Intracranial hypertension (ICH) is a well-recognized and potentially fatal complication of acute liver failure. It is rarely observed in patients with chronic liver disease or acute-on-chronic liver failure (ACLF). Only a few studies have investigated the management of ICH in ACLF. Here, we present an uncommon case of acute-onset severe hyperammonemia in a patient with cirrhosis who developed fulminant ICH. Rapid institution of renal placement therapy and therapeutic plasma exchange achieved a dramatic reduction in the serum ammonia level, but did not slow the patient’s rapid neurological deterioration.

Ectopic varices are portosystemic collaterals that form outside the esophagogastric region. If left untreated, they can carry a high mortality rate. Although ectopic variceal bleeding occurs rarely, it continues to pose both diagnostic and therapeutic challenges and remains an under-recognized source of gastrointestinal (GI) hemorrhage. We present a case of GI bleeding from varices formed at the choledochojejunosomy site in a post-Whipple patient. This case describes the rare finding of ectopic varices after pancreaticoduodenectomy, emphasizes the challenges in diagnosis and treatment, and highlights the need for a multidisciplinary approach managing GI bleeding in this patient population.

Introduction: Rectal variceal bleeding can be catastrophic if treated inadequately. It is often difficult to identify and treat the site of rectal variceal bleeding. Endoscopic ultrasound (EUS) provides diagnostic and therapeutic advantages over other endoscopic, radiologic, and surgical therapies such as sclerotherapy, band ligation, transjugular intrahepatic portosystemic shunt, balloon-occluded retrograde transvenous obliteration, and suture ligation. Case reports have described successful treatment of rectal varices with EUS guided cyanoacrylate injection, however, there are no established guidelines that dictate management of rectal varices. Case Description/Methods: We describe 4 patients with cirrhosis who presented with life-threatening hematochezia secondary to bleeding rectal varices (Table 1). Two of the 4 patients had failed prior endoscopic and surgical interventions. In this case series, each patient was treated with EUS-guided injection of cyanoacrylate-lipiodol glue mixture at a 4:1 ratio. Embolization coils were placed for patient 2 to treat particularly large varices with relatively high blood flow. Following EUS-guided treatments, diminished or absent blood flow was observed by color doppler in all 4 cases with complete resolution of rectal bleeding. Rebleeding was seen in patient 1 only, 8 months after glue injection. That patient underwent a second glue injection and since then no further bleeding has occurred. No post-operative complications, such as glue emboli or sepsis, developed in any patients. Discussion: Recent case reports have shown that EUS guided therapies should be considered during the multidisciplinary discussion to treat bleeding rectal varices. The advantage of EUS over endoscopic injection is the direct visualization of the intramural varix. Consequently, less glue is required, and lower dilutions can be used, which decreases the chance of embolization. Furthermore, the benefits of adding coils to the glue, especially for larger varices with increased flows increases the odds of successful treatment with minimal risks. EUS doppler is particularly useful to confirm obliteration of flow in the treated varix. Given the high safety and efficacy of this technique we argue glue injection should be considered first line treatment for bleeding rectal varices. Table 1. - Note: all glue injections were performed using n-butyl-2-cyanoacrylate-lipidiol glue at 4:1 ratio Patient # 1 2 3 4 Age 75 79 80 46 Gender Male Feman Feman Male Etiology of cirrhosis Cryptogenic Alcohol PBC Alcohol Prior rectal varices bleed + intervention Glue injection None Band ligation, suture ligation None Finding on EUS Multiple rectal varices 3-5mm in diameter Multiple varices 8mm Multiple small varices, largest 3mm Multiple small varices, largest 4mm Amount of glue injected 1st EUS: 2mL, second EUS: 2mL 2mL into 3 sites 4, 4mL injections into 2 sites 3mL Coils None 2 MReye embolization coils None None Time since intervention/last bleeding episode 18 months 10 months 18 months (died) 10 months

Phenomenon:Little is known about how participation in disaster relief impacts medical students. During the terror attacks of September 11, 2001, New York Medical College School of Medicine students witnessed the attacks and then became members of emergency treatment teams at St. Vincent's Hospital, the trauma center nearest to the World Trade Center. To date, only two reports describe how 9/11 influenced the lives of medical students. This study was designed to characterize the short- and long-term effects on NYMC students and to compare those effects between students assigned to St Vincent's Hospital and classmates assigned to rotations at facilities more remote from the attack site. We hypothesized that participation in direct relief efforts by students assigned to the St. Vincent's site might have long-lasting effects on their lives and these effects might vary when compared to classmates assigned elsewhere.Approach: This was a retrospective, survey-based, unmatched cohort study. Participants included all school of medicine graduates who were St. Vincent's rotators on 9/11 (N = 22) and classmates (N = 24) assigned to other sites who could be contacted and agreed to participate. Our primary measure was whether the 9/11 experience affected the participant's life, defined as an affirmative response to the item which asked whether the 9/11 experience affected the participant's "life thereafter, career choice, attitudes toward life or attitudes toward practice." Secondary measures included self-reported effects on career, life, attitudes, health, resilience, personal growth, personality features, and the temporal relationship between the attack and stress symptoms.Findings: Completed surveys were received from 16/22 (73%) St. Vincent's and 18/24 (75%) non-Saint Vincent's participants: 62% male, 82% had children, 74% identified as Caucasian/white and 76% employed full-time. Overall, slightly more than half (58%) of respondents reported an effect of 9/11 on their life, with a greater but non-significant proportion of St. Vincent's rotators reporting life impact (67% versus 50% for St. Vincent's versus other locations, respectively). High post-9/11 stress levels, current marriage, and ability to make and keep family and social relationships were associated with an effect on life which approached statistical significance. Participants reported positive or no post 9/11 effects on empathy and altruism (50%), resilience (47%), attitudes toward medical practice and career (32%), and charitable giving (24%), while positive, negative, or no effects were reported for attitude toward life, family and social relations, physical health, and conscientiousness. Mental health was the only domain in which all participants reported unchanged or negative effects. Two St. Vincent's rotators but no students assigned elsewhere believed they experienced 9/11-related post-traumatic stress disorder.Insights:Just over half of New York Medical College School of Medicine students rotating at St. Vincent's Hospital on 9/11 or elsewhere reported significant life-effects as a result of direct/indirect experiences related to the attack. Perceived stress may have been a more important driver of this life-change than other factors such as geographic proximity to the disaster site and/or direct participation in relief efforts. Further study of medical school interventions focused on stress reduction among students who participate in disaster relief is warranted.

Introduction: Solitary rectal ulcer syndrome (SRUS) is a rare benign disorder where one or more ulcers develop in the rectum. The exact pathogenesis is not well identified but thought to be related to local ischemia or direct trauma. SRUS is associated with chronic constipation, rectal prolapse, straining during defecation, rectal bleeding and pain, and a sense of incomplete evacuation. We report a case of SRUS in an elderly female patient where endoscopic appearance of her rectum was concerning for neoplasm. Case Description/Methods: A 71-year-old female with past medical history of chronic constipation presented to clinic with occasional intermittent bright red rectal bleeding and fecal incontinence. The patient subsequently underwent elective colonoscopy and rectal endoscopic ultrasound (EUS). On distal examination a soft mass was palpated inside the rectum. Colonoscopy showed a large congested and friable polypoidal mass of approximately 5 cm in size, located about 3 cm from the anal verge and involving more than 50% of the luminal circumference. Multiple cold forceps biopsies were obtained. Upon EUS staging the rectal mass appeared to be confined to the mucosa and submucosa and no lymph nodes were noticed. Both internal and external sphincters also appeared hypertrophied. Pelvic magnetic resonance imaging (MRI) findings were suspicious for SRUS. Pathology on the rectal mass did not show any malignant cells and features were consistent with SRUS. The muscularis mucosae was hypertrophied with splaying fibers, and the proliferated smooth muscles were accompanied by variable fibrosis. Discussion: The incidence of SRUS is estimated 1 in 100,000 people per year. Due to its rare occurrence and varying presentations, SRUS is easily mistaken with inflammatory bowel disease, neoplasms, or other rectal conditions. The diagnosis is typically made by direct visualization of the lesion by colonoscopy, sigmoidoscopy, or endoscopy, and confirmed with histological findings. More recently typical cross-sectional imaging findings have been recognized with SRUS. Rectal endoscopic sonogram would show increased thickness of anal sphincters as seen in our case. MRI would illustrate thickened mucosa, even though this appearance is non-specific. Our case highlights that SRUS can masquerade as rectal pseudotumor.Figure 1.: A large congested and friable polypoidal mass of approximately 5cm in rectum was visualized via colonoscopy.Figure 2.: Both internal sphincter (marked by yellow cross signs) and external sphincter (marked by blue cross signs) were measured around 5 mm in size with EUS.Figure 3.: A hypoenhancing polypoid mass (marked by the blue arrow) within the rectum shown on MRI.Figure 4.: Pathology on rectal mass showed colonic mucosa with surface ulceration, epithelial hyperplastic changes, crypt distortion, hypertrophy / proliferation of smooth muscle and fibrosis in lamina propria, 100x; note the hypertrophy of the muscularis mucosae with splaying of fibers course into the lamina propria (black arrows) and the surface erosion and inflammation (blue arrows).

INTRODUCTION: Black esophagus is a rare endoscopic finding caused by acute esophageal necrosis (AEN). Studies suggest the incidence of AEN is less than 1%. We describe a case of a patient presenting with coffee ground emesis and black esophagus. CASE DESCRIPTION/METHODS: A 60-year-old male with a past medical history of HIV on HAART (CD4 count of 399 and undetectable VL), alcohol abuse, ESRD, DMII and HTN presented to the ED for intermittent hematemesis. The patient described several episodes of coffee ground emesis associated with worsening abdominal distention, pain and diarrhea. Two days prior, he was discharged from another hospital after being treated for the same condition, however EGD was not performed. His surgical history was significant for G-tube placement, one year prior. He was afebrile and hemodynamically stable. His abdomen was soft, distended, non-tender with normal BS. His rectal exam revealed melena. Initial labs were significant for a leukocytosis of 15.4 K/uL, Hg of 8.2 (MCV 83), Platelets 373, INR 1.3 and lactate of 1.8. CT demonstrated distal esophageal wall thickening, suggestive of esophagitis. EGD confirmed esophagitis with eschar (LA Grade D) at the mid and distal esophagus (45 cm from the incisors.) There was no extension of the lesion beyond the GE junction. No blood, clots or old heme was seen in the stomach. However, multiple non-bleeding clean base ulcers (Forrest Class III) were visualized in the duodenum. The patient's hematemesis was caused by acute esophageal necrosis. The patient was treated with fluid resuscitation and intravenous proton pump inhibitors. The patient declined further workup. He was discharged to a skilled nursing facility with plan for repeat upper endoscopy in 4-6 weeks. He declined repeat endoscopy. DISCUSSION: The pathogenesis of AEN is not fully understood however poor vascular perfusion may be a contributor. AEN manifests in the distal portion of the esophagus and frequently coexists with duodenal pathologies. Treatment begins with nil-per-os (NPO) restriction, intravenous proton pump inhibitors, fluid resuscitation and treatment of underlying medical comorbidities. Follow-up upper endoscopy is recommended in 4–6 weeks. Although uncommon, clinicians should consider AEN when evaluating patients with an upper GI bleed, as early recognition and prompt treatment may improve outcomes. Further studies are needed on treatment outcomes and survival when multiple comorbidities coexist.

INTRODUCTION: Autoimmune hepatitis (AIH) is acute or chronic liver disease characterized by specific autoantibodies and histologic findings. The incidence of AIH in individuals with Multiple sclerosis (MS), a chronic autoimmune demyelinating disease of the central nervous system, is reported to be 10-fold higher than in the general population. We present a series of 3 cases of AIH diagnosed in patients with MS. CASE DESCRIPTION/METHODS: Case 1: A 61-year-old woman with MS, presented with elevated liver enzymes. She was not on any treatment for her MS. Labs showed transaminitis and hyperbilirubinemia. Liver imaging was unremarkable. Toxicology was negative. Viral hepatitis panel was negative. Anti-nuclear antibody (ANA) and smooth muscle antibody (SMA) were positive. IgG was elevated at 6521 mg/dL. Liver biopsy showed panlobular necrosis with abundant plasma cells. She responded to steroid therapy with normalization of LFTs on two-month follow up. Case 2: A 56-year-old woman with MS presented in April 2020 with two weeks of jaundice and fatigue. She was not on any MS therapy or hepatotoxic medications. She had elevated liver enzymes and bilirubin. Liver imaging and viral serologies were unrevealing. ANA and SMA were negative. Liver kidney microsomal antibody (LKM-1) was not checked. Liver biopsy showed plasma-cell predominant interface hepatitis consistent with AIH. She responded to steroid therapy. Case 3: A 40-year-old woman with MS presented in 2019 with elevated liver enzymes and a bilirubin of 12.6 mg/dL. Her beta-interferon therapy had been changed to Glatiramer therapy in 2016. Viral serologies were negative. ANA was positive, SMA negative. Liver biopsy showed panlobular necrosis and clusters of plasma cells. She responded to steroids and azathioprine. DISCUSSION: AIH is a rare cause of liver disease that has been linked to multiple autoimmune conditions.The incidence of AIH in the MS population is estimated to be 10-fold higher than the general population, suggesting a possible link. To date, concurrence of AIH and MS has rarely been reported and mostly in conjunction with the use of disease modifying agents including interferon beta (IFN-B). In our case series, two patients were not on any MS therapy or hepatotoxic medications. Early recognition and treatment of AIH is associated with histologic remission and improved survival. Clinicians should maintain a high degree of suspicion for AIH in patients with MS presenting for evaluation of abnormal enzymes.Figure 1.: Low power picture (40×) shows panlobular necrosis of hepatocytes with abundant inflammatory cells. Arrow points to a portal tract.Figure 2.: Higher power (200×) view shows the inflammatory cells at the interface comprised of neutrophils, lymphocytes and plasma cells (arrow).Figure 3.: High power (200×) view shows the inflammatory cells at the interface comprised of neutrophils, lymphocytes and plasma cells (arrow). There is more hepatocyte dropout on the right than on the left from the interface hepatitis.

INTRODUCTION: Protein-losing enteropathy is an excessive loss of serum protein into the gastrointestinal (GI) tract and typically presents with diarrhea, hypoalbuminemia and weight loss. Constrictive pericarditis (CP) is a rare cause of this condition. We present a case of severe protein malnutrition caused by CP. CASE DESCRIPTION/METHODS: An 80-year-old man presented with diarrhea and weight loss. He had a remote history of rectal cancer treated with chemotherapy, radiation and a colostomy many years prior. He now presented with diarrhea and a 20 lb weight loss with inability to gain weight despite adequate intake. He also developed ascites and bilateral lower extremity edema. Labs were significant for slightly elevated AST and ALT, total protein 5.5 g/dL and albumin of 3.0 g/dL. Urinalysis was negative for protein. GI multiplex PCR and C-difficile PCR were negative. Upper endoscopy and colonoscopy, including colonic biopsies, were unrevealing. A computed tomography of the abdomen was negative for bowel wall thickening, lymphadenopathy or cirrhosis. A diagnostic paracentesis showed a serum albumin ascites gradient of 1.3g/dL and an ascitic protein of 2.6 g/dL. Echocardiogram showed a severely dilated right atrium, pericardial thickening and pronounced respiratory variation in ventricular filling. Right heart catheterization confirmed these findings. He was diagnosed with CP, likely related to the radiation therapy he had received to the chest. He underwent a pericardiectomy with biopsy showing fibro-connective tissue and chronic inflammation. Post-procedure, his weight improved and his albumin and total protein normalized after 6 months. DISCUSSION: A variety of conditions are known to cause bowel inflammation and increased gut permeability such as Crohn’s disease, lymphoma, and Celiac disease. Right heart failure from CP is a more rare cause. Clinical presentation can include abdominal pain, diarrhea and weight loss. Patients are often hypoalbuminemic and have elevated fecal alpha-1-antitrypsin level. CP is thought to cause protein-losing enteropathy due to increased systemic venous and lymphatic pressure with congestion of bowel wall lymphatic vessels, resulting in leakage of protein into the intestinal lumen. Similar to our patient, the enteropathy improves after pericardiectomy. A high degree of suspicion is needed to make this diagnosis and should be on the differential in patients with suspected protein-losing enteropathy in which the classic workup is unrevealing.

INTRODUCTION: Clostridium difficile (C. difficile) is the cause of 20–25% of antibiotic-associated diarrhea. Extracolonic manifestations of C. difficile are rare but include osteomyelitis and reactive arthritis. More rarely, the bacteria can colonize the small bowel and cause active small bowel enteritis with mortality reported as high as 25%. We present a case of C. difficile enteritis. CASE DESCRIPTION/METHODS: A 39-year-old woman with history of alcoholic liver cirrhosis and splenic embolization for thrombocytopenia one week prior to presentation was admitted for abdominal pain after paracentesis. Her labs were notable for leukocytosis, thrombocytopenia, elevated lactate and elevated INR. Computed-tomography of the abdomen revealed thickening of the proximal small bowel and right colon consistent with enterocolitis, splenomegaly and ascites. Ultrasound showed portal vein thrombosis. Her course was complicated by hepatic encephalopathy and spontaneous bacterial peritonitis. She was transferred to the intensive care unit and an indwelling intraperitoneal catheter was inserted to drain the ascites. The WBC of the peritoneal fluid peaked at 55,000/ml and cultures grew C. difficile. The serum WBC peaked at 66,500/ml and blood cultures also grew C. difficile. Stool was positive for toxigenic C. difficile PCR. Oral vancomycin, intravenous vancomycin and intravenous metronidazole were started. On the 10th day of admission, a donor liver became available and she was taken to the operating room (OR) for transplant. Multiple splenic and intra-abdominal abscesses were noted during the exploration phase of surgery and the surgery was cancelled. Decision was made to take the patient back to the OR for abdominal wash out and possible splenectomy. Unfortunately, she had cardiac arrest intraoperatively and expired after failed resuscitative efforts. DISCUSSION: The exact pathophysiology of C. difficile enteritis is not well-understood, especially in patients with intact small bowel. Majority of patients had prior gastrointestinal surgery such as colectomy. Our patient, however, had no prior abdominal surgeries or intervention. We presume that as a complication of her splenic embolization and raging peritonitis, she developed a portal vein thrombosis causing small bowel wall edema, allowing for translocation of C. difficile from her bloodstream into her small bowel and peritoneal cavity. There must be a high index of suspicion for C. difficile enteritis, as it is not a common entity and has a high mortality rate.

INTRODUCTION: Plasma exchange (PE) has been used to successfully treat hyperbilirubinemia in conditions such as primary biliary cholangitis. However, it is not routinely used to prevent worsening renal function due to bile cast nephropathy, as a bridge to liver transplant. We present a case of using PE to treat severe hyperbilirubinemia in a patient with acute on chronic liver failure prior to orthotopic liver transplantation (OLT). CASE DESCRIPTION/METHODS: This is a 62 year old woman with a past medical of NASH cirrhosis, esophageal varices, hepatic encephalopathy, and factor V leiden deficiency who presented to our institution for management of worsening liver function. On admission, her total bilirubin was 29.3mg/dl (baseline was around 5mg/dl), direct bilirubin 18mg/dl and MELD was 21. Hemolysis workup was negative. She had completed a course of azithromycin for pneumonia, raising concern for drug induced liver injury. MRI abdomen revealed portal vein thrombosis, splenomegaly, portal enteropathy, ascites and no biliary ductal dilation. Her course was complicated by worsening bilirubin up to 70mg/dl and subsequent worsening kidney function with a creatinine of 3.90 mg/dl concerning for cholestatic nephrosis. She was transferred to the intensive care unit where she received two sessions of PE. After PE her bilirubin improved to 40 mg/dl, her creatinine improved to 1.39 mg/dl and urine output improved. Patient was re-MELDed at 40. She successfully underwent OLT ten days after her last PE session. Post-OLT, her bilirubin and creatinine normalized. DISCUSSION: While we are transplanting at higher MELD scores, we see patients with severe hyperbilirubinemia, which can cause significant complications, including bile cast nephropathy. Cholemic nephrosis can cause renal injury from proximal tubulopathy to intrarenal bile cast formation. PE has been used to successfully treat hyperbilirubinemia in few cases of fulminant hepatic failure not amendable to OLT in an attempt to allow hepatocyte recovery and regeneration. However, it is not routinely used to treat hyperbilirubinemia in liver failure as a bridge to transplant. Preserving renal function is important for a successful post-transplant course. Our case is one of the few cases in the literature where PE was used as a bridge prior to OLT in patients with hyperbilirubinemia in the setting of acute on chronic liver failure. Hence we conclude that PE can be considered to treat hyperbilirubinemia to prevent renal failure as a bridge to transplant.

Gastric outlet obstruction (GOO) is a clinical syndrome that can manifest with a variety of symptoms, including abdominal pain, postprandial vomiting, early satiety, and weight loss. It is caused by either a benign or malignant mechanical obstruction or a motility disorder interfering with gastric emptying. Anatomically, the mechanical obstruction can be at the distal stomach, pyloric channel, or duodenum; and can be intrinsic or extrinsic to the stomach.

INTRODUCTION: Aortoduodenal syndrome is a rare phenomenon in which duodenal obstruction occurs secondary to an abdominal aortic aneurysm (AAA). It presents with symptoms of gastrointestinal obstruction and is often difficult to treat. We report a case of 79-year-old man who presented with symptoms of gastrointestinal obstruction and was found to have a large AAA compressing the third portion of his duodenum. CASE DESCRIPTION/METHODS: We present a case of a 79-year-old man who presented with a two month history of progressively worsening nausea, vomiting and inability to tolerate oral intake. Three month prior to presentation he underwent an endovascular aneurysm repair (EVAR) for treatment of an enlarging abdominal aortic aneurysm. Physical exam was notable for a grossly distended abdomen. Labs showed a WBC 14.3 x k/mm3, hemoglobin 9.3 g/dL, sodium 141 mEq/L, potassium 4.3 mEq/L, BUN 37 mg/dL, creatinine 2.69 mg/dL. Enteroscopy showed extrinsic compression at the third portion of the duodenum. Computed tomography of the abdomen/pelvis showed a 7 cm fusiform abdominal aortic aneurysm post stent placement. There was compression of the third and fourth portion of the duodenum by the aneurysm sac causing proximal dilatation including a dilated duodenum and stomach and a fluid-filled esophagus. A nasogastric tube was placed for decompression, during which time he aspirated and required intubation and vasopressor support. Due to extensive comorbidities, he was deemed not a candidate for endovascular or surgical intervention and palliative care was initiated. DISCUSSION: Aortoduodenal syndrome is a rare entity that requires a high index of suspicion to make the diagnosis. It is generally caused by a large abdominal aortic aneurysm with a mean reported AAA diameter of 79 mm. The exact mechanism remains unclear. The most frequent findings at presentation are vomiting, a pulsatile abdominal mass, abdominal pain, weight loss and electrolyte disturbances. Computed tomography can confirm the diagnosis of AAA, locate the point and extent of obstruction and rule out other causes of duodenal obstruction. Most cases also report massive dilation of the stomach and duodenum. The condition has classically been managed with repair of the aneurysm; open surgical repair is the best option to prevent aortic rupture and relieve the bowel obstruction, however, post-operative mortality rates remain high. Management requires a multi-disciplinary approach and is challenging.Figure 1.: Compression of the duodenum as it crosses midline by the aneurysm sac. (Chevron) Axial computed tomography angiogram images of the abdomen. The proximal duodenum is distended, (solid arrow) with decompression of the distal duodenum and proximal jejunum. (Arrowhead) The dilated stomach is partially visualized. (S).Figure 2.: Axial computed tomography angiogram images of the abdomen. Large fusiform dilatation of an infrarenal aortic aneurysm status post stent placement, measuring approximately 7 cm. (white arrows).

AORN JournalVolume 52, Issue 1 p. 57-58 Clinical Common Bile Duct Strictures Diagnosis, Management, Follow-Up Mary E. Front RN, Mary E. Front RNSearch for more papers by this authorStephen R. Wise MD, Stephen R. Wise MDSearch for more papers by this authorLarry C. Carey MD, Larry C. Carey MDSearch for more papers by this author Mary E. Front RN, Mary E. Front RNSearch for more papers by this authorStephen R. Wise MD, Stephen R. Wise MDSearch for more papers by this authorLarry C. Carey MD, Larry C. Carey MDSearch for more papers by this author First published: July 1990 https://doi.org/10.1016/S0001-2092(07)67285-8AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Volume52, Issue1July 1990Pages 57-58 RelatedInformation

INTRODUCTION: Human echinococcosis (HE) is a zoonosis and can cause life-threatening tissue cysts in the liver. We present a case of an unusual presentation of hepatic echinococcosis diagnosed and treated in the United States (US). CASE DESCRIPTION/METHODS: A 23-year-old woman who emigrated from Ecuador to the United States presents to the ED with headaches, fevers and abdominal pain associated with vomiting and rigors. She denied sick contacts and recent travel. She noted 4-5 similar episodes in the past that self-resolved. Her vitals were significant for a fever of 102.3°F and heart rate in 120s. Her labs were significant for a WBC 14.8 k/mm 3 but the comprehensive metabolic panel was within normal limits. Exam revealed a supple neck and right lower quadrant tenderness. Due for concerns of appendicitis, she got a CT A/P C+, which was significant for a 4.2 × 2.6 cm and 1.3 cm low-density lesion in the right hepatic lobe. She was started empirically on flagyl and ciprofloxacin and discharged the next day. An outpatient MRI confirmed a cystic neoplasm in segment 5 of the liver with septation. Serum workup included a negative echinococcus antibody and she was referred to a surgical oncologist. She underwent liver segment 4 and 5-6 tumor resections without any complications. Pathology revealed calcified echinococcal cysts with extensive necrosis and abscess formation suggestive of microscopic areas of rupture. She was started on albendazole and is doing well. DISCUSSION: Echinococcosis is a zoonotic disease, with a two-host lifecycle and occurs in humans when Echinococcus eggs infect the liver (70%) and lungs (20%). After ingestion of eggs, the embryos escape and penetrate the intestinal mucosa, enter the portal circulation and then are trapped in the liver. Human cystic echinococcosis (CE) is the most common type of HE. The greatest prevalence of HE is in temperate areas such as parts of South America, North Africa, Asia and Australia. Diagnosis is made on imaging by US, CT and MRI. Treatment includes surgical resection, percutaneous drainage and anti-parasitic agents. Although the vast majority of cases occur outside the US, cases within the US exist and are incidentally found. Despite progress in controlling echinococossis, this condition remains a public health problem in endemic countries and a cause of morbidity and mortality. It should be included on the differential of a patient from an endemic area found to have cystic lesions on liver.

INTRODUCTION: ECMO has been rarely used in the perioperative setting during liver transplantation (LT) and there is scarce data on its use. Many patients requiring LT have cardiopulmonary compromise due to complications of liver disease or other co-morbidities. We present a use of VA-ECMO as cardiopulmonary support during LT in a patient with pulmonary hypertension. CASE DESCRIPTION/METHODS: A 57-year-old man with a medical history of ETOH cirrhosis complicated by recurrent ascites, portal hypertension, and hypothyroidism was admitted for management of fluid overload despite compliance with low-salt diet and diuretics. He had multiple prior hospitalizations for fluid overload which were managed with intravenous diuretics. He was listed for liver and kidney transplant in New York. He was eventually started on hemodialysis for end stage kidney failure secondary to hepatorenal syndrome. He also had severe pulmonary hypertension (PH). He had an offer for liver transplant and went to the operating room (OR). However, a Swan-Ganz catheter was placed and showed mean PA pressure of 47 mm Hg with high cardiac output despite the use of vasodilators. After careful evaluation, his transplantation was aborted. Few weeks later, he was taken back to the OR for liver transplant and was placed on VA-ECMO electively. His cardiopulmonary status was carefully monitored and remained stable with VA-ECMO support during the procedure even with severe PH. His transplantation was successful, after which his hemodynamics improved significantly and the ECMO was removed in the OR. Subsequently, he underwent kidney transplant and his PH improved postoperatively. DISCUSSION: Severe cardiac and pulmonary dysfunction is a contraindication for LT. Hepatopulmonary syndrome is a complication of liver failure and can cause pulmonary hypertension, which was the case with our patient. Intraoperative use of ECMO, both VA and VV, have been used sparingly. However, over the recent years, the use of ECMO peri-operatively has had wider use although poor outcomes are still reported. Bleeding risk is extremely high due to the coagulopathic nature of liver disease as well as the anti-coagulation necessity during ECMO so it is imperative to limit the duration of ECMO. In conclusion, VV or VA- ECMO can be a useful therapeutic option in patients undergoing transplantation with significant cardiac or pulmonary compromise especially in New York where the waiting period for transplant is long.

INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital anomaly characterized by multiple venous malformations that can appear anywhere on the body, but most often involve the skin and gastrointestinal (GI) tract. GI bleeding usually presents in childhood or early adulthood as iron deficiency anemia. We report a case of BRBNS as a rare presentation of GI bleeding in an elderly patient with no prior history of cutaneous or GI tract involvement of the disease. CASE DESCRIPTION/METHODS: 87-year-old man with a past medical history of hypertension, coronary artery disease and atrial fibrillation (on Xarelto) was transferred to our hospital for evaluation of melena. He presented to an outside institution initially with complaints of dizziness and black tarry stools for 5 days prior to admission. He was hypotensive, labs were significant for hemoglobin (Hgb) of 5.5 g/dL and a positive stool occult blood test. Anticoagulation was discontinued. He underwent esophagogastroduodenoscopy (EGD), which showed friable mucosa in the duodenal bulb but no active source of bleeding. He received 8 units of packed red blood cells (PRBC), but his Hgb only improved to 7 g/dL. A repeat EGD was also unrevealing. He then underwent a nuclear scan, which showed a focus of bleed in left hemi-abdomen, but it was unclear if it was from the small or large bowel. On presentation to our hospital, his Hgb was 6.6 g/dL and he continued to have melena, requiring multiple PRBC transfusions. Push endoscopy revealed diffuse discontinuous multiple vascular blebs of the mucosa in the mid jejunum (Figure 1a), one of which was found bleeding (Figure 1b). Successful hemostasis was achieved with cauterization of the bleeding bleb with argon plasma coagulation (APC) (Figure 1c,d) and a hemoclip placement 2 cm distal to the lesion. Post-procedure, his Hgb remained stable above 8 g/dL throughout the hospitalization. DISCUSSION: Although BRBNS lesions can occur anywhere from mouth to anus, the small bowel is the most common site of GI tract involvement. Its presentation can vary from iron deficiency anemia to massive hemorrhage, intestinal torsion, and rupture. Some patients require lifelong iron replacement and repeated blood transfusions. Push endoscopy is the most important diagnostic modality for GI lesions. Treatment depends on the severity of lesions and can range from supportive therapies such as iron supplementation and blood transfusion to more invasive therapies such as mucosal resection, APC, sclerotherapy or bowel resection.

INTRODUCTION: Lemmel syndrome is a rare condition in which a periampullary duodenal diverticulum compresses the distal common bile duct (CBD) resulting in obstructive jaundice in the absence of choledocholithiasis or tumor. We present a rare case of Lemmel syndrome in an elderly patient below. CASE DESCRIPTION/METHODS: An 83-year-old woman with a past medical history of Crohn's disease and pancreatic cysts was transferred to our facility for management of biliary obstruction. She had noted a four-month history of progressive early satiety, post-prandial right upper quadrant abdominal pain, post-prandial emesis, poor oral intake, and significant weight loss. On presentation, labs were significant for WBC of 11.6 k/mm 3 , AST of 242 U/L, ALT of 1677 U/L, alkaline phosphatase of 1347 U/L, total bilirubin of 3.4 mg/dL, and lipase of 260 U/L. She underwent ERCP and was found to have a large periampullary diverticulum containing the major papilla. Cholangiogram revealed a tortuous bile duct and severe intra- and extrahepatic biliary dilation. A sphincterotomy was performed and multiple balloon sweeps were done with no stone extraction. A 10 F × 8 cm fully covered metal biliary stent (FCSEMS) was placed in the bile duct and she had improvement in her appetite, abdominal pain, and liver function tests. On follow up ERCP, the bile duct maintained a straightened position. DISCUSSION: Lemmel syndrome is characterized by obstructive jaundice caused by periampullary duodenal diverticulum (PAD) compressing the intra-pancreatic part of the CBD with resultant upstream dilatation of the extra- and intra-hepatic bile ducts. PAD is a pseudo-diverticula consisting of an extraluminal out-pouching of duodenal mucosa and lacks a muscularis layer. PAD develops within a 2-3 cm radius from the ampulla of Vater. Periampullary duodenal diverticuli are mostly asymptomatic and majority are diagnosed incidentally. When inflamed, they can cause pancreaticobiliary complications such as obstructive jaundice, cholangitis, pancreatitis, and stone formation. They are diagnosed on imaging such as CT scan, MRCP, and barium studies, which can show lateral compression of the distal CBD by the diverticulum. ERCP with sphincterotomy and biliary stent placement has shown to reduce the risk of morbidity and mortality, although diverticulectomy remains standard of care for PAD. Lemmel syndrome is rare but should be considered in the differential diagnosis for obstructive jaundice in patients with a periampullary duodenal diverticulum.