PYELITIS CYSTICA AND URETERITIS CYSTICA REPORT OF A CASE DIAGNOSED BY UROGRAPHY AND CON-FIRMED BY I3IOPSY, WITH AN OUTLINE OF TREATMENT LLOYD KINDALL Oakland, California
Cysts of the mucous membrane of the upper urinary tract have long been known to the pathologist, Morgagni having reported the first case in 1761. The cystoscopist occasionally sees cystitis cystica-easily confused with bullous edema-but pyelitis cystica and ureteritis cystica represent practically unknown lesions to the clinician. Only 2 cases, diagnosed from urographic evidence, have been recorded-Jacoby and Joelson each having reported a case. The lesion is usually associated with a chronic inflammatory condition of the urinary tract and the presence of renal calculi is rather common. Several theories as to the origin of cysts have been advanced_: (1) The cysts are parasitic in origin; (2) they are retention cysts derived from preexisting glands in the pelvis, ureteral or vesical mucosa; (3) they originate from the cell "nests" of Bon Brunn, which are probably inflammatory in origin. The last is the most popular explanation. CASE
REPORT-PYELITIS
CYSTICA
AND
URETERITIS
CYSTIC.A,
DIAGNOSED BY UROGRAPHY AND CONFIRMED AT OPERATION ON A PATIENT EXAMINED BECAUSE OF RIGHT RENAL COLIC
L. K., aged forty-one, entered Highland Hospital on July 22, 1932, complaining of pain in the right kidney region, and gave a history of having passed a small stone. The pain was colicky in character, .radiating down to the scrotum and accompanied by nausea,_ vomiting, chills and a leukocytosis of 34,000. Twenty-four hours after admiss_ion his renal colic ceased, but a dull aching pain in the region of the kidney 64.5 THE JOURNAL OF UROLOGY, VOL. XXIXr NO.
6
646
LLOYD KINDALL
persisted. For five days he had afternoon elevations of temperature with morning remissions, and then his temperature remained normal. There was a history of fever and hematuria at the age of eight while living in Greece, and a constant pyuria since that time. He had hematuria as a complication of influenza in 1918, and this was present at intervals for the ensuing two years. In 1931, because of upper abdominal distress and jaundice, a cholecystgastrostomy was done at Highland Hospital. The common duct was patulous and there were no cysts in the gall bladder. The head of the pancreas was enlarged and the surgeon was in doubt as to whether this condition was due to a malignancy or an acute pancreatitis, but the subsequent course proved that the condition was inflammatory. The phthalein output was 57 per cent in two hours. A guinea pig was inoculated with a sample of twenty-four-hour urine and was negative for tuberculosis. The blood Wassermann was negative. The urea nitrogen, 15 mgm. per cent per 100 cc. Blood count: red blood cells, 4,670,000; hemoglobin, 71 per cent; white blood cells, 9700; polymorphonuclears, 66 per cent; lymphocytes, 32 per cent; monocytes, 2 per cent; slight poikilocytosis; achromia, and an occasional stippled cell. Cystoscopy. July 30, 1932. A No. 24 F. Brown-Buerger anterior cystoscope was passed without difficulty or discomfort to the patient. There was no residual urine and the bladder capacity was normal. No stones, tumors or other foreign bodies were seen. The bladder wall was not trabeculated. The mucous membrane was normal. The trigone was not hypertrophied, and there were no cysts or bullae seen. There was some redness with edema about the right ureteral orifice. No. 6 catheters passed readily into both kidneys, and the urine dripped normally from each. (It is interesting to note that the dull pain in the right kidney region was completely relieved by the passage of the catheter.) The laboratory reported that the bladder urine contained bacteria, 20 red blood cells, and 30 pus cells to the high dry field; the right kidney urine had 2 red blood cells and 80 pus cells to the high dry field; and in the left kidney urine were seen bacteria, 100 red blood cells and 30 pus cells to the high dry field. Indigocarmine was excreted from both kidneys in four minutes, the density of the dye being normal. The plain x-ray pictures showed a small shadow, which was thought to be a calculus in the lower calyx of the right kidney, but which proved to be cysts (pyelitis cystica). The ureterograms showed a bubbly or frothy appearance of both ureters that extended throughout their entire length. There were many small rounded rarefied areas due to the dis-
PYELITIS CYSTICA AND URETERITIS CYSTICA
647
placement of the opaque media by some non-opaque substance in the ureter (figs. 1, 2, 3 and 4).
FIG. 1. U RETERITIS
CYSTICA
Ureterograms appear bubbly or frothy throughout entire length, due to the displacement of the 13.5 per cent sodium iodide by the cysts in the ureters.
Operation. On September 7, 1932, under gas oxygen-ether anesthesia, the right kidney and ureter were exposed through the classical Young
648
LLOYD KINDALL
inc1s1on. There was no perinephritis or peri-ureteritis. The kidney appeared normal and the fatty capsules stripped easily. No calculi could be palpated in the kidney or pelvis. However, on deep palpation
FIG. 2. SAME AS FIG.
1
WITH CATHETERS REMOVED
The abnormal pelvic outlines are undoubtedly the result of inflammation
of both the pelvis and the ureter the sensation was not unlike that of feeling buckshot under the skin. There were only a few of these shot-like bodies in the pelvis, but many in the ureter.
PYELITIS CYSTICA AND URETERITIS CYSTICA
649
The ureter was opened longitudinally for about 2 cm. at the junction of the middle and upper third. The ureteral wall was somewhat thickened. The mucous membrane was not acutely inflamed and appeared
FIG. 3.
COMBINED INTRAVENOUS AND RETROGRADE PYELOURETEROGRAMS MADE A
Fmw
DAYS LATER
Note the complete filling and the sharp kidney outlines. The site of pyelitis cystica, at first thought to be a calculus, is indicated by the arrows.
normal in color. There were numerous round to ovoid cysts lying in the ureter, varying in diameter from 1 to 5 mm. They were not pedunculated. Some of the cysts were easily ruptured by the slightest manipu-
650
LLOYD KINDALL
lation and contained a yellowish-white mucoid substance that was of the consistency of mineral oil. A section of the wall with the cyst was removed for microscopical examination.
FIG. 4. PYELITIS CYSTICA
The patient made an uninterrupted recovery, and there was no urinary leakage from the operative incision. A pyelogram made one month later showed no evidence of obstruction or narrowing of the ureter at the site of the biopsy. The pathologist reported:
PYELITIS CYSTICA AND URETERITIS CYSTICA
651
"Microscopic examination shows a number of cysts which vary co.nsiderably in size, the largest of which completely fills a l9w power :fj.eld. The contents of the cyst is granular, hemogenous material containing a
Frn. 5.
Low PowER MAGNIFICATION
(100
TIMES).
FuLL
Vrnw OF
AVERAGE CYST
Note thin wall of cyst. Note the epithelial lining which is placed on an intact basement membrane. This cyst is lined by cuboidal epithelium. The surrounding tissue is composed of a reticular substance holding many lymphocytes, proliferating fibroblasts, large and small macrophages, and free red blood cells. The contents of this cyst consist of large oedematous epithelial cells disintegrated from the epithelial lining, macrophages, and cell detritus.
few leukocytes. The lining is smooth and made up of low cuboidal cells with relatively large nuclei which stain deeply. The outer layer is a fibrous connective tissue in narrow band& which are separated by lymphocytes. The blood supply is very free and the blood vessel walls are thin.
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LLOYD KINDALL
There is an accumulation of lymphocytes which project outward from the surface of the cyst in a broad mass which occupy about one-fifth of the circumference of the largest of these cavities. Dissection of the wall of the ureter shows the usual histologic structure without infiltration of any sort." (Dr. Gertrude Moore.) (Figs. 5, 6, 7, 8 and 9.)
Fm.
6.
HIGH POWER MAGNIFICATION OF FIGURE
5
This shows the cellular invasion of the surrounding structures due to inflammation and the cuboidal epithelium lining the cyst.
Inasmuch as many of the cysts ruptured easily at the time of operation, it occurred to us that prolonged dilatation with a large Blasucci catheter, followed by the injection of an astringent such as silver nitrate, would be efficacious in destroying the most prominent cysts. We considered using a Howard spiral stone remover as a curet; but, although it is theoretically the perfect instrument to destroy such pathology, we hesitated because of the possibility of untoward results.
PYELITIS CYSTICA AND URETERITIS CYSTICA
6.53
On October 17, 1932, a No. 10 Blasucci ureteral catheter was passed up the right ureter to the kidney pelvis and left in situ for three days. Immmediately before its removal, 10 cc. of 2 per cent silver nitrate solution was injected into the kidney pelvis and ureter. The catheter was then withdrawn. Four days later the same procedure was repeated on the left side.
Fm. 7. Low PowER
MAGNIFICATION
120 Tnrns
Some of the smaller and earlier cysts.· The cell type is of the low columnar and in some areas the high columnar epithelium. As these cysts enlarge in size they tend to develop a more flattened epithelium. The surrounding soft tissues undergo some compression atrophy, degeneration and subsequent inflammatory changes. These changes are due to a sterile inflammation.
Combined intravenous (Neo-Skiodan) and retrograde pyeloureterograms were made on October 28, 1932, and the cyst shadows had diminished in number (fig. 10). The total phthalein (two hours) on October 31, 1932, was 65 per cent.
654
LLOYD KINDALL
This case will be followed up with pyeloureterograms (combined intravenous and retrograde) every three months for a period of at least a year.
Fm.
8.
HIGHER MAGNIFICATION
(300
TIMES) oF RouND CYST IN FrnuRE
6
This shows a small young cyst lined by low columnar epithelium before disintegration of the lining has occurred. The surrounding tissues show new, budding capillaries, large numbers of early proliferating fibroblasts, lymphocytes, and endothelial macrophages. DISCUSSION
A study of the literature indicates that there are no characteristic symptoms of pyelitis cystica or ureteritis cystica. Diagnosis, based upon urography, has been made in only 2 reported cases, and in neither was it confirmed by biopsy. The report of
PYELITIS CYSTICA AND URETERITIS CYSTICA
655
the association of renal calculi with pyelitis cystica and ureteritis cystica is frequent. Many urograms in which shadows appear, that have generally been interpreted as air bubbles, should have further x-ray studies to determine, if possible, whether pyelitis cystica or ureteritis cystica is present. This patient had a dull
Fm. 9. Magnification 400 times. This shows a small cyst lined by high columnar epithelium. Disintegration of the epithelium has begun. The fibrous tissue surrounding the cyst is older and less reticular in arrangement than in the other sections seen.
aching pain in the right kidney region which was relieved immediately by the passage of a ureteral catheter; hence, it must have been due to the numerous cysts, causing back pressure and interference with the normal peristalsis. Because of the ease with which many of the cysts can be ruptured by the use of a large retention ureteral catheter, followed by the application of the
656
LLOYD KINDALL
astringent silver nitrate, it is suggested that it be tried as a therapeutic measure, since it is harmless and is a recognized treatment for the relief of hydronephrosis. In the past, since these cases have not been recognized, no treatment has been suggested.
Fm. 10. Following treatment number of cysts decreased. Combined intravenous and retrograde pyeloureterograms made after No. 10 Blasucci catheters had been left in each ureter for three days and 2 per cent silver nitrate then injected. The removing of the upper third of the right ureter is the result of the biopsy. The right lower calyx is occupied by three cysts as indicated by the arrows.
PYELITIS CYSTICA AND URETERITIS CYSTICA
657
CONCLUSIONS
1. A case of pyelitis cystica and ureteritis cystica, diagnosed by urography and confirmed at operation, and, so far as can be determined by a study of the literature, the first case conclusively diagnosed, i:;; reported in detail. 2. Cystoscopy was of no diagnostic value, since there was no cystitis cystica present. 3. Combined intravenous (Neo-Skiodan) and bilateral, retrograde pyeloureterograms were made throughout the study. 4. The dilated ureters were due to obstruction of the lumen of the ureters by the cysts, mechanically causing back pressure or interfering with the functional peristalsis of the ureter. 5. A method of treatment is suggested: Large ureteral catheters passed to the kidney pelvis and left in situ for several days mechanically rupture many cysts, and the injection of silver nitrate solution at the time of removal, by its astringent action, destroys other traumatized cysts.
I desire to express my appreciation to Dr. Miley B. Wesson for his many helpful suggestions throughout this study and in the preparation of the manuscript. 400 Twenty-ninth Street Oakland, Calif. REFERENCES ALBARRAN, JOAQUIN: Maladies de la Prostate. 1902, 526. ALBERSLOETTER, H.: tJ·ber die Pathogenese der Ureteritis cystica. Muncher, 1909. AscHOFF, LUDWIG: Ein Beitrag zur normalen and pathologischen Anatomie der Schleimhaut der Harnwege und ihrer drusigen Anhange. Virchows Arch. f. path. Anat., 1894, cxxxviii, 119. AuGIER, D., AND LEPOUTRE, C.: Etude d'un cas d'ureterite kystique. Ann. d. rnal. d. org. genito-urin., 1911, xxix, 880. BLAND-SUTTON, J.: Psorospermiae in ureter. Brit. Med. Jour., 1889, ii, 1392. BLAND-SUTTON, J.: On parasiticisrn by psorospermia. Lancet, 1889, ii, 1278. voN BRUNN, A.: ti'ber drusenachnliche Bildungen in der Schleirnhaut des Nierenbeckens des Ureters und der Harnblase beim Menschen. Arch. f. rnikr. Anat., 1893, xli, 294. CARSON, W. J.: Pyelitis, ureteritis and cystitis cystica. Arner. Jour. Surg., June, 1930, viii, 1256-58.
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CAULK, JoHN R., AND GRUBER, CHARLES M.: Nelson Looseleaf Surgery, Vol. VI (1932), fig. 55, page 771E. CLARK, J. J.: Mucous cysts of ureter. Brit. Med. Jour., 1892, i, 274. CLARK, J. J.: A case of psorospermial cysts of the left kidney and ureter, and of the bladder, with hydronephrosis of the left kidney. Tr. Path. Soc. London, 1892, xliii, 94. D' AJuToLo, D. G.: Ueber Uretheritis chronica cystica. Centralbl. f. allg. Pathol. u. path. Anat., 1890, i, 266. DoGIEL, A. S.: Zur frage uber das Epithel der Harnblase. Arch. f. mikr. Anat., 1890, xxxv, 389. EBSTEIN, W.: Zur Lehre von den chronischen Katarrhen der Schleimhaut der Harnwege und der Cystenbildung in derselben. Deutsches Arch. f. klin. Med., 1882, xxxi, 63. EGLI, T.: Ueber die Drusen des Nierenbeckens. Arch . .f. Mikr. Anat., 1873, ix, 653. EvE, F. S.: Psorospermial cysts of both ureters. Tr. Path. Soc. London, 1889, xl, 444. GIANI, R.: Neuer experimentellen Beitrage zur Entstehung der Cystitis cystica. Beitr. z. path. Anat. u. z. allg. Pathol., 1907, xlii, I. GILCHRIST, T. C.: The so-called protozoa occurring in psorospermosis follicularis vegetans (Darier). Rep. Johns Hopkins Hosp., 1896, i, 291. HAMBURGER, A. 0.: Zur Histologie des Nierenbeckens und des harnleiters. Arch. f. mikr. Anat., 1879, xvii, 14. HARRIS, H.: Cysts of the ureter. Amer. Med., 1902, iii, 731. HERXHEIMER, GoTTHOLD: Ueber Cystenbildungen der Niere und abfuhrenden Harnwege. Virchows Arch. f. path. Anat., 1906, clxxxv, 52. HIBBS, W. G.: Hyperplastic pyelitis and ureteritis cystica. Tr. Chicago Path. Soc., 1919, xi, 49. JACOBSON, V. C.: Pyelitis et ureteritis et cystitis cystica. Bull. Johns Hopkins Hosp., 1920, xxxi, 122. JACOBY, M.: Ureteritis cystica; case. Zeitschrift fur Urologie, 1929, xxiii, 722-23. J oELSoN, J. J.: Pyelitis, ureteritis and cystitis cystica. Arch. Surg., April, 1929, xviii, 1570-1583. JOHNSON: Quoted by Jacobson, V. C. VON KAHLDEN, C.: Ueber Ureteritis cystica. Beitr. z. path. Anat. u. z. allg. Pathol., 1895, xvii, 562. LIEBREJCH, A.: Ein Fall von Ureteritis cystica. Tubingen, 1906. VON LIMBECK, R.: Zur Kenntniss der Epithelcysten der Harnblase und der Ureteren. Ztschr. f. Heilk., 1887, viii, 55. LITTEN, M.: Ureteritis chronica cystica polyposa nebst cystischer, Degeneration der Niere. Virchows Arch. f. path. Anat., 1876, lxvi, 139. LowsLEY, 0. S.: Observations on certain obstructions at the vesical orifice. Jour. Amer. Med. Assoc., 1917; lxviii, 444. LuBARSCH, 0.: Ueber Cysten der albeitenden Harnwege. Arch. f. mikr. Anat., 1893, xli, 303. MARCHWALD: Die multiple Cystenbildung in den Ureteren und der Harnblase, sog. Ureteritis cystica. Munchen. med. Wchnschr., 1898, xlv, 104.9.
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MoRGAGNI, J. B.: De sedibus et causis morborum per anatomen indagatis libri quinque. William Cooke Translation, London, 1822, ii, 316, 411. MORRIS, H.: Surgical Diseases of the Kidney and Ureter. London, 1901, ii, 480. MoTz, B., AND CARIANI, A.: Contribution a l'etude des adenomes kystiques de l'appareil uninaire. Ann. d. mal. d. org. genito-urin., 1904, xxii, 1305. ·OSLER, WILLIAM: Psorospermiasis. The Principles and Practice of Medicine. New York, 1920, Ed. 9,336. P ASCHKIS, R.: Pyonephrosis occurring with cystic ureteritis, case. Zeitschrift furUrologie, 1929, xxviii, 64-73. PISENTI, G.: Ueber die parasitische N atur der Ureteritis chronica cystica. Central bl. f. allg. Pathol. u. path. Anat., 1894, v, 657. RADTKE, ERICH: Beitrage zur Kenntniss der U reteritis cystic a. Konisberg, 1900. RAYER, P. F.: Traite des Maladies des Reins. Paris, 1837, Atlas, Plate lii, Figs. 2, 3, and 4. RoKITANSKY, CARL: Manual of Pathological Anatomy. London, 1861, Ed. 2, 216. SAL'I'YKOW, S.: Epithelveranderungen der albeitenden Harnwege bei Entzundung. Beitr. z. path. Anat. u. z. allg. Pathol., 1908, xliv, 393. SATANI, Y.: Histologic study of the ureter. Ural., 1919, iii, 247. SILCOCK, A. Q.: Case of vesiculation of the mucous membrane of the bladder. Tr. PathoL Soc. London, 1888-1889, xl, 175. STOERK, OsKAR: Beitrage zur Pathologie der Schleimhaut der harnleitenden Wege. Beitr, z. path. Anat. u. z, allg. Pathol., 1899, xxvi, 367. STOERK, 0., AND ZucKERKANDL, 0.: Ueber Cystitis glandularis und den Drusenkrebs der Harnblase. Ztschr. f. Ural., 1907, i, 3, 133. STOW, B.: Ureteritis cystic a chronic a. Ann. Surg., 1907, xl vi, 233. URQUHART: Cyst formation in ureter associa.ted with Bilharziasis. Brit. Jour. Surg., 1931, iii, 21-25. VoELCKER, ARTHUR: Chronic interstitial nephritis with cysts in the renal pelvis, double ureter and malformation of the bladder. Tr. Path. Soc. London, 1898, xlix, 168. VrncHow, RuDOLPH: Die krankhaften Geschwulste. Berlin, 1863, Ed. I, 246. YOUNG, HUGH H.: Practice of Urology. Philadelphia, W. B . Saunders Co., 1926, Vol. I, page 191.