What causes aqueductal stenosis?
Aqueductal stenosis is one of the known causes of hydrocephalus and the most common cause of congenital (present at birth) hydrocephalus. It can also be acquired during childhood or adulthood. In some cases, this is due to a brain tumor compression (such as a pineal tumor) surrounding the aqueduct of Sylvius.
Which of the following is caused by stenosis of aqueduct of Sylvius?
Aqueductal stenosis is a narrowing of the aqueduct of Sylvius which blocks the flow of cerebrospinal fluid (CSF) in the ventricular system. Blockage of the aqueduct can lead to hydrocephalus, specifically as a common cause of congenital and/or obstructive hydrocephalus….
| Aqueductal stenosis | |
|---|---|
| Specialty | Neurosurgery |
How common is aqueductal stenosis?
Epidemiology. Congenital aqueductal stenosis has an estimated incidence of ~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.
Is aqueduct stenosis hereditary?
Hydrocephalus due to congenital stenosis of aqueduct of sylvius is inherited in an X-linked recessive manner. A condition is X-linked if the responsible gene is located on the X chromosome .
What causes aqueductal stenosis in fetus?
Prenatally, acquired causes are most commonly intrinsic, resulting from infection (aqueduct gliosis/web) or intraventricular hemorrhage. Extrinsic causes are less common in the prenatal period and include tectal plate mass, periaqueductal vascular malformation, or compression from a ventricular diverticulum.
Where is CSF most likely to get blocked?
The most common site of this blockage is at the cerebral aqueduct, called aqueductal stenosis. Though this occurs more commonly in children, it can present in adulthood. Other locations of blockage can occur at the third or fourth ventricles.
What is the treatment of aqueductal stenosis?
The treatment of choice is often CSF shunt diversion, while endoscopic third ventriculostomy and cerebral aqueductoplasty are alternative surgical therapies in selected patients (Cinalli et al., 2011). Fig. 58.1. Aqueductal stenosis.
How is aqueductal stenosis treated?
There are currently two alternate forms of surgical treatment for AS; shunt surgery and ventriculostomy. Shunt surgery is associated with high complication rates and many patients need revisions, but the effectiveness is high.
How is Aqueductal stenosis treated?
What causes Aqueductal stenosis in fetus?
How is aqueductal stenosis diagnosed?
The in utero diagnosis of aqueductal stenosis was confirmed by postnatal CT, MR imaging, sonography, or autopsy.
What is the most common cause of hydrocephalus in utero?
True fetal hydrocephalus has a variety of causes. The most common form of isolated, obstructive hydrocephalus is so-called “aqueductal stenosis,” which is the blockage of CSF passage through the aqueduct of Sylvius. It accounts for up to 20 percent of cases of fetal hydrocephalus.
Is there a connection between hydrocephalus and aqueductal stenosis?
Aqueductal stenosis is one of the known causes of hydrocephalus and the most common cause of congenital (present at birth) hydrocephalus. It can also be acquired during childhood or adulthood. In some cases, this is due to a brain tumor compression (such as a pineal tumor) surrounding the aqueduct of Sylvius. Symptoms are related to hydrocephalus.
How often does aqueductal stenosis occur in males?
Aqueductal stenosis. Aqueductal stenosis (AS) accounts for the majority of cases of nonsyndromic congenital hydrocephalus (Adle-Biassette et al., 2013 ). While it most often presents during infancy, it may manifest at any point in life from birth to adulthood. Males are affected nearly twice as often as females.
What are the symptoms of aqueduct stenosis ( X )?
In X-linked form ( Bickers-Adams-Edwards syndrome ), which is associated with profound intellectual disability, clinical assessment would reveal bilateral adducted thumbs. The usual symptoms and signs of raised intracranial pressure and chronic hydrocephalus may also be present, including headache, vomiting, decreased conscious state 3.
How is aqueductal stenosis transmitted from mother to son?
A genetic disorder called “Brickers-Adams-Edwards syndrome” or “X-linked hydrocephalus” has been discovered that leads to aqueductal stenosis. This disease is transmitted from mother to son. This disorder is caused by a point mutation in the gene for neural cell adhesion.