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Objective: To characterize the epidemiology and risk factors for nasal obstruction among subjects with cleft lip and/or cleft palate (CL/P) utilizing the well-validated Nasal Obstruction Symptom Evaluation (NOSE) survey. Design: Retrospective cross-sectional study. Setting: Cleft Lip and Palate Program, Children's Hospital of Philadelphia. Patients, Subjects: One thousand twenty-eight surveys obtained from 456 subjects (mean age: 10.10 (4.48) years) with CL/P evaluated between January 2015 and August 2017 with at least 1 completed NOSE survey. Interventions: Nasal Obstruction Symptom Evaluation surveys completed at each annual visit. Main Outcome Measures: Composite NOSE and individual symptom scores. Results: Sixty-seven percent of subjects had nasal obstruction at some point during the study period, with 49% reporting nasal obstruction at latest follow-up. subjects aged 14 years and older reported the most severe symptoms (P = .002). Subjects with cleft lip and alveolus (CL+A) and unilateral cleft lip and palate (CLP) reported more severe nasal blockage than other phenotypes (P = .021). subjects with a history of either posterior pharyngeal flap (PPF) or sphincter pharyngoplasty (SP) had significantly higher NOSE scores than subjects with no history of speech surgery (P = .006). There was no significant difference (P > .050) in NOSE scores with regard to history of primary tip rhinoplasty, nasal stent use, or nasoalveolar molding. Conclusions: There are more severe nasal obstructive symptoms among subjects older than 14 years of age, with CL+A or unilateral CLP, and with a history of PPF or SP. Future studies utilizing the NOSE are needed to evaluate and address this prevalent morbidity in the CLP population. [ABSTRACT FROM AUTHOR]

Objective. Asymmetric crying facies (ACF) is congenital hypoplasia of the depressor anguli oris muscle characterized by asymmetry of lower lip depression during crying. This has an overall incidence of 0.6%. This study determines the incidence of ACF in a large population of patients with 22q11.2 deletion. Patients and Methods. A retrospective review of medical records on patients with a confirmed 22q11.2 deletion was undertaken. Results. A total of 836 records were reviewed. Of these, 117 (14%) were noted to have ACF on physical examination. Within this latter group, palatal anomalies were common (77%), as was congenital heart disease (78%); however, these numbers did not differ significantly from their known prevalence in the 22q11.2 population. Conclusions. We report a 14% incidence of ACF in patients with a 22q11.2 deletion, significantly higher than in the general population. We suggest, therefore, that newborns with ACF be referred for further screening for the 22q11.2 deletion syndrome. [ABSTRACT FROM PUBLISHER]