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Genes (Basel) 2022 Jun 8;13(6). Epub 2022 Jun 8.

Faculty of Medicine, Macau University of Science and Technology, Macau, China.

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. Read More

Clin Exp Gastroenterol 2022 11;15:79-90. Epub 2022 Jun 11.

Faculty of Medicine, Mbarara University of Science and Technology, Mbarara, Uganda.

Background: The diagnosis of extrahepatic obstructive jaundice (EHOJ) remains a challenge and is often made late in low-resource settings. Systematic data are limited on the etiology and prognosis of patients with obstructive jaundice in Uganda. The objective of this study was to determine the etiology, clinical presentations, and short-term treatment outcomes of patients managed for EHOJ at Mbarara Regional Referral Hospital (MRRH) in south-western Uganda. Read More

J Minim Access Surg 2022 Jul-Sep;18(3):372-377

Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Background: Laparoscopic definitive surgery for choledochal cyst (CC) in infants requires advanced skills because of their small size. If patients with a prenatal diagnosis of CC have any biliary symptoms, they need semi-emergency definitive surgery. This study aimed to estimate whether laparoscopic definitive surgery for CC can be performed safely and effectively in infants, especially when emergency surgery is required. Read More

Pan Afr Med J 2022 14;41:204. Epub 2022 Mar 14.

Department of Internal Medicine, Al-Thora General Hospital, School of Medicine, Ibb University of Medical Science, Ibb, Yemen.

Caroli´s disease is a congenital hepatic disorder characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts with the absence of congenital hepatic fibrosis. Caroli´s disease is rare, with few reported cases in the literature, making it hard to distinguish from other liver abnormalities. We present a case of Caroli´s disease discovered indecently in a 16-year-old female who presented with recurrent abdominal pain and intermittent jaundice in the last three years. Read More

Ann Hepatobiliary Pancreat Surg 2022 Jun 7. Epub 2022 Jun 7.

Unit of Gastronterology and Digestive Endoscopy, Sandro Pertini Hospital, Rome, Italy.

Diverticula of the choledochus, better known as Todani type II cysts, are very rare and represent a predominantly pediatric pathology. Their identification by radiological methods, even if occasional, requires clinical doctors to request a surgical consultation, even for asymptomatic subjects, to proceed with their removal, given the risk of associated neoplasms. The laparoscopic approach for surgical treatment of these cysts has been recently introduced with excellent results. Read More

Int J Surg Case Rep 2022 May 1;94:107127. Epub 2022 May 1.

Department of Surgery, RIMS, Ranchi.

Introduction: Among a plethora of causes of acute abdomen, spontaneous common bile duct perforation (SCBDP) resulting in biliary peritonitis is almost never envisaged. Since the term SCBDP is often misconstrued as absence of an identifiable cause of perforation, 'nontraumatic perforation of CBD' is also in parlance to exclude relatively common causes such as trauma and iatrogenic injuries. In adults, choledochal cyst, cholangitis, infection, pancreatitis, pancreatobiliary maljunction have been identified as causes of perforation, however, choledocholithiasis remains the most common cause associated with spontaneous perforation of extra hepatic bile duct. Read More

Indian J Pediatr 2022 Jun 2. Epub 2022 Jun 2.

Department of Radiodiagnosis and Interventional Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.

Cholestatic jaundice characterized by elevated conjugated bilirubin can be due to multitude of factors in neonates and childhood. Extrahepatic biliary atresia (EHBA), choledochal cyst, neonatal hepatitis, cytomegalovirus (CMV), and biliary plug are some of the common causes in neonate and early infancy. Causes in late infancy and childhood comprises viral hepatitis, choledochal cyst, cholelithiasis, worm infestation, and biliary compression secondary to extrinsic causes (node, collection, tumor). Read More

J Robot Surg 2022 May 27. Epub 2022 May 27.

Department of Pediatric General Surgery and Urology, Assistance Publique-Hôpitaux de Paris (AP-HP), Robert-Debré Children University Hospital, 48 boulevard Sérurier, 75019, Paris, France.

Incisions in the supra-pubic region have been described robotic-assisted surgery to improve cosmetic results in adults, but seldom in children. We aimed to present an innovative trocar placement in the Pfannenstiel line, named Scarless Laparoscopic Incisions in Pfannenstiel (SLIP), and evaluate its feasibility for various intra-abdominal procedures and its cosmetic results in pediatric robotic surgery. We performed a monocentric prospective study, including children undergoing robotic-assisted surgeries using a SLIP approach (July 2019-September 2021). Read More

Cureus 2022 Apr 22;14(4):e24384. Epub 2022 Apr 22.

Department of Radiology, Nemours Children's Hospital, Orlando, USA.

Choledochal cysts are rare congenital cystic dilatations of the biliary tree. They are most commonly present in female infants and young children, and their pathology remains unclear. The triad of intermittent jaundice, abdominal mass, and pain is found only in a minority of patients. Read More

Ann Hepatobiliary Pancreat Surg 2022 May 27. Epub 2022 May 27.

Department of Surgical Gastroenterology and MIS, Sahasra Hospitals, Jayanagar, Bangalore, India.

Cystic lesions of the liver are commonly encountered in routine clinical practice with a reported prevalence of 15%-18%. They may range from a benign simple developmental cyst to a malignancy. Therefore, an accurate diagnosis is essential for adequate management. Read More

Asian J Endosc Surg 2022 May 24. Epub 2022 May 24.

Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, Kagoshima, Japan.

Laparoscopic choledochal cyst excision and hepaticojejunostomy is a representative surgical procedure requiring laparoscopic suturing including needle driving and knot tying. Laparoscopic needle driving and knot tying is a highly technically demanding skill, especially in small infants and children. We developed a laparoscopic hepaticojejunostomy simulator for training. Read More

ANZ J Surg 2022 06 17;92(6):1356-1364. Epub 2022 May 17.

Department of Surgery, North Shore Hospital, Auckland, New Zealand.

Background: Choledochal cysts should be treated with complete surgical resection, or, rarely liver transplantation. Treated patients can remain at risk of developing metachronous cholangiocarcinomas and lifelong follow up is indicated. However, there is no agreement on what constitutes an acceptable follow up strategy. Read More

Case Rep Surg 2022 20;2022:9605612. Epub 2022 Apr 20.

General Hospital "Prim. Dr. Abdulah Nakas", Department of Surgery, Sarajevo, Bosnia and Herzegovina.

Choledochal cysts (CCs) are rare congenital anomalies in the form of cystic dilatation of any part of the biliary tree, and they rarely reach the size over 10 cm. We present a case of a 51-year-old female with a one-year history of abdominal pain and palpable mass in the epigastrium and right upper abdomen. Diagnosis of giant CC was made using magnetic resonance cholangiopancreatography. Read More

Br J Radiol 2022 Jul 5;95(1135):20201189. Epub 2022 May 5.

Suzhou Institute of Biomedical Engineering and Technology, Chinese Academy of Sciences, 88 Keling Road, Suzhou, China.

Objectives: The aim of this study was to establish an automatic classification model for chronic inflammation of the choledoch wall using deep learning with CT images in patients with pancreaticobiliary maljunction (PBM).

Methods: CT images were obtained from 76 PBM patients, including 61 cases assigned to the training set and 15 cases assigned to the testing set. The region of interest (ROI) containing the choledochal lesion was extracted and segmented using the UNet++ network. Read More

J Hepatobiliary Pancreat Sci 2022 Apr 18. Epub 2022 Apr 18.

Department of Neonatal Surgery, Guangzhou Women and Children's Medical Center, Guangzhou, Guangdong, China.

Background/purpose: There is currently no consensus on the timing of operative correction for patients with a prenatal diagnosis of choledochal cyst (CDC). This study aims to retrospectively analyze patients with prenatally diagnosed CDCs to identify the optimal timing of operative correction and the importance of cyst size as a predictor of the appearance of symptoms related to the CDC.

Methods: We reviewed 125 patients with a prenatal diagnosis of CDC who were admitted to Guangzhou Women and Children's Medical Center from July 2015 to July 2020. Read More

Ann Med Surg (Lond) 2022 Mar 26;75:103412. Epub 2022 Feb 26.

Capital Institute of Pediatrics-Peking University Teaching Hospital, Beijing, 100020, China.

Objectives: Minimally invasive cyst excision and Roux-en-Y hepaticojejunostomies include laparoscopic and robotic-assisted operations. The current systematic review and meta-analysis compared the efficacy between the 2 groups.

Methods: A systematic search of PubMed, Web of Science, Embase, Wiley, Cochrane Library and Clinical Trials was performed from May 1995 to December 2021. Read More

J Surg Case Rep 2022 Mar 30;2022(3):rjac126. Epub 2022 Mar 30.

Division of HPB Surgery, Department of Surgery, Phramongkutklao Hospital, Thung Phaya Thai, Ratchathewi, Bangkok, Thailand.

Type VI choledochal cysts or cystic duct dilatation cysts are a relatively new and rare condition. We report the case of a 35-year-old man who presented with a history of recurrent episodes of epigastrium pain. Magnetic resonance cholangiography revealed a cyst lodged between the cystic duct and the right anterior sectoral bile duct. Read More

Pediatr Surg Int 2022 Jun 26;38(6):817-824. Epub 2022 Mar 26.

Section of Surgical Sciences, Department of Pediatric Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.

Purpose: The majority of pediatric patients with choledochal cysts (CDC) are symptomatic prior to undergoing CDC excision. This study investigated the impact of surgical timing of CDC excision on postoperative outcomes among children.

Methods: We performed a retrospective review of 59 patients undergoing open CDC excision with Roux-Y hepaticojejunostomy between 2000 and 2020. Read More

Medicina (Kaunas) 2022 Mar 11;58(3). Epub 2022 Mar 11.

Department of Pediatric Surgery, "Grigore Alexandrescu" Clinical Emergency Hospital for Children, 011743 Bucharest, Romania.

: Choledocal cyst is a rare congenital disease of the biliary tree defined by dilatation of the extrahepatic and/or intrahepatic biliary ducts. Untreated, it leads to complications such as cholangitis, stone formation and malignant degeneration. The standard treatment for choledocal cyst is complete excision and subsequent biliary reconstruction via hepaticojejunostomy or hepatiocoduodenostomy. Read More

Stem Cell Res Ther 2022 03 21;13(1):114. Epub 2022 Mar 21.

Department of Pediatric Surgery, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai Key Laboratory of Birth Defect, and Key Laboratory of Neonatal Disease, Ministry of Health, 399 Wan Yuan Road, Shanghai, 201102, China.

Background: The progression of Biliary Atresia (BA) is associated with the number of reactive ductular cells (RDCs) whose heterogeneity in origin and evolution in humans remains unknown. SOX9-positive liver progenitor-like cells (LPLCs) have been shown to participate in RDCs and new hepatocyte formation during cholestatic liver regeneration in an animal model, which implies the possibility that hepatocyte-reprogrammed LPLCs could be a source of RDCs in BA. The present study aimed to elucidate the characteristics of SOX9-positive LPLCs in BA for exploring new possible therapeutic targets by manipulating the bi-differentiation process of LPLCs to prevent disease progression. Read More

BMC Pediatr 2022 03 14;22(1):134. Epub 2022 Mar 14.

Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, 466-8560, Nagoya, Japan.

Background: The purpose of this study was to evaluate the utility of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric patients with pancreaticobiliary diseases.

Methods: A retrospective review was performed on patients who underwent ERCP for the treatment of biliary tract disease and detailed examination of pancreatitis at our institution from January 1999 to December 2020.

Results: ERCP was performed for congenital biliary dilatation (CBD) (n = 42), choledocholithiasis (n = 9), common bile duct stenosis (n = 1), and several types of pancreatitis (n = 13). Read More

Front Pediatr 2022 25;10:806919. Epub 2022 Feb 25.

Department of Pediatric Surgery, Fujian Provincial Hospital, Fuzhou, China.

Objective: We introduce the trans-umbilical single-site plus one robotic-assisted surgery for the treatment of pediatric choledochal cyst. Compare the intraoperative and postoperative outcomes between the new method and traditional laparoscopy-assisted procedure.

Method: We retrospectively analyzed the clinical data of 51 children diagnosed with choledochal cysts and received surgery from June 2019 to December 2020 at our department. Read More

Asian J Surg 2022 07 4;45(7):1432-1433. Epub 2022 Mar 4.

Department of Hepatobiliary and Pancreatic Surgery, The Affiliated Hospital of Yunnan University, The Second People's Hospital of Yunnan Province, Kunming, Yunnan Province, China. Electronic address:

Neonatology 2022 2;119(2):238-245. Epub 2022 Mar 2.

Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital, Tübingen, Germany.

Background: Gastric transposition (GT) is a possible option for esophageal replacement in long-gap esophageal atresia (LGEA). The present study aims to report and compare indications and outcome of laparoscopic-assisted GT (LAGT) versus open (OGT) GT for LGEA repair.

Methods: Retrospective single-center analysis of all LGEA patients undergoing GT between 2002 and 2021. Read More

BMJ Case Rep 2022 Feb 28;15(2). Epub 2022 Feb 28.

Child Health Department, Sultan Qaboos University Hospital, Muscat, Oman

Cystic biliary atresia (BA) is a rare but an important type of BA. An antenatally detected cystic lesion at the porta hepatis raises the suspicion of cystic BA. It is very important to differentiate this from choledochal cyst in infants with cholestasis and cystic lesions. Read More

Pathologica 2022 Feb;114(1):55-63

Department of Emergencies and Organ Transplantation, Pediatric Surgery, University of Bari, Bari, Italy.

Congenital anomalies of the liver, biliary tree and pancreas are rare birth defects, some of which are characterized by a marked variation in geographical incidence. Morphogenesis of the hepatobiliary and pancreatic structures initiates from two tubular endodermal evaginations of the most distal portion of the foregut. The pancreas develops from a larger dorsal and a smaller ventral outpouching; emergence of the two buds will eventually lead to the fusion of the duct system. Read More

Pediatr Surg Int 2022 Apr 14;38(4):541-545. Epub 2022 Feb 14.

Beijing Children's Hospital, Capital Medical University, Beijing, China.

Background: The aim of the current study was to evaluate the efficacy of one- and two-stage single-incision laparoscopic hepaticojejunostomy (SILH) for perforated CDCs with good medical conditions.

Methods: Between June 2015 and December 2020, 57 patients were reviewed: Group 1: patients who underwent one-stage SILH (n = 16); Group 2: patients who underwent two-stage SILH (n = 41). The demographic characteristics, operational details, postoperative outcomes and postoperative complications were evaluated. Read More

Pan Afr Med J 2021 15;40:224. Epub 2021 Dec 15.

Department of Surgery, Muhimbili National Hospital, Dar es Salaam, Tanzania.

Choledochal cyst are rare congenital disease of the biliary tree. It presenting as cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles or both. They are typically a surgical problem of infancy and childhood, but less than a quarter of the patients the diagnosis is delayed until adulthood as it presenting with vague and nonspecific signs and symptoms. Read More

Surg J (N Y) 2022 Jan 1;8(1):e41-e45. Epub 2022 Feb 1.

Department of General Surgery, Seth GS Medical College & KEM Hospital, Mumbai, Maharashtra, India.

 Choledochal cyst is a premalignant condition and surgical excision with biliary enteric anastomosis is the standard of care. Surgical treatment in adults may be difficult due to associated biliary pathology and high incidence of postoperative complications is reported. Postoperative pancreatic fistula (POPF) is a rare early complication following choledochal cyst excision. Read More

Surg Today 2022 Feb 6. Epub 2022 Feb 6.

Gateshead Health NHS Foundation Trust, Gateshead, UK.

Over the past two decades, there have been an increasing number of reports describing a sixth type of choledochal cyst (cystic duct cyst) in adults that was not included in Todani's classification. This sixth entity has not yet been systematically reviewed in the literature. We therefore explored this condition in adults from the perspective of the clinical presentation, diagnosis and treatment through a systematic review of the evidence. Read More