Neurogenic appendicopathy. A report of 8 cases

Abstract

INTRODUCTION: Neurogenic appendicopathy is not a very well-known disease. OBJECTIVE: To analyze the experience in the management of neurogenic appendicopathy in a tertiary hospital, assessing its clinical presentation, histological staging, the treatment carried out and its clinical evolution. METHOD: The study population included patients with histopathological criteria for neurogenic appendicopathy who did not present with MEN 2B syndrome, neurofibromatosis type I or Cowden syndrome. An analysis was carried out of tissue samples taken from a simple appendectomy after a diagnosis of neurogenic appendicopathy between 2000 and 2013, inclusive. The histopathological criteria were neurogenic hyperplasia with S-100 protein positivity and neuron-specific enolase in the immunohistochemical analysis. RESULTS: Of the 4,969 samples from the appendectomies analyzed, 0.16% (n = 8) met histopathological criteria of neurogenic appendicopathy. The age at presentation was 27.8 ± 12 years. Four patients were male and four were female. All patients started with abdominal pain in the right iliac fossa (RIF), and were operated on due to a diagnosis of acute appendix, with a simple appendectomy being performed. In four cases, another associated disease accounted for the pain in the RIF. With regard to histopathological type, submucosal neurogenic hyperplasia was present in five patients and fibrous obliteration in three patients. No statistically significant differences were found between the histological types. After surgery, during a mean follow up of 73.2 ± 28 months (15-105), all the patients remained asymptomatic. CONCLUSION: Neurogenic appendicopathy is an uncommon entity that can evolve as abdominal pain which is similar to acute appendix. Simple appendectomy is curative.