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PubMed1985

Evaluation and treatment planning for patients with cleft lip and cleft palate.

The Oral-Facial and Communicative Disorders Program of the University of North Carolina has provided comprehensive diagnostic evaluation and inpatient and outpatient treatment for patients with cleft lip, cleft palate, and related problems for 22 years. A major aspect of the program is the initial diagnostic evaluation of patients and long-term follow-up evaluation of the results of treatment. The Oral-Facial and Communicative Disorders Program acts as both a consultant in providing requested information to referring professionals and agencies and also as a multi- and interdisciplinary treatment center. The nature of the evaluation process and the manner in which findings and recommendations are agreed upon in order that a comprehensive and coherent treatment plan can be established has been presented. It has been stressed that the needs of the patient can best be met by representatives from disciplines important to the diagnosis and management of patients with cleft lip and cleft palate who can meet together, and after discussion and negotiation arrive at a consensus for every aspect of treatment that is required, as well as a time that is most desirable for each patient.

Child↗
PubMed1998

Our treatment of cleft lip and cleft palate.

Our principle in cleft lip and cleft palate repair is all-round personal treatment. That is, treatment concentrates not only on cleft lip and cleft palate repair, but also considers the patient comprehensively in terms of his or her personality. In this report, we present patients with cleft lips and/or cleft palates for whom we performed surgical treatments in the past 25 years. We performed primary operations for 402 patients and secondary operations for 120. Cleft lip and cleft palate should be managed with consideration for otolaryngeal aspects as well as plastic surgical ones because of the nature of the disease. We therefore treated our patients from the standpoints of both medical fields, and gained much appreciation from parents whose children suffered from the congenital anomaly.

Child↗
PubMed1995

Epidemiology of cleft lip and cleft palate in Mississippi.

We present an epidemiologic study of the cases of cleft lip and cleft palate in Mississippi from 1980 through 1989. Cases were stratified by race (white and nonwhite) but not by association with a major malformation. During this period, a total of 457 new patients with cleft lip or cleft palate were identified from a population of approximately 439,354 live births. The incidence of total clefts in live-born infants was 1.36/1,000 for whites and 0.54/1,000 for nonwhites. White boys had a higher incidence of cleft lip and palate than white girls; black boys had an extremely low incidence of cleft lip, whereas black girls showed a higher incidence of cleft palate alone. Examination of the rate of cleft malformations for each county in Mississippi revealed no clearly defined pattern, nor were we able to demonstrate that rates were increased in a major agricultural area of the state, in which use of potentially toxic products is high.

Cleft Lip↗
PubMed1984

Parental age and incidence of cleft lip and cleft palate anomalies.

Maternal and paternal age effects have been studied on 90 patients (61 males and 29 females) suffering from cleft lip, cleft palate and, cleft lip and palate anomalies. Younger mothers are at higher risk of getting a child with congenital oral clefts than the older (above 30 years of age). Parental age gaps increase the incidence of congenital malformations.

Cleft Lip↗
PubMed1996

Effects of reverse headgear treatment on sagittal correction in girls born with unilateral complete cleft lip and cleft palate--skeletal and dental changes.

Patients with cleft lip and cleft palate often develop maxillary retrognathism. This is due to the combined effects of the congenital deformity and surgical repairs. Early protraction of the maxilla with extraoral forces helps to achieve more balanced skeletal harmony and favorable occlusion for future growth to occur. The purpose of the present study is to investigate the proportion of the skeletal and dental changes contributing to the improvement in a group of Southern Chinese girls born with unilateral complete cleft lip and cleft palate treated by the reverse headgear. This study only focused on treating a homogenous sample group, i.e., only girls with unilateral complete cleft lip and cleft palate. This design was deliberate so as to avoid having boys and girls with various types of cleft all pooled together for analyses as seen in most of the previous reports. In addition, comparison was made with girls matched in having a similar deformity, presenting a similar skeletal structure and maturity status to reveal the genuine treatment effect. The 9.7 months of reverse headgear treatment improved the sagittal jaw relationship (p < 0.01) and overjet (p < 0.01), which was effected by about two-thirds skeletal and one-third dental changes.

Case-Control Studies↗
PubMed2000

Comparative study between children with and without cleft lip and cleft palate, part 2: electromyographic analysis.

OBJECTIVE: This study was conducted to compare electromyographic (EMG) activity of superior orbicularis oris muscle between children with repaired cleft lip and cleft palate and children without clefts. METHODS: This study included 28 children with mixed dentition. They were divided into two groups. The study group included 14 children with repaired unilateral cleft lip and cleft palate, ranging in age from 6 to 12 years, who presented clinically with a short upper lip, abnormal lip seal, and inhibition of sagittal development of the midface as assessed radiographically. The control group included 14 children without clefts ranging in age from 8 to 11 years. All had normal lip seal, nasal breathing, and a clinically normal body posture. DESIGN: Bipolar surface electrodes were used for EMG recordings of resting level activity and during swallowing of saliva, speech, and chewing and swallowing of an apple. RESULTS AND CONCLUSIONS: A significantly higher level of activity at rest and during swallowing of saliva was observed in the cleft lip and cleft palate group. Similar activity during speech and chewing and swallowing of an apple was observed in both groups. The higher level of activity at rest and during swallowing of saliva in children with cleft lip and cleft palate seems to suggest that upon higher functional demands their activity increases less than in children without clefts. From a clinical point of view, if increased EMG activity at rest and during swallowing of saliva reflects increased force on the maxilla, then our findings may corroborate Bardach's findings (1990) that surgical treatment of cleft lip has an iatrogenic effect on facial growth, although the lack of significant correlation between the cephalometric data and EMG findings in the present study.

Child↗
PubMed1986

Incidence of cleft lip and cleft palate in 39,696 Japanese babies born during 1983.

To estimate the incidence of cleft lip and/or cleft palate among the Japanese, 39,696 Japanese babies born during the period from January 1, 1983 to December 31, 1983, were investigated. It was found that 65 babies (0.163%) had these abnormalities, and that the ratio of the birth of such babies was approximately one per 611 in the population. Of the 65 affected babies, 41.3% presented cleft lip (CL), 46.0% cleft lip and palate (CL/P), and 12.7% cleft palate (CP). These results suggest that the incidence of CL/P in the Japanese is higher than that observed among other races.

Cleft Lip↗
PubMed2003

Identification and characterization of a novel gene disrupted by a pericentric inversion inv(4)(p13.1q21.1) in a family with cleft lip.

Cleft lip with or without cleft palate is a common birth defect affecting 1 in every 700 live births. Several genetic loci are believed to be involved in the pathogenesis of syndromic and non-syndromic clefting. We identified a pericentric inversion of chromosome 4, inv(4)(p13q21) that segregates with cleft lip in a two-generation family. By using a combination of fluorescence in situ hybridization, yeast artificial chromosome, bacterial artificial chromosome contig mapping, and database searching we mapped and sequenced the inversion breakpoint region. The pericentric inversion disrupts a gene (ACOD4) on chromosome 4q21 that codes for a novel acyl-CoA desaturase enzyme. The 3.0 kb human ACOD4 cDNA spans approximately 170 kb and is composed of five exons of ACOD4. The inversion breakpoint is located in the second exon. The 3.0 kb mRNA is expressed at high level in fetal brain; a lower expression level was found in fetal kidney. No expression of ACOD4 was detected in fetal lung or liver or in adult tissues. The five exons code for a protein of 330 amino acids, with a predicted molecular weight of 37.5 kDa. The protein is highly similar to acyl-CoA desaturases from Drosophila melanogaster to Homo sapiens. The catalytically essential histidine clusters and the potential transmembrane domains are well conserved.

Amino Acid Sequence↗
PubMed1993

Dermatoglyphics in cleft lip and cleft palate anomalies.

Dermatoglyphic characteristics of sixty nine cases of cleft lip with or without cleft palate and twenty eight isolated cleft palate cases were evaluated for digital patterns, interdigital patterns, palmar simian crease and sydney line, and model types of C- and D-line terminations. Increased frequency of ulnar and radial loops than the arches and whorls was observed in cleft lip with or without cleft palate patients compared to controls. Interdigital patterns were less frequent in cleft lip and cleft palate patients. Simian crease and Sydney line were more common in patients than in controls. Model types of C- and D-line terminations showed variations in patients and controls. Wider 'atd' angle (more than 30 degrees) and dermatoglyphic asymmetry were noted in the patient groups. The findings suggest the dichotomy or heterogeneity of cleft lip and cleft palate anomalies. The role and utility of dermatoglyphics in genetical etiology of congenital defects were emphasized.

Adolescent↗
PubMed1999

Bilateral infraorbital nerve block is superior to peri-incisional infiltration for analgesia after repair of cleft lip.

Cleft lip repair is a common operation in infants and requires that the child is pain-free during the postoperative period so that handling does not affect the integrity of the delicate surgical site. This study was designed to compare the efficacy and duration of effect of 0.125% bupivacaine given preoperatively as a bilateral infraorbital nerve block with peri-incisional infiltration of the same local anaesthetic for postoperative analgesia in cleft lip repair. It was a randomised, double blind, prospective study in 30 children aged 4-20 months (ASA grade 1). After a standard induction, group A (n = 15) were given a bilateral infraorbital nerve block with 0.125% bupivacaine and group B (n = 15) had peri-incisional infiltration with the same solution. No additional systemic analgesics were given before or during the operation. Intraoperative monitoring comprised measurement of heart rate and blood pressure and post-operatively pain relief was recorded using a behavioural pain relief score. The heart rate, respiratory rate, and blood pressure were also monitored at 0, 1, 2, 4, 8, and 24 hours after tracheal extubation. The results showed that group A had significantly better pain relief (higher scores) than group B for eight hours postoperatively (p < 0.05). The analgesic requirement in group B became significant at two hours postoperatively, while group A had significant analgesic requirements only after eight hours. The significant rise in heart rate and blood pressure that accompanied tracheal intubation in both groups suggested that while both methods of analgesia may be adequate to prevent responses to skin incision, they do not substitute for adequate systemic analgesia during the operation. We conclude that infraorbital nerve block with 0.125% bupivacaine provides better and more prolonged analgesia than peri-incisional infiltration in cleft lip repair.

Analgesia↗
PubMedDate not supplied

Spectrographic measures of the speech of young children with cleft lip and cleft palate.

Twenty-two consecutive children with repaired cleft lip and/or palate [isolated cleft lip (CL) 6, isolated cleft palate (CP) 7, unilateral cleft lip and palate (UCLP) 7, and bilateral cleft lip and palate 2] with a mean age of 27 months underwent spectrographic measures of tape-recorded speech (DSP Sona-Graph digital unit). Controls were 22 age- and sex-matched noncleft children. Data analyzed included (1) the Spanish vocalic variables [a, i, u, e, o]: first formant, second formant, duration, and context; (2) obstruent variables [p, t, k]: burst, voice onset time, and duration, and (3) nasal variables [m]: first formant, second formant, and duration. Statistically significant differences were observed between the CL group and the control group in the first formant of [e] and in the increase of the frequency of the [t] burst. Comparison between UCLP and controls showed differences in the second formant of [a], in the first formant of [o], and in the second formant of [o]. These results suggest a small but significant influence of either the cleft lip or its repair on lip rounding for [o] and [u]. In addition, tongue position differences were most likely responsible for the differences seen with [a] and [e]. Spectrographic differences in the current patients did not contribute to meaningful differences in speech sound development. Individualized care (orthodontics, surgery, speech therapy) in children with cleft lip and/or palate attended at specialized craniofacial units contributes to normalization of speech development.

Articulation Disorders↗
PubMed1989

[Cleft lip and cleft palate in the Craniofacial Center, Chang Gung Memorial Hospital: incidence, sex, seasonality and topographic distribution].

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.

Adolescent↗
PubMed2005

Early assessment of dental arch development in repaired unilateral cleft lip and unilateral cleft lip and palate versus controls.

OBJECTIVE: To evaluate dental arch relationships and dimensions, relative to an age matched noncleft sample, in Caucasian 3-year-old children with repaired unilateral cleft lip (UCL) or unilateral cleft lip and palate (UCLP). DESIGN: Prospective, cross-sectional, case-control study performed in Scotland, U.K. PARTICIPANTS: Eleven children with repaired unilateral cleft lip, 16 children with repaired unilateral cleft lip and palate, and 78 children as controls. MAIN OUTCOME MEASURES: Dental arch relationships and linear arch dimensions. RESULTS: Prevalence of Class III incisor relationship was 31.3% in children with unilateral cleft lip and palate compared with 9.1% in children with unilateral cleft lip. A buccal crossbite was present in 36% of children with unilateral cleft lip, compared with 75.6% of children with unilateral cleft lip and palate.Mean linear maxillary arch dimensions did not differ significantly between children with unilateral cleft lip and the controls. Except for second intermolar width, statistically significant differences existed in mean linear maxillary arch dimensions between the unilateral cleft lip and the unilateral cleft lip and palate groups; the mean linear maxillary arch dimensions were significantly greater in the control group than in the unilateral cleft lip and palate group. The mean cleft-affected anterior quadrant length appeared to be the arch dimension with the greatest power of discrimination among the three groups. There were no significant differences in mean linear mandibular arch dimensions among the three groups. CONCLUSIONS: Anterior crossbite was almost three times more common in the unilateral cleft lip and palate group than in the unilateral cleft lip group. Mean linear maxillary arch dimensions differed significantly between the unilateral cleft lip and palate group and the control group. There were no significant differences in mean linear maxillary arch dimensions between unilateral cleft lip and controls or between mean linear mandibular arch dimensions for unilateral cleft lip, unilateral cleft lip and palate, and controls.

Analysis of Variance↗
PubMed1984

Population and family studies of HLA in Japanese with cleft lip and cleft palate.

Population and family studies of HLA were performed in Japanese patients with cleft lip and/or cleft palate (CL/P). Frequency of HLA-Cw 7 was significantly increased in cleft lip (CL) patients (37.5%) and cleft palate (CP) patients (37.8%) but was not increased in cleft lip and palate (CLP) patients (17.5%), compared with control subjects (13.3%). However, the intensities of the associations were not great (relative risk = 4.0). The affected sib pairs method was studied in 13 families with CL- or CLP-affected sib pairs and 10 families with CP-affected sib pairs. However, in both groups of families the distributions of HLA haplotypes in affected sib pairs did not significantly differ from random Mendelian expectation. Thus, HLA-linked major genes (loci) which determine the development of CL/P were not found. These results seem indirectly to support the multifactorial theory of CL/P, but does not exclude other possible genetic mechanisms.

Cleft Lip↗
PubMed1999

Congenitally missing second premolars in cleft lip and cleft palate children.

Panoramic and periapical radiographs of 278 patients with cleft lip, cleft palate, or both (158 males and 120 females), age 5 to 18 years, were examined to determine the frequency of missing second premolars and the possible association between the cleft side and the side from which the premolar was absent. The prevalence (18%) of missing premolars found in this study is significantly higher than is found in the general population. A considerably higher incidence of missing second premolars was found in the maxilla compared with the mandible both for unilateral and bilateral missing teeth. The second premolar was absent more frequently on the left than on the right side, both in males and females and in both jaws. Interestingly but consistent with our previous reporting, this corresponded to the side where clefts occurred more often. The cause of the higher prevalence of left-sided clefts and missing second premolars in human beings is not known at the present time.

Adolescent↗
PubMed2005

[Anesthetic management of pediatric cleft lip and cleft palate repair].

We have developed a modern strategy for the anesthetic management of pediatric cleft lip and cleft palate repair using anesthetic drugs such as sevoflurane, desflurane, acetaminophen, remifentanil, and pirtitramide together with new techniques. It provides best conditions for the surgeon and maximum safety for the pediatric patient. A team of pediatricians, neonatologists, pediatric surgeons, and pediatric anesthetists have tackled the problem of management of children with craniofacial abnormalities such as cleft lip and cleft palate. The best and safest anesthetic techniques are outlined and the most frequent complications are discussed, e.g. management of the difficult airway, the airway in patients with complex craniofacial abnormalities, fiberoptic endotracheal intubation through a laryngeal mask, intraoperative dislocation of the endotracheal tube, postoperative airway obstruction and perioperative bleeding.

Abnormalities, Multiple↗
PubMed1980

Cleft lip and cleft palate in Santo Domingo.

The purpose of this study was to analyze the occurrence of isolated cleft lip (CL), cleft lip with cleft palate (CL+CP) and isolated cleft palate (CP) and their distribution according to sex and laterality in Santo Domingo, Dominican Republic, located in the Caribbean Archipelago. The sample consisted of 439 hospital records (204 males and 235 females) of patients attending a children's public hospital in Santo Domingo over the period of May 1973 to December 1976. Of all facial clefts, the highest percentage (36.4%) was presented by CL, followed by CP (32.1%) and CL+CP (31.4%). Of all facial clefts, males presented the highest percentage (53.5%). For both sexes, there was an equal number of cases with (17.54%) but more males had CL+CP (0.20 > P > 0.10) and more females presented CP (P < 0.001). The left-sided defects were almost twice as common as the right-sided defects. The ratio of unilateral clefts-to-bilateral clefts was 5.4:1.

Cleft Lip↗
PubMed1991

Treatment of cleft lip and cleft palate.

The first case of cheiloplasty recorded was in China at about 200 BC. The technique of course has been vastly improved over the centuries. Cleft lip and/or cleft palate are among the most common congenital anomalies in China and the world. There is controversy over some of the surgical techniques used and age for operation, especially in cleft palate, but the condition requires surgery. Unilateral cleft lip is operated on at 3-6 month of age and bilateral cleft lip at 6-12 month of age. Views on palatoplasty are: 1. Cleft palate should be repaired at an early age; 2. Supplementary orthodontic treatment to expand the upper arch postoperatively is necessary, and orthognathic surgery is performed if needed when the patient has matured. This is the best choice at present.

China↗