Osteonecrosis in paediatric acute lymphoblastic leukaemia: Incidence, risk factors, radiological patterns and evolution in a single-centre cohort.

Osteonecrosis (ON) is a well-known sequela of paediatric acute lymphoblastic leukaemia (ALL) treatment. Incidence differs substantially among studies and the clinical significance of radiological findings is not fully established. We analysed 256 consecutive patients with ALL treated in our Institution between October 2010 and December 2016. Within the cohort, 41 developed ON, with a mean 5-year cumulative incidence of 18.5 (standard error, SE, 5.7)% overall. The mean (SE) 5-year cumulative incidence of ON was 12.7 (2.1)% after censoring upon stem cell transplantation (SCT) and/or relapse. Patients aged ≥10 years and patients allocated to the high-risk stratum had a 10-fold and fivefold higher risk of ON respectively. The risk of ON was more than double in relapsed patients, whereas no significant impact of gender, immunophenotype and SCT was demonstrated. Multiple lesions (median four joints involved per patient) were detected by magnetic resonance imaging in all but one patient, with the knee being the most affected joint. Lesions affecting convex joint surfaces experienced the worst evolution, whereas most lesions on diaphyses and concave surfaces remained radiologically stable or disappeared during follow-up. ON has a high prevalence in paediatric ALL, presenting with multiple lesions. Lesions involving convex surfaces were at higher risk of radiological deterioration.

Osteopathic Treatment and Evaluation in the Clinical Setting of Childhood Hematological Malignancies.

Children: adolescents, and young who are adults affected with hematological malignancies (CAYA-H) and who are undergoing intensive phases of cancer treatment, including hematopoietic stem cell transplantation (HSCT), experience diminished functional ability. This study was aimed at assessing the feasibility, efficacy, safety, and satisfaction of an osteopathic intervention in CAYA-H attending an 11-week precision-based exercise program (PEx). All of the participants were given 4-10 treatments according to the prescription ordered by the sports medicine doctor in charge of the PEx, and the following outcomes were assessed: (1) spinal column range of motion (ROM) by palpation; (2) lower and upper limb joints ROM by a goniometer; (3) orthostatic posture by plumb line assessment; (4) chest and abdomen mobility by inspection and palpation; (5) cranial-sacral rhythmic impulse (CRI) by palpation; and (6) adverse effects. Goal attainment scaling (GAS) was used to identify the accomplishment of a desired clinical result. Moreover, HSCT patients who were affected with graft-versus-host disease and/or osteonecrosis had their joints assessed in terms of ROM as tools to monitor the effectiveness of immunosuppressive treatment. A total of 231 CAYA-H were identified, and 104 participated in the study (age 10.66 ± 4.51 yrs; 43% F). PEx plus osteopathy reached positive GAS scores by improving the ROMs of the spinal column and/or limbs (81% and 78%, respectively), chest and abdomen mobility (82%), and CRI (76%). Only minor reversible adverse effects were noticed during the study. Together, our data seem to initiate a new course where osteopathy could be useful in evaluating structural edges due to the clinical history of each CAYA-H. Given the contributions that were obtained by the GAS scores, osteopathic treatment seems to reveal interesting potential that can be targeted in the future.

Bilateral tibial Brodie's abscess in a young patient treated with BAG-S53P4: case report.

BACKGROUND: Brodie's abscess is a form of chronic pyogenic osteomyelitis that usually affects the cancellous part of the long bones in children. Its treatment is represented by antibiotic therapy alone or in association with surgical procedures. CASE PRESENTATION: A 12-years-old male affected by a Brodie's abscess of the tibia involving the distal growth plate was admitted to the Pediatric Department for a conservative treatment. After several attempts of antibiotic therapy interrupted for intolerance manifestations he was surgically treated with bioactive glass BAG-S53P4 (BonAlive, BonAlive Biomaterials Ltd., Biolinja, Finland), with excellent results. CONCLUSIONS: In our experience BAG-S53P4 has proven to be an effective bone substitute without side effects even in the pediatric population. In our case it eradicated the infection without interfere with the growth; neither epiphysiodesis nor other disorders were found during the follow-up. For the publication of this case report we followed the CARE guidelines for good clinical case reports; the parents gave consent for publication.

Orthopaedic challenges for mucopolysaccharidoses.

Mucopolysaccharidoses (MPS) are a group of diseases characterized by abnormal accumulation of glycosaminoglycans (GAGs). Although there are differences among the various disease types, the osteoarticular system is always involved. The aim of the present study was to establish a framework for MPS-related orthopaedic manifestations and for their treatment. The authors, affiliated to three different Italian Orthopaedic Centres, report data taken from the literature reviewed in light of their accumulated professional experience. Bone alterations make up what is known as dysostosis multiplex, involving the trunk and limbs and with typical radiological findings. Joints are affected by pathological tissue infiltrations. The cervical spinal cord is involved, with stenosis and cervical and occipitocervical instability. In MPS there is a much higher incidence of scoliosis compared with healthy subjects without any particular distinctive feature. Kyphosis of the spine is more frequent and also more severe because of its possible neurological complications, and it is localized at the thoracolumbar level with a malformed vertebra at the top of the deformity. Evolving forms, and those associated with neurological damage, require anteroposterior spine fusion. The hip is invariably involved, with dysplasia affecting the femoral neck (coxa valga), the femoral epiphysis (loss of sphericity, osteonecrosis), and the femoral acetabulum which is flared. All these features explain the tendency to progressive hip migration. Genu valgum is often found (a deviation of the physiological axis with an obtuse angle opening laterally). This deformity is often localized at the proximal tibial metaphysis; it causes functional limitations and leads to an irregular erosion of the articular cartilage. In young patients who still have the growth plate, it is possible to execute a medial hemiepiphysiodesis, a temporary inhibition of cartilage growth, with progressive axis correction. In this paper, the characterisation of clinical features and the review of treatments are divided into separate sections based on the part of the body involved. The conclusions of each section are presented as a summary. One section discusses the high risk of anaesthesia-related complications requiring the collaboration of specifically trained personnel.

Elbow arthroscopy in stiff elbow.

PURPOSE: The purpose of this study was to evaluate and review the functional outcomes after arthroscopic surgery in post-traumatic and degenerative elbow contractures. METHODS: Between 2004 and 2008, 243 patients with post-traumatic or degenerative elbow stiffness were treated with arthroscopic surgery. A total of 212 patients were reviewed at an average of 58 months follow-up (SD ± 17.3). The patients were divided into two groups: group A with post-traumatic stiffness, and group B with degenerative stiffness. Arthroscopic procedures performed included: synovectomy, debridement of osteophytes, removal of loose bodies, anterior and posterior capsulectomy, radial head excision. Ulnar nerve neurolysis was usually performed. The following data were recorded and analysed: sex, age, paraesthesia, previous surgical treatment and complications. Patient outcome was assessed pre- and post-operatively by a visual analogue scale and by the Mayo Elbow Performance Index (MEPI), which assesses pain, ROM, stability and function. RESULTS: The total average ROM improved by 33° in group A and 20° in Group B. The MEPI improved from 60 to 81 in group A, and from 65 to 91 in group B. CONCLUSIONS: Arthroscopic surgery in post-traumatic and degenerative elbow contractures can be considered a safe, useful, with a long learning curve procedure that offers important improvement of the ROM decreasing surgical morbidity.

An Attempt to Induce Transient Immunosuppression Pre-erythrocytapheresis in a Girl With Sickle Cell Disease, a History of Severe Delayed Hemolytic Transfusion Reactions and Need for Hip Prosthesis.

We report on a case of delayed hemolytic transfusion reaction (DHTR) occurred 7 days after an erythrocytapheresis or eritroexchange procedure (EEX) treated with rituximab and glucocorticoids in a 15-years old patient with sickle cell disease. EEX was performed despite a previous diagnosis of alloimmunization, in order to reduce hemoglobin S rate before a major surgery for avascular necrosis of the femoral head. A first dose of rituximab was administered before EEX. However, rituximab couldn't prevent DHTR that occurred with acute hemolysis, hemoglobinuria and hyperbilirubinemia. A further dose of rituximab and three boli of methylprednisolone were given after the onset of the reaction. It is likely that the combined use of rituximab and steroids managed to gradually improve both patient's general conditions and hemoglobin levels. Nor early or late side effects were registered in a 33-months follow-up period. This report suggests the potential effectiveness and safety of rituximab in combination with steroids in managing and mitigating the symptoms of delayed post-transfusional hemolytic reactions in alloimmunized patients affected by sickle cell disease with absolute need for erythrocytapheresis.