Background: Anterior cutaneous nerve entrapment syndrome (ACNES) is a frequently overlooked cause of chronic abdominal pain in children. Currently, both nonsurgical and surgical treatment options are available to treat this disease. The objective was to give insight into the success rate of different treatment strategies for children with ACNES, and provide treatment recommendations for physicians based on the published evidence. Method: A literature search of PubMed, Embase.com and the Wiley/Cochrane Library was conducted for studies published up to 25 February 2020. Randomized controlled trials, prospective or retrospective cohort studies, meta-analyses and literature reviews describing the outcome of different treatment strategies for children (b18 years old) with ACNES with a follow-up duration of at least four weeks were included. Results: Six studies, involving 224 patients, were included with an overall quality reported to be between fair and poor. Treatment success of local injections with an anesthetic agent into the trigger point ranged from 38% to 87% with a follow-up ranging from 4 weeks to 39 months. In addition, treatment success of anterior neurectomy ranged from 86% to 100%, with a follow-up duration ranging from 4 weeks to 36 months. Conclusion: A step-up treatment strategy should be applied when treating pediatric patients with ACNES. This strategy starts with an injection with a local anesthetic agent, reserving surgery (anterior neurectomy) as a viable option in case of persistent pain. Level of evidence: II.

a b s t r a c tBackground: Anterior cutaneous nerve entrapment syndrome (ACNES) has been described as a possible cause for chronic pain in the pediatric population. However, the exact pathophysiology of ACNES is unknown. It may be caused by compression or traction of cutaneous nerve branches of intercostal nerves, or it may be the result of an infection. Therefore, we present histopathological evidence to determine the pathophysiology of ACNES. Methods: A total of seven pediatric patients underwent a neurectomy for ACNES. All specimens were sent for histopathological evaluation, including immunohistochemical staining, to evaluate if there were any signs of infection, inflammation or compression. Results: Seven out of seven (100%) histopathological specimens showed non-specific nerve degeneration. Immunohistochemical evaluation showed there were several CD68-positive macrophages present in the specimens. Four out of seven (57%) specimens showed the presence of a few CD3-positive T-cells, however, this was not suggestive for inflammation or infection. Conclusion: Our study supports the hypothesis that ACNES is caused by compression of the nerves rather than inflammation. Level of evidence: III