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.

Evaluación de la radiografía de cadera frente a la tomografía tridimensional en pacientes con parálisis cerebral / Hip X-rays versus 3-D CAT scans in patients with cerebral palsy

Introducción. Se estudiaron 17 pacientes con diagnóstico de parálisis cerebral, para evaluar la utilidad de los rayos X de cadera frente a la tomografía 3D. Material y método. Se compararon 3 parámetros radiológicos con 4 parámetros tomográficos tridimensionales. Resultados. Se obtuvieron resultados que correlacionan significativamente a los estudios radiológicos con los tomográficos. El ángulo CE de Wieberg mostró 28 caderas displásicas y 6 normales. El índice de Reimers 29 caderas displásicas y 5 dentro de límites normales. El índice acetabular 4 caderas normales y 30 caderas subluxadas. En los parámetros tomográficos el ángulo cérvico-diafisiario femoral presentó una media de 147,09° con un rango entre 127° y 175°, lo que demuestra un promedio más alto de lo normal. El ángulo de anteversión femoral obtuvo los siguientes resultados: media de la muestra 33,43°, rango superior 50°, rango inferior 9°. Se observa un valor aumentado de la anteversión. El índice acetabular axial mostró un promedio de índice axial acetabular de 120,29°, siendo el valor normal 101,6° para los 9 años de edad, lo que se asocia con aplanamiento del acetábulo. El ángulo de anteversión acetabular presenta una media de 13,11° con un rango entre 24° y 7°, lo que representa una paridad en los resultados. Conclusiones. La indicación de radiografías está justificada para el diagnóstico y pronóstico de displasia de cadera. La cobertura acetabular y las evaluaciones para cirugías deben estudiarse y programarse con tomografías tridimensionales

Introduction. Seventeen patients diagnosed with cerebral palsy were studied to assess the results of X-rays vs. 3D CAT scans of the hip. Materials and methods. 3 X-ray parameters were compared with 4 3D CAT scan parameters. Results. The results obtained showed a significant correlation between X-rays and CAT scans. According to the assessment of the Weinberg Central Edge (CE) angle there were 28 dysplastic hips and 6 normal hips. According to the Reimers index there were 29 dysplastic hips and 5 normal hips. According to the acetabular index there were 30 subluxated hips and 4 normal hips. Using CAT scan parameters the mean value of the femoral neck-shaft angle was 147.09° (range: 127°-175°), a higher-than-normal figure. The mean value of the femoral anteversion angle was 33.43° (range 9°-50°), also higher than usual. The mean axial acetabular index was 120.29°, with a normal value of 101.6° at 9 years of age associated with acetabular flattening. The mean acetabular anteversion angle was 13.11° (range 7°-24°), which indicated a parity of results. Conclusions. X-rays are necessary for the diagnosis and prognosis of hip dysplasia. Acetabular coverage and pre-surgery assessment and planning must be done based on 3D CAT scans

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. This method is theoretically more physiological and does not destroy the joint cartilage further, as corticosteroids can.

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.

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. The protocols were different. It was observed that rifampicin is more effective when it is used in small joints (elbows and ankles), than when used in bigger ones (knees), and that a high number of injections predicts failure. Mention is also made of experimental studies in animals where it was shown that the healing pattern of rifampicin is similar to that of NSAIDs.

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.

Problemas ortopédicos del niño hemofílico / Orthopaedic problems of hemophilic children

En este artículo se revisan los principales problemas del niño hemofílico, especialmente los hemartros, los hematomas musculares y los quistes hemofílicos (pseudotumores). La sinoviortesis con rifampicina y el tratamiento percutáneo de los pseudotumores son dos de los métodos fundamentalmente analizados en este artículo (AU)

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. The injections were repeated once a week for 7 weeks. Patients were only covered with antihaemophilic factor on the day of the injection at 30% above their coagulation level. We evaluated the results using two measures: subjective reports from the patient and objective assessment by the examiner. In the subjective reports the patient graded the results from their own perspective from 1 (poor) to 10 (excellent): 1-3, poor; 4-6, fair; 7-8, good; and 9-10, excellent. In the objective reports the grading was: excellent ('dry joint', full function, no haemarthrosis, no synovitis); good (clinical improvement, synovitis, reduction of haemarthroses, full function); fair synovitis (reduction of haemarthroses, no change in function); poor synovitis (persistent haemarthroses). This paper reports on the results of 38 patients with 39 joints with more that 3 years follow up, mean 1.8 years. There were 22 knees, nine elbows and eight ankles. Subjectively, there were excellent results in 21 joints (11 knees, six elbows and four ankles) good results in 15 joints (eight knees, three elbows and four ankles), fair results in two knees and a poor result in one knee. Objectively, results obtained were excellent in 20 joints (11 knees, six elbows and three ankles); good in 17 (nine knees, three elbows and five ankles); fair in one knee and poor in one knee.

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.

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 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."

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.

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.

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.

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.