Mary Berger

A retrospective analysis of revision sphincter pharyngoplasty.

This retrospective study was undertaken to determine the revision rate for dynamic sphincter pharyngoplasty (DSP) at the University of Michigan Medical Center to analyze the determinants contributing to the need for revision pharyngoplasties, and ultimately to improve primary pharyngoplasty to avoid the need for revision. The records of 30 children with repaired palatal clefts who presented with velopharyngeal insufficiency and hypernasal speech, and who underwent DSP from January 1988 through July 1994 were reviewed. Clinical follow-up ranged from 6 to 48 months (mean, 20.2 months). Seven of the original 30 patients (23%) had persistent, moderate-to-severe hypernasality that required reoperation, while 1 patient (3%) demonstrated hyponasality requiring revision. Seven of 8 patients who underwent revision pharyngoplasty had acceptable speech after revision. Dehiscences, low-lying pharyngoplasty flaps, and end-to-end suturing of the flaps were the main determinants resulting in the need for revision. In our study, female gender and older age was associated with a higher success of primary operation.

Posterior pharyngeal fat grafting for velopharyngeal insufficiency.

Objective To determine if autologous fat grafting to the posterior pharynx can reduce hypernasality in patients with cleft palate and mild velopharyngeal insufficiency (VPI). Design Retrospective case series. Setting Tertiary care center. Patients Eleven patients with cleft palate status after palatoplasty (with or without secondary speech surgery) with nasendoscopic evidence of VPI. Interventions Autologous fat was harvested and injected into the posterior pharynx under general anesthesia. Main Outcome Measures Pre- and postoperative subjective, nasometry, and nasendoscopy data. Apnea-hypopnea indices (AHIs) were also assessed. Comparisons were made using Fishers exact test, Students t tests, and relative risk (RR) assessments. Results An average of 13.1 mL of fat was injected (range: 5 to 22 mL). Mean follow-up was 17.5 months (range: 12 to 25 months). Statistically significant improvements in speech resonance were identified in nasometry (Zoo passage; p = .027) and subjective hypernasality assessment (p= .035). Eight of the patients (73%) demonstrated normal speech resonance after posterior pharyngeal fat grafting (PPFG) on subjective or objective assessment (p = .001). All five patients with previous secondary speech surgeries demonstrated normal speech resonance on similar assessment (RR = 1.8; p = .13). Complete velopharyngeal closure was observed in seven patients on postoperative nasendoscopy. No changes in AHIs were observed (p=.581). Conclusion PPFG may be best used as an adjunct to secondary speech surgery. In this series, PPFG was not accompanied by the negative sequelae of hyponasality, sleep apnea, or airway compromise.

Skew deviation as the initial manifestation of left paramedian thalamic infarction.

We describe a 73-year-old man who developed diplopia as the initial manifestation of a left thalamic infarction. By the time he reached the emergency department, clouded consciousness precluded localization of the lesion. Results of brain MRI were initially interpreted as negative. Ophthalmologic examination several hours later disclosed a small vertical ocular misalignment attributed to skew deviation. This finding led to careful scrutiny of the upper brainstem on MRI. Comparison of the diffusion, apparent diffusion coefficient, and exponential apparent diffusion coefficient MRI studies allowed a diagnosis of subtle left thalamic infarction. The recognition of skew deviation in this setting is important because it may be the most specific indicator of a brainstem lesion.

Obstructive sleep apnea pretreatment and posttreatment in symptomatic children with congenital craniofacial malformations

STUDY OBJECTIVES Obstructive sleep symptoms are common in children with craniofacial malformations (CFM). However objective data about obstructive sleep apnea (OSA) is still limited. The aims of this study were to investigate the frequency of OSA in symptomatic children with CFM and to determine improvement in severity of OSA after treatment. METHODS Symptomatic children with CFM referred for a diagnostic polysomnogram (PSG) were identified. Obstructive sleep apnea was defined as an apnea/hypopnea index (AHI) ≥ 1, with moderate/severe OSA as an AHI ≥ 5. RESULTS Overall, 151 symptomatic children were identified; 87% were diagnosed with OSA, of whom 24% had moderate-to-severe OSA. Children with syndromic CFM, compared to non-syndromic CFM, were more likely to have an AHI ≥ 5 (syndromic 33% vs. non-syndromic 15%, p = 0.02). Of the 131 children with OSA, 64 were treated and 32 returned for a posttreatment PSG, with 22 treated with either positive airway pressure (PAP) or adenotonsillectomy (AT). Children treated with PAP demonstrated a decrease in AHI from 6.2 to 3.5 (p = 0.057) and an increase in SpO2 from 89.1% to 91.1% (p = 0.091). There were no significant improvements for those in the AT group for either AHI (2.5 to 1.8, p = 0.19) or SpO2 (90.4% to 91.3%, p = 0.46). Normalization of the AHI (AHI < 1) occurred in only one child in each group (7% and 14% of the PAP and AT groups, respectively). CONCLUSIONS The vast majority of children with CFM referred for OSA evaluation are found to have objective evidence of OSA and a quarter of children have moderate-to-severe OSA. It is likely that many children with underlying OSA are not identified and referred for evaluation. Residual OSA after treatment is common in children with CFM.

Timing of Furlow Palatoplasty for Patients With Submucous Cleft Palate

Background: Submucous cleft palate (SMCP) is the most common form of cleft involving the posterior palate, resulting in variable degrees of velar dysfunction and speech disturbance. Although early surgical intervention is indicated for patients with true cleft palate, the indications for palatoplasty and timing of surgical intervention for patients with SMCP remain controversial. Methods: Twenty-nine patients with SMCP were retrospectively reviewed. Patients treated with Furlow palatoplasty were dichotomized based on patient age at the time of surgical correction into early speech development and late speech development. Primary outcome measures included standardized assessments of hypernasal resonance and quantitative pre- and postoperative nasometry scores. Patients managed nonoperatively were included for comparison of early and late speech outcomes. Results: Both early and late groups demonstrated improvement in qualitative assessment of hypernasal resonance following Furlow palatoplasty. Early and late groups also had significant improvement in pre- to postoperative nasometry scores from 7.4 to 2.3 SD from norm (P = .01) and 6.0 to 3.6 SD from norm (P = .02), respectively. There was no difference in postoperative nasometry scores between early and late groups, 2.3 and 3.6 SD (P = .12). Conclusion: Furlow palatoplasty significantly improves the degree of hypernasality in patients with SMCP based on pre- and postoperative nasometry scores and on qualitative assessment of hypernasality. There were no differences in speech outcomes based on early compared with late operative intervention. Therefore, early palatal repair is not obligatory for optimal speech outcomes in children with SMCP and palatoplasty should be deferred until the emergence of overt velopharyngeal insufficiency.

Association between symptoms of sleep-disordered breathing and speech in children with craniofacial malformations

STUDY OBJECTIVE Sleep-disordered breathing (SDB) and speech difficulties are common problems in children with craniofacial malformations (CFM). The present study was designed to investigate whether resonance issues identified during speech assessment are associated with parental report of SDB symptoms in children with CFM. METHODS Children aged 2-18 years with congenital CFM attending at the Craniofacial Anomalies Program from March 2007 to April 2011 were screened for SDB symptoms using the Sleep-Related Breathing Disturbance Scale of the Pediatric Sleep Questionnaire. Speech evaluation, based on the Pittsburgh Weighted Speech Scale score, was the tool used to investigate velopharyngeal dysfunction (VPD) based on speech perceptual assessment. RESULTS A total of 488 children with congenital CFM were included. Overall 81% were Caucasian and 24% were overweight/obese. Twenty-four percent of children screened positive for SDB and 35% had VPD. Children with VPD were no more likely to screen positive for SDB than children without VPD (26% vs. 23%, p = 0.38). However, children with previous sphincter pharyngoplasty (SP) were more likely to have hyponasality (51% vs. 12%, p = 0.0001) and reduced or absent nasal emission (33% vs. 16%, p = 0.008). In a logistic regression, the adjusted odds ratio for SDB for those with hyponasality was 2.10 (95%CI 1.21-3.61, p = 0.008) and for those with reduced or absent nasal emission was 1.75 (95%CI 1.06-2.88, p = 0.028). CONCLUSION Symptoms of sleep disordered breathing are common in children with craniofacial malformations especially if they have undergone sphincter pharyngoplasty; many of these children can be identified by measures of resonance on routine speech evaluation.