Apendicopatía neurogénica: A propósito de 8 casos

espanolIntroduccion: la apendicopatia neurogenica es una patologia poco conocida. Objetivo: analizar la experiencia en el manejo de la apendicopatia neurogenica en un hospital de tercer nivel, valorando su presentacion clinica, su estadio histologico, el tratamiento realizado y su evolucion clinica. Metodo: la poblacion a estudio estuvo formada por pacientes con criterios histopatologicos de apendicopatia neurogenica que no presentaran sindrome de MEN IIb, neurofibromatosis tipo I ni enfermedad de Cowden. Se realizo un analisis de las piezas de apendicectomia simple con el diagnostico de apendicopatia neurogenica entre los anos 2000 y 2013 inclusive. Los criterios histopatologicos fueron hiperplasia neurogenica con positividad en la inmunohistoquimica para la proteina S-100 y enolasa neuronal especifica. Resultados: de las 4.969 piezas de apendicectomia analizadas, el 0,16% (n = 8) tenia criterios histopatologicos de apendicopatia neurogenica. La edad de presentacion fue de 27,8 ± 12 anos. Cuatro pacientes fueron varones y otros cuatro fueron mujeres. Todos debutaron con dolor abdominal en fosa iliaca derecha (FID) y se intervinieron con el diagnostico de apendicitis aguda, realizando una apendicectomia simple. En cuatro casos se asocio otra patologia que justificaba el dolor en FID. Respecto al tipo histopatologico, la hiperplasia neurogenica submucosa tuvo lugar en cinco pacientes y la obliteracion fibrosa en tres pacientes. No se objetivaron diferencias estadisticamente significativas entre los tipos histologicos. Tras la cirugia, durante un seguimiento medio de 73,2 ± 28 meses (15-105) todos los pacientes permanecen asintomaticos. Conclusion: la apendicopatia neurogenica es una entidad infrecuente que puede cursar con dolor abdominal similar a una apendicitis aguda. La apendicectomia simple es curativa. EnglishIntroduction: Neurogenic appendicopathy is not a very wellknown 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.