Rejjal A, Alaiyan S, Coates R, Abuzeid M
Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Neuropediatrics. 1994 Apr;25(2):85-8. doi: 10.1055/s-2008-1071591.
Over the past few years there has been increasing awareness of the association of congenital choanal atresia (CCA) with other congenital defects, including brain abnormalities. We obtained CT scans of the brain in twenty-three consecutive patients with CCA (10 boys and 13 girls) to determine the prevalence and the scope of associated brain abnormalities. CCA was associated with known syndromes or different congenital abnormalities in 7 and 10 patients, respectively, and was an isolated finding in 6 patients. Six patients (26%) had brain abnormalities including alobar holoprosencephaly, absent corpus callosum, benign enlargement of the subarachnoid space of infancy, cerebral calcification, small right hemisphere, small ventricles and microcephaly. None of the brain abnormalities were suspected clinically. Brain abnormalities were more common in boys compared to girls (40% vs 15.4%, p = 0.18), in bilateral compared to unilateral CCA (31.3% vs 14%, p = 0.38), and in membranous compared to bony CCA (75% vs 15.7%, p = 0.02). Brain abnormalities associated with CCA may be more common than generally recognized and more so in certain subgroups of patients. Obtaining a CT scan of the brain should be considered in all patients with CCA.
在过去几年里，人们越来越意识到先天性后鼻孔闭锁（CCA）与包括脑部异常在内的其他先天性缺陷之间的关联。我们对连续23例CCA患者（10名男孩和13名女孩）进行了脑部CT扫描，以确定相关脑部异常的患病率和范围。分别有7例和10例CCA患者与已知综合征或不同先天性异常相关，6例患者为孤立性发现。6例患者（26%）存在脑部异常，包括无脑叶全前脑畸形、胼胝体缺如、婴儿期蛛网膜下腔良性扩大、脑钙化、右半球小、脑室小和小头畸形。临床上均未怀疑有脑部异常。与女孩相比，男孩脑部异常更常见（40%对15.4%，p = 0.18）；与单侧CCA相比，双侧CCA更常见（31.3%对14%，p = 0.38）；与骨性CCA相比，膜性CCA更常见（75%对15.7%，p = 0.02）。与CCA相关的脑部异常可能比普遍认识到的更常见，在某些亚组患者中更是如此。所有CCA患者均应考虑进行脑部CT扫描。
