RESUMO
The postmortem findings in 672 Nigerian children with lethal congenital malformations are reviewed. Eighty-six percent of the patients were less than 1 year of age and the male-to-female sex ratio was 1.3:1. The alimentary tract was the most common anatomic site involved (30%), intestinal and biliary atresia being the two lesions most frequently identified in this location. Twenty-six percent of the children had cardiovascular malformations, of which ventricular septal defect, patent ductus arteriosus, and tetralogy of Fallot were most important. There was an accentuated male predominance among cases with urogenital malformations due to the high frequency of posterior urethral valve. Hydrocephalus, spina bifida, and anencephaly were the most common lesions observed in the craniospinal axis. One-fifth of the children had complex malformations of multiple organ systems, 20% of which were associated with chromosomal anomalies, rubella, and other malformation syndromes.
Assuntos
Anormalidades Congênitas/mortalidade , Anormalidades Múltiplas/mortalidade , Adolescente , Autopsia , Sistema Nervoso Central/anormalidades , Criança , Pré-Escolar , Anormalidades do Sistema Digestório , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Nigéria/epidemiologia , Razão de Masculinidade , Anormalidades UrogenitaisRESUMO
A case of polyostotic fibrous dysplasia, cutaneous pigmentation, and a bifid ureter is presented. The need for urinary tract investigation in McCune-Albright's syndrome is stressed.
