Aortic dissection associated with cogans's syndrome: deleterious loss of vascular structural integrity is associated with GM-CSF overstimulation in macrophages and smooth muscle cells

<p>Abstract</p> <p>Background</p> <p>Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease and vestibuloauditory symptoms. Systemic vasculitis is found in about 10% of cases.</p> <p>Case presentation</p> <p>A 46-year-old female with Cogans's syndrome and a history of arterial hypertension presented with severe chest pain caused by an aneurysm of the ascending aorta with a dissection membrane located a few centimeters distal from the aortic root. After surgery, histopathological analysis revealed that vascular matrix integrity and expression of the major matrix molecules was characterized by elastolysis and collagenolysis and thus a dramatic loss of structural integrity. Remarkably, exceeding matrix deterioration was associated with massively increased levels of granulocyte macrophage colony stimulating factor (GM-CSF).</p> <p>Conclusion</p> <p>Our data suggest that the persistently increased secretion of the inflammatory mediator GM-CSF by resident inflammatory cells but also by SMC may be the trigger of aortic wall structural deterioration.</p

Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener’s granulomatosis

Wegener’s granulomatosis (WG) frequently involves the subglottis and trachea and may compromise the upper airway. The objective of this study is to evaluate retrospectively the effect of treatment of subglottic stenosis (SGS) and tracheal stenosis (TS) by dilatation tracheoscopy (DT) in patients with WG. We performed a cohort study on all patients who underwent DT between February 2001 and September 2005 in our institution. From this cohort we identified a total of nine WG patients. In all patients, clinical, serological and histopathological data had been prospectively collected by a standardized protocol from the time point of diagnosis. In the nine patients that were identified with SGS or TS due to WG (eight women and one man), a total of 22 DT’s were performed. Two patients needed a tracheostoma (one temporarily). The mean follow-up after the first DT was 25.4 ± 14.1 months. Two patients did not experience a recurrence of SGS or TS. Six patients required a second DT without recurrence of local disease. The remaining patient underwent 8 DT's in a 4-year period. DT can offer a simple and repeatable solution to SGS and TS due to WG. Seven of the nine patients required more than one dilatation and some patients experience a functional restriction. One patient has a definitive tracheostoma

Early Loading After Single-Stage Bone-Anchored Implantation in Adults

Classically, processor loading after single-stage bone-anchored implantation (BAI) surgery follows a 3-month osseointegration period. The purpose of this study was to examine audiometric outcomes and postoperative complications in adult patients undergoing single-stage BAI with processor loading at less than 6 weeks postoperatively. Retrospective review. Otology clinic in a tertiary care academic center. A retrospective review was performed of all adult patients (>18 years) undergoing BAI from 2007 to 2010. Sixty-four patients met inclusion criteria. Fifty-five patients had unilateral hearing loss, including single-sided deafness, conductive hearing loss, or mixed hearing loss. Nine patients had bilateral hearing loss. Patients were divided into groups based on time to processor loading (>12 weeks, <12 weeks, <6 weeks). All patients were loaded with the external processor at less than 6 weeks when possible. Preoperative and postoperative audiometric evaluations were performed. There were no cases of osseointegration failure. All groups showed significant improvement in audiometric testing using their BAI (P .05). Major skin complications were seen in 9% of subjects and minor complications in 30%. Single-stage BAI implantation with early processor loading is safe and effective in adults. All groups demonstrated significant audiometric benefit that was not affected in patients loaded early. Major and minor skin-site complications frequently delayed processor loading, but there were no cases of osseointegration failure in any group

Self-crimping superelastic nitinol prosthesis and malleostapedotomy: a temporal bone study.

OBJECTIVE: The aim of this study was to compare the results of application of 2 types of superelastic nitinol prostheses. STUDY DESIGN: Temporal bones study with planned data collection. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Malleostapedotomy was performed in 15 human temporal bones implanting 3 different prostheses: manually crimping polytetrafluoroethylene (MC-PTFE) piston, nitinol self-crimping polytetrafluoroethylene (SC-PTFE) piston, and a modified nitinol self-crimping polytetrafluoroethylene (mSC-PTFE) piston. The first 2 have a diameter of 0.4 mm and length of 7 mm, whereas the mSC-PTFE piston has a diameter of 0.4 mm but a length of 7.75 mm. We evaluated various parameters of prosthesis attachment-that is, the time for implantation of SC and mSC nitinol loop pistons and the MC platinum loop piston, the quality of attachment of the prostheses to the malleus, their positions with respect to the center of the stapes footplate, and the protrusion of the piston into the vestibule. RESULTS: The mSC-PTFE superelastic nitinol prosthesis showed a statistically significant difference in mean operation time (mSC vs SC, P < .0001; SC vs MC, P < .0001; mSC vs MC, P < .0001). The protrusion of the piston into the vestibule was highly reproducible in all 3 prostheses. CONCLUSION: Because of its greater length, the mSC-PTFE allows for management of the most varied anatomical conditions. At the same time, its self-crimping nature prevents the risk of distortion of the prosthesis by the crimping process and reduces the operation time in combination with standardized bending of the prosthesis shaft