A preliminary study on predicting anastomotic occlusion in patients with cirrhosis underwent splenectomy combined with splenorenal shunt by ultrasonography / 中南大学学报(医学版)

To predict anastomotic occlusion after splenectomy combined with splenorenal shunt surgery by ultrasound technique.
 Methods: To retrospectively analyze 53 cases of splenectomy combined with splenorenal shunt surgery. We divided these patients into 2 groups: a patency group (n=39) and an occlusion group (n=14), which were based on the results of splenorenal venous anastomotic stoma with spiral CT. The statistical methods were used to analyze the ultrasound detection indicators (the internal diameter, blood flow velocity, blood flow volume, thrombosis and blood flow direction of portal vein, splenic vein, and superior mesenteric vein) for those 2 groups, and then to figure out the predictive factors that affect splenorenal venous anastomotic stoma.
 Results: Compared with the patency group, there are significant broadening of the portal vein diameter, narrowing of the splenic vein diameter, reduction of the splenic vein blood flow velocity, reduction of splenic venous flow volume, splenic vein thrombosis formation and changes of the splenic vein blood flow direction (all P<0.05).
 Conclusion: Ultrasound indicators of portal vein diameter broadening, splenic vein diameter narrowing, splenic vein blood flow velocity reduction, splenic venous flow volume reduction, splenic vein thrombosis formation and change of splenic vein blood flow direction are influential factors for the splenorenal anastomotic occlusion in patients after splenectomy combined with splenorenal shunt surgery.

Lessons Learned from Inappropriate Ligation of the Left Renal Vein for a Large Splenorenal Shunt in Living Donor Liver Transplantation / 대한이식학회지

During living donor liver transplantation, a large spontaneous splenorenal shunt (SRS) should be addressed to obtain adequate portal inflow. Various procedures such as direct ligation of the SRS, splenectomy, left renal vein ligation (LRVL), and renoportal anastomosis can be applied to treat a large SRS according to the hemodynamics of the portal flow and anatomic conditions. Of these surgical procedures, LRVL is a simple and effective solution for treatment of a large SRS. However, to perform a LRVL, rigorous evaluation of the recipient's anatomic and hemodynamic variations is mandatory. In the present case, we ligated the left renal vein to treat a large SRS, which resulted in an unexpected thrombosis of the left renal vein and remaining portal vein stenosis in the SRS. Therefore, we revised our decisions regarding whether the LRVL was properly applied.

Plug-Assisted Retrograde Transvenous Obliteration of Spontaneous Splenorenal Shunt for Refractory Hepatic Encephalopathy: Case Series

Intervention treatment such as balloon retrograde or anterograde transvenous obliteration has been used for management of refractory hepatic encephalopathy as well as gastric variceal bleeding. Recently, plug-assisted retrograde transvenous obliteration without a help of balloon was newly developed to treat these patients. Here, we report three cases suffering refractory hepatic encephalopathy who were treated with this new technique.

Renoportal Anastomosis in Living Donor Liver Transplantation; An Effective Technique for Patient with Diffuse Portal Vein Thrombosis and Large Splenorenal Shunts

End-stage liver disease is often accompanied by portal vein thrombosis (PVT) and large spontaneous splenorenal shunts (SRS). Recently, renoportal anastomosis (RP-A) of spontaneous splenorenal shunts in liver transplantation was reported as an effective method of portal vein reconstruction in cases of PVT with SRS. Here we report a successful case of RP-A in living donor liver transplantation (LDLT). A 46-year-old female with a large spontaneous splenorenal shunt and a portal vein thrombosis propagated to the superior mesenteric vein underwent living donor liver transplantation. At the operation, a side-to-end renoportal anastomosis was done using an interposing cadaveric iliac vein graft. Adequate portal venous blood flow was demonstrated by intraoperative and postoperative Doppler ultrasound studies. She has recovered well with normal graft function and renal function. Renoportal anastomosis for patients with large splenorenal shunts and expansive portal vein thrombosis to the superior mesenteric vein can be an effective and safe technique in patients PV thrombectomy.

The retrospective analysis of surgical outcome of portal hypertension / 中华外科杂志

<p><b>OBJECTIVE</b>To retrospectively analyze the surgical outcome of portal hypertension and explore the risk-factors of long-term survival after operation.</p><p><b>METHODS</b>The data of 149 patients (male 119, female 30, aged from 19 to 73 years old) with portal hypertension treated surgically from January 1996 to October 2007 was collected. Among these patients, there were 110 patients for Child A and 39 patients for Child B according to Child-Pugh classification. According to different surgical modality, all patients were divided into devascularization group (n = 85) and shunting group (n = 64).</p><p><b>RESULTS</b>The follow-up rate was 78.8% and the average follow-up time was (46.3 +/- 30.4) months. The overall survival rates of 1-, 3-, 5- and 10-years were 95.6%, 88.7%, 83.4% and 65.1% respectively. Meanwhile the survival rates of 1-, 3-, 5- and 10-years in devascularization group and in shunting group were 95.4%, 87.7%, 80.6%, 56.3% and 95.8%, 90.1%, 86.8%, 72.6% respectively. There was no significant difference in survival rate between these two groups (P > 0.05). Child-Pugh classification has been the most important risk-factor that could influence long-term survival after operation by analysis of COX regression and it showed that the long-term survival time in Child A was longer than in Child B. The re-hemorrhage rates of 1-, 3- and 5-years in shunting group would be much better than in devascularization group. The rate of postoperative encephalopathy in devascularization group and shunting group was 6.9% and 6.1% respectively and there was no significant difference (P > 0.05). The portal venous pressure and flow of portal vein decreased significantly after shunting operation (P < 0.05).</p><p><b>CONCLUSIONS</b>The mainly sole risk-factor of long-term survival for portal hypertension has been the classification of Child-Pugh, not surgical procedure. The individualized proximal splenorenal shunt is much better than devascularization in controlling variceal hemorrhage.</p>

Renal Hypoperfusion Associated with Splenorenal Shunts in Liver Cirrhosis

PURPOSE: To determine whether spontaneous a splenorenal shunt can be used as an imaging predictor of early renal hemodynamic changes in patients with cirrhosis. MATERIALS AND METHODS: The study included 82 cirrhotic patients and 41 control subjects. Three-phase CT was performed and CT attenuation values (Hounsfield units) of the renal cortex in three phases were measured to evaluate renal perfusion. Likelihood ratio tests for trend were conducted for age, presence of ascites, and Child's grade. RESULTS: The mean CT attenuation values of the renal cortex in cirrhotic patients were significantly lower than the values of control subjects in three phases: 153.3 +/- 37.9 versus 173.3 +/-25.2 in the arterial phase, 172.6 +/- 41.0 versus 197.6 +/- 26.5 in the portal phase and 136.9 +/- 26.0 versus 152.7 +/- 20.0 in the delayed phase, respectively. The mean CT attenuation value of cortices in patients with renal hypoperfusion was 119.9 +/- 11.8 in the portal phase. Child's class C (aOR: 58.4, 95% CI: 3.6-956.2; p < 0.01) and the presence of a renal shunt (aOR: 7.5, 95% CI: 1.8-30.5; p < 0.01) were associated with renal hypoperfusion. The incidence of renal hypoperfusion was associated with Child's grade (trend: p < 0.01), and not with the grade of ascites or age. CONCLUSION: A dilated spontaneous splenorenal shunt may be a risk factor for renal hypoperfusion in cirrhosis.

Living-Donor Liver Transplantation with Renoportal Anastomosis using an Interposition Polytetrafluoroethylene Graft for a Patient with Large Spontaneous Splenorenal Shunt: A Case Report / 대한이식학회지

Adequate portal perfusion is essential in liver transplantation. End-stage liver disease is often accompanied by a large spontaneous splenorenal shunt and poor portal flow. To secure an adequate portal perfusion of the graft, collaterals including splenorenal shunt should be interrupted during liver transplantation. However, this procedure is usually too demanding because of massive bleeding, as well as time-consuming. As in living-donor liver transplantation size-matched liver graft and vascular grafts are not always available, an alternative must be sought. We performed living-donor liver transplantation with renoportal anastomosis in a 52 year-old male with a large spontaneous splenorenal shunt. During surgery, left renal vein was divided at the caval junction and the distal stump was end-to-end anastomosed to the graft portal vein using 16 mm interposition polytetrafluoroethylene graft without ligation of collaterals. The initial postoperative course of this patient was uneventful. However, on postoperative day 6 and 12 perihepatic hematoma evacuation and portal vein graft thrombectomy were performed respectively. Since then, adequate portal blood flow and patency of the interposition polytetrafluoroethylene graft was maintained throughout the postoperative period. The patient was discharged with normal graft function 10 weeks after transplantation. Renoportal anastomosis using an interposition polytetrafluoroethylene graft in living-donor liver transplantation could be an acceptable alternative for patients with end-stage liver disease with a large spontaneous splenorenal shunt.

Influência do grau de insuficiência hepática e do índice de congestão portal na recidiva hemorrágica de cirróticos submetidos a cirurgia de Teixeira-Warren / Role of liver function and portal vein congestion index on rebleeding in cirrhotics after distal splenorenal shunt

RACIONAL: A hipertensão portal com sua principal complicação, a hemorragia digestiva alta varicosa, são importantes causas de morbimortalidade em cirróticos. A cirurgia de Teixeira-Warren é uma derivação portal seletiva, adotada em doentes Child-Pugh A e B para tratamento da hemorragia varicosa por hipertensão portal não responsiva à terapêutica clínico-endoscópica após o quadro agudo. O índice de congestão portal baseia-se em valores obtidos pela ultra-sonografia Doppler abdominal e encontra-se elevado em pacientes com hipertensão portal. OBJETIVO: Verificar se o índice de congestão portal e o grau de insuficiência hepática (Child-Pugh) são fatores preditivos de recidiva hemorrágica após a cirurgia de Teixeira-Warren. MÉTODO: Em estudo longitudinal retrospectivo analisaram-se 62 prontuários de cirróticos operados pela técnica de Teixeira-Warren na Santa Casa de São Paulo. Foram submetidos a avaliação quanto ao índice de congestão portal pré-operatório 36 doentes, e 58 quanto à classe Child-Pugh. Os doentes foram divididos em grupos - com recidiva e ausência de recidiva hemorrágica - sendo analisada a diferença estatística quanto aos valores do índice e Child-Pugh pré-operatórios, relacionando-os à recidiva hemorrágica pós-operatória. RESULTADOS: Dos doentes que apresentaram recidiva hemorrágica, 69 por cento eram Child B e possuíam índice de congestão portal médio de 0,09. Já entre aqueles que não ressangraram, 62 por cento eram Child A e o índice de congestão portal médio foi de 0,076. A diferença foi estatisticamente significante para a classe Child-Pugh, porém, o mesmo não ocorreu para o índice de congestão portal. CONCLUSÃO: O índice de congestão portal no pré-operatório não foi fator preditivo de recidiva hemorrágica em cirróticos submetidos a cirurgia de Teixeira-Warren. Doentes classificados como Child-Pugh B possuem maior chance de recidiva hemorrágica pós-derivação esplenorrenal distal em relação aos Child-Pugh A.

BACKGROUND: Bleeding from esophagogastric varices is the worst and most lethal complication of cirrhotic portal hypertension. Distal splenorenal shunt (Warrens surgery) is used in the therapeutic of this patients, Child A and B, with rebleeding after clinical endoscopic therapy. The portal vein congestion index is elevated in cirrhotic portal hypertension and could predict rebleeding after Warrens surgery in these patients. AIM: To verify if the portal vein congestion index or liver function (Child-Pugh) at preoperative are predictive factors of rebleeding after Warrens surgery. METHODS: Sixty-two cirrhotic patients were submitted to Warrens surgery at "Santa Casa" Medical School and Hospital - Liver and Portal Hypertension Unit, São Paulo, SP, Brazil. Fifty-eight were analyzed for Child-Pugh class and 36 for portal vein congestion index, divided in two groups: with or without rebleeding and statistical analysis was performed. RESULTS: In the rebleeding group, 69 percent were Child B, with portal vein congestion index = 0.09. The group without rebleeding show us 62 percent patients Child A with portal vein congestion index = 0.076. The difference was significant for Child-Pugh class but not to portal vein congestion index. CONCLUSION: Portal vein congestion index was not predictive of rebleeding after Warrens surgery, but cirrhotics Child B have more chance to rebleed after this surgery than Child A.

Derivação esplenorrenal distal com a interposição de enxerto de veia mesentérica inferior: Relato de caso / Use of inferior mesenteric vein as an interposition graft for distal renal shunt: case report

Hemorragia digestiva alta por ruptura de varizes esofágicas é uma gravecomplicação da hipertensão portal em pacientes cirróticos. Quando osangramento está associado à trombose venosa portal, os procedimentosendoscópicos ou anastomose (shunt) portossistêmica intra-hepáticatransjugular (TIPS) são difíceis e menos eficientes. Nesta situação, aanastomose esplenorrenal distal é uma boa alternativa. O objetivo é discutiruma anastomose esplenorrenal distal com a utilização de enxerto autólogode veia mesentérica inferior. Relatamos o caso de um paciente do sexomasculino, 52 anos, portador de cirrose hepática alcoólica e trombosede veia porta. Evoluiu com nove episódios de hemorragia digestiva alta,apesar do tratamento endoscópico. Como sua função hepática permaneciaboa, a anastomose esplenorrenal distal foi considerada a melhor opção terapêutica. Foi realizada uma anastomose esplenorrenal distal, cominterposição de enxerto autólogo de veia mesentérica inferior, em virtudede eventos inesperados durante a dissecção da veia esplênica. Nãoocorreram complicações pós-operatórias.

Upper digestive bleeding due to rupture of esophageal varices is asevere complication of portal hypertension in cirrhotic patients. Whenit is associated with portal vein thrombosis, transjugular intrahepaticportosystemic shunt or endoscopic procedures are difficult and lesseffective. In this situation, splenorenal shunt is a good alternative.The aim was to discuss a distal splenorenal shunt with autologousinferior mesenteric vein graft. We report a case of a male patient, 52years old, suffering from alcoholic hepatic cirrhosis and portal veinthrombosis. He had nine episodes of upper digestive bleeding, in spiteof endoscopic treatment. His hepatic function remained good and distalsplenorenal shunt was chosen as the best therapeutic option. Theinferior mesenteric vein was used as an interposition graft for distalrenal shunt due to unexpected events during splenic vein dissection.Postoperative recovery went uneventfully.

Lieno-adrenal shunt.

Portal hypertension with variceal bleed and a well functioning liver is an indication for shunt surgery. The commonly performed procedure is the lieno-renal shunt. When the left adrenal vein is long enough, and of healthy diameter a spleno-adrenal shunt is possible. Most of the reported cases are of distal spleno-adrenal type. A case of proximal spleno-adrenal patent shunt with a follow-up two years is presented along with review of literature.

Evaluation of distal splenorenal shunt as an urgent surgical intervention in the management of acute variceal bleeding

The aim of the work was to evaluate distal splenorenal shunt as an urgent surgical intervention in management of acute variceal bleeding in Egyptian patients with portal hypertension. This study included 26 patients with acute variceal bleeding needing urgent surgery. All patients underwent distal splenorenal shunt, and patients were followed up for at least one year. Preoperatively and during the follow up period hepatic functions, portal homodynamic, and the state of varices were assessed. Control of bleeding was achieved in 88.46% of patients, re bleeding varices was reported in 3 patients [11.54%]. No infra-operative mortality, with a reasonable operative time of 148.00 +/- 50.05 minutes. The immediate post-operative mortality was 7.69%, and one patient died during follow up. Post operative morbidity was in the form of encephalopathy in 23.08% of patients, severe ascites in 15.38%, and partial wound dehiscence in 11.54% of patients. The mean follow up period was 21 +/- 4.34 months. Distal splenorenal shunt is a good surgical option for management of urgent variceal bleeding after failure of conservative measures, provided that there are available well trained personnel

A case of post-operative chylous ascites after a splenorenal shunt operation in a child with congenital hepatic fibrosis / 소아과

Chylous ascites is a rare condition caused by various diseases and conditions that interfere with the abdominal or retroperitoneal lymphatics, and uncommonly it can manifest as a post-operative complication after abdominal, retroperitoneal or mediastinal surgery. Chylous ascites can be diagnosed by a high triglyceride content in ascites. The authors experienced a 5-year-old girl with congenital hepatic fibrosis who presented with chylous ascites after a splenorenal shunt operation, who was successfully managed by fasting and total parenteral nutrition, followed by a lipid-free diet with medium chain triglyceride supplementation. Here, the authors report this case of post-operative chylous ascites after a splenorenal shunt (Warren shunt) operation with a review of the pertinent literature.

The Efficiency of Laparoscopic Splenorenal Shunt: a Chronic Canine Model / 대한비뇨기과학회지

PURPOSE: Splenorenal bypass is a major surgical procedure that's used for the management of renal artery stenosis. Herein, we evaluate the feasibility and efficacy of performing laparoscopic splenorenal bypass in a chronic canine model. MATERIALS AND METHODS: A total of 12 animals were used for this study. The initial 6 acute animals were used to develop the technique. The remaining 6 surviving animals, which form the basis for this report, were used for a chronic study with up to 2 months follow-up. The renal artery and the distal splenic artery was dissected, its proximal end clamped and its distal end cut and spatulated. An end-to-end anastomosis of the splenic artery and renal artery was performed using only laparoscopic freehand suturing and knot-tying techniques. Upon revascularization, a laparoscopic doppler ultrasound probe was used to document blood flow in the renal artery. Three animals were each followed for 1 month and 2 months, respectively. RESULTS: The total operative time was 297+/-36 min. The mean number of suture bites per anastomosis was 14.3. The only intraoperative complication was hemorrhage from the anastomotic site. Intraoperative Doppler ultrasound documented good blood flow in all 6 animals upon releasing the clamp. At the time of euthanasia, intravenous pyelography (IVP) showed early visualization of the left kidney with prompt drainage in 5 of the 6 surviving animals. In one animal that had two left renal arteries, a distal thrombosis was found despite the patent anastomotic site. CONCLUSIONS: Laparoscopic splenorenal bypass can be performed in a reproducible fashion with using only intracorporeal techniques. We believe that with experience, complex urologic vascular procedures can be laparoscopically performed in the future.

Fatores preditores de recidiva hemorrágica em cirróticos submetidos à cirurgia de Warren / Predictive factors of rebleeding in cirrhotic patients submitted to the Warren's surgery

OBJETIVO: Estabelecer os fatores preditores determinados no pré-operatório envolvidos na recidiva hemorrágica dos cirróticos submetidos à cirurgia de Warren. MÉTODOS: Cinqüenta e sete cirróticos com boa reserva funcional hepática e antecedente de hemorragia digestiva alta que não responderam ao tratamento clínico-endoscópico, foram submetidos à cirurgia de Teixeira-Warren (derivação espleno-renal distal). Eles foram divididos em dois grupos: 1 = 31 (apresentaram recidiva hemorrágica no pós-operatório) e 2 = 26 (não apresentaram novo sangramento). O grupo 1 foi novamente dividido em dois grupos, segundo a época de recidiva: grupo 1.A = 12 com recidiva hemorrágica até a alta hospitalar e 1.B = 19 com recidiva hemorrágica após a alta. Onze doentes faleceram no período perioperatório e os 46 restantes foram seguidos ambulatorialmente por um período de 3,2 anos em média, sendo analisados os aspectos clínicos, laboratoriais como a dosagem de albumina e bilirrubinas séricas, ultra-sonográficos como o fluxo e calibre portais, e endoscópicos no pré e pós-operatório, bem como dados do intra-operatório como o volume de cristalóides infundido durante a cirurgia. RESULTADOS: As dosagens de albumina sérica e bilirrubinas totais foram em média de 3,33 mg por cento e 1,7 mg por cento no grupo 1, e 3,56 mg por cento e 1,16 mg por cento no grupo 2. O fluxo e o calibre portais foram em média 0,24 cm/s e 1,34 cm no grupo 1, e 0,18 cm/s e 1,21 cm no grupo 2, respectivamente. No grupo 1.A, o volume de cristalóide infundido durante a cirurgia foi em média de 3,692 ml contra 2,166 ml no grupo 1.B. CONCLUSÃO: A dosagem pré-operatória de albumina, bilirrubinas totais, valor do fluxo e calibre portais foram fatores preditores para recidiva hemorrágica em pacientes cirróticos submetidos à cirurgia de Warren. O volume de cristalóide infundido no intra-operatório foi fator preditor para ressangramento precoce.

Ligation of Left Renal Vein for Splenorenal Collateral Shunt to Prevent Portal Flow Steal in Adult Living Donor Liver Transplantation / 대한이식학회지

PURPOSE: To assess the safety and role of ligation of left renal vein (LRV) to avoid portal flow steal to the partial liver graft when living donor liver transplantation (LDLT) is performed for the cirrhotic patients with large spontaneous splenorenal shunt (SRS). METHODS: Between 2001 and 2005, 44 portal hypertensive patients with large SRS who underwent ligation of LRV were retrospectively reviewed. RESULTS: After ligation of LRV, thirty four patients of 44 pateints (77.3%) revealed hypo-attenuation of left kidney on computed tomography but 10 patients (22.7%) showed normal attenuation. Proteinuria and hematuria occurred in 22 patients (50%) and 43 patients (97.7%) respectively after operation, but nearly all of them recovered. Decreased urine outputs (less than 1,000 mL per day) appeared in 4 patients (9.1%), but disappeared after dialysis for 6+/-5.4 days. Serum creatinine increased in 43 patients (97.7%), but decreased to normal range in 40 patients (90.3%). During study period, portal flow steal to the liver graft did not occur after ligation of LRV, and liver regeneration was satisfactory. CONCLUSION: It seems to be a good graft salvage procedure for the portal hypertensive patients who demonstrate large SRS after partial liver engraftment.

Alteraçöes morfológicas e hemodinâmica tardias no território esplênico de pacientes com esquitomosse mansônica hepatoesplênica pós-anastomose esplenorrenal distal. (Estudo com ultra-som Doppler) / Late morphologic and hemodynamic changes in the splenic territory of patients with mansoni's hepatosplenic schistosomiasis after distal splenorenal shunt. (Ultrasonography-Doppler study)

RACIONAL: A anastomose esplenorrenal distal (operaçäo de Warren) tem sido indicada para tratamento do sangramento digestivo provocado pelas varizes esofágicas pois, idealmente, reduziria a pressäo venosa no território cardiotuberositário sem alterar o fluxo mesentérico-portal. Entretanto, as mudanças que esta operaçäo provoca no território esplênico näo foram totalmente esclarecidas. OBJETIVO: Avaliar alteraçöes morfológicas e hemodinâmicas tardias no território esplênico decorrentes da anastomose esplenorrenal distal, em pacientes com hipertensäo portal por esquistossomose mansônica hepatoesplênica complicada com hemorragia pelas varizes esofágicas. MÉTODO: Estudo, mediante ultra-som-Doppler, da regiäo esplênica de 52 pacientes com hipertensäo portal por esquistossomose hepatoesplênica divididos em dois grupos: 40 näo-operados e 12 com anastomose esplenorrenal distal. Neles foram comparados: a) parâmetros morfométricos (diâmetro da artéria e veia esplênicas, diâmetros esplênicos: longitudinal, transversal e ântero-posterior); b) parâmetros velocimétricos do fluxo nos vasos esplênicos (velocidade de pico sistólico na artéria esplênica e velocidade média de fluxo na veia esplênica); c) índice biométrico do baço (longitudinal x transversal); d) índice volumétrico do baço (longitudinal x transversal x ântero-posterior x 0,523); e) índices hemodinâmicos de impedância na artéria esplênica (pulsatilidade e resistividade). RESULTADOS: Nos pacientes com anastomose esplenorrenal distal observaram-se: a) reduçäo - índice volumétrico (näo-operados 903,83 ± 452,77 cm³/anastomose esplenorrenal distal 482,32 ± 208,02 cm³ (46,64 por cento)) e biométrico esplênico (näo-operados 138,14 ± 51,89 cm²/anastomose esplenorrenal distal 94,83 ± 39,83 cm² (33,35 por cento)); b) ausência de variaçäo - artéria esplênica: diâmetro (näo-operados 0,57 ± 0,16 cm/anastomose esplenorrenal distal 0,57 ± 0,23 cm)), velocidade de pico sistólico na artéria esplênica (näo-operados 107 ± 42,02 cm/seg/ anastomose esplenorrenal distal 89,81 ± 41,20 cm/seg)), índice de resistividade (näo-operados 0,58 ± 0,008/anastomose esplenorrenal distal 0,56 ± 0,06)) e índice de pulsatilidade (näo-operados 0,91 ± 0,19/anastomose esplenorrenal distal 0,86 ± 0,15); - veia esplênica (näo-operados 1,10 ± 0,30 cm/anastomose esplenorrenal distal 1,19 ± 0,29 cm)); c) aumento...

Two Cases of Congenital Hepatic Fibrosis with Polycystic Kidney Disease / 대한간학회지

Congenital hepatic fibrosis (CHF) is a rare developemental abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all, cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The pateint also had cystic dilation of subarchnoid space in the basal cistern and posterior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. He had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures.