The material basis for reduced mechanical properties in oim mice bones.

Osteogenesis imperfecta (OI), a heritable disease caused by molecular defects in type I collagen, is characterized by skeletal deformities and brittle bones. The heterozygous and homozygous oim mice (oim/+ and oim/oim) exhibit mild and severe OI phenotypes, respectively, serving as controlled animal models of this disease. In the current study, bone geometry, mechanics, and material properties of 1-year-old mice were evaluated to determine factors that influence the severity of phenotype in OI. The oim/oim mice exhibited significantly smaller body size, femur length, and moment of area compared with oim/+ and wild-type (+/+) controls. The oim/oim femur mechanical properties of failure torque and stiffness were 40% and 30%, respectively, of the +/+ values, and 53% and 36% of the oim/+ values. Collagen content was reduced by 20% in the oim/oim compared with +/+ bone and tended to be intermediate to these values for the oim/+. Mineral content was not significantly different between the oim/oim and +/+ bones. However, the oim/oim ash content was significantly reduced compared with that of the oim/+. Mineral carbonate content was reduced by 23% in the oim/oim bone compared with controls. Mineral crystallinity was reduced in the oim/oim and oim/+ bone compared with controls. Overall, for the majority of parameters examined (geometrical, mechanical, and material), the oim/+ values were intermediate to those of the oim/oim and +/+, a finding that parallels the phenotypes of the mice. This provides evidence that specific material properties, such as mineral crystallinity and collagen content, are indicative and possibly predictive of bone fragility in this mouse model, and by analogy in human OI.

Selective peripheral denervation for torticollis: preliminary results.

Herein we report the preliminary results in nine patients who have undergone selective peripheral denervation for spasmodic torticollis and have been followed up for at least 13 months. All patients had improvement immediately after surgical intervention, and the results have been maintained in five patients. In one patient who had recurrent torticollis, a second procedure in conjunction with injection of botulinum toxin has produced substantial improvement; however, follow-up was brief (6 months). No surgical complications occurred. We believe that selective peripheral denervation is safe and that it can benefit patients with torticollis who have not responded to other types of therapy. These favorable results confirm other published reports on the efficacy of selective peripheral denervation. Long-term follow-up, however, is necessary for determining the role of this procedure in the management of torticollis.

ECT and tardive dyskinesia: two cases and a review.

A brief review of the literature on electroconvulsive therapy in the management of tardive dyskinesia is presented and two patients are described who were treated for major functional disorders with ECT and showed dramatic remissions in tardive dyskinesia. This response was noted during the course of ECT and has been sustained at 1-year follow-up.

The prevalence of tardive dyskinesia in geropsychiatric outpatients.

The prevalence of tardive dyskinesia was studied in a geropsychiatric outpatient clinic. All currently active patients were examined using the Abnormal Involuntary Movement Scale (AIMS) and a standardized procedure. Data were collected on a number of previously reported risk factors. A higher prevalence was noted in both tardive dyskinesia and spontaneous involuntary movement disorders in this patient population. Only sex and duration of exposure to antipsychotic drugs were noted to be significant risk factors.

A musculoskeletal outreach screening, treatment, and education program for urban minority children.

PURPOSE: A hospital-based outreach program was initiated to screen minority children in medically underserved areas of New York City for musculoskeletal diseases. We examine the number of such diseases in this population, and evaluate the program's success to facilitate referral and follow-up of children with referral conditions. METHODS: Screenings were conducted at schools and day-care centers. Children requiring further evaluation were referred to the sponsoring hospital, a major referral center for musculoskeletal diseases. Bilingual educational strategies, transportation reimbursement, and coverage for uninsured children were used to foster participation and increase follow-up. RESULTS: A total of 2,523 children were screened, 168 (6.7%) of whom were referred for one of 45 different musculoskeletal disorders, including scoliosis and back problems, foot problems, in- and out-toeing, knee or hip pain, and problems of joint range of motion. Sixty-seven percent of those referred had a follow-up medical consultation. CONCLUSIONS: A substantial proportion of urban minority children have previously undiagnosed musculoskeletal disorders that, if left untreated, have the potential to lead to significant disability in later life. Targeted screening programs can be effective in identifying such disorders, and providing and opportunity for early diagnosis, treatment, and education.

Identification of the oim mutation by dye terminator chemistry combined with automated direct DNA sequencing.

The homozygous oim/oim mouse, a model of moderate-to-severe human osteogenesis imperfecta, contains a G-nucleotide deletion in the Cola-2 gene (the murine pro alpha(I) collagen gene) that results in accumulation of alpha1(I) homotrimer collagen. Although these mice have a distinctive phenotype that includes multiple fractures and deformities, genotyping is necessary to distinguish them from their wildtype (+/+) and heterozygote (oim/+) littermates. In this study, the dye primer and dye terminator chemistry methods, in combination with automated direct DNA sequencing, were compared for accuracy and ease in genotyping. A total of 82 mice from 14 litters were bred and genotyped; this resulted in 18 +/+, 35 oim/+, and 29 oim/oim mice. The dye primer and dye terminator chemistry methods worked equally well for identification of the deletion mutation and thus the genotype of all of the mice. However, the dye terminator method was found to be superior on the basis of the reduced amount of sample handling and reduced quantity of reagent required.

Results of surgical treatment for growth hormone-secreting pituitary adenomas.

The results of surgical therapy for acromegaly were reviewed in a series of 175 patients treated between 1972 and 1983. Patients with prior surgery or radiation therapy were excluded from the study. Postoperative radiation therapy was given to 54 patients. The criterion of achieving a postoperative basal or glucose-suppressed growth hormone level of 2 ng/ml or less was used to indicate remission. Utilizing the most recently available growth hormone determinations, 90 (51.7%) of 174 patients were in remission. The actuarial probability of remission at 1 and 5 years after surgery was 48.8% and 62.7%, respectively. Tumor size and the preoperative basal growth hormone level were correlated with outcome. Surgical excision of a pituitary adenoma is the most effective therapy currently available for acromegaly.

Osteotomy of the hip in children: posterior approach.

The posterior approach for varus rotational osteotomy of the hip with the patient prone is relatively bloodless, provides excellent visualization of the osteotomy site, and facilitates insertion of internal fixation devices. We performed this operation on 100 hips in fifty-nine patients. All but one of the osteotomies healed within eight weeks and there was one infection.

Hip adductor transfer compared with adductor tenotomy in cerebral palsy.

In a ten-year study in patients with cerebral palsy, fifty patients had ninety-eight adductor transfers and fifty-two patients had 102 adductor tenotomies with or without obturator neurectomy. The groups were similar with regard to severity of their disease, age, and associated concomitant surgery. Results were evaluated in three ways: functional change, change in passive motion of the hip, and change in stability of the hip. Our data support the view that although the adductor transfer operation takes longer and is associated with a higher incidence of postoperative drainage, the over-all improvement is greater and is maintained better than that after adductor tenotomy with or without neurectomy. The transferred muscle provides greater pelvic stability, decreases hip-flexion contractures, and reduces instability of the hip.

Posterior tibial-tendon transfer in patients with cerebral palsy.

The results of fifty-seven posterior tibial-tendon transfers through the interosseous membrane to the dorsum of the foot that were performed in fifty-one patients who had cerebral palsy, and who were followed for a mean of 9.3 years (range, five to twenty-six years), were evaluated in terms of pattern of gait, alignment of the foot, formation of callus on the sole of the foot, and requirements for bracing. A good or excellent result was achieved in twenty-seven of thirty feet in the hemiplegic patients, twelve of sixteen feet in the paraplegic patients, and two of eleven feet in the quadriplegic patients. We found that in order for the tendon transfer to be successful the foot had to be passively correctable to at least a neutral position and that the tendon had to be passed superficial to the extensor retinaculum and inserted into the lateral cuneiform bone. The heel cord should be lengthened before the tendon transfer.

The treatment of the painful hip in cerebral palsy by total hip replacement or hip arthrodesis.

The painful dislocated or subluxated hip in the patient with cerebral palsy presents a difficult problem in management. Twenty-three patients with cerebral palsy who had a painful subluxated or dislocated hip with degenerative changes were operated on at The Hospital for Special Surgery. Eight patients had a unilateral hip arthrodesis. Six had a successful arthrodesis initially, resulting in relief of pain and return to the preoperative functional level. A pseudarthrosis developed in two patients, but both had successful revision surgery, one by a second arthrodesis and the other by a total hip replacement. Thirteen of the fifteen patients with a total hip replacement were pain-free and functioning at a level consistent with their over-all involvement. One patient had migration of the greater trochanter and slight bending of the femoral component, with persistent pain. A second patient had progressive loosening of the femoral component over a period of seven years, but continued to walk with minimum pain. Two patients had a recurrent dislocation, one requiring revision of the femoral component and the other, of the acetabular component. We think that the presence of cerebral palsy, even in the patient with severe involvement, is not a contraindication for either a hip arthrodesis or a total hip replacement for a painful deformed or degenerated hip. These patients should be treated for the pain in the hip, and the presence of the underlying neuromuscular disorder should not deter the appropriate treatment. For unilateral hip disease in patients who are unable to walk and in young, active patients, we recommend hip fusion.(ABSTRACT TRUNCATED AT 250 WORDS)

Discoid lateral meniscus in children. Long-term follow-up after excision.

We retrospectively reviewed the results for fifteen patients, eight girls and seven boys, who had had a total of eighteen meniscectomies performed for a discoid meniscus at The Hospital for Special Surgery between January 1, 1955, and December 31, 1983. The average age was ten and a half years (range, six to sixteen years) at the time of the operation and twenty-eight years (range, twenty to thirty-six years) at the time of follow-up. The average duration of follow-up was seventeen years (range, eight to twenty-eight years). The primary indication for the meniscectomy was continuing pain in fourteen knees and locking or snapping in three. One patient had tenderness and walked with a limp. A meniscal tear was found in twelve knees at the time of the operation. According to the scale of Ikeuchi, the result was excellent for ten knees, good for three, and fair for five. Of the five patients (five knees) who had a fair result, four had mild intermittent discomfort after strenuous physical activity as well as clicking (three knees) or swelling (one knee), and one had clicking. One of these five patients was subsequently found to have rheumatoid arthritis, and two subsequently had a patellar realignment. No degenerative changes were evident on the roentgenograms of the eight patients (nine knees) for whom they were made at the latest follow-up evaluation.

Distal lengthening of the hamstrings in patients who have cerebral palsy. Long-term retrospective analysis.

One hundred and twenty-six patients who had cerebral palsy and who had been managed with distal lengthening of the hamstrings were followed for three to fourteen years so that the long-term results of the procedure could be determined. Twenty-four of sixty-two patients who could not walk before the procedure were able to walk about the house postoperatively. Mild recurvatum developed in only ten patients, and twenty-two patients had a reoperation because of recurrence. Although straight-leg raising and the popliteal angle had improved markedly at one year in all but two patients, both parameters regressed gradually over time. Of several selected variables, only limitation of straight-leg raising preoperatively was of statistical significance (p < 0.001) for the prediction of recurrence. Age, preoperative walking ability, concomitant operations, and postoperative immobilization had no significant effect on the rates of recurrence or recurvatum.

The severely unstable hip in cerebral palsy. Treatment with open reduction, pelvic osteotomy, and femoral osteotomy with shortening.

The results in thirty-one patients with cerebral palsy who had a total of thirty-five severely subluxated or dislocated hips were analyzed retrospectively a mean of seven years after open reduction, pelvic osteotomy, varus rotational osteotomy, and femoral shortening. Preoperatively, twenty-two patients had been unable to stand and thirteen had had pain; the mean acetabular index was 50 degrees, the mean center-edge angle was -19 degrees, and the mean migration index was 74 percent. At the latest follow-up examination, none of the hips were painful. Seven patients had an improvement of one level in their walking ability. All of the patients who were confined to a wheelchair had better sitting balance. The mean acetabular index was 40 degrees, the mean center-edge angle was 18 degrees, and the mean migration index was 25 percent. Four hips were subluxated (two of them posteriorly). One hip was treated with a repeat varus rotational osteotomy. Another hip, which was not dislocated, had a rotational osteotomy for excessive femoral anteversion. Eight femoral heads displayed signs of avascular necrosis. One tibial and two femoral fractures occurred after the cast was removed. Three of the four patients who had a subluxated hip had scoliosis. The combined approach improved coverage of the femoral head and decreased pain in the hip. Even though this procedure can be accompanied by serious complications, we believe that the results justify this extensive approach in these patients.

The treatment of osteogenesis imperfecta.

In this article, the author discusses the classification, genetic patterns, histologic and radiographic features, and treatment of osteogenesis imperfecta. Four types of treatment are available: (1) drugs or medications to increase the strength of bone and decrease fractures, (2) physical therapy, (3) bracing and splinting, and (4) surgery, with the most effective treatment being an operation consisting of multiple osteotomies and intramedullary rod fixations.

Osteogenesis imperfecta.

Osteogenesis imperfecta describes a group of heritable disorders characterized by excessive bony fragility and reduced skeletal mass. It is classified in terms of its clinical manifestations, but our understanding of the underlying genetic defects in collagen synthesis is increasing rapidly. The nonoperative and surgical orthopedic approaches to osteogenesis imperfecta aim at the maximum preservation of limb strength and the correction of deformities. Various pharmacologic agents have been administered to patients with osteogenesis imperfecta, but to date, none have proved effective in controlled trials. Prenatal diagnosis has been attempted and seems certain to assume greater importance as knowledge of the molecular genetic basis of the disease increases.

Treatment of hip problems in cerebral palsy.

The two major problems of the hip in cerebral palsy relate to gait abnormalities and hip instability. Gait abnormalities are a result of muscle imbalance and should be corrected with appropriate muscle transfers and releases. Frequently, femoral anteversion may be associated with internal rotation of the limb, and if severe, should be corrected. Hip instability leading to S/D is a very serious problem in cerebral palsy and is usually worse in the more severely involved patients. Early muscle releases should be done before the hips subluxate. Once subluxation occurs, muscle releases must be combined with a varus rotation osteotomy. If acetabular insufficiency is present, pelvic osteotomy is necessary to obtain stability. In the older patient who has a painful S/D hip, the author recommends either a hip arthrodesis or a total hip replacement.

Surgical management of intractable seizure disorders.

The surgical treatment of intractable seizures is still regarded by some as an experimental procedure with questionable rationale. However, in centers where care is taken in the selection process and facilities are available for adequate diagnosis, the procedure has been successful in complete alleviation of seizures in some cases. The decision about surgical interventions for epilepsy is never an easy one. Potential surgical candidates are evaluated by a team of specialists and undergo numerous diagnostic tests and examinations before a decision for surgery is made. It is essential for the neuroscience nurse to have knowledge of the underlying etiology of seizures, diagnostic testing and surgical procedures in order to effectively care for the seizure patient pre- and postoperatively. With this knowledge, nursing management becomes a vital part in the total treatment of the seizure patient.