Jose A. Morcuende

Acetabular dysplasia after treatment for developmental dysplasia of the hip. Implications for secondary procedures.

Untreated acetabular dysplasia following treatment for developmental dysplasia of the hip (DDH) leads to early degenerative joint disease. Clinicians must accurately and reliably recognise dysplasia in order to intervene appropriately with secondary acetabular or femoral procedures. This study sought early predictors of residual dysplasia in order to establish empirically-based indications for treatment. DDH treated by closed or open reduction alone was reviewed. Residual hip dysplasia was defined according to the Severin classification at skeletal maturity. Future hip replacement in a subset of these patients was compared with the Severin classification. Serial measurements of acetabular development and subluxation of the femoral head were collected, as were the age at reduction, type of reduction, and Tonnis grade prior to reduction. These variables were used to predict the Severin classification. The mean age at reduction in 72 hips was 16 months (1 to 46). On the final radiograph, 47 hips (65%) were classified as Severin I/II, and 25 as Severin III/IV (35%). At 40 years after reduction, five of 43 hips (21%) had had a total hip replacement (THR). The Severin grade was predictive for THR. Early measurements of the acetabular index (AI) were predictive for Severin grade. For example, an AI of 35 degrees or more at two years after reduction was associated with an 80% probability of becoming a Severin grade III/IV hip. This study links early acetabular remodelling, residual dysplasia at skeletal maturity and the long-term risk of THR. It presents evidence describing the diagnostic value of early predictors of residual dysplasia, and therefore, of the long-term risk of degenerative change.

Long-Term Outcome after Open Reduction through an Anteromedial Approach for Congenital Dislocation of the Hip*

We reviewed the long-term outcome of open reduction of ninety-three congenitally dislocated hips (in seventy-six children) through an anteromedial approach. The average age of the patients was fourteen months (range, two to fifty months) at the time of the reduction and eleven years (range, four to twenty-three years) at the time of the most recent follow-up evaluation. At the most recent follow-up evaluation, sixty-six hips (71 per cent) had an excellent or good result, twenty-four (26 per cent) had a fair result, and three (3 per cent) had a poor result, according to the Severin classification system. An inverted neolimbus at the time of the operation and postoperative growth disturbance of the femoral head were associated with a poor roentgenographic result. According to the classification of Bucholz and Ogden, twenty-two hips (24 per cent) had type-II avascular necrosis, thirteen hips (14 per cent) had type-III, three (3 per cent) had type-IV, two (2 per cent) had non-classifiable lesions, and fifty-three (57 per cent) did not have avascular necrosis. A high hip dislocation and an operation after the age of twenty-four months were associated with a higher rate of growth disturbances of the femoral head. With the numbers available for study, we did not find any association between short-term preoperative traction, ligation of the medial circumflex vessel, or the type of neolimbus and the prevalence of growth disturbances. Two hips redislocated postoperatively, and seven had transient stiffness. We consider the anteromedial approach to be useful in the management of patients with congenital dislocation of the hip who are twenty-four months old or less. The advantages of this approach include direct access to the obstacles to reduction, avoidance of damage of the iliac apophysis and the abductor muscles, minimum blood loss, the need for only a single operative session for treatment of both hips, and a cosmetically acceptable scar. The prevalence of type-II growth disturbances of the femoral head was higher than had been expected, emphasizing the need for additional investigation.

Prevalence of Neural Axis Abnormalities in Patients with Infantile Idiopathic Scoliosis

Background: Although reports in the literature have demonstrated an approximately 20% prevalence of neural axis abnormalities in patients with juvenile idiopathic scoliosis who have a curve of >20°, the prevalence of neural axis abnormalities in patients with infantile idiopathic scoliosis is not well documented. In two previous studies involving a total of only ten patients with infantile idiopathic scoliosis, five patients were noted to have a neural axis abnormality on magnetic resonance images.Methods: The records of forty-six consecutive patients who were seen between 1992 and 2000 at three spinal deformity clinics were retrospectively reviewed. The inclusion criteria included presumed idiopathic scoliosis at the time of presentation, an age of three years or less, a curve magnitude of ≥20°, normal neurological findings, no associated syndromes, and no congenital abnormalities. All patients were evaluated with a total spine magnetic resonance imaging protocol for examination of neural axis abnormalities from the skull to the coccyx.Results: Ten (21.7%) of the forty-six patients were found to have a neural axis abnormality on magnetic resonance imaging. This group included five patients with an Arnold-Chiari malformation and an associated cervicothoracic syrinx, three with syringomyelia, one with a low-lying conus, and one with a brainstem tumor. Eight of these ten patients needed neurosurgical intervention for treatment of the abnormality.Conclusions: The 21.7% prevalence of neural axis abnormalities in this group of patients with infantile idiopathic scoliosis was found to be almost identical to that reported in the literature on patients with juvenile idiopathic scoliosis. Because of the high prevalence of abnormalities and the fact that eight of the ten patients with abnormal findings on magnetic resonance images required neurosurgical intervention, a total spine magnetic resonance imaging evaluation at the time of presentation is recommended for all patients with infantile idiopathic scoliosis who have a curve measuring ≥20°.

Early Results of a New Method of Treatment for Idiopathic Congenital Vertical Talus

BACKGROUNDnThe treatment of idiopathic congenital vertical talus has traditionally consisted of manipulation and application of casts followed by extensive soft-tissue releases. However, this treatment is often followed by severe stiffness of the foot and other complications. The purpose of this study was to evaluate a new method of manipulation and cast immobilization, based on principles used by Ponseti for the treatment of clubfoot deformity, followed by pinning of the talonavicular joint and percutaneous tenotomy of the Achilles tendon in patients with idiopathic congenital vertical talus.nnnMETHODSnThe cases of eleven consecutive patients who had a total of nineteen feet with an idiopathic congenital vertical talus deformity were retrospectively reviewed at a minimum of two years following treatment with serial manipulations and casts followed by limited surgery consisting of percutaneous Achilles tenotomy (all nineteen feet), fractional lengthening of the anterior tibial tendon (two) or the peroneal brevis tendon (one), and percutaneous pin fixation of the talonavicular joint (twelve). The principles of manipulation and application of the plaster casts were similar to those used by Ponseti to correct a clubfoot deformity, but the forces were applied in the opposite direction. Patients were evaluated clinically and radiographically at the time of presentation, immediately postoperatively, and at the time of the latest follow-up. Radiographic measurements obtained at these times were compared. In addition, the radiographic data at the final evaluation were compared with normal values for an individual of the same age as the patient.nnnRESULTSnInitial correction was obtained both clinically and radiographically in all nineteen feet. A mean of five casts was required for correction. No patient underwent extensive surgical releases. At the final evaluation, the mean ankle dorsiflexion was 25 degrees and the mean plantar flexion was 33 degrees . Dorsal subluxation of the navicular recurred in three patients, none of whom had had pin fixation of the talonavicular joint. At the time of the latest follow-up, there was a significant improvement (p < 0.0001) in all of the measured radiographic parameters compared with the pretreatment values, and all of the measured angles were within normal values for the patients age.nnnCONCLUSIONSnSerial manipulation and cast immobilization followed by talonavicular pin fixation and percutaneous tenotomy of the Achilles tendon provides excellent results, in terms of the clinical appearance of the foot, foot function, and deformity correction as measured radiographically at a minimum two years, in patients with idiopathic congenital vertical talus.

Management of congenital talipes equinovarus using the Ponseti method: A SYSTEMATIC REVIEW

We present a systematic review of the results of the Ponseti method of management for congenital talipes equinovarus (CTEV). Our aims were to assess the method, the effects of modifications to the original method, and compare it with other similar methods of treatment. We found 308 relevant citations in the English literature up to 31 May 2010, of which 74 full-text articles met our inclusion criteria. Our results showed that the Ponseti method provides excellent results with an initial correction rate of around 90% in idiopathic feet. Non-compliance with bracing is the most common cause of relapse. The current best practice for the treatment of CTEV is the original Ponseti method, with minimal adjustments being hyperabduction of the foot in the final cast and the need for longer-term bracing up to four years. Larger comparative studies will be required if other methods are to be recommended.

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

BACKGROUNDnResearchers 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.nnnMETHODSnThe 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.nnnRESULTSnThe 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.nnnCONCLUSIONSnAlthough 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.

SNTG1, the gene encoding γ1-syntrophin: a candidate gene for idiopathic scoliosis

Idiopathic scoliosis (IS) affects approximately 2%–3% of the population and has a heritable component. The genetics of this disorder are complex. Here, we describe a family in which a pericentric inversion of chromosomexa08 co-segregates with IS. We have used fluorescence in situ hybridization to identify cloned DNAs that span the breakpoints on the two arms of the chromosome. We have identified a bacterial artificial chromosome (BAC) of 150xa0kb that crosses the q-arm breakpoint and a BAC of 120xa0kb that crosses the p-arm breakpoint. The complete genomic DNA sequence of these BACs has been analyzed to identify candidate genes and to localize further the precise breakpoints. This has revealed that the p-arm break does not interrupt any known gene and occurs in a region of highly repetitive sequence elements. On the q-arm, the break occurs between exonsxa010 and 11 of the γ-1 syntrophin (SNTG1) gene. Syntrophins are a group of cytoplasmic peripheral membrane proteins that associate directly with dystrophin, the Duchenne muscular dystrophy gene; γ1-syntrophin has been shown to be a neuronal cell-specific protein. Mutational analysis of SNTG1 exons in 152 sporadic IS patients has revealed a 6-bp deletion in exonxa010 of SNTG1 in one patient and a 2-bp insertion/deletion mutation occurring in a polypyrimidine tract of intronic sequence 20xa0bases upstream of the SNTG1 exonxa05 splice site in two patients. These changes were not seen in a screen of 480 control chromosomes. Genomic DNAs from seven affected individuals within the family of a patient carrying the 6-bp deletion were typed to determine whether the alteration co-segregated with IS. The deletion was only observed in five out of these seven individuals. Thus, although genetic heterogeneity or multiple alleles cannot be ruled out, the 6-bp deletion does not consistently co-segregate with the disease in this family.

Early results of a new method of treatment for idiopathic congenital vertical talus. Surgical technique.

BACKGROUNDnThe treatment of idiopathic congenital vertical talus has traditionally consisted of manipulation and application of casts followed by extensive soft-tissue releases. However, this treatment is often followed by severe stiffness of the foot and other complications. The purpose of this study was to evaluate a new method of manipulation and cast immobilization, based on principles used by Ponseti for the treatment of clubfoot deformity, followed by pinning of the talonavicular joint and percutaneous tenotomy of the Achilles tendon in patients with idiopathic congenital vertical talus.nnnMETHODSnThe cases of eleven consecutive patients who had a total of nineteen feet with an idiopathic congenital vertical talus deformity were retrospectively reviewed at a minimum of two years following treatment with serial manipulations and casts followed by limited surgery consisting of percutaneous Achilles tenotomy (all nineteen feet), fractional lengthening of the anterior tibial tendon (two) or the peroneal brevis tendon (one), and percutaneous pin fixation of the talonavicular joint (twelve). The principles of manipulation and application of the plaster casts were similar to those used by Ponseti to correct a clubfoot deformity, but the forces were applied in the opposite direction. Patients were evaluated clinically and radiographically at the time of presentation, immediately postoperatively, and at the time of the latest follow-up. Radiographic measurements obtained at these times were compared. In addition, the radiographic data at the final evaluation were compared with normal values for an individual of the same age as the patient.nnnRESULTSnInitial correction was obtained both clinically and radiographically in all nineteen feet. A mean of five casts was required for correction. No patient underwent extensive surgical releases. At the final evaluation, the mean ankle dorsiflexion was 25 degrees and the mean plantar flexion was 33 degrees . Dorsal subluxation of the navicular recurred in three patients, none of whom had had pin fixation of the talonavicular joint. At the time of the latest follow-up, there was a significant improvement (p < 0.0001) in all of the measured radiographic parameters compared with the pretreatment values, and all of the measured angles were within normal values for the patients age.nnnCONCLUSIONSnSerial manipulation and cast immobilization followed by talonavicular pin fixation and percutaneous tenotomy of the Achilles tendon provides excellent results, in terms of the clinical appearance of the foot, foot function, and deformity correction as measured radiographically at a minimum two years, in patients with idiopathic congenital vertical talus.

The Teardrop in Congenital Dislocation of the Hip Diagnosed Late. A Quantitative Study

We retrospectively reviewed the radiographs of the pelvis and hips of forty-five patients who had unilateral congenital dislocation of the hip treated with closed reduction and application of a cast without subsequent operations. The radiographs were made at the time of the initial diagnosis, two years after the reduction, when the child was ten years old, and at skeletal maturity. The width, shape, and type of the teardrop; the thickness of the acetabular floor; the acetabular index; the center-edge angle; the articulotrochanteric distance; and the Severin class at maturity were measured in the dislocated and contralateral, normal hips. At the time of the initial diagnosis, a well defined teardrop was seen in thirty-six (80 per cent) of the normal hips and in seven (16 per cent) of the dislocated hips. There was no difference in the width of the teardrop in the seven dislocated hips compared with that in the normal hips, although the v-shaped and crossed types of teardrops were more frequent in the dislocated hips. The v shape was not observed in the normal hips but was seen in sixteen dislocated hips two years after the reduction and in twelve dislocated hips when the children were ten years old. The superior and inferior widths of the teardrop of the dislocated hips were significantly greater than those of the normal hips (p < 0.001 and p < 0.05, respectively) when the children were ten years old. The hips with residual acetabular dysplasia had a v-shaped teardrop, widening of the superior width of the teardrop, and thickening of the acetabular floor. These hips, which were usually Severin class IV at the time of skeletal maturity, had a poor prognosis in adult life.

Long-term results of tibialis anterior tendon transfer for relapsed idiopathic clubfoot treated with the Ponseti method: a follow-up of thirty-seven to fifty-five years.

BACKGROUNDnRelapse of idiopathic clubfoot deformity after treatment can be effectively managed with repeat casting and tibialis anterior tendon transfer during early childhood. We evaluated the long-term effects on adult foot function after tibialis anterior tendon transfer for relapsed idiopathic clubfoot deformity during childhood.nnnMETHODSnThirty-five patients (sixty clubfeet) in whom idiopathic clubfoot was treated with the Ponseti method from 1950 to 1967 were followed. At an average age of forty-seven years (range, thirty-seven to fifty-five years), the patients underwent a detailed musculoskeletal examination, radiographic evaluation, pedobarographic analysis, and surface electromyography (EMG). They also completed three quality-of-life patient questionnaires.nnnRESULTSnFourteen patients (twenty-five clubfeet, 42%) had required repeat casting and tibialis anterior tendon transfer in childhood for relapsed clubfoot deformity after initial casting and served as the study group. Twenty-one patients (thirty-five clubfeet, 58%) were successfully treated with initial casting without relapse (the reference group). No patient in either group had subsequent relapse or required additional operative intervention associated with clubfoot deformity. The mean ankle dorsiflexion was similar between the groups. Radiographically, the tendon transfer group showed a smaller mean anteroposterior talocalcaneal angle and slightly more talar flattening than the reference group with no associated clinical differences. Peak pressures, total force distribution, and surface EMG results were not significantly different between the groups. Outcome questionnaires demonstrated no significant difference between the groups.nnnCONCLUSIONSnTibialis anterior tendon transfer is very effective at preventing additional relapse of deformity without affecting long-term foot function of patients with idiopathic clubfoot.