EN
TÜRKÇE
Oral Presentation - 13
Pyloric atresia: clinical presentation, associated abnormalities and surgical management strategies.
Z Nedra, M Marwa, BS Nessrine, BH Samia, K Amine, B Mohssen, M Mongi, S Lassaad
Pediatric surgery department, Fattouma Bourguiba Hospital.
Purpose: Pyloric atresia (PA) is a rare congenital anomaly. PA may occur as an isolated condition or associated with other abnormalities, the most common being epidermolysis bullosa (EB). The aim of this study is to describe clinical and management characteristics of this disorder through a 34-year review of all patients with PA.
Method: This is a retrospective study of patients who underwent surgery for PA in the department of pediatric surgery of Monastir between 1990 and 2024.
Results: Twenty-four cases (16 Males, 8 Females) were treated. Fourteen patients were full term and the remaining 10 were premature. Their mean birth weight was 2.1 kg. The average of age at presentation was 2,6. Polyhydramnios was seen in 13 patients (54%). One family has three affected siblings. The main presenting symptoms were non-bilious vomiting in 90% of cases. Radiological findings were a large gastric air bubble with no gas beyond in all cases. Gastric perforation was observed in one patient and was completely repaired. Ten patients underwent excision of the diaphragm and Heineke-Mikulicz pyloroplasty and 14 patients had gastroduodenostomy. Isolated PA was seen in 13 (54%), 11 had an associated condition: epidermolysis bullosa (EB) in 4 (16%) and down syndrome in 1 (4%), jejunoileal atresia in 3 (12.5%), colonic atresia in 1 (4%), anorectal malformation in 1 (4%), annular pancreas in 1 (4%). One patient was reoperated for recurrent vomiting with gastric dilatation and absence of gas in the rest of the intestinal tract on abdominal X-ray. Post-operative mortality rate was 41%.
Conclusion: PA is uncommon. Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation, that should be considered when managing a newborn with a gastric outlet obstruction. Isolated PA carries a good prognosis. Association of PA with other congenital anomalies carries a poor prognosis.