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.
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http://dx.doi.org/10.1053/jpsu.2000.9340 | DOI Listing |
Clin Case Rep
December 2024
Department of Dermatology, Institute of Medicine University of Tsukuba Ibaraki Japan.
When surgery is performed in patients with EB, risks of blisters and epidermal detachment are always present. The Heineke-Mikulicz pyloroplasty cannot always be performed because of anatomical constraints. In such cases, it is necessary to select a more time-consuming surgical procedure (i.
View Article and Find Full Text PDFGlob Med Genet
December 2024
Division of Genetics and Metabolism, Department of Pediatrics, University of Florida College of Medicine, Gainesville, Florida, United States.
We report a 7-year-old girl born with pyloric atresia but without congenital epidermolysis bullosa or skin fragility. Nail dysplasia developed at age 8 months and throughout childhood she suffered from onycholysis and mild nail hypertrophy. Whole-exome sequencing demonstrated biallelic mutations in alpha6 integrin (ITGA6): p.
View Article and Find Full Text PDFEur J Med Genet
December 2024
Acibadem Mehmet Ali Aydinlar University, Faculty of Medicine, Department of Pediatrics, Division of Pediatric Genetics, Rare Diseases and Orphan Drugs Application and Research Center (ACURARE), Istanbul, Turkey. Electronic address:
BMJ Case Rep
July 2024
MNR Medical College and Hospital, Sangareddy, Telangana, India.
A term male baby was born vaginally to a primi mother. An antenatal ultrasound revealed polyhydramnios and a distended stomach in the baby. At birth, the baby had well-defined areas of peeling skin on the face and blisters on the forearm region.
View Article and Find Full Text PDFJAAD Case Rep
August 2024
Department of Dermatology and Venereology, Faculty of Medicine, Public Health, and Nursing, Universitas Gadjah Mada/Dr Sardjito General Hospital, Yogyakarta, Indonesia.
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