BACKGROUND:Children's Hospital 2 in Ho Chi Minh City is the only public pediatric center performing pediatric liver transplantation (PLT) in Southern Vietnam. Before the COVID-19 pandemic, our PLT program was implemented and operated in collaboration with Belgian surgical teams who supported both graft procurement and implantation. The program enabled capacity-building and protocol development. METHODS:Travel restrictions necessitated a transition to a hybrid local model. Building on the Pre-COVID groundwork, the team successfully performed 12 living-donor PLTs between 10/2021 and 9/2022. Standardized protocols- developed through earlier international collaboration- were consistently applied. Procurement surgery was carried out by an experienced adult hepatobiliary surgeon from a local hospital, while the implantation was conducted by the pediatric surgical team. RESULTS:Donors had a mean age of 32 ± 6.1 years. Procurement surgery lasted 422 ± 78.1 min. Mean blood loss was 237.5 ± 122.7 mL, and two donors required transfusion. All donor complications were Clavien-Dindo grade I. Recipients had a median age of 28 months and a weight of 10.4 kg. Biliary atresia accounted for 91.7% of indications. Grafts included seven left lateral segments and five full-left lobes. Recipient surgeries lasted 650 ± 91.5 min; mean transfusion volume was 231.2 ± 164.3 mL; cold ischemia time was 83.2 ± 24.9 min. Complications included chylous ascites (n = 2), portal vein stenosis (n = 1), and acute rejection (n = 2). One patient died from a hospital-acquired infection. CONCLUSION:This crisis-driven transition to local autonomization demonstrated that prior supportive preparation and implementation enabled safe and effective PLT during a global health emergency.
BACKGROUND:Left liver resection and split liver surgery demand precise anatomical orientation during parenchymal transection, particularly in the context of organ procurement and pediatric liver transplantation. METHODS:We describe the "Arantius hanging maneuver", which involves preserving and transposing the caudal stump of the Arantius ligament into the parenchymal cut surface. A double hanging maneuver using the caudal Arantius' stump and the round ligament consistently guides dissection toward the left biliary plate, enhancing procedural safety. To illustrate its application, we retrospectively reviewed 134 living donor hepatectomies performed between 2015 and 2019 (119 left lateral sectionectomies, 15 left hepatectomies) with donor outcomes assessed at 3 months using the Clavien-Dindo classification. The maneuver was subsequently implemented in 32 split liver procedures (in-/ex-situ). RESULTS:Donor outcomes were favorable, with no complications in 128 donors (95.5%). Postoperative morbidity included three grade II complications (2.2%), one grade IIIa complication (0.7%) treated by percutaneous drainage, and two grade IIIb complications (1.5%) requiring surgical or endoscopic management. No grade IV or V complications occurred. Recipient 1-year biliary complication rate was 15.6%. In split liver procedures, the maneuver also facilitated identification and division of the left biliary plate. CONCLUSIONS:The « Arantius hanging maneuver » is a simple and reproducible technique for left liver resection and splitting procedures, associated with encouraging results in both donors and recipients. The caudal portion of the Arantius ligament, often overlooked, may represent an anatomical beacon precisely guiding the surgeon during the procedure.
Background: Kasai hepatoportoenterostomy (KPE) remains first-line therapy for biliary atresia(BA), yet the majority of patients will ultimately require liver transplantation. In centres where both KPE and paediatric liver transplantation are performed, KPE has been understood since the early 2000s as the first stage of a two-stage strategy, and the individual technical elements described here are established practice. What is reported is their uniform application as a single written protocol from the first case of the series, together with native liver and transplant outcomes in the resulting cohort. The technical conduct of KPE directly influences the safety and complexity of subsequent transplantation, yet operative decisions at the time of KPE have rarely been evaluated from a transplant-optimisation perspective. We describe a standardised KPE approach incorporating three technical modifications intended to preserve favourable conditions for eventual hepatic replacement and report native liver and transplant outcomes in the resulting cohort. Methods: A retrospective analysis was conducted of 74 consecutive KPE procedures performed by a single surgeon between 2014 and 2025 at Schneider Children's Medical Center, Israel. The operative approach incorporated three deliberate modifications applied uniformly from the first case: a transverse subcostal incision aligned with future transplant access, avoidance of liver mobilisation and exteriorisation, and standardisation of the Roux limb at 50 cm. Primary outcomes were native liver survival and transplant operative parameters. Results: Of the 74 patients, 39 (52.7%) maintained their native liver throughout follow-up, while 35 (47.3%) required liver transplantation. No peri-operative mortality occurred. Median age at KPE was 53 days. In the Cox model, post-KPE portal hypertension (adjusted hazard ratio (aHR) 3.63, 95% confidence interval (CI) 1.40-9.42, p = 0.008) and hepatopulmonary syndrome (adjusted HR 10.23, 95% CI 2.19-47.85, p = 0.003) were associated with eventual transplantation; complications were modelled as fixed (ever/never) covariates because onset dates were not consistently retrievable, and these estimates may be subject to immortal-time bias. Among transplanted patients, the median operative time was 8.0 h (interquartile range (IQR) 6.0-10.2) (n = 34); intraoperative blood loss was documented in 15 of 35 patients (median 300 mL (IQR 205-450)). Conclusions: A standardised, transplant-orientated KPE approach was applied uniformly across 74 consecutive cases; 52.7% maintained their native liver, and transplantation in the remainder proceeded without peri-operative mortality. This series was descriptive by design and included no comparator group that operated without these modifications; it therefore cannot establish whether the modifications influence the complexity of subsequent transplantation, and no such inference should be drawn. Post-KPE portal hypertension and hepatopulmonary syndrome are markers associated with transplant requirement and should prompt intensified surveillance. The approach represents the standardisation of existing practice rather than a new technique.
BACKGROUND:Access to surgical care is a global health burden. A broad spectrum of surgical competences is required in the humanitarian context whereas current occidental surgical training is oriented towards subspecialties. We proposed to design a course addressing the specificities of surgery in the humanitarian setting and austere environment. METHOD:The novelty of the course lies in the implication of academic medical doctors alongside surgeons working for humanitarian non-governmental organizations (NGO). The medical component of the National Defense participated regarding particular topics of war surgery. The course is aimed at trained surgeons and senior residents interested in participating in humanitarian missions. RESULTS:The program includes theoretical teaching on surgical knowledge and skills applied to the austere context. The course also covers non-medical aspects of humanitarian action such as international humanitarian law, logistics, disaster management and psychological support. It comprises a large-scale mass casualty exercise and a practical skills lab on surgical techniques, ultrasonography and resuscitation. Attendance to the four teaching modules, ATLS certification and succeeding final examinations provide an interuniversity certificate. Thirty participants originating from 11 different countries joined the course. Various surgical backgrounds, training levels as well as humanitarian experience were represented. Feedback from the participants was solicited after each teaching module and remarks were applied to the following session. Overall participant evaluations of the first-course session are presented. CONCLUSION:Teaching humanitarian surgery joining academic and field actors seem to allow filling the gap between high-income country surgical practice and the needs of the humanitarian context.
OBJECTIVES:The European Liver Transplant Registry (ELTR) has been collecting data on liver transplantation (LT) in Europe since 1968. The aim of this report is to outline the number, techniques utilized, indications for, and outcomes of pediatric LT (pLT) in Europe, focusing on the Year 2022 in comparison to the preceding 5 years. METHODS:Data were obtained from ELTR and Eurotransplant (ET). Summary statistics were performed. RESULTS:In 2022, 585 pLTs were performed in Europe. The annual number of pLT decreased for the third consecutive year. Living donor LT represented 34% (n = 201) of pLT. The proportion of living donation (LD) remained stable over time. The major indication for pLT in Europe is biliary atresia. Donor age is increasing overall and is associated with worse graft survival. Graft and patient survival were impacted by both types of donors and types of grafts, and were significantly worse after re-transplantation. Most graft failures (77%) and deaths (82%) occurred within the first 6 months after pLT. CONCLUSION:Annual numbers of pLT in Europe are decreasing over time. Given that the proportion of LD has remained stable, the shortage of deceased donor organs may not be the major reason for this trend, and other factors play a role. A focus on improving perioperative care is needed because the risk of graft loss and mortality is highest in the first 6 months after transplantation. New techniques like ex-situ machine perfusion may help mitigate risks with declining quality of deceased donor liver grafts.
Budd-Chiari syndrome (BCS) presents with various degrees of liver damage, and the choice of treatment depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the final treatment option when other interventions are not feasible and when the liver injury is irreversible. We report a case of a pediatric patient with BCS who underwent liver transplantation from a living donor in the context of thrombophilic disorder. CASE PRESENTATION:A 14-month-old girl was admitted to the hospital with ascites. She was malnourished, and an abdominal CT scan confirmed significant ascites with no visualization of the hepatic veins and retrohepatic inferior vena cava (IVC). A liver biopsy revealed fibrosis, necrosis, and parenchymal hemorrhage. Patient's portal hypertension was managed with prophylactic beta-blocker Propranolol and endoscopic esophageal variceal ligation. However, she was hospitalized four times due to gastrointestinal bleeding from ruptured esophageal varices. Protein C deficiency was found as probable etiology of BCS. The patient underwent liver transplantation at 3 years and 8 months old with a liver from a parental living donor. The surgery and postoperative course were uneventful, and the patient was discharged 25 days after the transplant. CLINICAL DISCUSSION:Hypercoagulability is often the underlying cause of BCS. Maintaining anticoagulation/thrombophilic balance postoperatively contributed to the successful liver transplantation in this pediatric patient. CONCLUSION:Liver transplantation is a safe and effective treatment for pediatric patients with BCS who meet the criteria for the procedure.
Background/Objectives: Liver retransplantation (reLT) is the only option for pediatric patients experiencing graft loss. Despite recent advancements in surgical techniques and perioperative management, it remains a high-risk procedure. Our aim is to describe our experience in pediatric reLT, focusing on the technical aspects and surgical challenges. Methods: We systematically analyzed surgical reports from pediatric reLT performed at our center between 2006 and 2023 to identify recurrent intraoperative findings and specific surgical techniques. We focused on challenges encountered during different phases of reLT, including hepatectomy, vascular, and biliary reconstruction. Additionally, we compared patient and graft survival rates among different groups. Results: During the study period, 23 children underwent 25 reLT procedures at our center. Major surgical challenges included complex hepatectomy and vascular reconstructions, necessitating tailored approaches. Our analysis shows that patient and graft survival were significantly lower for reLT compared to primary transplantation (p = 0.002). Early reLT had a significantly lower graft survival compared to late reLT (p = 0.002), although patient survival was comparable (p = 0.278). Patient and graft survival rates were comparable between the first and second reLT (p = 0.300, p = 0.597). Patient survival tended to be higher after living-donor liver transplantation (LDLT) compared to deceased-donor liver transplantation (DDLT), although the difference was not statistically significant (p = 0.511). Conclusions: Pediatric reLT involves significant technical challenges and lower survival rates. Advances in perioperative management are crucial for improving outcomes. Further research is needed to optimize surgical strategies and evaluate the long-term benefits of LDLT in pediatric reLT.
Summary: Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterise paediatric HCAs and determine their course, complications, and management. Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive review of the published literature. A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) and at a mean age of 11.5 (range 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and MODY3 (maturity-onset diabetes of the young type 3). Each of these diseases leads to a well-defined HCA molecular pattern. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in children with portosystemic shunts and in β-catenin-mutated HCAs, while haemorrhages were more frequent in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat adenomatosis, as well as any underlying diseases. Progress in understanding genetic and/or malformative contributions, which appear to be significant in paediatric HCAs, have provided insights into tumour pathogenesis and will further guide patient surveillance and management.
BACKGROUND:The concept of failure to rescue (FTR) has been used to evaluate the quality of care in several surgical specialties but has not been well-studied after living donor liver transplantation (LDLT) in children. METHODS:This study retrospectively reviewed 500 pediatric LDLT performed at a single center between 1993 and 2022. The recipient outcomes were assessed by means of patient and graft survival rates, retransplantation rates, and arterial/portal/biliary complication rates. Graft and patient losses secondary to these complications were calculated regarding FTR for patients (FTRp) and grafts (FTRg). RESULTS:Overall 1- and 5-year patient survival rates were 94.5% and 92.1%, respectively, the corresponding figures for graft survival being 92.7% and 89.8%. One-year hepatic artery complication rate was 3.6% (n = 18 cases), the respective rates for portal vein complications and biliary complications being 5.7% (n = 57) and 15.6% (n = 101). One-year FTRp rates for hepatic artery thrombosis, portal vein thrombosis, anastomotic biliary stricture, and intrahepatic biliary stricture were 28.6%, 9.4%, 3.6%, and 0%, respectively. The corresponding FTRg rates being 21.4%, 6.3%, 0%, and 36.4%. CONCLUSION:Such novel analytical method may offer valuable insights for optimizing quality of care in pediatric LDLT.
Introduction Initial allograft function determines the patient’s immediate prognosis in pediatric liver transplantation. Ischemia-reperfusion injuries play a role in initial poor graft function (IPGF). In animal studies, preconditioning with inhaled anesthetic agents has demonstrated a protective effect on the liver. In humans, the few available studies are conflicting. This study assesses the association between the hypnotic agent used to maintain anesthesia during hepatectomy in living donors and the occurrence of IPGF after pediatric transplantation. Methods We conducted a single-center retrospective analysis of children who received a living donor liver transplant (LDLT) between 2010 and 2019. We analyzed the incidence of EAD according to the hypnotic agent used to maintain general anesthesia during donor hepatectomy. Results We included 183 pairs of patients (living donors-recipients). The anesthetics used in the donor were propofol ( n = 85), sevoflurane ( n = 69), or propofol with sevoflurane started 30 min before clamping ( n = 29). Forty-two children (23%) developed IPGF. After multivariate logistic regression analysis, factors significantly associated with the occurrence of IPGF were the anesthesia maintenance agent used in the donor ( p = 0.004), age of the donor ( p = 0.03), duration of transplant surgery ( p = 0.009), preoperative receiver neutrophil to lymphocyte ratio ( p = 0.02), and albumin ( p = 0.05). Conclusion Significantly fewer children who received a graft from a donor in whom only sevoflurane was used to maintain anesthesia developed IPGF. Although additional research is needed, this preconditioning strategy may provide an option to prevent IPGF after living liver donation.
Hepatocellular adenomas (HCAs) are rare benign liver tumors. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterize pediatric HCAs and determine their course, complications, and management.Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive review of the published literature.A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) with a mean age of 11.5 (range: 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and maturity-onset diabetes of the young type 3 (MODY3). Each of these diseases leads to a well-defined HCA molecular pattern. For example, HCAs developed in conjunction with glycogenosis were largely inflammatory, while those in conjunction with MODY3 were HNF1A-mutated. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in portosystemic shunts and β-catenin-mutated-HCAs, while hemorrhages occurred significantly more frequently in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat not only the adenomatosis, but also any underlying diseases.HCAs occur predominantly in children with predisposing diseases, which, in turn largely determines the HCA molecular type and risk of complications. Progress in understanding the genetic and/or malformative contributions, which appears to be significant in pediatric HCAs, provides insights into tumor pathogenesis and will further guide patient surveillance and management.
Introduction: Deoxyguanosine Kinase (DGUOK) deficiency is a very rare disorder characterized by liver dysfunction, neurological manifestations, and metabolic disorders secondary to severely reduced mitochondrial DNA content. These patients develop early-onset liver failure, and their liver transplantation (LT) indication remains debatable due to the possibility of neurological involvement. Case Report: We present the case of a 6-month-old female diagnosed with DGUOK deficiency who developed liver failure. At 9 months, she underwent a living-related LT with an initial favorable evolution under immunosuppression therapy with tacrolimus. Four months after LT, she presented two prolonged bacterial and Rotavirus enteritis episodes. She developed classical post-transplant complications (severe renal tubular acidosis type IV, secondary to the high tacrolimus level, and post-transplant lymphoproliferative disease) during these episodes. Her condition deteriorated progressively, with reversible hypotonia and significant weight loss. However, the neurological evaluation did not reveal any signs suggestive of the progression of the underlying disease. A few months later, her clinical features and laboratory parameters improved considerably. Conclusions: This case highlights the unpredictable evolution of children with LT for liver failure due to DGUOK deficiency.
BACKGROUND:The Resection And Partial Liver Transplantation with Delayed total hepatectomy (RAPID) procedure involves left hepatectomy with orthotopic implantation of a left lobe and right portal vein ligation. This technique induces volumetric graft increase, allowing for a right completion hepatectomy within 15 days. Notably, there is a lack of data on the hemodynamics of small-for-size grafts exposed to portal overflow without triggering small-for-size syndrome. METHODS:A prospective single-center protocol included 8 living donors and 8 RAPID noncirrhotic recipients. Comprehensive clinical and biological data were collected, accompanied by intraoperative arterial and portal flow and pressure measurements. Early kinetic growth rate (eKGR%) and graft function were assessed using computed tomography and 99Tc-mebrofenin scintigraphy on postoperative days 7 and 14. Findings were compared with retrospective data from 13 left living donor liver transplantation (LDLT) recipients. RESULTS:The median Graft-body weight ratio was 0.41% (interquartile range: 0.34-0.49), markedly lower than in LDLT. However, there was no significant difference in eKGR between RAPID and LDLT grafts. Sequential analysis revealed variable eKGR per day: 10.6% (7.8-13.2) in the first week and 7.6% (6-9.1) in the second week posttransplantation. Indexed portal flow (indexed portal vein flow) was significantly higher in RAPID compared with left LDLT ( P = 0.01). No hemodynamic parameters were found to correlate with regeneration speed. We modulated portal flow in 2 out of 8 cases. CONCLUSIONS:This study presents the first report of hemodynamic and volumetric data for the RAPID technique. Despite initial graft volumes falling below conventional LDLT recommendations, the study highlights acceptable clinical outcomes.
We read with great interest and enthusiasm the article by Rela et al1 from Chennai, India, entitled "Experience in establishing a robotic donor hepatectomy program for pediatric liver transplantation," published in Transplantation, which received a constructive guest comment, but also a very pragmatic one by the transplant team from the University of California, San Francisco.2 We commend the high level of organization, the structure and all the measures such as simulator training, the 3-h limit of robotic procedure in the first cases, and also the establishment of a contingency plan implemented during the introduction of the robotic approach in their pediatric living donor liver transplantation (PLDLT) scenario. It is worth highlighting the competence of the team with >10 y of experience together, which has undoubtedly contributed to their success.1 We would like to participate in the debate by contributing some insights from our PLDLT program at the Cliniques Universitaires Saint-Luc in Brussels with currently >500 living donation (LD) procedures. Despite stating that there were no adverse events in recipients related to the robotic approach, the Indian authors reported 1 case in which the use of the triple-clamp robotic line resulted in a shortening of the length of the left hepatic vein, which required a venous extension. This prompted them to start using the laparoscopic stapler and dividing the left phrenic vein to obtain a longer left hepatic vein in subsequent cases. This parallels the 9 intraoperative adverse events (iAEs), described in our series of 438 open left hepatic resections for PLDLT. iAEs were defined as any deviation from the planned operative course, which could have put the donor and/or recipient at risk if not recognized and treated in time.3 All the iAEs led us to further modify our perioperative surgical protocol, in the same way that they have described it.1 We believe that iAEs are underreported in the context of LD and their reporting could be used as a safety tool to improve donor surgical protocols and for series comparison purposes. Regarding the complications, the Indian team reported that 16 patients (21.33%) developed a minor Clavien complication (≤IIIb), comparable to previously published series of LD left liver resection with laparotomy approach.4 It is worth highlighting the incidence of 4 biliary complications (5%), all with favorable evolution. However, its appearance should be a reminder that robotic surgeons should pay attention to an adequate evaluation of intrahepatic biliary anatomy. In line with the University of California, San Francisco, team's comment about the putative universal use of the robotic approach in LD, which could be affected by the stringency of the credentialing pathway in the United States, we are also concerned about the additional workload and manpower required to implement a safe robotic LD program.2 The Chennai team structure (senior surgeons, residents, scrub nurses, and circulators) is rather difficult, if not impossible to replicate here in Europe, where, in addition, to the stricter labor regulation, there is an immense nursing shortage and a high turnover rate, which may considerably impacts the team cohesion and interaction. As well, the number of residents is reduced and their weekly working time is regulated by law. Furthermore, institutional support is not comparable to that in Chennai and other centers in the Middle East and Asia.5,6 Furthermore, LDLT activity is limited in Europe, with few centers having a significant number of cases per year, which could prolong the time to achieve mastery of robotic LD procedures. Although robotic is a very innovative and a highly groundbreaking approach to LD hepatectomy, the reality in Europe is all these challenges must be first addressed to pave the way for a safe robotic LD program.
Pediatric solid organ transplantation (SOT) is a preferred treatment for medically suitable children with end-stage organ failure. Still, many of them have no access to transplantation owing to socioeconomic constraints or lack of transplant facilities in low- and middle-income countries (LMIC). Establishing pediatric SOT programs in LMIC offers children the opportunities to receive transplant care in more familiar home environments as well as help curtail transplant tourism and improve transplant outcomes as pediatric transplantation would be performed ethically and legally. The International Pediatric Transplant Association (IPTA) is a professional organization aiming to promote safe, ethical, and high-quality pediatric transplantation worldwide. This society paper describes major obstacles to pediatric SOT in LMIC and provides guidance on developing and/or expanding pediatric SOT programs in such countries. We also summarize available resources from the IPTA Outreach Program to help establish and support pediatric SOT programs in LMIC.
One of the challenges when caring for children admitted for acute liver failure (ALF) is to quickly identify those who will improve spontaneously and those for whom liver transplantation (LT) is the only therapeutic option. Retrospective study to review our experience, identify mortality risk factors and update our LT criteria in case of paediatric ALF. 111 children were admitted between March 1989 and May 2021 (mean age 59.1 months). 28 children never met our LT criteria; 17 had contraindication to LT; 66 were registered on the LT waitlist. 14 of these 66 were subsequently withdrawn because of spontaneous liver function recovery; 11 died before having received a liver; 41 were transplanted. Hospital survival rate was 63
IntroductionEsophageal replacement surgery in children is sometimes necessary for long-gap esophageal atresia. Ileocolic esophagoplasty in the retrosternal space can serve as a good alternative technique in case of hostile posterior mediastinum. We present two cases of successful ileocolic transposition performed at 6 months of age.MethodsEsophageal replacement was performed through a midline laparotomy incision associated with a left cervical approach. The ileocolic transplant was pediculized on the right superior colic artery after ligating the right colic and ileocolic vessels. A retrosternal tunnel was created, and the ileocolic transplant pulled through it to reach the cervical region. Proximally, esophageal-ileal anastomosis and, distally, colonic–gastric anastomosis were performed. Ileocolic continuity was repaired.ResultsThere were no early postoperative complications. In both cases, the patients presented oral feeding difficulties during the first 6 postoperative months. Thereafter, full oral feeding was achieved, and both patients were clinically asymptomatic during the following 18 and 20 years, respectively, with satisfactory oral radiological assessments, showing no redundancy or inappropriate growth of the graft and no anastomotic stricture. Currently, these patients do not complain of dysphagia, pathological reflux, or respiratory symptoms.ConclusionWhen native esophagus preservation in long-gap esophageal atresia is estimated unfeasible, ileocolic transposition in the retrosternal space might be considered a good and safe option, particularly in those difficult cases after multiple previous surgical attempts and mediastinitis. This technique is putatively associated with a beneficial anti-reflux effect, thanks to the presence of the ileocecal valve, in preventing cervical peptic esophagitis. Long-term follow-up confirms that the transposed colon in the retrosternal space did not suffer any abnormal modification in size and growth.
Background Deoxyguanosine kinase deficiency is mainly manifested by hepatic and neurological damage, hence it belongs to the hepatocerebral form of mitochondrial deoxyribonucleic acid depletion syndrome. The association between deoxyguanosine kinase deficiency and recurrent spontaneous pneumothorax has not currently been reported. Case presentation A 12-year-old Russian boy with deoxyguanosine kinase deficiency, a recipient of a liver transplant with amyotrophy secondary to his mitochondriopathy, presented with recurrent spontaneous bilateral pneumothorax refractory to drainage and surgery. Conclusion To our knowledge, this is the first documented case of deoxyguanosine kinase deficiency associated with recurrent spontaneous pneumothorax, which could be considered a late complication of deoxyguanosine kinase deficiency. At this point, this is only an association and further studies and research need to be performed to help confirm the pathogenesis of this association.
Background. Donor safety is paramount in living organ donation. Left liver resections are considered safer than right lobe hepatectomies. However, unexpected intraoperative adverse events (iAEs), defined as any deviation from the ideal intraoperative course, can also occur during left liver resections and may be life threatening or lead to postoperative complication or permanent harm to the donor and recipient. Methods. Records of 438 liver living donors (LDs) who underwent 393 left lateral sectionectomies (LLSs) and 45 left hepatectomies (LHs) between July 1993 and December 2018 in a pediatric living donor liver transplantation center were reviewed for the appearance of iAEs that could have influenced the donor morbidity and mortality and that could have contributed to the improvement of the LD surgical protocol. Results. Clinical characteristics of LLS and LH groups were comparable. Nine iAEs were identified, an incidence of 2%, all of them occurring in the LLS group. Seven of them were related to a surgical maneuver (5 associated with vascular management and 2 with the biliary tree approach). One iAE was associated with an incomplete donor workup and the last with drug administration. Each iAE resulted in subsequent changes in the surgical protocol. Donor outcome was at risk by 5 iAEs classed as type a, recipient outcome by 2 iAEs (type b) and both by 2 iAEs (type c). Postoperative complications occurred in 87 LDs (19.9%), with no differences between the LLS and LH groups (P = 0.227). No Clavien-Dindo class IVa or b complications or donor mortality (Clavien-Dindo class V) were observed. Conclusions. iAEs debriefings induced changes in our LD protocol and may have contributed to reduced morbidity and zero mortality. iAEs analysis can be used as a quality and safety improvement tool in the context of LD procedures, which may include right liver donation, laparoscopic, and robotic living liver graft procurement.