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Objective: To determine the results of gastric pull-up reconstruction following pharyngo-laryngo-esophagectomy., Design: Retrospective review of 59 patients who underwent gastric pull-up reconstruction for carcinoma of the hypopharynx, larynx, and cervical esophagus during a 10-year period from 1983 to 1993., Setting: Combined Head and Neck Service and Section of General Surgery, Department of Surgery, Yale University Medical School, New Haven, Conn., Main Outcome Measures: Morbidity, mortality, time to oral feeding, length of hospitalization, and long-term results., Results: The mortality rate was 5%, perioperative morbidity rate, 27%, and the medical morbidity rate, 32%. Most patients took oral feedings within 6 days after surgery and were discharged within 16 days. There were two temporary fistulas and four strictures, none of which were permanent. There were no intrathoracic or mediastinal injuries or hemorrhage. The overall survival was 1 to 100 months (median survival, 12 months); the median survival was 34 months for patients alive to date and 8 months for patients who died., Conclusions: The gastric pull-up reconstruction is a safe, effective operation with a low mortality rate and excellent long-term functional results for patients with extensive carcinoma of the hypopharynx, larynx, and cervical esophagus.

Although esophageal perforation complicates about 5% of pneumatic dilatations performed for achalasia, little is known about associated hospital and long-term courses. In order to assess the outcome of such patients undergoing emergency surgery for repair, records of seven patients sustaining perforation during pneumatic dilatation were compared to those of five patients undergoing elective myotomy during the same period. In perforation patients, mean intervals following the procedure were 3.6 hr to administration of antibiotics and 9.6 hr to surgery. The perforation and elective myotomy groups had similar mean durations of operation (3.8 vs 3.3 hr), intensive care stays (2 vs 1 days) and hospitalization (12 vs 11 days); perforation patients had a significantly longer mean interval from surgery to oral intake (7 vs 5 days). Postdischarge long-term outcomes were alike in the groups. It is concluded that patients with perforation from pneumatic dilatation that is recognized and treated promptly have outcomes that are comparable to those of patients who undergo elective myotomy.

Syndromes involving peptide or nonsex steroid hormone secretion due to aberrantly located tumors are rare. We report a collected series of 16 patients with ectopic hormone production from ovarian neoplasms, including 3 patients recently encountered at our institution as well as 13 additional cases identified in the recent literature. These tumors included 2 insulin-producing ovarian carcinoids, 1 ACTH-producing pituitary adenoma within a benign ovarian cystic teratoma, 2 cortisol-producing ovarian neoplasms, 8 gastrin-producing ovarian cystadenomata or cystadenocarcinomata, and 3 thyroxine-producing ovarian strumal carcinoids. All patients presented with syndromes of hormone excess. Only 62% of all tumors were localized preoperatively. Following ovarian resection, 87% of patients remained disease-free with a median follow-up period of 1.5 years. In addition to ovariectomy, 8 additional unnecessary ablative procedures were performed in 7 patients. These included distal pancreatectomy, pancreaticoduodenectomy, adrenalectomy, total gastrectomy, selective vagotomy, and subtotal thyroidectomy. Failure to localize the ovarian neoplasm preoperatively was associated with a significantly higher risk of subsequent unnecessary ablative procedures. Because of the potential for the ovary to act as a source of aberrant hormone secretion, we recommend complete preoperative evaluation of the pelvis in female patients presenting with nonlocalizable endocrine tumors.

The relationship between gastrointestinal neoplasms and Crohn's disease is poorly defined. The purpose of this study was to characterize the features of gastrointestinal malignancies that developed in Crohn's patients. In this retrospective review the authors identified six patients with Crohn's disease who developed such lesions over a 20-year period: four patients had colorectal cancers and two had ileal malignant neoplasms. Patients averaged 52.7 years of age (range, 21 to 61 years). Three patients were men and three women. Five of the six patients had endured Crohn's disease for more than 20 years. Only two lesions were diagnosed before surgery. The colorectal lesions were predominantly right-sided and all occurred in bowel segments with active Crohn's disease. The lesions demonstrated aggressive histologic features: three of six tumors were poorly differentiated, one of the five adenocarcinomas was mucinous, and three of the colorectal cancers were Dukes' B or C lesions. Four of six patients survived five or more years. There was a single malignant carcinoid, which represents the seventh case report of a carcinoid tumor occurring in a patient with Crohn's disease. This study indicates that patients with Crohn's disease develop a wide variety of small bowel and colorectal cancers. Furthermore, it suggests that Crohn's patients with colonic disease should periodically undergo surveillance colonoscopy.

A 25 year old woman presented with idiopathic thrombocytopenic purpura. She had undergon splenectomy 12 years previously for traumatic splenic rupture. Thrombocytopenia was ultimately resistant to steroid therapy. Howell-Jolly bodies were absent from the peripheral smear and 99mTC-spleen scan demonstrated foci of increased uptake thought consistent with accessory spleens. However, splenosis alone was demonstrated at laparotomy, and all visible splenotic tissue was surgically removed. The patient responded and adequate platelet counts were maintained after discontinuation of steroid therapy. The functional capacity of splenic implants has been previously demonstrated both in animal and man. However, reports linking splenosis to hematologic disease are rare. In the present case, characteristic splenic function was demonstrated by both the the -9mTc-spleen scan and the absence of the typical peripheral blood findings of asplenia. The hematologic response to the removal of the splenotic tissue attests to its importance in maintaining the thrombocytopenic state. In the setting of prior splenectomy for splenic trauma, splenosis may contribute to hematologic disease. Removal of this splenotic tissue may result in hematologic remission.

Diagnosis and management of pheochromocytoma, once dangerous and uncertain, have been dramatically altered in recent years by advances in imaging, assays, and pharmaceuticals. During the past ten years we have treated 18 patients who had pheochromocytoma. Biochemical diagnosis was made in all patients by measurement of 24-hour urinary total catecholamine excretion or by epinephrine-norepinephrine fractionation. Determination of epinephrine-norepinephrine ratios was instrumental in making the diagnosis of pheochromocytoma in two patients in whom total catecholamine levels were normal. Localization of the pheochromocytoma in the most recently treated cases was accomplished by ultrasound, computed tomography, or iodine I 131 iobenguane (iodine I 131 metaiodobenzylguanidine) scanning. Nine patients in the series were prepared for surgery with phenoxybenzamine hydrochloride and six with prazosin hydrochloride. Preoperative total alpha-adrenergic blockade with phenoxybenzamine offered no advantage over selective blockade with prazosin in terms of perioperative fluid requirements or intraoperative hemodynamic stability.

This is the first report in the American literature of a recently described histologic variant of primary sclerosing cholangitis (PSC). Only six examples of this unique entity, called cholangitis glandularis proliferans (CGP), or proliferative cholangitis, have previously been reported. This disorder typically presents as painless jaundice but differs clinically from PSC by occurring predominantly in females and lacking an association with inflammatory bowel disease. The unique histological features are characterized by florid intramural proliferation of glandular elements in addition to an intense inflammatory component. Anatomically, the lesion is confined to the extrahepatic biliary tree, which enables its successful surgical extirpation since it does not appear to be progressive.

Primary sclerosing cholangitis is an idiopathic disease characterized by progressive diffuse stricture of extrahepatic and intrahepatic bile ducts. Eighteen patients with end-stage symptoms of primary sclerosing cholangitis were evaluated during a 10-year period from 1976 to 1986. Nine patients presented with disease amenable to intrahepatic cholangiojejunostomy. All patients presented with elevated liver function test results, and six of nine patients had a history of ulcerative colitis. The mean survival after intrahepatic cholangiojejunostomy was 3.9 years (range, 4 months to 10 years). Two of three of the patients with biliary cirrhosis died within 1 year after surgery. Four of nine patients remain alive today, with a mean survival of 4.7 years. For patients with end-stage primary sclerosing cholangitis, intrahepatic cholangiojejunostomy provides effective surgical palliation in those without secondary biliary cirrhosis.

A simplified technic for performing intrahepatic cholangiojejunostomy has been described, which can be utilized to bypass proximal biliary tract obstruction where the usual methods of choledochoenteric decompression are not possible. This technic has been performed nine times in seven patients, with a mortality of 30 per cent. In all but the two patients who died of liver failure in the immediate postoperative period, jaundice and pruritus were relieved. There was one wound infection and one instance of stenosis of the anastomosis which responded to dilatation.

Gastrointestinal hemorrhage secondary to hemosuccus pancreaticus is a rare condition that poses a significant diagnostic and therapeutic challenge. It is reported to occur most commonly in the setting of acute or chronic pancreatitis with rupture of pseudoaneurysms of the spleen or hepatic artery into the pancreatic duct. In this report three such cases have been reported. Abdominal ultrasound and CT scanning can noninvasively define pancreatic pseudocysts with a high degree of accuracy. Real-time ultrasonography may document a pulsatile pseudoaneurysm. Radionuclide arterial scanning, by demonstrating pooling of blood in the area of a pseudocyst, can point to the source of bleeding in patients with pancreatitis and gastrointestinal hemorrhage. Selective celiac angiography, however, is the only diagnostic test that can definitively outline a pseudoaneurysm and demonstrate its rupture into a pseudocyst or into the pancreatic duct. Pancreatic resection including excision of the pseudoaneurysm and pseudocyst (when present) is the treatment of choice. In cases where resection is not possible, ligation of the artery proximal and distal to the pseudoaneurysm and drainage of the pseudocyst into the gastrointestinal tract is an acceptable alternative procedure. Although intraarterial catheter embolization of the bleeding vessel can be a lifesaving procedure in these very sick patients, subsequent resection of the lesion is warranted as the definitive treatment.

In our institution, segmental Crohn's colitis has been treated with segmental colectomy rather than more extensive resection. The purpose of this study was to review the rate of recurrence following surgical treatment of Crohn's colitis using this approach. From 1974 through 1984, 37 patients with Crohn's disease limited to the colon or rectum underwent resection. Average time of follow-up was 5.5 years. There were recurrences in 13 (62%) of 21 patients treated by segmental colectomy and four (67%) of six patients treated by total abdominal colectomy and small-bowel recurrences in two (20%) of ten patients treated by proctocolectomy. Intestinal continuity was maintained in 17 (81%) of the 21 patients treated by segmental resection. Although recurrence is likely, segmental colectomy improves the quality of life by delaying the need for a stoma and by preserving functioning bowel.

Percutaneous transhepatic cholangiography is a safe, effective diagnostic procedure for use in evaluating the jaundiced patient. As is the case with most invasive diagnostic procedures there is a risk: an overall mortality rate of 0.5% and morbidity rate of 3-10%. Fortunately hemobilia is an uncommon complication, encountered only four times in our series of 102 percutaneous cholangiograms. In every case of hemobilia the clotting parameters were normal. The one factor common to each case was distal obstruction of the extra hepatic bile ducts. However, this one factor may play an important role in the etiology and therapy of post cholangiographic hemobilia. The hemorrhage subsided spontaneously in every case following surgical decompression of the bile ducts and there was no further active bleeding postoperatively. The possible explanation for the cause of bleeding and the fact that it subsided following demcompression of the bile ducts is discussed. All four patients survived this complication and in the 102 patients there were no deaths attributable to percutaneous transhepatic cholangiography.

A patient with an hepatic artery aneurysm is presented with preoperative angiographs, and intra-operative photographs demonstrating erosion and fistulazation into the common duct. The aneurysm was ligated and excised, and the erosion of the common duct was treated successfully with a Roux-en-Y choledochoduodenstomy. This is the fourth case with erosion into the common duct to be treated successfully by surgery. The other three cases are discussed. A review of the literature reveals that hepatic artery aneurysms are rare, and successful treatment is based on several different techniques. Ligation of the hepatic artery, with or without excision of the aneurysm, is discussed as a method that may in fact be the safest treatment in a group of patients who because of their disease are already in a high risk category.

A technic for pancreatobiliary sphincteroplasty providing accurate approximation of tissues in a bloodless field has been presented. It is suitable and adaptable to the common duct with duodenum, as well as pancreatic duct with common duct.

To evaluate the use of biliary scintigraphy with 99m Tc-dimethyl analogs (HIDA) in traumatic liver injuries, a group of 26 patients with penetrating and blunt liver injuries were studied. The results indicate that HIDA scanning is an effective noninvasive method of evaluating the hepatobiliary tree in the post-traumatic setting. The HIDA scan is a sensitive tool for studying the hepatic parenchyma and the presence or absence of bile leaks. Its evaluation of the extrahepatic biliary ductal system is not specific and should be assessed with further studies. Gallbladder nonvisualization by HIDA scans in this setting cannot be presumed to be secondary to acute cholecystitis and should be interpreted with extreme caution.

Lymphomatoid granulomatosis is a necrotizing vasculitis involving multiple organs which may be associated with lymphomatous transformation. A case of lymphomatoid granulomatosis is presented in which gray scale ultrasound identified lymphomatous transformation involving the right kidney and retroperitoneum, thus preventing unnecessary surgery. Ultrasound is a valuable modality for identifying and characterizing abdominal masses in patients at risk for neoplastic disease.

When faced with a patient with possible acute cholecystitis, technetium-99m-HIDA scintigraphy should be the primary diagnostic procedure performed. If scintigraphy reveals a normal gallbladder, acute cholecystitis is excluded. If the scintigram fails to visualize the gallbladder, ultrasonography is deemed advisable to exclude potential false-positive scintigrams and confirm the presence of cholelithiasis.

Intra-abdominal abscesses after colon surgery usually appear within eight weeks. Over the past few years, we have observed nine patients who underwent colectomy for inflammatory bowel disease in whom large and relatively asymptomatic abscesses developed, requiring surgical drainage as long as three to five years after the operation. In five patients, there was no report of intraoperative complications such as perforation or fecal spillage. At the time of discharge from the hospital, all patients were afebrile. In three of these patients, abscesses were discovered when they were readmitted to the hospital for elective abdominal procedures; most of the others had localized abdominal pain.

Forty-five consecutive cases of primary small bowel malignant tumors treated at our institution from 1969-1983 were reviewed. Sixty-four per cent of the patients had surgical emergencies. Fourteen patients had intestinal obstruction, 11 had gastrointestinal (GI) hemorrhage, and 4 had perforation of the small bowel. The remaining 16 patients were explored for persistent symptoms, an abnormality on GI series, or a mass lesion on CT scan. Thirty-eight patients underwent resection of the tumor and seven had palliative bypass procedures. This study suggests that a high index of suspicion and early diagnostic evaluation including a small bowel series is necessary to prevent the large number of small bowel tumors presenting as surgical emergencies. In particular, small bowel tumors should be suspected in patients with abdominal pain of unknown etiology, unexplained weight loss, or occult GI bleeding.