Hemobilia como forma inusual de presentación en la enfermedad de Caroli / Hemobile as an unusual presentation form of the Caroli´s disease

La enfermedad de Caroli, o ectasia comunicante de las vías biliares intrahepáticas, es una entidad congénita, caracterizada por múltiples dilataciones saculares o quísticas de las vías biliares intrahepáticas. En este trabajo se hace presentación de un nuevo caso portador de enfermedad de Caroli. Se trata de una paciente de raza blanca, de 26 años de edad, con antecedentes de buena salud anterior, que ingresa por fiebre elevada de 39 ºC, hepatomegalia dolorosa e imágenes hipoecoicas en el hígado, detectadas en la ultrasonografia, con aminotransferasas hepáticas ligeramente elevadas. En el segundo ingreso sufre complicaciones febriles y de sangramiento digestivo alto por hemobilia importante, que compromete la hemodinamia y junto a la sepsis dan al traste con su vida. La paciente ingresa por crisis de colangitis y presentó un hemobilia importante que la llevó a complicaciones hemodinámicas, colangitis ascendente, y finalmente a sepsis generalizada como causa de muerte(AU)

Caroli´s disease, or communicant ectasia of the intrahepatic biliary ducts, is a congenital entity, characterized by multiple saccular or cystic dilatations of the intrahepatic biliary ducts. In this work we present a new case of a carrier of Carolis disease. It deals with a female patient, aged 26 years, with antecedents of good health status, who entered the hospital with high fever of 39oC, painful hepatomegaly and hyper echoic images in the liver, detected in the ultrasonography, with slightly raised hepatic aminotranferases. During the second staying in the hospital she suffered fever complications and high digestive bleeding caused by important hemobile that compromised the hemodynamics and together with sepsis caused her to die. The patient entered the hospital because of cholangitis and presented an important hemobile leading her to hemodynamic complications, ascendant cholangitis, and finally a generalized sepsis as death cause(AU)

Hemobilia como forma inusual de presentación en la enfermedad de Caroli / Hemobile as an unusual presentation form of the Caroli´s disease

La enfermedad de Caroli, o ectasia comunicante de las vías biliares intrahepáticas, es una entidad congénita, caracterizada por múltiples dilataciones saculares o quísticas de las vías biliares intrahepáticas. En este trabajo se hace presentación de un nuevo caso portador de enfermedad de Caroli. Se trata de una paciente de raza blanca, de 26 años de edad, con antecedentes de buena salud anterior, que ingresa por fiebre elevada de 39 ºC, hepatomegalia dolorosa e imágenes hipoecoicas en el hígado, detectadas en la ultrasonografia, con aminotransferasas hepáticas ligeramente elevadas. En el segundo ingreso sufre complicaciones febriles y de sangramiento digestivo alto por hemobilia importante, que compromete la hemodinamia y junto a la sepsis dan al traste con su vida. La paciente ingresa por crisis de colangitis y presentó un hemobilia importante que la llevó a complicaciones hemodinámicas, colangitis ascendente, y finalmente a sepsis generalizada como causa de muerte.

Caroli´s disease, or communicant ectasia of the intrahepatic biliary ducts, is a congenital entity, characterized by multiple saccular or cystic dilatations of the intrahepatic biliary ducts. In this work we present a new case of a carrier of Caroli’s disease. It deals with a female patient, aged 26 years, with antecedents of good health status, who entered the hospital with high fever of 39oC, painful hepatomegaly and hyper echoic images in the liver, detected in the ultrasonography, with slightly raised hepatic aminotranferases. During the second staying in the hospital she suffered fever complications and high digestive bleeding caused by important hemobile that compromised the hemodynamics and together with sepsis caused her to die. The patient entered the hospital because of cholangitis and presented an important hemobile leading her to hemodynamic complications, ascendant cholangitis, and finally a generalized sepsis as death cause.

Caroli disease patients have excellent survival after liver transplant.

BACKGROUND: Caroli disease (CD) is characterized by dilation of the intrahepatic biliary tree, which may result in malignancy. Treatments include management of symptoms and hepatic resection to decrease disease burden. In patients with CD not amenable to these treatments, orthotopic liver transplantation (OLT) has been used. This study examines if OLT is a reasonable treatment for patients with CD. MATERIALS AND METHODS: The United Network of Organ Sharing/Organ Procurement and Transplantation Network database between September 30, 1987 and March 31, 2011 was queried. Cases without patient or allograft survival time or without a diagnosis were excluded from analysis. Patients with CD were compared to patients with primary biliary cirrhosis (PBC), secondary biliary cirrhosis (BC), primary sclerosing cholangitis (PSC), and all indications for OLT. Survival analysis was performed by log-rank test and Kaplan-Meier. RESULTS: One hundred forty patients with CD were compared to 4797 patients with PBC, 489 patients with secondary BC, 6033 patients with PSC, and 92,210 patients post-OLT. Patient and allograft survivals of CD patients at 1, 3, 5, and 10 y are, respectively, 88.5%, 83.4%, 80.9%, and 77.8%; and 81.2%, 74.8%, 70.6%, and 67.9%. CD patients have significantly improved patient and allograft survivals after OLT compared to patients with secondary BC (P = 0.003, P = 0.015) and all other patients undergoing OLT (P = 0.003, P = 0.026). There is a trend towards long-term improved patient and allograft survival in transplanted patients with CD compared to patients with PBC and PSC. CONCLUSIONS: These results suggest that OLT should be considered an effective treatment modality for patients with CD resulting in excellent long-term outcomes.

Kidney and liver transplantation in patients with autosomal recessive polycystic kidney disease: a multicentric study.

BACKGROUND AND OBJECTIVES: In contrast to the improvement in our understanding of the pathogenesis and presentation of autosomal recessive polycystic kidney disease (ARPKD), data regarding the issue of kidney and liver transplantation in patients with ARPKD remain particularly scarce. Here, we report the results and outcome of renal and/or liver transplantation in a series of patients with ARPKD. METHODS: Fourteen ARPKD patients (age: 3-25 years) who underwent renal transplantation with or without liver transplantation were retrospectively identified in five French nephrology departments. The patients' medical charts were reviewed and relevant data were collected. RESULTS: The clinical and radiological presentation of the 14 patients was highly variable illustrating the heterogeneity of ARPKD. Six patients underwent kidney and/or liver transplantation in adulthood. First renal graft survival was 92, 78 and 14% at 1, 5 and 10 years after renal transplantation, respectively. Mortality rate was relatively high (3/14; 21%) in these young patients and was directly related to infectious complications (recurrent angiocholitis) of severe Caroli's disease (dilatation of intra- and/or extra-hepatic bile ducts), a typical feature of ARPKD. CONCLUSIONS: Our data suggest that ARPKD patients evaluated for renal transplantation should be carefully screened for severe Caroli's disease. Even though the limited number of patients included in our study precludes any definite recommendation, pre-emptive liver transplantation may be a therapeutic option in ARPKD patients with severe Caroli's disease evaluated for renal transplantation.

Caroli's disease: report of surgical options and long-term outcome of patients treated in Argentina. Multicenter study.

BACKGROUND: Caroli's disease (CD) management is still controversial. AIM: The purpose of this study is to report the most frequent clinical features, treatment options, and outcome obtained after surgical management of CD. METHODS: A voluntary survey was conducted. Demographic, clinical, surgical, and pathological variables were analyzed. RESULTS: Six centers included 24 patients having received surgical treatment from 1991 to 2009. Seventeen (70.8%) patients were female, with average age of 48.7 years old (20-71), and 95.5% were symptomatic. There was left hemiliver involvement in 75% of the patients. Surgical procedures included nine left lateral sectionectomies, eight left hepatectomies, and four right hepatectomies for those with hemiliver disease, while for patients with bilateral disease, one right hepatectomy and two Roux-en-Y hepaticojejunostomies were performed. The average length of hospitalization was 7 days. For perioperative complications (25%), three patients presented minor complications (types 1-2), while major complications occurred in three patients (type 3a). No mortality was reported. After a median follow-up of 166 months, all patients are alive and free of symptoms. CD diagnosis was confirmed by histology. Congenital hepatic fibrosis was present in two patients (8.3%) and cholangiocarcinoma in one (4.2%). CONCLUSIONS: CD in Argentina is more common in females with left hemiliver involvement. Surgical resection is the best curative option in unilateral disease, providing long-term survival free of symptoms and complications. In selected cases of bilateral disease without parenchymal involvement, hepaticojejunostomy should be proposed. However, a close follow-up is mandatory because patients might progress and a transplant should be indicated.

Long-term outcome of liver resection and transplantation for Caroli disease and syndrome.

OBJECTIVE: To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS). SUMMARY BACKGROUND DATA: The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined. PATIENTS AND METHODS: Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis. RESULTS: Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement. CONCLUSION: Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.

The role of surgery in Caroli's disease.

BACKGROUND: Caroli's disease is a rare congenital disorder characterized by multifocal segmental dilation of the intrahepatic bile ducts. Whether conservative or surgical strategies should be preferred is still a matter of debate. The aim of this study was to evaluate the role of surgery in the management of Caroli's disease. STUDY DESIGN: From April 1998 until August 2005, 12 consecutive patients with Caroli's disease were treated in the Department of General, Visceral, and Transplantation Surgery, University Hospital Essen, Germany. All patients were intended to receive liver resections or liver transplantations. RESULTS: There were seven men and five women, with a median age of 39 years (range 7 months to 70 years). Eight patients had monolobar and four patients had bilobar liver involvement. All patients had a history of recurrent cholangitis, with up to 16 unsuccessful conservative treatment attempts. Nine patients (75%) underwent liver resection and two (17%) had liver transplantation. Intraoperatively, three patients (25%) were found to have cholangiocarcinoma, of which one was unresectable. There was no mortality and only low morbidity (16%) postoperatively. After a median followup of 31 months, 11 patients are well with no recurrent symptoms. CONCLUSIONS: Surgery can offer a definite therapy, with an acceptable morbidity and virtually no mortality in localized Caroli's disease. In diffuse disease, the use of extended resections or liver transplantation can provide good longterm results.

Caroli's disease: liver resection and liver transplantation. Experience in 33 patients.

BACKGROUND: The aim of this study was to review and discuss our observations on 33 patients who underwent surgical treatment for Caroli's disease (CD), focusing on diagnosis, current surgical management, and long-term outcome. METHODS: Between May 1993 and June 2004, 642 liver resections and 286 liver transplantations in 252 patients were performed in our department of surgery. Thirty-three patients were referred to our center for diagnostic and therapeutic management of CD. Prior surgical interventions for hepatobiliary disorders, current diagnostic and surgical procedures, procedure-specific complications, duration of hospital stay, duration of follow-up, outpatient information, and long-term outcome were reviewed. RESULTS: Fifteen male and 18 female patients were treated in this study. Initial symptoms and signs of the disease noted in our patients included right upper quadrant pain, fever, and jaundice. In 2 of the 33 patients, we noted clinical evidence of cirrhosis followed by histologic confirmation. One patient suffered from variceal bleeding. In 26 patients, diagnoses were established by a combined endoscopic retrograde cholangiopancreatography, ultrasonography, and computed tomographic studies. The disease was localized in 25 and diffuse in 8 patients. Liver resection was carried out in 29 patients. Partial hepatectomies were performed in 27 of these 29 at our institution. Two female patients with the diffuse disease underwent orthotopic liver transplantation. Thirteen of the 31 patients who underwent surgery at our institution had an uneventful postoperative course. Fourteen patients had minor postoperative complications and responded well to medical management. Four patients had major complications that required further surgical treatment. Two patients died of complications related to postoperative hemorrhage and sepsis. Two patients with intrahepatic cholangiocarcinoma died because of primary tumor progress. One patient with cholangiocarcinoma died 1 year after a successful left hepatectomy because of metastatic disease recurrence. The long-term results of the 26 surviving patients were assessed during a mean follow-up of 3.7 years (range, 1-11 years). All 26 patients remained free of biliary symptoms or complications. In 25 patients, surgery including liver transplantation was curative. CONCLUSIONS: Partial hepatectomy for localized CD is potentially curative. In patients with diffuse CD, liver transplantation provides gratifying long-term results.

Caroli's disease: 1977-1995 experiences.

OBJECTIVE: Until recently, congenital cystic dilatation of the intrahepatic biliary ducts (Caroli's disease) has been recognized infrequently. This report aimed to analyse and discuss our observations on 21 adult patients with the disease. PATIENTS: From 1977 to 1995, 21 patients (eight women and 13 men), aged 17 to 68 years, were diagnosed and treated for Caroli's disease at Yuksek Ihtisas Hospital. The mean duration of the disease was 6.5 years. RESULTS: The commonest presenting symptom was abdominal pain, a feature in 18 cases. The distribution of the biliary lesions was bilobar in 12 patients and monolobar in nine. Two of them were congenital hepatic fibrosis. Twenty-one patients had coexisting hepatobiliary disease, associated with Caroli's disease. Approximately 95% of our patients had cystolithiasis, cholelithiasis or both. Surgical treatment was used in 18 patients, a partial hepatectomy being carried out in seven of them, an internal biliary drainage by choledocystojejunostomy in five cases, and by choledochotomy in four cases. Three cases with Caroli's disease were treated with endoscopic sphincterotomy and stone extraction. In the follow-up period, four of our patients died. CONCLUSION: Caroli's disease is being diagnosed more frequently as a result of improved diagnostic capabilities. The aim of the treatment is to obtain sufficient biliary drainage and to relieve the symptoms.