Recent Publications by CFE Educators

INTRODUCTION

Ampullary adenocarcinoma (AAC) is a rare malignancy with great morphological heterogeneity, which complicates the prediction of survival and, therefore, clinical decision-making. The aim of this study was to develop and externally validate a prediction model for survival after resection of AAC.

MATERIALS AND METHODS

An international multicenter cohort study was conducted, including patients who underwent pancreatoduodenectomy for AAC (2006-2017) from 27 centers in 10 countries spanning three continents. A derivation and validation cohort were separately collected. Predictors were selected from the derivation cohort using a LASSO Cox proportional hazards model. A nomogram was created based on shrunk coefficients. Model performance was assessed in the derivation cohort and subsequently in the validation cohort, by calibration plots and Uno's C-statistic. Four risk groups were created based on quartiles of the nomogram score.

RESULTS

Overall, 1007 patients were available for development of the model. Predictors in the final Cox model included age, resection margin, tumor differentiation, pathological T stage and N stage (8th AJCC edition). Internal cross-validation demonstrated a C-statistic of 0.75 (95% CI 0.73-0.77). External validation in a cohort of 462 patients demonstrated a C-statistic of 0.77 (95% CI 0.73-0.81). A nomogram for the prediction of 3- and 5-year survival was created. The four risk groups showed significantly different 5-year survival rates (81%, 57%, 22% and 14%, p  0.001). Only in the very-high risk group was adjuvant chemotherapy associated with an improved overall survival.

CONCLUSION

A prediction model for survival after curative resection of AAC was developed and externally validated. The model is easily available online via www.pancreascalculator.com.

PURPOSE

The self-rated health (SRH) item is frequently used in health surveys but variations of its form (wording, response options) may hinder comparisons between versions over time or across surveys. The objectives were to determine (a) whether three SRH forms are equivalent, (b) the form with the best construct validity and (c) the best coding scheme to maximize equivalence across forms.

METHODS

We used data from 58,023 respondents of the Swiss Health Survey. Three SRH forms were used. Response options varied across forms and we explored four coding schemes (two considering SRH as continuous, two as dichotomous). Construct validity of the SRH was assessed using 34 health predictors to estimate the explained variance.

RESULTS

Distributions of response options were similar across SRH forms, except for the "good" and "very good" options ("good" in form 1: 58.6%, form 2: 65.0% and form 3: 44.1%). Explained variances differed across SRH forms, with form 3 providing the best overall explained variance, regardless of coding schemes. The linear coding scheme maximised the equivalence across SRH forms.

CONCLUSION

The three SRH forms were not equivalent in terms of construct validity. Studies examining the evolution of SRH over time with surveys using different forms should use the linear coding scheme to maximise equivalence between SRH forms.

Facial paralysis is the most common cranial nerve paralysis and the majority of these are idiopathic. Idiopathic facial nerve paralysis, or Bell palsy, typically presents acutely, affects the entire face, may be associated with hyperacusis, a decrease in lacrimation, salivation, or dysgeusia, and typically resolves spontaneously. The diagnosis of idiopathic facial paralysis is made after a thorough history and physical examination to exclude alternative etiologies and follow-up to ensure recovery of facial function. Atypical presentation, recurrent paralysis, additional neurologic deficits, lack of facial recovery in 2-3 months, or a history of head and neck or cutaneous malignancy are concerning for alternative causes of facial paralysis requiring workup. The erroneous use of the eponym Bell palsy to refer to all causes of facial paralysis, regardless of the history and presentation, may result in cognitive errors, including premature closure, anchoring bias, and diagnosis momentum. Hence, we recommend replacing the eponym Bell palsy with idiopathic facial nerve paralysis.

PURPOSE

To describe a patient with Cobb Syndrome, a rare congenital disorder characterized by cutaneous and spinal arteriovenous malformations, who was found to have bilateral optic nerve pits.

OBSERVATIONS

A 15-year-old boy diagnosed with Cobb Syndrome, manifesting as a large cutaneous port-wine stain associated with an underlying left paraspinous arteriovenous malformation resulting in severe scoliosis, presented for a screening ophthalmological exam. The patient had no visual symptoms. On examination, his visual acuity was 20/20 in each eye; however, bilateral optic disc pits were discovered on biomicroscopy and confirmed by spectral-domain optical coherence tomography.

CONCLUSION AND IMPORTANCE

The unusual finding of bilateral optic disc pits in this rare congenital neurocutaneous disorder represents the first report of ophthalmic abnormalities in association with Cobb syndrome. Patients with Cobb Syndrome may be considered for screening ophthalmological exam for the detection of subclinical optic nerve abnormalities.