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Neurogenic appendicopathy: an underestimated disease—systematic review of the literature

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International Journal of Colorectal Disease Aims and scope Submit manuscript

Abstract

Purpose

Appendectomy is one of the most frequently performed surgeries worldwide, but neurogenic appendicopathy (NA) remains a poorly understood disease with controversial clinical management. The aim of this review was to obtain a clear definition of the disease and summarize its management.

Methods

We performed a systematic review of the literature on NA in PubMed, EMBASE, Web of Science, and Cochrane databases from inception to 19/01/2021 according to PRISMA statement standards. Eligibility criteria were original articles examining histopathology, clinical management, and/or follow-up of patients with NA. The literature review is complemented by a clinical case.

Results

In 40 articles, the estimated incidence of NA among appendectomies performed in patients with a suspicion of acute appendicitis (AA) was 10.4% (N = 740, range 1.8-32%). NA more frequently causes recurrent and longer lasting pain compared to AA; however, these diseases are usually not clinically or radiologically distinguishable. Based on our analysis, NA is defined as the presence of three criteria: (1) clinical presentation of AA, (2) absence of acute inflammation on histopathology, and (3) presence of S-100-positive spindle cells or proliferation of Schwann cells. Laparoscopic appendectomy has been shown to be a safe and successful treatment.

Conclusions

NA is a poorly known disease, which may clinically appear as AA but is often related to recurrent and longer lasting abdominal pain. Patients with NA may suffer for years before diagnosis. In cases of typical symptoms, appendectomy should be performed even in cases of macroscopically and radiologically normal-appearing appendices with normal laboratory results.

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Author notes

  1. Sarah Peisl and Oliver Burckhardt contributed equally to this work.

Authors and Affiliations

  1. Department of Surgery, HFR Fribourg – Cantonal Hospital, Chemin des Pensionnats 2-6, 1752, Villars-sur-Glâne, Switzerland

    Sarah Peisl, Oliver Burckhardt & Bernhard Egger

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  1. Sarah Peisl
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  2. Oliver Burckhardt
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  3. Bernhard Egger
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Contributions

BE conceived the presented idea. All authors participated in the conception and design of the paper. SP and OB performed the data acquisition, analysis, and interpretation. The manuscript was drafted by SP and then critically revised by OB and BE. All authors approved the final version of the manuscript and agreed to be accountable for all aspects of the work.

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Correspondence to Bernhard Egger.

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The presented patient and his legal guardian provided informed consent. Due to the nature of this paper, an approval from the local ethics committee was otherwise not required.

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The authors declare no competing interests.

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Peisl, S., Burckhardt, O. & Egger, B. Neurogenic appendicopathy: an underestimated disease—systematic review of the literature. Int J Colorectal Dis 36, 1995–2005 (2021). https://doi.org/10.1007/s00384-021-03901-1

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