Renal hydatidosis

RENAL HYDATIDOSIS* LUIS A. SURRACO, CIinicaI

Professor

of UroIogy,

MONTEVIDEO,

PATHOGENESIS

I. Circumstances Determining Location. (a) Frequency. Nicaise and other authors have reported percentages beIow the norma1 3 per cent. The countries most affected are AIgeria, IceIand, New ZeaIand, DaImatia, and above a11 Uruguay and Argentina in South America. My own contribution is represented by twentynine persona1 cases, which form the Iargest group in medica Iiterature thus far. (b) Path Traversed by the Parasite. The arteria1 route is the most IogicaI and probabIy the onIy one. (c) Primary location. The cyst is Iocated primarily in the kidney, since in genera1 there is no concomitant Iesion in other viscera. Secondary Iocation is exceptiona1. (d) Single Cysts. In generaI, just one cyst is present, but in exceptiona cases the cyst may be muItipIe or affect both kidneys. 2. Topography. The entity hydatid cyst pIus the pericystium and its parent membrane have important reIationships which I have been the first to stress. (a) Relationship to the Renal Structure. Since the cyst originates in the territory of the cortica1 zone and never in the pyramida region, it reproduces the forms of the visceral cyst of the Iiver, destroying the eIements of the kidney by the two * Read before the UroIogicaI Section, N.

of Medicine

processes of distention and congestion pIus toxemia. Its favorite site is the polar regions and it invades them up to the renaI periphery and to the pyelic cavities, where it opens. Indeed, once the cyst appears in the renaI periphery, it wiI1 be possibIe to note that the cIaim of a11 authors who have wished to consider the cyst as attached to the kidney (from which it couId easily be distinguished) is not justified, since it has been possibIe to ascertain the fohowing facts : I. It is impossibIe to find a Iine of cIeavage which determines where the kidney ends and the cyst begins. 2. There exists a parenchymatous zone which invests and accompanies the pericystium for its entire Iength. 3. This pericystium is at the same time invested by the renaI capsuIe, as proved by two things: in the first pIace, the cyst is aIways intrarena1; in the second pIace, the cyst is aIways infracapsuIar. AIthough other authors consider that the cyst is not invested by the renaI parenchyma (Fig. I), my own view is that the cyst is invested by the renaI parenchyma and aIso by the renaI capsule. (Fig. 2.) (b) Relationship to the Pyelic Cavities. Contrary to current reports, the cyst never opens directIy into the peIvis of the kidney without having its base aIways resting upon the end of a primary calyx through a solitary and smaI1 hoIe of communication. As a resuIt of this, we note a specia1 aspect of the cyst: the aspect which wiI1 give rise to the pyeIographic indication which I have caIIed the “gobIet sign.” This sign consists in a Iarge ova1 cavity represented by the pericystium, with one base

R

AND

Faculty URUGUAY

ENAL hydatidosis, a disease produced by Taenia echinococcus, has a Iow incidence, is not benign, and has been known for the past thirty years through pubhcations. The history of its deveIopment faIIs into three periods: (I) the cIinica1 period; (2) the operative period; (3) the period of pyeIography. ETIOLOGY

M.D.

Y. Academy

931

of Medicine,

Feb.

16, 1938.

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of support upon the extremity of the primary caIyx, and with one support represented by this primary caIyx.

FIG. I. CIassic anatomopathologic picture of a renal hydatid dist. The cyst is covered neither by the kidney nor by the capsule.

The gobIet sign may be identified as foI10~s: the pericystium rests upon the extremity of one caIyx, which serves as

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JUNE,1939

occur earIy, and they invoIve the parenchyma in toto, a fact which expIains the functiona aIteration of the organ.

FIG. 2. Our anatomopathologic picture of the same condition. The cyst is always intrarenal and infracapsular. ANATOMOPATHOLOGIC CYSTIC

DEVELOPMENT

OF THE

SACS

The cystic sacs are frequentIy regarded as simiIar to hydropyonephrosis. This, however, is a mistake, for two reasons: (I) the sacs in hydropyonephrosis represent permanent Iesions which have no tendency to regress once they have ruptured; (2) on the other hand, once the sacs of a hydatid cyst have burst, they tend to recede, to shrink, and to disappear by fibrous transformation. It is for this reason that I wish to stress the fact that it is not rare to see enormous cysts which had occupied the entire abdomen transformed into smaI1 stumps as soon as they have been drained. SYMPTOMATOLOGY

FIG. 3. The cyst opens out at the extremity of the primary caIyx, giving the goblet sign.

support to it and assumes gobIet. (Fig. 3.) PARENCHYMAL

the shape of a

LESIONS

These Iesions are important, they can be traced around or far from the cyst, they

Rena1 hydatidosis presents the characteristics of hydatid diseases: sIow, proinsidious deveIopment ; gressive, and destructive development; and tendency toward infection. Its symptomatoIogy is characterized by tumefaction; functiona urinary changes, and genera1 functiona changes. The tumor itseIf is gIobuIar, rounded, ffuctuating and not pitting, sometimes sometimes Iike parchment in consistency,

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and on rare occasions gives the sensation of a thriII. When the symptoms are thoracic and

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exaggerated and there appears nephritic triad of hydaturia, accordion-shaped tumor.

FIG. 4. A .caIcified cIosed cyst, showing

abdominal, the hypochondrium is apt to be invoIved. When the symptoms are abdomina1, the flank is apt to be involved. A cyst of the spIeen shouId not be confused with a renal cyst because the former never arrives inside the angIe of the coIon. We shouId further point out that thespIenic cyst usuaIIy dispIaces the angIe of the coIon which never occurs with a renal cyst. The characteristic feature of renal cystic tumefactions is that the kidney generaIIy loses motility, because the renal cyst, contrary to present cIaims, soon adheres to the Iumbar fossa. The urinary functional changes are always present. When we are deahng with a closed cyst they are evidenced by microscopic hematuria and functiona alteration of the kidney. When we are deaIing with an open cyst the above symptoms are

American

the goblet

933

the cIassic cohc, and

sign.

DIAGNOSIS

We are faced with three possibilities in differentiation: (I) a renal tumor; (2) a cystic tumor; (3) a cIosed or open cyst. I shaI1 stress here onIy the two main syndromes: the biologic and the pyelographic. Biologic Syndrome. Urticaria is very rare. Laboratory tests reveaI the foIIowing: EosinophiIia ranges between 3 per cent and IO per cent and can reach 20 per cent in rare cases. However, this point has no absoIute value, since it is not evidenced when the cyst is dead and caIcified, and may aIso occur in other diseases. I have found it in 30 per cent of my cases of renal tumors, Cassoni’s anaphyIactic reaction is found in 60 per cent of the cases, but it may also be present in renal cancer and in neopIasms of the lung. Weinberg’s reaction is found in 40 per cent of the cases.

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Radiographic

Surraco-Rena1

and Pyelographic

Syndrome.

SimpIe radiography may be of diagnostic aid in two circumstances: (I) in the pres-

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4.) (2) When there are spots in the kidney region; their vaIue is pureIy one of presumption.

FIG. 6. FIG. 5. FIGS. 5 and 6. Open hydatid cyst, showing the gobIet sign. (Note the rounded shadows on the inside of the goblet, which correspond to the daughter vesicIes.)

FIG. 7. Open

hydatid

cyst

and goblet

sign.

ence of caLfred cysts which are shaped Iike a gobIet or pear and appear in the renaI area; this sign is of great vaIue. (Fig.

FIG. 8. Open renaI cyst with pyeIogram showing crescent sign.

PyeIography settIes the cIinica1 diagnosis and gives picture of the cyst in two eventuaIities. We are sure of the diagnosis when

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we find: (a) what I caII the “gobIet sign,” a broad image resting upon a caIyx and taking the form of that caIyx. (Fig. 3.)

FIG. 9. Open renaI cyst with pyelogram

cluster

of grapes

sign (honeycomb

showing sign).

The center of the image may be distinct and uniform, or may present a succession of Iight and dark zones showing rounded regions corresponding to daughter cysts. (Figs. 6 and 7.) (b) The sign of Ihe crescent or segment of a moon is of enormous vaIue, and must be distinguished from faIse crescents (constituted by curvatures of the ureter). (Fig. 8.) (c) The sign of the bunch of grapes or honeycomb, the image given by the daughter cysts. (Fig. 9.) (d) The horizontal level sign indicates an air cyst of the kidney. This is the onIy case known to medicine. (Fig. IO.) These pyeIographic data are of fundamenta1 importance, and when the findings present the appearance noted they are pathognomonic of the disease. The diagnosis may be questioned in certain circumstances: (a) In the case of a ruptured cyst: when there are deformities Iimited to one caIix and simuIating crescents; when the rounded appearance of the cyst is outIined; when the areoIar aspect is seen to be confined to one caIyx. Apart from this, I do not beIieve that important

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consideration may be given to amputations of the caIyces and dispIaced ureters mentioned in the Iiterature.

FIG.

IO. Rena1 cyst with pyeIogram horizontal Ievel sign.

showing

In a cIosed cyst: where a shadow coincides with the cupoIa of a calyx; where dispIacements caused by torsion of the kidney make it appear that the kidney is supporting something, the existence of a cyst may be suspected. PROGNOSIS

The above mentioned views regarding pathoIogic anatomy, which show the occurrence of renal destruction, force me to contradict the present views. I beIieve that a hydatid cyst means an affection endangering the kidney. TREATMENT

EarIy treatment and conservative surgery are indicated for hydatid cysts. They must be treated at an earIy date because their deveIopment is progressive and destructive. SurgicaI treatment must be conservative because they remain a poIar affection for a Iong time, and, above aI1, because, as I pointed out before, cystic sacs tend to regress and to shrink once

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they have burst, being transformed into fibrous stumps. Nephrectomy is radicaI treatment. Conservative treatment is constituted by partial nephrectomy, excision of cysts and cIosure without drainage, and marsupialisation. In practice, we must decide between nephrectomy and marsupiahsation. Nephrectomy is necessary when marsupiahsation is contraindicated; it is obligatory in compIete destruction of the kidney; in muItipIicity of cysts in the same kidney; in caIcified fatty cyst, even when the kidney has not suffered much destruction; and when conservative surgery has not succeeded. In nephrectomy we must not forget that the cyst is aIways intrarena1 and infracapsuIar, as I have pointed out, and therefore the operation wiI1 have to be performed by the infracapsuIar route (which is easy, possible, and not dangerous). On the con-

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trary the operation is very diffIcuIt and very dangerous when, for any reason, it is done by the extracapsular route. MarsupiaIization must be borne in mind in a11 cases; it is also necessary: (I) as a preIiminary to tota nephrectomy; (2) when required by the patient’s condition. It has been unjustIy criticized because of the Iong duration of the fistuIa. However, I have always seen the fistuIa disappear within three months whenever the cystic sac was not attached to the waI1. CONCLUSIONS

Rena1 hydatidosis is a disease whose development is intrarena1 and infracapsular, whose diagnosis is determined by pyeIographic signs (gobIet sign, bunch of grapes sign, crescent sign, and horizonta1 IeveI sign), whose prognosis is bad, and which it is not aIways easy to treat.