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Oral Presentation - 11
Congenital Pyloric Atresia: Presentation, Management, and Outcomes – A Single-Center Experience
S Mani, N Kechiche, R Lamiri, N Boukhrissa, A Ksia, I Krichene, M Mekki, M Belghith, L Sahnoun
Fattouma Bourguiba Hospital Monastir Tunisia
Introduction:
Congenital pyloric atresia (CPA) is a rare anomaly, accounting for approximately 1% of all intestinal atresias, with an incidence of about 1 in 100,000 live births. CPA can occur as an isolated condition or be associated with other congenital abnormalities. The treatment is primarily surgical, but the prognosis remains poor, especially when associated with epidermolysis bullosa (EB). This study aims to share our experience in the management of CPA.
Patients and Methods:
We conducted a retrospective study of patients diagnosed and operated for CPA, between 1990 and 2019.
Results:
Fourteen cases of CPA were reviewed, with a male predominance (9 boys, 5 girls). The mean age at presentation was 1.8 days. The most common symptom was non-bilious vomiting. Five patients had associated anomalies: three with epidermolysis bullosa (EB), one with Down syndrome, and one with multiple intestinal atresias. A family history was positive in one case, affecting three siblings. Abdominal X-rays showed gastric dilatation with no gas in the remainder of the intestinal tract in 13 cases, and pneumoperitoneum in one. Upper gastrointestinal contrast studies revealed a dilated stomach with significant constriction at the prepyloric region in two cases. Surgical treatment involved excision of the diaphragm and Heineke-Mikulicz pyloroplasty in eight patients, while the remaining six underwent gastroduodenostomy. The overall mortality rate was 64%.
Conclusion:
CPA is a rare condition with a poor prognosis, particularly when associated with other congenital anomalies such as EB and multiple intestinal atresias. Early diagnosis and appropriate surgical intervention are crucial, but the prognosis remains guarded, highlighting the need for further research into improving outcomes for these patients.