الفهرس 
I- Physiological considerationsOnly 14 pages are availabe for public view
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Abstract
Hemophilia is an inherited disorder in which patient lack necessary factor proteins for homeostasis to occur. This bleeding attack may occur spontaneous or as a result of trauma leading to repeated attacks of hemarthrosis. Because of hemophilia is genetically determined, bleeding attacks and coagulation disorders may appear clinically during the child’s life.
Hemophilia is classified – according to factor deficiency – into hemophilia A, in which factor VIII is deficient and hemophilia b, in which factor IX is deficient, further classification of hemophilia based on factor level in plasma serum and clinical presentation into three type, mild, which factor level is more than 5% and clinically presented by bleeding rarely, specially, after significant trauma or surgery, moderate type, I which factor level in blood serum between 1% and 5% the patient may bleed several times per year and usually bleed only with mild trauma, and severe type, in which patient have factor level in blood serum less than 1% and the patient have severe episodes of bleeding per month and the bleeding may occur, in joints (hemarthrosis) soft tissue, and muscles.
Pathogenesis of chronic hemophilic synovitis is multifactorial physical, chemical, and inflammatory factors.
When repeated attacks of hemophilic hemarthrosis occur, the synovium begins to hypertrophy, and show focal area of villous formation which are highly vascular and rich of inflammatory cells, this reaction leads to chronic inflammatory hemophilic synovitis the early synovial reactions to intraarticular bleeding includes: synovial hypertrophy, hemosidren deposition in phagocytic cells, perivascular infiltration and early fibrosis of the subchondral layer, then, the recurrent hemarthrosis stimulate the synovium.
This abnormal-highly vascular synovium act as arteriovenous fistula causing abnormal growth of the epiphyseal growth plate, abnormal growth of bone leg length discrepancy, angular deformities, and axial deformities.
Hemophilia is a genetically determined, so that, the clinical manifestation may appear early in child’s life specially between 6 to 16 year, and the most common joints involved are, knee elbow, and ankle, predominantly the high weight bearing joints, these joints are more susceptible to hemarthrosis because of large amount of synovium within the joint space which can be nipped when the joint movements occur.
The clinical manifestations are: repeated attacks of intraarticular bleeding, painless swelling of the involved joints which is large but not tense, intermittent attack of pain due to rise of intraarticular pressure, hypertrophic palpable synovium, reduction of capacity and reduction of range of movements.
In late stage of chronic hemophilic synovitis, deformities of the joints can be detected clinically in form of: flexion deformities, angular deformities, valgus deformities, ankylosing and dislocation of the joints, which – in the end – may lead to complete immobilization of the joint.
Plain x ray is a good diagnostic investigation show characteristic findings in form of: increase density of joint space due to hypertrophic synovium and deposition of hemosedrin in phagocytic cells, epiphyseal over growth subchondral irregularities, subchondral cyst formation, widening of the intercondylar notch of the femur, erosive changes of articular surface, and osteoporosis.
In late stage of chronic hemophilic synovitis, we can show: osteophytosis, narrowing of joint space, irregularities of subchondral area, deformities in form of angular, axial, sublaxation and dislocation of the joints, fractures and leg length discrepancy.
By ultrasonography we can differentiate hemophilic synovial hypertrophy from effusion and can diagnose the cartilage erosions. Subchondral cyst formation, and soft tissue hematoma.
Computerized Tomography (C.T) and magnetic resonance imaging (M. R. I) are recent methods for investigations in such cases and help not only in diagnosis but also to confirm our diagnosis specially, soft tissue lesions, like, synovial abnormality, joint space narrowing, cartilage damage and erosions, capsular thickening tears of ligaments, periaticular bleeding soft tissue hematoma, subchondral cyst formation and pseudotumours.
Conservative management can be achieved through factor replacement therapy with supplementation by missing factor and restoration of normal hemostasis. One unit of factor VIII/kg rises the plasma factor VIII coagulant level 2% therefore, to achieve 80% of factor VIII level in a 70Kg man with severe hemophilia, 40 u/kg (2800 units) of factor VIII must be given, from anther point, one unit of factor IX concentrate is needed to rise the factor IX level 1% therefore, to achieve a level of 80% in a 70 kg man, 80u/kg (5600u) must be given.
Different methods can be administrated for replacement therapy, intravenous infusion, intravenous bolus, subcutaneous injections and intranasal spray in form of desmopressin acetate, intravenous infusion is preferred preoperatively and immediately post operatively to eliminate variability in absorption, and intranasal form after discharge from the hospital and can be continued at home.
Patient with factor inhibitors is difficult to treat, because of presence of an antibodies that inhibit the activity of factor VIII, so that, porcine factor VIII in dose of 50 to 300 u/kg has been used effectively in patient with factor VIII inhibitors, prothrombin complex concentrates or activated prothrombin complex concentrates have been used at dose 70 u/kg intravenously. Recently recombinant in inhibitors, factor IX inhibitors is very difficult to treat, so that the plasmapherisis is the only option for factor replacement therapy in such cases.
Steroid therapy is a second line of conservative management of chronic hemophilic synovitis and documented with successful clinical studies in many hemophilic centers all over the world, intraarticular injection of 2 mg dexamethasone sodium phosphate, which is rapidly soluble for immediate action and 8 mg dexamethasone acetate, less soluble for long acting action, diluted in 2% lidocaine Hcl in different proportions according to the target joint.
The procedure must repeated every 21 days interval, three times to comprise one cycle of treatment, 6 months after the treatment cycle. A new cycle of three injections is repeated for as many as three cycles.
Physiotherapy and rehabilitation programs are very important in conjunction with factor replacement therapy and this gives a good results and avoid further complication, different methods of physiotherapy can be appreciated e.g. : Isometric, Isotonic, Isotonic with gradual weights, dynamic, flexible exercises, many ways for physiotherapy as: pulsed short wave diathermy, ultrasonography and Ice therapy.
If conservative management fails to control bleeding and other attacks of hemarthrosis are still present or the state of joint not respond to conservative management, synovectomy become the suitable choice for control bleeding and prevent deterioration of state.
Open synovectomy aimed to remove the abnormal synovial membrane and good debridment of joint with removal of blood clotting, removal of loose bodies, resection of adhesions and shaving of affected cartilage. Open synovectomy can be achieved as a single procedure or integration with other procedures for management of complication as: correction of deformities e. g: conventional sypracondylar osteotomy in case of elbow osteoarthroapthy.
Arthroscopic Synovectomy can be performed in case of chronic hemophilic synovitis with a good results and minimize complication which occur by open synovectomy e. g: Post operative infection, limitation of range of movement, chronic disuse atrophy.
In open Synovectomy or arthroscopic Synovectomy factor replacement therapy must be applicated preoperatively and post operatively, even, with post operative physiotherapy, the administration of factor therapy is very important.
Non surgical synovectomy can be achieved through chemical or radioactive synoviorthesis, chemical synoviorthesis consists of aspiration of joint fluids, and injection of 100 mg of Rifampicin antibiotic substance diluted in 5 ml of normal saline solution, and this procedure is repeated every week for 6 weeks, chemical synoviorthesis is advised for use in small joints of adults and all joints of children without any fear of any complication.
Radioactive synoviorthesis is other method which used radioactive material for intraarticular injection under control screening to perform subsynovial fibrosis, radioactive material which used with encouraged results are : colloidal ythrium (Y90), Colloidal phosphorous (p32), Colloidal gold (Au 198) and Recolloidal rhenium (Re 186) and the dose of each one differ from one to other and from joint to other.
The evaluation of the results after radioactive synoviorthesis gives a good results about treatment and recovery of Hemophilia synovitis. Reduction of frequency and severity of hemarthrosis, decrease the requirement of antihemophilic factor.
whereas total jount replacement is a well – documented treatment for hemophilic hip, knee and elbow arthropathy.
The chromosomal stusies show that these abnormalities which found in hemophilic patients whom managed by radioactive synoviorthesis are transitory and disappear a few days after treatment, and non specific changes are similar to those which found after viral disease or the use of common drugs