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 patient's 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.

Lymphatic malformations of the head and neck. A proposal for staging.

OBJECTIVE: To propose a staging system for patients with lymphatic malformations of the head and neck. DESIGN: Retrospective chart review. PATIENTS: Fifty-six patients were treated for lymphatic malformations from 1983 to 1993 at Children's Hospital and Medical Center, Seattle, Wash. The charts were reviewed for anatomic location of the lesion, preoperative and postoperative complications, number of procedures to control disease, long-term sequelae, and persistence of disease. Lesions were characterized as being unilateral or bilateral and suprahyoid and/or infrahyoid. The five patient groups were then compared with respect to the above categories. RESULTS: Preoperative complications reviewed include preoperative infection, respiratory embarrassment necessitating airway intervention, and feeding difficulties. Postoperative complications assessed were cranial nerve injury, wound infection, and seroma formation. Long-term sequelae included malocclusion, speech delay, and cosmetic deformity. The rate of persistent disease was also assessed. A staging system was developed based on a progression of extent of disease. Stage I patients (n = 12) had unilateral infrahyoid disease and a 17% incidence of complications overall. Stage II patients (n = 17) had unilateral suprahyoid disease and a 41% incidence of complications. Stage III patients (n = 15) had unilateral suprahyoid and infrahyoid disease and a complication rate of 67%. Stage IV patients (n = 5) with bilateral suprahyoid disease had a complication rate of 80%, while stage V patients (n = 6) with bilateral suprahyoid and infrahyoid disease had a 100% incidence of complications. CONCLUSION: Anatomic location of lymphatic malformations of the head and neck can be used to predict prognosis and outcome of surgical intervention.

Bioelectric properties of gerbil middle ear epithelia.

Biochemical analysis of middle ear effusions provides indirect evidence of epithelial function, but no direct studies of middle ear epithelial function have been published. This study uses cell culture techniques to study the bioelectric properties of these cells and their response to pharmacologic agents. Middle ear epithelial cells were isolated by protease and DNase digestion and cultured on collagen matrices. A transepithelial potential difference (PD) measured by calomel half cells peaked by day 8 in culture (PD = -8.6 +/- 1.6 mV). Resistance and short-circuit current (ISC) were calculated to be 307 +/- 46 omega/cm2 and 28 +/- 4.1 A/cm2, respectively. At peak PD, cultures were mounted in flux chambers (Ussing), and pharmacologic agents were applied. Amiloride (10(-4) mol/L), an inhibitor of Na transport, decreased ISC by 52.3% +/- 4.9% but had no effect when added basolaterally. Carbachol (10(-4) mol/L), a cholinergic agonist, had no effect when added to the apical bath but increased ISC by 62.9% +/- 12.4% when added basolaterally. Isoproterenol (10(-4) mol/L), a beta-adrenergic agonist, increased ISC by 4.8% +/- 1.8% when added apically but had no effect when added basally. These results indicate that the epithelium lining the middle ear plays a role in regulating the quantity and composition of liquid in the middle ear by ion transport and that cell culture may be useful in studying this phenomenon.

Measuring quality of life in children with obstructive sleep disorders.

OBJECTIVE: To validate a disease-specific health-related quality of life (HRQOL) instrument for children with obstructive sleep disorders (OSDs). DESIGN: Prospective cohort study using a 6-item health-related instrument (OSD-6). SUBJECTS: One hundred caregivers of patients with OSDs secondary to adenotonsillar hypertrophy (age range, 2-12 years) from 2 tertiary care, pediatric otolaryngology practices. INTERVENTION: The OSD-6 was administered on initial presentation and 4 to 5 weeks after adenotonsillectomy. A subset of patients repeated the OSD-6 within 3 weeks after presentation to assess test-retest reliability. MAIN OUTCOME MEASURES: Test-retest reliability, internal consistency, construct validity, and responsiveness to clinical change of the OSD-6 score. RESULTS: Test-retest reliability was good (intraclass correlation coefficient = 0.74). Median OSD-6 score was 4.5 (0- to 6-point scale) with higher scores indicating poorer quality of life (QOL). Construct validity was demonstrated by the moderate correlation between OSD-6 score and global adenoid and tonsil-related QOL (R = -0.62), strong correlation between the OSD-6 change score and change in global adenoid and tonsil-related QOL (R = -0.63), and the moderate correlation between the change score and parent estimate of clinical change (R = 0.40). The mean change in OSD-6 score after adenotonsillectomy was 3.0 (95% confidence interval, 2.7-3.4). The mean standardized response was 2.3 (95% confidence interval, 1.9-2.7) indicating the instrument's large responsiveness to clinical change. The change score was very reliable (R = 0.85). CONCLUSIONS: The OSD-6 is a reliable, responsive, easily administered instrument. It is valid for detecting change after adenotonsillectomy in children with OSDs. Arch Otolaryngol Head Neck Surg. 2000;126:1423-1429

Tongue lesions in the pediatric population.

OBJECTIVE: To describe the spectrum of pediatric tongue lesions treated surgically at Columbia-Presbyterian Medical Center from January 1990 to December 1999. STUDY DESIGN AND SETTING: Retrospective case-series at the pediatric hospital of a tertiary care, academic medical center. RESULTS: Seventeen patients were identified. Their ages ranged from 1 to 132 months (median, 7 months). Eight lesions were located anteriorly: mucous cyst (1), polyp (1), chronic inflammatory mass (1), hamartoma (1), squamous papilloma (2), cavernous hemangioma (1), and vascular malformation (1). Four lesions were located posteriorly: teratoma (1), glial choristoma (1), osseous choristoma (1), and benign epithelial cyst (1). Finally, there were 5 diffuse lesions including macroglossia (4) and massively infiltrating congenital lymphatic malformation (1). Symptoms included respiratory distress (3) and dysarthria (3); all other children were asymptomatic. CONCLUSIONS: This series revealed an interesting spectrum of rare solid tumors; compared with other large series, fewer lymphatic and vascular malformations were seen. Presenting symptoms, differential diagnosis, and surgical approach were differentiated according to lesion location.

Congenital cystadenoma of the tongue in a neonate case report with review of literature.

We present a congenital cystadenoma of the tongue in a neonate, which presented at birth. Cystadenomas are uncommon tumors that form from salivary gland duct tissue and are more commonly seen in adults. This is the youngest case to be reported in the English literature. A review of literature with differential diagnosis and management is presented.

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 children's 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.