Outcome of surgical-orthodontic treatment in hemifacial microsomia with and without early mandibular distraction osteogenesis.

The aim of this study was to compare the outcomes of surgical-orthodontic treatment between hemifacial microsomia (HFM) patients who had and had not undergone early mandibular distraction osteogenesis (DO). Twenty adult unilateral HFM patients were included, seven who had undergone early mandibular DO (DO group) and 13 who had not (NDO group). All patients were type IIB, except for one type IIA patient in the NDO group. Mean age at definitive surgery was 20.72±2.96 years. Linear, cross-sectional, and volumetric measurements were obtained from serial cone beam computed tomography scans. Data were obtained pre-surgery (T0), 1 week after surgery (T1), and at treatment completion (T2) to determine surgical movement, post-surgical stability, and net gain movement. Surgical and ultimate outcomes did not differ significantly between the groups. The overall surgical movement among all patients was as follows (mean values): maxillomandibular complex (MMC) symmetry was achieved by Le Fort I differential roll movement (3.78mm extrusion on the affected side, 4.28mm impaction on the non-affected side), a combination of medial movement and yaw rotation of MMC, and genioplasty. Upper and lower dental midlines and deviated menton were shifted by 5.73mm, 5.08mm, and 12.38mm, respectively. Anterior impaction and advancement with counterclockwise rotation of MMC were also performed. Menton was advanced by 6.14mm and lower facial height was increased by 3.55mm. Neither group exhibited a significant difference in stability. Relapse at the maxilla was <1mm and relapse at the mandible was <1.5mm. The results suggest that early DO had limited beneficial effects on the definitive correction outcome. HFM patients achieved acceptable symmetry and a stable surgical outcome, regardless of early DO, following surgical-orthodontic correction at skeletal maturity with three-dimensional surgical simulation.

The genetic diversity and population structures of indigenous pig breeds in Zhejiang Province revealed by GGRS sequencing.

Chinese indigenous pigs in Zhejiang Province are well known for their high fecundity. In order to verify breed subdivision at the genomic level, we investigated genetic diversity and population structure of seven breeds and made comparisons with three Western pig breeds using next-generation sequencing data. Parameters obtained from allelic richness and proportion of polymorphic markers indicated that the genetic diversity of the Chinese indigenous pigs was higher than that of the Western pigs, with the highest and lowest values found in the Chaluand and the Landrace pigs respectively. Both neighbor-joining tree and principal components analysis could distinguish breeds from one another and structure analysis showed less differentiation among Western pigs than among the Chinese pigs. The average linkage disequilibrium decay over distance was significantly less in the Chinese pigs compared with the Western pigs, ranging from 188.2 to 280.6 kb for the Chinese pigs and 680.3 to 752.8 kb for the Western breeds and showing an average r2 threshold value of 0.3. Results obtained from high-density SNP comparison over the whole genome on genetic diversity and population structure were in agreement with the current breed classification of the pigs in Zhejiang Province. More importantly, the results presented here advances our current understanding of the genomic biology of Chinese indigenous pigs in Zhejiang Province and allows for implementation of conservation strategies in additional breeds.

Comparison of the adult three-dimensional craniofacial features of patients with unilateral craniofacial microsomia with and without early mandible distraction.

This study was conducted to analyze the long-term facial growth of patients with craniofacial microsomia (CFM) after early mandible distraction osteogenesis (DO), and compared adult three-dimensional (3D) craniofacial features of patients with and without early mandibular DO for Pruzansky grade II deformities. The study included 20 patients: 9 with early mandible DO (the DO group) and 11 without previous treatment (the NDO group). Longitudinal radiographs were measured for growth changes after DO. The 3D craniofacial images were constructed to compare the craniofacial forms between the two groups. The patients with early DO presented 8 to 9mm forward and downward maxillary growth and 4.6mm limited forward and 17.3mm substantial downward mandibular growth. The ramus length ratio (affected/nonaffected) was 90.8% at DO completion and decreased to 69.5% at growth completion during 13 years of follow-up. Both groups showed obvious craniofacial asymmetry, as indicated by occlusal plane canting, chin deviation, transverse and vertical condyle positions, and mandibular contours. Although all the bilateral differences were higher in the NDO group than in the DO group, no statistical differences were found. Early mandible distraction could not alter the inherent facial growth pattern in patients with grade II CFM. Limited changes are derived for definitive facial correction with early DO.

Immunologic assessment and KMT2D mutation detection in Kabuki syndrome.

Kabuki or Niikawa-Kuroki syndrome (KS) is a rare disorder with multiple malformations and recurrent infections, especially otitis media. This study aimed to investigate the genetic defects in Kabuki syndrome and determine if immune status is related to recurrent otitis media. Fourteen patients from 12 unrelated families were enrolled in the 9-year study period (2005-2013). All had Kabuki faces, cleft palate, developmental delay, mental retardation, and the short fifth finger. Recurrent otitis media (12/14) and hearing impairment (8/14) were also more common features. Immunologic analysis revealed lower memory CD19+ cells (11/13), lower memory CD4+ cells (8/13), undetectable anti-HBs antibodies (7/13), and antibody deficiency (7/13), including lower IgA (4), IgG (2), and IgG2 (1). Naïve emigrant lymphocytes, lymphocyte proliferation function, complement activity, and superoxide production in polymorphonuclear cells were all normal. All the patients had KMT2D mutations and 10 novel mutations of R1252X, R1757X,Y1998C, P2550R fs2604X, Q4013X, G5379X, E5425K, R5432X, R5432W, and R5500W. Resembling the phenotype of common variable immunodeficiency, KS patients with antibody deficiency, decreased memory cells, and poor vaccine response increased susceptibility to recurrent otitis media. Large-scale prospective studies are warranted to determine if regular immunoglobulin supplementation decreases the frequency of otitis media and severity of hearing impairment.

Craniofacial and dental dysmorphology in patients with median facial dysplasia: long-term follow-up.

Median facial dysplasia affects a subset of patients with cleft lip and palate exhibiting certain characteristics of median facial structure deficiencies without definable gross abnormalities of the brain. The aim of this study was to describe the craniofacial and dental morphology of almost skeletally mature patients with median facial dysplasia. Patients were selected for this retrospective study if they were diagnosed with median facial dysplasia and ≥15 years old. The craniofacial and dental morphology was evaluated by analysing cephalometric and panoramic radiographs. This sample of median facial dysplasia patients (9 males and 11 females; 6 unilateral and 14 bilateral clefts) had a mean age of 16.7 ± 1.9 years. Controls were age-, sex-, cleft type-matched, and nonsyndromic patients. The results showed that in patients with median facial dysplasia, the anterior cranial base and midface were shorter than in controls. The median facial dysplasia inter-orbital distance was shorter and the nasal bone was more retrusive than in controls. All patients with median facial dysplasia had several missing permanent teeth. These features require extensive surgical, orthodontic, and dental rehabilitation procedures.

The craniofacial anomalies archive at St. Louis Children's Hospital: 20 years of craniofacial imaging experience.

This article describes how the Craniofacial Imaging Laboratory at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital, Washington University Medical Center, has developed an electronic archive for the storage of computed tomography image digital data that is independent of scanner hardware and independent of units of storage media (i.e., floppy disks and optical disks). The archive represents one of the largest repositories of high-quality computed tomography data of children with craniofacial deformities in the world. Archiving reconstructed image data is essential for comparative imaging, surgical simulation, quantitative analysis, and use with solid model fabrication (e.g., stereolithography). One tertiary craniofacial center's experience in the establishment and maintenance of such an archive through three generations of storage technology is reported. The current archive is housed on an external 35-GB hard drive attached to a Windows-based desktop server. Data in the archive were categorized by specific demographics into groups of patients, number of scans, and diagnoses. The Craniofacial Imaging Laboratory archive currently contains computed tomography image digital data for 1827 individual scans. The earliest scan was done in 1980; the most recently stored scan for the purposes of this report occurred in May of 2000. The average number of scans archived per complete year was 94, with a range of 59 to 138. Of the 1827 total scans, 74 percent could be classified into specific diagnostic categories. The majority of the archive (55 percent) is composed of the following five diagnoses: sagittal synostosis (17 percent), unilateral coronal synostosis (11 percent), hemifacial microsomia (10 percent), plagiocephaly without synostosis (10 percent), and metopic synostosis (7 percent). Storage of computed tomography image data in a digital archive currently allows for continuous upgrading of image display and analysis and facilitates longitudinal and cross-sectional studies, both intramural and extramural. Internet access for clinical and research purposes is feasible, but contingent on protection of patient confidentiality. The future of digital imaging regarding craniofacial computed tomography scan storage and processing is also discussed.

Craniofacial surgical simulation: application of three-dimensional medical imaging and rapid prototyping models.

BACKGROUND: Adequate assessment of the deformity, formulation of good treatment planning, and sufficient rehearsal of procedures before actually performing surgery ensure successful craniofacial surgery. Three-dimensional computed tomography (CT) imaging and facsimile models were used in combination to evaluate their function in craniofacial surgery. METHODS: Three-dimensional imaging and facsimile models were used for reconstruction of craniofacial deformity. CT data were acquired, processed, and reconstructed to display 3-dimensional images. The images were used for evaluation of the deformity. The images were then manipulated to create multiple osseous objects. Surgical simulation was performed by moving the computer images. The image processing and manipulation were achieved using the AnalyzePC program. The raw CT data were transformed into a readable format and transferred to produce facsimile models using rapid prototyping technology. The skull models were used for evaluation and surgical simulation. Both methods were compared and used to assist in surgery, which was performed according to the simulations. RESULTS: Three-dimensional CT imaging and facsimile models were helpful for simulation of craniofacial surgery. The actual surgery results were satisfactory without complications. Particular advantages were the unlimited trials with the imaging method, and the feeling of reality with the model method. CONCLUSIONS: Craniofacial surgery is facilitated by preoperative simulation of procedures. Both 3-dimensional CT imaging and facsimile models are helpful for craniofacial surgical simulation.

The effect of hamulus fracture on the outcome of palatoplasty: a preliminary report of a prospective, alternating study.

OBJECTIVE: To determine whether, in performing palatoplasty, fracture of the pterygoid hamulus is beneficial, detrimental, or neutral with respect to intraoperative and perioperative complications, hearing outcome, and speech outcome. DESIGN: Prospective, alternating. SETTING: Institutional, tertiary cleft palate center, Chang Gung Memorial Hospital, Taipei, Taiwan. PARTICIPANTS: A total of 173 patients enrolled in the study, of whom 161 had charts available for analysis. INTERVENTIONS: During the performance of palatoplasty, 85 patients received hamulus fracture and 76 patients did not. All palatoplasties were performed by the same surgeon. MAIN OUTCOME MEASURES: (1) Surgical outcomes, including patient demographic data, palatoplasty type and duration, blood loss, incidences of oronasal fistulae, temporary mucosal dehiscence, and postoperative bleeding; (2) otolaryngological outcomes, including hearing results as judged by auditory brainstem response testing, myringotomy tube data describing rates of tube extrusion, and culture results from sampled effusions; and (3) preliminary speech outcomes as described by judgments of overall velopharyngeal function from perceptual speech samples. RESULTS: No statistically significant differences in any of the measured surgical, otolaryngological, or preliminary speech outcomes were found between the groups who did and did not receive hamulus fracture. CONCLUSIONS: On the basis of these results, we are unable to advocate the performance of hamulus fracture as an operative maneuver during the performance of primary palatoplasty. The historical rationale and theoretical advantage of this maneuver have not been demonstrated here nor have any detrimental effects of the maneuver been measured.

Major hematological diseases associated with cleft lip and palate.

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 hematological disorders and cleft lip, palate, or both is rare and is reported herein.

The course of the inferior alveolar nerve in the normal human mandibular ramus and in patients presenting for cosmetic reduction of the mandibular angles.

This study was undertaken to quantify the path of the inferior alveolar nerve in the normal human mandible and in the mandibles of patients presenting for cosmetic reduction of the mandibular angles. The goals were: (1) to provide normative information that would assist the surgeon in avoiding injury to the nerve during surgery; (2) to characterize gender differences in the normal population; and (3) to compare the course of the nerve in the normal population to its course in a group of patients who presented with a complaint of "square face." The study was based upon the computerized tomographic scans of 10 normal patients (six men, four women) and 8 patients (all women) complaining of "square face." Using AnalyzePC 2.5 imaging software, the mandibles were segmented and the position of the nerve was recorded within its osseous canal in the mandibular ramus on each axial slice in which it was identifiable. Distances were calculated between the nerve and the anterior, posterior, lateral, and medial cortices. The positions of the lateral ramus prominence and the lowest point on the sigmoid notch were also recorded. The position of the mental foramen was recorded in relation to the nearest tooth, and the three-dimensional surface distances from the foramen to the alveolar bone, the inferior border of the mandible, and the mandibular symphysis were determined. The distances from the entrance of the nerve into the mandible to the lateral ramus prominence and the lowest point on the sigmoid notch were calculated. Summary statistics were obtained, comparing differences in gender. The nerve was identifiable in each ramus over a mean distance of 12.7 mm. On average, the lateral ramus prominence was 0.3 mm higher on the caudad-cephalad axis than the point at which the nerve entered the bone, whereas the location of the lowest point on the sigmoid notch was 16.6 mm above the nerve. The average distances from the nerve to the anterior, posterior, medial, and lateral cortices were 11.6, 12.1, 1.8, and 4.7 mm, respectively. Gender differences were significant for all of these except the medial cortex to nerve distance. On average, the mental foramen exited the body of the mandible immediately below the second premolar and the average surface distances from the foramen to the symphysis, the most cephalad alveolar bone, and the inferior border of the body were 30.9, 14.2, and 19.3 mm, respectively. With regard to the patients presenting for mandibular angle reduction, there were a few statistically significant but small scalar differences from normal controls.

Quantitative measurement on three-dimensional computed tomography: an experimental validation using phantom objects.

BACKGROUND: The use of 3-dimensional computed tomography (CT) imaging has been applied to the craniofacial region as well as to many other parts of the human body. Quantitative measurements have frequently been performed on the 3-dimensional images. However, critical validation of the measurement has been insufficient in the literature. This study was designed to evaluate the errors of the 3-dimensional measurements. METHODS: Four phantom objects, a cube, a sphere, a cylinder, and a life-size adult skull model, were scanned using standard CT acquisition protocol. The data were transferred, reformatted, and displayed on an IBM-compatible personal computer running AnalyzePC 2.5 software. Linear, area, and volume measurements were obtained using one of the two methods. The first was physical measurement of the phantom objects using a caliper for linear measurement and mathematical calculations for area and volume measurements. The second was done by computer measurement on 3-dimensional images using the AnalyzePC 2.5 program. Each measurement was performed twice. The differences were compared between the repeated measurements and between the two methods. RESULTS: The images were displayed according to standard 3-dimensional CT protocol. The differences between the measurements were insignificant and ranged from 0.00 to 2.57%. CONCLUSION: This study validated the accuracy of the quantitative measurements on 3-dimensional CT images.

Temporal expression and T3 induction of thyroid hormone receptors alpha1 and beta1 during early embryonic and larval development in zebrafish, Danio rerio.

The effects of thyroid hormones on metabolism and development are mediated by thyroid hormone receptors (TRs). We report the cloning and characterization of a TR beta1 cDNA from zebrafish. Southern blot analysis revealed that there is a single genomic locus for the TR beta gene, while the TR alpha gene potentially has two loci. Multiple TR alpha and TR beta transcripts were detected in adult tissues. Using a semiquantitative RT-PCR assay, zygotic expression of TR alpha1 and TR beta1 were shown to occur before the midblastula transition stage. In transiently transfected HeLa cells, TR alpha1 displayed constitutive transactivation in the absence of ligands, which was slightly enhanced by triiodothyronine (T3). The transactivating activity of TR beta1 was strictly ligand-dependent and repressed in the absence of T3. Finally, the T3 induction of TR alpha1 and TR beta1 mRNAs was demonstrated in zebrafish embryos and larvae. The auto-induction of TR alpha1 and TR beta1 may serve a regulatory role during the embryonic and larval development of zebrafish.

Social support and life adjustment for teenagers with cleft lip and palate in Taiwan.

BACKGROUND: The teenage years (13-19 years) are a critical period for psychosocial development. Therefore, social support and life adjustment for teenagers with cleft lip/palate were chosen for investigation. METHODS: The sample survey included 101 cleft patients randomly selected from the Chang Gung Craniofacial Center, and 101 non-cleft controls selected from local middle and high schools for comparison. Questionnaires were used to evaluate the social support and life adjustment scales. Items of social support were subdivided into social activities, social service, and psychological support. Items of life adjustment were subdivided into social, psychological, and physical adjustments. RESULTS: The coefficients of alpha reliability were high at 0.9294 for social support, and 0.9389 for life adjustment. The results show that personal factors, family factors, and treatment status do not influence social support or life adjustment in either group; however, gender does. In the control group, non-cleft males had better social support and life adjustment than did females. Such a difference was not observed in the cleft group. Both groups received the same social support. The cleft teenagers have a significantly lower level of life adjustment. The association between the social support and life adjustment is high in both groups. CONCLUSION: The cleft group has a lower level of life adjustment. There is a positive relationship between social support and life adjustment. More social support is required for the cleft group in order to improve their level of life adjustment.

Maxillary growth after palatal denudation: an animal experiment.

BACKGROUND: Palate surgery at an early age may cause retardation of maxillary growth. The second intention healing of the raw bone surface created on the palate is considered to be the cause of the growth retardation. The animal experiment in this study was designed to evaluate this effect. METHODS: Four-week-old Sprague-Dawley rats were divided into 3 groups. In the first group, a strip of mucoperiosteum was excised on both sides of the hard palate. A second group of rats received a sham surgery in which bilateral mucoperiosteal flaps were raised and redraped. The third group served as controls with no surgery. Flap elevation with and without excision was performed under an operative microscope to facilitate the delicate manipulation of tissue and to avoid injury to the underlying bone. The animals were killed 11 weeks later and the skulls were prepared for measurements, which included the palatal inter-molar width, maxillary height, and maxillary length. RESULTS: The results revealed statistically significant decreases in palatal width and maxillary length in the experimental group (excision of mucoperiosteum). No differences were observed in the vertical height of the maxilla. CONCLUSION: This study confirms that surgically created bone denudation of the palate causes maxillary growth disturbances.

Airway obstruction in severe syndromic craniosynostosis.

Airway obstruction is frequent in patients with severe syndromic craniosynostosis, and must be treated for successful, complete care. The purpose of this study was to evaluate the incidence and the management of airway obstruction in patients with severe craniosynostosis. All patients with complex syndromic craniosynostosis at Chang Gung Craniofacial Center were evaluated retrospectively. Criteria for inclusion of patients were the presence of adequate follow-up and documentation. A total of 40 patients were included, of whom 13 had Apert's syndrome and 27 had Crouzon's disease. Clinical symptoms and signs related to airway obstruction and its treatment were evaluated carefully, and were verified further by telephone inquiry. The status of the airway was categorized into one of three groups: no obstruction; mild obstruction, for which positioning and medical treatment were needed; and severe obstruction, for which surgical intervention was needed. The results showed that 24 patients (60%) did not have airway obstruction, 11 patients (27.5%) had mild obstruction, and 5 patients (12.5%) had severe obstruction. There was no significant difference in the distribution of airway status between patients with Apert's syndrome and Crouzon's disease. Causes for the 5 patients with severe obstruction were midface hypoplasia, lower airway obstruction, tonsillar and adenoid hypertrophy, and choanal atresia. Tracheostomy was performed to control airway in 3 patients, and 1 patient died. Midface anterior distraction helped to decannulate 1 patient. In conclusion, airway obstruction was observed in 40% of patients with severe craniosynostotic syndromes. Most of them could be managed successfully with conservative or medical treatment, and surgical intervention should be considered on the basis of each patient's individual condition.

Cleft of the lip and palate in twins.

BACKGROUND: Cleft lip and palate is one of the most common congenital anomalies in Taiwan. Its etiology remains unknown for the majority of the patients. The study of twins is a classic method for evaluating the relative roles of genetic and environmental factors in the formation of the anomaly. METHODS: In this study, 37 pairs of twins and one set of triplets with cleft lip and palate were evaluated. Clinical data were collected for zygosity determination and analysis of etiologic factors. The concordance rate and heritability index were assessed. RESULTS: The results showed that the concordance rate was 26% for all twins and 57% among the monozygotic pairs, which is higher than those rates for the Caucasian population. The heritability index was 53%, higher than the other reports as well. The influence of the environment could not be ruled out. CONCLUSION: The results confirm a strong genetic role in the etiology of clefts in our patients. Environmental factors were acting as well. The findings in this study support the multifactorial threshold model in the development of cleft lip and palate.

Bilateral coronoid process hyperplasia with limitation on mouth opening: case report.

Coronoid process hyperplasia with limitation of mouth opening is rare. The pathology is often ignored, but it can be easily detected using dental panoramic view of x-ray films. Definition of the coronoid process hyperplasia can be made by measuring the height of coronoid process and the ratio of coronoid/condyle height on lateral cephalometric x-ray film. Etiology of the coronoid process hyperplasia can be congenital or acquired. Differentiation of the diagnosis may be difficult. The congenital type occurs at early age with clinical manifestations. Proposed hypotheses for the formation of coronoid process hyperplasia include increased activity within the temporalis muscle from conditions such as functional stress, compression, and tension. For patients with coronoid process hyperplasia and restriction on mouth opening, conservative treatment should first be attempted. Surgical treatment is considered if conservative treatment fails. Coronoidectomy with early mobilization and aggressive physiotherapy corrects the problem. We present a patient with coronoid process hyperplasia with limitation of mouth opening who was successfully treated.

Three-dimensional computed tomographic analysis of the primary nasal deformity in 3-month-old infants with complete unilateral cleft lip and palate.

The purpose of this study was to analyze the geometry of the primary cleft lip nasal deformity using three-dimensional computerized tomography in a group of 3-month-old infants with complete unilateral cleft lip and palate before surgical intervention. Coordinates and axes were reconfigured after the three-dimensional image was oriented into neutral position (Frankfurt horizontal, true anteroposterior, and vertical midline). Display and measurement of skin surface and osseous tissues were achieved by adjusting the computed tomographic thresholds. S-N, N-ANS, S-N-O, and S-N-ANS were measured from true lateral views. Biorbital (LO-LO), interorbital (MO-MO), intercanthal (en-en), and nasal (al-al) widths were measured from the anteroposterior view. The bony alveolar cleft width was measured from the inferior view. The study group was divided into two groups on the basis of skeletal alveolar cleft width: six patients with clefts narrower than 10 mm and six patients with clefts wider than 10 mm. Only the S-N-ANS angle differed between the two groups, i.e., it was greater in the group with the wider clefts (p < 0.05). Coordinates of six landmarks at the base of the nose [sellion (se), subnasale (sn), cleft-side and noncleft-side subalare (sbal-cl and sbal-ncl), and the most posterior point on the lateral piriform margins (PPA-CL and PPA-NCL)] were obtained for analysis of the nasal deformity. On average, the subnasale point was anterior to sellion and deviated to the noncleft side; the cleft-side sbal point was more medial, posterior, and inferior than the noncleft-side sbal point; and the PPA point on the cleft-side piriform margin was more lateral, posterior, and inferior than the PPA point on the noncleft side. These discrepancies were not universally observed. However, in all patients, four findings were observed without exception (p < 0.01): (1) subnasale (sn) was deviated to the noncleft side (mean distance from midline, 5.0 mm; range, 2 to 9.5 mm), (2) the cleft-side alar base (sbal-cl) was more posterior than the noncleft-side alar base (sbal-ncl) (mean difference, 3.6 mm; range, 1 to 5.5 mm), (3) the noncleft-side alar base (sbal-ncl) was further from the midline than the cleft-side alar base (sbal-cl) (mean difference in lateral distances of sbal-ncl and sbal-cl from the midline, 2.8 mm; range, 0.5 to 7 mm), and (4) the cleft-side piriform margin (PPA-CL) was more posterior than the noncleft side piriform margin (PPA-NCL) (mean difference, 2.1 mm; range, 0.5 to 4 mm). In conclusion, the nasal deformity in unilateral cleft lip and palate that has not been operated on is characterized by these four features and increased S-N-ANS angle with increased alveolar cleft width.

Palatoalveolar outcome at 18 months following simultaneous primary cleft lip repair and posterior palatoplasty.

It is frequently reported that early repair of the soft palate induces narrowing of the remaining palatal cleft and thus facilitates later hard palate closure. However, to the best of our knowledge, there have been no comparative studies to test this hypothesis. The purpose of this retrospective study was to evaluate the change of palatoalveolar morphology following primary lip repair and posterior palatoplasty. Dental plaster models of patients with complete unilateral cleft of lip and palate (UCLP) were used to measure the width of the cleft and palatal arch. Twenty-six patients received simple posterior palatoplasty (PP group) simultaneous with primary lip repair, and 20 patients did not (NPP group). The dental models included one preoperative cast at 2 months (T1) and two or three casts at 6 (T2), 12 (T3), and 18 (T4) months before final palate closure. The linear measurements performed were width of alveolar cleft (Ca); width of palatal cleft between the canines (Cc), molars (Cm), and tuberosities (Ct); the palatal arch distance between the canines (Dc); the widest distance between molars (Dm) and the tuberosities (Dt); and the palatal height between the canines (Hc) and tuberosities (Ht). The raw measurements and the calculated cleft-to-arch ratios of Cc/Dc, Cm/Dm, and Ct/Dt were compared between the two groups. The results showed gradual narrowing of the width of cleft from T1 to T4. Narrowing of alveolar cleft width (Ca) from T1 to T2 was dramatic. The palatal arch (Dc, Dm, Dt) showed no change to mild increase in width. The cleft-to-arch ratios decreased with time. The palatal height remained the same or slightly increased over time. There were no significant differences observed between the PP and NPP groups among these measurements except for the Ct and Ct/Dt at T4. In conclusion, after initial lip repair, there was a decrease of the width of cleft in patients with complete UCLP during the 18-month period, and simple posterior palatoplasty did not further narrow the cleft nor influence palatal arch development.