Cases reported "Spherocytosis, Hereditary"

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1/14. Endobiliary endoprosthesis without sphincterotomy for the treatment of biliary leakage.

    Endoscopic retrograde cholangiopancreatography with biliary drainage is an effective therapeutic tool in the management of bile duct injuries associated with laparoscopic cholecystectomy. Placement of a stent or a nasobiliary drain in the common bile duct, or biliary sphincterotomy, is an effective treatment for bile leaks and obviates the need for otherwise complex biliary tract surgery. Although there are no controlled comparative trials, placement of a 7-, 8.5-, or 10-Fr biliary stent without sphincterotomy may cause the least morbidity and be the most comfortable nonoperative management option. We report a child who presented with a bile leak that occurred after laparoscopic cholecystectomy and was successfully treated with the placement of a biliary stent without sphincterotomy. To our knowledge, this is the second pediatric case of a bile leak successfully treated by endoprosthesis placement without sphincterotomy.
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2/14. Postoperative pulmonary embolism in a young female accompanying with factor v Leiden mutation and hereditary sypherocytosis.

    A 20 year-old female, heterozygous for factor v Leiden mutation (FVLM) is presented. Her personal history was prominent for severe anaemia during her gestation. Aetiology of anaemia was found to be hereditary spherocytosis (HS). Intrauterine foetal death had occurred at 20 weeks of gestational age. Two days after curettage, she developed pulmonary embolism (PE). This is an unusual case of pulmonary embolism and intrauterine foetal death coexisting with FVLM and/or HS. We present the case so that a general practitioner or haematologist can hardly see such cases in daily practice. Hence, a young female with PE should be screened for hypercoagulable states including FVLM or HS.
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ranking = 4
keywords = operative
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3/14. A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis.

    BACKGROUND: Partial splenectomy is sometimes used for children with hereditary spherocytosis (HS) to reduce hemolysis while retaining some splenic immune function. Previous reports have described a partial splenic resection through a laparotomy incision. Whereas laparoscopic total splenectomy for HS is well-established, laparoscopic partial splenectomy (LPS) has not been described. The authors have developed a novel LPS technique that combines the benefits of partial splenectomy with those of a laparoscopic approach. methods: A chart review was conducted for three children with HS who underwent LPS, with approximately one-fourth of the spleen left on the basis of the short gastric arterial supply. RESULTS: The mean preoperative spleen size was 17.6 cm. The mean preoperative hemoglobin count was 100 g/l, and the postoperative hemoglobin count was 133 g/l. All three patients reported reduced malaise and increased energy levels. There was no recurrent anemia at the 1- to 2-year follow-up evaluation. CONCLUSION: The LPS procedure is a safe and effective approach to HS that resolves anemia, potentially retains some splenic immunity, and confers the benefits of a minimal access technique.
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ranking = 3
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4/14. Open heart operation in a child with congenital heart disease and hereditary spherocytosis.

    An 18-month-old girl with hereditary spherocytosis underwent closure of the ventricular septal defect, commissurotomy of the pulmonary valve, and patch angioplasty of the pulmonary trunk without previous splenectomy. No serious complications as a result of hemolysis occurred in the perioperative period. Open heart surgery can therefore be safely performed in young children with congenital heart disease and hereditary spherocytosis who have not previously undergone splenectomy.
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keywords = operative
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5/14. Preoperative computed tomography and scintigraphy to facilitate the detection of accessory spleen in patients with hematologic disorders.

    Accessory spleens of 1.5 cm in size were preoperatively identified by the combined use of computerized tomography and splenic scintigraphy in two patients with hematologic diseases. After the accessory spleen had been removed from the first patient, who had persistent hereditary spherocytosis and had undergone a splenectomy 15 months before, a postoperative decrease in hyperbilirubinemia was noted. In the other patient who had idiopathic thrombocytopenic purpura, a successful accessory splenectomy was done at the same time as her splenectomy, and was followed by 6 months' complete remission. These events indicate that preoperative investigations using computerized tomography and scintigraphy are indispensable for ruling out an accessory spleen in those patients for whom splenectomy needs to be done in order to alleviate hematologic disorders.
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ranking = 7
keywords = operative
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6/14. Extreme hyperbilirubinemia in a patient with hereditary spherocytosis, Gilbert's syndrome, and obstructive jaundice.

    hyperbilirubinemia may be of several etiologies in the individual patient. An 18-year-old man presented with extreme hyperbilirubinemia (direct bilirubin 23.0 mg/dl, total bilirubin 60.0 mg/dl), hepatosplenomegaly, and anemia. Hematologic studies prelaparotomy documented the presence of hereditary spherocytosis. Intraoperative liver biopsy revealed moderately reduced levels of glucuronyl transferase activity (Gilbert's syndrome). common bile duct obstruction secondary to choledocholithiasis was found, and a cholecystectomy and splenectomy were performed. This case stresses the potential confusion among several diseases which may present with hyperbilirubinemia.
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keywords = operative
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7/14. Isolated weakness of the extensor hallucis longus in a patient with hereditary spherocytosis.

    A 24-year-old woman with a history of hereditary spherocytosis and oral contraceptive use presented with a spontaneous, isolated loss of great toe extension. She had clinical and electrodiagnostic evidence of a localized lesion affecting the nerve supply to the extensor hallucis longus (EHL). Full clinical recovery was apparent within 6 months with nonoperative therapy. Isolated EHL weakness may occur as a rare neurologic complication of procedures such as high tibial osteotomies, but, to our knowledge, this has not been reported in the absence of trauma or surgery. While the coexistence of hereditary spherocytosis and a peripheral neuropathy may have been purely coincidental, it is plausible that the isolated lesion in this patient was due to ischemic changes from underlying rheologic conditions, possibly in association with a variation in the neural or nutrient vessel supply to the EHL.
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keywords = operative
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8/14. Laparoscopic splenectomy for massive splenomegaly: operative technique and case report.

    Although laparoscopic splenectomy is the preferred technique for the elective removal of normal-sized and moderately enlarged spleens, its value in the management of massive splenomegaly has not been defined. A 33-year-old woman with massive splenomegaly was managed by laparoscopic splenectomy. The splenic vessels were embolized preoperatively to reduce operative blood loss. Laparoscopic surgery involved dissection of the suspensory ligaments at the lower pole of the spleen followed by dissection and ligation of all splenic branches near the splenic parenchyma. The operative field was explored manually through a 10-cm long Pfannenstiel incision at the public hairline, and under videoscopic control the remaining structures were safely secured. The spleen was delivered into the pelvis, fragmented and removed in large pieces. The incisions were closed after proper irrigation and laparoscopic inspection of the operative field. Further clinical evaluation is required to determine if the procedure is more efficacious than the open technique for massive splenomegaly.
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ranking = 8
keywords = operative
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9/14. Laparoscopic splenectomy for congenital spherocytosis with splenomegaly: a case report.

    The traditional method of splenectomy involves a 7- to 10-cm abdominal incision, with its attendant morbidity that includes sepsis. Recently, the laparoscopic technique has been adopted for splenectomy. Many surgeons still believe that laparoscopic splenectomy should be carried out only for idiopathic thrombocytopenia purpura and Hodgkin's disease with a normal-sized spleen. A 16-year-old girl with marked splenomegaly (spleen size for times normal and weighing 600 g) due to congenital spherocytosis underwent laparoscopic splenectomy and retrieval of the spleen through the umbilical trocar site. Preoperative arterial embolization was not used, and the operative blood loss was estimated to be only 250 mL. Her postoperative course was uncomplicated, and she was discharged on the 4th postoperative day. The operative technique is described and the advantages of laparoscopic splenectomy are discussed.
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ranking = 5
keywords = operative
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10/14. Open heart operation in a young child with spherocytosis.

    Open heart operations on patients with hereditary spherocytosis have been reported rarely. Young children who have not yet undergone splenectomy have a high risk of intraoperative hemolysis because of the heart-lung machine and secondary renal failure. We report the case of a 15-month-old child with spherocytosis who underwent an open heart operation without serious complications as a result of careful and appropriate perioperative management.
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ranking = 2
keywords = operative
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