Apert syndrome is characterized by short, radially deviated thumbs, leading to difficulties in daily life such as holding a fork or a spoon and buttoning up. The main goal of surgery is to achieve thumb to index finger pinch to overcome these difficulties. Seven patients (14 extremities) followed up with Apert syndrome underwent distraction after a C-shaped osteotomy to simultaneously correct the brachydactyly and the angulation deformity of the bilateral thumbs. The patients ranged in age from 4 to 7 years at the distraction operation, with a mean (SD) of 4.7 (1.7) years, and the mean (SD) length of follow-up was 100.6 (14.95) months. The mean (SD) length of the phalanges at the beginning of distraction was 19.1 (3.26) mm, and the mean (SD) length of the distracted phalanx at long-term follow-up visit was 26.2 (5.63) mm. The mean (SD) correction of radial angulation was calculated as 42.6 (9.95) degrees, and the difference was considered as being statistically significant (P < 0.001). Minor complications such as pin loosening were observed in 6 extremities of 4 patients, and 2 patients were treated for pin tract infection. Index-to-thumb pinching was acquired by all the patients after distraction and correction of angulation. The hand features of Apert syndrome are as noteworthy as the craniofacial features and thus may lead to functional impairment and limitations in daily life. Distraction with a C-shaped osteotomy seems to be a promising method to correct both the shortness and the radial angulation of the thumb to achieve functional results.
Development of normal speech is the primary goal of successful palatoplasty. The purpose of this study was to determine the importance of the contribution of vomer flap to palatoplasty procedure for speech function. Eighty-one children who underwent 2 flap palatoplasty procedures for cleft palate repair between 2002 and 2010 were retrospectively reviewed in 3 groups. Group 1 underwent palatoplasty without contribution of vomer flap. Group 2 underwent palatoplasty with standard dissection of vomer flap, whereas group 3 underwent palatoplasty with extended dissection of vomer flap. Speech function of the patients was evaluated using objective assessment tools such as nasopharyngoscopy and nasometer. Eighty-one children who underwent 2 flap palatoplasty were included in this study. The mean age at palatoplasty was 10.17 months, and mean length of follow-up was 72.33 months. For most syllables, patients repaired using extended vomer flap demonstrated lower nasalance scores. Nasopharyngoscopic examination revealed velopharyngeal motility in 24 patients (80%) in group 1 and in 20 (83.3%) and 23 (85.2%) patients in groups 2 and 3, respectively (P = 0.930). In velopharyngeal closure, there were only 5 patients (18.5%) in group 3, whereas there were 6 patients (25.0%) for group 2 and 10 patients (33.3%) for group 1 with no closure (P = 0.311). Although most optimum results were observed in the group with extended dissection of the vomer flap, contribution of the extended vomer flap to the repair of the soft palate did not lead to significantly better speech results.
Velopharyngeal insufficiency (VPI) is certainly one of the most important problems confronted after cleft palate repairs. In this study, it was aimed to evaluate the preoperative and postoperative speaking results of patients who underwent modified superior-based pharyngeal flap. Sixty-six children who underwent modified superiorly based pharyngeal flap for treatment of VPI between 2005 and 2013 were retrospectively reviewed. The study population was evaluated in 2 distinctive groups depending on their preoperative velopharyngeal closure pattern as: coronal closure pattern or noncoronal closure patterns (ie, circular, sagittal with or without the presence of a Passavant ridge). The speech outcome of the patients was evaluated using the objective assessment tools of nasopharyngoscopy and nasometer. Coronal closure pattern was determined in 24 patients (36.4%) and noncoronal closure pattern in 42 patients (63.6%). The mean follow-up period was 14.67 ± 3.90 and 13.74 ± 3.53 months in the coronal and noncoronal groups, respectively. The results demonstrated that the postoperative nasalance scores of all syllables except (m) and (n) were found to be significantly lower compared to those of the preoperative period (P < 0.001). However, no significant difference between the preoperative and postoperative results was observed in means of closure pattern. The surgical approach of modified superior-based pharyngeal flap for treatment of VPI appears to be effective regardless of the preoperatively determined closure pattern.
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