Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Ultrasound-Guided Percutaneous Radiofrequency Ablation of Liver Tumors: How We Do It Safely and Completely

Ultrasound-guided percutaneous radiofrequency (RF) ablation has become one of the most promising local cancer therapies for both resectable and nonresectable hepatic tumors. Although RF ablation is a safe and effective technique for the treatment of liver tumors, the outcome of treatment can be closely related to the location and shape of the tumors. There may be difficulties with RF ablation of tumors that are adjacent to large vessels or extrahepatic heat-vulnerable organs and tumors in the caudate lobe, possibly resulting in major complications or treatment failure. Thus, a number of strategies have been developed to overcome these challenges, which include artificial ascites, needle track ablation, fusion imaging guidance, parallel targeting, bypass targeting, etc. Operators need to use the right strategy in the right situation to avoid the possibility of complications and incomplete thermal tissue destruction; with the right strategy, RF ablation can be performed successfully, even for hepatic tumors in high-risk locations. This article offers technical strategies that can be used to effectively perform RF ablation as well as to minimize possible complications related to the procedure with representative cases and schematic illustrations.

Vanishing bile duct syndrome in a Hodgkin's lymphoma patient with fatal outcome despite lymphoma remission

Vanishing bile duct syndrome [VBDS] is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts [ductopenia] leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma [HL]. We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.

Tratamento endoscópico das lesões biliares / Endoscopic treatment of the biliary injuries

As técnicas cirúrgicas convencionais ofertam uma apropriada condição de cura na maioria dos pacientes com estreitamento biliar benigno. Nesta condição, no entanto, o reparo cirúrgico está associado com recorrência tardia da re-estenose em 10 por cento a 30 por cento dos pacientes. Neste contexto, os avanços tecnológicos na endoscopia terapêutica promoveram a possibilidade alternativa do tratamento efetivo destas obstruções benignas. Considerações em relação ao tempo de reospitalização e de procedimentos devem ser averiguados em detalhes e ponderados em relação á cirurgia. Estenoses malignas estão relacionadas á colangite, icterícia e dor e, consequentemente, com as alterações sistêmicas relacionadas com a sepsis biliar. A conduta endoscópica cria uma derivação do suco biliar para o duodeno, sendo uma verdadeira derivação biliodigestiva endoscópica e utilizando-se próteses plásticas ou metálicas. O propósito desta revisão é ofertar aos leitores a eficácia do tratamento endoscópico na estenose benigna e maligna biliopancreática.

Standard surgical techniques offer a good chance of cure forthe majority of patients affected by extrahepatic benign biliarystricture. Nevertheless, operative repair has a long-termrecurrence rate of stricture in 10 percent to 30 percent of patients. Advancesin endoscopic procedures have provided alternative options ofrelieving biliary obstructions, but prolonged length of treatmentand rehospitalization have to be considered if endoscopy isperformed. Malignant stenoses are an important factor determining cholangitis, jaundice and pain and it's each systemic consequences. The endoscopic approach intends to create a deviation of bile juice to duodenum by means of metal or plastic stents aiming a safe palliation. The purpose of this review is to show to the readerseffectiveness of endoscopic treatments of benign and malignat stenosis of the bile duct and pancreatic.

Endoscopic management of postoperative bile duct injuries: a single center experience

Biliary endoscopic procedures may be less invasive than surgery for management of postoperative bile duct injuries [POBDI]. This retrospective work presents the experience of a single referral center during a period of 14 years in endoscopic management of POBDI. Between 1994 [March] and 2008 [May], ERCP had been performed on 277 patients suspected to have POBDI. Patients shown to have complete transaction of bile duct were prepared for definitive surgery. For patients with simple biliary leak, sphincterotomy was performed with stenting. Pneumatic dilatation and stenting were done on patients with biliary stricture and preserved ductal continuity. ERCP was repeated every 3 months till the site of narrowing disappeared. The mean age was 45.3 years, 162 [58.5%] were females. The most common previous surgery was cholecystectomy [open, [N=119] 44%, and laparoscopic, [N=77] 28%]. ERCP failed in 17 patients [6.1%]. For successfully cannulated cases [N=260, 93.9%], the type of bile duct injury diagnosed at ERCP was completely ligated CBD [N=31/260, 11.9%]. Bile leakage was detected in [N=167/260, 64.2%] all patients with endoscopic sphincterotomy and stent insertion, the leak stopped in all of them. Biliary stricture was diagnosed in 33/260 patients [12.7%] and 17 of them had repeated balloon dilatation with stenting while the remaining had surgical correction. The success rate of endoscopic therapy for biliary strictures was 82%. Cholangiogram was normal in 29 patients [11.2%]. Endoscopic therapy is safe and effective in the management of postoperative bile duct leak. For postoperative bile ductal strictures, ERCP is a less favorable option

Physiologic and pathologic experimental models for studying cholangiocytes / 대한간학회지

Cholangiocytes (epithelial cells lining the intra- and extrahepatic bile ducts) and hepatocytes are two major components of liver epithelia. Although cholangiocytes are less numerous than hepatocytes, they are involved in both bile secretion and diverse cellular processes such as cell-cycle phenomena, cell signaling, and interactions with other cells, matrix components, foreign organisms, and xenobiotics. Cholangiocytes are also targets in several human diseases including cholangiocarcinoma, primary sclerosing cholangitis, autoimmune cholangitis, and vanishing bile-duct syndrome. The rapid advances in experimental biology technologies are greatly expanding interest in and knowledge of the physiology and pathophysiology of cholangiocytes. This review focuses on the progress of in vivo and in vitro experimental models in elucidating the physiologic functions of cholangiocytes and the pathophysiology of various cholangiopathies. The following aspects are reviewed: isolation of cholangiocytes from the liver and their heterogeneity, various culture systems, establishment of cholangiocyte cell lines, isolation and usage of intrahepatic bile-duct units, three-dimensional modeling of the bile duct, experimental models for inducing cholangiocyte proliferation, and various cholangiopathies such as cholangiocarcinoma, primary sclerosing cholangitis, and autoimmune cholangitis.

Malformación del sistema pancreático-biliar como causa de perforación espontánea de la vía biliar extrahepática / A pancreatobiliary malformation causing spontaneous perforation of the extrahepatic bile duct

La ascitis biliar usualmente resulta de la perforación espontánea o traumática del conducto biliar principal, usualmente en la unión con el conducto cístico. La obstrucción ductal distal, el reflujo hacia la vía biliar común, la debilidad o una muy localizada malformación mural de la pared del conducto común han sido propuestas como causas probables. Presentamos el caso de una niña de 11 meses de edad quien sufrió ascitis biliar como consecuencia de la ruptura del colédoco. La perforación probablemente se debió a una malformación de la unión pancreático-biliar. Se le derivó la bilis hacia el exterior mediante una sonda.

Biliaryascitis usually results from a spontaneous or traumatic perforation of the common bile duct, usually at the junction with the cystic duct. Distal duct obstruction, pancreatic fluid reflux up the common bile duct, congenital weakness or a localized mural malformation of the wall of the common duct have been proposed as possible causes. We present an 11-months-old infant girl who developed a biliary ascitis as a consequence of biliary rupture. Perforation was due to malformation of the pancreatico-biliary junction. An uneventful external derivation by tube was performed.

The Balloon Dilatation and Large Profile Catheter Maintenance Method for the Management of the Bile Duct Stricture Following Liver Transplantation

OBJECTIVE: We wanted to evaluate the therapeutic efficacy of the percutaneous balloon dilatation and large profile catheter maintenance method for the management of patients with anastomotic biliary strictures following liver transplant. MATERIALS AND METHODS: From May 1999 to June 2003, 12 patients with symptomatic benign biliary stricture complicated by liver transplantation were treated with the percutaneous balloon dilatation and large profile catheter maintenance method (1-6 months). The patients were eight males and four females, and their ages ranged from 20 to 62 years (mean age: 44 years). Ten patients underwent living donor liver transplantation and two underwent cadaveric liver transplantation. Postoperative biliary strictures occurred from two to 21 months (mean age: 18 months) after liver transplantation. RESULTS: The initial technical success rate was 92%. Patency of the bile duct was preserved for eight to 40 months (mean period: 19 months) in 10 of 12 (84%) patients. When reviewing two patients (17%), secondary balloon dilatations were needed for treating the delayed recurrence of biliary stricture. In one patient, no recurrent stenosis was seen during the further 10 months follow-up after secondary balloon dilatation. Another patient did not response to secondary balloon dilatation, and he was treated by surgery. Eleven of 12 patients (92%) showed good biliary patency for 8-40 months (mean period: 19 months) of follow-up. CONCLUSION: The percutaneous balloon dilatation and large profile catheter maintenance method is an effective therapeutic alternative for the treatment of most biliary strictures that complicate liver transplantation. It has a high success rate and it should be considered before surgery.

A Case of Vanishing Bile Duct Syndrome Associated with Hypersensitivity to Allopurinol / 대한간학회지

Allopurinol is frequently used for the treatment of hyperuricemia and gout. Sometimes, a life-threatening reaction develops, as is illustrated by the following case report. We describe a 60-year-old male patient who was treated with allopurinol because of asymptomatic hyperuricemia, and he was presented with fever, skin rash, eosinophilia, worsening renal function and vanishing bile duct syndrome. In this report, we discussed vanishing bile duct syndrome as a serious side effect of allopurinol, and we briefly reviewed the etiology, prevention, and treatment modalities for vanishing bile duct syndrome.

Colour Doppler flow imaging findings in portal biliopathy.

Biliary abnormalities in extrahepatic portal vein obstruction (EHPVO) have been described in detail on endoscopic retrograde cholangiopancreaticography (ERCP), but have never before been reported on colour Doppler flow imaging (CDFI). These changes occur either due to extrinsic compression of the bile ducts or due to ischaemic biliary stenosis. The aim of this study was to evaluate the role of CDFI in demonstrating the biliary changes in patients with EHPVO. Three out of 46 patients with EHPVO (7%), who presented clinically with obstructive jaundice, were subjected to a detailed CDFI study of the abdomen, followed by ERCP and splenoportovenography (SPV). One of the patients also underwent a repeat CDFI examination at 8 weeks following a lienorenal shunt. In all 3 cases, CDFI easily distinguished the anechoeic structures seen on ultrasound at the porta hepatis as periportal collaterals, the hepatic artery and dilated bile ducts. It revealed the actual indentation made on the common bile duct (CBD) by the collaterals besides showing the presence of biliary calculi. Gallbladder varices were also well demonstrated in all the cases. ERCP confirmed the presence of portal biliopathy besides showing other changes, such as angulation, displacement and stricture of the CBD. SPV confirmed the presence of EHPVO. One patient who underwent shunt surgery showed persistent dilatation of the CBD with calculi. CDFI is a rapid, non-invasive and widely available modality which can be used to demonstrate the biliary changes in patients with EHPVO with obstructive jaundice. It may thus help screen patients who require a further by ERCP examination for the planning of treatment.

Bile duct-duodenal fistula caused by AIDS/HIV-associated tuberculosis

Allthough infrequent, digestive fistulae in HIV/AIDS patients have been reported throughout the digestive tract from the esophagus to the anus, with predominance of esophageal fistulae. AIDS/HIV-associated opportunistic infections may invade the digestive system and lead to fistula formation. Tuberculosis is the most common infection associated with these esophageal fistulae. We report here one case of bile duct-duodenal fistula in a female AIDS patient with associated abdominal Mycobacterium tuberculosis infection compromising lymphnodes of the hepatic pedicle where the fistula was found. According to the reviewed literature, this is the third case of bile duct-duodenal fistula associated with abdominal tuberculosis in AIDS patient, and the first where both the fistula and the tuberculosis infection were diagnosed at laparotomy for acute abdomen. Whether the AIDS patient with abdominal pain needs or not a laparotomy to treat an infectious disease is often a difficult matter for the surgeon to decide, as most of the times appropriate medical treatment will bring more benefit

Retained CBD stone as a cause of cystic bile duct leakage after laparoscopic cholecystectomy

One case of cystic bile duct leakage was encountered in 1400 cases of laparoscopic cholecystectomy. Endoscopic re t rograde cholangio-pancreatography [ERCP] showed an impacted stone in the lower end of the common bile duct. This small stone has passed through a wide cystic duct during manipulation at surgery. Selective operative cholangiography was recommended by the author in cases of small gall bladder stones in the presence of a wide, patent cystic duct. ERCP was recommended as the principle diagnostic as well as therapeutic tool in such cases

External biliary fistula.

A biliary fistula is almost invariably related to gallstone disease and commonly follows a hurried cholecystectomy by an inexperienced surgeon. This catastrophy which is largely preventable, often necessitates repeated surgical intervention and accrues an estimated 5-year mortality rate approaching 30%. Published series only show a slight increase in the incidence (one per 150-200) after laparoscopic cholecystectomy. The injury results from imprecise dissection and inadequate demonstration of the anatomical structures. The diagnosis is usually obvious and persistent tachycardia and hypotension inspite of an adequate intravenous infusion and a normal central venous pressure is another well known indicator of subhepatic collection of bile, which indicates an urgent ultrasonographic scanning of the upper abodmen. ERCP is a useful diagnostic and therapeutic tool when the continuity of the extra-hepatic biliary system has not been disrupted. An endobiliary stent can be placed across the defect in the same sitting, to tide over the immediate crisis and perhaps treat the patient on a permanent basis. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive technique of outlining both the intra and extrahepatic biliary tree, which can provide a better road map of the fistula than an ERCP. The management has to be tailored to the patient's condition and the expertise available. A bilio-enteric anastomosis, performed 4 to 6 months after the initial surgery on a dilated common hepatic duct is more likely to succeed than an operation on a septic, hypoproteinemic patient with sodden, friable, non-dilated bile ducts. On the other hand, waiting for the ducts to dilate in a patient with a complete transection of the bile ducts with complete biliary diversion only leads to depletion of the bile acid pool, severe electrolyte derangement and nutritional failure, leading on to sepsis and death.

Radiologic findings of Mirizzi syndrome with emphasis on MRI

We have reported a case of Mirizzi syndrome preoperatively diagnosed using MR cholangiopancreatography. MRCP and T2-weighted image using a single-shot fast spin-echo sequence accurately depicted all components of Mirizzi syndrome, including impacted stone in the neck of the gallbladder compressing the common hepatic duct and wall-thickening of the gallbladder without any evidence of malignancy. The combination of MRCP and T2-weighted image can be counted on to replace conventional modalities of diagnosing Mirizzi syndrome without any loss of diagnostic accuracy.

Lesäo iatrogênica das vias biliares: estudo de 11 casos / Iatrogenic injury of the bile ducts: study of 11 cases

Foram estudados 11 pacientes com lesao iatrogênica de via biliar. Em todos os casos a lesao ocorreu após uma colecistectomia com ou sem exploraçao das vias biliares. Dos 11 pacientes submetidos à cirurgia das vias biliares, nove (81,81 por cento) foram operados eletivamente e somente dois (18,18 por cento) em caráter de urgência. Considerando-se o local da lesao no trato biliar, os pacientes foram agrupados de acordo com a classificaçao de Bismuth. Dez dos 11 pacientes foram operados na tentativa de se corrigir a lesao da via biliar. O tratamento cirúrgico mais freqüentemente empregado foi a derivaçao jejunal em alça exclusa em y de Roux (nove pacientes). Um paciente foi tratado sem reintervençao cirúrgica. O resultado foi considerado bom em oito (72,72 por cento) dos 11 pacientes, estando relacionado ao número de reoperaçoes, ao local da lesao e da ocorrência de fístulas pós-operatórias.

Colangiopatia relacionada a Síndrome da Imunodeficiência Adquirida: relato de caso e revisäo da literatura / Cholangiopathy related to AIDS: case report and literature review

Anormalidades na via biliar de etiologia ainda näo bem estabelecida têm sido descritas de forma esporádica entre os pacientes com Síndrome de Imunodeficiência Adquirida. Os autores apresentam um caso em que foram detectadas tais anormalidades e revisam os principais aspectos referentes a etiologia, características clínicas, diagnóstico e tratamento desta síndrome

Congenital absence of gallbladder

Nine surgically proven congenital absence of gallbladder (CAGB) cases were reviewed. All of them had one or more kinds of biliary symptom. Tests such as abdominal ultrasonography, intravenous or oral cholecystography and even endoscopic retrograde cholangiography not only failed to predict CAGB but misleadingly indicated other similar conditions. Only the abdominal computed tomography (CT), performed on one patient, enabled the accurate diagnosis of CAGB. All the patients underwent abdominal exploration, and CAGB was confirmed by the meticulous dissection of the entire extrahepatic biliary tree and the operative cholangiography. Five patients had concomitant biliary pathologies responsible for their symptoms, but four patients had isolated CAGB. CAGB is a rarely encountered condition for a clinician, but extensive diagnostic work-ups including abdominal CT should be performed in all situations where CAGB is suspected. Thus unnecessary exploration can be avoided in the isolated CAGB case.

Biloma / Biloma

El Biloma es una colección encapsulada de bilis, secundaria a ruptura iatrogénica, traumatica o espontánea del árbol biliar, de localización intra o extrahepática con síntomas agudos o crónicos, de tratamiento médico o quirúrgico