Benjamin Joseph

Manipal University, Udupi, Karnātaka, India

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Publications (44)45.26 Total impact

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    ABSTRACT: AIM:: This study aimed to determine if a new measure of sphericity of the femoral head, the Sphericity Deviation Score (SDS), could be estimated reliably at the stage of healing of Legg-Calvé-Perthes disease (LCPD) and to determine if the SDS at the healing stage reflects the outcome at skeletal maturity. The SDS was measured with image analysis software on radiographs of 120 children with unilateral LCPD at healing of the disease and at skeletal maturity. The reproducibility of measurement was assessed. SDS values for hips in different Stulberg classes were calculated. On the basis of SDS values at healing of 82 children, a logistic regression model was developed to determine the probability of a good outcome at skeletal maturity. The validity of the model was tested on another 38 children. The SDS values at the stage of healing and at skeletal maturity were comparable. The SDS values were lowest for Stulberg Class I hips and highest for Class IV and V hips. The validated regression model showed a very high probability of a good outcome (Stulberg Class I or II) at skeletal maturity if the SDS at the stage of healing was below 10. A reliable estimate of the outcome of LCPD at skeletal maturity can be made by computing the SDS as soon as the disease heals.
    Journal of pediatric orthopedics 03/2014; · 1.23 Impact Factor
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    ABSTRACT: Mucopolysaccharidosis IVA is an autosomal recessive inborn error of metabolism caused by the mutations in the Nacetylgalactosamine-6-sulfatase (GALNS) gene and manifests with a spondyloepiphyseal dysplasia. Bidirectional sequencing of all the coding region was performed in 60 patients from 58 families. In silico analysis was carried out for novel missense mutations to peredict the effect of mutations on protein function. We identified 37 different mutations, 26 of which had not been previously described. The 26 novel mutations consisted of 22 missense mutations (p.MlI, p.N32T, p.L36R, p.P52L, p.P77S, p.C79R, p.S82P, p.L86P, p.H142P, p.G188S, p.Y191D, p.N204T, p.F216S, p.W230C, p.R251Q, p.A291S, p.T313M, p.G317R, p.H329P, p.R386S, p.E450G, p.C501S), one nonsense mutation (p.Q414X), 3 intronic variations (c.120+1G>C, c.1003-3C>G and c.1139+1G>A) that affect the splice sites and 11 previously reported mutations. The missense mutations p.S287L and p.A291S account for 9.1 % (10/111) and 7.3 % (8/111) of the cases respectively and were the common mutations in Indian patients. This is the first report of screening for mutations in GALNS gene in the Indian population and the largest number of patients studied in the literature till date. The identification of these mutations has helped 5 families for prenatal diagnosis.
    European Human Genetics Conference, Paris, France; 06/2013
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    ABSTRACT: Background Though there is an impression that proximal femoral varus osteotomy (FVO) can result in a valgus deformity at the knee there is no agreement on this issue. This study was undertaken to ascertain whether a FVO predisposes to the development of genu valgum in children with Legg–Calvé–Perthes disease (LCPD). Methods One hundred and one children with unilateral LCPD who underwent a FVO during the active stage of the disease and 32 children who were treated non-operatively were followed till skeletal maturity. The FVO was performed with a 20° varus angulation in all the patients and weight-bearing was not permitted till the stage of reconstitution. The alignment of the knee was assessed clinically at skeletal maturity. A subset of 33 operated children also had full length standing radiographs of the limbs. The mechanical axis deviation, femur-tibial angle, lateral distal femoral angle and the medial proximal tibial angle of both limbs were measured on these radiographs. Results The frequency of clinically appreciable mal-alignment of the knee was not greater on the affected side in patients who had undergone FVO when compared to the unaffected limb and also when compared to the affected limb in non-operated patients. The mechanical axis of the lower limb of operated children was relatively in more valgus than that of normal limbs but they fell within the normal range. Conclusion This study does not support the impression that a proximal femoral osteotomy for LCPD predisposes to clinically discernable degrees of genu valgum in children who have had 20° of varus angulation at the osteotomy site and who have avoided weight-bearing for a prolonged period following surgery. Further studies are needed to clarify if genu valgum would develop if early post-operative weight-bearing is permitted. Level of evidence III.
    Journal of Children s Orthopaedics 06/2013; 7(3).
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    ABSTRACT: Large sample sizes are needed if discrete outcome measures are used to perform outcome studies, whereas more practicable sample sizes are sufficient if continuous outcome measures are used. Recognizing the need to devise reliable continuous outcome measures for assessing the outcomes of treatment of Legg-Calvé-Perthes disease, we evaluated a method of quantifying the shape and size of the femoral head and the femoral-acetabular relationship from computer images of anteroposterior and lateral radiographs with the help of image-analysis software. Radiographs of 121 skeletally mature individuals with healed Legg-Calvé-Perthes disease were analyzed, and the sphericity deviation score, femoral head enlargement, femoral neck growth inhibition, and composite femoral congruency arc were calculated. Each of these measurements was reproducible, with distinctly different values for hips in each of the Stulberg classes. Measures of sphericity and congruence (the sphericity deviation score and the composite femoral congruency arc) could very clearly discriminate between spherical and aspherical hips, and there was a strong correlation between these two measurements. The study shows that it is possible to have reliable quantitative measures of the outcome of Legg-Calvé-Perthes disease at skeletal maturity.
    The Journal of Bone and Joint Surgery 02/2013; 95(4):354-61. · 3.23 Impact Factor
  • Stéphane Tercier, Hitesh Shah, Benjamin Joseph
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    ABSTRACT: We encountered problems with the Curtis and Fisher technique of quadricepsplasty for congenital quadriceps contracture, including wound dehiscence, insufficient lengthening of the quadriceps and instability of the knee. We modified the operative technique to address these three problems. We undertook this study to evaluate the results of the modified technique of quadricepsplasty to determine if we succeeded in overcoming these limitations of the original technique. Twenty children (33 knees) underwent the modified Curtis and Fisher quadricepsplasty through a lateral incision; a long tongue of the rectus femoris was raised and the vasti mobilised without dividing the lateral retinaculae till the collateral ligaments. The children were followed up for a mean period of 63 months and evaluated. The healing of the wound, active and passive range of motion (ROM) of the knee, the stability of the knee, quadriceps power and knee function were assessed. Primary wound healing occurred in 32 of 33 knees. Adequate lengthening of the quadriceps sufficient to facilitate knee flexion to 90° was possible. Considerable improvement in the ROM was noted. In non-syndromic congenital dislocation of the knee (CDK), the quadriceps power was Grade 5, but minor degrees of extensor lag was noted. In a proportion of patients, minor degrees of joint instability was present. The majority of children were community walkers. The overall results were better in non-syndromic CDK than in children with arthrogryposis, but differences of some variables were not significant. The modifications to the original Curtis and Fisher technique overcame the specific problems they were expected to avoid.
    Journal of Children s Orthopaedics 10/2012; 6(5):397-410.
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    ABSTRACT: The study was undertaken to: (1) describe the characteristic radiological features and problems of management of the loss of one condyle of the femur or tibia following septic arthritis of the knee in infancy and (2) test a hypothesis of the cause of the loss of a single condyle. Radiographs of eight children with the loss of one condyle of the femur or the tibia following septic arthritis in infancy were reviewed. The course and outcome in two of these children who underwent reconstructive operations were studied. The knees of 35 stillborn foetuses were dissected to determine if the presence of synovial septae could account for the isolated loss of one condyle following infection. All eight cases showed characteristic features of loss of half the epiphysis, the underlying physis and part of the adjacent metaphysis; the other condyle was totally spared. The two children who underwent elaborate reconstructive procedures had poor outcomes at skeletal maturity, despite a series of additional operations. The foetal cadaveric study showed that complete infrapatellar synovial septae are present in some foetuses approaching 40 weeks of gestation. The pattern of loss of a femoral or tibial condyle following septic arthritis is consistent with total preservation of the other condyle. The outcome of surgical reconstruction of the missing condyle is poor. The presence of a complete synovial septum could result in the localisation of infection to one half of the joint, with the destruction of one condyle.
    Journal of Children s Orthopaedics 08/2012; 6(4):319-25.
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    ABSTRACT: Objective: To determine if the frequency of left-handedness is high in children with spastic cerebral diplegia. Design: Case-control study. Methods: One hundred and eleven children with spastic diplegic cerebral palsy and 444 age- and gender-matched controls were studied. The handedness of each child was assigned on the basis of responses to questions on the hand preference for writing and drawing, feeding and throwing a ball. The data were analysed by conditional logistic regression and computing the odds ratio and 95% confidence intervals for left handedness. Results: Of the 111 children with spastic diplegic cerebral palsy, 45 were left-handed, while 13 of 444 normal children were left-handed. The odds ratio for left-handedness in children with diplegic cerebral palsy as compared to normal children was 27.33 (95% CI = 11.63, 64.25). Conclusion: The study shows that left-handedness is very frequently encountered in children with spastic diplegic cerebral palsy.
    Developmental neurorehabilitation 07/2012; 15(5):386-9.
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    ABSTRACT: PITX1 is a bicoid-related homeodomain transcription factor implicated in vertebrate hindlimb development. Recently, mutations in PITX1 have been associated with autosomal-dominant clubfoot. In addition, one affected individual showed a polydactyly and right-sided tibial hemimelia. We now report on PITX1 deletions in two fetuses with a high-degree polydactyly, that is, mirror-image polydactyly. Analysis of DNA from additional individuals with isolated lower-limb malformations and higher-degree polydactyly identified a third individual with long-bone deficiency and preaxial polydactyly harboring a heterozygous 35 bp deletion in PITX1. The findings demonstrate that mutations in PITX1 can cause a broad spectrum of isolated lower-limb malformations including clubfoot, deficiency of long bones, and mirror-image polydactyly.
    European journal of human genetics: EJHG 01/2012; 20(6):705-8. · 3.56 Impact Factor
  • N D Siddesh, Hitesh Shah, Benjamin Joseph
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    ABSTRACT: This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD(HG)). Radiographs of 271 adult patients with SEMD(HG) were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips. Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores. Degenerative arthritis of the hip develops in the majority of patients with SEMD(HG); the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned.
    Skeletal Radiology 11/2011; 41(8):939-45. · 1.74 Impact Factor
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    ABSTRACT: The etiology of Legg-Calvé-Perthes disease (LCPD) remains unknown. A few studies have suggested passive smoke inhalation may be a risk factor, although the association is not confirmed and a causal relationship has not been established. We therefore undertook this study to confirm an association between environmental tobacco smoke, firewood smoke, and socioeconomic status and the risk of LCPD. We prospectively recruited 128 children with LCPD and 384 children attending the hospital for other orthopaedic complaints. The control subjects were frequency-matched with the cases by age and gender. Conditional logistic regression was used to assess the association between the exposures and risk of LCPD. The main risk factors for LCPD were indoor use of a wood stove (adjusted odds ratio [OR], 2.56) and having a family member who smoked indoors (adjusted OR, 2.07). Children from the middle socioeconomic group appeared to be at a greater risk of developing LCPD (adjusted OR, 3.60). This study provides further evidence that environmental tobacco smoke is associated with an increased risk of LCPD. Exposure to wood smoke also appears to be a risk factor. However, it remains unclear why there are profound differences in the incidence of the disease between regions when the prevalence of smoking is comparable and why bilateral involvement and familial disease are infrequent.
    Clinical Orthopaedics and Related Research 11/2011; 470(9):2369-75. · 2.79 Impact Factor
  • Charles T Price, Benjamin Joseph
    Orthopedic Clinics of North America 07/2011; 42(3):xi. · 1.25 Impact Factor
  • Benjamin Joseph, Charles T Price
    Orthopedic Clinics of North America 07/2011; 42(3):437-40. · 1.25 Impact Factor
  • Benjamin Joseph
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    ABSTRACT: The shape of the femoral head at the time when Perthes disease heals is the most important determinant of the risk for degenerative arthritis; hence, the shape of the femoral head and congruence of the hip are the most useful outcome measures. Although several prognostic factors that predict femoral head deformation may be identified during the course of Perthes disease, only two prognostic factors may be identified early enough to institute preventive intervention: femoral head extrusion and the age at onset of the disease. Femoral head extrusion is the only factor that may be influenced by treatment.
    Orthopedic Clinics of North America 07/2011; 42(3):303-15, v-vi. · 1.25 Impact Factor
  • Benjamin Joseph, Charles T Price
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    ABSTRACT: The aim of treatment of Perthes in the early part of the disease is to prevent the femoral head from getting deformed by muscular forces and weight-bearing stresses transmitted across the acetabular margin. To achieve this, femoral head extrusion must be preempted in children who are older than 8 years at onset of the disease by ensuring containment as soon as the disease is diagnosed. In children younger than 8 years in whom femoral head extrusion occurs, containment must be obtained by the early stage of fragmentation.
    Orthopedic Clinics of North America 07/2011; 42(3):317-27, vi. · 1.25 Impact Factor
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    ABSTRACT: Crouch gait in cerebral palsy is associated with spasticity and contracture of the hamstrings and weakness of the extensors of the hip and knee and ankle plantar flexors. Different treatment options have been described in the literature to deal with this difficult problem. We devised a different protocol of treatment aimed at correction of the flexion deformity of the knee, weakening of the hamstrings, and augmenting the power of the knee and hip extension, which we used on 17 children with severe crouch. This surgery, performed in 2 stages, entailed shortening of the femur, plication of the patellar tendon, transfer of the semitendinous to the back of the femur, and fractional lengthening of the other hamstrings. The degree of fixed deformity, the popliteal angle, quadriceps power, range of knee motion, ambulatory status and the efficiency of gait, and the position of the patella were evaluated before surgery and again after a minimum 2-year follow-up. The gait improved and the power of the quadriceps and the range of knee motion increased. The flexion deformity and popliteal angle decreased significantly. Patella alta was corrected and all fragmentation of the tibial tuberosity and fractures of the patella healed. The Functional Mobility Scores and the ambulatory capacity increased in all the children. There was no evidence of damage to the sciatic nerve in any patient. The method of treatment of severe crouch gait outlined in this study seems to be an effective and safe method of dealing with this difficult problem. Level of evidence: IV.
    Journal of pediatric orthopedics 12/2010; 30(8):832-9. · 1.23 Impact Factor
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    ABSTRACT: Since the nature of complications following the harvesting of bone from the tibia in children is not well documented in the literature, we undertook this study in order to determine the frequency and nature of donor site complications following the harvesting of large cortical strut grafts from the tibial diaphysis in children. During the past 19 years, tibial cortical grafts were harvested from 47 children on 77 separate occasions, mainly for the treatment of congenital pseudarthrosis of the tibia. The technique of graft harvesting was identical in every case. Case records of these 47 children were reviewed. Forty of these children were reviewed at a mean period of 5.5 years. No immediate post-operative complications were noted and, at follow-up, apart from mild bowing of the anterior cortex of the tibia, no deformities were encountered. The tibial cortex reformed completely and this facilitated repeat graft harvesting when required. The only major complication was a stress fracture of the tibia in one boy. Harvesting cortical bone graft from the tibia is simple and is fraught with negligible morbidity.
    Journal of Children s Orthopaedics 10/2010; 4(5):417-21.
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    ABSTRACT: To evaluate the effect of prophylactic epiphyseodesis of the greater trochanter in Perthes' disease, 62 children with unilateral Perthes' disease who underwent trochanteric epiphyseodesis combined with varus osteotomy of the femur during the active stage of the disease (mean age at surgery: 8.4 y) and 20 controls were followed up until skeletal maturity. On radiographs taken at skeletal maturity, the articulo-trochanteric distance, the center-trochanteric distance, the length of the abductor lever arm, the neck-shaft angle, the radius of the femoral head, and the Reimer's migration index of normal and affected hips were measured. The shape of the femoral head was assessed according to the criteria of Mose. The range of hip motion, the strength of hip abduction, and limb lengths were measured and the Trendelenburg sign was elicited. The mean values of articulo-trochanteric distance and center-trochanteric distance were greater and the frequency of a positive Trendelenburg sign was less in children who had undergone trochanteric epiphyseodesis than in children who had no surgery (P<0.01). Trochanteric epiphyseodesis achieved optimal trochanteric growth arrest in 60% of operated children; the procedure was not effective in 30%, and in 10% of children there was overcorrection. Logistic regression analysis showed that the size of the femoral head at healing and the age at surgery were variables that significantly influenced the effectiveness of trochanteric growth arrest. At skeletal maturity, the mean shortening of the affected limb in operated children was 0.44 cm (SD 0.68 cm), whereas that of non-operated children was 0.86 cm (SD 0.78 cm) (P<0.05). The range of motion of the hip was excellent and there were no significant differences in the range of motion among children with optimal correction, under-correction, and overcorrection. A probability curve plotted on the basis of a logistic regression model suggests that effective trochanteric arrest may be achieved in a high proportion of children operated at or before 8.5 years of age and in half the children operated between the age of 8.5 years and 10 years. On the basis of this study, we recommend prophylactic epiphyseodesis of the greater trochanter as a means of minimizing trochanteric overgrowth and resultant Trendelenburg gait in older child with Perthes' disease.
    Journal of pediatric orthopedics 12/2009; 29(8):889-95. · 1.23 Impact Factor
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    ABSTRACT: We studied case records and radiographs of 20 children with congenital posteromedial bowing of the tibia (CPMBT) retrospectively to determine the pattern of correction of the bowing, the associated growth abnormalities of the tibia and fibula, and the role of surgical intervention in CPMBT. The magnitude of diaphyseal bowing in two planes and the physeal inclination were measured. Abnormalities of ossification of the distal tibial epiphysis and inclination of the distal articular surface if present were noted and shortening of the tibia was recorded. The rate of resolution of deformity was noted from sequential radiographs and expressed as percentage reduction per month of follow-up. At initial presentation the magnitude of deformity varied; the most severe posterior diaphyseal bow was 70 degrees whereas the most severe medial diaphyseal bow was 64 degrees. Two distinct mechanisms seem to be responsible for resolution of the deformity in CPMBT; one involves physeal realignment and the other involves diaphyseal remodeling. In the first year of life, rapid resolution of angulation was noted; the rate of resolution reduced significantly thereafter. In a proportion of children with CPMBT residual deformity may persist till over 4 years of age. Physeal realignment occurred at a faster rate than diaphyseal remodeling. The degree of shortening was related to the severity of bowing and shortening as great as 40% was noted in a patient. Wedging of the distal tibial epiphysis and fibular hypoplasia with valgus inclination of the distal tibial articular surface occur in some children with CPMBT. Eccentric ossification of the distal tibial epiphysis in early childhood may be a predictor of wedging of the distal tibial epiphysis later on. We recommend all the children with CPMBT to be followed up periodically till skeletal maturity, to identify cases with residual bowing, ankle deformity, muscle weakness, and limb length inequality as active surgical intervention may be needed to correct these problems.
    Journal of pediatric orthopaedics. Part B / European Paediatric Orthopaedic Society, Pediatric Orthopaedic Society of North America 04/2009; 18(3):120-8. · 0.66 Impact Factor
  • Renjit A Varghese, Benjamin Joseph
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    ABSTRACT: Congenital absence of the patella and aplasia of the muscles are very rare anomalies. We describe a 4-year-old boy with bilateral congenital aplasia of the patella and agenesis of the distal third of the quadriceps muscle who was unable to walk owing to the lack of active knee extension. The features of this child differed from all other conditions associated with patellar aplasia. The continuity of the quadriceps mechanism was restored and he began walking normally. This appears to be the first report of the combination of aplasia of the patella and the distal third of the quadriceps that was successfully treated.
    Journal of Pediatric Orthopaedics B 10/2007; 16(5):323-6. · 0.53 Impact Factor