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Pancreaticobiliary maljunction

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Pancreaticobiliary maljunction
Other namesPBM
SpecialtyGastroenterology

Pancreaticobiliary maljunction (PBM) is a congenital malformation where the pancreatic and bile ducts meet outside of the duodenum. There are two varieties of PBM: one with biliary dilatation and the other without.[1] When an abnormally long common channel is visible on direct cholangiography, such as endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography, PBM is diagnosed.[2]

Signs and symptoms

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The sphincter of Oddi does not control the pancreaticobiliary junction in patients with pancreaticobiliary maljunction (PBM).[3] Because the pressure in the pancreatic duct is typically higher than in the bile duct, pancreatic juice often refluxes into the biliary tract.[4] Patients with PBM experience continuous regurgitation, but this does not always result in symptoms.[3]

Increased pressure in the pancreatic and bile ducts as a result of a primary stricture of the distal bile duct or blockage of the common channel can cause symptoms like jaundice, vomiting, and abdominal pain. This is frequently transient and may be brought on by impaction from a protein plug.[5][6]

Complications

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In patients with PBM, regardless of the presence of biliary dilatation, acute pancreatitis is more common in children (30% of patients) than in adults (9%).[7] Although pancreatitis is often mild with subtle imaging findings, it can also be recurrent.[8] One possible cause of acute pancreatitis linked to PBM is protein plugs.[9] Chronic pancreatitis affects about 3% of PBM patients.[7]

Causes

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There is disagreement over PBM's pathophysiology. Nonetheless, a number of studies have indicated that it may be related to abnormal ventral pancreatic development.[10][11] It is thought that the ventral pancreatic duct is connected to the origin of the common channel because small pancreatic branch ducts have been observed emerging from the common channel in certain PBM patients.[10] Rarely, PBM is linked to additional congenital pancreatic abnormalities like pancreas divisum or annular pancreas.[12]

Diagnosis

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The JSPBM committee on PBM diagnostic criteria states that anatomical examination findings or imaging characteristics are used to diagnose PBM. The diagnosis is confirmed by the existence of an abnormally long common channel, an abnormal union between the pancreatic and bile ducts, or a pancreatic-biliary junction outside the duodenal wall.[2]

Treatment

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Regardless of whether symptoms are present, risk-reducing surgery is advised for patients with PBM because it is a risk factor for biliary cancer.[13]

References

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  1. ^ Kamisawa, Terumi; Honda, Goro (September 4, 2018). "Pancreaticobiliary Maljunction: Markedly High Risk for Biliary Cancer". Digestion. 99 (2). S. Karger AG: 123–125. doi:10.1159/000490816. ISSN 0012-2823. PMID 30179869.
  2. ^ a b Kamisawa, Terumi; Ando, Hisami; Hamada, Yoshinori; Fujii, Hideki; Koshinaga, Tsugumichi; Urushihara, Naoto; Itoi, Takao; Shimada, Hiroshi (December 5, 2013). "Diagnostic criteria for pancreaticobiliary maljunction 2013". Journal of Hepato-Biliary-Pancreatic Sciences. 21 (3). Wiley: 159–161. doi:10.1002/jhbp.57. ISSN 1868-6974. PMID 24307541.
  3. ^ a b Ono, Ayako; Arizono, Shigeki; Isoda, Hiroyoshi; Togashi, Kaori (2020). "Imaging of Pancreaticobiliary Maljunction". RadioGraphics. 40 (2): 378–392. doi:10.1148/rg.2020190108. ISSN 0271-5333. PMID 31951513.
  4. ^ Csendes, A.; Kruse, A.; Funch-Jensen, P.; Øster, M.J.; Ørnsholt, J.; Amdrup, E. (1979). "Pressure measurements in the biliary and pancreatic duct systems in controls and in patients with gallstones, previous cholecystectomy, or common bile duct stones". Gastroenterology. 77 (6). Elsevier BV: 1203–1210. doi:10.1016/0016-5085(79)90158-6. ISSN 0016-5085. PMID 499707.
  5. ^ Ando, Hisami; Ito, Takahiro; Nagaya, Masahiro; Watanabe, Yoshio; Seo, Takahiko; Kaneko, Kenitiro (1995). "Pancreaticobiliary maljunction without choledochal cysts in infants and children: Clinical features and surgical therapy". Journal of Pediatric Surgery. 30 (12). Elsevier BV: 1658–1662. doi:10.1016/0022-3468(95)90445-x. ISSN 0022-3468. PMID 8749917.
  6. ^ Kanek, K; Ando, H; Ito, T; Watanabe, Y; Seo, T; Harada, T; Ito, F (June 1997). "Protein plugs cause symptoms in patients with choledochal cysts". The American Journal of Gastroenterology. 92 (6): 1018–1021. PMID 9177522.
  7. ^ a b Morine, Yuji; Shimada, Mitsuo; Takamatsu, Hideo; Araida, Tatsuo; Endo, Itaru; Kubota, Masayuki; Toki, Akira; Noda, Takuo; Matsumura, Toshinobu; Miyakawa, Shuichi; Ishibashi, Hiroki; Kamisawa, Terumi; Shimada, Hiroshi (2013). "Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan-nationwide survey". Journal of Hepato-Biliary-Pancreatic Sciences. 20 (5): 472–480. doi:10.1007/s00534-013-0606-2. ISSN 1868-6974. PMID 23579999.
  8. ^ Kamisawa, Terumi; Kaneko, Kenitiro; Itoi, Takao; Ando, Hisami (2017). "Pancreaticobiliary maljunction and congenital biliary dilatation". The Lancet Gastroenterology & Hepatology. 2 (8). Elsevier BV: 610–618. doi:10.1016/s2468-1253(17)30002-x. ISSN 2468-1253. PMID 28691687.
  9. ^ Todani, Takuji; Urushihara, Naoto; Watanabe, Yasuhiro; Toki, Akira; Uemura, Sadashige; Sato, Yasuhisa; Morotomi, Yoshiki (1990). "Pseudopancreatitis in choledochal cyst in children: Intraoperative study of amylase levels in the serum". Journal of Pediatric Surgery. 25 (3). Elsevier BV: 303–306. doi:10.1016/0022-3468(90)90072-h. ISSN 0022-3468. PMID 1690281.
  10. ^ a b Matsumoto, Yoshiro; Fujii, Hideki; Itakura, Jun; Mogaki, Masatoshi; Matsuda, Masanori; Morozumi, Atsuro; Fujino, Masayuki A.; Suda, Koichi (2001). "Pancreaticobiliary maljunction: Etiologic concepts based on radiologic aspects". Gastrointestinal Endoscopy. 53 (6). Elsevier BV: 614–619. doi:10.1067/mge.2001.113920. ISSN 0016-5107. PMID 11323587.
  11. ^ KAMISAWA, T; EGAWA, N; NAKAJIMA, H; TSURUTA, K; OKAMOTO, A; MATSUKAWA, M (2005). "Origin of the long common channel based on pancreatographic findings in pancreaticobiliary maljunction". Digestive and Liver Disease. 37 (5). Elsevier BV: 363–367. doi:10.1016/j.dld.2004.11.007. ISSN 1590-8658. PMID 15843087.
  12. ^ Urushihara, Naoto; Hamada, Yoshinori; Kamisawa, Terumi; Fujii, Hideki; Koshinaga, Tsugumichi; Morotomi, Yoshiki; Saito, Takeshi; Itoi, Takao; Kaneko, Kenitiro; Fukuzawa, Hiroaki; Ando, Hisami (2017). "Classification of pancreaticobiliary maljunction and clinical features in children". Journal of Hepato-Biliary-Pancreatic Sciences. 24 (8): 449–455. doi:10.1002/jhbp.485. ISSN 1868-6974. PMID 28639336.
  13. ^ Working Committee of Clinical Practice Guidelines for Pancreaticobiliary Maljunction; Kamisawa, Terumi; Ando, Hisami; Suyama, Masafumi; Shimada, Mitsuo; Morine, Yuji; Shimada, Hiroshi (2012). "Japanese clinical practice guidelines for pancreaticobiliary maljunction". Journal of Gastroenterology. 47 (7): 731–759. doi:10.1007/s00535-012-0611-2. ISSN 0944-1174. PMID 22722902.

Further reading

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  • Kamisawa, Terumi; Takuma, Kensuke; Anjiki, Hajime; Egawa, Naoto; Kurata, Masanao; Honda, Goro; Tsuruta, Kouji; Sasaki, Tsuneo (2009). "Pancreaticobiliary Maljunction". Clinical Gastroenterology and Hepatology. 7 (11). Elsevier BV: S84–S88. doi:10.1016/j.cgh.2009.08.024. ISSN 1542-3565. PMID 19896105.
  • Ohuchida, Jiro (November 1, 2006). "Long-term Results of Treatment for Pancreaticobiliary Maljunction Without Bile Duct Dilatation". Archives of Surgery. 141 (11). American Medical Association (AMA): 1066. doi:10.1001/archsurg.141.11.1066. ISSN 0004-0010. PMID 17116798.
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