Cytokeratin expression in palatal and marginal mucosa of cleft palate patients

OBJECTIVE: The margin of a palatal cleft is a unique anatomical site since the palatal mucosa is continuous with the nasal or nasopharyngeal mucosa. The aim of this study was to compare the expression patterns of cytokeratins and basal membrane components of the mucosa in the area of the cleft. DESIGN: Biopsies from the mucosa of the hard palate and from the cleft margin in the soft palate were obtained from five patients during the primary surgical closure of the cleft. The tissues were processed for haematoxylin-eosin staining and for immunohistochemistry. Antibodies against the cytokeratins (CK) 4, 7, 8, 10, 13, 16 and 18, and the basal membrane components heparan sulphate (HS) and collagen type IV (CIV) were used for immunostaining. RESULTS: The nasopharyngeal epithelium was thinner than the epithelium of the soft palatal mucosa, and showed less interpapillary ridges. The nasopharyngeal epithelium was stratified but expressed the keratins of a simple epithelium (CK 7, 8 and 18). The expression pattern abruptly changed into that of a typical non-keratinized stratified epithelium (CK 4, 13) at the transition to the soft palatal epithelium. The epithelium of the hard palate was a fully differentiated, keratinized and stratified epithelium (CK 10, 16). The basal membrane was thinner in the nasopharyngeal epithelium, which might be related to the presence of abundant inflammatory cells. CONCLUSION: The area around the palatal cleft showed three different types of epithelium. There was an abrupt transition in phenotype of the epithelium from the oral side to the nasopharyngeal side.     

Cleft palate formation after palatal fusion occurs due to the rupture of epithelial basement membranes

2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD) induces cleft palate and hydronephrosis in the mouse embryo. Cleft palate occurs due to failure in palatal grow, but the underlying mechanisms are unclear. We investigated the mechanisms of cleft palate development in TCDD-exposed mouse embryos. We administered olive oil (control group) or TCDD diluted in olive oil (40 μg/kg) via gastric tubes to pregnant mice on gestational day (GD) 12. Embryos of control and TCDD-exposed groups were removed from pregnant mice on GD 14 and GD 15, respectively. One mouse embryo from the control group had anteroposterior palatal fusion. Palatal fusion was observed in three TCDD-exposed mouse embryos. Palates of TCDD-exposed mice fused from the interior to the middle of the palates, while the palates were separated in the posterior region. The middle of the embryonic palatal shelves in TCDD-exposed animals was narrow and split at the fusional position. At this position, palatal and blood cells were dispersed from the palatal tissue and the epithelium was split, with a discontinuous basement membrane. The results suggest that decreased intercellular adhesion or insufficient tissue strength of the palatal shelves may be involved in the development of cleft palate following palatal fusion.     

Rabbit palatal mucosa sialoglycoproteins: solubilization and biosynthesis in vitro

An extraction scheme is presented for rapidly and almost quantitatively solubilizing tissue sialoglycoproteins by selective means. Central to this approach is the use of protease-free bacterial collagenase for obtaining “collagen-associated” molecules from the extracellular matrix. Evidence is presented, based upon turnover rates during biosynthesis in vitro, that the extraction scheme is effective in solubilizing sialoglycoproteins from distinct “compartments” of the tissue. Of specific importance is the presence of connective tissue glycoproteins in the soluble compartment and the observation that the collagen-associated glycoproteins have the lowest turnover, befitting molecules of extracellular location.     

Cleft palate in a patient with Williams' syndrome

Cleft lip or palate has not been reported in the medical literature as a part of Williams' syndrome. We present a patient who had cleft palate among other congenital manifestations. This patient's immediate postnatal period clinically seemed to have a Pierre Robin sequence. With the development of the craniofacial complex, microgenia and micrognathia with glossoptosis gradually became apparent. On further assessment, the patient showed other clinical findings that suggested a syndromic association. This required a complete evaluation to discard other conditions that present with low psychomotor development and distinctive facies, such as Kabuki syndrome or fetal alcohol syndrome. The diagnosis for Williams' syndrome was established based on the clinical features and supported by the fluorescent in situ hybridization test. Williams' syndrome has been described as a rare, congenital disorder characterized by physical and developmental problems. Common features include characteristic "elfin-like" facies, supravalvular aortic stenosis, hypercalcemia, low birth weight, slow weight gain, feeding problems, impulsive and outgoing personality, limited spatial skills and motor control, and intellectual disability. Although individuals with Williams' syndrome may show competence in areas such as language, music, and interpersonal relations, their IQs are usually low and they are considered moderately to mildly retarded.     

Mitosis in epithelium of murine palatal mucosa in vivo and in vitro

Colchicine-arrested metapheses were accumulated at half-hourly intervals over a period of 5 hours in the palatal epithelium of adult male mice. From the rate of entry into mitosis, which was reasonably rectilinear, the mean cell cycle time of the basal layer in vivo was found to be 80.6 h and the duration of mitosis 1.4 h. When a similar accumulation of arrested metaphases was attempted over a period of 4 hours in palatal epithelium in vitro , the rate of entry into mitosis was not rectilinear and the mitotic activity was much lower than in vivo. It is concluded that this in vitro technique is not satisfactory for determining cell cycle characteristics in oral epithelium of the living animal.     

Cleft palate in two syndromes.

Patients with cleft palates with or without cleft lip need more special attention at birth when the cleft is associated with other malformations. For example, the Pierre-Robin and the Klippel-Feil Syndromes are both well known for the special problems they present. Reported here are three cases, with Crouzon's and two with Franceschetti Syndromes, all of whom have palatal clefts.     

Cleft palate rehabilitation using a resin-bonded split-post prosthesis

For the patient described in this report a nonprecious metal alloy that contains neither beryllium nor nickel was used. The retainers were sandblasted with 50 microns alumina and cemented by using a resin that bonds chemically to metal. With good oral hygiene and regular review the resin-bonded split-post prosthesis should provide a long period of service. In contrast to conventional fixed restorations, failure of this type of prosthesis is not highly destructive and other restorative options are still possible if failure occurs.     

Cleft lip and palate in Tehran.

Seventy-nine cleft lip and/or palate births were isolated from 21,138 live births between January 1, 1983 and December 31, 1988 in one hospital in Tehran. Among these, 21 (26.58 percent) were cleft lip (CL), 45 (56.96 percent) were cleft lip and palate (CLP), and 13(16.45 percent) were cleft palate (CP). Chemical sulfur mustard gas was indicated as a major factor in 30 (37.97 percent) of the bilateral cleft lip and palate infants.     

Cleft lip and palate: a review for dentists

The goals of primary closure of cleft lip and palate include not only re-establishing normal insertions for all of the nasolabial muscles but also restoring the normal position of all the other soft tissues, including the mucocutaneous elements. Conventional surgical wisdom, which recommends waiting until growth is complete before undertaking surgical correction of the postoperative sequelae of primary cheiloplasty, carries with it many disadvantages. If, after primary surgery of the lip, orolabial dysfunctions remain, they will exert their nefarious influences during growth and will themselves lead to long term dentofacial imbalances. These imbalances can significantly influence facial harmony. Unless accurate, symmetric and functional reconstruction of the nasolabial muscles is achieved during the primary surgery, not only will the existing dentoskeletal imbalances be exaggerated, but other deformities will be caused during subsequent growth, among which the most important are nasal obstruction and mouth breathing, reduced translation of the maxilla, dysymmetry of the nose and inability of the patient to symmetrically project the upper lip     

Cleft palate repair using a marginal musculo-mucosal flap.

OBJECTIVE: To describe a modified procedure consisting of a mucoso-periosteal flap palatoplasty with a marginal musculo-mucosal flap (3M flap). This is also the first report of a primary repair for complete cleft palate using the 3M flap. We describe the lengthening effect of the nasal mucous layer of the soft palate and evaluate the fistula formation rate associated with this method. METHODS: This procedure has been performed on 21 patients with unilateral complete clefts and on 27 patients with incomplete clefts. A mucoso-periosteal flap raised from the hard palate was used mainly for closure of the cleft and not for the push-back. The 3M flap repaired the deficit of the nasal mucosa, making sure that the soft palate was lengthened. Intravelar veloplasty was performed also. RESULTS: The dimension of the nasal mucosal defect that can be filled with the 3M flap is 10 to 12 mm in length, oriented anterior-posterior, and 15 to 20 mm wide. Oronasal fistula formation was recognized in only 3 of 48 cases (2 of 21 complete clefts, 1 of 27 incomplete clefts) and were located at the hard-soft palate junction at the anterior portion of the 3M flap. CONCLUSIONS: This method has the theoretical advantages of (1) preventing fistula formation by filling the tissue deficiency with the 3M flap; (2) achieving better velopharyngeal function due to elongation of the soft palate and retropulsion of the muscular bundle, utilizing the 3M flap; and (3) minimizing maxillary growth retardation by adopting a non-push-back method of hard palate repair.     

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 CL (17.54 %) but more males had CL + CP (0.20 > P0.10) 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.     

Collagen scaffolds implanted in the palatal mucosa

Scar formation after repair of the cleft palate leads to growth impairment of the upper jaw and midface. The implantation of a suitable scaffold during surgery may reduce this adverse effect. However, little is known about tissue reactions to scaffolds implanted in the oral cavity. Our goal was to analyze the tissue reactions to cross-linked type I collagen scaffolds after submucoperiosteal implantation in the palate of rats. Collagen type I scaffolds were implanted in the palate of 25 male Wistar rats. Groups of 5 rats were killed consecutively after 1, 2, 4, 8, and 16 weeks and were processed for histologic and immunohistochemical analyses. After 1 and 2 weeks, 3 rats from the sham group were also killed. On hematoxylin and eosin-stained sections, the cell density and the number of giant cells were determined. Blood vessels, inflammation, and the presence of myofibroblasts were detected by immunohistochemistry. An influx of inflammatory cells started after 1 week but had completely subsided after 8 weeks. Myofibroblasts were observed within the scaffolds only in the first 2 weeks. Angiogenesis already started after 1 week and showed a peak after 4 weeks, slowly declining afterward. The scaffolds were gradually integrated within the host tissue and only elicited a mild and transient inflammatory response. The scaffolds were biocompatible and seemed to be promising for future applications in cleft palate surgery.     

小鼠胚胎腭突间充质细胞体外培养及生物学特性研究

目的 研究小鼠胚胎腭突间充质（EPM）细胞的生物学特性。方法 在显微镜下解剖妊娠第13天的母鼠胚胎腭突，用0.25%胰蛋白酶进行消化获得游离分散的EPM细胞，在含10％胎牛血清的DMEM培养基中进行培养。采用免疫组化方法进行细胞鉴定，通过相差显微镜，生长曲线及透射显微镜观察细胞形态，增殖能力及超微结构。结果 EPM细胞为成纤维细胞样细胞，排列无序，细胞核大，核分裂象多，核膜内陷，核呈分叶状，胞浆含大量线粒体及粗面内质网。免疫组化显示角蛋白标记为阴性，S－100蛋白及波形蛋白标记为阳性。EPM细胞呈指数生长，有较强的增殖能力。结论 EPM细胞体外培养生长及增殖良好，可作为腭裂基础研究较理想的研究对象。     

Cleft palate in Kabuki syndrome: a report of six cases.

Kabuki syndrome is a syndrome of rare congenital anomalies that was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. Although cleft palate is a feature that is sometimes observed in patients with Kabuki syndrome, there are few clinical reports of cleft palate associated with Kabuki syndrome. This report presents six cases of Kabuki syndrome with cleft palate and reviews their clinical features. Our results suggest that (1) patients with cleft palate in Kabuki syndrome tend to fail in acquiring normal velopharyngeal function and (2) submucous cleft palate might be more common in patients with Kabuki syndrome than previously was reported.     

Cleft palate and toe malformations in a child with fetal methotrexate exposure

Methotrexate, a commonly administered chemotherapeutic agent, is a well-known human teratogen. Exposure of a fetus between 6 and 8 weeks of gestation is postulated to cause birth defects. However, fetal exposure to this drug after this critical period is thought to have little to no effect on eventual fetal development and growth. The authors report a case of an infant whose mother was exposed to methotrexate during pregnancy. The infant was evaluated at their clinic for an incomplete cleft palate and associated asymmetric deformities of the toes on both feet.