Aqueductal stenosis (AS) is a the most common cause of congenital obstructive hydrocephalus, but can also be seen in adults as an acquired abnormality.
Aqueductal stenosis can be divided into congenital and acquired, with different aetiologies and thus different demographics.
- aqueductal webs or diaphragms
Congenital aqueductal stenosis has an estimated incidence is at ~ 1 : 5000 births although the reported range varies greatly (3.7:1,000,000 to 1:2000) 5. Rarely it may be inherited in an X-linked recessive manner (Bickers-Adams-Edwards syndrome) 5.
The remainder of this article focus on congenital aqueductal stenosis.
The clinical presentation depends on the severity and age of presentation as well as whether or not it is x-linked.
In the infant enlarging head size, bulging fontanellles and gaping cranial sutures are seen. Setting sun phenomenon may also be present.
The usual symptoms and signs of raised intracranial pressure may also be present, including headache, vomiting, decreased conscious state.
Better delineates the extent of obstructive hydrocephalus, with enlargement (often marked) of the lateral and third ventricles. The aqueduct may show funnelling superiorly. The 4th ventricle is not dilated. In cases of secondary obstruction the underlying abnormality may also be evident.
An MRI CSF flow study is helpful.
Treatment and prognosis
- 1. Loevner LA. Case review, brain imaging. Mosby Inc. (1999) ISBN:032300430X. Read it at Google Books - Find it at Amazon
- 2. Mcmillan JJ, Williams B. Aqueduct stenosis. Case review and discussion. J. Neurol. Neurosurg. Psychiatr. 1977;40 (6): 521-32. doi:10.1136/jnnp.40.6.521 - Free text at pubmed - Pubmed citation
- 3. Stoquart-el sankari S, Lehmann P, Gondry-jouet C et-al. Phase-contrast MR imaging support for the diagnosis of aqueductal stenosis. AJNR Am J Neuroradiol. 2009;30 (1): 209-14. doi:10.3174/ajnr.A1308 - Pubmed citation
- 4. Levitsky DB, Mack LA, Nyberg DA et-al. Fetal aqueductal stenosis diagnosed sonographically: how grave is the prognosis? AJR Am J Roentgenol. 1995;164 (3): 725-30. AJR Am J Roentgenol (abstract) - Pubmed citation
- 5. Urbán P. Brainstem Disorders. Springer Verlag. (2011) ISBN:3642042023. Read it at Google Books - Find it at Amazon
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Stenosis of the aqueduct of Sylvius||✓|
|Perinatal aqueductal stenosis||✗|
|Perinatal aqueduct stenosis||✗|
|Perinatal aqueductal atresia||✗|