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Pelvic Single Kidney: Report of an Additional Case
PELVIC SINGLE KIDNEY REPORT OF AN ADDITIONAL CASE
HILAIRE D. OGDE~ From the Divfrion of Urologic
AND
EMILE MALTRY
the School of Jl[edicine of Louisiana State ['nii•ersity, and the of Charity Hospital of Louisiana at New Orleans
Solitary kidney is uncommon and pelvic solitary kidney is still more uncommon. Stevens, in a review of the literature, estimated that ectopic kidney occurs once in every 500 to 1,100 autopsies, single kidney once in every 700 to l ,610 cases, pelvic kidney once in every 2,150 to 3 cases, and pelvic solitary kidney once in every 22,000 cases. The same author, in 1937, collected and tabulated the 27 cases of the latter condition which were then on record, and a single case has since been added by Houtappel. The purpose of the present communication is to add to the literature another case of pelvic solitary kidney, and to review briefly the embryologic causes of the anomaly. Excellent descriptions of the embryology of the kidneys have been given by Huntington and by Pohlman. These organs appear first in the 5 mm. embryo as 2 buds which lie close together in front of the second sacral segment and which arise from the Wolfiian duct a short distance from its entrance into the cloaca. Just before the 10 mm. stage is reached the buds sprout and give rise to the primary branches which result in the primary calices. The kidney buds migrate upward, following the line of the degenerating mesonephros. receive a temporary blood supply from the aorta, the common and external iliac, and the median-sacral and other neighboring vessels. The permanent blood supply is acquired when the kidneys reach their normal position, which in human embryos is at the 25 to 30 mm. stage, at the end of the second month of intrauterine life. If for any reason the ascent is arrested, the temporary blood supply usually becomes permanent for the affected kidneys. Axial rotation occurs in the 14 mm. embryo at the mid-lumbar level. Ectopic kidneys, because of their position, as a rule do not undergo axial rotation, the pelves, instead, remaining anterior to the calices. For this reason a bizarre radiographic picture is produced that may be interpreted as a renal neoplasm. The complete absence of a kidney, according to Hinman, is best es13
14
HILAIRE D. OGDEN AND EMILE MALTRY
plained by the failure of the ureteral bud to develop rather than by the absence of the mesonephric blastoma. Early degeneration of the ureteral bud is a possible cause, which can be proved only when some remnant of the kidney remains in situ. Fortune has proposed 4 possible causes for single kidney: (1) The metanephric bud may fail to appear, in spite of a normal preceding mesonephros; (2) the metanephros may appear, but undergoes early degeneration; (3) the mesonephros may be imperfectly developed; (4) pronephros may fail to develop, and the mesonephros, as a result, does not grow. Unilateral renal agenesis and anomalies of the genital tract are frequently associated, which is not surprising, because of the close embryological relationship between the urinary and genital tracts, and because the Mi.illerian ducts develop later than the Wolffian ducts. As the result of the original embryological defect, therefore, the Mi.illerian duct on the agenetic side is often absent also. Genital anomalies were observed in 13 of the 27 cases collected by Stevens, and were also present in the case which we are reporting. Pelvic single kidney is a possibility in any case, particularly a case in which genital anomalies are present, and constant pitfalls await the careless surgeon. If a pathologic state develops in such a kidney, which is also a possibility in any case, the situation is always grave and the necessary surgery must be done with the utmost conservatism. Surgical treatment is often difficult because of the inaccessible situation of the kidney, the relatively poor drainage, and the mild hydronephrosiswhich is practically always present. Preliminary pyelography is essential in all such cases. Case report. C. G., a white female 17 years of age, was admitted to the gynecologic service of Charity Hospital of Louisiana at New Orleans September 28, 1939. She complained that she bad never menstruated, and that she had not been improved by an operation which had been undertaken 6 months before to correct this condition. She did not know the nature of the operation. She also complained of attacks of abdominal pain 2 years before and 6 months before the present admission to the hospital. On both occasions the pain was steady and was not cramping. It had not been associated with nausea and vom1tmg. The patient was aware that she had no vagina, and denied any symptoms relating to menstruation. Careful questioning failed to elicit a history of a similar anomaly in any other female of the family during the last 4 generations. The past history was without incident except for whooping cough at 3 weeks
PELVIC SINGLE KIDNEY
15
and measles and influenza at 14 years of age. There were no urinary symptoms; the diurnal index was 3-4 and the nocturnal index 1-0. The patient's mentality was at the proper level for her age and she wa:,; well developed. Her height was S feet, 7 inches, and her weight 128 pounds. Superficial examination revealed nothing which would lead to the suspicion of anomalies of development and growth. The breasts were well developed, and the distribution of genital hair typical of the female sex. Examination of the chest revealed no demonstrable abnormalities of the lung fields. The apex beat of the heart was just medial to the left nipple. Slight tenderness was elicited in the left lower quadrant on palpation of the abdomen, but no masses could be made out. The external genitalia were well formed, but tbe vaginal cavity was represented only by a small inpoucbing
FIG.
1. Drawing made at operation showing the relation of the structures
posterior to the urethral orifice. Rectal examination revealed a rather large mass in the pelvis, posterior to the rectum and overlying the sacroiliac articulation. This mass was assumed to be the uterus. Urinalysis revealed clear urine, with a specific gravity of 1.023. It contained no albumin or sugar, and microscopic examination revealed no casts or cells. The non-protein nitrogen of the blood was 72 mg. and the sugar 98 mg. per 100 cc. Phenolsulphonphthalein tests showed SO per cent excretion during the first and 30 per cent excretion during the second hour. The blood \Vassermann reaction was negative. The abdomen was opened September 29, 1939, under spinal analgesia and through a Pfannenstiel incision (fig. 1). Exploration revealed congenital absence of the tubes and uterus. In the normal location of the uterus was a
16
HILAIRE D. OGDEN AND EMILE MALTRY
rudimentary horn which could be traced upward on each side of the abdomen to the level of the tenth thoracic vertebra, where it terminated in a firm, white, glistening mass, 2 by 3 by S cm. in size, with a very thick capsule. To each mass was attached a structure of the same consistency as itself, and resembling a pedicle. When these structures were followed to their point of origin, it could be determined that the masses apparently arose from points just below the diaphragm. It was concluded that they represented the ovaries, although there was no external evidence that they were capable of function. On the left side the rudimentary ovary was attached to the spleen. The sigmoid was located on the right instead of on the left side. The ileocecal junction could not be found on either side and the appendix was also absent.
FIG. 2. Intravenous urogram showing location of pelvis and ureter
The bladder was in its normal position. Immediately below the promontory of the sacrum was a bean-shaped, retroperitoneal mass, 4 by S by 6 cm. in size, with its axis lateral. It apparently received its blood supply from the aorta, just above the bifurcation of the common iliac vessels. Careful examination failed to reveal any evidence of renal tissue in either of the renal fossae, and the pelvic mass, which had the consistency of normal kidney tissue, was therefore assumed to be an ectopic kidney. After the patient had recovered from the exploratory operation she was referred to the urological department for a study of the pel vie kidney. Cystoscopy (October 8) revealed the bladder mucosa to be normal in appearance. A well defined ureteral orifice was made out on the left side but none was present on the right. A large caliber (No. 8 F.) catheter was introduced into
PELVIC SINGLE KIDNEY
17
the left ureter and kept in situ while indigo-carmine was given intravenously. At the end of 30 minutes no trace of dye could be seen coming from any site in the bladder that might indicate a right ureteral orifice. An intravenous urogram, which was clone at the time of the cystoscopic examination, revealed an ectopic kidney overlying the sacrum (fig. 2). The single ureter apparently arose from the right side of the kidney mass and crossed the miclline to enter the bladder at the usual site of the left ureteral orifice. The renal pelvis was slightly dilated. No evidence of a kidney shadow could be made out on either side in the normal position. The patient was discharged October 12, 1939, in excellent condition, after being warned of the presence and implications of the pelvic solitary kidney.
Summary reported cases. At the present time, and including the case herewith reported, 29 cases of single pelvic kidney are on record in the literature. The first case was reported by Henot, in 1830, and concerned an 8-months fetus. Like the next 4 reported cases, it was discovered only at autopsy. Nineteen of the 29 cases on record been reported in the last 30 years. Thirteen of the 19 patients whose sex is recorded were females. The age was stated in 23 cases and, excluding the fetus already mentioned, ranged from 1 month to 68 years. Genital anomalies were present in 14 cases, extragenital anomalies in 2, and mental deficiencies in 2. In only 1 of the remaining cases is the definite statement made that no anomalies were present. The abdomen was opened in 12 cases, in 3 of which lithotomy was done, with 2 operative survivals. The third patient died of lobar pneumonia. Another patient was subjected to transperineal lithotomy for hydronephrosis with stones, developed a urinary fistula, and died of uremia 3 years later. In 2 cases the kidney was extirpated and death occurred promptly from anuria. One patient died of parenchymatous nephritis, another a month after operation for anovesical atresia, and a third of scarlet fever. In none of the other cases in which death occurred before the report was made does the cause seem to have been directly or indirectly related to the renal anomaly. In 4 of the 8 cases in which the blood supply was stated the origin was at or just above the bifurcation of the aorta. More than 1 renal artery was present in 4 cases. SUMMARY AND CONCLUSIONS
Another case of pelvic single kidney is added to the 28 cases now on record in the literature, 27 of which were tabulated Stevens.
18
HILAIRE D. OGDEN AND EMILE MALTRY
The frequency of associated anomalies, particularly of the genital organs, in cases of pelvic single kidney is demonstrated again in the case reported, in which the uterus and tubes were also absent, the ovaries congenitally displaced, the sigmoid displaced to the left, and the appendix and vagina absent. The embryology of the kidney is briefly reviewed, and a possible explanation for unilateral renal agenesis is presented. It is emphasized that renal anomalies should be suspected whenever genital anomalies are present. It is also emphasized that whenever the congenital absence of one kidney is suspected, careful pyelographic examination should be undertaken. This is particularly important if pathologic states requmng radical surgery should develop. REFERENCES FORTUNE, C. H.: The pathological and clinical significance of congenital one-sided kidney defect, with the presentation of 3 cases of agensia and one of aplasia. Ann. Int. Med., 1: 377, 1927. HINMAN, F.: Principles and Practice of Urology, Philadelphia, W. B. Sannders Co., 1935. HouTAPPEL, H. C. E. M.: Pelvic single kidney; a case. Brit. J. Urol., 9: 372, 1937. HUNTINGTON, G. S.: The genetic interpretation and surgical significance of some variations of the genitourinary tract. In Harvey Lectures, 2: 222, Philadelphia, J. B. Lippincott Co., 1908. POHLMAN, A. G.: Concerning the embryology of kidney anomalies. Am. Med., 7: 987, 1904. STEVENS, A. R.: Pelvic single kidneys. J. Urol., 37: 610, 1937.
HILAIRE D. OGDE~ From the Divfrion of Urologic
AND
EMILE MALTRY
the School of Jl[edicine of Louisiana State ['nii•ersity, and the of Charity Hospital of Louisiana at New Orleans
Solitary kidney is uncommon and pelvic solitary kidney is still more uncommon. Stevens, in a review of the literature, estimated that ectopic kidney occurs once in every 500 to 1,100 autopsies, single kidney once in every 700 to l ,610 cases, pelvic kidney once in every 2,150 to 3 cases, and pelvic solitary kidney once in every 22,000 cases. The same author, in 1937, collected and tabulated the 27 cases of the latter condition which were then on record, and a single case has since been added by Houtappel. The purpose of the present communication is to add to the literature another case of pelvic solitary kidney, and to review briefly the embryologic causes of the anomaly. Excellent descriptions of the embryology of the kidneys have been given by Huntington and by Pohlman. These organs appear first in the 5 mm. embryo as 2 buds which lie close together in front of the second sacral segment and which arise from the Wolfiian duct a short distance from its entrance into the cloaca. Just before the 10 mm. stage is reached the buds sprout and give rise to the primary branches which result in the primary calices. The kidney buds migrate upward, following the line of the degenerating mesonephros. receive a temporary blood supply from the aorta, the common and external iliac, and the median-sacral and other neighboring vessels. The permanent blood supply is acquired when the kidneys reach their normal position, which in human embryos is at the 25 to 30 mm. stage, at the end of the second month of intrauterine life. If for any reason the ascent is arrested, the temporary blood supply usually becomes permanent for the affected kidneys. Axial rotation occurs in the 14 mm. embryo at the mid-lumbar level. Ectopic kidneys, because of their position, as a rule do not undergo axial rotation, the pelves, instead, remaining anterior to the calices. For this reason a bizarre radiographic picture is produced that may be interpreted as a renal neoplasm. The complete absence of a kidney, according to Hinman, is best es13
14
HILAIRE D. OGDEN AND EMILE MALTRY
plained by the failure of the ureteral bud to develop rather than by the absence of the mesonephric blastoma. Early degeneration of the ureteral bud is a possible cause, which can be proved only when some remnant of the kidney remains in situ. Fortune has proposed 4 possible causes for single kidney: (1) The metanephric bud may fail to appear, in spite of a normal preceding mesonephros; (2) the metanephros may appear, but undergoes early degeneration; (3) the mesonephros may be imperfectly developed; (4) pronephros may fail to develop, and the mesonephros, as a result, does not grow. Unilateral renal agenesis and anomalies of the genital tract are frequently associated, which is not surprising, because of the close embryological relationship between the urinary and genital tracts, and because the Mi.illerian ducts develop later than the Wolffian ducts. As the result of the original embryological defect, therefore, the Mi.illerian duct on the agenetic side is often absent also. Genital anomalies were observed in 13 of the 27 cases collected by Stevens, and were also present in the case which we are reporting. Pelvic single kidney is a possibility in any case, particularly a case in which genital anomalies are present, and constant pitfalls await the careless surgeon. If a pathologic state develops in such a kidney, which is also a possibility in any case, the situation is always grave and the necessary surgery must be done with the utmost conservatism. Surgical treatment is often difficult because of the inaccessible situation of the kidney, the relatively poor drainage, and the mild hydronephrosiswhich is practically always present. Preliminary pyelography is essential in all such cases. Case report. C. G., a white female 17 years of age, was admitted to the gynecologic service of Charity Hospital of Louisiana at New Orleans September 28, 1939. She complained that she bad never menstruated, and that she had not been improved by an operation which had been undertaken 6 months before to correct this condition. She did not know the nature of the operation. She also complained of attacks of abdominal pain 2 years before and 6 months before the present admission to the hospital. On both occasions the pain was steady and was not cramping. It had not been associated with nausea and vom1tmg. The patient was aware that she had no vagina, and denied any symptoms relating to menstruation. Careful questioning failed to elicit a history of a similar anomaly in any other female of the family during the last 4 generations. The past history was without incident except for whooping cough at 3 weeks
PELVIC SINGLE KIDNEY
15
and measles and influenza at 14 years of age. There were no urinary symptoms; the diurnal index was 3-4 and the nocturnal index 1-0. The patient's mentality was at the proper level for her age and she wa:,; well developed. Her height was S feet, 7 inches, and her weight 128 pounds. Superficial examination revealed nothing which would lead to the suspicion of anomalies of development and growth. The breasts were well developed, and the distribution of genital hair typical of the female sex. Examination of the chest revealed no demonstrable abnormalities of the lung fields. The apex beat of the heart was just medial to the left nipple. Slight tenderness was elicited in the left lower quadrant on palpation of the abdomen, but no masses could be made out. The external genitalia were well formed, but tbe vaginal cavity was represented only by a small inpoucbing
FIG.
1. Drawing made at operation showing the relation of the structures
posterior to the urethral orifice. Rectal examination revealed a rather large mass in the pelvis, posterior to the rectum and overlying the sacroiliac articulation. This mass was assumed to be the uterus. Urinalysis revealed clear urine, with a specific gravity of 1.023. It contained no albumin or sugar, and microscopic examination revealed no casts or cells. The non-protein nitrogen of the blood was 72 mg. and the sugar 98 mg. per 100 cc. Phenolsulphonphthalein tests showed SO per cent excretion during the first and 30 per cent excretion during the second hour. The blood \Vassermann reaction was negative. The abdomen was opened September 29, 1939, under spinal analgesia and through a Pfannenstiel incision (fig. 1). Exploration revealed congenital absence of the tubes and uterus. In the normal location of the uterus was a
16
HILAIRE D. OGDEN AND EMILE MALTRY
rudimentary horn which could be traced upward on each side of the abdomen to the level of the tenth thoracic vertebra, where it terminated in a firm, white, glistening mass, 2 by 3 by S cm. in size, with a very thick capsule. To each mass was attached a structure of the same consistency as itself, and resembling a pedicle. When these structures were followed to their point of origin, it could be determined that the masses apparently arose from points just below the diaphragm. It was concluded that they represented the ovaries, although there was no external evidence that they were capable of function. On the left side the rudimentary ovary was attached to the spleen. The sigmoid was located on the right instead of on the left side. The ileocecal junction could not be found on either side and the appendix was also absent.
FIG. 2. Intravenous urogram showing location of pelvis and ureter
The bladder was in its normal position. Immediately below the promontory of the sacrum was a bean-shaped, retroperitoneal mass, 4 by S by 6 cm. in size, with its axis lateral. It apparently received its blood supply from the aorta, just above the bifurcation of the common iliac vessels. Careful examination failed to reveal any evidence of renal tissue in either of the renal fossae, and the pelvic mass, which had the consistency of normal kidney tissue, was therefore assumed to be an ectopic kidney. After the patient had recovered from the exploratory operation she was referred to the urological department for a study of the pel vie kidney. Cystoscopy (October 8) revealed the bladder mucosa to be normal in appearance. A well defined ureteral orifice was made out on the left side but none was present on the right. A large caliber (No. 8 F.) catheter was introduced into
PELVIC SINGLE KIDNEY
17
the left ureter and kept in situ while indigo-carmine was given intravenously. At the end of 30 minutes no trace of dye could be seen coming from any site in the bladder that might indicate a right ureteral orifice. An intravenous urogram, which was clone at the time of the cystoscopic examination, revealed an ectopic kidney overlying the sacrum (fig. 2). The single ureter apparently arose from the right side of the kidney mass and crossed the miclline to enter the bladder at the usual site of the left ureteral orifice. The renal pelvis was slightly dilated. No evidence of a kidney shadow could be made out on either side in the normal position. The patient was discharged October 12, 1939, in excellent condition, after being warned of the presence and implications of the pelvic solitary kidney.
Summary reported cases. At the present time, and including the case herewith reported, 29 cases of single pelvic kidney are on record in the literature. The first case was reported by Henot, in 1830, and concerned an 8-months fetus. Like the next 4 reported cases, it was discovered only at autopsy. Nineteen of the 29 cases on record been reported in the last 30 years. Thirteen of the 19 patients whose sex is recorded were females. The age was stated in 23 cases and, excluding the fetus already mentioned, ranged from 1 month to 68 years. Genital anomalies were present in 14 cases, extragenital anomalies in 2, and mental deficiencies in 2. In only 1 of the remaining cases is the definite statement made that no anomalies were present. The abdomen was opened in 12 cases, in 3 of which lithotomy was done, with 2 operative survivals. The third patient died of lobar pneumonia. Another patient was subjected to transperineal lithotomy for hydronephrosis with stones, developed a urinary fistula, and died of uremia 3 years later. In 2 cases the kidney was extirpated and death occurred promptly from anuria. One patient died of parenchymatous nephritis, another a month after operation for anovesical atresia, and a third of scarlet fever. In none of the other cases in which death occurred before the report was made does the cause seem to have been directly or indirectly related to the renal anomaly. In 4 of the 8 cases in which the blood supply was stated the origin was at or just above the bifurcation of the aorta. More than 1 renal artery was present in 4 cases. SUMMARY AND CONCLUSIONS
Another case of pelvic single kidney is added to the 28 cases now on record in the literature, 27 of which were tabulated Stevens.
18
HILAIRE D. OGDEN AND EMILE MALTRY
The frequency of associated anomalies, particularly of the genital organs, in cases of pelvic single kidney is demonstrated again in the case reported, in which the uterus and tubes were also absent, the ovaries congenitally displaced, the sigmoid displaced to the left, and the appendix and vagina absent. The embryology of the kidney is briefly reviewed, and a possible explanation for unilateral renal agenesis is presented. It is emphasized that renal anomalies should be suspected whenever genital anomalies are present. It is also emphasized that whenever the congenital absence of one kidney is suspected, careful pyelographic examination should be undertaken. This is particularly important if pathologic states requmng radical surgery should develop. REFERENCES FORTUNE, C. H.: The pathological and clinical significance of congenital one-sided kidney defect, with the presentation of 3 cases of agensia and one of aplasia. Ann. Int. Med., 1: 377, 1927. HINMAN, F.: Principles and Practice of Urology, Philadelphia, W. B. Sannders Co., 1935. HouTAPPEL, H. C. E. M.: Pelvic single kidney; a case. Brit. J. Urol., 9: 372, 1937. HUNTINGTON, G. S.: The genetic interpretation and surgical significance of some variations of the genitourinary tract. In Harvey Lectures, 2: 222, Philadelphia, J. B. Lippincott Co., 1908. POHLMAN, A. G.: Concerning the embryology of kidney anomalies. Am. Med., 7: 987, 1904. STEVENS, A. R.: Pelvic single kidneys. J. Urol., 37: 610, 1937.