OBJECTIVE: To systematically review the literature for reports on Wolcott-Rallison syndrome, focusing on the spectrum and natural history, genotype-phenotype correlations, patient and native liver survival, and long-term outcomes.
METHODS: PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow-up were extracted. Survival and correlation analyses were performed.
RESULTS: Sixty-two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5 months (IQR 2) and of death was 36 months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver-pancreas in one and combined liver-pancreas-kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (p = .0057). One-, five- and ten-year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (p = .013).
CONCLUSIONS: Wolcott-Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver- or multi-organ transplantation is a feasible treatment option to improve survival.

Nonossifying fibroma (NOF) is a common benign bone neoplasm and is usually observed in the first 2 decades of life. Most NOFs occur in the metaphysis of long bones of the lower extremities and migrate toward the diaphysis during skeletal maturation. Epiphyseal involvement by NOF has been rarely reported, with only one case found in the English literature. The authors report the second case of NOF involving the epiphysis of a long bone, the proximal tibia of a 21-year-old woman. Clinicians and pathologists should be aware of the rare possibility of epiphyseal involvement of long bones by this condition. Pathologists should select appropriate immunohistochemistry markers to rule out alternative diagnoses.

Chondroblastoma is a rare benign cartilaginous tumor mostly confined to the epiphyses and apophyses. Cases outside the epiphyseal region are exceedingly rare. Extramedullary chondroblastomas are exceptional; to our knowledge, only two cases qualified as \"periosteal chondroblastoma\" have been described in the literature. We report two cases of metaphyseal periosteal chondroblastoma both located on the inferior surface of the femoral neck. Both cases were paucicellular with an unusual dense sclerotic reaction. The diagnosis of chondroblastoma was supported by the expression of histone 3.3, K36M mutant in tumor cells.

The AGFAD (Arbeitsgemeinschaft für Forensische Alterdiagnostik, Study Group on Forensic Age Diagnostics) has published several recommendations regarding both technical aspects of computed tomography (CT) of the medial clavicular epiphysis (MCE) and the process of reading and interpreting the CT images for forensic age estimations (FAE). There are, however, no published recommendations regarding CT scan protocols and no dose reference values for CT of the MCE. The objective of this analysis was to assess adherence to AGFAD recommendations among practitioners of FAE and analyse reported dose-relevant CT scan parameters with the objective of helping to establish evidence-based dose reference values for FAE. A systematic literature search was conducted in PubMed and in Google Scholar with specific MeSH terms to identify original research articles on FAE with CT of the MCE from 1997 to 2022. A total of 48 studies were included. Adherence to AGFAD recommendations among practitioners of FAE is high regarding the use of Schmeling main stages (93%), bone window (79%), ≤ 1 mm CT slices (67%), axial/coronal CT images (65%), and Kellinghaus sub-stages (59%). The reporting of CT technique and CT dose-relevant scan parameters is heterogeneous and often incomplete in the current literature. Considering the success achieved by the AGFAD in creating standards of practice of FAE in living subjects, there is potential for the AGFAD to establish standards for radiation protection in FAE as well.

Guided growth affects the physis in children to produce a desired effect. Several devices achieve alteration of growth, including staples, plates, and screws. Complications can include device failures, failure to modulate growth as expected, and unintended physeal arrest. We present the results of a unique technique designed to minimize these complications. This was a retrospective review of guided growth at the knee at a single institution utilizing cannulated screws with epiphyseal-entry points. Each case was reviewed to determine the presence of complications related to guided growth, including implant breakage, implant pull-out or pull-through, iatrogenic physeal arrest, failure to modulate growth, and the incidence of revision surgeries. There were 89 patients who had 146 epiphyseal-entry guided growth procedures with a total of 221 4.5 mm cannulated screws. There were no iatrogenic physeal arrests. Five (2.26%) screws in 4 (4.49%) patients had either a broken screw or screw pull-out/pull-through requiring revision procedures. Three patients had osteotomies after skeletal maturity. Four had revision-guided growth for other reasons: 2 due to a lack of timely follow-up, 1 for iatrogenic genu varum without implant failure, and 1 due to recurrent deformity after implant removal. Revision procedures were unanticipated in 6 (6.74%) patients. This study describes a technique for placing cannulated screws at the knee with epiphyseal starting points. Our rate of complications and number of revision surgeries compare favorably with those noted for other techniques. Guided growth using epiphyseal-entry cannulated screws is a safe, effective option for most patients.

OBJECTIVE: To summarize the current peer-reviewed publications on minimally invasive surgery (MIS) for medial coronoid process disease (MCPD) in dogs.
METHODS: Invited review.
METHODS: Pubmed, CAB Abstracts and Scopus databases were utilized for literature review (1990-2023).
RESULTS: More than 60 papers have been published that involve MIS evaluation or treatment of MCPD. Six prospective trials incorporated quantitative gait analysis evaluating outcome following MCPD debridement, of which three included a control group. Whilst case numbers were limited, all studies employing a control group failed to demonstrate superiority of surgery over nonsurgical management. Of three studies employing gait analysis and no control group, all documented some improvement in function compared to pretreatment lameness. Multiple studies document progression of osteoarthritis in dogs following MCPD debridement although this may not be synonymous with worsened function. Subtotal coronoid osteotomy (SCO) is described as an alternative to MCP fragment removal, although the long-term biomechanical consequences are currently unclear and quantitative data on the efficacy of this treatment in isolation for MCPD is awaited.
CONCLUSIONS: Arthroscopy of the canine elbow joint is a safe means by which to evaluate MCP pathology. Quantitative longitudinal analysis of outcome following minimally invasive surgery for MCPD is required. Correlation with clearly defined subclassification inclusion criteria such as age, incongruity and the precise arthroscopic findings in each case are required to more accurately differentiate any superiority of surgery over nonsurgical management for this disease.

Only a few case reports regarding pediatric posterior cruciate ligament (PCL) ruptures without bone avulsion exist in the literature. The present study aims to share our experience in the diagnosis, treatment, and prognosis of a child with a proximal PCL tear.
This article reports a 5-year-old female diagnosed with a proximal PCL tear. The ruptured PCL was repaired with an all-epiphyseal suture tape augmentation (STA) without evidence of growth plate violation.
The suture tape was removed under arthroscopy and revealed the PCL was re-attached at 12 months after the first surgery. And at the time of this report, 36 months after surgery, she was doing well without any problems and with negative posterior drawer test.
Pediatric PCL tear without bone avulsion is rare. However, the torn PCL was noticed healed based on an arthroscopic second-look.

A total of 227 articles published within the last twenty years, indexed in the PUBMED and Researchgate databases, were reviewed for the purpose of comparing medical imaging-based methods of age estimation. The evaluated studies were analyzed in terms of the assessed parts of the body, age, and epiphyseal fusion ages in children, adolescents, and young adults. Our analysis showed that an overwhelming majority of studies had been based on computed tomography and magnetic resonance imaging. A comparison of the studies showed that, irrespective of the imaging modality and the nationality of study population cohorts, the rates of development and the ages at which the process of ossification begins and ends show certain trends.

BACKGROUND: Considerable anatomic variations of sagittal femoral condylar shape have been reported, with a continuum between spherical (or single-radius) and ovoid (or multi-radius) condyles. The purpose of this systematic review and meta-analysis was to critically appraise and synthesise the available literature on the sagittal femoral profile. The hypothesis was that studies would reveal considerable variability among individuals, but also in their methodology to quantify sagittal profiles.
METHODS: This systematic review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. On 10 September 2021 two authors searched for Level I to IV studies that reported on the sagittal curvature of the medial and/or lateral femoral condyles using the MEDLINE®, EMBASE® and Cochrane Library. Results were summarised by tabulating means, standard deviations and/or ranges for the reported radii-of-curvature, or ellipsoidal semi-major and semi-minor lengths of the condyles. To quantify sagittal \'ovoidicity\' and asymmetry, results were stratified according to coordinate reference frame (posterior condylar axis (PCA), clinical and surgical transepicondylar axis (cTEA and sTEA), unified sagittal plane (USP), or unclear) and summarised in forest plots as standardised mean differences (SMD).
RESULTS: Thirty-eight articles were eligible for full text extraction, quantifying sagittal radii-of-curvature by best-fit circles (BFC), ellipsoids, polynomials, spherical or cylindrical fitting. Studies with clear definition of the measurement plane revealed that both condyles were generally ovoid, with considerably greater \'ovoidicity\' at the medial condyle (SMD, 4.09) versus the lateral condyle (SMD, 3.33). In addition, distal condylar radii were greater medially when measured normal to the TEA (cTEA: SMD, 0.81; sTEA: SMD, 0.79), but greater laterally when measured in a USP (SMD, - 0.83). Posterior condylar radii were greater laterally when measured in a USP (SMD, - 0.60).
CONCLUSIONS: Studies reported considerable variability of sagittal femoral condylar radii-of-curvature, which are not incremental, but rather a continuum that ranges from spherical to ovoid. Although this variation could be accommodated by single-, dual- and multi-radii femoral components, a surgeon typically uses only one or two TKA designs. Hence, there is a risk of mismatch between the native and prosthetic sagittal profile that could result in mid-flexion ligament imbalance unless other parameters are changed. These findings support the drive towards patient-specific implants to potentially achieve accurate sagittal bone-implant fit through implant customisation.
METHODS: IV.

Pubertal children with significant growth retardation represent a considerable therapeutic challenge. In growth hormone (GH) deficiency, and in those without identifiable pathologies (idiopathic short stature), the impact of using GH is significantly hindered by the relentless tempo of bone age acceleration caused by sex steroids, limiting time available for growth. Estrogen principally modulates epiphyseal fusion in females and males. GH production rates and growth velocity more than double during puberty, and high-dose GH use has shown dose-dependent increases in linear growth, but also can raise insulin-like growth factor I concentrations supraphysiologically, and increase treatment costs. Gonadotropin-releasing hormone analogs (GnRHas) suppress physiologic puberty, and when used in combination with GH can meaningfully increase height potential in males and females while rendering adolescents temporarily hypogonadal at a critical time in development. Aromatase inhibitors (AIs) block androgen to estrogen conversion, slowing down growth plate fusion, while allowing normal virilization in males and stimulating longitudinal bone growth via androgen receptor effects on the growth plate. Here, we review the physiology of pubertal growth, estrogen and androgen action on the epiphyses, and the therapeutic impact of GH, alone and in combination with GnRHa and with AIs. The pharmacology of potent oral AIs, and pivotal work on their efficacy and safety in children is also reviewed. Time-limited use of AIs is a viable alternative to promote growth in pubertal males, particularly combined with GH. Use of targeted growth-promoting therapies in adolescence must consider the impact of sex steroids on growth plate fusion, and treatment should be individualized.