Liver Venous Deprivation for Rapid Liver Hypertrophy Before Major Hepatectomy: A Case Report.

Liver venous deprivation (LVD) is an emerging, minimally invasive strategy to induce rapid liver hypertrophy of the future liver remnant (FLR) before a major hepatectomy. LVD (aka "double vein embolization") entails same-session percutaneous embolization of the portal and hepatic veins of the planned liver resection. This report discusses LVD's utilization and technical challenges in managing a 49-year-old male with recurrent multifocal colorectal liver metastases (CRLM). The patient initially underwent neoadjuvant FOLFOX chemotherapy followed by a simultaneous laparoscopic sigmoid colectomy and liver surgery (microwave ablation of segment V and wedge resections of segment one and IVb), followed by completion of chemotherapy. The patient had an R0 resection with clear colon and liver surgical margins. Nine months after the initial surgery, the patient had a rise in tumor markers, and surveillance imaging demonstrated recurrence of liver metastases in segments I and V. LVD was performed by interventional radiology, which led to a 28% increase in FLR (segments II, III, and IV); initially measuring 464 cm3 before LVD and measuring 594 cm3 on post-procedure day 21. The patient underwent right hemi-hepatectomy and caudate resection on post-procedure day 29. The patient did not have any complications and was discharged on postoperative day 6. The patient remains disease-free with no evidence of recurrence at 12 months follow-up.

Associating liver partition and portal vein ligation for staged hepatectomy as bridging therapy for liver transplantation in an infant with an advanced hepatic rhabdoid tumor.

BACKGROUND: Malignant rhabdoid tumors (MRTs) are rare, aggressive tumors that mainly affect children and currently lack effective chemotherapeutic regimens. Liver MRTs are particularly challenging to manage due to the difficulty of performing one-stage liver resection, and preemptive liver transplantation is associated with high recurrence rates. However, the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) technique offers a promising surgical approach for advanced-stage liver tumors where conventional liver resection is not feasible. CASE REPORT: A patient with a large liver rhabdoid tumor that had invaded the three main hepatic veins underwent four courses of cisplatin-pirarubicin chemotherapy. ALPPS was performed due to insufficient residual liver capacity, with hepatic parenchymal dissection between the anterior and posterior liver zones in the first stage of surgery. After confirming adequate remaining liver volume, the liver was resected except for S1 and S6 on postoperative day 14. LDLT was performed 7 months after ALPPS due to the gradual deterioration of liver function caused by chemotherapy. The patient was recurrence-free 22 and 15 months after ALPPS and LDLT, respectively. CONCLUSIONS: The ALPPS technique is a curative option for advanced-stage liver tumors that cannot be managed with conventional liver resection. In this case, ALPPS was used successfully to manage a large liver rhabdoid tumor. Then, liver transplantation was performed after chemotherapy. The ALPPS technique should be considered a potential treatment strategy for patients with advanced-stage liver tumors, particularly those who can undergo liver transplantation.

Liver and kidney transplantation in polycystic liver and kidney disease. / Trasplante hepático y renal en la enfermedad poliquística hepatorrenal.

OBJECTIVE: To evaluate the results of isolated liver and combined liver and kidney transplantation in a retrospective series of 32 patients with hepatorenal liver and kidney disease. MATERIALS AND METHODS: A retrospective observational study that enrolled patients with polycystic liver disease (PLD) and polycystic liver and kidney disease (PLKD) who were evaluated for transplantation between January 1999 and December 2019 at Hospital Clínic de Barcelona [Clinical Hospital of Barcelona]. RESULTS: We included a total of 53 patients enrolled, 32 (60.3%) had indication for transplantation, of which 12 received a single liver transplant and 20 received a double liver and kidney transplant. The mean age was 52 years and 83.9% of the recipients were women. The main indication for liver transplantation was disabling symptomatic hepatomegaly (93.5%). Among the postoperative complications, in the combined liver and kidney transplant group, hepatic artery thrombosis in one case and renal artery thrombosis in other were detected. In both groups there was one case of inferior vena cava lesion. Three patients presented acute cellular rejection responding to corticosteroids and one presented humoral rejection which was treated with plasmapheresis. During the follow-up period of 80 (27-121) months, the liver transplant survival rate was 100% and the kidney transplant survival rate was 90%. Two patients in the combined liver and kidney transplant group died (one due to cardiovascular causes and the other due to intestinal adenocarcinoma). CONCLUSIONS: Isolated liver transplantation or combined liver and kidney transplantation in selected patients with polycystic disease yields excellent results, with few complications, very good transplant survival and excellent patient survival (93.8%).

Successful hematopoietic stem cell transplantation from an HLA-mismatched parent for engraftment failure after unrelated cord blood transplantation in patients with juvenile myelomonocytic leukemia: Report of two cases.

JMML is an aggressive hematopoietic malignancy of early childhood, and allogeneic HSCT is the only curative treatment for this disease. Umbilical cord blood is one of donor sources for HSCT in JMML patients who do not have an HLA-compatible relative, but engraftment failure remains a major problem. Here, we report two cases of JMML who were successfully rescued by HSCT from an HLA-mismatched parent after development of primary engraftment failure following unrelated CBT. Both patients had severe splenomegaly and underwent unrelated CBT from an HLA-mismatched donor. Immediately after diagnosis of engraftment failure, both patients underwent HSCT from their parent. For the second HSCT, we used RIC regimens consisting of FLU, CY, and a low dose of rabbit ATG with or without TBI and additionally administered ETP considering their persistent severe splenomegaly. Both patients achieved engraftment without severe treatment-related adverse effects. After engraftment of second HSCT, their splenomegaly was rapidly regressed, and both patients showed no sign of relapse for over 4 years. These observations demonstrate that HSCT from an HLA-mismatched parent could be a feasible salvage treatment for primary engraftment failure in JMML patients.

Portal vein aneurysm in thalassaemia.

Arterial aneurysms are more common than visceral venous aneurysms. Portal vein aneurysms being the most common type of visceral venous aneurysms. Here, we present an 18-year-old young woman with thalassaemia major, who presented with headache, palpitation, shortness of breath and a recent increase in blood transfusion rate. On clinical examination, she had hepatosplenomegaly. Ultrasonography revealed hepatosplenomegaly with fusiform dilatation of extrahepatic portal vein, which was confirmed to be portal vein aneurysm on contrast enhanced CT. Though portal vein aneurysms were previously thought to be rare, recently they are increasingly diagnosed with the use of cross-sectional imaging. Recognition of this finding can help to avoid potential confusion with other periportal cystic masses of different aetiologies, especially on sonography.

Liver Transplantation in Patients With Niemann-Pick Disease--Single-Center Experience.

Niemann-Pick disease (NPD) is a rare syndrome caused by abnormal intracellular sphingomyelin lipid storage in cells known as "Pick cells." NPD can start in childhood or develop insidiously, with a predilection for reticuloendothelial cells and the nervous system. NPD is a heterogeneous clinical, and biomolecular disorder which has 6 variants. There is no curable treatment for NPD. Generally, the treatment for all types of Niemann-Pick disease is to support. Type B NPD (NPD-B) is mostly characterized by hepatosplenomegaly, which can evolve to hepatic cirrhosis. In patients who progress to liver failure, liver transplantation may be improve liver function. The Transplant Service of Hospital Universitário Walter Cantídio performed its first liver transplants in patients with NPD-B with good results, demonstrating the efficacy of this procedure in selected cases.

Autoimmune lymphoproliferative syndrome with neonatal onset.

We describe 2 cases of autoimmune lymphoproliferative syndrome (ALPS), which is a rare disorder of auto-immunity, chronic persistent or recurrent lymphadenopathy, splenomegaly, hepatomegaly and hyper gamma globulinemia (1gG, 1gA). Both cases presented in neonatal period which is a rare age of presentation in this disease. A 20 days old female neonate presented with respiratory symptoms which rapidly progressed needing ventilatory support. There was hepatomegaly and no auscultatory findings in the chest. Serial CBCs (complete blood counts) showed persistent leucocytosis with predominant lymphocytosis. Her chest X-ray showed left sided consolidation which responded poorly to antibiotics. Her prompt clinical response to steroids raised the suspicion of autoimmunity and the diagnosis was established after a negative bone marrow examination for leukemia and a positive result for ALPS on flow cytometry. The second case presented with anemia, thrombocytopenia starting in neonatal period followed by persistent lymphadenopathy, hepatosplenomegaly and recurrent infections which responded poorly to antibiotics. Diagnosis was delayed due to low index of suspicion, and finally achieved with multiple radiological studies, histopathology and flow cytometry.

[Neonatal stage IV and IVs neuroblastoma: surgical treatment with silo and liver reduction]. / Neuroblastoma neonatal estadio IV y IVs: Tratamiento quirúrgico con silo y reducción hepática.

UNLABELLED: Hepatic metastases of neonatal neuroblastoma can cause multiple organ failure due to rapid growth and severe hepatomegaly. The objective of this work is to present two infants with neuroblastoma stage IV and IVs in a critical situation that needed a decompressive abdominal silo and surgical liver reduction for definitive abdominal closure. Currently both children are 6 years old and they are free from disease. CONCLUSION: The placement of a silo for abdominal decompression is still an indication for salvage. Surgical hepatic reduction is an alternative for the early closure of the abdomen avoiding complications due to infection or dehiscence of the silo.

[Orthotopic liver transplantation for polycystic liver disease with massive hepatomegaly]. / Orthotope Lebertransplantation bei polyzystischer Leberdegeneration mit massiver Hepatomegalie.

HISTORY: A 51-year-old man (126 kg, 192 cm) with massive hepatomegaly causing cardiopulmonary symptoms was referred to our transplant center 14 years after initial diagnosis of polycystic liver disease. TREATMENT AND COURSE: Uneventful hepatectomy was followed by orthotopic liver transplantation using caval replacement. Donor liver came from a 73-year-old woman (extended criteria donor organ offer). A portocaval shunting was not established during transplantation although the explanted liver weighed 22 kg. 18 months after transplantation liver function is stable and the patient enjoys normal quality of life. CONCLUSION: This case report demonstrates the value and success of transplantation for patients suffering from enormous hepatomegaly due to polycystic liver disease.

Liver transplantation for massive hepatomegaly due to polycystic liver disease: an extreme case.

BACKGROUND: Polycystic liver disease (PLD) is due to a genetic disorder and frequently coexists with polycystic kidney disease (PKD). If the cysts produce symptomatology owing to their number and size, many palliative treatments are available. When none of the liver parenchyma is spared, or kidney insufficiency is marked, the only potentially curable treatment is liver transplantation (LT). CASE REPORT: A 49-year old woman, diagnosed with PLD and PKD, was listed in January 2008 for combined LT and kidney transplantation (KT). A compatible organ became available 8 months later. Despite preserved liver function, the patient's clinical condition was poor; she experienced dyspnea, advanced anorexia, abdominal pain, and severe ascites. At LT, which took 9 hours and was performed using the classic technique, the liver was hard, massive in size (15.5 kg), and not dissociable from the vena cava. The postoperative course was complicated by many septic episodes, the last one being fatal for the patient at 4 months after transplantation. DISCUSSION: LT for PLD in many series shows a high mortality rate. The Model for End-Stage Liver Disease (MELD) score does not stage patients properly, because liver function is usually preserved. The liver can achieve a massive size causing many symptoms, especially malnutrition and ascites; in this setting LT is the only possible treatment. Patients with a low MELD score undergo LT with severe malnutrition that predisposes them to greater susceptibility to sepsis. To identify predictor factors, beyond MELD criteria that relate to the increased liver volume before development of late symptoms is essential to expeditiously treat patients with the poorest prognosis to improve their outcomes.

Peritonitis in myelofibrosis: a cautionary tale.

BACKGROUND: Primary myelofibrosis (PMF) is a myeloproliferative disorder characterized by bone marrow fibrosis. Extra-medullary hematopoiesis sometimes occurs even in the peritoneal cavity, apart from organs such as the liver, spleen, and lymph nodes. This may sometimes be complicated by spontaneous infection and complications. We report a rather unusual case of PMF, who presented as an emergency with spontaneous peritonitis to general surgery department and had a fulminant clinical course. METHOD: A clinical case note review was done and a literature search was undertaken. RESULTS: A rather unusual case of PMF, who presented as an emergency with spontaneous peritonitis to general surgery department. The patient underwent a laparotomy and had a fulminant clinical course. CONCLUSIONS: Peritonitis in myelofibrosis may have a number of causes. Clinicians need to be aware of them and provide conservative management prior to surgical treatment.

Extended liver resection for polycystic liver disease can challenge liver transplantation.

OBJECTIVE: In an attempt to refine the indications for liver resection (LR) for highly symptomatic polycystic liver disease (PLD), we focused on the characteristics, technical difficulties, postoperative outcome, and long-term follow-up of PLD patients who underwent either LR or liver transplantation (LT). METHODS: Since 1995, among 72 patients with massive hepatomegaly, 45 patients underwent LR associated with contralateral cyst fenestration whereas 27 underwent LT associated with simultaneous kidney transplantations in 23. The LR group was characterized by absence of end-stage renal insufficiency, absence of ascites, and better nutrition status. In the LR group, the volumetry of the spared noncystic parenchyma was preoperatively assessed whereas pathological analysis focused on fibrosis and vascular congestion. RESULTS: After LR, the mortality was nil and overall morbidity was 71%, including biliary leak in 20% and ascites in 42%. Persistent and massive ascites was observed in 8 patients who have undergone extensive resection and had significantly more frequently fibrosis on the analysis of their resected surgical specimens (P = 0.002). A volume of the remnant noncystic parenchyma less than 30% and the presence of vascular changes on the specimen were associated with higher risk of complications. The 5-year survival was 95% and among the 43 survivors, after a mean follow-up of 41 months, 36 (83%) patients stated that they were satisfied, with complete relief of symptoms in 30 (70%). After LT, the postoperative mortality was 15% and the overall morbidity was 85%, including 12 patients who required reoperation. Severe complications were more frequent in the presence of denutrition and preoperative ascites. The 5-year survival was 85% and after a mean follow-up of 36 months all survivors had complete relief of symptoms due to hepatomegaly. CONCLUSIONS: In case of massive hepatomegaly from PLD without end-stage renal failure, LR should be considered first when preserved remnant liver volume represents at least 30% of the total volume liver in the absence of vascular changes or fibrosis.

Liver transplantation for polycystic liver and massive hepatomegaly.

Liver tumor and other benign liver diseases such as polycystic liver disease can cause massive hepatomegaly and may represent an indication for liver transplantation (LT) in some instances. In this setting, LT can be extremely difficult and challenging due to its decreased mobility and access to vascular supply. Benefit from either a right or a left partial liver resection during the transplant procedure has been advocated to safely accomplish the hepatectomy of the native liver. Although we believe that partial hepatectomy adds some risk to intra-operative bleeding, we alternatively advise a different approach. We have a successful experience with LT in 6 massive hepatomegaly patients due to giant liver lesions. All the transplant procedures were performed without intermediate partial liver resection, showing that selective use of veno-venous bypass can play a significant role in the treatment of massive hepatomegaly.

Liver transplantation for polycystic liver with massive hepatomegaly: a case report.

A previous study has shown that liver or combined liver-kidney transplantation can be a valuable surgical technique for the treatment of polycystic liver disease. Herein, we present the case of a 35-year-old woman with polycystic liver disease, who underwent orthotopic liver transplantation (OLT) on November 11, 2008. The whole-size graft was taken from a deceased donor (a 51-year-old man who died of a heart attack). Resection in a patient with massive hepatomegaly is very difficult. Thus, after intercepting the portal hepatic vein, left hepatectomy was performed, then the vena cava was intercepted, the second and third porta hepatic isolated, and finally, right hepatectomy was performed. OLT was performed successfully. The recipient did well after transplantation. This case suggested that OLT is an effective therapeutic option for polycystic liver disease and left hepatectomy can be performed first during OLT if the liver is over enlarged.

Reduced-size liver transplantation for glycogen storage disease.

BACKGROUND: Glycogen storage disease (GSD) is an inherited metabolic disorder in which the concentration and/or structure of glycogen in tissues is abnormal. Essentially, abnormalities in all known enzymes involved in the synthesis or degradation of glycogen and glucose have been found to cause some type of GSD. Liver and muscle have abundant quantities of glycogen and are the most common and seriously affected tissues. This study was to assess reduced-size liver transplantation for the treatment of GSD. METHODS: The clinical data from one case of GSD type I with hepatic adenoma was retrospectively analyzed. The clinical manifestations were hepatomegaly, delayed puberty, growth retardation, sexual immaturity, hypoglycemia, and lactic acidosis, which made the young female patient eligible for reduced-size liver transplantation. RESULTS: The patient recovered uneventfully with satisfactory outcome, including 12 cm growth in height and 5 kg increase in weight during 16 months after successful reduced-size liver transplantation. She has been living a normal life for 4 years so far. CONCLUSIONS: Reduced-size liver transplantation is an effective treatment for GSD with hepatomegaly and hepatic adenoma. Delayed puberty, growth retardation, hypoglycemia and lactic acidosis can be cured by surgery.