Ileal exclusion for pruritus treatment in children with progressive familial intrahepatic cholestasis and other cholestatic diseases.

BACKGROUND: Pruritus is a major health-related quality-of-life burden in progressive familial intrahepatic cholestasis (PFIC) and other childhood cholestatic liver diseases. Several nontransplant surgical techniques were developed in an attempt to ameliorate symptoms and slow disease progression. Very few case-series have been published on a particular intervention, ileal exclusion (IE), which has been considered to be inferior to the other approaches. METHODS: We conducted a single-center retrospective chart-review case-series of patients submitted to IE as the first-line surgical treatment at our institution from 1995 to 2018. The primary goal was pruritus relief, followed by survival with the native liver and improvement in biochemical parameters. RESULTS: Eleven patients were submitted to IE, with a mean follow-up of 60 months. Complete resolution or significant reduction of pruritus was obtained in 72.7% (n = 8) of patients. One patient (9.1%) had a major postoperative complication that required surgery. No other morbidities were reported. Two cases progressed to end-stage liver disease (ESLD) within the short-term and one year after surgery. CONCLUSIONS: This case series study shows that IE provided excellent results in pruritus control and permitted survival with the native liver. We believe IE is a safe procedure, with few associated morbidities, and should be considered more often as primary surgical treatment for PFIC and other cholestasis. LEVEL OF EVIDENCE: IV.

Intrahepatic cholangiojejunostomy for complex biliary stenosis after pediatric living-donor liver transplantation.

The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached. A side-to-side anastomosis to the previous Roux-en-Y limb is performed over a silicone stent. Among 328 pediatric liver transplants performed between 1988 and 2015, 26 patients developed biliary stenosis. From nine patients requiring surgery, three patients who had received left lateral grafts from living-related donors due to biliary atresia were successfully treated with IHCJ. After a mean of 45.6 months, all patients are alive with normal liver morphological and function tests. The presented technique was a feasible and safe surgical option to treat selected pediatric recipients with complex biliary stenosis in whom percutaneous procedures or rehepaticojejunostomy were not possible, allowing complete resolution of cholestasis and thus avoiding liver retransplantation.

Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2.

Progressive familial intrahepatic cholestasis type 2 (PFIC 2) results from mutations in ABCB11 gene coding bile salt export pump (BSEP). Medical treatment is usually unsuccessful and surgery intervention is necessary. Partial external biliary diversion (PEBD) is regarded as the first choice of surgical treatment. Ileal exclusion (IE) is an alternative operation if external stoma is not tolerated; however, a favorable outcome is uncertain. In chronic liver diseases pregnancy brings additional risk of deterioration of liver function and generally is not recommended. We present the first case report of successful pregnancy in a genetically confirmed PFIC 2 patient after surgical conversion from PEBD to IE.

Bile acid pool dynamics in progressive familial intrahepatic cholestasis with partial external bile diversion.

OBJECTIVES: Partial external bile diversion (PEBD) is an established therapy for low-γ-glutamyl transferase (GGT) progressive familial intrahepatic cholestasis (PFIC). This study sought to determine whether the dynamics of the cholic acid (CA) and chenodeoxycholic acid (CDCA) pools in subjects with low-GGT-PFIC with successful PEBD were equivalent to those achieved with successful liver transplantation (LTX). METHODS: The kinetics of CA and CDCA metabolism were measured by stable isotope dilution in plasma samples in 5 subjects with PEBD, all with intact canalicular bile salt export pump expression and compared with subjects with low-GGT-PFIC with successful LTX. Stomal loss of bile acids was measured in subjects with PEBD. RESULTS: The fractional turnover rate for CA in the PEBD group ranged from 0.5 to 4.2/day (LTX group, range 0.2-0.9/day, P = 0.076) and for CDCA from 0.7 to 4.5/day (LTX group 0.3-0.4/day, P = 0.009). The CA and CDCA pool sizes were equivalent between groups; however, pool composition in PEBD was somewhat more hydrophilic. The CA/CDCA ratio in PEBD ranged from 0.9 to 19.5, whereas in LTX it ranged from 0.5 to 2.6. Synthesis rates computed from isotope dilution correlated well with timed output for both CA (r2 = 0.760, P = 0.024) and CDCA (r2 = 0.690, P = 0.021). CONCLUSIONS: PEBD results in bile acid fractional turnover rates greater than LTX, pool sizes equivalent to LTX, and pool composition that is at least as hydrophilic as produced by LTX.

[Partial external biliary diversion: a therapeutic alternative for Alagille syndrome. First case in Argentina]. / Derivación biliar externa parcial: alternativa terapéutica en el síndrome de Alagille. Primer caso en la Argentina.

BACKGROUND: Alagille 's syndrome is the main syndromic chronic intrahepatic cholestasis characterized by hypoplasia of the intrahepatic bile ducts. It is a multisystem disorder of autosomal dominant inheritance with involvement of multiple organs. Usually it becomes apparent in the neonatal period, presenting as subclinical cases or severe degrees of the disease with the consequent development of liver cirrhosis and subsequent liver failure associated with multiple abnormalities: defects in the vertebral arches, typical facies, pulmonary stenosis, mental retardation and hypogonadism. OBJECTIVE: To present the first case of partial external biliary diversion in Argentina, showing the surgical technique and the improvement in the quality of life, as an alternative to be considered in patients with Alagille's syndrome before the development of cirrhosis. RESULTS: It has been shown that partial external biliary diversion can stop the process of liver fibrogenesis, halting the progression of the disease and avoiding the need for transplantation in some types of intrahepatic cholestasis when cirrhosis has not been established. DISCUSSION: This surgical technique can improve the quality of life and morbidity associated with hypercholesterolemia in patients with Alagille's syndrome, delaying and maybe avoiding the need for liver transplantation.

Partial internal biliary diversion through a cholecystojejunocolonic anastomosis--a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report.

BACKGROUND/PURPOSE: The purpose of the study was to describe the initial experience with a novel approach to the surgical treatment of progressive familial intrahepatic cholestasis (PFIC), avoiding the creation of a permanent stoma. METHODS: Two teenaged patients, aged 15 and 17 years, underwent partial internal biliary diversion to treat uncontrollable pruritus associated with PFIC. The surgical technique involved the creation of an isolated jejunal conduit, anastomosed proximally in a terminolateral fashion to the gallbladder and distally to the ascending colon. This operation combines the advantages of partially diverting the biliary flow from the enterohepatic cycle, avoiding an external biliary fistula. In one of the patients, this technique was used as a primary procedure, whereas in the other, a previous partial external diversion was converted to an internal diversion. RESULTS: Both patients had complete resolution of their pruritus and normalization of hepatic laboratory tests. One of the patients developed a mild choleretic diarrhea that can be controlled with eventual use of cholestyramine. No complications were observed related to this operation. CONCLUSIONS: Biliary diversion appears to be a very attractive surgical option for the treatment of PFIC in children with a normal gallbladder. Long-term follow-up is necessary to evaluate late results and eventual complications of this approach.

Monolobar Caroli's disease in an adult. Case report.

Caroli's disease is the dilatation of the segmental intrahepatic bile ducts which generally presents in a diffuse form, but may occasionally involve only a single lobe, commonly the left one. We report the case of a 64-year-old male who presented with a clinical picture of obstructive jaundice, with Caroli's disease in segments II and III of the liver. Preoperative diagnosis was made using abdominal ultrasound and computed tomography scan, confirmed by endoscopic retrograde cholangiopancreatography. The treatment used was segmentectomy II and III (left lobectomy--Couinaud's classification) of the liver. Macroscopic examination of the resected specimen revealed cystic dilatation of the intrahepatic bile ducts and intrahepatic lithiasis. Histologically there was no evidence of malignancy. Liver resection is the treatment of choice for Caroli's disease confined to a single lobe or segment, eliminating the potential for cholangitis, lithiasis and carcinoma.

[Postoperative biliary stenosis: long-term results of endoscopic treatment]. / Estenosis biliar postoperatoria: resultados del tratamiento endoscópico a largo plazo.

BACKGROUND: Endoscopic stent placement is often the initial therapy in symptomatic patients with postoperative strictures because patients are usually diagnosed at the time of ERCP. Although stent insertion rapidly relieves symptoms of biliary obstruction and can even be live-saving in patients with cholangitis, all stents eventually clog, necessitating regular stent changes every 3 to 4 months. Results from several groups suggested that placing multiple stents for months to years could dilate the stricture permanently and thus also treat patients with postoperative biliary strictures palliatively. OBJECTIVE: The outcome of temporary biliary stent placement for postoperative bile duct stenosis was retrospectively evaluated. This is a review of our experience with endoscopic dilation and stent placement in postoperative biliary strictures. PATIENTS AND METHODS: Thirty patients with postoperative strictures diagnosed with ERCP were treated with long-term endoscopic stent placement. One 10 Fr stent was placed at first whenever possible, and stents were exchanged every 3 months for a total of 18 months as median. RESULTS: Four men and 26 women with mean age 42 years (range 16-69 years), and laparoscopic cholecystectomy in six and cholecystectomy (open procedure) in 24; surgical history was reviewed retrospectively. Five patients were lost to follow-up and 25 patients were followed for a median of 18 months. In all 25 patients, previous to stent placement, 8.5 or 10 Fr, a mechanical or hydrostatic dilation was necessary. Stents were exchanged every 3 months to avoid cholangitis caused by clogging. Three 10 Fr stents were inserted in one patient, two 10 Fr stents in 14 patients, one 10 Fr and one 8.5 Fr stent in nine patients, and in one patient, one 10 Fr stent. Six patients (24%) developed recurrent stenosis and required surgery. CONCLUSIONS: Endoscopic treatment with mechanical or hydrostatic dilation and stent insertion may improve long-term results for patients with postoperative biliary strictures.

Malignant hilar and perihilar biliary obstruction: use of MR cholangiography to define the extent of biliary ductal involvement and plan percutaneous interventions.

PURPOSE: To determine the usefulness of magnetic resonance (MR) cholangiography in defining the extent of biliary ductal involvement in patients with malignant hilar and perihilar biliary obstruction and to evaluate whether findings at MR cholangiography alone are sufficient to plan percutaneous interventions in these patients. MATERIALS AND METHODS: Twenty-nine patients with malignant hilar and perihilar biliary obstruction were examined with MR cholangiography. Two radiologists evaluated MR images and determined the extent of biliary ductal involvement. A hypothetical plan for biliary drainage was established prior to any intervention. All patients underwent percutaneous cholangiography, and 27 of 29 patients also underwent biliary drainage and/or stent placement within 7 days after MR cholangiography. By using direct cholangiography as the standard of reference, the usefulness of MR cholangiography in defining the extent of biliary ductal involvement was determined. The type of drainage performed was compared with the type that had been anticipated at MR cholangiography. RESULTS: MR cholangiography was adequate in helping predict the extent of biliary ductal involvement in 28 (96%) of 29 patients and led to underestimation of the extent of the disease in one patient. The therapeutic plan anticipated with MR cholangiography matched the one actually used in 24 (83%) of 29 patients. CONCLUSION: The high accuracy of MR cholangiography for defining extent of ductal involvement in patients with malignant hilar and perihilar obstruction allows adequate planning of percutaneous interventions in a majority of patients.

[Cholestasis in pregnancy. Report of a case]. / Colestasis del embarazo. Presentación de un caso.

We present a case of Cholestasis and pregnancy that developed associated in the puerperium, to tecaluteinic ovaries and hemoperitoneum. For this reason it was necessary surgical management. We considered that it is a very unusual case so we felt interest on presenting it. To finish we would to make reference to itns possible patogenia, symptoms, and management of it.

Evaluación de la utilidad clínica del efecto colangiográfico por RMN en el paciente con ictericia obstructiva / Utility of N.M.R in the evaluation of patients with obstructive jaundice

El efecto colangiográfico por RMN (CRMN) es una técnica relativamente nueva, no invasiva capaz de producir imágenes de alta calidad del árbol biliar. El propósito de este estudio fue determinar en forma prospectiva, la utilidad clínica del CRMN en la evaluación del paciente con ictericia obstructiva. Evaluamos por CRMN en 30 pacientes con ictericia obstructiva la causa y el nivel de obstrucción, comparando con PCRE en 28 pacientes y 2 con cirugía. En ambos casos radiólogos y endoscopistas desconocían el resultado de sus respectivas exploraciones. Se calculó sensibilidad, especificidad y exactitud diagnóstica utilizando como estándar de referencia los resultados de la PCRE y la cirugía. En el 100 por ciento de los casos de los pacientes con CRMN, evaluamos completamente las vías biliares, en un 20 por ciento de los pacientes con PCRE no pudo obtenerse un colangiograma completo. En 4 pacientes (13.33 por ciento), ambos métodos fueron complementarios. Identificamos el nivel de obstrucción obtuvimos una S: 63,33 por ciento, E: 100 por ciento y una exactitud diagnóstica de 63,33 por ciento. la CRMN es un método no invasivo que permite definir el nivel y la anatomía del árbol biliar obstruido, debe considerarse como método complementario en pacientes con obstrucción completa del conducta biliar común y en aquellos casos de PCRE técnica y anatómicamente difícil

Colestasis del embrazo. Presentación de un caso / Cholestasis and pregnancy. Presentation of one case

Presentamos un caso de colestasis severa y embarazo que evoluciona asociado en el puerperio, a ovarios con quistes tecaluteínicos y hemoperitoneo por lo que requirió manejo quirúrgico. Consideramos que es un caso muy poco común, de aquí el interés de su presentación. Hacemos referencia a su posible patogenia, cuadro clínico y manejo del mismo

[Endoscopic retrograde cholangiopancreatography and sphincterotomy in parasitic diseases]. / Pancreatocolangiografía retrógrada endoscópica y esfinterotomía en las parasitosis.

We report six patients with billary duct obstruction due to parasites (Fasciola hepatica, ascariasis and hydatid cyst) that were diagnosed and managed with the endoscopic approach. This is the first national paper which highlights the importance of endoscopic retrograde cholangiopancreatography in the management of this parasitosis. Despite choledocholithiasis as the most common cause of extrahepatic jaundice, biliary tree parasitosis must be considered in the differential diagnosis.

Estenosis congénitas e idiopáticas de las vías biliares / Idiopatic and congenital biliary tract stenosis

Desde 1960 hasta 1992 se recopilaron 17 casos de estenosis de las vías biliares. De ellas 13 son consideradas idiopáticas y 4 congénitas. La edad osciló desde los 16 hasta los 47 años con un promedio de 26. La localización fue en convergencia superior 1, hepático derecho y sus ramas 5, hepático izquierdo 7, hepático común 1, convergencia cístico-coledociana 1, y colédoco intrapancreático 2. Se practicó una anastomosis biliodigestiva, 2 calibradas intraoperatoriamente y 12 calibradas y dilatads postoperatoriamente con sondas ubicadas en forma prolongada ( 15 días a 2 meses). Se trataron 2 pacientes con estenosis en ramas terciarias. Las dilataciones fueron generalmente transfisturales, siendo en un caso realizadas por vía transparietohepática. Este método dio buen resultado en todos los casos aplicados, por lo que se considera de elección

Estenosis congénitas e idiopáticas de las vías biliares / Idiopatic and congenital biliary tract stenosis

Desde 1960 hasta 1992 se recopilaron 17 casos de estenosis de las vías biliares. De ellas 13 son consideradas idiopáticas y 4 congénitas. La edad osciló desde los 16 hasta los 47 años con un promedio de 26. La localización fue en convergencia superior 1, hepático derecho y sus ramas 5, hepático izquierdo 7, hepático común 1, convergencia cístico-coledociana 1, y colédoco intrapancreático 2. Se practicó una anastomosis biliodigestiva, 2 calibradas intraoperatoriamente y 12 calibradas y dilatads postoperatoriamente con sondas ubicadas en forma prolongada ( 15 días a 2 meses). Se trataron 2 pacientes con estenosis en ramas terciarias. Las dilataciones fueron generalmente transfisturales, siendo en un caso realizadas por vía transparietohepática. Este método dio buen resultado en todos los casos aplicados, por lo que se considera de elección

Nova tecnica de derivacao colangiodigestiva intra-hepatica no lobo direito do figado / New technique of intra-hepatic cholangiojejunostomy derivation in the right lobe of the liver

Nas situacoes de obstrucao ao nivel do hilo hepatico tornando impossivel a realizacao de derivacao colangiodigestiva extra-hepatica esta indicada a realizacao de anastomoses intra-hepaticas. As tecnicas atualmente mais utilizadas sao executadas em ductos esquerdos. Ha situacoes, no entanto, onde as derivacoes colangiodigestivas a direita sao necessarias. As tecnicas atualmente disponiveis sao de dificil realizacao. A tecnica proposta tem como principio basico a realizacao de pequena resseccao hepatica a direita, realizacao de anastomose colangiodigestiva latero-lateral apos incisao longitudinal do ducto. Foram operados cinco doentes com tres fistulas no pos-operatorio imediato que fecharam espontaneamente. Um doente com lesao benigna (trauma hepatico) esta anicterico tres anos apos a intervencao.