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[Early repair for infants with cleft lip. Retrospective study of 263 cleft lip repairs].

This study attempts to define the effect of early repair on the surgical procedure and immediate outcome of cleft lip surgery. The first part deals with 263 consecutive cleft lip repairs (218 infants) categorized retrospectively by age at operation. 123 patients (150 cleft lips) were operated on during the first 4 weeks of life; a subgroup of 40 infants was operated on at a week or less of age; 95 patients (113 cleft lips) were operated at an older age (1 to 12 months). There were no significant difference in immediate surgical result between the groups and there was no apparent difference in the operative results as defined by whether or not the child needed subsequent revision. We are currently encouraging early repair in the full-term baby as the optimum method of management of newborns with cleft lip.

Age Factors↗

Nasal deformity and microform cleft lip in parents of patients with cleft lip.

OBJECTIVE: To describe subtle nasal deformities and microform cleft lips in parents whose children have complete cleft lip deformities. DESIGN: Clinical analyses of three parents whose children had complete cleft lips. Subtle nasal deformities and microform cleft lips were identified. SETTING: An institutional general hospital: Manuel Gea González Cleft Lip and Palate Clinic in Mexico City, Mexico. PATIENTS: The study first examined the parents of all complete and incomplete cleft lip-palate patients who were seen from March 1994 to February 1997 by the authors (n = 1000). We identified three patients, each of whom had one parent who showed signs of subtle nasal deformity and microform cleft lip. INTERVENTIONS: None for the parents. Interventions in the children with cleft lips and palates were known. MAIN OUTCOME MEASURES: Qualitative photographic analyses were performed. Nostril symmetry, septal deviation, nasal floor position, and orbicularis oris malinsertions were carefully examined. RESULTS: Three of the evaluated parents had one alar cartilage caudally displaced and a deviated septum. One parent's nasal floor was depressed. Two parents also had evidence of a minimal orbicularis oris muscle fissure located in the upper lip. CONCLUSION: Genetic evaluations of children with complete and incomplete cleft lips might also include thorough evaluation of their parents. Although small in size, this study of three case histories identified nasal and lip deformities in the patients' parents.

Adult↗

Cleft lip mongolian spot: mongolian spot associated with cleft lip.

BACKGROUND: Mongolian spots in the cleft area of cleft lip have been found in some Japanese children. OBJECTIVE: Our purpose was to study the frequency of cleft lip mongolian spot in children with cleft lip of various severity. METHODS: Sixty-six babies with unilateral cleft lip were divided into three groups: namely, those with microform cleft lip (10 subjects), incomplete cleft lip (30 subjects), and complete cleft lip (26 subjects). The incidence of cleft lip mongolian spot in the three groups was studied. RESULTS: Thirty-six babies (55%) had a cleft lip mongolian spot. The mongolian spot was observed in no patients with microform cleft lip, in 18 patients (60%) with incomplete cleft lip, and in 18 patients (69%) with complete cleft lip. CONCLUSION: Cleft lip mongolian spot appears in high incidence when the cleft goes beyond the vermilion border.

Cleft Lip↗

Adverse outcomes following endoscopic repair of a fetal cleft lip using an ovine model.

OBJECTIVE: The purpose of this study was to determine if endoscopic techniques could be used to repair an epithelialized lip cleft with accuracy and with an outcome comparable to fetuses treated through an open hysterotomy. INTERVENTIONS AND RESULTS: In contrast to previous open fetal cleft lip repairs in the same model, none of the five fetuses reported here had a good aesthetic result. Although there was no evidence of scar histologically, the edges of the lip were poorly approximated. The epithelial lining and underlying dermis of the wound margins were notably inverted. The orbicularis oris muscle, which had been reapproximated, appeared thin and hypoplastic. Most of the vermilion elements were poorly aligned, and in one animal, there was a complete dehiscence of the repair. CONCLUSIONS: In a more representative model of cleft lip that is not an acute lip wound, in utero endoscopic suture repair of the ovine lip gave a poor result using current technology. Only a meticulously performed, multilayered, open repair of a cleft appears to give a good cosmetic and functional outcome. Further studies to improve the endoscopic repair as our technology advances are therefore warranted.

Animals↗

[Nutrition of children with cleft lip and cleft palate, a bibliographic study].

The present study aimed at describing the children with congenital lip-palate malformation, emphasizing their alimentation, such as the difficulties and alternative methods to feed these children and nursing care in this process. The bibliographic review showed that there are many aspects in the alimentation care to fissured children, involving complex affective, social, economic and cultural relations that influence in mother-child relationship, in social discrimination of the fissured children and in mothers' difficulty to feed and give care to the child. Authors found the need to implement breast feeding, pointing out the physiological, psychological and social limitations that involve the child and the family as well as the importance of alimentation to physical and emotional development, favouring the surgical correction of lips and/or palate fissures as well as the rehabilitation process.

Child↗

Mandibular dysmorphology in patients with unilateral cleft lip and cleft palate.

BACKGROUND: Conventional studies of the cleft lip/palate (CLP) dysmorphology have mainly focused on deformities of the lip, nose, and maxilla, while ignoring the mandible. Reasons for that were the lack of well-defined mandibular deformity and restriction from the research methodology. METHODS: This study used 3-dimensional computed tomography (CT) imaging data from 35 patients with unilateral CLP. The 3-dimensional images were rotated into a neutral position. Eight cephalometric landmarks were recorded: the pogonion (PG) and the infradentale (ID) from the frontal view; and the condylion (CO), the tip of coronoid process (CP), and the gonion (GO) from both sides of lateral views. The nasion was used as a reference point for the facial midline. Nine linear distances and four angular measurements were calculated from these landmarks. Each mandible was segmented into two hemi-mandibles for volume measurements. The image manipulation and measurements were performed using a personal computer running Analyze' program. Landmark deviation from the facial midline was computed, and comparisons were made between the cleft and non-cleft sides. RESULTS: The results showed that the precision and accuracy of landmark localization was high with an average error of 0.4%. Deviation from the midline of the ID and PG points, and spatial distances between bilateral CP, CO, and GO points varied without a specific pattern. The average differences were within 2 mm. The volume of the cleft side hemi-mandible was consistently larger than that of the non-cleft side (p < 0.0001). Among linear and angular measurements, CP-GO-PG, CO-ID, CP-ID, and CP-GO showed significant difference between the two sides. CONCLUSION: This study demonstrated that mandibular asymmetry and deformity existed and was measurable in patients with unilateral CLP. The influence of CLP to the mandibular development was expressed by the significant differences of hemi-mandible volume and some of the linear and angular measurements between the cleft and non-cleft sides.

Cleft Lip↗

Cleft sidedness and congenitally missing or malformed permanent maxillary lateral incisors in Korean patients with unilateral cleft lip and alveolus or unilateral cleft lip and palate.

INTRODUCTION: The purpose of this study was to investigate the differences in the prevalence of cleft sidedness, the number of congenitally missing or malformed permanent maxillary lateral incisors (MLIs), and the presence of supernumerary teeth in Korean patients with unilateral cleft lip and alveolus (UCLA) and those with unilateral cleft lip and palate (UCLP). METHODS: Seventy-five patients with UCLA and 129 patients with UCLP (ages, 6.0 to 13.0 years) were studied by using their charts, models, radiographs, and intraoral photographs. The chi-square test, the binomial test, and the relative risk assessment were used. RESULTS: There was a male dominant tendency in both UCLP (P <.001) and UCLA (P <.05) patients. The UCLP patients were 1.3 times more involved on the left side and had 2.2 times more congenital missing MLI than did the UCLA patients. When an MLI was present, malformation occurred more frequently than normal shape in the UCLA (P <.001) and UCLP (P <.01) patients. Incidence of supernumerary teeth was 4.6 times higher in UCLA than in UCLP patients. CONCLUSIONS: When the cleft extended to the secondary palate, left-sided involvement was more predominant than in UCLA patients. When cleft involvement was confined to the primary palate, present MLIs were more common than congenitally missing teeth, and supernumerary teeth were more prevalent than in UCLP patients. These are important characteristics to be considered in the diagnosis and treatment planning of cleft patients.

Adolescent↗

Facial balance in cleft lip and palate. II. Cleft lip and palate and secondary deformities.

The cleft abnormality is the cause of underdevelopment and subsequent loss of function. Primary cleft surgery and surgery to correct the secondary deformities of previous non-functional repair should aim to restore normal anatomy and physiology, with an emphasis on muscle reconstruction of the lip and soft palate if normal facial development is to be encouraged.

Cleft Lip↗

Congenital missing permanent teeth in Korean unilateral cleft lip and alveolus and unilateral cleft lip and palate patients.

OBJECTIVE: To investigate the differences in the congenital missing teeth pattern in terms of tooth type (permanent maxillary lateral incisor [MLI] and maxillary second premolar [MSP]) and sidedness (cleft vs noncleft) between boys and girls in Korean unilateral cleft lip and alveolus (UCLA) and unilateral cleft lip and palate (UCLP) patients. MATERIALS AND METHODS: This study used the charts, models, radiographs, and intraoral photographs of 90 UCLA patients and 204 UCLP patients (ages 6 to 13 years). Binomial test, chi-square test, Fisher exact test, maximum likelihood analysis of variance, and the odds ratio were performed. RESULTS: According to the relationship between the congenital missing teeth pattern and the cleft type, the UCLP patients had 2.98 times more missing MLIs and 1.80 times more missing MSPs than did the UCLA patients. The MLI was congenitally missing more in boys than in girls, but the MSP showed the opposite tendency. Boys had a higher frequency of congenital missing MLIs and MSPs on the cleft side than did girls. However, on the noncleft side and both sides, girls had a higher frequency of congenital missing MLIs and MSPs than did boys. Results showed a gender-dominant pattern of congenital missing MLIs and MSPs. CONCLUSION: These results suggest that gender and cleft type might affect the congenital missing teeth pattern in terms of tooth type and sidedness.

Adolescent↗

Craniofacial comparisons in 22-month-old lip-operated children with unilateral complete cleft lip and palate and unilateral incomplete cleft lip.

OBJECTIVE: The objective of the study was to analyze the craniofacial morphology in infants with unilateral complete cleft lip and palate (UCCLP) in which the lip and the anterior part of the palate had been surgically closed at 2 months of age and to compare the morphology with that of a control group with unilateral incomplete cleft lip (UICL) in which the lip had also been surgically closed at 2 months of age. DESIGN: The sample consisted of a total of 108 cleft children all fulfilling the entry criteria, besides diagnosis, as follows: The child was of Danish origin; the age of the child was between 650 and 750 days (approximately 22 months) at the time of examination; the child was healthy except for its single cleft malformation; the surgical procedure in each group had been performed at about 2 months of age by the same surgeon. The surgical methods used were a Tennison procedure (UICL group) and a Tennison procedure supplemented by palatovomer plasty (UCCLP group). METHODS: The method of investigation was infant cephalometry in the lateral, frontal, and axial projections. Linear, angular, and area variables describing the craniofacial morphology were calculated and supplemented by mean plots from the cephalometric projections in the two groups. RESULTS AND CONCLUSIONS: Statistical analysis based on Student's t test showed that the facial morphology in the 22-month-old UCCLP group differed significantly from that of the UICL group. The most pronounced differences were found in the maxillary complex and the mandible. The deviations observed in the UCCLP group at 22 months of age were similar to those previously observed at 2 months of age. However, several of the dysmorphic traits had become less pronounced; some had remained the same; and a few had become worse with time.

Anthropometry↗

Prevalence of aneuploidy and additional anatomic abnormalities in fetuses and neonates with cleft lip with or without cleft palate: a population-based study in Utah.

OBJECTIVE: To determine the prevalence of aneuploidy and additional major anatomic abnormalities in fetuses and neonates with cleft lip with or without cleft palate. METHODS: All cases of cleft lip with or without cleft palate (cleft lip/cleft palate) occurring in Utah from 1995 through 1999 were reviewed by using the Utah Birth Defect Network population-based surveillance system. All pregnancy outcomes are included (stillborn, live born, and termination) in this analysis. RESULTS: Of 263 cases of cleft lip/cleft palate, 72 (27.4%) were unilateral cleft lip, 112 (42.6%) were unilateral cleft lip and cleft palate, 12 (4.6%) were bilateral cleft lip, and 67 (25.5%) were bilateral cleft lip and cleft palate. Fifteen (5.7%) of the 263 fetuses and neonates were aneuploid. One (1.2%) with cleft lip (unilateral and bilateral combined) was aneuploid. Five (4.5%) of the fetuses and neonates with unilateral cleft lip and cleft palate were aneuploid compared with 9 (13.4%) of fetuses and neonates with bilateral cleft lip and cleft palate. In known or presumed euploid fetuses and neonates, additional sonographically occult major anatomic abnormalities occurred in 5 (7.0%) of 71 with unilateral cleft lip, 18 (16.8%) of 107 with unilateral cleft lip and cleft palate, 1 (8.3%) of 12 with bilateral cleft lip, and 12 (20.7%) of 58 with bilateral cleft lip and cleft palate. These abnormalities primarily involved the heart and the central nervous system. CONCLUSIONS: Amniocentesis for karyotype should be offered in all cases of cleft lip/cleft palate because of the risk of aneuploidy. Patients should be counseled that sonographically occult additional anatomic abnormalities might be present with all clefts.

Amniocentesis↗

A comparison of the craniofacial morphology in 2-month-old unoperated infants with unilateral complete cleft lip and palate, and unilateral incomplete cleft lip.

This paper reports a cephalometric analysis of the craniofacial morphology in infants with unoperated unilateral complete cleft lip and palate (UCCLP) and unoperated unilateral incomplete cleft lip (UICL). The purpose of the study was to determine the nature and extent of the craniofacial deviations in UCCLP as compared to the morphology in UICL, which has previously been shown to be close to normal. The samples comprised 82 infants with UCCLP (58 males and 24 females) and 75 with UICL (48 males and 27 females). The mean age was about 2 months in both groups. The cephalometric analysis of craniofacial morphology included the lateral, frontal, and axial projections. The data were presented as mean plots of the craniofacial region including the calvaria, cranial base, orbits, nasal bone, maxilla, mandible, cervical column, pharynx, and soft-tissue profile. The most pronounced deviations in the UCCLP group were observed in the maxillary complex and the mandible. The most striking findings were: markedly increased width of the maxilla, a short mandible, and bimaxillary retrognathia except for the premaxillary area, which was relatively protruding and asymmetric. The study did not support the hypothesis previously suggested in the literature that cleft lip and palate is a craniofacial anomaly as size and shape of the calvaria and cranial base were found to be normal. The etiology of cleft lip and palate is still incompletely understood. Based on the present study, we suggest that facial type may be a liability factor that could represent a developmental threshold increasing the probability of cleft lip and palate.

Anthropometry↗

Craniofacial growth in subjects with unilateral complete cleft lip and palate, and unilateral incomplete cleft lip, from 2 to 22 months of age.

This paper reports a longitudinal quantitative cephalometric analysis of the craniofacial growth in subjects with unilateral complete cleft lip and palate (UCCLP), and unilateral incomplete cleft lip (UICL), from 2 to 22 months of age. The purpose of the study was to determine the amount and direction of growth in UCCLP compared to UICL (control group) from 2 months of age (just prior to lip repair) to 22 months of age, 20 months later. The sample comprised of 49 subjects with UCCLP (37 males and 11 females) and 45 with UICL (29 males and 16 females). The cephalometric analysis of the craniofacial morphology included lateral, frontal, and axial projections. The data were presented as mean plots of the craniofacial region including the calvaria, cranial base, orbits, nasal bone, maxilla, mandible, cervical column, pharynx, and soft-tissue profile. A valid common coordinate system (registration according to the n-s line in the lateral projection, latero-orbitale line in the frontal projection, and meatus acusticus externus line in the axial projection for the landmark positions at examination 1 and 2) was ascertained. The growth at a specific anatomical location in a patient was defined as the displacement vector from the coordinate of the corresponding landmark in the X-ray at examination 1 to its coordinate at examination 2, corrected for X-ray magnification. The growth of an anatomical region in a patient was assessed by investigating the growth pattern formed by a collection of individual growth vectors in that region. The amount of growth in the UCCLP and UICL group was very similar. The general craniofacial growth pattern, in terms of the direction of growth, was also fairly similar in the UCCLP group and the control group. However, the maxilla and mandible showed a more vertical growth pattern than that observed in the control group. This study confirms that UCCLP is a localized deviation, and not a craniofacial anomaly, due to the fact that a normal growth potential has been observed in all craniofacial regions, except where the growth had been directly influenced by surgical intervention. Furthermore, the vertical growth pattern of the maxilla and mandible supports the hypothesis of a special facial type in cleft lip and palate individuals, and the facial type as a liability factor increasing the probability of cleft lip and palate.

Cleft Lip↗

Philtrum formation in cleft lip surgery.

This is a modification of Millard's rotation-advancement principle in closing unilateral lip clefts, published in 1971. In selected cases, if there is enough soft tissue at the base of the columella to fill the defect between the columella base and the rotated short part of the philtrum, a slightly curved scar will be formed resembling the normal philtral column. From 1967 till the end of 1981, out of a total number of 1305 unilateral cleft lip repairs, 189 were operated in this manner using the described modification of Millard's technique with good results. For most of the other cases and especially with wide clefts, the original method of Millard is preferred.

Adolescent↗

The Sri Lankan Cleft Lip and Palate Project: the unoperated cleft lip and palate.

Since 1984, the Sri Lankan Cleft Lip and Palate Project has developed a large surgical and research program collecting records on over 500 subjects with unrepaired cleft lip and palate. In addition, 410 operations were performed by Project and will be followed by individual reports on facial growth and morphology, speech, surgical and anesthetic aspects, and the otologic significance of cleft palate in this issue and subsequent ones.

Adolescent↗

[Nonsyndromic cleft lip with or without cleft palate in Chinese population: analysis of 3766 cases].

A retrospective study was conducted to evaluate the variation trend in the incidence and epidemiological characteristics of nonsyndromic cleft lip with or without cleft palate in Chinese population. From 1988 to 1992, data were collected from more than 500 hospital-based units involved in a monitoring program and were analyzed by the National Center for Birth Defects Monitoring. The monitored subjects were the hospitalized births from 28 weeks gestation to a period within 7 days after delivery, which included 3246 408 perinatals and 3766 cases. The total incidence of nonsyndromic cleft lip with or without cleft palate was 11.6/10,000; the total incidence of celft lip alone was 3.8/10,000 and that of cleft lip with cleft palate was 7.8/10,000; no significant variation was noted in the annual incidence from 1988 to 1992. The incidence in males was 13.3/10,000, and 9.5/10,000 in females; there was a significant difference (P < 0.01). Of all the 3766 cases, cleft lip with cleft palate accounted for 67%, unilateral cleft lip 28% and unilateral cleft lip with cleft palate 43%. Left cleft lip and left cleft lip with cleft palate constituted 21% and 29% respectively. The sex-ratio was 1.6:1 for nonsyndromic cleft lip with or without cleft palate. It could be concluded that the incidence of nonsyndromic cleft lip with or without cleft palate showed no variation trend in China. Although no difference was found between urban and rural areas, the susceptibility to the defect was higher in males than in females. Cleft lip with cleft palate was more common than other types. There was a predominance of unilateral defects, of which most were on the left side.

China↗