Amyloid heart disease manifested by systemic arterial thromboemboli.

Amyloid heart disease characteristically produces a stiff heart syndrome whereby diastolic filling is impaired yet systolic function is well preserved. We report two patients with this pattern of amyloid heart disease, both of whom developed cardiogenic thromboemboli. The rarity of this complication is striking given the pathophysiologic bases of amyloid heart disease. Investigation of contributing causes revealed that the phenomena appeared to represent the cumulative effects of disorders producing stasis, endothelial disturbance, and probable abnormalities in blood coagulability, the classic Virchow's triad revisited. Understanding of the pathophysiologic basis of this event leads to specific suggestions for workup and management in this patient population.

Clinical features of early haemarthroses in severely affected adolescent haemophiliacs.

Six hundred and ninety bleeds into the knees, ankles and elbows of severe haemophiliacs presenting for treatment within 3 h of the onset of symptoms have been studied with the aim of providing clinical information for the haemophiliac patient or his parent treating early bleeding episodes at home. Patients were resident at a boarding school and documentation and follow-up to complete resolution was possible. Stiffness was a presenting symptom in 61% of elbow bleeds, 49% of knee bleeds and 18% of ankle bleeds. Pain was a presenting feature in 79% of ankle bleeds, 55% of knee bleeds and 42% of elbow bleeds. Tenderness was a common feature of ankle bleeds (85%), less so in elbows (55%) and knees (69%). Swelling was a very common feature at all sites and the mean increases in girth of the knees, elbows and ankles were 1.42 cm, 0.88 cm, and 0.62 cm, respectively. All the knee and elbow bleeds and 85% of the ankle bleeds had demonstrable restriction of movement. There was a direct relationship between the degree of swelling, extent of movement restriction and time taken for complete restoration of function, the mean of which was 3.6 days for elbows, 2.5 for knees and 1.1 for ankles.

The clinical features of early bleeding into the muscles of the lower limb in severe haemophiliacs.

The clinical features, management and outcome of 178 early bleeding episodes into the musculature of the thigh and lower limb of 37 severe haemophiliacs are reported. Ninety-five per cent of all bleeds were treated in under three hours from onset of symptoms and the mean time to complete restoration of function was 3.5 days. The most frequent site of bleeding was the quadriceps (44 per cent) followed by the calf (35 per cent), anterior tibial compartment (seven per cent), adductors of the thigh (seven per cent), hamstrings (six per cent) and sartorius (one per cent). Bleeds of the quadriceps took longest to resolve (mean, four days), significantly longer than bleeds of the calf muscle (3.1 days). The first symptom in 66 per cent of bleeds was pain on movement. When the quadriceps was involved, this rapidly progressed to pain at rest. There was a significant prolongation of recovery time when bleeds of this muscle group were treated more than two hours after the onset of symptoms. Bleeds of the calf muscle required less transfusions and no prolongation in recovery time occurred in bleeds treated up to three hours from the onset of symptoms. The results of this study contrast markedly with earlier reports based on later presentations.

Intertrochanteric fracture of the femur with ipsilateral central fracture of the acetabulum.

The management of three patients each of whom sustained an intertrochanteric fracture of the femur with an ipsilateral central fracture of the acetabulum is described. Complications encountered in the first patient, using a standard nail and plate type of fixation, suggested that a screw compression system should be used for this combination of injuries. This method of internal fixation was successful in the treatment of the two other patients.

The management of late diagnosed congenital dislocation and subluxation of the hip-with special reference to femoral shortening.

Thirty hips affected by congenital dislocation or subluxation underwent surgical treatment between the ages of five and fifteen years, and the results are presented. Reduction was aided by concurrent femoral shortening in seventeen. An aggressive approach in older children not only relieves the presenting symptoms in most, but also, by improving the anatomical relationships, increases the prospect of success should total replacement become indicated in the future.

Anomalous insertion of the tibialis posterior tendon in congenital metatarsus varus.

Congenital metatarsus varus is a common deformity that usually responds to conservative treatment. In fifteen feet operated on for resistant deformity, an anomalous insertion of the tibialis posterior tendon was found in fourteen. Six cadaveric infant feet were dissected to confirm the normal insertion. The dynamic component of the deformity is stressed.