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[A study of the feasibility and efficacy of fetal surgical closure of spontaneous cleft lip in CL/fraser mice].

Cleft lip with or without cleft palate is one of the most common human craniofacial congenital malformations. Failure to form a continuous tissue in the upper lip during the early embryonic period results in cleft lip. There has been an increasing interest in the possibility of in-utero correction of life threatening fetal abnormalities. In the present study, the feasibility and efficacy of surgical suturing of the fetal mouse lip was examined by using CL/Fraser mouse fetuses (spontaneous of cleft lip) at embryonic 15, 16 or 17 days. The obtained results were as follows. #1. Abortion rate of pregnant dam applied inhalation of Ethrane was less than that of intraperitoneal injection of Nembutal. #2. Successful delivery rate of fetal mouse surgically treated at the embryonic 16 day or 17 day is higher than that on the embryonic 15 day. #3. Fusion rate of mouse fetal lip surgically treated on the embryonic 16 day was higher after 60 hours in dam's uteri than that after 36 hours. These results suggest that inhalation of Ethrane is more recommendable, and that the optimal timing of fetal surgery of spontaneous cleft lip in CL/Fr. mouse fetus is an embryonic 16 day, and that the average time of 60 hours after surgical operation is enough for sutured lip to fuse in dam's uteri.

Anesthesia↗

The effects of zero population growth on the incidence of cleft lip and palate in Western Australia.

A total of 613 cases of cleft lip, cleft palate, and combinations of both (CLP(P)) has been ascertained in the period from 1958 to 1975 in Western Australia. Near-total ascertainment of cases during this period was obtained from multiple sources. There has been a significant decline in the total incidence and the cleft lip and palate (CLP) incidence in males. The hypothesis is presented that these changes are linked to the changing fertility pattern throughout Australia (as technological improvements are made in the simplicity and efficacy of birth control), and abortion (as social attitudes change); and as the birthrate drops towards zero population growth (ZPG). The paper presents two critical observations: (i) the link between CLP(P) incidence, and demographic changes; (ii) in differing behaviour of cleft lip (CL) and CLP, which were previously regarded as linked conditions.

Australia↗

[Case report of long-term treatment of a cleft lip and palate patient].

Cleft lip and palate patients have narrow maxillary arches and mesio-distal disharmony between the jaws caused by cicatricial contracture from cleft lip and palate surgeries. In such cases, early orthodontic treatment is required including improvement of the mesio-distal disharmony of the jaws. Following orthodontic treatment, prosthodontic treatment is required to improve the masticatory function and esthetic appearance in the cleft area. Thus, improving the occlusion of cleft lip and palate patients takes a long time. This case report concerns such a longterm handling of over 17 years from the initial treatment at 5 years 3 months till the final prosthodontic treatment for an under-developed maxilla with partial closure of the fistula to which ilium was later grafted following orthodontic treatment. Considerations are discussed about timing of the initiation of orthodontic treatment, closure of the oronasal fistula and the final prosthodontic restoration.

Child↗

Unravelling the complex genetics of cleft lip in the mouse model.

Nonsyndromic cleft lip in "A" strain mice and humans is genetically complex and is distinct from isolated cleft palate. Cleft lip embryos recovered in 2.4% of 1485 first backcross (BC1) segregants from a cross of A/WySnJ (24% cleft lip) and C57BL/6J (no cleft lip) in A/WySnJ mothers, and in testcrosses of 10 recombinant inbred (RI) strains (AXB/Pgn or BXA/Pgn), were used for gene mapping and for inference of genetic architecture. The A/WySnJ maternal genotype increased cleft lip risk in reciprocal crosses; the relevant genetic difference between AXB-6/Pgn (8%) and A/WySnJ (24%) is entirely maternal. A combination of new mapping panels (325 meioses), new markers, and a recombinant cleft lip embryo redefined the location of a recessive factor essential to cleft lip risk, clf1, and candidate genes Itgb3 and Crhr, to between D11Mit146/360 and D11Mit166/147. A screen of 54 YACs for 46 genes and SSLP loci located Wnt15, Wnt3, Crhr, Mtapt, Itgb3, Dlx3, and Dlx7 within the clf1 candidate region. The clf2 locus was newly mapped to Chromosome (Chr) 13 by a genome screen of BC1 segregants, and further defined to a 4-cM region between D13Mit13/54 and D13Mit231 by strain distribution patterns of cleft lip liability and markers in testcrossed RI strains. Specific combinations of marker genotypes associated with cleft lip risk indicated that high risk in A/WySnJ mice is caused by epistatic interaction between clf1 and clf2 in the context of a genetic maternal effect. Human homologs of clf1 and clf2 are expected to be on 17q and 5q/9q.

Animals↗

Description of a clinical technique for tooth extraction in the cleft lip and palate area.

Cleft lip and palate are relatively common congenital malformations, which may require specialist paedodontic treatment. In this article, the case of a 9-year-old boy with bilateral complete cleft lip and palate is presented. He attended the Hospital for Rehabilitation of Craniofacial Anomalies (HRAC) for routine examination, during which the presence of pre-canine supernumeraries bilaterally in the cleft area was seen. The extraction of these dental elements was justified by extensive carious lesions and because they represented a potential problem during secondary palatoplasty. The precautions needed in tooth extraction in patients with cleft lip and palate are described, together with illustrations of the clinical procedure.

Child↗

Ambulatory surgery for cleft lip repair.

Standard of care for cleft lip repair has included preadmission testing, surgical correction, and postoperative hospital care. Driven not by managed care economics but to speed the safe home care of infants by parents, the authors have gained experience in ambulatory cleft lip repair. In this retrospective study the authors evaluated the outcome of patients who underwent ambulatory cleft lip repair compared with those patients who were hospitalized after surgery. From 1989 to 1998, 24 cleft lip repairs in 24 patients performed by the senior author were evaluated. Two groups were treated. Group 1 (N = 11) consisted of ambulatory unilateral cleft lip repairs and group 2 (N = 13) consisted of inpatient unilateral cleft lip repairs. Important surgical factors considered were technique of cleft lip repair, performance of ancillary procedures, type of local anesthetic administered, and intravenous steroid administration. Time to first postoperative feeding and complications, including bleeding, spontaneous or traumatic wound dehiscence, and infection, were considered important outcome parameters. There were no differences in surgical technique or use of antibiotics and postoperative analgesics between the two groups. None of the patients in group 1 underwent ancillary procedures. Four patients underwent soft palate repair and 3 patients underwent insertion of myringotomy tubes among group 2 patients. The use of a 1:1 mixture of 1% lidocaine and 0.5% bupivacaine with epinephrine vs. 1% lidocaine with epinephrine as a local anesthetic and intravenous steroid administration was greater in group 1 (92%) than in group 2 (33%) patients. The average time to the first postoperative feeding was more than 1 hour sooner in the ambulatory group (p < 0.05) compared with the hospitalized group (excluding the 4 patients who underwent soft palate repair). There were no complications among patients with ambulatory cleft lip repair, and there were two cases of minor wound separation in patients who received postoperative hospital care. Although many variables factor into the outcome after cleft lip repair, these data support the safety and continued practice of ambulatory cleft lip repair.

Acetaminophen↗

The prevalence of caries in children with a cleft lip and/or palate in Southern Vietnam.

OBJECTIVE: The aim of this study was to investigate the prevalence of caries in South Vietnamese children with a known oral cleft. SETTING: The children were screened at local dental hospitals in five different cities in southern Vietnam. PARTICIPANTS: One hundred fifty-four children aged 4 to 6, 11 to 13, and 14 to 16 years old with a cleft lip, cleft palate, or both were included in the study. OUTCOME MEASUREMENTS: For every age group, clefts were divided in four types. The dmft/DMFT was specified according to criteria of the World Health Organization (1997). RESULTS: The mean number of teeth affected by caries was 9.95 for 4- to 6-year-old children, 2.97 for 11- to 13-year-old children, and 4.93 for 14- to 16-year-old children, respectively. Four- to 6-year-old children with a unilateral or bilateral cleft lip and palate had significantly more caries and a higher dmft (decayed, missing, and filled teeth index) than children of the same age with only a cleft lip or a cleft lip and alveolus. CONCLUSIONS: Vietnamese children with an oral cleft have high numbers of teeth affected by dental caries, and special attention will be necessary for their oral health.

Adolescent↗

[Plastic surgery in unilateral cleft lip].

The result of cleft lip operations is judged after the external image, the complet function of circular muscle system and the unfoldable vestibulum oris. The MILLARD-plastic for wide cleft lips and the REICHERT-plastic with the own modification for narrow cleft lips are for the time the optimal methods of operation.

Cleft Lip↗

The anatomy of Cupid's bow in normal and cleft lip.

This is a gross and microscopic examination of the cutaneous-vermilion-mucosal region (Cupid's bow) in infants with normal lip as compared to cleft lip. Morphologic observations of lips in situ were made with magnification. Specimens for histology were from the normal upper lip of infants (n = 3) and adults (n = 4), and from redundant tissue made available during primary repair of cleft lip (n = 13) and cleft lip revision (n = 4). Tissues were analyzed with hematoxylin and eosin, Gomori trichrome, and Fontana-Masson stains. Immunoperoxidase stains were used to emphasize vascular endothelium and epithelial cytokeratins. Our observations support the hypothesis that the anterior projection of the pars marginalis of the musculus orbicularis oris gives rise to the normal cutaneous-vermilion junction ("white skin roll" of Gillies-Millard). The band of vellus hairs, found atop the vermilion-cutaneous junction, also highlights the rim of Cupid's bow. There was no abrupt alteration in vasculature, melanin, or cytokeratin content in the white roll. Proceeding caudally, the vermilion-mucosal region was characterized by progressively increasing epidermal thickness and size of rete ridges, decreasing melanin, more superficial capillaries, and an abrupt transition from keratinized to nonkeratinized squamous epithelium ("red line" of Noordhoff). In the cleft lip specimens, where white roll was absent, there was hypoplasia and disorientation of the underlying pars marginalis component of m. orbicularis oris. Vellus hair follicles were seen in the zone of presumptive white roll in clefts, just as in normal lip. Frequently, these pilar units contained disproportionately large sebaceous glands. In some cleft lip specimens, ectopic sebaceous glands were noted in the oral mucosa. We also observed a deficiency of vermilion width on the medial side of unilateral cleft lip, whereas the width of the vermilion at the crest of the bow was normal or slightly increased in the lateral cleft segment. A narrow vermilion band and exposed hypoplastic mucosa were characteristic of the prolabium in the bilateral complete cleft deformity. The findings of this study are pertinent to technical details for construction of the Cupid's bow and vermilion in unilateral and bilateral cleft lip.

Cleft Lip↗

A prospective evaluation of the prevalence of submucous cleft palate in patients with isolated cleft lip versus controls.

Although there is an established relationship between cleft lip and overt cleft palate, the relationship between isolated cleft lip and submucous cleft palate has not been investigated. To test the hypothesis that patients with isolated cleft lip have a greater association with submucous cleft palate, a double-armed prospective trial was designed. A study group of 25 consecutive children presenting with an isolated cleft lip, with or without extension through the alveolus but not involving the secondary palate, was compared with a control group of 25 children with no known facial clefts. Eligible patients were examined for the presence of physical criteria associated with classic submucous cleft palate, namely, (1) bifid uvula, (2) absence of the posterior nasal spine, and (3) zona pellucida. Nasoendoscopy was subsequently performed just after induction of general anesthesia, and the findings were correlated with digital palpation of the palatal muscles. Patients who did not satisfy all three physical criteria and in whom nasoendoscopy was distinctly abnormal relative to the control group were classified as having occult submucous cleft palate. Classic submucous cleft palate was found in three study group patients (12 percent), all of whom had flattening or a midline depression of the posterior palate and musculus uvulae on nasoendoscopy and palpable diastasis of the palatal muscles under general anesthesia. An additional six study group patients (24 percent) had similar nasoendoscopic criteria and palpable diastasis of the palatal muscles; they were classified as having occult submucous cleft palate. No submucous cleft palate was identified in the control group. Seventeen patients in the study group had an alveolar cleft with a 53 percent (9 of 17) prevalence of submucous cleft palate. In the present study, classic submucous cleft palate in association with isolated cleft lip was 150 to 600 times the reported prevalence in the general population. All children with an isolated cleft lip should undergo peroral examination and speech/resonance assessment no later than the age of 3 years. Any child with an isolated cleft lip with velopharyngeal inadequacy or before an adenoidectomy should be assessed by flexible nasal endoscopy to avoid missing an occult submucous cleft palate.

Adolescent↗

Extension of the cleft lip phenotype: the subepithelial cleft.

We present a previously undescribed occult malformation of the upper lip manifest by discontinuity of the orbicularis oris muscle. The relationship of this defect to our current concept of abnormal maxillary process development is discussed along with a proposal to extend the cleft lip phenotype.

Cleft Lip↗

Quantitative evaluation of the shape and the elasticity of repaired cleft lip.

Tight lip in repaired cleft lip patients with or without cleft palate (CL[P]) has been discussed as one of the causal factors of maxillary growth retardation and malocclusion. The purpose of this study was to evaluate the shape and the elasticity of repaired cleft lip quantitatively. Forty-one Japanese CL(P) patients were examined. Fifty-four noncleft patients under orthodontic treatment were used as a control group. An apparatus to stretch the anguli oris bilaterally was devised and the extensibility of the lip was measured to evaluate lip elasticity. Standardized photos of the lips were taken to examine lip shape. CL(P) patients showed poor elasticity of the lip. This trend was more apparent in bilateral CL(P) patients than in unilateral CL(P) patients. The upper lip of CL(P) patients was significantly shorter in vertical height. These results clearly demonstrated the tightness and shortness of the repaired lip in CL(P) patients.

Adolescent↗

Long-term effects of lip repair on dentofacial morphology in patients with unilateral cleft lip and palate.

OBJECTIVE: Lateral cephalograms from the growth archive of the Sri Lankan Cleft Lip and Palate Project were analyzed in a cohort design to study the long-term effects of lip repair on dentofacial morphology in patients with unilateral cleft lip and palate. METHODS: A total of 71 patients were recruited, including 23 adult patients with nonsyndromic unilateral cleft lip and palate without surgical repair and 48 adult patients with nonsyndromic unilateral cleft lip and palate who had lip repair, but without management of alveolus or anterior vomer. The design utilized exact matching on ethnicity and statistical control for gender and age. RESULTS AND CONCLUSIONS: The data support the hypothesis that lip repair primarily produces a bone-bending effect on the anterior maxillary alveolus (alveolar molding), accompanied by controlled uprighting of maxillary incisors, and secondarily produces a bone-remodeling effect (bone resorption) in the base of the anterior maxillary alveolus. When analyzed by the age at lip repair and the surgeon who performed lip repair, early lip repair produced a greater bone-remodeling effect than did late lip repair, and variation in the surgeon who performed lip repair had an insignificant impact on dentofacial morphology after adjusting for covariates.

Adult↗

Lip height and lip width after extended Mohler unilateral cleft lip repair.

The purpose of this study was to evaluate the symmetry in lip height and lip width after extended Mohler unilateral cleft lip repair, with long-term follow-up monitoring. In the Mohler repair procedure, Millard's C-flap is used to fill the entire defect created by the downward rotation of the medial lip element. Because a lateral advancement flap is not transposed into this defect, Mohler repair is often expected to produce a short lip. In a retrospective study of 120 patients, anthropometric measurements were made on black-and-white photographs. Of those patients, 49 met the study criterion of having a set of photographs taken 13 months or less postoperatively and another set taken at least 2 years postoperatively. The distance from the Cupid's bow peak to a line tangent to the base of the columella (lip height) and the distance from the Cupid's bow peak to the ipsilateral commissure (lip width) were measured with a Vernier caliper. The medial intercanthal distance was also measured, for standardization of all measurements. All values were normalized to the mean intercanthal distance at age 6, as reported by Farkas. Matched-pair test analyses were used to assess the statistical significance of differences in cleft-side versus non-cleft-side measurements for each group, as well as changes with time. No statistically significant difference in cleft side versus non-cleft-side lip height for the two groups or with time was observed (< or =13 months, p = 0.28; >2 years, p = 0.08; change with time, p = 0.69). Statistically significant differences in lip width between the cleft side and the non-cleft side were observed for both time groups. The average difference in lip width at 1 to 13 months was 8.6 percent (p < 0.001). The average difference in lip width at 2 years or more postoperatively was 5.8 percent (p < 0.001). In comparisons of early versus late measurements, it was noted that lip width significantly increased with time (mean, 0.91 mm; p = 0.035). The findings suggest that extended Mohler repair does not produce a short lip. Interestingly, lip width was observed to be significantly smaller on the cleft side in the immediate postoperative period. However, this deficiency was observed to decrease significantly during long-term follow-up monitoring.

Anthropometry↗