Diseases of bile duct in children.

Several diseases originate from bile duct pathology. Despite studies on these diseases, certain etiologies of some of them still cannot be concluded. The most common disease of the bile duct in newborns is biliary atresia, whose prognosis varies according to the age of surgical correction. Other diseases such as Alagille syndrome, inspissated bile duct syndrome, and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction. The majority of these diseases present with cholestatic jaundice in the newborn or infant period, which is quite difficult to differentiate regarding clinical acumen and initial investigations. Intraoperative cholangiography is potentially necessary to make an accurate diagnosis, and further treatment will be performed synchronously or planned as findings suggest. This article provides a concise review of bile duct diseases, with interesting cases.

Clinical implications of pediatric biliary intraepithelial neoplasia diagnosed from a choledochal cyst specimen.

BACKGROUND: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date. METHODS: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022. BilIN was diagnosed in 15 patients. Subsequent analyses were performed of the demographics, surgical procedures, clinical course, and outcomes in these patients. Subgroup analysis and multivariate logistic regression test were performed to identify factors influencing BilIN occurrence. RESULTS: The mean age of the patients included in our study was 40.1 ± 47.6 months. In 15 patients, BilIN of various grades was diagnosed. Todani type I was prevalent in 80% of the patients. The median age at surgery was 17 months. During a mean follow-up of 63.3 ± 94.0 months, no adverse events such as stone formation in the remnant intrapancreatic common bile duct and intrahepatic duct or cholangiocarcinoma were observed, indicating a favorable outcome until now. CONCLUSIONS: The potential progression of choledochal cysts to BilIN in children was demonstrated. These results could underscore the importance of early and comprehensive excision of choledochal cysts, including resection margins for associated lesions and more thorough postoperative surveillance in patients with or at risk of BilIN.

[Choledochal cysts: surgical treatment in newborns and infants]. / Kisty kholedokha: osobennosti khirurgicheskogo lecheniya u detei pervykh trekh mesyatsev zhizni.

OBJECTIVE: To improve postoperative outcomes in newborns and infants with choledochal cysts and to determine the indications for surgery. MATERIAL AND METHODS: There were 13 children aged 0-3 months with choledochal cyst who underwent reconstructive surgery between 2019 and 2023. In all children, choledochal cyst was associated with cholestasis. Acholic stool was observed in almost half of the group (n=7). All children underwent cyst resection and Roux-en-Y hepaticoenterostomy. RESULTS: Symptoms of cholestasis regressed in all patients. Mean surgery time was 128±27 min. There were no complications. Enteral feeding was started after 1-2 postoperative days, abdominal drainage was removed after 6.2±1.6 days. Mean length of hospital-stay was 16±3.7 days. Adequate bile outflow is one of the main principles. For this purpose, anastomosis with intact tissues of hepatic duct should be as wide as possible. Roux-en-Y loop should be at least 40-60 cm to prevent postoperative cholangitis. CONCLUSION: Drug-resistant cholestasis syndrome and complicated choledochal cysts (cyst rupture, bile peritonitis) are indications for surgical treatment in newborns and infants. When forming Roux-en-Y hepaticoenterostomy, surgeon should totally excise abnormal tissues of the biliary tract to prevent delayed malignant transformation.

Characteristics and malignancy rates of adult patients diagnosed with choledochal cyst in the West: a systematic review.

BACKGROUND: The approach to patients with choledochal cysts (CCs) remains varied and subject to institutional practices. Owing to the rarity of the disease, the optimal treatment remains poorly defined, particularly in the adult population. This study aimed to review the literature on adult patients with CCs to evaluate trends of diagnosis and management in Western countries. METHODS: A literature search of 3 electronic databases was performed on adult patients diagnosed with CCs in Western institutions. A review of published literature was completed with comprehensive screening by 2 independent reviewers. Studies were analyzed, and data on surgical approach, malignancies, and follow-up were collected. Findings are presented in concordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. RESULTS: Of the 3488 articles retrieved, 21 studies evaluated Western adults with CCs for a combined population of 1337 patients. The most common Todani subtypes included types I (64%) and IV (22%). Symptoms at presentation included abdominal pain and jaundice, although many were asymptomatic. Ultrasound was used most frequently for diagnosis, followed by computed tomography and endoscopic cholangiopancreatography. The combined malignancy rate was 10.9%, with cholangiocarcinoma being the most prevalent. Complete extrahepatic cyst resection was standard for type I and IV CCs. Among malignancies, 18.5% and 16.4% were observed in patients with prior resection and internal drainage, respectively. CONCLUSIONS: A significant proportion of patients who undergo resection of CC disease harbor malignancy. Cancer risk seems reduced but not eliminated with complete resection, which remains the standard treatment. Additional studies are needed to standardize guidelines for the diagnosis and postoperative care of patients in Western countries.

Biliary Disorders, Anomalies, and Malignancies in Children.

Biliary abnormalities in children are uncommon, and the spectrum of biliary disorders is broader than in adult patients. Unlike in adults, biliary disorders in children are rarely neoplastic and are more commonly rhabdomyosarcoma rather than cholangiocarcinoma. Pediatric biliary disorders may be embryologic or congenital, such as anatomic gallbladder anomalies, anomalous pancreaticobiliary tracts, various cholestatic processes, congenital cystic lesions, or genetic conditions. They may also be benign, such as biliary filling anomalies, biliary motility disorders, and biliary inflammatory and infectious disorders. Distinguishing these entities with a single imaging modality is challenging. US is the primary imaging modality for initial evaluation of biliary abnormalities in children, due to its wide availability, lack of ionizing radiation, and low cost and because it requires no sedation. Other examinations such as MRI, CT, and nuclear medicine examinations may provide anatomic and functional information to narrow the diagnosis further. Hepatobiliary-specific contrast material with MRI can provide better assessment of biliary anatomy on delayed images than can traditional MRI contrast material. MR cholangiopancreatography (MRCP) allows visualization of the intra- and extrahepatic biliary ducts, which may not be possible with endoscopic retrograde cholangiopancreatography (ERCP). Suspected biliary atresia requires multiple modalities for diagnosis and timely treatment. Determining the type of choledochal cyst calls for a combination of initial US and MRCP. Many benign and malignant biliary masses require biopsy for definitive diagnosis. Knowledge of the imaging appearances of different pediatric biliary abnormalities is necessary for appropriate imaging workup, providing a diagnosis or differential diagnosis, and guiding appropriate management. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Premalignant/malignant histology in excised choledochal cyst specimens from children. Experience and literature review.

BACKGROUND: Dysplasia, carcinoma in situ, and other malignant transformation or premalignant/malignant histopathology (PMMH) seem uncommon in pediatric choledochal cyst (CC). A literature review and the authors' experience are presented. METHODS: All reports about PMMH in CC patients 15 years old or younger published in English and all cases of PMMH in specimens excised from CC patients 15 years old or younger by the authors were reviewed. RESULTS: Of 20 published reports, PMMH was adenocarcinoma (n = 4), sarcoma (n = 4), and dysplasia (n = 12). Treatment for malignancies was primary pancreaticoduodenectomy (PD; n = 2) or cyst excision/hepaticojejunostomy (Ex/HJ; n = 6). Outcomes at the time of writing for malignancies: 2 deaths, 4 survivors after follow-up of 2 years, and 2 lost to follow-up. No dysplasia case has undergone malignant transformation. The authors have experienced 7 cases of PMMH; adenocarcinoma in situ (AIS; n = 1) and dysplasia (n = 6). CONCLUSIONS: The present study identified the youngest cases of AIS and dysplasia from specimens excised when they were 3 years old and 4 months old, respectively. Both are published for the first time as evidence that PMMH can complicate CC in young patients. Long-term protocolized postoperative follow-up is mandatory when PMMH is diagnosed in pediatric CC.

The impact of pre-operative cholecystostomy on laparoscopic excision of choledochal cyst in paediatric patients.

PURPOSE: This aim of this study was to identify the pre-operative risk factors for conversion during laparoscopic excision of choledochal cyst in paediatric patients. METHODS: A retrospective single-centre study was carried out. All paediatric patients (< 18 years) who had undergone laparoscopic excision of choledochal cyst between 2004 and 2021 were reviewed. The outcome was conversion to open surgery and pre-operative factors that affected the conversion rate were analyzed. RESULTS: Sixty-one patients were included. Conversion was required in 24 cases (39.3%). There was no difference in the conversion rate between the first (before 2012, n = 30) and second (after 2012, n = 31) half of the series (36.7% vs. 42.0%, p = 0.674). Majority was type 1 cyst (86.8%) and the median cyst size was 4.6 cm (IQR: 2.2-6.4 cm). Antenatal diagnosis was available in 18 patients (29.5%). The median age at operation was 23.0 months (IQR: 8.0-72.0 months). Pre-operatively, 19 patients (31.1%) suffered from cholangitis and 5 (8.2%) of them required cholecystostomy. Comparing patients with successful laparoscopic surgery (L) and converted cases (C), there were no differences in the age at operation (p = 0.74), cyst size (p = 0.35), availability of antenatal diagnosis (p = 0.23) and cholangitic episodes (p = 0.40). However, a higher percentage of patients required cholecystostomy in the converted group (L vs. C = 2.7% vs. 16.7%, p = 0.05). Using logistic regression analysis, it was also a risk factor for conversion (OR = 3.5 [1.37-5.21], p = 0.05). CONCLUSION: Pre-operative cholecystostomy is a potential risk factor for conversion during laparoscopic excision of choledochal cyst in children.

Robotic Surgery for Pediatric Choledochal Cysts: An American Case Series and Literature Review.

INTRODUCTION: Choledochal cysts are rare congenital biliary cystic dilations. The US incidence rate varies between 5 and 15 cases per 1,000,000 people. In contrast, Asians, which are a large subset of the population of Hawaii, have an incidence of approximately one in every 1000 births. We report our experience with robot-assisted laparoscopic surgical management with biliary reconstruction of choledochal cysts which to date is the largest American case series to be reported. MATERIALS AND METHODS: From 2006 to 2021, patients diagnosed with a choledochal cyst(s) at a tertiary children's hospital were retrospectively reviewed. Perioperative analysis was performed. Complications were defined as immediate, early, or late. The data underwent simple descriptive statistics. RESULTS: Nineteen patients underwent choledochal cystectomy and hepaticoduodenostomy. Thirteen underwent a robotic approach while the rest were planned laparoscopic. Eighteen of 19 were female with 15/19 of Asian descent. The ages ranged from 5 mo to 21 y. Presenting diagnoses included jaundice, primary abdominal pain, pancreatitis, and cholangitis. Sixty eight percent had type 1 fusiform cysts while the rest were type 4a. Operative time and length of stay for robotic versus laparoscopic were 321 versus 267 min and 8.2 versus 17.3 d, respectively. For the robotic group, there was one immediate complication due to peritonitis. One-year follow-up revealed two patients requiring endoscopic retrograde cholangiopancreatography with dilation/stenting for an anastomotic stricture. There were no anastomotic leaks. CONCLUSIONS: Robot-assisted laparoscopic choledochal cystectomy with hepaticoduodenostomy is associated with overall good outcomes with the most common long-term complication being anastomotic stenosis.

Giant choledochal cyst in infant: A rare case report.

The cystic dilatation of the common bile duct (CBD) is a rare pathology in an infant. It is the second-most common surgical cause of cholestatic jaundice in infants after biliary atresia. A 4-month-old female child was admitted to our department with complaints of abdominal distension. The physical examination revealed the presence of a huge palpable mass involving the right hypochondrium up to the right iliac fossa and umbilical region. Ultrasound abdomen revealed a large intra-abdominal cyst but unable to comment on the organ of origin of the cyst due to its huge size. Multidetector computed tomography of the abdomen was suggestive of possible origin of the cyst from CBD extending from porta hepatis to pelvis. At laparotomy, there was a huge choledochal cyst extending from porta hepatis to pelvis. The choledochal cyst was excised, followed by Roux-en-Y hepaticojejunostomy.

Correlation of ectopic distal location of papilla of Vater and clinical characteristics in pediatric choledochal cysts.

PURPOSE: Ectopic distal location of papilla of Vater (EDLPV) is an obvious pathological feature of choledochal cyst (CDC). This study aimed to investigate the correlation between EDLPV and clinical characteristics of CDCs. METHODS: Three groups were studied: Group 1 (G1), papilla in the middle third of second part of duodenum (n = 38); Group 2 (G2), papilla from the distal third of second part to the beginning of third part of duodenum (n = 168); Group 3 (G3), papilla from the middle of third part to fourth part of duodenum (n = 121). Relative variables among three groups were compared. RESULTS: Compared with G1 and G2, G3 patients had the largest cysts (relative diameter: 1.18 vs. 1.60 vs. 2.62, p < 0.001), the youngest age (20.52 vs. 19.47 vs. -3.40 months, p < 0.001), the highest rate of prenatal diagnosis (26.32% vs. 36.31% vs. 62.81%, p < 0.001), the lowest occurrence of protein plugs in common channel (44.74% vs. 38.69% vs. 16.53%, p < 0.001), and the most elevated total bilirubin level (7.35 vs. 9.95 vs. 28.70 µmol/L, p < 0.001). Prenatally diagnosed G3 patients had heavier liver fibrosis than G2 (13.16% vs. 1.67%, p = 0.015). CONCLUSION: The more distal papilla location, the more severe clinical characteristics of CDCs, suggesting a crucial role in its pathogenesis.

Study of Operative Events and Time Requirement of Hepaticoduodenostomy for the Treatment of Type I Choledochal Cyst- the Experience at BSMMU Hospital.

Of all varieties, Type I Choledochal cyst causing saccular or fusiform dilatation of the extra-hepatic biliary ductal system is the commonest (90.0 - 95.0%). Its presentations vary. To restore the continuity of the extra-hepatic biliary tract after excision of type I Choledochal cyst, surgeons have few alternatives to use, with their advantages and disadvantages. Roux en-Y Hepatico-jejunostomy (RYHJ) has been very popular and long studied standard surgical treatment for type I Choledochal cyst. But now Hepatico-duodenostomy (HD) is also being practiced and studied in different centers all over the world for the treatment of the same disease. For the last five years, we, at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, have been using Hepatico-duodenostomy as preferred anastomotic option in treating type I Choledochal cyst. Here, we are presenting our experience at BSMMU Hospital, regarding operative events and time requirement of Hepaticoduodenostomy for the treatment of type I Choledochal cyst and, to show whether this procedure can be safely practiced, producing acceptable results. It is a retrospective document study, from January 2013 to December 2017, at BSMMU Hospital, on forty two, MRCP confirmed type I Choledochal cyst patients of pediatric age. Patients' particulars, history, physical examination, investigations (including MRCP confirmation), assessment, surgical plan were collected from relevant medical records and documented in duly coded individual data collection sheet maintaining standard privacy protocol. Information regarding presentations, operative findings and procedural events including per-operative mortality, injury to the vital structures during operation, conversion to RYHJ, operative time (minutes), blood loss and transfusion requirements (ml) of Heaticoduodenostomy for type I Choledochal cyst, were specially searched for. There was no operative mortality. None of these patients required per-operative blood transfusion. Nor there was any inadvertent injury to the adjacent structures. The mean operative time required for Hepaticoduodenostomy was 88 minutes with a range of 75 to 125 minutes. Through this study, at BSMMU Hospital, operative events and time requirement of Hepatico-duodenostomy for treating type I Choledochal cyst, was found to be yielding acceptable results, for safe practice.

Experience of surgical management of rare cases of choledochal cyst in last five years-tertiary care reflection.

Choledochal Cyst (CC), also known as the biliary cyst, is one of the rare inherited anomalies of intrahepatic and/or extrahepatic biliary system characterised by varying degrees of cystic dilatation of the biliary tract without acute obstruction. The prevalence ranges from 1 in 13,000 people to 1 in 2 million people with preponderance in Asia1, particularly in Japan. Moreover, the presentation also varies in children and adults, and is usually vaguer and non-specific in adults. The prevalence is even lower in males, with female to male ratio being 3:1-4:12. We present here three cases of adult choledochal cysts excised in our surgical unit in the last five years. We discuss the aetiopathogenesis, presentation, diagnosis, surgical treatment, and complications of choledochal cysts based on the available literature. It is crucial to establish a multidisciplinary group of professionals, that comprises paediatric surgeons, pathologists, paediatric gastroenterologists, physiotherapists, nutritionists, oncologists, and radiologists, to get acceptable outcomes in diagnosing and treating children with choledochal cysts.

The Factors Associated with the Selection of Early Excision Surgery for Congenital Biliary Dilatation with a Prenatal Diagnosis.

PURPOSE: The aim of this study was to clarify the appropriate management after birth for congenital biliary dilatation (CBD, choledochal cyst) patients with a prenatal diagnosis. METHOD: Thirteen patients with a prenatal diagnosis of CBD who underwent liver biopsy during excision surgery were divided into two groups and retrospectively analyzed: group A, with liver fibrosis above F1 and group B, without liver fibrosis. RESULTS: Excision surgery was performed earlier in group A (F1-F2), at a median of 106 days old (p = 0.04). There were significant differences between the two groups in the presence symptoms and sludge, the cyst size, and the level of serum bilirubin and gamma glutamyl transpeptidase (GGT) before excision surgery (p < 0.05). Especially, in group A, prolonged serum GGT elevation and larger cysts were consistently observed from birth. The cut-off values of predictions for the presence of liver fibrosis in serum GGT and cyst size were 319 U/l and 45 mm. No significant differences were observed in the postoperative liver function or complications during the follow-up period. CONCLUSION: In patients with prenatally diagnosed CBD, the postnatal serial changes of serum GGT values and cyst size, in addition to symptoms, could help to prevent progressive liver fibrosis. LEVEL OF EVIDENCE: Ⅲ. TYPE OF STUDY: Treatment Study.

Surgical outcomes after re-operation for excision of choledochal cyst with delayed biliary complications: A retrospective study on 40 patients.

PURPOSE: The purpose of this study is to review our experience in patients who underwent re-operation for delayed biliary complications after choledochal cyst (CDC) excision. METHODS: All the patients who underwent re-operation. for delayed biliary complications after CDC excision between August 2007 and July 2020 were included in this retrospective study. The outcomes of these patients were compared with those who underwent primary surgery (CDC excision) at our institution. RESULTS: Of the total 40 patients with delayed biliary complications, 25 (62.5%) were female. Thirty-seven (92.5%) patients had a history of cholangitis. The median interval between CDC excision and the reoperation was 70 (4-216) months. The median duration of symptoms before reoperation was 12 (2.5-84) months. Re-do hepaticojejunostomy and direct hepaticojejunostomy were performed in 34 and in 6 patients respectively. Median operative time and blood losses were 219 min and 150 ml respectively. The median postoperative stay was 9 days. Postoperative complications developed in 10 (25%) patients. There was no operative mortality. Over a median follow-up of 71 months, a satisfactory outcome was achieved in 86% of patients. Restricture and intrahepatic stones developed in three and two patients respectively. Incidence of type IV cyst, cholangitis before operation, and operative blood loss were significantly more in the re-operative group. Clinical outcomes like the incidence of recurrent cholangitis, re-stricture, and postoperative hospital stay were comparable between the two groups. CONCLUSION: Surgery affords excellent results for majority of the patients with delayed biliary complications after CDC excision. Type IV cysts are more commonly associated with the development of delayed biliary complications.

[Robot-assisted choledochal cyst resection in a child]. / Robot-assistirovannaya rezektsiya kisty kholedokha u rebenka.

The generally accepted method for choledochal cysts is total resection of cystic extrahepatic bile ducts and gallbladder followed by biliodigestive anastomosis. Minimally invasive interventions have recently become the «gold¼ standard in pediatric hepatobiliary surgery. However, laparoscopic resection of choledochal cysts has certain disadvantages related to difficult positioning of instruments in narrow surgical field. The disadvantages of laparoscopy can be compensated by surgical robots. A 13-year-old girl underwent robot-assisted resection of hepaticocholedochal cyst, cholecystectomy and Roux-en-Y hepaticojejunostomy. Total anesthesia time was 6 hours. Laparoscopic stage took 55 min, docking of robotic complex - 35 min. Robotic stage of surgery required 230 min, removal of cyst and suturing the wounds - 35 min. Postoperative period was uneventful. Enteral nutrition was started after 3 days, and drainage tube was removed after 5 day. The patient was discharged after 10 postoperative days. The follow-up period was 6 months. Thus, robot-assisted resection of choledochal cysts in children is possible and safe.

Development and validation of non-invasive models in predicting advanced fibrosis of choledochal cyst.

PURPOSE: Patients with choledochal cyst (CDC) develop liver fibrosis, especially advanced fibrosis without prompt surgery. This study validated the aspartate aminotransferase (AST)-to-platelet ratio index (APRI) and Fibrosis-4 index (FIB-4) and constructed a model for predicting advanced fibrosis in pediatric CDCs. METHODS: Between January 2020 and March 2022, 330 CDCs (advanced fibrosis: 34, Ludwig staging 3-4; non-advanced fibrosis: 296, Ludwig staging 0-2) were reviewed. APRI and FIB-4 were validated. The area under the receiver operating characteristic (AUROC) curve was used to assess discrimination. Relevant variables were analyzed by backward stepwise logistic regression. Enhanced bootstrap method was used for internal verification with 1000 samples. RESULTS: The AUROCs of APRI and FIB-4 were 0.761 (0.673-0.850) and 0.561 (0.455-0.667). AST to prealbumin ratio (APAR), was constructed with an AUROC of 0.776 (0.693-0.860). The AUROCs of APAR + APRI and APAR + FIB-4 were 0.791 (0.713-0.869) and 0.782 (0.699-0.865). No significant differences were noted in the AUROCs of the indices or their combinations. APAR and APRI could be used together to reduce missed diagnosis rate. The risk of advanced fibrosis varied from different APAR and APRI scores. CONCLUSION: Both APAR and APRI were indispensable to identify CDC patients at high risk of advanced fibrosis.

Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East.

BACKGROUND AND STUDY AIMS: Choledochal cysts are rare congenital cystic dilatations of the bile ducts that occur in fewer than 1% of individuals. The disease is common in East Asia, and most of the literature concerns those populations, but some data about Western populations have been published recently. Long-term reports about the disease in Middle Eastern populations, however, are currently lacking. We report a single-center 20-year experience in diagnosing and managing choledochal anomalies. PATIENTS AND METHODS: Participants were adult patients in whom choledochal cysts were diagnosed over a 20-year (2000-2019) period at a single tertiary academic care center. Clinical data, including radiologic imaging findings, were retrieved from the patients' medical records. To describe the baseline characteristics of the population, we calculated descriptive statistics. RESULTS: Choledochal anomalies were diagnosed in 19 adult patients, whose median age was 30 years (interquartile range [IQR], 23-67 years). Of the choledochal cysts 13 (68.4%) were classified as Todani type I, 4 (21.1%) as Todani type IV, and 3 (15.8%) as Todani type V (Caroli's disease). No patient had underlying chronic liver disease, and liver synthetic function was preserved in all. Eighteen patients (94.7%) underwent surgery: cyst excision with Roux-en-Y hepaticojejunostomy in 17 and liver transplantation in 1. All 18 survived surgery, and the median postoperative hospital stay was 11 days (IQR, 5-34 days). All 18 were alive 90 days after surgery, and the median follow-up period was 40 months (IQR, 12-140 months). Seven patients (36.8%) developed postoperative surgical complications; 2 patients required rehospitalization, and 1 required reoperation. CONCLUSION: This description of adults with choledochal cysts is the latest long-term report about this disease in the Middle East. In our 20-year experience, the disease characteristics in our patients were moderately consistent with those described previously.

Diagnosis and minimally invasive treatment of type III choledochal cysts.

BACKGROUND: Type III choledochal cysts (CCs) are the rarest and least well-characterized of the five variants of biliary cysts. Only a few relevant studies have been conducted and a gold standard treatment for type III CCs is still lacking because of their scarcity. An unusual case of type III CC with choledochocele at the end of the distal common bile duct (CBD) with no bulging of the duodenum is presented here. CASE PRESENTATION: A 61-year-old woman presented to our department with repeated upper abdominal pain for one year. Laboratory examination revealed abnormal liver function and a slight increase in the white blood cell (WBC) count and proportion of neutrophils. In an MRCP of the patient, the distal CBD was found to have a cystic structure indicative of a type III CC. Endoscopic retrograde cholangiopancreatograpy (ERCP) revealed cystic findings compatible with Todani type III CCs. However, duodenoscopy did not show a bulge in the duodenum so laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy were performed. In her 6-month follow-up, the patient reported that recovery from the operation was uneventful. CONCLUSIONS: ERCP has become the gold standard for diagnosing type III CCs. Type III CCs can be treated minimally invasively with laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy.

Pediatric Hepatic Cystic Lesions: Differential Diagnosis and Multimodality Imaging Approach.

When a pediatric hepatic cystic lesion is identified at imaging, the differential diagnosis may be broad, including developmental, infectious, neoplastic, and posttraumatic or iatrogenic causes. The location of a cystic lesion and its number, size, composition, and relationship to the biliary system are features that help in narrowing the differential diagnosis. An incidentally detected simple hepatic cyst is the most commonly encountered. Ciliated foregut cysts are typically located in hepatic segment IVa. The presence of multiple cysts should raise suspicion for fibropolycystic liver disease, a group of related lesions-including biliary hamartoma and choledochal cyst-caused by abnormal embryologic development of the ductal plate. Communication of the cystic lesion with the biliary tree can confirm the diagnosis of choledochal cyst. In a neonate with jaundice, a cystic lesion at the porta hepatis should raise suspicion for choledochal cyst versus cystic biliary atresia. Hepatic abscess can appear cystlike, though typically with internal contents. In an immunocompromised child, multiple cystlike lesions should raise concern for fungal microabscesses. A complex cystic mass in a young child should raise suspicion for mesenchymal hamartoma, which can evolve into undifferentiated embryonal sarcoma if untreated. Hepatic hematoma and biloma can appear cystlike in children with a history of trauma or recent intervention. In neonates with an umbilical vein catheter (UVC), an intrahepatic cyst along the course of the UVC should raise concern for infusate extravasation. Familiarity with imaging findings and clinical features is essential for achieving accurate diagnosis of pediatric hepatic cystic lesions, which in turn can guide appropriate clinical management. Online supplemental material is available for this article. ©RSNA, 2022.