Edward J. Riseborough

Chondrosarcoma in children and adolescents.

Chondrosarcoma is rare in children. Only 12 patients with a diagnosis of chondrosarcoma were treated at Childrens Hospital Medical Center in Boston during the period from 1957 to 1980. Pediatric chondrosarcoma has a relatively rapid onset, manifested by pain, a palpable mass and neurological symptoms with the lesion localized in the spinal column. The pelvis was the most frequent location. Ten patients had primary and two patients had secondary chondrosarcoma. Of the latter two, one developed chondrosarcoma in a preexisting osteochondroma and the other had irradiation for neuroblastoma. The poorest outcomes were in patients who had involvement of the pelvic girdle and spinal column. Grade 2 and 3 lesions had the worst prognoses with metastases occurring within 12 months. Eight patients died at the time of study, and the longest survival time was 80 months. If surgically feasible, the treatment is radical excision. Radiation therapy and chemotherapy should be reserved for recurrences or distant metastasis.

Alterations in pulmonary function, coagulation and fat metabolism in patients with fractures of the lower limbs.

If the underlying pathology of the syndrome of fat embolism is the presence of circulating emboli of neutral fat with adherent platelets and red blood cells, then one could expect certain changes in normal vascular cellular elements and fat metabolism, with resultant alterations in pulmonary function. In our series of 118 patients with fractures of the lower limbs we found that more than half of them exhibited hypoxemia as well as a decrease in the hematocrit and platelet counts with a concomitant increase in platelet adhesiveness. In addition, those patients with hypoxemia showed increased fibrinogen degenerative product levels indicating an increased fibrinolysis. We found only a slight temporary rise in the serum triglycerides, but the nonesterified fatty acid levels rose sharply over the first three days following trauma associated with an increase in serum lipase. Our study demonstrated that this increase in serum lipase occurred slightly before the peaking tendency observed in the nonesterified fatty acids. Following trauma, fat emboli with adherent platelets and other vascular cellular elements are formed; we would expect that these emboli would lodge in the capillaries and small vessels of the lung, thereby producing a physiological shunt. An increase in the A-aDo2 confirmed this hypothesis and was associated with a decrease in the arterial oxygen level in over half the patients studied. Although 58 of our patients showed evidence of hypoxemia associated with a fall in hematocrit and platelet count, not one of them showed clinical signs and symptoms of the fat embolus syndrome. This study suggests that a subclinical form of fat embolism does exist. What causes a small percentage of those patients with subclinical fat embolism to progress to a clinical fat embolism is still unknown. The majority of our patients spontaneously returned to normal within 5 days. Reviewing the results of those patients who developed hypoxemia and the two patients who developed clinical fat embolism, there seems to be no indication of what causes the progression. Hypotension and shock do not seem to be relevant to the progression of the subclinical condition. Not one of 110 reviewed had evidence of shock or persistent hypotension, yet 58 of these patients developed changes in arterial saturation, vascular elements, and evidence of disseminated intravascular coagulation.

Irradiation induced kyphosis.

Eighty-one patients with Wilms tumor treated by irradiation and chemotherapy were studied. Despite the fact that multiple portals for irradiation were used, each crossing the midline, the amount of irradiation delivered to different parts of the vertebral body varied and it was this variation in delivered dose which produced axial skeletal deformities in 70% of the patients. Of the 57 patients with these deformities 32 had scoliosis, 22 kyphoscoliosis and 3 patients pure kyphosis; 12 patients had a kyphotic deformity of over 25 degrees, 7 patients requiring surgical correction. A high incidence of pseudarthrosis following posterior fusion has led to the preference of a 2-stage procedure, anterior interbody fusion followed by a posterior fusion with Harrington rods after 2 weeks of correction in halo femoral traction.

Alterations in serum lipid concentrations following skeletal trauma

Serial studies of 118 patients following fractures of the pelvis or lower extremity demonstrated a significant association between hypoxemia and the serum concentration of nonesterified free fatty acids. Serum lipase levels were similar but not as significant. No differences were noted in the serum concentrations of either triglycerides or cholesterol.