Revisiting the forgotten "full-right full-left" liver division: Simplified technique and new strategical considerations for clinical implementation in Italy.

BACKGROUND: Full-right/full-left liver splitting was introduced early in the 90s as part of the great wave of technical innovations that characterized that decade. One approach was to divide the liver on the right of the Cantlie's line and leave the middle hepatic vein with the left graft, with both grafts allocated to adults. Both grafts had some functional disadvantages and exposed the adult recipients to some early hepatic dysfunction, and the results were not great. An alternative approach consisted of an ex situ division of the liver, exactly along Cantlie's line, thus sharing the middle hepatic vein between the two grafts. None of these two techniques were really adopted, and there has been nearly no transplantation of this type in the last decade worldwide. METHOD AND RESULTS: The authors propose a variation of the latter technique that was used recently with success: The division of the liver is made simpler; the two grafts are prepared ex situ and need a simple vascular reconstruction (one venous patch on each graft); and the grafts can be implanted using very standard techniques. CONCLUSION: Because candidates for liver transplantation weighing 25-60 kg (old children, teenagers, and some small adults) are often at some disadvantage in getting size-matched livers (this range of weight is less represented in the donor population), implementing the latter technique would help provide adequate grafts for them. In Italy, where many livers offered for splitting are not used, there would be ample room for implementing this option within the actual donor pool and allocation system.

Combining living and deceased donation for pediatric first isolated liver transplantation: A win-win even in countries with high deceased donor donation rates.

BACKGROUND: Split and living donor liver transplantations are both key surgical strategies for development of pediatric liver transplant programs. Often, however, teams tend to prioritize only one preferentially. METHODS: In the context of a very active national split liver graft allocation program (Italy), retrospective study of 226 consecutive pediatric first isolated liver transplants performed by a single team using organs from both deceased and living donors. Clinical characterisitics and outcome were compared. RESULTS: In the context of a steadily slowly decreasing split graft offer, living donation activity steadily increased. Deceased and living donation accounted for 52.6% and 47.4% of transplantations, respectively. Both strategies were equally used for transplanting patients up to 30 kg of weight, while deceased donors were predominantly used for older recipients. Technical variants represented 86% of all transplants, with 183 conisting of left lateral segment grafts (76 split liver grafts and 107 left grafts from living donors). Outcome of both surgical strategies was similar, with excellent outcomes at early, mid-, and long-term. CONCLUSIONS: Splitting livers of deceased donors and using living donation were complementary and non-competitive strategies for developping pediatric liver transplant activity. Implementing both activities in parallell allowed to maintain stable the number of annual transplant in Italy and allowed to reach superior outcomes. This analysis provides evidence that living donation plays a role in Italy despite an existing very active "mandatory-split" national policy.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (2) intraoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.

European Liver Transplant Registry: Donor and transplant surgery aspects of 16,641 liver transplantations in children.

BACKGROUND AND AIMS: The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968. APPROACH AND RESULTS: Over a 50-year period (1968-2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to three successive periods (A, before 2000; B, 2000-2009; and C, since 2010), studying donor and graft characteristics and graft outcome. The use of living donors steadily increased from A to C (A, n = 296 [7%]; B, n = 1131 [23%]; and C, n = 1985 [39%]; p = 0.0001). Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (p < 0.0001) and to 79% in group C (B versus C, p < 0.0001). Graft half-life was 31 years, overall; it was 41 years for children who survived the first year after transplant. The late annual graft loss rate in teenagers is higher than that in children aged <12 years and similar to that of young adults. No evidence for accelerated graft loss after age 18 years was found. CONCLUSIONS: Pediatric liver transplantation has reached a high efficacy as a cure or treatment for severe liver disease in infants and children. Grafts that survived the first year had a half-life similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to revisit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age.

Addressing extreme size mismatch in pediatric intestinal transplantation: Outcomes of intestinal length reduction.

BACKGROUND: Bench liver reduction, with or without intestinal length reduction (LR) (coupled with delayed closure and abdominal wall prostheses), has been a strategy adopted by our program for small children due to the limited availability of size-matched donors. This report describes the short, medium, and long-term outcomes of this graft reduction strategy. METHODS: A single-center, retrospective analysis of children that underwent intestinal transplantation (April 1993 to December 2020) was performed. Patients were grouped according to whether they received an intestinal graft of full length (FL) or following LR. RESULTS: Overall, 105 intestinal transplants were performed. The LR group (n = 10) was younger (14.5 months vs. 40.0 months, p = .012) and smaller (8.7 kg vs. 13.0 kg, p = .032) compared to the FL group (n = 95). Similar abdominal closure rates were achieved after LR, without any increase in abdominal compartment syndrome (1/10 vs. 7/95, p = .806). The 90-day graft and patient survival were similar (9/10, 90% vs. 83/95, 86%; p = .810). Medium and long-term graft survival at 1 year (8/10, 80% vs. 65/90, 71%; p = .599), and 5 years (5/10, 50% vs. 42/84, 50%; p = 1.00) was similar. CONCLUSION: LR of intestinal grafts appears to be a safe strategy for infants and small children requiring intestinal transplantation. This technique should be considered in the situation of significant size mismatch of intestine containing grafts.

Inferior vena cava surgical cannulation for infants needing veno-venous extracorporeal membrane oxygenation.

INTRODUCTION: Femoral cannulation for veno-venous extracorporeal membrane oxygenation is challenging in infants because of the diameter of the vein. CASE REPORT: Prolonged ECMO support (67 days) was necessary for an 8-month-old (8 kg) girl with acute respiratory distress syndrome that was caused by H1N1 influenza. After 30 days on ECMO support and using a single 16 Fr double-lumen cannula (internal jugular vein), a second cannula was necessary to ensure adequate flow. This second 12 Fr single-lumen cannula was surgically placed through the right common iliac vein. An excellent flow profile was then achieved and ECMO continued successfully for 37 more days. DISCUSSION: As a lifesaving option, this double caval configuration successfully optimized the flow profile and oxygenation, outweighing the related risks. CONCLUSION: In small children, a surgical approach to the inferior vena cava can be considered safe, especially in those cases where there is a shortage of adequate cannulas, or when central venous access is difficult.

Unusual Clinical Course for Untreated Malformative Biliary Atresia Infant: Is Portal Hypertension an Important Driver of Liver Fibrosis?

ABSTRACT: In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years. Remarkably, she remained in satisfactory condition in absence of ascites or severe hepatic dysfunction, when 4 other similar patients-managed during the same period of time-all had the usual clinical deterioration and ascites, with the need for liver replacement. To our knowledge, there is no similar report in literature. Possible pathogenetic mechanisms and the role of portal hypertension as important factors are discussed.

Cavoportal Hemitransposition in Liver Transplantation: Toward a More Safe and Efficient Technique.

Extended splanchnic venous thrombosis represents a challenge for the liver transplantation (LT) surgeon. In the absence of large venous tributaries, the cavoportal hemitransposition (CPHTr) and the combined liver-intestinal or multivisceral transplantation are the only technical solutions. Because of the reported high morbidity and mortality rates due to infrequent use and a lack of standardization, the former technique has been almost abandoned by the transplant community. A newly designed technique of CPHTr is presented that is based on the combination of an inferior vena cava (IVC)-sparing hepatectomy and large laterolateral cavocaval and end-to-side cavoportal anastomoses separated only by a double vascular stapler line. This technique allows the splanchnic blood to be completely diverted toward the allograft and to eliminate low-flow IVC areas, which possibly lead to complications. The modified CPHTr technique proposed here offers a valuable alternative to much more complex and invasive intestinal transplantation procedures.

A national mandatory-split liver policy: A report from the Italian experience.

To implement split liver transplantation (SLT) a mandatory-split policy has been adopted in Italy since August 2015: donors aged 18-50 years at standard risk are offered for SLT, resulting in a left-lateral segment (LLS) graft for children and an extended-right graft (ERG) for adults. We aim to analyze the impact of the new mandatory-split policy on liver transplantation (LT)-waiting list and SLT outcomes, compared to old allocation policy. Between August 2015 and December 2016 out of 413 potentially "splittable" donors, 252 (61%) were proposed for SLT, of whom 53 (21%) donors were accepted for SLT whereas 101 (40.1%) were excluded because of donor characteristics and 98 (38.9%) for absence of suitable pediatric recipients. The SLT rate augmented from 6% to 8.4%. Children undergoing SLT increased from 49.3% to 65.8% (P = .009) and the pediatric LT-waiting list time dropped (229 [10-2121] vs 80 [12-2503] days [P = .045]). The pediatric (4.5% vs 2.5% [P = .398]) and adult (9.7% to 5.2% [P < .001]) LT-waiting list mortality reduced; SLT outcomes remained stable. Retransplantation (HR = 2.641, P = .035) and recipient weight >20 kg (HR = 5.113, P = .048) in LLS, and ischemic time >8 hours (HR = 2.475, P = .048) in ERG were identified as predictors of graft failure. A national mandatory-split policy maximizes the SLT donor resources, whose selection criteria can be safely expanded, providing favorable impact on the pediatric LT-waiting list and priority for adult sick LT candidates.

Unusual venous collateral pathways allow for reperfusion of the intrahepatic portal venous system in children with portal vein thrombosis after split liver transplantation: Clinical relevance and management implications.

PVT is the most frequent vascular complication after LT in small children, and a higher incidence has been observed in those transplanted for biliary atresia or with a LLSG. Thrombosis of the PV causes extrahepatic portal hypertension and is associated with splenomegaly and the development of venous neo-collaterals, including gastro-oesophageal varices and splenorenal shunts. It has also been incidentally suggested in the literature that patients who have had a Roux-en-Y loop for a biliary reconstruction may present with a cavernomatous transformation of the distal portion of the loop. In this study, 13 children with CEPH caused by thrombosis of the PV after LT were analysed. The study evidenced the development of two types of hepatopetal venous networks: (a) a large cavernoma along the Roux loop and around the biliary anastomosis, and (b) a network of neo-collaterals in the gastro-duodeno-pancreatic area that connected to the intrahepatic portal branches directly through the liver capsule. These hepatopetal venous networks between the venous system of the surrounding organs or the omentum and the intrahepatic portal branches can be identified by radiologists. The relevance for the transplanting physician and the transplant surgeon is discussed.

Case report: horse or zebra, ascites or pseudo-ascites? Care for pictural details!

BACKGROUND: Pseudo-ascites is a very rare condition in children and remains a challenging diagnosis. Targeted imaging may be helpful, but a high index of clinical suspicion is often necessary to guide the investigations, as pseudo-ascites may efficiently mimic true ascites. To date, still many cases of pseudo-ascites suffer diagnostic and therapeutic delay, and some are only diagnosed during surgical exploration. We report the case of a patient with a late laparoscopic diagnosis of pseudo-ascites. We retrospectively review our patient's imaging findings and suggest new characteristic features which may help differentiate pseudo-ascites from true ascites. CASE PRESENTATION: A 7-month-old infant was referred for a progressive abdominal distention. Physical examination and initial ultra-sonographic findings evoked free ascites. An extensive diagnostic workup was then performed and was negative for hepatic, renal, cardiac, intestinal, pancreatic, inflammatory or infectious diseases, malignancy and congenital metabolic disorders. Pseudo-ascites was evoked and dedicated ultra-sonographic and magnetic resonance studies were repeated but could not confirm this diagnosis. Symptomatic diuretic treatment with spironolactone and furosemide was then started. A temporary and limited effect was noted but, with time, repeated paracenteses were necessary as the abdominal distention progressed causing discomfort and breathing difficulty. Last, because the patient's quality of life deteriorated, a peritoneal-venous shunting was proposed; as the operation started with a diagnostic laparoscopy, a benign giant cystic mesenteric lymphangioma was identified and totally excised. The resolution of symptoms was immediate and the patient remained symptom-free throughout the subsequent observation period that lasted more than 1 year. CONCLUSIONS: Increased awareness about pseudo-ascites is necessary, as the diagnosis is often overlooked, and treatment delayed. Targeted imaging may be helpful, as some specific, although not pathognomonic, features exist which may aid in the diagnosis.