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Neuromuscular studies in clubfoot.

The fundamental pathology in rigid clubfoot appears to be a neuromuscular defect. In order to test this hypothesis, 90 muscle biopsies were obtained from the posteromedial, peroneal, and some intrinsic muscles at surgical correction of 13 clubfoot patients. All were examined ultrastructurally, and 48 biopsies in nine of these patients were studied using enzyme specific histochemical stains at the light microscopic level. Neuromuscular junctions were isolated in the latter group. Consistent ultrastructural abnormalities were observed in all specimens. Histochemistry revealed a dominant Type I fiber population and Type I fiber grouping. A correlated increase in Type I neuromuscular junctions occurred in these areas. Since these changes are recognized as being neurogenically determined, a neuromuscular abnormality may be significant in the etiology of clubfoot.

Biopsy↗

Prevalence of unsuspected myopathy in infants presenting for clubfoot surgery.

The objective of this study was an evaluation of the prevalence of myopathies in paediatric patients scheduled for orthopaedic surgery (clubfoot) performed under regional anaesthesia. Seventeen infants scheduled for lower limb orthopaedic surgery were studied to verify coexisting neuromuscular disorders with electromyography and muscle biopsy during surgery. All surgical procedures were performed under caudal block or spinal anaesthesia, associated with light general anaesthesia. No major cardiorespiratory, neurological or malignant hyperthermic complications (muscle rigidity, arrhythmias, hyperpyrexia) were observed. Combined neurological, electromyographic and biopsy studies showed a high rate of myopathic changes (70%). Performance of clubfoot surgery under light general anaesthesia with regional techniques was free from any problems. The high rate of myopathic changes (70%) observed in the muscle biopsies suggests that precautions should be taken with paediatric patients for clubfoot surgery and a regional anaesthesia technique with adequate monitoring may be helpful to prevent possible malignant hyperthermia related problems.

Anesthesia, Conduction↗

Prenatal ultrasound diagnosis of clubfoot.

Five cases of congenital clubfoot diagnosed prenatally by ultrasound are reported. The incidence of clubfoot may be higher within an affected family and may be associated with other structural anomalies or chromosomal abnormalities. Identifying a clubfoot in utero should therefore alert the sonographer that other anomalies may be present and should lead to a detailed structural survey.

Abnormalities, Multiple↗

Assessment of talar flattening in adult idiopathic clubfoot.

BACKGROUND: The radius/length (R/L) ratio was developed to evaluate the convexity of the talar dome in idiopathic clubfoot. However, the index has not been tested for its reliability and reproducibility. METHODS: The R/L ratio was determined by three independent observers on the radiographs of 21 adult patients with idiopathic clubfoot and 30 adult subjects with normal feet. The reproducibility and the reliability of the R/L ratio were calculated. RESULTS: For the normal feet the reproducibility and the reliability of the R/L ratio was high (correlation coefficient > 0.87). For the patients with clubfoot, the reliability and reproducibility depended on the severity of talar flattening. For a radius of less than 45 mm the mean intraobserver correlation coefficient was 0.74 (range 0.54 to 0.83) and the mean interobserver correlation coefficient was 0.58 (range 0.49 to 0.75). For a radius of more than 45 mm no statistically significant intraobserver and interobserver correlations were found. CONCLUSION: The current results indicate that the R/L ratio of talar flattening is reliable and reproducible for mild talar deformity but not for severe flattening (radius of more than 45 mm).

Adult↗

Correction of neglected clubfoot using the Ilizarov external fixator.

BACKGROUND: This study was conducted to evaluate the corrective capability of the Ilizarov external fixator in the treatment of neglected clubfoot. METHODS: Thirty patients (38 feet) with a mean age of 19 (5 to 39) years with severe deformities and stiff feet associated with neglected clubfoot were studied. A limited soft-tissue dissection, Achilles tenotomy, and plantar fasciotomy were done. Progressive correction of the deformities was achieved through a standard setting of the Ilizarov external fixator. The device was used for 16 weeks, on average, and after removal a short-leg walking cast was used for an additional 6 weeks, followed by an ankle-foot orthosis (AFO) for 6 months. RESULTS: The final outcome was scored as good (complete correction and no pain); fair (partial correction with plantigrade foot and occasional pain); or poor (nonplantigrade foot and continuous pain during walking). After a mean followup of 58 (range 12 to 107) months, the results were good in 30 feet (78.9%); fair in three feet (7.9%); and poor in five feet (13.2%). Early complications were a distal tibial fracture in one foot, dislocation of the first metatarsophalangeal joint in one foot, and arterial damage that resulted in amputation of the toes in one foot. Recurrence of the deformity was found in 19 feet (50%): 11 minor, three mild, and five severe. Spontaneous ankylosis developed in 28 feet (73.7%). Nine feet (23.7%) required arthrodesis for symptomatic arthritis of the ankle or midfoot and deformity that could not be treated with orthoses. CONCLUSION: The Ilizarov external fixator allows simultaneous correction of all the severe foot deformities associated with neglected clubfoot with minimal surgery, reducing risks of cutaneous or neurovascular complications and avoiding excessive shortening of the foot. Even in those patients in whom final corrective arthrodesis is necessary, this may be carried out with minimal bone resection, since the severe deformities of the foot and ankle have been corrected.

Adolescent↗

Validity and responsiveness of the Clubfoot Assessment Protocol (CAP). A methodological study.

BACKGROUND: The Clubfoot Assessment Protocol (CAP) is a multi dimensional instrument designed for longitudinal follow up of the clubfoot deformity during growth. Item reliability has shown to be sufficient. In this article the CAP's validity and responsiveness is studied using the Dimeglio classification scoring as a gold standard. METHODS: Thirty-two children with 45 congenital clubfeet were assessed prospectively and consecutively at ages of new-born, one, two, four months and two years of age. For convergent/divergent construct validity the Spearman's correlation coefficients were calculated. Discriminate validity was evaluated by studying the scores in bilateral clubfeet. The floor-ceiling effects at baseline (untreated clubfeet) and at two years of age (treated clubfeet) were evaluated. Responsiveness was evaluated by using effect sizes (ES) and by calculating if significant changes (Wilcoxons signed test) had occurred between the different measurement occasions. RESULTS: High to moderate significant correlation were found between CAP mobility I and morphology and the Dimeglio scores (rs = 0.77 and 0.44 respectively). Low correlation was found between CAP muscle function, mobility II and motion quality and the Dimeglio scoring system (rs = 0.20, 0.09 and 0.06 respectively). Of 13 children with bilateral clubfeet, 11 showed different CAP mobility I scores between right and left foot at baseline (untreated) compared with 5 with the Dimeglio score. At the other assessment occasions the CAP mobility I continued to show higher discrimination ability than the Dimeglio. No floor effects and low ceiling effects were found in the untreated clubfeet for both instruments. High ceiling effects were found in the CAP for the treated children and low for the Dimeglio. Responsiveness was good. ES from untreated to treated ranged from 0.80 to 4.35 for the CAP subgroups and was 4.68 for the Dimeglio. The first four treatment months, the CAP mobility I had generally higher ES compared with the Dimeglio. CONCLUSION: The Clubfoot Assessment Protocol shows in this study good validity and responsiveness. The CAP is more responsive when severity ranges between mild-moderate to severe, while the Dimeglio focuses more on the extremes. The ability to discriminate between different mobility status of the right and left foot in bilaterally affected children in this population was higher compared with the Dimeglio score implicating a better sensitivity for the CAP.

Child, Preschool↗

Radical reduction in the rate of extensive corrective surgery for clubfoot using the Ponseti method.

OBJECTIVES: The purpose of this study was to evaluate the efficacy of the Ponseti method in reducing extensive corrective surgery rates for congenital idiopathic clubfoot. METHODS: Consecutive case series were conducted from January 1991 through December 2001. A total of 157 patients (256 clubfeet) were evaluated. All patients were treated by serial manipulation and casting as described by Ponseti. Main outcome measures included initial correction of the deformity, extensive corrective surgery rate, and relapses. RESULTS: Clubfoot correction was obtained in all but 3 patients (98%). Ninety percent of patients required </=5 casts for correction. Average time for full correction of the deformity was 20 days (range: 14-24 days). Only 4 (2.5%) patients required extensive corrective surgery. There were 17 (11%) relapses. Relapses were unrelated to age at presentation, previous unsuccessful treatment, or severity of the deformity (as measured by the number of Ponseti casts needed for correction). Relapses were related to noncompliance with the foot-abduction brace. Four patients (2.5%) underwent an anterior tibial tendon transfer to prevent further relapses. CONCLUSIONS: The Ponseti method is a safe and effective treatment for congenital idiopathic clubfoot and radically decreases the need for extensive corrective surgery. This technique can be used in children up to 2 years of age even after previous unsuccessful nonsurgical treatment.

Braces↗

Validity of the anteroposterior talocalcaneal angle to assess congenital clubfoot correction.

OBJECTIVE: The anteroposterior talocalcaneal angle (Kite's angle) is still considered a common parameter for assessing clubfoot correction, although some dissenting opinions about its accuracy have been expressed. The purpose of this study was to evaluate the validity of the anteroposterior talocalcaneal angle for assessing correction of congenital clubfoot in adults who received the treatment as children. SUBJECTS AND METHODS. The anteroposterior talocalcaneal angle was measured in 48 treated idiopathic congenital clubfeet and 28 normal feet of 38 patients at the end of skeletal growth using both standing anteroposterior radiographs and 3D CT scan reconstructions. All the patients had been treated by manipulation, above-the-knee casting, and a complementary posteromedial release operation. RESULTS: The radiographic measurement of the anteroposterior talocalcaneal angle corresponded to the measurement of the same angle on the 3D CT scan reconstructions in only the normal feet and 12 clubfeet. In the other 36 clubfeet, a statistically significant difference of a mean of 15 degrees between the two measurements was noted, and the 3D CT scan reconstructions showed a superimposition of the talus and calcaneus, which had lost their normal anatomic divergence. In these cases, the marked medial angulation of the talar neck allowed a positive measurement of the anteroposterior talocalcaneal angle in the anteroposterior radiographic projection. CONCLUSION: According to our findings, the measurement of the anteroposterior talocalcaneal angle on radiography was misleading for assessing the degree of hindfoot correction in 75% of the treated congenital clubfeet. We believe that other imaging parameters should be considered instead of this angle to evaluate clubfoot correction.

Adult↗

Tibial torsion in untreated congenital clubfoot.

Current methods of measurement were reassessed by the study of tibial torsion in 1000 normal legs using the tropometer, the caliper and X-rays. No significant difference in the reproducibility of results was found and the simpler clinical methods appeared to be satisfactory for practical purposes. The spontaneous evolution of leg torsion in clubfoot was studied in forty-two neglected and previously untreated cases, who were seen for the first time after they had begun walking. Comparison with the torsion observed in the legs of healthy children of a corresponding age proved that untreated clubfoot is not associated with pathological torsion. The place of rotation osteotomy in the treatment of clubfoot is discussed.

Adolescent↗

Incidence of congenital clubfoot in Sweden. 128 cases in 138,000 infants 1946-1990 in Malmö.

The incidence of congenital clubfoot, neurological disorders excluded, was studied over a period of 45 years (1946-1990). The number of children, born alive with clubfoot, and detailed census data for the period were available. Altogether 137,614 living infants were born and of these 128 had congenital clubfoot. 56 (44 percent) were bilateral and 101 (79 percent) were boys. The overall incidence was 0.93 per thousand children. The annual incidence rose during the 45-year-period. This was, however, fully explained by the higher incidence among children of non-Nordic extraction.

Clubfoot↗

Results of ultrastructural analysis of the calf muscles in clubfoot.

Open biopsies were carried out on the muscles of 23 clubfoot patients, ages 9 months to 4 years. Electron microscopic analyses of the so-called "clubfoot muscles" and the peroneal muscles were performed. The changes found were not present in every area of the muscles, but were surrounded by fields of normal structure. Fatty degeneration with fibrosis was observed as the consequence of immobilization. More marked loss was found in the contractile elements. The authors believe that neuromuscular atrophy is a primary cause of congenital clubfoot. The most seriously affected muscles in this study were the tibialis posterior and peroneal muscles. The material failed to prove correlation with age. Based on their observations, the authors suggest finishing all types of immobilization before 1 year of age, when children begin to walk.

Actin Cytoskeleton↗

Sonographic diagnosis of clubfoot in utero.

The in utero sonographic diagnoses of 13 clubbed feet in eight fetuses are reported, and five representative cases are described in detail. The sonographic findings and clinical features of fetal clubfoot are discussed. The prenatal sonographic detection of clubfoot should alert the sonographer to search for other congenital anomalies associated with syndromes involving clubfoot.

Abnormalities, Multiple↗

Connective tissue structures in clubfoot: a morphologic study.

A morphologic study of connective tissue structures in clubfoot, with special emphasis on the presence of myofibroblasts, was undertaken to investigate the theory of retracting fibrosis as an etiologic factor. Nine idiopathic clubfeet from six patients were studied. Specimens from the medial and lateral capsule, medial and lateral fascia, spring and lacinate ligaments, and flexor digitorum longus, flexor hallucis longus, posterior tibialis, and Achilles tendon sheaths were investigated. Fifty specimens were examined using light microscopy and hematoxylin and eosin staining, and 26 were examined by transmission electron microscopy. Light microscopy failed to reveal any myofibroblast-like cells or any qualitative differences between specimens from capsule, fascia, ligaments, and tendon sheath. Using transmission electron microscopy, the authors identified two cell types: fibroblasts and mast cells. Some fibroblasts contained a network of microfilaments but all lacked microbundles, basal lamina, or plasmalemmal attachment plaques seen in typical myofibroblasts. Mast cells were rarely identified in capsular specimens. The absence of myofibroblast-like cells or typical myofibroblasts in clubfoot connective tissue structures does not support the theory of retracting fibrosis as a likely cause of contracture in idiopathic clubfoot.

Clubfoot↗

Ankle and first metatarsophalangeal joint dorsiflexion in children with clubfoot.

It has been hypothesised that the stiffness of the plantar aponeurosis after clubfoot surgery affects push-off. Because the first metatarsophalangeal (MTP) joint motion relies on the plantar aponeurosis, it was important to determine whether there was a lack of first MTP joint motion in children with clubfoot that also affected push-off. By examining the motion of the first MTP joint using a motion analysis system and passive motion techniques, then correlating these with gait characteristics, the authors found that the first MTP joint was not affected in children with clubfoot. The authors found that a motion analysis system could be used to determine range of motion accurately.

Analysis of Variance↗

[Treatment of congenital clubfoot with the Ponseti method].

OBJECTIVES: This study aimed to present the author's early experience with the Ponseti technique in correcting clubfoot and to evaluate the need for aggressive surgery in the first year of life. METHODS: The study included 44 feet of 31 patients (25 boys, 6 girls; mean age 3 months; range 4 days to 8 months) with idiopathic clubfoot deformities of grade II, III, or IV. Twenty-seven feet (19 patients) had not received any treatment before. Twelve patients (17 feet) had had unsuccessful prior conservative treatment elsewhere. All patients had manipulation casting in accordance with the Ponseti technique, and percutaneous Achilles tenotomy was performed in the presence of persistent equinus. Maintenance of correction was obtained with the use of a foot abduction brace. The mean follow-up period was 18 months (range 6.5 to 40 months). RESULTS: Forty-two feet (95%, 29 patients) were treated successfully using the Ponseti method. In terms of compliance with the foot abduction brace, a significant difference was noted between patients without previous treatment and those with deformities considered resistant to prior conservative treatment. The latter manifested more compliance with brace application. CONCLUSION: Ponseti method proved efficient in correcting idiopathic congenital clubfoot deformities irrespective of initial deformity grades, provided that the details of the technique are strictly adhered to. The results also suggest the role of brace application in order to avoid relapses along with the importance of increasing patients' compliance through family education and experienced orthotists.

Braces↗

Leg length discrepancy in unilateral congenital clubfoot following surgical treatment.

Length discrepancy secondary to limb hypoplasia has been described as an associated finding in patients with unilateral clubfoot. In this manuscript we bring attention to limb length discrepancy as a result of surgical treatment in unilateral clubfoot. Three patients who underwent extensive posterior, medial and lateral release were noted to have an average discrepancy in foot height of 2.1 centimeters (range, 2.0-2.3 centimeters). A decrease in foot height in addition to baseline limb hypoplasia may lead to a significant discrepancy that may justify surgical treatment. In this manuscript we point out that length discrepancy in such cases may not be adequately quantified on standard anteroposterior scanograms. Standing lateral foot radiographs will document loss in foot height as a possible factor in length discrepancy in surgically treated clubfoot patients.

Casts, Surgical↗

[Effect of the regulation of IGFs system components in retinoic acid-induced congenital clubfoot].

The fetal rat models with congenital clubfoot were constructed by treating 24 Wistar rats with all trans retinoic acid (ATRA). The MC-3T3-E1 cells were cultured with ATRA, 17 beta-estrogen (E2) or combinations of the two chemicals. The flow cytometer was used to determine the cell proliferation. The insulin-like growth factor-II (IGF-II ) and IGF conjugated protein-6 (IGFBP-6) mRNA level in rat calvaria bone tissue and MC-3T3-E1 cells were detected by northern blotting analysis and reverse transcription polymerase chain reaction. The congenital clubfoot of fetal rat was induced by ATRA in concentration of 100 approximately 140 mg/kg with dosage-dependence effect. The expression of IGF-II mRNA and cell proliferation were enhanced by E2(1 x 10(-6) mol/L) in rat calvaria bone tissue and MC-3T3-E1 cells, whereas the IGFBP-6 mRNA was increased. ATRA(1 x 10(-6) mol/L), however, inhibited the effect of E2 on regulation of IGF- II gene and IGFBP-6 gene as well as MC-3T3-E1 cell proliferation. These findings provide the evidence that ATRA can induce congenital skeleton malformation and congenital clubfoot in pregnant Wistar rats. IGF-II and IGFBP-6 are important regulative factors for skeleton development and osteoblast proliferation in rat.

Abnormalities, Drug-Induced↗

[The primary observation of congenital clubfoot].

OBJECTIVE: To explore the possible correlations between clinical and experimental pathological changes of congenital clubfoot and the pathodynamic developmental procedure. METHODS: Eighty-three female Wistar rats were administered with retinoic acid on the 10th day after pregnancy. And from February 2001 to February 2004, 48 patients were analyzed with electropysiological examination. RESULTS: There was clubfoot-like deformity in 53.7% of the experimental fetuses. Persistence of the embryonic position of the talus and tibia in fetuses was observed. Poor overlapping between talus and calcaneus was seen. Cell apoptosis at the anterior corner of spinal cord were seen. Of all the patients, 68.3% were abnormal with electropysiological examination. The pathological sites were frequently localized in lumbarsacral region. CONCLUSION: Congenital clubfoot is correlated closely with defects of neural tube and spinal cord.

Abnormalities, Drug-Induced↗