Portal Dynamics After Living Donor Liver Transplant in Pediatric Recipients: A 10-Year Follow-Up Retrospective Study.

BACKGROUND: It may be difficult for pediatric patients to evaluate the impact of liver transplantation (LT) on splenomegaly due to the natural growth course. The long-term dynamics of portal vein (PV) size and PV flow after LT in pediatric patients are unclear. We aimed to evaluate the long-term transition of the splenic size, PV size, and PV flow velocity in pediatric patients who underwent successful living donor liver transplantation (LDLT) and survived >10 years. METHODS: From October 2004 to December 2010, 39 pediatric patients (25 boys; 14 girls) underwent LDLT, received pre-LDLT and post-LDLT computed tomography scans and long-term ultrasound sonography follow-up, and survived >10 years without additional intervention at our institution. We analyzed the short- to mid-term and long-term impact of LDLT on splenic size, PV size, and PV flow velocity over time. RESULTS: The PV diameter increased throughout the 10-year follow-up (P < .001). The PV flow velocity increased 1 day after LDLT (P< .001); proceeded to decrease 3 days after LDLT, reaching a low point 6 to 9 months after LDLT; and remained stable throughout the 10-year follow-up. Regression of the splenic volume at 6 to 9 months after LDLT (P < .001) was noted. However, the splenic size steadily increased on long-term follow-up. CONCLUSIONS: Although LDLT has a significant short-term reduction effect on splenomegaly, the long-term transitional trend of the splenic size and PV diameter may increase along with children's growth. The PV flow reached a stable status 6 to 9 months after LDLT and remained so until 10 years after LDLT.

White matter hyperintensities in dementia with Lewy bodies are associated with poorer cognitive function and higher dementia stages.

Purpose: White matter hyperintensities (WMHs) are frequently found in elderly individuals with or without dementia. However, the association between WMHs and clinical presentations of dementia with Lewy bodies (DLB) has rarely been studied. Methods: We conducted a retrospective analysis of patients with DLB registered in a dementia database. WMHs were rated visually using the Fazekas scale, and its associated factors including dementia severity, cognitive functions, neuropsychiatric symptoms, and core clinical features were compared among different Fazekas scores. Domains in the Clinical Dementia Rating (CDR), Cognitive abilities Screening Instruments (CASI), and Neuropsychiatric Inventory (NPI) were compared among different Fazekas groups after adjusting for age, sex, education, and disease duration. Results: Among the 449 patients, 76, 207, 110, and 56 had Fazekas score of 0, 1, 2, and 3, respectively. There was a positive association between dementia severity and WMHs severity, and the mean sums of boxes of the Clinical Dementia Rating (CDR-SB) were 5.9, 7.8, 9.5, and 11.2 (f = 16.84, p < 0.001) for the Fazekas scale scores 0, 1, 2, and 3, respectively. There was a negative association between cognitive performance and WMHs severity, and the mean CASI were 57.7, 45.4, 4.06, and 33.4 (f = 14.22, p < 0.001) for the Fazekas scale scores 0, 1, 2, and 3, respectively. However, WMHs were not associated with the core clinical features of DLB. After adjustment, all cognitive domains in CDR increased as the Fazekas score increased. In addition, performance on all cognitive domains in CASI decreased as the Fazekas score increased (all p < 0.001). Among neuropsychiatric symptoms, delusions, euphoria, apathy, aberrant motor behavior, and sleep disorders were significantly worse in the higher Fazekas groups compared to those in the group with Fazekas score of 0 after adjustment. Conclusion: WMHs in DLB might contribute to deterioration of cognitive function, neuropsychiatric symptoms, and dementia stages. However, core clinical features were not significantly influenced by WMHs in DLB.

The value of eZIS analysis of Tc-99m ECD SPECT on identifying cerebellar hypoperfusion in a patient with superficial siderosis: A case report.

INTRODUCTION: Brain perfusion single photon computed tomography (SPECT) is a functional imaging modality and has been widely utilized in evaluation of various kinds of neurological disorders. Easy z-score imaging system (eZIS) is a computer-assisted statistical analysis, based on the comparison with age-classified ethyl cysteinate dimer (ECD) normal database, which provides objectively interpretation of Tc-99m ECD brain perfusion SPECT.Here we presented a 64-year-old male with dizziness, spin sensation, nausea, and vomiting in the emergency room, and brain computed tomography scan showed only small hypodensity lesion in cerebellum. Tc-99m ECD SPECT was performed for evaluating occult cerebral ischemia, infarction, and/or degeneration, but no remarkable abnormality could be identified by experienced readers on conventional display. The result of eZIS showed remarkable hypoperfusion in cerebellum and mild hypoperfusion in bilateral frontal and parietal lobes. Magnetic resonance imaging (MRI) confirmed severe atrophy of anterior cerebellar lobe. In addition, MRI showed diffuse hypointensity signals along with cerebrospinal fluid spaces, especially those areas with hypoperfusion on SPECT, compatible with typical appearances of superficial siderosis. CONCLUSION: This presented case demonstrates the value of software analysis with eZIS on enhancing the diagnostic value of brain perfusion SPECT for detecting brain lesions at an uncommon location due to a rare disease.

Clinical Impact of Gadoxetic Acid-Enhanced Magnetic Resonance Imaging on Hepatoma Management: A Prospective Study.

BACKGROUND: For patients with hepatocellular carcinoma (HCC), gadoxetic acid-enhanced magnetic resonance imaging (EOB-MRI) improved the diagnosis, migrated Barcelona Clinic Liver Cancer (BCLC) stage, and changed therapeutic decision in retrospective analysis. AIM: This prospective study was to evaluate the clinical impact of EOB-MRI on HCC management. METHODS: From September 2012 to February 2014, consecutive patients with suspicion of HCC in BCLC early stage by multidetector computed tomography or dynamic MRI with non-specific gadolinium, well liver function reserve, and admitted for resection evaluation were enrolled prospectively. Additional EOB-MRI was performed. The HCC diagnosis, BCLC staging, and treatment decision were obtained in a liver cancer conference. EOB-MRI impact on HCC management was analyzed. RESULTS: One hundred and three patients including 68 with typical and 35 with atypical HCC nodules in dynamic imaging studies were enrolled. EOB-MRI characterized 3 (4.4 %) benign and 33 (94.3 %) HCC for patients with typical and atypical HCC nodules, respectively. For 90 HCC patients, additional EOB-MRI changed BCLC stage in 25 (27.8 %) and treatment decision in 17 (18.9 %) patients. There were 66 patients with 78 resected nodules including 65 HCCs, 4 intrahepatic cholangiocarcinomas, and 9 benign nodules. Dynamic study and EOB-MRI detected and characterized 69 and 77 nodules, respectively. The sensitivity and accuracy in HCC diagnosis were 98.5 and 85.7 % for EOB-MRI, which were better than those of dynamic study (p < 0.001). CONCLUSIONS: Additional EOB-MRI improved HCC diagnosis in sensitivity, accuracy but not specificity. It changed BCLC staging and treatment decision in 27.8 and 18.9 % of early-stage HCC patients.

Interventional radiology in living donor liver transplant.

The shortage of deceased donor liver grafts led to the use of living donor liver transplant (LDLT). Patients who undergo LDLT have a higher risk of complications than those who undergo deceased donor liver transplantation (LT). Interventional radiology has acquired a key role in every LT program by treating the majority of vascular and non-vascular post-transplant complications, improving graft and patient survival and avoiding, in the majority of cases, surgical revision and/or re-transplant. The aim of this paper is to review indications, diagnostic modalities, technical considerations, achievements and potential complications of interventional radiology procedures after LDLT.

Section 1. Image evaluation of fatty liver in living donor liver transplantation.

Preoperative evaluation of donors for living-donor liver transplantation aims to select a suitable donor with optimal graft quality and to ensure donor safety. Hepatic steatosis, a common finding in living liver donors, not only influences the outcome of liver transplantation for the recipient but also affects the recovery of the living donor after partial hepatectomy. Histopathologic analysis is the reference standard to detect and quantify fat in the liver, but it is invasive, and results are vulnerable to sampling error. Imaging can be repeated regularly and allows assessment of the entire liver, thus avoiding sampling error. Selection of appropriate imaging methods demands understanding of their advantages and limitations and the suitable clinical setting. This article describes potential clinical applications for liver fat quantification of imaging methods for fat detection and quantification, with an emphasis on the advantages and limitations of ultrasonography, computed tomography, and magnetic resonance imaging for quantifying liver fat.

Section 8. Management of portal venous complications in pediatric living donor liver transplantation.

Portal vein (PV) complications after living donor liver transplant (LDLT) have been a major concern in pediatric liver transplantation. The incidence of PV complications is more in pediatric (0%-33%) than in adult recipients. Early diagnosis and treatment of PV complications may ensure optimal graft function and good recipient survival. Small preoperation PV size (<4 mm) and slow portal flow (<10 cm/s) combined with lower hepatic artery resistance index (<0.65) are strong warning signs that may predict the development of post LDLT PV complications. Portal vein angioplasty/stenting is conventionally performed through the percutaneous transhepatic approach; however, this can also be performed through transjugular, trans-splenic, and intraoperative approaches. Depending on the situation, using optimal method is the key point to minimize complication (5%) and gain high success rate (80%). PV occlusion of greater than 1 year with cavernous transformation seems to be a factor causing technical failure. Good patency rate (100%) with self-expandable metallic stents was noted in long-term follow-up. In conclusion, PV stent placement is an effective, long-term treatment modality to manage PV complications after pediatric LDLT. Early diagnosis and treatment are essential to maximize the use of stent placement and achieve good success rates.

Asymptomatic metastasis to the larynx detected by FDG PET/CT in a patient with recurrent rectal adenocarcinoma.

Metastasis of colorectal adenocarcinoma to the larynx is a very rare condition. Here, we report a 72-year-old woman with a history of rectal adenocarcinoma. She was referred for whole-body FDG PET/CT scanning because of an elevating serum level of carcinoembryonic antigen. PET images showed focally increased FDG accumulation in the larynx (SUVmax=12.9). Coregistered CT images showed mild wall thickening at the left subglottic area. The findings of after excisional biopsy confirmed that this FDG-avid lesion was metastatic adenocarcinoma of the colorectal origin.

Intra-operative management of low portal vein flow in pediatric living donor liver transplantation.

For pediatric living donor liver transplantation, portal vein complications cause significant morbidity and graft failure. Routine intra-operative Doppler ultrasound is performed after graft reperfusion to evaluate the flow of portal vein. This retrospective study reviewed 65 children who had undergone living donor liver transplantation. Seven patients were detected with suboptimal portal vein flow velocity following vascular reconstruction and abdominal closure. They underwent immediate on-table interventions to improve the portal vein flow. Both surgical and endovascular modalities were employed, namely, graft re-positioning, collateral shunt ligation, thrombectomy, revision of anastomosis, inferior mesenteric vein cannulation, and endovascular stenting. The ultrasonographic follow-up assessment for all seven patients demonstrated patent portal vein and satisfactory flow. We reviewed our experience on the different modalities and proposed an approach for our future intra-operative management to improve portal vein flow at the time of liver transplantation.

Regeneration and outcome of dual grafts in living donor liver transplantation.

In living donor liver transplantation (LDLT), the essential aims are to provide an adequate graft volume to the recipient and to keep a sufficient remnant liver volume in the donor. In some instances, these aims cannot be met by a single donor and LDLT using dual grafts from two donors is a good solution. From 2002 to 2009, five recipients in our hospital received dual graft LDLT. Two recipients received one right lobe and one left lobe grafts; the other three received two left lobe grafts. The mean final liver regeneration rate was 91.2%. Left lobe graft atrophy in the long term was observed in recipients who received a right and a left lobe grafts. The initial bigger volume graft in all recipients was noted to have better regeneration than the smaller volume grafts. Portal flow and bilateral grafts volume size discrepancy were considered as two major factors influencing graft regeneration in this study. We also noted that the initial graft volume correlated with portal flow in the separate grafts and finally contribute to individual graft regeneration. Because of compensatory hypertrophy of the other graft, recipients who experienced atrophy of one graft did not show signs of liver dysfunction.

Segmental regeneration in right-lobe liver grafts in adult living donor liver transplant.

Our aim is to evaluate the relationship and impact of right-lobe (RL) liver grafts procured with or without the middle hepatic vein (MHV) trunk and MHV tributary reconstruction on segmental regeneration of these grafts in adult living donor liver transplantation (ALDLT). Patients underwent primary ALDLT using a RL liver graft were divided into three groups according to graft type: with MHV tributary reconstruction (group I), without MHV tributary reconstruction (group II), and with inclusion of the MHV trunk (group III). The overall graft volume and the volumes of the anterior and posterior segments of the grafts six months post-transplant, evaluated using computed tomography, were calculated as the regeneration indices. Optimal regeneration of the RL liver graft was achieved in the three groups of patients. There was no significant difference in the regeneration indices between groups I (149.4%) and III (143.6%). However, in group II (112.4%) without MHV or tributary reconstruction, the anterior regenerative index was lower than the other two groups and exhibited transient prolonged hyperbilirubinemia. Segmental graft regeneration is maximized by adequate venous drainage. Inclusion of the MHV trunk or MHV tributary reconstruction influences segmental liver regeneration and preclude transient hyperbilirubinemia in the early post-liver transplant phase.

Magnetic resonance cholangiography in living donor liver transplantation.

OBJECTIVE: Exact knowledge of biliary anatomy is essential in living donor liver transplantation. The purpose of this study was to evaluate the accuracy of pretransplant magnetic resonance cholangiography (MRC) in depicting the biliary anatomy in comparison with intraoperative cholangiography (IOC). MATERIALS AND METHODS: From May 2006 to July 2009, 451 potential living liver donors underwent pretransplant evaluation at the Chang Gung Memorial Hospital-Kaohsiung Medical Center. Two hundred thirty-three donors underwent donor hepatectomy. Of these, only 203 donors with both preoperative MRC and IOC were included in this study. RESULTS: Of the 451 potential donors, 218 (48.3%) were considered unsuitable for liver donation, hence was immediately disqualified after the initial evaluation for various reasons. Six of the 218 unsuitable donors (2.8%) were excluded due to complicated biliary anatomy. The overall accuracy rate of MRC for defining the biliary anatomy was 91.6%, with 84.9% sensitivity, 96% specificity, 88.2% positive predictive value, and 94.7% negative predictive value. There were 14 misidentified cases. The errors in MRC reading was largely attributed to the poor contrast between the biliary ducts and the surrounding tissues and organs. The concurrence between MRC and IOC were commendable (κ=0.9). No significant biliary complications were noted in the mismatch group. CONCLUSION: MRC is essential for all pretransplantation evaluation with 91.6% accuracy rate.

Portal vein thrombosis in biliary atresia patients after living donor liver transplantation.

BACKGROUND: Vascular complications are major complications after living donor liver transplantation (LDLT), especially in biliary atresia (BA). Early diagnosis and treatment of portal vein thrombosis (PVT) may ensure optimal graft function and good recipient survival. Our aim was to find any association between pre- and post-transplant anatomic characteristics and hemodynamics, and the occurrence of post-transplant PVT in BA patients undergoing LDLT. METHODS: We evaluated the pre- and post-transplant findings in ultrasonography in 105 BA recipients who underwent primary LDLT to determine the possible associated factor(s) and ultrasonographic warning sign(s) that may cause PVT development after LDLT. RESULTS: There were 53 male and 52 female recipients. The mean age, height, and weight were 2.8 years, 83.6 cm, and 12.5 kg, respectively. Occlusion of the portal vein (PV) developed in 8 patients. On multivariate analysis, the only independent, pretransplant risk factor predisposing to PV occlusion post-transplant was small main PV size (P = .008). Post-transplant ultrasonographic warning signs included PV flow <10 cm/sec (P = .003), high hepatic artery flow >70 cm/sec (P = .027), and lesser hepatic artery resistance index <0.65 (P = .013). Both slow PV flow <10 cm/sec and lesser hepatic artery resistance index have high sensitivity and specificity in predicting post-LDLT PVT. CONCLUSION: Small PV size (<4 mm) and slow portal flow <10 cm/sec combined with lesser hepatic artery resistance index (<0.65) are strong warning signs that may predict the development of post-LDLT PVT in BA patients that require close monitoring.

Hepatic arterial embolization for massive bleeding from an intrahepatic artery pseudoaneurysm using N-butyl-2-cyanoacrylate after living donor liver transplantation.

Parenchymal pseudoaneurysm of the hepatic arteries with massive intraperitoneal bleeding is rare but a serious life-threatening complication when it occurs following liver transplantation. We report a case of an adult postliving donor liver transplant recipient who developed massive subcapsular bleeding combined with massive right pleural effusion from ruptured multiple small intrahepatic arteries, which developed from a pseudoaneurysm that was treated by hepatic arterial embolization. Successful embolization was performed via a percutaneous trans-catheter approach by depositing 20-25%N-butyl-2-cyanoacrylate (NBCA) through the multiple small intrahepatic arteries into the pseudoaneurysm. Complete occlusion of the feeding arteries and pseudoaneurysm cavity resulted to immediate cessation of bleeding. There was no re-bleeding; and normal liver graft function was noted postembolization. Hepatic arterial embolization with NBCA can be used as treatment for postliver transplant peripheral intrahepatic artery pseudoaneurysm bleeding.

Radiofrequency ablation of small hepatocellular carcinoma with intravenous pegylated liposomal doxorubicin.

PURPOSE: Synergy between radiofrequency ablation (RFA) and chemotherapy was demonstrated for liver malignancy. We assess the efficacy of intravenous pegylated liposomal doxorubicin (PLD) for RFA in patients with small hepatocellular carcinoma (HCC). METHODS: This study was designed as a non-randomized control trial. Patients received either PLD (20 mg) intravenously before RFA, or standard RFA alone. Computed tomography was performed immediately and 4 weeks after RFA to obtain ablative diameter, area and volume for each tumor. The changes in ablation size were analyzed by paired images for each tumor. All patients were followed up regularly. RESULTS: A total of 24 patients with 29 HCCs, including 12 patients with 16 tumors (mean 2.2 cm ± 0.9) in the PLD and RFA group, and 12 patients with 13 tumors (2.4 cm ± 0.5) in the RFA alone group, were enrolled. The ablative diameter, area and volume significantly decreased 4 weeks after RFA. The ablative volume decrease was significantly greater for the RFA alone group than for the combination group (26.1 vs. 12.1%, p = 0.018). The 3-year cumulative tumor progression and survival rates did not differ significantly between the two groups. CONCLUSION: Intravenous PLD before RFA reduced contraction of ablative volume and might have no impact on tumor progression and survival in patients with small HCC after RFA.

Alternative option in the treatment of very small ruptured intracranial aneurysms.

BACKGROUND: To evaluate the safety and protective effect of relative undersized coil with loose coil core in the clinical dilemma condition--very small (43.0 mm) ruptured intracranial aneurysm. METHODS: We studied 12 patients (4 men, 8 women) who had suffered from acute ruptured small intracranial aneurysms (2-3 mm in size, with SAH presentation). All subjects underwent a single coil embolization procedure. An undersized coil (equivalent to the neck size or 0.5 mm smaller than the aneurysm diameter) was chosen as the embolizer. Based on the postembolization angiogram, subjects were divided into 2 groups. One was labeled as the initial complete obliteration group (NR) and the other as the incomplete obliteration group (SR). Fisher exact test and the Wilcoxon rank sum test were used to for statistical analysis. RESULTS: The technical success rate was 100% without any procedure-related complication. The follow-up interval ranged from 6 to 32 months. No episode of rebleeding or coil migration could be defined in the admissive and following period. Loose coil core were seen in all patients. The total recurrent rate was 8.3% (1/12); only one patient suffered from recurrent lesion in SR group. CONCLUSION: The preliminary result showed that under-sized coil packing with loose coil core could provide the protective effect and prevent from further rebleeding for very small ruptured aneurysms. It should be considered as an alternative option in the treatment of acute ruptured very small aneurysms when other conventional strategies are not feasible.

Sonographic findings in esophageal achalasia.

BACKGROUND: There are only three reports using conventional sonography to detect the gastroesophageal junction through the left lobe liver window in patients with achalasia, and the results were inconsistent. This study further characterizes the sonographic features of achalasia and compares them to characteristics of subjects with gastroesophageal malignancies. METHODS: Conventional sonography was performed in 21 patients with achalasia (mean age 49.0 years, 11 male and 10 female, group A); 15 patients with malignancies at the gastroesophageal junction (n=10) and cardiac of the stomach (n=5) (mean age 55.3 years, 11 male and 4 female, group B); and 30 subjects with functional dyspepsia (mean age: 38.3 years, 15 male and 15 female, control group C). RESULTS: The median esophageal wall thicknesses were 5.1+/-2.3 mm (group A), 19.5+/-7.8 mm (group B), and 3.3+/-1.2 mm (group C). However, there was overlap in the esophageal wall thickness in groups A and C. Sonographic features in group A were regular hypoechoic thickening of the wall at the gastroesophageal junction; in group B, we found irregular hypoechoic thickening of the wall. Control subjects had a regular hypoechoic gastroesophageal wall. Dilated lumens of the distal esophagus were seen in all achalasia patients. CONCLUSION: Although conventional sonography is not a diagnostic tool for achalasia, it provides interesting sonographic information. It cannot reveal each layer of the wall of the lumen as endoscopic ultrasound does, but it may tentatively differentiate achalasia from malignancies and assists clinicians when endoscopic ultrasound is not available.