scholarly journals How is the face formed and when it goes wrong: In relation to Cleft Lip/Palate

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Fozia Khan

The normal development of the face relies upon the correct morphogenesis of structures in utero that usually occurs within the first trimester of embryonic life. The face is a very complex structure involving many genes and factors and with it being such a crucial part of life, both physically and aesthetically and therefore mentally, its important for everything to be just right. However, when the normal process doesn’t go to plan this results in dysmorphogenesis, which cleft lip and palate (CLP) is an example of as the lip/palate doesn’t fuse together and the infant is left with a gap. Although the exact cause of CLP is unknown, it is thought to be a mixture of genetics, environment and the teratogens the mothers are exposed to within the environment. This report will demonstrate the normal development of the face for the purpose of understanding how it goes wrong, resulting in CLP. Since there is still a lot to be understood about CLP it will also shed light on recent advances in relating SHH and certain genes as a possible cause for this dysmorphogenesis. The report will also briefly look at the relation of CLP with the genes associated with syndromic and non-syndromic diseases and the different types of CLP. There are many other facial defects that are a result of dysmorphogenesis, however as CLP is one of the most common yet poorly understood facial defect, it will be the main focus of this report.

2012 ◽  
Vol 1 (1-2) ◽  
Author(s):  
Ashwin R. Jadhav ◽  
Ana Monteagudo ◽  
Rosalba Santos ◽  
Ilan Timor

AbstractOrofacial clefts are the most common group of craniofacial anomalies. The detection rate of cleft lip during the mid-trimester, using two-dimensional (2D) ultrasound screening, has improved over the last three decades. However, the detection rates of defects involving the hard palate or isolated cleft palate have remained poor. Over the last decade, many investigators have studied the use of 3D ultrasound to improve the detection rates of these defects. With the increasing use and acceptance of first-trimester aneuploidy screening in the US, there is growing interest in performing first-trimester fetal anatomy surveys. Reports of first-trimester diagnosis of cleft lip and palate are rare, and this aspect of prenatal sonographic diagnosis remains underexplored. We report a case of unilateral cleft lip and cleft palate diagnosed during a routine first-trimester screening at 12 weeks’ gestation and review the pertinent literature on first-trimester diagnosis of cleft lip/palate.


Author(s):  
O. V. Dudnik ◽  
Ad. A. Mamedov ◽  
O. I. Admakin ◽  
A. A. Skakodub ◽  
Y. O. Volkov ◽  
...  

Relevance. Cleft lip and palate is one of the severe malformations of the face and jaw, requiring a comprehensive approach to the rehabilitation of the patients, including doctors of various specialties, one of which is orthodontists. A feature of orthodontic treatment is difficulty of fixing bracket systems, as well as lowering the level of oral hygiene, caused by deformation and displacement of fragments of the maxillofacial region.Purpose. Improving the effectiveness of orthodontic treatment and hygiene of the oral caviti in patients with cleft lip and palate in permanent bite period.Materials and methods. A comparison was made of the effetctiveness of fixing brackets systmes with direct and indirect bonding techniques and the effectiveness of oral hygiene during orthodontic treatment using irrigators.Results. The results of the study showed a difference in the effectiveness of using direct and indirect methods of fixing bracket systems in patients with cleft and palate. The use of irrigators as additional means of oral hygiene has demonstrated a positive dynamic of hygiene indices.Conclusions. Results of the study demonstrate the advantages of fixation the brackets by indirect bonding and use additional hygiene products irrigator for improving of oral hygiene.


2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Gamal A. Abdelhameed ◽  
Wael A. Ghanem ◽  
Simon H. Armanios ◽  
Tamer Nabil Abdelrahman

Abstract Background Cleft lip and palate is one of the commonest congenital anomalies, which have an impact on feeding, speech, and dental development away from the significant psychosocial sequel. Early surgical repair aims to restore appearance and function, and the modern techniques can leave many defects undetectable. Therefore, the anesthetic challenge facing the pediatric airway with such abnormalities is still of a great impact. The aim of our study among 189 patients enrolled is to correlate alveolar gap and maximum cleft width measurements as predictors of difficult laryngoscopy and intubation in infants with unilateral complete cleft lip/palate aging from 1 to 6 months. As a secondary outcome, their weight is to be correlated too as another parameter. Results The alveolar gap and maximum cleft width are both of equal high predictive power (p value ≤ 0.001) with 100% sensitivity for both and specificity of 76.10% and 82.39% respectively, with a cut off value of ≤ 10 mm and 11 mm for these dimensions respectively, and odds ratio of incidence of difficult intubation is 4.18 and 5.68 respectively, while body weight ≤ 5.75 kg has an odds ratio of 2.32. Conclusion Alveolar cleft and maximum cleft width can be used as predictors for anticipation of difficult laryngoscopy and intubation infant patients with unilateral complete cleft lip and palate, while body weight ≤ 5.75 kg increases the risk more than twice.


1990 ◽  
Vol 9 (3) ◽  
pp. 147-153 ◽  
Author(s):  
P.R. McElhatton ◽  
F.M. Sullivan ◽  
G.N. Volans ◽  
R. Fitzpatrick

A study was carried out to investigate the outcome of pregnancy in 115 women who had been exposed to paracetamol overdose. Follow up was obtained in 48 cases. Exposure occurred in all trimesters, and the most striking feature of this series is that the majority of the pregnancy outcomes were normal. None of the mothers died. There were 39 live born infants with no malformation, 14 of whom had been exposed in the first trimester. Four babies, exposed in the third trimester had neonatal problems, but these seem unrelated to paracetamol. There were two live born infants with gross malformations (spina bifida occulta; and cleft lip and palate). However, as the overdoses occurred at weeks 26 and 28 respectively, long after the structural development of these organs, the malformations could not have been caused by the paracetamol. There were two spontaneous abortions, both in the first trimester, which occurred two weeks after the overdose which may be related to the paracetamol. The overall conclusion is that paracetamol overdose per se is not necessarily an indication for termination of pregnancy.


Author(s):  
А.А. Мамедов ◽  
Ю.О. Волков ◽  
А.А. Корсунский ◽  
С.А. Паршикова ◽  
Л.А. Мазурина ◽  
...  

Накопленный опыт детской хирургии новорожденных и современные методы диагностики (компьютерная и магнитно-резонансная томография, пренатальная диагностика) позволили изменить существующие сроки коррекции врожденных пороков лица. В статье подробно описана «Система оказания помощи детям с расщелиной губы и нёба в период новорожденности», разработанная и внедренная на кафедре стоматологии детского возраста и ортодонтии Сеченовского университета. Сформулированы необходимые условия для оказания помощи данной категории пациентов и показания к предхирургической подготовке, обоснована тактика лечения, анестезиологического обеспечения у данной категории новорожденных. В работе приведены результаты ранней хирургической коррекции врожденных пороков лица 46 новорожденным с расщелиной губы и нёба. Детей с расщелиной губы и альвеолярного отростка разделили на 2 группы. В первую группу (n = 30) вошли пациенты, у которых диастаз в области альвеолярных отростков составлял 10 мм и менее. В этом случае сразу выполнялась хейлоринопластика. Во вторую группу (n = 16) вошли дети с диастазом альвеолярного отростка верхней челюсти более 10 мм. Этим новорожденным проводилась предхирургическая ортодонтическая подготовка в течение 10-12 дней (установка ортоимплантатов и наложение эластической тяги – цепочки). Критерием готовности к операции являлось достижение ширины диастаза между фрагментами альвеолярного отростка 10 мм и менее. После этого проводилось одномоментное удаление ортоимплантатов и операция – хейлоринопластика. Новорожденным с расщелиной твердого и мягкого нёба (n = 4) выполнялась уранопластика по методике А. А. Мамедова. У всех пациентов достигнуты хорошие эстетические и функциональные результаты. The accumulated experience of pediatric surgery of newborns and diagnostic methods (CT, MRI, prenatal diagnostics) have made it possible to change the existing terms for the correction of congenital facial defects. The article describes in detail «A system of care for children with cleft lip and palate in the neonatal period», developed and implemented at the Department of Pediatric Dentistry and Orthodontics, Sechenov University. The necessary conditions for rendering assistance to this category of patients are described, indications for pre-surgical preparation are formulated, the tactics of treatment, anesthetic management in this category of newborns are substantiated. The article presents the results of early surgical correction of 46 newborns with cleft lip and palate. Children with cleft lip and alveolar bone were divided into 2 groups. The first group (n = 30) included patients whose diastasis in the area of the alveolar processes was 10 mm or less. In this case, cheilorinoplasty was performed immediately. Group 2 (n = 16) included children with alveolar ridge diastasis of the upper jaw of more than 10 mm. This group of newborns underwent pre-surgical orthodontic preparation for 10-12 days, which consisted of installing ortho-implants and applying an elastic traction chain. The criterion for readiness for surgery was the achievement of a diastasis width between the fragments of the alveolar process of 10 mm or less. This was followed by simultaneous removal of ortho-implants and surgery – cheilorinoplasty. Newborns with a cleft of the hard and soft palate (n = 4) underwent uranoplasty according to the method of A. A. Mamedov. All patients received good aesthetic and functional results.


2018 ◽  
Vol 55 (9) ◽  
pp. 1296-1301 ◽  
Author(s):  
Yanfen Yang ◽  
Hui Liu ◽  
Ruixin Ma ◽  
Lei Jin

Objective: To estimate the cleft lip with or without cleft palate (CL/P) prevalence among births between 2006 and 2012 in Fangshan district of Beijing, China. Design: Surveillance data analysis. Setting: All hospitals that provide obstetric services in the district. Patients: The CL/P cases presented for this report were from 13 weeks’ gestation to 7 days postpartum. Main Outcome Measures: The CL/P prevalence was defined as the number of cases per 10 000 births, including live births and stillbirths at 28 weeks’ gestation or beyond. Results: The overall CL/P prevalence was 18.9 (95% confidence interval [CI]: 15.1-22.7) per 10 000 births. From 2006 to 2012, the CL/P prevalence was 19.3, 20.2, 10.9, 16.1, 17.5, 25.4, and 22.3 per 10 000 births; annually, no significant change was noted ( Pfor trend = .311). The prevalence of cleft palate, cleft lip, and cleft lip and palate were 3.4 (95% CI: 2.0-5.4), 6.2 (95% CI: 4.2-8.8), and 9.4 (95% CI: 6.9-12.4) per 10 000 births, respectively. The CL/P prevalence among the nonpermanent residents (31.4 per 10 000 births) was 2.31 times that of permanent residents (13.6 per 10 000 births). Among nonpermanent residents, the CL/P prevalence showed an upward trend over the study period ( Pfor trend = .036), that increased from 38.8 (95% CI: 16.5-76.6) per 10 000 births in 2006 to 54.6 (95% CI: 25.7-100.4) per 10 000 births in 2012. Conclusions: The overall CL/P prevalence was stable in the Fangshan district. However, the CL/P prevalence of the nonpermanent residents increased significantly.


2006 ◽  
Vol 43 (5) ◽  
pp. 563-570 ◽  
Author(s):  
Yu-Fang Liao ◽  
Michael Mars

Objective: To evaluate the effect of timing of hard palate repair on facial growth in patients with cleft lip and palate, with special reference to cranial base, maxilla, mandible, jaw relation, and incisor relation. Design: A systematic review. Methods: The search strategy was based on the key words “facial growth,” “cleft lip palate,” and “timing of (hard) palate repair.” Case reports, case-series, and studies with no control or comparison group in the sample were excluded. Results: Fifteen studies met the selection criteria. All the studies were retrospective and nonrandomized. Five studies used cephalometry and casts, seven used cephalometry, and three used casts. Methodological deficiencies and heterogeneity of the studies prevented major conclusions. Conclusion: The review highlights the importance of further research. Prospective well-designed, controlled studies, especially targeting long-term results, are required to elucidate the effect of timing of hard palate repair on facial growth in patients with cleft lip and palate.


2009 ◽  
Vol 46 (5) ◽  
pp. 549-554 ◽  
Author(s):  
Elżbieta Gawrych ◽  
Joanna Janiszewska-Olszowska ◽  
Anna Walecka ◽  
Maria Syryńska ◽  
Hanna Chojnacka

Holoprosencephaly is a congenital abnormality of the prosencephalon associated with median facial defects. Its frequency is 1 in 250 pregnancies and 1 in 16,000 live births. The degree of facial deformity usually correlates with the severity of brain malformation. Early mortality is prevalent in severe forms. This report presents a child with lobar holoprosencephaly accompanied by median cleft lip and palate. The treatment and 9 months’ follow-up are presented. This unique case shows that holoprosencephaly may present different manifestations of craniofacial malformations, which are not always parallel to the severity of brain abnormalities. Patients with mild to moderate brain abnormalities may survive into childhood and beyond.


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.


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