Achalasia presenting as an acute airway obstruction is a rarely reported phenomenon. The pathophysiology of this dramatic complication of achalasia remains an enigma. Our patient with achalasia presented with an acute airway compromise necessitating emergent endotracheal intubation. We review the available literature on this interesting yet rare complication of achalasia. We also add some information to help elucidate the possible pathophysiologic mechanism of this emergency, proposing that specific upper esophageal sphincter manometry may indicate abnormality of the "belch reflex."

An adolescent male developed severe unexplained cholestatic jaundice 3 mo before diagnosis of mediastinal non-Hodgkin's lymphoma (T-cell, late thymic phenotype). There was no anatomic obstruction to bile flow, no evidence for an infectious etiology, and no neoplastic involvement of the liver or bile ducts. A paraneoplastic phenomenon is postulated because the jaundice resolved after treatment of the lymphoma. We suggest that occult lymphoma must be added to the differential diagnosis of unexplained intrahepatic cholestasis.

After successful pancreatic transplantation blood glucose can be normalized without exogenous insulin, although oral and intravenous glucose tolerance remains impaired in 10-45% of the patients. There is no significant deterioration of glucose control with time in most patients. Since most recipients of pancreatic grafts have far advanced secondary diabetic lesions and the observation time after grafting is rather short, the effects of pancreatic transplantation on these complications are difficult to interpret. However, the development of diabetic nephropathy can be prevented, skin microcirculation improves significantly, while autonomic and peripheral neuropathy and diabetic retinopathy remain stable or improve slightly in most patients. But these ameliorations may be in part due to elimination of uraemia, since in almost all patients combined pancreas/kidney transplantations were performed. It is concluded that pancreas grafting probably has to be performed much earlier in the course of diabetes, although the improvement in the quality of life is striking even in the end-stage diabetics studied so far.

A major stumbling block preventing success with pancreatic transplantation has been the handling of the exocrine secretion from the pancreatic graft. As a consequence, numerous surgical techniques have been evaluated such as duct ligation, free intraperitoneal drainage, ductal filling with polymers, exocrine diversion to the urinary tract, to the bowel and to the stomach. Also, because of problems encountered with the duodenum, segmental body-and-tail pancreatic grafts have been used for many years. However, it seems today that mainly two different techniques for pancreatic transplantation remain. In both whole pancreaticoduodenal grafts are used with a vascular supply from the coeliac axis and the portal vein. The exocrine drainage is either to the urinary tract by means of a side-to-side anastomosis between donor duodenum and recipient urinary bladder, or to the bowel by a side-to-side anastomosis between donor duodenum and recipient proximal jejunum. With both these techniques the technical complications have been much reduced and as a consequence the graft survival rates are now approaching those obtained with the transplantation of the liver, heart and kidney.

For more than 20 years pancreas transplantation has been advocated as a therapeutic modality in patients with insulin-dependent diabetes mellitus. When successful, this procedure is the only method for attaining long-term normoglycaemia in diabetic recipients. However, because of the potential morbidity and mortality, pancreas transplantation should be restricted to diabetic patients in whom the complications of the diabetic state are more serious than those of surgery and chronic immunosuppression. Currently three recipient categories have been identified in which pancreas transplantation would seem justifiable. The first includes diabetic patients with end-stage nephropathy who are already obligated to life-long immunosuppressive therapy because of the kidney replacement. In this recipient category the main benefit of receiving a pancreas transplant in addition to a kidney is that the quality of life is markedly improved. In addition, it seems that a functioning pancreas transplant prevents the recurrence of diabetic nephropathy in the simultaneously transplanted kidney. Since the success rate with combined pancreas-kidney transplantations is approaching that of renal transplantation alone, there is little controversy about performing the combined procedure in diabetic uraemic patients. However, if the main objective of pancreas transplantation, namely to prevent the late diabetic microvascular complications, were to be fulfilled this intervention would have to be performed earlier in the course of the disease. Therefore, single pancreatic transplantations have recently been conducted in diabetic patients with early signs of clinical nephropathy which, currently, is the most powerful predictor of susceptibility to detrimental diabetic complications. Preliminary findings indicate that, in this second recipient category, single pancreatic transplantation and subsequent euglycaemia may prevent the progression of diabetic neuropathy and nephropathy; with regard to diabetic retinopathy the results remain obscure.(ABSTRACT TRUNCATED AT 250 WORDS)

The continuously improving success rate in transplantation imposes an obligation to implement the treatment for the increasing number of waiting recipients. In consideration of the increasing number of waiting recipients, all suitable organs of one donor should be harvested, including both liver and segmental or whole pancreas. The supreme goal of multiple organ harvesting is to ensure that none of the organs are damaged during preparation, perfusion and removal of one of the other organs. In general, several surgical teams are involved in multiple organ harvesting. The procedure therefore has to be highly standardized. One of the most important aspects of the procedure described here is the well-defined division into individual preparatory phases, each with its specific objectives: Phase I: preparation of organs, including evaluation of their suitability and delineation of important anatomical structures. Phase II: special preparations immediately before start of perfusion. Phase III: perfusion. Phase IV: removal of organs. Phase V: additional procedures and preparation of donor's body. Phase VI: preparation of transplant outside of body. This chapter describes the procedure by which liver and whole pancreas can be harvested from one donor. The surgical technique represents a compromise between two extremes: excessive dissection before perfusion and perfusion as the first step. The procedure described herein is considered to be an effective, safe, rapid and reasonably standardized one, especially for the co-operative effort between different surgical teams.

Liver transplantation for metabolic disorders continues to be an expanding field, both in the range of indications and patient numbers. In most large series it accounts for approximately 7% of all adult transplants and 29% of all childhood transplants. As the results of medical therapy are improved so too are the results of liver transplantation, and hence more patients are being considered for the latter therapy. The benefits of this treatment are not only the prolongation of and improvement in the quality of life for the patient, it has also given insight into the basic metabolic defects of many disorders.

The justification for pancreatic or islet transplantation in diabetes mellitus is to halt progression of diabetic vascular complications. Although this goal has so far not been achieved, technical progress in transplanting the whole or segmental, vascularized pancreas has been remarkable. However, the operation is complicated and not without risk; immune rejection is furthermore a major problem, and the availability of suitable organs is limited. Transplantation of isolated pancreatic islet grafts, i.e. isolated islets or fetal pancreas, has therefore emerged as an attractive alternative. Iso-, allo- or xenografts of such preparations have been found to reverse diabetes in experimental animals. Only a minor operation is required and islets, although highly immunogenic, may be accessible to immunomodulation in vitro in order to decrease or abolish the allograft rejection and prevent cells from becoming targets for the autoimmune assault. However, problems and difficulties have emerged in this context also. Thus, islets are extremely difficult to isolate from the adult human pancreas. Such glands, be they adult or fetal, are not easily available, and clinical trials have so far remained without documented success. Considerable efforts are being made to overcome the difficulties encountered in islet transplantation. Attempts are being made to improve the techniques for preparation of clean and viable islets and to understand critical factors in the cellular interactions between the graft and the host after transplantation. Factors of importance in this context are the nutritional requirements of the graft and the vascularization process at the site of implantation. In order to provide unlimited access to cells with a high potential for insulin production and adaptive growth in the diabetic recipient, ongoing research is also directed towards methods for preparation of porcine fetal and adult islets suitable for xenotransplantation. The obvious problems involved in immune rejection of the grafted tissue are being investigated with respect both to the possibility of immunomodulation of the graft in vitro, the design of new immunosuppressive drugs, and the possibility of immuno-isolation of the insulin-producing cells with the aid of artificial membranes.

Arachidonic acid metabolites are involved in a wide spectrum of hepatobiliary physiologic functions and disease. Prostanoids alter hepatic bile flow. Prostaglandins with a C9 ketooxygen stimulate a bicarbonate-rich choleresis and those with a C9 hydroxyloxygen produce a chloride-rich choleresis. Prostaglandin F2 alpha stimulates the release of the potent choleretic glucagon and the stimulatory effect of prostaglandin F2 alpha on bile flow is inhibited by cyclooxygenase inhibitors, suggesting that prostaglandins play a role in the release of choleretic hormones as well as in their action. Prostanoids are involved in gallbladder contraction and water absorption. Prostaglandins produce gallbladder contraction in various species and cause gallbladder relaxation in other species. Prostaglandins also may be mediators of cholecystokinetic hormone action; however, cyclooxygenase inhibitors do not inhibit the effect of cholecystokinetic hormones in all species. Prostanoids alter the normal process of water absorption by gallbladder mucosa and induce net water secretion. The inflamed gallbladder secretes rather than absorbs fluid. The demonstration that prostaglandin E2 inhibits gallbladder fluid absorption has led to subsequent studies that demonstrated that the secretion of fluid into the inflamed gallbladder lumen may be mediated by prostanoids. In cholecystitis, the prostanoids may mediate the distention produced by mucosal fluid secretion and the contraction of the diseased gallbladder. The inflammatory changes produced in various experimental models of cholecystitis can be prevented by cyclooxygenase inhibitors. Cyclooxygenase inhibitors decrease gallbladder prostaglandin formation and are effective in producing relief of the symptoms of gallbladder disease. In experimental cholesterol gallstone formation, prostaglandins are involved in the production of mucin, which acts as a nidus for stone formation, and cyclooxygenase inhibitors prevent the formation of experimental cholesterol gallstones. Prostaglandins have been shown to be cytoprotective in various types of experimental hepatic injury and leukotrienes have been shown to be injurious to hepatocytes and biliary tract tissues. Specific prostanoids and lipoxygenase inhibitors may be valuable in treating patients with various acute hepatic inflammatory disease processes. Continued evaluation of the role of arachidonic acid metabolites in hepatobiliary physiology and disease may lead to important new therapeutic modalities.

In summary, local excision is a useful tool in the management of selected colorectal carcinomas. The advent of the fibreoptic colonoscope has revised the concept of local excision when dealing with carcinoma-containing polyps of the colon. The clinician now has the means of locally excising certain carcinomas which would have required laparotomy in the not so distant past. In dealing with carcinoma of the rectum, local excision is not advocated for all rectal carcinomas. In fact, when the previously discussed tumour related factors are considered, local excision should be the ultimate procedure in less than 5% of operations performed for rectal carcinomas. However, when appropriately used, local excision provides a less morbid alternative to more radical procedures without compromising patient survival rates or local recurrence rates.

Obstructed large bowel carcinoma is a disease of the aged, often with concomitant disease and also advanced malignancy. The immediate mortality rate of operation is high and long-term prognosis is poor in comparison with elective surgery. It is important before operation that the diagnosis be established by sigmoidoscopy and emergency contrast studies. Staged procedures based on considerations of safety have given way to immediate resection. For right-sided colonic obstruction immediate resection and anastomosis is now almost universal and for left-sided tumours primary resection has overtaken staged resection in the UK. An anticipated survival advantage for primary resection has not, however, been confirmed. Obstruction complicated by perforation is an absolute indication for resection. After left-sided resection, making an anastomosis is associated with higher risk of leakage than after an elective operation. In the most adverse circumstances of associated sepsis, Hartmann's operation retains its place but immediate anastomosis is the most frequent option for many. Additional manoeuvres to make this safe include peroperative antegrade colonic irrigation and subtotal colectomy, although segmental resection with anastomosis and without bowel preparation is also practised and may be safe in selected patients. When major resectional surgery is undertaken in aged patients at high risk of mortality, the rule that the operator should be fully trained in elective large bowel surgery is incontrovertible. It is at least equally important that the anaesthetist is experienced and capable of instituting, interpreting and acting upon sophisticated cardiopulmonary monitoring.

Discrepancies in morbidity and local recurrence rates in published clinical trials of pelvic XRT for colorectal cancer are caused by a variety of complex factors. It is, however, clear that XRT is able to cure early rectal carcinomas and is particularly useful in alleviating the symptoms of advanced or recurrent disease. The selection of appropriate cases for combinations of XRT and surgery can be made on the basis of digital examination under anaesthetic. One aim of these combinations is to reduce the number of patients with mobile tumours of the lower two-thirds of the rectum who require a permanent colostomy. In the absence of effective chemotherapy for metastatic disease, liver XRT may have a place in controlling the growth rate of occult liver metastases in selected patients.