ABSTRACT
The presence of a bilobed tongue is a rare congenital malformation. There are multiple reports of a bifid anterior lobe; however, a case with complete separation of isolated anterior and posterior lobes has not been previously described. We report the case of a 2-year-old male, who presented with a V-shaped mandible and glossoptosis in the setting of respiratory distress and difficulty feeding, incidentally found to have a bilobed tongue with independent anterior and posterior lobes.
Subject(s)
Glossoptosis/congenital , Mandible/abnormalities , Tongue/abnormalities , Humans , Incidental Findings , Infant , Male , Respiratory Distress Syndrome, Newborn/complicationsABSTRACT
OBJECTIVES/HYPOTHESIS: To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS). STUDY DESIGN: Nested cohort cross-sectional study. METHODS: All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3. RESULTS: Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively. CONCLUSIONS: Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:502-508, 2018.
Subject(s)
Endoscopy/statistics & numerical data , Glossoptosis/classification , Glossoptosis/diagnosis , Pierre Robin Syndrome/complications , Cohort Studies , Cross-Sectional Studies , Endoscopy/methods , Female , Glossoptosis/congenital , Humans , Infant , Infant, Newborn , Likelihood Functions , Male , Prospective Studies , ROC Curve , Sensitivity and SpecificityABSTRACT
Glossoptosis causes varying degrees of airway obstruction and feeding difficulties. It can occur as a consequence of micrognathia in Robin Sequence, but can also occur in children with hypotonia. Despite several attempts to classify severity in Robin Sequence patients, taking into account symptoms, presence of concomitant syndromes or malformations, and even endoscopic findings, there is still no general consensus. Furthermore, several management recommendations have been reported without an agreement about indications, efficacy, or risks of each treatment option. The present article provides an overview of clinical presentation, diagnosis, management, and prognosis of patients with glossoptosis.
