[Bile duct obstruction caused by neurofibroma in a patient with Recklinghausen's disease].
BACKGROUND
Neurofibromatosis type-I (von Recklinghausen's disease) is characterised by multiple cutan neurofibromas, cafe-au-lait spots, bone abnormalities and tumours of central and peripheral nerves. Gastrointestinal involvement occurs in 25 percent of all cases.


METHODS
The authors report a case of a 53-year-old man presenting with rare hepatobiliary complication. He complained of abdominal pain and he looked jaundiced. The abdominal ultrasound revealed cholestasis confirmed by the laboratory results. The endoscopic retrograd cholangiography showed nearly total obstruction in the middle part of common bile duct. Subsequently laparotomy and choledochoenterostomy was performed. Plexiform neurofibroma was found in the wall of common bile duct and several gastrointestinal stromal tumour seedings were identified on the serosal surface of the small intestine. Post surgery he remained symptom free and showed no sign of jaundice.


CONCLUSIONS
The authors would like to draw attention to this extremely rare case, since only two similar cases appeared in the literature.
Bajor
Judit
J
Baranya Megyei Kórház, I.sz.Belgyógyászati és Gasztroenterológiai Osztály, Pécs.
Garamszegi
Mária
M
Grexa
Erzsébet
E
Anga
Béla
B
Papp
Gábor
G
Beró
Tamás
T
D002760:Cholangiopancreatography, Endoscopic Retrograde; D001651:Cholestasis, Extrahepatic / Q000000981:diagnostic imaging / Q000209:etiology*; D003138:Common Bile Duct Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D006801:Humans; D007414:Intestinal Neoplasms / Q000150:complications / Q000175:diagnosis; D007421:Intestine, Small; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging