Dor Yoeli

Portosystemic shunt as a bridge to liver transplantation in infants: A comparison of two techniques

Portosystemic shunts can serve as a bridge to liver transplantation in patients with end‐stage liver disease by providing portal decompression to treat life‐threatening variceal bleeding and prevent recurrent episodes until an organ becomes available. The conventional TIPS procedure, however, is technically challenging to perform in infants due to the small size of their intrahepatic vasculature. We report two cases of emergent creation of portosystemic shunts as a bridge to liver transplantation in infants with life‐threatening variceal bleeding using a conventional TIPS technique in the first case and a percutaneous DIPS technique in the other. Both procedures were successful at reducing the portosystemic pressure gradient and preventing further variceal bleeds until a liver transplant could be performed. The novel percutaneous DIPS procedure is a valuable alternative to the conventional TIPS in infants, as it is better suited for small or challenging intrahepatic vascular anatomy.

Are drowned donors marginal donors? A single pediatric center experience

Drowning, a common cause of death in the pediatric population, is a potentially large donor pool for OLT. Anecdotally, transplant centers have deemed these organs high risk over concerns for infection and graft dysfunction. We theorized drowned donor liver allografts do not portend worse outcomes and therefore should not be excluded from the donation pool. We reviewed our single‐center experience of pediatric OLTs between 1988 and 2015 and identified 33 drowned donor recipients. These OLTs were matched 1:2 to head trauma donor OLTs from our center. A chart review assessed postoperative peak AST and ALT, incidence of HAT, graft and recipient survival. Recipient survival at one year between patients with drowned donor vs head trauma donor allografts was not statistically significant (94% vs 97%, P=.63). HAT incidence was 6.1% in the drowned donor group vs 7.6% in the control group (P=.78). Mean postoperative peak AST and ALT was 683 U/L and 450 U/L for drowned donors vs 1119 U/L and 828 U/L in the matched cohort. These results suggest drowned donor liver allografts do not portend worse outcomes in comparison with those procured from head trauma donors.

Liver transplantation as definitive treatment of an unresectable mesenchymal hamartoma in a child with Beckwith–Wiedemann Syndrome

Abstract Mesenchymal liver hamartomas are benign tumors that can cause life-threatening abdominal distension and carry a risk for malignant transformation. In this case report, we describe a 13-month-old male with Beckwith–Wiedemann Syndrome (BWS) who presented with multiple mesenchymal liver hamartomas causing severe intra-abdominal mass effect. Imaging revealed six large multi-locular cystic lesions, ranging from 3.8 to 8.9 cm in diameter. The large size and spread of the tumors necessitated liver transplantation for complete removal. The patient successfully underwent cadaveric piggyback liver transplantation at 25 months of age. He was alive at 16-month follow-up without evidence of tumor recurrence or graft rejection. Histological examination of the hepatic masses revealed mucinous epithelial lining and abundant hepatocytes in varying stages of differentiation, supporting the diagnosis of mesenchymal hamartoma. To the best of our knowledge, this is the first reported case of liver transplantation in a patient with BWS as definitive treatment for unresectable mesenchymal liver hamartoma.

Trends in pediatric liver transplant donors and deceased donor circumstance of death in the United States, 2002-2015

While much of the discussion regarding expanding the donor pool for pediatric liver transplantation has surrounded the use of technical variant grafts, little attention has been directed toward changes in the deceased donor population. The aim of this study was to investigate trends in the circumstance of the death of deceased donors used for pediatric liver transplantation. All pediatric liver transplant recipients transplanted between 2002 and 2015 were identified in the UNOS database and were categorized based on the donor circumstance of death. There was no significant correlation between year of transplantation and number of pediatric liver transplants performed, pediatric donors, split livers, or living donors. There was a significant downward trend in donors from motor vehicle fatalities and an upward trend in suicide, non‐MVA, and death due to natural causes. There was also an upward trend in drowning, one of the most common mechanisms of death among non‐MVA in 2015. While the number of donors who died in MVA has fallen, the number of deceased donors who died from suicide, natural causes, and non‐MVA, especially drowning, has increased, maintaining the overall number of pediatric deceased donor livers transplanted.

Orthotopic liver transplantation for Sensenbrenner syndrome

Sensenbrenner syndrome, or cranioectodermal dysplasia, is a rare heterogeneic autosomal recessive disorder, affecting ~1 of 1 000 000 live births. The syndrome usually manifests within the first year of life and can present with progressive liver and renal involvement. For all Sensenbrenner patients, renal and liver diseases are the main contributors of morbidity and mortality. In this report, we present the case of a 7‐year‐old boy with congenital liver disease progressing to liver failure secondary to Sensenbrenner syndrome. For this patient, evidence of liver dysfunction was evident from 2 months of age and progressed to frank cirrhosis and severe portal hypertension with multiple episodes of life‐threatening variceal bleeding by age 6. This report illustrates the capability of orthotopic liver transplantation as a viable therapy for those pediatric patients suffering from severe liver failure secondary to a congenital ciliopathy, such as Sensenbrenner syndrome. In fact, early emphasis should be placed on the renal and liver involvement associated with Sensenbrenner syndrome with particular consideration for early referral for transplantation in cases with severe disease. Although the condition is rare, clinicians should be aware of it and its association with fatal liver disease to facilitate appropriate evaluation and referral.

Celiac Axis Extension Grafts in Orthotopic Liver Transplantation

The arterial reconstruction during orthotopic liver transplantation (OLT) is often the most challenging aspect of the procedure that must be achieved. This is due to the wide range of diversity in donor and recipient vasculature including poor arterial perfusion, arterial dissection, complex anatomy, mycotic aneurysms, difficulty in arterial mobilization, adhesions, and others.(1,2) The use of aortic conduits, specifically infrarenal, supraceliac, and others, has been demonstrated to overcome the difficulties in arterial reconstruction and to avoid hepatic artery thrombosis (HAT). Despite an alteration to the normal arterial anatomy, successful longterm outcomes have been achieved for many. However, the procedure requires clamping of the aorta and has been reported to increase the risk of postoperative morbidity and mortality in a number of recipients. HAT is one of the most feared complications because of subsequent high rates of allograft failure, need for retransplant, and patient mortality. Bekker et al.(3) reported in their systematic review an average 1-year graft survival close to 50% in patients diagnosed with HAT within 1 month of transplant. Other significant aortohepatic conduit complications in adults and children include biliary complications (ischemic cholangiopathy), bowel obstruction, recipient paralysis, aneurysm formation, and prolonged ventilation.(1) Although previous literature has shown various graft sources as solutions to vasculature issues during transplant, our current report focuses on cases in which the arterial flow was felt to be inadequate following the initial hepatic arterial anastomosis. Therefore, the celiac axis was maintained as the source of arterial inflow and a donor iliac artery graft was used (Fig. 1). The donor iliac artery was anastomosed to the recipient celiac axis to achieve sufficient vessel length, to remove unfavorable arterial vessel segments, and to improve inflow. The donor iliac artery was then anastomosed to the donor celiac axis to complete the arterial reconstruction (Fig. 2). Aortohepatic conduits have been reported to result in inferior outcomes compared with the standard arterial anastomosis.(1) Del Gaudio et al.(4) concluded that in recipients undergoing arterial reconstruction using the donor iliac artery and inflow using the infrarenal aorta, 26% of graft loss was due to HAT. In contrast, the use of the recipient celiac axis allows a source of adequate inflow without the need for excessive aortic dissection, aortic clamping, and disruption in the normal arterial anatomy. Up to this point, the use of donor iliac arterial grafts being placed on the recipient celiac axis has been underused. We characterize the outcomes of patients at our institution who were felt to have inadequate arterial flow following an initial arterial anastomosis without a history of celiac stenosis and who required arterial extension grafts from the recipient celiac axis to the donor celiac axis with patient and allograft survival comparable to the national published values.

Cold ischemia time is an important risk factor for post–liver transplant prolonged length of stay

Risk analysis of cold ischemia time (CIT) in liver transplantation has largely focused on patient and graft survival. Posttransplant length of stay is a sensitive marker of morbidity and cost. We hypothesize that CIT is a risk factor for posttransplant prolonged length of stay (PLOS) and aim to conduct an hour‐by‐hour analysis of CIT and PLOS. We retrospectively reviewed all adult, first‐time liver transplants between March 2002 and September 2016 in the United Network for Organ Sharing database. The 67,426 recipients were categorized by hourly CIT increments. Multivariate logistic regression of PLOS (defined as >30 days), CIT groups, and an extensive list of confounding variables was performed. Linear regression between length of stay and CIT as continuous variables was also performed. CIT 1‐6 hours was protective against PLOS, whereas CIT >7 hours was associated with increased odds for PLOS. The lowest odds for PLOS were observed with 1‐2 hours (odds ratio [OR], 0.65; 95% confidence interval [CI], 0.45‐0.92) and 2‐3 hours (OR, 0.65; 95% CI, 0.55‐0.78) of CIT. OR for PLOS steadily increased with increasing CIT, reaching the greatest odds for PLOS with 13‐14 hours (OR, 2.05; 95% CI, 1.57‐2.67) and 15‐16 hours (OR, 2.06; 95% CI, 1.27‐3.33) of CIT. Linear regression revealed a positive correlation between length of stay and CIT with a correlation coefficient of +0.35 (P < 0.001). In conclusion, post–liver transplant length of stay is sensitive to CIT, with a substantial increase in the odds of PLOS observed with nearly every additional hour of cold ischemia. We conclude that CIT should be minimized to protect against the morbidity and cost associated with posttransplant PLOS. Liver Transplantation 24 762–768 2018 AASLD.

Mending a Broken Heart: Treatment of Stress-Induced Heart Failure after Solid Organ Transplantation

Stress-induced heart failure, also known as Broken Heart Syndrome or Takotsubo Syndrome, is a phenomenon characterized as rare but well described in the literature, with increasing incidence. While more commonly associated with postmenopausal women with psychiatric disorders, this entity is found in the postoperative patient. The nonischemic cardiogenic shock manifests as biventricular failure with significant decreases in ejection fraction and cardiac function. In a review of over 3000 kidney and liver transplantations over the course of 17 years within two transplant centers, we describe a series of 7 patients with Takotsubo Syndrome after solid organ transplantation. Furthermore, we describe a novel approach of successfully treating the transient, though potentially fatal, cardiogenic shock with a percutaneous ventricular assistance device in two liver transplant patients, while treating one kidney transplant patient medically and the remaining four liver transplant patients with an intra-aortic balloon pump. We describe our experience with Takotsubos Syndrome and compare the three modalities of treatment and cardiac augmentation. Our series is novel in introducing the percutaneous ventricular assist device as a more minimally invasive intervention in treating nonischemic heart failure in the solid organ transplant patient, while serving as a comprehensive overview of treatment modalities for stress-induced heart failure.

Reoperative complications following pediatric liver transplantation

BACKGROUND The aim of this study is to describe the incidence and impact of reoperation following pediatric liver transplantation, as well as the indications and risk factors for these complications. METHODS All primary pediatric liver transplants performed at our institution between January 2012 and September 2016 were reviewed. A reoperative complication was defined as a complication requiring return to the operating room within 30 days or the same hospital admission as the transplant operation, excluding retransplantation. RESULTS Among the 144 pediatric liver transplants performed during the study period, 9% of the recipients required reoperation. The most common indications for reoperation were bleeding and bowel complications. There was no significant difference in the graft survival of patients with a reoperation and those without a reoperation (p = 0.780), but patients with a reoperation had a significantly longer hospital length of stay (median of 39 days vs. 11 days, p = 0.001). Variant donor arterial anatomy, transplant operative time, intraoperative blood loss, transfusion volume of packed red blood cells or cell saver per weight, and transfusion with fresh frozen plasma, platelets, or cryoprecipitate were significantly associated with reoperation upon univariable logistic regression, but none of these risk factors remained statistically significant upon multivariable regression. CONCLUSION At our institution, reoperation did not significantly impact graft survival. We identified variant donor arterial anatomy, transplant operative time, intraoperative blood loss, transfusion volume of packed red blood cells or cell saver per weight, and transfusion with fresh frozen plasma, platelets, or cryoprecipitate as risk factors for reoperation, although none of these risk factors demonstrated independent association with reoperation in a multivariable model. TYPE OF STUDY Prognosis Study. LEVEL OF EVIDENCE Level III.

A long-term experience with expansion of Milan criteria for liver transplant recipients

The Milan criteria (MC) have historically determined eligibility for transplantation for hepatocellular carcinoma (HCC). The United Network for Organ Sharing (UNOS) Region 4 expanded the criteria for transplantation in HCC to include a single tumor ≤6 cm or up to 3 tumors with the largest diameter ≤5 cm and total additive diameter ≤9 cm (R4C). The aim of this study was to report the 10‐year outcomes of this expanded criteria compared to MC. Transplants performed for HCC in Region 4 between October 2007 and December 2016 were reviewed using the UNOS database. Recipients were categorized based on imaging findings at initial evaluation. A total of 2068 patients were included in the study. There was no significant difference in 10‐year patient survival between the groups (53% MC vs 48% R4C, P = .23). There was also no significant difference in recurrence‐free survival (54% MC vs 47% R4C, P = .15) or allograft survival (53% MC vs 48% R4C, P = .16). Finally, there was no significant difference in outcomes between the MC and R4C groups when stratifying patients by locoregional therapy. This study demonstrates promising data that the criteria for liver transplantation in HCC can be safely expanded to the R4C without compromising outcomes.