Dental treatment need and dental general anesthetics among preschool‐age children with cleft lip and palate in northern Finland

Cleft lip and palate incidence is high in northern Finland. This study aimed to investigate the proportion of children in need of restorative dental treatment among cleft lip and palate patients in northern Finland, as well as their need for dental treatment under general anesthesia. The records of 183 cleft lip and palate patients, treated in Oulu University Hospital from 1997 to 2013, were reviewed. Data on dental caries were analyzed in association with cleft type, considering also the presence of syndromes. The frequency of dental general anesthetic (DGA) use, and of treatments, were also analyzed. Dental treatment need was most frequently observed, in this rather limited study population, in patients with the most severe deformities, namely bilateral cleft lip and palate, of whom 60% had caries. Among the study population, 11.5% (n = 21) had a syndrome. Of those, 57.1% had dental caries at the age of 3 or 6 yr, and only four could be treated without a DGA. Dental treatment under general anesthesia was performed in 14.8% of cleft patients without a syndrome, but in 38.1% of those with a syndrome. General anaesthesia is required for the provision of dental care more often in cleft (17.5%) than in non‐cleft (0.2%) patients, and especially for those with a syndrome.     

An epidemiologic study of oral clefts in Iran: analysis of 1,669 cases.

OBJECTIVE: The objective of this study was to assess the epidemiology and some genetic aspects of oral clefting in Iran. DESIGN: The study was a 15-year cross-sectional (prevalence) study from August 1976 to September 1991. SETTING: The setting for the study was two plastic surgery departments, both mostly referral centers, and a maternity hospital in Iran. PARTICIPANTS: The participants were 1,669 consecutive surgical cases with oral clefts (79% between 1 day and 18 months of age), registered in two centers. In a parallel study, 19,369 livebirths born in a maternity hospital within the same period were surveyed to ascertain prevalence of clefting at birth. Normal children of this population were used as the control group. Samples were analyzed by prevalence of clefting at birth, type of oral cleft, side of clefting, sex ratio, associated malformations, clefts in relatives, and parental consanguinity. RESULTS: The prevalence of clefts was 1.03 per 1,000 births. Cleft lip (without cleft palate) had a higher (34.9%) and cleft palate alone had a significantly lower prevalence (17.4%) than expected. Cleft lip (without cleft palate) was more common in females (53.6%) than in males (46.4%). The rate of associated malformations in cleft patients (7.73%) was higher than in controls (0.093%). Association between clefting and consanguinity was significant (45.8% in cases versus 43.1% in controls). Occurrence of noncleft congenital malformations among first-degree relatives of our cases (2.77%) was nearly two times that of controls (1.55%). CONCLUSION: Our study reveals that the population incidence of oral clefts in Iran is much closer to European than Arab-African or South East Asian countries. However, significant variations in other epidemiologic and some genetic features were observed.     

Maxillary arch dimensions in cleft infants in Northern Finland

Abstract

Objective. The aim of this study was to examine the maxillary arch dimensions in cleft lip and/or palate infants in Northern Finland before surgery. Materials and methods. The subjects consisted of 70 Finnish cleft patients born between 1997–2004 in Northern Ostrobothnia Hospital District in Finland. The study casts were obtained before surgery at the mean age of 5.6 months (SD = 2.2). There were 42 children with cleft palate (CP) (26 girls/16 boys), 13 with unilateral cleft lip and palate (UCLP) (eight girls/five boys), eight children with cleft lip (CL)(two girls/six boys) and seven with bilateral cleft lip and palate (BCLP) (two girls/five boys). Conventionally-used landmarks were marked on study casts and cleft width, arch circumference, anterior and posterior arch width and arch length were measured with a digital sliding calliper. The statistical method was ANOVA. Results. The prevalence of CP in this study, 60% of all clefts, is higher than the average standards. There were statistically significant differences in cleft width, posterior and anterior arch width, arch length and arch circumference, when different cleft groups were compared. When differences between girls and boys were compared, boys had larger cleft size and arch dimensions generally, but the results were not statistically significant. Conclusions. The results show the large variation in the severity of cleft lip and/or palate deformity at birth and in maxillary arch dimensions between different cleft types. It also demonstrates the effect of phenotypic variability within the groups of cleft lip and/or palate.     

Centre-based statistics of cleft lip with/without alveolus and palate as well as cleft palate only patients in Aden,Yemen

ObjectiveThe purpose of the study was to report the types and patterns of cleft lip with/without cleft alveolus and palate as well as cleft palate only as seen in Aden, Yemen.Design and settingRetrospective, centre-based study conducted at the Cleft Lip and Palate Centre, Aden University, Yemen.Material and methodsStatistical evaluation of the data from all cleft patients who were registered at or referred to this centre during the years 2005–2011.ResultsA total of 1110 cleft patients were seen during the period studied (2005–2011). Amongst these there were 183 (16.48%) with a cleft lip and 144 (12.98) with a cleft of lip and alveolus, 228 (20.54%) had a cleft palate, and 555 (50%) had a combination of cleft lip, alveolus, and palate. The clefts were found more often in males than in females (56.5% boys versus 43.5% girls). This difference was statistically significant (p ≤ 0.001). Statistically significant sex differences were also noted when evaluating the various cleft types. Isolated cleft palates were found most often in females. Among the cleft palate cases there were 102 (9.2%) with a cleft soft palate only. The ages of the patients were between one day and 40 years.Two hundred and one children (18%) had a positive family history of clefts. Among the risk factors considered in this study, consanguineous marriages among cousins were found most frequently (in 48% of the cases). In contrast to this, only 10% of the mothers had reported to have been taking medication directly prior to or during the first trimester of their pregnancy. On average the mothers were neither very young nor very old.ConclusionThe prevalence rate of orofacial cleft types among this Yemeni sample was similar to prevalence rates previously reported in white Caucasians. The present study did neither find many cases with medication before, nor during, pregnancy; there were few young or very old mothers; and the incidence of positive family histories was similar to those found in other studies on clefts. However, consanguineous marriages were encountered quite often.     

Genes causing clefting syndromes as candidates for non‐syndromic cleft lip with or without cleft palate: a family‐based association study

Clefts of the orofacial region are among the most common congenital defects, caused by abnormal facial development during gestation. Non‐syndromic cleft lip with or without cleft palate (NSCLP) is a complex trait most probably caused by multiple interacting loci, with possible additional environmental factors. As facial clefts form part of more than 300 syndromes, one strategy for identifying the genetic causes of NSCLP could be to study candidate genes responsible for clefting syndromes. Three genes were selected for this investigation: TP63, which codes for the tumour protein p63 and causes Ectrodactyly‐Ectodermal dysplasia‐orofacial Cleft syndrome; JAG2, a downstream gene of TP63; and MID1, which is responsible for Opitz syndrome. A linkage disequilibrium investigation was performed with intragenic single nucleotide polymorphisms on each of these genes in a sample study of 239 patients/parents trios. Evidence which suggests that JAG2 and MID1 may play a role in NSCLP was obtained.     

Non-syndromic orofacial clefts in Southern Italy: pattern analysis according to gender, history of maternal smoking, folic acid intake and familial diabetes.

BACKGROUND: Genetic studies have demonstrated that non-syndromic clefts of the lip, alveolus and palate have an heterogeneous genetic background, and that environmental factors contribute to the onset of this malformation. Therefore studies on different and homogeneous populations can be useful in detecting potentially related environmental and genetic factors. PURPOSE: The aim of the present study was to evaluate whether gender, folic acid intake, family history of diabetes and/or smoking during pregnancy were associated with a specific type of cleft in a group of patients affected by non-syndromic clefts, collected from Southern Italy. MATERIAL AND METHODS: Data from one hundred and twenty-six patients were evaluated retrospectively. Each cleft was described as composed by separate antomical entities such as lip, alveolus, primary and secondary palate. None had an isolated alveolar cleft and this was used as internal control. Pattern analysis was used to detect differences in the frequencies of any possible combination of 7 types of clefting stratified according to the studied variables. Data were analysed by comparing observed proportions. RESULTS: Isolated cleft palate as well as right-sided clefts of lip, alveolus and palate were more frequent in females (p = 0.0014 and 0.0281, respectively), while left sided clefts were more frequent in males (p = 0.0359). A lack of consumption of folic acid was associated with an higher incidence of clefts of the left lip (p = 0.018), while familial diabetes was associated more often with isolated cleft palate (p = 0.0014). CONCLUSIONS: Gender-related results were comparable with those found in Northern Italy and other countries. Environmentally related results disclosed specific subclasses of clefting associated with lack of folic acid consumption and familial diabetes.     

Left-sided predominance of hypodontia irrespective of cleft sidedness in a French population

Objective : Individuals with oral clefts exhibit considerably more dental anomalies than do individuals without clefts. Our aim was to evaluate the prevalence of tooth agenesis in a sample composed of 124 children (81 boys and 43 girls, mean age 12.5?years) with clefts registered with the Cleft Palate Center in Strasbourg (France). Design : Cleft types and dental agenesis were assessed by clinical and radiographic examination. Cleft types were divided into four groups according to the clinical extent of the cleft (cleft lip only [CL, 12.9%], cleft lip and alveolus [CLA, 4%], cleft lip and palate [CLP, 49.2%], and cleft palate only [CP, 33.9%]). Results : Of the subjects 63% had evidence of hypodontia: maxillary lateral incisors (54%) and upper and lower premolars (32%) were the most common missing teeth. The number of dental ageneses associated with CP (54%) and CLP (79%) was significantly higher than that with CL (33%). All these anomalies were found in proportionately higher frequencies as the severity of the cleft increased, and we found left side predominance for hypodontia (p < .01) irrespective of cleft sidedness. Conclusions : Both right-sided and left-sided clefts were more frequently correlated with left-sided dental agenesis. This left-sided prevalence suggests that common signaling malfunctions might be involved, both in dental development anomalies and cleft genesis.     

Research Reports

Abstract

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 centers. It is suggested the accuracy of the UK cleft lip and palate date 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.     

The prevalence of non-syndromic orofacial clefts and associated congenital heart diseases of a tertiary hospital in Riyadh,Saudi Arabia

BackgroundOrofacial clefts are considered one of the most common birth defects and are frequently associated with other malformations. Congenital heart disease is one of the most prevalent congenital malformation.ObjectiveTo investigate the prevalence of congenital heart diseases associated with non-syndromic orofacial clefts in the Saudi population.MethodsElectronic files of non-syndromic orofacial cleft patients who visited the Oral and Maxillofacial Surgery Department in King Abdulaziz Medical City of Riyadh, Saudi Arabia from January 2015 to December 2018 were retrospectively reviewed. Data were recorded in an excel sheet and analyzed using SPSS via frequency tests.ResultsIn the cleft children identified, the prevalence of non-syndromic orofacial clefts was (77%). Orofacial clefts showed a male predominance (62%). The most common orofacial phenotype was unilateral cleft lip and palate (34%). The prevalence of associated congenital malformations with orofacial clefts was (41%). The most prevalent congenital malformation was congenital heart disease (35%), mainly found in unilateral cleft lip and palate patients (33%). The prevalence of associated congenital heart disease with orofacial clefts was (19%). The most frequent type of congenital heart disease was atrial septal defect (37%).ConclusionThis study highlights the recognition of the associated congenital heart disease with non-syndromic orofacial cleft patients. Global screening protocols designed for newborns with non-syndromic orofacial cleft are needed to eliminate late diagnosis of critical congenital heart diseases which might present operative risks of anesthesia and/or surgical procedures.     

Epidemiology of Hoffmeister's "genetically determined predisposition to disturbed development of the dentition" in patients with cleft lip and palate.

OBJECTIVE: Type and prevalence rates of the symptoms of Hoffmeister's "genetically determined predisposition to disturbed development of the dentition" were studied in patients with clefts. PATIENTS: Data of 263 patients with nonsyndromic clefts of lip (alveolus and palate) or isolated cleft palates were examined in a retrospective study. SETTING: The clefts were classified as cleft lip or cleft lip and alveolus, cleft palate, unilateral cleft lip and palate, and bilateral cleft lip and palate. All patients were scrutinized for 28 individual symptoms. Prevalences of the individual symptoms were statistically evaluated regarding cleft type and gender by using the chi-square test and were also compared with findings in patients without clefts. RESULTS: In 97.7% of the patients with clefts, at least one symptom was found. Microdontia of individual teeth, hypodontia, and hyperodontia were the symptoms most frequently recorded. Comparison of the different cleft types revealed differences regarding the prevalences of supernumerary lateral incisors (p = .051), infraposition of deciduous molars (p < .001), and atypical tooth bud position (p = .030). Comparison of the prevalences of 10 symptoms recorded in the patients with clefts with the prevalences recorded in patients without clefts showed nine symptoms were found much more frequently in the population with clefts. CONCLUSION: These findings support the hypothesis that clefting is part of a complex malformation associated with other dental anomalies resulting from disturbed development of the dentition. Patients with clefts are also likely to present other deficiencies of dental development and tooth eruption in both dentitions, even in regions not affected by the cleft.     

Cleft lip and cleft palate in Santo Domingo

The purpose of this study was to analyze the occurrence of isolated cleft lip (CL), cleft lip with cleft palate (CL + CP) and isolated cleft palate (CP) and their distribution according to sex and laterality in Santo Domingo, Dominican Republic, located in the Caribbean Archipelago. The sample consisted of 439 hospital records (204 males and 235 females) of patients attending a children's public hospital in Santo Domingo over the period of May 1973 to December 1976. Of all facial clefts, the highest percentage (36.4%) was presented by CL, followed by CP (32.1%) and CL + CP (31.4%). Of all facial clefts, males presented the highest percentage (53.5%). For both sexes, there was an equal number of cases with CL (17.54 %) but more males had CL + CP (0.20 > P0.10) and more females presented CP (P < 0.001). The left-sided defects were almost twice as common as the right-sided defects. The ratio of unilateral clefts-to-bilateral clefts was 5.4:1.     

Prevalence of orofacial clefts in Saudi Arabia and neighboring countries: A systematic review

Cleft lip and/or palate are among the most common birth defects in the world. The prevalence of these conditions varies considerably across geographic areas and ethnic groups.ObjectiveThe aim of this study was to carry out a systematic review and appraisal of the literature on the prevalence of cleft lip and/or palate in Saudi Arabia and comparable Middle Eastern countries.Materials and methodsAll published articles on orofacial clefts (OFC) in Saudi Arabia and its bordering countries in the Middle East with similar and comparable population characteristics were reviewed in July 2010.ResultsAfter reviewing the articles, only eight matched the inclusion criteria. Three studies were carried out in two regions in Saudi Arabia (Riyadh and Al-Qaseem). The other five studies were set in Dubai, Oman, and Jordan. The prevalence of cleft lip and/or palate reported in these studies varied greatly from 0.3 to 2.4 per 1000 live births. The birth prevalence of orofacial clefts in males was reported to be higher than in females. The isolated cleft palate prevalence was reported to be higher in females in most of the studies.ConclusionThe eightfold variation in the prevalence of orofacial clefts between highest and lowest prevalence is likely to be due, at least in part, to problems with ascertainment, but there may also be underlying genetic or environmental factors that require further investigation.     

The Incidence of Cleft Lip and Palate in Northern Ireland from 1980–1990

Abstract

This study reports the incidence of the various types of cleft lip and/or palate drawn from a regional database of all affected children born in Northern Ireland during the period 1980–1990. The incidence of these anomalies was 1·28 per 1000 live births (1:781).

Fifty-three per cent of clefts involved the secondary palate only, 16 per cent the primary palate only, 26 per cent involved both primary and secondary palate, and 5 per cent were unconnected.

Overall, more males than females were affected and there were more males than females in the group having complete clefts. Separate clefts of lip and palate occurred exclusively in males with only one exception.

Unilateral clefts were more common on the left side.

Within the group showing complete unilateral cleft of the primary and secondary palate, left-sided clefts were more commonly male, right-sided clefts were more commonly female.

There were no statistically significant sex differences between sides in the unilateral primary palate cleft group.     

Modified VIP-CT flap in late maxillary alveolar cleft surgery

ObjectiveThis article describes a new application for the modified vascularized interpositional periosteal-connective tissue flap (modified VIP-CT flap) to solve problematic cases of late maxillary alveolar cleft bone grafting.MethodsThis study analysed the ability of the VIP-CT flap to provide coverage of bone grafts in maxillary alveolar cleft surgery. Donor site morbidity was also evaluated.ResultsThirteen patients were operated on using this technique, seven patients were female, the age range was between 12 and 25 years, and all of them were missing at least one permanent anterior tooth adjacent to the cleft. Two patients had bilateral alveolar clefts. In most of the operated patients one VIP-CT flap was used, but in two patients (cases 3 and 4) bilateral VIP-CT flaps were used for treatment of wide alveolar clefts. In case 3 both of the flaps were used for closure of the oral side and in case 4 one flap was used for nasal closure and another for oral side coverage. Two patients had alveolar cleft only with normal palatal anatomy, and the remaining patients had previously operated cleft palates (11 patients).ConclusionVIP-CT flaps can readily be used in adult patients with cleft lip (alveolar only) and in many operated unilateral and bilateral complete cleft lip and palate patients successfully.     

Malposition of unerupted mandibular second premolar in children with cleft lip and palate

OBJECTIVE: To determine whether distoangular malposition of the unerupted mandibular second premolar (MnP2) is more frequent in children with unilateral clefts of the lip and palate. MATERIALS AND METHODS: This retrospective study examined panoramic radiographs from 45 patients with unilateral clefts of the lip and/or palate who had no previous orthodontics. A control sample consisted of age- and sex-matched patients. The distal angle formed between the long axis of MnP2 and the tangent to the inferior border was measured. The mean, standard deviation, and range were calculated for the angles measured in the cleft and the control groups. The significance of the differences between the means was evaluated by the paired t-test. The angles of the cleft and noncleft sides were also measured and compared. RESULTS: The mean inclination of the MnP2 on the cleft side was 73.6 degrees , compared with 84.6 degrees in the control group. This difference was highly significant statistically (P < .0001). The difference in angles from the cleft and noncleft sides was 0.7 degrees , not statistically significant. A significant association was found between clefting and distoangular malposition of the developing MnP2, suggesting a shared genetic etiology. This association is independent of the clefting side, ruling out possible local mechanical effects. CONCLUSION: Clinicians should be aware of the potential for anomalous development of MnP2 in children with clefts.     

1320例唇腭裂患者临床统计分析

目的:了解唇腭裂的最新流行病学情况,寻找与唇腭裂发生相关的可能因素,为唇腭裂病因研究及预防提供临床资料.方法:对我院自2001年1月至2006年12月收治的1320例唇腭裂患者进行回顾性临床统计分析.结果:本组病例中,男性920例(69.70%),女性400例(30.30%);唇裂410(31.06%)、唇裂伴腭裂782例(59.24%)、单纯腭裂128例(9.70%) ;全部病例中合并牙槽突裂895例,占67.80%;单侧唇裂伴或不伴腭裂明显多于双侧者,两者之比为3.26:1,其中左侧多于右侧(1.71:1);有家族遗传史的病例占总病例的4.47%,其中唇裂伴或不伴腭裂有遗传史者占唇裂伴或不伴腭裂病例总数的3.86%,单纯腭裂有遗传史者占单纯腭裂病例总数的10.16%;唇腭裂患者母亲孕3个月经历危险因素者共267例,占唇腭裂总数的20.23%,其中唇裂伴或不伴腭裂患者224例,占唇裂伴或不伴腭裂患者总数的18.79%,单纯腭裂患者43例,占单纯腭裂患者总数的33.59%;所有患者中出生于7～9月者均少于其它其它季度.结论:唇腭裂发病以唇裂合并腭裂居多,且以单侧发病多于双侧,男性发病多于女性发病.多基因遗传和胚胎所处的环境因素是唇腭裂的重要病因.     

Assessment of patients' level of satisfaction with cleft treatment using the Cleft Evaluation Profile.

OBJECTIVES: Determination of the psychosocial status and assessment of the level of satisfaction in Malaysian cleft palate patients and their parents. DESIGN: Cross-sectional study. PARTICIPANTS AND METHODS: Sixty cleft lip and palate patients (12 to 17 years of age) from Hospital Universiti Sains Malaysia and their parents were selected. The questionnaires used were the Child Interview Schedule, the Parents Interview Schedule, and the Cleft Evaluation Profile (CEP), administered via individual interviews. RESULTS: Patients were teased because of their clefts and felt their self-confidence was affected by the cleft condition. They were frequently teased about cleft-related features such as speech, teeth, and lip appearance. Parents also reported that their children were being teased because of their clefts and that their children's self-confidence was affected by the clefts. Both showed a significant level of satisfaction with the treatment provided by the cleft team. There was no significant difference between the responses of the patients and their parents. The features that were found to be most important for the patients and their parents, in decreasing order of priority, were teeth, nose, lips, and speech. CONCLUSIONS: Cleft lip and/or palate patients were teased because of their clefts, and it affected their self-confidence. The Cleft Evaluation Profile is a reliable and useful tool to assess patients' level of satisfaction with treatment received for cleft lip and/or palate and can identify the types of cleft-related features that are most important for the patients.     

Tooth agenesis patterns in bilateral cleft lip and palate

Bartzela TN, Carels CEL, Bronkhorst EM, Rønning E, Rizell S, Kuijpers-Jagtman AM. Tooth agenesis patterns in bilateral cleft lip and palate. Eur J Oral Sci 2010; 118: 47–52. © 2010 The Authors. Journal compilation © 2010 Eur J Oral Sci
Individuals with cleft lip and palate present significantly more dental anomalies, even outside the cleft area, than do individuals without clefts. Our aim was to evaluate the prevalence of tooth agenesis and patterns of hypodontia in a large sample of patients with complete bilateral cleft lip and palate (BCLP). Serial panoramic radiographs (the first radiograph was taken at 10.5–13.5 yr of age) of 240 patients with BCLP (172 male patients, 68 female patients) were examined. Third molars were not included in the evaluation. Agenesis of at least one tooth was present in 59.8% of patients. Upper laterals and upper and lower second premolars were missing most frequently. Using the tooth agenesis code (TAC), 52 different agenesis patterns were identified, of which simultaneous agenesis of 12, 22, 15, 25, 35, and 45 was the most frequent pattern. Nine of the 240 patients showed combined BCLP and oligodontia.     

9720例唇腭裂患者临床资料分析

目的:了解近年来甘肃省先天性唇腭裂的发病情况,探讨与其发病有关的影响因素,为该病的预防与治疗提供依据。方法:收集并统计分析2005-03—2013-03间,在甘肃省人民医院口腔颌面外科进行唇腭裂手术的9 720例患者的临床资料。结果:唇裂3 546例、唇裂伴腭裂3 888例、腭裂2 286例;男女比为1.62∶1;单侧与双侧唇腭裂比为4.44∶1;左侧与右侧比为2.07∶1;农村与城市的患儿比为5.04:1;陇南市患者占总人数的40.78%;母亲文化水平为初中以下的占总人数的85.05%。结论:陇南地区唇腭裂患者占多数;唇裂合并腭裂居多;左侧多于右侧;男性多于女性;农村多于城市。遗传因素和地理环境因素可能是甘肃省内先天性唇腭裂的危险因素。而母亲文化程度的高低与唇腭裂的发生无相关性。