- Email: [email protected]
Unilateral Fused Kidneys1
UNILATERAL FUSED KIDNEYS1 A.HYMAN Mt. Sinai and Lebanon Hospitals, New Yorlc City
The comparative rarity of unilateral fused kidney, and its clinical importance, warrants, I believe, the following description of two cases observed within the past two years. Unilateral fused kidney is one of the most unusual forms of congenital renal anomalies. In this condition, one kidney (the right usually) is transposed to the opposite side of the body where it is fused to the lower end of the left organ, thus forming a single fused kidney (sigmoid kidney). Depending upon the degree of union and its location, three types of fused kidney have been described; the most common form is the horse-shoe kidney, in which the fusion is slight, and generally at the lower pole, one organ being placed on either side of the spinal column. In the second form, the fusion is complete, and the organ is generally situated in the median line; this is known as the discshaped kidney. Between these types is the unilateral fused kidney above described. The rarity of this condition may be gathered from the fact that at autopsy the unilateral fused kidney is found but once in over 8000 cases; whereas the· horseshoe kidney is encountered once in approximately 1000 cases. In operative material, the incidence is more frequent, Israel reporting two fused and five horse-shoe kidneys in 800 operations. The reason for such discrepancies lies in the fact, that congenitally dystopic kidneys, on account of anomalies of the blood vessels, and ureters, are more prone to disease than normally situated organs. Up to within a few years, seventy cases of this anomaly have been compiled from the literature. The diagnosis is seldom made previous to operation or autopsy. In both of our cases, the condition was recognized and an exact clinical and pathological 1
Froro the Surgical Service of Dr. Beer, Mt. Sinai H ospital. 321
322
A. HYMAN
diagnosis established on cystoscopic and pyelographic data. The practical importance of recognizing this condition is readily apparent, as errors in diagnosis have resulted in the removal of the single fused kidney in a few instances. The presence of such an organ, especially when ptosed into the pelvis, is likely to lead to unpleasant and dangerous complications during parturition. The symptomatology of unilateral fused kidney is very vague and misleading. There are no characteristic subjective complaints, in fact there may be no symptoms, unless there is a pathological condition of the kidney itself, or the organ is so placed, as to cause pressure symptoms. The diagnosis is seldom made by physical examination, for although an enlarged organ may be palpated, the difficulty of interpreting it as a fused kidney can readily be imagined. The diagnosis can only be made with certainty, by a combined cystoscopic and radiographic examination. Cystoscopy alone affords no information, on account of the normal trigonal region. The fused kidney has two ureters which always open normally into the bladder, one on each side of the trigone. Ureteral catheterization, especially when testing the pelvic capacity may give some clue to the condition, for the patient will feel the pain caused by the distention of both pelves on the same side of the body. A positive diagnosis can only be made by a pyelogram, or the insertion of opaque catheters. The surgery of fused kidney is necessarily more complicated and dangerous than that of the normal organ. Still, instances are reported by Albarran, Zuckerkandl and others in which the lower half of the diseased organ was successfully removed. In one of our cases a nephrotomy was performed, the other patient had no pathological condition warranting operation. Case I. Male, age twenty-four years, was admitted to the hospital April 18, 1920. His family and past history were negative. For the past two years, he has had attacks of pain in the left lumbar region, radiating anteriorly to the umbilicus and posteriorly to the spine. The pain never very severe in character, occurred once to twice a week, and lasted but ten to fifteen minutes. These attacks were accompanied by frequent urination and hematuria. The patient was discharged from
UNILATERAL FUSED KIDNEYS
323
the army on account of his trouble, no definite diagnosis having been made. Physical examination demonstrated a man in good general condition. On abdominal palpation, a mass rounded in contour and of a cystic consistence was felt, extending from the left lumbar region, down to below the umbilicus. This mass was slightly mobile, and at times tender. Liver, spleen and kidneys not palpable. The urine was slightly cloudy, specific gravity 1.020, and showed a moderate number of pus and red blood cells, and also a trace of albumen. The phenolsulphonephthalein test for two hours observation was 45 per cent; the blood chemistry was practically normal. The radiographic report was as follows: "There is a concretion the size of a cherry in the left lumbar region close to the crest of the ilium, which is suggestive of a uretera] calculus." Cystoscopic examination revealed a normal bladder, trigone and normally situated ureteral orifices. Both ureters were catheterized, and no obstruction encountered. From the right a profuse flow of cloudy urine was obtained, indicating retention in the pelvis. No indigo-carmine was secreted in thirty minutes observation. The capacity of this pelvis was determined by the distention test and found to be 12 cc. The urine from the left side, that is, the side of which the patient complained, and where the stone shadow was noted, was clear, with good indigo-carmine secretion in twenty minutes. The catheterized urines showed the following: Right. Urea 1.0, many pus cells, red blood cells epithelium. Left. Urea 1.5, red blood cells and epithelium. The result of this cystoscopy left us in doubt as to the exact condition of affairs, for all the symptoms, including the suspected stone shadow were on the left side; while normal urine was obtained from this side, and pathological urine from the right kidney. lt was then considered advisable to take a pyelogram of the left kidney, to determine the exact location of the shadow shown in the radiogram. The findings of this examination are shown in figure 1, as a result of which we were of the opinion that the shadow in question was either extra-renal or ureteral, and probably due to a calcified node. A second cystoscopy confirmed the first one. At this time two opaque catheters were introduced, and a radiogram taken, with the result that the paradoxical findings above described were immediately clarified (figs. 2 and 3). This shows the right ureter catheter crossing the spinal column, and coming in contact with the stone shadow, which was situated in the lower pelvis of a fused kidney. This immediately explained the urinary findings from this THE JOURNAL OF UROLOGY, VOL, VII, NO.
4
324
A. HYMAN
side. The left catheter enters the upper pelvis of the fused kidney. A diagnosis was then made of a calculus situated in the lower pelvis of a fused kidney. Operation (Dr. Beer); through a left lumbar incision
FIG. 1.
demonstrated a large kidney situated on the left side of the spine. The organ represented the fusion of both kidneys with a constriction at the junction of the middle and lower thirds. Both pelves came off anteri-
UNIL,ATERAL FUSED KIDNEYS
325
orly, the ureter of the upper pelvis, passing anterior to the lower pole. The vessels to the lower pole were derived from either the left common ilias, or the external iliac arteries. A calculus the size of a large cherry
FIG. 2.
was palpated in the lower pelvis, which was opened and the stone extracted. The pelvis was sutured, and the wound closed with drainage. The patient made an uninterrupted recovery and was discharged, well, in a few weeks time. This is one of the few cases reported, in which an
326
A. HYMAN
exact preoperative diagnosis of fused kidney was made, and demonstrates how exact a clinical and anatomical diagnosis can be made by utilizing the modern methods which we have at our disposal. Case II. Female, age thirty-two years, admitted to Mt. Sinai Hospital May 20, 1920. Family and past history negative. Complains of a dull pain in the right lumbar region for past four years. Pain does not
FlG . 3.
radiate, but is aggravated when in the sitting posture. No urinary disturbances. Physical examination is negative. The abdomen is lax, no ·masses are palpable. Liver, spleen and kidneys cannot be palpated. Urineacid-1.018-trace of albumen-moderate epethelial cells. Phenosulphonephthalein test 40 per cent for two hours. Blood chemistry normal.
UNILATERAL FUSED KIDNEYS
327
Cystoscopy demonstrates a normal bladder and trigone, with normally situated orifices. Both ureters were catheterized and clear urine with good equal indigo-carmine secretion was obtained from both kidneys. An X-ray of the urinary tract showed a suspicious shadow in the left
FIG.
4.
lower quadrant of the pelvis, and in order to determine its relation to the ureter radiograms were then taken with two opaque catheters in situ. These showed that the left ureter crossed the spine going towards the right kidney. A pyclogram was taken injecting through both catheters, revealing a fused right kidney, with two seperate pelves (fig. 4).
328
A. HYMAN
As far as could be determined there was nothing of a pathological nature besides this congenital anomaly, and the pain was probably due to pressure symptoms. These did not warrant an exploratory operation and the patient was therefore discharged from the hospital.
In both instances, the diagnosis was made by X-rays taken with shadowgraph catheters in situ. In all obscure cases, or when a renal anomaly is suspected, it would seem to be advisable to take radiograms with opaque catheters in both ureters. The information so obtained may prove invaluable.
The comparative rarity of unilateral fused kidney, and its clinical importance, warrants, I believe, the following description of two cases observed within the past two years. Unilateral fused kidney is one of the most unusual forms of congenital renal anomalies. In this condition, one kidney (the right usually) is transposed to the opposite side of the body where it is fused to the lower end of the left organ, thus forming a single fused kidney (sigmoid kidney). Depending upon the degree of union and its location, three types of fused kidney have been described; the most common form is the horse-shoe kidney, in which the fusion is slight, and generally at the lower pole, one organ being placed on either side of the spinal column. In the second form, the fusion is complete, and the organ is generally situated in the median line; this is known as the discshaped kidney. Between these types is the unilateral fused kidney above described. The rarity of this condition may be gathered from the fact that at autopsy the unilateral fused kidney is found but once in over 8000 cases; whereas the· horseshoe kidney is encountered once in approximately 1000 cases. In operative material, the incidence is more frequent, Israel reporting two fused and five horse-shoe kidneys in 800 operations. The reason for such discrepancies lies in the fact, that congenitally dystopic kidneys, on account of anomalies of the blood vessels, and ureters, are more prone to disease than normally situated organs. Up to within a few years, seventy cases of this anomaly have been compiled from the literature. The diagnosis is seldom made previous to operation or autopsy. In both of our cases, the condition was recognized and an exact clinical and pathological 1
Froro the Surgical Service of Dr. Beer, Mt. Sinai H ospital. 321
322
A. HYMAN
diagnosis established on cystoscopic and pyelographic data. The practical importance of recognizing this condition is readily apparent, as errors in diagnosis have resulted in the removal of the single fused kidney in a few instances. The presence of such an organ, especially when ptosed into the pelvis, is likely to lead to unpleasant and dangerous complications during parturition. The symptomatology of unilateral fused kidney is very vague and misleading. There are no characteristic subjective complaints, in fact there may be no symptoms, unless there is a pathological condition of the kidney itself, or the organ is so placed, as to cause pressure symptoms. The diagnosis is seldom made by physical examination, for although an enlarged organ may be palpated, the difficulty of interpreting it as a fused kidney can readily be imagined. The diagnosis can only be made with certainty, by a combined cystoscopic and radiographic examination. Cystoscopy alone affords no information, on account of the normal trigonal region. The fused kidney has two ureters which always open normally into the bladder, one on each side of the trigone. Ureteral catheterization, especially when testing the pelvic capacity may give some clue to the condition, for the patient will feel the pain caused by the distention of both pelves on the same side of the body. A positive diagnosis can only be made by a pyelogram, or the insertion of opaque catheters. The surgery of fused kidney is necessarily more complicated and dangerous than that of the normal organ. Still, instances are reported by Albarran, Zuckerkandl and others in which the lower half of the diseased organ was successfully removed. In one of our cases a nephrotomy was performed, the other patient had no pathological condition warranting operation. Case I. Male, age twenty-four years, was admitted to the hospital April 18, 1920. His family and past history were negative. For the past two years, he has had attacks of pain in the left lumbar region, radiating anteriorly to the umbilicus and posteriorly to the spine. The pain never very severe in character, occurred once to twice a week, and lasted but ten to fifteen minutes. These attacks were accompanied by frequent urination and hematuria. The patient was discharged from
UNILATERAL FUSED KIDNEYS
323
the army on account of his trouble, no definite diagnosis having been made. Physical examination demonstrated a man in good general condition. On abdominal palpation, a mass rounded in contour and of a cystic consistence was felt, extending from the left lumbar region, down to below the umbilicus. This mass was slightly mobile, and at times tender. Liver, spleen and kidneys not palpable. The urine was slightly cloudy, specific gravity 1.020, and showed a moderate number of pus and red blood cells, and also a trace of albumen. The phenolsulphonephthalein test for two hours observation was 45 per cent; the blood chemistry was practically normal. The radiographic report was as follows: "There is a concretion the size of a cherry in the left lumbar region close to the crest of the ilium, which is suggestive of a uretera] calculus." Cystoscopic examination revealed a normal bladder, trigone and normally situated ureteral orifices. Both ureters were catheterized, and no obstruction encountered. From the right a profuse flow of cloudy urine was obtained, indicating retention in the pelvis. No indigo-carmine was secreted in thirty minutes observation. The capacity of this pelvis was determined by the distention test and found to be 12 cc. The urine from the left side, that is, the side of which the patient complained, and where the stone shadow was noted, was clear, with good indigo-carmine secretion in twenty minutes. The catheterized urines showed the following: Right. Urea 1.0, many pus cells, red blood cells epithelium. Left. Urea 1.5, red blood cells and epithelium. The result of this cystoscopy left us in doubt as to the exact condition of affairs, for all the symptoms, including the suspected stone shadow were on the left side; while normal urine was obtained from this side, and pathological urine from the right kidney. lt was then considered advisable to take a pyelogram of the left kidney, to determine the exact location of the shadow shown in the radiogram. The findings of this examination are shown in figure 1, as a result of which we were of the opinion that the shadow in question was either extra-renal or ureteral, and probably due to a calcified node. A second cystoscopy confirmed the first one. At this time two opaque catheters were introduced, and a radiogram taken, with the result that the paradoxical findings above described were immediately clarified (figs. 2 and 3). This shows the right ureter catheter crossing the spinal column, and coming in contact with the stone shadow, which was situated in the lower pelvis of a fused kidney. This immediately explained the urinary findings from this THE JOURNAL OF UROLOGY, VOL, VII, NO.
4
324
A. HYMAN
side. The left catheter enters the upper pelvis of the fused kidney. A diagnosis was then made of a calculus situated in the lower pelvis of a fused kidney. Operation (Dr. Beer); through a left lumbar incision
FIG. 1.
demonstrated a large kidney situated on the left side of the spine. The organ represented the fusion of both kidneys with a constriction at the junction of the middle and lower thirds. Both pelves came off anteri-
UNIL,ATERAL FUSED KIDNEYS
325
orly, the ureter of the upper pelvis, passing anterior to the lower pole. The vessels to the lower pole were derived from either the left common ilias, or the external iliac arteries. A calculus the size of a large cherry
FIG. 2.
was palpated in the lower pelvis, which was opened and the stone extracted. The pelvis was sutured, and the wound closed with drainage. The patient made an uninterrupted recovery and was discharged, well, in a few weeks time. This is one of the few cases reported, in which an
326
A. HYMAN
exact preoperative diagnosis of fused kidney was made, and demonstrates how exact a clinical and anatomical diagnosis can be made by utilizing the modern methods which we have at our disposal. Case II. Female, age thirty-two years, admitted to Mt. Sinai Hospital May 20, 1920. Family and past history negative. Complains of a dull pain in the right lumbar region for past four years. Pain does not
FlG . 3.
radiate, but is aggravated when in the sitting posture. No urinary disturbances. Physical examination is negative. The abdomen is lax, no ·masses are palpable. Liver, spleen and kidneys cannot be palpated. Urineacid-1.018-trace of albumen-moderate epethelial cells. Phenosulphonephthalein test 40 per cent for two hours. Blood chemistry normal.
UNILATERAL FUSED KIDNEYS
327
Cystoscopy demonstrates a normal bladder and trigone, with normally situated orifices. Both ureters were catheterized and clear urine with good equal indigo-carmine secretion was obtained from both kidneys. An X-ray of the urinary tract showed a suspicious shadow in the left
FIG.
4.
lower quadrant of the pelvis, and in order to determine its relation to the ureter radiograms were then taken with two opaque catheters in situ. These showed that the left ureter crossed the spine going towards the right kidney. A pyclogram was taken injecting through both catheters, revealing a fused right kidney, with two seperate pelves (fig. 4).
328
A. HYMAN
As far as could be determined there was nothing of a pathological nature besides this congenital anomaly, and the pain was probably due to pressure symptoms. These did not warrant an exploratory operation and the patient was therefore discharged from the hospital.
In both instances, the diagnosis was made by X-rays taken with shadowgraph catheters in situ. In all obscure cases, or when a renal anomaly is suspected, it would seem to be advisable to take radiograms with opaque catheters in both ureters. The information so obtained may prove invaluable.