Congenital heart disease in a cohort of Sudanese patients with cleft defects

Background and objectives:Cleft defects are among the most visible congenital defects worldwide and congenital heart disease (CHD)being one of the most common associated anomalies. This study aimed to assess the prevalence of CHD among a cohort of Sudanese patients with cleft lip and/or palate.Patients and Methods:All patients who presented to Soba University Hospital with cleft lip and/or palate from March 2009 toMarch 2015 were included.Results:Out of 381 patients included; 168 patients (44%) had cleft lip and palate (CLP), 156 (41%) had cleft lip (CL)and isolated cleft palate (CP) was found in 57 patients (15%). Facial dysmorphic features were identified in 57 (15%) and cardiac defects in 42 (11%) patients. Ventricular septal defect (VSD) was diagnosed in 16 patients (38%), and ASD in 12 (30.9%). Other macroscopic anomalies were identified in 57 patients (15%)and were associated with CHD (P<0.001). Significant association was found between the type of cleft andCHD (P<0.002), as cardiac defects were maximally observed among CP cases (21%) followed by CLP cases (13%) then CL cases (5.12%). Significant association was also found between facial dysmorphicoccurrence and CHD (P < 0.001).Conclusion:CHD is a common anomaly in cleft population. The pattern of CHD is consistent with the literature withVSD being the most frequent. The cardiac defects are most prevalent in CP group. Echocardiography isjustified for screening of CHD due to the relatively higher incidence of CHD among clefts patients than ingeneral population

The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

Background. West syndrome (WS) is a rare epileptic encephalopathy of infancy. There is currently no research on the incidence or prevalence of WS in Africa.Methods. We aimed to describe the outcome of children with WS at a quaternary-level hospital in KwaZulu-Natal, South Africa (SA). This was a retrospective chart review conducted on patients diagnosed with WS over a 10-year period. Eight children (males, n=7; African, n=6; Asian, n=2) identified with WS out of 2 206 admitted with epilepsy. The median age (range) at diagnosis was 7.5 (1 - 9) months. The average time between onset of epileptic spasms and diagnosis was 3.1 months.Results. Six patients had abnormal neuroimaging (atrophy (n=2); corpus callosum agenesis (n=2); tuberous sclerosis (n=1); focal dysplasia (n=1)). Drug management included sodium valproate (n=8), topiramate (n=7) and levetiracetam (n=3). Subsequent definitive treatment was intramuscular adrenocorticotrophic hormone (n=3), vigabatrin (n=2) and oral prednisone (n=4). Four (50%) patients had complete seizure remission (neuromigratory disorder (n=2); tuberous sclerosis (n=1); and idiopathic (n=1)) and 4 had partial remission (neonatal complications (n=3); idiopathic (n=1).Discussion. Most of our patients had symptomatic WS, with 50% remission on treatment. Outcomes were poorer in our study when compared with those in published data.Conclusion. Further collaborative studies are still needed to evaluate the true impact and prevalence of WS in SA

Epilespie de l'enfant et de l'adolescent au Senegal

Introduction L'epilepsie constitue un probleme de sante publique au Senegal avec une prevalence de 8;3 a 14/1000. Elle concerne principalement les enfants. L'objectif de ce travail est d'etudier les aspects biographiques; phenotypiques et evolutifs de la maladie epileptique dans une cohorte d'enfants au Senegal. Methodologie Il s'agit d'une etude retrospective de dossiers d'enfants epileptiques suivis regulierement au CHU de FANN et a l'Hopital d'Enfants Albert Royer; de Juillet 2003 a decembre 2010. Les criteres d'inclusion etaient: epileptiques ages de moins de 18 ans; regulierement suivis depuis au moins 3 ans; ayant un traitement adapte; a dose efficace; avec une bonne observance therapeutique. Resultats Nous avons collige 522 enfants; ages de 3 mois a 16 ans; avec un sex-ratio de 1;7 en faveur des garcons. L'epilepsie etait idiopathique chez 57% des enfants et non idiopathique chez 43% des patients. Les facteurs etiologiques etaient domines par la consanguinite parentale; les anomalies de la grossesse et de l'accouchement; les infections du systeme nerveux central. Dans le groupe des epilepsies idiopathiques la consanguinite parentale et l'epilepsie familiale etaient retrouvees respectivement chez 64 enfants (21;62%) et 20 enfants (6;75%). Neuf enfants (3%) presentaient un trouble du langage isole; alors qu'un seul enfant (0;33%) avait un deficit cognitif global. Dans le groupe des epilepsies non idiopathiques; les signes associes a l'epilepsie etaient les troubles du langage (15;70%); du comportement (15%) et des deficits moteurs (10;32%). 22;41% des enfants scolarises avaient des difficultes d'apprentissage menant parfois a des redoublements scolaires ou une exclusion. Conclusion La classification syndromique a l'epilepsie est necessaire pour une bonne prevision pronostique et therapeutique. Le caractere idiopathique ou non en est pour une grande place; correle le plus souvent a une epilepsie familial ou une consanguinite ou affection perinatal ou infectieuse du systeme nerveux central

Symptomatic vitelline duct anomalies in children : paediatric surgery

A wide variety of anomalies may occur as a result of the vitelline duct (VD) failing to obliterate completely. Most reports on symptomatic VD focus on Meckel's diverticulum; while other anomalies are given little attention. A retrospective review was conducted at our institution. According to the records 18 symptomatic children with VD anomalies were seen over 22 years; including 10 boys and 8 girls aged 11 days - 14 years (median 7.5 months). Twelve patients aged below 10 years (median 28 days) had patent vitelline ducts (PVDs); 3 children aged 13 months; 13 years and 14 years respectively had Meckel's diverticulum (MD); presenting as inflammation; tapeworm incarceration and volvulus respectively. Two patients; both 8 years old; had umbilical sinus; and a 3- year-old had a vitelline cyst. Only 1 patient with PVD had an associated anomaly (intestinal malrotation). The diagnosis of PVD was obvious clinically; but in 1 patient the fistula was demonstrated by fistulogram. The diagnosis of MD was intraoperative in all 3 patients. Treatment was by various types of resection for PVD and MD and excision for umbilical sinuses and cysts. One patient with PVD developed postoperative intestinal obstruction from adhesions; requiring re-laparotomy and adhesiolysis. Two patients with PVD died from sepsis and anaestheticrelated complications; respectively. Although MD is the most commonly VD anomaly; PVD is the most common symptomatic presentation in our environment