Risk Assessment of Sleep Disordered Breathing in Cleft Lip and/or Palate.

OBJECTIVE: Children with cleft lip and/or palate (CL/P) are at increased risk for Sleep Disordered Breathing (SDB), particularly Obstructive Sleep Apnea (OSA). At our institution, routine screening for SDB is performed using the Chevrin Pediatric Sleep Questionnaire (PSQ). This analysis is a practice audit looking at the outcomes of screening children with CL/P. DESIGN/SETTING/PATIENTS/PARTICIPANTS: A single-center, retrospective analysis was done of all non-syndromic patients with CL/P over the age of 36 months over a 4-year period. Children with known OSA were eliminated from analysis. MAIN OUTCOME MEASURES: Univariate logistic regression was used to assess predictors for SDB (PSQ score > 8) amongst various patient, disease, and treatment characteristics. Outcomes of those screened were tracked. RESULTS: Of the 239 patients in the study cohort, 43 (18%) had positive PSQs. These subjects were more likely to have class III dental occlusion with maxillary retrusion (OR = 2.65, 95% CI: 1.2-5.8, p = 0.02). There were no differences amongst age, type of cleft, Veau classification, BMI, or history of pharyngeal surgery. One third of the group did not complete recommended testing. Twenty-five subjects with positive sleep screening underwent subsequent polysomnography and 21 (84%) had OSA. CONCLUSION: Routine screening reveals a significant proportion of patients with CL/P with symptoms suggestive of OSA. While several patients did not complete confirmatory testing, those who completed a PSG had a high rate of identification of OSA. After excluding children with known OSA, patients with SDB are also likely to have class III dental occlusion and maxillary retrusion.

Orofacial Manifestations of Stickler Syndrome: An Analysis of Speech Outcome and Facial Growth After Cleft Palate Repair.

PURPOSE: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair. METHODS: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth. RESULTS: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant. CONCLUSIONS: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.

Long Term Speech Outcomes Following Midface Advancement in Syndromic Craniosynostosis.

Midface advancement by distraction osteogenesis (DO) is commonly performed in patients with craniosynostosis for indications including midface hypoplasia, exorbitism, obstructive sleep apnea, class III malocclusion, and overall aesthetic facial deficiency. There is evidence to suggest that maxillary LeFort I advancement increases the risk of velopharyngeal dysfunction in the cleft palate population, yet few studies have investigated changes in speech following LeFort III or monobloc midface advancement in patients with syndromic craniosynostosis. The purpose of this study was to examine the effect of midface DO on speech as indicated by the Pittsburgh Weighted Speech Score in patients with Apert, Crouzon, and Pfeiffer Syndrome. Among 73 midface advancement cases performed during the study period, 19 cases met inclusion criteria. Overall, the highest post-advancement Pittsburgh Weighted Speech Score (PWSS) was significantly higher than the pre-advancement PWSS (0.52 versus 2.42, P = 0.01), indicating an acute worsening of VPI post-advancement. Specifically, the PWSS components nasal emission and nasality were significantly higher post-advancement than pre-advancement (nasal emission: 1.16 versus 0.21, P = 0.02) (nasality: 0.68 versus 0.05, P = 0.04). However, there was no significant difference between pre-advancement PWSS and the latest post-advancement PWSS (P = 0.31). Midface distraction is associated with an acute worsening of VPI post-operatively that is followed by improvement, and often resolution over time. Future work with additional patient accrual is needed to determine the effect of different advancement procedures and syndromes on VPI rates and profundity.

Change in Initial Tympanostomy Tube Placement Timing Yields Decreased Burden of Care Without Increased Complications in Patients With Cleft Lip and Palate.

OBJECTIVE: To report a practice audit of the consequences of a change in protocol in the timing of placement of tympanostomy tubes in infants with cleft lip and palate. PARTICIPANTS: All children with a diagnosis of cleft lip and palate, treated between November 1998 and May 2006 under the old protocol, and between December 2012 and July 2016 under a new protocol. Under the old protocol, tympanostomy tubes were first inserted at the time of lip repair at around age 2 months. Under the new protocol, tympanostomy tubes were deferred until the time of palate repair around the age of 9 months. Children with syndromic diagnoses other than Stickler syndrome and Van der Woude syndrome, and children who failed newborn hearing screen were excluded. MAIN OUTCOME MEASURES: Incidence of otorrhea from birth to 6 months after palate repair and presence of hearing loss at ages 1 and 2. RESULTS: Deferral of tympanostomy tubes until the time of palate repair decreases the burden of care due to otorrhea as compared to early tympanostomy tubes at the time of lip repair. There was no significant difference in the incidence of hearing thresholds at or below 15 dB at age 1 or 2. CONCLUSIONS: Placement of tympanostomy tubes at the time of palate repair balances the goals of minimizing the adverse effects from middle ear effusion and minimizing the burden of care on our patients and their families.

The Effects of a Nutrition Media Literacy Intervention on Parents' and Youths' Communication about Food.

Interventions addressing links between media exposure and obesity risk for school-age youth have not explicitly addressed the role of family communication about media. Youths' influence attempts on parents to purchase advertised foods can create conflict and negatively affect parental food choices. This study tested whether a family-based media literacy curriculum improves parents' media management skills and decreases youths' susceptibility to appealing but unrealistic food marketing. A matched-group pretest/posttest field experiment of parent-youth dyads with control group (N = 100 dyads, youth M = 11 years of age) tested the six-session curriculum. Hypotheses were analyzed using a Bayesian structural equation model. The curriculum increased parents' active negative mediation to foster youths' critical thinking about food marketing, b* = 0.35, 95% CCI [0.17, 0.50], increased parent Efficacy for making healthy dietary changes for their families, b* = 0.59, 95% CCI [0.41, 0.75], and fostered family discussion about nutrition labels (total effect = 0.22). Additionally, cumulative influences of Perceived Desirability and Wishful Identification on youths' requests for marketed foods were reduced (total effect = 0.04). Media literacy education can empower parents and improve youths' critical thinking to reduce effects of food marketing on families and improve use of media to obtain nutrition information.

International Adoptees With Cleft Lip and/or Palate: Lessons Learned From a Growing Population.

BACKGROUND: International adoptees with cleft lip and palate (CLP) are a growing population in the United States. They represent a clinical challenge, presenting at various ages and stages of cleft repair. METHODS: A retrospective review of patients seen at the CLP Program at the Children's Hospital of Philadelphia (CHOP) between 1998 and 2012 with a history of international adoption was performed. Demographics, surgical histories, and long-term speech outcomes were reviewed. RESULTS: Seventy-four female and 77 male patients were evaluated. Patients were adopted at an average age of 2.3 years (range, 0.4-8.6 years); 80.8% (n = 122) of patients were adopted from China. The rate of international cleft adoption increased by approximately 1.5 patients per year (r = 0.7739, P < 0.001); 13.2% (n = 19) of all subjects with cleft palates had oronasal fistulas (ONFs) that required repair. The ONF rates for primary palatoplasties at CHOP were significantly lower compared to both preadoption repairs (P = 0.002) and postadoption repairs at outside hospitals (P = 0.01); 14.8% (n = 21) of all patients had secondary surgeries for velopharyngeal incompetence (VPI). Rates of secondary surgery for VPI were also significantly lower for primary palatoplasties at CHOP compared to both preadoption repairs (P = 0.0018) and postadoption repairs at outside hospitals (P = 0.0033). CONCLUSIONS: International adoptees with CLP are a growing population and are clinically challenging with high ONF rates and high secondary surgery rates for VPI. We recommend expedited repair of unoperated cleft palates in adoptees older than 18 months. Adopted patients with CLP should be rigorously evaluated for the need for speech therapy and secondary surgeries to correct for VPI.

The Children's Hospital of Philadelphia modification of the Furlow double-opposing z-palatoplasty: long-term speech and growth results.

Of the 261 nonsyndromic patients we studied, over 90% had minimal or absent hypernasality, almost 86% had inconsistent or no nasal emission, and 95% had no articulation errors related to velar function. The patients with a Pittsburgh score indicating an incompetent velopharyngeal mechanism comprised only about 6% of the group. Ninety-four percent had a socially functional speech quality. Secondary surgery was done in 6.5% of patients and was done or was recommended in about 8% of patients. Patients with isolated cleft palate seemed to do less well, although their outcomes were not statistically different from those with complete unilateral and bilateral clefts. Relaxing incisions have kept our fistula rate to an acceptably low rate of 6.8%. No major soft palate dehiscences or hard palate flap losses have occurred. The speech outcomes we are achieving are improved over our historical results and compared with published reports using nondouble reversing z-palatoplasty techniques. Similar outcomes with the Furlow repair have been confirmed. Maxillary growth, occlusion, and the need for orthognathic surgery do not seem to be influenced by the CHOP modification of the Furlow double-opposing z-palatoplasty. These modifications facilitate a tension free-closure and a low fistula rate.

Long term speech outcomes following late cleft palate repair using the modified Furlow technique.

OBJECTIVES: Published reports and previous studies from our institution have reported worse overall speech results, including significantly higher rates of persistent articulation errors, in patients undergoing palatoplasty at age >18 months. This study further investigates the effects of late repair on long term speech outcomes. METHODS: A retrospective review was performed of non-syndromic patients undergoing primary palatoplasty at age >18 months between 1980 and 2006 at our institution. Longitudinal speech results were compared based on reason for late repair and age at repair. RESULTS: Forty-one patients were greater than 18 months of age at the time of palatoplasty, and 24 fit criteria for longitudinal data analysis. There was a statistically significant improvement in nasality scores at Time Point 1 for international adoptees compared to the non-adopted population (p=0.04). Patients with submucosal clefts were found to have significantly less severe nasal emission scores at Time Point 1 compared to those with overt clefts (p=0.04), but not at Time Point 2. There were no significant differences between scores if repair was performed between 18 and 36 months or >36 months, nor any difference in incidence of articulation errors between subgroups of patients with late repair at either Time Point. CONCLUSION: Our experience demonstrates that cleft palate repair after 18 months of age is associated with a significantly increased incidence of articulation errors associated with VPI, irrespective of reason for late repair, highlighting the persistence of learned compensatory behaviors in speech and the importance of proceeding with early repair.