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2023

ANUÁRIO DO HOSPITAL
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HYPERTROPHIC PYLORIC STENOSIS ON A DOWN SYNDROME INFANT WITH GASTROSTOMY FOR LONG GAP OESOPHAGEAL ATRESIA

Marta Pinto, Catarina Ladeira, Daniel Virella, Laura Oliveira, Cristina Borges, Patrícia Rodrigues.

XLIV Jornadas Nacionais de Neonatologia. Évora. 2015. Poster

Background. Long-gap oesophageal atresia requires a gastrostomy for enteral feeding until the programmed delayed correction can be done safely. Hypertrophic pyloric stenosis (HPS), a frequent surgical condition in the first few months of life, has an atypical presentation in patients with oesophageal atresia and gastrostomy. Case Report. A male neonate, third sibling of a Portuguese family, was born with hydramnios and high prenatal risk for Down syndrome. Delivered at term by caesarean section, oesophageal atresia was promptly detected and he was referred to a tertiary neonatal medico-surgical unit. Diversive gastrostomy was performed in the second day of life due to long gap oesophageal atresia without tracheal fistulae. Full enteral feeding was easily achieved. At day 50, gastric and oesophageal endoscopies were performed to reassess the gap length (3-4 cm). During the following week, feeding tolerance increasingly deteriorates and leakage of the gastrostomy develops. At day 61, he had the equivalent for projectile, non-bilious vomiting through the gastrostomy. Sonography reveals elongation (17 mm) and thickening of the muscular layer of the pylorus (4 mm). After pyloromiotomy the following day, full enteral feeding was again achieved. Programmed delayed correction of long gap oesophageal atresia continued as planned. Discussion. The causes of HPS are unknown, but genetic and environmental factors probably play a role. HPS has been reported to occur more frequently in patients with oesophageal atresia and tracheoesophageal fistula as compared to the general population. Vagal injury, performance of a gastrostomy, and transpyloric feeding tubes have been implicated in the increased incidence.

Palavras Chave: Down syndrome, gastrostomy, hypertrophic pyloric stenosis, long-gap oesophageal atresia, sonography.