The term infantile hypertrophic pyloric stenosis describes the condition well.  The pyloric sphincter undergoes muscular hypertrophy with resultant gastric outlet obstruction.  Although the clinical features and presentation are widely recognized following Hirschsprung’s description in 1888, the exact etiology remains obscure.  Genetic factors with a single dominant gene have been implicated, with a 7% incidence in children of affected parents.


Clinical Presentation-The typical infant begins to vomit around the third week of life.  The vomitus contains curdled milk or formula and excessive mucus.  It may also assume a coffee ground appearance from the stasis gastritis and subclinical bleeding.  Bile is conspicuously absent and its presence suggests other etiology such as distal intestinal obstruction, sepsis or gastroesophageal reflux (chalasia).

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