Interpretation of EASL Clinical Practice Guidelines on the management of cystic liver diseases / 中华消化外科杂志

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.

Poliquistosis hepática aislada asintomática / Asymptomatic isolated polycystic liver disease

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)

Polycystic liver disease: a rare case study

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.

A clinical study comparing two different treatment methods in patients with polycystic liver disease using super selective hepatic arterial embolization / 中华肝胆外科杂志

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.

Fenestration of liver cysts in polycystic liver disease to improve quality of life: a case report and literature review

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.

Polycystic liver disease with portal hypertension:a case report and literature review / 中国基层医药

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.

Resultados de la cirugía laparoscópica en quistes hepáticos no parasitarios / Results of laparoscopic surgery in nonparasitic hepatic cysts

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.

Autosomal Dominant Polycystic Kidney Disease with an Asymptomatic Type I Choledochal Cyst / 대한내과학회지

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.

Clinical research progress of polycystic liver disease / 国际外科学杂志

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.

Estudio imagenológico de poliquistosis renal autosómica dominante, reporte de un caso y revisión de la literatura / Imaging study autosomal dominant polycystic kidney disease, report of a case and review of the literature

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).

Transcatheter super-selective hepatic arterial embolization using a mixture of N-butylcyanoacrylate and iodized oil for treatment of symptomatic polycystic liver disease / 中华放射学杂志

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.

Efficacy of liver transplantation for the treatment of polycystic liver disease: a single center analysis / 中华消化外科杂志

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.

Feasible surgical treatment of autosomal dominant polycystic liver disease associated with autosomal dominant polycystic kidney disease / 第二军医大学学报

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.

Value of clinical classification of adult polycystic liver disease for selection of treatments / 第二军医大学学报

Objective: To study the role of clinical classification of adult polycystic liver disease (APLD) in guiding the selection of treatments for the patients. Methods: The clinical data of 43 APLD patients who were treated in our hospital from May 1995 to Oct. 2007 were retrospectively analyzed. The patients were divided into 3 stages according to the clinical manifestations and imaging examination: non-symptom or slight symptom stage, obvious symptom stage, and lethal symptom stage. Seventeen patients in our group received outpatient clinical follow-up; 26 were treated by partial hepatectomy combined with fenestration. Results: Among the 15 cases at non-symptom or slight symptom stage, one had primary hepatic carcinoma simultaneously and died 4 mouths after diagnosis; the rest 14 case were followed up for a mean of (42.8±37.9) months; 12 of them had slow progression and 2 had rapid progression of APLD; the latter 2 cases received operatioa The 24 cases at obvious symptom stage were treated by partial hepatectomy combined with fenestration. There were no perioperation deathes and the mean follow-up period was (61.4±43.0) months; 2 cases died of renal failure and 3 had post-operation recurrence. Two of the 4 cases at the lethal symptom stage were treated by partial hepatectomy combined with fenestration: one had no symptom relieve after operation and one developed hepatic dysfunction and received liver transplantation 8 months after operation; the other 2 patients received percutaneous cyst aspiration at the outpatient department, one patient died of upper gastrointestinal hemorrhage 5 months later, and one had the symptom slightly relieved. Conclusion: Our classification of APLD can help to select treatments for patients: patients at non-symptom or slight symptom stage should be followed up, those at the obvious symptom stage can be treated surgically, and those at the lethal symptom stage are not suitable for routine operation and liver transplantation may be the best choice for them.

Surgical Treatment of Unilobar Adult Polycystic Liver Disease

Adult polycystic liver disease (APLD) is an inherited, benign rare condition, often associated with polycystic kidney disease. Liver failure is unusual, but some patients may require therapy. Surgery appears to be more effective in relieving the symptoms of APLD for an extended period than nonsurgical therapies. We report on the successful surgical treatment of a case of APLD located in the left lobe of the liver.

Cystectomy and Hepatic Resection for Cystic Lesion of the Liver

PURPOSE: Cystic lesions of the liver are highly variable in respect to appearance and therapeutic approach. However, without cystectomy and hepatic resection, the patient is at risk for recurrent enlargement, infection, or progression of an unrecognized malignant neoplasm. The goal of this study was to discern the safest and most effective method, with special emphasis on a suitable therapeutic technique for various cystic lesions of the liver. METHODS: We reviwed the cystectomy and hepatic resection and outcome of patients with hepatic cysts from November, 1987 to April, 2000 at the Hanyang University Hospital. A retrospective study of 19 patients with various cystic lesions of the liver was performed. RESULTS: There were 8 simple cysts, 2 polycystic liver disease, 2 biliary cystadenoma, 4 biliary cystadenocarcinoma, 2 hydatid cysts, and 1 traumatic cyst. In examining the 6 cystectomies, 13 hepatic resections. (4 right lobectomies, 4 left lobectomies, 5 minor hepatic resections), there were no postoperative deaths in this series. Four patients (21.1%) developed operative complications. During the mean follow- up time of 78.2 months, symptomatic relief was complete and permanent in all of patients except the 1 biliary cystadenocarcinoma 1 of the 4 patients with biliary cystadenocarcinoma died of tumor recurrence (5.3%) approximately 27 months after hepatic resection. CONCLUSION: Cystectomy and hepatic resection is a more curative treatment for cystic lesions of the liver than other treatments. We recommend complete cystectomy and hepatic resection as the preferred therapy, particularly when the cyst is large, a malignancy cannot be ruled out, and a proper diagnosis is not confirmed.

A Case of Intrahepatic Cholangiocarcinoma in Polycystic Liver Disease / 대한간학회지

Cholangiocarcinoma has been associated with various fibrocystic diseases of liver and biliary tract, but cholangiocarcinoma in polycystic liver disease (PLD) was extremely rare. It was reported that the prognosis of cholangiocarcinoma associated PLD was very poor and distant metastases were common. We report a rare case of intrahepatic cholangiocarcinoma associated with PLD in 58- year- old female who presented vague abdominal pain. She had also polycystic kidneys with normal renal function. MRI showed well demonstrated tumor with central scar and a adjacent large cyst in the left lobe of the liver. She underwent extended left lobectomy. On microscopic examination, the tumor was moderately differentiated cholangiocarcinoma having abundant fibrous stroma and necrosis and the adjacent cyst showed focal in situ carcinomatous changes in the lining epithelium.

Surgical management of severe polycystic liver disease / 中华肝胆外科杂志

Objective To evaluate the effects of different surgical treatments on severe polycystic liver disease (SPLD). Methods A total of 22 patients with SPLD were surgically treated in our Department from December 1989 to July 1999. Of the patients, 5 were treated with the partial hepatic resection in combination with cyst fenestration (group A), 7 with laparotomic fenestration (group B), 4 with laparoscopic fenestration (group C) and 6 with puncture under the guidance of ultrasonography B (group D). The surgical outcome and long term follow up results were retrospectively analyzed. Results After the treatments, all the patients experienced immediate relief of symptoms. However, the follow up for an average of 3 years showed that 10 patients developed recurrence of the disease. The recurring rates were 0, 28.5%, 65.5% and 100% in groups A, B, C and D, respectively. Conclusions The approach of partial hepatic resection in combination with cyst fenestration is the most effective treatment for SPLD. Laparoscopic fenestration may not be an appropriate surgical way for treatment of SPLD.