Introduction. Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV is an extremely rare syndrome. Three clinical findings define the syndrome: insensitivity to pain, impossibility to sweat, and mental retardation. This pathology is caused by a genetic mutation in the NTRK1 gene, which encodes a tyrosine receptor (TrkA) for nerve growth factor (NGF). Methods. The consultation of a child female in our center with CIPA and a tibia fracture in pseudoarthrosis encouraged us to carefully review literature and examine the therapeutic possibilities. A thorough review of literature published in Pubmed was done about CIPA and other connected medical issues mentioned in the paper. Conclusions. The therapeutic approach of CIPA remains unclear. The preventive approach remains the only possible treatment of CIPA. We propose two new important concepts in the therapeutic approach for these patients: (1) early surgical treatment for long bone fractures to prevent pseudoarthrosis and to allow early weight bearing, decreasing the risk of further osteopenia, and (2) bisphosphonates to avoid the progression of osteopenia and to reduce the number of consecutive fractures.

Radial polydactyly occurs most frequently as an isolated defect, but it may also occur in association with other abnormalities, or as part of a syndrome. The Wassel classification is the universal classification for thumb duplication. One of the limitations of it is the misclassification of the immature epiphysis. The Wassel classification not only refers to the pathoanatomy of the polydactyly but also guides the selection of various surgical techniques. Three well-defined surgical objectives should be ensured: thumb alignment, stability, and an acceptable final esthetic appearance. Tada score is a validated functional scale that takes into account the most common and limiting complications, namely clinodactyly and instability. Based on some recent strong research evidence, the JSSH assessment system provides the most reliable outcome of scores. More studies are required to provide evidence-based conclusions regarding the treatment of radial polydactyly.

Objectives This study aims to analyze the functional results, management, and complications of acute Seymour fracture treatment and to generalize the understanding of Seymour fractures, as well as awareness about its controversial treatment and critical sequelae. Patients and methods Between January 1994 and December 2019, a total of 29 patients (20 males, 9 females; mean age: 7.9±3.9 years; range, 1 to 15 years) who presented within the first 24 h of injury and were diagnosed with Seymour fractures and treated in the emergency setting were retrospectively analyzed. Clinical and radiological data were collected from medical records at the time of diagnosis and during follow-up, within a week after the treatment and in the visits required until fracture healing, and no sequelae were observed. In the event of complications, a minimum of one year of follow-up was carried out. Radiographs were taken of the anteroposterior and lateral views during each visit. Results The mean follow-up was 10.8±8.6 (range, 2 to 36) months. Surgical treatment in the operating room was performed in 24 (82.7%) patients using a single longitudinal Kirschner wire (K-wire) fixation through the distal phalanx and the distal interphalangeal joint in 21 patients. Non-operative treatment based on closed reduction and splinting was performed in five (17.3%) patients. There was no statistically significant difference in the final passive range of motion and physeal growth arrest in relation to the use or non-use of K-wires. The use of antibiotics in any of the three possible administrations (intravenous antibiotic regimen, intravenous and later oral antibiotic at-home or oral antibiotics), in relation to the non-use of antibiotics seemed to be a protective factor against infections (odds ratio=0.04; 95% confidence interval: 0.006-0.2; p=0.001). Conclusion The identification of Seymour fractures is crucial for applying the correct treatment and reducing the risk of complications, such as osteomyelitis and physeal alterations. Based on our study results, we can suggest that the use of an antibiotic regimen causes a lower risk of infections in acute Seymour fractures. The prompt identification of these fractures with a standardized protocol covering irrigation, debridement, reduction, fixation, and prophylactic antibiotics is needed to avoid complications.

The term hemihyperplasia, defined as the pathological growth process that involves an abnormal proliferation of cells, has been replaced with the term hemihypertrophy, defined as the increase in size of existing cells. [1] Recently, newly proposed terms have been used rather than these histopathological and nonclinical concepts, as asymmetric regional body overgrowth and lateralized overgrowth (LO), are used synonymously to indicate a significant increase in the length and/or girth of most or all of one side Objectives: This study aims to increase the awareness of the association between lateralized overgrowth (LO) and abdominal tumor among the pediatric orthopedic community and to evaluate its incidence in our center. Patients and methods: Between January 1997 and December 2021, a total of 166 patients with Wilms tumors and hepatoblastomas were retrospectively analyzed. Data including age, sex, initial clinical signs (hematuria, abdominal mass with or without general discomfort), type of asymmetric regional body overgrowth (isolated or in relation with any syndrome), and tumor stage at diagnosis were recorded. In addition, age at which asymmetric regional body overgrowth was described and age at the time of tumor diagnosis were noted. Results: Of a total of 166 patients, 133 were diagnosed with Wilms tumors (nephroblastomas) and 33 were diagnosed with hepatoblastomas. In 94% of the cases, the initial clinical signs were an abdominal mass and/or hematuria. Overall, five (3%) patients presented with LO. Four patients with Wilms tumor presented it at the initial clinical examinations. In three of these cases (2.3%), we found it isolated and, in the remaining patient (0.75%), it was associated with Beckwith-Wiedemann spectrum. Only one patient affected from hepatoblastoma (3%) presented with an isolated LO at the time of tumor diagnosis. Conclusion:Our study results show an incidence of LO in relation to intra-abdominal tumors of 3%. The latest updates recommend genetic testing to identify subgroups with a higher risk for tumor development that are more likely to benefit from tumor protocol surveillance.