Bouveret's syndrome: a rare consequence of malignant cholecystoduodenal fistula

We report a case of duodenal gallstone obstruction, resulting from cholecystoduodenal fistula. Fistulization was associated with a repeat chronic inflammation and metastatic carcinomatous infiltration. The diagnosis was confirmed by ultrasonography, barium meal examination, and gastroscopy. An attempt at endoscopic extraction was unsuccessful and the patient was referred for operative therapy. We present a graphic algorithm for decision-making in Bouveret's syndrome.     

Duodenal obstruction by biliary lithiasis (Bouveret's syndrome)]

The case of an 81-year-old woman admitted for abdominal pain, nausea and vomiting diagnosed with Bouveret syndrome is presented. This rare disease, consisting in obstruction of gastric emptying by gallstones located in the pylorus or the duodenal bulb predominates in elderly women and represents 2% of the cases of biliary ileum. Diagnosis is based on the clinical manifestations, the existence of aerobilia, visualization of lithiasis by radiography, echography or fiber gastroscopy and the demonstration of duodenal obstruction. The treatment of choice is surgery consisting in gastrotomy or enterotomy and extraction of the stone. The importance of diagnosis and early treatment is emphasized to improve prognosis.     

Ultrastructure of the glial cells after spinal cord compression in rabbits

Sump syndrome is a rare complication of biliary-enteric anastomosis. Classically, the distal bile duct becomes obstructed by gastrointestinal debris after choledochoduodenostomy, resulting in cholangitis or, less commonly pancreatitis. Obstruction of the biliary tree by gastrointestinal contents after Roux-en-Y choledochojejunostomy or hepaticojejunostomy has not been described in the English-language literature. This report details the diagnostic and operative management of the first patient with sump syndrome after hepaticojejunostomy. The presumed pathophysiology was reflux of vegetable matter up the efferent limb, resulting in hepatic duct obstruction and cholangitis. The patient ultimately required complex choledochoscopic drainage of the intrahepatic biliary tree and revision of the previous Roux-en-Y hepaticojejunostomy.     

Gastroduodenal, ileocolic and perianal Crohn's disease

The authors present a case of Crohn's disease that initially showed common localisations, and later a rare gastro-duodenal involvement. When the definitive diagnosis of the latter was reached there were already signs of obstruction at the exit of the stomach. Treatment with Prednisolone I.M. and Omeprazole was not successful, and surgery was necessary as a last recourse to resolve the obstruction. A theoretic revision of the main clinical, diagnostic, and therapeutic aspects of this rare from of involvement of Crohn's disease is also presented.     

Cholestasis secondary to Hodgkin's disease: report of 2 cases of vanishing bile duct syndrome

Only a small percentage of patients with Hodgkin's disease become clinically Jaundiced during their disease. This Jaundice may be secondary to biliary obstruction, hemolysis, direct hepatic infiltration by the disease, drug toxicity or viral hepatitis. Vanishing bile duct syndrome secondary to Hodgkin's disease is a rare cause of cholestasis in these patients, only 13 cases having been reported so far. The authors describe 2 patients who developed severe Jaundice secondary to Hodgkin's disease due to vanishing bile duct syndrome affecting small intrahepatic bile ducts.     

Selected psychiatric symptoms associated with rate of cognitive decline in patients with Alzheimer''s disease

Endoscopy Sphincterotomy is an important therapeutic procedure in the treatment of some biliary and pancreatic disorders. In this paper, we report our experience over this subject. In our patients the main indication to perform this procedure was common bile duct stones; they were 83% of the total cases. In those cases, 67% were residual stones after cholecystectomy by laparotomy and laparoscopy. We performed sphincterotomy in 33% of patients with gallbladders in situ. In five patients (7.57%), we made the diagnosis of Oddi's sphincter dysfunction taken in account clinical, biochemical an radiological findings. No manometric measurements were done. We found some unusual cases, such as: choledococele and Sump syndrome. In five patients we had complications, 3 of them bled and the other two developed edematous pancreatitis. All these complications were solved with medical treatment.     

The rare problem of late recurrence in neuroblastoma

Two-year disease-free survival is considered highly predictive of therapeutic success with neuroblastoma and death from neuroblastoma greater than 5 years after diagnosis is a rare event. We report of an unusual case of death from neuroblastoma 20 years after diagnosis; a literature review and discussion of this rare phenomenon of late recurrence is also included. Five additional cases of late recurrence, defined as greater than 5 years after diagnosis, were found in the literature with adequate detail to be reviewed. Advanced disease was present at diagnosis in five of six cases; three of six had disseminated disease. No age or sex differences were noted. A peculiar tencency for recurrence during the teenage years, preceded by a long disease-free interval, was also present in all cases, perhaps a reflection of maturational alterations in the immune system or hormonal changes that previously had suppressed extensive disease. This phenomenon of late recurrence in neuroblastoma, albeit rare, adds another facet to the clinical behavior of an enigmatic and therapeutically unyielding childhood tumor.     

Indications, clinical value and complications of endoscopic retrograde cholangiopancreatography

Endoscopy combined with endoscopic retrograde cholangiopancreatography is a unique tool providing a broad, precise yield of diagnostic information about diseases of the upper gastrointestinal tract. In the absence of gross anatomic disease, its diagnostic accuracy should exceed 90 per cent and risk be minuscule. The incidence of complications and, therefore, risk to the patient and proportional to the magnitude and remedial nature of the disease processes found. In the absence of diagnostic procedures for diseases of the biliary tree and pancreas that are riskless, the relationship of endoscopic retrograde cholangiopancreatography risk to diagnostic value and patient salvage emphasizes the value of endoscopy combined with endoscopic retrograde cholangiopancreatography as the diagnostic procedure of choice for patients with suspected disease of the biliary tract or pancreas. The unique value of endoscopic retrograde cholangiopancreatography in providing rapid visualization of diseases of the upper gastrointestinal tract mucosa combined with cholangiography and pancreatography is dramatized in the patient with upper abdominal pain syndrome with or without jaundice. In the face of a rapidly evolving illness and the potential need for early surgical intervention, barium studies are relatively contraindicated and have a poor diagnostic yield. By contrast, endoscopic retrograde cholangiopancreatography combined with endoscopy rapidly rules out, or, in the presence of disease of the upper gastrointestinal tract, permits the immediate use of subsequent diagnostic procedures. An experience with more than 1,000 procedures reveals that endoscopic retrograde cholangiopancreatography is an invaluable tool for the diagnosis and management of disease of the pancreas and biliary tract.     

Tuberculous pseudotumor causing biliary obstruction. Report of a case with diagnosis by fine needle aspiration biopsy and bile cytology

Hepatobiliary tuberculosis is a rare but distinct clinical entity. We report an unusual case of biliary tract obstruction due to localized hepatic tuberculosis with periportal tuberculous adenitis. The lesion mimicked a malignancy clinically and radiologically. Fine needle aspiration biopsy revealed granulomas, epithelioid histiocytes and Langhans' giant cells. The cytodiagnosis was confirmed by identification of acid-fast bacilli in the bile cytology and isolation of Mycobacterium tuberculosis by culture. The patient responded to antituberculosis therapy. The usefulness of bile cytology in the diagnostic management of biliary tract obstruction is illustrated.     

Changes in the activities of antioxidant enzymes of the lymphoid organs of 21-day pregnant rats due to administration of fish oil by gavage

Budd-Chiari syndrome (BCS) due to membranous obstruction of the hepatic vein and the inferior vena cava is rare in children. We report a child with BCS that had a membranous obstruction at the level of the hepatic veins. The web was successfully dilated percutaneously by balloon catheters. Symptoms and signs of obstruction improved without any complication. As percutaneous catheterization is an effective, safe and repatable procedure, we recommend this technique for treatment of children and adults with BCS due to membranous obstruction of the hepatic veins.     

A case of abdominal cocoon

Abdominal cocoon is a rare disease of the peritoneum and almost invariably presents as an acute or subacute intestinal obstruction with or without a mass. The etiology of this disease is largely unknown and abdominal cocoon of unknown etiology has been limited to the tropical and subtropical zones and primarily affects young adolescent females. In the temperate zone, only one case has been reported from the United Kingdom, but the patient was also born in Pakistan. No case of abdominal cocoon purely developed in the temperate zone has been reported. Recently, we experienced a case of abdominal cocoon in a 34-year-old female patient(Korean) who had never been abroad. The diagnosis was made postoperatively by reviewing the literature. We herein report this rare condition developed in an unusual geographical location with a brief review of the literature.     

A comparison of the bactericidal and photobactericidal activities of aminoacridines and bis(aminoacridines)

The nasal cavity is a rare location for primary malignant melanoma, borne out by the few cases reported in the literature since 1940. Rarest of all is nasal melanoma in a patient with oculocutaneous albinism. An albino subject, aged 33, was referred to us with this diagnosis. Because of the numerous metastases in the liver and left lung, we did not advise radical surgical intervention and, despite hyperfractionated high dose radiotherapy and chemo-immunotherapy, the advanced stage of disease caused the death of the patient within a year. We have reviewed the diagnostic, clinical and therapeutic aspects, and the literature on melanomas of the nasal cavity in albino subjects. We performed a Medline literature search, examining only 'extensive' papers and excluding abstracts or case reports presenting poor data.     

A simplified percutaneous hepatogastric drainage technique for malignant biliary obstruction

We describe a technique to bypass a malignant biliary obstruction by creating a direct connection between the left biliary tree and the stomach. First, adherence between the left liver parenchyma and the stomach is achieved by a Cope anchor system. Then, the left biliary tree and stomach are connected by Colapinto needle puncture of the stomach from a left biliary duct with the needle inserted through a 9 Fr transhepatic sheath. Over a stiff guidewire, a Ring drainage catheter is placed. Later, the Ring catheter is replaced by a metallic stent. Four patients with malignant biliary obstruction underwent this procedure. The mean survival time was 77 days with maximum follow-up of 171 days. Neither obstruction nor dislocation of the metallic stents occurred.     

Primary gastric tuberculosis

Intestinal tuberculosis (TB) comprises 5% of all cases of TB and may be a major problem in immigrant communities, although the incidence of pulmonary TB is declining. Gastric TB is rare, constituting 0.1-2% of all cases of TB. Gastric TB usually develops secondary to other tuberculous lesions, most commonly pulmonary. On endoscopy antral infiltrative lesions are found. Primary gastric TB is very rare, only 8 cases having been reported in the English literature. We report a case of primary gastric TB in a 55-year-old woman who presented with abdominal pain and gastric outlet obstruction. The diagnosis was confirmed by endoscopic biopsies which showed granulomas, but no acid-fast bacilli. The Mantoux test was positive, acid-fast bacilli were found in the gastric juice, and a positive culture for TB was obtained on gastric lavage. There was an excellent response to antituberculous chemotherapy. With the relative rate of extra-pulmonary TB increasing, primary gastric TB should be taken into account in the differential diagnosis of infiltrative lesions of the antrum.     

Choledochoduodenostomy, choledochojejunostomy or sphincteroplasty for biliary and pancreatic disease

Choledochoduodenostomy, choledochojejunostomy, or sphincteroplasty are used in the treatment of selected patients with retained, recurrent, and impacted bile duct stones; strictures of the bile ducts; stenosis of the sphincter of Oddi; pancreatitis associated with biliary disease; choledochal cysts; fistulas of the bile duct; and biliary obstruction, either benign or malignant. From a group of approximately 1600 patients operated on for biliary and pancreatic disease during the 17-year period, 1962 to 1979, 153 patients who had choledochoduodenostomy, choledochojejunostomy or sphincteroplasty were identified. Follow-up information was available for 146 patients (95%). Overall, 84% of the patients had good results, 10% had fair results, and 3% had poor results. A 3% postoperative mortality rate was found, all in patients with unresectable malignancies. Treatment of bile duct obstruction, benign or malignant, was equally effective by choledochoduodenostomy or choledochojejunostomy. Jaundice resolved in all patients; three patients with benign strictures required reoperations for recurrent stricture formation, two after choledochoduodenostomy, and one after choledochojejunostomy. Recurrent cholangitis heralded the development of another stricture. Both choledochoduodenostomy and sphincteroplasty were used for patients with retained, recurrent or impacted duct stones. Pancreatitis did not occur in any patient after sphincteroplasty; the sump syndrome was not seen after choledochoduodenostomy. This review supports the view that choledochoduodenostomy is a safe and effective procedure. All three operative procedures were effective for the problems for which they were used; each procedure has a place in the treatment of recurrent or complicated biliary and pancreatic diseases. The procedures are complementary, not competitive. For certain problems, the operation performed depends upon the surgeon's preference and experience. The indications for and results of these operative procedures are discussed.     

Clonal expansion of T-cells in measles

Videolaparoscopic cholecystectomy is considered the treatment of choice for simple cholelithiasis. Now many surgeons consider the laparscopic procedure usable also in the complicated biliary lithiasis like acute cholecystitis and choledocholithiasis. The authors report their recent experience of the laparoscopic treatment of biliary lithiasis, regarding 221 non-selected patients (69% symptomatic cholelithiasis, 20% chronic cholecystitis, 4.5% acute cholecystitis, 4.5% coledocolithiasis, 2% hydrops). The diagnostic-therapeutic protocol and the results are described and compared with the beginning of their experience, when they treated only symptomatic gallbladder stone disease, and with the reports of the literature. The authors concluded that the laparoscopic procedure is a good chance for the surgeon in the treatment of all cases of benign biliary disease. But, in particular for patients with choledocholithiasis, he has be able to know all the diagnostic and therapeutic possibilities, to choose the best in every single case.     

Transient pancytopenia after non-A non-B non-C acute hepatitis preceding acute lymphoblastic leukemia

Transient pancytopenia preceding childhood acute lymphoblastic leukemia (ALL) is an unfrequent but well-known event. The association of this preleukemic syndrome with hepatitis is extremely rare, with only two such cases having been published in the literature. We report the case of a 16-year-old boy who was diagnosed with B-cell type ALL that was preceded by transient pancytopenia with absent hemopoietic cells in the bone marrow following a seronegative hepatitis episode. The clinical, morphologic and immunophenotypic features of this patient are described and the literature on this preleukemic syndrome reviewed, with special emphasis being made on its differential diagnosis with hepatitis-associated aplastic anemia.     

Flow injection amperometric determination of L-dopa, epinephrine or dopamine in pharmaceutical preparations

OBJECTIVE: The association of systemic lupus erythematosus (SLE) and multiple myeloma (MM) is an uncommon event. We report the relapse of SLE in a patient with a previous history of MM, treated with chemotherapy and, subsequently, with alpha-2b interferon (alpha-2b IFN) as a maintenance therapy. The case is discussed in light of past relevant literature. METHODS: The history and clinical, laboratory and radiographic findings of the patient, as well as the subsequent therapeutic approach are discussed. In our review of the literature, journal articles are identified by Medline search. RESULTS: We describe the case of a woman who developed a multiple myeloma 14 years after a diagnosis of SLE. A careful literature review confirms that the association of these two diseases has been reported only in a few cases. When the plasma cell neoplasia occurred, SLE had been quiescent for several years; the patient was treated with prednisone-melphalan and, subsequently, with alpha-2b IFN as a maintenance therapy. On admission to our department, SLE was in a relapse phase, probably because of IFN treatment. The disease was poorly responsive to steroid therapy and required the use of cytotoxic drugs. CONCLUSIONS: The coexistence of SLE and MM is very rare and the possible pathogenetic mechanisms underlying this association remain unclear. The use of interferon in a patient with an autoimmune disease always invites caution.     

Mesenteric panniculitis: case report and literature review

Mesenteric panniculitis is an extremely rare inflammatory condition of the adipose tissue of unknown etiology in which the mesentery is replaced with fibrosis. Knowledge of this rare syndrome should prevent any unwarranted aggressive therapy and help to use the clinical, radiological, and surgical sources to obtain the diagnosis. This paper is a review of symptomatology, pathology, treatment, and outcome of this disorder. A case report is described that presented with obstruction of the sigmoid colon.     

Hemophagocytic syndrome: pitfalls in its diagnosis

The hemophagocytic syndrome (HS) is characterized by a clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages. HS is considered rare and may be a primary disease or associated to viral, infection, neoplasias or autoimmune diseases. Treatment is controversial and its evolution is often fatal. Anatomo-pathological evaluation shows the phenomenon of hemophagocytosis in several organs, especially the hematopoietic tissues. We describe a case of HS, discuss its possible causes, its clinical and pathologic features, its pathophysiology and therapeutic possibilities.