Janousek Libor, Novotny Robert, Kudla Michal, Oliverius Martin, Wohl Petr, Minguet Joan, Martinek Jan, Hucl Tomas, Fronek Jiri
Transplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
Department of Diabetology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
Case Rep Surg. 2019 Dec 22;2019:6064720. doi: 10.1155/2019/6064720. eCollection 2019.
Desmoid tumours (DT) are commonly associated with Gardener's syndrome. Their surgical resection may be complicated by their close proximity to major vessels, multiple organ involvement, and frequent local recurrence. Multivisceral transplantation (MVTx) is an alternative treatment for patients with intestinal and liver failure. In patients with DT closely associated with renal structures but without end-stage kidney disease, concomitant excision of the patient's own kidney, ex vivo tumour resection with nephron-sparing surgery, or autotransplantation has been proposed.
A 36-year-old Caucasian female weighing 60 kg with Gardener's syndrome with a history of abdominal surgery was presented to our department with progressive abdominal distention associated with paroxysmal pain. With the use of CT, the patient was diagnosed with a mass arising from the mesenterial region. The patient had normal kidney function and nonalcoholic steatohepatitis. The patient was indicated for MVTx.
After 16 months on the waiting list, the patient received a multivisceral graft from a deceased donor. Following the restoration of graft vascular flow, the patient's right kidney was removed and the DT dissected ex vivo before autotransplantation into the right pelvic fossa. The patient received immunosuppressive, antithrombotic, and antibiotic treatment. There was no acute rejection, though the patient experienced pulmonary infection, dysphagia, and oesophageal reflux with fungal infection. The patient had required temporary dialysis for acute renal failure for 75 days. One year after the surgery, nausea and violent vomiting caused delayed gastric emptying caused by spastic pylorus. Clinical improvement was achieved using gastric peroral endoscopic myotomy (G-POEM).
MVTx with kidney autotransplantation is a feasible treatment option in patients with familiar adenomatous polyposis complicated by an abdominal DT. Precise tumour dissection with nephron-sparing surgery was carried ex vivo. G-POEM was used to relieve MVTx-related gastroparesis. The patient had no disease reoccurrence after one-year follow-up.
硬纤维瘤（DT）通常与加德纳综合征相关。其手术切除可能因与主要血管距离近、多器官受累及频繁局部复发而变得复杂。多脏器移植（MVTx）是治疗肠和肝功能衰竭患者的一种替代疗法。对于与肾脏结构密切相关但无终末期肾病的DT患者，有人提出可同时切除患者自身肾脏、采用保留肾单位手术进行体外肿瘤切除或自体移植。
一名36岁、体重60千克、患有加德纳综合征且有腹部手术史的白种女性因进行性腹胀伴阵发性疼痛就诊于我科。通过CT检查，该患者被诊断为肠系膜区肿物。患者肾功能正常，患有非酒精性脂肪性肝炎。该患者适合进行MVTx。
在等待名单上等待16个月后，患者接受了来自已故供体的多脏器移植。恢复移植血管血流后，切除患者右肾，并在体外解剖DT，然后自体移植至右盆腔。患者接受了免疫抑制、抗血栓和抗生素治疗。虽未发生急性排斥反应，但患者出现了肺部感染、吞咽困难以及伴有真菌感染的食管反流。患者因急性肾衰竭需要临时透析75天。术后一年，恶心和剧烈呕吐导致幽门痉挛引起胃排空延迟。采用经口内镜下胃肌切开术（G-POEM）实现了临床改善。
对于合并腹部DT的家族性腺瘤性息肉病患者，MVTx联合肾脏自体移植是一种可行的治疗选择。采用保留肾单位手术在体外进行了精确的肿瘤解剖。G-POEM用于缓解MVTx相关的胃轻瘫。随访一年，患者无疾病复发。
