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Fulltext Cleft Lip And Palate Centre: The University Hospital Experience

Ghani, S.H.A., Hussain, R., Hassan, S., Tan, K.K., Ahmad, M.H.

Ann Dent, 1996;3(1):-.
MyJurnal

The Combined Cleft Clinic at the University Hospital, Kuala Lumpur was organised in 1992. The team consists of Plastic Surgeon, Orthodontists, Speech therapist, Ear, Nose and Throat Surgeon, Audiologist, medical officers and the nurses. We attend the clinic on a regular basis. Specialists from other medical and dental disciplines as well as the members of the Cleft Lip and Palate Association of Malaysia (CLAPAM) do occasionally participate in this set-up.The team members formulate treatment plans for each cleft patient, monitor the patient's growth and development and manage the patient at different stages according to the individual needs. To date, the idea of team approach and an establishment of a centre towards management of cleft patients seem to be the most ideal as the patients are benefiting total treatment and care from various specialists from only one place. The experience of University Hospital as a centre for cleft patients is discussed.

Matched MeSH terms: Cleft Lip
Fulltext Orthodontic treatment of a unilateral cleft lip and palate patient with secondary bone graft and eruption of canine

Mathew, Thomas

Malaysian Dental Journal, 2010;31(2):84-89.
MyJurnal

The cleft lip and palate patients often have constricted maxillary arch, congenitally missing teeth, impacted teeth, and many kinds of malocclusion problems. Secondary bone grafting in cleft lip and palate patients is performed preferably before the eruption of permanent canine in order to provide adequate periodontal support for eruption and preservation of the teeth adjacent to the cleft. Secondary grafting with iliac marrow consistently produces trabecular bone to unify the maxilla and provide odontogenic support. Presented here is a case of unilateral cleft lip and palate, which was treated by expansion by quad helix and straight wire appliance for correction of dental malocclusion. Also discussed in detail the entire range of treatment procedures the boy underwent from 14 years of age, especially the role of secondary bone grafting with iliac marrow to facilitate the eruption of permanent impacted canine.

Matched MeSH terms: Cleft Lip
Fulltext Facial profile and maxillary arch dimensions in unilateral cleft lip and palate children in the mixed dentition stage

Gopinath VK, Samsudin AR, Mohd Noor SNF, Mohamed Sharab HY

Eur J Dent, 2017 4 25;11(1):76-82.
PMID: 28435370 DOI: 10.4103/ejd.ejd_238_16

OBJECTIVES: The aim of this study was to evaluate the vertical and sagittal facial profile and maxillary arch width, depth, and length of patients with unilateral cleft lip and palate (UCLP) and to compare them with healthy noncleft children in the mixed dentition stage (7-13 years).
MATERIALS AND METHODS: This study is conducted at Hospital Universiti Sains Malaysia. UCLP group comprised 48 patients with nonsyndromic UCLP who have had the lip and palate repaired, whereas the control group comprised 48 healthy noncleft cases. The lateral cephalometrics measurements were used to determine the vertical height, sagittal depth of the face, and cranial base length and angle. Maxillary arch dimensions were measured on the study cast including arch width, depth, and length.
RESULTS: Vertical facial height and sagittal depth measurements showed a significant decrease (P < 0.05) in the mean growth pattern in UCLP group. The anterior cranial base length (S-N) was shorter in UCLP children (P < 0.001), while Ba-N length had no significant difference (P = 0.639). Nasion-Sella Tursica-Basion angle was significantly higher in the UCLP group (P = 0.016). Dental arch width with reference to canine-to-canine and first premolar-to- first premolar distance was significantly larger in control (P = 0.001).
CONCLUSION: Mean vertical and sagittal facial dimensions in the UCLP children who do not undergo orthodontic treatment are significantly lesser in all directions of growth than healthy noncleft children. The maxillary dental arch had a normal depth but constricted in width and arch length.

Matched MeSH terms: Cleft Lip
Fulltext Three-dimensional facial analysis of Chinese children with repaired unilateral cleft lip and palate

Othman SA, Aidil Koay NA

Sci Rep, 2016 08 10;6:31335.
PMID: 27507713 DOI: 10.1038/srep31335

We analyzed the facial features of Chinese children with repaired unilateral cleft lip and palate (UCLP) and compared them with a normal control group using a three-dimensional (3D) stereophotogrammetry camera. This cross-sectional study examined 3D measurements of the facial surfaces of 20 Chinese children with repaired UCLP and 40 unaffected Chinese children aged 7 to 12 years old, which were captured using the VECTRA 3D five-pod photosystem and analyzed using Mirror software. Twenty-five variables and two ratios were compared between both groups using independent t-test. Intra- and inter-observer reliability was determined using ten randomly selected images and analyzed using intra-class correlation coefficient test (ICC). The level of significance was set at p

Matched MeSH terms: Cleft Lip
'Pai Syndrome' with anterior alveolar polyp: A variant of a rare clinical entity

Debnath KK, Kanapaty Y, Yong DJ, Chandran S, Bakri A

Med J Malaysia, 2019 Jun;74(3):229-230.
PMID: 31256179

'Pai syndrome' (PS) is a rare congenital syndrome. Presented here, a new-born baby-girl who exhibited the characteristic features of having a midline nasal (septal) polyp, an anterior alveolar process polyp, and a pericallosal lipoma associated with corpus callosum dysgenesis of the brain. Both polyps were lined with stratified-squamous epithelium. The overall features were largely consistent with those described by Pai et al., in 1987. A midline cleft-lip (with or without cleft-alveolus) is one of the most common features of the syndrome which was however absent in this case. Instead, an anterior alveolar polyp is present, which is relatively rare.

Matched MeSH terms: Cleft Lip
Effects of Multiple Factors on Treatment Outcome in the Three-Dimensional Maxillary Arch Morphometry of Children With Unilateral Cleft Lip and Palate

Haque S, Khamis MF, Alam MK, Ahmad WMAW

J Craniofac Surg, 2020 May 04.
PMID: 32371703 DOI: 10.1097/SCS.0000000000006464

Studies have claimed that the maxillary arch dimension of unilateral cleft lip and palate (UCLP) patient is significantly smaller than normal patient. Multiple factors (both congenital and post natal treatment factors) are believed to have an impact on the treatment outcome (maxillary arch retardation) of UCLP patient. The aim of this study was to evaluate the effects of multiple factors on the treatment outcome by assessing the maxillary arch dimension of UCLP Children. Eighty-five Malaysian UCLP children were taken who received cheiloplasty and palatoplasty. Laser scanned 3D digital models of UCLP subjects were prepared before any orthodontic treatment and bone grafting at 7.69 ± 2.46 (mean± SD) years of age. Intercanine width (ICW), intermolar width (IMW), and arch depth (AD) measurements of maxillary arch were measured with Mimics software. Multiple linear regression analyses were used to evaluate the association between multiple factors (gender, UCLP type, UCLP side, family history of cleft, family history of class III malocclusion, techniques of cheiloplasty, and techniques of palatoplasty) and maxillary arch dimensions (ICW, IMW, and AD). P value was set at 5%. Significant association was found between 2 techniques of cheiloplasty and ICW (P = 0.001) and also between 2 techniques of palatoplasty and ICW (P = 0.046) of maxilla. No significant association observed in IMW and AD in relation to all other factors. Modified Millard techniques of Cheiloplasty and Bardach technique of palatoplasty had unfavorable effect on the treatment outcome by assessing the maxillary arch dimension (ICW) using laser scanned 3D digital models in Malaysian UCLP children.

Matched MeSH terms: Cleft Lip
Fulltext Common dental anomalies in cleft lip and palate patients

Haque S, Alam MK

Malays J Med Sci, 2015 Mar-Apr;22(2):55-60.
PMID: 26023296 MyJurnal

Cleft lip and palate (CLP) is the most common orofacial congenital malformation in live births. CLP can occur individually or in combination with other congenital deformities. Affected patients experience a number of dental, aesthetic, speech, hearing, and psychological complications and have a higher incidence of severe dental conditions. The purpose of this study is to characterise the different types of dental anomalies that are frequently associated with CLP patients based on a literature survey.

Matched MeSH terms: Cleft Lip
Fulltext Assessment of nutrient intake in cleft lip and palate children after surgical correction

Gopinath VK

Malays J Med Sci, 2013 Oct;20(5):61-6.
PMID: 24643339 MyJurnal

This research aimed to compare the nutrient intake of children with cleft lip and palate (CLP) with that of normal children (without CLP) in the same age groups. The study was conducted at the School of Dental Sciences, Universiti Sains Malaysia, Kelantan, Malaysia. A total of 139 children, of both sexes, from two to six years of age, either with CLP (40 children) or without CLP (99 children), were selected. The CLP and non-CLP children were grouped according to age, which is a key determinant of nutrient intake. Children in subgroup I were between the ages of 2 and 4 years, the group comprising 48 normal children of mean age 39.85 months (SD 7.1), and 20 CLP children of mean age 37.05 months (SD 5.9). Children in subgroup II were those over 4 years and up to 6 years old, and the group comprised 51 normal children, of mean age 64.16 months (SD 7.9), and 20 CLP children of mean age 56.75 months (SD 9.9). A comparative cross-sectional study was conducted to evaluate nutrient intake in subgroups I and II, using a 24 hours diet recall method. The nutrient intake of CLP children was shown to have no significant differences from that of normal children. A comparison of intake per day with Recommended Dietary Allowances (RDA) for Malaysian children showed that the consumption of nutrients was inadequate in both the normal and CLP children, in both age sub-groups.

Matched MeSH terms: Cleft Lip
Postnatal treatment factors affecting craniofacial morphology of unilateral cleft lip and palate (UCLP) patients in a Japanese population

Alam MK, Iida J, Sato Y, Kajii TS

Br J Oral Maxillofac Surg, 2013 Dec;51(8):e205-10.
PMID: 23099108 DOI: 10.1016/j.bjoms.2012.10.001

We have evaluated the craniofacial morphology of Japanese patients with unilateral cleft lip and palate (UCLP) and assessed the various postnatal factors that affect it. Lateral cephalograms of 140 subjects (mean (SD) aged 7 (2) years) with UCLP were taken before orthodontic treatment. Surgeons from Hokkaido University Hospital had done the primary operations. The craniofacial morphology was assessed by angular and linear cephalometric measurements. Cheiloplasty, palatoplasty, and preoperative orthopaedic treatment were chosen as postnatal factors. To compare the assessments of the postnatal factors, we made angular and linear cephalometric measurements for each subject and converted them into Z scores in relation to the mean (SD) of the two variables. Subjects treated by the modified Millard cheiloplasty had larger sella-nasion-point A (SNA) and nasion-point A-pogonion (NA-POG) measurements than subjects treated by the modified Millard with a vomer flap cheiloplasty. Two-stage palatoplasty showed consistently better craniofacial morphology than the other palatoplasty. Subjects who had preoperative orthopaedic treatment with a Hotz plate had significantly larger upper incisor/sella-nasion (U1-SN) measurements than who had no preoperative orthopaedic treatment or an active plate. We conclude that in subjects treated by a modified Millard type of cheiloplasty, a two-stage palatoplasty, and a Hotz plate there were fewer adverse effects on craniofacial morphology.

Matched MeSH terms: Cleft Lip/pathology; Cleft Lip/surgery*
Haplotype diversity in 11 candidate genes across four populations

Beaty TH, Fallin MD, Hetmanski JB, McIntosh I, Chong SS, Ingersoll R, et al.

Genetics, 2005 Sep;171(1):259-67.
PMID: 15965248

Analysis of haplotypes based on multiple single-nucleotide polymorphisms (SNP) is becoming common for both candidate gene and fine-mapping studies. Before embarking on studies of haplotypes from genetically distinct populations, however, it is important to consider variation both in linkage disequilibrium (LD) and in haplotype frequencies within and across populations, as both vary. Such diversity will influence the choice of "tagging" SNPs for candidate gene or whole-genome association studies because some markers will not be polymorphic in all samples and some haplotypes will be poorly represented or completely absent. Here we analyze 11 genes, originally chosen as candidate genes for oral clefts, where multiple markers were genotyped on individuals from four populations. Estimated haplotype frequencies, measures of pairwise LD, and genetic diversity were computed for 135 European-Americans, 57 Chinese-Singaporeans, 45 Malay-Singaporeans, and 46 Indian-Singaporeans. Patterns of pairwise LD were compared across these four populations and haplotype frequencies were used to assess genetic variation. Although these populations are fairly similar in allele frequencies and overall patterns of LD, both haplotype frequencies and genetic diversity varied significantly across populations. Such haplotype diversity has implications for designing studies of association involving samples from genetically distinct populations.

Matched MeSH terms: Cleft Lip/ethnology; Cleft Lip/genetics
Mutation screening of IRF6 among families with non-syndromic oral clefts and identification of two novel variants: review of the literature

Salahshourifar I, Wan Sulaiman WA, Halim AS, Zilfalil BA

Eur J Med Genet, 2012 Jun;55(6-7):389-93.
PMID: 22440537 DOI: 10.1016/j.ejmg.2012.02.006

Non-syndromic oral clefts share the main clinical features of Van der Woude Syndrome (VWS), with the exception of the lower lip pit. Thus, about 15% of VWS cases are indistinguishable from cases with non-syndromic oral clefts. IRF6 mutations are the major cause of VWS; however, variants in this gene show strong association with non-syndromic oral clefts, with a higher increased risk among cases with cleft lip only (CLO). A total of 39 individuals, including 16 patients with CLO and 23 patients with a family history of cleft, were examined for IRF6 mutations in the present study. Seven variants, including five known (c.-75-4 A>; G, c.-73T>; C, c.459G>; T 5, c.820G>; A, and c.1060 + 37C>; T) and two novel (c.-75-23G>; C and c.1380G>; T), were found. Both novel variants were inherited from non-affected parents and we did not find also in the 120 control chromosomes. In silico analysis revealed that both c.1380G>; T and c.-75-23G>; C variants may disrupts a putative exonic splicing enhancer and intronic splicing binding site for SC35, respectively. Taken together, the presence of deleterious IRF6 variants in patients with non-syndromic oral clefts could be most likely an evidence for VWS. While, IRF6 variants could, at best, contribute to clefting as part of a complex inheritance pattern, with both additional genes and environmental factors having a role.

Matched MeSH terms: Cleft Lip/genetics*
De novo interstitial deletion of 1q32.2-q32.3 including the entire IRF6 gene in a patient with oral cleft and other dysmorphic features

Salahshourifar I, Halim AS, Sulaiman WA, Ariffin R, Naili Muhamad Nor N, Zilfalil BA

Cytogenet Genome Res, 2011;134(2):83-7.
PMID: 21447942 DOI: 10.1159/000325541

Microdeletion of the Van der Woude syndrome (VWS) critical region is a relatively rare event, and only a few cases have been reported in the medical literature. The extent of the deletion and the genotype-phenotype correlation are 2 crucial issues.

Matched MeSH terms: Cleft Lip/genetics
Maternal uniparental heterodisomy of chromosome 6 in a boy with an isolated cleft lip and palate

Salahshourifar I, Halim AS, Sulaiman WA, Zilfalil BA

Am J Med Genet A, 2010 Jul;152A(7):1818-21.
PMID: 20583164 DOI: 10.1002/ajmg.a.33526

We describe a chromosome 6 uniparental disomy (UPD6) in a boy, discovered during a screening for the genetic cause of cleft lip and palate. In the medical literature, almost all documented cases of UPD6 are paternal in origin, and only four were maternal. We present here a report of complete maternal chromosome 6 uniparental heterodisomy. Haplotype analysis was performed using highly polymorphic short tandem repeat (STR) markers that span both arms of chromosome 6. Analysis of these markers revealed the presence of two maternal alleles but no paternal allele, indicating an instance of maternal uniparental heterodisomy. Chromosome analysis of peripheral blood lymphocytes confirmed a normal male karyotype. Advanced maternal age at the time of the infant's birth and heterodisomy of markers around the centromere favors a meiosis-I error. No specific phenotype has been reported for maternal UPD6. Therefore, the cleft lip and palate in the present case probably occurred due to other risk factors. This report provides further evidence that maternal UPD6 has no specific clinical consequences and adds to the collective knowledge of this rare chromosomal finding.

Matched MeSH terms: Cleft Lip/genetics*
Unusual clinical presentation of a severely protruding premaxilla in patient with bilateral clefts

Burezq H, Bang RL, George A, Mukhtar A

J Craniofac Surg, 2007 Jul;18(4):971-4.
PMID: 17667698

Prominent premaxilla is one of the problems encountered when dealing with bilateral complete cleft lip and palate patients. Secondary alveolar bone grafting with these patients would achieve filling of the osseous defect, supports the alar base, eliminate the oro-nasal fistula and enhance the maxillary instability. This article describes the management of a bilateral cleft lip and palate patient with an extremely protruding premaxilla done in one stage surgery. To our knowledge, this is the first report of such degree of severity in the English literature.

Matched MeSH terms: Cleft Lip/surgery*
Modified Activation Technique for Nasal Stent of Nasoalveolar Molding Appliance for Columellar Lengthening in Bilateral Cleft Lip/Palate

Patil PG, Nimbalkar-Patil SP

J Prosthodont, 2018 Jan;27(1):94-97.
PMID: 27002917 DOI: 10.1111/jopr.12464

Bilateral cleft lip/cleft palate is associated with nasal deformities typified by a short columella. The presurgical nasoalveolar molding (NAM) therapy approach includes reduction of the size of the intraoral alveolar cleft as well as positioning of the surrounding deformed soft tissues and cartilages. In a bilateral cleft patient, NAM, along with columellar elongation, eliminates the need for columellar lengthening surgery. Thus the frequent surgical intervention to achieve the desired esthetic results can be avoided. This article proposes a modified activation technique of the nasal stent for a NAM appliance for columellar lengthening in bilateral cleft lip/palate patients. The design highlights relining of the columellar portion of the nasal stent and the wire-bending of the nasal stent to achieve desirable results within the limited span of plasticity of the nasal cartilages. With this technique the vertical taping of the premaxilla to the oral plate can be avoided.

Matched MeSH terms: Cleft Lip/surgery*
Fulltext Ectrodactary, ectodermal dysplasia, and cleft lip-palate syndrome

Choong YY, Norazlina B

Med J Malaysia, 2001 Mar;56(1):88-91.
PMID: 11503303

The EEC syndrome (ectrodactyly, ectodermal dysplasia and cleft lip-palate) is a rare disorder inherited as an autosomal dominant trait or can occur sporadically. We describe a case of this syndrome with chronic ocular surface disorder secondary to abnormalities of ocular adnexia.

Matched MeSH terms: Cleft Lip/complications*
A preliminary report on one stage open tip rhinoplasty at the time of lip repair in bilateral cleft lip and palate: the Alor Setar experience

Trott JA, Mohan N

Br J Plast Surg, 1993 Apr;46(3):215-22.
PMID: 8490700

This paper is a preliminary report on a strategy to perform open tip rhinoplasty at the time of lip repair in bilateral cleft lip and palate deformity. This method was devised to suit unique socio-economic circumstances in the Malaysian centre of Alor Setar. Of 8 cases having surgery in 1991, 7 returned for follow-up with results being documented photographically. It is concluded that this aggressive approach is justified in severe bilateral cleft lip and palate deformity because of the cost effectiveness of limiting the number of interventions. Furthermore, it provides optimally orientated nasal tip anatomy and reduces the social stigma of cleft lip nose appearance from the earliest possible time in the child's development.

Matched MeSH terms: Cleft Lip/surgery*
Fulltext EEC syndrome with urogenital anomalies and compound naevus

Omar I, Jidon AJ

Med J Malaysia, 1993 Sep;48(3):364-8.
PMID: 8183155
Matched MeSH terms: Cleft Lip/therapy*
Congenital cleft lip and palate in Singapore

Fong PH, Yeap CL, Lee ST

Ann Acad Med Singap, 1983 Apr;12(2 Suppl):363-5.
PMID: 6625521

Cleft lip and palate is the most common congenital abnormality seen in the Department of Plastic Surgery, Singapore General Hospital. A total of 461 operative cases seen over a period of 5 years (1977 to 1981) is analysed. There is a relatively high incidence in the Singapore population of 2.0 per 1000 live births. In both cleft lip and cleft palate deformities, there is no significant difference in the sex distribution. The cleft patterns show that 78% of the cases are unilateral clefts, 53% are left sided, 25% are right sided and 22% of the cases are bilateral clefts.

Matched MeSH terms: Cleft Lip/epidemiology*
A preliminary report on open tip rhinoplasty at the time of lip repair in unilateral cleft lip and palate: the Alor Setar experience

Trott JA, Mohan N

Br J Plast Surg, 1993 Jul;46(5):363-70.
PMID: 8369872

A method of open-tip rhinoplasty using nostril and columella rim incisions at the time of unilateral cleft lip repair is described. This preliminary report details experience gained using this technique in the Malaysian centre of Alor Setar following its introduction in September, 1991.

Matched MeSH terms: Cleft Lip/surgery*

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