The second group of eases begin first in the later months of life. In order to furnish a complete picture of Hirschsprung's disease in its clinical manifestation we must assume a eongenital anatomical dis position of the sigmoicl flexure (unusual length and tortuosity) which, indeed, means only an increase of the normal infantile condition; and which, under the influence of an alteration in dict, may lead to copros tasis, dilatation of the colon and hypertrophy of its walls; in short, to all those changes which we find already brought about in the first type.
The probability of such a method of origin, which has already been suggested by the anatomical findings of Martian, Neter, Saias and others, is increased by an interesting clinical observation of Ibrahim, to whose kindness I arn under obligations for the original notes of his case (see Figs. 3 and 4 on Plate 49). From this it is evident that the part of the intestine outlined on the intestinal wall, because of its great dilatation and peristaltic activity, corresponds to the widened and length ened sip-nob] fiC1,111.0. This ean also be verified by the introduction of an intestinal .ouricl, and indicates with great probability the first stage of the disease.
The corresponding anatomical findings are somewhat varied; their reliability, however, suffers somewhat because of the fact that the cases come to autopsy often after the illness has lasted a long time. On open:ng the abdom inal cavity, the colon is found dilated to the size of an arm and is bent up into two legs filling almost the whole peritoneal cavity. The length and free mobility of the mesentery of the signioid flexure can be ascertained. Ac cording to Ilirschsprung, the walls of the very much dilated and often lengthened colon are always hypertrophied. (Figure 22 shows this plainly.) Still this condition does not apply to all cases, so that Coneetti distinguishes t ree types, which he designates as mycrocolia (simple lengthening), ectocolia fectitsia of a more or less long section of the colon with or without compensatory dilation, or hypertrophy of the portion lying next to it), and, megalocolia (applying partieularly to a general enlargement in the diameter cif the lumen and to a thickening of the intestinal wall). In addition to clinical observation, anatomical experienee, too, is in favor of a congenital origin of many cases. Such congenital dilatation or hypertrophy may also oceur in other portions of the intestinal tract, as in the interesting case of a child who died in six days, reported by Schukowski, which, in addition to congenital dilatation and muscular hypertrophy of the colon, had a marked increase in the musculature of the whole small intestine, and, at the same time, a stenosis of the duodenum and an umbilical hernia. There
are moreover, also, cases of congenital origin which show only scattered areas of hypertrophy of the wall of the large intestine, which areas alternate with atrophic portions (Concetti); as well as those in which extensive thinning, and partial, or coinplete, disappearance of the muscle-coat in the walls of the colon is found (Beighing). I have already mentioned the frequent ulcerations of the mucous membrane and the subinueosal abscesses (Hirsehsprung) springing from them.
Even with the naked eye, it is possible to determine that the prin cipal part of the thickening of the wall of the large intestine takes place in the muscular coat; and, in histological sections, one of which I am able to present from the preparation of Dr. Gourevitch ,Fig. n, Plate .IS), one can s c e that the hypertrophy and also the hyper plasia, as the meas urement of a single cell indicates, c on cerns chiefly the cir ul a r muscle- layer and the muscularis mucosa. Among other microscopic changes arc connec tive-tissue prolifera tion, especially in thc neighborhood of the submucosa,clilatation of the vessels, obliterating arteritis, and leueocytie infiltration; and, also, a thickening of the serous coat, as made out from the researches of Concetti, Genersich and others.
The treatment of the affection has been attended, heretofore, with little SUCCCSS. Most of the patients, after the symptoms have lasted for a shorter or a longer time, (lie in consequence of weakness or chronic intoxication, from poisons from the sluggish intestinal contents. The first often very pressing indication for treatment consists in the evacuation of the fecal masses and the removal of the intestinal gas. To accomplish this latter result, Hirschsprung finally- recommends a puncture of the intestine. Enemata and high irrigations, in which often a large part of the water introduced remains behind, either fail entirely, or are, at most, incompletely successful; and, therefore, must be accompanied by a manual emptying of the rectum and massage of the abdomen. In this manner incredibly large fecal masses are often emptied out (in an observation of Concetti over 10 kilos; 25 lbs.) and the size of the lower abdomen is markedly reduced, as is indicated in the figures here reproduced (see Figs. I and 2 on Plate 49). When this method is not successful, one can attempt laxatives and glycerin suppositories (Fenwick, Levi).