Shabih Manzar, MD, MPH; Faiza Javed, MD

Pneumoperitoneum is a radiological diagnosis (ICD-10-Code K66.8), while necrotizing enterocolitis (NEC)- [ICD-10 Code P77 NEC of newborn, P77.1 Stage 1 NEC in newborn, P77.2 Stage 2 NEC in newborn, P77.3 Stage 3 NEC in newborn, P77.9 NEC in newborn, unspecified)] and spontaneous intestinal perforation (SIP)- ICD-10-Code K63.1, are clinical, radiological, and pathological diagnoses.

Pneumoperitoneum in this 5-day-old, 26-week infant could be due to SIP or NEC. Based on the Bell staging criteria, the infant could be classified as Bell Stage III (or modified Bell stage IIIB) 1 due to pneumoperitoneum. The differentiation is complex and requires direct visualization of the affected bowel and histopathological examination (2). The current data suggest increasing SIP and decreasing NEC among preterm infants (3). For bedside diagnosis of NEC and SIP, two out of three and three out of five rules have been suggested (3).

The infant underwent exploratory laparotomy. After removing 22 cm of the small intestine, a double barrel jejunostomy was done. The pathology showed chronic inflammatory and reparative changes compatible with NEC. 

NEC has many definitions. Patel et al. (4) summarized eight definitions of NEC with similarities and differences in clinical signs and radiographic features. They highlighted the importance of a global consensus on defining NEC to improve NEC research and outcomes. 

It remains a dilemma for NICU providers to diagnose and differentiate between SIP and NEC. Although they may mimic each other, the impact is very different. Juhl et al. (2) reported 60% mortality with stage 3 NEC while 18% with SIP. Also, NEC is a quality indicator associated with neuro-developmental outcomes (5-7). 

Artificial intelligence might help in the future, but we need more literature and research. Developing clinically valid and relevant AI requires large, high-quality multimodal datasets (8). 

References: 

1. Huda S, Chaudhery S, Ibrahim H, Pramanik A. Neonatal necrotizing enterocolitis: Clinical challenges, pathophysiology and management. Pathophysiology. 2014;21(1):3-12. doi:10.1016/j.pathophys.2013.11.009 
2. Juhl SM, Hansen ML, Gormsen M, Skov T, Greisen G. Staging of necrotising enterocolitis by Bell’s criteria is supported by a statistical pattern analysis of clinical and radiological variables. Acta Paediatr. 2019;108(5):842-848. doi:10.1111/apa.14469 
3. Swanson JR, Hair A, Clark RH, Gordon PV. Spontaneous intestinal perforation (SIP) will soon become the most common form of surgical bowel disease in the extremely low birth weight (ELBW) infant. J Perinatol. 2022;42(4):423-429. doi:10.1038/s41372-022-01347-z 
4. Patel RM, Ferguson J, McElroy SJ, Khashu M, Caplan MS. Defining necrotizing enterocolitis: current difficulties and future opportunities. Pediatr Res. 2020;88(Suppl 1):10-15. doi:10.1038/s41390-020-1074-4 
5. Doucette SM, Kelly EN, Church PT, Lee S, Shah V; Canadian Neonatal Network (CNN) Investigators and CNFUN Investigators and Steering Committee. Association of inotrope use with neurodevelopmental outcomes in infants <29 weeks gestation: a retrospective cohort study. J Matern Fetal Neonatal Med. 2022;35(25):6044-6052. doi:10.1080/14767058.2021.1904872 
6. Canvasser J, Patel RM, Pryor E, et al. Long-term outcomes and life-impacts of necrotizing enterocolitis: A survey of survivors and parents. Semin Perinatol. 2023;47(1):151696. doi:10.1016/j.semperi.2022.151696 
7. Mavis SC, Gallup MC, Meyer M, et al. A quality improvement initiative to reduce necrotizing enterocolitis in high-risk neonates. J Perinatol. 2023;43(1):97-102. doi:10.1038/s41372-022-01476-5 
8. Sitek A, Seliga-Siwecka J, Płotka S, et al. Artificial intelligence in the diagnosis of necrotising enterocolitis in newborns. Pediatr Res. 2023;93(2):376-381. doi:10.1038/s41390-022-02322-2

Disclosure: The authors has no conflicts of interests to disclose.