RESUMO
BACKGROUND: Hemifacial microsomia (HFM) is a rare congenital malformation characterized by a combination of various anomalies, including the face, ears, eyes, and vertebrae. Prenatal diagnosis for HFM is possible, and quite accurate ultrasound can detect obvious defects. The etiology is still unknown, although some hypotheses have been proposed, including gene mutation, chromosome anomaly, and environmental risk factors. However, there are few reports of pulmonary hypoplasia and dextrocardia in HFM. CASE SUMMARY: A 2-year-old boy presented to the ear reconstruction department of our hospital complaining of deviation of the face to the right side and auricular anomaly. Physical examination revealed facial asymmetry, preauricular skin tags, and concha-type microtia with stricture of the external auditory canal on the right side. Head magnetic resonance imaging showed bilateral semicircular canal dysplasia and bilateral internal auditory canals stenosis. Audiometric examination showed bilateral severe sensorineural hearing loss. Chest radiography and computed tomography showed dextrocardia and right pulmonary hypoplasia. CONCLUSION: This case presented a rare finding and an unusual association of 3 malformations, ipsilateral HFM, pulmonary agenesis, and dextrocardia.
RESUMO
It has been widely reported that the serum anion gap is significantly associated with mortality in intensive care unit (ICU); however, it remains unknown whether the association is present in aortic aneurysm (AA) patients. The present study aimed to investigate the association between the admission serum anion gap and ICU mortality in AA patients. Data extracted from a publicly accessible clinical database using a modifiable data mining technique were analyzed retrospectively, mainly by employing multivariable logistic regression analysis. The primary study outcome was ICU mortality. A total of 273 patient records were analyzed. The ICU mortality was 8.79% (24/273). The median serum anion gap was significantly higher in non-survivors [17.50 mEq/l, interquartile range (IQR) 15.75-22.50 mEq/l] compared with survivors [13.00 mEq/l, IQR 11.00-15.00 mEq/l, P<0.001]. Multivariate analysis resulted in identification of a clear association between admission serum anion gap and ICU mortality in AA patients [odds ratio (OR) 1.38 per 1 mEq/l increase, 95% confidence interval (CI) 1.08-1.76]. The area under the receiver operating characteristic curve showed an outstanding discrimination ability in predicting ICU mortality (area under curve 0.8513, 95% CI 0.7698-0.9328). In conclusion, admission serum anion gap may serve as a strong predictor of ICU mortality for AA patients.