Recurrent angioedema: Experience at a tertiary care urban medical center.

OBJECTIVE: To determine the demographics, presentation, management, and outcomes of patients with recurrent angioedema. To compare the findings to patients with ACE inhibitor related angioedema. METHODS: Retrospective case series with chart review of patients who presented to a tertiary-care hospital between January 2010 and December 2017 with two or more episodes of angioedema. Excluded were patients with anaphylactic reaction, medication induced angioedema, or angioedema secondary to an infectious etiology. A group of 88 patients who presented during the same time period with ACE inhibitor related angioedema was used as a control. Statistical analysis was conducted using a two-tailed Fisher exact test and a multivariate logistical regression model to determine significant associations. RESULTS: Ninety-one patients were identified; 61 met the selection criteria and had 217 total episodes of angioedema episodes presenting to the emergency department. Fifty percent were Caucasian or Hispanic. The average number of episodes was 3.5 (range: 2-23). The lips and tongue were the most commonly affected sites (37% and 39%). The larynx and floor of mouth were least likely to be involved (7% and 6%). Only 1 patient was found to have C1 esterase inhibitor deficiency. Twenty-eight percent of patients had asthma, allergic rhinitis, food allergies, or atopic dermatitis. Only 11% of episodes required airway intervention. No patients required airway intervention after admission. CONCLUSION: Recurrent angioedema was primarily idiopathic, was less severe than ACE inhibitor angioedema, and was associated with an atopic history. There was less frequent worsening of symptoms after admission, and recurrences occurred more frequently are at the same anatomic subsite. LEVEL OF EVIDENCE: IV.

Why Central Neck Dissection Works (and Fails) for Recurrent Thyroglossal Duct Remnants.

OBJECTIVE: To review the patient characteristics and outcomes for children and undergoing central neck dissection for control of recurrent thyroglossal duct cysts and fistula following prior Sistrunk procedures and children requiring surgery for refractory infection. METHODS: We performed a computerized review of all children who were evaluated for thyroglossal duct cysts during the years 1999-2018 by a single surgeon operating at an urban children's hospital and an outpatient surgical center. Those requiring a central neck dissection for control of recurrent disease or intractable infection were identified. Age at time of surgery, sex, surgical procedure, and postoperative complications were recorded. These data were combined with similar data from a published report by the same surgeon in the years 1990-1998 to complete a 28-year review. RESULTS: 18 central neck dissections were performed including 13 for recurrent thyroglossal duct remnants after Sistrunk procedures and 5 primary surgeries for intractable infection. Ages ranged from 3 to 19 years (median = 10 years) and 13 of 18 were girls (72%). Four children had their first Sistrunk surgery performed by the senior author. Three children operated elsewhere had intact hyoid bones at the time of revision surgery, suggesting less-than-Sistrunk primary surgeries. Central neck dissection controlled disease in the lower neck in all cases. One child re-fistulized at the level of the hyoid. CONCLUSIONS: Central neck dissection in combination with a Sistrunk-type dissection of the tongue base is effective in the control of recurrent infection following unsuccessful Sistrunk surgery and aids in dissection for children with intractable infection. Although this technique reliably controls infrahyoid disease and improves access to the hyoid and posterior hyoid space, it does nothing to address the difficulties of following the thyroglossal tract into the tongue base.

Air Pollution and Angioedema.

OBJECTIVE: To identify environmental factors that may precipitate angioedema. STUDY DESIGN: Case series with chart review. SETTING: An urban tertiary care medical center. SUBJECTS AND METHODS: After institutional review board approval was obtained, a chart review of all patients who presented to Temple University Hospital with angioedema from January 2012 to December 2014 was performed. Patient demographics and hospital course were gathered. Environmental data on the dates of presentation, including precipitation, humidity, and air pollution, were obtained from regional Environmental Protection Agency online data banks and used for statistical analysis. RESULTS: In total, 408 patients, representing 450 episodes of angioedema, met the inclusion criteria for the study. Most patients were female (58%), African American (74%), and on an angiotensin-converting enzyme inhibitor (ACE-I) upon presentation (61%). Days with unhealthy levels of air pollution were associated with an increased likelihood of angioedema presentation (odds ratio [OR], 1.82; confidence interval [CI], 1.01-3.30; P = .046). Subgroup analysis revealed that elevated ground-level ozone was the primary air pollutant significantly associated with angioedema presentations (OR, 4.95; CI 1.92-12.76; P = .0009). Higher ground-level ozone was also associated with ACE-I angioedema presentations (P = .017) but not with non-ACE-I angioedema presentations (P = .86). Air quality was not predictive of angioedema severity or need for intubation. CONCLUSION: Angioedema is a complex, multifactorial disease resulting in potentially life-threatening complications. This is the first study to demonstrate that higher levels of air pollution, specifically ground-level ozone, are associated with significantly increased rates of angioedema episodes, although not severity.

Spectroscopic Analysis of Human Tracheal Tissue during Decellularization.

OBJECTIVE: To use mid-infrared (IR) spectroscopy to assess changes in the cartilaginous framework of human trachea during decellularization. STUDY DESIGN: Laboratory-based study. SETTING: Research laboratory. METHODS: Six cadaveric human tracheas were decellularized using a detergent enzymatic method (DEM). Tissue samples were obtained from each specimen after 0, 1, 10, and 25 DEM cycles for histologic and spectroscopic analysis. Decellularization was confirmed using hematoxylin and eosin (H&E) and 2-(4-amidinophenyl)-1H-indole-6-carboxamidine (DAPI) staining. Changes in cartilaginous framework were examined using Fourier transform infrared imaging spectroscopy (FT-IRIS) and an attenuated total reflectance (ATR) probe in the mid-IR frequencies. Results were statistically analyzed using 1-way analysis of variance (ANOVA) and principal component analysis (PCA). RESULTS: Six decellularized tracheal scaffolds were successfully created using a DEM protocol. Histologic examination showed near-complete nuclear loss following 25 DEM cycles. As observed with FT-IRIS analysis, the collagen absorbance signal (1336 cm-1) was predominantly in the perichondria and remained stable after 25 DEM cycles ( P = .132), while the absorbance from sugar rings in proteoglycans and nucleic acids in hyaline cartilage (1080 cm-1) showed a significant decrease after 1 DEM cycle ( P = .0007). Examination of the luminal surface of the trachea with an ATR probe showed raw mid-IR spectra consistent with cartilage. PCA showed significant separation of spectra corresponding to treatment cycle along the principal components 1 and 2. CONCLUSION: Mid-IR spectroscopy is a viable method of monitoring changes in extracellular matrix components during the decellularization of human trachea.

Compositional Assessment of Human Tracheal Cartilage by Infrared Spectroscopy.

Objectives To assess the potential of infrared fiber-optic spectroscopy to evaluate the compositional properties of human tracheal cartilage. Study Design Laboratory-based study. Methods Twenty human cadaveric distal tracheas were harvested (age range 20-78 years; 6 females, 14 males) for compositional analysis. Histologic staining, Fourier transform infrared imaging spectroscopy data on collagen and proteoglycan (PG) content, and near-infrared (NIR) fiber-optic probe spectroscopic data that reflect protein and water content were evaluated. NIR fiber-optic probe data were also obtained from the proximal trachea in 4 human cadavers (age range 51-65 years; 2 females, 2 males) in situ for comparison to distal trachea spectral data. Results In the distal trachea cohort, the spectroscopic-determined ratio of PG/amide I, indicative of the relative amount of PG, was significantly higher in the tissues from the younger group compared to the older group (0.37 ± 0.08 vs 0.32 ± 0.05, P = .05). A principal component analysis of the NIR spectral data enabled separation of spectra based on tracheal location, likely due to differences in both protein and water content. The NIR-determined water content based on the 5200-cm-1 peak was significantly higher in the distal trachea compared to the proximal trachea ( P < .001). Conclusions Establishment of normative compositional values and further elucidating differences between the segments of trachea will enable more directed research toward appropriate compositional end points in regenerative medicine for tracheal repair.

Endoscope-assisted repair of CSF otorrhea and temporal lobe encephaloceles via keyhole craniotomy.

OBJECTIVE Temporal lobe encephaloceles and cerebrospinal fluid otorrhea from temporal bone defects that involve the tegmen tympani and mastoideum are generally repaired using middle fossa craniotomy, mastoidectomy, or combined approaches. Standard middle fossa craniotomy exposes patients to dural retraction, which can lead to postoperative neurological complications. Endoscopic and minimally invasive techniques have been used in other surgeries to minimize brain retraction, and so these methods were applied to repair the lateral skull base. The goal of this study was to determine if the use of endoscopic visualization through a middle fossa keyhole craniotomy could effectively repair tegmen defects. METHODS The authors conducted a retrospective review of 6 cases of endoscope-assisted middle fossa repairs of tegmen dehiscences at a tertiary care medical center within an 18-month period. RESULTS All cases were successfully treated using a keyhole craniotomy with endoscopic visualization and minimal retraction. Surgical times did not increase. There were no major postoperative complications, recurrences of encephaloceles, or cerebrospinal fluid otorrhea in these patients. CONCLUSIONS Endoscopic visualization allows for smaller incisions and craniotomies and less risk of brain retraction injury without compromising repair integrity during temporal encephalocele and tegmen repairs.

Risk Factors and Comorbidities in Chronic Rhinosinusitis.

Chronic rhinosinusitis (CRS) is a heterogeneous disorder that creates a significant burden on the healthcare system. It is caused by a combination of inflammatory, environmental, and host factors; however, the precise mechanism of how each factor leads to CRS continues to be a source of debate. Previous data regarding this topic is often inconsistent or of lower quality. In this article, we review the recent literature on the risk factors and comorbidities in CRS. Large population-based studies have helped establish smoking as a significant risk factor for CRS. The focus has now shifted towards smoking and its effect on long-term outcomes after endoscopic sinus surgery (ESS). Ciliary dyskinesia, both primary and secondary, can affect both the sinonasal cavity and lower airways simultaneously by decreasing the beat frequency of cilia and inducing mucostasis. The effects of secondary dyskinesia may be reversible and there is some evidence to suggest the use of topical mucolytics in patients with CRS. Allergy and variants of sinonasal anatomy have been hypothesized to increase the risk of developing CRS by inducing chronic inflammation and obstructing the sinus ostia. Nevertheless, emerging data regarding these topics continue to produce inconclusive results. Inflammation of the upper and lower airways can occur simultaneously as seen in patients with asthma and aspirin sensitivity. The connection between these pro-inflammatory disease states has been known for many years. Newer evidence include large population-based studies and studies that correlate objective tests, such as computer tomography scans to pulmonary function tests. However, the treatment of CRS and its effects on obstructive airway disease continues to be a topic of debate. More large prospective studies are needed in order to continue refining our knowledge of the disease processes in CRS.

Somatosensory-evoked potential monitoring during instrumented scoliosis corrective procedures: validity revisited.

BACKGROUND: Intraoperative monitoring (IOM) using somatosensory-evoked potentials (SSEPs) plays an important role in reducing iatrogenic neurologic deficits during corrective pediatric idiopathic procedures for scoliosis. However, for unknown reasons, recent reports have cited that the sensitivity of SSEPs to detect neurologic deficits has decreased, in some to be less than 50%. This current trend, which is coincident with the addition of transcranial motor-evoked potentials, is surprising given that SSEPs are robust, reproducible responses that were previously shown to have sensitivity and specificity of >90%. PURPOSE: Our primary aim was to assess whether SSEPs alone can detect impending neurologic deficits with similar sensitivity and specificity as originally reported. Our secondary aim was to estimate the potential predictive value of adding transcranial motor-evoked potentials to SSEP monitoring in idiopathic scoliosis procedures. DESIGN: This was a retrospective review to analyze the efficacy of SSEP monitoring in the group of pediatric instrumented scoliosis fusion cases. PATIENT SAMPLE: We retrospectively reviewed all consecutive cases of patients who underwent idiopathic scoliosis surgery between 1999 and 2009 at Children's Hospital of Pittsburgh. We identified 477 patients who had the surgery with SSEP monitoring alone. Exclusion criteria included any patients with neuromuscular disorders or unreliable SSEP monitoring. Patients who had incomplete neurophysiology data or incomplete postoperative records were also excluded. OUTCOME MEASURES: Major outcomes measured were clinically significant postoperative sensory or motor deficits, as well as significant intraoperative SSEP changes. METHODS: Continuous interleaved upper- and lower-extremity SSEPs were obtained throughout the duration of all procedures. We considered a persistent 50% reduction in primary somatosensory cortical amplitude or a prolongation of response latency by >10% from baseline to be significant. Persistent changes represent significant deviation in SSEP amplitude or latency in more than two consecutive averaged trials. Patients were classified into one of four categories with respect to SSEP monitoring: true positive, false positive, true negative, and false negative. The sensitivity, specificity, positive predictive value, and negative predictive value were then calculated accordingly. RESULTS: Our review of 477 idiopathic scoliosis surgeries monitored using SSEPs alone revealed a new deficit rate of 0.63% with no cases of permanent injury. Sensitivity = 95.0%, specificity = 99.8%, positive predictive value = 95%, negative predictive value = 99.8%. Using evidence-based epidemiologic measures, we calculated that the number needed to treat was 1,587 patients for one intervention to be performed that would have been missed by SSEP monitoring alone. In addition, the number needed to harm, which represents the increase in false positives with the addition of transcranial electrical motor-evoked potentials, was 200. CONCLUSION: SSEP monitoring alone during idiopathic scoliosis continues to be a highly reliable method for the detection and prevention of iatrogenic injury. Our results confirm the high sensitivity and specificity of SSEP monitoring alone published in earlier literature. As such, we suggest the continued use of SSEP alone in idiopathic scoliosis surgeries. At this time we do not believe there are sufficient data to support the addition of MEP monitoring, although more studies and revised criteria for the use of MEP may provide added value for its use in the future.