Presentation

Spontaneous, focal or isolated intestinal perforation (SIP, FIP, IIP) is a disease clinically related to necrotizing enterocolitis (NEC) that tends to occur primarily in very low birth weight (VLBW, birth weight less than 1500 g) and extremely low birth weight (ELBW, birth weight less than 1000 g) premature infants [1]. Identified risk factors include early postnatal indomethacin, steroids and the synergistic effect of both [2]. Although it is not clear whether enteral Staphylococcus epidermidis and Candida are causative agents, their presence in peritoneal cultures is high [3]. Chorioamnionitis has also recently been identified as a risk factor for SIP.

What differentiates spontaneous intestinal perforation from classic necrotizing enterocolitis?

Whether NEC and SIP are the same disease is controversial. While theoretically SIP can be differentiated from NEC preoperatively by the substantial pneumoperitoneum and absence of pneumatosis associated with SIP, many patients with classic NEC will not have pneumatosis [4]. Patients with SIP may have diminished signs of systemic sepsis.

NEC can affect the entire small and large bowel but SIP typically occurs in the terminal ileum. With the exception of contamination related to the localized perforation, during exploration the rest of the intestine in SIP patients appears normal while with NEC it appears diffusely involved and patchy.

(see Necrotizing Enterocolitis Presentation)