Chapter 24 (page 113)

The Pyloric Sphincteric Cylinder in Health and Disease

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Chapter 24 (page 113)

Lumsden and Truelove (l958) described cases which showed that AHPS might be a sequel to IHPS, or it might arise de novo in adults. In the first category 5 patients who had received medical treatment for hypertrophic pyloric stenosis in infancy, showed radiological abnormalities campatible with AHPS in later life. Similar cases were described by other authors (Chap. 23); most of these adult cases were symptom-free.

Skoryna et al. (l959) described 6 cases with the morphology based on Torgersen's anatominal concepts. In 2 cases there was hypertrophy of the circular musculature of the pyloric canal (i.e. presumably the pyloric sphincteric cylinder) without other macroscopic lesions in the stomach and duodenum (one of the cases showed microscopic evidence of chronic gastritis); these were labelled cases of diffuse primary pyloric hypertrophy without associated proximal gastric lesions. In one case hypertrophy of the circular musculature of the "pyloric canal" was associated with a gastric ulcer on the lesser curvature 6.0 cm orally to the pylorus (i.e. approximately 3.0 cm orally to the commencement of the sphincteric cylinder) together with associated chronic gastritis; this was called a case of diffuse primary pyloric hypertrophy associated with a proximal gastric lesion. In another case there was moderate hypertrophy of the musculature of the entire pyloric canal, with a relatively greater degree of hypertrophy of the circular muscle on the lesser curvature at the muscle knot; there was no associated gastric or duodenal lesion, and Skoryna et al. (1959) considered it to be an example of primary pyloric hypertrophy of the focal type. (This case is also quoted in Chap. 25). One case exhibited benign ulceration within the confines of the "pyloric canal" (with muscular hypertrophy of its walls), and another had pyloric muscle hypertrophy with extensive surrounding adhesions and fibrosis, suggesting a previous inflammatory process (probably a duodenal ulcer). These 2 cases were considered to be pyloric muscular hypertrophy secondary to a distal obstructive lesion, e.g. obstructive complications of pyloric or duodenal ulceration.

Knight (l961) described 7 cases of AHPS; four of these were of a focal nature and are considered elsewhere (Chap. 25). Two of the remaining cases had diffuse enlargement of the pyloric musculature similar to IHPS, without evidence of gastric or duodenal ulceration. Gallstones were present in one of these, for which a cholecystectomy was performed. Both cases needed pyloroplasty. In the last case there was diffuse pyloric muscular hypertrophy involving a segment 2.5 cm in length, with a benign gastric ulcer 6.0 cm proximal to the pylorus. According to Knight (1961), hypertrophy in IHPS involves the entire circumference, while in the adult it may involve only a localized segment of the pyloric musculature. In IHPS hypertrophy always seems to occur as a primary condition, without associated upper gastro-intestinal pathology, while in the adult associated peptic ulceration or gastritis is common. This author found the thickness of the "pylorus" in 10 fresh post-mortem adult stomachs to range from 4.0 to 7.0 mm, with an average of 5.1 mm, while the thickness ranged from 10 to 15 mm in AHPS. The gross appearance of the pylorus in AHPS was similar to that of IHPS.

Edwards (1961) recognized two types of AHPS, viz. an idiopathic form, in which no other gastric lesion was present, and a secondary form in which there was some associated lesion such as gastric or duodenal ulceration or gastric carcinoma. It was stated that the etiology remained doubtful and among the possibilities to be considered were persistence of the infantile form, degeneration of the myenteric plexus and long continued pylorospasm. Pathologically the circular musculature was increased in thickness from the usual 3.0 to 8.0 mm to a maximum of 30 mm with an average of 15 mm. Radiologically elongation and narrowing of the pyloric canal was present.

Christiansen and Grantham (l962) collected 56 case reports published subsequent to the review by North and Johnson (l950), and added 2 of their own. In both their cases the gross and histological appearances were indistinguishable from those of IHPS. Of the 58 cases reviewed, 34 had no associated lesion in the upper gastrointestinal tract, but the remaining 24 had associated pathology, the most common lesions being gastritis (9), cholecysititis (7) and hiatus hernia (4).

Seaman (l963) reported 27 cases of AHPS, of which l8 had an associated gastric ulcer and 2 more had evidence of previous gastric ulceration. It is not clear from the description what the position of the gastric ulcer was in each case. Most of the other cases had microscopic evidence of gastritis, while 2 had duodenal ulceration (one of these also had a gastric ulcer.) There was no evidence of obstruction in the duodenal ulcer cases. While accepting the normal measurements of Horwitz (l929), Craver (l957) and Knight (l961), Seaman (l963) found that the thickness of the pyloric musculature in his cases varied from 9.0 to 15 mm, with an average of 11.2 mm. He found the gross appearance of AHPS to be similar to that of IHPS, the thickest portion of the musculature being at the pyloroduodenal junction, ending abruptly at the duodenum, but decreasing gradually as it faded into the "antrum" on the gastric side. Seaman based the morphology of the lesion in AHPS on the muscular anatomy as determined by Forssell (l913), Cole (l928) and Torgersen (l942).

Wieser et al. (l963) reviewed 44 operatively confirmed cases of AHPS. The condition was defined as any non-carcinomatous wall-thickening in the canalis egestorius, irrespective of its etiology. Two types were recognized, viz. a purely muscular form and a mixed form in which additional evidence of gastritis was present in the mucosa and submucosa. Twenty percent of the cases were associated with hiatus hernia, and gastric ulceration was present in 50 percent; this was probably not coincidental, the ulceration being deemed to be secondary to the pyloric stenosis. Radiologically there was elongation and narrowing of the pyloric canal with a prepyloric outpouching, altered peristalsis and a concave indentation of the base of the duodenal bulb. The condition had to be differentiated from early pyloric carcinoma.