Cavovarus With a Twist: Midfoot Coronal and Axial Plane Rotational Deformity in Charcot-Marie-Tooth Disease

Background: The cavovarus deformity of Charcot-Marie-Tooth (CMT) disease is often characterized by a paradoxical relationship of hindfoot varus and forefoot valgus. The configuration of the midfoot, which links these deformities, is poorly understood. Accurate assessment of 3-dimensional alignment under physiologic loadbearing conditions is possible using weightbearing computed tomography (WBCT). This is the first study to examine the rotational deformity in the midfoot of CMT patients and, thus, provide key insights to successful correction of CMT cavovarus foot. Methods: A total of 27 WBCT scans from 21 CMT patients were compared to control WBCTs from 20 healthy unmatched adults. CMT patients with a history of bony surgery, severe degenerative joint disease, or open physes in the foot were excluded. Scans were analyzed using 3-dimensional software. Anatomic alignment of the tarsal bones was calculated relative to the anterior-posterior axis of the tibial plafond in the axial plane, and weightbearing surface in the coronal plane. Results: Maximal rotational deformity in CMT patients occurred at the transverse tarsal joints, averaging 61 degrees of external rotation (supination), compared to 34 degrees among controls ( P < .01). The talonavicular joint was also the site of peak adduction deformity in the midfoot, with an average talonavicular coverage angle measuring 12 degrees compared with −11 degrees in controls ( P < .01). Conclusion: This 3-dimensional WBCT analysis is the first to isolate and quantify the multiplanar rotational deformity in the midfoot of CMT patients. Compared with healthy unmatched control cases, CMT patients demonstrated increased axial plane adduction and coronal plane rotation at the talonavicular (TN) joint. These findings support performing soft tissue release at the TN joint to abduct and derotate the midfoot as a first step for targeted deformity correction. Level of Evidence: Level III, retrospective case-control study.

Neonatal osteofibrous dysplasia: Synchronous tibial and fibular involvement is common

Osteofibrous dysplasia is a rare non-malignant fibro-osseous bone tumour, first described and characterised under this name by Campanacci (1976). It is most commonly encountered in the tibia of children and young adults, but less frequently seen in the neonate with only few prior reports in the literature. We report a case of neonatal congenital osteofibrous dysplasia, presenting with unilateral limb deformity at birth. Radiographs demonstrated well-defined mixed lytic-sclerotic lesions, in a previously unreported distribution in this age-group, confined to the distal metadiaphysis of the affected tibia and fibula. Open surgery was performed for deformity correction, with tissue biopsy confirming the radiographically-suspected diagnosis. We present the up-to-date clinical, radiological, and pathological findings in this case of a rare pathology with some novel features, within this age group, in disease distribution and consequent radiographic appearances. OFD should be considered in the differential of similar congenital deforming bone lesions of the lower limb. We also review the small number of previously published cases of congenital OFD in the neonate, noting in particular that the frequency of ipsilateral fibular involvement appears to be higher than that observed in older patients.

Circular frames without x-ray – lessons learned regarding radiation exposure during tibial deformity correction from Low & Middle Income Countries?

Circular frames are a successful way of treating difficult fractures and non-unions. At our institution (CSC) in Phnom Penh, Cambodia, our method differs from developed healthcare systems in that we do not use x-ray to site the frames. A retrospective cohort study was performed between CSC and a UK LRS unit. Demographics, diagnosis, frame type, pre- and post-op deformity, proximal and distal construct alignment comparative to the tibia, and time to union or failure. 70 patients in total were identified and were randomly selected from a hospital in UK. Demographics & deformity were similar and failed to reach significant difference on testing: union rate 70% v. 82%, time to union 9.8 v. 8.5 months, and radiation exposure mean 0 v. 74 cGy/cm2 (range 6.4–326.7). These are startlingly homogenous results considering the differing resources available. We believe that ring fixators are a viable treatment method in austere environments where image intensifiers are unavailable, and demand no unnecessary radiation exposure.

Evaluation of Brace Treatment Using the Soft Brace Spinaposture: A Four-Years Follow-Up

The braces of today are constructed to correct the frontal plane deformity of idiopathic adolescent scoliosis (AIS). The Spinaposture brace© (Spinaposture Aps, Copenhagen, Denmark) is a soft-fabric brace for AIS and is designed to enhance rotational axial stability by inducing a sagittal plane kyphotic correction. This prospective observational study evaluated the brace in fifteen patients with AIS. The initial average CA was 16.8° (SD: 2.8). They were followed prospectively every 3 to 6 months during their brace usage until skeletal maturity of 25 months and at long-term follow-up of 44 months. In- and out-of-brace radiographs were performed in six subjects at inclusion. This resulted in an immediate in-brace correction of 25.3 percent in CA (14.3°→10.8°) and induced a kyphotic effect of 14.9 percent (40.8°→47.9°). The average in-brace improvement at first follow-up was 4.5° in CA, and the CA at skeletal maturity was 11° (SD: 7.4°) and long-term 12.0° (SD: 6.8°). In conclusion, the Spinaposture brace© had an immediate in-brace deformity correction and a thoracic kyphotic effect. At skeletal maturity, the deformities improved more than expected when compared to that of the natural history/observation and similar to that of other soft braces. No long-term deformity progression was seen. To substantiate these findings, stronger designed studies with additional subjects are needed.

Outcomes of Operative Treatment of Forearm Deformity in Children with Osteogenesis Imperfecta: 18 Cases

Osteogenesis imperfecta (OI) often results in recurrent fractures and/or progressive bowing of the long bones, including the arms. Upper extremity deformity has been shown to negatively impact function. The objective of this retrospective case series is to assess the ability to correct deformity, improve function and evaluate the complications and revision rates in our patients with OI who have undergone forearm deformity correction. A retrospective study, approved by The University of Nebraska Medical Center Institutional Review Board, was conducted with OI patients who underwent forearm osteotomy and fixation of one or both forearm bones between December 2011 and August 2018. There were no exclusion criteria. The electronic medical records were reviewed for patient demographics, surgical details, revisions and complications. A total of 48 procedures on 27 forearms in 18 patients were performed during the study. Surgery was performed in children with forearm deformity and recurrent fractures that were interfering with function. Half of the patients had surgery on one forearm and half had surgery on both forearms. The majority of the patients have Type III OI. There were multiple complications, the most common being wire migration which required either replacement or advancement of the wire. In conclusion, forearm deformity in OI is possible, with good healing of osteotomies and fractures, although many patients may require multiple surgical interventions.

Two-year clinical and economic burden, risk and outcomes following application of software-assisted hexapod ring fixation systems

Background Multiplanar external fixation systems that employ software-assisted deformity correction consist of rings connected by angled struts, defined as hexapod ring fixators (HRF). Costs and outcomes associated with the application of HRFs are not well documented. This study was designed to provide a nationwide baseline understanding of the clinical presentation, risks, outcomes and payer costs, and healthcare resource utilization (HCU) of patients requiring application of an HRF, from the day of, and up to 2 years, post-application. Methods Patients with HRF application (“index”) between 2007 and 2019 within the IBM Marketscan® Commercial Claims database were identified and categorized based on diagnosis: acquired deformity, arthropathy, congenital deformity, deep infection, nonunion, fracture, and other post-operative fracture sequelae. Demographics, comorbidities at index, complications post-index, HCU, and payments were analyzed. Payments were estimated using a generalized linear model and were adjusted for inflation to the 2020 consumer price index. Rates of deep infection and amputation were estimated up to 2 years post-index using Poisson regressions, and risk factors for each were estimated using logistic regression models. Results Six hundred ninety-five patients were included in our study (including 219 fractures, 168 congenital deformities, 68 deep infections, 103 acquired deformities). Comorbidities at index were significantly different across groups: less than 2% pediatrics vs 18% adults had 3 or more comorbidities, < 1% pediatric vs 29% adults had diabetes. Index payments ranged from $39,250–$75,350, with 12-months post-index payments ranging from $14,350 to $43,108. The duration of the HRF application ranged from 96 days to 174 days. Amputation was observed in patients with deep infection (8.9, 95% confidence interval (CI): 3.2–23.9%), nonunion (5.0, 95%CI: 1.6–15.4%) or fracture (2.7, 95%CI: 0.9–7.6%) at index. Complicated diabetes was the main predictor for deep infection (odds ratio (OR): 5.14, 95%CI: 2.50–10.54) and amputation (OR: 5.26, 95%CI: 1.79–15.51). Conclusions Findings from this longitudinal analysis demonstrate the significant heterogeneity in patients treated with HRF, and the wide range in treatment intensity, payments, and outcomes. Risks for deep infection and amputation were primarily linked to the presence of complicated diabetes at the time of HRF application, suggesting a need for careful management of comorbid chronic conditions in patients requiring HRF for orthopedic care.

Utility of the MISDEF2 Algorithm and Extent of Fusion in Open Adult Spinal Deformity Surgery With Minimum 2-Year Follow-up

Objective: Long-segment fusion in adult spinal deformity (ASD) is often needed, but more focal surgeries may provide significant relief with less morbidity. The minimally invasive spinal deformity surgery (MISDEF2) algorithm guides minimally invasive ASD surgery, but it may be useful in open ASD surgery. We classified ASD patients undergoing focal decompression, limited decompression and fusion, and full correction according to MISDEF2 and correlated outcomes.Methods: A retrospective study of ASD patients treated by 2 surgeons at our hospital was performed. Inclusion criteria were: age > 50, minimum 2-year follow-up, and open ASD surgery. Tumor, trauma, and infections were excluded. Patients had open surgery including focal decompression, short segment fusion, or full scoliosis correction. All patients were categorized by MISDEF2 into 4 classes based upon spinopelvic parameters. Perioperative metrics were assessed. Radiographic correction, complications and reoperation were recorded.Results: A total of 136 patients met inclusion criteria. Mean follow-up was 46 ± 15.8 months (range, 24–118 months). Forty-seven underwent full deformity correction, 71 underwent short segment fusion, and 18 underwent decompression alone. There were 24 cases of class I, 66 cases of class II, 23 cases of class III, and 23 cases of class IV patients. Patients in class I and II had perioperative complication rates of 0% and 16.7% and revision rates of 8% and 21.2% when undergoing focal decompression or limited fusion. However, class II patients undergoing full correction had higher perioperative complications rate (p = 0.03) and revision surgery rates (p = 0.047). This difference was not seen in class III patients (p > 0.05). All class IV patients underwent full correction, but they had higher perioperative complication rates (p < 0.019), comparable revision surgery rates (p = 0.27), and better radiographic realignment (p < 0.001). In addition, full deformity correction was associated with longer length of stay, increased blood loss, and longer operative time (p < 0.001).Conclusion: The MISDEF2 algorithm may help guide ASD surgical decision making even in open surgery, with focal treatment used in class I and II patients as a viable alternative and full correction implemented in class IV patients because of severe malalignment. However, class II patients with ASD undergoing full deformity correction do have higher complication rates.

Perioperative Predictive Factors for Positive Outcomes in Spine Fusion for Adult Deformity Correction

Purpose: Identifying perioperative factors that may influence the outcomes of long spine fusion for the treatment of adult deformity is key for tailored surgical planning and targeted informed consent. The aim of this study was to analyze the association between demographic or perioperative factors and clinical outcomes 2 years after long spine fusion for the treatment of adult deformity. Methods: This study is a multivariate analysis of retrospectively collected data. All patients who underwent long fusion of the lumbar spine for adult spinal deformity (January 2016–June 2019) were included. The outcomes of interest were the Oswestry disability index (ODI), visual analogic scale (VAS) preoperatively and at 1 and 2 years’ follow up, age, body mass index, American Society of Anaesthesiologists (ASA) score, upper and lowest instrumented vertebrae (UIV and LIV, respectively), length of surgery, estimated blood loss, and length of hospital stay. Results: Data from 192 patients were available. The ODI at 2 years correlated weakly to moderately with age (r = 0.4), BMI (r = 0.2), ASA (r = 0.3), and LIV (r = 0.2), and strongly with preoperative ODI (r = 0.6). The leg VAS at 2 years moderately correlated with age (r = 0.3) and BMI (r = 0.3). Conclusion: ODI and VAS at 2 years’ follow-up had no to little association to preoperative age, health status, LIV, or other peroperative data, but showed a strong correlation with preoperative ODI and pain level.