WOFAPS 2025 8th World Congress of Pediatric Surgery

Ana Isabel Barros, Mário Rui Correia, Maria Luisa Gaspar, Catarina Sousa-Lopes, Jorge Cagigal, Joana Sinde, José Banquart-Leitão, Ana Sofia Marinho

Department of Pediatric Surgery of Unidade Local de Saúde de Santo António

Introduction: Gastric volvulus (GV) is a rare condition characterized by abnormal stomach rotation, and classified as organoaxial, mesenteroaxial, or mixed. Its association with omphalocele is exceedingly rare, with only one case previously reported in the literature.

Case presentation: We present a preterm male neonate (35+1 weeks gestation) with prenatal diagnosis of Beckwith-Wiedemann syndrome and omphalocele, delivered via cesarean section due to worsening maternal preeclampsia (birth weight: 2050g). Within the first 24-hours of life, omphalocele’s primary closure was successfully performed. Bowel transit was established, and oral feeding initiated uneventfully by postoperative day (POD) 10. On POD 27, developed intermittent episodes of respiratory distress, necessitating non-invasive mechanical ventilation. Notably, these were exacerbated postprandially and associated with epigastric distension. Sepsis and pulmonary workups were unremarkable. Abdominal radiograph showed a distended stomach with an atypical rounded contour. Upper gastrointestinal contrast study demonstrated an antrum superiorly rotated and fundus inferiorly displaced, consistent with organoaxial GV. Exploratory laparoscopy confirmed the diagnosis and identified absent gastric fixation ligaments. As liver-abdominal wall adhesions limited safe port placement, laparotomy conversion was performed. Gastropexy was achieved with three sutures: one between the gastric fundus and diaphragm; two between the anterior gastric wall and anterior abdominal wall. Oral feeding resumed on POD 1 post-gastropexy with initial irregular tolerance, associated with discomfort and abdominal distension, attributed to non-invasive ventilation. After ventilatory optimization, full feeds were achieved. At reporting time, remains hospitalized for respiratory support unrelated to the surgical intervention.

Conclusion: This case illustrates a rare association of GV with omphalocele in a neonate with Beckwith-Wiedemann syndrome. It highlights the diagnostic challenge of nonspecific postoperative respiratory symptoms and emphasizes considering GV in cases of postprandial respiratory distress and epigastric distension, especially in neonates with abdominal wall defects. Prompt imaging and surgical management are crucial to avoid ischemic complications and improve outcomes.