Recurrent giant retroperitoneal liposarcoma: Review of literature and a rare case report.

INTRODUCTION AND IMPORTANCE: Retroperitoneal liposarcomas (RPLS) are usually grow large with frequent recurrences. Complete surgical excision remains the gold standard treatment for primary and even recurrent tumours. Their prognosis depends on their histological type and grade. We report a recurrent giant de-differentiated RPLS weighing 18.55 kg which was completely excised. To the best of our knowledge, this is one of the largest liposarcoma reported in the literature. CASE PRESENTATION: A 40 year old female presented with a gradually progressing large abdominal lump for 1year. She had had a similar large lump twice in the past and undergone excision of the tumour elsewhere. Firm non-tender mass felt all over abdomen with edema noted over abdominal wall and bilateral lower limbs. PET CT showed large heterogeneously enhancing mass occupying almost the entire abdominopelvic cavity. 50 × 40 × 40cm tumour was completely excised and biopsy showed grade 2 dedifferentiated liposarcoma (DDLS). She is under close follow up with no recurrence at 12months. CLINICAL DISCUSSION: DDLS have lower risk of distant metastases but have a high risk of local recurrence. The most important favourable prognostic factor in these tumours is complete resection with negative margins. Because of the ineffectiveness of current chemotherapy and the requirement of intolerably high radiation doses, surgical excision remains the most effective treatment even for the localized recurrences of RPLS. CONCLUSION: The dedifferentiated subtype should be suspected in locally aggressive RPLS. Close follow up with early detection of recurrences and prompt excision with negative margins lowers the risk of recurrences and improves survival.

Post hepatectomy liver failure - A comprehensive review of current concepts and controversies.

Post hepatectomy liver failure (PHLF) comprises of a conundrum of symptoms and signs following major hepatic resections. The pathophysiology essentially revolves around disruption of the normal hepatocyte regeneration and disturbed liver homeostasis. Prompt identification of the pre-operative predictors of PHLF in the form of biochemical parameters and imaging features are of paramount importance for any hepatic surgeon and forms the cornerstone of its management. Treatment revolves around a goal-directed resuscitation of the systemic organ failure. Auxiliary support systems such as liver dialysis devices and stem cell therapy are still under investigational trials for treatment of the same. Orthotopic liver transplantation (OLT) is the last resort in most cases not responding to other measures.

Effect of informed consent on patients undergoing gastrointestinal surgery and living donor liver transplantation and on their relatives in a developing country.

BACKGROUND: Informed consent is a systematic process for obtaining permission before conducting a healthcare intervention. In a developing country, gaining informed consent is generally perceived to be a ritual only to comply with legal requirements. The present study examined this by assessing the process of informed consent in patients undergoing gastrointestinal surgery or living donor liver transplantation (LDLT) and their relatives, based on their comprehension and overall satisfaction, in India. METHODS: All patients undergoing any gastrointestinal surgery or LDLT procedure between August 2015 and July 2016 and their relatives were included, and were administered a structured questionnaire 5 days after the procedure. RESULTS: The majority of patients (94·2 per cent) could recall the nature of their disease, the surgery performed (81·6 per cent) and anticipated complications (55·6 per cent). Among their relatives, these proportions were 97·8, 87·3 and 58·5 per cent respectively. Recall was associated with age, occupation and education among both patients and relatives. Patients undergoing LDLT, their donors and their relatives had better recall than those who had other gastrointestinal procedures (P < 0·001). Many patients found the process of informed consent useful and reassuring. CONCLUSION: The details and risks of an operation were understood by most of the patients, especially those undergoing liver transplantation. Patients from developing countries can generally understand 'informed consent', and value it.

Minimizing Hepatic Artery Thrombosis and Establishing Safety of Grafts With Dual Arteries in Living Donor Liver Transplantation.

BACKGROUND: Hepatic artery thrombosis (HAT) is a dreaded complication following living donor liver transplantation (LDLT) and can lead to graft failure and biliary complications. We evaluated the results of our arterial anastomotic technique and outcomes in grafts with dual arterial supply. PATIENTS AND METHODS: Between July 2010 and June 2015, 225 patients underwent LDLT. The hepatic artery anastomosis was done using our "W technique". In grafts with a dual arterial supply, two anastomoses were performed unless there was significant pulsatile back-bleeding in the smaller artery after the larger anastomosis. RESULTS: The mean age of the recipients was 43±15.2 years (6 months to 66 years). There were 184 right liver, 30 left liver, 10 left lateral segment, and 1 dual lobe (right liver and left lateral segment) grafts. Twenty-three (10.2%) patients had 2 graft arteries, 10 of which required 2 separate anastomoses, and an interposition saphenous vein conduit was used in one. HAT occurred in 3 (1.3%) patients. The median intensive care unit and postoperative hospital stays were 5 and 14 days, respectively. Post-transplant operative mortality was 12.4%. There was no difference in mortality (8.7% vs 12.4%, P = >.99) and biliary complications (11.9% vs 21.7%, P = .19) between recipients of grafts with single or dual graft arteries, respectively. CONCLUSIONS: A careful surgical "W technique" and intraoperative confirmation of a good arterial flow helps in reducing the incidence of early HAT. The presence of two arteries in the graft was not associated with increased incidence of HAT, mortality, or biliary complications.

Endoscopic management of post-liver transplant billiary complications: A prospective study from tertiary centre in India.

BACKGROUND: Liver transplantation has become common in India over the last decade and biliary strictures after the procedure cause a significant morbidity. Endoscopic retrograde cholangiopancreatography (ERCP) is a safe and effective treatment modality for post-transplant biliary strictures so we decided to evaluate prospectively the outcomes of endoscopic treatment in post-living donor liver transplantation (LDLT) biliary strictures. METHODS: We studied ten consecutive patients who had developed biliary strictures (out of 312 who had undergone liver transplantation between June 2009 and June 2013) and had been referred to the Department of Gastroenterology for management. All patients underwent liver function tests, ultrasound of the abdomen, magnetic resonance cholangiography and liver biopsy, if this was indicated. RESULTS: Of these 312 patients who underwent liver transplantation, 305 had living donors (LDLT) and 7 deceased donors (DDLT). Ten patients in the LDLT group (3.3%) developed biliary strictures. There were seven males and three females who had median age of 52 years (range 4-60 years). The biliary anastomosis was duct-to-duct in all patients with one patient having an additional duct-to-jejunum anastomosis. The mode of presentation was cholangitis in four patients (40%), asymptomatic elevation of liver enzymes in four (40%) and jaundice in two patients (20%). The median time from transplantation to the detection of the stricture was 12 months (2-42.5 months). ERCP was attempted as initial therapy in all patients: seven were managed entirely by endoscopic therapy, and three required a combined percutaneous and endoscopic approach. Cholangiography demonstrated anastomotic stricture in all patients. A total of 32 sessions of ERCP were done with mean of 3.2 (2-5) endoscopic sessions and 3.4 (1-6) stents required to resolve the stricture. The median time from the first intervention to stricture resolution was 4 months (range 2-12 months). In four patients, the stents were removed after one session and in two patients each after two, three and four sessions. In six patients more than one stent was placed and all of them required dilatation of stricture. Seven patients completed treatment and are off stents at a median follow up period of 9.5 months (7-11 months). Two patients developed recurrence of their stricture after 7.5 months. Both had long strictures and required a combined endoscopic and percutaneous approach. There was one mortality due to sepsis secondary to cholangitis. CONCLUSIONS: Post-LDLT biliary strictures can be successfully treated with ERCP, and most patients remain well on follow up (median 9.5 months). A combined endoscopic and percutaneous approach is useful when ERCP alone fails.

A Single Centre Analysis of Clinical Characteristics and Treatment of Endocrine Pancreatic Tumours.

Background. Endocrine Pancreatic Tumours (PENs) are rare and can be nonfunctioning or functioning. They carry a good prognosis overall though high grade lesions show a relatively shorter survival. The aim of the current study is to describe a single centre analysis of the clinical characteristics and surgical treatment of PENs. Patients and Methods. This is a cohort analysis of 40 patients of PENs who underwent surgery at Sir Ganga Ram Hospital, New Delhi, India, from 1995 to 2013. Patient particulars, clinical features, surgical interventions, postoperative outcome, and followup were done and reviewed. The study group was divided based on grade (G1, G2, and G3) and functionality (nonfunctioning versus functioning) for comparison. Results. PENs comprised 6.3% of all pancreatic neoplasms (40 of 634). Twenty-eight patients (70%) had nonfunctioning tumours. Eighteen PENs (45%) were carcinomas (G3), all of which were nonfunctioning. 14 (78%) of these were located in the pancreatic head and uncinate process (P = 0.09). The high grade (G3) lesions were significantly larger in size than the lower grade (G1 + G2) tumours (7.0 ± 3.5 cms versus 3.1 ± 1.6 cms, P = 0.007). Pancreatoduodenectomy was performed in 18 (45%), distal pancreatectomy in 10 (25%), and local resection in 8 (20%) and nonresective procedures were performed in 4 patients (10%). Fourteen patients (35%) had postoperative complications. All G3 grade tumours which were resected had positive lymph nodes (100%) and 10 had angioinvasion (71%). Eight neoplasms (20%) were cystic, all being grade G3 carcinomas, while the rest were solid. The overall disease related mortality attributable to PEN was 14.3% (4 of 28) and for malignant PENs was 33.3% (4 of 12) after a mean follow-up period of 49.6 months (range: 2-137 months). Conclusion. Majority of PENs are nonfunctioning. They are more likely malignant if they are nonfunctioning and large in size, show cystic appearance, and are situated in the pancreatic head. Early surgery leads to good long term survival with acceptable postoperative morbidity.

Management of bleeding in extrahepatic portal venous obstruction.

Extrahepatic portal venous obstruction, although rare in the western world, is a common cause of major and life threatening upper gastrointestinal bleeding among the poor in developing countries. Patients have large spleens and stunted growth. The diagnosis is easily confirmed by Doppler ultrasonography. Endoscopy sclerotherapy is the best option for the control of acute variceal bleeding. For secondary prophylaxis of bleeding, the choice lies between repeated sclerotherapy and a portosystemic shunt. We believe that due consideration should be given to performing a splenectomy and a lienorenal shunt. Performed by experienced surgeons, it carries a low operative mortality of 1%, a rebleeding rate of about 10%, removes the large spleen, reverses hypersplenism, and is not followed by portosystemic encephalopathy. Most importantly, it is a onetime procedure particularly suited to those who have little access to blood transfusion and sophisticated medical facilities.

Abdominal packing for surgically uncontrollable haemorrhage.

BACKGROUND: Using abdominal packs is often a life-saving technique for uncontrollable bleeding during operations. It prevents worsening of the hypothermia, coagulopathy and acidosis which usually accompanies massive bleeding till they may be corrected and the packs removed later. However, packing may be associated with a mortality of 56 to 82% due to continued bleeding, intra-abdominal abscesses and the compartment syndrome. We follow a policy of early abdominal packing (considering it after a 6 unit intraoperative blood loss) before the situation becomes irreversible. PATIENTS AND METHODS: Between January 1997 and September 2008, abdominal packing for uncontrollable bleed was done in 49 patients (M:F 34:15, mean age 43 years). The risk factors for mortality were analyzed. The reasons for uncontrollable bleed were: liver trauma (8), liver tumours (3), following liver transplantation (4), pancreatic necrosectomy (17) and miscellaneous causes (17). RESULTS: There were 16 postoperative deaths (32.7%). On univariate analysis, hypovolaemic shock, a low urine output, raised INR, blood requirement of more than 6 units, hypothermia <34 degrees C, metabolic acidosis and sepsis were associated with an increased mortality. However, on multivariate logistic regression only hypothermia was significantly associated with mortality. CONCLUSION: A fair survival rate can be achieved by early and judicious use of abdominal packing especially before hypothermia supervenes.

Right-sided diaphragmatic hernia in an adult after living donor liver transplant: a rare cause of post-transplant recurrent abdominal pain.

Right-sided diaphragmatic hernia (DH) after living donor liver transplant in adult recipients has not been described previously. There have been a few reports of right-side DH in paediatric living donor liver transplant recipients and following right lobe donor hepatectomy. We herein describe the first instance of right-sided DH in a 44-year-old man who underwent left lobe live donor liver transplant. Twenty-eight months later, he presented with recurrent right upper abdominal pain. Radiographic workup revealed a right-sided DH. He underwent laparotomy and primary repair of the diaphragmatic defect. Seven months after the repair, the patient remains well and free from symptoms, with a normal chest X-ray. Right-sided DH is rare but may occur after living donor liver transplant. Successful outcome can be achieved by an early diagnosis and prompt management.

Surgery for obscure lower gastrointestinal bleeding in India.

Western patients with obscure lower gastrointestinal hemorrhage (OLGIH) are usually 60 years or older, bleed from colonic diverticulosis or angiodysplasia, and need localizing investigations. In India, patients are younger, the causes of bleeding different, and health resources scarce. We followed a policy of early surgical exploration operation and excision of the bleeding source or, if this was not identified, did a right hemicolectomy. The outcome of this strategy was evaluated. Between 1996 and 2003, we managed 62 patients with OLGIH. Localizing investigations such as enteroclysis, radioisotope scanning, angiography, and peroperative enteroscopy were infrequently performed. Fifty patients underwent surgery, emergency (35 pts) or elective (15 pts), and comprised the study group. At operation the lesion was localized in 33 (66%) patients (jejunum in 9 and terminal ileum or cecum in 24) and was resected. In 17 patients no lesion was found and they had a right hemicolectomy. The 30-day mortality was six patients (12%) and included persistent bleeding (three), liver failure (one), and chest infection (one). Five (10%) patients rebled after operation at a mean follow-up of 31 months. Cirrhosis (P=0.003) as a comorbid illness was the only significant factor for rebleed in the right hemicolectomy group. Advanced age (>60 years; P=0.08) might be another risk factor in a larger study. In conclusion, patients with obscure OLGIH in India should have an early operation. If a lesion is not detected, a right hemicolectomy may be done. In this group those with cirrhosis have a higher chance of rebleed, as well as, perhaps, elderly patients.

Low anterior resection with total mesorectal excision for rectal cancer: functional assessment and factors affecting outcome.

OBJECTIVE: Many patients experience disordered defaecation after low anterior resection of the rectum (LARR). We analysed the anorectal function of these patients to determine which factors might contribute to this problem. PATIENTS AND METHODS: Between November 2002 and January 2004, 18 consecutive patients (11 males and 7 females) who underwent LARR with total mesorectal excision (TME) for rectal cancer were assessed by anorectal manometry, balloon proctometry and the Wexner continence questionnaire before operation and at 6 months and 1 year following stoma closure. Sixteen volunteers (11 males and 5 females) were evaluated for comparison. Stepwise logistic regression was performed for variables that were highly significant at univariate analysis. RESULTS: The mean daily preoperative stool frequency was 2, mean basal pressure (MBP) 43.12 cm H(2)O, maximum threshold volume (MTV) 181.8 ml, length of high-pressure zone (HPZ) 3.11 cm and the rectoanal inhibitory reflex (RAIR) was present in all the patients. Twelve months after stoma closure, the stool frequency was 3.3, MBP 37.7 cm H(2)O, MTV 146.3 ml, length of HPZ 2.88 cm and Wexner score 4.37. Comparing patients having a good anorectal function (Wexner score > or = 5) with those having an unsatisfactory function, we found that, on multivariate analysis, the factors that independently contributed to a poor outcome at 12 months after operation were the absence of RAIR as well as an MTV and HPZ below the fifth percentile of normal individuals. CONCLUSIONS: Many patients undergoing LARR with TME for rectal cancer experience an anterior resection syndrome that persists for at least 1 year. Those with no RAIR and subnormal MTV and HPZ lengths can be predicted to have an unsatisfactory outcome.

Evaluation of Pringle maneuver during liver resection in a rat model of surgical obstructive jaundice.

Temporary portal triad clamping (Pringle maneuver) during liver resection reduces intraoperative blood loss. A normal liver can safely tolerate normothermic ischemia for up to 60 min. However, its safety in patients with surgical obstructive jaundice (SOJ) is not known. Therefore, we investigated the effect of hepatic ischemia in an experimental rat model of SOJ created by ligating the bile duct. Four groups of rats were created: Group I (sham operation, 10 days later, liver resection); Group II (sham operation, 10 days later, liver resection with 5 min of hepatic ischemia); Group III (bile duct ligation, 10 days later, liver resection); and Group IV (bile duct ligation, 10 days later, liver resection with 5 min of hepatic ischemia). The ischemic injury was assessed by the survival of rats, liver tissue malondialdehyde and total glutathione (markers of free radical injury), serum alanine aminotransferase, aspartate aminotransferase, and liver histology. The results showed decreased survival (47.6% vs. 90% [p = .046]), increased liver tissue malondialdehyde (161 +/- 35 vs. 129 +/- 33 microg/gm liver tissue [p = .05]), and decreased liver tissue total glutathione (565 +/- 169 vs. 1075 +/- 276 nmol/gm liver tissue [p = .05]) in rats with SOJ subjected to hepatic ischemia when compared to nonjaundiced rats. The changes in serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), and alkaline phosphatase showed an increasing trend in the SOJ group but were not statistically significant. Ischemic changes in liver histology were seen more often in the SOJ group but were not statistically significant. These data suggest that temporary portal triad clamping in an experimental model of SOJ is detrimental to the outcome of liver resection.

Do the progesterone receptors have a role to play in gallbladder cancer?

BACKGROUND: Gallstone disease as well as gallbladder cancer are more common in women and female sex hormones may be involved in their etiology. AIM AND METHODS: To determine whether female sex hormones have a role in the pathogenesis, of gallbladder carcinoma and in its prognosis, we estimated, by enzyme immunoassay, the estrogen and progesterone receptors (ER and PgR) in the gallbladders of 21 patients with gallbladder cancer, 19 patients with cholelithiasis, and 6 patients who underwent incidental removal of essentially normal gallbladder as a component of wider resection. RESULTS: ER were present in the gallbladder mucosa in all the three groups in proportions which were not significantly different (9/21 in carcinoma, 4/19 in gallstones, and 1/6 normal), whereas the expression of PgR was greater in carcinomas (13/18), less in cholelithiasis (4/12), and absent in normal gallbladders. PgR expression was higher in tumors of lower stage (7/7) and lower in advanced disease stage IV tumors (6/11). PgR expression was associated with better disease stage (p=0.05) and significantly longer overall survival (median survival of 301 d vs 54 d) as well as better survival within the same stage (269 d vs 54 d for stage IV disease, p=0.011). Cox's regression analysis showed that PgR was an independent risk factor (R=0.2283, p=0.0035). CONCLUSIONS: Our findings suggest that the female sex hormones may have a role in the pathogenesis of gallbladder cancer and that PgR expression has a prognostic significance. We believe that when this relationship is reaffirmed by larger studies, gallbladder cancer may be treated with appropriate sex hormonal manipulation.

Complicated colonic diverticulosis: surgical perspective from an Indian Centre.

INTRODUCTION: Colonic diverticulosis was previously uncommon in India but its incidence seems to have increased recently. Patients with the disease in developing countries are also underdiagnosed and are therefore more likely to present with complications needing operation. However there is a paucity of surgical data on the condition. METHOD: Between August 1996 and February 2005 we operated on 32 patients (28 males, 4 females mean age 60 years) with colonic diverticulosis and analysed their characteristics from a prospective database. We here with describe our experience. RESULTS: Operations for diverticular disease constituted 3% of all the colorectal operations we performed. The diverticula were in the sigmoid colon in 28 (88%) and also in the descending colon in 4 (12%). Twenty-four patients were symptomatic. Twenty-two patients were diagnosed before surgery, 8 at operation and the rest from resected specimens. Emergency operations were performed in 23 and elective procedures in 9 patients. Ten patients were operated on for perforation and abscess, 8 for obstruction, 8 for colovesical fistula, 3 for peritonitis and 3 for haemorrhage. Emergency procedures were performed in 2 stages (resection plus a proximal diversion) in 20; unless done for bleeding in a stable patient where a primary anastomosis was done. One patient who had had an emergency procedure died of sepsis and ketoacidosis in the post-operative period. The 8 patients with colovesical fistulae were all males, had only sigmoid involvement and had had symptoms for a longer duration than the 24 without fistulae. CONCLUSIONS: Although operations for colonic diverticulosis still form a small proportion of the total number of colorectal operations, the diagnosis is often delayed till complications ensue and thus patients usually require emergency procedures. Males with long standing symptoms and sigmoid diverticula may develop colovesical fistulae.