ABSTRACT
The diagnosis of subglottic stenosis (SGS) is usually made by clinical assessment and definitively by a direct endoscopic examination. This study aimed to evaluate different spirometric values in relation to anatomical grading and severity of subglottic stenosis cases of upper airway obstruction. Cases of SGS that underwent dilatational procedures endoscopically at the otolaryngology department of the King Saud University Medical City, Riyadh, Saudi Arabia, from June 2015 to October 2017 were collected. Pulmonary function test (PFT) pre- and postoperative parameters and the grades of subglottic stenosis were extracted. We compared different spirometric values to the severity of SGS and compared the pre- and postoperative results for each patient. There were 19 cases with a valid PFT study within 7 days preoperatively in addition to a documented intraoperative grading according to the Myer-Cotton grading system; 7 (36.8%) were grade 1, 8 (42.1%) were grade 2, and 4 (21.1%) were grade 3. The actual preoperative ratio of forced expiratory volume (FEV1) in 1 second to peak expiratory flow (PEF) for all 19 patients ranged from 7.34 to 21.40 mL/L/min. We found a significant improvement in all spirometric parameters postdilatation including PEF ( P < .001), FEV1 ( P < .001), FEV1/PEF ( P = .001), forced expiratory flow (FEF) from 25%, 50%, and 75% of vital capacity, respectively, FEF25 ( P < .001), FEF50 ( P = .001), FEF75 ( P = .048), and maximum mid-expiratory flow ( P = .002). We did not find any correlation between the severity of stenosis and spirometric values. This study revealed that spirometry is a useful marker in following up patients with subglottic stenosis and is also a good indicator to determine postairway surgery outcomes. However, these markers do not correlate with anatomical grading and the severity of subglottic stenosis.
Subject(s)
Airway Obstruction/diagnosis , Laryngostenosis/diagnosis , Severity of Illness Index , Spirometry/statistics & numerical data , Adult , Airway Obstruction/etiology , Airway Obstruction/pathology , Biomarkers/analysis , Dilatation , Female , Humans , Laryngostenosis/complications , Laryngostenosis/pathology , Male , Postoperative Period , Preoperative Period , Reference ValuesABSTRACT
BACKGROUND: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with 12 reported patients to date. Additional features, previously described, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay, facial dysmorphism, osteopenia, sensorineural deafness, choanal atresia, craniosynostosis and pancreatic exocrine insufficiency. CASE PRESENTATION: We report a new case for consanguineous parents with homozygous novel mutation in GLIS3 gene who presented with neonatal diabetes mellitus, severe resistant congenital hypothyroidism, cholestatic liver disease, bilateral congenital glaucoma and facial dysmorphism. There were associated abnormalities in the external genitalia in form of bifid scrotum, bilateral undescended testicles, microphallus and scrotal hypospadias which might be a coincidental finding. CONCLUSIONS: We suggest that infants with neonatal diabetes associated with dysmorphism should be screened for GLIS3 gene mutations.
