The goal of this prospective randomized clinical trial was to compare 2 cohorts of standardized cleft patients with regard to functional speech outcome and the presence or absence of palatal fistulae. The 2 cohorts are randomized to undergo either a conventional von Langenbeck repair with intravelar velarplasty or the double-opposing Z-plasty Furlow procedure. A prospective 2 × 2 × 2 factorial clinical trial was used in which each subject was randomly assigned to 1 of 8 different groups: 1 of 2 different lip repairs (Spina vs. Millard), 1 of 2 different palatal repair (von Langenbeck vs. Furlow), and 1 of 2 different ages at time of palatal surgery (9-12 months vs. 15-18 months). All surgeries were performed by the same 4 surgeons. A cul-de-sac test of hypernasality and a mirror test of nasal air emission were selected as primary outcome measures for velopharyngeal function. Both a surgeon and speech pathologist examined patients for the presence of palatal fistulae. In this study, the Furlow double-opposing Z-palatoplasty resulted in significantly better velopharyngeal function for speech than the von Langenbeck procedure as determined by the perceptual cul-de-sac test of hypernasality. Fistula occurrence was significantly higher for the Furlow procedure than for the von Langenbeck. Fistulas were more likely to occur in patients with wider clefts and when relaxing incisions were not used.

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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.

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 NIH sponsored longitudinal, prospective clinical trial involving the University of Florida (USA) and the University of São Paulo (Brazil). Patients 627 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 3 parameters (length, weight, head circumference) were performed and compared to the median for the National Center for Health Statistics (NCHS, 2000) curves. The median values for length, weight, and head circumference at birth, 6, 12, 18 and 24 month 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 statue. This study established growth curves for children with UCLP.

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 NIH sponsored longitudinal, prospective clinical trial involving the University of Florida (USA) and the University of São Paulo (Brazil).Patients-627 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 3 parameters (length, weight, head circumference) were performed and compared to the median for the National Center for Health Statistics (NCHS, 2000) curves. The median values for length, weight, and head circumference at birth, 6, 12, 18 and 24 month of age were plotted against NCHS median values, and statistically compared at birth and 24 months. 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. Setting-HospitalConclusions-Weight was the most compromised parameter for both genders followed by length and then head circumference. There was no evidence of short statue. This study established growth curves for children with UCLP. Many physical growth studies of children with CLP have been published (Bowers et al., 1987;Felix-Schollaart et al., 1992;Ross and Jhonston, 1972; Cunninghan and Jerome, 1997;Becker et al., 1998; Montagnoli et al., 2005, Gopinath andMuda, 2005). Although the results vary between different investigators, most of them state that these children are smaller and lighter than the control subjects, mainly during the first years of age. A catch up growth may occur later in childhood (Ross and Jhonston, 1972; Lee et al., 1996). KeywordsMany factors such as feeding problems and recurrent respiratory infections have been suggested to negatively impact normal growth. Reports indicate that growth problems are more severe in early infancy and are more frequent in children with isolated cleft palate (ICP), and cleft lip and palate (CLP), than in children with isolated cleft lip or normal (unaffected) children (Felix-Schollaart et al., 1992; Lee et al., 1996, Montagnoli et al., 2005.The limitations of previous growth studies include relatively small number of children included in their sample, possible inclusion of children with chronic health conditions and syndromes, and inclusion of different types of clefts or age ranges. Most studies were retrospective.This article is part of a series of studies that summarize an eleven-year prospective randomized clinical trial. The objective of this paper was to analyze the physical growth of the large sample of nonsyndromic children with unilateral cleft lip and palate (UCLP), from birth to two years of age, according to gender, in a longitudinal...

Velo-cardio-facial syndrome, DiGeorge syndrome, conotruncal anomaly face syndrome, tetralogy of Fallot, and pulmonary atresia with ventricular septal defect are all associated with hemizygosity of 22q11. While the prevalence of the deletions in these phenotypes has been studied, the frequency of deletions in patients presenting with velopharyngeal insufficiency (VPI) is unknown. We performed fluorescence in situ hybridization for locus D22S75 within the 22q11 region on 23 patients with VPI (age range 5-42 years) followed in the Craniofacial Clinic at the University of Florida. The VPI occurred either as a condition of unknown cause (n=16) or as a condition remaining following primary cleft palate surgery (n=7). Six of sixteen patients with VPI of unknown cause and one of seven with VPI following surgery had a deletion in the region. This study documents a high frequency of 22q11 deletions in those presenting with VPI unrelated to overt cleft palate surgery and suggests that deletion testing should be considered in patients with VPI.

These data suggest the need for a larger-scale study using stable-isotope methods to further investigate this hypothesis.

Fatigue is related to family impact among CSHCNs, acting through the impairment in HRQOL.