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European and Northern American healthcare authorities increasingly encourage the use of Patient Reported Outcome Measures (PROMs) that complement clinical and laboratory assessments to help holistically evaluate reconstructive outcomes. This is the first study to evaluate PROMs in cleft lip/palate patients who have, or have not, undergone secondary alveolar bone grafting (SABG).A PROMs study was conducted; 40 consecutive consenting cleft lip/palate children between 8 and 14 years old were included. Twenty patients did, and 20 patients did not, have SABG. PROMs scores from children and parents in the 2 groups were compared.Forty patients completed the trial. No significant differences in total score from the Chang Gung Short Form-15 (CGSF-15) were found between children and their parents. Children with SABG reported no more oral-nasal regurgitation than children without SABG, but tended to report more nasal obstruction. There were no statistically significant differences in parent reported outcomes between the 2 groups.Cleft lip/palate patients who underwent SABG reported significantly less nasal regurgitation and more nasal obstruction compared to those patients who did not undergo SABG., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Presurgical orthodontic treatment before secondary alveolar bone grafting (SABG) is widely performed for cleft lip/palate patients. However, no randomized controlled trial has been published comparing SABG outcomes in patients with, and without, presurgical orthodontic treatment. This randomized, prospective, single-blinded trial was conducted between January 2012 and April 2015 to compare ABG volumes 6 months postoperatively between patients with and without presurgical orthodontic treatment. Twenty-four patients were enrolled and randomized and 22 patients completed follow-up. Patients who had presurgical orthodontics before SABG had significantly improved inclination (p < 0.001) and rotation (p < 0.001) of the central incisor adjacent to the defect, significantly improved ABG fill volume (0.81 ± 0.26 cm(3) at 6 months compared to 0.59 ± 0.22 cm(3); p < 0.05) and less residual alveolar bone defect (0.31 ± 0.08 cm(3) at 6 months compared to s 0.55 ± 0.14 cm(3); p < 0.001) compared to patients who did not have presurgical orthodontic treatment. In conclusion, orthodontic treatment combined with SABG results in superior bone volume when compared with conventional SABG alone.

Background: The purpose of this study was to evaluate progressive changes in surgical techniques and results, aiming for improved nasal shape in primary bilateral cleft rhinoplasty., Methods: This is an institutional review board-approved retrospective study. Ninety-one consecutive patients with bilateral complete cleft lip underwent primary cheiloplasty with four different techniques of nasal reconstruction from 1992 to 2007 as follows: group I, primary rhinoplasty alone; group II, nasoalveolar molding alone; group III, nasoalveolar molding plus primary rhinoplasty; group IV, nasoalveolar molding plus primary rhinoplasty with overcorrection; and group V, patients without cleft lip. The surgical results were analyzed using photographic records obtained at age 3 years. Four measurements and one angle measurement were obtained. A panel assessment was obtained to grade the appearance of the surgical results., Results: The results are expressed in order from groups I through V. The nostril height-to-width ratio was 0.49, 0.59, 0.62, 0.78, and 0.82, respectively. The nasal tip height-to-nasal width ratio was 0.29, 0.39, 0.49, 0.57, and 0.60. The columella height-to-nasal width ratio was 0.11, 0.18, 0.22, 0.27, and 0.28. The dome-to-columella ratio was 1.88, 1.25, 1.26, 1.14, and 1.10. The nostril area ratio was 1.2, 1.17, 1.13, 1.11, and 1.07. The nasolabial angle was 144.95, 143.98, 121.98, 120.99, and 100.88. Finally, group IV had the best panel assessment., Conclusions: The results revealed that group IV had the best overall result. Presurgical nasoalveolar molding followed by primary rhinoplasty with overcorrection resulted in a nasal appearance that was closer to the patients without cleft lip.

Smile Train was founded with one, clear aim--to help as many children as possible who are needlessly suffering with unrepaired clefts. Its unique "teach-a-man-to-fish" strategy supports local medical professionals to provide free, safe, and high-quality cleft care by offering free education and training opportunities, free equipment, and financial support for cleft treatment. Smile Train is continuing to expand its reach and enhance its ability to serve by developing and implementing new and innovative approaches in the delivery of comprehensive cleft care, so that each and every child born into the world with a cleft has the opportunity to live a full, productive life.

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., (Copyright © 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: This study was the result of a constant evaluation of surgical techniques and results to obtain excellence in primary cleft rhinoplasty., Methods: This was a retrospective study from 1992 to 2003 comparing the long-term outcomes of four techniques of nasal reconstruction. There were 76 patients divided into four groups: group I (n = 23 patients), primary rhinoplasty alone; group II (n = 16 patients), nasoalveolar molding alone; group III (n = 14 patients), nasoalveolar molding plus primary rhinoplasty; and group IV (n = 23 patients), nasoalveolar molding plus primary rhinoplasty plus overcorrection. The surgical results were analyzed using photographic records obtained at 5 years of age. A ratio of six measurements was obtained comparing the cleft and noncleft sides. A panel assessment was obtained to grade the appearance of the surgical results. All surgery was performed by the senior author (P.K.T.C.)., Results: The results are given for groups I to IV, respectively. The nostril height ratio was 0.73, 0.77, 0.81, and 0.95. The nostril width ratio was 1.23, 1.36, 1.23, and 1.21. The one-fourth medial part of nostril height ratio was 0.70, 0.87, 0.92, and 1.00. The nasal sill height ratio was 0.75, 1.02, 1.07, and 1.07. The nostril area ratio was 0.86, 0.89, 0.95, and 1.08. The nostril height-to-width ratio was 0.58, 0.58, 0.71, and 0.92. Finally, group IV had the best panel assessment., Conclusions: The results revealed that group IV had the best overall result. Overcorrection of 20 percent was necessary to maintain the nostril height. Further technical modifications are necessary to minimize widening of the nostril width.

The establishment of a craniofacial in a developing country is often difficult because of the availability of trained personnel. This requires gradually replacing or training personnel such as speech pathologists. Gradual improvement is possible for a period of 5 to 10 years to train individuals. This requires a dedicated person to achieve the goal of a multidisciplined center for patients with cleft.

Objective: To identify and analyze quantitatively the development of the maxillary dental arch before and after cheiloplasty., Design: Prospective, longitudinal study of maxillary dental arch development at age of 1, 3, 6, and 12 months., Setting: All patients were treated at a university hospital craniofacial center., Patients: Twenty-seven infants with nonsyndromic, unilateral complete cleft lip and palate., Intervention: Millard's rotation-advancement cheiloplasty was performed between the ages of 3 and 4 months., Results: The anterior portion of the nonclefted segment (I-G), anterior ridge length of the nonclefted segment (I-C), and anterior ridge length of the clefted segment (L-C') continuously increased from 1 to 12 months of age. The anterior cleft width (G-L), anterior arch depth (I perpendicular to CC'), anterior basal angle (angle GC-CC'), and anterior arch curature angle (angle GIC) continuously decreased after the cheiloplasty., Conclusions: Cheiloplasty could mold the anterior portion of the maxillary dental arch palatally by exerting continuous pressure.

Reconstruction of bilateral cleft lip nose deformity is difficult and the outcome is inconsistent. This study was conducted to evaluate the gross outcome and the difference in the assessment of nasal appearance as judged by two groups of raters, cleft surgeons and laypersons. Sixty-four patients with bilateral cleft lip were selected for review. The patients' ages ranged from 5 to 30 years. All patients had undergone primary cleft lip repair and secondary nasal reconstruction, and had been followed for at least 6 months. One image for each patient, which included a digitized frontal, lateral, and worm's-eye view, was projected for evaluation by the raters. The raters included five cleft surgeons and five laypersons. A rating scheme was used in which a score of 3 was given for a good, close to normal nasal appearance, 2 for an average result that needed minor revision, and 1 for a poor result that needed major reconstruction. The scores were averaged for each patient in each group and for each group as a whole. The final outcome was judged as good, fair, or poor on the basis of the mean score for each patient. Statistical analysis was performed. The mean score for all patients was 2.08 as assessed by the laypersons and 2.18 as assessed by the cleft surgeon group. There was no statistically significant difference between the two groups. Comparisons on rating scores among different raters revealed a fair agreement on the ratings within each of the two groups. The results were found to be good in 29.7 percent, fair in 64.1 percent, and poor in 6.3 percent of patients when evaluated by the surgeons. When rated by the laypersons, the nasal appearance was found to be good in 26.6 percent, fair in 60.9 percent, and poor in 12.5 percent of patients. This difference in distribution between the two groups was not statistically significant. When comparing the results given by the two groups of assessors, there was agreement on the nasal appearance in 65.6 percent of patients, and a difference in grading in the rest. For the patients who received different grading, the surgeons rated them one grade higher in 63.6 percent and one grade lower in 36.4 percent. There was no difference in grading between any of the evaluators that reflected a two-grade discrepancy in evaluation of results. This study shows that the surgical outcome of bilateral cleft lip nose deformity repair, at the authors' institution, is less than optimal. When assessing bilateral cleft lip nose appearance, the judgment of results by cleft surgeons was similar to that of the laypersons. However, different rating of results existed within each of the two groups, supporting the importance of clearly assessing patient/parent expectations and defining realistic surgical goals.

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 non-syndromic 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 non-syndromic 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 non-syndromic 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 non-syndromic 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.

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.

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.

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.

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.

The symmetry of the alar base and the nasal floor is very important in achieving a satisfactory result in primary lip and nasal reconstruction during cleft lip repair. The skeletal base of the nasal pyramid is usually hypoplastic even in incomplete clefts. A periosteal pocket filled with Surgicel can facilitate blood clot formation and can theoretically stimulate bone formation, thereby improving the asymmetry of the bone base. To test this hypothesis, a prospective study was performed in 64 primary lip repairs by comparing the effect of subperiosteal nasal floor augmentation with Surgicel. From February 1989 to June 1993, 64 patients with incomplete cleft lips (excluding occult cleft lip and complete clefts with Simonart's band) were operated by the same surgeon. The patients were divided randomly into two groups. One group had subperiosteal nasal floor augmentation with Surgicel and the other group did not. The lip repair was a rotation-advancement cheiloplasty with primary closed rhinoplasty of the tip and ala. In the first group, a subperiosteal pocket was created under the cleft alar base and the nasal floor. The pocket was filled with Surgicel up the point where the level of the alar base and the nasal floor was symmetrical with the noncleft side. The control group underwent a similar procedure, less the creation of the subperiosteal pocket and the use of Surgicel. All patients were followed for at least 3 years and their photographs were used to compare and analyze the results of their nasal correction. Critical attention was paid to the symmetry of their nasal floor and alar base. The results were evaluated by at least two plastic surgeons for consistency. The data indicate that the control group showed a higher number of subjects with asymmetry (10 of 32) compared with the study group (8 of 32). However, there was no statistical significance to the findings. Two patients in the study group who received Surgicel developed hypertrophic lip scars. This study, although unable to determine statistically the contributory affect of Surgicel in primary nasal reconstruction, does show a possible benefit from the use of osteogenic materials in the primary management of the deficient nasal floor. Hypertrophic scars developed by 2 patients in the study group may point to a possible soft-tissue reaction to the use of Surgicel in primary lip and nasal repair.

The speech problem in patients born with cleft lip and palate is mainly due to the insufficiency of the velopharyngeal (VP) valving mechanism, manifested as in visually seen nasal emission, and auditorily perceived hypernasality and articulation disorders. Evaluations of resonance and VP function can be conducted by: (1) perceptual rating scale based on speech pathologist's subjective judgement and (2) objective instrumental assessment, by the use of specific instrument. Nasopharyngoscopy has been commonly used clinically to diagnose VP function. The purpose of this study is: using nasopharyngoscopy to investigate the differences in VP mechanism between those with VP competence and VP incompetence in the dimension of velar displacement, lateral pharyngeal wall (LPW) displacement, degree of VP closure and pattern of VP closure. Ninty-eight subjects were studied from June 1990 to August 1991. They were divided into 4 groups: (1) group 1: normals with normal speech; (2) group 2: cleft of lip only, with normal speech; (3) group 3: cleft palate without nasal emission, but normal articulation; (4) group 4: cleft palate with nasal emission, with or without articulation error. Age range was from 6-47 years old with equal sex distribution. Ten Chinese sentences were used for standard test sentences, and were grouped according to Mandarin phonological distinctive features into 5 categories: (1) nasals; (2) vowels; (3) plosives; (4) fricatives/affricates; (5) connected number counting. The result indicates that the insufficient velar displacement to contact posterior pharyngeal wall is a determining factor causing velopharyngeal insufficiency in group 4; however, the lateral pharyngeal wall movement is not significantly different among 4 groups. Coronal pattern of velopharyngeal closure is the most common pattern among 4 groups of speakers.

The velopharyngeal dimensions and velopharyngeal functions of 58 patients having a cleft lip and palate were compared with those of 30 normal noncleft subjects. The patient group received perceptual speech assessment, nasopharyngoscopic examination, and lateral cephalogram, and was classified accordingly into three categories: velopharyngeal competency, borderline velopharyngeal competency, and velopharyngeal incompetency. The results indicated that (1) the need ratio differs significantly among the three cleft palate patient groups and (2) when compared to the control group, the velopharyngeal incompetency group has an excessive need ratio, narrower posterior pharyngeal wall, narrower linear dimensions in pharyngeal depth, and shorter velar and hard-palate length. The clinical implication and application of the findings are discussed.

Deficiency of the free border of the lip is frequently encountered in secondary cleft lip deformities. The free border of the lip consists of the orbicularis oris marginalis muscle and its overlying subcutaneous tissue, vermilion, and mucosa. The pathology may involve any or all of these components. When there is an absolute shortage of subcutaneous tissue or orbicularis oris marginalis muscle, extra tissue from another source must be used for augmentation. A temporoparietal fascial graft is a simple and reliable method to increase the fullness of the free border of the lip. The tissue is soft in consistency and displays minimal resorption. Most important, more complicated procedures such as an Abbé flap or tongue flap may be avoided. From July of 1988 until June of 1992, the temporoparietal fascial graft was used successfully to correct deficiencies of the free border of the lip in 20 patients with cleft lip deformities. There were two graft exposures that healed with conservative treatment. The procedure is a useful method of augmenting both lateral lip and central lip deficiencies in secondary and tertiary cleft lip deformities.

The development of the dental arch is well designed for adaptive and compensatory growth. In this study, the relationship between the sleep position and dental arch development was investigated. A group of 42 infants with unilateral complete cleft lip and palate with either prone (16) or supine (26) sleep position were seen in the craniofacial center. All infants were less than 1 month of age at the initial visit. Dental impressions of the maxillary arch were taken at the initial visit and just before cheiloplasty. Ten arch dimensions were measured in each dental cast and the longitudinal change in each dimension was compared between the prone sleep group and the supine sleep group. Statistically significant changes were detected in the growth rate of the following dimensions: intercanine width, intertuberosity width, alveolar cleft width, anterior cleft width, and posterior cleft width. This study indicated that sleep position affected maxillary arch development. Infants sleeping in the prone sleep position tended to have narrower arch width and cleft width.

A patient with Wolf-Hirschhorn syndrome had multiple congenital anomalies and a right cleft lip. Hemangioma involved the full thickness of the upper medial lip segment at the age of 2 months, which caused lip repair to be very difficult. The first attempt at adhesion cheiloplasty resulted in complete separation. The tumor did not respond to steroid treatment. A subsequent trial of lip repair with the assistance of a tension-relieving suture tied to the nasal septum was successful. This type of tension suture is helpful to prevent dehiscence when security of lip closure is questionable or cannot be achieved.

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.

The median facial dysplasia group of patients is a unique, distinct, definable group characterized by mid-line facial deficiencies in the presence of a unilateral or bilateral cleft lip with or without cleft palate and without clinically detectable anomalies of the brain. This group of patients comprised 2 percent of new cleft lip and palate patients. The midline facial deficiencies in median facial dysplasia are characterized by a poorly defined Cupid's bow, absence of the labial frenulum and anterior nasal spine, deficient columella, and poorly developed septal cartilage and premaxilla. One of the central incisors may be absent or rudimentary. There are no definable gross abnormalities of the brain. The results of intelligence tests of median facial dysplasia patients have a normal distribution and are not significantly different from those of a noncleft normal population. Unoperated and operated median facial dysplasia patients all have typical midfacial growth disturbances below the norm for their ages. The classification of median facial dysplasia patients as well as other median cerebrofacial anomalies, the embryologic basis of these deformities, and the clinical results of their treatment are helpful in understanding some of the problems related to the treatment of the cleft lip and palate patient. Median facial dysplasia patients demonstrate an inherent potential for poor midfacial growth. Deficiencies of soft tissue such as a lack of the Cupid's bow make it difficult to reconstruct the lip and nose satisfactorily.

An open tip rhinoplasty was used to correct moderate to severe secondary cleft lip nasal deformities in 122 patients from January 1986 to January 1988. The results of the surgery on 92 patients who were followed for 1 year or more were evaluated. When the surgery was performed after the age of 3 years, 60% of the patients had a satisfactory result. The pathology of the deformity included 32% with deficiencies of the nasal lining, 35% needed framework support such as a columellar strut, and 45% required correction of columellar deficiencies. Patients using a postoperative nasal stent had a 71% satisfactory result compared with a 37% satisfactory result when the stent was not used. The pathology and techniques to correct the nasal deformity are described.

Delayed hard palate closure (after age 6-7) was widely used for palatal repair in cleft palate children from 1976 to 1982. Since 1982, the approach has been revised into early complete closure of the soft and hard palate (before age 2). The articulation skills of 60 children with cleft lip and palate (age 3-5:11) who underwent early complete palate closure were compared with 47 children of similar age with soft palate closure only. The results indicated that the early complete closure group had more children with adequate articulation skills than the delayed hard palate closure group. Detailed analysis of the articulation patterns in each group revealed that the articulation substitution patterns made by the early complete closure group followed the trends of developmental articulation patterns, i.e. unaspirated, stopping and fronting phonological features. On the contrary, the articulation substitution patterns made by the delayed hard palate closure group were more related to structural deficits, i.e. compensatory articulation. Age 3-5 is a critical period for articulation maturation. We advocate that the complete palatal closure should be achieved before age 2 in order to equip cleft palate children with an equally normal oral structures to enhance speech/language development in a normal learning process during this critical period.

Over a period of 8 years 140 bilateral cleft lips were operated using a muscle-repositioning banked fork-flap cheiloplasty. The use of buccal mucosal flaps in the intercartilaginous incision is helpful to decrease scarring and contracture by facilitating alar cartilage repositioning and wound closure without tension. Adding mucosa from the inferior turbinate makes complete wound closure relatively easy without tension. A lateral lip orbicularis muscle flap with white skin roll and vermilion is recommended for reconstruction of the Cupid's bow. Muscle continuity by freeing the muscle in one sheet and repositioning in front of the premaxilla with creation of a buccal alveolar sulcus is stressed to prevent the necessity of reentering the lip in a second procedure. The elongation of the columella is done at 1 to 6 years of age by advancing nasal floor tissue onto the columella and repositioning the alar cartilages superiorly and medially. When nasal floor tissue is inadequate, columellar lengthening is done by the use of a composite free ear graft.

Cephalometric radiographs of a sample of complete unilateral cleft lip and palate patients (15 males, 15 females, aged 6.5 to 7.5 years) were analysed. In most cases, an adhesion cheiloplasty was done at 3 months of age, followed by definitive cheiloplasty at 6 to 12 months, then by posterior palatoplasty at 1.5 years. The hard palate was not repaired till the age of 5-7 years. This preliminary study indicated that: (1) The growth of maxilla and mandible shows no sex difference, except the anteroposterior maxillary length, which is longer in the cleft males. (2) The maxilla of our group is more retruded than that of normal noncleft children. (3) The mandibular growth is essentially normal except the higher mandibular plane in the cleft females. (4) The intermaxillary relation and skeletal profile of the cleft males are more favourable than in the early closure group.

The white skin roll is a useful term to describe the cutaneo-vermilion border of the lip. The muco-vermilion border line parallels the white skin roll and is described as the red line. The lip vermilion should be constructed so that these lines are parallel and widest at the base of the philtral column. It is suggested that the triangular lateral lip vermilion flap be used in unilateral cleft lips. In bilateral cleft lips, a white skin roll vermilion-mucosal muscle flap is used for reconstruction of vermilion.

A combined cleft palate and craniofacial was organized at Chang Gung Memorial Hospital, Taipei, in 1980. The team includes four plastic surgeons, four orthodontists, one prosthodontist, one otolaryngologist, one pediatrician, two speech pathologists, one social worker, one psychologist, two neurosurgeons, one ophthalmologist and a medical artist. Weekly combined clinics and meetings are held regularly to evaluate cleft lip/palate and all craniofacial cases. The team members set up treatment plans for each cleft palate and craniofacial patient, and follow up the patient's facial growth with critical evaluation of the result, complication, relapse and patient's or parent's satisfaction after surgery. Parents' support groups, summer speech camps and conferences between patients, parents and other public resources are held regularly. Longitudinal studies are strictly carried out on specific groups of cleft patients and major craniofacial patients.

Eight patients, aged 9 months to 9 years, each with unusual complete cleft of the primary and secondary palate on one side and a complete cleft of the secondary palate on the opposite side, were evaluated. All showed varying deficiencies of median facial mesodermal dysgenesis characterized by absent frenulum, indefinite cupid's bow, deficient septal cartilage and nasal spine, and absent central and lateral incisor of the cleft side of the premaxilla. Computerized tomographic scan confirmed the absence of the central nervous system anomalies seen in midline clefts. Cephalometric studies revealed maxillary hypoplasia and no evident hypotelorism. These patients demonstrate a definable lack of median mesodermal penetration along with failure of lateral mesodermal penetration. It is to be noted that median facial dysgenesis can occur without recognizable central nervous system disorders. It is quite possible that microforms of median facial dysgenesis occur in unilateral and bilateral cleft lip and palate patients. This would help explain the wide variations of clefting seen clinically.

Of 2963 patients registered at the Chang Gung Craniofacial Center, 1755 patients (906 males and 849 females) who did not have any previous surgery were carefully delineated according to cleft side, involvement of primary or secondary palate and severity of cleft. Cleft lip and palate (43.88%) was the most common cleft, cleft palate (30.88%) the second, while cleft lip (25.24%) the least. More males were found in cleft lip group or cleft lip and palate group, and more females in the cleft palate group. Regarding clefts involving both primary and secondary palates, the cleft of both palates was usually complete, but when the clefts involved either the primary or secondary palate, the cleft was usually incomplete. With regards to the laterality of unilateral cleft lip and/or palate, the left side was affected more than right side. With regard to seasonal variation, cleft palate patients showed the highest incidence from August to October and the lowest incidence from November to January. Cleft lip or cleft lip and palate patients showed the highest incidence from November to February but no obvious difference in the rest of the months. Topographic distribution disclosed that more patients were domiciled in the area north to Hsien-Chu, especially the urban Taipei area.