Nail changes in children with idiopathic congenital clubfoot deformity.

BACKGROUND/OBJECTIVES: Nail alterations are commonly seen in cases of idiopathic clubfoot and may cause parental concern. The nature of and whether these changes are congenital or develop secondary to treatment has been poorly investigated. The aim of this study was to evaluate toenail morphology in clubfoot patients at presentation, to re-evaluate them during the course of treatment for the clubfoot, and to analyze findings in the light of the few literature reports for healthy children of the same age. METHODS: Thirty infants (21 males and 9 females) with idiopathic clubfoot were prospectively enrolled at the Anna Meyer Children's University Hospital. Nails of affected and non-affected feet were evaluated by a team of pediatric dermatologists at presentation and re-evaluated once per patient during the bracing period of Ponseti treatment. RESULTS: Toenails of affected (47) and non-affected (13) feet were abnormal at presentation in 43.3% of patients, in both clubfeet (40.4%) and non-affected feet (38.5%), but most changes were physiologic or transitory alterations, commonly found in healthy children, with nail concavity (koilonychia) being the most common finding (29.7%). Changes were not related to clubfoot severity or laterality (P > .05). In most (76.9%) unilateral cases, there was concordance of nail changes between clubfoot and non-affected foot. At re-evaluation (follow-up time 410 ± 207 days), nail problems were more frequent (53.3%); ingrown toenail was the most common (21.6%). CONCLUSIONS: The presence of nail alterations seems not to be caused by clubfoot pathology and could be related to unfavorable local condition in the brace.

Comparison of Dimeglio and Pirani score in predicting number of casts and need for tenotomy in clubfoot correction using the Ponseti method.

PURPOSE: The Dimeglio and the Pirani scores are largely used to rate clubfoot at presentation and monitor correction. To date, the accuracy of these scores in predicting appropriate treatment is controversial. The aim of this study was to investigate the accuracy of Dimeglio and Pirani scores in predicting the number of casts and the need for tenotomy in clubfoot correction using the Ponseti method. METHODS: Ninety-one consecutive feet (54 patients; mean age at presentation: 28 ± 15 days) undergoing clubfoot correction using the Ponseti method were prospectively followed from first casting to correction. All feet were scored according to the Dimeglio and Pirani score. The relationships between the two scores, the number of casts and the need for tenotomy were analysed. RESULTS: Initial correction was achieved in all feet. Both Dimeglio (r = .73; p value < .0005) and Pirani scores (r = .56; p value < .000) showed good association with the number of casts. Multiple linear regression showed a high collinearity of the two scores but a more significant contribution of the Dimeglio score. Among subcomponents, hindfoot score, midfoot score, varus and muscular abnormality were independent predictors of the number of casts. Both Dimeglio and Pirani scores were significantly associated with the need for tenotomy (p value = .0000), and odds ratios and cut-off points were calculated. The receiving operator curve (ROC) analysis showed slightly better performance of the Dimeglio in comparison with the Pirani score in predicting the need for tenotomy, but the difference between the two areas under the curve (AUC) was not significant (p = .48). CONCLUSIONS: A quite accurate prediction of the number of casts and the need for tenotomy can be performed in most cases. The Dimeglio score showed slightly better accuracy in predicting both steps of Ponseti treatment.

Evolution of clubfoot deformity and muscle abnormality in the Ponseti method: evaluation with the Dimeglio score.

PURPOSE: The modality of progression of the correction along casting sessions of Ponseti method has been poorly investigated and information regarding evolution of muscular abnormalities is missing. The aim of the study was to investigate dynamics of correction of the different components of clubfoot deformity in a clinical setting. METHODS: In a prospective study, 124 clubfeet consecutively treated by a single orthopaedic surgeon were evaluated with the Dimeglio system at each casting session and score progression was determined. RESULTS: For each component a typical pattern was recorded. Cavus and medial crease showed a rapid correction. Rotation, adduction and varus corrected gradually and simultaneously. The posterior crease usually persisted until final cast was discontinued. Equinus improved progressively after each cast and then to a larger extent with Achilles tenotomy. The parameter describing poor muscular condition, reported at presentation in 39 feet (31.5%), was the only item showing extremely different dynamics of correction (from rapid and complete resolution to persistence at last cast removal), which could be explained by the large diversity of entities included (hypertonia, imbalance, fatty infiltration, fibrosis, aplasia). CONCLUSIONS: This study confirmed that dynamics of correction in clinical setting correspond essentially to theoretical principles of Ponseti method. Muscle abnormalities are not uncommon in clubfeet and have great influence on the progression of correction. If abnormalities are recorded, their evolution along the treatment should be monitored. A more objective evaluation would be required.

Residual elbow instability in children with posterior or postero-lateral elbow dislocation.

The purpose of this study is to evaluate the incidence and the severity of residual elbow instability in children treated for posterior (PED) or postero-lateral (PLED) elbow dislocation. This retrospective study included all children younger than 14 years of age with a confirmed diagnosis of acute post-traumatic elbow dislocation (ED). Subjective data such as perceived pain, stiffness and impact of daily activities, and objective data such as skin lesions, surgical-site infections, and range of motion were recorded. All patients underwent the milking test, the chair sign test (CST) and the Drawer test (DT). The functional status has been rated according to the Mayo Elbow Performance Score (MEPS) and the Roberts criteria (RC). Radiographs of the injured arm were performed at the last follow-up visit to evaluate axial alignment, growth disturbances, osteoarthrosis, heterotopic calcifications, and the presence of loose intra-articular bodies. Nineteen patients with a mean age of 9 years and 5 months at the time of injury were available for review. Six patients had PED (31.6%) and 13 PLED (68.4%); in five cases (26.3%) the dislocation was simple and in 14 cases (73.7%) it was complex. None of the patients complained of subjective symptoms of elbow instability, although 2 patients had positive CST, and one of them also had positive milking test. RC and MEPS ranged from good to excellent in all patients. Clinical examination with multiple stability tests is important to detect residual elbow instability in children treated for ED as functional scores alone may underestimate the clinical picture.

Flexible intramedullary nailing for supracondylar femoral fractures in children with Duchenne muscular dystrophy.

BACKGROUND Femoral fractures are common in patients with Duchenne muscular dystrophy (DMD) and represent a critical moment in the natural history of the disease. The immobilization required for fracture healing frequently leads to further weakening and worsening (or definitive loss) of functional abilities. Surgical treatment has been advocated in ambulatory and nonambulatory patients with rapid mobilization of patients as the main goal; however, it exposes patients to considerable anesthetic risk. CASE REPORT We present a previously unreported experience of flexible intramedullary nailing (FIN) in 2 DMD patients (aged 11.7 and 12.8 years) who were still able to walk or stand when the supracondylar femoral fractures occurred. The surgical procedures were performed with sufficient reduction and stabilization of fractures. Rapid mobilization of the patients was achieved, including muscle strengthening exercises. A prompt recovery of the upright standing position and successive ambulation was accomplished in the patient with the higher functional status before the fracture, whereas the standing ability was not recovered in the other patient. No increase of knee flexion contractures and no growth disturbances were recorded at the follow-up. CONCLUSIONS The operative treatment option should be considered by a multidisciplinary team; they should evaluate the advantages and risks for each patient considering their functional status. For ambulatory children (or patients still able to stand), FIN can represent a valid, minimally invasive, apparently growth-sparing and sufficiently stable osteosynthesis, allowing rapid rehabilitation of the patient that can limit, but not completely avoid the consequences of the femoral fracture.

The influence of laterality, sex and family history on clubfoot severity.

PURPOSE: Epidemiological studies on idiopathic clubfeet have shown a typical distribution consistent across ethnic groups: bilaterality in about 50% of cases and a male to female ratio of 2:1. Whether this corresponds also to differences in severity according to laterality and sex has been poorly evaluated. As well, the correlation between family history and severity has not been previously investigated. The aim of this study was to investigate how laterality, sex and family history influence severity and treatment. METHODS: In all, 97 infants with idiopathic clubfoot (81 male, 16 female; 55 unilateral, 42 bilateral; 19 with a first or second-degree relative affected) consecutively treated with Ponseti method were prospectively enrolled. Initial severity (according to Dimeglio and Pirani scores) and treatment (number of casts and need for tenotomy) were analyzed in the different subgroups. RESULTS: Initial severity according to Pirani (p = 0.020) and Dimeglio score (p = 0.006), number of casts (p = 0.000) and tenotomy (p = 0.045) were significantly higher in bilateral than in unilateral cases. In bilateral cases, a significant correlation was found between the right and left foot of each patient in terms of initial severity, number of casts and tenotomy performed. No statistically significant difference was found according to sex and family history. CONCLUSIONS: This study has confirmed the different behaviour of bilateral cases reported by previous studies; bilateral cases are more severe and show similar features in their right and left foot. This could be the result of different pathogenic mechanisms, likely on a genetic basis. Sex and family history did not seem to influence severity. LEVEL OF EVIDENCE: Level of evidence II.

Extraskeletal Chondroma: A Rare Cause of Trigger Finger in Children.

INTRODUCTION: Trigger finger is ten times less common than trigger thumb in infants and children and, unlike trigger thumb, may arise from a variety of underlying causes. To our knowledge, we describe the first case of pediatric trigger finger secondary to an extraskeletal chondroma. CASE PRESENTATION: We report the case of an 11-year-old girl presenting with a typical history of triggering of the fourth finger, in whom a nodule attached to the flexor digitorum superficialis was found; clinical, ultrasound, and operative findings are described. Histological analysis was diagnostic of extraskeletal chondroma, also known as chondroma of soft tissues. CONCLUSION: This is a very uncommon benign cartilaginous tumor, mostly reported in patients aged 30 to 60 years (just one pediatric extraskeletal chondroma of the hand has been described), and presentation with trigger finger has been reported just once, in a 76-year-old man. This condition should be considered in the differential diagnosis of pediatric trigger finger.

Surgical treatment of patellar dislocation in children with Down syndrome: a 3- to 11-year follow-up study.

We report results of surgical treatment of ten knees affected by patellar dislocation in six children with Down syndrome. Four knees showed a dislocatable patella (grade III according to Dugdale), two a dislocated reducible patella (grade IV) and four a dislocated irreducible patella (grade V). Symptoms included frequent falls, limping and pain. In all the cases a Roux-Goldthwait-Campbell procedure was performed. Mean age at surgery was 10 years (range 6 years and 6 months to 13 years and 4 months). Patients were reviewed at an average follow-up of 8 years and 8 months (range 3 years and 6 months to 11 years and 5 months). None showed signs of recurrence of the dislocation. The median Lysholm score improved from 57.5 to 91/100. Statistical analysis showed a significant effectiveness of the procedure in improving function, and that surgery was significantly more effective in patients with more severe disability.

Arthroscopic treatment of septic arthritis of the shoulder in a 6-year-old boy.

Septic arthritis of the shoulder in children is a rare condition. The diagnosis may present some difficulties and, consequently, appropriate treatment often is delayed. Main sequelae are humeral shortening, joint instability, premature arthritis and limited range of motion. We report a case of septic arthritis of the shoulder in a child who was treated by means of shoulder arthroscopy. A 6-year-old boy presented with a history of fever, pain and functional impairment of the shoulder that were lasting despite having undergone antibiotic therapy for 28 days (amoxicillin per os, and then teicoplanin intravenously combined with meropenem intravenously) and an arthrocentesis (no organisms were identified) in another hospital. Clinical examination (pain, swelling, warmness, functional impairment), laboratory tests (white blood cell count, 6.900/mm(3); C-reactive protein, 6.44 mg/dL; erythrocyte sedimentation rate, 119 mm), and imaging studies (radiographs, ultrasonography, computed tomography scan, magnetic resonance imaging, bone scan) performed in our department suggested the diagnosis of a stage IV (with osseous involvement) septic arthritis. Arthroscopic irrigation, debridement, synoviectomy and shaving of the osteochondral erosions were performed, in association with antibiotic therapy (teicoplanin and ceftriaxone disodium intravenously, and then amoxicillin/clavulanate per os). At 22-month follow-up, the patient was asymptomatic and showed a full range of motion. No limb length-discrepancy was found. Radiographs showed irregular profile of the humeral epiphysis without any physeal disturbances. Arthroscopic treatment for septic arthritis of the shoulder in children, though rarely reported, represents an adequate procedure for cases without bone involvement and may lead to good results, even in stage IV cases. Open arthrotomy should be reserved for cases with concomitant osseous infection after failure of arthroscopic treatment.

Pathological dislocation of the hip in neurofibromatosis: a case report.

The Authors report the case of a 28-year-old patient with type 1 neurofibromatosis, who presented a pathological dislocation of the hip. This event was preceded by the development of a neurological pattern of spastic paraparesis and decreased pain sensation in both lower limbs, secondary to the progression of a dystrophic kyphoscoliosis. Pathological dislocation of the hip in neurofibromatosis has been reported in the literature as a consequence of intra-articular growth of neurofibromas. Conversely, in the case described here, clinical and radiographic features and the absence of neurofibromas in the joint, verified by means of radiographic and histological examinations, suggested the diagnosis of neuropathic arthropathy. The treatment of hip dislocation should be differentiated according to the aetiology, given the underlying articular instability in cases of neuropathic arthropathy.

Remodelling and overgrowth after conservative treatment for femoral and tibial shaft fractures in children.

One thousand nine hundred and eighty-four children who had received conservative treatment for shaft (diaphyseal and metadiaphyseal) fractures of lower limbs (1162 femoral, 822 tibial fractures) at an average age of 8.5 years (range 0-14 years) were reviewed by clinical and radiographic investigations at an average follow-up of 6.6 years (1-15 years). Particularly, two main features were evaluated: remodelling of (angular and rotational) deformities and post-traumatic overgrowth. Mechanisms underlying these processes are discussed, based on a review of the literature, and parameters conditioning their evolution are analysed. Finally, criteria for an acceptable reduction (and limits for residual deformities that may be tolerated) at the time of conservative treatment are proposed.

Use of the Pirani score in monitoring progression of correction and in guiding indications for tenotomy in the Ponseti method: Are we coming to the same decisions?

PURPOSE: The Pirani score is widely used in the treatment of idiopathic clubfoot. Some authors recommended to base decision for Achilles tenotomy in Ponseti method on this score (hindfoot score [HFS] > 1, once reducibility of lateral head of talus (LHT) is zero) instead of originally described indications (dorsiflexion < -10-15° once complete abduction is achieved). Yet correspondence of these indications has not been evaluated. Aim of this study was to verify whether in a cohort, where decision is based on dorsiflexion, the Pirani score corresponds to the limits suggested. Secondarily, to describe temporal variation in Pirani score along treatment, which has not been previously investigated. METHODS: In a prospective study, 79 idiopathic clubfeet in 47 cases consecutively treated with Ponseti method by a single orthopaedic surgeon were evaluated at each casting session with Pirani system; score progression and scores at time of decision to perform tenotomy were determined. RESULTS: HFS and its subcomponents showed minimal improvement during subsequent sessions of casting and then rapid correction with tenotomy. Medial crease resolved rapidly. Midfoot score and its remaining subcomponents corrected gradually. Total Pirani score showed initially a progressive correction and then a more abrupt improvement with tenotomy. At the time of decision to perform tenotomy, in 8 (10.1%) of 79 cases, the decision whether or not to perform tenotomy based on dorsiflexion would have been different based on the cut-offs for Pirani score suggested. CONCLUSION: Using Pirani score in guiding indication for tenotomy may imply different decisions in a portion of cases, which should be considered when comparing series.

Stress fracture of the fourth metatarsal in a relapsed clubfoot of a 5.5-year-old child.

Stress fractures are uncommon in skeletally immature patients and usually affect adolescents involved in competitive sports, whereas they are very rare in young children. Involvement of the fourth metatarsal is very infrequent and has been associated with metatarsus adductus deformities. The authors describe a stress fracture of the proximal fourth metatarsal occurred in a 5.5-year-old child with a relapsed clubfoot. Operative correction of the deformity and cast immobilization provided consolidation of the fracture. Stress fractures of lateral metatarsals may represent a possible source of pain in recurrent clubfeet, even in young children. In these cases, early correction of the deformity is required.

Management of knee deformities in children with arthrogryposis.

Arthrogryposis includes heterogeneous disorders, characterized by congenital contractures of multiple joints. Knee involvement is very common (38-90 % of patients with amyoplasia) ranging from soft-tissue contractures (in flexion or hyperextension) to subluxation and dislocation. Flexion contractures are more common and disabling and show significant resistance to treatment and rate of recurrence. Surgical procedures vary with severity of contracture and patient age and include soft-tissue release, femoral shortening-extension osteotomy, gradual correction with Ilizarov, and femoral anterior epiphysiodesis. The presence of pterygium greatly complicates treatment, given the high rate of complications reported. Hyperextension deformities (recurvatum, anterior subluxation, and dislocation) have better prognosis for walking ability. Surgical options include percutaneous (or mini-open) quadriceps tenotomy, open quadricepsplasty, and femoral shortening osteotomy with limited arthrotomy. Knee dislocations usually require surgery and should be reduced early.

Management of hip contractures and dislocations in arthrogryposis.

Arthrogryposis represents a group of heterogeneous disorders, characterized by contractures of multiple joints at birth. Involvement of the hip is very common (55-90% of patients) ranging from soft tissue contractures to subluxation and dislocation. Isolated contracture of the hip can usually be managed conservatively: compensative mechanisms and contractures of other joints should be evaluated before planning surgery. For unilateral dislocations, open reduction is indicated to provide a level pelvis, even though the risk of stiffness, avascular necrosis and redislocation is significant. Indications for surgical reduction of bilateral dislocations are more controversial: an adequate evaluation of ambulatory potential of the child (muscular weakness, involvement of upper extremities, etc.) and of stiffness of the hips is suggested. An extensive and long-lasting programme of bracing, physiotherapy and multiple surgery may lead to some measure of functional ambulation in most cases, but high need for surgery and considerable risk of complications must be considered. This paper summarizes problems and principles of treatment for hip contractures and dislocations in arthrogryposis and provides a review of the current literature.

Femoral lengthening over elastic stable intramedullary nailing in children using the monolateral external fixator.

The authors report preliminary results of femoral lengthening performed with monolateral external fixation over elastic stable intramedullary nailing in children. Seven femoral lengthenings were performed in six patients, at a mean age at surgery of 6.6 years (range 1.5-12 years). All lengthenings were performed due to limb length discrepancy (congenital hypoplasia in four patients, growth arrest secondary to neonatal infective osteoarthritis in one, type II sacral agenesis in one). In six cases the elastic nails realised a bipolar ascending construct, in one case a descending construct. Cases were reviewed at a mean time from removal of external fixator of 34.2 months (range 15-75 months). The mean lengthening obtained was 4.8 cm (range 3.8-6.0 cm). Mean consolidation index was 42.9 days/cm. No case of axial deviation requiring surgery and no case of deep infection were recorded. No case of fracture of the regenerate occurred after removal of the external fixator. There were two premature consolidations requiring mobilisation under general anaesthesia, and a case of failure of consolidation requiring surgery. The technique reported provides indisputable biomechanical advantages, particularly increasing stability at the site of osteotomy. The characteristics of implants make this method ideal for lengthenings in children.

Sternoclavicular physeal fracture associated with adjacent clavicle fracture in a 14-year-old boy: a case report and literature review.

We report a very rare association of a physeal fracture of the medial clavicular growth plate with a fracture of the adjacent clavicle in a 14-year-old boy who fell on his shoulder while playing football. Clinical, radiographic and computed tomographic (with three-dimensional reconstruction) features are described. Open reduction, internal fixation of the lateral fracture (with a reconstructive plate) and suture of the periosteum were performed.

Osteoid osteoma of the distal thumb phalanx: a case report.

Osteoid osteomas of the hand are uncommon. Particularly, location in the thumb and involvement of the distal phalanx are very rarely reported. Long diagnostic delay and inadequate treatment are typical, since clinical features may mimic other causes more frequently found in the same site (post-traumatic, infective, rheumatic, neoplastic conditions, regional pain syndrome) and even the nail morphology may be altered, as in the reported case (watch-glass deformity). A case of an osteoid osteoma of the distal thumb phalanx in a 27-year-old man is presented, along with its clinical, radiographic and CT findings. In the Authors' opinion, curettage should be considered the treatment of choice.

Fractures of the proximal humerus in children.

Ninety-one children who had been treated for fractures of the proximal humerus (59 metaphyseal fractures; 32 epiphyseal fractures) from 1980 to 1992 at an average age of 10.7 years (range 3 to 14 years) were reviewed. In 82 cases a nonsurgical treatment (Desault bandage in 11 cases, hanging cast in nine cases, closed reduction and shoulder spica cast in 62 cases) was performed. At a mean time of 7.2 months (range 1 to 156 months), 96% of patients showed good/excellent clinical results. In 15 cases, radiographs were reviewed at a mean follow-up of 8 years (range 1 to 23.5 years): just a slight metaphyseal or meta-diaphyseal varus deformity was found in three cases. In nine cases surgery was required. Patients were reviewed by clinical examination at a mean time of 34.8 months (1-150 months), and in six cases radiographs were reviewed at a mean time of 5 years and 5 months (range 1 to 12.5 years) after surgery. In one case, a septic process occurred, that caused a severe deformity of the epiphysis and a noticeable functional deficit. Good/excellent clinical and radiographic results were achieved in the other patients. Conservative treatment of fractures of the proximal humerus in children is recommended. Surgery should be reserved for specific cases.

Congenital pseudarthrosis of the fibula and valgus deformity of the ankle in young children.

Treatment of cases of congenital pseudarthrosis of the fibula presenting with severe or progressive valgus deformity in early childhood is challenging. The purpose of the study presented was to analyse the deforming mechanisms to get therapeutic recommendations for this condition. A 2.5-year-old child was treated by resection of pseudarthrosis and distal tibiofibular fusion (Langenskiold operation) with autogenous bone grafting, associated with subtalar arthroereisis: progressive correction of the deformity was achieved. A 21-month-old child was treated with resection of pseudarthrosis, homologous bone grafting and fibular osteosynthesis: after failure, Langenskiold operation with autogenous bone grafting was performed and stabilization of the deformity was achieved. In young children with congenital pseudarthrosis of the fibula and valgus deformity, early surgical treatment is advisable: fibular ostheosynthesis has limited indications; distal tibiofibular fusion remains the treatment of choice, providing correction that may exceed the simple stabilization of deformity expected. Radiographic findings lead the authors to suggest that part of the effectiveness of the procedure is due to abnormal growth patterns.