The Pyloric Sphincteric Cylinder in Health and Disease

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Chapter 36 (page 181)

Peutz-Jeghers Syndrome

The three salient features of this syndrome are polyp formation in the gastrointestinal tract, mucocutaneous melanin pigmentation and inheritance through an autosomal dominant gene (Morson and Dawson l979). The polyps, which are hamartomas, are most common in the small bowel, but not infrequently occur in the stomach and colon. The condition usually presents in childhood with attacks of recurrent colic due to intussusception caused by the small bowel polyps, or it may present as iron deficiency anaemia due to haemorrhage. Macroscopically the polyps may be sessile but not infrequently they are pedunculated with short, broad pedicles (Morson and Dawson l979). Microscopically there is proliferation of the muscularis mucosae to form a branching pattern, clothed with essentially normal gastric mucosa.

The following case is an example:

Case Report

Case 36.2. A.P., girl aged 16 years, was admitted with a history of recurrent malaena (for which blood transfusions had been necessary) and symptoms of iron deficiency anaemia. On examination hyperpigmented "freckles" were seen around the mouth, on the buccal mucosa and on the fingers and feet. (Her father had similar areas of pigmentation). Other findings were a hypochromic microcytic anaemia and sedimentation rate of 20.0 mm/hr. Rectal examination revealed no abnormality but tests for stool occult blood were positive. Radiological examination showed two round, polypoid defects, each l.5cm in diameter, within the confines of the pyloric sphincteric cylinder (Fig 36.2A). The distal margins of the defects were 1.0 and 3.0cm respectively from the pyloric aperture; no stalks were seen. During contraction of the cylinder the defects moved in an orad direction, the distance from their distal margins to the pylorus increasing to 4.0cm and 5.2cm respectively (Fig 36.2B). These movements occurred regularly during the examination.

Fig. 36.2 Case A.P. Peutz-Jeghers syndrome. A Sessile polyps close to pyloric aperture in relaxed pyloric sphincteric cylinder.
Fig. 36.2 B.Sphincteric cylinder contracted. The polyps have moved in an orad direction

A number of polyps were also seen in the second and third parts of the duodenum, at the duodeno-jejunal junction and in the upper jejunum. A double-contrast barium enema examination revealed no abnormality in the colon.

At endoscopy the presence of 2 sessile prepyloric mucosal polyps was confirmed. In addition 2 smaller but similar polyps were seen in the body of the stomach; none of the polyps had a stalk. Because of technical complications biopsies were not done. A clinical and radiological diagnosis of Peutz-Jeghers syndrome was made. While under investigation symptoms of upper small bowel obstruction supervened, necessitating a laparotomy. A jejuno-jejunal intussusception was found and reduced. Gastrostomy, duodenostomy and jejunostomy were done at which polyps were removed from the stomach, duodenum and jejunum.

The histology of the gastric polyps showed prominent, branching processes of muscularis mucosae with overlying columnar cell epithelium, without evidence of dysplasia. The diagnosis of hamartomatous polyps of the Peutz-Jeghers type was made. The patient made an uneventful recovery.

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