Paper - A case of congenital absence of the left kidney and ureter (1915)
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Gladstone RJ. A case of congenital absence of the left kidney and ureter. (1915) J Anat. Physiol. 49(4): 418-428.PMID 17233045
|renal abnormality, congenital absence of the left kidney and ureter.
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A Case of Congenital Absence of the Left Kidney and Ureter
By Reginald J. Gladstone,, M.D. Aberd., F.R.C.S. Eng., F.R.S. Edin.,
Lecturer and Demonstrator in Anatomy at King’s College, University of London.
Congenital anomalies of the urino-genital system are of interest not only as teratological curiosities, but they are also of interest to the embryologist on account of the light that they sometimes throw upon normal developmental processes. Further, as in the case described below, they frequently have a very special and important bearing on surgical practice.
Description of Case
Complete absence of the left kidney and ureter was discovered during the dissection of a male subject in the Anatomy Department, King’s College, London.| The man, who was excessively fat, was certified to have died from chronic bronchitis, aged 56. The right kidney (fig. 1) was normal in position, and, as is usual in cases of single kidney, was greatly enlarged. It was rather more than double the average weight of the kidney in the male.
The measurements and weight of the “single kidney,” compared with the normal organ, are as follows :—
Single Kidney. Normal Kidney. | Length . . .| 52ins, (150 mm.) 4} ins. (105 mm.) | Width sos : 23 ,, (72 4, ) 24 ,, (60 ,, ) | Thickness . . . 24 ” (55 2” ) 13 ”? (30 ” ) | Weight . . . 11 oz. (315 grm.) 44 oz, (130 grm.)
The right kidney was elongated in the vertical diameter, and distinctly lobulated, but was otherwise normal in appearance. Both suprarenal bodies were present, and they were normal in size and position. The left, however, differed in form; for, owing to absence of the left kidney, the upper pole of which normally exerts pressure on the under surface of the suprarenal body, this surface, instead of being concave, was convex.
- 1 I have to thank the students who were engaged in the dissection of the subject, Mr H. O. Gunewardine and Mr A. M. G. Mishad, who first drew my attention to the defect.
Right suprarenal body.
Left suprarenal body.
Renal ; ae: arteries. 3-8
Renal veins. ( Left spermatic 2. vessels.
Orifice of ureter. Fic. 1.—Absence of left kidney and ureter,
The ureter on the left side was completely absent, and there was no indication of a left ureteral orifice in the interior of the bladder. The pelvis of the right ureter was larger than usual, but in other respects it appeared to be quite normal.
The testis, epididymis, deferent duct, and seminal vesicle of the left side were present but imperfectly developed. The veins of the spermatic cord were large and somewhat varicose. The cavity of the tunica vaginalis was obliterated. On section, the testis appeared to the naked eye white and fibrous. It was dense in consistence, and the tubules appeared to be imperfectly developed, and imbedded in dense fibrous tissue. The epididymis appeared to be formed almost entirely of a venous plexus supported by a loose connective tissue. The deferent duct was represented by a thin fibro-muscular band, which was with difficulty distinguished from the other constituents of the cord. It pursued the normal course, and terminated in the usual position at the base of the prostate gland by joining with a fibrous cord representing the duct of the seminal vesicle. The left seminal vesicle was, however, quite rudimentary, and no lumen could be recognised in it; nor could the opening of the common ejaculatory duct be found in the floor of the prostatic urethra.
The bladder was contracted and its walls greatly hypertrophied As previously stated, only one ureteral orifice, the right, was present. The hypertrophy was obviously due to urethral obstruction. There was, however, no definite stricture, but rather a general narrowing of the canal, which was sufficiently marked to prevent the passage of a mediumsized sound. Whether the small size of the canal was a congenital stenosis, or the result of inflammation, it is difficult to decide, more especially as I have been unable to obtain any history of the case. As, however, there were no obvious signs of inflammation, I am inclined to regard the small size of the urethra as a congenital defect associated with imperfect development of the left half of the urogenital sinus.
Microscopicaal Appearance of a Longitudinal Section of the Testicle
The organ was cut entire, stained with hematoxylin and counterstained with eosin. The outer fibrous tunic (t. albuginea) was greatly thickened. The substance of the organ (fig. 2) did not show the normal subdivision into compartments or loculi, by septa converging from the periphery to the mediastinum, but consisted of an irregular network of coarse bands, in the meshes of which the seminiferous tubules were embedded. The tubules differed markedly in appearance in different parts of the organ. In some parts the epithelium of the tubules had entirely disappeared, and the walls were greatly thickened, so that the lumen was reduced to a mere chink. Some tubules, on the other hand, were more or less completely filled with epithelium, the cells being mostly large, and rounded or polygonal in shape. They contained large spherical nuclei, the chromatic elements of which stained deeply with hematoxylin. Scattered about in the region of the mediastinum and between the tubules were other cells of an epithelial type; these, the “interstitial cells,’ formed irregular
Fig. 2.— Section through left testicle from case in which left kidney was absent, showing imperfect development of seminiferous tubules and group of interstitial cells, groups, which, owing to the degeneration of the epithelial cells in the tubules, appeared more than usually conspicuous.
In the epididymis the tubules were large and replaced by a vascular plexus. The walls of the tubules were greatly thickened, and the ‘epithelium degenerated.
Dr Shattock, who very kindly examined one of the sections for me, stated that the condition was very similar to that of an undescended testicle, and that he believes the thickening of the walls of the tubules to be due to irritation produced by toxic products from the degenerating epithelium.
The left renal artery was entirely absent. The right kidney received three arteries, which arose close together from the abdominal aorta. They occupied a normal position behind the inferior vena cava. The first or highest of these was given off at the level of the superior mesenteric artery. It supplied the upper part of the organ and a branch to the right suprarenal. One of the main terminal branches entered the anterior surface of the kidney above the hilum; another passed into the upper part of the hilum. The second and third branches arose from the right side of the abdominal: aorta, one in front of the other, and both of them about half an inch below the level of the first. The second passed in front of the renal vein and pelvis of the ureter, the third behind both. They supplied the middle and lower part of the kidney.
The left suprarenal body received its blood-supply from the left inferior phrenic, the aorta, and the left spermatic arteries.
The right suprarenal body was supplied by the right inferior phrenic, aorta, and highest right renal artery.
There were two left spermatic arteries; one arose at the level of the superior mesenteric artery from the left side of the aorta, the other about half an inch below this from the same side of the aorta. They joined below to form a single vessel.
The “left renal” vein was represented by a small trunk formed by the union of the left suprarenal vein with the left spermatic vein. The suprarenal was joined by the left inferior phrenic vein. The main vein received a small tributary from the second left lumbar vein. This vessel probably represented the communication of the left posterior cardinal vein with the transverse anastomosis joining the subcardinal and posterior cardinal veins; this anastomosis normally forms the terminal part of the left renal vein.
Two right renal veins were present, a larger one above, which joined the inferior vena cava just below the level of the vessel representing the left renal vein, and a smaller vein below, which joined the inferior vena cava half an inch below the level of the former. Case ‘of Congenital Absence of the Left Kidney and Ureter 423
Structure and Nature of Enlargement
On making a longitudinal section through the enlarged right kidney, it was obvious that the pyramids were also enlarged, and that the general structure of the organ was coarser than in the normal kidney. Further, on counting the number of pyramids visible in the section, I found that, notwithstanding the large size of the organ, there were only nine, which is approximately the number visible in a similar section of a normal kidney.
In making comparison with the normal kidney, I counted the number of pyramids visible in a mesial longitudinal section of twenty kidneys obtained from the dissecting room, and found that the average in twelve right kidneys was 99, and in eight left kidneys 9-5. Thus in the normal kidneys, though only half the size, there were in the average rather more pyramids visible in the mesial section, than in the enlarged single kidney.
Moreover, on examining sections of the enlarged “single kidney” with the microscope, and comparing these with the normal, the glomeruli and tubules were seen to be much larger than in the normal kidney. The average diameter of twenty-five glomeruli in the “single kidney ” was 0°2 mm.; in a normal human kidney used for comparison the average was 0:13 mm. There was a similar enlargement of the tubules of the “single kidney.”
Further, on counting the number of glomeruli visible in a given area of the “single kidney,” which I accomplished by enumerating those falling within an area of 16 squares ruled on a’ glass inserted inside the ocular of the microscope, I found that the average number falling within these 16 squares in 50 counts was 2°8 in the “single kidney,” 5°5 in the normal. The number of glomeruli falling within the given area, in the “single kidney,” thus averages about half that in the normal.
Average number of glomeruli seen in 50 counts of 16 squares.
——— 0S Single kidney. Normal kidney.
2°8 55 3 = approx. 2.
The enlargement of the kidney is thus a pure hypertrophy, or enlargement by increase in the size of the constituent elements, and not a hyperplasia, or enlargement by increase in their number. In other words, the total glomeruli in the enlarged “single kidney” must have been approximately equal in number to those contained in a normal kidney, in spite of the great difference in size between the two.
The credit of first pointing out that the enlargement in a case of “single kidney” is a pure hypertrophy is due to Professor A. E. Boycott, who cut serial sections of blocks having a definite weight from kidneys in the rabbit. One of these was taken from a case of single kidney, the others from two “controls.” He counted the glomeruli with the aid of camera lucida drawings. My observations on the human subject must therefore be regarded as confirmatory of his work carried out in the rabbit.
The possibility of one kidney being absent has an important bearing on modern surgical practice, and more especially with reference to the operation of nephrectomy.
In considering these cases it is necessary to distinguish between :—
1. Congenital absence of one kidney, including those cases in which one kidney is rudimentary and functionless.
2. Fusion of two kidneys into a single mass (horse-shoe and disc-shaped kidneys).
3. Atrophy or destruction of a kidney as the result of disease.
Secondly, it is of importance to have some idea as to the frequency of these conditions; and thirdly, we must consider the signs and symptoms which would lead one to expect the absence of one kidney, or the fusion of two into a single mass.
The frequency of congenital absence of one kidney, fusion of two kidneys, and atrophic kidneys has been very thoroughly worked out by Sir Henry Morris, who gives the following averages derived from the reports of post-mortem examinations conducted at ‘four London hospitals, combined with published statistics of other writers :—
1. Congenital absence of one kidney . lin 3,992 or 25 per 1000 2. Fusion of two kidneys (horse-shoe, etc.) 19 in 18,244 or 1 per 1000 3. Atrophied, small, shrunken, or wasted kidneys. . . . 59 in 8,178 or 7 2 per 1000
Congenital absence of one e kidney is thus seen to be an extremely rare abnormality. From the surgical standpoint, however, in neither of the first two groups should nephrectomy be performed; and probably; in the greater number of cases belonging to the third group, the operation would be contraindicated, as the atrophic kidney, if the other was removed, would be unequal to the strain which would suddenly be thrown upon it. The total number of cases, therefore, falling into one or other of these three groups in which nephrectomy is contraindicated is about 8 per 1000. This number is, however, considerably increased by the fact that when one kidney is absent, or is atrophied, the other kidney is frequently diseased. Thus it is probable that the proportion of cases in which one kidney is absent would be greater in those subjects suffering from symptoms of renal disease than in the total number of cases examined “post mortem ” in a general hospital. Moreover, the importance of recognising the condition is so great, that in all cases in which nephrectomy is contemplated a careful examination should be made to ascertain the existence and condition of the opposite kidney.
The most important signs of absence of one kidney are :—
1. Absence of an ureteral orifice on one side of the bladder.
2. Absence of the “kidney shadow” when the patient is examined with X-rays.
3. Some congenital defect of the external organs of generation on the same side.
In one instructive case described by Guthrie and Wilson, and operated on by W. J. Mayo, absence of the left kidney and ureter was diagnosed by cystoscopic examination previous to an operation for the removal of a tumour, which proved to be the uterus and upper part of the vagina distended with retained menstrual fluid. The lower portion of the vagina, the left ovary and Fallopian tube were completely absent. The labia majora were rudimentary, and the labia minora absent. The case emphasises the importance of cystoscopic examination of the bladder, and also the importance of examining the external genital organs, which, in congenital absence of one kidney, are frequently imperfectly developed on the same side as that on which the kidney is absent.
In the case which I have described, the atrophic condition of the left testicle and deferent duct would, taken in conjunction with absence of the ureteral orifice, have formed a very important clue to the absence of the left kidney.
Abnormalities of the genital organs are, however, not the only. con' genital defects which may accompany absence of one kidney. In a specimen (558°4) presented by Frank Deas to the Royal College of Surgeons, England, last year, absence of the right kidney was associated with imperforate anus and hydrocephaly. In specimen 7022, also added to the Museum of the Royal College of Surgeons last year, imperfect differentiation of the sexual organs in a newly born child is combined with an imperforate condition of the rectum. There was also an imperfect penis and scrotum, and no sexual glands.
In my own experience of teratological specimens, I find that one congenital defect is nearly always associated with other defects, and often of some quite distant part. The frequent occurrence in one subject of congenital defects in different and often distant parts of the body indicates, therefore, that special care should be taken to ascertain whether - one kidney is absent, in all those cases in which nephrectomy is proposed and some congenital defect, eg. an “accessory auricle” or “club foot,” is present.
It is curious that, since writing the above sentence, I have received a letter from Mr Frank Deas, informing me that the child from whom specimen 558-4 was
prepared also ‘had a curious deformity of the hands, which resembled feet.” This was not stated in the description in the catalogue.
Absence of the ureteral orifice on one side does not necessarily involve complete absence of the kidney on the same side; it may be present but in a cystic condition, as in a case of Sir J. Bland Sutton’s, reported by Sir H. Morris, or it may be otherwise diseased, as in a specimen (648°6) in the Museum of the Royal College of Surgeons, England, presented last year by Dr F. Mott. In this case the termination of the left ureter was congenitally deficient, and the left kidney was saccular and filled with a caseous substance. In these and similar cases, however, the kidney is functionally absent and from the surgical standpoint useless.
Two ureteral orifices may be present and yet one kidney may be absent, or quite rudimentary. The pelvis of the ureter in these cases is also small or absent altogether. The condition, if suspected, might be diagnosed by passing with a catheterising cystoscope an ureteral catheter (which is opaque to X-rays) as far as the renal pelvis. The renal pelvis may then be injected with collargol 10 per cent. in water, and the patient then examined with the X-rays. This should be done on both sides, as the condition is sometimes bilateral. Such a case occurred last year in a female subject in the Anatomy Department of King’s College, London. Both kidneys and ureters were atrophied, as were also the ureters, ovaries, and Fallopian tubes. The main blood-vessels of the abdomen and pelvis were also about half their normal size. The method of injecting with collargol would be useful also in the diagnosis of “horse-shoe” and other forms of fused kidney. In these cases the kidney and pelves of the ureters are usually situated near the middle line, and are lower in position than the normal kidney.
Should an operation for nephrectomy have already been commenced, without a previous cystoscopic and X-ray examination having been made, and it is found that the kidney (apart from the disease) is nearly double the normal size, and that there is more than one renal artery and vein, these conditions should lead one to suspect the absence of the opposite kidney, and the operation should not be completed without first having ascertained that the opposite kidney is present. Case of Congenital Absence of the Left Kidney and Ureter 427
The causation of congenital absence of one kidney and its ureter is, like that of other congenital abnormalities, obscure, but it is probable that the defect originates in an arrest of development of the distal end of the Wolffian duct, and of its union with that part of the cloaca from which the urogenital sinus is derived. The normal outgrowth of the renal diverticulum is therefore checked. The mesonephros, the genital gland, and the upper or proximal end of the Wolffian duct, if formed, subsequently atrophy. This atrophy in the male subject is probably due in part, at least, to there being no outlet for the escape of the testicular secretion.
The frequent cystic condition of the atrophic forms of kidney may be explained by a faulty union, or a complete failure in the union of the tubules of the metanephric blastema with the excretory tubules of the renal diverticulum.
The kidney is congenitally absent on the left side about twice as frequently as on the right. In twenty cases, including my own, the left kidney was absent in fourteen, the right in six. This may possibly be due to the fact that the umbilical cord is most frequently to the right side of the tail in young embryos of from 4-10 mm. length. It is during this period that the Wolffian duct first penetrates the wall of the cloaca and the renal diverticulum grows out from its lower end. Now, as the cloaca will be pulled over to the right with the umbilical cord, it will be farther away from the growing lower end of the Wolffian duct on the left than on the right side. This may account for greater frequency in the failure to unite with the cloaca on the left than on the right side.
In conclusion, I will give a brief summary of the more important points to be learnt from a consideration of these cases.
1. The enlargement of the remaining kidney, when one is congenitally absent, is a pure hypertrophy.
2. The importance of cystoscopic and X-ray examinations, and of noting the presence of other congenital defects, in all cases in which nephrectomy is contemplated.
3. Congenital absence of the kidney and ureter is in all probability due to an arrest in the development of the distal end of the Wolffian duct, and to a failure in its union with the cloaca. 428 Case of Congenital Absence of the Left Kidney and Ureter
References to Literature
Boycort, A. E., “A Case of Unilateral Aplasia of the Kidney in a Rabbit,” Jour. Anat. and Physiol., vol. xlv. p. 20.
Branca, Aupert, et Feuizet, G., “Sur les cellules intersticielles du testicule ectopique,” Paris, C’. R. Soc. biol., vol. liii, 1901, p. 311.
GREENFIELD, Path Soc. Trans., xxviii. p. 164.
GuturRiz, DonaLp, and Witson, L. B., Ann. Sury. Lond., vol. 1., 1909, p. 907.
Herrincuam, Kidney Diseases, 1912.
Moorz, Craven, Jour. Anat. and Physiol., 1899, p. 400.
Morris, H., Surgical Diseases of the Kidney and Ureter, vol. i. p. 18.
Petersen, Med. Times and Gazette, 1882, Oct. 7.
Poik, W., New York Med. Jour., Feb. 17, 1883.
Wer, R. F., New York Med. Jour., Dec. 27, 1884.
Wizson, L. B., see GUTHRIE.
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