Palmisani Francesca, Stundner-Ladenhauf Hannah N, Pichler Judith, Aldrian Denise, Heilos Andreas, Steinbauer Philipp, Metzelder Martin L, Patsch Janina M, Huber Wolf-Dieter, Oberhuber Rupert, Krois Wilfried
Department of Pediatric Surgery, Medical University of Vienna, Vienna, Austria.
Department of Visceral, Transplant and Thoracic Surgery, Medical University of Innsbruck, Innsbruck, Austria.
Front Pediatr. 2025 Jun 6;13:1525423. doi: 10.3389/fped.2025.1525423. eCollection 2025.
Pediatric living donor liver transplantation (pLDLT) has risen to standard of care for children with liver failure. Strong policies of centralization have led to exceptional results, reducing the risk of morbidity and mortality. As a counterpart, long term follow-up often occurs in centers with no dedicated transplant unit, that should familiarize themselves with the exceptional anatomy of the liver-transplanted child and their unique postoperative complications.
We reviewed all cases of acquired diaphragmatic hernia (DH) after pLDLT in the period following the establishment of a strong policy of centralization in Austria, between January 2017 and May 2023. All patients were referred for liver transplantation to the newly established national transplant reference center (Medical University of Innsbruck, Austria). Postoperative follow up was conducted either at the transplant center or at their home institutions.
Of the 42 patients which received a pLDLT in the national reference center during the study period, 3 developed an acquired diaphragmatic hernia within the first eight months postoperatively (7%). All patients required emergent surgical treatment in a non-transplant center. All the cases presented with a defect in the posteromedial aspect of the diaphragm, potentially related to thermal effects in the bare area of the diaphragm during transplantation.
Acquired diaphragmatic hernia is a rare complication of pLDLT, that mostly occurs in the long-term postoperative follow-up. Accurate knowledge of the surgical site is crucial to assure assessment and management in absence of the transplant-team. With this retrospective analysis we aim to enhance focus on post-liver-transplant complications and offer a guide for the non-transplant pediatric surgeon to raise awareness to post-operative anatomical alterations in these patients.
小儿活体肝移植（pLDLT）已成为肝衰竭患儿的标准治疗方法。强有力的集中化政策带来了卓越的治疗效果，降低了发病和死亡风险。相应地，长期随访往往在没有专门移植单元的中心进行，这些中心应熟悉肝移植患儿独特的肝脏解剖结构及其术后并发症。
我们回顾了2017年1月至2023年5月奥地利建立强有力的集中化政策后，所有pLDLT术后获得性膈疝（DH）的病例。所有患者均被转诊至新成立的国家移植参考中心（奥地利因斯布鲁克医科大学）进行肝移植。术后随访在移植中心或患者的家庭机构进行。
在研究期间，全国参考中心接受pLDLT的42例患者中，有3例在术后前八个月内发生了获得性膈疝（7%）。所有患者均在非移植中心接受了急诊手术治疗。所有病例均表现为膈肌后内侧缺损，可能与移植过程中膈肌裸区的热效应有关。
获得性膈疝是pLDLT的一种罕见并发症，大多发生在术后长期随访中。准确了解手术部位对于在没有移植团队的情况下确保评估和管理至关重要。通过这项回顾性分析，我们旨在加强对肝移植后并发症的关注，并为非移植小儿外科医生提供指导，以提高对这些患者术后解剖结构改变的认识。
