An exploratory open-label multicentre phase I/II trial evaluating the safety and efficacy of postnatal or prenatal and postnatal administration of allogeneic expanded fetal mesenchymal stem cells for the treatment of severe osteogenesis imperfecta in infants and fetuses: the BOOSTB4 trial protocol.

Introduction: Severe osteogenesis imperfecta (OI) is a debilitating disease with no cure or sufficiently effective treatment. Mesenchymal stem cells (MSCs) have good safety profile, show promising effects and can form bone. The Boost Brittle Bones Before Birth (BOOSTB4) trial evaluates administration of allogeneic expanded human first trimester fetal liver MSCs (BOOST cells) for OI type 3 or severe type 4.

Magnetic resonance imaging-based bone imaging of the lower limb: Strategies for generating high-resolution synthetic computed tomography.

This study aims at assessing approaches for generating high-resolution magnetic resonance imaging- (MRI-) based synthetic computed tomography (sCT) images suitable for orthopedic care using a deep learning model trained on low-resolution computed tomography (CT) data. To that end, paired MRI and CT data of three anatomical regions were used: high-resolution knee and ankle data, and low-resolution hip data. Four experiments were conducted to investigate the impact of low-resolution training CT data on sCT generation and to find ways to train models on low-resolution data while providing high-resolution sCT images.

Life span care for patients with skeletal dysplasia: A roadmap.

Patients with skeletal dysplasias usually experience health related problems in different parts and systems of the body. Therefore, they face challenges in multiple domains of functioning and health. To address these different domains, interdisciplinary care should be the standard for these patients.

Impact of bone and cartilage segmentation from CT and MRI on both bone forearm osteotomy planning.

Introduction: The use of MRI scans for pre-operative surgical planning of forearm osteotomies provides additional information of joint cartilage and soft tissue structures and reduces radiation exposure in comparison with the use of CT scans. In this study, we investigated whether using 3D information obtained from MRI with and without cartilage information leads to a different outcome of pre-operative planning.

Methods: Bilateral CT and MRI scans of the forearms of 10 adolescent and young adult patients with a unilateral bone deformation were acquired in a prospective study.

Acetabular rim extension using a personalized titanium implant for treatment of hip dysplasia in dogs: short-term results.

Hip dysplasia (HD) is a common orthopedic problem in young dogs. To decrease the laxity of the hip joint related to HD, the surgical treatments are recommended to increase femoral head coverage. ACEtabular rim eXtension (ACE-X) using a personalized 3-dimensional printed titanium shelf implant is a new surgical treatment to increase femoral head coverage and decrease laxity of the dysplastic hip joint, however, the efficacy is less know.

Active monitoring versus an abduction device for treatment of infants with centered dysplastic hips: study protocol for a randomized controlled trial (TReatment with Active Monitoring (TRAM)-Trial).

Background: Developmental Dysplasia of the Hip (DDH) is one of the most common pediatric orthopedic disorders, affecting 1-3% of all newborns. The optimal treatment of centered DDH is currently under debate. This randomized controlled trial aims to study the (cost-)effectiveness of active monitoring versus abduction treatment for infants with centered DDH.

Automatic Assessment of Lower-Limb Alignment from Computed Tomography.

Background: Preoperative planning of lower-limb realignment surgical procedures necessitates the quantification of alignment parameters by using landmarks placed on medical scans. Conventionally, alignment measurements are performed on 2-dimensional (2D) standing radiographs. To enable fast and accurate 3-dimensional (3D) planning of orthopaedic surgery, automatic calculation of the lower-limb alignment from 3D bone models is required.

From Genetics to Clinical Implications: A Study of 675 Dutch Osteogenesis Imperfecta Patients.

Osteogenesis imperfecta (OI) is a heritable connective tissue disorder that causes bone fragility due to pathogenic variants in genes responsible for the synthesis of type I collagen. Efforts to classify the high clinical variability in OI led to the Sillence classification. However, this classification only partially takes into account extraskeletal manifestations and the high genetic variability.

Osteoporosis in children and adolescents: how to treat and monitor?

Unlabelled: Osteoporosis is a condition of increased bone fragility associated with fractures. Apart from primary genetic osteoporotic conditions, secondary osteoporosis in children is being increasingly recognized. As a result, there is growing interest in its prevention and treatment.

Visual Inspection for Lower Limb Malalignment Diagnosis Is Unreliable.

Objective: Visual inspection of the lower limb is often part of standard clinical practice during a physical examination at the outpatient clinic. This study aims to investigate how reliable visual inspections are in terms of detecting lower limb malalignments without additional tools and physical examinations.

Design: This study enrolled 50 patients.

Efficient cascaded V-net optimization for lower extremity CT segmentation validated using bone morphology assessment.

Semantic segmentation of bone from lower extremity computerized tomography (CT) scans can improve and accelerate the visualization, diagnosis, and surgical planning in orthopaedics. However, the large field of view of these scans makes automatic segmentation using deep learning based methods challenging, slow and graphical processing unit (GPU) memory intensive. We investigated methods to more efficiently represent anatomical context for accurate and fast segmentation and compared these with state-of-the-art methodology.

Fractures in Osteogenesis Imperfecta: Pathogenesis, Treatment, Rehabilitation and Prevention.

Fractures in patients with osteogenesis imperfecta (OI) are caused by a decreased strength of bone due to a decreased quality and quantity of bone matrix and architecture. Mutations in the collagen type 1 encoding genes cause the altered formation of collagen type I, one of the principal building blocks of bone tissue. Due to the complexity of the disease and the high variation of the clinical problems between patients, treatment for these patients should be individually tailored.

Patient-specific 3D-printed shelf implant for the treatment of hip dysplasia tested in an experimental animal pilot in canines.

The concept of a novel patient-specific 3D-printed shelf implant should be evaluated in a relevant large animal model with hip dysplasia. Therefore, three dogs with radiographic bilateral hip dysplasia and a positive subluxation test underwent unilateral acetabular augmentation with a 3D-printed dog-specific titanium implant. The contralateral side served as control.

Good long-term outcomes of the hip Chiari osteotomy in adolescents and adults with hip dysplasia: a systematic review.

Background And Purpose: The Chiari osteotomy was a regular treatment for developmental hip dysplasia before it became mostly reserved as a salvage therapy. However, the long-term survival of the Chiari osteotomy has not been systematically investigated. We investigated the survival time of the Chiari osteotomy until conversion to total hip arthroplasty (THA) in patients with primary hip dysplasia, and factors which correlated with survival, complications, and the improvement measured in radiographic parameters.

Statistical Shape Modeling of US Images to Predict Hip Dysplasia Development in Infants.

Background The current widely applied Graf classification used on US images for developmental dysplasia of the hip in infants does not enable prediction of the development and outcome of well-centered stable dysplastic hips (Graf type II). Purpose To use statistical shape modeling on US images to identify acetabular shape characteristics of Graf type II hips, which enable prediction of the development of Graf type II hips, and to identify which hips benefit from Pavlik harness treatment. Materials and Methods In this secondary analysis of a prospective multicenter randomized trial on treatment of 104 infants aged 3-4 months with Graf type IIb or IIc hip dysplasia conducted between 2009 and 2015, a statistical shape model was developed on baseline US images.

Comparing Hip Dysplasia in Dogs and Humans: A Review.

Hip dysplasia (HD) is common in both humans and dogs. This interconnection is because humans and dogs descended from a common ancestor and therefore have a similar anatomy at micro- and macroscopic levels. Furthermore, dogs are the animals of choice for testing new treatments for human hip dysplasia and orthopedic surgery in general.

Multiscale characterization of pathological bone tissue.

Bone is a complex natural material with a complex hierarchical multiscale organization, crucial to perform its functions. Ultrastructural analysis of bone is crucial for our understanding of cell to cell communication, the healthy or pathological composition of bone tissue, and its three-dimensional (3D) organization. A variety of techniques has been used to analyze bone tissue.

CT to MR registration of complex deformations in the knee joint through dual quaternion interpolation of rigid transforms.

To develop a method that enables computed tomography (CT) to magnetic resonance (MR) image registration of complex deformations typically encountered in rotating joints such as the knee joint.We propose a workflow, denoted quaternion interpolated registration (QIR), consisting of three steps, which makes use of prior knowledge of tissue properties to initialise deformable registration. In the first step, the rigid skeletal components were individually registered.

MRI-based synthetic CT shows equivalence to conventional CT for the morphological assessment of the hip joint.

This study evaluated the accuracy of synthetic computed tomography (sCT), as compared to CT, for the 3D assessment of the hip morphology. Thirty male patients with asymptomatic hips, referred for magnetic resonance (MR) imaging and CT, were included in this retrospective study. sCT images were generated from three-dimensional radiofrequency-spoiled T1-weighted multi-echo gradient-echo MR images using a commercially available deep learning-enabled software and were compared with CT images through mean error and surface distance computation and by means of eight clinical morphometric parameters relevant for hip care.

Patient-specific 3D-printed shelf implant for the treatment of hip dysplasia: Anatomical and biomechanical outcomes in a canine model.

A solution for challenging hip dysplasia surgery could be a patient-specific 3D-printed shelf implant that is positioned extra-articular and restores the dysplastic acetabular rim to normal anatomical dimensions. The anatomical correction and biomechanical stability of this concept were tested in a canine model that, like humans, also suffers from hip dysplasia. Using 3D reconstructed computed tomography images the 3D shelf implant was designed to restore the radiological dysplastic hip parameters to healthy parameters.

A roadmap to surgery in osteogenesis imperfecta: results of an international collaboration of patient organizations and interdisciplinary care teams.

Background and purpose - Involvement of patient organizations is steadily increasing in guidelines for treatment of various diseases and conditions for better care from the patient's viewpoint and better comparability of outcomes. For this reason, the Osteogenesis Imperfecta Federation Europe and the Care4BrittleBones Foundation convened an interdisciplinary task force of 3 members from patient organizations and 12 healthcare professionals from recognized centers for interdisciplinary care for children and adults with osteogenesis imperfecta (OI) to develop guidelines for a basic roadmap to surgery in OI.Methods - All information from 9 telephone conferences, expert consultations, and face-to-face meetings during the International Conference for Quality of Life for Osteogenesis Imperfecta 2019 was used by the task force to define themes and associated recommendations.