A familial tetraphocomelia syndrome involving limb deformities, cleft lip, cleft palate, and associated anomalies ?A new syndrome

1976 ◽  
Vol 33 (3) ◽  
pp. 323-326 ◽  
Author(s):  
Kiran Kucheria ◽  
S. K. Bhargava ◽  
R. Bamezai ◽  
P. Bhutani
2008 ◽  
Vol 45 (6) ◽  
pp. 592-596 ◽  
Author(s):  
Aziza Aljohar ◽  
Kandasamy Ravichandran ◽  
Shazia Subhani

Objective: To report the patterns of cleft lip and/or cleft palate in Saudi Arabia from data collected at a tertiary care hospital. Design and Setting: King Faisal Specialist Hospital and Research Center, Riyadh. Patients: All the cleft lip and/or cleft palate patients registered in the Cleft Lip/Palate and Craniofacial Anomalies Registry from June 1999 to December 2005. Results: Retrospectively, 807 cases of cleft lip and/or palate were registered. There were 451 boys and 356 girls. Cleft lip and palate was more common (387) than isolated cleft palate (294) and isolated cleft lip (122). Boys predominated in cleft lip and palate and cleft lip; whereas, girls predominated in isolated cleft palate, with boy to girl ratios of 1.6:1, 1.2:1, and 0.9:1 for cleft lip and/or palate, isolated cleft lip, and isolated cleft palate, respectively. The Riyadh region had more cases (32.0%) than the Asir (15.6%) and Eastern (14.6%) regions. Parents of 439 individuals had consanguineous marriages. A positive family history of cleft was seen in 224 cases. Of 238 cases with associated anomalies, 91 had congenital heart disease. Of the children with isolated cleft palate, 40.5% had associated anomalies, whereas only 23.0% of the children with isolated cleft lip or cleft lip and palate had associated malformations. Conclusion: The pattern of cleft observed in this study does not differ significantly from those reported in the literature for Arab populations.


2014 ◽  
Vol 03 (04) ◽  
pp. 203-208
Author(s):  
Alpana Barman ◽  
B C Dutta ◽  
J K Sarkar

Abstract Background : Cleft lip and palate are some of the most common congenital deformities. They frequently occur as isolated deformities, but can be associated with other medical conditions and anomalies. Aim of the study: To study the distribution of different types of cleft lip and palate and associated anomalies. Materials and methods: Forty patients of cleft lip and palate were studied. Relevant history was taken, clinical examination done and recorded with photography with consent. The cases were classified as per Nagpur Classification. Results: In our study 24 cases (60%) were cleft lip and palate (type III) and 10 cases (25%) of cleft palate alone (type II) and 6 cases (15%) of cleft lip alone (type I). Males were found to predominate in type I and III. Females predominated in type II. Among the cleft lip, left side is found to be involved in most of the cases. In our study, out of 40 cases, 8 cases (20%) had other associated anomalies. Most of these were associated with cleft lip and palate (type III) and isolated cleft palate (type II). Conclusion: The typical universal distribution of cleft types and associated anomalies tallies with our results.


Author(s):  
Yuriy Anatol’evich Vasil’ev ◽  
A. N Red’ko ◽  
O. V Gulenko ◽  
I. G Udina

The goal of our study was investigation of prenatal diagnostics of pathology and distribution of associated anomalies in the group of children - patients of clinic with congenital maxillofacial anomalies. Basing on oral poll of mothers given birth to children with the pathology during the period 2012-2014 in Krasnodarskii krai (358 cases), the efficiency of prenatal detection of congenital clefts of lip and palate (diagnosis Q36, Q35 and Q37 by MKB-10) by ultrasonic investigation was studied. Prenatal 2D ultrasonic investigation is performed three times during pregnancy on 12, 18-24 and 32-34 weeks of pregnancy. Cleft lip or palate were detected on the second and third ultrasonic investigation: cleft palate (Q35) was revealed only in 5,6% cases of children born with cleft palate, and cleft palate (Q36 and Q37) - in 33,8% cases. Importance of prenatal ultrasonic diagnostics of pathology is not only predetermined by the necessity of mental conditioning of parents for the most urgent and proper rehabilitation of ill children, but, also, by the fact that patients with clefts of lip or palate frequently have associated pathology of other organs and chromosomal anomaly. For proper prenatal detection of cleft palate 3D ultrasonic investigation is necessary. We also studied the presence of associated pathology distribution for the cases of isolated clefts of lip or palate. Higher efficiency of cleft lip and palate detection in prenatal period by ultrasonic diagnostics would support more successful rehabilitation by well-timed mental conditioning and informing of parents about stages of rehabilitation in the process of child growing. Ethical aspects of probable abortion because of detected pathology during ultrasonic investigation.


1993 ◽  
Vol 30 (5) ◽  
pp. 447-451 ◽  
Author(s):  
Yvonne Hofstee ◽  
Nico Kors ◽  
Raoul C.M. Hennekam

A cleft lip, cleft palate, or both are associated with a high frequency of other anomalies. This study gives an inventory of associated anomalies in a consecutive group of children (n = 36) with clefts, referred to a local multidisciplinary cleft team in the Netherlands. In 47.2% of cleft patients associated anomalies were found, allowing diagnosis of provisionally unique syndromes or known entities. In 17 patients family history was positive for clefting; in five patients (13.9%) this influenced the occurrence risks for siblings. Both findings had an effect on genetic counseling of the parents of these children. Additional evidence is provided that all children with clefting should be carefully evaluated by a trained clinician for additional anomalies, including dysmorphic features.


2019 ◽  
Vol 9 (1) ◽  
pp. 6-10
Author(s):  
Sanzida Khatun

Introduction: Cleft lip and/or palate is a congenitally persistent space affecting orofacial region. It is generally divided into cleft lip (CL), cleft palate (CP) and cleft lip with cleft palate (CLP). The objective of the study was to evaluate distribution of patterns of orofacial clefts according to sex and laterality; predisposing factors and other congenital anomalies associated with cleft lip and/or palate. Materials & Method: A cross-sectional descriptive type of study was carried out using in 268 patients having cleft lip and/or palate. Types of clefts, predominance of sex and laterality, familial history, associated anomalies and drug and disease history of mother were recorded. Result: The results revealed 23.51% CL, 54.85% CLP and 21.64% CP. There were 58.21% males and 41.79% females. Positive family history of cleft was present in 28.0% and associated anomalies were observed in 3.7% of the cleft subjects. Mothers of 6.0% of the total patients were exposed to teratogenic agents during first trimester of pregnancy. Conclusion: It was concluded that among the three types of cleft (CL, CP and CLP), CLP is the most common type. The males are affected more with cleft lip and/or palate than the females. Clefts also have risks of associated congenital anomalies. Exposure to teratogenic agents in first trimester of pregnancy is also associated with orofacial cleft.


2021 ◽  
pp. 105566562198912
Author(s):  
Morgan Wishney ◽  
Aziz Sahu-Khan ◽  
Peter Petocz ◽  
M. Ali Darendeliler ◽  
Alexandra K. Papadopoulou

Objectives: To (1) survey Australian orthodontists about their involvement with a government-funded scheme for patients with clefts, the Medicare Cleft Lip and Cleft Palate Scheme (MCLCPS) and (2) investigate their attitude toward treating patients with clefts and their training in this respect. Design: A 13-question online survey was distributed to members of the Australian Society of Orthodontists. The survey gathered information regarding respondent demographics, the number of MCLCPS-eligible patients seen in the past 12 months and usual billing practices. Results: A total of 96 complete responses were obtained. About 70% of respondents had treated MCLCPS-eligible patients in the past 12 months and 55% saw between 2 and 5 patients during this time. The likelihood of treating patients with clefts increased by a factor of 4.8 (95% CI: 1.2-18.9) if practicing outside of a capital city and 1.5 times for each decade increase in orthodontist’s age (95% CI: 1.0-2.2). The MCLCPS was utilized by 81% of orthodontists with 26% of these respondents accepting rebate only. Most orthodontists felt their university training could have better prepared them to treat patients with clefts. A minority of orthodontists felt that a rebate increase would make them more likely to treat these patients. Conclusions: Australian orthodontists who treat patients with clefts tend to be older and work outside of capital cities. The decision to treat these patients tends to not be financially motived. Specialty orthodontic training programs could improve the preparedness of their graduates to treat patients with clefts.


2021 ◽  
pp. 105566562110132
Author(s):  
Justin Parry Weinfeld ◽  
Jakob Åsberg Johnels ◽  
Christina Persson

Objective: To investigate the prevalence of reading difficulties in children born with cleft palate at ages 9 and 10 in Sweden. Design: Using a cross-sectional design, a parental questionnaire assessing dyslexia-like reading difficulties (Short Dyslexia Scale, SDS) was administered together with separate questions regarding background data. Participants: Families with a child born with overt cleft palate with or without cleft lip in 4 regions of Sweden. A total of 245 families were approached of which 138 families responded. Data from 136 (56%) were complete with information on cleft type and could be analyzed. Results: Twenty-two percent (95% CI, 15-30) of the whole study group displayed risk for dyslexic reading difficulties on the SDS corresponding to the 7th to 10th percentiles in the population. Children with cleft palate only had a significantly higher prevalence of reading difficulties (37%) compared to children with unilateral cleft palate (19%) and bilateral cleft palate (10%). The frequency of reading difficulties in participants with comorbidity was 32%. Among a subgroup with reported comorbidity in areas of attention, language, and learning problems, there was a 2.5 times higher risk of reading disability compared to participants without this reported comorbidity. Conclusion: The prevalence of reading difficulties in the cleft palate population was higher than in the general population. Results showed that co-occurring difficulties were common in the cleft group and that reading difficulties often appear together with other, co-occurring neurodevelopmental difficulties.


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