Is hypertrophic pyloric stenosis a secondary disease?

Abstract

Hypertrophic pyloric stenosis (HPS) is the most common gastrointestinal obstructive disease in infants which requires surgery. Typical presentation is at 4-6 weeks of life but can present very earlier or congenitally. Here we present 3 cases of HPS which presented in the first day of life with  co-existing malrotation, annular pancreas, and duodenal atresia. The exact etiology of HPS is not fully understood, however, genetic and  maternal factors, hormonal factors, abnormalities of various components of the pyloric  muscle, growth factors, extracellular matrix elements, nerve and ganglion cells synapses, nerve supporting cells, neurotransmitters and interstitial cells, drugs, and feeding have been implicated. In the HPS  concomitant with a distal obstruction, mean that these obstructions may role in  pathogenesis of HPS or may exacerbate other factors to produce HPS in a very early age or congenitally. The purpose of this manuscript is to present three cases of HPS in the fi rst day of life with other GI anomalies. We hypothesize that the etiology of some cases of HPS may be secondary to distal intestinal obstructions especially when it presents very early in life,
or at least to concern a distal obstructions in this very early presentation.

Key words: Etiology, GI anomaly, hypertrophic pyloric stenosis