Aqueductal stenosis in infancy [Neurosurgery Wiki]

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====== Aqueductal stenosis in infancy ======

[[Aqueductal stenosis]] (AqS) is a frequent cause of [[congenital hydrocephalus]] (HCP) (up to 70% of cases
((Section of Pediatric Neurosurgery of the American
Association of Neurological Surgeons. Pediatric Neurosurgery. New York 1982))), 
but occasionally may be the result of HCP. Patients with congenital AqS usually have HCP at birth or develop it within ≈ 2–3 mos. Congenital AqS may be due to an X-linked recessive gene
((Matson DD. Neurosurgery of Infancy and Childhood. 2nd ed. Springfield: Charles C Thomas; 1969)).

Four types of congenital AqS described by Russell (summarized
((Nag TK, Falconer MA. Non-Tumoral Stenosis of the Aqueduct in Adults. Brit Med J. 1966; 2:1168–1170))):

1. forking: multiple channels (often narrowed) with normal epithelial lining that do not meet, separated by normal nervous tissue. Usually associated with other congenital abnormalities (spina bifida, myelomeningocele)

2. periaqueductal gliosis: luminal narrowing due to subependymal astrocytic proliferation

3. true stenosis: aqueduct histologically normal

4. septum