Cases reported "Postoperative Hemorrhage"

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1/13. Surreptitious bleeding in surgery: a major challenge in coagulation.

    Apart from inadequate surgical haemostasis, postoperative bleeding can be related to acquired disorders of platelet number, platelet function or coagulation proteins (e.g. vitamin k deficiency, DIC or liver injury). We highlight our experience with three patients who suffered life-threatening bleeding in the postoperative setting. The three patients - a 47-year-old man and 70- and 74-year-old women -- all had negative histories for excessive bleeding with prior surgeries, and all had normal preoperative PT and aPTT tests. Surgeries were resection of ischaemic bowel, cholecystectomy and coronary artery bypass grafting. All patients experienced unexpected bleeding within the first few postoperative days requiring multiple red cell transfusions and surgical re-explorations. Evaluations within the first 4--7 days after surgery revealed that these three patients had developed prolonged aPTT due to demonstrable factor viii antibodies initially at low titre. One patient was treated with high doses human factor viii, corticosteroids, intravenous gammaglobulin and plasma exchanges. The inhibitor was no longer demonstrable after 6 weeks of such therapy, and he has remained in remission without therapy. The second patient was initially treated with high-dose human factor viii infusions. Five months later, prednisone and 6-mercaptopurine were begun for worsening inhibitor titre and diffuse purpura and subcutaneous haematomas. The factor inhibitor remitted, but the patient died from liver failure related to post-transfusion hepatitis. The third patient was initially managed with high-dose human factor viii. Two months later, worsening inhibitor titre and tongue haematoma was treated with activated prothrombin complex, corticosteroids and cyclophosphamide. Eight years later, she is on no therapy, demonstrates a mild bleeding tendency and has a stable low-titre inhibitor. There have been a few case reports of inhibitors to coagulation factors including factor viii becoming manifest in the postoperative setting but surgery has not been widely recognized as an underlying cause for acquired haemophilia. This paper speculates on pathogenesis and reviews treatment options. This syndrome is remarkable for its abrupt onset in the first few postoperative days and for its substantial morbidity. The problem is potentially reversible with immunosuppressive therapy. Clinicians should be aware of this syndrome, considering acquired haemophilia in patients with unexpected postoperative bleeding.
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2/13. Successful use of recombinant factor viia for hemostasis during total knee replacement in a severe hemophiliac with high-titer factor viii inhibitor.

    A 32-year-old male patient with severe factor viii (FVIII) deficiency had developed a high-titer FVIII inhibitor at age 13. Recurrent hemarthroses caused bony destruction in both knees, significantly impairing his ability to walk. knee examination revealed 20 degrees of varus, destruction of the medial joint line, and flexion contracture. Total knee arthroplasty was performed using recombinant factor viia (rFVIIa, NovoSeven) for hemostatic control. rFVIIa (85 microg/kg given intravenously over 3-5 minutes) was given just prior to surgery. The dose was repeated every 2 hours during and for the first 48 hours after surgery. When the tourniquet was removed, rFVIIa had not been infused for 1.5 hours, and significant hemorrhage was noted. The hemorrhage responded promptly to rFVIIa infusion. The infusion interval was extended to every 4 hours for an additional 48 hours, and subsequent doses were given every 6 hours until the patient returned to the clinic 2 days postdischarge. Hemoglobin levels dropped from 16.9 gm/dL on admission to 9.1 gm/dL at discharge. After 2 months, the patient returned to work. We recommend that tourniquet release be performed immediately after rFVIIa administration and that aggressive physical therapy be considered in the early postoperative period when rFVIIa infusions are frequent.
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3/13. Unusual presentation of factor xiii deficiency.

    factor xiii deficiency is a rare inherited bleeding disorder that is often difficult to diagnose. The standard screening tests are normal in these patients and their bleeding phenotype may be variable. We report the case of a 3-year-old girl who presented with an intracranial haemorrhage. Several confounding factors, such as the suspicion of an arteriovenous malformation and the development of a deep venous thrombosis, led to a delay in the diagnosis of factor xiii deficiency. Subsequently, her brother was also found to have severe factor xiii deficiency. This case highlights the importance of a detailed history and of screening families in which index cases have been identified. It should also remind physicians that bleeding disorders may have unusual presentations and should be sought when investigating unexplained bleeding.
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keywords = deficiency
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4/13. Variable response of Hermansky-Pudlak syndrome to prophylactic administration of 1-desamino 8D-arginine in subsequent pregnancies.

    Hermansky-Pudlak syndrome is characterised by oculocutaneous albinism and haemorrhagic diathesis. The bleeding tendency that is associated with this autosomal recessive disease is caused by storage-pool deficiency and has been reported to be controllable by prophylactic administration of 1-desamino 8D-arginine (desmopressin, DDAVP). The DDAVP prophylaxis at the first delivery of our patient did not prevent the severe haemorrhagic sequeal requiring transfusion of packed red cells and platelets, but the same preventive measure was successful at her second childbirth. Response to prophylactic DDAVP administration varies between as well as within patients with Hermansky-Pudlak syndrome.
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ranking = 1
keywords = deficiency
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5/13. Adenotonsillectomy in children with von Willebrand's disease: how and when. A case report with review of the literature.

    The presence of coagulation pathology in children who are candidates for adenotonsillectomy (AT) is a challenge to the otolaryngologist. von Willebrand's disease (vWD) is the most common hereditary coagulopathy and is due to a quantitative and/or qualitative deficiency of von Willebrand's factor (vWF). In recent years, the administration of 1-deamino-8-D-arginine vasopressin (DDAVP) has been recommended as coadjuvant therapy for surgical procedure. This synthetic hormone promotes the release of vWF and factor viii from endothelial cells. In this report, the authors describe the history of a child with vWD undergoing successful AT after administration of DDAVP. Furthermore, a review of the literature with particular emphasis on the use of DDAVP is made.
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keywords = deficiency
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6/13. Initial report of retroperitoneoscopic renal surgery in a patient with factor viii deficiency (classic hemophilia).

    Bleeding disorders are generally considered absolute contraindications to laparoscopic surgery. We believe laparoscopic/retroperitoneoscopic renal surgery is an optimal choice in patients with hemophilia, because it minimizes tissue trauma. We present a case of successful retroperitoneoscopic renal surgery in a patient with factor viii deficiency with a perinephric abscess that failed less invasive treatments. A fully equipped coagulation laboratory, experienced hematologists, and an experienced laparoscopic surgeon play a large role in the outcome in patients with bleeding disorders undergoing minimally invasive procedures. We conclude that although uncorrected bleeding disorders are absolute contraindications for surgical procedures, those that are corrected and appropriately monitored are not.
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ranking = 5
keywords = deficiency
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7/13. Delayed hemorrhage after cervical conization unmasking severe factor xi deficiency.

    BACKGROUND: factor xi deficiency, a rare bleeding disorder found most commonly in patients of Ashkenazi Jewish background, may be present in patients with a history of abnormal bleeding after elective surgery. CASE: A patient of Ashkenazi Jewish descent presented 12 days after cervical conization for adenocarcinoma in situ with severe vaginal bleeding requiring multiple transfusions and uterine artery embolization. After a thorough workup, a severe factor xi deficiency was found. The patient ultimately required modified radical hysterectomy for treatment of early cervical cancer. With appropriate perioperative management, the patient underwent abdominal surgery without further bleeding complications. CONCLUSION: factor xi deficiency can present with severe bleeding episodes after elective surgery. Adequate preoperative assessment and perioperative management are necessary to prevent bleeding complications in these patients.
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ranking = 7
keywords = deficiency
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8/13. Bleeding prophylaxis in a child with cleft palate and factor vii deficiency: a case report.

    The association between factor vii deficiency and cleft palate has never been described. The case of a child with cleft palate and factor vii deficiency who successfully underwent palatoplasty is described in this article. To allow surgical treatment, through maintenance of a normal prothrombin time, the patient was given 15 microg/kg of recombinant factor viia every 12 hours, starting 20 minutes before surgery and ending the third postoperative day. No abnormal perioperative bleeding was observed. Use of recombinant factor viia in bleeding prophylaxis or treatment is widespread. Doses are much higher in these cases. The reduction of dosage allows easier administration, especially in pediatric patients, without affecting drug efficacy.
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ranking = 6
keywords = deficiency
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9/13. factor xi deficiency in an Ashkenazi Jewish child, causing severe postoperative hemorrhage.

    Although inherited coagulation factor deficiencies with negative bleeding histories are rare, cases such as the one presented herein may not be diagnosed at the time of surgery and may experience severe hemostatic problems postoperatively. It may be prudent to obtain an activated partial thromboplastin time preoperatively for all Ashkenazi jews, including those with a negative history for a hemostatic disorder, because of the high incidence of factor xi deficiency in their population. Surgeons and anesthesiologists should be aware of the existence of this and other uncommon hemostatic disorders and their treatment when faced with serious, apparently unexplained postoperative hemorrhage.
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ranking = 5
keywords = deficiency
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10/13. amoxicillin-related postextraction bleeding in an anticoagulated patient with tranexamic acid rinses.

    This report describes a patient who had significant postoperative bleeding 4 days after undergoing surgery while using a tranexamic acid (4.8%) mouth rinse protocol for local control of hemostatis. patients undergoing dentoalveolar surgery who are receiving chronic oral anticoagulants are treated with a tranexamic acid mouth rinse at our hospital. No systemic modification of their coagulation status is attempted. The postoperative bleeding problem that developed was determined to be caused by an antibiotic-induced vitamin k deficiency rather than a failure of the tranexamic acid protocol.
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ranking = 1
keywords = deficiency
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