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). Recombinant FVIIa has recently become available in Venezuela and, unlike pd-aPCC, has not been associated with an anamnestic response. The aim of this study was to assess our experience using rFVIIa as a first-line and sustained treatment in elective invasive surgical procedures at the National Haemophilia Centre in Venezuela. Surgical procedures were classified as major or minor, under haemostatic cover with rFVIIa. A total of 13 patients (12 with haemophilia A with high-responding inhibitors and one with von Willebrand's disease type 3) underwent a total of 19 surgical procedures under rFVIIa cover. Thirteen procedures were classified as major surgeries. Intraoperative haemostasis was achieved in the majority of patients. Only two patients required an additional dose of rFVIIa, at 30 min and 75 min, respectively, with good results. Postoperative haemostasis was considered effective in 16 of 18 (89%) of the procedures in haemophilia A patients. Treatment was considered to be ineffective in two patients because of excessive postoperative bleeding. Data from the study provide no safety concerns, and demonstrate that rFVIIa provides effective haemostatic cover in elective surgery in patients with inhibitors; research is ongoing to determine the optimal dose for such procedures.

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. 82 joints were injected. The dosage injected was 5 cm(3) of the drug (25 mg) in 5 cm(3) of anaesthesia for the knee, 2 cm(3) with 1 cm(3) of anaesthesia for the elbow and 1 cm(3) plus 1 cm(3) of anaesthesia for the ankle. These injections were administered once weekly with a reinforcement in 1 month. In case of failure the same can be administered repeatedly. Subjective parameters included pain, range of movement and use of the joint involved. Pain decreased from a mean of 6.5 to 0.9 (Likert scale). Range of movement increased from 5.9 to 9 and joint use increased from 5.9 to 9.2. Objective parameters included joint diameter and range of movement. Range of movement for flexion and extension improved from 72.2 and 149.2 to 73.7 and 167, respectively, for the knees. From 57.3 and 160 to 66.6 and 170, respectively, for the shoulder. And, from 22.7 and 10.8 to 34 and 18.6, respectively, for the ankle. This procedure has multiple advantages such as immediate therapeutic effect, short period of treatment, easy technique, much less AHF coverage (30% above coagulation level), less costly than radiocolloid treatment, which make it a perfect alternative treatment for developing countries.

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.

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 17.23% of metaphases. The former disappeared, but 1.7% of the nonspecific changes persisted 2 years after injection. In 31 patients treated with 186Rh (group B), CSCs were not found previous to radioactive synoviorthesis but were present as nonspecific changes in 1.25% of metaphases 6 months later; they disappeared 1 year after injection. In 34 patients treated with 90Y (group C), CSCs were not found previous to radioactive synoviorthesis but were present as nonspecific changes in 0.89% of metaphases 6 months later; they disappeared 1 year after injection. Only nonspecific CSCs were found in 0.79% of metaphases in patients not treated with radioactive synoviorthesis (group D). CSCs were not present in control subjects. The authors conclude that in some hemophilic patients with hemarthosis treated with radioactive synoviorthesis using 198Au, 186Rh, or 90Y, reversible premalignant or nonspecific CSCs could be present; nonspecific CSCs may persist in a low proportion of metaphases up to 2 years after injection when 198Au is used as the radioactive agent. Radioactive synoviorthesis seems to be from a cytogenetic point of view a safe alternative for these patients.

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. The report includes 11 girls and 7 boys, with observations of 11 left and 11 right tibiae. Familial antecedents were present in four patients: brother and sister and two brothers. According to the classification by Jones, Barnes, and Lloyd-Roberts, there were nine cases of type Ia, four of type Ib, three of type II, two of type III, and four of type IV. The foot of the affected limb was normal in only 12 cases. Treatment, always surgical, consisted of T/K amputation in 10 cases of types Ia and Ib, fibula pro tibia and B/K amputation in 1 case of type II, Syme amputations in 2 cases of type III, and astragalectomy and closure of diastasis in 4 cases of type IV deformity. Patients in five cases refused treatment. The sooner the amputation is performed, the easier and faster the rehabilitation and adaptation to the prosthesis. An early amputation is accepted by the child as a "congenital amputation."

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. All these transform the situation from a medical problem into a socio-economic problem typical of a third world country.

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. Long experience in the treatment of chronic arthropathy with intrarticular corticosteroids and hyaluronic acid has shown very promising results.

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. Both methods have been shown to result in significant improvement in joint contracture.

Congenital dislocation of the knee.

Congenital dislocation of the knee (CDK), first described in 1922, is a rare condition sometimes associated with other congenital malformations. There is a total anterior dislocation with recurvatum and upward displacement of the proximal tibia. This review covers our ten year experience at the hospital San Juan de Dios, in Caracas, Venezuela, a children's hospital with three orthopaedic services. We have seen 22 affected knees in 14 patients. Newborn patients were treated on traction until reduction was achieved, followed by serial plaster of Paris casts until, optimally, 90 degrees of flexion was gained. Surgical treatment was used if conservative treatment had failed after six months, or if the patient first presented after the age of 12 months. All our patients were eventually able to walk, three with the assistance of calipers. Some limitation of knee flexion was commonly seen. The best results were achieved when treatment was started early.

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. Sixty-five of these patients were under 12 years of age. The knees were injected in 61 cases, elbow in 26 cases, ankles in 14 cases and shoulders in three cases. The clinical results of this procedure show 80% of excellent results with no further bleeding. In case of failure, a new injection can be given in the same joint at a 6-month interval, or an injection for the same purpose in other joint. One of the criticisms against this method is the possible chromosomal damage induced by the radioactive material. In our centre, four studies have been carried out in order to see whether these changes, when they occur, are everlasting; all have demonstrated that chromosomal changes are reversible. The radioactive material used in the two first studies was Gold-189 (189Au). In 1978, 354 metaphases were studied with 61 ruptures, 17.23% (nonpremalign) and six structural changes, considered premalign (1.69%). Any number below 2% is considered not to be dangerous. A further study was done in 1982, in the same group of patients with a result of 21 ruptures (3.34%) and no structural changes. This demonstrated that the possible premalign changes disappeared with time. A third study was performed in a series of 13 patients that sustained radioactive synoviorthesis with Rhenium-186 (186Re) in November 1991. For comparison, we carried out a chromosomal study just before and 6 months after the radioactive material injection. The results confirmed that changes that could be linked to the radiation, appeared equally in nonirradiated patients, and those changes due to the radiation disappear with time, never reaching the dangerous zone of 2%. In the group treated with 186Re we studied an additional number of 130 metaphases with identical results and no structural changes. In a study on patients where 90Y was the radiocolloid, no premalign change was found before or after the synoviorthesis. It seems, in view of these results, that radioactive synovectomy is safe and gives great benefits to the haemophilic patients.

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.

Transfer of half the calcaneal tendon to the dorsum of the foot for paralytic equinus deformity.

Transfer of half of the calcaneal tendon to the dorsum of the foot will maintain correction of a paralytic equinus deformity. We have used this procedure on 97 feet since 1967. Seventy-six were reviewed and the overall results were excellent or good in 69%. The outcome was better in children with cerebral palsy (85% excellent or good) than in those with poliomyelitis (only 13% excellent or good).

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. A modified Riordan procedure was used until 1983, and thereafter a double Z-plasty. The best results were achieved in patients under 4 years of age.

Chromosomal study after radioactive synoviorthesis for haemophilic haemarthrosis.

Twenty joints in 19 haemophiliacs were treated by radioactive synoviorthesis with gold (Au 198) to prevent recurrent haemarthrosis. Twelve knees, six elbows and two ankles were treated in two separate groups (29. 9. 76 and 9. 5. 77). In eight cases (40%) no further haemarthrosis occurred. A diminution of bleeding was obtained in nine cases (45%), a total of 85% good results with 15% failures. One failure in the first group (an elbow) had a second synoviorthesis and was included in the second group also. Prior to synoviorthesis the joint was scanned with technetium (Tc99m) to compare the inflammation of the synovium with that of six months later. The technique including the dosage of Tc99m, Au 198, and factor VIII cover is presented. A leucocyte culture was performed in 16 cases to study any chromosomal breakage, by banding and fluorescent techniques.

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. The cause, whether by abnormal tension or incorrect use, development, and progression to chronic tendinopathy, as well as measures to prevent it, were analyzed in 19 cases. The methods of treatment, including conservative treatment with rest and refraining from dancing, local treatment such as ice and adhesive strapping, antiinflammatory drugs, local injections, thermotherapy, and laser therapy, were compared, and the time of recovery and ability to resume dancing were evaluated. Two cases required surgical treatment to subside, and the patients had to retire from professional dancing. The roentgenographic diagnosis of stage and progression of the tendinopathy is emphasized as a valuable accessory sign. The similarity in lesions between Achilles and patellar tendon problems was observed and confirmed.

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. Subjective and objective evaluations were done grouping the results in good, fair, and poor according to grade of patient satisfaction, presence of synovitis and pain, range of movement, and limitation of activities of daily living. In the subjective results there were 19 good results, 12 fair results, and four poor results, and in the objective evaluation there were 22 good results, nine fair results, and four poor results at an average followup of 1.5 years. The use of intraarticular dexamethasone as an alternative in the short to medium term for treatment of advanced chronic hemophilic synovitis with pain and limitation of function before doing an invasive surgical treatment is proposed.

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). Seventeen elbows, eight knees, and 23 ankles were treated. The mean age of the patients was 6 years (range, 4-23 years) and the mean followup was 29 months (range, 24-53 months). Overall, 40 excellent results and eight good results were obtained. The average number of weekly injections of Rifampicin was 3.06 (range, 1-10 injections). Eight patients experienced pain on the first injection, which subsided gradually with the subsequent procedures. Synoviorthesis with Rifampicin seems to be a good method for the treatment of hemophilic synovitis, especially in small joints (elbows and ankles) and in younger children.

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. In cases of failure, the procedure can be repeated after 6 months, and on as many as 3 occasions. Studies performed on the chromosomal changes that could be attributed to the radioactive material show the disappearance of these alterations a few years after treatment. No physical changes have been found that could be attributed to cytogenic alteration (hematologic or other) in any reported patients.

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 (14.2%) and neural involvement is as high as 37%. Three hundred patients are being observed in the authors' hemophilia center, 63 of whom have suffered 127 hemorrhages. Absolute bed rest and long term factor replacement are the mainstay of therapy. The experiences of physicians in other countries are appendixed to this article.