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1.
Case Rep Surg ; 2022: 9673901, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35677852

RESUMEN

Introduction: Over the past few years, liver surgery has been in constant evolution and gained many improvements that helped surgeons push limits further. A complex procedure such as left extended trisectionectomy, as described by Makuuchi in 1987, may be performed in selected cases. Aim: Describe a case of successful resection of a huge bilobar liver sarcoma involving all hepatic veins from a young female patient, in which the blood outflow was preserved through an inferior right hepatic vein, leaving only segment 6 as liver remnant. Case Report. A 19-year-old female with a 3-month history of abdominal pain, vomiting, and weight loss was referred for our evaluation. CT scan and MRI revealed a heterogeneous and bulky expansive hepatic lesion, sparing only segment 6, with an estimated volume of 530 cm3, corresponding to a 1.2 FLR/BW ratio. The tumor involved the three major hepatic veins, but an inferior right hepatic vein was present, draining the spared segment 6. She was submitted to a left trisectionectomy extended to the caudate lobe and segment 7, including resection of all hepatic veins and lymphadenectomy of the hepatic pedicle. She was discharged on the 7th postoperative day without complications. The histopathological and immunohistochemical analysis demonstrated an undifferentiated embryonal sarcoma of the liver. Conclusion: Inferior right hepatic vein-preserving left extended trisectionectomy is a safe and feasible procedure that should be performed by a hepatobiliary team experienced in major complex hepatectomies.

2.
Arq Bras Cir Dig ; 35: e1647, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35730876

RESUMEN

AIM: Colorectal cancer generally metastasizes to the liver. Surgical resection of liver metastasis, which is associated with systemic chemotherapy, is potentially curative, but many patients will present recurrence. In selected patients, repeated hepatectomy is feasible and improves overall survival. This study aimed to analyze patients with colorectal liver metastasis (CRLM) submitted to hepatectomy in three centers from Rio de Janeiro, over the past 10 years, by comparing the morbidity of first hepatectomy and re-hepatectomy. METHODS: From June 2009 to July 2020, 192 patients with CRLM underwent liver resection with curative intent in three hospitals from Rio de Janeiro Federal Health System. The data from patients, surgeries, and outcomes were collected from a prospectively maintained database. Patients submitted to first and re-hepatectomies were classified as Group 1 and Group 2, respectively. Data from groups were compared and value of p<0.05 was considered significant. RESULTS: Among 192 patients, 16 were excluded. Of the remaining 176 patients, 148 were included in Group 1 and 28 were included in Group 2. Fifty-five (37.2%) patients in Group 1 and 13 (46.5%) in Group 2 presented postoperative complications. Comparing Groups 1 and 2, we found no statistical difference between the cases of postoperative complications (p=0.834), number of minor (p=0.266) or major (p=0.695) complications, and deaths (p=0.407). CONCLUSIONS: No differences were recorded in morbidity or mortality between patients submitted to first and re-hepatectomies for CRLM, which reinforces that re-hepatectomy can be performed with outcomes comparable to first hepatectomy.


Asunto(s)
Neoplasias Colorrectales , Neoplasias Hepáticas , Brasil , Neoplasias Colorrectales/patología , Neoplasias Colorrectales/cirugía , Hepatectomía , Humanos , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/cirugía , Morbilidad , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos
5.
Pediatr Transplant ; 26(4): e14242, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35122453

RESUMEN

BACKGROUND: Perioperative pain management in small infants weighing <10 kg undergoing liver transplantation is challenging. The use of TEA in this setting has not been reported, as well as its potential role to facilitate IE, ie, in the OR. METHODS: We describe here the use of TEA in two small infants who had IE after a LDLT procedure. RESULTS: TEA was successfully performed and IE was achieved in both cases. Postoperative analgesia assessment in the OR was satisfactory according to the FLACC pain scale, with scores of 2 and 3 for each patient, respectively. There were no major complications in the postoperative period, and the two children were discharged home uneventfully. CONCLUSIONS: The use of TEA and its influence on IE rate and other perioperative outcomes should be more explored in small infants undergoing LDLT.


Asunto(s)
Analgesia Epidural , Trasplante de Hígado , Extubación Traqueal , Analgesia Epidural/métodos , Niño , Humanos , Lactante , Trasplante de Hígado/métodos , Donadores Vivos , Dimensión del Dolor
6.
ABCD (São Paulo, Online) ; 35: e1647, 2022. tab
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1383221

RESUMEN

ABSTRACT - BACKGROUND: Colorectal cancer generally metastasizes to the liver. Surgical resection of liver metastasis, which is associated with systemic chemotherapy, is potentially curative, but many patients will present recurrence. In selected patients, repeated hepatectomy is feasible and improves overall survival. AIM: This study aimed to analyze patients with colorectal liver metastasis (CRLM) submitted to hepatectomy in three centers from Rio de Janeiro, over the past 10 years, by comparing the morbidity of first hepatectomy and re-hepatectomy. METHODS: From June 2009 to July 2020, 192 patients with CRLM underwent liver resection with curative intent in three hospitals from Rio de Janeiro Federal Health System. The data from patients, surgeries, and outcomes were collected from a prospectively maintained database. Patients submitted to first and re-hepatectomies were classified as Group 1 and Group 2, respectively. Data from groups were compared and value of p<0.05 was considered significant. RESULTS: Among 192 patients, 16 were excluded. Of the remaining 176 patients, 148 were included in Group 1 and 28 were included in Group 2. Fifty-five (37.2%) patients in Group 1 and 13 (46.5%) in Group 2 presented postoperative complications. Comparing Groups 1 and 2, we found no statistical difference between the cases of postoperative complications (p=0.834), number of minor (p=0.266) or major (p=0.695) complications, and deaths (p=0.407). CONCLUSIONS: No differences were recorded in morbidity or mortality between patients submitted to first and re-hepatectomies for CRLM, which reinforces that re-hepatectomy can be performed with outcomes comparable to first hepatectomy.


RESUMO - RACIONAL: O câncer colorretal geralmente metastatiza para o fígado. Hepatectomia associada à quimioterapia sistêmica é potencialmente curativa para metástases hepáticas colorretais, entretanto, muitos pacientes apresentarão recidiva após a cirurgia. Em casos selecionados, a re-hepatectomia é viável, com relatos de melhora na sobrevida global. OBJETIVO: Analisar pacientes com metástase hepática colorretal operados em três centros do Rio de Janeiro, nos últimos 10 anos, comparando as morbidades da primeira hepatectomia e da re-hepectomia. MÉTODOS: De junho de 2009 a julho de 2020, 192 pacientes com metástase hepática colorretal foram submetidos à hepatectomia em três hospitais do Rio de Janeiro. Os dados dos pacientes, cirurgias e desfechos foram coletados de um banco de dados mantido prospectivamente. Pacientes submetidos à primeira hepatectomia e re-hepatectomia foram classificados como Grupo 1 e Grupo 2, respectivamente. Os dados dos grupos foram comparados e o valor de p<0,05 foi considerado significativo. RESULTADOS: Dentre 192 pacientes, dezesseis foram excluídos. Dos 176 pacientes restantes, 148 e 28 foram incluídos dos Grupos 1 e 2, respectivamente. Cinquenta e cinco (37,2%) pacientes no Grupo 1 e treze (46,5%) no Grupo 2 apresentaram complicações pós-operatórias. Comparando os Grupos 1 e 2, não foi observada diferença estatística entre o número de pacientes com complicações pós-operatórias (p = 0,834), complicações menores (p = 0,266) ou maiores (p = 0,695) e óbitos (p = 0,407). CONCLUSÕES: Não foram registradas diferenças na morbidade ou mortalidade entre os pacientes submetidos à primeira ou à re-hepatectomia em pacientes com metástase hepática colorretal, o que sustenta que a re-hepatectomia pode ser realizada com resultados comparáveis à primeira hepatectomia.

7.
Arq Bras Cir Dig ; 33(4): e1555, 2021.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-33503115

RESUMEN

BACKGROUND: Tourniquet for right hepatectomy tightened and secured with forceps (arrow). Laparoscopic liver resection is performed worldwide. Hemorrhage is a major complication and bleeding control during hepatotomy is an important concern. Pringle maneuver remains the standard inflow occlusion technique. AIM: Describe an extracorporeal, efficient, fast, cheap and reproducible way to execute the Pringle maneuver in laparoscopic surgery, using a chest tube. METHODS: From January 2014 to March 2020, our team performed 398 hepatectomies, 63 by laparoscopy. We systematically encircle the hepatoduodenal ligament and prepare a tourniquet to perform Pringle maneuver. In laparoscopy, we use a 24 Fr chest tube, which is inserted in the abdominal cavity through a small incision. We thread the cotton tape through the tube, pulling it out through the external end, outside the abdomen. To perform the tourniquet, we just need to push the tube as we hold the tape, clamping both with one forceps. RESULTS: The 24 Fr chest tube is firm and works perfectly to occlude blood inflow as the cotton band is tightened. It has an internal diameter of 5,5 mm, sufficient for a laparoscopic grasper pass through it to catch the cotton band, and an external diameter of 8 mm, which allows to be inserted in the abdomen through a tiny incision. The cost of this tube and the cotton band is less than US$ 1. No complications related to the method were identified in our patients. CONCLUSIONS: The extracorporeal Pringle maneuver presented here is a safe, cheap and reproducible method, that can be used for bleeding control in laparoscopic liver surgery.


Asunto(s)
Cavidad Abdominal/diagnóstico por imagen , Hepatectomía/métodos , Laparoscopía/métodos , Neoplasias Hepáticas/cirugía , Constricción , Humanos
9.
Ann Hepatobiliary Pancreat Surg ; 24(4): 522-525, 2020 Nov 30.
Artículo en Inglés | MEDLINE | ID: mdl-33234757

RESUMEN

Associated liver partition and portal vein ligation for staged hepatectomy - ALPPS - procedure emerged as an alternative to treat patients needing extensive hepatic resections, but with a small future liver remnant. Initially described using the left lateral segments as liver remnant, ALPPS has been adapted to leave as remainder only one segment. Describe a case of a patiente with bilobar colorectal liver metastasis submitted to segment 4-1 ALPPS. A 63-year-old man, previously submitted to transversostomy, due to a left colon stenosing adenocarcinoma, associated to bilobar liver metastasis, was referred for our evaluation, after receiving a FOLFOX based chemotherapy. Due to the large load of tumor within the liver, we opted to perform a segment 4-1 ALPPS, which was carried out with an interval of 21 days between first and second stages. The liver remnant increased from 250 cc to 694 cc (18% to 48% of standard liver volume). The patient was discharged 15 days after second stage surgery and was subjected to left colectomy after five months. He is disease-free ten months after liver surgery. Monosegment ALPPS is a challenging, but feasible procedure, that should be criteriously indicated in selected patients and performed by a hepatobiliary surgery team with experience in complex major hepatectomies.

10.
ABCD (São Paulo, Impr.) ; 33(4): e1555, 2020. graf
Artículo en Inglés | LILACS | ID: biblio-1152633

RESUMEN

ABSTRACT Background: Laparoscopic liver resection is performed worldwide. Hemorrhage is a major complication and bleeding control during hepatotomy is an important concern. Pringle maneuver remains the standard inflow occlusion technique. Aim: Describe an extracorporeal, efficient, fast, cheap and reproducible way to execute the Pringle maneuver in laparoscopic surgery, using a chest tube. Methods: From January 2014 to March 2020, our team performed 398 hepatectomies, 63 by laparoscopy. We systematically encircle the hepatoduodenal ligament and prepare a tourniquet to perform Pringle maneuver. In laparoscopy, we use a 24 Fr chest tube, which is inserted in the abdominal cavity through a small incision. We thread the cotton tape through the tube, pulling it out through the external end, outside the abdomen. To perform the tourniquet, we just need to push the tube as we hold the tape, clamping both with one forceps. Results: The 24 Fr chest tube is firm and works perfectly to occlude blood inflow as the cotton band is tightened. It has an internal diameter of 5,5 mm, sufficient for a laparoscopic grasper pass through it to catch the cotton band, and an external diameter of 8 mm, which allows to be inserted in the abdomen through a tiny incision. The cost of this tube and the cotton band is less than US$ 1. No complications related to the method were identified in our patients. Conclusions: The extracorporeal Pringle maneuver presented here is a safe, cheap and reproducible method, that can be used for bleeding control in laparoscopic liver surgery.


RESUMO Racional: Ressecções hepáticas laparoscópicas são realizadas em todo mundo. A hemorragia é complicação grave e o controle do sangramento durante a hepatotomia é preocupação importante. A manobra de Pringle continua sendo a técnica padrão de oclusão do influxo sanguíneo. Objetivo: Descrever uma maneira eficiente, rápida, barata e reproduzível de executar a manobra de Pringle extracorpórea, em operação laparoscópica, utilizando um dreno de tórax. Métodos: De janeiro/2014 a março/2020, realizamos 398 hepatectomias, 63 por laparoscopia. Nós sistematicamente laçamos o ligamento hepatoduodenal e preparamos um torniquete para a manobra de Pringle. Na laparoscopia, usamos um dreno de tórax 24 Fr, inserido na cavidade abdominal através de uma pequena incisão. Passamos a fita de algodão através do tubo, puxando-a pela extremidade externa, fora do abdome. Para apertar o torniquete, basta pressionar o tubo enquanto seguramos a fita, prendendo ambos com uma pinça. Resultados: O dreno de tórax 24 Fr é firme e funciona perfeitamente para ocluir influxo de sangue, à medida que apertamos o torniquete. Tem diâmetro interno de 5,5 mm, suficiente para passar uma pinça laparoscópica e puxar a fita de algodão, e um diâmetro externo de 8 mm, permitindo a inserção no abdome através de uma pequena incisão. O custo do tubo e fita é inferior a US$ 1, valor insignificante. Não foram identificadas complicações relacionadas ao método em nossos pacientes. Conclusões: A manobra extracorpórea de Pringle apresentada aqui é método seguro, barato e reproduzível, que pode ser utilizado para o controle do sangramento em hepatectomias laparoscópicas.


Asunto(s)
Humanos , Laparoscopía/métodos , Cavidad Abdominal/diagnóstico por imagen , Hepatectomía/métodos , Neoplasias Hepáticas/cirugía , Constricción
11.
Ann Hepatol ; 18(1): 258-262, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31113602

RESUMEN

Paracoccidioidomycosis is a systemic granulomatous disease caused by the dimorphic fungus Paracoccidioides brasiliensis and is restricted to Latin America. It normally affects lungs, skin and lymph nodes. Abdominal organs are usually not involved. In rare cases paracoccidioidomycosis may simulate neoplasm. Herein we describe our experience with four cases of paracoccidioidomycosis mimicking cholangiocarcinoma. To the best of our knowledge, this is the largest case series on this subject produced in English. Paracoccidioidomycosis must be considered as a differential diagnosis of cholangiocarcinoma, especially in individuals who come from endemic areas.


Asunto(s)
Enfermedades de los Conductos Biliares/diagnóstico , Neoplasias de los Conductos Biliares/diagnóstico , Colangiocarcinoma/diagnóstico , Paracoccidioides/aislamiento & purificación , Paracoccidioidomicosis/diagnóstico , Antifúngicos/uso terapéutico , Enfermedades de los Conductos Biliares/microbiología , Enfermedades de los Conductos Biliares/terapia , Biopsia , Colangiografía , Colecistectomía , Diagnóstico Diferencial , Femenino , Hepatectomía , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Paracoccidioidomicosis/microbiología , Paracoccidioidomicosis/terapia , Adulto Joven
12.
HPB (Oxford) ; 19(12): 1126-1129, 2017 12.
Artículo en Inglés | MEDLINE | ID: mdl-28917644

RESUMEN

BACKGROUND: A minimum future liver remnant (FLR) of 30% is required to avoid post hepatectomy liver failure (PHLF). Portal vein occlusion (PVO) is the main strategy to induce hypertrophy of the FLR, but some patients will not reach sufficient FLR hypertrophy to enable resection. Recently ALPPS has emerged as a "Salvage Procedure" for PVO failure. The aim of this study was to report the short term outcomes of ALPPS following PVO failure. METHODS: A retrospective analysis of patients enrolled within the international ALPPS Registry between October 2012 and November 2015 (NCT01924741) was performed. Patients with documented PVO failure were included. The outcomes reported included feasibility, FLR growth rate and safety of ALPPS. Complications were recorded as per Clavien-Dindo classification. RESULTS: From 510 patients enrolled in the Registry there were 22 patients with previous PVO failure. Two patients were excluded due to missing data and twenty patients were analysed. All of them completed the proposed ALPPS with a medium FLR increase of 88% (23-115%) between two stages and no 90-day mortality. CONCLUSION: In experienced centers, ALPPS following PVO failure is feasible and safe. The FLR hypertrophy was similar to other ALPPS series. ALPPS is a potential rescue strategy after PVO failure.


Asunto(s)
Embolización Terapéutica , Hepatectomía/métodos , Regeneración Hepática , Hígado/cirugía , Vena Porta/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Proliferación Celular , Embolización Terapéutica/efectos adversos , Femenino , Hepatectomía/efectos adversos , Humanos , Ligadura , Hígado/patología , Masculino , Persona de Mediana Edad , Sistema de Registros , Estudios Retrospectivos , Terapia Recuperativa , Factores de Tiempo , Insuficiencia del Tratamiento
13.
Ann Hepatol ; 16(5): 814-817, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28809736

RESUMEN

Associated liver partition and portal vein ligation for staged hepatectomy (ALPPS) has emerged as an alternative for patients with bilobar colorectal liver metastasis and a small future liver remnant (FLR). In cases of extensive disease, ALPPS can be performed, leaving only one segment of the liver as FLR. We describe a case of monosegmental ALPPS using segment 4 as FLR. In conclusion, ALPPS should be reserved for a selected group of patients. Monosegmental ALPPS is feasible, but should be performed by hepatobiliary surgeons in specialized centers.


Asunto(s)
Adenocarcinoma/cirugía , Neoplasias del Colon/patología , Hepatectomía/métodos , Neoplasias Hepáticas/cirugía , Metastasectomía/métodos , Vena Porta/cirugía , Adenocarcinoma/secundario , Adulto , Quimioterapia Adyuvante , Femenino , Humanos , Ligadura , Neoplasias Hepáticas/secundario , Terapia Neoadyuvante , Factores de Tiempo , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Carga Tumoral
14.
Case Reports Hepatol ; 2014: 616251, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25478255

RESUMEN

Background. An adequate blood flow is directly related to graft survival in living donor liver transplantation. However, in some cases, unfavorable conditions prevent the use of the hepatic artery for arterial reconstruction. Herein, we report a case in which the recipient right gastroepiploic artery was used as an option for arterial reconstruction in adult-to-adult living donor liver transplantation. Case Report. A 62-year-old woman, with cirrhosis due to hepatitis B associated with hepatocellular carcinoma, was submitted to living donor liver transplantation. During surgery, thrombosis of the hepatic artery with intimal dissection until the celiac trunk was observed, which precluded its use in arterial reconstruction. We decided to use the right gastroepiploic artery for arterial revascularization of the liver graft. Despite the discrepancy in size between donor hepatic artery and recipient right gastroepiploic artery, anastomosis was performed successfully. Conclusions. The use of the right gastroepiploic artery as an alternative for arterial revascularization of the liver graft in living donor liver transplantation should always be considered when the hepatic artery of the recipient cannot be used. For performing this type of procedure, familiarity with microsurgical techniques by the surgical team is necessary.

16.
Arq Bras Cir Dig ; 27(3): 204-5, 2014.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-25184773

RESUMEN

BACKGROUND: Portal vein embolization is an accepted procedure that provides hypertrophy of the future remnant liver in order to reduce post-hepatectomy complications. AIM: To present a series submitted to portal vein embolization using an adapted hysterosalpingography catheter via transileocolic route. METHODS: Were performed right portal branch embolization in 19 patients using hysterosalpingography catheter. For embolizing the vessel, was used Gelfoam(r) powder with absolute alcohol solution. Indications for hepatectomy were colorectal liver metastases in all cases. RESULTS: An adequate growth of the future remnant liver was achieved in 15 patients (78.9%) and second time hepatectomy could be done in 14 (73.7%). In one patient (5.2%), tumor progression prevented surgery. One patient presented acute renal failure after portal embolization. CONCLUSIONS: The hysterosalpingography catheter is easy to handle and can be introduced into the portal vein with a wire guide. There were no major post-embolization complication. Its use is safe, cheap and effective.


Asunto(s)
Embolización Terapéutica/instrumentación , Hepatectomía , Vena Porta , Catéteres , Embolización Terapéutica/métodos , Diseño de Equipo , Humanos , Histerosalpingografía/instrumentación
17.
ABCD (São Paulo, Impr.) ; 27(3): 204-205, Jul-Sep/2014. graf
Artículo en Inglés | LILACS | ID: lil-720393

RESUMEN

BACKGROUND: Portal vein embolization is an accepted procedure that provides hypertrophy of the future remnant liver in order to reduce post-hepatectomy complications. AIM: To present a series submitted to portal vein embolization using an adapted hysterosalpingography catheter via transileocolic route. METHODS: Were performed right portal branch embolization in 19 patients using hysterosalpingography catheter. For embolizing the vessel, was used Gelfoam(r) powder with absolute alcohol solution. Indications for hepatectomy were colorectal liver metastases in all cases. RESULTS: An adequate growth of the future remnant liver was achieved in 15 patients (78.9%) and second time hepatectomy could be done in 14 (73.7%). In one patient (5.2%), tumor progression prevented surgery. One patient presented acute renal failure after portal embolization. CONCLUSIONS: The hysterosalpingography catheter is easy to handle and can be introduced into the portal vein with a wire guide. There were no major post-embolization complication. Its use is safe, cheap and effective. .


RACIONAL: Embolização da veia porta é procedimento consagrado para estimular a hipertrofia do fígado remanescente, a fim de reduzir as complicações pós-hepatectomia. OBJETIVO: Apresentar série de casos submetidos à embolização da veia porta usando cateter adaptado de histerossalpingografia, por via transileocólica. MÉTODOS: Foi realizada embolização do ramo portal direito em 19 pacientes utilizando cateter de histerossalpingografia. Foi usado Gelfoam(r) em pó com solução de álcool absoluto, como material embolizante. As indicações para hepatectomia foram metástases hepáticas colorretais em todos os casos. RESULTADOS: Hipertrofia adequada do fígado remanescente foi alcançada em 15 pacientes (78,9%) e a hepatectomia foi realizada em 14 (73,7 %). Em um (5,2 %), a progressão do tumor impediu a realização da operação. Um paciente apresentou insuficiência renal aguda após embolização portal. CONCLUSÕES: O cateter de histerossalpingografia é fácil de ser manuseado e pode ser introduzido na veia porta com um fio guia. Não houve complicação grave pós-embolização. Seu uso é seguro, barato e eficaz. .


Asunto(s)
Humanos , Embolización Terapéutica/instrumentación , Hepatectomía , Vena Porta , Catéteres , Embolización Terapéutica/métodos , Diseño de Equipo , Histerosalpingografía/instrumentación
18.
Case Rep Surg ; 2013: 371264, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24024060

RESUMEN

Anatomical resection of segment VIII (SVIII) is one of the most difficult hepatectomies to perform. Although it is the best choice of surgical treatment for tumors located at SVIII, its feasibility can be compromised when the right hepatic vein (RHV) must be resected en bloc with SVIII. Herein we describe a case of a cirrhotic patient that was submitted to segmentectomy VIII in bloc with the main trunk of the RHV, due to hepatocellular carcinoma. The resection could only be performed because a well developed inferior right hepatic vein (IRHV) was present. Anatomical variations of the liver vascularization should be used by liver surgeons to improve surgical results.

19.
ISRN Surg ; 2011: 106487, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-22084740

RESUMEN

Caroli's disease (CD) is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts. Patients with bilobar or progressive disease may require orthotopic liver transplantation (OLT). In the MELD era, living donor liver transplantation (LDLT) raised as the ultimate treatment option for these patients, once their MELD score is usually low. Herein, we describe 2 cases of patients (a 2-year-old girl and a 19-year-old teenager) that successfully underwent LDLT as a treatment for diffuse CD. The good postoperative courses of the two cases indicate that LDLT is a feasible option in the treatment of this disorder, even in complicated or early age patients.

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