Sudeep R. Shah

Transabdominal extensive esophagogastric devascularization with gastroesophageal stapling for management of noncirrhotic portal hypertension: long-term results.

Abstract. Outside Japan portosystemic shunts have been favored as the surgical procedure of choice for the management of portal hypertension of noncirrhotic etiology. Devascularization procedures have resulted in high rebleed rates probably owing to a limited extent of devascularization. We performed this study to assess the efficacy of our modification of Sugiuras procedure for long-term control of variceal bleeding in patients with noncirrhotic portal hypertension. Forty-six patients with extrahepatic portal venous obstruction (EHPVO) and 22 with noncirrhotic portal fibrosis (NCPF) were subjected to transabdominal extensive esophagogastric devascularization with esophageal or gastric stapled transection (modified Sugiuras procedure), 38 in an emergency situation and 30 electively. Follow-up endoscopies were performed every 6 months. Operative mortality, morbidity, variceal status, and causes of recurrent bleeding were evaluated. The postoperative mortality was 4%. Early procedure-related complications were seen in 6%, and esophageal strictures formed in 7 of 45 survivors undergoing esophageal transection (15%). Over a mean ± SD follow-up of 53 ± 34 months, 95% of patients were free of varices. Seven survivors (11%) had a rebleed, but only 5% were due to varices (two esophageal, one gastric). Six (9%) patients developed gastropathy. The 5-year survival was 88%. The modified Sugiuras procedure is safe and effective for long-term control of variceal bleeding especially in the emergency setting and in patients with anatomy unsuitable for shunt surgery or if surgical expertise for a shunt operation is not available.

Colorectal cancers in ulcerative colitis from a low-prevalence area for colon cancer.

AIM To determine the incidence and risk factors for colorectal cancer (CRC) in patients with ulcerative colitis from a low prevalence region for CRC. METHODS Our prospective database yielded a cohort of 430 patients [age: 44 ± 14.6 years; 248 men (57.7%)] with ulcerative colitis (median disease duration 6, range: 1-39 years) for analysis. Of these, 131 (30.5%) had left-sided colitis and 159 (37%) extensive colitis. Patients with histologically confirmed CRC within the segment with colitis were compared with those without CRC, to determine the risk factors for the development of CRC. RESULTS Twelve patients (2.8%) developed CRC. The overall incidence density was 3.56/1000 patient-years of disease - 3/1000 in the first 10 years, 3.3/1000 at 10 to 20 years, and 7/1000 at > 20 years. Three of our 12 patients developed CRC within 8 years of disease onset. On univariate analysis, extensive colitis, longer duration of disease, and poor control of disease were associated with development of CRC. On multivariate analysis, duration of disease and extent of colitis remained significant. CONCLUSION CRC occurred in 2.8% of patients with ulcerative colitis in our population - an incidence density similar to that in Western countries in spite of a low overall prevalence of colon cancer in our population. The risk increased with extent and duration of disease.

Bile duct stricture due to caused by portal biliopathy: Treatment with one-stage portal-systemic shunt and biliary bypass

Portal biliopathy is a rare complication of extrahepatic portal vein obstruction. Jaundice occurs in symptomatic patients with fibrotic strictures. Short-term improvement in such patients can be achieved with endoscopic retrograde cholangio-pancreatography with balloon dilatation and stent placement. Surgery in these patients is traditionally two staged. We report the results of a one-stage procedure combining non-selective portal-systemic shunt surgery with biliary bypass, performed successfully on a 24-year-old man with a tight biliary stricture resulting from portal biliopathy. At 18-month follow up, the patient shows he is doing well, with normal liver function tests.

Temporal Change in Phenotypic Behaviour in Patients with Crohn’s Disease: Do Indian Patients Behave Differently from Western and Other Asian Patients?

BACKGROUND AND AIMS In Western studies, one-third of patients with Crohns disease have stricturing or penetrating disease at presentation and one-half will progress to complicated disease in 20 years. Asian studies indicate that the Asian disease phenotype may be different. Our aim was to study the disease behaviour in Indian patients with Crohns disease. METHODS In this hospital-based study, we analysed [Montreal classification] disease phenotype, presence of perianal disease, need for intestinal surgery, and changes in the Montreal classification over time in Crohns disease patients from our database. RESULTS In the 178 patients (median age 35, interquartile range [IQR] 21 years; 97 males) with Crohns disease, the proportion of various features was as follows. More patients had ileo-colonic[L3: 43.8%] than ileal[L1: 27.5%] or colonic[L2: 28.7%] disease. Perianal disease was seen in 11.8% at baseline. Non-stricturing, non-fistulising disease[B1] was seen in 74.7%, 65.7%, 50%, and 44.4% at baseline, at 5, 10 and 15 years, respectively. Stricturing disease[B2] was seen in 21.4%, 21.9%, 28.9%, and 33.3%; penetrating disease[B3] in 3.9%, 11.4%, 21%, and 16.7%; and intestinal surgery was required in 10.7%, 20%, 34.2%, and 55.5%, respectively. KaplanMeier analysis showed no association between progression of disease and patient age or the location of the disease. CONCLUSIONS Gender distribution and predominant ileo-colonic location of disease were similar to earlier Asian reports on Crohns disease. Perianal disease was less frequent than reported in Western and other Asian studies. One-fourth of Indian patients had aggressive disease at diagnosis, but the tendency to progress towards aggressive disease over time was less pronounced than in Western patients.

Choledochal Cysts with Chronic Pancreatitis in Adults: Report of Two Cases with a Review of the Literature

CONTEXT Choledochal cysts, rarely present with chronic calcific pancreatitis. We report two patients with choledochal cysts who had concomitant chronic pancreatitis. CASE REPORT #1: A 27-year-old female with a history of recurrent abdominal pain, fever and jaundice presented with a type I choledochal cyst with calcifications in the uncinate process of the pancreas on CT scan. Her magnetic resonance cholangiopancreatogram (MRCP) revealed calcifications in the region of the uncinate process of the pancreas, the presence of a type I choledochal cyst with dilatation of the right and left hepatic ducts at their confluence suggesting an anomalous pancreaticobiliary ductal junction. She underwent choledochal cyst excision with a Roux-en-Y hepaticojejunostomy. CASE REPORT #2: A 35-year-old male with colicky abdominal pain of four months duration whose CT scan was suggestive of an atrophic pancreas with a 1 cm dilatation of the pancreatic duct and a calculus in the pancreatic duct near the ampulla. MRCP showed significant atrophy of the pancreas with an isointense filling defect seen in the pancreatic duct at its distal end near the ampulla. A diagnosis of chronic calcific pancreatitis with type I choledochal cyst was made. He underwent choledochal cyst excision with a cholecystectomy, hepaticojejunostomy (end-to-side) and side-to-side pancreaticojejunostomy. CONCLUSION Chronic calcific pancreatitis is a rare occurrence in patients with choledochal cysts and only six cases have been reported in the literature. Our two patients with choledochal cysts associated with chronic pancreatitis were treated surgically.

MEN1 935-1G>C splicing mutation in an Indian patient with multiple endocrine neoplasia type 1.

AbstractBackground and Objective: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized mainly by multiple tumors involving parathyroid, pancreatic, and pituitary glands. To date, there have been no genetic studies reported on MEN1 in the Indian population. In order to begin to establish molecular diagnosis to improve the management of MEN1 in India, we performed a molecular analysis of the MEN1 gene in a patient of Indian origin. Methods: Molecular analysis of the MEN1 gene was performed to identify mutations in an Indian patient previously diagnosed with sporadic MEN1. All the 10 exons of the MEN1 gene were amplified using the polymerase chain reaction and screened by direct DNA sequencing. Results: The DNA sequencing results revealed the presence of an intronic, heterozygous, splicing mutation 935-1G>C in intron 5 of the MEN1 gene. Conclusion: This study provides the first data on genetic analysis of MEN1 in Indian patients.

Incidence and fate of antral varices.

OBJECTIVE To study the incidence of antral varices (AV) and their fate in patients with portal hypertension so as to formulate a management policy. DESIGN Prospective cohort study. SETTING Single surgical unit specializing in portal hypertension management in a tertiary level centre. PARTICIPANTS Three hundred and seventy-one patients [cirrhosis 170, non-cirrhotic portal fibrosis (NCPF) 53, extrahepatic portal venous obstruction (EHPVO) 148] with history of bleeding from oesophageal varices were inducted in the chronic sclerotherapy programme. INTERVENTIONS Protocol-based endoscopic sclerotherapy and management of bleeding for oesophageal varices. OUTCOME MEASURES Development or disappearance of AV, bleeding from AV. RESULTS No patient had AV on index endoscopy. Thirteen (3.5%) patients developed AV, in cirrhosis 2.9%, EHPVO 4.1%, NCPF 3.8% (P = 0.86). AV developed after a mean of 15 months. Oesophageal varices took a longer number of sessions to obliterate in patients with AV (11.1 vs 5.98 sessions, P<0.0001). Only one patient bled, having coexistent oesophageal varices and gastropathy. AV disappeared spontaneously in seven patients, recurring in only one. Of seven persisting AV, none have bled over a mean follow-up of 30 months (SD 23.2). CONCLUSIONS AV are seen in a small proportion of patients, and are distributed equally amongst the aetiologies of portal hypertension. They rarely bleed and may be ignored during sclerotherapy of oesophageal varices.

Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma.

Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.

Liver transplantation—Economics in the less developed world

Though liver transplant has been a well-established treatment for liver diseases in developed countries for several decades, developing countries have lagged behind in this field. However, in the last decade, there has been major progress in the field of liver transplantation in India. In particular, living-related liver transplant (LRLT) has flourished over the last five years [1]. Among developing countries, India currently performs the largest number of LRLTs and drains patients from some surrounding countries as well. In the Western world, the government bears the expenditure on transplant programs. In some countries, insurance companies have recognized that it is more economical to fund liver transplantation than to pay for the management of end-stage liver disease, and now cover for this procedure. India spends much less on healthcare than the West. Communicable diseases such as tuberculosis and malaria take precedence, and few government hospitals would spare the budget to set up an LDLT program. The vast majority of liver transplants are thus performed in the private sector [1]. A certain section of the population is able to struggle and gather the money required for liver transplant. However, the expenditure does not end with surgery; rather, a successful procedure entails lifelong immunosuppression, a costly undertaking. Expectedly, the patients and their families are keen to know the magnitude of this expenditure in advance, and whether this cost burden can be somehow reduced. Fortunately for these patients, India is blessed with a strong pharmaceutical industry that manufactures generic drugs [2]. These companies sell drugs such as tacrolimus and mycophenolate mofetil (MMF) at a fraction of the price in the developed world. A paper by Sudhindran et al. [3] from Cochin in this issue of the Journal provides valuable information on the cost of immunosuppression over the first one-year period after LDLT in Indian patients. The most interesting revelation of this study perhaps was that the cost of calcineurin inhibitor (CNI) therapy in India is extremely low. Using a generic preparation, the cost of this treatment was in the range of Indian Rupees (INR) 3,000 to INR 5,000 per month (1 USD0approximately INR 50). Interestingly, the largest component of the cost of CNI therapy was the expenditure on monitoring of drug levels. Using Abbott IMx kits, which are based on microparticle enzyme immunoassay technology, each measurement of tacrolimus drug level in their institution cost INR 3,900 which was roughly similar to the cost of onemonth supply of the CNI drug. The authors therefore suggest that it may be useful to find ways of tailoring downwards the need for monitoring of these levels in an attempt to reduce the cost of immunosuppressive treatment. However, the cost of drug monitoring that the authors have mentioned appears a bit high, since in many transplant centers in India such drug assay costs between INR 2,100 and INR 3,000 (personal communication). It must be noted that, as a protocol, the authors of this current paper accepted a lower level of CNI during the first few months after LRLT. This is similar to the practice in other transplant centers. It has been observed that CNI drug levels needed to prevent rejection are lower in the LRLT setting than following deceased donor liver transplantation (DDLT) [4]. Further, the authors of the current paper were fairly liberal in the use of antimetabolites such as MMF in support. The authors note that the blood levels produced by the generic CNI preparations used in the study were predictable S. Saigal Medanta, The Medicity, Sector 38, Gurgaon, Haryana 122 001, India

Emergency sclerotherapy for control of acute oesophageal variceal bleeding using 3% aqueous phenol: a 15-year experience.

Background:  The purpose of the present study was to compare the efficacy of emergency endoscopic variceal sclerotherapy (EVS) using 3% aqueous phenol as an initial procedure to control acute oesophageal variceal bleed.