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Background: Augmentation rhinoplasty with autologous fat grafting is a useful procedure to meet the demand for facial harmonization in the Asian population. We used this procedure during orthognathic surgery to address inadequate dorsum projection. This prospective study was conducted to determine the fat retention rate in patients undergoing simultaneous autologous fat injection augmentation rhinoplasty and orthognathic surgery. Methods: Nineteen patients were treated with simultaneous bimaxillary orthognathic surgery and autologous fat grafting of the nasal dorsum and tip. The paired t test was used to compare the nasal volumes before and at least 6 months after surgery measured by 3-dimensional computer tomography scans. All measurements were performed twice by the same evaluator at least 2 weeks apart for intrarater consistency. Results: Seventeen patients completed the study. The volume means before and after surgery were 22.3 ± 4.6 cm3 and 23.3 ± 4.7 cm3, respectively, with a mean difference of 1.0 ± 0.3 cm3 ( P < .001). The mean retention rate was calculated to be 50.5% ± 7.0% (range: 40.5%-64.7%). Intrarater consistency was high with a Cronbach α of .97 ( P < .001) and .98 ( P < .001), respectively. Conclusion: This prospective study provides objective graft retention measurements for fat injection augmentation rhinoplasty combined with orthognathic surgery. All patients were satisfied with the results and no complications or additional morbidity was noted in the postoperative course. We consider this procedure to be a safe, reliable, and powerful adjunct to improve the aesthetic results of orthognathic surgery.

Background: There is an increasing patient expectation for better esthetics, manifesting through anatomical harmony, projection, and volume of the upper lip relative to the lower lip, in patients with cleft deformity. The aim of this study is to investigate the outcome of vermilion augmentation using autologous dermofat graft (DFG) to enhance the lip using both quantitative and qualitative assessment. Methods: Patients with secondary cleft lip deformity who received the surgical treatment in our institution from 2015 to 2018 were recruited. Panel assessment was performed on standardized preoperative and postoperative digital photographs. A patient questionnaire was used for the reported outcome. Image processing and analyses were applied to measure the lip change. Statistical analyses were performed. Results: A total of 91 patients were included. The mean age at operation was 22.7 years, and postoperative follow-up was 3.6 years. There were no complications in the study group. The panel assessment showed significant improvement ( P < .00001) on upper lip vermilion in both frontal and lateral profile views. Ninety-five percent of patients reported improvement of the upper lip projection and volume. Quantitative image analysis showed an increase in the upper lip vermilion ratio in 97% of the cases in frontal views and improvement of the vermilion projection in 87% of the cases in the superimposed lateral views. Conclusions: The use of DFG is an effective and reproducible method for vermilion augmentation and aesthetic enhancement in patients presenting with upper lip insufficiency relative to the lower lip.

Background: Primary reconstruction of complete bilateral cleft lip and palate (BCLP) with protruding premaxilla in one-stage surgery is challenging because of the tension on muscle repair. Such patients are also common in the developing countries. For this condition, we have applied intraoperative “rapid premaxillary molding (RPM)” technique and obtained satisfactory results. Methods: We reviewed the data of patients with complete BCLP with protruding premaxilla applying intraoperative RPM including both our institution and cleft missions to developing countries in the past 20 years. Selection criteria were patients receiving either no or insufficient preoperative molding presenting with significant protruding premaxilla, had consecutive follow-ups after surgery. To perform this technique, the surgeon holds the patient’s face with both hands and compresses the protruding premaxilla with both thumbs. An intermittent but stable backward pressure is applied to the premaxilla until the segment is gradually flexible. Repeated compression is performed prior to tying the muscle sutures, at which time the premaxilla is retro-positioned and aligned with the lateral maxillary segments. Pre- and postoperative data were evaluated. Results: We have treated a total of 60 patients with complete BCLP with protruding premaxilla applying intraoperative RPM. All patients tolerated the operations and there were no major intra- and postoperative complications including lip dehiscence and vomer fracture. All of them had satisfactory results such as adequate muscle repair and symmetry of the lip and nostrils. Conclusion: The RPM is a reliable and valuable intraoperative adjunct procedure for patients with complete BCLP presenting the protrusive premaxilla.

Since the original description of Millard rotation advancement principle for cleft lip repair, many important contributions have subsequently been described by other surgeons worldwide. However, the reconstruction of the nasal floor and intraoral lining has received less attention over time. This article demonstrates a modified unilateral complete cleft lip repair using the rotation advancement principle plus multipurpose inferior turbinate mucosal flap. The accompanying videos display the 10 key steps for rationale, design, and proper execution of the inferior turbinate mucosal flap for the nasal floor and intraoral reconstruction.

Objective: Nonsyndromic oral clefts are common birth defect with complex etiology. In the present study, we attempt to further validate the possible role for ABCA4 and ARHGAP29 in the susceptibility to nonsyndromic oral clefts. Design: We performed allelic transmission disequilibrium test analysis, on 10 eligible single nucleotide polymorphisms (SNPs) and SNP haplotypes using the Family-Based Association Test. Participants: The study sample consisted of 334 case–parent trios of nonsyndromic oral clefts from Taiwanese population, separated into nonsyndromic cleft lip with or without cleft palate (NSCL/P) and nonsyndromic cleft palate only (NSCPO) groups. Results: We found only the SNP rs560426 within the ABCA4 gene showed strong association with NSCPO ( P = .03498; Permuted P = .05382). No association between other 9 selected SNPs in ABCA4-ARHGAP29 region and the risk of nonsyndromic oral clefts was found. For the haplotype analyses, we found only haplotype T-C (rs570926 and rs3789431) in ABCA4 block 2 showed significant association with nonsyndromic NSCL/P in these Taiwanese trios. Conclusions: We used a family-based analysis in 334 Taiwanese case–parent trios to validate the possible role for ABCA4 and ARHGAP29 in the susceptibility to nonsyndromic oral clefts. This study provides a new evidence for an association between the intron variant rs560426 within ABCA4 and nonsyndromic cleft palate which may contribute their regulatory role in craniofacial development.

Objective An inspiring early result with no oronasal fistula formation was recently described for a modified medial incision small double-opposing Z-plasty (MIsDOZ) for treating Veau type I cleft palate. This study describes an early single-surgeon experience in applying this newly proposed surgical approach. Design Retrospective single-surgeon study. Patients Consecutive nonsyndromic patients (n = 27) with Veau I cleft palate. Interventions Topographic anatomical-guided MIsDOZ palatoplasty with pyramidal space dissection (releasing of the ligamentous fibers in the greater palatine neurovascular bundle and pyramidal process region, in-fracture of the pterygoid hamulus, and widening of space of Ernst) performed by a novice surgeon (RD). Mean Outcome Measures Age at surgery, the presence of cleft lip, palatal cleft width, use of lateral relaxing incision, and 6-month complication rate (bleeding, dehiscence, fistula, and flap necrosis). A published senior surgeon-based outcome dataset (n = 24) was retrieved for comparison purposes. Results Twenty-two (81.5%) and 5 (18.5%) patients received the medial incision only technique and lateral incision technique, respectively ( P = .002). Age, presence of cleft lip, and cleft width were not associated (all P > .05) with the use of lateral incision. Comparative analysis between the novice surgeon- and senior surgeon-based datasets revealed no significant differences for sex (females: 74.1% vs 62.5%; P = .546), age (10.2 ± 1.7 vs 9.6 ± 1.2 months; P = .143), rate of lateral incision (18.5% vs 4.2%; P = .195), and postoperative complication rate (0% vs 0%). Conclusion This modified DOZ palatoplasty proved to be a reproducible procedure for Veau I cleft palate closure, with reduced need for lateral incision and with no early complication.

In rare instances, cleft lip and palate occur in association with synechia, intraoral fibrous bands connecting the maxilla and mandible. The main concern in synechia is a restricted mouth opening that leads to airway and feeding problems. This study reports our experience in the treatment of 3 cases and includes a review of the literature. Three patients with intraoral fibrous bands received treatment and follow-ups in our center. Division of the bands and reconstruction of the lip and palate were successfully performed. All reported cases in the literature were collected for a summary of clinical presentations and as references for management. Early management of the synechia improves the mouth opening and facilitates the treatment of associated anomalies.

Objective:To evaluate genetic variants within the regulatory regions of interferon regulatory factor 6 (IRF6) and TP63 for the etiology of nonsyndromic oral clefts risk factors.Design:We performed ...

Kabuki syndrome (KS) is a multiple congenital anomaly/mental retardation syndrome with characteristic facial features. Despite more than 350 documented cases and recent correlation of MLL2 mutations as a genetic cause, its full clinical spectrum is still being defined. This report describes two patients who were initially diagnosed with Van der Woude syndrome (VWS) based on the presence of lower lip pits. However, this finding can occur with KS, albeit infrequently. For patients with lower lip pits, a thorough evaluation should be made to distinguish between VWS and KS, as there are differences in long-term prognosis.

Objective To test the hypothesis that maxillary growth and lower lip form in patients with van der Woude syndrome (VDW) is different from patients with nonsyndromic cleft lip and palate. Design and Setting Retrospective, case-control study at a tertiary cleft center, Chang Gung Memorial Hospital, Taipei, Taiwan. Patients and Participants Records of 53 patients with VDW, who presented for treatment during the years 1968 through 1998 were obtained. Twenty-three of 53 patients had received at least one lateral cephalogram during the course of their treatment. Of these 23, in 17 it was possible to find nonsyndromic case controls with identical cleft type, sex, and method of cleft palate repair, with year of birth matching within 1 year of the corresponding VDW patient. For these 17 pairs of VDW and nonsyndromic cleft controls, cephalogram acquisition dates were checked to see how well the corresponding pairs matched. A total of 43 pairs of cephalograms were deemed to be acceptably matched because the ages at acquisition differed by less than 15% of the VDW patient's age. Main outcome Measures Thirteen measurements were derived from the 11 standard lateral cephalometric landmarks recorded on each cephalogram. The data were classified into five groups according to age at time of cephalogram, and sets of paired nonsyndromic cleft and VDW measurements were tested for differences using a Wilcoxon signed rank sum test in two ways, first including all cleft types and then including only those patients with complete bilateral cleft lip and palate. A longitudinal growth analysis considering the movement of the skeletal A and B points was performed on the patients with complete bilateral cleft lip and palate. Results For the osseous measurements, anteroposterior maxillary length as described by the anterior nasal spine (ANS)-posterior maxillary point distance was statistically significantly shorter in the VDW patients of age 13 years and older, by 5.3 mm. Maxillary height, as described by the nasion-ANS distance was shorter in the VDW patients, closely approaching statistical significance in the age range 7 through 11 years. The lip soft tissue measurements showed significantly greater protrusion over several age ranges in the VDW patients. The longitudinal growth analysis showed a significantly more inferior vertical position of the B point in the controls. Conclusions This study demonstrates a few statistically significant differences in maxillary growth and lip conformation between VDW and matching controls. Small sample sizes for each age group hamper the ability to fully interpret or generalize the pattern of these differences.

Objective Cleft lip and palate is among the most common congenital anomalies. Its association with major blood disorders has rarely been reported. The purpose of this study was to report two patients who had major blood diseases associated with cleft lip and palate. Patients and Results From June 1995 to December 1997, there were 2700 patients with cleft lip, cleft palate, or both who received treatment at Chang Gung Memorial Hospital. Two of them were found to have major hematological disorders. In both cases, the disorder was detected by preoperative blood cell counts and white cell differentiation. Case 1 was a 21-year-old woman patient with repaired right cleft lip. She was admitted for alveolar bone grafting and closure of oronasal fistula. Abnormal presentation of blast cells was found, and subsequent bone marrow study confirmed acute lymphocytic leukemia. Case 2 was a 26-year-old man with left secondary cleft lip nasal deformity scheduled to receive staged reconstructive operations. An elevated platelet count was found and subsequently confirmed to represent essential thrombocytosis. In both cases, reconstructive operations for the cleft-related deformities were performed. Conclusions Association of major hemotological disorders and cleft lip, palate, or both is rare and is reported herein.

Patients with unicoronal synostosis (UCS) or plagiocephaly without synostosis (PWS) have distinctive skull dysmorphologies. Associated mandibular dymorphologies have been suspected but not quantified. This study was performed to test the hypothesis that discrete mandibular dysmorphology exists in both UCS and PWS. All patients at a tertiary referral center at a medical school-affiliated children's hospital with confirmed diagnosis of UCS or PWS with adequate pretreatment CT data were included in the study population, which population was comprised of 20 UCS, 23 PWS, and 8 normal infants. Each patient had a head CT scan using 2-mm slices. 3-D images were created using AnalyzeTM, a biomedical imaging program. The mandibles were isolated, and the coordinates of 8 landmarks were sampled from each mandible by a single investigator: a single volume value was measured, and 9 linear distances and 4 angles were calculated. Corresponding measurements from each hemimandible were expressed as ratios of ipsilateral/contra lateral side. In UCS, the affected side was defined as the side ipsilateral to the synostosis; in PWS, the affected side was defined as the side ipsilateral to the occipital flattening. The results from both groups were t tested for statistical significance. For UCS, statistically significant (p < .001) findings included: ipsilateral hemimandibular volume 5% smaller than contralateral; affected hemimandibular body length 1.9% shorter; affected gonial angle 2.6% more acute; affected coronoid process tilted anteriorly 2.5%; and distances from condylion and tip of the coronoid process to the chin landmarks 4% shorter on the affected side. For PWS, significant findings included: affected hemimandibular volume 3.8% larger; ramal height 3.5% shorter; mandibular body length 3% longer; and coronoid process tilted anteriorly by 2.3% on the affected side. In the UCS/PWS comparison, findings included: affected hemimandibular volume in UCS 8.7% less; affected gonial angle in UCS 3% more acute; affected mandibular corpus length in UCS 5% shorter; distances from the condylion and the tip of the coronoid process to the chin landmarks 4% shorter on the affected side in UCS. The hypothesized presence of diagnosis specific mandibular dysmorphology in UCS and PWS is confirmed. This analysis forms the baseline for study of the effects of unperturbed growth or therapeutic interventions upon the dentoskeletal dysmorphology of these anomalies.

Unilateral coronal synostosis (UCS) produces overt craniofacial dysmorphology. UCS surgery in infancy aims to release the osseous restriction and normalize the fronto-orbital deformity. The quantitative effect of this surgery on the orbit and its contents is unknown. This study was conducted to quantify the preoperative orbital dysmorphology and its surgical outcome in patients with unilateral coronal synostosis. Twenty-eight UCS patients had preoperative three-dimensional computerized tomographic (CT) scans (at mean age 4.0 months), cranio-orbital reconstructive surgery (at 4.7 months), and postoperative scans (at 18.1 months). The CT data were analyzed using a computer workstation and AnalyzeTM biomedical imaging software. Four measurements were performed on both ipsilateral (same side as synostosis) and contralateral (opposite to synostosis) orbits of each scan: orbital index (OI, 100 × height/width of orbit), orbital cavity volume (OV), ocular globe volume (GV), and ventral globe index (VGI, 100 × globe volume ventral to the anterior surface of orbital cavity/GV). The data were analyzed for statistical significance using Student's t test. Preoperatively, the OI was significantly greater on the ipsilateral than on the contralateral side (113.7 vs. 87.3). There was a significant improvement on both sides of the orbit postoperatively, with ipsilateral 99.1 and contralateral 92.1. However, the difference between both sides remained significant. The OV was smaller in the ipsilateral orbits both pre- and postoperatively, with ipse/contralateral ratios of 95.8 and 95.2, respectively. Importantly, the GV was consistently smaller in the ipsilateral orbits preoperatively, with an ipse/contralateral ratio of 93.3. The ratio increased to 97.1 postoperatively, a statistically significant change. In the ipsilateral orbits, the preoperative VGI was significantly greater. The VGI improved postoperatively. These data indicate that UCS affects the development of the osseous orbit as well as its soft-tissue contents. After cranio-orbital surgery, there is diminution of asymmetry of both the bony orbit and its soft-tissue contents. Partial normalization of orbital dysmorphology occurred during the first postoperative year. UCS surgery in infancy does not prevent growth of orbital hard or soft tissues, and it seems to permit normalization of previously impaired growth.

A patient with bilateral complete cleft of the lip and palate (CLP) had a proximal deletion of the long arm of chromosome 1 (1q). This rare chromosomal abnormality was characterized by pre- and postnatal growth retardation, psychomotor retardation, and specific craniofacial and other systemic anomalies. There is a high incidence of CLP in proximal 1q deletion syndrome, especially bilateral CLP. Twelve other cases reported in the literature having this deletion and associated anomalies were reviewed.