Wilson Disease With Giant Splenic Artery Aneurysms Caused by Fibromuscular Dysplasia During Living Donor Liver Transplantation: A Case Report |
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| Institution: | 1. Department of Hepato-Biliary-Pancreatic and Breast Surgery, Ehime University Graduate School of Medicine, Ehime, Japan;2. Department of Lifestyle-related Medicine and Endocrinology, Ehime University Graduate School of Medicine, Ehime, Japan;3. Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, Japan;4. Department of Molecular Pathology, Ehime University Graduate School of Medicine, Ehime, Japan;1. Department of Surgery, Seoul National University Bundang Hospital, Seongnam, Korea;2. Department of Surgery, Seoul National University College of Medicine, Seoul, Korea;3. Department of Surgery, Chungbuk National University Hospital, Cheongju, Korea;4. Charm Vein Center, Seoul, Korea;1. Renal Transplant, Hospital Alta Complejidad Pte JD Perón, Formosa, Argentina;2. Foundation for Research and Assistance of Renal Disease (FINAER), Buenos Aires, Argentina;3. Renal Transplant, Hospital Privado Córdoba, Argentina;4. Renal Transplant, Hospital Provincial del Centenario de Rosario, Argentina;5. Crai Sur Cucaiba, La Plata, Argentina;6. Instituto de Nefrología (Nephrology), Buenos Aires, Argentina;7. Renal Transplant, Hospital Italiano, Buenos Aires, Argentina;8. Renal Transplant, Hospital JC Perrando, Resistencia, Argentina;9. Clínica de Nefrología, Urología y Enfermedades Cardiovasculares, Santa Fe, Argentina;1. Department of Neurology, Sanjay Gandhi Post Graduate medical Sciences, Lucknow, India;2. Department of Radiology, Sanjay Gandhi Post Graduate medical Sciences, Lucknow, India;1. Department of Paediatric Bone Marrow Transplantation, Oncology and Hematology, Wrocław Medical University, Wrocław, Poland;2. Department of Paediatrics, Nephrology and Hypertension, Medical University of Gdańsk, Gdańsk, Poland;3. Laboratory of Immunopathology and Genetics, Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Łódź, Łódź, Poland;4. Department of Paediatrics, Diabetology and Endocrinology, Medical University of Gdańsk, Gdańsk, Poland;5. Department and Clinic of Haematology, Blood Neoplasms, and Bone Marrow Transplantation, Wrocław Medical University, Wrocław, Poland;6. Department of Paediatrics, Haematology and Oncology, Medical University of Gdańsk, Gdańsk, Poland;7. Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Łódź, Łódź, Poland |
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| Abstract: | Liver cirrhosis can cause splenic artery aneurysms (SAA) that pose a threat to patients undergoing liver transplantation. However, liver transplantation with multiple visceral artery aneurysms including giant SAA caused by arterial fragility has never been reported. We describe a 36-year-old man with decompensated liver cirrhosis due to Wilson disease that was complicated by giant SAA and multiple aneurysms in the bilateral renal arteries caused by fibromuscular dysplasia (FMD). The maximal diameter of the triple snowball-shaped SAA was 11 cm. We planned a 2-stage strategy consisting of a splenectomy with distal pancreatectomy to treat the SAA and subsequent living donor liver transplantation (LDLT) to address the liver cirrhosis. This strategy was selected to prevent fatal postoperative infectious complications caused by the potential development of pancreatic fistula during simultaneous procedures and to histopathologically diagnose the arterial lesion before LDLT to promote safe hepatic artery reconstruction. However, a postoperative pancreatic fistula did not develop after a splenectomy with distal pancreatectomy, and the pathologic findings of the artery indicated FMD. The patient underwent ABO-identical LDLT with a right lobe graft donated by his brother. Other than postoperative rupture of the aneurysm in the left renal artery requiring emergency interventional radiology, the patient has remained free of any other arterial complications and continues to do well at 2 years after LDLT. |
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