Pathophysiology
Clinical meaning
Hypertrophic pyloric stenosis (HPS) is a condition of infancy in which progressive hypertrophy and hyperplasia of the pyloric sphincter muscle causes gastric outlet obstruction. The pylorus is the muscular valve between the stomach and the duodenum; as the circular muscle layer progressively thickens, the pyloric channel narrows until it becomes nearly completely obstructed, preventing gastric contents from passing into the duodenum. This results in forceful, projectile, non-bilious vomiting (the obstruction is proximal to the ampulla of Vater where bile enters the duodenum, so vomited material contains no bile). The condition typically presents between 2-8 weeks of age (most commonly at 3-5 weeks), with firstborn males affected 4-5 times more often than females. The infant is characteristically hungry immediately after vomiting ('hungry vomiter') because they are not nauseated โ the stomach simply cannot empty. Progressive vomiting leads to dehydration, weight loss, and a characteristic metabolic alkalosis: loss of hydrochloric acid (Hโบ and Clโป) from vomiting causes hypochloremic metabolic alkalosis, and the kidneys paradoxically excrete potassium and hydrogen ions to retain sodium ('paradoxical aciduria'). On physical examination, an 'olive-shaped' mass may be...
