Hypertrophic pyloric stenosis in a newborn with propionic acidaemia

Hypertrophic pyloric stenosis is exceedingly rare in the first week of life. This report describes a rare case of congenital pyloric stenosis in a newborn with propionic acidaemia. Although newborns with propionic acidaemia may present with feeding problems in the form of vomiting, associated congenital pyloric stenosis should be considered in the differential diagnosis. These patients should be investigated with abdominal ultrasound and or barium meal and once diagnosed, they should undergo pyloromyotomy