Type Hirschsprung Hypertrophic Pyloric Stenoss

hypertrophy, spasm, stenosis, primary, gastric, congenital, favorable, usually and stomach

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(II) According to another opinion, hypertrophy (congenital or acquired) is a secondary- manifestation, the explanation being as follows: 1. The hypertrophy is compensatory in order to overcome either primary stenosis of the pyloric ring or some other mechanical factor interfering with the removal of the gastric contents.

This idea is supported by the fact that the hypertrophy seems to preferably involve the antral musculature, upon which the task of removing the gastric contents principally devolves. The primary stenosis may then be either of a spastic or organic nature, and possibly be occasioned by abnormal folds of the niucous membrane, abnormal position, kinking of the contiguous parts of the intestine in the patho logical formation of the fixation bands, of the mesentery, of the omen tum, etc. (as may occur after fetal inflammatory processes). This explanation may, perhaps, fit part of the cases, but, generally speaking, the following doctrine is 110W given the preference.

2. The hypertrophy is tbe consequence of a primary spasm or a disturbance of coordination in the function of the entire gastric 0111S culature, a kind of hypertrophy- exciting to activity (Thomson, 1895).

The primary spasm was attributed to reflex spasm of the gastric mucosa, incited by- erosions, fissures, over-activity, hyperchlorhydria, achylia gastriea, abnormally fatty food or food remnants, in the presence of increased irritability of the mucous membrane owing to hereditary nervous disposition; or it may have been produced in the fcetus by faulty development of the nervous apparatus which would cause the muscular funetion of both gastric cavities to aet antagonistically insteacl of synergi cally (Thomson, and Still's stomach stuttering, "Alagenstottern").

There are many bases of support for the spasmogenic theory.

1. It is an almost established fact that, specifie fae.tors are also involved in this form of stenosis. The lumen of the pyloric ring is apparently, much reduced; the pathological course undergoes sudden interruptions; the stenosis may become latent, while the hypertrophy continues to be present; the growth stiffens under the palpating finger and the patient apparently suffers spasmodic painful paroxysms. Fur thermore, the clinical and anatomical findings in special cases show a remarkable divergence.

2. There is, according to almost unanimous opinion, a very great dissimilarity of shape between the hypertrophie and the purely systolic pylorus.

3. True hypertrophy and simple pylorospasm are met with in members of the same family-, such as brothers and sisters (Freund and others).

4. It is only the spasmodic theory that at the present time furnishes to some extent guiding points for the remarkable preponderance of breast fed children consisting in the relations between the suckling act and gastric function, high fat percentage, slight acidifying property of the human milk.

Deposits of denser tracts of connective tissue between the strong muscular layers, proliferation of certain elements of the mucous mem brane, which may be real or only simulated by- folds and oblique incisions, persistence of the pyloric tumor under anmsthesia, increased resistance of the pyloric ring to increased pressure in the stretching test, and similar arguments, may possibly be used against the assumption of a simple (persistent) spasm, but not against that of a spasmogenic hyper trophy.

Other hypotheses which hold a position between those advanced above assume that the hypertrophy is primary; that the stenosis occa sions only late clinical manifestations by spasm; that the stenosis is attributable to congenital malformation, leading to spasm and com pensatory, hy-pertrophy; that congenital faults of development may lead to the gravest results as well as to a favorable course of the hyper trophy with pure spasms, etc. An attempt at a new plausible explana tion of the probable origin of the hypertrophy- has recently been made by the author (Miinchner med. Wochenschrift and Jahrbuch f. kinder heilkunde, 1909).

The course of the illness is usually either slowly progressive or receding for several weeks. In nearly all published cases there was more or less rational treatment, commencing at a certain stage, so that it is hardly possible to form a definite judgment as to quite spontaneous eures. Vomiting, the cardinal symptom, may take a specially favorable course, as it may become less frequent and at the same time more bulky in cases where, in the beginning, it occurred twelve or more times in the course of a day ("second stage of the affection"). Hyperkinesis of the stomach may likewise improve in the course of the disease without, however, influencing its final outcome. With careful observation it is usually still possible to find it present one and one-half to two hours after the ingestion of food, and it may even persist in a curable case for weeks or months after vomiting has ceased (Ibrahim). The same holds good for the retention of the chyme in the stomach. In cases which run a favorable course there is usually a gradual decrease of the symptoms, first of votniting, then of pseudo-constipation and, soon after, of under nutrition. On the other hand, fifteen authors have made the remarkable observation that all (primary) manifestations may quite suddenly disappear either permanently or temporarily, but at the same Hine it should be noted that sudden cessation of vontiting may give rise to deceptive hopes and be the precursor of a fatal issue. True relapses have not been reported.

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