An uncommon case of right hepatic duct entering cystic duct associated to multiple complications of biliary tract disease: acute pancreatitis, hepatolithiasis, acute cholecystitis and hepatic abscess.

Anatomical variations of the biliary tree pose diagnostic and treatment challenges. While most are harmless and often discovered incidentally during procedures, some can lead to clinical issues and biliary complications, making knowledge of these variants crucial to prevent surgical mishaps. Here, we present an unusual and clinically significant case. A 61-year-old man is admitted to the hospital with epigastric pain and diagnosis of pancreatitis of biliary origin and intermediate risk of choledocholithiasis. Magnetic resonance cholangiopancreatography (MRCP) reported hepatolithiasis and choledocholithiasis, whereas endoscopic retrograde cholangiopancreatography showed cystic drain of the right hepatic duct. One month later the patient presented again to the emergency room with increasing abdominal pain and a computed tomography that demonstrated the presence of hepatic abscess and acute cholecystitis. The patient underwent percutaneous drain abscess and a subtotal laparoscopic cholecystectomy. Biliary anatomical variants present challenges on the diagnostic investigations, interventional and surgical procedures, understanding the possible complications is essential.

Neoplasia papilar intraductal de la vía biliar / Intraductal papillary neoplasm of the bile duct / Neoplasia papilar intraductal da via biliar

La neoplasia papilar intraductal de la vía biliar (NPIVB) es una entidad infrecuente caracterizada por el crecimiento exofítico papilar del epitelio biliar hacia la luz ductal. Previamente incluida en el grupo de tumores del mismo nombre de localización pancreática, presenta diferencias evidentes con ellos y desde 2010 se considera una entidad propia con demostrado potencial de malignización hacia colangiocarcinoma.

Papillary intraductal neoplasia of the bile duct (NPIVB) is a rare entity characterized by exophytic papillary growth of the biliary epithelium towards the ductal lumen. Previously included in the group of tumors of the same name in pancreatic location, it presents obvious differences with them and since 2010 it has been considered a separate entity with demonstrated potential for malignancy towards cholangiocarcinoma.

O neoplasma papilar intraductal da via biliar (NPIVB) é uma entidade infrequente por el creciento exofítico papilar do epitélio biliar hacia la luz ductal. Obviamente incluído no grupo de tumores do mismo nombre de localização pancreática, apresenta diferenças evidentes com ellos e desde 2010 se considerar uma entidade propia com potencial demonstrado de malignización hacia cholangiocarcinoma.

Caso clínico radiológico de colangitis piógena recurrente / Radiological clinical case of recurrent pyogenic cholangitis

RESUMEN Se presenta paciente de 28 años, bangladeshi, ingresado en el Cuban Hospital de Qatar por dolor abdominal, fiebre moderada e ictericia ligera, elevación de las enzimas hepáticas y pancreáticas, además de presentar patrón humoral de colestasis. La ecografía abdominal y la tomografía de abdomen con contraste endovenoso detectaron vesícula biliar distendida con pared gruesa, litiasis en las vías biliares extra e intrahepáticas con dilatación de las mismas y escasa cantidad de líquido y edema peri pancreático, concluyéndose como pancreatitis aguda secundaria a colecistitis litiásica. Se realizó una colangiopancreatografia retrógrada endoscópica, y se comprobaron estos hallazgos y estenosis del conducto hepático derecho asociado a no permeabilidad del conducto hepático izquierdo. El diagnóstico cambió a colangitis piógena oriental. Por esta vía se extrajeron los cálculos y se colocó stent. El paciente fue tratado complementariamente con antibióticos, piperacilina y tazobactam vía IV, egresado con evolución satisfactoria después de nueve días del ingreso. Actualmente se encuentra en seguimiento por equipo multidisciplinario para definir conducta quirúrgica definitiva.

ABSTRACT A 28-year-old Bangladeshi patient is presented, admitted to the Cuban Hospital in Qatar for:abdominal pain, moderate fever and mild jaundice, elevated liver and pancreatic enzymes, as well as a humoral pattern of cholestasis. Abdominal ultrasound and abdominal tomography with intravenous contrast detected a distended gallbladder with a thick wall, lithiasis in the extra and intrahapatic bile ducts with dilatation of the same and little amount of fluid and peri-pancreatic edema, concluding as acute pancreatitis secondary to lithiasic cholecystitis. An endoscopic retrograde cholangiopancreatography was performed, verifying these findings and stenosis of the right hepatic duct associated with non-patency of the left hepatic duct, the diagnosis changed to oriental pyogenic cholangitis. By this route, the stones were extracted and a stent was placed. The patient was treated with complementary antibiotics, piperazine and IV tazobactam, and was discharged with satisfactory evolution nine days after admission. He is now being followed by a multidisciplinary team to define definitive surgical conduct.

Hepatolitiasis izquierda manejada con hepatectomía: Reporte de un caso

La hepatolitiasis es un desorden de las vías biliares caracterizado por la presencia de cálculos en los conductos biliares intrahepáticos. Existen varios factores de riesgo asociados a esta patología, pero su complicación más temida es el desarrollo de un colangiocarcinoma. En el Ecuador no hay literatura sobre el manejo de esta patología y su pronóstico postoperatorio. Se presenta el caso de un paciente masculino de 50 años de edad, con un cuadro de dolor abdominal en epigastrio, acompañado de hiporexia y náusea, sin hallazgos relevantes en el examen físico. Se encontraron pruebas de función hepática alteradas y marcadores tumorales elevados, además, los estudios radiológicos evidenciaron dilatación de la vía biliar intrahepática, con litos en su interior. Se decidió realizar una hepatectomía izquierda y, posteriormente, el estudio histopatológico evidenció cambios sugestivos de displasia en los conductos intrahepáticos. El paciente es dado de alta con una evolución dentro de los parámetros esperados y es remitido a seguimiento por Consulta Externa. En conclusión, la hepatolitiasis es una condición que al no ser tratada puede desarrollar un colangiocarcinoma, aumentando el riesgo del paciente

Hepatolithiasis is a bile duct disorder characterized by the presence of stones in the intrahepatic bile ducts1. There are several risk factors associated with this pathology, but its most feared complication is the development of cholangiocarcinoma2. In Ecuador there is not published literature regarding the management and prognosis of this pathology. A 50-year-old male patient with a picture of abdominal pain in the epigastrium, accompanied by hyporexia and nausea, without relevant findings on the physical exam. Altered liver function tests and raised tumor markers were found, in addition, radiological studies showed dilation of the intrahepatic bile duct, with bile stones inside. A left hepatectomy was performed, and subsequently the histopathological study demonstrated suggestive dysplasia changes in the intrahepatic ducts. Patient is discharged with a positive evolution and is referred to clinic for further follow up. To sum up, hepatolithiasis is a condition that should promptly be treated to avoid the development of a cholangiocarcinoma, which could put in risk the patient's life.

Liver resection for non-oriental hepatolithiasis / Ressecção hepática no tratamento da hepatolitíase não oriental

ABSTRACT Background: Primary intrahepatic lithiasis is defined when the stones are formed in the liver and associated with local dilatation and biliary stricture. Liver resection is the ideal procedure. Aim: To evaluate the results of liver resection in the treatment of non-oriental intrahepatic lithiasis. Methods: Fifty-one patients with symptomatic benign non-oriental hepatolithiasis underwent surgical resection in six institutions in Brazil. Demography data, clinical symptoms, classification, diagnosis, management and postoperative course were analyzed. Results: Of the 51 patients, 28 were male (54.9%), with a mean age of 49.3 years. History of cholangitis was observed in 15 (29.4%). The types of intrahepatic lithiasis were type I in 39 (76.5%) and type IIb in 12 (23.5%), with additional type Ea in six (11.8%). Liver function test were normal in 42 patients (82.4%). Segmental atrophy was observed in 12 (23.5%). Treatments included left lateral sectionectomy in 24 (47.1%), left hepatectomy in 14 (27.5%) and right hepatectomy in eight (15.7%), with associated hepaticojejunostomy in four (7.8%). Laparoscopic liver resection was performed in eight (15.7%). Postoperative complications were observed in 20 (39.2%) with no mortality. Conclusion: Liver resection in patients with hepatolithiasis is the ideal procedure as it removes stones, stricture, atrophic parenchyma, and minimizes the risk of cholangiocarcinoma.

RESUMO Racional: Litíase intra-hepática primária é definida quando os cálculos são formados dentro do fígado, podendo estar associada à dilatação local e estenosa da via biliar. A ressecção hepática é considerada o procedimento ideal. Objetivo: Avaliar os resultados da ressecção hepática no tratamento da litíase intra-hepática não oriental. Métodos: Cinquenta e um pacientes com hepatolitíase benigna não oriental sintomática foram submetidos à ressecção hepática em seis instituições no Brazil. Os dados demográficos, sintomas clínicos, classificação, diagnóstico, tratamento e evolução pós-operatória foram analisados. Resultados: Dos 51 pacientes havia 28 homens (54,9%), e a idade média era de 49,3 anos. História de colangite foi observada em 15 pacientes (29,4%). Os tipos de litíase observados foram tipo I em 39 (76,5%) e tipo IIb em 12 (23,5%), com o tipo adicional Ea em seis pacientes (11,8%). Os testes de função hepática estavam normais em 42 (82,4%) e atrofia segmentar foi observada em 12 (23,5%). O tratamento incluiu setorectomia lateral esquerda em 24 (47,1%), hepatectomia esquerda em 14 (27,5%) e hepatectomia direita em oito pacientes (15,7%). A hepaticojejunostomia esteve associada ao procedimento inicial em quatro (7,8%). Hepatectomia por videolaparoscopia foi realizada em oito (15,7%). Complicações pós-operatórias foram observadas em 20 pacientes (39,2%) e não houve mortalidade. Conclusão: Ressecção hepática na hepatolitíase é o procedimento ideal, pois remove os cálculos, a estenose, o parênquima atrofiado e minimiza os riscos para colangiocarcinoma.

La autopsia de Santander: el primer caso autóctono de hepatocolecistocoledocolitiasis / Santander's autopsy: first autochthonous case of hepatolithiasis / Autópsia de Santander: primeiro caso autóctone de hepatolithiasis

RESUMEN El informe de la autopsia de Santander, efectuada en 1840, ha sido publicado sin corregir los errores de imprenta originando interpretaciones discutibles. Se corrige y se proponen diagnósticos anatómicos macroscópicos que se comentan a la luz de la fisiopatología.

SUMMARY Santander's autopsy report, dated in 1840, has been published without any regard for the list of errata and controversial interpretations have been made. The necessary corrections are made and gross anatomic diagnoses are proposed along with a comment.

RESUMO Relatório da autópsia do Santander, datada em 1840, tem-se publicado sem qualquer consideração para a lista de errata, e interpretações controversas foram feitas. As correções necessárias são feitas e diagnósticos anatômicas brutas são, juntamente com um comentário proposto.

Hepatolitiasis a propósito de un caso / Hepatolithiasis: with regard to a case

Introducción: La hepatolitiasis es endémica de las regiones del sudeste asiático con una prevalencia que puede llegar hasta el 50%, pero es rara en el mundo occidental. El diagnóstico temprano y una detallada investigación son imprescindibles para el manejo adecuado, que es todo un reto debido a la alta tasa de recurrencia y complicaciones. Caso: Paciente femenina de 24 años de edad sin antecedentes patológicos personales que ingresa a por un cuadro de ictericia de siete meses de evolución. Dolor a nivel de epigastrio e hipocondrio derecho, intermitente. Siete días antes del ingreso inicia un cuadro de coluria y luego cefalea holocraneana. Los exámenes de laboratorio muestran hiperbilirrubinemia a expensas de las dos bilirrubinas (Directa 8.82 mgdl y BI: 6.16 mg/dl), enzimas hepáticas elevadas (AST (SGOT) 138 mg/dl y ALT (SGPT): 116 mg/dl; Fosfatasa-Alkalina: 997 y GGT: 226 mg/dl. Discusión: Las opciones terapéuticas están encaminadas a erradicar o disminuir los factores predisponentes para el desarrollo de colangiocarcinoma incluyéndose en este la recurrencia de la hepatolitiasis. Pese a que el enfoque no quirúrgico es una herramienta diagnóstica y terapéutica no ha demostrado ventajas de su aplicación.

Introduction: Hepatolithiasisis is endemic in regions like Southeast Asia with a prevalence that may reach 50%, but in the Western world it is rare. The proper management requires a precise research and early diagnosis. This is a real challenge due to the high rate of recurrence and complications. Case report: A twenty-four year-old female patient, was admitted witn a six months history of jaundice. She complained of epigastric and upper right quadrant intermitent pain. A week before admission she presented coluria and headache. Lab-tests were the fololowing: Conjugated bilirrubin 8.82 mg/dl, unconjugated bilirrubin 6.16 mg/dl, AST 138mg/dl, ALT 116 mg/dl, alkaline phosphatase 997mg/dl and GGT 226 mg/dl. Discusion: Therapeutic alternatives are focused to erradicate predisposing risk factors of liver carcninoma, one of the is recurrent hepatolitiasis. Though, the surgical approach is a diangostic and therapeutic tool, there is no evidence of its advantages.

Evaluación del manejo interdisciplinario de una serie de 53 pacientes con hepatolitiasis / Evaluation of interdisciplinary care of a series of 53 patients with hepatholithiasis

Objetivos: Evaluar, en una serie de enfermos con hepatolitiasis, el resultado del manejo interdisciplinario. Pacientes y métodos: Durante 8 años se valoraron, en un estudio retrospectivo, 53 pacientes con diagnóstico de hepatolitiasis. Resultados: Se estudiaron 23 hombres (43%) y 30 mujeres con una edad promedio de 50 ± 15 años (25 a 83 años). Predominó el dolor (94%), seguido de ictericia (68%) y fiebre (57%). Según la clasificación de Tsunoda, 6 pacientes eran Tsunoda I, 12 Tsunoda II, 4 Tsunoda III y 5 Tsunoda IV. El compromiso más frecuente fue del lóbulo izquierdo (36%) o bilateral (34%). La colangiografía endoscópica tuvo éxito en el 64% y solo se manejaron 4 pacientes por la vía transparietohepática (2 éxitos). Se operaron 35 pacientes (66%) y lo más frecuente fue la hepatectomía izquierda en 40% (7 con asa subcutánea) seguida por hepaticoyeyunostomía con asa subcutánea (26%). Se realizaron cuatro trasplantes ortotópicos de hígado que evolucionan asintomáticos en pacientes con complicaciones por cirrosis. Las complicaciones postoperatorias tempranas fueron del 40%, la más frecuente la infección del sitio operatorio (14%) y cálculos residuales (9%). La complicación tardía más usual fue la litiasis residual (23%). El 80% de los pacientes operados están asintomáticos comparados con el 72% de los no operados. No hubo mortalidad quirúrgica. Conclusiones: La hepatolitiasis es una entidad con manejos no bien estandarizados, entre ellos, la opción quirúrgica cursa con buenos resultados, mejoría clínica y poca morbimortalidad; con el apoyo interdisciplinario dado la radiología intervencionista y la endoscopia biliar.

Objectives: Evaluate the results of interdisciplinary care in a series of patients with hepatolithiasis. Patients and Methods: A retrospective study of 53 patients with hepatolithiasis who were evaluated over an eight year period. Results: 23 men (43%) and 30 women were studied. The average age was 50 ± 15 years (range: 25-83 years). Pain was the predominant symptom (94%), followed by jaundice (68%) and fever (57%). According to the Tsunoda classification, 6 patients were Tsunoda I, 12 were Tsunoda II, 4 were Tsunoda III, and 5 were Tsunoda IV. Left lobe hepatolithiasis was most frequent (36%), followed by bilateral hepatolithiasis (34%). Endoscopic cholangiography was successful in 64% of patients. Only 4 patients were treated by percutaneous cholangiography, with 2 successes. 35 patients (66%) were operated on. The most frequent surgery was left hepatectomy with subcutaneous loop in 7 patients (40%), followed by hepatojejunostomy with subcutaneous loop (26%). Four orthotopic liver transplantations with good evolution were performed in patients with cirrhotic complications. 40% had early complications. Most frequent were infection of the operative site (14%) and residual stones (9%). The most frequent delayed complication was residual lithiasis (23%). 80% of the patients who underwent surgery were asymptomatic compared with 72% of those who did not undergo surgery. There were no mortalities resulting from surgery. Conclusions: Hepatolithiasis is a disease which does not have a well standardized treatment. Surgery is an alternative with good results, clinical improvement and low morbidity and mortality rates when there is interdisciplinary including interventionist radiology and biliary endoscopy.

Colangioscopia intraoperatoria en el manejo de cálculos biliares intrahepáticos / Intraoperative cholangioscopy management of intrahepatic gallstones

La incidencia de los cálculos intrahepáticos en las sociedades occidentales es muy baja, sin embargo, la presencia de estos es todo un reto, ya que el manejo con colangiopancreatografía retrógrada endoscópica, es efectivo sólo en un pequeño porcentaje de los pacientes. Las opciones quirúrgicas son muy variables. El objetivo de este trabajo es describir un caso clínico de litiasis biliar intrahepática resuelto con el uso de colangioscopia intraoperatoria mediante coledocotomía y extracción de cálculos bajo visión directa con canastillas helicoidales, la cual es considerada como una de las mejores opciones quirúrgicas

The incidence of intrahepatic lithiasis in western societys is low, however, the diagnosis of this entity is really a challenge. The endoscopic management using retrograde cholangiopancreatography is effective in a low percentage of the patients. There are many surgical options, the purpose of this paper is to describe a case of intrahepathic lithiasis treated using intraoperative cholangioscopic through choledochotomy and stones removal under direct vision with helicoidal baskets, as one of the best surgical option in this cases