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Management of Fetal hydrocephalus
1. Management of Fetal hydrocephalus Aboubakr Elnashar Benha university Hospital, Egypt
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2. (A) Normal atrium of lateral ventricle: <10 mm (B) Ventriculomegaly: Mild: 10-15 mm (C) Severe: >15 mm
Diagnosis
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3. (A)Normal: Bright echogenic choroid plexus (CP) entirely fills the lumen of the atrium, being closely apposed to both medial and lateral walls of the ventricle (arrowheads). (B)Ventriculomegaly: anterior displacement of the shrunken choroid plexus that appears clearly detached from the medial wall of the ventricle. FH, Frontal Horns of Lateral Ventricles; Ant, Anterior; Post, Posterior.
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5. Ventriculomegaly, also known as hydrocephalus, occurs when cerebrospinal fluid collects intracranially, resulting in enlargement of the ventricular system.
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6. Management
Depend on
1.gestational age at diagnosis
2.presence of other anomalies
3.results of the karyotype
4.infectious studies
5.views of the parents.
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7. If the diagnosis is made prior to fetal viability: patient may consider pregnancy termination.
If the diagnosis is made after viability or the couple chooses to continue with the pregnancy: the following procedures can be performed (depending on the circumstances)
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8. I. isolated or associated with other congenital anomalies.
Isolated ventriculomegaly is associated in 3% of cases with chromosomal anomalies.
If associated with other defects, this figure rises to 36%.
The most common associated anomaly (25- 30%), is spina bifida, followed by other defects (CNS, renal, GIT) in 7-15%
1.Fetal echocardiogram to check for cardiac anomalies
2.Amniocentesis to analyze the fetal karyotype
3.Maternal testing to check for recent or current infections
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9. 4. Genetic counseling: X-linked recessive aqueductal stenosis carries a 1 in 4 risk of recurrence for future pregnancies and a 1 in 2 risk for male fetuses. Cerebellar agenesis with hydrocephalus is rare but may also be sex-linked and thus have a similar recurrence risk.
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10. II. Degree of ventriculomegaly
Mild: > 10 mm cortical thickness + normal BPD Severe: < lOmm cortical thickness + abnormally increased BPD
Atrium of lateral ventricle: <10 mm Mild: 10-15 mm Severe: >15 mm
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11. Follow up
1.If it is an isolated and is mild to moderate, serial scans to follow the progression and/or regression. 2. Attempt to carry the pregnancy until fetal lung maturity
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12. Timing of delivery 1. Associated with other defects or chromosomal abnormalities: couple should be counselled about termination of the pregnancy 2. No clear indication for preterm delivery if the hydrocephalus is rapidly progressive prior to fetal lung maturity {respiratory distress syndrome, which would delay shunt placement, could actually worsen the final outcome}. 3. If the hydrocephalus is rapidly progressing and delivery is necessary prior to lung maturity: corticosteroids {decrease the severity of RDS}.
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13. Mode of delivery
1.CS: a. isolated disease and moderate to severe macrocephaly {facilitate the atraumatic delivery of the enlarged fetal head}. b. Macrocrania is present
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14. 2. Vaginal delivery
a.vertex presentation and has only mild macrocephaly.
b.Associated anomalies that are either incompatible with life or associated with the severest forms of neurologic dysfunction e.g., alobar holoprosencephaly, hydrancephaly, or thanatophoric dysplasia with cloverleaf skull), cephalocentesis and subsequent vaginal delivery are an acceptable alternative to cesarean delivery.
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15. Cephalocentesis prior to delivery –
This is a destructive procedure.
done to reduce the cranial size and potentially allow for vaginal delivery.
This is associated with significant fetal/neonatal morbidity and is indicated only in cases where the prognosis is thought to be extremely poor.
performed by passing a 14- to 18-gauge needle transabdominally or transvaginally under US guidance, and removing sufficient cerebrospinal fluid to allow overlapping of the cranial sutures.
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16. Ventriculo-amniotic shunt
Placement of a tube between the fetal ventricular system and the amniotic cavity to potentially reduce pressure preliminary experiments on human fetuses are not encouraging.
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17. Shunting after birth prognosis is usually improved when this occurs. outcome is better if performed before 6 months of age. If operative tt is not delayed, most cases of hydrocephalus are compatible with normal physical development and normal head size
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