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1.
Biosci Trends ; 12(4): 426-431, 2018 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-30146617

RESUMO

Congenital intrahepatic bile duct dilatation (Caroli's disease) is a rare biliary disease. Although multiple reports exist describing its surgical treatment, relatively few have provided long-term follow-up. Prospective data about 25 cases of monolobular Caroli's disease, with liver resection between 1974 and 2016, were retrospectively analyzed. Patient demographics together with postoperative outcomes and long-term follow-up were assessed. Our 25-patient cohort (average age 53.4 years (range: 27-82)) included 20 cases with disease limited to the left lobe, and 5 to the right. The average time interval between first symptoms and final diagnosis was 5 years (range: 0-34 years). The surgical procedures included left lobectomy in 11 cases, left hepatectomy in 8 cases, right hepatectomy in 3, and sub-segmentectomy in 3 cases. Biliodigestive anastomosis was performed in 7 cases. Complications were observed in 3 patients (25%). Metachronous cholangiocarcinoma was observed in one single case, 10 years after initial operation. In conclusion, surgical treatment for monolobular Caroli's disease is effective, with good short-term results and few complications. Median long-term follow-up was 18 months (range: 3-132), with favorable clinical evolution in 96% of patients.


Assuntos
Doença de Caroli/cirurgia , Fígado/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
2.
Internist (Berl) ; 59(3): 276-281, 2018 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-28939930

RESUMO

A 44-year-old Filipino woman presented with abdominal pain and fever. Clinical examination and blood tests revealed no pathological results; however, (cross-sectional) imaging showed saccular cystic bile duct dilatation in the right liver with solid intraductal masses. Due to the clinical presentation the patient was admitted for surgical intervention with the diagnosis of Caroli disease. During the surgical procedure histopathology showed an intraductal papillary neoplasm of the bile duct (IPNB). The planned segmentetomy was extended to hemihepatectomy. IPNB is a rare entity of premalignant lesions of the bile duct system first recognized by the World Health Organization in 2010.


Assuntos
Dor Abdominal/etiologia , Neoplasias dos Ductos Biliares/diagnóstico , Ductos Biliares Intra-Hepáticos , Carcinoma Papilar/diagnóstico , Doença de Caroli/diagnóstico , Febre de Causa Desconhecida/etiologia , Dor Abdominal/patologia , Adulto , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos/patologia , Ductos Biliares Intra-Hepáticos/cirurgia , Carcinoma Papilar/patologia , Carcinoma Papilar/cirurgia , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Diagnóstico Diferencial , Feminino , Hepatectomia , Humanos
3.
Transpl Infect Dis ; 19(6)2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28921746

RESUMO

This case describes a patient being considered for combined liver-kidney transplantation for Caroli's disease with a failed renal transplant. A chronic septic focus could not be located with standard imaging techniques, such as ultrasonography and computed tomography. This case report highlights the observation that a retained non-functioning transplant can be the cause of fever of unknown origin and PET-CT can be useful in diagnosing these challenging cases.


Assuntos
Aloenxertos/diagnóstico por imagem , Doença de Caroli/cirurgia , Febre de Causa Desconhecida/diagnóstico por imagem , Rim/diagnóstico por imagem , Transplante de Fígado/métodos , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Adulto , Aloenxertos/microbiologia , Aloenxertos/patologia , Aloenxertos/cirurgia , Febre de Causa Desconhecida/microbiologia , Febre de Causa Desconhecida/patologia , Febre de Causa Desconhecida/cirurgia , Rejeição de Enxerto/microbiologia , Humanos , Rim/microbiologia , Rim/patologia , Rim/cirurgia , Transplante de Rim/efeitos adversos , Transplante de Rim/métodos , Cirrose Hepática/congênito , Cirrose Hepática/cirurgia , Masculino , Necrose , Nefrectomia , Doenças Renais Policísticas/cirurgia , Cuidados Pré-Operatórios/métodos , Transplantados , Falha de Tratamento , Ultrassonografia
6.
Clin Transplant ; 30(1): 3-9, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26385435

RESUMO

Liver transplantation (LT) represents an uncommon indication for Caroli's disease (CD) or syndrome (CS). Excellent results of LT have been reported as shown by recent multicentric European and American registry reports. Clear therapeutic flowcharts to adopt in these diseases are still lacking. This review aims at analyzing highlighting recent transplant experiences in this field and also at focusing on the role of LT in case-specific comorbidities such as development of cholangiocellular cancer or renal failure are present.


Assuntos
Algoritmos , Doença de Caroli/cirurgia , Transplante de Fígado , Humanos , Síndrome
7.
J Gastrointest Surg ; 19(11): 2019-27, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26302876

RESUMO

BACKGROUND: Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS: With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months). CONCLUSIONS: Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndrome patients due to the associated hepatic fibrosis.


Assuntos
Doença de Caroli/cirurgia , Doenças Genéticas Inatas/cirurgia , Cirrose Hepática/cirurgia , Transplante de Fígado , Adolescente , Adulto , Doença de Caroli/complicações , Doença de Caroli/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/diagnóstico , Humanos , Lactente , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico , Masculino , Pessoa de Meia-Idade , Adulto Jovem
10.
Ann Surg ; 258(5): 713-21; discussion 721, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24121258

RESUMO

OBJECTIVE: To assess clinical presentation and long-term results of surgical management of congenital intrahepatic bile duct dilatation (IHBDD) (Caroli disease and syndrome) in a multicenter setting. BACKGROUND: Congenital IHBDD predisposes to biliary stasis, resulting in intrahepatic lithiasis, septic complications, and cholangiocarcinoma. Although liver resection (LR) is considered to be the treatment of choice for unilobar disease extent into the liver, the management of bilobar disease and/or associated congenital hepatic fibrosis remains challenging. METHODS: From 1978 to 2011, a total of 155 patients (median age: 55.7 years) were enrolled from 26 centers. Bilobar disease, Caroli syndrome, liver atrophy, and intrahepatic stones were encountered in 31.0%, 19.4%, 27.7%, and 48.4% of patients, respectively. A complete resection of congenital intrahepatic bile ducts was achieved in 90.5% of the 148 patients who underwent surgery. RESULTS: Postoperative mortality was nil after anatomical LR (n = 111) and 10.7% after liver transplantation (LT) (n = 28). Grade 3 or higher postoperative morbidity occurred in 15.3% of patients after LR and 39.3% after LT. After a median follow-up of 35 months, the 5-year overall survival rate was 88.5% (88.7% after LT), and the Mayo Clinic score was considered as excellent or good in 86.0% of patients. The 1-year survival rate was 33.3% for the 8 patients (5.2%) who presented with coexistent cholangiocarcinoma. CONCLUSIONS: LR for unilobar and LT for diffuse bilobar congenital IHBDD complicated with cholangitis and/or portal hypertension achieved excellent long-term patient outcomes and survival. Because of the bad prognosis of cholangiocarcinoma and the sizeable morbidity-mortality after LT, timely indication for surgical treatment is of major importance.


Assuntos
Ductos Biliares Intra-Hepáticos/anormalidades , Doença de Caroli/cirurgia , Neoplasias dos Ductos Biliares/complicações , Neoplasias dos Ductos Biliares/cirurgia , Feminino , França , Hepatectomia/métodos , Humanos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/cirurgia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias , Taxa de Sobrevida , Resultado do Tratamento
11.
J Pediatr Gastroenterol Nutr ; 57(2): 161-6, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23518487

RESUMO

BACKGROUND AND OBJECTIVE: Congenital hepatic fibrosis (CHF) and Caroli syndrome are frequently associated with renal cystic diseases. They have a variable clinical course, and the natural history is not well defined despite molecular advances. Our study describes the clinical manifestations and long-term outcome in children with this disorder. METHODS: A retrospective case review of children with CHF at a single centre diagnosed on the basis of clinical features, radiological and endoscopic evidence of portal hypertension (PHT), and compatible histopathological findings. Children were categorised based on hepatic phenotype-group 1 (Caroli syndrome) and group 2 (CHF). Hepatobiliary as well as renal manifestations were recorded at presentation, and their evolution followed up until transplant or last follow-up. RESULTS: There were 40 children (22 boys) with a median age of 1.3 years at clinical presentation. Fourteen of 40 (35%) children presented in the neonatal period with primarily renal disease, of whom 11 (78%) had Caroli syndrome (P = 0.02). Significant PHT with oesophageal varices was seen in 86%, with no difference in the incidence of gastrointestinal bleeding and varices between Caroli syndrome and CHF. Cholangitis developed in 10 of 40 (25%) and was more common in the Caroli syndrome group (P = 0.009). A higher proportion of children with Caroli syndrome developed chronic kidney disease (CKD) stage 3 and above as compared with CHF (85% vs 42%; P = 0.007). Twelve of 21 (57%) and 8 of 19 (42%) children in the Caroli syndrome and CHF groups required either combined liver-kidney or isolated liver transplant, with the most common indication for renal transplantation being end-stage renal disease (CKD5d) with or without advanced PHT or cholangitis. All 14 (100%) children with neonatal presentation developed CKD5d and required combined liver-kidney transplant before 14 years of age, whereas 77% of children presenting beyond the neonatal period survived without liver-kidney transplant (P < 0.001). Neonatal presentation was the best predictor of the need for transplant. CONCLUSIONS: Caroli syndrome is more likely to present in the neonatal period and these patients are more likely to develop CKD5d. CKD stage 3 or above with recurrent cholangitis is more common in Caroli syndrome presenting beyond the neonatal period and adds to the significant morbidity in these patients. Children presenting in the neonatal period have a more severe phenotype and should be considered early for combined liver-kidney transplant.


Assuntos
Doença de Caroli , Doenças Genéticas Inatas , Hipertensão Portal/etiologia , Falência Renal Crônica/etiologia , Rim/patologia , Cirrose Hepática , Fígado/patologia , Rim Policístico Autossômico Recessivo , Adolescente , Doença de Caroli/complicações , Doença de Caroli/epidemiologia , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Criança , Pré-Escolar , Colangite/epidemiologia , Colangite/etiologia , Colangite/genética , Varizes Esofágicas e Gástricas/epidemiologia , Varizes Esofágicas e Gástricas/etiologia , Varizes Esofágicas e Gástricas/genética , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/epidemiologia , Doenças Genéticas Inatas/patologia , Doenças Genéticas Inatas/cirurgia , Humanos , Hipertensão Portal/epidemiologia , Hipertensão Portal/genética , Lactente , Recém-Nascido , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/genética , Transplante de Rim , Cirrose Hepática/complicações , Cirrose Hepática/epidemiologia , Cirrose Hepática/patologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Masculino , Fenótipo , Rim Policístico Autossômico Recessivo/complicações , Rim Policístico Autossômico Recessivo/epidemiologia , Rim Policístico Autossômico Recessivo/patologia , Rim Policístico Autossômico Recessivo/cirurgia , Prevalência , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Estudos Retrospectivos
12.
Pol Przegl Chir ; 85(11): 663-5, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24413206

RESUMO

The study introduces a case of a 51-year old patient with Caroli's disease of left liver lobe. In 2011 the patient was admitted to Clinic of General, Vascular and Transplantation Surgery. She was after first in her life incident of an acute pancreatitis and subsequent ERCP procedure with left hepatic biliary tract drainage. The lady was qualified to left-hemihepatoctomy, which was successfully conducted in our clinic. The only complication of the procedure was surgical site infection which was properly treated with typical antibiotics. One-year observation occurred no other complications and liver function was fine.


Assuntos
Doença de Caroli/diagnóstico , Doença de Caroli/cirurgia , Hepatectomia , Feminino , Humanos , Fígado/diagnóstico por imagem , Fígado/cirurgia , Pessoa de Meia-Idade , Radiografia , Resultado do Tratamento
13.
Klin Med (Mosk) ; 91(10): 57-60, 2013.
Artigo em Russo | MEDLINE | ID: mdl-25696953

RESUMO

Caroli's disease is a rare congenital condition characterized by segmental non-obstructive fibrocystic dilation of intrahepatic bile ducts. Dilated ducts may be infected and contain stones. This review of the modern literature describes few known cases of the disease, analyses its clinical features and results of treatment. The principal diagnostic methods include visualization by ultrasound study, CT, MRT, retrograde and transhepatic cholangiography. Surgical intervention is the method of choice for the treatment of the disease including resection of liver, placement of hepatico-jejunal anastomoses and transplantation. The choice depends on the extent of the lesion and anticipated complications. The authors' observation of the patient with Caroli's disease in a 35 year-old woman is presented. It was successfully treated by left-hand bisegmentectomy even though the disease was diagnosed 14 years after the first symptoms. It is recommended that the disease, even if a rare one, should be included in the algorithm of differential diagnostics ofrecurrent abdominal pain with manifestations of cholestasis or fever of an unknown origin.


Assuntos
Doença de Caroli/diagnóstico , Adulto , Doença de Caroli/cirurgia , Feminino , Humanos
14.
Tunis Med ; 90(8-9): 630-5, 2012.
Artigo em Francês | MEDLINE | ID: mdl-22987378

RESUMO

BACKGROUND: Caroli's disease is a congenital dilatation of the intrahepatic biliary duct. AIM: To analyse and discuss diagnostic and therapeutics difficulties through 16 patients with Caroli's disease. METHODS: Between January 1990 and September 2010, 16 patients underwent surgical procedure for Caroli's disease. Data recorded for each patient included clinical symptoms, biologic findings, previous biliary procedures, and the presenting symptoms. The distribution of the biliary lesions, the surgical procedure and the postoperative outcomes and follow up were detailed. RESULTS: The mean age was 55 years. The mean interval between the first symptoms and diagnosis was 27 months. Five of 16 patients had undergone 12 surgical or endoscopic procedures prior to liver resection. Before the definitive diagnosis, 9 patients presented 15 episodes of acute cholangitis. The diagnosis was established preoperatively in 13 cases, 5 among them underwent previous biliary surgical procedures. The diagnosis was documented peroperatively in 2 cases and postoperatively in 1 case. The distribution of the biliary lesions was monolobar in 13 and bilobar in 3 patients. 13 patients underwent liver resection, in two cases we perfomed biliojejunostomy and the last one had endoscopic sphincterotomy. There was no mortality and the overall postoperative morbidity is about 43%. The follow-up shows that 12 patients still alive with a mean follow up for 53 months, from whom only one patient have intrahépepatic lithiasis. CONCLUSION: The clinical course of Caroli's disease is often complicated by recurrent episodes of angiocholitis and requires iterative surgery. The hepatectomy witch prevented septic complications and degenerescence is possible only in a restricted number of patients.


Assuntos
Doença de Caroli , Adulto , Idoso , Doença de Caroli/diagnóstico , Doença de Caroli/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
15.
J Surg Res ; 177(2): 365-72, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22640887

RESUMO

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.


Assuntos
Doença de Caroli/cirurgia , Transplante de Fígado , Adolescente , Adulto , Doença de Caroli/mortalidade , Criança , Pré-Escolar , Colestase Intra-Hepática/mortalidade , Colestase Intra-Hepática/cirurgia , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estados Unidos/epidemiologia
16.
Pediatr Transplant ; 16(2): 195-200, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22360404

RESUMO

Fibropolycystic liver disease includes CHF, Caroli's syndrome, and Caroli's disease. Patients with Caroli's disease and Caroli's syndrome have an increased risk of recurrent cholangitis, intrahepatic calculi, biliary cirrhosis, and cholangiocarcinoma. The aim of this study was to examine the post-transplantation outcomes of children with fibropolycystic liver disease. Of the 158 children transplanted at Seoul National University Hospital, there were four patients with Caroli's syndrome, two patients with CHF, and one patient with Caroli's disease. One patient underwent combined liver/kidney transplantation. Associated renal manifestations included ARPKD in three children and nephronophthisis in one child. The indications for LT were recurrent cholangitis, decompensated cirrhosis, and refractory complications of portal hypertension. Both graft and patient survival rates were 100% at a median follow-up period of two yr after LT. Three children with growth failure achieved catch-up growth after LT. In three patients with ARPKD, mean serum creatinine levels increased from 0.53 mg/dL at the time of LT to 0.91 mg/dL at the last follow-up (p = 0.01). LT is an excellent option for children with complications from fibropolycystic liver disease. Renal function should be monitored cautiously after LT in the patients with ARPKD.


Assuntos
Doença de Caroli/cirurgia , Cirrose Hepática/cirurgia , Transplante de Fígado , Doença de Caroli/complicações , Criança , Pré-Escolar , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Lactente , Doenças Renais Císticas/complicações , Doenças Renais Císticas/cirurgia , Transplante de Rim , Cirrose Hepática/complicações , Cirrose Hepática/congênito , Masculino , Rim Policístico Autossômico Recessivo/complicações , Rim Policístico Autossômico Recessivo/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
17.
Nephrol Dial Transplant ; 27(5): 2083-8, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22076432

RESUMO

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.


Assuntos
Doença de Caroli/cirurgia , Transplante de Rim , Transplante de Fígado , Rim Policístico Autossômico Recessivo/cirurgia , Adolescente , Adulto , Doença de Caroli/etiologia , Doença de Caroli/mortalidade , Criança , Pré-Escolar , Estudos de Coortes , Gerenciamento Clínico , Feminino , França , Humanos , Estudos Longitudinais , Masculino , Rim Policístico Autossômico Recessivo/complicações , Rim Policístico Autossômico Recessivo/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
18.
J Pediatr Surg ; 46(8): 1638-41, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21843735

RESUMO

Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.


Assuntos
Doença de Caroli/cirurgia , Transplante de Fígado , Doença de Caroli/diagnóstico , Criança , Feminino , Humanos
19.
Rozhl Chir ; 90(5): 281-4, 2011 May.
Artigo em Tcheco | MEDLINE | ID: mdl-21838130

RESUMO

Caroli disease is a rare congenital condition characterized by a non-obstructive saccular or fusiform multi-focal segmental dilatation of the intrahepatic bile ducts and the frequent formation of the intrahepatic calculi. It can affect the entire liver with manifestations in the childhood, or only some segments, which may be an asymptomatic condition found accidentally in the adulthood. In other cases, the condition is manifested primarily with tract infections. The authors of the three case reports describe pitafalls of the diagnosis and treatment of the segmental Caroli disease, which is manifested in the adulthood. The treatment was a resection of the affected liver segments.


Assuntos
Doença de Caroli/diagnóstico , Adulto , Idoso , Ductos Biliares Intra-Hepáticos/diagnóstico por imagem , Ductos Biliares Intra-Hepáticos/patologia , Doença de Caroli/diagnóstico por imagem , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Feminino , Humanos , Tomografia Computadorizada por Raios X
20.
J Gastrointest Surg ; 15(10): 1814-9, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21796462

RESUMO

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.


Assuntos
Doença de Caroli/cirurgia , Adulto , Idoso , Argentina , Doença de Caroli/mortalidade , Doença de Caroli/patologia , Feminino , Hepatectomia , Humanos , Jejunostomia , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
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