Tympanostomy Tube Otorrhea: Microbiological Differences Between Children with and Without Cleft Palate.

OBJECTIVE: To characterize and compare microbiological profiles in tympanostomy tube otorrhea for children with and without cleft palate. DESIGN: Retrospective cohort study. SETTING: Pediatric otolaryngology and multidisciplinary cleft/craniofacial clinic at a single tertiary care center. PATIENTS: Children with and without cleft palate <18 years of age who underwent tympanostomy tube placement between 2017-2021. MAIN OUTCOME MEASURES: Otopathogen profiles and antibiotic resistance patterns in ear culture specimens obtained in children presenting for treatment of recalcitrant post-tympanostomy tube otorrhea. RESULTS: Of the 886 children with tympanostomy tubes placed between 2017-2021, 345 (38.9%) had clinically significant otorrhea defined as requiring at least one otolaryngology visit for treatment. Children with cleft palate had higher rates of otorrhea (50.0% versus 35.7%; P < .01). In the 128 cultures obtained, Staphylococcus aureus was the most common organism in both groups present in 39.8% of cultures; 49% were methicillin-resistant (MRSA). Pseudomonas aeruginosa was also frequently isolated (20.0% versus 23.4%, P = .69) in children with and without cleft palate. Collectively, fluoroquinolone resistance was observed in 68.6% and 27.6% of the S. aureus and P. aeruginosa isolates, respectively, however, no differences in fluoroquinolone resistance were observed between cleft and non-cleft cohorts. Corynebacterium species were isolated more frequently in children with cleft palate (26.7% versus 6.1%, P < .01), a finding of unclear significance. CONCLUSIONS: Recalcitrant post-tympanostomy tube otorrhea is more common in children with cleft palate. MRSA was the most common isolate, which was commonly resistant to first-line fluoroquinolone therapy.

Three-Dimensional Printing in Cleft Care: A Systematic Review.

OBJECTIVE: To determine the current applications of 3-dimensional (3D) printing in the care of patients with cleft lip and palate. We also reviewed 3D printing limitations, financial analysis, and future implications. DESIGN: Retrospective systematic review. METHODS: Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines were used by 3 independent reviewers. Articles were identified from Cochrane library, Ovid Medline, and Embase. Search terms included 3D printing, 3 dimensional printing, additive manufacturing, rapid prototyping, cleft lip, and cleft palate. Exclusion criteria included articles not in English, animal studies, reviews without original data, oral presentations, abstracts, opinion pieces, and articles without relevance to 3D printing or cleft lip and palate. MAIN OUTCOME MEASURES: Primary outcome measure was the purpose of 3D printing in the care of patients with cleft lip and palate. Secondary outcome measures were cost analysis and clinical outcomes. RESULTS: Eight-four articles were identified, and 39 met inclusion/exclusion criteria. Eleven studies used 3D printing models for nasoalveolar molding. Patient-specific implants were developed via 3D printing in 6 articles. Surgical planning was conducted via 3D printing in 8 studies. Eight articles utilized 3D printing for anatomic models/educational purposes. 3-Dimensional printed models were used for surgical simulation/training in 6 articles. Bioprinting was utilized in 4 studies. Secondary outcome of cost was addressed in 8 articles. CONCLUSION: 3-Dimensional printing for the care of patients with cleft lip and palate has several applications. Potential advantages of utilizing this technology are demonstrated; however, literature is largely descriptive in nature with few clinical outcome measures. Future direction should be aimed at standardized reporting to include clinical outcomes, cost, material, printing method, and results.

Utility of Screening for Obstructive Sleep Apnea with the Pediatric Sleep Questionnaire (PSQ) in Children with Craniofacial Anomalies.

OBJECTIVE: To determine the accuracy of the Pediatric Sleep Questionnaire (PSQ) as a screening tool for obstructive sleep apnea in children with craniofacial anomalies. DESIGN: Retrospective cohort study. SETTING: Multidisciplinary cleft and craniofacial clinic at a tertiary care center. PATIENTS: Children with craniofacial anomalies 2 to ≤18 years of age who both completed a PSQ screen and underwent polysomnography (PSG) without interval surgery. MAIN OUTCOME MEASURES: Sensitivity and specificity of the PSQ in detecting an obstructive apnea-hypopnea index (AHI) ≥ 5 events/hour. RESULTS: Fifty children met study criteria, with 66% (n = 33) having an associated syndrome. Mean patient age at time of PSQ was 9.6 + 4.0 years. Overall, 33 (64%) screened positive on the PSQ, while 20 (40%) had an AHI ≥ 5. The sensitivity and specificity for identifying AHI ≥ 5 was 70% and 40%, respectively. With subgroup analysis, the sensitivity and specificity were higher (100% and 50%) in children with non-syndromic palatal clefting but lower (65% and 31%) in children with a syndrome or chromosomal anomaly. There was no correlation detected between PSQ score and AHI severity (p = 0.25). The mean obstructive AHI in the study population was 10.1 ± 22.7 despite 44% (n = 22) undergoing prior adenotonsillectomy. CONCLUSIONS: The PSQ was less sensitive and specific in detecting an AHI ≥ 5 in children with craniofacial anomalies than in a general population, and particularly poor in for children with syndrome-associated craniofacial conditions. Given the high prevalence of OSA in this patient population, a craniofacial-specific validated screening tool would be beneficial.

Comparison of Oral Loading Dose to Intravenous Acetaminophen in Children for Analgesia After Tonsillectomy and Adenoidectomy: A Randomized Clinical Trial.

BACKGROUND: Acetaminophen is a frequently used adjunct analgesic in pediatric patients undergoing tonsillectomy and adenoidectomy. We compared opioid administration following preoperative intravenous (IV) or oral acetaminophen in addition to a standard multimodal regimen to test the hypothesis that 1 loading dose approach would provide superior opioid sparing effects among pediatric surgical patients undergoing tonsillectomy and adenoidectomy. METHODS: This single-center, double-blind, double-dummy prospective randomized study was conducted in patients ages 3 to 15 years undergoing tonsillectomy and adenoidectomy with or without myringotomy and tube placement between September 2017 and July 2019. Subjects received 1 dose of either oral acetaminophen 30 mg/kg with IV placebo (oral group) or IV acetaminophen 15 mg/kg with oral placebo (IV group). Acetaminophen plasma levels were measured at 2 timepoints to evaluate safety and determine plasma levels attained by each dosing regimen. Intraoperative opioid administration and postoperative analgesia were standardized. Standardized postoperative multimodal analgesia included opioid if needed to control pain assessed by standardized validated pediatric pain scales. The primary outcome measure was total opioid administration in the first 24 hours after surgery. Continuous data were not normally distributed and were analyzed using the Wilcoxon rank sum test and the Hodges-Lehman estimator of the median difference. Clinical significance was defined as a 100 µg/kg IV morphine equivalents per day difference. RESULTS: Sixty-six subjects were randomized into and completed the study (29 women, 37 men; age 5.9 ± 3.0 years; percentile weight for age 49.5 ± 30.2; no differences between groups). There was no opioid dose difference between oral (median 147.6; interquartile range [IQR], 119.6-193.0 µg/kg) and IV groups (median 125.4; IQR, 102.8-150.9 µg/kg; median difference 21.3; 95% confidence interval [CI] -2.5 to 44.2 µg/kg IV morphine equivalents; P = .13). No acetaminophen levels exceeded the predefined safety threshold (40 mg/L). No difference was found in the percentage of patients with severe pain: 50.0% oral group, 47.2% IV group; relative risk of severe pain in IV 0.94; 95% CI, 0.57-1.6; P = .82. Postoperative plasma acetaminophen levels were higher in oral (22; IQR, 16-28 mg/L) than IV (20; IQR, 17-22 mg/L) group (median difference 7.0; 4.0-8.0 mg/L; P = .0001). CONCLUSIONS: Opioid-sparing effects did not differ following an oral or standard IV acetaminophen loading dose with no identified acetaminophen toxicity in pediatric patients undergoing tonsillectomy and adenoidectomy who received standardized multimodal postoperative analgesia. An oral loading dose may provide more consistent serum acetaminophen levels at lower cost compared to a standard IV dose.

A genome-wide association study implicates the BMP7 locus as a risk factor for nonsyndromic metopic craniosynostosis.

Our previous genome-wide association study (GWAS) for sagittal nonsyndromic craniosynostosis (sNCS) provided important insights into the genetics of midline CS. In this study, we performed a GWAS for a second midline NCS, metopic NCS (mNCS), using 215 non-Hispanic white case-parent triads. We identified six variants with genome-wide significance (P ≤ 5 × 10-8): rs781716 (P = 4.71 × 10-9; odds ratio [OR] = 2.44) intronic to SPRY3; rs6127972 (P = 4.41 × 10-8; OR = 2.17) intronic to BMP7; rs62590971 (P = 6.22 × 10-9; OR = 0.34), located ~ 155 kb upstream from TGIF2LX; and rs2522623, rs2573826, and rs2754857, all intronic to PCDH11X (P = 1.76 × 10-8, OR = 0.45; P = 3.31 × 10-8, OR = 0.45; P = 1.09 × 10-8, OR = 0.44, respectively). We performed a replication study of these variants using an independent non-Hispanic white sample of 194 unrelated mNCS cases and 333 unaffected controls; only the association for rs6127972 (P = 0.004, OR = 1.45; meta-analysis P = 1.27 × 10-8, OR = 1.74) was replicated. Our meta-analysis examining single nucleotide polymorphisms common to both our mNCS and sNCS studies showed the strongest association for rs6127972 (P = 1.16 × 10-6). Our imputation analysis identified a linkage disequilibrium block encompassing rs6127972, which contained an enhancer overlapping a CTCF transcription factor binding site (chr20:55,798,821-55,798,917) that was significantly hypomethylated in mesenchymal stem cells derived from fused metopic compared to open sutures from the same probands. This study provides additional insights into genetic factors in midline CS.

Prevalence of Renal and Cervical Vertebral Anomalies in Patients With Isolated Microtia and/or Aural Atresia.

OBJECTIVE: The objective of this study was to determine whether patients with isolated microtia or aural atresia have an increased prevalence of renal or cervical vertebral anomalies. DESIGN: The study design was a retrospective medical record review. SETTING: The setting was the following four distinct institutions: an urban tertiary care children's hospital, two urban academic medical centers, and a staff-model health maintenance organization. PARTICIPANTS: Patients diagnosed with microtia, aural atresia, or oculoauriculovertebral spectrum were identified. Patients with facial asymmetry, craniofacial microsomia, and other craniofacial abnormalities or syndromes were excluded. MAIN OUTCOME MEASURES: Main outcome measures were the number of patients with isolated microtia or aural atresia who underwent a renal ultrasound or cervical spine X-ray, the results of those studies, and further evaluation or treatment for any abnormalities found. STATISTICAL ANALYSIS: A binomial analysis using a one-sided 95% confidence level was performed. RESULTS: A total of 514 patients with isolated microtia and/or aural atresia were identified. Of these patients, 145 (28%) had undergone a renal ultrasound and 81 (16%) had undergone cervical spine X-rays. A total of 3 patients (2%) had minimal renal pelviectasis, all of which had resolved on repeat ultrasound and required no treatment. There were no structural renal abnormalities identified, and there were no cervical spine abnormalities identified. CONCLUSIONS: The data suggest that there is no increased prevalence of structural renal or cervical vertebral anomalies in patients with isolated microtia and/or aural atresia. Therefore, these patients do not require routine screening renal ultrasound or cervical spine X-rays.

Neurofibromatosis of the larynx causing stridor and sleep apnea.

BACKGROUND: Neurofibromatosis type 1 can rarely present in the larynx. Patients typically do not present with complete obstructive symptoms, but partial obstruction and stridor. We review our health centers' case series of two patients, the first of whom presented with persistent sleep apnea post tonsillectomy and adenoidectomy, and the second who presented with noisy breathing. Additionally, we will review the literature on the management and treatment options for children with this rare clinical entity. METHODS: Retrospective case review. CASE REPORT & RESULTS: A two-year old male underwent a sleep endoscopy following persistent evidence of obstructive sleep apnea on polysomnography after initial tonsillectomy and adenoidectomy. Family elicited concerns about noisy breathing at night and an accompanying video documented stridor while sleeping during the monitored polysomnography. Flexible fiberoptic laryngoscopy in the operating room revealed what appeared to be a cystic mass along the right aryepiglottic fold causing deviation of the laryngeal introitus towards the contralateral side. Subsequent direct laryngoscopy and excisional biopsy revealed pathology results consistent with a plexiform neurofibroma. A six-month-old patient with stertor and stridor was found to have a laryngeal mass, subglottic stensosis, and progressive airway obstruction due to plexiform neurofirboma in the supraglottis, subglottis, and trachea. We present a series of two patients incidentally diagnosed with neurofibromatosis type 1 by way of a laryngeal neurofibroma and review the literature on management options. Both patients were found to have accompanying café au lait spots. Both patients required tracheostomy for airway management, and one was successfully decannulated. CONCLUSION: Laryngeal neurofibroma is a rare anomaly that can manifest with airway obstruction. Both patients presented here subsequently were noted to have café au lait spots on physical examination. The Otolaryngologist should be reminded of this anomaly when evaluating a child with evidence of a submucosal laryngeal mass. We present our series including that of a patient whose diagnosis was prompted by persistent sleep apnea following adenotonsillectomy tonsillectomy and a patient with airway obstruction and subglottic stenosis due to a neurofibroma. The treatment of choice is complete excision of the neurofibroma while maintaining functionality of the larynx. This can lead to successful decannulation.

Whole genome sequencing identifies associations for nonsyndromic sagittal craniosynostosis with the intergenic region of BMP2 and noncoding RNA gene LINC01428.

Craniosynostosis (CS) is a major birth defect resulting from premature fusion of cranial sutures. Nonsyndromic CS occurs more frequently than syndromic CS, with sagittal nonsyndromic craniosynostosis (sNCS) presenting as the most common CS phenotype. Previous genome-wide association and targeted sequencing analyses of sNCS have identified multiple associated loci, with the strongest association on chromosome 20. Herein, we report the first whole-genome sequencing study of sNCS using 63 proband-parent trios. Sequencing data for these trios were analyzed using the transmission disequilibrium test (TDT) and rare variant TDT (rvTDT) to identify high-risk rare gene variants. Sequencing data were also examined for copy number variants (CNVs) and de novo variants. TDT analysis identified a highly significant locus at 20p12.3, localized to the intergenic region between BMP2 and the noncoding RNA gene LINC01428. Three variants (rs6054763, rs6054764, rs932517) were identified as potential causal variants due to their probability of being transcription factor binding sites, deleterious combined annotation dependent depletion scores, and high minor allele enrichment in probands. Morphometric analysis of cranial vault shape in an unaffected cohort validated the effect of these three single nucleotide variants (SNVs) on dolichocephaly. No genome-wide significant rare variants, de novo loci, or CNVs were identified. Future efforts to identify risk variants for sNCS should include sequencing of larger and more diverse population samples and increased omics analyses, such as RNA-seq and ATAC-seq.

Unilateral and bilateral expression of a quantitative trait: asymmetry and symmetry in coronal craniosynostosis.

Bilateral symmetry in vertebrates is imperfect and mild asymmetries are found in normal growth and development. However, abnormal development is often characterized by strong asymmetries. Coronal craniosynostosis, defined here as consisting of premature suture closure and a characteristic skull shape, is a complex trait. The premature fusion of the coronal suture can occur unilaterally associated with skull asymmetry (anterior plagiocephaly) or bilaterally associated with a symmetric but brachycephalic skull. We investigated the relationship between coronal craniosynostosis and skull bilateral symmetry. Three-dimensional landmark coordinates were recorded on preoperative computed tomography images of children diagnosed with coronal nonsyndromic craniosynostosis (N = 40) and that of unaffected individuals (N = 20) and analyzed by geometric morphometrics. Our results showed that the fusion pattern of the coronal suture is similar across individuals and types of coronal craniosynostosis. Shape analysis showed that skulls of bilateral coronal craniosynostosis (BCS) and unaffected individuals display low degrees of asymmetry, whereas right and left unilateral coronal craniosynostosis (UCS) skulls are asymmetric and mirror images of one another. When premature fusion of the coronal suture (without taking into account cranial dysmorphology) is scored as a qualitative trait, the expected relationship between trait frequency and trait unilateral expression (i.e. negative correlation) is confirmed. Overall, we interpret our results as evidence that the same biological processes operate on the two sides in BCS skulls and on the affected side in UCS skulls, and that coronal craniosynostosis is a quantitative trait exhibiting a phenotypic continuum with BCS displaying more intense shape changes than UCS.

Nasal trumpet as a long-term remedy for obstructive sleep apnea syndrome in a child.

We present a case of successful long-term use of nasal trumpet for severe obstructive sleep apnea syndrome in a child with cerebral palsy and complex medical issues. Obstructive sleep apnea syndrome is frequently seen in pediatric patients with cerebral palsy due to their abnormal airway tone and pulmonary vulnerability. Identifying children with cerebral palsy who are at risk for obstructive sleep apnea syndrome is important because its treatment can improve quality of life and seizure control. Although first-line treatment for obstructive sleep apnea syndrome is adenotonsillectomy, children with cerebral palsy are more likely to have residual obstructive sleep apnea syndrome postoperatively. Other options such as positive airway pressure therapy and other upper airway surgeries may pose significant challenges and tolerance issues, as in our patient. As demonstrated in our report, the low rate of complications and ease of use make nasal trumpets a potential long-term treatment option for children with obstructive sleep apnea syndrome who fail or cannot comply with the traditional treatment options.

The macroscopic and microscopic effects of radiofrequency injury in the porcine tongue: a pilot study.

OBJECTIVE: Current treatment protocols for obstructive sleep apnea/hypopnea syndrome with radiofrequency ablation of the base of the tongue are empiric. Injecting local anesthetics and saline into the treatment site is believed to shorten treatment duration and improve results. Our objective is to compare lesions at different energy levels, both macroscopically and histologically, and to determine if electrolyte solution injection results in a larger lesion. STUDY DESIGN: A prospective, experimental animal study. SETTING: University-affiliated institution. SUBJECTS AND METHODS: Five pigs each received four treatments on the right and four on the left side of the tongue. Three of four treatments were applied sequentially by increasing length of exposure (15, 30, or 60 seconds), and the fourth was conducted over 15 seconds after injecting 1 cc of a 1:1 local anesthetic-saline solution into the treatment site. The animals were recovered for three days and then sacrificed. Macroscopic measurements for each lesion were analyzed, and histological comparison was performed. RESULTS: The average volume of the lesions was 57.36, 65.18, and 60.50 mm(3) for treatment durations of 60, 30, and 15 seconds, respectively, and there was no significant difference. Lesion volume at sites where anesthetic-saline was injected had a mean volume of 36.72 mm(3). Paired comparison against the three treatment durations revealed significantly smaller lesion volume size (P = 0.0041, 0.0007, 0.0047, respectively). CONCLUSION: The lesions created with radiofrequency energy were consistent and predictable. The volumes of the lesions did not differ significantly with regard to different energy levels. The lesion was significantly smaller after injection of anesthetic-saline at the treatment site.

Children with Cleft Palate: Predictors of Otologic Issues in the First 10 Years.

OBJECTIVE: To evaluate the characteristics of children with cleft palate associated with persistent otologic issues in the first 10 years of life. STUDY DESIGN: Case series with chart review. SETTING: Single academic center. SUBJECTS AND METHODS: Children born with cleft palate from 2003 to 2007 and treated by the UC Davis Cleft and Craniofacial Team between January 2003 and December 2017 were included in the study. Data from 143 patients were analyzed via Wilcoxon rank sum and Fisher exact tests for univariate analysis and logistic regression to determine adjusted odds ratios. RESULTS: The median length of follow-up was 9.9 years, and the age at last ear examination was 10.7 years. At the last evaluation, unresolved otologic issues were common, with at least 1 ear having a tympanic membrane (TM) perforation (16.1%), a tympanostomy tube (36.2%), or conductive hearing loss (23.1%). After adjusting for demographic and clinical characteristics, history of palate revision or speech surgery was associated with having a TM perforation ( P = .02). The only clinical variables associated with conductive hearing loss was the presence of a TM perforation ( P < .01) or a genetic abnormality ( P = .02). Severity of palatal clefting was not associated with specific otologic or audiologic outcomes after adjusting for other characteristics. CONCLUSION: A large proportion of children with cleft palate have persistent otologic issues at age 10 years and would benefit from continued close monitoring well after the age when most children have normalized eustachian tube function. Prolonged otologic issues were not found to be associated with cleft type.

Children's pre-operative tonsillectomy pain education: clinical outcomes.

OBJECTIVE: To examine the effects of pre-operative tonsillectomy pain education on children's (7-13 years) self-reported pre-operative anxiety and post-operative clinical outcomes (i.e., anxiety, pain intensity, quality of pain and sleep, oral intake, perceptions of pre-operative education, and pain expectation). METHOD: A prospective, repeated measures, quasi-experimental design using an age appropriate pain education booklet (n = 30) and a standard care comparison group (n = 30) was employed to investigate children's pre- and post-education anxiety and post-operative tonsillectomy with or without adenoidectomy subjective experiences in the hospital and home settings. Group comparisons were performed using the Wilcoxon test, Fisher's exact test, repeated measures analysis of variance, and mixed model regression. RESULTS: There were no significant differences between groups for measures of anxiety, pain intensity, quality of pain and sleep, oral intake, or expected pain. There was no change in anxiety before or after pre-operative education (P = 0.85). Ninety-six percent (n = 25) of the children in the intervention group reported that pre-operative pain education helped with their post-operative pain and 72% (n = 16) in the control group stated that it would be helpful to learn about pain before surgery. The majority of children in both the intervention and control groups (96%, 91%, respectively) stated learning about the 0-10 numeric pain intensity scale helped or would be helpful to learn pre-operatively. CONCLUSION: Pre-operative pain education did not affect anxiety. Children valued pre-operative pain education. Pre-operative pain education may influence children's perceptions of medical care.

Marginal versus segmental mandibulectomy for pediatric desmoid fibromatosis of the mandible - Two case reports and review of the literature.

Desmoid fibromatosis (DF) is a rare, benign soft tissue neoplasm with high rate of local recurrence. Surgical management of DF in the head and neck can be challenging given the desire to balance the preservation of form and function with the need to minimize local recurrence by achieving complete resection. We present two contrasting cases which highlight the advantages of marginal mandibulectomy over segmental mandibulectomy in children with DF. We favor marginal mandibulectomy even with limited bone stock given the remarkable ability of children to generate new bone.

Restoration of eyelid closure in facial paralysis using artificial muscle: preliminary cadaveric analysis.

OBJECTIVES: Electroactive polymer artificial muscle is an emerging technology that has the potential to be used in rehabilitating facial movement in patients with paralysis. These electroactive polymers act like human muscles by expanding and contracting based on variable voltage input levels. The authors seek to establish a reproducible eyelid blink with artificial muscle. The aim of this proof of concept study is to determine whether eyelid closure can be created with a novel eyelid sling model. STUDY DESIGN: A human cadaver study was performed. METHODS: With use of four cadaver heads, an extended upper and lower blepharoplasty incision was used to secure an upper and lower expanded polytetrafluoroethylene implant in the medial orbital wall and tarsal plates. The slings were passed through a hole drilled in the lateral orbital wall or around a titanium screw. Lateral pull on the sling created eyelid closure, and the necessary distance of pull was measured. RESULTS: The eyelid sling mechanism functioned to achieve complete eyelid closure. Less tension was required for eyelid closure when the sling was placed in both eyelids (3 mm of pull instead of 6 mm). CONCLUSIONS: The application of artificial muscle to a range of problems that affect both patient morbidity and quality of life is promising. Eyelid closure was created in a cadaver model using a novel sling, but future studies will need to address the feasibility of a prototype artificial muscle eyelid device in humans.

Initial validation of a numeric zero to ten scale to measure children's state anxiety.

BACKGROUND: Although children experience physical and behavioral consequences from anxiety in many health care settings, anxiety assessment and subsequent management is not often performed because of the lack of clinically useful subjective scales. Current state anxiety scales are either observational or multidimensional self-report measures requiring significant clinician and patient time. Because anxiety is subjective, in this pilot study, we evaluated the validity of a self-report numeric 0-10 anxiety scale that is easy to administer to children in the clinical setting. METHODS: A descriptive correlation research design was used to determine the concurrent validity for a numeric 0-10 anxiety scale with the state portion of the State-Trait Anxiety Inventory for Children (STAIC). During clinic preoperative visits, 60 children, 7-13 yr, provided anxiety scores for the 0-10 scale and the STAIC pre- and posteducation. Simple linear regression and Pearson correlation were performed to determine the strength of the relationship. RESULTS: STAIC was associated with the anxiety scale both preeducation (beta = 1.20, SE[beta] = 0.34, F[1,58] = 12.74, P = 0.0007) and posteducation (beta = 1.97, SE[beta]) = 0.31, F[1,58] = 40.11, P < 0.0001). Correlations were moderate for pre-education (r = 0.424) and posteducation (r = 0.639). CONCLUSIONS: This initial study supports the validity of the numeric 0-10 anxiety self-report scale to assess state anxiety in children as young as 7 yr.

Should otolaryngologists perform pH probe studies?

PURPOSE OF REVIEW: This commentary discusses recent developments in the methods available to pediatric otolaryngologists in the diagnosis of laryngopharyngeal reflux. RECENT DEVELOPMENTS: The development of detachable wireless monitoring allows the otolaryngologists to perform their own pH monitoring. It can be deployed while performing other procedures, such as laryngoscopy and bronchoscopy. The procedure to insert the probe and collect data is described. SUMMARY: It is hoped that the pH probe will play an important role in understanding the pathological role of pharyngeal reflux in terms of laryngeal, pharyngeal, sinus and ear pathology.

Management of bilateral cleft lip and nasal deformity.

PURPOSE OF REVIEW: Management of bilateral cleft lip and nasal deformity can be a challenging task. This paper provides an overview of bilateral cleft lip and nasal deformity with an updated review of current management issues in the literature. RECENT FINDINGS: The Centers for Disease Control and Prevention recently reported that orofacial clefts are now the most common birth defect. While this statistic may be disheartening, the increased prevalence brings the problem to light at the forefront of the medical community, thus gaining more support and resources. Many techniques have been described for repair of bilateral cleft lip and nasal deformity. A recent advancement in presurgical orthopedics is the use of nasoalveolar molding to narrow wide clefts. SUMMARY: Surgical management of bilateral cleft lip and nasal deformity poses a challenge to the skill and judgment of the cleft surgeon. Although techniques continue to evolve over the decades, the basic principles of cleft surgery remain the same. The main principles are to achieve an appropriate philtral size and shape, to position the cartilages in a more optimal position, and to attain muscular continuity and symmetry for optimal appearance and function. Thus, while keeping the basic principles in mind, management of bilateral cleft lip and nasal deformity becomes a valuable and rewarding experience for the surgeon, patient and caregiver.

Prognosis of airway obstruction and feeding difficulty in the Robin sequence.

OBJECTIVE: To evaluate the course and prognosis of airway obstruction and feeding difficulty in the Pierre Robin sequence (PRS). METHODS: Retrospective review of 60 patients with PRS between 1993 and 2002 at the University of California, Davis Medical Center. Patients were placed into diagnostic subgroups: (1) Isolated PRS; (2) Syndromic PRS (known syndrome with PRS); (3) Unique PRS (unique anomalies with PRS). Data regarding severity, duration, and management of airway obstruction and feeding difficulty were collected. RESULTS: Airway obstruction requiring intervention beyond positional therapy was seen in 28% isolated, 42% syndromic, and 58% unique PRS. One-third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two-thirds of patients, who failed positional therapy required a surgical airway procedure. Four patients underwent mandibular distraction osteogenesis, resulting in successful decannulation or avoidance of tracheostomy. Thirteen patients underwent tracheostomy; mean duration of tracheostomy-dependence was 17.0 months in Isolated PRS and 31.7 months in Unique PRS (p < 0.01). Successful decannulation by age of 3 years was confirmed in 85% of patients who underwent tracheostomy. Tube feeding was required in 53% Isolated, 67% Syndromic, and 83% Unique PRS. Forty-two percent of PRS patients with a successful positional airway still demonstrated feeding difficulty. Short-term (0-3 months) and intermediate (4-18 months) tube feeding was more commonly required in Isolated and Syndromic PRS, while long-term (beyond 18 months) gastrostomy tube feeding was more commonly required in Unique PRS (p < 0.01). By 3 years of age, a successful oral diet was seen in 91% Isolated, 92% Syndromic, and 78% Unique PRS. CONCLUSIONS: Diagnostic subgroups based on the presence of additional anomalies help families and physicians in understanding the severity and duration of feeding and airway difficulty in PRS. Two-thirds of PRS patients who fail positional therapy may ultimately require a surgical airway procedure. Feeding difficulty can be present in the absence of clinically significant airway obstruction. Families and physicians should be encouraged that by 3 years of age, most patients were successfully taking an oral diet without airway obstruction.

Septorhinoplasty in the Pediatric Patient.

Pediatric septorhinoplasty has been an area of controversy because early surgical intervention can prevent normal growth. There are certain conditions where early correction of the nose is indicated, such as in cleft lip nasal deformities, severe traumatic deformities, and congenital nasal lesions. Animal and clinical studies have been helpful in elucidating certain areas of the nose that are potential growth zones that should be left undisturbed when performing nasal surgeries on pediatric patients. We discuss the timing, indications, and surgical technique in pediatric septorhinoplasty.