Protrusio acetabuli: its occurrence in the completely expressed Marfan syndrome and its musculoskeletal component and a procedure to arrest the course of protrusion in the growing pelvis.

Protrusio acetabuli [center-edge (C/E) angle > 60 degrees, teardrop collapsed, and pelvis invaded by acetabulum beyond ilioischial line (Köhler's)] was identified in 22 patients with classic Marfan syndrome. All had a preponderance of the syndrome's cardinal features (positive pedigree and musculoskeletal, ocular, and cardiovascular manifestations). Fifty-two immature patients with the syndrome's skeletal expressions predominating were studied for deep acetabuli. Half of these individuals (26 patients) had true protrusio acetabuli. Thirty-one percent (16 patients) had abnormal acetabular deepening (C/E angle > 50 degrees; teardrop partially collapsed without acetabular protrusion); 19% (10 patients) had normal pelves with C/E angle < 40 degrees and normal teardrop. To arrest the protrusio, the triradiate physis was closed surgically in 11 patients (21 hips), one unilaterally. In 19 operated-on hips followed-up to maturity by using teardrop, C/E angle, and acetabular relation to the ilioischial line as criteria, architecture was restored to normal in 12 hips and reduced from true protrusio to simply acetabular deepening in four. Three were unchanged. The youngest child operated on was 8; the oldest was 12 years. Laboratory examination of the physes was unrewarding.

Slipped capital femoral epiphysis. The prevalence of late contralateral slip.

We evaluated the prevalence of slipped capital femoral epiphysis in the contralateral hip of 169 children who had been managed with pinning in situ and thirty who had been managed with immobilization in a spica cast. Only children who had initially been seen with a unilateral slip and had been followed for a minimum of two years or until skeletal maturity were included in the study. The average duration of follow-up was 3.6 years (range, 0.5 to 9.5 years) for the group that had been managed with a cast and 2.8 years (range, 1.0 to 8.3 years) for the group that had been managed operatively. In sixty-one (36 per cent) of the 169 patients who had had operative treatment and two (7 per cent) of the thirty who had been managed with a spica cast, a slip subsequently developed in the contralateral hip; this difference was significant (p = 0.001). On the basis of these findings, we recommend that closer attention be paid to the potential development of a slip in the contralateral hip after pinning.

Triple innominate osteotomy for hip stabilisation and transiliac leg lengthening after poliomyelitis.

We reviewed our experience with a modified triple innominate osteotomy for hip instability and limb shortening due to poliomyelitis in 62 adolescent and adult patients, treated from 1973 to 1990. Their ages at surgery ranged from 12 years to 35 years (average 22.3). At a mean follow-up of 4 years (2 to 18) 59 of the patients (95.2%) had substantial improvement in hip stability, and all but one had radiological improvement as determined by the acetabular angle, centre-edge angle and acetabulum-head quotient. In 59 cases in which transiliac limb lengthening was attempted, the mean gain was 1.7 cm (0.6 to 3.0). When the abductor muscles had been partially paralysed, the operation produced an appreciable increase in power in 12 of the 39 hips examined.

A syndrome of dislocated hips and radial heads, carpal coalition, and short stature in Puerto Rican children.

An orthopaedic syndrome that apparently had not been reported previously was identified in twenty-three children. Characteristics shared by all twenty-three children included Hispanic descent, residence in Puerto Rico, bilateral dislocation of the hip, dislocated radial heads, short stature, and other osseous anomalies. Twelve dislocated hips in six patients were not treated. All of these hips were functioning satisfactorily at the time of the review, but only four of the children had reached skeletal maturity. Sixteen hips in eight patients remained reduced after closed reduction. Of these eight patients, the four who were skeletally immature at the time of the review had a satisfactory result, and the four who were skeletally mature had an unsatisfactory result because of discomfort or fibrous ankylosis. Eighteen hips in nine patients were treated with a reduction augmented by some form of operation. All of these hips redislocated. Of the forty-six elbows in the twenty-three children, thirty-three were dislocated, as seen clinically and radiographically; eight were normal, both clinically and radiographically; and there was dysplasia at the radiocapitellar articulation of the remaining five. Twenty of the twenty-three children were found to have carpal coalitions. Fourteen children had scoliosis, and five of them were managed with spinal arthrodesis and correction. Three patients had an anomaly of the cervical spine, with one deformity causing symptoms and signs that were treated with decompression. Eight patients had talipes cavus bilaterally, which was not treated.

Relationship between acquired spastic talipes equinovarus and dystonia musculorum deformans.

Acquired spastic talipes equinovarus associated with dystonia musculorum deformans (DMD) presents orthopaedic surgeons with a difficult treatment problem. We reviewed the natural history, diagnostic evaluation, and treatment of this deformity in 12 patients. Two patients initially had the generalized form of dystonia, and 10 patients had the focal form (in which acquired spastic talipes equinovarus was the only manifestation). With time, five of these 10 developed the generalized form. A positive family history and age < 14 years at onset were prognostic of this progression.

Both-bone forearm fractures in children.

Treatment of both-bone forearm fractures remains a difficult dilemma for the orthopedist. We assessed the results of 33 children treated with closed reduction and a long-arm cast using traction with finger traps in neutral rotation. Patients were grouped according to age and analyzed for residual angulation and range of motion (ROM). Despite some residual angulation and loss of rotation, all patients were fully active with no functional restriction at follow up. For patients 10 years old or younger with less than 30 degrees of post-casting angulation, full ROM and minimal residual angulation on radiograph can be expected. For patients ages 11 to 15 years (the oldest patient in this series was 15), residual angulation is likely and approximately 60% of patients will have residual loss of less than or equal to 30 degrees of rotation. In spite of this, we found no evidence of functional loss at follow up. Our series supports the continued use of closed manipulation as an effective treatment in children with both-bone forearm fractures.

Index finger pollicization for congenital aplasia or hypoplasia of the thumb.

Sensation, strength, dexterity, length, and range of motion were evaluated after index finger pollicization in 10 patients (14 hands). Diagnoses included congenital absence of the thumb (10 hands) and hypoplasia (4 hands). Average age at operation was 7 years, and follow-up averaged 9 years. Patients with unilateral pollicization averaged 67% grip strength, 60% lateral pinch, 56% palmar pinch, and 39% three-point pinch as compared with the normal contralateral hand. Manual dexterity averaged 70% efficiency as compared with normal standards defined according to age and sex. However, 55% of the patients, when stressed, used side-to-side pinch. It was noted that in those patients who used side-to-side pinch performance averaged 54% of normal standards, compared with 93% in patients who used tip-to-tip pinch for prehension.

Treatment of slipped capital femoral epiphysis. Spica-cast immobilization.

Thirty-two patients (thirty-seven hips) who had a so-called acute-on-chronic or chronic slipped capital femoral epiphysis were treated with traction for relief of symptoms and then with immobilization in a spica cast for eight to sixteen weeks. The disappearance on radiographs of a metaphyseal juxtaphyseal radiolucency, rather than closure of the physis, was used as the criterion for removing the cast. In one (3 per cent) of the thirty-seven hips, the slip progressed; possibly this could have been prevented by keeping the cast on for a longer period of time. Narrowing of the cartilage space was seen after treatment in a cast in seven (19 per cent) of the thirty-seven hips. In five of these seven hips, this was true chondrolysis; in one, the diagnosis of chondrolysis had been apparent before treatment. Avascular necrosis did not develop as a result of treatment in any patient. Treatment in a spica cast should be considered as an alternative for patients who have an acute-on-chronic or chronic slipped capital femoral epiphysis.

Scoliosis in pediatric spinal cord-injured patients.

One hundred thirty children who sustained spinal cord injuries between birth and age 21 years were reviewed to determine the progression rate of paralytic scoliosis and the effects of bracing and surgery. Patients were divided into two groups: those injured before and those injured after the adolescent growth spurt. Scoliosis developed in 97 and 52%, respectively. Bracing was effective in delaying progression in the preadolescent group. The progressive paralytic spinal deformity did not appear to be related to the level of injury. The older patient is at much less risk for paralytic scoliosis, but still requires routine examination.

Congenital pseudarthrosis of the tibia. Long-term follow-up study.

Review of the literature reveals how difficult it is to assess the results of treatment of pseudarthrosis of the tibia. There is disagreement as to when the result can be considered final. This study reviewed the long-term results of treatment to determine if skeletal maturity could be considered the definitive end point of treatment or if the results deteriorate past skeletal maturity. In addition, the effect of neurofibromatosis on pseudarthrosis of the tibia is analyzed. Forty-one patients were reviewed. Only 25 had sufficient follow-up data to be included in this study. Eighteen of the 25 had neurofibromatosis. The results were classified according to criteria developed by Morrissy et al. At skeletal maturity, there were ten good results, three fair results, and three poor results, with nine patients having had amputation. At long-term follow-up evaluation (average 36 years; range five to 62 years), one patient with a fair result had elected amputation. About one half of the patients with neurofibromatosis required amputation. This study suggests that the results at skeletal maturity are reliable indicators of long-term results.

Scoliosis surgery in neurofibromatosis.

Twenty-three patients who were treated by posterior spinal fusion with neurofibromatous scoliosis were reviewed to study the adequacy of spinal fusion, rate of pseudarthrosis, and incidence of complications. Twenty patients achieved a solid fusion with posterior surgery alone. Thirteen patients required one or more posterior augmentation procedures because of progressive deformity. Three patients with dystrophic kyphoscoliosis required an anterior spinal fusion in addition to the posterior fusion to achieve a solid fusion mass. The type of graft material, Harrington instrumentation, and degree of kyphosis or scoliosis had no effect on the rate of pseudarthrosis. Preoperative neuroradiographic evaluation was found to be warranted for all patients with neurofibromatous scoliosis.

Juvenile idiopathic scoliosis followed to skeletal maturity.

The authors reviewed 43 patients with the onset of idiopathic scoliosis between 4 and 9 years who were followed to skeletal maturity to document the natural history, effects of bracing, and factors associated with progression. Average age at onset was 7.1 years for female and 6.7 for male patients. Twelve were treated with observation only; six improved, five progressed, and one refused an orthosis and required surgery. Thirty-one patients wore an orthosis; five patients improved or were unchanged, and 26 progressed, including 13 who subsequently required surgery. All patients treated with observation had a rib vertebral angle difference (RVAD) of less than 10 degrees; whereas patients progressing despite orthosis and all but one patient requiring operation had an RVAD greater than 10 degrees. Thoracic hypokyphosis (less than 20 degrees) was present in 20% of observed patients, 64% of those treated with an orthosis, and 78% of those requiring operation.

Iliofemoral fusion for proximal femoral focal deficiency.

Iliofemoral fusion, as performed for proximal femoral focal deficiency, is designed so that the knee, acting at the level of the triradiate cartilage, will act as the hip. This procedure was indicated in four of our patients to promote fitting of a prosthesis. The results after follow-up of more than five years are reported. Two of the patients also had a Van Nes rotationplasty and two had a Syme amputation. The Syme amputation produced better results. Distal epiphyseodesis of the ipsilateral femur also was performed in three of the four patients to minimize the anterior prominence of the knee. Two patients required a closing wedge osteotomy of the distal part of the femur to improve the alignment. All four patients walked well as functional above-the-knee amputees.

Magnetic resonance imaging (MRI) in the evaluation of spinal cord injured children and adolescents.

In order to determine the indications and usefulness of MRI scanning in evaluating spinal cord trauma, MRIs on 43 subacute and chronic spinal cord injured children were compared with CT myelograms and other diagnostic tests. MRI scans were superior to CT myelograms in evaluating post-traumatic syrinx, disc pathology and the physiological status of the cord. CT myelogram remains an essential study before considering spinal cord decompression. The presence of internal fixation is not a contraindication to MRI scanning.

Bifurcated femur with a normal tibia and fibula.

Bifurcation of the distal femur is not a common anomaly; there have been only 27 cases of bifurcated femur with tibial hemimelia reported in the literature. This is the first report of a bifurcated femur associated with a normal tibia, fibula, ankle, and foot.

The metaphyseal blanch sign of slipped capital femoral epiphysis.

In the early stage of slipping of the capital femoral epiphysis, the epiphysis slips posteriorly in relation to the neck of the femur. When this occurs, the posterior portion of the femoral head is seen on the anteroposterior radiograph as a crescent-shaped area of increased density overlying the metaphysis adjacent to the epiphyseal plate. This metaphyseal blanch sign suggests that slipping of the capital femoral epiphysis has occurred and that a lateral radiograph should be made to confirm or exclude the diagnosis.

Congenital diastasis of the inferior tibiofibular joint: a review of the literature and report of two cases.

Congenital inferior tibiofibular diastasis is a rare condition of unknown etiology, possibly a form of tibial hypoplasia, marked by a clinical triad of talipes equinovarus, ankle diastasis, and limb length inequality. An analysis of 10 cases reported previously is given with two additional examples. Ankle mortise reconstitution and clubfoot correction by surgery have been the basis of treatment. We present two cases in which no ankle reconstruction was done with good results. Syme amputation is considered for cases with severe tibial shortening. Prognosis for a plantigrade foot is encouraging if limb length inequality is corrected operatively or non-operatively as indicated.

The effect of an incomplete interochanteric osteotomy on Legg-Calvé-Perthes disease.

In an attempt to accelerate the healing process, an incomplete intertrochanteric osteotomy was performed over a ten-year period in fifty-three patients (fifty-four hips) with Legg-Calvé-Perthes disease. Radiographs of the hips that had been operated on were reviewed to establish the length of time that was required to attain completion of bone-healing of the femoral head and completion of the resorption stage of the disease. These healing times were then compared with those of a control group of thirty-six patients (forty-three hips) who had had non-operative treatment for Legg-Calvé-Perthes disease in our institution, as well as with the data from other studies on this subject in the orthopaedic literature. In our experience, an incomplete intertrochanteric osteotomy did not in any way alter the rate of healing of Legg-Calvé-Perthes disease.