title: Orthopedic procedures in patients with congenital coagulation disorders: single center experience.
abstract: BACKGROUND
  Spontaneous intraarticular bleeds in congenital coagulation disorders result in early and extensive damage to the joints and periarticular structures. Total arthroplasty is the only effective method of treating these defects. Interim surgical procedures (arthroscopy, osteotomy, etc.) exist that can postpone arthroplasty, especially considering the fact that the condition affects young people. The aim of this paper is to discuss the range of trauma care and orthopedic procedures performed in patients with congenital coagulation disorders. Also presented are early results of joint arthroplasty in these patients.
  
  
  METHODS
  A total of 168 trauma care and orthopedic procedures were performed in patients with congenital coagulation disorders at the Clinical Department of Orthopedics and Traumatology of the Central Clinical Hospital of the Ministry of Interior in Warsaw in the years 2010-2013. Among them were total arthroplasties (79 arthroplasties of the knee, 30 of the hip, 3 of the ankle and 1 of the elbow), arthroscopies, filling bone cysts with grafts and trauma procedures. The HHS, KSS, AOFAS and MEPS scales were used to evaluate the respective clinical results of hip, knee, ankle and elbow arthroplasty procedures. A VAS was used to evaluate pain intensity. In knee arthroplasty patients, quality of life parameters were evaluated with the WOMAC index.
  
  
  RESULTS
  In patients post hip arthroplasty, HHS scores increased by 50.22 points and VAS scores increased by 6.34 points. An increase of 116.41 points in KSS scores and 6.67 points in VAS scores was recorded in patients after knee arthroplasty. Also, WOMAC scores improved by 53.8 points after surgery. Evaluation of early results of ankle arthroplasty in the AOFAS scale showed a mean improvement of 35.5 points and a 5-point improvement in VAS scores. MEPS scores, used for evaluation of elbow arthroplasty results, improved from 15 to 70 points, with an improvement from 6 to 2 points in VAS scores.
  
  
  CONCLUSIONS
  1. Orthopedic procedures in patients with congenital coagulation disorders require thorough preparation of the patient and close cooperation between the orthopedic and hematological teams. 2. Early clinical outcomes are promising. 3. Decreased pain intensity, increased joint range of motion and improved quality of life post-surgery are observed.
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Ireneusz
authors.initials: I
authors.lastname: Kotela
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Żbikowski
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Paweł
authors.initials: P
authors.lastname: Ambroziak
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kotela
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Lorkowski
authors.affiliation: Clinical Department of Hemostatic Disorders and Internal Diseases, Institute of Hematology and Transfusion Medicine in Warsaw, Poland.
authors.forename: Ewa
authors.initials: E
authors.lastname: Stefańska-Windyga
authors.affiliation: Department of Orthopedics and Rehabilitation, 2nd Faculty of Medicine, Medical University of Warsaw, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Wojdasiewicz
authors.affiliation: Clinical Department of Orthopedics and Traumatology, Central Clinical Hospital of the Ministry of Interior in Warsaw, Poland.
authors.forename: Filip
authors.initials: F
authors.lastname: Latawiec
authors.affiliation: Clinical Department of Hemostatic Disorders and Internal Diseases, Institute of Hematology and Transfusion Medicine in Warsaw, Poland.
authors.forename: Jerzy
authors.initials: J
authors.lastname: Windyga
mesh_terms: D000328:Adult; D000368:Aged; D016512:Ankle Injuries / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D018771:Arthralgia / Q000150:complications / Q000517:prevention & control; D001178:Arthroplasty / Q000379:methods; D001778:Blood Coagulation Disorders / Q000150:complications* / Q000151:congenital*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007718:Knee Injuries / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D007869:Leg Injuries / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D019637:Orthopedic Procedures / Q000379:methods*; D010027:Osteotomy; D010147:Pain Measurement; D010348:Patient Care Team / Q000458:organization & administration; D019106:Postoperative Hemorrhage / Q000209:etiology / Q000517:prevention & control*; D011788:Quality of Life; D011859:Radiography; D016059:Range of Motion, Articular; D000070599:Shoulder Injuries; D012785:Shoulder Joint / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome