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The diamond vermilion flap--a new technique for vermilion augmentation in cleft lip repair.

OBJECTIVE: In most cleft lips patients, the vermilion on the medial aspect of the repair is deficient in height. Not many surgeons have dealt with ways to augment the height of the vermilion at this location. In the unilateral cleft lip, the repair may include the insertion of a small triangular vermilion flap from the cleft side into an incision made for it in the muco-vermilion junction of the noncleft side. We have developed a new technique to further augment the vermilion in incomplete cleft lip patients. METHOD: Vermilion remnants may be present in these patients on the scar tissue between the margins of the cleft. A diamond-shaped vermilion flap based on its attachments to the orbicularis oris muscle may be transposed into the noncleft side to an incision made caudal and parallel to the muco-vermilion border. Then the triangular vermilion flap may be inserted just distal to the island flap. Using this method, the vermilion is augmented and the muco-vermilion line parallels more the white skin roll.

Cleft Lip↗

Soft tissue changes following maxillary osteotomies in cleft lip and palate and non-cleft patients.

A retrospective study of 25 patients with unilateral cleft lip and palate and 25 patients with hypoplastic maxillae without a cleft was carried out to evaluate the effects of maxillary osteotomies at the Le Fort I level on the lip and nose profile. The pre-surgical cephalometric tracing was superimposed twice on the post-surgical cephalogram, on the cranial structures and the anterior maxillary structures, for landmark movement measurement. Results showed statistically significant correlations between soft and hard tissue movement in the cleft group. On average, the ratios of horizontal nasal tip, nasal base and lip movement to underlying hard tissue movement were approximately one fourth, one half and two thirds respectively. The ratio of vertical lip to incisor movement was about one half. The correlations were less significant in the non-cleft group, only the upper lip movement showed statistically significant correlation with hard tissue movement, with a ratio of one half horizontally and one third vertically. It was concluded that in maxillary osteotomy, the cleft group showed a higher soft tissue to hard tissue movement ratio. The correlation between soft and hard tissue movements were more statistically significant in the cleft group than in the non-cleft group. However, though statistically significant, the level of correlation was not strong on an individual basis except in the horizontal lip response of the cleft group. Individual variation was wide and clinical judgement needs to be considered accordingly.

Adult↗

Evaluation of speech intelligibility for children with cleft lip and palate by means of automatic speech recognition.

OBJECTIVE: Cleft lip and palate (CLP) may cause functional limitations even after adequate surgical and non-surgical treatment, speech disorders being one of them. Interindividually, they vary a lot, showing typical articulation specifics such as nasal emission and shift of articulation and therefore a diminished intelligibility. Until now, an objective means to determine and quantify the intelligibility does not exist. METHOD: An automatic speech recognition system, a new method, was applied on recordings of a standard test to evaluate articulation disorders (psycholinguistic analysis of speech disorders of children PLAKSS) of 31 children at the age of 10.1+/-3.8 years. Two had an isolated cleft lip, 20 a unilateral cleft lip and palate, 4 a bilateral cleft lip and palate, and 5 an isolated cleft palate. The speech recognition system was trained with adults and children without speech disorders and adapted to the speech of children with CLP. In this study, the automatic speech evaluation focussed on the word accuracy which represents the percentage of correctly recognized words. Results were confronted to a perceptive evaluation of intelligibility that was performed by a panel of three experts. RESULTS: The automatic speech recognition yielded word accuracies between 1.2 and 75.8% (mean 48.0+/-19.6%). The word accuracy was lowest for children with isolated cleft palate (36.9+/-23.3) and highest for children with isolated cleft lip (72.8+/-2.9). For children with unilateral cleft lip and palate it was 48.0+/-18.6 and for children with bilateral cleft lip and palate 49.3+/-9.4. The automatic evaluation complied with the experts' subjective evaluation of intelligibility (p<0.01). The multi-rater kappa of the experts alone differed only slightly from the multi-rater kappa of experts and recognizer. CONCLUSION: Automatic speech recognition may serve as a good means to objectify and quantify global speech outcome of children with cleft lip and palate.

Adolescent↗

Maxillary and sella turcica morphology in newborns with cleft lip and palate.

OBJECTIVE: The first aim was to examine maxillary developmental fields by analyzing bone size parameters within the maxillary bone complex in newborns with unilateral cleft lip (UCL) and unilateral cleft lip and palate (UCLP). The second aim was to evaluate sella turcica morphology in unilateral cleft lip and unilateral cleft lip and palate. SUBJECTS AND METHODS: Axial and profile radiographs from 40 newborns (boy-girl, 1:1) in each group (20 unilateral cleft lip and 20 unilateral cleft lip and palate) were randomly selected among radiographs taken for optimizing treatment planning. Analysis of maxillary bone size was performed on axial radiographs and size parameters were measured. Furthermore, analysis of sella turcica morphology was performed on profile radiographs. The results were divided into groups with normal morphology and severe deviations in the morphology. RESULTS: The maxillary areas were significantly shorter and broader in unilateral cleft lip and palate than in unilateral cleft lip. A profound asymmetry in the maxillary areas was seen in unilateral cleft lip and palate, but not in unilateral cleft lip. In both cleft types, approximately half of the individuals had deviations in sella turcica morphology. The most severe deviations occurred in newborns with unilateral cleft lip and palate. CONCLUSIONS: In newborns with unilateral cleft lip and palate, the maxillary areas are significantly shorter, broader, and more asymmetric than in newborns with unilateral cleft lip. The present study showed that bone structures are a suitable parameter for characterizing the craniofacial developmental fields. Additionally, a high incidence of deviations in sella turcica morphology might indicate that this area is affected in individuals with clefts.

Cephalometry↗

Psychological study of cleft palate children with or without cleft lip by kinetic family drawing.

To clarify how cleft palate children with or without cleft lip perceive their family, we applied the Kinetic Family Drawing (KFD) technique to 60 cleft palate children (with or without cleft lip). The children were aged 7-9 years (in the 1-3 grade of elementary school) and attended the Department of Oral Surgery, Nagoya University Hospital, between 1990 and 1997. Controls consisted of 100 normal elementary school children of the same age. Major findings were: (1) Compared with normal children, cleft lip and palate boys and cleft palate only girls drew their self-images significantly more often in region D (lower right) and in region A (upper left), respectively. (2) Cleft palate children with or without cleft lip felt anxiety and fear toward their family, rarely viewing their homes as a restful place; this suggested insecurity. Uniquely dynamic relations were also suggested in their homes. (3) Cleft palate children with or without cleft lip often perceived their father or mother as persons easily acceptable in society. Rather remote psychological distance between parents and cleft palate children with or without cleft lip was suggested.

Activities of Daily Living↗

Effect of ENU (ethylnitrosourea) mutagenesis in cleft lip and/or palate pathogenesis in mice.

Cleft lip and/or palate (CL/P) are caused by many factors. The aim of this study was to investigate the effects of genetic point mutations in CL/P pathogenesis. ICR and AJ strain mice were used. Ethylnitrosourea (ENU) was injected into 10-week-old male mice (G0) intraperitoneally at a dose of 250 mg/kg. The males were bred with two untreated virgin females of the same strain on day 100 after injection. The uterine contents (G1) of one female were examined on day 18.5 of pregnancy. From the other female, the offspring were delivered naturally, and F3 mice (G3) were also examined in the same way. In ICR strain mice, cleft palate only (CPO) was increased in both the G1 and G3. The frequency was significantly higher in the G3 than the G1 generation. Cleft lip was not observed. In AJ strain mice, CL/P increased in both the G1 and G3. In the G3, the frequency of CPO was increased significantly. Genes related to CPO may be recessive in phenotype. CPO and cleft lip differ from a genetic viewpoint. Point mutations play a significant role in cleft lip and palate.

Animals↗

Natural history of fetuses with cleft lip.

The prospect of fetal surgery for cleft lip is predicated on our ability to accurately identify fetuses with clefts and exclude those that have associated anomalies. Prenatal ultrasound is currently the most appropriate means with which to do this. We reviewed the ultrasonographic data from two large perinatal referral institutions to determine the natural history of fetuses with cleft lip who may be candidates for fetal surgery. Forty fetuses had a cleft lip diagnosed prenatally by ultrasound. In this group, severe associated anomalies were common (30 of the 40) and multiple (23 of the 40) in a majority of fetuses. Life-threatening anomalies, such as central nervous system and cardiac anomalies, were the most common defects. As a result, many fetuses were aborted therapeutically or died in the perinatal period. Out of 12 surviving fetuses, only six had isolated clefts, and two surviving fetuses, diagnosed with isolated cleft lip, had no defect identified postnatally. This information has important implications for the perinatal management of fetuses with cleft lip and the potential role of fetal intervention.

Abnormalities, Multiple↗

Brief report: cleft lip and palate: longitudinal behavior and relationships of cleft conditions to behavior and achievement.

Analyzed longitudinal behavior ratings (ANOVAs) for 44 children (ages 4-12) with cleft lip and palate and cleft palate only (CLP) and examined the relationship of speech, hearing, and facial disfigurement at age 9 in predicting behavioral characteristics and school achievement (multiple regression). Results indicate increasing levels of social inhibition over age for girls with CLP, but less so for boys. There were also increasing levels of conduct problems for older girls with CLP with variable age fluctuation for boys. Attempts to relate speech, hearing, or facial disfigurement to prediction of behavior or achievement at age 9 were not revealing and further analysis with larger samples is indicated.

Achievement↗

Cleft lip and handedness: a study of laterality.

Research on the malformation cleft lip with or without cleft of the palate is complex and may involve studies of etiology, pathogenesis, natural history, and other disease associations. One of the more intriguing areas of study is developmental lateralization. The 3 extant reports of the relationship between laterality of cleft lip and handedness are in conflict. The purpose of the present study is an attempt to resolve the confusion and to explore possible hypotheses that could explain the data. A random sample of 149 probands with unilateral cleft lip +/- palate was ascertained from 2 Los Angeles area hospitals. Laterality of clefting and handedness was determined for each, the former by inspection and the latter by the method of Oldfield [1971]. Information was also obtained on the nuclear family about the number of sibs, presence of clefting, and handedness of the probands' parents. The probability of non-right-handedness (NRH) was much greater for probands with left-sided cleft lip than those with right-sided cleft lips; this was independent of the proband's sex. The cleft lip laterality in probands was independent of parental handedness, suggesting that the significantly increased frequency of left-sided clefting and NRH is embryologic in origin and may have a common etiology and/or pathogenesis. Several explanations are proposed and discussed.

Biometry↗

[Management of cleft lip and palate in university hospital of Rouen].

Cleft lips and cleft palates are managed in the department of Pediatric surgery in Rouen for the last 30 years. From the antenatal diagnosis, the parents got in touch with the surgeon who will coordinate this management. Around thirty new patients are treated every year. The chronology of the treatment is of "classic" manner. The cleft lip is repaired at about 3 weeks of age and the palatoplasty is performed after the age of 1 year. In view to maintain the intimacy of the consultation we did not institute multidisciplinary consultations. The other members of the interdisciplinary team will intervene during the follow up depending on the form of the cleft and the encountered problems. The information and the files circulate freely and are discussed together.

Academic Medical Centers↗

A monozygotic conjoined twin pregnancy discordant for laterality of cleft lip.

Conjoined twins discordant for phenotype of cleft lip are reported. One had right-sided cleft lip, while the co-twin had left-sided cleft lip. Both twins had a normal 46,XY karyotype. Genotyping at 50 polymorphic loci revealed that the twins were identical (monozygotic). Our analysis suggests that the laterality of cleft lip is more affected by the process of twinning than by genetic factors.

Adult↗

Genetic variation in transforming growth factor alpha: possible association of BamHI polymorphism with bilateral sporadic cleft lip and palate.

Non-syndromic cleft lip with or without cleft palate (CL/P) is one of the most common birth defects affecting 1/1000 Caucasians. Genetic factors are thought to contribute to the development of this disorder. A significant association between two restriction fragment length polymorphisms, the TGF alpha TaqI 2.7-kb allele and the TGF alpha BamHI 40-kb allele, at the transforming growth factor alpha (TGF alpha) locus and the occurrence of clefting has previously been reported. A total of 98 Caucasian patients of Alsacian ancestry was recruited from our registry of congenital malformations. These patients had isolated CL/P but no other anomalies. In addition 57 patients with cleft palate, but without cleft lip, were studied. A control group comprised 99 unrelated healthy Caucasians of the same Alsacian ancestry. TaqI and BamHI identify two-allele polymorphisms. The TGFA Taq and BamHI alleles showed no significant association with the presence of clefting, the only exception being that the BamHI 10.0-kb allele was significantly more frequent in patients with bilateral CL/P.

Child↗

From birth to maturity: a group of patients who have completed their protocol management. Part I. Unilateral cleft lip and palate.

The optimal management of the cleft lip and palate patient from birth to completion of treatment presents a formidable challenge to the plastic surgeon and the associated health care system. The multidisciplinary team approach for the management of these patients is widely accepted. However, a paucity of literature exists discussing specific protocol management, interventions, and the long-term outcomes of patients who have completed a strict treatment protocol with a consistent multidisciplinary team. The aim of this study was to present the details of the specific management protocol at the Australian Craniofacial Unit for cleft lip and palate patients and to present a group of patients who have completed this specific protocol and discuss the details of their long-term care. During a 28-year period from 1974 to 2002, the records of 337 patients treated for unilateral cleft lip and palate were evaluated. Of these 337 patients, 22 have completed the same specific protocol management. The same surgeon (David, the senior author) has been responsible for performing all operative interventions and for overseeing the care of each of the 22 patients, ensuring that the treatment protocol has been executed appropriately and without deviation. The interventions and outcomes were analyzed on the basis of speech, hearing, nasal airway, occlusion, psychosocial adjustment, and appearance. Because of the large volume of data and potential differences in outcomes, the authors' intention is to present this as part I of a four-part series beginning with unilateral cleft lip and palate. The results of isolated cleft palate, isolated cleft lip, and bilateral cleft lip and palate will be presented as parts II, III, and IV, respectively. Speech results were assessed as normal speech, mild abnormality, or severe abnormality by objective measures, and intervention for velopharyngeal insufficiency was noted. Seventeen patients were rated as having normal speech. Four patients were rated as having mild speech abnormality, one patient was rated as having severe speech abnormality, and seven patients required surgery for velopharyngeal insufficiency. Hearing results were measured objectively, and good hearing results were obtained in 18 cases. Five patients required tympanoplasty. All patients required alveolar bone grafting. The high Le Fort I osteotomy was performed in six cases. Bimaxillary surgery was performed in one case. Of all the patients assessed from birth to maturity, 13 required between three and five surgical interventions, and nine required six operations or more. Further details and photographs of preoperative and postoperative examples are provided.

Adolescent↗

The major locus for multifactorial nonsyndromic cleft lip maps to mouse chromosome 11.

Cleft lip with or without cleft palate, CL(P), a common human birth defect, has a genetically complex etiology. An animal model with a similarly complex genetic basis is established in the A/WySn mouse strain, in which 20% of newborns have CL(P). Using a newly created congenic strain, AEJ.A, and SSLP markers, we have mapped a major CL(P)-causing gene derived from the A/WySn strain. This locus, here named clf1 (cleft lip) maps to Chromosome (Chr) 11 to a region having linkage homology with human 17q21-24, supporting reports of association of human CL(P) with the retinoic acid receptor alpha (RARA) locus.

Animals↗

Developmental dental changes in isolated cleft lip and palate.

Isolated cleft lip and/or palate, CL(P), may be associated with multiple changes in the developing dentition. To test the hypothesis that permanent tooth formation is delayed in patients with CL(P), dental maturity was assessed from panoramic radiographs. The dental maturity ratio (DMR, dental age divided by chronological age) of a group of CL(P) patients (23 girls, 30 boys) was compared with matched control subjects (38 girls, 41 boys). The mean DMR in cleft boys (0.97 +/- 0.01) was significantly lower than in control boys (1.06 +/- 0.01), P < 0.05. The mean DMR in cleft boys was lower than in cleft girls (1.02 +/- 0.02), with a tendency toward statistical significance. No significant difference in DMR was found between the cleft versus control girls, or between the control girls and boys. Among cleft boys, the prevalence of dental age delay was 67% (20/30), with a mean delay of 0.6 +/- 0.4 years. These results suggested that CL(P) may be associated with delay in permanent tooth formation.

Analysis of Variance↗

Primary definitive nasal correction in patients presenting for late unilateral cleft lip repair.

Almost 25 percent of unilateral cleft lip and palate patients present with their deformity in their teens or later years in the developing world. Because more than 80 percent of the world population lives in the developing world, the established protocol for repair of these deformities is not applicable to these patients. Despite the magnitude, there are no significant reports in the literature that deal with this problem. Several issues need to be addressed, but the author limits himself here to the correction of the nasal deformity. The patients at this age are very much concerned with the aesthetic outcome. Procedures described hitherto for primary nasal correction in infants are not successful in restoring nasal shape and symmetry at this late age of presentation. Our experience with radical correction of secondary nasal deformity in unilateral cleft lip patients presenting late prompted us to extend the concept by undertaking a definitive primary correction of the nasal deformity in cleft patients presenting late. Twenty-two patients with unilateral cleft lip deformity (nine male patients and 13 female patients) with ages ranging from 13 to 22 years, presenting between August of 1997 and December of 2000, are included in this study. Of these, 11 patients had a cleft of the lip alone, eight also had a cleft of the alveolus, and three had a cleft of the palate continuous with the cleft lip. All patients showed some maxillary hypoplasia. An external rhinoplasty with lip repair was carried out in all patients. The corrective procedures on the nose included columellar lengthening; augmentation along the pyriform margin, nasal floor, and alveolus using bone grafts; submucous resection of the nasal septum; repositioning of lower lateral cartilages; and augmentation of nasal dorsum by bone graft. Clinical follow-up ranged from 4 to 24 months, and the median follow-up period was 13 months. Results have been very good, and much better than results seen earlier with other primary rhinoplasty techniques. While repairing unilateral cleft lip in adolescents, the author thinks it would be most appropriate to address the entire gamut of the deformity in a single stage, provide complete vector reorientation, and augment the hypoplastic elements by autologous tissue. It is not just the fear of poor follow-up, but that merely correcting the lip deformity in these patients without attempting definitive rhinoplasty, in the author's opinion, would be insufficient surgical intervention.

Adolescent↗

Use of osseointegrated implants to support a maxillary denture for a patient with repaired cleft lip and palate.

Although many cleft lip and palate patients retain their dentition, some develop partial or complete edentulism. The configuration of the residual maxillary alveolar ridge may provide little support for a complete or partial maxillary denture. The success of the Branemark dental implant has been well established, and although placement of implants in the maxilla may present a surgical challenge, implants are very effective in providing support and, therefore, retention and stability. Application of implants to the cleft palate maxilla is particularly valuable because of the difficulty in constructing well-fitting conventional dentures for CLP patients, and the relatively compromised nature of the prostheses when made. This report presents the prosthetic treatment of a nearly edentulous cleft lip and palate patient.

Cleft Lip↗