|Year : 2013 | Volume
| Issue : 1 | Page : 6-8
Clinical and echocardiographic findings in an African pediatric population of cleft lip/palate patients: A preliminary report
Barbara E Otaigbe1, Oladimeji A Akadiri2, Joycelyn O Eigbobo2
1 Department of Pediatrics, University of Port Harcourt Teaching Hospital, Rivers State, Nigeria
2 Department of Dentistry, University of Port Harcourt Teaching Hospital, Rivers State, Nigeria
|Date of Web Publication||21-Sep-2013|
Barbara E Otaigbe
Department of Paediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State
Source of Support: None, Conflict of Interest: None
Background: Every congenital structural defect in the body represents an inborn error in morphogenesis and may affect one or more systems and Orofacial clefts are one of the commonest birth defects.
Objective: Cleft lips and/or palate are frequently associated with other congenital anomalies including cardiovascular.
Methods: All pediatric orofacial cleft patients presenting for free treatment during a surgical outreach at the University of Port Harcourt Teaching Hospital were examined by the pediatric cardiologist, pedodontist, oral and maxillofacial surgeon, and anesthetist.
Results: Of the 20 patients studied, three (15%) had congenital heart disease (CHD), 1 (5%) had skeletal anomaly and one of the children with CHD had an ileo-vaginal hernia (digestive and urogenital system anomaly).
Conclusions: The most common associated congenital abnormality was CHD in 15% of children.
Keywords: African, congenital heart, orofacial cleft, pediatric
|How to cite this article:|
Otaigbe BE, Akadiri OA, Eigbobo JO. Clinical and echocardiographic findings in an African pediatric population of cleft lip/palate patients: A preliminary report. Nig J Cardiol 2013;10:6-8
|How to cite this URL:|
Otaigbe BE, Akadiri OA, Eigbobo JO. Clinical and echocardiographic findings in an African pediatric population of cleft lip/palate patients: A preliminary report. Nig J Cardiol [serial online] 2013 [cited 2020 Jul 13];10:6-8. Available from: http://www.nigjcardiol.org/text.asp?2013/10/1/6/118574
| Introduction|| |
Cleft lips and/or palate are frequently associated with other congenital anomalies.  However, the probability of this co-occurrence is not yet fully understood.  What is known most certainly is that combined orofacial and other congenital birth defects is more frequent than would be expected by chance.  About 70% of cases of cleft lip are believed to occur as isolated abnormalities and therefore are not part of a syndrome. By contrast, isolated cleft palate or combined cleft lip and palate are associated with a high incidence of other birth defects, and so have been suspected to be majorly syndromic.  More than 300 syndromes have now been described involving the combination of a cleft with multiple other congenital abnormalities.  In fact, it has been suggested that there is a connection between the severity of clefting defect and presence of additional malformations. ,
The reported prevalence of associated congenital malformations in cleft patients varies widely. ,,,,,,,,, Authors have reported incidence of associated anomalies varying from as low as 4.3%  to as high as 63.4%. 
There are contrasting reports on which system is most commonly affected. Cardiovascular malformations were found to be the most common in some studies. ,,, while skeletal/extremities malformations were observed in others. ,,,
In Nigeria, there are only few literatures reporting relative frequency of congenital anomalies co-occurring with facial cleft. ,, In fact, no previous report was found from Port Harcourt; an oil rich city in the Niger Delta region where there has been claims of increasing risks to child and maternal health as a result of environmental degradation and industrial pollution attributed to petroleum mining activities in the area.  We undertook a comprehensive clinical evaluation of a pediatric population of orofacial cleft patients as a preliminary investigation of prevalence and relative frequencies of congenital anomalies co-occurring with cleft lip and palate at the University of Port Harcourt Teaching Hospital.
| Materials and Methods|| |
A cross-sectional study of all pediatric orofacial cleft patients presenting for free treatment during a surgical outreach at the University of Port Harcourt Teaching Hospital in April, 2012 was conducted. Informed consent was obtained and parents/children were interviewed to elicit demographic information and relevant history. The subjects were thoroughly examined by a specialist team comprising pediatrician, pedodontist, oral and maxillofacial surgeon, and anesthetist. They were specifically screened for congenital malformations and routine Doppler echocardiography with color-flow mapping was achieved in all cases. The echocardiographic studies were performed by a pediatric cardiologist using a portable Sonosite Macromaxx Machine. SPSS version 17.0 was used for analysis.
| Results|| |
All twenty children presented with orofacial clefts at the surgical outreach and were included in the study. There were ten males and ten females within the age range of 3 months-16 years. Maternal and paternal age ranges were 18-38 years and 21-49 years, respectively. Gestational age was 37 weeks in 18 cases while delivery was postterm in 2 cases. There was a positive history of ingestion of herbs and drugs in one of patient and a positive family history of a father and a sibling with cleft lip and palate in only one case. All subjects were stable clinically, Heart rate (HR) was within 67-152 beats/min and partial pressure of oxygen (SPO 2 ) was between 96 and 99 mmHg.
There were more cases of isolated cleft palate among the patients with all patients with combined cleft and palate being males [Table 1]. Screening for associated congenital malformations was positive in four (20%) children. One (5%) had strictly skeletal malformations consisting of constriction bands, a missing phalangeal bone, syndactyly, and clubfoot; all suggestive of amniotic band syndrome and three (15%) presented with cardiovascular malformations. One patient with an acyanotic congenital heart defect had an ileovaginal hernia. All patients had successful repair of orofacial clefts. The echocardiographic findings revealed that three (15%) had congenital heart disease (CHD). All three children were aged between 4 months and 1 year.
The association of congenital heart anomalies with cleft types are presented in [Table 2] with isolated cleft lip being associated with multiple cardiovascular malformations.
|Table 2: Characteristics of cleft patients with other associated congenital heart disease|
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| Discussion|| |
Every congenital structural defect in the body represents an inborn error in morphogenesis and may affect one or more systems.  Orofacial clefts are one of the most common birth defects and may be associated with other congenital anomalies. 
The prevalence of associated anomalies with orofacial clefts is said to differ among different populations.  The prevalence of 20% seen in this study is similar to studies reported from Sweden with 21%,  Jordan  14.3%, Minnesota 32%,  and China 30%. 
In this study anomaly of the cardiovascular system was seen in 15%, all being CHD. This is lower than 45.5,  45.1,  44,  and 24%  reported from Jordan, China, Taiwan, and Sweden, respectively. This is probably due to the fewer study population in this cross-sectional study. The commonest CHD was acyanotic CHD; isolated ventricular septal defect (VSD) and in combination with a patent ductus arteriosus (PDA) (VSD/PDA), while atrial septal , and conotruncal defects  have been reported as common in other studies.
The other commonly associated anomalies in other studies were of the skeletal and central nervous system (CNS), which differs from our study where no CNS anomaly was noted probably because our study group was made of mainly infants while other studies involved mainly older children and adults.
The amniotic band syndrome resulting in multiple musculoskeletal anomalies is linked to disruption, a rare anomaly related to breakdown of the original normal fetal developmental process resulting in craniofacial cleft resulting from amniotic bands. 
We also noted an ileovaginal hernia in a child with cleft lip and a ventricular septal defect. The association of the digestive (such as anorectal atresia/stenosis) and urogenital (inguinal hernia) systems with orofacial cleft has been reported.  However, a careful search of literature shows that this is the first case of ileovaginal hernia being reported in the world among cleft lip/palate patients.
| Conclusion|| |
The high incidence (20%) of associated anomalies in patients with orofacial clefts noted in this study justifies the need for all patients to be examined by a pediatrician before surgical intervention. With cardiovascular anomalies accounting for 15% of all the anomalies, the use of echocardiographic evaluation of all patients is also recommended.
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[Table 1], [Table 2]