Augustin G, Romic I, Miličić I, Mikuš M, Herman M. Maternal choledochal cysts in pregnancy: A systematic review of case reports and case series. World J Gastrointest Surg 2023; 15(8): 1784-1798 [PMID: 37701693 DOI: 10.4240/wjgs.v15.i8.1784]
Corresponding Author of This Article
Goran Augustin, MD, MSc, PhD, Associate Professor, Doctor, Senior Scientist, Surgical Oncologist, Department of Surgery, University Hospital Centre Zagreb, Kišpatićeva 12, Zagreb 10000, Croatia. augustin.goran@gmail.com
Research Domain of This Article
Surgery
Article-Type of This Article
Systematic Reviews
Open-Access Policy of This Article
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World J Gastrointest Surg. Aug 27, 2023; 15(8): 1784-1798 Published online Aug 27, 2023. doi: 10.4240/wjgs.v15.i8.1784
Maternal choledochal cysts in pregnancy: A systematic review of case reports and case series
Goran Augustin, Ivan Romic, Iva Miličić, Mislav Mikuš, Mislav Herman
Goran Augustin, Department of Surgery, School of Medicine, University of Zagreb, Zagreb 10000, Croatia
Goran Augustin, Mislav Herman, School of Medicine University of Zagreb, Zagreb 10000, Croatia
Ivan Romic, Department of Surgery, University Hospital Centre Zagreb, Zagreb 10000, Croatia
Iva Miličić, Mislav Mikuš, Mislav Herman, Department of Obstetrics and Gynecology, University Hospital Centre Zagreb, Zagreb 10000, Croatia
Author contributions: Augustin G designed the research and wrote the paper; Romic I and Miličić I performed the research and wrote the paper; Mikuš M and Herman M analyzed the data and performed the literature review.
Conflict-of-interest statement: The authors declare no conflict of interest for this article.
PRISMA 2009 Checklist statement: The manuscript was prepared and revised according to the PRISMA 2009 Checklist statement.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Goran Augustin, MD, MSc, PhD, Associate Professor, Doctor, Senior Scientist, Surgical Oncologist, Department of Surgery, University Hospital Centre Zagreb, Kišpatićeva 12, Zagreb 10000, Croatia. augustin.goran@gmail.com
Received: December 20, 2022 Peer-review started: December 20, 2022 First decision: February 8, 2023 Revised: February 14, 2023 Accepted: June 11, 2023 Article in press: June 11, 2023 Published online: August 27, 2023 Processing time: 248 Days and 2.9 Hours
Abstract
BACKGROUND
Choledochal cysts (CC) are cystic dilatations of the biliary tract, usually diagnosed during childhood, with an estimated incidence in the general population of 1:100000. Complications related to CC include rupture, biliary obstruction, and cholangitis. Maternal CC in pregnancy are rarely reported, and there are no guidelines on optimal management.
AIM
To systematically review maternal CC diagnosed during pregnancy or postpartum with regard to the clinical presentation of CC, the mode of treatment and delivery, and maternal outcomes.
METHODS
A literature search of cases and case series of maternal CC in pregnancy and postpartum was conducted using MEDLINE/PubMed, Web of Science, Google Scholar, and Embase. There were no restrictions on language or publication year. Databases were lastly accessed on September 1, 2022.
RESULTS
Overall, 71 publications met the inclusion criteria, reporting 97 cases. Eighty-eight cases were diagnosed during pregnancy and nine in the puerperium. The most common symptoms were abdominal pain (81.2%) and jaundice (60.4%). Interventions for CC complications were required in 52.5% of the cases, and 34% of pregnancies were induced. Urgent cesarean section (CS) was done in 24.7%. The maternal mortality was 7.2%, while fetal mortality was inconsistently reported. Cholangitis, CC > 15 cm, and bilirubin levels > 80 mmol/L were associated with a higher likelihood of urgent CS and surgical intervention for CC. Bilirubin levels positively correlated with CC size. There was no correlation between age and cyst dimension, gestational age at cyst discovery, and CC size.
CONCLUSION
Although rare, maternal CC in pregnancy should be included in the evaluation of jaundice with upper abdominal pain. Symptomatology and clinical course are variable, and treatment may range from an expectative approach to emergent surgical CC treatment and urgent CS. While most cases were managed by conservative measures or drainage procedures, CC > 15 cm and progressive cholangitis carry the risk of CC rupture and septic complications, which may increase the rates of unfavorable maternal and fetal outcomes. Therefore, such cases require specific surgical and obstetric interventions.
Core Tip: Although rare, maternal choledochal cysts (CC) in pregnancy should be included in the evaluation of jaundice with upper abdominal pain. Symptomatology and clinical course are variable, and treatment may range from an expectative approach to emergent surgical treatment of CC and urgent cesarean section. While most cases were managed by conservative measures or drainage procedures, CC > 15 cm and progressive cholangitis carry the risk of CC rupture and septic complications, which may increase the rates of unfavorable maternal and fetal outcomes. Therefore, such cases require specific surgical and obstetric interventions.
