Pinealoblastoma in a patient with familial adenomatous polyposis: variant of Turcot syndrome type 2? Report of a case and review of the literature.

We report a case of a 23-year-old Turcot female patient who was first diagnosed as having a pinealoblastoma. Thyroid papillary carcinoma was diagnosed a few months later, and multiple colonic polyps were detected three years after that. A genetic workup revealed an APC gene mutation in her family. Long-term survival (i.e., >5 years) of pinealoblastoma is considered to be 20 percent. A review of 25 documented Turcot cases determined that the average age at death is 20.3 years, raising the difficult question of whether she should undergo restorative proctocolectomy. Restorative proctocolectomy may itself cause major morbidity but is currently the only way to prevent colon cancer.

Agenesis of the gallbladder found at laparoscopy for cholecystectomy: an unpleasant surprise.

Gallbladder agenesis is a rare condition that results from the failure of the cystic bud to develop in the 4th wk of intrauterine life. Agenesis is usually discovered at laparotomy for cholecystectomy since ultrasound examination of a patient with suggestive symptoms not visualizing the gallbladder is compatible with chronic cholecystitis (shrunken gallbladder). The surgeon must prove agenesis by thoroughly examining the most common sites for ectopic gallbladders and by performing intraoperative cholangiograms. For unexplained reasons, most of the patients become asymptomatic after the operation. We present a new case of adult agenesis of the gallbladder in a patient who was taken to laparoscopic cholecystectomy for presumptive cholelithiasis.

Laparoscopic vs conventional autopsy. A promising perspective.

OBJECTIVE: To analyze the sensitivity and specificity of laparoscopic autopsy when compared with the ulterior performance of conventional postmortem examination. DESIGN: Consecutive sampling, case-series study. SETTING: A general community referral medical center and the local institute of forensic medicine. PARTICIPANTS: A consecutive sample of 25 fresh cadavers (< 24 hours old) of victims of vehicle accidents, gunshot wounds, and in-hospital deaths. INTERVENTIONS: After insufflation with carbon dioxide, laparoscopy of the abdominal cavity and inspection of the retroperitoneal area was accomplished. Following, a conventional postmortem examination was performed and the findings of both procedures were recorded, compared, and analyzed. MAIN OUTCOME MEASURES: Prior to the beginning of the study, it was hypothesized that laparoscopic autopsy would reach an overall sensitivity and specificity of at least 85% and enable accurate inspection of the abdominal cavity and retroperitoneum in the search for the trauma-related cause of death in trauma victims or the cause of death for in-hospital patients. RESULTS: There was a 100% correlation of both procedures in all accidental cases. The sensitivity of laparoscopic autopsy was 93% for intra-abdominal lesions, with an overall sensitivity of 91%. For the retroperitoneal area, the sensitivity was 58%. The sensitivity for the retroperitoneum dropped, owing to an intrarenal tumor and an extraperitoneal rectal tear in the small sample of inhospital deaths. The specificity of laparoscopic autopsy reached 94%. CONCLUSIONS: Laparoscopic autopsy is accurate and easy to perform. It is highly sensitive for intra-abdominal abnormalities, especially in trauma victims. Laparoscopic autopsy is minimally invasive and not disfiguring, rendering it easier to accept among mourning families. It should be strongly considered when consent for a conventional autopsy is lacking.

The role of percutaneous transhepatic cholecystostomy in the management of acute cholecystitis in high-risk patients.

A method recently developed that may be an appropriate solution for high-risk patients with acute cholecystitis is percutaneous sonography-guided cholecystostomy. We report our experience in 10 high-risk elderly patients with clinical and sonographic diagnosis of acute cholecystitis. Immediate regression and resolution of septic symptoms was achieved in all cases. One patient was operated on as soon as his clinical condition stabilized, with uneventful postoperative recovery. The other nine were considered inoperable; of these, two were readmitted within a few months with recurrence of symptoms who underwent surgery, with a long and complicated postoperative course. The only complication we observed was temporary septicemia in one patient immediately after completion of the procedure. In view of these findings, we consider percutaneous transhepatic cholecystostomy an effective and safe method of treatment for acute cholecystitis in critically ill patients. However, this procedure should be regarded as a preliminary measure only, to render the patient more suitable for a formal cholecystectomy. We report our results and discuss technical and principal matters concerning percutaneous transhepatic cholecystostomy in the light of the current literature.

Multifactorial analysis of prognostic factors in major abdominal surgery in patients over 80.

OBJECTIVE: The principle aim of the study was to identify by multifactorial analysis the combination of factors predictive of mortality after major laparotomy in patients over 80 years old. DESIGN: A computer held data base established by the authors in 1978 was used to register all relevant information of all major laparotomies performed in patients above 80. RESULTS: Unifactorial analysis of mortality disclosed the following factors associated with increased postoperative mortality: 1. Age 85 or more; 2. ASA fitness status 4 or 4; 3. Emergency procedure; 4. Presence of advanced malignant disease; 5. Diagnostic group 5 or 9 (mesenteric occlusion, pancreato biliary malignancy). Multifactorial analysis disclosed low mortality (2.5%) in cases with no gravity factors and very high mortality (above 70%) in those with 3 or more gravity factors.

Amino acid alcohols: growth inhibition and induction of differentiated features in melanoma cells.

The effects of a series of D- and L-amino acid alcohols on the proliferation and phenotypic expression of B16 mouse melanoma cells were evaluated. B16 melanoma cells were incubated for different time intervals in the presence of D- or L-phenylalaninol (PHE), D- or L-alaninol (AL), D- or L-leucinol (LE), L-histidinol (HIS), L-tyrosinol (TYR) and L-methioninol (MET). All agents, including the D or L configuration, induced an anti-proliferative effect, although of considerably different magnitude. D-PHE was the most active growth inhibitor. The growth inhibitory effects were accompanied by phenotypic alterations, which included morphological changes and enhancement in the activities of NADPH cytochrome c reductase and tau-glutamyl transpeptidase. These phenotypic alterations correlated with the growth inhibitory effects of the different agents and seem to reflect a higher differentiated state.

State of the art in the diagnosis and management of acute cholecystitis.

The analysis of 664 cases operated upon for acute cholecystitis as well as a review of the literature indicate the dramatic increase in the age of patients presenting with this complication. Other obvious changes: increasing rate of diabetes mellitus and acalculous cholecystitis. The development of preoperative and operative sonography has contributed greatly to the safety of surgery for acute cholecystitis. The majority of patients classified as having low risks should, at the present state of our knowledge, undergo early and definitive surgery. This approach has shown to be the most beneficial from both the medical and economic standpoint. High risk patients as well as patients refusing surgery are optimally treated by sonar-guided percutaneous transhepatic cholecystostomy. Among the recent changes observed over the last 5 years, a marked decrease in septic complications as well as in the length of hospital stay was noted. Both changes improve the cost-benefit ratio of early surgery in this condition.

The risk of cholecystectomy for acute cholecystitis in diabetic patients.

In order to evaluate the risk of acute cholecystitis in diabetic patients, we analyzed 2,700 consecutive cholecystectomies, 566 of which were performed in the presence of acute cholecystitis. Of these patients 123 had diabetes mellitus (DM) and 433 had no diabetes (ND). The aim of this study was to establish the comparative risks in the two groups. We found that diabetics are more likely to be operated on in the acute stage of their disease (22% vs. 12%). The DM group had a higher rate of septic bile, gangrenous changes and perforations of the gallbladder wall. The morbidity rate was higher in the DM group (21% vs. 9%), and mortality was slightly higher in the DM group. The degree of additional operative risk does not in our view justify recommending cholecystectomy in diabetic patients with asymptomatic gallstones. Early surgery however, is highly recommended in diabetics with symptomatic gallstones and acute cholecystitis.

Multifactorial analysis of septic bile and septic complications in biliary surgery.

A unifactorial analysis for possible risk factors was applied to 2,700 consecutive operations for benign disease of the biliary tract. A series of high risk factors in relation to positive bacteriology and septic complication could be identified. These risk factors were patients who were elderly (greater than 70 years; p less than 0.001), those who were diabetic, those who had a serum bilirubin greater than 1.1 mg% (p less than 0.001), those who had acute cholecystitis (p less than 0.001), and those in whom choledochal stones were found (p less than 0.001). Using a multivariate analysis, we concluded that in patients with no risk factors (56.9%) the incidence of a positive bacteriology was low (10.9%) and they should receive no antibiotic prophylaxis. Patients with one risk factor (24%), had a 36% incidence of positive bacteriology and minimal pre-operative prophylaxis is recommended. Patients with two or more risk factors (19.1%) had a 77.6% incidence of positive bacteriology and full peri-operative prophylaxis is recommended, starting pre-operatively and continuing for 3 to 5 days postoperatively. The aim of this study was to identify patients at risk for septic complication in biliary surgery and to create new guidelines for the antibiotic treatment of selected groups.

Sensitivity to new generation of antibiotics in biliary surgery.

The treatment of septic complications of cholecystectomy patients requires special attention. In a prospective study of 1009 consecutive cholecystectomy patients (including all acute and elective patients, excluding cases of malignancy), we routinely took cultures from the cystic duct and the gallbladder, and checked the strain for sensitivity. Positive cultures were found in 31.4% of the series, the most frequent bacteria being E. coli (50.3%). The aminoglycosides and the second and third generations of cephaloridins were found more specific for the strains that were cultured. While the new cephaloridins are the first choice for prophylaxis, the combination of aminoglycosides with ampicillin is to be preferred from the medical and cost effective standpoint while the aim is therapeutic.

Choledochoduodenostomy an important surgical tool in the management of common bile duct stones. A review of 126 cases.

Of 2,000 patients who underwent cholecystectomy between 1965-1984, 457 had their common bile ducts explored and of these 126 had choledochoduodenostomies. There were 70 women and 56 men and a third were over 70 years old. Forty-three also had acute cholecystitis or pancreatitis, and 73 had an increased serum concentration of bilirubin. Of the 126, 103 had stones in the common duct, and 16 had benign strictures of the sphincter of Oddi. Morbidity included wound infections in 18 (14%) and bile discharge from the drain for over 14 days in four (3%). Five patients died (4%), and they were all over 70 years old. Ninety-seven of 103 patients followed-up over 1-19 years were symptom free (94%). Two patients required re-operation for a narrow anastomosis. Three patients developed a "sump" syndrome; two were treated with antibiotics, and the third responded to endoscopic sphincterotomy. Choledochoduodenostomies should be considered during operation for benign biliary disease, when stones or a stricture in the common bile duct are anticipated.

Results of the pelvic-pouch procedure in patients with Crohn's disease.

The pelvic-pouch procedure has become a standard operation for selected patients with ulcerative colitis, but is contraindicated in patients with Crohn's disease at our institution. However, the distinction between ulcerative colitis and Crohn's colitis can sometimes be difficult, if not impossible. Between January 1982, and March 1989, 272 patients with ulcerative colitis underwent pelvic-pouch procedures at our institution. Nine (3.5 percent) of these patients eventually were found to have Crohn's disease. The records of these patients were examined to assess their clinical outcome and complication rate. There were five females and four males with a mean age of 28.8 years. In five patients (Group I) the diagnosis of Crohn's disease was made postoperatively on histologic examination of the rectum. The ileostomy was closed in all patients. Two developed complications necessitating excision of the pouch. Three patients are well. In the other four cases (Group II) the mean time to diagnosis was 2.5 years after the pouch procedure. Three patients developed pouch-vaginal fistula, and one multiple anal fissures and stenosis. Two required excision of the pouch whereas two have a functioning pouch but with a persistent pouch-vaginal fistula (n = 1) or anal fissures (n = 1). Overall, four patients have had their pouches removed, and five patients have functioning pouches: three with no complications and two with persistent perianal disease. Thus, we would conclude that the pelvic-pouch procedure should not knowingly be performed in patients with Crohn's disease because of the high associated complication rate.

Hemosuccus pancreaticus and intraperitoneal bleeding secondary to spontaneous rupture of the splenic artery.

Hemosuccus pancreaticus is a rare condition where bleeding occurs through the pancreatic duct, usually due to a pseudoaneurysm of the splenic artery. We describe a patient with spontaneous rupture of the splenic artery that caused hemosuccus pancreaticus and later massive hemoperitoneum. The pathological process had lasted at least 10 days before the diagnosis was established. The patient underwent an emergency operation during which splenectomy and distal pancreatectomy were successfully performed.

Changing trends in surgery for acute cholecystitis.

Surgery for acute cholecystitis has become the most frequent abdominal surgery in most hospitals, yet aspects of its management remain controversial. The aim of this study was to focus on the recent trends by demonstrating the principal differences between 2 series of patients operated on during 10-year intervals. Two hundred fifty-six consecutive operations for acute cholecystitis were performed from 1970 to 1977 (group O) and were compared to 260 cases operated from 1980 to 1987 (group N). Thirty-six variables were recorded in each case. All data obtained were computer recorded and analyzed. Several trends were observed in group N: 1. The population was significantly older with a higher proportion of males and diabetics. 2. There was a marked increase in common bile duct stones, acalculous cholecystitis, and gangrenous changes in the gallbladder. 3. There was a significant increase in patients operated on within 48 hours of admission. 4. There was a significant increase in the number of patients without previous history of biliary symptoms. 5. There was a significant decrease in the rate of wound infections and no statistically significant differences in mortality (N: 3.0%, O: 2.7%). 6. A decrease of the average postoperative hospital stay of 3.2 days was observed. In spite of the older and sicker population in group N, no significant increase in mortality occurred and the postoperative hospital stay was reduced.

Stapler stricturoplasty for Crohn's disease.

The operation of stricturoplasty for Crohn's disease is now widely accepted as a procedure that has enabled the preservation of the small intestine that would otherwise have been resected. This is particularly important when there are multiple and scattered strictures, in which short intestinal syndrome can be avoided. We have used automatic staplers for this technique with excellent results for 30 strictures in nine patients.

Small bowel obstruction: a review of 264 cases and suggestions for management.

Two hundred and sixty-four cases of acute small bowel obstruction were retrospectively reviewed for the purpose of defining factors which could point to the presence of strangulated bowel. History, physical signs and investigations, including body temperature, X-rays, white blood count, and serum amylase, were not significantly different in the simple and strangulated groups. Although an elevated urinary white blood count and a palpable mass were more common in the strangulated group, they were not sufficiently reliable for early diagnosis of strangulation. In reviewing the literature, it is clear that all hernias with obstruction must undergo emergency surgery. Cases with intra-abdominal complete intestinal obstruction should also undergo emergency surgery. A more conservative attitude can only be taken when there is incomplete obstruction.

Technique of insertion of pelvic Vicryl mesh sling to avoid postradiation enteritis.

The increasing use of pelvic radiotherapy has necessitated the study of ways to avoid radiation enteropathy. The authors have been using a pelvic sling to keep the small intestine out of the pelvis and because of the difficulties encountered, believe the method that has evolved is worthy of description.

Multifactorial analysis of prognostic factors in emergency abdominal surgery in patients above 80 years. Analysis of 154 consecutive cases.

Patients above 80 have been the most rapidly growing group in the surgical department. The number of major laparotomies in this segment of the population has grown by 15% in the last two decades and is expected to grow by another 100% by the end of the century. The principal aim of the study was to identify by multifactorial analysis the combination of factors predictive of mortality in abdominal emergencies in this age group. A computer-held data base established by the Authors in 1978 was used to register all relevant information in 1327 major laparotomies performed in patients above 70. Of the base of 1327 patients, 219 (16.5%) were above 80. Of this group, 154 cases (70.3%) underwent emergency procedures and are the focus of this study. The principal conclusions of the Authors are as follows: A. The main differences between the two age groups 70-79 (Group A) and above 80 (Group B) are as follows: 1. Emergency laparotomies are much more frequent in Group B (70% versus 33%). 2. The overall mortality is over two and a half times as high (21.4% versus 8%). 3. The diagnostic profile and the mix of cases is different with an increased number of intestinal obstructions and malignancies in Group B. B. Unifactorial analysis of postoperative mortality disclosed the following factors associated with increased mortality: 1. Vital System Category III (Table 6-A). 2. Presence of generalized peritonitis or gangrenous bowel. 3. Presence of widespread malignant disease. 4. Belonging to the following diagnostic groups: carcinoma of pancreas, mesenteric thrombosis. 5. Age 85 or above.(ABSTRACT TRUNCATED AT 250 WORDS)