Anatomical Characteristics of Cervicomedullary Compression on MRI Scans in Children with Achondroplasia.

This retrospective study assessed anatomical characteristics of cervicomedullary compression in children with achondroplasia. Twelve anatomical parameters were analyzed (foramen magnum diameter and area; myelon area; clivus length; tentorium and occipital angles; brainstem volume outside the posterior fossa; and posterior fossa, cerebellum, supratentorial ventricular system, intracranial cerebrospinal fluid, and fourth ventricle volumes) from sagittal and transversal T1- and T2-weighted magnetic resonance imaging (MRI) scans from 37 children with achondroplasia aged ≤ 4 years (median [range] 0.8 [0.

European Achondroplasia Forum Practical Considerations for Following Adults with Achondroplasia.

Achondroplasia is a lifelong condition requiring lifelong management. There is consensus that infants and children with achondroplasia should be managed by a multidisciplinary team experienced in the condition. However, many people are lost to follow-up after the transition from paediatric to adult care, and there is no standardised approach for management in adults, despite the recent availability of international consensus guidelines.

European Achondroplasia Forum guiding principles for the detection and management of foramen magnum stenosis.

Foramen magnum stenosis is a serious, and potentially life-threatening complication of achondroplasia. The foramen magnum is smaller in infants with achondroplasia, compared with the general population, and both restricted growth in the first 2 years and premature closure of skull plate synchondroses can contribute to narrowing. Narrowing of the foramen magnum can lead to compression of the brainstem and spinal cord, and result in sleep apnoea and sudden death.

Spinal cord compression in patients with mucopolysaccharidosis.

Purpose: Spinal abnormalities frequently occur in patients with mucopolysaccharidosis (MPS) types I, II, IV, and VI. The symptoms are manifold, which sometimes prolongs the diagnostic process and delays therapy. Spinal stenosis (SS) with spinal cord compression due to bone malformations and an accumulation of storage material in soft tissue are serious complications of MPS disease.

Detecting Entanglement Structure in Continuous Many-Body Quantum Systems.

A prerequisite for the comprehensive understanding of many-body quantum systems is a characterization in terms of their entanglement structure. The experimental detection of entanglement in spatially extended many-body systems describable by quantum fields still presents a major challenge. We develop a general scheme for certifying entanglement and demonstrate it by revealing entanglement between distinct subsystems of a spinor Bose-Einstein condensate.

Programmable interactions and emergent geometry in an array of atom clouds.

Interactions govern the flow of information and the formation of correlations between constituents of many-body quantum systems, dictating phases of matter found in nature and forms of entanglement generated in the laboratory. Typical interactions decay with distance and thus produce a network of connectivity governed by geometry-such as the crystalline structure of a material or the trapping sites of atoms in a quantum simulator. However, many envisioned applications in quantum simulation and computation require more complex coupling graphs including non-local interactions, which feature in models of information scrambling in black holes and mappings of hard optimization problems onto frustrated classical magnets.

Postnatal Repair of Open Neural Tube Defects: A Single Center with 90-Month Interdisciplinary Follow-Up.

After publication of the Management of Myelomeningocele Study (MOMS) there is confusion regarding which treatment of open neural tube defects (NTD) is best. We report our results of postnatally repaired open NTDs born between 2007-2018 ( = 36) in critical reflection of the MOMS study. Neurosurgical, orthopedic, and urologic data were assessed.

Vertebral column growth in children with early onset scoliosis treated with magnetically controlled growing rods - Effects of distraction on vertebral and disc morphology.

Objectives: To report changes in vertebral and disc morphology following treatment of early onset scoliosis (EOS) with magnetically controlled growing rods (MCGR).

Methods: 30 Patients, 21 girls and 9 boys, treated with MCGR for EOS were compared to a matched control group of 19 patients (12 girls, 7 boys) treated with bracing or observation. Age at surgery in the MCGR group was 8.

Collisions of Three-Component Vector Solitons in Bose-Einstein Condensates.

Ultracold gases provide an unprecedented level of control for the investigation of soliton dynamics and collisions. We present a scheme for deterministically preparing pairs of three-component solitons in a Bose-Einstein condensate. Our method is based on local spin rotations which simultaneously imprint suitable phase and density distributions.

Management of spinal deformities and tibial pseudarthrosis in children with neurofibromatosis type 1 (NF-1).

Unlabelled: The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia. Early recognition of radiologic osseous dystrophy signs is of utmost importance because worsening of the deformities without treatment is commonly observed and surgical intervention is often necessary.

Vertical Expandable Rib-based Distraction Device for Correction of Congenital Scoliosis in Children of 3 Years of Age or Younger: A Preliminary Report.

Background: This study was designed to evaluate the treatment outcome of very young children with congenital scoliosis aged 3 years or under after surgery with a vertical expandable prosthetic titanium rib (VEPTR)-based distraction device.

Methods: A retrospective study of 13 children undergoing implantation of a vertical expandable rib-based distraction device. From September 2007 to June 2018, 13 children (7 male and 6 female patients) with congenital scoliosis were followed after treatment with a VEPTR.

Posterior fusion of the craniocervical junction in the pediatric spine: Wright's translaminar C2 screw technique provides for more safety and effectiveness.

Purpose: Posterior fusion of the craniocervical junction (CCJ) has always been challenging in children with rare congenital diseases and malformations. At our institution, the introduction of the translaminar C2 screw technique led to a significant improvement in the quality of treatment.

Methods: Retrospective analysis of a pediatric cohort at a single institution who underwent CCJ posterior fusion between 2007 and 2018.

Pedicle screw instrumentation with or without pelvic fixation in neuromuscular scoliosis: Outcome and complications in a series of 37 patients with a minimum 2-year follow-up.

Objective: Neuromuscular scoliosis (NMS) is often associated with rapid progressive spinal deformities. Indications, when to extend the instrumentation to the pelvis for pelvic obliquity are not generally accepted at this time. This study reports on the indications, surgical technique and results using pedicle screw instrumentation exclusively with or without pelvic fixation for spine fusion in patients with NMS.

Simultaneous Readout of Noncommuting Collective Spin Observables beyond the Standard Quantum Limit.

We augment the information extractable from a single absorption image of a spinor Bose-Einstein condensate by coupling to initially empty auxiliary hyperfine states. Performing unitary transformations in both the original and auxiliary hyperfine manifold enables the simultaneous measurement of multiple spin-1 observables. We apply this scheme to an elongated atomic cloud of ^{87}Rb to simultaneously read out three orthogonal spin directions and with that directly access the spatial spin structure.

Intrathecal baclofen therapy for treatment of spasticity in infants and small children under 6 years of age.

Purpose: The aim of this study is to prove the efficacy and safety of intrathecal baclofen therapy in infants and children below 6 years of age by retrospective analysis of our pediatric cohort of 135 primary pump implantations.

Methods: Between 2007 and 2018, 17 patients with pump implantations were below 6 years of age. Data were acquired retrospectively with a follow-up of 12 months to 11 years regarding complications.

Nerve transfer from the median to musculocutaneous nerve to induce active elbow flexion in selected cases of arthrogryposis multiplex congenita.

Background: Arthrogryposis multiplex congenita (AMC) is a rare disease which affects mainly upper and lower extremities. Affected patients are not able to eat unassisted due to elbow contracture and nonexistent active elbow flexion. In traumatic brachial plexus palsies, a nerve transfer from either median or ulnar nerve to the musculocutaneous nerve has proved to induce active elbow flexion, and we report our results of such a procedure in a nontraumatic condition, that is, arthrogryposis.

Observation of universal dynamics in a spinor Bose gas far from equilibrium.

Predicting the dynamics of quantum systems far from equilibrium represents one of the most challenging problems in theoretical many-body physics. While the evolution of a many-body system is in general intractable in all its details, relevant observables can become insensitive to microscopic system parameters and initial conditions. This is the basis of the phenomenon of universality.

Treatment of pediatric spinal deformity with use of recombinant human bone morphogenetic protein-2.

In pediatric spine surgery nonunion is a challenging issue. Instability may cause neurological impairment and lead to numerous surgeries in order to achieve fusion. The use of rhBMP-2 for pediatric spinal fusion has not been widely reported.

Strategies for Treating Scoliosis in Early Childhood.

Background: Scoliosis in early childhood is defined as abnormal curvature of the spine of any etiology that arises before age 10. The affected children are at high risk of developing restrictive pulmonary dysfunction. The treatment presents major challenges because of the complexity and high morbidity of the disease.

Spatially distributed multipartite entanglement enables EPR steering of atomic clouds.

A key resource for distributed quantum-enhanced protocols is entanglement between spatially separated modes. However, the robust generation and detection of entanglement between spatially separated regions of an ultracold atomic system remain a challenge. We used spin mixing in a tightly confined Bose-Einstein condensate to generate an entangled state of indistinguishable particles in a single spatial mode.

Posterior Only Vertebral Column Resection for the Treatment of Severe Spinal Deformities in Pediatric Patients: A Retrospective Case Series.

Objective: The treatment of severe spinal deformities in pediatric patients is very challenging. Posterior only vertebral column resection (PVCR) allows for correcting of severe deformities of the vertebral column via a posterior only procedure. We analyzed radiologic outcome of PVCR performed on a series of pediatric patients with severe congenital and acquired spinal deformities.

Preliminary Results of Magnetically Controlled Growing Rods for Early Onset Scoliosis.

Background: Growth-sparing techniques for the treatment of early onset scoliosis (EOS) have developed significantly over the last years. Traditional growing rods (GRs) require repeated surgical lengthening under anesthesia. Since June 2011 we have been using the magnetically controlled growing rods (MCGR) to treat patients with progressive EOS.

Prenatal latex sensitization in patients with spina bifida: a pilot study.

Object: Patients with spina bifida are particularly vulnerable to developing immunoglobulin E (IgE)-mediated latex sensitization. Even though many risk factors leading to latex allergy in these patients have been described, it is still unclear whether the increased prevalence of latex sensitization is disease associated or due to the procedures used to treat spina bifida. The aim of this study was to assess prenatal latex sensitization in patients with spina bifida by examining IgE levels in umbilical cord blood.