1/6. Ectopic or heterotopic liver (choristoma) associated with the gallbladder.A 43-year-old man with a history and symptomatology of hereditary spherocytosis underwent splenectomy and cholecystectomy for treatment of his underlying disease and of cholelithiasis. The removed gallbladder showed a serosal encapsulated nodule, supported by a short mesentery, measuring 11 x 6 x 4 mm, that histologically was an ectopic or heterotopic functioning liver. This nodule did not have any connection with the main liver. It is because of the comparative rarity of this anatomic anomaly that we are presenting a new case; a simple classification of these aberrant liver tissues, which are found in the wall of the gallbladder; the embryological development; and a complete review of the literature. We also stress the need to restore the term choristoma, now in disuse.- - - - - - - - - - ranking = 1keywords = cholelithiasis (Clic here for more details about this article) |
2/6. cholelithiasis in children with congenital spherocytosis.cholelithiasis was diagnosed in two children with congenital spherocytosis of 7 and 9 years of age. Surgery was performed at age 10 and 14, respectively. The presence of gallstones should be considered in patients with congenital spherocytosis even under the age of 10. Various radiologic methods and ultrasonography are useful in the diagnosis of cholelithiasis in children as well as in adults. Once the diagnosis of cholelithiasis is established the treatment of choice is cholecystectomy and splenectomy at the same operation. To perform both procedures an upper midline incision proved to be satisfactory.- - - - - - - - - - ranking = 2keywords = cholelithiasis (Clic here for more details about this article) |
3/6. Monoclonal gammopathy in hereditary spherocytosis: a possible pathogenetic relation.Two cases of monoclonal gammopathy in patients with hereditary spherocytosis led us to consider the possible pathogenetic relation between these two disorders. Twelve adult patients with hereditary spherocytosis had significant hypergammaglobulinemia in comparison to normal subjects. Retrospective analysis of previous illness in 140 patients with multiple myeloma showed a significant association between IgA myeloma and previous gallbladder disease. We propose that the chronic reticuloendothelial stimulation due to extravascular hemolysis, possibly potentiated by the inflammation associated with cholelithiasis and cholecystitis, may foster neoplastic transformation of immunocytes in patients with hereditary spherocytosis, ultimately leading to the development of monoclonal gammopathy.- - - - - - - - - - ranking = 1keywords = cholelithiasis (Clic here for more details about this article) |
4/6. An autopsy case of hemochromatosis and hepatoma combined with hereditary spherocytosis.A case of hemochromatosis and hepatocellular carcinoma secondary to hereditary spherocytosis is very rare and this is only the second case reported in an English language bulletin. When the patient, a 56 years old man, was admitted for receiving cholecystectomy for cholelithiasis, a diagnosis of hemochromatosis secondary to hereditary spherocytosis was made by liver biopsy and hematological examination data. The patient did not receive a blood transfusion nor was administered iron during the entire duration of the illness. Hepatoma was suspected at the time of splenectomy which was performed in 1981 because of severe anemia. Eight months later he died of massive abdominal cavity bleeding and subsequent autopsy findings were consistent with the disease mentioned above. Clinical and postmortem examinations suggested that conspicuously enhanced erythropoiesis in the bone marrow and unknown factors may be responsible for an increase in iron absorption from the gut and in the amount of stored body iron, leading to the development of hemochromatosis.- - - - - - - - - - ranking = 1keywords = cholelithiasis (Clic here for more details about this article) |
5/6. Concomitant laparoscopic cholecystectomy and splenectomy for surgical management of hereditary spherocytosis.Laparoscopic splenectomy is rapidly becoming a common treatment modality in the surgical management of hematological processes involving the spleen. Hereditary spherocytosis is the most common red blood cell membrane disorder, and its diagnosis is often associated with hemolytic crisis and premature cholelithiasis. This condition has not been successfully treated laparoscopically until recently, and to our knowledge, the technique of concomitant laparoscopic splenectomy and cholecystectomy described here is the first reported in U.S. literature. Our patients, a 16-year-old 5-foot 3-inch-tall 90 pound emaciated albino, presented with cholelithiasis, splenomegaly, and anemia. Because of persistent anemia and gastrointestinal symptoms, the patient underwent laparoscopic cholecystectomy and splenectomy. The cholecystectomy was performed in a standard laparoscopic fashion. An additional 12-mm trocar was utilized for takedown of the spleen. The umbilical incision was extended to 4.5 cm, and the spleen was extracted manually. Total operative time was 12 hours. Examination demonstrated a 15 x 10 x 5-cm spleen, which weighed 350 grams. The gallbladder microscopically showed cholecystitis and had several stones. In conclusion, we present a combined laparoscopic cholecystectomy and splenectomy for hereditary spherocytosis associated with splenomegaly, cholelithiasis, and cholecystitis.- - - - - - - - - - ranking = 3keywords = cholelithiasis (Clic here for more details about this article) |
6/6. Acute intravascular haemolysis with massive microspherocytosis in a 75-year-old woman.Acute intravascular haemolysis (AIH) sometimes occurs in patients with sepsis or bacteraemia, mainly due to clostridia or salmonella sp., and may be a life-threatening condition. We describe a case of AIH in a 75-yr-old woman with chronic cholelithiasis. Blood and stool cultures were repeatedly negative, but the massive microspherocytosis, typically observed in clostridia infections, oriented our diagnosis. The patient was treated with antibiotics and for a rapid worsening of her conditions, which could have led to the onset of a multi-organ dysfunction syndrome (MODS), with plasma exchange and, subsequently, haemodialysis, with satisfactory results.- - - - - - - - - - ranking = 1keywords = cholelithiasis (Clic here for more details about this article) |