Transesplenic access in the treatment of varicose gastrointestinal bleeding. Case and technical report / Uso do acesso transesplênico no tratamento do sangramento gastrointestinal varicoso. Relato de caso e técnica

ABSTRACT Varicose gastrointestinal bleeding is one of the major causes of morbidity and mortality in patients with chronic liver disease. Endoscopic treatment is the first therapeutic line for these patients, however, for those whom this therapeutic modality fail, a broad knowledge of alternative treatment options may improve the prognosis. We describe a case of a patient who were successfully embolized from gastroesophageal varices via transsplenic access.

RESUMO O sangramento gastrointestinal varicoso está entre as maiores causas de morbimortalidade nos paciente com doença hepática crônica. O tratamento endoscópico é a primeira linha terapêutica neste pacientes, porém naqueles que apresentam falha nesta modalidade terapêutica, o amplo conhecimento de opções alternativas de tratamento pode melhorar o prognóstico. Descrevemos um caso de paciente submetido à embolização com sucesso de varizes gastresofágicas por acesso transesplênico.

Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Portal-Splenic-Mesenteric Venous Thrombosis in a Patients with Protein S Deficiency due to Novel PROS1 Gene Mutation / 대한소화기학회지

Protein S (PS), a vitamin K-dependent glycoprotein, performs an important role in the anticoagulation cascade as a cofactor of protein C. Because of the presence of a pseudogene and two different forms of PS in the plasma, protein S deficiency (PSD) is one of the most difficult thrombophilias to study and a rare blood disorder associated with an increased risk of thrombosis. We describe a unusual case of previously healthy 37-year-old man diagnosed with portal-splenic-mesenteric vein thrombosis secondary to PSD. The patient was admitted to the hospital due to continuous nonspecific abdominal pain and nausea. Abdominal computed tomography revealed acute venous thrombosis from inferior mesenteric vein to left portal vein via splenic vein, and laboratory test revealed decreased PS antigen level and PS functional activity. Conventional polymerase chain reaction and direct DNA sequencing analysis of the PROS1 gene demonstrated duplication of the 166th base in exon 2 resulting in frame-shift mutation (p.Arg56Lysfs*10) which is the first description of the new PROS1 gene mutation to our knowledge. Results from other studies suggest that the inherited PSD due to a PROS1 gene mutation may cause venous thrombosis in a healthy young man without any known predisposing factor.

Two Cases of Portal Annular Pancreas / 대한소화기학회지

Portal annular pancreas is one of the pancreatic fusion anomalies in which the uncinate process of the pancreas extends to fuse with the dorsal pancreas by encircling the portal vein or superior mesenteric vein. We report two consecutive patients with portal annular pancreas. The first case is a 71-year-old male patient who underwent a pancreaticoduodenectomy for intraductal papillary mucinous neoplasm in the head of pancreas. His preoperative computed tomography scan showed the suprasplenic type portal annular pancreas. The second case is a 74-year-old female patient who underwent a laparoscopic anterior radical antegrade modular pancreatosplenectomy (RAMPS) for pancreatic body cancer. In operative finding, portal confluence (superior mesenteric vein-splenic vein-portal vein) was encased with the uncinate process of pancreas in both cases. Therefore, they required pancreatic division at the pancreatic neck portion twice. During the postoperative period, grade B and A, respectively, postoperative pancreatic fistulas occurred and were controlled by conservative management. Surgeons need to know about this rare pancreatic condition prior to surgical intervention to avoid complications, and to provide patients with well-designed, case-specific pancreatic surgery.

Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt / 대한간학회지

Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.

Primary Extraskeletal Mesenchymal Chondrosarcoma Arising from the Pancreas

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.

Sonographic signs in portal hypertension: a multivariate analysis.

A prospective case controlled study was conducted to evaluate the value of real time ultrasound in establishing the diagnosis and the etiology of portal hypertension. Patients attending the outpatient department of a tertiary care centre were included. There were 324 consecutive patients with portal hypertension due to cirrhosis (n = 229), non cirrhotic portal fibrosis (NCPF: n = 64) and extrahepatic portal venous obstruction (EHPVO: n = 31). During this period, 146 patients with dyspepsia, 35 with splenomegaly and 32 with ascites due to varied causes served as negative and positive controls. Real time ultrasonography using a 3.5 MHz linear array scanner was performed in a fasting state in all subjects. Portal and splenic vein diameter greater than 10 mm, splenomegaly, hepatic and splenic hilar collaterals were suggestive of portal hypertension. Non visualization of the portal vein which was replaced by a cavernoma had a diagnostic accuracy of 98% in EHPVO. Splenic infarcts and absence of ascites were features of non cirrhotic portal hypertension (NCPF and EHPVO). Sonography had an overall diagnostic accuracy of 80%. A stepwise logistic regression with multivariate analysis using discriminate function showed that collaterals at the hepatic and splenic hilum, hepatomegaly, ascites and splenic infarcts were independent markers to differentiate cirrhotic from noncirrhotic causes of portal hypertension. The discriminate equation generated had a mismatch of 9.8%. Correlations between the sonographic signs demonstrated that the variceal grade correlated positively with the presence of splenic hilar collaterals and the liver size inversely correlated with presence of ascites. It was concluded that real time ultrasonography is an accurate method to establish the presence and etiology of portal hypertension.