Surgical management of polycystic liver disease

Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD. Bile duct overgrowth after embryogenesis results in cystic hepatic dilatations that are known as biliary microhamartomas or von Meyenburg complexes. Further dilatation arises from cellular proliferation and fluid secretion into these cysts. There is a variable, broad spectrum of manifestations of PCLD. Although PCLD is most often asymptomatic, massive hepatomegaly can lead to disabling symptoms of abdominal pain, early satiety, persistent nausea, dyspnea, ascites, biliary obstruction, and lower body edema. Complications of PCLD include cyst rupture and cyst infection. Also, there are associated medical problems, especially intracranial aneurysms and valvular heart disease, which clinicians need to be aware of and evaluate in patients with PCLD. In asymptomatic patients, no treatment is indicated for PCLD. In the symptomatic patient, surgical therapy is the mainstay of treatment tailored to the extent of disease for each patient. Management options include cyst aspiration and sclerosis, open or laparoscopic fenestration, liver resection with fenestration, and liver transplantation. The surgical literature discussing treatment of PCLD, including techniques, outcomes, and complication rates, are summarized in this review.     

Evaluation of hepatic cystic lesions

Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making.     

Surgical management and longterm follow-up of non-parasitic hepatic cysts

Background:

Despite the increasing use of laparoscopic techniques, the optimal surgical approach for cystic liver disease has not been well defined. This study aims to determine the optimum operative approach for these patients.

Methods:

Data were identified from the Lothian Surgical Audit, case note review and general practitioner contact. Patients were contacted and asked to complete the SF-36 questionnaire on quality of life.

Results:

A total of 102 patients (67 with simple cysts, 31 with polycystic liver disease [PCLD], four with cystic tumours) underwent 62 laparoscopic deroofings, 15 open deroofings, 36 resections and one liver transplant between June 1985 and April 2006. The median follow-up was 77 months (range 3–250 months). Morbidity and recurrent symptom rates after laparoscopic surgery were greater in PCLD patients compared with simple cyst patients, at 31% (four patients) vs. 15% (seven patients) and 85% (11 patients) vs. 29% (24 patients), respectively. Four patients with simple cysts and eight with PCLD required further surgery. All patients with simple cysts had comparable quality of life after surgery. Patients with recurrent symptoms after surgery for PCLD had a significantly better quality of life following laparoscopic deroofing than after resection.

Conclusions:

Most simple cysts can be managed laparoscopically, but there is a definite role for open resection in some patients. Open deroofing is the preferred approach for a dominant cyst pattern in PCLD, whereas resection is necessary for diffuse cystic disease.     

Complications arising in simple and polycystic liver cysts

Liver cysts are common,affecting 5%-10% of the population.Most are asymptomatic,however 5% of patients develop symptoms,sometimes due to complications and will require intervention.There is no consensus on their management because complications are so uncommon.The aim of this study was to perform a collected review of how a series of complications were managed at our institutions.Six different patients presenting with rare complications of liver cysts were obtained from Hepatobiliary Units in the United Kingdom and The Netherlands.History and radiological imaging were obtained from case notes and computerised radiology.As a result,1 patient admitted with inferior vena cava obstruction was managed by cyst aspiration and lanreotide;1 patient with common bile duct obstruction was first managed by endoscopic retrograde cholangiopancreatography and stenting,followed by open fenestration;1 patient with ruptured cysts and significant medical co-morbidities was managed by percutaneous drainage;1 patient with portal vein occlusion and varices was managed by open liver resection;1 patient with infected cysts was treated with intravenous antibiotics and is awaiting liver transplantation.The final patient with a simple liver cyst mimicking a hydatid was managed by open liver resection.In conclusion,complications of cystic liver disease are rare,and we have demonstrated in this series that both operative and non-operative strategies have defined roles in management.The mainstays of treatment are either aspiration/sclerotherapy or,alternatively laparoscopic fenestration.Medical management with somatostatin analogues is a potentially new and exciting treatment option but requires further study.     

Pathophysiology,epidemiology, classification and treatment options for polycystic liver diseases

Polycystic liver diseases(PLD)represent a group of genetic disorders in which cysts occur in the liver(autosomal dominant polycystic liver disease)or in combination with cysts in the kidneys(autosomal dominant polycystic kidney disease).Regardless of the genetic mutations,the natural history of these disorders is alike.The natural history of PLD is characterized by a continuous increase in the volume and the number of cysts.Both genders are affected;however,women have a higher prevalence.Most patients with PLD are asymptomatic and can be managed conservatively.Severe symptoms can affect 20%of patients who develop massive hepatomegaly with compression of the surrounding organs.Rrarely,patients with PLD suffer from acutecomplications caused by the torsion of hepatic cysts,intraluminal cystic hemorrhage and infections.The most common methods for the diagnosis of PLD are cross sectional imaging studies.Abdominal ultrasound and computerized tomography are the two most frequently used investigations.Magnetic resonance imaging is more sensitive and specific,and it is a valuable test for patients with intravenous contrast allergies or renal dysfunction.Different treatment modalities are available to physicians caring for these patients.Medical treatment has been ineffective.Percutaneous sclerotherapy,transarterial embolization,cyst fenestration,hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of disease.This review outlines the current knowledge of the pathophysiology,clinical course,diagnosis and treatment strategies of PLD.     

Rare cystic liver lesions: A diagnostic and managing challenge

Cystic formations within the liver are a frequent finding among populations.Besides the common cystic lesions,like simple liver cysts,rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis.Thorough knowledge of each entity’s nature and course are key elements to successful treatment.Detailed search in PubMed,Cochrane Database,and international published literature regarding rare cystic liver lesions was carried out.In our research are included not only primary rare lesions like cystadenoma,hydatid cyst,and polycystic liver disease,but also secondary ones like metastasis from gastrointestinal stromal tumors lesions.Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided.A diagnostic and therapeutic algorithm is also proposed.The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities,diagnostic tools,and treatment modalities is stressed.Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team,in order to receive the most appropriate treatment,since many cystic liver lesions have a malignant potential and evolution.     

Co-existence of renal hydatid cyst and renal cell carcinoma in one kidney: A case report

Rationale: Cystic echinococcosis(hydatid disease) is a parasitic infection of humans, but renal hydatid cyst is rare. It is even more uncommon to find hydatid cyst and renal cell carcinoma(RCC) synchronously in one kidney.Patient concerns: This report presents a 47 years old Iranian man with the chief complaint of lower abdominal pain. Abdominal ultrasound and computed tomographic scan was performed for more evaluation.Diagnosis: RCC was the most probable diagnosis, so he went under left total nephrectomy. Eventually, after histopathologic examination of the excised kidney, cystic echinococcosis came to the first line and papillary RCC was the second diagnosis.Outcomes and lessons: Diagnosis of hydatid cyst and RCC is mostly based on imaging, but, as we noticed in this case, distinguishing between these two may sometimes be difficult due to radiological similarities.     

Unexpected outcome after sclerotherapy of simple renal cyst

ABSTRACT: BACKGROUND: Simple renal cysts usually have benign clinical features. We report a rare case of papillary renal cell carcinoma (RCC) associated with a large recurrent simple cyst following sclerotherapy. Case Presentation A 47-year-old Japanese woman received minocycline sclerotherapy for a large (9 cm in diameter) simple left renal cyst in May 2005. The cyst regrew, and second-line sclerotherapy with ethanol was performed in November 2005. Three years later, she developed papillary RCC on the wall of the recurrent renal cyst. Radical nephrectomy was performed, but the patient died of metastatic disease 15 months after surgery. CONCLUSION: Malignant transformation from recurrent simple renal cyst to RCC may occur in the years following sclerotherapy, underscoring the need for long-term follow-up. Key words: papillary renal cell carcinoma, simple renal cyst, sclerotherapy, malignant transformation.     

Cystic tumors of the liver： A practical approach

Biliary cyst tumors （cystadenoma and cystadenocarcinoma） are an indication for liver resection. They account for only 5% of all solitary cystic lesions of the liver, but differential diagnosis with multiloculated or complicated biliary cysts, atypical hemangiomas, hamartomas and lymphangiomas may be difficult. The most frequent challenge is to differentiate biliary cyst tumors from hemorrhagic cysts. Computerized tomography （CT） and magnetic resonance imaging （MRI） are often not diagnostic and in these cases fine needle aspiration （FNA） is used to confirm the presence of atypical biliary cells. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Pre-operative differentiation of cystadenoma from cystadenocarcinoma is impossible and surgery must be performed if a biliary cyst tumor is suspected. When multiple cystic lesions are observed throughout the liver parenchyma, it is important to exclude liver metastasis, of which colonic cancer is the most common primary site. Multiple biliary hamartomas （von Meyenburg complex） can appear as a mixture of solid and cystic lesions and can be confused with cystic metastasis. Strong and uniform T2 hyperintensity on MRI is usually diagnostic, but occasionally a percutaneous biopsy may be required.     

Successful living donor liver transplantation for polycystic liver in a patient with autosomal-dominant polycystic kidney disease

Orthotopic liver transplantation has been recommended for patients with disabling polycystic liver disease (PCLD). Because of the shortage of cadaveric donors, living donor liver transplantation (LDLT) has been developed as an alternative. We describe the case of a woman with PCLD as an extrarenal manifestation of autosomal-dominant polycystic kidney disease (ADPKD) who was successfully palliated by LDLT. The patient was a 48-year-old woman with abdominal distention. Computed tomography showed a massively enlarged liver containing innumerable cysts, as well as bilateral kidney cysts. Hepatic and renal functions were well preserved. Genetic analysis of the family did not exclude linkage to the PKD1 locus. Two and a half years after the first examination, the patient reported severely disabling symptoms caused by the PCLD. Living donor liver transplantation was performed using a right-lobe graft. The recipient and donor were both well 8 months after the transplantation. The excised liver weighed 7.4 kg, and the histopathology revealed multiple cysts and von Meyenburg complexes in the portal areas.     

Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease

Aim: The goal of this study was to compare the clinical features of patients with isolated polycystic liver disease (PCLD) with those of patients with polycystic liver and autosomal dominant polycystic kidney disease (ADPKD). Methods: Cases were identified from clinical records at the University of Colorado Hospital in Denver (USA) and at the Radboud University Hospital in Nijmegen (the Netherlands) by ICD‐10 codes. To be included in this analysis, patients had to have an initial diagnosis of PCLD within six years of presentation to our clinics. Medical records were reviewed for demographic information, medical history, physical examination, symptoms, complications, laboratory and imaging results, therapy and outcomes. Results: Out of a total of 94, 53 patients met our criteria for entering this study, 19 with PCLD and 34 with ADPKD. The mean time interval from diagnosis of PCLD to presentation in our clinics was 1.21 years for PCLD and 2.76 years for ADPKD (P=NS). PCLD was associated with female gender in both PCLD and ADPKD. Patients with PCLD had greater numbers (P=0.031), and larger sizes of liver cysts (P=0.0051), but had less associated morbidities than patients with ADPKD. Liver cyst decompressions were performed more frequently in PCLD patients (57.9 vs. 23.5%, P=0.012). However, serious hepatic complications, sufficient to require consideration of liver transplantation, were more frequent in patients with ADPKD (0/19 vs. 6/34, P<0.0001). Conclusions: Although PCLD in patients with PCLD is characterized by larger and greater number of hepatic cysts, the clinical course is relatively benign compared with ADPKD.     

Acute abdomen and ascites as presenting features of autosomal dominant polycystic kidney disease

We describe a patient with sudden onset of abdominal pain and ascites,leading to the diagnosis of autosomal dominant polycystic kidney disease(ADPKD).Her presentation was consistent with acute liver cyst rupture as the cause of her acute illness.A review of literature on polycystic liver disease in patients with ADPKD and current management strategies are presented.This case alerts physicians that ADPKD could occasionally present as an acute abdomen;cyst rupture related to ADPKD may be considered in the differential diagnoses of acute abdomen.     

Retroperitoneal localization of hydatid cyst disease

In this study we discuss a rare case; hydatid cyst disease that is located in the retroperitoneum. Two cases were treated in our department with hydatid cyst disease located in the retroperitoneum; one of the cysts was in the minor pelvis and the other was on the left psoas muscle. Diagnosis was confirmed with blood tests, and radiological examinations. The cyst wall was excised partially and was oversewn. Following the operation both of the patients were given albendasole tablets for 10 weeks with blood count and liver enzyme monitorization. Hydatic cysts located in retroperitoneum were treated successfully. Especially in the endemic areas hydatid cyst should be remembered when evaluating cystic masses in the retroperitoneum. It can be treated successfully with surgery.     

Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases

Background and aim: Isolated polycystic liver disease (PCLD) is characterized by the presence of multiple cysts in the liver in the absence of polycystic kidneys. The clinical profile of PCLD is poorly defined and we set up a study for the clinical characteristics of PCLD. Methods: We collected clinical data on 188 PCLD patients (defined as >10 liver cysts) from five tertiary referral centres, and 137 patients were selected for the purpose of this study. We performed molecular analysis of the PCLD associated genes PRKCSH and SEC63 in 91 patients. Results: A total of 118 (86%) patients were female. The majority of patients (88%) had >20 cysts. The median age at diagnosis was 47 years (range 23–84). 37 (41%) patients carried a mutation. Clinical symptoms at presentation were present in 111 (84%) patients. γ‐glutamyl transferase was elevated to 1.4 times upper limit of normal (interquartile range 1.0–2.7). The presence of a mutation and female gender predicted a more severe course: female patients were 9 years younger at the time of diagnosis (47 years; range 23–84) and 91% had symptoms (P<0.01); likewise, mutation carriers were younger at presentation (39 years; range 35–48) and 95% of this cohort had symptoms (P<0.01). During follow‐up [median 8.2 years (range 0–35)], 10% of untreated and 51% of treated patients developed complications. Mortality in this cohort was 8%, but only 2% died of PCLD‐related causes. 58% of patients were treated a median of 2 years (range 0–25) after diagnosis. Conclusion: Symptomatic PCLD patients are mainly females. Females and mutation carriers were younger at diagnosis and had a more severe course of disease.     

先天性胆总管扩张症38例分析

目的 研究胆总管扩张症的正确诊断与治疗方法。方法 回顾性分析1985年1月至2004年1月我科手术治疗的38例先天性胆总管扩张症患者的资料，术后平均随访50个月。结果 行胆总管十二指肠吻合术2例，胆总管空肠Roux—Y吻合术9例，行胆总管扩张部切除加肝总管空肠Roux—Y吻合术27例；术后胆瘘3例，胰瘘并腹腔出血各1例，无手术死亡。结论 胆总管扩张部切除加胆肠Roux—Y吻合是本病的主要治疗方法，疗效好，局限性肝内胆管扩张可行部分肝叶切除术。     

Pitfalls in the diagnosis of complicated pulmonary hydatid disease

The aim of this study was to present the pitfalls in the diagnosis of complicated pulmonary hydatid disease and to discuss the unusual radiological presentations of this endemic disorder in Turkey. We retrospectively evaluated 34 patients (12 females) aged between 8 and 64 years, who were operated on at our centre between 1991 and 1998 and diagnosed with complicated pulmonary hydatid cyst histopathologically. Computerized tomography (CT) scans of these patients were reviewed double-blind by two radiologists. The patients were then divided into two groups: group 1: initial radiological impression is pulmonary hydatid cyst and group 2: initial radiological impression is not pulmonary hydatid disease. These two groups were evaluated in terms of symptoms, radiographical presentation and laboratory tests. The presence of cystic appearance, water-lily sign, ring enhancement concomitant with intact cysts unanimously led the radiologists to the diagnosis of complicated pulmonary hydatid cyst, whereas solid appearance and presence of bronchial obliteration made the diagnosis unlikely. In such circumstances patient history, laboratory findings and bronchoscopic evaluation helped the diagnosis. In conclusion, in endemic regions like Turkey, atypical radiological presentation of complicated pulmonary hydatid disease should be considered in the differential diagnosis of solid pulmonary lesions.     

Management of polycystic liver disease.

Polycystic liver disease (PCLD) is characterized by multiple cysts throughout the liver. Patients may develop chronic intractable symptoms that may be debilitating. Others may develop medical complications that necessitate intervention. There is a variety of nonsurgical and surgical treatment options for symptomatic or complicated PCLD, which range from cyst aspiration and fenestration to liver transplantation. Studies have described variable efficacy and morbidity. Currently, there are no guidelines for the management of PCLD patients and the optimal intervention is controversial. This article reviews the pathogenesis, classification and spectrum of treatment options for PCLD.     

Large solitary retroperitoneal echinococcal cyst： A rare case report

Echinococcal disease remains a problem within some endemic areas. Echinococcal cysts usually involve the liver and lungs, but any other organ can potentially be involved. Extrahepatic localization is reported in 14%-19% of all cases of abdominal hydatid disease. We report the case of a large echinococcal cyst localized in the lower pelvis. A 28-year-old woman was admitted to a surgical ward with lower abdominal pain and discomfort lasting for a month. Ultrasonography and computed tomography scanning revealed a large retroperitoneal cystic mass （9 cm × 4 cm） in contact with the left ovary and left ureter. There were no cysts in any other location. Serological tests were positive for Echinococcus. The patient was operated on and the entire cyst was excised intact. Histopathological results confirmed the diagnosis of echinococcosis. Antihelminthics were administered postoperatively and the patient was discharged after 6 d, and is now being closely followed up. Total cystectomy when possible represents the treatment of choice for large extrahepatic echinococcal cysts.     

Polycystic liver diseases

Polycystic liver diseases (PCLDs) are genetic disorders with heterogeneous etiologies and a range of phenotypic presentations. PCLD exhibits both autosomal or recessive dominant pattern of inheritance and is characterized by the progressive development of multiple cysts, isolated or associated with polycystic kidney disease, that appear more extensive in women. Cholangiocytes have primary cilia, functionally important organelles (act as mechanosensors) that are involved in both normal developmental and pathological processes. The absence of polycystin-1, 2, and fibrocystin/polyductin, normally localized to primary cilia, represent a potential mechanism leading to cyst formation, associated with increased cell proliferation and apoptosis, enhanced fluid secretion, abnormal cell–matrix interactions, and alterations in cell polarity. Proliferative and secretive activities of cystic epithelium can be regulated by estrogens either directly or by synergizing growth factors including nerve growth factor, IGF1, FSH and VEGF.The abnormalities of primary cilia and the sensitivity to proliferative effects of estrogens and different growth factors in PCLD cystic epithelium provide the morpho-functional basis for future treatment targets, based on the possible modulation of the formation and progression of hepatic cysts.     

Levovist ultrasonography imaging in intracystic hemorrhage of simple liver cyst

The differential diagnosis between intracystic hemorrhage and cystadenocarcinoma of the liver is often difficult even with the use of various imaging modalities. A 73-year-old woman was admitted to our hospital with the complaint of right upper quadrant pain. Ultrasonography （US） demonstrated a heterogeneous echogenic cyst measuring 11 cm × 8 cm in size in S2 of the liver, indicated intracystic hemorrhage of simple liver cyst or cystadenocarcinoma, but the differential diagnosis was considerably difficult. Levovist （Schering, Berlin, Germany） US revealed no enhancement of the intracystic structures, suggesting a clot in the case of intracystic hemorrhage. An operation was performed and the resected lesion showed a solitary benign liver cyst, measuring 5.5 cm × 4.7 cm × 8.5 cm containing a large blood clot. The patient had an uneventful recovery after the surgery. Levovist US may play an important role in discrimination between intracystic hemorrhage of simple hepatic cysts and cystadenocarcinoma of the liver.