Pancreaticobiliary Maljunctions in European Patients with Bile Duct Cysts: Results of the Multicenter Study of the French Surgical Association (AFC).
Pancreaticobiliary maljunctions (PBMs) are congenital anomalies of the junction between pancreatic and bile ducts, frequently associated with bile duct cyst (BDC). BDC is congenital biliary tree diseases that are characterized by distinctive dilatation types of the extra- and/or intrahepatic bile ducts. Todani's types I and IVa, in which dilatation involves principally the main bile duct, are the most frequent. PBM induces pancreatic juice reflux into the biliary tract that is supposed to be one of the main factors of biliary cancer degeneration, although the diagnostic criteria of PBM that can be either morphological and/or functional are not well defined especially in Western series.



The aim of this study was to assess the relative prevalence of PBM in BDC in a large European multicenter study, to analyze the characteristics of PBM and try to propose diagnostic criteria of PBMs based on morphological and/or functional criteria and define the positive, negative predictive values, sensibility and specificity of either criteria.



From 1975 to 2012, 263 patients with BDC were analyzed. Among them, 190 (72.2 %) were considered to present PBM. Types I and IVa had a similar rate of PBM association. According to the "AFC classification," 57.2 % had a C-P type, 34.5 % a P-C type and 8.3 % a complex type ("anse-de-seau"). The median length of the common channel in patients with PBM was 15.8 ± 6.8 mm (range 5-40 mm). The median intrabiliary amylase and lipase levels were 65,249 and 172,104 UI/L, respectively. For the diagnostic of PBM, a common channel length of more than 8 mm and an intrabiliary amylase level superior to 8000 UI/L were associated with a predictive positive value and a specificity of more than 90 %. Synchronous biliary cancer had an incidence of 8.7 % in all patients with BDC and PBM 11.1 % in adults. Compared to type IV, the type I BDC was associated with statistically more cancer patients in the presence of PBM.



Characteristics of PBM associated with BDC in Western population are quite close to reported Eastern series. The results suggest considering both the intrabiliary value of amylase >8000 UI/L and a length of a common channel >8 mm as appropriate values for positive diagnosis of PBM.
Department of HPB Surgery, Hôpital Beaujon, Clichy, France.
Emilia
E
Ragot
Department of Digestive Surgery and Hepatic Transplantation, Hôpital de la Croix-Rousse, Lyon, France.
Jean-Yves
JY
Mabrut
Department of Digestive and Oncological Surgery, Hôpital Timone, Marseille, France.
Mehdi
M
Ouaïssi
Department of HPB Surgery, Hôpital Beaujon, Clichy, France.
Alain
A
Sauvanet
Department of HPB Surgery, Hôpital Beaujon, Clichy, France.
Safi
S
Dokmak
Department of HPB Surgery, Hôpital Gemelli, Rome, Italy.
Gennaro
G
Nuzzo
Department of Digestive Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Nermin
N
Halkic
Department of Pediatric Surgery, Hôpital Femme-Mère-Enfant, Lyon, France.
Remi
R
Dubois
Department of Digestive Surgery, Hôpital Michallon, Grenoble, France.
Christian
C
Létoublon
Paul Brousse University Hospital, Villejuif, France.
Daniel
D
Cherqui
Department of Digestive and HPB Surgery, Hôpital Henri Mondor, Creteil, France.
Daniel
D
Azoulay
Department of Pediatric Digestive Surgery, Hôpital Necker, Paris, France.
Sabine
S
Irtan
Rennes University Hospital, Rennes, France.
Karim
K
Boudjema
Department of Digestive Surgery and Transplantation, Hôpital Claude Huriez, Lille, France.
François-René
FR
Pruvot
Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint Luc, Brussels, Belgium.
Jean-François
JF
Gigot
Department of Digestive and Endocrine Surgery, Hospital Robert Debré, Ave du General Koenig, 51090, Reims, France. rkianmanesh@chu-reims.fr.
Reza
R
Kianmanesh
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000681:Amylases / Q000378:metabolism; D001661:Biliary Tract Neoplasms / Q000150:complications / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D015529:Choledochal Cyst / Q000150:complications / Q000201:enzymology* / Q000453:epidemiology*; D003135:Common Bile Duct / Q000002:abnormalities*; D000013:Congenital Abnormalities / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D005602:France; D006801:Humans; D015994:Incidence; D007223:Infant; D008049:Lipase / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010183:Pancreatic Ducts / Q000002:abnormalities*; D011237:Predictive Value of Tests; D015995:Prevalence; D055815:Young Adult
D008049:Lipase; D000681:Amylases