Risks and challenges of orthopaedic invasive interventions in haemo -philia in a low-resource country

Abstract

SummaryHaemophilic arthropathy is a defining feature and a debilitating condition of persons with haemophilia (PwH) in low resource countries. Orthopaedic surgery is unavoidable for patients with high occurrence of joint damage. Aims: We aimed to evaluate the spectrum and outcome of invasive orthopaedic therapies in PwH and von Willebrand diseases (VWD). Patients and methods: Our descriptive observational retrospective study included 131 invasive surgical procedures, performed on 76 consecutive patients, most of them (93.4%) with severe disease, treated in Timisoara's Haemophilia Center over a period of 12 years; 17.1% had pre-operation anti-FVIII inhibitors. Invasive elective procedures were predominant (90.8%) as compared to emergency measures (9.2%); according to their invasiveness, 20.6% of interventions were major, 44.3% intermediate and 35.1% minor. Results were good in the majority of cases; significantly reduced joint bleed rate and pain score were the most consistent achievements. The greatest proportion of complications occurred after major (66.7%), compared to moderate (25.6%) and minor (7.7%) interventions. The main threatening complication was the development (3.8%) or increase (4.6%) of inhibitor titer. Local bacterial infections and wound dehiscence complicated the evolution in 4.6% and 0.8 % of cases, respectively; we noticed no blood-borne infections or thrombotic accidents. Low dosage (10.7%) and short duration of substitution (21.4%) led to increased post-surgical bleeding and post-haemorrhagic anaemia. Conclusions: Surgery is a highly demanding intervention in haemophilia, which cannot be ignored in a low resource country. It represents a life or limb-saving and quality of life-improving measure.

Major and Minor Classifications for Surgery in People With Hemophilia: A Literature Review

“…10,11 Articles That Defined Major and Minor Surgery Seven (20%) publications provided formal prespecified definitions for major or minor surgery (Table 1). 8,[10][11][12][13][14][15] Five of these publications used the level of surgical invasiveness as a defining factor for major surgery, 8,10,[13][14][15] with criteria including: a requirement for a general or spinal anesthetic, provision of respiratory assistance, abdominal surgery, penetration of a major body cavity, orthopedic surgery (joints), and extraction of more than 3 teeth. One publication defined major surgery as any procedure estimated to require daily treatment with FVIII concentrate for at least a week 11 with preoperative target FVIII: C levels of 0.5 to 1.0 and 0.2 to 0.5 IU/mL for major and minor surgery, respectively.…”

Management of severe perioperative bleeding

“…However, recent data demonstrated that good surgical results are achievable over a range of procedures when there is appropriate careful preoperative planning, appropriate replacement/ substitution therapy, and multidisciplinary team management. [501][502][503][504][505][506] Although surgery is a highly demanding intervention in patients with severe IBDs, especially in low-resource countries, 507 it often represents a life or limb-saving and quality of life-improving measure, which has to be taken.…”

A Perspective on the Quality of Life of Hemophilia A Patients in Romania—A Study on 100 Patients

“…The functional deficits of the patients were remarkably good, and better than expected, but those who did have a moderate and severe deficit did not have orthopedic interventions. This may be due to the lack of comprehensive treatment centers, and the lack of orthopedic surgeons qualified for these types of patients in the majority of medical centers, or it could have been because the patients did not require the procedure [15].…”