Feeding difficulties and foregut dysmotility in Noonan's syndrome
N Shah, M Rodriguez, D St Louis, K Lindley, P J Milla
Great Ormond Street
Hospital and Institute of Child Health, London WC1 1EH, UK
Correspondence to: Dr Shah.
Accepted 20 November
1998
PURPOSE
Noonan's
syndrome is a common dysmorphic syndrome in which failure to thrive and
gastrointestinal symptoms are frequent but poorly understood.
DESIGNTwenty five
children with Noonan's syndrome were investigated by contrast
radiology, pH monitoring, surface electrogastrography (EGG), and
antroduodenal manometry (ADM).
RESULTSSixteen had
poor feeding and symptoms of gastrointestinal dysfunction. All 16 required tube feeding. Seven of 25 had symptoms of foregut dysmotility
and gastro-oesophageal reflux. In the most symptomatic children (four
of seven) EGG showed fasting frequency gradient loss along the stomach
fundus and pylorus with antral postprandial frequency loss. ADM showed
shortened fasting cycle length, with abnormal phase III and shortened
postprandial activity containing phasic contractions.
IMPLICATIONSGastroduodenal
motor activity was reminiscent of 32-35 week preterm patterns. The
feeding difficulties appear to resolve as gut motility matures. In
Noonan's syndrome, feeding problems appear to be the result of delayed
gastrointestinal motor development.
Keywords: Noonan's syndrome; failure to thrive; foregut dysmotility; gastro-oesophageal reflux
© 1999 by Archives of Disease in Childhood
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