BACKGROUND: The standard method of surgical correction of pyloric atresia is gastro-duodenostomy. The authors report a case of pyloric atresia associated with junctional epidermolysis bullosa, treated with a new technique of pyloric sphincter reconstruction by gastric and duodenal mucosa cul-de-sacs advancement and end-to-end anastomosis. METHODS: The patient was a premature 2,100-g baby girl. X-ray showed gastric dilatation suggesting a congenital gastric obstruction. At surgery a pyloric atresia was found, with the appearance of a well-vascularized solid cord about 1.5 cm long. By longitudinal pyloromyotomy the cul-de-sacs of gastric and duodenal mucosa were reached and then isolated in the respective gastric and duodenal sides to obtain better mobilization. The mucosal cul-de-sacs, thus mobilized, were advanced easily into the pyloric canal, opened longitudinally, and were sutured together using end-to-end anastomosis. The longitudinal pyloromyotomy then was closed diagonally above the reconstructed pyloric neocanal. RESULTS: The postoperative course was uneventful: oral feeding was started on the 11th postoperative day. At 4 year follow-up the child was well; no gastrointestinal disorders were present, confirmed by x-ray barium meal and by HIDA technetium Tc 99m hepatic scintiscan, which excluded any bilious duodeno-gastric reflux. CONCLUSION: This technique of pyloric sphincter reconstruction allows preservation of the pyloric sphincter, whose sphincter muscular layer, although hypoplastic, is present in cases of pyloric atresia.
BACKGROUND: The standard method of surgical correction of pyloric atresia is gastro-duodenostomy. The authors report a case of pyloric atresia associated with junctional epidermolysis bullosa, treated with a new technique of pyloric sphincter reconstruction by gastric and duodenal mucosa cul-de-sacs advancement and end-to-end anastomosis. METHODS: The patient was a premature 2,100-g baby girl. X-ray showed gastric dilatation suggesting a congenital gastric obstruction. At surgery a pyloric atresia was found, with the appearance of a well-vascularized solid cord about 1.5 cm long. By longitudinal pyloromyotomy the cul-de-sacs of gastric and duodenal mucosa were reached and then isolated in the respective gastric and duodenal sides to obtain better mobilization. The mucosal cul-de-sacs, thus mobilized, were advanced easily into the pyloric canal, opened longitudinally, and were sutured together using end-to-end anastomosis. The longitudinal pyloromyotomy then was closed diagonally above the reconstructed pyloric neocanal. RESULTS: The postoperative course was uneventful: oral feeding was started on the 11th postoperative day. At 4 year follow-up the child was well; no gastrointestinal disorders were present, confirmed by x-ray barium meal and by HIDA technetium Tc 99m hepatic scintiscan, which excluded any bilious duodeno-gastric reflux. CONCLUSION: This technique of pyloric sphincter reconstruction allows preservation of the pyloric sphincter, whose sphincter muscular layer, although hypoplastic, is present in cases of pyloric atresia.