Search Results (9)

Introduction: Near-infrared fluorescence (NIRF) imaging with indocyanine green (ICG) is now widely regarded as a valuable aid in decision-making for complex hepatobiliary procedures, with increasing support from recent studies. Methods: We performed a systematic review following PRISMA guidelines, utilizing PubMed, CINAHL, and EMBASE databases to locate studies on the perioperative use ICG in pediatric hepatobiliary surgeries. Two independent reviewers assessed all articles for eligibility based on predefined inclusion criteria. We collected data on study design, patient demographics, surgical indications, ICG dosing, timing of ICG injection, and perioperative outcomes. Results: Forty-three articles, including 930 pediatric patients, from 1989 to 2025 met the inclusion criteria for narrative synthesis in our systematic review, of which 22/43 (51.2%) were retrospective studies, 15/43 were case reports (34.9%), 3/43 (7.0%) were experimental studies, and the other three were prospective comparative studies (7.0%). The current clinical applications of ICG in hepatobiliary pediatric surgery include bile duct surgery (cholecystectomy, choledochal cyst, biliary atresia), reported in 17 articles (39.5%), liver tumor resection, reported in 15 articles (34.9%), liver transplantation, reported in 6 articles (14.6%), and liver function determination, reported in 5 articles (12.2%). Conclusions: ICG fluorescence navigation in pediatric hepatobiliary surgery is a highly promising and safe technology that allows for the intraoperative localization of anatomic biliary structures, aids in the identification and resection of liver tumors, and can accurately determine hepatic function. The lack of comparative and prospective studies, and the variability of the dose and timing of administration are the main limitations. Full article

Purpose: Forme fruste choledochal cyst (FFCC) is a choledochal cyst with minimal or no dilatation of the extrahepatic bile duct (EHBD) and is usually associated with an anomalous pancreaticobiliary junction (APBJ). While sharing similar symptoms, inflammation, and malignant potential with classic biliary cysts, FFCC is often overlooked on ultrasound. This paper aims to present the experience of two tertiary pediatric centers in managing FFCC. Methods: In this retrospective study, the clinical data of pediatric patients treated for FFCC at two tertiary pediatric surgical centers between 1 January 2008 and 31 December 2023 were analyzed. The primary outcome was the clinical success of the surgical procedure, defined by the resolution of symptoms and the absence of major complications. Secondary outcomes included postoperative complications, type and duration of surgical procedures, and length of hospital stay. All patients underwent biliary reconstruction via either Roux-en-Y hepatico-jejunostomy or hepatico-duodenostomy. Clinical outcomes, including postoperative complications and patient follow-up, were evaluated. Results: Fourteen children (9 girls, 5 boys; aged 18 months to 12 years) underwent surgical treatment of FFCC. The mean age at surgery was 5.3 ± 3.8 years, and the mean diameter of the common bile duct was 7.9 ± 1.2 mm. Thirteen patients underwent Roux-en-Y hepatico-jejunostomy, and one underwent hepatico-duodenostomy. Over a mean follow-up period of 6.2 ± 3.6 years, no cholangitis or anastomotic stricture cases were observed. Two patients (14.3%) experienced minor wound infections managed conservatively. Conclusions: FFCC remains a diagnostic challenge due to its subtle imaging findings and non-specific clinical presentation. However, once identified, surgical excision with biliary reconstruction, most commonly via Roux-en-Y hepatico-jejunostomy, is a safe and effective treatment with excellent long-term outcomes. Given the potential for serious complications if left untreated, FFCC should be actively considered in pediatric patients with unexplained pancreatitis or biliary symptoms, even in the absence of overt ductal dilatation. Full article

Choledochal cysts (CCs), a congenital anomaly resulting in the abnormal dilation of the biliary ductal system, are most often identified in patients younger than 10 years of age. Regardless of clinical presentation, the cornerstone of therapy for CCs is complete surgical excision with reconstruction with either hepaticoduodenostomy or hepaticojejunostomy. Although both procedures are used by surgeons for the correction of CCs, evidence on clinical outcomes following both approaches is inconclusive as to which may offer superior reconstruction. This narrative review aims to compare the current literature regarding both approaches by evaluating their anatomic and operative considerations, as well as their perioperative, postoperative, and long-term outcomes. Future studies should closely focus on long-term, comparative outcomes, including the risk of biliary malignancy, and refine techniques to minimize complications, such as biliary reflux and bowel obstruction, in order to improve care for pediatric patients undergoing treatment for CCs. Full article

Rhabdomyosarcoma (RMS) of the biliary tract is a rare tumor in children, constituting 0.5–0.8% of all pediatric RMS. Still, it is the most common malignancy in this location in children. Due to its rarity and location, it may cause diagnostic and treatment difficulties. Above all, there are no therapeutic guidelines specific for this tumor location. The aim of the study was to present an analysis of our experience with the treatment of children with biliary tract rhabdomyosarcoma (RMS) and discuss clinical recommendations for this specific location published in the literature. A retrospective analysis of medical records of eight children with biliary tree RMS treated in one center between 1996–2022 was performed. Records of eight children, five boys and three girls aged 2 yrs 6 mo to 16 yrs 9 mo (median—6 yrs) were analyzed. All patients presented with jaundice as the first symptom. In two patients, initial diagnosis of a tumor was established. For the remaining six, the primary diagnoses were as follows: choledochal cyst—one, malformation of the biliary ducts—one, choledocholithiasis—one, cholangitis—three. In four patients, the extrahepatic bile ducts were involved; in four patients, both the intrahepatic and extrahepatic bile ducts were involved. Embryonal RMS was diagnosed in seven patients (three botryoides type). Alveolar RMS was found in one patient. Biopsy (three surgical, four during endoscopic retrograde cholangiopancreatography (ERCP)) was performed in seven patients. One child underwent primary partial tumor resection (R2). Seven patients received neoadjuvant chemotherapy, followed by delayed resection in five, including liver transplantation in one (five were R0). Two patients did not undergo surgery. Radiotherapy was administered in four patients (two in first-line treatment, two at relapse/progression). Six patients (75%) are alive with no evidence of disease, with follow-up ranging from 1.2 yrs to 27 yrs (median 11 yrs. and 4 mo.). Two patients died from disease, 2 y 9 mo and 3 y 7 mo from diagnosis. Children presenting with obstructive jaundice should be evaluated for biliary tract RMS. The treatment strategy should include biopsy and preoperative chemotherapy, followed by tumor resection and radiotherapy for residual disease and in case of relapse. Full article

Hydatid cyst is a common name for the larval stage of a tapeworm species of Echinococcus granulosus, which is transmitted from animals to humans via the fecal–oral route. Hydatid cysts predominantly affect the liver (75%), followed by the lung (15%), and they can affect many organs in the human body. Medical imaging modalities are the keystone for the diagnosis of hydatid cysts with high sensitivity and specificity. Ultrasound imaging with high resolution is the first choice for diagnosis, differential diagnosis, staging, establishing a role in interventional management, and follow-up, and it can differentiate Type I hydatid cysts from simple liver cysts. Unenhanced computed tomography (CT) is indicated where or when an ultrasound is unsatisfactory, such as with chest or brain hydatid cysts, when detecting calcification, and in obese patients. Magnetic resonance imaging (MRI) is superior for demonstrating cyst wall defects, biliary communication, neural involvement, and differentiating hydatid cysts from simple cysts using diffusion-weighted imaging (DWI) sequences. According to the phase of growth, hydatid cysts occur in different sizes and shapes, which may mimic benign or malignant neoplasms and may create diagnostic challenges in some cases. Hydatid cysts can mimic simple cysts, choledochal cysts, Caroli’s disease, or mesenchymal hamartomas of the liver. They can mimic lung cystic lesions, mycetoma, blood clots, Rasmussen aneurysms, and even lung carcinomas. Differential diagnosis can be difficult for arachnoid cysts, porencephalic cysts, pyogenic abscesses, and even cystic tumors of the brain, and can create diagnostic dilemmas in the musculoskeletal system. Full article

Choledochal cysts (CCs) are rare occurrences presenting as dilatations of biliary structures, which can present as single or multiple dilatations and can appear as both intra- and extrahepatic anomalies. The most widespread classification of CCs is the Todani classification, but there have been numerous reports of cysts that do not fall into any of the types described. We present such a case—a male patient 36 years of age who underwent preoperative CT, MRCP, and ERCP, which mistakenly indicated a type II Todani CC, and intraoperatively was found to be located at the confluence of the hepatic ducts and encompassed the origin of the common bile duct. Complete resection of the cyst and the proximal segment of the common bile duct was performed, and reconstruction was carried out by Roux-en-Y double-tutorized hepaticojejunostomy. Considering the risk of malignant transformation, the frequent preoperative misdiagnosis, as well as the technically challenging surgery required in such cases, we advocate for a revision of the classification and raise awareness of the need for guidelines regarding the proper short-term and long-term management of this disease to ensure adequate quality of life and disease-free survival for patients. Full article

A choledochal cyst is a rare malformation primarily diagnosed in children. The only effective therapy remains surgical cyst resection followed by Roux-en-Y hepaticojejunostomy. Treating asymptomatic neonates remains a point of discussion. Between 1984 and 2021, we performed choledochal cyst (CC) excision in 256 children at our center. Out of this group, we retrospectively reviewed the medical records of 59 patients who were operated on under one year of age. Follow-up ranged from 0.3 to 18 years (median 3.9 years). The preoperative course was asymptomatic in 22 (38%), while 37 patients (62%) had symptoms before surgery. The late postoperative course was uneventful in 45 patients (76%). In symptomatic patients, 16% had late complications, while in asymptomatic patients, only 4%. Late complications were observed in the laparotomy group in seven patients (17%). We did not observe late complications in the laparoscopy group. Early surgical intervention is not followed by a high risk of complications and may prevent the onset of preoperative complications, giving excellent early and long-term results, especially after minimally invasive laparoscopic surgery. Full article

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. Full article

The concomitant occurrence of duodenal atresia (DA) and a choledochal cyst (CC) has rarely been reported. Knowledge of both the presentation and management of this rare co-occurrence is imperative in avoiding potential complications and sequelae, such as biliary metaplasia. Herein we describe a female infant born at 32 weeks gestational age who was diagnosed with duodenal atresia and annular pancreas postnatally, who had subsequent findings of malrotation and a choledochal cyst, as seen from contrast imaging. Uncomplicated repair of the DA and obstruction was performed at 4 days of life. She re-presented 2 years later with non-bloody, nonbilious emesis and was found to have elevated amylase, lipase and liver enzymes. Imaging revealed dilated intra-hepatic ducts, a distended gallbladder and a large choledochal cyst. She underwent a cholecystostomy tube placement followed by a definitive choledochal cyst excision with immediate improvement following surgery and full resolution of symptoms before discharge. Full article