What Do We C in Children With Scurvy? A Case Series Focused on Musculoskeletal Symptoms.

OBJECTIVES: Vitamin C deficiency in children commonly presents with musculoskeletal symptoms such as gait disturbance, refusal to bear weight, and bone or joint pain. We aimed to identify features that could facilitate early diagnosis of scurvy and estimate the cost of care for patients with musculoskeletal symptoms related to scurvy. METHODS: We conducted a retrospective chart review of patients at a single site with diagnostic codes for vitamin C deficiency, ascorbic acid deficiency, or scurvy. Medical records were reviewed to identify characteristics including presenting symptoms, medical history, and diagnostic workup. The Pediatric Health Information System was used to estimate diagnostic and hospitalization costs for each patient. RESULTS: We identified 47 patients with a diagnosis of scurvy, 49% of whom had a neurodevelopmental disorder. Sixteen of the 47 had musculoskeletal symptoms and were the focus of the cost analysis. Three of the 16 had moderate or severe malnutrition, and 3 had overweight or obesity. Six patients presented to an emergency department for care, 11 were managed inpatient, and 3 required critical care. Diagnostic workups included MRI, computed tomography, echocardiogram, endoscopy, lumbar puncture, and/or EEG. Across all patients evaluated, the cost of emergency department utilization, imaging studies, diagnostic procedures, and hospitalization totaled $470 144 (median $14 137 per patient). CONCLUSIONS: Children across the BMI spectrum, particularly those with neurodevelopmental disorders, can develop vitamin C deficiency. Increased awareness of scurvy and its signs and symptoms, particularly musculoskeletal manifestations, may reduce severe disease, limit adverse effects related to unnecessary tests/treatments, and facilitate high-value care.

Percutaneous Reduction and Provisional Pinning of the Distal Fibula: A Novel Way to Maintain Fibular Length in Temporary Stabilization.

Ankle fractures are commonly observed by orthopaedic trauma surgeons, foot and ankle surgeons, and general orthopaedists with more than 135,000 fractures surgically treated every year in the United States. 1 The soft tissue envelope surrounding the osseous anatomy of the ankle is limited and many injuries, including those that result from higher injury mechanisms and/or those occurring in hosts with vascular and metabolic comorbidities may benefit from a delay in definitive fixation; this may minimize risks of wound healing issues and fracture-related infection. 2,3 Open fractures may benefit from temporizing fixation after debridement and irrigation for the same reasons. 4-6 The benefits of temporizing external fixation in pilon fractures has been recognized for decades. 7 The use of ankle-spanning external fixation for ankle fractures and dislocations has become more frequently used to maintain coronal and sagittal alignment of the ankle mortise as a bridge to definitive fixation. 8-10 However, standard ankle-spanning external fixation techniques do not always directly restore fibular length and rotation, especially in fracture patterns with syndesmotic disruption and/or segmental fibular comminution. Some authors have advocated for limited internal fixation of fibula fractures at time of external fixation application to address this problem, 7 but this still necessitates a surgical approach through a traumatized soft tissue envelope and potentially compromises posterolateral and anterolateral approaches that could be used to address associated Volkman or Chaput fragments. Delayed distal fibular open reduction and internal fixation of shortened fractures has frustrated orthopaedic surgeons for decades and techniques such as "push-pull" screws 11 and extensive soft tissue dissection 12 are often necessary to restore anatomic fibular reduction of length and rotation. Given these challenges, the senior author began to use a technique of percutaneous reduction and buried provisional pinning of the distal fibula to the talus for fibula fractures with persistent shortening after ankle-spanning external fixation of injuries not deemed appropriate for immediate open reduction and internal fixation. We aim to report early results and describe this novel, low-cost surgical technique.

Outcomes After Operative Treatment of Pediatric Monteggia Fracture-Dislocations: Comparison Between Open and Closed Injuries.

BACKGROUND: Open pediatric Monteggia fracture-dislocations are a relatively uncommon injury pattern, with limited numbers reported in previous series. Open fracture-dislocations frequently represent more severe injury patterns with potential for contamination. We aim to determine differences in long-term clinical and functional outcomes in the operative management of closed versus open pediatric Monteggia fracture-dislocations. METHODS: A retrospective review of operatively treated pediatric Monteggia fracture-dislocations was performed. Closed versus open injuries were compared in both clinical outcomes, as well as patient-reported outcomes through Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire. RESULTS: Of 30 operatively treated injuries, 12/30 (40%) were open fracture-dislocations. Patients were followed clinically for an average of 15.65 months in open injuries and an average of 4.61 months in closed injuries. A trend toward increased time to union was observed, however, significance was not achieved; open injuries averaged 8.0 versus 5.8 weeks for closed injuries ( P =0.07). Two patients (11%) in the closed fracture group experienced postoperative complications; both were minor. Five patients (42%) in the open fracture-dislocation group experienced a total of 6 postoperative complications; 5 of the 6 complications were major. QuickDASH scores were obtained at an average of 5 years postoperatively; mean QuickDASH scores were higher in the open fracture group, 13.1, compared with the closed fracture group, 5.9 ( P =0.038). Increased QuickDASH scores were independently associated with presence of postoperative complications. QuickDASH score could be expected to increase by 12.5 points in those with major complications ( P =0.044). CONCLUSION: We present the largest single cohort of pediatric open Monteggia fracture-dislocation injuries to date. These injuries are predictive of poorer outcomes including trend toward increased time to union, increased risk of major complication, and can independently predict worse long-term patient-reported functional outcomes. LEVEL OF EVIDENCE: Level III-these data represent a retrospective comparative study of clinical and functional outcomes.

Metaphyseal Dysplasia, Spahr Type; A Case Report of Variable Expressivity in Non-Consanguineous Filipino Siblings.

Introduction: Metaphyseal dysplasia describes a heterogenous group of skeletal dysplasias with varying inheritance patterns, which preferentially demonstrate dysplastic changes within the metaphyseal region of long bones. The clinical consequences of these dysplastic changes are highly variable, but most uniformly include decreased stature, increased upper-to-lower segment proportions, genu varus, and knee pain. Metaphyseal dysplasia, Spahr type (MDST) [MIM: 250400] is a rare primary bone dysplasia that was first clinically described in 1961 in four of five siblings with moderate short stature, metaphyseal dysplasia, mild genu vara, and no biochemical signs of rickets. For many decades, MDST was a clinical diagnosis, but the underlying genetic etiology was determined to be due to biallelic pathogenic variants in matrix metalloproteinases 13 [MIM: 600108] in 2014. Clinical case reports of this disease are limited; this paper aims to present the clinical manifestations and treatment for 3 Filipino siblings with a confirmed of MDST. Case Report: Patient 1 presented at age 8 for medial ankle pain and bilateral lower extremity bowing of several years. Radiographs showed bilateral metaphyseal irregularities, and the patient underwent bilateral lateral distal femoral and proximal tibial physeal tethering at 9 years 11 months. At 16 months post tethering, she reports reduced pain although varus deformity persists. Patient 2 presented to clinic at age 6 for concern of bilateral bowing. He has had no reported pain and demonstrates milder metaphyseal irregularities than patient 1 on radiographs. To date, patient 2 has no significant changes or gross deformity. Patient 3 examined at 19 months without observable deformity. Conclusion: Suspicion for MDST should be elevated in the setting of short-stature, upper-to-lower segment disproportionality, focal metaphyseal irregularities, and normal biochemical presentation. At present, no standard of care exists for treatment of patients with these deformities. Further, identification and evaluation of impacted patients are needed to progressively optimize management.

Pathologic Femur Fracture through Osteoid Osteoma after Radiofrequency Ablation: Case Report and Review of the Literature.

This is a case report of a 4-year-old girl who sustained a femoral shaft fracture 2 weeks after radiofrequency ablation of an osteoid osteoma. The fracture occurred after a relatively low-energy impact, jumping off the second to last step of a staircase. The pathologic fracture was successfully treated with closed reduction and spica casting, with full return to activities. Cases have been reported in the literature of femoral shaft fractures in older patients after radiofrequency ablation, but all are farther out than 2 weeks and none in patients as young as 4 years.

Does Postoperative Radiation Decrease Heterotopic Ossification After the Kocher-Langenbeck Approach for Acetabular Fracture?

BACKGROUND: Controversy regarding heterotopic ossification (HO) prophylaxis exists after Kocher-Langenbeck for treatment of acetabular fracture. Prophylaxis options include antiinflammatory oral medications, single-dose radiation therapy, and débridement of gluteus minimus muscle. Prior literature has suggested single-dose radiation therapy as the best prophylaxis to prevent HO formation. However, recent reports have emerged of radiation-induced sarcoma after radiotherapy for HO prophylaxis, which has led many surgeons to reconsider the risks and benefits of single-dose radiation therapy. We set out to determine if radiotherapy, in addition to standard débridement of gluteus minimus muscle, affected postoperative HO formation after a Kocher-Langenbeck approach for acetabular fracture. QUESTIONS/PURPOSES: (1) After the Kocher-Langenbeck approach and gluteus minimus débridement, is single-dose radiotherapy associated with a decreased risk of HO? (2) Does addition of single-dose radiotherapy prolong length of stay after a Kocher-Langenbeck approach and gluteus minimus débridement as compared with patients without radiotherapy? METHODS: After institutional review board approval, all adult patients treated for acetabular fracture by a single surgeon with a Kocher-Langenbeck approach between August 2011 and October 2014 were identified (n = 60). Débridement of gluteus minimus muscle caudal to the superior gluteal bundle was standard in all patients. Radiotherapy was given with a single dose of 700 cGy within 72 hours of surgery from August 2011 until April 2013. Patients treated subsequently did not receive radiotherapy. Patients treated with indomethacin (n = 1) and with fewer than 10 weeks followup were excluded (n = 12) because several studies suggest that most HO that develops is visible by that point in time. Our study group totaled 46 patients with 24 in the radiotherapy and débridement group and 22 in the débridement group. Charts were reviewed to determine length of stay. Attending orthopaedic trauma surgeons who were blinded to the patient's treatment group graded all followup radiographs according to the Brooker system, and Classes III and IV HO were considered clinically important Fisher's exact test was used to analyze clinically significant differences HO between the two groups. Length of stay was compared using a t-test. RESULTS: Single-dose radiotherapy is associated with a decreased risk of clinically important (Brooker III-IV) HO after a Kocher-Langenbeck approach and gluteus minimus débridement (radiotherapy: one of 24 [4%], no radiotherapy: seven of 22 [32%], relative risk: 0.131 [95% confidence interval {CI}, 0.018-0.981], p = 0.020). Addition of single-dose radiotherapy did not result in increased length of stay (radiotherapy: 12 ± 7.0 days; no radiotherapy: 11 ± 7.2 days; mean difference: 1.0 [95% CI, -3.2 to 5.2] days, p = 0.635). CONCLUSIONS: Single-dose radiation in combination with gluteus minimus débridement decreases the risk of clinically important HO compared with gluteus minimus débridement alone after a Kocher-Langenbeck approach for acetabular fracture. No differences in length of stay were seen. Surgeons who chose not to use radiotherapy as a result of concern for future sarcoma may see higher rates of clinically significant HO after a Kocher-Langenbeck approach for acetabular fracture fixation. LEVEL OF EVIDENCE: Level III, therapeutic study.