Rosaline S. Zhang

Nasal Obstruction in Children With Cleft Lip and Palate: Results of a Cross-Sectional Study Utilizing the NOSE Scale

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: Perioperative Morbidity in 71 Midfacial Distraction Procedures in Syndromic Craniosynostosis

CONCLUSION: Patients born with sagittal craniosynostosis have abnormal connections in infancy in most of the neural networks compared to controls. There are specific connectivity changes that occur in the RFPN, LFPN, V2, and V3 networks, areas associated with executive function and emotional control, three months after surgery. Changes in anisotropy, measure of white matter tract microstructure, correlate with changes in functional connectivity of areas of the brain connected by white matter tracts after surgery relative to before. Surgery may produce positive changes in the brain microstructure, which could be leading to changes in neural connectivity in the brains of children born with craniosynostosis. As the child develops into adolescence, much of the abnormal network connections seen in infancy correct compared to age-matched controls. However some aberrancies remain in the SA and RFPN network and these residual irregularities may be best handled by other medical therapies.

Evaluation of parental and surgeon stressors and perceptions of distraction osteogenesis in pediatric craniofacial patients: a cross-sectional survey study

PurposeThere is a paucity of literature on how limitations of distraction osteogenesis (DO) are perceived by physicians and parents of pediatric patients. Specifically understanding which features of DO are most concerning to these two groups may better inform parent education, as well as direct improvements in distraction protocols and devices.MethodParents/guardians of patients (between January 2016 and October 2017) being treated with craniofacial distraction were recruited to complete a survey regarding level of stress (1u2009=u2009not stressful, 9u2009=u2009maximally stressful) associated with eight features of DO. Craniofacial surgeons completed a survey asking them to report (1) their personal level of stress and (2) their perceptions of parental stress regarding these same eight features of DO.ResultsThirty-five parents and 15 craniofacial surgeons completed the survey. The risk of the device getting infected was perceived as most stressful by parents (5.5u2009±u20092.3) followed by the device sticking through the skin (4.9u2009±u20092.6) and the second operation for removal (4.7u2009±u20092.3). These same three features also elicited the highest level of stress among surgeons. Surgeon-perceived parental stress regarding turning of the distractor (5.8u2009±u20091.5) was significantly higher than parent self-reported stress (4.2u2009±u20092.8, pu2009=u20090.042).ConclusionsBoth parents and surgeons perceive risk of device-associated infection, the protrusion of the device through the skin, and the requirement of a second operation for removal as the most stressful drawbacks of distraction. Infection reduction protocols, less obtrusive devices, and devices that do not require removal are potential targets for stress reduction.