The Pyloric Sphincteric Cylinder in Health and Disease

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Chapter 32 (page 155)

Chapter 32

Gastro-oesophageal Reflux Disease (GERD) and the Pyloric Sphincteric Cylinder

Hiatus Hernia in Infants

Roviralta (l951) described 3 cases of partial thoracic stomach in infants associated with hypertrophic pyloric stenosis (IHPS), and called the combination the phreno-pyloric syndrome. It was believed that raised intragastric pressure secondary to the obstruction at the pylorus forced the stomach into the chest. Among 115 children with a partial thoracic stomach, Astley and Carré (l954) encountered 5 who also had hypertrophic pyloric stenosis, while another 3 had "infantile pylorospasm". The pylorospasm in all 3 cases was described as an inconstant narrowing of the pyloric "antrum", (Chap. 20), the radiographic appearance simulating IHPS to such a degree that Astley (l956) later called them cases of pseudo-hypertrophic pyloric stenosis. The symptomatology suggested that initially there was gastroesophageal reflux due to a partial thoracic stomach, followed by the superimposition of hypertrophic stenosis a few weeks later. Thus vomiting commenced soon after birth, and at the age of 2 to 3 weeks the symptoms and signs of hypertrophic stenosis, such as projectile vomiting, visible peristalsis and a palpable mass were superadded.

Forshall (l955) described the findings in 93 infants with gastroesophageal reflux and hiatus hernia. In 58 cases the cardia was incompetent but situated below the diaphragm. Eight of these required Ramstedt's operation for IHPS, while others had visible gastric peristalsis with temporary palpable masses in the pyloric region.

Astley (l956) stated that the association of hiatus hernia and hypertrophic pyloric stenosis was not a very common combination, but that the frequency was enough to suggest something more than a chance occurrence. He found no ready explanation for the association of these two conditions.

Stewart (l960), in discussing a paper by Herrington (l960), was impressed by the frequency of pyloric hypertrophy in cases of hiatus hernia; in many instances it resembled infantile hypertrophic pyloric stenosis.

Johnston (l960), in a series of 76 cases of hiatus hernia in childhood, found that 8 (10.5 percent) also had hypertrophic pyloric stenosis. Some of those without hypertrophic stenosis showed visible gastric peristalsis with forcible or even projectile vomiting, which to him was an indication of a gastric emptying disorder, giving rise to functional pyloric obstruction. It was reasoned that this raised the intragastric pressure, thus forcing the cardia into the chest.

Bowen (l988) pointed out that criteria for diagnosing hiatus hernia in infants remained unsettled, but that it was generally agreed that the retrograde passage of material from the stomach into the oesophagus was the crux of the matter, regardless of whether or not a hiatal hernia could be demonstrated convincingly.

In a number of infants we have noted a combination of hiatus hernia and IHPS; however, no systematic study was done in infants to determine in which percentage of hiatus hernia cases IHPS also occurred. The following is an example of one of our cases:

Case Reports

Case 32.1. E.B., 5 weeks old female infant, was admitted with a history of vomiting after feeds and recurrent bilateral pneumonia. Radiographic examination showed a severe, constant narrowing of the pyloric sphincteric cylinder, with a "string sign" typical of IHPS (Fig. 32.1A). The gastro-oesophageal junction was patulous with free and persistent gastro-oesophageal reflux, diagnosed radiographically as a sliding hiatus hernia (Fig. 32.1B). Some aspiration of refluxed barium occurred. At operation the next day a pyloric "olive" measuring approximately 2.3 cm x 0.8 cm, typical of IHPS, was found. Ramstedt pyloromyotomy was done; post-operatively vomiting stopped and the patient made an uneventful recovery.

Fig. 32.1 A,B. Case E.B. A Constant narrowing of pyloric sphincteric cylinder with string sign (arrows), typical of idiopathic hypertrophic pyloric stenosis. B Patulous gastro-oesophageal junction with free reflux (arrow) B

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