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Sample records for cleft lip-palate patients

  1. Management of feeding Problem in a Patient with Cleft Lip/Palate.

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    Goswami, Mridula; Jangra, Babita; Bhushan, Urvashi

    2016-01-01

    In a child with cleft lip and/or palate, nutrition is the first priority as for any other child. These children have specific physical limitations. To fulfill their nutritional requirement, these children need modifications in order to thrive and grow. Failure to adjust to these needs could place the children into a potential life-threatening situation. One of the immediate problems to be addressed in a newborn with cleft lip/palate is difficulty in feeding. Nasal regurgitation and choking are common because of inability of the palate to separate the nasal and oral cavities. The case presented here discusses the management of feeding problem in the infant with cleft lip/palate. How to cite this article: Goswami M, Jangra B, Bhushan U. Management of feeding Problem in a Patient with Cleft Lip/ Palate. Int J Clin Pediatr Dent 2016;9(2):143-145. PMID:27365936

  2. Management of feeding Problem in a Patient with Cleft Lip/Palate

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    Goswami, Mridula; Bhushan, Urvashi

    2016-01-01

    ABSTRACT In a child with cleft lip and/or palate, nutrition is the first priority as for any other child. These children have specific physical limitations. To fulfill their nutritional requirement, these children need modifications in order to thrive and grow. Failure to adjust to these needs could place the children into a potential life-threatening situation. One of the immediate problems to be addressed in a newborn with cleft lip/palate is difficulty in feeding. Nasal regurgitation and choking are common because of inability of the palate to separate the nasal and oral cavities. The case presented here discusses the management of feeding problem in the infant with cleft lip/palate. How to cite this article: Goswami M, Jangra B, Bhushan U. Management of feeding Problem in a Patient with Cleft Lip/ Palate. Int J Clin Pediatr Dent 2016;9(2):143-145. PMID:27365936

  3. The status of oral hygiene in cleft lip, palate patients after surgical correction

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    Pandey S

    2005-01-01

    Full Text Available The cleft lip and palate patients usually present a number of problems viz. altered oral anatomy leading to changes in oral physiology diminishing the self-cleansing ability of individual. The handicapped children are unable to maintain their oral hygiene properly. The present study was formulated with the aim that does normalization of oral anatomy have its effect on improvement of oral hygiene? An assessment of oral hygiene index-simplified was performed between preoperative and postoperative values in the same patient at KGMU and KGDU. A total of 50 cases were recorded in two groups of 25 each: (i < 6 years old and (ii > 6 years. The observations are statistically analyzed by paired ′t′ test to get the significance of results. Results: The data analyzed showed the significant decrease in oral hygiene indices observed in both groups. A relative significance in oral hygiene status following surgery was observed. Both groups expressed greater significance when compared pre and postoperatively which is indicative of considerable improvement of oral hygiene after surgical correction. The study concludes that oral hygiene improves more in older cleft lip-palate cases following reconstruction of palatal vault, premaxilla and anterior lip seal by secondary bone grafting method when compared with oral hygiene indices results in primary periosteoplasty cases. The surgical correction of cleft lip palate enhances self-cleaning ability and better compliance to maintain oral hygiene in children as the age advances.

  4. Comparison of oral hygiene and periodontal status in patients with clefts of palate and patients with unilateral cleft lip, palate and alveolus

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    Mutthineni Ramesh

    2010-01-01

    Full Text Available Aim: This study was conducted to analyze and compare the oral hygiene and periodontal status in patients with clefts of palate (CP and patients with unilateral cleft lip, palate and alveolus (UCLPA. Materials and Methods: The study group consisted of 120 cleft patients. Subjects were divided into two groups of 60 each. Group I - patients with UCLPA and Group II - patients with CP. For comparison, all the four quadrants were defined, Q1-right upper quadrant, Q2-left upper quadrant, Q3-left lower quadrant and Q4-right lower quadrant, in both groups and the following parameters were recorded: Plaque Index (PI, Silness and Loe, Sulcus Bleeding Index (SBI, Muhlemann and Son, Probing Pocket Depth (PPD, Clinical Attachment Level (CAL, Mobility Index (Miller and Radiographic Amount of Bone Loss. Results: The periodontal destruction was seen to be higher in UCLPA patients compared with CP patients. The poor oral hygiene status, as indicated by higher values of PI, and the periodontal status, evaluated by SBI, PPD, CAL, mobility and Radiographic Amount of Bone Loss, were higher in patients with UCLPA than in patients with CP. Conclusion: In this study, patients with cleft lip, palate and alveolus had poor oral hygiene and periodontal status compared with patients with cleft palate.

  5. Comparative Evaluation of Prevalence of Upper Cervical Vertebrae Anomalies in Cleft Lip/Palate Patients: A Retrospective Study

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    Datana, Sanjeev; Kumar, Prasanna; Kumar Roy, Supriya; Londhe, Sanjay

    2014-01-01

    ABSTRACT% Purpose: The patients with cleft lip and palate have a higher risk of cervical vertebrae anomalies than do patients in general population. The aim of present study was to determine the prevalence of various upper cervical spine anomalies in different type of clefts. Procedures: Lateral cephalograms of 128 patients (66 males, 62 females) with cleft lip and palate, and 125 (60 males, 65 females) non syndromic patients without cleft lip and palate were selected at random from archive. Cephalograms of the patients were traced and the diagnosis of any cervical vertebrae anomaly was noted. Anomalies were categorized as either: posterior arch deficiency or fusions. Main findings: Prevalence of cervical vertebrae anomalies in the c lef t group was 20. 3% while it was 6.4% in the control group. Further cervical vertebrae anomalies were 16.6% in the CPO group, 19.1% in the BCLP group, and 22.2% in the UCLP group. Conclusion: A higher prevalence of cervical vertebrae anomalies was observed in cleft lip and palate patients. The prevalenc e obser ved is 3 times more in clef t group than c ontrol group. How to cite this article: Datana S, Bhalla A, Kumar P, Roy SK, Londhe S. Comparative Evaluation of Prevalence of Upper Cervical Vertebrae Anomalies in Cleft Lip/Palate Patients: A Retrospective Study. Int J Clin Pediatr Dent 2014;7(3):168-171. PMID:25709295

  6. The status of oral hygiene in cleft lip, palate patients after surgical correction

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    Pandey S; Pandey R

    2005-01-01

    The cleft lip and palate patients usually present a number of problems viz. altered oral anatomy leading to changes in oral physiology diminishing the self-cleansing ability of individual. The handicapped children are unable to maintain their oral hygiene properly. The present study was formulated with the aim that does normalization of oral anatomy have its effect on improvement of oral hygiene? An assessment of oral hygiene index-simplified was performed between preoperative and postoperati...

  7. Computer-Assisted Orthognathic Surgery for Patients with Cleft Lip/Palate: From Traditional Planning to Three-Dimensional Surgical Simulation

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    Lonic, Daniel; Pai, Betty Chien-Jung; Yamaguchi, Kazuaki; Chortrakarnkij, Peerasak; Lin, Hsiu-Hsia; Lo, Lun-Jou

    2016-01-01

    Background Although conventional two-dimensional (2D) methods for orthognathic surgery planning are still popular, the use of three-dimensional (3D) simulation is steadily increasing. In facial asymmetry cases such as in cleft lip/palate patients, the additional information can dramatically improve planning accuracy and outcome. The purpose of this study is to investigate which parameters are changed most frequently in transferring a traditional 2D plan to 3D simulation, and what planning parameters can be better adjusted by this method. Patients and Methods This prospective study enrolled 30 consecutive patients with cleft lip and/or cleft palate (mean age 18.6±2.9 years, range 15 to 32 years). All patients received two-jaw single-splint orthognathic surgery. 2D orthodontic surgery plans were transferred into a 3D setting. Severe bony collisions in the ramus area after 2D plan transfer were noted. The position of the maxillo-mandibular complex was evaluated and eventually adjusted. Position changes of roll, midline, pitch, yaw, genioplasty and their frequency within the patient group were recorded as an alternation of the initial 2D plan. Patients were divided in groups of no change from the original 2D plan and changes in one, two, three and four of the aforementioned parameters as well as subgroups of unilateral, bilateral cleft lip/palate and isolated cleft palate cases. Postoperative OQLQ scores were obtained for 20 patients who finished orthodontic treatment. Results 83.3% of 2D plans were modified, mostly concerning yaw (63.3%) and midline (36.7%) adjustments. Yaw adjustments had the highest mean values in total and in all subgroups. Severe bony collisions as a result of 2D planning were seen in 46.7% of patients. Possible asymmetry was regularly foreseen and corrected in the 3D simulation. Conclusion Based on our findings, 3D simulation renders important information for accurate planning in complex cleft lip/palate cases involving facial asymmetry that is

  8. Computer-Assisted Orthognathic Surgery for Patients with Cleft Lip/Palate: From Traditional Planning to Three-Dimensional Surgical Simulation.

    Directory of Open Access Journals (Sweden)

    Daniel Lonic

    Full Text Available Although conventional two-dimensional (2D methods for orthognathic surgery planning are still popular, the use of three-dimensional (3D simulation is steadily increasing. In facial asymmetry cases such as in cleft lip/palate patients, the additional information can dramatically improve planning accuracy and outcome. The purpose of this study is to investigate which parameters are changed most frequently in transferring a traditional 2D plan to 3D simulation, and what planning parameters can be better adjusted by this method.This prospective study enrolled 30 consecutive patients with cleft lip and/or cleft palate (mean age 18.6±2.9 years, range 15 to 32 years. All patients received two-jaw single-splint orthognathic surgery. 2D orthodontic surgery plans were transferred into a 3D setting. Severe bony collisions in the ramus area after 2D plan transfer were noted. The position of the maxillo-mandibular complex was evaluated and eventually adjusted. Position changes of roll, midline, pitch, yaw, genioplasty and their frequency within the patient group were recorded as an alternation of the initial 2D plan. Patients were divided in groups of no change from the original 2D plan and changes in one, two, three and four of the aforementioned parameters as well as subgroups of unilateral, bilateral cleft lip/palate and isolated cleft palate cases. Postoperative OQLQ scores were obtained for 20 patients who finished orthodontic treatment.83.3% of 2D plans were modified, mostly concerning yaw (63.3% and midline (36.7% adjustments. Yaw adjustments had the highest mean values in total and in all subgroups. Severe bony collisions as a result of 2D planning were seen in 46.7% of patients. Possible asymmetry was regularly foreseen and corrected in the 3D simulation.Based on our findings, 3D simulation renders important information for accurate planning in complex cleft lip/palate cases involving facial asymmetry that is regularly missed in conventional 2D

  9. [Ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome, report of a case with variable expressivity].

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    Meza Escobar, Luis Enrique; Isaza, Carolina; Pachajoa, Harry

    2012-10-01

    The ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome is a rare entity associated with mutations in the genes that express the protein p63. We present a case of a patient with right foot ectrodactyly associated with cleft lip and palate, without other evident anomalies. The patient has a positive familiar history for cleft lift and palate and mortality during the perinatal period. The management of each case must be specific and multidisciplinary. PMID:23070194

  10. Craniofacial and anthropometric phenotype in ankyloblepharon-ectodermal defects-cleft lip/palate syndrome (Hay-Wells syndrome) in a cohort of 17 patients.

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    Sutton, V Reid; Plunkett, Katie; Dang, Diane X; Lewis, Richard A; Bree, Alanna F; Bacino, Carlos A

    2009-09-01

    Ankyloblepharon-ectodermal dysplasia-cleft lip/palate (AEC) syndrome and Rapp-Hodgkin syndrome are well-characterized clinical entities caused by mutations in the TP63 gene. While AEC and Rapp-Hodgkin had been thought to be clinically distinct entities, the elucidation of their molecular etiology confirmed that they are a clinical continuum as opposed to distinct disorders. We have evaluated 17 patients with AEC syndrome using a systematic clinical approach. In our study, we have identified new features and others that were thought to occur only rarely. These include short stature and poor weight gain with preservation of head circumference in nearly all subjects, trismus in 35% and hypospadias in 78% of males. In addition, we describe the frequency of phenotypic features and demonstrate the extreme clinical variability in the largest cohort of AEC individuals reported in the literature thus far.

  11. Feeling Normal? Long-Term Follow-up of Patients with a Cleft Lip-Palate after Rhinoplasty with the Derriford Appearance Scale (DAS-59).

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    Albers, Andreas E; Reichelt, Andreas C; Nolst-Trenité, Gilbert J; Menger, Dirk Jan

    2016-04-01

    The stigma of nasal deformity due to a congenital cleft lip-palate has an undeniable influence on the affected patient's life. It is therefore of interest to investigate if efforts to reduce esthetic and functional impairments by rhinoplasty (single or multiple) can result in an increased satisfaction with appearance and a self-perception similar to the noncleft population. Retrospective scoring before and after rhinoplasty using the validated Derriford Appearance Scale (DAS-59) and subsequent statistical evaluation and comparison to datasets available in the literature for further classification was used. Of the 61 patients who underwent at least one rhinoplasty, 26 responded to all questions. The mean age of responders was approximately 30 years of age and the male:female ratio was 1:1.2. The scale showed a significant overall improvement after surgery. The full scale and all subscale scores of the DAS-59 were significantly reduced after surgery demonstrating an improvement in the respective categories. Most importantly, if postoperative results were compared with a population concerned and unconcerned about appearance, no difference "facial self-consciousness" of appearance was apparent. Also postoperative subscores for "general self-consciousness" (GSC) and "social self-consciousness" of appearance (SSC) showed no difference from those obtained from the population concerned about appearance. The postoperative subscore for "sexual and bodily self-consciousness" of appearance (SBSC) indicated improvement beyond the level found in the concerned control population. Due to only a low improvement in the difference compared with the subscore representing a "negative self-concept," a statistically significant difference to the concerned population remained, possibly indicating that therapy beyond surgery is needed for improvement. After rhinoplasty, the investigated group of cleft lip-palate patients with nasal deformities showed an improvement in their self

  12. Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

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    P K Shivaprakash; Joshi, Hrishikesh V.; Hina Noorani; Venugopal Reddy

    2012-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) is a rare syndrome having ectrodactyly, ectodermal dysplasia, and cleft lip/palate. So far, very few cases have been reported in literature. However, we report a case of incomplete EEC syndrome having ectrodactyly and cleft lip and palate with absence of signs of ectodermal dysplasia with no other systemic anomalies. Other feature noted is the syndactyly of toes which is reported rarely in this syndrome. A multidisciplinary approa...

  13. Ectrodactyly, Ectodermal dysplasia, and Cleft Lip-Palate Syndrome; Its Association with Conductive Hearing Loss

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    Robinson, Geoffrey C.; And Others

    1973-01-01

    Conductive hearing loss associated with the ectrodactyly, ectodermal dysplasia, and cleft lip palate syndrome was reported in one sporadic case and in a pedigree with four cases in three generations. (GW)

  14. Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

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    P K Shivaprakash

    2012-01-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC is a rare syndrome having ectrodactyly, ectodermal dysplasia, and cleft lip/palate. So far, very few cases have been reported in literature. However, we report a case of incomplete EEC syndrome having ectrodactyly and cleft lip and palate with absence of signs of ectodermal dysplasia with no other systemic anomalies. Other feature noted is the syndactyly of toes which is reported rarely in this syndrome. A multidisciplinary approach for treatment is needed which is co-ordinated by pedodontist or pediatrician.

  15. Cleft Lip/Palate, Short Stature, and Developmental Delay in a Boy with a 5.6-Mb Interstitial Deletion Involving 10p15.3p14

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    Gamba, Bruno F.; Rosenberg, Carla; Costa, Silvia; Richieri-Costa, Antonio; Ribeiro-Bicudo, Lucilene A.

    2015-01-01

    The chromosome interval 10p15.3p14 harbors about a dozen genes. This region has been implicated in a few well-known human phenotypes, namely HDR syndrome (hypoparathyroidism, sensorineural deafness, and renal dysplasia) and DGS2 (DiGeorge syndrome 2), but a number of variable phenotypes have also been reported. Cleft lip/palate seems to be a very unusual finding within the clinical spectrum of patients with this deletion. Here, we report a male child born with short stature, cleft lip/palate, and feeding problems who was found to have a 5.6-Mb deletion at 10p15.3p14. PMID:25852446

  16. Effect of cleft lip palate repair on craniofacial growth

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    Naqvi, Zuber Ahamed; Shivalinga, BM; Ravi, S; Munawwar, Syeda Sarah

    2015-01-01

    Objective: The aim of this cross-sectional study was to compare craniofacial growth among operated and unoperated unilateral cleft lip and palate non-syndromic subjects. Materials and Methods: A sample of 180 subjects of Indian origin was selected. Of them, 90 were operated, and 90 were unoperated complete unilateral cleft lip and palate individuals. The subjects were divided into three age groups of 3–5, 8–10, and 20–25 years comprised of 30 patients in each group. The following measurements were evaluated: Angle and length of the cranial base; maxillary spatial positioning and length; mandibular spatial positioning; morphology and length; maxillomandibular relationship. Comparative analysis of the means between the groups was performed with Student's t-test at the significance levels of 5%. The ANOVA test has been performed to test the effect of time. Results: No significant differences were observed between the measurements that represented the angle and length of the cranial base of unoperated and the operated patients (P>0.05). There was statistically significant decrease (P˂0.05) in the maxillary length (Co-A; 69.00 mm in 3–5 years, 68.33 mm in 8–10 years, and 67.17 mm in 20–25 years age group), and SNA angle (74.83° in 3–5 years, 74.17 ° in 8–10 years and 73.17 ° in 20–25 years age group) in operated group. No significant difference noticed on cephalometric values of the mandible, except Ar-Go-Me angle (P˂0.05), which showed vertical growth pattern in unoperated patients (132.50 ° in 3–5 years, 132.00 ° I 8–10 years and 138.33 ° in 20–25 years age group). Conclusion: Lip and palate repair has a significant influence on the maxilla and resulting in retarded growth of maxilla, which causes midface deficiency beyond acceptable sagittal limits. The Gonial angle showed vertical growth pattern in unoperated patients, but the cranial base angle and length of unoperated and the operated patients were similar. PMID:26229945

  17. Interstitial 9q34.11-q34.13 deletion in a patient with severe intellectual disability, hydrocephalus, and cleft lip/palate.

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    Tzschach, Andreas; Grasshoff, Ute; Schäferhoff, Karin; Bonin, Michael; Dufke, Andreas; Wolff, Markus; Haas-Lude, Karin; Bevot, Andrea; Riess, Olaf

    2012-07-01

    Interstitial deletions of chromosome bands 9q34.11-q34.13 are rare. We report on a 16-year-old female patient with severe intellectual disability, congenital hydrocephalus, cleft lip and palate, talipes equinovarus, epilepsy, kyphoscoliosis, convergent strabismus, severe short stature, dystrophy, and facial dysmorphic signs. Array analysis revealed a 3.7 Mb interstitial deletion in 9q34.11-q34.13. The deletion harbors more than 60 genes, including SPTAN1, DYT1/TOR1A, ABL1, ASS1, LAMC3, POMT1, DOLK, and GLE1, mutations in which have previously been associated with monogenic disorders. This is the first patient with a deletion of this size and position in 9q34.11-q34.13. Reports of additional patients with aberrations in this region will be needed to establish karyotype-phenotype correlations and to gain information on the contribution of individual genes for the clinical manifestations. PMID:22639460

  18. Novel FGFR1 and KISS1R Mutations in Chinese Kallmann Syndrome Males with Cleft Lip/Palate

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    Xu, Hao; Niu, Yonghua; Wang, Tao; Liu, Simin; Xu, Hua; Wang, Shaogang; Liu, Jihong; Ye, Zhangqun

    2015-01-01

    Kallmann syndrome (KS) is characterized by isolated hypogonadotropic hypogonadism (IHH) with anosmia and is sometimes associated with cleft lip/palate (CLP). In order to describe the clinical features, genetic etiology, and treatment outcome of KS males with CLP, we performed genetic screening for 15 known causal IHH genes (KAL1, FGFR1, NELF, FGF8, CHD7, WDR11, SEMA3A, KISS1R, KISS1, PROKR2, PROK2, TAC3, TACR3, GNRH1, and GNRHR) in four KS with CLP patients and six IHH patients without CLP. Two novel heterozygous missense mutations in FGFR1, (NM_001174066): c.776G>A (p.G259E) and (NM_001174066): c.358C>T (p.R120C), were identified in a 23-year-old KS male with cleft lip and an 18-year-old KS patient with cleft lip and palate, dental agenesis, and high arched palate, respectively. These two mutations were not presented in their healthy parents and 200 normal controls. One novel heterozygous missense mutation in KISS1R, (NM_032551): c.587C>A (p.P196H), was identified in an 18-year-old KS male with cleft lip and dental agenesis who developed sperm after being treated with gonadotropin. This mutation was also presented in his healthy father and grandfather. These results have implications for the diagnosis, genetic counseling, and treatment of KS and CLP males with mutations in FGFR1 gene. PMID:26199944

  19. Risk variants in BMP4 promoters for nonsyndromic cleft lip/palate in a Chilean population

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    Suazo José

    2011-12-01

    Full Text Available Abstract Background Bone morphogenetic protein 4 gene (BMP4 plays a key role during maxillofacial development, since orofacial clefts are observed in animals when this gene is conditionally inactivated. We recently reported the existence of association between nonsyndromic cleft lip/palate (NSCLP and BMP4 polymorphisms by detecting transmission deviations for haplotypes that include a region containing a BMP4 promoter in case-parent trios. The aim of the present study was to search for possible causal mutations within BMP4 promoters (BMP4.1 and BMP4.2. Methods We analyzed the sequence of BMP4.1 and BMP4.2 in 167 Chilean NSCLP cases and 336 controls. Results We detected three novel variants in BMP4.1 (c.-5514G > A, c.-5365C > T and c.-5049C > T which could be considered as cleft risk factors due to their absence in controls. Additionally, rs2855530 G allele (BMP4.2 carriers showed an increased risk for NSCLP restricted to males (OR = 1.52; 95% C.I. = 1.07-2.15; p = 0.019. For this same SNP the dominant genotype model showed a higher frequency of G/G+G/C and a lower frequency of C/C in cases than controls in the total sample (p = 0.03 and in the male sample (p = 0.003. Bioinformatic prediction analysis showed that all the risk variants detected in this study could create new transcription factor binding motifs. Conclusions The sex-dependent association between rs2855530 and NSCLP could indirectly be related to the differential gene expression observed between sexes in animal models. We concluded that risk variants detected herein could potentially alter BMP4 promoter activity in NSCLP. Further functional and developmental studies are necessary to support this hypothesis.

  20. Comparison of videonasoendoscopy and auditory-perceptual evaluation of speech in individuals with cleft lip/palate

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    Paniagua, Lauren Medeiros

    2014-01-01

    Full Text Available Introduction: The velopharyngeal sphincter (VPS is a muscle belt located between the oropharynx and the nasopharynx. Investigations of velopharyngeal function should include an auditory-perceptual evaluation and at least 1 instrument-based evaluation such as videonasoendoscopy. Aim:To compare the findings of auditory-perceptual evaluation (hypernasality and videonasoendoscopy (gap size in individuals with cleft lip/palate. Method: This was a retrospective, cross-sectional study assessing 49 subjects, of both sexes, with cleft lip/palate followed up at the Otorhinolaryngology Service and the Speech Therapy outpatient clinic of Hospital de Clínicas de Porto Alegre (HCPA. The results from the auditory-perceptual evaluation and the videonasoendoscopy test were compared with respect to the VPS gap size. Results: Subjects with moderate/severe hypernasality had more severe velopharyngeal closure impairment than those with a less severe condition. The interaction between hypernasality severity and the presence of other speech disorders (p = 0.035, whether compensatory and/or obligatory, increased the likelihood of having a moderate-to-large gap in the velopharyngeal closure. Conclusions: We observed an association between the findings of these 2 evaluation methods.

  1. Speech Analysis of Bengali Speaking Children with Repaired Cleft Lip & Palate

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    Chakrabarty, Madhushree; Kumar, Suman; Chatterjee, Indranil; Maheshwari, Neha

    2012-01-01

    The present study aims at analyzing speech samples of four Bengali speaking children with repaired cleft palates with a view to differentiate between the misarticulations arising out of a deficit in linguistic skills and structural or motoric limitations. Spontaneous speech samples were collected and subjected to a number of linguistic analyses…

  2. Polymorphisms in genes MTHFR, MTR and MTRR are not risk factors for cleft lip/palate in South Brazil

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    A.P.C. Brandalize

    2007-06-01

    Full Text Available Non-syndromic cleft lip and palate (CL/P occurs due to interaction between genetic and environmental factors. Abnormalities in homocysteine metabolism may play a role in its etiology due to polymorphisms in genes involved in this pathway. Because of the involvement of MTHFR, MTR and MTRR genes with folate metabolism and the evidence that maternal use of folic acid in early pregnancy reduces the risk for CL/P, we evaluated the influence of their polymorphisms on the etiology of CL/P through a case-control study. The analyses involved 114 non-syndromic phenotypically white children with clefts (case and 110 mothers, and 100 non-affected (control children and their mothers. The polymorphisms 677C>T of MTHFR, 2756A>G of MTR, and 66A>G of MTRR genes were analyzed by PCR-RFLP. Allelic frequencies did not differ from other studies conducted on white populations for MTHFR 677T allele (0.35 and for MTR 2756G allele (0.17, but MTRR 66G allele frequency (0.35 was lower than observed elsewhere. The genotypic distribution of the 677C>T polymorphisms under study did not show significant differences between CL/P patients, their mothers and controls. These results suggest that the alterations of folate metabolism related to these polymorphisms are not involved in clefting in the population under study.

  3. Case report: unusual dental morphology in a child with ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome.

    LENUS (Irish Health Repository)

    Fitzgerald, K

    2012-02-01

    BACKGROUND: Anomalies of dental anatomy are common in the ectodermal dysplasia syndromes. These anomalies, when found in combination with dental caries, can pose a restorative challenge for the paediatric dentist. Modification of traditional techniques and approaches may help the practitioner provide a successful treatment outcome. CASE REPORT: A 3 years and 11 months old girl with a diagnosis of ankyloblepharon-ectodermal dysplasiacleft lip\\/palate (AEC) syndrome was referred for treatment to a specialist paediatric dental service. Her abnormal dental anatomy, hypodontia and dental caries formed a triad of challenges for the team. Under general anaesthesia, her dentition was restored using a combination of restorative approaches and techniques, including the placement of both composite resin and preformed metal crown restorations. FOLLOW-UP: At 18-month followup, the family had successfully implemented good home care and dietary practices, and the local dental service had instituted a preventive programme consisting of regular examination, advice and fluoride varnish placement. The restorations remained intact and no further caries was detected. At 24-month follow-up, the first permanent molars were partially erupted, and displayed unusually deep fissures. There was also a degree of ectopic eruption of the first permanent molars, and possibly of one of the maxillary permanent incisors. CONCLUSION: Dental care for children with AEC syndrome is optimised by early intervention, good home care and regular professional review. Dental care providers should be aware of the possibility of complex dental anatomy, and bear this in mind should it become necessary to formulate a restorative treatment plan.

  4. A Comparative Study of Facial Asymmetry in Philippine, Colombian, and Ethiopian Families with Nonsyndromic Cleft Lip Palate

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    Liliana Otero

    2012-01-01

    Full Text Available Objective. To compare the asymmetry displayed by Philippine, Colombian, and Ethiopian unaffected parents of patients with nonsyndromic cleft palate (NSCLP and a control population. Methods. Facial measurements were compared between unaffected parents of NSCLP patients and those in the control group for three populations from South America, Asia, and Africa by anthropometric and photographic measurements. Fluctuating and directional asymmetries, height and width proportions, were analyzed and compared. Results. Fluctuating asymmetries (ear length, middle line to Zigion perpendicular for left and right sides and variations in the facial thirds demonstrated statistical significance in the study group of unaffected parents from Colombia and Philippines, while increased interorbital distance was evident in the unaffected Ethiopian parents of NSCLP patients. Conclusions. The facial differences in unaffected parents could indicate an underlying genetic liability. Identification of these differences has relevance in the understanding of the etiology of NSCLP.

  5. The association between dental arch dimensions and occurrence of Finnish dental consonant misarticulations in cleft lip/palate children.

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    Laitinen, J; Ranta, R; Pulkkinen, J; Haapanen, M L

    1998-10-01

    The aim of this study was to examine whether maxillary and mandibular dental arch width, length, and palatal height dimensions are associated with the occurrence of misarticulations (phonetic or phonologic errors) in the dental consonants /r/, /s/, and /1/ in different cleft types and sexes. The subjects were 263 (109 girls, 154 boys) 6-year-old Finnish-speaking non-syndromic children with isolated cleft palate (CP, n=79), deft lip/alveolus (CL(A), n=77), unilateral (UCLP, n=80), and bilateral (BCLP, n=27) cleft lip and palate. Dental plaster casts were measured by two authors using the technique of Moorrees, and auditive speech was analyzed with high reliability by two speech pathologists. The results showed that the occurrence of misarticulations increased and dental arch dimensions decreased with the severity of the cleft. Narrower and shorter maxillary arches as well as shallower palates were related to problems with the studied dental consonants. Mandibular arch dimensions were not related to the misarticulations. However, statistical analysis did not reveal significant differences in dental arch dimensions between subjects with and without misarticulations when they were compared separately for different cleft types. The etiology of clefting per se--isolated deft palate versus cleft lip with or without deft palate--did not seem to explain the associations between dental arch dimensions and the studied misarticulations.

  6. Oral health in 4-6 years children with cleft lip/palate: A case control study

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    Amandeep Chopra

    2014-01-01

    Full Text Available Background: Oro-facial clefts are a major public health problem. Children with clefts rarely escape dental complications. Aims: This study was to determine differences in the dental caries experience, gingival health, and prevalence malocclusion, enamel defects and oral mucosal lesions among 4-6 year old children with and without cleft in Panchkula. Materials and Methods: The sampling frame consisted of 4-6 year old children with clefts visiting Swami Devi Dyal Hospital and Dental College, Panchkula, India. As a control group an age (±3 months and gender-matched sample from the same geographical areas were recruited. Dental caries status, gingival health status, developmental defect of enamel, malocclusion and oral mucosal health were assessed and compared between the two groups. Results: Significant differences in dental caries and gingival health status were found between children with and without cleft. Anterior open-bite, increased overjet and oral mucosal lesions (P 0.05. Conclusion: Differences of oral health status exist among 4-6 year old children with and without clefts. Children fare worse in terms of dental caries, gingival health, oral mucosal health and malocclusion.

  7. A novel c.1037C > G (p.Ala346Gly) mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC) syndrome

    OpenAIRE

    Leandro Ucela Alves; Eliete Pardono; Otto, Paulo A.; Regina Célia Mingroni Netto

    2014-01-01

    Ectrodactyly – ectodermal dysplasia and cleft lip/palate (EEC) syndrome (OMIM 604292) is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD) region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather) affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly). The disorder in this family exhibits a broad ...

  8. Effects of an early psychological intervention on parents of children with cleft lip/palate%早期心理干预对唇腭裂患儿父母的影响

    Institute of Scientific and Technical Information of China (English)

    王杨洋; 信燕华; 马坚; 辛秀红; 石冰; 黄永清

    2013-01-01

    目的 对唇腭裂患儿父母的心身健康状况进行评估并进行早期心理干预,探讨早期心理干预对唇腭裂患儿父母的影响.方法 采用症状自评量表(SCL-90)对102名唇腭裂患儿的父亲或母亲在入院当天进行调查,在住院期间及出院后3个月内定期地进行心理干预,并于出院当天及3个月后再进行问卷调查.以126名正常儿童的父亲或母亲作为对照组.结果 病例组患儿父母在躯体化、强迫症状、抑郁、焦虑等9个维度上分值均显著高于对照组(P<0.05);病例组3个亚组(唇裂组、腭裂组、唇腭裂组)间差异无统计学意义(P>0.05);病例组出院当天与入院时的统计结果差异无统计学意义(P>0.05),出院后3个月与入院时的统计结果有显著差异(P<0.05).结论 唇腭裂患儿父母的心身健康状况较差,早期心理干预对唇腭裂患儿家长具有重大影响,而且对患儿的心身健康也具有积极的意义.%Objective To provide basis for effects of an early psychological intervention on parents of children with cleft lip/palate,and investigate the effects of an early psychological intervention to them.Methods One selfadministered questionnaire (SCL-90) was applied in 102 parents of children with cleft lip/palate,compared to 126 parents of healthy individuals on the day of admission.They were given the psychological intervention during hospitalization and 3 months after discharge.The questionnaire (SCL-90) was again applied to them on the day of discharge and 3 months after discharge.Results Using the questionnaire(SCL-90),the answer scores of somatization,obsessivecompulsive,depression and anxiety etc.were significantly higher than those of the control group (P<0.05).There were no statistical differences during 3 subgroups in the case group (P>0.05).There were no statistical differences on the day of admission and on the day of discharge (P>0.05),but there were statistical difference on the day

  9. A Rare Interstitial Duplication of 8q22.1–8q24.3 Associated with Syndromic Bilateral Cleft Lip/Palate

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    Regina Ferreira Rezek

    2014-01-01

    Full Text Available We present a rare case of 8q interstitial duplication derived from maternal balanced translocations in a patient with bilateral cleft lip and palate in syndromic form associated with other congenital malformations. G-banding cytogenetic analysis revealed a chromosomal abnormality in the form of the karyotype 46,XX der(22t(8;22(q22.1;p11.1mat. Chromosome microarray analysis evidenced a 49 Mb duplicated segment of chromosome 8q with no pathogenic imbalances on chromosome 22. Two siblings also carry the balanced translocation. We have compared this case with other “pure” trisomies of 8q patients reported in the literature and with genome wide association studies recently published. This work highlights the involvement of chromosome 8q in orofacial clefts.

  10. 唇腭裂患儿父母心身健康状况调查与思考%A study on the psychosomatic states of parents of children with cleft lip/palate

    Institute of Scientific and Technical Information of China (English)

    王杨洋; 翟堃; 信燕华; 马坚; 黄永清; 辛秀红

    2012-01-01

    目的:了解唇腭裂患儿父母躯体及心理健康状况,为其进行心理治疗提供依据.方法:采用康奈尔医学指数(CMI)、家庭功能量表(FAD)及社会支持评定量表(SSRS)对102名唇腭裂患者的父亲或母亲及126名正常儿童的父亲或母亲进行调查,以了解唇腭裂患儿父母的躯体表现特征、心理健康状况及父母所面对的家庭、社会方面问题.结果:CMI中,病例组父母在躯体性疾病如眼和耳、心血管系统等方面以及在精神症状方面如不适应、抑郁等方面各因子分值均显著高于对照组(P<0.05);FAD及SSRS中,病例组父母各因子分值显著高于对照组(P<0.01).结论:唇腭裂患儿的父母心身健康状况较差,所获得的家庭及社会支持较少,应给予足够的重视并积极开展心理干预.%Objective: To provide basis for the emotional support and psychological therapy for parents of the children with cleft lip/pa late. Methods; Three self-administered questionnaires ( CMI, FAD and SSRS) were applied in 102 parents (experimental group) of the children with cleft lip/palate and 126 parents(controls) with healthy children respectively. Results:Using the instrument of CMI, the answer scores of somatization such as eye and ear, cardiovascular system etc. And the answer scores of mental symptoms such as maladjustment, depression etc. In the experimental group were significantly higher than those in the control (P <0. 05). Using the instruments of FAD and SSRS, the factor scores in the experimental group were significantly higher than those in the control ( P < 0. 01). Conclusion; The psychosomatic states of parents of children with cleft lip/palate is poor, and they get little support from family and society. They should be given active reconstruction and intervening.

  11. A novel c.1037C > G (p.Ala346Gly) mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC) syndrome.

    Science.gov (United States)

    Alves, Leandro Ucela; Pardono, Eliete; Otto, Paulo A; Mingroni Netto, Regina Célia

    2015-03-01

    Ectrodactyly - ectodermal dysplasia and cleft lip/palate (EEC) syndrome (OMIM 604292) is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD) region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather) affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly). The disorder in this family exhibits a broad spectrum of phenotypes: two individuals were personally examined, one presenting the complete constellation of EEC syndrome manifestations and the other presenting an intermediate phenotype; the third affected, a deceased individual not examined personally and referred to by his daughter, exhibited only the split-hand/foot malformation (SHFM). Our findings contribute to elucidate the complex phenotype-genotype correlations in EEC syndrome and other related TP63-mutation syndromes. The possibility of the mutation c.1037C > G being related both to acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome and SHFM is also raised by the findings here reported. PMID:25983622

  12. A novel c.1037C > G (p.Ala346Gly mutation in TP63 as cause of the ectrodactyly-ectodermal dysplasia and cleft lip/palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Leandro Ucela Alves

    2015-03-01

    Full Text Available Ectrodactyly – ectodermal dysplasia and cleft lip/palate (EEC syndrome (OMIM 604292 is a rare disorder determined by mutations in the TP63 gene. Most cases of EEC syndrome are associated to mutations in the DNA binding domain (DBD region of the p63 protein. Here we report on a three-generation Brazilian family with three individuals (mother, son and grandfather affected by EEC syndrome, determined by a novel mutation c.1037C > G (p.Ala346Gly. The disorder in this family exhibits a broad spectrum of phenotypes: two individuals were personally examined, one presenting the complete constellation of EEC syndrome manifestations and the other presenting an intermediate phenotype; the third affected, a deceased individual not examined personally and referred to by his daughter, exhibited only the split-hand/foot malformation (SHFM. Our findings contribute to elucidate the complex phenotype-genotype correlations in EEC syndrome and other related TP63-mutation syndromes. The possibility of the mutation c.1037C > G being related both to acro-dermato-ungual-lacrimal-tooth (ADULT syndrome and SHFM is also raised by the findings here reported.

  13. Recurrence of split hand/foot malformation, cleft lip/palate, and severe urogenital abnormalities due to germline mosaicism for TP63 mutation.

    Science.gov (United States)

    Enriquez, Annabelle; Krivanek, Michael; Flöttmann, Ricarda; Peters, Hartmut; Wilson, Meredith

    2016-09-01

    We describe two sibling fetuses with urogenital abnormalities detected by prenatal ultrasound, in which post-delivery examination showed split hand and foot malformation, and bilateral cleft lip and palate. These findings are consistent with ectrodactyly-ectodermal dysplasia-cleft lip with or without cleft palate syndrome (EEC). Both fetuses were found to have the same missense mutation in TP63 (c.1051G > A; p.D351N). Parental clinical examinations and lymphocyte DNA analyses were normal. This report illustrates the potential severity of urogenital defects in TP63-related disorders, which may be detectable with fetal ultrasonography. It highlights the need to counsel for the possibility of germline mosaicism in TP63-associated disorders. © 2016 Wiley Periodicals, Inc. PMID:27351625

  14. Is an isolated cleft lip an isolated anomaly?

    NARCIS (Netherlands)

    Deelder, J. D.; Breugem, C. C.; de Vries, Iris; de Bruin, M.; van der Molen, A. B. Mink; van der Horst, C. M. A. M.

    2011-01-01

    Introduction: It is well known that patients with cleft lip/palate or cleft palate can have associated anomalies. However, there is a relative paucity of information about the possible anomalies associated with an isolated cleft lip. A recent study (Vallino et al., 2008) showed that children with cl

  15. Current Training and Continuing Education Needs of Preschool and School-Based Speech-Language Pathologists regarding Children with Cleft Lip/Palate

    Science.gov (United States)

    Bedwinek, Anne P.; Kummer, Ann W.; Rice, Gale B.; Grames, Lynn Marty

    2010-01-01

    Purpose: The purpose of this study was to obtain information regarding the education and experience of preschool and school-based speech-language pathologists (SLPs) regarding the assessment and treatment of children born with cleft lip and/or palate and to determine their continuing education needs in this area. Method: A 16-item mixed-methods…

  16. Técnicas cirúrgicas correntes para fissuras lábio-palatinas, em Minas Gerais, Brasil Current surgical techniques for cleft lip-palate in Minas Gerais, Brazil

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    Lívia Maris Ribeiro Paranaíba

    2009-12-01

    Full Text Available Fissuras do lábio e/ou palato (FL/P representam as anomalias congênitas crânio-facial mais comuns. OBJETIVO: Avaliar as técnicas cirúrgicas correntes na reabilitação de FL/P em um Serviço de referência no Estado de Minas Gerais. MATERIAL E MÉTODOS: Realizou-se estudo retrospectivo, 2002 a 2007, avaliando 109 portadores de FL/P não sindrômicas que tiveram o tratamento concluído. As dimensões de análise (identificação pessoal, classificação das FL/P e tratamento cirúrgico realizado foram obtidas a partir dos prontuários, sendo posteriormente construído banco de dados e as análises estatísticas realizadas pelo programa SPSS 13.0. Seguiu-se análise descritiva dos procedimentos cirúrgicos em função do tipo de FL/P encontrada. RESULTADOS: Entre os 109 pacientes, 65,1% foram do gênero masculino e 34,8% do feminino. Verificou-se que 45% dos pacientes apresentaram fissuras lábio-palatinas, 37,6% fissuras labiais e 17,4% fissuras palatinas. As técnicas correntes empregadas foram as de Millard e Spina para as queiloplastias, McComb para as rinoplastias e as de Veau e Van Langenbeeck para as palatoplastias. CONCLUSÃO: Este estudo é o primeiro a abordar reabilitação em FL/P em Minas Gerais. Nas FL/P unilaterais houve a associação das técnicas de McComb, Veau e Millard, respectivamente, para rinoplastia, palatoplastia e queiloplastia, em 76,9% dos pacientes.Cleft lip and palate (CL/P are the most common congenital anomalies of the craniofacial region. AIM: to evaluate the surgical techniques used in CL/P treatment in a craniofacial deformities ward, in Minas Gerais. MATERIALS AND METHODS: In this retrospective study, carried out between 2002 and 2007, we studied 109 individuals with non-syndromic CL/P submitted to treatment. The aspects analyzed (personal identification, classification of CL/P and surgical treatment performed were obtained from patient charts, and then we built a database and ran statistical analyses

  17. Prenatal diagnosis of cleft lip/palate: The surface rendered oro-palatal (SROP) view of the fetal lips and palate, a tool to improve information-sharing within the orofacial team and with the parents.

    Science.gov (United States)

    Levaillant, Jean-Marc; Nicot, Romain; Benouaiche, Laurence; Couly, Gérard; Rotten, Daniel

    2016-07-01

    The ultrasonographic surface rendered oro-palatal (SROP) view is a 3D reconstructed view of the fetal perioral region, which combines ultrasound insonation in a trans oral, upward directed axial direction and the surface rendered mode. It allows the simultaneous visualization on a single scan of the superior lip, alveolar ridge and secondary palate. It corresponds prenatally to the submental intra oral photography of the palate of neonates. The aim of the study was to demonstrate the benefice of using the SROP view in the management of cleft lip with or without cleft palate, uni- or bi-lateral, diagnosed prenatally (22-28 gestational weeks). The SROP view allowed the representation on a single view of the characteristics of the defect useful to the different members of the orofacial team to exactly evaluate the difformity and to plan the ulterior therapeutic steps (e.g. side, extension of the cleft to the secondary palate, tooth organization). Also, being easier to read by lay people thanks to the use of a surface rendered representation rather than the usual multiplanar reconstructions in the three traditional orthogonal planes, the SROP view makes it easier to bring exact information to the parents about the malformation and its consequences. PMID:27211349

  18. Avaliação da idade materna, paterna, ordem de paridade e intervalo interpartal para fissura lábio-palatina Maternal and paternal age, birth order and interpregnancy interval evaluation for cleft lip-palate

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    Daniella Reis Barbosa Martelli

    2010-02-01

    Full Text Available Fissuras do lábio e/ou palato representam as anomalias congênitas craniofaciais mais comuns. OBJETIVO: Avaliar fatores de risco ambientais em pacientes com fissuras lábio-palatinas não-sindrômicas, em um Serviço de Minas Gerais. CASUÍSTICA E MÉTODO: Realizou-se estudo caso-controle, avaliando 100 crianças com fissuras e 100 crianças sem alterações clínicas. As dimensões de análise (idade, cor de pele, sexo, classificação das fissuras, idade materna e paterna, ordem de paridade e intervalo interpartal foram obtidas a partir de um questionário, sendo posteriormente construído banco de dados e as análises realizadas pelo programa SPSS 17.0. Os resultados foram analisados com risco relativo para cada variável, para estimar odds ratios com intervalo de confiança de 95% seguido de análise bivariada e multivariada. RESULTADOS: Entre as 200 crianças, 54% foram do sexo masculino e 46% do feminino. Com relação à cor da pele, houve predomínio de parda, branca e preta, respectivamente. Entre os tipos de fissuras, as mais comuns foram as fissuras lábio-palatinas (54%, seguidas pela fissura labial (30% e fissura palatina (16%. CONCLUSÃO: Embora com uma população limitada, verificou-se associação entre idade materna e risco aumentado para fissuras lábio-palatinas, porém idade paterna, ordem de paridade e intervalo interpartal não foram significantes.Cleft lip and palate (CL/P are the most common congenital craniofacial anomalies. AIM: To evaluate environmental risk factors for non-syndromic CL/P in a reference care center in Minas Gerais. MATERIALS AND METHODS: we carried out a case-controlled study, assessing 100 children with clefts and 100 children without clinical alterations. The analysis dimensions (age, skin color, gender, fissure classification, maternal and paternal age, birth order and interpregnancy interval, obtained from a questionnaire; and later we build a data base and the analyses were carried out by the

  19. What is the Risk of Having Offspring with Cleft Lip/Palate in Pre-Maternal Obese/Overweight Women When Compared to Pre-Maternal Normal Weight Women? A Systematic Review and Meta-Analysis

    Science.gov (United States)

    Izedonmwen, Omoroghogho Maria; Cunningham, Claudia

    2015-01-01

    ABSTRACT Objectives The purpose of the study was to identify the risk of orofacial cleft in the offspring of women with pre-maternal obesity/overweight when compared with pre-maternal normal weight women. Material and Methods MEDLINE and EMBASE were searched from 1980 to July 2014 for cohort, case control and cross sectional studies. BMI were categorized according to WHO recommendation: normal weight (BMI 18.5 - 24.9), overweight (BMI 25 - 29.9) and obese (BMI ≥ 30). Results Six studies were identified; three case control studies which were used for the meta-analysis and two cross sectional studies and one cohort study. Compared with women of recommended BMI, obese women were at increased odds of pregnancy affected by CLP (OR = 1.16; 95% CI 1, 1.34) and CP (OR = 1.14; 95% CI 0.95, 1.37). Overweight women were also at increased odds of pregnancy affected by CLP (OR = 1.06; 95% CI 0.93, 1.21) but not CP (OR = 0.89; 95% CI 0.75, 1.06). The results of the risk ratios reported in the cross sectional and cohort studies were similar to the results of the meta-analysis. Conclusions The results of this study reveal that there is an increased risk of having offspring with orofacial cleft in obese/overweight women. The reason for this association is not known. Although, the risk is small, it is important because of the increasing incidence of obesity. PMID:25937872

  20. APR-246/PRIMA-1MET rescues epidermal differentiation in skin keratinocytes derived from EEC syndrome patients with p63 mutations

    OpenAIRE

    Shen, Jinfeng; van den Bogaard, Ellen H.; Kouwenhoven, Evelyn N.; Vladimir J.N. Bykov; Rinne, Tuula; Zhang, Qiang; Tjabringa, Geuranne S.; Gilissen, Christian; Van Heeringen, Simon J.; Schalkwijk, Joost; Van Bokhoven, Hans; Wiman, Klas G.; Zhou, Huiqing

    2013-01-01

    p53 and p63 share extensive sequence and structure homology. p53 is frequently mutated in cancer, whereas mutations in p63 cause developmental disorders manifested in ectodermal dysplasia, limb defects, and orofacial clefting. We have established primary adult skin keratinocytes from ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome patients with p63 mutations as an in vitro human model to study the disease mechanism in the skin of EEC patients. We show that these patien...

  1. Comparison of dermatoglyphic traits and dental anomalies associated with cleft lip or cleft lip and palate patients with normal healthy children

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    Neha Maheshwari

    2013-01-01

    Full Text Available Background: Dermatoglyphics are considered as a window of congenital abnormalities and is known to be one of the best available diagnostic tools in genetic disorders. Objective: The present study was conducted to observe and compare the differences in the dermatoglyphic patterns between cleft lip/palate (CL/P and normal healthy children and evaluate their associated dental findings. Setting: This is a cross-sectional prevalence study in which dermatoglyphic patterns and dental anomalies of 90 (CL/P and normal healthy children aged 0-15 years were recorded under standard conditions of seating and lighting. Materials and Methods: Dermatoglyphic traits were recorded using ink stamp pad method. Dental findings were recorded through clinical and radiographic examination. Results: The most frequently seen dermatoglyphic trait in the study and control group was loops followed by whorls and arches. A highly significant (P < 0.001 difference between loops in the study and control group and statistically significant difference in the whorls (P = 0.001 were found using Student′s t-test. The comparison of dental anomalies was accomplished using Chi-square test and hypodontia (50% was seen maximum in cleft patients. Conclusion: Any deviation in dermatoglyphics features indicates a genetic etiology.

  2. Cleft palate and ADULT phenotype in a patient with a novel TP63 mutation suggests lumping of EEC/LM/ADULT syndromes into a unique entity: ELA syndrome.

    Science.gov (United States)

    Prontera, Paolo; Garelli, Emanuela; Isidori, Ilenia; Mencarelli, Amedea; Carando, Adriana; Silengo, Margherita Cirillo; Donti, Emilio

    2011-11-01

    Acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome is a rare condition belonging to the group of ectodermal dysplasias caused by TP63 mutations. Its clinical phenotype is similar to ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) and limb-mammary syndrome (LMS), and differs from these disorders mainly by the absence of cleft lip and/or palate. We report on a 39-year-old patient who was found to be heterozygous for a c.401G > T (p.Gly134Val) de novo mutation of TP63. This patient had the ADULT phenotype associated with cleft palate. Our findings, rather than extend the clinical spectrum of ADULT syndrome, suggest that cleft palate can no longer be considered an element for differential diagnosis for ADULT, EEC, and LMS. Our data, added to other reports on overlapping phenotypes, support the combining of these three phenotypes into a unique entity that we propose to call "ELA syndrome," which is an acronym of ectrodactyly-ectodermal dysplasia-cleft lip and palate, limb-mammary, and ADULT syndromes.

  3. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

    OpenAIRE

    Buket Uysal Aladag; Fatma Hilal Yilmaz; Nadir Kocak; Ali Annagur

    2013-01-01

    Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC) is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate). A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atr...

  4. Base of the skull morphology and Class III malocclusion in patients with unilateral cleft lip and palate

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    Mariana Maciel Tinano

    2015-02-01

    Full Text Available OBJECTIVE: The aim of the present study was to determine the morphological differences in the base of the skull of individuals with cleft lip and palate and Class III malocclusion in comparison to control groups with Class I and Class III malocclusion. METHODS: A total of 89 individuals (males and females aged between 5 and 27 years old (Class I, n = 32; Class III, n = 29; and Class III individuals with unilateral cleft lip and palate, n = 28 attending PUC-MG Dental Center and Cleft Lip/Palate Care Center of Baleia Hospital and PUC-MG (CENTRARE were selected. Linear and angular measurements of the base of the skull, maxilla and mandible were performed and assessed by a single calibrated examiner by means of cephalometric radiographs. Statistical analysis involved ANCOVA and Bonferroni correction. RESULTS: No significant differences with regard to the base of the skull were found between the control group (Class I and individuals with cleft lip and palate (P > 0.017. The cleft lip/palate group differed from the Class III group only with regard to CI.Sp.Ba (P = 0.015. Individuals with cleft lip and palate had a significantly shorter maxillary length (Co-A in comparison to the control group (P < 0.001. No significant differences were found in the mandible (Co-Gn of the control group and individuals with cleft lip and palate (P = 1.000. CONCLUSION: The present findings suggest that there are no significant differences in the base of the skull of individuals Class I or Class III and individuals with cleft lip and palate and Class III malocclusion.

  5. Difference in the Surgical Outcome of Unilateral Cleft Lip and Palate Patients with and without Pre-Alveolar Bone Graft Orthodontic Treatment

    Science.gov (United States)

    Chang, Chun-Shin; Wallace, Christopher Glenn; Hsiao, Yen-Chang; Chiu, Yu-Ting; Pai, Betty Chien-Jung; Chen, I-Ju; Liao, Yu-Fang; Liou, Eric Jen-Wein; Chen, Philip Kuo-Ting; Chen, Jyh-Ping; Noordhoff, M. Samuel

    2016-01-01

    Presurgical orthodontic treatment before secondary alveolar bone grafting (SABG) is widely performed for cleft lip/palate patients. However, no randomized controlled trial has been published comparing SABG outcomes in patients with, and without, presurgical orthodontic treatment. This randomized, prospective, single-blinded trial was conducted between January 2012 and April 2015 to compare ABG volumes 6 months postoperatively between patients with and without presurgical orthodontic treatment. Twenty-four patients were enrolled and randomized and 22 patients completed follow-up. Patients who had presurgical orthodontics before SABG had significantly improved inclination (p < 0.001) and rotation (p < 0.001) of the central incisor adjacent to the defect, significantly improved ABG fill volume (0.81 ± 0.26 cm3 at 6 months compared to 0.59 ± 0.22 cm3; p < 0.05) and less residual alveolar bone defect (0.31 ± 0.08 cm3 at 6 months compared to s 0.55 ± 0.14 cm3; p < 0.001) compared to patients who did not have presurgical orthodontic treatment. In conclusion, orthodontic treatment combined with SABG results in superior bone volume when compared with conventional SABG alone. PMID:27041697

  6. Complex segregation analysis of nonsyndromic cleft lip/palate in a Chilean population

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    Blanco Rafael

    1998-01-01

    Full Text Available A população urbana Chilena contemporânea deriva da mistura de ameríndios nativos com espanhóis, apresentando uma incidência média de fissura labial não sindrômica associada ou não a fissura palatina (NSCLP de 1,8 por 1000 nascimentos vivos. A análise de segregação complexa usando o programa de computador POINTER foi feita em 249 pedigrees estendidos, distribuídos em 202 famílias simplex e 47 famílias multiplex obtidas de probands de NSCLP afetados (157 homens e 92 mulheres. Esses pedigrees deram origem a 326 indivíduos afetados e mais de 1454 parentes. Oito modelos hipotéticos foram examinados e comparados pelo teste c 2 log2 razão de máxima verossimilhança. Os modelos que postulam que NSCLP não era transmitida nestas famílias foram rejeitados, assim como os modelos que postulam apenas um componente multifatorial (P < 0,0001. O modelo que postula não haver componente poligênico para a transmissão não pôde ser rejeitado mas o modelo de não transmissão do efeito mais importante foi rejeitado (P < 0,0001. Entre os modelos do locus mais importante apenas o modelo recessivo de transmissão foi rejeitado, enquanto que as heranças codominante e dominante sem um componente multifatorial não puderam ser excluídas. O modelo não restrito sugere que a freqüência do alelo de suscetibilidade a NSCLP no locus mais importante é 0,0037 e sua penetrância é de 92%.

  7. Skull thickness in patients with clefts

    DEFF Research Database (Denmark)

    Arntsen, T; Kjaer, I; Sonnesen, L;

    2010-01-01

    The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP).......The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP)....

  8. Comparison of periodontal status among patients with cleft lip, cleft palate, and cleft lip along with a cleft in palate and alveolus

    Directory of Open Access Journals (Sweden)

    Boloor Vinita

    2010-01-01

    Full Text Available Background and Objectives : A healthy periodontium is an important prerequisite for unhindered dentition and long-term oral health. In cleft subjects, especially in those with cleft lip, alveolus and palate (CLAP, maintenance of oral hygiene is a difficult task for the patients because of the patent oro-nasal communication. Crowding of teeth in cleft patients is a common finding, especially in those with CLAP and those with cleft palate (CP. In the case of multiple tooth-malpositions , transverse deficiency, arch length deficiency and primary cross-bite; periodontal trauma increases and is detrimental to periodontal health . According to literature, a critical periodontal situation was found in patients with CLAP. Hence a study was conducted to analyze the periodontal status of patients with cleft lip (CL; those with cleft palate; and those with cleft lip, alveolus and palate. Materials and Methods: The present study consisted of 60 cleft subjects divided into 3 groups: those with cleft lip; those with cleft palate; and those with cleft lip, alveolus and palate. Subjects with permanent dentition were selected, and the clinical examination included determination of oral hygiene status using Oral Hygiene Index - Simplified (OHI-S index and periodontal status using community periodontal index (CPI. Results: Statistically significant increase in the periodontal disease in the CLAP group as compared with the other 2 groups, and the oral hygiene was seen to be generally poor with the CLAP group. Interpretation and Conclusion: Individuals with clefts are more prone to periodontal disease due to the presence of cleft, which causes retention of food in the defect sites and inability to maintain good oral hygiene; but the severity of periodontal disease is more if the defect is large and involving the lip, alveolus and palate.

  9. Influence of lip closure on alveolar cleft width in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Schmelzle Rainer

    2011-01-01

    Full Text Available Abstract Background The influence of surgery on growth and stability after treatment in patients with cleft lip and palate are topics still under discussion. The aim of the present study was to investigate the influence of early lip closure on the width of the alveolar cleft using dental casts. Methods A total of 44 clefts were investigated using plaster casts, 30 unilateral and 7 bilateral clefts. All infants received a passive molding plate a few days after birth. The age at the time of closure of the lip was 2.1 month in average (range 1-6 months. Plaster casts were obtained at the following stages: shortly after birth, prior to lip closure, prior to soft palate closure. We determined the width of the alveolar cleft before lip closure and prior to soft palate closure measuring the alveolar cleft width from the most lateral point of the premaxilla/anterior segment to the most medial point of the smaller segment. Results After lip closure 15 clefts presented with a width of 0 mm, meaning that the mucosa of the segments was almost touching one another. 19 clefts showed a width of up to 2 mm and 10 clefts were still over 2 mm wide. This means a reduction of 0% in 5 clefts, of 1-50% in 6 clefts, of 51-99% in 19 clefts, and of 100% in 14 clefts. Conclusions Early lip closure reduces alveolar cleft width. In most cases our aim of a remaining cleft width of 2 mm or less can be achieved. These are promising conditions for primary alveolar bone grafting to restore the dental bony arch.

  10. Associations of chromosomes 17q22, 10q25.3 and ABCA4 gene polymorphisms with non-syndromic cleft lip/palate in Ningxia Hui and Han population%宁夏回汉族人群中染色体17q22、10q25.3和ABCA4基因多态性与非综合征型唇腭裂的关联

    Institute of Scientific and Technical Information of China (English)

    周忠伟; 杨雄; 万应彪; 信燕华; 翟堃; 马坚; 黄永清; 姜敏; 王怡瑞

    2013-01-01

    with or without cleft palate (NSCL/P) in Ningxia Hui and Han population. Methods The study consisted of 415 NSCL/P patients( Han = 191 ,Hui = 224) and their parents (303 fathers and 311 mothers, including 158 complete families). They were divided into 3 groups: the cleft lip only (CLO), the cleft palate only(CPO) and both the cleft lip and palate( CLP) groups, and we merged the CLO and CLP groups into the cleft Up with or without palate ( CL/P) group. In addition, 385 healthy college students (Han =261, Hui = 104) consisted of the control group. The single nucleotide polymorphisms ( SNPs) were genotyped by TaqMan SNPs genotyping and the data were analyzed by case-control analysis, transmission disequilibrium test and family based association test. Results ① The distribution of alleles at rs227731 on chromosome 17q22 was significantly different between CLO and control groups in the Hans (P < 0.05), and significant difference was found between CL/P and control groups in the Hans (P<0.05). There were significant differences in the genotypic and allelic frequencies of CLP and CL/P groups between the two ethnics. ② The distributions of both genotypes and alleles at rs560426 in ABCA4 gene were also significantly different between CLP and control groups in the Hans (P < 0.05), and significant difference was found between CL/P and control groups in the Hans ( P < 0. 05 ). There were significant differences in the genotypic and allelic frequencies of CLO groups between the two ethnics. ③ No differences were found in genotypic and allelic frequencies of rs7078160 on chromosome 10q25 between the patients and their parents and the controls, and between the two ethnics. Conclusion rs227731 and rs560426 SNPs are associated with NSCL/P in Ningxia Han population, and rs7078160 SNPs are not associated with NSCL/P in Ningxia population.

  11. Periodontal Status Among Patients With Cleft Lip (CL), Cleft Palate (CP) and Cleft Lip, Alveolus and Palate (CLAP) In Chennai, India. A Comparative Study

    Science.gov (United States)

    John, Joseph

    2015-01-01

    Background: Long term health of the stomatognathic system as well as esthetic aspects is the therapeutic goals in patients with oro facial clefts. Aim: The aim of this study was to assess and compare the periodontal status of patients with cleft lip (CL), cleft palate (CP) and cleft lip, alveolus and palate (CLAP) reporting to a hospital in Chennai, India. Materials and Methods: The study group consisted of 80 cleft patients. Subjects were divided into three groups. Group 1: patients with cleft lip (CL), Group 2: subjects with cleft palate (CP) and Group 3: subjects with cleft lip alveolus and palate (CLAP). Community Periodontal Index for Treatment needs CPITN Index was recorded. Results: Among the 80 study subjects, 51 (63.8%) were males and 29 (36.2%) were females. Among the 26 study subjects with cleft lip, 10 (38.5%) had healthy periodontium, 4 (15.4%) had bleeding on probing and 12 (46.1%) had calculus. Mean number of sextants coded for healthy and bleeding was maximum among the subjects with cleft palate. Mean number of sextants coded for calculus was maximum among the subjects with cleft lip alveolus and palate. Prevalence of periodontal disease is high among patients with cleft lip, alveolus and palate (35%) than in Cleft lip (32.5%) and Cleft Palate (32.5%). Conclusion: Gingivitis and Calculus is predominantly high in patients with Cleft Palate and Cleft Lip respectively. PMID:25954706

  12. A Review of Hearing Loss in Cleft Palate Patients

    OpenAIRE

    Bilal Gani; Kinshuck, A. J.; Sharma, R.

    2012-01-01

    Background. Cleft palate is associated with recurrent otitis media with effusion and hearing loss. This study analysed the way these patients' hearing is managed in Alder Hey Children's Hospital. Method. A retrospective audit was carried out on cleft palate patients in Alder Hey Children's Hospital. Audiology assessment and treatment options were reviewed. Comparisons were made between the use of ventilation tubes (VTs) and hearing aids (HAs). The types of cleft, types of hearing loss, and th...

  13. Bilateral optic disc pit with maculopathy in a patient with cleft lip and cleft palate

    Science.gov (United States)

    Seth, Anisha; Gupta, Rajat; Gupta, Anika; Raina, Usha K; Ghosh, Basudeb

    2015-01-01

    Optic disc pit (ODP) is small, gray-white, oval depression found at the optic nerve head. It is a congenital defect that occurs due to imperfect closure of superior edge of the embryonic fissure. Cleft lip and palate are also congenital midline abnormalities occurring due to defect in the fusion of frontonasal prominence, maxillary prominence and mandibular prominence. There is only one case report describing the occurrence of ODP in a young patient with cleft lip and palate who also had basal encephalocele. We describe a 52-year-old patient with congenital cleft lip and palate with bilateral ODP with maculopathy but without any other midline abnormality. PMID:26044478

  14. Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients

    DEFF Research Database (Denmark)

    Andersen, Kristian

    2012-01-01

    Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients......Reconstruction of alveolar defects in patients with cleft lip and palate - 111 consecutive patients...

  15. Growth hormone deficiency in cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Shahin AbdollahiFakhim

    2015-11-01

    Full Text Available Introduction: Failure to thrive (FTT is relatively common among cleft patients, most commonly attributed to feeding problems during the first months of life. Close association between midline clefts and pituitary gland abnormalities prompted us to determine the frequency of growth hormone deficiency in cleft patients, which is easily treated. Methods: Any cleft patient with FTT was studied and when the patient’s height was under the 3rd percentile of normal, growth hormone was checked after clonidine administration. Growth hormone was checked before and 30, 60 and 90 minutes after clonidine use. Results: Of 670 patients with cleft lip or palate, 31 patients (4% had some kind of growth retardation according to weight, height or head circumstance. Eighteen patients were under the 3rd percentile of normal height. Growth hormone deficiency was detected in 8 patients out of 18 patients and overall frequency of growth hormone deficiency among cleft patients with growth retardation was 25.8% (8 out of 31. Seven patients of 8 were male whereas one was female and half of the patients were syndromic. Conclusion: Cleft patients have many problems with normal feeding and all kind of support should be provided to achieve near-normal feeding and they should be monitored for normal growth. Any patient with growth retardation, especially height decrease, should be assessed for growth hormone deficiency.

  16. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Mohita Marwaha

    2012-01-01

    Full Text Available The ectodermal dysplasias (EDs are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  17. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome)

    OpenAIRE

    Mohita Marwaha; Kanwar Deep Singh Nanda

    2012-01-01

    The ectodermal dysplasias (EDs) are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  18. COMPARATIVE STUDY OF DERMATOGLYPHIC FINGERTIP PATTERNS AND ‘ atd ’ ANGLE IN PATIENTS OF CLEFT LIP WITH OR WITHOUT CLEFT PALATE AND ISOLATED CLEFT PALATE WITH NORMAL POPULATION

    Directory of Open Access Journals (Sweden)

    Siraz M.

    2015-08-01

    Full Text Available Dermatoglyphics is the scientific study of epidermal ridges and their configurations on the volar aspect of palmar and plantar region. The main etiological factor of cleft lip and cleft palate is genetic in nature. The influences of genetic and environmental factors on early development are often reflected by the altered dermatoglyphics. AIMS & OBJECTIVES : To study the fingertip dermatoglyphic patterns in cleft lip and cleft palate patients. To compare the derm - atoglyphic ‘atd’ angle of cleft lip and cleft palate patients with normal population. MATERIAL & METHODS : The present study is a case control study carried out from Dec 2004 to Nov 2006. 86 cases of cleft lip with or without cleft palate and isolated cleft palate attending OPD of Govt. Medical College & Hospital, Miraj, Civil Hospital, Sangli, Aditya Burn and Plastic Surgery Hospital, Sangli and 100 controls with age and sex matched during the study period were included in study. The cases and controls divided in three groups : A ( C left lip with or without cleft palate ; B ( I solated cleft palate and C (Controls. In this study, ‘STANDARD INK METHOD’ for obtaining the dermatoglyphic prints described by Cummins (1936 and Cummins and Midlo (1961 was used. The parameters studied among different groups were sex wise distribution, hereditary basis, analysis of Fingertip Patterns and analysis of dermatoglyphic ‘atd’ angle. Appropriate statistical tests were applied like Mean, Standard Deviation (S.D., standard Error (S.E., Unpaired ‘t’ test of significance, for quantitative data, Chi - square’ test for qualitative data and ‘P’ value. OBSERVATIONS & RESULTS : Out of 82 Patients, 50 Patients are having cleft lip with or without cleft palate defect, while 32 patients are having isolated cleft palate defect with female dominance. The difference observed in percentage distribution of whorls and arches was statically significant in group A and C. The percentage

  19. Aspiration pneumonia in patients with cleft palate

    International Nuclear Information System (INIS)

    To assess the incidence of aspiration pneumonia in infants with cleft palate and to compare the incidence between complete and incomplete types of cleft palate. A review of medical records revealed 100 infants who had undergone initial surgery to repair cleft palate in our hospital during a recent three-year period. Aspiration pneumonia was defined as the coexistence of pneumonia at chest radiography with a history of frequent choking during feeding. The anatomic distribution of aspiration pneumonia was analyzed, and the incidences of aspiration pneumonia in infants with complete and incomplete cleft palate were compared. Among 100 children, aspiration pneumonia was found in 35 (35%). Those with complete and incomplete cleft palate showed similar incidences of the condition (27 of 70 [39%] vs 8 of 30 [27%], p=0.36). Pneumonia was most commonly seen in the left lower lobe (11 of 35), followed by the right upper and lower lobes. Aspiration pneumonia is frequently associated with infants with cleft palate. There is no statistical difference in the incidence of aspiration pneumonia between the complete and the incomplete cleft palate group

  20. Correlations between initial cleft size and dental anomalies in unilateral cleft lip and palate patients after alveolar bone grafting

    Science.gov (United States)

    Jabbari, Fatima; Reiser, Erika; Thor, Andreas; Hakelius, Malin; Nowinski, Daniel

    2016-01-01

    Objective To determine in individuals with unilateral cleft lip and palate the correlation between initial cleft size and dental anomalies, and the outcome of alveolar bone grafting. Methods A total of 67 consecutive patients with non-syndromic unilateral complete cleft lip and palate (UCLP) were included from the cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. All patients were operated by the same surgeon and treated according to the Uppsala protocol entailing: lip plasty at 3 months, soft palate closure at 6 months, closure of the residual cleft in the hard palate at 2 years of age, and secondary alveolar bone grafting (SABG) prior to the eruption of the permanent canine. Cleft size was measured on dental casts obtained at the time of primary lip plasty. Dental anomalies were registered on radiographs and dental casts obtained before bone grafting. Alveolar bone height was evaluated with the Modified Bergland Index (mBI) at 1 and 10-year follow-up. Results Anterior cleft width correlated positively with enamel hypoplasia and rotation of the central incisor adjacent to the cleft. There was, however, no correlation between initial cleft width and alveolar bone height at either 1 or 10 years follow-up. Conclusions Wider clefts did not seem to have an impact on the success of secondary alveolar bone grafting but appeared to be associated with a higher degree of some dental anomalies. This finding may have implications for patient counseling and treatment planning. PMID:26923345

  1. Presurgical nasoalveolar moulding treatment in cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Grayson Barry

    2009-10-01

    Full Text Available Presurgical infant orthopedics has been employed since 1950 as an adjunctive neonatal therapy for the correction of cleft lip and palate. Most of these therapies did not address deformity of the nasal cartilage in unilateral and bilateral cleft lip and palate as well as the deficiency of the columella tissue in infants with bilateral cleft. The nasolaveolar molding (NAM technique a new approach to presurgical infant orthopedics developed by Grayson reduces the severity of the initial cleft alveolar and nasal deformity. This enables the surgeon and the patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity. This paper will discuss the appliance design, clinical management and biomechanical principles of nasolaveolar molding therapy. Long term studies on NAM therapy indicate better lip and nasal form, reduced oronasal fistula and labial deformities, 60 % reduction in the need for secondary alveolar bone grafting. No effect on growth of midface in sagittal and vertical plane has been recorded up to the age of 18 yrs. With proper training and clinical skills NAM has demonstrated tremendous benefit to the cleft patients as well as to the surgeon performing the repair.

  2. Prosthodontic treatment of the edentulous adult cleft palate patient.

    Science.gov (United States)

    Sykes, Leanne M

    2003-03-01

    Clefts of the upper lip and plate are relatively common, yet dental treatment of these patients is still very poor and many grow up suffering dental neglect. Dental practitioners should become involved in the treatment team as dental needs are present from birth to death. Adult cleft patients often need tooth replacement with obturation of any residual clefts. They are best treated with tooth-supported removable appliances including partial and complete overdentures, thus preservation of their natural dentition is desirable. Edentulous cleft palate patients present with restorative difficulties due to their compromised maxillary arches as well as the presence of scar tissue in their palates and lips. An outline of these complications and guidelines for their treatment is illustrated in the form of three case reports from members of one family all presenting with varying cleft lip and palate defects. This article highlights the need for dental students to be exposed to dentally compromised patients so that they will feel confident enough to treat them in private practice. PMID:12800267

  3. Advances in the Description of the Speech of People with Lip-palatal Fissure

    Directory of Open Access Journals (Sweden)

    Maryluz Camargo Mendoz

    2006-12-01

    Full Text Available This article compiles information of currentresearch in the speech description of people withcleft lip and palate, in order to present the advancesin the field of clinical phonetics in theformation of the Speech Language Pathologists.This article also explores the case study of a 12years old subject with cleft palate, hypernasalspeech, nasal air escapes and glottal stops, whichwere detected perceptual and spectrographically.

  4. Postoperative morbidity after reconstruction of alveolar bone defects with chin bone transplants in cleft patients - 111 consecutive patients

    DEFF Research Database (Denmark)

    Andersen, Kristian; Nørholt, Sven Erik; Knudsen, Johan;

    Postoperative morbidity after reconstruction of alveolar bone defects with chin bone transplants in cleft patients - 111 consecutive patients......Postoperative morbidity after reconstruction of alveolar bone defects with chin bone transplants in cleft patients - 111 consecutive patients...

  5. Examine your orofacial cleft patients for Gorlin-Goltz syndrome.

    Science.gov (United States)

    Lambrecht, J T; Kreusch, T

    1997-07-01

    The Gorlin-Goltz syndrome is characterized by four primary symptoms: multiple nevoid basal cell epitheliomas that usually undergo malignant transformation; jaw keratocysts that show constant growth; skeletal anomalies; and intracranial calcifications. A myriad of additional findings may also be noted. Among the most frequent are: palmar and plantar pits, a characteristic flattened facies and broad nasal root, frontal and parietal bossing, mandibular prognathia, hypertelorism, strabismus, dystrophia of the canthi, and clefts of the lip, alveolus, and/or palate. In this study, we review the literature and our 25 cases of Gorlin-Goltz syndrome patients, questioning their incidence of cleft formations (8.5%) as compared to the general population (0.1%). It is our contention that all patients who present with an orofacial cleft warrant deeper investigation as to the presence of additional signs indicative of Gorlin-Goltz syndrome. The nevi turn malignant with time, and thus, early diagnosis, follow-up, and treatment are imperative.

  6. Is there an optimal resting velopharyngeal gap in operated cleft palate patients?

    OpenAIRE

    Rajesh Yellinedi; Mukunda Reddy Damalacheruvu

    2013-01-01

    Context: Videofluoroscopy in operated cleft palate patients. Aims: To determine the existence of an optimal resting velopharyngeal (VP) gap in operated cleft palate patients Settings and Design: A retrospective analysis of lateral view videofluoroscopy of operated cleft palate patients. Materials and Methods: A total of 117 cases of operated cleft palate underwent videofluoroscopy between 2006 and 2011. The lateral view of videofluoroscopy was utilised in the study. A retrospective analysis o...

  7. Surgical management of cleft lip in pedo-patients.

    Science.gov (United States)

    Taware, C P; Kulkarni, S R

    1991-01-01

    The Present article describes in short etiology of cleft lip and cleft palate. With this in-born defect, patient develops crucial problems with feeding, phonation, overall growth and development of affected and allied soft and hard tissue structures. This in turn results in deformity and asymmetry which is going to affect functional requirements as well as aesthetic outlook. Hence it really becomes mandatory to correct this defect surgically as early as possible, at stipulated timings so as to avoid present and future anticipated problems. PMID:1820390

  8. Surgical management of cleft lip in pedo-patients.

    Science.gov (United States)

    Taware, C P; Kulkarni, S R

    1991-01-01

    The Present article describes in short etiology of cleft lip and cleft palate. With this in-born defect, patient develops crucial problems with feeding, phonation, overall growth and development of affected and allied soft and hard tissue structures. This in turn results in deformity and asymmetry which is going to affect functional requirements as well as aesthetic outlook. Hence it really becomes mandatory to correct this defect surgically as early as possible, at stipulated timings so as to avoid present and future anticipated problems.

  9. An illusionary prosthetic design for a unilateral cleft palate patient

    Directory of Open Access Journals (Sweden)

    Andaç Barkın Bavbek

    2014-01-01

    Full Text Available The prosthetic rehabilitation is an important part of the cleft lip and palate therapy assisting orthodontic and orthognathic treatments. Prosthesis does not only help to improve function and aesthetics but also needs to facilitate a better oral health. The aim of this report is to introduce the prosthetic approach of a 21-year-old female unilateral cleft palate patient that considered reinforcing the mobile canine adjacent to the cleft, easing the elimination of dental plaque from the remaining fistula and reaching an accurate occlusion. Facial aesthetics was established by the illusionary effect of a removable crown complex which is joined onto a fixed partial denture with a precision attachment system.

  10. Strategies for Treating Compensatory Articulation in Patients with Cleft Palate

    OpenAIRE

    Del Carmen Pamplona, Maria; Ysunza, Antonio; Morales, Santiago

    2014-01-01

    Patients with cleft palate frequently show compensatory articulation (CA). CA requires a prolonged period of speech intervention. Some scaffolding strategies can be useful for correcting placement and manner of articulation in these cases. The purpose of this paper was to study whether the use of specific strategies of speech pathology can be more effective if applied according to the level of severity of CA. Ninety patients with CA were studied in two groups. One group was treated using stra...

  11. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome with Tetralogy of Fallot: a very rare combination

    Directory of Open Access Journals (Sweden)

    Deepak eSharma

    2015-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC syndrome is a rare genetic disorder with an incidence of around 1:90,000 live births. It is known with various names which includes split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis.

  12. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination

    Science.gov (United States)

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis. PMID:26137453

  13. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination

    OpenAIRE

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysi...

  14. Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination.

    Science.gov (United States)

    Sharma, Deepak; Kumar, Chetan; Bhalerao, Sanjay; Pandita, Aakash; Shastri, Sweta; Sharma, Pradeep

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1 in 90,000 in population. It is known with various names including split hand-split foot-ectodermal dysplasia-cleft syndrome or split hand, cleft hand, or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis. PMID:26137453

  15. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome) with Tetralogy of Fallot: a very rare combination

    OpenAIRE

    Deepak eSharma; Chetan eKumar; Sanjay eBhalerao; Aakash ePandita; Sweta eShastri; Pradeep eSharma

    2015-01-01

    Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome (EEC) syndrome is a rare genetic disorder with an incidence of around 1:90,000 live births. It is known with various names which includes split hand–split foot–ectodermal dysplasia–cleft syndrome or split hand, cleft hand or lobster claw hand/foot. We report first case of EEC with associated heart disease (Tetralogy of Fallot) who was diagnosed as EEC on the basis of clinical features and EEC was confirmed with genetic analysis...

  16. Prevalence of Associated Anomalies in Cleft Lip and/or Palate Patients

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahi Fakhim

    2016-03-01

    Full Text Available Introduction: Orofacial clefts are among the most common congenital anomalies. Patients presenting with orofacial clefts often require surgery or other complex procedures. A cleft lip or palate can be a single anomaly or a part of multiple congenital anomalies. The reported prevalence of cleft disease and associated anomalies varies widely across the literature, and is dependent on the diagnostic procedure used. In this study we determined the prevalence of associated anomalies in patients with a cleft lip and/or palate, with a specific focus on cardiac anomalies. Materials and Methods: In this cross-sectional study, 526 patients with a cleft lip and /or palate admitted to the children’s referral hospital between 2006 and 2011 were evaluated. All associated anomalies were detected and recorded. Patient information collected included age, gender, type and side of cleft, craniofacial anomalies and presence of other anomalies, including cardiac anomalies. Data were analyzed using SPSS version 16.   Results: Of the 526 patients enrolled in the study, 58% (305 were male and 42% (221 were female. In total, 75% of patients (396 were aged between 4 and 8 years and 25% (130 were aged less than 4 years. The most common cleft type in our study was bilateral cleft palate. The most commonly associated anomaly among cleft patients, in 12% of cleft patients, was a cardiac anomaly. The most common cardiac anomaly was atrial septal defect (ASD.   Conclusion:  The prevalence of associated anomalies among orofacial cleft patients is high. The most common associated anomaly is cardiac anomaly, with ASD being the most common cardiac anomaly. There are no significant relationships between type of cleft and associated cardiac anomalies.

  17. Investigations on the palatal rugae pattern in cleft patients. Part I: A morphological analysis.

    Science.gov (United States)

    Kratzsch, H; Opitz, C

    2000-01-01

    The characteristics of the palatal rugae zone (number of rugae, relief type, posterior limitation) were investigated on the maxillary casts of 44 patients with unilateral cleft lip and palate and 28 patients with bilateral clefts by means of reflex microscopy, a three-dimensional, computer-assisted, touch-free measuring system for the metrical registration and analysis of the parameters directly on the maxillary casts for the segments of the 2 cleft groups. The features "number of palatal rugae" and "relief type" (primary rugae) were determined both before and after surgical repair of the cleft palate. Both segments in unilateral cleft lip and palate and both lateral segments in bilateral clefts most commonly had 4 to 5 palatal rugae. The number of rugae in cleft patients is thus in a range that other authors have reported for non-cleft individuals. Following palatal cleft repair, the rugae counts per segment decreased significantly in patients with unilateral and bilateral cleft lip and palate but the 3rd rugae was never lost after surgery. The relief type identified in unilateral and bilateral cleft lip and palate was the same as in isolated cleft palates and did not differ from that in non-cleft subjects. The posterior limitation of the palatal rugae zone was determined both in a tooth-defined manner and as an absolute linear distance (at all time points). The most frequent tooth-defined posterior limitation of the rugae zone in unilateral and bilateral clefts was the second deciduous molar, which is also the position identified for non-cleft individuals. The linear distance from the tuberosity line to the rugae zone increased in all segments of unilateral and bilateral clefts during the interval up to palatal cleft repair, indicating sagittal maxillary development in the posterior area of the palate. Surgical repair of the cleft palate resulted in a significant shortening of the distance in both segments of the unilateral cleft, most likely due to the

  18. Bone and Soft Tissue Changes after Two-Jaw Surgery in Cleft Patients

    OpenAIRE

    Yun, Yung Sang; Uhm, Ki Il; Kim, Jee Nam; Shin, Dong Hyeok; Choi, Hyun Gon; Kim, Soon Heum; Kim, Cheol Keun; Jo, Dong In

    2015-01-01

    Background Orthognathic surgery is required in 25% to 35% of patients with a cleft lip and palate, for whom functional recovery and aesthetic improvement after surgery are important. The aim of this study was to examine maxillary and mandibular changes, along with concomitant soft tissue changes, in cleft patients who underwent LeFort I osteotomy and sagittal split ramus osteotomy (two-jaw surgery). Methods Twenty-eight cleft patients who underwent two-jaw surgery between August 2008 and Nove...

  19. Frequency of homologous blood transfusion in patients undergoing cleft lip and palate surgery

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    Adeyemo Wasiu

    2010-01-01

    Full Text Available Aim: The study aims to determine the frequency of homologous blood transfusion in patientsundergoing cleft lip and palate surgery at the Lagos University Teaching Hospital, Nigeria. Setting and Design: A prospective study of transfusion rate in cleft surgery conducted at the Lagos University Teaching Hospital, Nigeria. Material and Methods: One hundred consecutive patients who required cleft lip and palate surgery were recruited into the study. Data collected included age, sex and weight of patients, type of cleft defects, type of surgery done, preoperative haematocrit, duration of surgery, amount of blood loss during surgery, the number of units of blood cross-matched and those used. Each patient was made to donate a unit of homologous blood prior to surgery. Results: There were 52 females and 48 males with a mean age of 64.4 ± 101.1 months (range, 3-420 months. The most common cleft defect was isolated cleft palate (45% followed by unilateral cleft lip (28%. Cleft palate repair was the most common procedure (45% followed by unilateral cleft lip repair (41%. The mean estimated blood loss was 95.8 ± 144.9 ml (range, 2-800ml. Ten (10% patients (CL=2; CP=5, BCL=1; CLP=2 were transfused but only two of these were deemed appropriate based on percentage blood volume loss. The mean blood transfused was 131.5 ± 135.4ml (range, 35-500ml. Six (60% of those transfused had a preoperative PCV of < 30%. Only 4.9% of patients who had unilateral cleft lip surgery were transfused as compared with 50% for CLP surgery, 11% for CP surgery, and 10% for bilateral cleft lip surgery. Conclusions: The frequency of blood transfusion in cleft lip and palate surgery was 10% with a cross-match: transfusion ratio of 10 and transfusion index of 0.1. A "type and screen" policy is advocated for cleft lip and palate surgery.

  20. A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla

    DEFF Research Database (Denmark)

    Andersen, Kristian; Nørholt, Sven Erik; Küseler, Annelise;

    2012-01-01

    A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla......A Retrospective Study of Cleft lip and palate Patients' Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla...

  1. Morbidity of chin bone transplants used for reconstructing alveolar defects in cleft patients

    NARCIS (Netherlands)

    Booij, A; Raghoebar, GM; Jansma, J; Kalk, WWI; Vissink, A

    2005-01-01

    Objective: The aim of this study was to evaluate the objective and subjective morbidity of symphyseal chin bone harvesting used for reconstruction of alveolar defects in young cleft patients. Design: All patients who had undergone chin bone harvesting for alveolar cleft reconstruction in the period

  2. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

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    Buket Uysal Aladag

    2013-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate. A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atrophy, hair (sparse hair and eye brows, teeth (small, absent or dysplastic teeth, nails (nail dystrophy and exocrine glands (reduction/ absence of sweat, sebaceous and salivary glands. A multidisciplinary approach for treatment is needed which is co-ordinated by orthopedic, plastic, dental surgeons, ophthalmologist, dermatologists and speech therapists, psychologists. We presented EEC syndrome case with hydrocephaly by the literature. [Cukurova Med J 2013; 38(3.000: 531-535

  3. Cleft rhinoplasty.

    Science.gov (United States)

    Baskaran, M; Packiaraj, I; Arularasan, S Gidean; Divakar, T K

    2015-08-01

    It is universally accepted that correction of cleft lip nose deformity remains a formidable challenge for any cleft surgeon. The nose is a prominent part of the face, and hence a masterly executed cleft lip repair directs the beholders' eyes from the deformed lip to the deformed nose. A deformed nose that results from unilateral cleft of the lip and palate is likened to a tent whose one side is depressed. Many investigators believe that the deformity of the nose is produced by the malpositioning of essentially normal structures, on the other hand some cleft surgeons contend that it is the intrinsic defects in nasal structures that result in cleft nasal deformity. Depressed and hypoplastic bony scaffolding is the most important aspect of cleft nose deformity and addressing this aspect of cleft nose deformity is the secret of success of a perfect secondary rhinoplasty. Controversy still exists on timing of cleft nasal deformity. Proponents of delayed nasal repair suggest that altering the cartilages in early nasal repair at the time of lip repair would complicate future corrective nasal surgeries if the primary repair would prove unsatisfactory. The correction of nasal deformity could be performed with closed or open technique. This paper highlights one such challenging unilateral cleft lip nasal deformity in a adult patient treated by secondary rhinoplasty by open technique. PMID:26538945

  4. Cone-Beam Computed Tomography Assessment of Lower Facial Asymmetry in Unilateral Cleft Lip and Palate and Non-Cleft Patients with Class III Skeletal Relationship.

    Directory of Open Access Journals (Sweden)

    Yifan Lin

    Full Text Available To evaluate, using cone-beam computed tomography (CBCT, both the condylar-fossa relationships and the mandibular and condylar asymmetries between unilateral cleft lip and palate (UCLP patients and non-cleft patients with class III skeletal relationship, and to investigate the factors of asymmetry contributing to chin deviation.The UCLP and non-cleft groups consisted of 30 and 40 subjects, respectively, in mixed dentition with class III skeletal relationships. Condylar-fossa relationships and the dimensional and positional asymmetries of the condyles and mandibles were examined using CBCT. Intra-group differences were compared between two sides in both groups using a paired t-test. Furthermore, correlations between each measurement and chin deviation were assessed.It was observed that 90% of UCLP and 67.5% of non-cleft subjects had both condyles centered, and no significant asymmetry was found. The axial angle and the condylar center distances to the midsagittal plane were significantly greater on the cleft side than on the non-cleft side (P=0.001 and P=0.028, respectively and were positively correlated with chin deviation in the UCLP group. Except for a larger gonial angle on the cleft side, the two groups presented with consistent asymmetries showing shorter mandibular bodies and total mandibular lengths on the cleft (deviated side. The average chin deviation was 1.63 mm to the cleft side, and the average absolute chin deviation was significantly greater in the UCLP group than in the non-cleft group (P=0.037.Compared with non-cleft subjects with similar class III skeletal relationships, the subjects with UCLP showed more severe lower facial asymmetry. The subjects with UCLP presented with more asymmetrical positions and rotations of the condyles on axial slices, which were positively correlated with chin deviation.

  5. Prenatal Counseling, Ultrasound Diagnosis, and the Role of Maternal-Fetal Medicine of the Cleft Lip and Palate Patient.

    Science.gov (United States)

    James, Jeffrey N; Schlieder, Daniel W

    2016-05-01

    A multidisciplinary team is the standard of care and the cornerstone of management of cleft patients. With readily improving advanced diagnostic modalities, early prenatal diagnosis of cleft lip and palate increasingly becomes a topic of importance for both the team caring for and families of cleft patients. Maternal-fetal medicine is a fellowship subspecialty of obstetrics that can offer high-quality care and coordination to the cleft team. Both 3-D and 4-D sonography lead to early prenatal diagnosis of cleft patients; however, differences in training result in variations in its diagnostic accuracy. PMID:26928557

  6. Strategies for treating compensatory articulation in patients with cleft palate.

    Science.gov (United States)

    Del Carmen Pamplona, Maria; Ysunza, Antonio; Morales, Santiago

    2014-03-01

    Patients with cleft palate frequently show compensatory articulation (CA). CA requires a prolonged period of speech intervention. Some scaffolding strategies can be useful for correcting placement and manner of articulation in these cases. The purpose of this paper was to study whether the use of specific strategies of speech pathology can be more effective if applied according to the level of severity of CA. Ninety patients with CA were studied in two groups. One group was treated using strategies specific for their level of severity of articulation, whereas in the other group all strategies were used indistinctively. The degree of severity of CA was compared at the end of the speech intervention. After the speech therapy intervention, the group of patients in which the strategies were used selectively, showed a significantly greater decrease in the severity of CA, as compared with the patients in whom all the strategies were used indistinctively. An assessment of the severity of CA can be useful for selecting the strategies, which can be more effective for correcting the compensatory errors. PMID:24711749

  7. Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia.

    Science.gov (United States)

    Karthikeyani, Shanmugasundaram; Thirumurthy, Velliangattur Ramasamy; Yuvaraja, Bindhoo A

    2016-01-01

    Rapp-Hodgkin syndrome (RHS) is a rare type of autosomal dominant disorder characterized by association of ectodermal dysplasia (ED) with cleft lip/palate. The main features include dry, brittle hair with alopecia in adulthood, dental anomalies (hypodontia, microdontia with delayed eruption, fissured tongue, and retruded maxilla), hypohidrosis, dysplastic nails, and clefting. Palmar-plantar keratoderma is seen frequently. RHS has signs and symptoms that overlap considerably with those of ankyloblepharon-ED-clefting syndrome and ectrodactyly-ED-clefting syndrome. This manuscript discusses a case of RHS, one of the four members in three generations who had ED with variable degree of involvement of hair, teeth, nail, and sweat glands. PMID:27080974

  8. DENTAL CAST STUDY OF ADULT PATIENTS WITH UNTREATED UNILATERAL CLEFT-LIP OR CLEFT-LIP AND PALATE IN INDONESIA COMPARED WITH SURGICALLY TREATED PATIENTS IN THE NETHERLANDS

    NARCIS (Netherlands)

    SPAUWEN, PHM; HARDJOWASITO, W; BOERSMA, J; LATIEF, BS

    1993-01-01

    To determine differences in maxillary and dentoalveolar relationships between untreated and treated patients having unilateral clefts of the lip and alveolus (UCLA) or lip and palate (UCLP), dental cast assessments were done on 70 untreated adult Indonesian patients (UCLA-I, UCLP-I) and 67 Dutch pat

  9. Dual embryonic origin of maxillary lateral incisors: clinical implications in patients with cleft lip and palate

    Science.gov (United States)

    Garib, Daniela Gamba; Rosar, Julia Petruccelli; Sathler, Renata; Ozawa, Terumi Okada

    2015-01-01

    Introduction: Cleft lip and palate are craniofacial anomalies highly prevalent in the overall population. In oral clefts involving the alveolar ridge, variations of number, shape, size and position are observed in maxillary lateral incisors. The objective of this manuscript is to elucidate the embryonic origin of maxillary lateral incisors in order to understand the etiology of these variations. Contextualization: The hypothesis that orofacial clefts would split maxillary lateral incisor buds has been previously reported. However, recent studies showed that maxillary lateral incisors have dual embryonic origin, being partially formed by both the medial nasal process and the maxillary process. In other words, the mesial half of the lateral incisor seems to come from the medial nasal process while the distal half of the lateral incisor originates from the maxillary process. In cleft patients, these processes do not fuse, which results in different numerical and positional patterns for lateral incisors relating to the alveolar cleft. In addition to these considerations, this study proposes a nomenclature for maxillary lateral incisors in patients with cleft lip and palate, based on embryology and lateral incisors position in relation to the alveolar cleft. Conclusion: Embryological knowledge on the dual origin of maxillary lateral incisors and the use of a proper nomenclature for their numerical and positional variations renders appropriate communication among professionals and treatment planning easier, in addition to standardizing research analysis. PMID:26560830

  10. Dual embryonic origin of maxillary lateral incisors: clinical implications in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Daniela Gamba Garib

    2015-10-01

    Full Text Available Introduction:Cleft lip and palate are craniofacial anomalies highly prevalent in the overall population. In oral clefts involving the alveolar ridge, variations of number, shape, size and position are observed in maxillary lateral incisors. The objective of this manuscript is to elucidate the embryonic origin of maxillary lateral incisors in order to understand the etiology of these variations.Contextualization: The hypothesis that orofacial clefts would split maxillary lateral incisor buds has been previously reported. However, recent studies showed that maxillary lateral incisors have dual embryonic origin, being partially formed by both the medial nasal process and the maxillary process. In other words, the mesial half of the lateral incisor seems to come from the medial nasal process while the distal half of the lateral incisor originates from the maxillary process. In cleft patients, these processes do not fuse, which results in different numerical and positional patterns for lateral incisors relating to the alveolar cleft. In addition to these considerations, this study proposes a nomenclature for maxillary lateral incisors in patients with cleft lip and palate, based on embryology and lateral incisors position in relation to the alveolar cleft.Conclusion:Embryological knowledge on the dual origin of maxillary lateral incisors and the use of a proper nomenclature for their numerical and positional variations renders appropriate communication among professionals and treatment planning easier, in addition to standardizing research analysis.

  11. Evaluation of the cephalometric changes of Tweed triangle in patients with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Mohsen Shirazi

    2015-12-01

    Full Text Available Background and Aims: Cleft lip and palate patients require orthodontic treatments during their childhood and adolescence. Tweed diagnostic triangle as well as cephalometric assessments provides important data regarding the skeletal patterns for the treatment and diagnostic purposes. The present study determined the cephalometric changes of Tweed triangle in the cleft lip and palate patients compared to normal patients. Materials and Methods: In total, 101 cleft and palate patients as well as 95 normal individuals with the balanced age, gender and race were evaluated. All the cleft and palate patients had similar treatment histories. Radiographic clichés were obtained from both groups and the images were traced after identifying the anatomic landmarks. The studied landmarks included points, lines, and plans as well as dental and skeletal angles and distance ratios measured in radiographic images. The landmarks were statistically analyzed using Student t test. Results: Significant differences were found between the cleft lip and palate patients and normal individuals regarding craniofacial complex morphology (P0.05. Conclusion: In total, significant differences were observed between normal and cleft lip and palate individuals regarding Tweed diagnostic triangle area. These differences were decreased IMPA and increased FMA and FMIA angles. No significant differences were found in terms of other indices.

  12. Alveolar distraction osteogenesis applications in cleft lip and palate patients: a literature review

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    Öznur Mülayim

    2016-05-01

    Full Text Available Distraction osteogenesis technique has been successfully applied in the craniofacial area for long time, and it is being applied increasingly more in cleft lip and palate patients also. Especially in large cleft palate cases, bone grafting or surgical procedures such as distraction osteogenesis can be applied in order to ensure a smooth alveolar arc. In this literature review, alveolar (segmental distraction osteogenesis applications in patients with cleft lip and palate, indications of the technique, advantages and disadvantages, application methods and types of appliances used for this purpose have been evaluated. As conclusion, especially with the application of alveolar (segmental distraction osteogenesis, successful outcome can be achieved in cleft lip and palate patients with velopharyngeal insufficiency, maxillary hypoplasia and maxillary crowding, and this technique may be an alternative to conventional osteotomies and extraoral distractions.

  13. Electrophysiological assessment of auditory processing disorder in children with non-syndromic cleft lip and/or palate

    Science.gov (United States)

    McPherson, Bradley; Ma, Lian

    2016-01-01

    Objectives Cleft lip and/or palate is a common congenital craniofacial malformation found worldwide. A frequently associated disorder is conductive hearing loss, and this disorder has been thoroughly investigated in children with non-syndromic cleft lip and/or palate (NSCL/P). However, analysis of auditory processing function is rarely reported for this population, although this issue should not be ignored since abnormal auditory cortical structures have been found in populations with cleft disorders. The present study utilized electrophysiological tests to assess the auditory status of a large group of children with NSCL/P, and investigated whether this group had less robust central auditory processing abilities compared to craniofacially normal children. Methods 146 children with NSCL/P who had normal peripheral hearing thresholds, and 60 craniofacially normal children aged from 6 to 15 years, were recruited. Electrophysiological tests, including auditory brainstem response (ABR), P1-N1-P2 complex, and P300 component recording, were conducted. Results ABR and N1 wave latencies were significantly prolonged in children with NSCL/P. An atypical developmental trend was found for long latency potentials in children with cleft compared to control group children. Children with unilateral cleft lip and palate showed a greater level of abnormal results compared with other cleft subgroups, whereas the cleft lip subgroup had the most robust responses for all tests. Conclusion Children with NSCL/P may have slower than normal neural transmission times between the peripheral auditory nerve and brainstem. Possible delayed development of myelination and synaptogenesis may also influence auditory processing function in this population. Present research outcomes were consistent with previous, smaller sample size, electrophysiological studies on infants and children with cleft lip/palate disorders. In view of the these findings, and reports of educational disadvantage associated

  14. Bony defect of palate and vomer in submucous cleft palate patients.

    Science.gov (United States)

    Ren, S; Ma, L; Zhou, X; Sun, Z

    2015-01-01

    The aim of this study was to visualize bony defects of the palate and vomer in submucous cleft palate patients (SMCP) by three-dimensional (3D) computed tomography (CT) reconstruction and to classify the range of bony defects. Forty-eight consecutive non-operated SMCP patients were included. Diagnosis was based on the presence of at least one of three classical signs of SMCP: bifid uvula, a translucent zone in the midline of the soft palate, and a palpable 'V' notch on the posterior border of the bony palate. Patients were imaged using spiral CT. 3D reconstruction models were created of the palate and vomer. The sagittal extent of the bony cleft in SMCP was classified into four types: type I, no V-shaped hard palate cleft (8.3%); type II, cleft involving the partial palate (43.8%); type III, cleft involving the complete palate and extending to the incisive foramen (43.8%); type IV, cleft involving the complete palate and the alveolar bone (4.2%). The extent of the vomer defect was classified into three types: type A, vomer completely fused with the palate (8.3%); type B, vomer partially fused with the palate (43.8%); type C, vomer not fused with the palate up to the incisive foramen (47.9%). Significant variability in hard palate defects in SMCP is the rule rather than the exception. The association of velopharyngeal insufficiency with anatomical malformations may be complex.

  15. Value of acoustic perceptual method for analysis of compensatory articulation errors in postoperative patients with cleft palate

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective To establish an acoustic perceptual method analyzing the compensatory articulation errors in children with operated cleft palate via the formants of Chinese pure vowels. Methods The first three formants which represent vocal transmission character in Chinese pure vowels of 84 subjects with operated cleft palate, were measured by Computerized Speech Signal Processing System (CSSPS). The Chinese vowel graph of postoperative patients with cleft palate was stated by the first formant frequencies (F1) ...

  16. Pre: Surgical orthopedic pre-maxillary alignment in bilateral cleft lip and palate patient

    Directory of Open Access Journals (Sweden)

    Vijaya Prasad Kamavaram Ellore

    2012-01-01

    Full Text Available Pre-surgical orthopedic appliances are mainly used to retract and align the protruded and deviated pre-maxilla and to facilitate initial lip repair. This article presents a case report of a five year old male child patient with bilateral cleft lip and palate in whom a special custom made pre-surgical orthopedic appliance was delivered. Use of a special custom made presurgical orthopedic appliance for repositioning pre-maxilla in bilateral cleft lip and palate patient is discussed in this article.

  17. Current status of presurgical infant orthopaedic treatment for cleft lip and palate patients: A critical review

    Directory of Open Access Journals (Sweden)

    P Priyanka Niranjane

    2014-01-01

    Full Text Available Rehabilitation of cleft lip and palate (CLP patients is a challenge for all the concerned members of the cleft team, and various treatment modalities have been attempted to obtain aesthetic results. Presurgical infant orthopaedics (PSIO was introduced to reshape alveolar and nasal segments prior to surgical repair of cleft lip. However, literature reports lot of controversy regarding the use of PSIO in patients with CLP. Evaluation of long-term results of PSIO can provide scientific evidence on the efficacy and usefulness of PSIO in CLP patients. The aim was to assess the scientific evidence on the efficiency of PSIO appliances in patients with CLP and to critically analyse the current status of PSIO. A PubMed search was performed using the terms PSIO, presurgical nasoalveolar moulding and its long-term results and related articles were selected for the review. The documented studies report no beneficial effect of PSIO on maxillary arch dimensions, facial aesthetics and in the subsequent development of dentition and occlusion in CLP patients. Nasal moulding seems to be more beneficial and effective in unilateral cleft lip and palate patients with better long-term results.

  18. ANATOMICAL VARIATIONS FINDINGS ON CONE BEAM-COMPUTED TOMOGRAPHY IN CLEFT LIP AND PALATE PATIENTS

    Directory of Open Access Journals (Sweden)

    Yllka DECOLLI

    2015-09-01

    Full Text Available Introduction: Cone beam computed tomography (CBCT is frequently used in surgery treatment planning in patients with cleft lip and palate (CLP. The aim of this study was to investigate the presence of different anatomical variations of patients with cleft lip and palate using CBCT images. Materials and method: CBCTs taken from consecutive patients (n =25; mean age 10.7±4 years, range 6.5–23 years with a non-syndromic cleft lip and palate (CLP, between June 2014-2015, were systematically evaluated. Sinuses, nasopharynx, oropharynx, hypopharynx, temporo-mandibular joint (TMJ, maxilla and mandible were checked for incidental findings. Results: On 90.1 % of the CBCTs, incidental findings were found. The most prevalent ones were airway/sinus findings (78.1%, followed by dental problems, e.g. missing teeth (54%, nasal septum deviation (93%, middle ear and mastoid opacification, suggestive for otitis media (8% and (chronic mastoiditis (7%, abnormal TMJ anatomy (4.3%. Conclusions: Incidental findings are common on CBCTs in cleft lip and palate patients. Compared with the literature, CLP patients have more dental, nasal and ear problems. The CBCT scan should be reviewed by all specialists in the CLP team, stress being laid on their specific background knowledge concerning symptoms and treatment of these patients.

  19. Assessment of middle ear function in patients with cleft palate treated under "Smile Train Project"

    Directory of Open Access Journals (Sweden)

    Prachi Gautam

    2011-01-01

    Full Text Available Objective: To evaluate the middle ear function in patients of cleft palate treated under "Smile Train Project". Materials and Methods: Sixty patients (120 ears; 32 male and 28 female of cleft palate with or without cleft lip were evaluated in pediatrics and ENT OPD in preoperative period. A general medical check up was performed in pediatrics OPD to evaluate the associated craniofacial anomalies. Middle ear function was evaluated in ENT OPD. Otoscopy was done in all patients. Cases under five years of age were evaluated for middle ear function by tympanometry and cases with more than five years of age were evaluated by tympanometry for middle ear function and pure tone audiometry to establish the type of hearing loss. The results are presented as number of ears. Results: A total of 50% of the patients in the study group were in zero- to two-year age group. Otoscopy findings in 120 ears showed that 66.66% of the patients had dull tympanic membrane suggesting a diagnosis of otitis media with effusion. Tympanometry was done in 110 ears, of which, 72.72% of ears had type B tympanogram suggesting otitis media with effusion. Pure tone audiometry in more than five years age group showed that 81.25% ears had conductive hearing loss. Conclusion: This study suggests the need of preoperative ENT check up and audiological assessment in patients of cleft palate undergoing palatal surgery under "Smile Train Project". The association of otitis media with effusion in patients with cleft palate appears strong and a high degree of suspicion for the disease should be kept in them. The anatomical defect and possible causative pathophysiology also supports the inference. Otitis media with effusion causing hearing loss in these patients causes speech delay and unfavorable outcome despite aggressive speech therapy in postoperative period. A co-ordinate team approach of concerned specialists involved in managing these patients would definitely improve their outcome.

  20. Influence of palatoplasty on occlusion of patients with isolated cleft palate

    Directory of Open Access Journals (Sweden)

    Omar Gabriel da Silva Filho

    2012-04-01

    Full Text Available OBJECTIVE: This study compared the dental arch morphology of adult patients with isolated cleft palate in order to verify the influence of palatoplasty on occlusion. METHODS: Cast models of 77 patients, 30 males and 47 females, with an average age of 21 years and no syndromes were taken. They were in the permanent dentition and had not undergone orthodontic treatment. The sample was divided into non-operated and operated patients, the latter having been submitted to palatoplasty at a mean age of 2.2 years. RESULTS: Almost 80% of the sample exhibited sagittal discrepancies in the inter-arch relationship, with a Class II malocclusion prevailing (59.74% followed by Class III (20,78%, regardless of palatoplasty. Transverse analysis showed a 23% incidence of posterior crossbite also not influenced by palatoplasty. Intra-arch relationship indicated that constriction and crowding on the upper arch were more frequent in the operated group (p=0.0238 and p=0.0002, respectively, showing an influence of palatoplasty on its morphology. The predominant morphological characteristics in patients with isolated cleft palate were a Class II malocclusion, upper dental arch constriction and upper and lower anterior crowding. CONCLUSION: The influence of palatoplasty was restricted to constriction and crowding of the upper dental arch, with no interference from the extension of the cleft, except for the upper crowding, which occurred more in patients with complete cleft palates.

  1. Skeletal facial balance and harmony in the cleft patient: Principles and techniques in orthognathic surgery

    Directory of Open Access Journals (Sweden)

    Salyer Kenneth

    2009-10-01

    Full Text Available The management of the palatal cleft, dental arch, and subsequent maxillary form is a challenge for the craniomaxillofacial surgeon. The purpose of this paper is to present the experience of a senior surgeon (KES who has treated over 2000 patients with cleft lip and palate. This paper focuses on the experience of a recent series of 103 consecutive orthognathic cases treated by one surgeon with a surgical-orthodontic, speech-oriented approach. It will concentrate on not only correcting the occlusion, as others have described, but also on how a surgeon who was trying to achieve optimal aesthetic balance, harmony, and beauty, approached this problem.

  2. Orthognathic Surgery for Achieving Facial Balance and Harmony in Cleft Patients

    Institute of Scientific and Technical Information of China (English)

    Kenneth E Salyer; XU Hai-song

    2015-01-01

    Cleft surgery requires an expert team performing ongoing treatment in order to achieve optimal outcomes. The senior author's (KES) experiences of more than 2 000 patients with cleft lip and palate treated by a surgical-orthodontic protocol were introduced. The paper here will concentrate on not only correcting the occlusion as others have described, but also on what one surgeon can do to achieve optimal aesthetic balance, harmony and beauty. The results of orthognathic surgery in respect to function, stability, cosmesis, and complications are also audited.

  3. Clinical aspects of type 1 posterior laryngeal clefts: literature review and a report of 31 patients.

    NARCIS (Netherlands)

    Doef, H.P. van der; Yntema, J.L.; Hoogen, F.J.A. van den; Marres, H.A.M.

    2007-01-01

    OBJECTIVES: The clinical relevance of posterior laryngeal clefts (PLCs) of type 1 has only recently been highlighted in the medical literature. We present a review of the literature and a report of a contributive series of 31 type 1 PLC patients. STUDY DESIGN AND METHODS: We performed a MEDLINE sear

  4. Clinical Analysis of Treatment Effects of Postoperative Deformities of Cleft Lip and Palate with Orthodontics and Orthognathics%正畸正颌联合治疗唇腭裂术后牙颌面畸形的疗效观察

    Institute of Scientific and Technical Information of China (English)

    冯晓东; 张春蕾; 蒋子超

    2014-01-01

    目的:探讨正畸、正颌外科联合治疗唇腭裂术后牙颌面畸形的临床疗效。方法:选择2001—2012年,我院收治的唇腭裂术后牙颌面畸形患者56例,采用正颌联合正畸的方法进行治疗。随访6~24个月,观察牙颌面畸形改善情况。结果:56例患者面形改善明显,咬合关系良好,随访无复发。比较手术前后的头影测量值进行分析,差异具有统计学意义(P<0.05)。结论:正颌外科联合术前、术后正畸,可明显改善面部畸形,减少术后复发,是治疗唇腭裂术后牙颌面畸形的理想手术方式。%Objective: To investigate the clinical efficacy of orthodontics, orthognathic surgery combined treatment of postoperative maxillofacial deformities in cleft lip/palate patients. Methods:From 2001-07—2012-07, 56 cases were retro-spectively reviewed. Orthognathic surgery and orthodontics measures were used to correct the postoperative deformities and followed-up for 6~24 months. Results: Cephalometric comparison before and after operation were analyzed, the difference was statistically significant (P<0.05). 56 patients' facial appearance improved significantly, whose normal occlusion were established and without recurrences. Conclusion: Orthognathic surgery with preoperative and postoperative orthodontic treatment can obviously improve the postoperative facial deformities in cleft lip/palate patients.

  5. APR-246/PRIMA-1(MET) rescues epidermal differentiation in skin keratinocytes derived from EEC syndrome patients with p63 mutations.

    Science.gov (United States)

    Shen, Jinfeng; van den Bogaard, Ellen H; Kouwenhoven, Evelyn N; Bykov, Vladimir J N; Rinne, Tuula; Zhang, Qiang; Tjabringa, Geuranne S; Gilissen, Christian; van Heeringen, Simon J; Schalkwijk, Joost; van Bokhoven, Hans; Wiman, Klas G; Zhou, Huiqing

    2013-02-01

    p53 and p63 share extensive sequence and structure homology. p53 is frequently mutated in cancer, whereas mutations in p63 cause developmental disorders manifested in ectodermal dysplasia, limb defects, and orofacial clefting. We have established primary adult skin keratinocytes from ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome patients with p63 mutations as an in vitro human model to study the disease mechanism in the skin of EEC patients. We show that these patient keratinocytes cultured either in submerged 2D cultures or in 3D skin equivalents have impaired epidermal differentiation and stratification. Treatment of these patient keratinocytes with the mutant p53-targeting compound APR-246/PRIMA-1(MET) (p53 reactivation and induction of massive apoptosis) that has been successfully tested in a phase I/II clinical trial in cancer patients partially but consistently rescued morphological features and gene expression during epidermal stratification in both 2D and 3D models. This rescue coincides with restoration of p63 target-gene expression. Our data show that EEC patient keratinocytes with p63 mutations can be used for characterization of the abnormal molecular circuitry in patient skin and may open possibilities for the design of novel pharmacological treatment strategies for patients with mutant p63-associated developmental abnormalities. PMID:23355676

  6. Influence of palatoplasty on occlusion of patients with isolated cleft palate

    OpenAIRE

    Omar Gabriel da Silva Filho; Elisa Teixeira Marinho; Rita de Cássia Moura Carvalho Lauris

    2012-01-01

    OBJECTIVE: This study compared the dental arch morphology of adult patients with isolated cleft palate in order to verify the influence of palatoplasty on occlusion. METHODS: Cast models of 77 patients, 30 males and 47 females, with an average age of 21 years and no syndromes were taken. They were in the permanent dentition and had not undergone orthodontic treatment. The sample was divided into non-operated and operated patients, the latter having been submitted to palatoplasty at a mean age...

  7. Prospective Analysis of Secondary Alveolar Bone Grafting in Cleft Lip and Palate Patients

    Science.gov (United States)

    Reddy, M Gokul Chandra; Babu, V Ramesh; Rao, V Eswar; Chaitanya, J Jaya; Allareddy, S; Reddy, C Charan Kumar

    2015-01-01

    Background: To assess the success of the uptake of bone graft in cleft alveolus of the cleft lip and palate patients, quantitatively through computed tomography (CT) scan 6 months postoperative. To assess the successful eruption of permanent lateral incisor or canine in the bone grafted area. Materials and Methods: The children age group of 9-21 years with unilateral cleft lip and palate came to the hospital, needing secondary alveolar bone grafting. A detailed history and clinical examination of the patient was taken. A 3D CT scan was taken and the volume of the cleft was measured pre-operatively. After ambulatory period, 3D CT scan of the alveolar cleft region was taken and volume of the bone grafted was measured and patient was discharged from the hospital. After 6 months, patient was recalled and again 3D CT scan was taken and the volume of remaining bone was measured. Results: The mean volume of the defect pre-operatively is 0.80 cm3 with a standard deviation of 0.36 cm3 with minimum volume of the defect 0.44 cm3 and maximum volume of the defect 1.60 cm3. The mean volume of the bone post-operative immediately after grafting is 1.01 cm3 with a standard deviation of 0.52 cm3 with minimum of bone volume is 0.48 cm3 and maximum of 2.06 cm3. The mean volume of the bone after 6 months after bone grafting is 0.54 cm3 with a standard deviation of 0.33 cm3, minimum bone volume of 0.22 cm3 and maximum bone volume of 1.42 cm3. Conclusion: The CT scan is a valuable radiographic imaging modality to assess and follow the clinical outcome of secondary alveolar bone grafting. PMID:25954076

  8. Bilateral acute visual loss from Rathke's cleft cyst apoplexy in a patient with dengue fever

    Directory of Open Access Journals (Sweden)

    Ana Cláudia De Franco Suzuki

    2014-10-01

    Full Text Available Hemorrhagic complications of optic pathway diseases are extremely rare causes of acute visual loss associated with dengue fever. In this paper we report a patient presenting with dengue fever and bilateral acute visual loss caused by chiasmal compression due to Rathke's cleft cyst apoplexy. Considering the importance of early diagnosis and treatment to visual recovery, apoplexy of sellar and suprasellar tumors should be considered in the differential diagnosis of patients with acute visual loss and dengue fever.

  9. Velopharyngeal Insufficiency Rates After Delayed Cleft Palate Repair: Lessons Learned From Internationally Adopted Patients.

    Science.gov (United States)

    Follmar, Keith E; Yuan, Nance; Pendleton, Courtney S; Dorafshar, Amir H; Kolk, Craig Vander; Redett, Richard J

    2015-09-01

    Most surgeons recommend cleft palate repair between 6 and 12 months of age. Internationally adopted patients often undergo delayed repair due to social circumstances. There are few outcomes studies on this population. We conducted a 13-year retrospective review of all patients undergoing primary cleft palate repair at a single tertiary-care academic medical center between 1993 and 2006. The primary outcome was velopharyngeal insufficiency, defined as the recommendation for corrective surgery after multiple formal speech assessments. Two hundred one patients (102 males and 99 females) were identified. One hundred eighty-three repairs were performed before 18 months of age (standard repair group). Eighteen repairs were performed after 18 months of age (delayed repair group), with international adoption being a circumstance in 16 cases. The delayed and standard repair groups were similar with regard to sex, presence of craniofacial syndrome, Veau class, cleft size and laterality, type of repair, and operating surgeon. Mean follow-up was 9.3 years, with minimum follow-up of 5.0 years. Six (33%) of 18 patients in the delayed repair group developed velopharyngeal insufficiency compared to 23 (13%) of 183 in the standard repair group (P = 0.03 by Fisher exact test). These data demonstrate that internationally adopted patients undergoing delayed palate repair suffer especially poor speech outcomes. Physiologic differences in patients undergoing late repair, as well as social factors including adaptation to a new language and culture, may be factors. Early repair should be performed when possible. PMID:25046662

  10. 3-D shape analysis of palatal surface in patients with unilateral complete cleft lip and palate.

    Science.gov (United States)

    Rusková, Hana; Bejdová, Sárka; Peterka, Miroslav; Krajíček, Václav; Velemínská, Jana

    2014-07-01

    Facial development of patients with unilateral complete cleft lip and palate (UCLP) is associated with many problems including deformity of the palate. The aim of this study was to evaluate palatal morphology and variability in patients with UCLP compared with Czech norms using methods of geometric morphometrics. The study was based on virtual dental cast analysis of 29 UCLP patients and 29 control individuals at the age of 15 years. The variability of palatal shape in UCLP patients was greater than that in nonclefted palates. Only 24% of clefted palates fell within the variability of controls. The palatal form of UCLP patients (range from 11.8 to 17.2 years) was not correlated with age. Compared with control palates, palates of UCLP patients were narrower, more anteriorly than posteriorly. Apart from the praemaxilla region, they were also shallower, and the difference increased posteriorly. The UCLP palate was characterised by the asymmetry of its vault. The maximum height of the palatal vault was anterior on the clefted side, whereas it was posterior on the nonclefted side. The slope of the UCLP palate was more inclined compared with the control group. The praemaxilla was therefore situated more inferiorly.

  11. Risk of leukemia in first degree relatives of patients with nonsyndromic cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Eduardo GONÇALVES

    2014-01-01

    Full Text Available The aim of this study was to determine the frequency of leukemia in parents of patients with nonsyndromic cleft lip and/or cleft palate (NSCL/P. This case-control study evaluated first-degree family members of 358 patients with NSCL/P and 1,432 subjects without craniofacial alterations or syndromes. Statistical analysis was carried out using Fisher’s test. From the 358 subjects with NSCL/P, 3 first-degree parents had history of leukemia, while 2 out of 1,432 subjects from the unaffected group had a family history of leukemia. The frequency of positive family history of leukemia was not significantly increased in first-degree relatives of patients with NSCL/P.

  12. The nance obturator, a new fixed obturator for patients with cleft palate and fistula.

    Science.gov (United States)

    Borzabadi-Farahani, Ali; Groper, John N; Tanner, Aaron M; Urata, Mark M; Panossian, Andre; Yen, Stephen L-K

    2012-07-01

    In patients with fistulas that impair function (e.g., feeding, resonance, intelligibility), obturators are used to improve feeding and reduce nasal air emission by occluding the abnormal opening between the oral and nasal cavities. This report describes a novel method for occluding an anterior palatal fistula in patients with cleft palates. The new design for a fixed obturator is based on the Nance appliance, which was originally used as a space maintainer, but has been redesigned for closing an anterior palatal fistula in a patient with cleft lip and palate. The Nance obturator may be used when the surgical closure of the fistula is not feasible and a removable device is not successful. As it is a fixed device, it does not require remaking with maxillary growth. The new design may also function as a fixed space maintainer to preserve molar anchorage and maxillary transverse width.

  13. Preparation of Plaster Moulage (Cast in Plastic Surgery patients

    Directory of Open Access Journals (Sweden)

    Rana R

    2003-01-01

    Full Text Available The purpose of this paper is to describe the technique of making casts using alginate compound for negative and dental stone plaster for positive impressions. With certain modifications a cast could be made of any part of the body and one can make a museum of interesting cases. Casts serve as useful teaching material especially in cleft lip and palate patients to study the effect of surgery on growth and development of the cleft lip-palate-nose complex in relation to the remaining face. It also helps in planning reconstruction in cases of facial defects, recording serial changes in multistage surgery, pre-operative and post-operative comparison as in rhinoplasty, ear reconstruction, hand etc; for comparing results before and after treatment in keloid and hypertrophic scars, fabrication of implants and preparation of prosthesis. In spite of newer modalities like 3-D imaging and stereolithography, the usefulness of this old technique in certain interesting cases can not be denied.

  14. Closure of Huge Palatal Fistula in an Adult Patient with Isolated Cleft Palate: A Technical Note

    OpenAIRE

    Amin Rahpeyma, DDS; Saeedeh Khajehahmadi, DDS

    2015-01-01

    Summary: Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and th...

  15. Inferior Turbinate Flap for Nasal-side Closure of Palatal Fistula in Cleft Patients: Technical Note

    Directory of Open Access Journals (Sweden)

    Amin Rahpeyma

    2014-12-01

    Full Text Available Summary: Residual palatal fistula after repair of palatal cleft is common. Repair of residual oronasal fistula is not always successful. Two-layer closure techniques that close these fistulas with soft tissue are a common practice. Turnover flaps are the most used flaps and often the sole method for nasal-side closure of fistula. Anteriorly based inferior turbinate flap can be used to provide soft tissue for nasal-side closure when turnover flaps will not provide sufficient tissue for this purpose. Under general anesthesia with nasotracheal intubation, inferior turbinate was released from posterior attachment. After removing the inferior conchal bone, mucoperiosteal flap was used for nasal-side closure of anterior palatal fistula in patients with cleft. Anteriorly based inferior turbinate flap was used for nasal-side closure of residual palatal fistula in 3 patients with cleft. Age of the patients was 14, 16, and 18, and recurrence of palatal fistula has not occurred. Anteriorly based inferior turbinate flap is an axial pattern flap with appropriate surface of the paddle and vicinity to the oral cavity roof. It can be used in large anterior, palatal fistula for reconstruction of nasal floor. Considering appropriate another flap for oral side coverage of such fistula is mandatory.

  16. Distraction osteogenesis and orthognathic surgery for a patient with unilateral cleft lip and palate.

    Science.gov (United States)

    Kim, Ji Hyun; Lee, Il Hong; Lee, Sang Min; Yang, Byoung Eun; Park, In Young

    2015-03-01

    Maxillary deficiency is a common feature in patients with repaired cleft lip and palate. Orthognathic surgery has been the conventional approach for the management of cleft-related maxillary hypoplasia. However, for patients with a severe maxillary deficiency, orthognathic surgery alone has many disadvantages, such as high relapse rates of 25% to 40%, instability, limited amount of advancement, and a highly invasive surgical technique. As an alternative treatment method, distraction osteogenesis has been used successfully in the distraction of the mandible, the maxilla, the entire midface, and the orbits as well as the cranial bones, with stable outcomes. The type of distraction device, either external or internal, can be chosen based on the surgical goals set for the patient. In this study, we report on the use of a rigid external distraction device for maxillary advancement in a 22-year-old woman with a repaired unilateral cleft lip and palate and severe maxillary hypoplasia. After the distraction osteogenesis, 2-jaw surgery was performed to correct the maxillary yaw deviation and the mandibular prognathism.

  17. Do patients with isolated Pierre Robin Sequence have worse outcomes after cleft palate repair: A systematic review.

    Science.gov (United States)

    Wan, Teng; Chen, Yang; Wang, Guomin

    2015-08-01

    Cleft palate repair for Pierre Robin Sequence (PRS) patients has always been a challenge for surgeons and anesthetists. The aim of this systematic review is to investigate the outcome of cleft palate repair for PRS patients compared with cleft palate-only patients. All papers published before October 2014 were searched in the databases PubMed and MEDLINE. Search terms included "Pierre Robin Sequence," "cleft palate repair," and "speech result." Additional studies were identified by hand searching the reference lists of the papers retrieved from electronic search. Two independent reviewers assessed the eligibility of studies for inclusion, extracted the data, and assessed the quality of the studies. Six studies met the inclusion criteria. All but one study had multiple deficiencies in study designs. Four studies assessed the fistula rate of both groups, and all studies assessed some aspect of the speech results. Conflicting results and a lack of high-quality and long-term outcomes of reviewed studies provided no conclusive scientific evidence about whether the outcome of cleft palate repair for PRS patients was better or worse than cleft palate-only patients. Further well-designed, well-controlled, and long-term studies are needed.

  18. Cleft lip and palate and related factors: A 10 years study in university hospitalised patients at Mashhad — Iran

    Science.gov (United States)

    Noorollahian, Morteza; Nematy, Mohsen; Dolatian, Atiyeh; Ghesmati, Hengameh; Akhlaghi, Saeed; Khademi, Gholam Reza

    2015-01-01

    Background: Oral-facial clefts including cleft lip and palate are the most common congenital malformations of the head and neck. Environmental factors such as maternal hormonal disorders, use of psychiatric medications, vitamin and folic acid deficiency, hypoxia, cigarette smoking and maternal obesity and overweight can affect the incidence of these disorders. In Iran, one of the associated problems is a lack of accurate statistics regarding the present status of the patients, which can cause a disturbance in the health programmes of Ministry of Health and Medical Education. The aim of this study was to report the status of 398 cases of cleft lip and palate in Sheikh and Imam Reza Hospitals of Mashhad over a 10-year period. Materials and Methods: This retrospective descriptive study was performed using data collection method and included the evaluation of the recorded files and completing the data forms. In this study, the file records of 398 patients referring to Mashhad Sheikh and Imam Reza (P.U.H) Hospitals were studied, from the beginning of 2002 to the end of 2011; the obtained data from the files were collected and classified. Results: The highest frequency was related to cleft palate alone (40.7%); frequencies were lower regarding the cleft lip and palate and cleft lip alone (34.41% and 24.87%, respectively). Approximately, half of the patients were from rural areas of the city and had articulation disorders. Most of the patients were the first-born children of the family and their parents were consanguineously married; about one-third of the patients had a family history of the disease. Conclusion: According to the results of the present study, cleft lip is more frequent in males and cleft palate is more prevalent in females; the obtained results are consistent with the global statistics. PMID:26712297

  19. Cleft lip and palate and related factors: A 10 years study in university hospitalised patients at Mashhad - Iran

    Directory of Open Access Journals (Sweden)

    Morteza Noorollahian

    2015-01-01

    Full Text Available Background: Oral-facial clefts including cleft lip and palate are the most common congenital malformations of the head and neck. Environmental factors such as maternal hormonal disorders, use of psychiatric medications, vitamin and folic acid deficiency, hypoxia, cigarette smoking and maternal obesity and overweight can affect the incidence of these disorders. In Iran, one of the associated problems is a lack of accurate statistics regarding the present status of the patients, which can cause a disturbance in the health programmes of Ministry of Health and Medical Education. The aim of this study was to report the status of 398 cases of cleft lip and palate in Sheikh and Imam Reza Hospitals of Mashhad over a 10-year period. Materials and Methods: This retrospective descriptive study was performed using data collection method and included the evaluation of the recorded files and completing the data forms. In this study, the file records of 398 patients referring to Mashhad Sheikh and Imam Reza (P.U.H Hospitals were studied, from the beginning of 2002 to the end of 2011; the obtained data from the files were collected and classified. Results: The highest frequency was related to cleft palate alone (40.7%; frequencies were lower regarding the cleft lip and palate and cleft lip alone (34.41% and 24.87%, respectively. Approximately, half of the patients were from rural areas of the city and had articulation disorders. Most of the patients were the first-born children of the family and their parents were consanguineously married; about one-third of the patients had a family history of the disease. Conclusion: According to the results of the present study, cleft lip is more frequent in males and cleft palate is more prevalent in females; the obtained results are consistent with the global statistics.

  20. An unusual type of sucking habit in a patient with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Satyaprasad Savitha

    2009-01-01

    Full Text Available Digit sucking, a form of non-nutritive sucking, is a habit of concern to specialist in various fields such as psychiatrist, psychologist, pediatricians, pediatric dentists, orthodontist, speech pathologist and plastic surgeon. The habits have harmful unbalanced pressures to be born by the immature highly malleable alveolar ridges. Sucking behaviors have long been recognized to affect occlusion and dental arch characteristics. As early as 1870s, Campbell and Chandler recognized that prolonged finger or thumb sucking habits had deleterious effects on certain occlusal traits including anterior open bite, increased over jet and class II canine and molar relationships. However, little is known about digit sucking habit and its effect in a cleft lip and palate child as there is no literature till now reported on the digit sucking in a cleft lip patient.

  1. Long-term outcome of secondary alveolar bone grafting in cleft lip and palate patients

    DEFF Research Database (Denmark)

    Meyer, Steffen; Pedersen, Kirsten Mølsted

    2013-01-01

    The objective was to assess the long-term outcome of secondary alveolar bone grafting (SABG) in cleft lip and palate patients and to examine relationships between preoperative and postoperative factors and overall long-term bone graft success. The records of 97 patients with cleft lip and palate......, who had secondary alveolar bone grafting of 123 alveolar clefts, were examined. Interalveolar bone height was assessed radiographically a minimum of 10 years after grafting using a 4-point scale (I-IV), where types I and II were considered a success. After an average follow-up of 16 years after SABG...... (range = 10.2-22.7 years), 101 of the 123 grafts (82%) were categorised as successes. Mean age in the success group was 12.1 years and 13.6 years in the failure group (p = 0.03). It was found that the success rate was significantly lower (p = 0.02) if SABG was performed after eruption of the tooth distal...

  2. Impaired epithelial differentiation of induced pluripotent stem cells from ectodermal dysplasia-related patients is rescued by the small compound APR-246/PRIMA-1MET

    OpenAIRE

    Shalom-Feuerstein, Ruby; Serror, Laura; Aberdam, Edith; Müller, Franz-Josef; Van Bokhoven, Hans; Wiman, Klas G.; Zhou, Huiqing; Aberdam, Daniel; PETIT Isabelle

    2013-01-01

    Ectodermal dysplasia is a group of congenital syndromes affecting a variety of ectodermal derivatives. Among them, ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome is caused by single point mutations in the p63 gene, which controls epidermal development and homeostasis. Phenotypic defects of the EEC syndrome include skin defects and limbal stem-cell deficiency. In this study, we designed a unique cellular model that recapitulated major embryonic defects related to EEC. ...

  3. Comprehensive orthodontic treatment of adult patient with cleft lip and palate.

    Science.gov (United States)

    Leiva Villagra, Noemí; Muñoz Domon, Miguel; Véliz Méndez, Sebastian

    2014-01-01

    The aim of the paper is to present full orthodontic treatment of an operated cleft lip adult patient. Case Report. An 18-year-old patient consulted for severe crowded teeth. He comes from a poor family. At that time he already had four operations (velum, palate, lip, and myringotomy). Treatment included maxillary expansion, tooth extraction, and fixed orthodontic, as well as kinesiology and speech therapy treatment. A multidisciplinary approach allowed us to achieve successfully an excellent result for this patient and gave him a harmonic smile and an optimal function without orthognathic surgery. Two years after treatment, occlusion remains stable.

  4. Comprehensive Orthodontic Treatment of Adult Patient with Cleft Lip and Palate

    Directory of Open Access Journals (Sweden)

    Noemí Leiva Villagra

    2014-01-01

    Full Text Available The aim of the paper is to present full orthodontic treatment of an operated cleft lip adult patient. Case Report. An 18-year-old patient consulted for severe crowded teeth. He comes from a poor family. At that time he already had four operations (velum, palate, lip, and myringotomy. Treatment included maxillary expansion, tooth extraction, and fixed orthodontic, as well as kinesiology and speech therapy treatment. A multidisciplinary approach allowed us to achieve successfully an excellent result for this patient and gave him a harmonic smile and an optimal function without orthognathic surgery. Two years after treatment, occlusion remains stable.

  5. External frame distraction osteogenesis of the midface in the cleft patient

    Directory of Open Access Journals (Sweden)

    Hussain Syed

    2009-10-01

    Full Text Available Distraction osteogenesis has established itself as an accepted form of treatment in the management of midface deficiency in cleft patients. However, it is well known that some amount of relapse is inevitable in patients who undergo this procedure. Like most surgical techniques, it has its specific indications, limitations, and complications. The problems are amplified in some patients because of severe fibrosis resulting from previous palate and lip operations. This article reviews treatment planning, pre- and postoperative orthodontic management, operative technique, and mechanics of distraction. It also discusses long-term changes following distraction and protocols to optimize the results and minimize complications.

  6. A Model Humanitarian Cleft Mission: 312 Cleft Surgeries in 7 Days

    Directory of Open Access Journals (Sweden)

    Ghulam Qadir Fayyaz, MBBS, DSS, MS

    2015-03-01

    Conclusion: A locoregional cleft team can be more effective to care for the patients with cleft in countries where surgical and other expertise can be utilized by proper organization of cleft missions on a national level.

  7. Treatment Approach for Maxillary Hypoplasia in Cleft Patients: Class III Elastics with Skeletal Anchorage (Report of Two Cases)

    Science.gov (United States)

    Jahanbin, Arezoo; Kazemian, Mozhgan; Saeedi-Pouya, Iman; Eslami, Neda; Shafaee, Hooman

    2016-01-01

    Introduction: Treatment of cleft lip and palate patients requires a multidisciplinary plan. These patients usually have a hypoplastic maxilla due to the prior surgical scars. Orthognathic surgery to advance the maxilla in these patients is not very efficient; therefore, orthopedic interventions during an appropriate age seems to be essential. Case Report: In this article, two cleft lip and palate patients have been treated with Class III elastics anchored to the maxillary posterior and mandibular anterior miniplates in order to induce maxillary advancement. Conclusion: Both cases showed a significant improvement in their profiles with minimal dentoalveolar compensations. A counterclockwise rotation of the mandible occurred. PMID:27602343

  8. Prosthodontic Rehabilitation Alternative of Patients with Cleft Lip and Palate (CLP: Two Cases Report

    Directory of Open Access Journals (Sweden)

    Emrah Ayna

    2009-01-01

    Full Text Available Although patients with cleft lip and palate (CLP are not seen regularly in general dental practice, this is a frequent congenital anomaly; approximately one in every 800 live births results in a CLP. The cause of CLP is unknown, but possible causes are malnutrition and irradiation during pregnancy, psychological stress, teratogenic agents, infectious agents (viruses, and inheritance. Most clefts are likely caused by multiple genetic and nongenetic factors. Prosthetic reconstruction of the anterior maxilla is important for these patients. This paper describes the prosthetic rehabilitation of two patients with CLP, 19-year-old and 21-year-old women, both with surgically treated CLP. In both, an examination revealed a residual palatal defect of 2×3 mm and missing maxillary lateral incisors. The 19-year-old was treated with a fiber-reinforced composite resin-bonded fixed partial denture. The 21-year-old was treated with a removable partial denture with an extracoronal attachment system. The prosthetic rehabilitation of the two patients with CLP was evaluated clinically. In both, well-planned prosthetic, periodontal, and surgical therapy resulted in satisfactory function and esthetics, alleviating their deformities. With education and appropriate recall, the patients should be able to maintain their oral health.

  9. Closure of huge palatal fistula in an adult patient with isolated cleft palate: a technical note.

    Science.gov (United States)

    Rahpeyma, Amin; Khajehahmadi, Saeedeh

    2015-02-01

    Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and the effectiveness of this method are presented. PMID:25750845

  10. Closure of Huge Palatal Fistula in an Adult Patient with Isolated Cleft Palate: A Technical Note

    Directory of Open Access Journals (Sweden)

    Amin Rahpeyma, DDS

    2015-02-01

    Full Text Available Summary: Closure of huge palatal fistula surrounded by fully erupted permanent dentition in the adult patients with cleft is a challenge. Posteriorly based buccinator myomucosal flap is a neurovascular pedicled flap, with inherent nature of thin thickness, saliva secretion, and axial pattern blood supply. Vicinity of donor site to the palate and low donor-site morbidity are the other advantages. It is an ideal choice in such situation. In this article, the details of surgical technique and the effectiveness of this method are presented.

  11. Case series of undetected intranasal impression material in patients with clefts.

    Science.gov (United States)

    Jones, Simon D; Drake, David J

    2013-04-01

    We report the cases of two female patients in their twenties who had had corrective surgery for bilateral cleft lip and palate as babies. They had both had residual palatal fistulas and had had further treatment that required repeated dental impressions. Several years later both had complained of persistent nasal discomfort and discharge, and routine clinical examination and investigations had failed to identify the cause. Full examination of the whole nasal cavity under general anaesthesia, in both cases, showed the presence of displaced dental impression material in the nasal floor. Removal resulted in complete resolution of symptoms.

  12. [Nasal breath recovery and rhinoplasty in cleft lip and palate patient with unilateral choanal atresia].

    Science.gov (United States)

    Chkadua, T Z; Ivanova, M D; Daminov, R O; Brusova, L A; Savvateeva, D M

    2016-01-01

    The paper presents the analysis of clinical case of endoscopic nasal breath restoration and elimination of the secondary cleft lip nasal deformity in 27 years old patient with unilateral choanal atresia and secondary nasal deformity after rhinocheiloplasty. Preoperative examination revealed the absence of nasal breathing on collateral side due to complete bone choanal atresia. Surgical treatment included endoscopic choanal repair, elimination of the secondary nasal deformity, septoplasty, conchotomy and lateroposition of the inferior conchae. The treatment resulted in nasal breath restoration and elimination of nasal deformity. Long-term follow-up at 1 and 12 months post-operatively proved stable positive aesthetic and functional results. PMID:26925564

  13. Secondary alveolar bone grafting in cleft of the lip and palate patients

    Directory of Open Access Journals (Sweden)

    Abhilashaa Walia

    2011-01-01

    Full Text Available Aim: The aim was to restore the function and form of both arches with a proper occlusal relationship and eruption of tooth in the cleft area. Materials and Methods: Eleven patients were selected irrespective of sex and socio-economic status and whose age was within the mixed dentition period. Iliac crest is grafted in cleft area and subsequently evaluated for graft success using study models, and periapical and occlusal radiographs. Results: At the time of evaluation teeth were erupted in the area and good alveolar bone levels were present. Premaxilla becomes immobile with a good arch form and arch continuity. There are no major complications in terms of pain, infection, paraesthesia, hematoma formation at donor site without difficulty in walking. There is no complication in terms of pain, infection, exposure of graft, rejection of graft, and wound dehiscence at the recipient site. Discussion: It is evident that secondary alveolar grafting during the mixed dentition period is more beneficial for patients at the donor site as well as the recipient site. Conclusion: Long-term follow-up is required to achieve maximum advantage of secondary alveolar grafting; the age of the patient should be within the mixed dentition period, irrespective of sex, socio-economic status. It may be unilateral or bilateral.

  14. A longitudinal three-center study of dental arch relationship in patients with bilateral cleft lip and palate.

    NARCIS (Netherlands)

    Bartzela, T.N.; Katsaros, C.; Shaw, W.C.; Ronning, E.; Rizell, S.; Bronkhorst, E.M.; Okada, T.O.; S L Pinheiro, F.H. de; Dominguez-Gonzalez, S.; Hagberg, C.; Semb, G.; Kuijpers-Jagtman, A.M.

    2010-01-01

    OBJECTIVE: To compare and evaluate longitudinally the dental arch relationships from 4.5 to 13.5 years of age with the Bauru-BCLP Yardstick in a large sample of patients with bilateral cleft lip and palate (BCLP). DESIGN: Retrospective longitudinal intercenter outcome study. PATIENTS: Dental casts o

  15. The Management of Iatrogenic Obstructive Sleep Apnoea Syndrome Following Bimaxillary Surgery in a Patient with Cleft Lip and Palate.

    Science.gov (United States)

    Gerbino, Giovanni; Gervasio, Fernando Carmine; Blythe, John; Bianchi, Francesca Antonella

    2016-07-01

    A 26-year-old man presented with a 6-year history of severe obstructive sleep apnoea syndrome followed a bimaxillary osteotomy procedure for a class III skeletal pattern. The patient was born with a unilateral cleft lip and palate and underwent primary lip and palate repair and later a pharyngeal flap for severe velopharyngeal insufficiency. Surgical management of obstructive sleep apnoea syndrome with conventional osteotomy, in cleft lip and palate patients, is a difficult problem. Distraction osteogenesis may provide a safer alternative. The authors describe and discuss the indications and the technical challenge of a multistage treatment protocol with distraction osteogenesis. PMID:27391499

  16. Diagnostic implications of associated defects in patients with typical orofacial clefts

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    Isabella L. Monlleó

    2015-10-01

    Full Text Available ABSTRACT OBJECTIVES: To describe prevalence of associated defects and clinical-genetic characteristics of patients with typical orofacial clefts seen at a reference genetic service. METHODS: Descriptive study conducted between September of 2009 and July of 2014. Two experienced dysmorphologists personally collected and coded clinical data using a validated, standard multicenter protocol. Syndromic cases were defined by the presence of four or more minor defects, one or more major defects, or recognition of a specific syndrome. Fisher's exact and Kruskal-Wallis tests were used for statistics. RESULTS: Among 141 subjects, associated defects were found in 133 (93%, and 84 (59.5% were assigned as syndromic. Cleft palate was statistically associated with a greater number of minor defects (p < 0.0012 and syndromic assignment (p < 0.001. Syndromic group was associated with low birth weight (p < 0.04 and less access to surgical treatment (p < 0.002. There was no statistical difference between syndromic and non-syndromic groups regarding gender (p < 0.55, maternal age of 35 years and above (p < 0.50, alcohol (p < 0.50 and tobacco consumption (p < 0.11, consanguinity (p < 0.59, recurrence (p < 0.08, average number of pregnancies (p < 0.32, and offspring (p < 0.35. CONCLUSIONS: There is a lack of information on syndromic clefts. The classification system for phenotype assignment adopted in this study has facilitated recognition of high prevalence of associated defects and syndromic cases. This system may be a useful strategy to gather homogeneous samples, to elect appropriate technologies for etiologic and genotype-phenotype approaches, and to assist with multiprofessional care and genetic counseling.

  17. Morphology of bone defects in patient with unilateral cleft lip and palate. Cone beam x-ray CT evaluation

    International Nuclear Information System (INIS)

    Orthodontic treatment planning of the cleft lip and palate vary according to the morphology of the alveolar bone and palatal bone. The purpose of this study is to evaluate the three-dimensional anatomy of the alveolar and palatal bone in children with complete unilateral cleft lip and palate. Thirty-three nonsyndromic consecutive patients with complete unilateral cleft lip and palate were treated by the cleft palate team at Showa University. Each patient had lip and palate surgeries at Showa University. Cone beam CT radiographs (CB MercuRay, Hitachi) were taken prior to secondary bone grafting, and were classified according to the method of Kita et al. 1997. Cone beam CT radiographs showed multiple types of alveolar and palatal bone morphology, and focused on special types described in the method of Kita et al. It was most frequently found that bone defects in the alveolar crest showed similar patterns in both buccal and palatal aspect, and the buccal bone defect in the nasal floor was larger than the palatal bone defect in the nasal floor. In 80% of the patients, the palatal bone defect showed similar patterns in both anterior and posterior aspects, and the anterior palatal bone defect was smaller than the posterior palatal bone defect. In addition, inadequate bone bridges were frequently found at the cleft site. It is suggested that patients with unilateral cleft lip and palate have various types of alveolar and palatal bone morphology, and are required to take three-dimensional radiographic X-rays prior to any orthodontic treatment. (author)

  18. Value of acoustic perceptual method for analysis of compensatory articulation errors in postoperative patients with cleft palate

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective To establish an acoustic perceptual method analyzing the compensatory articulation errors in children with operated cleft palate via the formants of Chinese pure vowels. Methods The first three formants which represent vocal transmission character in Chinese pure vowels of 84 subjects with operated cleft palate, were measured by Computerized Speech Signal Processing System (CSSPS). The Chinese vowel graph of postoperative patients with cleft palate was stated by the first formant frequencies (F1) and the second formant frequencies (F2) of the Chinese pure vowels between the two groups. Results Values of F1 and F2 of vowels except [a] in the poor articulation group (Group A) were significantly different from those in the good articulation group (Group B) (P<0.05 or P<0.01). As compared with that in Group B, the vowel graph demonstrated the decreased perceptual distances in Group A. These findings indicated that there might still be the backward movements of tongue, perverted mandibular movements and disharmonious lip movements in addition to the velopharyngeal insufficiency (VPI) in Group A. Conclusion The speech articulation of children with repaired cleft palate should be gained by correcting the aberrant compensatory articulation errors in the condition of velopharyngeal competence. Computerized Speech Signal Processing System (CSSPS), which is regarded as the content of objective quantitative measurement, is a precise, simple, reliable and atroumatic technique for children with cleft palate to analyze pathological compensatory articulation errors.

  19. The effect of Delaire cheilorhinoplasty on midfacial growth in patients with unilateral cleft lip and palate.

    Science.gov (United States)

    Rullo, Rosario; Laino, Gregorio; Cataneo, Marisa; Mazzarella, Nicoletta; Festa, Vincenzo Maria; Gombos, Fernando

    2009-02-01

    The aim of this research was to evaluate the effect of the Delaire surgical technique on the midfacial morphology in a group of subjects with a congenital unilateral cleft of lip and palate (UCLP), prior to orthodontic treatment. Thirty-five UCLP (15 left and 20 right) patients (16 males and 19 females, mean age 7.03+/-0.9 years; age range 8.7-5.0 years), treated for the correction of congenital malformation, were retrospectively selected. Analysis of midfacial growth was undertaken on lateral cephalograms, and the data were compared with reference values (Ricketts analysis). A Mann-Whitney ranked sum test was used to detect significant differences between the findings and reference values. P cleft lip might be responsible for excessive maxillary retropositioning with a downward rotation.

  20. Neonatal Mandibular Distraction Osteogenesis Reduces Cleft Palate Width and Lengthens Soft Palate, Influencing Palatoplasty in Patients With Pierre Robin Sequence.

    Science.gov (United States)

    Collares, Marcus V M; Duarte, Daniele W; Sobral, Davi S; Portinho, Ciro P; Faller, Gustavo J; Fraga, Mariana M

    2016-07-01

    The aim of this study was to evaluate the influence of neonatal mandibular distraction osteogenesis (MDO) on cleft dimensions and on early palatoplasty outcomes in patients with Pierre Robin Sequence (PRS). In a prospective cohort study that enrolled 24 nonsyndromic patients with PRS, 12 submitted to the MDO group and 12 patients not treated (non-MDO group), the authors compared patients for cleft palate dimensions through 7 morphometric measurements at the moment of palatoplasty and for early palatoplasty outcomes. At palatoplasty, the MDO group presented a significant shorter distance between the posterior nasal spines (PNS-PNS, P < 0.001) and between uvular bases (UB-UB, P < 0.001), representing a reduction in cleft palate width. They also had significant soft palate lengthening represented by a larger distance between UB and retromolar space (UB-RM, P < 0.001) and UB and PNS (UB-PNS, P = 0.014). Their UB moved away from the posterior wall of the nasopharynx (UB-NPH, P < 0.001). The MDO group had a length of operative time significantly shorter (P < 0.001) and no early palatoplasty complications compared with the non-MDO group. In conclusion, MDO acted as an orthopedic procedure that reduced cleft palate width and elongated the soft palate in patients with PRS. These modifications enabled a reduction of around 11% in the length of operative time of palatoplasty (P < 0.001).

  1. Cleft Lip and Cleft Palate

    Science.gov (United States)

    ... months of age. Your child may need more surgery for oral clefts as he grows. Your baby gets treated ... months of age. Your child may need more surgery for oral clefts as he grows. Your baby gets treated ...

  2. American College of Prosthodontists

    Science.gov (United States)

    ... Mouth Broken or Chipped Tooth C Cavities or Tooth Decay Cleft Lip/Palate Congenital Dental Defects Cracked Tooth ... in Mouth Broken or Chipped Tooth Cavities or Tooth Decay Cleft Lip/Palate Congenital Dental Defects Cracked Tooth ...

  3. Amelogenin gene influence on enamel defects of cleft lip and palate patients

    Directory of Open Access Journals (Sweden)

    Fernanda Veronese OLIVEIRA

    2014-08-01

    Full Text Available The aim of this study was to investigate the occurrence of mutations in the amelogenin gene (AMELX in patients with cleft lip and palate (CLP and enamel defects (ED. A total of 165 patients were divided into four groups: with CLP and ED (n=46, with CLP and without ED (n = 34, without CLP and with ED (n = 34, and without CLP or ED (n = 51. Genomic DNA was extracted from saliva followed by conducting a Polymerase Chain Reaction and direct DNA sequencing of exons 2 through 7 of AMELX. Mutations were found in 30% (n = 14, 35% (n = 12, 11% (n = 4 and 13% (n = 7 of the subjects from groups 1, 2, 3 and 4, respectively. Thirty seven mutations were detected and distributed throughout exons 2 (1 mutation – 2.7%, 6 (30 mutations – 81.08% and 7 (6 mutations – 16.22% of AMELX. No mutations were found in exons 3, 4 or 5. Of the 30 mutations found in exon 6, 43.34% (n = 13, 23.33% (n = 7, 13.33% (n = 4 and 20% (n = 6 were found in groups 1, 2, 3 and 4, respectively. c.261 C > T (rs2106416, a silent mutation, was detected in 26 subjects, and found more significantly (p = 0.003 in patients with CLP (groups 1 and 2 – 23.75%, compared with those without CLP (groups 3 and 4 – 8.23%. In the groups without ED, this silent mutation was also found more significantly (p = 0.032 among subjects with CLP (17.65% in group 2, compared with those without CLP (7.8% in group 4. In conclusion, this study suggested that AMELX may be a candidate gene for cleft lip and palate.

  4. Association of Single Nucleotide Polymorphisms in IRF6 and TGFA Genes With Nonsyndromic Cleft Lip With Or Without Cleft Palate in Chinese Patients

    Institute of Scientific and Technical Information of China (English)

    Ya Shen; Yugui Cu; Weidong Wan; Xiaoping Zhou; Lu Cheng; Zuhong Lu; Jiayin Liu

    2009-01-01

    Objective:Nonsyndromic cleft lip with or without cleft palate(NSCL/P) is a common birth defect with unclear etiology.Both genetic and environmental factors may contribute to NSCL/P.Many genes have been identified as candidate genes associated with this disease.Interferon regulatory factor6(IRF6) gene and transforming growth factor-a(TGFA) gene seem to be cmcial in the predisposition of NSCL/P.Here we evaluated some single nucleotide polymorphisms(SNPs) loci of TGFA and IRF6 genes in Chinese nuclear families consisting of fathers,mothers and affected offspring with NSCL/P.Methods:Fifty patients of NSCL/P were confirmed by the plastic surgeons.They and their parents were included in the study,all with the informed consents.SNPs loci of TGFA and IRF6 genes were analyzed by microarray technology.Some PCR products were randomly chosen and sequenced to check microarray results.The distribution of gene type and allele frequency between patient group and parents group were compared.Then a Haplotype Relative Risk(HRR) and Transmis-sion Disequilibrium Test(TDT) were performed.Results:The sequences of randomly selected PCR products were all consistent with the microarray results.All loci were in Hardy-Weinberg equilibrium.There were no significant differences in the distribution of genotypes and alleles between patients and their parents.Using HRR and TDT analyses the V2741 of IRF6 was associated with NSCL/P,while another SNP locus of IRF6 was not.Strong evidence of linkage disequilibrium was found between the2 SNP loci of TGFA and disease with the HRR analysis,but not with the TDT analysis.Conclusion:Our study confirms the contribution of IRF6 in the etiology of NSCL/P in populations of Asian ancestry.The association of TGFA with NSCL/P requires further research.

  5. A comprehensive review of the genetic basis of cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Sarvraj Singh Kohli

    2012-01-01

    Full Text Available Cleft lip and palate (CLP are birth defects that affect the upper lip and the roof of the mouth. CLP has a multifactorial etiology, comprising both genetic and environmental factors. In this review we discuss the recent data on the etiology of cleft lip and palate. We conducted a search of the MEDLINE database (Entrez PubMed from January 1986 to December 2010 using the key words: ′cleft lip,′ ′cleft palate,′ ′etiology,′ and ′genetics.′ The etiology of CLP seems complex, with genetics playing a major role. Several genes causing syndromic CLP have been discovered. Three of them-T-box transcription factor-22 (TBX22, poliovirus receptor-like-1 (PVRL1, and interferon regulatory factor-6 (IRF6-are responsible for causing X-linked cleft palate, cleft lip/palate-ectodermal dysplasia syndrome, and Van der Woude and popliteal pterygium syndromes, respectively; they are also implicated in nonsyndromic CLP. The nature and functions of these genes vary widely, illustrating the high vulnerability within the craniofacial developmental pathways. The etiological complexity of nonsyndromic cleft lip and palate is also exemplified by the large number of candidate genes and loci. To conclude, although the etiology of nonsyndromic CLP is still largely unknown, mutations in candidate genes have been identified in a small proportion of cases. Determining the relative risk of CLP on the basis of genetic background and environmental influence (including smoking, alcohol use, and dietary factors will be useful for genetic counseling and the development of future preventive measures.

  6. Parental cigarette smoking, transforming growth factor-alpha gene variant and the risk of orofacial cleft in Iranian infants

    Science.gov (United States)

    Ebadifar, Asghar; Hamedi, Roya; KhorramKhorshid, Hamid Reza; Kamali, Koorosh; Moghadam, Fatemeh Aghakhani

    2016-01-01

    Objective(s): We investigated the influence of genetic variation of the transforming growth-factor alpha (TGFA) locus on the relationship between smoking and oral clefts. Materials and Methods: In this study 105 Iranian infants with non-syndromic cleft lip/palate and 218 controls with non-cleft birth defects were examined to test for associations among maternal exposures, genetic markers, and oral clefts. Maternal and parental smoking histories during pregnancy were obtained through questionnaire. DNA was extracted from newborn screening blood samples, and genotyping of the BamHI polymorphism in the TGFA gene was performed by polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) methods. A number of factors including gender of the newborns, type of oral cleft, consanguinity of the parents, as well as the mother’s age and education were evaluated as potential confounders and effect modifiers. Results: Maternal smoking, in the absence of paternal smoking, was associated with an increased risk for CL/P (OR = 19.2, 95% CI = [(6.2-59.5)]) and cleft palate only (OR =48.7, 95% CI = [(8-29.3)]). If both parents smoked, risks were generally greater (OR = 55.6, 95% CI = [12-20.25]). Analyses for the risk of clefting from maternal smoking, stratified by the presence or absence of the TGFA/BamH1variant, revealed that the risk of clefting among the infants with the TGFA/BamH1 variant when their mothers smoked cigarettes was much greater than the infants who had non-smoker mothers (P=0.001, OR=10.4,95% CI=[3.2,33.6]). Conclusion: The results of this study indicate that first-trimester maternal smoking and infant TGFA locus mutations are both associated with nonsyndromic cleft lip and/or palate (CL/P). PMID:27279979

  7. Lip movement in patients with a history of unilateral cleft lip.

    NARCIS (Netherlands)

    Rutjens, C.A.W.; Spauwen, P.H.M.; Lieshout, P.H.H.M. van

    2001-01-01

    OBJECTIVE: The influence of a repaired cleft lip on the stability of coordination between upper and lower lip in nonspeech and speech tasks was investigated. DESIGN: First, we looked at the effects of a secondary cleft lip repair in three individuals. Second, we compared subjects with a history of r

  8. Presurgical orthodontic treatment of patients with complete bilateral cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Radojičić Julija

    2014-01-01

    Full Text Available Introduction. Cleft lips and palates are the most common congenital orofacial anomaly. This type of clefts is the most severe from the orthodontic-surgical therapy aspect. Case report. A female newborn with a complete cleft of the primary and the secondary palate was admitted to the clinic, where a multiple-role orthodontic device was specially designed and applied to primarily manage the closure of the existing cleft and help to improve the suckling ability of the baby. Besides the fact that it allows breastfeeding, it has a significant orthodontic effect, too. Conclusion. Specificity of this device is the lack of extraoral fixation. What can easily be observed is a progressive reduction of the cleft between the separated segments and the premaxilla retrusion. It, thus, allows the creation of much better conditions for further surgical management of the said defect.

  9. IRF6 polymorphisms in Mexican patients with non-syndromic cleft lip

    Science.gov (United States)

    Ibarra-Arce, Aurora; García-Álvarez, Martín; Cortés-González, Daniel; Ortiz de Zarate-Alarcón, Gabriela; Flores-Peña, Laura; Sánchez-Camacho, Sandra; Arenas-Díaz, Silvia; Romero-Valdovinos, Mirza; Olivo-Díaz, Angélica

    2015-01-01

    Cleft lip with or without cleft palate (CL/P) is one of the most common birth defects; it is a multifactorial disease affecting > 1/1,000 live births in Europe, and its etiology is largely unknown, although it is very likely genetic and environmental factors contribute to this malformation. Orofacial development is a complex process involving many genes and signaling pathways. Mutations in the gene for the interferon regulatory factor 6 (IRF6) cause a hereditary dominant malformation syndrome including CL/P, and polymorphisms are associated with non-syndromic CL/P (MIM 119530). Five SNPs at the locus with high heterozygosity in Caucasian populations were chosen for the present research due to their very strong association with CL/P. A case–parent trio study was performed using 292 samples from Mexico. Association with the rs1319435-C/C genotype (P = 0.02) was found in patients (73) as compared to pseudocontrols (219), while the genotype rs1319435-T/C was related with protection (P = 0.041) in the triad design. Significant over-transmission of the G allele for marker rs2235375 (P = 0.049) was found. Only the TACGT haplotype was diminished in the affected child, either in single (P = 0.0208) or double (P = 0.0208) dose. The pairwise analysis showed rs2235543 and rs2235371 were in strong linkage disequilibrium. These results point to a substantial contribution of IRF6 in the etiology of non-syndromic CL/P in a sample of the Mexican population. PMID:25853057

  10. Evaluation of alveolar bone grafting in unilateral cleft lip and palate patients using a computer-aided diagnosis system

    Energy Technology Data Exchange (ETDEWEB)

    Sutthiprapaporn, Pipop; Kongsomboon, Supaporn; Limmonthol, Saowaluck; Pisek, Poonsak; Keinprasit, Chutimaporn [Khon Kaen University, Khon Kaen (Thailand); Tanimoto, Keiji; Nakamoto, Takashi [Dept. of Oral and Maxillofacial Radiology, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima (Japan)

    2012-09-15

    This study aimed to evaluate the trabecular bone changes after alveolar bone grafting in unilateral cleft lip and palate (UCLP) patients using a computer-aided diagnosis (CAD) system. The occlusal radiographs taken from 50 UCLP patients were surveyed retrospectively. The images were categorized as: 50 images in group 0 (before bone grafting), 33 images in group 1 (one month after bone grafting), 24 images in group 2 (2-4 months after bone grafting), 15 images in group 3 (5-7 months after bone grafting), and 21 images in group 4 (8 or more months after bone grafting). Each image was grouped as either 'non-cleft side' or 'cleft side'. The CAD system was used five times for each side to calculate the pixel area based on the mathematical morphology. Significant differences were found using a Wilcoxon signed ranks test or paired samples t test. The pixel area showed a significant difference between the 'non-cleft side' and 'cleft side' in group 0 (404.27{+-}103.72/117.73{+-}92.25; p=0.00), group 1 (434.29{+-}86.70/388.31{+-}109.51; p=0.01), and group 4 (430.98{+-}98.11/366.71{+-}154.59; p=0.02). No significant differences were found in group 2 (423.57{+-}98.12/383.47{+-}135.88; p=0.06) or group 3 (433.02{+-}116.07/384.16{+-}146.55; p=0.19). Based on the design of this study, alveolar bone grafting was similar to normal bone within 2-7 months postoperatively.

  11. Selected aspects of the oral environment in cleft palate patients – a problem evidently beyond dentists’ scope

    Directory of Open Access Journals (Sweden)

    Joanna AntoszewskaABCD

    2010-12-01

    Full Text Available Introduction:The oral cavity is a specialized ecosystem composed of dentition and mucosa exposed to the effects of saliva and gingival liquid. Its structure provides advantageous conditions for various microorganisms, both aerobic streptococci and anaerobic bacilli. The dynamic balance of the oral cavity ecosystem can be threatened by various factors. Lip and palatal clefts are the most frequent disorders in embryonic facial development. The aim of the study was to evaluate whether Streptococcus mutans and Lactobacillus acidophilus and oral cavity hygiene in patients with cleft palate treated orthodontically are significantly different compared with patients without these congenital malformations.Material/Methods:The study group consisted of 200 patients aged 6–21 who were divided into two groups depending on the presence of cleft palate and treatment method. Group C (control consisted of 50 orthodontically untreated patients with occlusal dental defects. The performed studies consisted of 2 parts: the clinical-laboratory part and statistical analysis.Clinical-laboratory partResults:High bacteria levels of Streptococcus mutans and Lactobacillus acidophilus in saliva of patients were comparably frequent between groups, but there were statistically significant differences in inter-group comparisons.Conclusions:As the orthodontic treatment changes the oral environment, control of proper hygienic behavior is an essential element of health education. Introduction of preventive programs is also very important.

  12. Reconstruction of maxilla alveolus for application of dental implant in patients with cleft defect.

    Science.gov (United States)

    Dusková, M; Kot'ová, M; Urban, F; Sosna, B; Jirkalová, R; Strnadel, T; Kristen, M; Leamerová, E; Gojis, O

    2004-01-01

    The prospective study of dental implant application into the reconstructed maxilla alveolus of cleft patients was started in 2001. Its aims are to specify precisely the indications, conditions and techniques, as well as the factors justifying the assumption that the results will be successful. Indication was based on an examination by a multidisciplinary therapeutic team (plastic surgeon, dental surgeon, orthodontist, and prosthetist), with the proviso that the patient should have a positive approach. Successful orthodontic treatment of intergnathic relation and shape of the dental arches served as the basis. It was followed by a reconstruction of the defective alveolus using autologous cancellous bone grafts, harvested from an iliac crest. Dental implants were inserted 12-15 weeks after the transplantation and subsequently a prosthetic component was applied with a time delay of at least 2 months. After the orthodontic preparation, reconstruction of the alveolus has so far been performed in 38 patients. Their age was at least 14 years, i.e. the age when growth of the orofacial region was finalised or already complete. The crucial tasks at this stage are to form a stable, three-dimensional voluminous alveolar crest, and to cover the grafts with a sufficient amount of quality soft tissue. We needed an average amount of 3.7 ccm of cancellous bone graft for the reconstruction. This amount can be harvested only from an enchondral bone. For soft tissue shell the mucoperiosteal shift of flap from cleft segment was used. In more serious cases contralateral or even bilateral shift was performed. The relief of tissue tension was performed by double cut-back. Using the approach described we attained a 84.2% success rate. Our results and experience derived within this project show that the success rate of this procedure depends on the continuous flow of the alveolar arch with a good intergnathic relation, with a length of defect between crowns of border teeth of at least 8 mm

  13. Survey of the patients with cleft lip and palate in China who were funded for surgery by the Smile Train Program from 2000 to 2002

    Institute of Scientific and Technical Information of China (English)

    ZHOU Qiao-juan; SHI Bing; SHI Zong-dao; ZHENG Qian; WANG Yan

    2006-01-01

    Background Cleft lip (CL) and cleft palate (CP) are two of the most frequent congenital malformations.Many epidemiologic studies on this deformity have been conducted worldwide, often producing inconsistent results. This study assessed epidemiology and some genetic aspects of cleft lip and palate in a Chinese sample from the Smile Train Program and to compare with other methodologically sound surveys.Methods The general information, family history, classification of cleft and associated malformations of 8000CL and CP surgery patients were analyzed.Results Of the 8000 cases, 7812 had complete data. The distribution of cleft types is 17.04% with CP, 23.39%with CL and 59.58% with cleft lip and palate (CLP). Unilateral clefts were more common than bilateral, with involved 29 CP cases, 41 CL and 156 CLP. The frequency of associated malformations in CLP (3.35%) was higher than CL (2.24%) and CP (2.22%) (P<0.05). Patients with CP or CLP were born less often in the winter than in the summer (P<0.05). A history of family members having clefts occurred in 6.84% of patients. The proportion of CLP cases (7.56%) was significantly higher than that of CL cases (5.64%) (P<0.05).Conclusions The different types of clefts appeared in the highest proportion in CLP and lowest proportion in CP. Males are more common with CL and CLP and less common with CP. These characteristics are the same as those of other Chinese surveys but different from some European reports.

  14. Psychological issues in cleft lip and cleft palate

    Directory of Open Access Journals (Sweden)

    Sousa Avinash

    2009-01-01

    Full Text Available Vocational and social issues affect rehabilitation and development of patients with cleft lip and cleft palate. However, psychological problems like lowered self esteem and difficulties in social interaction have also been noted in them. Not many pediatric reconstructive surgery teams have a psychiatrist on their panel. It is likely that psychological problems are higher in incidence than literature actually suggests. Hence it is very essential that such cases are identified by the surgical team to maximize positive outcome of surgery and rehabilitation. This study discusses psychological issues revolving around cleft lip and cleft palate along with lacunae in many psychological research studies.

  15. Prevalence and management of natal/neonatal teeth in cleft lip and palate patients

    Science.gov (United States)

    Yilmaz, R. Burcu Nur; Cakan, Derya Germec; Mesgarzadeh, Nasim

    2016-01-01

    Objective: The aim of this study was to determine the prevalence and distribution of natal/neonatal teeth in infants with cleft lip and palate (CLP) according to gender, involving jaw and side and to show the management of some cases. Materials and Methods: A retrospective study was carried out on medical history and photographic records of 69 infants with CLP, who were treated at the CLP clinic of Yeditepe University between years 2014–2015. The presence of neonatal teeth was determined, and if present the gender, type of cleft, and position were recorded. Statistical analysis was performed. Results: Neonatal teeth were observed in 7% of the study group. No significant differences were found between cleft types and gender (P > 0.05). The prevalence of neonatal teeth in bilateral, unilateral and isolated cleft type was 16.5%, 6.5%, and none, respectively. All neonatal teeth were located in the maxilla and on the cleft-side (100%). Conclusion: The presence of natal/neonatal teeth in infants with CLP was not rare. In all of these cases the teeth were located adjacent to the cleft region. In isolated palatal cleft, where the alveolar region including the teeth buds are away from the cleft, no neonatal teeth were observed. It may be concluded that neonatal teeth in infants with CLP are frequently present and located inside the borders of the presurgical orthopedic treatment (POT) plate. Therefore, if possible, immediate extraction of the neonatal teeth is advised or if not possible because of systemic health reasons, modifications of the plate are required. PMID:27011740

  16. Impaired epithelial differentiation of induced pluripotent stem cells from ectodermal dysplasia-related patients is rescued by the small compound APR-246/PRIMA-1MET.

    Science.gov (United States)

    Shalom-Feuerstein, Ruby; Serror, Laura; Aberdam, Edith; Müller, Franz-Josef; van Bokhoven, Hans; Wiman, Klas G; Zhou, Huiqing; Aberdam, Daniel; Petit, Isabelle

    2013-02-01

    Ectodermal dysplasia is a group of congenital syndromes affecting a variety of ectodermal derivatives. Among them, ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC) syndrome is caused by single point mutations in the p63 gene, which controls epidermal development and homeostasis. Phenotypic defects of the EEC syndrome include skin defects and limbal stem-cell deficiency. In this study, we designed a unique cellular model that recapitulated major embryonic defects related to EEC. Fibroblasts from healthy donors and EEC patients carrying two different point mutations in the DNA binding domain of p63 were reprogrammed into induced pluripotent stem cell (iPSC) lines. EEC-iPSC from both patients showed early ectodermal commitment into K18(+) cells but failed to further differentiate into K14(+) cells (epidermis/limbus) or K3/K12(+) cells (corneal epithelium). APR-246 (PRIMA-1(MET)), a small compound that restores functionality of mutant p53 in human tumor cells, could revert corneal epithelial lineage commitment and reinstate a normal p63-related signaling pathway. This study illustrates the relevance of iPSC for p63 related disorders and paves the way for future therapy of EEC. PMID:23355677

  17. The clinical study of ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome%先天性缺指(趾)-外胚层发育不良-唇/腭裂综合征的临床研究

    Institute of Scientific and Technical Information of China (English)

    宋宇鹏; 杨庆华; 蒋海越; 庄洪兴

    2010-01-01

    目的 探讨先天缺指(趾)-外胚层发育不良-唇/腭裂综合征的临床表现,诊断标准,遗传学特点及治疗措施.方法 2008年3月至2009年9月收集先天缺指(趾)-外胚层发育不良-唇/腭裂综合征女性患者一例,针对上唇缺损行手术修补,对其进行家系问卷和DNA检查,观察患者的临床表型和发病特点,分析可能的遗传方式.结果 术后患者伤口愈合良好,无并发症发生,此例患者随访半年,术后明显改善了患者的外观,研究收集的这个典型病例未追溯到明显家族遗传史.结论 收集的一个患者属典型的散发病例.通过对该患者的诊疗,证明治疗应是多学科的,主要是针对外观进行整形外科手术,临床的早期检查和正确诊断对后期治疗具有重要意义.产前诊断意义尤为重大.

  18. 先天缺指(趾)-外胚叶发育不良-唇/腭裂综合征的病例研究%Cases Study of Chinese Kindreds with Ectrodactyly, Ectodermal Dysplasia and Cleft Lip/palate Syndrome

    Institute of Scientific and Technical Information of China (English)

    张景霞; 郝杰兵; 金辉喜; 边专; 叶晓茜

    2007-01-01

    目的:探讨先天缺指(趾)-外胚叶发育不良-唇/腭裂综合征(EEC)的临床表型和遗传学特点.方法:收集具有典型症状的EEC病例并进行家系问卷和口腔检查,观察各家系患者的临床表型和发病特点,分析可能的遗传方式,绘制系谱图.结果:研究收集的2个EEC病例均未追溯到明显家族遗传史,患者均表现出明显的双侧多个缺指(趾),并指,唇/腭裂,家系2患者有毛发稀疏,指甲无光泽等明显外胚层发育不良表型,符合典型的EEC综合征.2名不同患者的严重程度存在明显差异.结论:收集的2个患者均属典型的散发EEC病例,临床的早期检查和正确诊断对后期治疗具有重要意义.

  19. Diced Cartilage Graft for Revision Rhinoplasty in a 64-year-old Cleft Patient: A Case Report.

    Science.gov (United States)

    Lin, Susie; Hsiao, Yen-Chang; Chang, Chun-Shin; Chen, Philip Kuo-Ting; Chen, Jyh-Ping

    2016-07-01

    Pure diced cartilage graft has been the technique of choice for revision rhinoplasty in cleft patients since 2003 at our center. This technique has several advantages over the traditional en bloc cartilage onlay graft including minimal risk of warping, its technical simplicity, and the ability to adjust the shape of the graft with manual massage for up to 3 weeks postoperatively. Calcification of the costal cartilage, however, poses a real concern for surgeons. In this case report, we are presenting a 64-year-old woman with a right unilateral complete cleft lip and palate deformity who presented to our clinic for secondary revision. Central-pattern rib calcification was encountered during the operation. Outcomes, details of the operation, and potential limitations of this technique are discussed in this case report. PMID:27536492

  20. Oral health related quality of life in cleft lip and palate patients rehabilitated with conventional prostheses or dental implants

    OpenAIRE

    Papi, Piero; Giardino, Rita; Sassano, Pierpaolo; Amodeo, Giulia; Pompa, Giorgio; Cascone, Piero

    2015-01-01

    Objectives: Cleft lip and/or palate (CLP) is the most common congenital craniofacial abnormality, with a prevalence of 9.92 per 10,000 live births. In treating patients with CLP, oral rehabilitation is definitely a very important phase of the treatment in order to improve the patient's oral health related quality of life (OH-QoL). The aim of this retrospective study is to assess the OH-QoL in patients rehabilitated with different prosthetic options, thus comparing the conventional treatments,...

  1. Cleft Lip – A Comprehensive Review

    OpenAIRE

    Shkoukani, Mahdi A.; Chen, Michael; Vong, Angela

    2013-01-01

    Orofacial clefts comprise a range of congenital deformities and are the most common head and neck congenital malformation. Clefting has significant psychological and socio- economic effects on patient quality of life and require a multidisciplinary team approach for management. The complex interplay between genetic and environmental factors play a significant role in the incidence and cause of clefting. In this review, the embryology, classification, epidemiology, and etiology of cleft lip ar...

  2. Exposure of Cleft Lip and Palate Patients to Toxic Elements Released during Orthodontic Treatment in the Study of Non-Invasive Matrices

    Science.gov (United States)

    Mikulewicz, Marcin; Kachniarz, Krzysztof; Chojnacka, Katarzyna

    2015-01-01

    The Objective The aim of the study was evaluation of metal ions (nickel and chromium) released from orthodontic appliances in cleft lip and palate patients and the usefulness of non-invasive matrices (saliva and hair). Materials and Methods The material studied consisted of 100 individuals, including 59 females and 41 males of 5 to 16 years of age, which were divided into 3 groups: experimental–patients with cleft lip and palate (36 individuals, the average treatment time 5.74 years); control group–patients without cleft lip and palate, during orthodontic treatment (32 individuals, the average treatment time 1.78 years) and the control group patients without cleft lip and palate, without any orthodontic appliances (32 individuals). Samples (saliva, hair) were collected and subjects underwent a survey by questionnaire. Multi-elemental analyses of the composition of non-invasive matrices was conducted in an accredited laboratory by inductively coupled plasma spectrometry technique ICP-OES. The results were reported as mean contents of particular elements (Cd, Cr, Cu, Fe, Mn, Mo, Ni, Si) in hair and in saliva. Results The concentration of Cr, Ni, Fe and Cu ions in saliva of cleft lip and palate patients were several times higher as compared with not treated orthodontically control groups and higher than in the group with orthodontic appliances. Among the assessed matrices, hair of cleft lip and palate patients seem to be not a meaningful biomarker. Conclusion It was found that orthodontic appliances used in long-term treatment of cleft lip and palate patients do not release toxic levels of Cr and Ni ions. PMID:26544176

  3. Exposure of Cleft Lip and Palate Patients to Toxic Elements Released during Orthodontic Treatment in the Study of Non-Invasive Matrices.

    Directory of Open Access Journals (Sweden)

    Marcin Mikulewicz

    Full Text Available The aim of the study was evaluation of metal ions (nickel and chromium released from orthodontic appliances in cleft lip and palate patients and the usefulness of non-invasive matrices (saliva and hair.The material studied consisted of 100 individuals, including 59 females and 41 males of 5 to 16 years of age, which were divided into 3 groups: experimental-patients with cleft lip and palate (36 individuals, the average treatment time 5.74 years; control group-patients without cleft lip and palate, during orthodontic treatment (32 individuals, the average treatment time 1.78 years and the control group patients without cleft lip and palate, without any orthodontic appliances (32 individuals. Samples (saliva, hair were collected and subjects underwent a survey by questionnaire. Multi-elemental analyses of the composition of non-invasive matrices was conducted in an accredited laboratory by inductively coupled plasma spectrometry technique ICP-OES. The results were reported as mean contents of particular elements (Cd, Cr, Cu, Fe, Mn, Mo, Ni, Si in hair and in saliva.The concentration of Cr, Ni, Fe and Cu ions in saliva of cleft lip and palate patients were several times higher as compared with not treated orthodontically control groups and higher than in the group with orthodontic appliances. Among the assessed matrices, hair of cleft lip and palate patients seem to be not a meaningful biomarker.It was found that orthodontic appliances used in long-term treatment of cleft lip and palate patients do not release toxic levels of Cr and Ni ions.

  4. Diagnosis and fine localization of deletion region in Wolf Hirschhorn syndrome patients

    Institute of Scientific and Technical Information of China (English)

    JI Tao-yun; David CHIA; WANG Jing-min; WU Ye; LI Jie; XIAO Jing; JIANG Yu-wu

    2010-01-01

    Background Wolf-Hirschhorn syndrome (WHS) results from the partial deletion of 4p. This study aimed to identify and fine map the chromosome deletion regions of Chinese children with Wolf-Hirschhorn syndrome among the developmental delay/mental retardation (DD/MR) patients.Methods We analyzed the relationship of phenotype and genotype. Inclusion criteria were: moderate to severe DD/MR, no definite perinatal brain injury, and no trauma, toxication, hypoxia, infection of central nervous system; routine karyotyping was normal, no evidence of typical inherited metabolic disorder or specific neurodegenerative disorders from cranial neuro-imaging and blood/urinary metabolic diseases screening; no mutation of FMR1 in male patients, no typical clinical manifestation of Rett syndrome in female patients. Multiplex ligation-dependent probe amplification (MLPA) and Affymetrix genome-wide human SNP array 6.0 assays were applied to accurately define the exact size of subtelomeric aberration region of four WHS patients.Results All four WHS patients presented with severe DD, hypotonia and microcephaly, failure to thrive, 3/4 patients with typical facial features and seizures, 2/4 patients with congenital heart defects and cleft lip/palate, 1/4 patients with other malformations. The length of the deletions ranged from 3.3 Mb to 9.8 Mb. Two of four patients had "classic" WHS, 1/4 patients had "mild"-to-"classic" WHS, and 1/4 patients had "mild" WHS.Conclusions WHS patients in China appear to be consistent with those previously reported. The prevalence of signs and symptoms, distribution of cases between "mild" and "classic" WHS, and the correlation between length of deletion and severity of disease of these patients were all similar to those of the patients from other populations.

  5. Separation anxiety in children ages 4 through 9 with oral clefts.

    Science.gov (United States)

    Tyler, Margaret C; Wehby, George L; Robbins, James M; Damiano, Peter C

    2013-09-01

    Background : Psychosocial research on children with oral clefts (OCs), i.e., clefts of the lip, palate, or lip and palate, has suggested that these children may have higher rates of anxiety in general, but overall results have been equivocal. In this study we estimated the prevalence of separation anxiety disorder (SAD) in a population-based sample of children with OCs and identified associated risk factors. Methods : Parents of 279 children with OCs, identified through three state birth defect registries, responded to a postal survey that included the Separation Anxiety subscale of the Screen for Child Anxiety-Related Emotional Disorders (SCARED) and items regarding the child's OC and its sequelae, parent and child health, parent mental health, family structure, and socioeconomic status indicators. Associations with SA were evaluated using bivariate and multivariate statistics. Results : One quarter (24%) of the children screened positive for SAD, which was substantially higher than the U.S. child population estimates of 3% to 5%. OC-related impairments in speaking and eating more than doubled the risk of SAD, as did lower socioeconomic status and lower maternal health rating. Maternal mental health and marital status remained in the logistic model but were not significant. Conclusions : Children with OCs appear to be at increased risk for SAD as measured by the SCARED instrument. OC-related problems with speech and eating more than doubled the risk of SAD. The use of targeted screening tools may help refine our understanding of psychosocial adjustment in children with OCs.

  6. Identification of germline mutations in the cancer predisposing gene CDH1 in patients with orofacial clefts

    NARCIS (Netherlands)

    Vogelaar, I.P.; Figueiredo, J.; Rooij, I.A. van; Simoes-Correia, J.; Post, R.S. van der; Melo, S.; Seruca, R.; Carels, C.E.L.; Ligtenberg, M.J.L.; Hoogerbrugge-van der Linden, N.

    2013-01-01

    Orofacial clefts (OFC) are among the most common birth defects worldwide. The etiology of non-syndromic OFC is still largely unknown. During embryonic development, the cell adhesion molecule E-cadherin, encoded by CDH1, is highly expressed in the median edge epithelium of the palate. Furthermore, in

  7. Craniofacial, craniocervical, and pharyngeal morphology in bilateral cleft lip and palate and obstructive sleep apnea patients

    NARCIS (Netherlands)

    Oosterkamp, B.C.M.; Remmelink, H.J.; Pruim, G.J.; Hoekema, A.; Dijkstra, P.U.

    2007-01-01

    Objective: The aim of this study was to analyze craniofacial, craniocervical, and pharyngeal morphology in surgically treated bilateral cleft lip and palate (BCLP) men, untreated men with obstructive sleep apnea (OSA), and a reference group of men. Subjects and methods: Lateral cephalograms were obt

  8. An assessment of orofacial clefts in Tanzania

    Directory of Open Access Journals (Sweden)

    Mazyala Erick

    2011-02-01

    Full Text Available Abstract Background Clefts of the lip (CL, the palate (CP, or both (CLP are the most common orofacial congenital malformations found among live births, accounting for 65% of all head and neck anomalies. The frequency and pattern of orofacial clefts in different parts of the world and among different human groups varies widely. Generally, populations of Asian or Native American origin have the highest prevalence, while Caucasian populations show intermediate prevalence and African populations the lowest. To date, little is known regarding the epidemiology and pattern of orofacial clefts in Tanzania. Methods A retrospective descriptive study was conducted at Bugando Medical Centre to identify all children with orofacial clefts that attended or were treated during a period of five years. Cleft lip and/or palate records were obtained from patient files in the Hospital's Departments of Surgery, Paediatrics and medical records. Age at presentation, sex, region of origin, type and laterality of the cleft were recorded. In addition, presence of associated congenital anomalies or syndromes was recorded. Results A total of 240 orofacial cleft cases were seen during this period. Isolated cleft lip was the most common cleft type followed closely by cleft lip and palate (CLP. This is a departure from the pattern of clefting reported for Caucasian and Asian populations, where CLP or isolated cleft palate is the most common type. The distribution of clefts by side showed a statistically significant preponderance of the left side (43.7% (χ2 = 92.4, p Conclusions Unilateral orofacial clefts were significantly more common than bilateral clefts; with the left side being the most common affected side. Most of the other findings did not show marked differences with orofacial cleft distributions in other African populations.

  9. 唇腭裂患者口腔健康状况的研究进展%Research progress on oral health status of patient with oral clefts

    Institute of Scientific and Technical Information of China (English)

    柴丛娜

    2011-01-01

    唇腭裂患者的口腔健康状况为近年来的研究热点之一.大量调查表明,唇腭裂患者是龋病和牙周组织疾病的易患人群.其龋病和牙周组织疾病的发生与外科手术、解剖因素、唇腭裂类型因素等密切相关.不同唇腭裂类型的患者口腔卫生状况不一致,不同国家地区的唇腭裂患者的口腔卫生状况也不尽相同.本文就唇腭裂患者的龋病流行特征、牙周疾病特征和口腔卫生状况等研究进展作一综述.%The oral health status of oral clefts has become a hot research spot in recent years. The survey shows that patients with oral clefts remain in the high-risk group for dental caries and periodontal disease. Surgical interventions combined anatomical defects, and cleft status was major factors for dental caries and periodontal disease in patients with oral clefts. For the past few years, significant difference was found in oral hygiene among different cleft types and in different countries of the cleft subjects. The studies of the caries prevalence, periodontal disease, influencing factors and oral hygiene of patients with oral clefts were summarized.

  10. Contribution to facial morphology study of the unilateral cleft lip and palate patients, by cephalometric radiographs, in frontal norm

    International Nuclear Information System (INIS)

    A study by cephalometric radiography, in frontal norm, is used to measure the magnitude of some linear dimensions of the face, in a sample of a 100 Brazilian, caucasian people. They were from both sexes, with average age of 20 years and were distributed into two groups: control group and unilateral cleft lip and palate group. Cephalometric radiography in frontal norm is obtained of each patient. In each teleradiography some parameters were measured by using a computer. An statistic analysis of the values obtained is presented. (M.A.C.)

  11. Micro-structured Beta-Tricalcium Phosphate for Repair of the Alveolar Cleft in Cleft Lip and Palate Patients : A Pilot Study

    NARCIS (Netherlands)

    de Ruiter, AP; Janssen, Nard; van Es, Robert; Frank, Michael; Meijer, Gert; Koole, Ron; Rosenberg, Toine

    2015-01-01

    OBJECTIVES: Can a synthetic bone substitute be used to repair the alveolar cleft to bypass donor site morbidity as well as to shorten the operating time? In earlier experimental studies, micro-structured beta-tricalcium phosphate (β-TCP) provided similar bone healing when compared with grafting with

  12. Inferior Turbinate Flap for Nasal-side Closure of Palatal Fistula in Cleft Patients: Technical Note

    OpenAIRE

    Amin Rahpeyma; Saeedeh Khajehahmadi

    2015-01-01

    Summary: Residual palatal fistula after repair of palatal cleft is common. Repair of residual oronasal fistula is not always successful. Two-layer closure techniques that close these fistulas with soft tissue are a common practice. Turnover flaps are the most used flaps and often the sole method for nasal-side closure of fistula. Anteriorly based inferior turbinate flap can be used to provide soft tissue for nasal-side closure when turnover flaps will not provide sufficient tissue for this pu...

  13. Three-dimensional imaging methods for quantitative analysis of facial soft tissues and skeletal morphology in patients with orofacial clefts: a systematic review.

    Directory of Open Access Journals (Sweden)

    Mette A R Kuijpers

    Full Text Available BACKGROUND: Current guidelines for evaluating cleft palate treatments are mostly based on two-dimensional (2D evaluation, but three-dimensional (3D imaging methods to assess treatment outcome are steadily rising. OBJECTIVE: To identify 3D imaging methods for quantitative assessment of soft tissue and skeletal morphology in patients with cleft lip and palate. DATA SOURCES: Literature was searched using PubMed (1948-2012, EMBASE (1980-2012, Scopus (2004-2012, Web of Science (1945-2012, and the Cochrane Library. The last search was performed September 30, 2012. Reference lists were hand searched for potentially eligible studies. There was no language restriction. STUDY SELECTION: We included publications using 3D imaging techniques to assess facial soft tissue or skeletal morphology in patients older than 5 years with a cleft lip with/or without cleft palate. We reviewed studies involving the facial region when at least 10 subjects in the sample size had at least one cleft type. Only primary publications were included. DATA EXTRACTION: Independent extraction of data and quality assessments were performed by two observers. RESULTS: Five hundred full text publications were retrieved, 144 met the inclusion criteria, with 63 high quality studies. There were differences in study designs, topics studied, patient characteristics, and success measurements; therefore, only a systematic review could be conducted. Main 3D-techniques that are used in cleft lip and palate patients are CT, CBCT, MRI, stereophotogrammetry, and laser surface scanning. These techniques are mainly used for soft tissue analysis, evaluation of bone grafting, and changes in the craniofacial skeleton. Digital dental casts are used to evaluate treatment and changes over time. CONCLUSION: Available evidence implies that 3D imaging methods can be used for documentation of CLP patients. No data are available yet showing that 3D methods are more informative than conventional 2D methods

  14. A new primary cleft lip repair technique tailored for Asian patients that combines three surgical concepts: Comparison with rotation--advancement and straight-line methods.

    Science.gov (United States)

    Funayama, Emi; Yamamoto, Yuhei; Furukawa, Hiroshi; Murao, Naoki; Shichinohe, Ryuji; Hayashi, Toshihiko; Oyama, Akihiko

    2016-01-01

    Various techniques have been described for unilateral cleft lip repair. These may be broadly classified into three types of procedure/concept: the straight-line method (SL; Rose-Thompson effect); rotation-advancement (RA; upper-lip Z-plasty); and the triangular flap method (TA; lower-lip Z-plasty). Based on these procedures, cleft lip repair has evolved in recent decades. The cleft lip repair method in our institution has also undergone several changes. However, we have found that further modifications are needed for Asian patients who have wider philtral dimples and columns than Caucasians, while following the principles of the original techniques mentioned above. Here, we have incorporated the advantages of each procedure and propose a refined hybrid operating technique, seeking a more appropriate procedure for Asian patients. To evaluate our new technique, a comparison study was performed to evaluate RA, SL, and our technique. We have used our new technique to treat 137 consecutive cleft lip cases of all types and degrees of severity, with or without a cleft palate, since 2009. In the time since we adopted the hybrid technique, we have observed improved esthetics of the repaired lip. Our technique demonstrated higher glance impression average scores than RA/SL.

  15. A new primary cleft lip repair technique tailored for Asian patients that combines three surgical concepts: Comparison with rotation--advancement and straight-line methods.

    Science.gov (United States)

    Funayama, Emi; Yamamoto, Yuhei; Furukawa, Hiroshi; Murao, Naoki; Shichinohe, Ryuji; Hayashi, Toshihiko; Oyama, Akihiko

    2016-01-01

    Various techniques have been described for unilateral cleft lip repair. These may be broadly classified into three types of procedure/concept: the straight-line method (SL; Rose-Thompson effect); rotation-advancement (RA; upper-lip Z-plasty); and the triangular flap method (TA; lower-lip Z-plasty). Based on these procedures, cleft lip repair has evolved in recent decades. The cleft lip repair method in our institution has also undergone several changes. However, we have found that further modifications are needed for Asian patients who have wider philtral dimples and columns than Caucasians, while following the principles of the original techniques mentioned above. Here, we have incorporated the advantages of each procedure and propose a refined hybrid operating technique, seeking a more appropriate procedure for Asian patients. To evaluate our new technique, a comparison study was performed to evaluate RA, SL, and our technique. We have used our new technique to treat 137 consecutive cleft lip cases of all types and degrees of severity, with or without a cleft palate, since 2009. In the time since we adopted the hybrid technique, we have observed improved esthetics of the repaired lip. Our technique demonstrated higher glance impression average scores than RA/SL. PMID:26653337

  16. Cleft Lip and Palate

    Science.gov (United States)

    Cleft lip and cleft palate are birth defects that occur when a baby's lip or mouth do not form properly. They happen early during pregnancy. ... baby can have a cleft lip, a cleft palate, or both. A cleft lip happens if the ...

  17. Oral health related quality of life in cleft lip and palate patients rehabilitated with conventional prostheses or dental implants

    Science.gov (United States)

    Papi, Piero; Giardino, Rita; Sassano, Pierpaolo; Amodeo, Giulia; Pompa, Giorgio; Cascone, Piero

    2015-01-01

    Objectives: Cleft lip and/or palate (CLP) is the most common congenital craniofacial abnormality, with a prevalence of 9.92 per 10,000 live births. In treating patients with CLP, oral rehabilitation is definitely a very important phase of the treatment in order to improve the patient's oral health related quality of life (OH-QoL). The aim of this retrospective study is to assess the OH-QoL in patients rehabilitated with different prosthetic options, thus comparing the conventional treatments, which include removable partial dentures and fixed partial dentures, with the implant-supported prostheses. Materials and Methods: Sixty-three patients were enrolled in this retrospective survey [44 females (69.84%) and 19 males (30.16%)] with a mean age of 34.93 ± 7.04 years (age range 21–53 years). They were all treated for CLP and rehabilitated with a conventional prosthesis or an implant-supported denture. Two different questionnaires were used in the present study to evaluate patients’ OH-QoL: The Italian version of the 49-item Oral Health Impact Profile (OHIP-49) and the Italian version of the Cleft Evaluation Profile (CEP). Statistical analysis was performed using analysis of variance (ANOVA) test, with a significant P < 0,05. Results: Data analysis revealed that patients rehabilitated with implant-supported dentures and fixed partial dentures showed a good level of satisfaction with their prostheses, scoring low values in the OHIP-49 and high values in the CEP, while subjects with removable partial dentures scored the highest values in the OHIP-49 and the lowest values in the CEP, which means an unsatisfactory feeling (P < 0.05). Conclusions: OH-QoL is a challenging demand for all prosthodontists. Our results show, clearly, that patients rehabilitated with implant-supported dentures are more satisfied compared to subjects with fixed partial dentures and removable partial dentures. PMID:26759802

  18. Controversies in the Management of Patients with Cleft Lip and Palate.

    Science.gov (United States)

    Rodman, Regina E; Tatum, Sherard

    2016-08-01

    Cleft lip and palate is one of the most common congenital anomalies. For many years, surgeons have been attempting to reduce the severity of the deformity before the surgical repair to achieve a better outcome. The nasoalveolar molding technique uses acrylic nasal stents attached to the vestibular shield of an oral molding plate to mold the nasal alar cartilages into a more normal form and position during the presurgical period. Proponents of nasoalveolar molding claim several benefits, including improved aesthetic outcome, reduced overall costs, and a psychosocial benefit to the family. Research on these outcomes is not conclusive. PMID:27400840

  19. Cleft palate - resources

    Science.gov (United States)

    Resources - cleft palate ... The following organizations are good resources for information on cleft palate : Cleft Palate Foundation -- www.cleftline.org March of Dimes -- www.marchofdimes.com/professionals/14332_1210.asp ...

  20. EEC syndrome without ectrodactyly: report of two new families.

    OpenAIRE

    Fryns, J P; Legius, E.; Dereymaeker, A M; van den Berghe, H

    1990-01-01

    In this report we describe two families with variable manifestations of the EEC syndrome. The findings in these families confirm that no symptom is obligatory for the diagnosis of EEC syndrome. In the absence of cleft lip/palate, EEC patients have a characteristic facial morphology with maxillary hypoplasia, short philtrum, and broad nasal tip.

  1. Susceptibility to DNA damage as a molecular mechanism for non-syndromic cleft lip and palate.

    Directory of Open Access Journals (Sweden)

    Gerson Shigeru Kobayashi

    Full Text Available Non-syndromic cleft lip/palate (NSCL/P is a complex, frequent congenital malformation, determined by the interplay between genetic and environmental factors during embryonic development. Previous findings have appointed an aetiological overlap between NSCL/P and cancer, and alterations in similar biological pathways may underpin both conditions. Here, using a combination of transcriptomic profiling and functional approaches, we report that NSCL/P dental pulp stem cells exhibit dysregulation of a co-expressed gene network mainly associated with DNA double-strand break repair and cell cycle control (p = 2.88×10(-2-5.02×10(-9. This network included important genes for these cellular processes, such as BRCA1, RAD51, and MSH2, which are predicted to be regulated by transcription factor E2F1. Functional assays support these findings, revealing that NSCL/P cells accumulate DNA double-strand breaks upon exposure to H2O2. Furthermore, we show that E2f1, Brca1 and Rad51 are co-expressed in the developing embryonic orofacial primordia, and may act as a molecular hub playing a role in lip and palate morphogenesis. In conclusion, we show for the first time that cellular defences against DNA damage may take part in determining the susceptibility to NSCL/P. These results are in accordance with the hypothesis of aetiological overlap between this malformation and cancer, and suggest a new pathogenic mechanism for the disease.

  2. Dental anomalies in different cleft groups related to neural crest developmental fields contributes to the understanding of cleft aetiology

    DEFF Research Database (Denmark)

    Riis, Louise Claudius; Kjær, Inger; Mølsted, Kirsten

    2014-01-01

    OBJECTIVE: To analyze dental deviations in three cleft groups and relate findings to embryological neural crest fields (frontonasal, maxillary, and palatal). The overall purpose was to evaluate how fields are involved in different cleft types. DESIGN: Retrospective audit of clinical photographs......, radiographs, dental casts, and medical records. PATIENTS: Ninety individuals (30 cleft lip, 30 cleft palate, and 30 combined cleft lip and palate), aged 5-27 years. MAIN OUTCOME MEASURES: Visual evaluation of tooth number and tooth morphology. RESULTS: Cleft lip: Dental deviations were predominantly observed...

  3. Secondary Alveolar Bone Grafting and Iliac Cancellous Bone Harvesting for Patients With Alveolar Cleft.

    Science.gov (United States)

    Pan, Weiyi; Wu, Chenzhou; Yang, Zheng; Duan, Zexi; Su, Zhifei; Wang, Peiqi; Zheng, Qian; Li, Chunjie

    2016-06-01

    To assess the efficacy of present interventions optimizing the result of secondary alveolar bone grafting (SABG) and the interventions alleviating the donor site morbidity after iliac cancellous bone harvesting. Researches were identified by searching the electronic database of MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Chinese BioMedical Literature Database, and the China National Knowledge Infrastructure. In addition, relevant journals and references of the included studies were searched manually. The Oxford 2011 Levels of Evidence were applied to assess the methodological quality of selected studies, and the best evidence synthesis system was applied afterward to measure the strength of evidence. As a result, 42 studies were considered eligible and included, among which 4 were of high quality while 38 were of low quality. Thirty lines of evidences were acquired after the synthesis, among which 13 were rated as moderate while 17 were rated as insufficient. As for the interventions optimizing the result of SABG, moderate evidence confirmed the efficacy of preoperative orthodontic treatment, the superiority of performing SABG before the eruption of canine, and the accuracy of cone beam computed tomography in preoperative estimation of the cleft volume. As for the interventions alleviating the morbidity of iliac cancellous bone harvesting, moderate evidence confirmed the treatment benefit of the interventions below: minimally invasive technique, including trephine and Shepard osteotomy; preemptive analgesia, including continuous bupivacaine infusion or transversus abdominis plane block. As for the rest interventions, only insufficient evidence was found. PMID:27244214

  4. Cleft Palate Foundation

    Science.gov (United States)

    ... Spanish , and Mandarin ! Information on Cleft Lip and Palate Our booklets and factsheets address a variety of ... Bear. –Paige with her Cleftline™ teddy bear– Cleft Palate Foundation 1504 East Franklin Street, Suite 102 Chapel ...

  5. Alveolar bone grafting in association with polyostotic fibrous dysplasia and bisphosphonate-induced abnormal bone turnover in a bilateral cleft lip and palate patient: a case report.

    Science.gov (United States)

    Kodama, Yasumitsu; Ogose, Akira; Oguri, Yoshimitsu; Ubaidus, Sobhan; Iizuka, Tateyuki; Takagi, Ritsuo

    2012-09-01

    A case is presented of extensive alveolar bone grafting in a patient with bilateral cleft lip and palate and polyostotic fibrous dysplasia. The patient previously underwent bisphosphonate therapy. Because of an abnormal and often decreased bone turnover caused by the fibrous dysplasia and the bisphosphonate therapy, bone grafting in such a patient poses several potential difficulties. In addition, the histomorphometric analysis of the bone grafts showed markedly decreased bone turnover. However, alveolar bone grafting using the iliac crest was performed successfully. Sufficient occlusion was achieved by postoperative low-loading orthodontic treatment.

  6. [Team management of orofacial clefts].

    Science.gov (United States)

    Kuijpers-Jagtman, A M; Borstlap-Engels, V M; Spauwen, P H; Borstlap, W A

    2000-11-01

    In the Netherlands 15 centres provide multidisciplinary care for cleft lip and palate patients. Usually the following disciplines participate in such teams: paediatrics, plastic and reconstructive surgery, orthodontics, genetics, social work or nursing, ENT, speech therapy, maxillofacial surgery, prosthetic dentistry, psychology and oral hygiene. An overview is given of the treatment protocol from birth until 20 years of age for a child with a complete UCLP or BCLP. It is concluded that properly designed prospective clinical trials are rare, resulting in a lack of evidence based care in the field of cleft lip and palate. Furthermore it should be investigated whether it is preferable to centralise the cleft care in less centres than the present 15 ones.

  7. Possible sex-discriminant variables in craniofacial growth in clefting.

    Science.gov (United States)

    Long, R E; Jain, R B; Krogman, W M

    1982-11-01

    In this investigation, 174 patients with orofacial clefts were examined for identification of possible sex differences in craniodentofacial measurements. The patients were selected from the longitudinal growth files of the H. K. Cooper Clinic. Records available for analysis were serial lateral cephalometric radiographs from the age of 1 month to 10 years. Patients were grouped by cleft type and sex within each cleft group (78 cleft palate only, 64 unilateral cleft of lip and palate, 32 bilateral cleft of lip and palate). Stepwise discriminant analysis of fourteen linear and angular craniofacial dimensions was used to identify those variables which contributed to sex differences within each cleft group over the growth/time intervals examined. Results suggested the possibility of sex-related differences in growth timing, that is, earlier maturation and growth in females in several craniofacial areas which did not appear to be related to the presence, absence, or type of cleft but which could possibly modify cleft-specific responses to treatment (cranial base dimensions, face heights). Other sex-related differences appeared to be more specifically related to known sex differences in original cleft type and severity (mandibular size and position, midfacial dimensions). The manner in which these various sex factors interface with environmental and therapeutic influences in producing the ultimate craniodentofacial morphology in a given sex and cleft type is discussed.

  8. International Task Force on Volunteer Cleft Missions.

    Science.gov (United States)

    Yeow, Vincent K L; Lee, Seng-Teik T; Lambrecht, Thomas J; Barnett, John; Gorney, Mark; Hardjowasito, Widanto; Lemperle, Gottfried; McComb, Harold; Natsume, Nagato; Stranc, Mirek; Wilson, Libby

    2002-01-01

    The International Task Force on Volunteer Cleft Missions was set up to provide a report to be presented at the Eighth International Congress of Cleft Palate and Associated Craniofacial Anomalies on September 12, 1997, in Singapore. The aim of the report was to provide data from a wide range of different international teams performing volunteer cleft missions and, thereafter, based on the collected data, to identify common goals and aims of such missions. Thirteen different groups actively participating in volunteer cleft missions worldwide were selected from the International Confederation of Plastic and Reconstructive Surgery's list of teams actively participating in volunteer cleft missions. Because of the time frame within which the committee had to work, three groups that did not respond by the stipulated deadline were omitted from the committee. The represented members and their respective institutions have undertaken more than 50 volunteer cleft missions to underdeveloped nations worldwide within the last 3 years. They have visited over 20 different countries, treating more than 3,500 patients worldwide. Based on the data collected and by consensus, the committee outlined recommendations for future volunteer cleft missions based on 1) mission objectives, 2) organization, 3) personal health and liability, 4) funding, 5) trainees in volunteer cleft missions, and 6) public relations. The task force believed that all volunteer cleft missions should have well-defined objectives, preferably with long-term plans. The task force also decided that it was impossible to achieve a successful mission without good organization and close coordination. All efforts should be made, and care taken, to ensure that there is minimal morbidity and no mortality. Finally, as ambassadors of goodwill and humanitarian aid, the participants must make every effort to understand and respect local customs and protocol. The main aims are to provide top-quality surgical service, train local

  9. A Retrospective Study of Cleft lip and palate Patients´ Satisfaction after Maxillary Distraction or Traditional Advancement of the Maxilla

    Directory of Open Access Journals (Sweden)

    Kristian Andersen

    2012-06-01

    Full Text Available Objectives: To compare cleft lip and palate patients’ satisfaction with aesthetics and functional parameters after conventional advancement of the maxilla or by the use of distraction osteogenesis.Material and methods: Case series observational study. Group of distraction osteogenesis (DO consisted of 15 patients treated with distraction osteogenesis while group conventional (CONV included 10 patients treated with traditional advancement of the maxilla. Patients were asked to fill out a questionnaire about their subjective evaluation of satisfaction with facial aesthetics and functional parameters on a continuous visual analog-scale (VAS when the treatment was finished.Results: The total response rate was 76%. Preoperatively the two groups did not differ significantly according to group characteristics. At follow-up both groups were satisfied with aesthetics and functional parameters. The DO group was less satisfied with the duration of the treatment than the CONV group. There were no statistically significant differences among the groups regarding functional parameters or facial aesthetics.Conclusions: Cleft lip and palate patients experienced a high level of satisfaction with functional parameters and aesthetics as a result of surgical maxillary advancement. The patients treated with distraction osteogenesis were less satisfied with the duration of the treatment. Further studies are needed.

  10. Cleft Palate; A Multidiscipline Approach.

    Science.gov (United States)

    Stark, Richard B., Ed.

    Nineteen articles present a multidisciplinary approach to the management of facial clefts. The following subjects are discussed: the history of cleft lip and cleft palate surgery; cogenital defects; classification; the operation of a cleft palate clinic; physical examination of newborns with cleft lip and/or palate; nursing care; anesthesia;…

  11. Protocols for Late Maxillary Protraction in Cleft Lip and Palate Patients at Childrens Hospital Los Angeles

    Science.gov (United States)

    Yen, Stephen L-K

    2011-01-01

    This paper describes the protocols used at Childrens Hospital Los Angeles (CHLA) to protract the maxilla during early adolescence. It is a modification of techniques introduced by Eric Liou with his Alternate Rapid Maxillary Expansion and Constriction (ALT-RAMEC) technique. The main differences between the CHLA protocol and previous maxillary protraction protocols are the age the protraction is attempted, the sutural loosening by alternating weekly expansion with constriction and the use of Class III elastics to support and redirect the protraction by nightly facemask wear. The CHLA protocol entirely depends on patient compliance and must be carefully taught and monitored. In a cooperative patient, the technique can correct a Class III malocclusion that previously would have been treated with LeFort 1 maxillary advancement surgery. Thus, it is not appropriate for patients requiring 2 jaw surgeries to correct mandibular prognathism, occlusal cants or facial asymmetry. The maxillary protraction appears to work by a combination of skeletal advancement, dental compensation and rotation of the occlusal planes. Microscrew/microimplant/temporary anchorage devices have been used with these maxillary protraction protocols to assist in expanding the maxilla, increasing skeletal anchorage during protraction, limiting dental compensations and reducing skeletal relapse. PMID:21765629

  12. Facts about Cleft Lip and Cleft Palate

    Science.gov (United States)

    ... children with orofacial clefts may have issues with self-esteem if they are concerned with visible differences between themselves and other children. Parent-to-parent support groups can prove to be ...

  13. Dental anomalies inside the cleft region in individuals with nonsyndromic cleft lip with or without cleft palate

    Science.gov (United States)

    Sá, Jamile; Araújo, Luana; Guimarães, Laís; Maranhão, Samário; Lopes, Gabriela; Medrado, Alena; Coletta, Ricardo

    2016-01-01

    Background Individuals with non syndromic cleft lip with or without cleft palate (NSCL±P) present high frequency of dental anomalies, which may represent complicating factors for dental treatment. The aim of this study was to investigate the prevalence of dental anomalies inside cleft area in a group of Brazilians with NSCL±P. Material and Methods Retrospective analysis of 178 panoramic radiographs of patients aged from 12 to 45 years old and without history of tooth extraction or orthodontic treatment was performed. Association between cleft type and the prevalence of dental anomalies was assessed by chi-square test with a significance level set at p≤ 0.05. Results Dental anomalies were found in 88.2% (n=157) of the patients. Tooth agenesis (47.1%), giroversion (20%) and microdontia (15.5%) were the most common anomalies. Individuals with unilateral complete cleft lip and palate (CLP, p<0.0001), bilateral complete CLP (p=0.0002) and bilateral incomplete CLP (p< 0.0001) were more affected by tooth agenesis than individuals with other cleft types. The maxillary lateral incisors were the most affected teeth (p<0.0001). Conclusions The present study revealed a high frequency of dental anomalies inside cleft region in NSCL±P patients, and further demonstrated that patients with unilateral complete CLP and bilateral incomplete CLP were frequently more affected by dental anomalies. Moreover, our results demonstrate that dental anomalies should be considered during dental treatment planning of individuals affected by NSCL±P. Key words:Nonsyndromic cleft lip with or without palate, dental anomaly, tooth agenesis, microdontia. PMID:26615505

  14. Implementing the Brazilian Database on Orofacial Clefts

    Directory of Open Access Journals (Sweden)

    Isabella Lopes Monlleó

    2013-01-01

    Full Text Available Background. High-quality clinical and genetic descriptions are crucial to improve knowledge of orofacial clefts and support specific healthcare polices. The objective of this study is to discuss the potential and perspectives of the Brazilian Database on Orofacial Clefts. Methods. From 2008 to 2010, clinical and familial information on 370 subjects was collected by geneticists in eight different services. Data was centrally processed using an international system for case classification and coding. Results. Cleft lip with cleft palate amounted to 198 (53.5%, cleft palate to 99 (26.8%, and cleft lip to 73 (19.7% cases. Parental consanguinity was present in 5.7% and familial history of cleft was present in 26.3% subjects. Rate of associated major plus minor defects was 48% and syndromic cases amounted to 25% of the samples. Conclusions. Overall results corroborate the literature. Adopted tools are user friendly and could be incorporated into routine patient care. The BDOC exemplifies a network for clinical and genetic research. The data may be useful to develop and improve personalized treatment, family planning, and healthcare policies. This experience should be of interest for geneticists, laboratory-based researchers, and clinicians entrusted with OC worldwide.

  15. Concomitant pituitary adenoma and Rathke's cleft cyst

    International Nuclear Information System (INIS)

    We reviewed the clinical, radiological and surgical findings in patients with both pituitary adenoma and Rathke's cleft cyst. We retrospectively selected patients with both lesions from the 374 patients in whom a sellar/juxtasellar lesion was detected on MRI at 1.5 tesla. All patients received intravenous contrast medium. Concomitant pituitary adenoma and Rathke's cleft cyst were found in eight patients (2.1 %). The frequency of the combination was 3.5 % of pituitary adenomas and 11 % of Rathke's cleft cysts. Symptoms were always due to the adenoma, secreting adrenocorticotrophin in two patients and growth hormone in six. The adenoma was larger in five patients, and the cyst in three. The cysts gave variable signal. The adenoma was adjacent to the cyst in seven patients, and enclosed it in the other patient. As a result of experience with MRI, concomitant pituitary adenoma and Rathke's cleft cyst are now known not to be as rare as thought previously. When a nonenhancing cyst-like structure is demonstrated in a patient with pituitary adenoma, the possibility of a coexisting Rathke's cleft cyst should be considered. (orig.)

  16. Cleft lip and palate repair

    Science.gov (United States)

    Orofacial cleft; Craniofacial birth defect repair; Cheiloplasty; Cleft rhinoplasty; Palatoplasty; Tip rhinoplasty ... these conditions at birth. Most times, cleft lip repair is done when the child is 6 to ...

  17. Congenital Cleft Hand

    OpenAIRE

    Aritamur, Ayhan; Cakmak, Mehmet; Taser, Omer; Berk, Hasan

    2004-01-01

    Congenital cleft hand deformity, which is also known with such names as cleft hand, lobster claw hand and Ectrodactyiy, is characterized by the absence of one or two fingers in the mid portion of the hand. A case of bilateral cleft hand deformity four years old, which is considerabiy rare, was reconstructed surgically. The result obtained has been presented. Because no sufficient experience has accumulated due to the fact that it is observed considerably rare, the therapeutical principales re...

  18. Cleft lip and palate: recommendations for dental anesthetic procedure based on anatomic evidences

    Directory of Open Access Journals (Sweden)

    Ivy Kiemle Trindade-Suedam

    2012-02-01

    Full Text Available Patients with cleft lip and palate usually present dental anomalies of number, shape, structure and position in the cleft area and the general dentist is frequently asked to restore or extract those teeth. Considering that several anatomic variations are expected in teeth adjacent to cleft areas and that knowledge of these variations by general dentists is required for optimal treatment, the objectives of this paper are: 1 to describe changes in the innervation pattern of anterior teeth and soft tissue caused by the presence of a cleft, 2 to describe a local anesthetic procedure in unilateral and bilateral clefts, and 3 to provide recommendations to improve anesthetic procedures in patients with cleft lip and palate. The cases of 2 patients are presented: one with complete unilateral cleft lip and palate, and the other with complete bilateral cleft lip and palate. The patients underwent local anesthesia in the cleft area in order to extract teeth with poor bone support. The modified anesthetic procedure, respecting the altered course of nerves in the cleft maxilla and soft tissue alterations at the cleft site, was accomplished successfully and the tooth extraction was performed with no pain to the patients. General dentists should be aware of the anatomic variations in nerve courses in the cleft area to offer high quality treatment to patients with cleft lip and palate.

  19. Primary unilateral cleft lip repair

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    Adenwalla H

    2009-10-01

    Full Text Available The unilateral cleft lip is a complex deformity. Surgical correction has evolved from a straight repair through triangular and quadrilateral repairs to the Rotation Advancement Technique of Millard. The latter is the technique followed at our centre for all unilateral cleft lip patients. We operate on these at five to six months of age, do not use pre-surgical orthodontics, and follow a protocol to produce a notch-free vermillion. This is easy to follow even for trainees. We also perform closed alar dissection and extensive primary septoplasty in all these patients. This has improved the overall result and has no long-term deleterious effect on the growth of the nose or of the maxilla. Other refinements have been used for prevention of a high-riding nostril, and correction of the vestibular web.

  20. Comparative study of three techniques of palatoplasty in patients with cleft of lip and palate via instrumental and auditory-perceptive evaluations

    Directory of Open Access Journals (Sweden)

    Paniagua, Lauren Medeiros

    2010-03-01

    Full Text Available Introduction: Palatoplasty is a surgical procedure that aims at the reconstruction of the soft and/or hard palate. Actually, we dispose of different techniques that look for the bigger stretching of the soft palate joint to the nasofaryngeal wall to contribute in the appropriate operation of the velopharyngeal sphincter. Failure in its closing brings on speech dysfunctions. Objective: To compare the auditory-perceptive' evaluations and instrumental findings in patients with cleft lip and palate operate through three distinctive techniques of palatoplasty. Method: A prospective transversal study of a group of patients with complete unilateral cleft lip and palate. Everybody was subjected to a randomized clinical essay, through distinctive techniques of palatoplasty performed for a single surgeon, about 8 years. In the period of the surgery, the patients were divided in three distinctive groups with 10 participants each one. The present study has evaluates: 10 patients of the Furlow technique, 7 patients of the Veau-Wardill-Kilner+Braithwaite technique and, 9 patients of the Veau-Wardill-Kilner+Braithwaite+Zetaplasty technique; having a total sample of 26 individuals. All the patients were subjected to auditory-perceptive evaluation through speech recording. An instrumental evaluation was also performed through video endoscopy exam. Results: The findings were satisfactory in the three techniques, in other words, the majority of the individuals does not present hyper nasality, compensatory articulatory disturbance and audible nasal air emission. In addition, in the instrumental evaluation, the majority of the individuals of the three techniques of palatoplasty present an appropriate velopharyngeal function. Conclusion: Was not found statistically significant difference between the palatoplasty techniques in both evaluations

  1. Is alveolar cleft reconstruction still controversial? (Review of literature)

    OpenAIRE

    Seifeldin, Sameh A.

    2015-01-01

    Cleft lip and palate (CL/P) is a frequent congenital malformation that manifests in several varieties including unilateral or bilateral and complete or incomplete. Alveolar cleft reconstruction remains controversial with regard to timing, graft materials, surgical techniques, and methods of evaluation. Many studies have been conducted addressing these points to develop an acceptable universal protocol for managing CL/P. The primary goal of alveolar cleft reconstruction in CL/P patients is to ...

  2. Diagnosis and presurgical orthopedics in infants with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Fundagul Bilgic

    2015-01-01

    Full Text Available Cleft lip and palate are one of the most common congenital craniofacial malformations. While preoperative treatment for infants with cleft lip and palate is still a scientific debate, patients with this malocclusion usually have to be treated from infancy to adulthood. Orthodontist plays an important role in the treatment of patients with cleft lip and palate. The purpose of this review is to give information about cleft lip and palate and presurgical nasoalveolar molding.

  3. Acute Liver Failure and Hepatic Encephalopathy After Cleft Palate Repair.

    Science.gov (United States)

    Kocaaslan, Nihal Durmuş; Tuncer, Fatma Betul; Tutar, Engin; Celebiler, Ozhan

    2015-09-01

    Paracetamol is the most commonly used analgesic after cleft palate repair. It has rarely caused acute hepatic failure at therapeutic or supratherapeutic doses. Only one case of therapeutic paracetamol toxicity after cleft palate repair had been reported previously. Here, we present a similar patient who developed acute liver failure and hepatic encephalopathy after an uncomplicated cleft palate surgery. Lack of large prospective trials in young children due to ethical concerns increases the value of the case reports of acetaminophen toxicity at therapeutic doses. The dosing recommendations of paracetamol may need to be reconsidered after cleft palate surgery.

  4. A Preliminary Three-Dimensional Analysis of Nasal Aesthetics Following Le Fort I Advancement in Patients With Cleft Lip and Palate.

    Science.gov (United States)

    Davidson, Edward; Kumar, Anand R

    2015-10-01

    Nasal aesthetic changes after cleft orthognathic surgery remain understudied. Previous scarring associated with prior cleft surgery may affect the predictability of outcomes after jaw surgery. This study evaluates changes in nasal aesthetics using three-dimensional photography after Le Fort I advancement in patients with nonsyndromic cleft-related maxillary hypoplasia. Cephalometric parameters were recorded pre- and postoperatively. Three-dimensional photogrammetric imaging analyzed changes in interalar width (IAW), internostril width (INW), nasal tip projection (NTP), collumelar length (CL), nasal labial angle (NLA), and nasal length (NL). Statistical significance between pre- and postoperative data was determined using T-tests for each parameter. Eleven patients underwent either single piece Le Fort I osteotomy and advancement, (3 bilateral, 4 unilateral cleft lip, and palate), or 2-piece advancement (2 bilateral, 2 unilateral). Average nasal soft tissue changes were IAW 1.9 mm (0.4-4.2), INW -0.2 mm (-2.8 to 1.6), NTP -1.0 mm (-4.0 to 2.0), CL -0.7 mm (-2.9 to 1.5), NLA -0.2° (-13.9 to 15.1), and NL -0.7 mm (-4.3 to 1.5), (P = 0.001, 0.6, 0.08, 0.01, 0.9, 0.2). For single-piece osteotomy alone changes were IAW 2.1 mm (0.6-4.1), INW -0.6 mm (-2.8 to 1.7), NTP -1.9 mm (-4.0 to 0.3), CL -1.2 mm (-2.9 to 0.03), NLA -1.3° (-13.9 to 15.0), and NL -1.1 mm (-4.3 to 0.7), (P = 0.007, 0.3, 0.009, 0.0002, 0.7, 0.2). For 2-piece osteotomy alone changes were IAW 1.6 mm (-0.4 to 3.3), INW 0.5 mm (0.4-1.6), NTP 0.5 mm (-1.1-2.0), CL 0.2 mm (-1.4 to 1.5), NLA 2.8° (-7.6 to 10.1), and NL -0.1 mm (-1.4 to 1.5), (P = 0.2, 0.4, 0.5, 0.6, 0.5, 0.9). Cleft-related scarring and malposition affect changes in nasal aesthetics following maxillary advancement that are different to the noncleft population. Two-piece Le Fort I increases variability of changes in nasal aesthetics compared with single-piece advancement.

  5. Disease: H00752 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00752 Ankyloblepharon-ctodermal defects-cleft lip/palate (AEC) syndrome and Rapp-H...odgkin syndrome Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome (also known as Hay-Wells ...5289 OMIM: 106260 129400 PMID:20556892 (descritpion, gene) Sutton VR, Bree AF, van Bokhoven H Ankyloblepharon-Ectodermal Defects

  6. Transverse facial cleft: A series of 17 cases

    Directory of Open Access Journals (Sweden)

    L K Makhija

    2011-01-01

    Full Text Available Introduction: Transverse facial cleft (Tessier type 7 or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births. Material and Methods: Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications. Result: Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17 were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin′s Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the17 cases, 15 were operated and in most of them the outcome was satisfactory.

  7. Median cleft lip: A new method of surgical repair

    Directory of Open Access Journals (Sweden)

    Khandekar B

    2010-01-01

    Full Text Available The aim is to discuss a new method of muscle repair in midline cleft lip. Three patients with midline cleft lip were repaired with our technique of muscle repair and the results evaluated. Our new method of muscle repair in the form of ′Z′ helps in forming the philtral dimple.

  8. Effects of Brazilian Propolis on Dental Plaque and Gingiva in Patients with Oral Cleft Malformation Treated with Multibracket and Removable Appliances: A Comparative Study

    Directory of Open Access Journals (Sweden)

    Agnieszka Machorowska-Pieniążek

    2016-01-01

    Full Text Available Orthodontic appliances modify the local environment of the oral cavity, increase the accumulation of dental plaque, and affect the condition of the gingiva. The aim of this study is assessment of Brazilian propolis toothpaste’s effect on plaque index (PLI and gingival index (GI in patients with CL/CLP treated using orthodontic appliances in the 35-day study period. The study population included 96 patients of an Orthodontic Outpatient Clinic, ACSiMS in Bytom. All the patients participated in the active phase of orthodontic treatment using buccal multibracket appliances or removable appliances. During the first examination, each patient was randomly qualified to the propolis group or control group. A statistically significant decrease in GI and PLI in the entire propolis group (P<0.01 was shown during repeated examination. Insignificant change in GI was in the entire control group during the repeated examination compared to the baseline. Similar result was obtained in patients treated with multibracket and removable appliances. The orthodontic appliance type did not affect the final dental plaque amount and gingival condition in patients using the propolis toothpaste. These results may be clinically useful to improve prevention and control oral infectious diseases during orthodontic treatment patients with oral cleft.

  9. Laryngo-tracheo-oesophageal clefts

    Directory of Open Access Journals (Sweden)

    Leboulanger Nicolas

    2011-12-01

    Full Text Available Abstract A laryngo-tracheo-esophageal cleft (LC is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%, mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4, and gastro-esophageal reflux disease (GERD. The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of

  10. 桂中地区先天性唇腭裂1021例发病因素分析%Analysis of Risk Factors of 1 021 Patients with Congenital Cleft Lip and Palate in Central Region of Guangxi

    Institute of Scientific and Technical Information of China (English)

    王伯钧; 韦元强; 玉铭; 秦小云; 韦进

    2012-01-01

    Objective To explore the risk factors of congenital cleft lip and palate in central region of Guangxi, and to investigate the preventive methods of congenital cleft lip and palate. Methods A clinical data of 1021 patients with congenital cleft lip and palate in central region of Guangxi were analyzed in the study. Results Of 1 021 patients, there were 652 men and 369 women, the ratio of male to female was 1.77 : 1,609 patients( 59.65 % ) with cleft lip, 299 patients ( 29.29% ) with cleft palate,113 patientsC 11.07% ) with cleft lip and palate. 940 patientsC 92.07% ) came from rural areas,81 patientsC 7.93% ) from towns;The proportion of patients from rural areas was significantly higher than that of patients from the city. The risk factors ranked in the top three as follows: flu occurred in the first 3 months of pregnancy ( 53. 57% ), pregnancy malnutrition( 14. 40% ), medicine intake during pregnancy( 9. 40% ). 954 patients ( 93. 43% ) with congenital cleft lip and palate experienced risk factors in the early stage of pregnancy,46 patients ( 4.50% ) had familial genetic history. Conclusion Virus infection and nutritional factors are the major risk factors of incidence of congenital cleft lip and palate. It should be paid more attention to universal health care knowledge during pregnancy to reduce the incidence of congenital cleft lip and palate.%目的 分析桂中地区先天性唇腭裂的发病因素,探讨先天性唇腭裂的预防方法.方法 对桂中地区先天性唇腭裂1 021例患者的临床资料进行分析.结果 1 021例患者中,男652例,女369例,男女之比为1.77:1;唇裂609例(59.65%),腭裂299例(29.29%),唇腭裂113例(11.07%).患者来自农村 940例(92.07%),城镇81例(7.93%),农村比例明显高于城市.发病因素前3位分别是妊娠前3个月感冒(53.57%)、孕期营养缺乏(14.40%)、孕期服药(9.30%).孕早期经历危险因素954例占93.43%,有家族遗传病史者46例占4.51%.结论 病毒感染及营养因素是先

  11. Patau sendromlu üç olgu: Sendroma klinik triad (mikroftalmi, yarık dudak/ damak, polidaktili) her zaman eşlik eder mi?

    OpenAIRE

    Hazan, F; Olukman, Ö; Gökaslan, F; Çalkavur, Ş; Meşe, T.; Tavlı, V; Atlıhan, F

    2013-01-01

    Trisomy 13 (Patau's syndrome) is a chromosomal disease characterized by midline defects, central nervous system, heart, urogenital system anomalies and motor mental retardation. The prevalence is between 1:10.000 and 1:20.000 births. A vast majority of the patients die in the first year. Patau's syndrome is characterized by the clinical triad including microphtalmia, cleft lip/palate, and polydactyly. Here, we present three patients who were diagnosed with Patau's syndrome both clinically and...

  12. Genetics of Cleft Lip and Cleft Palate

    OpenAIRE

    Leslie, Elizabeth J.; Marazita, Mary L.

    2013-01-01

    Orofacial clefts are common birth defects and can occur as isolated, nonsyndromic events or as part of Mendelian syndromes. There is substantial phenotypic diversity in individuals with these birth defects and their family members: from subclinical phenotypes to associated syndromic features that is mirrored by the many genes that contribute to the etiology of these disorders. Identification of these genes and loci has been the result of decades of research using multiple genetic approaches. ...

  13. Crecimiento sagital maxilar en fisurados unilaterales operados funcionalmente Sagittal maxillary growth in unilateral cleft lip and palate patients following functional surgery

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    F. Donoso Hofer

    2007-06-01

    Full Text Available Objetivo. Comparar el crecimiento sagital maxilar en pacientes con fisura labio-máxilo-palatina unilateral operados a los 6 meses con criterio funcional con pacientes normales que tengan relación consanguínea directa con los anteriores. Diseño del estudio. Análisis arquitectural y craneofacial de Delaire en telerradiografías de perfil en ambos grupos de pacientes cuyas edades fluctúan actualmente entre los 7 y los 12 años, determinando el crecimiento sagital del maxilar a través de la medida del ángulo del pilar maxilar anterior (C1/F1, sometiendo las medidas al test T de Student con una significación del 99,5%. Resultados.Se determinó el valor real y esperado para el ángulo del pilar maxilar anterior en todos los casos. Al comparar estadísticamente los resultados, no se encontraron diferencias significativas en los valores promedios obtenidos. Conclusiones.El crecimiento sagital maxilar de los pacientes con fisura labio-máxilo-palatina unilateral operados a los 6 meses con criterio funcional no difiere del de aquellos pacientes normales.Objective. To compare the sagittal maxillary growth between unilateral cleft lip and palate patients operated under functional criterion at the age of 6 months and normal patients who were blood-related. Design. Delaire’s Architectural and Structural craniofacial analysis in conventional lateral radiographs of all the patients with an age range of 7-12 years, determining the sagittal maxillary growth by the anterior maxillary pillar angle (C1/F1. These measurements were analyzed using the T-test with a 99.5% significance. Results. The real and expected value of the anterior maxillary pillar angle was determined in all cases. By comparing the results statistically, no significant differences were found in the mean values obtained. Conclusion. Maxillary sagittal growth in unilateral cleft lip and palate patients operated at the age of 6 months under functional criterion, does not differ from the

  14. Resultados del manejo multidisciplinario del labio y paladar fisurado unilateral Results of the multidisciplinary management of unilateral cleft lip and palate patients

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    M. Estrada Sarmiento

    2007-06-01

    Full Text Available Se diseñó un modelo para evaluar el protocolo y la estrategia del equipo multidisciplinario para el tratamiento del labio y paladar fisurado unilateral en el Servicio de Cirugía Máxilo Facial del Hospital Provincial Universitario "Carlos Manuel de Céspedes" de Bayamo. Cuba. El estado presente de 60 pacientes de 14-22 años de edad fue evaluado por un cirujano máxilo facial, un ortodoncista y un foníatra. Los resultados indicaron que más de la mitad de esos pacientes tenían completado el tratamiento por uno de los especialistas. Solo 27 pacientes (16,2% habían completado el tratamiento por los tres especialistas. Los factores que contribuyeron a este porcentaje fueron discutidos.A model was designed to evaluate the protocol and strategy of the interdisciplinary team treating unilateral cleft lip and palate patients at the Maxillofacial Surgery department of the "University Provincial Hospital Carlos Manuel de Cespedes" Bayamo, Granma, Cuba. A maxillofacial surgeon, an orthodontist and a phoniatrician evaluated the current state of 60 patients with ages ranging between 14 and 22. The results showed that more than half of the patients had finished their treatment by one of the specialists. Only 27 patients (16.2% had completed their treatment by the three specialists. The factors contributing to these percentages are discussed.

  15. Acro-cardio-facial syndrome

    OpenAIRE

    Dallapiccola Bruno; Digilio Maria

    2010-01-01

    Abstract Acro-cardio-facial syndrome (ACFS) is a rare genetic disorder characterized by split-hand/split-foot malformation (SHFM), facial anomalies, cleft lip/palate, congenital heart defect (CHD), genital anomalies, and mental retardation. Up to now, 9 patients have been described, and most of the reported cases were not surviving the first days or months of age. The spectrum of defects occurring in ACFS is wide, and both interindividual variability and clinical differences among sibs have b...

  16. Submucous cleft palate and the general practitioner

    Science.gov (United States)

    Lowry, R. B.; Courtemanche, A. D.; MacDonald, C.

    1973-01-01

    Submucous cleft palate refers to a situation where the soft palate is largely composed of mucosa with little or no muscle. The defect is often not obvious on inspection of the mouth and pharynx. There is considerable clinical variation, with speech ranging from normal or minimal nasality to severe nasality and defective articulation. Many patients who have latent submucous cleft palate have the condition unmasked by an adenoidectomy because the adenoid pad had served as a compensatory factor in effecting palatopharyngeal closure. All physicians who perform tonsillectomy and adenoidectomy should be aware of the signs and symptoms which may suggest the diagnosis. ImagesFIG. 2 PMID:4758872

  17. Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

    Science.gov (United States)

    Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

    2012-01-01

    The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes. PMID:22759387

  18. Alveolar bone grafting in the treatment of midline alveolar cleft and diastema in incomplete median cleft lip.

    Science.gov (United States)

    Liao, H-T; Chen, C-H; Bergeron, L; Ko, E W-C; Chen, P K T; Chen, Y-R

    2008-10-01

    Median cleft lip is a rare congenital anomaly. The wide diastema with mesial tipping observed in these patients has been largely overlooked. A midline submucosal alveolar cleft prevents adequate treatment. The purpose of this article is to describe an alveolar bone grafting (ABG) technique used in the combined surgical-orthodontic approach to diastema treatment in patients presenting with incomplete median cleft lip. Patients treated for incomplete median cleft lip and diastema were identified in the clinic registry from 1981 to 2007. Six patients were identified; 4 underwent ABG before permanent maxillary incisor eruption, the other 2 were seen later when they were 11 years old. All 6 ABGs were successful. The incisors erupted through the graft or were successfully moved into it with lasting results. Follow-up ranged from 8 to 21 years. The existence of a midline submucosal alveolar cleft and subsequent diastema should be recognized and addressed in all patients who present with incomplete median cleft lip repair. This includes taking maxillary occlusal view X-rays before the age of 5 years to detect the cleft, and proceed to ABG if necessary, generally before permanent maxillary incisor eruption. PMID:18771899

  19. Presurgical nasoalveolar moulding in unilateral cleft lip and palate

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    Mohammed Zuhaib

    2016-01-01

    Full Text Available Context: Presurgical nasoalveolar moulding (PNAM is a non-surgical method of reshaping the cleft lip, alveolus, palate and the nose to minimize the severity of the cleft deformity, before primary cheiloplastyand palatoplasty. In this context, PNAM proves to be an invaluable asset in the management of unilateral cleft lip and palate. Aims: The study was conducted to evaluate the effi cacy of PNAM in the management of unilateral cleft lip and palate with the following objectives: (1 To assess and compare the degree of reduction in the size of cleft palate and alveolus (pre-PNAM and post-PNAM. (2 To evaluate and compare the improvement in columellar length and correction of columellar deviation (pre-PNAM and post-PNAM. (3 To assess the changes in the position of the alar base and the alar cartilages. Settings and Design: Prospective study. Subjects and Methods: A prospective study consisting of, which included 20 patients with complete unilateral cleft lip and palate was conducted. The age at the start of PNAM treatment of the infants ranged from 2 to 44 days of age reporting to our institute between December 2011 and August 2013. All the patients underwent PNAM therapy before primary cheiloplasty at 6 months of age; clinical parameters were assessed pre- and post-therapy using photographs and dental study models of the maxilla. Statistical Analysis Used: Student's t-test for paired comparisons. Results: Results of the study showed a promising reduction in the cleft size before the surgery, signifi cant improvement in nasal symmetry, including the columellar length on the cleft side. Conclusions: PNAM is a valuable adjunct to our surgical armamentarium in dealing with the challenges of primary closure of unilateral cleft lip and palate thereby enhancing the overall surgical outcome. The advantages of this method include the simplicity of the procedure and improving the quality of surgical repair, particularly in obtaining tension free muscle

  20. Syndromes associated with labiopalatine clefting: A report of three cases

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    Thomas A

    2008-06-01

    Full Text Available Clefts of the lip and palate are a common craniofacial anomaly, The etiology is thought to be multifactorial, with both genetic and environmental factors playing a role. Because of the frequent manifestation of cleft lip and palate in association with some syndromes, it is essential that a detailed examination of these patients be carried out. This facilitates early formulation of preventive and treatment advice and aids in overcoming gross deformities in the early stages of development. In this paper, we review three such patients who presented with cleft lip and palate in association with a syndrome.

  1. Analysis of polymorphic TGFB1 codons 10, 25, and 263 in a German patient group with non-syndromic cleft lip, alveolus, and palate compared with healthy adults

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    Gressner Axel M

    2004-06-01

    Full Text Available Abstract Background Clefts of the lip, alveolus, and palate (CLPs rank among the most frequent and significant congenital malformations. Leu10Pro and Arg25Pro polymorphisms in the precursor region and Thr263Ile polymorphism in the prodomain of the transforming growth factor β1 (TGF-β1 gene have proved to be crucial to predisposition of several disorders. Methods In this study, polymorphism analysis was performed by real-time polymerase chain reaction (LightCycler and TGF-β1 levels determined by enzyme-linked immunosorbent assay. Results Only 2/60 Caucasian non-syndromic patients with CLP (3.3% carried the Arg25Pro and another 2/60 patients (3.3% the Thr263Ile genotypes, whereas, in a control group of 60 healthy Caucasian blood donors, these heterozygous genotypes were more frequent 16.7% having Arg25Pro (10/60; p Conclusions The genetic differences in codons 25 and 263 suggest that TGF-β1 could play an important role in occurrence of CLP, however, functional experiments will be required to confirm the mechanisms of disturbed development.

  2. The cleft team social worker.

    Science.gov (United States)

    Kaye, Alison; Lybrand, Sandra

    2016-04-01

    The birth of a child with significant medical problems poses challenges for most families. Congenital orofacial clefting is a common condition affecting families worldwide. Orofacial clefting requires long-term medical care and can affect multiple body systems. Having a child with a chronic medical condition such as cleft lip or palate creates many psychosocial ramifications for a family. This article describes the importance of medical social work involvement in the coordinated care for children with cleft lip and palate. Specific cases spanning prenatal care through adolescence are used to highlight the variety of complex psychosocial situations encountered in the multidisciplinary cleft team setting.

  3. Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts

    OpenAIRE

    Lim, Han Hyuk; Yang, Sei Won

    2010-01-01

    Purpose This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. Methods Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients....

  4. Facial aesthetics and perceived need for further treatment among adults with repaired cleft as assessed by cleft team professionals and laypersons.

    Science.gov (United States)

    Foo, Peter; Sampson, Wayne; Roberts, Rachel; Jamieson, Lisa; David, David

    2013-06-01

    The objectives of this study were to compare the ratings of professionals and laypeople with and without a cleft regarding the facial aesthetics of adult patients previously treated for orofacial clefting. The necessity for further treatment, as perceived by the respective groups, is also compared. The design of the study was a cross-sectional study. Professionals (two plastic surgeons, one dentist, one orthodontist, and one psychologist) and laypeople (one male and one female adult without a cleft and one male and one female adult with a cleft) were recruited to rate photographs of 80 non-syndromic cleft patients treated by the Australian Craniofacial Unit from 1975 to 2009. Facial aesthetics were measured by a visual analogue scale (VAS; 0-100 mm). High values indicated good aesthetics. Necessity for further treatment was also measured by a VAS (0-100 mm). High values indicated high perceived need for further treatment. The professionals rated facial aesthetics significantly lower and had a lower perception of need for further treatment than the raters with and without a cleft. The laypeople with a cleft rated facial aesthetics significantly higher and had a lower perceived need for further treatment than laypeople without a cleft. The non-surgical professionals rated facial aesthetics significantly lower and had a lower perceived need for further treatment than the surgical professionals. Differences exist in the facial aesthetics ratings and perceived need for further surgery between professionals and laypeople with and without a cleft. This should be considered when managing cleft treatment expectations.

  5. Root development of permanent lateral incisor in cleft lip and palate children: A radiographic study

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    Amarlal Deepti

    2007-01-01

    Full Text Available Objective: The objective of this study was to compare the root development of lateral incisor on the cleft side with the root development of its contralateral tooth in cleft lip and palate children. Setting: Cleft lip and palate wing, Meenakshi Ammal Dental College and Hospital, Chennai, South India. Materials and Methods: A sample of 96 orthopantamograms of patients with unilateral or bilateral cleft lip and/or cleft palate was selected, regardless of sex and race. Main Outcome Measure: Orthopantamograms were analyzed for root development of lateral incisor on the cleft and noncleft side. Associated anomalies like hypodontia, supernumerary teeth, malformed lateral incisors and root development of canine, if present, were recorded. Findings and Conclusions: Root development of permanent lateral incisor was delayed on the cleft side compared to the noncleft side. There was a statistically significant relationship between levels of root development of lateral incisors on the cleft side within the different study groups ( P < 0.05. Incidence of hypodontia increased in proportion to cleft severity. Frequency of missing second premolars, supernumerary teeth and malformed lateral incisors increased in cleft lip and palate patients. Root development of canine showed a slight delay on the cleft side when compared to the canine on the noncleft side.

  6. 正颌联合术后正畸矫治唇腭裂术后的牙颌面畸形%Orthognathic Surgery and Postsurgical Orthodontics for Correction of Secondary Dentofacial Deformities in Cleft Lip and Palate Patients

    Institute of Scientific and Technical Information of China (English)

    孙晓梅; 滕利; 丁波; 归来

    2012-01-01

    目的 探讨应用正颌手术联合术后正畸矫治唇腭裂术后牙颌面畸形的方法.方法 2002年1月至2010年8月,共收治25例唇腭裂术后颌骨畸形患者.男11例,女14例;年龄16~33岁;单侧唇腭裂19例,双侧6例.所有患者术前均未接受过正畸治疗.本组患者均联合应用正颌手术和术后正畸建(牙合)矫治唇腭裂术后颌骨畸形.结果 25例患者经过3~12个月的术后正畸治疗,均建立了良好的咬(牙合)关系,恢复正常咬合功能.随访6个月至3年,术后疗效稳定.结论 唇腭裂术后颌骨畸形采用正颌手术联合术后正畸能够有效矫治牙颌面畸形.%Objective To investigate an effective method of orthognathic surgery and postsurgical orthodontics for correction of secondary dentofacial deformities in cleft lip and palate patients. Methods From January 2002 to August 2010, 25 cleft patients suffering from secondary dentofacial deformities were treated. There were 11 males and 14 females, aged from 16 to 33 years (mean age: 22 years), 19 patients were unilateral cleft and 6 were bilateral cleft. All patients had not received presurgical orthodontics and were treated by orthognathic surgery accompanied with postsurgical orthodontics for deformities correction. Results All 25 patients were satisfied with their appearances and dental articulation after operation. All patients got the normal occlusion and the satisfactory intermaxillary relationship with orthodontics treatment of 3-12 months. After a follow up of 6 months to 3 years,the results of the treatment were stable. Conclusion The orthognathic surgery and postsurgical orthodontics is efficient for correction of secondary dentofacial deformities in cleft patients.

  7. Clefting and psychosocial adjustment. Influence of facial aesthetics.

    Science.gov (United States)

    Tobiasen, J M; Hiebert, J M

    1993-10-01

    This article briefly reviewed the research literature on the psychosocial correlates of facial clefts and described a program of research to study the relationship between severity of cleft impairment and psychosocial adjustment. In the past 40 years, there has been increasing recognition and research literature on the psychologic implications of facial clefts to patients and their families. Advances in both the knowledge base and the science of the psychologic correlates of facial clefts have been made. Children with clefts are not at greater risk for psychopathology than are individuals without clefts; however, they are at significant risk for social competence problems relating to development of friendships, progress in school, and participation in organizations. Problems with social competence have a negative effect on development. The ability of all children to make friends and to be liked by others is considered by most parents, teachers, and child development specialists to be a major developmental milestone. Not having friends and social withdrawal can cause parents or teachers to refer noncleft children to mental health professionals and is a predictor of impaired adult social competence and mental health. Studies of adults with clefts are consistent with studies of adults without clefts. Adults with repaired clefts are less likely to marry than are their noncleft siblings, and they have more problems with social withdrawal. Because facial attractiveness is well-known to affect peer acceptance, we hypothesized that the severity of the cleft deformity may have a significant impact on social competence. Consequently, we undertook a program of research to examine this question.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8275628

  8. Branchial cleft cyst: A case report and review of literature

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    Surekha Chavan

    2014-01-01

    Full Text Available First branchial cleft anomaly is a rare disease of the head and neck. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. In this article, we present a case of type II first branchial cleft anomaly. A middle-aged woman who had suffered from swelling on lower jaw visited our department with the chief complaint of a swelling. She underwent complete excision of the lesion with preservation of the facial nerve. The patient recovered well and had no recurrence at 1-year of follow up.

  9. Atendimento fonoaudiológico intensivo em pacientes operados de fissura labiopalatina: relato de casos Intensive speech therapy in patients operated for cleft lip and palate: case report

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    Maria do Rosário Ferreira Lima

    2007-09-01

    Full Text Available Devido à carência de fonoaudiólogos para atendimento ao paciente com fissura labiopalatina em várias regiões do Brasil, novos programas de atendimento devem ser desenvolvidos para esses indivíduos. A terapia intensiva de fala tem sido relatada na literatura como uma modalidade alternativa. Este trabalho relata a experiência com alguns casos de atendimento fonoaudiológico intensivo, e compara o desempenho na produção da fala de quatro pacientes operados de fissura palatina, antes e após a terapia fonoaudiológica intensiva. Foram atendidos, no período de férias escolares, três adultos e um adolescente que apresentavam distúrbios articulatórios compensatórios. O atendimento teve duração de três horas diárias para cada paciente, durante dez dias, divididos em terapia individual e em grupo. No início e fim do período de terapia, os pacientes foram avaliados por uma fonoaudióloga que não participou dos atendimentos. Também foi gravada em vídeo uma amostra de fala espontânea, contagem de 1 a 20 e repetição de uma lista de palavras e frases com fonemas oclusivos orais e fricativos. Todos os pacientes mostraram evolução satisfatória na terapia intensiva, com adequação dos fonemas trabalhados na fala dirigida, necessitando ainda de acompanhamento fonoterápico para sua automatização. A terapia intensiva mostrou ser uma alternativa eficaz e viável nesses casos, podendo também ser uma estratégia durante o início do tratamento fonoaudiológico convencional.Due to the lack of speech therapists at various regions of Brazil to assist patients with cleft lip and palate, new intervention programs must be developed for these individuals. Intensive speech therapy has been cited in literature as an alternative modality. This article relates the experience of four cleft lip patients, comparing their speech performances before and after the intensive intervention. The subjects, three adults and one adolescent with compensatory

  10. Kyphosis reduction and the rate of cement leaks after vertebroplasty of intravertebral clefts

    Energy Technology Data Exchange (ETDEWEB)

    Krauss, Martin; Tomandl, Bernd; Baer, Ingrid [Klinikum Sued, Institute for Diagnostic and Interventional Radiology, Nuernberg (Germany); Hirschfelder, Horst; Lichti, Gabriele [Klinikum Sued, Department of Physical and Rehabilitative Medicine, Nuernberg (Germany)

    2006-05-15

    To assess the results of vertebroplasty in patients with intravertebral clefts compared to patients with normal osteoporotic fractures, we evaluated the pre- and postoperative images and pain scores (VAS) of 114 patients with 192 vertebroplasty procedures treated between March 2002 and February 2005. Intravertebral clefts were identified on conventional radiographs, MR or CT images as gas- or fluid-filled spaces adjacent to an endplate of a fractured vertebra. Forty-four vertebrae showed intravertebral clefts. All clefts were filled with PMMA showing a typical filling pattern. Due to the prone positioning of the patient during vertebroplasty, a significant reduction of the kyphosis angle was achieved in the cleft group. Cement leakage occurred in 18.2% of clefts and 46% of regular osteoporotic fractures. In all patients, good filling of the cleft was achieved no matter where the needle tip was placed in the vertebra. The VAS score was 9.1 preoperatively, 3.6 before discharge and 3.9 6 months postoperatively, showing no significant difference between both groups. Patients with intravertebral clefts show a significant reduction of the kyphosis angle compared to non-cleft patients and have a significantly lower risk of experiencing cement leakage during vertebroplasty. Pain reduction is the same in both groups. (orig.)

  11. Alimentação da criança com fissura de lábio e/ou palato: um estudo bibliográfico Alimentación del niño com fisura del labio y/o paladar: um estudio bibliográfico Alimentation of children with lip-palate malformation: a bibliographic study

    Directory of Open Access Journals (Sweden)

    Raimunda da Costa Araruna

    2000-04-01

    ón.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.

  12. Contemporary Approaches in the Repair of Alveolar Clefts

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    Ufuk Tatli

    2014-08-01

    Full Text Available Cleft lip and palate is one of the most common craniofacial anomalies. The repair of the alveolar clefts is an important part of the treatment for patients with cleft lip and palate. The treatment concepts of alveolar bone grafting are still controversial. The corresponding controversial issues are; timing of alveolar bone grafting, graft materials, and timing of the orthodontic expansion. In the present article, aforementioned controversial issues and contemporary treatment modalities of the maxillary alveolar clefts were reviewed in the light of current literature. In conclusion, the most suitable time for alveolar bone grafting is mixed dentition period. Grafting procedure may be performed in the early or late phases of this period depending on some clinical features. Adjunct orthodontic expansion procedures should be performed before and/or after grafting depending on the patient's current features. [Archives Medical Review Journal 2014; 23(4.000: 563-574

  13. Considerations Regarding Age at Surgery and Fistula Incidence Using One- and Two-stage Closure for Cleft Palate

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    Simona Stoicescu

    2013-12-01

    Full Text Available Introduction: Although cleft lip and palate (CLP is one of the most common congenital malformations, occurring in 1 in 700 live births, there is still no generally accepted treatment protocol. Numerous surgical techniques have been described for cleft palate repair; these techniques can be divided into one-stage (one operation cleft palate repair and two-stage cleft palate closure. The aim of this study is to present our cleft palate team experience in using the two-stage cleft palate closure and the clinical outcomes in terms of oronasal fistula rate. Material and methods: A retrospective analysis was performed on medical records of 80 patients who underwent palate repair over a five-year period, from 2008 to 2012. All cleft palate patients were incorporated. Information on patient’s gender, cleft type, age at repair, one- or two-stage cleft palate repair were collected and analyzed. Results: Fifty-three (66% and twenty-seven (34% patients underwent two-stage and one-stage repair, respectively. According to Veau classification, more than 60% of them were Veau III and IV, associating cleft lip to cleft palate. Fistula occurred in 34% of the two-stage repairs versus 7% of one-stage repairs, with an overall incidence of 24%. Conclusions: Our study has shown that a two-stage cleft palate closure has a higher rate of fistula formation when compared with the one-stage repair. Two-stage repair is the protocol of choice in wide complete cleft lip and palate cases, while one-stage procedure is a good option for cleft palate alone, or some specific cleft lip and palate cases (narrow cleft palate, older age at surgery

  14. Clefting in pumpkin balloons

    Science.gov (United States)

    Baginski, F.; Schur, W.

    NASA's effort to develop a large payload, high altitude, long duration balloon, the Ultra Long Duration Balloon, focuses on a pumpkin shape super-pressure design. It has been observed that a pumpkin balloon may be unable to pressurize into the desired cyclically symmetric equilibrium configuration, settling into a distorted, undesired stable state instead. Hoop stress considerations in the pumpkin design leads to choosing the lowest possible bulge radius, while robust deployment is favored by a large bulge radius. Some qualitative understanding of design aspects on undesired equilibria in pumpkin balloons has been obtained via small-scale balloon testing. Poorly deploying balloons have clefts, but most gores away from the cleft deploy uniformly. In this paper, we present models for pumpkin balloons with clefts. Long term success of the pumpkin balloon for NASA requires a thorough understanding of the phenomenon of multiple stable equilibria and means for quantitative assessment of measures that prevent their occurrence. This paper attempts to determine numerical thresholds of design parameters that distinguish between properly deploying designs and improperly deploying designs by analytically investigating designs in the vicinity of criticality. Design elements which may trigger the onset undesired equilibria and remedial measures that ensure deployment are discussed.

  15. ENDOTRACHEAL INTUBATION IN A CHILD HAVING OCCIPITAL ENCEPHALOCELE WITH BILATERAL CLEFT LIP AND CLEFT PALATE: A CASE REPORT

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    Shridhar N

    2014-11-01

    Full Text Available : Basically endotracheal intubation in pediatric age group especially in infants is difficult due to large head, relatively large tongue, anteriorly placed larynx, longer and stiffer epiglottis which protrudes at 450 angle and short neck. When such pediatric patient comes with craniofacial congenital malformations, the management of airway becomes more challenging. Here we report a case of occipital encephalocele associated with bilateral cleft lip and cleft palate coming for V P shunt procedure.

  16. Unilateral Cleft Lip and Nasal Repair: Techniques and Principles

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    Mohammad-Ali Raisolsadat

    2011-06-01

    Full Text Available The Mashhad University of Medical Sciences and the Sheikh Hospital in Mashhad sponsored a Cleft Lip and Palate Workshop 30 April - 1 May 2009. During the Workshop, 6 surgical cases were performed and televised live to the audience attending the conference. Two of those cases were unilateral cleft lip repairs. The surgical technique used to repair these patients by the primary author (JGM is a hybrid technique. It has evolved over the last decade as a result of prior surgical literature as well as first hand observation of various surgical colleagues. The following manuscript describes the surgical technique used at the Cleft Workshop in a step-wise or atlas-like fashion. The technique portion of the paper describes the repair of the unilateral cleft lip and nasal deformity in roughly the order the first author typically performs the procedure. More importantly, the final section of the paper details the principles that form the foundation for the techniques described.

  17. Is alveolar cleft reconstruction still controversial? (Review of literature

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    Sameh A. Seifeldin

    2016-01-01

    Full Text Available Cleft lip and palate (CL/P is a frequent congenital malformation that manifests in several varieties including unilateral or bilateral and complete or incomplete. Alveolar cleft reconstruction remains controversial with regard to timing, graft materials, surgical techniques, and methods of evaluation. Many studies have been conducted addressing these points to develop an acceptable universal protocol for managing CL/P. The primary goal of alveolar cleft reconstruction in CL/P patients is to provide a bony bridge at the cleft site that allows maxillary arch continuity, oronasal fistula repair, eruption of the permanent dentition into the newly formed bone, enhances nasal symmetry through providing alar base support, orthodontic movement and placement of osseointegrated implants when indicated. Other goals include improving speech, improvement of periodontal conditions, establishing better oral hygiene, and limiting growth disturbances. In order to rehabilitate oral function in CL/P patients alveolar bone grafting is necessary. Secondary bone grafting is the most widely accepted method for treating alveolar clefts. Autogenous bone graft is the primary source for reconstructing alveolar cleft defects and is currently the preferred grafting material.

  18. 中国汉族不伴唇腭裂的缺指(趾)-外胚层发育异常-唇腭裂综合征TP63基因的杂合性突变一例%Heterozygous TP63 mutation in a Chinese patient with ectrodactyly-ectodermal dysplasia clefting syndrome without clefting

    Institute of Scientific and Technical Information of China (English)

    韩冬; 吴华; 张晓霞; 冯海兰

    2010-01-01

    Objective To determine if alteration in TP63 is responsible for a Chinese patient with ectrodactyly-ectodermal dysplasia clefting (EEC) syndrome, but without cleft palate/lip. Methods Screening of TP63 gene was performed in the patient with EEC syndrome and his family members using PCR-single strand conformational polymorphism (SSCP) analysis, then performed by direct sequencing of the coding region. Results A C > T substitution at nucleotide position 838 in exon 7 was detected in the patient, and the change predicted a heterozygous missense mutation, Arg280Cys. His parents showed the wild type. Conclusions The results indicate that the de novo mutation Arg280Cys of the TP63 gene observed in the patient maybe contribute to his EEC syndrome.%目的 研究不伴唇腭裂表现的缺指(趾)-外胚层发育异常-唇腭裂(ectrodactylyectodermal dysplasia clefting,EEC)综合征1例患者的TP63基因突变,探讨该病基因型与表型的关系.方法 利用DNA单链构象多态性实验对1个中国汉族不伴唇腭裂表现的EEC综合征核心家系进行突变初筛,聚合酶链反应扩增目的 基因片段,直接测序进行突变检测,以200名无先天性缺牙的健康者作为对照.结果 在患者TP63基因第7外显子cDNA838位存在C>T的单碱基杂合性点突变,使其编码的第280位精氨酸替换为半胱氨酸(Arg280Cys,R280C).患者父母在该位点均显示正常的野生基因型.结论 TP63基因的单碱基杂合性突变(Arg280Cys,R280C)是引起该患者EEC综合征的致病原因,此突变为新生突变.

  19. The trochlear cleft: the ''black line'' of the trochlear trough

    International Nuclear Information System (INIS)

    The ''cartilage black line sign'' is a recently described T2 dark cartilage lesion that we have identified appearing as a cleft in the trochlear trough. The purpose of our study was to define the MR imaging characteristics of a trochlear cleft, determine its incidence, and correlate the MR findings with arthroscopy. A total of 1,300 consecutive MR examinations of the knee were retrospectively reviewed by consensus of two fellowship-trained musculoskeletal radiologists. The MR imaging characteristics and location of a trochlear cleft were determined. Imaging results were compared to arthroscopy when available. Patient age and gender were compared to 25 randomly selected control patients without trochlear clefts. A total of 25 (1.9%) individuals (11 females and 14 males; age range 19-45 years; mean age 28 years) were diagnosed with a trochlear cleft. The control group consisted of 11 females and 14 males; age range 19-83 years; mean age 46 years. Mean cleft length was 7 mm (range 6-12 mm); cleft location was consistently in the lower trochlear trough. No full-thickness cartilage defects were identified in the eight individuals in whom arthroscopic correlation was available. A grade 2 cartilage lesion was identified in a single individual; another progressed from grade 0 to a full-thickness trochlear lesion over an 8-month interval. Eight individuals were athletes. No significant difference in gender was noted between the two groups, however, the study group was significantly younger p < 0.0001. A trochlear cleft is a rare finding in young active individuals. It most likely indicates an incomplete cartilage fissure which may rarely progress to a full-thickness defect. (orig.)

  20. Pattern of disocclusion in patients with complete cleft lip and palate Padrão de desoclusão em indivíduos com fissura lábio-palatina completa

    Directory of Open Access Journals (Sweden)

    Daniella Andaluza Dias Matos

    2006-06-01

    Full Text Available OBJECTIVE: to analyze the pattern of disocclusion during excursive mandibular movements and presence or absence of occlusal interferences and occlusal pathologies (gingival recession and abfraction. METHOD: examination of 120 individuals divided into two groups, as follows: Group 1 - 90 patients with complete cleft lip and palate (study group, subdivided into 30 patients with complete left unilateral cleft lip and palate, 30 patients with complete right unilateral cleft lip and palate and 30 patients with complete bilateral cleft lip and palate; Group 2 - 30 individuals without clefts (control group. RESULTS: 58.8% of patients in Group 1 presented unilateral or bilateral canine guidance, 26.6% presented unilateral or bilateral group function and 54.4% presented lateral movements through the posterior teeth. Regarding protrusive movements, 80% presented anterior guidance and 20% presented posterior guidance. In Group 2, 69.6% of individuals presented unilateral or bilateral canine guidance, 43.2% presented unilateral or bilateral group function and only 13.3% presented lateral movements through the posterior teeth; 3.4% presented protrusion through the posterior teeth. CONCLUSIONS: there was no difference in the pattern of disocclusion between subgroups of patients with clefts. Group 2 presented predominance of bilateral group function, whereas Group 1 presented a higher prevalence of posterior guidance during lateral movements. Protrusion occurred primarily through anterior guidance in Group 2 and through the posterior teeth in Group 1. There was high prevalence of occlusal interferences at the molar area for both groups, yet with no correlation with occlusal pathologies (recession and abfraction.OBJETIVO: avaliar o padrão de desoclusão apresentado nos movimentos excursivos mandibulares e a presença ou não de interferências oclusais e patologias relacionadas à oclusão (recessão gengival e abfração. MÉTODO: exame de 120 indiv

  1. A preliminary study of psychological intervention in patients with cleft lip and palate%唇腭裂患者心理干预影响的初步研究

    Institute of Scientific and Technical Information of China (English)

    王歆; 陈仁吉; 穆月; 杨增杰

    2011-01-01

    Objective To investigate the effects of psychological intervention on adult patients with cleft lip and palate. Methods Adult patients with cleft lip and palate were divided into two groups: control group ( 17 ) and experimental group (17 ). The control group only underwent surgery, and the experimental group received psychological intervention before and after operation during hospitalization. The patients received psychological test before and after psychological intervention to evaluate the effects of psychological intervention on adult clefts patients. Testing tool included interaction anxiousness scale,self-esteem scale and the social support scale. Results There was no significant difference in these parameters between experimental and the control group before intervention , but the scores of social support in experimental group were higher than those of the control group after psychological intervention ( P < 0. 05 ). Conclusion The level of social support significantly improved after psychological intervention for adult patients with clefts.%目的 探讨心理干预对成人唇腭裂患者心理状况的影响.方法 对34例住院成人唇腭裂患者按照随机分组原则分为对照组和干预组.对照组在住院期间仅进行手术治疗,干预组在进行手术治疗的同时接受心理干预.人院后、出院前分别对两组患者进行测评,以评价心理干预对成人唇腭裂患者心理状况的影响.施测工具为交往焦虑量表、自尊量表及社会支持量表.结果 干预组、对照组患者干预前各项指标间无明显差异.心理干预组干预前后社会支持总分差值显著高于对照组(P<0.05),其余各因子得分差值无显著差异.结论 心理干预后成年唇腭裂患者的社会支持感明显提高.该结果为进一步探讨心理干预对成人唇腭裂患者心理状况的影响提供一定的参考依据.

  2. Genetic determinants of facial clefting

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2009-01-01

    BACKGROUND: Facial clefts are common birth defects with a strong genetic component. To identify fetal genetic risk factors for clefting, 1536 SNPs in 357 candidate genes were genotyped in two population-based samples from Scandinavia (Norway: 562 case-parent and 592 control-parent triads; Denmark...

  3. 正畸结合上颌前部牵引成骨矫治严重骨性畸形唇腭裂患者%Treatment of maxillary deficiency with orthodontics and maxillary distraction osteogenesis in cleft patient

    Institute of Scientific and Technical Information of China (English)

    贾绮林; 伊彪; 李自力

    2011-01-01

    Objective To treat cleft patient with maxillary deficiency by means of orthodontics and maxillary distraction osteogenesis. Method Maxillary distraction osteogenesis was performed to increase bone mass and orthodontic treatment was carried out after surgery. Results The cleft patient with maxillary deficiency was treated successfully, ANB was increased by 9° and good occlusal relationship was achieved. Conclusions The cleft patient with maxillary deficiency could be treated successfully with maxillary distraction osteogenesis and orthodontics.%目的 探讨采用正畸结合部分上颌骨牵引成骨的方法,治疗上颌骨严重发育不足的唇腭裂患者.方法 使用正畸结合上颌骨部分牵引成骨的方法,首先进行上颌骨前部牵引成骨,增加上颌骨的骨量,再进行正畸治疗排齐牙齿,最终解决上颌骨发育不足和牙齿拥挤的问题.结果 患者矫治后ANB角增加9°,面型明显改善,(牙合)关系良好.结论 采用正畸结合上颌骨部分牵引成骨这一新技术,矫治上颌骨发育严重不足的唇腭裂患者能够获得良好的治疗效果.

  4. The impact of cleft lip and palate repair on maxillofacial growth

    Institute of Scientific and Technical Information of China (English)

    Bing Shi; Joseph E Losee

    2015-01-01

    Surgical correction is central to current team-approached cleft treatment. Cleft surgeons are always concerned about the impact of their surgical maneuver on the growth of the maxilla. Hypoplastic maxilla, concaved mid-face and deformed dental arch have constantly been reported after cleft treatments. It is very hard to completely circumvent these postoperative complications by current surgical protocols. In this paper, we discussed the factors that inhibit the maxillofacial growth on cleft patients. These factors included pre-surgical intervention, the timing of cleft palate and alveolae repair, surgical design and treatment protocol. Also, we made a review about the influence on the maxillary growth in un-operated cleft patients. On the basis of previous researches, we can conclude that most of scholars express identity of views in these aspects: early palatoplasty lead to maxilla growth inhibition in all dimensions; secondary alveolar bone graft had no influence on maxilla sagittal growth; cleft lip repair inhibited maxilla sagittal length in patients with cleft lip and palate;Veau’s pushback palatoplasty and Langenbeck’s palatoplasty with relaxing incisions were most detrimental to growth; Furlow palatoplasty showed little detrimental effect on maxilla growth;timing of hard palate closure, instead of the sequence of hard or soft palate repair, determined the postoperative growth. Still, scholars hold controversial viewpoints in some issues, for example, un-operated clefts have normal growth potential or not, pre-surgical intervention and pharyngoplasty inhibited maxillofacial growth or not.

  5. Cleft Crossing Medial Mucosal Flap for Deepening of the Gingivolabial Sulcus in a Complete Cleft Lip Repair.

    Science.gov (United States)

    Park, Jong Lim; Hwang, Kun

    2015-06-01

    The aim of the present study is to introduce a method of which the medial mucosal flap is used in a deepening of the gingivolabial sulcus in a complete cleft lip repair. An incision was made on the lateral vestibular lining and the contracture was released. The defect produced was filled by transposing the lateral mucosal flap (l-flap). The transposed l-flap was sutured except for the proximal part of the lateral side. The medial mucosal flap (m-flap) was elevated with its base above on the alveolus. The m-flap was turned over and crossed over the cleft thereafter and the distal end of the m-flap was sutured to the proximal part of the lateral side of the l-flap. The width of the m-flap was 4 to 5 mm, and the length depended on the width of the cleft. The raw surface of the m-flap faced the outer side and was covered with the repaired muscle layer. Thereafter, the m-flap became a part of the deepened gingivolabial sulcus. A total of 12 patients (8 men, 4 women) with a unilateral complete cleft lip (left 9, right 3) were operated on using this technique and at least 2 mm deepening effect of upper gingivolabial sulcus was observed in 8 patients followed up. The authors think this cleft crossing medial mucosal flap technique may be of use in the primary repair of a unilateral cleft lip. PMID:26080198

  6. Patrones de cierre velofaringeo: Estudio comparativo entre población sana y pacientes con paladar hendido Patterns of velopharyngeal closure: comparative study between healthy population and patients with cleft palate

    Directory of Open Access Journals (Sweden)

    J.R. Prada Madrid

    2010-12-01

    its frequency of appearance in a non-probabilistic sample with healthy volunteers and to compare these findings with the ones obtained in patients with surgically repaired cleft lip and palate. We designed a descriptive study of cross section. The evaluations were made in a fourth level hospital and in the Rehabilitation Centre for Children with Cleft lip and Palate (FISULAB, Santa fe de Bogotá (Colombia. One hundred healthy volunteers (university students and 82 patients with surgically repaired cleft palate participated in the study. Videonasopharyngoscopy was performed to determine patterns of velopharyngeal closure present and their frequency of appearance. The statistical analysis indicate that there is no difference in the frequency of presentation of the patterns of velopharyngeal closure between healthy volunteers and the patients with cleft palate. In the healthy group, the most frequently found pattern was the circular (56% of the sample, followed by the coronal (29 % and circular with Passavant´s ridge (15 %. In the group of patients with cleft lip and palate, the circular pattern also prevails (39,02 %, followed by the coronal pattern (24,39 %, and the circular pattern with Passavant´s ridge (19,51 %. In neither group was found the sagittal pattern. As a conclusion, there was not any statistical difference in the frequency of presentation of the patterns of velopharyngeal closure between the healthy volunteers and the patients with cleft palate.

  7. A review of cleft lip and palate management: Experience of a Nigerian Teaching Hospital

    Science.gov (United States)

    Efunkoya, Akinwale Adeyemi; Omeje, Kelvin Uchenna; Amole, Ibiyinka Olushola; Osunde, Otasowie Daniel; Akpasa, Izegboya Olohitae

    2015-01-01

    Background: Cleft lip (CL) and palate (CLP) management is multidisciplinary. A cleft team was formed in a Nigerian Tertiary Hospital to address the health needs of cleft patients in the centre. Aim: This paper aims at documenting the Aminu Kano Teaching Hospital (AKTH) management protocol for orofacial clefts and also to review our experience with CLP surgeries performed at AKTH since our partnering with Smile Train. Materials and Methods: A retrospective review of all the cleft patients surgically treated from January 2006 to December 2014 under Smile Train sponsorship was undertaken. A descriptive narrative of the cleft team protocol was also given. Results: One hundred and fifty-five patients (80 males, 75 females) had surgical repairs of either the lip or palate. CL patients were 83 (53.55%), while CLP patients were 45 (29.03%) and isolated cleft palate patients were 27 (17.42%). Conclusion: The inclusion of various specialities in the cleft team is highly desirable. Poverty level amongst our patients frequently limits our management to surgical treatment sponsored by the Smile Train, despite the presence of other residual problems. PMID:26712291

  8. Molecular basis of cleft palates in mice

    Institute of Scientific and Technical Information of China (English)

    Noriko; Funato; Masataka; Nakamura; Hiromi; Yanagisawa

    2015-01-01

    Cleft palate, including complete or incomplete cleft palates, soft palate clefts, and submucosal cleft palates, is the most frequent congenital craniofacial anomaly in humans. Multifactorial conditions, including genetic and environmental factors, induce the formation of cleft palates. The process of palatogenesis is temporospatially regulated by transcription factors, growth factors, extracellular matrix proteins, and membranous molecules; a single ablation of these molecules can result in a cleft palate in vivo. Studies on knockout mice were reviewed in order to identify genetic errors that lead to cleft palates. In this review, we systematically describe these mutant mice and discuss the molecular mechanisms of palatogenesis.

  9. 大龄腭裂患者咽后壁组织瓣咽成形术后语音特征%Articulatory characteristics of cleft palate patients over eight years old after pharyngoplasty with posterior pharyngeal flap

    Institute of Scientific and Technical Information of China (English)

    秦瑞峰; 胡开进; 封兴华; 吴炜

    2005-01-01

    surgery for these patients to improve palate form and articulatory function. OBJECTIVE: To study the characteristics of the cleft palate in patients of over 8 years old and the effects of palate repair and posterior pharyngeal flap transplantation (PPFT) on articulation. DESIGN:A controlled study with children patients as subjects. SETTING: Department of Maxillofacial Surgery, Medical College of Stomatology, Fourth Military Medical University of Chinese PLA PARTICIPANTS: Twenty-four children patients of cleft palate who were treated in the Department of Maxillofacial Surgery ,Medical College of Stomatology, Fourth Military Medical University of Chinese PLA from January 2000 through December 2003 were enrolled in the study. Fourteen were male and ten female. Their ages varied from 8 to 20 years old (mean 13.8 years). Three cases were bilateral complete cleft palate and twelve cases were unilateral complete cleft palate. Nine were incomplete cleft palate. METHODS :Two flap palatoplasty and upper pedicle PPFT were conducted in all the patients. In thirty days after surgical treatment, the patients were asked to repeat the words of a doctor in the phonetic lab with his or her lips 10 cm away from microphone. The words of the patients were recorded and evaluated subjectively by 5 doctors specialized in pathologic phones. MAIN OUTCOME MEASURES :The palatopharynx was observed with nasopharyngoscope before and after operation. And the improvement of nasal gas leakage and enhanced rhinophonia were also evaluated. RESULTS: The repaired cleft palate was healed in the first intention in all the patients. The soft palate retreated satisfactorily and the palatopharynx was apparently closed, which created good preconditions for articulation. The patients' articulation was also improved in different degrees. All the 24 patients had the fourth grade of enhanced rhinophonia and nasal gas leakage before repairing. After palate repair and PPFT, the enhanced rhinophonia and nasal gas leakage

  10. Epidemiologic Research on Malformations Associated with Cleft Lip and Cleft Palate in Japan

    Science.gov (United States)

    Koga, Hiroshi; Iida, Koichi; Maeda, Tomoki; Takahashi, Mizuho; Fukushima, Naoki; Goshi, Terufumi

    2016-01-01

    To investigate malformations associated with cleft lip and cleft palate, we conducted surveys at neonatal intensive care units (NICUs) and other non-NICU facilities and to determine whether there are differences among facilities. The regional survey investigated NICU facilities located in Oita Prefecture, including 92 patients with cleft lip and palate (CLP) or cleft palate (CP) that occurred between 2004 and 2013, and the national survey investigated oral surgery, plastic surgery, and obstetrics and gynecology facilities located in Japan, including 16,452 patients with cleft lip (CL), CLP, or CP that occurred since 2000. The incidence per 10,000 births was 4.2, 6.2, and 2.8 for CL, CLP, and CP, respectively, according to the national survey, and 6.3 and 2.9 for CLP and CP, respectively according to the regional survey. These results indicated comparable incidences between the two surveys. In contrast, when the survey results on malformations associated with CLP and CP according to the ICD-10 classification were compared between the national survey conducted at oral surgery or plastic surgery facilities and the regional survey conducted at NICU facilities, the occurrence of associated malformations was 19.8% vs. 41.3% for any types of associated malformation, 6.8% vs. 21.7% for congenital heart disease, and 0.5% vs. 16.3% for chromosomal abnormalities. These results indicated that the incidences of all of these associated malformations were significantly greater in the survey conducted at NICU facilities and similar to the findings from international epidemiological surveys. When comparing the survey conducted at obstetrics facilities vs. NICU facilities, the occurrence of associated malformations was similar results as above. The incidence of CLP and CP was not different between surveys conducted at NICU facilities vs. non-NICU facilities; however, when conducting surveys on associated malformations, it is possible to obtain accurate epidemiological data by

  11. Segment distraction to reduce a wide alveolar cleft before alveolar bone grafting.

    NARCIS (Netherlands)

    Binger, T.; Katsaros, C.; Rucker, M.; Spitzer, W.J.

    2003-01-01

    OBJECTIVE: To demonstrate a method for reduction of wide alveolar clefts prior to bone grafting. This method aims to facilitate bone grafting and achieve adequate soft tissue coverage of the graft with attached gingiva. CASE REPORT: Treatment of a patient with bilateral cleft lip and palate with a s

  12. Surgical treatment of cleft lip

    OpenAIRE

    Mateus Domingues Miachon; Pedro Luiz Squilacci Leme

    2014-01-01

    We performed a systematic review of the literature on the surgical treatment of cleft lip, emphasizing the prevalence, complications associated with the treatment and the points of disagreement between authors. We conducted a literature cross-sectional search that analyzed publications in books, articles and on the databases SciELO - Scientific Electronic Library Online, PubMed, of the National Center for Biotechnology Information. We conclude that: 1) the severity of the cleft will indicate ...

  13. Bilateral cleft lip nasal deformity

    OpenAIRE

    Singh Arun; Nandini R.

    2009-01-01

    Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the li...

  14. Current Controversies in Diagnosis and Management of Cleft Palate and Velopharyngeal Insufficiency

    Directory of Open Access Journals (Sweden)

    Pablo Antonio Ysunza

    2015-01-01

    Full Text Available Background. One of the most controversial topics concerning cleft palate is the diagnosis and treatment of velopharyngeal insufficiency (VPI. Objective. This paper reviews current genetic aspects of cleft palate, imaging diagnosis of VPI, the planning of operations for restoring velopharyngeal function during speech, and strategies for speech pathology treatment of articulation disorders in patients with cleft palate. Materials and Methods. An updated review of the scientific literature concerning genetic aspects of cleft palate was carried out. Current strategies for assessing and treating articulation disorders associated with cleft palate were analyzed. Imaging procedures for assessing velopharyngeal closure during speech were reviewed, including a recent method for performing intraoperative videonasopharyngoscopy. Results. Conclusions from the analysis of genetic aspects of syndromic and nonsyndromic cleft palate and their use in its diagnosis and management are presented. Strategies for classifying and treating articulation disorders in patients with cleft palate are presented. Preliminary results of the use of multiplanar videofluoroscopy as an outpatient procedure and intraoperative endoscopy for the planning of operations which aimed to correct VPI are presented. Conclusion. This paper presents current aspects of the diagnosis and management of patients with cleft palate and VPI including 3 main aspects: genetics and genomics, speech pathology and imaging diagnosis, and surgical management.

  15. Expression analyses of human cleft palate tissue suggest a role for osteopontin and immune related factors in palatal development

    DEFF Research Database (Denmark)

    Jakobsen, L.P.; Borup, R.; Vestergaard, J.;

    2009-01-01

    Cleft lip and/or palate (CL/P) is a common congenital malformation with a complex etiology which is not fully elucidated yet. Epidemiological studies point to different etiologies in the cleft lip and palate subgroups, isolated cleft lip (CL), isolated cleft palate (CP) and combined cleft lip...... and palate (CLP). In order to understand the biological basis in these cleft lip and palate subgroups better we studied the expression profiles in human tissue from patients with CL/P. In each of the CL/P subgroups, samples were obtained from three patients and gene expression analysis was performed....... Moreover, selected differentially expressed genes were analyzed by quantitative RT-PCR, and by immunohistochemical staining of craniofacial tissue from human embryos. Osteopontin (SPP1) and other immune related genes were significantly higher expressed in palate tissue from patients with CLP compared to CP...

  16. How to make young children produce cleft sentences.

    Science.gov (United States)

    Hupet, M; Tilmant, B

    1989-06-01

    The present study focuses on the effects of contextual demands on the selection of a particular syntactic device, in asking whether French-speaking children from 4 to 10 years old will spontaneously produce it-cleft sentences if there is a functional necessity arising from the context. Taking into account recent studies that have specified the discourse function(s) served by this marked sentence form, it was hypothesized that the cleft formulation would be more likely than its uncleft counterpart whenever the child's intention was to contrast their own belief or knowledge with that of their addressee. The study showed this to be the case when the matter of the disagreement concerned the agent of an action: that situation elicited an overwhelming majority (from 80% to 97%) of cleft constructions, even from the youngest children. On the other hand, when the matter of the disagreement concerned the patient, there were only a few cleft constructions, even with the oldest children; contrastive stress on the object constituent was the predominant device employed for marking information in that situation. The high proportion of clefts in the Agent condition, and the high proportion of stressed object constituents in the Patient condition, both differ from previously reported data. These differences are discussed with reference to differences in task requirements and to differences between the prosodic constraints of French and English. PMID:2760126

  17. Early orthodontic intervention followed by fixed appliance therapy in a patient with a severe Class III malocclusion and cleft lip and palate.

    Science.gov (United States)

    Zhang, He; Deng, Feng; Wang, Huaqiao; Huang, Qianqian; Zhang, Yi

    2013-11-01

    This case report describes the treatment of a girl, age 11 years 10 months, with a cleft lip and palate and a postsurgical scar. The clinical examination showed a concave profile, a retrusive maxilla, an asymmetric face, severe dental crowding, a Class III dental relationship, and a complete dental crossbite. Maxillary expansion and distraction, chincap, and high-pull headgear were used to moderate the skeletal discrepancy. These approaches, combined with tooth extraction and fixed orthodontic appliances, finally established a functional and esthetic occlusal relationship, normal overjet and overbite, and a well-balanced facial appearance.

  18. Risk of Oral Clefts in Twins

    DEFF Research Database (Denmark)

    Grosen, Dorthe; Bille, Camilla; Petersen, Inge;

    2011-01-01

    BACKGROUND:: Small studies have indicated that twinning increases the risk of oral cleft. METHODS:: We used data from a Danish national population-based cohort study to investigate whether twinning was associated with isolated oral cleft, and to estimate the twin probandwise concordance rate...... and heritability. Twins (207 affected/130,710) and singletons (7766 affected/4,798,526) born from 1936 through 2004 in Denmark were ascertained by linkage among the Danish Facial Cleft Database, the Danish Twin Registry, and the Civil Registration System. We computed oral cleft prevalence and prevalence proportion...... ratio for twins versus singletons, stratified for 3 subphenotypes. Probandwise concordance rates and heritability for twins were estimated for 2 phenotypes-cleft lip with or without cleft palate (CL/P) and cleft palate (CP). RESULTS:: The prevalence of oral cleft was 15.8 per 10,000 twins and 16.6 per...

  19. Surgical outcome and complications following cleft lip and palate repair in a teaching hospital in Nigeria

    Directory of Open Access Journals (Sweden)

    Taiwo O Abdurrazaq

    2013-01-01

    Full Text Available Background: Measurement of treatment outcome is important in estimating the success of cleft management. The aim of this study was to assess the surgical outcome of cleft lip and palate surgery. Patients and Methods: The surgical outcome of 131 consecutive patients with cleft lip and palate surgeries between October 2008 and December 2010 were prospectively evaluated at least 4 weeks postoperatively. Data collected included information about the age, sex, type of cleft defects, and type of surgery performed as well as postoperative complications. For cleft lip repair, the Pennsylvania lip and nose (PLAN score was used to assess the surgical outcome, while the integrity of the closure was used for cleft palate repair. Results: A total of 92 patients had cleft lip repair and 64 had palate repair. Overall, 68.8% cases of cleft lip and palate repair had good outcomes; 67.9% of lip repairs had good lip and nose scores, while 70.2% of palatal repair had a good surgical outcome. Oro-fistula was observed in 29.8% of cleft palate repairs Inter-rater reliability coefficient was substantially significant. Conclusions: The fact that 25.7% of those treated were aged >1 year suggests a continued need to enlighten the public on the availability of cleft lip and palate expertise and treatment. Although an overall good treatment outcome was demonstrated in this study, the nasal score was poorer than the lip score. Complication rate of about 14% following surgical repair is consistent with previous reports in the literature.

  20. Tissue-plastinated vs. celloidin-embedded large serial sections in video, analog and digital photographic on-screen reproduction: a preliminary step to exact virtual 3D modelling, exemplified in the normal midface and cleft-lip and palate

    Science.gov (United States)

    Landes, Constantin A; Weichert, Frank; Geis, Philipp; Wernstedt, Katrin; Wilde, Anja; Fritsch, Helga; Wagner, Mathias

    2005-01-01

    This study analyses tissue-plastinated vs. celloidin-embedded large serial sections, their inherent artefacts and aptitude with common video, analog or digital photographic on-screen reproduction. Subsequent virtual 3D microanatomical reconstruction will increase our knowledge of normal and pathological microanatomy for cleft-lip-palate (clp) reconstructive surgery. Of 18 fetal (six clp, 12 control) specimens, six randomized specimens (two clp) were BiodurE12-plastinated, sawn, burnished 90 µm thick transversely (five) or frontally (one), stained with azureII/methylene blue, and counterstained with basic-fuchsin (TP-AMF). Twelve remaining specimens (four clp) were celloidin-embedded, microtome-sectioned 75 µm thick transversely (ten) or frontally (two), and stained with haematoxylin–eosin (CE-HE). Computed-planimetry gauged artefacts, structure differentiation was compared with light microscopy on video, analog and digital photography. Total artefact was 0.9% (TP-AMF) and 2.1% (CE-HE); TP-AMF showed higher colour contrast, gamut and luminance, and CE-HE more red contrast, saturation and hue (P < 0.4). All (100%) structures of interest were light microscopically discerned, 83% on video, 76% on analog photography and 98% in digital photography. Computed image analysis assessed the greatest colour contrast, gamut, luminance and saturation on video; the most detailed, colour-balanced and sharpest images were obatined with digital photography (P < 0.02). TP-AMF retained spatial oversight, covered the entire area of interest and should be combined in different specimens with CE-HE which enables more refined muscle fibre reproduction. Digital photography is preferred for on-screen analysis. PMID:16050904

  1. Anterior Segmental Distraction Osteogenesis in the Hypoplastic Cleft Maxilla: Report of five cases

    OpenAIRE

    Sruthi Rao (Janardhan); Kotrashetti, S. M.; J. B. Lingaraj; Pinto, P. X.; Keluskar, K. M.; Siddharth Jain; Piyush Sone; Santhosh Rao

    2013-01-01

    Orthognathic surgery and distraction osteogenesis play a prime role in the correction of maxillary hypoplasia in patients with cleft lip and palate (CLP). Advancement of the anterior maxilla alone without interfering with the velopharyngeal sphincter may be advantageous in cleft patients, who more commonly have speech deficits and dental crowding. We present a case series of anterior maxillary segmental distraction for maxillary hypoplasia in 5 CLP patients with a one-year follow-up. A custom...

  2. 8~12岁唇腭裂患者的心理调查研究%Psychological research patients of 8-12 years with cleft of lip and/or palate*

    Institute of Scientific and Technical Information of China (English)

    郭世莉; 陈群

    2013-01-01

      目的探讨学龄期唇腭裂患者的心理状态和行为习惯。方法选取8~12岁一期手术前的唇腭裂患者及其家长50例作为观察组;选取8~12岁非唇腭裂患者及家长50例作为对照组,分别对其进行儿童社交焦虑量表、自尊量表、Achenbach儿童行为量表测定。结果家长用Achenbach儿童行为量表唇腭裂患者异常率为22%,高于对照组的8%(χ2=3.85,P<0.05);学龄期唇腭裂患者社交焦虑量表得分高于非唇腭裂患者( t=5.29,P<0.01);学龄期唇腭裂患者自尊量表得分低于非唇腭裂患者( t=4.25,P<0.01)。结论学龄期唇腭裂患者存在较为明显的心理和行为问题,应有针对性地采取相应的护理措施及健康教育。%Objective To investigate the pyschological states and behavior habits of school-age children with cleft of lip and /or palate .Methods 50 children at the age of 8-12 with cleft of lip before the first operation and their parents and 50 normal control children were tested with SAS (social anxiety scale) ,SEC(self esteem scale) ,and CBCL(children behavior checklist) .Results The rate of abnormal tested by CBCL (children behavior checklist)-parent in patients was 22% ,was higher than that in normal 8% (χ2 =3 .85 ,P<0 .05) .The scores of SAS in patients was higher than those in control (t=5 .29 ,P<0 .01) .Conclusion School-age chil-dren with cleft of lip and/or palate had obvious psychological and behavioral problems ,thus ,corresponding nursing and health edu-cation should be applied .

  3. Clinical photography among African cleft caregivers

    Directory of Open Access Journals (Sweden)

    Peter Babatunde Olaitan

    2011-01-01

    Full Text Available Objective: The aim of this paper is to document the practice of photography among clinicians whose daily work depends and is influenced so much by medical photography. Materials and Methods: Questionnaires documenting the bio data, place of practice, and experience of cleft caregivers with clinical photography were distributed. Knowledge of rules guiding clinical photography and adherence to them were also asked. Types of camera used were documented and knowledge of the value of clinical photographs were also inquired. Results: Plastic surgeons constitute the highest proportion of 27 (38.6%, followed by Oral and Maxillofacial surgeons with 14 (20.0%. Twenty one (30.0% of the respondents always, 21 (30.0% often, 12 (17.1% frequently, while 9 respondents sometimes took photographs of their patients. Suggested uses of clinical photographs included training, 52 (74.3%, education, 51 (72.9%, medicolegal, 44 (62.9% and advertisement, 44 (62.9% among others. Twenty two (31.4% did not know that there were standard guidelines for taking clinical photographs. Twenty three (32.9% of them did not seek the consent of the patients before taking clinical photographs. Conclusion: While the practice of clinical photography is high among African cleft caregivers, there is a need for further education on the issues of standard rules and obtaining consent from patients.

  4. Clinical photography among African cleft caregivers

    Science.gov (United States)

    Olaitan, Peter Babatunde; Oseni, Ganiyu Oladiran

    2011-01-01

    Objective: The aim of this paper is to document the practice of photography among clinicians whose daily work depends and is influenced so much by medical photography. Materials and Methods: Questionnaires documenting the bio data, place of practice, and experience of cleft caregivers with clinical photography were distributed. Knowledge of rules guiding clinical photography and adherence to them were also asked. Types of camera used were documented and knowledge of the value of clinical photographs were also inquired. Results: Plastic surgeons constitute the highest proportion of 27 (38.6%), followed by Oral and Maxillofacial surgeons with 14 (20.0%). Twenty one (30.0%) of the respondents always, 21 (30.0%) often, 12 (17.1%) frequently, while 9 respondents sometimes took photographs of their patients. Suggested uses of clinical photographs included training, 52 (74.3%), education, 51 (72.9%), medicolegal, 44 (62.9%) and advertisement, 44 (62.9%) among others. Twenty two (31.4%) did not know that there were standard guidelines for taking clinical photographs. Twenty three (32.9%) of them did not seek the consent of the patients before taking clinical photographs. Conclusion: While the practice of clinical photography is high among African cleft caregivers, there is a need for further education on the issues of standard rules and obtaining consent from patients. PMID:22279284

  5. Three-Dimensional Assessment of Early Surgical Outcome in Repaired Unilateral Cleft Lip and Palate: Part 2. Lip Changes.

    LENUS (Irish Health Repository)

    Ayoub, Ashraf

    2010-09-08

    Abstract Objective: To evaluate 3D lip morphology, following primary reconstruction in children with unilateral cleft lip and palate (UCLP) relative to contemporaneous non-cleft data Design: Prospective, cross-sectional, controlled study Setting: Glasgow Dental Hospital & School, University of Glasgow, The UK. Patients and Participants: Two groups of 3-year old children (21 with unilateral cleft lip and palate and 96 controls) with facial images taken using a 3D vision based capture technique. Methods: 3D images of the face were reflected so the cleft was on the left side to create a homogenous group for statistical analysis. Three-dimensional co-ordinates of anthropometric landmarks were extracted from facial images. 3D generalised Procustes superimposition was employed and a set of linear measurements were utilised to compare between cleft and control subjects for right and left sides, adjusting for sex differences. Results: Christa philteri on both the cleft and non-cleft sides were displaced laterally and posteriorly; there was also a statistically significant increase in philtrum width. No significant differences between cleft and control regarding the cutaneous height of the upper lip. The lip in the cleft cases was flatter than in the non-cleft cases with less prominence of labialis superioris. Conclusions: Stereophotogrammetry allows detection of residual dysmorphology following cleft repair. There was significant increase of the philtrum width. The lip appeared flatter and more posterior displaced in Unilateral Cleft Lip and Palate (UCLP) cases compared with control. Keywords: child, cleft lip and palate, lip repair, three-dimensional imaging.

  6. Cleft lip: The historical perspective

    Directory of Open Access Journals (Sweden)

    Bhattacharya S

    2009-10-01

    Full Text Available The earliest documented history of cleft lip is based on a combination of religion, superstition, invention and charlatanism. While Greeks ignored their existence, Spartans and Romans would kill these children as they were considered to harbour evil spirits. When saner senses prevailed Fabricius ab Aquapendente (1537-1619 was the first to suggest the embryological basis of these clefts. The knowledge of cleft lip and the surgical correction received a big boost during the period between the Renaissance and the 19th century with the publication of Pierre Franco′s Petit Traite and Traite des Hernies in which he described the condition as "lievre fendu de nativite" (cleft lip present from birth. The first documented Cleft lip surgery is from China in 390 BC in an 18 year old would be soldier, Wey Young-Chi. Albucasis of Arabia and his fellow surgeons used the cautery instead of the scalpel and Yperman in 1854 recommended scarifying the margins with a scalpel before suturing them with a triangular needle dipped in wax. The repair was reinforced by passing a long needle through the two sides of the lip and fixing the shaft of the needle with a figure-of-eight thread over the lip. Germanicus Mirault can be credited to be the originator of the triangular flap which was later modified by C.W. Tennison in 1952 and Peter Randall in 1959. In the late 50s, Ralph Millard gave us his legendary ′cut as you go′ technique. The protruding premaxilla of a bilateral cleft lip too has seen many changes throughout the ages - from being discarded totally to being pushed back by wedge resection of vomer to finally being left to the orthodontists.

  7. Surgical treatment of cleft lip

    Directory of Open Access Journals (Sweden)

    Mateus Domingues Miachon

    2014-06-01

    Full Text Available We performed a systematic review of the literature on the surgical treatment of cleft lip, emphasizing the prevalence, complications associated with the treatment and the points of disagreement between authors. We conducted a literature cross-sectional search that analyzed publications in books, articles and on the databases SciELO - Scientific Electronic Library Online, PubMed, of the National Center for Biotechnology Information. We conclude that: 1 the severity of the cleft will indicate the technique presenting more advantages; 2 the different approaches indicate that there is no consensus on the optimal technique; and 3 the surgeon experience contributes to choosing the best option.

  8. Oral cleft prevention program (OCPP

    Directory of Open Access Journals (Sweden)

    Wehby George L

    2012-11-01

    Full Text Available Abstract Background Oral clefts are one of the most common birth defects with significant medical, psychosocial, and economic ramifications. Oral clefts have a complex etiology with genetic and environmental risk factors. There are suggestive results for decreased risks of cleft occurrence and recurrence with folic acid supplements taken at preconception and during pregnancy with a stronger evidence for higher than lower doses in preventing recurrence. Yet previous studies have suffered from considerable design limitations particularly non-randomization into treatment. There is also well-documented effectiveness for folic acid in preventing neural tube defect occurrence at 0.4 mg and recurrence with 4 mg. Given the substantial burden of clefting on the individual and the family and the supportive data for the effectiveness of folic acid supplementation as well as its low cost, a randomized clinical trial of the effectiveness of high versus low dose folic acid for prevention of cleft recurrence is warranted. Methods/design This study will assess the effect of 4 mg and 0.4 mg doses of folic acid, taken on a daily basis during preconception and up to 3 months of pregnancy by women who are at risk of having a child with nonsyndromic cleft lip with/without palate (NSCL/P, on the recurrence of NSCL/P. The total sample will include about 6,000 women (that either have NSCL/P or that have at least one child with NSCL/P randomly assigned to the 4 mg and the 0.4 mg folic acid study groups. The study will also compare the recurrence rates of NSCL/P in the total sample of subjects, as well as the two study groups (4mg, 0.4 mg to that of a historical control group. The study has been approved by IRBs (ethics committees of all involved sites. Results will be disseminated through publications and presentations at scientific meetings. Discussion The costs related to oral clefts are high, including long term psychological and socio-economic effects. This study

  9. The Effect of Cleft Palate Repair on Contractile Properties of Single Permeabilized Muscle Fibers From Congenitally Cleft Goats Palates

    Science.gov (United States)

    A cleft palate goat model was used to study the contractile properties of the levator veli palatini (LVP) muscle which is responsible for the movement of the soft palate. In 15-25% of patients that undergo palatoplasty, residual velopharyngeal insufficiency (VPI) remains a problem and often require...

  10. Secondary alveolar bone grafting in combination with autologous iliac crest cortical plate and concellous bone for 19 adult cleft patients%自体髂骨皮质骨块与松质骨联合移植修复成人牙槽突裂19例分析

    Institute of Scientific and Technical Information of China (English)

    李增健; 卢利; 金山; 任媛媛; 刘强; 张斌; 张蕾; 吴楠; 杨鸣良; 王绪凯

    2012-01-01

    Objective To retrospectively study secondary alveolar bone grafting in combination with autologous iliac crest cortical bone and concellous bone for adult cleft patients. Methods Analveolar bone grafting procedure in combination with autologous illiac crest cortical bone and concellous bone has been applied in 19 adult cleft patients since 2008. A water-tight mucocusperiosteum pocket was formed around the alveolar cleft. The iliac concellous bone was put into the alveolar cleft with tender pressing. The carved iliac crest cortical bone was then covered on to the labial surface of alveolar cleft and pyriform aperture with titanium screws. Results Based on Bergland's criterion, the overall survival rate of bone grafting was 84.2% , clinical success rate was 73.7% , clinical failure rate was 26. 3%. Oral-nasal fistular recurred in 2 patients. Conclusion Secondary alveolar bone grafting in combination with autologous iliac crest cortical bone and concellous bone can effectively improve the grafting survival rate and keep a sufficient volume in regions of cleft and the pyriform aperture of adult cleft patients.%目的 观察自体髂骨块和松质骨联合移植修复成人牙槽突裂的效果.方法 对2008年以来在我院就诊的成人牙槽突裂患者19例采用自体髂骨块和松质骨联合移植修复.将髂松质骨填充牙槽突裂间隙内,用骨块恢复梨状孔边缘形态和鼻翼基底部高度后,采用微型钛板和单皮质钛钉固定.术后1年复查进行Bergland 分级评价.结果 植入骨成活率(Ⅰ~Ⅲ级)为84.2%,临床成功率(Ⅰ、Ⅱ级)为73.7%,临床失败率(Ⅲ、Ⅳ级)为26.3%.2例患者再次出现口鼻瘘孔.结论 自体髂骨块和松质骨联合移植修复成人牙槽突裂可以有效恢复牙槽突高度,增加患侧梨状孔周围骨量.

  11. Bone grafting, corticotomy, and orthodontics: treatment of cleft alveolus in a chinese cohort.

    Science.gov (United States)

    Mao, Li-Xia; Shen, Guo-Fang; Fang, Bing; Xia, Yun-Hui; Ma, Xu-Hui; Wang, Bo

    2013-11-01

    Objective : A multimodal therapy was applied to solve a set of related problems including collapse of the posterior segment, high level gingival margin of canine, and resorption of grafted bone in a cohort of Chinese youngsters with cleft lip and palate. This study aimed to evaluate the benefits of this treatment procedure. Methods : Thirty patients with unilateral cleft lip and palate were included in this prospective study. All patients had previously undergone only cleft lip and palate repair and presented with alveolar cleft and an obvious step in the gingival margin between the canine tooth and the teeth beside it. A multimodal therapy that included bone grafting, corticotomy, and orthodontics was applied to solve these problems. Grafted bone volume, parallelism of the roots, root resorption, gingival margin, and mobility of the canine on the cleft side were established before surgery, 1 week after surgery, and after straightening of the canine. Results : Less than 25% of the grafted bone was reabsorbed in 25 of the 30 patients, while less than 50% was resorbed in the remaining five. The roots of the canines on the cleft side were mostly parallel to the adjacent teeth. Root resorption and mobility of the canines were slight. The difference in the gingival margin between the canines on the cleft side and the other side was small. Conclusions : Canines moved into the grafted bone safely and effectively, thus achieving a normal gingival margin and retaining grafted bone volume in one operation. PMID:22849663

  12. Syndromic association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism.

    OpenAIRE

    Buntincx, I M; Van Overmeire, B; Desager, K; Van Hauwaert, J

    1993-01-01

    We describe a patient with the unusual association of cleft palate, bilateral choanal atresia, curly hair, and congenital hypothyroidism. This association has been reported before in two brothers and may represent a new syndrome.

  13. Orthodontic tooth movement into the grafted alveolar bone: a clinical study in 10 cleft lip and palate patients%唇腭裂患者牙槽突裂植骨区牙移入的临床观察

    Institute of Scientific and Technical Information of China (English)

    杨臣杰; 陈振琦; 杨育生; 钱玉芬

    2012-01-01

    PURPOSES: To explore the possibility of tooth movement into grafted alveolar bone in cleft lip and palate patients (CLP), and to evaluate the bone height of the grafted bone and alveolar bone support of the moved-in teeth. METHODS: Ten CLP patients were included in this study. Periapical radiographs were taken for the cleft-associated teeth 3 months later after bone grafting (Tl) and after tooth movement into the grafted bone (T2). Alveolar bone support of the moved teeth were measured at Tl and T2 stages. Paired t test were performed for statistical analysis with SPSS17.0 software package. The height of the grafted bone were evaluated serai -quantitatively using the Bergland system. RESULTS: All the cleft-associated teeth were bodily moved into the grafted area. The average alveolar bone support for the moved teeth was (89.85±2.51)% (Tl) and (90.22±2.44)% (T2), respectively. There was no significant difference between Tl and T2. No significant radiographic alteration was observed in the height of the grafted bone. CONCLUSIONS: Cleft-associated teeth could be bodily moved into the grafted bone. A successful level of alveolar bone support for the moved teeth was achieved. Moved-in teeth could reduce the resorption of grafted bone. Supported by Medical Research Fund of Science and Technology Commission of Shanghai Municipality(074119643).%目的:探讨牙槽突裂植骨区牙移入的可行性及牙移入的方式,评价移入牙的牙槽骨支持率和移植骨高度变化.方法:选取唇腭裂伴牙槽突裂患者10例,行牙槽突裂自体髂骨植骨术后,分别拍摄植骨后3个月(T1)及牙移入植骨区后(T2)的根尖片,观察牙移入植骨区的情况,测量T1和T2阶段移入牙的牙槽骨支持率,采用SPSS17.0软件包对测量数据进行配对t检验,并参照Bergaand四分法评价移植骨的高度变化.结果:①牙整体移入植骨区,牙槽骨支持率为(89.85±2.51)% (T1)和(90.22±2.44)%(T2),牙移入植骨区后的

  14. An evaluation of surgical outcome of bilateral cleft lip surgery using a modified Millard′s (Fork Flap technique

    Directory of Open Access Journals (Sweden)

    W L Adeyemo

    2013-01-01

    Full Text Available Background: The central third of the face is distorted by the bilateral cleft of the lip and palate and restoring the normal facial form is one of the primary goals for the reconstructive surgeons. The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilising the underlying musculature. The aim of this study was to review surgical outcome of bilateral cleft lip surgery (BCLS done at the Lagos University Teaching Hospital. Materials and Methods: A review of all cases of BCLS done between January 2007 and December 2012 at the Lagos University Teaching Hospital was done. Data analysis included age and sex of patients, type of cleft deformity and type of surgery (primary or secondary and whether the cleft deformity was syndromic and non-syndromic. Techniques of repair, surgical outcome and complications were also recorded. Results: A total of 39 cases of BCLS involving 21 males and 18 females were done during the period. This constituted 10% (39/390 of all cases of cleft surgery done during the period. There were 5 syndromic and 34 non-syndromic cases. Age of patients at time of surgery ranged between 3 months and 32 years. There were 24 bilateral cleft lip and palate deformities and 15 bilateral cleft lip deformities. Thirty-one of the cases were primary surgery, while 8 were secondary (revision surgery. The most common surgical technique employed was modified Fork flap (Millard technique, which was employed in 37 (95% cases. Conclusion: Bilateral cleft lip deformity is a common cleft deformity seen in clinical practice, surgical repair of which can be a challenge to an experienced surgeon. A modified Fork flap technique for repair of bilateral cleft lip is a reliable and versatile technique associated with excellent surgical outcome.

  15. Six years analysis of cleft palate in a university hospital center

    Directory of Open Access Journals (Sweden)

    Farahvash M

    1999-08-01

    Full Text Available Cleft palate is a congenital condition that occurs with the incidence rate of one out of 2000 births. This anomaly produces intraoral pressure changes (increase or decrease, can cause speech, sucking and feeding problems of involved patient. On the other hand, if cleft palate is associated with cleft of the lip or alveolar area, growth and alignment of teeth may change the appearance of the patient and affect the psychologic and occupational future of the patient. Eustatian tube malfunction in involved neonates increase. Many procedures are used to repair the cleft palate and correction of palatal muscles which are: 1 Von Langenbeck (18.5%. 2 Veau-Wardil-Kilner (72.5%. 3 Double opposing Z-Plasty (9%. In this research the demographic criteria of patients including age of the patient at operation rime (mean age 30.14 months, city of residence, family history of cleft palate (12.4%, familial relation of parents (15.2%, associated anomalies, complete or incomplete lesion, weight of patients at the time of surgery (mean 11.28 Kg, hemoglobin (11.3 mg/dl, complications, otitis media and the side of cleft palate are studied in 178 admitted patients to Imam General Hospital between 1989 and 1995.

  16. Unusual foreign body in the nasal cavity of an adult with repaired cleft lip and palate.

    Science.gov (United States)

    Ravikumar, Nagabhairava; GunaShekhar, M; Prasad, S Raghavendra; Lalitha, N; Raju, P Ramanjaneya; Natesh, Y A

    2015-03-01

    Intranasal foreign bodies arising from dental clinical practice, especially in patients with cleft lip and palate (CLP) occur rarely and are very scarce in the literature. This article reports an unusual case of a dental impression material presenting as a foreign body in the nasal cavity of an adult with repaired CLP who presented for dental prosthetic rehabilitation. To our knowledge, this is only the second report presenting nasal foreign body in a cleft patient arising due to a dental impression procedure.

  17. Association of generalized aggressive periodontitis and ectrodactyly-ectodermal dysplasia-cleft syndrome

    OpenAIRE

    Rosamma Joseph; Nath, Sameera G.

    2012-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly, ectodermal dysplasia, and facial clefting. Even though literature has documented the association of various genetic disorders with aggressive periodontitis, the periodontal manifestations in patients with EEC syndrome have never been addressed. This case report presents the periodontal status of three patients in a family with EEC syndrome. The presence of gener...

  18. Irf6 directly regulates Klf17 in zebrafish periderm and Klf4 in murine oral epithelium, and dominant-negative KLF4 variants are present in patients with cleft lip and palate

    Science.gov (United States)

    Liu, Huan; Leslie, Elizabeth J.; Jia, Zhonglin; Smith, Tiffany; Eshete, Mekonen; Butali, Azeez; Dunnwald, Martine; Murray, Jeffrey; Cornell, Robert A.

    2016-01-01

    Non-syndromic (NS) cleft lip with or without cleft palate (CL/P) is a common disorder with a strong genetic underpinning. Genome-wide association studies have detected common variants associated with this disorder, but a large portion of the genetic risk for NSCL/P is conferred by unidentified rare sequence variants. Mutations in IRF6 (Interferon Regulatory Factor 6) and GRHL3 (Grainyhead-like 3) cause Van der Woude syndrome, which includes CL/P. Both genes encode members of a regulatory network governing periderm differentiation in model organisms. Here, we report that Krüppel-like factor 17 (Klf17), like Grhl3, acts downstream of Irf6 in this network in zebrafish periderm. Although Klf17 expression is absent from mammalian oral epithelium, a close homologue, Klf4, is expressed in this tissue and is required for the differentiation of epidermis. Chromosome configuration capture and reporter assays indicated that IRF6 directly regulates an oral-epithelium enhancer of KLF4. To test whether rare missense variants of KLF4 contribute risk for NSCL/P, we sequenced KLF4 in approximately 1000 NSCL/P cases and 300 controls. By one statistical test, missense variants of KLF4 as a group were enriched in cases versus controls. Moreover, two patient-derived KLF4 variants disrupted periderm differentiation upon forced expression in zebrafish embryos, suggesting that they have dominant-negative effect. These results indicate that rare NSCL/P risk variants can be found in members of the gene regulatory network governing periderm differentiation. PMID:26692521

  19. Simulating clefts in pumpkin balloons

    Science.gov (United States)

    Baginski, Frank; Brakke, Kenneth

    2010-02-01

    The geometry of a large axisymmetric balloon with positive differential pressure, such as a sphere, leads to very high film stresses. These stresses can be significantly reduced by using a tendon re-enforced lobed pumpkin-like shape. A number of schemes have been proposed to achieve a cyclically symmetric pumpkin shape, including the constant bulge angle (CBA) design, the constant bulge radius (CBR) design, CBA/CBR hybrids, and NASA’s recent constant stress (CS) design. Utilizing a hybrid CBA/CBR pumpkin design, Flight 555-NT in June 2006 formed an S-cleft and was unable to fully deploy. In order to better understand the S-cleft phenomenon, a series of inflation tests involving four 27-m diameter 200-gore pumpkin balloons were conducted in 2007. One of the test vehicles was a 1/3-scale mockup of the Flight 555-NT balloon. Using an inflation procedure intended to mimic ascent, the 1/3-scale mockup developed an S-cleft feature strikingly similar to the one observed in Flight 555-NT. Our analysis of the 1/3-scale mockup found it to be unstable. We compute asymmetric equilibrium configurations of this balloon, including shapes with an S-cleft feature.

  20. Bilateral cleft lip nasal deformity

    Directory of Open Access Journals (Sweden)

    Singh Arun

    2009-01-01

    Full Text Available Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity. Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention. There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth. This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it′s management both at the time of cleft lip repair

  1. Preliminary study of endocrine dysfunction in patients with Rathke's cleft cyst%单纯性Rathke囊肿患者内分泌功能改变的初步研究

    Institute of Scientific and Technical Information of China (English)

    魏广玉; 吕朝晖; 窦京涛; 母义明; 陆菊明

    2008-01-01

    A retrospective study of endocrine dysfunction in 65 patients with Rathke's cleft cyst (RCC) was conducted. Before surgical operation, most patients with RCC had various degrees of pituitary dysfunction.Impaired gonadotropin secretion and prolactinemia were the prevalent abnormalities. Erectile dysfunction or diminished libido in male and menstrual disorder or galactorrhea in female were frequently seen. The panhypopituitarism appeared in a few patients with RCC. The patients would benefit by surgical removal, however,attention should be paid to avoid new damage of the pituitary gland during operation.%回顾性分析65例单纯性Rathke囊肿患者手术前后内分泌功能改变,术前存在的垂体激素分泌异常以垂体-性腺激素分泌受损和高催乳素血症多见,最常见症状女性为月经紊乱及溢乳、男性为性欲减退及勃起功能障碍,严重者可发生全垂体功能减退甚至危象.手术可以有效改善受损的垂体功能,也可能导致新的损害.

  2. Cleft lip and cleft palate relationship with familial marriage: a study in 136 cases

    Directory of Open Access Journals (Sweden)

    Azimi C

    2010-02-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Clefts of the lip and palate are one of the most common congenital birth anomalies. Genetic factors play a great role in the etiology of them and the high percentage of the consanguineous marriage of the parents of the affected persons is one of the reasons. These defects not only make abnormal changes on appearance of the neonate, but also make a lot of stress and psychological problems for the patients and their families. Study on the prevalence of clefts, their risk factors and also genetic counseling for affected persons and their families can be a guideline for general population and probably reduce these anomalies over the generations."n"nMethods: Patients referred to the Department of Genetics, Imam Khomeini Hospital, Tehran, Iran were studied. A total of 7374 pedigrees of all the patients admitted to the Department, were studied during 2002-2005 and 99 pedigrees with the patients with cleft lip± palate or isolated cleft palate were separated. The total number of cases among these 99 pedigrees was 136. The effects of consanguineous marriage, positive family history and sex were investigated among cases."n"nResults: 70.8% of patients with syndromic clefts and 58.7% of patients with nonsyndromic CL

  3. 腭裂不同程度与腭裂语音关系的临床研究%The clinical study of cleft palate speech with different cleft palate

    Institute of Scientific and Technical Information of China (English)

    杨静; 李永生; 冯雁; 王瑜; 邹弘驹; 曹阳

    2013-01-01

    Objective: To investigate the influence of speech for different cleft palate. Methods: 45 cases include 15 cases of soft cleft palate and 15 cases of uncompleted cleft palate and 15 cases of complete cleft palate had been studied with speech evaluate. Results: The speech unclearness appeared positive relation with severe degree of cleft palate. The fact is speech of soft palate cleft is most mild than that of other two groups, the speech of two groups of uncompleted and completed cleft were most severe , and there is no obviously difference between both of them. Conclusion: The pronunciation of patients with every kind of cleft palate were not clear, cleft palate must be accepted palate plastic operation.%目的:探讨不同程度的腭裂对患者语音的影响。方法选取45例腭裂患者,包括软腭裂、不完全性腭裂及完全性腭裂患者各15例,行语音评估。结果腭裂程度越重,患者腭裂语音越明显。软腭裂患者腭裂语音较轻,不全腭裂和完全腭裂组最重。结论不同程度的腭裂均会影响患者发音,腭裂均应行手术修复。

  4. Incidence of Cleft Lip and Palate in Uganda

    NARCIS (Netherlands)

    Dreise, Marieke; Galiwango, George; Hodges, Andrew

    2011-01-01

    Objective: The purpose of the study was to estimate the need for resources for cleft repairs in Uganda by determining the overall incidence of oral-facial clefts and the ratio of isolated cleft lip to isolated cleft palate to cleft lip and palate. Design: A 1-year prospective study was implemented i

  5. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

    OpenAIRE

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  6. An integrative approach for comparing microcirculation between normal and alveolar cleft gingiva in children scheduled for secondary bone grafting procedures

    NARCIS (Netherlands)

    D.M.J. Milstein; Y.W. Cheung; L. Ziukaite; C. Ince; H.P. van den Akker; J.A.H. Lindeboom

    2013-01-01

    Objective The aim of this study was to compare microcirculatory parameters in normal versus alveolar cleft gingiva in children selected for secondary bone grafting procedures. Study Design This study included 11 consecutive patients with complete unilateral alveolar clefts who required secondary bon

  7. Secondary bone grafting for alveolar cleft in children with cleft lip or cleft lip and palate

    NARCIS (Netherlands)

    J. Guo; C. Li; Q. Zhang; G. Wu; S.A. Deacon; J. Chen; H. Hu; S. Zou; Q. Ye

    2011-01-01

    BACKGROUND: Secondary alveolar bone grafting has been widely used to reconstruct alveolar cleft. However, there is still some controversy. OBJECTIVES: To compare the effectiveness and safety of different secondary bone grafting methods. SEARCH STRATEGY: The final electronic and handsearches were car

  8. Esthetic, Functional, and Everyday Life Assessment of Individuals with Cleft Lip and/or Palate

    Science.gov (United States)

    Papamanou, Despina A.; Karamolegkou, Marina; Dorotheou, Domna

    2015-01-01

    Objectives. To evaluate the level of satisfaction of individuals with cleft lip and/or palate (CLP) and their parents concerning the esthetic and functional treatment outcomes, the impact of the cleft on everyday life, and potential associations with treatment outcome satisfaction. Subjects and Methods. The sample consisted of 33 patients (7 CP, 20 unilateral CLP, and 6 bilateral CLP; median age: 17.1, range: 9.0–33.1 years) and 30 parents, who responded to a questionnaire in an interview-guided session. All participants received their orthodontic treatment at the Department of Orthodontics in the University of Athens. Results. Patients and their parents were quite satisfied with esthetics and function. Patients with UCLP primarily were concerned about nose esthetics (BCLP about lip esthetics and CP about speech). Increased satisfaction was associated with decreased influence of the cleft in everyday life (0.35 < rho < 0.64, P < 0.05). Parents reported significant influence of the cleft on family life, while patients did not. Conclusions. Despite the limited sample size of subgroups, the main concerns of patients with different cleft types and the importance of satisfying lip, nose, and speech outcomes for an undisturbed everyday life were quite evident. Thus, the need for targeted treatment strategies is highlighted for individuals with cleft lip and/or palate. PMID:26064918

  9. Objective tools to analyze the lower lateral cartilage in unilateral cleft lip nasal deformities.

    Science.gov (United States)

    Saleh, Mohamed A; Elshahat, Ahmed; Emara, Maher; Hussein, Heba; Gould, Lisa; Smith, David; Awad, Mostafa A

    2011-07-01

    Correction of cleft lip nasal deformity is an elusive goal. A controversy exists regarding the cause of the deformity, and therefore, there is a controversy of how to correct the deformity. Extrinsic theory is based on the presence of deformational forces from outside. The intrinsic theory is associated with deficiency of the lower lateral cartilage. The aim of this study was to use new objective tools to compare morphologically and histologically between the lower lateral cartilages of cleft and noncleft sides in patients with unilateral cleft lip nasal deformity. This study included 16 patients. They were operated on to correct unilateral cleft lip nasal deformity. Length, width, and thickness of lateral crura of the lower lateral cartilages of cleft and noncleft sides were measured. Punch biopsies from the middle part of the caudal ends of lateral crura were taken and sent for histologic and immunohistochemical studies. The lateral crura of the cleft side were significantly wider and shorter and tend to be thinner than those of the noncleft side. There was no significant difference in the chondroblast, chondrocyte, and total cellular number in the lower lateral cartilage of the cleft and noncleft sides. There was significantly less glycosaminoglycan content in the ground matrix of the lower lateral cartilage of cleft side. In conclusion, the use of digital sliding caliber in measuring the diminutions of the lower lateral cartilage and image analyzer to quantify the proteoglycans, glycosaminoglycans, fibroblast growth factor 18, and collagen content is very effective objective tools to compare the cleft and noncleft alar cartilage. PMID:21772161

  10. Patterns of Cleft Lip and Cleft Palate in Northern Pakistan

    OpenAIRE

    Mansoor Khan; Hidayat Ullah; Shazia Naz; Tahmeed Ullah; Hafeezullah Khan; Muhammad Tahir; Obaid Ullah

    2012-01-01

    Objectives: To determine the frequency of different types of cleft lip and palate, geographical distribution and its association with consanguinity, family history and other syndromes in the Northern Pakistani population. Study design: Descriptive cross-sectional study. Settings: This study was performed in Plastic and Reconstructive units of Hayat Medical Complex Peshawar Pakistan, Aman Hospital Peshawar Pakistan and Abasin Hospital Peshawar, Pakistan from November 2010 to December 20...

  11. Cleft and Craniofacial Mission Care: Management of Facial Clefts: International Missions.

    Science.gov (United States)

    Moses, Jeffrey J; Rochelle, Whitney J

    2016-05-01

    This article guides readers in the venture of creating, funding, or volunteering with a facial-cleft mission treatment team. This outline is intended to emphasize longitudinal care for all patients. Advanced planning is key to mission success. Part of this is researching and becoming familiar with the safety, regulations, culture, facilities available, and local service organizations. Many of the service organizations listed in this article have valuable contacts with local dentists and surgeons within the community. They also have insight into the local facilities, hospitals, water sources, sanitation conditions, and equipment potential. PMID:27150306

  12. Nasolabial symmetry and aesthetics in children with complete unilateral cleft lip and palate.

    Science.gov (United States)

    Fudalej, Piotr; Katsaros, Christos; Hozyasz, Kamil; Borstlap, Wilfred A; Kuijpers-Jagtman, Anne Marie

    2012-10-01

    The objective of this study was to evaluate the association between nasolabial symmetry and aesthetics in children with complete unilateral cleft lip and palate (CUCLP). Frontal and basal photographs of 60 consecutively treated children with CUCLP (cleft group: 41 boys and 19 girls, mean (SD) age 11 (2) years) and 44 children without clefts (control group: 16 boys and 28 girls, mean (SD) age 11(2) years), were used for evaluation of nasolabial symmetry and aesthetics. Nasal and labial measurements were made to calculate the coefficient of asymmetry (CA). The 5-grade aesthetic index described by Asher-McDade et al. was used to evaluate nasolabial appearance. Correlation and regression analysis were used to identify an association between aesthetics and CA, sex, and the presence of CUCLP. Ten measurements in the cleft, and 2 in the control, group differed significantly between the cleft and non-cleft (or right and left) sides, respectively. The significantly higher values of 9 of 11 CA in the children with CUCLP indicated that they had more asymmetrical nasolabial areas than children without clefts. However, the regression analyses showed that only a few CA were associated with nasolabial aesthetics. In conclusion, nasolabial aesthetics and nasolabial symmetry seem to be only weakly associated in patients with CUCLP.

  13. Environmental factors related to the occurrence of oral clefts in a Brazilian subpopulation

    Science.gov (United States)

    Campos Neves, Ana Thereza de Saboia; Volpato, Luiz Evaristo Ricci; Espinosa, Mariano Martinez; Aranha, Andreza Maria Fabio; Borges, Alvaro Henrique

    2016-01-01

    Background: A cross-sectional study was conducted at the Craniofacial Rehabilitation Center in the General Hospital of the University of Cuiabá, Cuiabá city, Mato Grosso, Brazil. Materials and Methods: Poisson regression model was used to analyze the relationship between antenatal factors and the occurrence of oral clefts in 116 patients. Results: Oral clefts were more common in males (64.66%) and White race (46.02%). The mean age of the children was 21.91 months. The most common type of cleft was cleft lip and palate (CLP, 55.17%). Maternal and paternal smoking in the first trimester of pregnancy and parity were significantly associated with the occurrence of CLP. Parent's age, educational level, and occupation did not interfere in the occurrence of oral clefts. There was also no significant association between maternal illness, medication use, alcohol consumption, and maternal exposure to chemicals in the first trimester of pregnancy and the occurrence of clefts in this population. Conclusion: The analysis of the environmental factors present during the pregnancy of children with oral clefts revealed a significant association between parity (second onward), maternal smoking, and paternal smoking and the occurrence of CL and/or palate in this population. PMID:27397957

  14. Puente adhesivo en paciente con labio leporino y fisura velopalatina: Una solución temporal con compromiso estético Adhesive bridge in a patient with cleft lip and palate: A temporary solution with esthetic commitment

    Directory of Open Access Journals (Sweden)

    N Leiva

    2013-04-01

    Full Text Available En la actualidad el aumento de la demanda estética y la necesidad de procedimientos mínimamente invasivos ha llevado a un aumento de la técnica adhesiva. Ribbond® es una marca de fibra de polietileno que permite un eficiente traspaso de fuerzas, es virtualmente plegable y se adapta fácilmente a la morfología dentaria y al contorno del arco dentario. Dentro de sus principales características podemos encontrar: Biocompatibilidad, inerte, translúcido y durable. Dentro de la odontología se le han dado distintos usos para esta fibra, ya sea como mantenedor de espacio, puente adhesivo, poste y núcleo endodóntico, ferulización, restauraciones tipo inlay y estabilización postortodóncica. En el caso de los pacientes fisurados se ha hecho relevante el uso de Ribbond® como puente adhesivo ya que permite mejorar la estética y rehabilitar temporalmente los espacios desdentados causados por la ausencia de un incisivo, principalmente una vez terminado el tratamiento de ortodoncia y en espera de una rehabilitación definitiva, aún más cuando el paciente no ha terminado su crecimiento y desarrollo. En este artículo se presenta un caso clínico de un paciente con labio leporino y fisura velopalatina operado demostrativo de la técnica de rehabilitación con Ribbond®.Today the increasing demand of aesthetic and minimally invasive procedures has led to a boom of the adhesive technique. Ribbond® is a polyethylene fiber that allows an efficient transfer of forces, is virtually foldable and is easily adapted to tooth morphology and dental arch contour. Among its main features we can find: Biocompatibility, inert, translucent and durable. In dentistry, different uses have been given to this fiber, whether as space maintainer, adhesive bridge, endodontic post and core, splinting, inlay type restorations and postorthodontic stabilization. In the case of cleft lip and palate patients the use of Ribbond® as an adhesive bridge has become relevant, as

  15. Bilaterally cleft lip, limb defects, and haematological manifestations: Roberts syndrome versus TAR syndrome.

    Science.gov (United States)

    Urban, M; Opitz, C; Bommer, C; Enders, H; Tinschert, S; Witkowski, R

    1998-09-23

    We report on a 13-year-old patient followed since birth. He is the only offspring of young, non-consanguineous German parents. His mother has an isolated left cleft of lip and a cleft palate. At birth, our patient presented with bilaterally cleft lip/cleft palate, phocomelia of upper limbs with normal hands, and mild symmetrical deficiencies of the long bones of the lower limbs. Haematological evaluation demonstrated a leukaemoid reaction during a urinary tract infection as well as intermittent thrombocytopenia and episodes of marked eosinophilia during the first two years of life. Intellectual development has been normal. Comparison with two similar cases from the literature suggests a non-random phenotypic overlap of Roberts syndrome (MIM 268300) and TAR syndrome (MIM 274000). Such clinical constellations may be key observations to understand the genetic relationship of Roberts syndrome and TAR syndrome in future phenotype-genotype correlations. PMID:9788553

  16. Quantitative assessment of healthy and reconstructed cleft lip using ultrasonography

    Science.gov (United States)

    Devadiga, Sumana; Desai, Anil Kumar; Joshi, Shamsunder; Gopalakrishnan, K.

    2016-01-01

    Purpose: This study is conducted to investigate the feasibility of echographic imaging of tissue thickness of healthy and reconstructed cleft lip. Design: Prospective study. Materials and Methods: The study was conducted in SDM Craniofacial Unit, Dharwad and was approved by Local Institutional Review Board. A total of 30 patients, age group ranging from 4 to 25 years, of which 15 postoperative unilateral cleft lip constituted the test group. The remaining 15 with no cleft deformities, no gross facial asymmetry, constituted the control group. The thickness of the mucosa, submucosa, muscle and full thickness of the upper lip were measured with the transversal images using ultrasonography at midpoint of philtrum, right and left side philtral ridges and vermillion border, at 1, 3, 6 months interval. Results: There was an increase in muscle thickness at the vermillion border (mean = 6.9 mm) and philtral ridge (5.9 mm). Equal muscle thickness were found between the normal and test group at 6 months follow-up in a relaxed position, which was statistically significant (P = 0.0404). Conclusion: Quantitative assessment of thickness and echo levels of various lip tissues are done with proper echographic calibration. Diagnostic potentials of this method for noninvasive evaluation of cleft lip reconstructions were achieved by this study. PMID:27134448

  17. FOXE1 Association with both Isolated Cleft Lip with or without Cleft Palate; and Isolated Cleft Palate

    DEFF Research Database (Denmark)

    Moreno, Lina M; Mansilla, Maria Adela; Bullard, Steve A;

    2009-01-01

    Nonsyndromic orofacial clefts are a common complex birth defect caused by genetic and environmental factors and/or their interactions. A previous genome-wide linkage scan discovered a novel locus for cleft lip with or without cleft palate (CL/P) at 9q22-q33. To identify the etiologic gene, we......) and rs4460498 (p=6.51E-12) were located inside a 70Kb high LD block containing FOXE1. Association signals for Caucasians and Asians clustered 5' and 3' of FOXE1, respectively. Isolated cleft palate (CP) was also associated indicating that FOXE1 plays a role in two phenotypes thought to be genetically...

  18. Análise do trato vocal em pacientes com nódulos, fendas e cisto de prega vocal Vocal tract analysis in patients with vocal fold nodules, clefts and cysts

    Directory of Open Access Journals (Sweden)

    Raquel Buzelin Nunes

    2009-04-01

    the frequency of supraglottic vocal tract adjustments in dysphonic women with nodules, clefts and cysts. METHODS: We assessed 31 dysphonic women, with age ranging between 18 and 45 years, with vocal alteration and a diagnosis of nodules, middle-posterior cleft and cyst, and we carried out a summarized evaluation of the sensory-motor and oral systems and the patients were submitted to video-laryngostroboscopy and nasal and laryngeal fibroscopy. Three distinct groups were selected: patients with bilateral nodules, clefts and cysts, with similar glottic configuration. Their vocal tracts were visually analyzed through exams of nasal and laryngeal fibroscopy, by speech and hearing therapists and otorhinolaryngologists, checking the following parameters: supraglottic constriction, larynx vertical mobility, pharyngeal constriction and tongue mobility. The data was statistically described and treated. RESULTS: during visual analysis we did not find statistically significant differences which would separate the glottic alterations groups. CONCLUSION: There was no correlation between supraglottic tract adjustments with any particular type of glottic alteration. These are individual behaviors that generate adjustments and justify the different vocal qualities in patients with the same type of laryngeal alteration.

  19. Parents' age and the risk of oral clefts

    DEFF Research Database (Denmark)

    Bille, C.; Skytthe, A.; Vach, W.;

    2005-01-01

    . METHODS: Among the 1,489,014 live births in Denmark during 1973-1996, there were 1920 children with nonsyndromic cleft lip with or without cleft palate and 956 children with nonsyndromic cleft palate. We used logistic regression to assess the impact of parental age on the occurrence of cleft lip...... with or without cleft palate and cleft palate. Interaction between mother's and father's age was included in the analysis. RESULTS: Separate analyses of mother's and father's age showed that older age was associated with increased risk of both cleft lip with or without cleft palate and cleft palate only....... In a joint analysis, both maternal and paternal ages were associated with the risk of cleft lip with or without cleft palate, but the contribution of each was dependent on the age of the other parent. In the analysis of cleft palate only, the effect of maternal age disappeared, leaving only paternal age...

  20. Stability after Cleft Maxillary Distraction Osteogenesis or Conventional Orthognathic Surgery

    DEFF Research Database (Denmark)

    Andersen, Kristian; Svenstrup, Martin; Pedersen, Thomas Klit;

    2015-01-01

    OBJECTIVES: To compare stability of maxillary advancements in patients with cleft lip and palate following distraction osteogenesis or orthognathic surgery. MATERIAL AND METHODS: INCLUSION CRITERIA: 1) cleft lip and palate, 2) advancement > 8 mm. Eleven patients comprised the distraction...... changed in CONVG. At follow-up (T3), VOB increased in CONVG compared with DOG, (P = 0.01). Vertical position of A point differed between the groups (P = 0.04). No significant intergroup differences between soft tissue parameters occurred. CONCLUSIONS: Distraction osteogenesis resulted in a stable position...... osteogenesis group (DOG). Seven patients comprised the orthognathic treatment group (CONVG). Skeletal and soft tissue points were traced on lateral cephalograms: T1 (preoperatively), T2 (after surgery), T3 (follow-up). Group differences were analyzed using Students t-test. RESULTS: At T1-T2, advancement of 6...

  1. Cleft Lip and Palate Care in Nigeria

    OpenAIRE

    Adeyemi, Tokunbo Abigail

    2016-01-01

    BACKGROUND; The World Health Organisation has recommended the need to standardise cleft care globally. In Europe, the Eurocleft project was a concerted effort to improve on the standards of care for children with cleft lip and palate. Certain recommendations were made that were used to judge the standards of care offered, this eventually led to reorganization of services. Improving on standards of cleft care in Nigeria, would require a starting point, by determining what is currently being of...

  2. Cleft Lip and Cleft Palate--What to Know and Who Can Help

    Science.gov (United States)

    Apel, Laura

    2008-01-01

    Craniofacial defects such as cleft lip and cleft palate are among the most common of all birth defects in the United States, with one in every 600 newborns affected. Cleft lip and/or palate can occur as an isolated condition or may be one component of an inherited disease or syndrome. Dealing with the condition is an extremely difficult and…

  3. High Dosage Folic Acid Supplementation, Oral Cleft Recurrence and Fetal Growth

    Directory of Open Access Journals (Sweden)

    Carla Padovani

    2013-02-01

    Full Text Available Objectives: To evaluate the effects of folic acid supplementation on isolated oral cleft recurrence and fetal growth. Patients and Methods: The study included 2,508 women who were at-risk for oral cleft recurrence and randomized into two folic acid supplementation groups: 0.4 and 4 mg per day before pregnancy and throughout the first trimester. The infant outcome data were based on 234 live births. In addition to oral cleft recurrence, several secondary outcomes were compared between the two folic acid groups. Cleft recurrence rates were also compared to historic recurrence rates. Results: The oral cleft recurrence rates were 2.9% and 2.5% in the 0.4 and 4 mg groups, respectively. The recurrence rates in the two folic acid groups both separately and combined were significantly different from the 6.3% historic recurrence rate post the folic acid fortification program for this population (p = 0.0009 when combining the two folic acid groups. The rate of cleft lip with palate recurrence was 2.9% in the 0.4 mg group and 0.8% in the 4 mg group. There were no elevated fetal growth complications in the 4 mg group compared to the 0.4 mg group. Conclusions: The study is the first double-blinded randomized clinical trial (RCT to study the effect of high dosage folic acid supplementation on isolated oral cleft recurrence. The recurrence rates were similar between the two folic acid groups. However, the results are suggestive of a decrease in oral cleft recurrence compared to the historic recurrence rate. A RCT is still needed to identify the effect of folic acid on oral cleft recurrence given these suggestive results and the supportive results from previous interventional and observational studies, and the study offers suggestions for such future studies. The results also suggest that high dosage folic acid does not compromise fetal growth.

  4. High Dosage Folic Acid Supplementation, Oral Cleft Recurrence and Fetal Growth

    Science.gov (United States)

    Wehby, George L.; Félix, Têmis Maria; Goco, Norman; Richieri-Costa, Antonio; Chakraborty, Hrishikesh; Souza, Josiane; Pereira, Rui; Padovani, Carla; Moretti-Ferreira, Danilo; Murray, Jeffrey C.

    2013-01-01

    Objectives: To evaluate the effects of folic acid supplementation on isolated oral cleft recurrence and fetal growth. Patients and Methods: The study included 2,508 women who were at-risk for oral cleft recurrence and randomized into two folic acid supplementation groups: 0.4 and 4 mg per day before pregnancy and throughout the first trimester. The infant outcome data were based on 234 live births. In addition to oral cleft recurrence, several secondary outcomes were compared between the two folic acid groups. Cleft recurrence rates were also compared to historic recurrence rates. Results: The oral cleft recurrence rates were 2.9% and 2.5% in the 0.4 and 4 mg groups, respectively. The recurrence rates in the two folic acid groups both separately and combined were significantly different from the 6.3% historic recurrence rate post the folic acid fortification program for this population (p = 0.0009 when combining the two folic acid groups). The rate of cleft lip with palate recurrence was 2.9% in the 0.4 mg group and 0.8% in the 4 mg group. There were no elevated fetal growth complications in the 4 mg group compared to the 0.4 mg group. Conclusions: The study is the first double-blinded randomized clinical trial (RCT) to study the effect of high dosage folic acid supplementation on isolated oral cleft recurrence. The recurrence rates were similar between the two folic acid groups. However, the results are suggestive of a decrease in oral cleft recurrence compared to the historic recurrence rate. A RCT is still needed to identify the effect of folic acid on oral cleft recurrence given these suggestive results and the supportive results from previous interventional and observational studies, and the study offers suggestions for such future studies. The results also suggest that high dosage folic acid does not compromise fetal growth. PMID:23380913

  5. [Cleft lip and palate in Campeche Mayas].

    Science.gov (United States)

    Weiss, K M; Georges, E; Levy, B; Aguirre, A; Portilla, R J; Gaitán, C L; Leyva, E; Rodríguez, T

    1988-07-01

    It has been suggested that among American Indians, as in some genetically-related Asiatic ethnic groups, incidence of cleft lip and/or cleft palate is higher than among people of Caucasian extraction. Such hypothesis, plus growing demand for services observed at a center for the surgery of cleft lip and cleft palate in Campeche state, led the authors to undertake research among the Maya residents of that region. However, neither careful review of case histories nor field research performed in several Indian communities could confirm the hypothesis of a higher incidence among this ethnic community.

  6. Improving Informed Consent for Cleft Palate Repair

    Science.gov (United States)

    2016-09-22

    Cleft Palate; Jaw Abnormalities; Maxillofacial Abnormalities; Mouth Abnormalities; Congenital Abnormalities; Jaw Diseases; Musculoskeletal Diseases; Craniofacial Abnormalities; Musculoskeletal Abnormalities; Stomatognathic Diseases; Stomatognathic System Abnormalities

  7. A clinical and demographic profile of the cleft lip and palate in Sub-Himalayan India: A hospital-based study

    Directory of Open Access Journals (Sweden)

    Jyoti Dvivedi

    2012-01-01

    Full Text Available Objectives: To study the cleft lip and cleft palate in the poverty stricken Sub-Himalayan Garhwal region of India, being a commonly seen congenital abnormality and scarcity of studies about the demography of cleft in this region. Design: A prospective cohort observational case series was performed on 4657 cleft patients at a Tertiary care Hospital in Dehradun, India, over a period of 5 years. Outcome measures: The authors investigated the differences between age and sex with cleft status and family history of clefts, birth order, religion, socioeconomic status, parent literacy, source of information for treatment, haematological investigations showing the status of infection and coagulation in such children and satisfaction after treatment. Results: Seventy-two percent parents of cleft lip patients were illiterate, and only 8% were graduates, the majority of patients were from the low socioeconomic class. The siblings of 1.1% of the cleft patients had similar deformity. Anemia was seen in 83.16% cases which was commonly microcytic hypochromic type and eosinophilia was seen in 25.50% of cases. In the coagulation profile, International Nationalized Ratio was found to be raised in 52.12%. Almost 95% of the families were fully satisfied by the treatment and results. Conclusion: This study will provide baseline information on the status of these less privileged cleft patients in this mountainous region for future reference to health workers.

  8. Dental anomalies in children with cleft lip and palate in Western Australia

    Science.gov (United States)

    Nicholls, Wendy

    2016-01-01

    Objective: The purpose of this paper was to describe the prevalence and type of dental anomalies in the primary and permanent dentition in children with a cleft condition at Princess Margaret Hospital in Perth, Western Australia. Materials and Methods: The details of 162 current dental patients extracted from the main dental database through their year of birth for the period 1998–2001 were selected consecutively. Dental records and X-rays were examined by one examiner (WN) and verified by a second examiner (RB) to determine dental development. The mean age of the subjects was 10.8 years with equal numbers of males and females. Subjects were further divided into cleft type; unilateral cleft lip (UCL) and palate, bilateral cleft lip (BCL) and palate, UCL, BCL, and cleft palate. Results: One hundred sixty-two subjects were grouped into 21 categories of anomaly or abnormality. Prevalence rates for the categories were calculated for the overall group and for gender and cleft type. Conclusion: Overall, 94% of patients were found to have at least one dental anomaly, with fifty-six (34%) patients having more than one anomaly or abnormality. PMID:27095907

  9. Percutaneous balloon kyphoplasty of osteoporotic vertebral compression fractures with intravertebral cleft

    Directory of Open Access Journals (Sweden)

    Bao Chen

    2014-01-01

    Full Text Available Background: Intravertebral cleft is a structural change in osteoporotic vertebral compression fractures (OVCF, which is the manifestation of ischemic vertebral osteonecrosis complicated with fracture nonunion and pseudoarthrosis and appears in the late stage of OVCF. Despite numerous studies on OVCF, few aim to evaluate the clinicoradiological characteristics and clinical significance of intravertebral cleft in OVCF. This study investigates clinicoradiological characteristics of intravertebral cleft in OVCF and the effect on the efficacy of percutaneous balloon kyphoplasty (PKP. Materials and Methods: PKP was performed on 139 OVCF patients without intravertebral cleft (group A and 44 OVCF patients with intravertebral cleft (group B. The frequency distribution of the affected vertebral body, bone cement infusion volume, imaging manifestation, leakage rate and type, preoperative and postoperative height of the affected vertebral body, visual analog scale (VAS and Oswestry disability index (ODI score were evaluated. Results: Significant differences were found in the frequency distribution of the affected vertebral body and bone cement leakage type between the two groups ( P 0.05 were not detected. In both groups, the postoperative height of the affected vertebral body was significantly improved ( P 0.05. Conclusion: Intravertebral cleft exhibits specific clinical and imaging as well as bone cement formation characteristics. PKP can effectively restore the affected vertebral body height, alleviate pain, and improve daily activity function of patients.

  10. Fetal genetic risk of isolated cleft lip only versus isolated cleft lip and palate: A subphenotype analysis using two population-based studies of orofacial clefts in scandinavia

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2010-01-01

    BACKGROUND: Cleft lip only (CLO) and cleft lip and palate (CLP) are commonly regarded as variants of the same defect and are traditionally combined to form the single group of cleft lip with or without cleft palate (CL/P) prior to analysis. However, recent data have suggested that at least a subg...

  11. Three-dimensional analysis of naso-maxillary complex symmetry in patients with unilateral complete cleft lip and palate%单侧完全性唇腭裂患者鼻上颌复合体对称性的三维分析

    Institute of Scientific and Technical Information of China (English)

    倪初蕾; 贾绮林

    2014-01-01

    Objective To evaluate the symmetry of naso-maxillary complex in patients with unilateral complete cleft lip and palate with no palatoplasty history before 17 years old.Methods 9 patients (4 males and 5 females) with unilateral complete cleft lip and palate (UCLP) were selected.They all underwent lip repair before 2.5 years old and had no history of palatoplasty before 17 years.They received spiral CT scan and 3D reconstruction of their naso-maxillary complex.22 variables were measured in the reconstructed images and the symmetry of the complex were analyzed.Results The distances of NC-HP and PF-MSP in cleft side were 18.01 mm and 19.72 mm,which were 13.35 mm and 17.31 mm bigger than that in the noncleft side(P<0.05).The distances of NC-CP and U3-HP in cleft side were 59.64 mm and 39.55 mm,which were 64.13 mm and 39.70 mm shorter than that in the non-cleft side (P<0.05).The measurements showed a collapsed and sunken nasal cavity,a wider posterior hard palate and elevated canine in cleft side.There were also deviations of the middle structures.Crista galli,nasal septum,and the mid-point of upper central incisors deviated to cleft side,while skeletal anterior nasal spine deviated to noncleft side.Conclusions Most asymmetries are found in nasal cavity and the dentoalveolar area near the cleft.No significant asymmetry is found in upper cranialfacial strutures,nor in regions not in the adjacency of the cleft.%目的 三维分析成年期前未行腭裂修复术的单侧完全性唇腭裂(UCLP)患者的鼻上颌复合体形态对称性.方法 对9例2.5岁前完成唇裂修复术、成年前未行腭裂修复术的UCLP患者进行螺旋CT扫描、三维重建,选择22个头颅标志点进行定点测量,分析鼻上颌复合体的三维形态.结果 成年前未行腭裂修复术UCLP患者裂侧NC-HP、PF-MSP距离分别为18.01mm、19.72mm,大于健侧13.35mm、17.31mm(P<0.05);裂侧NC-CP、U3-HP距离分别为59.64mm、39.55mm,小于健侧64.13mm、39.70mm(P<0

  12. Hypertrophic scarring in cleft lip repair: a comparison of incidence among ethnic groups

    Directory of Open Access Journals (Sweden)

    Soltani AM

    2012-07-01

    Full Text Available Ali M Soltani, Cameron S Francis, Arash Motamed, Ashley L Karatsonyi, Jeffrey A Hammoudeh, Pedro A Sanchez-Lara, John F Reinisch, Mark M UrataDivision of Plastic and Maxillofacial Surgery at Children's Hospital Los Angeles, CA, USA; The Division of Plastic and Reconstructive Surgery at the Keck School of Medicine of the University of Southern California, Los Angeles, CA, USABackground: Although hypertrophic scar (HTS formation following cleft lip repair is relatively common, published rates vary widely, from 1% to nearly 50%. The risk factors associated with HTS formation in cleft patients are not well characterized. The primary aim of this retrospective study of 180 cleft lip repairs is to evaluate the frequency of postoperative HTS among various ethnic groups following cleft lip repair.Methods: A retrospective chart view of patients undergoing primary cleft lip repair over a 16-year period (1990–2005 by the senior surgeon was performed. The primary outcome was the presence of HTS at 1 year postoperatively. Bivariate analysis and multivariable logistic regression were used to evaluate potential risk factors for HTS, including ethnicity, type and laterality of cleft, and gender.Results: One hundred and eighty patients who underwent cleft lip repair were included in the study. The overall rate of postoperative HTS formation was 25%. Ethnicity alone was found to be an independent predictor of HTS formation. Caucasian patients had the lowest rate of HTS formation (11.8% and were used as the reference group. HTS rates were significantly higher in the other ethnicities, 32.2% in Hispanic patients (odds ratio [OR]: 3.51; 95% confidence interval [CI]: 1.53–8.85, and 36.3% for Asian patients (OR 4.27; 95% CI: 1.36–13.70. Sex, cleft type, and cleft laterality were not associated with increased rates of HTS.Conclusions: Differences in ethnic makeup of respective patient populations may be a major factor influencing the wide variability of reported

  13. Classification and operation in the treatment of maxillary retrusion of adult patients with cleft lip and palate%成人唇腭裂上颌后缩的分类和治疗方法

    Institute of Scientific and Technical Information of China (English)

    郑益略; 尹宁北; 赵振民; 孙晓梅; 姜婵媛; 童海洲; 麻恒源; 宋涛

    2016-01-01

    Objective To classify the patients with cleft lip and palate who need orthognathic surgery and to propose the corresponding operations.Methods From January 2005 to May 2015,121 patients with cleft lip and palate diagnosed as maxillary retrusion were treated by orthognathic surgery.Inclusion criteria:①male aged over 16,female aged over 14;②diagnosed as non-syndromic cleft lip and palate without systemic disease and other genetic diseases;③without previous orthodontic and orthognathic treatment;④having no other craniofacial malformation.Maxillary features and repaired types were recorded.Results 93 patients were included and divided into two categories depended on the dental crowding.Class Ⅰ:the teeth quantity and bone quantity is coordinated,space analysis ≤4 mm (mild dental crowding).The forward distance of maxillary less than 6 mm was defined as Class Ⅰ a (36 cases),more than 6 mm as Class Ⅰ b (28 cases).Class Ⅱ:the teeth quantity and bone quantity is not coordinated,space analysis > 4 mm (moderate or severe dental crowding).After the simulation of distraction osteogenesis,the anterior crossbite was corrected defined as Class Ⅱ a (23 cases),not corrected defined as Class Ⅱb (6 cases).Class Ⅰ a were corrected by conventional orthognathic surgery.While Class Ⅰ b were corrected by Le Fort Ⅰ maxillary advancement using distraction osteogenesis.Class Ⅱ a were repaired just by anterior maxillary distraction.While Class Ⅱb need to combine conventional orthognathic surgery with anterior maxillary distraction.All the patients were satisfied with the treatment effect.Conclusions The patients of cleft lip and palate with maxillary retrusion who need orthognathic surgery can be classified as the method mentioned above,and then choose the appropriate operations.%目的 对需要正颌外科治疗的唇腭裂上颌后缩的患者进行分类,并提出相应的手术方法.方法 2005年1月至2015年5月,共收治唇腭裂上颌后缩患者121

  14. Short mandible - a possible risk factor for cleft palate with/without a cleft lip

    DEFF Research Database (Denmark)

    Hermann, Nuno Vibe; Darvann, Tron Andre; Ersbøll, Bjarne Kjær;

    2014-01-01

    Structured Abstract Objectives To estimate the influence of a short mandible on the risk of developing a cleft palate with/without a cleft lip (CP). Setting and sample population The retrospective sample consisted of 115 2-month-old Danish infants with CP, and 70 control infants with unilateral...... the risk of having a cleft palate. Results The mean mandibular length in the group with CP was about 4mm shorter than in the control group. Odds ratio (OR) was calculated to be 0.58 (95% confidence interval 0.48-0.68), implying that an individual's risk of cleft palate with/without a cleft lip increases...... about 50% per mm decrease in mandibular length. Conclusions A special facial type including a short mandible is a possible risk factor for cleft palate, and it was found that the risk of cleft palate increases 58% per mm decreases in mandibular length....

  15. Presurgical nasoalveolar molding in unilateral cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Rahul J Hegde

    2015-01-01

    Full Text Available Unilateral cleft lip and palate (UCLP patients have an esthetic and functional compromise of the middle third of the face and nasal structures. To improve the esthetic result of lip repair, the concept of presurgical nasoalveolar molding (PNAM was brought into conception. PNAM is an easy and passive method of bringing the alveolus and lips together by redirecting the forces of natural growth. This case report documents a 2-year follow-up of PNAM in UCLP.

  16. Presurgical nasoalveolar molding in unilateral cleft lip and palate

    Science.gov (United States)

    Hegde, Rahul J.; Kharkar, Viraj R.; Kamath, Shamika

    2015-01-01

    Unilateral cleft lip and palate (UCLP) patients have an esthetic and functional compromise of the middle third of the face and nasal structures. To improve the esthetic result of lip repair, the concept of presurgical nasoalveolar molding (PNAM) was brought into conception. PNAM is an easy and passive method of bringing the alveolus and lips together by redirecting the forces of natural growth. This case report documents a 2-year follow-up of PNAM in UCLP. PMID:26681868

  17. Chemical meningitis: a rare presentation of Rathke's cleft cyst.

    Science.gov (United States)

    Mrelashvili, Anna; Braksick, Sherri A; Murphy, Lauren L; Morparia, Neha P; Natt, Neena; Kumar, Neeraj

    2014-04-01

    Rathke's cleft cysts (RCC) are usually benign, sellar and/or suprasellar lesions originating from the remnants of Rathke's pouch. Rarely, RCC can present with chemical meningitis, sellar abscess, lymphocytic hypophysitis, or intracystic hemorrhage. We describe an unusual presentation of RCC in which the patient presented with a clinical picture of chemical meningitis consisting of meningeal irritation, inflammatory cerebrospinal fluid profile, and enhancing pituitary and hypothalamic lesions, in addition to involvement of the optic tracts and optic nerve.

  18. The trochlear cleft: the ''black line'' of the trochlear trough

    Energy Technology Data Exchange (ETDEWEB)

    Wissman, Robert D.; Nepute, Joshua; Fischer, Nathaniel von; Radhakrishnan, Rupa; Hendry, Daniel [University of Cincinnati Medical Center, Department of Radiology, Cincinnati, OH (United States); Ingalls, Jerrell [Desert Radiologists, Las Vegas, NV (United States); Kenter, Keith [University of Cincinnati Medical Center, Department of Orthopaedic Surgery, Cincinnati, OH (United States)

    2012-09-15

    The ''cartilage black line sign'' is a recently described T2 dark cartilage lesion that we have identified appearing as a cleft in the trochlear trough. The purpose of our study was to define the MR imaging characteristics of a trochlear cleft, determine its incidence, and correlate the MR findings with arthroscopy. A total of 1,300 consecutive MR examinations of the knee were retrospectively reviewed by consensus of two fellowship-trained musculoskeletal radiologists. The MR imaging characteristics and location of a trochlear cleft were determined. Imaging results were compared to arthroscopy when available. Patient age and gender were compared to 25 randomly selected control patients without trochlear clefts. A total of 25 (1.9%) individuals (11 females and 14 males; age range 19-45 years; mean age 28 years) were diagnosed with a trochlear cleft. The control group consisted of 11 females and 14 males; age range 19-83 years; mean age 46 years. Mean cleft length was 7 mm (range 6-12 mm); cleft location was consistently in the lower trochlear trough. No full-thickness cartilage defects were identified in the eight individuals in whom arthroscopic correlation was available. A grade 2 cartilage lesion was identified in a single individual; another progressed from grade 0 to a full-thickness trochlear lesion over an 8-month interval. Eight individuals were athletes. No significant difference in gender was noted between the two groups, however, the study group was significantly younger p < 0.0001. A trochlear cleft is a rare finding in young active individuals. It most likely indicates an incomplete cartilage fissure which may rarely progress to a full-thickness defect. (orig.)

  19. Cleft Lip and Palate (For Parents)

    Science.gov (United States)

    ... with your doctor about the treatment plan. Surgical Treatment A cleft lip is usually surgically repaired in the hospital using general anesthesia when a child is 3 to 6 months old. If the cleft lip is wide, special procedures like lip adhesion or a molding plate device might help bring ...

  20. Maternal occupational risk factors for oral clefts

    NARCIS (Netherlands)

    Lorente, C; Cordier, S; Bergeret, A; De Walle, HEK; Goujard, J; Ayme, S; Knill-Jones, R; Calzolari, E

    2000-01-01

    Objectives This study investigated the role of maternal exposures at work during pregnancy in the occurrence of oral clefts. Methods The occupational exposures of 851 women (100 mothers of babies with oral clefts and 751 mothers of healthy referents) who worked during the first trimester of pregnanc

  1. Vitamin A and clefting: putative biological mechanisms

    NARCIS (Netherlands)

    Ackermans, M.M.; Zhou, J.H.; Carels, C.E.L.; Wagener, F.A.D.T.G.; Hoff, J.W. Von den

    2011-01-01

    Nutritional factors such as vitamin intake contribute to the etiology of cleft palate. Vitamin A is a regulator of embryonic development. Excess vitamin A can cause congenital malformations such as spina bifida and cleft palate. Therefore, preventive nutritional strategies are required. This review

  2. Dental materials for cleft palate repair.

    Science.gov (United States)

    Sharif, Faiza; Ur Rehman, Ihtesham; Muhammad, Nawshad; MacNeil, Sheila

    2016-04-01

    Numerous bone and soft tissue grafting techniques are followed to repair cleft of lip and palate (CLP) defects. In addition to the gold standard surgical interventions involving the use of autogenous grafts, various allogenic and xenogenic graft materials are available for bone regeneration. In an attempt to discover minimally invasive and cost effective treatments for cleft repair, an exceptional growth in synthetic biomedical graft materials have occurred. This study gives an overview of the use of dental materials to repair cleft of lip and palate (CLP). The eligibility criteria for this review were case studies, clinical trials and retrospective studies on the use of various types of dental materials in surgical repair of cleft palate defects. Any data available on the surgical interventions to repair alveolar or palatal cleft, with natural or synthetic graft materials was included in this review. Those datasets with long term clinical follow-up results were referred to as particularly relevant. The results provide encouraging evidence in favor of dental and other related biomedical materials to fill the gaps in clefts of lip and palate. The review presents the various bones and soft tissue replacement strategies currently used, tested or explored for the repair of cleft defects. There was little available data on the use of synthetic materials in cleft repair which was a limitation of this study. In conclusion although clinical trials on the use of synthetic materials are currently underway the uses of autologous implants are the preferred treatment methods to date.

  3. Invariant properties of representations under cleft extensions

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    The main aim of this paper is to give the invariant properties of representations of algebras under cleft extensions over a semisimple Hopf algebra. Firstly, we explain the concept of the cleft extension and give a relation between the cleft extension and the crossed product which is the approach we depend upon. Then, by making use of them, we prove that over an algebraically closed field k, for a finite dimensional Hopf algebra H which is semisimple as well as its dual H*, the representation type of an algebra is an invariant property under a finite dimensional H-cleft extension . In the other part, we still show that over an arbitrary field k, the Nakayama property of a k-algebra is also an invariant property under an H -cleft extension when the radical of the algebra is H-stable.

  4. Oral Clefts and Academic Performance in Adolescence

    DEFF Research Database (Denmark)

    Clausen, Nicola G; Pedersen, Dorthe A; Pedersen, Jacob K;

    2016-01-01

    OBJECTIVE:   Early life exposure to anesthesia and surgery is suspected to associate with cognitive impairment later in life. We compared academic achievement among adolescents with cleft lip only (CL), cleft palate only (CP), and cleft lip and cleft palate (CLP) with a noncleft control group to ......:   Oral cleft type rather than number and timing of anesthesia and operations associate to poorer academic performance. Although a potential neurotoxic effect due to anesthetic agents is not reflected in the data, it cannot be completely excluded.......-grade exam unavailable." Data adjusted for sex, birth weight, parental age, and parental level of education. RESULTS:   Compared to controls, children with CL achieved higher scores (mean difference 0.12, 95% CI -0.05; 0.29) and children with CLP presented with lower scores (mean difference -0.06, 95% CI -0...

  5. 探究口腔正畸对牙槽突植骨术唇腭裂患者疗效影响%Effect of Orthodontic Treatment on Alveolar Bone Grafting for Patients with Cleft Lip and Palate

    Institute of Scientific and Technical Information of China (English)

    秦裕东

    2015-01-01

    目的:观察并探讨在唇腭裂牙槽突植骨术后的正畸治疗对治疗效果的影响。方法随机选择2008年1月-2010年12月于该院接受牙槽突植骨术治疗的唇腭裂患者82例。实验组患者41例在接受牙槽突植骨术前均有显效时间以上的固定矫治器治疗史;接受手术后,对患者病情进行3~5年的随访观察,对患者实施适当矫正措施。41例对照组患者仅施以常规治疗。记录并比较两组患者术前、术后生活质量状况并对治疗效果进行评价。结果结果显示院对比两组生活质量的评分,治疗后,实验组精神状况、心理状况、活动能力、社会活动评分(分)分别为(78.2±3.1)、(82.2±4.2)、(78.8±4.8)、(82.1±3.9),明显优于对照组相应数据(70.7±2.6)、(73.8±1.8)、(68.1±2.5)、(72.6±2.6),差异有统计学意义(P<0.05);两组疗效对比,实验组治疗后SNA角、SNB角、ANB角数据(单位院度)分别为(82.6±1.3)、(76.2±1.6)、(2.7±1.8),明显优于对照组,差异有统计学意义(P<0.05)。结论口腔正畸在唇腭裂患者的手术治疗中能够有效提高治疗效果,提高患者的生活质量。%Objective To observe and investigate the effects of orthodontic treatment on alveolar bone grafting for patients with cleft lip and palate. Methods January 2008 - December 2010 82 cases in our hospital alveolar bone grafting in the treatment of cleft lip and palate patients. 41 patients in the experimental group before accepting the alveolar bone grafting were effective in the history of fixed appliance therapy over time; after surgery, the patient's condition for 3 to 5 years of follow-up observation of the patient to implement appropriate corrective measures. Only 41 cases in the control group of patients subjected to conventional treatment. Recorded and compared two groups of patients before surgery, the quality of the living conditions and treatment evaluation. Results The results showed that

  6. Cleft deformities in adults and children aged over six years in Nigeria: Reasons for late presentation and management challenges

    Directory of Open Access Journals (Sweden)

    Wasiu L Adeyemo

    2009-11-01

    Full Text Available Wasiu L Adeyemo1, Mobolanle O Ogunlewe1, Ibironke Desalu2, Akinola L Ladeinde1, Bolaji O Mofikoya3, Michael O Adeyemi4, Adegbenga A Adepoju4, Olufemi O Hassan41Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, 2Department of Anaesthesia, 3Department of Surgery, Faculty of Clinical Sciences, College of Medicine, University of Lagos, Lagos, Nigeria; 4Department of Oral and Maxillofacial Surgery, Lagos University Teaching Hospital, Lagos, NigeriaAbstract: In developing countries, untreated cleft lips and palates are found with increasing frequency and patients often present to the surgeon far past the optimal time for closure of the cleft deformities. A prospective study was conducted between March 2007 and September 2009, to identify the reasons and treatment challenges of delayed presentation of cleft lip and palate deformities at the Lagos University Teaching Hospital, Nigeria. Out of a total of 150 patients with cleft defects during the period, 43 (28.7% were adults and children aged over six years. The mean age of these patients at the time of presentation was 17.3 years. The most common reasons for late presentation were lack of money (56.7%, lack of health care services nearby (18.4%, and lack of awareness of treatment availability (13.3%. Common challenges in these patients included surgical, orthodontic, speech, anesthetic, and psychological. Although adult clefts were significantly enlarged in three dimensions the anatomic landmarks were easier to discern than in an infant. However, extensive soft tissue dissection in adult cleft lip repair resulted in significant postoperative edema. Closure of wide palatal cleft often required the use of adjunct intraoral flaps. Despite late presentation, surgical outcome of these patients was satisfactory and comparable to cleft repair in infants.Keywords: cleft deformities, adults, adolescents, late presentation, management, challenges

  7. X-linked genes and risk of orofacial clefts

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Skare, Øivind; Lie, Rolv T;

    2012-01-01

    Orofacial clefts are common birth defects of complex etiology, with an excess of males among babies with cleft lip and palate, and an excess of females among those with cleft palate only. Although genes on the X chromosome have been implicated in clefting, there has been no association analysis...

  8. Strong association of variants around FOXE1 and orofacial clefting

    NARCIS (Netherlands)

    Ludwig, K.U.; Bohmer, A.C.; Rubini, M.; Mossey, P.A.; Herms, S.; Nowak, S.; Reutter, H.; Alblas, M.A.; Lippke, B.; Barth, S.; Paredes-Zenteno, M.; Munoz-Jimenez, S.G.; Ortiz-Lopez, R.; Kreusch, T.; Hemprich, A.; Martini, M.; Braumann, B.; Jager, A.; Potzsch, B.; Molloy, A.; Peterlin, B.; Hoffmann, P.; Nothen, M.M.; Rojas-Martinez, A.; Knapp, M.; Steegers-Theunissen, R.P.M.; Mangold, E.

    2014-01-01

    Nonsyndromic orofacial clefting (nsOFC) is a common, complex congenital disorder. The most frequent forms are nonsyndromic cleft lip with or without cleft palate (nsCL/P) and nonsyndromic cleft palate only (nsCPO). Although they are generally considered distinct entities, a recent study has implicat

  9. Velopharyngeal sphincter pathophysiologic aspects in the in cleft palat

    Directory of Open Access Journals (Sweden)

    Collares, Marcus Vinicius Martins

    2008-09-01

    Full Text Available Introduction: Cleft lip and palate are common congenital abnormalities with typical functional disorders on speech, deglutition and middle ear function. Objective: This article reviews functional labiopalatine disorders through a pathophysiological view. Method: We performed a literature search on line, as well as books and periodicals related to velopharyngeal sphincter. Our sources were LILACS, MEDLINE and SciELO databases, and we applied to the research Keywords of interest on the velopharyngeal pathophysiology, for articles published between 1965 and 2007. Conclusion: Velopharyngeal sphincter plays a central role in speech, swallowing and middle ear physiology in patients with labiopalatine cleft. At the end of our bibliographic review, pursuant to the velopharyngeal physiology in individuals with this disorder in the functional speech, deglutition and otologic function, we observed that although there is a great number of published data discussing this issue, further studies are necessary to completely understand the pathophysiology, due to the fact they have been exploited superficially.

  10. Congenital orofacial clefts: Etiology and Frequency

    Directory of Open Access Journals (Sweden)

    Kamil Serkan Ağaçayak

    2014-06-01

    Full Text Available Orofacial clefts are congenital structural anomalies of the lip and/or palate. These anomalies affect ~1/1000 in the community. The etiology of orofacial clefts is complex, including various genetic and environmental agents. Syndrome is not accompanied by any isolated orofacial clefts are more common, although environmental factors often play a role in the etiology. Mendelian or teratogenic origins; the non-syndromic forms of orofacial clefts are more common and are likely due to secondary gene–environment interactions. Latest researches in both molecular and quantitative approaches have begun to identify the genes responsible for the rare syndromic forms of cleft and have also identified both candidate genes and loci for the more common and complex non-syndromic variants. Animal models, have also contributed greatly to an comprehension of these anomalies. We aimed to describes genes that are involved in orofacial clefts in humans and animal models and explores genetic approaches to identifying additional genes and gene–environment interactions that constitute the many factors of orofacial clefts in this review.

  11. Association of generalized aggressive periodontitis and ectrodactyly-ectodermal dysplasia-cleft syndrome

    Directory of Open Access Journals (Sweden)

    Rosamma Joseph

    2012-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly, ectodermal dysplasia, and facial clefting. Even though literature has documented the association of various genetic disorders with aggressive periodontitis, the periodontal manifestations in patients with EEC syndrome have never been addressed. This case report presents the periodontal status of three patients in a family with EEC syndrome. The presence of generalized aggressive periodontitis was noticed in these patients. EEC syndrome could be a new addition to the group of genetic disorders associated with aggressive periodontitis.

  12. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

    Science.gov (United States)

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts. PMID:25737931

  13. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    OpenAIRE

    Lovya George; Sunil K Jain

    2015-01-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require co...

  14. Implementation of standardized medical photography for cleft lip and palate audit.

    Science.gov (United States)

    Jones, Marie; Cadier, Michael

    2004-12-01

    Much has been published relating to the treatment and surgical outcome of cleft lip and palate disorders. Clinical audit is one of the most important tools for assessing the quality of care provided, with medical photography an invaluable component of this process. The Clinical Standards Advisory Group Report 1998 recommended that cleft lip and palate patients should be audited when 0 (under 1 year of age), and then at the ages of 5, 10, 15 and 20 years. For both audit and research purposes, medical photographs need to be accurate and of a consistently high standard. This paper describes the development of a standardized photographic protocol for cleft patients of audit age, to the benefit of both the multidisciplinary team and the patients.

  15. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    Science.gov (United States)

    George, Lovya; Jain, Sunil K.

    2015-01-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  16. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip–Cleft Palate

    Directory of Open Access Journals (Sweden)

    Lovya George

    2015-10-01

    Full Text Available Preterm infants (PIs often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure.

  17. Use of Biphasic Continuous Positive Airway Pressure in Premature Infant with Cleft Lip-Cleft Palate.

    Science.gov (United States)

    George, Lovya; Jain, Sunil K

    2015-10-01

    Preterm infants (PIs) often require respiratory support due to surfactant deficiency. Early weaning from mechanical ventilation to noninvasive respiratory support decreases ventilation-associated irreversible lung damage. This wean is particularly challenging in PIs with cleft lip and cleft palate due to anatomical difficulties encountered in maintaining an adequate seal for positive pressure ventilation. PI with a cleft lip and palate often fail noninvasive respiratory support and require continued intubation and mechanical ventilation. We are presenting the first case report of a PI with cleft lip and palate who was managed by biphasic nasal continuous positive airway pressure. PMID:26495158

  18. The effect of infant orthopedics on the occlusion of the deciduous dentition in children with complete unilateral cleft lip and palate (Dutchcleft).

    NARCIS (Netherlands)

    Bongaarts, C.A.M.; Kuijpers-Jagtman, A.M.; Hof, M.A. van 't; Prahl-Andersen, B.

    2004-01-01

    OBJECTIVE: Evaluation of the effect of infant orthopedics (IO) on the occlusion of the deciduous dentition in patients with unilateral cleft lip and palate (UCLP). DESIGN: Prospective, two-arm, randomized, controlled clinical trial with three participating cleft palate centers (Dutchcleft). SETTING:

  19. Dental arch relationship in children with complete unilateral cleft lip and palate following warsaw (one-stage repair) and oslo protocols.

    NARCIS (Netherlands)

    Fudalej, P.S.; Hortis-Dzierzbicka, M.; Dudkiewicz, Z.; Semb, G.

    2009-01-01

    OBJECTIVE: To compare the dental arch relationship following one-stage repair of unilateral cleft lip and palate (UCLP) in Warsaw with a matched sample of patients treated by the Oslo Cleft Team. MATERIAL: Study models of 61 children (mean age, 11.2; SD, 1.7) with a nonsyndromic complete UCLP consec

  20. Comparison of the Pharyngeal Airway Volume between Non-Syndromic Unilateral Cleft Palate and Normal Individuals Using Cone Beam Computed Tomography

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    Shoaleh Shahidi

    2016-09-01

    Full Text Available Statement of the Problem: Individuals with cleft lip and cleft palate mostly have airway problems. Introduction of cone beam computed tomography (CBCT and imaging software has provided the opportunity for a more precisely evaluating 3D volume of the airway. Purpose: The purpose of this study was to analyze and compare 3D the pharyngeal airway volumes of cleft palate patients with normal individuals using CBCT. Materials and Method: 30 complete cleft palate patients were selected from the Department of Orthodontics; Dental University (Shiraz, Iran who had CBCT scans of the head. The control group included 30 individuals with Class I angle occlusion who were matched for age and gender with the experimental group. ITK-SNAP 2.4.0 PC software was used to build 3D models of the airways for the subjects and measuring airway volumes. The statistical analyses were performed using SPSS software (version 19. Mann-Whitney test was adopted with p< 0.05 as statistical significance. Results: The average volume of the pharyngeal airway of cleft group was 18.6 cm3, with mean volumes of 6.8 cm3 for the superior component and 11.3 cm3 for the inferior component. The total and superior airway volume of cleft group were significantly lower than non-cleft groups (p= 0.008, p= 0.00, respectively but the inferior airway volumes were not significantly different between the cleft and non-cleft groups. There was a significant and positive correlation between superior airway volume and inferior airway volume in cleft palate patients (r=+0.786, p< 0.001 and control group (r=+0.575, p= 0.001. Conclusion: 3D analysis showed that the nasal and total airway was restricted in individuals with cleft palate but the inferior airway was not compromised in these individuals. This would be a crucial data to be considered for surgeons during surgical planning.

  1. Congenital hypothyroidism, spiky hair, and cleft palate.

    OpenAIRE

    Bamforth, J S; Hughes, I A; Lazarus, J. H.; Weaver, C. M.; P.S. Harper

    1989-01-01

    Two brothers are described with athyroidal hypothyroidism, spiky hair, choanal atresia, cleft palate, and bifid epiglottis. Polyhydramnios was present in the third trimester of each pregnancy. These abnormalities appear to represent a new syndrome.

  2. Current concepts in genetics of nonsyndromic clefts

    Directory of Open Access Journals (Sweden)

    Murthy Jyotsna

    2009-01-01

    Full Text Available Nonsyndromic cleft lip and palate is a complex genetic disorder with variable phenotype, largely attributed to the interactions of the environment and multiple genes, each potentially having certain effects. Numerous genes have been reported in studies demonstrating associations and/or linkage of the cleft lip and palate phenotypes to alleles of microsatellite markers and single nucleotide polymorphisms within specific genes that regulate transcription factors, growth factors, cell signalling and detoxification metabolisms. Although the studies reporting these observations are compelling, most of them lack statistical power. This review compiles the evidence that supports linkage and associations to the various genetic loci and candidate genes. Whereas significant progress has been made in the field of cleft lip and palate genetics in the past decade, the role of the genes and genetic variations within the numerous candidate genes that have been found to associate with the expression of the orofacial cleft phenotype remain to be determined.

  3. Technique in Cleft Rhinoplasty: The Foundation Graft.

    Science.gov (United States)

    Gassner, Holger G; Schwan, Franziska; Haubner, Frank; Suárez, Gustavo A; Vielsmeier, Veronika

    2016-04-01

    Secondary cleft rhinoplasty represents a particular surgical challenge. The authors have identified the deficit in skeletal projection of the cleft-sided piriform rim as an important contributor to the pathology. A graft is described to augment the piriform crest on the cleft side. This foundation graft is suture fixated to the piriform crest after complete release of all soft tissue attachments to the alar base. The foundation graft is articulated with a long alar strut graft, which allows for powerful projection of the cleft-sided nasal tip. An advancement flap of vestibular skin is described to correct the vestibular stenosis. A transplant of diced cartilage in fascia is added to augment maxillary soft tissue volume. Subjective and objective measures of form and function are presented in a retrospective series of five cases, illustrating the efficacy of the techniques described. PMID:27097143

  4. Hidrosefali Ile Ilişkili Ektrodakli, Ektodermal Displazi, Yarık Dudak Ve Damak Sendromulu Bir Vakanın Değerlendirmesi

    OpenAIRE

    Aladağ, Buket Uysal; Yılmaz, Fatma Hilal; Koçak, Nadir; Annagür, Ali

    2013-01-01

    Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC) is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate). A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin...

  5. Effect of cleft palate repair on the susceptibility to contraction-induced injury of single permeabilized muscle fibers from congenitally-clefted goat palates.

    Science.gov (United States)

    Despite cleft palate repair, velopharyngeal competence is not achieved in ~ 15% of patients, often necessitating secondary surgical correction. Velopharyngeal competence postrepair may require the conversion of levator veli palatini muscle fibers from injury-susceptible type 2 fibers to injury-resi...

  6. [Laser navigation guided cleft lip repair].

    Science.gov (United States)

    Bing, Shi

    2016-06-01

    A new method using the ideal mid-facial line as the navigating reference was introduced to improve the outcome of cleft lip repair. Using the verticle coordinate crossing the middle point of the intercanthus line, surgeons could observe and correct the distortion of the fine structures in labial-nasal area. This laser projecting mid-facial-line navigation was repeatable, while not interfere the operating. In conclusion, generalizing laser navigation is a valuable supplementary for cleft lip repair. PMID:27526442

  7. Three-Dimensional Assessment of Early Surgical Outcome in Repaired Unilateral Cleft Lip and Palate: Part 1. Nasal changes.

    LENUS (Irish Health Repository)

    Ayoub, Ashraf

    2010-08-11

    Abstract Objective: To evaluate 3D nasal morphology following primary reconstruction in children with unilateral cleft lip and palate relative to contemporaneous non-cleft data Design: Prospective, cross-sectional, controlled study. Setting: Glasgow Dental Hospital & School, Faculty of Medicine, Glasgow University Patients and Participants: Two groups of 3-year old children (21 with unilateral cleft lip and palate and 96 controls) with facial images taken using a 3D vision based capture technique. Methods: 3D images of the face were reflected so the cleft was on the left side to create a homogenous group for statistical analysis. Three-dimensional co-ordinates of anthropometric landmarks were extracted from facial images by a single operator. A set of linear measurements was utilised to compare cleft and control subjects on right and left sides, adjusting for sex differences Results: the mean nasal base width and the width of the nostril floor on right and left sides differed significantly between control and Unilateral Cleft Lip and Palate (UCLP) groups. The measurements were greater in UCLP children. The difference in the mean nasal height and mean nasal projection between the groups were not statistically significant. Mean columellar lengths were different between the left and right sides in UCLP cases. Conclusions: There were significant nasal deformities following the surgical repair of UCLP. Keywords: child, cleft lip and palate, three-dimensional imaging.

  8. Analysis on the curative effect of orthodontic and orthognathic treatment for patients with dento-maxillofacial defor-mities after cleft lip and palate operation%关于正畸正颌联合治疗唇腭裂术后牙颌面畸形的疗效分析

    Institute of Scientific and Technical Information of China (English)

    倪国宇

    2016-01-01

    Objective:To observe and investigate the application effect of orthodontic and orthognathic treatment for patients with dento-maxillofacial deformities after cleft lip and palate operation.Methods:80 patients with dento-maxillofacial deformities after cleft lip and palate operation were selected.They were randomly divided into the two groups.The experimental group was treated with orthodontic and orthognathic treatment,while the control group was treated with orthodontic treatment.We observed the effect of the two groups.Results:In the experimental group,the restoration of dento-maxillofacial deformities was good;the teeth occlusal relationship improved;chewing function was also improved;deformity recurrence rate was significantly decreased.Conclusion:The effect of orthodontic and orthognathic treatment for patients with maxillofacial deformities after cleft lip and palate operation is significant.%目的:观察和探讨正畸正颌联合手术在唇腭裂术后的牙颌面畸形治疗中的应用效果。方法:收治唇腭裂术后牙颌面畸形的患者80例,随机平分为两组,试验组采取正畸正颌联合治疗,对照组采用正颌手术行矫正治疗,观察两组效果。结果:试验组的牙颌面畸形恢复状况良好,牙齿咬合关系改善,咀嚼功能提升,畸形复发率明显降低。结论:正畸正颌联合手术在治疗唇腭裂术后牙颌面畸形中效果显著。

  9. Retrospective subjective evaluation of aesthetic outcome in secondary cleft lip deformities operated with Abbe's flap

    Directory of Open Access Journals (Sweden)

    Kamal M. Kumar

    2015-01-01

    Full Text Available Background: The Abbe's flap is most commonly used to repair the full thickness defects of the lip that do not involve the commissure. The Abbe flap was first introduced to correct the secondary deformity of bilateral cleft lip. By this pedicled flap, we are able to equalize the disparity which existed between the tight upper lip and excessive lower lip. Aims to study retrospective subjective evaluation of aesthetic outcome in secondary cleft lip deformities operated with Abbe's flap. Methods: 29 patients operated during the period of January 2007 - December 2011 for correction of secondary cleft lip and nasal deformity with Abbe's flap with or without rhinoplasty were included in our retrospective study. The secondary corrective surgeries were performed by a single surgeon. The photographs of the patients were retrospectively assessed by two plastic surgeons and one oral and maxillofacial surgeon, other than the one who performed the surgery. 29 patients operated during the period of January 2007 - December 2011 for correction of secondary cleft lip and nasal deformity with Abbe's flap with or without rhinoplasty were included in our retrospective study. The secondary corrective surgeries were performed by a single surgeon. The photographs of the patients were retrospectively assessed by two plastic surgeons and one oral and maxillofacial surgeon, other than the one who performed the surgery. Results: Out of 29 patients with secondary cleft deformities 12 were male and 17 were female. Average follow up period was 3 years. Multiple variables of the nose and the lips were used to assess the aesthetic outcome of Abbe's flap. Conclusion: As per the subjective retrospective analysis of our study we have come to the conclusion that Abbe's flap is a work horse for correction of shortage of tissue for the secondary cleft lip deformities. Uniform satisfactory outcome was obtained with a more natural contour and more satisfactory proportion of both lips

  10. New trends in the complex treatment in the Cleft Centre in Bratislava.

    Science.gov (United States)

    Kokavec, R; Hedera, J; Fedeles, J; Janovic, J; Kratka, E; Klimova, I

    2001-01-01

    The last decade of the second millennium has brought some major changes into the concept of comprehensive treatment of the cleft lip and palate patients commonly accepted by the cleft center in Bratislava. Important events occurred, which surely had and in future they probably still would have an important impact on the comprehensive medical care of children with cleft lip and palate. There is beyond any doubt that an ongoing application of new trends in such fields as plastic surgery, anesthesiology, maxillofacial surgery, orthodontics, phonetics, speech therapy, paediatrics, human genetics or teratology will contribute to the progress and improvement of functional and aesthetic results and to better social adaptation of the cleft lip and palate patients. The study focuses on the following issues: cleft incidence, timing of the primary surgical repair, as well as the need of secondary operations (closures of communications, bone grafts, pharyngeal flaps, corrections of the lip and nose) and the achieved standard of speech quality and articulation, as well as on the early and late otological states and phonation. (Tab. 7, Fig. 3, Ref. 8.)

  11. Dental and maxillofacial characteristics of six Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome.

    Science.gov (United States)

    Okamura, Erika; Suda, Naoto; Baba, Yoshiyuki; Fukuoka, Hiroki; Ogawa, Takuya; Ohkuma, Mizue; Ahiko, Nozomi; Yasue, Akihiro; Tengan, Toshimoto; Shiga, Momotoshi; Tsuji, Michiko; Moriyama, Keiji

    2013-03-01

    Objective : Ectrodactyly-ectodermal dysplasia-clefting syndrome is a congenital anomaly characterized by ectodermal dysplasia, ectrodactyly, cleft lip and palate, and lacrimal duct anomalies. Because this syndrome is frequently accompanied by a congenital lack of teeth, narrow palate, and malocclusion, comprehensive orthodontic intervention is required. Design : To highlight the specific dental and maxillofacial characteristics of ectrodactyly-ectodermal dysplasia-clefting syndrome, six Japanese individuals diagnosed with the syndrome are described here. Patients : The subjects consisted of two boys and four girls (age range, 6.0 to 13.9 years) diagnosed with ectrodactyly-ectodermal dysplasia-clefting syndrome by medical and dental specialists. Their conditions included ectodermal dysplasia (hypodontia, microdontia, enamel hypoplasia, and abnormalities in hair and nails), cleft lip and/or palate, and ectrodactyly. Cephalograms, panoramic x-rays, and dental casts were taken; systemic complications were recorded at the first visit to our dental hospital. Results : All individuals had severe oligodontia with 9 to 18 missing teeth. The missing teeth were mainly maxillary and mandibular incisors and second bicuspids, arranged in a symmetrical manner. Cephalometric analysis showed retruded and short maxilla due to cleft lip and/or palate. It is interesting that all individuals showed a characteristically shaped mandibular symphysis with a retruded point B. It is likely that this unusual symphyseal morphology is due to the lack of mandibular incisors. Conclusions : This study demonstrates the presence of severe oligodontia in the incisal and premolar regions and describes a characteristic maxillary and mandibular structure in Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting syndrome. PMID:22236363

  12. Lack of association between IRF6 polymorphisms and nonsyndromic oral clefts in South Indian population

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    Venkatesh Babu Gurramkonda

    2013-08-01

    Full Text Available Objective: This present study is aimed to investigate the association between interferon regulatory factor 6 (IRF6, single nucleotide polymorphisms (SNPs, and nonsyndromic cleft lip without without cleft palate (NSCLP in the South Indian population. Subject and Methods: For this study, 190 unrelated NSCLP patients and 189 controls without clefts were genotyped with rs2235371 (V2741 and rs642961 SNPs using PCR-RFLP. The associations between NSCLP groups and IRF6 gene polymorphisms, as well as haplotypes, were analyzed using chi-squared test and 95% confidence interval (95%CI of the odds ratios were calculated with the control groups as reference. Results: For controls, the minor allele frequencies of both variants, V2741 and rs642961, were 7.1% and 21.1%, respectively. Genotype data for both variants in control and cleft groups follow the Hardy Weinberg Equilibrium. Between cases with NSCLP and controls, the two SNPs showed no differences in frequencies of the genotypes or alleles. The pairwise linkage disequilibrium (LD values (D’=1 and r2=0.027 between V2741 and rs642961 revealed that these two SNPs are not in strong LD. Haplotype G-T showed a significantly reduced risk for oral clefts (p<0.001 and haplotype A-T increased the risk for oral clefts (p=0.043. Gene-gene interaction showed that the higher risk group contains more GG-CC combination of cases that the controls, but this model was not significantly associated with cleft status (p=0.136 Conclusion: In conclusion, while IRF6 is strongly associated in other populations, this study demonstrated that variants in IRF6 may play a role in NSCLP in a South Indian population, but other genes are expected to play a role in this population as well.

  13. Roles of Medical Record and Statistic Staff on Research at the Tawanchai Center.

    Science.gov (United States)

    Pattaranit, Rumpan; Chantachum, Vasana; Lekboonyasin, Orathai; Pradubwong, Suteera

    2015-08-01

    The medical record and statistic staffs play a crucial role behind the achievements of treatment and research of physicians, nurses and other health care professionals. The medical record and statistic staff are in charge of keeping patient medical records; creating databases; presenting information; sorting patient's information; providing patient medical records and related information for various medical teams and researchers; Besides, the medical record and statistic staff have collaboration with the Center of Cleft Lip-Palate, Khon Kaen University in association with the Tawanchai Project. The Tawanchai Center is an organization, involving multidisciplinary team which aims to continuing provide care for patients with cleft lip and palate and craniofacial deformities who need a long term of treatment since newborns until the age of 19 years. With support and encouragement from the Tawanchai team, the medical record and statistic staff have involved in research under the Tawanchai Centre since then and produced a number of publications locally and internationally.

  14. Evaluation of the lesions of synovial-lined clefts in the Hoffa's infrapatella fat pad with MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Min Sun; Park, Jin Gyoon; Kim, Young Cheol; Hwang, Eai Hong; Joo, Jung Hyun; Chung, Tae Woong; Kang, Heoung Keun [Chonnam Univ., College of Medicine, Kwangju (Korea, Republic of); Oh, Hee Yeon [Namkwang Hospital, Kwangju (Korea, Republic of)

    1998-09-01

    Within Hoffa's infrapatellar fat pad there are two synovial clefts, horizontal and vertical, which communicate with the intra-articular space. Intra-articular lesions can also occur in these clefts, and are often difficult to differentiate from extra-articular lesions. The purpose of this study is to evaluate, using MR imaging, the lesions occurring in these synovial lined clefts, as well as associated abnormalities. Thirty-one knees of 31 patients with lesions in horizontal and vertical clefts in Hoffa's infrapatellar fat pad were retrospectively evaluated. Using a 1.5T MR imager, axial, sagittal, and coronal MR images of knees were obtained. Lesions in clefts, degree of joint effusion and associated knee abnormalities were analyzed. Horizontal cleft lesions were noted in 21 cases; there were 17 cystic dilatations, two loose bodies, one synovial chondroma, and one case of pigmented villonodular synovitis(PVNS). Vertical cleft lesions were noted in 15 cases; these comprised 11 cystic dilatations, two loose bodies, one synovial osteochondromatosis, and one PVNS. Among all cases, three cystic dilatations, one loose body, and one PVNS occurred in both horizontal and vertical clefts. Among 25 knees with cystic dilatations of clefts, five showed grade 1, joint effusion, ten grade 2, and ten grade 3, Associated abnormalities were meniscal tear in 16 cases, osteoarthritis in 13, cruciate ligament tear in five, osteochondritis dissecans in three, osteochondral fracture in two, osteonecrosis in one, loose body in one, and synovitis in one. Among synovial-lined clefts in Hoffa's fat pad, the most common lesion was cystic dilatation;there were various associated abnormalities and a close relationship to joint effusion. An awareness of the types of lesions found in clefts is helpful for narrowing the differential diagnosis of lesions occurring in the area of Hoffa's fat pad.=20.

  15. Straight line repair of unilateral cleft lip: new operative method based on 25 years experience.

    Science.gov (United States)

    Nakajima, T; Tamada, I; Miyamoto, J; Nagasao, T; Hikosaka, M

    2008-08-01

    The resultant scar in the primary repair of unilateral cleft lip should ideally be straight and the mirror image of the philtrum on the non-cleft side. In 1993, we reported a new operative technique for unilateral cleft lip, in which we designed a straight line for the incision on the white lip. In order to produce the nostril floor, we used the white lip tissue in the area between the alar base and alveolus at the cleft side as a flap. We also used a small triangular flap above the white skin roll to prevent Cupid's peak from being drawn up. Unlike the rotation-advancement method, our technique does not leave a transverse scar at the alar base. Instead, it leaves a scar only along the line coincident with the natural philtral ridge. However, during observations of our patients, we noticed that the small triangular flap designed to be 1.5mm tended to become a conspicuous angular scar as the patients grew older. In addition, drooping of Cupid's peak on the cleft side was often observed with this small triangular flap. To make it less conspicuous, we made some modifications to the small flap above the white skin roll. With this new technique, we designed a semi-circular flap (1.5 x 3mm) above the white skin roll, instead of the small triangular flap. The suture line of our refined procedure draws a gentle curve, which looks almost straight because of skin elasticity. Moreover, the semi-circular flap causes less drooping of the upper lip than the triangular flap. We believe that revising the shape of the small flap on the white skin roll greatly improves patients' appearance. In this report, we present our refined techniques of primary repair of unilateral cleft lip.

  16. MAXILLARY GROWTH PATTERNS IN ROMANIAN CHILDREN WITH CLEFT PALATE AGED BETWEEN 0-6 YEARS

    Directory of Open Access Journals (Sweden)

    Liliana-Gabriela Halitchi

    2011-12-01

    Full Text Available A study documenting spontaneous and coordinated growth changes in young Romanian children with cleft palate is developed, for identifying the functional adaptations of oral cavity to the disrupted architecture of palate in children with cleft palate, the 3D analysis of maxillary casts being applied to visualize, evaluate and measure maxillary growth in patients with cleft palate, as well as to identify the reactive morphological pattern that could be imagined from the dimensional changes produced in the first 6 years of life. Two experimental groups were involved in the study, a former one, including 44 patients with cleft palate (16 boys and 28 girls, aged between 2 months and 6 years, operated by the same surgical team in the St.Mary University Children Hospital from Ia[i. The resulting maxillary casts were 3D scanned at Multinr company, in Sf. Gheorghe. The maxillary alveolar arch lines, the 4 Stilmann points (TD, TS, CD, CS and the maxillary interincisive point were identified and marked by the orthodontist, being reproduced with 6 degrees of tridimensional freedom. The coordinates of this point were transferred to the CAD system, to graphically recreate the computerized three-dimensional alveolar arch. 4 direct measurements have been made, namely: anterior and posterior diameter of the alveolar maxillary arch, length of the alveolar arch and depth of the palate. Student’s t test, both simple and bivariate, performed with SPSS 13.0 soft, showed statistic significance for the dimensional differences of the alveolar maxillary arch in children with cleft palate, comparatively with the healthy control group. Conclusion: Cleft palate patients have narrower and shorter maxillary alveolar arch and a flattened palate.

  17. White Roll Vermilion turn down flap in primary unilateral cleft lip repair: A novel approach

    Directory of Open Access Journals (Sweden)

    R K Mishra

    2015-01-01

    Full Text Available Aim: Numerous modifications of Millard′s technique of rotation - advancement repair have been described in literature. This article envisions a new modification in Millard′s technique of primary unilateral chieloplasty. Material and Methods: Eliminating or reducing the secondary deformities in children with cleft lip has been a motivating factor for the continual refinement of cleft lip surgical techniques through the years. Vermilion notching, visibility of paramedian scars and scar contracture along the white roll are quite noticeable in close-up view even in good repairs. Any scar is less noticeable if it is in midline or along the lines of embryological closure. White Roll Vermilion turn down Flap (WRV Flap, a modification in the Millard′s repair is an attempt to prevent these secondary deformities during the primary cleft lip sugery. This entails the use of white roll and the vermilion from the lateral lip segment for augmenting the medial lip vermilion with the final scar in midline at the vermilion. Result: With an experience of more than 100 cases of primary cleft lip repair with this technique, we have achieved a good symmetry and peaking of cupid′s bow with no vermilion notching of the lips. Conclusion: WRV flap aims to high light the importance of achieving a near normal look of the cleft patient with the only drawback of associated learning curve with this technique.

  18. Evaluation of fecal microorganisms of children with cleft palate before and after palatoplasty

    Directory of Open Access Journals (Sweden)

    Narciso Almeida Vieira

    2013-09-01

    Full Text Available This study isolated and quantified intestinal bacteria of children with cleft palate before and after palatoplasty. A prospective study was conducted from May 2007 to September 2008 on 18 children with cleft palate, aged one to four years, of both genders, attending a tertiary cleft center in Brazil for palatoplasty, to analyze the effect of surgical palate repair on the concentration of anaerobes Bacteroides sp, Bifidobacterium sp and microaerophiles Lactobacillus sp in feces of infants with cleft palate before and 24 hours after treatment with cefazolin for palatoplasty. There was significant reduction of Lactobacillus sp (p < 0.002, Bacteroides sp (p < 0.001 and Bifidobacterium sp (p = 0.021 after palatoplasty, revealing that surgery and utilization of cefazolin significantly influenced the fecal microbiota comparing collections before and after surgery. However, due to study limitations, it was not possible to conclude that other isolated factors, such as surgical stress, anesthetics and other medications used in palatoplasty might have a significant influence on the microbiota. Considering the important participation of the intestinal microbiota on both local and systemic metabolic and immunological activities of the host, professionals should be attentive to the possible influence of these changes in patients submitted to cleft repair.

  19. Lexical selectivity in Danish toddlers with cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth

    2013-01-01

    Objective: To study if Danish toddlers with cleft palate display lexical selectivity in their early lexicon at 18 months of age. Design: A cross-sectional study. Participants: Thirty-four children with unilateral cleft lip and palate and 35 children without cleft palate, matched for gender and age...... productions establishing an observed productive vocabulary size for each participant. Results: At 18 months of age Danish toddlers with cleft palate showed marked lexical selectivity in their early words. The distribution of consonant classes observed at 11 months of age in a previous study of the children...... with cleft palate was almost perfectly reflected in their early lexicon at 18 months. The early lexicon of children with cleft palate differed from the early lexicon of their non cleft peers. Conclusions & Implications: Danish toddlers with cleft palate display lexical selectivity in the early lexicon...

  20. Dermatoglyphic peculiarities in children with oral clefts

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    Mathew L

    2005-01-01

    Full Text Available In humans, the development of the primary palate and the lip is completed by the 7th week of intra uterine life and that of secondary palate by 12th week. The dermal ridges develop in relation to the volar pads, which are formed by the 6th week of gestation and reach maximum size between 12th and 13th weeks. This means that the genetic message contained in the genome - normal or abnormal is deciphered during this period and is also reflected by dermatoglyphics.Hence this study was done in order to observe the differences in dermatoglyphic patterns between the children with oral clefts and normal children and to determine the usefulness of dermatoglyphics in studying the genetic etiology of oral clefts.Dermatoglyphic data from 50 oral cleft children and 50 normal children were collected using the ink method and comparison was done between them. In the present study, we found an increase in the ulnar loop patterns on the distal phalanges of the ten fingers, an increase in the atd angle and an increase in the fluctuating asymmetry of the atd angle in the oral cleft children which indicates the degree of developmental instability of the oral cleft individual.

  1. Notch-free vermillion after unilateral cleft lip repair: The Charles Pinto centre protocol

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    Narayanan P

    2008-01-01

    Full Text Available A notch on the vermillion is one of the most common complications following the repair of a unilateral cleft lip. Several methods have been described for the secondary correction of a notch. However, there are only a few reports on how the notch can be prevented during primary lip repair. Causes of a vermillion notch were analysed at the Charles Pinto Centre for Cleft Lip and Palate and each possible cause addressed by an appropriate procedure. This protocol was then followed in every patient. In this manner, we have been able to avoid notches in unilateral cleft lips altogether and more significantly, junior trainees in our department have also been able to consistently avoid a notch in their repairs.

  2. Primary unilateral and bilateral cleft lip and nose in an older population.

    Science.gov (United States)

    Rai, Kimit

    2005-01-01

    A one-stage procedure to reconstruct complete and incomplete unilateral/bilateral cleft lip and nose deformities is presented. Emphasis was made on closure of the lip muscles, correction of the nostril floor, correction of the alveolar cleft as well as reconstruction of the nose through an intranasal approach, with a supported suture technique for nasal correction. No dental or orthodontic treatment was available or performed in this older population. Emphasis was on primary closure of the muscles, using the rotation advancement principle. The repair that was performed was near anatomical, reconstructing the labial sulcus, the nostril floor, the alveolar cleft and the nasal deformity all in one stage. There was a high level of satisfaction both from the patient's and surgeon's point of view.

  3. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    OpenAIRE

    Reema Sharma Dhar; Amitava Bora

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and ro...

  4. Optimization of dental status improves long-term outcome after alveolar bone grafting in unilateral cleft lip and palate.

    Science.gov (United States)

    Jabbari, Fatima; Skoog, Valdemar; Reiser, Eicka; Hakelius, Malin; Nowinski, Daniel

    2015-03-01

    Objective : To evaluate the importance of dental status for long-term outcome after alveolar bone grafting in patients with unilateral cleft lip and palate. Design : Retrospective longitudinal study. Setting : Cleft lip and palate-craniofacial center, Uppsala University Hospital, Sweden. Patients : A total of 67 consecutive patients with unilateral complete cleft lip and palate. Interventions : Secondary alveolar bone grafting, prior to the eruption of the permanent canine, was performed at the average age of 10.0 years (range, 8.5 to 12.0 years). Main Outcome Measures : Alveolar bone height was evaluated with the modified Bergland index at 1 and 10 years after surgery. Results : Of the patients, 97% had modified Bergland index grade I and the remaining 3% had modified Bergland index grade II at 1 year after surgery. At 10 years' follow-up, 43% showed modified Bergland index grade I; 55%, modified Bergland index grade II; and 2% (one patient), modified Bergland index grade III. The degree of dental anomalies in the cleft area, such as enamel hypoplasia, incisor rotation, incisor inclination, canine inclination, and oral hygiene registered preoperatively, all correlated negatively to the modified Bergland index at 10 years after surgery. Enamel hypoplasia (ρ = 0.70195, P < .0001), followed by canine inclination (ρ = 0.55429, P < .0001), showed the strongest correlation to reduced bone height in the cleft area. Conclusions : In patients with unilateral cleft lip and palate, excellent results from secondary alveolar bone grafting in terms of bone height in the alveolar cleft tend to decrease with time. This seems to be correlated with factors that might to some extent be treated preoperatively through adequate planning and execution of the orthodontic treatment. PMID:24568558

  5. Reconstruction during phase II of unilateral cleft lip improving function of musculus orbicularis oris%单侧唇裂Ⅱ期整复改善口轮匝肌功能

    Institute of Scientific and Technical Information of China (English)

    马新亮; 陈传俊; 韩立显

    2002-01-01

    @@ Background:Nasolabial deformity secondary to operation of cleft lip seriously affect patients' physiological and psychological function.Plasticity or reposition of musculus orbicularis oris received more attention in recent years.Since 1995,nasolabial deformity secondary to operation of unilateral complete cleft lip was reduced in 17 patients,and therapeutic effect is satisfying.

  6. Histological comparison of the alar nasal cartilages in unilateral cleft lip

    Directory of Open Access Journals (Sweden)

    Modolin Miguel

    2002-01-01

    Full Text Available Patients with unilateral cleft lip display characteristic nasal changes that are independent of the degree of deformity. Defenders of the intrinsic theory consider these deformities to be due to embryogenic alterations of the alar nasal cartilages. Those that propose the extrinsic theory defend the thesis that the deformity is due to disorganization of the perioral muscles deformed by the cleft. The purpose of this study is to contribute histological evidence to help clarify the issue. PATIENTS AND METHODS: Specimens of the lateral portion of both the healthy and the cleft side of the alar cartilages were obtained from 18 patients. These uniformly cut specimens were stained by hematoxylin and eosin. Samples from 2 patients were excluded due to imperfections. The same pathologist examined all the slides. He was unaware of the origins of the specimens; he counted the number of chondrocytes and quantified the cartilage matrixes. RESULTS: All data was analyzed statistically, and no significant statistical differences were apparent, either in the number of chondrocytes or the cartilage matrix between the healthy side and the cleft side. DISCUSSION: These results apparently support the group that defend the extrinsic theory; nevertheless, the doubt about the composition of the cartilage matrix remains, not only concerning the glycosaminoglycans that compose them, but also regarding elastin and collagen and its linkages that can cause different degrees of collagen consistency.

  7. Weight Gain in Children with Cleft Lip and Palate without Use of Palatal Plates

    Directory of Open Access Journals (Sweden)

    Renato da Silva Freitas

    2012-01-01

    Full Text Available Goals/Background. To evaluate children’s growth in the first year of life, who have cleft palate and lip, without the use of palatal plates. Materials/Method. Chart review was conducted, retrospectively, in the Center for Integral Assistance of Cleft Lip and Palate (CAIF, in Brazil, between 2008 and 2009. Results for both genders were compared to the data published by the World Health Organization (WHO regarding average weight gain in children during their first year of life. Results. Patients with syndromic diagnosis and with cleft classified as preforamen were excluded, resulting in a final number of 112 patients: 56 male and 56 female. Similar patterns were seen comparing the two genders. Although it was observed weight gain below the average until the 11th month in male patients and until 9 months in female patients, both genders remained at the 50th percentile (p50 and improved after the 4th month of age for boys and the 9th month of age for girls. Conclusion. Children with cleft palate weigh less than regular children during their first months of life. At the end of the first year, weight gain is similar comparing normal and affected children. However, factors that optimized weight gain included choosing the best treatment for each case, proper guidance, and multiprofessional integrated care.

  8. Reliability of linear measurements on a virtual bilateral cleft lip and palate model

    NARCIS (Netherlands)

    Oosterkamp, B.C.M.; van der Meer, W.J.; Rutenfrans, M.; Dijkstra, P.U.

    2006-01-01

    Objective: To assess the reliability and validity of measurements performed on three-dimensional virtual models of neonatal bilateral cleft lip and palate patients, compared with measurements performed on plaster cast models. Materials and Methods: Ten high-quality plaster cast models of bilateral c

  9. An autosomal recessive syndrome of cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE).

    Science.gov (United States)

    Giannotti, A; Digilio, M C; Mingarelli, R; Dallapiccola, B

    1995-01-01

    We report a brother and sister affected by a constellation of malformations, including cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE). A similar association has been reported previously by Richieri-Costa and Orquizas in a male patient born to consanguineous parents. An autosomal recessive pattern of inheritance is proposed for this syndrome. Images PMID:7897634

  10. Expanding the phenotypic spectrum of Lenz-Majewski syndrome: facial palsy, cleft palate and hydrocephalus.

    Science.gov (United States)

    Wattanasirichaigoon, Duangrurdee; Visudtibhan, Anannit; Jaovisidha, Suphaneewan; Laothamatas, Jiraporn; Chunharas, Amornsri

    2004-07-01

    We report a sporadic case of Lenz-Majewski syndrome (LMS) with newly recognized manifestations including facial palsy, cleft palate and hydrocephalus developing later in infancy. The clinical course of the patient and neuroimaging studies are described. Increased intracranial pressure was recognized and treated early with the aim of preventing neurological morbidity.

  11. Primary septoplasty in the repair of unilateral complete cleft lip and palate

    NARCIS (Netherlands)

    Gosla Reddy, S.; Nagy, K.; Mommaerts, M.Y.; Reddy, R.R.; Bronkhorst, E.M.; Prasad, R.; Kuijpers-Jagtman, A.M.; Berge, S.J.

    2011-01-01

    BACKGROUND: The purpose of this study was to assess and compare nasal symmetry in patients who underwent correction of a complete unilateral cleft lip using the Afroze incision without and with primary septoplasty using a standardized two-dimensional photographic analysis. METHODS: A prospective coh

  12. An autosomal recessive syndrome of cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE).

    OpenAIRE

    Giannotti, A; Digilio, M C; Mingarelli, R; Dallapiccola, B.

    1995-01-01

    We report a brother and sister affected by a constellation of malformations, including cleft palate, cardiac defect, genital anomalies, and ectrodactyly (CCGE). A similar association has been reported previously by Richieri-Costa and Orquizas in a male patient born to consanguineous parents. An autosomal recessive pattern of inheritance is proposed for this syndrome.

  13. Craniofacial Morphology and Growth Comparisons in Children With Robin Sequence, Isolated Cleft Palate, and Unilateral Complete Cleft Lip and Palate

    DEFF Research Database (Denmark)

    Hermann, N. V.; Kreiborg, S.; Darvann, Tron Andre;

    2003-01-01

    Objective: Comparison of early craniofacial morphology and growth in children with nonsyndromic Robin Sequence (RS), isolated cleft palate (ICP), and unilateral complete cleft lip and palate (UCCLP). Subjects: One hundred eight children with cleft: 7 with RS, 53 with ICP, and 48 with UCCLP were...

  14. The Fetal Cleft palate: V. Elucidation of the Mechanism of Palatal Clefting in the Congenital Caprine Model

    Science.gov (United States)

    Maternal ingestion of Nicotiana glauca from gestation days 32 through 41 results in a high incidence of cleft palate in Spanish goats. This caprine cleft palate model was used to evaluate the temporal sequence of palatal shelf fusion throughout the period of cleft induction with the poisonous plant...

  15. Oblique facial clefts in Johanson-Blizzard syndrome.

    Science.gov (United States)

    Corona-Rivera, Jorge Román; Zapata-Aldana, Eugenio; Bobadilla-Morales, Lucina; Corona-Rivera, Alfredo; Peña-Padilla, Christian; Solis-Hernández, Elizabeth; Guzmán, Celina; Richmond, Erick; Zahl, Christian; Zenker, Martin; Sukalo, Maja

    2016-06-01

    Johanson-Blizzard syndrome (JBS) is considered as an infrequent, but clinically easily recognizable autosomal recessive entity by the pathognomonic combination of congenital exocrine pancreatic insufficiency and hypoplastic alae nasi, in addition to other distinctive findings such as scalp defects, hypothyroidism, and rectourogenital malformations. There are few reports of patients with JBS in association with facial clefting, referring all to types 2 to 6 of Tessier's classification that can be characterized properly as oblique facial clefts (OFCs). We describe the clinical aspects in four patients with JBS and extensive OFCs. In all of them, the diagnosis of JBS was confirmed by the demonstration of homozygous or compound-heterozygous mutations in the UBR1 gene. Additionally, we review three previously reported cases of JBS with OFCs. Taking into account a number of approximately 100 individuals affected by JBS that have been published in the literature we estimate that the frequency of OFCs in JBS is between 5% and 10%. This report emphasizes that extensive OFCs may be the severe end of the spectrum of facial malformations occurring in JBS. No obvious genotype phenotype correlation could be identified within this cohort. Thus, UBR1 should be included within the list of contributory genes of OFCs, although the exact mechanism remains unknown. © 2016 Wiley Periodicals, Inc. PMID:26989884

  16. [Presentation of a flap web space laterodigital in cleft foot].

    Science.gov (United States)

    Szwebel, J; Haddad, R; Mitrofanoff, M

    2012-08-01

    Cleft foot deformity is characterized by the absence of one or more median rays of the foot. This rare polymorphous congenital anomaly occurs more frequently in males, with a frequent autosomal dominant type of transmission. The purpose of surgical treatment is to narrow the width of the foot, but also to improve its global aesthetic look. Toe reparation, and more specifically web space reconstruction, provide the main technical challenges. We present an adaptation to the foot of a laterodigital cutaneous flap published by Barsky in 1964 for commissural reconstruction in cleft hand syndroms. The anatomical structure of fingers and toes commisures being different, this flap seems more adapted to the surgery of the foot. We gathered seven patients' files treated for ectrodactyly of the foot with this technique by the same surgeon from 2005 to 2008. No particular postoperative complications were noted, and the patients all expressed their satisfaction regarding the improvement of the appearance of their foot. We recommend to add the use of this flap in the "tool box" of the surgeon in charge of the management of foot deformities. PMID:20947236

  17. Occipital meningoencephalocele with Cleft Lip, Cleft Palate and Limb Abnormalities- A Case Report.

    Science.gov (United States)

    Ganapathy, Arthi; T, Sadeesh; Swer, Mary Hydrina; Rao, Sudha

    2014-12-01

    A 21-week-old still born female fetus with occipital encepholocele, cleft lip and cleft palate was received from the Department of Obstetrics and Gynecology, Mahatma Gandhi Medical College and Research Institute, Pondicherry and was studied in detail. It was born to Primigravida, of a second degree consanguineous marriage, with unremarkable family history. The biometric measurements were noted which corresponded to the age of the fetus. Further the fetus was embalmed and dissected. On examination an encephalocele of 2.7×1.5 cm was seen in the occipital region with a midline defect in the occipital bone and herniated brain tissue. Other anomalies observed were right unilateral cleft lip, right cleft palate, and bilateral syndactyly of the lower limbs and associated Congenital Talipus Equino Varus of the right foot. Other internal organs were developed appropriate for the age of the fetus.

  18. Fistula Rate after primary palatal repair with intravelarveloplasty: a retrospective three-year audit of six units (NorCleft) in the UK.

    Science.gov (United States)

    Moar, Kanwalraj K; Sweet, Christopher; Beale, Victoria

    2016-07-01

    Our aim was to evaluate the rate of fistulation after one-stage palatal repair by intravelarveloplasty in the NorCleft Cleft Services (Scotland and Northern England), this being a primary outcome measure after repair of a cleft palate. We designed a retrospective, three-year clinical audit of six cleft units in the UK, and retrospectively reviewed the casenotes of babies with cleft palate born in 2006-2008 who were treated by intravelarveloplasty. We recorded type of cleft and procedure, including lateral relieving incisions, and our main outcome measure was the presence of a fistula behind the incisive foramen at 3 years of age, or a history of repair of a fistula. A total of 743 patients had cleft palates, but 69 (9%) were excluded (because they had not been operated on, or had not been reviewed by the age of 3 years, or their records were unavailable). A total of 626 patients had had a Sommerlad intravelarveloplasty repair, and 48 had had mixed procedures including Veau-Wardill-Kilner, Furlow, or two-stage repairs, and were not studied further. Eighty-seven (14%) who had had intravelarvoloplasty had a fistula behind the incisive foramen. There was no significant difference in age at time of repair between those who developed a fistula and those who did not (p=0.65). The fistula rate of 14% is comparable with that of Sommerlad. The fistula rate was higher in patients who had had lateral releasing incisions (58/275, 21%) or who had bilateral cleft palate (16/63, 25%). To our knowledge this is the largest review of the fistula rate in patients who had primary palatal repair using the intravelarveloplasty technique in the UK, and shows significant correlation between lateral releasing incisions and formation of a fistula, except in the unilateral cleft lip and palate group (p=0.12). PMID:27118617

  19. Lexical selectivity in danish toddlers with cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth

    2013-01-01

    Objective : To study if Danish children with cleft palate display lexical selectivity in their early lexicon at 18 months of age. Design : A cross-sectional study. Participants : Thirty-four children with unilateral cleft lip and palate and 35 children without cleft palate, matched for gender...... also analyzed with respect to word productions, establishing an observed productive vocabulary size for each participant. Results : At 18 months of age Danish children with cleft palate showed marked lexical selectivity in their early words. The distribution of consonant classes observed at 11 months...... of age in a previous study of the children with cleft palate was almost perfectly reflected in their early lexicon at 18 months. The early lexicon of children with cleft palate differed from the early lexicon of their noncleft peers. Conclusions and Implications : Danish toddlers with cleft palate...

  20. The development of speech production in children with cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth; Chapman, Kathy

    2012-01-01

    The purpose of this chapter is to provide an overview of speech development of children with cleft palate +/- cleft lip. The chapter will begin with a discussion of the impact of clefting on speech. Next, we will provide a brief description of those factors impacting speech development for this p......The purpose of this chapter is to provide an overview of speech development of children with cleft palate +/- cleft lip. The chapter will begin with a discussion of the impact of clefting on speech. Next, we will provide a brief description of those factors impacting speech development...... for this population of children. Finally, research examining various aspects of speech development of infants and young children with cleft palate (birth to age five) will be reviewed. This final section will be organized by typical stages of speech sound development (e.g., prespeech, the early word stage...

  1. Real -time 3D ultrasound prenatal diagnosisof the clinical significance of fetal typical facial cleft deformity%实时三维超声产前诊断胎儿典型面裂畸形的临床意义

    Institute of Scientific and Technical Information of China (English)

    龚海英; 李琴; 艾明义

    2012-01-01

    Objective; To determine the value of Real - time 3D ultrasound prenatal diagnosis of the clinical significance of fetal for typical facial cleft deformity Improving the diagnosticaccuracy of fetal cleft lipand/or palate cleft. Methods; Application of real - time three dimensional echocardiography prenatal fetal normal fetal cleft lip and palate cleft lip and palate parts of two - dimension control. Result; 10 cases of normal fetal lip display rates 100% , On alveolar show 100% , and palate, 70% (7/10) . 10 cases of fetal cleft lip and palate cleft lip includes 7 simple, merge hard on 1 case of cleft lip and palate and 1 cleft lip patients with cleft of soft palate. Real - time 3D diagnostic all 7 example simple cleft palates, diagnostic rate 100% . Diagnosis of unilateral cleft lip with cleft palate in 1 case. There are 1 case of unilateral cleft lip with cleft palate only diagnosis and missed diagnosis of cleft lip and cleft palate, diagnosed in 70% ( Seven - tenths). 1 cases of cleft lip with cleft of soft palate only soft diagnosis and missed diagnosis of cleft lip and cleft palate. Conclusions: Real - time three dimensional echocardiography prenatal diagnosis of fetal cleft lip, especially hard and alveolar process cleft lip with cleft palate diagnostic has a large value, but on the fetus are not merged alveolar cleft of the soft palate and some of the hard palate to make diagnosis and still has a great deal of difficulty.%目的 探讨实时三维超声产前诊断胎儿面裂畸形的临床意义,寻求提高胎儿唇腭裂诊断准确性的有效方法.方法 应用实时三维超声产前对面裂畸形;唇腭裂胎儿和正常胎儿唇腭部位的二维进行对照.结果 10例正常胎儿面部显示率100%,上牙槽突显示率100%,硬腭显示率70% (7/ 10).10例唇腭裂胎儿包括7例单纯唇裂,2例上唇裂合并硬腭裂和1例唇裂合并软腭裂.实时三维诊断了所有7例单纯唇裂,诊断率100%;诊

  2. Neuroembryology and functional anatomy of craniofacial clefts

    Directory of Open Access Journals (Sweden)

    Ewings Ember

    2009-10-01

    Full Text Available The master plan of all vertebrate embryos is based on neuroanatomy. The embryo can be anatomically divided into discrete units called neuromeres so that each carries unique genetic traits. Embryonic neural crest cells arising from each neuromere induce development of nerves and concomitant arteries and support the development of specific craniofacial tissues or developmental fields. Fields are assembled upon each other in a programmed spatiotemporal order. Abnormalities in one field can affect the shape and position of developing adjacent fields. Craniofacial clefts represent states of excess or deficiency within and between specific developmental fields. The neuromeric organization of the embryo is the common denominator for understanding normal anatomy and pathology of the head and neck. Tessier′s observational cleft classification system can be redefined using neuroanatomic embryology. Reassessment of Tessier′s empiric observations demonstrates a more rational rearrangement of cleft zones, particularly near the midline. Neuromeric theory is also a means to understand and define other common craniofacial problems. Cleft palate, encephaloceles, craniosynostosis and cranial base defects may be analyzed in the same way.

  3. Dating brittle tectonic movements with cleft monazite

    DEFF Research Database (Denmark)

    Berger, Alfons; Gnos, E.; Janots, E.;

    2013-01-01

    Two millimeter-sized hydrothermal monazites from a cleft that developed late during a dextral transpressional deformation event in the Aar Massif, Switzerland, have been investigated using EMP and ion probe. The monazites are characterised by high Th/U ratios typical of other hydrothermal monazit...

  4. Maternal genes and facial clefts in offspring

    DEFF Research Database (Denmark)

    Jugessur, Astanand; Shi, Min; Gjessing, Håkon Kristian;

    2010-01-01

    BACKGROUND: Fetal conditions can in principle be affected by the mother's genotype working through the prenatal environment. METHODOLOGY/PRINCIPAL FINDINGS: Genotypes for 1536 SNPs in 357 cleft candidate genes were available from a previous analysis in which we focused on fetal gene effects [1...

  5. Controle químico da microflora oral em pacientes fissurados labiopalatais durante o tratamento ortodôntico-cirúrgico: estudo piloto Oral microflora chemical control in cleft lip and palate patients during orthodontic-surgical treatment pilot study

    Directory of Open Access Journals (Sweden)

    Alcion Alves Silva

    2004-06-01

    Full Text Available O risco de infecção é uma preocupação durante o tratamento ortodôntico-cirúrgico, principalmente em pacientes como os fissurados lábio palatais, pois as condições anatômicas e oclusais contribuem para a proliferação microbiana. Métodos manuais para o controle da microflora oral são muitas vezes insuficientes para a obtenção de uma higiene satisfatória. Técnicas alternativas, como agentes químicos, têm sido propostas como procedimentos auxiliares. O objetivo deste estudo foi comparar a eficiência de dois agentes químicos como auxiliares no controle da microflora, em pacientes ortodôntico-cirúrgicos (fissurados lábio-palatais. O delineamento da pesquisa foi um estudo exploratório, microbiológico (piloto, duplo-cego, para testar os seguintes agentes químicos: triclosan 0,03% (Plax-Colgate Palmolive e clorexidina digluconada 0,12% (Duplak-Herpro/Dentsply. A amostra foi composta por 30 voluntários divididos em 3 grupos: Grupo I (controle, Grupo II (teste _ clorexidina digluconada 0,12% e Grupo III (teste _ triclosan 0,03%. Todos os grupos receberam orientação de higiene bucal prévia à primeira coleta de material. Depois de sete dias de uso dos agentes químicos uma segunda coleta foi realizada e o uso do agente foi suspenso. Uma terceira coleta foi realizada após sete dias a partir da suspensão do agente. A técnica semiquantitativa proposta por Pilonetto e Pilonetto20 foi adotada para avaliar o crescimento de colônias bacterianas. Os resultados mostraram diferenças entre a eficácia dos agentes químicos, sendo que o Grupo II mostrou uma redução mais significativa da microflora quando comparado ao grupo III.The infection risk is a preoccupation during orthodontic-surgical treatment, mainly at cleft lip and palate patients, for their anatomical and occlusal condictions, that contributs to oral microflora proliferation. Manual methods for microflora control are frequently unsuccessful to obtain a sctisfactory

  6. Fetal cleft lip with and without cleft palate: Comparison between MR imaging and US for prenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Wang Guangbin, E-mail: wgb7932596@hotmail.com [Shandong Medical Imaging Research Institute, 324 Jingwu Road, Jinan, 250021 (China); Shan Ruiqin [Jinan Maternity and Child Care Hospital, Jinan (China); Zhao Lianxin; Zhu Xiangyu; Zhang Xinjuan [Shandong Medical Imaging Research Institute, 324 Jingwu Road, Jinan, 250021 (China)

    2011-09-15

    Objective: To describe the magnetic resonance (MR) imaging findings of fetal CL/CP and evaluate its diagnostic value. Methods and materials: Twelve fetuses with CL/CP diagnosed by transabdominal US underwent MR imaging within 2 days of US. Half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence on T2-weighted images was performed on sagittal, coronal, and axial planes anatomic to the fetuses during maternal breath holding. US and MR imaging findings were compared with final diagnoses obtained from post-natal physical examination or fetal autopsy. Results: Final diagnoses confirmed incomplete midline cleft lip (n = 1), unilateral cleft lip and palate (n = 7), bilateral cleft lip and palate (n = 1), midline cleft lip and palate (n = 3). US and MR imaging diagnosed all 12 fetuses with cleft lip and the laterality. 5 (45.5%) of 11 cleft palates were identified with US, 2 of 7 fetuses with unilateral cleft palate, 2 of 3 fetuses with midline cleft palate, and one fetus with bilateral cleft palate. On MR imaging, 10 (91%) of 11 cleft palates were correctly detected. One fetus with unilateral cleft palate was not detected. No false-positives occurred. Conclusion: MR imaging is valuable for diagnosis of fetal CL/CP. It can demonstrate additional findings and provide more information compared with US.

  7. Fetal cleft lip with and without cleft palate: Comparison between MR imaging and US for prenatal diagnosis

    International Nuclear Information System (INIS)

    Objective: To describe the magnetic resonance (MR) imaging findings of fetal CL/CP and evaluate its diagnostic value. Methods and materials: Twelve fetuses with CL/CP diagnosed by transabdominal US underwent MR imaging within 2 days of US. Half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence on T2-weighted images was performed on sagittal, coronal, and axial planes anatomic to the fetuses during maternal breath holding. US and MR imaging findings were compared with final diagnoses obtained from post-natal physical examination or fetal autopsy. Results: Final diagnoses confirmed incomplete midline cleft lip (n = 1), unilateral cleft lip and palate (n = 7), bilateral cleft lip and palate (n = 1), midline cleft lip and palate (n = 3). US and MR imaging diagnosed all 12 fetuses with cleft lip and the laterality. 5 (45.5%) of 11 cleft palates were identified with US, 2 of 7 fetuses with unilateral cleft palate, 2 of 3 fetuses with midline cleft palate, and one fetus with bilateral cleft palate. On MR imaging, 10 (91%) of 11 cleft palates were correctly detected. One fetus with unilateral cleft palate was not detected. No false-positives occurred. Conclusion: MR imaging is valuable for diagnosis of fetal CL/CP. It can demonstrate additional findings and provide more information compared with US.

  8. Syntax and Discourse in Near-Native French: Clefts and Focus

    Science.gov (United States)

    Donaldson, Bryan

    2012-01-01

    This study examines aspects of the syntax-discourse interface in near-native French. Two cleft structures--"c'est" clefts and "avoir" clefts--are examined in experimental and spontaneous conversational data from 10 adult Anglophone learners of French and ten native speakers of French. "C'est" clefts mark focus, and "avoir" clefts introduce new…

  9. Cranio-facial clefts in pre-hispanic America.

    Science.gov (United States)

    Marius-Nunez, A L; Wasiak, D T

    2015-10-01

    Among the representations of congenital malformations in Moche ceramic art, cranio-facial clefts have been portrayed in pottery found in Moche burials. These pottery vessels were used as domestic items during lifetime and funerary offerings upon death. The aim of this study was to examine archeological evidence for representations of cranio-facial cleft malformations in Moche vessels. Pottery depicting malformations of the midface in Moche collections in Lima-Peru were studied. The malformations portrayed on pottery were analyzed using the Tessier classification. Photographs were authorized by the Larco Museo.Three vessels were observed to have median cranio-facial dysraphia in association with midline cleft of the lower lip with cleft of the mandible. ML001489 portrays a median cranio-facial dysraphia with an orbital cleft and a midline cleft of the lower lip extending to the mandible. ML001514 represents a median facial dysraphia in association with an orbital facial cleft and a vertical orbital dystopia. ML001491 illustrates a median facial cleft with a soft tissue cleft. Three cases of midline, orbital and lateral facial clefts have been portrayed in Moche full-figure portrait vessels. They represent the earliest registries of congenital cranio-facial malformations in ancient Peru.

  10. Cranio-facial clefts in pre-hispanic America.

    Science.gov (United States)

    Marius-Nunez, A L; Wasiak, D T

    2015-10-01

    Among the representations of congenital malformations in Moche ceramic art, cranio-facial clefts have been portrayed in pottery found in Moche burials. These pottery vessels were used as domestic items during lifetime and funerary offerings upon death. The aim of this study was to examine archeological evidence for representations of cranio-facial cleft malformations in Moche vessels. Pottery depicting malformations of the midface in Moche collections in Lima-Peru were studied. The malformations portrayed on pottery were analyzed using the Tessier classification. Photographs were authorized by the Larco Museo.Three vessels were observed to have median cranio-facial dysraphia in association with midline cleft of the lower lip with cleft of the mandible. ML001489 portrays a median cranio-facial dysraphia with an orbital cleft and a midline cleft of the lower lip extending to the mandible. ML001514 represents a median facial dysraphia in association with an orbital facial cleft and a vertical orbital dystopia. ML001491 illustrates a median facial cleft with a soft tissue cleft. Three cases of midline, orbital and lateral facial clefts have been portrayed in Moche full-figure portrait vessels. They represent the earliest registries of congenital cranio-facial malformations in ancient Peru. PMID:26010214

  11. Definition of critical periods for Hedgehog pathway antagonist-induced holoprosencephaly, cleft lip, and cleft palate.

    Directory of Open Access Journals (Sweden)

    Galen W Heyne

    Full Text Available The Hedgehog (Hh signaling pathway mediates multiple spatiotemporally-specific aspects of brain and face development. Genetic and chemical disruptions of the pathway are known to result in an array of structural malformations, including holoprosencephaly (HPE, clefts of the lip with or without cleft palate (CL/P, and clefts of the secondary palate only (CPO. Here, we examined patterns of dysmorphology caused by acute, stage-specific Hh signaling inhibition. Timed-pregnant wildtype C57BL/6J mice were administered a single dose of the potent pathway antagonist vismodegib at discrete time points between gestational day (GD 7.0 and 10.0, an interval approximately corresponding to the 15th to 24th days of human gestation. The resultant pattern of facial and brain dysmorphology was dependent upon stage of exposure. Insult between GD7.0 and GD8.25 resulted in HPE, with peak incidence following exposure at GD7.5. Unilateral clefts of the lip extending into the primary palate were also observed, with peak incidence following exposure at GD8.875. Insult between GD9.0 and GD10.0 resulted in CPO and forelimb abnormalities. We have previously demonstrated that Hh antagonist-induced cleft lip results from deficiency of the medial nasal process and show here that CPO is associated with reduced growth of the maxillary-derived palatal shelves. By defining the critical periods for the induction of HPE, CL/P, and CPO with fine temporal resolution, these results provide a mechanism by which Hh pathway disruption can result in "non-syndromic" orofacial clefting, or HPE with or without co-occurring clefts. This study also establishes a novel and tractable mouse model of human craniofacial malformations using a single dose of a commercially available and pathway-specific drug.

  12. Definition of Critical Periods for Hedgehog Pathway Antagonist-Induced Holoprosencephaly, Cleft Lip, and Cleft Palate

    Science.gov (United States)

    Heyne, Galen W.; Melberg, Cal G.; Doroodchi, Padydeh; Parins, Kia F.; Kietzman, Henry W.; Everson, Joshua L.; Ansen-Wilson, Lydia J.; Lipinski, Robert J.

    2015-01-01

    The Hedgehog (Hh) signaling pathway mediates multiple spatiotemporally-specific aspects of brain and face development. Genetic and chemical disruptions of the pathway are known to result in an array of structural malformations, including holoprosencephaly (HPE), clefts of the lip with or without cleft palate (CL/P), and clefts of the secondary palate only (CPO). Here, we examined patterns of dysmorphology caused by acute, stage-specific Hh signaling inhibition. Timed-pregnant wildtype C57BL/6J mice were administered a single dose of the potent pathway antagonist vismodegib at discrete time points between gestational day (GD) 7.0 and 10.0, an interval approximately corresponding to the 15th to 24th days of human gestation. The resultant pattern of facial and brain dysmorphology was dependent upon stage of exposure. Insult between GD7.0 and GD8.25 resulted in HPE, with peak incidence following exposure at GD7.5. Unilateral clefts of the lip extending into the primary palate were also observed, with peak incidence following exposure at GD8.875. Insult between GD9.0 and GD10.0 resulted in CPO and forelimb abnormalities. We have previously demonstrated that Hh antagonist-induced cleft lip results from deficiency of the medial nasal process and show here that CPO is associated with reduced growth of the maxillary-derived palatal shelves. By defining the critical periods for the induction of HPE, CL/P, and CPO with fine temporal resolution, these results provide a mechanism by which Hh pathway disruption can result in “non-syndromic” orofacial clefting, or HPE with or without co-occurring clefts. This study also establishes a novel and tractable mouse model of human craniofacial malformations using a single dose of a commercially available and pathway-specific drug. PMID:25793997

  13. TGFA and IRF6 contribute to the risk of nonsyndromic cleft lip with or without cleft palate in northeast China.

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    Yongping Lu

    Full Text Available Nonsyndromic cleft lip with or without cleft palate (NSCL/P are common birth defects with a complex etiology. Multiple interacting loci and possible environmental factors influence the risk of NSCL/P. 12 single nucleotide polymorphisms (SNPs in 7 candidate genes were tested using an allele-specific primer extension for case-control and case-parent analyses in northeast China in 236 unrelated patients, 185 mothers and 154 fathers, including 128 complete trios, and 400 control individuals. TGFA and IRF6 genes showed a significant association with NSCL/P. In IRF6, statistical evidence of an association between rs2235371 (p = 0.003, rs2013162 (p<0.0001 and NSCL/P was observed in case-control analyses. Family based association tests (FBATs showed over-transmission of the C allele at the rs2235371 polymorphism (p = 0.007. In TGFA, associations between rs3771494, rs3771523 (G3822A, rs11466285 (T3851C and NSCL/P were observed in case-control and FBAT analyses. Associations between other genes (BCL3, TGFB3, MTHFR, PVRL1 and SUMO1 and NSCL/P were not detected.

  14. [The experience of complex treatment of children with bilateral cleft lip and palate].

    Science.gov (United States)

    Supiev, T K; Mamedov, A A; Negametzianov, N G; Nurmaganov, S B; Utepov, D K; Katasonova, E S; Kozhabekov, E M

    2014-01-01

    Three grades of premaxilla deformation were revealed in children with bilateral cleft lip and palate. All patients with grade I and some children with grade II deformation received early orthopedic treatment by Sharova appliance while in patients with grade III deformation orthodontic devices with bone fixation with microimplants were used or microimplants alone for orthodontic anchorage. This approach allowed achieving full side contact of premaxilla and maxillary fragments in 1-2 months thus creating favorable conditions for surgical procedure.

  15. 3D stereophotogrammetric analysis of lip and nasal symmetry after primary cheiloseptoplasty in complete unilateral cleft lip repair

    NARCIS (Netherlands)

    Loon, B. van; Reddy, S.G.; Heerbeek, N. van; Ingels, K.J.A.O.; Maal, T.J.J.; Borstlap, W.A.; Reddy, R.R.; Kuijpers-Jagtman, A.M.; Berge, S.J.

    2011-01-01

    BACKGROUND: The aim of this study was to evaluate symmetry of the lip and nose in patients with CUCLP after primary cheiloseptoplasty (Afroze technique), in comparison to non-cleft controls. METHODOLOGY: In this prospective study, forty-four patients with operated non-syndromic CUCLP were included.

  16. Clinical analysis of 302 cases of cleft palate%腭裂患者302例临床资料分析

    Institute of Scientific and Technical Information of China (English)

    刘瑞红; 李新明

    2011-01-01

    目的 通过回顾性调查,了解河南省先天性腭裂的患病情况与特点,从而规范河南省唇腭裂患者的综合序列治疗.方法 收集2005年8月至2009年12月期间在郑州大学第一附属医院口腔科进行腭裂手术及腭裂术后二期修复的302例腭裂患者的临床病历资料,并将所得数据进行统计分析.结果 在本组病例中,男性患者172例,女性患者130例,年龄1.5~41岁,中位年龄3.6岁,男女比例约为1.32∶1; 单纯腭裂的发生男女差异无统计学意义,而唇腭裂的发生男女性别差异有统计学意义; 单纯腭裂和腭裂伴有唇裂的发生率差异有统计学意义;在腭裂的构成比中,主要以不完全腭裂为主,占70.86%,完全腭裂占19.87%,腭裂术后穿孔占6.29%.结论 单纯腭裂的发生男女无明显性别差异;而唇腭裂的发生男女性别差异有统计学意义,男性约是女性的3倍;单纯腭裂的发病率明显高于腭裂伴有唇裂的发病率;在腭裂的构成比中,主要以不完全腭裂为主;腭瘘仍是目前腭裂术后最常见的并发症.%Objective To investigate the characteristics and features of cleft palate and improve comprehensive and sequential therapy of cleft lip and palate (CLP) in Henan province by a retrospective study. Methods A total of 302 CLP were included in the retrospective study, who underwent primary and secondary cleft palate repair during August 2005 to December 2009. Results Three hundred and two patients (172 males,130 females,age ranged from 1.5 years to 41 years with a median age of 3.6 years)were included in the study. There was no significant gender difference in cleft palate, but in cleft lip and palate. And there was significant rate difference between cleft palate and cleft lip and palate. According to cleft types, 214 patients (70.86%) were incomplete cleft palate, 60 patients(19.87%) were complete cleft palate, 12 patients(3.97%) were cleft soft palate, 19 patients(6.29%) were palate fistula

  17. FOXE1 association with both isolated cleft lip with or without cleft palate, and isolated cleft palate

    Science.gov (United States)

    Moreno, Lina M.; Mansilla, Maria Adela; Bullard, Steve A.; Cooper, Margaret E.; Busch, Tamara D.; Machida, Junichiro; Johnson, Marla K.; Brauer, David; Krahn, Katherine; Daack-Hirsch, Sandy; L'Heureux, Jamie; Valencia-Ramirez, Consuelo; Rivera, Dora; López, Ana Maria; Moreno, Manuel A.; Hing, Anne; Lammer, Edward J.; Jones, Marilyn; Christensen, Kaare; Lie, Rolv T.; Jugessur, Astanand; Wilcox, Allen J.; Chines, Peter; Pugh, Elizabeth; Doheny, Kim; Arcos-Burgos, Mauricio; Marazita, Mary L.; Murray, Jeffrey C.; Lidral, Andrew C.

    2009-01-01

    Nonsyndromic orofacial clefts are a common complex birth defect caused by genetic and environmental factors and/or their interactions. A previous genome-wide linkage scan discovered a novel locus for cleft lip with or without cleft palate (CL/P) at 9q22–q33. To identify the etiologic gene, we undertook an iterative and complementary fine mapping strategy using family-based CL/P samples from Colombia, USA and the Philippines. Candidate genes within 9q22–q33 were sequenced, revealing 32 new variants. Concurrently, 397 SNPs spanning the 9q22–q33 2-LOD-unit interval were tested for association. Significant SNP and haplotype association signals (P = 1.45E − 08) narrowed the interval to a 200 kb region containing: FOXE1, C9ORF156 and HEMGN. Association results were replicated in CL/P families of European descent and when all populations were combined the two most associated SNPs, rs3758249 (P = 5.01E − 13) and rs4460498 (P = 6.51E − 12), were located inside a 70 kb high linkage disequilibrium block containing FOXE1. Association signals for Caucasians and Asians clustered 5′ and 3′ of FOXE1, respectively. Isolated cleft palate (CP) was also associated, indicating that FOXE1 plays a role in two phenotypes thought to be genetically distinct. Foxe1 expression was found in the epithelium undergoing fusion between the medial nasal and maxillary processes. Mutation screens of FOXE1 identified two family-specific missense mutations at highly conserved amino acids. These data indicate that FOXE1 is a major gene for CL/P and provides new insights for improved counseling and genetic interaction studies. PMID:19779022

  18. Nasoalveolar Molding: A new Method for Cleft Lip and Palate Rehabilitation

    Directory of Open Access Journals (Sweden)

    A Jahanbin

    2014-04-01

    Full Text Available Introduction: Cleft lip and palate is a congenital anomaly occurring in 3 in 1000 live birth. One the major concern in this patient is about cleft gap and its surgical problems. Several surgical and non-surgical procedures have been done to decrease this gap. They aimed to achieve the more esthetic results as well as feeding success. Nasoalveolar Molding (NAM is a new method for reshaping nasal and alveolar bones, presurgically. Matsuo et al described that auricular cartilage could be molded permanently when treatment was done within 6 weeks of life. High levels of maternal estrogen in the fetal circulation can triggers hyaluronic acid which can alter the cartilage, ligament and connective tissue elasticity.Estrogen level continue to drop after 6 weeks of age. This concept was applied for the correction of nasal deformities in cleft lip patients. Nasolaveolar molding may stimulate immature nasal chondroblasts and produce interstitial expansion.   Aims of Nasoalveolar Molding: a. Active molding and repositioning of the deformed nasal cartilages and alveolar processes. b. Appropriate Lengthening of the columellac. Better bone healing after surgey due to reducing the gaped. Reduces the need for secondary alveolar bone grafts. Correction of lip position with minimal scarf. Reducing hospital stay for nasal esthetic surgeryg. Better weight gain in early infancy   Conclusion: Management of cleft lip and palate has been changed with more emphasis on the nasal and alveolar molding prior to the primary lip repair. This method reduces the number reconstructive surgeries  for the purpose of esthetics as well as may cause better feeding in early infancy.   Keywords: Cleft Lip and Palate, Infant Orthopedics, Nasal Molding.  

  19. Mutational analysis of the Sonic Hedgehog gene in 220 newborns with oral clefts in a South American (ECLAMC) population.

    Science.gov (United States)

    Orioli, Iêda M; Vieira, Alexandre R; Castilla, Eduardo E; Ming, Jeffrey E; Muenke, Maximilian

    2002-02-15

    Oral clefts generally have a multifactorial etiology. A number of genes contribute to the formation of the face and palate. Cleft lip and/or palate can occur in pedigrees with autosomal dominant holoprosencephaly due to mutations in Sonic Hedgehog (SHH). In addition, animal models have shown that SHH is involved in face development. We thus examined the human SHH gene in 220 newborn infants with nonsyndromic oral clefts registered by the Estudio Colaborativo Latinoamericano de Malformaciones Congenitas: ECLAMC (Latin American Collaborative Study of Congenital Malformations). We found 15 variant bands in 13 patients with oral clefts, representing five different base changes, all of which were found by sequencing to represent silent polymorphisms. Four occurred in introns. The alteration occurring in an exon, Ser190Ser, may create a consensus sequence for the 3'splice site 6 bp downstream of the original consensus sequence. Thus, we did not identify any clearly disease-causing mutation in SHH in these patients, and conclude that SHH mutations are not a frequent cause of isolated oral clefts in humans.

  20. Columella Lengthening with a Full-Thickness Skin Graft for Secondary Bilateral Cleft Lip and Nose Repair

    Science.gov (United States)

    Lee, Yoon Seok; Shin, Dong Hyeok; Choi, Hyun Gon; Kim, Jee Nam; Lee, Myung Chul; Kim, Soon Heum; Kim, Cheol Keun; Jo, Dong In

    2015-01-01

    Background Various techniques for lengthening short columellae have been used for bilateral cleft nose repair. However, previous methods have not yielded satisfactory results. We performed a full-thickness skin graft to lengthen short columellae during secondary cleft nose repair in adult patients. Methods Ten bilateral cleft lip and nose patients underwent secondary cheiloplasty with open rhinoplasty between July 2008 and August 2014. The patients underwent a full-thickness skin graft on the medial crura to elongate the columella. The average age of the patients at the time of surgery was 22.2 years. Nasal profiles were evaluated before and after the operation using the photogrammetric method. Results The nasal profiles were improved in all patients, and all skin grafts were well taken, with the exception of one patient. Columellar height, nostril height, and columella-lip angle increased, and nasal width decreased significantly. The ratios of columellar height to nasal height, columellar height to nasal width, and nasal height to nasal width increased to a statistically significant extent. Conclusions Columella lengthening with a full-thickness skin graft is a simple and effective method for the repair of severely short columellae in bilateral cleft nose patients. We had satisfactory outcomes, with good color matching and aesthetically pleasing contours. PMID:26618116

  1. Stability after Cleft Maxillary Distraction Osteogenesis or Conventional Orthognathic Surgery

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    Kristian Andersen

    2015-06-01

    Full Text Available Objectives: To compare stability of maxillary advancements in patients with cleft lip and palate following distraction osteogenesis or orthognathic surgery. Material and Methods: Inclusion criteria: 1 cleft lip and palate, 2 advancement > 8 mm. Eleven patients comprised the distraction osteogenesis group (DOG. Seven patients comprised the orthognathic treatment group (CONVG. Skeletal and soft tissue points were traced on lateral cephalograms: T1 (preoperatively, T2 (after surgery, T3 (follow-up. Group differences were analyzed using Students t-test. Results: At T1-T2, advancement of 6.98 mm (P = 0.002 was observed in DOG. Horizontal overjet increased 11.62 mm (P = 0.001. A point-nasion-B point (ANB angle increased 8.82° (P = 0.001. Aesthetic plane to upper lip was reduced 5.44 mm (P = 0.017 and the naso-labial angle increased 16.6° (P = 0.001. Vertical overbite (VOB increased 2.27 mm (P = 0.021. In T2-T3, no significant changes were observed in DOG. In T1-T2, horizontal overjet increased 8.45 mm (P = 0.02. The ANB angle, 9.33° (P = 0.009 in CONVG. At T2-T3, VOB increased, 2.35 mm (P = 0.046, and the ANB angle reduced, 3.83° (P = 0.003. In T2-T3, no parameters changed in CONVG. At follow-up (T3, VOB increased in CONVG compared with DOG, (P = 0.01. Vertical position of A point differed between the groups (P = 0.04. No significant intergroup differences between soft tissue parameters occurred. Conclusions: Distraction osteogenesis resulted in a stable position of the maxilla and movement upwards in vertical plane, however in case of orthognathic treatment sagittal relapse and a continued postoperatively downward movement was registered.

  2. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

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    Reema Sharma Dhar

    2014-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  3. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome.

    Science.gov (United States)

    Dhar, Reema Sharma; Bora, Amitava

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report. PMID:25231046

  4. TBX22基因变异与国人部分人群唇腭裂发病的关系%TBX22 Mutation Associated with Non-syndromic Cleft Lip and/or Cleft Palate in the Chinese Population

    Institute of Scientific and Technical Information of China (English)

    徐晨; 乌丹旦; 王国民; 梁赟; 杨育生

    2013-01-01

    Objective:This study purposed to investigate TBX22 gene population profile in Chinese patients and families with cleft lip and/or cleft palate (CL/P),and examine whether mutations or genetic polymorphisms in TBX22 are related to the formation of cleft lip and/or palate.Methods:100 CPI (cleft palate isolated) and 51 CL/P patients were enrolled in the study.TBX22 mutations were revealed by sequencing in patients with different cleft types,and analyzed by using statistical and bioinformatical methods.Results:Five TBX22 variants were found including a hemizygous missense mutation 874G>A (D292N) in a family with cleft lip,one synonymous variant and three single nucleotide alterations in introns.The first is potential pathogenic.Conclusion:The study provided a population profile in Chinese CPI and CL/P patients,and confirmed the important role of TBX22 in patients with cleft lip accompanied with/without cleft palate.%目的:在中国唇腭裂患者中,对TBX22基因进行检测,研究TBX22基因变异或者多态性与部分中国人群唇腭裂的关系.方法:采用基因测序的方法,在100例唇腭裂患者中进行TBX22基因测序,对得到的变异位点,在正常人中进行验证,并对结果进行生物信息学分析.结果:共发现5个TBX22基因变异位点:876G>A(D292N),72C>T(L24L)和其他3个内含子区域的单核苷酸多态性位点.结论:首次在中国人群唇腭裂患者中进行TBX22基因全部外显子区域及附近序列的测序,得出了中国唇腭裂患者TBX22基因的变异情况.

  5. THREE-DIMENSIONAL ASSESSMENT OF THE PHARYNGEAL AIRWAY AND MAXILLARY SINUS VOLUMES IN INDIVIDUALS WITH NON-SYNDROMIC CLEFT LIP AND PALATE

    Directory of Open Access Journals (Sweden)

    Ana NEMȚOI

    2015-09-01

    Full Text Available Introduction: Children with cleft lip and palate (CLP are known to have airway problems. Introduction of ConeBeam CT (CBCT and imaging software has facilitated generation of 3D images for assessing the volume of maxillary sinuses and pharyngeal airway. Consequently, the present study aimed at evaluating and comparing the maxillary sinus and pharyngeal airway volume of patients with cleft lip and palate in healthy patients, using cone beam computed tomography (CBCT images. Materials and method: The sample group included 27 individuals (15 with cleft lip and palate subjects and 12 healthy subjects. The pharyngeal airway and each maxillary sinus were three-dimensionally assessed, segmented and their volume was calculated. A comparison between the right and left sinus was performed by Student t-test, and the differences between the control and cleft groups were calculated using ANOVA. Results: No statistically significant differences were found when the maxillary sinuses volumes from each side were compared (p >0.05. The unilateral CLP patients presented the lowest sinus volume. Individuals with CLP did not exhibit a total airway volume smaller than the nonCLP controls. Conclusions: 3D imaging using CBCT and Romexis software is reliable for assessing maxillary sinus and pharyngeal airway volume. The present study showed that the pharyngeal airway is not compromised in CLP individuals. The unilateral CLP individuals present maxillary sinuses with smaller volumes, no differences being recorded between the cleft and non-cleft side.

  6. 唇腭裂患者的牙周健康状况及其影响因素%Periodontal status and its influencing factors in patients with cleft lip and palate

    Institute of Scientific and Technical Information of China (English)

    章筱悦; 陈振琦

    2014-01-01

    index compared with normal controls. Patients with unilateral and bilateral CLP(UCLP and BCLP) exhibit significant plaque accumulation and gingivitis bleeding. The prevalence of plaque accumulation and gingivitis is higher in patients with CLP than in controls, and plaque accumulation is significantly higher in patients with BCLP than in patients with UCLP. The cleft sides present a higher plaque index and probing depth than the noncleft sides. Periodontal problems in soft and hard tissues have often been reported in patients with CLP. Such problems include reduced keratinized gingiva, gingival recession, gingival inflammation, and decreased alveolar bone height, and all of which can affect the periodontal status. The colonization rate of Saccharomyces in patients with CLP is significantly higher than in healthy control subjects and is highest in patients with CLP who had undergone at least three surgeries and in patients with BCLP. Grafting an alveolar bone to the cleft area is a method to improve the alveolar morphology and increase bone mass. This method helps improve the periodontal status and provides healthy periodontal support for the teeth adjacent to the cleft. The incidence of gingival inflammation is also reduced. Therefore, the aim of this study is to summarize the oralhygiene and periodontal status, including its influencing factors, of patients with CLP.

  7. Aesthetic Evaluation of the Nasolabial Region in Children with Unilateral Cleft Lip and Palate Comparing Expert versus Nonexperience Health Professionals

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    Tatiana Saito Paiva

    2014-01-01

    Full Text Available Esthetic evaluation of cleft lip and palate rehabilitation outcomes may assist in the determination of new surgical interventions and aid in reevaluation of treatment protocols. Our objective was to compare esthetics assessments of the nasolabial region in children with a unilateral cleft lip and palate between healthcare professionals who were experienced in the treatment of cleft lip and palate and those who were inexperienced. The study group included 55 patients between 6 and 12 years of age who had already undergone primary reconstructive surgery for unilateral cleft lip. Standardized digital photographs were obtained, and the esthetic features of the nose, lip, and nasolabial region were evaluated. We used only cropped photographic images in the assessments of healthcare professionals with and without experience in cleft lip and palate. Interrater analysis revealed highly reliable assessments made by both the experienced and inexperienced professionals. There was no statistically significant difference in the esthetic attractiveness of the lip and nose between the experienced and inexperienced professionals. Compared with the inexperienced professionals, the experienced professional evaluators showed higher satisfaction with the esthetic appearance of the nasolabial region; however, no difference was observed in the analysis of the lip or nose alone.

  8. Behavioral pattern in Chinese school-aged children with cleft lip and palate

    Institute of Scientific and Technical Information of China (English)

    Pin Ha; Xu-Qing Zhuge; Qian Zheng; Bing Shi; Cai-Xia Gong; Yan Wang

    2013-01-01

    Objective:To obtain descriptive information of behavioral pattern inChinese school-aged children with cleft lip and palate.Methods:A total of93 cleft lip and palate patients between the age of6-11 year-old and treated atWestChinaStomatologyHospital were selected.And another 100 unaffected controls, matched for age and gender, were recruited randomly from a common primary school inChengdu.Chart review of medical records was used to obtain psychosocial checklists.Scores were compared with published norms and controls to evaluate the risk of problems, separately for three diagnostic groups.Results:The patients group had lower scores of social and academic competencies, especially those with facial deformity or speech problem. No difference was found in the aspect of activity competency.All patients showed elevations in behavior problems.But the type of behavior problems varied in different genders.Conclusions:Chinese school-aged children with cleft lip and palate are at raised risk for social and academic difficulties.Specific pattern of behavior problems displays differently depending on gender of the patient.

  9. Research on orthodontic treatment for complete unilateral cleft lip and palate patients in late permanent dentition%恒牙晚期单侧完全性唇腭裂患者正畸治疗临床疗效研究

    Institute of Scientific and Technical Information of China (English)

    刘毅; 王春玲; 孙慧芳; 王海任

    2011-01-01

    Objective By measuring the post-surgery characteristics of complete unilateral cleft lip and palate (UCLP) patients' teeth, occlusion, soft and hard tissues in late permanent dentition, the purpose of this study was to investigate the clinical effects of orthodontic treatments for complete UCLP patients on face types, maxillary dental arch and occlusion relations and to provide reliable theoretic basis for treating these kinds of patients. Methods Ten post-surgery complete UCLP patients who had cleft lip and palate in late permanent dentition( From 15 to 17 years old) were selected in this research. Every patient was treated with maxillary slow arch expansion and fixed appliance. The related data about X-ray lateral skull films before therapy and after six months' maxillary expansion, as well as teeth models before expansion, after expansion and after six months' were measured. The Statistical Methods were paired t-test for dependent samples and two-way ANOVA, LSD-test were used for the mutual comparison between two groups. Results After orthodontic treatments, some measured data of soft and hard tissues in complete UCLP patients' lateral skull films had relatively significant changes (P <0.01 ). Arch width increased significantly in canine area and premolar area( P < 0. 01 ). The increase in premolar area could be maintained steadily (P < 0.05). Conclusions After orthodontic treatments, bone, soft and hard tissues and occlusion relations of complete UCLP patients in late permanent dentition who had get cleft lip and palate had relatively ideal effects.%目的 通过测量恒牙晚期单侧完全性唇腭裂(UCLP)患者术后牙(牙合)、软硬组织的相关数据,观察正畸治疗对患者面型、牙弓及咬合关系的临床疗效.方法 选择10例UCLP恒牙晚期(15~17岁)术后患者,采用上颌慢速扩弓联合固定矫治,对治疗前、扩弓保持半年后X线头颅侧位片及扩弓治疗前、扩弓结束后、保持半年后牙(牙合)

  10. "Nara" knot for suturing of cleft lip in children to make removal easy

    Directory of Open Access Journals (Sweden)

    Obaidullah

    2006-01-01

    Full Text Available Cleft patients usually go through a lifetime of repeated hospital admissions and multiple procedures. Suture removal at a tender age and on a sensitive area like the lip becomes a challenge for the nursing staff. It is also emotionally demanding on the part of the parents. Hence, in most centres these patients are at least sedated if not anaesthetised. We have been using a simple knot and running prolene material so that undoing of the knot becomes easy and suture removal more or less atraumatic. We would like to share our experience with readers through this article. An analysis of 53 cleft lip repairs has shown that this knot is safe and easily removable.

  11. Three-Dimensional Upper Lip and Nostril Sill Changes After Cleft Alveolus Reconstruction Using Autologous Bone Grafting Versus Recombinant Human Bone Morphogenetic Protein-2.

    Science.gov (United States)

    Raposo-Amaral, Cassio Eduardo; Denadai, Rafael; Alonso, Nivaldo

    2016-06-01

    Cleft alveolus in patients with unilateral complete cleft lip and palate has been alternatively reconstructed with recombinant human bone morphogenetic protein (rhBMP)-2. However, its effects on upper lip and nostril sill anatomy are not known. Thus, the objective of this investigation was to assess and compare upper lip and nostril sill changes after cleft alveolus reconstruction with autologous bone from the iliac crest region and rhBMP-2. Patients were randomly allocated into 2 groups. In group 1, autologous bone from the iliac crest region was used to fill the cleft alveolus (n = 4), and in group 2, rhBMP-2 was used to fill the cleft alveolus (n = 8). Preoperatively and at one after the surgery, computerized tomography (CT) was performed. Reformatted CT imaging was used to perform cephalometric linear measurements of the upper lip and nostril sill regions. Inter- and intragroup data of the pre and postoperative reformatted CT measurements of the upper lip and nostril sill regions did not show differences (P >0.05) in cutaneous upper lip height and projection, nostril sill elevation, and subnasale projection. There were no significant upper lip and nostril sill anatomical changes after cleft alveolus reconstruction using autologous bone grafting and rhBMP-2. PMID:27244210

  12. Prenetal Detection of Oral Clefts : Diagnostic, Genetic and Ethical Aspects

    NARCIS (Netherlands)

    Maarse, W.

    2015-01-01

    Since the introduction of routine prenatal screening with ultrasound in the Netherlands in 2007, parents are confronted with the diagnosis of oral cleft (OC) already during pregnancy. This imposed a new dimension in cleft care in the Netherlands. As a consequence to increasing prenatal detection rat

  13. Lexical selectivity in danish toddlers with cleft palate.

    Science.gov (United States)

    Willadsen, Elisabeth

    2013-07-01

    Objective : To study if Danish children with cleft palate display lexical selectivity in their early lexicon at 18 months of age. Design : A cross-sectional study. Participants : Thirty-four children with unilateral cleft lip and palate and 35 children without cleft palate, matched for gender and age. Methods : All participants were video recorded at 18 months of age during play interaction with a parent. The video recordings were transcribed according to the International Phonetic Alphabet and an individual consonant inventory was established for each participant. The video recordings were also analyzed with respect to word productions, establishing an observed productive vocabulary size for each participant. Results : At 18 months of age Danish children with cleft palate showed marked lexical selectivity in their early words. The distribution of consonant classes observed at 11 months of age in a previous study of the children with cleft palate was almost perfectly reflected in their early lexicon at 18 months. The early lexicon of children with cleft palate differed from the early lexicon of their noncleft peers. Conclusions and Implications : Danish toddlers with cleft palate display lexical selectivity in the early lexicon as it has been described for English-speaking toddlers with and without cleft palate, even though some qualitative differences were found.

  14. Cone-Beam computed tomography evaluation of maxillary expansion in twins with cleft lip and palate

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    Luciane Macedo de Menezes

    2012-04-01

    Full Text Available OBJECTIVE: The establishment of normal occlusal relationships in patients with cleft lip and palate using rapid maxillary expansion may promote good conditions for future rehabilitation. OBJECTIVE: This study describes the clinical case of monozygotic twins with unilateral cleft lip and palate at the age of mixed dentition, who were treated using the same rapid maxillary expansion protocol, but with two different screws (conventional and fan-type expansion screw. Results were evaluated using plaster models, intraoral and extraoral photographs, and Cone-Beam computed tomography (CBCT scans obtained before the beginning of the treatment, (T1. METHODS: The patients were followed up for 6 months after maxillary expansion, when the same tests requested at T1 were obtained again for review (T2. T1 and T2 results were compared using lateral cephalometric tracings and measurements of the intercanine and intermolar distances in the plaster models using a digital caliper. RESULTS: The two types of expansion screws corrected the transverse discrepancy in patients with cleft lip and palate. The shape of the upper arches improved at 10 days after activation. CONCLUSION: CBCT scans provide detailed information about craniofacial, maxillary and mandibular changes resulting from rapid maxillary expansion. The most adequate screw for each type of malocclusion should be chosen after detailed examination of the dental arches.

  15. Photographs of dental casts or digital models: rating dental arch relationships in bilateral cleft lip and palate.

    NARCIS (Netherlands)

    Leenarts, C.M.; Bartzela, T.N.; Bronkhorst, E.M.; Semb, G.; Shaw, W.C.; Katsaros, C.; Kuijpers-Jagtman, A.M.

    2012-01-01

    Yardsticks have been developed to measure dental arch relations in cleft lip and palate (CLP) patients as diagnostic proxies for the underlying skeletal relationship. Travelling with plaster casts to compare results between CLP centres is inefficient so the aim of this study was to investigate the r

  16. Reproductive patterns among Danish women with oral clefts

    DEFF Research Database (Denmark)

    Yttri, Janne Elin; Christensen, Kaare; Knudsen, Lisbeth B.;

    2011-01-01

    cleft had an average of 1.98 child per woman. This did not differ significantly from the background population (2.02 child/woman). Furthermore, the present study found that Danish women born with oral clefts on average were older (27.3 years of age) than the background population (24.7 years of age......Objective: The aim of the study was to compare the reproduction pattern among Danish women born with isolated oral clefts with the Danish background population. Design and setting: A nationwide population based historic cohort-study based on three registers: The Danish Facial Cleft Register......, The Danish Civil Registration System and the Fertility of Women and Couples Dataset. Participants: Through linkages of the registers, number of children and the exact age at childbirth of all Danish women born with an oral cleft during 1950 through 1988 (N=1,931) were obtained. These data were compared...

  17. TESSIER CLEFT NO. 7: REPORT OF 12 CASES

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    Utpal

    2014-06-01

    Full Text Available Tessier cleft 7, also known as Transverse facial cleft or congenital macrostomia, is a rare congenital anomaly. It presents with varying degree of severity and is associated with anomalies of other structures developing from first and second branchial arches. We report twelve cases of Tessier cleft 7 presenting to us at various ages. The cases were studied on the basis of antenatal and family history, age, sex, laterality, severity and other associated anomalies. The clefts were classified complete or incomplete on the basis of their lateral extension. Surgical correction of the clefts consisted of soft tissue repair and correction of associated deformities with satisfactory functional and aesthetic results, without any intra-operative and post-operative complications.

  18. Spectrographic analysis of pain cry in neonates with cleft palate.

    Science.gov (United States)

    Michelsson, K; Sirviö, P; Koivisto, M; Sovijärvi, A; Wasz-Höckert, O

    1975-01-01

    52 phonations of 13 cleft palate neonates were analyzed by sound spectrographic methods. 17 phonetical attributes were included in the study and the first signal after the pain stimulus was analyzed. The cries of the cleft palate infants were compared with the crying of 75 normal babies of the same age. No change in the fundamental frequency, melody type and duration of the cries was seen in association with these anatomical defects. Two of the characteristics studied, vibrato and the 'tonal pit', occurred significantly more often in cries of the cleft palate infants than in cries of the control series. The changes in the qualities seen in association with cleft palate and/or cleft lip do not mimic the abnormalities produced by brain damage.

  19. Cross-linguistic perspectives on speech assessment in cleft palate

    DEFF Research Database (Denmark)

    Willadsen, Elisabeth; Henningsson, Gunilla

    2012-01-01

    This chapter deals with cross linguistic perspectives that need to be taken into account when comparing speech assessment and speech outcome obtained from cleft palate speakers of different languages. Firstly, an overview of consonants and vowels vulnerable to the cleft condition is presented. Then......, consequences for assessment of cleft palate speech by native versus non-native speakers of a language are discussed, as well as the use of phonemic versus phonetic transcription in cross linguistic studies. Specific recommendations for the construction of speech samples in cross linguistic studies are given....... Finally, the influence of different languages on some aspects of language acquisition in young children with cleft palate is presented and discussed. Until recently, not much has been written about cross linguistic perspectives when dealing with cleft palate speech. Most literature about assessment...

  20. Cleft Lip Repair: The Hybrid Subunit Method.

    Science.gov (United States)

    Tollefson, Travis T

    2016-04-01

    The unilateral cleft lip repair is one of the most rewarding and challenging of plastic surgery procedures. Surgeons have introduced a variety of straight line, geometric, and rotation-advancement designs, while in practice the majority of North American surgeons have been using hybrids of the rotation-advancement techniques. The anatomic subunit approach was introduced in 2005 by Fisher and has gained popularity, with early adopters of the design touting its simplicity and effectiveness. The objectives of this article are to summarize the basic tenets of respecting the philtral subunit, accurate measurement and planning, and tips for transitioning to this subunit approach.

  1. Analysis of Susceptibility Loci for Nonsyndromic Orofacial Clefting in a European Trio Sample

    NARCIS (Netherlands)

    Bohmer, A.C.; Mangold, E.; Tessmann, P.; Mossey, P.A.; Steegers-Theunissen, R.P.M.; Lindemans, J.; Bouwman-Both, M.; Rubini, M.; Franceschelli, P.; Aiello, V.; Peterlin, B.; Molloy, A.M.; Nothen, M.M.; Knapp, M.; Ludwig, K.U.

    2013-01-01

    Nonsyndromic cleft lip with or without cleft palate (NSCL/P), the most common type of orofacial clefting, is one of the most frequent congenital defects. Based on epidemiological data, NSCL/P can be distinguished from nonsyndromic cleft palate only (NSCPO). Both phenotypes have a complex etiology an

  2. Corneal changes in ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome: case series and literature review.

    Science.gov (United States)

    Felipe, Anthony F; Abazari, Azin; Hammersmith, Kristin M; Rapuano, Christopher J; Nagra, Parveen K; Peiro, Baltasar Moratal

    2012-10-01

    The aim of this study is to describe the corneal changes in three unrelated patients with ectrodactyly-ectodermal dysplasia-cleft lip and palate (EEC) syndrome and review the literature on the possible etiology and clinical presentation of similar cases. Case 1 is an 18-year-old female with cleft lip and palate, syndactyly, and bilateral corneal pannus superiorly and inferiorly. She was initially diagnosed and treated as herpes simplex virus keratitis. Case 2 is a 3-year-old female born with cleft lip and palate, absent radial digits in both hands, and bilateral lacrimal stenosis. She developed progressive stromal scarring and neovascularization in both eyes. Her cornea perforated after developing infectious ulceration. Case 3 is a 49-year-old male with cleft palate, claw-hand deformities, absent meibomian glands and lacrimal duct, right ankyloblepharon, and a superior wedge-shaped opacity in the left cornea. The clinical findings demonstrated the different spectrum of keratopathy seen in patients with EEC. All patients were treated medically and without any surgical intervention. Limbal stem cell deficiency (LSCD) is presumed to be the cause in all three cases. Corneal changes in EEC can have variable presentation. LSCD seems to be the etiology of such keratopathy. Recurrent infection from lacrimal drainage obstruction and tear film instability are other risk factors for disease severity and progression. PMID:22618129

  3. ALTERNATIVE APPROACH IN THE TREATMENT OF A GINGIVAL CLEFT ASSOCIATED WITH LABIAL FRENULUM. A 3-YEAR FOLLOW-UP. (Case Report

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    Kamen Kotsilkov

    2015-07-01

    Full Text Available INTRODUCTION: The term Gingival cleft refers to a fissure in the gingival tissues and is usually caused by traumatic oral hygiene, abnormal frenula, trauma from occlusion, orthodontic, or pierce related trauma. Gingival clefts are classified depending on the extent of the inclusion of the gingival thickness into red and white. The recommended treatment approach for the incomplete white clefts is the gingivectomy of the affected keratinized tissue followed by a coronally advanced flap for the root coveradge, while the complete white clefts are treated with a laterally moved, coronally advanced flap, or a free gingival graft procedure. OBJECTIVE: This report presents a case with a spontaneous healing of an incomplete gingival cleft associated with a maxillary labial frenulum after an alternative frenuloectomy approach. METHODS: M.G. (45 with a localized chronic periodontitis, an abnormal papillary maxillary frenulum and an incomplete white cleft on the marginal gingiva of the left central incisor. An alternative frenuloectomy approach combined with a free gingival graft was selected for the correction of the abnormal frenulum. RESULTS:On the sixth month after the surgical procedure an almost complete recovery of the gingival margin was observed.The result at the third year demonstrates a stable gingival margin with a complete tissue recovery. CONCLUSION: The applied combined surgical approach led to a complete gingival recovery of the incomplete white gingival cleft without the commonly used gingivectomy and CAF. Further research is recommended to clarify the adjunctive benefits of the FGG in patients with gingival clefts associated with abnormal frenula.

  4. Status of larynx in children with congenital cleft of upper lip and palate

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    Nigora Makhkamova

    2010-09-01

    Full Text Available Study of 215 children with congenital cleft of upper lip and palate in the age of 1.5 to 15 years has established the organic dysphonia in 27.4% of patients. Of the organic diseases of larynx there were edema, hypertrophy, nodules of vocal folds and chronic laryngitis whose frequency is directly dependent on duration and severity of the defect, age of patient and the timing of recovery of velopharyngeal ring. Pathology of the larynx gradually disappears with the growth of the patient after plastic reconstruction of oronasal defect.

  5. Unfavourable results in the repair of the cleft lip

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    Puthucode V Narayanan

    2013-01-01

    Full Text Available Introduction: Unfavorable results in unilateral and bilateral cleft lip repair are often easy to spot but not always easy to prevent as to treat. We have tried to deal with the more common problems and explain possible causes and the best possible management options from our experience. Unilateral cleft lip repair: Unfavorable results immediately after repair involve Dehiscence and Scaring. Delayed blemishes include vermillion notching, a short lip, deficiency in the height of the lateral vermillion on the cleft side, white roll malalignment, oro-vestibular fistula, the cleft lip nose deformity, a narrow nostril and a "high-riding" nostril. We analyze the causes of these blemishes and outline our views regarding the treatment of these. Bilateral cleft lip: Immediate problems again include dehiscence as also loss of prolabium or premaxilla. Delayed unfavorable results are central vermillion deficiency, a lip that is too tight, bilateral cleft lip nose deformity, problems with the premaxilla and maxillary growth disturbances. Here again we discuss the causation of these problems and our preferred methods of treatment. Conclusion: We have detailed the significant unfavorable results after unilateral and bilateral cleft lip surgery. The methods of treatment advocated have been layer from our own experience.

  6. Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP Part 4: Oral Rehabilitation

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    Jose Alberto de Souza FREITAS

    2013-06-01

    Full Text Available Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients.

  7. Rehabilitative treatment of cleft lip and palate: experience of the Hospital for Rehabilitation of Craniofacial Anomalies/USP (HRAC/USP) - Part 4: oral rehabilitation.

    Science.gov (United States)

    Freitas, José Alberto de Souza; Almeida, Ana Lúcia Pompéia Fraga de; Soares, Simone; Neves, Lucimara Teixeira das; Garib, Daniela Gamba; Trindade-Suedam, Ivy Kiemle; Yaedú, Renato Yassutaka Faria; Lauris, Rita de Cássia Moura Carvalho; Oliveira, Thais Marchini; Pinto, João Henrique Nogueira

    2013-01-01

    Treatment of patients with cleft lip and palate is completed with fixed prostheses, removable, total, implants and aims to restore aesthetics, phonetics and function and should be guided by the basic principles of oral rehabilitation, such as physiology, stability, aesthetics, hygiene and the expectations of the patient. In order to obtain longevity of a prosthetic rehabilitation, the periodontal and dental tissue as well as the biomechanics of the prosthesis are to be respected. The purpose of this article is to describe the types of prosthetics treatment, which are performed at HRAC/USP for the rehabilitation of cleft area in adult patients.

  8. Osteogenesis effect of guided bone regeneration combined with alveolar cleft grafting: assessment by cone beam computed tomography.

    Science.gov (United States)

    Xiao, W-L; Zhang, D-Z; Chen, X-J; Yuan, C; Xue, L-F

    2016-06-01

    Cone beam computed tomography (CBCT) allows for a significantly lower radiation dose than conventional computed tomography (CT) scans and provides accurate images of the alveolar cleft area. The osteogenic effect of guided bone regeneration (GBR) vs. conventional alveolar bone grafting alone for alveolar cleft defects was evaluated in this study. Sixty alveolar cleft patients were divided randomly into two groups. One group underwent GBR using acellular dermal matrix film combined with alveolar bone grafting using iliac crest bone grafts (GBR group), while the other group underwent alveolar bone grafting only (non-GBR group). CBCT images were obtained at 1 week and at 3 months following the procedure. Using Simplant 11.04 software, the bone resorption rate was calculated and compared between the two groups. The bone resorption rate from 1 week to 3 months following bone grafting without the GBR technique was 36.50±5.04%, whereas the bone resorption rate using the GBR technique was 31.69±5.50% (P=0.017). The application of autogenous iliac bone combined with the GBR technique for alveolar bone grafting of alveolar cleft patients can reduce bone resorption and result in better osteogenesis.

  9. Osteogenesis effect of guided bone regeneration combined with alveolar cleft grafting: assessment by cone beam computed tomography.

    Science.gov (United States)

    Xiao, W-L; Zhang, D-Z; Chen, X-J; Yuan, C; Xue, L-F

    2016-06-01

    Cone beam computed tomography (CBCT) allows for a significantly lower radiation dose than conventional computed tomography (CT) scans and provides accurate images of the alveolar cleft area. The osteogenic effect of guided bone regeneration (GBR) vs. conventional alveolar bone grafting alone for alveolar cleft defects was evaluated in this study. Sixty alveolar cleft patients were divided randomly into two groups. One group underwent GBR using acellular dermal matrix film combined with alveolar bone grafting using iliac crest bone grafts (GBR group), while the other group underwent alveolar bone grafting only (non-GBR group). CBCT images were obtained at 1 week and at 3 months following the procedure. Using Simplant 11.04 software, the bone resorption rate was calculated and compared between the two groups. The bone resorption rate from 1 week to 3 months following bone grafting without the GBR technique was 36.50±5.04%, whereas the bone resorption rate using the GBR technique was 31.69±5.50% (P=0.017). The application of autogenous iliac bone combined with the GBR technique for alveolar bone grafting of alveolar cleft patients can reduce bone resorption and result in better osteogenesis. PMID:26876144

  10. 完全性唇腭裂婴儿期手术对上颌骨发育的影响%The effect of early cleft palate repair on maxillary growth in infantile complete cleft lip and palate children

    Institute of Scientific and Technical Information of China (English)

    赵瑞红

    2011-01-01

    Objective: To obsewe the effect of infantile cleft palate repair on maxillary growth in children with complete cleft lip and palate. Methods: 18 children with complete cleft lip and palate were divided into two groups according to whether cleft palate repair was performed or not in infancy. In the one-stage repair group, 10 children received cleft lip and palate repair in infancy, the mean age was (10.15±0.76) years in follow-up. In the cleft lip repair group (10 patients),cleft lip repair was performed without cleft palate surgery in infancy, the mean age of this group was (10.25±0.75) years in follow-up. 20 cases of noncleft children (ages lO) were normal control group. The X-ray cephalometry and dentognathic models of three groups were obtained and compared. Results: The data analysis of cephalometry and dental cast showed that the difference between the two groups had no obvious significance (P>0.05); but compared with the normal group, the cleft lip and palate patients all had the growth inhibition (P<0.05). Conclusion: The cleft palate repair is not the reason of the difference existing;. Earlier cleft palate operation is favorable to functional recovery for infants.%目的:观察婴儿早期行唇腭裂手术对完全性唇腭裂患儿上颌骨发育的近期影响.方法:18例完全性唇腭裂患儿在婴儿期接受唇腭裂手术作为唇腭裂修复组,随访时平均年龄(10.15±0.76)岁;10例未行唇腭裂手术的单纯唇裂患儿为单纯唇腭裂组,随访时平均年龄(10.25±0.75)岁;另20例正常的10岁龄学童作为正常对照组.三组分别取头颅侧位片与上牙颌模型,进行测量分析.结果:两组唇腭裂患儿之间的上颌骨发育无明显差异(P>0.05),与正常对照组比较,均有生长抑制(P<0.05).结论:早期对完全性唇腭裂患儿行唇裂修复术对上颌骨发育的影响不大,还可以使患者获得正常的语言功能.

  11. Genetic determinants of facial clefting: analysis of 357 candidate genes using two national cleft studies from Scandinavia.

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    Astanand Jugessur

    Full Text Available BACKGROUND: Facial clefts are common birth defects with a strong genetic component. To identify fetal genetic risk factors for clefting, 1536 SNPs in 357 candidate genes were genotyped in two population-based samples from Scandinavia (Norway: 562 case-parent and 592 control-parent triads; Denmark: 235 case-parent triads. METHODOLOGY/PRINCIPAL FINDINGS: We used two complementary statistical methods, TRIMM and HAPLIN, to look for associations across these two national samples. TRIMM tests for association in each gene by using multi-SNP genotypes from case-parent triads directly without the need to infer haplotypes. HAPLIN on the other hand estimates the full haplotype distribution over a set of SNPs and estimates relative risks associated with each haplotype. For isolated cleft lip with or without cleft palate (I-CL/P, TRIMM and HAPLIN both identified significant associations with IRF6 and ADH1C in both populations, but only HAPLIN found an association with FGF12. For isolated cleft palate (I-CP, TRIMM found associations with ALX3, MKX, and PDGFC in both populations, but only the association with PDGFC was identified by HAPLIN. In addition, HAPLIN identified an association with ETV5 that was not detected by TRIMM. CONCLUSION/SIGNIFICANCE: Strong associations with seven genes were replicated in the Scandinavian samples and our approach effectively replicated the strongest previously known association in clefting--with IRF6. Based on two national cleft cohorts of similar ancestry, two robust statistical methods and a large panel of SNPs in the most promising cleft candidate genes to date, this study identified a previously unknown association with clefting for ADH1C and provides additional candidates and analytic approaches to advance the field.

  12. Blepharophimosis, ptosis, epicanthus inversus syndrome (BPES and cleft lip and palate. Report of two Brazilian families

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    N.M. Kokitsu-Nakata

    1998-06-01

    Full Text Available We have evaluated a girl and a boy with the blepharophimosis, ptosis and epicanthus inversus syndrome (BPES. The girl presented cleft palate and the boy showed cleft lip and palate as additional clinical signs. Both showed familial recurrence in fourth and third generations, respectively. The other family members also presented blepharophimosis, ptosis, and epicanthus inversus, but without lip and palatal involvement. There were no additional clinical signs nor infertility in these patients. To our knowledge this is the first instance of cleft lip and palate reported as additional signs of the BPES syndrome.Os autores descrevem uma menina e um menino com a síndrome de blefarofimose, ptose e epicanto inverso (BPES. A menina apresentou fissura de palato e o menino mostrou fissura de lábio e palato como sinais clínicos adicionais. Ambos mostraram recorrência familial em quatro e três gerações, respectivamente. Outros membros de ambas as famílias apresentaram também blefarofimose, ptose e epicanto inverso, mas sem envolvimento de lábio e palato. Não existem sinais clínicos adicionais nem infertilidade nestes pacientes. De acordo com o nosso conhecimento, este é o primeiro relato de fissura de lábio e palato registrada como sinal adicional na síndrome BPES.

  13. [Evaluation of the timing of orthodontic arch expansion and graft in cleft lip and palate].

    Science.gov (United States)

    Chang, Le; Wang, Yingnan; Liu, Hongyan

    2016-04-01

    Maxillary transverse growth is inhibited by congenital cleft, early surgical scar strain, and oppression of lipmuscles in patients with cleft lip and palate. Clinical manifestations have shown severely constricted maxilla, insufficientmaxillary width, mismatch of upper and lower dental arches, and crossbite. Alveolar bone graft and arch expansion can effectively correct the deficiency in maxillary width. This paper discusses the timing and success rate of alveolar bone graft, as wellas the relationship between alveolar bone graft and arch expansion. Secondary alveolar bone graft is optimally performed beforepermanent canine eruption, especially when the teeth have formed between half and three quarters of their roots. Rapid maxillaryexpansion prior to alveolar bone graft is beneficial because this process increases the gap of the cleft, expands bone graft, andreduces the difficulty. However, the stability of this process remains controversial. Small-scale studies have reported that rapidmaxillary expansion after alveolar bone graft can open the midpalatal suture without bone graft loss. Slow maxillary expansioncan provide continuous light forces to reconstruct the bone. However, these studies are coordinated with fixed orthodontictreatment. Further research is necessary to determine the effects of maxillary expansion on long-term stability of teeth. PMID:27337934

  14. Modified technique of presurgical infant maxillary orthopedics for complete unilateral cleft lip and palate.

    Science.gov (United States)

    Choo, Hyeran; Maguire, Meg; Low, David W

    2012-01-01

    This article introduces a modified device fabrication and facial taping method that increases the efficiency and efficacy of presurgical infant maxillary orthopedic therapy for babies born with complete cleft lip and palate. Interarch and intra-arch relationships of the maxillary and mandibular dental arches were evaluated on mounted stone models before and after treatment. The palatal plate device was custom-fabricated in a manner that bypassed the need for periodic acrylic addition and removal, thereby eliminating the risk of natural maxillary growth restriction during therapy. Elastic labial tapes were fabricated and applied in a configuration that mimicked normal function of the orbicularis oris muscle. A nasal stent wire was utilized from the initiation of therapy to enhance intraoral retention and stability of the device. Examples of infants undergoing a unilateral complete cleft lip and palate treatment protocol are presented. Treatment objectives were achieved within 7 to 8 weeks of therapy for patients who had an initial alveolar cleft size more than 10 to 12 mm. The modified protocol of presurgical infant maxillary orthopedic therapy is an effective and efficient treatment modality in reversing the pre-existing orofacial dysmorphism by redirecting the infant's natural growth.

  15. 3D Printed Models of Cleft Palate Pathology for Surgical Education

    Science.gov (United States)

    Lioufas, Peter A.; Quayle, Michelle R.; Leong, James C.

    2016-01-01

    Objective: To explore the potential viability and limitations of 3D printed models of children with cleft palate deformity. Background: The advantages of 3D printed replicas of normal anatomical specimens have previously been described. The creation of 3D prints displaying patient-specific anatomical pathology for surgical planning and interventions is an emerging field. Here we explored the possibility of taking rare pediatric radiographic data sets to create 3D prints for surgical education. Methods: Magnetic resonance imaging data of 2 children (8 and 14 months) were segmented, colored, and anonymized, and stereolothographic files were prepared for 3D printing on either multicolor plastic or powder 3D printers and multimaterial 3D printers. Results: Two models were deemed of sufficient quality and anatomical accuracy to print unamended. One data set was further manipulated digitally to artificially extend the length of the cleft. Thus, 3 models were printed: 1 incomplete soft-palate deformity, 1 incomplete anterior palate deformity, and 1 complete cleft palate. All had cleft lip deformity. The single-material 3D prints are of sufficient quality to accurately identify the nature and extent of the deformities. Multimaterial prints were subsequently created, which could be valuable in surgical training. Conclusion: Improvements in the quality and resolution of radiographic imaging combined with the advent of multicolor multiproperty printer technology will make it feasible in the near future to print 3D replicas in materials that mimic the mechanical properties and color of live human tissue making them potentially suitable for surgical training. PMID:27757345

  16. CORRECTION OF ALVEOLAR ARCH MALALIGNMENT AFTER TOTAL CORRECTION IN UNILATERAL AND BILATERAL GR III CLEFTS

    Directory of Open Access Journals (Sweden)

    Mohana Rao

    2015-06-01

    Full Text Available AIMS AND OBJECTIVES: Aim of this study is to document and analyse the pre - operative and postoperative alveolar arch impressions . MATERIALS AND METHODS: 20 cases of both unilateral and bilateral GR III clefts selected for this study. These pati ents were operated at the age of not less than 9 months, preferably below the age of 18 months. Below the age of 9 months general condition of these children may not cope up the duration of general anesthesia and amount of surgical trauma. After intubating the patient preoperatively, with the help of custom made metal arch plates, at first, Medical grade Alginate material with optimal hydration used to get negative impression, from these , positive impressions were obtained by the use of optimally hydrated m edical grade stone powder routinely used by dental surgeons. These positive impressions along with negative impression s and metal plates allowed to settle down for 10 hours undisturbed, after that positive impressions retrieved carefully without breaking a nd stored for analysis. Similarly postoperative impressions were taken after a gap of 2 years in the same manner like that of pre - operative ones under general anesthesia. Both impressions w ere compared and analysis was done regarding alveolar arch malalign ment, asymmetry and arch collapse correction. RESULTS & CONCLUSION: The status of the alveolar arch after single stage operation for cleft lip and palate in the unilateral cleft was much improved and there was considerable correction of the arch collapse a s well; in the bilateral cleft the correction of the premaxillary protrusion was better appreciated than the degree of correction of the alveolar gap.

  17. Orthodontically guided bone transport in the treatment of alveolar cleft: A case report

    Science.gov (United States)

    Gómez, Elena; Otero, Marta; Berraquero, Rosario; Wucherpfennig, Begona; Hernández-Godoy, Juan; Guiñales, Jorge; Vincent, Germán; Burgueño, Miguel

    2016-01-01

    Introduction Conventional treatments are sometimes not possible in certain alveolar cleft cases due to the severity of the gap which separates the fragments. Various management strategies have been proposed, including sequential surgical interventions or delaying treatment until adulthood to then carry out maxillary osteotomies. A further alternative approach has also been proposed, involving the application of bone transport techniques to mobilise the osseous fragments and thereby reduce the gap between lateral fragments and the premaxilla. Case Report We introduce the case of a 10-year-old patient who presented with a bilateral alveolar cleft and a severe gap. Stable occlusion between the premaxilla and the mandible was achieved following orthodontic treatment, making it inadvisable to perform a retrusive osteotomy of the premaxilla in order to close the alveolar clefts. Faced with this situation, it was decided we would employ a bone transport technique under orthodontic guidance using a dental splint. This would enable an osseous disc to be displaced towards the medial area and reduce the interfragmentary distance. During a second surgical intervention, closure of the soft tissues was performed and the gap was filled in using autogenous bone. Conclusions The use of bone transport techniques in selected cases allows closure of the osseous defect, whilst also preserving soft tissues and reducing the amount of bone autograft required. In our case, we were able to respect the position of the premaxilla and, at the same time, generate new tissues at both an alveolar bone and soft tissue level with results which have remained stable over the course of time. Key words:Alveolar cleft, bone transport, graft. PMID:26855699

  18. Comparison of patients’ age receiving therapeutic services in a cleft care team in Isfahan

    Science.gov (United States)

    Soheilipour, Saeed; Soheilipour, Fatemeh; Derakhshandeh, Fatemeh; Hashemi, Hedieh; Memarzadeh, Mehrdad; Salehiniya, Hamid; Soheilipour, Fahimeh

    2016-01-01

    Background: Due to numerous difficulties in patients suffering from varieties of cleft lip and palate, their therapeutic management involves interdisciplinary teamwork. This study was conducted to compare the age of commencing treatments such as speech therapy, secondary palate and alveolar bone grafting and orthodontics between those who sought treatment early and late. Materials and Methods: In this retrospective study, 260 files of patients with cleft lip and palate based on their age at the time of admission to a cleft care team were divided into two groups: The early admission and late admission. Both groups compared based on four variables including the mean age of beginning speech therapy, palatal secondary surgery, alveolar bone grafting, and receiving orthodontics using t-test. Results: Based on the results, among 134 patients admitted for speech therapy, the mean age of initiating speech therapy in early clients was 3.3 years, and in the late ones was 9 years. Among 47 patients with secondary surgery, the mean age in early clients was 3.88 years, and in the late clients was 15.7 years. Among 17 patients with alveolar bone grafting, the mean age in the first group was 9 years, and in the other was 16.69 years. Among 24 patients receiving orthodontic services, the mean age in early clients was 7.66 years, and in the second group was 17.05 years. Conclusion: There was a significant difference between the age of performing secondary surgery and alveolar bone grafting and the age of beginning speech therapy and receiving orthodontic services in early references and late references to the team. PMID:27274350

  19. A genome-wide association study of cleft lip with and without cleft palate identifies risk variants near MAFB and ABCA4

    DEFF Research Database (Denmark)

    Beaty, Terri H; Murray, Jeffrey C; Marazita, Mary L;

    2010-01-01

    Case-parent trios were used in a genome-wide association study of cleft lip with and without cleft palate. SNPs near two genes not previously associated with cleft lip with and without cleft palate (MAFB, most significant SNP rs13041247, with odds ratio (OR) per minor allele = 0.704, 95% CI 0...... and ABCA4. Expression studies support a role for MAFB in palatal development....

  20. The role of the psychologist as a member of the cleft and craniofacial team in looking after patients and their families from the perspective of the literature and clinical experience.

    Science.gov (United States)

    Dutkiewicz-Częścik, Kinga

    2014-01-01

    The aim of the paper is to present the psychological situation of the family of a child with a craniofacial condition, as well as to describe the role of the psychologist in the therapeutic process. The perspective of the paper is based on both the author's clinical experience and a review of the literature. The paper adopted the parents' perspective and is therefore focused on their experiences concerning the diagnosis of the congenital craniofacial malformation. The author made an attempt to define what comprises a problem for parents and identify how their difficulties may influence the child's psychological development. Attention was devoted to challenges stemming from the unknown etiology of the malformation, difficulties in accepting and conducting the multistage treatment, changes in the look of the face, and the broadly considered imperfection. In the review of the literature, particular attention was drawn to identifying the risk factors for psychological development disorders associated with the cleft condition, as well as factors concerning the risk of the disorder influencing the parent-child relationship. It was concluded that the quality of that relationship may be influenced by the psychological state of the parents. Relying upon both the literature and own research results, it was established that the parents' psychological situation can be classified as atrauma. Its significance for the parent-child relationship was identified - especially in reference to parental sensitivity to the child's needs and their ability to support the child. The parent-child relationship may be meaningful for the child's psychological development - especially his or her emotions, self-esteem, and perception of the world. The basic idea was that the parents experience a period of mourning over the imagined "ideal" child, going through which is crucial for them to adapt to their role of parenting a disfigured one. Adjusting to the requirements of the situation, i

  1. Ankyloglossia with cleft lip: A rare case report

    Science.gov (United States)

    Jangid, Kritika; Alexander, Aurelian Jovita; Jayakumar, Nadathur Doraiswamy; Varghese, Sheeja; Ramani, Pratibha

    2015-01-01

    Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development. PMID:26941523

  2. Cleft lip as a presentation of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Richa Gupta

    2012-01-01

    Full Text Available Congenital syphilis may present with unusual symptoms in early stages which needs to be identified for prompt treatment. Here, we present a case of 13-day-old female child with congenital syphilis presenting with cleft lip.

  3. Helping parents cope with a cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Jenny Steyn

    1980-09-01

    Full Text Available For the layman, the area of cleft palate is one shrouded in mystery and half-knowledge. An unrepaired cleft lip and palate is an unpleasant, if not alarming sight, and parents are not always totally convinced by the reassuring words of doctors and nurses that “ plastic surgeons do a marvellous job on cleft palate babies.” They need proof that their baby will indeed look and be like any other baby within a few months. Equally important, they need reassurance that whatever feelings they may experience when their babies are born are natural and appropriate ones. As the baby grows and develops parents may need practical assistance, emotional support and information regarding their baby’s condition. It is essentially for these reasons that a fellowship group has been established under the auspices of SAIDA (Southern Africa Inherited Disorders Association to help parents and families of cleft lip and palate children.

  4. Changing lifestyles and oral clefts occurrence in Denmark

    DEFF Research Database (Denmark)

    Bille, Camilla; Knudsen, Lisbeth B.; Christensen, Kaare

    2005-01-01

    ; furthermore, smoking among pregnant women decreased considerably. Design and settings There are few places in which ecological studies of oral clefts are possible. Denmark provides a particularly good setting for this kind of study, due to a high ascertainment and a centralized registration of cleft cases...... births, 95% CI=1.37 to 1.52). The introduction of folic acid and the decrease in smoking prevalence among pregnant women do not seem to have reduced the birth prevalence negatively. This may be due to non-compliance in respect to the folic acid recommendation and/or only a weak causal association between......The aim of this project was to access whether any changes in the birth prevalence of cleft lip with/without cleft palate (CL(P)) occurred in Denmark during the period 1988 through 2001. In this period an official recommendation of a supplementation of folic acid to pregnant women was introduced...

  5. Proteomic Analysis of Unbounded Cellular Compartments: Synaptic Clefts.

    Science.gov (United States)

    Loh, Ken H; Stawski, Philipp S; Draycott, Austin S; Udeshi, Namrata D; Lehrman, Emily K; Wilton, Daniel K; Svinkina, Tanya; Deerinck, Thomas J; Ellisman, Mark H; Stevens, Beth; Carr, Steven A; Ting, Alice Y

    2016-08-25

    Cellular compartments that cannot be biochemically isolated are challenging to characterize. Here we demonstrate the proteomic characterization of the synaptic clefts that exist at both excitatory and inhibitory synapses. Normal brain function relies on the careful balance of these opposing neural connections, and understanding how this balance is achieved relies on knowledge of their protein compositions. Using a spatially restricted enzymatic tagging strategy, we mapped the proteomes of two of the most common excitatory and inhibitory synaptic clefts in living neurons. These proteomes reveal dozens of synaptic candidates and assign numerous known synaptic proteins to a specific cleft type. The molecular differentiation of each cleft allowed us to identify Mdga2 as a potential specificity factor influencing Neuroligin-2's recruitment of presynaptic neurotransmitters at inhibitory synapses. PMID:27565350

  6. Ankyloglossia with cleft lip: A rare case report

    Directory of Open Access Journals (Sweden)

    Kritika Jangid

    2015-01-01

    Full Text Available Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development.

  7. Ankyloglossia with cleft lip: A rare case report.

    Science.gov (United States)

    Jangid, Kritika; Alexander, Aurelian Jovita; Jayakumar, Nadathur Doraiswamy; Varghese, Sheeja; Ramani, Pratibha

    2015-01-01

    Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman syndrome or Simpson-Golabi-Behmel syndrome. This article presents a rare case of ankyloglossia associated with cleft lip treated with diode laser in a 12-year-old Indian boy who had undergone surgical correction of associated cleft lip soon after birth. Correction of ankyloglossia at a young age would lead to enhanced phonetics, improved oral hygiene, and overall personality development.

  8. Computational Embryology and Predictive Toxicology of Cleft Palate

    Science.gov (United States)

    Capacity to model and simulate key events in developmental toxicity using computational systems biology and biological knowledge steps closer to hazard identification across the vast landscape of untested environmental chemicals. In this context, we chose cleft palate as a model ...

  9. A modified presurgical orthopedic (nasoalveolar molding) device in the treatment of unilateral cleft lip and palate.

    Science.gov (United States)

    Subramanian, Chitravelu Siva; Prasad, N K K Koteswara; Chitharanjan, Arun B; Liou, Eric Jein Wein

    2016-01-01

    Nasoalveolar molding (NAM) can be done effectively to reshape the nasal cartilage and mold the maxillary dentoalveolar arch before surgical cleft lip repair and primary rhinoplasty. Presurgical NAM helps as an adjunct procedure to enhance the esthetic and functional outcome of the surgical procedures. We have developed a modified NAM device to suit to the needs of the patients coming from distant places for the treatment. This device helps in reducing the number of frequent visits the patient needs to take to the craniofacial center. The purpose of this presentation is to report this treatment technique and discuss its application. PMID:27403068

  10. Un Futuro Prometedor para su Nino con Labio Hendido y Paladar Hendido. Edicion Refundida (Bright Promise for Your Child with Cleft Lip and Cleft Palate. Revised Edition).

    Science.gov (United States)

    McDonald, Eugene T.; Berlin, Asa J.

    The booklet, written in Spanish, is intended to help parents of babies with cleft lip and/or cleft palate. Topics covered include the sequence of prenatal development and the effects of birth defects, common misconceptions about what causes the conditions, possible hereditary and environmental causes, and what it means to have a cleft palate or a…

  11. Ankyloglossia with cleft lip: A rare case report

    OpenAIRE

    Kritika Jangid; Aurelian Jovita Alexander; Nadathur Doraiswamy Jayakumar; Sheeja Varghese; Pratibha Ramani

    2015-01-01

    Ankyloglossia or tongue-tie is a congenital anomaly affecting the tongue, which is characterized by thick, short lingual frenulum. This condition causes many difficulties such as limited tongue protrusion, breastfeeding difficulties, speech impairment and lack of self-confidence. It is very rarely associated with any other congenital craniofacial disorders such as cleft lip, X-linked cleft palate, Van der Woude syndrome, Smith-Lemli-Opitz syndrome, Orofacial digital syndrome, Beckwith Weidman...

  12. EEC syndrome sans clefting: Variable clinical presentations in a family

    OpenAIRE

    Thakkar Sejal; Marfatia Yogesh

    2007-01-01

    Ectrodactyly, ectodermal dysplasia and cleft palate/lip syndrome (EEC) is a rare autosomal dominant syndrome with varied presentation and is actually a multiple congenital anomaly syndrome leading to intra- and interfamilial differences in severity because of its variable expression and reduced penetrance. The cardinal features include ectrodactyly, sparse, wiry, hypopigmented hair, peg-shaped teeth with defective enamel and cleft palate/lip. A family comprising father, daughter and son prese...

  13. Ectrodactyly, cleft lip and palate in two half sibs.

    OpenAIRE

    Lewis, M B; Pashayan, H M

    1981-01-01

    Two half sibs with bilateral complete cleft lip and complete cleft of the palate associated with ectrodactyly of the hands and feet, born to the same phenotypically normal mother, are reported. The younger of the two sibs also has dominantly inherited tremors (also referred to as essential heredofamilial tremors) as did her biological father. Possible genetic causes to explain the recurrence of the facial and limb malformations in the half sibs with additional central nervous system malformat...

  14. A case report of brachial cleft cyst in the neck

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Young Mi; Choi, Soon Chul; Park, Tae Won; You, Dong Soo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1993-08-15

    Branchial cleft cyst is the most common lateral neck cyst ; the vast majority are of the second branchial cleft origin. This presumably reflects the greater depth and longer persistence of the second cleft, compared with the first, third, and fourth clefts. We experienced a 49-year-old male whose chief complaint was a abnormal mass of the cleft parotid gland area and neck. As a result of careful analysis of clinical, radiological, and histopathological findings. We diagnosed it as a second branchial cleft cyst in the neck and obtained results as follows: 1. In clinical examination, there was a 10 X 15 cm sized, fluctuant painful mass in the left neck and parotid area. 2. In radiographic examination, a low echogenic mass with internal cystic change in the inferior parotid gland area was noted sonographically. Computed tomograph showed a 3 X 4 cm sized, well-defined cystic mass with heterogenous solid component in the anterior border of sternocleidomastoid muscle. MRI revealed 5 X 6 cm sized, well-marginated multiseparated mass in the same area. 3. In histopathological examination, lining of cyst was stratified squamous epithelium with typical lymph node pattern and inflammatory cell infiltration.

  15. A case report of brachial cleft cyst in the neck

    International Nuclear Information System (INIS)

    Branchial cleft cyst is the most common lateral neck cyst ; the vast majority are of the second branchial cleft origin. This presumably reflects the greater depth and longer persistence of the second cleft, compared with the first, third, and fourth clefts. We experienced a 49-year-old male whose chief complaint was a abnormal mass of the cleft parotid gland area and neck. As a result of careful analysis of clinical, radiological, and histopathological findings. We diagnosed it as a second branchial cleft cyst in the neck and obtained results as follows: 1. In clinical examination, there was a 10 X 15 cm sized, fluctuant painful mass in the left neck and parotid area. 2. In radiographic examination, a low echogenic mass with internal cystic change in the inferior parotid gland area was noted sonographically. Computed tomograph showed a 3 X 4 cm sized, well-defined cystic mass with heterogenous solid component in the anterior border of sternocleidomastoid muscle. MRI revealed 5 X 6 cm sized, well-marginated multiseparated mass in the same area. 3. In histopathological examination, lining of cyst was stratified squamous epithelium with typical lymph node pattern and inflammatory cell infiltration.

  16. Psychological status as a function of residual scarring and facial asymmetry after surgical repair of cleft lip and palate.

    Science.gov (United States)

    Millar, Keith; Bell, Aileen; Bowman, Adrian; Brown, Denise; Lo, Tsz-Wai; Siebert, Paul; Simmons, David; Ayoub, Ashraf

    2013-03-01

    Objective : Objective measure of scarring and three-dimensional (3D) facial asymmetry after surgical correction of unilateral cleft lip (UCL) and unilateral cleft lip (UCLP). It was hypothesized that the degree of scarring or asymmetry would be correlated with poorer psychological function. Design : In a cross-sectional design, children underwent 3D imaging of the face and completed standardized assessments of self-esteem, depression, and state and trait anxiety. Parents rated children's adjustment with a standard scale. Setting : Glasgow Dental School, School of Medicine, College of Medical, Veterinary and Life Sciences. Patients : Fifty-one children aged 10 years with UCLP and 43 with UCL were recruited from the cohort treated with the surgical protocol of the CLEFTSIS managed clinical network in Scotland. Methods : Objective assessment to determine the luminance and redness of the scar and facial asymmetry. Depression, anxiety, and a self-esteem assessment battery were used for the psychological analysis. Results : Cleft cases showed superior psychological adjustment when compared with normative data. Prevalence of depression matched the population norm. The visibility of the scar (luminance ratio) was significantly correlated with lower self-esteem and higher trait anxiety in UCLP children (P  =  .004). Similar but nonsignificant trends were seen in the UCL group. Parental ratings of poorer adjustment also correlated with greater luminance of the scar. Conclusions : The objectively defined degree of postoperative cleft scarring was associated with subclinical symptoms of anxiety, depression, and low self-esteem. PMID:21846256

  17. Centralisation of services for children with cleft lip or palate in England: a study of hospital episode statistics

    Directory of Open Access Journals (Sweden)

    Fitzsimons Kate J

    2012-06-01

    Full Text Available Abstract Background In 1998, a process of centralisation was initiated for services for children born with a cleft lip or palate in the UK. We studied the timing of this process in England according to its impact on the number of hospitals and surgeons involved in primary surgical repairs. Methods All live born patients with a cleft lip and/or palate born between April 1997 and December 2008 were identified in Hospital Episode Statistics, the database of admissions to English National Health Service hospitals. Children were included if they had diagnostic codes for a cleft as well as procedure codes for a primary surgical cleft repair. Children with codes indicating additional congenital anomalies or syndromes were excluded as their additional problems could have determined when and where they were treated. Results We identified 10,892 children with a cleft. 21.0% were excluded because of additional anomalies or syndromes. Of the remaining 8,606 patients, 30.4% had a surgical lip repair only, 41.7% a palate repair only, and 28.0% both a lip and palate repair. The number of hospitals that carried out these primary repairs reduced from 49 in 1997 to 13, with 11 of these performing repairs on at least 40 children born in 2008. The number of surgeons responsible for repairs reduced from 98 to 26, with 22 performing repairs on at least 20 children born in 2008. In the same period, average length of hospital stay reduced from 3.8 to 3.0 days for primary lip repairs, from 3.8 to 3.3 days for primary palate repairs, and from 4.6 to 2.6 days for combined repairs with no evidence for a change in emergency readmission rates. The speed of centralisation varied with the earliest of the nine regions completing it in 2001 and the last in 2007. Conclusions Between 1998 and 2007, cleft services in England were centralised. According to a survey among patients’ parents, the quality of cleft care improved in the same period. Surgical care became more

  18. Variable expressivity in Patau syndrome is not all related to trisomy 13 mosaicism.

    Science.gov (United States)

    Hsu, Hui-Fang; Hou, Jia-Woei

    2007-08-01

    Patau syndrome (trisomy 13) is very rare in live-born babies. Individuals with this chromosomal syndrome have a short lifespan and are rarely seen beyond infancy. This study is aimed at the clinical spectrum, natural history, and survival of patients with trisomy 13. We reviewed the detailed data of 13 Patau syndrome live-born babies. Among them two individuals were delivered from continuation of pregnancy even after prenatal diagnosis. The remaining 11 patients were born to younger mothers who did not undergo amniocentesis because no major anomalies except for cleft lip/palate were found on prenatal sonograms. The common features of Patau syndrome including the clinical triad (microphthalmia, cleft lip/palate, and polydactyly) and non-cyanotic heart defects were always found in our series. However, certain serious central defects (holoprosencephaly, omphalocele, and single umbilical artery), which are easily recognized from prenatal sonogram, occurred less frequently than those stated in the literature. The median survival time was 95 days and was longer than that previously reported. There were two infants with trisomic mosaicism with different outcomes in both clinical spectrum and survival. Otherwise, we also found the increased recurrence risks of aneuploidy in two individuals, and the longest survivor (84 months) of non-mosaic trisomy 13 in Taiwan. We thus suggest that long-term survival in our series is strongly correlated with different expressivity after prenatal selection, in addition to cytogenetic mosaicism. Less associated anomalies such as polyhydramnios, oligohydramnios, intrauterine growth retardation, single umbilical artery, eye defects, holoprosencephaly, omphalocele, and polycystic kidney may contribute to their clinical courses. PMID:17603803

  19. Secondary bilateral cleft lip-nose deformity correction by rhinoplasty with simultaneous Abbe flap

    Directory of Open Access Journals (Sweden)

    Nitin J Mokal

    2014-01-01

    Full Text Available Aim: The purpose of this article is to review modification and outcome of secondary rhinoplasty along with Abbι flap for correction of secondary bilateral cleft lip deformity. Materials and Methods: A total of thirteen patients of secondary bilateral cleft lip-nose deformity having tight upper lip, lack of acceptable philtral column, Cupid′s bow definition, irregular lip scars, and associated nasal deformity were selected. All the patients received Abbι flap and simultaneous nasal correction. All cases were treated during a period of three years. Mean patient age at the time of the operation was 21 years, and ranged from 16 to 27 years. The average follow-up period was three years. Results: Assessment of results was based on comparing preoperative and postoperative clinical photographs done by surgeon and patient relatives and patient satisfaction questionnaires. The columellar lengthening and upper lip vermillion correction achieved was satisfactory. There were no perioperative complications such as airway obstruction, bleeding, infection, wound disruption, or flap necrosis.

  20. Postoperative analgesia for cleft lip and palate repair in children

    Science.gov (United States)

    Reena; Bandyopadhyay, Kasturi Hussain; Paul, Abhijit

    2016-01-01

    Acute pain such as postoperative pain during infancy was ignored approximately three decades ago due to biases and misconceptions regarding the maturity of the infant's developing nervous system, their inability to verbally report pain, and their perceived inability to remember pain. More recently, these misconceptions are rarely acknowledged due to enhanced understanding of the developmental neurobiology of infant pain pathways and supraspinal processing. Cleft lip and palate is one of the most common congenital abnormalities requiring surgical treatment in children and is associated with intense postoperative pain. The pain management gets further complicated due to association with postsurgical difficult airway and other congenital anomalies. Orofacial blocks like infraorbital, external nasal, greater/lesser palatine, and nasopalatine nerve blocks have been successively used either alone or in combinations to reduce the postoperative pain. Since in pediatric population, regional anesthesia is essentially performed under general anesthesia, association of these two techniques has dramatically cut down the risks of both procedures particularly those associated with the use of opioids and nonsteroidal anti-inflammatory drugs. Definitive guidelines for postoperative pain management in these patients have not yet been developed. Incorporation of multimodal approach as an institutional protocol can help minimize the confusion around this topic. PMID:27006533

  1. Three Familial Cases with Ectrodactyly Ectodermal Displazia-Clefting Syndrome

    Directory of Open Access Journals (Sweden)

    Selma Bakar Dertlioğlu

    2012-03-01

    Full Text Available A family of four children where mother and father had history of consanguineous marriage presented at our clinic due to lack of hair growth, thinning of all body hair and finger deformities in their three daughters aged 10 years, six years and three months. Dermatological examination of the cases showed thinning of the hair on the scalp, eyebrows, and extremities, subungual hyperkeratosis in nails, palmoplantar hyperkeratosis, cutaneous xerosis and loss of sweating, conical appearance and hypodontia in the teeth. All three siblings were seen to have the 3rd and 4th digits on the hand and the 2nd, 3rd and 4th digits on the feet to be proximally conjoined. Histopathologic examination of the cutaneous biopsy materials taken from the patients revealed a decrease in the number and size of hair follicles and sebaceous glands. We present these familial cases who were diagnosed with Ectrodactyly-ectodermal dysplasia-clefting syndrome as a result of clinical and histopathologic evaluation due to their rarity.

  2. 成批唇腭裂患儿的治疗%Treatment in batches for children with cleft lip and palate

    Institute of Scientific and Technical Information of China (English)

    傅跃先; 甘立强; 邱林; 田晓菲; 刘燕; 向代理

    2009-01-01

    Objective:To summarize the experience on treatment in batches for the child patients with cleft lip and palates, including pre-operative preparation, communication with the parents, operative technique, postoperative care and follow-up. Methods: The 745 cases were treated in batches, including 180 cases of cleft lip,373 cases of clef palate and 192 cases of cleft lip companied with cleft palate. Preoperative preparation were completed according to medical treatment criterion.The knowledge of the sequence treatment of cleft lip and palate was informed to the parents. The individual surgery treatment was operated to improve effects in routine way. All cases received the routine postoperative care. Results: All patients left hospital with primary healing of wound. The patients with cleft lip had a good wound healing and a good diaplasis of anatomic sign. The patients with cleft palate had a good wound healing too, without palate fistulas and the uvulas were reconstructed well. The phonetic function was improved to certain extent. The parents of the patients were satisfied. Conclusion: It is feasible to treat in batches the patients with cleft lip and palate in the hospital, which has a good conditions of surgery, anaesthesia and nursing.%目的:总结成批收治唇腭裂患儿过程中,围术期处理与手术治疗的特点和经验.方法:成批收治唇腭裂患儿745例,其中单纯唇裂180例,腭裂373例,唇裂伴腭裂192例.术前准备按规范的医疗程序进行,注重医患沟通.手术按常规方式操作,并寻求个体化处理,以提高整复质量.结果:所有病例均一期愈合出院.唇裂患儿创口愈合好,解剖标志复位.腭裂患儿腭部愈合好,悬雍垂重建良好,无腭瘘发生,语言功能有不同程度改善.患儿家长均感满意.结论:在具备可靠的手术、麻醉与护理条件的单位,成批收治唇腭裂患儿是可行的.

  3. Getting under the skin of p63.

    OpenAIRE

    Rinne, T.K.

    2010-01-01

    Mutations in the transcription factor gene p63 are causative for human developmental syndromes characterized by three main hallmarks: ectodermal dysplasia, limb malformations and orofacial clefting. Five different dominantly inherited human syndromes and two non-syndromic conditions have been linked to p63 gene defects. Ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome (EEC) is the most common p63-associated condition, since approximately 60 percent of all p63 mutations have be...

  4. p63 Gene Mutations in EEC Syndrome, Limb-Mammary Syndrome, and Isolated Split Hand–Split Foot Malformation Suggest a Genotype-Phenotype Correlation

    OpenAIRE

    van Bokhoven, Hans; Hamel, Ben C. J.; Bamshad, Mike; Sangiorgi, Eugenio; Gurrieri, Fiorella; Duijf, Pascal H. G.; Vanmolkot, Kaate R. J.; van Beusekom, Ellen; van Beersum, Sylvia E. C.; Celli, Jacopo; Merkx, Gerard F. M.; Tenconi, Romano; Fryns, Jean Pierre; Verloes, Alain; Newbury-Ecob, Ruth A.

    2001-01-01

    p63 mutations have been associated with EEC syndrome (ectrodactyly, ectodermal dysplasia, and cleft lip/palate), as well as with nonsyndromic split hand–split foot malformation (SHFM). We performed p63 mutation analysis in a sample of 43 individuals and families affected with EEC syndrome, in 35 individuals affected with SHFM, and in three families with the EEC-like condition limb-mammary syndrome (LMS), which is characterized by ectrodactyly, cleft palate, and mammary-gland abnormalities. Th...

  5. p63-associated disorders.

    OpenAIRE

    Rinne, T.K.; Brunner, H.G.; Bokhoven, J.H.L.M. van

    2007-01-01

    Heterozygous mutations in the transcription factor gene p63 are causative for several syndromes, with ectodermal dysplasia, orofacial clefting and limb malformations as the key characteristics. Different combinations of these features are seen in five different syndromes, of which ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome (EEC) is the most common one. Mutations in p63 can also cause non-syndromic single malformations, such as split hand foot malformation (SHFM4) and iso...

  6. Association of TFAP2A gene polymorphism with susceptibility to non-syndromic cleft lip with or without palate risk in south Indian population.

    Science.gov (United States)

    Babu Gurramkonda, Venkatesh; Syed, Altaf Hussain; Murthy, Jyotsna; V K S Lakkakula, Bhaskar

    2016-09-01

    The aetiology of non-syndromic cleft lip with or without cleft palate (NSCL/P) is complex involving multiple interacting genes and environmental factors. The primary objective of the present study was to investigate the role of TFAP2A gene single nucleotide polymorphisms (SNPs) in the pathogenesis of NSCL/P. In this study, 173 unrelated NSCL/P patients and 176 controls without clefts were genotyped with TFAP2A rs1675414 (Exon 1), rs3798691 (Intron 1), and rs303050 (Intron 4) variants by allele-specific amplification using the KASPar SNP genotyping system. The method of multifactor dimensionality reduction (MDR) was used to analyze gene-gene interactions. TFAP2A polymorphisms are not found to be associated with non-syndromic cleft lip with or without cleft palate (NSCL/P) at either the genotype or allele levels. No linkage disequilibrium (LD) was found between TFAP2A variants. MDR analysis did not show a significant effect of the TFAP2A gene polymorphisms on susceptibility to NSCL/P (p > 0.05). These results suggest that the analyzed variations in TFAP2A gene might not be associated with NSCL/P pathogenesis in south Indian population. PMID:27617216

  7. Association of TFAP2A gene polymorphism with susceptibility to non-syndromic cleft lip with or without palate risk in south Indian population

    Directory of Open Access Journals (Sweden)

    Venkatesh Babu Gurramkonda

    2016-09-01

    Full Text Available The aetiology of non-syndromic cleft lip with or without cleft palate (NSCL/P is complex involving multiple interacting genes and environmental factors. The primary objective of the present study was to investigate the role of TFAP2A gene single nucleotide polymorphisms (SNPs in the pathogenesis of NSCL/P. In this study, 173 unrelated NSCL/P patients and 176 controls without clefts were genotyped with TFAP2A rs1675414 (Exon 1, rs3798691 (Intron 1, and rs303050 (Intron 4 variants by allele-specific amplification using the KASPar SNP genotyping system. The method of multifactor dimensionality reduction (MDR was used to analyze gene-gene interactions. TFAP2A polymorphisms are not found to be associated with non-syndromic cleft lip with or without cleft palate (NSCL/P at either the genotype or allele levels. No linkage disequilibrium (LD was found between TFAP2A variants. MDR analysis did not show a significant effect of the TFAP2A gene polymorphisms on susceptibility to NSCL/P (p > 0.05. These results suggest that the analyzed variations in TFAP2A gene might not be associated with NSCL/P pathogenesis in south Indian population.

  8. Cleft lip and palate review: Epidemiology, risk factors, quality of life, and importance of classifications

    Directory of Open Access Journals (Sweden)

    Laureen Supit

    2008-12-01

    Full Text Available Cleft lip with or without cleft palate is the most occurring craniofacial anomaly in human, resulting from a complex etiology involving multiple genetic and environmental factors. The defect carries lifelong morbidity and economic burden. Children with clefts will require continuous medical interventions for at least the first 18 years of life, affecting many aspects of their lives. The extent and complexity of clefts vary infinitely, later determining individual management and outcome. Identification and classification play significant roles in initial assessment of these unique cleft cases, which affect options for following correctional attempts. Some classifications even allow measurement of progress after anatomical repositioning, and success rate after surgical repairs. The challenge of developing one such widely inclusive classification is discussed. (Med J Indones 2008; 17: 226-39Keywords: Cleft lip, cleft palate, congenital anomaly, cleft  classfications

  9. Cleft-lift operation for pilonidal sinuses under tumescent local anesthesia

    DEFF Research Database (Denmark)

    Bertelsen, Claus Anders

    2011-01-01

    The use of tumescent local anesthesia in the Bascom cleft-lift procedure has not been described before.......The use of tumescent local anesthesia in the Bascom cleft-lift procedure has not been described before....

  10. Subphenotyping and Classification of Orofacial Clefts: Need for Orofacial Cleft Subphenotyping Calls for Revised Classification.

    Science.gov (United States)

    McBride, W A; McIntyre, G T; Carroll, K; Mossey, P A

    2016-09-01

    Nonsyndromic orofacial clefting (OFC) describes a range of phenotypes that represent the most common craniofacial birth defects in humans, with an overall birth prevalence of 1:700 live births. Because of the lifelong negative implications on health and well-being associated with OFC and the numbers of people affected, quality research into its etiology, diagnosis, treatment outcomes, and preventative strategies is essential. A range of different methods is used for recording and classifying OFC subphenotypes, one of which is the International Classification of Diseases (ICD) system. However, there is a general perception that research is being hampered by a lack of sensitivity and specificity in grouping those with OFC into subphenotypes, with potential heterogeneity and confounding in epidemiologic, genetic, and genotype-phenotype correlation studies. This article provides a background to the necessity of OFC research, discusses current controversies within cleft subphenotyping, and provides a brief overview of current OFC classifications as well as their limitations. The LAHSHAL classification is described in the context of a potentially useful tool for OFC that could complement the ICD-10/ICD-11 Beta coding systems to become a simply understood, universally accepted, clinically friendly, and research-sensitive instrument. Empowering registries, clinicians, and researchers to use a common classification system would have significant implications for OFC research across the world at a time when accurate subphenotyping is crucial and health care research is becoming increasingly tailored toward the individual. PMID:26171570

  11. Study on the short-term effect of maxillary expansion using self-ligating brackets in patients with cleft lip and palate%自锁托槽对唇腭裂腭扩展短期效果的研究

    Institute of Scientific and Technical Information of China (English)

    曾飞煌; 钱玉芬; 潘晓岗

    2014-01-01

    目的:探讨自锁托槽对单侧完全性唇腭裂患者上颌腭扩展的短期治疗效果。方法选取5例恒牙期单侧完全性唇腭裂(UCLP)患者,未行牙槽突裂骨移植手术,上颌牙齿粘贴自锁托槽(AO, Time2),放置高弹性镍钛弓丝,采集患者治疗前和腭扩展6~17个月后的牙颌模型、头颅定位后前位片进行测量,分析上颌腭扩展前后患者的牙弓、牙槽骨、腭穹窿以及颌骨的形态变化。结果牙颌模型测量结果提示:第一前磨牙区牙弓宽度明显增大,其次为尖牙区牙弓宽度,第一磨牙区牙弓宽度增加最少,第二磨牙区宽度减小。牙弓长度变化不明显。第一磨牙近中颊向扭转。腭部宽度增加,深度减小。头影测量结果提示:鼻腔宽度、上颌基骨宽度和上颌磨牙宽度稍有增加。结论唇腭裂自锁托槽腭扩展后上颌牙颌形态在横向宽度的变化较矢状向的变化明显。短期腭扩展治疗效果以牙齿移动和牙槽改建为主。%Objective To assess the short-term effects of maxillary palatal expansion using self-ligating brackets in patients with unilateral complete cleft lip and palate. Methods Five unilateral cleft lip and palate patients (UCLP) with severe maxillary constriction and without alveolar bone transplant were included in this study. Self-ligating brackets (AO, Time2) with superelastic nickel-titanium archwires were used during expan-sion. Tooth and jaws models and head positioning X-ray before treatment and after expansion for 6~17 months were gathered and measured to analyze the morphological changes of dental arch, alveolar bone, palatal vault, and jaw through digital cephalometric and model analysis methods. Result The model analysis demonstrated that a significant increase on arch width appeared especially at the area of first premolar, followed by the area of canine, and the area of first molar was the least, while the width of the area of second

  12. Comparison of bupivacaine alone and in combination with fentanyl or pethidine for bilateral infraorbital nerve block for postoperative analgesia in paediatric patients for cleft lip repair: A prospective randomized double blind study

    Directory of Open Access Journals (Sweden)

    Rajesh S Mane

    2011-01-01

    Conclusion: Thus we conclude that addition of fentanyl or pethidine to bupivacaine for Bilateral Intraoral Infraorbital Nerve Block prolong the duration of analgesia with no complications and can be used safely in paediatric patients.

  13. Theme and Information structure in French and English : A contrastive study of journalistic clefts

    OpenAIRE

    Carter-Thomas, Shirley

    2002-01-01

    In this article I will discuss factors motivating the use of the French c'est cleft structure, a marked-word order structure encountered with greater frequency in French discourse than the comparable it-cleft in English. Previous studies dealing with the English it-cleft, or predicated theme as it is usually termed in systemic linguistics, have generally sought to account for the structure in terms of contrastivness or specification, with the clefted element providing a certain thematic promi...

  14. Analysis on IRF6 gene polymorphism in patients with non-syndromic cleft lip and/or palate of Uyghur nationality and Han nationality in Xinjiang%新疆维、汉两民族非综合征性唇腭裂IRF6基因多态性分析

    Institute of Scientific and Technical Information of China (English)

    杨明; 谢金敏; 洪玉

    2013-01-01

    Objective: To explore the differences of genotypes and allele frequencies of single nucleotide polymorphism (SNP) of non - syndromic cleft lip and/or palate ( NSCL/P) interferon regulatory factor -6 (IRF -6) rs2013162 locus between the patients of Uyghur nationality and the patients of Han nationality.Methods: One hundred NSCL/P patients were selected as NSCL/P group, 50 patients of Uyghur nationality and 50 patients of Han nationality were included; while one hundred patients without NSCL/P were selected as control group, 50 patients of Uyghur nationality and 50 patients of Han nationality were included.PCR - RELF technique was used to analyze IRF6 gene polymorphism, a case - control study was conducted to analyze frequencies of genotypes and allele between the two groups and the differences between the patients of Uyghur nationality and the patients of Han nationality.Results: Among the patients of Uyghur nationality and the patients of Han nationality, there were statistically significant differences in frequencies of GG genotype, allele G, and allele T of rs2013162 locus between NSCL/P group and control group ( P 0.05) .Conclusion: There is a correlation between GG genotype and allele G of rs2013162 locus in patients with NSCL/P of Uyghur nationality and Han nationality.%目的:探讨非综合征性唇腭裂(NSCL/P)干扰素调节因子6(IRF6) rs2013162位点单核苷酸多态性(SNP)在新疆维、汉两民族内和民族间基因型和等位基因型的频率差异.方法:抽取100例NSCL/P患者作为NSCL/P组(维吾尔族50例、汉族50例),对照组100例(维吾尔族50例、汉族50例),运用聚合酶链式反应-限制性片段长度多态性(PCR-RELF)技术来分析IRF6基因的多态性,病例-对照研究分析两组基因型和等位基因型频率及两民族内和民族间频率的差异.结果:rs2013162位点GG基因型和等位基因G和T频率在NSCL/P组和对照组中分布差异有统计学意义(P<0.05),维、汉两民族内rs2013162位

  15. Clinical and research progress on the treatment effect and the long term stability of cleft lip and palate patients with maxillary hypoplasia during prepuberty%青春期早期唇腭裂继发上颌发育不足的治疗及其长期稳定性的临床研究进展

    Institute of Scientific and Technical Information of China (English)

    胡心怡

    2011-01-01

    Maxillary protraction and distraction osteogenesis are commonly used in cleft lip and palate patients with maxillary hypoplasia during prepuberty. The effect of the former one is dealing with the timing and the appliance selected while the latter one is associated with the types of distraction osteogenesis including face mask distraction, rigid external distraction and internal distraction. The long term stability of both treatment techniques is still under controversy. This paper reviews the effect and long term stability of the maxillary protraction and distraction osteogenesis.%上颌前方牵引技术和牵张成骨技术前移上颌骨,是目前常用的治疗青春期早期唇腭裂继发上颌发育不足患者的2种方法.上颌前方牵引技术的疗效主要与其牵引时机、牵引装置相关,而关于其治疗后的长期稳定性尚存在争议.牵张成骨技术的疗效主要与其不同的牵张方式有关,包括面罩式牵引、坚固外固定牵引和内置式牵引3种,而有关该治疗技术的长期稳定性也与其牵张方式相关.现本文就这2种常用治疗技术的疗效及其长期稳定性的临床研究进展作一综述.

  16. Speech and language development in toddlers with and without cleft palate

    NARCIS (Netherlands)

    Priester, G. H.; Goorhuis-Brouwer, S. M.

    2008-01-01

    Objective: The effect of early palate closure on speech and language development in children with cleft palate. Design: Comparative study. Setting: University Medical Center Groningen, Cleft Palate Team (The Netherlands). Materials and methods: Forty-three toddlers with cleft palate and thirty-two t

  17. Exploring Subclinical Phenotypic Features in Twin Pairs Discordant for Cleft Lip and Palate

    DEFF Research Database (Denmark)

    Leslie, Elizabeth J; Carlson, Jenna C; Cooper, Margaret E;

    2016-01-01

    OBJECTIVE: Monozygotic twins of an individual with an orofacial cleft have a significantly elevated risk for orofacial cleft compared with the general population, but still the concordance rate for orofacial cleft in monozygotic twins is about 40% to 50%. The goal of this study was to determine w...

  18. Osteopontin, osteocalcin, and osteoprotegerin expression in human tissue affected by cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Smane L.

    2016-01-01

    Full Text Available Cleft lip and palate (CLP is a common congenital anomaly with a complex etiology which has not been elucidated yet. This study investigated whether expression of osteopontin (OPN, osteoprotegerin (OPG, and osteocalcin (OC, which are essential for the normal craniofacial bone remodelling, is not regulated in children with CLP. Alveolar bone tissue samples were obtained from patients with complete bilateral (CB CLP (n = 14 during corrective plastic surgery and unaffected control subjects (n = 9. OPN, OPG, and OC expression was assessed by immunohistochemistry, and data were analyzed with the Mann-Whitney test. OPN expression was observed only sporadically in the alveolar bone of 3 patients, in contrast to the control group (z = −2.962; P < 0.003. The number of OPG-positive bone cells varied from occasional to moderate, in contrast to the control group (z = −2.247; P = 0.025. OC-positive osteocytes were present in moderate to numerous numbers in both patients and controls, with no significant difference between them (z = −1.356; P < 0.175. The prominent expression of OC characteristic for CBCLP affected hard tissue indicates a high potential of bone mineralization. Few OPG-positive osteocytes in the bone tissue implicate the disregulation of osteoclast differentiation, maturation, and activity, but few OPN-containing cells may prove the common disregulation of bone remodelling during cleft morphopathogenesis.

  19. Facial esthetics in children with unilateral cleft lip and palate 3 years after alveolar bonegrafting combined with rhinoplasty between 2 and 4 years of age

    NARCIS (Netherlands)

    Offert, B.; Janiszewska-Olszowska, J.; Dudkiewicz, Z.; Brudnicki, A.; Katsaros, C.; Fudalej, P.S.

    2013-01-01

    OBJECTIVES: To evaluate facial esthetics in patients with unilateral cleft lip and palate (UCLP) after alveolar bone grafting combined with rhinoplasty between 2 and 4 years of age. DESIGN: Retrospective case-control study. SETTING: The Department of Pediatric Surgery, Institute of Mother and Child,

  20. Facial esthetics in children with unilateral cleft lip and palate 3 years after alveolar bonegrafting combined with rhinoplasty between 2 and 4 years of age.

    NARCIS (Netherlands)

    Offert, B.; Janiszewska-Olszowska, J.; Dudkiewicz, Z.; Brudnicki, A.; Katsaros, C.; Fudalej, P.S.

    2013-01-01

    OBJECTIVES: To evaluate facial esthetics in patients with unilateral cleft lip and palate (UCLP) after alveolar bone grafting combined with rhinoplasty between 2 and 4 years of age. DESIGN: Retrospective case-control study. SETTING: The Department of Pediatric Surgery, Institute of Mother and Child,

  1. Pedunculated cavernous hemangioma originating in the olfactory cleft.

    Science.gov (United States)

    Su, Kaiming; Zhang, Weitian; Shi, Haibo; Yin, Shankai

    2014-09-01

    Sinonasal cavernous hemangioma is a rare condition that usually affects the lateral wall of the nasal cavity. We report the case of a 77-year-old man who presented with severe epistaxis, nasal congestion, and olfactory dysfunction. Endoscopic examination of the nasal cavity revealed the presence of a red-blue tumor that had almost completely filled the nasopharynx. Preoperatively, it was difficult to distinguish this lesion from a juvenile nasopharyngeal angiofibroma. During endoscopic surgery, the tumor was found to originate in the left olfactory cleft, and it had a long peduncle that contained blood vessels. Postoperative histopathologic examination indicated that the mass was a cavernous hemangioma. To the best of our knowledge, this is the first case of an olfactory cleft cavernous hemangioma and the first case of olfactory cleft disease associated with a cavernous hemangioma to be reported in the English-language literature. PMID:25255356

  2. Dislocated Tongue Muscle Attachment and Cleft Palate Formation.

    Science.gov (United States)

    Kouskoura, T; El Fersioui, Y; Angelini, M; Graf, D; Katsaros, C; Chiquet, M

    2016-04-01

    In Pierre Robin sequence, a retracted tongue due to micrognathia is thought to physically obstruct palatal shelf elevation and thereby cause cleft palate. However, micrognathia is not always associated with palatal clefting. Here, by using the Bmp7-null mouse model presenting with cleft palate and severe micrognathia, we provide the first causative mechanism linking the two. In wild-type embryos, the genioglossus muscle, which mediates tongue protrusion, originates from the rostral process of Meckel's cartilage and later from the mandibular symphysis, with 2 tendons positive for Scleraxis messenger RNA. In E13.5 Bmp7-null embryos, a rostral process failed to form, and a mandibular symphysis was absent at E17.5. Consequently, the genioglossus muscle fibers were diverted toward the lingual surface of Meckel's cartilage and mandibles, where they attached in an aponeurosis that ectopically expressed Scleraxis. The deflection of genioglossus fibers from the anterior-posterior toward the medial-lateral axis alters their direction of contraction and necessarily compromises tongue protrusion. Since this muscle abnormality precedes palatal shelf elevation, it is likely to contribute to clefting. In contrast, embryos with a cranial mesenchyme-specific deletion of Bmp7 (Bmp7:Wnt1-Cre) exhibited some degree of micrognathia but no cleft palate. In these embryos, a rostral process was present, indicating that mesenchyme-derived Bmp7 is dispensable for its formation. Moreover, the genioglossus appeared normal in Bmp7:Wnt1-Cre embryos, further supporting a role of aberrant tongue muscle attachment in palatal clefting. We thus propose that in Pierre Robin sequence, palatal shelf elevation is not impaired simply by physical obstruction by the tongue but by a specific developmental defect that leads to functional changes in tongue movements.

  3. Epidemiology, Etiology, and Treatment of Isolated Cleft Palate

    OpenAIRE

    Burg, Madeleine L.; Chai, Yang; Yao, Caroline A.; Magee, William; Figueiredo, Jane C.

    2016-01-01

    Isolated cleft palate (CPO) is the rarest form of oral clefting. The incidence of CPO varies substantially by geography from 1.3 to 25.3 per 10,000 live births, with the highest rates in British Columbia, Canada and the lowest rates in Nigeria, Africa. Stratified by ethnicity/race, the highest rates of CPO are observed in non-Hispanic Whites and the lowest in Africans; nevertheless, rates of CPO are consistently higher in females compared to males. Approximately fifty percent of cases born wi...

  4. Epidemiology, Etiology and Treatment of Isolated Cleft Palate

    OpenAIRE

    Burg, Madeleine L.; Yang eChai; Caroline eYao; William eMagee; Figueiredo, Jane C.

    2016-01-01

    Isolated cleft palate (CPO) is the rarest form of oral clefting. The incidence of CPO varies substantially by geography from 1.3 to 25.3 per 10,000 live births, with the highest rates in British Columbia, Canada and the lowest rates in Nigeria, Africa. Stratified by ethnicity/race, the highest rates of CPO are observed in non-Hispanic Whites and the lowest in Africans; nevertheless, rates of CPO are consistently higher in females compared to males. Approximately fifty percent of children born...

  5. Simplified feeding appliance for an infant with cleft palate

    Directory of Open Access Journals (Sweden)

    Shaila Masih

    2014-01-01

    Full Text Available A child born with cleft palate may experience difficulties while feeding. Early surgical treatment may need to be postponed until certain age and weight gain of the infant. The case presented here is of a 1-month-old neonate born with cleft palate, assisted with a new feeding appliance made with ethylene vinyl acetate using pressure molding technique to aid in proper feeding. The patient′s weight and health significantly improved after the insertion of obturator. The advantages of this material included being lightweight, moldability, good palatal fit and decreased soft tissue injury.

  6. EEC syndrome sans clefting: Variable clinical presentations in a family

    Directory of Open Access Journals (Sweden)

    Thakkar Sejal

    2007-01-01

    Full Text Available Ectrodactyly, ectodermal dysplasia and cleft palate/lip syndrome (EEC is a rare autosomal dominant syndrome with varied presentation and is actually a multiple congenital anomaly syndrome leading to intra- and interfamilial differences in severity because of its variable expression and reduced penetrance. The cardinal features include ectrodactyly, sparse, wiry, hypopigmented hair, peg-shaped teeth with defective enamel and cleft palate/lip. A family comprising father, daughter and son presented to us with split hand-split foot deformity (ectrodactyly, epiphora, hair changes and deafness with variable involvement in each family member.

  7. Periconceptional health and lifestyle factors of both parents affect the risk of live-born children with orofacial clefts

    NARCIS (Netherlands)

    Krapels, Ingrid P. C.; Zielhuis, Gerhard A.; Vroom, Fokaline; de Jong-van den Berg, Lolkje T. W.; Kuijpers-Jagtman, Anne-Marie; van der Molen, Aebele B. Mink; Steegers-Theunissen, Regine P. M.

    2006-01-01

    BACKGROUND: Nonsyndromic cleft lip with or without cleft palate (CL/P) or cleft palate only (CPO) are orofacial clefts and have a multifactorial etiology. The identification of amendable parental risk factors may contribute to a reduced occurrence of these malformations in the future. METHODS: Stand

  8. GFA Taq I polymorphism and cleft lip with or without cleft palate (CL/P) risk

    Science.gov (United States)

    Dong, Lijia; Ma, Lian

    2015-01-01

    The transforming growth factor alpha (TGFA) Taq I polymorphism has been indicated to be correlated with cleft lip with or without cleft palate (CL/P) susceptibility, but study results are still debatable. Thus, a meta-analysis was conducted. We conducted a comprehensive search of Embase, Ovid, Web of Science, the Cochrane database, PubMed, the Chinese Biomedical Literature Database (CBM-disc, 1979-2014), the database of National Knowledge Infrastructure (CNKI, 1979-2014) and the full paper database of Chinese Science and Technology of Chongqing (VIP, 1989-2014) to identify suitable studies. There were 18 studies suitable for this meta-analysis, involving a total of 3135 cases and 3575 controls. Significantly increased CL/P risk was observed (OR = 1.49; 95% CI 1.17-1.89; P = 0.001). In subgroup analyses stratified by ethnicity, there was evidence in the Caucasian population for an association between this polymorphism and CL/P risk (OR = 1.52; 95% CI 1.14-2.02; P = 0.004). However, no significant association was found between this his polymorphism and CL/P risk in African and Hispanic populations. According to a specific CL/P type, increased clip lip and palate risk and clip palate risk were found (OR = 1.38; 95% CI 1.10-1.73; P = 0.005; OR = 1.29; 95% CI 1.01-1.66; P = 0.042). In conclusion, the present meta-analysis found that the TGFA Taq I polymorphism may be associated with CL/P susceptibility. PMID:26064247

  9. Auditory complaints and audiologic assessment in children with surgically repaired cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Cerom, Jaqueline Lourenço

    2013-04-01

    Full Text Available Introduction: At the initial consultation, the speech-language pathologist and audiologist may consider possible diagnostic hypotheses based on the child's history and the parents' complaint. Aim: To investigate the association of hearing complaints with the findings obtained in the conventional audiologic assessment in children with cleft lip and palate. Retrospective study. Methods: We analyzed medical charts of 1000 patients with cleft lip and palate who underwent surgical repair between 1988 and 1995 at a mean age of 6 years 8 months. We excluded charts with records of inconsistent audiological responses and charts with missing data for any of the audiologic evaluations considered. Thus, the sample consisted of 393 records. Results: Two hundred thirty-nine patients presented hearing loss in one or both ears, but only 3.8% reported hearing complaints. The most frequent were otorrhea followed by otalgia. There was no statistical significance between the complaint and gender (p = 0.26 nor between the complaint and hearing loss (p = 0.83. Conclusion: This study showed no association between the hearing complaint and the conventional audiologic assessment.

  10. Ectrodactyly ectodermal dysplasia clefting (EEC) syndrome: a rare cause of congenital lacrimal anomalies.

    Science.gov (United States)

    Elmann, Solly; Hanson, Sarah A; Bunce, Christopher N; Shinder, Roman

    2015-01-01

    A 9-year-old girl with a medical history significant for ectrodactyly ectodermal dysplasia clefting (EEC) syndrome was referred for evaluation of congenital left-sided epiphora. The patient had undergone successful right external dacryocystorhinostomy at age 5 to treat congenital right-sided epiphora. On examination, several ocular anomalies were noted, including absence of the upper eyelid puncta, absence of the left inferior punctum, a left lacrimal fistula opening at the left caruncle, increased left tear lake, bilateral hypoplastic meibomian glands, mild conjunctival injection, and thin eyelid cilia and brow hair. Systemic findings included cleft lip and palate status-post repair, ectrodactyly of the hands and feet, adontia and microdontia, a pointed nose, and lightly pigmented, dry hair and skin. The patient underwent examination under anesthesia and left conjunctivodacryocystorhinostomy with insertion of a Jones tube with resolution of lacrimation postoperatively. To the authors' knowledge, this is the second report detailing management of congenital lacrimal anomalies in EEC syndrome, and the first describing management of punctal atresia with conjunctivodacryocystorhinostomy and Jones tube placement. PMID:24801258

  11. Diagnóstico Pré-Natal de Fenda Labial e Palatina: Experiência de 40 Casos Prenatal Diagnosis of Lip and Palate Cleft: Experience of 40 Cases

    Directory of Open Access Journals (Sweden)

    Victor Bunduki

    2001-10-01

    Full Text Available Objetivos: avaliar casos de fenda facial fetal quanto ao tipo de lesão, associação com outras malformações e aneuploidias. Métodos: as fendas faciais foram estudadas quanto a idade materna e antecedentes, idade gestacional no diagnóstico, lado da lesão, tipo de lesão, presença de malformações associadas e/ou aneuploidias, mortalidade e seguimento pós-natal. Resultados: em 40 fetos com fenda, a lesão era labial pura em 18 casos (45%, labiopalatina em 19 (47,5% e palatina em 3 (7,5%. Em 10 casos a fenda era isolada (25%, todas unilaterais. No grupo de malformações associadas, a aneuploidia esteve presente em 10/30 (33,3%. A lesão labiopalatina predominou neste grupo (18/30 - 60%, seguida por fenda bilateral (8/30 - 26,7% e mediana (10/30 - 33,3%. Conclusões: as fendas faciais constituíram excelente marcador para malformações associadas e aneuploidias fetais. Casos de fendas faciais devem ser encaminhados para centros especializados para que se realizem adequados exames ultra-sonográfico e genético tecendo em seguida a conduta. O caráter isolado da fenda facial esteve associado a excelente prognóstico.Purpose: to evaluate fetuses with facial cleft as to type of lesion, associated malformations and aneuploidies. Method: the following parameters were evaluated: maternal age and previous history, gestational age at diagnosis, lesion side, type of lesion, presence of associated malformations and aneuploidies, mortality rate and postnatal follow-up. Results: forty fetuses had facial cleft, 18 (45% cases had cleft lip, 19 (47.5% had cleft lip and palate, and 3 (7.5% cases presented with cleft palate. Isolated facial cleft was observed in 10 fetuses (25%, all of them unilaterally located. Aneuploidies were identified in 10/30 (33.33% of the patients with associated malformations. Cleft lip and palate was more often seen in this group (18/30 - 60%, followed by bilateral lesion (8/30 - 26.7% and median cleft (10/30 - 33

  12. Maxillary growth in a congenital cleft palate canine model for surgical research.

    Science.gov (United States)

    Paradas-Lara, Irene; Casado-Gómez, Inmaculada; Martín, Conchita; Martínez-Sanz, Elena; López-Gordillo, Yamila; González, Pablo; Rodríguez-Bobada, Cruz; Chamorro, Manuel; Arias, Pablo; Maldonado, Estela; Ortega, Ricardo; Berenguer, Beatriz; Martínez-Álvarez, Concepción

    2014-01-01

    We have recently presented the Old Spanish Pointer dog, with a 15-20% spontaneous congenital cleft palate rate, as a unique experimental model of this disease. This study aimed to describe the cleft palate of these dogs for surgical research purposes and to determine whether congenital cleft palate influences maxillofacial growth. Seven newborn Old Spanish Pointer dogs of both sexes, comprising a cleft palate group (n = 4) and a normal palate group (n = 3), were fed using the same technique. Macroscopic photographs and plaster casts from the palate, lateral radiographs and computer tomograms of the skull were taken sequentially over 41 weeks, starting at week 5. The cleft morphology, the size and the tissue characteristics in these dogs resembled the human cleft better than current available animal models. During growth, the cleft width varies. Most of the transverse and longitudinal measures of the palate were statistically lower in the cleft palate group. The cleft palate group showed hypoplasia of the naso-maxillary complex. This model of congenital cleft palate seems suitable for surgical research purposes. A reduced maxillofacial pre- and post-natal development is associated to the congenital cleft palate in the Old Spanish Pointer dog.

  13. Comparing caries risk profiles between 5- and 10- year-old children with cleft lip and/or palate and non-cleft controls

    OpenAIRE

    Lena Sundell, Anna; Ullbro, Christer; Marcusson, Agneta; Twetman, Svante

    2015-01-01

    Background: Previous studies have suggested that children with oral clefts may have higher caries prevalence in comparison with non-cleft controls but the relative importance of the potential risk factors is not clear. The aim of this study was to compare the caries risk profiles in a group of cleft lip and/or palate (CL(P)) children with non-cleft controls in the same age using a computerized caries risk assessment model. Methods: The study group consisted of 133 children with CL(P) (77 subj...

  14. A case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft

    Directory of Open Access Journals (Sweden)

    Miyake T

    2016-07-01

    biomicroscopy revealed that almost all directions were open except for the peripheral anterior synechia. Since magnetic resonance imaging revealed ventriculomegaly associated with an interhemispheric cyst at birth, a ventriculoperitoneal shunt was inserted at 12 days of age. At 25 months of age, her condition suddenly deteriorated due to occlusion of the ventricular shunt catheter, and she died 5 days later. In this patient, amniotic band syndrome was presumed to be the primary cause due to the clinical findings. Conclusion: We experienced a case of optic-nerve hypoplasia and anterior segment abnormality that occurred with facial cleft. The cause of these abnormalities is unclear, yet amniotic band syndrome is a possible candidate. Keywords: facial cleft, optic-nerve hypoplasia, amniotic band syndrome

  15. Genetic studies in congenital anterior midline cervical cleft

    DEFF Research Database (Denmark)

    Jakobsen, L P; Pfeiffer, P; Andersen, M;

    2012-01-01

    Congenital anterior midline cervical cleft (CAMCC) is a rare anomaly, with less than 100 cases reported. The cause of CAMCC is unknown, but genetic factors must be considered as part of the etiology. Three cases of CAMCC are presented. This is the first genetic study of isolated CAMCC. Conventional...

  16. First trimester exposure to corticosteroids and oral clefts

    NARCIS (Netherlands)

    Pradat, P; Robert-Gnansia, E; Di Tanna, GL; Rosano, A; Lisi, A; Mastroiacovo, P

    2003-01-01

    BACKGROUND: The possible association between oral cleft in the newborn and maternal exposure to corticoids during pregnancy is still controversial. The aim of this study was to test this association by a case-control analysis using the large multicentric MADRE database. METHODS: The MADRE database i

  17. On the cleft construction : is it simplex or complex?

    OpenAIRE

    長谷川, 信子; ハセガワ, ノブコ; Nobuko, Hasegawa

    2011-01-01

    The cleft construction, as its name indicates, involves two parts, a focused part and a presupposition, each of which involves a separate predicate. In the framework of GB and previous to it, each predicate necessarily constitutes a sentence; hence, a Cle

  18. PARENTAL PESTICIDAL EXPOSURE AND RI SK OF CLEFTS IN THE CRANIOFACIAL REGION: A CASE-CONTROL STUDY IN SOUTH INDIA

    OpenAIRE

    Jose Anna Betty; Mokhasi Varsha; Subramani S A; Shashirekha M; Jayanthi K.S; T.Rajini

    2015-01-01

    Obje ctive: The multifactorial etiology of cleft can be due to envi ronmental factors or genetic factors or combination of both. Many studies were conducted to detect the epidemiology of the clefts and the genetic factors causing clefts. There is no or very less studies conducted in India to identify the risk of pesticidal exposure in occurrence of nonsyndromic clefts. The present study is to investigate the risk of parental pesticidal exposure in causing clefts in the craniofacial region. Me...

  19. Differential diagnosis of pituitary adenomas and Rathke's cleft cysts by diffusion-weighted MRI using single-shot fast spin echo technique

    International Nuclear Information System (INIS)

    The purpose of the present study was to prospectively evaluate the diagnostic ability of diffusion-weighted magnetic resonance imaging (DWI) using single-shot fast spin echo (SSFSE) technique to discriminate pituitary adenomas from Rathke's cleft cysts. DWIs were obtained from 40 patients with pathologically proven pituitary macroadenomas and 15 patients with proven Rathke's cleft cysts. Pituitary adenomas were divided into 27 cases with solid components alone, five with non-hemorrhagic large cysts, and eight with intratumoral hemorrhage. On SSFSE DWI, solid components of pituitary adenomas revealed iso or slightly increased intensity and intratumoral hemorrhage showed higher intensity than normal brain parenchyma, whereas Rathke's cleft cysts and intratumoral cysts demonstrated very low intensity. SSFSE DWI did not display the susceptibility artifacts that are seen close to the skull base and sinonasal cavities on echo planar diffusion imaging. On the basis of our preliminary findings, DWI may enable us to differentiate pituitary adenomas with only solid components and hemorrhagic pituitary adenomas appearing hyperintense on T1-weighted images from Rathke's cleft cysts without administration of gadolinium-DTPA. SSFSE DWI appears to be a useful technique for characterizing pituitary diseases without the susceptibility artifacts. Our study is the first report to demonstrate the identification of pituitary disorders on SSFSE DWI. (author)

  20. Bite force evaluation in subjects with cleft lip and palate

    Directory of Open Access Journals (Sweden)

    Carla Renata Sipert

    2009-04-01

    Full Text Available The purpose of this study was to evaluate the masticatory function of subjects with cleft lip and palate by analyzing the bite force developed by these individuals. Bite force was evaluated in a group of 27 individuals with repaired unilateral cleft lip and palate (14 males and 13 females - aged 18-26 years and compared to the data achieved from a group of 20 noncleft subjects (10 males and 10 females - aged 18-26 years. Measurement was achieved on three positions within the dental arch (incisors, right molars and left molars, three times at each position considering the highest value for each one. Statistical analysis was performed by ANOVA and Mann-Whitney test ( α = 5%. There was a significant deficit in bite force in male individuals with cleft lip and palate compared to the male control group (p=0.02, p=0.004, p=0.003 for incisors, right and left molars, respectively. For the female group, the difference was not statistically significant (p=0.79, p=0.06, p=0.47. In the group of individuals with clefts, 92.6% were under orthodontic treatment, which could be a reason for the present findings, since it can decrease the bite force more remarkably in males than in females. In conclusion, the bite force is significantly reduced in men when comparing the cleft group to the noncleft group. In females, this reduction was not significant in the same way. However, the main reason for this reduction and for the different behavior between genders should be further investigated.