nesidioblastosis is a pathological condition caused by neo - differentiation of islets of langerhans from pancreatic ductal epithelium . this condition later renamed as persistent hyperinsulinemic hypoglycaemia of infancy ( phhi ) exists in two forms . one corresponds to a focal pancreatic adenomatous hyperplasia ( focal phhi ) and the other is characterized by a diffuse cell abnormality ( diffuse phhi ) . the underlying pathology and treatment strategies are different for both the types however differentiation by clinical and biochemical parameters is not feasible . no definite role of imaging to differentiate diffuse versus focal condition has been reported in the literature . the child was born at 35 weeks of gestation , with breathing difficulty since birth . on investigation the child was found to be hypoglycemia ( blood glucose level = 16.8 mg / dl ) and hyperinsulinemia with serum insulin levels of 149.70 iu / ml ( n = 2.0 iu / ml ) . the child required intravenous glucose infusion up to 10 mg / kg / min and oral feeds fortified with glucose to maintain euglycemic state . ga-68 dotatate positron emission tomography / computerized tomography ( pet / ct ) scan showed diffusely increased tracer uptake in the entire pancreas with no abnormal tracer uptake anywhere else in the body , suggestive of a diffuse variant of nesidioblastosis [ figure 1 ] . the child is currently on injection octreotide but not fit for definitive surgical management ( near total pancreatectomy ) . ga-68 dotatate positron emission tomography / computed tomography ( ct ) ( a ) maximum intensity projection ( b ) transaxial ct ( c ) transaxial fused images showing diffusely increased tracer uptake in the entire pancreas ( arrow ) with no abnormal tracer uptake elsewhere in the body , suggestive of a diffuse variant of nesidioblastosis laidlaw in 1938 first identified the disease and coined the term nesidioblastosis to describe the neodifferentiation of islets of langerhans from pancreatic ductal epithelium . one corresponds to a focal pancreatic adenomatous hyperplasia ( focal phhi ) and the other is characterized by a diffuse cell abnormality ( diffuse phhi ) . these two forms could not be differentiated by clinical or biochemical data , although their underlying pathological mechanisms and the treatment remains totally different . f18-fluoro - dihydroxyphenylalanine ( f - dopa ) pet scan has been used to detect the hyperfunctional pancreatic islet tissue and to differentiate between focal and diffuse phhi with a reported accuracy of 96% in diagnosing focal or diffuse disease and 100% in localizing the focal lesion . the principle behind the use of f - dopa pet in phhi is that neuroendocrine pancreatic cells have an affinity for taking up f - dopa and decarboxylate it into dopamine through aromatic amino acid decarboxylase . 68-ga dotatate specifically binds to the sstr type ii , which are highly concentrated in the pancreatic islet cells . previously only one case report was published where ga-68 dotatoc pet scan was used to differentiate focal versus diffuse nesidioblastosis with limited success . this is the first case where ga-68 dotatate pet scan had been used to successfully differentiate focal versus diffuse nesidioblastosis and thus help in tailoring the management in the infant .
the authors describe a 50 days old pre - term infant with persistent hyperinsulinemic hypoglycemia of infancy in whom ga-68 dotatate positron emission tomography / computerized tomography scan showed diffusely increased tracer uptake in the entire pancreas with no abnormal tracer uptake anywhere else in the body , suggestive of a diffuse variant of nesidioblastosis .