ABSTRACT
ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.
RESUMO Relato de caso de uma paciente adolescente de 16 anos de idade com diagnóstico prévio de doença renal policística autossômica recessiva (DRPAR), que apresentou quadro agudo de pneumonia bilateral e hemorragia digestiva alta por ruptura de varizes esofágicas, bem como ascite e edema de membros inferiores. Necessitou de estabilização clínica intensiva e tratamento endoscópico do sangramento digestivo. Após investigação dos diagnósticos diferenciais da hepatopatia crônica, diagnosticou-se shunt esplenorrenal espontâneo, e realizou-se biópsia hepática guiada por ecografia com diagnóstico de cirrose, espectro típico da DRPAR. Recebeu alta hospitalar assintomática e foi encaminhada para avaliação de transplante duplo.
Subject(s)
Humans , Female , Adolescent , Arteriovenous Anastomosis/pathology , Polycystic Kidney, Autosomal Recessive/complications , Caroli Disease/complications , Liver Cirrhosis/complications , Arteriovenous Anastomosis/diagnostic imaging , Referral and Consultation , Renal Veins/diagnostic imaging , Biopsy , Brazil , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Intensive Care Units, Pediatric , Treatment Outcome , Polycystic Kidney, Autosomal Recessive/drug therapy , Polycystic Kidney, Autosomal Recessive/diagnostic imaging , Caroli Disease/pathology , Caroli Disease/drug therapy , Magnetic Resonance Angiography , Adrenergic beta-Agonists/therapeutic use , Diuretics, Potassium Sparing/therapeutic use , Liver Cirrhosis/pathology , Liver Cirrhosis/drug therapyABSTRACT
No abstract available.
Subject(s)
Child , Female , Humans , Caroli Disease/complications , Cholangiopancreatography, Magnetic Resonance , Creatinine/blood , Liver/pathology , Liver Transplantation , Polycystic Kidney, Autosomal Recessive/complications , Tomography, X-Ray ComputedABSTRACT
Grumbach-Auvert disease represents a type of Obstructive Disease of the Intrahepatic Biliary Tree. We presents a case report of a patient with hepatic abscess caused by Ascaris which ascended into hepatic parenquima through hepaticojejunostomy, resolved by endoscopic extraction of it after the jejunostomy of permanent access was opened.
Subject(s)
Animals , Female , Humans , Middle Aged , Ascaris lumbricoides , Ascariasis/surgery , Common Bile Duct , Jejunostomy , Ascariasis/complications , Caroli Disease/complicationsABSTRACT
No abstract available.
Subject(s)
Adult , Humans , Male , Bile Ducts, Intrahepatic/pathology , Caroli Disease/complications , Gallstones/complications , Tomography, X-Ray ComputedABSTRACT
Resumen: La enfermedad de Caroli corresponde a la dilatación sacular congénita de la vía biliar intrahepática. Objetivo: Revisar la experiencia del Servicio de Cirugía del Hospital Clínico de la Universidad de Chile en el diagnóstico y tratamiento quirúrgico de esta enfermedad entre 1994 y 2005. Resultados: La serie consta de 18 pacientes, 11 mujeres (61,1 por ciento) y 7 hombres (38,8 por ciento), con 49.8 + 14,5 años de edad promedio. El principal síntoma de presentación fue el dolor abdominal en 16 de 18 pacientes (88,8 por ciento). La enfermedad se presentó localizada en 16 de 18 pacientes (14 lob. izquierdo y 2 lob. derecho), y generalizada en 2. El 100 por ciento de los pacientes fueron estudiados con algún método de imagen, siendo el más usado la ecografía abdominal (10 de 18). Los hallazgos más comunes fueron dilatación de la vía biliar intrahepática, litiasis intrahepática, e imágenes quísticas. El diagnóstico fue confirmado por Colangiografía Endoscópica Retrógrada (CER) o Colangio Pancreato Resonancia Magnética (CPRM) en 10 de 18 pacientes. Todos los enfermos recibieron tratamiento quirúrgico, 17 de ellos (94,4 por ciento) segmentectomía y 1 (5,88 por ciento) destechamiento de quiste. La cirugía duró en promedio 3,4 horas, y la hospitalización 8,7 días. La incidencia de complicaciones fue 11,76 por ciento. No hubo mortalidad en el intra o postoperatorio. El seguimiento promedio fue de 29,38 meses, encontrándose aparición de enfermedad en el lóbulo hepático contralateral en un paciente.
Background: Caroli disease is a congenital sacular dilatation of intrahepatic bile tract. Aim: To report a series of patients with Caroli disease. Material and methods: Retrospective review of medical records of patients with a pathological diagnosis of Caroli disease, operated in a surgical service, between 1994 and 2005. Results:Ne report 18 patients aged 50 + 14 years (11 women). The main presentation symptom was abdominal pain in 16 patients (89 percent). The disease was localized in 16 patients (in the left lobe in 14 and in the right lobe in 2) and generalized in two. The most common findings in abdominal ultrasound or CAT scan, were intrahepatic bile tract dilatation, intrahepatic lithiasis and cystic images. The diagnosis was confirmed by endoscopio retrograde colangiography in six patients and magnetic resonance of the bile tract in four. Seventeen patients were subjected to a segmentectomy and one to a cyst resection. Mean operative time and hospital stay were 3.4 hours and 8.7 days, respectively. No patient died. Patients were followed for a mean of 30 months and in one, the disease reappeared in the contralateral lobe. Conclusions: Partial hepatectomy is a definitive treatment for localized Caroli disease, with a low incidence of complications and mortality
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Caroli Disease/surgery , Caroli Disease/diagnosis , Chile/epidemiology , Abdominal Pain/etiology , Caroli Disease/complications , Follow-Up Studies , Length of Stay , Retrospective Studies , Treatment OutcomeABSTRACT
Caroli disease is a rare congenital malformation characterized by cystic dilatations of large bile ducts. There are two forms of Caroli disease. Simple form entails the bile duct dilatation or ectasia. Complex form is copresense of hepatic fibrosis and portal hypertension. Here is presented a case of central diabetes insipidus (CDI) associated with Caroli disease.
Subject(s)
Caroli Disease/complications , Child, Preschool , Diabetes Insipidus/complications , Humans , MaleABSTRACT
Caroli's disease is a rare congenital disorder and occasional cases have been reported from Japan and other parts of Asia. It comprises of congenital dilation of the lower (segmental) intrahepatic bile duct. Cholangitis liver, cirrhosis and cholangiocarcinoma are its potential complication. A case of caroli's disease in an 8-years-old boy with bilobar involvement of liver, (specially affecting right superior lobe) presenting with intermittent abdominal pain, fever and hepatosplenomegaly is reported here.
Subject(s)
Anemia, Sickle Cell/complications , Caroli Disease/complications , Child , Humans , MaleABSTRACT
Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.
Subject(s)
Male , Infant , Humans , Polycystic Kidney, Autosomal Recessive/complications , Kidney/diagnostic imaging , Hepatic Duct, Common/pathology , Caroli Disease/complicationsABSTRACT
Carolis disease is a rare communicating segmental or diffuse dilatation of the intrahepatic biliary tree. Cholangitis, liver cirrhosis and cholangiocarcinoma are its potential complications. A case of Carolis disease in a boy of 6 years with bilobal involvement presenting with intermittent abdominal pain, fever and hepatomegaly is reported here.
Subject(s)
Caroli Disease/complications , Child , Humans , MaleSubject(s)
Adult , Caroli Disease/complications , Diagnosis, Differential , Edema/etiology , Fatigue/etiology , Hepatitis C, Chronic/complications , Humans , Kidney Failure, Chronic/complications , Male , Polycystic Kidney, Autosomal Dominant/complications , Splenomegaly/diagnosis , Urination Disorders/etiology , Vomiting/etiologySubject(s)
Adult , Caroli Disease/complications , Child , Diagnosis, Differential , Female , Humans , Hypertension, Portal/diagnosis , MaleABSTRACT
Hepatic resection for benign pathology is rare. We report our experience in two patients with hepaticolithiasis. One patient underwent left hemihepatectomy with removal of segments II, III and IV for localized Caroli's disease with multiple intrahepatic calculi. The second patient underwent resection of segment II and III with Roux-en-Y hepaticojejunostomy for right and left intrahepatic calculi with an abscess in the left lobe of the liver and a choledochal cyst.
Subject(s)
Adult , Caroli Disease/complications , Cholelithiasis/complications , Female , Hepatectomy , Hepatic Duct, Common , Humans , Liver Diseases/complications , Middle AgedABSTRACT
Caroli's disease or communicating ectasia of the intrahepatic biliary tree is a rare disease with unknown aetiology. The coexistence of this along with the uncommon condition of a gastric diverticulum has never been reported before. A deficiency in the fibromuscular matrix of both the bile ducts and the gastric wall may explain why these two pathologies may coexist in a single patient.
Subject(s)
Adult , Female , Humans , Diverticulum, Stomach/complications , Caroli Disease/complications , Diverticulum, Stomach/diagnosis , Caroli Disease/diagnosisABSTRACT
A fin de determinar la prevalencia epidemiología y comportamiento clínico-terapêutico de la hepatolitiasis (HL) en el país se envió un cuestionario a 10 centros de referencia, en Radiología invasiva de vía biliar. De 10 centros encuestados, 7 (70 por ciento) contestaron en tiempo y forma. Reuniéndose un total de 8.736 colangiografías (C) en los últimos 5 años: 5.920 (68 por ciento) fueron litiasis biliares y 53 de estas resultaron HL (0.9 por ciento, rango 0,5-2.6 por ciento). El método diagnóstico fue la C. retrógrada en el 36 (68 por ciento) y en el 17 (32 por ciento) la C. transhepática. De 53 pacientes con HL (53 por ciento, x de edad 52, rango 23-85); el 79 por ciento (42/53) se presentó clínicamente con una colangitis; un 6 por ciento 3/53) padeció una pancreatitis aguda y un 9.4 por ciento (5/53), evolucionó a una cirrosis biliar. Las enfermedades predisponentes a HL fueron: en el 28 por ciento (15/53) estenosis postquirúrgica de la via biliar (EPQ); en el 20 por ciento (11/53) Enfermedad de Carolí en otro 28 por ciento (15/53) panlitiasiscoledociana. Mientras que un 9,4 por ciento (5/53) presentó una "historia biliar" (dos o más intervenciones sobre la via biliar) y en un 5,7 por ciento (3/53) no se hallaron factores predisponentes. En un 77 por ciento se observó un follow-up de 38 meses (rango 8-60), con una mortalidad de 4,8 por ciento (2/41): siendo tratados con cirurgía en el 58 por ciento de los casos (31/53); papilotomía en el 17 por ciento (9/53) y tratamiento combinados en el 15 por ciento (8/53) que incluían a litotripsia extracorpórea y Ac. Ursodesoxicólico(AUDC). Cuatro de 53 (7.5 por ciento) recibieron AUDC como única terapéutica. Se concluye que la HL es una entidad con alta morbilidad biliar (85 por ciento) y heoática (cirrosis en el 9.4 por ciento). Cuando se diagnostica en Occidente, debe buscarse EPQ o un Carolí. Siendo los tratamientos combinados o el AUDC como única alternativa, una nueva modalidad terapéutica del mundo occidental.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Caroli Disease/complications , Lithiasis/complications , Lithiasis/epidemiology , Liver Diseases/complications , Liver Diseases/epidemiology , Aged, 80 and over , Argentina , Caroli Disease/diagnosis , Caroli Disease/epidemiology , Diagnosis, Differential , Lithiasis/diagnosis , Lithiasis/therapy , Liver Diseases/diagnosis , Liver Diseases/therapy , Prevalence , Surveys and QuestionnairesABSTRACT
A Síndrome de Caroli é uma entidade incomum caracterizada pela presença de dilatações císticas da árvore biliar intra-hepática e fibrose hepática congênita. Pode comportar-se como condição pré-maligna, evoluindo em alguns casos para colangiocarcinoma. Relatamos um caso diagnosticado em paciente do sexo masculino, 37 anos, complicado por carcinomatose peritoneal de sítio desconhecido, fazendo também revisão da literatura
Subject(s)
Humans , Male , Adult , Caroli Disease/complications , Caroli Disease/physiopathology , Cholangiocarcinoma/physiopathology , Peritoneal Neoplasms/etiologySubject(s)
Caroli Disease/complications , Humans , Infant , Polycystic Kidney Diseases/complicationsABSTRACT
Presentamos el caso de una mujer de 51 años con dolor abdominal después de una colecistectomía. El estudio demostró dilataciones de la vía biliar y litiasis intrahepática en el lóbulo izquierdo, en la ecografía y tomografía computarizada. La paciente se operó con el diagnóstico de enfermedad de Caroli, confirmándose el diagnóstico. Se practicó una lobectomía izquierda de la cual evolucionó bien y el estudio histológico reveló un colangiocarcinoma in situ
Subject(s)
Humans , Female , Middle Aged , Bile Duct Neoplasms/complications , Caroli Disease/complications , Cholangiocarcinoma/complications , Caroli Disease/surgery , Cholangiocarcinoma/pathology , Cholangiocarcinoma/surgeryABSTRACT
Las patologías mixtas de hígado (ductos biliares intrahepáticos dilatados asociados con colangiocarcinoma), han sido ampliamente estudiados en la literatura a pesar de su infrecuencia. Presentamos el caso de un paciente masculino de 30 años con colangiocarcinoma moderadamente diferenciado, infiltrante a vesícula, encontrándose como hallazgo a la autopsia: ductos biliares dilatados intrahepáticos asociados a un foco hermatoso de vasos sanguíneos en un estroma laxo