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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; 15:1784-1798. [PMID: 37701693 PMCID: PMC10494602 DOI: 10.4240/wjgs.v15.i8.1784] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2022] [Revised: 02/14/2023] [Accepted: 06/11/2023] [Indexed: 08/25/2023] Open
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.
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Affiliation(s)
- Goran Augustin
- Department of Surgery, School of Medicine, University of Zagreb, Zagreb 10000, Croatia
- School of Medicine University of Zagreb, Zagreb 10000, Croatia
| | - Ivan Romic
- Department of Surgery, University Hospital Centre Zagreb, Zagreb 10000, Croatia
| | - Iva Miličić
- Department of Obstetrics and Gynecology, University Hospital Centre Zagreb, Zagreb 10000, Croatia
| | - Mislav Mikuš
- Department of Obstetrics and Gynecology, University Hospital Centre Zagreb, Zagreb 10000, Croatia
| | - Mislav Herman
- Department of Obstetrics and Gynecology, University Hospital Centre Zagreb, Zagreb 10000, Croatia
- School of Medicine University of Zagreb, Zagreb 10000, Croatia
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Ye Y, Lui VCH, Tam PKH. Pathogenesis of Choledochal Cyst: Insights from Genomics and Transcriptomics. Genes (Basel) 2022; 13:genes13061030. [PMID: 35741793 PMCID: PMC9223186 DOI: 10.3390/genes13061030] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2022] [Revised: 06/07/2022] [Accepted: 06/07/2022] [Indexed: 12/10/2022] Open
Abstract
Choledochal cysts (CC) is characterized by extra- and/or intra-hepatic b\ile duct dilations. There are two main theories, “pancreaticobiliary maljunction” and “congenital stenosis of bile ducts” proposed for the pathogenesis of CC. Although family cases or CC associated with other anomalies have been reported, the molecular pathogenesis of CC is still poorly understood. Recent advances in transcriptomics and genomics analysis platforms have unveiled key expression signatures/genes/signaling pathways in the pathogenesis of human diseases including CC. This review summarizes insights from genomics and transcriptomics studies into the pathogenesis of CC, with the aim to improve (i) our understanding of its underlying complex pathomechanisms, and (ii) clinical management of different subtypes of CC, in particular their associated hepatic fibrotic change and their risk of malignancy transformation.
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Affiliation(s)
- Yongqin Ye
- Faculty of Medicine, Macau University of Science and Technology, Macau, China;
- Department of Surgery, School of Clinical Medicine, University of Hong Kong, Hong Kong, China;
| | - Vincent Chi Hang Lui
- Department of Surgery, School of Clinical Medicine, University of Hong Kong, Hong Kong, China;
| | - Paul Kwong Hang Tam
- Faculty of Medicine, Macau University of Science and Technology, Macau, China;
- Correspondence:
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Ranucci G, Della Corte C, Alberti D, Bondioni MP, Boroni G, Calvo PL, Cananzi M, Candusso M, Clemente MG, D'Antiga L, Degrassi I, De Ville De Goyet J, Di Dato F, Di Giorgio A, Vici CD, Ferrari F, Francalanci P, Fuoti M, Fusaro F, Gaio P, Grimaldi C, Iascone M, Indolfi G, Iorio R, Maggiore G, Mandato C, Matarazzo L, Monti L, Mosca F, Nebbia G, Nuti F, Paolella G, Pinon M, Roggero P, Sciveres M, Serranti D, Spada M, Vajro P, Nicastro E. Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group. Dig Liver Dis 2022; 54:40-53. [PMID: 34688573 DOI: 10.1016/j.dld.2021.09.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Revised: 08/23/2021] [Accepted: 09/12/2021] [Indexed: 12/11/2022]
Abstract
Neonatal and infantile cholestasis (NIC) can represent the onset of a surgically correctable disease and of a genetic or metabolic disorder worthy of medical treatment. Timely recognition of NIC and identification of the underlying etiology are paramount to improve outcomes. Upon invitation by the Italian National Institute of Health (ISS), an expert working grouped was formed to formulate evidence-based positions on current knowledge about the diagnosis of NIC. A systematic literature search was conducted to collect evidence about epidemiology, etiology, clinical aspects and accuracy of available diagnostic tests in NIC. Evidence was scored using the GRADE system. All recommendations were approved by a panel of experts upon agreement of at least 75% of the members. The final document was approved by all the panel components. This position document summarizes the collected statements and defines the best-evidence diagnostic approach to cholestasis in the first year of life.
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Affiliation(s)
- Giusy Ranucci
- Department of Pediatrics, Santobono-Pausilipon Children's Hospital AORN, Naples, Italy
| | - Claudia Della Corte
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | | | - Maria Pia Bondioni
- Pediatric Radiology, University of Brescia, ASST Spedali Civili di Brescia, Brescia, Italy
| | | | - Pier Luigi Calvo
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin, Italy
| | - Mara Cananzi
- Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of Children with Liver Transplantation, University Hospital of Padova, Padova, Italy
| | - Manila Candusso
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maria Grazia Clemente
- Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy
| | - Lorenzo D'Antiga
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Irene Degrassi
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Jean De Ville De Goyet
- Pediatric Department for the Treatment and Study of Abdominal Diseases and Abdominal Transplantation, ISMETT UPMC, Palermo, Italy
| | - Fabiola Di Dato
- Department di Translational Medical Science, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
| | - Angelo Di Giorgio
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Carlo Dionisi Vici
- Division of Metabolism and Metabolic Diseases Research Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | | | - Paola Francalanci
- Department of Pathology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maurizio Fuoti
- Pediatric Gastroenterology and Endoscopy Unit Children's Hospital, ASST Spedali Civili di Brescia, Brescia, Italy
| | - Fabio Fusaro
- Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Paola Gaio
- Unit of Gastroenterology, Digestive Endoscopy, Hepatology and Care of Children with Liver Transplantation, University Hospital of Padova, Padova, Italy
| | - Chiara Grimaldi
- Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Maria Iascone
- Medical Genetics Laboratory, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Giuseppe Indolfi
- Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy
| | - Raffaele Iorio
- Department di Translational Medical Science, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
| | - Giuseppe Maggiore
- Hepatology, Gastroenterology, Nutrition and Liver transplantation Unit, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Claudia Mandato
- Department of Pediatrics, Santobono-Pausilipon Children's Hospital AORN, Naples, Italy
| | | | - Lidia Monti
- Department of Radiology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Fabio Mosca
- Neonatal Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Gabriella Nebbia
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Federica Nuti
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Giulia Paolella
- Service of Paediatric Hepatology, Department of Paediatrics, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Michele Pinon
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin, Italy
| | - Paola Roggero
- Neonatal Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
| | - Marco Sciveres
- Pediatric Hepatology and Liver Transplantation, ISMETT UPMC, Palermo, Italy
| | - Daniele Serranti
- Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy
| | - Marco Spada
- Division of Abdominal Transplantation and Hepatobiliopancreatic Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
| | - Pietro Vajro
- Department of Medicine, Surgery and Dentistry, "Scuola Medica Salernitana" University of Salerno, Baronissi, Italy
| | - Emanuele Nicastro
- Hepatology, Gastroenterology and Transplantation Unit, Hospital Papa Giovanni XXIII, Bergamo, Italy
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Brudvik KW, Yaqub S, Kemsley E, Coolsen MME, Dejong CHC, Wigmore SJ, Lassen K. Survey of the attitudes of hepatopancreatobiliary surgeons in northern Europe to resection of choledochal cysts in asymptomatic Western adults. BJS Open 2019; 3:785-792. [PMID: 31832585 PMCID: PMC6887667 DOI: 10.1002/bjs5.50208] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2019] [Accepted: 05/29/2019] [Indexed: 12/03/2022] Open
Abstract
Background Todani type 1 and 4 choledochal cysts are associated with a risk of developing cholangiocarcinoma. Resection is usually recommended, but data for asymptomatic Western adults are sparse. The aim of this study was to investigate diagnostic interpretation and attitudes towards resection of bile ducts for choledochal cysts in this subgroup of patients across northern European centres. Methods Thirty hepatopancreatobiliary centres were provided with magnetic resonance cholangiopancreatograms and asked to discuss the management of six cases: asymptomatic non‐Asian women, aged 30 or 60 years, with variable common bile duct (CBD) dilatations and different risk factors in the setting of a multidisciplinary team (MDT). The Fleiss κ value was calculated to estimate overall inter‐rater agreement. Results For all case scenarios combined, 83·3 and 86·7 per cent recommended resection for a CBD of 20 and 26 mm respectively, compared with 19·4 per cent for a CBD of 13 mm (P < 0·001). For patients aged 30 and 60 years, resection was recommended in 68·5 and 57·8 per cent respectively (P = 0·010). There was a trend towards recommending resection in the presence of a common channel, most pronounced in the 60‐year‐old patient. High amylase levels in the CBD aspirate led to recommendations to resect, but only for the 13‐mm CBD dilatation. There were no differences related to centre size or region. MDT discussion was associated with recommendations to resect. Inter‐rater agreement was 73·3 per cent (κ = 0·43, 95 per cent c.i. 0·38 to 0·48). Conclusion The inter‐rater agreement to resect was intermediate, and the recommendation was dependent mainly on the diameter of the CBD dilatation.
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Affiliation(s)
- K W Brudvik
- Department of Hepato-Pancreato-Biliary Surgery Oslo University Hospital Oslo Norway
| | - S Yaqub
- Department of Hepato-Pancreato-Biliary Surgery Oslo University Hospital Oslo Norway
| | - E Kemsley
- Department of Clinical Surgery University of Edinburgh Royal Infirmary, Edinburgh UK
| | - M M E Coolsen
- Department of Surgery Maastricht University Centre Maastricht the Netherlands.,Department of Surgery Rheinisch-Westfälische Technische Hochschule Universitätsklinikum Aachen Aachen Germany
| | - C H C Dejong
- Department of Surgery Maastricht University Centre Maastricht the Netherlands.,Department of Surgery Rheinisch-Westfälische Technische Hochschule Universitätsklinikum Aachen Aachen Germany
| | - S J Wigmore
- Department of Clinical Surgery University of Edinburgh Royal Infirmary, Edinburgh UK
| | - K Lassen
- Department of Hepato-Pancreato-Biliary Surgery Oslo University Hospital Oslo Norway
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Villasenor A, Stainier DYR. On the development of the hepatopancreatic ductal system. Semin Cell Dev Biol 2017; 66:69-80. [PMID: 28214561 DOI: 10.1016/j.semcdb.2017.02.003] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2016] [Revised: 02/03/2017] [Accepted: 02/13/2017] [Indexed: 12/13/2022]
Abstract
The hepatopancreatic ductal system is the collection of ducts that connect the liver and pancreas to the digestive tract. The formation of this system is necessary for the transport of exocrine secretions, for the correct assembly of the pancreatobiliary ductal system, and for the overall function of the digestive system. Studies on endoderm organ formation have significantly advanced our understanding of the molecular mechanisms that govern organ induction, organ specification and morphogenesis of the major foregut-derived organs. However, little is known about the mechanisms that control the development of the hepatopancreatic ductal system. Here, we provide a description of the different components of the system, summarize its development from the endoderm to a complex system of tubes, list the pathologies produced by anomalies in its development, as well as the molecules and signaling pathways that are known to be involved in its formation. Finally, we discuss its proposed potential as a multipotent cell reservoir and the unresolved questions in the field.
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Affiliation(s)
- Alethia Villasenor
- Department of Developmental Genetics, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany.
| | - Didier Y R Stainier
- Department of Developmental Genetics, Max Planck Institute for Heart and Lung Research, Bad Nauheim, Germany.
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Arruda PCLD, Coelho ARDB, Lima Filho JFC, Machado RJC, Souza APD, Mathias CADC, Ferraz ÁAB, Ferraz EM. Cystic dilatation of the common bile duct in adults: report of five cases and review of literature. Acta Cir Bras 2000. [DOI: 10.1590/s0102-86502000000400009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
The authors report five cases of cystic dilatation of the common bile duct Type I (Todani’s classification) in adults patients, in Division of General Surgery of a University Hospital, treated over a- 25-year- period from 1974 to 1999, among 16.057 operations, and not previously published. Diagnosis was obtained by operative cholangiogram (OC) in the first case, percutaneous transhepatic cholangiogram on the second one (PTHC) and by ultrasonography (US), endoscopic retrograde cholangiopancreatography (ERCP), and operative cholangiogram (OC), respectively, on the last three cases. The second patient had an adenocarcinoma arising in the cystic wall associated with peritoneal metastasis. The first two cases were treated by internal drainage and the last three by excision of the cysts and bilioenteric anastomoses. Classification, incidence, etiology, diagnosis, malignization and surgical treatment of biliary cystic disease (BCD) were revised, with the conclusion that resection must be the preferable method of treatment, when possible, especially due to the concern of malignization.
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