Changes of craniofacial growth and development in males with complete unilateral cleft lip and palate between the age of 5 to 20 years

The study is based on an anthropometric assessment of X-ray films obtained in two series of males with complete unilateral cleft lip and palate. The first series was examined at the age of 5, 10 and 15 years, the second series at 15 and 20 years. The films were assessed with Jarabak's analysis. The aim of our study was to compare the amount of growth and character of developmental changes in the prepubertal, pubertal and postpubertal period of life. The highest growth rate of skeletal structures was present in the prepubertal period, it was somewhat slighter in the pubertal period and it still continued in the postpubertal period. The high growth rate in the prepubertal period was probably related to the eruption of permanent teeth. In spite of a marked deterioration of sagittal jaw relations during the prepubertal period an improvement of an overjet was attained. However during the puberty and the postpubertal period a further improvement was not recorded. The results are in agreement with facial type of growth characterized by a slight pubertal spurt.     

Alveolar bone grafting in unilateral complete cleft lip and palate patients

Autogenous bone graft of an alveolar cleft area has the following advantages: (1) assistance in the closure of buccoalveolar oronasal fistula; (2) provision of bony support for unerupted teeth and teeth adjacent to the cleft; (3) formation of a continuous alveolar ridge to facilitate orthodontic correction of malocclusion; (4) supporting the nostril floor and alar base to improve nasal aesthetics. It has been well accepted in most craniofacial centers as routine procedure in cleft lip and palate rehabilitation. A new surgical technique for alveolar bone grafting has been introduced to the Chang Gung Craniofacial Center since July 1991. It provided a good exposure of the alveolar cleft, primary closure of the fistula and adequate volume of bone graft. A review of 27 consecutive alveolar bone grafting procedures performed in unilateral cleft lip and palate patients from July 1991 to June 1992 was presented. Patients have been followed up for at least 6 months. The alveolar bone graft was evaluated clinically and radiologically at one week, six months and one year after the surgery. The preliminary results indicated that the new surgical technique produced less chance of recurrent fistula, good postoperative gingival height, and improvement of nasal aesthetics. Based on the results of this new study we strongly advocate the use of this new surgical technique.     

Fetal unilateral cleft lip and palate: detection of enzymic anomalies in the amniotic fluid

BACKGROUND: Tracheal intubation frequently results in an increase in respiratory system resistance that can be reversed by inhaled bronchodilators. The authors hypothesized that insertion of a laryngeal mask airway would be less likely to result in reversible bronchoconstriction than would insertion of an endotracheal tube. METHODS: Fifty-two (45 men, 7 women) patients were randomized to receive a 7.5-mm (women) or 8-mm (men) endotracheal tube or a No. 4 (women) or No. 5 (men) laryngeal mask airway. Anesthesia was induced with 2 microg/kg fentanyl and 5 mg/kg thiopental, and airway placement was facilitated with 1 mg/kg succinylcholine. When a seal to more than 20 cm water was verified, respiratory system resistance was measured immediately after airway placement. Inhalation anesthesia was begun with isoflurane to achieve an end-tidal concentration of 1% for 10 min. Respiratory system resistance was measured again during identical conditions. RESULTS: Among patients receiving laryngeal mask airways, the initial respiratory system resistance was significantly less than among patients with endotracheal tubes (9.2+/-3.3 cm water x 1(-1) x s(-1) [mean +/- SD] compared with 13.4+/-9.6 cm water x 1(-1) x s(-1); P < 0.05). After 10 min of isoflurane, the resistance decreased to 8.6+/-3.6 cm water x 1(-1) x s(-1) in the endotracheal tube group but remained unchanged at 9.1+/-3.3 cm water x 1(-1) x s(-1) in the laryngeal mask airway group. The decrease in respiratory system resistance in the endotracheal tube group of 4.7+/-7 cm water x 1(-1) x s(-1) was highly significant compared with the lack of change in the laryngeal mask airway group (P < 0.01). CONCLUSIONS: Resistance decreased rapidly only in patients with endotracheal tubes after they received isoflurane, a potent bronchodilator, suggesting that reversible bronchoconstriction was present in patients with endotracheal tubes but not in those with laryngeal mask airways. A laryngeal mask airway is a better choice of airway to minimize airway reaction.     

Analyses of craniofacial and dental morphology in monozygotic twins discordant for cleft lip and unilateral cleft lip and palate

AIMS: To compare the efficiency of flow cytometry and computed image cytometry; and to see if a reliable set of guidelines regarding interpretation of histograms could be drawn up. METHODS: The two methods were applied to a series of 111 formalin fixed renal cell carcinomas. Data generated by both methods were compared. RESULTS: The methods agreed in 85 cases. Hypodiploidy was detected by computed image cytometry in seven cases in which flow cytometry had shown only an aneuploid peak. Aneuploidy in seven in which the corresponding flow cytometry histogram was diploid. There was an overlap in the second peak proportions on flow cytometry histograms between those classed as diploid or tetraploid by computed image cytometry. In six cases the flow cytometry histograms had unacceptably high coefficients of variation and in all of these cases computed image cytometry demonstrated aneuploidy. CONCLUSIONS: Computed image cytometry is particularly useful for clarifying difficult areas in flow histograms--specifically, high coefficients of variation, high G2M phase, as well as possible near diploid aneuploidy and hypodiploidy.     

Nomogram for assessment of restoration of a positive overjet in unilateral cleft lip and palate

In 92 patients with unilateral cleft lip and palate investigated on a long-term basis correlation-regression analysis of the development of overjet according to the development of interalveolar relations assessed in a defined method from X-ray films at the ages of 5, 10, 15 years and in adult age was implemented. The limits comprising 95% of the assembled data were also defined. Based on this analysis a formerly elaborated nomogram for age 10-15 years was extended for the whole postnatal period. Nomogram makes it possible to estimate from a single X-ray cephalometric parameter the risk of failure of the restoration of a positive overjet according to the age of the patient. The system defines critical boundaries which must be achieved for safe restoration of a positive overjet and makes it possible to check the course of treatment. The simple work with the nomogram and readily obtainable baseline data (age and X-ray cephalometric parameter on the interalveolar relations) make its application in clinical practice possible. It can serve as part of the documentation of the patient's therapy.     

Effects of primary bone grafting on facial development in patients with unilateral complete cleft lip and palate

The study is based on an anthropometric assessment of X-ray films obtained in 22 adult males with complete unilateral cleft lip and palate treated during childhood with primary bone grafts and in 32 males with the same type of cleft without bone grafting. In the series with bone grafts was recorded a more marked reduction of maxillary depth associated with a larger retrusion than in the series without bone grafts. This deviation was therapeutically compensated by a larger displacement of the mandible backwards which contributes to the increase of mandibular posterior rotation. This provided the possibility to attain an edge to edge bite. Our results confirmed the unfavourable effects of primary bone grafting on maxillary growth and development.     

Sagittal craniofacial morphology in repaired unilateral and bilateral complete cleft lip and palate cases

Using guidelines, students independently complete a written preparation for class assignment to develop a knowledge base needed for active participation in one or more class activities. The authors present a two-part method that is key to developing critical-thinking skills, using case studies.     

Repair of unilateral cleft lip: a comparison of five techniques

Repair of unilateral cleft lip is a challenging procedure with no single technique satisfactory for all types of unilateral cleft deformity. This study compares retrospectively five techniques of unilateral cleft lip repair in 72 children (45 boys, 27 girls). Twenty-two children had a Millard rotation-advancement repair, 5 children had a Davies Z-plasty, 22 had a modified Z-plasty, 10 had a Tennison-Randall triangular flap repair, and 13 had a Nakajima-Yoshimura straight-line repair. Assessment was performed clinically by a plastic surgeon not involved in the original surgery. Repairs were assessed objectively by measurement of the vertical length of both the repaired and normal sides of the lip with calipers. Subjective criteria used to evaluate the repair were the symmetry of Cupid's bow, the quality of scar, the alignment of white roll, the evenness of the vermilion, and the lip pout. The mean follow-up period was 5.4 years (range, 9 months-29 years). Sixty-five of the 72 repairs measured (90%) were within two standard deviations (SDs) of normal and thus were considered to be of "acceptable" length. Seven repairs were unacceptably short (>2 SDs) on measurement, six of which included a rotation-advancement repair for a complete cleft lip. Subjective results paralleled the objective results. The outcome following repair of unilateral cleft lip was similar for all five surgical methods assessed except for complete cleft lips repaired by the rotation-advancement technique, which tended to result in an unacceptably short lip as measured on the repaired side.     

Association between non-right-handedness and cleft lip with or without cleft palate in a Chinese population

The ori region of an Erwinia stewartii plasmid, pSW1200 (106 kb), has been cloned and sequenced. This region consists of a gene encoding a protein which has 91% similarity and 73% identity with the RepA protein of bacteriophage P1. The ori region also consists of eight copies of 19-bp iterons which are highly homologous to the iterons of P1. Similar to plasmid P1, pSW1200 replicon has a copy number of approximately 1. On the other hand, the copy number increases about ninefold if three of the iterons located downstream from repA gene are deleted. We also demonstrate that pGEM-5Z consisting of a copy of P1 iteron is incompatible with a pSW1200 derivative, pSW1201, suggesting that pSW1200 and P1 DNA are incompatible and both belong to the IncY group.     

Prevalence of malocclusion in the primary and early mixed dentition in a group of Egyptian children with complete unilateral cleft lip and palate

This study was carried out to investigate the prevalence of malocclusion in the primary and early mixed dentition for a group of Egyptian children with complete unilateral cleft lip and palate following surgical rep. air of the anomaly. The material consisted of 35 children (20 males and 15 females) with complete unilateral cleft lip and palate aged 3 to 7 years who were compared with 60 non-cleft Egyptian children (37 males and 23 female) of the same age.     

Speech outcome and maxillary growth in patients with unilateral complete cleft lip/palate operated on at 6 versus 12 months of age

A prospective study of speech outcome and maxillofacial growth was carried out in cleft palate patients. Seventy-six cleft palate patients were randomly selected for the study group; 41 patients were operated on at 12 months of age, and 35 patients were operated on at 6 months of age. All patients were followed until they were 4 years of age. All patients underwent a complete speech evaluation, videonasopharyngoscopy, videofluoroscopy, and maxillofacial assessment. The rate of velopharyngeal insufficiency did not differ between the two groups (17 to 19 percent; p > 0.05). However, phonologic development was significantly better (p < 0.05) in the patients operated on at 6 months of age. Furthermore, none of the patients operated on at 6 months of age showed compensatory articulation disorder. In contrast, 62 percent of the patients with postoperative velopharyngeal insufficiency operated on at 12 months of age showed compensatory articulation disorder (p < 0.05). Maxillofacial assessment showed that there were non-significant differences (p > 0.05) in maxillofacial growth in both groups of patients. All patients showed similar degrees of maxillary collapse (p > 0.05). The results of this study suggest that cleft palate repair performed at 6 months of age significantly enhances speech outcome and prevents compensatory articulation disorder.     

Abnormalities of the maxillary incisors in children with cleft lip and palate

Dental anomalies of the maxillary anterior teeth were studied in seventy-seven children affected by unilateral and bilateral clefts of the lip and alveolar process, with or without involvement of the palate. As for the permanent lateral incisor in the cleft area, our results show that its congenital absence is the most frequent abnormality followed by anomalies in size and shape and supernumerary teeth. Enamel hypoplasia was found to affect the permanent central incisor on the cleft side more frequently. Early recognition of tooth abnormalities during the primary dentition phase for an interceptive treatment of potentially severe problems was emphasized.     

Nonsyndromic cleft lip with or without cleft palate: evidence of linkage to BCL3 in 17 multigenerational families

Nonsyndromic cleft lip with or without cleft palate (CL/P) is a common craniofacial developmental defect. Recent segregation analyses have suggested that major genes play a role in the etiology of CL/P. Linkage to 22 candidate genes was tested in 11 multigenerational families with CL/P, and 21 of these candidates were excluded. APOC2, 19q13.1, which is linked to the proto-oncogene BCL3, gave suggestive evidence for linkage to CL/P. The study was expanded to include a total of 39 multigenerational CL/P families. Linkage was tested in all families, using an anonymous marker, D19S178, and intragenic markers in BCL3 and APOC2. Linkage was tested under two models, autosomal dominant with reduced penetrance and affecteds only. Homogeneity testing on the two-point data gave evidence of heterogeneity at APOC2 under the affecteds-only model. Both models showed evidence of heterogeneity, with 43% of families linked at zero recombination to BCL3 when marker data from BCL3 and APOC2 were included. A maximum multipoint LOD score of 7.00 at BCL3 was found among the 17 families that had posterior probabilities > = 50% in favor of linkage. The transmission disequilibrium test provided additional evidence for linkage with the 3 allele of BCL3 more often transmitted to affected children. These results suggest that BCL3, or a nearby gene, plays a role in the etiology of CL/P in some families.     

Evaluation of the results of cleft lip and palate surgical treatment: preliminary report

We have analysed randomized controlled studies which reported the incidence of postoperative nausea and vomiting (PONV) after propofol anaesthesia compared with other anaesthetics (control). Cumulative data of early (0-6 h) and late (0-48 h) PONV were recorded as occurrence or non-occurrence of nausea or vomiting. Combined odds ratio and number-needed-to-treat were calculated for propofol as an induction or maintenance regimen, early or late outcomes, and different emetic events. This was performed for all control event rates and within a range of 20-60% control event rates. We analysed 84 studies involving 6069 patients. The effect of propofol on PONV was dependent mainly on the method of administration, time of measurement and range of control event rates. When all studies were included the number-needed-to-treat to prevent PONV with propofol was more than 9 when used for induction of anaesthesia and at best 6 when used for maintenance. Within the 20-60% control event rate range, best results were achieved with propofol maintenance to prevent early PONV: the number-needed-to-treat to prevent early nausea was 4.7 (95% confidence interval 3.8-6.3), vomiting 4.9 (4-6.1) and any emetic event 4.9 (3.7-7.1). Within the 20-60% control event rate, of five patients treated with propofol for maintenance of anaesthesia, one will not vomit or be nauseated in the immediate postoperative period who would otherwise have vomited or been nauseated. This may be clinically relevant. In all other situations the difference between propofol and control may have reached statistical significance but was of doubtful clinical relevance. Treatment efficacy should be established within a defined range of control event rates for meaningful estimates of efficacy and for comparisons.     

Nonsyndromic cleft lip and palate: no evidence of linkage to HLA or factor 13A

Nonsyndromic cleft lip with or without cleft palate (CLP) is a common craniofacial anomaly, the etiology of which is not known. Population studies have shown that a large proportion of cases occur sporadically. Recently, segregation analyses applied to CLP families have demonstrated that an autosomal dominant/codominant gene(s) may cause clefting in cases. Associations of autosomal dominant CLP and nonsyndromic cleft palate (CP) with HLA and F13A genes on chromosome 6p have been suggested previously. Linkage to these two areas on chromosome 6p were tested in 12 autosomal dominant families with CLP. With a LOD score of -2 or less for exclusion, no evidence of linkage was found to four chromosome 6p markers. Multipoint analysis showed no evidence of a clefting locus in this region spanning 54 cM on chromosome 6p in these CLP families.     

Role of the pediatrician in the treatment of children with cleft lip and palate

The authors studied the relation between human central dopamine (DA) metabolism and the clinical effects of neuroleptics. The neuroleptic-induced increase in central DA turnover (an indicator for the degree of DA receptor blocking) was found to be positively correlated with the therapeutic effect of neuroleptics and the development of hypokinetic-rigid symptoms. This supplies a direct argument in support of the contention that DA antagonism is related to the occurrence of clinical effects. The authors also found indications that neuroleptics of different chemical types do not significantly differ in their intrinsic ability to provoke hypokinetic-rigid symptoms, that development of these symptoms depends on the patient's individual susceptibility, and that individual susceptibility is based on relatively low DA turnover.