Biliary Cyst: An Unusual Cause of Cholestasis Post Cholecystectomy.

Biliary cysts are relatively uncommon and they can be congenital or acquired and can have various presentations such as cholelithiasis, cholangitis, jaundice, and pancreatitis. Biliary cysts are associated with a high risk of biliary cancers and such risk increases with age. Identification of biliary cysts warrants an aggressive approach to lower cancer risk. Surgical management has a high success rate and it lowers morbidity, mortality, and cancer risk. We present a 40-year-old female who had a cholecystectomy in 2016. She presented with obstructive jaundice and was found to have a class I biliary cyst. She underwent endoscopic retrograde cholangiopancreatography with stenting which led to complete resolution of her symptoms. Later, she underwent elective Roux-en-Y hepaticojejunostomy with cyst resection three months later. She underwent a successful recovery.

Biliary cysts in adults: Cerrahpasa experience.

Objectives: Biliary cysts are biliary duct dilatations, with 20% of the cysts being diagnosed in adulthood. Abdominal pain, jaundice and palpable abdominal mass are defined as the classical triad. However, nausea, vomiting, fever, itching and weight loss are frequent complaints. There are several treatment options depending on the type of the cyst. This study aimed to share our experience with biliary cysts and contribute to the literature on this subject. Material and Methods: Thirty patients, who received treatment for biliary cyst from January 1981 to December 2018 at our clinic, were studied retrospectively. The patients were analyzed based on age, sex, type of the cyst, diagnosis and treatment methods, post-op follow up and complications. Results: Twenty-seven of the patients were females, and three were males. The patients were aged between 16 and 76 years, and the median age was 41.9 years. All patients presented with abdominal pain, which was accompanied by cholangitis in nine patients, nausea and vomiting in four patients, dyspepsia in three patients and palpable mass in one patient. According to the Todani classification, biliary cyst findings were consistent with Type I in 23 patients, Type V in three patients, Type IV in two patients, Type II in one patient and Type III in one patient. Conclusion: Diagnosis and treatment are complex in biliary cysts due to anatomical proximity and variations. Therefore, it would be beneficial to refer them to referral centers. Choice of treatment should be based on the type of the cyst.

Case Report: A rare case of choledochal cyst.

Background: Choledochal cysts are dilated portions of the biliary tract that account for 1% of all benign biliary diseases. It is prevalent among Asian and female populations and the incidence is 1:100,000-150,000. Among the different types, only 15-35% of all choledochal cysts are type IV cysts, with type I being the most common representing 50-80%. Clinical presentation and therapy of biliary cysts (BC) differ depending on the type. Case: We present a case of a 2-year-old male who presented with non-specific symptoms of multiple episodes of vomiting. Laboratory investigations revealed raised alkaline phosphatase and gamma-glutamyl transpeptidase. His symptoms of acute pancreatitis were resolved with conventional therapy. Ultrasonography of the abdomen showed intra and extra-hepatic cystic biliary tree dilatation suggestive of choledochal cyst Type IV A. Conclusions: Choledochal cysts present with clinical features varying with age and anatomical variants and can pose challenges in management that can be addressed by surgery to avoid further complications.

Caroli Syndrome in a 6-Year-Old Rottweiler Dog.

A 6-year-old entire female rottweiler dog with a recent history of ascites and respiratory disease was submitted for necropsy examination. The dog had been diagnosed ultrasonographically with biliary cysts as a puppy. Grossly, the liver was smaller than expected with an irregular surface. Islands of hepatocytes were separated by bands of fibrosis and many bile ducts were markedly dilated. Histologically, extensive fibrosis extended beyond the limiting plate and into the surrounding hepatic parenchyma and was associated with abundant small bile ducts throughout. In conjunction with the detection of biliary cysts early in life, the gross and histological findings were consistent with a diagnosis of Caroli syndrome. In man, Caroli syndrome is frequently associated with renal and pancreatic cysts; a single renal cyst was identified in this case.

[A cough in a patient with cholangitis in the course of caroli's disease. Case report].

Cough may be a manifestation of pulmonary and extra-pulmonary diseases. However, rarely liver disease is suspected as a cause of acute cough. A case of a 34-year-old patient with diagnosed Caroli's disease has been described, in which cough with fever was the main manifestation of cholangitis in the course of complications of the underlying disease. Caroli's disease is a congenital pathology of intrahepatic bile ducts, causing their cysts to widen. It usually has an asymptomatic course, however it promotes infectious complications such as liver abscesses and recurrent cholangitis. The patient was admitted to the ward because of high fever and cough, without any symptoms from the digestive system. On the basis of laboratory, microbiological and visual examinations, various causes of cough were excluded and a diagnosis of cholangitis was made. After appropriate antibiotic therapy, a rapid improvement in the clinical condition and resolution of symptoms was achieved.

Outcomes associated with fetal hepatobiliary cysts: systematic review and meta-analysis.

OBJECTIVE: To explore the outcomes associated with fetal hepatobiliary cysts. METHODS: MEDLINE and EMBASE were searched for studies reporting on outcomes of fetal hepatobiliary cysts. Outcomes observed were resolution/reduction and increase in cyst size, associated congenital anomalies of the biliary tract and liver, abnormal postnatal liver function tests, clinical symptoms, need for surgery, postsurgical complications and predictive accuracy of prenatal ultrasound in identifying correctly hepatobiliary cysts. Meta-analysis of proportions was used to analyze the data. RESULTS: The search identified 1498 articles, and 22 studies (252 fetuses) were included in the systematic review. For fetal hepatic cysts, resolution or reduction in cyst size either pre- or postnatally occurred in 59.3% (95% CI, 30.9-84.7%) of cases, while an increase in cyst size occurred in 8.7% (95% CI, 1.1-22.4%). No case of hepatic cyst had associated malformations of the biliary tract at birth. Clinical symptoms occurred in 14.8% (95% CI, 6.3-26.1%) of cases and, in 5.4% (95% CI, 0.9-13.6%), they were related to the presence of bile obstruction due to compression of the cyst on the biliary tract. No case of hepatic cyst had abnormal liver function at birth. For fetal biliary cysts, resolution or reduction in cyst size occurred in 8.7% (95% CI, 2.7-17.5%) of cases and an increase in size occurred in 34.4% (95% CI, 20.5-49.8%). Congenital anomalies of the biliary tract and liver, such as fibrosis, occurred in 21.5% (95% CI, 10.2-35.6%) and 17.4% (95% CI, 5.4-34.4%) of cases, respectively. 57.3% (95% CI, 33.9-79.0%) of cases showed impairment in liver function after birth, while 55.0% (95% CI, 37.5-71.9%) showed clinical symptoms, mainly due to bile obstruction (47.9% (95% CI, 29.4-66.7%)). Postsurgical complications occurred in 10.9% (95% CI, 3.7-21.3%) of operated cases. Risk assessment according to different cut-offs of cyst size could not be performed in view of the very small number of included studies. CONCLUSIONS: Fetal hepatic cysts are benign, with a low likelihood of associated anomalies of the hepatobiliary tract, abnormal liver function or clinical symptoms. Congenital biliary cysts are associated with a high rate of progression, abnormal liver function after birth and clinical symptoms. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares / Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts

Las dilataciones quísticas de las vías biliares, conocidas como lagunas biliares, se han descrito en el seguimiento de los pacientes sometidos a portoenterostomía o cirugía de Kasai por atresia de las vías biliares (AVB). Su aparición se ha asociado con el desarrollo de colangitis y consecuentemente a un peor pronóstico. OBJETIVO: Describir las características clínicas y pronóstico de pacientes con AVB, sometidos a cirugía de Kasai, con énfasis en aquellos que desarrollaron lagunas biliares. PACIENTES Y MÉTODO: Estudio retrospectivo de pacientes sometidos a portoenterostomía de Kasai por AVB durante los años 2008 a 2016. Se tabuló la información demográfica y variables asociadas a la portoenterostomía de Kasai, trasplante hepático, lagunas o quistes biliares y episodios de colangitis. Se crearon curvas de Kaplan Meier y comparación mediante el test de Log Rank para evaluar sobrevida global, libre de colangitis y con hígado nativo, considerando un valor p como significativo. RESULTADOS: Se analizó el seguimiento de 13 pacientes. El tiempo promedio de realización del Kasai fue a los 85 meses (rango 42-193, DS 40,3), seis pacientes (46%) fueron sometidos a cirugía de Kasai después de los 90 días de vida por derivación tardía. Cuatro (31%) desarrollaron lagunas biliares múltiples, todos presentaron episodios de colangitis. La sobrevida libre de colangitis fue significativamente menor para los portadores de lagunas biliares. Nueve pacientes (69%) recibieron un trasplante hepático, en promedio a los 16 meses de edad (rango 6-40, DS 12,1), en 3 de ellos la causa fue colangitis recurrente. No se encontraron diferencias significativas en la sobrevida con hígado nativo ni en sobrevida global entre portadores y no portadores de lagunas biliares. CONCLUSIONES: En esta cohorte, la incidencia de lagunas biliares luego de la portoenterostomía es similar a la descrita en la literatura. Los resultados concuerdan con la relación propuesta entre ellas y el desarrollo de colangitis. En los pacientes de nuestra serie, el diagnóstico y derivación de AVB fue realizado tardíamente, determinando un peor pronóstico.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.

Cholestasis beyond the Neonatal and Infancy Periods.

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.

Cholestasis beyond the Neonatal and Infancy Periods / 대한소아소화기영양학회지

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.

Biliary cysts: a review and simplified classification scheme.

Biliary cystic disease has been known of for centuries. It has traditionally been classified as 5 major types of disease, each with different clinical profiles and attributes. In this article, the basis for the existing classification schemes is reviewed and a simplified classification scheme and treatment regimen are suggested.

Biliary cysts: etiology, diagnosis and management.

Biliary cysts (BC) are rare dilatations of different parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. BC occur the most frequently in Asian and female populations. They are an important problem for pediatricians, gastroenterologists, radiologists and surgeons. Clinical presentation and management depend on the BC type. Cholangiocarcinoma is the most serious and dangerous BC complication. The other complications associated with BC involve cholelithiasis and hepatolithiasis, cholangitis, acute and chronic pancreatitis, portal hypertension, liver fibrosis and secondary liver cirrhosis and spontaneous cyst perforation. Different BC classifications have been described in the literature. Todani classification dividing BC into five types is the most useful in clinical practice. The early diagnosis and proper treatment are very important, because BC are associated with a risk of carcinogenesis. A malignancy risk increases with the age. Radiological investigations (ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography) play an important role in BC diagnostics. Currently, prenatal diagnosis using ultrasonography is possible. It allows to differentiate biliary disorders in fetals and to perform the early surgical treatment that improves results. In most patients, total cyst excision with Roux-Y hepaticojejunostomy is the treatment of choice. Surgical treatment of BC is associated with high success rate and low morbidity and mortality. The early treatment is associated with a lower number of complications. Patients following BC surgery require permanent and careful postoperative observation using laboratory and imaging investigations because of possibility of biliary anastomosis stricture and biliary cancer in tissue remnant.