Congenital radioulnar synostosis.

Congenital radioulnar synostosis can be severely disabling, especially if it is bilateral or if severe hyperpronation exists. Functionally, patients with severe deformity have trouble getting a cup to the mouth, using eating utensils, or accepting objects in an open palm. Of 33 patients (17 bilateral and 7 unilateral) underwent derotational osteotomy, with the majority being performed through the synostosis held with, an intramedullary wire and secondary transfixing device. There were eight complications, four involving neurovascular compromise. In bilateral cases, the best end position appears to be 10% to 15% of pronation in the dominant extremity and neutral in the other. Eighty-two percent of the patients had good or excellent results.

Osteotomy of the fusion mass in scoliosis.

The value of an osteotomy of the fusion mass in attaining realignment of the spine when a lateral shift of the trunk over the pelvis has developed in the frontal plane was studied in fifty-five patients. All had had a previously fused but progressive spinal deformity. The indications for osteotomy were progression of the curve, imbalance of the trunk, pain, deteriorating cardiopulmonary function, or pseudarthrosis, or a combination of these conditions. Twenty-one patients had idiopathic scoliosis; fourteen, congenital scoliosis; ten, paralytic scoliosis; four, myelodysplastic scoliosis; and six had scoliosis secondary to miscellaneous conditions. A total of 154 osteotomies of the fusion mass, or 2.8 osteotomies per patient, were performed. Nine patients underwent an anterior spinal osteotomy or wedge resection as well. Most had a two-stage procedure which consisted of a posterior osteotomy, halo-femoral traction, and a posterior re-fusion with Harrington instrumentation two weeks later. At an average follow-up of thirty-two months, it was found that osteotomy of the fusion mass enabled the surgeon to realign the trunk over the pelvis and to correct pelvis obliquity, but correction of the angular deformity was only secondary. Although the rate of complications was high (51 per cent, including one postoperative death), osteotomy of the fusion mass and re-fusion may be indicated in a patient with a previously fused but progressively unbalanced, painful deformity of the spine.

Chondrosarcoma in children and adolescents.

Chondrosarcoma is rare in children. Only 12 patients with a diagnosis of chondrosarcoma were treated at Children's 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.

Combined anterior and posterior fusion in seventy-three spinally deformed patients: indications, results and complications.

Seventy-three patients with complex spinal deformities undergoing combined staged anterior and posterior fusion were analyzed. The indications for the combined approach were (1) severe kyphosis with or without neural compromise; (2) major thoracolumbar or lumbar curves with trunk imbalance or marked pelvic obliquity; (3) severe congenital scoliotic curves associated with hemivertebrae or anterior unsegmented bars; (4) absent posterior elements, e.g., myelodysplasia, post laminectomy; and (5) failed anterior fusion. In 35 patients with kyphosis, the deformity was reduced on the average from 78 degrees to 48 degrees. In 20 patients with marked trunk decompensation and/or pelvic obliquity due to a major thoracolumbar or lumbar curve, the scoliotic deformity was reduced from an average 85 degrees to 33 degrees. In 18 scoliotic patients with marked vertebral body deformation (hemivertebrae, anterior bars, lateral subluxation), the scoliotic curves were reduced from an average 60 degrees to 46 degrees. Postoperative morbidity was not significantly increased because of the combined procedure. The overall complication rate was high (32/73 patients). In view of this high rate of postoperative morbidity, the combined approach should not be undertaken by the occasional spinal surgeon. In selected far advanced or complex spinal deformities, combined anterior and posterior fusion may be indicated to attain optimal correction of the deformity as well as a solid pain-free fusion.

Lengthening of the lower extremity by the Wagner method. A review of the Boston Children's Hospital Experience.

Thirty-eight patients underwent forty lengthenings of the lower extremity between October 1972 and January 1979. There were twenty-four femoral and sixteen tibial lengthenings. The average length gained was 6.59 centimeters for the patients with femoral lengthenings and 4.77 centimeters for those with tibial lengthenings. While the over-all complication rate was high (92 per cent), it did not significantly affect the ultimate goal of equalization of limb length. We think that the Wagner method is the procedure of choice for continuous distraction lengthening when the severity of the limb-length inequality merits major surgical intervention.

Spinal deformity in children treated for neuroblastoma.

Of seventy-four children who were treated at a mean age of seventeen months for neuroblastoma and survived more than five years, fifty-six (76 per cent) had spinal deformity due either to the disease or to the treatment after a mean follow-up of 12.9 years. Of these fifty-six, 50 per cent had post-radiation scoliosis (mean, 18 degrees; range, 5 to 79 degrees), and 16 per cent had post-radiation kyphosis, most frequently at the thoracolumbar junction (mean, 39 degrees; range, 13 to 61 degrees), at the time of follow-up. Two kyphotic thoracolumbar curve patterns were identified: (1) an angular kyphosis with a short radius of curvature and its apex at the twelfth thoracic and first lumbar vertebrae, and (2) a thoracic kyphosis with a long radius of curvature that extended into the lumbar spine. The post-radiation deformity--both the scoliosis and the kyphosis--progressed with growth, the scoliosis at a rate of 1 degree per year and the kyphosis at a rate of 3 degrees per year. Epidural spread of the neuroblastoma was associated with most of the cases of severe scoliosis and kyphosis. The deformity was due either to the laminectomy or to the paraplegia acting in conjunction with the radiation. Eighteen per cent of 419 children with this malignant disease survived more than five years, and of the survivors, 20 per cent had spinal deformity severe enough to warrant treatment. The factors associated with the development of spinal deformity in patient treated for neuroblastoma were: (1) orthovoltage radiation exceeding 3000 rads, (2) asymmetrical radiation of the spine, (3) thoracolumbar kyphosis, and (4) epidural spread of the tumor.

Congenital pseudarthrosis of the tibia.

Forty cases of congenital pseudarthrosis of the tibia were reviewed. The results were assessed so as to emphasise function rather than simply the presence or absence of union. At the time of review, 14 patients had undergone amputation; eight others had non-union or tenuous union. No surgical procedure except the Farmer operation (a composite skin and bone pedicle graft from the other leg) showed any clear superiority. Among the factors associated with a poor result were considerable shortening, older children, and rapid resorption of the bone graft. It is felt that congenital pseudarthrosis of the tibia is a biological problem and not merely a mechanical one; consequently biological approaches to its treatment are needed.

Diastematomyelia and structural spinal deformities.

Sixty patients with diastematomyelia were seen over a thirty-year period and congenital scoliosis was found in 60 per cent. All of the patients had associated vertebral abnormalities and most (87 per cent) had a neural deficit. Myelography was helpful in the diagnosis, particularly prior to any procedure that might cause traction on the spinal cord. Laminectomy for removal of the spur was indicated when neural deficits were progressive or before corrective surgery on the spine, and in ten patients the operation alleviated neural sequelae. Observation of patients with diastematomyelia who have no neural deficit or a stable, non-progressing deficit is recommended.

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.

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.

Neurofibromatous scoliosis. Natural history and results of treatment in thirty-seven cases.

Of 400 patients with the diagnosis of neurofibromatosis on their hospital records, 141 actually had the disease. The presence of at least two of the following features was considered diagnostic: positive family history; positive biopsy; a minimum of six cafe-au-lait spots, each with a diameter of at least 1.5 centimeters; and multiple subcutaneous neurofibromas. Scoliosis was present in thirty-seven patients (26 per cent), most commonly associated with cafe-au-lait spots (thirty-five patients). In many of the patients with scoliosis there were associated medical and surgical problems. Although no standard pattern of spinal deformity could be identified, a sharp single right thoracic curve involving more than five vertebrae was the most common. For the whole group the initial measurement of the scoliosis averaged 42 degrees. Double curves were more sever, buth kyphosis was uncommon and no cases of paraplegia were recorded. In patients with progressive scoliosis, the best results were obtained with early Harrington instrumentation and posterior spine fusion. Progression of the scoliosis was observed both before treatment and postoperatively. The amount of progression was not necessarily related to the severity of other manifestations of neurofibromatosis, and was not significantly dependent on the length of the curve.

Skeletal alterations following irradiation for Wilms' tumor: with particular reference to scoliosis and kyphosis.

The roentgenographic changes in the axial skeleton after irradiation for Wilms' tumor were studied in eighty-one patients. In addition to the initial alterations found in the individual vertebrae within the field of irradiation, spinal deformity subsequently developed in fifty-nine patients (pure scoliosis in thirty-eight, kyphoscoliosis in nineteen, and pure kyphosis in two). Unitl the adolescent growth spurt these deformities tended to remain slight, but some progression did occur. In seven patients the scoliosis became severe enough to require spine fusion. A Milwaukee brace used in three patients failed to correct the curve. The trapezoidal shape of the vertebrae and scarring of the soft tissues within the concavity made correction difficult. Recognizable roentgenographic alterations failed to develop in twenty-two patients who, in general, were older and had received less irradiation. There appeared to be a correlation between the amount of irradiation and the severity of the spinal deformity (p is less than 0.05) and between the age of irradiation and the amount of deformity (p is less than 0.02).

Skeletal growth and development of the achondroplastic dwarf.

Certain patterns in growth and development can be seen in the typical achondroplastic dwarf. Height is approximatley 5 standard deviations below average size for age through the age of 14. Sitting height is within normal limits at all ages. On the average the femur is 8 standard deviations and the tibia 7 standard deviations below normal size. Compared to normal, both femur and tibia exhibit approximately a 50 per cent inhibition in growth. A slight decrease in the deviance from the norm is observed for all parameters during growth until the time of the adolescent growth spurt when an increased deviance is seen. Related to normal children of the same sex and age, females are more severely affected than male achondroplastic dwarfs. The skeletal development of the hand is irregular, often having extra metacarpal epiphyses and a wide variance in skeletal maturation assessment between the carpals and the rest of the hand. The skeletal age is generally below chronological age until the adolescent growth spurt when a rapid maturation pattern occurs. Standard skeletal age assessment techniques cannot be applied to the achondroplastic individual.

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.