Whipple's Procedure in a Renal Transplant Recipient with Polycystic
Total Page：16
File Type：pdf, Size：1020Kb
JOP. J Pancreas (Online) 2008; 9(4):515-519. CASE REPORT Whipple’s Procedure in a Renal Transplant Recipient with Polycystic Liver Disease Hugo Bonatti1, Kevin Huguet1, Sarah McLaughlin1, Andrew Stockland2, Jaime Aranda-Michel3, Mohamed Al Haddad3, Marjorie Dougherty1, Peter Fitzpatrick4, George Kim4, Kirk Martin1, Ronald Hinder1, Justin H Nguyen1 Departments of 1Surgery, 2Radiology, 3Gastroenterology, and 4Medicine; Mayo Clinic. Jacksonville, FL, USA ABSTRACT [6, 7]. Hepatic cysts can be treated by percutaneous drainage, laparoscopic de- Context Polycystic disease is a rare disorder, roofing, or liver resection and occasionally, which most commonly manifests in the polycystic liver disease can lead to kidney and liver. Recently an increased risk complications that require liver transplant- for pancreatic malignancies in subsets of ation [8, 9]. Some authors have suggested patients with polycystic disease has been combined liver/kidney transplantation if both reported. organs are involved [10]. Case report We report a patient with Recently, an increased mortality rate due to polycystic liver and kidney disease who other malignancies has been reported in successfully underwent a Whipple’s patients who previously underwent renal procedure for pancreatic adenocarcinoma. transplantation for polycystic kidney disease [11]. Among these patients, one patient died Conclusion Although technical difficulty from pancreatic cancer [11]. In particular, for may increase, pancreaticoduodenectomy can polycystic kidney disease Potter III patients be safely performed in patients with an increased rate of developing pancreatic polycystic liver disease. cancer is well established [12, 13]. Polycystic livers can grow to an enormous size and occupy a large amount of the available intra- INTRODUCTION abdominal space [14]. This results in Pancreatic resection remains associated with a significant discomfort for the patient and high morbidity, particularly in patients with dislodgement of other intra-abdominal organs underlying comorbidities or those who have with distortion of the vascular anatomy. had previous abdominal surgery [1, 2]. Frequently, the entire small and large bowels Polycystic disease is a rare disorder, which are forced into the left lower quadrant. most commonly manifests in the kidney [3, Understandably, liver transplantation in these 4]. The liver is the second most commonly cases is technically challenging especially involved organ [5]. Polycystic kidney disease when dealing with a liver weighing as much is a common indication for kidney as 20 kilograms [8]. Similarly, any upper transplantation and frequently requires gastrointestinal tract procedure must be bilateral nephrectomy due to pain, considered difficult due to the lack of space hemorrhage into cysts or infection of the cysts and the abnormal anatomy [15]. We report a JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 9, No. 4 - July 2008. [ISSN 1590-8577] 515 JOP. J Pancreas (Online) 2008; 9(4):515-519. patient with polycystic liver and kidney disease who successfully underwent a Whipple’s procedure for pancreatic adenocarcinoma. CASE REPORT The patient was a 71-year-old slightly obese African American female with polycystic liver and kidney disease. She underwent bilateral native nephrectomy with splenectomy in 1997 and subsequently a cadaveric kidney transplantation in 1999. Graft function was excellent and the current immunosuppression consisted of triple drug therapy including cyclosporine A with trough levels between 50 and 100 ng/mL, mycophenol-mofetil (2 g daily), and prednisone (5 mg daily). Because of persistent abdominal pain over the last four years, she had multiple percutaneous cystotomies and ablations of liver cysts. In November 2005, the patient presented with symptoms of cholangitis and sepsis. Ultrasound of the right upper quadrant revealed a dilated common bile duct and a markedly enlarged liver with Figure 2. CT scan. a. Pancreatic mass. b. Polycystic multiple cysts replacing virtually all of the liver with dislocation of intra-abdominal organs. normal hepatic parenchyma. An endoscopic retrograde cholangiopancreatogram (ERCP) Brushings were obtained and a 10 French was then performed which confirmed the long biliary endoprosthesis was placed. Pathologic distal common bile duct stricture (Figure 1). examination of the brushings revealed adenocarcinoma. Further investigations included a computed tomography (CT) scan (Figure 2) showing a hypodense mass measuring 1.8x2.2 cm in the head of the pancreas and a massive liver with multiple intrahepatic cysts. A FDG-PET scan revealed a focus of intense hypermetabolic activity correlating with the pancreatic head mass seen on CT. An endoscopic ultrasound demonstrated a 1.8x1.6 cm hypoechogenic mass in the head of the pancreas. There was some focal dilation of the proximal pancreatic duct. No peripancreatic lymphadenopathy was identified. In December 2005, the patient underwent pancreaticoduodenectomy. A bilateral subcostal incision was used to access the abdominal cavity. Because of the polycystic liver disease and the extremely enlarged liver, Figure 1. ERCP: stenosis of the common bile duct. the right hepatic lobe extended caudally JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 9, No. 4 - July 2008. [ISSN 1590-8577] 516 JOP. J Pancreas (Online) 2008; 9(4):515-519. across the midline pushing all intra-abdominal comorbidities and has undergone kidney organs inferiorly and to the left. A classical transplantation. Her postoperative course was pancreaticoduodenectomy including a prolonged but she ultimately recovered well, thorough retroperitoneal lymph node however she died unrelated to the surgical dissection was carried out despite the procedure from her underlying comorbidities. enormous size of the liver. After mobilization Several aspects make this case unique. First, of the right hemicolon and completion of little is known on the development of Kocherization of the duodenum, the pancreatic cancer following solid organ gallbladder was taken down and the common transplantation [15]. However, there is bile duct was divided immediately below the evidence that patients with polycystic diseases insertion of the cystic duct. The proximal bile have an increased risk of developing a duct was submitted for frozen section that was pancreatic malignancy [16, 17]. Second, it is negative. During the reconstruction phase, not known whether the conventional pancreatic and biliary stents were placed immunosuppression with calcineurin inhibitor across the respective anastomoses and would promote pancreatic malignancy. externalized through the right abdominal wall. Recent reports show that mTOR inhibitors do A Jackson-Pratt drain was placed beneath the have an antitumor effect [18]. It has thus been pancreaticojejunostomy and brought out suggested that patients who develop de novo through the left abdominal wall. The post-transplant malignancies should be intraoperative course was unremarkable and switched to sirolimus or everolimus [19]. the patient was returned to the surgical Sirolimus may have a beneficial impact on the intensive care unit and was extubated on the course of pancreatic cancer [20]. Sirolimus as following day without incident. The final part of the chemotherapeutic regimen may be pathology revealed a 2.3 cm pancreatic superior to the standard regimen included adenocarcinoma with three of 21 lymph nodes gemcitabine [21]. However, judious care must positive for metastatic disease. The final be taken when using mTOR inhibitors during staging was pT3N1M0, G2. the immediate postoperative period due to the Stress steroid doses were given antiproliferative effects that may result in perioperatively and tapered to the baseline severe wound healing disturbances [22]. A level. Her postoperative course was failure of pancreatic anastomosis can lead to complicated by poor nutritional intake and disastrous complications [23]. oral herpes simplex virus infection. The The third interesting aspect in this case is the herpes simplex virus infection was surgical procedure. Due to previous bilateral successfully treated with acyclovir 400 mg nephrectomies and a splenectomy, there were twice daily. The function of the kidney multiple adhesions. Moreover, the anatomy of allograft remained stable throughout her the abdominal organs was significantly hospitalization with measured creatinine distorted due to the enormous liver [24]. levels less than 1.0 mg/dL. She was Nevertheless, the intraoperative blood loss discharged four weeks later. She was doing was low. Comprehensive management of this well at three months follow up and conversion patient requires multi-specialty collaboration of immunosuppression to a mammalian target including transplant nephrology, hepatology, of rapamycin (mTOR) inhibitor was interventional radiology, oncology, discussed. Unfortunately, the patient expired hepatobiliary and transplant surgery. For this due to cardiac failure unrelated to the surgical particular case the involvement of a transplant procedures few weeks later. team certainly may have been beneficial. Maeda et al. reported on an emergency DISCUSSION pancreaticoduodenectomy for pancreatic This case shows the feasibility of pancreatic metastasis from renal cell carcinoma in a resection in a patient with a massively patient with von Hippel-Lindau disease [25]. enlarged polycystic liver who suffers multiple Also this patient had undergone nephrectomy. JOP. Journal of the Pancreas - http://www.joplink.net - Vol.