[Diffuse ganglioneuromatosis: infrequent pathology communication and review of literature].
Ganglioneuromatosis is an infrequent disease characterized by mienteric plexus and enteric nervous fiber hyperplasia, habitually manifested by constipation, pain and abdominal distension. There are two ways of presentation: mucosa or transmural. Regularly may be associated with another pathologies like multiple endocrine neoplasia type 2 and Von Recklinghausen's disease. The objective of this publication is to present an extremely infrequent pathology and make a bibliographic review of its clinical, diagnostic, prognostic and therapeutical aspects. We present a fifty-six year old male with a six month history ofabdominal pain and pseudo-obstruction syndrome. During the hospitalization he presents infectious complications, with septic shock secondary to nosocomial pneumonia and death. A Necropsy was done and ganglioneuromatosis was diagnosed. Due to the low incidence of this pathology, it is rarely included in the differential diagnosis of pseudo-obstructive syndrome in adults. The post-mortem diagnosis moved us to perform a bibliographic search to amplify our knowledge and compare this particular case with those previously communicated by other authors.
Godoy
Natalia
N
Servicio de Clínica Médica, Hospital Provincial del Centenario, Rosario, Santa Fe, Argentina. nataliacgodoy@yahoo.com.ar
Parodi
Roberto
R
Díaz
Marisol
M
Valenti
José
J
Carlson
Damián
D
Greca
Alcides
A
D017809:Fatal Outcome; D005729:Ganglioneuroma / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007414:Intestinal Neoplasms / Q000175:diagnosis* / Q000601:surgery; D007813:Laparotomy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D017253:Neurofibromatoses / Q000175:diagnosis* / Q000601:surgery; D011379:Prognosis; D014057:Tomography, X-Ray Computed