Standardized Diagnosis and Treatment of Pancreaticobiliary Maljunction

Summary

Pancreaticobiliary maljunction predisposes to recurrent pancreatitis, pancreatobiliary stones, and biliary tract tumors. This article aims to enhance clinical recognition to facilitate early diagnosis and standardized management of this condition.

Abstract

According to extensive case analyses compiled by the Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM), patients with Pancreaticobiliary Maljunction (PBM) exhibit a significantly elevated incidence of biliary tract tumors, ranging from 34% to 39%. This rate is dramatically higher than the population-based incidence of biliary tract tumors in the general population (approximately 0.002%). Beyond malignancy, PBM is associated with multiple common pancreatobiliary disorders, including recurrent pancreatitis, stones within the common pancreatobiliary channel, and pancreatic duct stones, which substantially impair patients' quality of life. Despite its insidious nature and frequent delayed or missed diagnosis, key clinical features such as recurrent pancreatitis, acalculous cholecystitis, and extrahepatic bile duct dilation provide crucial diagnostic clues. Standard diagnostic modalities include abdominal ultrasound, computed tomography (CT), and magnetic resonance cholangiopancreatography (MRCP). Endoscopic retrograde cholangiopancreatography (ERCP) serves as the definitive diagnostic and classification tool. Upon confirmation of diagnosis, patients should undergo curative surgery to mitigate the risk of biliary tract carcinogenesis. This article provides a detailed protocol for the diagnostic and therapeutic approaches to PBM.

Introduction

Pancreaticobiliary maljunction (PBM) was first reported by Japanese scholars Kozumi K and Kodama T in 1916. However, it was not until 1969 when Babbitt D P proposed an etiological explanation for congenital biliary dilatation and highlighted the correlation between PBM and biliary dilatation that the medical community began to recognize its significance¹.

In 1990, the Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM) established diagnostic criteria for PBM, with the defining feature being the union of pancreatic and biliary ducts outside the duodenal wall. JSPBM has maintained an annual registry of PBM c....

Protocol

This protocol was approved by the Ethics Committee of Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, and written informed consent was obtained from the patient who underwent surgery.

1. Diagnostic methods

1. Magnetic Resonance Cholangiopancreatography (MRCP): MRCP serves as a reliable modality for determining the length of the pancreaticobiliary common channel in the diagnosis of PBM. Ensure the imaging protocol includes half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences, obtained using thin-slice coronal and axial T2-weighted parameters (repetition time: 1200 ms; echo time: 80 ms; slice....

Results

The key outcomes in standardized diagnosis and treatment of PBM are as follows:

Definitive diagnosis of suspected cases
Magnetic resonance cholangiopancreatography (MRCP) revealed an anomalous pancreaticobiliary junction, dilated extrahepatic bile ducts, and suspected filling defects in the common bile duct (Figure 1). Endoscopic retrograde cholangiopancreatography (ERCP) was critical for confirmation (Figure 2). .......

Discussion

Pancreaticobiliary maljunction (PBM) is a congenital anomaly characterized by an abnormal connection between the pancreatic and biliary ducts beyond the duodenal wall, often leading to serious complications such as recurrent pancreatitis and biliary cancer. This study demonstrates a standardized protocol that integrates endoscopic and surgical techniques to enhance diagnostic accuracy and therapeutic safety in the management of PBM.

A critical innovation in our protocol is the preoperative pla.......

Materials

List of materials used in this article

Name	Company	Catalog Number	Comments
ENDOFLATOR 40	STORZ	UI400
ENDOPATH XCEL Trocars	ETHICON	B12LP
ENDOPATH XCEL Trocars	ETHICON	B5LT
HARMONIC 700 with Advanced Hemostasis	ETHICON	HAR736
HOPKINS Rubina NIR/ICG telescopes	STORZ	26003BRA
The Autotome RX Cannulating Sphincterotome	Boston Scientific	M00545170	Autotome RX 44 Cannulating Sphincterotome
The Fujifilm ED-580XT Duodenoscope	Fujifilm	ED-580XT
The Jagwire High Performance Guidewire	Boston Scientific	M00556581	Jagwire Guidewire ST
Weck Hem-o-lok Polymer Locking Ligation System	WECK	544220 Medium
Zimmon Pancreatic Stent	COOK MEDICAL	SPSOF-5-7