Successful Surgery in a Patient With Haemophilia B. Report of a Case With Abdominal Aneurysm

Abstract

Modern development in physiopathology, diagnosis and medical treatment of haemocoagulative syndrome, allows to operate electively upon various organs and apparatus.3. 4. 8 Quite numerous appear in literature, particularly, the references regarding surgical operations,. performed in haemophilics. The availability of adequate preparations which replace the lacking factors of the coagulation, in fact, allows in such patient to arrive to a normal haemostasis as long as the length of the operation and during the post-operative period, with very low operative risk. In literature there is only one case2 regarding surgical intervention on haemophilics on cardiorespiratory apparatus. We think it worthwhile to report a case of voluminous abdominal aneurysm in an haemophilia B patient with good result. CASE REPORTThe patient was a 55-year-old male. Since childhood he noted the appearance of post-traumatic hematomas and haemorrhagic episodes of high intensity and duration during dental avulsions. In November 1970 he suffered of acute posteroinferior myocardial infarction. During 1971 the patient noted a pulsatile tumefaction in the mesogastric region. He was hospitalized in May 1971.On admission his general conditions were good. The blood pressure was 160/ 80 mmHg, the pulse 80, rhythmical, and the respirations were 16 for minute. The abdomen was irregularly globous due to the presence in the mesogastric region of a voluminous pulsatile tumefaction about 10 X 8 cm in diameter. The palpation confirmed the speculative character of the mass and, moreover, permitted to appreciate the smooth, regular surface of it, the elastic consistency and the reduced motion. The mass presented a typical expansive pulsation, with small retard on the peripheral pulses. A systolic murmur was heard on the tumefaction. The pheripheral pulses were slightly reduced bilaterally, especially on the left. The haemogenic tests revealed a haemocoagulative defect originated from lack of factor IX or haemophilia B. The abdominal roentgenogram showed an opacity of hemispheric form, on the right at the median line, immediately under the corresponding renal image, and extending from the II to V lumbar space, without calcifications, with pulsation synchronous with the arterial pulse. The electrocardiogram showed signs of old postero-inferior my-

Endovascular Abdominal Aortic Aneurysm Repair in a Patient With Severe Hemophilia B

“…To our knowledge, no patient with severe hemophilia has been treated with EVAR; only 4 other AAA patients, 3 with hemophilia A 3-5 and 1 with hemophilia B, 6 are reported in the literature to have been successfully treated with conventional surgery. All of them, however, had a moderate or mild form of the disease.…”

Endovascular Abdominal Aortic Aneurysm Repair in a mild Haemophilia A Patient: a Case Report

“…This figure must be compared with total blood loss estimated at 1300 and 1700 mL in haemophilia patients following surgical graft replacement [5,7]. A distinctive feature pertaining to our patient was the short duration of hospitalization, namely, 6 days in our case versus 18, 17 and 20 days in other haemophilia cases [5,7,8]. Another positive issue to focus on is the cost-saving aspect of EVAR, as the amount of recombinant FVIII units to be administered to haemophilia patients during this procedure is significantly less than in standard surgery.…”

“…To our knowledge, five other haemophilic patients treated with open surgery or EVAR were reported in the literature: one with severe haemophilia B, three with, respectively, moderate, mild and severe haemophilia A, and the remaining one with haemophilia B (severity not reported) [4][5][6][7][8]. In patients with normal coagulation patterns, open abdominal aortic aneurysm (AAA) surgical repair usually leads to major intraoperative blood loss, whereas EVAR is associated with a significant reduction in blood loss and transfusion requirement.…”

Management of a Giant Renal Artery Aneurysm in a Patient with Severe Hemophilia A

“…The aim was to preserve as much renal parenchyma as possible, taking into account the possibilities to preserve the accessory artery to the upper pole of the right kidney. Endovascular treatment is associated with a significant reduction of blood loss during minimally-invasive operations, with reduction in the requirement for blood transfusions and shorter hospital stay [ 15 – 17 ]. With the development of endovascular treatment technics, embolization of RAAs has become the method of choice for treating RAAs, with the preservation of renal parenchyma, as described by Gutta et al [ 8 ].…”