Kathleen C. Y. Sie

Airway Management in Children with Craniofacial Anomalies

Craniofacial anomalies (CFA) predispose children to airway obstruction. A retrospective study was conducted to describe airway intervention required to manage patients with craniofacial syndromes and diseases involving the midface and mandible (i.e., Pierre Robin, Apert, Treacher Collins, Saethre-Chotzen, CHARGE, Nager, Stickler, Goldenhar, and Pfeiffer). The type of airway intervention, duration of intervention, and associated physical and medical conditions were evaluated. One hundred nine patients had charts available for review and met inclusion criteria. Sixty-five of these patients required airway management, most commonly in the first month of life, ranging from positioning to tracheotomy. Nineteen patients required a tracheotomy. Associated medical conditions and feeding difficulties were associated with airway obstruction. This study evaluates factors that predispose children with CFA to have airway problems that need treatment, as well as the types of airway management that are necessary.

Robin Sequence: From Diagnosis to Development of an Effective Management Plan

The triad of micrognathia, glossoptosis, and resultant airway obstruction is known as Robin sequence (RS). Although RS is a well-recognized clinical entity, there is wide variability in the diagnosis and care of children born with RS. Systematic evaluations of treatments and clinical outcomes for children with RS are lacking despite the advances in clinical care over the past 20 years. We explore the pathogenesis, developmental and genetic models, morphology, and syndromes and malformations associated with RS. Current classification systems for RS do not account for the heterogeneity among infants with RS, and they do not allow for prediction of the optimal management course for an individual child. Although upper airway obstruction for some infants with RS can be treated adequately with positioning, other children may require a tracheostomy. Care must be customized for each patient with RS, and health care providers must understand the anatomy and mechanism of airway obstruction to develop an individualized treatment plan to improve breathing and achieve optimal growth and development. In this article we provide a comprehensive overview of evaluation strategies and therapeutic options for children born with RS. We also propose a conceptual treatment protocol to guide the provider who is caring for a child with RS.

Results with Furlow palatoplasty in the management of velopharyngeal insufficiency.

A retrospective study was undertaken to assess speech outcomes in patients undergoing Furlow palatoplasty. Since 1994, the authors have used the position of the levator veli palatini musculature to determine type of surgical intervention recommended for the management of velopharyngeal insufficiency. Furlow palatoplasty has been used in patients with clinical evidence of sagittally oriented levator veli palatini musculature. Forty‐eight patients who underwent a Furlow palatoplasty between June of 1994 and August of 1998 were included. All patients underwent preoperative and postoperative perceptual speech analyses to describe velopharyngeal insufficiency severity, nasal air emissions, and resonance, and preoperative nasendoscopy to assess velopharyngeal gap size and palatal and lateral pharyngeal wall movement. Other patient characteristics considered included gender, age at time of surgery, previously repaired cleft palate, submucous cleft palate, and syndrome diagnosis. Speech outcomes were determined on the basis of postoperative perceptual speech analyses and were categorized in one of three ways: (1) complete resolution of velopharyngeal insufficiency, (2) substantial improvement of velopharyngeal insufficiency, and (3) audible residual velopharyngeal insufficiency. Complete resolution of velopharyngeal insufficiency was defined as normal resonance and an absence of nasal air emissions. Substantial improvement ofvelopharyngeal insufficiency was defined as an improvement of at least two categories in velopharyngeal insufficiency severity in those patients without complete resolution. Audible residual velopharyngeal insufficiency refers to patients with postoperative velopharyngeal insufficiency severity ratings of mild, moderate, or severe. The male:female ratio in the study was 27:21. Twelve patients were syndromic; three had velocardiofacial syndrome. The median age at surgery was 6.5 years (range, 2 to 22 years). The average duration of follow‐up was 14.7 months (range, 1.3 to 58.6 months). Postoperatively, the severity of velopharyngeal insufficiency was rated as none in 19 of the 48 patients (39.6 percent), minimal in eight (16.7 percent), mild in six (12.5 percent), moderate in nine (18.75 percent), and severe in six (12.5 percent). Substantial improvement was seen in seven of the 29 patients without complete resolution. There was a significant association between male gender and complete resolution of velopharyngeal insufficiency (p < 0.05). Presence of syndrome and female gender was associated with audible residual velopharyngeal insufficiency (p < 0.05). The main complication was palatal fistula (two cases). In conclusion, most patients who underwent a Furlow palatoplasty had a complete resolution or substantial improvement ofvelopharyngeal insufficiency postoperatively, and there were few surgical complications. (Plast. Reconstr. Surg. 108: 17, 2001.)

Results with sphincter pharyngoplasty and pharyngeal flap

OBJECTIVE To evaluate speech outcomes and complications of sphincter pharyngoplasty and pharyngeal flap performed for management of velopharyngeal insufficiency (VPI). DESIGN Case series. SETTING Tertiary care childrens hospital. PATIENTS All patients who underwent pharyngeal flap or sphincter pharyngoplasty from 1990 to 1995. METHODS Perceptual speech analysis was used to assess severity of VPI, presence of nasal air emissions and quality of nasal resonance (hyper, hypo, or normal). Pre-operative measures of velopharyngeal function were based upon nasendoscopy and videofluoroscopic speech assessment. Recommendations for management were made by the attending surgeon. Complications of hyponasality and obstructive sleep symptoms (OSS) were noted. Patient characteristics were compared using univariate analysis. RESULTS Sixteen patients underwent sphincter pharyngoplasty and 18 patients underwent superiorly based pharyngeal flap. Patients were similar in terms of lateral pharyngeal wall medial motion and palatal elevation. The groups were also similar with regard to VPI severity, though there was a trend for more severe VPI in patients undergoing sphincter pharyngoplasty than pharyngeal flap (50 vs. 33.3%, respectively). Patients with pharyngoplasty had a higher rate of resolution of VPI than those who had pharyngeal flap (50 vs. 22.2%, respectively), although this was not statistically significant. Post-operative hyponasality and obstructive sleep symptoms were present in both groups. However, only patients who underwent PF and had postoperative OSS had obstructive sleep apnea (OSA). CONCLUSIONS There were no detectable anatomic differences between treatment groups implying that treatment selection during the study period was not guided by strict anatomic criteria. Sphincter pharyngoplasty may have a higher success rate with a lower risk of OSS.

Furlow palatoplasty for management of velopharyngeal insufficiency: a prospective study of 148 consecutive patients.

Background: The objectives of the study were to describe speech outcomes in a large series of patients undergoing Furlow palatoplasty for management of velopharyngeal insufficiency and to test whether preoperative velopharyngeal gap size and other patient characteristics significantly affect those outcomes. Methods: Data collected included age at the time of surgery, surgeon, type of cleft, syndrome diagnosis, preoperative velopharyngeal gap size as determined by videonasendoscopy, and preoperative and postoperative perceptual speech assessments. Descriptive statistics were generated and ordinal logistic regression on the outcome variable, postoperative velopharyngeal insufficiency severity score, was performed. Results: In this series of 154 patients, 148 had complete perceptual speech data. Of these 148 patients, 72 percent had improvement in velopharyngeal insufficiency severity after the procedure and 56 percent had complete resolution of velopharyngeal insufficiency. Postoperative insufficiency was scored as none or minimal (i.e., resolution) in 38 of 52 patients (73 percent) with a small preoperative velopharyngeal gap, 26 of 51 patients (51 percent) with a moderate preoperative gap, and four of 21 patients (19 percent) with a large preoperative gap. Preoperative velopharyngeal gap size was significantly associated (p < 0.0001) with postoperative insufficiency on ordinal multivariate logistic regression after controlling for preoperative insufficiency and other covariates. There was not a significant association between syndrome diagnosis, age at Furlow palatoplasty (younger than 5 years versus older), gender, surgeon, or presence of submucous cleft palate and postoperative speech outcome, in either the unadjusted or adjusted analyses. Conclusions: Preoperative velopharyngeal gap size, as determined with nasendoscopy, was significantly associated with postoperative velopharyngeal insufficiency severity after Furlow palatoplasty. Small gap size is associated with a greater likelihood of resolution.

A comparison of nasendoscopy and multiview videofluoroscopy in assessing velopharyngeal insufficiency.

OBJECTIVES: 1) To compare nasendoscopy (NE) and multiview fluoroscopy (MVF) in assessing velopharyngeal gap size; and 2) to determine the relationship between these assessments and velopharyngeal insufficiency (VPI) severity. STUDY DESIGN AND SETTING: Retrospective review of consecutive patients with VPI at a tertiary care childrens hospital, assessed with NE and MVF between 1996 and 2003. RESULTS: 177 subjects. NE and MVF gap areas were correlated (R = 0.34, 95% CI 0.26-0.41). In adjusted analysis, VPI severity was associated with: 1) NE gap area (OR = 2.78, 95% CI 1.96-3.95), 2) MVF gap area (OR 1.64, 95% CI 1.37-1.95), 3) age <5 years (OR 3.30, 95% CI 1.47-7.38), and 4) previously repaired cleft palate (OR 0.48, 95% CI 0.25-0.94). CONCLUSIONS AND SIGNIFICANCE: NE and MVF assessments provide complementary information and are correlated. Both are associated with VPI severity. However, the “birds-eye view” provided by NE has a stronger correlation with VPI severity than MVF.

Sphincter pharyngoplasty: Speech outcome and complications

Objectives: To assess the speech outcomes and complications in children who had undergone sphincter pharyngoplasty (SP) for management of velopharyngeal insufficiency (VPI). Study Design: Retrospective chart review. Methods: Charts from patients who had sphincter pharyngoplasty between January 1993 and June 1996 were reviewed. Syndrome diagnosis and presence of repaired cleft palate were reviewed. Preoperative speech assessment, videofluoroscopic and nasopharyngoscopic evaluations, age at time of surgery, and postoperative speech assessments were reviewed for all patients. Postoperative videofluoroscopy and nasopharyngoscopy were performed for those patients who had persistent VPI. Obstructive sleep symptoms were also assessed. Results: Thirty patients were identified; six patients had no follow‐up evaluation, leaving 24 patients included in this study. Average follow‐up was 11.7 months (range, 2–35 mo). Velocardiofacial syndrome (VCFS) was the most commonly identified syndrome. Postoperatively, 15 of 24 patients (62.5%) had complete resolution of their VPI; five of 24 (20.8%) had significant improvement; one of 24 (4.2%) had minimal to no change; and three of 24 (12.5%) were hyponasal. Of the six patients with some degree of persistent VPI, three underwent revision surgery. All three patients had complete resolution of their VPI after revision surgery, resulting in an overall success rate of 18 of 24 (75%). Conclusions: Sphincter pharyngoplasty has wide application in the management of children with VPI, including those with VCFS. The procedure is readily modified to accommodate an individual patients needs as determined by preoperative VPI evaluation. A modification of the procedure is described to minimize the risk of postoperative airway obstruction and hyponasality, both regarded as airway complications of sphincter pharyngoplasty.

Recent Epidemiology of Pediatric Cochlear Implantation in the United States: Disparity Among Children of Different Ethnicity and Socioeconomic Status†

Objectives/Hypothesis: Congenital severe to profound sensorineural hearing loss (SNHL) is found in higher proportions of children with minority and/or lower socioeconomic status (SES). Cochlear implants were approved by the U.S. Food and Drug Administration for use in children with bilateral severe to profound SNHL in 1990. The objectives of the study were as follows: 1) to study the epidemiology of pediatric cochlear implantation, assessing whether cochlear implant technology is provided to children with severe to profound SNHL in proportion to their racial/ethnic or SES, and 2) to compare data provided by a national health care database with data provided by cochlear implant manufacturers.

Semicircular canal dehiscence in the pediatric population

OBJECTIVES (1) Identify the existence of semicircular canal bony dehiscence in the pediatric population; (2) determine the frequency of this finding in children over 3 years of age who have had temporal bone computed tomography (CT) imaging; (3) correlate the clinical history and audiological data to CT findings in this population. DESIGN AND SETTING Retrospective review of temporal bone CT scans performed at a tertiary care childrens hospital and retrospective chart review. PATIENTS Children over 3 years of age who underwent a temporal bone CT scan between January 2006 and December 2006. RESULTS Review of 131 temporal bone CT scans using multiplanar imaging techniques revealed evidence of semicircular canal bony dehiscence in 18 children older than 3 years of age. Dehiscence of the superior semicircular canal was identified in 14 patients while dehiscence of the posterior semicircular canal was identified in 5 patients. One patient had both the superior and posterior semicircular canal dehiscences. Retrospective chart review comparing children with and without semicircular canal dehiscence showed no significant difference in clinical history or audiological data. CONCLUSIONS Semicircular canal dehiscence exists in the pediatric population. In this series, 18 of 131 temporal bone CT scans were positive for bony dehiscence of the superior or posterior semicircular canals. Further study is required to determine the clinical significance of this radiographic finding.

Recurrent Thyroglossal Duct Cysts: A 23-Year Experience and a New Method for Management

Objectives: We present an experience in the management of primary and recurrent thyroglossal duct cysts (TGDCs) and describe a novel method for recurrent TGDC removal. Methods: We performed a retrospective review of TGDC surgery at Childrens Hospital in Seattle from 1980 to 2003. The surgical techniques for primary and recurrent TGDCs and the factors associated with TGDC recurrence were evaluated and analyzed. Results: During the study period, 231 patients underwent 296 TGDC surgeries. Thirty-four of the 231 patients (15%) underwent a total of 88 procedures for recurrent TGDCs. Successful procedures used for secondary TGDC management included central neck dissection with directed base of tongue (BOT) excision in 6 of 9 patients (67%), secondary Sistrunk operation with limited BOT resection in 12 of 27 patients (44%), revision Sistrunk operation with BOT dissection in 7 of 11 patients (64%), and suture-guided transhyoid pharyngotomy in 8 of 8 patients (100%). Ten of the 231 patients (4%) had initial TGDC incision and drainage and then underwent a total of 21 procedures, excluding the incision and drainage. The factors associated with TGDC recurrence were inaccurate initial diagnosis (17 of 34 or 50%), infection (5 of 34 or 15%), unusual TGDC presentation (5 of 34 or 15%), and lack of BOT musculature removal (7 of 34 or 20%). The level of surgeon training affected the surgical outcome. Conclusions: Successful TGDC treatment requires consideration of factors associated with recurrence. Recurrent TGDCs can be treated by several methods, including suture-guided transhyoid pharyngotomy.