Vol. 99, Feb, Printed in U,8. A ,
THE JOUR:'{A.L OF UROLOGY
Copyright @ 1968 by The Williams &-Wilkins Co.
THE DIAGNOSTIC PROBLKH OF THE HYDATID DISEASE OF THE KIDNEY A. DELIVELIOTIS, P. KEHAYAS
AND
M. VARKARAKIS
Frorn the S1lrgical Department of Urology, Evangelismos Hospital, A. thens, Greece
Echinococcosis of the kidney is not a common disease, but it occurs in patients in any part of the world because of increasing numbers of travelers and emigrants.' 'IVe wish to discuss the diagnostic problems of hydatid disease of the kidney, based on an experience of 12 personal cases. MATERIAL
From January 1, 1961 to November 22, 1966, and among 2,011 operations on the kidney, 12 patients with hydatid cysts of the kidney underwent operation in our Department of Urology. The sex and age and the side involved have been tabulated in the table. DISCUSSION
The case history is valuable in making the diagnosis especially when a discharge of daughter cysts or parts of membrane is clearly reported to have occurred with urination. Of course, this discharge may come from. echinococcosis of the retrocystic area or of the bladder. The discharge of hydatic elements from the kidney, usually accompanied by colic, was observed in 2 cases. One of our patients (ca~e 6) had had occasional attacks of colic in the left kidney and turbid urine without hematuria. During the 2 years prior to admission he had noticed pieces resembling the skin of a grape in his urine. His left kidney was not palpable. The white blood count (WBC) was 10,000; eosinophils, 3 per cent. Other laboratory studies were within normal limits. A plain film of the kidneys, ureters and bladder (KUB) showed a homogeneous round shadow in the lower pole of the left kidney, and excretory urography revealed a non-functioning kidney (see fig. 7, B). The Weinberg and the Casoni tests were positive (4 plus). At operation threequarters of the left kidney was found to be occupied by a hydatid cyst, which originated in the
lower pole and communicated freely with the lower calyx. Another patient 8) had experienced attacks of left renal colic within a 5-month period. During the third attack some daughter cyst.s were discharged in the urine. After hospita.lization the patient again had an attack of colic and passed an intact daughter cyc;t with the urine. The kidney was not palpable. The sedimentation rate was 45 mm.; vVBC, 8,000 and eosinophils, 2 per cent. Other laboratory studies were within normal limits. An x-ray showed calcification in the left kidney (see fig. 4, B) and excretory urography revealed a non-functioning kidney. A retrograde pyelogram was made (see fig. 8, C). At operation an hydatid cyst was found in the lower half of the anterior surface of the kidney. The cyst communicated freely with the draining part of the kidney. A daughter cyst plugged the initial part of the ureter (fig. 1). Besides the positive evidence of hydatiduria, the physician should direct his attention to rennl echinococcosis when the patient reports localiza. tion of the disease elsewhere; this applies in instances of secondary involvement. 2 The history did not yield any positive information in our other cases. Hydatid disease of the kidney may evolve without symptoms for several years. Nevertheless, it can cause a great of symptoms such as pain in the lumbar region, renal colic, hematuria and frequency of mitturi tion (which occurs when a cyst cornnmnicates with the excretory passages), m dyspnea (due to pressure on an intra-abdomi nal organ or the diaphragm), and finally arm phylaxis without evident cause. 3 · 4 In our case 12 the patient was hospitalized in the medical clinic for unexplained anaphylactiu phenomena (urticaria). A thorough investigation was of no avail but an excretory nrogram re-
Accepted for publication January 19, 1967. 1 Henry, J. D., Utz, D. C., Hahn, R. G., Thompson, J. H., JI'. and Stilwell, G. G .. Echinococcal disease of the kidney: Report of case . .J. Urol., 96: 431, 1966.
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2 Kirkland, K.: Urological aspects of hydatid disease. Brit. J. Urol., 38: 241, 1966. 3 Baurys, vY.: Echinococcns disease of the kidney. J. UroL, 68: 441, 1952. 4 Begg, R. C.: Hydatid uisease of kidney. Bri L J. Surg., 24: 18, 1936.
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DELIVELIOTIS, KEHAYAS AND VARKARAKIS
Review of cases Patient No.
1 2 3 4 5 6
7 8 9 10 11 12
Sex
F F M M M M M F
F M F F
Age (yrs.)
52 23 15 35 60 38 30 18 45 48 62 35
Kidney EosinInfected ophils (%)
Lt. Rt. Rt. Rt. Lt. Lt.
Lt. Lt.
Rt. Lt. Lt.
Lt.
Weinberg Test
Casoni Test
0 2 12
++++
+
No
No
+
+
7
No
No
4 3 4 2 2 4 3 0
++++ ++ ++++ ++++ ++++ No
+ + + + + + + +
vealed some calcification in the lower pole of the left kidney (see fig. 4, D), which was functionally silent (see fig. 7, C). For that reason the patient was transferred to our department. Palpation of the left kidney was not helpful and no eosinophils were found. However, the combination of calcifications, a positive Casoni's test and the findings on retrograde pyelography (fig. 2) led to the diagnosis of hydatid disease of the kidney. At operation a big hydatid cyst of the lower pole was found communicating with the excretory passages. A nephrectomy was performed. Physical examination is of little help in making the diagnosis. The presence of a constant swelling in the renal area, usually tensive and rather mobile, will raise the question of echinococcosis only as a last thought, as more common spaceoccupying lesions can be incriminated. If the mass is large, the attention of the examiner is directed to intra-abdominal organs rather than to the kidney. Hydatic thrill is seldom found. 4 The laboratory data are important in making the diagnosis. Eosinophilia is not specific as it can be found in other parasitic and anaphylactic conditions (asthmatic bronchitis, eczema, etc.); it can, however, direct attention toward echinococcosis. In our patients the eosinophil count exceeded 3 per cent in 5 instances. The complement fixation test (Weinberg) and the intradermal Casoni test are useful in making the diagnosis even though they are not always positive nor is a positive reaction definite proof of a hydatid cyst. In our series the Weinberg test was positive in 8 of 9 cases. The Casoni test was positive in all 10 cases in which it was performed (see table).
Fm. 1. Case 8
Radiologic examinations provide more positive information in making the diagnosis. A simple radiogram of the kidneys, if calcifications do not exist, offers very little assistance. A round, homogeneous shadow with regular contour is usually seen. The similarity of this picture to other more common space-occupying lesions of the kidney, and especially the solitary cyst, rarely directs one's attention toward echinococcosis. No calcification was present in 4 of our cases. In case 1 a uniform swelling of the whole kidney was observed (fig. 3, A), while in case 4 a round shadow was found in the lower pole of the kidney (fig. 3, B). In one case a round shadow was observed in the lower pole of the left kidney while in another case no lesion could be demonstrated in the renal shadow. If calcification is present in the plain film, echinococcosis should be suspected. In our series calcification was seen in 7 cases. Figure 4 shows several types of calcifications in echinococcosis of the kidney. One should remember that neoplasms of the kidney,2· 5 polycystic disease 2 and pyonephrosis can cause similar calcifications. Figure 5 is a plain film (KUB) of a patient who was hos5 Simpson, W.: Curvilinear calcification renal carcinomata. Brit. J. Urol., 38: 129, 19fi6.
111
HYDATID DISEASE OF KIDNEY
pitalized because of high temperature and pain in the left lumbar region. A left nephrectomy was done because of pyonephrosis and the diagnosis was proven by histologic examination. On the
FIG. 2. Case 12
Bl
other hand, calcification in t.he form of in cases of echinococcosis of the kidney may lead to an erroneous diagnosis of lithiasis. One of our patients (case 2) had had occasional c'"·''·"·'"'' of fever and pain at the right costal for approximately a year. She entered the medica,l clinic with a probable diagnosis of A.n investigation did not demonstrate in the biliary tract. The shadow.~ in the film were taken for stones of the renal of one calyx (fig. 6, A). This diagnosi.~ 1rns not altered even by excretory urography (fig. G, B). vVBC was 8,000; eosinophils, 2 per cent J\fore specific examinations were not as echinococcosis was not included in the differential diagnosis. The patient was transferred to the Department of Urology. On au datid cyst was found in the lower half of the kidney without any communication t.o the drainage system. Deformities of the pclvicalycca1 system due to one or more hydatid cysts are revealed by excretory urography. In ma11y ca~cs, however, lesions of the parenchyma an, far advanc<~d so that excretory urography ~hows non-functioning kidney. In 8 of our 12 c:ascs, echinococcosis had caused functional sileuce of the kidney affected (see figures 7 and these cases retrograde pyelography useful. It should be performed under strict
Fm. 3. A, plain film (KUB) in case l reveals swelling of left kidney with elimination of psoas sha.dO\v B, plain film (KUB) of case 4 reveals round shadow on lower pole of right kidney.
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DELIVELIOTIS, KEHAYAS AND VARKARAKIS
Fm. 4. Calcifications on plain film (KUB) due to echinococcosis of kidney. A, case 5. B, case 8. C, case 11. D, case 12.
conditions as it can lead to infection of an open hydatid cyst. In case 4 the patient had colic in the right kidney and was studied elsewhere a n1onth before admission to our hospital. An excretory urogram showed no delineation of the affected kidney. Following retrograde pyelography it was decided that the right kidney should be explored. The tentative diagnosis was hydronephrosis. The patient refused operation. The
day after he left the hospital he returned with high temperature (39C), rigor, vomiting and general malaise. Antibiotics were of no avail and finally the patient was admitted to our department. A round swelling was palpated in the lower pole of the right kidney; it was rather elastic but very painful. The hematocrit was 25, WBC, 19,000; eosinophils, 7 per cent; sedimentation rate, 15 mm. and blood urea level, 50 mg. per cent.
HYDATID DISEASE OF KIDNEY
A plain x-ray of the kidneys is depicted in figure 3, B and an exeretory urogram in figme 7, il. Since the patient's general condition was poor and the circulatory s_vstcm had already been affected by infection (tachycardia, falling blood pressure), an exploratory operation wa.s
Fm. 5
14:3
done immediately. /\ hydatid cyst was found in the lower pole with a fistulous communication Lo the excretory passages which was infected when the retrograde mack. Even in those ca.,es in whieh die excretory capacity is retained, the deformity of calyceal system does not appear so clear retrograde pyelography (see fig. 91. The defurrnities of the pelvicalyceal system, a~ demon.,trakd by retrograde pyelography, have beni scl1ematically classified by some authors. 7 The mo.st. characteristic forms are l) the bell of the stairs, 2) the soap bubble in casec of a closed cyst localized in the superior pole, 3) th,, ccescent form in cases of middle localizatioE., 4) the in,. terrogation point form in the lower Joeali· zation or 5) the form of the prey bird ,,mb independently of localization. In c1n open cyst and in a case of infiltration of the opaque rneclium. through many daughter cysts, ihe form of a bunch of grapes is described, and in cases of half-closed cavity, the form of a CU[). These forms depend to a great extent on the surgeon's imagination. They may also rc~nlt other tumoral processes of the kiclney. 2 On the other hand, deformities or Lhe calyceal system clue to cchinocoecosis arc nol; 6 Surraco, L. A. and J\Iezzera, H.: Ei tico pielografico de! quiste hidatico del signo de la copa. Rev. med. latino-am., 16: 145'.\ 1931. 7 Imbert, JU.: L'llfetero-pyelographie clans Jes kystes hydatiques du rein. J. d'Urol, 54: 1"l3, 1948.
Fw. 6. Case 2. A, plain film (KUB). B, excretory urogram
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DELIVELIOTIS, KEHAYAS AND VARKARAKIS
Frn. 7. Excretory urography reveals functional silence of kidney, due to echinococcosis. A, case 4. B, case 6. C, case 12.
Fm. 8. Retrograde pyelograms in cases of renal echinococcosis. A, case 1. B, case 7. C, case 8 always so typical and, frequently, resemble those produced by 1nalignant tumors, tuberculosis, etc. In case 1 the patient had had periodic pain in the left lumbar region, frequency of micturition and turbid urine for a year. Palpation of the swollen left kidney revealed it to be hard, painless and movable. The hematocrit was 38; sedimentation rate, 23 mm.; WBC, 10,000; eosinophils, 0.
A plain film (KUB) showed a swelling of the left kidney (fig. 3, A). On excretory urography the right kidney was found to be normal; the left deviated towards the midline with an elongation of the superior calyx and partly of the middle one, as well as broadening of the renal pelvis. The picture was rather obscure but a retrograde pyelogram clearly showed the deformities and directed suspicion to a neoplasm of the kidney
HYDATJD DlSEASE 01<' KIDNEY
FrG. 9. Case \l. A, exerelory urogram. B, retrograde pyelogrnm. C, removed cysL. D, excret,nr:y gram 1 month after regeneration .
.Fm. 10. Case 10. A, plain film (KUB). B, excretory urogram. C, retrograde pyelogra.m. D, rcm1ov<-:d kidney. Cyst in upper pole i.s opened and daught.er cysts are seen.
146
DELIVELIO'I'IS, KEHAYAS AND VARKARAKIS
•
FIG. 11. Case 10. Renal scan
(fig. 8, A). Even though the patient's general condition was good and the sedimentation rate relatively low, the fact that she was from a rural region infected with echinococcosis induced us to have the Weinberg and the Casoni tests performed. Since both were positive, the tentative diagnosis was hydatid cyst of the left kidney. At operation the kidney was almost totally occupied by a huge hydatid cyst which communicated with the excretory passages. A nephrectomy was performed. In case 7 the patient had suffered from a continuous feeling of weight in the left renal area for 18 months. The kidney was not palpable. In a blood cell count, eosinophils amounted to 4 per cent. Weinberg and Casoni tests were negative. A plain film showed a crescent-shaped calcification in the upper pole of the left kidney. 011 excretory urography the left kidney was not delineated. Retrograde pyelography demonstrated the tumoral process of the superior pole which included the calcification and hydronephrosis (fig. 8, B). On exploration a big hydatid cyst of the upper pole was found communicating with the drainage system. The hydronephrosis probably was due to hydatic elements causing obstruction of the superior part of the ureter. A nephrectomy was perfomed.
Renal scans do not provide information about the nature of the space-occupying lesion, but they afford the opportunity of evaluating the remaining parenchyma and of establishing preoperatively whether the operation will be conservative or radical. An estimate of the parenchyma is sometimes feasible by excretory urography only (our case 9). The patient had had dull but constant pain in the right side of the abdomen. Irradiation had been applied to the right lumbar region. An x-ray of the kidneys showed a round calcification in the right renal area. A hard, painless swelling about 10 cm. in diameter was palpated in the right kidney. The WDC was 6,500 and eosinophils 2 per cent. The \Veinberg reaction was negative and the Casoni test slightly positive. Excretory urography showed that the calcification was related to the kidney, the superior calyx of which was pushed aside without infiltration frorn the right and external side (fig. 9, A). Retrograde pyelography demonstrated the situation more clearly (fig. 9, B). The functional capacity of the kidney was good. The calcified hydatid cyst which was found at operation was removed ,vithout substantial injury to the kidney (fig. 9 . C). Postoperative renal function was satisfactory (fig. 9, D). Renal scans are usually more clarifying than excretory urograms. In case 10 the patient entered the hospital because of a dull pain in the left lumbar region, (5 years in duration) and, recently, associated with colic. The eosinophilia (4 per cent), the calcifications shown on the plain film, the retrograde pyelogram and the positive Weinberg and Casoni tests led to the diagnosis of hydatid cyst (fig. 10). An excretory urogram showed non-function of the left kidney, but the scan confirmed complete parenchymal alteration and established the necessity of nephrectomy (fig. 11). Finally, cystoscopy, by itself, may contribute to the diagnosis, only if it can prove the existence of hydatid elements in the bladder or coming out of the ureteral meatus. CONCLUSIONS
The diagnosis of echinococcosis of the kidney would be facilitated if the physician thought of it in every instance as a tumoral process in the kidney. Hydatiduria, eosinophilia, calcifications on
HYDATID DlfiEASE OF KID~"IEY
a plain film (KUB) and characteri~tic pydographic may direct attention to echinococcosis. 1'1orc specifie exmninations, such as the \Y einberg and the Caso11i tests, can then be made. Sir,ce no is pathognomonie of hydatid disease the kidney, all suitable examinations data should should be exhausted and the
be jointly cva,luatecl so that or a tentative diagnosis may be made. ,STTMM.A.RY
Tlw diagno,tic problems pa,tients with hydatid cysts of been disctis,sed.