Distribution of Orofacial Clefts and Frequent Occurrence of an Unusual Cleft Variant in the Rift Valley of Kenya

2007 ◽  
Vol 44 (4) ◽  
pp. 374-377 ◽  
Author(s):  
Richard A. Spritz ◽  
Thomas D. Arnold ◽  
Samuel Buonocore ◽  
David Carter ◽  
Tasha Fingerlin ◽  
...  

Objective: To investigate the pattern and distribution of nonsyndromic orofacial clefts among patients in the Rift Valley region of northwestern Kenya. Methods: Subjects were categorized anatomically for occurrence of an atypical cleft lip variant (ACL), typical cleft lip (CL), cleft lip and palate (CLP), or cleft palate (CP), and family history of orofacial clefts. Tribal ethnicity data were obtained from both cleft and noncleft clinic attendees. Results: There were 194 patients with CL (52.7%), 153 with CLP (41.6%), and 21 with CP (5.7%). CL constitutes a greater fraction of orofacial clefts in the Rift Valley region than reported elsewhere in Africa, principally due to frequent occurrence of ACL (52.5% of all CL). Among noncleft clinic attendees there was a lower fraction of Bantu and larger fraction of Nilotic-Paranilotic tribal ethnicity than in Kenya overall. In contrast, among patients with orofacial clefts there was significant underrepresentation of Bantu and overrepresentation of Nilotic-Paranilotic tribes, particularly Kalenjin. Patients of Kalenjin origin had a much higher rate of positive family history of orofacial clefts than Bantu patients. Conclusions: There is an unusual anatomic distribution of orofacial clefts in the Kenya Rift Valley, with frequent occurrence of an atypical CL variant. Our findings indicate that Bantu tribes have lower risk of orofacial clefts than Nilotic-Paranilotic tribes, possibly due to inherited genetic differences, perhaps accounting for the relatively low prevalence of orofacial clefts through much of Africa.

2020 ◽  
pp. 105566562098063
Author(s):  
Sima Dabbaghi Galeh ◽  
Masoud Nouri-Vaskeh ◽  
Mahdieh Alipour ◽  
Shahin Abdollahi Fakhim

Objective: Orofacial clefts (OFCs) can occur as an isolated defect or as a manifestation of other syndromes. The current study aimed to evaluate demographic characteristics and distribution of different types of accompanying anomalies for OFCs in the northwest of Iran. Design: A retrospective cohort study. Setting: Tertiary pediatric hospital. Patients and Participants: This study was conducted on 1500 cleft lip and/or palate patients born between July 2010 and June 2020 in the northwest of Iran. Main Outcome Measures: Demographic and clinical characteristics of the children with OFCs including familial history, accompanying anomalies and syndromes, maternal passive smoking, mothers’ and fathers’ age, consanguineous marriage, and birth order. Results: Among 1500 patients, 441 had cleft lip, 615 had cleft palate, and 444 had cleft lip and palate. The positive family history of OFCs was found to be 20.9% to 25.4% depending on the cleft type. Accompanying anomalies were identified in 29.8% of cases. Cardiac, facial, and ear abnormalities were the most common types. Also, 2.9% were identified with syndromes and sequences. These included Pierre Robin Sequence, Velo-cardio-facial syndrome, and Down syndrome most frequently. Conclusion: These findings may provide references for appropriate resources to establish and direct counseling and primary preventive projects in the northwest of Iran.


Author(s):  
Robin D. Clark ◽  
Cynthia J. Curry

This chapter reviews background information about the incidence, risk factors, family history, genetics, recurrence risk, and epidemiology of isolated and syndromic cleft lip with or without cleft palate. Microforms of cleft lip are described. The distinctive anatomy of bilateral cleft lip is contrasted with premaxillary agenesis, a wider defect caused by absence of the frontonasal process. The discussion on the differential diagnosis of cleft lip summarizes its common causes, including teratogenic agents (alcohol, maternal diabetes, phenytoin), chromosome anomalies (aneuploidy, recurrent deletion syndromes), and Mendelian clefting disorders that include associated malformations in other organ systems. The chapter gives recommendations for evaluation and management. A clinical case presentation features an infant with an oral cleft who has a positive family history of cleft lip and palate with paramedian lip pits due to Van der Woude syndrome.


2016 ◽  
Vol 83 (3) ◽  
pp. 265-268 ◽  
Author(s):  
Adrianna Mostowska ◽  
Kamil K. Hozyasz ◽  
Piotr Wójcicki ◽  
Barbara Biedziak ◽  
Joanna Wesoły ◽  
...  

The project “Searching for new genes and loci involved in cleft lip and palate in the Polish population – genome-wide association study” is a case-control study in a group of unrelated subjects with non-syndromic cleft lip with or without cleft palate (NSCL/P) and healthy individuals with no family history of clefting or other congenital disorders. The overall goal of this grant proposal is to identify novel genetic factors, which can play a significant role in the pathogenesis of orofacial clefts in the Polish population. To accomplish the proposed aim, a two stage genome-wide association study will be performed. In the first stage, Illumina's HumanOmni Express BeadChips arrays will be used to genotype over 700,000 polymorphisms in NSCL/P patients and controls. In the second stage, SNPs showing the most compelling association with the risk of orofacial clefts will be tested in an independent sample set using standard genotyping methods. This research project is expected to be completed in July 2015.


2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
P. Agbenorku ◽  
M. Yore ◽  
K. A. Danso ◽  
C. Turpin

Background. Cleft lip and cleft palate are among the most common orofacial congenital anomalies. This study is to establish Orofacial Clefts Database for Kumasi, Ghana, with a view to extend it to other cities in future to obtain a national orofacial anomaly database. Methods. A descriptive prospective survey was carried out at eleven selected health facilities in Kumasi. Results. The total number of live births recorded was 27,449. Orofacial anomalies recorded were 36, giving an incidence of 1.31/1000 live births or 1 in 763 live births. The mean maternal age of cleft lip/palate babies was 29.85 years (range 18–40 years). The male : female ratio for the orofacial anomalies babies was 1.3 : 1; the male : female ratio was 0.5 : 1 in the cleft lip group, 1.3 : 1 in the cleft lip and palate group, and 4 : 1 in the cleft palate group. The majority of clefts were unilateral (69.4%, n=25), with females (n=14) outnumbering males (n=11). A family history of cleft was recorded with five babies (13.9%). Associated congenital anomalies were recorded in seven (19.4%) cleft lips and/or palates. Conclusion. The incidence of 1 in 763 live births found in this study indicates that cleft lip/palate is a common congenital anomaly in Kumasi.


2014 ◽  
Vol 13 (3) ◽  
pp. 46-49
Author(s):  
Kazi Md Noor-ul Ferdous ◽  
Md Mafuzul Haq ◽  
S M Mashfiqur Rahman ◽  
Sabbir Karim ◽  
Md Mahabubul Alam ◽  
...  

Backgroud: Timing of surgery in the patients with cleft lip palate is an important factor of prognosis. Delaying in surgical repair of cleft lip and palate patient may lead to difficulty. It may causes wide, extensive and difficult dissection. There are always chances of wound infection, wound dehiscence, complete wound disruption, fistula formation, even there is also chance of maxillary hypoplasia and failure to articulate lifelong. The purpose of this study to observe the effects of cleft lip repair in early age on cleft alveolar and cleft palatal gap thus helps during cleft hard palate repair in patients with unilateral complete cleft lip and palate (UCLP). Methods: A retrospective study was done from January 2008 to July 2013. Patients with unilateral complete cleft lip and palate included in this study, who under went cleft lip at first admission and then and after 3 months of cleft palate was repaired. Patients with previous cleft lip-palate surgery were excluded. The gaps of cleft alveolus and posterior border of the cleft hard palate were recorded during 1st and 2nd operations. Age, gender, side of the cleft, associated anomalies, family history of cleft, cleft alveolar and cleft palatal gap noted, postoperative complications were also recorded. All the data were Results: A total of 63 patients included in this study. Age ranged from 4 months to 8 years. 38 patients were male and 25 were female. Right side was involved in 20 and 43 patients involved in left side. Eight patients had positive family history. Nine had associated congenital anomalies. Cleft alveolar and palatal gap reduced more in the patients who were below the age of 18 months. Postoperative complications were mild respiratory distress, notching of vermilion border developed oronasal fistula.Conclusion: In unilateral complete cleft lip palate patient, early cleft lip repair results, reduction of gaps of alveolar cleft and that of hard palate remarkably, especially in the patients who came for cleft lip surgery in appropriate time.DOI: http://dx.doi.org/10.3329/cmoshmcj.v13i3.21023


2016 ◽  
Vol 23 (05) ◽  
pp. 516-521
Author(s):  
Tajammal Abbas Shah

A prospective study was conducted to look for prevalence of cleft lip and palate ina population presenting to a surgical unit in a teaching hospital. Objectives: To see prevalenceof cleft lip and palate alone, lip and palate combined, right or left sided, male to femaledistribution, and possible factors responsible for clefting. Study Design: A prospective study.Setting: Surgical Unit II at Allied Hospital Faisalabad. Period: March 2009 to March 2010 forone year. Materials and Methods: Total 55 patients were treated in year 2009 out of total17900 (0.3 %) patients admitted in all surgical wards and 6508 patients admitted in surgicalunit II (0.8%). Patients were divided into three groups, cleft lip alone (group A), cleft palatealone (group B) and combined cleft lip and palate (group C). Children up to the age of 5 yearswith congenital abnormality were included in study. Results: Out of 6508 patients admitted insurgical unit II 55 patients (0.8%) had cleft lip and palate defect. 55 patients were divided inthree groups. In group A, 32 patients presented with cleft lip alone ( 58.1 % ), 16 ( 29 % ) weremales and 16 ( 29 % ) were females, 21 patients have left sided ( 38 % ), 4 right sided (7.27% )and 7 patients have bilateral ( 12.72 % ) defects. 2 patients (3.63 %) had family history of cleft lipand both were males. In group B, 12 patients ( 21.8 %) had cleft palate alone, 7 patients ( 12.72% ) were males and 5 patients ( 9 % ) were females, 10 patients ( 1.18 % ) had soft palate onlywhile 2 patients ( 3.63 % ) had compete ( hard and soft ) palatal defect. In group C, 11 patients,had cleft lip and palate combined ( 20 % ), 6 patients were males ( 10.9 % ) and 5 patients ( 9% ) were females, 8 patients ( 14.54 % ) had only soft palate defect while 3 patients ( 5.45 % )had complete palatal defect associated with 8 patients ( 14.54 % ) left sided unilateral lip defectand 3 patients ( 5.45% ) had bilateral cleft lip. All patients were operated without any mortality.Ages of mothers at earliest were 16 and 18 years, 3 cousin marriages, ( 5.45 % ) all fathers weresmokers, belonged to poor socio economic families and no history of mother’s exposure toradiation, drug abuse during gestational life. Conclusion: As it is obvious from this study thatall patients belongs to poor socio economics group, and all fathers were smokers, 3 patientsborn in parents who had cousin marriages ( 5.45 % ) 2 patients ( 3.63 % ) with family history,cleft lip and palate are multifactorial congenital abnormalities, runs in families and is influencedby various environmental factors.


2021 ◽  
pp. 105566562110543
Author(s):  
Aluísio Eustáquio de Freitas Miranda-Filho ◽  
Heloisa de Sousa Gomes ◽  
Roberta Bessa Veloso Silva ◽  
Nelson Pereira Marques ◽  
Hercílio Martelli ◽  
...  

Objective This study aimed to correlate the prevalence of iron deficiency anemia and breastfeeding with orofacial clefts in children. Design Data on the participant profile, presence and type of the cleft lip and/or palate (CL/P), and records on anemia and breastfeeding were collected from patients’ charts, and submitted to statistical analysis by χ2 test ( p < .05; software SPSS 23.0). Results Two-hundred and ten files were divided according to: CL/P presence (cleft group;    n = 132) or absence (control group;    n = 78). Group CL/P was subdivided according to the type of cleft: CL/P-I (cleft lip;    n = 35); CL/P-II (cleft lip and palate;    n = 45); CL/P-III (cleft palate;    n = 43); and CL/P-IV (rare orofacial clefts;    n = 9). Group CL/P had significantly more records on anemia ( p = .016) and fewer records on breastfeeding (P<.01) than controls. More records on anemia occurred in CL/P-II ( p = .004) and CL/P-IV ( p = .006) than the control group. The comparison among the orofacial cleft types regarding the anemia records showed no statistically significant differences ( p = .123). Group CL/P-I had more records on breastfeeding than the other cleft types ( p < .01). Conclusions Thus, it is suggested that the breastfeeding process is more complex, and the history of anemia is more frequent, in children with cleft lip and palate or rare orofacial clefts than in children without clefts.


2017 ◽  
Vol 41 (3) ◽  
pp. 236-242 ◽  
Author(s):  
Sanjida Haque ◽  
Mohammad Khursheed Alam ◽  
Mohd Fadhli Khamis

Objectives: Multiple factors are whispered to be crucial cause of unfavourable dental arch relationship in cleft lip and palate (CLP).This study aims to evaluate the dental arch relationship of Bangladeshi children with non syndromic unilateral cleft lip and palate (UCLP) following cheiloplasty and palatoplasty. Also to explore the various congenital (UCLP type, UCLP side, family history of cleft, family history of class III) and environmental (cheiloplasty, palatoplasty) factors that affects dental arch relationship of UCLP patients. Study design: This was a retrospective study where 84 dental models were taken before orthodontic treatment and alveolar bone grafting. The mean age was 7.69± 2.46 (mean± SD). The dental arch relationship was assessed by GOSLON (Great Ormond Street, London and Oslo) Yardstick. According to GOSLON Yardstick, five categories are rated; named- 1: excellent; 2: good; 3: fair; 4: poor; 5: very poor. Also the groups have been dichotomized into favorable (category ratings 1–3) and unfavorable (category ratings 4 and 5) groups. Kappa statistics was used to evaluate the intra- and inter-examiner agreements and logistic regression analysis was used to explore the responsible factors that affect dental arch relationship. Results: Total 37 subjects (44% of all subjects) were categorized into unfavourable group (category rating 4 and 5) using GOSLON yardstick. Intra- and inter-examiner agreements were very good. The mean GOSLON score was 3.238. Using crude and stepwise backward regression analysis, significant association was found between family history of skeletal class III malocclusion (p = 0.015 and p = 0.014 respectively) and unfavourable dental arch relationship. Complete UCLP (p = 0.054) and left sided UCLP (p = 0.053) also seemed to be correlated but not significant with unfavourable dental arch relationship using crude and stepwise backward regression analysis respectively. Conclusion: This analysis suggested that family history of skeletal class III was significantly correlated with unfavourable dental arch relationship of Bangladeshi UCLP children.


2008 ◽  
Vol 45 (6) ◽  
pp. 597-602 ◽  
Author(s):  
Somchit Jaruratanasirikul ◽  
Vichai Chichareon ◽  
Nuria Pattanapreechawong ◽  
Pasuree Sangsupavanich

Objective: To study the clinical characteristics, demographic data, and associated congenital anomalies of pediatric patients with cleft lip and/or palate in Southern Thailand. Design: Retrospective, hospital-based study. Setting: Cleft Clinic Center, Songklanagarind Hospital Participants: Children with cleft lip and/or palate who were born or seen at Songklanagarind Hospital between January 1997 and December 2006. Main Outcome Measure: Clinical features including demographic data, types of cleft, associated anomalies, family history of clefts, and physical growth. Results: A total of 153 children were seen during the studied period, of whom 36 (23.5%) had isolated cleft lip, 32 (20.9%) had isolated cleft palate, and 85 (55.6%) had combined cleft lip and palate. Twenty-seven children (17.7%) had a family history of clefts. Congenital malformations (syndromic cleft) were found in 20 children (13%), and chromosomal abnormalities were found in four of these (20%). There were no significant differences among the three groups (isolated cleft lip, isolated cleft palate, and combined cleft lip and palate) in maternal and paternal ages, gestational age, birth weight, family history of cleft, or associated malformations. The physical growth parameters of children with nonsyndromic cleft were the same as in the general population. Children with syndromic cleft were significantly lighter at birth and had grown up significantly shorter and lighter, with smaller head circumference. Conclusions: Chromosomal abnormalities are commonly found in children with syndromic cleft. Children with nonsyndromic cleft have normal growth; whereas, those with syndromic cleft have some degree of prenatal and postnatal growth restriction.


Author(s):  
Alexandre Rezende Vieira

Multifactorial cleft lip and palate is relatively common in populations (1 in every 700 livebirths). Individuals born with clefts require lifelong treatment after initial surgical repair and data suggested that their lifespan is shorter, possibly due to cancer or psychiatric conditions. Molecular defects that alter facial development in utero appear to later in life predispose to cancer. Common polymorphisms in e-cadherin and an endoplasmic reticulum transmembrane sensor gene appear to hold the promise to be biomarkers that may help to define individual risks to cancer, in the presence or not of family history of clefts.


Sign in / Sign up

Export Citation Format

Share Document