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Non-visualisation of cavum septi pellucidi: implication in prenatal diagnosis?

Hosseinzadeh K, Luo J, Borhani A, Hill L - Insights Imaging (2013)

Bottom Line: This manuscript reviews congenital anomalies and imaging findings associated with non-visualisation of the cavum septi pellucidi (CSP) found on prenatal sonogram.Isolated septal deficiency, a rare but controversial entity, is considered a variant of normal.Common pitfalls in the sonographic evaluation of CSP include columns of the fornix that mimic CSP, and prominent cavum vergae that can simulate non-visualisation of the CSP.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Presbyterian South Tower, University of Pittsburgh Medical Center, 200 Lothrop Street, Suite 3950, Pittsburgh, PA, 15213, USA, hosseinzadehk@upmc.edu.

ABSTRACT

Objective: This manuscript reviews congenital anomalies and imaging findings associated with non-visualisation of the cavum septi pellucidi (CSP) found on prenatal sonogram.

Background: Observation of a normal cavum septi pellucidi (CSP) is an important landmark in the second and third trimester prenatal ultrasound evaluation of the fetal brain, and its visualisation provides reassurance of normal central forebrain development. Non-visualisation of the CSP is a prenatal sonographic finding, which in most cases is associated with neuroanatomical anomalies that include agenesis of the corpus callosum, schizencephaly, septo-optic dysplasia, holoprosencephaly, chronic hydrocephalus and acquired fetal brain injury. Isolated septal deficiency, a rare but controversial entity, is considered a variant of normal. Common pitfalls in the sonographic evaluation of CSP include columns of the fornix that mimic CSP, and prominent cavum vergae that can simulate non-visualisation of the CSP. When non-visualisation of the CSP is suspected, magnetic resonance imaging (MRI) of the fetal brain can confirm and evaluate associated anomalies.

Conclusion: Visualisation of the CSP is an integral component of the prenatal ultrasound and its non-visualisation is associated with other malformations, diagnosis of which is aided by MRI.

Teaching points: • Cavum septi pellucidi (CSP) is an important landmark in the prenatal ultrasound evaluation of the fetal brain, and is a marker for normal central forebrain development. • Non-visualisation of the CSP is most commonly associated with other neuroanatomical abnormalities. • Examination of the fetal brain by MRI can confirm the sonographic findings and evaluate for associated anomalies.

No MeSH data available.


Related in: MedlinePlus

SOD. a Coronal ultrasound through the frontal horns demonstrates non-visualisation of the CSP and “squared off” morphology of the frontal horns (asterisk) suggestive of SOD. Columns of fornix are inferior to the CSP (arrows). b Coronal T2-weighted MRI displays characteristic “batwing” configuration of the frontal horns with communication between the horns (asterisk). c Axial T2-weighted MRI at 32 weeks of gestation confirms the absence of CSP and colpocephaly. There is separation of the foremost portion of the anterior horns (arrows). No cortical dysplasia was present. Postnatal ophthalmological evaluation was consistent with optic nerve hypoplasia
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Fig4: SOD. a Coronal ultrasound through the frontal horns demonstrates non-visualisation of the CSP and “squared off” morphology of the frontal horns (asterisk) suggestive of SOD. Columns of fornix are inferior to the CSP (arrows). b Coronal T2-weighted MRI displays characteristic “batwing” configuration of the frontal horns with communication between the horns (asterisk). c Axial T2-weighted MRI at 32 weeks of gestation confirms the absence of CSP and colpocephaly. There is separation of the foremost portion of the anterior horns (arrows). No cortical dysplasia was present. Postnatal ophthalmological evaluation was consistent with optic nerve hypoplasia

Mentions: SOD (De Morsier Syndrome) is a congenital malformation syndrome that includes non-visualisation of the CSP, optic nerve/chiasm hypoplasia and hypothalamic pituitary dysfunction. It is a rare disorder, with an incidence of 1 in 50,000 live births [20]. Optic nerve hypoplasia can result in varying degrees of visual impairment and can be either unilateral or bilateral. Patients with SOD may have deficiencies of anterior pituitary gland hormones, resulting in hyperprolactinemia, hypothyroidism, diabetes insipidus and dwarfism [21]. Children with SOD should have regular endocrinology follow-up as hormonal insufficiencies may progress over time. Additional prenatal imaging findings associated with SOD include optic nerve hypoplasia, communication between anterior horns of lateral ventricles with separated foremost portions, mild ventriculomegaly and squared shape of anterior horns on coronal image (“batwing” configuration) (Fig. 4). However, since optic nerve/chiasm pathologies cannot be adequately evaluated on imaging, diagnosis of SOD cannot be confidently made with imaging alone, often requiring post-natal neuro-opthalmic evaluation for final diagnosis.Fig. 4


Non-visualisation of cavum septi pellucidi: implication in prenatal diagnosis?

Hosseinzadeh K, Luo J, Borhani A, Hill L - Insights Imaging (2013)

SOD. a Coronal ultrasound through the frontal horns demonstrates non-visualisation of the CSP and “squared off” morphology of the frontal horns (asterisk) suggestive of SOD. Columns of fornix are inferior to the CSP (arrows). b Coronal T2-weighted MRI displays characteristic “batwing” configuration of the frontal horns with communication between the horns (asterisk). c Axial T2-weighted MRI at 32 weeks of gestation confirms the absence of CSP and colpocephaly. There is separation of the foremost portion of the anterior horns (arrows). No cortical dysplasia was present. Postnatal ophthalmological evaluation was consistent with optic nerve hypoplasia
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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Fig4: SOD. a Coronal ultrasound through the frontal horns demonstrates non-visualisation of the CSP and “squared off” morphology of the frontal horns (asterisk) suggestive of SOD. Columns of fornix are inferior to the CSP (arrows). b Coronal T2-weighted MRI displays characteristic “batwing” configuration of the frontal horns with communication between the horns (asterisk). c Axial T2-weighted MRI at 32 weeks of gestation confirms the absence of CSP and colpocephaly. There is separation of the foremost portion of the anterior horns (arrows). No cortical dysplasia was present. Postnatal ophthalmological evaluation was consistent with optic nerve hypoplasia
Mentions: SOD (De Morsier Syndrome) is a congenital malformation syndrome that includes non-visualisation of the CSP, optic nerve/chiasm hypoplasia and hypothalamic pituitary dysfunction. It is a rare disorder, with an incidence of 1 in 50,000 live births [20]. Optic nerve hypoplasia can result in varying degrees of visual impairment and can be either unilateral or bilateral. Patients with SOD may have deficiencies of anterior pituitary gland hormones, resulting in hyperprolactinemia, hypothyroidism, diabetes insipidus and dwarfism [21]. Children with SOD should have regular endocrinology follow-up as hormonal insufficiencies may progress over time. Additional prenatal imaging findings associated with SOD include optic nerve hypoplasia, communication between anterior horns of lateral ventricles with separated foremost portions, mild ventriculomegaly and squared shape of anterior horns on coronal image (“batwing” configuration) (Fig. 4). However, since optic nerve/chiasm pathologies cannot be adequately evaluated on imaging, diagnosis of SOD cannot be confidently made with imaging alone, often requiring post-natal neuro-opthalmic evaluation for final diagnosis.Fig. 4

Bottom Line: This manuscript reviews congenital anomalies and imaging findings associated with non-visualisation of the cavum septi pellucidi (CSP) found on prenatal sonogram.Isolated septal deficiency, a rare but controversial entity, is considered a variant of normal.Common pitfalls in the sonographic evaluation of CSP include columns of the fornix that mimic CSP, and prominent cavum vergae that can simulate non-visualisation of the CSP.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Presbyterian South Tower, University of Pittsburgh Medical Center, 200 Lothrop Street, Suite 3950, Pittsburgh, PA, 15213, USA, hosseinzadehk@upmc.edu.

ABSTRACT

Objective: This manuscript reviews congenital anomalies and imaging findings associated with non-visualisation of the cavum septi pellucidi (CSP) found on prenatal sonogram.

Background: Observation of a normal cavum septi pellucidi (CSP) is an important landmark in the second and third trimester prenatal ultrasound evaluation of the fetal brain, and its visualisation provides reassurance of normal central forebrain development. Non-visualisation of the CSP is a prenatal sonographic finding, which in most cases is associated with neuroanatomical anomalies that include agenesis of the corpus callosum, schizencephaly, septo-optic dysplasia, holoprosencephaly, chronic hydrocephalus and acquired fetal brain injury. Isolated septal deficiency, a rare but controversial entity, is considered a variant of normal. Common pitfalls in the sonographic evaluation of CSP include columns of the fornix that mimic CSP, and prominent cavum vergae that can simulate non-visualisation of the CSP. When non-visualisation of the CSP is suspected, magnetic resonance imaging (MRI) of the fetal brain can confirm and evaluate associated anomalies.

Conclusion: Visualisation of the CSP is an integral component of the prenatal ultrasound and its non-visualisation is associated with other malformations, diagnosis of which is aided by MRI.

Teaching points: • Cavum septi pellucidi (CSP) is an important landmark in the prenatal ultrasound evaluation of the fetal brain, and is a marker for normal central forebrain development. • Non-visualisation of the CSP is most commonly associated with other neuroanatomical abnormalities. • Examination of the fetal brain by MRI can confirm the sonographic findings and evaluate for associated anomalies.

No MeSH data available.


Related in: MedlinePlus