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Bilateral cleft lip repair.

From 1970 to 1987 we treated 213 patients with bilateral cleft lip at Hokkaido University Hospital and its several branch hospitals. The patients were grouped into 3 types according to the shape of the clefting prolabium. Group 1: complete clefts bilaterally, group 2: incomplete clefts bilaterally, group 3: combination of complete and incomplete clefts. From 1974 to 1987 we treated 176 bilateral cleft lip patients by the Manchester method using several modifications. Presurgical oral orthopaedics, using head cap and orthodontic appliance devised by Dr Peat, was applied to 110 of the 176 cases. Surgical results were satisfactory and the Manchester method is our preferred procedure for bilateral cleft lip repair.

Child↗

A study of fluctuating dermatoglyphic asymmetry in the sibs and parents of cleft lip propositi.

Fluctuating asymmetry was studied in cleft lip propositi and their normal sibs and parents. The traits examined were a-b ridge counts and fingerprint patterns. Propositi with a family history of this congenital malformation and their normal sibs and parents were significantly different from the controls for this type of asymmetry. Propositi without a family history and their normal sibs and parents were similar to the controls. These results support the hypothesis that familial and sporadic cases of congenital cleft lip are different entities and give evidence for a genetic mechanism in the parents and sibs of the familial cases that may account for this congenital disorder and, concomitantly, increased fluctuating asymmetry.

Cleft Lip↗

Size and form of the cranial base in isolated cleft lip and palate.

Most cases with cleft lip and palate (CLP) have no other obvious malformation and are termed isolated CLP. The implication is that these individuals experienced a time-specific and anatomically-localized disturbance during palatogenesis but are otherwise normal. The cranial base was studied from cephalograms in subadults with isolated CLP (n = 43) to test for alterations in size and shape. Controlling for age and gender, analyses showed no size difference, but flexure of the cranial base was significantly greater in the CLP group. Taken in conjunction with increased risks of other conditions with prenatal onsets, it appears that "isolated CLP" is a misnomer and the cleft should be viewed as an overt manifestation of a more generalized growth disturbance.

Adolescent↗

Associated malformations in infants with cleft lip and palate: a prospective, population-based study.

OBJECTIVE: Infants with cleft lip and palate may often have other associated congenital defects although the reported incidence and the types of associated malformations vary between different studies. The purpose of this investigation was to assess the prevalence of associated malformations in a geographically defined population. METHODS: The prevalence of associated malformations in infants with clefts were collected prospectively between 1975 to 1992 on all infants born in greater Stockholm, Sweden. The patient records were also compared with data from the National Malformation Registry and other hospital records if any. RESULTS: Of the 616 cleft infants (367 boys, 249 girls) born during this period, 21% had associated malformations that either required follow-up or treatment. Associated malformations were more frequent in infants who had both cleft lip and palate (28%) than in infants with isolated cleft palate (22%) or infants with isolated cleft lip (8%). Malformations of the upper or lower limbs or the vertebral column were the most common other anomalies and accounted for 33% of all associated defects. Twenty-four percent of associated malformations were in the cardiovascular system and congenital heart disease was the most common isolated associated malformation. Fifteen percent of all associated malformations were multiple and they were frequently associated with mental retardation or chromosomal anomalies. Twenty-two percent of infants with associated malformations were born preterm, compared with an expected 5% incidence of preterm delivery in Sweden. CONCLUSION: A more extensive cleft seems to be associated with a higher risk for associated malformations. Although many associated congenital defects can be detected at a physical examination, the high prevalence of congenital heart disease (16 times that of general population) may justify a routine echocardiographic screening.

Abnormalities, Multiple↗

Management of cleft lip and palate in primary care.

Cleft lip and palate malformations are the most common congenital craniofacial abnormalities. Patients with these deformities require coordinated care involving multiple disciplines from birth throughout adolescence. The primary care provider plays a vital role in facilitating the provision of care for these patients, who often have numerous health care needs, including feeding difficulties, speech disorders, chronic ear infections, and dental and orthodontic problems. The emotional issues of patients with these deformities and their families are significant and include disrupted parent and child bonding, body image disturbances, and impaired socialization. The role of the primary care provider is to be a stable source of support for these patients and to assist in coordinating care for the many physical and emotional problems they may have.

Child, Preschool↗

Speech outcomes in adolescents with cleft lip and palate.

Speech results were surveyed in 110 adolescent patients with clefts: 53 with unilateral cleft lip and palate, 46 with bilateral cleft lip and palate, and 11 with isolated clefts of the secondary palate. Only 12 of the 110 teenagers had received consistent team care from infancy. The early physical management was impossible to reliably determine in the remaining 98. Speech was normal in 22.7%, characterized by a variety of problems in approximately 66%, and a complete habilitative failure in 10.9%. Cleft palate and craniofacial teams who first encounter incompletely managed cases in the teenage years are faced with complex and interrelated challenges of providing appropriate physical management, speech habilitation, and psychosocial support, including determination of that approach which will be most likely to assure future compliance with treatment recommendations to each child and family.

Adolescent↗

Assessment of growth and feeding practices in children with cleft lip and palate.

Feeding difficulties in cleft lip and palate (CLP) infants is commonly observed and is the most traumatic experience the family has to face. These infants are undernourished and have compromised growth. The purpose of this study was to 1) assess general health and growth parameters in children with CLP and in normal children; and 2) investigate the feeding methods of CLP infants and normal infants. A total of 221 children from birth to six years of both sexes, with CLP (60 children) and normal (161 children) were selected. The CLP and normal children were divided into three subgroups by age. The practice of feeding the infants in subgroup I was assessed using standard piloted questionnaires. The assessment of growth was done at baseline and at six months in all the subgroups.The general well being of the children was assessed by noting the number of common infections. Results showed that a significantly higher percentage of mothers with normal babies (p < 0.01) had a positive attitude towards breast feeding. When compared to normal children, CLP children were more susceptible to infections (p < 0.05) and measured significantly lower on the height growth curve(p < 0.05). Hence, height can be used to monitor growth in CLP children.

Body Height↗

[Electromyography study on complete bilateral cleft lip after reparation.].

PURPOSE: This study was undertaken to evaluate the motion effect of complete bilateral cleft lips objectively using electromyography. METHODS: 45 patients of complete bilateral cleft lips after operations several years which included 20 patients repaired using orbicularis oris muscle restoring functional method and 25 patients using straight line suture method were examined with electromyography (EMG) in prolabium, right and left lateral lips respectively. The most potential in every area of each patient at the maximal lip contraction position was measured and the values of two different method groups were subject to statistical analysis. RESULTS: The prolabium and lateral lips of both groups had no electric activity at posture position. At maximal contraction position, the mean potential of prolabium of orbicularis oris muscle restoring method group had no difference with the lateral lips. But the mean potential of prolabium of straight line suture method group was lower than that of the lateral lips significantly,also,it was lower than the potential of prolabium in the muscle functional reparation group. CONCLUSIONS: The reparation method of restoring orbicularis oris muscle to complete bilateral cleft lips was better than the straight line suture method in the motion effect. These findings suggest the superiority and necessity of functional muscle reparation for complete bilateral cleft lips.

Cleft Lip↗

Dental rotation in cleft lip.

In patients with untreated cleft lip and permanent dentition, the central superior incisors are usually rotated. We use a technique that we call "dental rotation" to adjust these teeth into normal position and occlusion. This technique avoids having a hard structure (a rotated tooth) press against the mucosal sutures in a lip closure. In addition, a better aesthetic appearance of the dental arch results.

Child↗

A new approach to bilateral cleft lip repair.

The basis of this new approach to bilateral cleft lip repair is the emphasis placed on a thorough understanding of the deformity itself and a detailed knowledge of the normal anatomy of the upper lip, especially that of the philtrum. A population survey has been carried out to determine what the normal philtral dimensions are at the different age groups relevant to the operation milestones in cleft lip surgery. A separate study to determine the histological basis of the philtral configuration has also been carried out. Based on the results obtained from these separate studies, a new approach and technique of bilateral cleft lip repair has been developed and applied to a series of 42 consecutive cases operated on by the author during the past 5 years.

Adult↗

The incidence of cleft lip and palate deformities in the south-east of Scotland (1971-1990).

This retrospective study reports the incidence of infants born with the cleft lip and palate anomaly within the Edinburgh Cleft Units catchment area, between 1 January, 1971, and 31 December, 1990. The importance of accurate data collection for local, regional, and national data bases is discussed with reference to the recent CSAG report on cleft lip and palate services in the UK. Five-hundred-and-two cleft lip and palate patients were identified (291 males, 211 females). The incidence is reported as 1.4 per 1000 live births (1 in 711). Twenty-five per cent of clefts affected the primary palate, 45 per cent affected the secondary palate, and the remaining 30 per cent were clefts of both the primary and secondary palate. Overall, a higher percentage of males were affected (58 per cent males to 42 per cent females). Clefts of the secondary palate, however, were more common in females (56 per cent females to 44 per cent males). Data presented in this study is similar to that previously reported from UK centres. It is suggested the accuracy of the UK cleft lip and palate data collection needs to be improved. Prospective data collection in a standardized format carried out on a national basis has to be a priority as recommended by the CSAG report.

Catchment Area, Health↗

Long term follow up study of survival associated with cleft lip and palate at birth.

OBJECTIVE: To assess the overall and cause specific mortality of people from birth to 55 years with cleft lip and palate. DESIGN: Long term follow up study. SETTING: Danish register of deaths. PARTICIPANTS: People born with cleft lip and palate between 1943 and 1987, followed to 1998. MAIN OUTCOME MEASURES: Observed and expected numbers of deaths, summarised as overall and cause specific standardised mortality ratios. RESULTS: 5331 people with cleft lip and palate were followed for 170 421 person years. The expected number of deaths was 259, but 402 occurred, corresponding to a standardised mortality ratio of 1.4 (95% confidence interval 1.3 to 1.6) for males and 1.8 (1.5 to 2.1) for females. The increased risk of mortality was nearly constant for the three intervals at follow up: first year of life, 1-17 years, and 18-55 years. The participants had an increased risk of all major causes of death. CONCLUSIONS: People with cleft lip and palate have increased mortality up to age 55. Children born with cleft lip and palate and possibly other congenital malformations may benefit from specific preventive health measures into and throughout adulthood.

Adolescent↗

Primary repair of unilateral cleft lip.

The author's technique for the primary repair of unilateral cleft lip is described, including several improvements made over the last eight years. The technique aims at anatomical reconstruction of congenital anomalies of the orbicularis oris muscle as well as prevention of developmental disturbance and occurrence of deformity after surgical intervention. Cleft lip repair without muscle reconstruction may leave a concavity or a lump in the upper lip movement because of incomplete continuity of the orbicularis oris. No such result was found when muscle reconstruction was performed. In the primary repair of cleft lip, reconstruction of the orbicularis oris muscle is vital to preservation of good function and appearance of the lips.

Cleft Lip↗

Outpatient cleft lip repair.

The emphasis on cost reduction and increased efficiency in health care delivery has prompted an increase in outpatient (ambulatory) surgical procedures. A retrospective review of the perioperative management of patients undergoing cleft lip repair at two urban tertiary pediatric hospitals was performed to assess the safety of outpatient cleft lip repair. The hospital database at Childrens Hospital Los Angeles was searched to find all patients who had been operated on for cleft lip repair during calendar years 1999 and 2000. Two groups were identified from Childrens Hospital Los Angeles: the outpatient cleft lip repair group (patients discharged the same day as the operation; n = 91) and the inpatient cleft lip repair group (n = 14). A data set was acquired from the Royal Children's Hospital in Melbourne, Australia, using the same criteria, for fiscal years 1998 to 2000 (n = 50). All patients from Royal Children's Hospital had operations as inpatients. Parameters considered for each group were age, sex, race, ethnicity, length of hospital stay, preexisting medical conditions or diagnoses, complications, and readmissions or presentation to the emergency department within 4 weeks of operation. The Childrens Hospital Los Angeles outpatient group had three readmissions that were considered to be complications of the operation. The Childrens Hospital Los Angeles inpatient group had one readmission attributable to a complication. The Royal Children's Hospital group also had one readmission for a complication. There was no significant difference in the complication rate of the Childrens Hospital Los Angeles outpatient group and the Royal Children's Hospital group (p > 0.05). There was also no significant difference in the complication rate of both of the Childrens Hospital Los Angeles groups compared with the Royal Children's Hospital group (p > 0.05). This study indicates that cleft lip repair performed in an outpatient setting may be a safe alternative to the inpatient operation. Certain preexisting medical conditions, however, may dictate the need for inpatient hospitalization after repair.

Ambulatory Surgical Procedures↗

Dominantly inherited cleft lip and palate in two families.

Two families with non-syndromic cleft lip and cleft palate are described. The linear pattern of inheritance through several generations is difficult to explain by conventional multifactorial models. The pedigrees strengthen the suggestion that a dominantly inherited mutation exists with a major influence on clefting of the lip and palate alone.

Adult↗

In a Vietnamese population, MSX1 variants contribute to cleft lip and palate.

PURPOSE: To identify causes of nonsyndromic cleft lip and palate in a Vietnamese population. METHODS: In this study, 175 families with at least one case of cleft lip and/or palate were studied using the candidate genes TGFA, MSX1, and TGFB3. RESULTS: Transmission distortion for alleles of MSX1 were demonstrated for the whole population and two missense mutations were identified, including one (P147Q) that is found in approximately 2% of the population. The P147Q appears to arise from a founder individual based on shared haplotypes in unrelated families. CONCLUSIONS: MSX1 contributes to nonsyndromic clefting in a Vietnamese population, and consistent with other studies, identifiable mutations in this gene cause about 2% of cases of nonsyndromic clefting.

Alleles↗

Oral disease status of a sample of Jordanian people ages 10 to 28 with cleft lip and palate.

OBJECTIVE: To determine the levels of periodontal disease and dental caries in subjects with cleft lip and palate and to compare them with matched noncleft control subjects. DESIGN: A total of 32 subjects with cleft lip and palate, ages 10 to 28 years, and a similar number of noncleft control subjects were examined for plaque biofilm deposits, gingivitis, periodontitis, and dental caries by using the Silness and Löe plaque index (PI), Löe and Silness gingival index (GI), probing pocket depth (PPD), and the decayed-missing-filled-teeth (DMFT) index, respectively. SETTING: Faculty of Dentistry, Jordan University of Science and Technology, and Prince Rashed Hospital, Royal Medical Services, northern Jordan. RESULTS: Scores for PI, GI, PPD, and DMFT were significantly higher in subjects with cleft lip and palate than in control subjects. CONCLUSION: Subjects with cleft lip and palate are at an increased risk for dental caries and periodontal disease when compared with a noncleft population.

Adolescent↗

Cleft lip and palate in southwestern Iran: an epidemiologic study of live births.

BACKGROUND: Cleft lip with or without cleft palate (CL/P), is the most common congenital anomaly in the head and neck worldwide. We studied the prevalence of cleft lip (CL) and palate (CP) in Shiraz, which is located in southwestern Iran. METHODS: Data was collected from the teaching hospitals of the Shiraz University of Medical Sciences. Records of 147,608 consecutive live births delivered in three hospitals during the 10 years from November 1993 to November 2003 were reviewed. RESULTS: One hundred nineteen cases of CL/P or CP alone were registered for a prevalence of 0.80 per thousand live births. The overall male/female ratio was 1.25. The male/female ratio was 1.22 in the CL patients and 1.58 in the CL/P patients. A male predominance was obvious in both groups. CONCLUSION: This study shows that the prevalence rate of CL and CP in southwestern Iran is closer to the low prevalence reported in African countries and is lower than the prevalence reported in previous reports in Caucasians.

Cleft Lip↗