Med. Assoc., December 13, 1924, lxxxiii, 1887 Felty.. .. ......................... .. Jour. Urol., October, 1918, ii, 421 Cannelli, A. F...... . . . . . . . . . . . . . . . . Riv. di. Olin. Pediat. Firenze, 1918, xvi, 577 Meloy...... . . . . . . . . . . . . . . . . . . . . . . . . Grace Hosp. Bull. Detroit, October, 1918, iii, 16 Stewart and Lodge.. . .. . . .. .. . . ... . Brit. Jour. Surg. Bristol, July, 1923, xi, 27' Day........ . ............... . ... .. .. Surg. Gyn. Obst., January, 1924, xxxviii, 51
From Papin and Palizolli the following facts may be noted : They emphasize the difference between the fused unilateral and
402
W. G. SEXTON
the unilateral solitary kidney. In the latter there is a more or less complete lack of development of entire genito-tirinary tract of the opposite side, while in the fused unilateral kidney there is a change in _development due to displacement of the renal anlage. The fusion is not always exactly end_to end and may form an acute angle or even L shape. The condition seems to be more common in males and most cases have been recognized in the third decade. The kidneys are usually unequal in size. The anterior surface is nearly always lobulated while the posterior is smooth. The line of fusion may be distinct or very indefinite. The ureters in nearly all cases present normal openings in the bladder. The hilus of the upper kidney may face inwards, while that of the lower kidney may be forward making an angle of 45 degrees with the normal. The arterial supply is subject · to great variations. The ectopic kidney usually has more arteries than the fixed one. The number yaries from one to six which may come from the aorta, external or internal iliacs or combinations of these. Association with other defects is common. Bugbee (19) believes that the malformed or dystopic kidney is subject to infection on account of poor drainage, faulty blood supply and liability to pressure. Mayo says that hydronephrosis is common in young people and pyonephrosis more likely in older cases. He disagrees with Hymen (21) who thinks the ectopic kidney is more subject to tuberculosis. A study of the literature up to date shows that 18 cases of unilateral fused kidney have been operated upon. Four were not recognized as such, and all died of anuria. Ten were subjected to various palliative operations with recovery, and in 4 cases (including this one) the ectopic portion was resected with recovery (table 2). CASE REPORT
E. H ., white, male, aged nineteen, cheese maker. Admitt'ed to hospital August 29, 1924, for treatment for abdominal pain. _ Family history. Unimportant. Past history. Unimportant; He has never had any symptoms referable to his urinary tract. Admits -exposure-to Neisser infection: fourweeks prior to admission.
TABLE 2
Operations upon unilateral fuse,d kidneys OPERATOR AND REFERE?\CES
OPERATION
RESULT
Group I. Condition not recognized at operation Housel, 1898, quoted m ' Papain and Palizolli Craisin, Arch. gen. de Chira, 1913, iv, "155 Voutein, Rev. de Chir., 1910, xiii, 447 Dennis, 1994, N. Y. Med. Jour., January 30, 1904, lxxii, 200
Removed e1;1-tire kidney
Died of anuria
Nephrectomy of entire mass
Died of anuria
Nephrectomy of entire mass
Died of anuria
Nephrectomy of entire mass
Died
Group II. Steiner, 1901, Verhdlgd. dtsch. Ges. f. Chir., 1901, xxx, 15 Israel, 1908, F olia Urol., 1908, 1, 617 Israel, Folia Urol. , 1908, i, 617 Albarran, 1908, quoted by Papain and Palizolli Bugbee-Losee, Surg. Gyn. and Obst., February, 1919, xxviii, 97 Bugbee-Losee, Surg. Gyn. and Obst., February, 1919, xxviii, 97 Quain, reported by Victor G. LaRose, Jour. Urol., vii, 127 A. Hymen, Zeitsch. F . Urol. Chir. , ix, 157 Rathburn, Urol. and Cut. Rev., 1919, xxiii, 77 Caulk, John R., Ann. Surg., July, 1923, lxxvii, 65
Separated and sutured lower hydronephrosis to skin
Recovery
Removed calculus upper half
Recovery
Decapsulation of both for Recovery nephritis Decapsulation of upper half Not much provement and fixation for mobility Attempt m ade to replace kid- Recovery ney in more normal posit ion
Gro up III. Weibel, 1908, Weiner K lin. Wochenschrift, 1908, xlvii, 1632 Dermiteanu, Deut. Med. Wochschr., 1908, xxx, 1333 Zuckerkandl, Wiener K lin. Wochschrift, 1909, !ix, 1767 Sexton
Palliat ive operations
Kidney divided. Bot h halves placed as high as possible
Recovery
Kidney elevated
Recovery
Stone remove d from pelvis of one kidney Calculus removed from one ureter Decapsulation and fixation of mass
Recovery Recovery Recovery
Resection of ectopic portion Removed ectop ic left kidney
(upper)
Recovery
Resection of lower h alf
Recovery
Resected lower segment of fused kidney for tuberculosis Resection ectopic kidney
Recovery
403
Recovery
im-
- - - - - - - - - - - - - - - -- - - - - - - -- - - - - - - - - -
404
W. G. SEXTON
Present illness. Began three weeks previous to admission with severe abdominal pain followed by vomiting. This lasted about two hours and then gradually lessened in severity. Two weeks later he had a second attack of very sharp pain in the abdomen and noticed a mass just below the umbilicus. He then detected a purulent discharge from the urethra which was followed on the next day with bloody urine. This attack of pain lasted three days, and was accompanied by a mass below the umbilicus. Four days ago pain again appeared and was present at time of admission. The patient did not notice whether or not the mass disappeared between attacks. His urine has remained bloody and he has had a more or less constant urethral discharge, and for the past four days he has been having some chills and fever.
Fm. I.
LATERAL
Vrnw
OF PATIENT SHOWING PROMINENT MAss JusT BELOW UMBILICUS.
Physical examination. Well developed and well nourished white male, nineteen years of age. H e is 6 feet 1 inch tall, weighing 155 pdunds. Walked into the hospital. Temperature 101, pulse 84. Respirations 20. General physical examination with exception of abdomen revealed nothing abnormal. Abdomen : On inspection t here is a definite mass (fig. 1) visible just below the umbilicus and nearly in the median line but slight ly toward the left side. This forms a prominent mass 12 cm. in length in the mid line and about the size of a grape fruit. The right lateral border was 9 cm. from the mid line. On the left t he fullness extends to t he anterior superior spine of the ilium and fills the left flank. The mass is very tense but feels like fluid llnder pressure. There is a slight degree
RESECTION OF UNILATERAL FUSED KIDNEY
405
of lateral mobility. There is very slight movement during deep inspiration. The left flank is more prominent than the right. The right kidney cannot be felt. Neither the liver nor the spleen can be palpated. Genitalia normal. Prostrate slightly softer than normal. Laboratory findings. Urine: Specific gravity 1016; acid; sugar 0; albumin; pus; red blood cells. Blood: Red blood cells, 4,3)0,000; white blood cells, 7600; hemoglobin, 85 per cent; differential count-Neutrophiles 75, basophiles 0, eosinophiles 1, small mononuclears 16, large mononuclears 6, transitional 2. Blood chemistry: Non-protein nitrogen, 42.4 mgm.; urea nitrogen, 23.83 mgm. Phenolsulphonephthalein: First hour, 5 per cent; second hour, 20 per cent. Urethral smears: Negative for gonococci. During the first twelve hours that the patient was in the hospital the mass disappeared, his pain left and his temperature dropped to normal. First cystoscopy September 1, 1924. Bladder practically normal. No evidence of calculi, tumor or diverticulum. Ureteral orifices appeared small but in normal position. Both sides catheterized with No. 6 ureteral catheters. The urine from the right side was purulent and contained many pus cells and red blood cells. That from the left side was clear with only an occasional pus cell microscopically. During the night of September 6 his pain reappeared and the mass was again noticed. Second cystoscopy performed September 6, 1924. Both sides catheterized and catheters left in place. In two hours the right side excreted 750 cc. The left 125 cc. roentgenogram shows the right catheter passes across the spine over the sacrum to the left side. Thirty-five cubic centimeters sodium bromide was injected into the right catheter. Pyelogram shows a greatly distorted pelvis, with the left catheter pushed laterally. This suggested a fused kidney. The urine coming through the right catheter was much clearer than at former examination, but pus could be seen coming from the ureteral orifice around the catheter which suggested a partial duplication of the ureter. Six days later (September 12) the mass had reappeared and upon catheterization of right ureter 1250 cc. were obtained in two and onehalf hours with disappearance of the tumor. At this examination a
406
W. G. SEXTON
No. 8 catheter was used and after injecting 30 cc. of sodium bromide the catheter was partially withdrawn and 10 cc. more were injected in an attempt to visualize the ureter. This was unsuccessful, but the pelvic shadow showed it was very much larger and lower than was at first suspected. On September 16 the left side was injected with 10 cc. of sodium bromide and the pelvis of the left kidney located above the mass but in about normal position (fig. 2). The right catheter turned downwards after entering the pelvis, indicating that there was a very large pyonephrosis.
FIG.
2.
PYELOGRAM SHOWING CATHETER IN RIGHT URETER CROSSING OVER TO LEFT SIDE
Catheter in left ureter pushed laterally and pelvis injected with sodium bromide solution.
On September 19 the patient was in such good condition tha( he insisted upon leaving the hospital. He returned for observation October 6. He was advised to submit to operation. He went to Sheboygan, Wisconsin, his home where he was seen October 13 by Dr. Siegfried Kraft of the Sheboygan Clinic. At this time the mass had reappeared, and the patient was very ill. Dr. Kraft upon prostatic massage obtained many gonococci. Dr. Kraft very kindly sent the patient back to me with advice to submit to an immediate operation. He was readmitted October 17, complaining that his pain was very intense and that the tumor mass was larger than it had ever been and
RESECTION OF UNILATERAL FUSED KIDNEY
407
noticed to be above the umbilicus. Temperature 100.6. Pulse 84. Respiration 20. White blood cells 12,950. On examination of his abdomen the mass extended farther across the mid line than on the previous admission and was so large that extirpation seemed almost impossible. He was prepared for operation next morning. September 18. Previous to operation the patient was cystoscoped and both ureters catheterized. Twelve ounces of clear urine was obtained from the catheter in the left ureteral orifice and about 5 ounces of very thick pus obtained from the catheter in the right orifice. This was so thick that it ran out very slowly and was facilitated by suction. The catheters were left in place. At the end of one hour the large mass seen across the abdomen disappeared. This lead to the conclusion that we were dealing with a hydronephrosis of the left kidney due to pressure of an infected ectopic kidney upon the ureter of the left side. The patient was then taken to the operating room with the catheters in place to aid in locating the ureters. Description of operation. A long incision was made extending from the costo-vertebral angle down parallel to the crest of the ilium about two inches below the anterior superior spine. The muscles were cut and the kidney exposed (fig. 3). It was found upon palpation that there was a very large mass occupying the entire flank extending upward as far as could be reached with the hand, and downward as far as the brim of the pelvis, and medianly as far as the median line of the body. The lower half was a distinctly cystic sack which showed definite fluctuation. The upper half appeared to be quite normal kidney structure. There was a fairly definite line of demarcation evidenced by a white fibrous band that crossed this mass and indicated the site of fusion of the two kidneys, but it was impossible· to separate the two kidneys at this point. A trocar was then inserted into the lower mass and a little over a quart of purulent matter was drawn out by suction. We then began a careful separation of the lower half and found that the blood supply came from the left iliac and entered the kidney on the lateral surface. This was clamped, cut and tied. Both ureters were located, and it was found that they were both lying on the anterior surface of the kidney indicating that neither kidney had completely rotated. The ureter from the upper half was the thickness of one's finger, and passed under the ectopic portion while the ureter of the lower half was not enlarged. The mass extended so far downward and so far toward the middle line that its removal seemed almost impossible, but it was finally freed and delivered, and a resection was made about one inch
408
W. G. SEXTON
below the line of fusion which has been described. Several vessels were encountered in doing this resection from the fixed kidney; these were tied as they were met. There was no hemorrhage during the operation, but the patient's pulse became quite weak before the resection was complete. Hypodermoclysis of Boline and glucose was given while the patient was on the table and his pulse improved rapidly. The wound was packed with two large compresses and the muscles closed in layers of continuous catgut. Skin was closed with interrupted silkworm gut sutures.
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FIG.
3.
_:;;.:::~
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DRAWING ( DIAGRAMMATIC) OF CONDITION FOUND AT OPERATION
Descri ption of the specimen. The specimen is an irregularly elongated mass 18 cm. in length, 10 cm. wide at its widest portion and 6 cm. thick (fig. 4) . The anterior surface is very irregularly lobulated. Nearly all of the divisions between t he lobules run transversely across the mass but there is one large elongated lobule that follows the long axis of the mass. The lower end of the tumor has been broken into and the upper end follows the line of section. The posterior surface is comparatively smooth. The ureter cannot be recognized. The arterial supply seems to enter t he middle of the posterior surface. Upon section it is found
RESECTION OF UNILATERAL FUSED KIDNEY
409
that the mass is composed of merely a thin shell of renal tissue and the anterior is composed of many multilocular spaces. There apparently is very little normal renal tissue. Post-operative. His temperature ranged from 103 to 100 and became normal on the sixteenth day and then remained normal. November 22, phenolsulphonephthalein first hour 20 per cent, second hour, 30 per cent. He was discharged from the hospital November 25, 1924, with wound healed with the exception of two very small superficial granulating areas at either angle of the wound. He returned for observation December 30, 1924, at which time he was in excellent health. Weight 170 pounds. He had been working at his trade as a cheese maker for several weeks. He had a small sinus at the upper angle of the wound that had been discharging for about two weeks and may be a urinary fistula.
FIG. 4. PHOTOGRAPH OF ANTERIOR SURFACE OF ECTOPIC PORTION REMOVED
CONCLUSIONS
The number of cases of fused kidney that have been reported in recent literature indicates that this condition is probably not as rare as we have believed. By utilizing our modern urological methods of diagnosis in a careful methodical manner we should be able to detect more of these cases. REFERENCES
(1) (2)
Surgical pathology of the urinary tract in infants. Jour. Amer. Med. Assoc., December 13, 1924, lxxxiii, 1887. BRAASCH: Clinical diagnosis of congenital anomaly in kidney and ureter. Ann. Surg., November, 1912, lvi, 726. BuGBEE-WoLLSTEIN:
--
410
~--- - - - - - - - -
W. G. SEXTON
(3) ErsENDRATH: The relation of variation in the renal vessels to pyelotomy and nephrotomy. Ann. Surg., June, 1920, lxxi, 726. (4) MERTZ: Bilateral duplication of ureters with compilation of recorded cases. Urol. and Cut. Rev., November, 1920, xxiv, 637. (5) BuERGER-LEo: Infection of a fused kidney with duplication of left renal pelvis. Surg. Gny. Obst., xxviii, 183. (6) HEPBURN, THo. N.: Kidney and- ureter abnormalities. Ann. Surg., September, 1918, !xviii. (7) DORLAND: Consideration of renal anomalies with presentation of two cases. Surg. Gyn. and Obst., 1911, xiii, 303. (8) EISENDRATH: Congential malformation of ureters. Ann. Surg., April, 1912, Iv, 571. (9) MAYO, CHAS. H.: The surgery of the single and horse-shoe kidney. Ann. Surg., April, 1913, xvii, 511. (10) RATHBURN: Solitary· kidney with unusual anomaly of the ureter. Case report . Urol. and Cut. Rev., 1919, xxiii, 77. (11) MUELLER, A. : Zur Diagnose und Operation fur Akzessorisches Niere. Zeitsch. f. Urol. Chir., ix, 141. (12) KRETSCHMER: Supernumerary Kidney. Jour. Amer. Med. Assoc., 1915, !xv, 1447. (13) PILSHER, L. A.: Kidney with double pelvis and double ureter. Nephrolithiasis, Pyonephrosis, Nephrectomy. Ann. Surg., May, 1917, !xv, 534. (14) HAMER AND MERTZ: Radiographic findings in double kidneys. (15) MERTZ: A study of t he pelvis of the double kidney. Jour. Urol., xi, 259 (16) EISENDRATH: Double kidney. Ann. Surg., lxxvii, 450. (17) HuFMAN: Congenital displacement of kidneys. Jour. Urol., October, 1924, xii, 364. (18) KEIBEL AND MALL (Felix, W. ): The development of the genito-urinary organs. Human Embryology, ii, 654, Lippincott Co. (19) BUGBEE AND LosEE: Clinical significance of congenital anomalies of kidney a nd ureter. Surg. Gyn. and Obst., February, 1919, xxviii, 97. (20) PAPIN AND PALIZOLLI: Ann. Mal. G. U., 1909, xxvii, 1681. (21) HYMEN, A.: Unilateral fused kidneys. Zeitsch. f . Urol. Chir., ix, 157.
Anomalies of the urinary organs have been reported since the time of Aristotle, but it has only been in recent years that the frequency of their occurrence has been noted and that emphasis has been laid upon their clinical importance. Bugbee and Wollstein (1) in 4900 autopsies in the Babies Hospital found anomalies in 3 per cent of children. From the clinical side Braasch (2) reports that in a series of 649 renal operations one anomaly was found in every 26 cases. The studies of Eisendrath (3) have shown us the variety and frequency of accessory renal vessels. Mertz (4), Buerger (5), Hepburn (6), Dorland (7), and Eisendrath (8) have demonstrated numerous malformations of the ureters, ·including duplications, abnomal insertions and strictures. Chas. Mayo (9) and Rathburn (10) have reported cases of solitary kidneys. A. Mueller (11) and Kretschmer (12) have produced splendid articles on accessory kidneys. Double kidneys have been studied by Pilcher (13), Hamer and Mertz (14, 15), Braasch (2) and especially by Eisendrath (16). The most unusual renal anomaly is the unilateral fused kidney which is also called crossed ectopia or crossed dystopia of the kidney. Since, according to Hufman (17), one may have a crossed dystopia with or without fusion, it seems that unilateral fused kidney is a more exact term. 1
Read before the Chicago Urological Society, Chicago, Ill., January 22, 1925. 399 THE JOURNAL OF U ROLOGY, V OL. X IJT, N O. 4
400
W. G. SEXTON
The fused kidneys may be classified as follows: A. Horseshoe kidney (Hufeisenniere)
B. Clump kidney (Kuchenniere) C. Long kidney (Langniere) D. S-shaped kidney or sigmoid kidney
The horseshoe kidney has its fusion by an isthmus connecting either the uppe.r or lower poles with the kidney on either side of the spine. The clump kidney is a fusion in a very irregular shape, usually on one side. The long kidney is a fusion of the kidneys end to end. The sigmoid kidney is an end to end fusion with great variations in the amount of rotation of the two kidneys. These fusion types are all embryological developmental defects, and can be explained upon these grounds. So many articles have appeared bearing upon the embryology of the urinary organs, that one wishing to know the details of this may refer to Keibel and Mall (18) or the articles by Bugbee and Losee (19) or Chas. H. Mayo (9). It is sufficient at this time to merely recall that the adult kidney is the final stage in development of three primitive kidneys, pronephros, metanephros, and mesonephros. "The first two degenerate while the third remains as the permanent kidney. The second begins to develop while the first degenerates and the third while the second degenerates. A constructive and destructive process at the same time." Their arterial supply comes from many sources and changes as the _kidney ascends from the pelvis to its final resting place. The ureter and pelvis develop as outgrowths of Wolffian duct near the cloaca while the secreting portion comes from mesothelial cells. Thus the kidneys begin their development low down and ascend out of the pelvis. It is during this early stage that the fusion takes place and the dystopic kidney is dragged to the opposite side to form a unilateral fused kidney. The best article on unilateral fused kidney is that by Papin and Palizolli (20), and I am greatly indebted to Dr. Eisendrath for a complete translation of this study. They made a
RESECTION OF UNILATERAL FUSED KIDNEY
401
critical survey of the 70 cases in literature up to 1909. I have been able to find 30 cases reported since then-as shown in table 1. TABLE 1 REFERENCE
AUTHOR
Cases diagnosed by operative or cystoscopic methods Joseph Rejsek.. ... . . ............... J . d'Urol Med. Et. Chir: Paris, October 1924, xviii, 317 Bugbee and Losee (3 cases)..... . . . . Surg. Gyn. Obst., February, 1919, xxviii 97 Victor G. LaRose. . . . . . . . . . . . . . . . . . Jour. Urol., xii, 127 A. Hymen (2 cases)... .. . . ....... ... Zeitsch. f. Urol. Chir., ix, 157 Stein .. . .. ...... . .. . ....... ...... . . . Amer. Jour. Obst., March, 1916, lxxiii, 449 Rathburn. ........ .. .... .. . . ... . .. . Urol. and Cut. Rev., 1919, xxiii, 77 Dunavant...... .. . ... .. .. .. ... ... .. South Med. Jour., 1919, xii, 689 Lowsley and Kingery and Clark. ... Jour. Urol., February, 1923, xi, 293 Bieberback.............. . . . . . . . . . . . . Urol. and Cut. Rev., January, 1924, xxviii~ 35 Dourmashkin.. .......... . .... . . . .. . Jour. Amer. Med. Assoc., June 21, I924, lxxxii, no. 2, 25 Braasch (3 cases)... .. ..... . . ....... Ann. Surg., November, 1912, lxvi, 726 Buerger. ..... . .. . .... .. ..... . ..... . Surg. Gyn. Obst., xxviii, 183 Caulk, John R ... . ..... ... ... .. .... Ann. Surg., July, 1923, lxxvii, 65 Cases found at autopsy Mayer and Nelken. . . . ........ . ... .. Jour. Amer. Med. Assoc., October 14, 1911 lvii, 1262 Schilling. . . . . . . . . . . . . . . . . . . . . . . . . . . Arch. Gynack, 1921, cxiv, 428 Craisin.... . ............. . .. . ....... Arch. Gen. de Chir., 1913, iv, 455 Vautrin ...... ......... ... .. .. .. . . .. Rev. de Chir., 1910, xlii, 447 Bugbee-Wollstein (3 cases) ..... . .... Jour. Amer. Med. Assoc., December 13, 1924, lxxxiii, 1887 Felty.. .. ......................... .. Jour. Urol., October, 1918, ii, 421 Cannelli, A. F...... . . . . . . . . . . . . . . . . Riv. di. Olin. Pediat. Firenze, 1918, xvi, 577 Meloy...... . . . . . . . . . . . . . . . . . . . . . . . . Grace Hosp. Bull. Detroit, October, 1918, iii, 16 Stewart and Lodge.. . .. . . .. .. . . ... . Brit. Jour. Surg. Bristol, July, 1923, xi, 27' Day........ . ............... . ... .. .. Surg. Gyn. Obst., January, 1924, xxxviii, 51
From Papin and Palizolli the following facts may be noted : They emphasize the difference between the fused unilateral and
402
W. G. SEXTON
the unilateral solitary kidney. In the latter there is a more or less complete lack of development of entire genito-tirinary tract of the opposite side, while in the fused unilateral kidney there is a change in _development due to displacement of the renal anlage. The fusion is not always exactly end_to end and may form an acute angle or even L shape. The condition seems to be more common in males and most cases have been recognized in the third decade. The kidneys are usually unequal in size. The anterior surface is nearly always lobulated while the posterior is smooth. The line of fusion may be distinct or very indefinite. The ureters in nearly all cases present normal openings in the bladder. The hilus of the upper kidney may face inwards, while that of the lower kidney may be forward making an angle of 45 degrees with the normal. The arterial supply is subject · to great variations. The ectopic kidney usually has more arteries than the fixed one. The number yaries from one to six which may come from the aorta, external or internal iliacs or combinations of these. Association with other defects is common. Bugbee (19) believes that the malformed or dystopic kidney is subject to infection on account of poor drainage, faulty blood supply and liability to pressure. Mayo says that hydronephrosis is common in young people and pyonephrosis more likely in older cases. He disagrees with Hymen (21) who thinks the ectopic kidney is more subject to tuberculosis. A study of the literature up to date shows that 18 cases of unilateral fused kidney have been operated upon. Four were not recognized as such, and all died of anuria. Ten were subjected to various palliative operations with recovery, and in 4 cases (including this one) the ectopic portion was resected with recovery (table 2). CASE REPORT
E. H ., white, male, aged nineteen, cheese maker. Admitt'ed to hospital August 29, 1924, for treatment for abdominal pain. _ Family history. Unimportant. Past history. Unimportant; He has never had any symptoms referable to his urinary tract. Admits -exposure-to Neisser infection: fourweeks prior to admission.
TABLE 2
Operations upon unilateral fuse,d kidneys OPERATOR AND REFERE?\CES
OPERATION
RESULT
Group I. Condition not recognized at operation Housel, 1898, quoted m ' Papain and Palizolli Craisin, Arch. gen. de Chira, 1913, iv, "155 Voutein, Rev. de Chir., 1910, xiii, 447 Dennis, 1994, N. Y. Med. Jour., January 30, 1904, lxxii, 200
Removed e1;1-tire kidney
Died of anuria
Nephrectomy of entire mass
Died of anuria
Nephrectomy of entire mass
Died of anuria
Nephrectomy of entire mass
Died
Group II. Steiner, 1901, Verhdlgd. dtsch. Ges. f. Chir., 1901, xxx, 15 Israel, 1908, F olia Urol., 1908, 1, 617 Israel, Folia Urol. , 1908, i, 617 Albarran, 1908, quoted by Papain and Palizolli Bugbee-Losee, Surg. Gyn. and Obst., February, 1919, xxviii, 97 Bugbee-Losee, Surg. Gyn. and Obst., February, 1919, xxviii, 97 Quain, reported by Victor G. LaRose, Jour. Urol., vii, 127 A. Hymen, Zeitsch. F . Urol. Chir. , ix, 157 Rathburn, Urol. and Cut. Rev., 1919, xxiii, 77 Caulk, John R., Ann. Surg., July, 1923, lxxvii, 65
Separated and sutured lower hydronephrosis to skin
Recovery
Removed calculus upper half
Recovery
Decapsulation of both for Recovery nephritis Decapsulation of upper half Not much provement and fixation for mobility Attempt m ade to replace kid- Recovery ney in more normal posit ion
Gro up III. Weibel, 1908, Weiner K lin. Wochenschrift, 1908, xlvii, 1632 Dermiteanu, Deut. Med. Wochschr., 1908, xxx, 1333 Zuckerkandl, Wiener K lin. Wochschrift, 1909, !ix, 1767 Sexton
Palliat ive operations
Kidney divided. Bot h halves placed as high as possible
Recovery
Kidney elevated
Recovery
Stone remove d from pelvis of one kidney Calculus removed from one ureter Decapsulation and fixation of mass
Recovery Recovery Recovery
Resection of ectopic portion Removed ectop ic left kidney
(upper)
Recovery
Resection of lower h alf
Recovery
Resected lower segment of fused kidney for tuberculosis Resection ectopic kidney
Recovery
403
Recovery
im-
- - - - - - - - - - - - - - - -- - - - - - - -- - - - - - - - - -
404
W. G. SEXTON
Present illness. Began three weeks previous to admission with severe abdominal pain followed by vomiting. This lasted about two hours and then gradually lessened in severity. Two weeks later he had a second attack of very sharp pain in the abdomen and noticed a mass just below the umbilicus. He then detected a purulent discharge from the urethra which was followed on the next day with bloody urine. This attack of pain lasted three days, and was accompanied by a mass below the umbilicus. Four days ago pain again appeared and was present at time of admission. The patient did not notice whether or not the mass disappeared between attacks. His urine has remained bloody and he has had a more or less constant urethral discharge, and for the past four days he has been having some chills and fever.
Fm. I.
LATERAL
Vrnw
OF PATIENT SHOWING PROMINENT MAss JusT BELOW UMBILICUS.
Physical examination. Well developed and well nourished white male, nineteen years of age. H e is 6 feet 1 inch tall, weighing 155 pdunds. Walked into the hospital. Temperature 101, pulse 84. Respirations 20. General physical examination with exception of abdomen revealed nothing abnormal. Abdomen : On inspection t here is a definite mass (fig. 1) visible just below the umbilicus and nearly in the median line but slight ly toward the left side. This forms a prominent mass 12 cm. in length in the mid line and about the size of a grape fruit. The right lateral border was 9 cm. from the mid line. On the left t he fullness extends to t he anterior superior spine of the ilium and fills the left flank. The mass is very tense but feels like fluid llnder pressure. There is a slight degree
RESECTION OF UNILATERAL FUSED KIDNEY
405
of lateral mobility. There is very slight movement during deep inspiration. The left flank is more prominent than the right. The right kidney cannot be felt. Neither the liver nor the spleen can be palpated. Genitalia normal. Prostrate slightly softer than normal. Laboratory findings. Urine: Specific gravity 1016; acid; sugar 0; albumin; pus; red blood cells. Blood: Red blood cells, 4,3)0,000; white blood cells, 7600; hemoglobin, 85 per cent; differential count-Neutrophiles 75, basophiles 0, eosinophiles 1, small mononuclears 16, large mononuclears 6, transitional 2. Blood chemistry: Non-protein nitrogen, 42.4 mgm.; urea nitrogen, 23.83 mgm. Phenolsulphonephthalein: First hour, 5 per cent; second hour, 20 per cent. Urethral smears: Negative for gonococci. During the first twelve hours that the patient was in the hospital the mass disappeared, his pain left and his temperature dropped to normal. First cystoscopy September 1, 1924. Bladder practically normal. No evidence of calculi, tumor or diverticulum. Ureteral orifices appeared small but in normal position. Both sides catheterized with No. 6 ureteral catheters. The urine from the right side was purulent and contained many pus cells and red blood cells. That from the left side was clear with only an occasional pus cell microscopically. During the night of September 6 his pain reappeared and the mass was again noticed. Second cystoscopy performed September 6, 1924. Both sides catheterized and catheters left in place. In two hours the right side excreted 750 cc. The left 125 cc. roentgenogram shows the right catheter passes across the spine over the sacrum to the left side. Thirty-five cubic centimeters sodium bromide was injected into the right catheter. Pyelogram shows a greatly distorted pelvis, with the left catheter pushed laterally. This suggested a fused kidney. The urine coming through the right catheter was much clearer than at former examination, but pus could be seen coming from the ureteral orifice around the catheter which suggested a partial duplication of the ureter. Six days later (September 12) the mass had reappeared and upon catheterization of right ureter 1250 cc. were obtained in two and onehalf hours with disappearance of the tumor. At this examination a
406
W. G. SEXTON
No. 8 catheter was used and after injecting 30 cc. of sodium bromide the catheter was partially withdrawn and 10 cc. more were injected in an attempt to visualize the ureter. This was unsuccessful, but the pelvic shadow showed it was very much larger and lower than was at first suspected. On September 16 the left side was injected with 10 cc. of sodium bromide and the pelvis of the left kidney located above the mass but in about normal position (fig. 2). The right catheter turned downwards after entering the pelvis, indicating that there was a very large pyonephrosis.
FIG.
2.
PYELOGRAM SHOWING CATHETER IN RIGHT URETER CROSSING OVER TO LEFT SIDE
Catheter in left ureter pushed laterally and pelvis injected with sodium bromide solution.
On September 19 the patient was in such good condition tha( he insisted upon leaving the hospital. He returned for observation October 6. He was advised to submit to operation. He went to Sheboygan, Wisconsin, his home where he was seen October 13 by Dr. Siegfried Kraft of the Sheboygan Clinic. At this time the mass had reappeared, and the patient was very ill. Dr. Kraft upon prostatic massage obtained many gonococci. Dr. Kraft very kindly sent the patient back to me with advice to submit to an immediate operation. He was readmitted October 17, complaining that his pain was very intense and that the tumor mass was larger than it had ever been and
RESECTION OF UNILATERAL FUSED KIDNEY
407
noticed to be above the umbilicus. Temperature 100.6. Pulse 84. Respiration 20. White blood cells 12,950. On examination of his abdomen the mass extended farther across the mid line than on the previous admission and was so large that extirpation seemed almost impossible. He was prepared for operation next morning. September 18. Previous to operation the patient was cystoscoped and both ureters catheterized. Twelve ounces of clear urine was obtained from the catheter in the left ureteral orifice and about 5 ounces of very thick pus obtained from the catheter in the right orifice. This was so thick that it ran out very slowly and was facilitated by suction. The catheters were left in place. At the end of one hour the large mass seen across the abdomen disappeared. This lead to the conclusion that we were dealing with a hydronephrosis of the left kidney due to pressure of an infected ectopic kidney upon the ureter of the left side. The patient was then taken to the operating room with the catheters in place to aid in locating the ureters. Description of operation. A long incision was made extending from the costo-vertebral angle down parallel to the crest of the ilium about two inches below the anterior superior spine. The muscles were cut and the kidney exposed (fig. 3). It was found upon palpation that there was a very large mass occupying the entire flank extending upward as far as could be reached with the hand, and downward as far as the brim of the pelvis, and medianly as far as the median line of the body. The lower half was a distinctly cystic sack which showed definite fluctuation. The upper half appeared to be quite normal kidney structure. There was a fairly definite line of demarcation evidenced by a white fibrous band that crossed this mass and indicated the site of fusion of the two kidneys, but it was impossible· to separate the two kidneys at this point. A trocar was then inserted into the lower mass and a little over a quart of purulent matter was drawn out by suction. We then began a careful separation of the lower half and found that the blood supply came from the left iliac and entered the kidney on the lateral surface. This was clamped, cut and tied. Both ureters were located, and it was found that they were both lying on the anterior surface of the kidney indicating that neither kidney had completely rotated. The ureter from the upper half was the thickness of one's finger, and passed under the ectopic portion while the ureter of the lower half was not enlarged. The mass extended so far downward and so far toward the middle line that its removal seemed almost impossible, but it was finally freed and delivered, and a resection was made about one inch
408
W. G. SEXTON
below the line of fusion which has been described. Several vessels were encountered in doing this resection from the fixed kidney; these were tied as they were met. There was no hemorrhage during the operation, but the patient's pulse became quite weak before the resection was complete. Hypodermoclysis of Boline and glucose was given while the patient was on the table and his pulse improved rapidly. The wound was packed with two large compresses and the muscles closed in layers of continuous catgut. Skin was closed with interrupted silkworm gut sutures.
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FIG.
3.
_:;;.:::~
JP.E...,,..CR
DRAWING ( DIAGRAMMATIC) OF CONDITION FOUND AT OPERATION
Descri ption of the specimen. The specimen is an irregularly elongated mass 18 cm. in length, 10 cm. wide at its widest portion and 6 cm. thick (fig. 4) . The anterior surface is very irregularly lobulated. Nearly all of the divisions between t he lobules run transversely across the mass but there is one large elongated lobule that follows the long axis of the mass. The lower end of the tumor has been broken into and the upper end follows the line of section. The posterior surface is comparatively smooth. The ureter cannot be recognized. The arterial supply seems to enter t he middle of the posterior surface. Upon section it is found
RESECTION OF UNILATERAL FUSED KIDNEY
409
that the mass is composed of merely a thin shell of renal tissue and the anterior is composed of many multilocular spaces. There apparently is very little normal renal tissue. Post-operative. His temperature ranged from 103 to 100 and became normal on the sixteenth day and then remained normal. November 22, phenolsulphonephthalein first hour 20 per cent, second hour, 30 per cent. He was discharged from the hospital November 25, 1924, with wound healed with the exception of two very small superficial granulating areas at either angle of the wound. He returned for observation December 30, 1924, at which time he was in excellent health. Weight 170 pounds. He had been working at his trade as a cheese maker for several weeks. He had a small sinus at the upper angle of the wound that had been discharging for about two weeks and may be a urinary fistula.
FIG. 4. PHOTOGRAPH OF ANTERIOR SURFACE OF ECTOPIC PORTION REMOVED
CONCLUSIONS
The number of cases of fused kidney that have been reported in recent literature indicates that this condition is probably not as rare as we have believed. By utilizing our modern urological methods of diagnosis in a careful methodical manner we should be able to detect more of these cases. REFERENCES
(1) (2)
Surgical pathology of the urinary tract in infants. Jour. Amer. Med. Assoc., December 13, 1924, lxxxiii, 1887. BRAASCH: Clinical diagnosis of congenital anomaly in kidney and ureter. Ann. Surg., November, 1912, lvi, 726. BuGBEE-WoLLSTEIN:
--
410
~--- - - - - - - - -
W. G. SEXTON
(3) ErsENDRATH: The relation of variation in the renal vessels to pyelotomy and nephrotomy. Ann. Surg., June, 1920, lxxi, 726. (4) MERTZ: Bilateral duplication of ureters with compilation of recorded cases. Urol. and Cut. Rev., November, 1920, xxiv, 637. (5) BuERGER-LEo: Infection of a fused kidney with duplication of left renal pelvis. Surg. Gny. Obst., xxviii, 183. (6) HEPBURN, THo. N.: Kidney and- ureter abnormalities. Ann. Surg., September, 1918, !xviii. (7) DORLAND: Consideration of renal anomalies with presentation of two cases. Surg. Gyn. and Obst., 1911, xiii, 303. (8) EISENDRATH: Congential malformation of ureters. Ann. Surg., April, 1912, Iv, 571. (9) MAYO, CHAS. H.: The surgery of the single and horse-shoe kidney. Ann. Surg., April, 1913, xvii, 511. (10) RATHBURN: Solitary· kidney with unusual anomaly of the ureter. Case report . Urol. and Cut. Rev., 1919, xxiii, 77. (11) MUELLER, A. : Zur Diagnose und Operation fur Akzessorisches Niere. Zeitsch. f. Urol. Chir., ix, 141. (12) KRETSCHMER: Supernumerary Kidney. Jour. Amer. Med. Assoc., 1915, !xv, 1447. (13) PILSHER, L. A.: Kidney with double pelvis and double ureter. Nephrolithiasis, Pyonephrosis, Nephrectomy. Ann. Surg., May, 1917, !xv, 534. (14) HAMER AND MERTZ: Radiographic findings in double kidneys. (15) MERTZ: A study of t he pelvis of the double kidney. Jour. Urol., xi, 259 (16) EISENDRATH: Double kidney. Ann. Surg., lxxvii, 450. (17) HuFMAN: Congenital displacement of kidneys. Jour. Urol., October, 1924, xii, 364. (18) KEIBEL AND MALL (Felix, W. ): The development of the genito-urinary organs. Human Embryology, ii, 654, Lippincott Co. (19) BUGBEE AND LosEE: Clinical significance of congenital anomalies of kidney a nd ureter. Surg. Gyn. and Obst., February, 1919, xxviii, 97. (20) PAPIN AND PALIZOLLI: Ann. Mal. G. U., 1909, xxvii, 1681. (21) HYMEN, A.: Unilateral fused kidneys. Zeitsch. f . Urol. Chir., ix, 157.