Recent Publications by CFE Educators

INTRODUCTION

Children with cleft lip and palate (CLP) are known to have airway problems. Previous studies have shown that individuals with CLP have a 30% reduction in nasal airway size compared to non-cleft controls. No reports have been found on cross-sectional area and volume of the pharyngeal airway in clefts. Introduction of Cone-Beam CT (CBCT) and imaging software has facilitated generation of 3D images for assessment of the cross-sectional area and volume of the airway.

OBJECTIVE

To assess the pharyngeal airway in individuals with CLP using CBCT by measuring volume and smallest cross-sectional areas and compare with 19 age- and sex-matched non-cleft controls.

METHODS

Retrospective study of CBCT data of pre-adolescent individuals (N = 19, Mean age = 10.6, 7 females, 12 males, UCLP = 6, BCLP = 3) from the Center for Craniofacial Anomalies. Volumetric analysis was performed using image segmentation features in CB Works 3.0. Volume and smallest cross-sectional were studied in both groups. Seven measurements were repeated to verify reliability using Pearson correlation coefficient. Volume and cross-sectional area differences were analyzed using paired t-tests.

RESULTS

The method was found to be reliable. Individuals with CLP did not exhibit smaller total airway volume and cross sectional area than non-CLP controls.

CONCLUSION

3D imaging using CBCT and CB Works is reliable for assessing airway volume. Previous studies have shown that the nasal airway is restricted in individuals with CLP. In our study, we found that the pharyngeal airway is not compromised in these individuals.

Lung cancer mortality after exposure to radon decay products (RDP) among 16,236 male Eldorado uranium workers was analyzed. Male workers from the Beaverlodge and Port Radium uranium mines and the Port Hope radium and uranium refinery and processing facility who were first employed between 1932 and 1980 were followed up from 1950 to 1999. A total of 618 lung cancer deaths were observed. The analysis compared the results of the biologically-based two-stage clonal expansion (TSCE) model to the empirical excess risk model. The spontaneous clonal expansion rate of pre-malignant cells was reduced at older ages under the assumptions of the TSCE model. Exposure to RDP was associated with increase in the clonal expansion rate during exposure but not afterwards. The increase was stronger for lower exposure rates. A radiation-induced bystander effect could be a possible explanation for such an exposure response. Results on excess risks were compared to a linear dose-response parametric excess risk model with attained age, time since exposure and dose rate as effect modifiers. In all models the excess relative risk decreased with increasing attained age, increasing time since exposure and increasing exposure rate. Large model uncertainties were found in particular for small exposure rates.

BRAF(V600E) mutation has emerged as a marker of aggressive behavior in papillary thyroid carcinoma but its significance in microcarcinoma is not entirely clear. One-hundred and twenty-nine papillary thyroid microcarcinomas were tested for BRAF(V600E) mutation by single-strand conformation polymorphism, and their clinicopathologic features (age, sex, tumor size, multifocality, nodal metastases, histologic subtype, tumor cell morphology, architecture, tumor-associated stromal reaction, tumor interface to non-neoplastic thyroid (well circumscribed vs infiltrative), extrathyroidal extension, lymphovascular invasion, intratumoral multinucleated giant cells, and adjacent non-neoplastic thyroid pathology) were examined. Compared with tumors without the mutation (39/129, 30%), the mutated microcarcinomas (90/129, 70%) showed significantly higher prevalence of infiltrative tumor borders (78/90 vs 23/39, P=0.001), tumor-associated stromal desmoplasia/fibrosis and/or sclerosis (80/90 vs 25/39, P=0.002), classic nuclear features of papillary thyroid carcinoma (90/90 vs 35/39, P=0.008) and cystic change (43/90 vs 11/39, P=0.05). BRAF(V600E) mutation was more frequent in classic (75%), tall cell (91%), and other variants (>70%) than in follicular variant (21%) of papillary thyroid microcarcinoma. Tumors without the mutation were significantly more likely to be solid, well circumscribed, and lacked desmoplasia/fibrosis or sclerosis. However, on multivariate analysis, only the follicular variant of papillary microcarcinoma was significantly associated with the absence of mutation (odds ratio (95% confidence interval): 0.09 (0.01-0.54)). Lymph node metastases (n=24) were more frequent in microcarcinomas with mutation than without (21/24 vs 3/24, P=0.02). All patients with lateral cervical node metastasis (n=9), and all but one tumor with extrathyroidal extension (n=17/18) showed BRAF(V600E) mutation. No significant differences were noted in age, sex, tumor size, multifocality, lymphovascular invasion, psammoma bodies, stromal calcification, intratumoral multinucleated osteoclastic-type giant cells, and lymphocytic infiltration between the two groups of tumors. BRAF(V600E) mutation is an early event in thyroid carcinogenesis, and is associated with distinctive morphology and aggressive features even in papillary thyroid microcarcinomas.

BACKGROUND

Cri du chat syndrome (CCS) is a genetic disorder resulting from the deletion of the short arm of chromosome 5. Perhaps the most distinctive characteristic of this syndrome is the congenital high-pitched cry, which frequently brings these patients to the attention of an otolaryngologist. Speech and language development in children with CCS is notable for a reduced receptive vocabulary and a profound deficit in expressive language. Currently, no clear guidelines have been established for the treatment of the speech and language difficulties exhibited by these patients. In this article, we present a case report and discuss the current literature regarding the challenges to effective communication in CCS.

METHODS

Case report.

CASE

We present a 7-year-old girl with CCS who sought help to improve her ability to communicate. The patient presented with a persistent high-pitched voice unchanged since birth and a breathy dysphonia. Findings on examination were significant for an abnormally oriented larynx with atrophic vocal folds. She continues to undertake intensive speech therapy to assist in her language development.

CONCLUSION

CCS is a genetic disorder that universally results in profound deficits in expressive speech. Although patients with CCS commonly present with a high-pitched voice and marked laryngeal abnormalities, they are unlikely to benefit from surgical intervention. Speech and language therapy, including augmentative communication devices, may enhance effective communication and improve the quality of life of these patients.

OBJECTIVE

To study the effect of a neurohospitalist service on length of stay, cost, patient satisfaction, and education at an academic medical center.

METHODS

This was a retrospective cohort study using administrative data, educational surveys, and standardized patient satisfaction surveys to compare outcomes in the 21 months before (n = 343) and 27 months after (n = 436) the introduction of a neurohospitalist service in October 2006 at a single tertiary care academic medical center.

RESULTS

The most common diagnoses treated in both periods were demyelinating disease, neuromuscular disease, seizure, CNS infection, and cerebrovascular disease. Mean length of stay was reduced during the neurohospitalist period compared with that during the preneurohospitalist period (4.6 days vs 6.3 days; p 0.001), but there was no difference in median cost ($6,758 vs $7,241; p = 0.25) or in-hospital mortality (1.6 vs 1.2%; p = 0.61). After adjustment for diagnosis, admission source, and severity of illness, both length of stay (coefficient -1.85, 95% confidence interval [CI] -2.47 to -1.24) and cost (coefficient -1,558, 95% CI -2,645 to -470) were reduced during the neurohospitalist period. Thirty-day readmission rates were not different between the 2 periods in adjusted analysis. There were no differences in patient satisfaction or resident trainee satisfaction between the 2 periods, but medical student satisfaction was higher on standard educator evaluations after the neurohospitalist program was introduced.

CONCLUSIONS

The introduction of a neurohospitalist service at an academic medical center coincided with a reduction in length of stay and cost and a nonsignificant trend toward improvement in medical student satisfaction without affecting mortality, readmission rates, or patient satisfaction.

PURPOSE

Gender disparities have long existed in medicine but they have not been well examined in urology. We analyzed a large cohort of graduating urology residents to investigate gender disparities in academic productivity, as measured by peer reviewed publications and academic career choice.

MATERIALS AND METHODS

We assembled a list of urology residents who graduated from 2002 through 2008 who were affiliated with the top 50 urology hospitals, as ranked by 2009 U.S. News & World Report. PubMed® was queried to determine the publication output of each resident during the last 3 years of residency. We used an Internet search to determine the fellowship training, career choice and academic rank of each subject. Gender effects on each factor were evaluated.

RESULTS

A total of 459 male (84.5%) and 84 female (15.5%) residents were included in analysis. During residency women produced fewer total publications (average 3.0 vs 4.8, p = 0.01) and fewer as first author (average 1.8 vs 2.5, p = 0.03) than men. A higher proportion of women than men underwent fellowship training (54.8% vs 48.5%, p = 0.29) and ultimately chose an academic career (40.5% vs 33.3%, p = 0.20), although these differences were not statistically significant. Of residents who chose an academic career a higher proportion of men than women (24.7% vs 2.9%, p = 0.01) obtained associate vs assistant professor rank.

CONCLUSIONS

Women produced fewer peer reviewed publications than men during residency but they were equally likely to undergo fellowship training and choose an academic career. During the study period a higher proportion of men achieved associate professor rank.

PURPOSE

We describe the etiology, presentation, treatment and outcomes of men diagnosed with an acquired urethral diverticulum.

MATERIALS AND METHODS

We retrospectively analyzed the records of men with an acquired urethral diverticulum in an 11-year period (2000 to 2011) at a tertiary care reconstructive practice. Patient demographics, history, presentation, anatomical details such as diverticulum size and location, management and outcomes were recorded. Technical success was defined as unobstructed urination without urinary tract infection.

RESULTS

A total of 22 men with an acquired urethral diverticulum were included in analysis. Median age at presentation was 48.5 years (range 18 to 86). Most commonly, patients presented with recurrent urinary tract infection, urinary dribbling, incontinence or a weak urinary stream. Of the 22 men 12 (54.5%) underwent urethral diverticulectomy and urethroplasty, 3 (13.5%) underwent ileal conduit urinary diversion and 7 (32%) were treated nonoperatively. Select cases were managed conservatively when the urethral diverticulum was confirmed in a nonobstructed urethra, it was small or asymptomatic and it could be manually emptied after voiding. At a mean followup of 2.3 years there was a 91% urethral diverticulum recurrence-free rate.

CONCLUSIONS

Acquired male urethral diverticula are rare but should be considered when there is recurrent urinary tract infection, obstructive voiding symptoms, a history of hypospadias, urethral stricture or trauma, or prolonged urethral catheterization. Treatment options may include surgical excision of the urethral diverticulum or urinary diversion. Some patients may be adequately treated nonoperatively with post-void manual decompression.

BACKGROUND

Hurthle cell carcinoma (HCC) is an uncommon and more aggressive thyroid cancer. To date, there is a paucity of data at a population level. In this study, demographic, clinical, and pathologic characteristics of HCC were investigated and compared with other types of differentiated thyroid cancers (ODTCs). The authors also evaluated disease-specific survival and compliance with American Thyroid Association (ATA) management guidelines from 2009.

METHODS

The Surveillance, Epidemiology, and End Results (SEER) database from 1988 to 2009 was used to obtain data on patients with thyroid cancer. Data analyses were performed using chi-square tests, analysis of variance, Kaplan-Meier analysis, binary logistic regression, and Cox proportional hazards regression.

RESULTS

In total, 3311 patients with HCC and 59,585 patients with ODTC were identified. Compared with ODTC, HCC was more common among men (31.1% vs 23.0% for ODTC; P .001) and among older patients (mean age, 57.6 years vs 48.9 years for ODTC; P .001). Patients with HCC presented with higher SEER disease stage (P .001), and their tumors were larger (36.1 mm vs 20.2 mm for ODTC; P .001). Fewer patients underwent total thyroidectomy (P = .028). Both overall and disease-specific survival were lower for patients with HCC (P .001), and neither improved over the last 2 decades (P = .689). After adjustment, age ≥45 years, not undergoing surgery, and metastatic disease were strongly associated with a worse prognosis (hazard ratio >3.0). Compliance with recommended surgical treatment according to ATA guidelines was lower among patients with HCC aged ≥65 years (odds ratio [OR], 1.43; P = .002) and among unmarried patients (OR, 1.29; P = .004). Predictors of noncompliance with ATA guidelines for treatment with radioactive implants or radioisotopes were age ≥65 years (OR, 1.31; P = .017), diagnosis between 1988 and 1997, no surgery, and partial thyroidectomy (OR, 1.81, 19.48, and 4.02, respectively; P .001).

CONCLUSIONS

HCC has more aggressive behavior and compromised survival compared with ODTC. The current results indicated that it may be important to consider a different staging system or separate practice guidelines.