General Surgery in Haemophiliac Patients Postgrad Med J: First Published As 10.1136/Pmj.76.898.494 on 1 August 2000

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494 Postgrad Med J 2000;76:494–495 General surgery in haemophiliac patients Postgrad Med J: first published as 10.1136/pmj.76.898.494 on 1 August 2000. Downloaded from

Elias Bastounis, Emmanouil Pikoulis, Ari Leppäniemi, Dimitrios Alexiou, Christos Tsigris, Andreas Tsetis

Abstract ease, the possible presence of inhibitory factors With improvements in medical technol- to factor VIII and IX, as well as antierythrocyte ogy, more and larger surgical procedures antibodies. In addition, liver function tests were are performed in haemophiliac patients, performed. The levels of factor deficiencies in but rarely reported in the surgical litera- the diVerent disease groups are presented in ture. A retrospective study from a 10 year table 2. period from one referral centre identified The replacement therapy was conducted a total of 68 operations performed in immediately before the operation, and the dose haemophiliac patients. The levels of the required was calculated using the following defective factors were carefully monitored formula: weight (kg) × desired factor level (%) preoperatively and postoperatively, and × 0.5. Twenty minutes after replacement replaced according to a standard formula. therapy, the factor level was rechecked, and Special caution was taken to avoid any additional doses of the missing factor were postoperative medication or procedures given intraoperatively, as needed. that could provoke haemorrhagic compli- At operation, the aim was to achieve meticu- cations. lous haemostasis using ligation for all visible Two patients suVered postoperative bleeding and cautery only for capillary bleed- bleeding complications that were man- ing, and also trying to keep the operative time aged conservatively. Inhibitory factors to a minimum. Creation of closed spaces was were detected preoperatively in one case, avoided and pressure bandages were used in and postoperatively in another, and were patients with soft tissue injuries. managed with aggressive replacement Postoperatively, replacement therapy was therapy. There was no mortality, and the continued every eight hours for haemophilia A, overall morbidity rate was 6%. and every 12 hours for haemophilia B, with With adequate preoperative and post- daily blood measurements to maintain the fac- operative monitoring of the clotting factor level above 30% for 2–3 weeks. The interval tors, meticulous haemostasis during and dose of the postoperative replacement surgery, careful postoperative nursing, therapy were similar in patients undergoing and timely replacement therapy, haemo- major or minor surgery for the first three days philiac patients can be operated with good but the interval was longer, the dose smaller,

results. Postoperative bleeding complica- and the duration shorter in the latter group http://pmj.bmj.com/ tions are rare, and usually amenable to during subsequent days. Analgesic drugs af- conservative management. fecting the clotting mechanism and unneces- (Postgrad Med J 2000;76:494–495) Table 1 Surgical procedures performed in haemophiliac Keywords: surgery; haemophilia; factor VIII concen- patients trates; von Willebrand’s disease Surgical procedure No of patients

Abdominal on September 30, 2021 by guest. Protected copyright. A better understanding of the clotting mecha- Inguinal herniorrhaphy 16 Gastrectomy 14 nisms and the ability to manufacture factor Cholecystectomy 7 concentrates have enabled more and larger Appendicectomy 3 surgical procedures to be performed in haemo- Hemicolectomy 2 Splenectomy 2 philiac patients. This study reviews the experi- Miscellaneous 4* ence of surgical treatment of haemophiliac Excision or incision of cysts and abscesses patients managed in one referral hospital with Pilonidal cyst 8 1st Surgical Perianal abscess or ﬁstula 5 Department, special experience in haematological problems. Thyroglossal duct cyst 1 University of Athens, Extremity procedure Athens, Greece Patients and methods Wound excision for severe soft tissue injury 4 Above knee amputation 1 E Bastounis During a 10 year period, from 1985 to 1994, a Excision of a soft tissue tumor 1 E Pikoulis total of 65 haemophiliac patients underwent Total 68 A Leppäniemi surgical treatment in the 1st Surgical Depart- D Alexiou *Vagotomy and pyloroplasty, small bowel resection, adhesioly- C Tsigris ment of the University of Athens. Their mean sis, ventral hernia repair. A Tsetis (range) age was 42 (8–72) years, and 62 (95%) were male. Three patients were operated twice Table 2 The severity (factor level as % of normal) of factor deﬁciency in 65 hemophiliac patients Correspondence and reprint for diVerent indications. Forty three patients requests to: Dr Emmanouil Pikoulis, Artemidos 28 Str, (66%) had haemophilia A, 15 (23%) had hae- Haemophilia Haemophilia von Willebrand’s Paleo Faliro, 17561 Athens, mophilia B, and seven patients (11%) had von Severity A (n=43) B (n=15) disease (n=7) Greece Willebrand’s disease. The type of surgical pro- Severe (0%–2%) 16 9 — cedures performed are listed in table 1. Moderate (2%–10%) 11 3 3 Submitted 16 December Before operation, laboratory tests were Mild (10%–20%) 13 1 3 1998 Latent (>21%) 3 2 1 Accepted 13 January 2000 performed to determine the severity of the dis-

www.postgradmedj.com General surgery in haemophiliac patients 495 Postgrad Med J: first published as 10.1136/pmj.76.898.494 on 1 August 2000. Downloaded from sary moving of the patients were avoided. The bandages were changed only when necessary, Learning points and after supplying the missing factor. Sutures x Adequate preoperative and postoperative were removed after 2–3 weeks, and always after monitoring of the clotting factors. factor replacement. x Meticulous haemostasis during surgery. Results x Careful postoperative nursing. Four patients had postoperative complications. x Timely replacement therapy. The ﬁrst patient underwent a cholecystectomy complicated by intraoperative haemorrhage from the gall bladder bed which continued for 12 days. Blood and fresh frozen plasma were or inhibitors, which have been demonstrated in given intraoperatively and postoperatively com- 8%–20% of patients with severe haemophilia A bined with factor replacement therapy, and the and in 2.5%–16% of patients with severe patient was discharged, although he had to be hemophilia B.56 Inhibitors develop usually at reoperated on three years later for obstructive an early age after few infusions of factor VIII or common bile duct stone in another hospital. factor IX, and the treatment of these patients The second patient developed a haematoma aims at controlling episodes of severe acute after wound excision for soft tissue injury and bleeding, preparing patients for essential sur- was treated successfully non-operatively with gery, and to induce immune tolerance by blood transfusions and replacement therapy. elimination of the antibodies.6 In our study, The third patient who had undergone sub- only one patient produced inhibitory factors total gastrectomy two years before for gastric postoperatively, and was managed successfully haemorrhage underwent a total gastrectomy with additional replacement therapy. In one of for Zollinger-Ellison syndrome, and developed our patients with history of gastric bleeding, a postoperative subphrenic abscess managed high levels of inhibitor factor VIII were successfully with percutaneous drainage. detected preoperatively and managed success- The fourth patient underwent a successful fully with augmented replacement therapy. In cholecystectomy and was discovered postop- general, if the patient has an inhibitor to factor eratively to have an inhibitor to factor VIII, VIII or IX, we have used either FVIII porcine which was managed with additional replace- or aPCC (Autoplex), although during the last ment therapy using aPCC (Autoplex) with no two years in patients with inhibitors as well as further complications. In addition, in one in patients with acquired hemophilia, we have patient an inhibitor to factor VIII was detected used recombinant FVIIa with very encourag- preoperatively and managed successfully by ing results. However, in cases where the risk of inducing supranormal levels of clotting factors bleeding is clearly increased, the postponement with aggressive replacement therapy. of the elective procedure has been suggested.7 Postoperative thrombophlebitis has been re- Discussion ported in the literature,7 but was not detected Until the 1960s, haemophilia patients under- in this series. In addition, there were no cases of http://pmj.bmj.com/ went only emergency surgical treatment and allergic reactions to the infused factor, or there were high mortality rates of 25%–50%.1 haemolytic reactions caused by antierythrocyte After the introduction of the use of part I of antibodies. plasma (fraction I) in 1960, the mortality decreased below 10%, and further below 2% 2 1 Craddock CG Jr, Fenniger LD, Simmons B. Hemophilia with the use of cryoprecipitate. After the problem of surgical interventions for accompanying dis- eases: review of the literature and a report of case. development of a highly active factor VIII and Ann Surg on September 30, 2021 by guest. Protected copyright. 1980;128:888–904. IX concentrates, the mortality rate fell below 2 Pudowski WJ. Major surgery in haemophilia. Ann R Coll 1%, and enabled the scheduling of increasingly Surg Engl 1981;63:111–17. 3 George JN, Brickenbridge RT. The use of factor VIII and complex elective cardiovascular and neurosur- factor IX concentrates during surgery. JAMA 1970;214: gical procedures.3 1673–6. 4 Rudauski WJ, Schorf R, Ziemski JM. Is major surgery in In haemophiliac patients, the reported post- hemophiliac patients safe? World J Surg 1987;11:378–86. operative bleeding complication rate is 3%– 5 Shapiro SS, Hultin M. Acquired inhibitors to the blood coagulation factors. Semin Thromb Hemost 1975;1:336. 6%, and the postoperative infection rate about 6 Nilsson IM. Factors VIII/IX andibodies. In: Nilsson IM, ed. 4 1%, conﬁrmed also in this study. Hemophilia. Pharmacia Plasma Products, 1994: 58. One of the complications in patients with 7 Kasper CK, Boylen AC, Ewing NP, et al. Haematologic management of hemophilia after surgery. JAMA 1985;253: haemophilia is the development of antibodies 1279–83.