Familial Adenomatous Polyposis and Desmoid Tumor Treated with Multivisceral Transplantation and Kidney Autotransplantation: Case Report and Literature Review

Introduction. 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...

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Bibliographic Details
Published in:Case reports in surgery 2019, Vol.2019 (2019), p.1-5
Main Authors: Fronek, Jiri, Hucl, Tomas, Minguet, Joan, Wohl, Petr, Oliverius, Martin, Kudla, Michal, Novotný, Robert, Janousek, Libor, Martinek, Jan
Format: Article
Language:English
Subjects:
Abdomen
Abdominal surgery
Antibiotics
Autografts
Blood vessels
Care and treatment
Case Report
Case reports
Chronic kidney failure
Colorectal cancer
Computed tomography
Dialysis
Dysphagia
End-stage renal disease
Endoscopy
Esophagus
Familial adenomatous polyposis
Gardens & gardening
Gastric emptying
Genetic aspects
Genetic disorders
Graft rejection
Health aspects
Infections
Intestine
Kidney diseases
Kidney transplantation
Kidneys
Literature reviews
Liver
Liver diseases
Localization
Mortality
Nausea
Ostomy
Pain
Pancreas
Patients
Polyps
Renal failure
Restoration
Surgery
Transplantation
Transplants & implants
Tumors
Valganciclovir
Vomiting
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Summary:Introduction. 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. Case Presentation. 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. Management and Outcome. 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). Conclusion. 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.
ISSN:2090-6900
2090-6919
DOI:10.1155/2019/6064720