Right superior resection (segments 7 and 8) is an uncommon resection for liver malignancies, with most of the literature limited to case reports and small series. Resection of segments 4, 7, and 8 has been reported in only a few cases. When the right hepatic vein is resected, venous reconstruction or identification of one or more right inferior hepatic veins is considered mandatory, to maintain segmentary function of segments 5 and 6. We present a case of liver resection of segments 4, 7, and 8 including the right and middle hepatic veins for symptomatic benign liver disease with no right hepatic vein reconstruction, nor a prominent right inferior hepatic vein(s). After the resection, there was no change in liver function tests, and the patient made an unremarkable recovery. Three months after the operation, partial atrophy of segments 5 and 6 with hypertrophy of the left lateral section was observed, while two and one half years after resection, the patient is asymptomatic. When right hepatic vein reconstruction would add unnecessary operative time, and there is low likelihood of the need for repeated resection, particularly when the hepatic vein is difficult to dissect, this approach can be safe and useful, while providing an adequate postoperative liver mass in the short-term to recover uneventfully from major liver resection.

Intra-abdominal hemorrhage resulting from a ruptured, large hepatic cyst in a polycystic liver disease (PCLD) patient is rare and potentially fatal if not addressed promptly. Only a few isolated cases have previously been reported. The usual patient profile consists of elderly patients on anticoagulation, as is demonstrated in our case. Intra-hepatic cysts are broadly classified into congenital, traumatic, infectious, parasitic, and neoplastic. Congenital intra-hepatic cysts can consist of both simple and PCLD, as is outlined in our case. Simple cysts are usually asymptomatic, but occasionally they may achieve larger dimensions and lead to complications such as rupture, obstruction, infection, hemorrhage, and even portal hypertension. We present an uncommon case of a 78-year-old patient with PCLD on rivaroxaban who presented initially with diffuse abdominal pain, distension, and progression into hemodynamic instability. A computerized tomography (CT) scan revealed a ruptured left hepatic lobe cyst, causing hemoperitoneum and resulting in an acute abdomen. This case was complicated by the patient\'s anticoagulation status and anomalous hepatic vasculature pattern. Interventional radiology (IR) successfully identified the aberrant bleeding vessel and stopped the active extravasation with super-selective coil embolization.

UNASSIGNED: Timely recognition, accurate diagnosis, and proper management are vital for preventing complications and improving outcomes in polycystic liver disease.
UNASSIGNED: Polycystic liver disease is an uncommon genetic condition characterized by the presence of over 20 liver cysts. It is symptomatic in only 5% of cases. Surgical intervention remains the primary treatment approach for managing symptoms in affected patients. Herein, we report a case of PLD revealed by severe abdominal pain.

Polycystic liver disease (PLD) is a rare condition observed in three genetic diseases, including autosomal dominant polycystic liver disease (ADPLD), autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). PLD usually does not impair liver function, and advanced PLD becomes symptomatic when the enlarged liver compresses adjacent organs or increases intra-abdominal pressure. Currently, the diagnosis of PLD is mainly based on imaging, and genetic testing is not required except for complex cases. Besides, genetic testing may help predict patients\' prognosis, classify patients for genetic intervention, and conduct early treatment. Although the underlying genetic causes and mechanisms are not fully understood, previous studies refer to primary ciliopathy or impaired ciliogenesis as the main culprit. Primarily, PLD occurs due to defective ciliogenesis and ineffective endoplasmic reticulum quality control. Specifically, loss of function mutations of genes that are directly involved in ciliogenesis, such as Pkd1, Pkd2, Pkhd1, and Dzip1l, can lead to both hepatic and renal cystogenesis in ADPKD and ARPKD. In addition, loss of function mutations of genes that are involved in endoplasmic reticulum quality control and protein folding, trafficking, and maturation, such as PRKCSH, Sec63, ALG8, ALG9, GANAB, and SEC61B, can impair the production and function of polycystin1 (PC1) and polycystin 2 (PC2) or facilitate their degradation and indirectly promote isolated hepatic cystogenesis or concurrent hepatic and renal cystogenesis. Recently, it was shown that mutations of LRP5, which impairs canonical Wnt signaling, can lead to hepatic cystogenesis. PLD is currently treated by somatostatin analogs, percutaneous intervention, surgical fenestration, resection, and liver transplantation. In addition, based on the underlying molecular mechanisms and signaling pathways, several investigational treatments have been used in preclinical studies, some of which have shown promising results. This review discusses the clinical manifestation, complications, prevalence, genetic basis, and treatment of PLD and explains the investigational methods of treatment and future research direction, which can be beneficial for researchers and clinicians interested in PLD.

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BACKGROUND: Enterococcus casseliflavus is a rare pathogenic bacterium that is characterized by vancomycin resistance and can lead to multiple infections in the human body. This report describes a rare case of polycystic intrahepatic infection with E. casseliflavus which necessitated antibiotic treatment and surgical intervention involving cystic drainage.
METHODS: A 59-year-old woman, a long-term hemodialysis patient, was hospitalized due to a 5-day history of fever, abdominal pain, and diarrhea, which were possibly caused by the ingestion of contaminated food. Her blood culture yielded a positive result for E. casseliflavus, and she was initially treated with piperacillin/tazobactam and linezolid. Later, the antibiotic regimen was adjusted to include meropenem and linezolid. Despite treatment, her body temperature remained elevated. However, subsequent blood cultures were negative for E.casseliflavus.Conventional CT scans and ultrasound examinations did not identify the source of infection. However, a PET-CT examination indicated an intrahepatic cyst infection. Following MRI and ultrasound localization, percutaneous intrahepatic puncture and drainage were performed on the 20th day. Fluoroquinolones were administered for 48 days. On the 32nd day, MRI revealed a separation within the infected cyst, leading to a repeat percutaneous drainage at a different site. Subsequently, the patient\'s temperature returned to normal. The infection was considered resolved, and she was discharged on the 62nd day. Follow-up results have been favorable thus far.
CONCLUSIONS: Based on the findings from this case, it is recommended to promptly conduct PET-CT examination to exclude the possibility of intracystic infection in cases of polycystic liver infection that are challenging to control. Furthermore, timely consideration should be given to puncture drainage in difficult cases.

Persistent symptoms of pain, early satiety, dyspnea, and gastrointestinal reflux due to significant liver enlargement are indications for surgical debulking in patients with polycystic liver disease (PCLD) due to the lack of effective medical therapies; however, few data exist on outcomes of surgical intervention for PCLD.
We conducted a retrospective analysis of consecutive patients who underwent operative intervention due to persistent symptoms secondary to PCLD. Preoperative patient characteristics, 30-day postoperative outcomes, and long-term postoperative outcomes, including complications and symptom resolution, were analyzed.
We identified 50 patients who underwent hepatic resection for symptomatic PCLD. Nine patients (19%) had concomitant polycystic kidney disease, and 14 (28%) had previously undergone interventions for PCLD management. The overall complication rate was 30%, with 8 patients (16%) experiencing Clavien-Dindo Grade III-V complications and no mortalities. The median relative reduction in liver volume was 41%. At a median follow-up of 2 years, 94% has sustained symptom resolution.
This is among the largest case series exploring PCLD operative outcomes, revealing that surgical intervention for debulking for advanced PCLD is safe and effective for symptom management. Furthermore, patients with PCLD undergoing hepatectomy tolerate significant liver volume loss without evidence of impaired hepatic function.

BACKGROUND: Factors related to the development and severity of polycystic liver disease (PLD) have not been well established. We aimed to evaluate the genetic and epidemiologic risk factors of PLD in patients with autosomal dominant polycystic kidney disease (ADPKD).
METHODS: Adult patients with inherited cystic kidney disease were enrolled from May 2019 to May 2021. Demographic, clinical, and laboratory data were collected at the initial study visit. The severity of PLD was graded based on the height-adjusted total liver volume: < 1,000 mL/m (Gr1), 1,000-1,800 mL/m (Gr2), and > 1,800 mL/m (Gr3). Targeted exome sequencing was done by a gene panel including 89 ciliopathy-related genes. We searched out the relative factors to the presence and the severity of PLD using logistic regression analysis.
RESULTS: Of 602 patients with typical ADPKD, 461 (76.6%) patients had PLD. The patients with PLD showed female predominance and a higher frequency of other ADPKD-related complications. The genetic variants with truncating mutation of PKD1 (PKD1-protein-truncating [PT]) or PKD2 commonly affected the development and severity of PLD. An older age, female sex, and higher kidney volume with Mayo classification 1C-1E was significantly associated with the development of PLD, but not with the severity of PLD. On the other hand, higher body mass index, lower hemoglobin, and higher alkaline phosphatase (ALP) were the significant risk factors of severe PLD (≥ Gr2).
CONCLUSIONS: Hepatic involvement in ADPKD could be related to kidney manifestations and genetic variants including PKD1-PT or PKD2. Monitoring hemoglobin and ALP and evaluating the genetic variants might help predict severe PLD.
BACKGROUND: Clinical Research Information Service Identifier: KCT0005580.

A 57-year-old man complained about abdominal distension and pain, constant feeling of early satiety. He was diagnosed with polycystic kidneys at the age of 24 and liver cysts discovered at the age of 38. The CT scan revealed 33 x 21 x 27 cm polycystic liver with cysts up to 7 cm in diameter. In 2009-2019 the patient was repeatedly punctured for liver cysts. Considering the continued enlargement of the liver and the worsening of complaints, the patient was put on the waiting list for a liver transplant in the spring of 2019. The patient went through liver transplantation on 11th of July 2022, the liver measures were 53 x 37 x 39 x 16 cm and weight 14,75 kg. The postoperative course was uneventful. Liver transplantation can be very effective treatment method that significantly improves the quality of life in PLD patients.

Protein-truncating variants in α-1,3-glucosyltransferase (ALG8) are a risk factor for a mild cystic kidney disease phenotype. The association between these variants and liver cysts is limited. We aim to identify pathogenic ALG8 variants in our cohort of autosomal dominant polycystic liver disease (ADPLD) individuals. In order to fine-map the phenotypical spectrum of pathogenic ALG8 variant carriers, we performed targeted ALG8 screening in 478 ADPLD singletons, and exome sequencing in 48 singletons and 4 patients from two large ADPLD families. Eight novel and one previously reported pathogenic variant in ALG8 were discovered in sixteen patients. The ALG8 clinical phenotype ranges from mild to severe polycystic liver disease, and from innumerable small to multiple large hepatic cysts. The presence of <5 renal cysts that do not affect renal function is common in this population. Three-dimensional homology modeling demonstrated that six variants cause a truncated ALG8 protein with abnormal functioning, and one variant is predicted to destabilize ALG8. For the seventh variant, immunostaining of the liver tissue showed a complete loss of ALG8 in the cystic cells. ALG8-associated ADPLD has a broad clinical spectrum, including the possibility of developing a small number of renal cysts. This broadens the ADPLD genotype-phenotype spectrum and narrows the gap between liver-specific ADPLD and kidney-specific ADPKD.