Haemostatic radiation therapy for a bleeding intraductal papillary neoplasm of the biliary tree.

Haemostatic radiation was effectively used as a novel rescue therapy in a 60-year-old man who presented with recurrent melaena refractory to all conventional medical and surgical measures. He needed multiple transfusions and was diagnosed to be bleeding from an intraductal papillary biliary neoplasm which was not amenable to surgical resection in view of the background liver disease. He received conventional radiation therapy (RT) of a dose of 3 Gy per fraction for 3 consecutive days after which he stabilised. After cessation of the RT, he did not require transfusion for the next 2 months. His quality of life improved and it gave us time to evaluate for other definitive measures.

Blockade of Hedgehog Signaling Attenuates Biliary Cystogenesis in the Polycystic Kidney (PCK) Rat.

Caroli disease represents a hepatic manifestation of autosomal recessive polycystic kidney disease, and belongs to a class of cholangiociliopathies. The role of Hedgehog signaling, a major pathway regulated by primary cilia, in biliary cystogenesis in Caroli disease remains unknown. Using the polycystic kidney (PCK) rat as an animal model of Caroli disease, this study investigated the involvement of Hedgehog signaling in its pathogenesis. In vitro experiments revealed that PCK cholangiocytes overexpressed Smoothened, Gli1, and Gli1's target molecule cyclin D1. The nuclear expression of Gli1, Gli2, and Gli3 was observed in PCK cholangiocytes by immunocytochemistry. An immunohistochemical analysis using liver sections confirmed the overexpression of Smoothened and cyclin D1, and the nuclear expression of the Gli proteins in the biliary epithelium of PCK rats as well as human Caroli disease. The treatment of PCK cholangiocytes with cyclopamine inhibited cell proliferative activity that was associated with the inhibition of nuclear translocation of Gli1 and Gli2, and reduced cyclin D1 expression. The in vivo administration of cyclopamine to PCK rats decreased abnormally elevated serum liver enzymes, and significantly attenuated bile duct dilation as well as kidney cyst formation. These results suggest that cholangiocyte hyperproliferation is causally associated with the aberrant activation of Hedgehog signaling, and the inhibition of the signaling has potential as a therapeutic strategy for biliary cystogenesis in Caroli disease.

Contrast-enhanced magnetic resonance cholangiography with gadoxetic-acid-disodium for the detection of biliary-cyst communication in Caroli disease / Contraste colangiografía RM con gadoxetic-ácido disódico para la detección de la comunicación biliares-quistes en la enfermedad de Caroli

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[Curative partial hepatectomy in adult Caroli's disease]. / Hepatectomía parcial curativa en la enfermedad de Caroli del adulto.

Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult polycystic kidney disease. The most effective therapeutic option in symptomatic unilobar Caroli's disease is resection of the affected lobe. In bilobar involvement, liver transplantation should be considered. Due to their rarity, we report three cases of Caroli's disease successfully resolved through lobectomy of the affected lobe: two patients with recurrent cholangitis and one patient with associated adult polycystic kidney disease.

Enfermedad de Caroli / Caroli’s disease

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Síndrome de Caroli e carcinomatose peritoneal / Caroli's syndrome and peritoneal carcinomatosis

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

Value of Doppler sonography in the assessment of patients with Caroli's disease.

PURPOSE: We examined the intracystic flow pattern and portal hemodynamics with Doppler sonography in patients with Caroli's disease. METHODS: Sonographic characteristics and portal hemodynamics were studied by color Doppler sonography and spectral analysis in 5 patients (4 children and 1 adult) with Caroli's disease using high-frequency transducers. Caroli's disease was associated with infantile polycystic kidney disease in 4 patients and congenital hepatic fibrosis in 2 patients. Four patients had no clinical or imaging evidence of portal hypertension. The adult patient had congenital hepatic fibrosis and portal hypertension. RESULTS: Color Doppler sonograms and spectral analyses disclosed distinctive hepatic arterial and portal venous flow within the fibrovascular projections in the bile ducts of all the children. The adult with advanced portal hypertension presented with a no-flow state in the intracavitary part of the portal vein and a strong arterial signal related to disturbed hemodynamics in the arterialized liver. CONCLUSIONS: The fact that portal hemodynamics change over time should be taken into account when Doppler assessment of Caroli's disease is attempted. Doppler sonographic monitoring of the portal system to indirectly diagnose and follow the progression of so-called congenital hepatic fibrosis may be an effective alternative to liver biopsy.

Enfermedad de Caroli / Caroliïs disease

La enfermedad de Caroli es una causa poco común de colangitis recurrente secundaria a obstrucción ductal segmentaria. Dicha entidad, cuya transmisión es de carácter autosómico recesivo, se caracteriza por la dilatación sacular de los conductos biliares intrahepáticos, que predispone a la estasis de bilis, y precipitación de cristales que contribuyen a la obstrucción del árbol biliar con la formación de cálculos intraductales. El diagnóstico se apoya en imágenes tomográficas y ultrasonográficas; sin embargo, el estudio de elección para estudiar el árbol biliar es la colangio-pancreatografía retrógrada endoscópica. El tratamiento consiste en la administración de antibióticos y en la remoción de los litos de la vía biliar. Cuando la enfermedad se limita a un solo lóbulo hepático, debe de efectuarse lobactomía. Se presenta el caso de un paciente masculino de 42 años de edad, con cuadro de colangitis, en el que posteriormente se demostró dilatación quística de los conductos biliares intrahepáticos. Se expone la metodología diagnóstica seguida y el tratamiento adoptado

Long-term (over 22 years) follow-up of four familial cases of Caroli's disease.

The authors report a long-term (over 22 years) follow-up of four familial cases, 1 brother and 3 sisters, affected by classic Caroli's disease. The male patient experienced acute cholangitis at the age of 6 and 1 of the 3 sisters at 12 years. The other two sisters were asymptomatic at the time of diagnosis. At the last control, all the patients were alive and have remained symptom-free for more than 22 years. Nevertheless, some data suggest a slowly evolving impairment of the disease even in the absence of a clinical expression. In this paper, the ways in which Caroli's disease is inherited and managed are also discussed.