Spontaneous intestinal perforation followed by necrotizing enterocolitis in an extremely low birth weight neonate: case report and review of the literature

Abstract

Background: Spontaneous intestinal perforation (SIP) is usually an isolated perforation occurring most commonly in the terminal ileum of neonates with very low birth weight (VLBW) and extreme low birth weight (ELBW) of unknown etiology. It can be identical to perforated necrotizing enterocolitis (NEC) both clinically and radiologically raising a diagnostic challenge. In such cases, the differentiation is only possible by laparotomy and histopathology. This is important because the differentiation has management and prognostic consideration. The authors are presenting a case of SIP which was followed by NEC after 4 days only of its onset. Case presentation: The authors report on a unique case of SIP which was followed by perforated NEC after 4 days of its onset in a 28-week gestational age with an ELBW. On the 3rd day of life, abdominal X-ray showed a significant pneumoperitoneum with neither pneumatosis intestinalis nor portal venous gas. The diagnosis of SIP was made at laparotomy. On the 4th post-operative day the patient developed pneumoperitoneum again due to perforated NEC as confirmed later on by histology. In this odd case (which we believe to be the youngest in the literature), the sequence of SIP followed after few days by perforated NEC is presented. Methods of differentiating these two conditions and their management are discussed. Conclusion: SIP should be differentiated from perforated NEC by laparotomy and histopathology if this is not possible clinically and radiologically, and the patient is stable. Primary peritoneal drainage (PPD) is reserved as a step to stabilize the patient only in both conditions.