Extended sternotomy with lateral neck incision: An alternative approach for children with large apical chest masses with thoracic inlet involvement

Pediatric tumors in the apex of the thoracic cavity are often diagnosed late due to the absence of symptoms. These tumors can be quite large at presentation with involvement of the chest wall, sympathetic chain, spine, and aortic arch. The tumors can also extend into the thoracic inlet and encircle the brachial plexus. Depending on the diagnosis, treatment may involve chemotherapy with subsequent surgery or require primary resection. Optimal exposure to resect large apical tumors with thoracic inlet extension is a surgical challenge. To date, several surgical techniques have been described to resect these tumors – including both anterior and posterior thoracic approaches. Each of these techniques can be limited by inadequate exposure of the mass. We describe an alternative approach to surgical resection of these masses that employs an extended sternotomy with a lateral neck incision. This report details two successful resections of large left apical masses with thoracic inlet involvement in children using this technique (Level of evidence 4).     

Timing of parathyroidectomy for tertiary hyperparathyroidism with end-stage renal disease: A cost-effectiveness analysis

BackgroundTertiary hyperparathyroidism associated with end-stage renal disease is characterized by progression from secondary hyperparathyroidism to an autonomous overproduction of parathyroid hormone that leads to adverse health outcomes. Rates of parathyroidectomy (PTX) have decreased with the use of calcimimetics. Optimal timing of PTX in relation to kidney transplant remains controversial. We aimed to identify the most cost-effective strategy for patients with tertiary hyperparathyroidism undergoing kidney transplant.MethodsWe constructed a patient level state transition microsimulation to compare 3 management schemes: cinacalcet with kidney transplant, cinacalcet with PTX before kidney transplant, or cinacalcet with PTX after kidney transplant. Our base case was a 55-year-old on dialysis with tertiary hyperparathyroidism awaiting kidney transplant. Outcomes, including quality-adjusted life years, surgical complications, and mortality, were extracted from the literature, and costs were estimated using Medicare reimbursement data.ResultsOur base case analysis demonstrated that cinacalcet with PTX before kidney transplant was dominant, with a lesser cost of $399,287 and greater quality-adjusted life years of 10.3 vs $497,813 for cinacalcet with PTX after kidney transplant (quality-adjusted life years 9.4) and $643,929 for cinacalcet with kidney transplant (quality-adjusted life years 7.4).ConclusionCinacalcet alone with kidney transplant is the least cost-effective strategy. Patients with end-stage renal disease-related tertiary hyperparathyroidism should be referred for PTX, and it is most cost-effective if performed prior to kidney transplant.     

Response of the ENPP1-Deficient Skeletal Phenotype to Oral Phosphate Supplementation and/or Enzyme Replacement Therapy: Comparative Studies in Humans and Mice

Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1^asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1^asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1^asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1^asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR).     

Representative dynamic ranges of spinal alignment during gait in patients with mild and severe adult spinal deformities

BACKGROUND CONTEXTSurgical correction strategies for adult spinal deformity (ASD) relies heavily on radiographic alignment goals, however, there is often debate regarding degree of correction and how static alignment translates to physical ability in daily life. Kinematic analysis has the potential to improve the concept of ideal spinal alignment by providing clinically meaningful estimates of dynamic changes in spinal alignment during activities of daily life.PURPOSEEstimate representative dynamic ranges of spinal alignment during gait among ASD patients using 3D motion tracking; compare dynamic alignment between mild and severe deformity patients and to healthy adults.STUDY DESIGN/SETTINGRetrospective review at a single institution.PATIENT SAMPLEFifty-two ASD patients and 46 healthy adults.OUTCOME MEASURESRadiographic alignment, kinematic spine motion, spatiotemporal gait measures, patient reported outcomes (VAS pain, ODI, SRS-22r).METHODSSpinal alignment was assessed radiographically and during standing and overground walking tests. Dynamic alignment was initialized by linking radiographic alignment to kinematic alignment during standing and at initial heel contact during gait. Dynamic changes in maximums and minimums during gait were made relative to initial heel contact for each gait cycle. Total range-of-motion (RoM) was measured for both ASD and healthy subjects. Dynamic alignment measures included coronal and sagittal vertical axes (CVA, SVA), T1 pelvic angle (TPA), lumbar lordosis (LL), and pelvic tilt (PT). ASD patient's deformities were classified as either Mild or Severe based on the SRS-Schwab ASD classification.RESULTSSevere ASD patients had significantly larger dynamic maximum and minimums for SVA, TPA, LL, and PT (all p<.05) compared with Mild ASD patients. ASD patients exhibited little difference in dynamic alignment compared with healthy subjects. Only PT had a significant difference in dynamic RoM compared with healthy (p<.001).CONCLUSIONSMild and Severe ASD patients exhibited similar global dynamic alignment measures during gait and had comparable RoM to healthy subjects except with greater PT and reduced spatiotemporal performance which may be key compensatory mechanisms for dynamic stabilization.