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The Korean Journal of Pathology 1994;28(5): 514-521.
Aqueductal Atresia with Forking Anomaly: Report of 3 cases.
Na Hye Myong, Mi Kyung Kim, Je G Chi
1Deparment of Pathology, Dankook University, Chunan, Korea.
2Deparment of Pathology, Seoul National University, College of Medicine, Seoul, Korea.
ABSTRACT
Aqueductal forking was first described by Russell (l949) as a cause of aqueductal obstruction and a form of congenital malformation with simple stenosis, it is a relatively common cause of congenital hydrocephalus not associated with spina bifida or meningomyelocele. Pathologically it is characterized by two distinct channels separated by non-gliotic brain tissue. We describe variable clinicopathologic findings of 3 autopsy cases showing hydrocephaly due to aqueductal atresia with forking case 1 was a 35-week-old female showing Potter's syndrome, dextrocardia, and skeletal anomaly. case 2 was a 29-week-old male abortus with micrognathia, simian crease, club feet, and minor defects of visceral organs. Case 3 was a 32-week-old female abortus with associated anomalies such as a low-set ear, ectopic thymus and thyroid, and Meckel's diverticulum. On serial sections of brain stems of all 3 cases, were seen variably shaped and atretic lumina of aqueducts with distinct two channe1s and intervening brain tissues of normal cellularity.
Key Words: Aquductal atresia with forking anomaly; Congenital hydrocephalus; Associated anomalies