What's new in pediatric lower limb reconstruction?

The last years brought many advances relevant to lower limb reconstruction. It feels like guided growth has been looked at from every angle, and still there are new emerging concepts like rotational guided growth waiting to be validated. New hexapod external devices are more accurate and easier to use, and new unilateral fixators allow for more versatile and stable fixation and lengthening.

[External fixation for the correction of complex deformities : From the classic Ilizarov ring fixator to modern software-driven hexapod fixators].

The history and development of circular fixation is closely aligned with the history of limb lengthening. Gavriil A. Ilizarov not only developed his circular fixator, but he also was the first to research and publish on distraction osteogenesis.

Persistent Lower Limb Deformities Despite Amelioration of Rickets in X-Linked Hypophosphatemia (XLH) - A Prospective Observational Study.

Background: Gait deviations, lower limb pain and joint stiffness represent key symptoms in patients with X-linked hypophosphatemia (XLH, OMIM 307800), a rare disorder of mineral homeostasis. While the pathomechanism for rickets is well understood, the direct role of PHEX (Phosphate-regulating neutral endopeptidase) deficiency in non-rachitic features including complex deformities, skull and dental affections remains unclear. FGF23-inhibiting antibody treatment can normalize serum phosphate levels and to improve rickets in XLH patients.

Limb Lengthening With Precice Intramedullary Lengthening Nails in Children and Adolescents.

Background: The Precice intramedullary bone lengthening nail has been used in our department since 2013. We sought to determine the efficacy and safety of intramedullary limb lengthening with Precice nails in children and adolescents.

Methods: We retrospectively investigated patients 18 years and younger who underwent lower-limb lengthening using the Precice nail.

The Treatment of Recurrent Congenital Clubfoot.

The Ponseti method for treatment of congenital clubfoot is well established and has been introduced in most pediatric orthopedic centers worldwide. However, reported rates of recurrence are largely variable and open joint surgery is still performed frequently, even in the age group younger than 6 years of age. Preventing recurrence and residual deformity can be achieved by strict adherence to the Ponseti method, ensuring and enforcing brace compliance, frequent follow-up, and early treatment of recurrence.

Lower Limb Deformity and Gait Deviations Among Adolescents and Adults With X-Linked Hypophosphatemia.

Background: X-linked hypophosphatemia (XLH) is a rare genetic disorder characterized by lower limb deformity, gait and joint problems, and pain. Hence, quality of life is substantially impaired. This study aimed to assess lower limb deformity, specific radiographic changes, and gait deviations among adolescents and adults with XLH.

Optimized expression and purification of adipose triglyceride lipase improved hydrolytic and transacylation activities in vitro.

Adipose triglyceride lipase (ATGL) plays a key role in intracellular lipolysis, the mobilization of stored triacylglycerol. This work provides an important basis for generating reproducible and detailed data on the hydrolytic and transacylation activities of ATGL. We generated full-length and C-terminally truncated ATGL variants fused with various affinity tags and analyzed their expression in different hosts, namely E.

Normalization of Forefoot Supination After Tibialis Anterior Tendon Transfer for Dynamic Clubfoot Recurrence.

Background: We aimed to evaluate the effect of tibialis anterior tendon transfer (TATT) on foot motion in children with clubfoot recurrence after initial Ponseti treatment.

Methods: Children with dynamic clubfoot recurrence after initial Ponseti treatment who underwent TATT between 2014 and 2017 were considered for inclusion. Exclusion criteria were neurological disease, split transfer of the tendon, additional bone or joint invasive surgery, and initial treatment abroad.

Long-term results and comparison of the Green-Anderson and multiplier growth prediction methods after permanent epiphysiodesis using Canale's technique.

Purpose: The aim of the study was to evaluate the accuracy and radiographic outcomes of Canale's method in patients with idiopathic leg-length discrepancy (LLD) following percutaneous epiphysiodesis. The accuracy of two common growth prediction methods was assessed.

Methods: A total of 18 patients with 26 affected bones (eight distal femur, two proximal tibia, five combined) were clinically and radiologically analyzed after reaching skeletal maturity.

[Osteotomies for deformity correction on the pediatric knee].

Background: Fractures in childhood can result in malalignment and/or leg shortening due to insufficient reduction, malunion or injury to the growth plate.

Objective: Different types of posttraumatic deformities around the knee as well as the diagnostic approach and correction are discussed.

Material And Methods: While deformities without shortening can often be corrected during surgery with internal fixation, limb shortening with or without malalignment has to be treated with external fixation or internal lengthening nails.

Genes Activated by upon Exposure to Reveal the Mannose-Sensitive Hemagglutinin To Be Essential for Colonization.

During its life cycle, the facultative human pathogen , which is the causative agent of the diarrheal disease cholera, needs to adapt to a variety of different conditions, such as the human host or the aquatic environment. Importantly, cholera infections originate from the aquatic reservoir where persists between the outbreaks. In the aquatic environment, bacteria are constantly threatened by predatory protozoa and nematodes, but our knowledge of the response pathways and adaptation strategies of to such stressors is limited.

Hypoxia-inducible lipid droplet-associated protein inhibits adipose triglyceride lipase.

Elaborate control mechanisms of intracellular triacylglycerol (TAG) breakdown are critically involved in the maintenance of energy homeostasis. Hypoxia-inducible lipid droplet-associated protein (HILPDA)/hypoxia-inducible gene-2 (Hig-2) has been shown to affect intracellular TAG levels, yet, the underlying molecular mechanisms are unclear. Here, we show that HILPDA inhibits adipose triglyceride lipase (ATGL), the enzyme catalyzing the first step of intracellular TAG hydrolysis.

The unstable knee in congenital limb deficiency.

Purpose: Instability of the knee is a common finding in patients with congenital limb deficiency. The instability can be attributed to soft tissue abnormalities, frontal, sagittal or rotational deformity of the lower limb and bony dysplasia of the patella or of the femoral condyles. In most of the cases, these pathomorphologic changes stay asymptomatic in daily activity.

[Pediatric clubfoot : Treatment of recurrence].

Over the last 10 years the Ponseti method has become established as the gold standard for initial treatment of clubfeet nearly worldwide. Nevertheless, there are considerable fluctuations regarding the authenticity and quality in the application of the Ponseti method. Especially the efforts to ensure and promote compliance with the foot abduction brace and subsequently the recurrence rate show great variation.

Rebound of Frontal Plane Malalignment After Tension Band Plating.

Background: Despite the popularity of tension band plating (TBP) current literature lacks clinical data concerning recurrence ("rebound") of frontal plane malalignment. This study investigated the rebound phenomenon after TBP in idiopathic genu varum/valgum deformities. We analyzed factors that may contribute to the development of rebound after removal of TBP.

Treatment of Severe Recurrent Clubfoot.

Understanding the pathoanatomy of severe recurrent clubfoot and its implication on treatment options is important for the successful treatment. A comprehensive clinical evaluation of the different components helps in selecting procedures. Individual needs and social and psychological factors influencing treatment and the impact of treatment on the child have to be considered.

Results of gait analysis including the Oxford foot model in children with clubfoot treated with the Ponseti method.

Background: The aim of the study was to evaluate how clubfeet treated with the Ponseti method compare with control feet in gait analysis and whether additional information can be provided by the Oxford foot model.

Methods: All patients with a minimum age of three years in our prospective database of clubfeet treated with the Ponseti method were considered for inclusion. Exclusion criteria were an associated syndrome or neurological disease, positional (slight) clubfoot, and presentation at an age of more than three months.

Prenatal diagnosis of congenital femoral deficiency and fibular hemimelia.

Objectives: Routine ultrasonography can detect congenital femoral deficiency (CFD) and fibular hemimelia (FH), but prenatal detection rate and its relation to deformity severity have never been reported. Whether mothers prefer prenatal diagnosis is also unknown. We aimed to determine whether mothers prefer prenatal diagnosis, to report detection rates for CFD and/or FH, and to correlate detection rates to severity of limb shortening.

Long-term results of a nationwide general ultrasound screening system for developmental disorders of the hip: the Austrian hip screening program.

Background: Diagnosis and early treatment of developmental dysplasia of the hip (DDH) continue to be issues of discussion. In 1992, a nationwide general ultrasound screening program using Graf technique was introduced to detect DDH in Austria. We investigated the effects of this program on the rates of operative and conservative interventions and the influence of the program on the number of hospital admissions for the treatment of DDH.

Broad spectrum of skeletal malformation complex in patients with cleidocranial dysplasia syndrome: radiographic and tomographic study.

Purpose: Cleidocranial dysplasia is an autosomal dominant disorder characterized by defective ossification of the intramembraneous ossification (primarily the clavicles, cranium, and pelvis), and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. Spine deformities were of progressive nature and considered to be the major orthopedic abnormalities encountered in our practice in patients with cleidocranial dysplasia. We aimed to further delineate the underlying spine pathology and its etiological understanding.

The Ponseti method for the treatment of congenital club foot: review of the current literature and treatment recommendations.

The Ponseti method has become the gold standard of care for the treatment of congenital club foot. Despite numerous articles in MEDLINE reporting results from around the globe there are still crucial details of the Ponseti method which seem to be less commonly known or considered. The Ponseti method is not only a detailed method of manipulation and casting but also of preventing and treating relapse.