Paediatric Imaging

Neuronal dysplasia

an ill understood condition in which intestinal peristalsis is deficient. Children present with variable symptoms which include acute abdominal distension, dehydration secondary to intraluminal fluid loss, poor food absorption and constipation. The disease may run an intermittent course. The radiographic features are also variable, but usually include distended fluid-filled loops of bowel with air fluid levels. The distension may be massive. Contrast studies show poor or incoordinated intestinal peristalsis with failure of ordered passage of the food bolus and stasis (Fig.1).

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Fig.1

An infant who presented with abdominal distension at birth. She had complete failure of food absorption. This contrast study, one and a half hours after instillation of contrast into the stomach, shows a dilated, featureless small bowel secondary to neuronal dysplasia. This is one pattern seen on imaging.
Neuronal dysplasia, Fig.1