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PITUITARY STALK TRANSECTION SYNDROME : ABOUT FIVE CASES

NR38. PITUITARY STALK TRANSECTION SYNDROME : ABOUT FIVE CASES. H. ZAGHOUANI BEN ALAYA, N. MALLAT, Z. ACHOUR, S. MAJDOUB, H. AMARA, D. BAKIR, CH. KRAIEM Imaging department , Farhat Hached Hospital , Sousse, Tunisia. INTRODUCTION :.

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PITUITARY STALK TRANSECTION SYNDROME : ABOUT FIVE CASES

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  1. NR38 PITUITARY STALK TRANSECTION SYNDROME : ABOUT FIVE CASES H. ZAGHOUANI BEN ALAYA, N. MALLAT, Z. ACHOUR, S. MAJDOUB, H. AMARA, D. BAKIR, CH. KRAIEMImaging department, FarhatHachedHospital, Sousse, Tunisia

  2. INTRODUCTION : • Advances in magnetic resonance imaging (MRI) of the hypothalamic pituitary region may actually explain a number of hormonal disturbances associated with an abnormality of the pituitary stalk • The syndrome of interruption of the pituitary stalk (SIPS) is a rare cause of anterior pituitary deficiencies

  3. It is defined by morphological abnormalities revealed by MRI: • Interruptionorsignificant thinningof the pituitarystalk • ahypoplastic anterior pituitary • anectopicorlacking posterior pituitary • The aim of this work is to illustrate the contribution of MRI in this syndrome through five observations.

  4. MATERIAL AND METHODS : • Retrospective study of 5 patients • Over a period of 5 years from 2006 to 2011 collected from department of medical imaging • The age varies from 09 to 23 years : 4 children aged between 09 and 12 years old and an adult of 23 years • Sex : 3 males and 2 females

  5. The reason for consultation and exploration : • Short stature with anterior pituitary deficit (n=5) • Primaryamenorrhea (n=1) • Diabetesinsipidus (n=1) • A pituitary MRI was performed in all patients : • MRI 1.5 Tesla GE • ThinSagittal and coronal (3mm) Fast spin echo T2 and T1before and after gadolinium injection

  6. RESULTATS : • MRI showed an abnormality of the pituitary stalk in all cases : • Interruption of the pituitary stalk : completetransection (n=4)  and incomplete with a filiform pituitary stalk (n=1) • Ectopic posteriorpituitary gland appearedas an area of high signal intensity in the midline at the median eminence(n=4) • Hypoplasia of the anterior pituitary (n=5) • Absence of the posterior pituitary gland associated with Arnold Chiari I malformation (n=1)

  7. Fig.1 (a,b) : coronal unenhanced T1-weighted MR imaging : ectopicposteriorpituitary gland ( ) seen as an area of high signal intensity in the midline at the medianeminence (a). the pituitary stalk is not visible ( ) (b). • Case 1 :9 year old child, panhypopituitarism (a) (b) Fig.1 (c,d) : Gadolinium-enhanced coronal and midsagittalT1-weighted MR imaging :The pituitary stalk is not visible (b). The ectopic posterior pituitary gland is visible (a,b) (c) (d)

  8. Case 2 :10 year old child, short stature with diabetesinsipidus (a) (b) Fig.2 (a,b) : midsagittalunenhanced and contrast material– enhancedT1-weighted MR imaging : small anterior pituitary gland( ), absence of the habitualpituitary posterior lobe hyperintense signal within the sellaturcica cavity and also within the median eminence (a). the pituitary stalk is also not visible even after administration of Gadolinium ( ) (b). (c) Fig.2 (c) : midsagittal T2 T1-weighted MR imaging : ectopia of cerebellar tonsils ( ) with V4 in place : Arnold Chiari I malformation

  9. Case 3 :12 year old child, short stature with Growth Hormone deficiency Fig.3 (a,b,c) : sagittal and coronal unenhanced T1-weighted MR imaging : small anterior pituitary gland( ) (a), presence of the ectopic posterior pituitary ("bright spot“) at the median eminence level ( ) (b,c).the pituitary stalk is not visible ( ) (b). (a) (b) (c)

  10. Case 4 :23 year old patient, primaryamenorrheawithgonadotropin deficiency Fig.4 : midsagittalunenhancedT1-weighted MR imaging : ectopicposteriorpituitary in hypersignal ( ), small anterior pituitary gland, pituitary stalk interruption in its incomplete form (thin pituitary stalk) ( ).

  11. DISCUSSION : • Definition : • The SITP was first described in 1987 by Fujisawa et al •  It is a syndrome defined by morphological abnormalities revealed by MRI: • a thin  or interruptedpituitary stalk • a hypoplasticanterior pituitary • an ectopic  or absentposterior pituitary 

  12. Physiopathology : • The etiology of pituitary stalk interruption is not completely understood. Two theories have been proposed : • Traumatic theory:facing a high proportion of history of fetal distress, breech presentation and of head trauma in patients with a SIPS • Malformative theory: • The SITP is frequently associated with abnormalities of the midline • Facial dysmorphism may be associated • There is as familial forms • The genetic theory remains the most creditable versus the traumatictheory

  13. Clinical : • SIPS is often revealed in the neonatal period and childhood. His revelation in adults is exceptional • This syndrome is clinically discussed in presence of hypopituitarism: • Isolated most often a Growth hormone deficiency • Multiple with a normal posteriorpituitaryfunction • Classically, there is an isolated GH deficiency if the pituitary stalk is thinand apanhypopituitarism if the pituitary stalk interruption is complete • Isolated GH deficiency can progress to panhypopituitarism and requires biological monitoring for life

  14. Imaging : • The diagnostic strategy of growth retardation currently leaves an important place to imaging and particularly to cerebral MRI thanks to: • its high contrast resolution • its character multiplanar • the absence of bone artifacts  of the base (limit of CT) MRI offers a morphological study of the hypothalamic-pituitary region and search for  associated brain abnormalities  of the midline

  15. The browsingprotocol: • Sagittal and coronalthin (2-3 mm) • Centered on the hypothalamic-pituitary • FSE T1-weightedsequence • FSE T2-weightedsequence • Injection of contrastmaterialparamagnetic The wholebrain must beexplored to eliminate the associated malformations

  16. 1. Anomaly of the posterior pituitary: • It appears in spontaneous hypersignalon T1-weighted  and enhances after gadolinium injection • It is ectopic and it is localized whether at the infundibulum(50%) or at the pituitary stalk or sometimes even in the hypothalamus This ectopic hyperintensity can be located anywhere along the pituitary stalk

  17. 2. Anomaly of the pituitary stalk: • Complete form:not visualized pituitary stalk whatever the sequence • Incomplete form: with a filiform pituitary stalk or visible only after gadolinium injection This classification has a prognostic value, since in the complete forms the hormonal deficiency is most often multiple

  18. 3. Anomaly of the anterior pituitary: • The anterior lobe is often hypoplastic(height less than -2SD compared to normal values according to age) • But even when it is of normal size, the dynamic sequence may reveal, among patients with GH deficiency, delayed contrast enhancement of the anterior pituitary

  19. 4. Malformations of the midline: • Arnold Chiari malformationtype I • Basipharyngial Canal • Total or partial agenesis of the corpus callosum • Agenesis or hypoplasia of the septum and the optic chiasma (septo-opticdysplasia) • Dandy-Walker malformation

  20. If MRI is normal in the context of growth hormone deficiency:   • It has a prognostic impact because the deficit is often incomplete or even transitional • Some studies have shown the  higher frequency of genetic abnormalities in the group of patients with GH deficiency and normal MRI, which allows to select patients for genetic studies

  21. CONCLUSION : • The SIPS is a rare congenital malformation, responsible for most cases of growth hormone isolated deficiency but also for multiple anterior pituitary deficits.  • MRI is currently the most performed imaging means for the diagnosis of this malformation and the prognostic approach : • Morphological study of the hypothalamic-pituitary • Establish clinical and radiological correlations • Detect associated brain malformations.

  22. BIBLIOGRAPHIE : • Van der Linden A S A and Van Es Hendrik W. Case 112: Pituitary Stalk Transection Syndrome with Ectopic Posterior Pituitary Gland. Radiology 2007;243:594–597. • Vijayanand P, Mahadevan S, Shivbalan So et al. PituitaryStalk Interruption syndrome (PSIS). Indian Journal of Pediatrics 2007;74:874-5. • L.Tabelsi, M.Mnif, N.Rekik et al. Anomalies de la tigepituitaire à l’IRM : aspects étiologiques à propos de 11 cas. Ann. Endocrinol. 2006; 67, 6 : 604-612. • G.Zuccoli, F.Nicoli, G.Tognini, F.Ferrozzi. Pituitary stalk interruption syndrome : magnetic resonance findings. URL: http://www.eurorad.org/case.php?id=1591 • Lippincott Williams & Wilkins. Pituitarystalklesions. Curr Opin Endocrinol Diabetes Obes 15:339–345. • C Barbeau, B Jouret, D Gallegos et al. Syndrome d'interruption de la tige pituitaire. Arch Pédiatr 1998 ; 5 : 274-9. Elsevier, Paris. • N. Hammami, C. Drissi, M. Salah, A. Kerkeni, R. Sebai, L. Belghith, S. Nagi, M. Ben Hamouda. S Syndrome d'interruption de la tige pituitaire : à propos de cinq observations. Service de neuroradiologie - Institut national de neurologie de Tunis. JFR 2010.

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