Otologic and audiologic outcomes with the Furlow and von Langenbeck with intravelar veloplasty palatoplasties in unilateral cleft lip and palate.

OBJECTIVE: Cleft palate increases the risk of chronic middle ear disease and hearing loss. The goal of this report was to determine which of two palate surgeries and which timing of palate surgery were associated with better otologic and audiologic outcomes in children with unilateral cleft lip and palate at 5 to 6 years of age. DESIGN: Subjects were randomly assigned to the von Langenbeck with intravelar veloplasty or Furlow palate repair, to palate surgery at 9 to 12 months or 15 to 18 months of age, and to the Spina or Millard lip repair. SETTING: Centralized, tertiary care craniofacial treatment center. PATIENTS: A total of 673 infants with unilateral cleft lip and palate. INTERVENTIONS: Palate and lip were repaired using established techniques. Serial otoscopic and audiometric evaluations were performed. MAIN OUTCOME MEASURES: Hearing and otoscopic findings at 5 to 6 years old. RESULTS: There were 370 children available for analysis. Hearing and need for tympanostomy tube placement did not differ by palatoplasty, age at palatoplasty, cheiloplasty, or surgeon. Risk of developing cholesteatoma or perforation was higher with Millard cheiloplasty (odds ratio  =  5.1, 95% confidence interval  =  1.44 to 18.11, p  =  .012). Type and age at palatoplasty were not significantly associated with either the rate of developing these sequelae or the rate of achieving bilaterally normal hearing and ear examinations. CONCLUSIONS: Type of palatoplasty did not influence otologic and audiologic outcomes in 5- to 6-year-olds with unilateral cleft lip and palate. The potential influence of lip repair on otologic outcomes warrants further investigation.

Symptomatic atlantoaxial instability in an adolescent with trisomy 21 (Down's syndrome).

Atlantoaxial instability (AAI) occurs in 15% of children with Trisomy 21. Health supervision guidelines were revised by the American Academy of Pediatrics in 2011 to reflect advances in care for children with special health care needs (CSHCN). Previous guidelines recommended cervical spine radiological screenings in preschool years to evaluate for atlantoaxial instability. For patients with negative screening, re-screening was recommended if they wished to compete in the Special Olympics, or became symptomatic. We present the case of an adolescent who developed a symptomatic atlantoaxial dislocation despite previous negative radiological screening at the age three (under the 2001 guidelines). This case report highlights the revisions in the 2011 guidelines for health supervision and anticipatory guidance. It underlines the need for a high index of suspicion if symptoms develop. It also addresses the need for a medical home for CSHCN, with health care providers who know the child's baseline health status.

Improving developmental screening in pediatric resident education.

OBJECTIVE: Given that pediatricians cite low competency in developmental screening, this study aims to effectively teach screening to residents. DESIGN: Using a quasi-experimental design, residents received an educational module and one-on-one teaching of 3 validated developmental screeners (Denver II, ASQ [Ages and Stages Questionnaire], and PEDS [Parents' Evaluation of Developmental Status]), with subsequent independent use with all 3 screeners with their own continuity patients. Outcome measures included changes in knowledge, skills, and preferences. RESULTS: All residents achieved significantly increased skills with all screeners. They strongly preferred the ASQ (70%), citing that this taught them normal (30.2%) and pathological (27.9%) development while negatively noting time (72.1%), scheduling issues (30.2%), and difficulties with child cooperation (20.9%). Knowledge specifics did not significantly increase. CONCLUSIONS: In-depth developmental screening education revealed marked improvement in skills and preferences. These evaluations led to full adoption of the ASQ in resident clinics. Future research must test if effective development teaching in residency leads to increased routine screenings in practice.

Quality of life information and trust in physicians among families of children with life-limiting conditions.

PURPOSE: To examine information that parents of children with life-limiting conditions want to discuss with children's physicians to assist decision-making, and whether the desire for this information is associated with parents' trust in physicians. STUDY DESIGN: A cross-sectional study using a telephone survey. PATIENTS AND METHODS: Subjects comprised a random sample of 266 parents whose children were enrolled in Florida's Medicaid Program. Parents were asked if they wanted to discuss information related to their children's treatment, including quality of life (QOL), pain relief, spiritual beliefs, clinical diagnosis/laboratory data, changes in the child's behavior due to treatment, changes in the child's appearance due to treatment, chances of recovery, and advice from the physician and family/friends. The Wake Forest Physician Trust Scale was used to measure parents' trust in physicians. We tested the relationships between parents' age, race/ethnicity, education, parent-reported children's health status, and the desired information. We also tested whether the desire for information was associated with greater trust in physicians. RESULTS: Most parents wanted information on their children's QOL (95%), followed by chance of recovery (88%), and pain relief (84%). Compared with nonHispanic whites, nonHispanic blacks and Hispanics showed a greater desire for information and a chance to discuss QOL information had greater trust in their children's physicians than other information after adjusting for covariates (P < 0.05). CONCLUSIONS: Among children with life-limiting conditions, QOL is the most frequently desired information that parents would like to receive from physicians as part of shared decision-making. Parents' desire for QOL information is associated with greater trust in their children's physicians.

Longitudinal study of growth of children with unilateral cleft-lip palate from birth to two years of age.

OBJECTIVE: To study the growth of children with complete unilateral cleft lip and palate (UCLP) from birth to 2 years of age and to construct specific UCLP growth curves. DESIGN: Physical growth was a secondary outcome measure of a National Institutes of Health-sponsored longitudinal, prospective clinical trial involving the University of Florida (United States) and the University of São Paulo (Brazil). PATIENTS: Six hundred twenty-seven children with UCLP, nonsyndromic, both genders. METHODS: Length, weight, and head circumference were prospectively measured for a group of children enrolled in a clinical trial. Median growth curves for the three parameters (length, weight, head circumference) were performed and compared with the median for the National Center for Health Statistics (NCHS) curves. The median values for length, weight, and head circumference at birth and 6, 12, 18, and 24 months of age were plotted against NCHS median values and statistically compared at birth and 24 months. SETTING: Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, Brazil (HRAC-USP). RESULTS: At birth, children of both genders with UCLP presented with smaller body dimensions in relation to NCHS median values, but the results suggest a catch-up growth for length, weight, and head circumference for girls and for weight (to some degree) and head circumference for boys. CONCLUSIONS: Weight was the most compromised parameter for both genders, followed by length and then head circumference. There was no evidence of short stature. This study established growth curves for children with UCLP.