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The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in⁃depth review of the relevant literature, this guideline provides recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence⁃based Medicine system and categorized as "weak" or "strong". This guideline aims to provide the best available evidence to aid the clinical decision⁃making process in the diagnosis and treatment of patients with cystic liver diseases, and presents the readers with translations and summarizations of the above mentioned recommendations.
ABSTRACT
The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in-depth review of the relevant literature, this guideline provide recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence-Based Medicine system and categorized as "weak" or "strong" . This guideline aims to provide the best available evidence to aid the clinical decision-making process in the management of patients with cystic liver disease, and presents the readers with translations and summarizations of the above mentioned recommendations.
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In October 2022, the European Society for the Study of the Liver (EASL) publishes officially the EASL Clinical Practice Guidelines on the management of cystic liver diseases, which covers the diagnosis, treatment, and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, Caroli disease, Caroli syndrome and peribiliary cysts. Combined with the contrast-enhanced imaging examination, the guideline is of great significance for standardized diagnosis and treatment of hepatic cystic lesions. The authors interpret the guideline in detail, and systematically introduce the key points of assessment of various types of cystic liver disease and related recommendations for treatment. Meanwhile, the future applications and research directions are further prospected in order to provide more evidence basis for the clinical manage-ment and decision-making for patients with cystic liver diseases.
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La poliquistosis hepática aislada del adulto es una enfermedad congénita autosómica dominante de los conductos biliares intrahepáticos, con una aparición muy rara. Es más frecuente en el sexo femenino, y los quistes adquieren su máxima expresión a partir de la quinta década de la vida. A pesar de los hallazgos físicos y radiológicos a veces impresionantes, solo en una minoría de los pacientes la enfermedad progresa a lo largo de los años hacia una enfermedad hepática avanzada o desarrolla complicaciones como resultado de hepatomegalia masiva. Se presenta el caso de un paciente masculino que ingresa con el diagnóstico de infarto agudo de miocardio, asintomático desde el punto de vista abdominal y que se le diagnostica la enfermedad poliquística hepática(AU)
Isolated adult polycystic liver disease is rare autosomal dominant congenital disease of the intrahepatic bile ducts. It is more frequent in women, and the cysts acquire their maximum expression from the fifth decade of life. Despite the sometimes impressive physical and radiological findings, in only a minority of patients, the disease progresses over the years to advanced liver disease or develops complications as a result of massive hepatomegaly. We report here the case of a male patient who is admitted with the diagnosis of acute myocardial infarction, asymptomatic from the abdominal point of view and he was diagnosed with polycystic liver disease(AU)
Subject(s)
Humans , Male , Female , Tomography, X-Ray Computed/methods , Ultrasonography/methods , Cysts/diagnostic imaging , Liver Diseases/diagnostic imagingABSTRACT
Polycystic liver disease is a rare inherited disorder which affects 1 in 1,00,000 population. There are two forms of polycystic liver disease (PLD) i.e. isolated PLD and autosomal dominant PLD which is associated with renal cysts. PLD is usually asymptomatic and incidentally detected. Some may present with complaints of abdominal distension, bloating, early satiety, weight loss. Computed tomography (CT) or magnetic resonance imaging is the investigation of choice to diagnose polycystic liver disease. Below we report you a case of 55-year-old female who came with the complaints of pain abdomen since 8 days and was diagnosed as polycystic liver disease on CT, who received treatment by laparoscopic fenestration surgery of cysts and showed symptomatic improvement.
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Objective To compare the effectiveness and safety of two different treatment methods for patients with polycystic liver disease using super selective hepatic arterial embolization. Methods This retrospective study enrolled 41 patients with polycystic liver disease treated from June 2008 to June 2016 in the First Medical Center of Chinese PLA General Hospital. The patients underwent transcatheter super-selective hepatic arterial embdization (TAE). There were 5 men and 36 women. The age ranged from 36 to 68 years. The patients were divided into the observation group (n=14) and the control group ( n=27). The observation group underwent treatment using an emulsion of iodized oil and bleomycin and N-butyl-2-cyanoacrylate. The control group underwent iodized oil and N-butyl-2-cyanoacrylate. The clinical symptoms, operative complications, volumes of intrahepatic cysts, hepatic parenchyma volumes of pre-TAE and post-TAE at 3, 6 months and every 6 monthly were compared. Results TAE was technically successful in all the patients. The follow-up periods ranged from 24 to 72 months. The clinical symptoms improved re-markably in the observation group at 3 month, 6 months, 12 months, and 24 months, being 92. 9% (13/14), 92. 9% (13/14), 92. 9% (13/14), 92. 3% (12/13), respectively. One patient was lost to follow-up 24 months after operation. The clinical symptoms improved remarkably in the control group at 3 month, 6 months, 12 months, and 24 months, being 88. 9% ( 24/27 ), 85. 2% (23/27), 84. 6% ( 22/26 ), 81. 8% (18/22), respectively. One patient was lost to follow-up 12 months after operation and five patients were lost to follow-up 24 months after operation. Compared with preoperative, the abdominal circumference of two groups had decreased, the total volume of intrahepatic cyst and liver decreased at 6 months, 12 months, and 24 months after surgery (P＜0. 05). Conclusions TAE was a safe and effective treatment for polycystic liver disease. Patients in the observation group had more effective treatment outcomes on follow-up.
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La poliquistosis hepática sin poliquistosis renal es una condición clínica infrecuente y más aún su complicación hemorrágica, con muy pocos casos reportados, siendo fatal sin tratamiento precoz. Mujer, 40 años, consulta por dolor en hipocondrio derecho, una hora de evolución, punzante, intensidad moderada; náuseas. En Emergencia: PA 130/80mmHg, FC 100 lpm, T 37°C, saturación O2 99 %, FR 22 rpm. Abdomen blando. Dolor en hipocondrio derecho. No visceromegalias. Punto cístico y signo de Murphy positivo. Laboratorio: Hematocrito 34 %, hemoglobina 11,4g/dL, leucocitos 12190, amilasa 70U/L. Ecografía Abdominal: Poliquistosis hepática con ruptura de quiste, líquido subcapsular y abundante libre en cavidad, sin lesiones en páncreas ni riñones. Se realiza laparoscopía exploradora de emergencia, drenaje y aspiración de líquido más quistectomía parcial. Evolución favorable, cita con familiares directos para estudios complementarios.
Polycystic liver disease without the presence of polycystic kidney disease is a rare clinical condition and its hemorrhagic complication is even rarer, with very few cases being reported. It can be life-threatening without a rapid intervention. Case report: A 40 year-old female who presented with moderate sharp right upper quadrant abdominal pain and nausea for one hour. Emergency department: blood pressure 130/80 mmHg, heart rate 100 beats per minute, temperature 37 °C, O2 saturation 99 %, respiratory rate 22 breaths per minute. On examination, soft and relaxed abdomen, right upper quadrant abdominal pain, no visceromegaly, positive Murphy's sign and rebound sign. Labs: hematocrit 34 %, hemoglobin 11.4 g/dL, white blood cells 12190, amylase 70 U/L. Abdominal ultrasound: Polycystic liver disease with ruptured cyst, abundant free subcapsular fluid within cavity, without pancreas and kidneys injury. Procedures: emergency exploratory laparoscopy, fluid drainage and aspiration, and partial cystectomy. Favorable evolution. Appointment with patient´s direct relatives for further studies.
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Objective:To investigate the super selective transhepatic arterial embolization (TAE) for effects of cyst volume and liver function in patients with symptomatic polycystic liver disease (PLD), and provide a reliable basis for clinical diagnosis and treatment. Methods: 14 cases of symptomatic patients with PLD were selected from October 2014 to October 2015 in our hospital using super selective hepatic arterial embolization. The upper abdomen was performed CT scan one year after surgery, and the volume of patients with liver cyst was tested. We observed the changes of total volume of liver before TAE and postoperative kidney function, adverse reactions, and the improvements after four months’ follow-up.Results: The angiography for the 14 patients showed liver volume increased in different degrees. It also showed that blood supply was mainly hepatic artery wall branch. There were different sizes of liver round unstained area. There was consistent between CT examination and unstained area in polycystic liver swollen area. The difference of the total volume of the liver cyst was statistically significant in 14 cases of the patients with PLD before TAE treatment and after 12 months’ treatment (t=2.8627,P0.05). Compared with the preoperative, there was little effect on liver function by TAE. After 3 to 12 months, symptoms gradually improved and physical rating increased. The improvement rate was 100% after 12 months and the physical score was from 90 to 100 points. The embolism syndrome in 14 cases of patients gradually disappeared after 1 to 5 days, and five days later the symptoms disappeared. Conclusions: There is a significant effect for symptomatic polycystic liver patients with TAE. It can improve efficacy and reduce patient suffering with a low recurrence rate. It is worthy of clinical application.
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Polycystic liver disease (PCLD) is characterized by a large number of liver cysts scattered throughout the liver parenchyma. We herein intend to present the beneficial effect of palliative fenestration treatment on quality of life in a patient with symptomatic PCLD. A 48-year-old female patient had been followed up for 5 years for both polycystic liver and kidney diseases at another institution. During follow-up for last 1 year, we recognized that she had barely maintained her ability of function in daily activities due to progressive worsening of fatigue and dyspnea on exertion. The patient finally underwent surgical fenestration treatment. Multiple cysts in the enlarged liver were opened and the cyst walls were excised with electrocautery. No surgical complication occurred and the patient was discharged 10 days after the open fenestration surgery. The total liver volume was 3,870 ml before surgery and 3,125 ml at 1 week after surgery, showing a volume reduction of 19.3%. After surgery, her performance status improved significantly. In the present case, significant improvement in quality of life and daily activity performance was achieved after open fenestration treatment over 18 months of follow-up without disease recurrence.
Subject(s)
Female , Humans , Middle Aged , Dyspnea , Electrocoagulation , Fatigue , Follow-Up Studies , Hepatomegaly , Kidney Diseases , Liver Diseases , Liver Transplantation , Liver , Quality of Life , RecurrenceABSTRACT
Objective To study the clinical features,diagnosis and treatment of polycystic liver disease (PLD)complicated with portal hypertension (PHT).Methods The clinical data of one patient with PLD and PHT was retrospectively analyzed,and relevant literature was reviewed.Results The patient presented fatigue,dyspepsia, abdominal distension and lower limb edema.Laboratory examination showed mild liver dysfunction(Alkaline phospha-tase 291.2U/L,gamma glutamyl transpeptidase 168.1U/L,59.9g/L,total protein,albumin 32.2g/L,21.0μmol /L, total bilirubin,direct bilirubin 11.5μmol /L).Abdominal ultrasound (US)and computerized tomography (CT) showed multiple noncommunicating cysts of varying size in both liver and kidney.Antioxidant supplements and diuretic were introduced,and the therapy was approved to be effective.Conclusion The case in this report illustrates that PLD could occasionally present with PHT.Physician should be alert to prevent misdiagnosis.
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Objective To evaluate the clinical efficacy of transcatheter super-selective hepatic artery embolization (TAE) in treating symptomatic polycystic liver disease (PLD). Methods A total of 8 patients with PLD, who were admitted to authors’ hospital during the period from 2009 to 2013 to receive TAE, were enrolled in this study. The patients included 6 females and 2 males with a mean age of 59.5 years (54-65 years). The used embolic agents were polyvinyl alcohol (PVA) microspheres and micro spring coils. Both plain and contrast-enhanced CT scans of the upper abdomen were performed before TAE as well as at 12 months after TAE; the total volume of the hepatic cysts was measured and the changes of the cystic volume were determined. Statistical analysis was conducted using paired t test. Results The technical success rate was 100%. After TAE, the patients developed fever and different degrees of discomfort at liver area, which were disappeared after active symptomatic medication, and no serious complications occurred. The patients were followed up for 12 months , the mean total volume of the intrahepatic cysts decreased from preoperative (5 794±2 066) cm3 (range 3 120-8 935 cm3) to postoperative (3 832±1 525) cm3 (range 2 019-5 925 cm3), the difference was statistically significant (t=6.971, P<0.001). The reduction ratio of total volume of intrahepatic cysts was 34.6%±11.3%(24.3%-60.4%). Conclusion For symptomatic polycystic liver disease, transcatheter super-selective hepatic arterial embolization is a newly-developed treatment. This technique is safe and effective with reliable response and fewer complications. Therefore, it should be recommended in clinical practice.
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Los quistes hepáticos no hidatídicos (QHNH) se detectan de forma incidental, pueden ser únicos y múltiples. Desde que se describió el tratamiento laparoscópico de estos, se han reportado diversas series; existiendo por ende evidencia que avala la cirugía laparoscópica de estas lesiones. El objetivo de este estudio, es reportar los resultados del tratamiento laparoscópico de QHNH en términos de morbilidad postoperatoria (MPO). Serie de casos. Se incluyeron pacientes intervenidos quirúrgicamente vía laparoscópica por QHNH en el Hospital Clínico de la Universidad Mayor entre enero de 2008 y diciembre de 2012. El estudio preoperatorio consistió en exámenes generales, determinación de ELISA-IgE e IgG para hidatidosis, ecotomografía abdominal o tomografía computarizada. En el período estudiado, se intervinieron 17 pacientes con QHNH, resecándose en ellos, un total de 39 quistes. El 76,5 por ciento de los casos eran de sexo femenino; y la mediana de edad de la serie fue de 56 años. La mediana del diámetro ecográfico de las lesiones fue de 7,5 cm. Se realizó quistectomía subtotal en todos ellos, sin utilización de drenaje. Se realizó epiploplastia en 4 pacientes. En el 82,4 por ciento de los casos se ejecutó de forma simultánea una colecistectomía por colelitiasis. La mediana del tiempo quirúrgico fue de 51 minutos. No fue necesario convertir a ningún paciente. La serie no registra MPO ni mortalidad. La mediana de estancia hospitalaria fue de 1 día. El estudio histopatológico confirmó "quiste de tipo biliar" en la totalidad de los casos. Con un seguimiento mínimo de 6 meses, no se ha evidenciado MPO tardía ni reaparición de las lesiones resecadas. El tratamiento aplicado a esta serie de casos de QHNH, se asocia a escasa estancia hospitalaria y buena evolución a corto y mediano plazo.
Nonparasitic hepatic cysts (NPHC) can be detected incidentally, and may be single or multiple. Since described laparoscopic treatment of NPHC, several series have been reported, therefore there is enough evidence that support the role of laparoscopic surgery in the treatment of NPHC. The aim of this study is to report the results of laparoscopic treatment of QHNH in terms of postoperative morbidity (POM). Case series. Patients operated laparoscopically by NPHC in the Universidad Mayor Clinical Hospital, Temuco, between January 2008 and December 2012 were included. The preoperative evaluation consisted of general exams, ELISA-IgE and IgG determination for hydatid disease, abdominal ultrasonography or abdominal CT scan. During the study period, 17 patients with NPHC were operated, resecting them a total of 39 cysts. 76.5 percent of patients were female, and the median age of the series was 56 years. The median sonographic diameter of the lesions was 7.5 cm. Subtotal cystectomy was performed in all of them, without using drainage. Epiploplasty was performed in 4 patients. In 82.4 percent of cases simultaneously cholecystectomy for cholelithiasis was performed. The median operative time was 51 minutes. Neither patient was converted. The series does not register MPO or mortality. The median hospital stay was 1 day. Histopathology confirmed the "biliary type cyst" in all cases. With a minimum follow up of 6 months, there is no evidence or late MPO or recurrence of the lesions resected. The treatment applied to this series of NPHC, is associated with short hospital stay and good outcome in the short and medium term.
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Humans , Male , Female , Middle Aged , Liver Diseases/surgery , Laparoscopy/methods , Cysts/surgery , Follow-Up Studies , Length of Stay , Treatment OutcomeABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease. Polycystic liver disease is commonly observed in ADPKD but rarely has it been associated with a choledochal cyst. We report a case of a choledochal cyst with ADPKD in a 60-year-old female patient with ADPKD and concomitant chronic renal failure who was admitted because of acute deterioration of kidney function due to poor oral intake. There was no history of a biliary disorder and her kidney function recovered promptly after fluid replacement. Non-enhanced computed tomography was performed to evaluate ADPKD and revealed numerous cysts in both kidneys and liver. It also showed fusiform dilatation of the extrahepatic bile ducts, a finding indicative of a choledochal cyst. Liver function was within the normal range and there was no evidence of extrahepatic biliary obstruction. Magnetic resonance cholangiopancreatography confirmed the diagnosis of a type I choledochal cyst combined with ADPKD.
Subject(s)
Female , Humans , Bile Ducts, Extrahepatic , Cholangiopancreatography, Magnetic Resonance , Choledochal Cyst , Cysts , Dilatation , Kidney , Kidney Failure, Chronic , Liver , Liver Diseases , Polycystic Kidney, Autosomal Dominant , Reference ValuesABSTRACT
The full name of the polycystic liver disease is autosomald ominant polycystic liver disease.Surgical treatment is the main method to deal with it at present.With the deep study of the polycystic liver disease into the molecular genetic level,it is possible for molecular diagnostics to achieve presymptomatic and prenatal diagnosis.The article mainly introduce the research progress of the polycystic liver disease's etiology,pathogenesis,diagnosis,treatment,and so on.
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La poliquistosis renal autosómica dominante (PRAD), es una enfermedad hereditaria multiorgánica, caracterizada por el progresivo crecimiento y desarrollo de quistes renales que destruyen el parénquima funcional. Es la patología quística renal más frecuentemente transmitida de forma genética y es causa de insuficiencia renal crónica (IRC) que en ocasiones precisa de tratamiento renal sustitutivo. Describimos el caso de una paciente adulta con PRAD asociada a poliquistosis hepática que tiene antecedente del progenitor de PRAD, fue diagnosticada hace ocho años por estudio ecográfico, se le realizó el seguimiento correspondiente. Actualmente empezó a presentar alteración de la función renal, pero preserva la función hepática. Existen muy pocos casos reportados en nuestro medio, a pesar de ser una patología relativamente frecuente. Por lo que se decide hacer una revisión enfocada en el diagnóstico imagenológico, dejando en claro la utilidad de la ecografía en el diagnóstico de poliquistosis renal, ya que es el método de elección en el diagnóstico por imagen, adicionalmente nos permite hacer un seguimiento del caso y confirmar o descartar la frecuente asociación de una poliquistosis en otro órgano (fundamentalmente hepático).
Autosomal dominant polycystic kidney disease (PRAD) is a multisystem hereditary disease characterized by the progressive growth and development of renal cysts that destroy functional parenchyma. Renal cystic disease is the most common genetic form transmitted and causes of chronic renal failure (CRF) which sometimes requires renal replacement therapy. We describe an adult patient with polycystic liver PRAD associated with the parent who has a history of PRAD, was diagnosed eight years ago by ultrasonography, underwent the follow-up. Today started presenting impaired renal function, but preserved liver function. There are very few cases reported in our area, despite being a relatively common condition. It is decided to review focuses on imaging diagnosis, making clear the usefulness of ultrasonography in the diagnosis of polycystic kidney disease, since it is the method of choice for imaging additionally allows us to monitor the case and confirm or rule out the frequent association of polycystic in another organ (mainly liver).
Subject(s)
CystsABSTRACT
Objective To evaluate the safety and efficacy of transcatheter arterial embolization (TAE) of massive polycystic liver disease (PLD).Methods A total of 21 patients with symptomatic PLD were enrolled.The patients consisted of seventeen women and four men (aged 36-64 years,mean age,49 years).Transcatheter superselective embolization was performed with the mixture of N-butylcyanoacrylate (NBCA) and iodized oil.All patients underwent contrast enhanced computed tomography (CT) of the liver before TAE and at every 3 months for the first half year after TAE,and at 6-monthly intervals thereafter.Laboratory data,including routine blood tests and liver enzymes,were collected.T test was used for statistics.Results All procedures were successful without serious complications.There was no obvious improvement during the first three months.At follow-up of 6-12 months,symptoms notably improved in 18 of 21 patients,and these patients experienced further relief of the symptoms in the follow-up period.TAE failed to benefit in 3 patients,but there were no complaints of worsening of the symptoms.At follow-up CT,the total liver volume and total intra-hepatic cyst volume decreased significantly (t =6.75,7.73,P ＜0.01)compared with pre-TAE in 18 patients at 12 months after TAE.The total liver volume decreased from (8270 ± 3016) cm3 to (6120 ± 2680) cm3 and the total intra-hepatic cyst volume decreased from (7120 ±3070) cm3 to (4560±2488) cm3.Mild elevation of the liver enzymes returned to the normal range within 1 month in all patients.Conclusions It is suggested that transcatheter super selective embolization with the mixture of NBCA and iodized oil is a safe and effective treatment for PLD patients.This technique is a supplemental option for traditional therapy.
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La enfermedad poliquística del hígado en el adulto es una condición infrecuente y benigna, autosómica dominante, asociada con frecuencia a enfermedad poliquística renal. Se caracteriza por la formación de quistes hepáticos numerosos, con o sin afectación renal. Algunos otros factores probablemente implicados en su génesis son la edad, sexo femenino, embarazo, y el uso de anticonceptivos orales. Las terapias quirúrgicas incluyen la aspiración percutánea del quiste con el escleroterapia, fenestración laparoscópica, fenestración laparotómica, la resección del hígado y el trasplante del hígado. Caso: Hombre de 50 años de edad con diagnóstico de hígado poliquístico asociado a un quiste esplénico diagnosticado por TAC abdominal y que se trató con una técnica de videoloparoscopia. Conclusión: La intervención quirúrgica para la enfermedad del hígado poliquístico sintomático se ha asociado a una morbilidad significativa. La fenestración videolaparoscópica de los quistes es un método seguro y extremadamente efectivo y se debe considerar como tratamiento inicial para la enfermedad del hígado poliquístico.
Polycystic liver in the adult is a rare and benign, autosomal dominant disorder, frequently associated with renal polycystic disease. It is distinguished by formation of numerous hepatic cysts, with or without renal involvement. Some other factors are also thought to be involved in its genesis, such as age, female gender, pregnancy, and the use of oral birth control pills. Surgical therapies include percutaneous cyst aspiration with sclerotherapy, laparoscopic fenestration, open fenestration, liver resection and fenestration and liver transplantation. Case: Polycystic liver disease associated with splenic cyst was diagnosed in a 50 years old man by abdominal computed tomography imaging and videolaparoscopic finding. Conclusion: Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity. Laparoscopic cysts fenestration was safe and extremely effective and should be considered as initial treatment for polycystic liver disease.
Subject(s)
Humans , Male , Middle Aged , Kidney Diseases, Cystic/pathology , Liver/injuries , Splenic Neoplasms/complications , Splenic Neoplasms/diagnosis , Biliary Tract , EndocrinologyABSTRACT
Objective To investigate the efficacy of liver transplantation for the treatment of polycystic liver disease.Methods The clinical data of 8 patients with polycystic liver disease who received liver transplantation at the Third Affiliated Hospital from September 2003 to July 2009 were retrospectively analyzed.All patients underwent modified piggyback liver transplantation with vena cava plasty,and 1 of the patients received simultaneons liver-kidney transplantation.Data including acute rejections,complications,graft functions and survival time of patients were recorded.Results Operation was successfully performed on all patients.The mean operation time,anhepatic phase and operative blood loss were (7.5 +2.8)hours (range,4-11 hours),(42 + 14)minutes (range,25-70 minutes) and (2250 ± 1850)ml (range,2000-6500 ml),respectively.One patient received liver retransplantation at 45 days after primary liver transplantation because of hepatic artery stenosis.Two patients died during perioperative period.One patient died of pulmonary infection and multiple organ disfunction syndrome (MODS) at 39 days after operation,and the other one died of MODS at 59 days after operation.The median follow-up time was 60 months (range,37-93 months).Six patients survived for more than 3 years,4 patients survived for more than 5 years and 2 patients survived for more than 7 years.No acute rejection of gratis was observed at the end of the follow up.Conclusion Liver transplantation is safe and effective for the treatment of polycystic liver disease.
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Objective: To investigate the feasibility and outcome of partial hepatectomy and fenestration combined with renal cyst decortication for treatment of autosomal dominant polycystic liver disease (APLD) associated with autosomal dominant polycystic kidney disease (APKD). Methods: From July 2007 to Oct. 2007, three patients with APLD associated with APKD were treated by partial hepatectomy and fenestration combined with renal cyst decortication in our hospital. Their preoperative symptoms, operation procedure, operation time, complications and the clinical outcomes were retrospectively analyzed. Results: The 3 patients were successfully treated and discharged. The operation time periods of the 3 patients were 475, 402, and 430 minutes. Pleural effusion and ascites occurred in all the 3 patients and disappeared after puncture and drainage. Follow-up was conducted 5, 6, and 9 mouths later; the symptoms disappeared in all patients and the renal function became normal. The blood pressure of 2 patients recovered to normal level after operation. Conclusion: Partial hepatectomy and fenestration combined with renal cyst decortication is a safe and acceptable procedure for treatment APLD associated with APKD. The complication rate is comparatively more and the long-term outcomes need to be evaluated further.