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Development of the lip and palate in staged human embryos and early fetuses.

Cleft lip and palate are the most common congenital malformations in humans. Using 43 staged human embryos and early fetuses ranging from the 4th to 12th week of development, we investigated the development of the lip and palate in order to provide the basic developmental concepts required for managing these anomalies. The lower lip appeared as bilateral mandibular arches at Carnegie stage 11, and these were completely merged at stage 15. The components of the upper lip, medial nasal prominence and maxillary process, appeared at stage 16, and completely merged at stage 20. The median palatine process appeared at stage 16, and the lateral palatine process, at stage 17. The palatine processes and the nasal septum started to fuse abruptly at stage 23, and from external observation seemed to be fused at the 9th week. However, complete fusion did not take place until the 12th week of development. The tongue was prominent at stage 16, showed differentiation of the muscular tissue at stage 21, and was located superior to the lateral palatine process before stage 23. These results may be used in understanding the different mechanisms present in the formation of various congenital anomalies in this region.

Female↗

The status of oral hygiene in cleft lip, palate patients after surgical correction.

UNLABELLED: The cleft lip and palate patients usually present a number of problems viz. altered oral anatomy leading to changes in oral physiology diminishing the self-cleansing ability of individual. The handicapped children are unable to maintain their oral hygiene properly. The present study was formulated with the aim that does normalization of oral anatomy have its effect on improvement of oral hygiene? An assessment of oral hygiene index-simplified was performed between preoperative and postoperative values in the same patient at KGMU and KGDU. A total of 50 cases were recorded in two groups of 25 each: (i) 6 years. The observations are statistically analyzed by paired 't' test to get the significance of results. RESULTS: The data analyzed showed the significant decrease in oral hygiene indices observed in both groups. A relative significance in oral hygiene status following surgery was observed. Both groups expressed greater significance when compared pre and postoperatively which is indicative of considerable improvement of oral hygiene after surgical correction. The study concludes that oral hygiene improves more in older cleft lip-palate cases following reconstruction of palatal vault, premaxilla and anterior lip seal by secondary bone grafting method when compared with oral hygiene indices results in primary periosteoplasty cases. The surgical correction of cleft lip palate enhances self-cleaning ability and better compliance to maintain oral hygiene in children as the age advances.

Age Factors↗

The effect of tongue appliance on the nasomaxillary complex in growing cleft lip and palate patients.

Midfacial deficiency is a common feature of cleft lip and palate patients due to scar tissue of the lip and palate closure procedure. The aim of this study was to evaluate the effectiveness of the physiological force of the tongue to move the maxilla in forward position. This research has been done experimentally by, before and after treatment following up in private practice. Ten patients (6 female, 4 male) with complete bilateral cleft lip and palate were selected. All of them had Cl III malocclusion with maxillary deficiency due to scar tissue of lip and palate surgery. Their age ranged from 7.6 to 9.8 years. All the patients were delivered tongue appliance to transfer the force of the tongue to maxillary complex. The mean observation time was 13+/-2 months to achieve positive overjet. Pre- and post-lateral cephalograms were compared to evaluate the skeletal changes with paired t-test. The results showed that after the application of tongue appliance, normal sagittal maxillomandibular relationship was achieved. SN-ANS angle was increased 1.9+/-1.8 - P < 0.03. This study showed that the tongue appliance could transfer considerable force during rest and swallowing period to the maxilla. This method might be considered to improve the deficient maxilla by means of growth modification and redirect concept.

Cephalometry↗

[Integrated treatment of cleft lip and palate. Organization of a treatment team].

OBJECTIVE: Collect the team experience in the treatment of children with cleft lip and palate, indicating the evolution of the team composition, advantages and improvement aspects, trying to transmit the need of team treatment. METHOD: The Bilbao cleft palate team was created in 1983, since then a cleft palate clinic, a parents group and a unit of velopharyngeal function has been developed. At present the team is composed by: pediatric reconstructor surgeon, speech therapist, orthodontist, dentist, pediatrician, ENT, maxillofacial surgeon, dismorphologyst, geneticist, nursing. RESULTS: One of the achievements has been the data unification, obtaining speech cephalometrics, photographic dental casts and video images with prospective view. At this time 403 cleft lip and palate children have been intervened, being essential the transdisciplinar team approach between surgeon, speech therapist and orthodontist. The importance of the team coordinator is pointed. The results of an audit of the two stage cleft palate closure in complete unilateral cleft lip and palate have obligated us to vary our surgical policy. The unresolved aspects are the lack of multidisciplinary team recognition at official level and the non existence of orthodontist in staff, without cost coverage of this treatment by public health system. CONCLUSIONS: In our experience the team treatment of cleft lip and palate has resulted in improvement of the clinic results, treatment protocols and training.

Adolescent↗

Increased occurrence of cleft lip in glycogen storage disease type II (GSDII): exclusion of a contiguous gene syndrome in two patients by presence of intragenic mutations including a novel nonsense mutation Gln58Stop.

Genetic deficiency of lysosomal acid alpha-glucosidase (acid maltase) results in the autosomal recessive disorder glycogen storage disease type II (GSDII) in which intralysosomal accumulation of glycogen primarily affects function of skeletal and cardiac muscle. During an earlier review we noted 3 in 100 cases of GSDII with incidental description of cleft lip. In addition, we identified 2 of 35 GSDII patients referred to us for molecular studies with co-occurence of cleft lip, considerably greater than the estimated frequency of nonsyndromic cleft lip with or without cleft palate of 1 in 700 to 1,000. Because several lines of evidence support a minor cleft lip/palate (Cl/P) locus on chromosome 17q close to the locus for GSDII, we defined the molecular basis for the GSDII in these two patients to determine if they represented a contiguous gene syndrome. Patient I (of Dutch descent) was homozygous and the parents heterozygous for an intragenic deletion of exon 18 (deltaex18), common in Dutch patients. Patient II was heterozygous for delta525T, a mutation also common in Dutch patients and a novel nonsense mutation (172 [corrected] C-->T; Gln58Stop) in exon 2, the first coding exon. The mother was heterozygous for the delta525T and the father for the 172 [corrected] C-->T; Gln58Stop. The finding that both patients carried intragenic mutations eliminates a contiguous gene syndrome. Whereas the presence of cleft lip/cleft palate in a patient with GSDII could be coincidental, these co-occurences could represent a modifying action of acid alpha-glucosidase deficiency on unlinked or linked genes that result in increased susceptibility for cleft lip.

Base Sequence↗

[Management of cleft lip and/or palate diagnosed in utero].

OBJECTIVE OF THE STUDY: To assess the usefulness of fetal karyotyping in the management of facial cleft lip with or without cleft palate diagnosed during pregnancy and to determine which etiologic and prognosis criteria are helpful to consider for prenatal counselling. MATERIAL AND METHODS: Retrospective study on 35 cases of facial cleft lip and/or palate prenatally diagnosed by ultrasound examination and managed in our fetal medicine unit from 1 January 1989 to 31 December 1996. Complete follow-up was obtained for all fetuses. RESULTS: In our series, the mean gestational age at diagnosis was 25 weeks. In 43% of the cases, additional sonographic anomalies were also recognized. These associated anomalies were more frequent when amniotic fluid quantity was abnormal. Fetal chromosomal determination was conducted in 2/3 of fetuses and numeric or structural abnormalities were found in 20% not related to the cleft size or type (lip and/or palate). All fetuses with isolated facial cleft were chromosomally normal. We report 4 midline clefts, all of them were associated with additional sonographic findings and 3 were part of an holoprosencephaly. CONCLUSION: Prenatal diagnosis of cleft lip and/or palate must draw attention to associated sonographic anomalies especially when amniotic fluid quantity is abnormal. Chromosomal karyotyping is not necessary when facial clefting is isolated except in cases diagnosed early in pregnancy. Midline clefts must draw attention on cerebral midline integrity.

Abnormalities, Multiple↗

The mathematics of cleft lip and palate treatment evaluation: measuring the desirability of treatment outcomes.

A study was undertaken to investigate the applicability of utility theory to cleft lip and palate treatment decision making. Questionnaires were used to assess value judgements and attitudes toward risk from 119 individuals associated with seventeen cleft lip and palate treatment centers. Significant differences were found to exist between the values expressed by clinicians grouped according to their specialty or the facility to which they were affiliated. Fathers of cleft lip and palate children differed from cleft palate teams in their judgements regarding the relative values of speech and cosmetic treatment outcomes. No such differences were found between team members and mothers, however. It is suggested that these differences affect the desirability of treatment decisions and the outcomes that follow from them. Therefore, cleft lip and palate treatment planning and the evaluation of alternative therapeutic interventions might be based on a mathematical theory of decision making that explicitly incorporates the subjective assessment of a child's habilitative needs and the goals of the habilitative process.

Cleft Lip↗

Patients' evaluation of the final result of sagittal split osteotomy: is it influenced by impaired sensitivity of the lower lip and chin?

The purpose of this article was to evaluate whether neurosensory disturbances such as impaired sensitivity of the lower lip and chin influence patients' final evaluation of the treatment result. Information about the patients' degree of satisfaction and about lip and chin sensitivity were obtained from final follow-up documents of 215 patients. All patients had undergone sagittal split ramus osteotomy for mandibular advancement. In patients with normal sensitivity on both sides of the lower lip and chin, the degree of satisfaction was equally distributed at a very high level through all age groups. Among those with some degree of numbness on either or both sides of the lower lip and chin, patients in the younger quarter and middle half of the group were as satisfied as those with normal sensitivity, while those in the oldest quarter with impaired sensitivity demonstrated a lower degree of satisfaction than the rest of the patients. Although the difference was not statistically significant, the finding strongly indicates that older patients seem to suffer more from neurosensory disturbances than do younger patients with similar conditions.

Adolescent↗

Double lip: an unusual presentation.

Double lip is a term used to describe a deformity of the upper or lower lip and consists of an accessory fold of redundant mucous membrane inside the vermilion border. Double lip is an uncommon congenital anomaly and is usually diagnosed and treated by a dentist or an oral surgeon. We present an unusual case of double lip--unusual because it was diagnosed and treated by a dermatologist and because of the histopathologic findings.

Adult↗

Dynamic correction of unilateral paralysis of the lower lip.

We present a simple operation, performed under local anesthesia, to restore essential movement to the side of a lower lip paralyzed by injury to the mandibular branch of the facial nerve. The operation consists of a limited lateral resection of the paralyzed lower lip, plus transposition of the segment of orbicularis muscle from the paralyzed lower lip to the normal upper lip, through a subcutaneous tunnel.

Adolescent↗

An effective treatment of dark lip by frequency-doubled Q-switched Nd:YAG laser.

BACKGROUND: Dark lip is a common cosmetic problem in Southeast Asia. There is no known effective treatment. OBJECTIVE: To propose an effective method for treating dark lips of varying causes with frequency-doubled Q-switched Nd:YAG laser. METHODS: Seventy patients with dark lip, of which 22 were congenital, 24 acquired, and 24 of uncertain cause, were treated by frequency-doubled Q-switched Nd:YAG laser at a fluence of 2-3.5 J/cm2 (mode 2.5 J/cm2) after application of topical anesthesia. The endpoint of treatment was complete clearance of the pigment. Follow-up time was 24-36 months (mean 29 months). RESULTS: All patients attained complete clearance of the lesion after an average of 2.5 treatments in the congenital group, 2.2 treatments in the acquired group, and 1.8 treatments in the group with uncertain etiology. The mean (+/-SD) number of treatments required by the whole group was 2.1 +/- 1.4. Recurrence was observed in one case of congenital origin 3 months after the last treatment. In the remaining cases, results persisted up to the time of follow-up. Herpes simplex reactivation was noted in one case 3 days after treatment. There was no dyschromia, scar formation, or change of skin texture. CONCLUSION: Dark lip can be effectively treated by frequency-doubled Q-switched Nd:YAG laser without major adverse effects.

Adolescent↗

[Pseudoprognathism frequency and masseter muscle activity in operated cleft lip patients].

BACKGROUND: As a consequence of a growth inhibition of middle third of the face, cleft lip surgery could lead to a pseudoprognathism. However, this is not always the case. Also, when facial architecture is changed, the activity of muscles such as the masseter could be disturbed. AIM: To study masseter electromyographic activity in operated cleft lip patients. MATERIAL AND METHODS: Forty two patients with cleft lip (25 male, aged 13 to 35 years old) were studied. All were operated during their first year of life. An electromyography of masseter muscle was performed and from a teleradiography, ANB and Gonion angles were obtained. Forty healthy subjects conformed the control group. RESULTS: The sample was divided in two groups. A group of 20 patients had a significantly lower EMG voltage than control subjects (662 +/- 228 and 1511 +/- 340 microV respectively, p < 0.001). Only seven patients had an anteriorly inverted bite with a mean ANB of + 1.7 and a mean molar relation of -4.8, figures that confirm the presence of pseudoprognathism. CONCLUSIONS: Eighty three percent of cleft lip patients do not have cephalometric parameters of pseudoprognathism and half of the patients have a lower masseter activity.

Adolescent↗

Management of the cleft lip deformity.

Repair of the unilateral cleft lip deformity is a challenging and rewarding procedure. Historically, many techniques have been described to reconstruct the unilateral cleft lip. These have included straight-line repairs and various geometric flap closures. The rotation-advancement flap technique of Millard is a reliable and versatile method for repair of the unilateral cleft lip deformity. This technique allows lip repair and tip rhinoplasty while camouflaging the scars in the newly formed philtral border. If properly applied, the rotation-advancement repair produces excellent functional and aesthetic results (Figs. 18A and 18B).

Cleft Lip↗

Premolar development in Greek children with cleft lip and palate.

OBJECTIVE: The purpose of this investigation was to study the development of the premolars in a sample of Greek children with cleft lip and palate. METHOD AND MATERIALS: The study material consisted of panoramic radiographs of 34 individuals (22 boys and 12 girls) with cleft lip with or without cleft palate. The boys ranged from 9.0 to 14.0 years of age and the girls ranged from 8.0 to 14.5 years of age. The mean age was 11.3 years. Each panoramic radiograph was matched with a corresponding one of an unaffected individual of the same sex and chronologic age (+/- 3 months). The panoramic radiographs were used to categorize the stages of tooth development according to the 10 stages of mineralization described by Nolla. The assessment was repeated by a second observer. RESULTS: Individuals with clefts exhibited a statistically significant delay of formation and mineralization of the maxillary right and left second premolars and the mandibular right second premolar in comparison to individuals without clefts. In the cleft group, there was no statistically significant difference between boys and girls in premolar development. No asymmetric development of contralateral premolar teeth was observed in individuals with cleft lip and palate. CONCLUSION: Although the exact biologic explanation remains to be found, the formation of premolars is delayed in children with cleft lip and palate.

Adolescent↗

[The study and application of the alar cartilage ring in the treatment of deformities of unilateral cleft lip nose].

OBJECTIVE: To explore the possible pathogenesis and to look for effective reconstructive procedures of unilateral cleft lip nose. METHODS: The author has had a detailed comparative study by comparing the cartilaginous skeleton of 20 normal cadaver specimens with that of one unilateral cleft lip stillborn, as well as intraoperative findings of 24 clinical patients. RESULTS: There are two alar cartilaginous rings, namely alar cartilaginous fibrous ring and alar cartilaginous muscular ring, in external nose. Both rings are hypoplastic in the cleft lip patients. According to the findings, the author designed and applied a new operation procedure to reconstruct alar cartilage ring for 24 patients from 1994 to 1997. An acceptable short-term results were found. CONCLUSION: Alar cartilaginous fibrous ring and alar cartilaginous muscular ring are very important anatomic structures to maintain the shape of the lower part of the nose. Hypoplasia of these rings is an important factor leading to deformities of the nose. It is very important to reconstruct alar cartilage ring for the patients with unilateral cleft lip nose.

Abnormalities, Multiple↗

Management of an infant with cleft lip and palate with phocomelia in dental practice.

Cleft lip and palate is a severe birth defect occurring approximately one in 800-1000 newborn infants. The incidence varies widely among races. Cleft lip and palate together account for approximately 50% of all cases whereas isolated cleft lip and isolated cleft palate occur in about 25% of cases. Many of these congenital anomalies appear to be genetically determined though the majority are of unknown causes or teratogenic influences. Presented here is a 3 day old infant with bilateral cleft lip and palate and phocomelia for whom a feeding obturator was made and delivered to facilitate feeding.

Cleft Lip↗

Caries experience and oral health behavior in Chinese children with cleft lip and/or palate.

PURPOSE: The aims of this study were to investigate whether there were different caries levels in 3- to 6-year-old Chinese children who had a cleft lip compared to those with a cleft lip/palate. The goal also was to evaluate parental attitudes toward the feeding habits and oral health care for their children. METHODS: A cross-sectional study was carried out through a dental examination of a sample of children and a questionnaire to their parents. A sample of 104 3- to 6-year-old children (66 boys and 38 girls) with cleft lip, cleft palate or both were selected from those receiving pre-surgical treatment. Each child was examined and a short questionnaire was given to parents of the children. The form included questions about infant feeding practices, frequency consumption of specific drinks and, children's toothbrushing frequency. RESULTS: One hundred and four children were examined. Seventy-five percent had some caries experience and rampant caries was present in 26%. Children with cleft palate had a higher prevalence of rampant caries (30%) compared to those with cleft lip (12%). A multivariate analysis yielded feeding practices and mother's education as the variables significantly associated with caries and rampant caries. CONCLUSIONS: The results of this study show that: 1. Children with a cleft lip/palate have higher levels of dental caries compared to those with a cleft lip alone; 2. The two most important factors for dental caries were: a. whether the child had been bottle-fed and; b. the educational attainment level of the mother.

Bottle Feeding↗

[Carcinoma of the lips developing in discoid lupus erythematosus].

OBJECTIVE: This clinical study was designed to learn the diagnosis and treatment of lip carcinoma developing in discoid lupus erythematosus. METHODS: One hundred and eighty-one of squamous cell carcinoma of the lips treated in our department from 1980 to 1995 were retrospectively studied. RESULTS: Ten cases of the 181 lip carcinoma (5.5%) had concomitant discoid lupus erythematosus (DLE) of the vermilion border of the lower lip. CONCLUSION: Bleomycin or radiotherapy is the best treatment for the disease.

Adult↗