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Absence of the septum pellucidum associated with a midline fornical nodule and ventriculomegaly: a report of two cases.

Chun YK, Kim HS, Hong SR, Chi JG - J. Korean Med. Sci. (2010)

Bottom Line: The posterior portion of the septum pellucidum was absent and the fornices were fused in a single midline nodule, abnormally displaced to a caudal position and lodged in the foramina of Monro.The brain base showed no apparent abnormalities; the optic nerves were well developed.We conclude that the caudally displaced fornix in the absence of the septum pellucidum may have intermittently obstructed the foramina of Monro and induced mild ventriculomegaly.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Cheil General Hospital and Women's Healthcare Center, Kwandong University College of Medicine, Seoul, Korea. ykcmd@naver.com

ABSTRACT
We report two autopsy cases that revealed the partial absence of the septum pellucidum with ventriculomegaly. In each case, the brain showed mild dilatation of both frontal horns of the lateral ventricles, normal third and fourth ventricles and no aqueductal stenosis. The posterior portion of the septum pellucidum was absent and the fornices were fused in a single midline nodule, abnormally displaced to a caudal position and lodged in the foramina of Monro. The brain base showed no apparent abnormalities; the optic nerves were well developed. We conclude that the caudally displaced fornix in the absence of the septum pellucidum may have intermittently obstructed the foramina of Monro and induced mild ventriculomegaly.

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Related in: MedlinePlus

Coronal section of the Case 1 brain through the interventricular foramen. The lateral ventricles are mildly distended and the third ventricle is normal. A single midline fornical nodule (arrow) almost totally obstructs the foramina of Monro. The septum pellucidum is absent in its posterior portion and the corpus callosum seems normal.
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Figure 1: Coronal section of the Case 1 brain through the interventricular foramen. The lateral ventricles are mildly distended and the third ventricle is normal. A single midline fornical nodule (arrow) almost totally obstructs the foramina of Monro. The septum pellucidum is absent in its posterior portion and the corpus callosum seems normal.

Mentions: Pregnancy in a 27-yr-old woman was terminated at 36 weeks of gestation after ultrasonography revealed Potter's syndrome in the fetus. The female fetus weighed 1,500 gm and measured 29 cm in crown-rump length and 43 cm in crown-heel length. The head circumference was 26 cm, chest circumference 24 cm and abdominal circumference 20.2 cm. She showed features typical of Potter's syndrome: agenesis of the left kidney with cystic dysplasia of the right kidney, calcaneovarus, pulmonary hypoplasia and exaggerated facial creases. Spina bifida with myeloschisis was present in the lumbosacral area. The brain weighed 294 gm and revealed no abnormalities of gyration, sulcation or cranial nerves. The brain base showed well developed optic chiasma and optic nerves. The optic nerve measured 2.5 mm in diameter. After coronal sectioning, the cerebral hemispheres showed mildly distended lateral ventricles. The corpus callosum was well preserved, which measured 3.0 mm and 1.0 mm in thickness in genu and body, respectively. The anterior commissure appeared normal. The septum pellucidum was absent in its posterior portion. Both fornices was fused to form a single midline nodule, which was inferiorly displaced (Fig. 1). The foramina of Monro were occluded by this round fornical nodule of an abnormally low position. The hippocampus was well formed and fimbria was also present in its usual position and size. The third and fourth ventricles were not dilated. Microscopically, there was no evidence of malformations of cerebral cortical development. Chromosomal analysis of the peripheral blood revealed 46, XX. Maternal serum and urinary estriol and fetal pituitary hormones were not studied.


Absence of the septum pellucidum associated with a midline fornical nodule and ventriculomegaly: a report of two cases.

Chun YK, Kim HS, Hong SR, Chi JG - J. Korean Med. Sci. (2010)

Coronal section of the Case 1 brain through the interventricular foramen. The lateral ventricles are mildly distended and the third ventricle is normal. A single midline fornical nodule (arrow) almost totally obstructs the foramina of Monro. The septum pellucidum is absent in its posterior portion and the corpus callosum seems normal.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2877245&req=5

Figure 1: Coronal section of the Case 1 brain through the interventricular foramen. The lateral ventricles are mildly distended and the third ventricle is normal. A single midline fornical nodule (arrow) almost totally obstructs the foramina of Monro. The septum pellucidum is absent in its posterior portion and the corpus callosum seems normal.
Mentions: Pregnancy in a 27-yr-old woman was terminated at 36 weeks of gestation after ultrasonography revealed Potter's syndrome in the fetus. The female fetus weighed 1,500 gm and measured 29 cm in crown-rump length and 43 cm in crown-heel length. The head circumference was 26 cm, chest circumference 24 cm and abdominal circumference 20.2 cm. She showed features typical of Potter's syndrome: agenesis of the left kidney with cystic dysplasia of the right kidney, calcaneovarus, pulmonary hypoplasia and exaggerated facial creases. Spina bifida with myeloschisis was present in the lumbosacral area. The brain weighed 294 gm and revealed no abnormalities of gyration, sulcation or cranial nerves. The brain base showed well developed optic chiasma and optic nerves. The optic nerve measured 2.5 mm in diameter. After coronal sectioning, the cerebral hemispheres showed mildly distended lateral ventricles. The corpus callosum was well preserved, which measured 3.0 mm and 1.0 mm in thickness in genu and body, respectively. The anterior commissure appeared normal. The septum pellucidum was absent in its posterior portion. Both fornices was fused to form a single midline nodule, which was inferiorly displaced (Fig. 1). The foramina of Monro were occluded by this round fornical nodule of an abnormally low position. The hippocampus was well formed and fimbria was also present in its usual position and size. The third and fourth ventricles were not dilated. Microscopically, there was no evidence of malformations of cerebral cortical development. Chromosomal analysis of the peripheral blood revealed 46, XX. Maternal serum and urinary estriol and fetal pituitary hormones were not studied.

Bottom Line: The posterior portion of the septum pellucidum was absent and the fornices were fused in a single midline nodule, abnormally displaced to a caudal position and lodged in the foramina of Monro.The brain base showed no apparent abnormalities; the optic nerves were well developed.We conclude that the caudally displaced fornix in the absence of the septum pellucidum may have intermittently obstructed the foramina of Monro and induced mild ventriculomegaly.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Cheil General Hospital and Women's Healthcare Center, Kwandong University College of Medicine, Seoul, Korea. ykcmd@naver.com

ABSTRACT
We report two autopsy cases that revealed the partial absence of the septum pellucidum with ventriculomegaly. In each case, the brain showed mild dilatation of both frontal horns of the lateral ventricles, normal third and fourth ventricles and no aqueductal stenosis. The posterior portion of the septum pellucidum was absent and the fornices were fused in a single midline nodule, abnormally displaced to a caudal position and lodged in the foramina of Monro. The brain base showed no apparent abnormalities; the optic nerves were well developed. We conclude that the caudally displaced fornix in the absence of the septum pellucidum may have intermittently obstructed the foramina of Monro and induced mild ventriculomegaly.

Show MeSH
Related in: MedlinePlus