Surgery--a challenge in haemophiliacs with inhibitors.

UNLABELLED: Treatment of haemophiliacs with inhibitors is of great concern in low-income countries confronting shortage in substitutive treatment. Invasive interventions on these patients represent a major challenge due to the fact that costs are significantly higher in comparison to similar procedures conducted on patients without inhibitors. OBJECTIVE: In the context of insufficient availability of clotting factor, we aimed at highlighting the experience of surgical treatment in inhibitor patients. We analyzed the indications, types of performed interventions and outcomes. PATIENTS, METHODS: This single center, retrospective analysis has been conducted on 7 inhibitor patients registered and treated in Haemophilia Center of Timisoara over ten years (1997-2007): six patients with severe hemophilia A (3 - high titer, 3 - low titer), one patient with von Willebrand disease (low titer).Three patients developed inhibitors only after 2-5 days post surgery. RESULTS: A total of 15 invasive procedures were carried out: 2 orthopedic interventions (1 arthrodesis, 1 arthroscopic synovectomy), 2 urogenital interventions (1 surgical testicular detorsion, 1 orchiectomy), 4 limb amputations (2 bilateral upper and 2 lower limb amputation), 2 pseudotumour (PT) surgery interventions, 5 drainages (2 massive pyohaemothorax, 1 drainage of shank haematoma, 1 drainage of compressive forearm haematoma, 1 drainage of thigh haematoma). Haemostasis was achieved in patients with low level inhibitors (< 5 BU/ml) with high doses of FVIII concentrates; in those with high inhibitor level (> 5 BU/ml), surgery was managed using by-passing agents. Supplementation with local fibrin glue and intravenous or local antifibrinolytic agents was given in 68.75% of interventions. Postoperative complications consisted of haemorrhagic shock in 13.33% of interventions and infection in 6.66%. Haemostatic outcome was evaluated by blood loss and duration of treatment, compared to expectations for non-inhibitor patients. The outcome was excellent and good in 66.66% of interventions, and fair in 33.33%. Discussion, conclusion: Indication of invasive procedures in haemophiliacs with inhibitors was limited to life and/or limb-threatening situations. In low-income countries, inhibitor and recovery of FVIII monitoring is mandatory in the postoperative follow-up of patients with low or no substitution prior to surgery due to false negative results at the preoperative investigation.

Orthopedic approach of haemophiliacs. A single center experience in Romania.

Clinical expression of inadequately treated haemophilia is dominated by orthopedic complications, requiring invasive or non-invasive interventions. OBJECTIVE: In Romania, with under dosed and late introduced "on demand" substitution, we aimed at highlighting the experience of orthopedic treatment and its outcome. PATIENTS, METHODS: Single center retrospective analysis regarding orthopedic interventions and their outcomes was conducted on 59 hemophilia A, B, and von Willebrand disease patients, between 2002 and 2007. RESULTS: The majority of interventions, invasive (60.71%) and non-invasive (39.28%), were elective, only two being emergencies. Postoperative functional evolution after synovectomies was good in 68.28%, fair in 24.39%, satisfactory in 7.31%. Results of 33 non-invasive (extensive releases) procedures were very good in 27.27%, good in 63.63%, poor in 9.09%. DISCUSSION, CONCLUSIONS: The important number and complexity of orthopedic interventions are proving the precarious musculoskeletal state in persons with hemophilia, demonstrating the need of improving substitution, at least with discontinue prophylaxis in patients with severe forms.