Unilateral Multiple Cysts of the Kidney1

THE JOURNAL OF UROLOGY

Vol. 61, No. 2, February 1949

Print£d in U.S.A.

UNILATERAL MULTIPLE CYSTS OF THE KIDNEY1 CHARLES P. HOWZE

AND

JOHN H. HILL

In 1944, Braasch and Hendrick proposed the following classification of renal cysts: 1) retention or infl.arn.1natory cysts; 2) simple cysts-single, multiple, multilocular and hemorrhagic; 3) peripelvic (pyelogenic cysts); 4) parapelvic cysts; 5) cysts secondary to renal pathological changes (calculus, hypernephroma, tuberculosis, pyelonephritis, hematoma and ecchinococcus); and 6) polycystic kidneys. We wish to report 2 cases of unilateral polycystic kidney. The term polycystic is used to denote multiple discrete cysts of the kidney and not multilocular cysts, which are large cysts subdivided into multiple smaller divisions. The reported cases of unilateral cystic disease are few in number. In 1933 Braasch and Meland presented 10 cases of multilocular cysts of the kidney in adults and in 1944, Braasch and Hendrick added 2 similar cases. Six cases have been reported in children. The 2 cases in this communication bring the total to 8. The unilaterality of renal cysts is open to controversy. Clinically there are only 2 ways of determining accurately their presence: 1) by pyelographic studies and 2) inspection and palpation of the kidney on the operating table. Either method could fail to discover minute cysts. In spite of this fact, we believe that unilateral cysts do occur as do other unilateral congenital anomalies of the upper urinary tract. In a study of the reported cases in infants and children (table 1) it is noted that 4 are reported as multicystic (discrete cysts) and 4 as multilocular. A pertinent observation in the first group was the absence or incomplete development of the primodial renal arteries, collecting tubules, calyces and pelvis; namely the lateral segmental branch of the dorsal artery and the metanephric diverticulum. The age of the youngest patient in this series was 20 days and the oldest 13 years. There were 6 males and 2 females; I Negro and 7 white. The predominating sign in the infant cases of unilateral cystic kidneys has been a mass in the region of the kidney. Hematuria was observed in 2 cases. In 6 instances, the mass was on the left and in 2 on the right. Except for an occasional pus cell in 2 cases, the urinalyses were normal. Seven cases were treated by nephrectomy and later reported cured. Campbell's case was found at autopsy. Many explanations for renal cysts have been advanced. Hildebrant's belief that cyst formation results from failure of union of the nephroblastemicelements (glomeruli) and the collecting tubules was accepted for years, but other experimental studies have offered interesting speculation. Kampmeier shows a separate origin for the convoluted and collecting tubules of the kidney. In a study of fetal kidneys he found that there is a period of fetal development characterized by the existance of cystic renal tubules. Normally this generation of tubules collapse and disappear. However, the persistence of these is thought 1 Read at annual meeting, American Association of Genito-Urinary Surgeons, Skytop, Pa., May 14, 1948.

187

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CHARLES P. HOWZE AND JOHN H, HILL TABLE CASE REPORTS

AGE

SEX

1

SIGNS AND SYMPTOMS

SIDE

PATHOLOGICAL FINDINGS

1. Schwartz .......

7 mos.

M

Mass

Left

Multicystic: No definite renal artery, pelvis, calyces, pyramids or cortex. Pea-size renal tissue; nonfunctioning

2. Lynch and Thompson ....

7 mos.

F

Mass

Left

Multicystic: Condition of renal artery and pelvis not stated in report. No normal renal tissue

3. Howze and Hill (Case 1) ...... 20 days

M

Mass

Left

Multicystic: Minute renal vessel at upper pole. Small ureter but no definite renal pelvis or calyces. No normal renal tissue

Pain RLQ hematuria

Right

Multicystic: No renal artery or pelvis noted. No functioning renal tissue Multilocular: No renal pelvis, ureter or calyces. No urogram and no histological report given

4. Howze and Hill (Case 2) ...... 13 yrs.

M (Negro)

5. Dakin ..........

2 yrs.

F

Pain and mass

Left

6. Campbell (Autopsy Case) ..

4 wks.

M

History not reported

Right

7. Burrell .........

3 yrs.

M

Mass and hematuria

Left

M

Mass

Left

8. Toulson and Wagner ....... 19 mos.

Multilocular: No pelvis or calyces; ureter impervious. No histological report given. Left kidney normal Multilocular: Renal pedicle normal. Island of cysts at lower pole; surrounded by functionbg renal tissue Renal Multilocular: pedicle normal. Cysts circumscribed by functioning renal tissue

to be the origin of renal cysts. Virchow thinks cystic formation results from obstruction of the papillary ducts by urinary salts or inflammation, but Patterson

UNILATERAL MULTIPLE CYSTS OF KIDNEY

189

has shown the obstruction results in tubular atrophy. Hinman and Morison found that fetal renal circulation persists in the polycystic kidney and that the finer vessels of the cortex are compressed and displaced. Hepler notes that interference with the intraparenchymal blood supply leads to cystic degeneration in the involved area. He later produced a large solitary cyst of the kidney by ligating the posterior branch of the renal artery and simultaneously fulgurating the papilla of a rabbit's one lobed kidney. His experimental data offer an insight to the understanding of the pathological observations in these cases of renal cysts in children. The location, degree and duration of the disturbance with the renal blood supply seem to govern the extent of renal tissue degeneration. In the 4 multicystic cases, normal renal tissue was inadequate or noticably absent. In the multilocular cystic case of Toulson and ·wagner and that of Burrell, there ·was intrarenal vascular compression, but, functioning renal tissue was present as evidenced by excretory urograms. No histological study was reported in Dakin's and Campbell's cases. In the case of our 13 year old Negro boy, the kidney was found in an ectopic position and no true renal artery was found. Functioning renal tissue was apparently inadaquate as shown by its failure to excrete dye in the urogram. The presence of a renal cystic mass at birth in these infant cases proves their congenital origin. The fetal arrest in the development of the renal artery and pelvis, as evidenced by the pathological findings in many of the operative specimens, seems to influence the formation of cysts and the diminution of the functioning renal tissue. CASE REPORTS

Case 1. J.P., an infant white boy aged 14 days, entered Childrens Hospital May 13, 1947. A routine post-delivery examination revealed a mass in the left side of the abdomen, and the absence of the left testicle from the scrotum. No other abnormality was found. There was a history of a normal delivery. This was the first child and there were no miscarriages. Physical examination showed a well developed, bottle fed infant, weighing 8½ pounds. He was apparently not acutely ill. The head, neck and chest presented no abnormalities. The abdomen was soft and distended. There was a globular, nontender, slightly movable mass filling the entire left side of the abdomen. The liver was palpated two finger breadths below the costal margin. A plain film of the abdomen showed the presence of a soft tissue mass occupying the left flank and displacing the stomach upwards and the intestines to the right. The mass appeared to be the size of an orange. Excretory urograms, after subcutaneous injection of diodrast, revealed the presence of dye in the right kidney but none on the left. After 10 minutes the calyces and pelvis on the right ·were faintly outlined and appeared normal. At the end of 30 minutes the right kidney was emptied but still no dye Yrns seen on the left. The bladder appeared normal in size and contour. A gastro-intestinal x-ray study was reported normal. Urine: clear; specific gravity not (sufficient quantity); reaction, acid; albumin

190

CHARLES P. HOWZE AND JOHN H. HILL

and sugar, negative; microscopic: 6-10 pus cells per high power field. Red cell count: 5,500,000; white cell count 7,400; hemoglobin 17.5 gm. The possibility of a Wilms' tumor was considered and surgical exploration was advised. A transverse incision was employed, beginning 2 cm. above the umbilicus and extending to the left lumbar region midway between the lower ribs and iliac crest. The peritoneum was opened and the right kidney was palpated and found normal in size and free of cysts on its surface. The intestines and stomach were retracted to the right. Behind the posterior peritoneum was a multicystic mass extending from under the diaphragm to the pelvic brim. The posterior peritoneum was

Fm. 1. Multiple cysts replacing kidney.

A, testicle; B, ureter

incised over the cystic tumor. The tumor consisted of multiple cysts varying in size from a golf-ball to a small grape. No renal tissue was apparent. The mass was freed from the surrounding tissue and found attached to a normal size ureter. On the lateral border of the mass was found the left testicle, which was removed with the tumor after severing ligaments at each pole of the testicle. Only one small vessel was ligated near the upper portion of the cystic mass. The wound was closed in the conventional manner. Postoperative course was uneventful and the baby left the hospital 2 weeks following operation (fig. 1). The specimen consisted of a transparent multicystic mass measuring 18 by 7 by 6 cm. There was no apparent functional renal tissue present. Five centimeters of the ureter was attached to the specimen. Aspiration of the cysts showed a golden clear fluid. The wall of the cysts was about 1 mm. in thickness. There was a small piece of testicular tissue present. The position and ligamentous attachments of the testicle are embryologically understood. In the

UNILATERAL MULTIPLE CYSTS OF KIDNEY

191

human embryo the gonadal ridge is on the ventral border of the mesonephros. The evanescent mesonephric tubules later become the diaphragmatic ligament. This ligament and the inguinal ligament were found attached to the upper and lower poles of the testicle respectively. Sections of the cysts showed a connective tissue wall lined by a thin layer of cuboidal to flat epithelial cells, sharply defined from sub-epithelial structures. Scattered blood vessels were also seen. There was no evidence of malignancy in this specimen. Sections of the testicle consisted of well differentiated, rather congested and atrophic testicular tissue. There was nothing suggestive of neoplasm or any other specific pathological process. Diagnosis: Congenital multicystic kidney; atrophic testicle. Case 2. C. M., a Negro boy, aged 13 years, was admitted to Garfield Memorial Hospital June 19, 1940, complaining of fever, chills and sweats for a period of 3 weeks. There had been some pain in the lower right quadrant, but he had had no nausea or vomiting. The past history disclosed hematuria on several occasions not accompanied by other urinary signs or symptoms. Physical examination revealed a well nourished, well developed Negro boy, apparently ill. There was tenderness in the lower right quadrant of the abdomen, associated with some rebound tenderness. No mass or muscle splinting was noted. A mitral murmur and accentuated pulmonic second sound were found on heart examination. The blood pressure was 120 systolic and 62 diastolic. The external genitalia and rectal examinations were normal. The urine was normal except for a trace of albumin. Blood urea: 32 mg. Red blood count 3,420,000; white blood count 6,050; hemoglobin 57 per cent. The blood calcium was 10 mg. and the inorganic phosphorus 2.7 mg. per 100 cc. The Wassermann and Kahn reactions were negative. Blood smears for malaria were positive for Plasmodium vivax on 2 occasions. The phenolsulphonpthalein was excreted in the following concentration: after 15 minutes, 50 per cent; 30 minutes, 9 per cent; 60 minutes 12.5 per cent; total 71.5 per cent. A flat film of the urinary tract revealed both psoas outlines to be fairly distinct. There were no shadows suggestive of urinary calculi. The left renal shadow was identified but the right ,vas not seen. Intravenous pyelograms revealed a normal left kidney pelvis. X-ray exposures at 3, 5, 5½ and 7 hours failed to show any dye excreted by the right kidney. Cystoscopy was performed to determine the cause of hematuria. The bladder was normal and the ureteral orifices were normal in size, shape and position. A No. 5 ureteral catheter was passed easily to the left kidney and normal urine was obtained. On the right side the catheter was obstructed at the 10 cm. marking. No urine was obtained from this side. A left retrograde pyelogram was made and found to be normal. The right catheter was injected with skiodan, which regurgitated into the bladder without delineating a right renal pelvis (fig. 2). Operation was performed through the usual lumbar incision. There was no kidney in the lumbar fossa. After considerable search, a small multicystic mass, about the size of small oyster, was found in the iliac fossa. It was loosely attached to the peritoneum. No definite renal pedicle was apparent. The cystic mass

192

CHARLES P. HOWZE AND JOHN H. HILL

Fm. 2. Normal left pyelogram. Right catheter obstructed at 10 cm.

fl <",,

U,Usl 1111 I I! j 11 l1j I! l II 11 !111 j l Il ":,_V

FIG. 3. Multiple cysts of congenital atrophic kidney.

A, Ureter

was attached to a small ureter which -was divided and ligated. The wound was closed in layers. The postoperative course was uneventful and the patient was discharged from the hospital in 12 days following operation. The specimen consisted of a cystic mass weighing 18.5 gm. and the portion of the ureter measuring 2 cm. The capsule appeared injected and showed beneath

UNILATERAL MULTIPLE CYSTS OF KIDNEY

193

its surface numerous cysts, averaging 5 mm. in diameter. A small amount of perirenal fat was attached (fig. 3). The sections of the kidney showed, between the cystic areas, dense fibrous tissue and thick walled blood vessels and on occasional tubule occurring singularly and in small groups. In some instances they contained homogenous pink staining material. Calcium granules were occasionally seen. Diagnosis: Multicystic kidney with fibrosis. CONCLUSIONS

One case of unilateral multicystic kidney in a 20 day old boy and 1 case of unilateral multicystic kidney in a 13 year old boy are presented. Similar cases collected from the literature are reviewed. The clinicopathological finding of absence or incomplete development of renal artery and pelvis in children, concomitant with retardation of the renal tissue growth and cyst formation, lends added significance to Hepler's laboratory experiments. 1150 Connecticut Ave., Washington, D. C. REFERENCES BRAASCH, W. F. AND HENDRICK, J. A.: J. Urol., 51: 1-10, 1944. BURRELL, N. L.: J. Urol., 43: 656-663, 1940. CAMPBELL, M.: Pediatric Urology. New York: Macmillan, 1937, vol. 1, p. 205. DAKIN, W. A.: Canad. Med. Assoc. J., 42: 531-533, 1940. HEPLER, A. B.: Surg., Gynec. & Obst., 53: 688, 1930. - : J. Urol., 44: 206, 1940. HILDEBRANT: Arch. klin. Chir., 48: 343, 1894. HINMAN, F. AND MomsoN, D. M.: J. Urol., 11: 131, 1924. KAMPMEIER, V. F.: Surg., Gynec. & Obst., 36: 208-216, 1923. LYNCH, K. D. AND THOMPSON, R. F.: J. Urol., 38: 58-60, 1937. MELAND, E. L. AND BRAASCH, W. F.: J. Urol., 20: 505-519, 1933. PATTERSON: Beitr. z. allg. Path., 33: 606, 1903. SCHWARTZ, J.: J. Urol., 35: 259-263, 1936. SrnKoE, S. J.: J. Med. Assoc. Ga., 34: 171-178, 1945. TouLsON, W. H. AND WAGNER, J. H.: Bull. School of Med. Univ. Md., 25-26: 177-184, 1940-42. VrncHO'"~ R.: Ges. Abhandl. z. Wissensch. Med., 1856, p. 864.