Whole Body, Whole Life, Whole Family: Patients' Perspectives on X-Linked Hypophosphatemia.

The rare genetic disorder X-linked hypophosphatemia (XLH) is often exclusively considered to impact children, and, as such, adult patients with XLH may receive inadequate care because their symptoms are not associated with XLH. However, studies have shown that XLH has long-term adverse health consequences that continue throughout adulthood requiring comprehensive lifelong care. Indeed, XLH impacts patients' whole body, whole life, and whole family. XLH does not just affect the bones; symptoms are chronic and progressive, worsening throughout adulthood, and the burden of XLH overflows into the lives of a patient's family, friends, peers, and colleagues. To ensure early recognition, comprehensive care, and adequate management of XLH, there are key steps that clinicians can incorporate into their daily practice. These include education, a multidisciplinary approach, open communication, and support. Clinician education on rare disorders such as XLH is critical, and healthcare professionals (HCPs) should ensure that patients and their caregivers have access to XLH-related information. As a whole-body disorder, XLH requires a coordinated approach to treatment across specialties. Frequent open communication among members of the healthcare team is needed to increase HCPs' knowledge about XLH, and open communication must extend to the patient as well to ensure the patient's concerns and needs are addressed and treatment is tailored to their specific individual needs. Multiple networks of support, including social and psychological support, should be offered to patients and their families. A basic understanding that XLH affects patients' whole bodies, whole lives, and whole families is the first step toward accomplishing improved patient care.

The Role of Prophylactic Peroneal Nerve Decompression in Patients with Severe Valgus Deformity at the Time of Primary Total Knee Arthroplasty.

Background: Common peroneal nerve (PN) palsy after total knee arthroplasty (TKA) is a serious complication. Although many authors suggest delayed or immediate PN decompression after TKA in these patients, little is known about the role of prophylactic peroneal nerve decompression (PPND) at the time of TKA. The aim is to report the results of PPND in high-risk patients at the time of TKA. Materials and methods: A multi-institutional retrospective study review of nine patients (10 knees) who underwent PPND at the time of TKA was conducted. Patients who had severe valgus deformities (≥15° of femorotibial angle and not fully correctable by examination under anaesthesia) with or without flexion contractures were included. PPND was performed through a separate 3-4-cm incision at the time of TKA. The demographics, preoperative and postoperative anatomical and mechanical alignments, range of motion, operation time, postoperative neurological function and complications were recorded. Results: All patients had a completely normal motor and sensory neurological function postoperatively and no complications related to PPND were reported. All patients followed the standard physical therapy protocol after TKA without modifications.The mean preoperative femorotibial angle was 20° (range 15-33°) and the mean postoperative femorotibial angle was 6.3° (range 5-9°) (p = 0.005). The mean preoperative flexion contracture was 9 (range 0-20) and the mean residual contracture was 1.2° (range 2-5°) (p = 0.006). Conclusion: PPND at the time of TKA is an option to minimise the risk of PN palsy in high-risk patients. This approach can be considered for patients undergoing TKA in selected high-risk patients with a severe valgus deformity. How to cite this article: Makhdom A, Hamilton AA, Rozbruch SR. The Role of Prophylactic Peroneal Nerve Decompression in Patients with Severe Valgus Deformity at the Time of Primary Total Knee Arthroplasty. Strategies Trauma Limb Reconstr 2022;17(1):38-43.

Core psychosocial issues for children and adolescents in the context of limb lengthening and reconstruction surgery treatment.

PURPOSE: Addressing the psychosocial needs of adolescents can improve surgical outcomes. The aim of this retrospective comparative study was to understand the core psychosocial factors that shaped the experiences of adolescents who underwent multiple limb lengthening/reconstruction surgeries (LLRS). METHODS: A novel 62-question survey was developed and administered to 31 patients from the study institution. Data was obtained using a self-report inventory assessing medical care, communication/connection to doctor, peer relations, physical space, self-esteem, counselling/clergy, physical/emotional support, school issues and concerns about future. This survey and demographic questions were administered to young adults (now aged 18 to 30 years) who underwent LLRS treatment between the ages of 11 to 20 years. RESULTS: Psychosocial needs were determined to be within the categories of body image/self-esteem, subjective perception of treatment, patient-physician relationship, role of parents, peer interactions, academic performance and hospital experience. Patients valued parental involvement while also wanting their surgeon to speak directly to them. They preferred to be in private rooms on the paediatric floor and to not socialize while in the hospital. They were indifferent to keeping up with friends, speaking to a counsellor and having their surgeon inquire about their emotions. They expressed concern about pain, carrying out activities and the financial impact of surgery. CONCLUSIONS: Adolescent LLRS patients value focused psychosocial support from their surgeon and caregivers. This perceived level of support influences their ability to cope with their condition. These findings helped us understand the psychological issues and preferences of adolescents who underwent LLRS and can assist orthopaedic surgeons in providing holistic care. LEVEL OF EVIDENCE: IV.