Congenital heart defects in children with cleft lips and/or palates at an academic hospital in central South Africa

Background: Globally, cleft deformities are the most common craniofacial anomalies and show an association with congenital heart defects. Little research on cleft lips and/or palates (CL/P) and congenital heart defects has been reported from Africa, and none from South Africa. In 2001, it was proposed that CL/P be listed as one of six priority conditions for monitoring and notification to South African health authorities. This goal of creating a national registry has not been achieved. A near-fatal anaesthetic incident following a missed cardiac lesion in a child with a cleft lip and palate prompted this study. Objectives: To describe the prevalence of congenital heart defects diagnosed in children with CL/P presenting for corrective surgery during the Smile Week over three consecutive years (2013­2015) at an academic hospital in South Africa. Methods: A retrospective, descriptive file review of 62 patients with CL/P was performed. Since 2013, echocardiography has been performed on all patients with CL/P. Results: Twenty-three, 21 and 18 patients were operated in 2013, 2014 and 2015, respectively. Of these patients, 85.5% (n = 53) had no clinical evidence of a cardiac defect, of which eight did have clinically significant cardiac defects on echocardiography. Sixteen patients (25.8%) in total (n = 16/62) with a cleft deformity had a clinically significant congenital heart lesion. Of the 16 patients with a cardiac defect on transthoracic echocardiography, only four had clinical evidence of cardiac defect. Therefore, sensitivity of clinical examination was 25%, whereas the specificity was 89.1%. Three of the four patients with a syndrome had a clinically significant echocardiographic finding. Conclusion: A national guideline for the preoperative care of patients with CL/P, including routine echocardiography, is needed. Furthermore, a national registry is required for patients with CL/P with associated congenital anomalies

Demographic and clinical profile of craniofacial clefts at comprehensive rehabilitation service in Uganda

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

Associated anomalies in cleft lip and palate: Analysis of 811 consecutive patients

Introduction: Clefts are common birth defects and may be associated with oro-facial congenital anomalies. It has not been established if specific types of anomalies are frequently related with clefts, or which organ is most commonly affected. This study aimed to assess the prevalence of associated anomalies in consecutive cleft lip and palate patients treated at two referral centres in Northern Nigeria.Methods: Cleft lip and palate at two referral hospitals in Northern Nigeria from January 2012 to December 2015 were studied. Data were analysed using Statistical Package for Social Sciences (SPSS) version 16.Results: A total of 811 cleft lip and palate patients were managed. Fifty-five percent (447) were male and 45% (364) were female while 71% (578) were children and 29% (233) were adults. The prevalence of associated anomalies was 11.5%. The most common associated anomaly among cleft patients was facial anomaly (64% of cleft patients). Associated anomalies were most prevalent in patients with isolated cleft palate. Hypertelorism was the commonest type of facial anomaly recorded.Conclusion: Our study showed a low incidence of associated anomalies with a higher incidence in isolated cleft palate cases

The impact and cost-effectiveness of the Amref Health Africa-Smile Train Cleft Lip and Palate Surgical Repair Programme in Eastern and Central Africa

Introduction: cleft lip with or without cleft palate (CLP) is a congenital malformation that causes significant morbidity in low and middle income countries. Amref Health Africa has partnered with Smile Train to provide CLP surgeries since 2006.Methods: we analyzed anonymised data of 37,274 CLP patients from the Smile Train database operated on in eastern and central Africa between 2006 and 2014. Cases were analyzed by age, gender, country and surgery type. The impact of cleft surgery was determined by measuring averted Disability-Adjusted Life Years (DALYs) and delayed averted DALYs. We used mean Smile Train costs to calculate cost-effectiveness. We calculated economic benefit using the human capital approach and Value of Statistical Life (VSL) methods.Results: the median age at time of primary surgery was 5.4 years. A total of 207,879 DALYs were averted at a total estimated cost of US$13 million. Mean averted DALYs per patient were 5.6, and mean cost per averted DALY was $62.8. Total delayed burden of disease from late age at surgery was 36,352 DALYs. Surgical correction resulted in $292 million in economic gain using the human capital approach and $2.4 billion using VSL methods.Conclusion: cleft surgery is a cost-effective intervention to reduce disability and increase economic productivity in eastern and central Africa. Dedicated programs that provide essential CLP surgery can produce substantial clinical and economic benefits

Neonatal Cleft Lip Repair in Babies with Breastfeeding Difficulties at Polokwane Mankweng Hospital Complex

Background. A cleft lip (CL) is a congenital abnormality resulting from failure of union of the medial and nasal prominences with the maxillary prominence during embryonic development. CL may be classified as incomplete; complete; unilateral; bilateral or median. It may be associated with a cleft alveolus or a cleft palate. Definitive correction of a cleft lip is by surgery. In most African settings; the birth of a cleft lip and cleft palate (CLP) baby is associated with witchcraft and ancestral spirits. The parents; particularly mothers; are stigmatised.Objective. To repair CLs in neonates with difficulties in breastfeeding. Methods. Non-syndromic term neonates referred to Polokwane Mankweng Hospital Complex (PMHC) from primary and secondary hospitals with CLP and difficulties in breastfeeding were prospectively admitted to the neonatal unit. Our breastfeeding team supervised and assisted them with breastfeeding. The neonates whose breastfeeding was found to be unsatisfactory were considered for neonatal CL repair.Those who breastfed adequately were booked for later lip repair as per the rule of tens and discharged.Results. From June 2009 to March 2012; 60 children with CLP were referred to PMHC; including 36 neonates. Of these; 23 neonates were unable to breastfeed satisfactorily and were operated at a median age of 9 (range 3 - 28) days. The median weight was 2.8 (1.8 - 3.7) kg. The median haemoglobin was 13.1 (11.5 - 16) g/dL. Conclusion. Neonatal CL repair is an alternative for those with breastfeeding difficulties. Eagerness to breastfeed increased following the lip repair with subsequent improvement in maternal confidence and interaction with the baby. At follow up; weight gain was above the 50th centile on the road to health charts. Early surgery prevents exposure of CL to the public with highly positive possible outcome of decreasing the potential for stigmatisation

Les Fentes labio-palatines non syndromiques a l'est de la Republique Democratique du Congo. Aspects epidemiologiques; cliniques et therapeutiques

But de l'etude:decrire le profil epidemiologique; clinique et therapeutique des fentes labio-palatines observees a l'Est de la RDC (Republique Democratique du Congo) de novembre 2010 a mars 2011 soit durant 5 mois.Malades et methode:c'est une etude descriptive transversale realisee au Centre Hospitalier 'Doctors on Call for Service (DOCS)' de Goma en RDC et qui a porte sur 160 cas de fentes labiales et/ou palatines selectionnees au moyen d'un echantillonnage non probabiliste; a tout venant et operees au cours de cette meme periode. Le traitement des donnees a ete facilite par l'usage du progiciel SPSS 17.0. Les resultats ont ete valides par le test de Khi-carre au seuil de 0;05.Resultats:la majorite de nos patients (76;3) sont venus des milieux ruraux et ont a la consultation et a l'operation un age moyen de 15;19 ans avec comme extreme trois mois et 62 ans; dont 47;5 des cas sont operes apres l'age de 10 ans. Le manque de moyen financier (58;7 des cas) suivi par le manque d'information (22;5) constituent les principaux motifs du retard a la consultation et a l'operation. Les deux sexes sont concernes avec une predominance masculine (60;6). La scolarisation de ces patients est significativement plus faible pour les sujets feminins (54;5 non scolarisees) par rapport aux sujets masculins (45;5 non scolarises) (x2=15;417; ddl=3; p=0;001). Il y a plus d'hommes maries que d'hommes celibataires ayant atteint la maturite ; l'inverse est observe chez les sujets feminins (x2=1;137; ddl=2; p=0;556).La fente labiale isolee est le type le plus frequent (97;5); elle est unilaterale dans 81;9 des cas; incomplete (50;1) et siegeant a gauche (49;9)(x2=10;055 ;ddl=9 ;p=0;346). Aucun antecedent heredo-collateral de malformation congenitale n'a ete enregistre chez 96;3 des cas.La technique de Millard modifiee a ete utilisee dans 94;2 des cas pour corriger la fente labiale et toutes les fentes palatines ont ete corrigees par la technique de Von Langenbeck. L'anesthesie generale avec intubation orotracheale (IOT) a ete utilisee chez tous les enfants d'age inferieur ou egal a cinq ans alors que l'anesthesie locale a ete utilisee chez 96;4 des patients ages de 16 ans et plus (x2=152;182; ddl=6; p=0;000). Nous n'avons enregistre aucune complication postoperatoire dans 98;8 des cas et la duree moyenne d'hospitalisation est de deux jours

Epidemiology of orofacial clefts in Africa: methodological challenges in ascertainment

BACKGROUND:To carry out a systematic review of the birth prevalence of cleft lip with or without cleft palate (CLP) and cleft palate (CP) in Africa based on available published data.METHOD:Using the Cochrane search strategy and the following keywords words "cleft palate", "prevalence", "incidence", "cleft lip" and "Africa" to screen Ovid Medline {1966 to March 2007), Cinahl {1982-March 2007}, PubMed, Scopus, and Web-Google. All identified published, prospective and retrospective studies on the birth prevalence of CLP and CP in Africa were included. The dates, location, sources, number of births (live births, still births, number of cleft cases, prevalence rates, sex ratio, cleft types, and clefts with associated anomalies were extracted.RESULTS: Ascertainment of cases was through the hospitals. Overall there were 57 CL/P, 56 CL and 36 CP reported from all the studies. From seven studies combined, 21 males and 20 females had CL, 10 males and 22 females with CP and 26 males and 24 females with CL/P. There were 3 cases with CL/P, 2 with CP and 2 with CL from the three studies that reported clefts with associated anomalies.CONCLUSION: For an improved ascertainment of cleft cases, there is a need to set up a birth defects surveillance system in the form of a national birth registry. Future studies should then aim to include the entire population in geographically defined regions. Reliable data on incidence is an essential pre-requisite for studies into aetiology and prevention