The child underwent Ramstedt s myotomy which relieved the symptoms. The parents were second cousins. Unfortunately, the child died of septicemia before he could be operated upon. Histopathology of the pancreas showing ductular insular complexes and large hyperchromatic beta cells with nuclear enlargement. Barium study showing pyloric stenosis.
Arrow shows the site of obstruction.
Discussion Nesidioblastosis, a term first coined by Laidlaw in 1938, characterizes a condition with diffuse proliferation of disordered islet cells from pancreatic ductal cells. This term has now been replaced by persistent hyperinsulinemic hypoglycemia of infancy. The present case satisfied all the above mentioned criteria in addition to the histological features. The present case appears to have autosomal recessive inheritance due to normal but consanguineous parents and another affected sibling. The point of interest in this patient was the presence of congenital hypertrophic pyloric stenosis, an association, which has not been described earlier, to the best of our knowledge.