SPECT imaging of para-axial neurofibromatosis with technetium-99m DTPA.

Single photon emission computed tomography (SPECT) was used to study uptake of technetium-99m diethylenetriaminepentaacetic acid ([99mTc]DTPA) by para-axial neurofibromas in 13 patients. SPECT imaging led to better resolution of uptake in nine instances and detection of 12 lesions unsuspected on planar imaging (PI). Two false-positive instances and one false-negative instance of uptake are described. The planning of the surgical approach and placement of graft material for spinal fusion was assisted by SPECT in two patients. One patient with disability secondary to recurring abdominal pain had detection of the causative lesion by SPECT and subsequent relief of her symptoms following excision of the tumor. SPECT imaging of soft-tissue tumors of neurofibromatosis appears to have potential use in preplanning surgery on structural or cosmetic lesions, in the detection of occult lesions, and the monitoring of patients with neurofibromatosis at regular intervals.

Monoaminergic responses to spinal trauma. Participation of serotonin in posttraumatic progression of neural damage.

The monoamines norepinephrine (NE), dopamine (DA), and serotonin (5-HT) and their major metabolites were measured in the spinal cord of rabbits following laminectomy or impact injury to the thoracic cord. Samples were taken 30 minutes, 60 minutes, 4 hours, and 6 weeks after injury. Utilization ratios (metabolite/transmitter) were calculated from the data. Turnover rates for NE and DA were also calculated at 30 minutes using the alpha-methylparatyrosine method. Trauma resulted in rapid and sustained elevations in 5-HT concentration at and around the injury site. The catecholamines were depleted slightly at the injury site. Levels of 5-hydroxyindole-3-acetic acid were elevated at 30 minutes but fell to baseline by 4 hours, resulting in a decrease in the 5-HT utilization ratio. The utilization and turnover of NE was increased at the injury site, while DA function was not affected. The large short-term increase in 5-HT levels may have been due to extravasation of platelet 5-HT stores into spinal tissue, rather than due to changes in neuronal 5-HT metabolism. At 6 weeks after injury, each monoamine and metabolite appeared to accumulate in spinal cord tissue proximal to the insult. Distal to the injury, depleted amine stores displayed augmented utilization. The data are discussed in terms of a serotonergic hypothesis of the progression of neural damage after trauma, with the interaction of 5-HT with raphe-spinal nerve terminals as a principal event.

Growth disturbance of the proximal part of the femur after treatment for congenital dislocation of the hip.

The radiographs of ninety patients in whom treatment of unilateral congenital dislocation of the hip was complicated by disturbance of growth of the proximal part of the femur were studied retrospectively. All patients were followed until closure of the affected proximal femoral physis. We divided the patients into three groups, according to the degree of vascular insufficiency: patients who had mild vascular insufficiency of the hip, which had little effect on growth; those who had moderate vascular insufficiency, which produces partial arrest of growth; and those who had severe vascular insufficiency, which causes complete arrest of growth. Good correlation was found between the initial degree of vascular insufficiency and the radiographic results at the most recent follow-up. The radiographic signs that were used to predict the extent of physeal involvement were a crescent-shaped epiphysis, medial bowing of the femoral neck (a shorter and more concave curve between the lesser trochanter and the proximal femoral metaphysis [the lateral portion of the Shenton line]), lateral tilting of the capital epiphysis, and premature physeal closure. Signs that were diagnostic of existing physeal involvement were elevation of the greater trochanter and shortening of the affected extremity. The presence and severity of these signs correlated well with the degree of vascular insufficiency. Medial bowing was the most reliable prognostic factor for the determination of the fate of the hip joint at maturity.

Treatment of congenital dislocation of the hip in children between the ages of one and three years.

The results in fifty-one congenitally dislocated hips in forty-two children who were between one and three years old when treatment was begun have been reviewed. Thirty-eight hips (75 per cent) were treated by traction prior to reduction, gentle closed reduction under anesthesia, selective adductor tenotomy, and immobilization in a hip-spica cast. Thirteen hips (25 per cent) required an open reduction when stable closed reduction could not be achieved. Secondary femoral or acetabular procedures were performed after either form of treatment if subluxation became apparent after the child had resumed walking. At an average twelve-year follow-up (range, five to twenty-two years), thirty-six hips (71 per cent) were rated as Class I; six, as Class II; eight, as Class III; and one, as Class IV, according to the classification system of Severin. Significant avascular necrosis developed in three hips. We think that in this age group congenital dislocation of the hip is best treated by closed reduction, followed by femoral or acetabular procedures as needed. Open reduction should be done only in those hips that cannot be reduced by closed methods.

Spontaneous improvement of post-traumatic tibia valga.

We reviewed the cases of seven children with post-traumatic tibia valga in order to determine whether spontaneous improvement of this deformity occurs with growth. The children were between eleven months and six years and four months old at the time of injury and were followed for an average of thirty-nine months after the fracture. The valgus deformity appeared to progress during the period of fracture-healing as well as after union of the fracture, as determined clinically and radiographically. The angulation progressed most rapidly during the first year after the injury, and then continued at a slower rate for as long as seventeen months. Overgrowth of the tibia by as much as 1.7 centimeters accompanied the angular deformity. Adequate clinical correction then occurred spontaneously in six of the seven patients. Because this spontaneous improvement of the deformity usually occurred with growth, we recommend a conservative approach to the management of both the acute fracture and the subsequent valgus deformity.

Avascular necrosis following treatment of congenital dislocation of the hip.

A review of 119 patients with congenital dislocation of the hip complicated by avascular necrosis, of whom fifty-one patients were skeletally mature, showed that damage to the physis was very common. Changes in the secondary ossification center (ossific nucleus) alone were found to be of very little value in predicting the nature of the development of the hip, while the change in the proximal femoral physis was the key to predicting residual deformity. The vascular disturbances were classified into four groups depending on the amount of damage involving the ossific nucleus and the physis during treatment of the dislocation. This classification was found to be accurate in predicting the natural history of avascular necrosis. The more severe forms of avascular necrosis were found to be most prevalent in those patients in whom treatment was begun between birth and the age of six months. We also found that preliminary traction and the use of general anesthesia reduced the incidence of the more severe form of avascular necrosis. The functional results found in the skeletally mature patients coincided with the types of vascular changes; the presence of avascular necrosis enhanced the development of arthritis, especially if residual dysplasia and subluxation also were present.

Treatment of Legg-Calvé-Perthes disease. Prognostic value of Catterall's Classification.

Catterall's classification of Legg-Calvé-Perthes disease is of prognostic value. When indicated, early energetic treatment in the form of containment and achievement of a full range of motion significantly decreases the percentage of poor results.

Osteochondritis dissecans in Legg-Calvé-Perthes disease.

Seven cases of osteochondritis dissecans following Legg-Calvé-Perthes disease are presented and the literature is reviewed. This complication could not be predicted during the early stages of the Legg-Calvé-Perthes process. A high index of suspicion is necessary to recognize the lesion. Conservative treatment is suggested.

Spinal deformity associated with heritable neurological conditions: spinal muscular atrophy, Friedreich's ataxia, familial dysautonomia, and Charcot-Marie-Tooth disease.

Children with progressive neurological conditions such as spinal muscular atrophy, Friedreich's ataxia, familial dysautonomia (Riley-Day syndrome), and Charcot-Marie-Tooth disease have a significant risk of acquiring a serious spinal deformity. As with paralytic scoliosis following poliomyelitis, the curves are difficult to control with bracing, and progression does not cease with maturation. An increasing spinal curvature may lead to loss of ambulation or, for the wheelchair-bound patient, loss of sitting balance. The curvature may further compromise pulmonary function that may already be compromised by the neurological deficit. Twenty patients are reported with an average follow-up of six years (range, one to fourteen years). For the properly selected patient, surgical stabilization of the spine arrested the progress of the curve and improved function. Complications were few; however, pseudarthrosis was more common than in patients with non-neurological problems.

Cervical spine abnormalities in neurofibromatosis.

Fifty-six patients with neurofibromatosis were examined for abnormalities of the cervical spine and seventeen of them had demonstrated lesions there. Of the thirty-four patients who had scoliosis or kyphoscoliosis, fifteen (44 per cent) had cervical lesions. Many of the patients with those lesions were asymptomatic. To avoid the complications attributable to the cervical spine, we recommend roentgenographic examination in all neurofibromatosis patients who are about to have general anesthesia or skull traction for treatment of scoliosis.

Congenital dislocation of the hip. Use of the Pavlik harness in the child during the first six months of life.

From 1968 to 1972, twenty-three infants under six months old with twenty-seven dislocated hips were treated with a Pavlik harness. All the dislocations except three were successfully reduced. Only one child required hospitalization. All the patients were followed for more than two years. All but three of the hips were clinically and roentgenographically normal at follow-up, and none had avascular necrosis. In infants, the Pavlik harness successfully utilizes the principle of reduction in flexion, avoiding forced abduction.

Acute neurological complications in the treatment of scoliosis. A report of the Scoliosis Research Society.

A survey conducted by the Scoliosis Research Society found eighty-seven patients with acute neurological complications resulting from the treatment of scoliosis. The incidence of these complications was determined to be 0.72 per cent. Seventy-four major complications involving the spinal cord were reported, half of them complete paraplegia and half partial paraplegia. Thirty-six per cent recovered completely, 32 per cent had partial recovery, and 32 per cent had no return of function. Thirteen minor complications involving cranial and peripheral nerves were reported. Major complications occurred in forty-two cases of posterior spine fusion with Harrington instrumentation and in twenty cases of posterior spine fusion without instrumentation. Six patients became paraplegic following skeletal traction alone.

The use of plastic jackets in the non-operative treatment of idiopathic scoliosis. Preliminary report.

One method of treatment for scoliosis consists of using a custom-made plastic underarm jacket made of Orthoplast. The results of treatment in the first forty-eight patients with idopathic scoliosis to complete the treatment program showed arrest of the progression of the curve in forty-two. Comparisons were made with the Milwaukee brace. The key prognostic factors for success appeared to be the severity and flexibility of the curve at the start of treatment.

Orthopaedic aspects of the VATER association.

The cases of twenty-eight patients with the VATER association, which consists of various combinations of vertebral anomalies, anal atresia, tracheoesophageal fistula with esophageal atresia, radial dysplasia, and renal anomalies, were reviewed. Associated anomalies included deformities of the ribs and lower limbs, a two-artery umbilical cord, a congenital heart defect, and auricular deformities. Three children died in the first two years of life. Of the remaining twenty-five patients, twelve have required a total of seventeen orthopaedic procedures for the treatment of congenital scoliosis or deformity of the upper or lower extremities.

Treatment of idiopathic scoliosis with the Wilmington brace. Results in patients with a twenty to thirty-nine-degree curve.

The results in seventy-nine adolescent patients (ninety-five curves) who had idiopathic scoliosis treated with the Wilmington brace are reported. The average follow-up was two years and six months (range, one to nine years). Before treatment, all of the patients had a curve that measured 20 to 39 degrees and a Risser sign of zero or 1. Although the magnitude of the curve was generally reduced by about 50 per cent with the initial application of the brace, a gradual loss of this initial improvement was observed both during active treatment and after the patient was weaned from the brace. Although twenty-seven (28 per cent) of the curves had progressed more than 5 degrees at follow-up, Lonstein and Carlson reported that the projected probability of progression of untreated 20 to 29-degree curves is 68 per cent. Thirty-six per cent of the thoracic curves, 16 per cent of the thoracolumbar and lumbar curves, and 28 per cent of the double major curves had progression of more than 5 degrees. However, only 11 per cent of the patients had a curve that progressed sufficiently to warrant fusion. Our findings indicate that the Wilmington brace favorably alters the natural history of 20 to 39-degree idiopathic curves.

Klippel-Feil syndrome; a constellation of associated anomalies.

Of fifty patients with the diagnosis of Klippel-Feil syndrome, less than half had the classic clinical triad of findings, while more than half had scoliosis, and a third had renal anomalies. All patients were at risk of having other serious, but less apparent, anomalies, including: Sprengel's deformity (twenty-one patients), impairment of hearing (fifteen patients), synkinesia (nine patients), and congenital heart disease (seven patients). The discovery of one of these lesions should, therefore, stimulate a careful search for associated anomalies.

Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment.

We reviewed the long-term outcomes of treatment of fibrous dysplasia of the proximal part of the femur in twenty-two patients (twenty-seven femora). There were fifteen male patients and seven female patients. Patients who had monostotic disease had no involvement of the calcar femorale, fewer microfractures, less deformity, and stronger bone that could support internal fixation. Patients who had polyostotic disease had frequent involvement of the calcar femorale; more microfractures; severe deformity, including shepherd's crook deformity; and, in many instances, bone that could not support internal fixation. Twenty-two of the twenty-seven femora had a microfracture at the time of the initial presentation. At least one osteotomy was performed in four femora that had monostotic disease and in nine femora that had polyostotic disease. Curettage and cancellous or cortical bone-grafting did not appear to have any advantage compared with osteotomy alone in the treatment of symptomatic lesions, as all grafts resorbed with persistence of the lesion. At the time of the latest follow-up evaluation, no lesion had been eradicated or had decreased in size. A satisfactory clinical result was achieved in twenty patients (twenty-four femora): nine who had monostotic disease and eleven who had polyostotic disease. Two patients who had polyostotic disease and an endocrinopathy (one of whom had bilateral involvement) had an unsatisfactory result. All three femora in these two patients had a neck-shaft angle of less than 90 degrees at the time of the most recent follow-up evaluation. Varus deformity of the proximal part of the femur is best treated with valgus osteotomy and internal fixation early in the course of the disease. If the calcar of the femoral neck is involved or if the quality of the bone is such that internal fixation is not possible, a medial displacement valgus osteotomy can provide a more mechanically favorable position for healing of the microfracture.

Varus derotation osteotomy in the treatment of persistent dysplasia in congenital dislocation of the hip.

We have attempted to define the indications for and limitations of varus derotation osteotomy in the treatment of persistent dysplasia in congenital dislocation of the hip. We studied the cases of thirty-four patients (forty-four hips) who were divided into three groups according to age at operation, and evaluated the influence of femoral remodeling, age, acetabular response, instability, and pre-existing avascular necrosis with respect to the final results. The length of follow-up ranged from five to twenty-two years. Rapid return to a valgus femoral neck-shaft angle by remodeling was not a cause of failure in any age group. There were consistently good results in the patients who were less than four years old at the time of operation. Acetabular correction by remodeling occurred through the age of eight years, but four of thirteen hips in patients who were between the ages of four and eight showed persistent dysplasia despite the operative procedure. The results were less predictable as the patients approached the age of eight years. There was no benefit from isolated femoral osteotomy in ten of eleven hips in patients who were older than eight. Pre-existing avascular necrosis appeared to compromise the results of the procedure in all age groups.

Chiari pelvic osteotomy in children and young adults.

We conducted a follow-up study of twenty-four patients who had been treated with a Chiari osteotomy at the Alfred I. duPont Institute between 1966 and 1981. The length of follow-up ranged from three to twenty years, and the age at operation ranged from ten to twenty-three years. The indication for the operation was either painful dysplasia or gross instability of the hip. Twelve patients had had congenital dislocation of the hip; six, poliomyelitis; three, cerebral palsy; and three had had another disorder. A good or excellent result was obtained in twenty-one of the twenty-four patients. Preoperative pain and antalgic gait were consistently improved. In twenty-one patients, the osteotomy had to be displaced more than 50 per cent to provide adequate coverage of the femoral head, and bone-grafting was necessary at the site of the osteotomy to prevent problems with healing.

Scoliosis and pregnancy.

The effects of pregnancy on patients who have idiopathic scoliosis were investigated in terms of increased risk of progression of the curve. The charts, radiographs, and other pertinent data on 355 affected women who had reached skeletal maturity (Risser Grade 4) before 1975 were reviewed and analyzed. One hundred and seventy-five patients had had at least one pregnancy each (Group A) and 180 patients had never been pregnant (Group B). The groups were comparable with regard to the treatments that they had received. After skeletal maturity was reached, the curve progressed more than 5 degrees in 25 per cent and more than 10 degrees in 10 per cent of the patients in each group. The age of the patient at the time of the first pregnancy did not influence the risk of progression, and the stability of the curve before pregnancy did not decrease the risk of its progression during pregnancy. In patients who had had a spinal fusion, progression in the unfused portion of the spine was negligible in both Group A and Group B. The presence of a pseudarthrosis did not result in progression of the curve during pregnancy. The effects of scoliosis on pregnancy and delivery were evaluated in the 175 women in Group A. No specific problems that were directly related to the scoliosis were noted except for four patients, in whom delivery posed difficulties. The incidence of cesarean section was one-half of the national average, and no sections were directly related to the mother's scoliosis.(ABSTRACT TRUNCATED AT 250 WORDS)