Syed Amer

De Novo Malignancies After Transplantation: Risk and Surveillance Strategies

De novo malignancies are one of the leading causes of late mortality after liver and kidney transplantation. Nonmelanoma skin cancer is the most common malignancy, followed by posttransplant lymphoproliferative disorder and solid organ tumors. Immunosuppression is a key factor for cancer development, although many other transplant-related and traditional risk factors also play a role. In this review, the authors summarize risk factors and outcomes of frequently encountered de novo malignancies after liver and kidney transplantation to stratify recipients at highest risk. Future efforts in prospectively validated, cost-effective surveillance strategies that improve survival of these complex patients are greatly needed.

Gastrointestinal bleeding with continuous-flow left ventricular assist devices

Continuous-flow left ventricular assist device (CF-LVAD) insertion is a life-saving procedure that is being increasingly used in patients with advanced heart failure. However, patients with CF-LVADs are at an increased risk of gastrointestinal bleeding (GIB). Bleeding can occur anywhere in the GI tract with lesions being more prevalent in the upper GI tract than in the lower GI tract. The pathophysiology of GIB in patients with CF-LVADs is unique and likely involves three synergistic mechanisms—coagulopathy, acquired von Willebrand disease and continuous non-pulsatile blood flow. Management strategies vary depending on the presentation and site of bleeding. Prevention strategies to prevent GIB in these patients include low pump speed, close hemodynamic monitoring and a low threshold for endoscopy. We aim to review in detail the pathophysiology, management, complications and preventive strategies in patients with CF-LVAD who present with GIB.

Images of the Month: Gastric Pneumatosis Due to Sevelamer-Mediated Necrosis.

A 64-year-old-man with a history of end-stage renal disease (ESRD) who was on hemodialysis presented with bloody emesis and abdominal pain. Abdominal computed tomography showed gastric pneumatosis (left; arrows point to air in the posterior wall of the gastric fundus). Upper endoscopy revealed diffuse, severely ulcerative mucosa in the gastric body (center). Biopsy revealed sevelamer crystals (right; arrows), in the lamina propria of the body of the stomach. Notice the two-tone color, with a pale, brownish background and pink veins, defining the broad curved scales. Sevelamer is an anion-exchange resin used to treat hyperphosphatemia in ESRD patients. Sevelamer was discontinued, and the patient was treated with a proton pump inhibitor and a low-phosphate diet.

Endoscopic retrograde cholangiopancreatography in patients with surgically altered gastrointestinal anatomy

Endoscopic retrograde cholangiopancreatography (ERCP) in patients with surgically altered upper gastrointestinal anatomy, such as Roux-en-Y gastric bypass (RYGB), can be more challenging compared to those with a normal anatomy. Detailed assessment of cross-sectional imaging features by the radiologist, especially the pancreaticobiliary anatomy, strictures, and stones, is very helpful to the endoscopist in planning the procedure. In addition, any information on enteral anastomoses (for e.g., gastrojejunal strictures and afferent limb obstruction) is also very useful. The endoscopist should review the operative note to understand the exact anatomy prior to procedure. RYGB, which is performed for medically complicated obesity, is the most commonly encountered altered anatomy ERCP procedure. Other situations include patients who have had a pancreaticoduodenectomy or a hepaticojejunostomy. Balloon-assisted deep enteroscopy (single and double-balloon enteroscopy) or rotational endoscopy is often used to traverse the length of the intestine to reach the papilla. In addition, ERCP in these patients is further challenging due to the oblique orientation of the papilla relative to the forward viewing endoscope and the limited enteroscopy-length therapeutic accessories that are currently available. Overall, reported therapeutic success is approximately 70–75% with a complication rate of 3–4%. Alternative approaches include percutaneous transhepatic cholangiography, laparoscopy-assisted ERCP, or surgery. Given the complexity, ERCP in patients with surgically altered anatomy should be performed in close collaboration with body imagers, interventional radiology, and surgical services.

A Comprehensive Review of Progressive Familial Intrahepatic Cholestasis (PFIC): Genetic Disorders of Hepatocanalicular Transporters

Progressive familial intrahepatic cholestasis or PFIC is a general term used to describe a group of genetic disorders involving the hepatocanalicular transporters. These diseases are characterized by persistent cholestasis, pruritus and jaundice. Type I PFIC is characterized by defect in the gene that codes for aminophospholipid translocase protein and maintains canalicular membrane stability. Types 2 and 3 are caused by defect in genes that code for bile acid transporter and a phospholipid translocase, respectively. This review summarizes the genetics, clinical features, diagnosis and treatment of the three types of PFIC.

Rare case of congenital absence of left internal carotid artery

While many patients remain asymptomatic due to robust collateral pathways, problems can arise later in life, particularly with progression of atherosclerotic disease. The absence of the carotid artery has also been associated with an increased incidence of cerebral aneurysms, which are reported to be 24-34%, compared to 2-4% in the general population. This is thought to be related to increased and altered flow dynamics though the collateral vessels.[3]

Images of the Month

An 80-year-old man who had had an esophagectomy for esophageal adenocarcinoma complicated by multiple dilations of the anastomosis presented with cardiogenic shock and abdominal pain following a repeat esophageal dilation.(a) A chest X-ray showed bowel loops in the left hemithorax. (b) Computed tomography showed a large hiatal hernia with distended stomach in the right chest, with the colon and small bowel occupying the left hemithorax, displacing the surrounding organs to the contralateral side. The loops caused an evolving tension colothorax leading to tamponade. The patient recovered after an emergent surgical reduction of the hernia and repair of the diaphragm.

Tension colothorax following esophageal dilation

An 80-year-old man who had had an esophagectomy for esophageal adenocarcinoma complicated by multiple dilations of the anastomosis presented with cardiogenic shock and abdominal pain following a repeat esophageal dilation.(a) A chest X-ray showed bowel loops in the left hemithorax. (b) Computed tomography showed a large hiatal hernia with distended stomach in the right chest, with the colon and small bowel occupying the left hemithorax, displacing the surrounding organs to the contralateral side. The loops caused an evolving tension colothorax leading to tamponade. The patient recovered after an emergent surgical reduction of the hernia and repair of the diaphragm.

Images of the Month: Tension Colothorax Following Esophageal Dilation.

An 80-year-old man who had had an esophagectomy for esophageal adenocarcinoma complicated by multiple dilations of the anastomosis presented with cardiogenic shock and abdominal pain following a repeat esophageal dilation.(a) A chest X-ray showed bowel loops in the left hemithorax. (b) Computed tomography showed a large hiatal hernia with distended stomach in the right chest, with the colon and small bowel occupying the left hemithorax, displacing the surrounding organs to the contralateral side. The loops caused an evolving tension colothorax leading to tamponade. The patient recovered after an emergent surgical reduction of the hernia and repair of the diaphragm.

Pyogenic granuloma of the gastrointestinal tract

Introduction: Pyogenic granuloma (PG) or granuloma pyogenicum is essentially a capillary hemangioma on the skin or a mucosal surface which shows an exophytic growth pattern and has a lobulated appearance. The most common sites are skin (40%) and mucosal surfaces (predominantly oral cavity, 60%). We intend to report all available cases of PG of gastrointestinal (GI) tract, diagnosed at the Henry Ford hospital, a tertiary referral center. Patients and Methods: A retrospective review of pathological database was performed on all GI biopsies in the last 10 years using diagnostic codes and pathology codes searching for PG of the GI tract. Results: A total of 23 cases of pathologically diagnosed PG was diagnosed over a 10 year period. The median age of patients was 64 with almost equal gender distribution (47.8% were males, and 52.2% were females). The most common location of PG was sigmoid colon (65.2%), esophagus (17.4%) and transverse colon (13%). PG presented as a polyp in 16 patients (69.6%). The most common indication for endoscopy in these cases was screening colonoscopy (30.4% cases). Discussion: PG of GI tract is rare. To date, only about 15-20 cases have been reported in the literature and most cases have been reported from Japan and Korea. This is the largest case series of this rare pathological lesion of the GI tract. Most cases of PG were diagnosed on an endoscopy done for an unrelated reason in our series. Hence, most cases were asymptomatic, unlike previously reported cases which were mostly associated with GI bleeding.