Appendiceal perforation: A rare case of massive neonatal pneumoperitoneum

A pre-term newborn male was admitted to the pediatric cardiac intensive care unit with severe left ventricular and aortic hypoplasia. On day-of-life-13, massive pneumoperitoneum was incidentally identified on a chest radiograph after central line placement. Emergent exploration with thorough examination revealed only a focal perforation at the appendiceal tip. Standard appendectomy was performed; pathology showed appendiceal perforation with focal mucosal ulceration without inflammation.Literature review demonstrated neonates with appendiceal perforation have nonspecific symptoms at presentation (emesis, poor feeding, abdominal erythema) which may be difficult to distinguish from other etiologies of perforation. Infants with concomitant cardiac pathology are at increased risk for spontaneous intestinal perforation due to malperfusion. However, appendiceal perforation does not lead the differential for neonatal massive pneumoperitoneum; rather, large volume pneumoperitoneum typically results from gastric perforation. The true incidence is unknown with fewer than 10 reported cases; importantly, the literature often lacks distinction between spontaneous-perforation versus appendicitis-with-perforation.Given its rarity, discrete examination of the appendix and appendiceal pathology may be overlooked when evaluating neonates with pneumoperitoneum during a standard of care operative exploration. Particularly in patients with high-risk comorbidities where gastric or ileal pathology is not appreciated after thorough exploration, appendiceal perforation should be considered.

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