gms | German Medical Science

Introduction: Described for the first time in 1921, neurogenic appendicopathy (NA) still represents a not well known pathological entity. Clinically, NA cannot be differentiated from acute appendicitis. It is characterized by nerve proliferation and an increased number of endocrine cells. The aim of this study is to describe epidemiology, clinical signs, histological findings, and the therapy of NA in a paediatric cohort.

Materials and methods: Beetwen 02/2006 and 12/2008, the histopathological examinations were reviewed retrospectively. Only children with diagnosis NA were included. Analysis included patients age, sex, clinical signs, blood tests, the intraoperative macroscopic assessment of the appendix by the surgeon, the histopathological type and the therapy of NA. NA were classified in different histopathological types as mucosal, submucosal and axial neuroma. Neurogenic appendicopathy were diagnosed by haematoxylin-eosin (H.E.) staining and /or S-100 immunochemistry.

Results: Of 385 examined appendix specimen, 29 (8.3%) met the histopathological criteria of NA. All these children presented with abdominal pain in the right lower quadrant. The median values of the infectious parameters were 8.6 (range, 5.1-20.5, normal 5-10 x 10⁹/l) for leucocytes and 6.5 (range, 5-303, normal < 5 mg/l) for CRP. All the children underwent a preoperative ultrasound. The median mural diameter of the appendix was 7 mm (range, 5-18, normal < 6 mm). By the operating surgeon, the appendix was macroscopically assessed characterized intraoperatively as normal in 5 cases (17.2%), as acute in 17 cases (58.6%), phlegmonous in 4 cases (13.8%) and as perforated in 3 cases (10.4%). The appendix-specimen were classified as axial neuroma 2 (7%), mucosal 12 (41%), mucosal-submucosal 6 (21%) and submucosal 8 (28%).

Conclusion: Neurogenic appendicopathy is frequently (8.3 %) diagnosed by pathologists, especially if the appendix seems macroscopically normal or only acute infected. In children with appropriate clinical signs of acute appendicitis, in particular in combination with recurrent pain episodes and no other intraabdominal pathology, NA must be considered as causative. Therefore we recommend appendectomy and subsequent histopathological examination.