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Two- and three-dimensional sonographic assessment of the fetal face. 2. Analysis of cleft lip, alveolus and palate.

OBJECTIVES: To describe the sonographic appearance of cleft lip with or without cleft palate (CL +/- P) using two-dimensional and three-dimensional (3D) ultrasound imaging. Also, to evaluate the accuracy of ultrasound to delineate with precision the bony extent of facial clefts, i.e. to differentiate clefts limited to the lips, or extending to the alveolus/premaxilla or the secondary palate. METHODS: This was a retrospective study based on the examination of fetuses diagnosed with an isolated CL +/- P. Cases included were either discovered at systematic screening or referred for further investigation. Clefts were characterized by their precise anatomical location and extent. The defect could include a cleft lip (CL), a cleft alveolus (CA), or a cleft of the secondary palate (CSP). RESULTS: We analyzed 96 cases of CL +/- P. The mean gestational age at examination was 28.2 +/- 4.1 weeks. The sonographic appearance of CL, CA, and CSP was depicted. Strict concordance of the sonographic report with the anatomical defect was present in 84 cases (87.5%). In eight cases, the severity of the cleft was underestimated: three cases of CA, four of CA + CSP and one of CSP were missed. In four cases, the cleft was overestimated as CA was incorrectly suspected. CONCLUSIONS: Systematic screening with sonography to detect prenatally CL +/- P requires the imaging of at least the mid-sagittal and the anterior coronal 'nose-mouth' views. Once the presence of a facial cleft is suspected, the three reference orthogonal planes are imaged in order to characterize the anatomical defect, and for each plane, the serial scans are thoroughly examined. This protocol allows precise delineation of the defect. Inclusion of 3D and 4D ultrasound imaging in the examination protocol allows easier and more rapid screening and more precise evaluation of the different cleft constituents.

Cleft Lip↗

Surgical management of cleft lip in pedo-patients.

The Present article describes in short etiology of cleft lip and cleft palate. With this in-born defect, patient develops crucial problems with feeding, phonation, overall growth and development of affected and allied soft and hard tissue structures. This in turn results in deformity and asymmetry which is going to affect functional requirements as well as aesthetic outlook. Hence it really becomes mandatory to correct this defect surgically as early as possible, at stipulated timings so as to avoid present and future anticipated problems.

Child↗

[Anatomical study and improvement of rotation-advancement method in repair of cleft lip].

A new method of cleft lip repair, based on anatomical research of levator septi nasi and a peach-shaped musculocutaneous flap has been designed. The special features of this method are: 1. A small peach-shaped musculocutaneous flap is used as Millard's "c" flap, 2. all skin incisions are made on the edge of the cleft, 3. rotation-advancement repair is applied in the muscle layer. 32 patients have been followed for 6 years after the operation.

Cleft Lip↗

Histological comparison of the alar nasal cartilages in unilateral cleft lip.

UNLABELLED: Patients with unilateral cleft lip display characteristic nasal changes that are independent of the degree of deformity. Defenders of the intrinsic theory consider these deformities to be due to embryogenic alterations of the alar nasal cartilages. Those that propose the extrinsic theory defend the thesis that the deformity is due to disorganization of the perioral muscles deformed by the cleft. The purpose of this study is to contribute histological evidence to help clarify the issue. PATIENTS AND METHODS: Specimens of the lateral portion of both the healthy and the cleft side of the alar cartilages were obtained from 18 patients. These uniformly cut specimens were stained by hematoxylin and eosin. Samples from 2 patients were excluded due to imperfections. The same pathologist examined all the slides. He was unaware of the origins of the specimens; he counted the number of chondrocytes and quantified the cartilage matrixes. RESULTS: All data was analyzed statistically, and no significant statistical differences were apparent, either in the number of chondrocytes or the cartilage matrix between the healthy side and the cleft side. DISCUSSION: These results apparently support the group that defend the extrinsic theory; nevertheless, the doubt about the composition of the cartilage matrix remains, not only concerning the glycosaminoglycans that compose them, but also regarding elastin and collagen and its linkages that can cause different degrees of collagen consistency.

Cartilage↗

Early repair for infants with cleft lip and nose.

OBJECTIVE: To study the results 10 years after early surgical cleft lip and nose repair. PATIENTS AND METHODS: We present the outcome of 123 early cleft lip repairs whose condition was managed in a multidisciplinary team according to a strict protocol. We give the observation results of operations of a single surgeon's neonatal surgery over a 10-year period in term of aesthetic assessments and dental arch relationships. One hundred and twenty-three patients were operated on during the first 4 weeks of life; a subgroup of 40 child had been operated on at a week or less of age. RESULTS: The results show good aesthetic assessments and dental relationships, with facial growth appropriate for the age. CONCLUSIONS: We are currently encouraging early cleft lip and nose repair in the full-term baby as the good method of management of newborns with cleft.

Cleft Lip↗

Comparison of velopharyngeal growth patterns between cleft lip and/or palate patients requiring or not requiring pharyngeal flap surgery.

This investigation compares the patterns of velopharyngeal growth in cleft lip and/or palate patients. Those who had velopharyngeal competence and acceptable speech are compared with those who presented with velopharyngeal incompetence requiring pharyngeal flap surgery or prosthesis later. Lateral cephalograms of 30 cleft palate only (CPO), 35 unilateral cleft lip and palate (UCLP), and 20 bilateral cleft lip and palate (BCLP) children of the Lancaster Cleft Palate Clinic were studied. These records were taken at 6 month intervals during the first 2 postnatal years and annually thereafter up to 6 years of age. Soft tissue landmark points in the velopharyngeal region were digitized. Length and thickness of the soft palate and height and depth of the nasopharynx were measured. Evaluation of the growth curves of these four cephalometric variables indicated only two significant differences between children who later required pharyngeal flap surgery and those who did not. These differences were found in the growth in length of the soft palate of the CPO group and in the growth in depth of the nasopharynx of the BCLP group. Based on the present cephalometric data, it is impossible to predict at an early age those cleft lip and/or palate patients who will later require pharyngeal flaps.

Cephalometry↗

Unilateral cleft lip repair: an anatomical subunit approximation technique.

BACKGROUND: A technique of unilateral cleft lip repair is described. The repair draws from a variety of previously described repairs and adheres to a concept of anatomical subunits of the lip. Cases from within the spectrum of the deformity have been chosen from a series of 144 consecutive cases to demonstrate the applicability of the technique in all forms of unilateral cleft lip. METHODS: Incisions cross the lip perpendicular to the cutaneous roll at the cleft side peak of Cupid's bow of the medial lip and at the base of the philtral column of the lateral lip. Above this level, incisions ascend the lip to allow for approximation along a line symmetrical with the non-cleft-side philtral column. Incisions then ascend superolaterally bordering the lip columellar crease to the point of closure in the nostril sill. A Rose-Thompson lengthening effect occurs just above the level of the cutaneous roll. If necessary, a small triangle positioned just above the cutaneous roll is often used. Any central vermilion deficiency is augmented by a laterally based triangular vermilion flap from the lateral lip element. RESULTS: Since January of 2000, this technique has been used in 144 consecutive unilateral cleft lip repairs. The inferior triangle is small (average, 1.24 mm; range, 0 to 2 mm). The technique can be applied to all degrees of unilateral cleft lip. CONCLUSIONS: A technique of unilateral cleft lip repair is described. The repair allows for a repair line that ascends the lip at the seams of anatomical subunits.

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 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↗

New technique for correction of the microform cleft lip using vertical interdigitation of the orbicularis oris muscle through the intraoral incision.

A microform cleft lip has three major components: (1) a minor defect of the upper vermilion border with loss of the mucocutaneous ridge; (2) a narrow ridge of tissue, resembling an exaggerated philtral column extending to the nostril sill; and (3) a deformity of the nostril. To attain the muscle continuity without an external scar on the upper lip, the author introduced a new method for the correction of a microform cleft lip deformity using vertical interdigitation of the orbicularis oris muscle through the intraoral incision to create the philtrum. Through the intraoral incision, a full-thickness incision is made down to the mucosa and the posterior portion of the muscle. Then, the remaining portion of the muscle is dissected. The medial and lateral muscle flaps are also detached from the oral mucosa and completely exposed and split into two leaves. The upper leaf of the lateral muscle flap is sutured to the dermis on the philtral dimple and base of the upper leaf of the medial muscle flap. Two leaves of each muscle flap are sutured together to create a vertical interdigitation to increase the thickness of the philtral column and to provide continuity of the muscle. A total of 12 patients with microform cleft lip were treated between August of 2001 and October of 2002. Seven of the patients were male and five were female, with an age range of 1 to 43 years. The follow-up period ranged from 6 months to 15 months, with an average follow-up of 9 months. The results of vertical interdigitation of the muscle were examined. All patients were satisfied with their results. The orbicularis oris muscle provided continuity and preserved good function. In all cases, the operation scar was not visible on the depressed philtral groove on the cleft side. Correction of cleft lip nasal deformity was performed in four patients and alar base advancement was performed in two patients. The advantages of the proposed procedure include the creation of an anatomically natural philtrum without an external visible scar through the intraoral incision, preservation of the continuity and function of the muscle, and sufficient augmentation of the philtral column by the vertical interdigitation of the muscle.

Adolescent↗

Midfacial morphology in adult unoperated complete unilateral cleft lip and palate patients.

OBJECTIVE: To examine lateral cephalometric radiographs of adult unoperated cleft lip and palate patients for the purpose of clarifying whether maxillary deficiencies observed in treated cleft patients result from intrinsic defects or surgical intervention early in life. MATERIALS AND METHODS: This retrospective study examined lateral cephalograms of 30 adult patients with nonsyndromic complete unilateral cleft lip and palate (CUCLP). The lateral cephalograms were traced and evaluated for size and position of the cranial base, maxilla, maxillary dentition, mandible, and mandibular dentition as well as for vertical relationships. Comparisons with 30 adult noncleft individuals were made. RESULTS: In unoperated adult cleft lip and palate patients, the cranial base angle was increased with the anterior cranial base reduced in length. The maxilla was found to be normal in size and somewhat prognathic in position. Both the maxillary and mandibular incisors were relatively upright. The mandible was smaller in size and posteriorly positioned. CONCLUSIONS: The potential for normal growth of the maxilla exists in patients with CUCLP. It is likely that disturbances of maxillary growth in surgically operated cleft patients are related primarily to the surgical intervention.

Adolescent↗

An unusual case of median cleft lip with orbital hypotelorism--a missing link in the classification.

De Myer (1963) classified median-facial anomalies into two groups: 1. Median cleft lip with orbital hypotelorism 2. Median cleft lip with orbital hypertelorism. The first group is characterised by median-cleft lip, absence of premaxilla, nasal skeleton, and crista galli. In addition, there is a holoprosencephaly which may involve the whole forebrain or a smaller part of the brain. In this group, the children die within the first year or during childhood and always suffer from mental retardation. In this report, we present a 12-year-old child with an unusual form of median cleft lip with hypotelorism without brain deformity.

Child↗

[Association of the genetic variation of (MSX1-7) and non syndromic cleft lip palate in Chilean subjects].

BACKGROUND: Homeotic genes have regulatory functions during development. It has been postulated that the human Msx-1 homeotic gene can be involved in the etiology of non syndromic cleft lip palate, since its homologous Msx-1 is involved in cleft palate of mice. AIM: To perform an association analysis between the genetic variation of Msx-1 and non syndromic cleft lip palate in Chilean subjects. PATIENTS AND METHODS: Seventy patients with non syndromic cleft lip palate, 136 healthy relatives of these patients and 69 non related normal individuals were studied. CA microsatellite in Msx-gene, that was amplified with PCR, was studied. RESULTS: No differences in the genetic frequencies of Msx-1 alleles, were observed in the three groups studied. Allelic heterogeneity for allele 2 seems to be related to cases of non syndromic cleft lip palate from multiplex families and heterogeneity for allele 3 is related with simplex families cases. CONCLUSIONS: These results seem to support the hypothesis of genetic heterogeneity in the etiology of non syndromic cleft lip palate.

Alleles↗

Results of randomized controlled trial of soft palate first versus hard palate first repair in unilateral complete cleft lip and palate.

OBJECTIVE: To compare the outcomes for primary repair of unilateral cleft lip and palate, operating on the soft palate first versus the hard palate first. DESIGN: Randomized controlled trial. SETTING: The Regional Cleft Service of West Nepal. PATIENTS: Forty-seven consecutive patients with nonsyndromic unilateral cleft lip and palate, of whom 37 were assessed 4 to 6 years after completing primary surgical repair. INTERVENTIONS: Primary repair of unilateral cleft lip and palate by two differing sequences: (1) soft palate repair, with hard palate and lip repair 3 months later; and (2) lip and hard palate repair, followed by the soft palate repair 3 months later. MAIN OUTCOME MEASURES: Analysis of dental study models, weight gain, and speech recordings. RESULTS: Four to 7 years after completing the cleft closure, there was no significant difference in facial growth between the two types of repair sequencing. Completing posterior repair first had no effect on anterior alveolar gap width. It narrowed the hard palate gap by reducing the intercanine distance. Anterior repair dramatically closed the anterior alveolar gap, and narrowed the intercanine distance. Comparing anterior alveolar gap width with age at first presentation demonstrated that there was no spontaneous narrowing of the cleft in older children. Completing posterior closure first had a weight gain advantage over anterior closure first. Improved oropharyngeal closure, and thus swallowing, is the likely explanation. CONCLUSION: Changing the sequencing of cleft closure has no demonstrable difference in facial growth at 4 to 7 years after completion of the primary surgery.

Asian People↗

Secondary repair of unilateral cleft lip nose deformity with bilateral reverse-U access incision.

Unilateral cleft lip is often accompanied by bilateral deformity. We use bilateral reverse-U incisions for the primary repair of unilateral cleft lip nose. This principle can also be applied to achieve symmetry of the nose in the secondary correction of the unilateral cleft lip nose. By the use of bilateral reverse-U access incisions and an inverted trapezoid suture between alar cartilages and nasal dome, it has become possible to achieve nasal symmetry and clearly defined alar groove. The surgical procedure is presented along with some representative cases.

Adolescent↗

Differences in frequency of cleft lip among the A strains of mice.

The frequency of spontaneous cleft lip on Day 14 of gestation was examined in the A/J, A/HeJ, A/WySn, A.BY/Sn, A.SW/Sn, B10.A/SgSn, and B10.A(2R)/SgSn inbred strains of mice. A/WySn was found to have a higher frequency of cleft lip (20-30%) than A/J or A/HeJ (10%). Differences among strains in laterality of clefting and in rates of differential mortality of affected embryos were also found. Results of crosses between A/WySn and A/HeJ suggested that the difference in rate of clefting may be due to a genetic maternal effect. The results for A.BY/Sn, A.SW/Sn, B10.A/SgSn and B10.A(2R)/SgSn indicated that no association of cleft lip liability and the H-2 complex was detectable.

Animals↗

Bone grafting the piriform aperture deformity in isolated cleft lip patients: indication, technique, and results.

PURPOSE: This study was undertaken to determine the effect of a bone graft in the piriform aperture on the nasal deformity and orthodontic treatment of the cleft side teeth in isolated cleft lip patients. PATIENTS AND METHODS: All primary cleft lip repair was done 3 months after birth. Nine patients, four female and five male, with a mean age of 12.5 years (range, 8.2 to 24.8 years) and with a repaired cleft lip, were bone grafted between 1992 and 1996. The mean postoperative period was 2 years (range, 1 to 4 years). An iliac crest bone graft was placed in the piriform aperture deformity on the side of the cleft lip. The improvement in the nasal symmetry and angulation of the cleft side teeth were evaluated. The eight growing cleft lip patients (mean age, 11 years; range, 9 to 13 years) were compared with a control group of eight healthy growing children (mean age, 11 years; range, 9 to 13 years). The improvement of nasal symmetry was measured by the formula of the lobule portion of the columella index preoperatively and postoperatively. RESULTS: The mean lobule portion of the columella index preoperatively was 41.8% (SD, 4.4%; SE of Mean, 1.5%) and postoperatively was 44.2% (SD, 4.9%; SE of Mean, 1.6%) (P > .006, t-test for paired samples). The angulation of the cleft side teeth was improved by orthodontic treatment. CONCLUSION: Bone grafting the piriform aperture deformity results in a stable result and improves nasal symmetry and the angulation of the cleft side teeth.

Adolescent↗

Cleft lip and palate associated with malformation of the central nervous system: a prospective neurosonographic study.

A prospective neurosonographic study was undertaken on 190 patients with non-syndrome cleft lip and/or palate. Among them, six cases (3.2%) demonstrated malformations of the central nervous system. Included were four cases of midline cleft lip and palate showing holoprosencephaly; one case of bilateral cleft lip and midline cleft palate showing dysplasia of the septum pallicidum and one case of bilateral cleft lip and palate showing dysgenesis of corpus callosum. The patients with midline clefts were not obligatory associated with malformation of the brain, but their incidence was higher (four of nine case or 44.4%). The neurosonogram provides a portable, low cost, non-invasive and efficient technique in the diagnosis of gross anomaly of the neonatal brain, and can thus serve as a screening test in those patients with clefts.

Abnormalities, Multiple↗