- Email: [email protected]
Renal Ectopia: Report of Two Cases1
RENAL ECTOPIA: REPORT OF TWO CASES 1 CARL F. RUSCHE
AND
JAMES L. BRAY
From the Department of Surgery (Urology), University of Southern California School of Medicine, Los Angeles
Ectopia of the kidney is a particularly interesting urinary anomaly; because the clinical picture is often so masked as to defy recognition. For a clear understanding of the following, the term ectopia is applied to the kidney which is congenitally misplaced and which has therefore never occupied its normal anatomical position. Bearing this definition in mind will serve to avoid confusion with renal dystopia or ptosis, which indicates an acquired displacement. The ptosed kidney is freely movable, has a normal blood supply and a ureter of usual length; while the ectopic organ is generally fixed, has an abnormal blood supply and a short ureter. Prior to the introduction of urography renal ectopia was rarely diagnosed clinically, and most of the reported cases were discovered either at the time of surgery or at autopsy. Symptoms are not purely renal, but frequently simulate pelvic or intra-abdominal disease, and while an ectopic kidney may be the seat of any disease capable of involving the normally placed kidney, it may in addition present symptoms attributable to its abnormal locale. For this reason, as well as due to its rare incidence, the condition probably received little consideration, attention being directed to the more common disturbances of adjacent viscera to which its symptoms apparently referred. Modern urologic methods, however, have simplified this diagnostic problem, although a common pitfall is the assumption that a urine within normal limits indicates a sound urinary tract. Certain cases of renal malposition present a strong argument against this misconception, and drive home the need for urologic study in cases of obscure pelvic and abdominal symptoms. EMBRYOLOGY
Ectopia of the kidney is an embryologic maldevelopment. In the 5 mm. embryo, the kidneys appear as 2 buds which lie close together in front of the second sacral segment (9). As the kidney buds migrate upward, they receive a temporary blood supply from the aorta, the common and external iliac, the median sacral and other neighboring vessels. The permanent blood supply is not obtained until the kidneys reach their normal position at the 25-30 mm. stage of the embryo. For some reason, the ascent of the ectopic kidney is arrested, a phenomenon which may take place at any stage of embryologic evolution. The temporary blood supply to it then becomes its permanent supply, and represents the stage at which the anomaly has occurred (1). This explains the numerous vessels furnishing the ectopic kidney and the fact that it is seen in a variety of positions and degrees of rotation. The adrenal gland usually occupies its 1 Read at annual meeting, American Urological Association, New York, N. Y., June 1, 1942.
123
124
CARL F. RUSCHE AND JAMES L. BRAY
normal position and is rarely fwrn;l in proximity to the ectopic kidney. velopmental disturbances in the sexual organs m~y coexist (2).
De-
INCIDENCE
With the advent of urography, the reported cases of this anomaly have shown a marked increase. Campbell (3), in 1930, published a survey of 47,477 autopsies, among which there were 72 ectopic kidneys, an incidence of 1 to 660. Thomas and Barton (10) in 1936, in a series of 22,000 autopsy reports, found 22 ectopic kidneys, an incidence of 1 to 1,000, and during 3,285 urologic examinations discovered 6 ectopic kidneys-an incidence of 1 to 547. Thompson and Pace (11), in reviewing the records of the Mayo Clinic up to February 1, 1936, were able to find 97 cases, of which 9 were encountered in approximately 11,000 autopsies and the remaining 88 were seen clinically. Fifty-two of these 88 cases were revealed during urologic examination, and the other 36 at the time of surgical exploration. These 88 clinical cases represent an incidence of 1 in 10,000 patients. MacKenzie and Hawthorne (8) reported 13 cases in 15,000 admissions to their service. Herman (6), in 1938, contended that ectopia comprised 16.9 per cent of renal anomalies. Eisendrath and Rolnick (4) found 205 cases of simple renal ectopia in 207,321 autopsy reports occurring in the literature. They are of the opinion that the clinical incidence is greater, estimates "varying from 1 in 500 to 1 in 800 patients." Lowsley and Kirwin (7) state that statistics variously place the incidence of ectopic kidney at approximately 1 out of 660 to 1,000 persons. SYMPTOMS
There is no definite group of symptoms peculiar to this anomaly. An ectopic kidney is subject to all the morbid changes which may beset its normal counterpart, and is rendered even more vulnerable as a consequence of its position. This is explained on the basis of impaired drainage and possible trophic defects due to its aberrant blood supply. Symptoms may appear as the result of renal pathology or of pressure from crowded contiguous structures, and often lead to a confused interpretation and a mistaken diagnosis of some pelvic or intra-abdominal condition of retroperitoneal neoplasm. On the other hand, and ectopic kidney may produce no symptoms, and the patient may live his normal life span without being aware of his condition. Fowler (5) states, in reviewing a clinical group of 22 cases: "The predominating symptom was pain. The pain varied in intensity from a deep-seated ache to intermittent attacks of acute sharp, stabbing colicky pains. In character, location and radiation, it does not resemble a typical pain of renal or ureteral origin." Because of the position of an ectopic kidney, pain is referred to the lower abdomen, and therefore suggests a low abdominal lesion in the male or a pelvic involvement in the female. While this anomaly occurs with approximately equal frequency in the sexes, it is more likely to be discovered in the female because pelvic distress will bring her to examination or possibly interfere with normal delivery. It is extremely difficult to distinguish between the clinical picture of ectopic kidney and that of certain gynecologic disturbances, and here urography becomes a valuable diagnostic aid.
RENAL ECTOPIA
125
DIAGNOSIS
Diagnosis is not difficult with the aid of urography, and information obtained by this means is more complete from retrograde study than from the intravenous urogram. An ectopic kidney may lie anywhere between the third lumbar vertebra and the hollow of the sacrum (5), although in rare cases it has been found in the thoracic cavity or has been known to herniate through the inguinal canal (10). As a rule it is smaller than average, may be smooth or lobulated, and its shape is influenced by the surrounding structures. In some instances it appears smooth and round, accounting for the descriptive terms "disk" and "cake" kidney. Usually it is in incomplete rotation, the pelvis lying anteriorly, or it may be rotated so that the pelvis occupies a posterior position. Two main types of renal ectopia exist: simple and crossed, which in turn may appear in unilateral or bilateral arrangement. Simple unilateral malposition is the commonest form. In crossed ectopia, the anomalous organ lies on the same side and below the level of the normal kidney, and its ureter empties into the opposite side of the bladder. This is the chief point of differentiation from unilateral double kidney, which is marked by absence of the contralateral organ. In unilateral double kidney, the ureter either joins that of its mate or empties into the bladder on the same side. Fusion of the crossed ectopic with the ipsilateral normal kidney may or may not be present. There should be no difficulty in distinguishing between the ptosed and the ectopic kidney, since the latter is fixed in position because of a short ureter, manifests an aberrant blood supply composed of short trunks and absence of the fatty capsule. The ptosed kidney is usually freely movable, if it is not bound in position by adhesions, and always has a ureter approximating normal length. Opaque catheters in place will reveal the length and course of the ureters on roentgenologic examination. A renal calculus showing on the x-ray film in a position lower than normal is diagnostic evidence of a misplaced kidney, and a palpable mass such as that of an enlarged kidney but at an inferior location is significant. Since the symptoms of ectopic kidney are predominantly lower abdominal or pelvic, complete urologic study will prevent an erroneous diagnosis as well as the chagrin of the surgeon who removes an appendix or female pelvic organs only to. find that the original symptomatology persists. TREATMENT
The ectopic kidney, if symptomless, should be left alone. When symptoms warrant, and if the condition of the other kidney is good and its function adequate, operative intervention should be undertaken. Such a kidney, because it is fixed in position by adhesions and numerous aberrant vessels, does not lend itself to plastic surgery, and therefore nephrectomy is the procedure of choice. The transperitoneal route is the surgical approach recommended, because it provides easy access to the kidney, permits ligation of blood vessels as they are encountered and minimizes damage to the large vessels which may lie directly beneath the organ. Anson, Pick and Cauldwell (1), in their study of the anatomy of the ectopic kidney, found the bed of the kidney to be made up almost totally
126
CARL F. RUSCHE AND JAMES L. BRAY
of large pelvic arteries and veins, an anatomical arrangement of grave importance in urologic surgery. Some operators prefer the extraperitoneal route, approaching the kidney as one would a stone in the lower ureter. MacKenzie and Hawthorne (8} have used the extra-peritoneal approach in all their cases, even the low pelvic type. To demonstrate several of the above conclusions, which are in no way original with us, we shall present the clinical histories of two recent personal cases of unilateral ectopic kidney, and our surgical technic will be shown in motion pictures in color. CASE REPORTS
Case 1. A white female, aged 31 years, was admitted to the hospital on October 26, 1941. She complained of attacks of cramp-like pain in the abdomen associated with distention for six years. Appendectomy had been performed 5 years ago without relief of symptoms. X-ray examination of the gastrointestinal tract 3 years ago was reported normal. During the last 6 months, the attacks of abdominal cramps had become so frequent and severe that the patient was unable to continue her work as a stenographer. Familial and early personal history were not relevant. Examination revea~ed a very well nourished female, who was alert, cooperative, and in no acute distress. Temperature was 98.6°; pulse 80. The abdomen was characterized by a hard, fixed mass about the size of a large orange, located to the right of and somewhat below the umbilicus. Urine was both chemically and microscopically normal. Blood non-protein nitrogen 28 mg per 100 cc. Blood Wassermann and Kahn were reported negative. Hemoglobin 82 per cent; red blood cells 4,520,000; white blood cells 9,600; 70 per cent polymorphonuclear leucocytes. Cystoscopic examination revealed no bladder pathology. Indigocarmine appeared in good concentration from the left ureteral orifice in 5 minutes and from the right in 9. The ureters were readily catheterized and specimens of urine obtained from the renal pelves were normal. Kidney, ureter and bladder roentgenogram presented a normal left kidney shadow, but the right renal shadow was not visualized. Bilateral pyelograms (fig. 1) disclosed a normal left kidney outline with a well filled, normal appearing pelvis. On the right there was a mass, apparently the right kidney shadow, with the upper pole at the level of the fourth lumbar vertebra. The calyces and pelvis were directly over the sacroiliac joint. The ureter appeared shortened and kinked. When the patient assumed the erect position, the right kidney moved less than two centimeters. The diagnosis of right ectopic kidney was definitely established. Surgical removal of the right kidney was recommended and accepted. Operation (October 27, 1941, recorded in motion pictures in color): Under spinal anaesthesia, an incision was made parallel to and 2 inches to the right of the abdominal midline, commencing just above the pubis and extending a few inches above the umbilicus. After the peritoneum had been opened, the intraabdominal contents were readily displaced to expose the right ectopic kidney. The posterior peritoneum was incised longitudinally, bringing the anomalous
RENAL ECTOPIA
127
kidney into the field. The renal pelvis faced anteriorly, indicating failure of rotation. Blood vessels, branches of the internal iliac artery, entered the kidney. These were clamped and ligated separately. After division of the short ureter the kidneywas removed, giving excellent visualization of the iliac vessels. The parietal peritoneum was closed, followed by the closure of the other abdominal structures without drainage. Pathologic Report: "Specimen consists of a flat, somewhat lobulated kidney measuring 8½ by 6 by 3 cm. On the posterior surface is a groove apparently to
Frn. 1. Case l. Bilateral pyelogram showing normal left kidney and ureter. Pelvic position of right kidney shows bizarre outline of pelvis and calyces, demonstrating failure of ascent and rotation of organ.
accommodate the iliac vessels. There are three or four separate arteries making up the blood supply. Anteriorly there are three extrarenal calyces uniting to form the renal pelvis, which gives origin to the ureter." At no time did the temperature exceed 100°F. or the pulse 100. The abdominal wound healed by primary union. The patient was discharged from the hospital on the eleventh postoperative day, ambulatory and apparently in good health. Patient was interviewed 6 months following discharge and stated that she had been free from abdominal pain. Case 2. A white female, aged 29 years, was admitted to the hospital on Jan-
128
CARL F, RUSCHE AND JAMES L, BRAY
uary 7, 1942. She complained of right lower abdominal pain, nausea, vomiting and abdominal distention for three weeks. There was some associated frequency, and nocturia 2 to 3 times. She said that her bowels moved daily. Because of attacks of pain in the lower right abdomen, associated with nausea, vomiting and distention, the patient was operated upon for appendicitis in 1936. Some bilateral pelvic disease existed and bilateral salpingectomy was carried out at the same time. Due to the fact that symptoms continued following the above surgery, a subtotal hysterectomy, oophorectomy and separation of ad-
FIG. 2. Case 2. Bilateral pyelogram showing normal left kidney with right ectopic kidney occupying medial position directly in front of sacrum.
hesions was performed. She had been admitted to the hospital on at least. 3 previous occasions with similar symptoms, and discharged after medical treatment with the diagnosis of partial intestinal obstruction due to abdominal adhesions. Her last attack requiring hospitalization was 3 months previously. She was under observation for 10 days. Examination revealed a poorly nourished female, who was co-operative and in no acute distress. Temperature 99.3°; pulse 95. The abdomen was moderately distended and no masses were palpable. Pelvic examination was not satisfactory, but bimanual examination with the finger in the rectum disclosed a tender, palpable mass in the pelvis.
RENAL EC'IOPIA
129
The urine was grossly cloudy and contained a few red blood cells, pus cells and gram-positive cocci in clumps. Blood non-protein nitrogen 33 mg per 100 cc. Blood vVassermann and Kahn -were reported negative. Blood count revealed a secondary anemia. Cystoscopic examination presented mild evidence of cystitis. Ureteral orifices were normal in position, and indigo carmine appeared in good concentration from the left in 5 and from the right in 6 minutes. The ureters were readily catheterized, and specimens obtained from the renal pelves revealed a right renal infection. The plain x-ray and pyelograms (fig. 2) disclosed no evidence of calculi. There was present a normal left kidney and ureter, but no right renal shadow. The right ureteral catheter was coiled opposite the sacrum and an anomalous renal pelvis outline. Right nephrectomy ,vas recommended and accepted. Operation (January 27, 1942, recorded in motion pictures in color): Under spinal anesthesia, the same surgical procedure was carried out as in the previous patient. Pathologic Report: "Specimen consists of a kidney measuring 11 by 7 by 3.5 cm and weighing 120 gm. Structure does not have normal renal architecture and retains some fetal lobulation. Pedicle has a broad attachment and apparently adherent to its bed of an area 8 by 5 cm. Ureter bifurcates approximately 2 cm prior to entering the parenchyma. There is one rather large aberrant artery entering the periphery. Capsule strips with relative ease, leaving a smooth brown homogenous surface." Convalescence was uneventful, and the patient was discharged from the hospital February 8, 1942. The patient was interviewed on :VIay 21, 1942 and stated that she had had no further symptoms. · SUMMARY
Two cases of unilateral ectopic kidney are reported, their clinical history given and surgical treatment (transperitoneal nephrectomy) recorded through motion pictures in color. Symptoms are those of pelvic or intra-abdominal conditions rather than renal. Roentgenographic procedures, especially bilateral pyelography, offer a conclusive diagnosis. The procedure of nephrectomy is radical treatment but mandatory in obtaining a cure. The transperitoneal route is the approach of choice in dealing with this anomaly. 906 Taft Bldg., Los Angeles, Calif. 1137 Roosevelt Bldg., Los Angeles, Calif. REFERENCES (1) ANSON, B. J., PICK, J. W. AND CAULDWELL, E. W.:Theanatomyofcommonerrenal
anomalies: Ectopic and horseshoe kidneys. J. Urol., 47: 112-130, 1942. (2) BEER, E. AND FERBER, W. L. F.: Crossed renalectopia (unilateral fused or elongated kidney). J. Urol., 38: 541-561, 1937.
130
CARL F. RUSCHE AND JAMES L. BRAY
(3) CAMPBELL, M. F.: Renal ectopy. J. Urol., 24: 187-198, 1930. (4) ErsENDRATH, D. J. AND ROLNICK, H. C.: Urology. Philadelphia: J.B. Lippincott Co., 1938, ed. 2, p. 577. (5) FowLER, H. A.: Bilateral renal ectopia. J. Urol., 45: 795-812, 1941. (6) HERMAN, L.: Practice of urology. Philadelphia: W. B. Saunders Co., 1938, p. 170. (7) LowSLEY, 0. S. AND Kmwrn, T. J.: Clinical Urology. Baltimore: The Williams & Wilkins Co., 1940. Vol. 11, p. 1300. (8) MACKENZIE, D. W. AND HAWTHORNE, A. B.: The ectopic kidney. J. Urol., 39: 479-486, 1938. (9) OGDEN, H. D. AND MALTRY, E.: Pelvic single kidney; Case report. J. Urol., 44: 13-18, 1940. (10) THOMAS, G. J. AND BARTON, J.C.: Ectopic pelvic kidney. J. A.M.A.,106:197-201, 1936. (11) THOMPSON, G. J. AND PACE, J.M.: Ectopic kidney, a review of 97 cases. Surg., Gynec. and Obst., 64: 935-943, 1937.
AND
JAMES L. BRAY
From the Department of Surgery (Urology), University of Southern California School of Medicine, Los Angeles
Ectopia of the kidney is a particularly interesting urinary anomaly; because the clinical picture is often so masked as to defy recognition. For a clear understanding of the following, the term ectopia is applied to the kidney which is congenitally misplaced and which has therefore never occupied its normal anatomical position. Bearing this definition in mind will serve to avoid confusion with renal dystopia or ptosis, which indicates an acquired displacement. The ptosed kidney is freely movable, has a normal blood supply and a ureter of usual length; while the ectopic organ is generally fixed, has an abnormal blood supply and a short ureter. Prior to the introduction of urography renal ectopia was rarely diagnosed clinically, and most of the reported cases were discovered either at the time of surgery or at autopsy. Symptoms are not purely renal, but frequently simulate pelvic or intra-abdominal disease, and while an ectopic kidney may be the seat of any disease capable of involving the normally placed kidney, it may in addition present symptoms attributable to its abnormal locale. For this reason, as well as due to its rare incidence, the condition probably received little consideration, attention being directed to the more common disturbances of adjacent viscera to which its symptoms apparently referred. Modern urologic methods, however, have simplified this diagnostic problem, although a common pitfall is the assumption that a urine within normal limits indicates a sound urinary tract. Certain cases of renal malposition present a strong argument against this misconception, and drive home the need for urologic study in cases of obscure pelvic and abdominal symptoms. EMBRYOLOGY
Ectopia of the kidney is an embryologic maldevelopment. In the 5 mm. embryo, the kidneys appear as 2 buds which lie close together in front of the second sacral segment (9). As the kidney buds migrate upward, they receive a temporary blood supply from the aorta, the common and external iliac, the median sacral and other neighboring vessels. The permanent blood supply is not obtained until the kidneys reach their normal position at the 25-30 mm. stage of the embryo. For some reason, the ascent of the ectopic kidney is arrested, a phenomenon which may take place at any stage of embryologic evolution. The temporary blood supply to it then becomes its permanent supply, and represents the stage at which the anomaly has occurred (1). This explains the numerous vessels furnishing the ectopic kidney and the fact that it is seen in a variety of positions and degrees of rotation. The adrenal gland usually occupies its 1 Read at annual meeting, American Urological Association, New York, N. Y., June 1, 1942.
123
124
CARL F. RUSCHE AND JAMES L. BRAY
normal position and is rarely fwrn;l in proximity to the ectopic kidney. velopmental disturbances in the sexual organs m~y coexist (2).
De-
INCIDENCE
With the advent of urography, the reported cases of this anomaly have shown a marked increase. Campbell (3), in 1930, published a survey of 47,477 autopsies, among which there were 72 ectopic kidneys, an incidence of 1 to 660. Thomas and Barton (10) in 1936, in a series of 22,000 autopsy reports, found 22 ectopic kidneys, an incidence of 1 to 1,000, and during 3,285 urologic examinations discovered 6 ectopic kidneys-an incidence of 1 to 547. Thompson and Pace (11), in reviewing the records of the Mayo Clinic up to February 1, 1936, were able to find 97 cases, of which 9 were encountered in approximately 11,000 autopsies and the remaining 88 were seen clinically. Fifty-two of these 88 cases were revealed during urologic examination, and the other 36 at the time of surgical exploration. These 88 clinical cases represent an incidence of 1 in 10,000 patients. MacKenzie and Hawthorne (8) reported 13 cases in 15,000 admissions to their service. Herman (6), in 1938, contended that ectopia comprised 16.9 per cent of renal anomalies. Eisendrath and Rolnick (4) found 205 cases of simple renal ectopia in 207,321 autopsy reports occurring in the literature. They are of the opinion that the clinical incidence is greater, estimates "varying from 1 in 500 to 1 in 800 patients." Lowsley and Kirwin (7) state that statistics variously place the incidence of ectopic kidney at approximately 1 out of 660 to 1,000 persons. SYMPTOMS
There is no definite group of symptoms peculiar to this anomaly. An ectopic kidney is subject to all the morbid changes which may beset its normal counterpart, and is rendered even more vulnerable as a consequence of its position. This is explained on the basis of impaired drainage and possible trophic defects due to its aberrant blood supply. Symptoms may appear as the result of renal pathology or of pressure from crowded contiguous structures, and often lead to a confused interpretation and a mistaken diagnosis of some pelvic or intra-abdominal condition of retroperitoneal neoplasm. On the other hand, and ectopic kidney may produce no symptoms, and the patient may live his normal life span without being aware of his condition. Fowler (5) states, in reviewing a clinical group of 22 cases: "The predominating symptom was pain. The pain varied in intensity from a deep-seated ache to intermittent attacks of acute sharp, stabbing colicky pains. In character, location and radiation, it does not resemble a typical pain of renal or ureteral origin." Because of the position of an ectopic kidney, pain is referred to the lower abdomen, and therefore suggests a low abdominal lesion in the male or a pelvic involvement in the female. While this anomaly occurs with approximately equal frequency in the sexes, it is more likely to be discovered in the female because pelvic distress will bring her to examination or possibly interfere with normal delivery. It is extremely difficult to distinguish between the clinical picture of ectopic kidney and that of certain gynecologic disturbances, and here urography becomes a valuable diagnostic aid.
RENAL ECTOPIA
125
DIAGNOSIS
Diagnosis is not difficult with the aid of urography, and information obtained by this means is more complete from retrograde study than from the intravenous urogram. An ectopic kidney may lie anywhere between the third lumbar vertebra and the hollow of the sacrum (5), although in rare cases it has been found in the thoracic cavity or has been known to herniate through the inguinal canal (10). As a rule it is smaller than average, may be smooth or lobulated, and its shape is influenced by the surrounding structures. In some instances it appears smooth and round, accounting for the descriptive terms "disk" and "cake" kidney. Usually it is in incomplete rotation, the pelvis lying anteriorly, or it may be rotated so that the pelvis occupies a posterior position. Two main types of renal ectopia exist: simple and crossed, which in turn may appear in unilateral or bilateral arrangement. Simple unilateral malposition is the commonest form. In crossed ectopia, the anomalous organ lies on the same side and below the level of the normal kidney, and its ureter empties into the opposite side of the bladder. This is the chief point of differentiation from unilateral double kidney, which is marked by absence of the contralateral organ. In unilateral double kidney, the ureter either joins that of its mate or empties into the bladder on the same side. Fusion of the crossed ectopic with the ipsilateral normal kidney may or may not be present. There should be no difficulty in distinguishing between the ptosed and the ectopic kidney, since the latter is fixed in position because of a short ureter, manifests an aberrant blood supply composed of short trunks and absence of the fatty capsule. The ptosed kidney is usually freely movable, if it is not bound in position by adhesions, and always has a ureter approximating normal length. Opaque catheters in place will reveal the length and course of the ureters on roentgenologic examination. A renal calculus showing on the x-ray film in a position lower than normal is diagnostic evidence of a misplaced kidney, and a palpable mass such as that of an enlarged kidney but at an inferior location is significant. Since the symptoms of ectopic kidney are predominantly lower abdominal or pelvic, complete urologic study will prevent an erroneous diagnosis as well as the chagrin of the surgeon who removes an appendix or female pelvic organs only to. find that the original symptomatology persists. TREATMENT
The ectopic kidney, if symptomless, should be left alone. When symptoms warrant, and if the condition of the other kidney is good and its function adequate, operative intervention should be undertaken. Such a kidney, because it is fixed in position by adhesions and numerous aberrant vessels, does not lend itself to plastic surgery, and therefore nephrectomy is the procedure of choice. The transperitoneal route is the surgical approach recommended, because it provides easy access to the kidney, permits ligation of blood vessels as they are encountered and minimizes damage to the large vessels which may lie directly beneath the organ. Anson, Pick and Cauldwell (1), in their study of the anatomy of the ectopic kidney, found the bed of the kidney to be made up almost totally
126
CARL F. RUSCHE AND JAMES L. BRAY
of large pelvic arteries and veins, an anatomical arrangement of grave importance in urologic surgery. Some operators prefer the extraperitoneal route, approaching the kidney as one would a stone in the lower ureter. MacKenzie and Hawthorne (8} have used the extra-peritoneal approach in all their cases, even the low pelvic type. To demonstrate several of the above conclusions, which are in no way original with us, we shall present the clinical histories of two recent personal cases of unilateral ectopic kidney, and our surgical technic will be shown in motion pictures in color. CASE REPORTS
Case 1. A white female, aged 31 years, was admitted to the hospital on October 26, 1941. She complained of attacks of cramp-like pain in the abdomen associated with distention for six years. Appendectomy had been performed 5 years ago without relief of symptoms. X-ray examination of the gastrointestinal tract 3 years ago was reported normal. During the last 6 months, the attacks of abdominal cramps had become so frequent and severe that the patient was unable to continue her work as a stenographer. Familial and early personal history were not relevant. Examination revea~ed a very well nourished female, who was alert, cooperative, and in no acute distress. Temperature was 98.6°; pulse 80. The abdomen was characterized by a hard, fixed mass about the size of a large orange, located to the right of and somewhat below the umbilicus. Urine was both chemically and microscopically normal. Blood non-protein nitrogen 28 mg per 100 cc. Blood Wassermann and Kahn were reported negative. Hemoglobin 82 per cent; red blood cells 4,520,000; white blood cells 9,600; 70 per cent polymorphonuclear leucocytes. Cystoscopic examination revealed no bladder pathology. Indigocarmine appeared in good concentration from the left ureteral orifice in 5 minutes and from the right in 9. The ureters were readily catheterized and specimens of urine obtained from the renal pelves were normal. Kidney, ureter and bladder roentgenogram presented a normal left kidney shadow, but the right renal shadow was not visualized. Bilateral pyelograms (fig. 1) disclosed a normal left kidney outline with a well filled, normal appearing pelvis. On the right there was a mass, apparently the right kidney shadow, with the upper pole at the level of the fourth lumbar vertebra. The calyces and pelvis were directly over the sacroiliac joint. The ureter appeared shortened and kinked. When the patient assumed the erect position, the right kidney moved less than two centimeters. The diagnosis of right ectopic kidney was definitely established. Surgical removal of the right kidney was recommended and accepted. Operation (October 27, 1941, recorded in motion pictures in color): Under spinal anaesthesia, an incision was made parallel to and 2 inches to the right of the abdominal midline, commencing just above the pubis and extending a few inches above the umbilicus. After the peritoneum had been opened, the intraabdominal contents were readily displaced to expose the right ectopic kidney. The posterior peritoneum was incised longitudinally, bringing the anomalous
RENAL ECTOPIA
127
kidney into the field. The renal pelvis faced anteriorly, indicating failure of rotation. Blood vessels, branches of the internal iliac artery, entered the kidney. These were clamped and ligated separately. After division of the short ureter the kidneywas removed, giving excellent visualization of the iliac vessels. The parietal peritoneum was closed, followed by the closure of the other abdominal structures without drainage. Pathologic Report: "Specimen consists of a flat, somewhat lobulated kidney measuring 8½ by 6 by 3 cm. On the posterior surface is a groove apparently to
Frn. 1. Case l. Bilateral pyelogram showing normal left kidney and ureter. Pelvic position of right kidney shows bizarre outline of pelvis and calyces, demonstrating failure of ascent and rotation of organ.
accommodate the iliac vessels. There are three or four separate arteries making up the blood supply. Anteriorly there are three extrarenal calyces uniting to form the renal pelvis, which gives origin to the ureter." At no time did the temperature exceed 100°F. or the pulse 100. The abdominal wound healed by primary union. The patient was discharged from the hospital on the eleventh postoperative day, ambulatory and apparently in good health. Patient was interviewed 6 months following discharge and stated that she had been free from abdominal pain. Case 2. A white female, aged 29 years, was admitted to the hospital on Jan-
128
CARL F, RUSCHE AND JAMES L, BRAY
uary 7, 1942. She complained of right lower abdominal pain, nausea, vomiting and abdominal distention for three weeks. There was some associated frequency, and nocturia 2 to 3 times. She said that her bowels moved daily. Because of attacks of pain in the lower right abdomen, associated with nausea, vomiting and distention, the patient was operated upon for appendicitis in 1936. Some bilateral pelvic disease existed and bilateral salpingectomy was carried out at the same time. Due to the fact that symptoms continued following the above surgery, a subtotal hysterectomy, oophorectomy and separation of ad-
FIG. 2. Case 2. Bilateral pyelogram showing normal left kidney with right ectopic kidney occupying medial position directly in front of sacrum.
hesions was performed. She had been admitted to the hospital on at least. 3 previous occasions with similar symptoms, and discharged after medical treatment with the diagnosis of partial intestinal obstruction due to abdominal adhesions. Her last attack requiring hospitalization was 3 months previously. She was under observation for 10 days. Examination revealed a poorly nourished female, who was co-operative and in no acute distress. Temperature 99.3°; pulse 95. The abdomen was moderately distended and no masses were palpable. Pelvic examination was not satisfactory, but bimanual examination with the finger in the rectum disclosed a tender, palpable mass in the pelvis.
RENAL EC'IOPIA
129
The urine was grossly cloudy and contained a few red blood cells, pus cells and gram-positive cocci in clumps. Blood non-protein nitrogen 33 mg per 100 cc. Blood vVassermann and Kahn -were reported negative. Blood count revealed a secondary anemia. Cystoscopic examination presented mild evidence of cystitis. Ureteral orifices were normal in position, and indigo carmine appeared in good concentration from the left in 5 and from the right in 6 minutes. The ureters were readily catheterized, and specimens obtained from the renal pelves revealed a right renal infection. The plain x-ray and pyelograms (fig. 2) disclosed no evidence of calculi. There was present a normal left kidney and ureter, but no right renal shadow. The right ureteral catheter was coiled opposite the sacrum and an anomalous renal pelvis outline. Right nephrectomy ,vas recommended and accepted. Operation (January 27, 1942, recorded in motion pictures in color): Under spinal anesthesia, the same surgical procedure was carried out as in the previous patient. Pathologic Report: "Specimen consists of a kidney measuring 11 by 7 by 3.5 cm and weighing 120 gm. Structure does not have normal renal architecture and retains some fetal lobulation. Pedicle has a broad attachment and apparently adherent to its bed of an area 8 by 5 cm. Ureter bifurcates approximately 2 cm prior to entering the parenchyma. There is one rather large aberrant artery entering the periphery. Capsule strips with relative ease, leaving a smooth brown homogenous surface." Convalescence was uneventful, and the patient was discharged from the hospital February 8, 1942. The patient was interviewed on :VIay 21, 1942 and stated that she had had no further symptoms. · SUMMARY
Two cases of unilateral ectopic kidney are reported, their clinical history given and surgical treatment (transperitoneal nephrectomy) recorded through motion pictures in color. Symptoms are those of pelvic or intra-abdominal conditions rather than renal. Roentgenographic procedures, especially bilateral pyelography, offer a conclusive diagnosis. The procedure of nephrectomy is radical treatment but mandatory in obtaining a cure. The transperitoneal route is the approach of choice in dealing with this anomaly. 906 Taft Bldg., Los Angeles, Calif. 1137 Roosevelt Bldg., Los Angeles, Calif. REFERENCES (1) ANSON, B. J., PICK, J. W. AND CAULDWELL, E. W.:Theanatomyofcommonerrenal
anomalies: Ectopic and horseshoe kidneys. J. Urol., 47: 112-130, 1942. (2) BEER, E. AND FERBER, W. L. F.: Crossed renalectopia (unilateral fused or elongated kidney). J. Urol., 38: 541-561, 1937.
130
CARL F. RUSCHE AND JAMES L. BRAY
(3) CAMPBELL, M. F.: Renal ectopy. J. Urol., 24: 187-198, 1930. (4) ErsENDRATH, D. J. AND ROLNICK, H. C.: Urology. Philadelphia: J.B. Lippincott Co., 1938, ed. 2, p. 577. (5) FowLER, H. A.: Bilateral renal ectopia. J. Urol., 45: 795-812, 1941. (6) HERMAN, L.: Practice of urology. Philadelphia: W. B. Saunders Co., 1938, p. 170. (7) LowSLEY, 0. S. AND Kmwrn, T. J.: Clinical Urology. Baltimore: The Williams & Wilkins Co., 1940. Vol. 11, p. 1300. (8) MACKENZIE, D. W. AND HAWTHORNE, A. B.: The ectopic kidney. J. Urol., 39: 479-486, 1938. (9) OGDEN, H. D. AND MALTRY, E.: Pelvic single kidney; Case report. J. Urol., 44: 13-18, 1940. (10) THOMAS, G. J. AND BARTON, J.C.: Ectopic pelvic kidney. J. A.M.A.,106:197-201, 1936. (11) THOMPSON, G. J. AND PACE, J.M.: Ectopic kidney, a review of 97 cases. Surg., Gynec. and Obst., 64: 935-943, 1937.