Lower limb deformities in patients with hypophosphatemic rickets are multi-apical and require multiple osteotomies for correction. Intramedullary nails (IMNs) are used to fix multiple osteotomies. Authors of this studyaimed to assess the clinical and radiographic outcomes of using IMNs for correcting lower limb deformity in adolescents with hypophosphatemic rickets. This prospective study included patients with hypophosphatemic rickets who underwent deformity correction between November 2020 and November 2023 using IMNs, with a minimum follow-up of 2 years. Clinical outcomes were assessed using the Lower Limb Deformity-Scoliosis Research Society (LD-SRS) score. Radiographic outcomes measured included the mechanical tibiofemoral angle (mTFA), mechanical axis deviation (MAD), mechanical lateral distal femoral angle (mLDFA), mechanical medial proximal tibial angle (mMPTA), and Stevens’ knee joint zoning. Twenty patients (25 limbs and 35 bones) were included (mean age: 16 years; range: 13–22 years). The LD-SRS score improved from 3.2 ± 0.4 preoperatively to 4.3 ± 0.7 postoperatively. Preoperative mechanical tibiofemoral angle was 27.8° ± 12.1° and 17.33° ± 7.9° in the varus and valgus groups, respectively, improving to 3.4° ± 6.4° and 2° ± 5.2°, respectively. Preoperative mechanical axis deviation was 82.2 ± 35.6 mm and 41.8 ± 22.2 mm in the varus and valgus groups, respectively, improving to 9.2 ± 20.1 mm and 4.6 ± 12.6 mm, respectively. Preoperative mechanical lateral distal femoral angle was 102.4° ± 9.7° and 77.9° ± 9.8° in the varus and valgus groups, respectively, improving to 89.9° ± 3.1° and 88.1° ± 3.1°, respectively. Preoperative mechanical medial proximal tibial angle was 78.7° ± 8° and 89.7° ± 3.9° in the varus and valgus groups, respectively, improving to 88.6° ± 3.4° and 88.2° ± 2.9°, respectively. Preoperative Stevens’ knee joint zoning was Zone 3 in all patients, improving to Zone 1 in 16 limbs, Zone 2 in eight limbs, and Zone 3 in one limb. Correction of severe frontal plane lower limb deformities in adolescents with hypophosphatemic rickets using IMNs yields good clinical and radiographic outcomes at 2-year follow-up.
Asthma has been associated with early vascular changes that elevate the risk of atherosclerosis and cardiovascular diseases. Carotid intima-media thickness (CIMT) serves as a non-invasive marker for subclinical atherosclerosis and cardiovascular risk. However, existing literature presents conflicting results regarding CIMT measurements in asthmatic patients. This study aims to evaluate whether CIMT is elevated in asthma patients compared to healthy controls and to explore potential modifiers. We conducted a systematic review following PRISMA guidelines, performing a literature search across PubMed, Web of Science, ScienceDirect, and the WHO VHL. We calculated pooled standardized mean differences (SMD) with 95% confidence intervals (CI) to assess the differences in CIMT values. Heterogeneity was evaluated using the I² statistic, and subgroup analyses and meta-regression were utilized to identify sources of heterogeneity. Our review included a total of 10 studies. The analysis indicated significantly higher CIMT values in asthma patients compared to healthy controls, with a pooled SMD of 0.65 (95% CI: 0.19 to 1.12, p = 0.005). A limited number of studies addressed various factors, such as disease severity and the use of inhaled corticosteroids, which may influence CIMT measurements. Notably, asthmatic patients, particularly children and adolescents, exhibited higher CIMT values compared to healthy controls, suggesting a potential association with subclinical atherosclerosis in this demographic. However, these findings should be interpreted with caution due to the observational nature of the included studies and the risk of residual confounding. Further longitudinal research is necessary to elucidate the effects of disease characteristics and treatment on vascular health.
While heterotopic ossification (HO) around the hip is frequently cited as a late complication leading to stiffness or technical difficulty during reconstructive surgery, its role as a primary mechanical impediment to the reduction of acute intertrochanteric fractures is rarely documented. We present the case of a 45-year-old male with a significant history of polytrauma, traumatic brain injury, and conservatively managed bilateral acetabular fractures. The patient presented with mature peritrochanteric and periacetabular HO and an acute, comminuted right intertrochanteric femur fracture following a low-speed motor vehicle collision. Preoperative imaging confirmed extensive ossification bridging the greater trochanter and lateral acetabulum. Intraoperatively, standard closed reduction on a traction table proved impossible, as the proximal femoral fragment was anchored by a rigid, extra-articular bony block. To achieve reduction, a limited osteotome-assisted release of the obstructing HO bridge was performed without requiring radical excision. This maneuver restored proximal femoral mobility, facilitating successful reduction and internal fixation. The postoperative course was unremarkable; follow-up imaging confirmed fracture union and stable hardware. This case emphasizes that mature periarticular HO can act as a definitive mechanical block to closed reduction. Surgeons should utilize CT-based preoperative planning to identify such obstacles early, allowing for a targeted surgical release when standard reduction maneuvers fail.
The rising cost of new health technologies and limited healthcare budgets challenge health systems worldwide, including Oman. Local value judgments are crucial for informed decision-making. This study aims to establish a cost-effectiveness thresholds (CETs) framework for Oman, aligned with the country’s economic status and healthcare priorities. The key findings from a recent literature review on global CETs and their applications were shared and discussed during a multi-stakeholder workshop in March 2024. Presenting these findings has provided participants a broad understanding of global CET practices and highlighted the use of multiple thresholds across different settings. Afterwards, a survey was distributed, asking participants to anonymously vote on six key concepts: the basis for the threshold, the baseline threshold multiplier, the use of multiple thresholds, the criteria for multiple thresholds, the number of multipliers, and which criterion should receive the 3X multiplier. The results from this voting guided the design and final agreement on the threshold values. Twenty respondents supported adopting multiple CET values based on cost per quality-adjusted life-year (QALY), linked to Oman’s economic status. A baseline CET equal to 1x Gross Domestic Product (GDP) per capita was suggested. Participants recommended three different threshold multipliers to reflect policy priorities. A continuous multiplier (1–3x GDP), based on incremental QALY gains, was used to favor technologies with greater health gains. Additionally, a fixed multiplier of 2 was chosen for both orphan drugs and treatments for priority diseases. The resulting CET values reflect technical and expert consensus and do not include patient or public perspectives. In Oman, the incremental cost per QALY gained should not exceed 1x GDP per capita for new health technologies that offer minimal health gains in common diseases. However, thresholds might increase to 12x GDP per capita for curative treatments in rare priority disease areas. It is recommended that CET values be reviewed and updated within three years, based on initial implementation experiences.
Nasal continuous positive airway pressure (CPAP) is widely used in premature infants but may cause pressure-related nasal injury. Severe deformities involving structural loss can result in significant airway compromise. We report a preterm infant presenting at 3 months of age with progressive nasal deformity, near-complete unilateral nostril occlusion, and features of obstructive sleep apnea complicated by pulmonary hypertension. Early reconstruction was performed at 6 months using autologous conchal cartilage to restore the lateral crus and a helical root composite chondrocutaneous graft for columellar support. Postoperative nasal stenting was utilized. The patient demonstrated marked improvement in airway patency, respiratory function, and tolerance of respiratory support, with stable structural and aesthetic outcomes at 3 months. This case highlights that early airway-directed reconstruction in selected infants is feasible and may prevent progression of functional and cardiopulmonary sequelae.
