Frederick R. Dietz

Treatment of idiopathic clubfoot. A thirty-year follow-up note.

We evaluated forty-five patients who had seventy-one congenital clubfeet. The average age was thirty-four years (range, twenty-five to forty-two years). Twenty-nine of these patients had been evaluated and reported on in 1980. We performed pedobarographic and electrogoniometric analyses in addition to the clinical and radiographic studies performed previously. With the use of pain and functional limitation as the outcome criteria, thirty-five (78 per cent) of the forty-five patients had an excellent or good outcome compared with eighty-two (85 per cent) of ninety-seven individuals who did not have congenital deformity of the foot. The patients occupation, passive dorsiflexion as measured with a hand-held goniometer, the antero-posterior calcaneus-fifth metatarsal angle, the total foot pressure time integral, and the number of rapid single-limb toe-ups that could be performed were the only variables that differed significantly between the feet that had an excellent or good result and those that had a poor result (p < 0.05). A comparison of the feet that had an excellent or good outcome with those that had a poor outcome with regard to body-mass index, peak pressure under the heel, and force time integral under the metatarsal heads revealed a p value that was between 0.05 and 0.08 for each variable. The technique of treatment led to good long-term results in our patients who had clubfoot. The data suggest that a sedentary occupation and avoidance of excessive weight gain may improve the over-all long-term result. Excessive weakening of the triceps surae may predispose patients to a poor result; therefore, it is prudent to avoid overlengthening of this muscle. The outcome could not be predicted from the radiographic result.

Long-term follow-up after fractures of the tibial and fibular shafts.

Thirty-seven patients who sustained a closed or a Grade-I open tibial and fibular fracture were evaluated an average of twenty-nine years after injury. All of the patients had had uncomplicated treatment with a plaster cast. Clinically, 78 per cent of the ankles were rated good or excellent, and 92 per cent of the knees were rated excellent. Radiographic assessment for osteoarthritic changes revealed a good or excellent result for 76 per cent of the ankles and an excellent result for 92 per cent of the knees. The clinical and radiographic outcomes were unaffected by the amounts of anterior or posterior and of varus or valgus angulation, as well as by the level of the fracture. The length of immobilization, which did not exceed one year, also did not affect the outcomes.

Two novel point mutations in the long-range SHH enhancer in three families with triphalangeal thumb and preaxial polydactyly†

Spatio‐temporal expression of sonic hedgehog (SHH) is driven by a regulatory element (ZRS) that lies 1 Mb upstream from SHH. Point mutations within the highly conserved ZRS have been described in the hemimelic extra toes mouse and in four families with preaxial polydactyly [Lettice et al., 2003 ]. Four North American Caucasian families were identified with autosomal dominant triphalangeal thumb. DNA from 20 affected and 36 unaffected family members was evaluated by sequence analysis of a 774‐bp highly conserved ZRS contained within LMBR1 intron 5. Mutations within ZRS were identified in three of four families. In pedigree A and C, a novel A/G transition was identified near the 5′ end of ZRS at bp 739 that segregated with disease or carrier status. Pedigree A, described previously [Dobbs et al., 2000 ], is a large family with 19 affected members who exhibit a milder phenotype, including predominantly triphalangeal thumbs and low penetrance (82%) relative to other families. Pedigree C is a small family with two affected family members with triphalangeal thumb, and one affected with both triphalangeal thumb and preaxial polydactyly. A novel C/G mutation at bp 621 was identified in pedigree B that segregated with the disease in all four affected individuals who manifested both preaxial polydactyly and triphalangeal thumb. Both mutations alter putative Cdx transcription factor binding sites. Mutations within ZRS appear to be a common cause of familial triphalangeal thumb and preaxial polydactyly. A genotype/phenotype correlate is suggested by pedigree A, whose mutation lies near the 5′ end of ZRS; this family demonstrates a higher rate of nonpenetrance and milder phenotype. However, modifier genes may be contributing to the milder phenotype in this family.

The weight-bearing abduction brace for the treatment of Legg-Perthes disease.

We reviewed the cases of thirty-one patients (thirty-four hips) who had had severe Legg-Perthes disease (Catterall group III, five hips and group IV, twenty-nine hips) and had been treated with a weight-bearing abduction orthosis, to determine the effectiveness of the brace. The mean age of the patients when they were first seen was six years (range, three to twelve years). The mean duration of follow-up was seven years (range, two to thirteen years). At the most recent follow-up, all hips were rated with the method of Mose and the classification of Stulberg et al. According to the criteria of Mose, no hip had a good result, twelve (35 per cent) had a fair result, and twenty-two (65 per cent) had a poor result. On the basis of the classification of Stulberg et al., there were fourteen (41 per cent) class-II results, eighteen (53 per cent) class-III and IV results, and two (6 per cent) class-V results. Although containment is the most widely accepted principle of treatment for patients who have Legg-Perthes disease, and the Atlanta Scottish Rite-type orthosis is the most commonly used orthosis for this condition, there are few clinical data supporting the effectiveness of this device. On the basis of our results, we do not recommend the use of a weight-bearing abduction brace for the treatment of severely involved hips.

Stulberg Classification System for Evaluation of Legg-Calvé-Perthes Disease: Intra-Rater and Inter-Rater Reliability*

BACKGROUND Researchers and clinicians commonly use the classification system of Stulberg et al. as a basis for treatment decisions during the active phase of Legg-Calvé-Perthes disease because of its putative utility as a predictor of long-term outcome. It is generally assumed that this system has an acceptable degree of reliability. This assumption, however, is not convincingly supported by the literature. METHODS The purpose of the present study was to assess the inter-rater and intra-rater reliability of the classification system of Stulberg et al. with use of a pre-test, post-test design. During the pre-test phase, nine raters independently used the system to evaluate the radiographs of skeletally mature patients who had been managed for Legg-Calvé-Perthes disease. The intervention between the pre-test and post-test phases consisted of a consensus-building session during which all raters jointly arrived at standardized definitions of the various joint structures that are assessed with use of the classification system. The effect of these definitions on reliability then was assessed by reevaluating the radiographs during the post-test phase. RESULTS The pre-test intra-rater reliability coefficients ranged from 0.709 to 0.915, and the post-test coefficients ranged from 0.568 to 0.874. The pre-test inter-rater reliability coefficients ranged from 0.603 to 0.732, and the post-test coefficients ranged from 0.648 to 0.744. Contributing to the variance was a lack of agreement concerning the assessment of joint structures and the way in which the raters translated these evaluations into a classification according to the system of Stulberg et al. CONCLUSIONS Although intra-rater reliability was marginally acceptable, the degree of variability between the classifications assigned by different raters even after the intervention - calls into question the reliability of the system of Stulberg et al.; consequently, the validity of any treatment decisions, outcome evaluations, or epidemiological studies based on this system is also in question.

The importance of positive bacterial cultures of specimens obtained during clean orthopaedic operations

Microbiological cultures of specimens of tissue and of fluids from the wound in forty patients who had had consecutive clean, elective orthopaedic operations (excluding total joint replacements) and had not received antibiotics preoperatively were analyzed. Of the forty patients, twenty-three (58 per cent) had a positive culture on at least one of the media that were used and seventeen (43 per cent) had negative cultures. Of the forty specimens that were obtained from swabbing of the wound, eight (20 per cent) were positive on culture, compared with twenty (50 per cent) that were obtained from biopsy of tissue. Of these twenty-eight positive cultures, thirteen (46 per cent) were on routine blood-agar plates and fifteen (54 per cent), in broth only. Of the thirty-three bacterial organisms that were identified in the twenty-eight positive cultures of the wound, nineteen (58 per cent) were coagulase-negative Staphylococcus; eight (24 per cent), Propionibacterium acnes; two (6 per cent), Peptostreptococcus; and four (12 per cent), miscellaneous organisms. In all of the positive cultures on the blood-agar plates, except in those showing Propionibacterium acnes, there were five colonies or fewer. One patient had a clinical infection with Staphylococcus aureus that developed later, but the initial cultures of the wound had been positive for Staphylococcus epidermidis only. None of the bacteria that grew on culture were Staphylococcus aureus or the less common pathogenic gram-negative bacteria, such as Escherichia coli, Pseudomonas, or Klebsiella.(ABSTRACT TRUNCATED AT 250 WORDS)

Rapid progression of hip subluxation in cerebral palsy after selective posterior rhizotomy

Summary: Rapid progression of hip subluxation was noted in the year after selective dorsal rhizotomy in seven hips (six patients). The hips that subluxed progressed from a lateral extrusion index averaging 25% preoperatively as compared with 50% after rhizotomy. Although preexistent hip dysplasia was a predisposing factor, hips with an intermediate degree of preoperative lateral extrusion (12–25%) had variable results. Ongoing and more frequent evaluation of hip stability is necessary after dorsal rhizotomy.

What is the best evidence for the treatment of slipped capital femoral epiphysis

Background: There are many different treatment methods for slipped capital femoral epiphysis (SCFE). It was the purpose of this study to review the results from the literature for different methods of SCFE treatment and on the basis of level of evidence determine the current best evidence treatment. Methods: A systematic review of the literature was undertaken. Treatment results were grouped into 2 categories. The first was all methods without surgical hip dislocation, and the second was all methods in which surgical dislocation was used. Results: For stable SCFEs without surgical dislocation, the best recommended treatment (mostly level IV) recommends in situ single screw fixation over multiple pin fixation, epiphysiodesis, osteotomy, or spica cast. For the unstable SCFEs without surgical dislocation (all level IV), the best recommended treatment is urgent reduction with decompression and internal fixation. For both stable and unstable SCFEs, the short-term small series in the literature (all level IV) does not demonstrate an advantage or improvement in outcomes compared with in situ single screw fixation for stable SCFE and urgent reduction, decompression, and internal fixation in unstable SCFEs. Conclusions: A systematic review of the literature recommends on the basis of level of evidence that the best treatment for a stable SCFE is single screw in situ fixation and for unstable SCFEs urgent gentle reduction, decompression, and internal fixation. Level of Evidence: Level IV, systematic review of level IV studies.

Traction reduction of acute and acute-on-chronic slipped capital femoral epiphysis.

Gentle reduction of acute or the acute portion of acute-on-chronic severe slipped capital femoral epiphyses (SCFE) is generally recommended. Reports of the use of longitudinal traction with medial rotation traction, although few, are uniform in describing a high rate of success with rare complications. Of 30 acute or acute-on-chronic SCFEs examined at the authors institution between 1970 and 1990, 13 underwent attempted traction reduction with longitudinal traction and medial rotation. Only five of these 13 hips had discernible reduction. One of these five hips developed aseptic necrosis; however, this hip was clearly distracted from the acetabulum by excessive longitudinal traction that may have contributed to the development of osteonecrosis. One of the seven hips that failed traction reduction developed aseptic necrosis, and one of the 17 acute SCFEs pinned in situ developed aseptic necrosis. All three hips developing necrosis were in a group of 14 hips with moderate to severe SCFEs and complete inability to bear weight with severe pain.

Reflex sympathetic dystrophy in children.

Reflex sympathetic dystrophy (RSD) is an uncommonly reported entity in children. This paper reports five cases of RSD in children and summarizes 80 cases of pediatric RSD reported in the literature. The diagnosis is based on the clinical findings of pain, dysesthesia, and autonomic instability. Tache cérébrale, not previously described in the diagnosis of RSD, is a helpful sign of vasomotor dysfunction. RSD in childhood frequently affects the lower extremities, in contrast to the adult localization around the shoulders and hands. Noninvasive, nonpharmacologic management is generally successful. A simple outpatient program of massage and mobilization was beneficial in four of the five patients treated in this study.