Demographic and clinical profile of craniofacial clefts at Comprehensive Rehabilitation Service in Uganda

Abstract

Background: The true incidence of Craniofacial cleft (CFC) is unknown because of their scarcity and because of the difficulty in recognizing sometimes subtle physical findings in mild malformations. Craniofacial anomalies in the African population are reported infrequently. Aim: To contribute to the general literature on rare CFC in Uganda and Africa. Methods: we conducted a retrospective search of patient data over the period 2005 to May 2017 in the unit of plastic surgery of CoRSU (Comprehensive Rehabilitation Service in Uganda) hospital, a tertiary hospital in Uganda. Patient with a diagnosis of CFC were picked out. Sixty-six patient's files with clinical diagnosis of CFC including their clinical photographs were found. Frequency data was generated and a frequency distribution table with the observed data was constructed. Results: Sex distribution showed no significant difference between male and female (1:1,2);the age on admission ranged from 1 day to 83 years; according to the laterality of the cleft, unilateral CFC (left or right side) are more common than midline clefts (Tessier 0; 14; 0,14;30); however, according to the clinical type, Tessier cleft (TC) 0 is the most common TC in our series and is associated with holoprosencephaly. Fifty percent of CFC in our series are syndromic. TC 7 are common in male and have a bilateral predilection. Conclusion: CFC are a rare set of malformations for which there is a paucity of literature. There is a need to conduct a study with a larger series including CT-Scan in order to analyze more accurate clinical diagnosis. Keywords: Craniofacial, Cleft, Tessier, Uganda Résumé Contexte: L'incidence réelle de la fente craniofaciale est inconnue en raison de sa rareté et de la difficulté à reconnaître des signes physiques parfois subtils lors de malformations légères. Les anomalies craniofaciales dans la population africaine sont rarement rapportées. But: Contribuer à la littérature générale sur les fentes craniofaciales rares en Ouganda et en Afrique. Méthodes: nous avons effectué une recherche rétrospective des données des patients sur la période 2005 à Mai 2017 dans l'unité de chirurgie plastique de l'hôpital CoRSU (Comprehensive Rehabilitation Service in Uganda), un hôpital tertiaire en Ouganda. Les patients avec un diagnostic de fente craniofaciale ont été sélectionnés. Soixante-six dossiers de patients avec un diagnostic clinique de fente craniofaciale, y compris leurs photographies cliniques ont été trouvés. Des données de fréquence ont été générées et des tableaux de distribution de fréquence avec les données observées ont été construits. Résultats: Les fentes craniofaciales constituent une malformation rare. La distribution par sexe n'a pas montré de différence significative entre les hommes et les femmes (1: 1,2), l'âge à l'admission variait de 1 jour à 83 ans; selon la latéralité de la fente, les fentes craniofaciales unilatérales (côté gauche ou droit) sont plus fréquentes que les fentes médianes (Tessier 0;14; 0,14; 30); cependant, selon le type clinique ...

Rare primary craniofacial clefts: pattern, challenges, and management in a Nigerian population

“…The present study revealed no gender predilection in the occurrence of rare craniofacial cleft, which is consistent with previous studies [8,9,11]. However, some researchers reported a male predominance [10,12].…”

“…The wide variation in age of the investigated patients was similar to findings from African studies [8,11], which was in contrast with other reports where most patients presented less than 2 years [10,12]. Delayed presentation in our environment remains a challenge to achieve optimal care, which is partly due to religious, socio-cultural, and economic factors.…”

“…However, some researchers reported a male predominance [10,12]. It may be that certain types of Tessier clefts tend to show gender predilection [11,12].…”

“…In the present study, Tessier number 0 cleft had the highest frequency of occurrence among the investigated patients. Moreover, Tessier number 0 has been reported [9,11,15] as the commonest type of rare craniofacial clefts. Other studies reported a preponderance of Tessier number 1 and 6 clefts [8,10], respectively.…”

“…Disorders associated with rare craniofacial clefts include Treacher-Collins and Gardner's syndromes, and hemifacial microsomia. In a review of 66 craniofacial clefts [11], half of the patients were observed to have associated syndrome, of which holopro sencephaly was the most common disorder. However, other studies [8,10], reported no associated syndromes.…”