Elective surgery in patients with congenital coagulopathies and inhibitors: experience of the National Haemophilia Centre of Venezuela.

Elective surgery in patients with congenital haemophilia with inhibitors carries a high risk of bleeding. However, inhibitor patients also have a high risk of haemarthroses and other orthopaedic complications, and surgery could improve their quality of life. Successful elective surgery has been reported in inhibitor patients under haemostatic cover with plasma-derived activated prothrombin complex concentrate (pd-aPCC) or recombinant activated factor VII (rFVIIa).

Radiosynoviorthesis in haemophilia: how safe?

One of the best procedures to prevent haemarthrosis in haemophilia has been radioactive synovectomy (radiosynoviorthesis). Since 1976 we have performed 119 radiosynoviortheses in 110 patients, aged from 3 to 40 years (mean 10), and of whom 71 were under 12 years of age. The knees were injected in 71, elbow in 29, ankles in 16, and shoulders in 3 cases.

Adductor myotomy in cerebral palsy: uni or bilateral.

Introduction: In patients with cerebral palsy, the prevention of hip dislocation should be the "primum momens" of early surgery. The surgery consists of a myotomy of the adductor medium, mayor and gracilis, and, in non-ambulatory cases, a neurectomy of the second branch of the obturator nerve. The purpose of this study was to examine whether the adductor myotomy should be performed on the contralateral side at one sitting.

Vascularised iliac graft as a method of acetabular augmentation in subluxed hip in adolescents.

This article presents the clinical experience of patients with a dysplasic subluxated hip treated with the same procedure involving a vascularised graft of iliac crest by sartorius muscle. This study was performed in three different centres, in two different countries, and in 31 patients with 37 hips (9 bilateral). All patients were clinically evaluated according to the Merle d Aubigne scale and radiological measuring of acetabular coverage, Sharp angle, and CE angle.

Chemical synoviorthesis with oxytetracycline clorhydrate (Emicine) in recurrent haemarthrosis.

This is a non-controlled experimental prospective clinical study that evaluates the satisfactory results in the chemical synovectomy (synoviorthesis) with oxytetracycline clorhydrate (Emicine, Lab. Pfizer Ltda, Guarulhos, Sao Paulo, Brazil) in recurrence haemarthrosis in different joints, demonstrating that it is an effective method in the treatment of these recurrent haemarthrosis in haemophilia. 84 patients of whom 77 concluded the full course of treatment.

Elbow interposition arthroplasty in children and adolescents: long-term follow-up.

A long-term follow-up was made of 12 elbows operated upon between 1971 and 1986, with more than 20 years' follow-up, in nine males and three females, age at the time of surgery between 10 and 19 years . Eight right and four left elbows were involved, and there were three aetiological causes. Seven cases were sequelae of elbow fractures, of which five were supracondylar and two were of the olecranon.

Percutaneous treatment of haemophilic digital pseudo tumours.

In young patients with haemophilia, blood cysts in the finger bones may grow rapidly and imitate a malignant tumour (pseudo tumour). The condition must initially be treated with administration of factor VIII-IX. If this does not control the growth, surgical treatment is indicated.

Haemophilic pseudotumours of the limbs and their percutaneous treatment.

The percutaneous treatment of limb pseudotumours is a nonaggressive method of treating haemophilic pseudotumours. However, efforts should be directed to the prevention of such pseudotumours by ensuring that all patients receive adequate treatment of their bleeding episodes through education and the elimination of geographical or social barriers that prevent access to such treatment. Prevention of pseudotumours by means of early substitution treatment of muscular bleeding episodes is the best treatment.

Intra-articular hyaluronic acid in the treatment of haemophilic chronic arthropathy.

We report our preliminary experience with the use of hyaluronic acid (Synvisc) in 29 joints from 25 different haemophilic patients (17 knees, six shoulders, four ankles, one elbow and one hip). All the joints were grade III of our classification, characterized by synovial thickening, axial deformities and muscle atrophy (chronic arthropathy). In view of the very satisfactory results obtained with this procedure, we have substituted Synvisc for the previous use of intra-articular long-standing corticosteroids that we had been used for some years.

On the safety of synoviorthesis in haemophilia.

One of the best procedures to prevent haem-arthrosis has been radioactive synovectomy (synoviorthesis). Since the first report of radioactive synovectomy in haemophilia of Ahlberg in 1971 [1], many centres adopted this procedure as the one of choice, through fibrosing the synovial membrane, prevent further haemarthrosis. Since 1976 we have performed 104 such radioactive synoviorthesis in 97 patients, age ranging from 6 to 40 years with a mean of 10 years.

Chemical synoviorthesis with rifampicin in haemophilia.

Rifampicin is an antibiotic that has been currently used for the treatment of noninfectious articular lesions with satisfactory results. The first experience was performed with patients who presented rheumatoid arthritis, and later with haemophilic patients. The clinical experience of three haemophilia centres which used rifampicin for the treatment of chronic haemophilic synovitis is presented here.

Synovectomy with rifampicine in haemophilic haemarthrosis.

The purpose of this paper was to assess the effectiveness of intra-articular injected rifampicine in haemophilic patients in order to achieve synovectomy by preventing repeated intra-articular bleeding. We have used this technique in haemophilic patients previously and reported our results on 13 cases [1]. Two hundred and fifty milligrams of rifampicine was injected into the elbow and ankle joints and 500 mg was injected into knee joints with 3-10 mL of lidocaine, depending on the joint size.

Cytogenetic studies in patients with hemophilic hemarthrosis treated by 198Au, 186Rh, and 90Y radioactive synoviorthesis.

This study assesses chromosomal structural changes (CSCs) studied by conventional lymphocyte cultures and banding techniques in 79 hemophilic patients with hemarthrosis treated with radioactive synoviorthesis, 31 hemophilic patients with hemarthrosis not treated by this procedure, and 110 nonhemophilic patients matched by age and sex (control group). In 14 patients treated with 198Au (group A), premalignant CSCs and nonspecific CSCs were found, respectively, in 1.69% and 17.

Non-operative treatment of flexion contracture of the knee in haemophilia.

For the non-operative treatment of flexion contracture of the haemophilic knee we have used serial casting and wedging in 58 patients, and extension/de-subluxation orthoses in 13 patients. On average it was possible to achieve -5 degrees of extension by 4 weeks, with only a little improvement in the following 4 weeks. The short--to medium-term results using either the extension/de-subluxation hinges or serial casting were similar.

Treatment of acute and chronic synovitis by non-surgical means.

This paper reviews personal experience in the treatment of recurrent haemarthrosis and chronic synovitis by non-surgical means. Experience with synoviorthesis with rifampicine and radioactive colloids is analyzed, and a multiple chromosomal study to demonstrate safety of radioactive injections is described. The results obtained are so very satisfactory as to recommend non-aggressive synoviorthesis as the treatment of choice to prevent recurrence of bleeding.

Congenital deficiency of the tibia: a report on 22 cases.

Congenital deficiency of the tibia (tibial hemimelia, aplasia, or dysplasia) is very rare, with an incidence of approximately 1 per 1 million live births. The disorder usually is accompanied by other congenital anomalies on the same limb or other parts of the body. A report is made of 22 limbs observed in 18 patients from 1970 to 1987 at San Juan de Dios Hospital in Caracas, Venezuela, with more than a 5-year follow-up.

Chemical synoviorthesis for hemophilic synovitis.

For many years, Rifampicin has been used empirically for the treatment of hemophilic chronic synovitis with encouraging results. A study was performed in which Rifampicin was shown to reduce the inflammation of joints affected by hemophilic synovitis. A clinical study was performed on 48 hemophilic patients (48 joints).

Intraarticular dexamethasone in advanced chronic synovitis in hemophilia.

From 1988 to 1966, 34 patients with advanced chronic hemophilic synovitis (25 Grade III and nine Grade IV) were treated with intraarticular injections of long acting dexamethasone (sodium phosphate of dexamethasone plus acetate of dexamethasone) in cycles of three injections with 3-week intervals between each injection with 6-month rest intervals between cycles for as many as three cycles, depending on the evolution of each case. All patients had chronic severe synovitis, axial deformity, muscular atrophy, and diminution of range of movement. There were 31 knees, two ankles, and two shoulders.

Hematomas within the iliopsoas muscles in hemophilic patients: the Latin American experience.

The majority of bleeding episodes in patients who have hemophilia occur within the musculoskeletal system, primarily in the joints, but approximately 30% occur within the muscles. Iliopsoas muscle bleeding episodes are often large in volume, causing muscular function inhibition, angular deformities, and nerve involvement. Recurrent hemorrhages are common (14.

Radioactive synoviorthesis in hemophilic hemarthrosis: materials, techniques, and dangers.

Radioactive synoviorthesis with 198Au, 90Y, 186Re, and 31P would seem to be the treatment of choice for recurrent hemarthroses in hemophilia. The clinical results, obtained by different centers, show a definite diminution of hemarthroses in 88% of cases. The advantages of radioactive synoviorthesis compared with surgical synovectomy are: better results, the requirement of substantially reduced antihemophilic factor, the possibility of performing the procedure on multiple cases concurrently on an ambulatory basis, no interference with articular range of movement, and the low cost of the procedure.

Congenital absence of the radius.

Between 1971 and 1987, we treated 26 patients with congenital absence of the radius which had produced a club hand; 37 hands were affected. Treatment before the age of 8 months was by gentle manipulation and serial plasters. If a functional position was not obtained, and in patients over 8 months, surgical reduction was carried out.

Report on a case of Hutchinson-Gilford progeria, with special reference to orthopedic problems.

Hutchinson-Gilford progeria is a very rare syndrome of premature aging and often features many orthopedic abnormalities. This is a case report on a young boy suffering from progeria. His orthopedic history included bilateral talus deformities of the feet, bilateral dislocated hips, pes planus, a fractured femur (which healed without complications), aseptic necrosis in the left nuclear head of the femur, bilateral fixed hip flexion deformities, bone dysplasia, osteoporosis and osteolysis.

The care of the limb deficient child in Venezuela.

An overview of the situation in Venezuela is made by sampling the cases of congenital limb deficiency treated at the Hospital San Juan de Dios in Caracas from 1961-1989. The major longitudinal deficiencies are analysed. The socioeconomic situation of the patients is of most importance being the cause of final decisions in relation to surgery, the ordering of prosthesis and orthosis, supplied mainly by the Venezuelan Institute of Social Security (IVSS), the maintenance of these items and the possibility of travelling to the hospital for diagnosis, treatment and follow-up.

Achilles tendinitis in ballet dancers.

Overuse injuries of tendons are known to occur in persons whose activities submit the tendon to excessive stress. Classical ballet dancers performing en pointe, demie point, or plié exert forces that, although normal in magnitude, are increased in frequency, thus overusing the Achilles tendon. In the present study all cases of Achilles tendinopathy seen in a period of three years in three ballet companies were reviewed by a special orthopedic clinic.