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Congenital Aqueductal Stenosis
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Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Introduction
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Dr. Charu Venkatesen and Dr. Kara Markham opents Day 1 of the Frontiers in Fetal Neurology 2-day interactive webinar series.
video2:56 · Oct 2024
If a clinician is not comfortable letting a patient go for two weeks without follow-up ultrasound or examination, that is a red flag warning sign that the patient needs shunting rather than continued observation. — Jesse Skoch, 2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Jesse Skoch · 18:00
2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Jesse Skoch
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Dr. Jesse Skoch speaks on the Surgical management of Congenital Aqueductal Stenosis on Day 1 of the Frontiers in Fetal Neurology 2-day interactive webinar series.
video19:51 · Oct 2024
Many prenatally identified neurological conditions including agenesis of the corpus callosum and vermian hypoplasia can be categorized as isolated or complex, but complex is a very heterogeneous category requiring better genotype-phenotype characterization. — Charu Venkatesen, 2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Charu Venkatesen · 9:00
2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Charu Venkatesen
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Dr. Charu Venkatesen speaks on the outcomes of patients with prenatal diagnosis of Congenital Aqueductal Stenosis on Day 1 of the Frontiers in Fetal Neurology 2-day interactive webinar series.
video12:51 · Oct 2024
Maintaining decompression for 6-7 weeks in utero without pressure is beneficial even if postnatal redo ETV or shunt placement is needed, because the brain will have developed better. 2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Hour 1 Q&A · 8:12
2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Hour 1 Q&A
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Dr. Charu Venkatesen, Dr. Jesse Skoch, and. Dr. Jose Peiro discuss the hour 1 sessions and answers questions from the audience.
video9:06 · Oct 2024
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2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Introduction
Day 1 session 1 (12:00-1:00 PM) covers congenital aqueductal stenosis, including outcomes and fetal MRI findings
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Day 1 session 2 (1:00-2:00 PM) addresses uncertainties in neurological diagnoses, including fetal intracranial hemorrhage, genetic conditions with brain malformations, and palliative care in fetal counseling
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Day 2 (October 9th) will cover fetal therapeutics, treatment of tuberous sclerosis, and toxic/environmental exposures affecting brain development
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Doctor Jessie Scotch will discuss fetal MRI findings for congenital aqueductal stenosis
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Doctor Don Gano from UCSF will discuss fetal intracranial hemorrhage
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Doctor Sonika Agarwal from CHOP will review genetic conditions and brain malformations
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
Doctor Donna Maria Corteso will review the role of palliative care in fetal counseling
Host summaryCharivenka Tayson summarizing a resource — not the host's own clinical position0:41 ↗
2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Jesse Skoch
Congenital aqueductal stenosis accounts for 5-20% of congenital hydrocephalus etiology, with approximately 600-1500 cases per year in the United States.
epidemiologicalJesse Skoch0:16 ↗
Walter Dandy in the 1920s used a crude cystoscope to burn choroid plexus in hydrocephalic patients, with 24 out of 25 patients dying of complications.
clinicalJesse Skoch2:00 ↗
Dr. Mixer in 1923 is credited with the modern version of ETV involving fenestration of the floor of the third ventricle using a urethroscope.
clinicalJesse Skoch3:00 ↗
Scarf et al. in 1936 published a series of 20 ETV cases with 10 showing long-term improvement, 7 failures, and 3 immediate post-surgical deaths.
clinicalJesse Skoch3:30 ↗
ETV technology reached its modern form in the 1980s with advances in camera and endoscope technology, after being relatively quiescent from the mid-1930s due to the invention of VP shunt pressure-regulated valves in the 1950s.
clinicalJesse Skoch4:00 ↗
Serial slow dilation with balloon catheters (such as 3 French Fogarty catheters) can successfully open the majority of the accessible floor of the third ventricle, even in patients with narrow third ventricles.
clinicalJesse Skoch5:00 ↗
Making the ETV ostomy as large as possible is important in young children because it helps fight against closure in the growing infant brain.
opinionJesse Skoch6:00 ↗
The ETV Success Score assigns 30 points for aqueductal stenosis etiology and 50 points for age greater than 10 years, with points translating directly to percentage success rates.
clinicalJesse Skoch9:00 ↗
Patients with aqueductal stenosis who are greater than 10 years old have an 80% chance of ETV success, while those less than 1 month old have success rates in the 30-50% range.
clinicalJesse Skoch10:00 ↗
ETV procedures carry risks including hemorrhage, injury to the basilar artery, damage to the fornix (which can cause memory loss), and procedure failure.
clinicalJesse Skoch10:30 ↗
Successful ETV patients typically do not show the same degree of ventricular reduction as shunted patients, but developmental outcomes can be very good.
clinicalJesse Skoch12:00 ↗
A visible CSF jet stream (appearing black on sagittal MRI) through the ETV ostomy is a reassuring sign of patency, though not required for success.
clinicalJesse Skoch12:30 ↗
ETV failures occur through four mechanisms: ostomy closure, ostomy narrowing, patent ostomy with membrane formation elsewhere in the third ventricle or basilar cisterns, or insufficient CSF diversion possibly due to mixed communicating hydrocephalus.
clinicalJesse Skoch13:00 ↗
The Toronto group's large study showed that infants less than 1 month old fare poorly with ETV outcomes over time, with a big improvement in success rates at the 6-month age mark.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position14:00 ↗
Mohammadi et al. showed that pre-term versus post-term status makes a significant difference in ETV outcomes, with corrected age being important to consider in premature babies.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position14:30 ↗
According to Rhodes et al., congenital acute aqueductal stenosis (big ventricles from birth with progression) has better neurologic outcomes with shunting than with ETV, while chronic slowly progressive hydrocephalus shows no difference between procedures.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position15:00 ↗
A 2011 meta-analysis showed ETV has a permanent morbidity rate of 2.4% and mortality of 0.21%.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position16:30 ↗
Shunting has an in-hospital mortality of 0.3-0.8%, but Norwegian longitudinal studies over 40 years showed 48% overall mortality with 8% attributed to shunt failures and shunt-related issues.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position17:00 ↗
If an ETV works and shows success at 6 months to 1 year, very few patients will have long-term delayed complications, unlike the constantly ticking time bomb of shunt dependency.
clinicalJesse Skoch17:30 ↗
If a clinician is not comfortable letting a patient go for two weeks without follow-up ultrasound or examination, that is a red flag warning sign that the patient needs shunting rather than continued observation.
opinionJesse Skoch18:00 ↗
Stented ETV using hardware to hold the ostomy open has not panned out well because foreign body reactions can occur at any time in the patient's life, similar to shunt failures.
clinicalJesse Skoch18:30 ↗
Dr. Warf's sub-Saharan African study showed a 30% improvement in ETV success when combined with choroid plexus cauterization, but meta-analysis of other published studies has not replicated those numbers in larger datasets.
Host summaryJesse Skoch summarizing a resource — not the host's own clinical position19:00 ↗
2024 Fetal Care Center Frontiers in Fetal Neurology Day 1 - Dr. Charu Venkatesen
Ventriculomegaly is the most common CNS abnormality identified on fetal imaging.
epidemiologicalCharu Venkatesen0:00 ↗
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