Pediatric ex-vivo liver resection and auto-transplantation for complex hepatic alveolar echinococcosis

Hepatic echinococcosis (HE) is primarily classified into cystic echinococcosis (CE) and alveolar echinococcosis (AE), both zoonotic diseases caused by larval infestation of Echinococcus tapeworms. The disease is strongly associated with a history of residing in pastoral areas or exposure to intermediate host animals, posing a significant threat to the health and development of children in endemic regions (1).

The treatment of hepatic alveolar echinococcosis (HAE) relies on radical surgical resection combined with albendazole therapy, with radical surgical resection being the cornerstone of long-term survival (2,3). However, in complex cases where conventional resection is unfeasible due to large tumor size, critical location, or major vascular involvement, PELRA may be considered to achieve curative treatment (4,5). Given the low incidence of pediatric HAE and the technical complexity of PELRA, globally reported cases remain extremely limited. This study presents a rare pediatric case of unresectable HAE successfully managed with PELRA, achieving complete disease eradication.

A 7-year-old asymptomatic female from pastoral regions with 2-year history of hepatic mass was diagnosed with progressive HAE based on computed tomography (CT) findings and positive immunoglobulin G (IgG) serology. Preoperative imaging revealed extensive right hepatic and caudate lobe involvement with vascular invasion [hepatic veins, portal branches, and retro-hepatic inferior vena cava (IVC)], prompting multidisciplinary evaluation for PELRA (Figure 1). The patient’s favorable parameters [standard liver volume 634 mL, projected remnant 460 mL (72.5%), graft-to-recipient weight ratio (GRWR) 1.67%, indocyanine green retention test at 15 min (ICG-R15) 1.5%] confirmed surgical candidacy. Preoperative venography was planned to assess IVC patency, with possible of reconstruction if occluded. Although segment IV preservation was technically feasible, our team elected for complete resection due to high complication risks.

Figure 1 CTA Findings: right hepatic lobe and caudate demonstrate alveolar echinococcosis, with invasion of RHA, right/left portal branches, main portal trunk, RHV/MHV/LHV, and retro-hepatic IVC. CTA, computed tomography angiography; IVC, inferior vena cava; LHV, left hepatic vein; MHV, middle hepatic vein; RHA, right hepatic artery; RHV, right hepatic vein.

The procedure was performed through a reverse L-shaped incision. Exploration revealed lesions involving segments 1, 4a, 5, 6, 7, and 8. After mobilizing the hepatic ligaments, the lesions was found to be densely adherent to the retro-hepatic IVC and the second hepatic hilum, precluding in situ resection. Given the preserved IVC flow confirmed intraoperatively, PELRA was initiated. The first hepatic hilum was dissected without initial vascular ligation. Following complete mobilization of the IVC from the retroperitoneum, supra-hepatic IVC and infra-hepatic IVC clamping enabled total hepatectomy. Given minimal IVC tension, direct end-to-end anastomosis was performed, supplemented by temporary portocaval shunt. The explanted liver was perfused with cold (0 ℃) histidine-tryptophan-ketoglutarate (HTK) solution for preservation. Meticulous resection along the lesion margins demonstrated extensive invasion of the right bile duct, partial IVC wall, right and middle hepatic veins, right hepatic artery, and portal vein branches. After radical excision of the right lobe and segment 4a, segment 4b (draining via the fissure vein into the left hepatic vein) was preserved. The residual liver (467 g) was reimplanted after shunt removal, with sequential anastomoses: left hepatic vein-to-IVC (end-to-side), left portal vein-to-main portal vein (end-to-end), hepatic artery (end-to-end), and left hepatic duct-to-common bile duct (end-to-end). Post-reperfusion Doppler ultrasound confirmed patent vasculature. Hemostasis was secured, drains placed, and the abdomen closed (Figure 2).

Figure 2 Surgical procedures of ex vivo resection: (A) hepatic artery stump, (B) portal vein stump, (C) supra-hepatic IVC segment, (D-F) lesion dissection along IVC, (G) parenchymal transection with portal vein reconstruction, (H) left hepatic vein root reconstruction, (I) leak test, (J) completed hepatic vein anastomosis, (K,L) final portal vein and hepatic artery reconstructions. IVC, inferior vena cava.

Pathological showing the liver architecture was disrupted with extensive coagulative necrosis and cystic cavity formation. The cavities contained irregularly degenerated laminated layers and hooklets of the parasite with focal calcification. The necrotic areas were surrounded by prominent inflammatory infiltrates with epithelioid histiocytes and multinucleated giant cell reactions. These features are diagnostic of HE. Notably, the liver capsule was uninvolved, and the surgical margins were free of disease (Figure 3).

Figure 3 The explanted liver specimen demonstrates intact lesion morphology with histologically confirmed negative resection margins.

The patient demonstrated excellent recovery, and postoperative CT on postoperative day (POD) 5 confirmed radical resection (Figure 4). She was discharged on postoperative day 14 and has maintained disease-free survival exceeding 12 months.

Figure 4 POD 5 CT imaging confirmed radical resection. CT, computed tomography; POD, postoperative day.

HAE exhibits malignancy-like infiltrative growth, frequently causing extensive invasion of hepato-vascular structures (2,6). While radical resection combined with albendazole remains the therapeutic cornerstone, part of pediatric cases present unresectable disease due to massive lesion size, critical location or major vascular involvement (7). For pediatric patients with HE involving critical anatomical structures—including the hepatic veins, IVC, or secondary/tertiary hepatic hila—particularly those with lesions located in the following high-risk triangular zones: the triangle formed by the right hepatic vein, middle hepatic vein, and infra-hepatic IVC, the portal left branch-middle hepatic vein-retro-hepatic IVC triangle, the portal right branch-middle hepatic vein-retro-hepatic IVC triangle, ex vivo liver resection and auto-transplantation (ELRA) offers a pathway to R0 resection (8).

Since Pichlmayr’s pioneering ELRA in 1988. Its subsequent development by P Dong’s team in China, and presenting the first systematic delineation of end-stage HAE as a cardinal indication for autologous liver transplantation (9,10). PELRA remains exceptionally rare, due to restricted indications, technical complexity, elevated operative difficulty, and significant surgical risks. Fusai’s 2006 case, 15-year-old with paraganglioma, marked its first pediatric application (11). To date, the literature on ELRA in pediatric patients remains sparse and is largely confined to isolated case reports and small case series from highly specialized centers (11-13). The limited available reports mainly involve children with hepatic malignancies or complex benign liver diseases that are not amenable to conventional surgical resection.

PELRA entails total hepatectomy, ex vivo hypothermic perfusion, precise lesion excision with meticulous vascular reconstruction, and reimplantation. Current indications include: end-stage HAE, benign/low-grade malignant tumors, and malignancies responsive to neoadjuvant therapy (14). Preoperative evaluation requires a comprehensive assessment of disease characteristics and risk-benefit analysis, establishing a multidimensional evaluation system. The West China Classification—an innovative four-type anatomical categorization based on the extent of IVC, portal vein, and hepatic vein infiltration—provides critical guidance for ELRA surgical planning (15). Drawing from our team experience with adult ELRA cases, we recommend focusing on three pivotal determinants: (I) anatomical characteristics of lesions: assessing the feasibility of radical in situ resection; (II) hemodynamic risks: predictive evaluation of potential massive hemorrhage and circulatory stability; (III) functional hepatic reserve: ensuring sufficient remnant liver volume (GRWR ≥0.8) with qualified parenchymal quality (16,17). Compared with allograft liver transplantation, the potential advantages of ELRA include elective scheduling without donor dependency, avoidance of lifelong immunosuppression, and a potentially more favorable oncologic outcome (16). However, available evidence indicates that the perioperative mortality associated with ELRA is approximately 10% (18), which remains substantially higher than that reported for pediatric liver transplantation performed at experienced centers, where perioperative mortality is generally below 2% (19). In regions where donor organs are relatively accessible, this difference in perioperative risk may reasonably influence both families and clinicians to favor allograft transplantation. It should be emphasized, however, that in clinical practice ELRA is typically not selected on the basis of a direct risk comparison with allograft transplantation, but rather in specific scenarios in which conventional liver resection is technically infeasible and allograft transplantation is unavailable, contraindicated, or oncologically inappropriate. Accordingly, the choice between these two strategies should be regarded as highly dependent on the individual clinical context rather than competitive. Given its substantial technical complexity and potential risks, the application of PELRA should be strictly limited to highly selected patients following comprehensive multidisciplinary team (MDT) evaluation (14), particularly those with localized disease and adequate hepatic functional reserve. In such exceptional clinical circumstances, the use of PELRA as a last-resort surgical strategy represents a rational decision based on a careful balance between anticipated risks and potential benefits. Therefore, we strongly recommend implementing this technique exclusively at certified transplant centers with established expertise in liver transplantation.

Successful PELRA hinges on three pillars. Precision Assessment-Location-structure-quantity-surgical design enhanced by three-dimensional (3D) reconstruction for virtual hepatectomy, advanced techniques-vascular reconstruction, temporary portocaval shunt, hypothermic machine perfusion, and computer-assisted planning, risk mitigation-perioperative protocols adapted from pediatric transplant expertise (15,20-22). Although PELRA currently benefits from a solid theoretical foundation, relatively mature technical systems, its widespread adoption remains challenging due to narrow indications, low disease prevalence, limited patient numbers, and stringent requirements for preoperative evaluation, surgical expertise, and postoperative care. Consequently, enhanced collaboration among leading liver transplant centers worldwide is imperative to facilitate experience sharing and promote the advancement and refinement of this technique.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardian for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Acknowledgments

None.

Footnote

Provenance and Peer Review: This article was a standard submission to the journal. The article has undergone external peer review.

Peer Review File: Available at https://hbsn.amegroups.com/article/view/10.21037/hbsn-2025-aw-832/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://hbsn.amegroups.com/article/view/10.21037/hbsn-2025-aw-832/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardian for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

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