The enhanced recovery after surgery (ERAS) protocol is not routine management in paediatric orthopaedics. Cultural differences or assumptions about the financing of medical care in different countries encourage creative adaptation of general assumptions in local communities. The aim of this study was to compare the quality of the perioperative period before and after the introduction of an interdisciplinary protocol adopted to organisational conditions. A group of 4098 children were included in the “before–after” observational study. The data of 1553 patients (BEFORE group) were analysed in terms of compliance with the enhanced recovery after surgery protocol guidelines and the time and cost of hospitalisation over a 20-month period. A novel interdisciplinary protocol was developed, including an education and training app called BackOnFeet (BOF^®), standardised hospital management, and the introduction of methods novel to Poland (intraoperative nerve cryoanalgesia in children). A further 2545 patients (AFTER group) were reassessed over a period of 20 months. It was found that the groups differed in hospitalisation time (p < 0.001), type of procedures, and percentage ratio of costs incurred to revenue generated. The usefulness of the BOF^® app as an effective educational tool was demonstrated. The optimisation of perioperative management in paediatric orthopaedics based on novel tools and the interdisciplinary ERAS protocol is possible and brings tangible benefits in psychological, organisational, and financial terms.

Previously, dominant partial interferon-gamma receptor 1 (IFN-g-R1) susceptibility to environmental mycobacteria was found with IFNGR1 deletions or premature stop. Our aim was to search for IFNGR1 variants in patients with mycobacterial osteoarticular lesions. Biopsies from the patients were examined for acid-fast bacilli, inflammatory cell infiltration, and mycobacterial niacin. Mycobacterial rRNA was analyzed using a target-amplified rRNA probe test. Peripheral-blood-leukocyte genomic DNA was isolated from 19 patients using the QIAamp DNA Mini Kit, and all IFNGR1 exons were sequenced using an ABIPRISM 3130 device. After the discovery of an exon 5 variant, a Polish newborn population sample (n = 100) was assayed for the discovered variant. Splice sites and putative amino acid interactions were analyzed. All patients tested were positive for mycobacteria; one was heterozygous for the IFNGR1 exon 5 single-nucleotide-missense substitution (g.20746A>G, p.Ile183Val). No other variant was found. The splice analysis indicated the creation of an exonic splicing silencer, and alternatively, molecular graphics indicated that the p.Ile183Val might alter beta-strand packing (loss of van der Waals contacts; Val183/Pro205), possibly altering the IFN-g-R1/IFN-g-R2 interaction. The probability of non-deleterious variant was estimated as <10%. Heterozygous IFNGR1:p.Ile183Val (frequency 0.003%) was found to be coincidental with mycobacterial osteomyelitis. The small amount of variation detected in the patients with osteoarticular lesions indicates that screens should not yet be restricted: Intronic variants should be analyzed as well as the other genes affecting Type 1 T-helper-cell-mediated immunity.

Background. Orthopedic bracing is used in conservative treatment of spinal curvatures. Apart from rigid braces, SpineCor dynamic braces with a flexible design have recently become available. The idea behind dynamic bracing is that derotational and correcting forces are transmitted via a system of corrective bands. The essence of this technique is maintenance of spine mobility while effecting a position in which all components of the three-plane deformity are corrected.The aim of this study is to evaluate early outcomes of SpineCor dynamic brace treatment for idiopathic scoliosis according to SRS methodology and criteria. Material and methods. The study group included 50 patients who were using SpineCor braces due to idiopathic scoliosis. The indication for bracing was the finding of a >15° spinal curvature in skeletally immature patients (Risser grade 0-3). Correction or stabilization of the scoliosis (Cobb angle change of ±5°) were recognized as positive outcomes, while a negative outcome was defined as progression of the curve of more than 5° or to a value necessitating operative treatment. The study group was divided in to subgroups at enrolment, according to gender and degree of scoliosis.

Caffey-Silverman syndrome, or infantile hyperostosis, is a rare condition of unclear etiology and pathogenesis affecting the skeletal system and the surrounding soft tissues. It is characterized by indurated swelling of soft tissues and cortical bone hyperostosis. The changes are usually multiple and affect such parts as the mandible, scapulae, ribs, clavicles, and forearm and shank bones. When long bones are affected, the lesions are typically limited to shafts, with the sparing of meta- and epiphyses. The prognosis is usually good and, in most patients, the changes resolve spontaneously after several months to over a year, leaving no permanent sequelae. Caffey-Silverman syndrome needs to be distinguished from osteitis, for which it is most often mistaken. We present a case of an early form of Caffey-Silverman syndrome. The course of disease in this form is usually severe, with multifocal lesions, and the typical self-limiting regression is not complete. In our patient, a systemic musculoskeletal condition was already suspected following the detection of skeletal defects in a prenatal US examination. Physical and radiological work-up in the first weeks of life revealed the typical signs of congenital Caffey-Silverman syndrome. Several years of follow-up showed gradual regression of the bone deformities with persistent lower-extremity bowing. Due to a positive family history for Caffey-Silverman syndrome, the patient's pedigree was prepared. On the basis of the history data, existing radiographs and in- and outpatient medical records, Caffey-Silverman syndrome was confirmed in 10 family members. It was established that the cases of Caffey-Silverman syndrome in the patient's family were characterized by diverse phenotypic expression and different times of onset.