Ellis–van Creveld is a dwarfing syndrome transmitted as an autosomal recessive trait. The constant features of the condition include acromelic–micromelic dwarfism, ectodermal dysplasia involving the nails, teeth and gums, postaxial polydactyly of the hands and congenital heart disease. Congenital heart disease affects 50–60 % of all patients and nearly 50 % of patients die by 18 months of age from cardiopulmonary complications. This study is intended to characterise the orthopaedic manifestations of Ellis–van Creveld based on the authors’ unique opportunity to interview and examine the largest group of patients to date in the literature.
Detailed interviews, physical examinations and/or radiographs were available on 71 cases of Ellis–van Creveld syndrome. Data were collected from physical examinations, radiographs, computed tomography (CT) reconstruction and magnetic resonance imaging (MRI) of the knee. Pathoanatomy of the knee was reinforced by the direct surgical observation of 25 limbs surgically managed during adolescence and puberty.
A number of interesting clinical and radiographic abnormalities were noted in the upper extremities and lower extremities, but by far the most significant orthopaedic finding was a severe and relentlessly progressive valgus deformity of the knee. Although many patients had difficulties making a “fist” with the hand, no patient reported any functional disability. The severe valgus deformity of the knee is the result of a combination of profound contractures of the iliotibial band, lateral quadriceps, lateral hamstrings and lateral collateral ligament, leading to lateral patellar subluxation and dislocation. The lateral portion of the upper tibial plateau presents with cupping and progressive depression of the lateral plateau, along with severe valgus angulation of the proximal tibia and fibula. A proximal medial tibial exostosis is seen in nearly all cases.
This is the largest group of Ellis–van Creveld syndrome patients identified in the literature. An understanding of the orthopaedic pathoanatomy of the knee deformity is critical to determining the appropriate surgical management. This paper characterises the orthopaedic manifestations of Ellis–van Creveld syndrome and especially identifies the pathoanatomy of the severe and progressive valgus knee deformity.
Level of evidence
Electronic supplementary material
The online version of this article (doi:10.1007/s11832-013-0541-4) contains supplementary material, which is available to authorized users.
Pathoanatomy; Chondroectodermal dysplasia; Ellis–van Creveld syndrome
Slipped capital femoral epiphysis (SCFE) represents the most common disorder of the hip in adolescents and a preliminary stage of degenerative joint disease. Up to now, functional outcome evaluation measured by objective instruments has been commonly neglected. The present study investigates whether the pathoanatomy of the hip joint after SCFE—analyzed on a standard X-ray—match functional results gained by three-dimensional gait analysis. A variation of functional outcome depending on the radiological findings after growth arrest is hypothesized.
Thirty-seven SCFE patients after growth arrest [mean age 18.5 years, standard deviation (SD) 4.61] with unilateral affection were included. The pathoanatomy of the hip joint was classified according to the radiological index of Heyman and Herndon and to aspherity. Three-dimensional gait analysis parameters were evaluated and subgroup analysis was performed according to the radiological results.
The radiological findings revealed very good results in general (average comprehensive index of Heyman and Herndon 94 ± 9 %, aspherity grade <2). Significant deviations of gait parameters in relation to the radiological result were an increase in step width, sagittal range of motion (ROM) of the pelvis and foot progression for the worse subgroup.
Taken as a whole, the pathoanatomy of the hip joint after SCFE matched the functional results gained by gait analysis. Functional outcome varied slightly depending on the radiological findings after growth arrest. Differences were most pronounced for foot progression. Only with the help of gait analysis was it possible to describe deviations more precisely and objectively. Further studies are required in order to show which alterations are relevant for the development of secondary osteoarthritis.
Slipped capital femoral epiphysis; Instrumented gait analysis; Radiological outcome; Growth arrest
Various surgical techniques for thumb duplication have been described. The main goal in thumb reconstruction is to achieve optimal function, stability, and shape. Few publications have quantified the functional results of each surgical option and factors that may affect these outcomes.
We performed a retrospective review during the period from 1990 to 2011 of 115 surgery patients, 99 of whom had a mean postoperative follow-up of 44.1 months. They were classified according to the Wassel classification. We assessed the surgical technique, functional results, and complications, and evaluated the probable prognostic value of age and Wassel classification.
This case series comprised mainly female patients (62 %) and the right hand (74 %), with a mean age at operation of 20 months. The most common group was Wassel type IV (54 %). On the basis of the Tada scoring system, we obtained 91 good results (91 %). Complications occurred in 27 patients (27 %), mainly instability and axis deformation. In a comparison of preoperative factors (age at the time of surgery and Wassel classification) with the final results (Tada score and complications), the association between older age at surgery and complications (p = 0.0001) and Wassel types III and IV and complications (p = 0.0210 and p = 0.0095) was statistically significant.
Following the basic concepts of anatomical reconstruction, we obtained good results in most patients. The main complications were instability and axial deformity. These were most common in patients who underwent operations at an age of more than 12 months, and patients with Wassel types III and IV deformities.
Prognostic value; Polydactyly; Thumb duplication; Wassel classification; Tada score; Hand reconstructive surgery
Patients with lipomyelomeningocele (LMMC) represent a unique population within the spectrum of spinal dysraphism. The natural history of LMMC remains poorly defined. The description and prevalence of the presenting orthopaedic clinical signs and symptoms for LMMC have been infrequent and often documented only in general terms. The goal of this study is to define the patterns and prevalence of presenting clinical musculoskeletal signs and symptoms in LMMC patients.
This study was a retrospective review of charts of all patients identified as having LMMC in our spina bifida clinic. Patient charts with incomplete data or diagnoses other than LMMC were excluded from the analysis. Data collected included age at initial tethered cord release (TCR); repeat TCR; limb length discrepancy; foot deformities; asymmetry of motor and sensory deficits; presence of scoliosis; orthotic needs; assistive devices; functional status.
We identified 32 patients with LMMC (21 female and 11 male patients). The majority of patients had their primary TCR by ≤1 year of age (59 %), with 22 and 19 % having primary TCR at ages 1–15 and >15 years, respectively. Fifteen patients had at least one repeat TCR, with ten of these having more than one repeat TCR. A significant relationship was noted between low back/radicular pain and repeat TCR (p < 0.001). Ten patients (31%) had a limb length discrepancy of >2.5 cm, and 53 % of patients had asymmetric involvement. Nine patients (28 %) had scoliosis of whom only one required operative treatment. Fifteen patients had foot deformities. Thirteen patients (41 %) had two or more orthopaedic procedures in addition to other neurologic or urologic procedures.
The presenting musculoskeletal clinical signs and symptoms in patients with LMMC are uniquely different in terms of both pattern and frequency compared to myelomeningocele and other forms of spinal dysraphism. We noted a high prevalence of asymmetrical involvement, a high operative burden, and a high rate of repeat symptomatic tethered cord syndrome requiring TCR. As previously noted by others, TCR in LMMC does not prevent long-term functional deterioration. These findings may be important to our colleagues providing counsel to their patients with LMMC and to their families.
Lipomyelomeningocele; Spinal dysraphism; Spina bifida; Myelomeningocele; Tethered cord
Studies of pediatric and adolescent fractures in general report a significant increase in the incidence of upper-extremity fractures as well as in their surgical treatment. The aim of this study was to determine the trends of the incidence and treatment of distal humeral fractures in hospitalized 0- to 18-year-old patients in Finland.
The study included the entire pediatric and adolescent (<19 years) population in Finland during the 24-year period from 1 January 1987 to 31 December 2010. Data on hospitalized patients were obtained from the nationwide National Hospital Discharge Registry where information is collected from all hospital categories (private, public, and other). Surgical treatment was categorized into three groups; (1) reposition with casting; (2) reposition or reduction and osteosynthesis; (3) reposition or reduction and external-fixation and other fixation methods. Patients were classified into three groups according to age: 0–6 years, 7–13 years, and 14–18 years. Annual incidences were calculated using the annual mid-year population census obtained from the Official Statistics of Finland.
During the 24-year study period, there were a total of 12,590 hospitalizations with a main or secondary diagnosis of distal humeral fracture. In children aged 0–12 years the overall incidence of hospitalization increased 30 % during the 24-year study period, from 4.5 per 10,000 person-years in 1987 to 5.8 per 10,000 person-years in 2010. There were a total of 5,548 operations. During the study period, surgical treatment by repositioning or reduction with osteosynthesis due to a distal humeral fracture increased by fivefold in patients aged <6 years and by twofold in patients aged 7–12 years of age. The incidences of fracture and treatment in children older than 13 years did not change.
The incidence of distal humeral fractures and the incidence of repositioning with osteosynthesis increased remarkably in prepubertal children during the 24-year study period in Finland.
Distal humeral fracture; Incidence; Pediatric; Treatment
Metachondromatosis is a rare genetic disease of osteochondroma and enchondroma formation, caused by loss of function of the PTPN11 gene. It is distinct from other similar conditions such as multiple osteochondromas and hereditary multiple exostoses by the distribution and orientation of lesions, and pattern of inheritance. Lesions typically occur in hands, feet, femora, tibiae and the pelvis. Lesions are typically reported to regress in adulthood.
We reviewed the current literature on metachondromatosis, and present four new cases in a family with metachondromatosis.
Long-term follow up data reveal spontaneous regression of lesions by skeletal maturity. Complications may include nerve palsy due to the mass effect of lesions, avascular necrosis of the femoral head and angular deformity of long bones. Histopathological analysis has demonstrated that lesions in metachondromatosis are a mix of osteochondromas and enchondromas; however, one case of chondrosarcoma has been reported.
Lesions associated with metachondromatosis may cause a variety of complications due to mass effects; however, they are often asymptomatic, cause cosmetic concerns and, importantly, most regress spontaneously. Regular clinical review with selective imaging to monitor for such complications is appropriate, but uncomplicated lesions are unlikely to require surgical intervention.
Metachondromatosis; Review; Case report; Osteochondroma; Enchondromatosis
To evaluate functional electrical stimulation (FES) neuroprothesis as a method to improve gait in hemiplegic patients, using three different gait scoring methods as measures.
Five hemiplegic patients (four with cerebral palsy at GMFCS I, one with diffuse pontine glioma) with a mean age of 16.5 years were given a FES neuroprosthesis (NESS® L300™) that was applied and calibrated individually. After an adaptation period during which the participants increased their daily use of the neuroprosthesis, gait was assessed with the stimulation off and with the FES on. Kinematic, kinetic, and temporal spatial data were determined using motion analysis and summarized by three scoring methods: Gait Profile Score (GPS), Gait Deviation Index (GDI), and Gillette Gait Index (GGI). Indices were calculated using the Gaitabase program available online. Patients were followed for a minimum of 1 year.
When comparing gait with and without stimulation, all scoring methods showed improvement. GPS and GDI of the affected leg were significantly improved: 12.23–10.23° (p = 0.017) and 72.36–78.08 (p = 0.002), respectively. By applying the movement analysis profile, the decomposed GPS score, we found that only the ankle dorsiflexion and the foot progression angle were significantly changed. GGI of the affected leg showed improvement, but without statistical significance: 168.88–131.64 (p = 0.221). Total GPS of legs and the GPS, GDI, and GGI of the nonaffected leg showed improvement without statistical significance. At the 1-year follow-up, all patients expressed high satisfaction and continued to use the device.
Dorsiflexion functional electrical stimulation improves gait in hemiplegic patients, as reflected by GPS, GDI, and GGI.
Neuroprosthesis; Gait analysis; Gait indices; Cerebral palsy; Drop foot; Functional electrical stimulation
To investigate the intra- and inter-observer reliability of the coronal curvature asymmetry of children with adolescent idiopathic scoliosis (AIS) using the center of lamina (COL) method on ultrasound (US) images.
A cadaver spinal column phantom which was manipulated to present 30 scoliotic curves of varying severity of scoliotic deformities was scanned using both the US and laser scanner (LS) systems. Three observers of varying experience and skill measured the coronal curvature using the Cobb method on the LS images and the COL method on the US images. All of the measurements were performed twice, with a 1-week interval to reduce memory bias. The intra-class correlation coefficient (ICC), the mean absolute differences (MAD), and the error index (EI) were calculated to determine the agreement on selecting the end vertebrae. In addition, five AIS subjects were scanned using the US system. One observer measured the coronal curvature on the US images twice, and the measurements were compared with the Cobb angle reported in the clinical records.
In the phantom study, the COL method showed high intra- and inter-observer reliabilities, with all ICC values >0.88. The maximum MAD of the COL measurements between different sessions among all observers was <4.1°. The EI values of the US method had similar end-vertebra selections as the LS method. The results of the pilot study showed a high intra-reliability for the US measurements. The measured difference between the Cobb and COL methods was 0.7° ± 0.5°.
The COL method using US images appears to be a very reliable method for measuring the coronal curvature in AIS without the need to expose the patient to radiation.
Adolescent idiopathic scoliosis; Center of lamina method; Coronal curvature; Reliability; Ultrasound image
Hip dysplasia is prevalent in nonambulatory children with cerebral palsy, and may contribute to a decreased quality of life (Lonstein in J Pediatr Orthop 6:521–526, 1). Reconstructive procedures such as a femoral varus derotation osteotomy with or without a pelvic osteotomy are commonly employed with the goal of achieving and maintaining well reduced hips.
The goals of this study are both to characterize the complications of reconstructive procedures and to identify risk factors that may contribute to these complications.
Patients and methods
A retrospective analysis was conducted among 61 nonambulatory children (93 hips) with cerebral palsy who underwent a femoral varus derotation osteotomy, with or without an open reduction and/or pelvic osteotomy, from 1992 through 2008 at our institution. The average patient age was 8.1 years (2.6–14.7) and the mean follow-up time was 5.9 years (2.1–15.9).
The cumulative complication rate per patient including failures to cure was 47.6 %. Spica casting was found to be a risk factor for all complications (P = 0.023); whereas patients younger than 6 years old (P = 0.013) and children with a tracheostomy (P = 0.004) were found to be risk factors for resubluxation following surgery.
Although reported complication rates of hip reconstructive procedures performed upon children with cerebral palsy have varied considerably, those with more severe disease have experienced more complications. We report our tertiary referral center’s complication rate and our institutional experiences with risk factors for complications and failures to cure.
Level of evidence
IV, Retrospective case series.
Cerebral palsy; Nonambulatory; Complications; Hip surgery
This study assessed whether avascular necrosis (AVN) is correlated with the presence or absence of the ossific nucleus (ON) at the initiation of conservative treatment for developmental dysplasia of the hip (DDH). To date, the correlation between the presence of the ON and AVN manifestations remains ambiguous.
The medical records of 148 patients with 234 dislocated hips who presented at our institution between January 2006 and December 2007 were reviewed. Based on ultrasound examination, the hips were classified according to Graf IIIa, IIIb, and IV criteria. Patients aged >6 months were simultaneously examined by standardized pelvis radiography.
The ON was present in 84 hips (35.9 %) at the beginning of treatment. Treatment was begun at a mean age of 5 months, with overhead traction for 2 weeks followed by arthrography and a spica cast for 4 weeks. Afterwards, we used a Tübingen hip-flexion splint. The mean age at final follow-up was 87 months. Hips were radiographically evaluated at last follow-up according to the Ogden–Bucholz AVN classification scheme. There was no significant difference in AVN prevalence between ON− versus ON+ hips in children aged ≤10 months (P = 0.681), whereas when all age groups were analyzed together, AVN was significantly increased in ON+ hips (P = 0.002). Clinical examination revealed no differences in limping, leg length inequality, and range of motion of hips in the ON− versus ON+ groups.
We conclude that DDH treatment should be performed early without regard to the presence or absence of the ON. Reduction should not be delayed beyond >10 months of age because any delay in treatment increases the incidence of AVN.
Ossific nucleus; Avascular necrosis; Developmental dysplasia of the hip
Longitudinal epiphyseal bracket or bracket epiphysis is an uncommon disorder of growth. Alternatively known as a delta phalanx, it is due to an anomalous secondary ossification center that extends longitudinally along the diaphysis. Although rare, longitudinal epiphyseal bracket most commonly manifests in the hands as clinodactyly and in the feet as hallux varus. Previously, longitudinal epiphyseal bracket has been treated with angular osteotomy, but we recommend early surgical physiolysis. We describe this uncommon disorder, our current recommendation for treatment, and present three illustrative cases.
Longitudinal epiphyseal bracket; Bracket epiphysis; Delta phalanx; Hallux varus; Clinodactyly; Physiolysis
Locking compression plates (LCP) have been developed for paediatric patients, and these provide safe bone fixation and allow for immediate weight-bearing. These plates are becoming increasingly popular and are proving to especially useful for the treatment of proximal and distal (supracondylar) femoral osteotomies. They are thought to provide better grip in osteoporotic bone. The technique has been described in the provider’s manual as well as recently by Joeris et al. (Int Orthop 2012: 36:2299–2306). The LCP system offers optimal stability after the final fixation, but temporary fixation using the originally described method is unstable, and we consider the medialisation instrument unsatisfactory as it has very small contact area with the lateral aspect of the femur and does not provide sound rotational control. For this reason we have developed a technique whereby temporary, stable fixation using fine K-wires can be attained for the proximal, diaphyseal fragment, following sound fixation with locking screws of the distal fragment. This procedure allows for full clinical (including rotation) and radiographic assessment intraoperatively, as well as fine adjustment in all three planes. This modification enables optimal correction before the final fixation and avoids unnecessary, large drill holes which may otherwise act as dangerous tress risers, endangering stability and weight-bearing. Here we describe a refined technique of the supracondylar femoral osteotomy using the paediatric LCP Condylar Plate.
Locking compression plate; Femoral osteotomy; Supracondylar osteotomy; Technique
The purpose of this study was to evaluate the two-stage surgical technique combining induced membrane, spongy autograft and intramedullary fixation for the treatment of congenital pseudarthrosis of the tibia (CPT).
Three boys and two girls were treated by this technique between 2003 and 2008. All patients had type IV CPT in Crawford’s classification. Four of them had a limited dystrophic form, whereas one case presented an extensive tibia bone dystrophy. The average age of patients at the time of surgery was 23 months (range 10–30 months), with an average follow-up of 5.8 years (range 2.4–8.1 years).
Satisfactory tibial bony union was achieved in all cases at the last follow-up. Bone healing was obtained in the four limited forms after an average term of 4 months. One patient suffered from a non-displaced fracture that healed by casting in a usual period of time. The patient with an extensive dystrophic bone had to undergo a secondary inter-tibiofibular bone graft to finally achieve bone union.
The preliminary results show that this technique is successful in CPT. It may be used even in young children and offers a good alternative to other treatments available, avoiding external fixation and the technical difficulties of microvascular surgery.
Congenital pseudarthrosis of the tibia; Induced membrane
The goal of prosthetic fitting is to provide comfort and functionality to the patient. It is thought that incorporating the use of standing anterior–posterior long leg radiographs (LLR) into the fitting of lower extremity prostheses will provide an objective guide when making adjustments, and be a better assessment of alignment. This study compares prosthetic alignment before and after radiography-guided adjustments.
This retrospective study was performed at a multidisciplinary amputee clinic on patients with congenital and/or acquired limb deficiencies. Their prosthetic alignment was evaluated by LLR and adjusted as needed. Satisfactory alignment was defined as a mechanical axis angular deviation of ≤1° and a leg length discrepancy of ≤10 mm.
A total of 45 unique prostheses from 24 subjects (10 female and 14 male) were included. Post-adjustment radiographs were obtained from 29 prostheses. After the initial prosthetic fitting, the probability of a satisfactory fit was 20.0 % (95 % CI 10.9–34.9 %). Following the baseline adjustment, the probability of a satisfactory fit improved to 53.3 % (95 % CI 37.5–70.9 %). After adjustment number 4, the probability of a satisfactory fit further improved to 76.7 % (95 % CI 41.9–98.0 %). There were also significant improvements in distal offset distance (p = 0.0040) and leg length discrepancy (p = 0.0206). The distal offset distance decreased by an average of 10.7 mm (95 % CI 3.6–17.8), and leg length discrepancy decreased by an average of 3.0 mm (95 % CI 00.48–5.5).
The addition of LLRs to existing fitting methods significantly improves prosthetic alignment and length.
Long leg radiographs; Prosthetic alignment; Lower extremity; Amputees
Closed reduction and percutaneous fixation with Kirschner wires (KWs) is the standard of care of pediatric supra-condylar humerus fractures (SCHFs). Failure modes leading to loss of reduction are not clear and have not been quantified. Multiple factors may weaken the KW–bone interface bonding conditions. To the best of our knowledge, the possible effect of this decrease on different KW configurations and fracture stability has never been studied.
To investigate the effect of bone–KW friction conditions on SCHF post-operative mechanical stability and to formulate clinical guidelines for KW configuration under different conditions.
Finite element-based model of a fixated SCHF was used to simulate structure stability for two lateral divergent versus crossed lateral and medial KW configurations under varying KW–bone friction conditions.
Finite element simulations demonstrated that crossed KWs provide superior stability compared with the divergent configuration when KW–bone bonding is compromised. When KW–bone bonding conditions are adequate, crossed and divergent KW configurations provide similar, sufficient fracture stability.
Under normal bone–implant interface conditions, the two diverging lateral KW configuration offers satisfactory mechanical stability and may be the preferred choice of SCHF fixation. When KW–bone bonding is suboptimal, as when one or more of the lateral KWs are re-drilled, addition of a medial KW should be considered in order to improve stability despite risk to ulnar nerve.
Pediatric; Supra-condylar; Finite elements; Kirschner wires
Since the September 11, 2001 terrorist attacks on the World Trade Center in New York City, travel security has become an ever-increasing priority in the United States. Frequent parent and patient inquiry and recent literature reports have generated interest in the impact of heightened security measures on patients with orthopaedic implants, and have indicated increasing rates of metal detector triggering. There are no reports to date, however, evaluating children and adolescents who have undergone posterior spinal fusion for scoliosis, so responses to patient and parent inquiries are not data-driven. The purpose of this study is to determine the frequency of airport metal detector triggering by patients who have had posterior-only spinal fusion and to characterise any potential predictors of metal detector activation.
A cross-sectional study was performed by interviewing 90 patients who underwent posterior-only spinal fusion for a diagnosis of juvenile or adolescent idiopathic scoliosis and have travelled by air in the past year. Demographic, clinical and surgical instrumentation data were collected and evaluated, along with patients’ reports of airport metal detector triggering and subsequent screening procedures.
Five patients with stainless steel instrumentation (5.6 % of the cohort) triggered an airport walkthrough metal detector, and an additional five patients who did not trigger an airport detector triggered a handheld detector at a different venue. All patients who triggered an airport metal detector had stainless steel instrumentation implanted prior to 2008, and no patient with titanium instrumentation triggered any detector in any venue. All trigger events required subsequent screening procedures, even when an implant card was presented.
In this cohort of children and adolescents with posterior spinal instrumentation, airport walkthrough metal detector triggering was a rare event. Therefore, we advise patients and families with planned posterior scoliosis fusions using titanium instrumentation that airport detection risk is essentially non-existent, and only rare for those with planned stainless steel instrumentation. We no longer issue implant cards postoperatively, as these did not prevent further screening procedures in this cohort.
Level of evidence
Prognostic level 2. Study design: cross-sectional.
Adolescent idiopathic scoliosis; Spinal instrumentation; Hardware; Stainless steel; Titanium; Air travel; Metal detector; Security screening
Overactivity or contractures of the hamstring muscles in ambulatory children with cerebral palsy (CP) can lead to either a jump gait (knee flexion associated with ankle plantar flexion) or a crouch gait (knee flexion associated with ankle dorsiflexion). Hamstring lengthening is performed to decrease stance knee flexion. However, this procedure carries the potential risk of weakening hip extension power as well as recurrence over time; therefore, surgeons have adopted a modified procedure wherein the semitendinosus and gracilis are transferred above the knee joint, along with lengthening of the semimembranosus and biceps femoris.
The purpose of our study is to evaluate the differences between hamstring lengthening alone (HSL group) and hamstring lengthening plus transfer (HST group) in the treatment of flexed knee gait in ambulatory children with CP. We hypothesized that recurrence of increased knee flexion in the stance phase will be less in the HST group at long-term follow-up, and hip extensor power will be better preserved.
Fifty children with CP who underwent hamstring surgery for flexed knee gait were retrospectively reviewed. All subjects underwent a pre-operative gait study, a follow-up post-operative gait study, and a long-term gait study. The subjects were divided into two groups; HSL group (18 subjects) or HST group (32 subjects). The mean age at surgery was 9.9 ± 3.3 years. The mean follow-up time was 4.4 ± 0.9 (2.7–6.3) years.
On physical examination, both groups showed improvement in straight leg raise, knee extension, popliteal angle, and maximum knee extension in stance at the first post-op study, and maintained this improvement at the long-term follow-up, with the exception of straight leg raise, which slightly worsened in both groups at the final follow-up. Both groups improved maximum knee extension in stance at the initial follow-up, and maintained this at the long-term follow-up. Only the HST group showed significant (p < 0.05) improvement in the peak hip extension power in stance at the first post-op study, and this increased further at the final follow-up. In the HSL group, there was an initial slight decrease in the hip extension power, which subsequently increased to pre-operative values at the long-term study. Only the HST group showed increase of the average anterior pelvic tilt at the long-term follow-up study, although this was small in magnitude. There were two subjects who developed knee recurvatum at the post-op study, and both were in the HST group.
There is no clear benefit in regards to recurrence when comparing HST to HSL in the long term. In both HSL and HST, there was reduction of stance phase knee flexion in the long term, with no clear advantage in either group. Longer follow-up is needed for additional recurrence information. There was greater improvement of hip extension power in the HST group, which may justify the additional operative time of the transfer.
This study helps pediatric orthopedic surgeons choose between two different techniques to treat flexed knee gait in patients with CP by showing the long-term outcome of both procedures.
Cerebral palsy; Hamstring lengthening; Hamstring transfers; Motion analysis; Long-term study
The aim of this study was to describe a subtalar extra-articular screw arthroereisis (SESA) technique for the correction of flexible flatfoot (FFF) in children and report the outcome.
From 1990 to 2012, data were collected on 485 patients who underwent SESA at the San Raffaele Hospital. The average age of the patient cohort was 11.5 ± 1.81 years (range 5.0–17.9 years; median 11.5 years). Inclusion criteria were FFF and marked flexible hindfoot valgus, and the exclusion criterion was rigid flatfoot. SESA was performed in 732 cases of FFF—bilaterally in 247 patients and monolaterally in 238 patients.
The values of the pre- and post-SESA weight-bearing X-ray angles were 146° ± 7° and 129° ± 5°, respectively, for the Costa-Bartani angle, 43° ± 8° and 25° ± 6°, respectively, for the talar inclination angle and 11° ± 6° and 14° ± 5°, respectively, for calcaneal pitch (p <0.001). All data were analysed statistically with Student’s t test. Data on 398 patients were ultimately available for analysis. In 93.7 % of cases the results were good in terms of improved clinical aspects and X-ray measurement, absence of complications, normal foot function 3 months post-SESA and no requirement for further surgery. The complication rate was 6.3 % and included ankle joint effusion, painful contracture of peroneal muscles and fourth metatarsal bone stress fractures. A sample of 76 patients (121 feet) were evaluated after screw removal, which occurred on average 2.9 years after SESA. The angle measurements of this sample showed no statistically significant modification.
Based on our >20 years of experience, we believe that SESA is an optimal technique for the correction of FFF as it is simple and can be performed rapidly, and the corrective effect results from the screw’s mechanical and proprioceptive effect. The indication for surgery must be accurate. We suggest that the patient be at least 10 years of age in order that all of the foot’s growth potential can be utilized and to allow for spontaneous resolution and thereby avoid the possibility of over-treatment.
Flexible flatfoot; Arthroereisis; Calcaneo-stop; Minimally invasive surgery
We assessed the radiographic changes of the acetabulum during the course of Perthes’ disease and investigated whether they were associated with femoral head sphericity 5 years after diagnosis.
We studied 123 children with unilateral Perthes’ disease, femoral head necrosis more than 50 % and age at diagnosis 6 years or older. Pelvic radiographs were taken at onset, 1 year and 5 years after diagnosis. Sharp’s angle, acetabular depth-to-width ratio (ADR) and lateral acetabular inclination were measured.
Compared to the unaffected hips, the Perthes’ hips developed significantly higher Sharp’s angles (p < 0.001) and a higher proportion with an upward-sloping lateral acetabular margin (Perthes’ hips: 49 %, unaffected hips 1 %). The mean ADR values were significantly lower on the affected side at all stages (p < 0.001). ADR values at diagnosis were associated with a more spherical femoral head at the 5-year follow-up [odds ratio (OR) 1.012, 95 % confidence interval (CI) 1.002–1.022, p = 0.016]. None of the other acetabular parameters were significantly associated with the femoral head shape 5 years after diagnosis.
The acetabulum developed an increasingly dysplastic shape in the course of Perthes’ disease. Early dysplastic changes of the acetabulum were not associated with a poor radiological outcome 5 years after diagnosis. Routine measurement and monitoring of acetabular changes in plain radiographs were of little prognostic value and can, therefore, hardly be recommended in clinical practice.
Legg–Calvé–Perthes’ disease; Acetabulum; Hip; Secondary hip dysplasia
The management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.
This is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.
Group 1: shepherd crook deformity—included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.
Shepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.
Fibrous dysplasia; Shepherd crook deformity; McCune-Albright syndrome
The effect of paediatric flexible flatfeet (PFF) on health-related quality of life (HRQOL) has not been investigated. In this prospective cross-sectional study, the HRQOL of children with PFF was compared to those with typically developing feet (TDF) using two validated measures. We hypothesised that reduced HRQOL would be observed in children with PFF. The reliability of parents’ perceptions of their child’s symptoms was also investigated.
48 children with PFF and 47 with TDF between the ages of 8 and 15 completed The Oxford Ankle Foot Questionnaire for Children (OxAFQ-C) and Pediatric Quality of Life Inventory (PedsQL™ 4.0). Proxy questionnaires were also completed. Reliability of parent and child questionnaire scores was assessed using the intraclass correlation coefficient (ICC) and Student’s t test. Differences between HRQOL between PFF and TDF were assessed using the Student’s t test.
ICCs overall demonstrated good reliability between parent and child questionnaire domain scores. There was a tendency for parents to overestimate the impairment of the child in the PFF group. PFF children demonstrated clinically significant decreased HRQOL than TDF children. This was most marked in the physical domain scores.
Although parents may overestimate their child’s impairment, children with PFF still have significantly impaired HRQOL when compared to TDF children. The impairment can be as severe, or worse, than published HRQOL for acutely and chronically unwell children. As such, PFF cannot be regarded as just a benign normal variant. The management of PFF should involve consideration of the symptom profile and HRQOL.
Level of evidence:
Electronic supplementary material
The online version of this article (doi:10.1007/s11832-014-0621-0) contains supplementary material, which is available to authorized users.
Pes planovalgus; Flatfeet; Outcome measures; Health-related quality of life