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Renal Ectopia—Special Reference to Crossed Ectopia without Fusion1
RENAL ECTOPIA-SPECIAL REFERENCE TO CROSSED ECTOPIA WITHOUT FUSION 1 AUGUSTUS HARRIS
From the Department of Urology, St. John's Hospital, Brooklyn, New York
Renal ectopia must be considered among the significant anomalies of the kidney. Its potential clinical importance to urologist, diagnostician, general surgeon and practitioner, and gynecologist should be stressed. As a result of perfection of intravenous and retrograde urographic diagnosis and the ever-increasing number of cystoscopic stu'dies, with the natural growth of the specialty, greater numbers of these lesions are being demonstrated and correctly interpreted. While a number of the cases recorded in the older literature leave doubt as to the accuracy of diagnosis, it is likely that from now on few, if any, errors will occur where thorough examination is made. Ectopia is derived from two Greek words meaning "out of place." The lay dictionary and older medical dictionaries state that it is a displacement of the part, either congenital or traumatic. The newer medical dictionaries imply that the malposition is only congenital, which we know to be a fact, and is practically always associated with a short ureter. On this basis, final differential diagnosis should not be confused. An extremely rare exception to this is the autopsy case reported by Campbell with long ureter and kidney above the diaphragm. The word dystopia, sometimes used as a synonym, would seem to be somewhat confusing and could profitably be altogether discarded. Frederick Carey, a Greek and Latin scholar (quoted by Mayers) has stated "The prefix dys always increases the bad sense of a word or destroys the good sense in Classical Greek; it connotes difficulty, pain, misfortune, and even evil. The prefix ec (really ek) simply means 'away from, out of, off'. Topia is topos, place, region; on the whole, then, ectopia would be the simpler and more natural word, but the solution must depend on current and accepted usage among physicians". The early masters of anatomy of the sixteenth and seventeenth centuries described and studied ectopia on the cadaver. A review of the literature from scattered sources, early and late, is instructive, enter1 Read before annual meeting, American Urological Association, White Sulphur Springs, W. Va., June 1, 1939. 1051
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taining and romantic. Ectopia may be unilateral, bilateral, single crossed with fusion and single crossed without fusion (figs. 1 to 4). The ureters may be double crossed with kidneys in normal position (fig. 6). Ectopia also occurs with absence of the opposite kidney, and as a single fused FIG.1
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FIG.5 FIG.6 FIG. 1. Simple unilateral ectopia FIG. 2. Simple bilateral ectopia FIG. 3. Crossed ectopia with fusion FIG. 4. Crossed ectopia without fusion FIG. 5. Pelvic ectopic solitary kidney FIG. 6. Double crossed ureters-normal kidneys (Beer and Ferber)
kidney (fig. 5) not infrequently prevertebral in position. Fetal lobulations are common, as well as wide variations in size and shape-some represent transition forms. The fused types of ectopia have been well described recently by Wilmer in 7 forms. Stevens has estimated that about 4 to 5 per cent of single kidneys are "pelvic" in position. He col-
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lected 25 cases from the literature and reported 2 of his own. With this anomaly it is obvious that nephrectomy, without full knowledge of the actual condition preoperatively, will result in the removal of all renal substance. It is unfortunate that the term unilateral fused kidney has found its place in the nomenclature, as it is really a misnomer. Crossed ectopia with fusion is the proper term. The other exception would be unilateral double kidney with double ureters descending to the normal position in the bladder on the same side. Gerard (1905) gave the term "concrescent" to designate all fused kidneys other than horseshoe. This word could well be discarded. The word "hetero-lateral," used to express crossed ectopia, could also profitably be left out of the terminology. The total number of simple unilateral ectopia cases reported would be somewhat less than 300; of simple bilateral ectopia less than 30; of crossed ectopia with fusion (isthmus or band) almost 300, and of crossed ectopia without fusion about 25. It has been estimated that only about 12 to 15 per cent of crossed ectopia occur without fusion. In any event, ectopia is a relatively rare lesion. The statistics of incidence, however, vary widely with different authors in reporting numbers of cases found, on the basis of the number of autopsies done and the number of urologic examinations made. According to Wilmer's thorough study of crossed ectopia with fusion it may be estimated that nearly half the cases were found at autopsy and nearly 15 per cent at operation. There is no doubt that many clinical and autopsy cases have not been recorded. Moreover, absence of symptoms in certain individuals will add to the number not discovered during life. Ectopia, like other anomalies of the kidney and ureter, apparently renders the patient more susceptible to urinary stasis, infection, calculus and clinical syndromes incident to obstruction and pressure, often produced by the anomalous blood supply and other factors. A number of these anomalies, however, have failed to cause difficulty during life. Another infrequent complication of ectopia is dystocia. The literature indicates that this is much less frequent than is commonly supposed. The scope of this presentation does not permit a description of the many and varied types of kidneys previously encountered, and types of position and blood supply. The organ may be so firmly fixed that any degree of mobilization of the kidney at the operating table is mechanically impossible, because of the increased number and unusual distribution of
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blood vessels. We are chiefly concerned with the clinical aspects of dealing with this lesion where surgery is necessitated. Not a few of the lesions produce no symptoms; a fair number have been discovered by accident at the operating table during other procedures; a majority have been discovered at autopsy. Many have been previously operated upon under a great variety of mistaken diagnoses. Single and fused kidneys have been erroneously removed with resultant uremia and death. The embryology has been so frequently and fully described that it is superfluous for us to review it at this time. Anatomists and pathologists are unable to explain the cause of the developmental defects. E. B. Frazer (1935) in quoting the experienced opinion of Dr. Carey, a state veterinarian, stated that the latter had never seen ectopia in animals. This is of interest from the standpoint of comparative anatomy and suggests the possibility of the relationship of the erect posture in man to the development of this anomaly. In crossed ectopia (except single ectopic kidney), the ureteral orifices are normal in position but the path of one of them leads across the midline of the body to the ectopic organ, which lies at some level on the opposite side of the body. The organ may be lateral or prevertebral in position and may lie at any level from above the crest of the ilium, down to the hollow of the sacrum. The lower pole of an ectopic kidney has been found in a scrotal hernia in two autopsy cases (Campbell). The prevertebral type may have a slightly crossed ureter or lie midway between the crossed and the uncrossed. The crossed kidney may be fused with its fellow organ or it may lie well separated. Hypoplasia may also occur and more commonly affects the crossed than the uncrossed kidney. In the more complete forms of crossed ectopia (with or without fusion) the pelvis faces the spine in relatively the same manner as that of its normal mate. However, the pelvis of the lower kidney has been known to be the complete reverse to this, namely with the calices in precisely the same direction as if the organ had reached its normal place on the opposite side of the body. In crossed ectopia with fusion the crossed ureter usually leads to the lower kidney. Reports of rare cases (Kidd, Dickinson and others) reveal that the crossed ureter may lead to the upper kidney. In our first case the organ was widely separated from the other kidney and in a fixed pelvic, prevertebral, position~a rare abnormality. In the course of necropsies at the Mayo Clinic from 1905 to 1938 this anomaly was never
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encountered. Mayers, in 1936, presented a survey of 20 cases which were predominantly left-sided. Most of them were found at autopsy. Ten were reported before 1909, and about 50 per cent of the total series showed no pathological condition. In Mayers' own case the ectopic -r····'"t
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FIG. 7 FIG.8 FIG.9 FIG. 7. Unilateral ectopia with ectopic ureter communicating with urethra (Sparks). FIG. 8. Crossed ectopia with fusion with hypoplastic kidney on opposite side (Beer and Ferber). FIG. 9. Crossed ureter with normally placed kidneys, single ureter orifice bifurcating above bladder. Only case of its type reported (Braasch). (Recorded by Beer and Ferber)
FIG. 10. Composite sketch suggesting many and varied types and positions of ectopia
kidney was not diseased, while the normally-developed kidney on the same side required pyelotomy for removal of calculi. In only 3 of 17 crossed ectopia without fusion, collected by Alcock in 1926, was a complete diagnosis made.
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Diagnosis. It would appear that the wider one's experience in urology the more one would be likely to suspect anomalous lesions, where unusual location and type of pain is manifest. If a mass also is encountered in an exceptional location this may add to the suspicion of such a condition. About 6 months ago a patient consulted us for a dull, pressure-type of pain in the lower abdomen and genital regions. The pain had existed for 7 years and was gradually growing more severe. The "dull, aching pain" was aggravated by the sitting posture and usually disappeared entirely on standing. There were no urinary symptoms. Because of the type of pain and negative findings on physical examination, we suspected an anomaly, as there was an associated mild pyuria. Urography showed a horseshoe kidney. The patient's clinical history pointed the way to precise diagnosis. He had been treated for a period of over a year by prostatic massage, by a trained urologist, without relief. He has been greatly relieved by ureteral catheterization. If the ectopic kidney lies in the region of the brim of the bony pelvis or below it, the organ may be palpated by vaginal or rectal examination as was done in our second case. If such a mass is felt, especially when not related to the reproductive tract and more readily palpable by rectum, one should suspect the lesion. Goldberger and Beebee each (independently) made a presumptive diagnosis by this method which was later confirmed. Where urography reveals both pelves on one side of the body with one crossed ureter, an effort should be made to determine, if possible, whether fusion exists or not. It occurred to the writer that fluoroscopy in conjunction with pyelography might be of practical value in estimating the relative mobility or fixation of the kidney. Accordingly, we used this method in our second case. We, then, thought this was an original idea, only to find that it had been previously suggested by Beer and others in attempting to determine presence or absence of fusion. Overlapping shadows may be suggestive of separation. Again the ability to change the relative positions of the two injected pelves by manual pressure under fluoroscopy may be helpful in diagnosis. There should be no difficulty in confusing the urographic diagnosis where marked renal ptosis, retroperitoneal masses or large renal cysts are encountered. Intravenous urography should always be done in all suspected anomaly cases. Unfortunately, poor shadows or absent shadows of the pelvis commonly occur in ectopia due to impaired function. Absence of a pelvic shadow should be considered suspicious of the possibility of ectopia. One recorded case with absence of a pelvic
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shadow, with ectopic kidney and ectopic ureter to the urethra, was subjected to operation by a surgeon who could not find the organ at the operating table through the usual high loin incision. A second operation was required for removal of a "pelvic" kidney after catheterization of the ectopic ureter. Shadows cast by calculi in an ectopic kidney appearing in an abnormal position on the plain film may prove to be a clue to such a possibility. The coexistence of various other anomalies, especially of the generative organs (cloacal origin), with renal ectopia is a fairly frequent condition. Complete investigation of the urinary organs in such cases would seem to be always indicated. We have noted that many of these anomalies of ectopia, of double kidney, as well as of the varied forms of non-calculous ureteropelvic obstruction, have been found in persons in the third and fourth decades of life. Although the lesions were congenital, troublesome symptoms did not appear before adult life. Surgical management. Calculi have been successfully removed from the pelvis or ureter by the abdominal extraperitoneal route. Double pyelotomy at one operation has been successfully done by Beer in fused kidney; he has also performed ureterolysis for adhesions. Experience of the past bears out the fact that both pyelotomy and nephrectomy may be reasonably easy or extremely difficulty. Conservative plastic procedures for pelvic obstruction would seem to have a very limited place. Persistence of pain associated with renal infection would logically call for nephrectomy as the method of choice, provided the other kidney is normal. Nephrectomy has not infrequently been done for relief of pain even in the absence of renal infection or calculi. The prime consideration in performing nephrectomy is obviously the management of the blood supply. Opinion is definitely divided as to method of approach. Some advise the extraperitoneal abdominal route, while others prefer the transperitoneal route. Some have found it necessary to convert a retroperitoneal operation into a transperitoneal at the operating table. If the kidney is attached and fixed with multiple vessels and lying over the spine, transperitoneal approach seems to the writer to be more advantageous. This was done in 2 of our cases. A crossed ectopic kidney without fusion, very similar to our first case, was removed by Beer by the extraperitoneal route. Again, it is possible that fluoroscopy during pyelography may aid in estimating the degree of renal mobility before surgery is attempted. However, manipulations by rectum of the
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injected "pelvic" kidney under the fluoroscope in one of our cases, led us to underestimate the degree of vertical mobility of the organ. High Trendelenburg position at the operating table proved this error. For this reason we recommend that fluoroscopic examination include more extreme degrees of elevation and lowering of the head. Past reports of operations indicate that certain ectopic kidneys have a fair amount of mobility with limited number and distribution of blood vessels, while others are seemingly almost anchored by a network. Gilbert Thomas and David W. MacKenzie have each successfully performed 6 nephrectomies for ectopia by the extraperitoneal route. It is well to remember that the pelvis is more frequently anterior in position, owing to failure of rotation; it may, however, be located at any angle of the arc from complete to incomplete rotation and it may lie inferiorly. At operation, each vessel requires identification, isolation and ligation as the organ is being slowly and cautiously mobilized step by step. Rupture of a vessel by traction may, otherwise, result in severe hemorrhage, abandonment of operation, or even death. Some have found the vessels easy to handle and feel that there should be no difficulty, while other competent and experienced operators have had trouble with bleeding and proper exposure during the course of operation. Case 1. Crossed ectopia without fusion; Zumbo-sacral "pelvic" kidney with calculus; transperitoneal nephrectomy. A. E., a male, 41 years of age, a machinist, consulted me July 15, 1938 for dull, aching pain in the left sacral and sacroiliac regions, in the left groin, perineal and rectal regions, and extending downward and forward into the left thigh and leg. The pain occurred intermittently every 3 or 4 weeks, lasted about 2 or 3 days, and often was relieved by aspirin. The pain began about 1 year ago and gradually increased in frequency and severity. Occasionally there was. nausea but no vomiting. There were no urinary symptoms. He was cystoscoped at the New York Hospital 4 months ago and was told he had a misplaced and malformed left kidney for which operation was advised. The pain was completely relieved for a period of about 2 months after this cystoscopic examination. After consulting me, he delayed further hospitalization and study for 6 weeks. The pain was so sharp and severe he sought relief and was admitted to the hospital. Physical examination showed a thick muscular abdominal wall with no masses palpable beneath. There was slight tenderness in the suprapubic region. The urine contained abundant pus cells, some red cells and a trace of albumin. The blood count was 15,600 white cells with 79 per cent polymorphonuclears. The hemoglobin, red cell count, and blood chemistry
FIG.11 FIG.12 FIG. 11. Case 1. Small calculus in lower calyx of normally-developed right kidney. Suggestion of shadow of lower pole of crossed left kidney with shadow of calculus in renal pelvis to the left of the right sacroiliac joint. FIG. 12. Case 1. Intravenous urogram-Nine-minute interval shows outline of part of ectopic kidney. Outline of pelvis of kidney fails to show. Normal pyelogram of right kidney.
FIG.14 FIG. 13 FIG. 13. Case 1. Plain film with opaque catheter placed in pelvis of crossed ectopic kidney. FIG. 14. Case 1. Pyelogram reveals peculiar position and formation of kidney. Large calcified gland adjacent to renal pelvis. 1059
FIG. 15. Case 1. Lateral pyelogram shows ectopic kidney with malformed pelvis and dilated calices. Calcified lymph gland seen just anterior to kidney.
FIG. 16. Case 1. Half-tone showing anterior aspect of kidney removed (actual size)
FIG. 17. Case 1. Sketch of posterior surface of kidney showing 3 vessels entering directly in to renal cortex. 1060
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tests were normal. On cystoscopic examination under spinal anesthesia, the right ureter was purposely not catheterized as intravenous urograms had revealed a normal right kidney and renal pelvis; but with a small calculus in a lower calyx. The pelvis of the ectopic (left) kidney failed to show in any of the urograms. The right kidney function, urine and pyelogram were reported as normal 5 months ago at the New York Hospital. A No. 6 F. catheter, introduced into the left ureter, passed for a distance of only 12 cm. where it was completely arrested. Phenolsulphonphthalein returned from the left kidney
FIG. 18. Case 1. Hemisection of kidney showing apparent anomalous development of renal substance with calculus at uretero-pelvic outlet with resultant dilatation of pelvis and calices.
in moderate traces 6 minutes after intravenous injection and concentrated only fairly well after a 10-minute interval. The urinary drip was hydronephrotic with pelvic capacity of about 16 cc. The left renal urine contained large numbers of pus cells. A plain film taken with opaque catheter in situ showed the catheter to cross to the right side of the midline with tip of catheter angulated and with a small shadow in all of the films, suggestive of a calculus in the pelvis. The pyelogram showed kidney lying obliquely across the fifth lumbar vertebra and sacrum with pelvis facing somewhat downward and to the right, with a bizarre-shaped pelvis with dilated calices. The left ureter was very
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short, apparently about 10 cm. in length. In some of the films the lower pole of the left kidney could be distinctly seen well below the brim of the bony pelvis and to the left of the midline. At operation a vertical left rectus incision was made 11 cm. in length extending from a point just below the level of the umbilicus to the pubic bone. The peritoneum was fully incised and coils of intestines packed well out of the way with warm lap sponges, using Trendelenburg position. The kidney was readily palpated, lying across the lumbo-sacral spine and was somewhat larger than its normal mate. The posterior peritoneum was then freely incised and the kidney slowly and carefully dissected free, mostly by blunt dissection, partly by Mayo scissors and strip-gauze. Seven fairly good-sized aberrant vessels were encountered and found to enter directly into the kidney substance in the upper, middle and lower poles, giving the kidney a "fixed" position. After considerable dissection we were fortunately able to skeletalize these vessels, one by one, without rupture and to clamp and ligate each of them. These vessels arose from the aorta, common and external and internal iliac trunks. The left ureter was also isolated, clamped in two places well below the brim of the bony pelvis, amputated and stumps cauterized with pure phenol. The kidney was then removed and retroperitoneal space left practically bloodless. A large cigarette drain was left in this dead space and the posterior peritoneum closed with a running double-looped plain catgut suture. No packing was necessary. The abdominal wall was closed in layers about the drain and patient returned to bed in very satisfactory condition. The operative procedure required about 1½ hours for completion. Convalescence was uneventful. The wound remained clean and the last portion of the cigarette drain was removed 6 days after operation. The patient left the hospital, with the wound healed by primary union, on the fourteenth postoperative day and has since enjoyed perfect health. The kidney specimen revealed numerous fetal lobulations, some deep sulci with fairly marked hydronephrosis both intra- and extrarenal. An ovalshaped stone about the size of a pistachio-nut was found in the pelvis. Only one vessel entered the kidney at the hilus. Four other fairly good-sized vessels were found to enter the kidney cortex directly with a small area of indentation on the surface at the site of entrance. On section of the kidney there was chronic pyelonephritis with many tubular changes especially in the convoluted tubules with swollen cells with indistinct margins and poor staining-power. Some hyalinization of glomeruli was also noted.
Case 2. Ectopic pelvic presacral kidney; transperitoneal nephrectomy. Miss L. S., 18 years of age, was seen January 17, 1939 suffering from intermittent attacks of nausea and vomiting of about 6 months' duration. In August 1938 she had a severe attack with fever of 103 degrees and was bedridden for 1 week. The onset began with prostration, fainting and cold sweats.
Frc.. 19 FrG. 20 Frc.. 19. Case. 2. Intravenous urogram-half-hour interval. Presacral ectopic right kidney, pelvis and part of ureter seen. Xonnal left renal pelvis outlined. First interval film failed to show pelvis of ectopic kidney. FrG. 20. Case 2. Bilateral retrograde pyelogram shows ectopic right kidney with ureteropelvic outlet just to left of mid line. Long spindle-like calices in part of kidney.
FIG. 21. Case 2. Second pyelogram of ectopic right kidney 1063
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A second attack occurred 10 days ago with pain in the lower right abdomen and low backache. Physical examination showed deep pressure-tenderness over and below McBurney's point. A presumptive diagnosis of acute appendicitis with abscess was made on the basis of finding leucocytosis, also a palpable mass low on the right side.
FIG. 22. Case 2. Half-tone of kidney in position as found in the patient. distribution of blood vessels and location of pelvis (actual size).
Note sulci,
Rectal examination also revealed a palpable mass on the right side. The urine contained a small number of leucocytes and was otherwise negative. Examination was otherwise essentially negative. The surgeon in attendance advised temporizing and that no operation be done. She was admitted to the hospital 1 month later and appendectomy was performed the following day. The mass proved to be an ectopic kidney
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which was not disturbed. A normal appendix was removed and recovery from this operation was uneventful. Urological examinations 2 weeks later showed the left kidney to be entirely normal; the right kidney ectopic with ureter about 8 cm. long, with kidney lying in the hollow of the sacrum, and ureteropelvic junction slightly to the left of the midline, pelvis facing to the left and slightly downward. The appearance time of phenolsulphonphthalein from the right (ectopic) kidney was delayed 7 minutes longer than the left, concentrated slowly, and the urine from this kidney contained 30 to 40 pus cells per field. Intravenous diodrast did not appear in the right kidney on the first interval film, but showed fairly well on the thirty-minute film, indicating delayed concentration. The kidney was readily palpable by rectal examination. The patient was removed to the fluoroscopic room where pyeloscopy and manipulation of the kidney, by the rectal finger, seemed to indicate rather limited vertical mobility and slightly greater lateral mobility of the kidney. The patient, however, was not placed in high Trendelenburg position during this examination. Failure to do this led us to draw false conclusions regarding the actual mobility, as subsequently proven at the operating table. In view of the symptoms and above findings, nephrectomy was advised. A right rectus abdominal incision was employed, opening the peritoneum and packing the coils of bowel out of the field with lap sponges and with aid of Trendelenburg position. On opening the posterior peritoneum, the renal pelvis and upper ureter were found lying in direct anterior position. With patient in high Trendelenburg position, the kidney had moved to a higher level than was seen on pyelography and fluoroscopy, indicating a greater degree of mobility than we had anticipated. The kidney was somewhat smaller than normal and had a notch at the upper pole through which a large vein passed downward over the anterior surface to enter the hilus. Three other fair-sized arteries accompanied by veins, all from the iliacs, entered the kidney anteriorly near the hilus. One of them encircled the right half of the kidney obliquely in a deep groove in the cortex. The kidney was rather mobile during manipulations, so that isolation and ligation of vessels including the ureter was relatively easy. The kidney was then removed, posterior peritoneum completely closed, anterior peritoneum closed and abdominal wound sutured without drainage. The entire procedure required about 45 minutes. Convalescence was uneventful and the patient left the hospital, in good condition, on the sixteenth postoperative day. She has remained well smce that time. Section of a small portion of the kidney substance indicated good preservation of the renal elements. Case 3. Crossed ectopia without fusion asymptomatic; no operation required. A corpulent steel-worker 65 years of age was admitted to the service of
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Dr. F. L. Senger in January 1939. In 1933 transurethral prostatic resection had been performed for relief of urinary obstruction. Two years later recurrence of obstructive symptoms necessitated a 2-stage prostatectomy. There had never been symptoms referable to the upper urinary tract. In January 1939, the patient complained of localized pain in the left costo-vertebral area, which did not radiate. There were no urinary symptoms. Physical examination was essentially negative. Intravenous urography showed the dye in the normally developed kidney and none elsewhere. (The ectopic kidney failed to concentrate the medium.) Retrograde pyelography demonstrated the left
FIG. 23 FIG. 24 Case 3. Bilateral retrograde pyelograms revealed typical crossed ectopia of left kidney without fusion, with pelvis overlying sacrum. FrG. 24. Case 3. Second retrograde pyelogram showing clearly the pelvis and shadow of the crossed kidney. FIG. 23.
ureter to cross over to the right side to the ectopic kidney, located overlying the right sacroiliac joint. There was no ureterectasis, pyelectasis or indication of calculus. The patient was treated for myositis of the muscles of the back on the left side with complete relief of pain. SUMMARY
We have endeavored to clarify the definition of ectopia and its various forms, and to delete confusing terms and synonyms given in the literature. The incidence of the different types, as well as their description, is given. The diagnostic features and surgical management are discussed.
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A review of three recent personal cases is included, two with transperitoneal nephrectomy and one with diagnostic fluoroscopy. Urologists are becoming more "anomaly-minded". Only by complete urological examination will earlier and more accurate diagnosis be made. and many errors avoided. 306 Park Place, Brooklyn, LY.
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REFERENCES ALCOCK, N. G.: .\ case of calculus pyonephrosis in a crossed ectopic non-fused kidney. Tr . .\m. A. Genito-Urin. Surgeons, 19: 299-310, 1926. BARRY, T. R., AND GARVEY, F. K.: Crossed renal ectopia. J. A. M ..\. 94: 1232-1233, 1930. BEEBEE, JYI. L.: Case reports; crossed ectopic kidney; accidental concealed hemorrhage. Colorado Med. 24: 255-256, 1927. BEER, EDWIN: Infected left calculous hydronephrosis in right iliac fossa. Am. Surg. 183: 433, 1926. BEER, E., AND FERBER, W. L. F.: Crossed renal ectopia. J. Ural. 38: 541-561, 1937. BEER, E., AND FERBE;i, W. L. F.: Crossed renal ectopia. J. Ural. 39: 479-486, 1938. BEGG, R. CAMPBELL: Heterolateral ectopia of right kidney. Brit. M. J. 1: 1049, 1938. BRAASCH, V\'. F.: The clinical diagnosis of congenital anomaly in the kidney and ureter. Tr. Am. A. Genito-Urin. Surgeons 8: 281-292, 1913. C.rnPBELL, M. F.: Renal ectopy. J. Urol., 24: 187-198, 1930. CARLETON, .\.: Crossed ectopia of the kidnev ancl its possible cause. J. Anat. 7: 292-298, 1937. CLARK,,\. M.: Ectopic kidney. Glasgo\\· M. J. 128: 45-47, 1937. COLLIXGS, C. W.: Ectopic kidney. ,\m. J. Surg. 7: 425-426, 1929. CmtPTON, A. J.: Crossed dystopia of kidney. Lancet 1: 810, 1926. COPPRIDGE, \V. M.: Bilateral pelvic kidneys. J. Ural. 32: 231-235, Sept, 1934. DAVIDSON, BERNARD: Renal ectopia with abdominal and pelvic symptomatology. Urol. and Cutan. Rev., 42: 334-336, 1938. DICKINSON, W. LEE: Solitary kidney. Ibid, 46: 80, 1895. FRASER, E1n!FTT B.: Congenital renal dystopia. Urol and Cutan. Rev., 39: 381-385, 1935. GOLDBERGER, M ..\.: Ectopic kidney. J. l\lt. Sinai Hosp., 4: 24-27, 1937. GUTIERREZ, R.: Role of anomalies of kidney and ureter in causation of surgical conditions. J. .-\. M. A. 106: 183, 1936. HERBST, R.H.: Case of crossed dystopia of right kidney. S. Clin. North America, 7: 579-581, 1927. HFR1IA:--i, LEON: Crossed renal ectopia. Pennyslvania M. J. 37: 1009, 1934. Ho\Y,\RD, T. LEON: Crossed ectopia of right kidney without fusion and a large hydronephrosis of the normally placed left kidney. Urol. and Cutan. Rev. 39: 145-149, 1935. Hun11A:-1, L. F.: Congenital displacements of the kidney. J. Urol.12: 363, 1924. JONES, R. TREVOR: Calculus in an ectopic kidney. Brit. J. Surg. 22: 886-887, 1935. KIDD, FRANK: Urinary surgery. London, Longmans Green & Co., 31, 1910. KRETSCHMER, H. L.: Unilateral fused kidney. Surg., Gynec. and Obst. 40: 360-366, 1925. KREUTZMANN, H. ,\. R.: Renal dystopia. v\'est J. Surg., 43: 605-608, 1935. LOONEY, W. W., AND DODD, D. L.: An ectopic completely fused kidney associated with various anomalies of the abdominal viscera. .\nn. Surg. 84: 522-524, 1926. JYIAcKExzrn, D. vV., AND HAWTHORNE, ALLAN B.: The ectopic kidney: report of 13 cases exclusive of horse-shoe kidneys. J. Ural. 39: 479-486, 1938. MAYERS, 11. M.: Crossed renal ectopia. J. Urol., 36: 111-112, 1936. McCowN, l'. E.: Bilateral renal ectopia. J. Ural. 22: 653-665, 1929. MORGAN, E. K., AND STONE, C. M.: Bilateral renal ectopia. J. Urol., 38: 427-429, 1937. MORGAK. J. L.: Ectopic kidney: case report. Memphis M. J., 13: 30-32, 1938. PAPIN, E .. AKD ErsENDRATH, D. N.: Classification of renal and ureteral anomalies. Ann. Surg. 85: 735-756, 1927. PATCH, F. S.: Carcinoma in a crossed heterolateral ectopic kidney without fusion. Brit. J. Ural., 9: 339-359, 1937. l'LU11MER, S. C.: Case of misplaced kidney. Surg., Gynec. and Obst. 10: 431-437, 1910.
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POHLMAN, A. G.: Concerning the embryology of kidney anomalies. Amer. Medicine, 7: 987-990, 1904. SCHOFIELD, F. S.: Crossed renal dystopia. Pennsylvania M. J. 32: 771-773, 1929. SEXTON, W. G.: Renal ectopia. J. Urol. 33: 521-525, June, 1935. SHOEMAKER, R., AND BRAASCH, W. F.: Fused kidney-a description of gross pathologic changes in 35 cases. J. Urol., 41: 1, 1939. SrGAL, J. B.: Crossed renal dystropia without fusion. J. Urol. 32: 236-241, 1934. SPARKS, A. J.: Ureteral ectopia with congenital hypoplastic pelvic kidney. J. Urol. 33: 559-564, 1935. STEVENS, A. R.: Pelvic single kidneys. J. Urol. 37: 610-618, 1937. STEWART, M. J., AND LODGE, S. D.: Unilateral fused kidney and allied renal malformations. Brit. J. Surg. 11: 27, 1923-1924. SUTHERLAND, L. R., AND EDINGTON, G. H.: A series of specimens illustrative of certain congenital affections of the urinary apparatus. Glasgow, M. J. 49: 81-105, 1898. TooMAs, G. J., AND BARTON, J. C.: Ectopic pelvic kidney. J. A. M.A., 106: 197-199, 1936. THOMPSON, G. J., AND PACE, JOHN M.: Ectopic kidney: a review of 97 cases. Surg., Gynec. and Obst. 64: 935-943, 1937. TOLSON, H. L.: Ectopic pelvic kidney. Ann. Surg. 93: 880-885, 1931. TOLSON, H. L.: Ectopic pelvic kidney. Indian M. Gaz. 72: 613-614, 1937. WEAR, H. H.: Congenital renal ectopia with crossed dystopia. Colorado Med. 27: 194-197, 1930. WYNEKOOP, C. I., AND GERNON, J. T.: Crossed renal dystopia without fusion. Urol. and Cutan. Rev. 34: 307-308, 1935.
From the Department of Urology, St. John's Hospital, Brooklyn, New York
Renal ectopia must be considered among the significant anomalies of the kidney. Its potential clinical importance to urologist, diagnostician, general surgeon and practitioner, and gynecologist should be stressed. As a result of perfection of intravenous and retrograde urographic diagnosis and the ever-increasing number of cystoscopic stu'dies, with the natural growth of the specialty, greater numbers of these lesions are being demonstrated and correctly interpreted. While a number of the cases recorded in the older literature leave doubt as to the accuracy of diagnosis, it is likely that from now on few, if any, errors will occur where thorough examination is made. Ectopia is derived from two Greek words meaning "out of place." The lay dictionary and older medical dictionaries state that it is a displacement of the part, either congenital or traumatic. The newer medical dictionaries imply that the malposition is only congenital, which we know to be a fact, and is practically always associated with a short ureter. On this basis, final differential diagnosis should not be confused. An extremely rare exception to this is the autopsy case reported by Campbell with long ureter and kidney above the diaphragm. The word dystopia, sometimes used as a synonym, would seem to be somewhat confusing and could profitably be altogether discarded. Frederick Carey, a Greek and Latin scholar (quoted by Mayers) has stated "The prefix dys always increases the bad sense of a word or destroys the good sense in Classical Greek; it connotes difficulty, pain, misfortune, and even evil. The prefix ec (really ek) simply means 'away from, out of, off'. Topia is topos, place, region; on the whole, then, ectopia would be the simpler and more natural word, but the solution must depend on current and accepted usage among physicians". The early masters of anatomy of the sixteenth and seventeenth centuries described and studied ectopia on the cadaver. A review of the literature from scattered sources, early and late, is instructive, enter1 Read before annual meeting, American Urological Association, White Sulphur Springs, W. Va., June 1, 1939. 1051
1052
AUGUSTUS HARRIS
taining and romantic. Ectopia may be unilateral, bilateral, single crossed with fusion and single crossed without fusion (figs. 1 to 4). The ureters may be double crossed with kidneys in normal position (fig. 6). Ectopia also occurs with absence of the opposite kidney, and as a single fused FIG.1
FIG.2
FIG.
3
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FIG.4
FIG.5 FIG.6 FIG. 1. Simple unilateral ectopia FIG. 2. Simple bilateral ectopia FIG. 3. Crossed ectopia with fusion FIG. 4. Crossed ectopia without fusion FIG. 5. Pelvic ectopic solitary kidney FIG. 6. Double crossed ureters-normal kidneys (Beer and Ferber)
kidney (fig. 5) not infrequently prevertebral in position. Fetal lobulations are common, as well as wide variations in size and shape-some represent transition forms. The fused types of ectopia have been well described recently by Wilmer in 7 forms. Stevens has estimated that about 4 to 5 per cent of single kidneys are "pelvic" in position. He col-
RENAL ECTOPIA
1053
lected 25 cases from the literature and reported 2 of his own. With this anomaly it is obvious that nephrectomy, without full knowledge of the actual condition preoperatively, will result in the removal of all renal substance. It is unfortunate that the term unilateral fused kidney has found its place in the nomenclature, as it is really a misnomer. Crossed ectopia with fusion is the proper term. The other exception would be unilateral double kidney with double ureters descending to the normal position in the bladder on the same side. Gerard (1905) gave the term "concrescent" to designate all fused kidneys other than horseshoe. This word could well be discarded. The word "hetero-lateral," used to express crossed ectopia, could also profitably be left out of the terminology. The total number of simple unilateral ectopia cases reported would be somewhat less than 300; of simple bilateral ectopia less than 30; of crossed ectopia with fusion (isthmus or band) almost 300, and of crossed ectopia without fusion about 25. It has been estimated that only about 12 to 15 per cent of crossed ectopia occur without fusion. In any event, ectopia is a relatively rare lesion. The statistics of incidence, however, vary widely with different authors in reporting numbers of cases found, on the basis of the number of autopsies done and the number of urologic examinations made. According to Wilmer's thorough study of crossed ectopia with fusion it may be estimated that nearly half the cases were found at autopsy and nearly 15 per cent at operation. There is no doubt that many clinical and autopsy cases have not been recorded. Moreover, absence of symptoms in certain individuals will add to the number not discovered during life. Ectopia, like other anomalies of the kidney and ureter, apparently renders the patient more susceptible to urinary stasis, infection, calculus and clinical syndromes incident to obstruction and pressure, often produced by the anomalous blood supply and other factors. A number of these anomalies, however, have failed to cause difficulty during life. Another infrequent complication of ectopia is dystocia. The literature indicates that this is much less frequent than is commonly supposed. The scope of this presentation does not permit a description of the many and varied types of kidneys previously encountered, and types of position and blood supply. The organ may be so firmly fixed that any degree of mobilization of the kidney at the operating table is mechanically impossible, because of the increased number and unusual distribution of
1054
AUGUSTUS HARRIS
blood vessels. We are chiefly concerned with the clinical aspects of dealing with this lesion where surgery is necessitated. Not a few of the lesions produce no symptoms; a fair number have been discovered by accident at the operating table during other procedures; a majority have been discovered at autopsy. Many have been previously operated upon under a great variety of mistaken diagnoses. Single and fused kidneys have been erroneously removed with resultant uremia and death. The embryology has been so frequently and fully described that it is superfluous for us to review it at this time. Anatomists and pathologists are unable to explain the cause of the developmental defects. E. B. Frazer (1935) in quoting the experienced opinion of Dr. Carey, a state veterinarian, stated that the latter had never seen ectopia in animals. This is of interest from the standpoint of comparative anatomy and suggests the possibility of the relationship of the erect posture in man to the development of this anomaly. In crossed ectopia (except single ectopic kidney), the ureteral orifices are normal in position but the path of one of them leads across the midline of the body to the ectopic organ, which lies at some level on the opposite side of the body. The organ may be lateral or prevertebral in position and may lie at any level from above the crest of the ilium, down to the hollow of the sacrum. The lower pole of an ectopic kidney has been found in a scrotal hernia in two autopsy cases (Campbell). The prevertebral type may have a slightly crossed ureter or lie midway between the crossed and the uncrossed. The crossed kidney may be fused with its fellow organ or it may lie well separated. Hypoplasia may also occur and more commonly affects the crossed than the uncrossed kidney. In the more complete forms of crossed ectopia (with or without fusion) the pelvis faces the spine in relatively the same manner as that of its normal mate. However, the pelvis of the lower kidney has been known to be the complete reverse to this, namely with the calices in precisely the same direction as if the organ had reached its normal place on the opposite side of the body. In crossed ectopia with fusion the crossed ureter usually leads to the lower kidney. Reports of rare cases (Kidd, Dickinson and others) reveal that the crossed ureter may lead to the upper kidney. In our first case the organ was widely separated from the other kidney and in a fixed pelvic, prevertebral, position~a rare abnormality. In the course of necropsies at the Mayo Clinic from 1905 to 1938 this anomaly was never
1055
RENAL ECTOPIA
encountered. Mayers, in 1936, presented a survey of 20 cases which were predominantly left-sided. Most of them were found at autopsy. Ten were reported before 1909, and about 50 per cent of the total series showed no pathological condition. In Mayers' own case the ectopic -r····'"t
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FIG. 7 FIG.8 FIG.9 FIG. 7. Unilateral ectopia with ectopic ureter communicating with urethra (Sparks). FIG. 8. Crossed ectopia with fusion with hypoplastic kidney on opposite side (Beer and Ferber). FIG. 9. Crossed ureter with normally placed kidneys, single ureter orifice bifurcating above bladder. Only case of its type reported (Braasch). (Recorded by Beer and Ferber)
FIG. 10. Composite sketch suggesting many and varied types and positions of ectopia
kidney was not diseased, while the normally-developed kidney on the same side required pyelotomy for removal of calculi. In only 3 of 17 crossed ectopia without fusion, collected by Alcock in 1926, was a complete diagnosis made.
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AUGUSTUS HARRIS
Diagnosis. It would appear that the wider one's experience in urology the more one would be likely to suspect anomalous lesions, where unusual location and type of pain is manifest. If a mass also is encountered in an exceptional location this may add to the suspicion of such a condition. About 6 months ago a patient consulted us for a dull, pressure-type of pain in the lower abdomen and genital regions. The pain had existed for 7 years and was gradually growing more severe. The "dull, aching pain" was aggravated by the sitting posture and usually disappeared entirely on standing. There were no urinary symptoms. Because of the type of pain and negative findings on physical examination, we suspected an anomaly, as there was an associated mild pyuria. Urography showed a horseshoe kidney. The patient's clinical history pointed the way to precise diagnosis. He had been treated for a period of over a year by prostatic massage, by a trained urologist, without relief. He has been greatly relieved by ureteral catheterization. If the ectopic kidney lies in the region of the brim of the bony pelvis or below it, the organ may be palpated by vaginal or rectal examination as was done in our second case. If such a mass is felt, especially when not related to the reproductive tract and more readily palpable by rectum, one should suspect the lesion. Goldberger and Beebee each (independently) made a presumptive diagnosis by this method which was later confirmed. Where urography reveals both pelves on one side of the body with one crossed ureter, an effort should be made to determine, if possible, whether fusion exists or not. It occurred to the writer that fluoroscopy in conjunction with pyelography might be of practical value in estimating the relative mobility or fixation of the kidney. Accordingly, we used this method in our second case. We, then, thought this was an original idea, only to find that it had been previously suggested by Beer and others in attempting to determine presence or absence of fusion. Overlapping shadows may be suggestive of separation. Again the ability to change the relative positions of the two injected pelves by manual pressure under fluoroscopy may be helpful in diagnosis. There should be no difficulty in confusing the urographic diagnosis where marked renal ptosis, retroperitoneal masses or large renal cysts are encountered. Intravenous urography should always be done in all suspected anomaly cases. Unfortunately, poor shadows or absent shadows of the pelvis commonly occur in ectopia due to impaired function. Absence of a pelvic shadow should be considered suspicious of the possibility of ectopia. One recorded case with absence of a pelvic
RENAL ECTOPIA
1057
shadow, with ectopic kidney and ectopic ureter to the urethra, was subjected to operation by a surgeon who could not find the organ at the operating table through the usual high loin incision. A second operation was required for removal of a "pelvic" kidney after catheterization of the ectopic ureter. Shadows cast by calculi in an ectopic kidney appearing in an abnormal position on the plain film may prove to be a clue to such a possibility. The coexistence of various other anomalies, especially of the generative organs (cloacal origin), with renal ectopia is a fairly frequent condition. Complete investigation of the urinary organs in such cases would seem to be always indicated. We have noted that many of these anomalies of ectopia, of double kidney, as well as of the varied forms of non-calculous ureteropelvic obstruction, have been found in persons in the third and fourth decades of life. Although the lesions were congenital, troublesome symptoms did not appear before adult life. Surgical management. Calculi have been successfully removed from the pelvis or ureter by the abdominal extraperitoneal route. Double pyelotomy at one operation has been successfully done by Beer in fused kidney; he has also performed ureterolysis for adhesions. Experience of the past bears out the fact that both pyelotomy and nephrectomy may be reasonably easy or extremely difficulty. Conservative plastic procedures for pelvic obstruction would seem to have a very limited place. Persistence of pain associated with renal infection would logically call for nephrectomy as the method of choice, provided the other kidney is normal. Nephrectomy has not infrequently been done for relief of pain even in the absence of renal infection or calculi. The prime consideration in performing nephrectomy is obviously the management of the blood supply. Opinion is definitely divided as to method of approach. Some advise the extraperitoneal abdominal route, while others prefer the transperitoneal route. Some have found it necessary to convert a retroperitoneal operation into a transperitoneal at the operating table. If the kidney is attached and fixed with multiple vessels and lying over the spine, transperitoneal approach seems to the writer to be more advantageous. This was done in 2 of our cases. A crossed ectopic kidney without fusion, very similar to our first case, was removed by Beer by the extraperitoneal route. Again, it is possible that fluoroscopy during pyelography may aid in estimating the degree of renal mobility before surgery is attempted. However, manipulations by rectum of the
1058
AUGUSTUS HARRIS
injected "pelvic" kidney under the fluoroscope in one of our cases, led us to underestimate the degree of vertical mobility of the organ. High Trendelenburg position at the operating table proved this error. For this reason we recommend that fluoroscopic examination include more extreme degrees of elevation and lowering of the head. Past reports of operations indicate that certain ectopic kidneys have a fair amount of mobility with limited number and distribution of blood vessels, while others are seemingly almost anchored by a network. Gilbert Thomas and David W. MacKenzie have each successfully performed 6 nephrectomies for ectopia by the extraperitoneal route. It is well to remember that the pelvis is more frequently anterior in position, owing to failure of rotation; it may, however, be located at any angle of the arc from complete to incomplete rotation and it may lie inferiorly. At operation, each vessel requires identification, isolation and ligation as the organ is being slowly and cautiously mobilized step by step. Rupture of a vessel by traction may, otherwise, result in severe hemorrhage, abandonment of operation, or even death. Some have found the vessels easy to handle and feel that there should be no difficulty, while other competent and experienced operators have had trouble with bleeding and proper exposure during the course of operation. Case 1. Crossed ectopia without fusion; Zumbo-sacral "pelvic" kidney with calculus; transperitoneal nephrectomy. A. E., a male, 41 years of age, a machinist, consulted me July 15, 1938 for dull, aching pain in the left sacral and sacroiliac regions, in the left groin, perineal and rectal regions, and extending downward and forward into the left thigh and leg. The pain occurred intermittently every 3 or 4 weeks, lasted about 2 or 3 days, and often was relieved by aspirin. The pain began about 1 year ago and gradually increased in frequency and severity. Occasionally there was. nausea but no vomiting. There were no urinary symptoms. He was cystoscoped at the New York Hospital 4 months ago and was told he had a misplaced and malformed left kidney for which operation was advised. The pain was completely relieved for a period of about 2 months after this cystoscopic examination. After consulting me, he delayed further hospitalization and study for 6 weeks. The pain was so sharp and severe he sought relief and was admitted to the hospital. Physical examination showed a thick muscular abdominal wall with no masses palpable beneath. There was slight tenderness in the suprapubic region. The urine contained abundant pus cells, some red cells and a trace of albumin. The blood count was 15,600 white cells with 79 per cent polymorphonuclears. The hemoglobin, red cell count, and blood chemistry
FIG.11 FIG.12 FIG. 11. Case 1. Small calculus in lower calyx of normally-developed right kidney. Suggestion of shadow of lower pole of crossed left kidney with shadow of calculus in renal pelvis to the left of the right sacroiliac joint. FIG. 12. Case 1. Intravenous urogram-Nine-minute interval shows outline of part of ectopic kidney. Outline of pelvis of kidney fails to show. Normal pyelogram of right kidney.
FIG.14 FIG. 13 FIG. 13. Case 1. Plain film with opaque catheter placed in pelvis of crossed ectopic kidney. FIG. 14. Case 1. Pyelogram reveals peculiar position and formation of kidney. Large calcified gland adjacent to renal pelvis. 1059
FIG. 15. Case 1. Lateral pyelogram shows ectopic kidney with malformed pelvis and dilated calices. Calcified lymph gland seen just anterior to kidney.
FIG. 16. Case 1. Half-tone showing anterior aspect of kidney removed (actual size)
FIG. 17. Case 1. Sketch of posterior surface of kidney showing 3 vessels entering directly in to renal cortex. 1060
RENAL ECTOPIA
1061
tests were normal. On cystoscopic examination under spinal anesthesia, the right ureter was purposely not catheterized as intravenous urograms had revealed a normal right kidney and renal pelvis; but with a small calculus in a lower calyx. The pelvis of the ectopic (left) kidney failed to show in any of the urograms. The right kidney function, urine and pyelogram were reported as normal 5 months ago at the New York Hospital. A No. 6 F. catheter, introduced into the left ureter, passed for a distance of only 12 cm. where it was completely arrested. Phenolsulphonphthalein returned from the left kidney
FIG. 18. Case 1. Hemisection of kidney showing apparent anomalous development of renal substance with calculus at uretero-pelvic outlet with resultant dilatation of pelvis and calices.
in moderate traces 6 minutes after intravenous injection and concentrated only fairly well after a 10-minute interval. The urinary drip was hydronephrotic with pelvic capacity of about 16 cc. The left renal urine contained large numbers of pus cells. A plain film taken with opaque catheter in situ showed the catheter to cross to the right side of the midline with tip of catheter angulated and with a small shadow in all of the films, suggestive of a calculus in the pelvis. The pyelogram showed kidney lying obliquely across the fifth lumbar vertebra and sacrum with pelvis facing somewhat downward and to the right, with a bizarre-shaped pelvis with dilated calices. The left ureter was very
1062
AUGUSTUS HARRIS
short, apparently about 10 cm. in length. In some of the films the lower pole of the left kidney could be distinctly seen well below the brim of the bony pelvis and to the left of the midline. At operation a vertical left rectus incision was made 11 cm. in length extending from a point just below the level of the umbilicus to the pubic bone. The peritoneum was fully incised and coils of intestines packed well out of the way with warm lap sponges, using Trendelenburg position. The kidney was readily palpated, lying across the lumbo-sacral spine and was somewhat larger than its normal mate. The posterior peritoneum was then freely incised and the kidney slowly and carefully dissected free, mostly by blunt dissection, partly by Mayo scissors and strip-gauze. Seven fairly good-sized aberrant vessels were encountered and found to enter directly into the kidney substance in the upper, middle and lower poles, giving the kidney a "fixed" position. After considerable dissection we were fortunately able to skeletalize these vessels, one by one, without rupture and to clamp and ligate each of them. These vessels arose from the aorta, common and external and internal iliac trunks. The left ureter was also isolated, clamped in two places well below the brim of the bony pelvis, amputated and stumps cauterized with pure phenol. The kidney was then removed and retroperitoneal space left practically bloodless. A large cigarette drain was left in this dead space and the posterior peritoneum closed with a running double-looped plain catgut suture. No packing was necessary. The abdominal wall was closed in layers about the drain and patient returned to bed in very satisfactory condition. The operative procedure required about 1½ hours for completion. Convalescence was uneventful. The wound remained clean and the last portion of the cigarette drain was removed 6 days after operation. The patient left the hospital, with the wound healed by primary union, on the fourteenth postoperative day and has since enjoyed perfect health. The kidney specimen revealed numerous fetal lobulations, some deep sulci with fairly marked hydronephrosis both intra- and extrarenal. An ovalshaped stone about the size of a pistachio-nut was found in the pelvis. Only one vessel entered the kidney at the hilus. Four other fairly good-sized vessels were found to enter the kidney cortex directly with a small area of indentation on the surface at the site of entrance. On section of the kidney there was chronic pyelonephritis with many tubular changes especially in the convoluted tubules with swollen cells with indistinct margins and poor staining-power. Some hyalinization of glomeruli was also noted.
Case 2. Ectopic pelvic presacral kidney; transperitoneal nephrectomy. Miss L. S., 18 years of age, was seen January 17, 1939 suffering from intermittent attacks of nausea and vomiting of about 6 months' duration. In August 1938 she had a severe attack with fever of 103 degrees and was bedridden for 1 week. The onset began with prostration, fainting and cold sweats.
Frc.. 19 FrG. 20 Frc.. 19. Case. 2. Intravenous urogram-half-hour interval. Presacral ectopic right kidney, pelvis and part of ureter seen. Xonnal left renal pelvis outlined. First interval film failed to show pelvis of ectopic kidney. FrG. 20. Case 2. Bilateral retrograde pyelogram shows ectopic right kidney with ureteropelvic outlet just to left of mid line. Long spindle-like calices in part of kidney.
FIG. 21. Case 2. Second pyelogram of ectopic right kidney 1063
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AUGUSTUS HARRIS
A second attack occurred 10 days ago with pain in the lower right abdomen and low backache. Physical examination showed deep pressure-tenderness over and below McBurney's point. A presumptive diagnosis of acute appendicitis with abscess was made on the basis of finding leucocytosis, also a palpable mass low on the right side.
FIG. 22. Case 2. Half-tone of kidney in position as found in the patient. distribution of blood vessels and location of pelvis (actual size).
Note sulci,
Rectal examination also revealed a palpable mass on the right side. The urine contained a small number of leucocytes and was otherwise negative. Examination was otherwise essentially negative. The surgeon in attendance advised temporizing and that no operation be done. She was admitted to the hospital 1 month later and appendectomy was performed the following day. The mass proved to be an ectopic kidney
RENAL ECTOPIA
1065
which was not disturbed. A normal appendix was removed and recovery from this operation was uneventful. Urological examinations 2 weeks later showed the left kidney to be entirely normal; the right kidney ectopic with ureter about 8 cm. long, with kidney lying in the hollow of the sacrum, and ureteropelvic junction slightly to the left of the midline, pelvis facing to the left and slightly downward. The appearance time of phenolsulphonphthalein from the right (ectopic) kidney was delayed 7 minutes longer than the left, concentrated slowly, and the urine from this kidney contained 30 to 40 pus cells per field. Intravenous diodrast did not appear in the right kidney on the first interval film, but showed fairly well on the thirty-minute film, indicating delayed concentration. The kidney was readily palpable by rectal examination. The patient was removed to the fluoroscopic room where pyeloscopy and manipulation of the kidney, by the rectal finger, seemed to indicate rather limited vertical mobility and slightly greater lateral mobility of the kidney. The patient, however, was not placed in high Trendelenburg position during this examination. Failure to do this led us to draw false conclusions regarding the actual mobility, as subsequently proven at the operating table. In view of the symptoms and above findings, nephrectomy was advised. A right rectus abdominal incision was employed, opening the peritoneum and packing the coils of bowel out of the field with lap sponges and with aid of Trendelenburg position. On opening the posterior peritoneum, the renal pelvis and upper ureter were found lying in direct anterior position. With patient in high Trendelenburg position, the kidney had moved to a higher level than was seen on pyelography and fluoroscopy, indicating a greater degree of mobility than we had anticipated. The kidney was somewhat smaller than normal and had a notch at the upper pole through which a large vein passed downward over the anterior surface to enter the hilus. Three other fair-sized arteries accompanied by veins, all from the iliacs, entered the kidney anteriorly near the hilus. One of them encircled the right half of the kidney obliquely in a deep groove in the cortex. The kidney was rather mobile during manipulations, so that isolation and ligation of vessels including the ureter was relatively easy. The kidney was then removed, posterior peritoneum completely closed, anterior peritoneum closed and abdominal wound sutured without drainage. The entire procedure required about 45 minutes. Convalescence was uneventful and the patient left the hospital, in good condition, on the sixteenth postoperative day. She has remained well smce that time. Section of a small portion of the kidney substance indicated good preservation of the renal elements. Case 3. Crossed ectopia without fusion asymptomatic; no operation required. A corpulent steel-worker 65 years of age was admitted to the service of
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AUGUSTUS HARRIS
Dr. F. L. Senger in January 1939. In 1933 transurethral prostatic resection had been performed for relief of urinary obstruction. Two years later recurrence of obstructive symptoms necessitated a 2-stage prostatectomy. There had never been symptoms referable to the upper urinary tract. In January 1939, the patient complained of localized pain in the left costo-vertebral area, which did not radiate. There were no urinary symptoms. Physical examination was essentially negative. Intravenous urography showed the dye in the normally developed kidney and none elsewhere. (The ectopic kidney failed to concentrate the medium.) Retrograde pyelography demonstrated the left
FIG. 23 FIG. 24 Case 3. Bilateral retrograde pyelograms revealed typical crossed ectopia of left kidney without fusion, with pelvis overlying sacrum. FrG. 24. Case 3. Second retrograde pyelogram showing clearly the pelvis and shadow of the crossed kidney. FIG. 23.
ureter to cross over to the right side to the ectopic kidney, located overlying the right sacroiliac joint. There was no ureterectasis, pyelectasis or indication of calculus. The patient was treated for myositis of the muscles of the back on the left side with complete relief of pain. SUMMARY
We have endeavored to clarify the definition of ectopia and its various forms, and to delete confusing terms and synonyms given in the literature. The incidence of the different types, as well as their description, is given. The diagnostic features and surgical management are discussed.
RENAL ECTOPIA
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A review of three recent personal cases is included, two with transperitoneal nephrectomy and one with diagnostic fluoroscopy. Urologists are becoming more "anomaly-minded". Only by complete urological examination will earlier and more accurate diagnosis be made. and many errors avoided. 306 Park Place, Brooklyn, LY.
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REFERENCES ALCOCK, N. G.: .\ case of calculus pyonephrosis in a crossed ectopic non-fused kidney. Tr . .\m. A. Genito-Urin. Surgeons, 19: 299-310, 1926. BARRY, T. R., AND GARVEY, F. K.: Crossed renal ectopia. J. A. M ..\. 94: 1232-1233, 1930. BEEBEE, JYI. L.: Case reports; crossed ectopic kidney; accidental concealed hemorrhage. Colorado Med. 24: 255-256, 1927. BEER, EDWIN: Infected left calculous hydronephrosis in right iliac fossa. Am. Surg. 183: 433, 1926. BEER, E., AND FERBER, W. L. F.: Crossed renal ectopia. J. Ural. 38: 541-561, 1937. BEER, E., AND FERBE;i, W. L. F.: Crossed renal ectopia. J. Ural. 39: 479-486, 1938. BEGG, R. CAMPBELL: Heterolateral ectopia of right kidney. Brit. M. J. 1: 1049, 1938. BRAASCH, V\'. F.: The clinical diagnosis of congenital anomaly in the kidney and ureter. Tr. Am. A. Genito-Urin. Surgeons 8: 281-292, 1913. C.rnPBELL, M. F.: Renal ectopy. J. Urol., 24: 187-198, 1930. CARLETON, .\.: Crossed ectopia of the kidnev ancl its possible cause. J. Anat. 7: 292-298, 1937. CLARK,,\. M.: Ectopic kidney. Glasgo\\· M. J. 128: 45-47, 1937. COLLIXGS, C. W.: Ectopic kidney. ,\m. J. Surg. 7: 425-426, 1929. CmtPTON, A. J.: Crossed dystopia of kidney. Lancet 1: 810, 1926. COPPRIDGE, \V. M.: Bilateral pelvic kidneys. J. Ural. 32: 231-235, Sept, 1934. DAVIDSON, BERNARD: Renal ectopia with abdominal and pelvic symptomatology. Urol. and Cutan. Rev., 42: 334-336, 1938. DICKINSON, W. LEE: Solitary kidney. Ibid, 46: 80, 1895. FRASER, E1n!FTT B.: Congenital renal dystopia. Urol and Cutan. Rev., 39: 381-385, 1935. GOLDBERGER, M ..\.: Ectopic kidney. J. l\lt. Sinai Hosp., 4: 24-27, 1937. GUTIERREZ, R.: Role of anomalies of kidney and ureter in causation of surgical conditions. J. .-\. M. A. 106: 183, 1936. HERBST, R.H.: Case of crossed dystopia of right kidney. S. Clin. North America, 7: 579-581, 1927. HFR1IA:--i, LEON: Crossed renal ectopia. Pennyslvania M. J. 37: 1009, 1934. Ho\Y,\RD, T. LEON: Crossed ectopia of right kidney without fusion and a large hydronephrosis of the normally placed left kidney. Urol. and Cutan. Rev. 39: 145-149, 1935. Hun11A:-1, L. F.: Congenital displacements of the kidney. J. Urol.12: 363, 1924. JONES, R. TREVOR: Calculus in an ectopic kidney. Brit. J. Surg. 22: 886-887, 1935. KIDD, FRANK: Urinary surgery. London, Longmans Green & Co., 31, 1910. KRETSCHMER, H. L.: Unilateral fused kidney. Surg., Gynec. and Obst. 40: 360-366, 1925. KREUTZMANN, H. ,\. R.: Renal dystopia. v\'est J. Surg., 43: 605-608, 1935. LOONEY, W. W., AND DODD, D. L.: An ectopic completely fused kidney associated with various anomalies of the abdominal viscera. .\nn. Surg. 84: 522-524, 1926. JYIAcKExzrn, D. vV., AND HAWTHORNE, ALLAN B.: The ectopic kidney: report of 13 cases exclusive of horse-shoe kidneys. J. Ural. 39: 479-486, 1938. MAYERS, 11. M.: Crossed renal ectopia. J. Urol., 36: 111-112, 1936. McCowN, l'. E.: Bilateral renal ectopia. J. Ural. 22: 653-665, 1929. MORGAN, E. K., AND STONE, C. M.: Bilateral renal ectopia. J. Urol., 38: 427-429, 1937. MORGAK. J. L.: Ectopic kidney: case report. Memphis M. J., 13: 30-32, 1938. PAPIN, E .. AKD ErsENDRATH, D. N.: Classification of renal and ureteral anomalies. Ann. Surg. 85: 735-756, 1927. PATCH, F. S.: Carcinoma in a crossed heterolateral ectopic kidney without fusion. Brit. J. Ural., 9: 339-359, 1937. l'LU11MER, S. C.: Case of misplaced kidney. Surg., Gynec. and Obst. 10: 431-437, 1910.
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