Intraoperative Neurological Monitoring in Lower Limb Surgery for Patients With Mucopolysaccharidoses.

BACKGROUND: There are reports of spinal cord injury (SCI) occurring after lower extremity (LE) surgery in children with mucopolysaccharidoses (MPS). Intraoperative neurological monitoring (IONM) has been adopted in some centers to assess real-time spinal cord function during these procedures. The aim of this investigation was to review 3 specialty centers' experiences with MPS patients undergoing LE surgery. We report how IONM affected care and the details of spinal cord injuries in these patients. METHODS: All pediatric MPS patients who underwent LE surgery between 2001 and 2018 were reviewed at 3 children's orthopaedic specialty centers. Demographic and surgical details were reviewed. Estimated blood loss (EBL), surgical time, positioning, use of IONM, and changes in management as a result of IONM were recorded. Details of any spinal cord injuries were examined in detail. RESULTS: During the study period, 92 patients with MPS underwent 252 LE surgeries. IONM was used in 83 of 252 (32.9%) surgeries, and intraoperative care was altered in 17 of 83 (20.5%) cases, including serial repositioning (n=7), aggressive blood pressure management (n=6), and abortion of procedures (n=8). IONM was utilized in cases with larger EBL (279 vs. 130 mL) and longer operative time (274 vs. 175 min) compared with procedures without IONM. Three patients without IONM sustained complete thoracic SCI postoperatively, all from cord infarction in the upper thoracic region. These 3 cases were characterized by long surgical time (328±41 min) and substantial EBL (533±416 mL or 30.5% of total blood volume; range, 11% to 50%). No LE surgeries accompanied by IONM experienced SCI. CONCLUSIONS: Patients with MPS undergoing LE orthopaedic surgery may be at risk for SCI, particularly if the procedures are long or are expected to have large EBL. One hypothesis for the etiology of SCI in this setting is hypoperfusion of the upper thoracic spinal cord due to prolonged intraoperative or postoperative hypotension. IONM during these procedures may mitigate the risk of SCI by identifying real-time changes in spinal cord function during surgery, inciting a change in the surgical plan. LEVEL OF EVIDENCE: Level III-retrospective comparative series.

Does Intrathecal Baclofen Therapy Increase Prevalence and/or Progression of Neuromuscular Scoliosis?

STUDY DESIGN: Retrospective, case-matched review. OBJECTIVES: Compare a group of individuals with cerebral palsy (CP) who had intrathecal baclofen (ITB) pumps to a group of individuals with CP who did not have ITB pumps in order to determine if there was a difference in the prevalence of new-onset neuromuscular scoliosis, an increased rate of progression of preexisting neuromuscular scoliosis, or an increased rate of posterior spine fusion surgery in skeletally immature individuals with CP who had ITB pumps. SUMMARY OF BACKGROUND DATA: Various authors report conflicting findings, with some reporting an increased incidence or prevalence of scoliosis in individuals with CP who have ITB pumps whereas others report no difference in the rate of scoliosis between groups. METHODS: Retrospective chart and radiographic case-matched study in which individuals were matched by gender and Gross Motor Function Classification Scale (GMFCS) level. RESULTS: We found no difference in the rates of new-onset neuromuscular scoliosis for those with CP and ITB pumps and those without ITB pumps. However, we did see a higher rate of progression as well as an increased rate of posterior spine fusion surgery in individuals with CP who had ITB pumps than for those with CP who did not have an ITB pump. CONCLUSIONS: We continue to recommend ITB pump therapy for individuals with severe spasticity associated with CP (GMFCS IV and V). There is a significant risk of complications for individuals in general. The risk of neuromuscular scoliosis is relatively high in this population. Our findings suggest that individuals with CP who have ITB pumps and who do or do not have preexisting scoliosis should be monitored closely for either developing new neuromuscular scoliosis or progression of preexisting scoliosis.

Comparative outcomes and cost-utility following surgical treatment of focal lumbar spinal stenosis compared with osteoarthritis of the hip or knee: part 2--estimated lifetime incremental cost-utility ratios.

BACKGROUND CONTEXT: Although total hip arthroplasty (THA) and total knee arthroplasty (TKA) have been widely accepted as highly cost-effective procedures, spine surgery for the treatment of degenerative conditions does not share the same perception among stakeholders. In particular, the sustainability of the outcome and cost-effectiveness following lumbar spinal stenosis (LSS) surgery compared with THA/TKA remain uncertain. PURPOSE: The purpose of the study was to estimate the lifetime incremental cost-utility ratios for decompression and decompression with fusion for focal LSS versus THA and TKA for osteoarthritis (OA) from the perspective of the provincial health insurance system (predominantly from the hospital perspective) based on long-term health status data at a median of 5 years after surgical intervention. STUDY DESIGN/SETTING: An incremental cost-utility analysis from a hospital perspective was based on a single-center, retrospective longitudinal matched cohort study of prospectively collected outcomes and retrospectively collected costs. PATIENT SAMPLE: Patients who had undergone primary one- to two-level spinal decompression with or without fusion for focal LSS were compared with a matched cohort of patients who had undergone elective THA or TKA for primary OA. OUTCOME MEASURES: Outcome measures included incremental cost-utility ratio (ICUR) ($/quality adjusted life year [QALY]) determined using perioperative costs (direct and indirect) and Short Form-6D (SF-6D) utility scores converted from the SF-36. METHODS: Patient outcomes were collected using the SF-36 survey preoperatively and annually for a minimum of 5 years. Utility was modeled over the lifetime and QALYs were determined using the median 5-year health status data. The primary outcome measure, cost per QALY gained, was calculated by estimating the mean incremental lifetime costs and QALYs for each diagnosis group after discounting costs and QALYs at 3%. Sensitivity analyses adjusting for +25% primary and revision surgery cost, +25% revision rate, upper and lower confidence interval utility score, variable inpatient rehabilitation rate for THA/TKA, and discounting at 5% were conducted to determine factors affecting the value of each type of surgery. RESULTS: At a median of 5 years (4-7 years), follow-up and revision surgery data was attained for 85%-FLSS, 80%-THA, and 75%-THA of the cohorts. The 5-year ICURs were $21,702/QALY for THA; $28,595/QALY for TKA; $12,271/QALY for spinal decompression; and $35,897/QALY for spinal decompression with fusion. The estimated lifetime ICURs using the median 5-year follow-up data were $5,682/QALY for THA; $6,489/QALY for TKA; $2,994/QALY for spinal decompression; and $10,806/QALY for spinal decompression with fusion. The overall spine (decompression alone and decompression and fusion) ICUR was $5,617/QALY. The estimated best- and worst-case lifetime ICURs varied from $1,126/QALY for the best-case (spinal decompression) to $39,323/QALY for the worst case (spinal decompression with fusion). CONCLUSION: Surgical management of primary OA of the spine, hip, and knee results in durable cost-utility ratios that are well below accepted thresholds for cost-effectiveness. Despite a significantly higher revision rate, the overall surgical management of FLSS for those who have failed medical management results in similar median 5-year and lifetime cost-utility compared with those of THA and TKA for the treatment of OA from the limited perspective of a public health insurance system.

Operative treatment of femoral neck osteochondroma through a digastric approach in a pediatric patient: a case report and review of the literature.

A digastric approach has been used successfully to treat adult patients with femoral neck osteochondromas; however, to our knowledge, this has not been described in pediatric patients with open proximal femur growth plates. A case of femoral neck osteochondroma in an 11-year-old boy is presented and treatment using a digastric approach is described. No intraoperative femoral neck fracture or postoperative avascular necrosis occurred. There is no recurrence of the tumor at the 7-year follow-up. Surgical hip dislocation through a digastric approach provides adequate exposure of the femoral neck for osteochondroma resection and this technique should be considered for such circumstances.

Differential effects of chronic ethanol exposure on cytochrome P450 2E1 and the hypothalamic-pituitary-adrenal axis in the maternal-fetal unit of the guinea pig.

BACKGROUND: Ethanol neurobehavioural teratogenicity is a leading cause of developmental mental deficiency, in which the hippocampus is a target site of injury. The multi-faceted mechanism of ethanol teratogenicity is not completely understood. This study tested the hypothesis that chronic ethanol exposure (CEE), via chronic maternal ethanol administration, increases cytochrome P450 2E1 (CYP2E1) expression and alters hypothalamic-pituitary-adrenal (HPA) axis activity in the maternal-fetal unit during the third-trimester-equivalent of gestation. METHODS: Pregnant Dunkin-Hartley-strain guinea pigs received daily oral administration of ethanol (4 g ethanol/kg maternal body weight) or isocaloric-sucrose/pair-feeding (control) throughout gestation (term, about gestational day (GD) 68). On GD 45, 55 and 65, pregnant animals were euthanized 2h after the last daily dose. Maternal and fetal body weights and fetal hippocampal brain weight were determined. Maternal and fetal samples were collected for the determination of liver CYP2E1 enzymatic activity and plasma free cortisol and ACTH concentrations. RESULTS: CEE, with maternal blood ethanol concentration of 108-124 mg/dl at 2h after the last dose, decreased fetal hippocampal weight only at GD 65 and had no effect on fetal body weight compared with control. CYP2E1 activity increased with gestational age in the fetal liver microsomal and mitochondrial fractions. CEE increased CYP2E1 activity in the microsomal and mitochondrial fractions of maternal liver at the three gestational ages and in both hepatic subcellular fractions of the GD 65 fetus compared with control. There was a gestational-age-dependent increase in maternal and fetal plasma free cortisol concentrations, but no effect of CEE compared with control. Maternal and fetal plasma ACTH concentrations were unaffected by CEE compared with control, and were virtually unchanged during the third-trimester-equivalent that was studied. CONCLUSION: These data demonstrate that, in the pregnant guinea pig, this CEE regimen increases liver CYP2E1 activity, without affecting HPA axis function, in the maternal-fetal unit during near-term gestation. The CEE-induced increase in liver CYP2E1 activity and potential oxidative stress in the maternal-fetal unit may play a role in the pathogenesis of ethanol teratogenicity.