The timing of minimally invasive surgery for prenatally diagnosed choledochal cysts.

OBJECTIVE: There are no clear evidence-based recommendations concerning when patients with prenatally diagnosed choledochal cysts (CCs) should undergo surgery. This study was primarily designed to explore the proper timing of minimally invasive surgery for prenatally diagnosed CC patients. METHODS: Seventy-three patients with prenatally diagnosed CC were enrolled in this study and divided into 4 subgroups according to age at surgery (15 patients in the < 1 month group, 27 in the 1-2 months group, 14 in the 2-3 months group and 17 in the > 3 months group). Eighty-five healthy infants were recruited and divided into 4 age groups (29 in the < 1 month group, 20 in the 1-2 month group, 19 in the 2-3 month group and 17 in the > 3 month group). Preoperative data were collected and compared between CC patients and healthy controls in 4 age groups. Additionally, 73 patients were divided into laparoscopic and open groups to compare postoperative recovery indices and the occurrence of complications to determine the safety and feasibility of laparoscopic CC application in neonates and young infants. RESULTS: Twenty-one of 73 (28.8%) patients who were prenatally diagnosed with CCs experienced various clinical symptoms, and 15 of 21 (71.4%) patients experienced clinical symptoms less than 2 months after birth. No differences were found in alanine transaminase (ALT), aspartate transaminase (AST) or aspartate transaminase (APRI) levels between CC patients and controls at ≤ 1 month or 1-2 months of age (all p > 0.05), while higher levels were found in CC patients at 2-3 months or > 3 months of age (all p < 0.05). ALT, AST and DBIL levels 1 week after surgery were significantly lower than those before surgery in CC patients who underwent laparoscopic CC excision at > 2 months of age, while DBIL levels 1 week after surgery were also significantly lower than those before surgery in patients who underwent CC excision at ≤ 2 months of age. The initial oral feeding time in the laparoscopic surgery group was significantly earlier than that in the open surgery group for both CC patients who underwent CC excision at ≤ 2 months of age and those > 2 months of age (all p < 0.05). No differences were found in the rates of anastomotic leakage or stricture formation between the laparoscopic and open surgery groups at ≤ 2 months or > 2 months of age. CONCLUSION: Most clinical symptoms attributed to CC occur less than 2 months after birth, while liver function and liver fibrosis may deteriorate after 2 months of age in patients with prenatally diagnosed CC. Laparoscopic surgery for CC in newborns and young infants (either less than or more than 2 months old) is safe and feasible and can shorten the initial oral feeding time without increasing complications such as postoperative anastomotic leakage or stricture. Thus, performing laparoscopic CC excisions within 2 months after birth in patients with prenatally diagnosed CC may be appropriate.

Cholangiocarcinoma identified in perforated choledochal cyst in a 3-year-old boy.

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient.

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.

An observational study on the prevalence of choledochal cyst with pancreatitis: Geographical implications and management.

The objective of this study was to analyse the prevalence, risk factors and need for intervention in a sample of Indian children with choledochal cyst (CDC) complicated by pancreatitis with a special focus on chronic pancreatitis. A retrospective review of medical records of children admitted with CDC over 11 years was done and pancreatitis identified using INSPPIRE guidelines. Children were divided into two groups-one having choledochal cyst alone and the other choledochal cyst along with pancreatitis to determine associated risk factors. 40.2% of children with CDC had pancreatitis based on elevation of enzymes or radiological imaging. Age, total bilirubin and indirect bilirubin, requirement of intervention was significantly higher in the group with pancreatitis. 47% of those with radiological features of pancreatitis had imaging features of chronic pancreatitis. Chronic pancreatitis has not been reported previously in children with CDC and maybe peculiar to the Indian subcontinent.

Surgical Strategies for Neonates with Prenatally Diagnosed Congenital Biliary Dilatation.

BACKGROUND: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. METHODS: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. RESULTS: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. CONCLUSIONS: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. LEVEL OF EVIDENCE: Level IV.

Does Congenital Biliary Dilatation Todani Type II (Diverticular Type) Really Exist?

AIM OF THE STUDY: The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD). METHOD: Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated. RESULTS: Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases. CONCLUSION: Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications. LEVEL OF EVIDENCE: Level III.

Choledochocele with hyperplastic epithelium in a patient who developed severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy: a case report.

Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia.

Laparoscopic indocyanine green fluorescence imaging technique in rare type II cystic biliary atresia.

In recent years, it has been reported that indocyanine green can be used for intraoperative navigation in Kasai surgery. However, there are no reports of its application in surgery for rare type II cystic biliary atresia. We report a girl presented with jaundice and light-colored stools. Laboratory tests showed impaired liver function with elevated serum bilirubin and bile acid levels. The abdominal ultrasound and MRCP suggested a common hepatic duct cyst. A diagnosis of choledochal cyst was suspected and biliary atresia could not be excluded. Conservative treatment was unsatisfactory. Laparoscopic exploration with indocyanine green fluorescence was performed on the 38th day of her life, and intraoperative diagnosis of type II CBA was made because the common hepatic duct cyst and its downstream anatomical structures did not show fluorescence. The postoperative bilirubin and bile acid levels decreased significantly and she was discharged two weeks after surgery. This result suggests that indocyanine green can be safely used in laparoscopic surgery for type II CBA, which not only helps in the differential diagnosis of CBA and choledochal cyst, but also confirms bile flow in real time.

Risk factors analysis for clinical symptoms of prenatally diagnosed choledochal cysts: a retrospective study.

BACKGROUND: This study aimed to screen the impact factors for clinical symptoms of prenatally diagnosed choledochal cysts (CDCs), to warn about the occurrence of clinical symptoms and the timing of surgery. METHODS: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital from April 2013 to April 2018 were retrospectively reviewed. Fetal hilar or abdominal cysts were found by prenatal ultrasonogram. All patients underwent laparoscopic cyst excision and hepaticojejunostomy in our center. Univariate analysis and multivariate logistic regression analysis were performed to screen the factors related to clinical symptoms intimately. RESULTS: Two hundred eighteen cases were included. One hundred thirty-four patients (134/218, 61.5%) presented clinical symptoms before surgery. The results of univariate analysis showed that patients with clinical symptoms had earlier time of prenatal diagnosis (P = 0.002), higher values of GGT, TBIL, DBIL (P < 0.001, P < 0.001, P < 0.001, respectively) and larger maximum diameter of cyst before surgery (P = 0.012). Multivariate logistic regression analysis suggested that the time of prenatal diagnosis (P = 0.001, OR = 0.898, 95% CI: 0.845 ~ 0.955) and the GGT value within one week of life (P = 0.028, OR = 1.002, 95% CI: 1.000 ~ 1.003) were independent influencing factors for symptoms. CONCLUSIONS: For children with prenatally diagnosed CDCs, approximately 2/3 patients presented noticeable clinical symptoms before surgery. The time of prenatal diagnosis and the GGT value within 1 week of life were independent impact factors for the occurrence of clinical symptoms.

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.

The strategy of laparoscopic surgery for asymptomatic antenatally diagnosed choledochal cyst.

BACKGROUND: The aim was to investigate the characteristics, surgical management, and outcomes of asymptomatic patients with antenatally diagnosed choledochal cysts (ADCCs) and to justify the strategy of laparoscopic surgery (LS) for them in our hospital. METHODS: We developed our LS strategy for asymptomatic ADCCs. Patients with asymptomatic ADCCs who underwent LS or open surgery (OS) during January 2010 and January 2020 were included. Patients with recorded symptomatic ADCCs were exclude. All data of group LS and group OS were statistically compared and analyzed. RESULTS: Twenty-five patients received LS and 18 patients received OS were included. There was no significant difference in baseline characteristics between the groups. A total of 65.1% of biliary sludge formation was detected preoperatively by ultrasonography (US) (72.0% in group LS, 55.6% in group OS, P = 0.26). Compared to the OS group, the LS group had a longer operative time (313.4 ± 27.2 vs. 154.0 ± 11.9 min, P = 0.02), shorter postoperative fasting period (3.1 ± 1.2 vs. 6.2 ± 2.3 days, P = 0.03), and shorter postoperative hospital stay (5.1 ± 1.9 vs. 9.2 ± 1.1 days, P = 0.03). The incidence of late complications, such as reflux cholangitis, adhesive intestinal obstruction, and biliary enteric anastomotic stricture with stone formation, was not significantly different between the two groups. The liver function and liver stiffness of all patients in the two groups were normal. CONCLUSIONS: Based on the strategy for asymptomatic ADCCs in our hospital, the perioperative safety and midterm follow-up results after LS were satisfactory and comparable to those after OS.

Choledochal cyst.

Choledochal cyst (CC) or congenital biliary dilatation, has a skewed distribution with hereditary features that is far more common in East Asian females. CC is usually associated with pancreaticobiliary malunion (PBMU) forming a common channel. CC requires early definitive diagnosis, since there is a risk for malignancy occurring in the CC and/or intrahepatic bile ducts (IHBD). Complete CC excision and Roux-en-Y hepaticoenterostomy is required and can be performed by open or minimally invasive surgery with hepatojejunostomy the recommended procedure of choice. Principles of open surgical intervention form the basis of minimally invasive management with laparoscopy and robotic assistance. Current surgical management is associated with fewer early and late complications, such as hepaticoenterostomy anastomotic leakage, cholangitis, anastomosis stricture, and cholangiocarcinoma. Specific features of CC management at Juntendo include: intraoperative endoscopy of the common channel and IHBD for inspecting and clearing debris to significantly reduce post-operative pancreatitis or stone formation; near infra-red fluorescence with indocyanine green for visualizing tissue planes especially during minimally invasive surgery for CC; and a classification system for CC based on PBMU that overcomes inconsistencies between existing classification systems and clinical presentation.

Management of complicated choledochal cyst in children: ultrasound-guided percutaneous external drainage and subsequent definitive operation.

OBJECTIVE: The purpose of this study was to analyze the outcomes of the combination of ultrasound (US)-guided percutaneous external drainage and subsequent definitive operation to manage complicated choledochal cyst in children. METHODS: This retrospective study included 6 children with choledochal cyst who underwent initial US-guided percutaneous external drainage and subsequent cyst excision with Roux-en-Y hepaticojejunostomy between January 2021 and September 2022. Patient characteristics, laboratory findings, imaging data, treatment details, and postoperative outcomes were evaluated. RESULTS: Mean age at presentation was 2.7 ± 2.2 (0.5-6.2) years, and 2 patients (2/6) were boys. Four patients (4/6) had a giant choledochal cyst with the widest diameter of ≥ 10 cm and underwent US-guided percutaneous biliary drainage on admission or after conservative treatments. The other 2 patients (2/6) underwent US-guided percutaneous transhepatic cholangio-drainage and percutaneous transhepatic gallbladder drainage due to coagulopathy, respectively. Five patients (5/6) recovered well after US-guided percutaneous external drainage and underwent the definitive operation, whereas 1 patient (1/6) had liver fibrosis confirmed by Fibroscan and ultimately underwent liver transplantation 2 months after external drainage. The mean time from US-guided percutaneous external drainage to the definitive operation was 12 ± 9 (3-21) days. The average length of hospital stay was 24 ± 9 (16-31) days. No related complications of US-guided percutaneous external drainage occurred during hospitalization. At 10.2 ± 6.8 (1.0-18.0) months follow-up, all patients had a normal liver function and US examination. CONCLUSIONS: Our detailed analysis of this small cohort suggests that US-guided percutaneous external drainage is technically feasible for choledochal cyst with giant cysts or coagulopathy in children, which may provide suitable conditions for subsequent definitive operation with a good prognosis. TRIAL REGISTRATION: Retrospectively registered.

Prenatal diagnosis at different gestational times and clinical features of choledochal cysts: a single tertiary center report.

PURPOSE: This study aimed to evaluate the effect of prenatal diagnosis at different gestational times on the clinical features of patients with choledochal cysts (CDCs). METHODS: Medical records of patients with prenatally diagnosed CDCs admitted to our hospital (April 2013-April 2018) were retrospectively reviewed. The clinical characteristics and pathological CDC features were analyzed. RESULTS: Two hundred eighteen cases were included. Patients were divided into two groups. Group 1 and group 2 had a prenatal diagnosis at ≤ 27 weeks of gestation (second trimester of gestation, n = 157) and > 27 weeks (third trimester of gestation, n = 61), respectively. The incidence of jaundice and the TBIL, IBIL and GGT levels were higher in Group 1 (P = 0.021, P = 0.029, P = 0.042, P = 0.007, respectively). The maximum cyst diameter at the time of surgery was larger in Group 1 (P = 0.015). An association study showed that the time of prenatal diagnosis was negatively correlated with the maximum cyst diameter both postnatally (r = - 0.223, P = 0.001) and at the time of surgery (r = - 0.268, P < 0.001). CONCLUSION: Unlike patients diagnosed at a late prenatal age, patients diagnosed at an early prenatal age tend to present clinical symptoms (jaundice, manifested as high indirect bilirubin), hepatic function damage, and large cysts at the time of surgery.

Cystic duct cyst in adults: a systematic review of the sixth entity.

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. The final analysis included 33 reported cases, with 55% of them reported in Asia. The mean age was 39.3 years old, with a female-to-male ratio of 1.5:1. Magnetic resonance cholangiopancreatography was accurate in establishing the diagnosis in 69% of cases. Where reported, standard laparoscopic/open cholecystectomy was performed in about 74% of patients, while around 25% of them needed extensive surgery. Associated malignancy was reported in 6.1% of cases, while 28% of patients had co-existent gallstone-related disease. No significant post-operative morbidity or mortality was reported. In this era of emergency laparoscopic cholecystectomy, surgeons should be aware of this rare condition, with the particular understanding that it is associated with gallstone-related disease in a significant number of reported cases.

Diagnosis and management of choledochal cysts.

BACKGROUND: Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs. METHODS: MEDLINE/PubMed and Web of Science databases were queried for "choledochal cyst", "bile duct cyst", "choledochocele", and "Caroli disease". Data were synthesized and systematically reviewed. RESULTS: Classified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3-7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%. CONCLUSION: Most CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation.

Laparoscopic bile duct plasty for hilar bile duct stenosis (HBDS) in patients with congenital biliary dilatation: Diagnosis of HBDS by preoperative MRCP and laparoscopic strategy to relieve HBDS.

BACKGROUND/PURPOSE: Congenital biliary dilatation (CBD) is a disease associated with pancreaticobiliary maljunction. The most frequent postoperative complication is intrahepatic stones, which are caused by hilar bile duct stenosis (HBDS). METHODS: We retrospectively reviewed the records of patients who underwent primary surgery for CBD between 2013 and 2021. We evaluated images and videos of HBDS, the laparoscopic technique of releasing the stenosis, and its occurrence rate and compared intraoperative bile duct findings with those of preoperative magnetic resonance cholangiopancreatography (MRCP). RESULTS: There were 87 CBD cases in this study. HBDS occurred in 52% and preoperative MRCP for HBDS showed a 96% sensitivity and 74% specificity in this study. Bile duct plasty was performed in 45% of the cases and videos demonstrated typical methods of laparoscopic bile duct plasty. The mid- to long-term complications were hepatolithiasis in three patients, anastomotic site stricture in three, and postoperative obstruction in two. CONCLUSIONS: Our study revealed that preoperative imaging studies are useful for the prediction of HBDS. Our laparoscopic meticulous probing method for finding BDS reveals more intrahepatic BDS through magnification. Therefore, this may reduce the incidence of intrahepatic stones. Preoperative imaging and intraoperative findings can be complemented to plan management that reduces long-term complications.