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Research ArticlePEDIATRICS

Ectopic Posterior Pituitary Lobe and Periventricular Heterotopia: Cerebral Malformations with the Same Underlying Mechanism?

L. Anne Mitchell, Paul Q. Thomas, Margaret R. Zacharin and Ingrid E. Scheffer
American Journal of Neuroradiology October 2002, 23 (9) 1475-1481;
L. Anne Mitchell
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Paul Q. Thomas
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Margaret R. Zacharin
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Ingrid E. Scheffer
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  • Fig 1.
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    Fig 1.

    Images from case 1, with typical findings of ectopic posterior pituitary lobe.

    A, Unenhanced midline sagittal spin-echo T1-weighted image (625/13/4) shows hyperintensity corresponding to ectopic posterior pituitary lobe at the median eminence (short arrow). The pituitary gland and sella turcica are small, with a thin infundibulum (long arrow).

    B, Coronal T1-weighted spoiled gradient-recalled image shows a small heterotopic nodule, isointense to gray matter, above the left trigone (arrow).

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    Fig 2.

    Images from case 2.

    A, Unenhanced midline sagittal spin-echo T1-weighted image (625/13/4) shows a small hyperintensity at the median eminence typical of ectopic posterior pituitary lobe, with a second small hyperintensity extending downward into the upper infundibulum (arrows). The infundibulum is thinned, with a small pituitary gland and sella turcica. The splenium of the corpus callosum is small.

    B, Axial fast spin-echo T2-weighted images (5200/102/3) show small heterotopic nodules isointense to gray matter, above the frontal horns (arrowheads).

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    Fig 3.

    Image from case 3. Unenhanced midline sagittal view spin-echo T1-weighted image (625/13/4) shows hyperintensity at the median eminence, typical for ectopic posterior pituitary lobe (short arrow). A small hyperintense focus can also be seen lying posterosuperiorly on the superior aspect of the small pituitary gland (curved arrow), which probably also represents ectopic pituitary tissue. The sella has an abnormal morphology, with a sloping anterior wall. It contains a thin layer of soft tissue lining the floor of the dysplastic anterior sella and more recognizable pituitary tissue in the base of the sella. The infundibulum is difficult to definitely identify because it is markedly thinned but may lie more anteriorly than usual (long arrow).

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    Fig 4.

    Images from case 4.

    A, Unenhanced midline sagittal spin-echo T1-weighted image (625/13/4) shows two brightly hyperintense foci: one involving the mammillary bodies and the other in the interpeduncular cistern (black arrowheads). There is also a smaller less hyperintense focus located at the expected site of the median eminence, suggestive of ectopic posterior pituitary lobe (white arrow). The pituitary gland and sella are small, with a suggestion of a tiny posteriorly angled infundibulum.

    B, Unenhanced sagittal spin-echo T1-weighted image (650/9/2) with fat suppression, obtained at the same position as the image shown in A, shows suppression of the two larger more posteriorly located hyperintensities (black arrowheads), indicating that these represent lipomas. The smaller anterior hyperintensity of ectopic posterior pituitary lobe does not suppress (white arrow). A little flow-related artifact is seen in the floor of the third ventricle between the median eminence and the mammillary bodies.

    C, Contrast-enhanced sagittal spin-echo T1-weighted image (650/9/2) with fat suppression shows enhancement of the median eminence, confirming that the anterior hyperintensity lies at a typical location for ectopic posterior pituitary lobe (short arrow). Enhancement of the infundibulum can also be seen (long arrow). The two lipomas remain suppressed, with no enhancement (arrowheads).

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    TABLE 1:

    Summary of clinical and hormonal abnormalities

    Clinical FeatureCase 1Case 2Case 3Case 4
    Age at presentation2 yr14 mo5 yr 9 mo3 yr 11 mo
    SexMMMF
    Presenting symptomsShort stature, poor growthShort stature, poor growth, episodes suggesting hypoglycemiaShort stature, poor growthShort stature, poor growth
    HeightOn 1st centile<1st centile<1st centile<1st centile
    Antenatal/perinatal eventsIUGR at 34 wk gestation, neonatal hypoglycemiaBreech presentation, elective CSNeonatal hypoglycemiaMaternal hypertension, forceps delivery for fetal distress
    Seizures01 Generalized seizure00
    Family history0Cousin with epilepsy, maternal GM with anosmia00
    Vision/fundiAsymptomatic, n/tNAsymptomatic, n/tN
    Other examination findingsMicropenis, small nasal dimpleMicropenis00
    Maximum GH response to glucagon (normal >20 mI U/L)192733
    Free thyroxine (normal range, 10–25 pmol/L)7.2Total T4 = 110 nmol/L (normal range, 70–155 nmol/L), TSH = 2.1 mIU/L (normal range, <5 mIU/L)6.312.1
    Plasma cortisol (8:30 am) (normal range, 200–750 nmol/L)330546200n/t
    Prolactin (normal range, 50–500 mIU/L)n/tn/t4076
    Response to GH treatmentOn 15th centile>1st centile after 6 moOn 3rd centile (on GH, thyroxine, hydrocortisone)>25th centile
    HESX1 mutation0n/tn/t+ (father and sister also have mutation)
    • Note.—M indicates male; F, female; IUGR, intrauterine growth; CS, caesarian section; GM, grandmother; n/t, not tested; N, normal; 0, absent; GH, growth hormone; TSH, thyroid-stimulating hormone.

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    TABLE 2:

    Summary of MR imaging findings

    MR Imaging FindingCase 1Case 2Case 3Case 4
    Posterior pituitary lobeEPPEPPEPP Second EPP focus on posterosuperior aspect of glandEPP
    Anterior pituitary lobeSmallSmallSmallSmall
    InfundibulumThinThin, hyperintensity at root of infundibulumMarkedly thin, anteriorly placedThin
    Sella turcicaSmallSmallSmall, dysplastic anterior sellaSmall
    Periventricular heterotopia2 Nodules, unilateral, posterior body of lateral ventricleApproximately 7 nodules, bilateral, frontal horns and bodies of lateral ventricles2 Nodules, bilateral, right trigone, left temporal horn1 Nodule, unilateral, left frontal horn
    Septum pellucidum/optic nervesNNNN
    Other abnormalities0Mild callosal dysgenesis02 suprasellar lipomas
    • Note.—EPP, ectopic posterior pituitary lobe; N, normal; 0, absent.

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American Journal of Neuroradiology: 23 (9)
American Journal of Neuroradiology
Vol. 23, Issue 9
1 Oct 2002
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Cite this article
L. Anne Mitchell, Paul Q. Thomas, Margaret R. Zacharin, Ingrid E. Scheffer
Ectopic Posterior Pituitary Lobe and Periventricular Heterotopia: Cerebral Malformations with the Same Underlying Mechanism?
American Journal of Neuroradiology Oct 2002, 23 (9) 1475-1481;

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Ectopic Posterior Pituitary Lobe and Periventricular Heterotopia: Cerebral Malformations with the Same Underlying Mechanism?
L. Anne Mitchell, Paul Q. Thomas, Margaret R. Zacharin, Ingrid E. Scheffer
American Journal of Neuroradiology Oct 2002, 23 (9) 1475-1481;
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