Ollier disease (OD) and Maffucci syndrome (MS) are characterized by multiple enchondromas. Patients with MS also have benign vascular overgrowths that become malignant in 8.5% of cases. OD is characterized by multiple enchondromas, typically unilateral in distribution with a predilection for the appendicular skeleton. MS is characterized by multiple enchondromas bilaterally distributed in most of the cases. Both disorders feature multiple swellings on the extremity, deformity around the joints, limitations in joint mobility, scoliosis, bone shortening, leg-length discrepancy, gait disturbances, pain, loss of function, and pathological fractures. About 50% of patients with OD or MS develop a malignancy, such as chondrosarcoma, glioma, and ovarian juvenile granulosa cell tumor. To better understand the natural history of OD and MS, we reviewed 287 papers describing patients with OD and MS. We also created a survey that was distributed directly to 162 patients through Facebook. Here, we compare the review of the cases described in the literature to the survey's responses.The review of the literature showed that: the patients with OD are diagnosed at a younger age; the prevalence of chondrosarcomas among patients with OD or MS was~30%; in four patients, vascular anomalies were identified in internal organs only; and, the prevalence of cancer among patients with OD or MS was~50%. With these data, health care providers will better understand the natural history, severity, and prognosis of these diseases and the prevalence of malignancies in these patients.Here, we recommend new guidelines for the care of patients with OD and MS.

Introduction: The goals of orthopaedic treatment for most patients with osseous metastases are to control pain, maintain function, and maximize quality of life and time at home. The aim of this study was to determine differences in 30-day postoperative morbidity and mortality between patients who underwent prophylactic versus postfracture stabilization for metastatic lesions of long bones. Methods: The American College of Surgeons National Surgical Quality Improvement Program database was queried for patients who underwent prophylactic fixation (n = 461) or postfracture stabilization (n = 856) for pathologic fractures because of metastatic lesions of long bones from 2006 to 2016. The groups were compared with respect to several potential confounders using Student t, Kruskal-Wallis, and χ2 tests. Logistic and Poisson regression models (inclusion threshold of P < 0.1) were used to assess the associations of functional status with outcomes. The alpha level was set at 0.05. Results: Prophylactic fixation was associated with a lower risk of major medical complications (odds ratio = 0.64; 95% confidence interval [CI], 0.45 to 0.93; P = 0.02), discharge to a care facility rather than home (odds ratio = 0.48; 95% CI, 0.36 to 0.63; P < 0.01), and lower risk of a longer hospital stay (incidence risk ratio = 0.86; 95% CI, 0.74 to 0.96; P = 0.01) compared with postfracture stabilization. No significant difference was found in the risk of unplanned revision surgery or 30-day postoperative mortality between the two groups. Conclusion: Although prevention of pathologic fractures caused by metastatic disease may not always be possible, patients who underwent prophylactic stabilization had a lower risk of major complications within 30 days postoperatively and shorter hospital stays compared with patients who underwent postfracture stabilization. Level of Evidence: Level IV, retrospective cohort

Plasma deoxy-sphingoid bases are elevated in type 2 diabetes patients and correlate with the stage of diabetic distal sensorimotor polyneuropathy; however, associations between deoxy-sphingolipids (DSL) and neuropathy in type 1 diabetes have not been examined. The primary aim of this exploratory pilot study was to assess the associations between multiple sphingolipid species including DSL and free amino acids and the presence of symptomatic neuropathy in a DCCT/EDIC type 1 diabetes subcohort. Using mass spectroscopy, plasma levels of DSL and free amino acids in DCCT/EDIC type 1 diabetes participants (n = 80), with and without symptoms of neuropathy, were investigated. Patient-determined neuropathy was based on 15-item self-administered questionnaire (Michigan Neuropathy Screening Instrument) developed to assess distal symmetrical peripheral neuropathy in diabetes. Patients who scored ≥4, or reported inability to sense their feet during walking or to distinguish hot from cold water while bathing were considered neuropathic. Plasma levels of ceramide, sphingomyelin, hexosyl- and lactosylceramide species, and amino acids were measured and analyzed relative to neuropathy status in the patient. Deoxy-C24-ceramide, C24- and C26-ceramide were higher in patients with neuropathy than those without neuropathy. Cysteine was higher in patients with neuropathy. No differences in other sphingolipids or amino acids were detected. The covariate-adjusted Odds Ratios of positive patient-reported neuropathy was associated with increased levels of deoxy-C24-, and deoxy-C24:1-ceramide; C22-, C24-, and C26-ceramide; and cysteine. Plasma deoxy-ceramide and ceramide species may have potential diagnostic and prognostic significance in diabetic neuropathy.

Background Reconstructive surgery is often required following lower extremity soft tissue sarcoma resection. The relationship between the method of plastic surgery reconstruction and postoperative wound healing or functional oncologic outcomes in this patient population is poorly understood. Methods We performed a retrospective review on all adult patients that underwent soft tissue reconstruction following resection of lower extremity soft tissue sarcomas between 1996 and 2016 at our institution. Results One hundred and thirty‐six patients were identified. Wound complications occurred within 6‐months postoperatively in 72 patients (52.9%). Average time to heal was 13.0 weeks. Limb survival was 94.9%. 16.9% and 36.8% of patients had evidence of local recurrence or metastatic disease, respectively. There was no significant difference in the incidence of overall wound complications, time to heal, limb survival, local recurrence, or metastatic disease between the different reconstructive methods. Conclusions In our cohort, the utilization of different reconstructive techniques did not correlate with a difference in postoperative wound complications or oncologic outcomes. Local flaps can effectively reconstruct the majority of lower extremity sarcoma defects that cannot be closed primarily. However, alternative reconstructive techniques may be utilized when indicated without a significant increase in postoperative morbidity in this patient population.