WOFAPS 2025 8th World Congress of Pediatric Surgery

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From diagnosis to outcome: managing meconium ileus in cystic fibrosis – A three-patient report

Arije Zouaoui, Fatma Thamri, wiem Hamouda, Yosra Kerkeni, Riadh Jouini
Pediatric Surgery Department, Children's Hospital Bechir Hamza, Tunis, Tunisia

Introduction:
Cystic fibrosis (CF) is a severe autosomal recessive disorder affecting exocrine glands, leading to multisystem involvement. Gastrointestinal manifestations, particularly meconium ileus, represent the first clinical sign in neonates. Early clinical recognition and contrast enema can permit non-operative resolution. However, surgical intervention remains essential in complicated forms. This study reports three cases of neonatal meconium ileus, highlighting diagnostic difficulties, therapeutic strategies, and prognostic outcomes.

Case Presentation:
Case 1: A 4-day-old neonate presented with clinical features of meconium ileus confirmed by water-soluble contrast enema. Failure of non-operative treatment necessitated terminal ileostomy. The postoperative period was marked by recurrent severe bronchopulmonary infections, resulting in death at 11 months of age.
Case 2: A 45-day-old infant underwent emergency surgery for acute small bowel obstruction due to meconium ileus. Intraoperative decompression was achieved using gastrografin lavage via enterotomy. The infant’s clinical course was complicated by severe bronchopulmonary infections leading to death at 6 months.
Case 3: A 2-day-old neonate was admitted for meconium ileus complicated by small bowel volvulus discovered intraoperatively. Surgical management involved resection of necrotic bowel loops, water-soluble contrast lavage, and primary anastomosis. Postoperative recovery was uneventful with favorable short-term outcomes.

Conclusion:
Despite significant advances in genetic diagnosis and medical management of cystic fibrosis, meconium ileus remains a serious complication in the neonatal period. Therapeutic management requires early diagnosis and individualized treatment based on disease severity and complications. Prognosis is mainly determined by respiratory and digestive complications, which continue to pose significant morbidity and mortality risks in affected infants.

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