First case of cross-auxiliary double domino donor liver transplantation

We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the firststage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava(IVC). The orifices of the left, middle, and right hepatic veins were reconstructed using an iliac vein patch and then anastomosed to the right side of the IVC. Thirty days later, a second domino liver graft was implanted. The second domino graft was from a 3-yearold female child with an ornithine carbamyl enzyme defect, and it replaced the residual native liver(left lobe). To balance the function and blood flow between the two grafts, a percutaneous transcatheter selective portal vein embolization was performed, and "the left portal vein" of the first graft was blocked 9 mo after the second transplantation. The liver function indices, blood ammonia, and 24-h urinary copper levels were normal at the end of a 3-year follow-up. These two domino donor grafts from donors with different metabolic disorders restored normal liver function. Our experience demonstrated a new approach for resolving metabolic disorders with domino grafts and utilizing explanted livers from children.

Multimodality-imaging manifestations of primary renal-allograft synovial sarcoma:First case report and literature review

BACKGROUND Primary renal synovial sarcoma (PRSS) is an extremely rare tumor with a poor prognosis. Its imaging and immunohistochemical characteristics may overlap with other renal tumors, which renders its early diagnosis in a dilemma. The diagnosis of primary renal synovial sarcoma requires histopathology and the confirmation of SYT-SSX gene fusion using molecular techniques. Cases of primary renal synovial sarcoma have been previously reported in the literature. However, to our knowledge, primary renal allograft synovial sarcoma was never described. CASE SUMMARY A 43-year-old male patient who underwent kidney transplantation 9 months ago came to our hospital for regular follow-up. Traditional ultrasonography revealed multiple hypo-echo neoplasms in the renal allograft. Contrast-enhanced computed tomography (CECT) showed slightly hyper-density masses with slow homogeneous enhancement. Ultrasound-guided biopsy was conducted for accurate pathological diagnosis. The neoplasms were diagnosed as synovial sarcoma by pathological, immunohistochemical, and genetic analyses. Positron emission tomography/CT showed no evidence of metastasis. At approximately one week post biopsy, contrast-enhanced ultrasound was conducted to eliminate active hemorrhage. One month later, CECT showed that the biggest neoplasm grew from 3.3 cm to 5.7 cm in diameter. Parametric imaging was conducted with SonoLiver CAP to conduct further quantitative analysis, which showed that the enhancement pattern was heterogeneous hyper-vascular enhancement. Radical surgical resection of the whole renal allograft and ureter was conducted without additional adjuvant chemotherapy or external radiotherapy. Anlotinib was chosen for targeted therapy with a good response. CONCLUSION We propose multimodality imaging for accurate diagnosis of renal allograft synovial sarcoma especially when it is formed by spindle-shaped cells.

Diagnosis and spontaneous healing of asymptomatic renal allograft extra-renal pseudo-aneurysm:A case report

BACKGROUND Transplant renal artery stenosis is a relatively frequent vascular complication after transplantation.However,extra-renal pseudo-aneurysms(EPSAs)are rare after transplantation;they can be life-threatening and usually need open surgical repair.We discuss the diagnosis and spontaneous healing of an asymptomatic renal allograft EPSA caused by renal artery anastomotic stenosis,which was diagnosed in a timely manner and managed by conservative treatments.CASE SUMMARY We present a 37-year-old male patient diagnosed with a renal allograft EPSA caused by renal artery anastomotic stenosis due to multiple atherosclerotic plaques with ultrasonographic examination 6 mo post transplantation.The stenosis rate of 90%and the EPSA were verified by computed tomography angiography.The diagnosis was further confirmed with digital subtraction angiography.Percutaneous transluminal angiography was conducted,and a metallic stent was successfully implanted at the stenosed site of the main renal artery trunk.No further intervention for the EPSA was undertaken due to the difficulty of stenting and the risk of bleeding;regular ultrasonographic follow-ups were recommended.The stenosis was significantly relieved immediately after stent implantation and the EPSA was healed spontaneously by completely filling with hypo-echoic thrombosis 8 mo after stenting.CONCLUSION Ultrasonography combined with a high-frequency linear probe can detect vascular complications post renal transplantation at an early stage and improve prognosis.

Perioperative characteristics and management of liver transplantation for isolated methylmalonic acidemia—the largest experience in China

Background:There are few detailed consensus and guidelines on perioperative clinical characteristicsof liver transplantation(LT)in patients with methylmalonic acidemia(MMA).This retrospective studyinvestigated details of the clinical course and individualized treatment plan of the center with largestexperience in China.Methods:A total of 7 MMA patients undergoing LT in Beijing Friendship Hospital from June 2013 toDecember 2017 were enrolled in the study,whose clinical data(clinical characteristics,laboratory findings,chronological changes in urine MMA levels,treatment,etc.)during perioperative period were analyzedretrospectively.All the patients received strict postoperative management.Results:All the 7 cases were confirmed to have isolated MMA,among which,3 cases received livingdonor liver transplantation(LDLT),4 cases received deceased donor liver transplantation(DDLT).A wildfluctuate of metabolic condition was observed within the first few days after surgery and two weeks afterLT,the mean base excess of blood value(BE-B)restored to normal whereas plasma bicarbonate(HCO3-)was still below normal value even with intermittent sodium bicarbonate correction.It also showed markedreduction in propionylcarnitine(C3)and C3/C2 level and the mean urine MMA by gas chromatographymassspectrometry(GC-MS)was reduced by 81.7%(P<0.01)but remained>72×higher than upper limitof normal.The metabolism-correcting medications were administered as before.The renal function ofone case with renal insufficiency before LT(serum creatinine rising)maintained stable by adjusting theimmunosuppressive regimen during the observation period.All patients survive to date.Conclusions:LT is an effective treatment to prevent metabolic crisis,but patients with MMA tend to bemetabolically fragile even after surgery.During perioperative period,close monitoring should be given foracidosis episodes so as to implement sodium bicarbonate correction.Metabolism-correcting medications arestill needed.Special immunosuppressive regimen is an effective way of maintaining renal function for thosewith kidney dysfunction.