Fig. 251.Echinococcus veterinorum: the fibrous sac enclosing the echinococcus has been opened and laid back in five parts, so that the surface of the bladder worm may be seen, with the brood capsules, visible to the naked eye, showing through it. Natural size. (After Leuckart.)

An echinococcus is a spherical or roundish bladder full of a watery liquid, which originates by liquefaction of the oncosphere, and in man may attain the size of a child’s head, but remains smaller in cattle (the size of an orange or apple). The thin wall of the bladder is composed of an external laminated cuticle (ectocyst) and an internal germinal or parenchymatous layer (endocyst). The latter again exhibits two layers: an outer layer of small cells that are less sharply defined, and an inner layer of larger cells. It contains, in addition, calcareous corpuscles, muscular fibres and excretory vessels. It is rich in glycogen.

Fig. 252.

Figs. 252 and 252A.—Diagrams of mode of formation of brood capsule and scolices. (1) Wall of mother cyst, consisting of ectocyst and endocyst; (2) theoretical stage of invagination of wall; (3) a brood capsule with the layers of the wall in the reverse position to that in the mother cyst; (4) evagination of wall; (5) invagination; (6) fusion to form the solid scolex; (7) invagination of fore-part of scolex into hind-part. (Note.—The size of the scolex is much out of proportion to the brood capsule.) (Stephens.)

Fig. 252a.

The development in cattle often remains stationary at the bladder stage, and they are then called “acephalocysts,” or Echinococcus cysticus sterilis. According to Lichtenheld, sterile cysts occur in 80 per cent. of cases in cattle, in 20 per cent. in pigs, and in 7·5 per cent. in sheep. In other cases large numbers of small, hollow BROOD CAPSULES are formed in the germ layer, but are not arranged in any particular order. The order of the layers is just the reverse in them to what it is in the parent cyst, that is to say, they have inside a thin non-laminated cuticle and the parenchymatous layer on their external surface. These, theoretically at least, may be regarded as invaginations of the bladder wall giving rise to a cavity with the cuticle internal and the parenchymatous layer external. If we suppose the orifice to close, we should then get an isolated cavity with cuticle internal and parenchymatous layer external, as in the brood capsule (fig. 252). If we next suppose an evagination of the wall of the brood capsule to occur at one point we should get a hollow process lined with cuticle; at the bottom of this we get the scolex and hooklets formed, and a little higher up the tube the suckers (fig. 252, 4). If this hollow scolex is now pictured as being invaginated we get a hollow scolex covered with cuticle and lined by a parenchymatous layer projecting into the cavity of the brood capsule. The two sides of this hollow scolex now fuse and we get a solid scolex projecting into the cavity. Finally, if we imagine once more the rostellum and suckers invaginated into the posterior part of the scolex we get the condition as frequently found in the brood capsules, i.e., a scolex covered with cuticle projecting into the cavity, with the rostellum and suckers invaginated into the posterior portion of the scolex (fig. 252A, 7).

Fig. 253.—Section through an in­va­gin­ated echinococcus scolex. Cf. fig. 252A, 7. × 300. (After Dévé.)

A large hydatid may contain many thousands of brood capsules. Each brood capsule is about as big as a pin’s head, and may contain ten to thirty or more scolices. The delicate wall of the brood capsules may rupture, so that the scolices are now free in the mother cyst. These free scolices and also free brood capsules constitute what is known as “hydatid sand,” which settles at the bottom of a glass when hydatid fluid is poured into it. This form occurs chiefly in domesticated animals and is termed E. veterinorum, Rud., or E. cysticus fertilis.

In man, and only rarely in cattle, the mother cyst first forms “daughter cysts” (E. hominis, Rud. [fig. 255]), which, though smaller than the “mother cyst,” resemble it in the structure of their walls; thus they are covered externally by a laminated cuticle and internally by the parenchymatous layer. They originate:

(1) Between the laminæ of the cuticle of the mother cyst from small, detached portions of the parenchymatous layer; during their growth they bulge inwardly or outwardly and may separate themselves entirely from their parent cyst. In the latter case they lie between the mother cyst and the capsule of connective tissue formed by the host (E. granulosus or E. hydatidosus exogenus); when growing inwardly they reach the interior of the mother cyst (E. hydatidosus endogenus). Their number is very variable and does not depend on the size of the mother cyst. They are as big as, or bigger than, gooseberries.

Fig. 254.—A piece of the wall of an Echinococcus veterinorum stretched out and seen from the internal surface. A few brood capsules (the outline of which is only faintly shown), with scolices directed towards their interior and exterior. 50/1.

(2) According to some authors, endogenous daughter cysts arise also from a metamorphosis of scolices that have separated off from the brood capsule. This takes place in the following way: Fluid accumulates in the interior of the scolex, so that eventually nothing remains except a sac consisting of cuticle lined by parenchyma. The cuticle gradually thickens and several layers form (fig. 257).

Fig. 255.Echinococcus hominis in the liver. The fibrous capsule and the wall of the echinococcus have been incised, so that the endogenous daughter cysts may be seen. Reduced. (After Ostertag, from Thoma.)

Fig. 256.—Section through an echinococcus scolex in process of vesicular metamorphosis, twenty-six days after insertion in the pleural cavity. × 250. (After Dévé.)

(3) Transformation of Brood Capsules into Daughter Cysts.—This is also held to be possible by various observers. New epithelial layers are deposited between the cuticle which lines the brood capsule and the outer parenchymatous layer. This parenchymatous layer gradually disappears and a new parenchymatous layer forms in the interior from the parenchyma of the scolex or scolices. Although it appears strange that a completely formed scolex with specifically differentiated tissues and organs should retrogress to more primitively organized matter, and again become a proliferating bladder, yet we can hardly doubt that the older observations, regarding such a vesicular metamorphosis, of Bremser (1819), v. Siebold (1837), Naunyn (1862), Rasmusser (1866), Leuckart (1881), Alexinsky (1898), Riemann (1899), Dévé (1901), and Perroncito (1902) are correct.

(4) Further, a fourth method of formation of daughter cysts is described by Naunyn as occurring in sterile hydatids, i.e., those containing no brood capsules. In this case a portion of the mother wall of the hydatid gets constricted off.

Fig. 257.

Figs. 257 and 257a.—Diagram of transformation of a scolex into a daughter cyst (1 to 3): 1, scolex in brood capsule; 2, liquefaction of scolex; 3, daughter cyst; and (4 to 6) of a brood capsule into a daughter cyst; 4, brood capsule with scolex; 5, deposition of new epithelial layers on the inner layer of the parenchyma; 6, disappearance of outer parenchyma and formation of inner parenchyma from the parenchyma of scolex, which has now disappeared. (Note.—The scolices are out of proportion to the brood capsules and to the daughter cysts. Stephens.)

Fig. 257A.

It has also been established that not only daughter cysts transplanted into animals develop further (Lebedeff, Andrejew, Stadnitzky, Alexinsky, Riemann), but that this also holds good if only hydatid scolices from man or animals are transplanted into animals (rabbits). They develop into echinococci and can then give rise to brood capsules and scolices. As Dévé further established, hydatid scolices are not capable of developing in guinea-pigs, while corresponding experiments with rabbits are in the large majority of cases successful where the scolices are introduced subcutaneously or into the pleural or peritoneal cavities. It is only in the case of daughter cysts that further growth is obtained in the case of guinea-pigs. Finally it appears, as has been already stated, that brood capsules can transform themselves into daughter cysts, but according to Dévé only within the mother cyst, not after transplantation. Daughter cysts that have been formed in the mother cyst of man and animals behave themselves just as the mother cyst does, i.e., they can remain sterile, or give rise to brood capsules and scolices, or even again to fresh cysts—granddaughter cysts. The mother cyst can also die, so that the daughter cysts then lie in the cavity of the connective tissue capsule. The number of the daughter cysts in either case may attain several thousands.

The echinococcus fluid, which originally is formed from the blood of the host, is light yellow, with a neutral or slightly acid reaction; its specific gravity averages 1009 to 1015. It contains about 1·5 per cent. of inorganic salts, half of which is common salt; in addition (besides water) it contains sugar, inosite, leucine, tyrosin, succinic acid (associated with lime or soda) and albumens which are not coagulated by heat; occasionally also the fluid has been found to contain hæmatoidin and uric acid salts (in echinococcus of the kidneys), which doubtless demonstrates that the echinococcus liquid originates from the host. It has been generally assumed that echinococcus fluid contains a toxic substance the escape of which into the body cavity (at operation or by bursting of a hydatid cyst) produces more or less severe symptoms (fever, peritonitis, urticaria), so much so that one speaks of hydatid intoxication. The investigations of Kobert, Joest, etc., have, however, shown the harmlessness of fresh undecomposed hydatid and cysticercus fluid for rabbits, mice and guinea-pigs, whether inoculated intraperitoneally, subcutaneously or intravenously. Contrary data or clinical experience must accordingly depend on other factors.

According to the researches of Leuckart, the growth of the echinococcus is very slow; four weeks after infection the average size is only 0·25 to 0·35 mm., at the age of eight weeks it is 1 to 2·5 mm., and at this period the formation of the central cavity commences; at the age of five months, and with a size of 15 to 20 mm., the first brood capsules with scolices are formed. The consequence of this gradual increase of size is that the organ attacked can maintain its functions by vicarious hypertrophy, and that many echinococci induce no special symptoms and cannot even be diagnosed, the latter circumstance being due to their hidden position.

The echinococcus cannot be said to be scarce in man, as is shown by the following table for Central Europe:—

Place
Period
No. of
post-mortems
No. of cases
of echino.
Percentage
Rostock
1861–83
1,026
25
2·43
Greifswald
1862–93
3,429
51
1·48
Jena
1866–87
4,998
42
0·84
Breslau
1866–76
5,128
39
0·761
Berlin
1859–68
4,770
33
0·69
Würzburg
2,280
11
0·48
Göttingen
639
3
0·469
Dresden
1852–62
1,939
7
0·36
Münich
1854–87
14,183
35
0·25
Vienna
1860
1,229
3
0·24
Prague
1,287
3
0·23
Kiel
1872–87
3,581
7
0·19
Zürich, Basle, Berne
7,982
11
0·13
Erlangen
1862–73
1,755
2
0·11

These, however, are only cases that have become known by post-mortem; in addition, there are cases that have been treated medically, of which there are a few statements, at all events relating to the principal districts of Germany. According to Madelung, one case of echinococcus occurs in every 1,056 inhabitants in the town of Rostock, in the district of Rostock one to every 1,283, in Schwerin one to every 5,887, and in Ludwigsort one to every 23,685; according to Peiper, in Upper Pomerania one case occurs to every 3,336, in the district of Greifswald one to every 1,535 inhabitants. The northern districts of Pomerania are more affected than the southern ones.

Accordingly, echinococcus is also considerably more frequent in cattle in Pomerania. On an average in Germany 10·39 per cent. oxen, 9·83 per cent. sheep, and 6·47 per cent. pigs are infected, whereas in Upper Pomerania 37·73 per cent. oxen, 27·1 per cent. sheep, and 12·8 per cent. pigs are infected; in Greifswald, indeed, 64·58 per cent. oxen, 51·02 per cent. sheep, but only 4·93 per cent. pigs are infected. In accordance with these figures Tænia echinococcus must be frequent in dogs in Pomerania, especially in Upper Pomerania; on the other hand, the conjecture that the frequency of echinococcus in Mecklenburg is explained by the occurrence of Tænia echinococcus in foxes has not been confirmed, as the fox does not harbour this worm in Mecklenburg.

Fig. 258.—Hooklets of echino­coccus. a, of Echino­coccus veteri­norum; b, of Tænia echino­coccus, three weeks after infection; c, of the adult Tænia echino­coccus; d, the three forms of hooklets out­lined one within the other. 600/1. (After Leuckart.)

Beyond the European continent, echinococcus is frequent in the inhabitants of Iceland, Argentine, Paraguay and Australia. In Iceland, according to Finsen, 1 in every 43 inhabitants is affected with echinococcus; according to Jonassen the proportion is 1 to 63; this is due to the habits of the people of Iceland or, in fact, to the frequency of Tænia echinococcus in dogs, and the prevalence of the hydatid in cattle. In certain districts of Australia it is just as frequent. In Cape Colony, Egypt and Algeria echinococcus is not rare, but it is scarce in America and in Asia, with the exception of the nomadic tribes of Lake Baikal.

Echinococcus attacks persons of every age, though it is rare in children up to 10 years of age and in old people. It occurs most frequently between the ages of 21 and 40 years. According to all statistics it preponderates in women (about two-thirds of the cases). The liver is its favourite seat (57·1 per cent. of the cases); next in order come the lungs (8 per cent.), kidneys (6 per cent.), cranial cavity, genitalia, organs of circulation, spleen (3·8 per cent.), etc. As a rule one organ only is invaded; multiple occurrence may originate from one infection, or eventually from a later infection (?), or it may come to pass that from some cause (through the spontaneous rupture of an echinococcus, or the rupture of one caused by an injury or surgical operation) daughter cysts, brood capsules or scolices escape into the abdominal cavity,294 where they settle or become transformed and go on growing. In the distribution of this secondary echinococcus the great powers of motility of the free scolices must be taken into account (Sabrazès, Muratet, and Husnot).

Human echinococci may also die at various stages of development, become caseous or calcified, or may be absorbed, the cause for this being either disease of the hydatid itself or inflammation of its connective tissue capsule; the discovery of the laminated cuticle, which has great powers of resistance, or the finding of the hooklets of the scolices is sufficient to form a conclusion as to the nature of such formations.

Siebold (1853) was the first to rear Tænia echinococcus in the dog by feeding it with the echinococcus of cattle and especially of sheep. Küchenmeister, van Beneden, Leuckart, Railliet and others obtained similar results, and Thomas, Naunyn, Krabbe and Finsen succeeded in rearing T. echinococcus in dogs from the bladder worms of human beings; these grow comparatively slowly (one to three months295) and only during the process of growth develop their hooklets in their definite form (fig. 258). It lies in the nature of things that dogs, whether experimentally or naturally infected, almost always harbour T. echinococcus in large quantities. That cats exceptionally harbour these worms has been already mentioned (Dévé). Finally, Leuckart infected young pigs by feeding them with mature segments.

Echinococcus multilocularis (alveolar colloid).

In addition to the form of echinococcus already described, and which is also frequently termed Echinococcus unilocularis, there is a second form which occurs in man as well as in animals, and which is termed E. multilocularis, s. alveolaris (alveolar colloid).

It was originally regarded as a tumour; its animal nature was first established by Zeller and R. Virchow. The parasite, which varies in size from that of a fist to a child’s head, presents a collection of numerous cysts, measuring between 0·1 and 3 to 4 mm. to 5 mm. in diameter, which are embedded at first in a soft, connective tissue stroma; the cut surface has therefore a honeycomb appearance. The cysts are surrounded by a pellucid and laminated cuticle, and each according to its size encloses either a small-celled tissue or a cavity lined by a parenchymatous layer; the fluid contained in such a cavity may be transparent, or is rendered opaque by globules of fat, bile-pigment, hæmatoidin and fat crystals. According to some authors all or most of these cysts intercommunicate; others state that this is the case at least as regards the cuticle. The scolices are by no means found in all the cysts, and when present only a few, rarely half, of the cysts contain scolices (one or more); it is supposed that at least some of these scolices are formed in brood capsules, and that the former are capable of undergoing a cystic metamorphosis.

One circumstance is peculiar to the multilocular echinococcus of man, namely, the disintegration that sets in at certain stages; in the centre of the parasite a cavity forms that frequently becomes very large and is filled with a purulent or brownish or brownish-green viscid fluid; in this fluid one finds shreds of the wall of the cavity, calcareous bodies, echinococcus cysts, also scolices and hooklets, as well as fat globules and crystals of hæmatoidin, margarine and cholesterin and concretions of lime. Such ulcerative processes, according to Ostertag, are never present in the multilocular echinococcus of oxen,296 in which the separate cysts are larger and the connective tissue integument less powerfully developed.

Fig. 259.Echinococcus multilocularis in the liver of the ox. Natural size. (After Ostertag.)

Hardly anything positive is known with regard to the development of the alveolar echinococcus; its peculiar conformation is attributed by some to enormous infection of oncospheres, by others to the abnormal situation of one oncosphere; a few authors ascribe it to infection of lymphatic vessels, others to infection of the biliary ducts or to peculiarities of the surrounding hepatic tissue; Leuckart ascribes it to a grape-like variety of form which continues budding; a few more recent authors consider multilocular echinococcus to be specifically different from unilocular echinococcus, and therefore also different the species of Tænia arising from them. Melnikow-Raswedenkow is also of this opinion. According to this author the oncospheres infect the lumen of a branch of the portal vein in Glisson’s capsule of the liver and grow into an irregularly shaped formation (chitinous coil), which breaks through the vascular walls and thus forms the alveoli. So far the data coincide well with Leuckart’s opinion of the original grape-like form of the Echinococcus multilocularis; according to Melnikow-Raswedenkow the “granular protoplasmic substance” (parenchymatous layer) is not only present in the interior of the loculi but also outside, and, moreover, “ovoid embryos” are supposed to develop in the chitinous coils, which, “thanks to their amœboid movements, reach the lumen of a vessel, where, under favourable circumstances, they begin to develop further,” that is to say, they become “chitinous cysts with fantastic outlines,” or also “single-chambered chitinous cysts”; scolices may develop in both. Dévé, however, considers that these embryos are only prolongations of the protoplasmic layer which secondarily cuticularize.

The multilocular echinococcus, which in man produces a severe disease and almost always leads to premature death, infects most frequently the liver, but may also be found primarily in the brain, the spleen and the suprarenal capsule; from the liver by means of metastasis it may reach the most various organs, especially those of the abdomen, but also the lungs, the heart, etc. Up to 1902, 235 cases have been described and up to 1906, 265, being 70 from Russia, 56 from Bavaria, 32 from Switzerland, 30 from the Austrian Alps, 25 from Würtemberg; the remaining cases are distributed over Central Germany, Baden, Alsace, France, Upper Italy, North America. In some the origin is doubtful; in any case after Russia, the mountainous South of Europe is the principal region of distribution. As to the domesticated animals, the same parasite is found principally in the ox (according to Meyer, in Leipzig, in 7 per cent. of the oxen affected with echinococcus); it is rarer in the sheep and very scarce in the pig.

It has already been mentioned above that recently the multilocular echinococcus has been stated to be specifically different from hydatid or unilocular echinococcus. To this may be added the fact that Mangold, who fed a young pig with oncospheres of a Tænia reared from the multilocular echinococcus, found two growths in the liver four months later, which he took to be E. multilocularis, and consequently one has to assume the existence of two different worms. The chief defender of this view, already put forward by Vogler, Mangold, and Müller, is Possett. He bases his opinions on (1) the more restricted distribution of the multilocular hydatid, the former occurring in districts where only cattle are raised, the latter where sheep-breeding is established; (2) that those engaged in looking after sheep are attacked by multilocular, whereas those looking after cattle are attacked by unilocular hydatid; (3) that among the cases of unilocular hydatid occurring in the distribution areas of multilocular hydatid no transitions between the two forms are observed; (4) on the difference in the hooks both in the hydatid as well as in the Tænia stage; the hooks of Tænia echinococcus are plump, sharply curved, and have a short posterior root process the length of which is to that of the total length as 1 to 4·7, whereas on the contrary the hooks of the alveolar echinococcus are more slender, slightly bent, and have a long posterior root process (1 to 2·5); and (5) on the form of the uterus, which in the alveolar Tænia has the form of a spherically distended sac anteriorly.

Serum Diagnosis of Echinococcus.

(1) Precipitin Reaction.—Mix equal parts of hydatid fluid (of the sheep) and serum of patient. Keep at 37° C. The reaction is not decisive as it may be given by normal sera.

(2) Complement Deviation.—Required: (1) Hydatid fluid of sheep (antigen), (2) guinea-pig complement, (3) patient’s serum, (4) red cells of sheep, (5) hæmolytic serum (of rabbit) against sheep’s red cells, (6) 0·8 per cent. salt solution. Mix the antigen + patient’s serum (heated) + complement + salt solution at 37° C. for one hour. Add red cells of sheep + hæmolytic serum. Allow to stand for half an hour at 37° C. It is imperative to make adequate control observations. An example will indicate the method. Salt solution 1·3 c.c. + patient’s serum (heated) 0·2 c.c. + hydatid fluid 0·4 c.c. + complement 0·1 c.c. of serum diluted to a quarter strength + hæmolytic serum and red cell emulsion 1 c.c. Result: no hæmolysis, i.e., the patient’s serum contains specific (echinococcus) antibodies.