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Development of the object permanence concept in cleft lip and palate and noncleft lip and palate infants.

Studies of language acquisition in cleft lip and palate infants have not investigated the development of specific cognitive concepts, such as object permanence, which may be related to early linguistic skills. This study obtained comparative data on the development of the object permanence concept in cleft lip and palate and noncleft lip and palate infants to determine whether there were significant differences in rate or sequence of development. Infants were tested for the object permanence concept from 12 through 18 months of age. Results revealed significant improvement in all infants' scores with age, indicating progressive development of the concept. Further, while scores were not significantly different between the cleft lip and palate groups, scores for these groups were significantly better than scores for noncleft lip and palate infants. Superior performance of the cleft lip and palate infants may have resulted from increased environmental stimulation provided by their parents. Implications for intervention and future research in this area are presented.

Attention↗

Sri Lankan cleft lip and palate study model analysis: clefts of the secondary palate.

The study models of a group of adult Sri Lankan patients with clefts of the secondary palate were investigated. Tooth-size and arch-dimension comparisons were made with a comparable control group. Significant differences were found between the cleft and control groups in tooth sizes, chord lengths, and arch widths. The cleft group dimensions were generally smaller than those of the control group. Overjets were larger in the cleft group.

Adult↗

Anaesthesia for cleft lip surgery--challenge in rural Nepal.

Cleft lip and palate is quite common in Nepalese population residing in hilly areas and interior of the country. Nepalese doctor with limited resources and fund and International aided team of fully equipped doctors from developed world conduct operative procedure in the centre or district hospital setup quite frequently. But very few patients will come to the hospital for treatment as it is not an emergency for them. Anaesthesia for cleft lip and palate surgery carries a high risk and difficulty in airway management. Associated congenital anomalies further complicate the management. Still a team of Nepalese physicians are providing this service in a field. How to provide a safe anaesthesia was a main concern for an anaesthesiologist with a limited supply. During the last three years we have anaesthetized safely four hundred children age between 2 months to 14 years. The team worked at an altitude ranging from 100 feet to 10,000 feet of Nepal in a temporary erected operation room of the village. Most of the operation was conducted under total Ketamine anaesthesia and general anaesthesia with halothane. In our context we found Ketamine anaesthesia was safe and effective anaesthetic technique in simple clefts in children more than 2 years. This technique reduces the cost by minimizing the supply to be carried in the field.

Adolescent↗

The history of treatment of cleft lip and palate.

The history of surgery of cleft lip and palates reaches as far backwards as the prechristian era to 390 B.C. when for the first time a cleft lip was closed successfully in China. Although Egyptian and Greek medicines developed to a remarkable degree, no descriptions of cleft operations have survived. In the middle ages operations on cleft lip have been several times described. A successful operation on a cleft palate did however not occur until 1816. This can be explained by the fact that cleft palates were thought to be secondary to syphilis, but also because without anaesthetic this operation was extremely painful and difficult. Graefe in 1816 [20a] and Roux in 1819 [49] published the first satisfactory results. After the introduction of chloroform cleft surgery made remarkable progress. The development of cleft surgery has been chronologically described and finally the present state of affairs is discussed.

Child↗

The prevalence of congenital heart disease among the population of a metropolitan cleft lip and palate clinic.

The entire infant population of the Tufts-New England Medical Center Cleft Palate Clinic was reviewed, and the prevalence rates of congenital cardiac anomalies were calculated. The overall prevalence rate of congenital heart disease among the facial cleft clinic population was 6.7%, with a prevalence rate of 9.1% among patients with submucous cleft palate, 7.7% with congenital palato-pharyngeal incompetence, 0% with cleft lip only, 5.3% with unilateral cleft lip and palate, 12.5% with bilateral cleft lip and palate, 4.3% with cleft palate only, 13.6% with Pierre-Robin anomaly, 7.1% with cleft lip and palate and a syndrome diagnosis, 16.7% with cleft palate and a syndrome diagnosis.

Child↗

[Cleft lip: apropos of 80 cases].

Patient, with cleft lip do not go unnoticed because the malformation is obvious. But in our countries it's not rare to receive some teenagers and even adults with cleft lip: among the patients of our series 20% were more than 14 years old. Cleft lip was unilateral in 92.5% of the cases. All these patients were operated on by a same E.N.T. specialist's team using a same method: the modified rotation--advancement of MILLARD Results were judged good in 85% of the cases. Minimum fare of malformation must begin at birth by many specialist.

Adolescent↗

Thermoplastic resin used to modify an alveolar orthopedic prosthesis in a patient with cleft lip before cheiloplasty: a clinical report.

Correction of cleft lip and cleft palate is a multidisciplinary team effort. Severe bilateral clefts with protruded and rotated premaxillae may complicate surgical correction. Prosthetic intervention to reposition segments before surgical correction may improve the bony base for tissue molding and avoid excess tension at the surgical site. A conventional bulb prosthesis with bonnet and straps is often used as an extraoral device to retract a protruded premaxilla for severe cleft patients before cheiloplasty. Sometimes, because of lack of retention, stability, patient compliance, and controllable forces from a bonnet and straps, movement of the premaxillae to a desirable position is difficult to achieve. The use of thermoplastic resin to modify a bulb prosthesis as described in this clinical report is a simple method to provide good retention for the bulb prosthesis and to move a laterally displaced premaxillae to the facial midline. The wedging effect created by the thermoplastic resin generated "compressive forces" to the premaxilla that was more controllable than the pulling forces created by the bonnet and straps therapy. Thermoplastic resin is also a better material than a conventional chemically polymerized acrylic resin in dealing with an infant's delicate mucosa before cheiloplasty.

Cleft Lip↗

[The range of congenital malformations associated with cleft lip and palate].

The congenital malformations associated to cleft lip and cleft palate cover a wide range of pathologies with incidence and acuteness that vary according to the authors. In the 284 patients followed and/or referred to the Cleft Consultation of Dona Estefânia Hospital, 78 had associated malformations (27.5%). In three cases there was consanguinity in parents. A familial incidence of cleft and/or other congenital malformations was found in 13 and 10 patients respectively. Thirty patients had malformative syndromes. In the remaining 48, 127 associated congenital malformations (M.C.A.) were identified, in accordance with the Smith criteria, 81 major and 46 minor. The most frequent associated malformations are those of the face (25.9%) and of the cardiovascular system (16.5%). The multiple anomalies (of various systems) are the most frequent (47.9%), followed by the isolated anomaly (29.1%) and the multiple of one system (22.9%). In what concerns the association of syndromes with the type of cleft, primary, secondary or total palate, those of the secondary were the most frequent, particularly that of the Pierre Robin sequence (S.P.R.)--19 in 36 secondary cleft palate.

Abnormalities, Multiple↗

The bacteriology of children before primary cleft lip and palate surgery.

OBJECTIVE: Bacterial infections can complicate any surgery. Knowledge of potentially pathogenic bacterial flora in children with cleft lip and palate allows appropriate risk management, including the need for prophylactic antibiotics. This project reviewed the bacteriology of children before primary cleft lip and palate surgery. DESIGN: A retrospective study of the results of nose, throat, and ear microbiological swabs taken from children, aged 1 to 26 months, before repair of primary cleft lip, cleft palate, or both was carried out. Swabs with Staphylococcus aureus and beta-hemolytic streptococcus were considered positive. RESULTS: From October 1987 to May 2002, 321 primary cleft lip or palate operations were performed in 250 patients. Results from 326 sets of preoperative swabs were available, including five repeat sets from patients whose operations were postponed. There were 235 (72.1%) negative sets and 91 (27.9%) positive sets. Of the positive swabs, 86 sets grew S. aureus, and 10 sets grew beta-hemolytic streptococcus. CONCLUSIONS: Children with unrepaired cleft lip and palate have a significant risk of carrying S. aureus and a small risk of carrying beta-hemolytic streptococci. These risks need to be considered when deciding on protocols for preoperative bacteriology tests and prophylactic antibiotics.

Antibiotic Prophylaxis↗

[Analysis of 5459 cleft lip and palate cases].

OBJECTIVE: To analyse the constitution of 5459 cleft lip and palate cases and to review the development of surgical treatment to them. METHODS: The data of the 5459 cleft lip and palate cases were collected and classified. The ratio of sex and disease pattern were calculated and analyzed statistically. Also, by means of classification of the disease pattern, the operation methods and the operation time, the change of the surgical methods and the first operation age were analyzed. RESULTS: In the cleft lip and palate cases, the sex ratio is different. There were obvious differences in the first operative ages in different periods. The main repair methods for cleft lip and palate changed with time. CONCLUSION: The male is more than the female in the cleft lip and palate patients. The average first operation age has been decreased and the main repair methods have changed since 1980.

China↗

Targeted scan of fifteen regions for nonsyndromic cleft lip and palate in Filipino families.

Cleft lip with or without cleft palate (CL/P) is a congenital anomaly with variable birth prevalence based on geographic origins, with the highest rates commonly found in Asian populations. About 70% of cases are nonsyndromic (NS), in which the affected individual has no other abnormalities. NS CL/P is a complex disorder with genetic and environmental effects and no specific genetic loci yet confirmed. Fifteen candidate regions were examined for linkage to NS CL/P. Regions were chosen based on previous suggestive linkage and/or association in human families, or suggestive animal model data. Polymorphic markers in these regions were genotyped for analysis on 36 Filipino families comprised of 126 affected and 218 unaffected individuals. An additional 70 families with 149 affecteds were used for replication of suggestive results. Parametric (LOD score) and nonparametric (SIMIBD) linkage analyses were performed as well as transmission disequilibrium test (TDT) analysis. Five markers yielded suggestive results from the 36 families. The parametric LOD scores for the MSX1-CA and D4S1629 were >1.0 and the SIMIBD P values for D6S1029 and RFC1 are suggestive (<0.06), while the SIMIBD P value of 0.01 for TGFA was significant. Since the Msx1 mouse knockout has cleft palate and MSX1 mutations have been found in rare cases of syndromic CL/P, this locus is especially plausible for linkage. Previous studies have also found linkage of NS CL/P to 4q31 and 6p23. These regions contain several candidate genes, including AP2 at 6p23 and FGF2, BMPR1B, and MADH1 at 4q31. TGFA has both linkage and linkage disequilibrium data supporting it as a candidate gene for NS CL/P. While no region was definitively confirmed for linkage to NS CL/P, the data do support further investigation using larger sample sizes and candidate gene studies at 2p13.2, 4p16.2, 4q31, 6p23, and 16q22-24.

Chromosome Mapping↗

Cystathionine beta-synthase c.844ins68 gene variant and non-syndromic cleft lip and palate.

Non-syndromic cleft lip with or without cleft palate (CL/P) is a common birth defect with substantial clinical and social impact and whose causes include both genetic and environmental factors. Folate and homocysteine (Hcy) metabolism have been indicated to play a role in the etiology of CL/P, and polymorphisms in folate and Hcy genes may act as susceptibility factors. We investigated a common polymorphism in the cystathionine beta-synthase (CBS) gene (c.844ins68) in 134 Italian CL/P cases and their parents using the transmission disequilibrium test (TDT). Although no overall linkage disequilibrium was observed, considering the parent-of-origin transmission of the CBS 68 bp insertion a significant (P = 0.002) transmission distortion was detected. When children receive the c.844ins68 allele from the mother compared to the father, they show a 18.7-fold increase in risk for CL/P. This evidence suggests CBS as a candidate gene for CL/P and supports a role of maternal-embryo interactions in the etiology of CL/P.

Alleles↗

Size of the cranium in parents and their children with cleft lip.

Fifty-two children with cleft lip with or without cleft palate), CL(P), fifty-three non-cleft children, and their respective parents were studied. Area measurements for three craniofacial components of brain case, upper face and lower face were obtained from lateral and frontal roentgenographic cephalograms. The most important finding of this investigation was that the CL(P) patients and their parents had a significantly smaller brain case than did the control subjects. A small brain case may well be one morphological characteristic predisposing toward the cleft anomaly.

Cephalometry↗

The relationship between lip pressure and facial growth after cleft lip repair: an experimental study.

Two different methods of lip repair were used on rabbits with surgically created unilateral clefts of the lip, alveolus, and palate. Control groups involved both unoperated animals and those with unrepaired surgically created clefts. The resulting lip pressure in the rabbits with repaired cleft lips was found to be significantly higher than in the control groups. Direct skull measurements showed that the rabbits whose lips had been surgically repaired also had significantly shorter maxillae that did the control groups. The correlation found between the amount of lip pressure and the degree of growth inhibition indicated a causal relationship.

Animals↗

[Cleft lip and palate in Campeche Mayas].

It has been suggested that among American Indians, as in some genetically-related Asiatic ethnic groups, incidence of cleft lip and/or cleft palate is higher than among people of Caucasian extraction. Such hypothesis, plus growing demand for services observed at a center for the surgery of cleft lip and cleft palate in Campeche state, led the authors to undertake research among the Maya residents of that region. However, neither careful review of case histories nor field research performed in several Indian communities could confirm the hypothesis of a higher incidence among this ethnic community.

Cleft Lip↗

Assessment of the patient with cleft lip and palate. A developmental approach.

Children with cleft lip and palate require interdisciplinary team care from infancy through adolescence. An understanding of developmental stages allows the cleft palate team to adapt and integrate its services into the rapidly changing life of the child. This article discusses the maturational, developmental stages of childhood and the services the child with cleft lip and palate and the child's family deserve through each stage. Health care providers in all settings may continue to provide appropriate care for all patients with cleft lip and palate, despite the challenges of a changing health care environment, by emphasizing the needs of the child in all developmental stages.

Adolescent↗

[Repair of bilateral cleft lip with unilateral composite lip flap: report 19 patients].

To repair bilateral cleft lip seems to be more difficult than that of unilateral one. In order to correct insufficiency of fore-lip, we have applied the composite lip flap of the thicker side for bilateral cleft lip in 19 patients. Overall results were satisfactory. There was no peculiar appearance indicating that fore-lip is too long or tension in the postoperative follow up from six months to four and half years. In this article, the methods of rotating repair were described in detail. The advantage and key points of the teehnique were discussed. So it may be regarded as one of the ideal measures for repairing bilateral cleft lip.

Child, Preschool↗