Schumacher Jonathan, Gutschow Christian Alexander, Inci Ilhan, Koelzer Viktor H, Opitz Isabelle
Department of Thoracic Surgery, University Hospital Zurich, Zurich, Switzerland.
Department of Surgery and Transplantation, University Hospital Zurich, Zurich, Switzerland.
Int J Surg Case Rep. 2022 Sep;98:107537. doi: 10.1016/j.ijscr.2022.107537. Epub 2022 Aug 24.
The management of large malignant tracheo-esophageal fistulas (TEF) is not standardized. Herein, we report a case with a malignant TEF associated with esophageal post-transplant lymphoproliferative disorder (PTLD) for whom we successfully performed a surgical repair. This contributes to the knowledge on how to treat large acquired malignant TEFs.
A 69-year old male presented with a one-week history of fever, productive cough and bilateral coarse crackles. In addition, he described a weight loss of 10 kg during the past three months. The patient's history included a kidney transplantation twenty years ago. Esophagogastroduodenoscopy with a biopsy of the esophagus was performed nine days before. Histopathology showed a PTLD of diffuse large B-cell lymphoma subtype. Subsequent diagnostics revealed a progressive TEF (approx. 2.0 × 1.5 cm) 3.0 cm above the carina. PET-CT scan showed an esophagus with slight tracer uptake in the middle third (approx. 11.5 cm length, SUV max 7.4). After decision against stenting, transthoracic subtotal esophagectomy with closure of the tracheal mouth of the fistula by a pedicled flap was performed. PTLD was treated with prednisone and rituximab. Tumor progression (brain metastasis) led to death 95 days after surgery.
The treatment of a malignant TEF is complex and personalized while both the consequences of the esophago-tracheal connection and those of the underlying responsible diagnosis have to be considered concurrently. In this case, we considered surgery as the best treatment option due to a relatively good prognosis of the underlying diagnosis (PTLD) and a large fistula. Esophageal or dual stenting, the treatment of choice for small malignant TEF, would have been associated with a high risk of failure due to the wide trachea, extensively dilated esophagus, proximal location and large diameter of the fistula.
Surgery can be considered for patients with a large acquired malignant TEF and positive long-term prognosis of the underlying diagnosis. Due to the complexity of TEF management, immediate pre-operative multidisciplinary discussion is advised.
大型恶性气管食管瘘（TEF）的治疗尚无标准化方案。在此，我们报告一例与食管移植后淋巴组织增生性疾病（PTLD）相关的恶性TEF病例，我们成功为其实施了手术修复。这有助于了解如何治疗大型后天性恶性TEF。
一名69岁男性，有一周发热、咳痰及双侧粗湿啰音病史。此外，他自述在过去三个月体重减轻了10千克。患者有20年前肾移植病史。九天前进行了食管胃十二指肠镜检查并取食管活检。组织病理学显示为弥漫性大B细胞淋巴瘤亚型的PTLD。后续诊断发现隆突上方3.0厘米处有一进展性TEF（约2.0×1.5厘米）。PET-CT扫描显示食管中三分之一处有轻微示踪剂摄取（约11.5厘米长，SUV最大值7.4）。在决定不进行支架置入后，实施了经胸次全食管切除术，并用带蒂皮瓣封闭瘘管的气管口。PTLD采用泼尼松和利妥昔单抗治疗。肿瘤进展（脑转移）导致患者术后95天死亡。
恶性TEF的治疗复杂且需个体化，同时必须兼顾食管气管连接的后果以及潜在病因诊断的后果。在本病例中*，*由于潜在诊断（PTLD）预后相对较好且瘘管较大，我们认为手术是最佳治疗选择。小型恶性TEF的首选治疗方法——食管或双支架置入，因气管宽、食管广泛扩张、瘘管位置靠近近端且直径较大，失败风险会很高。
对于大型后天性恶性TEF且潜在诊断有良好长期预后的患者，可考虑手术治疗。鉴于TEF管理的复杂性，建议术前立即进行多学科讨论。
