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[Mayer-Rokitansky syndrome. Case report].

Uterine anomalies associated with congenital renal agenesis and skeletal abnormalities represent an uncommon pathology that often presents important diagnostic problems. A case of Mayer-Rokitansky syndrome is reported. The embryologic, clinical, pathologic and therapeutic implications of this disease are briefly discussed.

Abnormalities, Multiple↗

Local excision of rectal cancer with transanal endoscopic microsurgery (TEM).

BACKGROUND: Local excision for T1 rectal cancers with Transanal Endoscopic Microsurgery (TEM) is an accepted standard of care. However for T2/T3 rectal cancers, the high local failure indicates that this is not a valid option. MATERIALS AND METHODS: Between 1990 and 2000, 83 patients with rectal adenocarcinoma underwent complete full thickness local excision. The mean diameter of the tumor was 3.4+/-1.7 cm, 60% were located more than 5 cm from the anal verge; 43% of patients received radiation therapy (26 pre- and 10 postoperatively). RESULTS: Postoperative complications occurred in 15 patients (18%); there were no postoperative deaths. Mean follow-up was 37 months (range 18-118). The pathological stage was: Tis 9, T1 39, T2 23, T3 12. The overall local recurrence rate was 0% for Tis, 13% for T1, 17% for T2 and 50% for T3. Recurrence was managed surgically in 65% and nonsurgically in 35% because of advanced disease or poor general condition. Overall 5-year survival rates were 100%, 92%, 75% and 69% for Tis, T1, T2 and T3, respectively. CONCLUSION: Local excision with TEM is effective for early (Tis, T1) rectal cancers. Patients with T2 tumors can be treated with preoperative chemoradiation and subsequently local resection. Patients with T3 should not be treated with local excision unless they are unable to tolerate more extensive surgery.

Adult↗

Surgical treatment of adenocarcinoma of the cardia.

BACKGROUND: Adenocarcinoma of the gastric cardia presents different features from other gastric carcinomas. This study was performed to analyze the results of a 40-year experience with these lesions. METHODS: Of the 365 patients reviewed, 211 (57.8%) underwent resection. One hundred fifty patients underwent total gastrectomy with lower esophageal resection (TGER) and 46 underwent proximal gastrectomy with distal esophageal resection (PGER). More recently, 15 patients were submitted to total gastrectomy with subtotal esophagectomy (TGSE) without thoracotomy. RESULTS: The tumors were far advanced in most patients: extraparietal invasion in 77.7% of patients, lymph node involvement in 55%, and distant metastases in 11%. The postoperative mortality rate was 25.1% in patients who underwent resection: 26.7% after TGER, 17.4% after PGER, and 33.3% after TGSE (difference not significant). Cardiovascular and respiratory complications were common causes of death after both TGER and PGER. After TGSE, deaths were related exclusively to local complications, mainly as a result of cervical anastomotic leaks. The actuarial 5-year survival rate for all patients surviving resection was 16.7%. No improvement in the results of surgical therapy was observed during the past 20 years. The actuarial 5-year survival rate was significantly affected by pathologic staging: 61.0% stage I, 23.3% stage II, 9.8% stage III, and 0% stage IV (p less than 0.001). No significant differences in actuarial 5-year survival rates were observed between TGER (17.8%) and PGER (14.9%). Sex, duration of symptoms, and histologic type did not reveal prognostic significance. CONCLUSIONS: In early tumors a total gastrectomy with resection of 10 cm of esophagus above the tumor is advocated.

Adenocarcinoma↗

[Surgical management of pancreatic endocrine tumors in patients with MEN 1 syndrome. Considerations on one case observed].

INTRODUCTION: Particular problems in MEN 1 syndrome come from the morphological identification of pancreatic tumors because of their are often small [<1 cm] and multiple [89% of the cases]. However intraoperatively it could be difficult to identify with palpation the tumors described by preoperative investigations and to decide the most suitable surgical treatment. The authors describe one case recently observed to underline and update the correct management. CASE REPORT: A 34 year old woman was admitted for the surgical treatment of an insulinoma. Polimenorrea, hypercalcemia and familiarity for MEN 1 syndrome were also present. A CT scan showed the tumors in the body and tail of the pancreas [diameter 0.5-1 cm]. MRI described only a small mass in pancreatic head. A calcium angiography was positive for insulin secretion after calcium infusion in hepatic and gastroduodenal artery, and for glucagon secretion after infusion in splenic artery. An intraoperative ultrasonography discovered three nodules that were enucleated. They were one insulinoma and two glucagonomas respectively. After enucleation glycemia became immediately normal. CONCLUSION: To avoid wide surgical resections [es. left pancreatectomy] we suggest a conservative treatment [multiple enucletion with or without a pancreatic-jejunum side-to-side anastomosis] with a meticulous preoperative and intraoperative evaluation of all pancreatic nodules.

Adult↗

[Endoscopic-laparoscopic treatment of neoplastic occlusion of the left colon].

About a third of patients with colorectal carcinoma have acute colonic obstruction requiring emergency surgery. The surgical options are: intraoperative lavage and resection of the colonic segment involved with primary anastomosis; subtotal colectomy with primary anastomosis; colostomy followed by resection; and resection of the colonic segment involved with an end colostomy (Hartman's procedure) requiring a second operation to reconstruct the colon. These procedures present risks and are associated with a poor quality of life. Endoscopic colonic stent insertion effectively decompresses the obstructed colon allowing bowel preparation and elective resection. In this article we present 2 cases successfully treated with the use of stents followed by a laparoscopic resection. We also describe technical details concerning the endoscopy and laparoscopy procedure, discuss the advantages of this treatment and present a review of the literature. One patient underwent a left hemicolectomy; while the other was treated with splenic flexure resection. No complications occurred after surgery. Histological staging revealed a pT3 pNO pMx G2 and a pT4 pN1 pM1 G2 adenocarcinoma, respectively. This initial experience shows that endoscopic colonic stent insertion can effectively resolve the neoplastic obstruction, allowing safe elective surgery. The use of stents does not prevent a laparoscopic approach.

Adenocarcinoma↗

Functional results of a personal technique of reconstruction after pancreaticoduodenectomy.

The aim of the study is to propose a new technique of reconstruction after pancreaticoduodenectomy with more attention to the functional aspects. From 1995 and 2003, 25 patients underwent pancreaticoduodenectomy for pancreatic or periampullary cancer. The reconstruction was carried out by: end-to-end gastro-jejunal anastomosis (first jejunal loop); a Roux-en-Y T-T pancreatico-jejunal anastomosis leaving a silastic catheter in the Wirsung; hepatico-jejunostomy and jejuno-jejunostomy below the biliary anastomosis; superselective vagotomy. Mortality was 8%. Regarding the complications, we observed 3 biliary fistulas, mean duration 5 days, with spontaneous healing; 8 pleural effusions and 7 wound infections. Postoperative 3 months reevaluation showed weight gain in 14 patients with no other digestive symptoms (vomiting, fullness, dumping). With a scintigraphic meal we observed a good rythmic and regular gastric emptying. No jejunal peptic ulcers were noted in all patients after the gastric protonic pump inhibitors were discontinued. Fecal fats were evaluated in all cases for malabsorption 3 months after operation with low fat fecal levels. The preliminary results of our recent experience seem to be encouraging. This technique may have a useful application in the clinical setting as far as radicality and quality of life of the patients with pancreaticoduodenectomy.

Ampulla of Vater↗

[Adenocarcinoma of the cardia: results of resection with nic interposition].

The incidence of cancer of the cardia is increasing. Surgical treatment, in a small percentage of patients in early stage, consists in esophagectomy and total gastrectomy, followed by colon interposition. The operation can be performed through the thorax or by an abdominal and cervical approach. From 1982 to 1989 twenty-five patients were operated on. Between 1980 and 1982 the cancer was resected opening the thorax in 9 patients (group A), while from 1983 to 1989 the esophagus was removed through diaphragmatic hiatus in 16 patients (group B). All cases were analyzed as for histology, staging and resection margin. Average clinical follow-up was 22 +/- 32.7 months (range 1-102). Morbidity was 77.7% in group A and 68% in group B. Mortality dropped to 14% in the last 7 patients. Mean survival time was 23 +/- 34 months in group A and 11.2 +/- 13.7 months in group B (p = N.S.). Follow-up controls showed that all patients, in whom the esophagus was replaced with colon, were able to feed adequately.

Adenocarcinoma↗

Extracapsular lymphnode metastases in the staging and prognosis of gastric cancer.

The aim of this study was to evaluate the influence of the degree of nodal involvement (extracapsular vs intracapsular) on survival of 121 patients radically resected for gastric adenocarcinoma with nodal metastases at the Department of Clinica Chirurgica I of "La Sapienza" University of Rome. Patients with extracapsular nodal metastases had a worse 10-year survival rate than those with intracapsular nodal involvement (7.9% vs 22.4%). A better prognosis among patients with intracapsular node metastases was observed in each p-TN subgroup. In the multifactorial analysis (3-way ANOVA) survival was correlated with the depth of invasion of the gastric wall and the degree of lymphnode involvement (p less than 0.01) but not with the level of nodal involvement (N1 vs N2). Our results suggest that the degree of nodal involvement is an important independent prognostic factor that should be considered in the current staging system for curative resection in gastric carcinoma.

Adenocarcinoma↗

[Low colorectal anastomosis using the Knight and Griffen technique].

The low anterior resection currently represents the procedure of choice in the treatment of middle and low rectal cancer. The double stapling technique described by Knight and Griffen may be considered a safe and valid alternative able to decrease the incidence of complications related to a low colorectal anastomosis.

Anastomosis, Surgical↗

[Occlusion of the duct with a fibrin glue and preservation of the pylorus after resection of the duodenum and head of the pancreas for periampullary carcinoma].

Dehiscence of pancreaticojejunostomy represent the main technical postoperative complication after duodenocephalopancreasectomy for periampullary carcinoma. The incidence of this complication is particularly high in cases of narrow duct and a tender pancreatic gland. In this case the authors suggest a technique of occlusion of the residual pancreatic stump using a fibrin sealant. This approach was utilized in 6 consecutive patients affected by resectable periampullary carcinoma. No postoperative mortality was observed. Pancreatic fistula developed in 5 cases and all of them resolved spontaneously in 1-4 months. The sixth patient underwent, at 3 months p-o, a CT-guided percutaneous aspiration of an intraabdominal fluid collection and with no further complications. 3 patients died at 3, 9 and 11 months because of liver metastases. Currently 3 patients are alive and apparently disease free at 25, 7 and 5 months. Pancreatic endocrine function was assessed in 5 patients at 3 months p-o. Blood glucose and insulin, glucagon and C-peptide plasma levels, all fasting and 1 our after a standard meal, revealed a normal glucose metabolism. The authors conclude that, since fibrin sealant avoids the pancreatic fibrosis which could be induced by non-absorbable polymers and the benign evolution of this type of pancreatic fistula, this method for handling the exocrine secretion is a safe and satisfactory approach which is particularly indicated in case of a pancreatic stump at risk for intestinal anastomoses.

Adenocarcinoma↗

[The echographic aspects of the splenoportal axis in patients with esophageal varices].

In the present study the A. try to assess the usefulness of ultrasound in detecting blending risk in portal hypertension. Endoscopic and sonographic findings of 35 cirrhotic patients with esophageal varices were compared to a control group of 27 hepatopathy patients without portal hypertension, observed in the same department and in the same interval. The medium caliber of portal vein was 14.64 +/- 2.59 and 11.8 +/- 1.33 mm, respectively in the study and in the control group (p less than 0.01). Statistic correlations were also found in subgroups of patients with varices of different degrees. A portal vein greater than 16 mm was found in 10 of 11 patients with large varices; this pattern provides a sensitivity of 71.4% and a specificity of 94.1%. In our experience this echographic finding should represents a clear indication for close endoscopic follow-up, even in patients without previous bleeding from esophageal varices.

Adult↗

Heller-Belsey and Heller-Nissen operations for achalasia of the esophagus.

From 1972 to 1985, 101 consecutive patients underwent Heller's myotomy and Belsey repair (H + B) (n = 43) or Nissen fundoplication (H + N) (n = 58) for achalasia of the esophagus. There was no operative mortality after either operation; minor pulmonary complications occurred after H + B procedure in 9.3 per cent of the patients. Good to excellent long term results were achieved in 87 per cent of the patients after H + B repair and 83 per cent of the patients undergoing H + N. The failure rates were 2.5 and 11.3 per cent, respectively. The analyses of postoperative esophageal symptoms showed that the incidence of heartburn was greater after H + B repair and the incidence of obstructive symptoms was greater after H + N. The inability to vomit or belch, or both, was 10.2 per cent in the H + B group and 13.1 per cent in the H + N group. Finally, 56.4 per cent of patients after H + B repair and 41.0 per cent of those after myotomy and H + N were considered to be cured after the operative procedure was performed. Patients were improved in 41.0 and 47.3 per cent, respectively.

Adolescent↗