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eISSN: 2083-8441
ISSN: 2081-237X
Pediatric Endocrinology Diabetes and Metabolism
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SCImago Journal & Country Rank
3/2019
vol. 25
 
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abstract:
Educational article

Distinguishing between post-trauma pituitary stalk disruption and genetic pituitary stalk interruption syndrome – case presentation and literature overview

Anna Ruszała
1
,
Małgorzata Wójcik
1
,
Andrzej Krystynowicz
2
,
Jerzy B. Starzyk
1

  1. Department of Pediatric and Adolescent Endocrinology, Chair of Pediatrics, Pediatric Institute, Jagiel-lonian University Medical College, Krakow, Poland
  2. Department of Radiology, Children’s University Hospital in Krakow, Poland
Pediatr Endocrinol Diabetes Metab 2019; 25 (3): 155-162
Online publish date: 2019/09/24
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Introduction
The diagnosis of post-trauma pituitary stalk transection, which is often life-threatening condition, is frequently delayed. In medical litera-ture still exist conflicting data concerning distinguishing this pathology with genetic developmental pituitary stalk interruption syndrome (PSIS).

Case presentation
We present a case of patient with post-trauma pituitary stalk transection resulting in combined life-threatening pituitary hormone defi-ciency (CPHD) and typical MRI picture: atrophic not visible stalk and posterior pituitary and hypotrophic anterior pituitary with most typical for this disorders hyperintense signal of distal regenerating axon of hypothalamus (pseudo posterior lobe) at median eminence with not visible posterior pituitary. This latter finding is often confused with ectopic posterior lobe in genetically determined PSIS.

Conclusions
MRI image together with medical history of the head trauma and its strict temporal relation with transient diabetes insipidus and the occurrence of CPHD signs, as well as the lack of extrapituitary midline defects differentiate posttraumatic pituitary stalk transection syndrome (PSTS) from genetic PSIS. In every case of severe traumatic head injury hormonal evaluation and MRI of hypothalamic-pituitary axis should be performed.

keywords:

combined pituitary hormone deficiency (CPHD), pituitary stalk interruption syndrome (PSIS), post trauma pituitary stalk disruption (PSTS), MRI


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